Foregut dysmotility complicating persistent hyperinsulinaemic hypoglycaemia of infancy.
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Biomedical subjects
Publications and source records attributed to M D Stringer.
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AIM: To evaluate long term detailed pancreatic endocrine and exocrine function in children with persistent hyperinsulinaemic hypoglycaemia of infancy (PHHI) after 85-95% pancreatectomy. METHODS: Six children with PHHI between 0.9 and 12.7 years after pancreatic resection underwent clinical and investigative follow up at 1.0 to 14.9 years of age. One child with PHHI who had not had pancreatectomy was also assessed. Standard endocrine assessment, pancreatic magnetic resonance imaging (MRI), and detailed direct and indirect tests of exocrine pancreatic function were performed. RESULTS: Pancreozymin-secretin stimulation test results were normal in only one child, borderline in two, and deficient in four, one of whom requires daily pancreatic enzyme supplements. Pancreolauryl tests performed in three children were borderline in two and abnormal in the other. Only one child had low faecal chymotrypsin values. One child developed insulin dependent diabetes at 9 years and two children at 1.0 and 13.3 years require diazoxide to maintain normoglycaemia. MRI showed no major regrowth of the pancreatic remnant after resection (n = 5). CONCLUSIONS: Clinical evidence of endocrine or exocrine dysfunction has developed in only two patients to date, but detailed pancreatic function testing suggests subclinical deficiency in all but one of our patients with PHHI. Although 95% pancreatectomy results in postoperative control of blood glucose, subclinical pancreatic insufficiency is present on long term follow up and development of diabetes mellitus and exocrine failure remain ongoing risks.
AIM: To quantify the exposure of very low birthweight neonates to ionising radiation from diagnostic x-rays. METHODS: Retrospective analysis was made of all radiographs performed over 18 months in an integrated special care baby unit and regional neonatal surgical unit in a large teaching hospital of surviving inborn babies of very low birthweight (< 1500 g) admitted to the unit. RESULTS: Fifty five VLBW neonates were treated for a total of 3296 days and received 498 radiographs (median 5 per infant). The mean effective radiation dose was 0.04 mSv and the maximum for one infant was 0.54 mSv. CONCLUSIONS: The radiation burden in this group of neonates is low and the benefits of diagnostic radiographs far outweigh any potential radiation risks.
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Rectal excision for non-malignant conditions using a posterior sagittal approach is described in three patients. The technique allows excellent exposure of the rectum, meticulous haemostasis, minimal risk of pelvic nerve injury and accurate reconstruction of the pelvic floor without the need for drainage.
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A 14-year-old girl presenting with acute massive gastric dilatation secondary to duodenal obstruction by the superior mesenteric artery is described. The diagnosis was facilitated by contrast-enhanced abdominal computerized tomography. She was successfully treated by gastrostomy and subsequent duodenal derotation. This unique presentation of the superior mesenteric artery syndrome is discussed.
An 8-year-old boy presented with acute pancreatitis and was found to have a fenestrated duodenal web (windsock) and associated anomalies. After partial excision of the web and duodenoplasty, he has remained well with no further symptoms.
Isolated gastroschisis in two children of unrelated mothers but with the same father is described. There was no evidence of consanguinity, abnormal karyotype or a specific teratogen in either case. Genetic factors may be involved in the aetiology of some cases of gastroschisis.
AIMS: To assess the long term morbidity and quality of life in survivors of gastroschisis. DESIGN: All babies born with gastroschisis between 1972 and 1984 and who survived more than one year were identified. Those who could be traced were questioned about their general health, growth, abdominal symptoms, cosmetic concerns, education, employment, and fertility. RESULTS: Of the 35 patients, two have died, seven could not be traced, and three declined to be interviewed. Twenty three subjects (70% of survivors) with a median age of 16 years (range 12-23 years) responded. Twenty two (96%) were in good health and overall growth was within normal limits. Eight subjects (35%) have had further surgery related to gastroschisis, including two for adhesive small bowel obstruction and three for scar complications. In 13 (57%), absence of an umbilicus caused distress during childhood. CONCLUSION: Most gastroschisis survivors can eventually expect normal growth and good health. Adhesive bowel obstruction is an uncommon, but potentially late, complication. The umbilicus should be conserved during gastroschisis repair.
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A six year old boy underwent extensive investigation for recurrent abdominal pain and was found to have a persistently raised serum amylase. Endoscopic retrograde cholangiopancreatography was normal and macroamylasaemia was excluded. Serum amylase concentrations were found to be raised in other family members spanning three generations, all of whom were asymptomatic. Clearance studies suggested no evidence of a renal tubular defect and serum lipase concentrations were normal. This is the first report of apparently familial hyperamylasaemia and the mode of inheritance is consistent with an autosomal dominant pattern.
BACKGROUND: Femoral hernias are rare in children, accounting for fewer than 1 per cent of all paediatric groin hernias. Misdiagnosis is common and a source of complications. There is no consensus on the age and sex distribution or the optimum method of repair. METHODS: A personal experience of four children with femoral hernia is reported together with an institutional review of a further ten hernias encountered during the past 11 years. RESULTS: Peak incidence was between 5 and 10 years of age. Misdiagnosis was common, partly because of the variability in presenting symptoms and signs. In this series, boys were more commonly affected but a literature review indicated a similar sex incidence. CONCLUSION: A femoral hernia should be positively excluded if the operative findings at inguinal exploration are inconsistent with the preoperative signs and in any child with a suspected recurrent inguinal hernia. Excision of the sac and repair of the femoral canal is curative.
BACKGROUND: Perianal sepsis is a relatively common surgical problem in children and yet the contribution of rare aetiological factors, the frequency of fistula formation complicating perianal abscess, the significance of bacteriological findings and the optimum treatment of fistula in ano are poorly understood. METHODS: A consecutive series of 58 children (51 boys, seven girls) with a perianal abscess or fistula attending a tertiary referral centre over a 4-year period was reviewed. RESULTS: Thirty-eight children (34 boys, four girls), ranging in age from 1 month to 12 years, presented with an abscess. After treatment, three developed a further abscess but only four developed a fistula. Fistula formation only occurred in those with enteric bacterial infection. Of 24 children with a perianal fistula five required further surgery for recurrence, four of whom were initially treated by fistulotomy. In most patients, the fistula was simple, low and direct, and treatment by fistulectomy resulted in significantly fewer recurrences. Apart from Crohn's disease, identifiable aetiological factors were rare. CONCLUSION: Perianal sepsis is most common in otherwise healthy boys under 2 years of age. Abscess is best treated by incision and drainage, and fistulectomy is successful for the small proportion who develop a fistula.
The accuracy of the modified Alvarado score was assessed prospectively in the preoperative diagnosis of acute appendicitis in children. A consecutive series of 118 patients (54 boys, 64 girls) with acute abdominal pain was studied prospectively over a 6 month period. Appendicitis was confirmed in 38 of 43 children undergoing appendicectomy, giving a false-positive appendicectomy rate of 11.6%. No child under active observation was subsequently found to have a perforated appendix. The overall sensitivity of a modified Alvarado score of > or = 7 was 76.3% and its specificity was 78.8%. Current clinical practice is more accurate than the modified Alvarado score in the diagnosis of acute appendicitis in children.
Traumatic rupture of a splenic epidermoid cyst is a rare but recognised cause of "acute abdomen" in a child. We describe such a case occurring after very minor trauma, show the influence that imaging had in making an accurate diagnosis, and discuss how this influenced clinical management.
During the last 4 years, three infants have presented with finger-tip injuries secondary to entrapment in woollen/synthetic mittens. The accident happened at home in one case but the other two occurred in different neonatal units. Spontaneous amputation of the terminal phalanx of the index finger occurred in two patients but in the other there was complete healing. This problem may be avoided by restricting the use of mittens, by changing their design, and by a greater awareness of this hazard.