Metabolic Bone Disease in Children With Transfusion-Dependent Thalassemia

Objective: This study aimed to detect metabolic bone disease and endocrinopathies in a cohort of patients with transfusion-dependent thalassemia (TDT). Methods: This prospective study was conducted between March 2020 - August 2021. Children with TDT older than 5 years, receiving regular blood transfusion, underwent comprehensive endocrine and metabolic bone disease evaluation, which included screening for short stature, delayed puberty, diabetes mellitus, hypothyroidism, adrenal insufficiency and hypoparathyroidism. Children older than 10 years also underwent. X-ray of thoracolumbar spine, and dual energy X-ray absorptiometry (DXA) scanning. Results: Out of 37 patients (19 males), with mean (SD) age 15 (6) years, hypogonadism was the commonest endocrine deficiency seen in 15 (62%), followed by short stature, abnormal glucose metabolism, subclinical adrenal insufficiency, hypothyroidism, and hypoparathyroidism. Vitamin D insufficiency/deficiency was seen in 12 (60%) and hypocalcemia in 2 patients. Low bone mass was seen in 8, and osteoporosis, as evidenced by vertebral fractures, in 4 patients. Of the four patients with vertebral fracture, three were aged ?18 years, one was symptomatic, two each had grade 1 and grade 2 fractures, one had multiple vertebral fractures, and all four had hypogonadism and multiple endocrine deficiencies. Conclusion: Vertebral fractures occur even in the second decade among patients with TDT, and are often associated with endocrinopathies, most commonly hypogonadism. Early screening and prevention of vertebral fractures is necessary.

Formulation and Evaluation Of Gastroretentive Losartan Floating Tablets.

The objective of this research was to formulate and evaluate hydrodynamically balanced controlled drug delivery system of Losartan. This dosage form is associated with many advantages especially increased bioavailability and reduction in dosing frequency. The formulation was designed adopting optimization technique, which helps in setting up experiments in such a manner that the information is obtained as efficiently and precisely as possible. Initially, considering buoyancy as the main criteria, blank tablets were compressed for different formulae with various polymers like HPMC, MC and EC. The formula selected for design had a combination of Losartan, HPMC, EC and MC. The tablets were prepared by direct compression method and evaluated for Losartan content in vitro release profile and buoyancy. The dissolution study was carried out in simulated gastric fluid using USP dissolution test apparatus employing paddle stirrer. Duration of buoyancy was observed simultaneously when the dissolution has carried out The variation in weight was within the range of ±4% complying with pharmacopoeial specifications (±Z5%). The drug content of Losartan floating tablet 8.455±0.0085 mg in of optimized formulations indicating content uniformity. The buoyancy of the tablets was range 15.345±0.1321 hrs the maximum buoyancy was seen in P6, which has a high level of drug to polymer ratio. The in-vitro release was found to be in the range between the 79.12% to 90.45%.. The formulation P6 has an in vitro release of 79.12% showed the release of the drug in the controlled manner. The optimized formulation P6 exhibited responses that were comparable with that of the predicted values of the design in optimization technique. This indicates the suitability of the technique chosen for the present dosage form.

Opsismodysplasia.

Opsismodysplasia is a rare osteochondrodysplasia with micromelia and platyspondyly. The authors report on a neonate with opsismodysplasia. During the antenatal period, polyhydramnios was noted. This is the first report of opsismodysplasia from India. Significant observation was antenatal polyhydramnios.

Subdural effusion complicating neonatal meningitis: successful treatment with acetazolamide.

Postmeningitis subdural effusion is rare in neonates when compared to infants and children. For treatment, various modalities are described. Serial subdural punctures and surgical drain placement are advised for cases having a mass effect on imaging. We report a neonate with symptomatic postmeningitis subdural effusion, who failed to respond to serial subdural punctures, but subsequently managed successfully with acetazolamide. He had no recurrence further. His development was normal at 18 months of age.

Neonatal thrombocytopenia associated with maternal pregnancy induced hypertension.

OBJECTIVE: To evaluate the prevalence of thrombocytopenia in neonates born to mothers with pregnancy induced hypertension (PIH) and identify the associated material and neonatal characteristics. METHODS: In the current, prospective study, platelet counts were assessed serially. Maternal and neonatal characteristic were recorded in pre-designed proforma. Primary outcome measures were thrombocytopenia defined as platelet count of <150,000/mm(3) and severe thrombocytopenia if counts were <30,000/mm(3) or <50,000/mm(3) with bleeding. RESULTS: Of 97 neonates born to PIH mothers 35 (36.1%) had thrombocytopenia. In 20 (20.6%) thrombocytopenia was severe. Higher percentage of thrombocytopenia was associated with male gender (47.7%), low birth weight (71.4%) and prematurity (67.4%). Severe thrombocytopenia was significantly associated with low birth weight (OR: 4.58; 95% CI: 0.98-21.3; p<0.03) and prematurity (OR: 2.52; 95% CI: 0.87-7.24; p<0.05). Material parity, onset of PIH, and medications did not seem to be associated significantly. CONCLUSION: Premature and low birth weight neonates born to mothers with pregnancy induced hypertension would require scrutiny for thrombocytopenia during early neonatal period.

Transcutaneous bilirubin in predicting hyperbilirubinemia in term neonates.

OBJECTIVE: To assess the utility of 24 and 48 hours transcutaneous bilirubin (TcB) index for predicting subsequent significant hyperbilirubinemia in healthy term neonates. METHODS: TcB indices were obtained for healthy, breastfed, term AGA newborns at 24 +/- 2, 48 +/- 2 and subsequently at intervals of 24 hours. Neonates with illness, on treatment and positive Direct Coomb's test were excluded. Serum bilirubin levels were obtained whenever indicated. Neonates having serum bilirubin > or = 17 mg/dL were considered as significant hyperbilirubinemia. The 24 and 48 hour TcB indices, as risk predictors for such hyperbilirubinemia were determined. RESULTS: Study included 461 healthy term neonates. The mean birth weight was 2949 (+/- 390) gm and mean gestation of 38.6 (+/- 1.1) weeks. Eight one (17.6%) had significant hyperbilirubinemia. Of 461, 135 (29.3%) had TcB index. CONCLUSION: The 24 and 48 hour TcB indices are predictive for subsequent significant hyperbilirubinemia and can guide clinician in early discharge of healthy term newborns.

Renal subcapsular abscess.

An 18-day-old neonate presented with fever and abdominal distension. Renal subcapsular abscess was diagnosed with sonography and CT scan. Percutaneous drainage resulted in aspiration of 20 mL of pus, which grew Staphylococcus aureus.