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The aetiology and complications of exchange transfusion (ET) were studied over a 6-year period in northern Jordan. During that time, 336 neonates (0.46% of total live births) underwent 386 ETs. There was a yearly reduction in the number of ETs, varying from 8.2% in the 1st year of the study to 2.7% in the last year. Thirty-nine (11.9%) required more than one ET. Twenty-five (7.4%) were preterm babies and the remainder full-term. The commonest cause of ET overall was G6PD deficiency, either alone or concomitant with ABO incompatibility (38.1%). ET complications occurred in 51 neonates (15.2%), the commonest being anaemia and bradycardia. Septicaemia occurred in only 3% of cases. Only one baby died. G6PD deficiency, either alone or concomitant with ABO isoimmunization, is the most common cause of ET in northern Jordan. Fewer preterm babies require ET and there is a low incidence of septicaemia following ET. [ABSTRACT FROM AUTHOR]

The objective of this study was to evaluate the rate of acute splenic sequestration (ASSC) in patients with sickle beta-thalassaemia and sickle cell anaemia, the risk of recurrence in those who survive the first episode, and the relationship between ASSC episodes and subsequent hypersplenism. All patients with confirmed diagnosis of sickle cell disease at a tertiary referral teaching hospital, between January 1994 and December 2002 were interviewed and had their medical records reviewed. Seventy-seven patients with sickle cell disease were identified. Their ages ranged between 2 and 18 years (mean, 10.1 years). There were 35 females and 38 males. Thirty-seven (50.6%) had sickle beta-thalassaemia, and 36 (49.4%) had homozygous sickle cell anaemia. Of these, 26 had high level of Hb F and 11 had normal level of fetal haemoglobin (Hb F). Twenty-one patients (28%) had 63 episodes of acute splenic sequestration. Thirty-seven episodes were experienced by 12 patients with sickle beta-thalassaemia; of these 11 were major attacks with one fatality. Twenty-six episodes were experienced by nine patients with sickle cell anaemia. Splenomegaly and hypersplenism were greater in the acute splenic sequestration group than in the rest of the sickle cell anaemia patients, and the differences were extremely significant. ASSC was found in nine siblings of sickle beta-thalassaemia group, while none were found in the sickle cell anaemia group. The mean age of the first episode was significantly higher in sickle beta-thalassaemia, with significant differences in the levels of Hb F, Hb S, size of spleen and severity of crisis between both groups. In the sickle cell anaemia group the only significant difference between patients with and these without acute splenic sequestration was the difference in the size of spleen. In this study, the rate of ASSC in the sickle beta-thalassaemia patients was 32%, in contrast to 25% in the sickle cell anaemia patients. The risk of recurrence was about 70% in those who survived their first episodes. There was a close relationship between ASSC and subsequent hypersplenism. Important predictable factors for ASSC in sickle beta-thalassaemia patients were the presence of splenomegaly of more than 5 cm below the costal margin, history of acute splenic sequestration in siblings and high Hb F. Most of first episodes in sickle cell anaemia occur under the age of 2 years, while in sickle beta-thalassaemia the majority of patients have their first crisis at the age of > or =3.5 years.

Aim: The aim of this study was to assess the prevalence and distribution of dental caries in subjects with thalassaemia major., Design: Clinical examination for dental caries, diagnosed according to the WHO criteria., Participants: A total of 54 thalassaemic patients, 23 aged 6-9 (14 males and 9 females) and 31 aged 12-18 (17 males and 14 females) were examined., Outcome Measures: dmft, DMFT and plaque scores., Results: The mean dmft was 6.92 for 6-7 year olds and 4.72 for 8-9 year olds. The DMFT values were 6.57 and 5.95 for ages 12-14 and 15-18, respectively. There was no statistically significant difference in caries prevalence (dmft/DMFT) between gender or between primary and permanent teeth. Only 17.4 per cent of the children aged 6-9 and 21.4 per cent of 12-18 years olds were caries free. The prevalence of dental caries in the thalassaemia patients was considerably higher (22.7 per cent) than that reported in a normal Jordanian sample (DMFT 6.26 vs 4.84). Very few fillings (1.4 per cent of the examined teeth) were observed, indicating a negligible rate of conservative treatment. More than half (61.1 per cent) of the patients had poor oral hygiene (plaque score > or = 2.0)., Conclusions: The need for effective preventive measures, education and dental treatment need to be stressed for this caries risk group.

Homozygous sickle cell anaemia (SCA) is an inherited red blood cell disorder in which haemoglobin A is replaced by haemoglobin S. The disease exhibits a wide spectrum of clinical behaviour which has been well described in neighbouring countries. In Jordan, however, the disease has never been characterized. We used reviews of patients' notes and clinic interviews at Princess Badi'a Teaching Hospital, Irbid, Jordan to describe the clinical presentation of the disease in our area. The total number of patients was 41 (28 boys and 13 girls) and the median age was 9 years (range 1.5-21). The median age at presentation was 2.5 years (range 0.5-11). The commonest presenting feature was pallor (62%). An unusual feature of the disease in this series is the presence of a palpable spleen in 44% of patients older than 8 years. Associated G6PD deficiency was present in 29% of boys. Different clinical patterns of SCA were observed. Haemoglobin F is unlikely to be an explanation for this variability since it has not been shown to correlate with a number of severity indices of the disease such as the frequency of blood transfusions, hospitalizations and painful crises. Although the disease haplotype is not known in Jordan, our geographical location between Asia and Africa may suggest the presence of more than one haplotype and consequently different clinical patterns.

A prospective study was undertaken over a 1-year period in northern Jordan to determine the incidence, causes and characteristics of neonatal septicaemia which is a major cause of mortality and morbidity in newborns. The study identified 47 septicaemic neonates, representing an incidence of 2.3/1000 live births. Respiratory distress and hyperbilirubinaemia were the prominent presenting clinical findings. Gram-negative organisms were cultured from 46 neonates with Klebsiella species, accounting for 64% of cases. There were no cases of infection with group B Streptococcus or Listeria monocytogenes. The overall mortality rate was 40%. Prematurity and low birthweight, early onset septicaemia and concomitant meningitis were associated with high mortality. The high incidence of Gram-negative septicaemia and the antibiotic sensitivity pattern of the organisms indicated the use of cefotaxime or ceftazidime as initial therapy while awaiting culture results. Continued surveillance of neonatal septicaemia is mandatory due to temporal changes in the causative organisms and their antibiotic sensitivity.