RESUMO
Cloacal malformations are characterized by the confluence of the lower urinary tract, the female reproductive tract, and the rectum to create a common channel with a single opening on the perineum. The presence of a cloaca is a normal phase of early human embryological development. Between the 4th and 7th weeks of gestation, the cloaca undergoes subdivision to form the hindgut and urogenital sinus. Failure of this process results in the congenital anomaly termed persistent cloaca (PC). The term urorectal septum malformation sequence (URSMS) is also used to describe this anomaly. The classic description of this process which is still cited in many standard textbooks dates from the 19th century. However, this has been increasingly called into question by the findings of studies using modern scientific methodology. Urogenital sinus anomalies are defined by the confluence of the urethra and vagina to form a common channel of varying length with a single perineal opening. In this condition, the anorectal canal opens separately on the perineum. The presence of a urogenital sinus represents a transient phase of the normal development of the lower genital tract in the female fetus. However, the form of urogenital sinus most commonly encountered in the developed world is a feature of disordered sexual differentiation and does not arise simply from the persistence of the anatomical structure which is a feature of normal fetal development. Thomas David 1 University of Leeds, Leeds, West Yorkshire LS2 9JT Hendren WH. Cloaca, the most severe degree of imperforate anus: experience with 195 cases. Ann Surg 1988; 228: 331. Tennant PW, Glinianaia SV, Wellesley D, Draper ES, Kurinczuk JJ, et al. Epidemiology of partial urorectal septum malformation sequence (or 'persistent cloaca'): a population-based study in seven regions of England and Wales, 1985-2010. Arch Dis Child Fetal Neonatal Ed 2014; 99: 413-8. Keppler-Noreuil KM, Conway KM, Shen D, Rhoads AJ, Carey JC, et al. Clinical and risk factor analysis of cloacal defects in the National Birth Defects Prevention Study; National Birth Defects Prevention Study. Am J Med Genet A 2017; 73: 2873-85. Sheldon C, Defoor W. Anorectal malformation and cloaca. The Kelalis-King-Belman Textbook of Clinical Pediatric Urology. 5th ed. London: Informa; 2007. p.1161-86. Levitt MA, Peña A. Cloacal malformations: lessons learned from 490 cases. Semin Pediatr Surg 2010; 19: 128-38. Bischoff A, Levitt MA, Breech L, Louden E, Peña A. Hydrocolpos in cloacal malformations. J Pediatr Surg 2010; 45: 1241-5. van der Putte SC. Normal and abnormal development of the anorectum. J Pediatr Surg 1986; 21: 434-40. Kubota Y, Shimotake T, Yanagihara J, Iwai N. Development of anorectal malformations using etretinate. J Pediatr Surg 1988; 33: 127-9. Kluth D, Hillen M, Labrecht W. The principles of normal and abnormal hindgut development. J Pediatr Surg 1995; 30: 1143-7. Penington EC, Hutson JM. The absence of lateral fusion in cloacal partition. J Pediatr Surg 2003; 38: 1287-95. Huang YC, Chen F, Li X. Clarification of mammalian cloacal morphogenesis using high-resolution episcopic microscopy. Dev Biol 2016; 409: 106-13. Kruepunga N, Hikspoors JP, Mekonen HK, Mommen GM, Meemon K, et al. The development of the cloaca in the human embryo. J Anat 2018; 233: 724-39. Haraguchi R, Mo R, Hui C, Motoyama J, Makino S, et al. Unique functions of sonic hedgehog signaling during external genitalia development. Development 2001; 128: 4241-50. Mo R, Kim JH, Zhang J, Chiang C, Hui CC, et al. Anorectal malformations caused by defects in sonic hedgehog signalling. Am J Pathol 2001; 159: 765-74. Seifert AW, Harfe BD, Cohn MJ. Cell lineage analysis demonstrates an endodermal origin of the distal urethra and perineum. Dev Biol 2018; 318: 143-52. Solomon BD. The etiology of VACTERL association: current knowledge and hypotheses. Am J Med Genet C Semin Med Genet 2018; 178: 440-6. Vilanova-Sanchez, Reck CA, Sebastião YV, Fuchs M, Halleran DR, et al. Can sacral development as a marker for caudal regression help identify associated urologic anomalies in patients with anorectal malformation? J Pediatr Surg 2018; 53: 2178-82. Wilcox DT. The urinary tract in anorectal malformation, multisystem disorders and syndromes. In: Thomas DF, Duffy PG, Rickwood AM, editors. Essentials of Paediatric Urology. 2nd ed. London: Informa; 2008. p.189-98. Fernando MA, Creighton SM, Wood D. The long-term management and outcomes of cloacal anomalies. Pediatr Nephrol 2015; 30: 759-65. Lottman H, Thomas DF. Disorders of sex development. In: Thomas DF, Duffy PG, Rickwood AM, editors. Essentials of Paediatric Urology. 2nd ed. London: Informa; 2008. p.189-98. Schoenwolf GC, Bleyl SB, Brauer PR, Francis-West PH. Larsens Human Embryology. Philadelphia: Elsevier Saunders; 2015. p.413. Robboy SJ, Kurita T, Baskin L, Cunha GR. New insights into human female reproductive tract development. Differentiation 2017; 97: 9-22. Kurita T. Developmental origin of vaginal epithelium. Differentiation 2010; 80: 99-105. Shen J, Cunha GR, Sinclair A, Cao M, Isaacson D, et al. Macroscopic whole-mounts of the developing human fetal urogenital-genital tract: indifferent stage to male and female differentiation. Differentiation 2018; 103: 5-13. Isaacson D, Shen J, Overland M, Li Y, Sinclair A, et al. Three-dimensional imaging of the developing human fetal urogenital-genital tract: indifferent stage to male and female differentiation. Differentiation 2018; 103: 14-23. Li Y, Sinclair A, Cao M, Shen J, Choudhry S, et al. Canalization of the urethral plate precedes fusion of the urethral folds during male penile urethral development: the double zipper hypothesis. J Urol 2015; 193: 1353-60. Overland M, Li Y, Cao M, Shen J, Yue X, et al. Canalization of the vestibular plate in the absence of urethral fusion characterizes development of the human clitoris: the single zipper hypothesis. J Urol 2016; 195(4 Pt 2): 1275-83. Leslie JA, Cain MP, Rink RC. Feminizing genital reconstruction in congenital adrenal hyperplasia. Indian J Urol 2009; 25: 17-26. Hutson JM, Baskin LS, Risbridger G, Cuhna GR. The power and perils of animal models with urogenital anomalies: handle with care.
RESUMO
BACKGROUND: There are little data available on the rates of occurrence, risk factors and mortality due to acute myocardial infarction among the various ethnic groups living in Middle-East countries. Therefore, we did a study to compare Arabs and South Asians living in Kuwait. METHODS AND RESULTS: The data for this retrospective study were collected from the computerized database of the Coronary Care Unit at the Mubarak Al-Kabeer Hospital (a 476-bed teaching hospital) and the 1997-2000 Census data for the State of Kuwait. Arab and South Asian men above 25 years admitted between September 1997 and August 2000 with a diagnosis of acute myocardial infarction were included in the study A total of 866 Arabs and 277 South Asian men were admitted. The rate of admission for the entire patient population was two-fold higher among Arabs as compared with South Asians (6.7/1000 population and 3.3/1,000, respectively). Diabetes mellitus was present in 453 Arabs (52.3%) and 109 South Asians (39.4%) (p< or = 0.001) of those >25 years of age. Hypertension was recorded in 247 Arabs (28.5%) and 57 South Asians (20.6%) (p < or = 0.01). Among patients <55 years of age (454 Arabs and 226 South Asians); the rate of admission was 4.0/1,000 in Arabs and 3.5/1,000 in South Asians (not significant). Hypertension was present in 97 Arabs (21.3%) and 43 South Asians (19%) (not significant). Diabetes mellitus was present in 202 Arabs (44.5%) and 80 South Asians (35.4%) (p < or = 0.05). Smoking was recorded in 353 Arabs (77.8%) and 160 South Asians (70.8%) (notsignificant). Hypercholesterolemia was presentin 182 (40.1%) and 88 (39%), respectively (not significant). While in hospital, 11 Arabs and 9 South Asians died (not significant). CONCLUSIONS: Among men >55 and <75 years of age, Arabs had a higher rate of admission with acute myocardial infarction compared with men of South Asian origin. The incidence of diabetes and hypertension was significantly higher among Arabs in this age group. In younger patients (<55 years), the rate of acute myocardial infarction was not different between the two groups; however, diabetes was present more often among Arabs. The smoking rate is very high in both groups and is an important risk factor for both Arab and South Asian men living in the Middle East.