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Biomedical subjects

A Fallah

Publications and source records attributed to A Fallah.

5 recordsLinked to original sources

Treatment of early onset multiple sclerosis with suboptimal dose of interferon beta-1a.

BACKGROUND: Patients with early onset multiple sclerosis may develop disability at a younger age than adults. There are several reports about safety of beta interferons in childhood and juvenile MS with different doses. OBJECTIVES: To determine safety and efficacy of substandard dose of intramuscular interferon beta-1a in a prospective randomized trial in patients with multiple sclerosis under the age of 16. METHODS: Sixteen patients were divided into two groups randomly. The first group was treated with intramuscular interferon beta-1a 15 micrograms once a week and the second group received no disease-modifying therapy. RESULTS: The patients were followed for four years. There was no significant side effect and none of the treated patients discontinued the drug. There were significant differences between two groups regarding relapse rates (p = 0.04), disability progression (p = 0.01), and new T2 lesions (p = 0.006). CONCLUSION: Treatment with interferon beta-1a is well tolerated for a long period of time and may be effective in substandard doses in early onset multiple sclerosis.

Adolescent↗

[Acute encephalopathy and recurrent hepatic steatosis with normal long and medium chain fatty acyl-CoA-dehydrogenase activity].

A boy suffering from recurrent episodes of acute encephalopathy and hepatic steatosis died at 40 months of age. The symptoms started when he was 13 months old and he appeared completely normal in the intervals. Pertinent biologic findings were as follows: slight labile hypoglycemia and hyperammonemia having no direct correlation with neurologic derangement, no elevation of ammonia levels in loading tests, complete failure to generate ketones and the absence of organic aciduria during a fast, normal plasma carnitine levels and normal activity of long and medium chain acyl-CoA-dehydrogenase in skin fibroblasts. Pertinent autopsy findings were marked steatosis of liver and renal tubular cells with many foamy histiocytes in bone marrow. An error in metabolic pathways, particularly a derangement in lipid metabolism, was considered.

Acute Disease↗

[Diffuse cutaneous vasculitis disclosing acute lymphoblastic leukemia].

Biopsy of skin for etiologic evaluation of generalized erythematous eruptions in a 4 1/2 years old child, starting four months ago, led to diagnosis of acute lymphoblastic leukemia. Leukocyte counts five and eight weeks and four months after appearance of eruptions were normal and no blasts were found. A leukocyte count performed simultaneously with biopsy of skin also showed few (less than 10%) blasts. Histologic examination of skin revealed two types of pathologic lesions: a perivascular cuffing of lymphoblasts and a leukocytoclastic type of small vessel vasculitis in other parts. This type of vasculitis which have been reported in association with chronic lymphocytic and hairy cell leukemias, is uncommon in acute lymphoblastic leukemia.

Child, Preschool↗

[Coexistence of late spondyloepiphyseal dysplasia and congenital megaloblastic anemia with proteinuria in the same family].

A case of spondyloepiphyseal dysplasia tarda was noted in a dwarf (130 cm tall) 18 years old boy associated with congenital megaloblastic anemia and proteinuria. His two sisters and a cousin are also suffering from similar hematologic disorder. One of his brothers, 145 cm tall, is also involved by spondyloepiphyseal dysplasia, but there is no known hematologic abnormalities. Review of family history revealed that two aunts from mother's side were deceased in adulthood following a chronic anemic disease. The findings in this anemia are compatible with Imerslund-Grâsbech syndrome and coexistence of these two rare genetic disorders in a single family has not been reported previously.

Adolescent↗