Primary sclerosing cholangitis and multiple autoimmune disorders in a patient with Down syndrome.
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Biomedical subjects
Publications and source records attributed to I D Hill.
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A retrospective cross-sectional study of the roentgenograms of 300 hip fracture patients and 300 age- and gender-matched controls was performed to determine the relationship between fracture type, femoral neck trabecular bone integrity (as measured by the Singh index), osteoarthrosis, and age. Coxarthrosis was associated with a low incidence of intracapsular fracture but unchanged rates of extracapsular fracture. Singh grade declined with age in all groups of patients, although the rate of decline was reduced in control female patients with coxarthrosis compared with other diagnostic groups. When the coxarthrosis patients were included, the mean Singh grade for the female fracture patients was significantly reduced in patients compared with controls with or without age adjustment (3.88 versus 4.17). When patients with coxarthrosis were excluded from the analysis, this effect disappeared. It is suggested that the differences in Singh grades observed previously between femoral fracture patients and controls may have resulted from an undetected negative association between coxarthrosis and retention of trabecular integrity with aging.
Bleeding from esophageal varices is a common cause of major upper gastrointestinal tract blood loss in children with portal hypertension but usually ceases spontaneously or is satisfactorily managed by nonoperative measures. Massive hemorrhage from gastric fundal varices may be difficult to control with compression and sclerotherapy; in these cases, a direct surgical approach may be indicated. Since 1984, 27 children have undergone aggressive injection sclerotherapy for bleeding esophageal/gastric varices. Nine (6 with portal vein thrombosis) bled from gastric fundal varices. In 5 of these, medical management and sclerotherapy failed to control the acute bleed. In all 5 there was "rupture" of a large gastric fundal varix or "pile" and bleeding was controlled at emergency laparotomy by underrunning the varices through a high anterior gastrotomy. Four have subsequently been successfully managed by continued sclerotherapy and one patient with cirrhosis has died of liver failure. In 3 of the survivors both esophageal and gastric fundal varices have been completely obliterated. No further life-threatening hemorrhage has occurred in any case during a follow-up period of 1 to 5 years. Bleeding from gastric varices is more common than previously recorded and more difficult to control by nonoperative management, including injection sclerotherapy. In uncontrolled hemorrhage from gastric varices, surgical underrunning offers a means of providing initial control. Thereafter, the inevitable variceal recurrence may be successfully treated with sclerotherapy.
Dietary manipulation is often the first step in the treatment of infants with persistent acute dehydrating diarrhoea. This usually entails elimination of lactose, but other disaccharides or whole protein may be causing the disease. Serial elimination of these takes time and it may be preferable to use a whole protein and disaccharide-free formula as the first feed change. This study assessed the effect on stool weight of a change from cow's milk formula feeds to one of four different formulae in infants with severe diarrhoea persisting after 3 days in hospital. Two feeds were lactose-free soy formulae containing sucrose, one was disaccharide-free soy formula and one a disaccharide-free protein hydrolysate. Regardless of which feed the infants received, diarrhoea resolved in approximately 50% following the change in diet. Comparing those who got better with those who did not, the former were generally better nourished and had an initial lower stool output, but it was impossible to predict on clinical grounds which individual would respond to the removal of cow's milk. The results suggest that elimination of lactose in infants with persistent severe diarrhoea will benefit a significant number in the early stage of the disease. As there is no additional benefit from eliminating sucrose or whole protein at this stage, the cheapest available lactose-free formula should be used initially.
Traditional methods for collecting duodenal fluid are time consuming and technically difficult. A simple endoscopic method is proposed in this report as a means of collecting duodenal fluid to perform exocrine pancreatic function tests. Thirty-five patients between 24 and 36 months of age were studied for pancreatic exocrine function. Twenty-seven presented with chronic diarrhea and 8 with failure to thrive. In 20 patients (group 1), duodenal fluid was collected by means of a double-lumen tube and sequential administration of pancreozymin (PZN) and secretin (SEC). The rest (group 2) had duodenal aspiration from the level of the papilla of Vater through a fiberoptic endoscope following administration of SEC only. The procedure took approximately 3 h in group 1 and 45 min in group 2. Secretin administration produced comparable levels of enzymes in both groups. Pancreozymin produced the highest enzyme levels, but this was only significantly higher than SEC-induced levels in the case of lipase. Endoscopic collection of duodenal fluid following SEC administration is a safe, quick, and reliable method of collecting pancreatic secretion.
Thirty-three children with esophageal varices due to portal hypertension underwent injection sclerotherapy over a period of 6 yr. Thirty-one completed the sclerotherapy course, and the varices were eradicated in all. In nine, the procedure was performed as an emergency because of continued bleeding and, in each case, a gastric fundal varix was the source of the blood loss. Sclerotherapy successfully controlled the bleeding in four of these, whereas five required surgical underrunning of the fundal varix. After surgery, these five continued sclerotherapy until the esophageal varices were eradicated. Complications included transient pyrexia (39%), retrosternal discomfort (30%), esophageal ulceration (18%), and esophageal stricture (12%). Rebleeding before initial eradication of the varices occurred in 12 patients but, thereafter, was very uncommon and always small in amount. Esophageal varices recurred after initial eradication in 33% of cases but were easily sclerosed with further injections. This study demonstrates that sclerotherapy is effective in reducing bleeding frequency in children with portal hypertension, but emphasizes the need for regular follow-up endoscopy after initial eradication of esophageal varices.
In a retrospective survey, the clinical details of all children with primary peptic ulcer seen at Red Cross War Memorial Children's Hospital over a 12-year period were analysed. The diagnosis was confirmed in 31 cases, 22 of whom had a duodenal ulcer. Gastro-intestinal bleeding was the presenting symptom in 19 patients. In 7 of these there were no preceding symptoms, rendering earlier diagnosis impossible. Those presenting with abdominal pain had a mean interval of 2.8 years between onset of symptoms and diagnosis. Useful clinical clues in these children were epigastric tenderness and anaemia. To avoid diagnostic delays, peptic ulcer disease should be considered more often in children with abdominal pain. When available, gastro-intestinal tract endoscopy should be the diagnostic investigation of choice.
Cannula tubing (1.6 mm external, 1 mm internal diameter) was fabricated from medical grade polyurethane containing 2% 2,4,4'-trichloro-2'-hydroxydiphenyl ether ('Irgasan', Ciba-Geigy). When shaken with 10 ml of phosphate-buffered saline an equilibrium was set up with only traces of 'Irgasan' in the aqueous phase. When phosphate-buffered saline flowed through the tubing, 0.26 mg of 'Irgasan' per g of tubing per day was eluted, and this was not detectably increased by buffers of pH 3 or pH 8.5. However, a 20% lipid emulsion eluted 6 mg per g of tubing per day with a flow rate of 250 ml per day and 11 mg with a flow rate of 11 per day. These results scaled up show that a 7.11 g Hickman catheter would lose 78 mg of Irgasan in the first day with Intralipid, compared to 1.8 mg with PBS. If elution by tissue fluids (including blood) does not exceed that by Intralipid then comparison with available toxicological data suggests an adequate margin of safety for adults, but not for premature babies.
This study was designed to assess the effect of loperamide, given to infants in higher than recommended doses, on the severity and duration of acute diarrhea. Thirty infants with acute diarrhea and dehydration were given loperamide (0.8 mg/kg/day), in addition to standard fluid therapy, for 48 hours after admission to the hospital. The stool output in grams per kilograms of body weight per day and the duration of diarrhea in these infants were compared with those in 30 matched control infants receiving only standard fluid therapy. Two infants given loperamide had to be withdrawn from the trial because ileus developed in one and the other had persistent severe vomiting. In four other infants receiving loperamide, drowsiness developed but resolved rapidly on discontinuation of the drug. Infants receiving loperamide had a shorter duration of diarrhea (median 2.5 vs 6.0 days) and lower daily stool output than the control subjects had. The study confirmed the efficacy of loperamide in reducing the duration and severity of diarrhea but raised doubts regarding its safety in the treatment of young infants.
The relative frequency of causes of cholestatic disorders of infancy in a developing area was established in a prospective study. During a 10-year period, 145 infants with conjugated hyperbilirubinaemia were investigated. Intrahepatic disorders accounted for 68 per cent with no identifiable cause (idiopathic hepatitis) in the majority. Syphilis, urinary tract infection and septicaemia together made up 30 per cent of intrahepatic causes with metabolic disorders accounting for 12 per cent. Outcome in those with idiopathic hepatitis, and those treated for syphilis and UTI was relatively good. Complete recovery from syphilitic hepatitis on average took 11 months. Extrahepatic disorders occurred in 32 per cent and were almost entirely due to biliary atresia. Results of hepatic portoenterostomy for biliary atresia were poor because of late referral in many instances. Compared to developed countries, infantile cholestasis in developing areas is more commonly associated with treatable bacterial infection. Referring agencies should be aware of this fact and the need for early referral of cases with possible biliary atresia.
15N-yeast protein absorption, nitrogen and fat retention and stool reducing substances and lactate were measured in 6 infants who had acute diarrhoea and 15 who had had severe diarrhoea for 4 d. The results were compared with those of previously reported infants, who had had diarrhoea for 8 d. The infants were fed a full cream cows' milk, soy based or low lactose formula. In all cases the losses of nitrogen and energy in stool rose as stool weight increased. In severe diarrhoea, the losses of nutrients in stool were so great that oral feeds did not provide adequate nitrogen and energy. The smallest loss of nitrogen and fat were found in infants who had had diarrhoea for 4 d and who were fed a soy based formula.
A cross-sectional survey was conducted among 60 families with a child with cystic fibrosis to assess their medical knowledge of the illness. A 63-item, multiple-choice test with acceptable psychometric properties was administered to 60 mothers, 54 fathers, 29 siblings (aged 10 to 23 years), and 18 patients (aged 9 to 22 years). Parents and patients correctly answered approximately three quarters and siblings two thirds of all items. Family members were most knowledgeable about general cystic fibrosis facts, physiotherapy, gastrointestinal symptomatology and treatment, and anatomy. They were less well-informed about respiratory symptomatology and treatment and nutrition. Parental knowledge of genetics and reproductive risks was mediocre, and that of patients and siblings was poor. Knowledge of terminology was uniformly low. Social class was a significant predictor of parental knowledge. If left uncorrected the misconceptions, gaps, and errors in family members' knowledge of cystic fibrosis identified in this study could result in inadverent noncompliance in treatment of the patient.
A cross-sectional survey of 60 families with a child with cystic fibrosis was undertaken to assess how much family members wanted to be told about the illness, to determine specific information needs concerning selected medical and psychosocial topics, and to determine whether this information had been provided in the past. Self-administered index measures were designed for this purpose. Most family members wanted complete and comprehensive information about cystic fibrosis. A minority of family members claimed they had not received basic medical information. Fathers and siblings wanted more medical information than mothers and patients. The majority of parents and patients wanted a great deal more information on the possible effects of cystic fibrosis on the patient's career, social life, marriage, and reproductive capacity. In general, family members had been given more medical than psychosocial information. Index measures were independent of the effects of patients' age, Shwachman score, and period since diagnosis. Social class was significantly associated with fathers' index measures of their specific information needs and information not given previously.
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Ongoing acute diarrhoea in infancy may respond to a change from a cows' milk to a soya-based formula. This is usually ascribed to the change in carbohydrate content of the feed but the ideal carbohydrate composition of the soya feed is uncertain. Twenty infants with severe watery diarrhoea persisting 2 days after initial rehydration and refeeding with a cows' milk formula were randomly allocated to one of two soya-based formulas. In one the carbohydrate was a mixture of sucrose and glucose polymers and in the other all the carbohydrate glucose polymers. No advantage was noted for either formula. Approximately 50% of each group responded to dietary change. The prompt cessation of diarrhoea and the disappearance of evidence of carbohydrate malabsorption suggests the response is due to the removal of lactose from the diet. Those infants that did not respond had evidence of continuing carbohydrate malabsorption and were not simply lactose-intolerant. The pathogenesis of this carbohydrate malabsorption and the possible relationship to small-bowel bacterial overgrowth requires further investigation.
A retrospective study of all patients with cystic fibrosis (CF) seen at the Red Cross War Memorial Children's Hospital was undertaken. A total of 106 patients were identified and of these 64 still attend the CF clinic regularly. By 1 year of age 95% had symptoms but only 63% had been correctly diagnosed. In 27% the diagnosis was only made after 5 years of age. Chronic lung disease, failure to thrive, and diarrhoea or steatorrhoea were the most common presenting symptoms. Clinical scores of those still attending the clinic show that 79% are in the mild or better categories. The overall mortality rate was 21% and death was due to severe chest disease in the vast majority of cases. On the basis of the number of new patients with CF born during a 4-year period and the total number of live births recorded in this time, the calculated incidence of CF in Cape Town was 1/2,000 for whites and 1/12,000 for coloureds. Calculated prevalence rates for both whites and coloureds were much higher in urban Cape Town than in the rural areas of the western Cape. This suggests that many cases remain undiagnosed in the rural areas.