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Biomedical subjects

S Fujikawa

Publications and source records attributed to S Fujikawa.

At least 55 records · Page 3Linked to original sources
PubMed1997

Clinical analysis of 570 cases with juvenile rheumatoid arthritis: results of a nationwide retrospective survey in Japan.

The purpose of the present study was to investigate the incidence of juvenile rheumatoid arthritis (JRA) among Japanese children and to evaluate the clinical features of this disease. A questionnaire was sent to the department of pediatrics of 1290 hospitals in Japan, in 1994, asking for the number of rheumatic patients during the past 10 years. Subsequently, a second questionnaire was sent asking for the type of onset, clinical features, treatment, and other details. The results of 570 cases were obtained. Of these, 310 cases (54%) were the systemic onset type, 140 cases were the polyarticular onset type (25%), and 120 cases (21%) were the pauciarticular onset type. Hence, in the present series of children, the proportion of the pauciarticular type was less than the other two types of JRA. In the laboratory findings of the systemic onset type, hyperferritinemia and thrombocytosis were noted, in addition to leukocytosis, positive C- reactive protein (CRP) and accentuated erythrocyte sedimentation rate (ESR). The rheumatoid factor was positive in 50% of patients with the polyarticular onset type. Chronic uveitis was recognized in 13 cases (10.8%) of the pauciarticular onset type. In four girls, uveitis started before the onset of arthritis. Non-steroidal anti-inflammatory drugs were used in almost one-third of cases, and methotrexate (MTX) was used in 12.8% of cases. The quality of life of children with JRA was disturbed in almost 20% of cases. Therefore, for the early and definitive diagnosis of the systemic type of JRA, diagnostic procedures including thrombocyte counts and serum ferritin level, should be performed. In order to obtain good results and to avoid side effects, a protocol for the use of disease modifying anti-rheumatic drugs and immunosuppressants, especially for the use of MTX, must be established.

Anti-Inflammatory Agents, Non-Steroidal↗
PubMed1997

Clinical features of Japanese children and adolescents with systemic lupus erythematosus: results of 1980-1994 survey.

Marked advances have been made in the past decade in the management of adults with systemic lupus erythematosus (SLE). Therefore, a nationwide retrospective survey was conducted between 1980 and 1994 to investigate the clinical manifestations of SLE in Japanese children and adolescents. Questionnaires were sent to 340 hospitals. Of 405 patients reported by 176 hospitals, 373 patients, diagnosed by the criteria established by the Pediatric Study Group of the Japanese Ministry of Health and Welfare in 1985, were enrolled in the study. Forty-nine of the 354 patients (13.8%) had relatives with a connective tissue disease within the third degree of consanguinity. The frequent manifestations in 373 patients were the presence of antinuclear antibody (98.9%), immunologic disorders (93.0%), hypocomplementemia (87.1%), malar rash (79.6%) and fever (74.0%). Lupus nephritis was present in 148 of the 309 patients (47.9%) at their first visit to a clinic, and 261 of the 373 patients (70.0%) developed renal involvement during the observation period. Of 370 patients, 92 patients (24.9%) exhibited central nervous system lupus. Of 368 patients, 192 patients (52.2%) were treated by methylprednisolone pulse therapy and 148 patients (40.2%) received immunosuppressants in combination with steroid therapy at some stage during the observation period, Survival rate at 5 years from onset was 95.9%. Management of infection, coagulopathies, and central nervous system involvement is essential to improve the prognosis of SLE in Japanese children and adolescents.

Adolescent↗
PubMed1997

Characteristics of juvenile dermatomyositis in Japan.

Questionnaires were sent to 1290 hospitals in Japan asking for data on patients with juvenile dermatomyositis (JDM) diagnosed between June 1984 and May 1994. Of the 204 patients identified by these questionnaires, 102 met the criteria for JDM. JDM is categorized into three subtypes: Banker-type JDM, Brunsting-type and fulminant-type; patients with the latter exhibit markedly elevated serum levels of creatinine phosphokinase (> 10,000 U/mL) and appear to be at risk of renal failure. Cutaneous manifestations were present in 98% of patients and preceded the appearance of other symptoms. This tendency is one of the reasons for the difficulty in some cases in diagnosing the onset of JDM. Better criteria for early treatment of JDM are needed. The results of the present study suggest that itching and calcinosis are factors that indicate a poor prognosis in patients with JDM. Muscle enzyme levels do not always reflect disease activity, suggesting that methods other than measurement of muscle enzymes, such as measurement of the levels of neoprerin and von Willebrand factor antigen, as well as magnetic resonance imaging should be used to be evaluate disease severity. Patients with Brunsting-type JDM who exhibit dysphagia and antinuclear antibody positivity and patients with Banker-type JDM should be treated aggressively. Pulse therapy should be selected as the initial therapy in patients with fulminant-type JDM.

Child↗
PubMed1997

Systemic sclerosis in children: a national retrospective survey in Japan.

A retrospective questionnaire survey of pediatric departments, for childhood collagen disease from 1985 to 1994 was used to clarify the clinical features of scleroderma in Japan. In the primary survey, 0.9% of the children with a rheumatic condition and scleroderma. Answers to this questionnaire were received on 18 (localized 9; systemic 9) patients from 15 institutions. In order to examine systemic sclerosis (SSc), seven cases of SSc in Japanese articles during the same period as the questionnaire were added to these answers and compared to the Japanese epidemic study investigated by Fukuyama in 1974. There were 16 children, seven boys and nine girls, with SSc during the 10-year period in Japan. The mean age of onset of symptoms was 8.0 +/- 2.8 years and the age at diagnosis 10.1 +/- 3.0 years. Eighty percent of children had Raynaud's phenomenon at the onset of SSc, and skin and musculoskeletal involvement was highly recognized during the course of the disease. Atrophy of the frenulum linguae and lung fibrosis were commonly seen in SSc. In serological studies, 80% of children have antinuclear antibodies and approximately 50% of patients have anti-Scl-70 (topoisomelase I) antibodies at the onset and during the course of childhood SSc. The prognosis is poor, as remission occurred in only one child. The clinical symptoms and examination of serological autoimmune antibodies were supportive of an early diagnosis of SSc. When compared to the previous national survey of children with SSc, the present results showed that the male-to-female ratio was reduced, the age at onset was low, the positive incidence of serological autoimmune antibodies elevated, and the usage of vasodilators and nonsteroid anti-inflammatory drugs (NSAID) increased, with corticosteroids decreased. But, the positive percentage of clinical symptoms were not changed in both studies. For a complete retrospective nationwide epidemic survey carried out on children with scleroderma, especially SSc, it is important to include dermatology departments.

Adolescent↗
PubMed1997

The clinical features of Sjögren's syndrome in Japanese children.

Sjögren's syndrome (SS) is thought to be uncommon in children. An epidemiological study to describe the clinical features distinguishing SS in Japanese children was performed by sending questionnaires to hospitals. A total of 61 cases of SS were reported from 1290 hospitals. The diagnosis of SS was based on histopathological changes and/or sialographic changes in the salivary glands. Forty-two cases had primary SS and 19 were secondary SS with other autoimmune disorders. Fourteen cases (65%) of secondary SS were associated with systemic lupus erythematosus. In primary SS, the initial symptoms were systemic manifestations (fever, exanthema, arthralgia, etc) except for sicca symptoms. In laboratory studies, antinuclear antibodies, elevated serum IgG, rheumatoid factor, anti-Ro/SS-B antibodies were frequently observed.

Child↗
PubMed1997

Mixed connective tissue disease in childhood: a nationwide retrospective study in Japan.

Sixty-six children with mixed connective tissue disease (MCTD) were analyzed by a nationwide prospective study. The diagnostic significance of Raynaud's phenomenon and positive anti-RNP antibody was confirmed, and additional symptoms including swelling of fingers, facial erythema, and polyarthralgia, and laboratory findings such as positive rheumatoid factor, hypergammaglobulinemia, and increased levels of myogenic enzymes, were variably positive. These clinical and laboratory characteristics of MCTD were critically different from those of systemic lupus erythematosus, indicating that MCTD is an independent entity of disease.

Adolescent↗
PubMed1997

Clinical observation of 14 cases of childhood polyarteritis nodosa in Japan.

An epidemiological survey of childhood polyarteritis nodosa was conducted at 1290 hospitals followed by a secondary survey of hospitals that had therapeutic experience with the disease. Fourteen cases obtained from these surveys were studied clinically. The male-female ratio was 4:3. The mean age at onset was 10.6 years. To established the diagnosis, skin biopsy was performed in 43%, retrograde aortography in 21%, and kidney biopsy in 14% of cases. Non-specific symptoms such as fever (86%), skin eruption (50%), arthritis (50%), myalgia (50%), abdominal pain (43%) and hypertension (36%) were often noted as clinical findings at the initial visit. Throughout the clinical course, symptoms considered to be caused by local angiopathy, including interrupted blood circulation, were frequently noted. On blood tests, no specific findings other than acute inflammatory reaction were observed. Steroid preparations were used for treatment in all cases but one, and about half of them are presently free from steroids. Immunosuppressants were administered in 10 cases. As for prognosis, all patients are alive, but untoward sequelae were noted in 36% of cases. The results of the present survey reinforced the difficulty of establishing a final differential diagnosis because specific findings were scarce for this disease, as stated in previous reports. Establishing a method for early diagnosis by gathering and analyzing more data in detail will be necessary in the future.

Child↗
PubMed1997

Aortitis syndrome in children: clinical observation of 35 cases in Japan.

The results of clinical observation of 35 patients with aortitis syndrome (AS) in childhood, obtained by a nationwide survey in Japan, are reported. The male to female ratio was 1:2.5, the estimated age of onset averaged 10.2 years, and the duration from the estimated age of onset to the diagnosis averaged 15 months. In HLA examination A24, Bw52, Cw7 and DR2 were relatively common. Arterial lesions tended to extensively involve the aortic arch and its branches. Fever was the most frequently noted clinical symptom, followed by abdomen, joint and muscle pain. The physical findings in order of frequency were impaired circulation of the upper extremities, cardiac and vascular murmurs, hypertension, impaired cerebral circulation, visual disorder and impaired circulation of the pulmonary artery. The murmurs were found not only over the chest wall but also over the cervical area and abdomen. Pulselessness of the upper extremities occurred in 66% of patients. Percutaneous retrograde aortography and/or intravenous digital subtraction angiography to make the final diagnosis was employed except for three cases. There were not any specific abnormal signs in laboratory data. Steroid hormones were administered in 34 cases, and immunosuppressive agents in 8 cases. Five cases had percutaneous transluminal angioplasty to the right renal artery as an interventional treatment. The high frequency of abdominal pain is considered to be one of the characteristics of AS in childhood. The high frequency of pulselessness of the upper extremities and cardiac and vascular murmurs in this report is considered significant for the diagnosis of AS in childhood.

Aortitis↗
PubMed1997

Behçet disease in children: a nationwide retrospective survey in Japan.

The purpose of the present study was to evaluate the incidence of Behçet disease among Japanese children and to compare their clinical features with adult patients. A first questionnaire was sent to the department of pediatrics of 1290 hospitals in Japan. Fifty-one cases of Behçet disease among children under 16 years of age were reported. From a second survey, 31 cases satisfied the criteria of the Behçet's Syndrome Research Committee of Japan. Of 31 patients (14 boys and 17 girls), three cases were the complete type, 24 cases were the incomplete type, and four cases were the possible type. As to the specific type of the disease, 10 cases were of the entero-Behçet type, two were a combination of the entero-vasculo-Behçet type, and four were the neuro-Behçet type. In the first 6 months after onset, oral ulcers were observed in 77% of cases, genital ulcers in 45%, uveitis in 10%, and skin lesions were observed in 39% of cases. During the course of the disease, the frequency of oral ulcers was 100%, genital ulcers, 58%; ocular complications, 29%; and skin lesions, 55%. Gastrointestinal signs and symptoms were more frequent in childhood Behçet disease than in adults, while ocular complications were less frequent. The diagnosis of Behçet disease in children is difficult because of the long interval before the onset of enough manifestations to satisfy the diagnostic criteria. Specific diagnostic criteria are necessary, based on a discussion of the many childhood cases of Behçet disease.

Adolescent↗
PubMed1997

[Transient hyperphosphatasemia observed in a boy with acute lymphoblastic leukemia].

A detailed time course of alkaline phosphatase (ALP; EC3.1.3.1) activity of transient hyperphosphatasemia (TH) in a 9-year-old boy with acute lymphoblastic leukemia (ALL) is described. The patient's serum ALP activity rose transiently to 49 times the upper limit of normal adult, without any evidences of hepatic and bone disease. The half-life of ALP activity was calculated about 10 days. We characterized ALP isoenzymes by usual electrophoresis using cellulose acetate membrane (Titan III iso-vis) and polyacrylamide disc gel (AlkPhor), and isoelectric focusing using polyacrylamide slab gel. The former two methods showed typical two bands (fast-alpha 2 and alpha 2 beta bands) and the latter one method revealed more basic bands of liver and bone, suggesting the extensive sialylation. The patient complained fever and diarrhea. Enterococcus faecium was detected from his stool. Etiologically, two more patients in the same ward showed TH in the same period. It suggested TH would be occurred by infectious states. Awareness of such benign forms of hyperphosphatasemia not related to malignancy will aid the physician in the differential diagnosis of elevated ALP activity.

Alkaline Phosphatase↗
PubMed1996

A case of malignant fibrous histiocytoma of the ilium, evaluation by blood-pool scintigraphy.

A rare case of malignant fibrous histiocytoma (MFH) of the ilium was presented and blood-pool scintigraphic images and angiographic images were correlated. Hypervascular tumor and lacking of contrast material with arteriovenous shunts were shown by angiography. Intensive tracer accumulation was shown by blood-pool scintigraphy. After radiation therapy and transcatheter arterial embolization of the tumor, tracer accumulation was noticeable reduced. These findings suggested a vascular tumor such as angiosarcoma, but surgery revealed MFH of the bone. Blood-pool scintigraphy was useful in the evaluation of the vascular characteristics of the tumor.

Aged↗
PubMed1996

99mTc-MIBI accumulation in the parathyroid autograft in a patient with recurrent hyperparathyroidism.

A case with recurrent hyperparathyroidism secondary to chronic renal insufficiency is reported. The patient had undergone total parathyroidectomy and autotransplantation of parathyroid tissue five years ago. Bone scintigraphy clearly demonstrated skeletal involvement of secondary hyperparathyroidism and 99mTc-methoxyisobutylisonitrile scintigraphy clearly demonstrated a hyperfunctioning parathyroid autograft.

Bone and Bones↗
PubMed1996

Effects of ultraviolet B irradiation on cell-cell interaction; implication of morphological changes and actin filaments in irradiated cells.

We studied the effects of ultraviolet B (UV-B) irradiation on cell-cell interactions using mouse lymphoma RMA cells and T cell hybridoma HTB-176.10. RMA cells act as stimulators by presenting H-2Kb surface antigens to HTB-176.10 cells, inducing IL-2 production in HTB-176.10 cells. Irradiating RMA cells with 1000J/m2 UV-B suppressed cell cluster formation between RMA and HTB-176.10 cells and reduced the level of IL-2 production in HTB-176.10 cells, although H-2Kb surface antigens of RMA cells were still expressed. Electron microscopic observations of irradiated RMA cells revealed that UV-B irradiation damaged cell structures, resulting in the disappearance of microvilli on the cell surface, destruction of mitochondria, vacuolation of cytoplasm and swelling of the perinuclear cisterna space. We found that these alterations were accompanied by polymerization of filamentous actin quantified by flow cytometry after NBD-phallacidin staining. Our results suggest that a target of UV-B-induced alterations is actin filaments, which support the cell morphology as the cytoskeleton, and that modification of filamentous actin inhibits interaction between RMA and HTB-176.10 cells. This underlying mechanism may account for the impaired interaction between antigen-presenting cells and T cells after transfusion with UV-B-irradiated allogeneic blood components.

Actins↗
PubMed1996

Evaluation of routine sonography for early detection of pancreatic cancer.

The diagnostic accuracy of routine abdominal sonography for the detection of pancreatic cancer was examined. During the one-year period of 1994, sonographic examination of the upper abdominal region was performed 12,761 times on a total of 9410 patients for the screening of abdominal disorders. In 655 cases (7%) part of the pancreas could not be observed. Based on the "Diagnostic criteria for pancreatic cancer" published by the Japanese Society of Ultrasound in Medicine, sonographic finding was evaluated to be positive for pancreatic tumor in a total of 411 cases. At the end of 1995, 51 patients were proven to have pancreatic cancer, and 45 of these cases were ductal adenocarcinoma. In 26 cases the tumor was surgically resected. Fifty cases were true sonographic positives and one was a false negative. The sensitivity, specificity, overall accuracy, and positive and negative predictive values of sonography for pancreatic cancer were 98.0%, 95.9%, 95.9%, 12.2% and 100.0%, respectively. Among the 50 true positive cases, the tumor diameter was less than 1 cm in four (8%). In conclusion, the diagnostic accuracy of sonography for the detection of pancreatic cancer is sufficiently high. Therefore, a detailed study aimed at mass screening for pancreatic cancer using sonography as the main modality seems warranted as a countermeasure for the rapid increase of pancreatic cancer in Japan.

Abdomen↗
PubMed1995

Single-channel analysis of two types of Na+ currents in rat dorsal root ganglia.

The properties of voltage-gated Na+ channels were studied in neurones isolated from rat dorsal root ganglia using the outside-out configuration of the patch-clamp technique. Two types of single-channel currents were identified from the difference in unit amplitudes. Neither type was evoked in the medium in which extracellular Na+ ions were replaced by an equimolar amount of tetramethylammonium ions. The two types of single-channel currents differed in their sensitivity to tetrodotoxin (TTX). The smaller channel current was insensitive to 1 microM TTX (referred to as TTX-I), while the larger channel current was blocked by 1 nM TTX (TTX-S). The unit amplitudes measured during a step depolarization to -30 mV (1.4 mM internal and 250 mM external Na+ concentrations) were 1.16 pA for TTX-S and 0.57 pA for TTX-I, respectively. The slope conductance measured at -30 mV was 16.3 pS for TTX-S and 8.5 pS for TTX-I. TTX-S could be activated by step depolarizations positive to -60 mV, while TTX-I could be activated at potentials positive to -40 mV. When the test pulse was preceded by a depolarizing prepulse, the prepulse positive to -50 mV preferentially inactivated TTX-S with a minimal effect on TTX-I. Activation and inactivation time courses of the averaged ensemble currents computed from TTX-S showed remarkable resemblances to the time courses of the macroscopic TTX-sensitive Na+ current. Similarly, the ensemble currents of TTX-I mimicked the macroscopic TTX-insensitive Na+ current. It was concluded that the two types of Na+ channels in rat dorsal root ganglia differ not only in their sensitivity to TTX, but also in their single-channel conductances.

Animals↗