[Transplantation of the small intestine].
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Biomedical subjects
Publications and source records attributed to C Ricour.
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Amongst the uncommon forms of congenital severe colitis, we wish to draw attention to a peculiar and probably previously never described condition that we propose calling provisionally, epithelio-exfoliative colitis. This condition appears to be characterized by the following features: its early beginning within the first weeks of life; the smooth, glossy appearance of the mucosa, without ulcerations visible to the naked eye; the prevalent degenerative changes of the epithelial cells which become vacuolated, break away prematurely from the basement membrane and finally exfoliate within the glandular lumens; the distension and rupture of the glands, the mucous contents of which intrude into the lamina propria and induce a localized, mild and non suppurative inflammatory reaction; accessory reactive traits: intense mucus production actively regenerating epithelium (high mitotic activity, syncytial cells) and increase of the cholinergic fibers within the lamina propria. Although patchily distributed, these lesions involve the colon exclusively. The cause of epithelio-exfoliative colitis is unknown. However, the ultrastructural studies and immunocytochemical investigations using anti-collagen IV, antilaminin, anti-fibronectin antibodies disclose in some glands localized thinning and rupture of the basement membrane. These data suggest a primary disorder within the molecular arrangement of either the basement membrane itself or the proteins which anchor the glandular cells to the basement membrane.
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From 1960 to 1984, 27 cases of total colonic aganglionosis were treated at the Sick Children's Hospital in Paris; in 19 cases there was ileal involvement, 16 of them extending more than 15 cm above the ileocecal valve. Five had a family history of Hirschsprung's disease. Nine infants died without having had definitive surgery, because of delayed diagnosis, or intractable malabsorption in extensive ileal aganglionosis. Two cases were diagnosed only at the ages of 6 and 13 years. Eleven children had Martin's modification of the Duhamel operation, the oldest of these being now 13 years old; and one girl aged 13 underwent a Swenson operation with ileoanal anastomosis, and one child has had a Kimura procedure. Four infants still have a diverting ileostomy. One out of the 14 operated children died 3 years after operation with fulminating enterocolitis. Late surgical nutritional results are analyzed with regard to the length of the side-to-side ileocolorectal anastomosis, and to the size of the ileorectal anastomosis, on which adequate pouch emptying depends. The essential problem in total colonic aganglionosis is not the surgical management of the condition, but rather its prompt diagnosis and the handling of the neonatal intestinal obstruction.
We report the beneficial effects of calcium infusions in a child with hereditary resistance to 1,25(OH)2D and alopecia. This patient after transient responsiveness to vitamin D derivatives became unresponsive to all therapy despite serum 1,25(OH)2D concentrations maintained at levels approximately 100-fold normal. A 7-mo trial with calcium infusions led to correction of biochemical abnormalities and healing of rickets. Bone biopsies (n = 3) showed a normal mineralization and the disappearance of the osteomalacia. Cultures of bone-derived cells demonstrated a lack of activation of 25-hydroxyvitamin D 24-hydroxylase and osteocalcin synthesis by 1,25(OH)2D3 (10(-9) and 10(-6) M). These results demonstrate that even in the absence of a normal 1,25(OH)2D3 receptor-effector system in bone cells, normal mineralization can be achieved in humans if adequate serum calcium and phosphorus concentrations are maintained; and calcium infusions may be an efficient alternative for the management of patients with this condition who are unresponsive to large doses of vitamin D derivatives.
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The relationship between physical activity, nutritional requirements and protein-energy metabolism is well-established. However, and contrary to bedside practice at home, non-ambulatory techniques of enteral or parenteral nutrition are often used in hospitalized patients, even those who are able to move about. The purpose of the present study is: (a) to review the relationship, as we know it at present, between physical activity and protein metabolism in both normal subjects and patients with denutrition, and (b) to demonstrate that using from the start, or as early as possible, ambulatory techniques of enteral or parenteral nutrition does represent, both physiologically and psychologically, a decisive step forward in the care of hospital patients.
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Heart rate (HR) was studied separately for three behavioral states (waking, paradoxical sleep, quiet sleep) at four periods of the nycthemeron, in 8 infants continuously fed from birth. Continuously fed infants show no significant differences for each of the three behavioural states between the HR values of the four periods of the nycthemeron, while controls show a clear decrease during the night-time. Differences between term behavioral states exist both in continuously fed infants and in controls. Long term continuous nutrition does not allow for the establishment of the circadian modulation of HR.
The case reported concerns a child with chronic intestinal pseudo-obstruction (CIPO) whose digestive manifestations (intestinal adynamia and distension) were present from the age of 6 months and lasted, despite medical and surgical treatments until 4 years of age, when death occurred. The multiple samplings showed important inflammatory reactions centred on the muscular layers of the small intestine, together with degenerative lesions of the muscular fibres, progressively leading to fibrosis and atrophy of the intestinal wall with secondary and final impairment of the myenteric plexuses. The diagnosis of myositis of the small intestine is extremely rare. It is not part of the usual causes of intestinal adynamia and CIPO, which were reviewed. Hollow visceral myopathy and systemic sclerosis of the GI tract were more especially discussed. For lack of etiopathogenic convincing data and of similar observation in the literature, this case may be temporarily considered as an idiopathic myositis of the small intestine, a potentially new cause of CIPO.
Thirteen children aged 2 to 16 years have had a subtotal resection of small bowel, following a mid-gut volvulus in 10 cases. All children are still alive, and their growth was normal; 36 cumulative patient-years of parenteral nutrition and 11 years of constant rate enteral nutrition were performed. In 7 cases, where residual small bowel varied between 30 to 120 cm, termination of all artificial nutritional support was possible at a mean of 30 months after intestinal resection. On the other hand if resection was near total with less than 20 cm remaining, life long dependence on parenteral nutrition is unavoidable unless intestinal transplantation becomes feasible; with cyclic parental nutrition at home, their quality of life is near normal.
The modalities of growth were determined with anthropometric measurements in 7 children under total parenteral nutrition for more than 2 years. Their speed of height growth is normal and correlated with their increase in lean body mass. Their weight growth is normal, however with a relative excess in fat mass explained by its high energy cost; on the contrary, the cost of maintenance activity is reduced. In order to limit the energy cost and, overall the risk of an excessive load of lipid deposits, the authors emphasize the importance of regularly determining the lean body mass and the composition of weight gain to manage properly the intake in prolonged total parenteral feeding.
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In 8 children who were fed exclusively for an average of 30 months with parenteral nutrition containing 25% of the energetic intake as lipid emulsion (Intralipid) acute complications similar to those observed in the fat storage syndrome occurred. One child died from gastrointestinal bleeding. In the other cases, evolution was dramatically improved by corticosteroid therapy. An histiocytic hyperactivation induced by the artificial emulsion might be responsible for these complications. The authors emphasize the risks of long-term use of lipid emulsion and the preventive measures which should be taken in children under prolonged parenteral nutrition.
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