General Paeds Surgery Flashcards

1
Q

Paediatric renal transplant

A

In children obstructive uropathy is the leading cause
followed by structural abnormalities and
focal segmental glomerulosclerosis.
Glomerulonephritis is more common in adult patients.
The first choice of donor for a pediatric transplant is a living donor graft, followed by an adult size cadaver source. If a pediatric donor must be used, the age should be at least 6 years old and preferably over age 10

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2
Q

What are the energy requirement of term PN fed? And enteraly fed?

A

85-90 kcal/kg/day if PN +10% if enteral fed

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3
Q

What are the pathogens in Neutropenic necrotizing fasciitis?

A

gastrointestinal flora, gram negative rods and vancomycin resistant enterococcus are more commonly isolated

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4
Q

In which pathology(s) 11:22 translocation is found?

A

Ewing’s sarcoma

PNET; primitive neuro-ectodermal tumour

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5
Q

Is bi directional flow on echo an indication for PDA ligation?

A

Bi-directional shunting in the PDA suggests that pulmonary hypertension has not resolved. Ligation of the PDA in this setting may cause serious right heart failure and demise of the infant

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6
Q

What is Fontan procedure?

A

is a palliative surgical procedure used in children with univentricular hearts. It involves diverting the venous blood from the inferior vena cava (IVC) and superior vena cava (SVC) to the pulmonary arteries without passing through the morphologic right ventricle

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7
Q

What are the indications for endocarditis prophylaxis?

A

The current recommendations in CHD are:

a. Respiratory related procedures may require prophylaxis if the mucosa is incised such as in a lobectomy.
b. Congenital heart repairs that have prosthetic materials that may incompletely epithelialized(6 month) and may be exposed to soilage within are also considered appropriate for prophylaxis.
c. Certain dental procedures may require prophylaxis.

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8
Q

What is the type of abdominal injury in NAI?

A

Solid organ

Small intestine perforation (not usually colonic)

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9
Q

What is Ludwig’s angina?

A

is a rapidly spreading, firmly indurated cellulitis that originates intraorally and involves supramylohyoid and inframylohyoid compartments bilaterally, but without abscess or lymphadenopathy
It presents with acute left neck pain, difficulty swallowing, drooling, and displacement of the tongue superiorly

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10
Q

What are the signs and cause of cat scratch disease?

A
Papule on extremity 
Long Hx of lymphadenopathy 
Low grade temperature 
Bartonella is the causative organism 
Management is reassurance, adequate follow-up and analgesics
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11
Q

In PNLAD, what are the proposed mechanism to explain the beneficial effect of ω3FAs (omega3, omegavin).

A

First, the addition of ω3FAs results in an improvement in bile flow. Second, ω3FAs result in decreased steatosis.
Third, the addition of ω3FAs shifts the eicosanoid profile towards a reduced inflammatory state

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12
Q

What are the carbs in breast milk?

A

Carbohydrates in breast milk include lactose & oligosaccharides.
The oligosaccharides act as probiotics stimulating growth of Lactobacillus and Bifidobacterium

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13
Q

What’s Reye syndrome and Reye-like syndrome?

A

Reye syndrome is an acute metabolic encephalopathy, largely affecting children and adolescents.
In Reye-like syndrome, because of inborn errors of metabolism, hypoglycemia, hypoketonemia, elevated ammonia, and organic aciduria are often evident.

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14
Q

What’s the genetic insult in DiGeorge syndrome?

A

DiGeorge syndrome (DGS) is one of a group of phenotypically similar disorders including velocardiofacial syndrome (VCFS, or Shprintzen syndrome) and conotruncal anomaly face (CTAF) syndrome that share a microdeletion of chromosome 22q11.2, a region known as the DGS critical region

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15
Q

Loss of heterozygosity (LOH) of chromosome arm 11p markers occurs commonly in hepatoblastoma identified in association with?

A

Beckwith-Wiedemann syndrome (BWS) and hemihypertrophy

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16
Q

What is the management of Pediatric adrenocortical tumors (ACT) ?

A

Surgical resection, no role for neo-adjuvant chemotherapy

Mitotane is an adrenolytic agent which blocks adrenal steroid hydroxylation; it may help alleviate symptoms in patients who cannot undergo complete resection.

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17
Q

What is Paget von Schrötter syndrome?

A

axillary-subclavian effort-induced venous thrombosis, commonly after pole vaulting sport. secondary to compression of the subclavian vein at the thoracic outlet between the clavicle and first rib.
Neurological symptoms are rare
Venous gangrene is rare
Treatment is catheter directed thrombolysis and resection of first rib

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18
Q

How do you correct coagulopathy in a patient with inherent coagulopathy or on warfarin sustained head trauma?

A

Recombinant factor VIIa (rFVIIa, 7)

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19
Q

What is the success rate of re-do fundoplication?

A

70% regardless of the technique of the first procedure

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20
Q

What are the complications of fundoplication?

A

herniation of the wrap through the hiatus, “slipped wrap” onto the stomach, disruption of the wrap and gas bloat. Wrap herniation through the hiatus appears to be the most common complication associated with recurrence of symptoms.

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21
Q

What are the complications/ presentations of Meckel’s diverticulum?

A

Bleeding 50%
Obstruction (intuss-volvulus) 15-20%
Diverticulitis 15-20%
Umbilical symptoms

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22
Q

Which type of button batteries cause more injury?

A

Lithium is more dangerous than alkaline cells with necrosis beginning in fifteen minutes

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23
Q

What are the manifestation and treatment of local anaesthetic toxicity?

A

Toxicity manifests with neurologic (agitation, seizures) and cardiac (dysrythmias) side effects.
timely infusion of 1 to 2 mg/kg of 20% intralipid emulsion can reverse the toxic effects of bupivicaine

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24
Q

In Preoperative preparation for general anesthesia in sickle cell, transfusion aim is?

A

Transfusion to a hemoglobin of 10g/dL was found to be just as effective at decreasing the incidence of postoperative sickle exacerbations as a more aggressive transfusion strategy (decreasing the Hg S fraction to less than 30%).

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25
Q

What are the factors associated with increased risk of anaesthesia in mediastinal masses?

A
  • orthopnea (although the absence of symptoms does not imply safety)
  • greater than a 50% decrease in tracheal cross-sectional area on CT
  • greater than a 50% decrease in supine peak expiratory flow rate
  • severe narrowing or occlusion of one or both mainstem bronchi
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26
Q

What are the causes of Congenital hydrocolpos?

A

Cloaca, congenital imperforate hymen, complete transverse vaginal septum, or partial vaginal agenesis, as well as several genetic syndromes including the McKusick-Kaufman syndrome and Bardet-Biedl syndrome

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27
Q

What are the potential risks after splenectomy?

A

overwhelming postsplenectomy sepsis (OPSI).
Splenectomy increases the risk of vascular thrombosis.
increased intravascular hemolysis.

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28
Q

What is the best test to differentiate focal hyperinsulinism from focal HI?

A

PET scans with 18-fluoro-L-3,4-dihydroxyphenylalanine (18F-DOPA) have been shown to discriminate focal HI from diffuse

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29
Q

What does the medical management of HI involve?

A

First-line therapy for HI is medical, using diazoxide, a KATP channel agonist. Some patients do not respond to diazoxide because of mutations in KATP channel. Second-line therapy is with octreotide, a somatostatin analog
Glucagon to maintain euglycaemia

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30
Q

What is the most common malignant for of SCT?

A

Endodermal sinus tumour
If the tumor is malignant, chemotherapy with vincristine, actinomycin C, and cytoxan (VAC), and cisplatin, etoposide, and bleomycin (PEB) results in improved survival.

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31
Q

What is the rate of anatomical abnormalities in patients with reflux?

A

5%

UGI contrast have 50% false positive rate in GORD

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32
Q

What is the risk of GU anomalies in ARM?

A

50%
Cloaca or rectovesical fistula have a nearly 90% incidence of GU abnormalities, while perineal fistulas have a less than 10% incidence
Müllerian abnormalities are found in about 30- 45% of females with ARMs
Tethered cord 25%
Trisomy 21 is the most common chromosomal anomaly associated with ARMs

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33
Q

What are viral B, C & HIV transfusion risk?

A

HIV & C 1 in 2 million

B 1 in 200,000

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34
Q

What Skin changes can be seen in ulcerative colitis?

A

erythema nodusum (painful red nodules on the legs and arms)

pyoderma gangrenosum (small painful purulent ulcers, usually on the legs)

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35
Q

What is Lamber-Eaton syndrome?

A

Lambert-Eaton myasthenic syndrome (LEMS) is a rare presynaptic disorder of neuromuscular transmission. It’s a para-neoplastic syndrome; This is usually a small cell lung cancer (SCLC), lymphosarcoma, malignant thymoma, or carcinoma of the breast, stomach, colon, prostate, bladder, kidney, or gallbladder.

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36
Q

What are the indications of bariatric surgery in adolescents?

A

adolescent with a :
-BMI of 35 kg/m2 or greater in the presence of severe obesity related co-morbidities
- in the absence of co-morbidity if the BMI is 40 kg/m2
Comorbidities: joint replacement, type 2 diabetes mellitus, pseudotumor cerebri, sleep apnea (apnea-hypopnea index > 15) and severe steohepatitis

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37
Q

What are the types of blunt cardiac injury and how to diagnose?

A

BCI, 95% are cardiac contusions followed by valvular dysfunction and ventricular septal defect
Troponin I is a sensitive and more specific measure than CK-MB, levels become elevated within hours of injury and remain elevated for 4-7 days

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38
Q

Ina newborn infant when does the kidney reach GFR and concentration ability?

A

GFR 90% by 3 months
Renal tubular maturation (concentration) takes two to three years to reach adult levels.
Creatinine clearance reaches adult levels by two years of age.

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39
Q

What’s shunt fraction, and what affects it?

A

The shunt fraction is the percentage of blood pumped from the heart that is not completely oxygenated.
“physiological shunt,” and is rarely over 4%
Atelectasis, pulmonary contusion and aspiration increase shunt fraction
Placing a patient prone will often redistribute blood from the dependent areas full of inflammatory fluid to those areas with better ventilation, decrease shunt fraction

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40
Q

Stress ulcer prophylaxis in intensive care unit (ICU) patients is associated with decreased?

A

Blood in gastric aspirated

There is no evidence to suggest reduced significant GI bleed, mortality or ventilator associated pneumonia

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41
Q

How much bile acid is secreted daily?

A

3 grams per day in 4-12 cycles, 95% reabsorbed and only 2-5% is excreted in faeces

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42
Q

What is the indicative ratio of The CPAM volume to head circumference ratio (CVR)?

A

Those with a CVR >1.6 have a large mass lesion that will result in fetal morbidity or mortality in about 80% of cases without fetal intervention.
The CPAM lung lesions with a CVR < 1.6 will often not continue to grow past the 28th week of gestation.

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43
Q

What is the management of cholestasis in premature infants?

A

Conservative main; continue enteral feeding
Gallstones in neonates, even from TPN, may be expected to resolve spontaneously.
Biliary tree irrigation has also been reported to be beneficial in instances where there is not biliary tree dilation, but where cholestasis is not resolving.
no benefits from cholecystokinin-octapeptide or ursodeoxycholic acid in this condition

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44
Q

What’s delayed gastric emptying?

A

more than 50% retained labeled liquid or solid meal within the stomach after 120 minutes in children younger than two years and after 60 minutes in children older than two years

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45
Q

What percentage of GORD patient have delayed gastric emptying?

A

It is estimated that forty to fifty percent of children with symptomatic gastroesophageal reflux have delayed gastric emptying.
Studies have demonstrated that fundoplication alone decreases gastric emptying time

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46
Q

What is the percentage of malformation in Prune belly?

A

Thirty percent of patients will have intestinal malrotation. Although jejunoileal atresia remains rare, imperforate anus and anorectal agenesis have been reported. The latter has been noted in a significant number of patients, 5/13 in one series.

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47
Q

What is the Na concentration of normal saline and ringer lactate?

A
lactated ringers (LR) (134 mEq/l Na) 
Normal saline (154 meq Na/l)
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48
Q

What percentage of rectal prolapse have CF?

A

Cystic fibrosis accounted for 11% of patients with rectal prolapse in one historical series and current recommendations include cystic fibrosis screening in patients with rectal prolapse. The recommendations are based on 30 year old literature. In a recent 15 year review there were no patients diagnosed with CF on evaluation for rectal prolapse.

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49
Q

In PN patient, which trace element deficiency can cause DM like symptoms?

A

Chromium deficiency

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50
Q

What are the common mutations in HD?

A

The more common mutations associated with Hirschsprung’s disease are the RET gene, the EDNRB and the END3 gene

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51
Q

What percentage of OA/TOF develops trachiomalacia?

A

Up to 75% of infants with esophageal atresia and a TEF will have structural abnormalities in the trachea with weakened cartilaginous rings, though only 25% may become symptomatic.

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52
Q

In ITP how does steroids and IVIG work?

A

Corticosteroids work by inhibition of reticuloendothelial binding of platelet/antibody complexes
IVIG inhibits Fc receptor binding of platelets by macrophages

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53
Q

What is The most significant factor affecting post-operative recurrence of Crohn‘s?

A

Smoking

at least double the risk of recurrence

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54
Q

What is the most common site of rebleeding after sclerotherapy for oesophageal varices in extra hepatic portal venous obstruction?

A
Gastric varices (30%) 
As opposed to (4.6%) oesophageal varices in one study
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55
Q

What is the definition of portal hypertension?

A

Pressure more than 5-10 mmHg

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56
Q

What is The most common presentation of extrahepatic portal venous obstruction (EHPVO) in children?

A

is variceal bleed and splenomegaly

Non cirrhotic, not jaundice

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57
Q

Post splenectomy sepsis is higher post traum or haematology?

A

Splenectomy from trauma is associated with significantly lower PSS risk than for other indications, specifically hematologic abnormalities

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58
Q

What percentage of spleen is required to prevent PSS?

A

It is felt that preservation of more than 40% of the native splenic volume is necessary for prevention of PSS, although the evidence behind this recommendation is relatively weak

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59
Q

What percentage of Extralobar sequestration is associated with other anomalies?

A

30% including cardiac, hindgut and gastrointestinal abnormalities.

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60
Q

Does intralobar sequestration associate with GI anomalies?

A

Communication between the lesion and the GI tract occurs in about 10%.

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61
Q

What percentage of the <2 years develop FIA following perineal abscess I&D?

A

anal fistula in up to 30% of patients following Incision and drainage of a perianal abscess in the < two-year-old age group

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62
Q

Is neonatal torsion associated with difficult delivery?

A

There is retrospective evidence to suggest that neonatal torsions occur in mothers with difficult deliveries

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63
Q

What are calcinurin inhibitors?

A

Calcineurin inhibitors (eg, cyclosporin A [CSA], tacrolimus [TAC]) are steroid-sparing agents that can also be used in children who fail to respond to, or who subsequently relapse after, treatment with cyclophosphamide (from nephrotic syndrome)

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64
Q

In coach what are the rates for GU anomalies?

A

50% will develop renal insufficiency
50-67% will have gyne abnormality
30-40% menstrual obstruction

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65
Q

What’s the accepted pre- post- ductal saturations in CDH?

A

preductal oxygen saturation levels from 85-95%,
post ductal oxygen saturation >70% and
paCO2 of 45 – 60 mm Hg

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66
Q

What is undrained traumatic hemothorax (UTH) and how you manage it?

A

Residual collection in the chest following tube thoracostomy alone in trauma case, it occurs in 8% of cases of haemothorax treated by tube thoracotomy.
Administration of intrapleural thrombolytic agents has been found to be cost effective and successful in treating UTH in over 90% of patients and should be the preferred initial therapy.

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67
Q

In inhumanly hernias what is the rate of:
Incarceration as first presentation?
Contralateral PPV?
Need for groin exploration in adulthood?

A

<20% will present with incarceration
contralateral PPV 63% < 2months and 41% >2years, highest rate of metachronous hernia development approaching 31%
repeat groin exploration was 8% (with a median time to re-exploration being 38 years)

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68
Q

In circumcision what’s the rates of UTI prevention?

A

Given the normal risk of male urinary tract infection (UTI) of one percent, the number-needed-to-treat (NNT) to prevent a single UTI is 111. For patients with high grade vesicoureteral reflux who have a 30% rate of UTI the NNT is 4.

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69
Q

In Meckel’s diverticulum what is the rate of pancreatic mucosa?

A

15%
radionuclide technetium 99m pertechnetate excreted by the acid -secreting parietal cells of gastric mucosa and does not detect pancreatic mucosa

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70
Q

What is the histopathology findings of neuronal intestinal dysplasia (NID)?

A

Type B: 15-20% giant ganglia in the submucosal layer, increased Ach staining in the lamina propria or around submucosal vessels

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71
Q

What is the incidence of recurrent symptoms after Heller’s and how to manage?

A

30-50%. When symptoms recur, an attempt at balloon dilation with or without Botox injection is a reasonable option; however, revision Heller myotomy provides the best long term results

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72
Q

What is Petersen’s window?

A

In Roux en Y gastric bypass, left to right bowel herniation through Petersen’s window between the Roux en Y limb and the transverse mesentery can occur and can give The “mushroom sign” CT, suggests a herniation of the small bowel with streaming edematous mesentery through a mesenteric defect.

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73
Q

What is the incidence of typhlitis?

A

2-30% of patients
more prevalent during induction chemotherapy and in AML vs ALL
The presence of clostridium septicum should also alert one to the potential for ileocecal disease

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74
Q

How do you manage leg ischemia from femoral artery ECMO cannula?

A

placement of a femoral artery catheter in a distal direction at the common femoral access site, to allow distal perfusion via the ECMO circuit

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75
Q

Which factor is associated with the highest risk of developing recurrent gastroesophageal reflux disease (GERD) following fundoplication in children?

A

postoperative retching and postoperative esophageal dilation highest risk (Ngerncham et al, 2007)
Age less than six years, preoperative hiatal hernia were significant factors

neurologic impairment was not associated with increased risk for recurrent disease when controlled for the other factors

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76
Q

What is the presentation of MEN 2A?

A

All patients 100% develop MTC on the basis of C-cell hyperplasia. About 50% of patients with MTC manifest pheochromocytomas (usually late in life), and
20% of patients have hyperparathyroidism.

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77
Q

Which Abx is most effective in pouchitis?

A

Cochrane Reviews suggest that ciprofloxacin is a more effective treatment for acute pouchitis than other antibiotics like metronidazole

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78
Q

In long term NB, what is the rate of secondary malignancy?

A

second malignancies in 3.5% of patients at 25 years and 7% at 30 years.
The most common second malignancy is thyroid cancer, followed by renal cell cancer

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79
Q

What is the risk of malignancy in UDT compared to general population?

A

Historically 40-60 times, recent reports 2-8 times

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80
Q

What is the risk of malignancy in intra abdominal compared to other types of UDT?

A

The risk of cancer of an intra-abdominal testicle is about x4-5 four to five times that of other undescended testicles.

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81
Q

What are the types of malignancies that develops in UDT?

A

Seminoma is the predominant cancer that develops in uncorrected undescended testicles while
embyronal tumors or teratocarcinoma make up the majority of cancers found in testicles that have undergone orchipexy.

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82
Q

which mechanism an oscillatory ventilator provides its protection against lung injury as compared to conventional ventilators ?

A

minimizing tidal volume
A high frequency ventilator uses small tidal volumes delivered at a fast rate while maintaining an appropriate end expiratory pressure to keep an optimal amount of alveoli open

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83
Q

What factors increase long term lung morbidity in CDH?

A

The need for patch repair is associated with a x3 three-fold higher risk and
a right-sided hernia a x2 two-fold higher risk of bronchopulmonary dysplasia in patients who survive past thirty day

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84
Q

What percentage of ovarian torsion are due to malignancy ?

A

Twenty percent of patients presenting with ovarian torsion will have an underlying ovarian neoplasm

Five percent of ovarian teratomast will present with torsion.

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85
Q

What percentage of incidental adrenal masses in children are malignant?

A

One third

adrenalectomy is warranted

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86
Q

What is the risk of oesophageal CA in OA patient compared to general population?

A

X50
The risk of a patient with esophageal atresia developing carcinoma of the esophagus may be up to fifty times greater than that of the general population

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87
Q

What is the incidence of right aortic arch and OA?

A

between 4 and 16%.
Patients with a right-sided arch are more likely to have:
a long gap atresia,
more likely to have a cardiac anomaly,
twice as likely to have perioperative complications and
ten times as likely to have a vascular ring (38 vs. 3.8%).
In cases associated with a vascular ring, left thoracotomy allows for division of the patent ductus which facilitates esophageal mobilization.

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88
Q

In sickle cell disease what are the indications of splenectomy?

A

1- acute splenic sequestration crisis; Splenectomy is indicated after one major sequestration crisis (Hg decrease more than 3 g/dl) or multiple minor crises (Hg drop less than 3 g/dl).
2- hypersplenism
3- splenic abscess
4- massive splenic infarction

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89
Q

What are the outcomes of anal sphincter Botox injection?

A

early improvement rates of 50-80%, and a long term success in over 50%.

90
Q

In H type fistula what is the incidence of RLN neuropraxia?

A

The most common complication is recurrent laryngeal nerve neuropraxia, (50%) and usually resolves over time.

Fistula is normally found between C5 &T2

Prone tube esophagrams is described

91
Q

What is the definition of bowel dilatation on antenatal scans?

A

bowel >6mm in maximal diameter on second trimester ultrasound. By 35 weeks gestation, bowel may be dilated up to 25-30 mm.

92
Q

What is the defect in CHI?

A

abnormality of the KATP channel encoded by the sulfonylurea receptor 1 (SUR1) and Kir6.2 genes.

Recessive mutations cause diffuse HI, whereas loss of heterozygosity together with inheritance of a paternal mutation causes focal adenomatous HI.

93
Q

What is the diagnostic tool of choice for CHI?

A

PET-CT scan using 18-Fluoro-L- DOPA has become the diagnostic procedure of choice

94
Q

What is the normal pulmonary shunt fraction?

A

Less than 5%

95
Q

What is the incidence of malignancy in operated and non operated choledocal cysts?

A

In non operated 9.9% (half bile duct and half gallbladder CA, from a Korean study)
In excised choledocals incidence of cancer development 0.6%, and is higher than the general population
Cholangiocarcinoma is the most common malignant tumor associated with choledochal cyst, and the most common site of involvement is the hepatic duct at or near the choledochoenteric anastomosis,
followed by the intrahepatic duct.

96
Q

Does routine Frusemide administration in neonate improved PDA?

A

No, Furosemide is associated with a potential increased risk of PDA
Also the risk of nephrocalcinosis and nephrolithiasis is increased, as is the risk of cholelithiasis

97
Q

What is the management of a seizure following a phosphate enema?

A

intravenous calcium administration
Retention of the enema can cause hypertonic dehydration, systemic hyperphosphatemia and resultant hypocalcemia, particularly in children with predisposing factors
Aggressive fluid hydration is important, but in the setting of seizures likely due to hypocalcemia

98
Q

What is the incidence of HD in colonic atresia?

A

2%
Colon atresia occurs in 1 in 20,000 births, representing 2-10% of all intestinal atresias. remove the bulbous colon just proximal to the atresia since the motility and function of this segment may be poor.

Non-fixation of the distal colon may predict the presence of concomitant Hirschsprung’s disease in colon atresia

99
Q

In cloaca what is the continence rate?

A

In Pena’s series, voluntary bowel movements were achievable in 66% of patients with a channel less than three cm, and voluntary urinary control was seen in 73% of patients.
In patients with common channels greater than three cm, urinary control was seen in only 26% and voluntary bowel function was achieved in only 34% of patients

100
Q

What is the most common cause of renal cyst disease in infants?

A

MCDK

101
Q

What is the most common cause of unilateral neonatal hydronephrosis?

A

PUJO

102
Q

I Wilm’s what is the rate for synchronous and metachronus tumours?

A

proportion of synchronous bilateral tumors among all nephroblastoma patients ranges from 4.4 to 7.0% while that of
metachronous tumors is 1.0–1.9%

103
Q

In massive haemothorax what the volumes that indicate surgical intervention?

A

More than 15ml/kg initial drainage or more than 2-3ml/kg/hr continuous drainage

104
Q

What is the most frequent complication of conservative liver trauma?

A

Bleeding

Bile leak

105
Q

The most common complication of non operative management of renal trauma?

A

Urinoma

106
Q

Lichen amyloidosis is a manifestation of which endocrine neoplasia?

A

MEN2A

107
Q

What is the likelihood of having a second child with CDH?

A

1-2%

108
Q

What stage of lung development is mostly affected by CDH?

A

Cannalicular

Embryonal -> pseduoglandular -> Cannalicular -> saccular -> Alveolar

109
Q

What is the most common site of oesophageal foreign body impaction?

A

The thoracic inlet, which is the area between the clavicles on cxr, this is the site of change between skeletal and smooth muscles and it’s where the cricopharyngeus sling is at C6

110
Q

What is the most common CXR finding after aspiration of foreign body?

A

Unilateral hyperlucency 30%
Radio-opaque FB 24%
Atelectasis 21%
Normal 15%

111
Q

What’s the most common site of aspirated FB?

A

Right main bronchus 55%
Left main bronchus 19%
Trachea 17%
Coughing is the most common symptom 73%

112
Q

What’s the incidence of OA in cleft patient?

A

20-36%
Cleft repair is through right extrapleural thoracotomy
Two approaches: anterior through the trachea or
Lateral through the trachea-oesophageal groove (higher incidence or RLN injury)

113
Q

In acid ingestion, which part of the GI tract is most affected?

A

The antrum of the stomach

114
Q

What’s the incidence of oesophageal CA after caustic injury?

A

5%

115
Q

What’s the incidence of stricture following caustic injury?

A

5% grade 1-2

90-100% G3

116
Q

What is the incidence and peak of reflux in infants?

A

2/3 at 4-5 months
5% by 12 months
75% on Neuroimpaired children
The peak incidence is around 4months of age

117
Q

What is the O2 saturation in the fetal aorta?

A

60-65%

118
Q

What is the most common site of aortic coarctation?

A

Juxtaductal

119
Q

Where do the different types of ASDs originate from?

A

Ostium secundum: septum premium
Sinus venous: junction of SVC with right atrium
Ostium premium: endocardial cushions
Foramen ovale: septum secundum

120
Q

Partial anomalus pulmonary venous return is mostly associated?

A

Sinus venosus ASD

121
Q

Which types of VSD does NOT close spontaneously?

A

Inlet

Conal (supracristal)

122
Q

What is the most common type of TAPVR?

A
Supracardiac type (45%) non obstructed
In obstructed TAPVR; infra cardiac is most common
123
Q

Where does left SVC drain?

A

Commonly to the coronary sinus

Less commonly to the roof or LA (Raghib association)

124
Q

What is the incidence of genital anomalies in ectopic kidneys?

A

15% in males

75% in females

125
Q

What is the risk increase of Wilm’s tumour in horseshoe kidney?

A

1.5 - 8 fold increase

There is an association between horseshoe kidney and later development of AAA

126
Q

What is the incidence of solitary kidney in screened populations?

A

3:1000

127
Q

What is the radiation dose of DMSA scan ?

A

0.7 mSv = 1 abdominal X-ray

128
Q

What is the normal AP diameter of renal pelvis in neonate?

A

5-7 mm
Mild dilatation < 12-15 mm (likely reflux)
> 15mm possible PUJO and MAG3 is warranted

129
Q

What is the normal flora of the urethra?

A

Female: E-Coli
male: E-Coli and Proteus

130
Q

What’s the incidence of VUR?

A

1% of healthy

30-40% of children investigated for UTI

131
Q

In VUR what is the incidence in offspring and sibling of affected individuals?

A

66% in offspring more common in females

25-33% of siblings

132
Q

How do you estimate bladder capacity in children?

A

<2 years = weight x 8

>2 years = age+2 x 30

133
Q

What is the incidence of nocturnal enuresis?

A

15-20% in 5years old children
Initially more common in boys but evens out in early adolescence
Risk of NE: 40% if one and 70% if both parents had NE

134
Q

What’s the genetic insult in Currarino?

A

Deletion in Ch7

135
Q

What’s the relation between sacral agenesis and gestational diabetes?

A

1% of gestational diabetic mother give birth to infants with sacral agenesis
16% of sacral agenesis are born to gestational diabetic mothers

136
Q

What is the incidence of cryptorchadism in PUV?

A

13%

5% are bilateral

137
Q

What is the risk of recurrent UTIs in males with VUR?

A

30% uncircumcised

10% in circumcised

138
Q

What governs the cremasteric reflex?

A

The genitofemoral nerve

139
Q

What enzymes/ proteins are elevated in 21 hydroxylase, 11B hydroxylase and 3B hydroxysteroid dehydrogenase deficiency?

A

21 hydroxylase: 17 hydroxy progesterone
11B hydroxylase: deoxycorticosterone
and 3B hydroxysteroid dehydrogenase: 17 hydroxy pregnenolone and dehydroepiandrosterone

140
Q

What is the risk of bladder exstrophy in siblings and offspring?

A

1: 100 in families
1: 70 in offspring of affected individuals

141
Q

At which phase of menstruation do haemorrhagic ovarian cysts develop?

A

Luteal phase (may represent persistence of the corpus luteum)

142
Q

What are the prevalence of associated anomalies in DA?

A

66% multi systems
25% cardiac
33% down’s
10% OA

143
Q

When does the thyroglossal cyst involute?

A

Weeks 8-10

144
Q

What is the commonest single suture craniosynostosis?

A

Sagittal synostosis 40-60%
Coronal 20-30%
Metopic < 10%
Lambdoid

145
Q

Which type of chiari malformation accompany spina bifida?

A

Type II (Arnold-Chiari)

146
Q

What’s the difference between maternal mirror syndrome and HELLP syndrome?

A

In maternal mirror syndrome LFTs, platelets and coagulation are normal
HELLP; haemolytic anaemia, elevated liver enzyme, low platelet.

147
Q

What is the incidence of NEC and cryptorchidism in gastroschisis?

A

NEC 18.5%
Cryptorchidism 15-30%
GORD 16%

148
Q

What is the incidence of inguinal hernia?

A
3-5%
Preterm 10-30%
Right side 60%, bilateral 10%
Male twins 10.6%, female twins 4.1%
Metachronous 7.2%. Left 10.2%, right 6.3%
149
Q

What’s Litter’s and Amyand’s hernia?

A

Litter’s: Mickel’s in sac
Amyand’s: appendix in sac

Risk of incarceration in general 12-17%

150
Q

What medication is used in rat model of small bowel atresia?

A

Doxorubicin

151
Q

What is the most common association with intestinal duplication?

A

Spinal anomalies

Vertebral and GU anomalies

152
Q

What is the incidence of spinal communication in oesophageal duplication?

A

20%

25% have associated abdominal duplication

153
Q

Gastric hyper secretion result following resection of which portion of the GI tract?

A

Jejunum; intestinal hormones suppressing gastric secretions are produced in the jejunum

154
Q

In HD what’s the prevalence of long segment and TCA?

A

10% each

155
Q

What factors are contributed to the pathology of HC enterocolitis?

A

Alteration in mucin composition
Decreased expression of MUC2 gene
Stasis
Reduction in IG A, T lymphocytes and gut associated lymphoid tissue

156
Q

What is the most frequent urological malformation with ARM?

A

Absent kidney: 11%

The most common urology problem is VUR: 21%

157
Q

What is the GFR at birth?

A

15-20 in term

10-15 in preterm ml/min/1.7m2

158
Q

How do you calculate GFR?

A

Estimated in a range between:

33x hight/creatinine) to (40x height / creatinine

159
Q

What is the incidence of associated anomalies in horseshoe kidney?

A

80% other anomaly
80% PUJ (L>R)
5% isthmus at upper poles

160
Q

In VUR what is the risk of offspring and sibling developing reflux?

A

Offspring 60%
Sibling 25%
25% shows scarring on DMSA following a symptomatic VUR

161
Q

What is ambiguous genitalia, mesangial sclerosis and renal mass?

A

Denys Darsh’s syndrome

WT1 mutation on 11p13

162
Q

Which anomaly of Müllerian duct anomalies has the highest risk of reproductive complications?

A

Septate uterus ; failed resorption of the central septum

163
Q

What is the subset gene mutation in EB that present with associated anomalies?

A

α6β4 integrin in epidermolysis bullosa with pyloric atresia,
plectin in epidermolysis bullosa with muscular dystrophy

164
Q

What are the associated anomalies with microgastria?

A

VACTREL

Asplenia, malrotation, situs inversus, cleft, Pierre Robin

165
Q

What procedure is used to augment the stomach in microgastria?

A

Hunt-Lawrence pouch

166
Q

What type of small bowel atresia is most commonly associated with immunodeficiency?

A

Type IV multiple atresia

167
Q

What percentage of small bowel atresia present with jaundice?

A

A third 32%

168
Q

What is microtuble associated protein 2?

A

Is a marker for neuronal differentiation that stain ganglion cells in HD

169
Q

PJS Patient presenting with gynaecomastia, what do you investigate?

A

Underlying testicular or gynaecological malignancy

170
Q

In polyposis syndromes what are the increased risk malignancy found I Gardners and Turcot’s?

A

Gardner’s; hepatoblastoma, desmoid, thyroid, osteosarcoma and adrenal ca
Turcot’s; basal cell carcinoma, focal nodular hyperplasia and CNS malignancy

171
Q

What is the exclusive enteral nutrition in IBD?

A

Elemental or polymeric diet (both have same results)
Diet is continued for 6 weeks at 120% caloric intake
It induces remissions 60-80% of cases

172
Q

What is the prevelance of AN hydronephrosis and what is the M:F ratio?

A

1-5%, 0.9% in the UK

M:F 5:1

173
Q

What is the pathogen in cat scratch disease?

A

Bartonella henselae

174
Q

What is the radiological ‘steeple sign’?

A

AP soft tissue radiograph of the neck shows the steeple sign in the subglottis region in cases of croup
Croup; para-influenza 1,2 or influenza A

175
Q

What is the most common post transplant lymphoma?

A

B cell lymphoma following heart or lung transplant

176
Q

What is the cause of Early (D1) cyanosis not responding to Prostaglandin and NO with hypotension and worsening perfusion?

A

Infracardiac total anomalous pulmonary venous return

177
Q

What are diffuse hepatic haemangiomas associated with?

A

Consumptive hypothyroidism due to expression of type III iodothyronin deiodinase

178
Q

What ethnicity is renal medullary cell carcinoma normally affect?

A

Young adults, black, with sickle cell trait

It was originally described as the 7th sickle cell nephropathy

179
Q

When is Leukovorin used?

A

Leukovorin (folinic acid) is used to limit the of methotrexate

180
Q

When are Neuroblasts identified in the Fetal adrenal glans?

A

10-12 weeks

They increase in number by the 20 week and diminish by the 3rd trimester

181
Q

What is the diagnostic feature of focal nodular hyperplasia on liver CT?

A

Early contrast enhancement and the presence of a central scar

182
Q

What is the risk of SCT recurrence?

A

25%

40% if coccyx not removed

183
Q

Which lymphoma is the most commonly present with mediastinal mass?

A

Lymphoblastic lymphoma

Rapid progression causing respiratory distress

184
Q

What’s the incidence of appendicitis in the general population?

A

8.6%

CF patients have a lower incidence 1.5-2%

185
Q

What is the recurrence rate of TGDC?

A

5% if central part of hyoid cartilage excised

40% if not

186
Q

What is the incidence of chia all atresia and where is it most common?

A

1:5000-8000

More common on the right

187
Q

What are the types of chow all atresia?

A

29% bony atresia;

71% mixed bony-membranous

188
Q

What is the risk of associated anomalies with chia all atresia? What are they?

A

50% of unilateral cases and 75% of bilateral cases are associated with a syndrome

189
Q

When do you repair choanal atresia?

A

Unilateral: non urgent around one year
Bilateral: establish airway and GASTRIC feeding immediately

190
Q

What is the difference between Hurler & Hunter syndromes?

A

Hurler: AR, MPS I, deficiency of α-L-iduronidase

Hunter: X Linked recessive, MPS II

191
Q

A rough guide formula to the lower limit (5th percentile) of normal systolic blood pressure is?

A

70 mm Hg + 2 x (Age in years)

192
Q

The most commonly diagnosed metabolic disorder in association with Reye syndrome?

A

medium-chain acyl coenzyme A dehydrogenase deficiency

193
Q

what are The causes of nephrogenic form of DI?

A

due to a lack of sensitivity of the kidneys (V2 receptor gene) to excess circulating ADH,
or abnormalities of the water channel proteins (aquaporins) involved in water transport

194
Q

What is the genetic defect in malignant hyperthermia?

A

AD

mutation of the ryanodine receptor in the sarcoplasmic reticulum of the skeletal muscle

195
Q

What is the congenital defect in congenital central hypoventilation syndrome (CCHS)?

A

HOX2B mutation

associated with Hirschsprung’s disease and tumors of neural crest origin

196
Q

What is the rate of ureteral duplication?

A

most common renal abnormality, occurring in approximately 1% of the population and 10% of children who are diagnosed with UTI

197
Q

What is the Risk of hypospadias is subsequent male siblings?

A

is 15%

198
Q

What is the criteria for diagnosis of abdominal compartment syndrome?

A
distention with an intra-abdominal pressure > 20 cm H2O (or 15 mm Hg) and at least two (2) of the following: 
1-oliguria or anuria, 
2-respiratory decompensation, 
3-hypotension or shock and 
4-metabolic acidosis.
199
Q

What are the associations of Morgagni’s hernia detected in infancy?

A

Morgagni hernia detected during infancy is often associated with congenital anomalies such as: congenital heart disease, Turner syndrome with coarctation, pectus carinatum, Prader-Willi syndrome, Cantrell’s syndrome, Noonan syndrome, Down’s, omphalocoele, retroperitoneal teratoma, and genitourinary anomalies

200
Q

What is the genetic mutation in alpha one anti trypsin deficiency?

A

autosomal-codominant condition with more than 120 alleles identified
mutations in the SERPINA1 gene located in the long arm of chromosome 14q

201
Q

What are the amino acids that conjugate bile acids?

A

bile acids cholic and chenodeoxycholic acids, are conjugated to an amino acid (glycine or taurine)

202
Q

What is the incidence of biliary atresia?

A

1 in 10,000-20,000 births

203
Q

Standard chemotherapy for hepatoblastoma?

A

The cisplatin/5-fluorouracil (5-FU)/vincristine (VCR) combination is regarded as standard chemotherapeutic treatment in hepatoblastoma

204
Q

What is the most common type of paratesticular RMS?

A

The most common histology is embryonal (spindle cell variant)

205
Q

What is the imaging modality of choice in gastronomy?

A

Somatostatin-receptor scintigraphy (SRS) has a sensitivity of 70-90%, which is more than that of any other imaging procedure in gastrinoma

206
Q

In sentinel lymph node biopsy, what tracer is commonly used?

A

usually Tc99m sulfur microcolloid

207
Q

In cellular mesoblastic nephroma, what is the genetic insult and characteristics?

A

The appearance is similar to that of infantile fibrosarcoma. Indeed both tumors share morphologic as well as genetic abnormalities [t(12;15)(p13;q25) translocation which results in ETV6-NTRK3gene fusion]
the cellular type presents later, usually after age 3 months. It demonstrates more aggressive imaging characteristics

208
Q

What is the mutation in Rabdoid tumour of the kidney?

A

Mutation SMARCB1 / 22q11.2 HSNF/INI 1

209
Q

In rabdoid tumor of the kidney, what is the risk of calcification, fluid collection and metastasis?

A
  • Calcification 10% Wilms, more common in MRTK
  • fluid collection 9% Wilms, 25% CCSK, 14% MBN & 71% MRTK
  • 81% lung mets at presentation
  • About 15% of renal rhabdoid tumors are associated with embryonal tumors in the posterior cranial fossa such as medulloblastoma.
210
Q

What is the chemotherapy recommend for rhabdoid tumour of the kidney?

A

ifosfamide-carboplatin-etoposide (ICE) or ifosfamide-etoposide (IE) alternating with vincristine-doxorubicin-cyclophosphamide (VDC)

211
Q

what is the main secretion of pheochromocytomas in children?

A

Most pheochromocytomas in children predominantly produce norepinephrine

212
Q

What pathologies raise urine dopamine?

A

high urine dopamine (DA) levels are found in the following conditions, pheochromocytoma, NB, Costello syndrome, Leukemia, Menkes disease & Rhabdomyosarcoma of the bladder

213
Q

What are the common congenital anomalies associated with Wilms tumor?

A

cryptorchidism, a double collecting system, horseshoe kidney, and hypospadias

214
Q

How does Mesna work?

A

Inactivates acrolein and prevents urothelial toxicity without affecting cytostatic activity

215
Q

What is Filgrastim?

A

G-CSF; These act as a hematopoietic growth factor that stimulates the development of granulocytes. They are used to treat or prevent neutropenia in patients receiving myelosuppressive cancer chemotherapy and to reduce the period of neutropenia associated with bone marrow transplantation.

216
Q

What’s the chemotherapy for germ cell Tumours?

A

First-line therapy includes; bleomycin, etoposide, and cisplatin (BEP)

217
Q

What’s cystic testicular tumour seen in the first 6 months of life?

A

Granulosa cell tumors are not hormonally active, and are benign. They are often cystic, and usually seen in the first year of life, and often in the first 6 months. They make up about one-third to one-half of neonatal testicular tumors. Associated Y chromosome abnormalities, mosaicism, and ambiguous genitalia are common.

218
Q

What are the associations of Sertoli cell testicular tumour?

A

The Large-cell calcifying Sertoli tumor occurs both as a part of the Carney complex and in patients with Peutz-Jeghers syndrome

219
Q

gynecomastia / impotence and testicular mass?

A

Sertoli cell tumour

Charcot-Bottcher filaments (perinuclear aggregates of intermediate filaments) are pathognomonic

220
Q

What is tetralogy of Fallout?

A

VSD
Sub pulmonary stenosis
Overriding aorta
Right ventricle hypertrophy

221
Q

What is associated with interrupted aortic arch?

A

DiGeorge 22q11.2 deletion

222
Q

What does the neonatal blood screening involve ?

A

Sickle cell disease (1:2000)
CF (1:2500)
Hypothyroidism (1:3000)
phenylketonuria (PKU) (1:10,000)
medium-chain acyl-CoA dehydrogenase deficiency (MCADD) (1:10,000)
maple syrup urine disease (MSUD) (1:100,000)
isovaleric acidaemia (IVA) (1:100,000)
glutaric aciduria type 1 (GA1) (1:100,000)
homocystinuria (pyridoxine unresponsive) (HCU) (1:100,000)