Renal Autotransplantation in a Patient with Bilateral Renal Artery Stenosis Secondary to Takayasu Arteritis.

Involvement of the renal artery is common in Takayasu arteritis. We, herein, present on a patient with Takayasu arteritis causing severe renal failure and a successful auto-transplantation. This case shows that early diagnosis and immediate appropriate interventions are life-saving in patients with Takayasu arteritis. Renal auto-transplantation performed in selected cases increases dialysis-free survival.

Clinical assessment of primary and secondary hypertension in children and adolescents.

The aim of this study was to compare the clinical features of patients with elevated blood pressure and to detect variables associated with the diagnosis of primary hypertension. We identified 383 (69%) hypertensive children (197 [51.5%] with primary hypertension, and 186 [48.5%] with secondary hypertension) out of 553 children referred to our clinic with a history of elevated blood pressure. The primary hypertension group was significantly older and had higher BMI, positive family history of hypertension, and lower prevalence of preterm birth compared with those with secondary hypertension. No difference was found between the two groups in terms of the frequency of target organ damage. Multiple regression analysis showed that a family history of hypertension, obesity, age over 10 years, elevated uric acid, and presence of higher systolic blood pressure values at admission were independent predictors of primary hypertension; therefore, these parameters can be considered important clues for diagnosing primary hypertension.

Clinical and radiological evaluation of surgical management in olecranon fracture-dislocations.

PURPOSE: The treatment of olecranon fracture-dislocations (OFDs) remains challenging. OFDs are often misdiagnosed as Monteggia lesions, and the real frequency is actually higher. However, studies on OFDs are limited. This study aimed to report on the surgical management of OFDs and to highlight the importance of three-dimensional computed tomography (3D CT) evaluation in the treatment of OFDs. MATERIALS AND METHODS: The study participants included 18 patients (11 men, 7 women, mean age 44 years (range 24-78) with OFDs. Each patient's medical records, radiographs, and 3D CT scans were reviewed for demographics, injury details, operative findings, and information about radiological and functional outcomes. The patients were divided into 2 groups according to the direction of the dislocation: the posterior dislocation group (group 1, 7 patients) and anterior dislocation group (group 2, 11 patients). The clinical evaluation was performed according to Broberg-Morrey and the American Shoulder and Elbow Surgeons-Elbow (ASES-E) scoring systems. RESULTS: The mean follow-up period was 39 months (range 25-62 months). The Broberg-Morrey results were excellent in 4, good in 9, fair in 3, and poor in 2 patients. The mean ASES-E score was 84.83 (range 48-100) points. There were signs of ulna-humeral arthrosis in 5 elbows. Arthrosis was graded as grade 1, grade 2, and grade 3 in 3, 1, and 1 elbows, respectively. Partial sensory recovery was observed in one patient with postoperative ulnar neuropathy at the last follow-up visit. CONCLUSIONS: OFDs are complex injuries of the proximal ulna and may involve the radial head, coronoid process, and lateral collateral ligament. The effective treatment of OFDs begins with the proper identification of the injury with 3D CT. A secure fixation including the coronoid process is mandatory for the elbow joint stability. Insufficient restoration of the trochlear notch may lead to problems with loss of motion and arthrosis. Although an application of a pre-contoured locking anatomical olecranon plate can simplify the fixation procedure in most cases, the surgeons' equipment should also include radial head implant, coronoid plates, headless screws, small cannulated screw system, suture anchors, fluoroscopy, and articulated external fixator.

Open reduction in pilon variant posterior malleolar fractures: Radiological and clinical evaluation.

BACKGROUND: Pilon variant posterior malleolar (PVPM) fractures significantly disrupt joint congruency and cause tibiotalar instability. They are often underestimated and inadequately treated. PURPOSE: This study assessed the outcomes of surgical treatment of this subtype of malleolar fracture, and examined the importance of computed tomography (CT) in diagnosis and surgical treatment. MATERIAL AND METHODS: CT images and radiographs of 67 patients with trimalleolar ankle fractures were retrospectively analyzed. Fourteen patients (6 women and 8 men) were studied. The mean age was 37.7 (range, 21-58) years, and mean follow-up period was 17.1 (range, 12-24) months. All patients underwent open reduction. Reconstruction of the joint surface was assessed with postoperative CT images. The outcomes were assessed with the American Academy of Orthopaedic Surgeons (AAOS) and Osteoarthritis (OA) scoring systems. RESULTS: The ratio of PVPM fractures to trimalleolar ankle fractures was 20.1%. Postoperative CT images demonstrated that anatomic reconstruction was achieved in 11 patients. The mean AAOS scores were 85.6 in Type 1 and 81.1 in Type 2 cases. The mean OA scores were 1 in Type 1 and 1.1 in Type 2 cases (P>0.05). The only statistically significant difference between the 2 groups was in osteochondral impaction (P<0.05). CONCLUSION: CT imaging is essential for the accurate diagnosis and management of PVPM fractures. Posteromedial and posterolateral incisions enable direct exposure, and therefore facilitate joint surface reconstruction. LEVEL OF EVIDENCE: Level IV. Retrospective study.

Metatarsal head resurfacing arthroplasty in the treatment of hallux rigidus: is it reliable treatment option?

BACKGROUND: This study looks at the failure and complications arising secondary to resurfacing and hemi-arthroplasty done at the metatarsal head in patients with hallux rigidus. Our report includes a review of the relevant literature to verify the validity of our techniques. MATERIALS AND METHODS: We performed metatarsal head resurfacing with hemi-arthroplasty using the HemiCap(®), on 12 patients with hallux rigidus between the dates of March 2010 and October 2013. The mean follow-up period was 22.3 months (range 12-54). All patients were clinically and radiologically evaluated according to the American Orthopedics Foot and Ankle Society (AOFAS) functional scale and the Coughlin and Shurnas classification. RESULTS: The recorded mean AOFAS score showed an increase from the preoperative score of 49.2 ± 13.1 to a postoperative follow-up score of 80.8 ± 13.1 (p < 0.001). Pain scores also showed an improvement from 16.5 ± 7.1 points preoperatively to 32.5 ± 6.9 points during the postoperative follow-up (p < 0.001). The mean function score improved from 17.7 ± 7.6 points preoperatively to 33.2 ± 7.6 points during the final postoperative follow-up (p < 0.001). Furthermore, the mean range of motion improved from 16.3 ± 4.8° preoperatively to 45.4 ± 13.2° postoperatively (p < 0.001). Three patients (25 %) reported pain at rest. Surgical revision was done on these patients who have significant pain that limited their range of motion. CONCLUSION: Favorable outcomes were achieved by performing minimal bone resection which also helps maintain metatarso-phalangeal joint function through metatarsal head resurfacing arthroplasty. We expect the failure rates to decrease with the advancements of surgical techniques. Selecting the appropriate patient populous in the application of the technique is crucial in attaining successful clinical results.

Anti-IL-1 treatment in familial Mediterranean fever and related amyloidosis.

Colchicine is the standard treatment in familial Mediterranean fever (FMF) patients. New treatment strategies are needed in FMF patients who were unresponsive to colchicine therapy or who had developed amyloidosis. The aim of this study was to present clinical-laboratory features and treatment responses of pediatric FMF patients that were treated with anti-IL-1 therapies. Files of patients who had been followed in our department with diagnosis of FMF were retrospectively evaluated. Patients that have been receiving anti-IL-1 therapies (anakinra or canakinumab) were included to the study. All patients were interpreted with respect to the demographic data, clinical and laboratory features of the disease, genetic analysis of MEFV mutations and treatment responses. Among 330 currently registered FMF patients, 13 patients were included to the study. Seven of them received anti-IL-1 therapy due to colchicine resistance and 6 due to FMF-related amyloidosis (1 of them with nephrotic syndrome, 2 with chronic kidney disease, 3 with renal transplantation). In all treated patients, attacks completely disappeared or decreased in frequency; partial remission occured in nephrotic syndrome patient; and their life quality improved. Anti-IL-1 therapies can be successfully used in colchicine-resistant FMF patients and patients with amyloidosis during childhood and adolescent period without major side effects.

Primary Hyperoxaluria Type 1: A Cause for Infantile Renal Failure and Massive Nephrocalcinosis.

Primary hyperoxaluria type 1 is a rare autosomal-recessive disease caused by the deficient activity of the liver specific enzyme alanine-glyoxylate aminotransferase. Increased endogenous oxalate production induces severe hyperoxaluria, recurrent urolithiasis, progressive nephrocalcinosis and renal failure. Here we report a 6 month old boy who presented with vomiting and decreased urine volume. He was diagnosed with chronic kidney failure at 4 months of age and peritoneal dialysis was introduced at a local hospital. His parents were third degree cousins and family history revealed 2 maternal cousins who developed end stage renal disease during childhood. When he was admitted to our hospital, laboratory studies were consistent with end stage renal disease, ultrasound showed bilateral massive nephrocalcinosis. As clinical presentation was suggestive for primary hyperoxaluria type 1, plasma oxalate was determined and found extremely elevated. Genetic testing proved diagnosis by showing a disease causing homozygous mutation (AGXT-gene: c.971_972delT). The patient was put on pyridoxine treatment and aggressive dialysis programme. In conclusion; progressive renal failure in infancy with massive nephrocalcinosis, especially if accompanied by consanguinity and family history, should always raise the suspicion of PH type 1. Increased awareness of the disease would help physicians in both treating the patients and guiding the families who have diseased children and plan to have further pregnancies.