Bone density, fractures and the associated factors in iranian children and adolescent with Osteogenesis Imperfecta.

BACKROUND: Osteogenesis imperfecta(OI) is a frequent bone fragility disorder in children. The purpose of this study was to assess the BMD and Vitamin D level in children with OI in southern Iran. METHOD: This case-control study was conducted on 23 children, clinically diagnosed as osteogenesis imperfecta and 23 age- and gender-matched healthy controls. Demographic and anthropometric data, biochemical parameters, puberty, sun exposure and physical activity were assessed. Bone mineral density (BMD) was measured by Dual-energy X-ray absorptiometry (DXA). Data analysis was done by SPSS22. RESULTS: Forty-three point four percent of OI patients and fifty-six point five percent of control group had vitamin D deficiency (P = 0.376). Thirteen OI patients (56%) had low bone mass for chronological age in lumbar area (P < 0.001). Fracture episodes during treatment was significantly influenced by time of Pamidronate start, courses of Pamidronate injection, puberty and sun exposure (P values = 0.015, 0.030, 0.044 and 0.032, respectively). Fracture episodes during treatment had significantly increased in patients who had received Pamidronate more than 3 years compared with those received less than 3 years(P values = 0.047). CONCLUSIONS: This study showed that vitamin D deficiency is prevalent amongst OI children in southern Iran. More than half of the OI children had low bone mass for chronological age in lumbar area, despite receiving bisphosphonate therapy. The present results revealed that early initiation of Pamidronate and number of Pamidronate courses are associated with lower fracture rate. However, treatment period more than 3 years can have adverse effect on fracture rates.

Interaction between serum FGF-23 and PTH in renal phosphate excretion, a case-control study in hypoparathyroid patients.

BACKGROUND: phosphate homeostasis is mediated through complex counter regulatory feed-back balance between parathyroid hormone, FGF-23 and 1,25(OH)2D. Both parathyroid hormone and FGF-23 regulate proximal tubular phosphate excretion through signaling on sodium- phosphate cotransporters IIa and IIc. However, the interaction between these hormones on phosphate excretion is not clearly understood. We performed the present study to evaluate whether the existence of sufficient parathyroid hormone is necessary for full phosphaturic function of FGF-23 or not. METHODS: In this case-control study, 19 patients with hypoparathyroidism and their age- and gender-matched normal population were enrolled. Serum calcium, phosphate, alkaline phosphatase,parathyroid hormone, FGF-23, 25(OH)D, 1,25(OH)2D and Fractional excretion of phosphorous were assessed and compared between the two groups, using SPSS software. RESULTS: The mean serum calcium and parathyroid hormone level was significantly lower in hypoparathyroid patients in comparison with the control group (P < 0.001 and P < 0.001, respectively). We found high serum level of phosphate and FGF-23 in hypoparathyroid patients compared to the control group (P < 0.001 and P < 0.001, respectively). However, there was no significant difference in Fractional excretion of phosphorous or 1,25OH2D level between the two groups. There was a positive correlation between serum FGF-23 and Fractional excretion of phosphorous just in the normal individuals (P < 0.001, r = 0.79). CONCLUSIONS: Although the FGF-23 is a main regulator of urinary phosphate excretion but the existence of sufficient parathyroid hormone is necessary for the full phosphaturic effect of FGF-23.

Bone mineral density loss in ambulatory children with epilepsy in spite of using supplemental vitamin D in Southern Iran: a case-control study.

Epilepsy might have adverse effect on bone density due to underlying disease, drugs, vitamin D deficiency, immobilization and malnutrition. We investigated the bone mineral density in ambulatory vitamin-D supplemented children with epilepsy. This case-control study was conducted on 90 epileptic children aged 11.4 ± 3.3 years, and age and gender matched controls in pediatric neurology clinics of Shiraz, in Southern Iran, 2016. Anthropometric measurements, puberty, sun exposure, physical activity and biochemical variables were assessed. Bone mineral density was evaluated by dual-energy X-ray absorptiometry method. Data were analyzed by SPSS.v21. Prevalence of low bone mass in femur was more in patients (27%) than the controls (9%) (P value = 0.002). Age, weight Z score and height Z score were the most significant associated factors on lumbar BMD, BMAD, and femur BMD. Seizure duration and how it responded to anticonvulsants were the most associated factors with both lumbar and femur bone density. Sodium valproate and carbomazepin usage had negative association with lumbar Z score (beta = - 0.216, P = 0.017 and beta = - 0.336, P = 0.027, respectively). We hypothesized that epilepsy per se could affect bone density by an unknown pathophysiology, which was independent from vitamin D deficiency, effects of anticonvulsant and physical activity.

Short-course treatment in neurobrucellosis: a study in Iran.

Neurobrucellosis is a rare neurological complication of brucellosis. This report describes 19 patients of neurobrucellosis and they accounted for 8% of all cases of brucellosis admitted to Shiraz University Hospitals over a period of eight years. Headache, fever, fatigue, drowsiness and neck stiffness were the common clinical features. Cerebrospinal fluid (CSF) showed pleocytosis in 100%, elevated protein levels in 89% and low glucose level in 47% of the patients. All the patients improved with specific antibiotic treatment. Of the 19 patients, 10 (52.5%) patients received treatment for 8 to 28 weeks. Duration of antibiotic treatment was: 8-14 weeks in 8 (42%) patients; 24-28 weeks in 2 (10.5%) patients; 6 months in 7 (37%) patients; 12 months in 1 (5.3%) patient; and 18 months in 1 (5.3%) patient. Clinicians in endemic areas should consider the likelihood of neurobrucellosis in patients with unexplained neurological and psychiatric symptoms.