bluuebunny/crossref_metadata_2025 huggingface dataset:
Hirschsprung's Disease in a Family with Multiple Endocrine Neoplasia Type 2
Authors: Verdy Maurice , Weber Andrée M. , Roy Claude C. , Morin Claude L. , Cadotte Marcel , Brochu Pierre
Published: January 2024

Gastrointestinal symptoms are known to occur in association with multiple endocrine neoplasia (MEN) type 2. Patients with the variant 2b are at special risk and show diffuse intestinal ganglioneuromatosis. Megacolon is frequently an early X‐ray finding and is associated with hyperplastic ganglion cells and nerve plexuses. The unusual association of MEN type 2a with aganglionic megacolon is described in a kindred of 92 individuals. There were 15 cases of medullary thyroid carcinoma (MTC), one case each of pheochromocytoma and hyperparathyroidism, and eight cases of Hirschsprung's disease (five histologically proven). In these eight patients, the neonatal period was characterized by a delay in the passage of meconium. Three infants died from intestinal obstruction within the first 2 weeks of life. The diagnosis was missed in one girl who died despite laparotomy. Pull‐through procedures were successfully performed in three other infants. The last individual suffered from constipation and several bouts of intestinal obstruction before being operated on at the age of 19. He was subsequently found to have MTC and underwent surgery, but recurrence was recently documented. The four living patients were reevaluated. Rectal manometry could not elicit internal sphincter relaxation, thus confirming the diagnosis of Hirschsprung's disease. Biopsies of the remaining colon contained normal ganglion cells and nerve fibers, thereby eliminating the possibility of aganglionosis and ganglioneuromatosis in different parts of the large bowel of the same individual. It is concluded that Hirschsprung's disease might be responsible for bowel obstruction in MEN type 2. In familial cases of aganglionic bowel, the possibility of MEN should be ruled out by family history and appropriate testing.
Hormone Maturative Effect on Perinatal Rat Colon Glycosidases
Authors: Litin Bradley S. , Swan Kathleen R. , Koldovský Otakar
Published: January 2024

Suckling rats were injected with thyroid and adrenal cortex hormones to explore their maturative effect on the activities of colon glycosidases. In experiment 1, rats 2, 5, and 8 days old were injected daily with thyroxine (2 $mUg/g body weight) or cortisone acetate (50 $mUg/g body weight) for 3 continuous days. Rats were sacrificed on the 4th day. In experiment 2, rats 4 and 11 days old were injected for 4 continuous days with T3(0.1 or 0.5 $mUg/g body weight) or a synthetic analog. DIMIT (at the same dose), with sacrifice on the 5th day. Activities of 3‐N‐acetylglucosaminidase, β‐galactosidase, $aP‐***ucosidase, and β‐glucuronidase were assayed in homogenates of colon divided into two segments, proximal and distal. Administration of hormones did not influence the protein content of the colonic segments, but exhibited an effect on enzymatic activity, depending on age and on colonic segment. Specific activity of the first three enzymes was precociously decreased in the proximal segment in the younger rats (5 and 8 days old), but not in the older groups (11 and 15 days old). No effect of these hormones was noted on the activities of these enzymes in the distal segment.
Colonic Stricture Complicating Formula Protein Intolerance Enterocolitis
Authors: Schwarzenberg Sarah Jane , Whitington Peter F.
Published: January 2024

Cases of formula protein intolerance (FPI), in which many of the features of neonatal necrotizing enterocolitis (NEC) have been exhibited (vomiting, diarrhea, hematochezia, abdominal distention, and pneumatosis intestinalis). have been reported (1–6). This case of combined cow's milk and soy protein intolerance illustrates further the difficulty in distinguishing clinically between NEC and FPI. Moreover, it demonstrates that intestinal stricture is a potential long‐term complication of FPI. as it is also of NEC, and suggests that significant morbidity may result when the diagnosis of FPI is not considered as a cause of the NEC syndrome.
Calcium, Phosphorus, and Magnesium Contents of Human Milk During Early Lactation
Authors: Feeley Ruth M. , Eitenmiller Ronald R. , Jones J. Benton , Barnhart Harold
Published: January 2024

Early milk samples from 102 American mothers were examined for Ca, P, and Mg contents in relation to stage of lactation, intake of prenatal mineral supplements, maternal age, parity, and previous history of lactation. A total of 415 samples were collected at three stages of lactation: early transitional (4–7 days postpartum): transitional (10–14 days postpartum): and mature (30–45 days postpartum). No diurnal variations in element concentrations were observed in representative samples of late evening (PM) and early morning (AM) feedings collected during the transitional and mature stages. The mean concentrations for the major elements were highest in early transitional milk and in some cases decreased significantly (p < 0.05) as lactation progressed. Ca, P, and Mg contents (means ± SEM) were 26.3 ± 0.6., 14.6 ± 0.4, 5.3 ± 0.1 mg/100 g in early transitional milk and 26.2 ± 0.5, 13.3 ± 0.3, and 5.0 ± 0.1 in mature milk, respectively. Increasing uniformity in the elemental content of milk was noted among the mothers as lactation became established. No significant relationship was found between intake of dietary supplements containing Ca and Mg and levels of these elements in milk. Also, no significant correlations were found between maternal age, parity, or previous history of lactation and the elemental content of milk. From these data, it was estimated that fully breast‐fed infants would receive approximately 33, 18, and 6.5 mg/kg/day of Ca, P, and Mg, respectively, during the neonatal period.
Scanning Electron Microscopy of Soy Protein‐Induced Damage of Small Bowel Mucosa in Infants
Authors: Poley J. Rainer , Klein Albert W.
Published: January 2024

Observations are reported by scanning electron microscopy (SEM) of soy protein‐induced villous atrophy and mucosal recovery in two infants aged 5 weeks and 4 1/2 months. Whereas, by light microscopy, the mucosal lesions appeared similar, i.e., a flat mucosa, their appearance by SEM was different: the damage appeared more severe in the younger infant, although there was a shorter period of exposure to soy protein. The mucosal architecture was restudied 6 weeks after the initial biopsy. The degree of mucosal reconstruction was more advanced in the older infant—the one who showed less severe damage by SEM on the first biopsy. Although these investigations by SEM of damaged small bowel mucosa in soy protein intolerance did not contribute definite information to clarify the pathogenesis of villous atrophy in this condition, the injury was consistent with a lectin‐induced toxicity, similar to the one postulated for celiac disease. SEM seems eminently suited to study of the effect of interactions between environment and host at mucosal surfaces; and finer gradations of damage to small bowel mucosa can be determined better by SEM, while this is not possible by light microscopy. Of interest was the rather extensive colonization of the mucosal surface by microorganisms in one of the two patients. However, the contribution of microbial colonization to mucosal damage could not be assessed.
99mTc‐HIDA Cholescintigraphy in Children with Dubin‐Johnson Syndrome
Authors: Bujanover Yoram , Bar‐Meir Simon , Hayman Israel , Baron Jack
Published: January 2024

Tc‐HIDA cholescintigraphy in Dubin‐Johnson syndrome (DJS) demonstrates intense prolonged homogeneous visualization of the liver, together with delayed visualization of the gall bladder and extrahepatic ducts. Such a cholescintigram has not been described for any other hyperbilirubinemic state. Because it is a nonin‐vasive and easy technique, it is recommended for the evaluation of children suspected of having DJS.
Partial Hydrolysis of Cow's Milk Proteins by Human Trypsins and Elastases In Vitro
Authors: Jakobsson Irene , Borulf Stefan , Lindberg Tor , Benediktsson Birgitta
Published: January 2024

The hydrolysis of bovine a‐lactalbumin, β‐lactoglobulin, and casein by human anodal and cathodal trypsins and elastases was studied with the aid of electroimmunoassay and sodium dodecyl sulfate‐polyacrylamide gel electrophoresis. The rate of hydrolysis of the various proteins by cathodal elastase exceeded that by anodal or cathodal trypsin and anodal elastase. Casein was hydrolyzed more efficiently than a‐lactalbumin or β‐lactoglobulin. The hydrolysis of the three proteins occurred at a considerably slower rate when present in crude form, as in cow's milk, than when in purified form.
Linoleic Acid Absorption from Lipid Supplements in Patients with Cystic Fibrosis with Pancreatic Insufficiency and in Control Subjects
Authors: McKenna Mary C. , Hubbard Van S. , Bieri John G.
Published: January 2024

To determine the relative role of malabsorption as the cause of decreased linoleic acid in blood and tissue lipids of patients with cystic fibrosis (CF) and pancreatic insufficiency, the increase in plasma linoleic acid was determined after ingestion of various lipid supplements. CF patients with documented pancreatic insufficiency and normal control subjects were given each of four different lipid supplements on separate days (a minimum of 3 days apart). The supplements were commercial safflower oil, Microlipid, Captex 810D, and Captex 810B. Fasting subjects consumed 36 g of lipid in a milk shake containing 15 g of protein and 45 g of carbohydrate. Plasma samples obtained at 0, 2, 4, 6, and 8 h after the meal showed that CF patients absorbed linoleic acid from all of the lipid preparations tested when administered with their regular dose of pancreatic enzyme supplement. The mean maximal increase in percent plasma linoleic acid in CF patients was not different from controls after ingestion of safflower oil, Microlipid, and Captex 810B. With Captex 810D the CF patients had a significantly higher increase in percent plasma linoleic acid than controls, 6.75% vs. 2.27%, respectively, at 2 h (p < 0.01), and 11.10% vs. 4.65% at 8 h(p < 0.01). The CF patients also appeared to absorb the Captex products faster than controls, suggesting that presence of medium chain length fatty acids in these structured lipids facilitated their utilization by CF patients. The results indicate that malab‐sorption alone cannot account for the inadequate or marginal essential fatty acid status of CF patients. The fact that linoleic acid was absorbed as well by CF patients as controls in the present study suggests that CF patients in many previous supplementation studies either have not been given sufficient linoleic acid or have used the linoleic acid ingested to meet caloric needs. There is increasing evidence that adequate total caloric intake may be a far more important factor in determining the essential fatty acid status of CF patients than previously recognized. Our results suggest that long‐term consumption of supplemental linoleic acid in addition to adequate caloric intake should improve the linoleic acid status of most, if not all, CF patients.
Giardiasis with Protein‐Losing Enteropathy
Authors: Sutton D. L. , Kamath K. R.
Published: January 2024

Generalized edema and ascites were the main presenting features of giardiasis in a 3‐year‐old boy. Hy‐poalbuminemia, jejunal villous atrophy, Giardia lamblia in the duodenal aspirate, and abnormal gastrointestinal protein loss were present before therapy with metronidazole. Eradication of parasites resulted in complete clinical, histological, and biochemical remission. Giardiasis must now be included in the etiology of protein‐losing enteropathy.
Magnesium Metabolism Studies in Children with Chronic Inflammatory Disease of the Bowel
Authors: LaSala Marie‐Agnes , Lifshitz Fima , Silverberg Mervin , Wapnir Raul A. , Carrera Eduvigis
Published: January 2024

Magnesium metabolism was studied in seven patients with severe chronic inflammatory disease of the bowel (CIDB), and in 20 children without intestinal pathology. Four of the CIDB patients had ulcerative colitis and three had granulomatous disease of the bowel. All had diarrhea as well as other gastrointestinal complaints for 1 to 6 years prior to the study. All were being treated with sulfasalazine and were also receiving corticosteroids intermittently. All but one had had intestinal surgery. Basal plasma and urine were obtained in all patients and, if surgery was performed, a piece of muscle was excised. The CIDB patients received an intravenous magnesium infusion of 2 mEq/kg/day for 4 days, 2 days postsurgery. Electrocardiograms were recorded throughout the study. The mean basal plasma magnesium levels were reduced in CIDB patients as compared with controls. Mild hypomagnesemia was observed in six of seven CIDB patients. The mean basal urine excretion of magnesium was also reduced in CIDB patients, but the muscle concentrations of this element were similar to controls. Basal hypomagnesuria was present in only two of the three patients with granulomatous disease and in one patient with ulcerative colitis. The three patients with granulomatous disease excreted minimal amounts of magnesium in the urine during intravenous administration of this ion. A positive magnesium balance persisted throughout the 4‐day period of infusion. In contrast, only two of the four patients with ulcerative colitis had magnesium retention during the first day of intravenous administration, and all four had negative magnesium balances thereafter. The data suggest that hypomagnesemia in CIDB patients may occur with or without magnesium deficiency. However, the excretion of magnesium in urine after a parenteral magnesium load was the best index of magnesium deficiency. In three CIDB patients, with involvement of small bowel, magnesium depletion was found.
Homeostatic Mechanisms in the Utilization of Exogenous Iron in Children Recovering from Severe Malnutrition
Authors: Caballero Benjamin , Solomons Noel W. , Batres Ramiro , Torún Benjamin
Published: January 2024

We evaluated the role of initial iron stores on iron accumulation during recovery from severe edematous protein‐energy malnutrition in children. Twenty‐six preschool children were divided in two groups according to their initial iron reserves, as estimated from serum ferritin concentration, using a cutoff criterion of 30 ng/ml. The low ferritin (LF) group had a mean serum ferritin level of 12 ± 8 ng/dl, and the high ferritin (HF) group, 86 ± 32 ng/dl. Both groups had similar degrees of malnutrition and of anemia, as defined by hemoglobin concentration. All children received an adequate therapeutic diet and 60 mg iron daily as ferrous sulfate. The recovery of biochemical and anthropometric indicators of nutritional status, as well as of hemoglobin concentration, was similar in both groups. On the contrary, the LF group showed a marked increase in serum ferritin concentration from the onset of treatment, whereas the HF group had a net decline in this parameter by 30 days, and a stable level thereafter. The difference in serum ferritin concentration between groups was maintained until day 60, and both groups ended the study (90 days) with similar levels. Estimation of the utilization of exogenous iron from changes in total‐body iron during the first 60 days of recovery showed the LF group to retain an average of 9.3% of iron intake, whereas the HF group retained only 1.4%. These results confirm that similar degrees of anemia in severe malnutrition can be associated with markedly different iron reserves, and suggest that the homeostatic regulation of intestinal iron absorption by the storage iron pool may produce a lower utilization of oral iron supplements in malnourished children with high iron reserves at the onset of treatment.
Membranous Glomerulonephritis in a Patient with Crohn's Disease of the Small Bowel
Authors: O'Loughlin E. V. , Robson L. , Scott B. , Alexander F. , Gall D. G.
Published: January 2024

A 12‐year‐old girl presented with concurrent onset of membranous glomerulonephritis and Crohn's disease of the small intestine. Subsequent investigations failed to implicate any other systemic disease as a cause of the giomerulonephritis. Membranous giomerulonephritis is an immune complex‐mediated glomerulopathy frequently associated with underlying systemic disease: The association of immune complex phenomenon with Crohn's disease and the variation of renal abnormalities in parallel with activity of bowel disease in this patient suggest that the glomerulonephritis may be due to the underlying Crohn's disease.
Intrahepatic Cholangitis Associated with Mucocutaneous Lymph Node Syndrome
Authors: Edwards Kathryn M. , Glick Alan D. , Greene Harry L.
Published: January 2024

Although gallbladder hydrops occurs frequently in patients with mucocutaneous lymph node syndrome (MCLS), the etiology of the gallbladder lesion is unclear. Recently we performed a liver biopsy on a child with severe MCLS and demonstrated selective invasion of biliary ductular epithelial cells by polymorphonuclear leukocytes with sparing of the hepatocytes. The possible role of the selective destruction of biliary cells in the pathogenesis of biliary disease in MCLS is discussed.
γ‐Glutamyl Transpeptidase Activity and Its Serial Measurement in Differentiation Between Extrahepatic Biliary Atresia and Neonatal Hepatitis
Authors: Fung K. P. , Lau S. P.
Published: January 2024

The liver functions of 17 babies with extrahepatic biliary atresia (EHBA) and 19 babies with neonatal hepatitis (NNH) were analyzed. The serum γ‐glutamyl transpeptidase (GGTP) level and its rate of rise were significantly higher in the EHBA group. Both cross‐sectional and longitudinal studies showed a positive cor‐relation between the GGTP activity and the duration of the disease. The diagnostic accuracy is 79% for EHBA and 95% for NNH. It is suggested that GGTP and its rate of rise are the most important biochemical parameters in the differential diagnosis of these two diseases.
Nitrogen and Mineral Balance in Preterm Infants Fed Human Milks or Formula
Authors: Schanler Richard J. , Oh William
Published: January 2024

Growth as well as nitrogen, calcium, sodium, and potassium balances were evaluated in 16 preterm infants weighing less than 1,600 g at birth, who were fed either their mother's milk, donated mature human milk, or standard commercial formula. Birthweight, gestational age, age of balance, and energy and fluid intakes were similar between groups. There were no differences between groups in the rate of growth. The infants fed their mother's milk (obtained 11–30 days into lactation) demonstrated nutrient balance similar to infants fed mature human milk. Infants fed standard commercial formula demonstrated significantly greater intake and retention of calcium compared to either human milk group. Infants fed either their mother's milk or mature human milk demonstrated net nitrogen and calcium retention below estimates of fetal nitrogen and calcium accretion. Infants fed standard formula demonstrated retentions that more closely approach the fetal estimates. This study did not demonstrate an advantage to feeding premature infants their mother's milk when compared to the feeding of mature donor milk.
Postnatal Development of Bile Secretory Physiology in the Dog
Authors: Tavoloni Nicola , T. Jones Mary Jane , Berk Paul D.
Published: January 2024

To determine whether bile formation in the dog is an immature process at birth, several determinants of bile secretion were studied in anesthetized, bile duct‐cannulated puppies of 0–42 days of age and adult dogs. Basal canalicular bile flow rate, estimated by 14C‐erythritol biliary clearance, averaged 0.182 μl/min/g liver in 0–3 day‐old puppies and increased to 0.324 and 0.461 μl/min/g in puppies 7–21 and 28–42 days of age, respectively. The latter two values were not significantly different from that observed in adult dogs (0.348 μl/min/g). This age‐related increase in canalicular bile flow could be accounted for primarily by an increase in bile acid independent bile flow (0.103, 0.277, 0.375, and 0.252 μl/min/g in 0–3, 7–21, and 28–42 day‐old puppies and adult dogs, respectively). Calculated ductular bile water reabsorption (14C‐erythritol biliary clearance‐bile flow) was virtually absent in 0–3 day‐old puppies (0.005 μl/min/g), and averaged 0.017 and 0.092 μl/min/g in puppies of 7–21 and 28–42 days of age, respectively. In adult dogs, ductular bile water reabsorption was 0.132 μl/min/g. These functional deficiencies of the newborn dog were associated with an increased biliary permeability to 3H‐inulin which could not be accounted for solely by an increased solute diffusion due to the lower rate of canalicular bile flow. Administration of taurocholate up to 2000 nmol/min/kg produced in all animals a similar increase in canalicular bile flow and bile acid excretion, and was not associated with changes in ductular bile water reabsorption rate. These findings are interpreted to indicate that, in the dog, bile secretory function is immature at birth and develops during postnatal life.
Effects of Dietary Zinc Deficiency on Hepatic Ornithine Carbamoyltransferase and Alcohol Dehydrogenase Activities in Rats
Authors: Indo Yasuhiro , Nagata Noriyuki , Higashi Akimasa , Matsuda Ichiro , Kashiwabara Norio , Nakashima Ichiro
Published: January 2024

Hepatic ornithine carbamoyltransferase (OCT) and alcohol dehydrogenase (ADH) activities were measured in six groups of rats: (A) fed a severe zinc‐(Zn‐) deficient diet (1.98 ppm) for 5 weeks; (B) pair‐fed control for group (A); (C) fed a less severe Zn‐deficient diet (6.10 ppm) for 5 weeks; (D) pair‐fed control for group (C); (E) fed a Zn‐supplemented control diet (90.4 ppm) for 5 weeks; and (F) first fed the severe Zn‐deficient diet for 5 weeks and then replaced on the Zn‐supplemented control diet until a body weight corresponding to the final weight of group (E) was obtained. Hepatic OCT was similar in all these six groups. On the contrary, hepatic ADH was significantly reduced in groups (A) and (C) and in each of the corresponding pair‐fed groups, (B) and (D). No differences were found between groups (A) and (B) or between groups (C) and (D). In group (F), ADH activity improved to a level equivalent to that in group (E). The changes in ADH activities were accompanied by changes in the hepatic Zn content. Thus, it is clear that: (1) the hepatic Zn content may not be affected by the amount of Zn intake alone, but by the combination of Zn and food intake; and (2) ADH, and not OCT, reflected the hepatic Zn content.
Amebiasis Presenting as Rectal Bleeding Without Diarrhea in Childhood
Authors: Jammal Mary Ann , Cox Kenneth , Ruebner Boris
Published: January 2024

A 6‐year‐old boy with no history of foreign travel had presented with rectal bleeding without diarrhea for 2 months. Despite negative stool cultures, a diagnosis of intestinal amebiasis was made by colonoscopy with tissue biopsy. In cases of persistent rectal bleeding, even without diarrhea, a diagnosis of amebiasis should be considered. When routine stool parasite examinations are negative, unsuspected amebiasis may be diagnosed by sigmoidoscopy or colonoscopy with tissue biopsy.
Lymphoid Polyps of the Rectum
Authors: McNicholas T. , Brereton R. J. , Raafat F.
Published: January 2024

In children lymphoid polyps of the rectum are uncommon benign lesions with a good prognosis following local treatment. We report a girl in whom a cluster of lymphoid polyps occurred 9 months following removal of a single lymphoid polyp. All of the polyps, including the original lesion, displayed a monoclonal nature on immunocytochemical examination.
Effect of Non‐Nutritive and Nutritive Suck on Gastric Emptying in Premature Infants
Authors: Szabo Joanne S. , Hillemeier A. Craig , William O h
Published: January 2024

Ten healthy preterm infants were studied to determine if non‐nutritive and nutritive suck significantly altered gastric emptying patterns when compared with gav‐age feeding alone. We used a 10% dextrose meal with phenol red marker and a double sampling technique to determine gastric emptying at 10‐min intervals over a 30‐min test period. A crossover study design compared the effects of the three feeding methods in each infant. The gastric residual volumes expressed in milliliters per kilogram did not differ significantly when comparisons were made among groups at 10, 20, and 30 min following the test meal. Non‐nutritive suck and nutritive suck and swallow of a liquid dextrose meal do not significantly improve gastric emptying in healthy preterm infants. The beneficial effects of non‐nutritive and nutritive sucking on the nutritional status of preterm infants, demonstrated by others, are not related to improved gastric evacuation of feeds. Alternative explanations for these beneficial effects require further investigation.
Influence of Epidermal Growth Factor on the Maturation of the Fetal Mouse Duodenum in Organ Culture
Authors: Beaulieu J. F. , Ménard D. , Calvert R.
Published: January 2024

The purpose of this work was to study the direct influence of epidermal growth factor (EGF) on the maturation of the fetal mouse duodenum in organ culture. Duodenal explants, resected at 17 days of gestation, were cultured during 48 h at 37°C in Leibovitz L‐15 serum‐free medium alone or supplemented with EGF (100 ng/ml). Differentiation of absorptive cells was evaluated by measuring brush border hydrolytic activities. After 48 h of culture with and without EGF, villous architecture and the fine structural characteristics of the tissues are preserved. In control explants, the level of alkaline phosphatase, maltase, trehalase, and sucrase activities as well as the protein and DNA contents remain comparable to the values measured in 17‐day explants at the beginning of the culture period, while lactase activity falls drastically. In explants cultured with EGF, the level of alkaline phosphatase, maltase, and trehalase activities and the protein contents significantly increase while sucrase activity and DNA contents are unchanged, and lactase activity remains under the onset level. From these results, it was concluded that EGF influences directly the maturation of some brush border enzymes in the duodenum during the fetal period.
Intestinal Tapering and Lengthening for Short Bowel Syndrome
Authors: Thompson Jon S. , Vanderhoof Jon A. , Antonson Dean L.
Published: January 2024

Intestinal tapering and lengthening were performed in a child with short bowel syndrome to prevent the need for home parenteral nutrition. The intestinal lengthening was not successful, presumably because the vascular anatomy was not favorable. Significant clinical improvement and avoidance of home parenteral nutrition resulted from the tapering procedure. These procedures have a role in the treatment of selected patients with short bowel syndrome
Platelet Monoamine Oxidase Activity in Reye's Syndrome
Authors: Faraj Bahjat A. , Newman Stephen L. , Caplan Daniel B. , Ahmann Peter A. , Kutner Michael , Ali Farouk M. , Lindahl Julie A.
Published: January 2024

Platelet and liver monoamine oxidase (MAO) activity (mean ± SD) was evaluated in patients with liver‐biopsy‐proven Reye's syndrome. MAO was measured by a radioenzymatic technique with [3H]tyramine as a substrate. A marked decrease in MAO activity [3.3 ± 2.4 nmol of [3H]4‐hydroxyphenylacetic acid formed X (mg protein)‐1 x h‐1] was observed in platelets on admission in all patients (n = 13) with Reye's syndrome when compared with hospitalized patients without liver disease (n = 8) [9.8 ± 2.5 nmol of [3H]4‐hydroxyphenylacetic acid formed x (mg protein)‐1 x h‐1] and with liver disease (n = 10) [9.1 ± 2.0 nmol of [3H]4‐hydroxyphenylacetic acid formed x (mg protein)‐1 x h‐1]. Following recovery from the disease, platelet MAO approached levels that were not significantly different from those of controls. Contrastingly, reduction of hepatic MAO in Reye's syndrome was similar to that seen in patients with liver disease of different etiologies. These studies suggest that reduced platelet MAO activity is a specific abnormality in Reye's syndrome, and it may be representative of generalized impairment of mitochondrial function in these patients. Furthermore, the pattern of liver and platelet MAO activity in Reye's syndrome may allow for the differentiation of this disease from other hepatopathologic conditions.
Rumination Syndrome in Children Treated by Increased Holding
Authors: Whitehead William E. , Drescher Vincent M. , Morrill‐Corbin Elizabeth , Cataldo Michael F.
Published: January 2024

Rumination syndrome the frequent regurgitation of previously ingested food into the mouth where it is chewed is a common, life‐threatening disorder of retarded individuals. Four cases are described in which holding a retarded, ruminating child for 10–15 min before, during, and after meals, was associated with remission of rumination. Simple holding was effective in three; in the fourth, it was necessary to punish the child by putting her into a separate room for 3 min immediately after regurgitations. A within‐subject reversal experimental design suggested that holding and not simple distraction was the effective component of the treatment. Treatment benefits were well maintained when the child returned to a home environment in which he or she continued to be held periodically. It is proposed that there are two behavioral etiologies for idiopathic rumination syndrome social deprivation and reward learning through increased attention for regurgitation. Holding is the treatment of choice for the first type, and punishment with time out may be necessary to suppress regurgitation in the second type.
Intestinal Sugar Permeability
Authors: Ford R. P. K. , Menzies I. S. , Phillips A. D. , Walker J. A. , Turner M. W.
Published: January 2024

The permeability of the intestine was studied in 39 children (1 month to 3 years of age) with diarrhoea and in 28 children (6 months to 15 years of age) undergoing duodenal biopsy. Permeability was measured by differential absorption from an isotonic oral load containing 3.5 g lactulose, 0.5 g L rhamnose, 0.5 g D xylose, and 5 g lactose. Urinary sugar excretion was determined by quantitative thin layer chromatography. Children with acute gastroenteritis had a greatly increased permeability, with a mean lactulose/L rhamnose excretion ratio of 0.43 ± 0.31 (normal < 0.07). Children retested 3 16 weeks after complete recovery of their gastroenteritis had normal permeability (0.045 ± 0.018). Children with chronic diarrhoea also had an increased permeability (0.12 ± 0.074), but significantly less than the acute gastroenteritis group (p < 0.01). Abnormal proximal small bowel morphology was associated with increased permeability, and a strong correlation between crypt depth and permeability was observed (r = 0.66, p < 0.001). Abnormal intestinal permeability was associated with diarrhoeal disease and with mucosal damage. It appears to be a reliable and useful index of mucosal integrity.
Early Intravenous Correction of Vitamin E Deficiency in Premature Infants
Authors: Gutcher Gary R. , Farrell Philip M.
Published: January 2024

We undertook to determine the efficacy of intravenousα tocopheryl acetate in rapidly correcting the vitamin E deficiency of the premature infant. Twenty nine infants were assigned to either a control or treatment group. The latter group received a median intravenous dose of 3 IU/kg/day α tocopheryl acetate as MVI 12 (USV Pharmaceuticals, Inc.). On days 1, 2, 3, 7, 14, and 21, plasma tocopherol isomers and peroxide induced hemolysis were analyzed. While all but one control infant with initial tocopherol deficiency were still deficient on day 3, all but two of the treatment infants were normal. Rapid, safe correction is possible with an intravenous multivitamin preparation.
Efficient Storage System for Breath Hydrogen
Authors: Murray Robert D. , Kerzner Benny , MacLean William C. , McClung H. Juhling , Gilbert Merry
Published: January 2024

Summary:

Recommended materials for breath hydrogen collection (plastic syringes with twist lock closure) are only adequate for relatively brief periods because of gradual hydrogen loss and considerable variability between duplicate samples. To document the most favorable storage conditions for breath hydrogen, we compared hydrogen retention in plastic syringes using a conventional twist‐in‐lock closure versus a simple, inexpensive syringe closure, a Critocap. Hydrogen retention was studied at 25, 5, and – 20°C in two different syringe brands over 72 h of storage. An analysis of variance confirms the superiority of Critocaps over twist‐in‐lock closures (p < 0.001). Reliability was maximal when samples were placed in environments <5°C. When storage time was extended to 7 days, mean hydrogen retention was 86 ± 6% (± SD).
Crohn's Disease Activity
Authors: Zielen S. , Bender S. W. , Kreuz W. , Posselt H. ‐G. , Solem E. , Scharrer I.
Published: January 2024

Summary:

Twenty‐eight patients (mean age 13.5 years) with proven Crohn's disease were examined for factor VIII coagulation activity (VIII:C), factor VIII‐related antigen (VIII R:Ag), and factor VIII‐related ristocetin cofactor activity (VIII R:RCF). The factor VIII coagulation proteins were correlated with disease activity index according to van Hees and were compared with inflammatory parameters such as erythrocyte sedimentation rate, c‐reactive protein, and orosomucoid. Among the factor VIII proteins, factor VIII‐related protein (VIII R:Ag) correlates best with van Hees disease activity index, orosomucoid, c‐reactive protein, and erythrocyte sedimentation rate (p < 0.001 for all parameters). Since acute phase proteins are produced by liver cells, whereas VIII R:Ag is synthesized by endothelial cells, we postulate that this coagulation factor is highly sensitive in reflecting the inflammatory process in the intestine.
Serial Serum 25‐Hydroxyvitamin D and Mineral Homeostasis in Very Premature Infants Fed Preterm Human Milk
Authors: Hillman Laura S. , Salmons Sharon J. , Slatopolsky Eduardo , McAlister William H.
Published: January 2024

Summary:

Fourteen very low birthweight infants (mean ± SD 1,070 ± 180 g and 29.3 ± 1.9 weeks gestation) fed their own mother's milk were clinically followed until 3–4 months of age with frequent measurements of serum calcium, phosphorus, magnesium, 25‐hydroxyvitamin D (25‐OHD), parathyroid hormone, alkaline phosphatase, and albumin, and urine calcium, phosphorus, and magnesium. These infants were matched for birthweight and gestation with 14 infants (1,075 ± 152 g and 29.0 ± 1.7 weeks) who had been similarly followed during concomitant studies of infants fed standard formula (Similac 20 cal/oz). Urine phosphorus was markedly lower in the breast milkfed group from initiation of feedings, and serum phosphorus became significantly lower at and after 6 weeks of age. The fall in serum phosphorus was accompanied by a marked calciuria. Parathyroid hormone was suppressed in the breast milk‐fed group, although serum calcium was not elevated and did not differ from formula‐fed infants. A high incidence of moderate‐severe hypomineralization on radiographs was seen in both breast milk‐ and formula‐fed groups. Six of 14 breast‐fed infants required phosphorus supplementation at 8–10 weeks of age because of significant hypophosphatemia. hypercalciuria, and hypomineralization. These infants differed from those not requiring phosphorus supplements by being smaller at birth but not of lower gestation, and having persistently low serum 25‐OHD at and after 6 weeks of age.
Effect of Protein Source and Iron Content of Infant Formula on Stool Characteristics
Authors: Malacaman Edgardo E. , Abbousy Fadhil K. , Crooke Donald , Nauyok George
Published: January 2024

Summary:

Formula‐fed, term infants were evaluated in two studies that assessed the influence on stool characteristics of protein source and iron content of the formula. Parental observations of infants' stool characteristics were recorded on standard forms. Green was the primary stool color for infants receiving whey‐predominant formula containing iron at 12 mg/L. A low‐iron (1.5 mg/L), wheypredominant formula produced yellow stools. A caseinpredominant, iron‐fortified (12 mg/L) formula produced primarily yellow or brown stools, although many of the infants fed this formula also had green stools. Soft stools were common in infants fed either whey‐or casein‐predominant formulas. However, more infants receiving whey‐predominant formulas passed watery or slimy stools than did infants receiving casein‐predominant formulas. We conclude that green stools in the absence of signs of disease should be considered a normal variation of stool cofor.
Assessment of Intestinal and Cardiorespiratory Function in Children with Congenital Heart Disease on High‐Caloric Formulas
Authors: Yahav J. , Avigad S. , Frand M. , Shem‐Tov A. , Barzilay Z. , Linn S. , Jonas A.
Published: January 2024

Summary:

Fourteen infants with congenital heart disease were investigated for failure to thrive. Assessment of intestinal function revealed minor absorptive abnormalities (mild steatorrhea in three patients, bile salt loss in four patients), delayed gastric emptying, and abnormal triglyceride loading tests. Low caloric intake (88.3 ± 19.3 kcal/kg/day) seemed the main reason for failure to gain weight. Weight accession and cardiorespiratory rates were monitored daily during voluntary intake, a high‐caloric diet by mouth, and nasogastric tube feeding. Providing 169 ± 29 kcal/kg/day by tube resulted in weight gain with mild and transient elevation of respiratory rate at the end of the meal and increased heart rate 90 min after the meal. This‐regimen is a metabolically inexpensive and efficient method of supporting weight gain in children with congenital heart disease.
Impairment of Lipid Emulsion Metabolism Associated with Carnitine Insufficiency in Premature Infants
Authors: Yeh Yu‐Yan , Cooke Richard J. , Zee Paulus
Published: January 2024

Summary:

Carnitine status and its relation to lipid metabolism were determined in 11 premature infants (<34 weeks of gestation) receiving parenteral nutrition. Intravenous administration of lipid emulsion increased serum concentrations of free fatty acids at 2, 4, and 8 h after infusion. Despite this increase, serum levels of ketone bodies remained low reaching only 40 to 45% of levels in full‐term infants receiving enteral feeds. In premature infants, levels of plasma total and free carnitine were about half those in full‐term infants and were not altered by lipid infusion. The results indicate limited potential for lipid emulsion as a source of energy in premature infants, possibly because carnitine insufficiency restricts fatty acid oxidation.
Solitary Rectal Ulcer Syndrome in a Teenaged Boy
Authors: Sondheimer Judith M. , Slagle Terri A. , Bryke Christine R. , Hill Rolla B.
Published: January 2024

Summary:

Since the original description of solitary rectal ulcer by Cruveilheir in 1830, about 250 cases have been reported (1). The condition most frequently presents in adults between 30 and 50 years of age. There are very few pediatric case reports and none have come from North America. We report here a 13‐year‐old boy with solitary rectal ulcer syndrome and a review of the pediatric experience with this rare condition.
Nephritis Associated with an Infected Peritoneovenous LeVeen Shunt
Authors: Salcedo Jose R. , Sorkin Lawrence
Published: January 2024

Summary:

A patient with chronic active hepatitis and medically unmanageable ascites developed staphylococcal bacttfcaua after insertion of a peritoneovenous LeVeen shunt. The patient developed glomerulonephritis, manifested by moderate proteinuria, microscopic hematuria with red cell casts, azotemia, and evidence of complement activation. The protracted infection, urinary, and renal abnormalities subsided after removal of the LeVeen shunt and a 3‐week course of antibiotics. The clinical course of this patient is typical of the ± mediated glomer ulonephritis that has been previously well described in hydrocephalic patients with chronically infected ventriculoatrial shunts.
Digestion and Absorption Rates of Lactose, Glucose, Galactose, and Fructose in Three Infants with Congenital Glucose‐Galactose Malabsorption
Authors: Beyreiss K. , Hoepffner W. , Scheerschmidt G. , Müller F.
Published: January 2024

The digestion rates of lactose and the absorption rates of glucose, galactose, and fructose were studied by continuous perfusion of the jejunum in three patients aged 7 weeks to 9 months with congenital glucose‐galactose malabsorption (infusion rate:1.0 ml min‐1; concentration of each sugar: 200 mM; perfusion distance: 30 cm). The mean absorption rates of glucose and galactose were 26.5 and 43.8 $mUmol min‐1 30 cm‐1, respectively, and were significantly reduced (p < 0.001) to 13 and 22%, respectively, of intake. On the other hand, the absorption of fructose was 133.3 $mUmol min‐1 30 cm‐1, i.e., as high as in the controls. The hydrolysis rate of lactose was also normal (134.0 $mUmol min‐1 30 cm‐1). However, the absorption rates of glucose and galactose released from the disaccharide were as low as the perfusion of free monosaccharides. In patients with glucose‐galactose malabsorption the glucose absorption rate is as low as that of galactose. No additional glucose transport system seems to exist. A hydrolase related transport system is also of no importance in compensating for the primary defect of monosaccharide absorption.
Influence of Hydrocortisone on Human Fetal Small Intestine in Organ Culture
Authors: Arsenault Pierre , Ménard Daniel
Published: January 2024

The influence of hydrocortisone on the differentiation and proliferation of human fetal small intestine was studied. Fetal intestine (12− to 14‐week gestation) was cultured during 5 days at 37$dEC in serum‐free Leibovitz L‐15 medium alone or supplemented with hydrocortisone (12.5, 25, and 50 ng/ml). The addition of different concentrations of hormonc did not affect the morphology of the intestinal explants. Brush border membrane hydrolytic activities, namely, sucrase, lactase, glucoamylase, trehalase, and alkaline phosphatase activities, were assayed in the intestinal tissue. A specific increase of lactase and alkaline phosphatase activities was induced by the addition of 25 and 50 ng hydrocortisone/ml culture medium. The DNA synthesis evaluated by the incorporation of [3H]thymidinc was increased by the addition of 50 ng hydrocortisone/ml. The sites of incorporation into the different layers of the intestinal wall were studied by radioautography. The incorporation of the radioactive precursor occurred mainly in the epithelium and to a lesser degree in the mesenchyme and muscular layers. Labeled epithelial nuclei were located in the intervillous areas and developing crypts but not on the villi. The addition of hydrocortisone induced a significant increase of the labeling index of the epithelial cells. The present work provided for the first time some basic data on the influence of hydrocortisone on brush border hydrolytic activities and on epithelial cell proliferation of human fetal small intestine.
Immunoregulatory Functional Abnormalities in Children Affected by HBsAg‐Positive Chronic Active Hepatitis
Authors: Pignata Claudio , Vajro Pietro , Monaco Giuseppe , Fontanella Angiola
Published: January 2024

Suppressor cell function was evaluated in children affected by HBsAg‐positive chronic active hepatitis. Circulating concanavalin A‐ (ConA) precultured lymphocytes failed to suppress the proliferative response of autologous responder cells to a mitogen. In four of eight patients with a failure of ConA‐induced suppressor activity, indomethacin added during the induction phase of T suppressor cells abolished the defect, indicating that prostaglandin‐producing adherent cells may influence ConA‐induced suppressor activity. An inverse relation ship between suppressor cell activity and the number of suppressor/cytotoxic subsets defined by the OK T8 monoclonal antibody was found. Our findings strongly support the hypothesis that an abnormality in the immunoregulatory system plays a role in the pathogenesis of HBsAg‐related chronic active hepatitis. It is also suggested that non‐T regulatory cells are implicated in the immunological abnormality in chronic active hepatitis.
Evaluation of Microvilli in Gluten‐Sensitive Enteropathy by Means of Scanning and Transmission Electron Microscopy
Authors: Hardoff D. , Levanon D. , Gitay H. , Nir I.
Published: January 2024

Biopsies from jejunum of nonceliac patients and patients with active celiac disease were studied and compared by means of transmission (TEM) and scanning (SEM) electron microscopy. Fracturing of dried specimens enabled the visualization of the epithelial microvilli with SEM. Alteration in microvillous appearance in celiac patients, as determined by TEM, could also be clearly detected in SEM images. The SEM preparation is easier to accomplish and less time‐consuming compared to the TEM. This may be an advantage for clinical assessment of jejunal biopsies in syndromes in which early detection of alterations in microvilli in the epithelial mu‐cosa is of diagnostic value.
Childhood Celiac Disease
Authors: Shmerling David H. , Franckx Johan
Published: January 2024

The diagnosis of celiac disease (CD) was uniformly established between 1960 and 1983 in 314 infants and children according to the following criteria: a flat proximal small bowel mucosa in untreated patients and an unequivocal response and remission on a gluten‐free diet (GFD). In 91 patients, one or more interruptions of the GFD (challenges) were documented by repeated intestinal biopsies. Sixty‐eight percent of the challenges were initiated by the patients themselves. Seventy‐one patients (Group A, 81.4%) had a flat mucosa after 0.25–14.67 years off diet. In 11 (Group B, 12%), a successive deterioration of the mucosa occurred during 0.5–6.67 years after interruption of GFD without becoming flat. Six patients (Group C, 6.6%) had a normal intestinal mucosa, after 2.24–6.92 years off diet. Three patients are still under study, being off diet for less than 2 years. Median age at diagnosis was similar in all three groups (1.16, 1.16, and 1.12 years). Median duration of GFD prior to challenge was 4.54 years in Group A, 12.16 years in Group B (p < 0.001), and 6.38 years in Group C. A mucosal relapse within 2 years after the beginning of challenge was observed in 21 of 24 patients (87.5%) studied longitudinally during planned challenges. It is concluded that only a minority (6.6%) of CD patients, when diagnosed as above, will not relapse after an interruption of GFD and that another small group (12%) will deteriorate very slowly. Routine gluten challenges in all CD patients, interrupting established life and dietary habits and including repeated biopsies in the majority of patients who deteriorate and relapse, seem therefore not justifiable.
Retinol‐Binding Protein and Prealbumin
Authors: Helms Richard A. , Dickerson Roland N. , Ebbert Mary Lou , Christensen Michael L. , Herrod Henry G.
Published: January 2024

Plasma prealbumin (PA) and retinol‐binding protein (RBP) concentrations were serially measured in 25 critically ill, malnourished infants requiring parenteral nutrition to determine if these visceral protein markers are useful in assessing acute protein repletion. Significant increases in both proteins (p > 0.05) were noted as early as 5 to 7 days after institution of parenteral nutrition and continued significantly above baseline values through 2 weeks of observation. Gestational development (in infants less than 4 weeks old) and mean protein intake influenced visceral protein responses. Appropriate for ges‐tational age neonates had more rapid and quantitatively greater PA responses (p > 0.05) than small for gestational age neonates. Small for gestational age neonates never exceeded baseline RBP responses. Average protein intake of ≥ 2 g/kg/day resulted in PA and RBP concentrations below baseline and significantly lower than infants on higher protein intakes (p > 0.05), at the end of 2 weeks. Average calorie intake of < 100 cal/kg/day had no differential influence on PA or RBP when compared with infants on less calories. Prealbumin values correlated with RBP values observed simultaneously (r = 0.588, p > 0.0001). We conclude that PA and RBP are useful measures of protein repletion in critically ill infants requiring parenteral nutrition.