Antibody response to influenza A vaccination in children with nephrotic syndrome

The aim of this study was to determine the antibody response to influenza vaccination in children with nephrotic syndrome (NS). Nineteen children with NS and 10 healthy controls were vaccinated with a 1999–2000 influenza vaccine. A dose of 0.25 ml was used for those under 6 years and 0.5 ml for those over 6 years. All children were given two doses with a month between each dose. Antibody titers were measured before vaccination and 1 month after vaccination in both groups and 6 months after vaccination in 8 patients with NS. The proportion of subjects in the nephrotic group with protective antibody titers before immunization (10.5%) was significantly lower than the proportion at 1 (78.9%) and 6 months (87.5%) post vaccination. The mean concentration of specific IgG antibodies to influenza A in the NS group increased 6-fold at 1 month and approximately 14-fold at 6 months. These results suggest that pediatric patients with NS have an adequate antibody response to influenza A vaccine. Protective antibody titers to influenza A were maintained at 6 months after immunization in 8 patients with NS.     

Factors influencing the response to growth hormone in children with renal disease

The effects of age, height velocity over the preceding year, glomerular filtration rate (GFR) and prednisolone dose on growth response have been assessed by single and multiple linear regression analysis in 23 prepubertal children [age, mean (SD), 8.2 (2.5) years] with chronic renal failure (CRF) and 16 prepubertal children [12.1 (2.3) years] with renal transplants treated for 1 year with recombinant human growth hormone (rhGH), 30 U/m² per week. Height velocity [mean (SD), cm/year increased from 4.7 (1.3) to 9.7 (2.1) (P<0.0001) in the CRF group and 3.1 (1.6) to 7.3 (2.8) (P<0.0001) in the transplant group. In the CRF group, there was a correlation between age and height velocity, both in the pretreatment year (r=–0.755,P<0.0001) and during treatment (r=–0.421,P=0.045). There was no correlation between pretreatment height velocity or GFR and response to rhGH. In the transplanted children height velocity during the treatment year correlated with age (r=–0.647,P=0.007), prednisolone dose (r=–0.689,P=0.003), GFR (r=0.542,P=0.030) and pretreatment height velocity (r=0.655,P=0.006). Multiple regression analysis showed prednisolone dose and age to be the most important predictors of response.     

Towards guidelines for dialysis in children with end-stage renal disease

There are few data describing the current practices of treatment selection for children with end-stage renal disease (ESRD). In an effort to establish a consensus among Spanish pediatric nephrologists for inclusion and exclusion criteria for renal replacement therapy in children with ESRD, in 1995 we surveyed members of the Spanish Pediatric Nephrology Association. Although only 43% of members responded, pediatric nephrologists and bioethicists studied the results and compiled a list of ten guidelines for treatment of children with ESRD. The proposed guidelines are meant to be a starting point for further discussion. An emphasis on flexibility, individual case assessment, and consideration of the best interests of the patient must remain central to any treatment plan. Decision making should ideally be shared by parents, professionals, the child, when appropriate, and ethics committees, as necessary. Received: 9 February 1999 / Revised: 21 June 1999 / Accepted: 28 June 1999     

The dilemmas of patient treatment for end-stage renal disease

In past years, physicians responsible for the treatment of chronic uremia have faced dilemmas that have been methodologic and economic while attempting to provide good patient care. These have been overcome, but in the course of time a larger one has developed. The current dilemma is one of high costs for end-stage renal disease (ESRD) management and the failure of current treatment programs to adequately rehabilitate the ESRD patient. In spite of widespread concern about this dilemma, few current data and even fewer projections exist about the eventual costs for their care. Existing data demonstrate several problems that are the basis of this dilemma: (1) the projections of incidence and prevalence of ESRD patients have been too low; (2) renal transplantation has failed to develop into a dominant (and least costly) form of ESRD therapy; (3) home dialysis programs have failed to offset the rapidly expanding in-center dialysis population; and (4) prevalence of and costs for chronic hemodialysis have increased far beyond expected levels. Using current data for the US population as to the incidence and overall mortality rate of ESRD patients, it is apparent that the dialysis population is only 39% of the way toward a steady state-corresponding to only the 4th year of a calculated 25-year growth curve. Although the current costs for maintenance of ESRD patients exceeds $1.3 billion, based upon such projections with the current distribution of patient treatment modalities, the overall annual cost will be in excess of $3.3 billion before a steady state is achieved. Improvement in mortality rates or increases in the incidence of patients will increase the steady state prevalence and the overall costs. Renal transplantation, unless kidney survival rate is increased so that it approximates patient survival, is unlikely to offset the rapidly increasing costs. New technology that would reduce the costs for center-based chronic hemodialysis has not been identified. Emphasis upon home dialysis modalities as a method of increasing patient rehabilitation and reducing costs appears to be a short-term necessity. Increased research and development in prevention of ESRD and in achieving better transplant kidney survival appear to be extremely important as long-term goals.     

老年终末期肾病患者的透析方式选择

随着人口老龄化加剧,在全球范围内需要接受肾脏替代治疗的老年终末期肾脏病(ESRD)患者人数日益增加。老年ESRD患者由于年龄老化、生理机能下降、合并症多、自我维护能力差、预期生存时间短等特点给透析治疗亦带来了诸多困难。老年ESRD患者在透析的治疗时机、透析方式的选择上均具有其特殊性,需要根据患者原发病因、评估透析治疗可能带来的并发症、所在地医疗卫生条件等制定个体化的治疗方案。除了医疗技术因素外,还需综合考虑如患者预期寿命、对生活质量的期望以及对患者家庭的影响等其他非医疗因素。     

Quality of life in children with end-stage renal disease undergoing renal replacement therapy: a cross-sectional survey

Objective To evaluate the quality of life (QOL) of children with uremia who underwent renal replacement therapy (RRT) and identify the influencing factors for QOL in order to improve the QOL of children with uremia. Methods Children with ESRD who underwent dialysis or kidney transplantation (KT) at Children's Hospital of Fudan University between November 2016 and October 2017 were enrolled. The children and/or their parents completed and returned the Pediatric QOL Inventory Measurement Models (PedsQLTM) 4.0 questionnaire. Moreover, the clinical data of these children were collected. According to the way of RRT, children were divided into dialysis group and KT group. The differences of scores between two groups were compared. Multiple linear regression analysis was used to analyze the factors affecting the QOL of children. Results A total of 79 children undergoing RRT were enrolled. Among them, 48 cases in the dialysis group and 31 cases in the KT group. For children in KT group, the total PedsQL scores of child-self and parent-proxy assessment were higher than those in dialysis group (P＜0.05). The total scores for the QOL of child-self and parent-proxy assessment were roughly the same for KT children (P＞0.05). The total scores for the QOL of child-self and parent-proxy assessment were different for dialysis children (P=0.05). Short stature (height＜3th percentile) and elevated left ventricular mass index (LVMI) were the independent influencing factors for the QOL of child-self and parent-proxy assessment in children undergoing KT, respectively (B=12.162, t=2.681, P＜0.05; B=-0.240, t=-4.276, P＜0.01). Conclusions QOL was higher in children undergoing KT than those on dialysis. Short stature and elevated LVMI were the independent influencing factors for QOL in children undergoing KT.     

Limitations of the pediatric risk of mortality score in assessing children with acute renal failure

The pediatric risk of mortality score (PRISM) incorporates 14 physiological and laboratory variables to calculate a patient's score, which is then adjusted for operative status and age to determine the probability of death. Because of the ethical issues surrounding the initiation of dialysis in critically ill children, a scoring system which could differentiate survivors from nonsurvivors prior to the initiation of dialysis would be useful to the clinician. Similarly, a score which could accurately estimate the probability of mortality in children with acute renal failure would be useful to third party payors attempting to evaluate the performance of individual care providers. We calculated PRISM scores on the day dialysis was initiated, retrospectively, in 31 children seen from 1984–1988 with the diagnosis of acute renal failure and requiring dialysis, in order to determine if the PRISM score was accurate in prediction of mortality. In addition, we calculated scores on the day of admission to the intensive care unit (DICU) in order to see if DICU scores accurately reflected mortality risk. The mean PRISM scores of nonsurvivors were significantly higher than the mean scores of survivors on the day dialysis therapy was initiated. However, overlap in the scores of survivors and nonsurvivors would limit the applicability of PRISM scores for clinical decision making. Children that developed acute renal failure requiring dialysis due to extrarenal diseases had a higher mortality rate than those that had primary renal disease (57% versus 12.5%,P<0.05). DICU scores underestimated the mortality of these patients. The decision to institute dialysis for children with acute renal failure cannot be based on PRISM scores calculated during the hospital course. The use of PRISM scores in quality assurance activities for children with acute renal failure is inappropriate.     

End-stage renal disease secondary to renal malignancy: Epidemiologic trends and survival outcomes

Objectives

Loss of renal parenchyma after surgery may contribute to chronic kidney disease; however, the long-term consequences of chronic kidney disease may differ by cause. We analyzed the outcomes of patients with end-stage renal disease (ESRD) based on various medical and surgical causes.

Screening for Fabry disease in male patients with end-stage renal disease in western France

ContextFabry disease is a rare X-linked genetic disease due to pathogenic variants in the GLA gene. Classic Fabry disease is characterized by glycosphingolipids accumulation in all organs including the kidney, resulting in end-stage renal disease in a subset of male patients. Fabry disease should therefore be considered in the differential diagnosis of patients with unexplained end-stage renal disease.ObjectiveWe performed a prospective screening study in Western France to determine the prevalence of Fabry disease in a large population of dialyzed and transplanted patients.Patients and methodsPatients meeting the inclusion criteria (males, 18-70 years with end-stage renal disease of unknown or vascular origin) were selected from the REIN® registry and the CRISTAL® database. Screening on filter papers was performed after patient consent was obtained during either a dialysis session or a transplantation follow-up visit.ResultsOne thousand five hundred and sixty-one end-stage renal disease male patients were screened and 819 consented (dialysis: n = 242; transplant: n = 577). One single patient was found with decreased alpha-galactosidase levels <25%. GLA sequencing identified the p.Phe113Leu variant in favor of an unknown superimposed kidney disease responsible for end-stage renal disease since this GLA pathogenic variant is associated with a later-onset cardiac form of Fabry disease with minimal kidney involvement. Family cascade genotyping revealed a previously undiagnosed affected brother.ConclusionThe prevalence of Fabry disease in end-stage renal disease patients was 0.12%, questioning the efficacy of this screening strategy with respect to the low prevalence. However, beside the benefit for the patient and his family, the increased awareness of Fabry disease among participating nephrologists may be of interest for future patients.     

Valve replacement surgery in patients with end-stage renal disease: long-term results

BACKGROUND: The life expectancy of patients with chronic renal failure who are dependent on dialysis is very poor. This study was undertaken to determine time-related outcomes in dialysis patients requiring cardiac valve replacement. METHODS: From 1994 to 2001, 29 end-stage renal disease (ESRD) patients on hemodialysis (HD) program underwent 30 valve replacement operations: 29 received mechanical valves (97%), and one received bioprosthetic valves. The sites of valve replacement were 11 aortic (36.7%), 18 mitral (60%), and one both aortic and mitral (3.3%). Mean age was 42.46 +/- 14.26 years (range 17-75 years). Follow-up was completed in 28 patients (96.5%). RESULTS: Early postoperative mortality (in the first 30 days) was 3.4% (n = 1). The overall estimated Kaplan-Meier survival was 56.7% at 36 months, 46.7% at 60 months, and 43.3% at 96 months. HD program was discontinued for two patients after renal transplantation in the follow-up period. All patients, except the one with bioprosthesis, used warfarin sodium for anticoagulation and none of them had bleeding. One of the patients had a major cerebrovascular accident (CVA) and another one had a minor CVA at the follow-up (6.7%). CONCLUSIONS: Life quality is better and life expectancy is longer after valve replacement in ESRD patients who have valvular disease. Also, longer life expectancy increases the probability for finding donors for kidney transplantation.     

儿童甲型流感合并川崎病的临床特征

目的分析儿童甲型流感合并川崎病（KD）的临床特征,增强对甲型流感合并川崎病的早期识别。 方法对首都医科大学附属北京地坛医院2018年1月1日至2019年12月31日收治的682例实验室确诊的甲型流感患儿资料进行回顾性分析,筛选甲型流感病毒感染合并川崎病儿童共14例（KD+甲流组）。选择同期就诊的符合纳入标准及排除标准的28例KD患儿为KD组,28例单纯甲型流感患儿为甲流组。比较KD+甲流组和KD组患儿临床表现、发热持续时间及冠状动脉病变发生率。比较KD+甲流组、KD组和甲流组患儿白细胞（WBC）、中性粒细胞百分比、淋巴细胞百分比、血小板、快速C-反应蛋白（CRP）、红细胞沉降率（ESR）、降钙素原（PCT）、血清淀粉样蛋白酶A（SSA）、白蛋白、转氨酶和心肌酶水平。 结果KD+甲流组患儿发热持续时间[（11.21 ± 3.36）d]较KD组[（6.29 ± 2.21）d]更长,差异有统计学意义（t = 5.81、P < 0.001）。KD+甲流组丙种球蛋白无反应型（IVIG）比例（42.9%）较KD组（14.3%）更高,差异有统计学意义（χ² = 4.1、P = 0.41）。KD+甲流组患儿WBC[（13.18 ± 4.39）× 10⁹/L]、ESR[（60.5 ± 15.82）mm/h]、CPR[55.5（17.56,61.0）g/L]、SSA[76.5（23.0,311.6）mg/L]和丙氨酸氨基转移酶（ALT）[49.5（23.1,78.0）U/L]均较KD组和甲流组升高,差异均有统计学意义（F = 27.92、P < 0.001,F = 47.97、P < 0.001,Z = 3.15、P = 0.007,Z = 4.36、P < 0.001,Z = 2.68、P = 0.027）。KD+甲流组较KD组患儿冠状动脉扩张发生概率更高（57.0% vs. 25.0%）,差异有统计学意义（χ² = 4.2、P = 0.041）。KD+甲流组患儿IVIG无反应型发生率较单纯KD组高（42.9% vs. 14.3%）,差异有统计学意义（χ² = 4.1、P = 0.041） 结论甲型流感合并KD患儿发热时间、明确诊断所需时间较长,其IVIG无反应型发生率更高,且血清SSA和ALT水平显著升高。     

Therapeutic camping for children with end-stage renal disease

Therapeutic camping experiences for children with end-stage renal disease (ESRD) have proliferated in the United States and abroad. This report is based on the results of a survey designed to accumulate data on the development and implementation of 20 such camps. Children attending camp ranged in age from 1 year to 19 years. Single disease-specific camps were most common, while camps for children with a variety of chronic illnesses, including ESRD, and mainstream camps were also conducted. Facilities were available for hemodialysis and continuous ambulatory peritoneal dialysis, but not automated peritoneal dialysis, in the majority of surveryed camps. Dialysis nurses, pediatric nephrologists, dietitians and social workers were the medical personnel that most frequently participated in the camps. On average, 32 dialysis/transplant patient campers (range 6–100) attended camp for a 1-week session. Therapeutic camping experiences for children with ESRD are extremely successful and attempts to increase the availability of similar camps should be encouraged.Presented in part at the 13th Annual Conference on Peritoneal Dialysis, 7–9 March 1993, San Diego, California, USA.     

Coronary artery calcifications in children with end-stage renal disease

Coronary artery calcification (CAC) is common in adults with end-stage renal disease (ESRD), but little is known about the prevalence and the extent of it in children. We used multidetector spiral computed tomography (MDCT), echocardiography, and carotid and brachial high-resolution ultrasonography to screen for the presence and predisposing factors of CAC in 53 children with ESRD [15 hemodialysis (HD) patients, 24 peritoneal dialysis (PD) patients, and 14 renal transplant (rTx) recipients]. CAC was present in 15% of patients (three HD patients, three PD patients, and two rTx). The mean age of the patients with CAC was 16.4 years (range: 11.0–21.2 years), and their median CAC score was 101.3, ranging from 8.5 to 4,322 according to the Agatston method. The patients with CAC had longer duration of total dialysis (P=0.005), had higher time-integrated serum phosphorus (P<0.001), calcium-phosphate (CaxP) product (P=0.012), intact parathyroid hormone (P=0.010), vitamin B₁₂ levels (P=0.010), the amount of cumulative calcium-containing oral phosphate binders (OBPs) (P<0.001), and calcitriol intake (P<0.001), and had lower serum hemoglobin level (P=0.014). Interventricular septum systolic thickness (P=0.033) was significantly higher, relative wall thickness (P=0.062) tended to be higher, and flow-mediated endothelium-dependent dilatations (P=0.071) were lower without reaching statistically significant levels in those with CAC. A stepwise logistic regression analysis revealed that serum phosphorus (P=0.018) and the cumulative exposure to calcium-containing OPBs (P=0.016) were the most significant independent predictors in the development of CAC. These results indicate that even adolescents and children with ESRD may have coronary calcifications. We concluded that impaired divalent ion metabolism is the main factor in the formation of CAC in this age group.     

继发性甲状旁腺功能亢进症与终末期肾病轻度认知障碍的关系研究

目的 探讨继发性甲状旁腺功能亢进症(secondary hyperparathyroidism,SHPT)与终末期肾病(end stage renal disease,ESRD)轻度认知障碍(mild cognitive impairment,MCI)发生的关系.方法 选取2018年1月1日至2019年12月31日于保...     

Electrocardiographic monitoring in children with chronic renal failure

Continuous electrocardiographic (ECG) monitoring was performed over 24 h in 44 children at various stages of chronic renal failure in order to determine the incidence and nature of cardiac dysrhythmias. In addition the ECG was followed during haemodialysis sessions and during dialysate exchanges in continuous ambulatory peritoneal dialysis (CAPD) patients. In contrast to adult patients on haemodialysis life-threatening dysrhythmias were not observed. The proportion of children with premature ventricular complexes (41%) was at the upper limit of that in healthy children. A relatively high heart rate was found in children on CAPD, which varied during the exchange procedure. In 57% of all patients a transient marked prolongation of the QT interval up to 40% greater than normal was observed without obvious changes in the serum electrolyte levels. Continuous ECG monitoring is a useful tool for detecting alterations of cardiac rhythm and conduction in at-risk children with renal failure.     

Extrapulmonary tuberculosis in patients with end stage renal disease

Chronic renal failure is a risk factor fortuberculosis. In the past five years we have identifiedtwo cases of tuberculosis in our dialysis population.The first patient, showed chronic failure to thrive onhemodialysis. An enlarged cervical lymph node wasbiopsied and, although no acid fast bacilli (AFB) wereseen, a culture grew Mycobacterium tuberculosum (TB). Her chest X-ray did not show evidence of pasttuberculosis. The second patient was a long timesmoker who presented with an enlarged cervical node,which was biopsied. AFB were not seen on her biopsy,but her culture grew TB. Extrapulmonary TB is commonin patients with ESRD, and lymph node involvement isthe most common extrapulmonary presentation. Screening with the purified protein derivative (PPD)is not helpful in ESRD patients, since defects in cellmediated immunity are common. A high index ofsuspicion for TB is warranted in patients with ESRD. This revised version was published online in August 2006 with corrections to the Cover Date.     

Acquired renal cystic disease in children prior to the start of dialysis

This report describes the clinical course and serial sonographic findings in three children who developed acquired renal cystic disease (ARCD) prior to the institution of dialysis. The children were aged from 3 years to 13 years and their estimated glomerular filtration rate varied from 8 to 13 ml/min per 1.73 m² when ARCD was diagnosed. Their primary renal disorders, which included hemolytic-uremic syndrome and focal segmental glomerulosclerosis, had been present for 1.5–11.5 years prior to the cysts being discovered. These patients show that ARCD may develop in children with chronic progressive renal parenchymal disease prior to the institution of specific therapy for end-stage renal disease.     

Overview: end-stage renal disease in the developing world

Although the vast majority of patients with end-stage renal disease (ESRD) worldwide live in what is called the developing world, little is known about its epidemiology and management. With the current paucity of credible and adequately representative registries, it is justified to resort to innovative means of obtaining information. In this attempt, world-renowned leading nephrologists in 10 developing countries collaborated in filling a 103-item questionnaire addressing epidemiology, etiology, and management of ESRD in their respective countries on the basis of integrating available data from different sources. Through this joint effort, it was possible to identify a number of important trends. These include the expected high prevalence of ESRD, despite the limited access to renal replacement therapy, and the dependence of prevalence on wealth. Glomerulonephritis, rather than diabetes, remains as the main cause of ESRD with significant geographical variations in the prevailing histopathological types. The implementation of different modalities of renal replacement therapy (RRT) is inhibited by the lack of funding, although governments, insurance companies, and donations usually constitute the major sponsors. Hemodialysis is the preferred modality in most countries with the exception of Mexico where chronic ambulatory peritoneal dialysis (CAPD) takes the lead. In several other countries, dialysis is available only for those on the transplant waiting list. Dialysis is associated with a high frequency of complications particularly HBV and HCV infections. Data on HIV are lacking. Aluminum intoxication remains as a major problem in a number of countries. Treatment withdrawal is common for socioeconomic reasons. Transplantation is offered to an average of 4 per million population (pmp). Recipient exclusion criteria are minimal. Donor selection criteria are generally loose regarding tissue typing, remote viral infection, and, in some countries, blood-relation to the recipient in live-donor transplants. Cadaver donors are accepted in many countries participating in this survey. Treatment outcomes with different RRT modalities are, on the average, inferior to the internationally acknowledged standards largely due to infective and cardiovascular complications.     

Acquired renal cystic disease in children and young adults on maintenance dialysis

Acquired renal cystic disease (ARCD) is a well-known complication of end-stage renal disease (ESRD). We studied 24 patients, aged 8 – 27 years (mean 19.8±5.3 years), on chronic maintenance dialysis in our service. The duration of dialysis ranged between 13 and 192 months (mean 77.8±44.3 months). High-resolution ultrasonography revealed ARCD in 11 (45.8%) patients. No cysts were seen in 7 (29.1%) patients and solitary cysts in one or both kidneys were seen in 6 (25%) patients. Renal malignancy was diagnosed in 2 patients. One, 15 years old, had renal cell carcinoma after being on dialysis for 6 years. She did well after bilateral nephrectomy, left salpingo-oophorectomy, and regional lymphadenectomy. The second patient, 23 years old, had been on dialysis for 16 years when she developed renal oncocytoma. She died of congestive cardiomyopathy 6 months later. We conclude that ARCD is common in children and young adults with ESRD. Neoplastic transformation, although rare, is a potential complication. Annual follow-up with ultrasonography with selective use of computed tomography or magnetic resonance imaging is advised. Received July 29, 1996; received in revised form and accepted November 15, 1996