A case of multiple intrahepatic portosystemic venous shunts associated with multiple hemangioma-like lesions of the liver

Intrahepatic portosystemic venous shunts (IPVS) are rare, except for the minute ones observed in cirrhotic liver. This report concerns a case with frequent episodes of consciousness loss, diagnosed as IPVS and multiple hemangioma-like lesions of the liver by US, CT and angiography. To our knowledge, this is the first report of association of IPVS and such angiographically evident hemangioma-like lesions of the liver. The etiology of IPVS is mostly considered to be congenital though, there are still many arguments about it. The relation between IPVS and the hemangioma-like lesions of the liver is discussed.     

Electron microprobe analysis of human labial gland secretory granules in cystic fibrosis.

X-ray microanalysis of freeze-dried labial gland cryosections revealed that Na concentration was doubled and the Ca/S concentration ratio was decreased in secretory granules of labial glands from patients with cystic fibrosis (CF) when compared with glands from normal subjects. Other results suggested that the decrease in the Ca/S concentration ratio resulted from an increase in S concentration. These findings imply that mucous granules in labial saliva showed a CF-related increase in Na and S content, and such changes would be expected to affect the rheology of the mucus after exocytosis. In contrast with a previous study in human parotid glands, no evidence was found for CF-related changes in cytoplasmic or nuclear Na, K, and Ca concentrations. Significant elemental differences were found between secretory granules and nuclei and cytoplasm of control cells.     

Flow cytometric study on the action of progestogen in patients with adenocarcinoma and precancerous lesions of the endometrium

Synthetic progestogens have been used in 21 patients with endometrial adenocarcinoma and 6 patients with precancerous lesions. During a program of management with medroxyprogesterone acetate (MPA) in an attempt to identify the mechanism by which a remission rate in 30-40% occurred, adenocarcinoma cells were analysed. Histopathologic reactions of endometrial adenocarcinoma treated with MPA 400mg/day through 7 days have been found similar those of normal secretory endometrium such as subnuclear vacuolation, loss of mitotic figure, diminished nuclear size and stromal decidual reaction and regressive changes are highly dose dependent. In detailed analysis of the cell cycle, computerized flow cytometry was employed to quantitate total DNA content following propidium iodide fluorescent staining after RNase treatment. Possible modes of action of MPA on endometrial adenocarcinoma are an increase in the G0+1 fraction corresponding to ploidy reduction. Adenomatous hyperplastic lesions were able to be reversed completely in all patients compared with adenocarcinoma in sixty-six percent.     

Aggregates Formed During Storage of β-Galactosidase in Solution and in the Freeze-Dried State

Aggregates formed during storage of freeze-dried -galactosidase were compared with those formed in solutions. Freeze-dried -galactosidase aggregated during storage in the presence of moisture, producing a protein precipitate which was soluble in guanidine hydrochloride solution but not in buffer solution. The protein precipitate dissolved in guanidine solution exhibited a large molecular size by high-performance size exclusion chromatography and converted to proteins of original size in the presence of dithiothreitol. It is suggested that the aggregation involves chemical interaction via covalent disulfide bonding. In contrast, -galactosidase in aqueous solution aggregated without formation of protein precipitates. Soluble aggregates were converted to proteins of original size in guanidine solution without dithiothreitol, suggesting noncovalent bonding. The difference in aggregation behavior may be ascribed to the difference in the water:protein ratio. We propose that inactivation of -galactosidase is due to formation of thermally denatured (unfolded) protein, which aggregates dependent on the water:protein ratio, either via noncovalent interactions at a high water:protein ratio in solution or via covalent interaction at a low water:protein ratio in the freeze-dried state.     

(Na + + K + )-ATPase activity in the dog submandibular gland

Microsomes prepared from dog submandibular glands contain a (Na⁺ + K⁺)-activated Mg²⁺-dependent ATPase (ATP phosphohydrolase, EC 3.6.1.3) with a relatively high specific activity (105 μmoles P_i × mg protein⁻¹ × hr⁻¹). In the range tested, maximal activation of the enzyme was attained at Na⁺ and K⁺ concentrations of 200 and 10 mM respectively in the presence of 5 mM Mg²⁺, Rb⁺. Cs⁺, NH₄⁺, Li⁺, and choline could not substitute for Na⁺, but could partially substitute for K⁺ ions. Chloride was not essential as an anion, but could be substituted for by nitrate, bromide, iodide or sulphate without loss of activity. The pH optimum for the enzyme was 7.2 and the concentration of ouabain for half-maximal inhibition of enzyme activity was 2 × 10⁻⁷ M.     

The quality of life, mental health, and perceived stigma of leprosy patients in Bangladesh

The present study aims to determine the quality of life (QOL) and general mental health of leprosy patients compared with the general population, and evaluate contributing factors such as socio-economic characteristics and perceived stigma. A total of 189 patients (160 outpatients, 29 inpatients) and 200 controls without leprosy or other chronic diseases were selected from Dhaka district, Bangladesh, using stratified random sampling. A Bangladeshi version of a structured questionnaire including socio-demographic characteristics-the Bangla version of the World Health Organization Quality of Life Assessment BREF (WHOQOL-BREF)-was used to assess QOL; a Self-Reporting Questionnaire (SRQ) was used to evaluate general mental health; the Barthel Index to control activities of daily living (ADL); and the authors' Perceived Stigma Questionnaire was used to assess perceived stigma of patients with leprosy. Medical records were examined to evaluate disability grades and impairment. QOL and general mental health scores of leprosy patients were worse than those of the general population. Multiple regression analysis revealed that factors potentially contributing to the deteriorated QOL of leprosy patients were the presence of perceived stigma, fewer years of education, the presence of deformities, and a lower annual income. Perceived stigma showed the greatest association with adverse QOL. We conclude that there is an urgent need for interventions sensitive to the effects of perceived stigma, gender, and medical conditions to improve the QOL and mental health of Bangladeshi leprosy patients.     

Cardiac complications after haploidentical HLA-mismatched hematopoietic stem cell transplantation using in vivo alemtuzumab

Alemtuzumab is a humanized monoclonal antibody directed against human CD52 with a strong lympholytic effect. We have performed unmanipulated hematopoietic stem cell transplantation (HSCT) from 2- or 3-locus-mismatched family donors in 14 patients using in vivo alemtuzumab. All achieved complete donor cell engraftment and grade III-IV acute graft-versus-host disease was observed in only one patient. However, eight of the 14 patients developed grade II-IV cardiac complications according to Bearman's criteria. Next, we retrospectively analyzed the records of 142 adult patients who underwent allogeneic HSCT from 1995 to 2004 to evaluate whether the use of alemtuzumab was an independent risk factor for cardiac complications. Among several factors that increased the incidence of grade II-IV cardiac complications with at least borderline significance, a multivariate analysis identified the cumulative dose of anthracyclines (P=0.0016) and the use of alemtuzumab (P=0.0001) as independent significant risk factors. All of the cardiac complications in the alemtuzumab group were successfully treated with diuretics and/or catecholamines. Patient selection and close monitoring of cardiac function may be important in HLA-mismatched HSCT using in vivo alemtuzumab.     

Unrelated bone marrow transplantation for non-Hodgkin lymphoma: a study from the Japan Marrow Donor Program

There is little information available regarding the outcome of unrelated bone marrow transplantation (BMT) for non-Hodgkin lymphoma (NHL). Therefore, we retrospectively analyzed the data of 124 patients who underwent unrelated BMT through the Japan Marrow Donor Program (JMDP) between July 1992 and August 2001. The overall survival (OS), progression-free survival (PFS), cumulative incidences of disease progression, and nonprogression mortality at 3 years after BMT were 49.7%, 42.6%, 24.5%, and 32.9%, respectively, with a median follow-up duration of 565 days among survivors. The incidence of grades II-IV acute graft-versus-host disease (GVHD) was 40.9%. Recipient age, previous history of autologous transplantation, and chemosensitivity at transplantation were independent prognostic factors for OS and PFS. The development of grades II-IV acute GVHD was associated with lower incidence of disease progression after transplantation, which suggested the existence of a graft versus lymphoma effect. Unrelated BMT should be considered as a treatment option for patients with high-risk NHL without an HLA-matched related donor.     

Primary pancreatic low-grade mucosa-associated lymphoid tissue lymphoma presenting with multiple masses

The case concerns a 55-year-old Japanese man with multiple pancreatic tumors that were incidentally detected by ultrasonography (US) in a routine medical examination. He consulted a doctor since either neuroendocrine tumors or metastases of small-cell lung carcinoma were suspected, because he had an abnormal shadow in the lung. Then he was transferred to our hospital. Imaging studies showed multiple pancreatic tumors of 3.0-cm diameter in the head, 1.0 cm in the body and 1.5 cm in the tail, respectively. Specimen by endoscopic ultrasonography fine-needle aspiration cytology (EUS–FNAC) revealed numerous B-cell lymphocytes, but a definite diagnosis of malignant lymphoma could not be obtained. Therefore, a segmental pancreatic body resection was performed to clarify the features of the tumor for appropriate therapy. Consequently, the final diagnosis was obtained as a low-grade B-cell lymphoma having a lymphoepithelial lesion, which indicated mucosa-associated lymphoid tissue (MALT) lymphoma. Radiation therapy with 31 Gy successfully resulted in complete remission. We report here for the first time on primary pancreatic low-grade MALT lymphoma presenting with multiple pancreatic masses.     

A case of primary adenosquamous/squamous cell carcinoma of gallbladder directly invaded duodenum

A rare case of primary gallbladder carcinoma is reported. A 67 year-old woman was admitted to our hospital for treatment of suspected duodenal carcinoma. A series of radiographic examinations demonstrated a giant tumor involving the duodenum, gallbladder, pancreatic head, and transverse colon. These extensions made it difficult to identify the primary origin of the carcinoma. Pancreatoduodenectomy, cholecystectomy, and resection of the transverse colon were performed. Macroscopically, ulcerative lesions were seen in both the gallbladder and the duodenum. Microscopic examination revealed adenosquamous cell carcinoma of the gallbladder, invasive of the adjacent organs, including circumferential invasion of the second portion of the duodenum. The patient tolerated the operation well and was discharged 28 days post-operatively, but died of liver metastasis 4 months after surgery. Local invasion of the surrounding tissues is characteristic of adenosquamous/squamous cell carcinoma of the gallbladder. Although surgery for cure is deemed possible, the rapid growth rate of this type of tumor may cast doubt on the value of extensive radical surgery.