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Hirschsprung's disease: associated abnormalities and demography.

We here examine the demographic parameters of patients with Hirschsprung's disease. The study population includes all patients with histologically confirmed disease treated at the Children's Hospital Medical Center of Boston over the 25-year period extending from 1961 through the first quarter of 1986. There were 179 cases. Overall, children with Hirschsprung's disease were found less likely to be first born (P less than .01). This relationship was seen to persist irrespective of maternal age, maternal race, or type of disease. The implications of this finding are discussed. Overall, 22% of these children had one or more associated abnormalities involving the neurological, cardiovascular, urological, and gastrointestinal systems. Many of the disorders appeared to be related to neurocrestopathies. Frequent associations included Down's syndrome, defects in cardiac septation, tetralogy of Fallot, and Dandy-Walker syndrome. These conditions occurred more frequently than would have been predicted through chance alone. This study found no association between an increased maternal age and the occurrence of Hirschsprung's disease. This study also found that approximately 7% of the affected children had been born prematurely.

Abnormalities, Multiple↗

High prevalence of glucose intolerance even among young adults in south India.

India is experiencing an epidemic of Type 2 diabetes mellitus (DM) in young adults. This study reports the prevalence of glucose intolerance, and insulin profiles, and their relationship to lifestyle factors in 2218 young adults (aged 26-32 years; 997 urban, 1221 rural) in south India. They were drawn from a cohort of 10,691 individuals born during 1969-1973 in Vellore and nearby villages. Family history, socio-economic status, physical activity and tobacco and alcohol use were recorded. Oral glucose tolerance tests were performed for diagnosis (WHO recommendations). Insulin resistance and secretion were derived from plasma insulin concentrations. Median BMI was 20.0kg/m(2). The prevalence of Type 2 DM and impaired glucose tolerance (IGT) was higher in urban than in rural subjects (3.7% versus 2.1%, p=0.02; 18.9% versus 14.3%, p=0.002, respectively), while prevalence of impaired fasting glycaemia (IFG) was similar in urban and rural populations (3.8% versus 3.4%, p=0.04). Type 2 DM, IGT, IFG or higher insulin resistance and increment were associated with higher socio-economic status (more household possessions) and higher percentage body fat, body mass index and waist/hip ratio. Insulin increment was lower in men with higher alcohol consumption. Our data suggest high levels of glucose intolerance in young rural and urban adults highlighting an urgent need for preventive action to avert a public health catastrophe in India.

Adult↗

First cesarean birth and subsequent fertility.

OBJECTIVE: To determine whether cesarean delivery is independently associated with later subfertility. DESIGN: Retrospective cohort study. SETTING: Maternity records kept for Scotland, 1980-1999. PATIENT(S): The study included 109,991 women who had first births between 1980 and 1984, excluding multiple or preterm births and perinatal deaths. INTERVENTION(S): Exposures studied were spontaneous vaginal birth, operative vaginal birth, planned cesarean delivery for breach presentation, planned cesarean delivery for other indications, and emergency cesarean delivery. MAIN OUTCOME MEASURE(S): The relative risk of not having a second pregnancy over the following 15 years, the interpregnancy interval, and the number of spontaneous early pregnancy losses between the first and second birth. RESULT(S): Women who delivered by planned cesarean section for breech presentation had an increased risk of not having a second birth compared with women whose first birth was a spontaneous vertex delivery (relative risk [RR]: 1.21, 95% confidence interval [CI]: 1.14 to 1.29). However, after adjustment for maternal and obstetric characteristics, there was no longer a strong association (adjusted RR: 1.07, 95% CI: 1.00 to 1.15). Operative vaginal delivery (forceps and vacuum extraction) and all types of cesarean delivery were associated with longer interpregnancy intervals. There was no relationship between mode of delivery and the number of spontaneous early pregnancy losses between the first and second birth. CONCLUSION(S): It is unlikely that delivering by cesarean section in a first pregnancy decreases a woman's likelihood of having a second viable pregnancy.

Abortion, Spontaneous↗

Observed frequency of monozygotic twinning in Holstein dairy cattle.

Bonnier's equation is used to mathematically estimate the frequency of monozygotic (MZ) twinning in epidemiologic studies of twinning in dairy cattle; however, no empirical determination of MZ twinning has been reported in the literature. Our objectives were to empirically determine the frequency of MZ twinning in lactating Holstein cows and to compare this result with published estimates predicted using Bonnier's equation. Ear biopsies were collected from 107 sets of Holstein twins from six Wisconsin dairies resulting in 40 opposite-sex twins, 29 same-sex male twins, and 38 same-sex female twins. To empirically determine the frequency of MZ twinning, DNA extracted from ear biopsies collected from the 67 same-sex twins was PCR amplified using primers for a minimum of 5 polymorphic microsatellite DNA markers. Opposite-sex twins were classified as dizygotic (DZ) as well as same-sex twins differing in at least one microsatellite DNA marker. Same-sex twins were classified as MZ when all genotypes for a minimum of five markers were identical. Of the 67 same-sex twins, 62 were classified as DZ and 5 MZ resulting in a MZ twinning frequency of 7.5% of same-sex twins and 4.7% of all twins. The estimated frequency of MZ twinning in this population of twin calves using Bonnier's equation was 39.5% of same-sex twins and 24.7% of all twins. We concluded that MZ twinning occurred infrequently in Holstein cattle and perhaps less frequently than that reported in studies using Bonnier's equation to estimate MZ twinning.

Animals↗

[Treatment of chronic anal fissure].

Chronic anal fissure is a common benign anorectal problem in Western countries that substantially impairs the patient's life. Consequently, a rapid and effective solution is required. We reviewed the various treatments for chronic anal fissure described in the literature, with the aim of establishing a therapeutic protocol. We recommend surgical sphincterotomy (preferably open or closed lateral sphincterotomy) as the first therapeutic approach in patients with chronic anal fissure. However, we prefer the use of chemical sphincterotomy (preferably botulinum toxin) in patients aged more than 50 years old and in those with previous incontinence, risk factors for incontinence (previous anal surgery, multiple vaginal births, diabetes, inflammatory bowel disease, etc.), or without anal hypertonia, despite the higher recurrence rate with medical treatments, since this procedure avoids the greater risk of residual incontinence described in the literature with surgical sphincterotomy in this group of patients.

Botulinum Toxins↗

Relationship of follicle numbers and estradiol levels to multiple implantation in 3,608 intrauterine insemination cycles.

OBJECTIVE: To determine the relationship of follicle numbers and estradiol (E(2)) levels to multiple implantations in human menopausal gonadotropin (hMG) and clomiphene citrate (CC) cycles. DESIGN: Fifteen-year prospective study. SETTING: Private infertility clinic. PATIENT(S): Women who underwent 3608 cycles of husband or donor intrauterine insemination (IUI). INTERVENTION(S): Ovulation induction (OI) with CC, hMG, or CC+hMG. MAIN OUTCOME MEASURE(S): Pregnancy and multiple implantations. RESULT(S): Triplet and higher-order implantations-but not twin implantations-were related to age, E(2) levels, and number of follicles > or = 12 mm and > or = 15 mm, but not number of follicles > or = 18 mm, in hMG and CC+hMG cycles. For patients less than 35 years old, three or more implantations tripled when six or more follicles were > or = 12 mm, in CC, hMG, and CC+hMG cycles, and when E(2) was > or = 1000 pg mL in hMG and CC+hMG cycles. For patients 35 or older, pregnancy rates in hMG and CC+hMG cycles doubled when six or more follicles were > or = 12 mm, or E(2) levels were >1000 pg mL, whereas 3 or more implantations were not significantly increased. CONCLUSIONS: Withholding hCG or IUI in CC, hMG, and CC+hMG cycles when six or more follicles are > or = 12 mm may reduce triplet and higher-order implantations by 67% without significantly reducing pregnancy rates for patients under 35 years of age.

Adult↗

A comparative prospective study of conventional regimen with chronic low-dose administration of follicle-stimulating hormone for anovulation associated with polycystic ovary syndrome.

OBJECTIVE: To compare efficiency of conventional and chronic low-dose regimens for treatment of anovulation associated with polycystic ovary syndrome (PCOS). DESIGN: Fifty participants divided into two equal groups. The first group was treated with urinary human FSH using a conventional stepwise protocol and the second group was treated with a regimen of chronic low-dose and small incremental rises with urinary human FSH or with recombinant human FSH for a maximum of three cycles. SETTING: Tertiary referral university hospital fertility unit. PATIENTS: Fifty infertile women with clomiphene citrate-resistant anovulation associated with PCOS. MAIN OUTCOME MEASURES: Pattern of follicular development, amount of FSH required, serum E2 concentrations, cycle fecundity, cumulative conception, and live birth rates. Multiple pregnancy and ovarian hyperstimulation syndrome (OHSS) rates. RESULTS: Compared with the conventional dose protocol, the chronic low-dose regimen yielded slightly improved pregnancy rates (40% versus 24%) while completely avoiding OHSS and multiple pregnancies, which were prevalent (11% and 33%, respectively) with conventional therapy. Monofollicular development was induced in 74% versus 27% of cycles, and the total number of follicles > 16 mm and E2 concentrations were half those observed on conventional therapy. CONCLUSIONS: For women with PCOS, a chronic low-dose regimen of FSH eliminated complications of OHSS and multiple pregnancies while maintaining a satisfactory pregnancy rate. This modality, thus, has distinct advantages and could well replace conventional gonadotropin therapy for these patients.

Adult↗

Complete and incomplete forms of Beckwith-Wiedemann syndrome: their oncogenic potential.

The diagnosis of Beckwith-Wiedemann syndrome may be missed because of variable or incomplete clinical expression. Recognition of such patients is important, however, because they have the potential for development of neoplasia. It seems likely that BWS and the congenital asymmetry-abdominal malignancy syndrome are at either end of the same spectrum, and that intermediate forms are the connecting links.

Abnormalities, Multiple↗

Impact of environmental sanitation and crowding on infant mortality in rural Bangladesh.

In two villages of Bangladesh, 2471 infants born in 1976 and 1977 were followed up for a year to study the impact of environmental sanitation and crowding on their mortality. Neonatal and postneonatal mortality rates in the study cohort were 100 and 75 per 1000 live-births, respectively. Multiple logistic regression analysis was done to estimate the effects of the selected risk factors while controlling for some socioeconomic, demographic, and biological characteristics. Risk of postneonatal mortality (PNNM) in the households which did not use latrines was 3 X 12 times (p less than 0.01) higher than in those which did and 1.5 times (p less than 0.05) higher in the households with 10 or more persons than in smaller households. PNNM in the households which did not use tube-wells (hand-pump) water was higher, but not significantly so, than in those which used tube-well water for all purposes. Neonatal mortality was completely unrelated to the environmental factors investigated.

Bangladesh↗

[Mid and long-term neurological prognosis of preterm infants less than 28 weeks gestational age].

The study of the long-term outcome of extremely premature babies is specially difficult because data in the literature is very heterogeneous. Recruitment (inborn, outborn), type of obstetrical management, and criteria and means used for interrupting curative treatment have varied greatly. We present the outcome of 204 infants born before 28 weeks of gestation between 1992 and 1997. The minimal follow up is 6 years. 82 infants (40.2%) died during the neonatal period. Significantly associated with neonatal death were absence of prenatal steroid course, male gender, elevated lactic acid at birth, and occurrence of pulmonary complications. When major neurological lesions (ventricular hemorrage stage III or IV and kryptic leucomalacia) developed, most infants died following a decision to stop active treatment. Out of the 114 survivors, 17 (14.9%) developed cerebral palsy (CP) or a low IQ. 31 (27.2%) had minor disorders, 66 (57.9%) were completely normal. The predictive factors of CP were major brain lesions, elevated lactic acid at the time of birth and multiple pregnancy. We also detail the minor neurological sequelae, cognitive behavioral, and psychological disorders observed in this population of extremely premature children and discuss the need for early and continuous care for these high risk babies.

Cerebral Palsy↗

Delayed interval delivery of two remaining fetuses in quintuplet pregnancy after embryo reduction: report and review of the literature.

A case report is presented with a prolonged interval between delivery of 25 days. A quintuplet pregnancy resulted from hormonal stimulation of ovulation. Two fetuses remained after an embryo reduction was performed at 11 weeks gestation. At 22.5 weeks gestation the first twin (310 g) was delivered after spontaneous rupture of membranes. Using tocolytic agents, the second twin (710 g) was born at 26 weeks of gestation. This case is discussed and a review of the literature is given.

Abortion, Induced↗