• Childhood Kidney Diseases
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• 제1권2호
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• pp.183-188
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• 1997

Familial juvenile hyperuricemic nephropathy is an autosomal dominant disease characterized by progressive renal disease and hyperuricemia or gout, affecting young people of either sex equally. There are two biochemical markers of this disorder. The first is hyperuricemia disproportionate to the degree of renal dysfunction; the second is a grossly reduced clearance of uric acid relative to creatinine, dispropotionate to age, sex and degree of renal failure. We experienced 2 family members with hyperuricemia. One family member, a 13-year-old girl who had suffered from tophaceous gout and chronic renal failure. Her younger brother also had hyperuricemia and moderately reduced renal function. Their urinary excretion fractions of uric acid($FE_{uric\;acid}$) were reduced and renal biopsy specimens showed interstitial fibrosis with tubular atrophy and interstitial urate crystal deposition. We have treated these two patients with allopurinol but we have done renal transplantation because she progressed to end stage renal disease at 16 year old age.

• Kidney Research and Clinical Practice
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• 제35권2호
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• pp.69-77
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• 2016

Diabetic nephropathy (DN) is the leading cause of end-stage renal disease, and its pathogenesis is complex and has not yet been fully elucidated. Abnormal glucose and lipid metabolism is key to understanding the pathogenesis of DN, which can develop in both type 1 and type 2 diabetes. A hallmark of this disease is the accumulation of glucose and lipids in renal cells, resulting in oxidative and endoplasmic reticulum stress, intracellular hypoxia, and inflammation, eventually leading to glomerulosclerosis and interstitial fibrosis. There is a growing body of evidence demonstrating that dysregulation of 50 adenosine monophosphate-activated protein kinase (AMPK), an enzyme that plays a principal role in cell growth and cellular energy homeostasis, in relevant tissues is a key component of the development of metabolic syndrome and type 2 diabetes mellitus; thus, targeting this enzyme may ameliorate some pathologic features of this disease. AMPK regulates the coordination of anabolic processes, with its activation proven to improve glucose and lipid homeostasis in insulin-resistant animal models, as well as demonstrating mitochondrial biogenesis and antitumor activity. In this review, we discuss new findings regarding the role of AMPK in the pathogenesis of DN and offer suggestions for feasible clinical use and future studies of the role of AMPK activators in this disorder.

• 멤브레인
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• 제34권2호
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• pp.105-113
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• 2024

만성 콩팥병은 많은 사람들이 말기병으로 진단받고 있다. 이 질병은 혈액이 막과 투석기에 의해 세척되는 과정인 혈액투석에 의해 치료된다. 혈액투석을 위한 막은 혈액에서 불순물인 요소를 선택적으로 제거하는 역할을 하기 때문에 이 과정은 중요한 첫 단계이다. 다양한 종류의 고분자로 구성된 막이 사용되지만, 낮은 선택도와 혈액적합성과 같은 다양한 단점을 가지고 있다. 이를 해결하기 위해 여러 논문에서 서로 다른 고분자의 혼합물을 사용하여 기공의 크기를 변화시키고 혈액적합성을 높이며 선택성을 높이는 연구를 진행하였다. 그동안 보고된 논문들을 통해 혼합막이 더 큰 장점을 가지고 있음을 알 수 있었다.

• 대한치과마취과학회지
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• 제3권2호
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• pp.98-102
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• 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.

• Childhood Kidney Diseases
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• 제24권2호
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• pp.91-97
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• 2020

Purpose: Alport syndrome (AS) is one of the most common inherited renal diseases caused due to mutations of genes encoding specific proteins of the type IV collagen family, and its major clinical manifestations include progressive renal failure, sensorineural deafness, and ocular abnormalities. We investigated the clinical characteristics and long-term prognosis of AS in Korean pediatric and adult populations. Methods: We conducted a retrospective review of medical records of 33 children and adults who had been diagnosed or treated with AS from 1985 to 2019. Results: The mean age of the 33 patients diagnosed with AS was 16.2±13.6 years, and the male-to-female ratio was 2:1. At the first visit, recurrent gross hematuria was the most common initial symptom. In 10 of 33 patients (30.3%), sensorineural hearing loss (SNHL) was diagnosed, but none had ophthalmic problems. Moreover, 11 of 33 patients (33.3%) had advanced to end-stage renal disease (ESRD), and a significant difference was observed in the age of the patients who progressed to ESRD based on the presence or absence of SNHL (P=0.035). Conclusion: SNHL in AS can be an important prognostic factor for long-term deterioration of renal function. Further investigation is required to confirm the clinical course and the genetic characteristics of AS in Korea through prospective national cohort studies.

• Journal of Genetic Medicine
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• 제11권2호
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• pp.74-78
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• 2014

Sotos syndrome (SS, OMIM 117550) is characterized by prenatal and postnatal overgrowth with multiple congenital anomalies. However, there have been few cases of growth retardation caused by renal failure from infancy. We report a case of dysplasia of the bilateral kidneys with renal failure and poor postnatal growth. A 2-month-old boy visited the emergency room owing to poor oral intake and abdominal distension. He was born at the gestational age of 38 weeks with a birth weight of 4,180 g. After birth, he had feeding difficulty and abdominal distension. Upon physical examination, his height and weight were in less than the 3rd percentile, while his head circumference was in the 50th percentile on the growth curve. He also showed a broad and protruding forehead and high hairline. Blood laboratory tests showed severe azotemia; emergent hemodialysis was needed. Abdominal ultrasonography revealed bilateral renal dysplasia with multiple cysts and diffuse bladder wall thickening. A posterior urethral valve was suggested based on vesicoureterography and abdominal magnetic resonance findings. Results of a colon study to rule out congenital megacolon did not reveal any specific findings. The conventional karyotype of the patient was 46, XY. Array comparative genomic hybridization study revealed a chromosome 5q35 microdeletion including the NSD1 gene, based on which SS was diagnosed. We describe a case of SS presenting with end stage renal disease due to posterior urethral valve. The typical somatic overgrowth of SS in the postnatal period was not observed due to chronic renal failure that started in the neonatal period.

• Journal of Yeungnam Medical Science
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• 제40권4호
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• pp.381-387
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• 2023

Background: Frailty is defined as a condition of being weak and delicate, and it represents a state of high vulnerability to adverse health outcomes. Recent studies have suggested that the cingulate gyrus is associated with frailty in the elderly population. However, few imaging studies have explored the relationship between frailty and the cingulate gyrus in patients with end-stage renal disease (ESRD) undergoing hemodialysis. Methods: Eighteen right-handed patients with ESRD undergoing hemodialysis were enrolled in the study. We used the FreeSurfer software package to estimate the cortical thickness of the regions of interest, including the rostral anterior, caudal anterior, isthmus, and posterior cingulate gyri. The Beck Depression Inventory, Beck Anxiety Inventory, and laboratory tests were also conducted. Results: The cortical thickness of the right rostral anterior cingulate gyrus (ACG) was significantly correlated with the Fried frailty index, age, and creatinine level. Multiple regression analysis indicated that the cortical thickness of the right rostral ACG was associated with frailty after controlling for age and creatinine level. Conclusion: Our results indicate that the cortical thickness of the rostral ACG may be associated with frailty in patients with ESRD on hemodialysis and that the rostral ACG may play a role in the frailty mechanism of this population.

• Journal of Yeungnam Medical Science
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• 제30권2호
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• pp.136-140
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• 2013

Diabetic nephropathy (DN) is a common complication and the leading cause of end-stage renal disease (ESRD) in diabetic patients. The occurrence of non-diabetic renal disease (NDRD) in diabetic patients has been increasingly recognized in recent years. Generally, renal injuries in DN are deemed difficult to reverse, whereas some NDRDs are often treatable and even remittable. Thus, the diagnosis of NDRD in patients with diabetes mellitus (DM) via a kidney biopsy would be significant for its prognosis and therapeutic strategy. According to recent studies, the most common NDRD is IgA nephropathy in type 2 diabetic patients, and some cases of minimal change disease and membranous glomerulonephritis have been reported in Korea. However, membranoproliferative glomerulonephritis (MPGN) is an uncommon condition in diabetic patients. To our knowledge, there has been no case yet of MPGN, except in a child with type 1 DM. We present an unusual case of a 27-year-old woman who had type 2 DM with MPGN, as confirmed via a kidney biopsy.

• Journal of Chest Surgery
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• 제33권4호
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• pp.338-341
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• 2000

Renal transplant recipients who received immunosuporessive agent are in high risk of development Kaposi's sarcoma. In Korea a few report of Kaposi's sarcoma has been pubilshed but any report of intrathoracic Kaposi's sarcoma provedn by open lung biopsy has not been pulbilshed until now. We report a case of intrathoracic Kaposi's sarcoma developed in a 25 year old Korean man, who had been operated renal transplantation due to end stage renal disease and received cyclosporine and prednisolone as immunosuppessive agent, without any other organ involvment and was proven by open lung biopsy. Although discontinuation of immunosuppressive agent, temporary symptomatic and radilolgic improvement were observed, he died 11 days later after open lung biopsy because of intractable resiratory failure.

• Childhood Kidney Diseases
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• 제15권2호
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• pp.107-115
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• 2011

소아에서 급성 신손상의 흔한 원인들로는 신허혈, 신독성 약물들, 그리고 패혈증 등이 있으며, 신대체요법 시작시의 저혈압, 신대체 요법 동안 승압제의 사용, 그리고 신대체 요법 시작시의 수액 과부하 정도가 환자의 생존(소아 중환자실 퇴원)에 영향을 미치는 요인들로 알려져 있다. 지속적 신대체 요법의 빠른 시작은 급성 신손상을 가진 환자들에게서 사망률과 예후에 나쁜 영향을 미치는 수액 과부하를 감소시키는 것으로 보고되었다. 이에 저자들은 소아 환자에게서 지속적 신대체 요법의 실제 처방과 급성 신손상, 수액 과부하, 그리고 지속적 신대체 요법간의 연관성 및 치료결과를 살펴보고자 한다. 결론적으로, 급성 신손상을 가진 소아의 치료에 있어서 과도한 수액 과부하가 발생하기 전에 빠른 지속적 신대체 요법의 시작이 필요하다고 제시하는 바이다.