Infantile cholestasis in the Central-Eastern Province Saudi Arabia.

In the King Khalid University Hospital (Central Province) and King Fahad Hospital of the University (Eastern Province) Saudi Arabia, we identified 64 infants with cholestasis. The causes of cholestasis were idiopathic neonatal hepatitis in 29; extrahepatic biliary atresia in 17; neonatal hepatitis secondary to Rubella and Cytomegalovirus in six and four infants, respectively; paucity of intrahepatic bile ducts in six and galactosaemia in two. The diagnosis was confirmed by liver biopsy and or operative cholangiography, in all infants.

First case of human infection caused by Pasteurella gallinarum causing infective endocarditis in an adolescent 10 years after surgical correction for truncus arteriosus.

OBJECTIVE: To report the first case of human infection (infective endocarditis [IE]) caused by Pasteurella gallinarum and to review the literature regarding IE caused by the genus Pasteurella. SETTING: University hospital based. PATIENT: An adolescent boy who underwent successful correction for truncus arteriosus 10 years before the present illness. RESULTS: Persistent fever, pallor, and a palpable spleen suggested IE clinically. Echocardiography documented vegetation in the conduit that was used for surgical correction. Blood cultures grew P. gallinarum and confirmed its role as the causative organism for IE in the patient. CONCLUSION: This case illustrates that IE may develop in a child with congenital heart disease several years after surgical intervention using material that is foreign to the body (conduit), and that such a complication may involve unusual pathogens. These observations emphasize the need for careful long-term follow-up of children with congenital heart disease even after successful surgical correction.

Percentage glycosylated haemoglobin in normal, G6PD deficient and HbSS Saudi Arabs.

Reference values have been established for erythrocyte glycosylated haemoglobin levels in a normal Saudi population and in subjects with various haematological disorders. The mean glycosylated haemoglobin levels (SE) were 7.28% (0.039) for normal, 6.04% (0.057) for G6PD deficient, 4.40% (0.081) for homozygous sickle cell, and 6.44% (0.109) for heterozygous sickle cell subjects. Values of 6.26% (0.103) and 4.75% (0.127) for glycosylated haemoglobin were determined for heterozygous and homozygous sickle cell subjects with G6PD deficiency, respectively. Statistical analysis of the data shows significant differences in the extent of glycosylation between G6PD deficient, HbSS and normal controls. Where possible the results are compared to values reported for other populations.

Myelofibrosis and pancytopenia in systemic lupus erythematosus.

A 13-year-old Saudi girl presented with severe clinical features of systemic lupus erythematosus of 3 months' duration. In addition to laboratory evidence of the disease, the patient was found to have pancytopenia and myelofibrosis. While pancytopenia was considerably improved by steroids, myelofibrosis was not reversed. The coexistence of myelofibrosis and pancytopenia in patients with systemic lupus erythematosus has been reported, but their possible causal relationship remains unresolved.

Clinical presentation of systemic lupus erythematosus in Saudi patients.

A study conducted to assess the incidence and clinical expression of systemic lupus erythematosus in the Eastern Province of Saudi Arabia revealed that the disease is uncommon in this region, only 32 cases being recorded in five years. However, the clinical manifestations of the disease are similar to those observed elsewhere.