Multi-center analysis of calcinosis in children with juvenile dermatomyositis]

OBJECTIVES: To reveal the frequency and the clinical characteristics of dystrophic calcification that occurs in children with juvenile dermatomyositis, multi-center analysis was constructed. METHOD: Fifty children with JDM were enrolled, and 14 of them (28.0%) were complicated with calcinosis. Clinical symptoms and laboratory tests at onset, initial therapy and disease course were compared in children with and without calcinosis. RESULTS: The mean age of the onset of calcinosis was 4.78 +/- 3.33 years, and it was younger than those of children without calcinosis (8.66 +/- 3.85 years) (P = 0.0017). No differences of clinical manifestation except Gower's sign were observed. The frequency of positive anti-nuclear antibody was 7.1% in children with calcinosis and 52.9% without calcinosis (P = 0.0112). The initial therapy of methylprednisolon pulses gave no effects on prognosis of calcium deposition. The calcinosis appeared in 1.56 +/- 1.91 year after the onset of the disease. The various types of calcium deposition including large tumorous clumps, subcutaneous plaques or nodules, sheet-type calcification were deserved. They appeared over knee joints (64.3%), elbow joint (64.3%), and hip processes (50.0%). Calcinosis affecting the subcutaneous tissues frequently resulted in painful superficial ulceration of the overlying skin (42.9%), local infection (50.0%), and limitation of joint movement (14.3%). Although aluminum phosphate was effective in 2 children among 7, no other effective treatment was recommended. In 5 cases, surgical removal of tumorous clumps was operated. Thus, juvenile dermatomyositis is frequently complicated with calcinosis. This type of calcinosis was found to be unlikely to resolve completely, and resulted in severe disability in children.     

A case of drug eruption induced by hydroxyzine pamoate]

A 62-year-old woman with rheumatoid arthritis, Basedow's disease and arrhythmia has been treated with antirheumatic, antiarrhythmic drugs and so on. She developed pruritic diffuse erythema with papules on the trunk and extremities 2 days after taking hydroxyzine pamoate for asteatotic eczema. Laboratory data showed increased levels of eosinophils. Histopathological examination revealed a infiltrate of inflammatory cells in the upper dermis. Patch tests with hydroxyzine pamoate and hydroxyzine hydrochloride were positive. From these findings, we diagnosed this case as drug eruption due to hydroxyzine. Her eruption subsided after she discontinued hydroxyzine pamoate and other drugs which were started within 5 days before the onset of the eruption and was treated with systemic steroid, systemic antiallergic drug and topical steroid.     

Bronchial venous plexus and its communication with pulmonary circulation

This study was undertaken to clarify the microvascular anatomy of the normal human bronchial circulation, focusing on the bronchial venous system and its communication with the pulmonary circulation. Ten normal lungs obtained at autopsy were studied. Bronchial arteriography was performed in six lungs, pulmonary arteriography in two lungs, and pulmonary venography in another two. Using radiologic methods, including microradiography, and serial histologic sections, we found numerous bronchial venous plexuses around the airways and blood vessels into which the bronchial capillaries drained. Frequent communications of the venous plexuses with the pulmonary veins and neighboring alveolar capillaries were also demonstrated. The bronchial venous plexus and its close connection with the pulmonary circulation may be important in various pathologic conditions involving the interstitial space, such as interstitial pulmonary edema.     

Successful Treatment of Adult T-cell Leukemia/Lymphoma with MACOP-B,M-FEPA and VEPP-B Combination Chemotherapy

A 45-year-old man was referred to our department in March of 1989. Physical examination showed erythroderma, palmo-plantar hyperkeratosis, generalized lymphadenopathy, hepatosplenomegaly, and leukemic manifestation. The lymphocyte count in the peripheral blood before treatment was 1.7 × 10⁴ cells/mm³. Atypical lymphocytes such as flower cells and lobulated cells were seen in the peripheral blood. A sample excised from a lymph node showed immunoblastic, pleomorphic T cells by a modified classification scheme of the Working Formulation. A high level of serum LDH was detected (2.1 times the upper normal limit). Anti HTLV-1 antibody was also detected in the serum. The atypical lymphocytes were positive for CD3, CD4, CD5, CD7 and HLA-DR, and negative for CD8. Thus, the clinical, pathologic and immunologic features were those of typical acute-type ATL. The patient was treated with VEPA-M for three months starting in March of 1989. Because of poor response, the patient was then treated with MACOP-B, M-FEPA, and VEPP-B for about one year from June of 1989 and has been free of disease up to the time of writing, March of 1993.     

Spontaneous spinal epidural hematoma diagnosed by MRI: a case report

Appropriate diagnostic procedure for spinal epidural hematomas has not been established yet. The authors reported a case of spontaneous epidural hematomas at the thoracic level, in which correct diagnosis was made with MRI and good results were obtained by surgery. A 63-year-old female experienced a severe back pain which appeared suddenly during a walk and was followed by motor weakness in both legs deteriorating quickly to paraplegia. The patient had no history of hypertension, trauma or bleeding tendency. The laboratory data were normal. On admission, neurological examination revealed flaccid paraplegia, total sensory loss below the level of Th 6 and urinary and fecal incontinence. Myelograms showed incomplete block at the Th 6 level and postmyelographic CT scan showed an isodense mass, which was suspected to be an epidural tumor located behind the spinal cord. Emergent MRI confirmed an epidural hematoma as a high intensity area extending from Th 3 through Th 11. Sixty-five hours after onset, laminectomy of Th 4 through Th 11 and the evacuation of epidural hematoma were performed without identification of the origin of the bleeding. Neither vascular malformation nor tumor was recognized during operation. Neither was it noticed on histological examination. The patient made favorable progress after the surgery. During the first two weeks in the postoperative period, she regained muscle strength enough to do standing exercise, and satisfactory improvement was made in sensory function including urination and defecation. We emphasize that MRI is indispensable to make a differential diagnosis of thoracic lesions. In the reported case, a correct diagnosis was made with MRI, and an extremely good result was obtained by an emergency operation.     

Characteristics of Pubertal Growth in Japanese Children from the Standpoint of Skeletal Growth

Since the end of the 2nd World War, Japan has seen quite rapid socioeconomical development. With this development the physical size of Japanese children has increased, but the final size, especially the stature, is still shorter than that of Americans or Europeans. Bone maturation velocities were compared among Japanese and Chinese children and adolescents aged 7 to 18 (in 1986) and English TW2-subjects, using the TW2 method. Asian children and adolescents may have a different tempo of skeletal maturation during pubertal growth from that of English children and adolescents. This, probably genetic, difference in the tempo of skeletal maturation, especially that of RUS, between Japanese and English during pubertal growth may be one of the main causes of the final difference in the stature of the two groups.