Evaluation of anticoagulant system in Turkish children with Perthes disease

BACKGROUND: Perthes Disease (PD) is generally a self-limiting disease of childhood but it causes severe pain and may lead to deformity of the femoral head. Intravascular thrombosis seems to form the main mechanism in the pathogenesis of the disease. The aim of this study was to determine hereditary thrombotic risk factors in Turkish children with PD. METHODS: In 46 Perthes patients (35 male, 11 female), family history of thrombotic events was investigated, Protein C (PC), free-Protein S (f-PS), antithrombin (AT) activities, fibrinogen level, and resistance to activated Protein C (APC) were measured. The results were compared with a healthy control group of 79 children matched by age and sex. The relationship between the severity of disease and coagulation system abnormalities was evaluated. RESULTS: While the mean PC and AT activities were significantly lower in the patients than those of the controls, the proportions of patients with low AT activity, resistance to APC, and a history of hereditary thrombophilia were significantly higher than those of the controls. No difference was observed in coagulation system disorders relative to severity of the disease and bilateral or unilateral disease involvement. CONCLUSIONS: This study shows that a possible association between PD and inherited hypercoagulability. Determination of thrombotic risk factors in these patients may bring a new approach to the treatment. Most importantly, this may be a stimulant to take precautions for other thrombotic events, which patients may face later in life.     

Relationship between SP1 polymorphism and osteoporosis in β-thalassemia major patients

Background: β-Thalassemia is an autosomal recessive disease characterized by defective β-globin chain production. Osteoporosis is an important cause of morbidity in patients with β-thalassemia major. The pathogenesis of reduced bone mineral density (BMD) is multifactorial. A range of genetics factors have been implicated in other populations of patients with osteoporosis. Polymorphism at the Sp1 binding site of the collagen type I A1 ( COLIA1 ) gene is thought to be an important factor in the development of osteoporosis.
Methods: Alleles S and s , detected by presence of a G or T nucleotide, respectively in a regulatory site of the COLIA1 gene were investigated in 37 β-thalassemia major patients with osteoporosis and 92 controls without osteoporosis or osteopenia using polymerase chain reaction–restriction fragment length polymorphism.
Results: Fifteen and nine β-thalassemia major patients displayed SS and Ss genotypes, respectively, whereas 13 were found to have an ss genotype. The mean BMD of the β-thalassemia major patients with ss genotype was similar to those with the Ss and SS genotypes. In the control group, 77 and 15 subjects had SS and Ss genotypes, respectively, with no ss genotype. Allelic and genotypic distribution in patients were significantly different from controls.
Conclusion: Determining base substitutions at the Sp1 binding site on the COLIA1 gene in early years may be important in preventing osteoporosis in children with β-thalassemia major.     

Long-term follow-up of tonsillectomy efficacy in children with PFAPA syndrome

Introduction

The role of tonsillectomy in the periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome, is controversial. Although some studies reported high success rates with tonsillectomy, further investigations are needed with larger numbers of patients.

THE EFFECT OF TRAVOPROST AND BI-MATOPROST ON BLOOD FLOW VELOCI-TY OF THE RETROBULBAR VESSELS

Objective: To investigate the effects of topical travoprost 0.004% and topical bimatoprost 0.03% on retrobulbar blood flow (RBF). Methods: Nineteen young healthy men without any known ocular or systemic disorder were enrolled in this prospective, double-masked, randomized, cross-over study. Baseline RBF mea-     

Retinacular band excision improves outcome in treatment of plica syndrome

Mild symptoms usually continue after excision of the medial patellar plica. We noticed that the palpable tender cord, located on the anteromedial aspect of the knee in patients with plica syndrome, did not disappear completely after excision of the synovial fold. Beneath all plicae, a retinacular band was visible, and only after excisions of this band did the cord become impalpable. We conducted a study to determine the role of these medial retinacular bands in the symptomatology of the disorder. Twenty-four knees of 22 patients diagnosed with medial patellar plica syndrome were divided into two groups. In the first group, arthroscopic excision of the synovial plica was performed. In the second group, retinacular bands beneath the plica were additionally excised. When Lysholm scores were compared, we found that the second group showed significantly greater improvement. We believe that the retinacular bands play a role in the symptomatology and the pathophysiology of plica syndrome and that excision improves the outcome.

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Résumé Habituellement des symptômes discrets persistent après excision du plica médiopatellaire. Nous avons constaté que le cordon palpable présent dans la partie antéromédiale du genou chez les malades avec un syndrome de plica na pas complètement disparu après excision du repli synovial. Sous tous les plicas une bande retinaculaire était visible et seulement après excision de cette bande le cordon est devenu impalpable. Nous avons conduit une étude pour déterminer le rôle de ces bandes rétinaculaire médiales dans la symptomatologie persistante. Vingt-quatre genoux de 22 malades présentant un syndrome du plica médio patellaire ont été divisés en 2 groupes. Lexcision arthroscopique du plica synovial était faite dans le premier groupe et dans le deuxième groupe la bande rétinaculaire sous le plica a été excisée en plus. Quand les scores de Lysholm ont été comparés nous avons vu que le deuxième groupe avait une meilleure amélioration, statistiquement significative. Nous croyons que cette bande rétinaculaire joue un rôle dans la symptomatologie et la physiopathologie du syndrome du plica et que son excision améliore le résultat.

     

Intraspinal metalloma resulting in late paraparesis

The metal-related complications caused by orthopedic implants have long been a concern, but these problems have been considered mostly in the field of arthroplasty or internal fixation of fractures. The recent prevalence of spinal instrumentation has evoked a similar concern among spine surgeons. Here, we present a case of intraspinal metallosis adjacent to the pedicular hook occurring after treatment of vertebral fracture by posterior spinal instrumentation and fusion, and causing paraparesis at the 3rd postoperative year. Metallic granulomas can appear around the pedicular hooks as in the reported case. Crevice and fretting corrosion are results at the junctions of rod-screw, rod-hook, transverse connector rod and other connector rods in modular spinal implants. Adequate usage of transpedicular screws may inhibit the occurrence of such a complication. For this reason, further studies are necessary to increase metallic corrosive resistance to inhibit crevice and fretting corrosion.     

Development of calciphylaxis after long-term steroid and methotroxate use in a patient with rheumatoid arthritis

Calciphylaxis may be considered a small vessel vasculopathy which is generaly associated with end-stage renal disease and hyperparathyroidism. The precise pathogenesis of the disease is not known. It needs sensitizers and challengers to occur. Steroids and immunosuppressive drugs including methotrexate are among those challenger agents. Calciphylaxis in collagen vascular diseases is rare. Only one case in rheumatoid arthritis was recently reported. Here we describe a case of calciphylaxis associated with active rheumatoid arthritis. This patient had active disease despite treatment of steroids and methotrexate for a long time. She died shortly after the diagnosis of calciphylaxis due to sepsis.