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5th year > Pediatrics > Flashcards

Pediatrics Flashcards

1
Q

MoA and therapeutic uses of Emicizumab

A

Coagulation factor VIII normally activates factor X. In Heamophilia A, factor VIII is missing - Emicizumab binds to factor IX and factor X, mediating the activation of factor X.

Heamophilia A patients

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2
Q

What can be the cause of purpura that occurs in children following a viral infection?

A

Immune thrombocytopenia - purpura

IgA Vasculitis - purpura

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3
Q

Explain the hydrogen breath test

A

There is no source for Hydrogen gas production in humans other than bacterial metabolism of carbohydrates. If the patient has a lactase deficiency then lactose is not broken down into monosaccharides and instead passes into the colon where it is metabolised by bacteria, causing H release into the blood where it then travels to the lungs where it is exhaled. An increase in H in the breath at increments following lactose injestion is indicative of lactose intolerance.

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4
Q

What is the cause of fructose malabsorption?

A

Due to an absence/deficiency in fructose carriers (GLUT5) in the intestinal enterocytes. Can also be caused by diseases such as Celiac, where the damage to the intestine has resulted in a decrease/absence of GLUT5 transporters.

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5
Q

Clinical characteristics of WAGR syndrome

A

Wilms tumor
Aniridia (a partial or complete loss of the iris)
Genitourinary abnormalities (psuedohemaphrotidism, undescended testes, early onset nephrotic syndrome)
Range of intellectual disabilities

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6
Q

What is the cause of WAGR syndrome

A

Deletion of 11p13 that leads to deletion of WT1 and Pax6

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7
Q

What is the cause of Denys-Drash syndrome

A

Point mutation in WT1

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8
Q

Clinical characteristics of Denys Drash syndrome

A

Wilms tumor

Pseudohemaphrotidism, undescended tests, early onset nephrotic syndrome

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9
Q

What sort of gene is WT1?

A

Tumor supressor gene

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10
Q

What is the purpose of the omphalomesenteric duct?

A

It connects the midgut with the yolk sac. It is obliterated in the 6th to 7th week of embryological development

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11
Q

What is the cause of Meckel diverticulum?

A

Incomplete obliteration of the omphalomesenteric duct

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12
Q

What is hematochezia?

A

Lower GI bleeding - bright red blood in stool,

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13
Q

What is a Meckel scan?

A

99 Technetium scintigraphy
It is radiolabeled technetium which is preferentially absorbed by the gastric mucosa which can identify ectopic gastric tissue within the diverticulum.

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14
Q

Name of the pyloromyotomy

A

Ranstedt procedure

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15
Q

Explain the Rovsing sign?

A

If pressure is applied to the LLQ and pain is felt in the LRQ, it is said to be positive Rovsing sign for acute appendicitis.

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16
Q

Name the bradykinin B2 receptor antagonist

A

icatibant

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17
Q

Name the clinical use of icatibant

A

Hereditary angioedema due to C1 deficiency (it is a bradykinin antagonist)

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18
Q

What type of hypersensitivity reaction is urticaria?

A

T1 HSN reaction

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19
Q

What is the pathophysiology and location of urticaria?

A

Mast cell activation and degranulation in the superficial dermis

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20
Q

What are the acquired caused of nephrogenic DI?

A

Demeclocycline, lithium, Hypokalemia and hypocalcemia

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21
Q

What is the osmolarity of urine in DI?

A

Low urine osmolarity

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22
Q

What is the normal osmolarity of urine?

A

500-800 mOsmol/kg

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23
Q

What is the ADH level in CDI?

A

Low

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24
Q

What is the ADH level in NDI?

A

high or normal (to compensate for the high urine output)

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25
Q

What should be done if a diagnosis of CDI is made?

A

A CT or MRI of the head to rule out tumors (craniopharyngioma)

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26
Q

What is desmopressin?

A

A synthetic vasopressin without any vasoconstrictor effects

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27
Q

What are the clinical uses of desmopressin?

A

vWF disease
CDI
Heamophilia A
Sleep enuresis

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28
Q

What type of lymphadenopathy is seen in EBV virus?

A

Bilateral cervical lymphadenopathy

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29
Q

What malignancies are associated with EBV virus?

A

Burkitt’s lymphoma, NHL (mainly affects the jaw), Hodkins lymphoma, nasopharyngeal carcinoma

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30
Q

Is cough more indicative of viral or bacterial pharyntonsilitis?

A

Viral

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31
Q

In bacterial tonsillitis, what lymph nodes are inflamed?

A

Tender or inflamed, anterior cervical lymph nodes

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32
Q

When is ASO useful?

A

An elevated ASO titer indicates previous S. pyogenes infection. It is indicated in the nonsuppuritive complications of GAS

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33
Q

What is the most common age for Croup?

A

6 months to 3 years

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34
Q

Causative pathogens of Croup?

A

Parainfluenza virus, RSV, adenovirus, influenza virus

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35
Q

Differences between Stridor and Wheeze

A

Stridor: extrathoraic, inspiratory, AW open
Wheeze: Intrathoracic, expository, AW closed

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36
Q

What is pulses paradoxuses?

A

Can auscultate Heart sounds during auscultation but cannot feel pulse.

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37
Q

What is a pseudo allergy?

A

IgE independent.
Direct mast cell activation
Treat same as real allergy/anaphylaxis

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38
Q

Is bradypnea or tachypnea seen in anaphylaxis?

A

Tachypnea

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39
Q

What sort of murmur is heard in PDA?

A

A loud continuous murmur

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40
Q

When do you want to keep the PDA open?

A

TGV, TF

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41
Q

Do prostaglandins open or close the PDA?

A

Open

42
Q

In what genetic disease is CoA seen?

A

Turner’s syndrome

43
Q

Why is CoA asymptomatic when PDA is present?

A

the presence of a PDA allows blood to bypass the coA (as it allows for a bigger surface area)

44
Q

What sort of murmur is heard in CoA?

A

Loud systolic ejection murmur

45
Q

In what CHD is the figure of 3 sign seen on Xray?

A

CoA

46
Q

What is the ‘figure of three’ sign?

A

It represents the pre-stenotic dilation of the aortic arch and left subclavian artery, the dentation caused by the coarctation and then the post-stenotic dilation of the descending aorta

47
Q

What murmur is heard in ASD?

A

Systolic ejection murmur

48
Q

What heart sounds are heard in ASD?

A

Systolic ejection murmur

Splitting of S2 that is fixed (due to the volume overload of the pulmonary/right side)

49
Q

When do symptoms of heart failure occur in children with VSD?

A

When PVR drops to that of adult levels, allowing the left to right shift

50
Q

What murmur is heard in VSD?

A

Loud holosystolic murmur

Loud S2 if pulmonary hypertension occurs

51
Q

What childhood infections does Coxsackie A cause?

A

Herpangina

Hand, foot and mouth disease

52
Q

What sort of rash is found in Hand, foot and mouth?

A

Maculopapular rash, some vesicles on the hand and feet. Ulcers in the mouth

(also fever and malaise)

53
Q

What is the causative pathogen of hand, foot and mouth?

A

Coxsackie A Virus

54
Q

What is the causative pathogen of Roseola Infantum?

A

HHV-6/HHV-7

55
Q

Which lymph nodes are enlarged in Roseola Infantum?

A

Occipital, cervical and post-auricular

56
Q

What are Nagayama spots?

A

Papula enanthem found on the uvula and soft palate

57
Q

In which childhood exanthema are Nagayama spots found?

A

Roseola Infantum - cause by HHV-6/HHV-7

58
Q

What sort of rash is seen in Roseola Infantum?

A

Non-puritic, blanching maculopapular rash that begins on the trunk and spreads to the extremities and face

59
Q

What is the peak incidence of Roseola infantum?

A

6 months to 2 years

60
Q

What is the cause of erythema infectiosum? (5th disease)

A

Parvovirus B19

61
Q

What age range does erythema infectiosum usually affect?

A

Years 5-15

62
Q

What type of rash is seen in erythema infectiosum?

A

Initially confluent, maculopapular rash with perioral sparing that occurs on the cheeks. then spreads to the trunk and extremities.

63
Q

How is parvovirus B19 transmitted?

A

Aerosol, hematogenously, transplacental

64
Q

What are the consequences of erythema infectiosum?

A

Arthritis, aplastic crisis, transient anemia

65
Q

What is the result of parvovirus B19 infection in pregnancy?

A

Hydrops fetalis

66
Q

In what disease do we see ‘target signs’ on US?

A

Intususeption

67
Q

When are you likely to feel an ‘olive’ in the RUQ?

A

Pyloric stenosis

68
Q

When are you likely to see stomach peristalsis on a physical exam?

A

Pyloric stenosis

due to the compensatory hypertrophy of the rest of the stomach proximal to the stenosis

69
Q

What is the name for pyloromyotomy?

A

Ramstedt procedure

70
Q

What is the peak age for testicular torsion?

A

First 30 days of life and ages 10-14

71
Q

What is the Prehn sign?

A

Elevation of the scrotum releives testicular pain; positive in Epidymitis and negative in testicular torsion

72
Q

Why is ovarian necrosis uncommon in ovarian torsion?

A

Because of dual blood supply from the uterine and ovarian arteries

73
Q

Why would you do a biopsy in lactose intoleranece?

A

To distinguish between primary and secondary lactose intolerance: primary: no damage intestinal epithelium, secondary: injured tips of intestinal villi

74
Q

What is the tetrad of symptoms seen in IgA vasculitis?

A

palpable purpura
renal disease
arthralgia/arthritis
abdominal pain

75
Q

Where would you see palpable purpura in IgA vasculitis?

A

Legs, buttocks and other areas of pressure constraint

76
Q

If you took a biopsy of the skin in IgA vasculitis, what would you see?

A

IgA and C3 immune complex deposition in the small vessels of the superficial dermis

77
Q

If you took a biopsy of the kidney in IgA vasculitis, what would you see?

A

Mesangial IgA deposition

78
Q

What is an acquired form of haemophilia?

A

Liver failure
Vit K deficiency
Autoimmunity against coagulation factors
DIC (consumes clotting factors)

79
Q

Which coagulation factors does the liver synthesise?

A 
I
II
V
VII
VIII
IX
X
XI
XIII
80
Q

What does the Prothrombin time (PT) measure?

A

Extrinisic and common pathway

factors 7, 10, 5, 2 & 1

81
Q

What does the partial thromboplastin time (PTT) measure

A

Common and intrinsic pathway

factors 12, 11, 9, 8, 10, 5, 11, 1

82
Q

What coagulopathy would you see in the labs of a patient with Hemophillia A or B?

A

PT normal

PTT prolonge

83
Q

Explain mixing studies

A

Mixing studies are used to differentiate between factor deficiencies or if an inhibitor is present. They are indicated when the aPTT is prolonged. They take advantage of the fact that factor levels that are 50% of normal should give a normal aPTT time.

84
Q

What type of disorders are inherited Hemophilias?

A

X linked recessive

85
Q

What coagulopathy would you see in a patient with Vitamin K deficiency of the newborn?

A

Prolongued PT

Prolongued or normal aPTT

86
Q

What can be causes of acquired VWD?

A

Lympho and myeloproliferative disorders
Autoimmune disorders (such as SLE)
Drugs (Valproate)
Cardiovascular defects (aortic stenosis)

87
Q

Does vWFD affect primary or secondary homeostasis?

A

vWFD affects primary and secondary hemostasis (primary - vWF and secondary - factor 8)

88
Q

What lab findings would you see in vWFD?

A 
Prolongued bleeding time
Prolongued aPTT
Normal PT
Normal platelet count
Decreased VIII
Decreased vWF antigen levels
89
Q

What is considered portal hypertension?

A

Hepatic Venous Pressure Gradient > 6mmHg

90
Q

Explain the pathophysiology of Budd CHiaria formation

A

Obstruction in the hepatic vein - impaired venous drainage of the liver - venous congestion - tissue hypoxia - centrilobular necrosis - congestive hepatopathy - nutmeg liver

91
Q

What is hematamesis?

A

Vomiting blood

92
Q

Venous drainage of the esophagus

A

Upper 2/3 is drained by oesophageal veins –> azygous vein –> IVC

Lower 1/3 superficial veins –> left gastric vein –> portal vein

93
Q

In which part of the oesophagus do oesophageal varix occur?

A

lower 1/3 due to the venous drainage –> superficial oesophageal veins –> left gastric vein –> portal vein

94
Q

What is the definition of maldigestion?

A

Impaired breakdown of food in the intestinal lumen

95
Q

What is the definition of malabsorption?

A

impaired absorption of digested food cause by alterations in intestinal mucosa

96
Q

What is Schwachman syndrome?

A

It is an AR disorder that causes exocrine pancreatic insufficiency due to the absence of acinar cells which produce the digestive enzymes

97
Q

What circumstances can cause a (increased) positive osmotic fragility test?

A

Hereditary spherocytosis

Hypernatremia

98
Q

What circumstances can cause a (decreased) negative osmotic fragility test

A

hronic liver disease, iron deficiency anemia, thalassemia, hyponatremia, polycythemia vera, hereditary xerocytosis, and sickle cell anemia after splenectomy.

99
Q

Why would you perform a Coombs test in hereditary spherocytosis?

A

As spherocytes are seen also in autoimmune haemolytic anaemia and you want to rule out AHA

100
Q

What drugs are associated with the oxidative stress which can trigger transient haemolytic anaemia in patients with G6PD

A 
Nitrofuratoin
Sulfonamides
Vit K derivitives
anti malarial 
ASA

5th year (2 decks)

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