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Biomedical subjects

N Blanc

Publications and source records attributed to N Blanc.

At least 19 recordsLinked to original sources
PubMed2006

Prenatal isolated mild ventriculomegaly: outcome in 167 cases.

OBJECTIVE: To define the contribution of prenatal investigation and evaluate the prognosis of isolated mild ventriculomegaly (IMV). DESIGN: Retrospective study. SETTING: University hospital between January 1992 and December 2002. POPULATION: One hundred and sixty-seven cases of prenatal unilateral or bilateral IMV without any associated anomaly at the time of initial diagnosis. METHODS: Complementary investigations were performed: amniocentesis with karyotyping, screening for viruses and acetylcholinesterase electrophoresis, magnetic resonance imaging (MRI), and ultrasonography every 3-4 weeks. MAIN OUTCOME MEASURES: Results of prenatal investigations, pregnancy outcome, and postnatal psychomotor development. RESULTS: IMV was diagnosed around 26.5 weeks. Amniocentesis revealed four chromosomal anomalies and two cytomegalovirus infections. MRI diagnosed brain-associated anomalies in 15 cases and ultrasonographic monitoring highlighted malformations not initially diagnosed in 28 cases. Termination of pregnancy (TOP) was considered in 21 pregnancies (12.6%). Indications were aneuploidy, fetal infectious disease or associated malformations. In women for whom a TOP was considered, consanguinity, fetus of female sex and frontal horn enlargement were statistically more frequent, ventriculomegaly was more often bilateral and asymmetrical, atrial width, and the rate of progressive ventricular enlargement were significantly higher. One hundred and one children with prenatal IMV were assessed between 19 and 127 months (mean age 54.68 +/- 2.87 months). Twelve children had neurological disease or psychomotor delay and 89 children had a normal psychomotor development. Poor neurological outcome was more often associated with atrial width greater than or equal to 12 mm, asymmetrical bilateral enlargement, and progression of the ventriculomegaly. CONCLUSION: The detection of IMV raises the question of the child's psychomotor development and justifies meticulous prenatal investigation. In addition to associated anomalies, three criteria are often associated with an unfavourable outcome: atrial width greater than 12 mm, progression of the enlargement, and asymmetrical and bilateral ventriculomegaly.

Adult↗
PubMed2005

High-density electrode array for imaging in vitro electrophysiological activity.

The development of a high-density active microelectrode array for in vitro electrophysiology is reported. Based on the Active Pixel Sensor (APS) concept, the array integrates 4096 gold microelectrodes (electrode separation 20 microm) on a surface of 2.5 mmx2.5 mm as well as a high-speed random addressing logic allowing the sequential selection of the measuring pixels. Following the electrical characterization in a phosphate solution, the functional evaluation has been carried out by recording the spontaneous electrical activity of neonatal rat cardiomyocytes. Signals with amplitudes from 130 microVp-p to 300 microVp-p could be recorded from different pixels. The results demonstrate the suitability of the APS concept for developing a new generation of high-resolution extracellular recording devices for in vitro electrophysiology.

Animals↗
PubMed2003

[Factors associated with childhood diabetes manifesting as ketoacidosis and its severity].

INTRODUCTION: Type 1 diabetes in children in France is frequently diagnosed at the stage of ketoacidosis (DKA). PATIENTS AND METHODS: A prospective study was performed in a group of 72 children (mean age = 9.4 years) at onset of diabetes, in order to determine which factors were associated to DKA and to the severity of DKA (pH < 7.10) at diagnosis. RESULTS: Younger age was related to DKA (p = 0.03), but not to its severity. A lesser frequency of DKA was found in children with a family history of insulin-treated diabetes ( p = 0.04). Misdiagnosis was more frequently observed in children with DKA than in children without DKA (p = 0.02) and in case of severe DKA at admission by comparison with non severe cases (76 vs 23%; p = 0.002). Children in low economic intake families exhibited more frequently a severe DKA (77 vs 23%; p = 0.002) and were more frequently misdiagnosed before admission (48% vs 10%; p < 0.01). Urine strips for glucose and ketone determinations were underused for diagnosis before admission (15% only). CONCLUSION: Those results underline the need to both inform physicians and ameliorate the access to health care for low social class families, in order to take up the challenge of reducing the incidence of DKA at diagnosis in diabetic children in our country.

Adolescent↗
PubMed2001

Late-onset cystic periventricular leukomalacia in premature infants: a threat until term.

The purposes of this study are (1) to describe a "late-onset" form of cystic periventricular leukomalacia eventually appearing in premature infants whose neurological assessments were normal in the first month of life; (2) to retrospectively evaluate its incidence among a large population of premature infants; (3) to suggest that a few unexpected complications of prematurity may trigger the development of white matter damage, even several weeks after birth. Retrospective study in a population of 1452 surviving infants after 5 days born before 33 weeks. We identified 10 cases of late-onset cystic periventricular leukomalacia appearing beyond the first 5 weeks of life. In 8 cases, an intercurrent event associated with a systemic inflammatory response preceded the appearance of cysts: necrotizing enterocolitis (n = 5), septicemia (n = 2 cases), strangulated inguinal hernia in one infant. Neurological surveillance should be repeated until discharge in very preterm infants, especially after the occurrence of an intercurrent complication coming along with a systemic inflammatory response.

Age of Onset↗
PubMed2001

Pulmonary sarcoidosis in children: a follow-up study.

Progression of pulmonary sarcoidosis in children remains poorly documented. The aim of this work was to gather follow-up information on pulmonary outcomes in children with sarcoidosis and to obtain data of relevance to a discussion of the optimal length and regimen of glucocorticoid therapy. In the present study, the authors experience of pulmonary sarcoidosis in 21 children referred to the paediatric pulmonary department over a 10-yr period is reported with a documented follow-up of at least 4 yr. Evaluation of the disease during the follow-up included analysis of clinical manifestations, chest radiographs, pulmonary function tests with measurements of the vital capacity (VC), dynamic lung compliance (CL,dyn), lung transfer for CO (TL,CO), and arterial blood gases, as well as bronchoalveolar lavage (BAL) with determination of total and differential cell counts. After initial evaluation the decision was a careful observation of four children without therapy. Corticosteroid treatment was initiated in 17 children. Analysis of results indicated that after 6-12 months of treatment most clinical manifestations of the disease and chest radiograph abnormalities disappeared, and beneficial effects on VC and TL,CO were apparent. After 18 months of steroids no benefit on pulmonary function tests could be noticed, with mainly persistence of alterations of CL,dyn. Results of BAL studies documented the presence of an alveolitis with increased lymphocyte populations throughout the follow-up. Relapses were observed in four children during tapering of prednisone; they were not reported after discontinuation of steroid therapy. Taken together data obtained in the presented population can lead to the following suggestions for the management of pulmonary sarcoidosis in children. BAL should be performed at the initial evaluation to document alveolitis; however, nothing seems to be gained from repeating this investigation during follow-up in the absence of specific reasons. Once the decision to initiate glucocorticoid therapy is made, 18 months may be a reasonable treatment duration. Discontinuation of therapy can be decided even if the pulmonary function tests remain abnormal, but the child should then be carefully monitored for a relapse.

Adolescent↗
PubMed2000

Infantile spasms and Menkes disease.

Epileptic seizures are a common feature in Menkes disease, an X-linked genetic disorder of copper metabolism. Details of type of seizures are rarely reported. We report the evolution of infantile spasms in two patients with Menkes disease and the relation with subcutaneous administration of copper-histidine.

Brain↗
PubMed1997

[Severe ketoacidosis because of delayed diagnosis of diabetes in children. 4 cases to alert physicians].

BACKGROUND: In France, 48% of children with insulin-dependent diabetes mellitus (IDDM) are seen for the first time at the stage of keto-acidosis (DKA), a figure far higher than in other countries. The four consecutive cases reported in this paper suggest that severity at presentation is directly related to the delay of diagnosis. CASE REPORTS: All children, aged 6 to 11, presented with severe clinical and metabolic conditions: coma, hemodynamic failure, severe dehydration and acidosis (pH = 6.86 to 7.06). However, clinical symptoms were a present from 2 to 4 months, parents did not worry about them. Family practitioners visited 2-21 days before admission failed to immediately make the diagnosis of IDDM. True DKA was however present in all cases 48 hours prior to admission. CONCLUSIONS: Earlier diagnosis of IDDM in children is necessary to reduce morbidity and mortality of DKA. General practitioners, pediatricians and emergency care practitioners must be made fully aware to consider diabetes in children and to use more extensively glucose-tests based on urine and blood strips.

Child↗
PubMed1989

Epstein-Barr virus associated lymphoproliferative diseases (B cell lymphoma) after transplantation.

We report 12 cases of lymphomas which occurred among 1670 patients with kidney or combined renal and pancreatic transplantation. Group 1 comprised nine patients presenting with the diffuse form of the disease where immunoblasts or mature plasma cells massively infiltrated all organs. The first symptom was a viral syndrome, associated with a restriction of heterogeneity of immunoglobulins; oligoclonal to monoclonal peaks of immunoglobulins appeared about 50 days after transplantation. All patients received antilymphocyte globulins (ALG), and seven were treated with cyclosporin. EBV infection could be demonstrated in almost all patients; three EBV lymphoblastoid cell lines were established, their HLA phenotype being the same as the recipient of the graft. All patients finally died with renal and hepatic failure. Group 2 comprises three patients who presented solid B cell tumours of tonsils, lungs, and spleen at onset, extending to liver, kidney graft, lymph nodes, and brain. All received cyclosporin; two patients were treated with ALG, and one with OKT3. Immunoglobulins were polyclonal, oligoclonal, or decreased. Cell surface immunoglobulins were monoclonal on two tumours. EBV-DNA was positive within two tumours. Two patients presented EBV and CMV primary infection. CD4+T lymphocytes subsets were diminished at onset, and increased after cessation of immunosuppressive therapy. One patient died because of brain involvement; the two others are alive, one with perfect graft function. Therapy consisted of stopping immunosuppressive treatment, Acyclovir, and in two patients of group 2, monoclonal antibodies to pan-B and EBV receptor antigens.

Acyclovir↗
PubMed1983

Endocrine responses of type 1 (insulin-dependent) diabetic patients following successful pancreas transplantation.

The aim of the present study was to evaluate the insulin and glucagon responses to various stimuli in patients following pancreatic transplantation. Four Type 1 (insulin-dependent) diabetic patients with end-stage renal failure who had received a cadaveric segmental, neoprene-injected, pancreas transplant, in association with kidney transplantation, were investigated. Free-insulin, pancreatic glucagon, and growth hormone concentrations were measured after both oral and intravenous glucose tolerance tests, and following tolbutamide, arginine and arginine plus somatostatin infusions. Tests were performed 1 month (three cases) and 30 months (one case) after surgery, when no insulin administration was required. Four non-diabetic kidney grafted patients, matched for duration of graft survival and immunosuppressive treatment (steroids, azathioprine and anti-lymphocyte-globulins), served as control subjects. Impaired glucose tolerance was present in all diabetic and control patients. This was possibly related to immunosuppressive treatment. In comparison with control subjects, insulin release was normal in response to arginine and tolbutamide but was reduced in response to oral and intravenous glucose, while glucagon and growth hormone release were similar in both groups. Somatostatin was less effective in diabetic patients than in control subjects in suppressing insulin and glucagon release.

Adult↗
PubMed1983

A method for early detection of graft failure in pancreas transplantation.

Pancreatic transplantation is intended to normalize carbohydrate metabolism in insulin-dependent diabetics by restoring endogenous insulin release, and it is usually performed together with kidney transplantation in patients with end-stage renal failure. A major problem in these patients is the daily control of the grafted pancreas because traditional measurements do not appear to be adequate to evaluate pancreatic function. Aiming at early detection of graft failure, we have analyzed in 8 such patients and in 20 nondiabetic kidney-grafted patients (a control group) the following variables: 24-hr glycosuria (absolute values, or values after natural logarithmic transformation) and 24-hr urinary C-peptide excretion (corrected for 24-hr urinary creatinine). These measurements, considered alone, did not detect pancreatic graft failure; for instance, glycosuria can depend on immunosuppressive steroid treatment, and it was often found even in the control group. On the contrary, the ratio Ln 24-hr glycosuria: 24-hr urinary C-peptide varied from 0.00 to 0.18 in the control group and in normally working pancreatic grafts; when the pancreatic grafts failed, however, as confirmed by arteriographic evidence, histologic findings, or dynamic endocrine tests, this ratio rose far higher than 0.18, reaching values as high as 12.2. Use of this ratio provides a simple technique for daily evaluation of pancreatic graft function and for early detection of graft failure.

Diabetes Mellitus↗