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Biomedical subjects

P Reinert

Publications and source records attributed to P Reinert.

At least 73 records · Page 4Linked to original sources
PubMed1994

Immunohistochemical analysis of dystrophin-associated proteins in Becker/Duchenne muscular dystrophy with huge in-frame deletions in the NH2-terminal and rod domains of dystrophin.

The absence of dystrophin causes the drastic reduction of the dystrophin-associated proteins (DAPs) in the sarcolemma and the loss of the linkage between the subsarcolemmal cytoskeleton and the extracellular matrix in Duchenne muscular dystrophy (DMD) skeletal muscle. Here, we report a mild reduction of the DAPs in the unique Becker muscular dystrophy patients with huge deletions in the rod domain of dystrophin and a moderate reduction of the DAPs in patients with huge deletions that involve both the NH2-terminal and rod domains of dystrophin. The phenotype of the latter patients was more severe than that of the former. In both cases, however, the reduction in the DAPs was milder than in typical DMD patients or DMD patients lacking the COOH-terminal domains of dystrophin. Our results suggest that (a) the NH2-terminal and rod domains of dystrophin may not be essential for the interaction with the sarcolemmal glycoprotein complex; and (b) defects in the actin binding activity of dystrophin may cause disruption of the anchorage of the dystrophin-glycoprotein complex to the subsarcolemmal cytoskeleton, which may render muscle fibers susceptible to degeneration.

Adolescent↗
PubMed1994

[Targetted antibiotic therapy. Acute sore throat: streptococcus A update].

Acute sore throat is a very common pathology, but should not because of this be considered as banal. In effect, the beta-haemolytic streptococcus A, which is responsible for most of the bacteriological etiologies is not only responsible for distant inflammatory complications, acute articular rheumatism (RAA) and glomerulonephritis, which are re-appearing in the United States, but also a fulminating septicemia and a syndrome of visceral failure that makes a grave prognosis for life. Moreover, today, streptococcus A is one of the factors involved in a series of fatal fasciites and necrosing myosites seen in several European countries. Understanding of these complications gives better definition of the causative immunological mechanisms and particularly the adverse role of the "superantigens" of streptococci in the start of an increase in the responses of immunocompetent cells and pro-inflammatory and prothrombic mediators. Finally, availability now of rapid diagnostic tests with monoclonal antibody techniques confirms the presence of streptococci A in acute sore throat and should help the physician to make an etiological diagnosis that takes into account the clinical signs. Unfortunately, these tests are not widely available in France and are not subject to reimbursement. All these factors justify the introduction of an antibiotherapy targetted at streptococcus A in the context of bacterial sore throat. Oral penicillin V (phenoxymethyl penicillin, Oracilline) is always the reference, with an excellent anti-bacterial and clinical activity and without risk of production of strains of streptococcus that are of reduced sensitivity or resistant.(ABSTRACT TRUNCATED AT 250 WORDS)

Acute Disease↗
PubMed1994

[Erythrocytosis in a 4 year-old child: diagnostic, prognostic, therapeutical problems].

BACKGROUND: Pure erythrocytosis is rare in children. This report describes such a case. CASE REPORT: A 4 year-old boy was admitted because erythrocytosis had been found routinely before adenoidectomy. He was born in Guatemala, was adopted just after his birth, and had been living in France since that age. Clinical examination was normal. His hemogram showed: erythrocytes: 8,800,000/mm3; hemoglobin: 20.1 g/dl; hematocrit: 66.8%; reticulocytes: 262,000/mm3; platelets: 200,000/mm3; leukocytes: 6,800/mm3. The patient had been given iron salts for the past 3 months without an earlier hemogram. Total red cell mass was 1200 ml (N: 600). The myelogram was normal as was the leukocyte alkaline phosphatases, serum lysozyme and vitamin B12. Blood ferritin was low (3.5 ng/ml). In vitro cultures of erythroid precursors were normal, as was the karyotype of myeloid cells. Blood erythropoietin concentration was 20-293 mU/ml (N:4-14). All the causes of secondary polycythemia were eliminated by appropriate investigations. The patient was treated by phlebotomy in aliquots of 25 ml/kg, twice a month, for 10 months, and was given iron therapy. At the end of treatment, his hemoglobin was 14 g/dl and his hematocrit was 45%. Both progressively increased again one year later, requiring new phlebotomies. The patient was followed for 4 years but no cause for this erythrocytosis was found; it was probably congenital in origin. CONCLUSION: This case of pure erythrocytosis was associated with elevated erythropoietin production. Whether this high secretion is related to receptor dysfunction remains to be determined.

Bloodletting↗
PubMed1994

[Septicemia due to Streptococcus milleri with pulmonary complications].

BACKGROUND: Streptococcus milleri can occasionally cause serious septicemia, that is often complicated by abscesses, particularly pulmonary. CASE REPORT: A 12.5 year-old boy was admitted because he had suffered from fever for 6 days. He also had headache, diffuse abdominal pain, chills and a cough. Laboratory investigations showed leukocytes: 9,300/mm3 (PMN: 6,700/mm3; lymphocytes: 1,400/mm3; monocytes: 1,200/mm3); hemoglobin: 12.5 g/dl; platelets: 106,000/mm3; sedimentation rate: 120 mm at 1 hour; blood fibrinogen: 5.6 g/l and C-reactive protein: 193 mg/l. His chest X-rays was normal. Four blood cultures showed Streptococcus milleri. The patient was given amoxicillin (150 mg/kg/d) plus gentamicin (3 mg/kg/d). Two foci of dental infection were found and treated by tooth extraction. Blood cultures remained positive 2, 3 and 6 days after onset of treatment and a second chest X-rays and CT scan showed several bilateral pulmonary abscesses. Cardiac ultrasonographs showed no valvular vegetations. The patient was then given ceftriaxone (100 mg/kg/d) plus rifampicin (20 mg/kg/d) and netilmicin (6 mg/kg/d). Blood culture was negative two days later, but the fever persisted and ceftriaxone was then successfully replaced by vancomycin (40 mg/kg/d) for three weeks. CONCLUSION: There are several reasons for the apparent resistance to treatment of this potentially severe infection; they are discussed by the authors.

Bacteremia↗
PubMed1993

Infusion of Fc gamma fragments for treatment of children with acute immune thrombocytopenic purpura.

Treatment of acute immune thrombocytopenic purpura (ITP) with intravenous immunoglobulin (IVIG) induces partial or complete responses, shown by transient or persistent increases in platelet count. The clinical benefit could be due to blockade of the Fc gamma receptor (Fc gamma R); platelets sensitised by IgG could not be cleared by cells of the reticuloendothelial system if Fc gamma R on these cells was blocked with IVIG. To find out whether this putative mechanism is correct, we treated twelve children who had acute ITP with intravenous infusions of Fc gamma fragments. Eleven children showed rapid increases in platelet counts to above the critical value of 50 x 10(9)/L, thereby avoiding major haemorrhagic risk. The response was stable in six patients and transient in five. No adverse reactions were observed. In responders who had detectable platelet-associated IgG before treatment (> 1500 IgG per platelet), platelet IgG fell substantially with treatment. Serum soluble CD16 (sCD16 or sFc gamma RIII) concentrations, measured in five children, showed transient or stable increases that correlated with the rise in platelet count. No sCD16 was detected in the Fc gamma preparation used. We conclude that the infusion of Fc gamma fragments is an efficient treatment of acute ITP in children. The efficacy of Fc gamma fragments strengthens the hypothesis that Fc gamma R blockade is the main mechanism of action of IVIG in ITP, although other immunoregulatory mechanisms triggered by the presence of increased sCD16 concentrations in serum could be involved in the clinical benefit observed.

Adolescent↗
PubMed1993

Epidemiology of Haemophilus influenzae type b disease in France.

Results are reported of an epidemiological study which spanned ten years and was carried out in two French departments. A total of 277 cases of Hib disease occurring in children < 5 years old are reported and, in this age group, the incidence is evaluated at 21/100,000. Meningitis accounted for 64% of infections and epiglottitis for 7%. While the overall mortality rate was 3.3%, death was secondary to meningitis in 7/8 cases. Sequelae, which were all related to meningitis, were major in 1.2% of cases, severe in 9% of cases, and involved some hearing loss in 3.3% of cases. The monitoring networks set up in the two departments were characterized by sensitivities of 87 and 94%, respectively. They should prove useful in assessing the impact of vaccination, when large-scale implementation of vaccination has spread to both departments.

Child, Preschool↗
PubMed1993

[Treatment of neutropenia in Shwachman's syndrome with granulocyte growth factor (G-CSF)].

BACKGROUND: Patients with Shwachman syndrome have neutropenia and depressed neutrophil chemotaxis and are therefore susceptible to recurrent infections. The diversity of causative microbial agents makes prevention of infection difficult. Some may be life-threatening, despite antibiotic therapy and even leukocyte transfusion. PATIENT: A 15 day-old boy presented with a staphylococcal cutaneous abscess. Neutropenia was detected when he was 45 day-old and Shwachman syndrome was diagnosed at the age of 8 months. He was then suffering from pneumonia plus pancreatic insufficiency, metaphysical chondroplasia and short stature. Frequent infections continued through childhood, but became less frequent from the age of 11 years. At 17 years, he still had neutropenia (polymorphonuclear leukocytes less than 300/mm3) and profound depressed chemotaxis. He was given subcutaneous injections of recombinant human granulocytes colony stimulating factor (rhG-CSF), 1 microgram/kg/day, for 15 days. The polymorphonuclear count increased above 1000/mm3 during the second week of treatment, and this effect was seen again during a second course of rhG-CSF. The benefit was not sustained when treatment was discontinued. CONCLUSION: These results confirm earlier reports of the effect of 5 micrograms/kg/day of rhG-CSF but the responses were greater and earlier. While more precise information concerning the treatment of this disease is required, rhG-CSF can be useful in patients with severe infections.

Agranulocytosis↗
PubMed1992

[Changes in the fundus in subacute sclerosing panencephalitis. Apropos of 23 cases].

Subacute sclerosing panencephalitis (SSPE) is a fatal slow encephalitis. It is related to a chronic infection of the central nervous system by a defective measles virus. Among 41 patients with SSPE, 23 had ocular involvement. The ophthalmologic involvement preceded the onset of neurological signs in 4 cases, they were detected at first presentation in 9 cases, and they occurred in the course of the disease in 10 cases. The ocular involvement was bilateral in 19 cases. Various ophthalmologic features were reported: retinitis (16 eyes), macular pigment disturbances (4 eyes), optical neuritis (33 eyes), visual agnosia (1 patient). The macular lesions were typical of the disease, but at the acute stage they were liable to be confused with toxoplasmosis. This complication of measles should disappear with the systematic vaccination of 12 month old children.

Adolescent↗
PubMed1991

Thrombosis in systemic lupus erythematosus: a French collaborative study.

A retrospective study was undertaken of 120 children with systemic lupus erythematosus (SLE) seen in Paris and its immediate suburbs who fulfilled at least four of the American College of Rheumatology diagnostic criteria for SLE, and in whom the disease was diagnosed before the age of 16 and between January 1975 and December 1987. Eleven of these children (eight girls and three boys) all more than 10 years of age (mean follow up 8.1 years; range 3-13) had thrombotic episodes (9%). Thrombosis was one of the presenting signs in seven patients; in five it was associated with typical symptoms of SLE, and in the other two the thrombotic episode was isolated and diagnosis of SLE was delayed one and three years. Of a total of 16 thrombotic episodes (six of which were recurrent), 14 involved the leg veins, and in four there was associated pulmonary embolism. There were two episodes that affected cerebral arteries. The American College of Rheumatology diagnostic criteria for SLE as well as the incidence of lupus anticoagulant, positive direct Coombs test, and vasculitis in this group of patients was compared with the incidence in patients with SLE but no thrombosis. Only lupus anticoagulant was significantly associated with thrombotic episodes: eight of 11 (73%) of patients with SLE and thrombotic (arterial or venous) episodes had lupus anticoagulant compared with only 10 of 74 patients (14%) with no history of thrombotic events in the same age group.

Adolescent↗
PubMed1991

[Evaluation of tixocortol pivalate-neomycin combination versus ++a placebo excipient in acute rhinopharyngitis in children].

Effectiveness and clinical tolerance of the tixocortol-neomycin combination (Pivalone-Neomycin nasal suspension) used as monotherapy were evaluated in a double-blind placebo-controlled study (placebo: vehicle i.e., N-cetylpyridinium chloride, sodium chloride, sodium hydroxide solution, benzyl alcohol, purified water, monosodium phosphate) in 211 pediatric patients (aged 6 months to 8 years) with uncomplicated acute rhinopharyngitis. After seven days therapy, improvement in symptoms of acute rhinopharyngitis, especially rhinorrhea and nocturnal cough, was greater in the tixocortol-neomycin group. Physical evaluation documented significant improvements in local superinfection with disappearance of mucopurulent nasal secretions and posterior drip. Locoregional outcome, evaluated on severity of infectious complications and antibiotic use, was also more favorable in the tixocortol-neomycin group. These results, together with the good clinical tolerance of the study drug, demonstrate the value of single-drug therapy with this local corticosteroid-neomycin combination in children with uncomplicated acute rhinopharyngitis. They confirm that local administration of corticosteroids to combat inflammatory phenomena is useful not only in the well-recognized lower respiratory tract indications (asthma, respiratory syncitial virus infections) but also in nasal diseases (rhinitis).

Administration, Intranasal↗