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Biomedical subjects

Shankar Kanumakala

Publications and source records attributed to Shankar Kanumakala.

6 recordsLinked to original sources

Pamidronate treatment of less severe forms of osteogenesis imperfecta in children.

BACKGROUND: Bisphosphonate therapy improves bone quality in children with severe osteogenesis imperfecta (OI). Children with milder phenotypes also have prepubertal fractures, bone pain and reduced bone mass, predisposing them to adult osteoporosis. OBJECTIVE: To evaluate treatment effects of pamidronate in children with mild phenotypes of OI. METHODS: Open label, 2-year observational study of 18 patients, using pamidronate, with clinical, biochemical and radiological monitoring. RESULTS: Over 2 years, bone pain decreased from 16 to 1 patient and disturbed sleep from 12 children to 0. Independent mobility improved from 10 to 17 children. Fracture incidence decreased from 1.6 to 0.5 fractures/child/year. Surgical interventions decreased from a mean 1.3 procedures/patient to 0 in the second year of treatment. Growth velocity remained stable at a mean 4.8 cm/year. Mean lumbar vertebral bone mineral density improved by 40.8%, from 0.375 to 0.528 g/cm2 (p <0.0001), z-score from -3.77 to -2.44 (p <0.0001). Mean vertebral height improved by 17.3%, from 15.6 to 18.38 mm (p = 0.07); plasma alkaline phosphatase decreased from 222 to 169 U/l (p = 0.0009) and urinary deoxypyridinoline crosslinks decreased from 26.7 to 21.8 nmol/mmol creatinine (p = 0.21). Two children with vitamin D insufficiency were concurrently treated. A significant association (r = -0.6, p = 0.008) was shown between age at start of treatment and percentage change in BMD after 2 years. CONCLUSIONS: Pamidronate treatment improves bone quality in children with mild types of OI. It ameliorates clinical symptoms, improves mobility, reduces fracture frequency and thus improves quality of life and in future is likely to reduce the severity and consequences of adult osteoporosis by improved peak bone mass in these children.

Absorptiometry, Photon↗

Bone mineral density outcomes following long-term treatment with subcutaneous testosterone pellet implants in male hypogonadism.

BACKGROUND: Osteoporosis is a common complication of untreated male hypogonadism. Even mild hypogonadism due to suboptimal testosterone replacement may result in decreased bone mineralization and osteoporosis. OBJECTIVE: To assess bone mineral density in hypogonadal men following long-term long-acting subcutaneous testosterone pellet implants as replacement therapy. PATIENTS: A cross-sectional study of 37 patients with primary or secondary hypogonadism receiving long-term (mean 6.6 years) subcutaneous testosterone pellet implants as replacement therapy. MEASUREMENTS: Bone mineral density was measured in all patients using dual energy X-ray absorptiometry. Serum testosterone 3-4 months after insertion of pellets was measured in all patients to assess adequacy of replacement therapy. RESULTS: Mean areal bone mineral density were 1.02 (SD 0.14) g/cm2 with a mean Z score of -0.64 (SD 1.3) and 0.87 (SD 0.13) g/cm2 with a mean Z score of -0.72 (SD 1.2) at lumbar spine and neck of femur, respectively. Mean serum testosterone 3-4 months after pellets insertion was 15.45 nmol/l (SD 4.2 nmol/l). There was no significant correlation between bone mineral density and patient's age at start or duration of testosterone therapy. CONCLUSIONS: Bone mineral density in long-term regularly treated hypogonadal men was not different from the age-matched reference range for normal men. Long-acting subcutaneous testosterone pellet implants as replacement therapy in male hypogonadism are safe, acceptable to the patient, result in adequate bone mass accumulation and maintenance of normal bone mineral density. By provision of sustained physiological levels of testosterone they may contribute to increased androgen effect at the receptor level.

Adult↗

Linear growth and height outcomes in children with early onset type 1 diabetes mellitus--a 10-yr longitudinal study.

OBJECTIVE: To assess the linear growth and height outcomes and the influence of metabolic control on the near-final height in children with early onset type 1 diabetes mellitus (DM). STUDY DESIGN AND METHODS: Retrospective longitudinal evaluation of 99 children with prepubertal onset of type 1 DM before 8 yr of age, who were regularly assessed, clinically and metabolically, from 8 yr of age to 17.99 yr of age. RESULTS: The mean prepubertal height Z scores at 8 yr of age were -0.17 (standard deviation, SD = 0.99) for boys and -0.29 (1.19) for girls, respectively. There was normal linear growth in girls with their mean near-final height Z score being -0.13 (1.07). This was not statistically different from the mean at 8 yr (p = 0.13). The mean near-final height Z score in boys was -0.39 (0.98), which was 0.22 SD lower than their mean prepubertal height Z score (p = 0.03). There was no significant correlation between metabolic control and linear growth in either males or females. CONCLUSIONS: Linear growth and near-final height in children with type 1 DM compares favorably with the general population. Although there was some evidence of suboptimal peripubertal growth in boys, the actual extent of this height reduction was minimal and was not correlated with their metabolic control.

Journal Article↗

Molecular endocrinology of sex differentiation.

Differentiation of a testis or an ovary from a bipotential gonad is a complex developmental process involving various genes and hormones. Similarly, internal and external genital organs develop from an indeterminate stage from the complex differentiation of Wolffian and Müllerian ducts. Differentiation of the Wolffian ducts, urogenital sinus, and external genitalia is androgen dependent. Female sex differentiation appears to be a more passive process that is independent of estrogen. This article reviews the process of differentiation of the gonads and the genitalia and the factors affecting it. In addition, a clinical approach to patients with sexual ambiguity or sex reversal is discussed.

Animals↗

Massive ovarian edema causing early puberty.

Massive ovarian edema is a rare tumor-like condition found predominantly in young women. Patients usually present with abdominal pain and/or abdominal mass. Pre-operative diagnosis is often difficult. Awareness of this rare and benign lesion in young women may allow conservative management and prevention of oophorectomy in some patients.

Child↗