Archives francaises de pediatrie最新文献_第2页

[Aptitude to physical effort in children with operated tetralogy of Fallot]. [法洛四联症患儿的体力活动能力]。

Archives francaises de pediatrie Pub Date : 1993-12-01

M Kabaker, A Bouchikhi, H Darwiche, A Azancot-Benisty, A Casasoprana

{"title":"[Aptitude to physical effort in children with operated tetralogy of Fallot].","authors":"M Kabaker, A Bouchikhi, H Darwiche, A Azancot-Benisty, A Casasoprana","doi":"","DOIUrl":"","url":null,"abstract":"Background: Long-term follow-up of patients after total surgical correction of tetralogy of Fallot indicates that they have a smaller working capacity than controls, with an increased incidence of late onset complete heart block and sudden death. These abnormalities may be less frequent when surgery is undertaken at an early age.Population and methods: A cardio-pulmonary stress test was performed on 18 patients aged 8 to 20 years who had undergone correction of tetralogy of Fallot when they were 3 months to 7 years (mean age: 3 years). The basic ventilatory tests, exercise ventilatory tests and gas exchange were also performed.Results: The cardiopulmonary stress test was maximal in 16 of the 18 cases. No stress-induced PVCs, or chronotropic insufficiency was found despite a basic long PR interval in 4 cases. A restrictive ventilatory syndrome was seen in 4 cases with low respiratory reserve at exercise (defined as the ratio between maximal observed ventilation and maximal theoretical ventilation, VEMS x 40). The respiratory function was normal in 14 cases with an aerobic capacity of over 40 ml/kg/min.Conclusion: Patients with a normal chronotropic function and preserved aerobic capacity show no post-operative restriction or cardiac of pulmonary exercise capacity.","PeriodicalId":8169,"journal":{"name":"Archives francaises de pediatrie","volume":"50 10","pages":"867-70"},"PeriodicalIF":0.0,"publicationDate":"1993-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"19047397","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

[Radiological case of the month. Hydatid cyst of the lung]. [本月放射学病例。肺包虫病[]。

Archives francaises de pediatrie Pub Date : 1993-12-01

L Michaud, D Turck, M Ribet, M Rémy-Jardin, E Dutoit, J P Farriaux

引用次数: 0

[Nucleotides in the diet of infants during the first months of life]. [生命最初几个月婴儿饮食中的核苷酸]。

Archives francaises de pediatrie Pub Date : 1993-12-01

引用次数: 0

[An uncommon site of malignant germ cell tumor secreting alpha fetoprotein: the posterior mediastinum]. [一种罕见的分泌甲胎蛋白的恶性生殖细胞肿瘤:后纵隔]。

Archives francaises de pediatrie Pub Date : 1993-12-01

C Thomas, C Kalifa, D Couannet, C Bayle, A Pierre-Kahn

{"title":"[An uncommon site of malignant germ cell tumor secreting alpha fetoprotein: the posterior mediastinum].","authors":"C Thomas, C Kalifa, D Couannet, C Bayle, A Pierre-Kahn","doi":"","DOIUrl":"","url":null,"abstract":"Background: While germ cell tumors generally occur in the gonads, they may also appear at other sites, from the sacrococcygeum area to the central nervous system. This report describes a case of such a tumor in the posterior mediastinum that developed intraspinally in a dumb-bell fashion.Case report: A 2 1/2 year-old girl was admitted for abdominal tenderness, gait disturbance and fever. Clinical examination showed spastic paraparesis and bladder dysfunction. Thoracic X-rays showed a left postero-superior mediastinal mass with rib erosion. MRI showed that this mass had developed intraspinally between the intervertebral foramina and caused spinal cord compression at T4, T5, T6. The tumor was not calcified. Surgical resection via laminectomy was performed in emergency, but the T5 root had to be excised. Pathologic examination showed histologic features of yolk sac carcinoma; the serum alpha-foetoprotein was elevated (12, 400 IU/ml). The patient was given chemotherapy for 6 months and is well 2 years later.Conclusion: Germ cell tumors may appear in unusual sites. They can be identified by measuring biological markers, and this avoid primary surgery.","PeriodicalId":8169,"journal":{"name":"Archives francaises de pediatrie","volume":"50 10","pages":"879-81"},"PeriodicalIF":0.0,"publicationDate":"1993-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"18529427","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

[Articular manifestations in anguilluliasis. A case]. 鳗纹病的关节表现。一个案例)。

Archives francaises de pediatrie Pub Date : 1993-12-01

A Ayadi, M Koubaa, I Ammar, F Makni, H Hachicha, A Triki

引用次数: 0

[Biotinidase deficiency. Progressive encephalopathy curable with biotin]. [Biotinidase缺乏症。进行性脑病可用生物素治疗]。

Archives francaises de pediatrie Pub Date : 1993-12-01

B Héron, A Gautier, O Dulac, G Ponsot

{"title":"[Biotinidase deficiency. Progressive encephalopathy curable with biotin].","authors":"B Héron, A Gautier, O Dulac, G Ponsot","doi":"","DOIUrl":"","url":null,"abstract":"Background: Multiple carboxylase deficiency is a rare cause of progressive encephalopathy. There are 2 forms: the neonatal-onset form of holocarboxylase synthetase deficiency and the late-onset form of biotinidase deficiency. This report describes a case of biotinidase deficiency.Case report: A boy began to have repeated convulsions at the age of 3 months. The brain CT-scan and MRI were normal and the patient was given valproic acid and carbamazepine. Progressive neurological degradation was noted from the 4th month of life and myoclonic seizures began 2 months later. At admission, the patient had seizures, myoclonic fits and hypotonia. He had a skin rash but no alopecia. Biochemical investigation showed lactic acidosis, mainly in the CSF, moderate hyperammonemia and hyperalaninemia. Chromatography of organic acids showed several abnormal peaks suggesting biotinidase deficiency. The patient was given biotin (20 mg/day) orally. This treatment produced a pronounced, rapid, clinical and biochemical improvement and antiepileptic drugs were discontinued. There was no developmental delay at the age of 18 months.Conclusion: The clinical findings of neurologic abnormalities and dermatological signs led to the diagnosis of a metabolic disease that, fortunately, can be treated. This disease could benefit from a mass neonatal screening program.","PeriodicalId":8169,"journal":{"name":"Archives francaises de pediatrie","volume":"50 10","pages":"875-8"},"PeriodicalIF":0.0,"publicationDate":"1993-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"19047399","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

[Recurrent respiratory distress and idiopathic subglottic stenosis: what treatment?]. 复发性呼吸窘迫和特发性声门下狭窄:怎样治疗?

Archives francaises de pediatrie Pub Date : 1993-12-01

E Bodart, G de Bilderling, G Lawson, A Mayné, M Remacle

引用次数: 0

[Lethal osteogenesis imperfecta in a Congolese newborn infant]. [刚果新生儿致死性成骨不全]。

Archives francaises de pediatrie Pub Date : 1993-12-01

G Moyen, J L Nkoua, M Pongui, A M Mafouta, S Nzingoula

引用次数: 0

[Robinow's syndrome associated with deafness]. [与耳聋相关的罗比诺综合症]。

Archives francaises de pediatrie Pub Date : 1993-12-01

A Samoud, K Menif, M Boulaares, M F Ben Dridi