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Biomedical subjects

P Czernichow

Publications and source records attributed to P Czernichow.

At least 19 recordsLinked to original sources
PubMed1992

Islet cell antibodies in normal French schoolchildren.

Islet-cell antibodies have been reported to be of predictive value for the future development of Type 1 (insulin-dependent) diabetes in first degree relatives of diabetic patients with the risk increasing in these subjects with the islet-cell antibodies titre. However, very little is known about islet-cell antibodies in background populations. Sera (n = 8363) from schoolchildren (6-17 years) in the French background population were screened for the presence of islet-cell antibodies by the indirect immunofluorescence technique. Islet-cell antibodies greater than 4.5 Juvenile Diabetes Foundation units were found in 150 sera (prevalence rate 1.8%; 95% confidence interval 1.5-2.1%). Only 17 sera demonstrated islet-cell antibody titre greater than or equal to 24 JDF units. No particular feature was found to be significantly different between islet-cell antibody-positive and islet-cell antibody-negative children (age, family history of diabetes, fasting plasma glucose, insulin autoantibodies). A second blood sample was obtained from 80 of 150 islet-cell antibody positive children after a mean interval of 8 months. Only 11 sera became less than 4.5 JDF units with islet-cell antibody titres being stable in the remaining sera, including the high-titre positive sera (greater than or equal to 24 JDF units). HLA-DQB typing was performed by restriction mapping techniques in 80 islet-cell antibody-positive, in 93 islet-cell antibody-negative and in 213 Type 1 diabetic children. The distribution of the susceptibility alleles (DQB1-Asp57-negative) was not significantly different between islet-cell antibody-positive and islet-cell antibody-negative children.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1992

Relationship between clinical symptoms of anal incontinence and the results of anorectal manometry.

The aim of this work was to analyze clinical symptoms in light of anorectal manometry results. We compared the frequency of clinical symptoms in relation with the presence or absence of functional anomalies. Using this methodology, the following relationships may be suggested: the need to wear a pad, with a decreased resting pressure at the upper part of the anal canal; the inability to delay rectal evacuation, with decreased anal voluntary contraction; interference of incontinence with social activities, with decreased duration of anal voluntary contraction; urinary symptoms, with an increased threshold volume of rectal distention needed to elicit the rectoanal inhibitory reflex; and complete rectal prolapse, with reduced length of the anal canal.

Adult↗
PubMed1992

Insulin, glucagon and somatostatin in fetal rat islets maintained free-floating in culture: immunocytochemistry and radioimmunoassay.

Fetal rat islets maintained free-floating in tissue culture represent a source of B-cells. Because we recently noted the occurrence of other cell types during long-term tissue culture, this in vitro model was used to examine the possible development of non B-cells. The changes in the numbers and percentages of B, A and D-cells in vitro were estimated by counting the hormone-positive cells after immunocytochemical staining. Insulin, glucagon, and somatostatin contents were determined in extracts of the cultured tissue. The experiments described here showed that the cultured islets maintained their viability over a two-week culture period, as evidenced by the increase of both the number of B-cells per islet and the DNA content per islet. During the first few days of culture, immunocytochemically stained free-floating islets indicated the presence of rare A- and D-cells at the periphery of B-cells; thereafter, numerous A- and D-cells were seen interdigitating with B-cells. Expressed per islet, the number of A- and D-cells increased during the culture; within the endocrine cell population, the percentage of these cells increased with time, at the expense of the percentage of B-cells. The glucagon and somatostatin contents of the free-floating islets were also increased. These converging observations suggest that additional non B-cells may have been produced by free-floating islets during long-term tissue culture.

Animals↗
PubMed1992

Clinical and laboratory features of type 1 diabetic children at the time of diagnosis.

The French incidence study has registered all new cases of Type 1 diabetic children under 20 years of age, from a population of 2.32 million, in an exhaustive and prospective manner. Three hundred and forty cases were identified between 1 January 1988 and 31 December 1989, yielding a mean annual incidence rate 7.3 per 10(5). The lowest rate was observed in the youngest age group (0-4 yr: 4.1 per 10(5)) and the highest around pubertal development (10-14 yr: 11.5 per 10(5)). Details of the previous personal and family history, and the clinical and biological pictures of the disease at diagnosis were recorded. Almost 8 per cent of the children had a first-degree relative with Type 1 diabetes. Polyuria, weight loss, fatigue and abdominal pain were the most frequently reported symptoms, which were of median duration 4.4 months. Mean weight loss before diagnosis was 9.4 +/- 6.8 (+/- SD)% of body weight and was not significantly related to age. Ketonuria was detected in 83.8 per cent and acidosis (total CO2 less than or equal to 18 mmol l-1, if measured) in 48 per cent of the cases. Ketonuria and acidosis were significantly more frequent in the younger age group than in the rest of the group (p less than 0.001).

Adolescent↗
PubMed1992

Hypothalamopituitary deficiency and precocious puberty following hyperhydration in diabetic ketoacidosis.

We report on a 5-year-old child who survived an intracerebral crisis, following ketoacidosis-revealing diabetes (DKA), with visual impairment due to a vascular occipital lesion. Two and 4 months after the initial episode, a unique hypothalamopituitary disorder consisting in GH, ACTH, TSH deficiencies and central precocious puberty, was detected. Cranial magnetic resonance images showed no visible lesion in the hypothalamopituitary region. The most likely hypothesis is the ischemia of hypothalamopituitary and occipital regions following possible cerebral edema after hyperhydration. She survived with low visual acuteness and received a combined replacement therapy for the neuroendocrinological deficiencies. This case emphasizes that the rehydration at the initial period of DKA is critical, especially when risk factors for cerebral edema are present (young age, marked hyponatremia). The neuroendocrinological consequences of acute cerebral edema are rare, but physicians must be attentive in survivors of these accidents.

Adrenocorticotropic Hormone↗
PubMed1992

Are cysteine-rich and COOH-terminal domains of dystrophin critical for sarcolemmal localization?

It has been hypothesized that the tight localization of dystrophin at the muscle membrane is carried out by its cysteine-rich and/or carboxyl domains. We report the results of biochemical and immunocytochemical investigations of dystrophin in muscle from a 1-yr-old patient with a large deletion that removes the distal part of the dystrophin gene, thus spanning the exons coding for the cysteine-rich and the carboxy-terminal domains, and extends beyond the glycerol kinase and congenital adrenal hypoplasia genes. Immunological analysis of muscle dystrophin shows that the deletion results in the production of a truncated, but stable, polypeptide correctly localized at the sarcolemma. These data indicate that neither the cysteine-rich domain, nor the carboxyl domain, are necessary for the appearance of normal dystrophin sarcolemmal localization.

Cysteine↗
PubMed1992

[Risk markers of insulin-dependent diabetes in the normal population].

Risk factors for IDD have been studied in relatives of patients, in whom the risk is far higher than in the children from the background population. Several factors have been identified as the genetic background at the HLA level (DR and DQ regions) and autoantibodies like islet cells antibodies (ICA) and insulin autoantibodies (IAA). 8,363 sera from schoolchildren (age 6-17 yr) have been tested for the presence of ICA. 150 sera (1.8%) were found positive; the prevalence rate of high ICA titres (> or = 20 uJDF) was very low: 0.2%. ICA titres remained stable over a 8 mth follow-up period. IAA have been measured on the first 2,000 sera and the prevalence rate was 1.2%. Only 3 sera were found positive for the two antibodies, one coming from a diabetic girl. The distribution of the susceptibility alleles at the DQ-HLA region was similar among the ICA-positive and ICA-negative children.

Adolescent↗
PubMed1992

[French-language adaptation of an evaluation test of knowledge meant for insulin-dependent children: methodology and value for research in clinical practice].

A measure of knowledge level of diabetic patients is useful in evaluating their educational needs as well as the impact of the educational programs specifically designed for them. The lack of such scales in French, led us to translate and validate an existing knowledge scale for diabetic children, the Test of Diabetes Knowledge (TDK) (Johnson et al.). After several translations and back translations, the 33 item scale was submitted to the staff members of a pediatric diabetology unit, to establish its content validity. An assessment the questionnaire was performed in a group of 49 children, aged 7.9 to 12.8 years. The mean duration of diabetes was 4.6 +/- 2.9 years and their scholastic level varied from 1st to 6th grades. Time spent in filling out the questionnaires was 12 to 25 minutes. Few missing values were noted. The reliability of the scale was excellent as shown by Cronbach's alpha coefficient: 0.83 for the overall scale, 0.72 for the general knowledge subscale (20 items) and 0.70 for the problem solving subscale (13 items). The level of correct responses was correlated with age and scholastic level (p < 0.0001), but not with sex, duration of diabetes, or HbA1c levels. This study shows that the French version of the TDK is a valid and reliable tool for measuring the level of knowledge in children and adolescents with diabetes. This scale provides the researcher and clinician with a tool that facilitates the performance of an educational diagnosis and the evaluation of educational programs based on the transmission knowledge.

Adolescent↗
PubMed1992

[Should adult nephrotic syndrome secondary to idiopathic extramembranous glomerulonephritis be treated? An approach by decisional analysis].

Treatment of nephrotic syndrome (without renal insufficiency) due to idiopathic membranous glomerulonephritis in adults is not clearly defined. A decisional analysis was done from data published in literature in order to define the best therapeutic choice. Three therapeutic strategies were studied: corticosteroids (either 125 mg/48 h or 30 to 60 mg/24 h), alternate therapy according to Ponticelli protocol (corticosteroids and chlorambucil), abstention of therapy. The data were divided in two groups according to the time of follow-up. The first group allows a decisional analysis for a short period of time (16 to 37 months), the second group allows this analysis for a longer period of time (54 to 65 months). The results differed according to the length of post treatment follow-up. For the shorter follow-up, corticosteroids or the association corticosteroids and chlorambucil appeared to be better than no treatment. For longer follow-up, any treatment shows an advantage in comparison to abstention of therapy. Our tentative conclusion is to propose only symptomatic treatment for this disease.

Adrenal Cortex Hormones↗
PubMed1992

Direct double monoclonal immunoradiometric assay of urinary human growth hormone.

Several reports indicate that urinary growth hormone (GH) excretion might reflect central release of the hormone, and that measurement of urinary GH shows promise in the investigation of physiological and pathological GH secretion. We have developed and evaluated a direct immunoradiometric assay (IRMA) in which two monoclonal antibodies are used to measure GH in the urine of children. The detection limit is approximately 0.018 pmol/L for a sample volume of 2 mL. Within- and between-run variations (CVs) were 5.6% and 14.2%, respectively. Analytical recovery and dilution experiments showed the specificity of the method for GH. In normal-stature prepubertal children ages 3-12 years, 24-h urinary GH excretion was 0.189 (SD 0.100) pmol and correlated well with the amount of GH in the first morning miction, which showed wide day-to-day variations. Like others, we found a strong correlation between GH concentrations in serum and urine during stimulation tests with GH-releasing hormone (somatoliberin) and (or) during physiological nocturnal secretion, confirming that urinary GH measurement may be of help in investigating patients (particularly young children) with diseases in which GH secretion is impaired.

Antibodies, Monoclonal↗
PubMed1992

[Critical study of diabetic ketoacidosis in children. Initial description and course during the first 24 hours of treatment].

A critical analysis of the evolution during the first 24 hours was undertaken in 41 children and adolescents (age: 10.1 +/- 4.6 years) treated for diabetic ketoacidosis. Three of 4 children presented with ketoacidosis revealing diabetes. One of 4 was less than 6 years of age. Severe ketoacidosis (pH less than 7.15) concerned one third of children and were more frequent in the group of adolescents with already known diabetes. In these patients, ketoacidotic decompensation was attributed to psychosocial factors in most cases. Evolution was favorable in all cases, without complication. Blood glucose levels decreased from 28.7 mmol/l on arrival to 16.2 mmol/l after 2 hours of treatment and became stable at 10 mmol/l from the 12th to the 24th hours. The corrected blood sodium levels were stable, showing the adequacy of infusion solute osmolarities. Blood potassium was maintained at a normal level owing to early potassium supplementation. Ketoacidosis was corrected after about 12 hours, without bicarbonate administration when pH was greater than 7.15. Average perfused volumes were 3 l/m2/24 hours. Insulin doses were 2 UI/kg/24 hours and were inversely correlated with the admission pH (r = -0.6; p = 0.0001). This study shows the efficacy of a treatment taking into account the pathophysiology of diabetic ketoacidosis and the knowledge of the complication risk factors, by foreseeing the adjustments to be done with respect to individual and/or at risk situations. These precise descriptive data, collected on a large group of patients, establish a reference basis to follow evolution in the course of the treatment of diabetic ketoacidosis in children.

Adolescent↗
PubMed1991

[Hypocholesterolemia: prevalence, diagnostic and prognostic value. Study in a department of internal medicine].

Serum cholesterol was measured in all patients admitted to the Department of Internal Medicine over one year. 6.2 percent had hypocholesterolaemia defined as total serum cholesterol level below 4 mmol/l. Comparison with non-hypocholesterolaemic patients showed male predominance and indirect arguments for increased morbidity: longer duration of hospital stay, greater number of rehospitalizations, greater number of associated diseases. In over a quarter of the cases, hypocholesterolaemia was associated with malignant disease. These results should entice physicians to caution when finding a low serum cholesterol level: this could be a marker of associated disease, especially malignant.

Adult↗
PubMed1991

Growth hormone treatment and diabetes: survey of the kabi pharmacia international growth study.

Out of a total of 8136 children registered in the Kabi Pharmacia International Growth Study, 12 have been reported to have diabetes either before or during treatment with growth hormone (GH). Two of these have non-insulin-dependent diabetes mellitus (NIDDM), of whom one had risk factors for the development of his condition, namely gross obesity and familial occurrence of NIDDM. One is a rare case of McCune-Albright's syndrome with insulin-dependent diabetes mellitus (IDDM), and 9 other patients have IDDM. Of these 9, 6 have idiopathic GH deficiency. In 8 of the 9 patients with IDDM, the condition was diagnosed before GH therapy was commenced, at ages ranging from less than 2 years to 16 years. The association is probably fortuitous, however, as the onset of IDDM in 7 patients was immediately before or during puberty, as often occurs in IDDM in general.

Adolescent↗
PubMed1991

Superiority of radiobinding assay over ELISA for detection of IAAs in newly diagnosed type I diabetic children.

OBJECTIVE: Liquid- or solid-phase assays have been used for insulin autoantibody (IAA) determination, and the method of IAA measurement has not been standardized. RESEARCH DESIGN AND METHODS: IAAs were determined by radiobinding assay (RBA) and enzyme-linked immunosorbent assay (ELISA) in two large age-matched groups of nondiabetic and newly diagnosed insulin-dependent (type I) diabetic children. RESULTS: Positivity for IAA by RBA (greater than or equal to nondiabetic mean + 3SD) was 2 of 178 (1.1%) and 55 of 173 (32%) in nondiabetic and diabetic children, respectively. Prevalence of IAA by RBA was significantly higher in the youngest age-group (63% between 0-4 yr). Positivity for IAA by ELISA was 1 of 178 (0.6%) and 8 of 169 (4.7%) in nondiabetic and diabetic children, respectively. Concordance rates between both assays were 0 of 3 (0%) in control subjects and 5 of 58 (8.6%) in diabetic children. CONCLUSIONS: We conclude that RBA is more appropriate than ELISA for IAA detection at the onset of the disease. In addition, because available data suggest that IAAs detected by RBA only are high-affinity antibodies, it is tempting to speculate that IAAs reflect a mature immune reaction against endogenous insulin.

Adolescent↗