Variability of sickle cell disease in the Eastern Province of Saudi Arabia

Mouzan, M. I. El; Awamy, B H al; Torki, Mehdi; Niazi, Gulzar A.

doi:10.1016/s0022-3476(89)80440-8

Cited by 25 publications

(21 citation statements)

References 11 publications

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“…Such mildness is postulated to be due to the cumulative effect of multiple factors, chief among them being the higher HbF levels. The inhibitory effect of HbF upon the process of RBC sickling by interfering with the formation of HbS polymers has been extensively documented; it has been further observed that patients having HbF levels >20% are more likely to have milder sickling manifestations in sickle cell disease [1,[3][4][5]7,11,22,25]. In the present study, 75% of total patients, belonging to the ''SCA with high HbF'' and ''S-b thalassemia'' groups, had HbF levels > 20%.…”

Section: Discussionsupporting

confidence: 50%

“…Two distinctive patterns of sickle cell disease occur in western and eastern Saudi Arabia regions; the disease in western Saudi Arabia resembles that associated with Benin haplotype of b S globin gene. It is characterized by a high frequency of vascular occlusive crises, nephropathy, priapism, stroke, infections, major-type acute splenic sequestration crises, and early autosplenectomy [1][2][3][4][5][6][7][8][9][10][11]. Sickle cell disease in the eastern Saudi Arabia is typically associated with the Asian haplotype of b S globin gene and has been reported to be of mild nature with lower incidences of stroke, priapism, nephropathy, fewer vasoocclusive crises, late splenomegaly persistence, late and variable functional asplenia, and lower mortality [1][2][3][4][5][6][7][8][9][10][11].…”

Section: Discussionmentioning

confidence: 99%

“…Multiple responsible factors have been evaluated to explain this progression, the most widely accepted being the occurrence of Asian haplotype of b S globin gene, high levels of fetal hemoglobin (HbF), and frequent a-thalassemia [1][2][3][4][5][6][7][8][9].…”

Section: Introductionmentioning

confidence: 99%

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Fibrocongestive splenomegaly in sickle cell disease: A distinct clinicopathological entity in the Eastern province of Saudi Arabia

Chopra¹,

Al-Mulhim²,

Al-Baharani³

2005

American J Hematol

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Sickle cell disease displays a unique progression in the Eastern province of Saudi Arabia, where splenomegaly with hypersplenism is noted with high frequency in the adolescent and adult patients. The late persistence of splenomegaly although likely reflects the milder progression of sickle cell disease in this region; nevertheless, it predisposes the patients to increased morbidity. The present study documents the characteristic clinicopathological features of splenomegaly associated with sickle cell disease in the Al-Hassa region of Eastern province Saudi Arabia. Forty-four cases of sickle cell disease patients in whom splenectomy was performed during 1999-2003 were studied. The hemoglobinopathy profiles of the patients (age range 5-42 years) comprised sickle cell anemia (8 cases), sickle cell anemia with high fetal hemoglobin (23 cases), and sickle cell-b thalassemia (13 cases). All patients had manifestations of hypersplenism and 39 patients experienced episodes of minor-type sequestration crisis. Splenectomy was effective in ameliorating the hematological abnormalities in all cases, without any major complications in the follow-up period. The splenectomy specimens showed moderate-to-marked enlargement in most cases, with histological features of fibrocongestive splenomegaly and prominent Gandy-gamma body formations. Micro-infarcts in 27 cases and gross infarctions in 9 cases were evident. The relationship of persistent splenomegaly with higher fetal hemoglobin levels and splenic hypofunction is examined along with the significance of splenectomy in these cases. Am.

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Section: Discussionsupporting

confidence: 50%

Section: Discussionmentioning

confidence: 99%

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Fibrocongestive splenomegaly in sickle cell disease: A distinct clinicopathological entity in the Eastern province of Saudi Arabia

Chopra¹,

Al-Mulhim²,

Al-Baharani³

2005

American J Hematol

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“…17 Accurate characterization of this complication become necessary in the Eastern Province of Saudi Arabia where SCD is relatively benign in comparison to other parts of Saudi Arabia and the world. [18][19][20] The frequency of 7.7% is low in comparison to other areas where the frequency may reach over 90%. 5 It was found, as stated in other studies, that ACS was significantly more frequent in SCA than other SCD variants.…”

Section: Discussionmentioning

confidence: 82%

Acute Chest Syndrome in Sickle Cell Disease in Saudi Arab Children in the Eastern Province

Al-Dabbous

2002

Ann Saudi Med

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“…High HbF, 16,17 alpha-thalassemia 17,18 and G6PD deficiency 19,20 have been considered as ameliorating factors in SCD, although disputed by others. 19,21 In this study, lower HbF among those with SNHL with statistically significant difference may suggest a protective effect of this hematologic parameter.…”

Section: Discussionmentioning

confidence: 99%

Sensorineural Hearing Loss in Homozygous Sickle Cell Disease in Qatif, Saudi Arabia

et al. 1996

Ann Saudi Med

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Sensorineural hearing loss (SNHL) is a known complication of sickle cell disease (SCD). There is a paucity of information on this problem in Saudi 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.

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Variability of sickle cell disease in the Eastern Province of Saudi Arabia

Cited by 25 publications

References 11 publications

Fibrocongestive splenomegaly in sickle cell disease: A distinct clinicopathological entity in the Eastern province of Saudi Arabia

Fibrocongestive splenomegaly in sickle cell disease: A distinct clinicopathological entity in the Eastern province of Saudi Arabia

Acute Chest Syndrome in Sickle Cell Disease in Saudi Arab Children in the Eastern Province

Sensorineural Hearing Loss in Homozygous Sickle Cell Disease in Qatif, Saudi Arabia

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