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Biomedical subjects

T Lamireau

Publications and source records attributed to T Lamireau.

At least 37 records · Page 2Linked to original sources

Expression of collagens type I and IV, osteonectin and transforming growth factor beta-1 (TGFbeta1) in biliary atresia and paucity of intrahepatic bile ducts during infancy.

BACKGROUND/AIMS: Biliary atresia and paucity of intrahepatic bile ducts are the main causes of neonatal cholestasis leading to hepatic fibrosis. Fibrotic evolution is slow in paucity of bile ducts as compared to the rapid progression to biliary cirrhosis in biliary atresia when cholestasis persists despite hepatoportoenterostomy. Our aim was to compare the expression of collagens type I and IV, alpha-smooth muscle actin, osteonectin and transforming growth factor beta1 in biliary atresia and paucity of bile ducts. METHODS: Liver biopsies were obtained in 12 children with biliary atresia and in five with paucity of bile ducts. Collagens type I and IV, alpha-smooth muscle actin were detected with immunostaining. Collagens type I and IV, osteonectin and transforming growth factor beta1 mRNAs were detected by in situ hybridization. RESULTS: Expression of mRNA and proteins was roughly parallel. In ductular proliferation areas of biliary atresia: (1) the expression of collagens type I and IV and osteonectin was increased, and was localized to periductular myofibroblasts; (2) transforming growth factor beta1 was expressed around biliary ductules, probably in inflammatory cells, and also in biliary cells. Osteonectin expression was also increased in the lobules. In paucity of bile ducts, there was no overexpression of collagens type I and IV and transforming growth factor beta1, except in the only child with marked fibrosis. However, osteonectin expression was enhanced at the periphery of the lobules, even when fibrosis was mild or absent. CONCLUSIONS: These findings suggest that in biliary atresia ductular proliferation areas are the site of a marked production of extracellular matrix proteins in periductular myofibroblasts, probably secondary to transforming growth factor beta1 production by inflammatory cells and by biliary cells. The weak expression of transforming growth factor beta1 could explain the slow progression of fibrosis in paucity of bile ducts.

Actins↗

[Acute severe asthma in children. Epidemiological, physiopathological and clinical aspects].

Therapy for asthma is being prescribed more frequently. However, asthma mortality remains high in many countries. Fatal outcome is not always related to inadequate follow-up. In this article we report that children with mild to moderate symptoms may present a fatal attack. Many factors are responsible for such an outcome: asphyxia +3 due to ventilation/perfusion mismatch and/or bronchospasm, cardiac failure, cardiac arrhythmia, intrinsic positive expiratory pressure, or metabolic disturbances (hypokalemia, for example). Such problems can occur in predisposed patients: it has been shown that the chemosensitivity to hypoxia and the perception of dyspnoea are altered in certain patients with near-fatal asthma. It is very important to identify children at risk of severe asthma and to organize care so as to optimize the management of such children.

Acute Disease↗

Abdominal lymphatic dysplasia and 22q11 microdeletion.

We report the case of a child with 22q11 microdeletion who presented with abdominal lymphatic dysplasia resulting in exsudative enteropathy. This primitive and localized lymphatic malformation is consistent with the vascular theory in the velocardiofacial syndrome.

Chromosome Deletion↗

Acute urticaria in infancy and early childhood: a prospective study.

OBJECTIVES: To establish the clinical, etiological, and prognostic features of acute urticaria in infancy and early childhood and to define its optimal management. DESIGN: Prospective study. The inception cohort was collected from April 1, 1992, through March 31, 1994. After initial evaluation, the course of the disease was assessed at 2 months and after 1 to 2 years. SETTING: Emergency department of a regional teaching pediatric hospital (referral center), which is also the only pediatric hospital for the general community in the city (population, 600,000 inhabitants). PATIENTS: Fifty-seven consecutive infants, aged 1 to 36 months, hospitalized with a final diagnosis of acute urticaria. Follow-up at 1 to 2 years was available in 40 of 57 patients. INTERVENTION: Oral antihistamines (dexchlorpheniramine maleate, terfenadine, or hydroxyzine hydrochloride) for 2 weeks. MAIN OUTCOME MEASURES: Recurrence and chronicity. RESULTS: Annular or geographic papules and plaques with hemorrhagic lesions were seen in 28 patients (49% of cases) and angioedema in 34 patients (60% of cases). An underlying cause was suspected or identified in 52 patients (91% of cases). Infection, either associated or not with drug intake, was the cause in 46 patients (81%) and foods were the cause in 6 (11%). Parasitic infestations were noncontributory. Hemorrhagic lesions and association with articular symptoms were statistically more frequent in urticaria caused by infections. Atopy in the patient or family was associated in 33 patients (58% of cases), and particularly atopic dermatitis was associated with urticaria caused by food. At 1- to 2-year follow-up, 12 (30%) of 40 patients surveyed had chronic or recurrent urticaria. CONCLUSIONS: Causative factors in urticaria are dominated by benign viral illnesses, often associated with antibiotic drug therapy. In most patients, laboratory investigations are not required. Twenty percent to 30% of cases evolve into chronic or recurrent disease.

Acute Disease↗

Rhabdomyolysis in European viper bite.

The case of a child who presented with severe rhabdomyolysis associated with renal failure after a viper bite is reported. Rhabdomyolysis is a serious complication resulting from systemic envenomation and is uncommon after viper bites in Europe. It may be due to oedema, myotoxic agents and haemorrhagic factors and may be responsible for two types of potentially fatal complications, i.e. acute renal failure and hyperkalaemia. The present case highlights the need to investigate routinely for rhabdomyolysis after viper bites. Antivenom therapy is recommended as soon as signs of envenomation are present, without waiting for the onset of complications.

Animals↗

Oxygen saturation during esophagogastroduodenoscopy in children: general anesthesia versus intravenous sedation.

BACKGROUND: Hypoxia may occur in children undergoing upper digestive endoscopy under sedation. The purpose of this study was to compare the occurrence of desaturation during intravenous sedation with that which occurs during general anesthesia. METHODS: Thirty-six patients between 3 months and 6 years old underwent a diagnostic esophagogastroduodenoscopy under sedation (n = 18) or general anesthesia (n = 18). Oxygen pulse oximetry, heart rate, and mean arterial pressure were monitored throughout the procedure. At the end of the procedure, the operator gave the value of the endoscopy satisfaction score on a scale of I (very good conditions) to IV (impossible procedure). RESULTS: The minimum oxygen pulse oximetry value was significantly lower in the sedation group compared with that in the general anesthesia group (89 +/- 5 vs. 97 +/- 1; p < 0.001). In the general anesthesia group, the oxygen pulse oximetry level declined to less than 95% in only one child; but in the sedation group, it declined to less than 95% in 16 patients (5.5% vs. 89%). Nine patients had a profound desaturation in sedation group (oxygen pulse oximetry < 90%); no patients in the general anesthesia group had desaturation (50% vs. 0%). In the general anesthesia group, heart rate and mean arterial pressure remained stable during the whole procedure, whereas in the sedation group, heart rate and mean arterial pressure increased significantly during the procedure. The endoscopy satisfaction score was I in all 18 patients in the general anesthesia group, whereas in the sedation group, it was I in only 2 patients, II in 8 patients, and III in 10 patients. CONCLUSIONS: These results confirm that hypoxia during upper digestive endoscopy in patients under sedation is a frequent occurrence in children. When compared with sedation, general anesthesia is a safer technique that prevents hypoxia and allows the gastroenterologist to perform the endoscopy under better conditions.

Anesthesia, General↗

Oesophageal atresia, VACTERL association: Fanconi's anaemia related spectrum of anomalies.

Oesophageal atresia usually occurs without any genetic background. Three cases associated with Fanconi's anaemia are reported. One neonate had growth retardation and numerous malformations including oesophageal atresia and four other components of the VACTERL association. In the two others, oesophageal atresia was isolated. In patients with such malformations an early diagnosis of Fanconi's anaemia may have important genetic and therapeutic implications.

Esophageal Atresia↗

[Severity of ingestion of caustic substance in children].

BACKGROUND: Caustic ingestion is frequent in children, sometimes leading to esophageal stricture. PATIENTS AND METHODS: Between 1988 and 1994, esogastroscopy was performed in 65 children after caustic ingestion. The children were classified in three groups: no lesion (group A), minimal lesions (group B) and severe lesions (group C). Nature of the caustic substance, clinical signs and evolution were compared in the three groups. RESULTS: Median age was 2 years for the 65 children (24 girls, 41 boys). Ingestion occurred at home (94%) during meal periods. Substances were dishwater detergents (n = 14), oven cleaner (n = 10), bleach (n = 9), washing powder (n = 4), others (n = 20), more often in a liquid form (n = 37) than solid (n = 28). Children had no symptoms (57%), presented emesis (n = 20) or abdominal pain (n = 10) not correlated to endoscopic findings, and hematemesis (n = 3) or respiratory distress (n = 4), both symptoms seen only in group C. Buccal lesions (41%) were not correlated to endoscopic findings. After endoscopy, 28 children (43%) were classified into group A and 20 children (31%) in group B. Among the 17 children (26%) of the group C, eight developed an esophageal stricture: seven long strictures requiring replacement of the esophagus, one short stricture requiring repeated dilations. CONCLUSION: Esophageal stricture is still a severe complication after caustic ingestion. These data stress the interest of controlled studies to confirm the preventive role of high dose corticosteroids, and the importance of the prevention of accidental caustic ingestions in children.

Accidents, Home↗

[Increasing frequency and diagnostic difficulties in intestinal stenosis after necrotizing enterocolitis].

BACKGROUND: Stenosis after necrotizing enterocolitis (NEC) has increased from 15 to 57% over the last 10 years in our unit. The aim of this study is to point out the difficulty of diagnosis and treatment, and search for factors explaining this increase. PATIENTS AND METHODS: From 1986 to 1991, 42 newborns had NEC, followed by intestinal strictures in 19 of them (57% of the 33 survivors). Data from these 19 patients were compared with those of the 14 without intestinal strictures. The 33 survivors were also compared with those of an earlier study including 25 NEC seen from 1979 to 1986. RESULTS: After medical treatment (n = 12), intestinal stenosis led to occlusion after three weeks, was located to both small and large intestine and was short and tight. After surgical treatment (n = 7), stenosis was shown by opacification before digestive anastomosis (n = 5) or revealed by occlusion (n = 2); it stayed on the colon, was long or multiple, requiring extensive resections. No difference could be found between data from patients with or without stenosis. Although newborns were actually more premature, the risk of stenosis was more frequent when newborns of same gestational ages and/or weights were compared. CONCLUSIONS: Intestinal stenosis is a frequent complication after NEC; its diagnosis is often difficult and requires extensive digestive resections. No clinical or therapeutic factor could be found to explain the actual increase in frequency.

Birth Weight↗

Decreased activity of intestinal and urinary intrinsic factor receptor in Gräsbeck-Imerslund disease [corrected].

BACKGROUND/AIMS: The pathogenesis of inherited intestinal cobalamin malabsorption (Gräsbeck-Imerslund disease) remains unknown. The authors studied whether the disease corresponds to a defective expression and/or function of the intrinsic factor-cobalamin receptor in the ileum. METHODS: Intrinsic factor-cobalamin receptor activity was measured using radioisotope assay and gel-filtration exclusion chromatography in ileal biopsy specimens and urine concentrates from 4 patients with Gräsbeck-Imerslund disease and 5 controls. RESULTS: Receptor activity was 164 +/- 13 fmol/mg of protein in control biopsy specimens and < 2.6 fmol/mg protein in specimens from patients. The association constant was estimated to be 3.8 +/- 0.4 (nmol/L)-1 in controls. A dramatic decrease in receptor activity was also observed in urine concentrate from patients with an association constant of 1.9 and 3.3 (nmol/L)-1. Isoelectrofocusing of the cross-linked intrinsic factor-cobalamin receptor complex showed an isoelectric point at 4.8 in a patient as well as in control samples. CONCLUSIONS: It is concluded that Gräsbeck-Imerslund disease is related to decreased intrinsic factor-receptor activity in intestinal mucosa; the receptor assay in urine can be helpful for diagnosis.

Adult↗

[Helicobacter pylori gastritis in children].

BACKGROUND: The role of Helicobacter pylori in chronic gastric and duodenal ulcer disease has now been proven in adults as well as in children. MATERIAL AND METHODS: H pylori has been looked for during endoscopy in 130 children suffering from recurrent abdominal pain (78 patients) or other symptoms (52 patients). Biopsy samples were taken from the antrum and the fundus, and were examined for histology and bacteriology (Gram staining, urease test, culture). RESULTS: H pylori was found in 30 cases (H pylori+ group, medium age: 12 years) and was absent in the 100 other cases (H pylori- group, medium age: 6 years). H pylori was present in 27% of children suffering from recurrent abdominal pain and 17% of children with other symptoms. Epigastric location of the pain and vomiting were equally frequent in both groups. Endoscopic gastritis, often nodular, was noted in 60% of the cases in the H pylori+ group and in 46% in the H pylori- group (NS). Chronic gastritis was noted in 90% of the cases in the H pylori+ group, and in 32% of the cases in the H pylori- group (p < 0.01). Culture was positive in 27 out of 28 cases (97%) in the H pylori+ group, including 3 cases with negative histologic examination. Twenty-two children in the H pylori+ group were treated with amoxicillin and metronidazole. Endoscopy performed about 2 months later showed eradication of H pylori in 63% of the cases, associated with disappearance of histologic lesions in 6 out of 14 cases and of symptoms in 5 out of 7 cases. CONCLUSIONS: H pylori is associated with some pediatric cases of antral gastritis. Further studies are needed to determine the influence of its eradication on symptoms and the interest to search this pathology (by serology) in children suffering from chronic abdominal pain.

Adolescent↗

[Antigliadin, antireticulin, antiendomysium antibodies: value in the diagnosis and follow-up of celiac disease in children].

In recent years, the clinical spectrum of coeliac disease has changed and forms with mild aspecific symptoms are today frequent. Therefore many infants are submitted to jejunal biopsy in order to exclude coeliac disease or to allow an early diagnosis. This has led to a search for a simple and reliable diagnostic test of coeliac disease in order to limit the use of jejunal biopsy. Recent data suggest that the study of serum antigliadin, antireticulin and antiendomysium antibodies may possibly play the role. In this paper the working group on coeliac disease of the Groupe Francophone de Gastroentérologie et Nutrition Pédiatriques expresses its view on the place of the dosages of these antibodies in the diagnosis and follow up procedures of coeliac disease in infants and children. At the present time, although it allows a simplification of the procedures, these dosages are presently not sufficiently reliable to serve as a substitute of jejunal biopsy.

Autoantibodies↗

Fatal neonatal liver failure and mitochondrial cytopathy (oxidative phosphorylation deficiency): a light and electron microscopic study of the liver.

Mitochondrial cytopathies are multisystemic disorders of extremely variable expression due to a deficiency in oxidative phosphorylation. Cases have recently been reported in which fatal liver failure with neonatal onset was the major clinical and biochemical syndrome. In this series we reviewed the liver histology of 10 such patients who died in the first weeks of life (from 3 days to 6 mo). In six cases the diagnosis was confirmed by study of the mitochondrial respiratory chain in the muscle, liver or both; in the other four, appropriate tests were not available for diagnosis but symptoms were identical and all other diagnoses were ruled out. In all 10 cases we noted significant steatosis, mostly microvesicular; widespread hepatocytic, canalicular and bile duct cholestasis with bile thrombi and cholangiolar proliferation; and different degrees of hepatosiderosis and glycogen depletion. Fibrosis took varying forms: perisinusoidal, periportal with the formation of septa, even precirrhosis. In the two cases of infants who died, one at 5 and one 6 mo, micronodular cirrhosis was also present. Mitochondria, either densely or loosely packed, were abnormal-pleiomorphic with few or no cristae and a granular fluffy matrix. Dense, large granules were seen in two cases. The association of neonatal liver failure and hyperlactacidemia should lead to immediate examination of the respiratory chain. The expression of this mitochondrial cytopathy can be lethal, associated with severe liver damage due to the deficiency in oxidative phosphorylation.

Female↗

[Apple-peel jejunal atresia. Nutritional management].

Apple-peel jejunal atresia is a rare digestive tract congenital defect often accompanied with a short gut. Two cases managed by a three-stage medical and surgical approach are reported. A protein hydrolysate-jejunal secretion mixture was drip-fed through the distal ileostomy to stimulate trophicity of the distal gut.

Enteral Nutrition↗