Mesna, Doxorubicin, Ifosfamide and Dacarbazine (MAID) Combination Chemotherapy for Retroperitoneal Sarcoma: A Single-center Experience.

BACKGROUND/AIM: There is limited evidence regarding the systemic treatment of retroperitoneal soft-tissue sarcoma, and the current Japanese guidelines fail to make definitive suggestions. Here, we report our experience with combination chemotherapy of mesna, doxorubicin, ifosfamide, and dacarbazine (MAID) in this population. PATIENTS AND METHODS: We retrospectively reviewed the records of eight patients (three male and five female) who received MAID for pathologically diagnosed metastatic unresectable retroperitoneal sarcoma (either leiomyosarcoma or pleomorphic sarcoma) between October 2019 and January 2022. Treatment efficacy, tolerability (need for dose reduction), and safety profiles were evaluated and summarized. RESULTS: At initiation, the median age was 56.0 years, and the body mass index was 20.0 kg/cm2 Six patients had Eastern Cooperative Oncology Group performance status scores of 0. The net clinical benefit was a partial response in three (37.5%) patients, stable disease in four (50.0%), and progressive disease in one (12.5%). During the median 90.8 weeks of follow-up, disease in five patients progressed, resulting in a median progression-free survival of 48.4 weeks, and five deaths occurred, resulting in an overall survival of 95.1 weeks. Commonly observed adverse events were neutropenia (eight patients), anemia (eight patients), and decreased platelet count (seven patients), which led to dose reduction (60-80%) in six patients. CONCLUSION: MAID combination therapy may be an acceptable option for advanced retroperitoneal sarcoma; however, its benefits must be carefully assessed owing to its not insignificant toxicity.

Survival Impact of Glucocorticoid Administration for Adverse Events During Immune Checkpoint Inhibitor Combination Therapy in Patients with Previously Untreated Advanced Renal Cell Carcinoma.

BACKGROUND: The impact of glucocorticoid administration for adverse events (AEs), including immune-related AEs, on the effectiveness of immune checkpoint inhibitor (ICI) combination therapy for advanced renal cell carcinoma (RCC) remains unknown. OBJECTIVES: To clarify the prognostic impact of glucocorticoid use for AEs during first-line ICI combination therapy for advanced RCC. PATIENTS AND METHODS: We retrospectively evaluated data from 194 patients who received dual ICI combination therapy [i.e., immunotherapy (IO)-IO] or combinations of ICIs with tyrosine kinase inhibitors (TKIs) as first-line therapy. The patients were divided into two groups according to the history of glucocorticoid administration in each treatment group. Survival based on glucocorticoid administration was assessed. RESULTS: A total of 101 (52.0%) and 93 (48.0%) patients received IO-IO and IO-TKI combination therapy, respectively. Glucocorticoids were administered to 46 (46%) and 22 (24%) patients in the IO-IO and IO-TKI groups, respectively. In the IO-IO group, progression-free survival (PFS) and overall survival (OS) were significantly longer in patients with glucocorticoid administration than in those without administration (median PFS: 14.4 versus 3.45 months, p = 0.0005; median OS: 77.6 versus 33.9 months, p = 0.0025). Multivariable analysis showed that glucocorticoid administration was an independent predictor of longer PFS (hazard ratio: 0.43, p = 0.0005) and OS (hazard ratio: 0.35, p = 0.0067) after adjustment for covariates. In the IO-TKI group, neither PFS nor OS significantly differed between patients treated with and without glucocorticoid administration (PFS: p = 0.0872, OS: p = 0.216). CONCLUSIONS: Glucocorticoid administration did not negatively impact the effectiveness of ICI combination therapy for RCC, prompting glucocorticoid treatment use when AEs develop.

Effects of remote ischemic preconditioning on renal protection in patients undergoing robot-assisted laparoscopic partial nephrectomy.

We aimed to evaluate the renoprotective effects of remote ischemic preconditioning (RIPC) in patients undergoing robot-assisted laparoscopic partial nephrectomy (RAPN). Data from 59 patients with solitary renal tumors who underwent RAPN with RIPC comprising three cycles of 5-min inflation to 200 mmHg of a blood pressure cuff applied to one lower limb followed by 5-min reperfusion by cuff deflation, from 2018 to 2020 were analyzed. Patients who underwent RAPN for solitary renal tumors without RIPC between 2018 and 2020 were selected as controls. The postoperative estimated glomerular filtration rate (eGFR) at the nadir during hospitalization and the percentage change from baseline were compared using propensity score matching analysis. We performed a sensitivity analysis with imputations for missing postoperative renal function data weighted by the inverse probability of the data being observed. Of the 59 patients with RIPC and 482 patients without RIPC, 53 each were matched based on propensity scores. No significant differences in the postoperative eGFR in mL/min/1.73 m2 at nadir (mean difference 3.8; 95% confidence interval [CI] - 2.8 to 10.4) and its percentage change from baseline (mean difference 4.7; 95% CI - 1.6 to 11.1) were observed between the two groups. Sensitivity analysis also indicated no significant differences. No complications were associated with the RIPC. In conclusion, we found no significant evidence of the protective effect of RIPC against renal dysfunction after RAPN. Further research is required to determine whether specific patient subgroups benefit from RIPC.Trial registration number: UMIN000030305 (December 8, 2017).

Therapeutic role of deferred cytoreductive nephrectomy in patients with metastatic renal cell carcinoma treated with nivolumab plus ipilimumab.

OBJECTIVES: To explore the therapeutic role of deferred cytoreductive nephrectomy in patients with metastatic renal cell carcinoma treated with nivolumab plus ipilimumab. PATIENTS AND METHODS: Forty-one patients with synchronous metastatic renal cell carcinoma who received nivolumab plus ipilimumab as first-line systemic therapy at our affiliated institutions were retrospectively evaluated. We focused on the prognosis, including tumor responses in primary kidney and metastatic lesions in patients treated with deferred cytoreductive nephrectomy. In addition, the overall survival according to nephrectomy status (i.e. deferred cytoreductive nephrectomy vs. upfront cytoreductive nephrectomy vs. without cytoreductive nephrectomy) was compared. RESULTS: During a median follow-up period of 12.0 months, seven (30%) patients received deferred cytoreductive nephrectomy at a median time of 10.4 months after nivolumab plus ipilimumab initiation. All the patients showed tumor shrinkage in their primary kidney lesions, including six (86%) patients with ≥30% of shrinkage. Metastatic lesions were also shrunk by ≥30% in six (86%) patients, including two (29%) obtaining complete response. At the last time of follow-up, three (43%) patients were disease-free. The overall survival rate after nivolumab plus ipilimumab initiation tended to be higher in patients with deferred cytoreductive nephrectomy compared with those with upfront cytoreductive nephrectomy (1-year survival rate: 100% vs. 72.4%, P = 0.0587) and those without cytoreductive nephrectomy (vs. 58.2%, P = 0.0613). CONCLUSIONS: The present retrospective data showed that deferred cytoreductive nephrectomy had the potential to exert a therapeutic effect in a subset of patients who obtained favorable tumor responses to nivolumab plus ipilimumab for a certain period. Prospective randomized clinical trials are needed to confirm the prognostic impact of deferred cytoreductive nephrectomy after frontline immunotherapy in synchronous metastatic renal cell carcinoma.

Urinary Tract Infection in Hemodialysis-Dependent End-Stage Renal Disease Patients.

INTRODUCTION: The aim of this study was to analyze urinalysis findings and urinary bacterial culture in hemodialysis-dependent end-stage renal disease patients. The research goal was to understand the proportion, risk factors, and the causative organisms of urinary tract infection in hemodialysis-dependent end-stage renal disease patients. MATERIALS AND METHODS: Between May 2020 and June 2021, this study included 100 hemodialysis-dependent end-stage renal disease patients (50 male patients and 50 female patients). The urine underwent microscopic examination, pyuria was defined as ≥5 white blood cells per high-power field, and urinary bacterial cultures were conducted for patients with pyuria. Bacteriuria was defined as ≥104 colony-forming units/mL in men and ≥105 colony-forming units/mL in women. Daily urine output was investigated by oral listening. Postvoiding residual urine volume was measured. RESULTS: Fifty-six percent of male patients and 30% of female patients had normosthenuria, 24% of male patients and 38% of female patients had pyuria, and 20% of male patients and 32% of female patients had a urinary tract infection. A comparison of normosthenuria and urinary tract infection revealed no statistically significant difference in age, time on dialysis, daily urine output, and postvoiding residual urine volume. The proportion of female patients among those with normosthenuria was 34.8%, whereas the proportion of female patients among those with UTI was 61.5%. Urinary bacterial cultures showed that the major causative organisms were Escherichia coli (45%; 18/40 cultures) and extended spectrum beta-lactamase-producing Escherichia coli (17.5%; 7/40 cultures). CONCLUSION: The incidence of urinary tract infection was higher in female patients than in male patients. The proportion of resistant bacteria as the causative organisms was high in hemodialysis-dependent end-stage renal disease patients. Urinary bacterial culture should be checked while patients are able to void urine.

New Longitudinal Component of the RENAL Nephrometry Score for Predicting the Operative Complexity in Transperitoneal Robot-Assisted Partial Nephrectomy.

Background: In transperitoneal robot-assisted partial nephrectomy (RAPN), an L score of 3 points according to the RENAL nephrometry scoring system does not necessarily denote operative complexity. This study aimed to assess the efficacy of the newly defined longitudinal component to analyze the operative complexity of RAPN. Materials and Methods: We retrospectively analyzed transperitoneal RAPNs performed by a single experienced surgeon for renal tumors between 2017 and 2020. L component was defined as L'1 for midlocated tumors, L'2 for >50% below the polar line, and L'3 for >50% above the polar line. Multivariate regression analysis was performed to test associations between prolonged console time and preoperative factors. The perioperative outcomes were compared among the three cohorts of L' components using propensity score matching: L'1 vs L'3 and L'1 vs L'2. Results: A total of 220 cases (L'1: 107, L'2: 65, L'3: 48) were analyzed. The median console time was prolonged (>130 minutes) in 55 patients (median 108, interquartile range: 90-130 minutes). Longitudinal location (L'3 odds ratio [OR]: 2.93, p = 0.01; L'2 OR: 2.32, p = 0.04), high Mayo adhesive probability score (p = 0.001), multiple renal arteries (p = 0.03), and large size (p = 0.04) were significantly associated with prolonged console time. After matching, 26 cases of L'1 and L'3 and 43 cases of L'1 and L'2 were selected. Console time (108 minutes vs 132 minutes, p = 0.017) and warm ischemia time (17 minutes vs 22 minutes, p = 0.03) were significantly longer in L'3 than in L'1. The difference in console time between L'1 and L'2 was not statistically significant (100 minutes vs 111 minutes, p = 0.08). Conclusion: In the new longitudinal assessment, upper location predicted prolonged console time compared with a middle or lower location. The L' component may help preoperatively assess operative complexity.

Albumin-to-Alkaline Phosphatase Ratio as a Novel Prognostic Marker of Nivolumab Monotherapy for Previously Treated Metastatic Renal Cell Carcinoma.

BACKGROUND/AIM: The relationship between albumin-to-alkaline phosphatase ratio (AAPR) and the outcome of patients with metastatic renal cell carcinoma (mRCC) treated with immune checkpoint inhibitors remains unresolved. We aimed to clarify the prognostic role of AAPR in nivolumab monotherapy for previously treated mRCC. PATIENTS AND METHODS: We retrospectively evaluated 60 patients with mRCC treated with nivolumab after failure of at least one molecular targeted therapy. The patients were stratified into two groups based on the baseline AAPR. The threshold of AAPR was determined using receiver-operating characteristics and Youden index analyses. Overall survival (OS), progression-free survival (PFS), and objective response rate (ORR) of nivolumab therapy were compared between the high and low AAPR groups. RESULTS: The threshold of AAPR was set at 0.3, and 20 patients (33%) were assigned to the low AAPR group. The median OS and PFS were significantly lower in the low AAPR group than those in the high group (OS: 8.3 months vs. not reached, p<0.0001; PFS: 2.9 vs. 10.4 months, p=0.0006). Moreover, ORR was significantly lower in the low AAPR group than in the high group (16% vs. 45%, p=0.0397). Multivariate analyses further showed that AAPR was an independent factor for OS [HR=0.27 (95% CI=0.09-0.77), p=0.0151] but not for PFS (p=0.174). CONCLUSION: Baseline AAPR was significantly associated with outcome in patients with mRCC receiving nivolumab monotherapy and may, therefore, constitute an effective prognostic factor for nivolumab treatment.

Efficacy of Cabozantinib in Metastatic Papillary Renal Cell Carcinoma Following Ineffective Treatment With Initial Therapy of Nivolumab and Ipilimumab.

Sunitinib is listed as first-line therapy for non clear-cell renal cell carcinoma (RCC) in several guidelines. However, in the era of immunotherapy, there is an urgent need for updated evidence for the treatment of metastatic non clear-cell RCC. Herein, we present three cases of patients with type 2 papillary RCC who were effectively treated with cabozantinib. The first case was a 48-year-old woman who underwent radical nephrectomy (pT3aN0M0). The tumor relapsed in the retroperitoneum 3 months postoperatively and was unresponsive to first-line nivolumab plus ipilimumab (NI). After the use of cabozantinib, the tumors drastically shrunk in 2 weeks, and complete response was achieved 3 months later. The second case was a 55-year-old man who underwent radical nephrectomy (pT3aN2M1). Metastatic lesions continued to grow with first-line NI, and cabozantinib was used as the second-line therapy. All metastatic lesions had shrunk by 50% after 4 months. The third case was a 36-year-old man with multiple tumors in the left solitary kidney and iliopsoas muscle metastasis. First-line therapy with NI was ineffective; subsequently, second-line axitinib was used for 5 months, and the disease was identified as progressive. Cabozantinib was started as third-line therapy. Multiple tumors shrunk in 2 weeks. There is little evidence concerning the treatment of papillary RCC. We experienced low efficacy of NI for first-line treatment of papillary RCC for three patients who were subsequently effectively treated with cabozantinib. Cabozantinib inhibits multiple tyrosine kinase receptors, which may suppress aggressive tumor progression of type 2 papillary RCC. Cabozantinib or combination with immuno-oncological drugs may be a promising treatment option for papillary RCC.

[Psychological Support for Children and Adolescents with Gender Dysphoria -From a Clinical Psychologist's Viewpoint-].

This paper considers what psychological support should be provided for children and ado- lescents with gender dysphoria based on lessons learnt as a member of a comprehensive medi- cal care team for patients with Gender Identity Disorder (GID), and as a school counselor (SC). The characteristics of adult patients with GID and the results of psychological tests, together with the issues and problems these patients experienced in childhood are analyzed. The need to provide care in consideration of their low self-esteem caused by the inconsiderate remarks of others, how to lessen the conflicts arising from the stereo-typed gender images imposed at school, and the capability to face gender dysphoria and build their future are pointed out Based on actual school cases, ideas for the support needed for these children and adolescents with gender dysphoria are presented. This takes a developmental viewpoint of a clinical psychologist who endeavors to provide children and adolescents with a safe place to talk about gender dysphoria problems, and serve as a liaison between the school and home.

Neuropsychological Evaluation and Cerebral Blood Flow Effects of Apolipoprotein E4 in Alzheimer's Disease Patients after One Year of Treatment: An Exploratory Study.

BACKGROUND: Alzheimer's disease (AD) is affected by apolipoprotein E (ApoE); however, its effects assessed by means of cognitive tests and by neuroimaging have not been sufficiently studied. METHODS: We administered the Alzheimer's Disease Assessment Scale (ADAS) and single-photon emission computed tomography imaging in patients with AD medicated with donepezil at baseline and after 1 year. Patients were classified as with or without ApoE4 and we evaluated the progress of AD. RESULTS: Analysis of covariance showed that cerebral blood flow after 1 year in subjects with ApoE4 is significantly reduced in some areas including the left lenticular nucleus, left thalamus, and right hippocampus compared with subjects without ApoE4. Paired t tests showed significantly reduced blood flow in several regions including the right hippocampus in subjects with ApoE4 and significant deterioration of ideational praxis in subjects without ApoE4. CONCLUSION: This study provides evidence that supports the notion of ApoE4 playing an important role in the progress of AD.

Psychological characteristics of Japanese patients with chronic pain assessed by the Rorschach test.

BACKGROUND: The increasing number of patients with chronic pain in Japan has become a major issue in terms of the patient's quality of life, medical costs, and related social problems. Pain is a multi-dimensional experience with physiological, affective, cognitive, behavioral and social components, and recommended to be managed via a combination of bio-psycho-social aspects. However, a biomedical approach is still the dominant method of pain treatment in Japan. The current study aimed to evaluate comprehensive psychological functions and processes in Japanese chronic pain patients. METHODS: The Rorschach Comprehensive System was administered to 49 in-patients with non-malignant chronic pain. Major variables and frequencies from the test were then compared to normative data from non-patient Japanese adults by way of the t-test and chi-square test. RESULTS: Patients exhibited high levels of emotional distress with a sense of helplessness with regard to situational stress, confusion, and ambivalent feelings. These emotions were managed by the patients in an inappropriate manner. Cognitive functions resulted in moderate dysfunction in all stages. Information processing tended to focus upon minute features in an inflexible manner. Mediational dysfunction was likely to occur with unstable affective conditions. Ideation was marked by pessimistic and less effective thinking. Since patients exhibited negative self-perception, their interpersonal relationship skills tended to be ineffective. Originally, our patients displayed average psychological resources for control, stress tolerance, and social skills for interpersonal relationships. However, patient coping styles were either situation- or emotion-dependent, and patients were more likely to exhibit emotional instability influenced by external stimuli, resulting in increased vulnerability to pain. CONCLUSIONS: Data gathered from the Rorschach test suggested psychological approaches to support chronic pain patients that are likely to be highly beneficial, and we thus recommend their incorporation into the course of current pain treatments.

Periodontal disease adversely affects the survival of patients with end-stage renal disease.

Periodontal disease is associated with cardiovascular disease and is thought to accelerate systemic atherosclerosis. Here we examined the relationship between periodontitis and cardiovascular disease mortality in outpatients on hemodialysis using a retrospective analysis of 168 adult patients in New York City and North Carolina. During 18 months of follow-up, cardiovascular disease and all-cause mortality were determined from a centralized dialysis registry. One hundred patients had mild or no periodontal disease but the remaining 68 had moderate-to-severe disease defined as 2 or more teeth with at least 6 mm of inter-proximal attachment loss. At baseline, the proportion of males was significantly lower in the moderate-to-severe group. Compared with mild or no periodontal disease, moderate-to-severe disease was significantly associated with death from cardiovascular causes. Adjustment for age, gender, center and dialysis vintage, smoking status, and history of diabetes mellitus or hypertension did not diminish the strength of this association. Our findings suggest a need for larger studies to confirm this connection, along with intervention trials to determine if treating periodontitis reduces cardiovascular disease mortality in dialysis patients.

Severe periodontitis is associated with low serum albumin among patients on maintenance hemodialysis therapy.

The relationship between periodontitis and two measures of systemic inflammation, serum albumin and C-reactive protein (CRP), were examined among patients who were receiving chronic outpatient hemodialysis. Adult patients at two locations, North Carolina and New York City, were evaluated by dentist examiners. Six sites per tooth (up to 32 teeth per patient) were examined. A periodontitis case was defined as > or = 60% of sites with attachment level > or = 4 mm. Multivariable logistic regression was used to determine the association of periodontitis with low serum albumin, defined as < 3.5 mg/dl, and with high CRP, defined as > 3.0 mg/dl. A total of 154 patients completed the study. The mean age was 54.6 yr (SD 13.3), and average duration of dialysis was 4.0 yr (3 mo to 16 yr). Eighty-six (54.6%) were men, and 89 (58.2%) were black. Common causes of end-stage kidney disease were hypertension (12.3%), diabetes (22.1%), glomerulonephritis (7.1%), and other (58.4%). The average number of teeth was 20.3 (SD 8.4). Thirty-five (23%) patients were periodontitis cases. Severe periodontitis was associated with low serum albumin (odds ratio 8.20; 95% confidence interval 1.61 to 41.82; P = 0.01) compared with individuals without severe periodontitis disease after adjustment for age, gender, race, diabetes, hypertension, body mass index, smoking, study site, total cholesterol, serum calcium, serum phosphorus, and normalized protein catabolic rate. There was no observed association of severe periodontitis with CRP. Investigation of the potential contribution of periodontitis to serum albumin and possibly to morbidity and mortality among patients with end-stage kidney disease seems warranted.

Body protein index based on bioelectrical impedance analysis is a useful new marker assessing nutritional status: applications to patients with chronic renal failure on maintenance dialysis.

BACKGROUND: Evaluation and monitoring of nutritional status is a fundamental concept in providing nutritional care to patients with end-stage renal failure. There have been, however, few practically available indices assessing whole body protein stores of patients. METHODS: We enrolled 448 end-stage renal disease patients, 394 on maintenance hemodialysis (HD) and 54 on continuous ambulatory peritoneal dialysis (PD) in this study. 83 Age- and sex-matched subjects (controls) whose creatinine clearance was more than 70 ml/min and urinary protein excretion was less than 1.0 g/day were also recruited for comparison. To assess whole body somatic protein stores, we devised the body protein index (BPI). The volume of body protein mass was measured by multifrequency bioelectrical impedance analysis and then BPI was calculated as body protein mass (kg) divided by height in meters (m2). Based on BPI, we defined the nutritional status of the patients as normal if the value was within -10% of the mean value of control subjects, -10 to -14% as mild malnutrition, -15 to -19% as moderate malnutrition, and <-20% as severe malnutrition. RESULTS: The required time for measurement was 5.2 +/- 1.3 min and coefficient of variation of measurements was 0.8 +/- 0.2%. Among men the mean BPI in both HD and PD patients was significantly lower than those of control subjects (4.25 +/- 0.37, 4.38 +/- 0.34 vs. 4.72 +/- 0.37 kg/m2, p < 0.001). In women, BPI was significantly lower in HD patients than in control subjects (3.65 +/- 0.34 vs. 4.00 +/- 0.34 kg/m2, p < 0.033), whereas only a nonsignificant lower tendency was found in PD patients (3.83 +/- 0.39 kg/m2, p = 0.067). There were no significant differences in BPI values between diabetic and non-diabetic subjects, both in men (4.26 +/- 0.41 vs. 4.25 +/- 0.36 kg/m2) and women (3.69 +/- 0.36 vs. 3.65 +/- 0.34 kg/m2). Based on BPI nutritional categories, 113 (28.7%) of all HD patients were classified as having mild malnutrition, 57 (14.5%) as having moderate malnutrition, 40 (10.1%) as having severe malnutrition, and 184 (46.7%) were classified as normal. The patients of longer dialysis history groups showed a tendency of lower BPI compared to those of shorter dialysis history groups (p < 0.05), although the ages of the patients of the two groups did not significantly differ. No correlations were found between BPI and serum albumin or transferrin concentrations. Only weak correlations were found with albumin in male and transferrin in female HD patients. CONCLUSION: BPI calculated from measurement of multifrequency bioelectrical impedance analysis could evaluate whole body somatic protein stores, and is a potentially useful new marker assessing nutritional status in patients with chronic renal failure. Decreased body somatic protein stores, mainly due to muscle wasting, was prevalent in end-stage renal failure patients on maintenance dialysis.

International differences in dialysis mortality reflect background general population atherosclerotic cardiovascular mortality.

Existing national, racial, and ethnic differences in dialysis patient mortality rates largely are unexplained. This study aimed to test the hypothesis that mortality rates related to atherosclerotic cardiovascular disease (ASCVD) in dialysis populations (DP) and in the background general populations (GP) are correlated. In a cross-sectional, multinational study, all-cause and ASCVD mortality rates were compared between GP and DP using the most recent data from the World Health Organization mortality database (67 countries; 1,571,852,000 population) and from national renal registries (26 countries; 623,900 population). Across GP of 67 countries (14,082,146 deaths), all-cause mortality rates (median 8.88 per 1000 population; range 1.93 to 15.40) were strongly related to ASCVD mortality rates (median 3.21; range 0.53 to 8.69), with Eastern European countries clustering in the upper and Southeast and East Asian countries in the lower rate ranges. Across DP (103,432 deaths), mortality rates from all causes (median 166.20; range 54.47 to 268.80) and from ASCVD (median 63.39 per 1000 population; range 21.52 to 162.40) were higher and strongly correlated. ASCVD mortality rates in DP and in the GP were significantly correlated; the relationship became even stronger after adjustment for age (R(2) = 0.56, P < 0.0001). A substantial portion of the variability in mortality rates that were observed across DP worldwide is attributable to the variability in background ASCVD mortality rates in the respective GP. Genetic and environmental factors may underlie these differences.

[A pregnant woman with nephrotic syndrome and focal segmental lesions].

A 32-year-old woman was admitted at 36 weeks' gestation because of increasing proteinuria and generalized edema. At the time of admission, serum creatinine was 1.3 mg/dl, and urinalysis demonstrated 4+ protein and 2+ occult blood. During her pregnancy, her blood pressure had been in the normal range. A normal healthy female neonate was delivered by caesarean section at 38 weeks' gestation. After delivery, the woman's 24-hour urine protein excretion was 11 g/day and serum albumin was 1.4 g/dl , hence nephrotic syndrome was diagnosed. Eleven days after delivery, a renal biopsy showed focal segmental lesions with glomerular epithelial cell injury. She was given 50 mg/day prednisolone and after a month, her 24-hour urinary protein excretion decreased to 2 g/day. One year later, she achieved complete remission. Although she had a relapse of nephrotic syndrome after twenty-one months, steroid therapy again achieved a good response.

Differences in cardiovascular mortality rates among hemodialysis patients in the United States and Japan: the importance of background cardiovascular mortality.

Mortality rates among hemodialysis patients differ greatly among the United States, Europe, and Japan and it has been hypothesized that this is mainly due to differences in practice patterns. Results from the international DOPPS study, however, indicate that differences in practice patterns among the United States, Japan, and Europe are small and not alone explanatory for the differences in mortality rates. Ethnic variability in predisposition to atherosclerotic cardiovascular disease in the general population may lead to significant differences in background cardiovascular mortality in the United States, Japan, and Europe. It is our hypothesis that cardiovascular mortality in dialysis patients is to a great extent dependent on cardiovascular background mortality of the general population. We are currently studying the relationship between all-cause and cardiovascular death rates in countries worldwide using the WHO database. Preliminary data from 35 countries show that all-cause and cardiovascular death rates differ significantly among regions, with Eastern European countries reporting four- to sevenfold higher death rates than Asian countries. A strong linear relationship between cardiovascular and all-cause death rates is observed among these countries. The next step of our study will be to compare country-specific cardiovascular death rates of dialysis populations with those of the respective general populations. Ethnic differences in cardiovascular morbidity and mortality may be explained by genetic variability based upon polymorphism of genes involved in the pathogenesis of atherosclerosis and myocardial infarction.

[Henoch-Schönlein purpura nephritis in a patient infected with the human immunodeficiency virus].

There are various forms of renal lesions in patients with human immunodeficiency virus(HIV), however reported cases of immune-complex glomerulonephritis are scarce. Here we describe an HIV-positive patient with Henoch-Schönlein purpura nephritis(HSPN), which presented as nephrotic syndrome. In addition to therapy combined with glucocorticosteroid and inhibition of the renin-angiotesin system(RAS), plasmapheresis and antiretroviral therapy produced a favorable outcome. A 26-year-old HIV positive man was admitted for purpura on both lower limbs. Despite glucocorticosteroid treatment, purpura recurred and urinary protein increased to 5-10 g daily. HSPN was diagnosed based on the skin and renal biopsies. During 2 months of treatment with combined glucocorticosteroid and RAS inhibition, nephrotic syndrome persisted. He received double filtration plasmapheresis(DFPP). Soon after, urine protein decreased to 2-3 g daily and macrohematuria decreased. The second renal biopsy showed a decrease in IgA deposition and improvement of acute inflammatory changes. In addition, highly active antiretroviral therapy was started to reduce the high viral load. After 3 weeks, HIV-1-RNA rapidly decreased and urine protein decreased to 1 g daily. After a year, urinary protein was negative, but mild microhematuria persisted. We speculate that the refractory nephrotic syndrome in this patient might be associated with the abnormal immunological condition due to HIV infection.

Nutritional management of dialysis patients: balancing among nutrient intake, dialysis dose, and nutritional status.

BACKGROUND: Recommended protein intake for dialysis patients is much higher compared with allowance for normal adults. The authors tried to make a consideration on balancing among nutrient intake, dialysis dose, and nutritional status in maintenance dialysis patients. METHODS: In 57 patients, 13 on hemodialysis (HD) and 44 on continuous ambulatory peritoneal dialysis (CAPD), serum urea nitrogen (SUN), albumin, weekly creatinine clearance of CAPD, and body protein mass by multifrequency bioelectrical impedance analysis were measured. Energy intake was examined from the patients' food diary by a registered dietitian, and protein intake was estimated by both the patients' food diary and calculation of protein equivalent nitrogen appearance. RESULTS: In HD patients, predialysis SUN levels significantly correlated with the amounts of protein intake (r = 0.893; P < 0.001), and there was no correlation between post- and next predialysis SUN levels. In CAPD patients, SUN levels significantly correlated with the amounts of protein intake (r = 0.645; P < 0.001), and there was no correlation between SUN levels and weekly Ccr. The amounts of protein intake were significantly correlated with body protein mass (r = 0.365; P = 0.014), and there were significantly positive relationships between the amounts of protein intake and energy intake in both HD (r = 0.798; P < 0.001) and CAPD patients (r = 0.631; P = 0.006). CONCLUSION: Whereas higher intake of protein requires higher doses of dialysis, lower intake of protein with sufficient energy intake requires lower doses of dialysis, and both could give the same effects on nutritional status.

[Fluctuation of the rate of renal function decline associated with fluctuation of compliance with a low protein diet in patients with diabetic renal failure].

We investigated fluctuation of compliance with a low protein diet and the influence on the rate of decline in renal function. Twenty-seven patients with diabetic renal failure who were prescribed a low protein diet of 0.6 g/kg/day were followed during a period of 12 months. Dietary compliance was evaluated based on a 4-day dietary diary, interview with patients and calculation of the protein catabolic rate from 24-hour urea excretion at every hospital visit. They were judged on a 4-rank system, A(adhered over 75% of the days), B(74-50%), C(49-25%), D(less than 24%). During the twelve months, 55.5% of the patients showed fluctuation of their compliance with the diet. In 17 patients whose compliance fluctuated between rank A and B, their rate of GFR decline(ml/min/month) was significantly faster(-2.40 +/- 2.59 vs 0.99 +/- 1.41, p < 0.01), their rate of serum creatinine elevation(mg/dl/month) was significantly higher(0.90 +/- 0.79 vs -0.42 +/- 0.45, p < 0.01) and their rate of serum urea nitrogen increase(mg/dl/month) was significantly larger(15.3 +/- 12.4 vs -10.0 +/- 12.9, p < 0.01) during the period of rank B than rank A. There were no significant differences in the rates of changes in serum levels of albumin, transffering, HbA1c and body weight between the two periods. In conclusion, fluctuation of compliance with a low protein diet affects the rate of renal function decline synchronizedly in patients with diabetic renal failure.