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BPT Clinical exam > Long case > Flashcards

Long case Flashcards

1
Q

A common complication of hypoplastic left heart repair is protein losing enteropathy.

How do you diagnose and manage this?

A

Dx: Low albumin, and 24 hour stool A1AT to diagnose

Mx: High protein, high MCT, low sodium and low fat diet

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2
Q

What is the most common cancer associated with Hypoplastic left heart sx and how do you monitor/surveillance for this?

A

Hepatocellular carcinoma

Liver imaging every 3-5 years -> if abnormal monitor AFP and consider Q6mo imaging.

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3
Q

What regular assessments are important in a child with Congenital heart disease?

A

Exercise tolerance test, BP monitoring, lung function tests, consider BMD

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4
Q

What are four common complications post cardic surgery?

A

Post op Horner’s, RLN palsy, diaphragmatic palsy and protein losing enteropathy

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5
Q

Why do you get restrictive lung disease post cardiac surgery?

A

Die to the sternotomy and thoracotomy related scoliosis

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6
Q

What emergency planning might be important in a child with Congenital heart disease?

A

ED letter stating congenital anomaly with baseline observations +

Consider a MedicAlert bracelet

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7
Q

What are you told to avoid in children with hypertrophic cardiomyopathy?

A

Avoid competitive sports and dehydration

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8
Q

What further investigations are required with marfan’s syndrome?

A

1) annual echo to review aortic root
2) MRI spine for spinal canal enlargement
3) Spinal XRAY for scoliosis

Opthal assessments

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9
Q

6 medical management points for Marfan’s syndrome?

A

1) Beta blockers and ARBs to slow progression of aortic dissection
2) opthal review for lens subluxation, cataracts and glaucoma
3) Review joints due to laxity, review scoliosis and consider orthopaedic referral
4) Individualised sports program, no scuba dividing , no sit ups, weights, contact sports or strenuous exercise
5) Avoid - Stimulants, vasoconstrictors (triptans) and avoid laser eye surgery
6) pregnancy prophylaxis due to risk of aortic rupture

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10
Q

How would you optimise nutrition and growth in a child with CKD?

A

1) monitor: centile charts, yearly wrist XRAY for bone age (rickets)
2) Adequate protein/energy; Ix and Mx salt losing; Manage acidosis (bicarb supplementation?)
3) Monitor for hypothyroidisim
4) Consider feed supplementation; link with renal dietitian
5) Consider GH for older children (refer to endocrine team)

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11
Q

How do you manage bone mineral disease in CKD?

A

1) Monitor for bone pain and deformity (osteitis fibrosa cystica and rickets)
2) Low phosphate diet
3) Consider phosphate binders to prevent secondary hyperparathyroidisim
4) Vit D supps
5) Monitor PTH and ALP;
6) DEXA 2 yearly especially post transplant/chronic nephrotic syndrome

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12
Q

Which allied health services might be important in a child with CKD/Nephrotic syndrome?

A

1) Nutrition/dietician - ensure adequate protein/energy/low phosphate diet
2) Social work - impact on parents, link with Kidney health Australia, dialysis/transplant associations
3) psychology or neuropsych as needed

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13
Q

What are the four primary features of Nephrotic syndrome?

A

Proteinuria + hypoalbuminaemia + hypercholestrolaemia + oedema

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14
Q

What are other secondary features of nephrotic syndrome?

A

Hypocalcaemia, hyperkalaemia, hyponatraemia, hypercoagulability, hypothyroidism

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15
Q

What emergency management plans do you need for a child on dialysis?

A

Plan for:

  • What if catheter falls out?
  • What if the peritoneal fluid becomes cloudy?
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16
Q

What workup is necessary prior to transplant?

A
  • Ensure all live vaccines are done prior
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17
Q

What are the major side effects associated with Mycophenolate?

A

Myelosuppression, malignancy, gut effects

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18
Q

What are major side effects associated with Tacrolimus?

A

Htn, GI sx, nephrotoxicity, infection, malignancy, low Mg

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19
Q

What infections do you need to monitor for pre-post/transplant?

A

1) PJP proph - Bactrim or pentamidine
2) screening for CMV, EBV, PCP, BK
3) Valgancyclovir for CMV (usually first 3-6 month)
4) Annual flu vax
5) Consider UTI prophylaxis

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20
Q

What are the four domains of routine follow up post renal transplant?

A

1) Cardiovascular - increased incidence of Htn and hyperlipidaemia
2) Bones - osteoporosis from steroids, vitamin D deficiency; bone density monitoring bi-annually
3) Cancer risk - skin protection and regular review. HPV vaccine
4) Disease recurrence
- MPGN, HSP, IgA nephropathy, FSGS, HUS
- Monitor proteinuria and biopsy for recurrence

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21
Q

What surveillance is required for post renal transplant?

A

1) Monthly - growth, BP, oedema, EUC, immunosuppressant levels, UA
2) Second yearly US, eye/skin/bone density reviews
3) Careful dosing of nephrotoxic drugs

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22
Q

What future planning is required after a transplant?

A

1) managing fertility issues and genetic counselling

2) need for re-transplant

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23
Q

What other things should you screen for when reviewing a patient with T1DM?

A

Thyroid, coeliac, adrenal issues

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24
Q

What Ix should you ask about for hx of T1DM?

A

OGTT or Insulin antibodies

Ask about any liver US and any other testing related to other autoimmune conditions

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25
Q

What should you aim for your HbA1C in smaller children?

  • IN school aged children?
  • IN adolescents?
A

Small children - 8-9.5%

School aged children: 8%

Adolescents: HbA1c as low as possible

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26
Q

What screening do children with hypoglycaemia need?

A

annual eye exam
Annual BP and albumin Cr ratio
Neuro/feet exam

Flu vax
Regular dentist review

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27
Q

What emergency plans do children with T1DM need?

A

Hypo plan
Sick day plan
Prevent dehydration

Medic alert bracelet
Emergency sugar

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28
Q

What support services are available for diabetes kids?

A

Diabetes australia - diabetes camps, education

National Diabetes service scheme

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29
Q

Immediate emergency plans for adrenal insufficiency?

A
  • Emergency home IM dose of hydrocortisone
  • Emergency care plan and prescription
    Letter for ambulance/ED
  • Medic alert bracelet
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30
Q

What investigations do children with long term steroids need?

A

Bloods - HbA1C, fasting BGL, lipids, Vit D, ALP, CMP

BMD - every 6-12mo
Opthalmology for cataracts/glaucoma

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31
Q

Whats the synthacen test?

A

Assess renal response to ACTH

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32
Q

What Allied health input is particularly important for children with CAH?

A

Social work
- Help link with financial supports and CAH groups
Psychology
- One on one/discussion groups

Sexual counselling

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33
Q

What immediate/emergency interventions are important for CAH?

A
  • Medicalert bracelet

- Emergency plan and letter

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34
Q

What medical follow up is important for CAH?

A
  • Should be 3-4 monthly bloods - review renin activity, electrolytes and also androgens
  • Optimise hydrocortisone dose
  • Annual bone age (increased androgens can advance bone age)
  • Monitor height/weight and tanner staging
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35
Q

What investigations are important for hypopituitarism?

A
  • Bloods: UEC, BGL, pituitary hormones
  • Genetic testing: CHD7
  • Imaging: MRIB - tumour, septo-optic dysplasia
  • Opthal Ax
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36
Q

Which allied health specialist are helpful in managin bone health and why?

A
  • Physiotherapist - encourage weight bearing exercise

- Nutrition/Dietician - assess and optimise Ca intake, optimise nutrition

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37
Q

What medications are RF for impaired bone health?

A

AEDs, steroids, warfarin

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38
Q

what further investigations might be helpful in exploring constipation?

A
  • Coeliac serology, TSH, iron studies
  • Imaging: bowel transit studies, MRI spine?
  • Bowel transit studies
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39
Q

3 behavioural strategies to manage constipation?

A

1) Sit on toilet after breakfast + regular habits
2) Foot stool at the toilet
3) Avoidance of stool holding

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40
Q

What allied health input may be helpful for constipation?

A
  • Nutrition/dietician

- Psychology for behaviour issues

41
Q

What investigations might be important in a child with chronic liver disease?

A
  • Bloods - LFTs, INR, Albumin, Vit ADEK, BGL, Ammonia
  • Tacrolimus/CSA level
  • ?Ascitic tap
  • Imaging: Abdo USS + DOPPLER
  • Procedure: endoscopy for varices
42
Q

The pneumonic HEPATIC is used to explore chronic liver disease implications:

A

H - Hepatorenal/pulmonary syndrome, Hypersplenism
E - Encephalopathy, Esophageal varices
P - Portal Htn (haematemesis, PR bleeding, caput medusae)
A - Ascites
T - Thrombosis of portal vein
I - Infection (SBP, cholangitis)
C - Coagulopathy, Carcinoma

43
Q

Which medications can help manage itch in chronic liver disease?

A

Rifampicin, Ursodeoxycholic acid, cholestyramine

44
Q

Prophylactic ___ may be needed after a Kasai procedure to prevent _______

A

Prophylactic antibiotics to prevent ascending cholangitis

45
Q

What nutritional deficiencies can occur in children with Chronic liver disease?

A

Vit ADEK deficiency (fat soluble)

46
Q

What complications can occur with neonatal UVCs?

A

Risk of clots after neonatal UVC -> leading to portal hypertension

47
Q

How is the spleen affected from portal hypertension?

A

Can lead to splenic sequestration of platelets

48
Q

How is education impacted from Chronic liver disease?

A

Long term hospital stay + low grade encephalopathy can effect background performance at school

49
Q

What are some routine investigations required in IBD?

A
  • Bloods - regular bloods + Vit ADEK, Coags (vit K), albumin, zinc, copper, selenium
  • Drug level monitoring - 6MMP levels, MTX levels
  • Faecal calprotectin, stool culture - every 6 mo
  • Rule out CMV, C Diff and TB
  • Imaging: MRI enterography (cronhs) annually, MRI pelvis if concerned about fistualising disease
  • Can consider Dexa every 2-3 years
    Procedure: endoscopy as needed
50
Q

For UC _____ are mainstay of inducing remission

For CD _______ are mainstay of inducing remission

A

UC - Steroids

CD - EEN

51
Q

What are four common side effects of Azathioprine?

A

Bone marrow suppression, risk of skin cancer, hepatotoxicity, pancreatitis, T cell Lymphoma

52
Q

For Methotrexate what are some side effects?

A

Nausea and vomitting; teratogenecity if fertile

53
Q

What do you need to ensure prior starting immunosuppression?

A

TB workup, IUTD

54
Q

What cancer monitoring is important for IBD? (3)

A

1) Colon Ca in UC - after 8 years of disease, yearly colonoscopy and biopsy
2) Lymphoma - biological or immunomodulator theraoy
3) Monitoring for skin cancers long term on Imuran

55
Q

Conner’s questionaire is used to help diagnose?

A

ADHD

56
Q

What investigations might you consider before diagnosing a child with ADHD? (3)

A
  • Hearing and vision assessments
  • Sleep study;
  • Microarray, Fragile X testing; Fe def and lead toxicity
57
Q

What further investigations may be required for a child with oral aversion? (3)

A

1) imaging
2) assessment for sensory processing issues
3) Nutritional bloods - Vitamins and ferritin

58
Q

What might be triggers for escalating behaviour in any child? (6)

A

1) Sleep - equipment compliance, hours of sleep, waking, snoring
2) Infection - dental/UTI
3) Constipation
4) Pain - hips, contractures
5) Seizures
6) Puberty

59
Q

What assessments might be helpful for speech delay? (5)

A

1) Audiology
2) Swallow assessment/barium swallow
3) MRIB fro CN VIII lesions/cochlear planning
4) Neurological examination
5) Allied health review

60
Q

What interventions can a paediatrician help implement for speech delay?

A

1) Improve parental involvement in language and learning
2) Removal of screen times
3) Refer to Hanen it takes two to talk program (online/small groups)
4) Specialised play groups with Allied health supervision
5) NDIS early childhood intervention program

61
Q

Differentials for sensorineural hearing loss?

A

1) Genetic
2) Infection
3) Ototoxic medication
4) HIE

62
Q

Syndromic causes for hearing loss?

A

Usher, Waardenburg, Pendred

63
Q

What audiology testing would you use for a 12mo infant?

A

Visual field reinforcement test

64
Q

What other testing would you consider for hearing loss?

A

1) Bloods: TFT, Genetic testing
2) MRI inner ear/brain
3) Othal review (retinal changes in Usher Sx)
4) ENT review - Middle ear review and consideration of cochlear impant

65
Q

What support services could you refer to for hearing loss?

A
  • Hearing australia - provide hearing aids/help monitoring

- Early intervention groups - playgroups, allied health review

66
Q

What are some side effects with Desmopressin?

A

Headaches, vomitting, epistaxis and nasal congestion

67
Q

What assessments exist for Austism?

Screening?
Formal developmental?
IQ testing?
Teachers reports?

A
  • Screening - MCHAT (parents complete)
  • Formal developmental - ADOS
  • IQ testing -WISC
68
Q

What investigations can you consider with diagnosis of autism?

A
  • AUDIOLOGY
  • HEARING ASSESSMENT
  • If concerns - Genetics -> Fragile X, CGH array, karyotype
  • Urine metabolic screen, EEG
69
Q

Who can help with autism assessment?

A

Speech pathologist + Psychologist -> essential for diagnostic assessment to qualify for early intervention

70
Q

What educational guides are available for families with a child with a diagnosis of Autism?

A
  • School based special education
  • Stepping stones, Triple P - parent training programme
  • Raising children Network government page (information about diagnosis, assessment, benefit of early intervention)
71
Q

There are many commonly associated conditions with Down Syndrome (15 on my list).

1 - if you name 1-3
2 - if you name 4-6
3 - if you name 7-10
4 - if you name 11 -13
5 - if you name 14- 15
A

1) Coeliac disease
2) OSA
3) Seizures, strokes (Cyanotic CHD)
4) Recurrent infections and silent aspiration
5) ENT interventions
6) SCFE
7) Atlantoaxial subluxation
8) Arthritis and foot problems
9) Oral health
10) Weight concerns - obesity, diet and exercise
11) Hypothyroidism and DM
12) Menstrual hygeine, oral contraceptive, PMS
13) Skin issues
- Sebhorreic dermatitis, folliculitis, fungal infections
14) Transient myeloproloferative disorder and risk of AML
15) Psychiatric and behavioural management

72
Q

Screening for Down syndrome - what are the 9 domains?

A

1) Cardiac
- Follow up with ECHO and consider ongoing follow up for PPHn
2) Respiratory/Sleep - OSA
- Monitor for symptoms and consider sleep study
- Sleep study by age 4; ENT review
3) Endocrine - Thyroid
- Annual TSH
4) GI - Coeliac
- Review for Sx - f/u with TTG IgA as needed
5) Haematology -
- Annual FBC, blood film and iron studies
6) Ortho - atlantoaxial instability
- XRAY and specialist referral if symptomatic
- Education for parents
7) Eyes - strabismus/cataracts/nystagmus
- within 6mo and then annual from 1-5 years
8) Hearing- OM and hearing
- SWISH 6 monthly to annually audiology review
- ENT review if hearing loss for middle ear abnormalities
9) Behaviour and development
- Optimise vision and hearing; glasses, hearing aids
- ALWAYS consider medical causes
- Psychologist, psychiatrist, medications
- Sexual safety

73
Q

What are the medical complications of PWS?

A

1) Resp - OSA
- Risk of CSA and OSA
2) GI - Obesity, overeating, poor feeding as infant
3) Neuro - Hypotonia, poor suck as baby, idiopathic hypothermia/hyperthermia,
4) Endocrine - T2DM risk, Hypothalamic hypogonadism, thyroid disease, central adrenal insufficiency, osteoporosis
5) Ortho - Scoliosis/kyphosis, risk of osteoporosis
6) Behaviours - Global developmental delay, high pain threshold, behaviours issues and defiance
7) opthal - monitor for strabismus
8) Dentition - soft enamel, poor hygeine
9) Skin - monitor for picking and easy bruising
10) Meds - caution when starting new meds

74
Q

What does CHARGE stand for?

A

Coloboma of the eye

Heart defect - TOF

Atresia of nasal choanae

Retardation of growth

Genital abnormalities

Ear abnormalities

75
Q

How do you monitor for aspects of iron overload?

A
  • Yearly opthal review
  • Cardiac ECHO
  • Liver depoits (US)
  • Audiology
76
Q

What are some medical impacts of sickling crisis?

A
  • Pain
  • Splenic infarct and sequestration
  • Kidneys - hypertension
  • Acute chest syndrome - lung infarcts
  • Brain infrcts and strokes
  • Retinopathy
  • Dactylitis
  • Risk of septic arthritis
77
Q

Most common type of CP is spastic diplegia which is due to -_________

A

PVL

78
Q

Sibling + partner neglect =

A

Siblings Australia, Relationships Australia

79
Q

What are side effects of anticholinergic medication like glycopyrolate that used for secretion scotnrol?

A

Side effects: Constipation, urinary retention, irritability

80
Q

What medications could help manage dystonia?

A
  • Isolated or idiopathic dystonia - trial of levodopa for dopamine responsive dystonia
  • First line: Artane (trihexyphenidyl) (Gradual uptitration and monitor for side effects)
  • Second line: baclofen

Consider botox and surgery

81
Q

If cerebral blood flow is compromised you get -> Cushings triad which is ___ ___ ___

A

1) Hypertension
2) Bradycardia
3) Irregular respiratory rate

82
Q

How do you manage idiopathic intracranial hypertension?

A
  • Weight loss
  • Avoid tetracyclines which may worsen IIH
  • Treat sleep apnea
  • Acetazolamide first line
  • Cautious use of analgesia, risk of angelsia rebound headache
83
Q

What endocrine anomalies might need to be addressed with Spina Bifida?

A

Precocious puberty, short stature, obesity

84
Q

What are the medical complications of Duchene’s muscular dystrophy?

A
  • GI - constipation, incontinence, reflux
  • Urinary issues - micturition difficultues
  • Joints - contractures, surgeries, splints, Scoliosis
  • Respiratory - Resp failure, sleep disordered breathing, NIV
  • Cardiac - HCM, arrhythmia
  • Cognitive/behavioural - ADHD, aggression, depression, learning difficultu
  • Endocrine: Puberty and growth, steroid side effects
85
Q

What investigations important for Duchene’s?

A
  • DEXA/Spine XRAYs for scoliosis
  • Pulmonary function tests 6mo
  • Sleep study when first in wheelchair
86
Q

Questions to ask parents with child with Chronic lung disease?

A
  • Paviluzimab
  • Influenzae and pneumococcal vaccine
  • Avoid smoke exposure + parents must NOT smoke if baby on home O2
87
Q

Name a Class III gene mutation in CF?

A

G551D

88
Q

When is Ivacaftor helpful in CF?

A

It is a potentiator - it partially increases the channel transport defect allowing chloride through

Lumacaftor and Tezacaftor improve the folding and trafficking to cell surface to increase CFTR

89
Q

What cardiac complications can happen with CF?

A

Right ventricular hypertrophy and pulmonary artery dilatation

90
Q

What liver complications can happen with CF?

A

Hepatic steatosis and portal hypertension

91
Q

What renal complications can happen with CF?

A

Renal stones more likely - calcium based due to abnormal oxalate absorption

92
Q

In a child with poorly managed asthma, what other investigations might be helpful?

A

CF genotyping, alpha-1 antitrypsin phenotyping, FBC, T-Cell subsets, immunoglobulins including IgG subclasses, vaccine antibody response

93
Q

Other medical issues to manage with asthma?

A
  • Monitor for side effects of steroids

- Treat allergic rhinitis (oral/nasal antihistamine, nasal steroids) and treat OSA

94
Q

What medications can help with OSA?

A
  • Intranasal corticosteroids if adenoidal hypertrophy, allergic rhinitis
  • Leukotreine antagonists
95
Q

What allied health staff might be helpful with CPAP use?

A

Play therapy - desensitise from therapy and mask
Nutrition/Dietician - if OSA secondary to obesity
Social work - access to NDIS/ENABLE

+ REFER TO ENT

96
Q

How can you help manage health literacy in families?

A
  • Written information and even informative videos from reputable sources
  • Avoid jargon
  • Use diagrams
  • Utilise CNCs
  • Test understandin
97
Q

Examining an obese child - what should you look for?

A

Acanthosis nigricans, cushingoid features, hypertension

98
Q

In a child with drooling - look for ______

A

perioral dermatitis

99
Q

In a child with OSA/Sleep issues look for _______

A

ENT, BP, RVH , midface hypoplasia?

BPT Clinical exam (3 decks)

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