Paeds 2 - GI, Liver, Renal Flashcards

1
Q

What does dark green bile-stained vomit indicate?

A

Intestinal obstruction

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2
Q

What condition is indicated by projectile non-bilious vomiting in the first few weeks of life, up to an hour after feeding?

A

Pyloric stenosis

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3
Q

What condition is indicated by red-currant jelly stool?

A

Intussuception

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4
Q

What is the cause of gastro-oesophageal reflux in infants?

A

Inappropriate relaxation of the lower oesophageal sphincter as a result of functional immaturity

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5
Q

What is a possible complication of GO reflux?

A

Chest infection from pulmonary aspiration
Oesophageal strictures
Barrett’s oesophagus
Failure to thrive

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6
Q

How is refractory (no spontaneous resolution) GO-reflux treated?

A

Overnight oesophageal pH study - acid in oesophagus for more than 4% of the day
Thickening agents, small feeds, nurse upright (head up slope of 30 degrees and prone)
Gaviscon
Ranitidine
Domperidone (prokinetic drug)
Omeprazole
Nissen fundoplication

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7
Q

What is the cause of pyloric stenosis?

A

Hypertrophy of the pyloric muscle

Idiopathic

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8
Q

What metabolic abnormality is seen in patients with pyloric stenosis?

A

Hypochloraemic hypokalaemic metabolic alkalosis

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9
Q

What can be found on investigation in a patient with pyloric stenosis?

A

Test feed - visible gastric peristalsis, pyloric tumour
Abdo exam - pyloric mass (olive-like) in RUQ
USS - confirms or excludes diagnosis

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10
Q

What is the treatment of pyloric stenosis?

A

IV 0.45% saline, 5% dextrose, 20mmol/L K+ supplements
Withold feeds and empty stomach with NGT
Ramstedt’s pyloromyotomy

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11
Q

What presentation is characteristic of infant colic?

A

Paroxysmal inconsolable crying with knees drawn up

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12
Q

What is Meckel’s diverticulum?

A

Ileal remnant of the vitello-intestinal duct containing ectopic gastric mucosa or pancreatic tissue

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13
Q

What is the rule of 2s with Meckel’s diverticulum?

A
2% of the population
2 inches long
2 feet proximal to ileo-caecal valve
2 years of age onset
2 types of ectopic tissue
2% symptomatic
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14
Q

What is the symptom of Meckel’s diverticulum?

A

Painless severe rectal bleeding

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15
Q

How is Meckel’s diverticulum diagnosed?

A

Technetium scan - increase uptake by ectopic gastric mucosa

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16
Q

What is the GI anatomy in malrotation?

A

Caecum goes from RIF to right hypochondrium
SI has a narrow base (volvulus)
Duodenum covered by fibrous bands of Ladd

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17
Q

What is the presentation of intestinal obstruction in days 1-3 of life?

A

Dark green bilious vomiting
Abdo pain and distension
Blood and mucous in stools
Circulatory collapse

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18
Q

How is malrotation diagnosed?

A

Urgent upper GI contrast study

AXR - double bubble

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19
Q

What are the symptoms of appendicitis?

A

Anorexia, vomiting, pain initially central –> RIF
Tenderness and guarding over McBurney’s point
Fever

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20
Q

How is appendicitis diagnosed?

A

Abdominal X-Ray - faecoliths

Ultrasound - thickened appendix with increased blood flow

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21
Q

What is intussusception?

A

The invagination of proximal bowel into a distal segment

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22
Q

What is the most common location for intussusception?

A

Ileum passing into caecum through ileo-caecal valve

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23
Q

What are the risk factors for intussusception?

A

Viral infection leading to enlargement of Peyer’s patches (lymphoid hyperplasia)
Childhood leukaemia/small bowel lymphoma

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24
Q

What investigations are required in the diagnosis of intussusception?

A

Abdo exam - palpable sausage shaped mass
AXR - distended small bowel, absence of gas distally, outline of intussusception
AUS - target sign

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25
What are the symptoms of intussusception?
Paroxysmal colicky episodes, refusal of feeds, bile stained vomiting, redcurrant jelly stool, abdo distension Child falls asleep between episodes
26
What is the gold standard of treatment of intussusception?
Rectal air insufflation under fluoroscopic control
27
What is an abdominal migraine?
Functional abdominal pain and functional headaches. Can have vomiting
28
What is the eradication regime for h.pylori infection?
Amoxicillin/clarithromycin, metronidazole
29
What is the treatment of functional dyspepsia?
Hypoallergenic diet
30
What is the most common gasteroenteritis causing organism for under 2s?
Rotavirus
31
Blood in the stool signifies a bacterial cause for gastroenteritis, such as
E.coli Campylobacter jejuni Shigella
32
What are the complications of gastroenteritis in children?
Dehydration | Malnutrition
33
What is the management of a child with gastroenteritis?
Antidiarrhoeals and antibiotics not routinely used | Self resolution
34
When are antibiotics prescribed for gastroenteritis?
Sepsis Extra-GI spread of infection Malnutrition Immunocompromised
35
What is post-gastroenteritis syndrome?
Following episode of gastroenteritis, intro of normal diet produces watery stools, and temporary lactose and dietary intolerances.
36
Why are infants at a greater risk for dehydration?
Greater surface area:weight ratio and greater insensible losses
37
What are the symptoms of dehydration in infants?
``` Decreased urine output Decreased consciousness Sunken fontanelle Dry mucous membranes Prolonged capillary refill Pale/mottled skin Cold extremities ```
38
What are the three degrees of dehydration in infants?
No clinically detectable dehydration (<5% body weight loss) Clinical dehydration (5-10%) Shock (>10%)
39
What is the most common type of dehydration?
Isonatraemic
40
Children with diarrhoea can suffer with what type of dehydration and why?
Hyponatraemic - they drink water so there is a greater net loss of Na than water
41
What is a consequence of hyponatraemic dehydration?
Water shifts from extracellular to intracellular compartment, increasing brain volume and causing seizures
42
What are the causes of hypernatraemic dehydration?
High insensible losses (high fever, hot environment)
43
How do you treat shock from dehydration?
Rapid infusion of 0.9% NaCl solution
44
What is the presentation of malnutrition?
Abnormal stools Failure to thrive Nutrient deficiencies
45
What are three causes of malnutrition
Biliary atresia Short bowel syndrome Exocrine pancreatic dysfunction Coeliac disease
46
When is coeliac disease most likely to present?
8-24 months
47
How is coeliac disease diagnosed?
IgA, IgA tissue transglutaminase, anti-gliaden and endomysial antibodies
48
What vitamin deficiencies are most likely to be seen in coeliac disease?
Vitamin B12, Iron Folate
49
What is characteristic of coeliac disease in the small bowel?
Flat mucosa, villous atrophy, crypt hypertrophy
50
What are the signs and symptoms of Crohn's disease?
``` Growth failure, abdominal pain, diarrhoea Delayed puberty Oral lesions Perianal skin tags Uveitis Erythema nodosum ```
51
What is the treatment of Crohn's disease?
Glucocorticoid monotherapy for flares: prednisolone | Enteral nutrition if concerns about growth
52
What is the difference between Crohn's disease and Ulcerative colitis?
Crohn's - transmural inflammation mouth --> anus | UC - inflammation confined to colon (90% children pancolitis)
53
What are the red flags for constipation in children?
``` Sacral dimple Abdominal distension Decreased growth Abnormal lower limb neurology Perianal fistulae/abscess/fissures Fever ```
54
At what age should children have a comparable stool pattern to adults?
4 years | 4/day in first week, 2/day by 1 year
55
How would you manage a child with constipation?
Fluid and diet advice Osmotic laxative/movicol If no effect, stop and add senna
56
What are the causes of constipation in babies?
``` Hirschprung's disease Anorectal abnormalities Hypothyroidism Hypercalcaemia Dehydration ```
57
Which large bowel nerve plexuses are missing ganglion cells in Hirschprung's disease?
Myenteric and submucosal
58
What is the cause of Hirschprung's?
Defect in the craniocaudal migration of neural crest cells in the first 12 weeks of gestation
59
What is the gold standard for diagnosis of Hirschprung's?
Suction rectal biopsy to demonstrate absence of plexuses or large AChE positive nerve
60
How does Hirschprung's disease present?
Failure to pass meconium, DRE produces watery stool Bowel and abdo distension Bile stained vomiting Enterocolitis
61
How is Hirschprung's disease treated?
Anastamose innervated bowel to anus
62
What is the main laboratory tool used to assess the kidneys and urinary tract?
PCr (plasma-creatinine ratio) | Then eGFR
63
What is an MCUG?
Micturating cystourethrogram - contrast introduced to bladder via urethral catheter to visualise anatomy
64
What is Potter syndrome and how is it caused?
Deficiency of amniotic fluid from renal agenesis or bilateral multicystic dysplastic kidney
65
How are most congenital renal conditions diagnosed?
Antenatal ultrasound
66
What is the most common inherited cystic kidney disease?
Autosomal dominant polycystic kidney disease - cysts on both kidneys fill with fluid and enlarge, compress the renal parenchyma and compromise function
67
What is the treatment of ADPKD?
RRT: dialysis or transplant
68
What is ARPKD?
Underdeveloped kidneys leading to neonatal death, or hypertension and haematuria in childhood
69
What is the cause of multicystic dysplastic kidney?
Failure of the union of the ureteric bud and nephrogenic mesenchyme.
70
What are the characteristics of multicystic dysplastic kidney?
Ureteric atresia, non functioning/no renal tissue with no connection to the bladder
71
Name two other congenital malformations of the kidney
Horseshoe kidney, duplex kidney
72
What is a risk factor for vesicoureteric reflux?
Family history
73
Why do children with VU reflux have recurrent UTIs?
Renal or ureteric scarring (especially if intrarenal reflux)
74
What is reflux nephropathy?
Shrunken, poorly functioning, scarred renal tissue from recurrent infection
75
When should you investigate for VUR and what would this entail?
Atypical/recurrent UTIs Ultrasound MCUG to diagnose DMSA to look for renal scarring
76
How is VUR managed?
Prophylactic antibiotics | Endoscopic injection/surgery
77
What are three causes of primary nocturnal enuresis?
UTI Polyuria Faecal retention severe enough to cause bladder neck dysfunction
78
How is primary nocturnal enuresis treated?
Star chart, enuresis alarm, desmopressin for short term treatment
79
What are three common organisms associated with UTI?
E.coli (95%) Klebsiella Pseudomonas
80
How is UTI diagnosed?
Pyuria (neutrophils) | Leucocyte esterase and nitrites in the urine
81
What are the differing symptoms of UTI in infants and older children?
Infants - poor feeding, fever, vomiting, offensive urine | Children - dysuria and frequency, loin pain, fever and rigors, v+d, offensive/cloudy urine
82
A triad of proteinuria, hypoalbuminaemia, and oedema, corresponds to what condition?
Nephrotic syndrome
83
Give three causes of primary nephrotic syndrome
``` Minimal change disease Focal segmental glomerulosclerosis Membranous glomerulonephritis Membranoproliferative glomerulonephritis Rapidly progressive glomerulonephritis ```
84
Give three causes of secondary nephrotic syndrome
SLE Diabetic nephropathy Syphillis Multiple myeloma
85
What are the signs of nephrotic syndrome?
Periorbital oedema, particularly on waking Scrotal/vulval/leg oedema Breathlessness from pleural effusion and ascites Foamy urine Anaemia
86
What investigations would you commence in suspected nephrotic syndrome?
Urine protein dipstick MCS or MSU UEs, electrolytes, creatinine Biopsy if atypical picture
87
What level of protein: creatinine in the urine is likely to cause hypoalbuminaemia?
<600mg (normal is less than 20mg)
88
What is the management of steroid sensitive nephrotic syndrome?
PO prednisolone 60mg/m2, 8 weeks
89
What are the characteristics of steroid resistant nephrotic syndrome?
Increased BP Haematuria Decreased renal function
90
What is the management of steroid resistant nephrotic syndrome?
Manage oedema with diuretics, salt restriction, and ACEIs
91
Who is the typical patient with nephrotic syndrome?
Male, 2-5 years
92
What are the complications of nephrotic syndrome?
Hypovolaemia Thrombosis Infection Hypercholesterolaemia
93
What are the causes of persistent proteinuria in children?
Hypertension Reduced renal mass Glomerular abnormalities
94
What investigations should be carried out in a chid with haematuria?
Check for FH of stone formation and nephritis History of trauma MCS of MSU and bloods Renal biopsy if kidney function abnormal or persistent macroscopic haematuria
95
What are the symptoms of Henoch Schonlein Purpura?
``` Trunk-sparing, symmetrical skin rash on extensors and buttocks Knee and ankle arthralgia Periarticular oedema Abdominal pain Golmerulonephritis ```
96
Who is the typical patient with HSP?
Boys aged 3-10 years
97
What can precipitate HSP?
URTI
98
What is the treatment of HSP?
Analgesia and symptom control
99
What are three causes of acute nephritis?
IgA nephropathy Post-strep Vasculitis
100
What are the symptoms of acute nephritis?
Decreased urine output Hypertension that may cause seizures Periorbital oedema Haematuria and proteinuria
101
What is the treatment of acute nephritis?
Diuretics
102
Give three causes of haematuria in children
``` Nephritis Stones Infection Trauma Sickle cell disease Renal vein thrombosis ```
103
How is blood pressure measured in children?
Cuff over 2/3 the length of the upper arm
104
Define hypertension in children
Blood pressure above the 95th percentile for height, age, sex
105
Give three causes of hypertension in children
Renal parenchymal disease e.g. PKD Aortic coarctation Catecholamine excess (phaechromocytoma/neuroblastoma) CAH/Cushing's
106
What are the symptoms of hypertension in children?
Vomiting Headaches Hypertensive retinopathy Facial palsy
107
What are the predisposing causes for renal calculi?
UTI Structural/metabolic abnormalities Proteus infection
108
What is nephrocalcinosis?
Deposition of calcium in renal parenchyma
109
How do renal calculi present?
Haematuria Loin to groin pain UTI Stone passage
110
What is the syndrome that causes generalized proximal tubular dysfunction?
Fanconi syndrome
111
Give three causes of Fanconi syndrome
``` Vitamin D deficiency Idiopathic Heavy metals Wilson disease Tyrosinaemia ```
112
Which molecules are lost to the urine in Fanconi syndrome?
Amino acids, Glucose, phosphate, bicarbonate, Na, K, Ca, urate
113
What is the presentation of Fanconi syndrome?
``` Polydipsia and polyuria Dehydration Hyperchloraemic metabolic acidosis Ricketts Failure to thrive Hypokalaemia Hypophosphataemia/hyperphosphaturia ```
114
What is the treatment of Fanconi syndrome?
Replace substances lost in urine
115
What are the three categories of AKI causes in children?
Prerenal, renal, post-renal
116
Name some renal causes of AKI
Glomerulo/interstitial/pyelonephritis HUS Renal vein thrombosis
117
What is the ultrasound finding in AKI?
Chronic - small kidneys | Acute - large bright kidneys
118
What is the presentation of AKI?
Oliguria Metabolic acidosis Hyperphosphataemia and hyperkalaemia
119
How would you manage a patient with AKI?
Severe acidosis/multisystem failure: dialysis Increased phosphate: calcium carbonate Metabolic acidosis: Sodium bicarbonate Increased potassium: calcium gluconate
120
What is the triad of symptoms in haemolytic uraemic syndrome?
Acute renal failure Microangiopathic (haemolytic) anaemia Thrombocytopenia
121
What infection is HUS usually secondary to?
GI infection with verocytotoxin producing e.coli
122
What is the prodrome in HUS?
Bloody diarrhoea and miserable
123
How is HUS managed?
Supportive therapy Isolation Dialysis
124
Define chronic renal failure
GFR<15ml/min/1.73m2
125
What are the symptoms of CKD?
``` Anorexia Polydipsia and polyuria Failure to thrive Bony deformities - renal rickets HTN ```
126
What is the most common cause of persistent neonatal jaundice?
Unconjugated hyperbilirubinaemia (resolves spontaneously)
127
What are the laboratory finding and symptoms in PNJ cause by liver disease?
Raised conjugated bilirubin (>20mcmol) | Dark urine, pale stools, bleeding, failure to thrive
128
Give three causes of liver disease producing PNJ
Biliary atresia Neonatal hepatitis Haemolytic anaemia Alagille syndrome
129
What are the symptoms of clotting dysfunction in liver disease?
Epistaxis Bruising Petechiae
130
What is biliary atresia?
Progressive destruction and absence of the extra-hepatic biliary tree and intrahepatic bile ducts
131
Why does portal hypertension occur in neonatal liver disease?
Hepatomegaly and splenomegaly
132
What is the gold standard diagnostic test for biliary atresia?
Operative cholangiography
133
What type of inheritance is alpha-anti-trypsin deficiency?
Autosomal recessive
134
Galactosaemia is caused by a lack of galactose-digesting enzymes. What are the symptoms?
Poor feeding, vomiting Jaundice and hepatomegaly When fed milk
135
What are the features of viral hepatitis?
Nausea and vomiting Abdominal pain and jaundice (70%) Lethargy Large tender liver and splenomegaly
136
What are the causes of acute liver failure in children?
``` Hepatitis (A, B, C) Paracetamol overdose Wilson disease Tyrosinaemia Reye syndrome ```
137
What can acute liver failure progress to?
Encephalopathy - irritability, confusion, drowsiness Cerebral oedema Haemorrhage from gastritis or coagulopathy Pancreatitis Sepsis
138
Elevation of what occurs in acute liver failure?
Transaminases Serum ammonia Bilirubin in late stages
139
What is the name of the condition characterized by an acute non-inflammatory encephalitis and fatty infiltration of the liver, and what is its cause?
Reye syndrome | Taking aspirin under the age of 12
140
How is chronic liver disease diagnosed?
``` Elevated total protein Hypergammaglobulinaemia Autoantibodies Low serum C4 Histology - liver biopsy ```
141
What is the most common liver abnormality in cystic fibrosis?
Hepatic steatosis
142
Thick, concentrated bile in cystic fibrosis can cause what?
Biliary fibrosis --> cirrhosis --> portal hypertension
143
What is the treatment of biliary cirrhosis in CF?
Urseodeoxycholic acid
144
The condition where copper accumulates in the liver, brain, kidney, and cornea from decreased caeruloplasmin synthesis is called what?
Wilson disease
145
How are oesophageal varices best diagnosed?
Endoscopy
146
What is a serious complication of liver disease?
Spontaneous bacterial peritonitis
147
What are the indications for liver transplant in children?
Severe unresponsive malnutrition Recurrent complications Growth failure Poor quality of life
148
What are the differentials for gastroenteritis?
Lactose intolerance Intussusception HUS (blood in stool and miserable)
149
What is the most common cause of AKI in children?
Haemolytic uraemic syndrome
150
What investigation confirms the diagnosis of HUS?
FBC and blood film Low Hb, low platelets Schistocytes (fragmented RBC)
151
How is pyloric stenosis diagnosed?
Test feed - hyperperistalsis, pyloric mass, projectile vomit
152
In what condition does perianal itching occur and why?
Lactose intolerance - acidic stool
153
What conditions is coeliac disease associated with?
``` Dermatitis herpetiformis Vitiligo Pernicious anaemia Hashimoto's thyroiditis Type 1 diabetes Small bowel lymphoma ```
154
What is the most common inflammatory bowel disease in children?
Crohn's disease
155
What is the most common location for Crohn's disease?
Terminal ileum
156
How does terminal ileum involvement in Crohn's disease lead to malabsorption?
Loss of bile salts
157
What are three presentations of Crohn's disease in children?
Weight loss with diarrhoea (bloody) and abdominal pain Growth failure with delayed puberty Toxic megacolon
158
What is seen on barium enema in Crohn's disease?
Thickening of wall Strictures Cobblestone mucosa Rose thorn ulcers
159
What is the treatment of Crohn's disease?
Elemental diet for 6 weeks to induce remission Sulfasalazine/mesalazine Steroids for relapses Azathioprine
160
In what condition are children born with blue sclerae and what is the pattern of inheritance?
Osteogenesis imperfecta | Autosomal dominant but can be sporadic
161
What is a Mongolian blue spot?
Blue/grey dermal melanocytosis
162
What is the most common cause of nephrotic syndrome?
Minimal change disease
163
What is HSP?
A type of IgA vasculitis
164
What is physiological jaundice and why does it occur?
Onset days 2-5 of life, self resolving | High RBC volume at birth, decreased RBC survival, decreased hepatic uptake
165
How long can breast milk jaundice last for?
2 weeks
166
What is kernicterus?
Dystonic cerebral palsy due to extreme hyperbilirubinaemia
167
What is the treatment of biliary atresia?
Kasai hepatoportoenterostomy
168
At what age should a child be able to walk, speak in 2 word sentences, and ride a tricycle?
Walk - 18 months 2 words - 2 years Tricycle - 3 years
169
When is hand preference shown in children?
2 years
170
What are the risk factors for recurrent UTI?
``` FH of VUR Spinal lesion Structural anomaly Constipation Dysfunctional voiding ```
171
What is a common cause of urinary obstruction in boys?
Posterior urethral valve
172
When should recurrent UTI be investigated further?
One or more UTIs first 6m | 2 or more UTIs over 6m
173
What is a DMSA scan and what is it used for?
Radioisotope that binds to proximal tubules | Used in recurrent UTI to detect renal parenchymal damage
174
What is the management of posterior urethral valves?
Cystoscopic ablation
175
What is a risk factor for necrotizing enterocolitis?
Prematurity
176
What causes hyperperistalsis in pyloric stenosis?
Stomach tries to push food past the obstruction
177
Obstruction proximal to the 2nd part of the duodenum produces what type of vomiting?
Non-bilious - bile has not entered the duodenum yet
178
What does a scaphoid abdomen suggest?
Diaphragmatic hernia | With bilious vomiting - malrotation and intussusception
179
What histological finding confirms minimal change disease?
Fused podocytes
180
What is seen on MCUG in VUR?
Gross dilatation of the ureter, pelvis, and calyces | Ureteral tortuosity
181
What is Rovsing's sign?
Palpation of the LIF produces pain in the RIF in appendicitis
182
What is seen on X-Ray in intussusception?
Alternating echogenic and hypoechogenic rings
183
What is the pathophysiology behind the hypochloraemic hypokalaemic metabolic alkalosis in pyloric stenosis?
Hydrogen chloride loss from vomiting results in hypochloraemic metabolic alkalosis Renal compensation by maximising bicarbonate reabsorption, exchanging K+ and Na+ ions for H+ ions
184
Midgut malrotation predisposes to...
Midgut volvulus
185
What are the symptoms of a midgut volvulus?
High intestinal obstruction at duodenal level that is rapidly followed by infarction of the entire midgut
186
What is the treatment of volvulus?
Laparotomy to untwist the volvulus
187
What is a common consequence of volvulus?
Massive intestinal necrosis (seen on second look laparotomy) leading to short gut syndrome requiring IV feeding
188
Apart from rectal air insufflation, what else is needed in the management of intussusception?
IV fluid Antibiotics Analgesia NGT
189
One third of babies with duodenal atresia have what genetic anomaly?
Trisomy 21
190
What is the presentation of duodenal atresia?
Bile stained vomiting
191
What is seen on AXR in duodenal atresia?
Double bubble sign
192
What is the treatment of duodenal atresia?
Side to side duodenoduodenostomy
193
What is the gold standard diagnosis of necrotizing enterocolitis and what is seen?
``` Abdominal X Ray Pneumatosis intestinalis (gas in intestinal wall) ```