PERORAL ENDOSCOPIC DUODENAL BIOPSY IN INFANTS AND CHILDREN

ABSTRACT. Peroral endoscopic duodenal biopsy was used in the diagnosis and follow-up of forty-nine infants and children with suspected small intestine disease. Their ages ranged between one month and 12 years. Fifty-four upper gastrointestinal endoscopy procedures were performed and 139 biopsies were taken. The mean procedure time was 3.2 min with a range of 1.5–5.5 min. There were no complications. Tissue adequate for histopathologic examination was obtained in all but one of the fifty-four procedures. On the basis of this experience we think that peroral endoscopic duodenal biopsy is faster, safer and as diagnostic as conventional suction biopsy in infants and children. It is also more informative when other diseases of the upper gastrointestinal tract are suspected.     

TEMPORARY INTESTINAL LACTASE DEFICIENCY IN LIGHT-TREATED JAUNDICED INFANTS

Abstract The intestinal lactase activity in six newborn jaundiced light-treated infants with diarrhea and in eight normal controls were compared by lactose tolerance test (LTT). The ability to hydrolyze lactose was minimal in the jaundiced infants during light-treatment compared to the controls which could absorb lactose very well. Peroral intestinal biopsies were taken from the newborn jaundiced infants during light-treatment. By histochemical technique no intestinal lactase activity was found in these intestines. When the jaundiced infants with diarrhea were given lactose-free diet, the stools normalized. The effect was reversed when breast milk was given while the baby was still jaundiced and light-treated. These findings indicate that the increased amounts of unconjugated bilirubin in the intestine of jaundiced infants during light-treatment inhibit the intestinal brush-border lactase. When the icterus fades the lactase is again active. The practical consequence is to give light-treated infants lactose-free diet if they get diarrhea, and to reintroduce breast milk or other lactose containing diet when the baby is no longer icteric.     

JEJUNAL MUCOSA IN INFANTILE MALNUTRITION

Twenty-six infants with malnutrition and seven controls were studied by routine haematological tests, serum protein levels, glucose tolerance and D-Xylose absorption tests, jejunal biopsies and intestinal disaccharidase, assays. In conclusion, it can be said that in malnutrition the jejunal mucosa shows some mild or non-specific histologic changes compatible with malabsorption, which may be reversible. No correlation could be shown between these changes and absorption tests. Malnutrition may also result in secondary intestinal disaccharidase deficiencies which improve with correction of the nutritional state. These secondary enzyme deficiencies may be responsible for some of the diarrhoeas seen in marasmic children. No direct correlation between anaemia, iron-deficiency, folic acid levels, protein deficiency and intestinal mucosal changes could be established in this study.     

EFFECT OF INTRAVENOUS HYDROCORTISONE ADMINISTRATION ON GLUCOSE HOMEOSTASIS IN SMALL FOR GESTATIONAL AGE INFANTS

Abstract. The effects of I.V. hydrocortisone (H) (10 mg/kg) on glucose homeostasis were evaluated at 25 to 85 hours of age in 14 infants who were small for gestational age (SGA) in comparison to 17 control SGA infants. Three hours after H administration, higher levels of plasma glucose than in controls were detected (mean ±S.E.M.): 4.78±0.2 vs. 2.88±0.2 mmol/1 ( p <0.01), while lower levels were found for blood pyruvate (38±7 vs. 89±12 μmol/l— p <0.01), plasma insulin (6.4±0.5 vs. 12±0.8 μIU/ml— p <0.05) and plasma glucagon (62.25±6.6 vs. 81.6±6.6 pmol/l— p <0.05). Three hours after H administration, I.V. injection of l -alanine (150 mg/kg) produced a significant rise over baseline of plasma glucose concentration from 4.78±0.2 to 5.94±0.2 mmol/l at 50 min ( p <0.05), whereas no significant change was observed in controls. There was no significant change in plasma glucagon and insulin concentrations after l -alanine injection in either group. These results show that in SGA infants primed with H, the rise of plasma glucose concentration after l -alanine administration is observed with low plasma insulin levels and without stimulation of glucagon secretion. They suggest that H induced a reduced peripheral utilization of glucose by lowering the plasma levels of insulin and a production of glucose from alanine through gluconeogenesis.     

THE NOISE LEVEL IN A CHILDRENS HOSPITAL AND THE WAKE-UP THRESHOLD IN INFANTS

One hundred and twenty-six children, 3 to 63 weeks of age, have been exposed to a mixed noise (100-7000 Hz) in intensities between 50 and 80 dB under steady conditions of room acoustic during different periods of time between 10.30 p.m. and 01.00 a.m. A noise level of 75 dB led to obvious sleep disturbance or waking-up in ? of the children after 3 minutes and in all after 12 minutes. The waking threshold according to these investigations is higher than in adults. A 24-hour recording of the noise level in different types of infants' and children's care units demonstrated that the acoustic stress– especially by “interior working-noise”–in care units of light construction reaches or exceeds the obligate waking-noise threshold of infants during most of the day and night hours. This is regarded as a deficiency of hospital hygiene.     

ASSOCIATION BETWEEN MULTIPLE INTESTINAL ATRESIA AND OMPHALOCELE: A CASE REPORT

Multiple intestinal atresia is a rare disorder with vascular or hereditary etiology. So far, the occurrence of this malformation along with omphalocele has not been reported. We describe a boy born from a nonconsanguineous gypsy couple with intrauterine growth retardation, omphalocele, and multiple intestinal atresia from the pylorus to the rectum. The microscopic examination of the intestine shows multiple small lumina with a sieve-like appearance. This is characteristic of the hereditary atresias and suggests development of a defect in (re)canalization during embryogenesis. The association with omphalocele indicates a common developmental defect may be present.     

IRON RELEASE FROM THE STORES: A MECHANISM IN MAINTENANCE OF CONCENTRATION OF HEMOGLOBIN IN LOW-BIRTH-WEIGHT INFANTS

Abstract. Lundström, U. (Pediatric Hematology, Children's Hospital, University of Helsinki, Findland). Iron release from the stores: A mechanism in maintenance of concentration of hemoglobin in low-birth-weight infants. Acta Paediatr Scand, 69: 249, 1980.—After the resuming of the postnatal red cell production at two months of age infants are dependant on storage iron due to the great need for iron at a time when the iron content of the diet is low. This is even further accentuated in low-birth-weight infants. In this study the release of storage iron in the hemoglobin pool. During the two month period from two to four months of age at least 20 mg of iron per month was transferred from the storage sites for hemoglobin production. This amount represents 5 mg per kg of body weight and exceeds the rate iron was mobilized from storage sites in an adult male under experimental conditions. Rapid weight gain was associated with early depletion of iron stores. However, residual iron stores in infants with the slowest growth rate could not maintain the level of hemoglobin achieved in iron-supplemented low-birth-weight infants. These findings suggest that in rapidly growing low-birth-weight infants the need of iron for erythropoiesis is so great that iron deficient erythropoiesis may develop in the presence of iron stores if the diet is not supplemented with iron.     

PROTEIN-LOSING ENTEROPATHY WITH SMALL INTESTINAL STENOSES AND A STAGNANT LOOP IN A NINE-YEAR-OLD BOY

ABSTRACT. This report concerns a 9-year-old boy with protein-losing enteropathy (PLE), small intestinal stenoses and a stagnant loop. The diagnosis of PLE was made after a ⁵¹Cr-labelled albumin study, but that of intestinal stenoses only after repeated barium examinations. All the symptoms disappeared after resection of the lesions. In the investigation of any PLE, surgically treatable lesions must be identified.     

EVIDENCE FOR A LARYNGEAL CHEMOREFLEX IN SOME HUMAN PRETERM INFANTS

ABSTRACT. Studies in animals have documented the presence of a laryngeal chemoreflex. This reflex reflex in apnea when water is placed in the laryngeal area. This study of ten premature infants presents data that would support the existence of a similar reflex response in some premature infants. All ten infants studied had apnea of unknown etiology.     

VASOACTIVE INTESTINAL POLYPEPTIDE (VIP)-PRODUCING GANGLIONEUROMA IN A CHILD WITH CHRONIC DIARRHEA

Abstract. Hansen, L. P., Lund, H. T., Fahrenkrug, J. and Søegaard, H. (The Department of Paediatrics, Central Hospital, Randers, The Department of Clinical Chemistry, Bispebjerg Hospital, Copenhagen, and the Department of Pathology, Municipal Hospital, Árhus, Denmark). Vasoactive intestinal polypeptide (VIP)-producing ganglioneuroma in a child with chronic diarrhea. Acta Paediatr Scand, 69: 419, 1980. In a 2½-year-old girl with persistent watery diarrhea a retroperitoneal tumour and lymph node composed of benign ganglioneuroma cells were found. The histological picture was compatible with an original metastasizing neuroblastoma which has passed through a stage of late maturation. Electron microscopy of the ganglioneuroma showed cytological evidence of increased secretory activity. The tumour contained large amounts of vasoactive intestinal polypeptide (VIP) and preoperative plasma concentration of this peptide was increased. Postoperatively the VIP concentration was normalized, and the diarrhea ceased. Analysis of catecholamine metabolites in urine was normal. The findings suggest that the watery diarrhea was due to the benign VIP-producing neural crest tumour.     

INCIDENCE OF COELIAC DISEASE AND TRANSIENT GLUTEN INTOLERANCE IN CHILDREN IN A SWEDISH URBAN COMMUNITY

Abstract. The incidence of coeliac disease in children in the city of Malmö, South Sweden, was 1: 982 during 1966 to 1975. The diagnostic criteria were: flat intestinal mucosa on gluten-containing diet, free of symptoms, and improvement in mucosal morphology on gluten-free diet, and morphological and/or evident clinical relapse (three times) on gluten challenge. 6 (12 %) of 49 children with initially a flat mucosa still had a normal mucosa on a gluten-containing diet for two years or longer, having so-called transient gluten intolerance.     

A SUGGESTED ROLE FOR PRECOLOSTRUM IN PRETERM AND SICK NEWBORN INFANTS

ABSTRACT. Samples of precolostrum (colostrum gravidarum), colostrum and mature milk obtained from five women during their antenatal and postnatal periods were measured for IgA, IgG, IgM, alpha-1-antitrypsin, lactoferrin, lysozyme, B, A globulin (C3) and B, E globulin (C4) by single radial immunodiffusion. Protein concentrations in precolostrum were equal to or greater than those found in colostrum obtained during the first 12-48 hours following delivery. Secretion of precolostrum is common, occurs early in the antenatal period and may often be of considerable volume. The anti-microbial proteins contained within this milk can be preserved intact by freezing. This represents an untapped pool of bacteriostatic proteins with specific activity against neonatal pathogens. We suggest that a potential protective effect against serious infection may be obtained by administering precolostrum to "at risk" infants during the first few days of life.     

GLUCOSE-GALACTOSE MALABSORPTION A Study with Biopsy of the Small Intestinal Mucosa

Intestinal biopsies from two patients with glucose-galactose malabsorption have been subjected to biochemical and histological studies and the absorption of glucose, mannitol and sodium has been studied with a perfusion technique in one of the patients. No accumulation of glucose against a concentration gradient was found during in vitro incubation of mucosa from the patients. Phloridzin did not further decrease the slow glucose uptake by the mucosa. L-Alanine uptake was normal. Intestinal mucosa from both parents of a patient with glucose-galactose malabsorption accumulated glucose. In mucosa from patients with glucose-galactose malabsorption disaccharidase activities were normal, sodium activation of invertase could be demonstrated and ouabain-inhibited (pump-) ATP-ase activity was present. The histology was essentially normal both by light microscopy and electron microscopy. The per fusion study in vivo demonstrated not only a very slow absorption of glucose but also leakage of glucose into the lumen. Sodium absorption was not impaired. From the data obtained it is suggested that this inborn error of metabolism is localized at the level of the glucose-galactose membrane carrier of the small intestinal mucosa.     

Anal‐transitional and bronchial metaplasia within the ileostomy of a small bowel allograft: A case report

Talmon GA, Wisecarver JL. Anal‐transitional and bronchial metaplasia within the ileostomy of a small bowel allograft: A case report.
Pediatr Transplantation 2011: 15:E34–E37. © 2009 John Wiley & Sons A/S. Abstract: Epithelial metaplasia is a phenomenon where a native mucosal cell type is replaced by another due to altered stem cell differentiation, usually as the result of long‐standing injury or inflammation. It is common in ileostomies performed with native small bowels for both neoplastic and inflammatory conditions. We present a case of anal‐transitional and bronchial epithelial metaplasia that occurred near the ileostomy of a small bowel allograft in a seven‐month‐old male transplanted for short bowel syndrome related to gastroschisis. The anal‐transitional metaplastic mucosa had an immunophenotype (CK7‐ and p63‐positive) similar to that of normal mucosa at the anal transition zone and a DNA STR profile consistent with the donor. To our knowledge, this represents the first report of this type of metaplasia occurring in ileostomies in the English literature. It is important for clinicians and pathologists to recognize these benign mucosal alterations to avoid diagnostic confusion.     

A Case of Multiple Pterygium Syndrome Associated with Chronic Idiopathic Intestinal Pseudo-Obstruction Syndrome

Multiple pterygium syndrome (MPS) is a rare condition characterized by pterygia of the neck, finger, antecubital, popliteal, and intercrural areas. The present case study describes a male patient afflicted with this condition. This case has two unique features 1) the patient exhibited both mental and physical retardation at 2 years of age, a rare symptom of this disorder, and 2) the patient also had chronic idiopathic intestinal pseudo-obstruction syndrome (CIIPS). Although the pathogenesis of both MPS and CIIPS is unclear, some features of the present case suggest a possible pathogenic mechanism.     

EFFECT OF SOMATOSTATIN INFUSION ON INTERMEDIARY METABOLISM AND ENTERO-INSULAR HORMONE RELEASE IN INFANTS WITH HYPERINSULINAEMIC HYPOGLYCAEMIA

Abstract. Aynsley-Green, A., Barnes, N. D., Adrian, T. E., Kingston, J., Boyes, S., and Bloom, S. R. (University Department of Paediatrics, John Radcliffe Hospital, Oxford, Department of Paediatrics, New Addenbrookes Hospital, Cambridge, and Department of Medicine, Hammersmith Hospital, London, England). Effect of somatostatin infusion on intermediary metabolism and enteroinsular hormone release in infants with hyperinsulinaemic hypoglycaemia. Acta Paediatr Scand, 70: 889, 1981.-The hypoglycaemia of infantile hyper-insulinism is often exceedingly difficult to control. The use of somatostatin has been advocated recently in such infants because of its effect on inhibiting insulin release, but nothing is known of the wider effects of this potent hormone in the young child. Two infants presenting at 9 weeks and 5 days of age with severe hyperinsulinaemic hypoglycaemia were studied during an infusion of somatostatin. In both infants normoglycaemia was restored with suppression of insulin secretion. An increase in blood ketone bodies occurred, but no change was seen in blood pyruvate, lactate or alanine concentrations. The plasma concentrations of glucagon, Cortisol, growth hormone, motilin.-pancreatic polypeptide, gastric inhibitory polypeptide, neurotensin, gastrin and vasoactive intestinal peptide decreased markedly during the somatostatin infusion. No consistent change occurred in plasma enteroglucagon or secretin values. We conclude that somatostatin effectively suppresses abnormal insulin secretion in infants, but it has profound effects on the release of nine other hormones. Further studies are needed to define the consequences of suppressing the release of these hormones before somatostatin can be used routinely in the management of infantile hyperinsulinism.     

INCREASED EXCRETION OF A BRAIN DEPRESSOR AMINE IN INFANTILE COELIAC DISEASE AND IN HEALTHY INFANTS ON A HIGH PROTEIN MILK DIET

Abstract. Lindblad, B. S. and Rafter, J. J. (Department of Paediatrics at St. Göran's Children's Hospital and the Department of Medical Nutrition, Karolinska Institutet, Stockholm, Sweden). Increased excretion of a brain depressor amine in infantile coeliac disease and in healthy infants on a high protein milk diet. Acta Paediatr Scand, 69: 643, 1980.—Urinary excretion of piperidine, a heterocyclic pressor amine of gut bacterial origin and nicotine-like activity in the brain, has been estimated by a gas chromatography method in healthy men and women, in normal breast-fed and formula-fed infants and in infants with untreated coeliac disease. The excretion of piperidine cannot usually be detected during the first week of life. The amount present in urine increases upon weaning with higher excretion in formula-fed than in breast-fed infants at four to six months of age. When premature infants fed on human milk are weaned, the urinary content of piperidine rises from undetectable amounts to normal for age. The high content present in untreated coeliac disease may be responsible for the initial mental depression commonly seen in this disease and suggests that piperidine is one of the "auto-intoxicating" substances arising from the bacterial decomposition of protein postulated by Metchnikoff in 1903 but hitherto unidentified.