A rare case of sirenomelia

Sirenomelia also called mermaid syndrome is a congenital, rare, lethal, multisystemic human malformation of unknown etiology. The characteristic feature of sirenomelia is the fusion of the lower limbs, resulting in the appearance of a mermaid’s tail, and thus the name “mermaid syndrome.” This condition is also characterized by various severe urogenital abnormalities and the presence of a singular umbilical cord blood vessel, and it is more common in infants of diabetic mothers and in monozygotic twins. The incidence is around 1 in 60,000–70,000 pregnancies. The majority of affected fetuses are stillborn, whereas the rest of the live-born die in the early neonatal period due to complications of the gastrointestinal and urogenital systems. We are reporting a case of sirenomelia in a neonate born to an unregistered multipara mother. The baby had perinatal asphyxia, sirenomelia, dextrocardia, low set ears, lymphatic malformation, bilateral renal agenesis, absent external genitalia and anus, single umbilical artery, and congenital corneal clouding. There was no antenatal ultrasonography done and the baby died at 6 h of life.

Early predictors of early onset neonatal hypocalcaemia in infants of diabetic mother

Background: The objective of this study was the magnitude of hypocalcemia and to assess the predictors for early onset neonatal hypocalcemia in infants of diabetic mother.Methods: Total 100 infants of diabetic mother were followed for development of hypocalcaemia. weight length, gestational age, Apgar score, cord calcium was correlated for serum calcium at 48 hours of life.Results: In present study 91% babies were term and 9% babies were preterm, the incidence of hypocalcemia was high in preterm babies i.e.22% when compared to term babies i.e. 4.3% was not significant and incidence of hypocalcemia was high in babies with Apgar <7 at 1 min (27.27%) was significant statistically. The association of hypocalcemia with cord calcium is not significant statistically.Conclusions: The incidence of hypocalcaemia is more among preterm babies and babies with risk factors, so these babies need close monitoring for hypocalcaemia. No need of regular monitoring of calcium for healthy term babies unless they are symptomatic.

Risk factors for cardiac complications in infants of diabetic mothers / 中华实用儿科临床杂志

Objective To discuss the risk factors for cardiac complications in infants of diabetic mothers (IDMs).Methods One hundred and seventy-one out of 228 IDMs hospitalized in Department of Pediatrics,Shenzhen Hospital of Peking University from Jan.2011 to Apr.2014,were involved,among them,there were 171 cases who had the complete mother's data during pregnancy,and they were received B ultrasonography whin 1 week after birth.All the infants were divided into 2 groups according to echocardiography within 1 week after birth:the observation group (the patients had cardiac complications) and the control group (the patients had normal heart).Conditions of IDMs and their mothers between the 2 groups were recorded and compared.Results In the 171 IDMs,there were 69 cases(38male and 31 female) in the observation group and 102 cases(59 males and 43 females) in the control group.There were 24 preterm infants,16 large for gestational age(LGA) infants in the observation group,while 47 preterm infants and 15 LGA in the control group,and which had no statistical difference between the both groups(x2 =0.129,2.163,1.995 ; all P ＞ 0.05).In the observation group,there were 7 (4.1％) newborns with hypoglycemia,24 (14.0％) women with advanced maternal age,24(14.0％) women with other gestational complications,19(11.1％) women having good control of their glucose serum levels,and 9(5.3％) women with impaired glucose tolerance.All the mentioned factors had statistical differences when compared with the control group[1 (0.6％) case,19 (11.1％) cases,15 (8.8％) cases,80(66.8％) cases,26(15.2％) cases] (x2 =7.752,5.707,9.423,43.735,3.917 ;all P ＜ 0.05,0.01).Logistic regression showed that women with other gestational complications as risk factors(OR =3.29,95％ CI 1.248-8.671),while having a good control of the glucose serum levels acted as a protectable factor(OR =0.098,95％ CI 0.045-0.217).Conclusions IDMs are prone to have cardiac complications if their mothers couldn't control the glucose serum levels during pregnancy,with other gestational complication and advanced maternal age and with more serious diabetes.

Follow-up of infants of diabetic mother combined with heart disease / 中华实用儿科临床杂志

Objective To investigate the cardiac structure and hypertrophic cardiomyopathy of infant of diabetic mother(IDM),to analyze of the outcome of hypertrophic cardiomyopathy in IDM.Methods Totally 23 cases of IDM admitted in NICU from Feb.2012 to Jan.2013 were selected as observation group,randomly selected from the same period with gestational age of diabetic mother baby 23 cases as control group.The interventricular septum thickness,left ventricular posterior wall thickness,aortic valve internal diameter,right ventricular diameter,ejection fraction were detected and compared between the 2 groups.And echocardiography was followed up in 3 months and 6 months old.Results In IDM group,interventricular septum thickness [(3.20 ± 0.28) mm],left ventricular posterior wall thickness [(3.40 ± 0.31) mm] were significantly higher than those in control group (all P ＜ 0.05).Ejection fraction [(54.00 ± 3.76) × 10-2] was significantly lower than that in control group(P ＜ 0.05).Follow-up after 3 months,6 months old,interventricular septum thickness,left ventricular posterior wall thickness and ejection fraction returned to normal.Four of the 23 cases(17.4％) had cardiac malformations.Conclusions IDM has high morbidity of congenital heart disease,echocardiography should be done to identify hypertrophic cardiomyopathy and congenital heart disease after birth.IDM combined with hypertrophic cardiomyopathy is a transient,benign process,prognosis is good.

Two Cases of Hypertrophic Cardiomyopathy in the Infants of Diabetic Mother

Hypertrophic cardiomyopathy(HCM) in the infant of diabetic mother is transient, nonfamilial condition with morphologic and functional abnormalities that resemble familial HCM. However, unlike ""true"" HCM, the cardiomyopathy of infants of diabetic mothers is usually innocent and transient, resolving during the first 6 months of life. Cardiorespiratory distress may improve within 2 to 4 weeks. Reported are two cases of infants with hypertrophic cardiomyopathy, attributed to maternal diabetes. These infants developed respiratory distress within a few hours after birth, but needed only supportive care. Echocardiogram showed myocardial hypertrophy regressing over two to four months.

The Perinatal Complications in Infants of Mothers with Gestational Diabetes Mellitus

PURPOSE: This study was performed to examine the impact of gestational diabetes mellitus (GDM) on perinatal outcomes, especially macrosomia and metabolic complications in Korean women. Subjects and METHODS: We studied perinatal outcomes of 65 women with GDM who delivered a singleton infant and were not complicated by medical disease that might effect the fetal growth, 65 women with normal glucose tolerance were selected to match subject with GDM matched to age, height, and prepregnancy weight. RESULTS: 1) Mean birth weight in infants of mothers with GDM was significantly heavier than that in infants of control mothers, even though the delivery was almost one week earlier (3524 vs. 3364gm). 2) Although length and head circumference of infants were not different between 2 groups, chest circumference and Ponderal index were significantly higher in infants of mothers with GDM. 3) The frequencies of LGA infants and macrosomia were higher in GDM group, but frequency of prematurity was not different between 2 groups. 4) The proportion of disproportionate growth among macrosomic infants was significantly higher in GDM group. 5) In infants of mothers with GDM, the frequencies of LGA infants were not significantly different between 2 groups which were separated according to the fasting glucose level measured at the time of diagnosis. 6) In infants of mothers with GDM, the frequencies of hypoglycemia, polycythemia, hypocalcemia and hyperbilirubinemia were 21.5%, 18.5%, 3.1% and 33.8% respectively. When infants of mothers with GDM were divided to 2 groups (LGA, AGA), the frequencies of polycythemia and hyperbilirubinemia in LGA infants were higher than those in AGA infants (30.8 vs. 10.3% and 50.5 vs. 23.1%, respectively). 6) There was a significant positive correlation between Ponderal index and chest circumference/head circumference ratio (r=0.37, p<0.01). CONCLUSIONS: Inspite of tight metabolic control of mothers with GDM, perinatal complications, such as macrosomia, hypoglycemia, hyperbilirubinemia were frequent in infants of mothers with GDM. In case of infants of mothers with GDM, perinatal complication should be treated earlier or prevented by careful physical examination and intensive management.

A clinical study on infants of diabetic mothers

Thirty one infants of diabetic mothers(IDM) who had been admitted in Neonatal Intensive Care Unit at Chonnam University Hospital from January 1987 to July 1991 were studied for evaluation of their perinatal outcome and prognosis. The results were as follows; 1) The distribution of diabetic mothers according to modified White's classification was GD & class A in 12 cases(38.7%), B in 16 cases(51.6%), C in one case, D4 in one case, and R in one case. 2) Of the 31 diabetic mothers, 8(25.8%) had a history of infertility or stillbirth, 20(64.5%) had prognostically bad signs of pregnancy (PBSP), and 12(38.7%) were treated with insulin and/or oral hypoglycemic agent during pregnancy. 3) C-section rate was 32.2%, and the rates of shoulder dystocia and birth asphyxia were 35.5% and 29.0%, respectively. 4) The rates of large for gestational age were 75.0% in class GD & A, 68.8% in class B, and the rates of small for gestational age were 8.3% and, 6.3% respectively, Characteristic face with body composition was noted in all babies, hepatosplenomegaly in 90.3%, heart murmur in 41.9%, respiratory distress syndrome in 12.9%, transient tachypnea of mewborn in 19.4%, hypertrophic cardiomyopathy in 40.0%, and seizure in 6.5%. 5) Laboratory abnormalities were hypoglycemia in 77.4%, hyperbilirubinemia in 83.9%, polycythemia in 16.1%, hyperinsulinemia in 45.5%, hypercalcemia in 22.6%, and hypomagnesemia in 23.1%. 6) Birth injuries were noted in 61.3%, The most common were caput succedaneum and/or cephal-hematoma, brachial palsy, and fracture. Congenital anomalies were noted in 16.1%. The most common were musculoskeletal, cardiovascular, and gastrointestinal anomalies. 7) In the short term follow up of 19 patients, there were mild developmental delay in 2 cases, cerebral palsy in 2 cases, and epilepsy in 1 case. These results suggested that infants of diabetic mothers had greater morbidity than infants of nondiabetic mothers. Therefore, long term follow up is recommended to achieve their normal growth and development.

An autopssy case of infant of diabetic mother with d-transposition of great arteries and hypertrophic cardiomyopathy

No abstract available.