Thyroid Status in Children with Transfusion Dependent Thalassemia in a Tertiary Level Hospital.

Most of the thalassemic children of Bangladesh are receiving repeated blood transfusion. But they do not receive chelation therapy due to financial constraints. As a result, iron overload occurs in various organs of these children. Extra iron that is loaded in thyroid gland causes thyroid dysfunction. This study was undertaken to evaluate thyroid status in children with transfusion dependent Thalassemia patient. This cross-sectional analytical study was conducted in the Department of Pediatrics, Mymensingh Medical College Hospital, Bangladesh from September 2016 to April 2018. Children having thalassemia diagnosed by Hb electrophoresis, aged 3-12 years of both sexes were included as study group. Children of same age and sex admitted in indoor of Mymensingh Medical College Hospital with minor illness and without thalassemia were taken as comparison group. Purposive Sampling technique was applied. Serum FT4, TSH and ferritin level were estimated in all children. Data analysis was done with Statistical Package for Social Science (SPSS) version 21.0. A total of 60 patients were enrolled as study group and another 60 patients were compared as comparison group. Mean ages of study group was 7.88±2.55 years and comparison group were 7.22±2.48 years. The mean pre-transfusion hemoglobin, serum ferritin, serum FT4 and serum TSH level were found 6.23±0.60 gm/dl, 2658.33±879.39 ng/ml, 15.14±4.40 fmol/mL, 4.29±4.60 µIU/mL respectively in study group. The mean serum FT4 was found significantly lower and mean serum TSH was significantly higher in thalassemic children in comparison to non-thalassemic children (p= <0.05). Frequency of subclinical hypothyroidism was found significantly higher in study group (25.0%) compared to comparison group (3.3%) (p=0.001). Mean serum ferritin level was found significantly higher in hypothyroid cases. Mean FT4 level was significantly lower and mean TSH level was significantly higher in hypothyroid thalassemic patients (p= <0.001). Significant positive correlation between serum ferritin level and serum TSH level was found. Higher serum ferritin level was found significantly associated with the development of hypothyroidism in thalassemic patients.

Risk Factors and Immediate Neonatal Outcome of Multiple Pregnancies in a SCANU of A Tertiary Care Hospital, Bangladesh.

Multiple pregnancies are a significant risk factor for maternal and perinatal morbidity due to inherent biological risks. The present study was aimed to determine the risk factors and to evaluate the immediate neonatal outcome of multiple pregnancies. This descriptive observational study was conducted in the newly established Special Care Neonatal Unit (SCANU) of Mymensingh Medical College Hospital (MMCH), Bangladesh from January 2015 to March 2015. Total 86 newborns of multiple pregnancies who were admitted during the study period were included in the study. Among them 34 pairs were twin, and 6 sets of triplets were present. Among all infants in the study 79.1% were twin and 20.9% were triplet. According to our observation most of the mother aged between 20-29 years (67.5%) and majorities of them were multi-para (62.5%). Preterm delivery occurred in 70.0% cases. 75% newborns were delivered by caesarian section and remaining by vaginal route. The most common risk factors in present study are use of ovulation induction drugs (52.5%) for sub fertility followed by family history of multiple gestations. In 35.0% cases we did not find any cause. Common morbidities of the infants were preterm low birth weight (95.3%), neonatal jaundice (81.3%), RDS (34.8%), perinatal asphyxia (PNA) (17.4%), neonatal sepsis (25.0%) and congenital anomalies (23.2%). Mortality rate of twin was 30% and for triplets it was 50%. Among total study population mortality rate was 30.2%. Main causes of mortality was preterm low birth weight (76%), RDS (57.6%), septicaemia (26%), PNA (19.0%). Morbidities and mortalities in infants of multiple pregnancies are observed much higher than singleton pregnancy. We hope that in light of our findings regarding risk factors like assisted reproduction and their consequences over mother and newborn, periodic reviews will be done in future to reduce the incidence and the unfavourable outcome of multiple pregnancy.

Klippel Trenaunay Syndrome: A Case Report.

Klippel Trenaunay syndrome refers to a rare congenital anomaly which is characterized by capillary malformation, venous malformation and sometimes lymphatic malformation associated with overgrowth of a limb, with soft tissue hypertrophy and/or bony hypertrophy. The anomaly, if present, is present at birth and usually involves the lower limbs as well as portion of trunk, face, uppper limb or head. Our reporting case is a preterm male neonate having port wine stain, varicose veins and excessive growth of soft tissue of left lower limb clinically consistent with Klippel Trenaunay Syndrome.

Incomplete Pentalogy of Cantrell--A Case Report.

Cantrell's Pentalogy is a rare congenital malformation consists of supraumbilical abdominal wall defect, defect in the lower part of sternum, agenesis of anterior portion of diaphragm, an absence of the diaphragmatic part of the pericardium and intracardiac malformation. This case report presents a female neonate, who was born at 40 weeks of gestation weighing 2400 gm and was admitted 4 hours after delivery with the complaints of something coming out from chest. On physical examination her vital signs were within normal limit, she had a systolic murmur on heart at lower left sternal area and there was a vascular structure present on the upper part of abdomen which was pulsatile and pulsation was synchronized with cardiac pulsation. On investigation chest X-ray lateral view showed absence of lower part of sternum, echocardiography findings were different in different institutes. Echocardiography findings at Mymensingh medical college hospital (MMCH) were large VSD (Ventriculo septal defect), ASD (Atrial septal defect) and rotated heart. On colour Doppler ultrasonogram at MMCH showed there were VSD, ASD, and a small epigastric swelling which was vascular and appears to be attached to the apex of the heart. On the other hand echocardiography findings of LAB AID hospital in Dhaka were Dextrocardia, complete AV (atrio ventricular) canal defect, almost common atrium, almost single ventricle, common AV valve, double outlet right ventricle (DORV), Cortriatriatum, mild A-V valve regurgitation and severe pulmonary hypertension (PAH). Echocardiography was also done at national heart foundation hospital in Dhaka. Findings were situs solitus, mesocardia to dextrocardia, DORV, large VSD, mild mitral inflow, mild TR (triuspid regurgitation), good LV (left ventricle) and RV (right ventricle) systolic function. Cardiologists at Dhaka in Bangladesh were suggested for surgery. Then the patient was consulted at Naryan Institute of Cardiac Science, Chennai in India. Here echocardiography findings were dextroversion/dextrocardia, DILV (Double inlet left ventricle), large inlet VSD with bidirectional shunt, mild TR, severe PAH with good ventricular function. Cardiologists in India were given comment about this patient. This patient was highly risky for surgery. They advised medical treatment and requested to review after one year. By taking medical treatment patient condition is well except failure to thrive and cyanosis develops during feeding and crying according to the statement of guardian of the patient. This case has 3 criterias among the five criteria of Cantrell's Pentalogy. So, it is incomplete Pentalogy of Cantrell.