The long－term management of cystic kidney disease in children / 中华实用儿科临床杂志

Cystic kidney disease in children is a group of kidney diseases with single or multiple cysts in the kidney that are caused by different reasons. According to the etiologies,cystic kidney disease can be divided into genetic or non_genetic type,and few of those are caused by another disease. Cyst in kidney can be isolation,and also can be one of the phenotypes of another syndrome disease. It can occur in infancy,childhood or adulthood,or both of them. The therapies are different because of the different pathogenesis and pathology,and also because of the changing clinical fea_ture in different age stage. These diseases should be detected,diagnosed and treated early,and it is absolutely important to make a systematic long_term management of these diseases.

Progress of diagnosis and treatment for cystic kidney disease in children / 中华实用儿科临床杂志

Cystic kidney disease is the main disease of cystic kidney change in children. It may be caused by non_genetic fetal malformations or genetic diseases,or may be acquired rarely. Most renal cysts are usually isolated oraspart of a syndrome. However,fatal renal cystic diseases can develop from these space occupying lesions. Although renalcystic diseases are similar in presentation,they possess distinct features and variable prognosis later in life. In order to correctly diagnose this kind of disease in the early stage,it claim to accurately grasp its pathogenesis,pathology,clini_cal characteristics and radiological findings. A comprehensive analysis of common cystic kidney disease in children is carried out to help clinicians to aid in early distinction and appropriate treatment.

Ultrasonographic findings of common cystic kidney disease in children / 中华实用儿科临床杂志

Cystic kidney disease is a major disease which can cause kidney cystic change in children. Cystic kidney disease refers to a series of congenital or acquired diseases with one or multiple cysts in the kidney due to different causes. With the development and wide application of ultrasound technology,it is better than CT and magnetic resonance imaging in reflecting the renal cystic disease,and more and more recognized by pediatricians. Now,the ultrasonographic findings of common cystic kidney disease in children were summarized and analyzed,in order to provide help for clinical diagnosis.

Cystic kidney disease in children / 中华实用儿科临床杂志

Both the incidence and prevalence of chronic kidney disease are rising rapidly all over the world, which brings heavy economic and mental burden to the patients and the society. A retrospective study of the European Renal Association _ European Dialysis and Transplant Association( ERA _ EDTA) Registry showed cystic kidney disease was one of the three most prevalent rare diseases leading to renal replacement therapy before the age of 20 years. Renal cystic disease encompasses a variety of diseases that cause single or multiple cysts in the kidneys,and can be divided into 3 categories:cystic kidney disease due to fetal renal malformations,hereditary cystic kidney diseases and acquired cystic kidney diseases. Acquired renal cysts rarely occur in the general pediatric population. Renal cystic disease can present during fetus,infancy,childhood or adulthood. The onset of the disease is occult,and the clinical manifestations are heterogeneous. The manifestations may be confined to the kidney or as part of a syndrome. On renal imaging,the kidney size is normal,enlarged or small,and cysts can involve unilateral or bilateral kidneys. Pamily history investigation should be performed when collecting first_hand clinical data. Genetic testing can confirm the diagnosis of hereditary renal cystic disease. Cystic kidney disease is currently treated symptomatically,and patients with end_stage renal disease require renal replacement therapy.

Diagnosis and treatment of cystic kidney disease in children / 国际儿科学杂志

There are four type of Potter typing of cystic kidney disease in children:autosomal recessive polycystic kidney disease,multicystic dysplastic kidney,autosomal dominant polycystic kidney disease and obstructive cystic dysplastic kidney.However,simple cyst of kidney multilocular cystic renal tumors and other genetic syndrome also can make kidney cystic change.Due to the different pathogenesis and pathological basisn,the clinical diagnosis and treatment is also different.The correct diagnosis of these diseases requires chinical classification and determination of the causes of the diseases.This paper will make a review on the common cystic kidney disease in children.

Renal Cell Carcinoma in Kidney Transplant Recipients and Dialysis Patients

PURPOSE: In a group of surgery patients diagnosed with renal cell cancer, those who underwent dialysis were compared with those who received a kidney transplant. MATERIALS AND METHODS: The 43 subjects included in this study were patients who had been undergoing dialysis because of end-stage renal disease or had undergone kidney transplantation. The patients were diagnosed with renal cell carcinoma (RCC) during follow-up and underwent radical nephrectomy from May 1996 to December 2010. Their medical records were retrospectively analyzed as part of the study. RESULTS: In the transplantation group, the renal replacement therapy period averaged 54 months, and the period from transplantation to RCC averaged 119 months (range, 0 to 264 months). In the dialysis group, RCC was observed after an average of 124 months (range, 2 to 228 months) of dialysis, and nephrectomy was then conducted. Acquired cystic kidney disease (ACKD) was found more frequently in the dialysis group, and it had a statistically relevant effect on the occurrence of RCC by comparison with the transplantation group (p<0.01). CONCLUSIONS: Although the incidence rate of ACKD was significantly higher in the dialysis group among patients undergoing surgery for RCC, cancer was found even without ACKD development in some transplant recipients. Considering that the transplant recipients also underwent dialysis, an informative prospective study will be necessary to determine whether other immunosuppressive agents besides ACKD may function as a cancer risk factor.

A Case of Erythrocytosis Improved after Kidney Transplantation / 대한신장학회지

Renal anemia is a common complication of chronic kidney disease and known to be caused by erythropoietin or iron deficiency. However, erythrocytosis in patients on dialysis has rarely been reported and usually associated with renal cell carcinoma, polycythemia vera or acquired cystic kidney disease. Here we report a case of erythrocytosis in an ESRD patient with resolution after kidney transplantation. A 38-year-old man on peritoneal dialysis for 5 years was admitted for kidney transplantation. On admission, blood Hgb and Hct was 19.7 g/dL and 61.4%, respectively. Serum erythropoietin level was 347 mIU/mL. Multiple variable sized cystic lesions were identified on both kidneys without evidence of internal malignancy in abdomen and pelvis CT scan. After kidney transplantation, Hgb was 12.5 g/dL and serum erythropoietin level was 13.1 mIU/mL. Some of renal cysts on CT scan disappeared or decreased in size. This finding suggests that erythrocytosis in this patient can be associated with acquired cystic kidney disease.

Renal masses in end-stage renal disease patients on dialysis / 대한내과학회지

BACKGROUND/AIMS: End-stage renal disease (ESRD) patients are at a higher risk for cancer, especially renal cell carcinoma (RCC). Acquired cystic kidney disease (ACKD) and long duration of dialysis predict RCC in these patients. We explored the cilnical and pathologic characteristics of renal masses and the factors predicting malignant tumors. METHODS: The study examined the medical records of 38 ESRD patients who underwent partial/total nephrectomy due to renal masses diagnosed by abdominal ultrasound and computed tomography at YUHS from January 1995 to December 2009. RESULTS: Renal masses were recorded in 38 patients (21 males) with a mean age of 48.3+/-13.2 years. Twenty patients (52.6%) were on hemodialysis and 18 patients (47.4%) were on peritoneal dialysis, for a mean period of 106.4+/-62.5 months. ACKD was reported in 23 patients (67.6%). Of the 38 renal masses, 23 (60.5%) were diagnosed as malignant tumors and 15 (39.5%) were benign tumors. Clear cell RCC (n=16) and hematoma (n=6) was the predominant respective types. The patients with malignant tumors had a significantly longer duration of dialysis (122.3+/-61.8 vs. 81.1+/-56.7 months, p<0.05) and a higher prevalence of ACKD (87.0 vs. 46.7%, p<0.01) compared to the patients with benign tumors. CONCLUSIONS: The most common renal mass in ESRD patients was RCC. Malignant tumors were significantly associated with a longer duration of dialysis and ACKD. Therefore, early evaluation and treatment of RCC are mandatory in long-term dialysis patients with ACKD.

Spontaneous Bilateral Renal Rupture in an End-stage Renal Disease Patient Treated with Hemodialysis / 대한신장학회지

Bilateral spontaneous renal rupture is a rare event that has been documented in only a few reports. We report a spontaneous, but not simultaneous rupture of both kidneys in a 64-year-old man with end-stage renal disease who had been treated with hemodialysis for 78 months. He complained of sudden left flank pain. Abdominal computed tomography (CT) revealed a huge perirenal hematoma with multiple renal cysts. The patient underwent an urgent left nephrectomy and was diagnosed as multiple acquired renal cysts with rupture and focal incidental papillary renal cell carcinoma. Twenty-two days after the left nephrectomy, he complained of sudden right flank pain and abdominal CT showed another massive perirenal hematoma. The patient underwent a right nephrectomy and was diagnosed as renal cyst rupture with perirenal hemorrhage. However, there was no evidence of renal cell carcinoma in the right kidney. Postural hypotension and frequent hypotensive episodes developed during hemodialysis several months after both nephrectomies. He was diagnosed as primary adrenal insufficiency with rapid adrenocorticotropic hormone (ACTH) stimulation test and the level of plasma ACTH and was treated with prednisolone and fludrocortisone. The patient died of acute myocardial infarction 32 months after bilateral nephrectomies.

Renal cell carcinoma developed in a patient treated with huge simple renal cyst sclerotherapy / 대한내과학회지

Simple renal cysts are a common cystic disease of the kidneys, which is not symptomatic in most cases and is diagnosed by radiological examination. However, if the cyst is huge or symptomatic, it must be treated. Renal cyst aspiration and alcohol sclerotherapy is a safe and effective treatment for symptomatic simple renal cysts. Simple renal cysts have benign clinical features in the main and transformation of a simple renal cyst into renal cell carcinoma has rarely been reported. However, one case of renal cell carcinoma during renal cyst follow-up has been reported. We report a case of renal cell carcinoma that developed in a patient who was being treated with huge simple renal cyst sclerotherapy.

Renal Cell Carcinoma Occurred in Native Kidneys before and after Renal Transplantation / 대한신장학회지

One of the major risk factors of renal cell carcinoma is acquired cystic kidney disease (ACKD) developed in course of end-stage renal failure (ESRF) and the incidence of ACKD increases with the duration of dialysis. Moreover, the overall incidence of renal cell carcinoma after renal transplantation has been reported to be being more than 8 times higher compared with that of general population. But renal cell carcinomas occurred in native kidneys before and after renal transplantation in ESRF patient with ACKD are very rare. We experienced a case of renal cell carcinoma occurred in native kidneys 14 months before and 5 months after renal transplantation in an ESRF patient with ACKD.

Spontaneous Rupture of Native Kidney in a Patient Receiving Kidney Transplantation : A case report / 대한마취과학회지

Spontaneous rupture of a kidney can occur as a complication of an acquired cystic kidney lesion. Herein, one case of a spontaneous rupture of a contralateral native kidney, due to the rupture of a cystic lesion during kidney transplantation surgery, is described. The patient was a 24 year old female admitted for left kidney transplantation due to chronic renal failure. During the operation, her arterial blood pressure and central venous pressure gradually decreased after anastomosis of the renal vessel, but without significant bleeding in the operative field or any suspected cause of the decreasing arterial blood pressure. On the first postoperative day, she underwent an abdominal CT, where a retroperitoneal hematoma was found. Therefore, a right nephrectomy was performed. She also had a right perirenal hematoma due to the rupture of cystic lesion in the native kidney. While a spontaneous rupture of the kidney is very rare, especially during the perioperative period, it is important to consider this complication when evaluating the corresponding clinical picture.

Comparison of acquired cystic kidney disease between hemodialysis and continuous ambulatory peritoneal dialysis

OBJECTIVES: ACKD has been described mainly in patients treated with hemodialysis(HD), and there are only a few reports about the prevalence of ACKD in continuous ambulatory peritoneal dialysis (CAPD) patients. Therefore, we compared the prevalence of ACKD in patients receiving HD and CAPD, and evaluated the possible factors which may affect the development of ACKD. METHODS: Forty nine HD and 49 CAPD patients who had received dialysis therapy for at least 12 months were enrolled in this cross-sectional study. Patients who had a past history of polycystic kidney disease and had acquired cystic kidney disease on predialysis sonographic exam were excluded. Detection of ACKD was made by ultrasonography and ACKD was defined as 3 or more cysts in each kidney. RESULTS: The prevalence of ACKD was about 31+ACU- (30/98) and there was no significant difference between HD and CAPD patients(27+ACU- vs. 34+ACU-, p +AD4- 0.05). The prevalence of ACKD was not associated with age, sex, primary renal disease, the levels of hemoglobin, BUN, and serum creatinine. However, the duration of dialysis was significantly related to the development of ACKD (presence of ACKD, 74.4 42.4 months vs. absence of ACKD, 37.8 24.1 months, p +ADw- 0.05). CONCLUSION: The prevalence of ACKD is not different according to the mode of dialysis, and the major determinant of acquired cyst formation is duration of dialysis.

Acquired Cystic Kidney Disease in Patients Undergoing Long-term Hemodialysis Treatment / 대한비뇨기과학회지

PURPOSE: The present study was designed to evaluate the incidence and characteristics of acquired cystic kidney disease (ACKD) in patients with end-stage renal disease who were treated by chronic hemodialysis, using renal ultrasonography. MATERIALS AND METHODS: We performed renal ultrasonography as a screening modality in 112 patients treated with long-term hemodialysis due to end-stage renal disease. We classified the patients years from one to six years. RESULTS: In our study, cystic lesions were noted in 41 patients (36.6%) and there was no sexual predilection of ACKD. Also, a prolonged period of hemodialysis increased the incidence of ACKD, especially for those treated more than 49 months. We encountered a case of renal cell cancer, evaluated it with CT, and then treated it by radical nephrectomy. CONCLUSIONS: With these results, renal ultrasonography can be used as a screening modality in patients treated with long-term hemodialysis more than 49 months due to end-stage renal disease. As complications occur, further evaluation will be needed.

Cystic Kidney Diseases According to Potter's Classification

The cystic disease of the kidney include a heterogeneous group of developmental, hereditary, and acquired disorders. Based on extensive microdissection studies, Potter concluded all renal cystic diseases could be categorized into four types. We have experienced 5 cases of cystic kidney disease which were confirmed by aoutopsy and classified as Type I, Type II, Type III, Boderline between types II and III and Type IV according to Potter's classification. We report these cases with a review of literatures.