Scoliosis in Elderly and Age-Related Bone Loss: A Population-Based Study

Osteoporosis, a common metabolic condition resulting in reduced bone mass, causes significant morbidity in affected individuals by predisposing them to osteoporotic fractures. To determine the relationship of scoliosis and osteoporosis, dual-energy X-ray absorptiometry (DXA) scans of 493 men and 762 women were analyzed according to their scoliotic status. No association was observed in the few scoliotic men without osteoarthritis of the spine compared to nonscoliotic men. Without osteoarthritis, scoliotic women had significantly decreased bone mass of the total hip and the femoral neck (p < 0.05) compared to nonscoliotic women, whereas in spine, bone mass was also decreased compared to nonscoliotic women, but not statistically significant. This suggests that scoliosis associates with bone loss, and affected women may benefit from early institution of therapeutic measures.     

Parosteal ossifying lipoma of the fibula: a case report with contrast-enhanced MR study and a review of the literature

This report describes a rare case of parosteal ossifying lipoma of the fibula. Very few reports have described the magnetic resonance (MR) imaging features with gadolinium enhancement of this neoplasm. In this case, low-signal-intensity strands within the lipomatous mass on T1-weighted image with varying degrees of enhancement were detected. Thus, parosteal ossifying lipoma should be included within the group of gadolinium-enhanced benign lipomatous tumours that may mimic liposarcoma on MR imaging. However, the characteristic radiographic appearance, together with computed tomography or MR imaging features, should aid in the correct diagnosis of this condition.     

Comprehensive and long-term outcomes of enzyme replacement therapy followed by stem cell transplantation in children with Gaucher disease type 1 and 3

Background

Gaucher disease (GD) is a lysosomal storage disorder, characterized by hepatosplenomegaly, pancytopenia, bone diseases, with or without neurological symptoms. Plasma glucosylsphingosine (lyso-Gb1), a highly sensitive and specific biomarker for GD, has been used for diagnosis and monitoring the response to treatment. Enzyme replacement therapy (ERT) is an effective treatment for the non-neurologic symptoms of GD. Neuronopathic GD (type 2 and 3) accounts for 60%–70% of the Asian affected population.

Methods

We explored combination therapy of ERT followed by hematopoietic stem cell transplantation (HSCT) and its long-term outcomes in patients with GD type 3 (GD3).

Results

Four patients with GD3 and one with GD type 1 (GD1) underwent HSCT. The types of donor were one matched-related, one matched-unrelated, and three haploidentical. The age at disease onset was 6–18 months and the age at HSCT was 3.8–15 years in the patients with GD3. The latest age at follow-up was 8–22 years, with a post-HSCT duration of 3–14 years. All patients had successful HSCT. Chronic graft-versus-host disease occurred in one patient. The enzyme activities were normalized at 2 weeks post HSCT. Lyso-Gb1 concentrations became lower than the pathological value. All of the patients are still alive and physically independent. Most of them (4/5) returned to school. None of the patients with GD3 had seizures or additional neurological symptoms after HSCT, but showed varying degrees of cognitive impairment.

Conclusions

ERT followed by HSCT could be considered as an alternative treatment for patients with GD3 who have a high risk of fatal neurological progression.     

Study of hematopoietic progenitor cells, hematological values and lymphocyte subsets in cord blood: application for cord blood transplantation

Hematological values, lymphocyte subsets and hematopoietic progenitor cells from normal term cord blood samples were studied, compared with normal adult blood, and analysed to determine whether a single collection of cord blood is sufficient for transplantation in adults. The parameters were assayed by automatic cells counter, flow cytometry and semisolid cell culture. All of the hematological values except RBC and MCHC were higher than in normal adult blood. Sex had an influence on RBC, Hb, Hct, Plt and reticulocyte counts. For lymphocyte subsets, all of the absolute CD3+, CD4+, CD8+ counts and T helper: suppressor ratio were higher than those of adult blood. All of the hematopoietic progenitor cells in cord blood were also higher than in adult blood. The mean volume of cord blood for each collection was 80.75 +/- 4.81 ml and the mean numbers of nucleated cells, CFU-GM and CD34+ were 13.51 +/- 0.38 x 10(8) cells, 4.33 +/- 0.66 x 10(5) colonies and 42.65 +/- 7.00 x 10(5) cells respectively. This 80 ml of cord blood would contain sufficient marrow repopulating cells for a recipient weighing about 20 kg. Recently developed technology, including ex vivo expansion may even permit transplants in adults.     

The effect of religious practice on the prevalence of knee osteoarthritis

The aim of this study is to evaluate the effect of religious practice on the prevalence, severity, and patterns of knee osteoarthritis (OA) in a Thai elderly population with the same ethnicity and culture but different religions. A house-to-house survey was conducted in two subdistricts of Phranakhon Sri Ayutthaya province where inhabitants are a mixture of Buddhists and Muslims. One hundred fifty-three Buddhists and 150 Muslims aged ≥ 50 years were evaluated demographically, physically, and radiographically. Those suffering knee pains were questioned about severity using the Western Ontario and McMaster University Osteoarthritis Index (WOMAC) scores and examined for their range of knee motion. Radiographic knee OA (ROA) was defined as Kellgren–Lawrence radiographic grade ≥2 while symptomatic knee OA (SOA) was defined as knee symptoms of at least 1 month in a knee with ROA. Muslims had on average a higher number of daily religious practices than their Buddhist neighbors (p < 0.001). The prevalence of knee pain and ROA was significantly higher in Buddhists than in Muslims (67.11 vs. 55.80, p = 0.02 for knee pain; 85.62 vs. 70.67, p = 0.02 for ROA). For SOA, Buddhists showed a trend towards higher prevalence than Muslims (47.71 vs. 37.32, p = 0.068). No significant difference was found when the range of motion and WOMAC scores were compared between the two groups. Muslims had a lower prevalence of OA than their Buddhists counterparts with the same ethnicity but different religious practice. The Muslim way of praying since childhood, forcing the knees into deep flexion, may stretch the soft tissue surrounding the knee and decrease stiffness and contact pressure of the articular cartilage.     

The serine protease inhibitor trappin-2 is present in cartilage and synovial fluid in osteoarthritis

OBJECTIVE: Trappins are small serine protease inhibitors bound to extracellular matrix (ECM) through the actions of transglutaminase (TGase) enzymes. Trappin-2 is present in many tissues and is upregulated at sites of injury. In osteoarthritis (OA), serine proteases contribute to articular cartilage destruction, and TGase activity is increased. Yet little is known about matrix-bound serine protease inhibitors or TGase substrates in articular cartilage. Our purpose was to determine if trappin-2 was present in OA cartilage and synovial fluid (SF). METHODS: OA knee articular cartilage and SF were assayed for trappin-2 protein by Western blotting, ELISA, and immunohistochemistry. Trappin-2 mRNA was detected with RT-PCR. The ECM components bound to trappin-2 were identified by 2-D gel electrophoresis and peptide fingerprinting. RESULTS: Trappin-2 was detectable in OA articular cartilage extracts, cultured chondrocytes, conditioned media, and SF by Western blotting. OA cartilage protein extracts contained significantly higher quantities of trappin-2 than normal cartilage protein extracts (22.98 +/- 1.28 ng/mg wet weight vs 14.97 +/- 1.92 ng/mg wet weight; p < 0.01). RT-PCR confirmed the presence of trappin-2 mRNA in OA chondrocytes. Immunohistochemical studies of OA cartilage revealed trappin-2 protein in chondrocytes. Peptide mapping of trappin-2 binding partners showed that fibromodulin was bound to trappin-2 in cartilage. CONCLUSION: We confirmed the presence of trappin-2 in OA cartilage and SF. Elevated levels of TGase activity in OA cartilage may increase levels of this serine protease inhibitor in response to injury.     

Prevention and control of thalassemia in Ramathibodi Hospital, Thailand

Eight thousand seven hundred and thirty-six pregnant women were screened for thalassemia and hemoglobinopathies by mean corpuscular volume less than 80 femtolitres (fl). Three thousand six hundred and seventy women (42%) were MCV less than 80 fl. In this group there were 2,390 women (70%) who had positive Hb typing by high performance liquid chromatography (HPLC) such as beta-thalassemia major, beta-thalassemia hemoglobin E disease, beta-thalassemia trait, heterozygous and homozygous hemoglobin E, alpha-thalassemia-1 trait and hemoglobin H disease and 77% of their partners came and had hemoglobin typing done. Seventy-five couples at risk for having severely affected thalassemia fetuses were detected from this screening program. Prenatal diagnosis was performed in 58 couples (77.3%). Eight affected fetuses were detected. All pregnancies with affected fetuses except one with beta-thalassemia/HbE were terminated. There were 3 fetal losses (6%) as the result of prenatal diagnosis procedure.