2019 remembered Flashcards

Question 1

Q

1.	What is the target antigen of the influenza vaccine?
A.	Neuraminidase
B.	Polysaccharide capsule
C.	Factor F
D.	Haemagglutinins

Answer

A

D. Haemagglutinins

Question 2

Q

2.	You are seeing a child with CAPS. What is the target for the treatment?
A.	IL-1
B.	IL-2
C.	TNFa-receptor
D.	INFy-receptor

Question 3

Q

3.	What is the defect in class 1 CFTR mutation?
A.	No production of CFTR
B.	Reduced trafficking of CFTR
C.	Reduced production of CFTR
D.	Reduced function of CFTR

Answer

A

A. No production of CFTR

Question 4

Q

4.	Ivicaftor is a new drug for CFTR. Which class of mutation does it target?
A.	Class 1
B.	Class 2
C.	Class 3
D.	Class 4

Answer

A

C. Class 3

Question 5

Q

5.	Goodpasture disease is an example of what type of hypersensitivity
A.	Antibody dependent cytotoxic killing
B.	Immune complex mediated
C.	Delayed hypersensitivity
D.	Immediate hypersensitivity

Answer

A

A. Antibody dependent cytotoxic killing

Question 6

Q

6.	What is the protein target of ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif)?
A.	Neutrophil elastase
B.	Myosin
C.	Melanocytes
D.	Von Willebrand factor

Answer

A

D. Von Willebrand factor

Question 7

Q

7.	A girl got her ears pierced. 12 hours later she notices them become itchy and red. What type of hypersensitivity is this an example of?
A.	Antibody dependent cytotoxic killing
B.	Immune complex mediated
C.	Delayed hypersensitivity
D.	Immediate hypersensitivity

Answer

A

C. Delayed hypersensitivity

Question 8

Q

8.	Which phase of cardiac contraction does sodium enter the cell? (The actual question also had a ECG aligned with this – but I just looked at the action potential)
A.	0
B.	2
C.	3
D.	4

Question 9

Q

During which phase does isovolumetric relaxation occur?

A. 3
B. 4
C. 5
D. 6

Question 10

Q

How does glucagon-like peptide-1 (GLP-1) contribute to glucose metabolism?
A. Promotes gastric emptying
B. Increases gluconeogenesis
C. Increases glycogen release from muscles
D. Increases insulin release

Answer

A

D. Increases insulin release

Question 11

Q

What type of receptor is the insulin receptor?
A. Transmembrane G-protein coupled
B. Nuclear receptor
C. Transmembrane receptor linked to an enzyme
D. Transmebrane receptor linked to an ion channel

Answer

A

C. Transmembrane receptor linked to an enzyme

Question 12

Q

12.	Loss of which protein results in the procoagulant state in nephrotic syndrome
A.	Protein C
B.	Antithrombin III
C.	Factor VIII
D.	Factor V Leiden

Answer

A

B. Antithrombin III

Question 13

Q

13.	Tranexamic acid works by directly inhibiting the action of which substance
A.	Plasminogen
B.	Fibrinogen
C.	Thrombomodulin
D.	Factor V

Answer

A

A. Plasminogen

Question 14

Q

14.	Patients with conversion disorder are most likely to have what type of affect?
A.	Anxious
B.	Apathetic
C.	Blunted
D.	Irritable

Answer

A

A. Anxious

Question 15

Q

15.	Which paediatric cancer is the best example of Knudson’s two-hit hypothesis?
A.	Neuroblastoma
B.	Retinoblastoma
C.	Medullary thyroid cancer
D.	Burkitt lymphoma

Answer

A

B. Retinoblastoma

Question 16

Q

What is the mechanism of action of methotrexate?
A. Competitive inhibition of dihydrofolate reductase
B. Inhibition of microtubule formation
C. Inhibition of DNA intercalation
D. Topoisomerase inhibition

Answer

A

A. Competitive inhibition of dihydrofolate reductase

Question 17

Q

17.	You see a child with a likely UTI and want to prescribe antibiotics. He has a history of G6PD. Which drug should you avoid?
A.	Erythromycin
B.	Cephalexin
C.	Amoxicillin
D.	Trimethoprim-sulfamethoxazole

Answer

A

D. Trimethoprim-sulfamethoxazole

Question 18

Q

What is the mechanism of chronic haemolysis in pyruvate kinase deficiency?
A. Lack of resistance to oxidative stress
B. Reduced production of ATP
C. Production of protoporphyrin
D. Impaired cytoskeleton formation

Answer

A

B. Reduced production of ATP

Question 19

Q

19.	Infant mortality is measure as the number of deaths under 1 year of age per
A.	1000 births
B.	1000 live births
C.	100 live births
D.	10000 births

Answer

A

B. 1000 live births

Question 20

Q

20.	How often should adrenaline be given when performing CPR
A.	Every 2 minutes
B.	Every 3 minutes
C.	Every 4 minutes
D.	Every 6 minutes

Answer

A

C. Every 4 minutes

Question 21

Q

21.	What inheritance is most in keeping with this pedigree? (You do not know the sex of the miscarriages).
A.	Autosomal dominant
B.	Mitochondrial
C.	X-linked dominant
D.	Imprinted

Answer

A

C. X-linked dominant

Question 22

Q

22.	Which of these drugs is most likely to cause fulminant liver failure in a patient with a POLG mutation?
A.	Sodium valproate
B.	Carbamazepine
C.	Phenytoin
D.	Leviteracetam

Answer

A

A. Sodium valproate

Question 23

Q

23.	Which of these drugs would decrease the level of levonorgestrel implant?
A.	Lamotrigine
B.	Sodium valproate
C.	Carbamazepine
D.	Levetiracetam

Answer

A

C. Carbamazepine

Question 24

Q

24.	A baby suffers severe respiratory distress at birth requiring intubation and ventilation. It improves briefly following surfactant administration but deteriorates and dies. Deficiency of which surfactant protein was most likely the cause?
A.	SP-A
B.	SP-B
C.	SP-C
D.	SP-D

Question 25

Q

Which of these is a necessary criterion for the diagnosis of autism spectrum disorder?
A. Intellectual impairment
B. Poor receptive language ability
C. Restrictive and repetitive patterns of behaviour, interests or activities
D. Obsessive traits

Answer

A

C. Restrictive and repetitive patterns of behaviour, interests or activities

Question 26

Q

26.	In Burkitt’s lymphoma what is expressed by most cells that can used as a target for chemotherapy?
A.	CD3
B.	CD20
C.	CD56
D.	CD22

Question 27

Q

27.	Which of these drugs used for ADHD is most likely to cause suicidal ideation?
A.	Atomoxetine 
B.	Methylphenidate
C.	Clonidine
D.	Dexamphetamine

Answer

A

A. Atomoxetine

Question 28

Q

28.	Co-morbid diagnoses are common in ADHD. Which of these occurs most frequently?
A.	Anxiety
B.	Depression
C.	ODD
D.	Anti-social personality disorder

Question 29

Q

29.	What is the most common cause of ischaemic arterial stroke in children
A.	Arteriopathy
B.	Metabolic
C.	Cardiac embolism
D.	Hypovolaemic

Answer

A

A. Arteriopathy

Question 30

Q

What is the most likely phenotype in a XY patient with a mutation of steroidogenic acute regulating protein (StAR)?
A. Normal female external genitalia with testes palpable in the labia
B. Normal male external genitalia with undescended testes
C. Normal male genitalia with testes in the scrotum
D. Bifid scrotum, severe hypospadias, testes in inguinal canal

Answer

A

A. Normal female external genitalia with testes palpable in the labia

Question 31

Q

A 12yo girl is being investigated for new onset cough and dyspnoea. What is the most likely results given her lung function tests?
Pre-bronchodilator Post-bronchodilator
Observed %Predicted Predicted Observed %Predicted %Change
FVC 3.323 75% 4.43 3.40 88% /1%
FEV1 2.791 78% 3.57 2.80 78%/0%
FEV1/FVC 84 104% 80

A. Obstructive pattern with bronchodilator response
B. Restrictive pattern
C. Obstructive pattern without bronchodilator response
D. Upper airways disease

Answer

A

?B. Restrictive pattern

Question 32

Q

32.	Which of these enzymes is low or normal in progressive familial intrahepatic cholestasis type 1 and 2?
A.	Alkaline phosphatase
B.	Aspartate aminotransferase
C.	Alanine aminotransferase
D.	Gamma-glutamyl transferase

Answer

A

D. Gamma-glutamyl transferase

Question 33

Q

33.	Infiltrate of which cells is seen on liver biopsy of a patient with autoimmune hepatitis?
A.	Monocytes
B.	Neutrophils
C.	Plasma cells
D.	Eosinophils

Answer

A

C. Plasma cells

Question 34

Q

34.	Which of these disorders is characterised by deficiency of melanocyte migration?
A.	Waardenberg syndrome
B.	Oculocutaneous albinism
C.	McCune Albright
D.	Hypomelanosis of Ito

Answer

A

B. Oculocutaneous albinism

Question 35

Q

35.	Why do you get varied manifestations in different tissues in McCune-Albright syndrome?
A.	Somatic mosaicism
B.	Lyonisation
C.	Uniparental disomy
D.	Chromosomal fragility

Answer

A

A. Somatic mosaicism

Question 36

Q

36.	What is the target of nusinersen in SMA?
A.	SMN1
B.	SMN2
C.	Myelin
D.	Nerve terminals

Question 37

Q

37.	Following infection, EBV establishes latency in B cells. In which other cell does it also establish latency?
A.	T-cells
B.	Epithelial cells
C.	Dendritic cells
D.	Neutrophils

Answer

A

B. Epithelial cells

Question 38

Q

38.	A researcher discovers that the countries that have highest rates of ice-cream sales also have the highest levels of drownings. They conclude that ice-cream must cause drowning. This is an example of what type of bias?
A.	Anchoring
B.	Ecological bias
C.	Interpretative
D.	Recall

Answer

A

C. Interpretative

Question 39

Q

39.	What is the purpose of blinding the clinicians in a double-blinded RCT
A.	To minimise allocation bias
B.	To control for placebo affect
C.	To avoid bias is evaluating outcomes
D.	Reduce confounding

Answer

A

C. To avoid bias is evaluating outcomes

Question 40

Q

40.	On the NNST, what is used to detect neonatal hypothyroidism?
A.	T4
B.	TSH
C.	TRH
D.	Anti-TSH antibodies

Question 41

Q

41.	Which infection is most likely to trigger toxic epidermal necrolysis?
A.	Staphylococcal aureus
B.	Mycoplasma pneumoniae
C.	Salmonella typhi
D.	Streptococcus pneumoniae

Answer

A

B. Mycoplasma pneumoniae

Question 42

Q

42.	What percentage of sodium is reabsorbed in the ascending loop of Henle
A.	95%
B.	25%
C.	5%
D.	<1%

Question 43

Q

43.	Which cells in the kidney are responsible for production of erythropoietin
A.	Glomerular mesangial cells
B.	Afferent artierole endothelia cells
C.	Podocytes
D.	Peritubular interstitial cells

Answer

A

D. Peritubular interstitial cells

Question 44

Q

44.	At what age do most children achieve object permanence?
A.	6 months
B.	9 months
C.	12 months
D.	15 months

Answer

A

B. 9 months

Question 45

Q

45.	What is the initial acid-base disturbance in salicylate poisoning
A.	Respiratory acidosis
B.	Respiratory alkalosis
C.	Metabolic acidosis
D.	Metabolic alkalosis

Answer

A

B. Respiratory alkalosis

Question 46

Q

46.	What is the interpretation of this blood gas?
pH – 7.3
CO2 – 85
HCO3 – 30 
A.	Metabolic acidosis
B.	Compensated respiratory acidosis
C.	Respiratory alkalosis
D.	Compensated metabolic alkalosis

Answer

A

B. Compensated respiratory acidosis

Question 47

Q

47.	Which of these autoantibodies likely to positive in Wegener’s?
A.	ANCA
B.	ANA
C.	dsDNA
D.	Anti-Ro

Question 48

Q

48.	What is the dominant effect of parathyroid hormone on phosphate homeostasis?
A.	Increased gastric absorption
B.	Decreased bone demineralisation
C.	Decreased renal reabsorption
D.	Decreased gastric absorption

Answer

A

C. Decreased renal reabsorption

Question 49

Q

49.	What is the main determinant of CO2 level in a neonate
A.	Tidal volume
B.	Minute volume
C.	Vital capacity 
D.	Lung resistance

Answer

A

B. Minute volume

Question 50

Q

50.	Which enzyme catalyses the reaction which results in HCO3 absorption in the kidney?
A.	Alkaline phosphatase
B.	Carbonic anhydrase
C.	D-amino acid oxidase
D.	HCO3 convertase

Answer

A

B. Carbonic anhydrase

Question 51

Q

51.	A child presents with salmonella gastroenteritis. Which pet is most high risk for transmitting salmonella?
A.	Bird
B.	Dog
C.	Rabbit
D.	Turtle

Answer

A

D. Turtle

Question 52

Q

52.	In hepatitis B, development of which antibody is suggestive of decreased infectivity?
A.	Hepatitis B surface antibody
B.	Hepatitis B core antibody 
C.	Hepatitis B envelope antibody
D.	Hepatitis B surface antigen

Answer

A

C. Hepatitis B envelope antibody

Question 53

Q

53.	Child has recurrent Neisseria infections. Her initial investigations demonstrate a low CH50. What is the next diagnostic step to confirm complement deficiency as the cause of her recurrent infections?
A.	AH50 
B.	C3
C.	C2
D.	C1q

Question 54

Q

54.	Which of these inotropes is the least vasoactive?
A.	Milrinone
B.	Dopamine
C.	Dobutamine
D.	Noradrenaline

Answer

A

C. Dobutamine

Question 55

Q

55.	Which of these induction agents has the most cardiovascular stability?
A.	Ketamine
B.	Propofol
C.	Fentanyl
D.	Thiopental

Answer

A

A. Ketamine

Question 56

Q

56.	You see a patient who has been assessed to have a mild intellectual disability. Which IQ would be in keeping with mild intellectual impairment?
A.	45
B.	55
C.	65
D.	75

Question 57

Q

57.	What is the most common genetic cause of Prader-Willi syndrome?
A.	Microdeletion
B.	Uniparental disomy
C.	Duplication
D.	Unbalanced translocation

Answer

A

A. Microdeletion

Question 58

Q

What is the mechanism of paralysis following tick bite?
A. Decreased neurotransmitter release
B. Post-synaptic neurotransmitter blockade
C. Loss of myelin
D. Neuronal necrosis

Answer

A

A. Decreased neurotransmitter releas

Question 59

Q

59.	What is the most common cause of respiratory depression in paediatric trauma?
A.	Head injury 
B.	Pneumothorax
C.	Upper airway obstruction
D.	Pulmonary haemorrhage

Answer

A

?A. Head injury

Question 60

Q

60.	Galactorrhoea may occur in children being treated with risperidone. What action of risperidone is responsible for this?
A.	Dopamine antagonism
B.	Serotonin antagonism
C.	Alpha 1 adrenergic antagonism
D.	Histamine H1 antagonism

Answer

A

A. Dopamine antagonism

Question 61

Q

61.	Clonidine is used in paediatrics for a variety of conditions. What is the main mechanism of action?
A.	Serotonin antagonist
B.	Noradrenaline re-uptake inhibitor
C.	Dopamine antagonist
D.	Alpha adrenergic agonist

Answer

A

D. Alpha adrenergic agonist

Question 62

Q

62.	What is most common HLA type in coeliac disease
A.	HLA-DQ2
B.	HLA-DR8
C.	HLA-B27
D.	HLA-DS2

Answer

A

A. HLA-DQ2

Question 63

Q

AV node conduction

A.	P wave
B.	PR interval
C.	Q wave
D.	QRS duration
E.	QT interval
F.	ST segment
G.	T wave
H.	RR interval

Answer

A

B. PR interval

Question 64

Q

refractory period

A.	P wave
B.	PR interval
C.	Q wave
D.	QRS duration
E.	QT interval
F.	ST segment
G.	T wave
H.	RR interval

Answer

A

F. ST segment

Answer 53

A

?C. Acute liver failure

Answer 54

A

F. Acute hepatitis

Answer 55

A

D

odds ratio = AD/BC
= 30x10 / 20x40 = 300/800

Answer 56

A

D

odds ratio = AD/BC
= 45/5 = 9

Answer 57

A

Tumor Necrosis Factor Receptor Associated Periodic Syndrome (TRAPS) is a rare, genetic disease that causes recurrent episodes of fever that typically last more than one week and are associated with chills and severe muscle pain in the torso and the arms.

FMR - <3 days; recurrent episodes of fever that are typically accompanied by pain in the abdomen, chest, or joints. It most often occurs in individuals of Mediterranean and Middle Eastern descent, and the first episodes typically begin in childhood.

Hyper-IgD syndrome is a rare autosomal recessive disorder in which recurring attacks of chills and fever begin during the first year of life. Episodes usually last 4 to 6 days and may be triggered by physiologic stress, such as vaccination or minor trauma.

Deficiency of interleukin-1 receptor antagonist (DIRA) is a rare life-threatening autoinflammatory disease caused by autosomal recessive mutations in IL1RN. DIRA presents clinically with early onset generalized pustulosis, multifocal osteomyelitis, and elevation of acute phase reactants.

Answer 58

A

C. RAG 1-2, Omenn syndrome

All diff forms of SCID

CD56 = NK cells

T-, B+, NK+: IL7R, CORO IA, CD3D, E & Z
T-, B+, NK -: JAK3, IL2RG
T -. B-, NK +: RAG 1-2. DCLREIC,
T-, B-, NK -: ADA, AK2

Answer 59

A

C. 25%

Most cases of Down’s syndrome result from free trisomy of chromosome 21, but in about 5% of Down’s syndrome cases the abnormality is a Robertsonian translocation.1 Translocation type Down’s syndrome may either be de novo or inherited from a balanced carrier parent. Theoretically a balanced 21 Robertsonian translocation carrier person (13/21, 14/21, 15/21, 22/21) has a 25% risk of having Down’s syndrome offspring.

Answer 60

A

C. Macrophage activation syndrome

A febrile patient with known or suspected sJia must be considered for macrophage activation if:

Ferritin >684 ng/ml

and any 2 of the following:

Hemoglobin <90g/L (in infants <4 weeks: <100g/L)

Platelets <100 x 109/L

Neutrophils <1.0 x 109/L

Fasting triglycerides ≥3.0 mmol/L (i.e., ≥ 265mg/dl)

Fibrinogen ≤1.5 g/L

Answer 61

A

A. asleep

Landau Kleffner syndrome (LKS) is a rare childhood disorder characterized by the loss of language comprehension (auditory verbal agnosia) and verbal expression (aphasia) in association with severely abnormal electroencephalographic (EEG) findings during sleep and clinical seizures in most patients.

EEG - Bilateral independent temporal or temporoparietal spikes, bilateral 1–3 Hz slow-wave maximally temporal activity, generalized sharp- or slow-wave discharges, and multifocal or unilateral spikes are described

The EEG is characterized by slow spike-wave discharges that are of higher amplitude over the temporal regions and spread diffusely through both hemispheres, giving the appearance of a generalized discharge. A striking EEG abnormality often occurs during sleep, also called “continuous spikes and waves during slow sleep” or “electrical status epilepticus during slow-wave sleep.”

Answer 62

A

C. Loey-Dietz

Marfan
the incidence (35-80%) of aortic root dilatation

Loeys-Dietz syndrome

resembles Marfan syndrome, which is also inherited otosomal dominant, aortic aneurysm
vascular pathology is more probable than in Marfan patients.
differentiated from Marfan syndrome by palatal involvement and hyperteleroism
an aggressive aortic aneurysm syndrome that can be addressed by prophylactic aortic root replacement with low operative risk

In Ehler Danlos type IV collagen type III α-1 gene mutation leads to abnormal type III collagen in the vascular wall, skin and other organs. These patients carry aortic dissection risk and it does commonly occur after the 3rd decade. Aortic aneurysm can be seen in Ehler-Danlos type IV patients, but dissection is a rare condition in these patients

Turner syndrome is an aneuploidy syndrome [45, XO) characterized by short stature, webbed neck, and infertility. Bicuspid aortic valve, coarctation of the aorta, aortic dilatation, pseudocoarctation, aortic aneurysm, and dissection are common in Turner patients.
The prevalence of aortic dilation and aneurysm is lower in the young girls and women with Turner syndrome than in older Turner syndrome population

Answer 63

A

C. Low temp

Multiple logistic regression analysis revealed that younger age at onset of first seizure, lower temperature during the seizure, brief duration between the onset of fever and the initial seizure, and family history of febrile seizures were risk factors significantly associated with recurrence of febrile seizures in children.

Answer 64

A

B. Partial seizure with Todd’s paresis

Focal weakness in a part or all of the body after a seizure. This weakness typically affects appendages and is localized to either the left or right side of the body. It usually subsides completely within 48 hours. Todd’s paresis may also affect speech, eye position (gaze), or vision.

The cause of Todd’s paresis has been attributed to the affected cortex being ‘exhausted’ or silenced due to increased inhibition, but these conjectures are not supported. It has been observed that the impairments that follow seizures are similar to those that follow strokes, where for a period of time blood flow to certain areas of the brain is restricted and these areas are starved of oxygen.

Answer 65

A

D. Triglycerides

Answer 66

A

C. zinc

Acrodermatitis enteropathica is an inherited deficiency of the zinc carrier protein ZIP4 resulting in inadequate zinc absorption. It presents as growth retardation, severe diarrhea, hair loss, skin rash (most often around the genitalia and mouth) and opportunistic candidiasis and bacterial infections.

Answer 67

A

C. Anterior uveitis

Answer 68

A

B. Hypothyroidism

Answer 69

A

A. ARPKD

Autosomal recessive polycystic renal disease is a hereditary condition characterized by renal cystic disease and hepatic fibrosis in varying degrees of severity. Neonatal patients have prominent renal enlargement with renal failure and minimal liver dysfunction. Approximately 30%–50% of affected infants die during the perinatal period owing to pulmonary hypoplasia and pulmonary insufficiency.

At US, the kidneys are massively enlarged and diffusely echogenic bilaterally (,,,,Figs 7, ,,,8). Corticomedullary differentiation is absent. High-resolution US (linear-array transducer, 7.5 mHz or greater) allows visualization of numerous cylindrical cysts in the medulla and cortex, which represent ectatic collecting ducts.

Multicystic dysplastic kidney

the second most common cause of an abdominal mass in the neonate, after hydronephrosis. The condition is thought to be caused by severe ureteral obstruction early during gestation.
most often occurs unilaterally and does not affect renal function unless the contralateral kidney has other associated anomalies.
Common associated abnormalities include obstruction of the ureteropelvic junction (10%–20% of cases) or reflux nephropathy (30%–40%) (,13,,14).
US appearance of multicystic dysplastic kidney is a mass of noncommunicating cysts of variable size. Unlike severe hydronephrosis, in which the largest cystic structure (the renal pelvis) lies in a central location and is surrounded by dilated calices, in multicystic dysplastic kidney the cyst distribution shows no recognizable pattern. Scant dysplastic, echogenic parenchyma may be visible between the cysts, but no normal renal parenchyma is seen

Answer 70

A

C. Non-compliance

Answer 71

A

rare inherited disease characterised by a defect in the thick ascending limb of the loop of Henle, which results in low potassium levels (hypokalemia),[2] increased blood pH (alkalosis), and normal to low blood pressure.

Neonatal:

90% of cases seen between 24 and 30 weeks of gestation with polyhydramnios
After birth, the infant has polyuria, and polydipsia .Life-threatening dehydration may result if the infant does not receive adequate fluids.
About 85% of infants have hypercalciuria and nephrocalcinosis

Classic:
- may have symptoms in the first two years of life, but they are usually diagnosed at school age or later
- also have polyuria, polydipsia, and a tendency to dehydration, but normal or just slightly increased urinary calcium excretion without the tendency to develop kidney stones.
- vomiting and growth retardation
Kidney function is also normal if the disease is treated, but occasionally proceed to end-stage kidney failure
- low levels of potassium in the blood, alkalosis, normal to low blood pressures, and elevated plasma renin and aldosterone

Diagnostic pointers include high urinary potassium and chloride despite low serum values, increased plasma renin, hyperplasia of the juxtaglomerular apparatus on kidney biopsy, and careful exclusion of diuretic abuse. Magnesium wasting may also occur
- present symptoms that are identical to those of patients who are on loop diuretics like furosemide, given that the loop diuretics target the exact transport protein that is defective in the syndrome (at least for type 1 Bartter syndrome).

Mx: Medically supervised sodium, chloride and potassium supplementation is necessary, and spironolactone can be also used to reduce potassium loss.[2] Free and unqualified access to water is necessary to prevent dehydration, as patients maintain an appropriate thirst response.

Answer 72

A

C. Posterior urethral valves

PUV is the most common cause of lower urinary tract obstruction in male neonates.
Usually diagnosed before birth or at birth when a boy is evaluated for antenatal hydronephrosis.

Every child with antenatal hydronephrosis should be assessed with renal and bladder ultrasonography (US) in the immediate postnatal period, [14] with a focus on the appearance of the renal parenchyma, any evidence of renal collecting system dilatation, the thickness of the bladder wall, and the presence or absence of ascites.

VCUG should be performed under fluoroscopy, with imaging of the posterior urethra, especially during the voiding phase. Cycling the bladder during the study several times improves the sensitivity of the evaluation.

The diagnosis of PUV is indicated by visualization of the valve leaflets. Other clues to the diagnosis are a thickened trabeculated bladder, a dilated or elongated posterior urethra, and a hypertrophied bladder neck

Answer 73

A

D. Interpretative

Answer 74

A

C. Discharge with reassurance

Patients requiring assessment
Acute ingestion of >200mg/kg
Ingestion of unknown quantity
Repeated supratherapeutic ingestion of >100mg/kg/day

Pediatric liquid paracetamol ingestion
In children under 6 years who have ingested > 200mg/kg a serum paracetamol level should be taken 4 hours after the time of ingestion. A standard two bag acetylcysteine infusion should be commenced if the level is > 160mg/L (1000µmol/L).

Answer 75

A

A. TPN peritonitis
?abdo distension only

passing stool, no mention of blood. no bilious aspirates
no enteral feeds

Intraperitoneal extravasation of (TPN) has been cited as one of the complications associated with malpositioned UVC, which may lead to vessel perforation or liver capsule disruption as a result of hepatic
necrosis.

Answer 76

A

A. Hemarthrosis

Haemophilia is an X-linked bleeding disorder affecting 1 in 6,000-10,000 males and less than 1 in 300,000 females.
Haemophilia A is clotting Factor VIII (8) deficiency.

Severe: spontaenous bleeding into joint

The presence of a new inhibitor should be suspected in any child who fails to respond clinically to adequate factor replacement, particularly if the child has been previously responsive.
If the child has factor inhibitors (proven or suspected), a special product is required to ’bypass’ the inhibitor to initiate clotting. i.e. A bypassing agent

Answer 77

A

B. 4 months

The half-life of IVIG is 25 - 32 days in immunodeficient patients.

5 half lives ~5 months

Answer 78

A

B. 4 months

The half-life of IVIG is 25 - 32 days in immunodeficient patients.

5 half lives ~5 months

Answer 79

A

Neonatal lupus erythematosus is the occurrence of systemic lupus erythematosus (SLE) symptoms in an infant born from a mother with SLE, most commonly presenting with a rash resembling subacute cutaneous lupus erythematosus, and sometimes with systemic abnormalities such as complete heart block or hepatosplenomegaly.[1]

The infants have no skin lesions at birth, but sometimes develop them during the first weeks of life.[2] Neonatal lupus is usually benign and self-limited.[1]

The most serious complication of neonatal lupus is a heart condition known as congenital heart block. If the block is complete it can potentially be life-threatening and requires a pacemaker.

It is associated with mothers who carry the Anti-SSA/Ro antibodies, which is associated most closely with the subacute cutaneous lupus erythematosus form of the disease.

Answer 80

A

Human parechovirus (HPeV) is increasingly being recognized as a potentially severe viral infection in neonates and young infants. 
HPeV-1 is the most prevalent genotype and most commonly causes gastrointestinal and respiratory disease. 
HPeV-3 is clinically the most important genotype due to its association with severe disease in younger infants, which may partly be explained by its distinct virological properties. In young infants, the typical clinical presentation includes fever, severe irritability, and rash - a sepsis-like picture. Infants with severe CNS infections are at an increased risk of long-term sequelae.

Children under the age of 2 years are at the greatest risk of developing symptomatic HPeV infection. Generally, HPeV-1 is the predominant cause of disease, followed by types 3 and 6, respectively.

The majority of severe HPeV infections are caused by HPeV-3 and present in infants <3 months of age as asepsis, sepsis-like illness, or CNS infection. When HPeV is known to be circulating in the community, presentations of “hot, red, angry babies” with features of sepsis should make clinicians consider HPeV infection. During an HPeV-3 outbreak in Australia, the recognition of this triad of fever, rash, and severe irritability allowed the rapid and accurate identification of infected infants.

Seizures are a common presentation of HPeV infections of the CNS, with one study describing seizures in 90% of HPeV-infected infants with CNS involvement.

More recent attention has been focused on the development of white matter damage in young infants (11, 110–112). HPeV white matter lesions are indistinguishable from those reported for enterovirus infections and hypoxic-ischemic encephalopathy and vary from diffuse signal intensity changes and punctate white matter lesions to cysts within the white matter.

A nationwide study from Australia on HPeV encephalitis reported an ICU admission rate of 89% and invasive mechanical ventilation in 56% of the patients and showed that infants with HPeV encephalitis have a high risk of long-term complications related to the extent of white matter abnormalities. At discharge, three out of nine patients were described to have neurodevelopmental sequelae, but after 12 months of follow-up, this increased to five out of eight patients, two of whom were diagnosed with cerebral palsy and one of whom had central visual impairment (11

Answer 81

A

Congenital diaphragmatic hernia (CDH) is a birth defect of the diaphragm. The most common type of CDH is a Bochdalek hernia; other types include Morgagni hernia, diaphragm eventration and central tendon defects of the diaphragm. Malformation of the diaphragm allows the abdominal organs to push into the chest cavity, hindering proper lung formation.
CDH is a life-threatening pathology in infants and a major cause of death due to two complications: pulmonary hypoplasia and pulmonary hypertension.

Types:

Bochdalek hernia: aka postero-lateral diaphragmatic hernia, i
- most common, more than 95% of cases
- hole in the postero-lateral corner of the diaphragm which allows passage of the abdominal viscera into the chest cavity. - majority (80–85%) occur on the left side of the diaphragm, a large proportion of the remaining cases occur on the right side.
Mogagni hernia: rare anterior defect aka retrosternal, or parasternal hernia.
- ~2% of all CDH cases
- characterized by herniation through the foramina of Morgagni which are located immediately adjacent and posterior to the xiphoid process of the sternum.
3) congenital diaphragmatic eventration
- abnormal displacement (i.e. elevation) of part or all of an otherwise intact diaphragm into the chest cavity.
- occurs because in the region of eventration the diaphragm is thinner, allowing the abdominal viscera to protrude upwards.

Mx

OGT
I&V
surg: stitch, patch

Prognosis
- mortality rate of 40–62% with outcomes being more favorable in the absence of other congenital abnormalities. I

Answer 82

A

One of the major consequences of OSAS is an impact on neurocognitive functioning. Several studies have shown that OSAS has an adverse effect on inductive and deductive reasoning, attention, vigilance, learning, and memory. Neurocognitive impairment can be measured objectively with tests such as the Wechsler Adult Intelligence Scale-Revised, the Psychomotor Vigilance Task, the Steer Clear Performance Test, and tests of repetitive finger tapping. In children, OSAS may cause attention-deficit hyperactivity disorder in addition to behavioral problems and learning disabilities. Risk factors for cognitive impairment include increasing age, male sex, apolipoprotein E ε4 allele positivity, current cigarette smoking, obesity, hypertension, diabetes mellitus, metabolic syndrome, Down syndrome, hypothyroidism, significant alcohol consumption, stroke, and the use of psychoactive medications. At a cellular level, OSAS likely causes cognitive impairment through intermittent hypoxia, hormonal imbalance, and/or systemic inflammation, either independently or via the resultant endothelial dysfunction that occurs.

Answer 83

A

Young children are exposed to many types of traumatic experiences, placing them at risk for PTSD. These include:
Abuse, Witnessing interpersonal violence, MVAs, Experiences of natural disasters, Conditions of war. Invasive medical procedures (12)

How Is the Diagnosis Different in Preschool PTSD? Because young children have emerging abstract cognitive and verbal expression capacities, research has shown that the criteria need to be more behaviorally anchored and developmentally sensitive to detect PTSD in preschool children

Immediate reaction to traumatic event criterion - may not have a feasable way to know about these reactions (unable to verbalise, unwitnessed by adults)
intrusion symptoms: Previously required (1) recurrent, (2) intrusive, and (3) distressing. Research showed empirically that preschool children do not always manifest overt distress with their intrusive, unwanted thoughts. Some children were neutral or “over bright”
avoidance symptoms: major change was to require only one symptom in either the avoidance symptoms or negative alterations in cognitions and mood - difficult to detect as internalised
increased arousal sxs: irratibility or outbursts of anger changed to “increased temper tantrums”

DSM V:
“extreme reaction at the time of the event”
- not required vs DSM IV

Re-experiencing sxs: (1 out of 5):

Intrusive recollections not required to be distressing
Nightmares
Dissociation
Psychological distress at reminders
Physiological distress at reminders

Avoidance and numbing symptoms (1 of 5)

Avoid thoughts, feelings, conversations
Avoid activities, places, people
Diminished interests emphasize play constriction
Socially withdrawn behavior
Restricted affect limited to positive emotions

Increased arousal symptoms (2 pf 5)

Sleep difficulty
Irritability includes excessive temper
Concentration difficulty
Hypervigilance
Exaggerated startle response

Answer 84

A

In children, depressive disorders may manifest as sadness or irritability.

DSM-5 Criteria
5 or more symptoms present during a 2 week period; (1) depressed or irritable, cranky mood (outside being frustrated) or (2) loss of interest or pleasure and any three of:
1. Significant weight loss or decrease in appetite (more than 5 percent of body weight in a month or failure to meet expected weight gains.)
2. Insomnia or hypersomnia
3. Psychomotor agitation or retardation
4. Fatigue or lack of energy
5. Feelings of worthlessness or guilt
6. Decreased concentration or indecisiveness
7. Recurrent thoughts of death or suicide

In addition to the above DSM-5 criteria, children and adolescents may also have some of the following symptoms:
• Persistent sad or irritable mood
• Frequent vague, non-specific physical complaints
• Frequent absences from school or poor performance in school
• Being bored
• Alcohol or substance abuse
• Increased irritability, anger or hostility
• Reckless behavior

Symptoms cause significant distress or impairment in functioning.

Depression Scales such as the Beck Depression Inventory, Children’s Depression Inventory or the Reynolds Adolescent Depression
Inventory can be used to establish severity, baseline functioning, and to monitor the progress of treatment.

Answer 85

A

DSM-5 does not distinguish adult-onset from childhood- or adolescent-onset symptoms of bipolar disorder.

The hallmark of bipolar disorder is the manic episode. Manic episodes alternate with depressive episodes, which can be more frequent. During a manic episode in adolescents, mood may be very positive or hyperirritable and often alternates between the 2 moods depending on social circumstances. Speech is rapid and pressured, sleep is decreased, and self-esteem is inflated. Mania may reach psychotic proportions (eg, “I have become one with God”). Judgment may be severely impaired, and adolescents may engage in risky behaviors (eg, promiscuous sex, reckless driving).

Prepubertal children may experience dramatic moods, but the duration of these moods is much shorter (often lasting only a few moments) than that in adolescents.

Onset is characteristically insidious, and children typically have a history of always being very temperamental and difficult to manage.

DSM-5 criteria for a manic episode are as follows:

has a distinct period of abnormally and persistently elevated, expansive, or irritable mood lasting at least 1 week (or any duration if hospitalization is necessary).
during the period of mood disturbance, 3 or more of the following symptoms persisted (4 if the mood is only irritable) and have been present to a significant degree:
- Inflated self-esteem to levels of grandiosity
- Decreased need for sleep
- More talkativeness than usual, often characterized by pressured speech with a sense of a need to keep talking
- Flight of ideas or a subjective feeling that thoughts are racing
- Distractibility
- Increased goal-directed activity or psychomotor agitation
- Excessive involvement in pleasurable activity that has a high potential for painful consequences (eg, hypersexuality, excessive spending, impetuous traveling)
the symptoms do not meet the criteria for a mixed episode.
the mood disturbance is severe enough to cause marked social impairment in occupational functioning, social activities, or relationships with others. Hospitalization may be necessary to prevent harm to self or others or if psychotic features are present.
the symptoms are not due to the direct physiologic effects of a substance or a general medical condition.

Answer 86

A

Two types of anorexia nervosa are recognized:

Restricting type: Patients restrict food intake but do not regularly engage in binge eating or purging behavior; some patients exercise excessively.
Binge-eating/purging type: Patients regularly binge eat and then induce vomiting and/or misuse laxatives, diuretics, or enemas

DSM V:
A. Restriction of energy intake relative to requirements, leading to a significant low body weight in the context of the age, sex, developmental trajectory, and physical health (less than minimally normal/expected1).
B. Intense fear of gaining weight or becoming fat or persistent behavior that interferes with weight gain.
C. Disturbed by one’s body weight or shape, self-worth influenced by body weight or shape, or persistent lack of recognition of seriousness of low bodyweight.

Specify type:
1. Restricting type: During the last 3 months, has not regularly engaged in binge-eating or purging.
2. Binge-eating/purging type: During the last 3 months, has regularly engaged in binge-eating or purging.2
3. Partial remission: After full criteria met, low bodyweight has not been met for sustained period, BUT at least one of the following two criteria still met:
Intense fear of gaining weight/becoming obese or behavior that interferes with weight gain OR Disturbed by weight and shape.
4. Full remission: After full criteria met, none of the criteria met for sustained period of time.

Answer 87

A

DSM V:

A. Recurrent episodes of binge eating, as characterized by both:
Eating, within any 2-hour period, an amount of food that is definitively larger than what most individuals would eat in a similar period of time under similar circumstances.
A feeling that one cannot stop eating or control what or how much one is eating.
B. Recurrent inappropriate compensatory behaviors in order to prevent weight gain such as self-induced vomiting; misuse of laxatives, diuretics, or other medications; fasting or excessive exercise.
C. The binge eating and inappropriate compensatory behaviors occur, on average, at least once a week for 3 months.
D. Self-evaluation is unjustifiability influenced by body shape and weight.
E. The disturbance does not occur exclusively during episodes of anorexia nervosa

Specify if:
Partial remission: After full criteria were previously met, some but not all of the criteria have been met for a sustained period of time.
Full remission: After full criteria were previously met, none of the criteria have been met for a sustained period of time.

Current severity1:
Mild: An average of 1–3 episodes of inappropriate compensatory behaviors per week.
Moderate: An average of 4–7 episodes of inappropriate compensatory behaviors per week.
Severe: An average of 8–13 episodes of inappropriate compensatory behaviors per week.
Extreme: An average of 14 or more episodes of inappropriate compensatory behaviors per week.

Specify type (*no longer a criterion)
Purging vs Nonpurging Type

Answer 88

A

an autosomal dominant inherited condition characterized by hamartomas and an increased lifetime risk of breast, thyroid, uterine, and other cancers. Often underdiagnosed due to variabilty of disease presentation.

Incidence is about 1 in 200,000, making it quite rare.[4] It is associated with mutations in PTEN on 10q23.3, a tumor suppressor gene otherwise known as phosphatase and tensin homolog, that results in dysregulation of the mTOR pathway leading to errors in cell proliferation, cell cycling, and apoptosis.[5] The most common malignancies associated with the syndrome are adenocarcinoma of the breast (20%), followed by adenocarcinoma of the thyroid (7%), squamous cell carcinomas of the skin (4%), and the remaining from the colon, uterus, or others (1%).

Adolescent patients affected with Cowden syndrome develop characteristic lesions called trichilemmomas (solitary papule or mass of small skin-coloured papules that are 1-5 cm in diameter), which typically develop on the face, and verrucous papules around the mouth and on the ears.
Oral papillomas are also common.
Furthermore, shiny palmar keratoses with central dells are also present.
At birth or in childhood, classic features of Cowden’s include pigmented genital lesions, lipomas, epidermal nevi, and cafe-au-lait spots.
Squamous cell carcinomas of the skin may also occur

Answer 89

A

Peutz–Jeghers syndrome (often abbreviated PJS) is an autosomal dominant genetic disorder characterized by the development of benign hamartomatous polyps in the gastrointestinal tract and hyperpigmented macules on the lips and oral mucosa (melanosis).

It has an incidence of approximately 1 in 25,000 to 300,000 births.

Patients with the syndrome have an increased risk of developing carcinomas of the liver, lungs, breast, ovaries, uterus, testes and other organs. Specifically, it is associated with an increased risk of sex-cord stromal tumor with annular tubules in the ovaries.

On chromosome 19, the gene known as STK11 (LKB1)[7] is a possible tumor suppressor gene. 90-100% have this mutation.

The main criteria for clinical diagnosis are:

Family history
Mucocutaneous lesions causing patches of hyperpigmentation in the mouth and on the hands and feet. The oral pigmentations are the first on the body to appear, and thus play an important part in early diagnosis. Intraorally, they are most frequently seen on the gingiva, hard palate and inside of the cheek. The mucosa of the lower lip is almost invariably involved as well.
Hamartomatous polyps in the gastrointestinal tract. These are benign polyps with an extraordinarily low potential for malignancy.

The oral findings are consistent with other conditions, such as Addison’s disease and McCune–Albright syndrome, and these should be included in the differential diagnosis.

Answer 90

A

Most cases of premature thelarche are idiopathic and present under two years of age (and may even start at birth). Many cases will remit spontaneously, and most others do not progress. However, follow-up is warranted because premature thelarche can represent the initial presentation of true CPP in as many as 10 to 20 percent of children referred to pediatric endocrine units [85-87].

Key features of premature thelarche are:

●Isolated breast development, either unilateral or bilateral – Typically not developing beyond Tanner stage 3

●Absence of other secondary sexual characteristics

●Normal height velocity for age (not accelerated)

●Normal or near-normal bone age

Serum luteinizing hormone (LH) and estradiol concentrations are typically in the prepubertal range, but one should be cautious in interpreting these levels in children under the age of two years because elevations can be seen as part of the normal transient “mini-puberty of infancy,” and CPP can be diagnosed inappropriately.

Premature thelarche occurs in two peaks: one during the first two years of life and the other at six to eight years of age [87], with potentially different underlying pathophysiology accountable for each of these peaks. Postulated mechanisms include transient activation of the hypothalamic-pituitary-gonadal axis with excess follicle-stimulating hormone (FSH) secretion [88]. In infants, soy-based formulas have been implicated, although the evidence is weak and may represent only a slower waning of breast tissue during infancy [89-91]. Use of lavender oil, tea tree oil, or hair care products that contain placental extract has also been implicated in some cases of premature thelarche [63]. In most instances, no cause can be found.

Answer 91

A

Premature adrenarche is characterized by the appearance of pubic and/or axillary hair (pubarche) prior to the age of eight years in girls and nine years in boys, in conjunction with a mild elevation in serum dehydroepiandrosterone sulfate (DHEAS) for age.

In the child presenting with isolated pubarche, monitoring for the development of other secondary sexual characteristics (breast or testicular enlargement) is important to ensure that the child’s presentation is not the first feature of CPP or a form of peripheral precocity. Premature adrenarche can be associated with mild growth acceleration and advance in bone age. In children with progressive virilization or with a more advanced bone age (>2 standard deviations [SD] beyond chronologic age), further investigation for other causes of early pubertal development should be considered.

Answer 92

A

induction: anthracycline (danurubicin, idarubicin) and extended exposure to cytarabine. Complete remission rates: >70%

post-remission therapy: high-dose cytarabine (HiDAC).

Answer 93

A

Bronchiectasis is a structural abnormality characterized by abnormal dilation and distortion of the bronchial tree, resulting in chronic obstructive lung disease. This condition is typically the end result of a variety of pathophysiologic processes that render the bronchial walls weakened, easily collapsible, chronically inflamed, and plugged with mucus secretions. Associated findings include atelectasis, emphysema, fibrosis, and hypertrophy of the bronchial vasculature.

The most common symptom in children with bronchiectasis is persistent cough, which is present in 80 to 90 percent of pediatric patients with bronchiectasis and is typically “wet” or productive [1,2]. Fifty-seven to 74 percent of children also produce purulent sputum.

Causes:

bronchial narrowing/obstruction - trahomalacia, bronchomalacia, CPAM, BPS, FB asp, tumour
immunodeficiency
abnorm secretion: CF, PCD
infection: persistent bacterial bronchitis, recurrent infections or aspirations

Sgns:

crackles
wheeze lss common
clubbing
chest wall deformity - hyperinflation of the lungs results in increased anterior to posterior chest diameter, or severe scoliosis

imaging

CXR: linear atelectasis, dilated and thickened airways (ie, tram-tracking or parallel lines on longitudinal view or ring shadows on cross-section) (image 1), and irregular peripheral opacities that represent mucopurulent plugs. Other findings may include loss of lung volume and peribronchial fibrosis
HRCT: he diagnosis of bronchiectasis is made when the internal diameter of the airway is larger than the diameter of the adjacent artery and/or bronchi, visualized within 2 cm of the pleural surface [14], in association with chronic productive cough. One study suggests that a bronchoarterial ratio of >0.76 should be considered abnormal in children. Airway wall thickening (“signet ring” shadows) (image 2) with or without air fluid levels, volume loss, mucus plugging, and air trapping

Answer 94

A

If you see an anemia and an increase in reticulocytes (young red cells) in a patient, that usually means one of two things:

1) there is hemolytic anemia and the marrow is compensating by making more red cells
2) there was a deficiency (like an iron deficiency, for example), but the patient is now on replacement therapy, and the marrow now has enough building blocks to start making more red cells.

iron deficiency without replacement
or B12 or folate deficiency without replacement. Other causes: systemic inflammation, acute blood loss, portosystemic shunt, invasion of marrow by disease (aplastic anaemia, leukemia)

HS: anemia (from the inherent instability of the red cell membrane, which leads to early destruction of the red cells) and – assuming the bone marrow is capable – a reticulocytosis

blood loss: anaemia and retics over time

Answer 95

A

X-linked disorder caused by mutations in the gene that encodes the Wiskott-Aldrich syndrome protein (WASp).

WASp is expressed exclusively in the cytoplasm of hematopoietic cells and plays a crucial role in actin cytoskeleton remodeling [6]. Its absence impacts the formation of the immunologic synapse, the site of interaction between T cells and antigen-presenting cells.
Thus, T cell function is defective due to abnormal cytoskeletal reorganization, leading to impaired migration and adhesion and insufficient interaction with other cells due to abnormal synapse formation. B cell homeostasis is perturbed due to the abnormalities in T cell function, resulting in the depletion of circulating mature B cells, splenic marginal zone precursors, and marginal zone B cells.
NK cell numbers are normal or increased, but cytotoxicity of WASp-deficient NK cells is impaired as a result of defective immune synapse formation on the cell surface.

Thrombocytopenia has been explained by increased clearance, ineffective thrombocytopoiesis [22], reduced platelet survival due to intrinsic platelet abnormalities, and/or immune-mediated events.

Mechanisms for the high incidence of autoimmunity in WAS include inadequate Treg cell function [17-19], B cell-intrinsic loss of tolerance via positive selection of self-reactive transitional B cells [29], expansion of autoreactive B cells [30] and production of autoantibodies [31], impaired Fas-mediated apoptosis of self-reactive lymphocytes [32], and defective phagocytosis of apoptotic cells resulting in chronic inflammation

Classic WAS - severe

thrombocytopenia - petechia, or bleeding from umbi stump
immunodeficiency: recurrent bacterial (encapsulated: step pneumo, H infor, N meningitidis), viral (VZV, CMV), and fungal infections (candida, rare); opportunistic: PCP, Moluscum contagiosum
extensive eczema.
Lymphadenopathy, hepatosplenomegaly.
autoimmune: hemolytic anemia, neutropenia, vasculitis involving both small and large vessels, inflammatory bowel disease, and renal diseases
malignancies: . B cell lymphoma (often Epstein-Barr virus positive) and leukemia

Answer 96

A

Vaginal voiding

aka vaginal reflux of urine is leakage of urine in toilet-trained girls when they stand up after voiding and is a common cause of daytime urinary leakage in girls [40]. This is due to urine being temporarily trapped in the vagina during micturition. It occurs particularly in girls who urinate with their legs too closely approximated to permit free egress of urine. It is a pattern that can be seen during voiding cystourethrography with refluxing urine appearing within the vaginal vault during voiding.
Girls with this condition may also have irritation of the labia and complain of dysuria as the urine passes over the irritated skin.

Overactive bladder

abnormal bladder contraction during the filling phase and is detected by urodynamic evaluation
second most common bladder dysfunction disorder following nocturnal enuresis
The hallmark symptom of this disorder is urgency - can make clinical diagnosis
prevalence of overactive bladder decreases with increasing age: At seven years of age, 21 percent of girls and 18 percent of boys had moderate to severe urinary urgency. Daytime wetting occurred in 6 and 4 percent of girls and boys, respectively. At 17 years of age, 6 percent of girls and 1 percent of boys reported urgency, daytime incontinence, emptying difficulties, or enuresis.

voiding postponement and under active bladder

occurs in children who habitually postpone micturition, often in specific settings (eg, school). Resulting in a low frequency of voiding
commonly have behavioral issues or have a psychological comorbidity
utilize holding maneuvers to prevent voiding
generally have a low voiding frequency with long-time intervals between voids that result in increasing bladder capacity over time. With overdistension of the bladder, the detrusor muscle becomes overstretched and hypoactive leading to a weak or absent contraction (underactive bladder)

Dysfunctional voiding

detrusor contractions during voiding against a closed external urinary sphincter) is caused by an inability to relax the urethral sphincter and/or pelvic floor musculature during voiding
urodynamic studies demonstrate the abnormal contraction of the sphincter and/or pelvic floor musculature during voiding. Urinary flow is interrupted, producing a staccato pattern and a prolonged voiding time.
The contraction of the sphincter during voiding and overactivity of the detrusor muscle can lead to high voiding pressure, bladder decompensation, and a predisposition for infection and renal damage

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