Acute renal failure as a result of bilateral ureteral obstruction by Candida albicans fungus balls

A 73-year-old male with a history of diabetes mellitus was admitted to our hospital for acute renal failure. An ultrasonogram revealed bilateral hydronephrosis, which worsened despite insertion of a bladder catheter. Nephrostomy catheters were positioned bilaterally, and Candida albicans was found in the urine culture. The patient was successfully treated with intermittent direct irrigation and i.v. antifungal agent therapy. Since 1977, approximately 50 cases of fungus balls or fungal bezoars in the urinary tract have been reported, but the majority of these cases have been characterized by unilateral ureteral or bladder involvement. Herein, we report a case of acute renal failure as a result of bilateral ureteral obstruction by Candida albicans fungus balls.     

Acute heart failure and acute renal failure in Kawasaki disease

Acute renal failure and acute heart failure are rare in Kawasaki disease. We experienced two patients with Kawasaki disease who presented acute renal failure and acute heart failure. These two patients gave us an important insight into the understanding of water balance and fluid therapy in Kawasaki disease. One patient showed acute prerenal failure due to fluid exudation from the intravascular to the extravascular space, and subsequent acute heart failure. The other patient showed acute heart failure caused by fluid infusion for the treatment of dehydration. It is suggested that acute renal failure could be caused by a fluid shift from the intravascular to the extravascular space in Kawasaki disease. It is also demonstrated that the reserve of cardiac function could be decreased in patients with Kawasaki disease due to myocarditis even with normal echocardiography and chest X-rays.     

Liver transplantation in a case of hypoproteinemia and coagulopathy

A female infant with hypoproteinemia and coagulopathy associated with hypertyrosinemia was successfully treated with living-related liver transplantation (LRLT). On the 12th day of life plasma amino acid analysis revealed a marked elevation of tyrosine, so the patient was fed on a low-tyrosine and low-phenylalanine diet. However, hepatosplenomegaly. hypotonia, alopecia, eczema and psychomotor delay did not improve and recurrent episodes of disseminated intravascular coagulation (DIC) caused her condition to deteriorate. Liver biopsy on the 230th day revealed marked fatty change accompanied by mild to moderate cholestasis. Therefore. LRLT from her father was performed on the 286th day resulting in improvement of all the aforementioned signs and symptoms. Despite a thorough examination, no diagnosis of a known disorder could be established. However, her elder brother had also been born with severe hypoproteinemia and coagulopathy, and died of DIC on the second day of life. Thus, the disorder is designated as a new entity, namely ‘congenital hypoproteinemia and coagulopathy associated with hypertyrosinemia’.     

Paget''s disease of the vulva: clinicopathologic study of type 1 cases treated at a single institution

The aim of this study was to evaluate clinicopathologic characteristics of primary cutaneous Paget's disease of the vulva. Between 1986 and 2005, 22 patients with primary cutaneous Paget's disease of the vulva (type 1) were treated at Tohoku University Hospital. Medical records were reviewed for pathologic diagnosis, patient age, associated neoplasms, type(s) of eczema, symptom duration, treatment, surgical procedures, recurrence, and length of follow-up. Patient age ranged from 51 to 85 years (median 71.5 years). Median duration of symptoms was 24 months (range 2-60 months). Type 1a (intraepithelial) Paget's disease accounted for 18 patients, with 3 type 1b (invasive) cases and 1 type 1c (intraepithelial disease with underlying adenocarcinoma) case. Mean length of follow-up was 53.7 months, and median follow-up was 49 months (range 6-199 months). Only two patients had an associated internal malignancy: T-cell leukemia and breast cancer. Mapping biopsy was performed in 14 of the 18 type 1a cases. All patients were free of disease at the surgical margins and are alive without recurrence. The four patients with type 1b or 1c disease had lymph node metastases. Two has died of disease, and two are alive with no recurrence. The rate of secondary malignancy seems to be low in primary cutaneous Paget's disease of the vulva. Mapping biopsy with careful examination of characteristic skin surface may be useful for surgery of type 1a cases. Inguinal lymphadenectomy is recommended in cases with question of invasion or known underlying adenocarcinoma.     

Defective activity of Epstein-Barr virus (EBV) specific cytotoxic T lymphocytes in children with chronic active EBV infection and in their parents

Antibodies of Epstein-Barr virus (EBV), EBV-specific cytotoxic T lymphocyte (EBVCTL) activity and the lymphocyte subset of CTL were examined in 13 Japanese children with chronic active EBV infection (CAEBV) and their parents (eight fathers and 10 mothers). Anti-virus-capsid antigen (VCA)-IgG antibody titers ranged from 1: 640 to 1: 5120 in the patients with CAEBV and from 1: 40 to 1: 640 in the parents. While anti-VCA-IgM antibody was detected in three patients, anti-VCA-IgA antibody in five and anti-early-antigen (EA)-IgG antibody in 11, no antibody was detected in the parents except anti-EA antibody, which was positive in the mothers of cases 5 and 13 (1: 10 and 1: 40). Anti-EBV-associated nuclear antigen (EBNA) antibody was ≥ 1: 10 in six out of 13 patients with CAEBV and in 10 out of 18 parents tested. Epstein-Barr virus activity was significantly lower (P < 0.005) both in the children with CAEBV and in their parents than in seropositive age-matched controls. Proportions of a CTL subset (CD8⁺ CD11^? lymphocytes) in the patients with CAEBV were significantly higher (P< 0.005) than in controls, while those in the parents were at the same level as in controls. Defective EBVCTL activity and anti-EBNA-antibody responses were frequently observed both in children with CAEBV and in their parents, which may suggest that the abnormal immune response to EBV may be based on a familial disorder, though no familial involvement has been reported in Japanese children with CAEBV.     

Defective natural killer cell activity and deficient production of interferon-γ in children with acute lymphoblastic leukemia

Natural killer (NK) cell activity, OK-432-augmented-NK cell activity, concentrations of interferon-γ (IFN-γ) in the culture supernatants of lymphocytes stimulated with OK-432, and subsets of NK cells and memory T cells were analyzed in 42 children with acute lymphoblastic leukemia (ALL) receiving maintenance chemotherapy. Natural killer and augmented-NK cell activities, and concentrations of IFN-γ in the supernatants of cultured lymphocytes, were significantly lower in the patients with ALL than in age-matched control children. Among the NK cell subsets, proportions of CD57⁺ cells in the patients with ALL were significantly higher than in the controls, and proportions of a memory T cell subset (CD4⁺ CD29⁺ T cells) in the patients were also significantly higher than in the controls. These results suggest that the function of NK cells and memory T cells that are considered as IFN-γ producing cells, may be defective in ALL, and that CD57⁺ cells and CD4⁺ CD29⁺ cells may be resistant to or recover rapidly from suppression by cytotoxic chemotherapy.     

Obliterative portal venopathy: A comparative study of 184 cases of extrahepatic portal obstruction and 469 cases of idiopathic portal hypertension

Abstract A total of 184 cases of extrahepatic portal obstruction (EHPO), mostly demonstrated by intraoperative portography and studied at 17 institutes during the period 1957–1983, were compared with 469 cases of idiopathic portal hypertension (IPH) similarly studied. Of the cases of EHPO, there were 101 males and 83 females; 93 were under 20 years of age and the average age was 25.9 years (i.e. much younger than that of IPH cases). There were two age peaks, one before age 19 years and the other at age 40–49 years. One out of three adult cases had a history of abdominal surgery, but otherwise the aetiologic factor was difficult to elicit. Bleeding was the initial symptom in the majority, and splenectomy and haematological findings of hypersplenism were less pronounced compared with IPH. Liver function tests were almost always normal. The liver appeared normal macroscopically in 69% and histologically in 35%. The changes seen in the remainder were similar to those in IPH; they were less frequent in young patients than in cases above age 20 years. Compared with IPH, the wedged hepatic venous pressure in patients with EHPO was lower and the gradient from the portal venous pressure was greater. It is concluded that extrahepatic portal obstruction is less common compared with IPH in Japan, and that there are cases particularly among adults that present clinicopathological features very similar to those of IPH. It is unclear at present whether these two disorders represent two different disease entities, or whether they represent one disorder with differences in the site of involvement along the portal vein system.     

An anecdote in the dawn of Japanese psychiatry: The tragedy of Dr. Noboru Ishida

Abstract This is a historical review of the tragic life of a psychiatrist in the dawn of Japanese psychiatry, who was sentenced to life imprisonment for first-degree murder by an American court and imprisoned in America. The purpose of this article is firstly to clarify the strange circumstances under which he was sentenced guilty in spite of his psychosis. In the author's opinion there appeared to be an incorrectness and injustice of the judicial decision in the American court from consideration of the documents of his trial. Secondly, the author would like to acknowledge Dr. Ishida's pioneering contributions to the Japanese psychiatry which, because of the circumstances, have been long buried in the history of Japanese psychiatry.     

An association study between the Cys311 variant of dopamine D2 receptor gene and schizophrenia in the Okinawan population

Abstract  Neuroleptic drugs have a high affinity for the dopamine D₂ receptor (DRD2); therefore DRD2 is thought to be a candidate gene for schizophrenia. Arinami et al . have reported a positive association between schizophrenia and the Cys311 variant of the DRD2 gene. We determined the allele frequency of this polymorphism in 78 Okinawan schizophrenic patients and 112 control subjects. The patients and controls did not differ significantly in allele frequencies of Cys311.