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Biomedical subjects

J M Limal

Publications and source records attributed to J M Limal.

At least 37 records · Page 2Linked to original sources

[A rare cause of severe diarrhea in children: pseudomembranous colitis].

BACKGROUND: Pseudomembranous colitis is a rare and serious complication of treatment by antibiotics. The case of a patient with a protracted pseudomembranous colitis followed by two relapses is reported. CASE REPORT: A 4 year-old boy was admitted after 18 days of profuse and feverish diarrhea. He had been given amoxycillin for 10 days, one and a half months previously. His temperature was 40 degrees C; he had abdominal pain and leucocytosis was 30,000/mm3. The situation rapidly improved with digestive rest and i.v. antibiotic therapy. Relapse of diarrhea together with bilious vomiting and acute abdominal pains required readmission three days after his discharge. Search for Clostridium difficile in stools remained negative. The diagnosis of pseudomembranous colitis was confirmed by sigmoidoscopy and intestinal biopsy. The patient was given parenteral nutrition for 3 weeks and vancomycin. The disease was complicated by anasarca related to severe protein-loosing enteropathy but evolution was finally favourable after a two month period. CONCLUSION: Pseudomembranous colitis remains a serious affection in childhood; its prognosis largely depends on the precocity of diagnosis and treatment.

Anti-Bacterial Agents↗

Combined analysis of islet cell antibodies which cross-react with mouse pancreas, antibodies to the M(r) 64,000 islet protein, and antibodies to glutamate decarboxylase in subjects at risk for IDDM.

With regard to progression to diabetes, ICA cross-reactive with mouse pancreas, antibodies to the M(r) 64,000 islet antigen (64K), antibodies immunotrapping brain GAD activity, and IAA were analysed in 53 ICA-positive first-degree relatives of IDDM patients and 18 ICA-positive schoolchildren without a family history of diabetes. Sera from 29 (55%) relatives did not bind to mouse pancreas, whereas 24 (45%) displayed cross-species reaction. ICA titres on human and mouse pancreas were weakly correlated in the overall population (p < 0.05) but more strongly (p < 0.01) in only those subjects who displayed antibodies on tissues from both species. GAD and 64K antibodies were detected in 31% and 35% of relatives. In schoolchildren, the frequencies of cross-species reactive ICA (22%), GAD antibodies (6%), 64K antibodies (22%), and IAA (6%), were lower (p < 0.05) than in relatives. A strong correlation (p < 0.0001) was observed between GAD and 64K antibodies. GAD or 64K antibodies were strongly correlated with ICA on human pancreas (p < 0.0001) but poorly with ICA on mouse pancreas (p = 0.05). After pre-incubation of sera with brain homogenate, ICA titres were unaffected on mouse pancreas but reduced on human pancreas. ICA-positive subjects who displayed neither cross-species reactive ICA nor GAD or 64K antibodies were more frequent (p < 0.05) among schoolchildren than relatives, whereas subjects who displayed all antibody specificities were more numerous (p < 0.04) in relatives. All relatives with ICA binding only to human pancreas, as well as all schoolchildren, permanently displayed an AIRG higher than the first control percentile and remained non-diabetic.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗

Study of final height in Turner's syndrome: ethnic and genetic influences.

In understanding Turner's syndrome, spontaneous adult height is a prerequisite for an accurate assessment of the therapeutic efficiency of growth hormone treatment. The heights described in the literature reveal significant differences (136-147 cm). Our collaborative study pooled results from 16 pediatric endocrinology centers and obtained a large number of spontaneous adult heights (n = 216). The selective criteria were: chronological age (CA) > 18 years, bone age (BA) > 16 years, typical karyotype, no treatment with growth hormone or anabolic steroids. Mean CA was 23.3 +/- 5.6 years. Chromosomal anomalies were: monosomy X 56%; mosaicism 37.2%; structural aberration 10.6%. Mean final height in the whole group was 141.5 (129-160) cm. There was no significant difference in height between the three groups: monosomy X (n = 121: 141.1 +/- 6.4 cm); mosaicism (n = 72: 141.5 +/- 7.5 cm); X anomaly (n = 23: 141.4 +/- 5.0 cm). Mean parental height was 170.4 +/- 7.1 cm (father) and 160.1 +/- 6.2 cm (mother). Parental height and patients' heights correlated significantly, but more so with fathers' heights (r = 0.50) than with mothers' (r = 0.42). The correlation was still apparent with the target height (r = 0.55). The results of different series in the literature show the existence of significant variations as mean final heights are between 136 and 147 cm. These differences can be explained by the variations in normal female heights in each country. We have found in these different countries a very strong correlation (r = 0.91) between normal height and final height in Turner's syndrome.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Combined analysis of islet cell antibodies that cross-react with mouse pancreas, antibodies to the M(r) 64,000 islet protein, and antibodies to glutamate decarboxylase in type I diabetic patients.

OBJECTIVE: A combined analysis of whether islet cell autoantibodies (ICAs) are cross-reactive with mouse pancreas, with glutamate decarboxylase (GAD) antibodies, and with 64K antibodies was performed in a large sample of recently diagnosed type I diabetic patients. The disappearance rates of these different autoantibodies were compared in some patients after onset of the disease. The aims were to determine patterns in GAD/64K antibodies with regard to cross-species reaction of ICA and to assess whether GAD could contribute to ICA positivity in mouse and human pancreases and whether the simultaneous search for all the antibody specificities enhances the detection of autoimmune stigma. RESEARCH DESIGN AND METHODS: ICA detected by immunofluorescence in human and mouse pancreases, antibodies immunoprecipitating the 64K rat islet antigen, and antibodies immunotrapping brain GAD activity were quantified at diagnosis of diabetes in 95 patients and in sequential samples during 1 year after diagnosis in 13 patients. The contribution of GAD to ICA positivity in mouse and human pancreases was evaluated by the analysis of correlations between tests and by the ability of brain homogenate to block ICA reactivity in pancreases from both species. RESULTS: ICAs were detected in human pancreases in sera from 63 (66%) patients, among which 61% bound also to a mouse pancreas. GAD and 64K antibodies were strongly correlated (P < 0.0001) and were detected in 69 and 73% of the patients, respectively. All but two patients with ICA in human pancreas also displayed either ICA in mouse pancreas or GAD/64K antibodies. Among 32 patients without ICA in human pancreas, 54% displayed either GAD/64K antibodies or ICA in mouse pancreas. Only 16% of the patients displayed neither ICA nor GAD/64K antibodies. A correlation (P < 0.005) was found between ICA in human and mouse pancreases. GAD or 64K antibodies were strongly correlated with ICA in human pancreas (P < 0.0001), but not with ICA in mouse pancreas. After preincubation of six sera with GAD-containing brain homogenate, ICA titers were unaffected in mouse pancreas but reduced in human pancreas. ICA titers in mouse pancreas were decreased after 3 months (P < 0.01) in diabetic patients, contrasting with the stability of ICA in human pancreas and GAD antibodies by 1 year after diagnosis. CONCLUSIONS: According to cross-species reaction, we confirm the heterogeneity of ICA in a large series of type I diabetic patients, ICAs that cross-reacted with mouse pancreas being more frequent than ICAs without cross-species reactivity. GAD and 64K antibodies were also present in a majority of patients. The simultaneous search for all the antibody specificities enhances the detection of autoimmune stigma so that only a few patients did not display any autoantibody at diagnosis. GAD is not the target of ICAs in mouse pancreas, whereas GAD accounts for ICA positivity in human pancreas. The conclusion that ICAs in mouse pancreas are not GAD-reactive is reinforced by the fact that they are more transient after onset of diabetes than are GAD antibodies or the complex mixture of ICAs in human pancreas.

Adolescent↗

[Diffuse sclerosing papillary carcinoma of the thyroid gland. Apropos of a pediatric case of association of an unilateral tumor with diffuse lymphocytic thyroiditis].

A 11-year-old girl presented in 1990 with bilateral goiter, hypothyroidism and thyroid auto-antibodies and was treated for Hashimoto's thyroiditis. In 1991, a cervical lymph node metastasis revealed diffuse sclerosing papillary carcinoma (DSPC) of the right thyroid lobe and chronic lymphocytic thyroiditis of the left lobe. The patient is in remission in 1993 (total follow-up, 39 months). The review of 60 previously reported cases of DSPC shows a predilection for young people, a high incidence of lymph node and pulmonary metastases; however the mortality rate is quite low, reflecting either the good prognosis linked to a young age in papillary thyroid carcinomas, or the short duration of the follow-up preventing assessment of the behaviour of DSPC.

Carcinoma, Papillary↗

[Detection of subjects at risk of type 1 diabetes. GOFEDI. Groupe Ouest-France pour l'Etude du Diabète Insulino-dépendant].

The so-called type 1 (insulin-dependent) diabetes is an autoimmune disease occurring in genetically predisposed subjects. The clinical onset of the disease is preceded by a subclinical period during which insulin-producing cells are progressively destroyed by immunological effectors. This prediabetic phase can be detected by the presence of autoantibodies directed against islet cells and sometimes associated with anti-insulin antibodies in children, and later on by the disappearance of the early insulin secretion peak in response to intravenous glucose. It is at this prediabetic phase that immunomodulators specific to the antipancreas process and devoid of side-effects will be used, when available, and that an early insulin therapy will be instituted.

Biomarkers↗

[Hypothalamic syndromes. Review of clinical and endocrinal semiology].

A case of hypothalamic dysfunction in a girl with a twelve-year follow-up is reported. Onset occurred at the age of three with severe obesity, hypothermia, hypersomnia, and lethargy. Somatotropic, gonadotropic, and thyrotropic hormones were low, whereas prolactin was increased. Imaging techniques failed to disclose any lesion of the hypothalamus or pituitary. Clomipramine improved the vegetative disorders. The literature on clinical and hormonal disorders of hypothalamic dysfunction is reviewed.

Child, Preschool↗

[Insulin-like growth factor I (somatomedin C) in premature infants on total parenteral nutrition. Relations with nutritional status and protein-energy intakes].

Insulin-like growth factor I (IGF I) is like prealbumin and transferrin a marker of nutritional status. Its level increases with gestational age. The levels of IGF I (96 times), transferrin (86 times) and prealbumin (69 times) were measured in blood samples from 26 premature infants aged 8 to 78 days (gestational age: 28 to 34 weeks, birth weight: 840 to 1,800 g). At the time of sampling, all the infants were on total parenteral nutrition (360 +/- 42 kJ/kg/day and 2.5 +/- 0.3 g of proteins/kg/day). The results were analysed with reference to anthropometric parameters (weight, height, head circumference, skinfolds and arm circumference). There was no correlation between plasma IGF I and anthropometric measurements. There were significant correlations between IGF I and transferrin (p less than 0.01), prealbumin (p less than 0.05), protein intake (p less than 0.01) an energy intake (p less than 0.05). Plasma IGF I increased at the end of the first week of parenteral nutrition in all the 5 infants having initial low values. The plasma IGF I was not correlated with the duration of parenteral nutrition in the 26 infants after the second week of nutrition. IGF I measurement is useful for evaluating the protein nutritional status of premature infants on total parenteral nutrition.

Energy Intake↗

Acute insulin response to intravenous glucose, glucagon and arginine in some subjects at risk for type 1 (insulin-dependent) diabetes mellitus.

The relationships between first-phase insulin secretion to i.v. glucagon and i.v. arginine were studied in 19 healthy adult volunteers (Group I) and in 21 subjects at risk for Type 1 (insulin-dependent) diabetes mellitus with either a "normal" (n = 11; Group IIa) or a "low" insulin response to i.v. glucose (n = 10; Group IIb). Groups I and IIa displayed similar insulin responses to the three secretagogues. In contrast, Group IIb demonstrated lower insulin responses to both glucagon and arginine than control subjects (p less than 0.007 and p less than 0.04 respectively) or than "normo-responders" to glucose (p less than 0.007 and p less than 0.04 respectively). In Group IIb however, arginine-stimulated insulin release was increased compared to the response to glucose (p less than 0.006), while glucagon and glucose led to non-statistically different responses. Five "low-responders" developed Type 1 diabetes. As a group, they displayed lower responses to glucagon and to arginine than subjects who up to now have not developed the disease (p less than 0.05 and p less than 0.0003 respectively). In the subjects who progressed to diabetes, the responses to glucose and glucagon were similarly blunted. In the "low-responders" who have not developed the disease, no statistical difference could be detected between mean responses to glucagon and glucose, but four out of these five subjects had a glucagon-stimulated response within the control range and higher than their corresponding response to glucose. Arginine led to a higher stimulation than glucose, in subgroups that either progressed to diabetes (p less than 0.006) or did not (p less than 0.002).(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Telematic transmission of computerized blood glucose profiles for IDDM patients.

OBJECTIVE: To improve the analysis of self-monitoring of blood glucose (SMBG) and its communication between patients and physicians by a telematic transmission of computerized SMBG and to study the consequences of its use on glucose control of insulin-dependent diabetic (IDDM) patients. RESEARCH DESIGN AND METHODS: A prospective randomized crossover trial with two 3-mo periods, one with SMBG recorded on traditional booklets (booklet period) and another with computerized SMBG transmitted to a central data base through a telematic network (telematic period), comprised the study. During the latter phase, patients could receive computerized SMBG analysis on individual terminals connected to the telephone network (Minitel system). Blood glucose recordings and HbA1c were measured at inclusion and end of each period. Eleven pairs of IDDM patients on intensified insulin therapy were randomized within each pair to start with the telematic period (group A) or the booklet period (group B). RESULTS: Telematic transmissions were successful (less than 1% failure rate). Although initial HbA1c was low (6.7%), it declined during the telematic period (delta = -0.41%) compared with the booklet period (delta = +0.37%, P = 0.05). The percentage of low (less than 3.3 mM) blood glucose values correlated with HbA1c changes during the telematic period (r = 0.714, P = 0.0014) but not the booklet period. The patients favored the telematic tool to analyze SMBG. CONCLUSIONS: Telematic transmission of SMBG is feasible. It can improve SMBG analysis and perhaps glucose control, therefore offering a new way of communication between diabetic patients and their physicians.

Blood Glucose↗

[Neonatal lupus presenting as telangiectasic and atrophic lesions].

The authors report on a new case of neonatal lupus erythematosus, with an un-usual dermatological presentation: sequelar lesions of telangiectasias associated with areas of skin atrophy. They emphasize the diagnostic difficulties that can be encountered when the mother is symptom-free, and the uncertainties of long-term prognosis of these children.

Atrophy↗

[Munchausen's syndrome by proxy and chronic intestinal pseudo-obstruction].

A case of intestinal pseudo-obstruction in a 4 1/2 year-old boy is reported. All etiologic investigations remained negative. Management successively required continuous enteral feeding, ileostomy then total parental nutrition. The proof of a chronic barbiturate intoxication, induced by the mother, was made only after 2 1/2 years of follow-up, when the patient was in a critical condition. Separation of the child from his family led to complete disappearance of symptoms within a few days.

Barbiturates↗

[Mediastinal pseudocyst in hereditary pancreatitis].

A 6 year-old child was hospitalized for thoracoabdominal pain. There was a clinical and radiological right pleural effusion. Ultrasonography showed a pseudocyst of the head of the pancreas. CAT scan and operative opacification showed a mediastinal extension of the pseudocyst, with no communication with the pleural effusion. This pseudocyst illustrates an unusual variation of a known complication of this disease. Recovery was obtained via fistulization into the jejunum.

Child↗

[Respiratory function during wakefulness and sleep in a 7-year-old child with congenital alveolar hypoventilation of central origin].

Pulmonary function tests were performed in a 7 year-old girl with central alveolar hypoventilation syndrome treated with mechanical ventilation during sleep. Results showed: 1. during wakefulness decrease in residual functional capacity, in dynamic lung compliance and in lung transfer factor for CO; 2. during sleep the characteristics of the syndrome as reported in the neonatal period i.e. central alveolar hypoventilation in stages 2 and 3-4 which justified maintenance of mechanical ventilation when asleep.

Child↗

Nyctohemeral rhythm of plasma renin activity and plasma aldosterone in children.

Circadian rhythms of plasma renin activity (PRA) and plasma aldosterone (PA) were studied in eight healthy children, 7 to 15 years old. Blood samples were obtained at 7:00 AM, 10:00 AM, 1:00 PM, 4:00 PM, 5:00 PM, 8.00 PM, and midnight on day 1 and 4:00 AM and 7:00 AM on day 2. Children had normal activity during this test. Intra- and interindividual changes were noted in PRA in plasma taken in the upright position. Mean PRA values for samples taken in the upright position were maximal at 1:00 PM (7 ng/ml/hr) and minimal at 5:00 PM (4.1 ng/ml/hr). Mean PRA in the supine position was maximal at 4:00 AM on day 2 (5.6 ng/ml/hr), and its rise was statistically significant compared with the mean PRA at 7:00 AM on the same day (3.9 ng/ml/hr). Plasma aldosterone varied without any definite pattern. No correlation was found between PRA and PA and 24-hr urinary sodium excretion.

Adolescent↗

Variation in plasma ketone bodies during a 24-hour fast in normal and in hypoglycemic children: relationship to age.

The variations in blood ketone bodies, blood glucose, and insulin were studied in 19 normal and 14 hypoglycemic children, 4 months to 13 years of age, during a 24-hour fast. Except in four patients (two with hyperinsulinism and two with congenital defect in ketogenesis), a significant increase in blood ketone bodies was observed in both controls and patients. A progressive decrease in glucose concentrations was observed up to but not after 20 hours. A highly negative correlation between blood ketone bodies and blood glucose was found, with a large dispersion of blood ketone bodies, especially for those corresponding to the blood glucose between 45 and 65 mg/dl. This dispersion was consistently reduced in a homogenous age group of 4 to 6 years with similar glucose values. There was a positive correlation between age and blood glucose from hour 21 on, and an inverse relationship between age and blood ketone bodies from hour 15 on. The same high inverse relationship between age and blood ketone bodies was again observed when the variable of glucose concentration was factored out, demonstrating that the variation in blood ketone bodies is indeed related to age. These findings need to be taken into account in the interpretation of fasting blood ketone bodies, especially when used as an aid in the diagnosis of the various forms of childhood hypoglycemia, and of hypoketotic states.

Age Factors↗