Purpose : Alport syndrome is a hereditary nephrotic disease characterized by progressive nephrotic symptom, sensorineural hearing loss, ophthalmic abnormality, typical microscopic findings, and familial occurrence. In this study, we tried to find the risk factors related with its prognosis by taking a close observation on clinical symptoms of children with Alport syndrome reviewing retrospectively. Materials & methods : We chose children diagnosed as Alport syndrome in renal biopsy during 20 years(from 1980, Jan. until 1999, Dec.) who could receive follow up studies in tile department of pediatrics. They were divided into two groups by comparing renal function at the time of diagnosis and at current status. We compared several clinical aspects in them, and applied nonparametric test for statistical analysis. Results : The sex ratio(male:female) of 24 children was 3:1. The most common clinical symptom presented at their first visit was gross hematuria. Among those 24 children, 11 cases($46\%$) of progressing into chronic renal failure(Group II) were observed. Hypertension, proteinuria and edema were seen much frequently in group II. The level of serum protein, albumin, and creatinine clearance were decreased while BUN, creatinine were relatively increased. All the results were statistically significant. Conclusion Clinically significant risk factors related to prognosis in Alport syndrome were the presence of hypertension, edema, and proteinuria at the time of diagnosis. Also, the level of serum protein, albumin, BUN, creatinine, and glomerular filtration rate were proved to be important factors in predicting prognosis. We believe that studies on these possible risk factors would be of great help in treating and predicting prognosis of children suffering with Alport syndrome. (J Korean Soc Pediatr Nephrol 2001;5 : 164-75)
Park Jae-Hyun;Kim Pyung-Kil;Jeong Hyeon-Joo;Choi In-Joon
Childhood Kidney Diseases
/
v.1
no.1
/
pp.17-23
/
1997
After the first description of IgA nephropathy by Berger in 1968, the prognosis of this disease was considered favourable. However recent studies have revealed that IgA nephropathy result in end stage renal desease in 25-30% by 20 years. Heavy proteinuria, hypertension, histological high class are regarded as poor prognostic factors. In 1996, Yagame et al reported the new histopathologic grading with a strong correlation between the grading, heavy proteinuria, high s-Cr level and renal survival. The aims of this study are to determine whether the pathological grading and other clinical parameters could contribute to predicting the outcome of this disease eventhough pediatric patients. Seventy nine patients (59 males, 20 females) with IgA nephropathy were examined. Patients were 2.08-15.17 years of age ($9.85{\pm}2.83$). The mean follow-up duration were $27{\pm}28$ months. Six of seventy nine patients progressed to chronic renal failure during the follow-up periods. High 24h urinary protein excretion at diagnosis were significantly higher in chronic renal failure patients (p<0.05). Hypertension at diagnosis were the significant associated factors in progression of chronic renal failure (p<0.05). Histological changes of IgA nephropathy in light microscopy were classified into five classes by WHO classification, four grades in Yagame's gradings. Among the seventy nine patients, 24 were as class 1, 30 as class 2, 23 as class 3; 4 as class 4, 0 as class 5 by WHO classification. 23 were classified grade 1, 31 as grade 2, 24 as grade 3, 1 as grade 4 by Yagame's grading. Among six patients who progressed to chronic renal failure, 1 clssified as class 1, 1 as class 2, 3 as class 3, 1 as class 4, 0 as class 5 by WHO Classification. 1 patients were classified as grade 1, 1 as grade 2, 3 as grade 3, 1 as grade 4 by Yagame's grading. (p>0.05) In conclusion, hypertension and heavy proteinuria at initial presentation were significantly associated with progression of chronic renal failure. The classification of WHO & Yagame's grading has no significant association with the progression of chronic renal failure in pediatric patients.
Kim Jung-Sue;Song Jung-Han;Park Hye-Won;Cheong Hae-Il;Kim Jin-Q;Choi Yong;Ko Kwang-Wook
Childhood Kidney Diseases
/
v.1
no.2
/
pp.109-116
/
1997
Purpose : Chronic renal failure is often accompanied by severe dyslipidemia, a known risk factor for cardiovascular disease. Lipoprotein(a) [Lp(a)] has recently been characterized as a risk factor for atherosclerosis and thrombosis. Cardiovascular disease is the leading cause of death in adult patients on dialysis. However, there are only limited data available concerning risk factors for atherosclerosis in uremic children. We have measured serum levels of lipids, lipoproteins, apolipoproteins and Lp(a) in uremic children with maintenance dialysis. Methods : Ten uremic children with hemodialysis (HD) and 14 with peritoneal dialysis (PD) in our dialysis unit were included in this study. The mean age of HD patients was $162{\pm}59$ months and the male to female ratio was 7:3. The mean age and sex ratio of PD patients were $123{\pm}63$ months and 6:8, respectively. The levels of cholesterol, triglyceride, lipoproteins, apolipoproteins and Lp(a) were measured from serum sampled after 14 hours of fasting. The normal control levels were cited from 2 articles presenting the normal blood lipid and lipoprotein levels of primary school and middle school children in Korea. Results : There was no difference in age, sex ratio, body mass index and duration of dialysis between the HD and the PD group. The serum concentration of the cholesterol, triglyceride and apolipoprotein B were significantly elevated in dialysis patients compared with normal subjects. The serum level of Lp(a) was significantly elevated in only PD group. The serum Lp(a) level was below 30 mg/dl in 13 and above 30 mg/dl in 11 patients. The serum albumin level was significantly decreased in high Lp(a) group than in low Lp(a) group. Conclusion : The uremic children receiving dialysis reveal abnormal serum lipid and lipoprotein profiles. These results suggest that they have a higher risk for coronary heart disease, although there has been no clinical evidence of coronary heart disease at present. A long-term follow-up study of these children to clarify the suggestion should be started now.
The patient with end-stage renal disease show several nervous complications. The factors contributing to the nervous complications are still incompletely characterized. Cyanate, known as one of the uremic toxins, is derived spontaneously from urea. To investigate the mechanism of cyanate-induced effect on C6 glioma cells, the glioma cells were treated with 0, 1, 5, 10, 20 and 40 mM cyanate. There was a dose-dependent decrease in cell viability and the decreased number of cell was observed in glioma cells by treatment with cyanate. Western blot showed the down- regulation of procaspase-3, which means up-regulation of caspase-3, and the up-regulation of caspase-8, but the down-regulation by cyanate. In addition, cDNA microarray showed 934 down-regulated genes and 165 up-regulated genes on 1,099 genes in cyanate treated group. Treatment with cyanate led to 16 down-regulated genes and 6 up-regulated genes on apoptosis category, and especially heat shock 70 kD protein 1A gene on the category of apoptosis was significantly up-regulated. These results suggest that cyanate can induce apoptosis through caspase-8 and caspase-3 in glioma cells and decrease of gene expression including apoptosis category in glioma cells. These effects of cyanate may play a role in the nervous complications of patient with end-stage renal disease.
Purpose : To find and solve the common problems of peritoneal dialysis(PD) by analysing the clinical data of pediatric PD performed in Korea. Methods : 264 cases of CAPD and acute PD had been performed from Nov.1987 to Oct. 1997 in 17 institutions of pediatric nephrology in Korea. Results : CAPD was performed in 114 cases. The mean age of the patients was $10.5{\pm}6.6$ years and male to female ratio was 1.4:1. The original renal diseases of ESRD were proven in 92 cases($80\%$). The common renal disease of ESRD were FSGS($17\%$), reflux nephropathy ($11\%$), chronic glomerulonephritis($9.6\%$). Mean duration of CAPD was $20{\pm}16.9$ months. Peritonitis was the most common complication and incidence was one episode/18.2 patient-months. Other complications were exit site infection in 10 cases, obstruction in 7 cases, leakage of dialysate in 6 cases. The most common etiologic organism of peritonitis was staphylococcus aureus and the next was staphylococcus coagulase(-). Acute PD was performed in 150 cases. Most common underlying causes were congenital heart disease, hemolytic uremic syndrome, sepsis and dehydration. The mean duration was $10.3{\pm}11.3$ days. The most common complication was peritonitis($20.6\%$). The most common etiologic organism was staphylococcus aureus and coagulase(-), acinetobactor and pseudomonas. Conclusion : Reflux nephropathy should be emphasized in early diagnosis and treatment to prevent ESRD. Incidence of congenital anomaly($7\%$) as a original disease of ESRD was relatively low in Korea. Growth status was not significantly improved after CAPD. In acute PD, the incidence of peritonitis was rapidly increased at 2weeks after beginning of dialysis.
Park, Chang-Joo;Park, Jong-Chul;Kang, Young-Ho;Myoung, Hoon;Lee, Jong-Ho;Kim, Myung-Jin;Kim, Hyun-Jeong;Yum, Kwang-Won
Journal of The Korean Dental Society of Anesthesiology
/
v.3
no.2
s.5
/
pp.98-102
/
2003
Patients in end-stage renal disease (ESRD) and chronic renal failure present a number of challenges to the anesthesiologist. They may be chronically iii and debilitated and have the potential for multiorgan dysfunction. A 65-year-old male patient with ESRD was scheduled for oral cancer surgery under general anesthesia. He was in regular hemodialysis three times a week and secondary hypertension with left ventricular hypertrophy was accompanied. He also had chronic metabolic acidosis and hyperkalemia. The day after hemodialysis, general anesthesia was carried out. Uneventful anesthetic induction using thiopental and vecuronium and nasotracheal intubation were carried out. General anesthesia was maintained with isoflurane for 9 hours. During the anesthesia, he did not have any problem but persistently increasing serum potassium level. After anesthetic emergence, he was transferred to intensive care unit for mechanical ventilation. So we report this successful case of anesthetic management in a patient with ESRD for oral cancer surgery, which massive bleeding and long anesthetic time were inevitable in, from the preoperative preparation to anesthetic emergence.
Wegener's granulomatosis(WG) is a necrotizing granulomatous small vessel vasculitis with a clinical predilection for involvement of the upper airways, lungs and kidneys. The disease usually manifests in adults between 25 and 50 years of age, but it can also rarely occur-in childhood with some features different from those of adults. WG may be easily overlooked in young patients by misinterpretion of the symptoms as caused by an infectious disease of the respiratory tract. Delayed diagnosis and treatment of the disease may cause more rapid progression of the glomerulonephritis to end stage renal disease. We report a boy who was diagnosed with WG with involvement of multiple organs at 13 years of age.
This study was attempted to understand the phenomenon in depth by exploring the food craving of hemodialysis patients experienced during hemodialysis. Participants selected seven adult patients diagnosed with end-stage renal failure and receiving hemodialysis treatment from medical institutions. he collected data were analyzed using a phenomenological analysis method. As a result of the analysis the food craving of hemodialysis patients was derived into two categories included craving experience in the cognitive domain craving experience in the emotional domain and five themes: 'Looking for foods that are water and watery as a result of thirst','Food thoughts come to mind all day as a result of a limited diet', 'Always hungry', 'Faced with appetizing situations every hour', 'experiencing negative emotions due to failure to control meals'. The results of this study which sheds light on food craving from the perspective of hemodialysis patients should improve the understanding of hemodialysis patients appetite control water restrictions and dietary compliance and allow them to understand the food craving attributes of hemodialysis patients and provide customized education optimized for that extent when applying dietary education and nursing interventions suitable for them.
A 3-year-old male Maltese dog(weighing 2.5 kg) was referred with an excessive movement of mandible and depression. Comprehensive diagnostic investigation revealed an end-stage of renal disease with secondary hyperparathyroidism. The renal allograft was performed after the condition of dog was stabilized by hemodialysis and medical treatment. After transplantation, the renal function of this dog was returned to normal. thereafter, the dog died suddenly without apparent clinical signs. The subsequent pathological studies revealed the actual etiology of death was not clearly identified However acute pancreatitis caused by abrupt introduction of food after prolonged luting might be involved in the etiology. This case study showed the necessity of pancreatic function test in postoperative management after renal transplantation.
Purpose: The objectives of this study were to investigate the current status of the pain in and the dysfunction related to the musculoskeletal system experienced by patients with end-stage renal failure receiving hemodialysis. Methods: A questionnaire survey was given to 107 patients undergoing hemodialysis in M Hospital. The survey was conducted by using a brief pain inventory(BPI) to investigate the body parts in pain, degree of pain, difficulty in daily life, correlation with hemodialysis and the degree of satisfaction with their own health. Results: The investigation of the patients undergoing hemodialysis showed that 89(83.2%) of the 107 patients experienced pain in their musculoskeletal system. Their pain's incidence was highest in the knees(46.1%) and 68.5% of the patients reported that the most severe pain they had experienced within the past 24 hours was at a level between "moderate" and "severe." The limitation of daily life by pain is high most as 47.7% in the accomplishment of works. Conclusion: Most hemodialysis patients experience musculoskeletal related pain that affects their daily life. Hemodialysis patient management programs should include an assessment and management of such pain.
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