Double-barreled wet colostomy

An easy and simple method for constructing a urinary diversion in patients undergoing pelvic exenteration for advanced rectal cancer is described. This procedure features double-barreled colostomy with a segment of the sigmoid colon 8 to 10 cm distal to the stoma as a urinary conduit.Read at the meeting of the Japanese Research Society for Cancer of the Colon and Rectum, Tokyo, Japan, July 29, 1994.     

B cell hyperactivity and its relation to distinct clinical features and the degree of disease activity in patients with systemic lupus erythematosus

Peripheral blood B cells that were actively proliferating, those actively secreting immunoglobulin, and those expressing an early activation marker, Ba antigens, on the surfaces were quantitated in 25 patients with systemic lupus erythematosus (SLE). B cell hyperactivity was found in almost all of the SLE patients, as demonstrated by any one of these measures of B cell activity. Moreover, we observed a strong positive correlation between the degree of disease activity and the amount of spontaneous incorporation of 3H-thymidine by B cells; the magnitude of the increase in frequency of spontaneous Ig-secreting cells in peripheral blood correlated strikingly with certain clinical features in these patients. Our findings suggest that there is heterogeneity of B cell hyperactivity in individual patients with SLE and, thus, that clinical subsets of SLE can be identified on the basis of B cell hyperactivity.     

Orthostatic hypertension with nephroptosis and aortitis disease

A 53-year-old woman with nephroptosis and aortitis disease was found also to have orthostatic hypertension. When standing, she had high renin levels and normal catecholamine values, with a reduced baroreflex sensitivity. This orthostatic hypertension largely may be due to an activation of the renin system caused by nephroptosis and partly due to a reduced baroreflex sensitivity caused by aortitis. Captopril and propranolol hydrochloride were effective for the treatment of hypertension.     

Anterior pituitary function in patients with cerebrovascular disease: with special reference to growth hormone secretion in response to TRH administration (author's transl)]

HGH secretion in response to TRH was studied in patients with cerebrovascular disease in order to elucidate an influence of cerebrovascular lesions on the hypothalamus-anterior pituitary function. Blood specimens were obtained before and at the time of 10, 20, 30, 40, 60, 90 and 120 minutes after the intraveneous administration of 200 microgram of TRH in 17 patients with cerebral hemorrhage and in 8 patients with cerebral infarction. With regards to the natural fluctuations of serum HGH caused by cerebrovascular disease, the blood specimens were obrained at the time of 0, 30, 60, 90 and 120 minutes without the administration of TRH in 4 patients with cerebral hemorrhage and in 4 patients with cerebral infarction. Serum HGH was measured using the RIA method and the following results were obtained: 1) An increase in serum HGH was observed in 1 patient with cerebral hemorrhage out of 8 patients with cerebrovascular disease without TRH administration. 2) In 5 out of 17 patients with cerebral hemorrhage, an increase in serum HGH was observed following TRH administration. Three out of these 5 patients were included in the group of non-coma, and 4 out of the 5 patients were female. 3) In 2 out of 8 patients with cerebral infarction, an increase in serum HGH was observed following TRH administration.     

Genetic Analysis of Non-Hydrogen Sulfide-Producing Salmonella enterica Serovar Typhimurium and S. enterica Serovar Infantis Isolates in Japan

Whole-genome sequencing of non-H₂S-producing Salmonella enterica serovar Typhimurium and S. enterica serovar Infantis isolates from poultry meat revealed a nonsense mutation in the phsA thiosulfate reductase gene and carriage of a CMY-2 β-lactamase. The lack of production of H₂S might lead to the incorrect identification of S. enterica isolates carrying antimicrobial resistance genes.     

In Vitro Analysis of the Functional Effects of an NLRP3 G809S Variant with the co-Existence of MEFV Haplotype Variants in Atypical Autoinflammatory Syndrome

Purpose

Hereditary periodic fever syndromes have been considered monogenic diseases. However, some recent reports have described patients with co-existence of recurrent fever responsible genes. This study assessed whether a rare variant, found in Japanese children showing atypical autoinflammatory syndrome, located in the leucine-rich repeat domain of Nod-like receptor family, pyrin domain containing 3 (NLRP3) with co-existence of Mediterranean fever (MEFV) haplotype variants may contribute to a proinflammatory phenotype using a systematic approach.

Methods

Cytokine production in serum or from peripheral blood monocytes was measured by ELISA. DNA sequence analysis of genes including NLRP3, MEFV, mevalonate kinase (MVK), and tumor necrosis factor receptor superfamily, member 1A (TNFRSF1A) were performed on patient samples. In vitro functional assays determined the effects of the NLRP3 variants and pyrin using NF-κB activation and speck formation assays.

Results

A heterozygous genetic variant of NLRP3, G809S, was found in samples from both patients. Additionally the previously reported heterozygous MEFV variants (P369S-R408Q or E148Q-P369S-R408Q) were also detected in both patients. Serum IL-1ra and sTNFR1 levels increased in the attack phase of the disease in both patients. The production levels of IL-1β from monocytes isolated from both cases were elevated following LPS and IFN-γ stimulation. The NLRP3 G809S variant demonstrated no increase of NF-κB activity following monosodium urate stimulation, whereas it significantly increased speck formation by interacting with apoptosis-associated speck-like protein with caspase recruitment domain.

Conclusions

The phenotype of atypical autoinflammatory disease in patients could be modified by a synergistic effect with two other variants of autoinflammatory-associated genes.