[Intolerance to milk proteins and to soya].
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Biomedical subjects
Publications and source records attributed to B Santini.
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A group of 45 children affected with Coeliac Disease (CD) was typed for HLA-A, B, C, D, and DR specificities. The most significant associations were found with two alleles of the D series, with both cellular and serological typing. It is suggested that the susceptibility to CD is determined by two different genes within the HLA region, the first in common with organ-specific autoimmune diseases and associated with DW3, the second possibly specific for CD and associated with Dw7.
Of 80 children with proved coeliac disease, 2 presented with an associated disease of the distal portion of the large intestine. In one child the family history and the extension, localisation, and characteristics of intestinal lesions made us suspect ulcerative colitis; in the other we made a diagnosis of milk-induced colitis.
The incidence of connective tissue antibodies was assessed in 3,000 adults and in 388 pediatric patients with gastroenterological disorders. Following a previous classification, connective tissue antibodies were distinguished in 5 different types, R1 and R2 reacting with reticulin components, KC with Kupffer cells, AC and Rs with intra- and extracellular mesenchymal antigens. R1 predominated in children and was observed only in patients with malabsorption; the great majority of them had celiac disease and an abnormal small bowel mucosa. Ac was rarely seen in children and occurred in several unrelated gastrointestinal diseases of the adult; its incidence, however, was significantly higher in disorders with idiopathic and secondary malabsorption. R2, KC and Rs were seen only occasionally in unrelated gastroenterological disorders without malabsorption. R1, therefore, seems to be strongly suggestive of a malabsorption syndrome with abnormal small bowel mucosa and AC is a nonspecific reaction occuring predominantly in primary and secondary malabsorption; the other connective tissue antibodies have no clinical significance in gastroenterological disorders.
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Intestinal function in children with very short bowel syndrome and related intestinal failure may improve after isolated liver transplantation. An infant with an ultrashort gut, ileo-cecal valve, and whole colon received total parenteral nutrition from the first days of life. Enteral feeding failed because of the progressive dilatation of the jejunal portion and motility disorders. He developed early severe cholestatic liver disease (aspartate transferase 186, alanine transferase 103 U/L, serum bilirubin 8.4 mg/dL) and subsequent liver failure. At 8 months of age, he benefited from isolated liver transplantation (left segment graft from living donor). His early posttransplant evolution was characterized by recovery of oral alimentation, improvement of digestive and absorption functions, but he did not achieve TPN-independence. At 20 months, 50% to 60% of his energy needs were covered by parenteral nutrition and he has satisfactory growth indices (3rd percentile for weight and height), reduced stool volume, and frequency. Isolated liver transplantation allowed, in this particular case, time for further intestinal adaptation thereby avoiding the need for intestinal transplantation early in life.
BACKGROUND: Pancreatic exocrine insufficiency is a common condition in patients with cystic fibrosis. Large amounts of pancreatic enzyme supplements are required to reduce malabsorption but patient compliance is not always optimal. AIMS: To compare patients' preference and the efficacy of two enteric coated microsphere preparations in patients with cystic fibrosis. PATIENTS: Patients with pancreatic exocrine insufficiency due to cystic fibrosis. METHODS: Patients were assigned to the crossover treatment with Creon or Pancrease for 1 week and then to the alternative treatment. Patients had to follow a fixed diet (at least 2 g fat/kg) and had to assume 1000 units lipase/g fat. The evaluation parameters were: patients' preference, acceptance of therapy, stool fat excretion, stool weight, gastrointestinal symptoms, and tolerance. RESULTS AND CONCLUSIONS: Of the 33/60 patients who expressed a preference for one of the two treatments, 30 preferred Creon while only 3 patients preferred Pancrease (p<0.001). No difference between the two treatments was observed regarding stool characteristics, gastrointestinal symptoms and tolerance. The mean number of capsules taken daily was reduced by 35% with Creon. The results of this study showed a preference in favour of Creon probably due to the reduction of daily capsule intake of 35%, supporting digestion as well as Pancrease.
The purpose of this report is to present an overview on vitamin E distribution, requirements, absorption and biochemical and nutritional aspects. A continuous interest in biochemical functions is recently developed and vitamin E certainly plays an important role throughout the body. The best known of its effects and still actively considered in recent years is the role as an important biological antioxidant. The red blood cell is an ideal model for studying the antioxidant role of vitamin E in cell membranes. Nutritional deprivation is a rare occurrence in developed countries. In prematurely delivered newborns the deficiency is due to marginal stores and to transient malabsorption but it can also be iatrogenic. In infants and adults vitamin E deficiency does occur in syndromes characterized by increased consumption or reduced absorption. Various gastrointestinal disorders induce, with steatorrhoea, marked alteration of vitamin E levels. Cystic fibrosis (CF), the commonest cause of pancreatic insufficiency during the first decades of life, is of particular interest. The fat malabsorption, often severe, may not well respond to pancreatic therapy and the hepatobiliary disease, increased in frequency with improved survival, induce a further reduction in intestinal bile salt concentration. Several manifestations have been attributed to vitamin E deficiency in CF and, although overt neurological complications seem to be relatively uncommon, it is recommended to maintain an adequate supplementation.
The irritable bowel syndrome (IBS) is the most common chronic functional gastroenterological disorder both in adults and in children. In this study we evaluate the different aspects of this syndrome, comparing our observations on 332 children with other studies. Epidemiological data (frequency, sex, age) are examined so as the family histories of gastroenterological disorders. We take in account several pathogenic hypotheses, especially with reference to the alterations of gastrointestinal motility, which could be caused by several factors (psychological, prostaglandins, bile acids, etc.). The clinical picture is very variable, variations depending on the age of children and on the time of onset of IBS. The colic of neonate, caused by retention of air, is the main symptom in the first months of life, followed by chronic diarrhoea, also defined as toddler's diarrhoea, sometime alternating with constipation. In later childhood, recurrent abdominal pain represents a common complaint, in association with diarrhoea or constipation. The principal steps for a proper diagnosis so as the main differential diagnosis are defined. We explain the most important features of management (reassuring parents, free diet), excluding prescription of drugs, that produce only a transitory and symptomatic relief, so as elimination diets, that cause only a failure to thrive without any improvement of symptoms.
The replacing therapy in the treatment of pancreatic insufficiency is considered the first and most important step. At present, the choice is between highly concentrate or enteric-coated enzymatic compounds. Their administration before or during each meal has to be the one able to reduce fecal fat and nitrogen, allowing a satisfactory growth. A long term therapy even at low dosage with antacids or cimetidine must be considered with caution and evaluated in each case. In CF children the nutritional care has now been emphasized for the possible relationship between malnutrition and lung disease; the caloric intake must be higher than normal. The limitation of fat intake is not so important any more when the enzymes replacement is adequate. The vitamins requirement is usually satisfied doubling the normal daily requirement. An increase of vitamins and/or minerals must be considered when clinical or laboratory signs of deficiency are found.
Little attention has been given to the gastric secretion of pepsin in children. The purpose of this paper is to define the behaviour of peptic concentration (UI/ml) and Basal (BPO) and Stimulated (SPO) Peptic Outputs in children with normal and altered gastric acid secretion. 54 children, aged 15 months to 12 years, were studied: 20 of them were normal, 20 had gastric hypersecretion and 14 were hyposecretors. In the normal subjects the BPO is inversely correlated with age, while the SPO increases with the body weight, but this increment diminishes after the age of 6 years. From these data one can assume that the gastric principal cells reach their functional maturity around the age of 6 years. In the hypersecretors peptic and acid secretions are not correlated in basal conditions, while after pentagrastrin stimulation peptic secretion is greater than in normal subjects but not as the acid one. The SPO is correlated with body weight and height. The different behaviour of hydrochloric acid and pepsin is probably due to the use of pentagrastrin, which is a specific stimulant of oxyntic but not of principal cells. In the hyposecretors both BPO and SPO are lower than in normal children and no correlation was found with age, body weight and acid secretion.