Mahmoud Al-Sheyyab

Effectiveness of iron therapy on breath-holding spells

OBJECTIVE The objective of this study is to investigate the effect of iron therapy on breath-holding spells (BHS). METHODOLOGY Sixty-seven children with BHS were enrolled in a clinical trial to evaluate the effect of iron therapy on BHS. At the beginning of therapy, the clinical, laboratory, and demographic characteristics of the patients in the treatment group (n = 33) and placebo group (n = 34) were comparable. Patients were assessed weekly for the first 8 weeks and then every 2 weeks for the next 8 weeks. Response to therapy was assessed by the change in the frequency of BHS. RESULTS Children treated with iron showed significant reduction in the frequency of BHS (88%) compared with the frequency (6%) in the placebo group. As expected, the treated group showed a significant improvement of a number of blood indexes compared with the placebo group. Baseline mean levels of hemoglobin and total iron binding capacity were predictive of a favorable response to iron treatment. CONCLUSION Results of this study indicate that iron therapy is effective in the treatment of BHS and that iron-deficient children seem to be more likely to benefit from such therapy. Response to iron therapy was strongly correlated with improvement in blood indexes.

Lack of effectiveness of dexamethasone in neonatal bacterial meningitis.

Abstract A clinical trial was conducted to determine whether dexamethasone as adjunctive therapy alters the outcome of bacterial meningitis in neonates. Fifty-two full-term neonates with bacterial meningitis were enrolled in a prospective study. Infants were alternately assigned to receive either dexamethasone or not. Twenty-seven received dexamethasone in addition to standard antibiotic treatment and 25 received antibiotics alone. Dexamethasone therapy was started 10–15 min before the first dose of antibiotics in a dose of 0.15 mg/kg per 6 h for 4 days. Baseline characteristics, clinical and laboratory features in the two groups were virtually similar. Both groups showed a similar clinical response and similar frequency of mortality and sequelae. Six (22%) babies in the treatment group died compared to 7 (28%) in the control group (P = 0.87). At follow up examinations up to the age of 2 years, 6 (30%) of dexamethasone recipients and 7 (39%) of the control group had mild or moderate/severe neurological sequelae. Audiological sequelae were seen in two neonates in the dexamethasone group compared to one in the control group. Conclusion Adjunctive dexamethasone therapy does not improve the outcome of neonatal bacterial meningitis.

The Prevalence of Hepatitis B, Hepatitis C and Human Immune Deficiency Virus Markers in Multi‐transfused Patients

All patients presenting with hereditary hemolytic anemia, (n = 143) over a period of 18 months were enrolled in a study to evaluate the prevalence of hepatitis B, hepatitis C and HIV in multi-transfused patients in Jordan, and to identify possible related risk factors. All patients were treated in the Thalassemia Unit at Princess Rahma Teaching Hospital. Relevant clinical data were collected. Blood specimens were taken from these patients and tested for HbsAg, HbsAb, hepatitis core IgMAb, hepatitis core IgGAb, HCVAb, and ELISA for HIV. Fifty-eight (40.5 per cent) of the specimens were HCVAb positive, while only five (3.5 per cent) of them were positive for HBsAg. None of the specimens were positive for HIV. The frequency of blood transfusion and the time of diagnosis before or after 1995, were investigated as possible risk factors for viral seropositivity. Only the time of diagnosis was a statistically significant risk factor for HCVAb positivity (OR = 4.49; p = 0.005). In conclusion, hepatitis C acquisition is a serious risk for multi-transfused patients in Jordan. Hepatitis B is relatively less common. Blood screening initiated after 1995 in Jordan has significantly reduced the risk of hepatitis C associated with blood transfusion.

Henoch-Schonlein purpura and streptococcal infection: A prospective case-control study

A prospective, matched, case-control study conducted over a period of 3 years was designed to examine the association of group A beta-haemolytic streptococcal infections and Henoch-Schonlein purpura. Demographic and clinical data were collected as well as measurement of antistreptolysin O titres and throat swab culture on all children admitted with Henoch-Schonlein purpura, as well as their matched controls. Antistreptolysin O titre positivity was associated with a 10-fold increase in the risk of Henoch-Schonlein purpura. Renal involvement was common among cases with positive antistreptolysin O titres (27%) compared with cases with a negative titre (8%) but this difference has no statistical significance.

The clinical spectrum of Henoch-Schönlein purpura in infants and young children

Henoch-Schönlein purpura is a common cause of childhood vasculitis. The rarity of the disease under 2 years of age has been the subject of few reports. We present the clinical spectrum of Henoch-Schönlein purpura in 12 children younger than 2 years of age at presentation. The median age at presentation was 11 months. The purpuric skin rash was present in all patients and involved the face in 10 of them. While oedema was a prominent feature in all of our patients only one third had involvement of the kidneys, gastro-intestinal tract or joints. All patients recovered completely after a mean duration of follow up of 10.6 months (range 2–39 months).ConclusionHenoch-Schönlein purpura under the age of 2 years is characterized clinically by oedema and a purpuric skin rash which frequently affects the face. Involvement of the joints, kidneys and gastro-intestinal tract is uncommon and the prognosis is excellent. The clinical spectrum in this age group is a continuation with that of Henoch-Schönlein purpura in older children suggesting a nosological entity.

Bleeding tendency in Wolfram syndrome: a newly identified feature with phenotype genotype correlation

Abstract Wolfram syndrome (WS) is a recessively inherited disorder associated with recognised clinical features. Bleeding tendency was noticed in some of our patients, although this has not been reported before. We therefore studied this problem in all our WS patients and tried to postulate a possible pathogenesis. At the same time, a genetic linkage study provided evidence of locus heterogeneity of this syndrome and showed that the majority of our patients belong to the second WS locus identified in that study. Our study group consisted of 13 WS patients, belonging to WSF2 locus (group I). Controls consisted of 4 healthy siblings of WS patients (group II) and 7 diabetics who do not have WS (group III). Relevent clinical data were obtained, and a coagulation screen was carried out for all groups. All individuals in the three study groups have normal platelet count, thrombin time (TT), prothrombin time (PT), activated partial thromboplastin time (aPTT), clot retraction, Factor VIII activity (FVIIIc) and von Willebrand factor antigen (vWAg). Eleven of the WS patients have prolonged template bleeding time (BT) compared with both control groups. Patients with WS have a longer BT (mean 9.6 min, 95% CL 8.61–10.53 min) than the siblings group (mean 6.75 min, 95%CL 5.52–7.98 min) and the diabetic group (mean 5.49 min, 95%CL 4.56–6.42 min). The differences between the study group and controls are statistically significant, p=0.02 and 0.0002, respectively. In the three groups, platelet aggregation studies were normal using adenosine diphosphate (ADP), ristocetin and epinephrine. Aggregation with collagen was either absent or impaired, with failure of secondary wave being noticed in 11 of the WS patients (85%) and normal in the control groups. The pathogenesis of this problem is not known, but could be due to an inhibitory effect of vWAgII, deficiency of thrombospondin or a defect in the platelet membrane GPIa/IIa. Bleeding diathesis is a new additional feature to the clinical spectrum of WS, which is probably a feature of the disorder WFS2 and not WFS1, as bleeding has never been reported in the latter. This provides further evidence for the phenotypic and genotypic heterogeneity of this complex disorder and may provide clues to the search for the second gene responsible for this phenotype.

INHIBITION OF K+ TRANSPORT IN HUMAN SICKLE CELL ERYTHROCYTES BY OKADAIC ACID AND SODIUM FLUORIDE

1. The effect of okadaic acid and sodium fluoride on swelling‐and N‐ethylmaleimide (NEM)‐stimulated KC1 cotransport was examined in blood cells from homozygote sickle cell anaemia patients.

Effect of Desferrioxamine in Acute Haemolytic Anaemia of Glucose-6-Phosphate Dehydrogenase Deficiency

The effectiveness of desferrioxamine (DFO) in ameliorating the severity of the acute haemolysis of glucose-6-phosphate dehydrogenase (G6PD) deficiency was studied in 167 children with G6PD deficiency during an acute haemolytic crisis. All patients received packed cell transfusion on admission if their Hb levels were <8 g/dl, which was repeated as needed. Eighty patients also received a single dose of DFO 30–40 mg/kg by slow intravenous infusion (DFO group). The remaining 87 children did not receive DFO (control group). The need for more than one transfusion was less frequent in the DFO group as compared to the control group (p = 0.01). The need for late transfusion (transfusion after 36 h of admission) was also less in the DFO group (7%) compared to 21% in the control group (p = 0.02). On average, children in the DFO group needed less packed red blood cells (16.5 ml/kg body weight) than the control group (22.8 ml/kg body weight) and the difference was highly significant (p = 0.0001). We conclude from this study that DFO in a small dose is effective in the treatment of acute haemolytic crises of G6PD deficiency. It shortens the duration of the crisis and decreases the amount of blood transfusion needed.

Detection of b-thalassemia carriers in Jordan.

Hemoglobinopathies are a major health problem in many areas of the world. Two of the most prevalent hemoglobinopathies are sickle hemoglobin (Hb S) and βthalassemia. Thalassemia is a molecular abnormality with underproduction of one of the globin chains. 3 Heterozygous states for β-thalassemia genes, known as β-thalassemia minor, are usually not associated with clinically significant disease. β-thalassemia has a high prevalence in the Mediterranean area, and in the Middle and Far East. Thalassemia screening programs have demonstrated effectiveness as antecedents to more successful management of the disease, including early intervention and prevention of complications. However, the value of screening programs has not been without controversy, due to the social and psychological impact on the individual and the family, 1 such as parental frustration, the lack of adequate information on the disease among the local population, and a cultural system which makes a carrier feel inferior. Jordan is located in an area where β-thalassemia is prevalent, but no definite screening program has yet been developed. The aim of this study is to determine the real prevalence of thalassemia carriers in Jordan, using our own cutoff points, and to develop a better understanding of this disease. This should result in a more efficient utilization of health care resources and improved planning in the provision of health care services.