Eight children with chronic diarrhea from glucose-galactose malabsorption from eight different families are presented. Six children are Saudi Arabs and two are of the other Arab nationalities. The mean age of the children at the time of presentation was 10.6 months. They were first seen for chronic watery diarrhea, present since birth, and failure to thrive. Laboratory investigations, including small-bowel biopsy, histology, and small-bowel enzyme assay, confirmed the diagnosis of glucose-galactose malabsorption. One child had a renal stone at the first visit, and another was discovered to have one on follow-up. All the children responded clinically to fructose-based formula, and they are thriving at follow-up.
All newborn infants delivered at King Fahd Hospital of the University in Al-Khobar between June 1982 and May 1987 were subjected to routine physical examinations, including the Ortolani and Barlow maneuvers for detection of hip instability. A total of 12,733 neonates were screened, and a positive test was found in 62 (0.49%). Infants with a positive test were placed in a von Rosen splint for 6 to 8 weeks. Five "missed" cases were diagnosed later, all of whom were born during the first year of the study. Male-to-female ratio was 1:4.2. Bilateral hip instability was seen more commonly in males than females. Known factors that increase the risk of hip instability include breech presentation and large weight for gestation. Follow-up was possible in 47 patients; all were asymptomatic except for one with poor compliance who required further surgical intervention. The importance of neonatal screening and continued follow-up for detection of hip instability among all hospital-delivered babies is stressed to prevent the hazards of late diagnosis.
To define the natural history of sickle cell anaemia a cord blood screening programme was initiated and during the first 3 years a total of 129 newborns with FS phenotype were detected, of whom 83 infants were followed up regularly. Forty-three of them had attained the ages of between 1 and 3 years and the analysis of their data revealed that in 21 of 43 cohort children (14 FS and 7 FS + Barts) who had almost identically raised levels of haemoglobin F, the clinical manifestations of the disease appeared to be influenced by the size of the RBC, with the microcytic group having a relatively milder clinical course as compared with the normocytics. Similar observations were made in about 50% (47/93) of non-cohort older children and adults with sickle-cell anaemia, suggesting that alpha thalassaemia in combination with increased levels of fetal haemoglobin was probably an important predictor of the clinical severity in patients with sickle-cell anaemia in the Eastern Province of Saudi Arabia.
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