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Biomedical subjects

F Varlet

Publications and source records attributed to F Varlet.

28 records · Page 2Linked to original sources
PubMed1990

[2,8-dihydroxyadenine lithiasis. 2 new pediatric cases of an unknown metabolic deficit. The use of extracorporal lithotripsy].

Inherited adenine phosphoribosyltransferase (APRT) has a recessive transmission. When it is very important, adenine can't be restored into nucleic acids pool and will changed into 2,8-dihydroxyadenine (2,8-DHA) by xanthine oxidase. To date in all countries but Japan, 2,8-DHA urolithiasis is observed only into homozygotic subjects with complete APRT deficiency Commonly, its onset is observed in childhood often dramatically. The authors report two new pediatric cases into new french families. First a 8 years old boy with spontaneous elimination of two lithiasis after right lumbar pain. Secondly an infant (nineteen months) who has presented an acute renal failure with anuria. Bilateral lithiasis included into pyelourectal junctions have been pulled out by bilateral surgical pyelotomy. In each case, lithiasis were radiolucent and diagnosis made by ultrasonography. The uric acid metabolism was normal and it is the infra red spectrophotometric study of stones that had recognised the 2,8-DHA component. In the second case, bilateral residual lithiasis have been broken by piezoelectric extra-corporeal lithotripsy with good tolerance and favorable result. The two children received preventive treatment. After 36 and 19 months they have no recurrence. In the literature, the frequency of 2,8-DHA lithiasis is very more low than the theoretical of homozygotics in population (1/100,000). The common confusion with uric lithiasis is one possible explanation. So spectrophotometric study of radiolucent stones was meant to be realised when uric metabolism is not disturbed.(ABSTRACT TRUNCATED AT 250 WORDS)

Adenine↗
PubMed1989

[Isolated congenital stenoses of the extrahepatic bile ducts. Review of the literature apropos of a new case].

The authors describe a case of congenital stenosis of the common hepatic duct, in ten-year-old girl, with jaundice for two months. Ultrasonography showed an intra and extrahepatic duct dilatation. The operative cholangiogram demonstrated severe stenosis at the end of the common hepatic duct. A Roux-en-Y anastomosis of the jejunum to this duct relieved the obstruction. Histopathological examination of the removed stricture showed non inflammatory fibrous structures. The congenital biliary strictures are exceptional. The review of the literature allowed to collect nine other cases, which symptoms began before fifteen years old. Only four were treated in childhood. The arguments for a malformative origin of the stricture are the patient's age, the seat of the stenosis and its histological structure. Several physiopathological theories allowed to explain the usually delayed beginning of first symptoms: distortion of the stenosis by gradual distension, biliary sludge, lithiasis and infection. The preoperative diagnosis of the stricture may now be made by transhepatic or endoscopic cholangiography, but before to assert its congenital feature, it must eliminate many intrinsic or extrinsic causes: post operative, traumatic, inflammatory and tumoral. The Roux-en-Y hepaticojejunostomy is the usual treatment.

Child↗
PubMed1989

[Multiple congenital esophago-tracheal fistulas (excluding atresia of the esophagus). Review of the literature, apropos of a new case].

Congenital tracheoesophageal fistulas (CTOF) without esophageal atresia are uncommon and exceptional when multiple. Authors report a new personal case treated in two times. Others eight cases in literature are studied. Only one pre-operative diagnosis was made. Two old no-operated cases were discovered at autopsy. For five, no clinical modification was noted after the first CTOF surgical care. In them two no-reoperated died and the second fistula was seen after. For the others, the same radiological or endoscopic exams that have not marked the second fistula were positive after surgery. No explanation was done. For a better result, systematic complete investigation of trachea and esophagus are necessary even if first fistula is diagnosed. Luckily per-operative discovery of second fistula (one case) do not allowed initial extensive surgery. To support a second congenital tracheoesophageal fistula after a surgical time, many criterias are necessary: 1. a undoubted fistula recognised during the first intervention; 2. recurrent symptoms after surgery with no free interval; 3. identification by radiology or endoscopy of a new tracheoesophageal shunt in other place that the first; 4. its surgical discovery in no previous dissected area. In all cases where fistulas are described, the aspect is not the same: the upper fistula is a "H" type and the lower is shorter and larger ("X" type). To note a case of three simultaneaous fistulas in the same patient.

Diseases in Twins↗
PubMed1989

[2,8-dihydroxyadenine lithiasis. 2 new pediatric cases of this misdiagnosed metabolic abnormality. The value of extracorporeal lithotripsy].

Inherited adenine phosphoribosyltransferase (APRT) has a recessive transmission. When it is very important, adenine can't be restored into nucleic acids pool and will changed into 2,8-dihydroxyadenine (2,8-DHA) by xanthine oxydase. To date in all countries but Japan, 2,8-DHA urolithiasis is observed only into homozygotic subjects with complete APRT deficiency. Commonly, its onset is observed in childhood often dramatically. The authors report two new pediatric cases in two new french families. First a 8 year old boy with spontaneous elimination of two lithiasis after right lumbar pain. Secondly an infant (nineteen months) who has presented an acute renal failure with anuria. Bilateral lithiasis incluted into pyeloureteral junctions have been pulled out by bilateral surgical pyelotomy. In each case, lithiasis were radioluscent and diagnosis made by ultrasonography. The uric acid metabolism was normal and it is the infra red spectrophotometric study of stones that had recogniseed the 2,8-DHA component. In the second case, bilateral residual lithiasis have been broken by piezoelectric extracorporeal lithotrypsy with good tolerance and favorable result. The two children received permanent preventive treatment. After 36 and 19 months they have no recurrence. In the literature, the frequency of 2,8-DHA lithiasis is very more low than the theorical incidence of homozygotics in population (1/100,000). The common confusion with uric lithiasis is one possible explanation. So spectorophotometric study of radioluscent stones was meant to be realised when uric metabolism is not disturbed. Prevention associates alimentary diet without purins and permanent treatment by allopurinol (10 mg/kg/day in a child). Not used to date, piezo-electric extracorporeal lithotrypsy seems to take a place for treatment of initial, residual or recurrent 2,8-DHA lithiasis like for our young patient.(ABSTRACT TRUNCATED AT 250 WORDS)

Adenine↗
PubMed1985

[The calcium bile syndrome in children].

The "limy bile" is uncommon in adults and very exceptional in childhood. The authors report a new case of a 7 years old girl. The review of the literature since Churchman's description (1911) allowed to collect eleven other cases of children, less than fifteen old. The usual sexual distribution in biliary diseases with female predominance is not true for them: eight boys for four girls. The youngest was three years and six months old. All but one have presented mainly right upper abdominal pain and vomiting. The diagnosis was made by an abdominal plain film showing a spontaneous visualization of the gallbladder which contained a material made of carbonate of calcium. One was an operative detection. All presented a stone of neck of gallbladder or cystic duct. The origin of "limy bile" is unknown. Nine children have been operated (cholecystectomy) with good result. Spontaneous disappearance of "limy bile" was noted in three cases. It was marked by acute abdominal pain, inconstant jaundice or pancreatic reaction. No surgical care was brought with nor further problems or recurrence.

Adolescent↗
PubMedDate not supplied

Epstein-Barr virus--positive undifferentiated thymic carcinoma in a 12-year-old white girl.

Thymic epithelial malignant diseases are extremely rare in children. The authors report a 12-year-old white girl admitted for a polymetastatic tumor of the anterior mediastinum. Tumor proliferation was typical of an undifferentiated thymic carcinoma. A close link between Epstein-Barr virus (EBV) and the tumor was established by a high titer of anti-VCA IgA and the presence of EBV RNA and DNA in the tumor. In addition, monoclonal viral episomes were present in tumor cells, indicating that EBV infection was an early event in the oncogenic process. The patient died despite resection, irradiation, and chemotherapy.

Carcinoma↗
PubMedDate not supplied

[Priapism in the course of generalized atopic dermatitis].

BACKGROUND: Priapism is a pathologically prolonged and painful penile erection, not resulting in ejaculation. Causes include certain oral medication, perineal trauma, thrombo-embolic process or primary priapism. We report a case of priapism secondary to diffuse general atopic dermatitis. CASE REPORT: An 11 year-old child, with atopic dermatitis, presented over the last 36 hours an irreduced painful penile erection. Other than priapism, he presented numerous scratched lesions on the body and the penis having developped over the last 2 months (treated by daily topical corticosteroid application) and consistent with atopic dermatitis exacerbation. Due to the urological emergency, a puncture of the corpora cavernosa was made, followed by an injection of etilephrine in the penis. Complete and definitive detumescence was obtained. Application of betamethasone healed the cutaneous lesions. The child was treated with ciclosporine for his atopic dermatitis with good results at 6 months. DISCUSSION: In our case, hypothesis of myeloid leukemia, sickle-cell disease and essential thrombocythemia were turned down. The young child did not exhibit any recent perineal trauma but the scratched lesions can be considered as microtraumas. The formation of an inflammatory oedema obstructed venous drainage of the penis and provoked priapism. We can not rule out the responsibility of topical corticosteroids in the formation of the priapism in this patient. Indeed, it was the only drug therapy prescribed, and since introduction of ciclosporine, there has been no relapse.

Anesthesia, General↗