Serum Sclerostin Level and Bone Mineral Density in Pediatric Hemophilic Arthropathy.

OBJECTIVE: To assess serum sclerostin levels in relation to severity of arthropathy and bone mineral density (BMD) in children with hemophilic arthropathy. METHODS: This cross-sectional study included 40 male children suffering from Hemophilia A, and 10 matched healthy controls. Assessment of factor VIII deficiency degree, frequency of bleeding, type of treatment, body mass index (BMI), disease severity using the Hemophilia Joint Health Score (HJHS) and lumbar spine (LS) Z score for bone mineral density (BMD) using dual-energy X-ray absorbiometry was done. Serum sclerostin levels were measured for all patients and controls. RESULTS: Significant difference of serum sclerostin levels between the patient and control groups with Mean ± SD (0.09 ± 0.07 ng/ml) and (0.04 ± 0.01 ng/ml) (P value = 0.028) respectively was found. Significant positive correlations between serum sclerostin levels and the patients' age, and HJHS (P value <0.05) were found, while it had negative correlation with DEXA Z score, not reaching a significant value. LS-BMD-Z score levels ranged from (-4.5 to 1.2), with 15 patients with low BMD Z score (less than -2) representing 37.5% of total patients. CONCLUSIONS: Serum sclerostin levels are elevated in hemophilic children denoting bone metabolism affection and correlates with increased age, and HJHS. Increased levels of serum sclerostin may identify hemophilic patients at high risk for developing osteoporosis.

The Egyptian Arabic version of the Juvenile Arthritis Multidimensional Assessment Report (JAMAR).

The Juvenile Arthritis Multidimensional Assessment Report (JAMAR) is a new parent/patient-reported outcome measure that enables a thorough assessment of the disease status in children with juvenile idiopathic arthritis (JIA). We report the results of the cross-cultural adaptation and validation of the parent and patient versions of the JAMAR in the Egyptian Arabic language. The reading comprehension of the questionnaire was tested in ten JIA parents and patients. Each participating centre was asked to collect demographic, clinical data and the JAMAR in 100 consecutive JIA patients or all consecutive patients seen in a 6-month period and to administer the JAMAR to 100 healthy children and their parents. The statistical validation phase explored descriptive statistics and the psychometric issues of the JAMAR: the 3 Likert assumptions, floor/ceiling effects, internal consistency, Cronbach's alpha, interscale correlations, test-retest reliability, and construct validity (convergent and discriminant validity). A total of 100 JIA patients (20.0% systemic JIA, 40.0% undifferentiated arthritis, 24.0% RF negative polyarthritis, 16.0% other categories) and 100 healthy children were enrolled in one paediatric rheumatology centre. The JAMAR components discriminated well healthy subjects from JIA patients. All JAMAR components revealed satisfactory psychometric performances. In conclusion, the Egyptian Arabic version of the JAMAR is a valid tool for the assessment of children with JIA and is suitable for use both in routine clinical practice and in clinical research.

Sympathetic Dysfunction in Patients With Chronic Low Back Pain and Failed Back Surgery Syndrome.

BACKGROUND: Chronic low back pain (CLBP) is defined as pain that persists longer than 12 weeks and is often attributed to degenerative or traumatic conditions of the spine. Failed back surgery syndrome is a condition in which chronic pain persists after spinal surgery. Electrodiagnostic studies can be used to confirm the diagnosis of lumbosacral radiculopathy, but other diagnostic methods are often needed to assess sympathetic nervous system dysfunction. OBJECTIVES: The aim of this study was to investigate the affection of sympathetic skin response (SSR) in cases of chronic low back pain (LBP) and failed back surgery syndrome (FBSS) and to assess the association of SSR abnormalities with perceived functional disability and pain among these patients. METHODOLOGY: Twenty patients with CLBP and 10 patients with failed FBSS who fulfilled the inclusion criteria were recruited to the present study. All cases had back, leg, or back and leg pain of at least 3-month duration or following spinal surgery. The control group consists of 10 healthy participants matched in age and sex. Electrophysiologic nerve conduction studies and SSR recording were applied on the symptomatic and normal side in study cases and on both sides in the control group. Pain intensity was analyzed by the visual analogue scale (VAS) and perceived functional disability was assessed with the Oswestry disability index (ODI). CONCLUSIONS: It was concluded that the sympathetic nervous system is affected in CLBP and FBSS patients with abnormalities in SSR and that the dysfunction of sympathetic nervous system may contribute to the intensity and chronicity of pain in these groups of patients. Moreover, a strong association was found between SSR and functional disabilities in these patients.

Orthopaedic manifestations of Proteus syndrome in a child with literature update.

BACKGROUND: Proteus syndrome is a rare developmental disorder of unknown aetiology. It is a disorder characterized by postnatal overgrowth affecting multiple tissues. Proteus syndrome is most frequently manifested in skeletal changes. As manifestations of Proteus syndrome are highly variable, and many are found in other overgrowth syndromes, and due to inconsistent application of diagnostic criteria, the literature has more reports of patients misdiagnosed than correctly diagnosed. The purpose of this study is to report the clinical and radiographic patterns of affection of the musculoskeletal system in Proteus syndrome in the light of the proposed diagnostic criteria and cases reported in the literature. METHODS: The clinical and radiographic musculoskeletal characteristics of a child with Proteus syndrome are illustrated along with a literature update. The orthopaedic manifestations in our patient are correlated to cases and proposed diagnostic criteria reported in the literature. RESULTS: The study of the presented case and review of available literature show that there tends to be a highly characteristic pattern of skeletal abnormalities in Proteus syndrome. CONCLUSION: The rarity of Proteus syndrome and the variability of signs make the diagnosis challenging. Clinical and radiographic examinations are important contributors to the diagnosis. The clinical utility of the reported cases is significantly dependent on consistent application of diagnostic criteria that augment diagnostic accuracy. The present case reinforces the need for supplementary musculoskeletal imaging modalities to be implemented in the diagnosis of Proteus syndrome.

Endoscopic-assisted achilles tendon reconstruction with free hamstring tendon autograft for chronic rupture of achilles tendon: clinical and isokinetic evaluation.

PURPOSE: To evaluate the clinical and functional outcome of endoscopic-assisted reconstruction of chronic ruptures of the Achilles tendon using free hamstring tendon autograft. METHODS: We present a case series of 15 patients who had chronic ruptures of the Achilles tendon (>6 weeks earlier) and underwent endoscopic-assisted reconstruction with a free hamstring autograft. The graft loop was passed through and fixed to the proximal stump of the tendon. The graft was then passed through suture to the distal stump and finally inserted into a tunnel in the anterior calcaneus to the Achilles tendon insertion and fixed with an bioabsorbable interference screw. The mean follow-up period was 27 months (SD, 3 months; range, 24 to 33 months). All patients underwent magnetic resonance imaging preoperatively, immediately postoperatively, and at follow-up 2 years postoperatively. All patients were functionally evaluated with the American Orthopaedic Foot & Ankle Society (AOFAS) score for the hindfoot preoperatively and postoperatively. Calf muscle power was evaluated by isokinetic strength testing at 2 years' follow-up. RESULTS: The mean size of the gap on preoperative magnetic resonance imaging was 49 mm (SD, 9 mm). The mean preoperative AOFAS score was 32.6 (SD, 7.5). There was a statistically significant improvement in the postoperative AOFAS score after 2 years to 90.8 (SD, 3.54) (P < .05). The mean time of return to all daily activities (except running and other sports) was 12.6 weeks (SD, 1.39 weeks). Isokinetic testing showed a nonsignificant deficit (<10%) between the involved and uninvolved plantar flexors and dorsiflexors with regard to peak torque, average power, and total work. CONCLUSIONS: Endoscopic-assisted Achilles tendon reconstruction with free hamstring tendon autograft for chronic ruptures of the Achilles tendon showed good to excellent results in all patients. Isokinetic testing showed a nonsignificant deficit between the involved and uninvolved sides at 2 years' follow-up. LEVEL OF EVIDENCE: Level IV, therapeutic cases series.