Medial malleolar screw hemiepiphysiodesis for ankle valgus in children with spina bifida.

Medial malleolar screw hemiepiphysiodesis has been shown to be an effective procedure for the treatment of ankle valgus in children with spina bifida. The purpose of this study was to highlight the guided growth technique and discuss its efficacy. We retrospectively reviewed 10 spina bifida patients with 18 ankle valgus deformities treated with medial malleolar screw hemiepiphysiodesis. Medical reports were obtained to document age at surgery, gender, concurrent operative procedures and postoperative early and late complications. Anteroposterior and lateral weight bearing radiographs of both ankles were evaluated using the tibiotalar angle, pre and postoperatively. The average age at operation was 10.05 years. Average follow up was 15.33 months (11-21 months). During follow-up, the average preoperative distal tibiotalar angle of 16.27 degrees improved to an average of 2.88 degrees with statistical significance (p < 0.05). No major complications were detected. In conclusion, hemiepiphysiodesis is a safe and sufficient method to correct ankle valgus deformity in children with spina bifida, with a low incidence.

Osteoporosis-pseudoglioma syndrome: three novel mutations in the LRP5 gene and response to bisphosphonate treatment.

BACKGROUND/AIMS: Osteoporosis-pseudoglioma (OPPG) syndrome is a rare disorder characterized by congenital or infancy-onset visual loss and severe juvenile osteoporosis. OPPG is caused by homozygous mutations in the low-density lipoprotein receptor-related protein 5 (LRP5) gene. We present three novel homozygous LRP5 mutations found in 3 unrelated Turkish children with consanguineous parents, along with clinical phenotypes and response to treatment with bisphosphonates (bisP). METHODS/RESULTS: The LRP5 gene was analyzed by direct sequencing after PCR amplification. Mutation screening for LRP5 revealed homozygous nonsense R1002X mutation in the first patient and homozygous missense mutations V336M and G507S in the second and third patient, respectively. The parents were heterozygous for these mutations. The patients' eye symptoms began during the first months of life but the OPPG diagnoses were made based on skeletal deformities and osteopenia after 4 years of age. The patients' bone mineral density Z scores were very low and consistent with osteopenia. All patients were treated with bisP for 3.5-7 years. CONCLUSION: We report three novel LRP5 mutations in 3 Turkish patients with OPPG. We show that the response of bisP therapy has improved the lumbar spinal bone mineral density Z scores and the patients' quality of life as the bone pains decreased.

Species diversity of ixodid ticks feeding on humans in Amasya, Turkey: seasonal abundance and presence of Crimean-Congo hemorrhagic fever virus.

Ticks (Acari:Ixodidae) are important pests transmitting tick-borne diseases such as Crimean-Congo hemorrhagic fever (CCHF) to humans. Between 2002 and 2009, numerous CCHF cases were reported in Turkey, including Amasya province. In the current study, species diversity, seasonal abundance of ticks, and presence of CCHF virus (CCHFV) in ticks infesting humans in several districts of Amasya province were determined. In the survey, a total of 2,528 ixodid ticks were collected from humans with tick bite from April to November 2008 and identified to species. Hyalomma marginatum (18.6%), Rhipicephalus bursa (10.3%), Rhipicephalus sanguineus (5.7%), Rhipicephalus (Boophilus) annulatus (2.2%), Dermacentor marginatus (2.5%), Haemaphysalis parva (3.6%), and Ixodes ricinus (1.6%) were the most prevalent species among 26 ixodid tick species infesting humans in Amasya province. Hyalomma franchinii Tonelli & Rondelli, 1932, was a new record for the tick fauna of Turkey. The most abundant species were the members of Hyalomma and Rhipicephalus through summer and declined in fall, whereas relative abundances of Ixodes and Dermacentor ticks were always low on humans in the province. Of 25 Hyalomma tick pools tested, seven pools were CCHFV positive by reverse transcription-polymerase chain reaction. Results indicated diversity of ixodid tick species infesting humans was very high, abundance of ticks changed by season, and ticks infesting humans had potential for transmitting CCHFV.

Scintigraphic evaluation of the effectiveness of intranasal calcitonin therapy in Sudeck's atrophy.

The aim of this study was to evaluate the efficacy of intranasal salmon calcitonin compared to a nonsteroidal anti-inflammatory drug (NSAID) in patients with Sudeck's atrophy by means of quantitative three-phase bone scanning. Included in the study were 14 patients with Sudeck's atrophy whose diagnoses were confirmed by clinical and radiological examinations. Seven patients were treated with intranasal salmon calcitonin (100 IU twice daily) and 1 g elementary calcium daily for 5 weeks. The other group of seven patients was treated with 75 mg indomethacin daily for 5 weeks. The effectiveness of both treatments was analysed by comparing quantitative three-phase bone scans which were performed before and after the treatment. Bone-to-soft tissue ratios were calculated from static (delayed) images and scintigraphic evaluations were done both visually and quantitatively. Patients who received the calcitonin/calcium regimen showed reduced bone/soft tissue indices after therapy whereas patients who received indomethacin showed no change or showed increased indices. In conclusion, the results show that intranasal salmon calcitonin and calcium treatment is more effective than NSAIDs in patients with Sudeck's atrophy.