JMA Journal

Relapsing Polychondritis

2019-10-10T08:30:01+09:00

Cross-Sectional Survey of Childhood Acetabular Development in Japan

2019-09-10T08:30:02+09:00

: We aimed to clarify childhood acetabular development and to identify the incidence of children’s hip dysplasia in Japan using radiographs of the contralateral hip.　: We performed radiological cross-sectional evaluation of hip development in 211 patients (106 boys, 211 hips) in different age groups (age range: 3–9 years). We excluded patients who complained of bilateral coxalgia at the first visit or had received a diagnosis of acetabular dysplasia. We measured the acetabular index (AI), center-edge angle (CEA), and acetabular head index (AHI) in plain radiographs taken at the first visit.　: A significant correlation was found between age and CEA in boys, but other parameters had no significant correlation. The mean AI values in boys and girls were 18 ± 3° and 20 ± 4° (p < 0.01), respectively, and the mean CEA values were 25 ± 5° and 24 ± 5° (p = 0.43), respectively. The mean AHI values in boys and girls were 83 ± 6% and 81 ± 7%, respectively (p < 0.01). Two of the 120 children (66 boys and 54 girls) aged ≥6 years old had a hip CEA < 15°; both were girls.　: We found decreased acetabular development in girls, and 4% (2/54) of girls without any history of dislocation belonged to Severin’s group III. Acetabular dysplasia was observed more frequently in children from Japanese than in those from other countries. Girls with less than two standard deviations in hip dysplasia indices had an AI of 28°, an AHI of 67%, and a CEA of 14°. These reference values may be useful as prognostic indicators for hip dysplasia and OA in adulthood.

Clinical Genetics in Japan: Efforts of Human Genetics Societies and Related Organizations

2019-09-10T08:30:02+09:00

The Japanese government finally started measures to promote the realization of genomic medicine that can promote the accumulation of individual genomic information for improving medical care in 2015. However, readiness in terms of social infrastructure (including legal, administrative, ethical, and educational aspects in Japan) remains inadequate. Associations related to medical genetics have been making consistent efforts to realize genomic medicine by establishing guidelines, nurturing genetic professionals, providing support for constructing cross-disciplinary medical systems, enriching genetic education, etc., and it is important that the Japanese government supports these initiatives.

Bilateral Corneal Opacity of Fish-eye Disease

2019-09-10T08:30:02+09:00

Management of End-stage Renal Disease Associated with Systemic Rheumatic Diseases

2019-09-10T08:30:01+09:00

The outcomes of rheumatic diseases (RDs) have improved over the past decades. However, a significant proportion of the patients still suffer from end-stage renal disease (ESRD) and have to bear the burden of hemodialysis. It is crucial to prevent patients with RDs from developing ESRD from viewpoints of medicine and medical economics. For those who already have ESRD, it is important to improve vial prognosis and quality of life through appropriate management of disease activity and comorbidities related to ESRD. Thus, rheumatologists and nephrologists need to recognize risk factors associated with progression to ESRD along with their appropriate management. Although the activity of most RDs tends to decrease after initiation of hemodialysis, disease activity may still increase, and recognizing how to appropriately use immunosuppressive agents even after the development of ESRD is crucial. The treatment of RDs needs extra attention as hydroxychloroquine requires more frequent monitoring for adverse drug reactions; therapeutic drug monitoring is necessary for mycophenolate mofetil, cyclosporine A, and tacrolimus; cyclophosphamide and azathioprine need dose adjustments; methotrexate and bucillamine are contraindicated in patients with ESRD; leflunomide and sulfasalazine do not require significant dose reduction and iguratimod should be carefully administered. The pharmacokinetics of biological agents such as rituximab or belimumab are not affected by ESRD, and dose adjustments are not necessary. Collaboration between rheumatologists and nephrologists is needed more than ever and is expected to produce a complementary effect and achieve better outcomes in clinical settings, although this cooperation has not always been conducted appropriately.