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Biomedical subjects

A R Sa'di

Publications and source records attributed to A R Sa'di.

5 recordsLinked to original sources

Agnogenic myeloid metaplasia in children.

Agnogenic myeloid metaplasia is a chronic myeloproliferative disorder characterized by splenomegaly, leukoerythroblastosis, extramedullary hematopoiesis, teardrop-shaped red blood cells, and fibrosis of the bone marrow. It is a disease of adults, with only one case report in the pediatric literature. The symptoms of the patient in this case clearly fit the diagnostic criteria of this disease. Myelofibrosis in children is usually of the acute type, presenting in infancy and running a fulminant, fatal course with minimal or mild splenomegaly. Red blood cells are usually normal on morphologic examination. Three infants, including two siblings, presented at ages 9, 10, and 16 months with clinical and laboratory findings consistent with agnogenic myeloid metaplasia. The occurrence of the disease in these siblings is suggestive of an autosomal recessive mode of inheritance.

Biopsy

Persistent neonatal hyperinsulinism.

Over a 3-year period, the diagnosis of persistent neonatal hyperinsulinism (PNH) was made in seven infants, from an unselected cohort of 18,726 births, all of Saudi Arabian origin. Thus the incidence of PNH was one in 2,675 births. The high incidence, associated consanguinity, and occurrence in siblings suggest that PNH may be inherited as an autosomal recessive disorder.

Consanguinity

White blood cells and bone marrow in typhoid fever.

Leukopenia and/or neutropenia are thought to be characteristic findings in patients with typhoid fever. In a study of 29 children 8 months to 15 years of age mild neutropenia was found in 1 (3%) and leukopenia in 6 (20%) patients. These findings are similar to those reported recently. Epinephrine stimulation tests done in 3 patients excluded the possibility of excessive margination as a cause of neutropenia or leukopenia. In 3 neutropenic or leukopenic patients bone marrow examination showed hemophagocytosis with an increased number of histiocytes that had phagocytized neutrophils, red blood cells and platelets. This phenomenon was not seen in the bone marrow of 3 patients with normal white blood cell counts. Hemophagocytosis is an important mechanism in producing neutropenia, anemia and thrombocytopenia in several infectious and noninfectious disorders.

Adolescent

Hereditary pyropoikilocytosis: report of two cases from Saudi Arabia.

Hereditary pyropoikilocytosis is a rare type of congenital hemolytic anemia reported only in American black children. We report the first two occurrences in Saudi children. This is an autosomal-recessive trait as proved by normal parents and two affected children. A pathogenetic and probably causal relationship with apparent elliptocytosis seems clear as three sibs have that condition.

Anemia, Hemolytic, Congenital

Salicylate hepato toxicity in rheumatic fever.

Two children with rheumatic fever developed anicteric hepatitis while on high-dose aspirin therapy. The striking chemical abnormality was an elevation of serum glutamic oxalacetic transaminase (SGOT) [aspartate aminotransferase (AST)]. A percutaneous liver biopsy obtained from one of the patients showed ultrastructural abnormalities consistent with a toxic hepatitis and hepatic congestion. An awareness of this potential complication is important when treating children with aspirin at doses previously considered to be non-toxic.

Aspartate Aminotransferases