The aim of this prospective study was to evaluate the prevalence and pattern of gastrointestinal malformations (GIM) among Iranian newborns in Gorgan, North of Iran. From 1998 through 2003, 37,951 live births in Dezyani hospital in Gorgan, North of Iran, were screened for gastrointestinal malformations. Clinical and demographic factors of diagnosed cases were recorded in a pre-designed questionnaire for analysis; sex, ethnicity, type of GIM and associated anomalies. The overall prevalence rate of gastrointestinal malformations was 10 per 10,000 births. The imperforate anus (5 per 10,000) was the commonest birth defect in gastrointestinal tract. The prevalence rate of GIM was 8.2 per 10,000 in males and 10.7 per 10,000 in females. According to the parental ethnicity, the prevalence rates of GIM were 6.7, 15.8 and 17.6 per 10,000 in Fars, Turkman, and Sistani, respectively. There were eight cases (21%) with associated anomalies. The prevalence rate of GIM in North of Iran is not similar to the previous studies in Iran and Middle East and ethnic background may be a causative factor in the rate of GIM in this area. © 2006 Springer-Verlag

Golalipour, M.J.

Hoseinpour, K.-R.

Keshtkar, A.A.

Mobasheri, E.

Golestan University of Medical Sciences Repository

Pediatr Surg Int (2007) 23:75–79 DOI 10.1007/s00383-006-1814-5 ORIGINAL ARTICLE Gastrointestinal malformations in Gorgan, North of Iran: epidemiology and associated malformations Mohammad Jafar Golalipour Æ Elham Mobasheri Æ Kaniz-Reza Hoseinpour Æ Abbas Ali Keshtkar Accepted: 26 September 2006 / Published online: 17 October 2006 © Springer-Verlag 2006 Abstract The aim of this prospective study was to evaluate the prevalence and pattern of gastrointestinal malformations (GIM) among Iranian newborns in Gorgan, North of Iran. From 1998 through 2003, 37,951 live births in Dezyani hospital in Gorgan, North of Iran, were screened for gastrointestinal malformations. Clinical and demographic factors of diagnosed cases were recorded in a pre-designed questionnaire for analysis; sex, ethnicity, type of GIM and associated anomalies. The overall prevalence rate of gastrointes­tinal malformations was 10 per 10,000 births. The imperforate anus (5 per 10,000) was the commonest birth defect in gastrointestinal tract. The prevalence rate of GIM was 8.2 per 10,000 in males and 10.7 per 10,000 in females. According to the parental ethnicity, the prevalence rates of GIM were 6.7, 15.8 and 17.6 per 10,000 in Fars, Turkman, and Sistani, respectively. There were eight cases (21%) with associated anoma­lies. The prevalence rate of GIM in North of Iran is not similar to the previous studies in Iran and Middle East and ethnic background may be a causative factor in the rate of GIM in this area. Keywords Gastrointestinal malformations · Prevalence · Ethnicity Introduction So far, a comprehensive report focusing speciﬁcally on congenital anomalies of gastrointestinal tract, in Iran, has not yet been documented. In some previous gen­eral surveys performed in a short period on congenital malformations in Tehran and Gorgan, North of Iran, the gastrointestinal system was found to be one of the important systems affected [1–3]. The majority of infants need surgery in the neonatal period in order to survive [4]. A high proportion of infants with gastrointestinal malformations (GIM) have associated malformations [5] and mortality rate among these infants is higher than those infants with isolated GIM [6, 7]. Although there were some reports on congenital anomalies of gastrointestinal tract in the world [4, 8– 10] there is no focused study in Iran and North of Iran. Therefore, this study was done to determine the prevalence pattern of congenital lesions, associated anomalies and ethnicity among Iranian newborns in Gorgan, North of Iran; South-East of Caspian Sea border. M. J. Golalipour (&) · E. Mobasheri · K.-R. Hoseinpour · A. A. Keshtkar Gorgan Congenital Malformations Research Center, Gorgan University of Medical Sciences, P.O. Box 49175-553, Gorgan, Iran e-mail: mjgolalipour@yahoo.com Patients and methods This investigation was undertaken on 37,951 live births, to identify all newborns with gastrointestinal malfor­mations, born between January 1, 1998 and December 31, 2003, in Dezyani—a teaching hospital and a referral center which is the main site for about 70% of deliv­eries in Gorgan—a capital city in Golestan province in the north of Iran. All live newborns delivered in this hospital during the investigation were examined and 123 76 Pediatr Surg Int (2007) 23:75–79 screened for GIM including atresia (Esophageal and Anal atresia), Gastroshisis, Omphallocell, Umbelical hernia and diaphragmatic hernia. The charts of newborns with GIM were subse­quently extracted for detailed study. Variables re­corded included: the date of birth, sex, type of malformations, ethnicity of parents and the presence of other congenital malformations. The total number of live ﬁgures of every year was recorded. According to their ethnicity, three ethnic groups (Native Fars, Turkman and Sistani) who are residing in this region were considered. The native Fars group is the predominant inhabitant of the region. The Turk-mans are those who have emigrated from the central Asia since 250 years ago. The Sistani group emigrated from Iran–Pakistan–Afghanistan border (Sistan and Baluchestan province in South-East of Iran) since half a century ago. Statistical methods Descriptive data are presented as percentages. Descriptive statistics was calculated for GIM preva­lence per 10,000 live births. The prevalence of GIM is calculated as follows: The GIM cases Annual rate = : The total live birth Conﬁdence interval (CI 95%) for prevalence was calculated by binomial exact methods. Chi-square for trend test was used to assess trend over time. STA­TA 8/SE statistical package was used for statistical analysis. Results A total of 38 newborns with 40 GIM, were registered during the study period, giving a prevalence of 10 cases (CI 95% 7.1–13.7) per 10,000 births and 10.54 (CI 95% 7.5–14.4) malformations per 10,000 births. In two cases, there were more than one GIM and both of them in­cluded anal Atresia. The prevalence of GIM was 11.39 per 10,000 (7.1– 17.1) and 8.71 per 10,000 (5–14.1) during the two periods 1998–2000 and 2001–2003, respectively (Fig. 1). The prevalence of GIM in males and females was 8.3 per 10,000 and 10.8 per 10,000, respectively. Risk of GIM in female was 1.3 times more than male (odds ratio = 1.3) but this difference was not signiﬁcant (CI 95% 0.7–2.5) (Table 1). 10 88.16.5 8.5 19.1 0 5 10 15 20 25 1998 1999 2000 2001 2002 2003 year per 10000 Fig. 1 Prevalence of gastrointestinal malformations in North of Iran 1998–2003. Rate per 10,000 birth trend time Anal Atresia with prevalence rate of 5 per 10,000 was the most common GIM malformations. This prevalence rate was 3.1 (1.1–6.7) per 10,000 and 6.46 (3.3–11.3) per 10,000 in males and females, respec­tively. The prevalence rate of gastrointestinal malfor­mations is depicted in Table 1. Risk of anal atresia in females was 2.1 times more than males (odds ra­tio = 2.1) but this difference was not signiﬁcant (CI 95% 0.7–6.7) (Chi-square = 2.3, P = 0.3). The prevalence of GIM was 6.7 per 10,000 births in the native Fars community, 15.8 per 10,000 in the Turkmans and 17.6 per 10,000 in Sistanis. Risk of GIM in Turkman and Sistani ethnicity groups were 2.4 (CI 95% for odds ratio 1–5.6) and 2.6 (CI 95% for odds ratio 1.2–5.8) times more than native Fars population. Twenty-one percent of the newborns with GIM had associated major anomalies including neural tube de­fects (two cases), Limb defects (four cases), cleft palate (one case) and epispadiases (one case). Discussion This investigation was undertaken to establish the prevalence rate of newborns with gastrointestinal malformations in Gorgan, in North of Iran. This study Table 1 Total number of malformations in newborns with GIM, prevalence per 10,000 and proportion of cases with isolated gastrointestinal malformation Number Prevalence Proportion with per1,0000 isolated GM (%) Esophageal atresia 4 1 4 (100) Anal atresia 19 5 12 (63) Diaphragmatic hernia 4 9 4 (100) Omphalocel 7 1.84 6 (86) Gastroshisis 4 9 4 (100) Umbelical hernia 2 0.53 2 (100) 123 77 Pediatr Surg Int (2007) 23:75–79 was limited to newborns with defects that were clini­cally symptomatic during the newborn age bracket. It undoubtedly misses out some newborns whose con­genital lesions, though present at birth, were silent or could have been misdiagnosed during the neonatal period. By considering all these factors, our ﬁgures on birth prevalence are very likely to be an underestimation of the real situation. Besides, since Dezyani hospital is the only referral center in this region, it can be assumed that this center had attracted the bulk of Iranian newborns born with GIM anomalies in this area. Therefore, it is hoped that the results of this study demonstrate a clue, regarding the pattern of congenital gastrointestinal tract lesions in this area and perhaps in the entire Iran. In this report, the prevalence of GIM was 10 cases per 10,000 births. Review of the literature did not re­veal any investigations that reported the prevalence of GIM in separate long time study focused on GIM in Iranian population. There have been some studies on congenital mal­formations that pointed out on GIM [1–3] that these investigations have been done in short period and limited population. The results of the present investigation are higher than Farhouds’ study [1] in Tehran with a prevalence rate of 6.9 per 10,000, but lower than another study in Tehran [2] with a prevalence rate of 39 per 10,000 and our previous study with prevalence rate of 17 per 10,000 [3]. In addition, our results are lower than a similar study in Saudi Arabia, with prevalence rate of 13 per 10,000 live births [8] and higher than other two studies in Egypt (6.6 per 10,000) and Bahrain (9 per 10,000) [11, 12]. Also, in a study in Denmark [4], the preva­lence rate of GIM was 15.3 per 10,000 in all births. Prevalence rate of GIM according to the types of malformations as reported in literature is depicted in Table 2. The difference between the results of different investigations in various parts of the world may be due to racial/ethnic, geographic, nutritional differences and also may be owing to difference in some place selec­tions, data sources including live birth, still birth, abortion; classiﬁcation and deﬁnition of GIM type and design of the study. In this study, the prevalence rate of Atresia (esophageal and anal Atresia) was 6 per 10,000, which is lower than other studies in Saudi Arabia (12 per 10,000), in Denmark with 9.1 per 10,000 and in Europe with 7.6 per 10,000 [4, 5, 8]. The prevalence rate of anal Atresia was 5 per 10,000, which is lower than Asindi’s study (6.1 per 10,000) in Saudi Arabia [8] and higher than other studies in Iran and in Denmark with 3.8 per 10,000 [1, 4] and in European countries with 4.05 per 10,000 [9]. The birth prevalence of anorectal malformation (5 per 10,000) observed in our region is nearly 2.5 times higher than the global ﬁgure with prevalence rate of 1:5,000 live births [13]. Other studies have shown a prevalence rate of esophageal Atresia of 4.1 per 10,000 [4], 2.8 per 10,000 [14], and 3 per 10,000 [8]. All these prevalence rates are higher than that found in our study. But the above-mentioned difference may be due to regional proper­ties such as Saudi Arabia and design of study such as in Denmark that included data from induced abortions. In this study, the prevalence rate of diaphragmatic hernia, omphalocell and gastrochisis were, 1.05, 1.85 and 1.05 per 10,000, respectively. Other studies have shown a prevalence of diaphragmatic hernia of 2.4 per 10,000 [15], 2.1 per 10,000 [16], omphalocell 2.5 per 10,000 [17], 3 per 10,000 [4] and gastoschisis with 0.9 per 10,000 [17] and 1.3 per 10,000 [4]. The cause of low prevalence rate in our study in comparison with other studies [15–17] is due to sample setting, because these studies include data from live, stillbirth and induced abortions, but in our study only live births were considered. Table 2 prevalence rate of GIM according to the types as reported in literature Location Time span Esophageal Anal Omphalocel Gastroshisis Diaphragmatic Total of study atresia atresia hernia Asindi et al. [8] Saudi Arabia 1990–1995 3.28 6.1 – – – 13 Farhud et al. [1] Tehran Iran 1969–1977 1.53 3.8 – – 0.8 6.9 Garne et al. [4] Funen country Denmark 1980–1993 4.1 3.8 3.0 1.3 2.7 15.3 Galzolari et al. [17] European Country 1980–1990 – – 2.5 0.9 – – Cuschieri [9] Europe 1980–1994 – 4.05 – – – – Present study Gorgan, Iran 1998–2003 1.05 5.01 1.85 1.05 1.05 10 123 78 Pediatr Surg Int (2007) 23:75–79 Females are more affected than males. The female preponderance is seen in this survey in contrast with other researches [8, 18–20]. In this study, associated anomalies were seen in 21% of cases, but this rate was 35% in Garne’s study in Denmark [4] and 22.7% in Saudi Arabia [8]. In present study, the commonest associated anom­alies were limb anomalies, but in Garne’s study in Denmark and Asindi’s study in Saudi Arabia, it was cardiac malformations and Down’s syndrome, respec­tively [4, 8]. Furthermore, another study was done in European countries on anorectal anomalies a long with other anomalies or as part of syndrome indicated al­most 15% of cases that were chromosomal, monogenic or teratogenic syndromes, while the rest were of un­known causes including sequences (9.3%), VACTERL associations (15.4%) and multiple congentital anoma­lies (MCA) (60.2%) [10]. According to the ethnic factor the prevalence rate of GIM in Sistani group was higher than the other groups in this area. Although, a study in Hawaii did not ﬁnd any relation between Anal atresia and ethnicity [21], but in other studies on the other congential malfor­mations, it is shown that the ethnic factor could be an effective one in prevalence rate of malformations [3, 22–25]. High prevalence rate reported in Sistani ethnic group could also be not only due to ethnicity but also to the cultural habits or practices, linked to these particular ethnic groups. While, according to Asindi’s ﬁndings in Arab population, the high prevalence of anorectal malformations in Saudi Arabia is due to inbreeding [8]. These differences in GIM risk between racial/ethnic groups within a given population may be due to dif­ference in genetic factor or environmental risk factor associated with birth defects. Conclusion The present study showed the prevalence rate of GIM among Iranian newborns in Gorgan, in north of Iran, for the ﬁrst time. These ﬁndings will assist in estab­lishing the basis for a database in this area, but for determining the difference rate between ethnic groups and for surveying the etiological factors, further studies are required. Acknowledgment We would like to thank the personnel of Dezyani hospital, Dr Arezoo Mirfazeli, Mrs. Hajezade, Mrs Hajilari, Mrs Najdaghi, Mrs Hajimashhadi and Mrs Sharifei for typing the manuscript and special thanks to Dr Ali Rayej and Dr Ali Reza Khoshbin for their valuable guidance. References 1. Farhud DD, Walizadeh GR, Kamali MS (1986) Congenital malformations and genetic diseases in Iranian infants. Hum Genet 74(4):382–385 2. Arbabi AH, Babak D (1991) The study of major anomalies in newborns. In: Proceedings of the tenth pediatric congress. Tehran, Islamic Republic of Iran, pp 583–590 3. Golalipour	 MJ, Ahmadpur-Kacho M, Vakili MA (2005) Congenital malformations at a referral hospital in Gorgan, Islamic Republic of Iran. East Mediterr Health J 11(4):707–715 4. Garne E, Rasmussen L, Husby S (2002) Gastrointestinal malformations in Funen contry, Denmark-epidemiology, associated malformation, surgery and mortality. Eur J Pe­diatr Surg 12:101–106 5. Harris J, Kallen B, Robert E (1995) Descriptive epidemiol­ogy of alimentary tract atresia. Teratology. 52:15–29 6. Rankin J, Dillon E, Wright C et al (1999) Congential ante­rior abdominal wall come. Prenat Diagn 19:662–668 7. Spitz	 L, Kiely EM, Morecroft JA, Drake DP (1994) Oesophageal atresia: at-risk groups for the 1990s. J Pediatr Surg 29:723–725 8. Asindi AA, Al-Daama SA, Zayed MS, Fatinni YA (2002) Congenital malformation of the gastrointestinal tract in Aseer region, Saudi Arabia. Saudi Med J 23(9):1078–1082 9. Cuschieri A (2001) EUROCAT Working Group. Descrip­tive epidemiology of isolated anal anomalies: a survey of 4.6 million births in Europe. Am J Med Genet 103(3):207–215 10. Cuschieri A (2002) EUROCAT Working Group. Anorectal anomalies associated with or as part of other anomalies. Am J Med Genet 110(2):122–130 11. Temtamy SA et al (1998) A genetic epidemiological study of malformations at brith in Egypt. East Mediterr Health J 4(2):252–259 12. Al Arrayed SS (1995) Epidemiology of congenital abnor­malities in Bahrain. East Mediterr Health J 1(2):248–252 13. Wesson DE, Haddock G (1996) The intestines: congenital anomalies. In: Walker WA, Durie PR, Hamilton JR, Walker-Smith JA, Watkins JB (eds) Pediatrc gastrointestinal disease. 4th ed. Mosby Boston, MA pp 5–563 14. Torfs CP, Curry CJR, Bateson TF (1995) Population-based study of tracheoesophageal ﬁstula and esophageal atresia. Teratology 52:220–232 15. Roberts E, Kallen B, Harris J (1997) The epidemiology of diaphragmatic hernia. Int J Epidemiol 13:665–673 16. Garne E, Quataert P, de Vigan C et al (1999) Congenital diaphragmatic hernia: a European population-based study of epidemiology, prenatal diagnosis and mortality. Prenat Ne­onat Med 4:441–447 17. Galzolari E, Bianchi F, Dolk H, Milan M, Eurocat Working Gorup (1995) Omphalocele and gastroschisis in Europe: a survey of 3 million births 1980–1990. Am J Med Genet 58:187–194 18. Hegazy IS, Al-Beyari TH, Al-Amar AH, Qureshi NA, Ab­delgdir MH (1995) Congenital malformations in primary health care in Al-Qassim Region. Ann Saudi Med 15:48–53 19. Mir NA, Galczek WC, Soni A (1992) Easily identiﬁable malformations in children: survey of incidence and pattern in 312332 live born neonates. Ann Saudi Med 12:366–371 20. Peckham CS, Ross EM, Farmer RDT (1982) Congenital malformations. In: Miller DL, Farmer RDT (eds) Epidemi­ology of diseases, 3rd ed. Blackwell London, pp 425–465 21. Forrester MB, Merz RD (2002) Descriptive epidemiology of anal atresia in Hawaii, 1986–1999. Teratology 66(Sup­pl1):S12–S16 123 79 Pediatr Surg Int (2007) 23:75–79 22. Forrester MB, Merz RD (2004) Descriptive epidemiology of oral clefts in a multiethnic population, Hawaii, 1986–2000. Cleft palate Craniofac J 41(6):622–628 23. Amidei	 RL, Hamman RF, Kassbaum DK, Marshall JA (1994) Birth prevalence of cleft lip and palate in Colorado by sex distribution, seasonality, race/ethnicity, and geographic variation. Spec Care Dent 14:233–240 24. Amaratunga AN, Chandrasekera A (1989) Incidence of cleft lip and palate in Sri Lanka. J Oral Maxillofac Surg 47(6):559– 561 25. Ray JG,	 Vermeulen MJ, Meier C, Cole DE, Wyatt PR (2004) Maternal ethnicity and risk of neural tube defects: a population-based study. CMAJ 171(4):343–345 123 

Gastrointestinal malformations in Gorgan, North of Iran: Epidemiology and associated malformations

Abstract

Similar works

Full text

Available Versions

Golestan University of Medical Sciences Repository