Clinical Exam Flashcards

1
Q

Dextrocardia investigations and associations

Mesocardia is heart in midline

A

Cardiac anomalies associated include mirror image, clinically corrected TGA, single ventricle with PS, TA, or TS, TOF, can be normal in 2%

Situs solitus or inversus using AXR of the atria (midline liver indicates situs ambiguous or heterotaxy)

ECG to review atria and ventricle orientation
p axis and SA node in RA position, q waves in V1/2 L loop ventricles and in V6 D loop
Arrhythmia

Echo to review IVC and great vessels

Primary ciliary dyskinesia in Kartanger syndrome
(PCD)
Heterotaxy: aspleenia syndrome (ivemarks syndrome) and polysplenia syndrome

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2
Q

Pain management strategy

A

Emotional/psychological aspects addressed
WHO analgesia triad
Review frequently

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3
Q

What does TESTS stand for

A
To check diagnosis
Etiology and differentials
Severity
Treatment baseline
Suspected complications
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4
Q

Surgical sieve

A
Vascular
Infective inflam
Trauma
Autoimmune
Metabolic
Iatrogenic 
Neoplastic
Congenital
Developmental or degenerative
Endocrine or environment
Functional
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5
Q

Social history mnemonic

A

Pat fat hams

Patient
Family
Home
Access
Money
Smoking
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6
Q

Don’t forget systems review mnemonic

A

This misbehaving bony pubertal sheep

Teeth
Hopes
Incontinence or toilet training
Sz

Behaviour

Bones/immobility complications

Puberty

Sleep
Hearing
Eyes
Examiners ask that I didn't ask
Pain
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7
Q

Genetic causes of developmental delay and other medical to screen for

A
Medical: 
Hearing and vision 
thyroid (hypothyroid)
Iron and B12 (malnutrition)
Metabolic causes
Abuse or Neglect
Brain imaging
EEG or lead (neurotoxin)

Genetic with genetic test:
Fragile x
Downs
Rett syndrome

Other from history: fetal alcohol, TOrCH, perinatal, other cerebral insult

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8
Q

Wilms tumour associations

A
Hemi hypertrophy
Genitourinary d/o
Syndrome:
- WAGR
(Wilms, aniridia, GU abnormalities and mental retardation)
- Denys-drash syndrome
- BWS
-trisomy 18
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9
Q

Hypertension causes

A

MONSTER
Cardiac - pulses, Bp, murmur

Medications
Obesity (steroids/Cushing syndrome/Phaeo)
Neonatal history
Symptoms and signs and syndromes (collagen and NF, TS, SLE)
Trends in family
Endocrine or
Renal (think of aldosterone and potassium - cramp)

White coat HTN

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10
Q

CF management

A

“The CF plan”

Team approach including GP
Hope - life expectancy steadily improving, novel therapies
Educational status - need information from reliable sources

Compliance/prevent and screen for Complications: diabetes, gord, Cor pulmonale, pneumothorax, bronchiectasis, asthma, intestinal obstruction, arthritis, osteoporosis, jaundice, nasal polyps and sinusitis
Family and sibling Emotional status

Prognosis (life limiting disorder)
Long term infections and resistance
Asospermia with Delayed puberty (males infertile)
Nutrition - often have growth failure and vit def (DEKA)

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11
Q

Causes of cafe au lai

A
neurofibromatosis type 1 and 2
McCune Albright syndrome
ataxia telangiectasia
fanconi anemia
Russell-silver syndrome
Tuberous sclerosis
Gaucher disease
Chediak – Higashi syndrome
Turner syndrome
Proteus syndrome
Legius syndrome
LEOPARD
Bloom syndrome
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12
Q

Clubbing causes

A
CLUBBING 
Mostly cardio/respiratory:
Congenital Cyanotic heart disease or reversal L to R shunt or eisenmenger complex/atrial myxoma 
Lung:
Abscess
Bronchiectasis
CF
(Don't say asthma)
Empyema
Fibrosis

UC/CD (IBD)
Biliary cirrhosis usually or other cirrhosis cause or chronic hepatitis
Birth defects/familial
IE
Neoplasm
GI malabsorption (coeliac)
Other: thyroid acropatchy/TB/sickle cell/pulm AV malformation

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13
Q

Hypotonic baby causes and management

A

Distribution and course of disease (degeneration or static)

Investigations: Rule out sepsis and electrolyte imbalance (mg and ca), left, urine drug screen, TFT, TORCH, EEG prognosis, CK, MRI, metabolic tests

Causes: central (cerebral due to HIE, haemorrhage, malformation, chromosomal, syndrome (Lowe), peroxisomal - zellweger and adrenal leukodystophies, metabolic like pompe, drug) and peripheral causes (including muscle myopathies and dystrophies, neuromuscular junction (botulism MG), peripheral n (CMT) anterior horn cell (SMA))

Management:
Monitor contracture/hip dislocation
Swallow/feeding safety (oro-motor dysfunction)
Reflux
Respiration and overnight oximetry 
Syndromes associations
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14
Q

Complications of childhood obesity

A

CV: High lipids, HTN, LVH, Risk heart disease stroke
Respiratory: sleep disordered breathing, Sleep apnoea, asthma
Renal
GIT: Non alcoholic fatty liver disease, Gallstones, GORD
Orthopaedic: SUFE, Risk degenerative joint disease
Haem: Thrombus
Surgical: Hernia
Endocrine: dm2, Precocious puberty, PCOS
Neuropsych: Pseudotumor cerebri, psychosocial, negatively affects school performance

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15
Q

Causes of hepatomegaly

A
CHIMPS
CT d/o
Haematological
Infective and infiltrative
Metabolic
Parenchymal (cardiac fluid overload)
Systemic
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16
Q

Clinical signs of chronic liver disease

A
Don't forget hep b at the FLAP JAP SEX CLUB
Hepatic flap
Jaundice
Ascites/atrophy testicles with gunaecomastia/anaemia
Palmar erythema
Spider naevi
Encephalopathy
Xanthelasma
Clubbing/colour change nails
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17
Q

Hurlers syndrome

A

HURLERS a mucopolysaccharoidosis (lysosomal disorder)

HSM
Unusual facial features
Recessive
Liduronidase def
Eyes clouded
Retardation 
Short stubby fingers
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18
Q

Marfans

A
MVP
Aortic aneurysm
Retinal detachment
Fibrillin gene on chromosome 15
Arachnodactyly
PNeumothraces
Skeletal abn
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19
Q

Causes of obesity

A

Overeating and under activity

Drugs

Cushing

Genetic such as prader willi

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20
Q

Steroid side effects

A

CUSHINGOID MAP
Cataracts/ Cushing moon face and buffalo hump (interscspjlar adiposity)
Ulcers - ulcers and duodenal
Skin striae, thin, bruise
HTN hirsutism hyperglycaemia heart failure
InfectionsImmunosuppressive with poor wound healing
Necrosis (avascular necrosis femoral head)
Glycosuria/growth (short)
Osteoporosis/obesity
ICP pseudotumour cerebri
Diabetes/ glucose intolerance

Myopathy (proximal) mood
Acne fat/ adrenal (suppressed hypothesis-pit-axis)
Pancreatitis psychosis/behavioural

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21
Q

Macrocephaly causes

A

Megalencephaly (large brain)
Hydrocephalus - communicating and noncommunicating choroid plexus papilloma increases production
Hyperostosis (bone overgrowth): bossing - osteogenesis imperfecta, chronic haemolytic anaemia, rickets, achondroplasia
Familial
Sturg-Weber/NF1/TS/sotos/weaver/Noonan/Costello/gorlin
Fragile x, leukodystrophies, metabolic
Tumour
abscess
NAI
AV malformation great vein of Galen (bruit temporal areas..)

(Autism kids 25% have this)

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22
Q

Causes of microcephaly

A

Genetic: trisomies: downs, Edward, patau, deletions 4p (wolf-hirschhorn) and 5p (cri-du-chat), 22q11 and 7q11.23 (Williams), smith-Leslie-opitz, Cornelia de lange, rett, Nijmegen breakage, ataxia telangiectasia

Stroke/trauma/cerebral injury

TORCH zika
Fetal hydantoin or alcohol syndrome
Perinatal insult

Inborn error of metabolism

Other: lead, chronic renal failure, anaemia, hypothyroid, congenital heart disease, malnutrition

Rett syndrome decelerated growth acquired microcephaly

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23
Q

Causes of short stature

A

Familial
Constitutional delay
Idiopathic
Endocrine - hypopit, GH def, hypothyroid, pSeudohypoparathyroidism, CAH, Cushing

Chronic disease - anaemia, endocrine: hypothyroid, GIT: coeliac/IBD, renal and liver
GH deficiency (order IGF-1)

Syndrome - Genetic such as turners syndrome, others like downs syndromes
Skeletal dysplasia - Chondrodystrophy (achondroplasia)
Scoliosis
Side effects of Drugs - chronic steroid use, ADHD meds, anticonvulsants, radiotherapy
IUGR
Nutritional deficiency
Schwachman-diamond syndrome
Klippel-Fiel
Seckel syndrome

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24
Q

Tall stature causes

A
Familial or normal variant obesity
Klinefleter
Marfan
Precocious puberty
Kallman 
Proteus syndrome
MEN2B
NF1
Fragile x
Sotos 
Weaver
BWS
Homocysteinuria 
Hyperthyroidism
GH excess, acromegaly
McCune Albright
Hyperthyroidism
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25
Q

Mid parental height formula

A

Father + mother +/- 13cm

Divided by 2 (+/- 10cm)

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26
Q

Three extra primitive reflexes

A

Galant
Swimming
Parachute

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27
Q

Jaundice causes

A

Haemolysis (G6PD)
Haemoglobinopathies
Infection/inflammation
Reduced clearance: Gilbert’s, crigler-najjar, hypothyroid
Biliary atresia/allagile/choledochal cyst
Metabolic (galactosemia and tyrosinaemia)
Sepsis
TPN related

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28
Q

Splenomegaly causes

A

Hyperplasia: due to haemolysis
Infiltrative: such as amyloid or sarcoidosis
Congestive: (portal HTN from cirrhosis)
Neoplastic: such as ALL/lymphoma
Storage:such as gaucher hurler Neiman pick
Infections: (EBV CMV malaria)
Autoimmune

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29
Q

ADHD management extra information gathered by

A

Connors scale
Teacher report
Educational psychology assessment

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30
Q

Complications haemochromatosis

A

Haemochromatosis can cause deposits anywhere

Hypogonadism 
Cancer
Cirrhosis
Cardiomyopathy 
DM
Arthropathy
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31
Q

Down’s syndrome important conditions

A

Cardiovascular: CHD especially endocaridal cushion defects/AVSD
Respiratory: sleep disordered breathing
Gastrointestinal: hirsprungs, atresia, coeliac, constipation
Endocrine: Thyroid dysfunction, obsess the risk
Genitourinary:
Musculoskeletal: Hip abnormalities, Atlanto-axial instability, scoliosis
Neurological: seizures, psychiatric such as depression, Alzheimer’s after 35y, abuse
Immune-Haematological: Leukaemias/ immune dysfunction, iron deficiency
Dermatological
Eye disease: cataracts and refractive errors
Hearing/ENT: serous otitis media, hearing impairment

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32
Q

Causes of intellectual disability

A

Prenatal: chromosomal - trisomy, fragile x, 22q11 deletion, genetic - TS, metabolic, syndromic - Williams, Prader-Willi, Cornelia de Lange, infections TORCH, toxins and drugs and major structural abnormalities
Perinatal: HIE, stroke, trauma, infection, hypoglycaemia
Post-natal: Injury, infection, poisoning

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33
Q

Grading murmur

A

1-6
4 = thrill
5 = heard with stethoscope just on skin
6 = heard without stethoscope

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34
Q

Continuous murmur differential

A
PDA
Operative Shunt murmur
AV fistula
Ruptured sinus of valsava 
Severe coarctation
Left coronary artery origin from pulmonary artery anomaly
Anomalies of origin of the pulmonary artery
Truncus 
  • aortic-pulmonary communications
  • arteriovenous communications (fistulas)
  • changes in arterial flows
  • changes in veins flows
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35
Q

Holosystolic murmur causes and continuous murmur causes

A

VSD
MR
TR

Continuous murmur:
PDA
Venous hum
CoA
Surgical shunt such as BT
AV fistulae
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36
Q

The four causes of a diastolic murmur

A

AR
PR
TS
MS

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37
Q

Flu vaccine funded for?

A
  • asthma and regularly use a preventer medication (brown, orange, red or purple inhaler)?
  • under 5 years and has a significant respiratory illness or been in hospital for a respiratory illness (such as pneumonia, bronchiolitis, asthma)?

does your child have a heart condition (such as congenital heart disease or rheumatic heart disease)?

does your child have cancer?

does your child have an ongoing chest (respiratory) condition, such as bronchiectasis or cystic fibrosis?

does your child have diabetes?

does your child have ongoing kidney (renal) disease?

does your child have HIV or AIDS, an auto-immune disease or another immune deficiency?

Flu immunisation is also recommended for those sharing a house with children and young people with long term (chronic) medical conditions. The flu spreads rapidly within households and children are particularly efficient spreaders. It may not be free for household members - they could ask their employer about free or subsidised flu immunisation as many employers offer this to their employees. Flu immunisation is free for some adults; for example, all those over 65 years of age and those with certain long term conditions.

Flu immunisation is recommended and free to pregnant women

Flu immunisation offers protection in pregnancy. Flu is likely to be more severe in pregnancy and can affect the mother and the unborn baby. Immunisation also offers some protection to the newborn baby.

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38
Q

Causes of ptosis

A
  1. Neurogenic:
    - third nerve palsy,
    - horners syndrome
  2. Myogenic:
    - myasthenia gravis,
    - myopathy,
    - myotonic dystrophy,
    - congenital
  3. Mechanical
    - trauma
    - eyelid oedema
    - inflammation of eyelid
    - neoplastic
  4. Neurotoxic:
    - envenomation
    - chronic opioid abuse
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39
Q

Management approach to obesity

A

Causes

  • Lifestyle (sedentery, diet)
  • metabolic: Cushings, hypothyroidism
  • inherited
  • drugs: antipsychotics, antiepileptics, HAART

Consequences:

  • Accelerated atherosclerosis
  • Dyslipidaemia
  • diabetes
  • hypertension
  • sleep disordered breathing
  • restrictive ventilation defects
  • fatty liver
  • FSGS
  • Osteoarthritis
  • Psychological

Management

  • lifestyle factors initially (evidence for short term LOW)
  • Diet: low fat, low calories< 1500kcal, low salt<2g
  • exercise: mod workload for 30mins most days of the week
  • involve dietitian, family, exercise trainer
  • Review regularly, aiming weight loss of 10-15%
  • Pharmacological therapy (limited benefit): orlistat (fat binder, Cx GIT/steatorrhoea), fentomene (appetite suppressant Cx cardiomyopathy), topiramate (off label), metformin (if have insulin resistance)
  • bariatric surgery (evidence for long term weight loss): lap banding, sleeve gastrectomy, gastric bypass
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40
Q

Causes of myopathy

A
  • Hereditary muscular dystrophy
  • Congenital myopathies - rare
  • Acquired: PACE, PODS
  • —polymyositis, dermatomyositis
  • –alcohol
  • –Carcinoma
  • –Endocrine (hypothyroid, hyperthyroid, Cushings, Acromegaly, hypopituitarism)
  • –Periodic paralysis
  • –Osteomalacia
  • –Drugs (chloroquine, steroids, statins, fibrates)
  • –Sarcoid
  • –Myasthenia Gravis
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41
Q

How would you assess nutrition

A

History:

  • diet intake
  • nutritional quality of food: red meat, vegetables, protein, dairy products
  • appetite intake
  • dysphagia
  • malabsorptive symptoms
  • comorbidities, chronic illness
  • cognitive and functional affects

Examination:

  • BMI, waist circumference
  • Fe deficiency: pallor, koilonychia, angular stomatitis
  • B12: atrophic glossitis, peripheral neuropathy, pallor
  • Thiamine - dementia, Wernicke, kosakoff
  • Vit D - osteomalacia/osteoporosis
  • folate - pallor
  • Vitamin A: poor eyesight

peripheral neuropathy, osteoporosis, osteomalacia, dementia, visual distrubance

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42
Q

Differentiating JvP

A
  • non pulsatile
  • double flicker
  • moves with respiration (falls with inspiration)
  • moves with position
  • accentuates with hep
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43
Q

If hypermobile joints consider

A

Ethers danlos (bruises and scars)
Marfans
Osteogenesis imperfecta

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44
Q

If VSD murmur and limb abnormalities (wrist) think

A

VACTERL

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45
Q

Types of gaits

A

Gaits

Circumduction
Hemiplegia 
Wide-based
Cerebellar
Hip joint abnormality
Weakness or hypotonia of legs or pelvic girdle
Waddling
Proximal myopathies
Foot drop (CMT)
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46
Q

Signs of chronological c liver disease

A

Signs of chronic liver disease (ABCDEFGHIJ)

Asterixis, Ascites, Ankle oedema, Atrophy of testicles
Bruising
Clubbing/ Colour change of nails (leuconychia)
Dupuytren’s contracture
Encephalopathy / palmar Erythema
Foetor hepaticus
Gynaecomastia
Hepatomegaly
Increase size of parotids
Jaundice
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47
Q

Complications of hereditary haemochromatosis

A

(HaemoChromatosis Can Cause Deposits Anywhere)

Hypogonadism
Cancer (hepatocellular)
Cirrhosis
Cardiomyopathy
Diabetes mellitus
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48
Q

Power grades

A

0/5: no contraction
1/5: muscle flicker, but no movement
2/5: movement possible, but not against gravity (test the joint in its horizontal plane)
3/5: movement possible against gravity, but not against resistance by the examiner
4/5: movement possible against some resistance by the examiner (sometimes this category is subdivided further into 4–/5, 4/5, and 4+/5)
5/5: normal strength

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49
Q

Drooling management

A
SLT to promote oro-motor function
Behavioural intervention
Bibs
Pharmacological options
Hyoscine q6h po
Hyoscine Patch q72/24
Dental intervention
Surgical
Botulium toxin A
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50
Q

complications of CLD….

A

Hepatic VCPBT:

Hepatic encephalopathy
Esophageal varices
Portal hypertension
Ascites
Thrombosis of portal vein
Infection
Carcinoma
Vitamin deficiencies
Coagulopathy
Pruritis 
Bile salt build up
Transplant issues
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51
Q

Creon

A

Pancreatic enzymes amylase protease lipase

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52
Q

Bullying plan

A

1) Patient
→ teaching assertiveness skills and confidence building
2) Parent
• Source of support for child if having problems
• Act as advocate for the child to liase with the school
3) School
• Acknowledge problem exists
• Establish support mechanisms for students (assigned group of staff)
• Anti-bullying policies
• Education in classroom (organise project on bullying
• Not tolerate bullying @ school
• Provision of counsellors or local psychologists

Whole School Approaches / Intervention Programmes:
Kia Kaha, Police Youth Education Services Resource Kit. Contact your local police education officer at the nearest Police Station.
Keeping Ourselves Safe, Police Youth Education Services Resource Kit. Contact your local police education officer at the nearest Police Station.
Eliminating Violence Managing Anger, contact your local Specialist Education Services, Internet: http://www.ses.org.nz

Helplines / Advice Agencies
0800 NO BULLY (0800 66 28 55). An automated phone helpline that offers advice for pupils who are being bullied. Developed and supported by the New Zealand Police and Telecom New Zealand Limited.
The Police / Telecom “Stop Bullying” websitehttp://www.nobully.org.nz offers practical advice and gives some excellent links to other anti-bullying sites.
New Zealand Children Young Persons and Their Families Service (see phone book blue pages Government Phone Listings for local number).
Mental Health Foundation, refer to your local Health Authority.
Youthline, phone 0800 376 633.
• Lifeline: 0800 543 354 (available 24/7)

  • Suicide Crisis Helpline: 0508 828 865 (0508 TAUTOKO) (available 24/7)
  • Youthline: 0800 376 633
  • Kidsline: 0800 543 754 (available 24/7)
  • Whatsup: 0800 942 8787 (Mon-Fri 1pm to 10pm. Sat-Sun 3pm-10pm)
  • Depression helpline: 0800 111 757 (available 24/7)
  • Rainbow Youth: (09) 376 4155
  • Samaritans 0800 726 666
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53
Q

Other primitive reflexes

A
  1. Placing, stepping (both from birth to 6 weeks).
  2. Landau re ex, a two-stage re ex. With the child supported prone (with your
    hand under the abdomen), the child should (normally) extend head, trunk and hips. is is the rst, and more important, stage. Next, ex the head and neck; normally the response is exion of trunk and hips, but this is less constant than the rst stage ( rst stage from 4 months, plus second stage from 9 months; gone by 2 years).
  3. ATNR. With the child supine, the head is rotated to one side. A ‘fencing’ posture develops, with extension of the ipsilateral upper and lower limb (i.e. the side towards which the head is turned) and exion of the opposite side (2–6 months). Persistence beyond 6 months is indicative of upper motor neurone problems, especially CP. Maintaining the ATNR posture throughout the time that the head is held turned, such that the child cannot ‘break’ from that position, is similarly signi cant.
  4. Neck-righting re ex. Rotation of the trunk to conform with the position of the head when the head is rotated to one side (6 months to 2 years).
  5. Moro re ex (birth to 4 months). As with most primitive re exes, persistence beyond the usual time of disappearance is pathological. Make a point of focusing not only on the limb movements but also the facial response, for asymmetry (e.g. in hemiplegic CP).
  6. Parachute re ex. With the infant held in the prone position, move him or her rapidly, face downwards, towards the oor. e normal reaction is to extend both upper limbs as if to break the fall (appears between 6 and 12 months, usually at 9 months, and persists; its absence beyond 12 months is abnormal). Asymmetry occurs with hemiplegia.
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54
Q

Causes of pes cavus

A
CMT
Frederich ataxia (spinocerebellar degeneration)
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55
Q

Floppy weak and floppy strong infant causes

A

Floppy weak: SMA, congenital myotonic dystrophy, congenital myopathy, werdnig-Hoffman

Floppy strong: downs, hypotonic CP

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56
Q

Cause of scoliosis

A

Idiopathic
Paralytic (neuromuscular)
Bony or ligamentous (CT disorders)

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57
Q

Biprosthetic valve types

A

Xenografts - porcine and bovine

Allograft - cadaveric or auto

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58
Q

Causes of cardiomyopathy

A
Congenital
HTN
Infective
Idiopathic
Toxin (chemo)
Infiltration - amyloid, sarcoidosis, metabolic, haemochromatosis
Endocrine Hypothyroidism and acromegaly 
CTD
Ischaemic
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59
Q

Causes of cardiac failure

A
Congenital heart disease 
Valvular
Arrhythmia 
Ischaemic
High output state: anaemia, thyrotoxicosis, Av fistula..
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60
Q

DDx of ejection systolic murmur

A

AS
PS
Hypertrophic cardiomyopathy
ASD

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61
Q

Ascites causes

A

Prehepatic: CHf, Budd-Chiari syndrome, IVC obstruction
Hepatic: decompensated cirrhosis with portal HTN
Nephrotic, TB, chylous, pancreatitis

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62
Q

Atrophic glossitis due to

A

Iron def, B12 and folate def

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63
Q

Can’t smell in what syndrome

A

Kallman

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64
Q

Ptosis causes

A

Congenital, MG, Myotonic dystrophy, thyrotoxicosis, horners, third nerve palsy

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65
Q

Causes of facial n palsy

A

UMN

LMN: bells, OM, acoustic neuroma, meningioma, MS, fracture, Ramsey hunt, parotid enlargement, vascular

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66
Q

Stamping or slapping gait with what

A

Sensory neuropathy

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67
Q

Peripheral neuropathy or nerve stuff DDx

A
Congenital such as CMT
Vascular/vasculitis
Infectious/inflam - GBS/transverse myelitis, syphillis, botulism, Lyme
Trauma
Autoimmune MS
Metabolic - DM, thyroid, uraemia, vit B12/E, B1 and B6 def
Iatrogenic/ idiopathic
Neoplastic - 
A
B
C
Drugs - vincristine, lead
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68
Q

Causes of foot drop

A

CommonPerineal nerve palsy, sciatic nerve palsy, lumbosacral plexus lesion, L4/L5 lesion, PMN, distal myopathy, spinal cord lesion

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69
Q

Genetic syndromes and gene defects assoc obesity

A
Prader-willi 
Alstrom 
Cohen
Albrights 
Carpenter 
Rubinstein-taybi
Fragile x
Lawrence moon bardet biedl
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70
Q

Pain and temperature and proprioception test what

A

Pain and temp is spinothalamic pathway

Proprioception/vibration is dorsal columns

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71
Q

Hypertension causes in NF1 (5)

A

Renal artery stenosis, phaeo, coarct, neurofibromas, essential

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72
Q

Waddling and wide based gait indicates

A

Waddle weakness in proximal myopathy and wide in cerebellar

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73
Q

Romberg sign looks for

A

Dorsal column pathology

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74
Q

Bullying plan 5 steps

A

1: explore and fix cause eg smell or drooling
2: increase self esteem highlight strengths and increase support peers and family home environment and ‘buddy’ or mentor at school and adult he can trust to report to
3: school policy, avoid situations that exacerbate or change schools, karate or other activity to help
4. list some immediate things your child can do when the bullying happens. For example: first, ignore the bullying behaviour. If that doesn’t work, tell the person to stop. If the bullying continues, walk away. Tell someone.
5. Books on bullying or website WHATSUP
Cyber bullying may need restrict websites/internet use, kia kaha police programme safety in schools
6. Regularly check in with child and reassure that telling them is the right thing

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75
Q

Drooling (sialorrhoea) amount per day and causes and complications and management

A

500ml-2L per day
Overproduction or inability to swallow
Complications are social implications and embarrassment of pt and family, skin irritation, frequent bib and clothes change, smell, increased risk aspiration, choking and coughing episodes

Mx:
Saliva clinic SSH
Medications - anticholinergics such as atropine and glycopyrolate
Behavioural modifications and physical therapy (positioning and seating)
Oromotor rehabilitation / therapy by Speech Therapist (increase sucking - straw) and increase oral awareness
Orthodontic treatment
Improvement of nasal and oral airway patency - Otolaryngology (ENT)
Catellio moreles prosthesis
Surgery to the salivary glands - side effect dry mouth and dental problems
Botox injection to submandibular / parotid glands
No treatment but use waterproof bib or clothes

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76
Q

FTt causes

A

Inadequate intake, neglect
Malabsorption
Increased energy use or demands

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77
Q

Oral aversion plan

A

Eliminate cause if can - tubes, reflux, nausea
Educate
Introduce food early age and make it fun, fa,ily meals
Monitor growth
Monitor dentition

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78
Q

Causes of scoliosis

A

SCOLI

Syndrome NF1 marfans
Congenital 
Orthopaedic (leg length)
Low and high tone neuromuscular - CP, Spina bifid a, SMA, DMD
Idiopathic
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79
Q

Sz managment

A
Safety
Emergency plan
Avoid triggers
AED
Assessments
Monitoring (phenytoin and phenobarb) CMZ
Avoid drugs that aggravate 
Surgery
Vagal stimulation
Ketogenic diet
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80
Q

Involuntary movements causes

A

Bells intend to keep moving:
Cerebellar lesions are associated with intention tremor.

Opposite BallS:
Hemiballismus is due to contralateral subthalamic nucleus lesion.

Put at globe:
Putamen / globus pallidus damage causes Atheosis

2 C’s:
Chorea is caused by Caudate nucleus lesion

CP

Drugs

Tics

Infection

Trauma NAI AV malformation

Autoimmune MS

Seizure

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81
Q

Mental state examination

A

Mnemonics for MSE: ASEPTIC

A- Appearance and Behaviour 
S- Speech 
E- Emotion [mood and affect] 
P- Perception [Hallucination and illusion] 
T- Thought content and process 
I- Insight and Judgement 
C- Cognition 

Mnemonics for examination for cognition: GOAL-CRAMP

‘I’d like to start off by asking you a few questions to test your concentration and memory……….’

G- general: Alertness and Co-operation
O- orientation: Time and Place
A- attention: WORLD backwards and Serial Sevens
L- language: Naming and Repetition
C- calculation: Division and Subtraction
R- right Hemisphere Function: Intersecting pentagons and Clock-face
A- abstraction: Proverbs and Similarities
M- memory: Short term and Long-term memory
P- praxis: Wave good-bye and Comb hair

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82
Q

Mouthing toys age

A

6-15/18m

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83
Q

Removes clothes and independent with spoon which age?

A

18m

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84
Q

Prepositions, object use and 6 body parts what age

A

24m

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85
Q

Points to the right image of cat…what age

A

15-18m

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86
Q

Imaginative play at what age

A

3y

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87
Q

Catch well and kick well and jump two feet what age

A

3y

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88
Q

Throw ball what age

A

18m

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89
Q

Tense and big/little opposites at age..

A

4y

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90
Q

Stand on one foot for how many seconds

A

3 at 3 and one second per year of age after that

Can skip at 5y

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91
Q

Handedness by what age

A

18m

Mature pen grip by 5y

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92
Q

Short with polydactyly think

A

Bardt-biedl

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93
Q

Clindodactyly and short think

A

Downs, Russell-silver, seckels, shwachmans

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94
Q

A body habitus that is tall, slim and underweight, with long legs and long arms (i.e., arm span exceeds height by 5 cm or more).

A

Eunuchoid habitus. Definition

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95
Q

Long limbs

A

Dolichostenomelia is a human condition or habitus in which the limbs are unusually long. The name is derived from Ancient Greek (dolichos - long, steno - short, narrow, close, melia - of the limbs). It is a common feature of several kinds of hereditary disorders which affect connective tissue, such as Marfan Syndrome and homocystinuria.

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96
Q

Facial nerve palsy causes

A

Idiopathic Bell’s palsy most common (unknown or viral)
Trauma I.e. due to delivery traumas
AV malformation
Infective -EBV/Ramsay Hunt
Iatrogenic
Tumour/neoplastic (schwannoma)
Congenital: Möbius syndrome, Goldenhar, Poland syndrome, pseudobulbar palsy, Arnold-chiari
NMJ: MG, botulism
Muscular: hereditary myopathies, such as myotonic dystrophy

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97
Q

CXR in cardio patient DDx of increased and decreased pulmonary vascularity

A

Increased:
Truncus, TAPD, TGA, (large L to R shunt, ASD, VSD, PDA)

Decreased:
TOF, Pulmonary atresia, TA, Ebsteins, critical PS

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98
Q

Causes of RAH

A
TA
Hypoplastic R heart
Ebsteins
PA
Complex congenital
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99
Q

Deep Q waves on ECG seen in

A

HCM
TGA
anomalous left coronary artery

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100
Q

LAD causes

A

Endocardium cushion defect
TA
HCM
Inlet VSD

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101
Q

RAH causes

A
Ebsteins
TA
PA with intact septum
Truncus
TOF large VSD
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102
Q

Short forth metacarpal in

A

Turners, fetal alcohol, pseudopseudohypoparathyroidism

Trauma, infection

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103
Q

Micropenis causes

A

Kallman s and hypopit

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104
Q

Froehlich syndrome is

A

a rarely encountered condition that occurs mainly in males and is characterized by obesity, small testes, and a delay in the onset of puberty.

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105
Q

Bronchiectasis causes

A

bx causes
There are multiple causes including - CIRICCI
Cystic fibrosis
Infection with pertussis, measles, TB, adenovirus (7 &14)
Recurrent infections or aspirations
Less commonly
Immune deficiency
Ciliary dyskinesia
Congenital abnormality of CT (→ obstruction →↑ infections)
If localised areas of bronchiectasis
Inhalation of foreign body

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106
Q

Qualities of apex beat and causes

A
Quality of apex beat
Quality
Sustained – pressure overload in AS
Forceful – LVH
Thrusting – volume overload (MR/AR) or L→R shunt
Parasternal heave – RVH

Palpable S2 = pulmonary hypertension

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107
Q

Causes of an abdominal mass

A
Appendiceal Abscess
Tumors
Wilms
Neuroblastoma Hepatoblastoma
HCC
Teratoma
Lymphoma
Ovarian Cysts
Renal
Hydronephrosis
Cystic disease
Obstructed bladder
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108
Q

Short child syndrome characterized by a progressive loss of vision and hearing, a form of heart disease that enlarges and weakens the heart muscle (dilated cardiomyopathy), obesity, type 2 diabetes mellitus (the most common form of diabetes), and short stature.

A

Alström syndrome

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109
Q

Bardet-Biedl syndrome (BBS) is an autosomal recessive multisystemic genetic disorder characterised by six major defects which are…

A

Ciliopathy

obesity, learning disability, renal anomalies, polydactyly, retinal dystrophy and hypogenitalism

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110
Q

Pickwickian syndrome: 4 features

A

The combination of obesity, somnolence (sleepiness), hypoventilation (underbreathing), and plethoric (red) face.

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111
Q

Features of coffin lowrey syndrome

A

Severe ID
short stature, an unusually small head (microcephaly), progressive abnormal curvature of the spine (kyphoscoliosis), and other skeletal abnormalities.

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112
Q

Abnormal RAPD in affected eye less constriction both pupils - name and causes

A

Marcus Gunn pupil

Causes by optic nerve lesion, severe retinal disease, optic neuritis

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113
Q

What is an Argyll Robertson Pupil

A

It is bilateral small pupils that reduce in size on a near object (i.e., they accommodate), but do not constrict when exposed to bright light (i.e., they do not react to light). They are a highly specific sign of neurosyphilis; however, Argyll Robertson pupils may also be a sign of diabetic neuropathy. In general, pupils that accommodate but do not react are said to show light-near dissociation (i.e., it is the absence of a miotic reaction to light, both direct and consensual, with the preservation of a miotic reaction to near stimulus (accommodation/convergence).[1]

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114
Q

Beak nose and pancreatic insufficiency

A

Johnston-Blizzard syndrome

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115
Q

Ortolani and Barlow test

A

Ortolani opens up lower limbs and Barlow bumps back

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116
Q

Erbs palsy roots

A

C5,6

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117
Q

What is galant reflex used for?

A

To look at structural integrity T2 to S1

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118
Q

What conditions cause leukocoria

A

Many conditions cause leukocoria including cataract, retinal detachment, retinopathy of prematurity, retinal malformation, coats disease, intraocular infection endophthalmitis), retinal vascular abnormality, and intraocular tumor (retinoblastoma).

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119
Q

What is pellagra

A

Pellagra is a disease characterised by diarrhoea, dermatitis (scaly brown) and dementia. If left untreated, death is the usual outcome. It occurs as a result of niacin (vitamin B-3) deficiency. Niacin is required for most cellular processes

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120
Q

Brown nails can indicate?

A

Chronic Kidney Disease

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121
Q

Bleomycin important side effects

A

Interstitial pneumonitis leading to Lung fibrosis from oxidative damage

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122
Q

Bulbar palsy weather is it and causes?

A

Bulbar palsy results from bilateral impairment of function of the IXth, Xth and XIIth cranial nerves. This gives rise to dysarthria, dysphagia (often with choking episodes and nasal regurgitation of fluids), dysphonia and poor cough, and susceptibility to aspiration pneumonia. The lowermost part of VII may, infrequently, be involved.

The disturbance is of the motor nuclei rather than of the corticobulbar tracts. It is distinguished from pseudobulbar palsy by the presence of lower motor neurone signs. Autonomic features are uncommon.

Causes of bulbar palsy include:
MND
Guillain Barre syndrome
poliomyelitis
diphtheria
myasthenia gravis
neurosyphilis
Tumour
Stroke
Syndromic or genetic
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123
Q

Anti epileptic med’s that can measure serum levels

A

Carbamazepine
Phenytoin
Phenobarbitone

Not valproate (unless suspect non compliance)

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124
Q

90% of Spina bifida have…

A

Hydrocephalus

Arnold chiari II

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125
Q

Scoliosis management

A

Monitor X-ray watch and see
Brace or cast
Surgery

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126
Q

Lens displacement in marfans and homocysteinuria

A

Marfans superior intellect so superior

Homocysteinuria inferior

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127
Q

Optic nerve hypoplasia association

A

Absent corpus callosum

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128
Q

Noonan complications

A
Noonan syndrome have an eightfold increased risk of developing leukemia or other cancers over age-matched peers.
Bleeding tendency
Pubertal delay
Short
AD
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129
Q

Denys-Drash

A

Denys–Drash syndrome (DDS) or Drash syndrome is a rare disorder or syndrome characterized by gonadal dysgenesis, nephropathy, and Wilms’ tumor.

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130
Q

Impairment, disability and handicap definition

A

Impairment – residual limitation resulting from disease, injury or congenital defect
Disability – Difficulties an individual may have in executing activities – inability to perform a functional skill
Handicap – Problems an individual may experience in life situations – interaction of a disability with the environment

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131
Q

Cyanosis implies?

A

Right to left shunt at level of heart or great vessels

Inadequate oxygenation by kings

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132
Q

Left lateral chest wall scar causes?

A
Coarctation repair
PDA ligation
BT shunt
PA banding
Thoracotamy
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133
Q

Absent or reduced brachial pulse causes

A
Congenital
Embolisation
Previous catheter
Cervical rib
BT shunt

Absent left could be from coarctation repair

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134
Q

Flat foot pes planus causes

A

Dyspraxia, ligamentous laxity, hypermobility (collagen disorder ethers-danlos and marfan), CP

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135
Q

Causes of pes cavus

A
CMT
Fredereichs ataxia
Neurological 
Trauma
Orthopaedic 
Neuromuscular
Spina bifida
Diastometamyelia
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136
Q

Jerky pulse and collapsing pulse causes

A

HOCM

AI and PDA

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137
Q

Mean diastolic and systolic values

A

Age + 90/ age + 55

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138
Q

Causes of displaced apex

A

Cardiomegaly
Scoliosis
Pectus

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139
Q

Loud second heart sound from?

A

Pulmonary HTN

Increased pulmonary flow from left to right shunt in ASD, VSD, PDA

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140
Q

Murmur description in presentation

A

X is a child with cyanotic/acyanotic repaired/unrepaired congenital/acquired heart disease

There was a murmur:
Timing and duration
Intensity
Pitch and quality

Loudest
Changes with position
Radiated to

Second heart sound

No/ was Evidence of failure

Differential diagnosis and investigations

141
Q

Radiation of murmur to back in

A

Coarctation, Ps, PDA

142
Q

Complications in cystic fibrosis

A

Head to toe approach

Psychological
Nasal polyps
Respiratory infections and bronchiectasis
Pancreatic failure and diabetes
Bowel obstruction
Liver (focal biliary)
Reproductive fertility issues
Pubertal delay
Bone health (osteoporosis)
Electrolyte imbalance
143
Q

Macroglossia is causes

A
Hypothyroid
BWS
Mucopolysaccharoidoses
Pompe
Amyloid
Downs is not true
144
Q

Causes of visual impairment in children

A
Trauma to eye
Corneal clouding (multiple causes)
Glaucoma (assoc sturge weber)
Uveitis
Cataracts/lens dislocation
ROP
Retinoblastoma 
Retinitis pigmentosa
Cherry red spots from metabolic disease
Optic nerve neuritis or atrophy (Cp/septoopticdysplasia/tumour)
Optic nerve pathology (such as glioma)
Cerebral causes
145
Q

Leg length discrepancy causes

ASIS to medical malleolus

A
DDH
Trauma or surgery
Arthritis
Hemiparesis
Osteogenesis imperfecta 
Henihypertrophy
polyostotic fibrous dysplasia in MAS
146
Q

Conditions associated with cafe au lait spots

A
NF
TS
Fanconi
McCune Albright
Ataxia telangiectasia 
RUssle silver
Blooms
Gaucheries
Chediak-Higashi
147
Q

Bitemporal hemianopia due to

A

Optic chasm compression

148
Q

Causes of a nonreactive pupil to light

A
Third nerve palsy
Cataracts corneal opacitoes, vitreous and retinal haemorrhage or detachment
Infection iris and ciliary muscles
Trauma
Optic nerve pathology
149
Q

Causes of CN 5 lesion

A

Peripheral from trauma, tumour, infection (shingles)

Central cavernous sinus lesions, tumour, bulbar palsy, pseudobulbar palsy

150
Q

Nystagmus direction in vestibular and cerebellar causes

A

Vestibular away from lesion
Cerebellar towards side of lesion
(Fast phase)

151
Q

Spinal roots and reflexes

A

Triceps C7,8
Supination C5/6
Biceps C5/6

Knee L3/4
Ankle S1/2

152
Q

Causes of proprioception loss

A
Peripheral neuropathy
Vincristine
GBS
Fredeichs ataxia
CMT
Hereditary neuropathy
153
Q

Holt Oram syndrome characterised by these two things

A

Upper limb skeletal abnormalities and heart problem

154
Q

Mono brow also called

A

Synophrys

155
Q

Short fingers and toes called

A

Brachydactyly

156
Q

Lesion CN 9 & 10 suggests

A

Bulbar palsy (MND)

157
Q

LEOPARD stands for

A

L – lentigines (multiple brown-black spots on the skin)
E – electrocardiographic (ECG) conduction defects
O – ocular hypertelorism
P – pulmonary stenosis
A – abnormalities of genitals
R – retarded growth resulting in short stature
D – deafness or hearing loss due to inner ear malfunction

158
Q

Atlanto-axial subluxation causes

A
congenital
os odontoideum
Down syndrome (20%)
Morquio syndrome
spondyloepiphyseal dysplasia
osteogenesis imperfecta
Marfan disease
neurofibromatosis type 1 (NF1)
arthritides
rheumatoid arthritis
psoriatic arthritis
Reiter syndrome (reactive arthritis)
ankylosing spondylitis
systemic lupus erythematosus (SLE)
acquired
trauma
retropharyngeal abscess / Grisel syndrome
surgery
upper respiratory tract infection
159
Q

Kearns-Sayre syndrome triad

A

Kearns-Sayre syndrome triad includes onset in persons younger than 20 years, chronic, progressive, external ophthalmoplegia, pigmentary degeneration of the retina and cardiac problems

160
Q

Surgically-created cardiac shunts (mnemonic)

A

Mnemonic
Great flow really would be perfect
G: Glenn shunt
superior vena cava to right pulmonary artery
F: Fontan procedure
inferior vena cava or right atrium to right pulmonary artery
R: Rastelli procedure
right ventricle to main pulmonary artery
W: Waterston shunt
ascending aorta to right pulmonary artery
B: Blalock-Taussig shunt
subclavian artery to pulmonary artery
P: Pott shunt
descending thoracic aorta to left pulmonary artery

161
Q

Pleural effusion causes

A
Exudate:
CT diseases, pneumonia, TB, drugs and radiation
Transudate:
Low albumin
Low thyroid
Low cardiac output in failure
162
Q

Bronchiectasis causes

A

Congenital and acquired

Congenital: PCD, CF, immune deficiency
Acquired: infective, FB, TB

163
Q

Glossitis cause by

A

B vitamin deficiencies esp B12
Folate
Iron

Geographical tongue by def B vitamins

164
Q

Systemic signs of IBD and complications

A
Ocular changes/signs of anaemia
Clubbing
CLD - cirrhosis/PSC
Gallstones and renal stones
Arthritis
Skin manifestations

Complications: of treatment, mega colon, bleed or perforation, cancer

165
Q

Ascites causes

A
Cirrhosis
Veno oclusive disease
CHF
Constrictive pericarditis
TB
Pancreatic
Nephrotic syndrome
166
Q

Causes of abdominal mass

A
Important not to miss:
Cancer
Abscess
AAA
BO

Common:
Constipation
Bladder enlargement
Organomegaly

Other:
Transplanted kidney
Gastric mass
Ovarian cyst
Hernia
Uterus causes

B

167
Q

Causes of fasiculations

A
MND (SMA)
Motor root compression
Peripheral neuropathy
Primary myopathy
Thyrotoxicosis
168
Q

Palmar drift causes

A

Cerebellar
Proprioception
UMN pyrimidial weaknss

169
Q

Causes of foot drop

A
Common peroneal nerve palsy
CMT
L4/L5 lesion
Stroke
Distal myopathy
MS
Muscular dystrophy
Peripheral motor neuropathy
170
Q

Waddling gait causes

A

A waddling gait is the style of walking that is seen in a patient with proximal myopathy.

It is characterised by:

a broad-based gait with a duck-like waddle to the swing phase
the pelvis drops to the side of the leg being raised
forward curvature of the lumbar spine
marked body swing
This gait may also be seen in patients with congenital hip dislocation and pregnancy.

171
Q

US/LS ratio

A

1.7 at birth
1 by age 10
0.8 by puberty

172
Q

Scoliosis management

A

Observe
Conservative - physiology and exercises, chair adjusted, bracing
Surgical

173
Q

Management points for diabetes

A

Assess current control with reviewing investigations and BSL record - may need to alter insulin regime
Survellience for complications
Ongoing education everyone involved including safety with alcohol, avoid smoking
Diet and exercise discussed
Sick day management, hypoglycaemia plan and travel plan (letters, contacts,supplies)
Medic alert
Health optimisation with vaccinations

Psychosocial support with diabetes youth groups diabetesyouth.org.nz
Diabetes NZ

Transition plan

174
Q

Complications of diabetes screening plan

A

Retinopathy and diabetic nephropathy [urine sample, ophthalm] yearly from 5 years after diagnosis or from 10 years or 2 years after diagnosis in adolescent
BP yearly

Lipids checked 5 yearly or from 12 years or annually after puberty

Yearly FT and coeliac screen for 5 years then 2 yearly

Neuropathy, joint and skin problems, CV risk

175
Q

DMD management

A

Medical treatment with steroids

Survellience and management of complications:
- mood and behaviour
- Rarely have cognitive impairment - wisckott score to identify areas requiring assistance and strengths, individualised learning plan for appropriate assistance with educational and physical needs
- Cardiomyopathy Echocardiogram treat failure
- respiratory nocturnal failure/polysomnography - bipap
- Contractures and scoliosis - passive and active exercises physiotherapy assisted programmes
OT assisted supports and equipments and review suitability of home environment
Orthotics and surgery
- Nutrition with exercise and diet advice

Other:

  • end of life and palliative (life limiting illness) support from MDA for coping grief and loss
  • Steroid side effects
  • Avoid smoke and treat infections aggressively
  • avoid patronising and overprotective
  • workbridge can assist with vocation planning
  • parent project muscular dystrophy
  • Nzord.org.nz
176
Q

Care of sibling

A
Alleviate feelings of fear or guilt
Spend quality time and attention one on one
Include in family decision making
Relieve care burden
Give meaningful responsibilities in family
Honesty
Compassion
Respect
177
Q

Five steps to help protect your child online

A
  1. Keep it private
    Ensure your child keeps their profile and online friends private. Most social networking sites and other technologies (such as chat rooms) have both private and open/public settings. They should also avoid divulging personal information, including identifying photographs, to anyone they don’t already know.
    Ensure they understand that once posted/uploaded, content is non-retractable and will be part of their permanent record online. Someone, somewhere will always find it.
  2. Keep it friendly
    Cyber-bullying is poised to turn into the biggest online concern, already affecting up to 35% of all children*. Ensure your child knows what to do when they encounter cyber-bullying (Stop, Block and Tell!), and who to report it to - make sure you are on their list!
    ‘Think before you send’ is a great online rule for your child to live by. Ask them to think about the potential consequences to themselves and others of every video uploaded, every comment, every text, every email.
  3. Keep it online
    Ensure your child only ever meets online friends for the first time in the company of yourself or a trusted adult. Ensure your child never, ever organises to meet an online friend face-to-face for the first time on their own - no exceptions.
  4. Keep it locked
    Make sure your child’s mobile phone uses a PIN, so when lost or stolen it can’t be used to auto-sign into their online profiles (auto-sign-on is where a website auto-remembers a password). If in doubt, ask to set their Bluetooth-enabled phone to ‘undiscoverable’ - otherwise strangers can potentially access your children’s phone and its data.
  5. Keep it real
    Children find the internet an easier place to explore their identity, to challenge adult norms and boundaries, experiment with relationships and practice a range of behaviours. But many of the consequences of these things are amplified online.
    Reinforce strong and positive personal and societal values and behaviours online. Help build resilience in your child to what they will undoubtedly come across on the internet: inappropriate content and cyber-bullying. Make sure they know how to handle themselves online.
178
Q

In truncus or TOF or small thymus think…

A

22q11

179
Q

For difficult areas of management always mention these three things…

A
  1. Active partnership
  2. Highlight strengths and positive family experiences
  3. Review methods of coping

Enlist individual support fm friends, family, clergy, community
Offer information and websites to connect with other families and support groups

180
Q

Management of sickle cell

A

Medical:
Education and plan including written materials
Health maintenance: Vaccinations and penicillin prophylaxis
Blood transfusions
Hydroxyurea to increase HbF
Stem cell

Psychosocial

Follow up

Complications screen:
Infection, infarction, splenic sequestration, aplastic crisis, priapism, haematuria, anaemia, jaundice, splenomegaly, carsiomegaly, enuresis, cholelithiasis, delay growth and puberty, restrictive lung, pulm HTN, avasular necrosis, proliferators retinopathy, leg ulcers, transfusion iron overload

181
Q

How to approach any management issue?

Management medical and psychosocial (highlight strengths)

A

CAST ICE

Confirm
Assess
Set treatment goals
Treat
Include others in MDT
Prevention and survellience of complications/triggers
Ensure follow up
182
Q

Management goals

A

Culturally appropriate family centred care working in partnership as the advocate and health coordinator to maximise health function, development, and family functioning

183
Q

Short stature causes

A

Normal variant

  • constitutional delay
  • familial

Disproportionate

  • skeletal dysplasias
  • metabolic Bone disease
  • radiation

Proportional congenital or acquired
- chromosomal, syndrome (Noonan, Russell silver, PWS), adverse psychosocial, drugs, malnutrition, chronic disease, endocrine such as GH def, hypothyroidism, Cushings

184
Q

Short stature management

A

Confirm information with history including family history and psychosocial screen, perinatal hx, feeding and growth

Investigate
FBC, inflammatory markers, electrolytes urea and creatinine, coeliac screen, Arafat, karyotupe, bone age
IGF-1, IGF-BP3
GH provocation test
Neuroimaging 

Manage cause and ensure adequate nutrition and induce puberty or use GH (leg lengthening)

Follow up with monitoring growth in 6 months and
Establishing growth velocity

185
Q

Causes of cafe au lai

A
neurofibromatosis type 1 and 2
McCune Albright syndrome
ataxia telangiectasia
fanconi anemia
Russell-silver syndrome
Tuberous sclerosis
Gaucher disease
Chediak – Higashi syndrome
Turner syndrome
Proteus syndrome
Legius syndrome
LEOPARD
Bloom syndrome
186
Q

Clubbing causes

A
CLUBBING 
Mostly cardio/respiratory:
Congenital Cyanotic heart disease or reversal L to R shunt or eisenmenger complex/atrial myxoma 
Lung:
Abscess
Bronchiectasis
CF
(Don't say asthma)
Empyema
Fibrosis

UC/CD (IBD)
Biliary cirrhosis usually or other cirrhosis cause or chronic hepatitis
Birth defects/familial
IE
Neoplasm
GI malabsorption (coeliac)
Other: thyroid acropatchy/TB/sickle cell/pulm AV malformation

187
Q

Hypotonic baby causes and management

A

Distribution and course of disease (degeneration or static)

Investigations: Rule out sepsis and electrolyte imbalance (mg and ca), left, urine drug screen, TFT, TORCH, EEG prognosis, CK, MRI, metabolic tests

Causes: central (cerebral due to HIE, haemorrhage, malformation, chromosomal, syndrome (Lowe), peroxisomal - zellweger and adrenal leukodystophies, metabolic like pompe, drug) and peripheral causes (including muscle myopathies and dystrophies, neuromuscular junction (botulism MG), peripheral n (CMT) anterior horn cell (SMA))

Management:
Monitor contracture/hip dislocation
Swallow/feeding safety (oro-motor dysfunction)
Reflux
Respiration and overnight oximetry 
Syndromes associations
188
Q

Complications of childhood obesity

A

CV: High lipids, HTN, LVH, Risk heart disease stroke
Respiratory: sleep disordered breathing, Sleep apnoea, asthma
Renal
GIT: Non alcoholic fatty liver disease, Gallstones, GORD
Orthopaedic: SUFE, Risk degenerative joint disease
Haem: Thrombus
Surgical: Hernia
Endocrine: dm2, Precocious puberty, PCOS
Neuropsych: Pseudotumor cerebri, psychosocial, negatively affects school performance

189
Q

Causes of hepatomegaly

A
CHIMPS
CT d/o
Haematological
Infective and infiltrative
Metabolic
Parenchymal (cardiac fluid overload)
Systemic
190
Q

Clinical signs of chronic liver disease

A
Don't forget hep b at the FLAP JAP SEX CLUB
Hepatic flap
Jaundice
Ascites/atrophy testicles with gunaecomastia/anaemia
Palmar erythema
Spider naevi
Encephalopathy
Xanthelasma
Clubbing/colour change nails
191
Q

Hurlers syndrome

A

HURLERS a mucopolysaccharoidosis (lysosomal disorder)

HSM
Unusual facial features
Recessive
Liduronidase def
Eyes clouded
Retardation 
Short stubby fingers
192
Q

Marfans

A
MVP
Aortic aneurysm
Retinal detachment
Fibrillin gene on chromosome 15
Arachnodactyly
PNeumothraces
Skeletal abn
193
Q

Causes of obesity

A

Overeating and under activity

Drugs

Cushing

Genetic such as prader willi

194
Q

Steroid side effects

A

CUSHINGOID MAP
Cataracts/ Cushing moon face and buffalo hump (interscspjlar adiposity)
Ulcers - ulcers and duodenal
Skin striae, thin, bruise
HTN hirsutism hyperglycaemia heart failure
InfectionsImmunosuppressive with poor wound healing
Necrosis (avascular necrosis femoral head)
Glycosuria/growth (short)
Osteoporosis/obesity
ICP pseudotumour cerebri
Diabetes/ glucose intolerance

Myopathy (proximal) mood
Acne fat/ adrenal (suppressed hypothesis-pit-axis)
Pancreatitis psychosis/behavioural

195
Q

Macrocephaly causes

A

Megalencephaly (large brain)
Hydrocephalus - communicating and noncommunicating choroid plexus papilloma increases production
Hyperostosis (bone overgrowth): bossing - osteogenesis imperfecta, chronic haemolytic anaemia, rickets, achondroplasia
Familial
Sturg-Weber/NF1/TS/sotos/weaver/Noonan/Costello/gorlin
Fragile x, leukodystrophies, metabolic
Tumour
abscess
NAI
AV malformation great vein of Galen (bruit temporal areas..)

(Autism kids 25% have this)

196
Q

Causes of microcephaly

A

Genetic: trisomies: downs, Edward, patau, deletions 4p (wolf-hirschhorn) and 5p (cri-du-chat), 22q11 and 7q11.23 (Williams), smith-Leslie-opitz, Cornelia de lange, rett, Nijmegen breakage, ataxia telangiectasia

Stroke/trauma/cerebral injury

TORCH zika
Fetal hydantoin or alcohol syndrome
Perinatal insult

Inborn error of metabolism

Other: lead, chronic renal failure, anaemia, hypothyroid, congenital heart disease, malnutrition

Rett syndrome decelerated growth acquired microcephaly

197
Q

Causes of short stature

A

Familial
Constitutional delay
Idiopathic
Endocrine - hypopit, GH def, hypothyroid, pSeudohypoparathyroidism, CAH, Cushing

Chronic disease - anaemia, endocrine: hypothyroid, GIT: coeliac/IBD, renal and liver
GH deficiency (order IGF-1)

Syndrome - Genetic such as turners syndrome, others like downs syndromes
Skeletal dysplasia - Chondrodystrophy (achondroplasia)
Scoliosis
Side effects of Drugs - chronic steroid use, ADHD meds, anticonvulsants, radiotherapy
IUGR
Nutritional deficiency
Schwachman-diamond syndrome
Klippel-Fiel
Seckel syndrome

198
Q

Tall stature causes

A
Familial or normal variant obesity
Klinefleter
Marfan
Precocious puberty
Kallman 
Proteus syndrome
MEN2B
NF1
Fragile x
Sotos 
Weaver
BWS
Homocysteinuria 
Hyperthyroidism
GH excess, acromegaly
McCune Albright
Hyperthyroidism
199
Q

Mid parental height formula

A

Father + mother +/- 13cm

Divided by 2 (+/- 10cm)

200
Q

Three extra primitive reflexes

A

Galant
Swimming
Parachute

201
Q

Jaundice causes

A

Haemolysis (G6PD)
Haemoglobinopathies
Infection/inflammation
Reduced clearance: Gilbert’s, crigler-najjar, hypothyroid
Biliary atresia/allagile/choledochal cyst
Metabolic (galactosemia and tyrosinaemia)
Sepsis
TPN related

202
Q

Splenomegaly causes

A

Hyperplasia: due to haemolysis
Infiltrative: such as amyloid or sarcoidosis
Congestive: (portal HTN from cirrhosis)
Neoplastic: such as ALL/lymphoma
Storage:such as gaucher hurler Neiman pick
Infections: (EBV CMV malaria)
Autoimmune

203
Q

ADHD management extra information gathered by

A

Connors scale
Teacher report
Educational psychology assessment

204
Q

Complications haemochromatosis

A

Haemochromatosis can cause deposits anywhere

Hypogonadism 
Cancer
Cirrhosis
Cardiomyopathy 
DM
Arthropathy
205
Q

Down’s syndrome important conditions

A

Cardiovascular: CHD especially endocaridal cushion defects/AVSD
Respiratory: sleep disordered breathing
Gastrointestinal: hirsprungs, atresia, coeliac, constipation
Endocrine: Thyroid dysfunction, obsess the risk
Genitourinary:
Musculoskeletal: Hip abnormalities, Atlanto-axial instability, scoliosis
Neurological: seizures, psychiatric such as depression, Alzheimer’s after 35y, abuse
Immune-Haematological: Leukaemias/ immune dysfunction, iron deficiency
Dermatological
Eye disease: cataracts and refractive errors
Hearing/ENT: serous otitis media, hearing impairment

206
Q

Causes of intellectual disability

A

Prenatal: chromosomal - trisomy, fragile x, 22q11 deletion, genetic - TS, metabolic, syndromic - Williams, Prader-Willi, Cornelia de Lange, infections TORCH, toxins and drugs and major structural abnormalities
Perinatal: HIE, stroke, trauma, infection, hypoglycaemia
Post-natal: Injury, infection, poisoning

207
Q

Grading murmur

A

1-6
4 = thrill
5 = heard with stethoscope just on skin
6 = heard without stethoscope

208
Q

Continuous murmur differential

A
PDA
Operative Shunt murmur
AV fistula
Ruptured sinus of valsava 
Severe coarctation
Left coronary artery origin from pulmonary artery anomaly
Anomalies of origin of the pulmonary artery
Truncus 
  • aortic-pulmonary communications
  • arteriovenous communications (fistulas)
  • changes in arterial flows
  • changes in veins flows
209
Q

Holosystolic murmur causes and continuous murmur causes

A

VSD
MR
TR

Continuous murmur:
PDA
Venous hum
CoA
Surgical shunt such as BT
AV fistulae
210
Q

The four causes of a diastolic murmur

A

AR
PR
TS
MS

211
Q

Flu vaccine funded for?

A
  • asthma and regularly use a preventer medication (brown, orange, red or purple inhaler)?
  • under 5 years and has a significant respiratory illness or been in hospital for a respiratory illness (such as pneumonia, bronchiolitis, asthma)?

does your child have a heart condition (such as congenital heart disease or rheumatic heart disease)?

does your child have cancer?

does your child have an ongoing chest (respiratory) condition, such as bronchiectasis or cystic fibrosis?

does your child have diabetes?

does your child have ongoing kidney (renal) disease?

does your child have HIV or AIDS, an auto-immune disease or another immune deficiency?

Flu immunisation is also recommended for those sharing a house with children and young people with long term (chronic) medical conditions. The flu spreads rapidly within households and children are particularly efficient spreaders. It may not be free for household members - they could ask their employer about free or subsidised flu immunisation as many employers offer this to their employees. Flu immunisation is free for some adults; for example, all those over 65 years of age and those with certain long term conditions.

Flu immunisation is recommended and free to pregnant women

Flu immunisation offers protection in pregnancy. Flu is likely to be more severe in pregnancy and can affect the mother and the unborn baby. Immunisation also offers some protection to the newborn baby.

212
Q

Causes of ptosis

A
  1. Neurogenic:
    - third nerve palsy,
    - horners syndrome
  2. Myogenic:
    - myasthenia gravis,
    - myopathy,
    - myotonic dystrophy,
    - congenital
  3. Mechanical
    - trauma
    - eyelid oedema
    - inflammation of eyelid
    - neoplastic
  4. Neurotoxic:
    - envenomation
    - chronic opioid abuse
213
Q

Management approach to obesity

A

Causes

  • Lifestyle (sedentery, diet)
  • metabolic: Cushings, hypothyroidism
  • inherited
  • drugs: antipsychotics, antiepileptics, HAART

Consequences:

  • Accelerated atherosclerosis
  • Dyslipidaemia
  • diabetes
  • hypertension
  • sleep disordered breathing
  • restrictive ventilation defects
  • fatty liver
  • FSGS
  • Osteoarthritis
  • Psychological

Management

  • lifestyle factors initially (evidence for short term LOW)
  • Diet: low fat, low calories< 1500kcal, low salt<2g
  • exercise: mod workload for 30mins most days of the week
  • involve dietitian, family, exercise trainer
  • Review regularly, aiming weight loss of 10-15%
  • Pharmacological therapy (limited benefit): orlistat (fat binder, Cx GIT/steatorrhoea), fentomene (appetite suppressant Cx cardiomyopathy), topiramate (off label), metformin (if have insulin resistance)
  • bariatric surgery (evidence for long term weight loss): lap banding, sleeve gastrectomy, gastric bypass
214
Q

Causes of myopathy

A
  • Hereditary muscular dystrophy
  • Congenital myopathies - rare
  • Acquired: PACE, PODS
  • —polymyositis, dermatomyositis
  • –alcohol
  • –Carcinoma
  • –Endocrine (hypothyroid, hyperthyroid, Cushings, Acromegaly, hypopituitarism)
  • –Periodic paralysis
  • –Osteomalacia
  • –Drugs (chloroquine, steroids, statins, fibrates)
  • –Sarcoid
  • –Myasthenia Gravis
215
Q

How would you assess nutrition

A

History:

  • diet intake
  • nutritional quality of food: red meat, vegetables, protein, dairy products
  • appetite intake
  • dysphagia
  • malabsorptive symptoms
  • comorbidities, chronic illness
  • cognitive and functional affects

Examination:

  • BMI, waist circumference
  • Fe deficiency: pallor, koilonychia, angular stomatitis
  • B12: atrophic glossitis, peripheral neuropathy, pallor
  • Thiamine - dementia, Wernicke, kosakoff
  • Vit D - osteomalacia/osteoporosis
  • folate - pallor
  • Vitamin A: poor eyesight

peripheral neuropathy, osteoporosis, osteomalacia, dementia, visual distrubance

216
Q

Differentiating JvP

A
  • non pulsatile
  • double flicker
  • moves with respiration (falls with inspiration)
  • moves with position
  • accentuates with hep
217
Q

If hypermobile joints consider

A

Ethers danlos (bruises and scars)
Marfans
Osteogenesis imperfecta

218
Q

If VSD murmur and limb abnormalities (wrist) think

A

VACTERL

219
Q

Types of gaits

A

Gaits

Circumduction
Hemiplegia 
Wide-based
Cerebellar
Hip joint abnormality
Weakness or hypotonia of legs or pelvic girdle
Waddling
Proximal myopathies
Foot drop (CMT)
220
Q

Signs of chronological c liver disease

A

Signs of chronic liver disease (ABCDEFGHIJ)

Asterixis, Ascites, Ankle oedema, Atrophy of testicles
Bruising
Clubbing/ Colour change of nails (leuconychia)
Dupuytren’s contracture
Encephalopathy / palmar Erythema
Foetor hepaticus
Gynaecomastia
Hepatomegaly
Increase size of parotids
Jaundice
221
Q

Complications of hereditary haemochromatosis

A

(HaemoChromatosis Can Cause Deposits Anywhere)

Hypogonadism
Cancer (hepatocellular)
Cirrhosis
Cardiomyopathy
Diabetes mellitus
222
Q

Reflexes and nerve roots

A
Biceps stretch reflex (C5) 
Brachioradialis reflex (C6) 
Triceps stretch reflex (C8) 
Patellar reflex or knee-jerk reflex (L3) 
Achilles reflex (S1) 
Plantar reflex or Babinski reflex (S1)
223
Q

Power grades

A

0/5: no contraction
1/5: muscle flicker, but no movement
2/5: movement possible, but not against gravity (test the joint in its horizontal plane)
3/5: movement possible against gravity, but not against resistance by the examiner
4/5: movement possible against some resistance by the examiner (sometimes this category is subdivided further into 4–/5, 4/5, and 4+/5)
5/5: normal strength

224
Q

Drooling management

A
SLT to promote oro-motor function
Behavioural intervention
Bibs
Pharmacological options
Hyoscine q6h po
Hyoscine Patch q72/24
Dental intervention
Surgical
Botulium toxin A
225
Q

complications of CLD….

A

Hepatic VCPBT:

Hepatic encephalopathy
Esophageal varices
Portal hypertension
Ascites
Thrombosis of portal vein
Infection
Carcinoma
Vitamin deficiencies
Coagulopathy
Pruritis 
Bile salt build up
Transplant issues
226
Q

Creon

A

Pancreatic enzymes amylase protease lipase

227
Q

Bullying plan

A

1) Patient
→ teaching assertiveness skills and confidence building
2) Parent
• Source of support for child if having problems
• Act as advocate for the child to liase with the school
3) School
• Acknowledge problem exists
• Establish support mechanisms for students (assigned group of staff)
• Anti-bullying policies
• Education in classroom (organise project on bullying
• Not tolerate bullying @ school
• Provision of counsellors or local psychologists

Whole School Approaches / Intervention Programmes:
Kia Kaha, Police Youth Education Services Resource Kit. Contact your local police education officer at the nearest Police Station.
Keeping Ourselves Safe, Police Youth Education Services Resource Kit. Contact your local police education officer at the nearest Police Station.
Eliminating Violence Managing Anger, contact your local Specialist Education Services, Internet: http://www.ses.org.nz

Helplines / Advice Agencies
0800 NO BULLY (0800 66 28 55). An automated phone helpline that offers advice for pupils who are being bullied. Developed and supported by the New Zealand Police and Telecom New Zealand Limited.
The Police / Telecom “Stop Bullying” websitehttp://www.nobully.org.nz offers practical advice and gives some excellent links to other anti-bullying sites.
New Zealand Children Young Persons and Their Families Service (see phone book blue pages Government Phone Listings for local number).
Mental Health Foundation, refer to your local Health Authority.
Youthline, phone 0800 376 633.
• Lifeline: 0800 543 354 (available 24/7)

  • Suicide Crisis Helpline: 0508 828 865 (0508 TAUTOKO) (available 24/7)
  • Youthline: 0800 376 633
  • Kidsline: 0800 543 754 (available 24/7)
  • Whatsup: 0800 942 8787 (Mon-Fri 1pm to 10pm. Sat-Sun 3pm-10pm)
  • Depression helpline: 0800 111 757 (available 24/7)
  • Rainbow Youth: (09) 376 4155
  • Samaritans 0800 726 666
228
Q

Other primitive reflexes

A
  1. Placing, stepping (both from birth to 6 weeks).
  2. Landau re ex, a two-stage re ex. With the child supported prone (with your
    hand under the abdomen), the child should (normally) extend head, trunk and hips. is is the rst, and more important, stage. Next, ex the head and neck; normally the response is exion of trunk and hips, but this is less constant than the rst stage ( rst stage from 4 months, plus second stage from 9 months; gone by 2 years).
  3. ATNR. With the child supine, the head is rotated to one side. A ‘fencing’ posture develops, with extension of the ipsilateral upper and lower limb (i.e. the side towards which the head is turned) and exion of the opposite side (2–6 months). Persistence beyond 6 months is indicative of upper motor neurone problems, especially CP. Maintaining the ATNR posture throughout the time that the head is held turned, such that the child cannot ‘break’ from that position, is similarly signi cant.
  4. Neck-righting re ex. Rotation of the trunk to conform with the position of the head when the head is rotated to one side (6 months to 2 years).
  5. Moro re ex (birth to 4 months). As with most primitive re exes, persistence beyond the usual time of disappearance is pathological. Make a point of focusing not only on the limb movements but also the facial response, for asymmetry (e.g. in hemiplegic CP).
  6. Parachute re ex. With the infant held in the prone position, move him or her rapidly, face downwards, towards the oor. e normal reaction is to extend both upper limbs as if to break the fall (appears between 6 and 12 months, usually at 9 months, and persists; its absence beyond 12 months is abnormal). Asymmetry occurs with hemiplegia.
229
Q

Causes of pes cavus

A
CMT
Frederich ataxia (spinocerebellar degeneration)
230
Q

Floppy weak and floppy strong infant causes

A

Floppy weak: SMA, congenital myotonic dystrophy, congenital myopathy, werdnig-Hoffman

Floppy strong: downs, hypotonic CP

231
Q

Cause of scoliosis

A

Idiopathic
Paralytic (neuromuscular)
Bony or ligamentous (CT disorders)

232
Q

Biprosthetic valve types

A

Xenografts - porcine and bovine

Allograft - cadaveric or auto

233
Q

Causes of cardiomyopathy

A
Congenital
HTN
Infective
Idiopathic
Toxin (chemo)
Infiltration - amyloid, sarcoidosis, metabolic, haemochromatosis
Endocrine Hypothyroidism and acromegaly 
CTD
Ischaemic
234
Q

Causes of cardiac failure

A
Congenital heart disease 
Valvular
Arrhythmia 
Ischaemic
High output state: anaemia, thyrotoxicosis, Av fistula..
235
Q

DDx of ejection systolic murmur

A

AS
PS
Hypertrophic cardiomyopathy
ASD

236
Q

Ascites causes

A

Prehepatic: CHf, Budd-Chiari syndrome, IVC obstruction
Hepatic: decompensated cirrhosis with portal HTN
Nephrotic, TB, chylous, pancreatitis

237
Q

Atrophic glossitis due to

A

Iron def, B12 and folate def

238
Q

Can’t smell in what syndrome

A

Kallman

239
Q

Ptosis causes

A

Congenital, MG, Myotonic dystrophy, thyrotoxicosis, horners, third nerve palsy

240
Q

Causes of facial n palsy

A

UMN

LMN: bells, OM, acoustic neuroma, meningioma, MS, fracture, Ramsey hunt, parotid enlargement, vascular

241
Q

Stamping or slapping gait with what

A

Sensory neuropathy

242
Q

Peripheral neuropathy or nerve stuff DDx

A
Congenital such as CMT
Vascular/vasculitis
Infectious/inflam - GBS/transverse myelitis, syphillis, botulism, Lyme
Trauma
Autoimmune MS
Metabolic - DM, thyroid, uraemia, vit B12/E, B1 and B6 def
Iatrogenic/ idiopathic
Neoplastic - 
A
B
C
Drugs - vincristine, lead
243
Q

Causes of foot drop

A

CommonPerineal nerve palsy, sciatic nerve palsy, lumbosacral plexus lesion, L4/L5 lesion, PMN, distal myopathy, spinal cord lesion

244
Q

Genetic syndromes and gene defects assoc obesity

A
Prader-willi 
Alstrom 
Cohen
Albrights 
Carpenter 
Rubinstein-taybi
Fragile x
Lawrence moon bardet biedl
245
Q

Pain and temperature and proprioception test what

A

Pain and temp is spinothalamic pathway

Proprioception/vibration is dorsal columns

246
Q

Hypertension causes in NF1 (5)

A

Renal artery stenosis, phaeo, coarct, neurofibromas, essential

247
Q

Waddling and wide based gait indicates

A

Waddle weakness in proximal myopathy and wide in cerebellar

248
Q

Romberg sign looks for

A

Dorsal column pathology

249
Q

Bullying plan 5 steps

A

1: explore and fix cause eg smell or drooling
2: increase self esteem highlight strengths and increase support peers and family home environment and ‘buddy’ or mentor at school and adult he can trust to report to
3: school policy, avoid situations that exacerbate or change schools, karate or other activity to help
4. list some immediate things your child can do when the bullying happens. For example: first, ignore the bullying behaviour. If that doesn’t work, tell the person to stop. If the bullying continues, walk away. Tell someone.
5. Books on bullying or website WHATSUP
Cyber bullying may need restrict websites/internet use, kia kaha police programme safety in schools
6. Regularly check in with child and reassure that telling them is the right thing

250
Q

Drooling (sialorrhoea) amount per day and causes and complications and management

A

500ml-2L per day
Overproduction or inability to swallow
Complications are social implications and embarrassment of pt and family, skin irritation, frequent bib and clothes change, smell, increased risk aspiration, choking and coughing episodes

Mx:
Saliva clinic SSH
Medications - anticholinergics such as atropine and glycopyrolate
Behavioural modifications and physical therapy (positioning and seating)
Oromotor rehabilitation / therapy by Speech Therapist (increase sucking - straw) and increase oral awareness
Orthodontic treatment
Improvement of nasal and oral airway patency - Otolaryngology (ENT)
Catellio moreles prosthesis
Surgery to the salivary glands - side effect dry mouth and dental problems
Botox injection to submandibular / parotid glands
No treatment but use waterproof bib or clothes

251
Q

FTt causes

A

Inadequate intake, neglect
Malabsorption
Increased energy use or demands

252
Q

Oral aversion plan

A

Eliminate cause if can - tubes, reflux, nausea
Educate
Introduce food early age and make it fun, fa,ily meals
Monitor growth
Monitor dentition

253
Q

Causes of scoliosis

A

SCOLI

Syndrome NF1 marfans
Congenital 
Orthopaedic (leg length)
Low and high tone neuromuscular - CP, Spina bifid a, SMA, DMD
Idiopathic
254
Q

Sz managment

A
Safety
Emergency plan
Avoid triggers
AED
Assessments
Monitoring (phenytoin and phenobarb) CMZ
Avoid drugs that aggravate 
Surgery
Vagal stimulation
Ketogenic diet
255
Q

Involuntary movements causes

A

Bells intend to keep moving:
Cerebellar lesions are associated with intention tremor.

Opposite BallS:
Hemiballismus is due to contralateral subthalamic nucleus lesion.

Put at globe:
Putamen / globus pallidus damage causes Atheosis

2 C’s:
Chorea is caused by Caudate nucleus lesion

CP

Drugs

Tics

Infection

Trauma NAI AV malformation

Autoimmune MS

Seizure

256
Q

Mental state examination

A

Mnemonics for MSE: ASEPTIC

A- Appearance and Behaviour 
S- Speech 
E- Emotion [mood and affect] 
P- Perception [Hallucination and illusion] 
T- Thought content and process 
I- Insight and Judgement 
C- Cognition 

Mnemonics for examination for cognition: GOAL-CRAMP

‘I’d like to start off by asking you a few questions to test your concentration and memory……….’

G- general: Alertness and Co-operation
O- orientation: Time and Place
A- attention: WORLD backwards and Serial Sevens
L- language: Naming and Repetition
C- calculation: Division and Subtraction
R- right Hemisphere Function: Intersecting pentagons and Clock-face
A- abstraction: Proverbs and Similarities
M- memory: Short term and Long-term memory
P- praxis: Wave good-bye and Comb hair

257
Q

Mouthing toys age

A

6-15/18m

258
Q

Removes clothes and independent with spoon which age?

A

18m

259
Q

Prepositions, object use and 6 body parts what age

A

24m

260
Q

Points to the right image of cat…what age

A

15-18m

261
Q

Imaginative play at what age

A

3y

262
Q

Catch well and kick well and jump two feet what age

A

3y

263
Q

Throw ball what age

A

18m

264
Q

Tense and big/little opposites at age..

A

4y

265
Q

Stand on one foot for how many seconds

A

3 at 3 and one second per year of age after that

Can skip at 5y

266
Q

Handedness by what age

A

18m

Mature pen grip by 5y

267
Q

Short with polydactyly think

A

Bardt-biedl

268
Q

Clindodactyly and short think

A

Downs, Russell-silver, seckels, shwachmans

269
Q

A body habitus that is tall, slim and underweight, with long legs and long arms (i.e., arm span exceeds height by 5 cm or more).

A

Eunuchoid habitus. Definition

270
Q

Long limbs

A

Dolichostenomelia is a human condition or habitus in which the limbs are unusually long. The name is derived from Ancient Greek (dolichos - long, steno - short, narrow, close, melia - of the limbs). It is a common feature of several kinds of hereditary disorders which affect connective tissue, such as Marfan Syndrome and homocystinuria.

271
Q

Facial nerve palsy causes

A

Idiopathic Bell’s palsy most common (unknown or viral)
Trauma I.e. due to delivery traumas
AV malformation
Infective -EBV/Ramsay Hunt
Iatrogenic
Tumour/neoplastic (schwannoma)
Congenital: Möbius syndrome, Goldenhar, Poland syndrome, pseudobulbar palsy, Arnold-chiari
NMJ: MG, botulism
Muscular: hereditary myopathies, such as myotonic dystrophy

272
Q

CXR in cardio patient DDx of increased and decreased pulmonary vascularity

A

Increased:
Truncus, TAPD, TGA, (large L to R shunt, ASD, VSD, PDA)

Decreased:
TOF, Pulmonary atresia, TA, Ebsteins, critical PS

273
Q

Causes of RAH

A
TA
Hypoplastic R heart
Ebsteins
PA
Complex congenital
274
Q

Deep Q waves on ECG seen in

A

HCM
TGA
anomalous left coronary artery

275
Q

LAD causes

A

Endocardium cushion defect
TA
HCM
Inlet VSD

276
Q

RAH causes

A
Ebsteins
TA
PA with intact septum
Truncus
TOF large VSD
277
Q

Short forth metacarpal in

A

Turners, fetal alcohol, pseudopseudohypoparathyroidism

Trauma, infection

278
Q

Micropenis causes

A

Kallman s and hypopit

279
Q

Froehlich syndrome is

A

a rarely encountered condition that occurs mainly in males and is characterized by obesity, small testes, and a delay in the onset of puberty.

280
Q

Bronchiectasis causes

A

bx causes
There are multiple causes including - CIRICCI
Cystic fibrosis
Infection with pertussis, measles, TB, adenovirus (7 &14)
Recurrent infections or aspirations
Less commonly
Immune deficiency
Ciliary dyskinesia
Congenital abnormality of CT (→ obstruction →↑ infections)
If localised areas of bronchiectasis
Inhalation of foreign body

281
Q

Qualities of apex beat and causes

A
Quality of apex beat
Quality
Sustained – pressure overload in AS
Forceful – LVH
Thrusting – volume overload (MR/AR) or L→R shunt
Parasternal heave – RVH

Palpable S2 = pulmonary hypertension

282
Q

Causes of an abdominal mass

A
Appendiceal Abscess
Tumors
Wilms
Neuroblastoma Hepatoblastoma
HCC
Teratoma
Lymphoma
Ovarian Cysts
Renal
Hydronephrosis
Cystic disease
Obstructed bladder
283
Q

Short child syndrome characterized by a progressive loss of vision and hearing, a form of heart disease that enlarges and weakens the heart muscle (dilated cardiomyopathy), obesity, type 2 diabetes mellitus (the most common form of diabetes), and short stature.

A

Alström syndrome

284
Q

Bardet-Biedl syndrome (BBS) is an autosomal recessive multisystemic genetic disorder characterised by six major defects which are…

A

Ciliopathy

obesity, learning disability, renal anomalies, polydactyly, retinal dystrophy and hypogenitalism

285
Q

Pickwickian syndrome: 4 features

A

The combination of obesity, somnolence (sleepiness), hypoventilation (underbreathing), and plethoric (red) face.

286
Q

Features of coffin lowrey syndrome

A

Severe ID
short stature, an unusually small head (microcephaly), progressive abnormal curvature of the spine (kyphoscoliosis), and other skeletal abnormalities.

287
Q

Abnormal RAPD in affected eye less constriction both pupils - name and causes

A

Marcus Gunn pupil

Causes by optic nerve lesion, severe retinal disease, optic neuritis

288
Q

What is an Argyll Robertson Pupil

A

It is bilateral small pupils that reduce in size on a near object (i.e., they accommodate), but do not constrict when exposed to bright light (i.e., they do not react to light). They are a highly specific sign of neurosyphilis; however, Argyll Robertson pupils may also be a sign of diabetic neuropathy. In general, pupils that accommodate but do not react are said to show light-near dissociation (i.e., it is the absence of a miotic reaction to light, both direct and consensual, with the preservation of a miotic reaction to near stimulus (accommodation/convergence).[1]

289
Q

Ortolani and Barlow test

A

Ortolani opens up lower limbs and Barlow bumps back

290
Q

Erbs palsy roots

A

C5,6

291
Q

What is galant reflex used for?

A

To look at structural integrity T2 to S1

292
Q

What conditions cause leukocoria

A

Many conditions cause leukocoria including cataract, retinal detachment, retinopathy of prematurity, retinal malformation, coats disease, intraocular infection endophthalmitis), retinal vascular abnormality, and intraocular tumor (retinoblastoma).

293
Q

What is pellagra

A

Pellagra is a disease characterised by diarrhoea, dermatitis (scaly brown) and dementia. If left untreated, death is the usual outcome. It occurs as a result of niacin (vitamin B-3) deficiency. Niacin is required for most cellular processes

294
Q

Brown nails can indicate?

A

Chronic Kidney Disease

295
Q

Bleomycin important side effects

A

Interstitial pneumonitis leading to Lung fibrosis from oxidative damage

296
Q

Bulbar palsy weather is it and causes?

A

Bulbar palsy results from bilateral impairment of function of the IXth, Xth and XIIth cranial nerves. This gives rise to dysarthria, dysphagia (often with choking episodes and nasal regurgitation of fluids), dysphonia and poor cough, and susceptibility to aspiration pneumonia. The lowermost part of VII may, infrequently, be involved.

The disturbance is of the motor nuclei rather than of the corticobulbar tracts. It is distinguished from pseudobulbar palsy by the presence of lower motor neurone signs. Autonomic features are uncommon.

Causes of bulbar palsy include:
MND
Guillain Barre syndrome
poliomyelitis
diphtheria
myasthenia gravis
neurosyphilis
Tumour
Stroke
Syndromic or genetic
297
Q

Anti epileptic med’s that can measure serum levels

A

Carbamazepine
Phenytoin
Phenobarbitone

Not valproate (unless suspect non compliance)

298
Q

90% of Spina bifida have…

A

Hydrocephalus

Arnold chiari II

299
Q

Scoliosis management

A

Monitor X-ray watch and see
Brace or cast
Surgery

300
Q

Lens displacement in marfans and homocysteinuria

A

Marfans superior intellect so superior

Homocysteinuria inferior

301
Q

Optic nerve hypoplasia association

A

Absent corpus callosum

302
Q

Noonan complications

A
Noonan syndrome have an eightfold increased risk of developing leukemia or other cancers over age-matched peers.
Bleeding tendency
Pubertal delay
Short
AD
303
Q

Denys-Drash

A

Denys–Drash syndrome (DDS) or Drash syndrome is a rare disorder or syndrome characterized by gonadal dysgenesis, nephropathy, and Wilms’ tumor.

304
Q

Impairment, disability and handicap definition

A

Impairment – residual limitation resulting from disease, injury or congenital defect
Disability – Difficulties an individual may have in executing activities – inability to perform a functional skill
Handicap – Problems an individual may experience in life situations – interaction of a disability with the environment

305
Q

Cyanosis implies?

A

Right to left shunt at level of heart or great vessels

Inadequate oxygenation by kings

306
Q

Left lateral chest wall scar causes?

A
Coarctation repair
PDA ligation
BT shunt
PA banding
Thoracotamy
307
Q

Absent or reduced brachial pulse causes

A
Congenital
Embolisation
Previous catheter
Cervical rib
BT shunt

Absent left could be from coarctation repair

308
Q

Flat foot pes planus causes

A

Dyspraxia, ligamentous laxity, hypermobility (collagen disorder ethers-danlos and marfan), CP

309
Q

Causes of pes cavus

A
CMT
Fredereichs ataxia
Neurological 
Trauma
Orthopaedic 
Neuromuscular
Spina bifida
Diastometamyelia
310
Q

AF causes

A

AEIOU

ASD
Ebsteins
Infective (RF)
Open heart or atrial surgery
Unknown
311
Q

Jerky pulse and collapsing pulse causes

A

HOCM

AI and PDA

312
Q

Mean diastolic and systolic values

A

Age + 90/ age + 55

313
Q

Causes of displaced apex

A

Cardiomegaly
Scoliosis
Pectus

314
Q

Loud second heart sound from?

A

Pulmonary HTN

Increased pulmonary flow from left to right shunt in ASD, VSD, PDA

315
Q

Murmur description in presentation

A

X is a child with cyanotic/acyanotic repaired/unrepaired congenital/acquired heart disease

There was a murmur:
Timing and duration
Intensity
Pitch and quality

Loudest
Changes with position
Radiated to

Second heart sound

No/ was Evidence of failure

Differential diagnosis and investigations

316
Q

Radiation of murmur to back in

A

Coarctation, Ps, PDA

317
Q

Complications in cystic fibrosis

A

Head to toe approach

Psychological
Nasal polyps
Respiratory infections and bronchiectasis
Pancreatic failure and diabetes
Bowel obstruction
Liver (focal biliary)
Reproductive fertility issues
Pubertal delay
Bone health (osteoporosis)
Electrolyte imbalance
318
Q

Macroglossia is causes

A
Hypothyroid
BWS
Mucopolysaccharoidoses
Pompe
Amyloid
Downs is not true
319
Q

Causes of visual impairment in children

A
Trauma to eye
Corneal clouding (multiple causes)
Glaucoma (assoc sturge weber)
Uveitis
Cataracts/lens dislocation
ROP
Retinoblastoma 
Retinitis pigmentosa
Cherry red spots from metabolic disease
Optic nerve neuritis or atrophy (Cp/septoopticdysplasia/tumour)
Optic nerve pathology (such as glioma)
Cerebral causes
320
Q

Leg length discrepancy causes

ASIS to medical malleolus

A
DDH
Trauma or surgery
Arthritis
Hemiparesis
Osteogenesis imperfecta 
Henihypertrophy
polyostotic fibrous dysplasia in MAS
321
Q

Conditions associated with cafe au lait spots

A
NF
TS
Fanconi
McCune Albright
Ataxia telangiectasia 
RUssle silver
Blooms
Gaucheries
Chediak-Higashi
322
Q

Bitemporal hemianopia due to

A

Optic chasm compression

323
Q

Causes of a nonreactive pupil to light

A
Third nerve palsy
Cataracts corneal opacitoes, vitreous and retinal haemorrhage or detachment
Infection iris and ciliary muscles
Trauma
Optic nerve pathology
324
Q

Nystagmus direction in vestibular and cerebellar causes

A

Vestibular away from lesion
Cerebellar towards side of lesion
(Fast phase)

325
Q

Spinal roots and reflexes

A

Triceps C7,8
Supination C5/6
Biceps C5/6

Knee L3/4
Ankle S1/2

326
Q

Causes of proprioception loss

A
Peripheral neuropathy
Vincristine
GBS
Fredeichs ataxia
CMT
Hereditary neuropathy
327
Q

Holt Oram syndrome characterised by these two things

A

Upper limb skeletal abnormalities and heart problem

328
Q

Mono brow also called

A

Synophrys

329
Q

Short fingers and toes called

A

Brachydactyly

330
Q

Lesion CN 9 & 10 suggests

A

Bulbar palsy (MND)

331
Q

LEOPARD stands for

A

L – lentigines (multiple brown-black spots on the skin)
E – electrocardiographic (ECG) conduction defects
O – ocular hypertelorism
P – pulmonary stenosis
A – abnormalities of genitals
R – retarded growth resulting in short stature
D – deafness or hearing loss due to inner ear malfunction

332
Q

Atlanto-axial subluxation causes

A
congenital
os odontoideum
Down syndrome (20%)
Morquio syndrome
spondyloepiphyseal dysplasia
osteogenesis imperfecta
Marfan disease
neurofibromatosis type 1 (NF1)
arthritides
rheumatoid arthritis
psoriatic arthritis
Reiter syndrome (reactive arthritis)
ankylosing spondylitis
systemic lupus erythematosus (SLE)
acquired
trauma
retropharyngeal abscess / Grisel syndrome
surgery
upper respiratory tract infection
333
Q

Kearns-Sayre syndrome triad

A

Kearns-Sayre syndrome triad includes onset in persons younger than 20 years, chronic, progressive, external ophthalmoplegia, pigmentary degeneration of the retina and cardiac problems

334
Q

Surgically-created cardiac shunts (mnemonic)

A

Mnemonic
Great flow really would be perfect
G: Glenn shunt
superior vena cava to right pulmonary artery
F: Fontan procedure
inferior vena cava or right atrium to right pulmonary artery
R: Rastelli procedure
right ventricle to main pulmonary artery
W: Waterston shunt
ascending aorta to right pulmonary artery
B: Blalock-Taussig shunt
subclavian artery to pulmonary artery
P: Pott shunt
descending thoracic aorta to left pulmonary artery

335
Q

Pleural effusion causes

A
Exudate:
CT diseases, pneumonia, TB, drugs and radiation
Transudate:
Low albumin
Low thyroid
Low cardiac output in failure
336
Q

Bronchiectasis causes

A

Congenital and acquired

Congenital: PCD, CF, immune deficiency
Acquired: infective, FB, TB

337
Q

Glossitis cause by

A

B vitamin deficiencies esp B12
Folate
Iron

Geographical tongue by def B vitamins

338
Q

Systemic signs of IBD and complications

A
Ocular changes/signs of anaemia
Clubbing
CLD - cirrhosis/PSC
Gallstones and renal stones
Arthritis
Skin manifestations

Complications: of treatment, mega colon, bleed or perforation, cancer

339
Q

Ascites causes

A
Cirrhosis
Veno oclusive disease
CHF
Constrictive pericarditis
TB
Pancreatic
Nephrotic syndrome
340
Q

Causes of abdominal mass

A
Important not to miss:
Cancer
Abscess
AAA
BO

Common:
Constipation
Bladder enlargement
Organomegaly

Other:
Transplanted kidney
Gastric mass
Ovarian cyst
Hernia
Uterus causes

B

341
Q

Causes of fasiculations

A
MND (SMA)
Motor root compression
Peripheral neuropathy
Primary myopathy
Thyrotoxicosis
342
Q

Palmar drift causes

A

Cerebellar
Proprioception
UMN pyrimidial weaknss

343
Q

Causes of foot drop

A
Common peroneal nerve palsy
CMT
L4/L5 lesion
Stroke
Distal myopathy
MS
Muscular dystrophy
Peripheral motor neuropathy
344
Q

Waddling gait causes

A

A waddling gait is the style of walking that is seen in a patient with proximal myopathy.

It is characterised by:

a broad-based gait with a duck-like waddle to the swing phase
the pelvis drops to the side of the leg being raised
forward curvature of the lumbar spine
marked body swing
This gait may also be seen in patients with congenital hip dislocation and pregnancy.

345
Q

US/LS ratio

A

1.7 at birth
1 by age 10
0.8 by puberty

346
Q

Scoliosis management

A

Observe
Conservative - physiology and exercises, chair adjusted, bracing
Surgical

347
Q

Management points for diabetes

A

Assess current control with reviewing investigations and BSL record - may need to alter insulin regime
Survellience for complications
Ongoing education everyone involved including safety with alcohol, avoid smoking
Diet and exercise discussed
Sick day management, hypoglycaemia plan and travel plan (letters, contacts,supplies)
Medic alert
Health optimisation with vaccinations

Psychosocial support with diabetes youth groups diabetesyouth.org.nz
Diabetes NZ

Transition plan

348
Q

Complications of diabetes screening plan

A

Retinopathy and diabetic nephropathy [urine sample, ophthalm] yearly from 5 years after diagnosis or from 10 years or 2 years after diagnosis in adolescent
BP yearly

Lipids checked 5 yearly or from 12 years or annually after puberty

Yearly FT and coeliac screen for 5 years then 2 yearly

Neuropathy, joint and skin problems, CV risk

349
Q

DMD management

A

Medical treatment with steroids

Survellience and management of complications:
- mood and behaviour
- Rarely have cognitive impairment - wisckott score to identify areas requiring assistance and strengths, individualised learning plan for appropriate assistance with educational and physical needs
- Cardiomyopathy Echocardiogram treat failure
- respiratory nocturnal failure/polysomnography - bipap
- Contractures and scoliosis - passive and active exercises physiotherapy assisted programmes
OT assisted supports and equipments and review suitability of home environment
Orthotics and surgery
- Nutrition with exercise and diet advice

Other:

  • end of life and palliative (life limiting illness) support from MDA for coping grief and loss
  • Steroid side effects
  • Avoid smoke and treat infections aggressively
  • avoid patronising and overprotective
  • workbridge can assist with vocation planning
  • parent project muscular dystrophy
  • Nzord.org.nz