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Biomedical subjects

J M Lévy

Publications and source records attributed to J M Lévy.

At least 19 recordsLinked to original sources

[Fibromatosis and fibrodysplasia ossificans progressiva. An avoidable diagnostic error].

BACKGROUND: Myositis ossificans progressiva is a rare progressive disease of connective tissue and muscle whose early diagnosis, before the lesions become ossified, can be difficult. The congenital malformations that accompany the disease may help in this diagnosis. CASE REPORT: A 12 year-old boy developed a localized swelling in the right trapezius muscle. Muscle biopsy showed fibromatosis lesions. Surgical excision was incomplete and despite chemotherapy, new lesions appeared periodically in the cervical and dorsal regions where they became ossified. Bilateral hallux valgus was noticed at that time and used to rectify the diagnosis as myositis ossificans progressiva. CONCLUSION: Many children suffering from myositis ossificans progressiva have congenital malformations, most commonly of big toes and thumbs. These anomalies are important for distinguishing myositis ossificans progressiva from other inflammatory diseases of muscle.

Child↗

[Role of allogeneic transplantation of bone marrow in juvenile chronic myelomonocytic leukemia].

Two boys with clinical and haematological evidence of juvenile chronic myelomonocytic leukaemia had no chromosomal anomaly. In addition, one presented with an unbalanced Epstein-Barr virus serology, and the other with xantholeukaemia. Allogenic bone marrow transplantation was performed in the first boy after an 18-month period during which treatment with 6-mercaptopurine, intensive chemotherapy and splenectomy had failed. Conditioning included cyclophosphamide, high-dose cytarabine and whole-body irradiation. There was no complication, and 16 months after transplantation the patient was in complete remission. The second boy received a bone marrow transplant on the 6th month of the disease, after failure of 6-mercaptopurine. Conditioning included etoposide, busulfan and cyclophosphamide. On the 35th post-transplantation day the child had severe pancytopenia and his spleen remained enlarged. A second transplantation was performed after treatment with melphalan and whole-body irradiation. Twelve months later, the patient was in complete remission. The indications and modalities of allogenic bone marrow transplantation in juvenile chronic myelomonocytic leukaemia and the value of pre-transplantation splenectomy are discussed.

Antineoplastic Combined Chemotherapy Protocols↗

[Congenital carotid to jugular aneurysm].

A congenital carotid--jugular aneurysm was responsible for severe heart failure in a two day old baby. The child recovered after surgery. The signs suggesting an arteriovenous fistula (a continuous murmur and thrill, hyperdynamic circulation) may be absent, as in this case, when the child is in severe cardiac failure. The signs should be sought when the circulation improves.

Arteriovenous Malformations↗

[Clinics and genetics of Glanzmann's thrombasthenia (author's translation)].

Glanzmann's thrombasthenia begins in infancy. The most important clinical signs are cutaneous and mucous hemorraghes (purpura, epistaxis, bleeding gums, menorraghia after puberty). The disease proceedes by alternate periods of activity and remission, the frequency and severity of which decrease with age. Death occurred during any of the attachs. Our 10 cases (7 girls, 3 boys) were in 5 "Manouche" gypsies sibships, interrelated by consanguineous marriages. Genetic studies demonstrated that the disease was inherited in the autosomal recessive pattern. The detection of heterozygotes is now possible with an antibody which appeared in a thrombasthenic patient. This antibody agglutinated the normal platelets, but not the ones of thrombasthenic patients. In heterozygotes the agglutinating effects is intermediate between the homozygote and normal.

Adolescent↗