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pg 42 Flashcards

1
Q

How is inguinal hernia managed?

A

Reduction (Taxis) under anaesthesia then plan surgery when oedema
settles. Prompt surgical repair is needed due to high incarceration risk.

Procedure: Herniotomy (ligation and division of the processus vaginalis)

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2
Q

What differentiate a hydrocele from a hernia?

A

Can get above it

Transilluminate

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3
Q

How does Varicocele present and how is it managed?

A
  • Post-puberty
  • Bag of warms
  • Left-sided
  • TX: Gonadal vein ligation/ embolization if symptomatic
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4
Q

What is the management of undescended testes?
How common are
they?

A 
1:20
Repair around 1 year of age
o Cosmetic
o Fertility
o Malignancy
o Torsion
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5
Q

DDX of the acute scrotum? How is it managed?

A

Testicular torsion: repair quickly
Incarcerated hernia
Epidydimo-orchitis
Appendix testes torsion

Mgt: Emergency testicular exploration and detorsion

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6
Q

indications and contraindications of circumcision?

A

Indications:
o BXO causes true phimosis
o UTIs
o Need for IC in SP

Contraindication:
o Hypospadias.

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7
Q 
What is hypospadias? 
What causes it? 
What are the
associated features? 
And how is it managed?
.
A
  • Opening of urethral meatus on the ventral penile shaft
  • Failure of ventral urethral closure
  • Repair around two years to achieve straight stream and erection
  • Types: Glandular, coronal, Mid-shaft and penoscrotal
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8
Q

What are the genital conditions seen in female infants?

A

Vulvovaginitis due to nappy rash

Labial adhesions

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9
Q

What are the manifestations of hepatic dysfunction?

A
  • Impaired hepatic synthetic function
  • Impaired hepatic detoxification function - encephalopathy
  • Portal hypertension
  • Cholestasis
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10
Q

Impaired hepatic synthetic function Sx

A

abnormal coagulation
bruising
petechial
hypoalbuminaemia

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11
Q

Portal hypertension

A 
Varicies
splenomegaly
hypersplenism
ascites
SBP
hepatorenal syndrome
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12
Q

Cholestasis

A

Pruritus, jaundice, pale stool, dark urine, fat
malabsorption, vitamin deficiency, malnutrition, loss of fat stores and
muscle wasting

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13
Q

main causes of prolonged neonatal jaundice?

A

• Unconjugated (resolves spontaneously):
Breast milk jaundice,
infection, and haemolysis.

• Conjugated > 25:
o Biliary atresia, Choledochal cyst
o Neonatal hepatitis syndrome: Viral, metabolic (A1-antitrypsin,
Galactosemia)
o Intrahepatic biliary hypoplasia: Alagille syndrome
o Choledochal cysts

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14
Q

Biliary atresia TX:

A

Kasai hepatoportoenterostomy => 80% clear jaundice if

performed before 60 days but most develop cholangitis with cirrhosis=> nutritional support and transplantation

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15
Q

Choledochal cysts:

A

Abdominal mass
DX: MRCP
TX: excision and roux-en-Y
anastomosis => Cholangitis and 2% malignancy risk.

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16
Q

Alagille syndrome

A

AD, JAG-1 mutation, Intrahepatic biliary

hypoplasia+ pulmonary stenosis+ triangular faces

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17
Q

A1-Antitrypsin

A

PiZZ, chr.14, + emphysema

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18
Q

Galactosemia

A

Vomiting
hepatomegaly
cataracts
G- sepsis

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19
Q

acute liver failure causes:

A
  • Infection
  • Metabolic
  • Paracetamol
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20
Q

acute liver failure Presentation:

A
  • Encephalopathy
  • Jaundice
  • Bleeding
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21
Q

acute liver failure Diagnosis:

A

• Bloods: Sky-high LFTS, hypoglycaemia, abnormal coagulation, high
bilirubin and ammonia.
• Abnormal EEG
• Cerebral oedema on CT.

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22
Q

acute liver failure Mgt:

A

Transfer to hepatic centre while preventing:
• Hypoglycaemia (Dextrose)
• Infection (BS AB and AF)
• Bleeding (V.K and PPI)
• Oedema (Fluid and salt restriction with Manitol).

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23
Q

What are the causes of chronic liver disease in older children?

A
  • Infection hepatitis B and C
  • Metabolic Wilson, A1ATD, CF
  • Drugs NSAIDS
  • NAFLD
  • Wilson
  • Fibro polycystic (cilopathies):
  • CF
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24
Q

Wilson

A

o Abnormal cerulopalsim production and copper release
o Neuropsychiatric, KF ring, rickets, haemolytic anaemia
o DX: Low serum co and CP, high in urine
o TX: penicillamine or trientene, Zinc, pyridoxine

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25
Q

Fibro polycystic (cilopathies)

A

congenital hepatic fibrosis (bands and
ductules) with normal LFTS+ kidney cysts (Abnormal RFTs and HTN)
=> Combined transplant.

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26
Q

CF

A

Results from steatosis and bile plugging.
TX:
nutritional support, UDCA, transplant (+ liver and lung)

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27
Q

most common malignancies of childhood?

A
  • ALL and lymphomas.

* Brain (younger) => Bone (adolescent)

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28
Q

What are the main malignancies of childhood investigations?

A
  • Radiology

* Pathology (Biopsy, aspiration) => Histology and cytogenetic

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29
Q

malignancies of childhood TX MODALITIES

A

Chemo
Radio
Surgery
Targeted

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30
Q

malignancies of childhood Chemotherapy:

A 
o Primary (ALL)
o Neoadjuvant (soft tissue)
o Adjuvant (others)

SE
Bone marrow suppression (Bleeding, anaemia),
Immunosuppression (infection) ,gut and mucosal damage (Under nutrition), alopecia

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31
Q

malignancies of childhood Radiotherapy:

A 
o EBR
o Proto beam (more targeted)
o IV: MIBG for neuroblastoma
o Damage to surrounding structures and severe neurocognitive
impairment (Intracranial irradiation)
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32
Q

malignancies of childhood Surgery:

A

o Initial biopsy

o Later: remove residual disease

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33
Q

malignancies of childhood Targeted:

A

o Ph+ ALL
o Rituximab
o Anti-GD2 (Neuroblastoma)

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34
Q

Acute Leukaemia Presentation

A

o Bone marrow failure: Anaemia, bleeding, infection, bone pain.
o Reticuloendothelial infiltration: Lymphadenopathy, HSM
o CNS infiltration: Raised ICP and CN palsies
o General: Anorexia, malaise, weight loss

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35
Q

Acute Leukaemia investigations

A 
o FBC
o Clotting screen
o Blood film
o Bone marrow aspirate
o CSF
o CXR
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36
Q

Acute Leukaemia Tx

A

o Remission induction (Transfusion, hydration, allopurinol, Chemo
and steroids)
o Intensification (High dose chemo to maintain remission)
o CNS: Intra-thecal chemo
o Maintenance: 3 years of moderate intensity chemo with
Clotrimazole prophylaxis
o Relapse: High dose chemo, total body irradiation and BMT

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37
Q

Brain tumor types

A

o Supratentorial (cortex): Astrocytoma/ GBM (most common):
Behavioural change
o Midline: Craniopharyngioma: Visual defects and pituitary failure
o Cerebellar: Medulloblastoma: Ataxia
o Brain stem: BS Gliomas (nerve palsies)

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38
Q

Brain tumor Dx + Tx

A

INX: MRI and lumbar puncture

TX: surgery +/- CRT

39
Q

Lymphoma

What are the main types and how do they present?

A

Hodgkin: Adolescents, Painless lymphadenopathy

Non-Hodgkin: T-cell (Mediastinal mass), B-cell (Lymphadenopathy,
abdominal Intussisuption)

Burrkitt: Endemic: Africa, Jaw, EBV. Sporadic: HIV

40
Q

Lymphoma INVX + Tx

A

INVX: CT nodes, biopsy (nodes and marrow), CSF.

Mgt: Combination chemo; curable

41
Q

Wilm’s:

A 
• Nephroblastoma
• Abdominal mass in otherwise well child, does not cross the midline,
Haematuria and hypertension
• DX: US +CT TAP (mets to lungs)
• TX: Surgery.
42
Q

Neuroblastoma:

A

• Neural crest
• Abdominal mass + Mets+ cord compression, crosses the midline
• Dx confirmed by urinary catecholamine and MIBG scan
• TX: Surgery and high dose chemo with stem cell rescue,
Radiosensitive, Anti-GD2, retinoic acid

43
Q

Soft tissue tumors:
Sarcomas:
Bone:

A

Sarcomas: Rhabomyosarcoma of the H+N is the most common

Bone: Osteosarcoma is the most common, Ewing in younger children
(Soft tissue mass, radiosensitive). Male

44
Q

Retinoblastoma

A

AD inheritance with incomplete penetrance, CH.13. Bilateral always
inherited.
Child < 3 yo with white reflex and squint
DX: EUA and MRI
TX: Neoadjuvant chemo and Laser, Radio for relapse and enucleation
for advanced.
PX: Severe visual loss, secondary sarcomas

45
Q

What are Langerhans cells histiocytosis?

A

Rash+ lytic lesions

DI

46
Q

What are the long-term implications of childhood tumors?

A
  • Organ damage
  • Growth and endocrine
  • Neuropsychiatric
  • Educational and social
  • Secondary malignancies
  • Infertility
47
Q

classical triad of diabetes?

A
  • Polydipsia
  • Polyuria
  • Weight loss
48
Q

WHO criteria for diabetes diagnosis?

A

Random > 11.1 mmol/ l or fasting > 7 mmol/L

49
Q

Diabetic children targets

A

Target 4-7 before meals, HBA1C (3-monthly) < 48 or 65%.

Sick day roles: Check ketones 5 times and increase insulin.

50
Q

What are the commonly used types of insulin regimen?

A
  • Basal-bolus: Basal (long acting, Detemir or glargine, at night or morning) + 3 bolus doses before meals (Gluilisine, aspart, lispro).
  • 2 injections (mixed short and intermediate), for simplicity but less flexible.
51
Q

long-term complications of diabetes?

A

Micro vascular: Retinopathy, neuropathy, nephropathy (decreased by
tight glycaemic control, and screened for annually from 12 yo)

Macro vascular: MI, stroke, PVD

52
Q

DKA Clinical features:

A 
o Kaussmal breathing
o Ketotic breath
o Abdominal pain and vomiting
o Dehydration
o Hypovolemic shock
o Altered consciousness => coma and death
53
Q

DKA Diagnosis:

A

o Blood glucose > 11.1
o Blood ketones > 3
o U+E+ C: initial hyperkalaemia and high urea (dehydration)
o ABG < 7.3
o Cardiac monitor (T wave)
o Weight and compare to last visit (dehydration if > 10% wt. loss).
o Identify infection focus (urine and blood cultures).

54
Q

DKA Tx

A

IV fluids
Initial resuscitation: 10 ml/ kg: 0.9% NACL + 40 mmol/L
KCL for first 12 hrs (Add 5% glucose if plasma glucose
below 14). After 12 hrs exchange normal saline with
0.45%.
Maintenance fluids: 100 ml/ Kg (first 10). 50 ml/kg (next
10). 20 mls/ Kg thereafter (subtract initial fluids).
Correct deficit slowly over 48 hours to avoid cerebral
oedema

Monitor: Glucose, ketones hourly. K, PH and electrolytes
2-hourly, fluid balance and neurological status.

Insulin infusion: No bolus. Give after fluids running for 1 hour:
0.1 unit/kg/ hr.

K: Start with fluids, monitor twice hourly and ECG until stable.
o Re-establish (oral fluids, SC insulin and diet): Do not stop IV insulin until 1 hr after SC insulin.

treat cause (infection?)

55
Q

non-diabetic causes of hypoglycaemia?

Do not ever forget glucose in any sick child

A 
Fasting:
High insulin: Exogenous
Normal insulin: 
Ketotic hypoglycaemia of childhood
Glycogen
storage disorder.

Non-fasting:
Galactosemia,
fructose intolerance.

56
Q

congenial hypothyroidism causes

A

Maldescent (most common), Iodine deficiency (most common world-wide), dyshormonogenesis

Detected on heel prick and is preventable cause of severe learning
disability

57
Q

congenial hypothyroidism clinical:

A 
Feeding difficulty
hypotonia
prolonged jaundice
constipation
dry, mottled and cold skin hoarse cry
coarse facial
features
protruding tongue
umbilical hernia
faltering growth
developmental delay
severe learning disability
58
Q

congenial hypothyroidism TX:

A

Thyroxine before 2 weeks of age

59
Q

CAH cause

A

21-hydroxylase deficiency => decreased aldosterone and cortisol and excess sex steroids

60
Q

CAH presents as

A

virilisation in females and salt wasting in 80% of males and

tall stature and precocious puberty in 20%

61
Q

CAH TX:

A

lifelong hormone replacement + steroids before surgery and acute illness,
Surgery for females.
Monitor growth, skeletal maturity, serum androgens and 17-ahydroxyprogesterone.

62
Q

most common cause of Cushing’s syndrome, how can we prevent it?

A

Long-term steroid therapy (in any route).
Prevent by morning doses
and alternate day therapy

63
Q

Steroids SE

A

Growth, behaviour, Hyperglycaemia and hypertension,

osteopenia and muscle wasting, cushioned features, bruising

64
Q

red flags for headache in children?

A

o Worse when lying down or straining
o Wake them up
o EM N&V
o Change in concentration, behaviour, personality

o Eyes: Altered visual fields, squint, papilledema
o Gait, growth, co-ordination and balance
o CN palsies.

65
Q

Seizure ddx

A 
• Epilepsy - 2 or more unprovoked
• Acute symptomatic (Brain insult. E.g. hypoglycaemia)
• Febrile
• Non-epileptic:
o Convulsive syncope (Cardiac, neural, apnoeic)
o Sudden rise in ICP
o Sleep disorders
o Functional
66
Q

What are febrile seizures and how are they managed?

A

Non-epileptic seizures, happens in 3% of 6M-6Y children, with genetic
predisposition

Need infection screen and provide rescue meds (buccal midazolam)

67
Q

Blue breath-holding spells

A

Cry
arrest in expiration
Goes blue, stiff then limp
quickly recover

68
Q

reflex asystolic syncope

A

Pain =>

Pale, collapse, seizure => recover or sleep for 1 hour

69
Q

types of epileptic seizures?

A

Generalised (No warning, LOC> 3 sec, Symmetrical)
Tonic-clonic: Stiff-Jerks (Tongue biting, cyanosis, Incontinence,
Post-ictal fatigue)
Tonic: Stiff
Atonic: Fall
Myoclonic: Jerky.

Focal: Sensory/ motor phenomenon depending on the lobe.

70
Q

epilepsy Dx

A

• Inter-ictal EEG, Ambulatory 24 hr. EEG, Video telemetry
• ECG: Exclude cardiac syncope
• Structural: MRI/ CT:
• Functional: PET/ SPECT:
• Metabolic/ genetic: Only if intractable epilepsy with developmental
arrest/ delay/ regression

71
Q

common types of epilepsy syndromes?

A 
West syndrome
Lennox-Gestaut syndrome (LGS)
Panayiotopoulos syndrome
Benign Rolandic epilepsy
Juvenile abcense epilepsy
Juvenile myoclonic epilepsy
72
Q

West syndrome

A

o Infants
o Clustered spasms
o Hypsarrhythmia
o Poor prognosis

73
Q

Lennox-Gestaut syndrome (LGS)

A

o Toddlers
o Multiple seizure types
o Slow Generalised spike and wave (1-3 HZ)
o Poor prognosis

74
Q

Panayiotopoulos syndrome

Early onset benign occipital epilepsy

A

o Pre-schoolers
o Unresponsive stare in sleep+head and eye deviation+ vomiting
o Focal occipital waves and discharges when eyes shut
o Remits in childhood

75
Q

Benign Rolandic epilepsy (Benign epilepsy with centro-temporal spikes)

A 
o Primary school
o TCS in sleep, abnormal mouth sensations, gurgling sounds and
face distortions.
o Focal spikes in the rolandic area.
o Remits in Adolescence
o 15% of childhood epilepsies
o May not require AED
76
Q

Juvenile abcense epilepsy

A

o 10-20 years
o Lapses of unresponsive stare and motor arrest, last 30 secs,
can be brought by hyperventilation
o Fast Generalised spike and wave (304 hz)
o Lifelong AED. Vs. Chldhood onset: 80% remission

77
Q

Juvenile myoclonic epilepsy

A

o 10-20 years
o Myoclonic seizures (dropped the bowel of cereal in the morning)
+/- TCS, absence. Triggered by lack of sleep and alcohol
o EEG: Genralised spike and wave form
o Good response to TX but remission unlikely

78
Q

What anti-epileptics drugs are used for seizures

A
  • Generalised: Valproate => Clobazam
  • Focal: Carbamazepine, leveteriacetam => Clobazam
  • Avoid Carbamazepine in Juvenile epilepsies
79
Q

central movement

disorders features

A 
o Floppiness/ hypotonia
o Difficulty feeding and breathing
o Delayed Motor Milestones
o Muscle weakness
o Unsteady/ abnormal gait (Waddling in proximal myopathy)
o Fatigability
o Cramps
80
Q

central movement

disorders categories

A

o Anterior horn: LMN
o Nerve: Distal weakness, altered sensation
o NMJ: Fatigability
o Myopathy: Proximal weakness, Gower’s sign
o Myositis: Pain
o Metabolic myopathy: Cramps

81
Q

peripheral movement disorders

A

Anterior horn:
o Polio
o Spinal muscular atrophy (Werding-hoffman disease)

Peripheral nerve:
Hereditary Motor sensory neuropathies (CMT)
Guillain–Barré syndrome (acute post-infectious
polyneuropathy)
Duchenne Muscular dystrophy

82
Q

Spinal muscular atrophy (Werding-hoffman disease)

A

Type 1 is the worst

Child never sit and dies by 1 year from resp failure

83
Q

Hereditary Motor sensory neuropathies (CMT):

A

Genetics: AD CMT1A gene => Abnormal myelin

Sx
o Progressive symmetrical distal muscle
wasting
o Bilateral foot drop => Tripping over
o Pes Cavus
o Initial presentation similar to Friedreich ataxia

Dx: Nerve conductions studies + Nerve biopsy
(Onion bulb formation)

84
Q

Guillain–Barré syndrome (acute post-infectious

polyneuropathy)

A

Sx
o 2–3 weeks after an upper respiratory tract
infection or campylobacter gastroenteritis
o Ascending neuropathy
o Difficulty chewing and swallowing with risk
of respiration
o Dysautonomia: Urinary retention, ileus and
loss of sweating

Dx
MRI to exclude SC lesion
CSF - High protein but normal WBC
Nerve conduction slow

Tx:
IVIG or plasma exchange

85
Q

Duchenne Muscular dystrophy genetics

A 
• X-linked recessive
• Dystrophin gene deletion
• Myofibril necrosis
• The plasma creatine kinase (CK) is markedly
elevated.
86
Q

Duchenne Muscular dystrophy features

A

• Waddling gait
• They have to mount stairs one by one and run
slowly compared with their peers.
• Gowers sign (the need to turn prone to rise).
• Pseudohypertrophy of the calves
• In the early school years, affected boys tend to be
slower and clumsier than their peers
• Learning difficulties. Scoliosis is a common
complication
• Language/ Motor delay;

87
Q

Duchenne Muscular dystrophy mgt

A

• Prevent contractures (physio and splints)
• Scoliosis surgery
• Nocturnal hypoxia (weak intercostal muscles) =>
Headache, irritability, sleepiness, reduced appetite
=> Overnight CPAP
• Ambulant children receive steroids to preserve
mobility and prevent scoliosis
• Ataluren: for non-sense (stop mutation): Bypass
the mutation and produce some Dystrophin.

88
Q

DDX in a floppy infant?

A
  • Central: HIE, Syndromes
  • SC: Birth trauma, SMA
  • NMJ: MG
  • Peripheral nerves: CMT
  • Muscle: Dystrophies, metabolic myopathies
89
Q

Frederick ataxia:
Can not see, speak, eat sweet
Sx:

Signs:

A 
Progressive ataxia
Dysarthria
Diabetes
Cardiomyopathy
Optic atrophy
Distal wasting
Pes cavus
Kiphoscoliosis
Absent reflexes
No vibration sense or proprioception
Positive Rhomberg sign
90
Q

Frederick ataxia genetics:

A

AR triplet repeat expansion of Frataxin gene = absent

frataxin protein

91
Q

Meningomyelocele:

A

o Closed at birth
o Hydrocephalus (Chiari 2) => P shunt
o Lower limb paresis
o Loss of sensation => skin damage
o Neuropathic bladder => Intermittent catheterisation
o Neuropathic bowel => Bowel washouts, special diet
o Hip dislocation, Talipes and scoliosis => Surgery
o Regular checks for renal function, hypertension and UTIs=>
Prophylactic antibiotics and oxybutynin

92
Q

NFM Dx 2/6 criteria

A

Skin:
Six or more café-au-lait spots greater than 5 mm in size
before puberty, greater than 15 mm after puberty
More than one neurofibroma
Axillary freckling

Eye:
Optic glioma which may cause visual impairment
One leish nodule: Hamartoma of the Iris seen on slit lamp
examination

Bone:
Lesions from sphenoid dysplasia => Eye protrusion or
fractures
FHX - FDR with NFM1

93
Q

Other clinical features:

A 
Brain:
Learning and behavioural difficulties
Epilepsy
personality changes
weakness on one side of the body
difficulties with balance and co-ordination
o Hypertension and auditory
o Physical development:
Abnormal growth
Scoliosis
o Malignancy:
Change in a neurofibroma

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