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5th year > Pediatrics > Flashcards

Pediatrics Flashcards

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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
What should be done if a diagnosis of CDI is made?
A CT or MRI of the head to rule out tumors (craniopharyngioma)
26
What is desmopressin?
A synthetic vasopressin without any vasoconstrictor effects
27
What are the clinical uses of desmopressin?
vWF disease CDI Heamophilia A Sleep enuresis
28
What type of lymphadenopathy is seen in EBV virus?
Bilateral cervical lymphadenopathy
29
What malignancies are associated with EBV virus?
Burkitt's lymphoma, NHL (mainly affects the jaw), Hodkins lymphoma, nasopharyngeal carcinoma
30
Is cough more indicative of viral or bacterial pharyntonsilitis?
Viral
31
In bacterial tonsillitis, what lymph nodes are inflamed?
Tender or inflamed, anterior cervical lymph nodes
32
When is ASO useful?
An elevated ASO titer indicates previous S. pyogenes infection. It is indicated in the nonsuppuritive complications of GAS
33
What is the most common age for Croup?
6 months to 3 years
34
Causative pathogens of Croup?
Parainfluenza virus, RSV, adenovirus, influenza virus
35
Differences between Stridor and Wheeze
Stridor: extrathoraic, inspiratory, AW open Wheeze: Intrathoracic, expository, AW closed
36
What is pulses paradoxuses?
Can auscultate Heart sounds during auscultation but cannot feel pulse.
37
What is a pseudo allergy?
IgE independent. Direct mast cell activation Treat same as real allergy/anaphylaxis
38
Is bradypnea or tachypnea seen in anaphylaxis?
Tachypnea
39
What sort of murmur is heard in PDA?
A loud continuous murmur
40
When do you want to keep the PDA open?
TGV, TF
41
Do prostaglandins open or close the PDA?
Open
42
In what genetic disease is CoA seen?
Turner's syndrome
43
Why is CoA asymptomatic when PDA is present?
the presence of a PDA allows blood to bypass the coA (as it allows for a bigger surface area)
44
What sort of murmur is heard in CoA?
Loud systolic ejection murmur
45
In what CHD is the figure of 3 sign seen on Xray?
CoA
46
What is the 'figure of three' sign?
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
What murmur is heard in ASD?
Systolic ejection murmur
48
What heart sounds are heard in ASD?
Systolic ejection murmur | Splitting of S2 that is fixed (due to the volume overload of the pulmonary/right side)
49
When do symptoms of heart failure occur in children with VSD?
When PVR drops to that of adult levels, allowing the left to right shift
50
What murmur is heard in VSD?
Loud holosystolic murmur | Loud S2 if pulmonary hypertension occurs
51
What childhood infections does Coxsackie A cause?
Herpangina | Hand, foot and mouth disease
52
What sort of rash is found in Hand, foot and mouth?
Maculopapular rash, some vesicles on the hand and feet. Ulcers in the mouth (also fever and malaise)
53
What is the causative pathogen of hand, foot and mouth?
Coxsackie A Virus
54
What is the causative pathogen of Roseola Infantum?
HHV-6/HHV-7
55
Which lymph nodes are enlarged in Roseola Infantum?
Occipital, cervical and post-auricular
56
What are Nagayama spots?
Papula enanthem found on the uvula and soft palate
57
In which childhood exanthema are Nagayama spots found?
Roseola Infantum - cause by HHV-6/HHV-7
58
What sort of rash is seen in Roseola Infantum?
Non-puritic, blanching maculopapular rash that begins on the trunk and spreads to the extremities and face
59
What is the peak incidence of Roseola infantum?
6 months to 2 years
60
What is the cause of erythema infectiosum? (5th disease)
Parvovirus B19
61
What age range does erythema infectiosum usually affect?
Years 5-15
62
What type of rash is seen in erythema infectiosum?
Initially confluent, maculopapular rash with perioral sparing that occurs on the cheeks. then spreads to the trunk and extremities.
63
How is parvovirus B19 transmitted?
Aerosol, hematogenously, transplacental
64
What are the consequences of erythema infectiosum?
Arthritis, aplastic crisis, transient anemia
65
What is the result of parvovirus B19 infection in pregnancy?
Hydrops fetalis
66
In what disease do we see 'target signs' on US?
Intususeption
67
When are you likely to feel an 'olive' in the RUQ?
Pyloric stenosis
68
When are you likely to see stomach peristalsis on a physical exam?
Pyloric stenosis | due to the compensatory hypertrophy of the rest of the stomach proximal to the stenosis
69
What is the name for pyloromyotomy?
Ramstedt procedure
70
What is the peak age for testicular torsion?
First 30 days of life and ages 10-14
71
What is the Prehn sign?
Elevation of the scrotum releives testicular pain; positive in Epidymitis and negative in testicular torsion
72
Why is ovarian necrosis uncommon in ovarian torsion?
Because of dual blood supply from the uterine and ovarian arteries
73
Why would you do a biopsy in lactose intoleranece?
To distinguish between primary and secondary lactose intolerance: primary: no damage intestinal epithelium, secondary: injured tips of intestinal villi
74
What is the tetrad of symptoms seen in IgA vasculitis?
palpable purpura renal disease arthralgia/arthritis abdominal pain
75
Where would you see palpable purpura in IgA vasculitis?
Legs, buttocks and other areas of pressure constraint
76
If you took a biopsy of the skin in IgA vasculitis, what would you see?
IgA and C3 immune complex deposition in the small vessels of the superficial dermis
77
If you took a biopsy of the kidney in IgA vasculitis, what would you see?
Mesangial IgA deposition
78
What is an acquired form of haemophilia?
Liver failure Vit K deficiency Autoimmunity against coagulation factors DIC (consumes clotting factors)
79
Which coagulation factors does the liver synthesise?
``` I II V VII VIII IX X XI XIII ```
80
What does the Prothrombin time (PT) measure?
Extrinisic and common pathway | factors 7, 10, 5, 2 & 1
81
What does the partial thromboplastin time (PTT) measure
Common and intrinsic pathway | factors 12, 11, 9, 8, 10, 5, 11, 1
82
What coagulopathy would you see in the labs of a patient with Hemophillia A or B?
PT normal | PTT prolonge
83
Explain mixing studies
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
What type of disorders are inherited Hemophilias?
X linked recessive
85
What coagulopathy would you see in a patient with Vitamin K deficiency of the newborn?
Prolongued PT | Prolongued or normal aPTT
86
What can be causes of acquired VWD?
Lympho and myeloproliferative disorders Autoimmune disorders (such as SLE) Drugs (Valproate) Cardiovascular defects (aortic stenosis)
87
Does vWFD affect primary or secondary homeostasis?
vWFD affects primary and secondary hemostasis (primary - vWF and secondary - factor 8)
88
What lab findings would you see in vWFD?
``` Prolongued bleeding time Prolongued aPTT Normal PT Normal platelet count Decreased VIII Decreased vWF antigen levels ```
89
What is considered portal hypertension?
Hepatic Venous Pressure Gradient > 6mmHg
90
Explain the pathophysiology of Budd CHiaria formation
Obstruction in the hepatic vein - impaired venous drainage of the liver - venous congestion - tissue hypoxia - centrilobular necrosis - congestive hepatopathy - nutmeg liver
91
What is hematamesis?
Vomiting blood
92
Venous drainage of the esophagus
Upper 2/3 is drained by oesophageal veins --> azygous vein --> IVC Lower 1/3 superficial veins --> left gastric vein --> portal vein
93
In which part of the oesophagus do oesophageal varix occur?
lower 1/3 due to the venous drainage --> superficial oesophageal veins --> left gastric vein --> portal vein
94
What is the definition of maldigestion?
Impaired breakdown of food in the intestinal lumen
95
What is the definition of malabsorption?
impaired absorption of digested food cause by alterations in intestinal mucosa
96
What is Schwachman syndrome?
It is an AR disorder that causes exocrine pancreatic insufficiency due to the absence of acinar cells which produce the digestive enzymes
97
What circumstances can cause a (increased) positive osmotic fragility test?
Hereditary spherocytosis | Hypernatremia
98
What circumstances can cause a (decreased) negative osmotic fragility test
hronic liver disease, iron deficiency anemia, thalassemia, hyponatremia, polycythemia vera, hereditary xerocytosis, and sickle cell anemia after splenectomy.
99
Why would you perform a Coombs test in hereditary spherocytosis?
As spherocytes are seen also in autoimmune haemolytic anaemia and you want to rule out AHA
100
What drugs are associated with the oxidative stress which can trigger transient haemolytic anaemia in patients with G6PD
``` Nitrofuratoin Sulfonamides Vit K derivitives anti malarial ASA ```

5th year (2 decks)

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