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Biomedical subjects

O Goulet

Publications and source records attributed to O Goulet.

At least 127 records · Page 7Linked to original sources
PubMed1991

Glucose versus glucose-fat mixture in the course of total parenteral nutrition: effects on substrate utilisation and energy metabolism in malnourished children.

Energy substrate utilisation was evaluated over 21 days in two groups of malnourished children on total parenteral nutrition (TPN). Non-protein energy was infused as glucose (Group A; n = 7) or as a glucose/fat (1:1 v/v) mixture (Group B; n = 10). Results indicated that: 1) net glucose oxidation was related to glucose intake; 2) glucose storage was elevated in group A; 3) net fat synthesis occurred earlier in group B together with constant net fat oxidation which was inversely related to glucose intake (r = -0.89, p < 0.001); 4) lipogenesis from glucose occurred only when glucose intake exceeded 19.3g/kg/d; 5) energy expenditure increased by 36% (group A) and 18% (group B) during renutrition; 6) 73% and 82% of the energy administered in excess of energy required was stored in group A and B respectively. Hence, glucose/fat infusion appears to be more energy-efficient than glucose-alone in TPN of malnourished children.

Journal Article↗
PubMed1991

Calcium metabolism in children during long-term total parenteral nutrition: the influence of calcium, phosphorus, and vitamin D intakes.

Hypercalciuria and bone disease are frequently associated with total parenteral nutrition (TPN) in children and adults. The aim of this study was to assess the influence of calcium, phosphorus, and vitamin D intakes on hypercalciuria. We observed seven children aged 4-13 years receiving home cyclic TPN for 4 consecutive years. Calcium and phosphorus intakes, constant during the 1st year, were reduced during the last 3 years to 50 and 30% of the initial intakes, and vitamin D was stopped during the 3rd and the 4th years. All children had hypercalciuria and one of them had acute painful osteopenia and nephrocalcinosis at the beginning of the study. Hypercalciuria was corrected and painful bone disease did not occur during the three following years, with TPN daily intakes of calcium, 0.35 mmol/kg, and phosphorus, 0.70 mmol/kg. Cessation of vitamin D administration during 48 months led to no further decrease in calciuria nor to the occurrence of clinical or biological signs of vitamin D deficiency. However, we hypothesize that excessive vitamin D intake may have facilitated the occurrence of the TPN-related bone disease in one patient and should be avoided. The possible role of parenteral aluminum loading is also discussed.

Adolescent↗
PubMed1991

[Severe Crohn's disease in children. Retrospective study of 38 cases].

Thirty-eight children presenting with severe Crohn's disease (CD) were studied retrospectively over a mean period of 6 years. The severity of CD was estimated according to an activity score. This was initially 66 +/- 19 reaching 100 in 16% of cases. Initial involvement included ileon and large intestine only (n = 23), large intestine (n = 11) and ileon only (n = 4), with upper GI tract or perineal involvement in 32 and 75% of cases respectively. The treatments consisted of corticosteroids (n = 34), azathioprine (n = 11), continuous enteral feeding (CEF) (n = 25), parenteral nutrition (PN) (n = 30). Mean recurrence rates per patient and per year were 0.6 in the groups including ileon and large intestine or the large intestine only and 0.3 in the ileal group. A surgical resection was performed in 20 cases with a mean rate of reoperation of 40% at 5 years. Reoperations were necessary only in the group involving the ileon and large intestine. Mean weight catch-up in 3 months and height growth speed were 6 kg and 1.7 to 3.1 cm/year under CED and/or PN. After a 6 year follow-up the activity index was decreased by 70% with a quality of life considered to be good in 60% of cases.

Child↗
PubMed1991

[Pearson's syndrome. Pancytopenia with exocrine pancreatic insufficiency: new mitochondrial disease in the first childhood].

Pearson's syndrome is a lethal disorder of a still unknown cause, responsible for pancytopenia and exocrine pancreatic dysfunction in the first months of life. Permanent hyperlactacidemia and increased mitochondrial and cytoplasmic oxidoreduction ratios were observed in 6 unrelated children presenting with this disease. This led to study the oxidative phosphorylation in lymphocytes and thus to relate Pearson's syndrome to an enzymatic disease of the respiratory chain (NADH oxidase activity deficiency). The study of the mitochondrial genome allowed identifying major changes in mitochondrial DNA in all patients and all studied tissues. Thus, Pearson's syndrome is the first mitochondriopathy with a non neuromuscular expression reported to date.

DNA, Mitochondrial↗
PubMed1990

Classification of intractable diarrhea in infancy using clinical and immunohistological criteria.

Several experimental studies have demonstrated that activated intestinal T cells can induce villous atrophy. This observation led us to examine the possible role of activated T cells in the pathogenesis of intestinal lesions in a group of 13 children with intractable diarrhea and villous atrophy of unknown origin. Immunohistochemical study showed signs of intestinal mononuclear cell activation in seven patients. These signs included a marked increase in the number of mucosal T cells, mainly TCR alpha beta+, the appearance of lamina propria interleukin-2-receptor-bearing cells, and an increased expression of HLA-DR antigens by enterocytes. No local cause of intestinal T-cell activation was found. However, four out of seven patients had extradigestive symptoms of autoimmunity, suggesting that the intestine might also be the site of an autoimmune reaction responsible for the epithelial lesions. In these patients, presence of crypt necrosis and colic extension of the lesions suggested poor prognosis. In contrast, in six other patients, no immunohistochemical evidence of mucosal T-cell activation was obtained. In the latter cases, analysis of clinical data favored the hypothesis of a primary inborn defect of enterocyte differentiation.

Atrophy↗
PubMed1990

Home parenteral nutrition in children: 8 years of experience with 112 patients.

It is essential that children on prolonged parenteral nutrition for anatomical or functional loss of small bowel should enjoy a quality of life which is as normal as possible. Their return home is a major factor in this. Over the past 8 years, 112 children were able to remain at home on cyclic parenteral nutrition. Forty-nine of them are no longer on home parenteral nutrition (HPN), 45 are still on HPN, and 18 have died. Growth and quality of life were good in most cases. Most of the complications were from infection, 1 septicaemia per 594 days on HPN. In the light of these results, HPN seems to be the best option for children requiring prolonged parenteral nutrition, although it can only be considered within the framework of a specialized centre, which ensures patient follow-up, and provides the logistical support required for this high-technology treatment.

Journal Article↗
PubMed1990

Catheter related sepsis during long-term parenteral nutrition in paediatric gastroenterology patients: a study of 185 consecutive central venous catheters.

We report the results of a prospective study of catheter-related sepsis (CRS) in 154 gastroenterology paediatric patients treated for medical (n = 102) or surgical (n = 52) disorders. Over 2 years, 185 central venous catheters (CVC) were placed, including 155 Broviac and 30 Jonathan Shaw CVC. CVC remained in place 13,331 days (6,539 days of cyclic parenteral nutrition), with a mean catheter life of 72 days. Forty-eight episodes of CRS occurred, most of them due to coagulase-negative staphylococci (n = 29) and Staphylococcus aureus (n = 17). CRS rate was 0.26, with an overall incidence of 1 infection per 278 days of parenteral nutrition (PN). Factors significantly associated with CRS were: age 1-5 years (p < 0.01), a medical indication for treatment (p < 0.001) and summer months of PN (p < 0.01). Interestingly, CRS occurred predominantly (65%) within the first 2 months following CVC placement and the risk of infection correlated with local haemorrhage at the time of insertion (p < 0.01). Appropriate systemic therapy, without removing the catheter, permitted resolution of 90.9% (40/44) of all CRS and 100% (28/28) of CRS due to coagulase-negative staphylococci.

Journal Article↗
PubMed1990

Aluminium loading in children receiving long-term parenteral nutrition.

Eight children on long-term, total parenteral nutrition (TPN) were found to have elevated aluminium (Al) levels in plasma (51 +/- 11 microg 1/1), urine (223 +/- 78 microg 24 h) and bone. The load of Al in TPN solution was 232 +/- 89 microg/day, and, among the different parenteral solution components high Al concentrations were found in amino-acids, calcium gluconate, potassium lactate, and trace elements, representing respectively 40%, 30%, 15%, and 10% of the total Al intakes. The authors conclude that children receiving long-term TPN have excessive Al intakes and are exposed to Al toxicity. The prevention of Al contamination requires careful control of the TPN components.

Journal Article↗
PubMed1990

An unknown source of vitamin K1 in patients on total parenteral nutrition.

We found a level of 20.8 +/- 3.4 mug/dl of vitamin K1 (VK1) in Intralipid 20% and initiated a study based on the follow-up of children receiving total parenteral nutrition without any other exogenous VK1 intake than from Intralipid 20% (1 g/kg/day). This lipid administration was sufficient to cover the needs during a study period of 4 to 29 weeks.

Journal Article↗
PubMed1990

Role of in vivo activated T cells in the mechanisms of villous atrophy in humans: study of allograft rejection.

Four children aged 6 months to 9 years received fully HLA-mismatched ABO identical small intestinal allografts. In order to monitor the rejection process and to study epithelial changes induced by intestinal T cells activated by an allogeneic reaction, iterative biopsies were performed through the ileal enterostomy and processed for histology and immunohistochemistry. Episodes of acute histological reaction were observed in all 4 patients between day 10 and 160. It was preceded by appearance of pericryptic CD3+CD4+ or CD8+ T cells of recipient origin, increasing numbers of which expressed CD25. Simultaneously, early epithelial changes were noted: increased HLA-DR expression by enterocytes and decreased mitotic rate as shown by decreased numbers of KI67+ cells in crypts. During acute histological rejection, massive infiltration of mucosa by CD25+CD3+ T-cells and activated macrophages (KIM6+CD25+), was associated with crypt necrosis and then, destruction of surface epithelium. Successful treatment of graft rejection episodes with antilymphocytic serum (2), anti-CD3 monoclonal antibody (2), anti-CD25 monoclonal antibody (1) resulted in a rapid decrease of CD3+ cells, a more progressive decrease of CD25+ and KIM6+ macrophages, reappearance of KI67+ cells in crypts followed after a variable delay by recovery of villous architecture. Chronic histological rejection was observed in 1 patient after 7 months. It was characterized by total villous atrophy, fibrosis, endarteritis, infiltration of lamina propria and epithelium by CD3+CD8+ cells, a small number of which CD25+, strong HLA-DR expression by crypt and surface epithelium, increased numbers of KI67 enterocytes. Altogether these data suggested that activated T cells can induce two types of villous atrophy.(ABSTRACT TRUNCATED AT 250 WORDS)

Atrophy↗
PubMed1990

[Severe digestive manifestation of rheumatoid purpura. Retrospective study of 19 cases in children].

Digestive and/or nutritional manifestations of Henoch-Schoenlein purpura (HSP) in childhood may be so severe as to require nutritional assistance. The study of 19 cases with this form of the disease allows to suggest a therapeutic protocol. When enteral nutrition cannot be used from the onset, parenteral nutrition is required. Associated steroid therapy may prove helpful for the regression of the digestive signs. The observed slowness of the weight catch-up, in spite of high protein-energy intakes, emphasizes the severity of catabolism in these severe forms of HSP.

Adolescent↗
PubMed1989

Organ transplantation in children.

Organ transplantations have opened a new field in medicine and particularly in pediatrics. The kidney was the first organ to be regularly transplanted and there are now more than 2000 children who have received a kidney graft. Cadaver kidneys or living-related donor (LRD) kidneys can be used since an adult kidney may be grafted in a young child. Cadaver graft survival exceeds 85% at 1 year in recent single centre reports and patient survival is above 95%, the results being even better with LRD. Some complications may be observed in the long term, such as bone osteonecrosis, hypertension or infections. Rehabilitation is generally remarkable and growth which remained abnormal in 1/3 of cases under conventional treatment seems to improve markedly with cyclosporin. In the future, the development of kidney transplantation could lead hopefully to a drastic decrease in the number of children on dialysis. Liver transplantation is now performed in children with biliary atresia and metabolic diseases. There is no age limit for grafting a liver, the only problem being to find a pediatric cadaver donor of similar size. It is also possible to use an adult liver after hepatectomy. There were 170 liver grafts recorded in children in Europe in December 1986, and the long-term survival thanks to cyclosporin is exceeding 80% in some units. Thanks to cyclosporin, programs of cardiac transplantation for children are rapidly developing. The main indications are gross-congenital cardiac malformations or severe functional abnormality not compatible with life. Newborns have been grafted with success. The only problem is also to find a suitable pediatric donor.(ABSTRACT TRUNCATED AT 250 WORDS)

Age Factors↗
PubMed1989

Primary intestinal myopathy, a cause of chronic idiopathic intestinal pseudoobstruction syndrome (CIPS): clinicopathological studies of seven cases in children.

Clinicopathological data on seven instances of primary intestinal myopathy in children are reported. The ages of the patients ranged from eleven months to thirteen years. A persistent intestinal obstruction was the main and constant clinical feature. An ineffective intestinal propulsion was documented on manometric studies. Various urological abnormalities were present in three cases. One patient died and six survive but are dependent on enteral and parenteral nutrition. The morphological findings consisted of degenerative changes involving the muscular layers of the intestinal wall. These changes varied from cytoplasmic vacuolation to definite atrophy and disappearance of the muscular fibers. An extensive interstitial fibrosis underlined these atrophic changes in the late stages of the disease. A familial history was identified in three cases, one consistent with an autosomal dominant transmission.

Child↗
PubMed1989

[Home parenteral nutrition in children. Evaluation after 8 years' activity with 88 patients].

In cases where an anatomic or functional amputation of the small bowel dictates that parenteral nutrition needs to be prolonged, it is essential to offer the child the best possible quality of life. In this regard, being in his home environment is an important component. Over a 8 year-period (1980-1988), 88 children went home with cyclic parenteral nutrition. In 34, parental nutrition was eventually discontinued: 40 are still parenteral nutrition-dependent and 14 died. In most cases, the growth and quality of life were satisfactory. Complications consisted essentially of infections, with 1 septicemia for 692 days of home parenteral nutrition (HPN). In the light of these results, HPN appears to be the best option for prolonged parenteral nutrition-dependent children. However, it cannot be set up without the help of a suitable center able to follow the children and to ensure the logistic support necessary for this highly technical and demanding type of care.

Adolescent↗