A One-Year Study of the Epidemiology and Outcomes of Various Birth Defects in Pediatric Patients at Georgetown Public Hospital Cooperation

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Authors : Dr. Unarain

Abstract:

 BACKGROUND

Although, birth defects are one of the leading causes of infant deaths and long term disabilities and have contributed significantly to the global burden of diseases, extensive researches have not been carried out in Guyana.

METHODS

A retrospective study, of 138 patients, diagnosed with Congenital Malformations at Georgetown Public Hospital Cooperation, between January 2007- December 2007was carried out. These patients’ medical records; inclusive of infant, neonatal intensive care and postnatal; were located and information was recorded in the form of a questionnaire which had structured and detailed parameters. Exclusion criteria were records for all stillborns, pregnanciesterminated at or after 20 weeks of gestation, out- patients and or patients in specialized clinics / institutions. Analysis on various variables (demographic distribution, clinical outcomes, clinical presentation, Apgar score, birth weight, maternal age, sex, ethnicity, previous infant with a birth defect, family history of a birth defect, known teratogenic exposure, maternal medical history, lack of folic acid supplementation and average length of hospital stay) was done using Microsoft Office Excel version 2003 for information on the epidemiology, management and clinical outcomes of the condition in the Guyanese population.

RESULTS

I found an estimate incidence 2240 cases of birth defects occurring annually in Guyana. The majority of cases were from Regions 4 (46.8%) and 3 (10.9%). The majority (73.9 %) of cases was seen in the age group birth-5 months, followed by (9.4%) > 50 months (>12 years old), 6-10 month (5 %) and 21-25 month (4.4 %). There was a male to female ratio of 1.3: 1 of having a birth defect. Afro-Guyanese accounted for almost one half (48%) of the cases followed by Indo-Guyanese, Mixed and Amerindians. The most common types of birth defects were congenital heart disease, Hirshsprung disease, hypospadia, cryptorchidism, accessory breast, club feet and syndactyl. The average length of stay was 6.5 days. One mother used alcohol and another used an abortificient during the current pregnancy. 82% of patients underwent surgery while 18% had medical interventions. Few patients had laboratory testing done. Patients generally had excellent clinical outcomes given a mortality rate of 0 %.

CONCLUSION

My findings highlight the clinical and public health importance that should be placed on Congenital Malformations in Guyana and to re-evaluate our approach to the condition and to introduce nation-wide screening practices.

References:

[1.] Akre, O., Boyd, H. A., Ahlgren, M. et al (2008). Maternal and gestational risk factors for hypospadias. Environ Health Perspect, 116(8), 1071-6.

[2.] Beers, M., Berkow (1999). The Merk Manual. N.J: Merk Research Laboratories.

[3.] Chiquet BT, Hashmi SS, Henry R et al. Genomic screening identifies novel linkages and provides further evidence for a role of MYH9 in nonsyndromic cleft lip and palate. Eur J Hum Genet.2008 Aug 20.

[4.] D., Kim, Waller, Gary, M., Shaw, Sonja, A., Rasmussen, et al (2007). Prepregnancy Obesity as a Risk Factor for Structural Birth Defects. Arch PediatrAdolesc Med, 161(8),745-750. pp. 559-563

[5.] Dorothy, M., Sendelbach, Gregory, L., Jackson, Susanna, S., Lai (2000). Pulse Oximetry Screening at 4 Hours of Age to Detect Critical CongenitalHeart Defects.

[6.] Famà, F., Gioffrè, Florio, M., A., Villari SA et al(2007). Breast abnormalities: a retrospective study of 208 patients. Chir Ital. Jul-Aug;59(4):499-506

[7.] Fleming, A., Poonai, N., Yang, C., et al. Toronto Notes. Paediatrics, 2004.

[8.] Familial ankyloglossia (tongue-tie). Int J PediatrOtorhinolaryngol.2007 Aug;71(8):1321-4. Guyana (webpage on Guyana Profile).

[9.] H.,E., Virtanen and J. Toppari (2008). Epidemiology and pathogenesis of cryptorchidism. Human Reproduction Update 14(1):49-58.

[10.]   Jeanne L. Ballard, Christine E. Auer and Jane C. Khoury.Ankyloglossia: Assessment, Incidence, and Effect of Frenuloplasty on the Breastfeeding Dyad.

[11.]   Jun Zhang, M. B.1 and Wen-wei, Cai (1993). Association of the Common Cold in the First Trimester of Pregnancy WithBirthDefects. . PEDIATRICS Vol. 92 No. 4.

[12.]   Kalfa, N., Philibert, P., Sultan, C(2008). Is hypospadias a genetic, endocrine or environmental disease, or still an unexplained malformation? Int J Androl..

[13.]   Kalfa, N., Liu, B., Klein, O. et al(2008). Mutations of CXorf6 are associated with a range of severities of hypospadias. Eur J Endocrinol.

[14.]   Kawahara H, Kubota A, Hasegawa T et al. Anorectal sleeve micromanometry for the iagnosis of Hirschsprung's disease in newborns. J Pediatr Surg.2007 Dec;42(12):2075-9.

[15.]   Little J, Gilmour M, Mossey PA et al. Folate and clefts of the lip and palate--a U.K.-based case-control study: Part II: Biochemical and genetic analysis. Cleft Palate Craniofac J. 2008 Jul;45(4):428-38.

[16.]   Malik, S., Cleves, M., Zhao, W., et al (2007). DefectsAssociation Between Congenital Heart and Small for Gestational Age. PEDIATRICS,119(4), 976-982.

[17.]   Peg Nopoulos, Douglas R. Langbehn, John Canady et al. Abnormal Brain Structure in Children With Isolated Clefts of the Lip or Palate. Arch PediatrAdolesc Med. 2007;161(8):753-758.

[18.]   Sato, D., Liang, D., Wu, L. et al(2007). A syndactyly type IV locus maps to 7q36. J Hum Genet,52(6),561-4

[19.]   Solanki RChoksi DBDuttaroy DD. Accessory breast tissue presenting as a large pendulous mass in the axilla: a diagnostic dilemma. N Z Med J. , , 2008 Jul 4;121(1277):76-8.

[20.]   Sheldon P. Rothenberg, Maria P. da Costa, Jeffrey M. Sequeira et al. Autoantibodies against Folate Receptors in Women with a Pregnancy Complicated by a Neural-Tube Defect. J Pediatr Surg. 2007 Dec;42(12):2075-9.

[21.]   Suzan, L., Carmichael, Gary, M. Shaw, Cecile, Laurent, et al(2005). 21) Maternal Progestin Intake and Risk of Hypospadias. Arch PediatrAdolesc Med,159,957-962.

[22.]   Thai, H. T., Söderhäll, C., Lagerstedt, K., Omraniet, al (2008). A new susceptibility locus for hypospadias on chromosome 7q32.2-q36.1.Hum Genet. .