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We analyzed 10 children diagnosed as Kawasaki disease between August 1, 2005 and November 11, 2006.Fifty percent of the patients were resistant to initial high-dose intravenous immunoglobulin（IVIG） treatment.All the patients resistant to IVIG were improved by the additional IVIG treatment. No patient had coronary artery aneurysma. The high rate of the registance might be caused by the deviation of the age or small size of the subject. We compared between resistance and responder : clinical course of illness, laboratory data and symptoms.The resistance visited to our hospital earlier, had high percentage of neutrophilic leukocyte and low serum sodium concentration. The resistance had high platelet count and tended to have low Hb and low serum albumin concentration. We investigated our patients using 4 prediction score which were previously reported. Positive predictive values were 50 to 60％. Negative predictive value was 100％ in Sato’s prediction score. It might be effective to predict the responder.

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[in Japanese]

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[in Japanese]

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[in Japanese]

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[in Japanese]

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[in Japanese]

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[in Japanese]

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[in Japanese]

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We report a female patient with massive vaginal bleeding. She was treated initially as endometrial cancer and ITP, then presented gradually with evident hemolysis and bone marrow failure. A small dose of danazol was effective for over 4 years. The diagnosis of PNH was made finally by a flow cytometric analysis for detection of PNH type cells in peripheral blood.PNH is a relatively rare disease, and belongs together with AA and MDS to bone marrow failure syndromes.They may present simultaneously , or one may evolve into another. Awareness of PNH is essential for differential diagnosis.

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A 79-year-old woman was admitted to our hospital for general malaise, appetite loss and fever. She was diagnosed with upper respiratory infection one month ago. After admission, high fever persisted but we couldn’t detect its cause. She started complaining of loss of consciousness, and finally drifted into shock. We suspected acute adrenal insufficiency and injected hydrocortisone after taking a blood sample for the measurement of serum cortisol and ACTH.　She improved immediately upon administration of hydrocortisone. Hormonal examinations indicated that she had isolated ACTH deficiency. Patients with isolated ACTH deficiency develop signs and symptoms, which are respectively not specific, and relatively few patients complain of sustained fever. Furthermore, acute adrenal insufficiency is a life-threatening emergency. It is important to suspect adrenal insufficiency in patients with general malaise, tiredness, appetite loss, nausea, and hyponatremia, all of which occurred in our case.　In conclusion, this is a rare case of isolated ACTH deficiency associated with acute adrenal insufficiency and fever.

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　We report a case of Gallstone ileus that was diagnosed preoperatively in a 87-year-old man admitted for abdominal pain and vomiting. Abdominal computed tomography（CT）showed a large stone in the small intestine and cholecystodeodenal fistula. The patient was diagnosed as having Gallstone ileus and surgery was perfomed.Intraoperatively, a stone measuring 30mm in a diameter was observed to be causing an impaction, the stone was successfully removed from the ileum. Because of severe adhesion, we followed this case without biliary surgery.With the development in preoperative care and the improvement of the diagnostic imaging, gallstone ileus can now be treated by one stage. But as patients with gallstone ileus are aged and preoperative conditions are poor, the indication and operative procedure to be performed should be determined carefully.

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　Background. Osteosarcoma as a primary uterine tumor is extremely rare ; to our knowledge, only 22 cases have been reported to date. We report here a case of osteosarcoma originating from the uterus, and review the previous reports.　Case. A 60-year-old woman, presented with lower abdominal pain. A diagnosis of uterine sarcoma was made, and the patient underwent total abdominal hysterectomy, bilateral salpingo-oophorectomy, and pelvic lymphadenectomy. There was no apparent extrauterine extension of the tumor, although the serosal surface of the posterior uterine wall was involved by the tumor. Histologic examination of the surgical specimen showed that the tumor consisted of anaplastic-appearing spindle neoplastic cells with irregular nuclei, focal chondroid areas, and osteoclast-like multinucleated cells. No epithelial component was found in any section of the tumor.Immunohistochemically, any other mesenchymal components were not identified. The final pathological diagnosis was pure type of osteosarcoma originating from the uterine corpus. Postoperatively, the patient received pelvic radiation therapy and chemotherapy, consisting of carboplatin and docetaxel. Despite treatment, the patient developed multiple intra-abdominal nodules with local recurrence of the tumor 6 months following surgery.　Conclusion. Neither epithelial nor any other mesenchymal components were identified by immunohistochemical evaluation. Histological features enabled to distinguish the pure type of heterologous sarcoma from carcinosarcoma and mixed sarcoma. There were no literature, including present case, which reported long term survives over five years of primary osteosarcoma originating from the uterine corpus.

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Waterproofing spray, which is often used in homes, can cause pulmonary disorders when a person either inhales the spray directly or is affected by the highly toxic spray components produced by thermochemical decomposition.　In this case, a 40+X-year-old man experienced dyspnea after using a waterproof spray in his house while the windows were kept closed. He was therefore admitted to our hospital. On the third hospital day, the dyspnea and cough symptoms worsened, and we diagnosed him to have chemical pneumonitis which had been caused by the inhalation of waterproofing spray. The pathophysiological mechanism of chemical pneumonitis is considered to be as follows ; Fluorine resin, which is used as a water repellent agent antagonizes the pulmonary surfactant, thereby causing alveolar collapse and sometimes inducing chemical pneumonitis. We herein report a case, in which the onset in this patient occurred a few days after the patient’s exposure to the waterproofing spray. He was treated by glucocorticoid and neutrophil elastase inhibitor, and received respiratory care by BiPAP（ bi-level positive airway pressure）. His symptoms improved after these treatments. We think that it is important to carefully observe the clinical course of respiratory symptoms in detail even when the initial symptoms of the patient are not severe.

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[in Japanese]

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[in Japanese]

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