• Childhood Kidney Diseases
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• v.24 no.2
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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.

• Korea-Australia Rheology Journal
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• v.17 no.3
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• pp.117-123
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• 2005

Background: Reduced deformability of red blood cells (RBCs) may play an important role on the pathogenesis of chronic vascular complications of diabetes mellitus. However, available techniques for measuring RBC deformability often require washing process after each measurement, which is not optimal for day­to-day clinical use at point of care. The objectives of the present study are to develop a device and to delineate the correlation of impaired RBC deformability with diabetic nephropathy. Methods: We developed a disposable ektacytometry to measure RBC deformability, which adopted a laser diffraction technique and slit rheometry. The essential features of this design are its simplicity (ease of operation and no moving parts) and a disposable element which is in contact with the blood sample. We studied adult diabetic patients divided into three groups according to diabetic complications. Group I comprised 57 diabetic patients with normal renal function. Group II comprised 26 diabetic patients with chronic renal failure (CRF). Group III consisted of 30 diabetic subjects with end-stage renal disease (ESRD) on hemo-dialysis. According to the renal function for the diabetic groups, matched non-diabetic groups were served as control. Results: We found substantially impaired red blood cell deformability in those with normal renal function (group I) compared to non-diabetic control (P = 0.0005). As renal function decreases, an increased impairment in RBC deformability was found. Diabetic patients with chronic renal failure (group II) when compared to non-diabetic controls (CRF) had an apparently greater impairment in RBC deformability (P = 0.07). The non-diabetic cohort (CRF), on the other hand, manifested significant impairment in red blood cell deformability compared to healthy: control (P = 0.0001). Conclusions: The newly developed slit ektacytometer can measure the RBC deformability with ease and accuracy. In addition, progressive impairment in cell deformability is associated with renal function loss in all patients regardless of the presence or absence of diabetes. In diabetic patients, early impairment in RBC deformability appears in patients with normal renal function.

• Journal of Chest Surgery
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• v.35 no.9
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• pp.680-683
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• 2002

Infective endocarditis that involves the right side of the heart has been estimately 5% of all cases of infective endocarditis. It has been shown that about 70% of right-sided heart infective endocarditis cases have preexisting congenital heart disease or acquired valvular lesion. It would occur in intravenous drug users or end-stage renal disease patients with indwelling venous dialysis catheter. Antibiotic therapy is more effective in the right and, when it fails, the consequence of valve disruption and emboli are less. Patients receiving long-term hemodialysis are a unique population with regard in the risk of bacteremia and subsequent infective endocarditis. We experienced one case of the active infective endocarditis with right atrial vegetation without tricuspid or pulmonary valve involvement in patient with end-stage renal disease receiving long-term hemodialysis, who needed surgical correction after medical treatment failure. Then we reported it with references that right-sided heart infective endocarditis is rare, but difficult to diagnose, life-threatening because of delayed medical treatment.

• Childhood Kidney Diseases
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• v.28 no.1
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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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• v.23 no.2
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• pp.67-76
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• 2019

Kidney disease is a major global health issue. Hemodialysis and kidney transplantation have been used in the clinic to treat renal failure. However, the dialysis is not an effective long-term option, as it is unable to replace complete renal functions. Kidney transplantation is the only permanent treatment for end-stage renal disease (ESRD), but a shortage of implantable kidney tissues limits the therapeutic availability. As such, there is a dire need to come up with a solution that provides renal functions as an alternative to the current standards. Recent advances in cell-based therapy have offered new therapeutic options for the treatment of damaged kidney tissues. Particularly, cell secretome therapy utilizing bioactive compounds released from therapeutic cells holds significant beneficial effects on the kidneys. This review will describe the reno-therapeutic effects of secretome components derived from various types of cells and discuss the development of efficient delivery methods to improve the therapeutic outcomes.

• Childhood Kidney Diseases
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• v.20 no.2
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• pp.92-96
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• 2016

Henoch-$Sch{\ddot{o}}nlein$ purpura (HSP) is the most common vasculitis in children, mainly affecting the small vessels of the skin, joints, gastrointestinal tract, and kidneys. Although most cases of HSP resolve spontaneously without sequelae, serious nephrological and intestinal problems may occur in some cases. We experienced a case of HSP complicated by simultaneous intussusception and nephritis in a 14-year-old boy who developed a sudden abdominal pain and gross hematuria on the 11th day after onset of the disease. Imaging studies revealed intussusception that required emergency laparotomy. Despite treatment with steroid and angiotensin-converting enzyme inhibitors, nephritis and nephrosis progressed for 4 weeks, and renal biopsy was performed to confirm the diagnosis. Cyclosporin A therapy was started, and remission of proteinuria was achieved after 5 months. However, the nephritis recurred and worsened to end-stage renal failure during 15 years of follow-up.

• Journal of Chest Surgery
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• v.46 no.6
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• pp.426-432
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• 2013

Background: Heart transplantation has become a widely accepted surgical option for end-stage heart failure in Korea since its first success in 1992. We reviewed early postoperative complications and mortality in 239 patients who underwent heart transplantation using bicaval technique in Asan Medical Center. Methods: Between January 1999 and December 2011, a total of 247 patients aged over 17 received heart transplantation using bicaval technique in Asan Medical Center. After excluding four patients with concomitant kidney transplantation and four with heart-lung transplantation, 239 patients were enrolled in this study. We evaluated their early postoperative complications and mortality. Postoperative complications included primary graft failure, cerebrovascular accident, mediastinal bleeding, renal failure, low cardiac output syndrome requiring intra-aortic balloon pump or extracorporeal membrane oxygenation insertion, pericardial effusion, and inguinal lymphocele. Follow-up was 100% complete with a mean follow-up duration of $58.4{\pm}43.6$ months. Results: Early death occurred in three patients (1.3%). The most common complications were pericardial effusion (61.5%) followed by arrhythmia (41.8%) and mediastinal bleeding (8.4%). Among the patients complicated with pericardial effusion, only 13 (5.4%) required window operation. The incidence of other significant complications was less than 5%: stroke (1.3%), low cardiac output syndrome (2.5%), renal failure requiring renal replacement (3.8%), sternal wound infection (2.0%), and inguinal lymphocele (4.6%). Most of complications did not result in the extended length of hospital stay except mediastinal bleeding (p=0.034). Conclusion: Heart transplantation is a widely accepted option of surgical treatment for end-stage heart failure with good early outcomes and relatively low catastrophic complications.

• Journal of Chest Surgery
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• v.38 no.5 s.250
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• pp.389-390
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• 2005

We report a case of a 48-year-old woman with end-stage renal failure who had a Polytetrafluoroethylene graft for hemodialysis and who had developed complications of venous outflow stenosis and venous backflow. Although venous backflow is an harbinger of graft failure, it is not enough reason to abandon the graft immediately. The patient was able to utilize her graft for 6 further months.

• Clinical and Experimental Pediatrics
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• v.64 no.2
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• pp.49-59
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• 2021

Since the initial International Society of Heart Lung Transplantation registry was published in 1982, the number of pediatric heart transplantations has increased markedly, reaching a steady state of 500-550 transplantation annually and occupying up to 10% of total heart transplantations. Heart transplantation is considered an established therapeutic option for patients with end-stage heart disease. The long-term outcomes of pediatric heart transplantations were comparable to those of adults. Issues affecting long-term outcomes include acute cellular rejection, antibody-mediated rejection, cardiac allograft vasculopathy, infection, prolonged renal dysfunction, and malignancies such as posttransplant lymphoproliferative disorder. This article focuses on medical issues before pediatric heart transplantation, according to the Korean Network of Organ Sharing registry and as well as major problems such as graft rejection and cardiac allograft vasculopathy. To reduce graft failure rate and improve long-term outcomes, meticulous monitoring for rejection and medication compliance are also important, especially in adolescents.

• The Korean Journal of Nuclear Medicine
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• v.30 no.3
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• pp.338-343
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• 1996

In this preliminary study, plasma osteocalcin, PTH level and $^{99m}Tc$-HMDP (hydro-xymetylene diphosphonate) bone uptake(BU) were measured in 14 patients with chronic end-stage renal failure who were on maintenance hemodialysis. The aim of this study was to determine the difference of bone uptake between renal failure patients and normal volunteers, and to determine the correlation between bone uptake and osteocalcin - a sensitive and specific marker of osteoblastic activity and PTH - a important hormone of bone metabolism. There was a statistically significant increase in 180 minute uptake in the patient group when compared to the normal volunteers while there was no statistically significant difference in 20 minute uptake. Plasma osteocalcin and PTH levels were also significantly elevated compared to normal values. But the correlation between osteocalcin, PTH and 20 and 180 minute bone uptake was not significant. In conclusion, our preliminary study suggests that, in chronic renal failure patients, 180 minute $^{99m}Tc$-HMDP bone uptake is increased significantly without direct correlation with serum osteocalcin or PTH levels. It seems that further study is needed to evaluate other unknown factors that may influence the direct correlation between bone uptake and plasma osteocalcin and PTH in patients with chronic renal failure.