Prognosis and pathological characteristics of five children with non-Shiga toxin-mediated hemolytic uremic syndrome

BACKGROUND: The three major signs of hemolytic uremic syndrome (HUS) are hemolytic anemia, thrombopenia and acute renal failure. HUS is classified into Shiga toxin-mediated HUS (Stx-HUS) and non-Shiga toxin-mediated HUS (nStx-HUS). The prognosis of nStx-HUS is reported to be less favorable than that of Stx-HUS. Although the association between the prognosis and pathological characteristics of HUS have been reported such that the prognosis was considered to be poor for thrombotic microangiopathy (TMA) with predominant arterial involvement (arterial TMA), good for TMA with predominant glomerular involvement (glomerular TMA) and dependent on the extent of necrosis in cases of renal cortical necrosis, it is not yet clear whether pathological findings are also related to the renal prognosis of nStx-HUS cases. Therefore the purpose of the present paper was to analyze renal biopsy findings and prognosis for five children with nStx-HUS. METHODS: Clinical records of five cases of nStx-HUS among 74 cases of diagnosed HUS were reviewed, and information and data were summarized. RESULTS: Histological examination of the kidney led to the diagnosis of arterial TMA in three cases, and glomerular TMA and severe renal cortical necrosis in one case each. Analysis of the relationship between renal histological findings and the prognosis found that three patients with arterial TMA and one patient with severe renal cortical necrosis later developed end-stage renal failure while one patient with glomerular TMA has continued to show normal renal function. CONCLUSIONS: These findings indicate that pathological findings are closely related to the prognosis in cases of nStx-HUS.     

High-dose mizoribine treatment for adolescents with systemic lupus erythematosus

BACKGROUND: In treating pediatric patients with systemic lupus erythematosus (SLE), it is necessary to quickly attain remission to avoid sequelae in various organs and to maintain it over a long period. However, to maintain remission, the prolonged use of immunosuppressants which have various adverse effects, is often necessary in addition to steroids, and complications due to such immunosuppressants pose very important problems. A regimen of mizoribin (MZR) at 150 mg/day divided into two or three doses has been recommended, but while this regimen has been safe, its efficacy has not been satisfactory. However, MZR produces effects dose-dependently, and the dose recommended to date may have been insufficient for the treatment of children with SLE. METHODS: The authors administered oral MZR at 300 mg/day in two divided doses, which is twice the conventional dose for adults, to five adolescents with SLE. Three of these five were markedly steroid-dependent patients and two had previously been treated with steroids only. Thereafter, the authors evaluated the safety and efficacy of the regimen by following the patients for at least 7 months after the beginning of treatment. RESULTS: Patients 1 and 2 had been treated with prednisolone (PSL) and cyclosporine (CyA), but as the duration of CyA administration became long, it was replaced with 300 mg MZR. This transition could be accomplished smoothly. Patient 3 showed repeated recurrence during the treatment with PSL and CyA or CPM, but the symptoms could be controlled by the addition of 300 mg MZR. In patients 4 and 5, the control of symptoms with PSL alone was judged to be difficult, and concomitant administration of MZR at 300 mg was started. This resulted in a decrease in the dose of PSL. The Cmax (C2) of MZR was 1.33 microg/mL or higher in all five patients, and the efficacy of the treatment was satisfactory. Concerning side-effects, hyperuricemia was noted in two patients, but it was resolved in one of them by reducing the dose of MZR and in the other spontaneously while the treatment was continued. Temporary exacerbation of hair loss was observed in two patients, but it disappeared in both of them after a few months. CONCLUSION: MZR could be administered at a high dose effectively and safely. However, monitoring of the serum uric acid level was necessary. High-dose MZR therapy showed an efficacy and safety that would warrant its application to steroid-dependent pediatric patients with SLE.     

Tobacco use among Japanese schoolchildren: results from preliminary study of Japan adolescent smoking survey (JASS)

Over the past two decades many studies have shown associationsbetween smoking and increased health problems. Smoking amongchildren is especially a major public health concern. Preventingyoung people from developing a smoking habit is an importantgoal for school health education. In Japan, it is difficult to collect accurate data regardingadolescent smoking behaviour, because in Japan smoking underthe age of 20 is prohibited by law. Therefore, a large surveyof smoking habits of young Japanese people was overdue. Numeroussurveys have been carried out since the late 1970s, but thesehave been limited in sample size or geographic area. The Japan Adolescent Smoking Survey (JASS) is probably the largestnumerical survey of smoking behaviour and opinions of schoolchildrenever carried out in Japan. Here the authors show the preliminaryresults of JASS. The goals of the JASS study are to standardize the method ofmeasurement in the smoking behaviour of young children. We believeit is now possible to compare national and international smokingrates of young people, and further, to develop and evaluateteaching methods about smoking for schoolchildren on the basisof this standardization. This report presents the tobacco use among schoolchildren inJapan.     

Malignant Paraganglioma of the Stomach

Abstract: We report a case of malignant paraganglioma of the stomach. A gastric endoscopic examination on a 67-year-old female, performed for follow-up purposes, revealed an irregularly elevated lesion with a central ulcer on the lesser curvature of the lower body. The lesion showed characteristics of both an epithelial tumor and a submucosal tumor by endoscopy and radiography. The biopsy specimen suggested that the lesion was a malignant poorly differentiated tumor. On abdominal ultrasonography and computed tomography, several space occupying lesions were found in both lobes of the liver. Therefore, this case was diagnosed as having an advanced gastric cancer with liver metastases and underwent distal gastrectomy. Postoperatively, a final diagnosis of gastric paraganglioma was made by histological and immunohistochemical findings (positive for Grimelius, neuron specific enolase and other stains). These findings also revealed lymph node metastases. To date, only seven cases of gastric paraganglioma have been reported. Furthermore, two cases of malignant gastric paraganglioma have also been described in the literature. This is the first case of malignant gastric paraganglioma evaluated in detail by endoscopic and radiographic examinations.