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Biomedical subjects

Y Inamo

Publications and source records attributed to Y Inamo.

11 recordsLinked to original sources

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

Antinuclear antibody positivity in pediatric patients with autoimmune thyroid disease.

OBJECTIVE: The incidence of antinuclear antibody (ANA) positivity was investigated in pediatric patients with autoimmune thyroid diseases. METHODS: Subjects were 21 untreated patients with Graves' disease, 12 untreated patients with Hashimoto's disease, and 16 untreated patients with non-autoimmune thyroid disease, including one patient with Plummer's disease, 11 patients with simple goiter (probable), and 4 patients with cretinism. Patients with Graves' disease were treated with either propylthiouracil or methimazole. ANA was measured using HEp-2 cells. Anti-dsDNA antibody was measured using double stranded DNA derived from an Escherichia coli plasmid. RESULTS: The incidence of ANA positivity was significantly higher in patients with untreated Graves' disease (n = 21; age range 9-15 yrs) than in Hashimoto's thyroiditis (n = 12; age range 7-15 yrs) (p < 0.03, Fisher's exact probability test). However, the 2 diseases were not significantly different with respect to anti-dsDNA antibody positivity. Most of the ANA positive patients with Graves' disease required treatment for more than 2 years, unlike the 6 ANA negative patients (p < 0.002, Fisher's exact probability test). CONCLUSION: We conclude that ANA positivity may predict a poor response to antithyroid drugs in Graves' disease.

Adolescent

Increase of activated factor VIIA and haemostatic molecular markers in juvenile chronic arthritis.

Activated factor VIIa (FVIIa), von Willebrand factor antigen (vWF:Ag), D-dimer and thrombin-antithrombin III complex (TAT) were measured to monitor coagulation status in patients with juvenile chronic arthritis (JCA). Subjects included 14 patients with systemic JCA, 16 with pauciarticular JCA and 16 with polyarticular JCA without disseminated intravascular coagulopathy, thrombosis or liver dysfunction. All types of JCA showed an increase of FVIIa, D-dimer and TAT, indicating enhanced activation of coagulation. In systemic JCA only there was also characteristically an elevation of vWF:Ag. We conclude that all types of JCA constitute a state of subclinical hypercoagulopathy caused by tissue damage and that additionally systemic JCA involves a prothrombotic state associated with or precipitated by vasculitis.

Antifibrinolytic Agents

Findings on magnetic resonance imaging of the spine and femur in a case of McCune-Albright syndrome.

Polyostotic fibrous dysplasia, a major osseous change in McCune-Albright syndrome, is seen in the cranium, facial bones, bones of the extremities, and ribs, but rarely in the spine. Spinal X-rays revealed no abnormalities in an 8-year-old girl with this syndrome, but 99mTc-methylene diphosphonate bone scintigraphy disclosed high-density areas in the thoracic and lumbar vertebrae. Multiple well-circumscribed areas of low signal intensity were seen on T1-weighted magnetic resonance imaging (MRI) of the spine. Although MRI spine scans in this disease have never been reported, our findings in this case proved interesting for evaluating osseous lesions. MRI made it possible to differentiate between fibrous lesions (low signal intensity on T1- and T2-weighted MRI) and cartilaginous lesions (low signal intensity on T1-weighted MRI and high signal intensity on T2-weighted MRI).

Child

Host responses and neuroendocrinological changes in pyrexia in childhood.

Host responses and neuroendocrinological changes during pyrexia in childhood were studied. Serum IL-1 beta could not be detected in most subjects either during pyrexia or in afebrile periods. IL-1 beta was detected in two cases, increasing during pyrexia and decreasing during the afebrile period. Plasma ACTH increased during pyrexia, but this was not statistically significant. Serum cortisol during pyrexia increased to around twice the normal value, and many subjects showed a high level of arginine vasopressin. The subjects, who did not develop dehydration, showed a decrease in serum osmolality and serum sodium during the pyrexia period. Serum Fe and Zn decreased, but serum Cu increased during both periods. It could not be confirmed that IL-1 activates the hypothalamic-pituitary-adrenal axis during pyrexia. It is concluded that AVP is important in the control of fever and the maintenance of homeostasis of body fluid during pyrexia.

Acute Disease

Precocious puberty caused by an hCG-producing tumor of the septum pellucidum.

A 6 year 9 month old boy with rapidly progressing precocious puberty was immunohistochemically and histologically diagnosed as having an hCG-producing mixed tumor consisting of choriocarcinoma and teratoma in the septum pellucidum. His serum hCG was elevated, but the serum LH was low as determined by LH immunoradiometric assay (IRMA). He did not exhibit a characteristic endocrinological pattern, e.g., high basal levels of LH and failure to respond with high LH levels to the LH-RH stimulation test using the conventional LH RIA method.

Cerebral Ventricle Neoplasms

Serum content of zinc and vitamin C in severely handicapped children.

As fracture is known to occur more easily in severely handicapped children (SHC), bone mineral content, serum zinc level and serum vitamin C level were determined in 65 SHC. These parameters were found to be greatly reduced in all patients. The decreases in zinc and vitamin C in the serum appeared to be associated with that in bone mineral content. Supplements of zinc and vitamin C may be important to decrease the chance of fracture due to diminished bone mineral content in SHC.

Adolescent

Chronic, infantile, neurological, cutaneous and articular syndrome in Japan; two case reports.

We report two Japanese children with chronic, infantile, neurological, cutaneous and articular syndrome. Although the shoulder joint lesion has been rare in the literature of Europe and America, our first case showed overgrowth of the epiphysis in the bilateral shoulder joints. The second case exhibited severe growth retardation caused by early closure of the physis. The influence of GH is irrelevant in this syndrome. The existence of this syndrome in the Orient should be recognized.

Abnormalities, Multiple