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Two cases of cyclopic malformations are described among 450 infants of diabetic mothers during a period of four years. Both died within 30 min. of birth. Both belonged to unrelated Libyan families with absent consanguinity and family history. No chromosomal defect was found in either. A possible etiological association with uncontrolled maternal diabetes is discussed. [ABSTRACT FROM AUTHOR]

A 9 year old Libyan boy presented with a history of delayed walking and abnormal gait. The presence of marked muscle under-development with hypotonia led to the initial diagnosis of primary muscle disease; later, he was found to have hyperelastic, fragile skin and hypermobile joints-the cardinal features of Ehlers Danlos syndrome. In this instance the disease seems to have been inherited in an autosomal recessive manner. [ABSTRACT FROM PUBLISHER]

Visceral leishmaniasis is an important public health problem in Libya, but its exact prevalence is not known. Prompted by the paucity of information in the literature relevant to Libyan children, we reviewed the records of 21 children treated at El-Fatah Children's Hospital, Benghazi between March 1982 and May 1990. Visceral leishmaniasis was diagnosed on the basis of the history, physical findings and confirmatory laboratory tests including examination of bone marrow. The duration of illness before seeking medical advice ranged from 3 months to 1.5 years. The commonest presenting features were fever, abdominal distension, anorexia with weight loss, hepatosplenomegaly and pallor. The consistent laboratory findings were anaemia with reticulocytosis and normal serum iron, neutropenia, thrombocytopenia, high ESR and hyperglobulinaemia. The bone marrow was positive for L. donovani in 86% of cases and the indirect haemagglutination test was positive in all patients. Bronchopneumonia was the most common complication and responded rapidly to antibiotics. All patients were treated with sodium stibogluconate 10 mg/kg/day. There were no major side-effects or complications of drug therapy. The relative paucity of cases and their late presentation may reflect a lack of awareness of the occurrence of visceral leishmaniasis by doctors in the community.

Priapism is an unusual and distressing complication of sickle cell anaemia and its management has been varied and generally unsatisfactory. We report priapism in a Libyan boy with sickle cell anaemia, managed successfully by blood transfusion.

We report three Libyan children from one family with the syndrome diabetes insipidus, diabetes mellitus, optic atrophy and deafness, (DIDMOAD). Two children presented with diabetic ketoacidosis while one was discovered during screening of the family. All three children are alive, two of them on desmopressin (DDAVP) and insulin therapy and one on DDAVP only.

Melkersson-Rosenthal syndrome (MRS) is a rare condition with variable presentation. In some cases there is sequential development of clinical features. This report describes the syndrome in two Arab children who showed partial response to therapy with oral steroids. Since the natural course of the disease is unpredictable and there may be natural remission of symptoms, the efficacy of steroids is difficult to establish.

Chronic myeloid leukaemia (CML) is a rare disease in children. Three varieties of CML occur in childhood. Juvenile (Infantile), adult and familial types, each with distinct clinical and laboratory findings. Prognosis of all these types of CML in childhood is uniformly bad. The mean survival of a juvenile CML is 9 months and that of adult variety is 2.5 years. The adult form of childhood CML responds to therapy, but the patient succumbs to the disease during the blast crisis. Various modalities of treatments are being tried in the juvenile CML without any beneficial effect. We report a case of juvenile CML with characteristic findings in a four year old boy.

An infant with moderate growth retardation, convulsions, cranio-facial dysmorphia, absence deformity of limbs and vertebral segmentation defect is described. She was born to a young mother who had taken castor oil seed (Ricinus communis) orally as a contraceptive for eight weeks after conception. No other drugs were taken during pregnancy. The features of this case partially resemble Fetal Hydantoin Syndrome.

Immerslund-Grasbeck syndrome is an uncommon disease, characterized by megaloblastic anaemia and persistent proteinuria. A Libyan boy with the characteristic findings is presented. He received intramuscular vitamin B12 injections and there followed a remarkable clinical and haematological improvement.