Clustering of cri du chat syndrome among the Bedouins

Fårag, T I; Al-Awadi, S A; Marafie, Makia J.; Bastaki, Lailá; Murthy, D. S. Krishna; Al-Othman, S A; Mohamed, Fawziyah M.; Reda, Amira A.; Hasan, Sawsan J. Abul; Reda, Mariam A.; Al‐Jeryan, L.; Al‐Hashash, Nawal; Al‐Khorafi, Hutham; Haji, Iman A. Q.; Al-Suliman, Ibrahim; Al‐Nagdi, Kadr; Al-Ghanem, Mohammed M.; Usha, R.; Mady, S.A.; Uma, R.

doi:10.1002/ajmg.1320460323

Cited by 3 publications

(4 citation statements)

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“…Our computed figure is considered triple the Danish figure reported by Niebuhr [27]. Our study confirms the clustering of cri-duchat in Kuwait among Bedouins, which is so similar to the study by Farag et al [3]. The other numerical and structural chromosomal abnormality cases are scarce and unreliable for statistical analysis.…”

Section: Discussionsupporting

confidence: 78%

“…Our study included 3 cases of cri-duchat with an incidence of 0.06/1,000 live births, which is higher than the incidence reported by Niebuhr [26]. Other studies carried out in Kuwait reported a similar figure [3,11]. Our computed figure is considered triple the Danish figure reported by Niebuhr [27].…”

Section: Discussioncontrasting

confidence: 49%

“…The difference between the figures reported in the Al-Jahra region and that reported in other regions of Kuwait could be explained by the population pattern in this area (80% Bedouins) and the high rate of consanguineous marriage among those people. The average inbreeding coefficient (™F) of babies with different autosomal trisomies was 0.044, which is higher than that computed from other regions of Kuwait (™F = 0.0219) [2][3][4][5]. In the present study, 2 cases of translocation type of trisomy 21 were reported with an incidence of 0.04/1,000 live births which is close to the incidence reported by Wright et al [15].…”

Section: Discussionsupporting

confidence: 38%

“…Chromosomal analysis was ascertained at the Al-Jahra Hospital, Kuwait, which serves an Arab population of about 300,000, 80% of whom belong to the Bedouin ancestry. Most of the studies on chromosomal abnormalities carried out in the same area were not comprehensive [2][3][4][5][6][7][8]. Classic trisomy 21 and trisomy 18 were the most common trisomies.…”

Section: Introductionmentioning

confidence: 99%

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Profile of Chromosomal Abnormalities in the Al Jahra Region of Kuwait

et al. 1999

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Objective: One hundred and seventy-seven newborn babies with chromosomal abnormalities were studied at the Al-Jahra satellite clinic of the Kuwait Medical Genetics Centre (KMGC), Kuwait, over a 7-year period from January 1983 to December 1989 to make a reanalysis and recomputation of the statistical results of the previous short-term studies. Methods: The data were collected from the registry of chromosomal abnormalities and from the clinical records of the patients in KMGC retrospectively. Controls were selected from the newborn babies delivered in the same period of study. The logistic regression analysis was computed using dependent/independent variables. Results: Ninety-five percent of the cases had numerical chromosomal abnormalities, and 5% had structural chromosomal abnormalities. One hundred and forty-five cases had classic trisomy 21, with an incidence of 2.9/1,000 live births (LB), 16 cases had trisomy 18 (0.3/1,000 LB), 5 cases had trisomy 13 (0.1/1,000 LB), 1 case had triploidy 69, XXY (0.02/1,000 LB) and 1 had Turner’s syndrome (0.05/1,000 female LB). Nine cases with structural chromosomal abnormalities were enumerated, dir dup(1) (p21–p32), del(1)(q32–q42), del(13)(q22–q34) and inversion Y chromosome, the incidence was 0.02/1,000 LB for each. Two cases of translocation trisomy 21 (0.04/1,000 LB) and 3 cases of cri-du-chat (0.06/1,000 LB) were enumerated too. Bedouins had a double-fold increased risk of trisomy 21 (odds ratio = 1.819, p = 0.032), and advanced maternal age was also a risk factor while paternal age showed inconsistent pattern of risk. Conclusion: This study confirmed the high incidence of the common trisomies and the role of advanced maternal age and the Bedouin ethnicity as a risk factor.

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Section: Discussionsupporting

confidence: 78%

Section: Discussioncontrasting

confidence: 49%

Section: Discussionsupporting

confidence: 38%

Section: Introductionmentioning

confidence: 99%

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Profile of Chromosomal Abnormalities in the Al Jahra Region of Kuwait

et al. 1999

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Lachman

2007

Taybi and Lachman's Radiology of Syndromes, Metabolic Disorders and Skeletal Dysplasias

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Disease profile of 400 institutionalized mentally retarded patients in Kuwait

et al. 1993

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Farag TI, Al‐Awadi SA, El‐Badramary MH, Aref MA, Kasrawi B, Krishna Murthy DS, El‐Khalifa MY, Yadav G, Marafie MJ, Bastaki L, Wahba RA, Mohammed FM, Abul Hasan S, Redha AA, Redha MA, Al‐aboud H, Al‐Hijji S, Al‐Dighashem D, Al‐Hashash N, Al‐Jeeryan L, Al‐Khor‐afi H, Qurban EA, Al‐Sulaiman I. Disease profile of 400 institutionalized mentally retarded patients in Kuwait. Clin Genet 1993: 44: 329–334. © Munksgaard, 1993 In this preliminary report we summarize the results of a 4‐year multidisciplinary systematic, etiological clinicogenetic survey of 400 institutionalized mentally retarded patients in Kuwait. All had an intelligence quotient below 50. A constitutional disorder, as the direct cause of the mental retardation, was found in 203 patients (50.75%)): a chromosomal abnormality in 37 (9.25%), Mendelian disorders in 137 (34.25%), MCA/MR in 22 (5.55%) and CNS malformations in 7 cases (1.75%). In 157 patients (39.35%) a pre‐, peri or postnatal cause was ascertained. No etiological diagnosis was detected in 40 patients (10%). A detailed analysis of the “disease profile” is given and compared with the results of previous diagnostic genetic surveys among different institutionalized mentally retarded populations in Western and developing countries.

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Clustering of cri du chat syndrome among the Bedouins

Cited by 3 publications

References 8 publications

Profile of Chromosomal Abnormalities in the Al Jahra Region of Kuwait

Profile of Chromosomal Abnormalities in the Al Jahra Region of Kuwait

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Disease profile of 400 institutionalized mentally retarded patients in Kuwait

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