Parathyroid hormone in pediatric patients with b-thalassemia major and its relation to bone mineral density; a case control study

Background: Thalassemia syndromes are heterogeneous groups of inherited anemias. Its treatment depends on recurrent blood transfusion with a problem of iron overload, which leads to multiple endocrinopathies including hypoparathyroid. The aim of the study is to estimate the level of serum parathyroid hormone and its relation to bone mineral density in transfusion dependent beta-thalassemia major children

Subjects and methods: We measured serum calcium, phosphorus and parathyroid hormone in a sample of pediatric patients with thalassemia, compared them with age and sex matched healthy control. Measurement of bone mineral density by dual-energy X-ray absorptiometry was done in 2 sites: lumbar spine [L2-L4] in the anteroposterior position and left femur neck using Lunar Densitometry in osteoporosis Unit Ain Shams University Hospital for thalassemia patients

Results: Thalassemic patients had significantly higher alkaline phosphatase and lower bone mineral density

Conclusion: Osteopenia in b-thalassemia major patients is multifactorial and is mainly predisposed by defective function of the parathyroid gland and excessive iron deposition

Study of auditory brain stem latencies and peripheral neuroconductivity in childhood diabetes

This study included 32 diabetic children, in addition to 20 healthy children served as a control group. The aim of this study was to evaluate the central and the peripheral neuroconductive affection in children with insulin dependent diabetes mellitus [IDDM] and to assess their importance as predictors of early neuropathy. All children were subjected to thorough clinical examination. They were investigated for blood glucose, serum glycosylated hemoglobin [HbA1c], serum creatinine and blood urea nitrogen [BUN] levels. They were also subjected to estimation of distal latencies of the median and ulnar nerves bilaterally as well as brain stem auditory evoked potentials [BAEP] recording. Results showed that type I diabetes was associated with prolonged auditory brainstem latencies [62.5% of patients]. Most of them [85%] had no clinical manifestations indicating subclinical central diabetic neuropathy. Metabolic control age of the patient and duration of the disease had no relationship with prolonged BAEP latencies. There was a significant increase in the distal latencies of both median and ulnar nerves in diabetic children as compared to the controls. At least one electrophysiological abnormality was found in 81% of patients. Asymptomatic peripheral neuropathy was found in 27% of patients. Neuropathic diabetic children had significantly higher values of serum glucose and HbAlc than the non-neuropathic patients. There was also a significant increase in the age and duration of illness in the former group than the latter one

Extended cricopharyngeal myotomy in the tratment of cricophryngeal achalasia

Extended Cricopharyngeal myotomy was performed on 27 patients suffering from cricopharengeal dysphagia. Cineradiography revealed a posterior indentation at the level of cricoid cartilage. The surgical procedure included cutting the muscle fibres of the cricopharyngeus which extended proximally to the hypopharynx and distally to the upper esophagus to a total length of 4 cm, abroad flap of the muscle with a width of 1/2 cm was then excised. Results were satisfactory in 26 patients. Pathological examination of the cricopharyngeal muscle demonstrated no pathological change could be detected. So, extended cricopliaryngeal myotomy can be an effective procedure for the treatment of swallowing disorders due to cricopharyngeal achalasia