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Biomedical subjects

S Ben Becher

Publications and source records attributed to S Ben Becher.

34 records · Page 2Linked to original sources
PubMed1991

[Recurrent multifocal periostosis in children. Report of a familial form].

Recurrent multifocal periostosis of childhood is an infrequent disease. All cases reported to date have been sporadic. A familial form affecting three siblings is reported herein. Clinical and biologic features were similar to those described in the previously reported cases. This case is of particular interest for two reasons: 1) the pattern of familial clustering suggests autosomal recessive transmission; 2) repeated CT scans were performed in these patients (but not in previously reported cases) and demonstrated early involvement of the three bone layers, suggesting panostosis rather than periostosis.

Bone Diseases↗
PubMed1991

[Budd-Chiari syndrome in children. Apropos of 7 cases].

Seven cases of Budd-Chiari syndrome are reported in children. The mode of onset was fulminant in one case with rapidly lethal liver failure, acute in 5 cases with rapid appearance of hepatomegaly and ascites and insidious in one case, with isolated hepatomegaly. Hepatomegaly, which is a constant sign, was present in the 7 patients. Ascites and collateral venous circulation were present in 6, splenomegaly in 2 and moderate jaundice in one only. Liver function tests, deeply abnormal in the patient with fulminant liver failure, was only slightly abnormal in the 6 others. Diagnosis was corroborated by ultrasonography, cavography, hepatic veins angiography and liver biopsy in 6 patients and by post mortem examination in the 7th. Etiologic investigations did not allow finding the cause of Budd-Chiari syndrome. However, this series can be distinguished by associated total villous atrophy in 3 cases, psoriasis in one, hepatitis B in one, hepatitis A and intestinal giardiasis in one. Portasystemic shunts were performed in 3 patients. One died in the immediate postoperative period, the 2 others are presently in good health with a 5 and 6 1/2 year-follow-up. One patient died rapidly from fulminant liver failure. Another, untreated, died 16 years after the onset of the disease, from an unknown cause. Two patients are lost to follow-up.

Budd-Chiari Syndrome↗
PubMed1990

[Waldenström's hyperglobulinemic purpura. Apropos of a case].

The authors report the case of a thirteen-year-old girl who developed clinical manifestations suggestive of Schönlein-Henoch purpura and in whom biologic tests outruled this condition and led to the diagnosis of Waldenström hyperglobulinemic purpura. Investigations illustrated the immunologic nature of this disease, outruled an underlying disease and established the diagnosis of primary hyperglobulinemic purpura.

Adolescent↗
PubMed1990

[Familial syndrome combining short stature, microcephaly, mental deficiency, seizures, hearing loss, and skin lesions. A new syndrome].

We report the observations of three sisters with the same autosomal recessive syndrome characterized by growth retardation, microcephaly, mental deficiency, seizures, sensorineural hearing loss, and skin lesions. The congenital nature of these symptoms was confirmed by their high prevalence among other family members. This syndrome is one of the many neurocutaneous syndromes and does not seem to fit any of the previously published descriptions.

Adolescent↗
PubMedDate not supplied

Childhood diabetic neuropathy: a clinical and electrophysiological study.

Clinical diabetic neuropathy in childhood is rare, but electrophysiological involvement of the peripheral nerve is more frequent. We assessed clinically and electrophysiologically the peripheral nervous system of 69 children and adolescents suffering from diabetes mellitus (DM). The mean age of the patients was 12.8 years and the mean age at onset of DM was 6.8 years with a mean disease duration of 6.3 years. Seven patients (10%) had clinical neuropathy of which ankle jerk reflex abolition was the most frequent sign. Twenty patients (29%) had a neurophysiological neuropathy prevalently affecting the lower limbs. Peripheral neuropathy was correlated with patient age, older age at onset, duration of DM, height and poor glycaemic control.

Action Potentials↗
PubMedDate not supplied

Abdominal tuberculosis in children. Report of 10 cases.

BACKGROUND: Abdominal tuberculosis is a rare location of this infection with a lot of diagnostic difficulties. The aim of this study was to review our experience of pediatric abdominal tuberculosis with that of the literature data. PATIENTS AND METHODS: A retrospective study was conducted over a 7-year period; 10 cases of abdominal tuberculosis in children were reviewed (6 girls and 4 boys, mean age: 11 years, extremes 4-14). Eight patients enrolled in this study satisfied the following criteria: a positive culture for mycobacterium tuberculosis on samples of ascites fluid, sputum, urine, abscess puncture and/or caseating granulomas on histologic examination of biopsies obtained by endoscopy, percutaneous aspiration or needle biopsy or exploratory laparotomy. Two other patients (patients No 1 & 5) had a favorable response within 10 weeks of antituberculous therapy. RESULTS: We observed 8 cases of peritoneal tuberculosis and 2 cases of intestinal tuberculosis. Extra-abdominal tuberculosis was found in 4 of those patients. Two patients had a contact with a tuberculosis positive individual. Nine children had BCG immunization. The main clinical features were abdominal distension (6 cases) and abdominal pain (6 cases). Abdominal ultrasonography visualized mesenteric lymphadenopathies (6 cases), an abdominal mass (5 cases), free (1 case) and localized ascites (1 case). Barium enema and small bowell series showed small bowel stenosis (1 case) and ileal ulcerations (1 case). Exploratory laparotomy, performed in 3 patients, showed whitish granulations and peritoneal abscesses with caseous necrosis on histology. Quadruple therapy with tuberculostatic agents was prescribed in all patients, associating isoniazid, rifampicin, pyrazinamide combined at the start of the treatment with streptomycin (7 cases) and ethambutol (3 cases). Short term outcome was favorable with no deaths. The long term outcome was complicated by persistent ascites (1 case) and the development of portal hypertension (1 case). CONCLUSION: Abdominal tuberculosis remains a rare localization with a lot of diagnostic difficulties.

Abdomen↗
PubMedDate not supplied

[Misleading forms of visceral leishmaniasis in children. Apropos of 5 cases].

The authors report the cases of five children in whom kala-azar was undiagnosed at first instance. In these cases, the diagnosis was misled because of incomplete features (lack of fever, splenomegaly or hypergammaglobulinemia) an associated disease (hydatic cyst of the liver) or a complication dominating the clinical pattern (septicemia, staphylococcus respiratory infection). In one case, the patient was explored in order to diagnose portal hypertension.

Antimony↗
PubMedDate not supplied

[Protée syndrome associated with renal lithiasis and vesico-ureteral reflux].

BACKGROUND: Proteus syndrome is characterized by a range of various manifestations. The main ones are partial gigantism of hands and feet, nevi, hemihypertrophy, macrocephaly. Urinary tract abnormalities are exceptional. CASE REPORT: A 6 year-old boy was examined because he had presented numerous abnormalities from birth. His weight was 26 kg (+3 SD) and his height was 135 cm (+4 SD). The main abnormalities were ptosis, pterygium colli, nevi of the cervical area, plagiocephaly, frontal bossing, scoliosis, hemihypertrophy involving the skin, mucosa and bones, macrodactyly, varicose veins and lipomatosis. He underwent surgery at the age of 3 years for urinary lithiasis associated with an ureterovesical reflux on the left side, i.e. the side of hemihypertrophy. CONCLUSION: This case suggests that Proteus syndrome may be an example of ectomesodermal dysembryoplasy but it requires confirmation by a report of at least one other case of obstruction of the ureteropelvic junction on the same side as hemihypertrophy.

Abnormalities, Multiple↗