Acute chest syndrome in adult sickle cell disease in eastern Saudi Arabia

Al-Suleiman, Ahmad M.; Aziz, Gowher; Bagshia, M; S, El Liathi; Homrany, Hassan

doi:10.5144/0256-4947.2005.53

Cited by 7 publications

(8 citation statements)

References 6 publications

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“…I studied the summation of results in this study together with those done in Qatif and Hofuf previously[2123] versus the results reported by Taylor, et al and Bernard Maitre, et al [2425] Tables 2 and 3. I found that recurrence is significantly lower ( P <0.025) in patients from Eastern province compared to patients with African haplotype, mortality also is lower but not statistically significant [Figure 2].…”

Section: Resultsmentioning

confidence: 93%

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Sickle cell disease patients in eastern province of Saudi Arabia suffer less severe acute chest syndrome than patients with African haplotypes

Alabdulaali

2007

Ann Thorac Med

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BACKGROUND AND OBJECTIVES:Genetic studies suggest that the sickle cell mutation has arisen on at least four separate occasions in Africa and as a fifth independent mutation in the Eastern Province of Saudi Arabia or India. The pathophysiology of sickle cell disease (SCD) is essentially similar in these different areas although the frequency and severity of complications may vary between areas. The aim of this study was to evaluate the prevalence and outcome of acute chest syndrome (ACS) in SCD patients from Eastern province of Saudi Arabia in comparison with patients with African haplotypes.MATERIALS AND METHODS:This was a retrospective study involving 317 SCD patients who were two years or older, admitted to King Fahad Hospital Hofuf between January-May 2003 for different etiologies. Twenty six patients presented with different causes of ACS; 11 patients presented with different pathologies other than ACS, but had past history of ACS; 280 patients presented with different pathologies and never presented with ACS. Clinical features, CBC, Hb-electrophoresis, G6PD activity, cultures, chest X-ray, arterial oxygen saturation, blood transfusion rates and outcome were studied. Univariate and multiple regression analysis were carried out to evaluate influence on ACS. Comparison between SCD patients with ACS from this study and from Eastern province of Saudi Arabia to patients with African haplotypes were carried out, using data reported in the literature.RESULTS:During the period of this study, 37 patients with new or previous episodes of ACS were studied (accounting for 11.67% of admitted SCD patients). Most of the patients with ACS had only one episode, but five patients (13.51%) had had episodes or more. One patient died giving an in-hospital mortality rate of 1/26 (3.85%). Comparison of recurrence of ACS and mortality between SCD patients in Eastern province of Saudi Arabia to that of patients with African haplotype showed that recurrence is significantly lower (P<0.025) in patients from Eastern province compared to patients with African haplotype, mortality also is lower but not statistically significant.CONCLUSION:Acute chest syndrome in SCD patients in Eastern province of Saudi Arabia is relatively uncommon, but causes significant morbidity and mortality. Its prevalence and recurrence is low if compared to that of patients with African haplotypes.

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Section: Resultsmentioning

confidence: 93%

“…[2123] On the other hand in patients with African haplotypes slight male predominance or even female predominance was reported. [2425] Udezue et al , reported some differences in the severity and features of SCD between males and females in Eastern area of Saudi Arabia.…”

Section: Resultsmentioning

confidence: 99%

Sickle cell disease patients in eastern province of Saudi Arabia suffer less severe acute chest syndrome than patients with African haplotypes

Alabdulaali

2007

Ann Thorac Med

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“…A formal study of mortality in the AI haplotype has not been done so it is not possible to compare survival in AI haplotype patients with other patient groups. In one analysis, the leading cause of death in Saudi patients was acute chest syndrome, followed by sepsis (Al‐Suleiman et al , ; Al‐Suliman et al , ). Adult patients with AI haplotype SCD bear some resemblance to patients with HbSC, given their higher haemoglobin levels, less haemolysis and a lower incidence of stroke and leg ulcers.…”

Section: Discussionmentioning

confidence: 99%

Sickle cell disease in Saudi Arabia: the phenotype in adults with the Arab‐Indian haplotype is not benign

et al. 2013

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Summary Sickle cell disease (SCD) in Saudi patients from the Eastern Province is associated with the Arab-Indian (AI) HBB (β-globin gene) haplotype. The phenotype of AI SCD in children was described as benign and was attributed to their high fetal haemoglobin (HbF). We conducted a hospital-based study to assess the pattern of SCD complications in adults. A total of 104 patients with average age of 27 years were enrolled. Ninety-six percent of these patients reported history of painful crisis; 47% had at least one episode of acute chest syndrome, however, only 15% had two or more episodes; symptomatic osteonecrosis was reported in 18%; priapism in 17%; overt stroke in 6%; none had leg ulcers. The majority of patients had persistent splenomegaly and 66% had gallstones. Half of the patients co-inherited α-thalassaemia and about one third had glucose-6-phosphate dehydrogenase deficiency. Higher HbF correlated with higher rate of splenic sequestration but not with other phenotypes. The phenotype of adult patients with AI SCD is not benign despite their relatively high HbF level. This is probably due to the continued decline in HbF level in adults and the heterocellular and variable distribution of HbF amongst F-cells.

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“…90 Al-Suleiman et al reported that 79% of their hospitalized patients with SCD subsequently developed acute chest syndrome. 91 Acute chest syndrome is characterized by the triad of chest pain, fever and appearance of a new infiltrate (often first at the bases and then involving whole lungs) on the chest radiograph. Chest pain typically starts with bone pain in the thoracic cage and often accompanied by pleurisy, tachycardia and tacypnoea.…”

Section: Sickle Cell Diseasementioning

confidence: 99%

Respiratory disorders in the Middle East: A review

et al. 2011

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The spectrum of pulmonary pathology in the Middle East is as versatile as its civilizations and cultures. In this review, we outline the key challenges confronting pulmonologists in the Middle East. We shed light on the diverse conditions commonly encountered in the region, from the centuries-old illnesses of tuberculosis, to contemporary problems such as lung complications from chemical warfare. We specifically highlighted unique aspects related to respiratory illnesses in the Middle East, for example, climate factors in the desert region, cultural habits, for example, water-pipe smoking and disorders unique to the region, such as Behçet's disease. Pulmonologists are also faced with the consequences of modernization, including large immigrant population and associated social and health issues, rising incidence of obesity and sleep apnoea, and drug-resistant tuberculosis. Tackling these health issues will require an integrated approach involving public health, primary care as well as specialist pulmonology input, taking into consideration the unique cultural and environmental factors to ensure effective management and compliance to medical care.

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Acute chest syndrome in adult sickle cell disease in eastern Saudi Arabia

Cited by 7 publications

References 6 publications

Sickle cell disease patients in eastern province of Saudi Arabia suffer less severe acute chest syndrome than patients with African haplotypes

Sickle cell disease patients in eastern province of Saudi Arabia suffer less severe acute chest syndrome than patients with African haplotypes

Sickle cell disease in Saudi Arabia: the phenotype in adults with the Arab‐Indian haplotype is not benign

Respiratory disorders in the Middle East: A review

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