Human heme oxygenase-1 deficiency: A lesson on serendipity in the discovery of the novel disease

The first case of human heme oxygenase (HO)-1 deficiency was reported by Yachie et al. at our laboratory in the Department of Pediatrics, Angiogenesis and Vascular Development, Kanazawa University Graduate School of Medical Science, in 1999. In the present paper I would like to review this novel disease. Our studies into HO-1 deficiency were called by us 'Kanazawa version Project X'. From the story of our successful discovery we have learned that serendipity is a very important spiritual factor. Serendipity is the making of fortunate and unexpected discoveries by chance (from its possession by the heroes in the Persian fairy tale The Three Princes of Serendip).     

A case of adenoid cystic carcinoma of the breast

A 57-year-old woman was referred to our institution 4 months after she noticed a palpable, painless mass in her left breast. Physical examination revealed a mobile and elastic mass. An axillary or subclavicular lymph node was not palpable. Mammography revealed a lobulated mass with a partially ill-defined border. Ultrasonography depicted a 2.5 × 1.5 cm irregularly shaped mass with heterogeneous internal echo and posterior acoustic enhancement. The border of the mass was poorly defined at the anterior and lateral aspects of the mass. Results of ultrasonography-guided fine-needle aspiration cytology were strongly suggestive of a malignant tumor. Thus, the patient underwent breast-conserving surgery with axillary lymph node dissection. The gross resected specimen revealed a gray-to-white and well-demarcated solid tumor measuring 3 × 2 × 2.5 cm, with an irregular border and heterogeneous internal structure. Histological examination showed the characteristic patterns of adenoid cystic carcinoma of the breast. Immunohistochemical studies for both estrogen receptors (ER) and progesterone receptors (PgR) were negative. The patient remains well and has no clinical recurrence of the disease after 5 years of follow-up without radiotherapy or adjuvant therapy.     

High prevalence of sensitization to cat allergen among Japanese children with asthma, living without cats

BACKGROUND: Cat allergy is common among children with asthma. Many cat-allergic patients in Japan and elsewhere do not keep cats, but nonetheless become sensitized through environmental exposure to cat allergen. OBJECTIVE: To assess the frequency of cat allergy and cat-specific immunoglobulin E (IgE) and immunoglobulin G (IgG) antibody responses in young Japanese patients with asthma in relation to self-reported cat exposure and Fel d 1 levels in dust samples. METHODS: Cat dander-specific IgE antibody was measured in sera from asthma patients using the CAP system. IgE and IgG antibody to Fel d 1 was measured by antigen binding radioimmunoassay and by chimeric enzyme immunoassay. Fel d 1 levels in dust samples from a subset of patients' homes were measured by monoclonal antibody-based enzyme immunoassay. RESULTS: Cat-specific IgE (CAP class>/=2) was found in sera from 70% of 44 patients who kept cats and 34% of 394 patients who had never kept cats. The prevalence of sensitization increased progressively to age 6 years (40%: positive), and then increased gradually to age 16 years (approximately 60%: positive) in patients who had never kept cats. There was an excellent correlation between cat CAP values and IgE levels to Fel d 1. The absolute amount of IgE antibody to Fel d 1 ranged from 0.01 to 15.6% of total IgE. Most patients who did not keep cats were exposed to Fel d 1 levels ranging from 0.07-8 microg/g dust. CONCLUSIONS: Sensitization to cat allergen is common among young asthmatic patients in Japan, even among patients who do not keep cats. Use of CAP and the chimeric enzyme-linked immunosorbent assay allows accurate diagnosis of cat allergy and quantification of specific IgE antibody levels.     

Complete response of a recurrent advanced urachal carcinoma treated by S-1/cisplatin combination chemotherapy

We report a case of recurrent advanced urachal carcinoma with right internal iliac node and left lung metastases in a 34-year-old man. After receiving partial cystectomy including en bloc resection of the urachus and with bilateral pelvic lymph node dissection, he was treated with five courses of S-1 and cisplatin combination chemotherapy. He remains free from the disease after a 30-month follow-up period. S-1/cisplatin combination chemotherapy is suggested to be a potent tool for controlling advanced urachal carcinoma.     

MASSIVE PERICARDIAL EFFUSION IN SCLERODERMA: A REVIEW OF FIVE CASES

Medical records of five patients with sclerodenna (SSc), eachof whom had pericardial effusion with an estimated volume ofmore than 200 ml, were reviewed to study the clinical and immunologicalsignificance of massive pericardial effusion in SSc. DiffuseSSc (4/5), with a wide area of pigmentation (4/5), flexion contracture(4/5), oesophageal hypomotility (5/5), pulmonary fibrosis (4/5)and autoantibodies to topoisomerase I (3/5) were the commonfeatures in this group. High protein, lactate dehydrogenaseand low white blood cell count were the characteristics of pericardialfluid. None of the patients had signs of acute pericarditis.Four of the five cases died within 9 months of the diagnosisof pericarditis; two with renal failure, one with cardiac tamponadeand another with sudden death. The pericarditis in diffuse SSc,especially in cases with anti-topoisomerase I, may be characterizedby a chronic form of pericarditis with poor prognosis, oftencomplicated by ,renal failure. KEY WORDS: Scleroderma, Pericardial effusion, Antitopoisomerase I antibodies, Antinuclear antibodies, Renal failure     

Effects of arachidonic acid metabolites and interleukin-1 on platelet activating factor production by hepatic sinusoidal endothelial cells from mice

The effects of interleukin-1 (IL-1) and arachidonic acid metabolites, prostaglandin (PG) E1, leukotriene (LT) B4 and LTC4, on the amount of platelet activating factor (PAF) produced and released by mouse hepatic sinusoidal endothelial cells were investigated. When hepatic sinusoidal endothelial cells were incubated with 1 microgram/mL of calcium ionophore (CaI) A23187, PAF production increased until 20 min after the start of incubation, but when the cells were incubated with PGE1 and CaI A23187, PAF production was significantly suppressed. On the other hand, when hepatic sinusoidal endothelial cells were incubated with IL-1 beta, LTB4 or LTC4 alone, PAF production significantly increased compared with that of the cells incubated without these substances. However, when the cells were incubated with PGE1 alone or with IL-1 beta, LTB4 or LTC4 and CaI A23187, these effects were not observed. These results indicated that PAF produced by hepatic sinusoidal endothelial cells is affected by IL-1 and arachidonic acid metabolites, suggesting that immune and inflammatory reactions in these cells may be regulated by chemical mediators produced by the cells themselves.