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Biomedical subjects

M Coquet

Publications and source records attributed to M Coquet.

At least 37 records · Page 2Linked to original sources
PubMed1998

Functional outcome and specific complications of gastrocystoplasty for failed bladder exstrophy closure.

PURPOSE: The encouraging initial results of gastrocystoplasty led us to perform it for failed bladder exstrophy closure. We assess the functional outcome of the augmented bladder and evaluate complications related directly to use of the stomach in this specific group of children. MATERIALS AND METHODS: We performed gastrocystoplasty in 22 children an average of 9.5 years old with a small, poorly compliant bladder after staged reconstruction of bladder exstrophy failed. Followup ranged from 6 months to 6 years (mean 3 years). RESULTS: Complete urinary continence was achieved in 14 children (64%). Voiding via the urethra was possible in 13 patients (60%) but post-voiding residual urine was significant in 12. Bladder capacity increased from a mean of 77 to 270 ml. Bladder capacity decreased during followup in 3 children, requiring repeat augmentation. Six children had isolated dysuria and 2 had dysuria with hematuria. Perforation of the gastric patch and a bleeding gastric ulcer occurred in 1 patient each. CONCLUSIONS: The disadvantages of gastrocystoplasty outnumber its advantages after failed bladder exstrophy closure. Urethral sensation makes dysuria a major discomfort. Safety is not optimal, since perforation may occur. Voiding is not efficient because gastrocystoplasty provides continence only when it is associated with intermittent catheterization. Bladder capacity is insufficiently augmented and inconsistent during followup. We believe that the use of gastrocystoplasty in cases of failed bladder exstrophy closure should be reconsidered.

Adolescent↗
PubMed1997

[Persistent urination disorders after treatment of posterior urethral valve: incidence and semiology].

Micturition disorders were studied retrospectively in a series of 165 children over a period of 15 years. Among patients without lesions of the upper tract at the time of diagnosis (group A, n = 131), 18 (14%) had persistent nocturnal and diurnal enuresis: one urethral stenosis and six vesicoureteric reflux required surgery; three experienced persistent pollakiuria and enuresis. Among patients with upper urinary tract damage at the time of diagnosis (group B, n = 34), seven presented with recurrent urinary tract infection, five with nocturnal and diurnal enuresis and three with urolithiasis. In the long term, only 52% of them had normal renal function and two were successfully transplanted. Micturition disorders following treatment of posterior urethral valves are frequent and usually related to the so-called valve bladder syndrome. Iatrogenic complications and mortality rate have dramatically decreased during the recent years but long-term renal function impairment remains the most critical problem.

Child↗
PubMed1996

[Pneumoblastoma in children. A clinical case and review of the literature].

Pulmonary blastoma is a rare malignant tumor. A new case is reported in a 3 years 6 month-old girl. An apparent clinical remission was first obtained after a surgical treatment followed by a conventional chemotherapy during six months. Afterwards the persistence of a microscopic residual pulmonary disease lead us to deliver successfully an intensive chemotherapy followed by autologous peripheral blood stem cells reinjection. The child remains disease free 12 months after graft. Problems set by the histogenesis of this tumor, its unspecific clinical and paraclinical features and the role of conventional and intensive chemotherapy followed by autologous bone marrow transplantation are discussed on the basis of a review of 50 cases in the literature.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1996

[Muscular biopsy].

Muscle biopsy is a simple procedure of great diagnostic interest when correct processing is obtained. Specimens for molecular genetics are very important. Paraffin embedded material is not sufficient in most of the cases except in some inflammatory processes. Histochemical techniques on frozen material are of major interest. Immunohistochemistry and electron microscopy can also be used. The aim of this paper is to describe the procedure of the muscle biopsy, the various techniques and the possible artefacts.

Biopsy↗
PubMed1995

[Localized muscular hypertrophy in a familial form of sarcoidosis].

Muscular hypertrophy, rarely reported in muscular sarcoidosis, is usually associated with the myositic, myopathic and nodular types. We report a case with an isolated localised muscular hypertrophy simulating a tumor formation developed after repetitive efforts, in a familial context of sarcoidosis. MRI shows muscular high intensity lesions in the thighs and calves, and after treatment, central areas of low intensity signal. Muscular biopsy confirms diagnosis of sarcoidosis. Gallium-67 scintigraphy shows systemic extension of disease. This case highlights the usefulness of recent imaging techniques in diagnostic strategy and follow up.

Adult↗
PubMed1995

Gastrocystoplasty in the treatment of bladder exstrophy.

A wedge-shaped segment of stomach based on the right gastroepiploic artery was used for bladder augmentation in 11 patients. Initial diagnosis in these 11 patients was bladder exstrophy. Indications for the use of stomach in bladder reconstruction were important bilateral upper tract deterioration in 10 patients, dederivation in 1. A continent appendicostomy (Mitrofanoff) has been performed in all patients. In post-operative follow-up (average 24 months), all patients have stable upper tract X-rays and stable or improved renal function. All patients require intermittent clean catheterization, 8 are totally continent, 2 are partially continent, 1 patient is still incontinent. No serious digestive problem was encountered. The increase in bladder capacity was 300 to 500% after 6 months. Mucus production is reduced relative to other intestinal segments and the patients require no bladder irrigation. The authors recommend the use of stomach for urinary tract reconstruction in compromised patients.

Adolescent↗
PubMed1995

New regimen for interpleural block in children.

A new regimen for postoperative analgesia after thoracic surgery is proposed. Eight children received an interpleural infusion using bupivacaine 0.1% in a regimen from 0.5 ml.kg-1.h-1 up to 1 ml.kg-1.h-1, for 48 h according to the pain scores. The plasma levels after 24 h and 48 h were measured as well as the pleural level and in two patients the free fraction of plasma bupivacaine and the plasma PPX (a metabolite of bupivacaine) and one patient the orosomucoid (main plasma protein involved in bupivacaine protein binding) were also measured pre and postoperatively. The results shows the safety of such a regimen, for two days of postoperative analgesia.

Analgesia↗
PubMed1994

Skewed inactivation of an X chromosome deleted at the dystrophin gene in an asymptomatic mother and her affected daughter.

A girl with severe Becker muscular dystrophy and apparently normal chromosomes had a heterozygous deletion for exons 51, 52, and 53 of the dystrophin gene. This deletion was transmitted by her mother, who was unaffected. To differentiate the normal and the deleted X chromosomes, fluorescence in situ hybridization (FISH) was applied to metaphase chromosomes, using probes for both exons 51 and 52, which are only 388 and 113 base pairs long, respectively. FISH signals were observed in one or both chromatids of one chromosome, but never on both chromosomes, suggesting the lack of hybridization on the deleted X chromosome. Using 5-bromodeoxyuridine incorporation to differentiate the late (inactive) and the early replicating (active) X chromosomes, 77% of the signals were observed on the active X chromosomes in the mother. This percentage was only 18% in the daughter, suggesting that skewed inactivation of the X chromosomes was responsible for the phenotypic differences.

Adolescent↗
PubMed1993

Fatal neonatal liver failure and mitochondrial cytopathy (oxidative phosphorylation deficiency): a light and electron microscopic study of the liver.

Mitochondrial cytopathies are multisystemic disorders of extremely variable expression due to a deficiency in oxidative phosphorylation. Cases have recently been reported in which fatal liver failure with neonatal onset was the major clinical and biochemical syndrome. In this series we reviewed the liver histology of 10 such patients who died in the first weeks of life (from 3 days to 6 mo). In six cases the diagnosis was confirmed by study of the mitochondrial respiratory chain in the muscle, liver or both; in the other four, appropriate tests were not available for diagnosis but symptoms were identical and all other diagnoses were ruled out. In all 10 cases we noted significant steatosis, mostly microvesicular; widespread hepatocytic, canalicular and bile duct cholestasis with bile thrombi and cholangiolar proliferation; and different degrees of hepatosiderosis and glycogen depletion. Fibrosis took varying forms: perisinusoidal, periportal with the formation of septa, even precirrhosis. In the two cases of infants who died, one at 5 and one 6 mo, micronodular cirrhosis was also present. Mitochondria, either densely or loosely packed, were abnormal-pleiomorphic with few or no cristae and a granular fluffy matrix. Dense, large granules were seen in two cases. The association of neonatal liver failure and hyperlactacidemia should lead to immediate examination of the respiratory chain. The expression of this mitochondrial cytopathy can be lethal, associated with severe liver damage due to the deficiency in oxidative phosphorylation.

Female↗
PubMed1993

Ventricular arrhythmia revealing mitochondrial myopathy in a 69-year-old woman.

We report a case of mitochondrial myopathy (MM), assessed by histological and biochemical studies. This illness was diagnosed in a 69-year-old patient with myocardiopathy revealed by ventricular arrhythmias. The originality of this case lies in the patient's age, the mode of onset and the biochemical features (i.e. normal mitochondrial enzymatic complexes but very low respiration when using glutamate as a substrate).

Aged↗
PubMed1993

[Limb-girdle syndrome. A study of 46 cases].

We report a series of 46 patients (32 women and 14 men) with limb-girdle syndrome. After reappraisal, another diagnosis was made in 10 of them. Becker's muscular dystrophy was the most frequent cause among men (near 50 p. 100). A Duchenne muscular dystrophy manifesting carrier was discovered among 13 reevaluated women. Among the 36 cases (29 women and 7 men) without any defined etiology, 29 were without any other known familial history. Fifteen of these women had similar clinical findings: incipient weakness in the pelvic girdle and onset of symptoms most often in the forties. In these cases serum creatine kinase activity was normal or slightly elevated, and muscle biopsy showed non-specific patterns. "Late onset muscular dystrophy in females" should be reevaluated.

Adult↗
PubMed1993

[Regressive mitochondrial abnormalities in the muscular biopsy of a bicycling champion].

The onset of severe muscle cramps in the course of road racing led a champion to undergo a muscle biopsy. Fuscinophilic deposits were detected around muscle cell fibers together with a few PAS positive deposits. These findings were related to the accumulation of abnormal mitochondria and glycogen granules. The subsequent study of the mitochondrial respiratory chain did not reveal any modification and so ruled out a genuine mitochondrial myopathy. Such mitochondrial modifications are probably related to the onset of muscle cramps, but no metabolic disturbance could be found to account for these two phenomena.

Adult↗
PubMed1992

Fatal neonatal liver failure and mitochondrial cytopathy: an observation with antenatal ascites.

Mitochondrial cytopathies are multisystemic diseases of extremely variable expression caused by a deficiency in oxidative phosphorylation. Only five cases of neonatal liver failure in the context of mitochondrial cytopathy have been reported, with incomplete morphological data of the liver in three. In the case presented here, ascites had been diagnosed prenatally and liver failure was particularly severe (factor V less than 15% with fatal coma the fourth day). Histologically there were incomplete cirrhosis, microvesicular steatosis, major canalicular cholestasis with proliferative neocholangioles, and bile duct thrombi. There were also some iron pigments in the periportal area and partial glycogen depletion. By electron microscopy, mitochondria in numerous hepatocytes appeared abnormal with occasional cristae in a fluffy matrix, some containing dense inclusions. Study of respiratory chain activity showed a defect in cytochrome c oxidase (complex IV), revealed by oxygraphic measurement on fresh muscle biopsy and confirmed by spectrophotometric enzymatic assays performed on muscle and liver homogenates. The association of neonatal liver failure with hyperlactacidemia warrants investigation into a deficiency in oxidative phosphorylation.

Ascites↗
PubMed1992

Surgical management of duplex ureteroceles.

We reviewed the cases of 95 children with duplex ureteroceles treated in this department over an 18-year period. There were 101 ureteroceles (6 bilateral). Diagnosis and treatment were analyzed. Special attention was paid to newborns screened in utero. We always strove to preserve functional renal tissue whenever possible. In keeping with this goal, three surgical techniques were used: (1) upper pole heminephrectomy; (2) ureterocele excision, bladder neck reconstruction, and ureter reimplantation with or without cutaneous ureterostomy of the upper pole ureter; and (3) endoscopic ureterocele incision. Follow-up studies using x-ray and radionuclide imaging demonstrated satisfactory renal function in 86.6% of patients. These findings support a conservative approach to ureteroceles using endoscopic ureterocele incision as the primary treatment. Lower urinary tract reconstruction may be associated in cases involving urinary tract infection, obstruction or incontinence. Upper pole heminephrectomy should be performed only after functional evaluation following ureterocele incision or cutaneous ureterostomy.

Adolescent↗
PubMed1992

Mitochondrial myopathy studies on permeabilized muscle fibers.

Respiratory parameters of skeletal muscle were determined in permeabilized muscle fibers by adapting a technique described by Veksler et al. for cardiac fibers (Biochim Biophys Acta, 892:191-196, 1987). This method consists of the permeabilization of muscle fibers by saponin by allowing respiratory substrates and inhibitors to reach the mitochondria. In this way, the mitochondria may be studied inside the fibers as if they were isolated. We have verified, using various techniques, that the mitochondria remain intact during this procedure. This method has been applied to the study of six newborn infants for whom a diagnosis of a mitochondrial defect was suspected. In all cases, the defect was to be found on the permeabilized fibers, and this was confirmed by an enzymatic study. The advantage of this new method, associated with the measurement of the enzymatic activities on a crude homogenate, is to enable a simple and rapid diagnosis on a small amount of sample without damaging the mitochondria during the isolation procedure.

Female↗