Haematological, clinical and some molecular genetic features of homozygous sickle cell (SS) disease in Saudi Arabia have been compared in 33 patients from the Eastern Province (Eastern) and 30 from the South Western Province (Western). Eastern patients all had the Asian beta globin haplotype whereas Western patients were more variable but predominantly of the Benin haplotype. Eastern patients had more deletional alpha thalassaemia, higher total haemoglobin and fetal haemoglobin levels, and lower HbA2, mean cell volume, reticulocytes, and platelet counts. Clinically, Eastern patients had a greater persistence of splenomegaly, a more normal body build and greater subscapular skin fold thickness, and Western patients had more dactylitis and acute chest syndrome. Painful crises and avascular necrosis of the femoral head were common and occurred equally in both groups. The disease in the Eastern province has many mild features consistent with the higher HbF levels and more frequent alpha thalassaemia but bone pathology (painful crises, avascular necrosis of the femoral head, osteomyelitis) remains common. The disease in the West is more severe consistent with the Benin haplotype suggesting an African origin.

Results of a double-blind randomized non-crossover study of rapid (45 min) versus slow (4 h) infusion of amphotericin B administered to 20 patients with proven or suspected fungal infection are reported. Toxicity was higher in the rapid infusion group than it was in the slow infusion group (mean total 7-day chill score, 173 ± 276 versus 20 ± 30 [P < 0.01]; mean total 7-day dosage of meperidine required to abate rigors, 180 ± 133 versus 58 ± 78 mg [P < 0.05]; and mean maximum total 7-day pulse rise, 225 ± 64 versus 135 ± 56 beats per min [P < 0.02], respectively). When analyzed on a daily basis, the mean chill score, meperidine dosage, and pulse rise were also higher; and in addition, nausea and vomiting (5 of 11 patients who received a rapid infusion versus 0 of 9 patients who received a slow infusion [P < 0.01]) appeared to be more common in those who received amphotericin B rapidly. The daily analysis approach proved that tolerance to these side effects developed with each subsequent infusion day, and by day 7 the incidence and severity were the same. This development of tolerance was signfficant for the mean chill score in the rapid infusion group (P < 0.05) and for the proportion of patients with chills (P < 0.005 for the slow infusion group; P < 0.05 for the rapid infusion group). A decrease in creatinine clearance to >51% of the baseline value was seen in two patients in each group. There were five deaths (four in the rapid infusion group, 1 in the slow infusion group) within 1 month, but none was clearly related to the amphotericin B infusion. The mean time to defervescence was similar for each group (10.8 ± 4.1 days in the slow infusion group versus 9.9 ± 5 days in the rapid infusion group). A rapid infusion regimen for amphotericin B cannot be recommended, at least during the first 5 to 7 days of treatment.Amphotericin B is an antimycotic polyene antibiotic that is commonly used for the treatment of proven or suspected severe fungal infections. Its administration requires intravenous access and, conventionally, a 6-h-daily infusion for days, weeks, or months, depending on the nature of the fungal infection. The prolonged infusion time consumes excessive patient and nursing time, but it is said (9) to be necessary to minimize the formidable toxicity of amphotericin B, which includes rigors, hypotension, renal toxicity, and cardiac dysrhythmias. Occasional anecdotal reports (11) and studies (2, 5) have suggested no increase in toxic side effects if amphotericin B is administered over a much shorter period. The present study compares the toxicities of rapid versus slow amphotericin B infusion rates when it is given to patients with proven or suspected fungal infections. MATERIALS AND METHODSTwenty patients with suspected or proven severe fungal infections were randomly ascribed by a computer-generated code held by the Department of Pharmacy Services, King Faisal Specialist Hospital and Research Centre, to receive amphotericin B over either 45 min (rapid infusion) or 4 h (slow infusion). Patients were recru...

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Hickman catheters (Evermed, Medina, WA) were inserted into patients at the King Faisal Specialist Hospital and Research Centre (KFSHRC), Riyadh, Saudi Arabia. One hundred sixty catheters were placed in patients with malignant disease, many of whom were immunosuppressed at the time of catheter insertion. Seventy of 160 (44%) patients received prophylactic antibiotics and 90 (56%) did not. The mean age of each group was 23 years (range, 2 to 70 years), and the patients in each group were statistically similar in sex, underlying disease, and routine preoperative hematologic and biochemical evaluation. Exit-site wound infections occurred in 50 of 90 (55.5%) patients who did not receive prophylaxis and in 12 of 70 (17%) patients who received prophylaxis (P < 0.0001). There was no statistically significant difference in the incidence of tunnel and incision site infections. The mean duration of antibiotic prophylaxis was 2.9 days (SD, 1.2 days) Organisms cultured from catheter associated infections, included Staphylococcus epidermidis 36, S. aureus 30, Klebsiella pneumoniae 1, Pseudomonas aeruginosa 3, Eschericia coli 1, and diphtheroids non-CDC-JK 3. Vancomycin was used as antibiotic prophylaxis in 64 patients, Kefzol (Eli Lilly, Indianapolis, IN) in one, oxacillin in three, nafcillin in one, and Septra (Burroughs Wellcome, Research Triangle Park, NC) in one. The data indicate that the use of intravenous antibiotic prophylaxis significantly reduces exit site infection and may reduce both tunnel and incision site infection. Prophylactic antibiotic coverage should be provided to patients during insertion of long-term indwelling right atrial catheters.

We examined mean corpuscular volume (MCV), fetal hemoglobin (HbF) levels, and severity of clinical disease in 81 patients with sickle cell anemia from various regions of Saudi Arabia. We found that patients from eastern Saudi Arabia had less severe symptoms, higher mean Hb levels, and lower MCV than patients from other regions. Using a severity index, calculated as the mean value of individual symptoms and physical signs in patients in each region, we found that less severe symptoms were associated with lower MCV. High HbF levels were not confined to Eastern Province patients but were seen in other regions. There was a statistically significant inverse correlation between HbF% and the severity index. We also found that only two of the 24 minimally symptomatic patients had an HbF level less than 18%, whereas among the 57 severely symptomatic patients, 14 (25%) had an HbF level less than 18%, confirming the protective value of a certain minimum level of HbF.