Syndrome of the sea-blue histiocyte--the first case report in Japan and review of the literature--.

A case of the syndrome of sea-blue histiocyte is presented in a 53-year-old Japanese woman, which is the first recorded case in Japan. The patient had hepatosplenomegaly, bleeding manifestations, mild thrombocytopenia, fatty metamorphosis and cirrhosis of the liver, as well as abnormal serum lipid profiles. Her parents were consanguineous and her maternal grandmother with hepatomegaly died of hepatic failure. Histologically, peculiar histiocytes containing numerous, intracytoplasmic sea-blue stained granules on May-Giemsa stain were demonstrated in biopsy materials of the bone marrow, lymph node and liver. The sea-blue granules in these histiocytes proved to have histochemical staining characteristics of lipogenic ceroid-like pigment. Ultrastructurally, these granules showed membrane-bound, pleomorphic inclusions of heterogeneous nature, including electron-dense amorphous or variegatedly osmiophilic, frequently laminated materials. Enzyme cytochemically, localization of acid phosphatase activity was demonstrated in and around the intracytoplasmic inclusions. With regard to the pathogenesis of the sea-blue histiocytes in this case, it may be suggested that the existence of the abnormality in lipid metabolism plays an important role in intralysosomal ceroidogenesis in these histiocytes.     

CEROID-LIKE HISTIOCYTIC GRANULOMA OF GALL-BLADDER — A PREVIOUSLY UNDESCRIBED LESION —

In the present study, 13 cases of a peculiar gall-bladder granuloma characterized by marked proliferation of ceroid-fllled brown histiocytes were pathomorphologically, histochemically and ultrastructurally examined to define the pathologic features of such a lesion previously undescribed in the literature. The lesion grossly showed a granulomatous appearance of yellow brown to dark brown color developing in the wall of gall-bladder. Histologically, there was proliferation of histiocytes containing abundant brown pigment granules In their cytoplasm. The pigment granules proved to have staining characteristics closely resembling those of lipogenic ceroid-like pigment. Ultrastructurally, these granules showed membrane-bound, pleomorphic osmiophilic inclusions of heterogenous materials. With regard to the pathogenesis of this granuloma, it may be suggested that lipid components of bile juice, particularly unsaturated fatty acids and phospholipids, play an Important role as a source of ceroidogenesis In the proliferating histiocytes.     

SYNDROME OF THE SEA-BLUE HISTIOCYTE —The First Case Report in Japan and Review of the Literature—

A case of the syndrome of sea-blue histiocyte is presented in a 53-year-old Japanese woman, which is the first recorded case in Japan. The patient had hepatosplenomegaly, bleeding manifestations, mild thrombocytopenia, fatty metamorphosis and cirrhosis of the liver, as well as abnormal serum lipid profiles. Her parents were consanguineous and her maternal grandmother with hepatomegaly died of hepatic failure. Histologically, peculiar histiocytes containing numerous, intracytoplasmic sea-blue stained granules on May-Giemsa stain were demonstrated in biopsy materials of the bone marrow, lymph node and liver. The sea-blue granules in these histiocytes proved to have histochemical staining characteristics of lipogenic ceroid-like pigment. Ultrastructurally, these granules showed membrane-bound, pleomorphic inclusions of heterogeneous nature, including electron-dense amorphous or variegatedly osmiophilic, frequently laminated materials. Enzyme cyto-chemically, localization of acid phosphatase activity was demonstrated in and around the intracytoplasmic inclusions. With regard to the pathogenesis of the sea-blue histiocytes in this case, it may be suggested that the existence of the abnormality in lipid metabolism plays an imporant role in intralysosomal ceroidogenesis in these histiocytes.     

Immunohistochemical profile of primary sclerosing Iipogranuloma of the scrotum: Report of five cases

Five cases of primary sclerosing scrotal lipogranuloma were examined histologically and immunohistochemically. Every case lacked a history of injection or trauma, and revealed Common histologicat features; a typical granuloma composed of epithelioid cells and multinucleated giant cells, and inflammatory infiltrates of eosinophils, lymphocytes and macrophageimonocytes in the interstitium. lmmunahistochemistry disclosed the epithelioid cells and multinuclaated giant cells of the granuloma to be monocytetr in nature, as bath types of cells were positive for lyso-yme, α-1-antltrypin, α-1-antichymotrypsin, and KP-1. In the interstitium, KP-1 positive monocytes, L-26 positive B lymphocytes, UCHL-1 positive T lymphocytes and 5–100 protein positive Langerhans-like cells were frequently found. 5100 protein positive cells could not be detected in the granuloma. Primary sclerosing lipogranuloma of the scrotum, therefore, is a peculiar inflammation characterized by granulomas consisting of monocytes and marked tissue eosinophilia of unknown etiology.     

Risky drug practices and treatment needs of youth detained in New South Wales Juvenile Justice Centres

Demographic, drug use and criminal correlates of risky drug practices were examined among 279 detained youth in NSW Juvenile Justice Centres. Binge drinking was common, with 63% reporting the consumption of at least five drinks in a row in the 2 weeks prior to incarceration. It was associated with being older, being from an English-speaking background, regular tobacco consumption and having a current drug problem. A quarter (24%) of the sample had injected an illicit drug, over one-third (37%) of whom also reported sharing needles. Injectors were more likely to be polydrug users, regular amphetamine users and have a past history of drug treatment. While 16% of the sample indicated a need for drug treatment, only 10% were currently receiving some form of treatment, most commonly counselling. Appropriate strategies are needed among this population to address gaps in treatment provision.     

Route of administration and adverse effects of amphetamine use among young adults in Syndney, Australia

The relationship between route of administration and the adverse effects of amphetamine use (dependence symptoms, treatment-seeking, adverse psychological symptoms and violence) were examined among 231 Australian amphetamine users, half of whom usually injected amphetamine. Although 87% of users were recruited from non-treatment sources, a third had experienced symptoms of dependence on amphetamine, and a third had experienced an amphetamine-related health problem for which one in four had sought medical attention. There was also a high prevalence of psychological symptoms, such as depression, anxiety, paranoia, hallucinations and violence which were related to frequency of amphetamine use, and use by injection. Amphetamine users need to be better informed about the potential adverse effects of amphetamine use, and about ways in which they can reduce their risks of experiencing these harms by avoiding injecting use, and the regular use of high doses of amphetamines.     

Nasal teratocarcinosarcoma - a case report

A 79-year-old man noticed nasal obstruction and bleeding caused by a pedunculated tumor in the left nasal cavity. The tumor revealed a malignant neoplasm predominantly composed of immature neuroepithelial cells with rosettes and palisading patterns, rhabdomyoblastic cells with positivity for desmin and cross-striations, and immature mucin-producing glandular cells. Small amounts of mature chondroid tissue and neurofibrillary matrix were also present. Therefore, the tumor was diagnosed as nasal teratocarcinosarcoma.     

The effect of niacinamide on reducing cutaneous pigmentation and suppression of melanosome transfer

BACKGROUND: Cutaneous hyperpigmentation occurs in multiple conditions. In addition, many Asian women desire a lighter skin colour. Thus, there is a need for the development of skin lightening agents. Niacinamide is a possible candidate. OBJECTIVES: To investigate the effects of niacinamide on melanogenesis in vitro and on facial hyperpigmentation and skin colour in vivo in Japanese women. METHODS: Melanin production was measured in a purified mushroom tyrosinase assay, cultured melanocytes, a keratinocyte/melanocyte coculture model, and a pigmented reconstructed epidermis (PREP) model. The clinical trials included 18 subjects with hyperpigmentation who used 5% niacinamide moisturizer and vehicle moisturizer in a paired design, and 120 subjects with facial tanning who were assigned to two of three treatments: vehicle, sunscreen and 2% niacinamide + sunscreen. Changes in facial hyperpigmentation and skin colour were objectively quantified by computer analysis and visual grading of high-resolution digital images of the face. RESULTS: Niacinamide had no effect on the catalytic activity of mushroom tyrosinase or on melanogenesis in cultured melanocytes. However, niacinamide gave 35-68% inhibition of melanosome transfer in the coculture model and reduced cutaneous pigmentation in the PREP model. In the clinical studies, niacinamide significantly decreased hyperpigmentation and increased skin lightness compared with vehicle alone after 4 weeks of use. CONCLUSIONS: The data suggest niacinamide is an effective skin lightening compound that works by inhibiting melanosome transfer from melanocytes to keratinocytes.     

Renal artery stenosis in a patient with Leriche syndrome: brachial artery access for stent placement

We encountered a patient with Leriche syndrome and general atherosclerotic disease. His renal function had deteriorated, and diabetic nephropathy was suspected. Severe left renal artery stenosis was also found and considered, if untreated, to be an important factor in aggravation of his renal function. Because the infrarenal abdominal aorta was completely occluded, we treated the patient by insertion of a stent into the left renal artery from the brachial approach. The operation was successful, without residual stenosis or complications. Renal blood flow was remarkably improved after stent placement.