• Childhood Kidney Diseases
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• 제19권1호
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• pp.23-30
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• 2015

NPHP is the most common monogenic cause of CKD in children or adolescents. Extra-renal symptoms often accompany, therefore examination of retina, hearing, and skeleton is necessary in patients with CKD with insidious onset. Genes involved in NPHP-RC are mostly related in primary cilia. While genetic diagnosis is necessary for definitive diagnosis, there is no curative treatment.

• 대한한방내과학회지
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• 제28권4호
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• pp.791-796
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• 2007

Objective : This study was conducted to find the relationship between syndrome-differentiation and Sasang-constitution in chronic fatigue patients. Methods : The subjects were 72 adults with chronic fatigue who visited Dunsan Oriental Hospital of Daejeon University between March 2007 and April 2007. Their main complaint was fatigue for longer than 6 months and they did not have any physical or mental problems. We measured their fatigue degree by using Chalder fatigue scale and health habits. To evaluate relationship between syndrome-differentiation with sasang-constitution, The patients were divided into four syndrome-differentiations, such as liver and kidney asthenia of Yin, spleen and kidney deficiency of Yang, deficiency of lung and spleen qi, and deficiency of heart and spleen blood. We also diagnosed Sasang-constitution by using Questionnaire for Sasang Constitution Classification II (QSCCII). Results : Among the 72 patients, 41.4%, 34.5% and 24.1% of belonged in Soyangin, Soeumin and Taeumin respectively. Interestingly, we found a meaningful correlation between syndrome-differentiation and physiological function by Sasang-constitution. Conclusion : The result may help Oriental medicine understanding and treatment of chronic fatigue-related diseases and patients.

• Childhood Kidney Diseases
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• 제27권1호
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• pp.19-25
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• 2023

Proteinuria is an important risk factor for renal and cardiovascular disease. It is associated with a risk for glomerulonephritis, chronic kidney disease, and end-stage renal disease. Therefore, if persistent proteinuria is detected, kidney biopsy is considered to diagnose and treat the underlying disease. Recently, variants in the cubilin (CUBN) gene, which is associated with albuminuria, have been reported. This gene encodes cubilin, a membrane glycoprotein receptor expressed in the renal proximal tubules. Cubilin is a component of the megalin and cubilin-amnionless complex that mediates albumin reabsorption into the proximal tubules through endocytosis. A defect in cubilin leads to a reduction in albumin reuptake, resulting in albumin-dominant proteinuria. Although numerous controversies exist, several reports suggest that cubilin defects lead to proteinuria with a high portion of albuminuria but may not impair renal filtration function. If albuminuria due to reduced cubilin function is confirmed as a benign condition, we can consider using genetic studies to detect CUBN mutations in patients with proteinuria and they may not require any treatment or kidney biopsy. Here, we review recent papers on CUBN mutations and discuss the prognosis and management of individuals with this mutation.

• Childhood Kidney Diseases
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• 제28권1호
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• pp.1-7
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• 2024

Antenatally diagnosed urinary tract dilatation (UTD), previously referred to as antenatal hydronephrosis, is the most commonly detected abnormality by prenatal ultrasonography. Several grading systems have been developed for the classification of antenatal UTD using prenatal and postnatal ultrasonography. UTD comprises a wide variety of congenital abnormalities of the kidney and urinary tract ranging from transient UTD to more significant abnormalities such as vesicoureteral reflux, ureteropelvic junction obstruction, ureterocele, ureterovesical junction obstruction, posterior urethral valves, and non-refluxing megaureter. Optimizing the evaluation of antenatally detected UTD is essential to recognize children with important disorders while avoiding excessive investigations. Conservative approach with close follow-up is increasingly accepted as an appropriate treatment option for patients with asymptomatic vesicoureteral reflux and ureteropelvic junction obstruction in recent years. However, predicting permanent kidney damage in an unselected group of children with antenatal UTD is still challenging. The management and follow-up of children with UTD should be individualized based on recommendations from a pediatric nephrologist, a pediatric urologist, or both. Future research directed at predicting long-term outcomes of children diagnosed with UTD from mild findings to severe disease is needed to refine management for those at higher risk of kidney disease progression.

• Childhood Kidney Diseases
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• 제22권1호
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• pp.7-11
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• 2018

Significant advances in the diagnosis and medical care of children with chronic kidney disease (CKD) are major reasons for the better survival rates of children and adolescents with CKD than the survival rates reported in previous decades. These patients are reaching adulthood, and therefore require a transition to adult medical care. This transition phase is well-recognized to be associated with considerably increased morbidities and medical problems, such as non-adherence, graft loss after transplantation, and loss to follow-up. Low adherence increases morbidity and medical complications and contributes to poorer qualities of life and an overuse of the health care system. However, these tragic outcomes may be avoidable through a structured and well-defined transition program. In the last decade, there has been increasing interest to resolve these medical and psychological problems that occur during the transfer of young adult patients from pediatric to adult renal units. The aims of a successful transition from pediatric to adult medical care include enhancing the individual development of better health-competence and stabilizing, or even improving, the state of health. This review will focus on various aspects of the transition phase of adolescents who have CKD or who underwent kidney transplantation from pediatric to adult nephrology care.

• Childhood Kidney Diseases
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• 제28권1호
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• pp.44-50
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• 2024

Systemic lupus erythematosus (SLE) is a chronic autoimmune inflammatory disease that affects multiple organs. More than half of the patients with SLE have kidney involvement, and up to 10% of patients with lupus nephritis develop end-stage renal disease (ESRD). Central nervous system (CNS) involvement in SLE occurs in 21% to 95% of patients. Severe neurological manifestations such as seizures, cerebrovascular disease, meningitis, and cerebrovascular accidents can develop in childhood-onset SLE, but cerebral infections, such as brain abscess and hemorrhage, are seldom reported in lupus nephritis, even in adults. Here, we report a rare case of childhood-onset SLE with ESRD, cerebral abscess, and hemorrhage. A 9-year-old girl diagnosed with lupus nephritis was administered high-dose steroids and immunosuppressant therapy to treat acute kidney injury (AKI) and massive proteinuria. The AKI deteriorated, and after 3 months, she developed ESRD. She received hemodialysis three times a week along with daily peritoneal dialysis to control edema. She developed seizures, and imaging showed a brain abscess. This was complicated by spontaneous cerebral hemorrhage, and she became unstable. She died shortly after the hemorrhage was discovered. In conclusion, CNS complications should always be considered in clinical practice because they increase mortality, especially in those with risk factors for infection.

• Childhood Kidney Diseases
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• 제15권1호
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• pp.22-28
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• 2011

고혈압은 소아 청소년시기에 발생하는 흔한 만성질환 중 하나이다. 고혈압을 치료하지 않으면 여러가지 소아의 장기에 악영향을 미칠 수 있다. 여기서는 고혈압이 중추신경계에 미치는 영향에 대해 알아 보았다. 혈압의 변화에 따라 대뇌가 받는 영향은 다른 장기에 비해 덜 하다. 그것은 대뇌 자동 조절 기전때문이다. 즉 혈압의 변화에 따라 뇌혈관의 저항이 변하기 때문에 뇌혈류량이 일정하게 유지 된다. 그러나 이런 자동 조절 기전의 항상성이 파괴되면 급성으로 또는 만성으로 급성 고혈압 뇌병증, 허혈성, 출혈성 뇌경색, 학습 장애와 인지 기능 장애 등이 발생한다. 이에 고혈압이 대뇌에 미치는 영향에 대해 숙지하고 빠른 시일 내에 적절한 치료를 하면 이런 합병증을 예방할 수 있다.

• 한국동물위생학회지
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• 제24권2호
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• pp.139-146
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• 2001

To examine the prevalence and types of kidney lesions in Korean cattle, a survey was carried out at a abattoir in september 2000 and January 2001. The collected kidneys were examined grossly and histopathologically and investigated for the patterns of renal diseases with considering season and sex. Of 735 cattle (99 bulls and 246 cows in September and 140 bulls, 3 steers, and 247 cows in January) surveyed, 301 (41%) cattle had various renal lesions. The most common prominent finding was focal or multiple interstitial nephritis (36.2%). Other lesions included focal or multiple renal cyst (9.1%), infarction (0.1%), hemorrhage (0.3%), and renal atrophy (0.3%). Microscopically, main pathological lesions were acute or chronic interstitial nephritis with moderate infiltration of neutrophil or lymphocyte, respectively. Finally, the kidney lesions are more or less correlated with the meat quality, especially in female Korean cattle.

• Childhood Kidney Diseases
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• 제15권1호
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• pp.1-13
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• 2011

Hypertension is a major risk factor of atherosclerosis which results in cardiovascular disease, and remains a major health problem worldwide. While children are more likely to have secondary hypertension, recent studies support the theory that the prevalence of essential hypertension in children and adolescents is increasing with the global epidemic of childhood obesity, and close attention is needed. Evaluation of hypertension in the pediatric age group should be guided by the age at presentation, and renal diseases must be considered in every child with hypertension, because of the prevalence of renovascular and renal parenchymal disorders as the etiology in any age group. The majority of children with chronic kidney disease are hypertensive, and many have associated end organ damage. Thus, once hypertension has been confirmed, end organ care as well as pharmacologic therapy must be continued. In renovascular hypertension, as cure could be gained with surgical/endovascular intervention, accurate diagnosis is important and it is recommended that every suspected child should undergo angiography.

• Parasites, Hosts and Diseases
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• 제54권3호
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• pp.315-317
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• 2016

Hookworm infections as well as other intestinal nematodiases are endemic in China. In this case, a 70-year-old male showed symptoms of chest tightness, shortness of breath, and both lower extremities edema. The diagnostic result was chronic renal insufficiency, chronic kidney disease (5th stage), and renal anemia at first. Then, he received treatment with traditional drugs. However, this treatment did not help to alleviate the symptoms of the patient significantly. The results of gastroendoscopy showed hookworms in the duodenum, also confirmed by pathology examination. Anemia was markedly ameliorated after eliminating the parasites. The results mentioned above suggested that ancylostomiasis was the leading causes of anemia in this patient, and the etiology of anemia in uremic patients should be systematically considered. Especially when anemia could not be cured by regular treatments, rare diseases should be investigated.