Objective Low volume sweat samples are considered unreliable for the diagnosis of cystic fibrosis, based on the assertion that sweat conductivity and chloride are reduced at lower sweating rates. We aimed to re-evaluate the relationship between sweat volume and test results.Design We reviewed all sweat tests performed in our institution to assess the relationship between sweat volume and conductivity, and between sweat volume and sweat chloride. We also compared results between pairs of sweat tests taken simultaneously from a single patient, one with sweat volume below and the other above the currently accepted minimum volume (15 mu l).Results A weak inverse relationship between sweat volume and sweat conductivity was found (n=1500, R(2)=0.105, p < 0.001). There was no correlation between sweat volume and sweat chloride (n=463, R(2)=0.002, p > 0.05). In discordant pairs (one below and one exceeding the accepted minimum volume), the mean test result in the low volume sample was slightly higher than its counterpart. In 76 such pairs, mean conductivity was 41.1 +/- 14.6 mmol/l in the lower volume sample, compared with 36.8 +/- 16.0 mmol/l in the higher volume sample (p < 0.001). Similarly, in 33 of the pairs, mean sweat chloride was 28.4 +/- 15.7 mmol/l in the lower volume sample compared with 25.1 +/- 15.2 mmol/l in the higher volume sample (p=0.004).Conclusion A normal sweat conductivity and/or chloride value from a sweat volume < 15 mu l in a patient whose clinical symptoms are not very suggestive of cystic fibrosis, renders this diagnosis unlikely. In contrast, elevated sweat chloride or conductivity measured from a sample whose volume is < 15 mu l may represent an artefact related to the low volume.

Objectives:Most patients with cystic fibrosis (CF) have pancreatic insufficiency; however, 15% of the patients are pancreatic sufficient (PS). Several laboratory tests have been developed to distinguish between pancreatic insufficiency and PS. The gold standard to determine pancreatic function apart from direct pancreatic stimulation test is the 72-hour fecal fat excretion, expressed as coefficient of fat absorption (CFA). The aim was to test the correlation between 2 other tests, fecal elastase-1 and serum immunoreactive trypsinogen (IRT), as compared with fecal fat excretion. Patients and Methods:21 patients with CF-PS performed the 3 tests of fecal fat excretion, fecal elastase-1, and IRT. Correlation between the tests was evaluated by the κ statistics test, sensitivity and specificity, and positive and negative predictive values. Results:CFA was abnormal in 5 patients, elastase was <200 μg/g in 4 patients, and IRT was <20 ng/mL in 2 patients. The correlation between CFA and IRT was negative (κ = −0.154), and between CFA and fecal elastase-1 was poor (κ = 0.213). The sensitivity, specificity, and positive and negative predictive values of IRT versus CFA were 0%, 88%, 0%, and 78%, and for fecal elastase-1 were 40%, 81%, 40%, and 81%, respectively. Conclusions:In CF-PS, poor correlation was found between IRT, fecal elastase-1, and CFA, therefore neither fecal elastase-1 in the stool nor IRT in the serum reaches the sensitivity or the specificity of the fecal fat excretion. Thus, fecal fat excretion is required in patients with CF for evaluation of pancreatic function.

BACKGROUND:Increased levels of oxidative stress result in pulmonary damage contributing to the development of chronic lung disease in cystic fibrosis (CF). The aim of this study was to investigate the longitudinal effect of serum vitamin A and E levels on the incidence of pulmonary exacerbations in pancreatic insufficient (PI) and pancreatic sufficient (PS) patients with CF.MATERIALS AND METHODS:Patient records were retrospectively examined over a 3-year period and serum vitamin A and E levels were retrieved. Subsequently, levels of vitamin A and E were prospectively measured over a 2-year period at the onset of intravenous antibiotic therapy for acute exacerbation and at the first recovery visit.RESULTS:Retrospectively, 597 pulmonary exacerbations were identified in 102 patients, 74 PI and 28 PS, with a mean age of 11.1 +/- 6.4 years (range, 1.5-27 y). An increased number of exacerbations was directly correlated with lower vitamin A and E levels, even within the normal range. Prospectively, 62 exacerbations were analyzed (43 PI patients and 19 PS patients). At onset of exacerbation, vitamin A and E levels were reduced in the PI patients (P < 0.001; P < 0.001) and the PS patients (P < 0.005; P < 0.07).CONCLUSIONS:Reduced serum levels of vitamin A and E even in the normal range are associated with an increased rate of pulmonary exacerbations in CF. Further studies are required to confirm the necessity of supplementation of vitamins A and E to PS patients.

Background: Carbohydrate malabsorption of lactose, fructose and sorbitol has already been described in normal volunteers and in patients with functional bowel complaints including irritable bowel syndrome. Elimination of the offending sugar(s) should result in clinical improvement.Objective: To examine the importance of carbohydrate malabsorption in outpatients previously diagnosed as having functional bowel disorders, and to estimate the degree of clinical improvement following dietary restriction of the malabsorbed sugar(s).Methods: A cohort of 239 patients defined as functional bowel complaints was divided into a group of 94 patients who met the Rome criteria for irritable bowel syndrome and a second group of 145 patients who did not fulfill these criteria and were defined as functional complaints. Lactose (18 g), fructose (25 g) and a mixture of fructose (25 g) plus sorbitol (5 g) solutions were administered at weekly intervals. End-expiratory hydrogen and methane breath samples were collected at 30 minute intervals for 4 hours. Incomplete absorption was defined as an increment in breath hydrogen of at least 20 ppm, or its equivalent in methane of at least 5 ppm. All patients received a diet without the offending sugar(s) for one month.Results: Only 7% of patients with IBS and 8% of patients with FC absorbed all three sugars normally. The frequency of isolated lactose malabsorption was 16% and 12% respectively. The association of lactose and fructose-sorbitol malabsorption occurred in 61% of both patient groups. The frequency of sugar malabsorption among patients in both groups was 78% for lactose malabsorption (IBS 82%, FC 75%), 44% for fructose malabsorption and 73% for fructose-sorbitol malabsorption (IBS 70%, FC 75%). A marked improvement occurred in 56% of IBS and 60% of FC patients following dietary restriction. The humber of symptoms decreased significantly in both groups (P < 0.01) and correlated with the improvement index (IBS P < 0.05, FC P < 0.025).Conclusions: Combined sugar malabsorption patterns are common in functional bowel disorders and may contribute to symptomatology in most patients. Dietary restriction of the offending sugar(s) should be implemented before the institution of drug therapy.

Journal of Pediatric Gastroenterology and NutritionVolume 15, Issue 3 p. 343-343 ORIGINAL ARTICLE: PDF Only 17 STUDY OF THE DISCREPANCY BETWEEN INTESTINAL ABSORPTION OF VIT E AND TRIGLYCEPIDES (TG) Robert Goldstein, Robert Goldstein Shaare-Zedek Medical Center, Jerusalem, IsraelSearch for more papers by this authorHalina Stankiewicz, Halina Stankiewicz Shaare-Zedek Medical Center, Jerusalem, IsraelSearch for more papers by this authorJoseph Faber Serem Freier, Joseph Faber Serem Freier Shaare-Zedek Medical Center, Jerusalem, IsraelSearch for more papers by this author Robert Goldstein, Robert Goldstein Shaare-Zedek Medical Center, Jerusalem, IsraelSearch for more papers by this authorHalina Stankiewicz, Halina Stankiewicz Shaare-Zedek Medical Center, Jerusalem, IsraelSearch for more papers by this authorJoseph Faber Serem Freier, Joseph Faber Serem Freier Shaare-Zedek Medical Center, Jerusalem, IsraelSearch for more papers by this author First published: 01 October 1992 https://doi.org/10.1002/j.1536-4801.1992.tb10687.xAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onEmailFacebookTwitterLinkedInRedditWechat No abstract is available for this article. Volume15, Issue3October 1992Pages 343-343 RelatedInformation

Rats made uremic by 2-stage 5/6 nephrectomy and sham-operated control animals were fed either a normal laboratory chow, a high-sucrose (60%) or a high-fat (10% cholesterol; 20% olive oil) diet, all containing 21% protein and identical amounts of electrolytes, vitamins and trace elements. Serum creatinine levels remained unchanged in the control animals but rose in the 5/6 nephrectomised uremic animals by a factor of 2.7 from a mean of 0.44 +/- 0.05 mg/dl to 1.20 +/- 0.11 mg/dl at 8 weeks, without differences between the dietary groups. During 8 weeks of dietary regimen the high-sucrose and high-fat diets induced significant hypertriglyceridemia, generally similar in control and uremic rats. The uremic animals on a high-sucrose and high-fat diet had the most pronounced rise in serum triglycerides, 331.5 +/- 89.0 and 298.0 +/- 45.0 mg/dl, respectively (control: 159.9 +/- 14.0 mg/dl). After 4 and 8 weeks, only the animals on the high-fat diet had significant hypercholesterolemia, most pronounced in the uremic animals (356 +/- 56.3 mg/dl; control: 71.6 +/- 12.9 mg/dl). The animals in the latter group also had significant proteinuria and renal histologic abnormalities consisting of xanthoma-like glomerular lesions, infiltrates and fibrosis not seen in the other groups of animals. These data indicate that dietary-induced hyperlipidemia of short duration causes or aggravates renal damage in the rat with mild-moderate uremia, induced by ablation.

The effect of 8 weeks of daily oral fish oil supplementation in a dose of 3 to 8 g/d on serum lipid levels was studied in 16 patients, 7 to 8 years of age, who had end-stage renal disease and were receiving renal replacement therapy. Fasting serum cholesterol (CHOL), triglyceride (TG) levels, and lipoprotein profiles were measured before therapy, 8 weeks after fish oil supplementation, and 4 weeks after its cessation. During 8 weeks of treatment the mean serum CHOL level did not change. The mean serum TG level, however, decreased significantly (P less than .01) from 236 +/- 31 mg/dL to 171 +/- 21 mg/dL (27.5%). Four weeks after treatment was stopped, the mean serum TG level returned to a value not significantly different from the pretreatment level (208 +/- 30 mg/dL). In a subgroup of 11 excessively hyperlipidemic patients, with serum CHOL and TG levels greater than or equal to 50% of the 90th percentile for age and sex, the mean serum TG level decreased even more (30.8%), from 286 +/- 35 mg/dL to 198 +/- 24 mg/dL (P less than .01), and the mean CHOL/high-density lipoprotein CHOL ratio decreased from 8.4 +/- 1.2 to 7.4 +/- 1.3 (P less than .05). Blood pressure and platelet counts remained stable during the entire study period. Side effects of the treatment were minimal. These results show that dietary fish oil supplementation reduces serum TG levels in young patients receiving renal replacement therapy and improves their "atherogenic" serum lipoprotein profile.

Ornithine decarboxylase (ODC) is the first and rate-limiting enzyme in the polyamine biosynthetic pathway. Polyamines have been studied as potential markers of neoplastic diseases, including colonic cancer. Previous studies have pointed out the possible value of this enzyme as a biochemical marker of colonic neoplasia, we studied 100 patients undergoing diagnostic total colonoscopy. There were 40 normal controls and 20 patients in each of the following groups: 1) family members of patients diagnosed as having colonic tumors, 2) patients with adenomas, and 3) patients with colonic adenocarcinoma. Six forceps biopsies were obtained from the normal-appearing sigmoid mucosa for the analysis of ODC. No difference was found among the four groups studied. We therefore conclude that ODC is unreliable for clinical use as a biochemical marker for the identification of population groups at risk for colonic neoplasia.

Two brothers of Arab origin, aged 15 and 10 years, with isolated congenital lipase and colipase deficiency are described. Both were normally developed with a history of passing greasy stools since early infancy. Both have remarkable steatorrhoea and low serum carotene and vitamin E concentrations. After exocrine pancreatic stimulation, lipase and colipase activities in the duodenal fluid were almost completely absent, while amylase trypsin, bile salt, and pH values were normal. No other aetiology for exocrine pancreatic insufficiency was found. This is the first report of congenital combined lipase and colipase deficiency in two brothers.

Following previous observations that medium chain triglycerides (MCT) are absorbed from the stomach of suckling rats, this study was devoted to studying absorption of MCTs in human infants. Four groups of patients were studied: (a) infants suffering from pyloric stenosis, (b) premature infants, (c) children suffering from cystic fibrosis, (d) infants with miscellaneous conditions. Infant formulae with known amounts of MCT were introduced by gastric tube and samples were removed at 0, 20, 40, and 60 min. In patients with pyloric stenosis there was an 18.1% decrease in MCT during the first 20 min. No significant changes in MCT took place during the subsequent 40 min. A similar response was observed in the group of premature infants. Older infants with miscellaneous diagnoses and children with cystic fibrosis showed an even rate of disappearance of MCT during the 60-min test period, and approximately 30% of the original MCTs present disappeared during this period. We conclude that MCTs are absorbed in the stomach of infants and children. Absorption appears to improve with age. Because MCT are an important constituent of formulae for premature infants and children with defects of small intestinal digestion and absorption of fat, these observations have practical implications.

We studied plasma vitamin E levels in children/adolescents 3-19 years of age (10.9 +/- 4.5; mean +/- 1 SD) with chronic renal failure treated conservatively, on hemodialysis, on continuous ambulatory peritoneal dialysis and after renal transplantation. In all 4 groups of patients vitamin E levels (0.66-0.90 mg/dl) were within the normal range (0.76 +/- 0.19 mg/dl). The lowest levels of vitamin E (0.59 +/- 0.1 mg/dl) were found in nonuremic patients with a good functioning renal transplant. These data do not support the notion that uremia is accompanied by vitamin E malabsorption.

In the past we have shown that patients with chronic renal failure (CRF) on hemodialysis show evidence of intestinal malabsorption of fat. The present study was designed to verify this finding in an animal model. Male rats weighing +/- 200 g were studied. Uremia (U) was induced by 2-stage subtotal (5/6) nephrectomy. Control (C) animals were sham-operated. Fat absorption was studied after 6 weeks of uremia with the oral fat loading test. Twenty percent intralipid (0.25 g/100 gBW) was given by gastric tube feeding to fasting animals and the appearance of chylomicrons (CHYL) and the rise of triglycerides (TG) in the serum was followed for 5 hrs. In order to isolate the effect of fat absorption, an additional group of U and C animals was pretreated with orotic acid and triton, thus blocking hepatic TG synthesis and neutralizing peripheral lipoprotein lipase activity. The absorption of CHYL was significantly (p less than 0.01) impaired in all U animals and averaged 43 and 70 percent of that of the C animals, 1 and 2 hrs after the load respectively. The rise in serum TG did not differ from C in mildly U animals (Scr 1.0 +/- 0.04). In the more severely uremic animals (Scr 2.6 +/- 0.2), however, pretreated with orotic acid and triton, the rise in serum TG was far less (p less than 0.01) than in C animals (111 +/- 26-903 +/- 111 delta % V.780 +/- 170-5032 +/- 746 delta %) 1 and 5 hrs after the load.(ABSTRACT TRUNCATED AT 250 WORDS)

The outcome of acute poststreptococcal glomerulonephritis (APSGN) is still controversial. Whereas many studies show a good prognosis, particularly in children, late morphologic and functional renal abnormalities have been described. To verify this latter finding, we evaluated the sodium handling of the kidney several years after APSGN. Eleven subjects (9 children and adolescents and 2 adults) were studied 2 to 3 years after APSGN. At the time of the study all patients were well, without pathologic urinary findings and with normal blood pressures. Five healthy subjects (3 adults and 2 children) served as controls. Following two control periods of 30 min, an i.v. saline load (2.5% NaCl, 1 l/1.73 m2 body surface area) was administered over 45 min. Sodium excretion was measured during the control and salt-loading periods, and during four additional 30-min collection periods. No exaggerated natriuresis was found. We therefore conclude that epidemic APSGN in Israel carries not only an excellent clinical but also a good renal functional prognosis.

We have shown previously that MCT are hydrolysed and absorbed from the stomach of suckling rats. We now report evidence that MCT are absorbed from the stomach of infants. Three groups of infants were chosen: 5 suffering from pyloric stenosis (P.S.), 7 from prematurity and 5 from miscellaneous problems. Infants with P.S. were chosen because of the delayed gastric emptying inherent to this condition so that the more rapid disappearance of MCT as compared to LCT must be due to absorption rather than to possibly more rapid gastric emptying of MCT. To each infant 10 ml/kg of formula were administered by gastric tube. Prematures received Enfalac (50% of fat as MCT) while the other groups received Pregestimil (40% MCT) (Mead-Johnson Co, Evansville, 111). Aliquots of 3 ml. were removed at 0, 20, 40 and 60 minutes. We found that in the P.S. group 35% of Total MCT were absorbed in the first hour. Corresponding figures for the prematures and the miscellaneous groups were 22.8% and 58.5% respecticely.C8 was absorbed more rapidly than C10. These results show that MCT are absorbed from the gastric mucosa of infants. In view of the presence of MCT in breast milk and in infant formulas, this may be of physiological importance.

Journal of Pediatric Gastroenterology and NutritionVolume 5, Issue 3 p. 508-508 Letters to the Editor: PDF Only Increased Bile Acid Level in Preterm Infants Treated with Phenobarbital Michael S. Schimmel, Michael S. Schimmel Department of Neonatology, Shaare zedek Medical Center, Biochemical Laboratories, Institute of Gastroenterology, Shaare zedek Medical Center, Jerusalem, IsraelSearch for more papers by this authorArthur I. Eidelman, Arthur I. Eidelman Department of Neonatology, Shaare zedek Medical Center, Biochemical Laboratories, Institute of Gastroenterology, Shaare zedek Medical Center, Jerusalem, IsraelSearch for more papers by this authorHalina Stankiewicz Robert Goldstein, Halina Stankiewicz Robert Goldstein Department of Neonatology, Shaare zedek Medical Center, Biochemical Laboratories, Institute of Gastroenterology, Shaare zedek Medical Center, Jerusalem, IsraelSearch for more papers by this author Michael S. Schimmel, Michael S. Schimmel Department of Neonatology, Shaare zedek Medical Center, Biochemical Laboratories, Institute of Gastroenterology, Shaare zedek Medical Center, Jerusalem, IsraelSearch for more papers by this authorArthur I. Eidelman, Arthur I. Eidelman Department of Neonatology, Shaare zedek Medical Center, Biochemical Laboratories, Institute of Gastroenterology, Shaare zedek Medical Center, Jerusalem, IsraelSearch for more papers by this authorHalina Stankiewicz Robert Goldstein, Halina Stankiewicz Robert Goldstein Department of Neonatology, Shaare zedek Medical Center, Biochemical Laboratories, Institute of Gastroenterology, Shaare zedek Medical Center, Jerusalem, IsraelSearch for more papers by this author First published: 01 May 1986 https://doi.org/10.1002/j.1536-4801.1986.tb09116.xAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onEmailFacebookTwitterLinkedInRedditWechat No abstract is available for this article. Volume5, Issue3May 1986Pages 508-508 RelatedInformation

In view of the technical difficulties inherent in using stool fat estimations as a parameter of malabsorption, we used a fatty meal absorption test. Children under investigation for failure to thrive were divided into two groups, those having a normal stool fat output (less than 3.2 g/day) constituting the control group, and those having steatorrhea. After a fatty meal containing 25 g of margarine and 25 g of butter fat, we measured the rise of serum triglycerides and chylomicrons hourly for 5 h. Serum triglyceride rise of less than 100 mg/dl or less than 100% above basal values and the appearance of less than 7% of chylomicrons were considered pathological. Of our control group 95% had a normal rise of triglycerides, and 96% of our patients with steatorrhea had an abnormal rise. This test was more reliable than the two-point triglyceride test previously described for the diagnosis of fat malabsorption. The fatty meal test as described here is considered to be a useful test of absorptive function.

The aim of this study was to verify whether exaggerated natriuresis is always present after PSAGN,as previously reported.Nine patients(7 children,2 adults) two years after PSAGN and four healthy control subjects(3 adults,1child) were studied.All subjects had normal urinalysis,GFR and BP.Following two control periods of 30 min(I,II) an i.v. saline load (2.5%NaCl, 1L/1.73m2) was given over 45 min(III).GFR,UVna and venous HTC were measured during these periods as well as during four 30 min collection periods following completion of the saline load (IV,V,VI,VII). GFR was comparable in the two groups.No significant differences were found in absolute or fractional excretion of sodium.We thus found no evidence for exaggerated natriuresis two years after PSAGN.We conclude that PSAGN as seen by us in Israel, carries a good (short-term) clinical and renal functional prognosis.