ABSTRACT
OBJECTIVE: This study was carried out to identify the trend and the frequency of neural tube defects from July 1998 to June 2004. METHODS: A total of 310 babies were born with neural tube defects with the overall frequency of 5.7/1000 births compared to 2.3/1000 births observed earlier in our hospital. RESULTS: The most common defect was spina bifida (54.8%) followed by anencephaly (31.6%), and encephalocele (11.6%). More neural tube defects were observed in female and low birth weight babies, still births and unbooked mothers. Neural tube defect was significantly higher among babies born to parents of consanguineous marriage (p< 0.01). Associated congenital defects were observed in thirty nine (12.6%) cases. CONCLUSION: The rise in the frequency of NTDS may indicate the current trend of NTDs in Southern India. A further prospective study is desired to measure the effectiveness of regular folic acid supplementation in bringing down this frequency.
Subject(s)
Consanguinity , Female , Humans , India/epidemiology , Infant, Low Birth Weight , Infant, Newborn , Male , Mothers , Neural Tube Defects/classification , Pregnancy , Prenatal Care , Risk FactorsABSTRACT
Neural tube defects [NTD] are one of the most important malformations of newborns. The aim of this research is to determine the rates of NTD, anencephaly and spina bifida cystica and the relations of these abnormalities to the factors including sex, race, ethnicity, maternal age and familial marriage in the south-east of the Caspian Sea border. A three-year descriptive-analytic and cross-sectional study was carried out on 19545 newborns delivered in Dezyani hospital in Gorgan during 1998-2000. From the total sample population [19545], the NTD rate was 3.12 per 1000 deliveries. In males the rate was 2.49 per 1000, in females 3.79 per 1000, and the female to male ratio was 1.44:1. The rate of anencephaly and spina bifida was 1.25 per 1000 and 1.74 per 1000 respectively. In regard to different races the NTD rate was 7.48 per 1000, 9.5 per 1000, and 2.35 per 1000 among Turkmans, Sistanis and Farses, respectively. It can be concluded that there is a higher rate of NTD in this region that may be due to the race-ethnicity and the ecological variations
Subject(s)
Humans , Male , Female , Neural Tube Defects/classification , Anencephaly , Spinal DysraphismABSTRACT
Spinal lipomatous malformations (SLM) include all the closed neural tube defects (NTD) with excessive lipomatous tissue in the spinal cord or filum terminale. We evaluated 65 cases of SLM seen & operated at our department in the last 7 years. Of these only 9 were asymptomatic and 8 were below 2 years of age. There were more males than females. In addition to subcutaneous lipoma many patients also had hypertrichiosis and dermal sinus as cutaneous makes. Twenty patients had foot deformity and 5 had unilateral limb shortening. Sixty-two patients had MRI and 3 had CT myelogram for evaluation. These revealed 7 patients with Chiari malformations, 10 with focal syrinx and 2 patients underwent VP shunt for hydrocephalus. Sacral agenesis was seen in 5 patients. Clinical features were similar to other cases of spinal dysraphism except that sensory loss and trophic ulcer were more frequent. Intradural lipoma and tethering was seen in 18 cases whereas intramedullary lipoma & conus lipoma was seen in 40 & 7 cases respectively. Additional tethering lesion was seen in 1/3 cases and was treated simultaneously. Preoperative deficits improved in 28 cases and stabilized in 33 cases. Three patients developed fresh deficits after surgery. We observed 8 CSF leaks and 4 wound infections in postoperative period. It is very clear from our data that a patient has about 95% chances that his neurological status may improve or stabilize following surgery and the risk of developing fresh deficits is about 5%. We, therefore, suggest that all patients of SLM should be treated with aggressive surgical management for best results.
Subject(s)
Adolescent , Adult , Child , Child, Preschool , Female , Humans , Infant , Lipoma/surgery , Male , Middle Aged , Neural Tube Defects/classification , Postoperative Complications , Prognosis , Risk FactorsABSTRACT
El término "Disrrafismo" expresa cierre defectuoso del tubo neural en la primeras 2 a 3 semanas del estadío embrionario, debido a múltiples factores genéticos que pueden involucrar antecedentes familiares, elementos ambientales o nutricionales en su etiología. Se hace necesario una actualización de dichas patologías, su repercusión clínica y las técnicas quirúrgicas a emplearse para preservar o mejorar el daño neural establecido. De igual manera, hacer conciencia que éstas afecciones implican un equipo multidisciplinario que logre una mejor calidad de vida al paciente y apoyo a sus familiares para salir adelante en ésta dificil tarea. Se revisa el estado actual del disrrafismo en el Hospital Escuela de Tegucigalpa, Honduras
Subject(s)
Neural Tube Defects/classification , Neural Tube Defects/diagnosis , Neural Tube Defects/therapyABSTRACT
Las estadísticas nacionales de la mortalidad perinatal se revisaron entre el quinquenio 1973-77 en la Maternidad Concepción Palacios y se halló 47 casos con defecto del tubo neural y se estudiaron las variables sexo, edad gestacional, edad materna. Los defectos fueron anencefalia 40,81 por ciento , hidrocefalia: 27,21 por ciento ; espinas bífidas con o sin hidrocefalia: 19,72 por ciento , anomalías diversas (iniencefalia, ciclopia, cebocefalia, etc.)
Subject(s)
Infant, Newborn , Humans , Male , Female , Neural Tube Defects/classification , Neural Tube Defects/epidemiology , Perinatal MortalityABSTRACT
Myelodysplasia, characterized by varied reductions of peripheral blood elements with normal or hypercellular bone marrow, is reltively frequent among older patients and may evolve to acute leukemia. We reviewed findings in 35 patients whon, according to the FAB classification were distributed as follows: simple refractory anemia (RA) 34%, sideroblastic refractory anemia (SRA) 14%, refractory anemia with excess blast forms (RAEB) 31%, chromic myelomonocytic leukemia (CMML) 12% and refractory anemia eith excess blast forms in transformation (RAEBT 9%). Cytogenetic studies performed in 16 patients were abnormal in 5(31%), al among patients with poor prognosis forms of the disorder. All patients had anemia; thrombopenia and neutropenia were more frequent in subtypes RAEB, CMML and RAEBT). Mean survival rate was 30 months, significantly greater in RA and SRA comapred to the other groups. Infections and development of acute leukemia were the causes of death