Search PubMed⌕ Search

Biomedical subjects

Z Weizman

Publications and source records attributed to Z Weizman.

At least 55 records · Page 3Linked to original sources

Transient protein-losing gastropathy (Menetrier's disease) in childhood.

Two cases of transient protein-losing gastropathy (PLG) or Menetrier's disease in childhood are described. Both cases presented with an abrupt onset of edema due to hypoalbuminemia and were diagnosed by an upper gastrointestinal series. One of the cases was further diagnosed and followed by ultrasound. We believe that this is the first description of the ultrasonic picture of transient PLG in children. In both cases complete recovery was evident after a few weeks.

Child, Preschool↗

Impaired chloride secretion, as well as bicarbonate secretion, underlies the fluid secretory defect in the cystic fibrosis pancreas.

Pancreatic fluid and electrolyte secretion was assessed in 56 patients with cystic fibrosis (CF) and 56 non-CF control subjects undergoing pancreatic function testing while stimulated with cholecystokinin and secretin. Both CF patients and control subjects exhibited a wide range of pancreatic function. Fluid and trypsin outputs were positively correlated in both groups. Fluid output in CF subjects was significantly lower, however, than that of control subjects at any given level of trypsin output. Sodium, bicarbonate, and chloride secretions were all significantly decreased in CF subjects. Bicarbonate and chloride were important determinants of fluid secretion, but at any given bicarbonate or chloride output CF subjects secreted significantly less fluid than control subjects. When bicarbonate and chloride were analyzed as simultaneous predictor variables, adjusted fluid secretion was not significantly different in CF and control subjects. Diminished fluid secretion in CF subjects is therefore caused by impaired chloride, as well as bicarbonate, secretion.

Bicarbonates↗

Superior mesenteric artery syndrome presenting as an anorexia nervosa-like illness.

Anorexia nervosa is often overdiagnosed in adolescent females with anorexia and extreme weight loss. In some cases, an anorexia nervosa-like illness is due to a treatable organic disorder. We describe a 16-year-old female with anorexia, vomiting, extreme weight loss, and amenorrhea who was referred as an anorexia nervosa case and was subsequently found to have superior mesenteric artery syndrome that was causing a duodenal obstruction. Conservative treatment with oral hypercaloric liquid feeding resulted in optimal weight gain and complete recovery. This syndrome should be considered in adolescents with an anorexia nervosa-like illness associated with vomiting and postprandial epigastric discomfort.

Adolescent↗

Acute pancreatitis in childhood.

Sixty-one patients (1 to 18 1/2 years of age) with acute pancreatitis were evaluated. In over one third of cases, acute pancreatitis was one feature of a multisystem disease (Reye syndrome, sepsis, shock, hemolytic-uremic syndrome, viral infections). Other common causes included blunt trauma (15%), acquired or congenital structural defects (10%), metabolic diseases (10%), and drug toxicity (3%). In 25% of cases, no cause was identified. All conscious patients complained of abdominal pain, but the location, severity, and duration of pain were extremely variable. Vomiting was a common symptom. Ultrasonography was helpful in establishing the diagnosis and for assessment of complications such as pseudocyst formation. Endoscopic retrograde cholangiopancreatography was used to identify structural or anatomic lesions in patients with recurrent acute pancreatitis. Serum cationic trypsin(ogen) was superior to amylase in the early diagnosis of acute pancreatitis, and was more consistently elevated during the first 5 days in the hospital. Patients were managed conservatively with complete bowel rest, gastric decompression, intravenous fluid therapy, and pain relief. Pancreatic pseudocysts occurred in 10% of patients. There were 13 fatalities, all in patients with a severe multisystem disorder. Recurrences of acute pancreatitis were noted only in certain diagnostic groups: idiopathic pancreatitis, structural anomalies of the pancreaticobiliary tree, metabolic disorders, and (in a single patient) familial pancreatitis.

Acute Disease↗

Early vs. late refeeding in acute infantile diarrhea.

A randomized ambulatory trial was performed to compare early (6-h) vs. late (24-h) refeeding in acute infantile diarrhea. Ninety infants with mild dehydration were enrolled in the study. Following an initial oral rehydration period (WHO formula), refeeding was introduced using a diet based on either breast milk or cow's milk. Early (n = 53) and late (n = 37) refeeding groups were similar in ethnic background, socioeconomic level, relevant past history, nutritional and clinical state, and stool pathogens. Infants were assessed upon their initial visit, at 24 and 48 h, and at 7 and 14 days thereafter for evaluation of weight, hydration state, stool frequency and need of hospitalization. No significant differences in the above parameters were observed between the two groups. Different patterns of refeeding (breast milk vs. cow's milk) in both early and late refeeding groups showed no significant differences in the features studied. Since the short-term clinical outcome following early refeeding in acute infantile diarrhea is not different from late refeeding, we suggest that early refeeding should be preferred, particularly in developing populations, in order to minimize the adverse nutritional effects of prolonged fasting during recurrent bouts of gastroenteritis.

Acute Disease↗

Treatment failure in celiac disease due to coexistent exocrine pancreatic insufficiency.

A 17-year-old white adolescent had a history of chronic diarrhea, delayed puberty, and growth failure. Investigations excluded cystic fibrosis, Shwachman syndrome, and endocrine causes of growth failure. Severe steatorrhea was diagnosed from fecal fat studies, and a jejunal suction biopsy showed total villus atrophy, consistent with a diagnosis of celiac disease. Following introduction of a gluten-free diet, his appetite and growth improved, but he continued to have abdominal discomfort and loose offensive bowel motions. One year later, severe steatorrhea was present. A repeat jejunal biopsy showed partial recovery of villus architecture. Serum immuno-reactive trypsinogen level was low, which was highly suggestive of exocrine pancreatic failure. Results of quantitative pancreatic stimulation test confirmed the presence of primary pancreatic insufficiency. After introduction of oral pancreatic enzyme supplements with meals, his gastrointestinal symptoms resolved and growth velocity accelerated. Previously, primary pancreatic insufficiency has only been described in elderly patients with long-standing untreated celiac disease. This case, however, emphasizes that pancreatic failure can occur with celiac disease at any age. Determination of a serum immunoreactive trypsinogen level should be considered a useful screening tool for pancreatic insufficiency in patients with celiac disease who have not responded to a gluten-free diet.

Adolescent↗

Ethmoiditis-associated periorbital cellulitis.

Seventy-two children with periorbital cellulitis were followed prospectively during a 30-month study period. Twenty patients (28%) had mucopurulent rhinorrhea with obvious radiological ipsilateral opacity of the ethmoid sinuses. This group of patients, as opposed to patients with periorbital cellulitis and no associated ethmoiditis, is characterized by the following features: Higher incidence of temperature greater than 38.5 degrees C (16/20 vs 24/52. P less than 0.01) and elevated white blood count greater than 15,000 cells/mm3 on admission (10/20 vs 5/52. P less than 0.001). Delayed recovery (6.7 +/- 3.4 vs 3.4 +/- 1.6 days. P less than 0.001). Higher rate of recurrent periorbital cellulitis with associated ethmoiditis within 6 months of follow-up (5/20 vs 1/52. P less than 0.01). This ethmoiditis-associated serious form of periorbital cellulitis requires an aggressive treatment regimen and a prolonged follow-up.

Anti-Bacterial Agents↗

Bile acid secretion in cystic fibrosis: evidence for a defect unrelated to fat malabsorption.

In order to define basic biliary defects not related to steatorrhoea in cystic fibrosis, we studied 12 control and 18 cystic fibrosis subjects, with a wide range of pancreatic function. Duodenal aspirates were collected over three consecutive 20 minute periods, during continuous intravenous infusion of cholecystokinin and secretin using a marker perfusion technique, and analysed for pancreatic enzyme output (colipase, lipase, trypsin), bile acid output and concentration, and biliary lipids. Cystic fibrosis patients, at all levels of pancreatic function, had significantly reduced total bile acid output (mumol/kg/h) with delayed appearance of the bile acid peak, compared with control subjects. Actual duodenal bile acid concentrations were significantly higher in cystic fibrosis subjects than in controls, however, probably because of the markedly reduced water output shown in these patients. The lithogenic index was not raised in cystic fibrosis patients at any level of pancreatic function. The reduced bile acid output and the delayed peak appearance probably reflect a defect in gall bladder responsiveness which is independent of pancreatic function and steatorrhoea. Whether this defect is related to gall bladder filling or a defective peptide hormone response awaits further study.

Adolescent↗

Pancreatic fluid secretion and protein hyperconcentration in cystic fibrosis.

To study pancreatic protein and water secretion in 28 patients with cystic fibrosis and 21 controls matched for pancreatic acinar function as defined by trypsin secretion, we used a quantitative-marker perfusion technique and continuous intravenous secretin-pancreozymin stimulation. Regardless of the level of pancreatic acinar function, secretions from the patients contained significantly higher concentrations of protein than those from the controls. Total protein output and albumin:protein ratios were not increased in secretions from the patients, but their fluid secretion was significantly decreased at any level of pancreatic function. A significant linear correlation was found between protein and volume secretion in the patients (r = 0.86, P less than 0.001), most of whom had a fluid output of less than 4.2 ml per kilogram of body weight per hour. No such relation was found in the control subjects, whose flow was always above 4.2 ml per kilogram per hour. We conclude that fluid secretion in patients with cystic fibrosis may be a rate-limiting factor in protein output and that a limited flow of hyperconcentrated protein secretions may predispose to protein precipitation and ductal obstruction in the pancreas.

Adolescent↗

Altered PABA pharmacokinetics in cystic fibrosis. Implications for bentiromide test.

The bentiromide test has been proposed as a useful noninvasive method for assessing exocrine pancreatic function in cystic fibrosis (CF) patients. Following oral administration, this peptide is selectively cleaved by pancreatic chymotrypsin liberating PABA which is passively absorbed. Recent studies have suggested that PABA measured in plasma is superior to the more established method of estimating urinary recovery of this marker. However, in using the plasma test in CF patients, one makes the assumption that the PABA marker has similar distribution and elimination patterns in normal and CF subjects. Since many drugs display altered pharmacokinetics in CF patients, we studied the disposition of PABA following ingestion of free PABA in six controls (age 19-28 years) and 18 CF patients (13-18 years; seven steatorrheic and 11 nonsteatorrheic). Elimination of T1/2 of PABA was significantly shorter in CF patients (58 +/- 21 min) compared to controls (93.5 +/- 28) (P less than 0.005). PABA clearance was similar in the control and CF patients (2.99 +/- 1.21 and 3.27 +/- 1.02 ml/min/kg, respectively). PABA distribution volume was smaller, although not significantly so, than in the controls (268 +/- 107 vs 376 +/- 140 ml/kg). Good correlation was found between PABA distribution volume and T1/2 (r = 0.51 P less than 0.02). Our simulation data suggest that altered pharmacokinetics of PABA in CF patients would cause their PABA levels to be 7% lower than controls at 90 min, 18% at 120 min, 29% at 150 min, and 38% at 180 min.(ABSTRACT TRUNCATED AT 250 WORDS)

4-Aminobenzoic Acid↗

Bentiromide test for assessing pancreatic dysfunction using analysis of para-aminobenzoic acid in plasma and urine. Studies in cystic fibrosis and Shwachman's syndrome.

We evaluated the bentiromide test by analyzing para-aminobenzoic acid (PABA) in plasma and urine (a) for the identification of patients with complete pancreatic insufficiency and (b) as an alternative to the secretin-cholecystokinin test. Nine control subjects, 18 patients with cystic fibrosis, and 4 patients with Shwachman's syndrome were studied. Based upon the secretin-cholecystokinin test, pancreatic function was judged to be less than 0.1% of normal in 7 patients with cystic fibrosis and malabsorption and between 0.7% and 90% of control values in 11 patients with cystic fibrosis and 4 patients with Shwachman's syndrome without malabsorption. The bentiromide test was performed in two stages: first with bentiromide alone, then with equimolar free PABA. After ingestion of free PABA, the plasma profile and urinary excretion of PABA were comparable in controls, patients with cystic fibrosis, and patients with Shwachman's syndrome. Thirty minutes after oral bentiromide, plasma PABA values in patients with and without malabsorption were significantly lower than in the control group. From 60 to 180 min after ingestion, plasma PABA levels in patients without malabsorption were no different from controls; whereas levels in patients with malabsorption were significantly lower than in controls and in those without malabsorption, reaching the highest significance at 90 min. Similar results were obtained when the urinary excretion of PABA was considered. Only the 90-min plasma test reliably detected cystic fibrosis patients with steatorrhea, however. Duodenal colipase output was highly correlated with both the 90-min plasma test and the urinary excretion of PABA, with similar results for lipase and trypsin output. Reliable detection of pancreatic dysfunction, nevertheless, was not obtained even with the plasma test, in cystic fibrosis patients with greater than 5%-10% of the mean normal enzyme output. In patients with Shwachman's syndrome, none of whom had malabsorption, the plasma and urinary test failed to detect pancreatic dysfunction even with enzyme output as low as 1% of normal.

4-Aminobenzoic Acid↗

Multiple hornet stings with features of Reye's syndrome.

Hornet's venom is known to possess a variety of toxic effects. A 19-mo-old girl who developed a Reye-like syndrome following multiple stings by the Oriental hornet (Vespa orientalis) is described. She presented with encephalopathy associated with hepatomegaly, elevated transaminase levels, low prothrombin time, and hyperammonemia. Liver biopsy demonstrated microvesicular fatty infiltration and diffuse mitochondrial changes. Additional features were acute renal tubular necrosis and massive hemolysis.

Antibodies↗

Elevated serum immunoreactive pancreatic cationic trypsinogen in acute malnutrition: evidence of pancreatic damage.

We used a sensitive probe of pancreatic dysfunction, serum immunoreactive cationic trypsinogen, to study 50 infants and children with varying degrees of malnutrition. Patients were classified into subgroups according to the severity of malnutrition. Mean serum trypsinogen concentration was significantly elevated in 25 patients with "severe" malnutrition (77.4 +/- 42.0 ng/ml, P less than 0.001) and in 23 with "moderate" malnutrition (55.2 +/- 16.1 ng/ml, P less than 0.02) compared with the mean value (32.5 +/- 10.4 ng/ml) for well-nourished controls. The level of circulating trypsinogen tended to rise with increasing severity of malnutrition. There was no relationship between serum trypsinogen and other variables such as age, specific diagnosis, or mode of feeling. Elevated serum trypsinogen levels could not be attributed to renal disease or cystic fibrosis. In patients who showed an improvement in nutritional status, serum trypsinogen tended to revert toward normal. Elevated serum trypsinogen values in acutely malnourished infants and children may result from pancreatic acinar cell damage or regurgitation of enzymes from obstructed pancreatic ducts.

Acute Disease↗