A girl having congenital chloride diarrhea treated with spironolactone for seven years

We report on a girl having congenital chloride diarrhea (CCD) who has been followed for 7 years and 6 months sequentially. Dilated intestinal loops, marked enlargement of the abdominal circumference of the fetus and hydramnios were noted by ultrasound examination at 31 weeks of gestation. After delivery by cesarean section for hydramnios, she excreted profuse watery yellow green stools with marked abdominal distension. At 4 months of age, hypochloremia, hyponatremia and a high concentration of chloride in the stool were identified. She was diagnosed as having CCD. Because it was difficult to administer a large volume of potassium chloride (KCl), and sodium chloride (NaCl), we decided to administer spironolactone. After administration of spironolactone, we could generate correct serum electrolytes using less amounts of KCl. At 7 years and 6 months of age, her body size was within normal limits and her intellectual, mental and physical development had been normal. In spite of normal serum electrolytes, blood pH and the presence of chloriduria, secondary hyperaldosteronism was noted. We consider that spironolactone may be useful to decrease the amount of KCl administration in the neonatal period, but frequent measurements of renin, angiotensin and aldosterone would be necessary for adequate control in CCD cases.     

Endoscopic Hemostasis in Hemorrhagic Gastric Ulcer—Effectiveness of the Hemoclipping,Ethanol Injections and Heat Probe—

Abstract: The hemostatic effects of hemoclipping, the pure ethanol local injection method and the heat probe methods on hemorrhagic gastric ulcers associated with exposed blood vessels, were compared. The locations of the ulcers and exposed blood vessles, as well as characteristics and the severity of the hemorrhage were discussed. The hemoclipping method was used on 59 patients, the pure ethanol local injection on 32 patients, and the heat probe methods on 26 patients. The hemoclipping and pure ethanol local injection methods were effective in all of the patients in whom the exposed blood vessels were present around the margin of ulcers. The efficacy rate was 91%, 92% and 92%, respectively, when exposed blood vessels were found at the base of the ulcer. The efficacy rate was 94%, 100% and 80% for the three hemostatic methods, respectively, when an oozing hemorrhage was present. The three methods had an efficacy rate of 100% when only blood clots were present. The efficacy rate for a spurting hemorrhage was 86%, 71% and 67%, respectively, for the three methods. The hemoclipping and pure ethanol local injection were effective in 100% of slight and moderate hemorrhage cases, while the heat probe method was effective in only 89% of moderate cases. The efficacy rate was 85%, 85% and 83%, respectively, for severe cases. Overall, the efficacy rate was 93% for the hemoclipping method, 94% for the pure ethanol local injection and 92% for the heat probe method, and there was no significant difference between the three methods with regard to the overall efficacy rate.     

Prognostic Factors and Therapeutic Results in Multiple Myeloma

Prognostic factors in 68 consecutive patients with myeloma treatedat the National Cancer Center Hospital from 1962 to 1984 wereanalyzed. Median survival time from onset was 100 months forstage I, 72 months for stage II, and 26 months for stage IIIof the Durie and Salmon's clinical staging system. It was 55months in patients with normal renal function and 18 monthsin those with abnormal renal function. All early deaths occurredin patients with stage III disease. Hemoglobin level, bone lyticlegions and presence of Bence Jones protein were also significantprognostic factors. On the other hand, heavy chain as well aslight chain subtypes of monoclonal immunoglobulin (M-component)and M-component production rate did not influence the survivalof myeloma patients. The analysis of chemotherapeutic responsesand survival curves according to the chemotherapy used in thisstudy (alkylating agent vs Vinca-alkaloid plus alkylating agent)did not disclose any significant difference between the twogroups. The overall response rate was 67%. The survival timefrom the initial chemotherapy of responding patients was significantlylonger than that of nonresponders.     

Pemphigoid vegetans

A Japanese case of pemphigoid vegetans is described. The clinical, histopathological and immunological features were similar to the previously reported cases. The patient also developed vesicular lesions. Indirect immunoelectronmicroscopy revealed that the autoantibody in this patient's serum reacted with basal cell hemidesmosomes. This study provides further evidence that pemphigoid vegetans is a subtype of bullous pemphigoid.     

Mature helper T cell requirement for immunoglobulin production by neonatal naive B cells injected intraperitoneally into severe combined immunodeficient (SCID) mice

It is accepted that human neonatal naive B cells produce mainly IgM in vivo as well as in vitro. Our previous work has demonstrated that i.p. injection of neonatal B cells together with adult mature T cells induces substantial levels of human IgG in the serum of SCID recipient mice. The present study was further attempted to determine the cellular components required for immunoglobulin production by neonatal B cells in SCID mice. When neonatal B and adult T cells were transferred into the SCID mice, human immunoglobulins, largely of IgG, were maximally detected in the serum around 6 weeks after a cell transfer. Depletion of CD4⁺ T cells from adult T cells resulted in undetectable levels of human immunoglobulin in the serum. By contrast, CD4⁺ T cell-enriched populations exhibited an enhancing effect on immunoglobulin production by neonatal B cells. Higher levels of immunoglobulin, including IgA and IgM, were detected in the peritoneal fluid than in the serum as early as 2 weeks after the cell transfer. Human T cells expressing activation antigens such as CD45RO and HLA-DR antigens were identified in the peritoneal lavages. These results suggest that neonatal naive B cells are able to differentiate into cells producing all classes of immunoglobulin in the presence of mature CD4⁺ T cells in a SCID mouse environment. The peritoneal cavity of SCID mice appears to provide a suitable place for immune responses by human cells, possibly in association with a certain xenogeneic reaction.     

Erythropoietin-producing renal cell carcinoma in chronic hemodialysis patients: A report of two cases

Erythropoietin (EPO)-producing renal cell carcinomas in two hemodialysis patients are reported. Despite deteriorated kidney function, these patients did not manifest anemia at diagnosis and their elevated serum EPO levels rapidly returned to within the normal range after nephrectomy. Immunohistochemical staining of the resected specimens showed production of erythropoietin in the tumor cells in one case and in the lining cells of the cyst wall in the other case. Renal cell carcinoma could cause an increase of blood hematocrit level in dialysis patients.     

Impact of radical perineal prostatectomy on urinary continence and quality of life: A longitudinal study of Japanese patients

AIM: We used self-completed questionnaires to obtain a longitudinal assessment of urinary continence and urinary, bowel, and sexual domain-related quality of life (QOL) in Japanese patients undergoing radical perineal prostatectomy (RPP). METHODS: A total of 41 Japanese patients with a median age of 69 years who underwent RPP between February 2002 and February 2004 were included in the study. We measured QOL by the University of California, Los Angeles, Prostate Cancer Index (UCLA-PCI) and assessed urinary continence on the basis of three different definitions of continence. The International Prostate Symptom Score (I-PSS) was also included to evaluate lower urinary tract symptoms (LUTS). RESULTS: When urinary continence was de fi ned as none, one, or two protective pads per day, 100%, 73%, 94%, 97%, or 100% of the patients were continent before, and 1, 3, 6, and 12 months after, RPP, respectively. When it was de fi ned as total control or occasional dribbling, the corresponding values were 97%, 70%, 84%, 94%, and 97%. Urinary function returned to the preoperative baseline level by 6 months postoperatively and scores for urinary bother had significantly surpassed the baseline by 12 months (P = 0.043). The I-PSS was significantly improved (P = 0.014), with a mean 4.7-unit decrease. Sexual function worsened significantly after surgery, and its recovery was less favorable. No significant change was observed in scores for bowel function or bowel bother. CONCLUSIONS: The majority of patients who undergo RPP rapidly regain urinary continence and QOL within 3-6 months. RPP has a favorable impact on LUTS.     

EFFECT OF OCTAPEPTIDE SOMATOSTATIN ANALOGUE (SMS 201-995) ON PLASMA 7B2 (A NEUROENDOCRINE POLYPEPTIDE) LEVELS IN PATIENTS WITH ACROMEGALY

We studied the sequential changes of plasma levels of immunoreactive '7B2' (IR-7B2), a neuroendocrine polypeptide, after a subcutaneous injection of 50 micrograms of synthetic octapeptide somatostatin analogue (SMS 201-995) in seven patients with acromegaly due to GH-producing pituitary adenoma. Compared to the basal levels, mean plasma IR-7B2 and GH levels significantly decreased, until 5 and 10 h respectively after the administration of SMS 201-995. The mean (+/- SEM) nadir levels of plasma IR-7B2 and GH were 68.1 +/- 10.1 and 13.1 +/- 6.9%, respectively, compared to mean plasma levels before treatment (100%). Plasma IR-7B2 as well as GH levels did not change significantly when saline was administered subcutaneously to three acromegalic patients. In addition, plasma IR-7B2 levels did not change significantly after the administration of SMS 201-995 in normal subjects or in patients with primary hypothyroidism in whom SMS 201-995 induced a decrease of plasma TSH levels. These results strongly suggest that SMS 201-995 has an unequivocal suppressive effect on the synthesis and/or the secretion of 7B2 in human somatotroph adenoma cells.