CMU Children's Hospital Pediatric Nephrology
Professor Ching-Yuang Lin
Hsin (alias), a 22-year-old young man, underwent a kidney transplantation right after graduating from university so that he would no longer have to undergo lifelong dialysis. Diagnosed with the end stage of renal disease, he visited the emergency room nearly every month due to some unknown fever since he was born. Up until sixth grade, he was found to have proteinuria and pyuria (higher white blood cell counts). Later, he was admitted to the hospital again because of high fever, which is when the physician suspected that he might have some issue with his kidney. He was found to have hematuria with hydronephrosis and renal atrophy on one side after getting an ultrasound of his kidney. However, treatment was delayed until seventh grade, when he was referred to Prof. Ching-Yuang Lin‘s OPD at the Department of Pediatric Nephrology, CMU Hospital, through a referral from St. Martin de Porres Hospital, and the diagnosis of “congenital anomalies of the urinary tract ‘with progression to develop’ chronic renal disease (CKD)” was confirmed. He had missed the best timing for treatment so he started his 10-year OPD follow-up to control disease progression and slow down the timing of kidney dialysis.
Hsin’s mother brought him to visit Dr. Ching-Yuang Lin‘s OPD of Pediatric Nephrology for help at that time. Dr. Lin had arranged the“ voiding cystourethrography (VCUG)“ and “nuclear medicine scan” to confirm if he had bilateral severe vesicoureteral reflux (VUR) and to assess the severity of his renal fibrosis (renal scar). After the examinations, Hsin was identified to have grade V of VUR on one side and renal atrophy on the other side due to his continuing bilateral VUR, which meant Hsin’s renal functions had progressed to stage IIIb of “chronic renal disease”. For the following 10 years, Dr. Lin continued to follow up Hsin‘s disease progression to slow it down. However, Hsin’s estimated Glomerular filtration rate (eGFR) continued to gradually decline every year (renal dialysis shall be initiated while GFR<5, as shown in Figure 2) so he had to start accepting hemodialysis treatment this past June. Hsin’s mother hated to see Hsin having to undergo renal dialysis three times a week, she decided to share a thought that she had considered for over 10 years – the decision to donate her kidney to her son. Hsin’s mother explained, “He is my son! He’s just become a grownup and is about to join society, I wish to give him a new life！”She actively discussed with Prof. Lin about her thought since she’s the only healthy person without any chronic disease in the family so she was assessed at the OPD of the Urology Department at CMU Hospital for the pre-operation procedure of kidney transplantation. Afterward, the kidney transplantation was successfully implemented by Prof. Chao-Hsiang Chang, and both mother and son were discharged quickly and safely. The completion of the kidney transplantation did not mean the end of treatment, and Hsin still has to be followed up regularly at the OPD in order to track his renal functions and adjust the dosages and class of the immunosuppressants; meanwhile, his mother’s renal function shall also be taken care of to ensure its health as well.
“Chronic renal disease” can be classified into five stages, with the fifth stage being “End Stage Renal Disease (ESRD)”. The prevalence rate of ESRD in Taiwan is number one in the world. Hemodialysis or peritoneal dialysis are renal replacement therapies that can save lives but are unable to resolve the fundamental issues of the disease. Only successful kidney transplantation can successfully treat the disease. Prof. Lin said that the kidney transplantation rate in Taiwan is currently only 4%, and the longest time recorded for a dialysis patient in Japan was about 40 years. Therefore, kidney transplantation for children is the best treatment approach to maintain growth and development, upgrade quality of life, and raise the survival rate. In 1985, Prof. Ching-Yuang Lin started his first kidney transplantation case, and the most successful, with more than 35 years of healthy living. According to the 2017 annual report of renal disease, the 5-year survival rate of kidney transplantation was 92.3%, and the 10-year survival rate was 80.7%. However, as the concept of organ donation is not common among Taiwanese, it is uncertain to have matching and compatible kidneys from donors for patients who have registered for kidney transplantation during the past 10 years.
Regarding the study results of the National Health Insurance database of Prof. Chih-Cheng Hsu and Prof. Chi-Pon Wen from the National Health Research Institute, approximately 200,000 children are diagnosed with chronic renal disease in Taiwan, but the disease causes differ significantly between pediatric chronic renal disease (CRD) and adult chronic renal disease. More than 50% of adult CRDs belong to diabetic nephropathy, which is closely associated with the three highs, whereas 50% of pediatric CRDs may be caused by congenital anomalies of the kidney and urinary tract and 20-30% by glomerulonephritis, as well as the other metabolic disorders, gene mutation, genetic disorder, etc. Therefore, the screening strategy for the early detection of pediatric CRDs is different from the adult options, including the calculation equation of eGFR and so on. Furthermore, the common complications of pediatric CRDs are different. All strategies of the 3 Levels and 5 Stages of Preventive Medicine, treatment guidelines, and care indicators for children should be determined based on their growth, development, and psychological stages, such as the definitions of pediatric hypertension and hyperlipidemia.
When the prenatal or postpartum ultrasound check, urine screening, or urine routines have abnormal results or urinary infection is recurrent in children, parents should be alert to seek assistance from pediatric nephrologists and recheck the kidney ultrasound for further diagnosis. Doing so can identify the cause of disease earlier at Level 2 and then control it, thus preventing disease progression to chronic renal disease. Once the disease progression moves to Level 3 of chronic renal disease, it will ultimately progress to ESRD, at which point kidney transplantation will be the only and last treatment option.