Splenic function in sickle cell anemia patients in Qatif, Saudi Arabia.

A prospective study was conducted to study the splenic function among sickle cell anemia (SCA) patients in Qatif (Eastern Province of Saudi Arabia). Seventy-seven patients (30 children and 47 adults aged 2-57 years) were included. (99m)Tc stannous colloid liver-spleen scan was done for each patient during steady state. The splenic function was graded from 0 to 4 in relation to liver uptake. Seventy percent of our patients showed evidence of splenic hypofunction, and most of them (83%) had severe hyposplenism. Up to the age of 4 years, only 17% of the children showed evidence of functional hyposplenism, but by the age of 10 years >50% were hyposplenic. Most of the hyposplenic children had functional hyposplenism, whereas only one-third of hyposplenic adults had autosplenectomy. There was no effect of level of HbF on the frequency of hyposplenism, but on the other hand low MCV seems to be protective against hyposplenism. A significant number of adult SCA patients have clinically enlarged spleens, and almost a third have normally functioning spleens. Because of the low prevalence of hyposplenism in children younger than 4 years of age, routine penicillin prophylaxis is probably not indicated in this population, an issue which needs further evaluation.

Isoxsuprine in the treatment of sickle cell painful crises: A double-blind comparative study with narcotic analgesic.

BACKGROUND: Isoxsuprine is a tocolytic agent which improves erythrocyte deformability. It was accidentally found to be effective in the management of sickle cell disease (SCD) painful crises. The experience with the drug in the treatment of sickle cell disease is limited. This double-blind randomized comparative study was undertaken to test its efficacy and safety in the treatment of SCD painful crises. PATIENTS AND METHODS: Forty-three SCD patients (33 males and 10 females) with 44 episodes of pain were included in the study (i.e., one patient was included twice). Only those with painful crises requiring hospital admission were included. Patients were randomized to receive either isoxsuprine 5-10 mg, or meperidine 50100 mg intramuscularly, according to body weight, every four hours. A random selection of 23 patients received isoxsuprine, and 21 received meperidine. Pain score, duration of crisis, hospital stay, and side effects were monitored. RESULTS: No significant difference was found in any parameter except for pain score at 30 and 60 minutes. CONCLUSION: We conclude that isoxsuprine appears to be effective in the treatment of sickle cell painful crises. Confirmation of its efficacy in studies involving a larger number of patients is warranted.

Elective, postoperative ventilation in the management of esophageal atresia and tracheoesophageal fistula.

The management of esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) has undergone many changes. As a result of recent advances in neonatal intensive care and pediatric anesthesia, the survival of infants with EA and TEF has improved markedly, but the occurrence of anastomotic complications has remained constant. To overcome this problem, various techniques and suture materials have been used. This review of 20 consecutive cases of EA/TEF stresses the importance and influence of non-reversal of anesthesia, paralysis, and elective ventilation for protection of the esophageal anastomosis following repair of EA and TEF.

Sensorineural hearing loss in homozygous sickle cell disease in Qatif, Saudi Arabia.

Sensorineural hearing loss (SNHL) is a known complication of sickle cell disease (SCD). There is a paucity of information on this problem in Sausi SCD patients. A prospective controlled study was conducted over 27 months in Qatif Central Hospital. One hundred patients with sickle cell anemia (SCA), aged five to 40 years, were studied. The control group consisted of 100 healthy individuals. A detailed history, otologic and audiologic examinations were carried out on both groups. Further investigations to rule out local retrocochlear pathology were performed on those having unilateral SNHL. No case of SNHL was detected in the control group, while it was recorded in 19 (19%) of SCA patients, 13 males and six females. There was a significant association between the SNHL and the onset of first vaso-occlusive crisis at six years of age or less. There was no significant difference in the frequency of SNHL between children and adults. Twenty-one percent of patients who had SNHL completely recovered. Patients with SCD need a careful regular hearing assessment. Those detected to have SNHL need a careful plan implemented by both the clinician and otolaryngologist for proper care of this important complication.

Perinatal morbidity and mortality in offspring of diabetic mothers in Qatif, Saudi Arabia.

Diabetic mothers and their offspring were prospectively studied. Perinatal and neonatal morbidity and mortality data were analysed. Out of 11,677 deliveries in the hospital, 133 (1.14%) were delivered by diabetic mothers. They were made up of six (4.5%) stillbirths and 127 (95.5%) live births. Seventy-seven (57.9%), 55 (41.3%) and 1 (0.8%) were large, appropriate and small for gestational age, respectively. Hypoglycemia occurred in 49 (38.6%) of the 127 infants. Other associated problems were bacterial infections, congenital anomalies, birth trauma, preterm delivery, respiratory distress, polycythaemia and anaemia. Only 52 (39.1%) of the mothers received appropriate treatment for their diabetes during pregnancy. Poor maternal diabetic control resulted in high perinatal morbidity and mortality in the offspring. In order to improve the outcome in offspring of diabetic mothers in Qatif and probably Saudi Arabia as a whole, health education and improved care of the diabetic mothers during pregnancy urgently needed. This may be true of other developing countries where data on diabetes in pregnancy are scarce.

PIP: In Saudi Arabia, pediatricians compared data on infants of 52 mothers who received insulin therapy during pregnancy (group A) with data on infants of 81 mothers who either did not receive insulin therapy during pregnancy or prenatal care (group B) to evaluate the outcome in infants of diabetic mothers (IDMs) managed at Qatif Central Hospital. These 133 IDMs comprised 1.14% of the 11,677 deliveries at this hospital during 1988-92. 19.7% of all mothers had gestational diabetes mellitus. Mothers in group A were younger than those in group B (31.5 vs. 35.1 years; p 0.01). They were less likely than those in group B to suffer fetal loss (23.1% vs. 53.1%; p 0.001 and [mean fetal loss] 0.62 vs. 1.33; p 0.05). All six stillbirths (2 in group A and 4 in group B) were large for gestational age (LGA) (4.543 vs. 3.753 kg for overall birth weight; p 0.001). One stillbirth was macerated and had multiple congenital anomalies including Down's syndrome. Two liveborn IDMs also had Down's syndrome. There were no early neonatal deaths. The perinatal mortality rate (PMR) was not significantly different between the groups, but the PMR for both groups was higher than it was for the same period for the hospital overall (45.1 vs. 16.6/1000; p 0.02). 57.9% of IDMs from both groups were LGA. 38.6% of all IDMs had a blood glucose level less than 30 mg/dl. Other problems identified in IDMs included bacterial infections, birth trauma, preterm delivery, respiratory distress, polycythemia, and anemia. These findings suggest that poor maternal diabetic control contributed to the high perinatal morbidity and mortality in IDMs. Health education and improved care of diabetic pregnant women are seriously needed.

The patterns of oropharyngeal microflora in patients with homozygous sickle cell disease.

A controlled prospective study was carried out between 16 July 1991 and 31 January 1992 to evaluate the oropharyngeal flora in homozygous sickle cell disease (SS). Throat swabs for cultures were taken from 163 asymptomatic SS patients attending sickle cell clinics (90 children and 73 adults). Throat swabs were also taken from 84 asymptomatic healthy individuals as a control group (44 children and 40 adults). Group A beta hemolytic Streptococci were isolated from 16.6% of children with SS compared to 4.5% of children without SS (P<0.05). Staphylococcus aureus was isolated from 16.4% of adults with SS compared to 0% of adults without SS (P<0.0001). Group G beta hemolytic Streptococci and nongroupable Streptococci were also isolated from 11% and 13.7% of adults with SS compared to 0% and 2.5% of adults without SS respectively. Several other organisms were isolated only from SS patients. We conclude that the oropharyngeal flora of patients with SS is disturbed. This disturbance may contribute to morbidity of these patients.