Case Report of Tetralogy of Fallot Associated with Tight Lesion of the Left Anterior Descending Coronary Artery.

A small subset of patients with TOF present later in life with unsuspected or untreated disease. Tetralogy of Fallot in adults represents a special group with peculiar problems related to the effects of prolonged cyanosis over the heart and other organs .Older age has been considered as incremented risk factor for surgical mortality and long-term survival in patients with TOF. Based on this assumption, indication for surgery after long-standing cyanosis has caused controversy. There are few reports that studied this issue have proved some benefit in repairing adults with TOF .The greatest benefit of complete repair at this age is the functional improvement. We report a male patient with TOF with tight lesion of the left anterior descending coronary artery diagnosed at age 40 years and successfully underwent total correction along with CABG. This patient was the oldest documented in Bangladesh undergoing successful corrective surgery for TOF with CABG.

Hepatitis B viral infection with nephrotic syndrome treated with lamivudine.

A 04 years old boy with 02 months history of generalized oedema and scanty micturition was diagnosed as nephrotic syndrome with hepatitis B viral infection. He had evidence of active viral replication. After 01 month treatment with oral lamivudine, his urine became protein free and after 04 months, he had seroconversion from HBeAg+ve to HBeAg-ve. Lamivudine was continued for 01 year. He had no relapse after discontinuation of therapy and remained well after 36 months of completion of therapy. He had no evidence of active viral replication during this period, however HBsAg remained positive indication carrier state. As most children with HBV associated nephropathy have no evidence of chronic hepatitis, all such children must undergo HBV screening and for chronic liver disease if HBV screening is positive. As such children do not respond to prednisolone or other immunosuppresive therapy which might harm them, antiviral therapy should be considered. Lamivudine is a suitable alternative to IFN alpha owing to its low cost, ease of administration and fewer side effects.

Camptomelic dysplasia with sex reversal.

Camptomelic dysplasia is a disorder of the newborn characterized by congenital bowing and angulation of long bones together with other skeletal and extraskeletal defects. The affected newborn had dysmorphic features with bowing of the legs and bilateral talipes equinovarus. Radiology showed marked anterior bowing of both tibia with disproportionately short fibula, anterolateral bowing of the femurs and wide pelvic outlet with small iliac wings. She had sex reversal with normal female genitalia and 46, XY karyotype. Camptomelic dysplasia is generally considered to be a lethal skeletal dysplasia and most patients die in the neonatal period due to severe respiratory distress. Survivors may have learning difficulties, developmental delay, conductive hearing loss, myopia and recurrent chest infections. Because of its high associated mortality, prenatal diagnosis of camptomelic dysplasia is mandatory. The birth of a child with skeletal dysplasia is an emotionally difficult experience for parents.

Evaluation of hospitalized infants and young children with bronchiolitis - a multi centre study.

Four hundred and twenty nine young children with bronchiolitis admitted consecutively in different hospitals of Bangladesh were evaluated. Three hundred and forty eight children studied for their putative risk factors, clinical profile, management and the outcome. Both cases and controls were examined for respiratory syncytial virus (RSV) antibody status. The diagnosis of bronchiolitis was made on the basis of first attack of wheeze in previously healthy children below two years of age. Detailed history including the possible risk factors, the management and daily follow-up on the ward and the outcome at discharge were documented through a structured questionnaire. Chest x-ray was done in each case to find out the radiological changes. Blood of 266 patients and 30 controls were studied for RSV IgM and IgG antibody by ELISA. There were 66% male and 34% female children. The median age of the children was 3.0 months and 82.7% were below 6 months of age. Most of the babies were born term (88%), with ABW (73%), by normal vaginal delivery (88%). Exclusive or predominant breast-feeding were given in 72% cases. The location of the patient was rural in 55% cases. Around half of the parents were illiterate or slightly educated (up to 5 years schooling) fathers 46.5% and mothers 56% and majority of the parents were poor (74%). In 52% cases the number of family members in one room were four or more. Half of the parents (52%) were smokes and there was atopy in 26.5% families. The clinical features of bronchiolitis were mostly cough (99%), respiratory distress (97%), feeding difficulty (93%) and fast breathing (96%) (median RR 68/min). Fever (1000F or more) was in only 33% cases, though parents complained in 90% cases. All children (100%) had wheeze and crackles in lungs in 96% cases. Liver could be palpable in 83% and spleen in 42% cases. Important radiological features were increased translucency (96%), increased interstitial markings (87%), hyperinflation (75%) and streaky densities (61%). In 69.6% cases TLC was 12,000 or less and only 15% with a neutrophil fraction greater than 60%. Children were positive for IgM antibody in 43.6% cases and both IgM and IgG in 5.3% cases. The main modalities of treatment were antibiotics (99%) (Ampicillin, 76%), oxygen therapy (83%), nebulised salbutamol (76%) and intravenous fluid (51%). The median duration of hospital stay was 4 days. Most of the children were discharged with improvement (96%) with 2% mortality. Not a single case was diagnosed as bronchiolitis in hospitals outside Dhaka. Cefrtiaxone (72.5%) and parenteral steroids (70.5%) were the mainstay of therapy there.

Perinatal outcome in relation to mode of delivery in meconium stained amniotic fluid.

Meconium staining of the amniotic fluid is a common complication during labour. When facilities like electronic monitoring, foetal blood sampling are not available, it is difficult to decide whether labour should be allowed to continue or caesarean section should be done. Even when caesarean section is done, meconium aspiration syndrome (MAS) can still occur and considerable morbidity and mortality may result in the newborn. Amino infusion is being considered as useful in decreasing MAS and its sequelae. Before resorting to amino infusion, we decided to analyse the perinatal outcome in meconium stained liquor to compare whether early caesarean section offered any advantage. This is a retrospective study of 150 labours complicated by thick meconium stained liquor, during a 12 month period (1992-93).

Serum immunoglobulin profiles of septicemic versus healthy neonates.

We studied 125 clinically suspected septicemic neonates (Patient) aged from 1 to 28 days and 25 healthy neonates (control) of comparable age and sexes. Cultures of blood were done and serum immunoglobulins (IgG, IgA) were estimated in all the subjects. Blood cultures were found positive in 45 (36%) patients. Preterm patients showed significantly higher number of positive blood cultures as compared to term patients. The mean serum IgG level in patients was found significantly lower than that of the controls. The serum IgG levels were also found significantly lower in 75 preterm as compared to 50 term, and in 45 blood culture positive patients as compared to 80 blood culture negative patients. On the other hand, the mean serum IgM level in patients was found significantly higher as compared to controls. Similarly, serum IgM levels were found higher in preterm patients as compared to term patients and in blood culture positive patients as compared to blood culture negative patients. No significant difference of mean serum IgA level was found among the subjects. It is evident from our study, that blood culture positive patients were mostly preterm, in whom transplacental passage of IgG is insufficient and due to low IgG level, preterm baby cannot counteract bacterial invasion and as such, suffer from septicemia more frequently. Septicemic neonates as a rule showed higher level of serum IgM due to synthesis by themselves in primary response to infection.

Male pseudohermaphroditism due to early foetal testicular dysgenesis--a case report.

An 8.5 years old girl presented with enlargement of clitoris. She was chromatin-negative with 46XY karyotype. Gonadotropin value was high with slightly raised testosterone level for female. Laparotomy showed only streakes of testicular tissue and no Uterus or Fallopian tubes were found. She was short and had malrotated kidney.