Search PubMed⌕ Search

Biomedical subjects

P Cochat

Publications and source records attributed to P Cochat.

At least 163 records · Page 9Linked to original sources

[Nephropathy and Charcot-Marie-Tooth disease. A case report].

A 12 year-old girl with a family of Charcot-Marie-Tooth neuropathy was referred to us with mild proteinuria without hematuria or renal insufficiency; a renal biopsy specimen showed focal glomerulosclerosis. Two years later, proteinuria and renal function were quite stable and there was no arterial hypertension. Of 13 other cases in the literature, the outcome of renal involvement has the same profile:proteinuria at onset with or without microhematuria, sometimes with nephrotic syndrome; the prognosis is often poor, since 9 out of 13 patients have end-stage renal failure after 6 months to 17 years of follow-up. The pathological examination show focal glomerulosclerosis in most cases. Nerve deafness is uncommon in Charcot-Marie-Tooth disease but was present in 7 out of 13 patients with the nephritis. Such an association may be a variant of the dominant autosomal form of the disease, whose gene is located on chromosome 1.

Charcot-Marie-Tooth Disease↗

[Membranous glomerulonephritis in children: 20 cases].

Twenty cases of membranous glomerulonephritis have been diagnosed between 1978 and 1988 in children (13 girls, 7 boys) aged 4 to 15 years, observed for a 5.1 +/- 2.9 year period. The conditions of the diagnosis were: routine urinalysis in 10 cases, edema in eight, and the surveyance of a D-penicillamine treatment in two. All the patients had proteinuria (0.3 to 15 g/24 h) ranging to nephrotic syndrome in nine children. Microscopic hematuria was found in 16 children (80%). Elevated blood pressure was recorded in two cases at the time of diagnosis, and developed in two other cases during the follow-up. One child experienced renal failure at the onset of the disease. Most histological lesions consisted in stage II membranous glomerulonephritis. Immunofluorescence study (18 biopsies/20) always showed granulosus and intensive IgC deposits, associated with IgM and IgA deposits which were less marked; intensive extra-membranous C3 deposits were noted in 11 cases. As to the etiology, D-penicillamine was responsible for two cases (10%) and HBs antigen in one (5%); the nephropathy was considered as idiopathic in the 17 remaining cases (85%). Regarding the evolution: in eight cases (38%) proteinuria disappeared by 54 +/- 28 months; in 10 cases (55%), proteinuria persisted after 41 +/- 31 months; hematuria, which was present at onset, disappeared in most cases (13/17); in one case (5%), end-stage renal failure occurred within 3 years. The patient with initial renal failure has been last sight off.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Relapsing urinary ascites.

A 2-year-old boy was operated on for a left ureteral duplication with bilateral vesicoureteric reflux. Ten years later, he experienced two episodes of transient ascites following abdominal traumatism. A cystogram disclosed a peritoneal fistula. The fistulous orifice was localized by cystoscopy, and surgery was undertaken with success.

Ascites↗

A phase-II study of adoptive immunotherapy with continuous infusion of interleukin-2 in children with advanced neuroblastoma. A report on 11 cases.

Nine children with poor prognosis neuroblastoma have been treated by continuous infusion of IL-2 and autologous LAK cells, as described previously by West et al. in adult patients. Six patients were in relapse after high-dose chemotherapy and autologous BMT and three presented with primary refractory disease after conventional therapy. Although patients were very young (median age 6 years; average weight 17 kg), infusion of IL-2, cytapheresis and reinjection of LAK cells appeared feasible with the usual and transient complications observed with IL-2. Haematological toxicity, although reversible, was more important than usually described and due to the presence of bone-marrow metastases in 8 of the 9 patients. Life-threatening toxicity was observed in only one of the admission centres and was probably due to the rapid reinjection of a very large number of activated cells. Two patients presenting with very active disease after high-dose chemotherapy and autologous or allogeneic BMT received IL-2 alone, at 120 days and at 90 days after the graft. The reactivation of grade-II GVHD was the major complication in the patient treated after an allograft, whereas no BMT-related toxicity was observed in the patient treated after the autologous BMT. Immunological modifications induced by IL-2 were very different between these patients. As expected, a preferential outgrowth of NK cells with both NK and LAK activity was observed in the patient treated just after the autograft. In contrast, in the patient treated after an allograft and in the 9 patients in relapse, T lymphocytes remained the major mononuclear cell population with a very large excess of CD8+ T cells. All patients progressed after the first induction cycle with the exception of the only patient treated after autologous BMT who reached a very good partial remission with disappearance of the local tumor and bone metastases. Although very preliminary, these data clearly show that the efficacy of IL-2 largely depends on the patient's immunological status with the optimal effect being observed when IL-2 is given in the first few months following an autograft.

Bone Marrow Transplantation↗

[Congenital mega-urethra. Comments apropos of a case report].

Congenital megalourethra is a malformation of the male urethra with a major dilatation of its penile part without obstruction downwards. It is either due to hypoplasia ore agenesia of spongy tissue. Two types are described, scaphoide and fusiform types, according to the presence or the absence of erectile tissue. Megalourethra is a rare condition, and 55 cases were previously reported until 1987. A new case of this severe anomaly is reported and management discussed.

Humans↗

Systemic interleukin-2 therapy in children with progressive neuroblastoma after high dose chemotherapy and bone marrow transplantation.

Two children with active metastatic neuroblastoma after high dose chemotherapy and bone marrow transplantation (BMT) received a high dose continuous infusion of interleukin-2 (IL2) 120 days after an autologous BMT for patient 1 and 90 days after an allogeneic non T cell-depleted BMT for patient 2. Usual side effects of IL2 therapy were observed without life-threatening complications or any major hematological toxicity. The reactivation of graft-versus-host disease during IL2 infusion in patient 2 was the major BM-related complication but it improved with IL2 interruption and corticosteroids. IL2 induced a complete remission (9+ months) in patient 1 with the disappearance of bone metastases and local tumor but patient 2 progressed after cessation of therapy. Patient 1 presented with a large excess of circulating NK cells in the period after autologous BMT and IL2 induced a preferential outgrowth of this lymphocyte subset.

Adrenal Cortex Hormones↗

[Broncho-alveolar lavage by fibroscopy in immunodepressed children].

Thirty broncho-alveolar lavage (BAL) were performed in order to investigate 30 infectious episodes in immunocompromised children. Twenty patients were previously treated by high-dose chemotherapy and autologous bone marrow transplantation and 6 other patients by conventional methods. A specific etiologic diagnosis was obtained in 16 of 30 episodes (56%), 22 microorganisms were identified by BAL. The most frequently involved microorganism was Candida albicans and the other agents were as follows: 3 cytomegalovirus, 2 Pneumococcus, 2 Pneumocystis carinii, 1 Aspergillosis, 1 syncytial respiratory virus, 1 myxovirus, 1 Pseudomonas aeruginosa, 1 Mycoplasma pneumoniae, 1 Haemophilus influenzae and 1 Escherichia coli. In 5 cases, more than 2 agents were involved. This study emphasizes the diagnostic interest of BAL for infectious diseases of the immunocompromised child. BAL appears to be a non invasive, rapid and reproducible method, and a useful therapeutic approach in the treatment of infectious episodes occurring in grafted children where several microorganisms could be involved at the same time.

Bacterial Infections↗

[Acute Borrelia burgdorferi encephalitis (Lyme disease) in an Algerian child].

A case of acute hemorrhagic leukoencephalitis is described in an Algerian child with renal insufficiency that had been diagnosed at the terminal stage. Convulsions, hemiplegia and consciousness disorders pointed to a necrotizing viral or bacterial form of encephalitis, but the definitive diagnosis was based on the detection of IgM and IgG specific serum antibodies raised against Borrelia burgdorferi.

Acute Disease↗

[Berger's disease or primary IgA nephropathy in children].

Primary IgA mesangial nephropathy was first described in adults by Berger, and has been increasingly recognized in children. IgA nephropathy is a frequent type of glomerulonephritis in 3 to 15 year-old children in France. Clinical features and outcome have been defined and the progression to renal failure is possible. The pathogeny of IgA nephropathy remains unclear and is under multifactorial control and, at present, no satisfactory specific treatment is available.

Acute Kidney Injury↗

[Enuresis and common bladder disorders in children].

Enuresis affects 5 to 10% of primary-school age children. Nocturnal enuresis, or bedwetting, is often familial and boys are mainly concerned; daytime micturitions are normal, without urine loss or urinary tract infection. Hygienic rules associated with desmopressin or, in some cases, tricyclic antidepressant agents, alarm procedures or psychotherapy, result in a 70% success rate after 1 year. Bladder instability consists of diurnal and nocturnal disturbances, mainly in girls with recurrent urinary tract infections; affected children experience pollakiuria, urine loss and voiding emergencies. Urodynamic assessment of daytime enuresis is of major interest, mainly when dysuria is present. The treatment of non complicated bladder instability needs reeducation, i.e. biofeedback and/or administration of oxybutynin chlorhydrate.

Child↗

[Familial infantile nephrotic syndrome with ocular abnormalities].

Two siblings born from consanguineous parents experienced infantile nephrotic syndrome with ocular and neurological abnormalities and a micropenis in the boy; both patients died before age 1. The opportunity to perform successively a renal biopsy and a two-step binephrectomy permitted a good histological follow-up. The lesions were characterized by mesangial involvement, followed by an extensive extracapillary proliferation and tubular dilatations with a high mitotic activity of the epithelium with anisocaryosis; the main features consisted in major ultrastructural changes of the glomerular basement membrane which were more significant and different from those previously described in the diffuse mesangial sclerosis. These 2 cases may either constitute a new entity or an extreme form of diffuse mesangial sclerosis, supported by recessive autosomal inheritance.

Basement Membrane↗

[Hemoperfusion on charcoal and hemodialysis in acute poisoning caused by methotrexate].

Acute methotrexate intoxication occurred in 4 patients despite adequate alkaline hyperhydration and classical folinic acid rescue. Three of these patients had no previous risk factor. Charcoal haemoperfusion with haemodialysis was promptly instituted and the methotrexate blood levels rapidly decreased, avoiding further renal damage and multisystemic involvement. Chemotherapy could subsequently be performed in 3 of the 4 patients without delay and toxicity. Charcoal haemofiltration appears to be an excellent treatment of methotrexate intoxication.

Aged↗

[Nephrotic syndrome after a medusa sting].

A steroid-responsive nephrotic syndrome after medusa prick is not common. It occurred in a 15 year-old girl: this case allows to discuss the role of allergy in such a situation.

Adolescent↗

[Neurologic manifestations of arterial hypertension in children].

Neurological complications of arterial hypertension are analyzed in 31 children (mean age = 9 years). All patients presented a renal or renovascular disease (acute nephritis + hypoplastic dysplasia , transplantation = 58%) for which malignant hypertension was the first symptom in 16%. The mean +/- SD initial blood pressure was 189 +/- 33/113 +/- 25 mm Hg and was preceded by previous symptoms in 1 patient out of 6. Neurological abnormalities consisted in seizures (48%), acute intracranial hypertension (39%), cranial palsy (23%), coma (19%), hemiplegia/paresia (16%), retinal changes (6%) or aphasia (6%). The EEG was abnormal in 50% of the patients, sometimes showing permanent paroxysmal activity. Neuroradiologic investigations revealed hemorrhagic and/or ischemic lesions in 1/5 patients. On follow-up, hypertension disappeared in 41% of the children; a decrease in renal function was noted in 56% of the patients at the last examination; neurological sequellae were present in 40% (EEG anomalies +/- epilepsy, motor deficit, retinal changes, psychomotor delay, cranial palsy) and 1 patient died. The morbidity of malignant hypertension stresses the importance of early diagnosis and treatment (calcium channel blockers) when its prevention is not possible.

Adolescent↗

[Suppurated pneumococcal meningitis in infants and children: complications and prognostic factors].

This paper investigates 21 patients (16 infants and 5 children) with Pneumococcal meningitis, 15 of which presented with a severe form. Intracranial pressure (IP) monitoring was performed in 7 patients who all had severe (4) or mild (3) intracranial hypertension (IH). Twelve children had a favourable outcome, 7 had sequelae (3 severe) and 2 died from coning, one before monitoring, the second after exhibiting the highest IP and the lowest cerebral perfusion pressure of the series. The 3 children with severe sequelae had a severe form (2) or a mild but prolonged form (1) of IH. The 4 patients who recovered with moderate sequelae presented severe (1), mild IH (1) or were not monitored (2). There were 14 cases with neurological complications. In 8 patients, this seemed to be related to cerebral oedema and IH; coning caused the death of 2 of them; the 6 others exhibited lesions of cerebral oedema on CT-scan, isolated or associated with cerebral infarction or subdural effusion. In the 6 others patients, IP monitoring was not performed; there was no evidence of cerebral oedema on CT-scan; 2 exhibited cerebral infarction and 3 a moderate ventricular dilatation; 2 had hemodynamic problems from acute pneumococcemia in one case and neurovegetative disturbances associated with cerebral infarction in the second. Pneumococcus meningitis remains a severe disease. The prognosis of severe forms can be improved by IP monitoring since IH seems to be the most frequent mechanism of complication.

Brain Edema↗