• Childhood Kidney Diseases
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• v.22 no.2
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• pp.81-85
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• 2018

Amyloidosis is a rare disease that results from the deposition of extracellular protein in various body tissues, causing progressive organ dysfunction. Secondary renal amyloidosis is a rare but serious complication of chronic inflammatory bowel disease, particularly in patients with Crohn's disease or ulcerative colitis. We report a case of secondary renal amyloidosis in a pediatric patient who reported a 16-year history of "very early onset inflammatory bowel disease". Intensive treatment including repeated infliximab infusions improved clinical parameters of inflammatory bowel disease, although renal dysfunction showed progression. Amyloidosis should be considered in patients with IBD, particularly if they suffered disease progression.

• Journal of Chest Surgery
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• v.23 no.3
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• pp.482-487
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• 1990

The leading cause of death in patients with chronic renal failure is cardiovascular diseases. The problems relevant to cardiac surgery in these patients are occurring more frequently with a growing number of patients at risk. Among these, important risk factors related to uremic patients undergone open heart surgery are fluid and electrolytes imbalance, coagulopathy, increased susceptibility to infection. Since 1968 when Lansing and colleagues reported the first successful aortic valve replacement in patients with chronic renal failure and infective endocarditis, there have been increasing reports of the cardiopulmonary bypass surgery in chronic renal failure patients with acceptable perioperative morbidity and mortality From Jan. 1988 to Nov. 1989 we have experienced four uremic patients necessitating open heart surgery ; one needing a coronary artery bypass graft and the other 3 needed cardiac valve replacement. Based on our observations we would like to suggest followings 1]Intraoperative ultrahemofiltration during C-P bypass thought to be an excellent means for the control of hyperkalemia and fluid balance. 2] The immediate postoperative application of peritoneal dialysis instead of hemodialysis is beneficial in controlling fluid and electrolyte imbalance. 3]The cause of one early postoperative death was not associated to renal failure, rather it was the result of an accidental rupture in the right ventricular wall.

• Childhood Kidney Diseases
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• v.24 no.2
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• pp.115-119
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• 2020

Distal renal tubular acidosis (dRTA) is a rare renal tubular disorder characterized by normal anion gap metabolic acidosis, hypokalemia, and high urine pH. It can be inherited or acquired. In untreated pediatric patients with dRTA, rickets and growth retardation are common. We report the case of a 12-year-old Lao girl who presented with typical clinical features of dRTA with severe bone deformities that developed after a bed-ridden state due to a bicycle accident at the age of 8 years. Initial laboratory tests revealed metabolic acidosis with a normal anion gap, hypokalemia, and alkali urine. Renal ultrasonography revealed bilateral medullary nephrocalcinosis. Whole exome sequencing revealed no pathogenic mutations. After treatment with oral alkali, potassium, and vitamin D, she could walk and run. Later, she underwent corrective orthopedic surgeries for bony deformities. Thus, in pediatric dRTA patients, despite severe symptoms remaining untreated, accurate diagnosis and proper management can improve quality of life.

• Childhood Kidney Diseases
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• v.23 no.1
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• pp.48-52
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• 2019

The ketogenic diet (KD) has been used as an effective antiepileptic therapy for intractable childhood epilepsy. However, various adverse effects have been reported with use of the KD. We report a case of a child who developed acute tubular necrosis subsequent to therapy with KD. A 5-year-old girl had myoclonic epilepsy with developmental delay. She was under the treatment with antiepileptic drugs since the age of 3 months and on the KD during the past 18 months. Proteinuria persisted intermittently with the initiation of the KD and subsequently increased in the past 2 months. She was admitted with intermittent mild fever, vomiting, and lethargy for the past 3-4 weeks. At the time of admission, she presented with hypertriglyceridemia, heavy proteinuria, renal Fanconi syndrome, and acute kidney injury. Renal sonography showed a marked increase in the size and parenchymal echogenicity of both kidneys. A renal biopsy revealed acute tubular necrosis accompanied by early interstitial fibrosis. After the withdrawal of the KD and supportive therapy, without changing other anticonvulsants and their dosages, improvement of renal function was observed. Proteinuria had disappeared after 1 month and kidney size returned to normal after 8 months. It is hypothesized that the KD can induce and/or aggravate the renal tubulointerstitial injury in some patients who are under the treatment with anticonvulsants.

• Childhood Kidney Diseases
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• v.12 no.1
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• pp.30-37
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• 2008

Purpose: The present study is an investigation of the progression and prognosis of acute intrinsic renal failure in neonates and children with a diagnosis of acute renal failure or other diseases on admission. Methods: This research is based on a retrospective analysis conducted on 59 patients(male: female=2.2:1) diagnosed with acute intrinsic renal failure between January 2000 and June 2006 at Busan Paik Hospital. The clinical diagnostic criteria of acute renal failure used was serum creatinine <1.2 mg/dL, oliguria with urine output$\leq$0.5 mL/kg/hr and anuria with urine output <50 mL per day. Results: Among those placed under investigation, 7 patients were neonates, 10 patients were 2 months-2 years old, 12 patients were 3-6 years old, 21 patients were 7-12 years old and 9 patients were 13-16 years old. It took 3.1${\pm}$2.8 days on average until the diagnosis was made. The urine output distribution was 21 persons for the oliguria group, and 36 persons for the non-oliguria group, and 2 persons for the anuria group. For the underlying causes, 30 persons were classified in the primary renal disease group, 14 persons in the infection group, 9 persons in the malignancy group, and 6 persons were categorized in another group. As for age distribution, the infected group was predominantly neonates, whereas the dominant age ranges for the primary renal disease and infection categories were 2 months to 2 years old. Also, the primary renal disease was dominant among older children, aged 3 and up. No difference was detected according to seasonal prevalence. However, there was a high morbidity rate among hemolytic uremic syndrome diagnosed in the summer. Peritoneal dialysis was used to treat 4 patients. It took 10.0${\pm}$6.7 days until the patients improved. 18 patients died. The non-oliguria group's mortality rate was lower than other groups. There was a high mortality rate in the neonates and malignancy group. Conclusion: Acute renal failure in childhood seems to take a better clinical course than in adulthood when there is an early diagnosis and proper treatment of underlying diseases.

• Childhood Kidney Diseases
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• v.19 no.2
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• pp.131-135
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• 2015

Purpose: The aim of this study was to establish a simple formula to predict renal length in children using a Technesium-99m dimercaptosuccinic acid (DMSA) scan data, and to compare it with the formula derived from ultrasonography, which is widely accepted. Methods: Children who underwent a DMSA scan and ultrasonography were reviewed retrospectively, and those who had anatomical urinary tract abnormalities or urinary tract infections were excluded. Results: A total of 230 children (84 males and 146 females; age, 1 month to 16 years; mean age, $16.8{\pm}27.4$ months). Mean renal length measured by DMSA scan was longer than that by ultrasonography ($6.38{\pm}1.16$ vs. $6.02{\pm}1.14cm$; P < 0.001). Renal length was correlated with age, weight, height, and body surface area on the DMSA scan and ultrasonography, and showed the strongest positive correlation with height. The following formulae were established to predict renal length: mean renal length (cm) = 5.433 ${\times}$ height (m) + 2.330 (R2, 0.833) using the DMSA scan data, and mean renal length (cm) = 5.367 ${\times}$ height (m) + 2.027 (R2, 0.853) using ultrasonography data. Conclusion: We propose a simple height-based formula to predict renal length in children using a DMSA scan data, and validate it by comparing with ultrasonography formula.

• Childhood Kidney Diseases
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• v.12 no.1
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• pp.105-110
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• 2008

Renovascular hypertension results from a lesion that impairs blood flow to a part of or all, of one or both kidneys. Renal artery stenosis is the major cause of renovascular hypertension and the most common cause of treatable secondary hypertension. Recently, percutaneous transluminal renal angioplasty(PTRA) with or without stent placement, has become the preferred choice for correcting symptomatic renal artery stenosis since it is less invasive than surgical reconstruction. PTRA with balloons designed for the dilatation for the dilatation of the coronary artery can be tried in small sized renal artery stenosis. We report a case of renovascular hypertension in a 13-year-old male who had small sized renal artery stenosis. Hypertension was controlled by PTRA with balloon dilatation.

• Journal of Chest Surgery
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• v.32 no.9
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• pp.835-839
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• 1999

Since the first report of coronary artery bypass grafting (CABG) in patients with end-stage renal disease in 1974, numerous reports have documented the feasibility of CABG in patients with chronic renal diseases. Patients with chronic renal failure often have comorbid disorders such as hypertension, and diabetes mellitus, each with their own complications and associated impact on both short and long-term survivals. In addition, infection and sepsis have been identified as significant causes of morbidity and mortality in most series of patients with end-stage renal disease undergoing cardiac surgical procedure. As a result of these and other factors such as perioperative volume and electrolyte disturbances, patients with chronic renal failures are at an increased risk of complication and mortality after CABG. We report 3 cases of "Off-pump" CABG in the chronic renal failure patients. patients.

• Childhood Kidney Diseases
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• v.17 no.1
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• pp.6-12
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• 2013

Thrombotic microangiopathy (TMA) is a microvascular thrombotic lesion caused by endothelial injury and subsequent formation of platelet rich thrombus. TMA is first described as a classical pathologic feature of HUS/TTP. Renal biopsy finding of TMA represents kidney involvement of HUS/TTP as well as other diseases such as malignant hypertension, drug toxicity, eclampsia, pre-eclampsia, and several systemic infections. Autoimmune diseases and transplant kidney sometime also have TMA. It is needed to consider a complete autoimmune work-up of patients presenting with TMA including tests for ANA, ANCA, and ADAMTS13 inhibitory antibodies, because there are several reports of association with TMA in patients of SLE, anti-phospholipid syndrome, and ANCA-associated vasculitis.

• Korean Journal of Clinical Laboratory Science
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• v.38 no.2
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• pp.111-116
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• 2006

One of the cardinal findings of the renal diseases is proteinuria, which appears in the early phase of kidney diseases and is very important in diagnosis, prognosis and decision making in the treatment process and results of the treatment. The study subjects were 126 patients who visited the nephrology department of Kangbuk Samsung Hospital. Serum was requested for urine protein electrophoresis. Total protein was measured with Bayer Advia 1650 (Biuret). Quantitation of each fraction was done by multiplying the percentage of each fraction by the total protein. Serum creatinine and BUN were also measured with Bayer Advia 1650 (Jaffe and Urease). Serum protein EP was done with REP(rapid electrophoresis) using Helena Kit reagents (REP Ultra SPE Kit, Ponceau S stain, Acetic acid, Methanol, EP Control). Concentrated urine was used for urine protein EP. The SPSS package was used for statistics analysis. Percentage and quantitation of the level of albumin in renal diseases were significantly lower than those in healthy controls. Total protein was correlated with albumin. In terms of proportion, ${\alpha}1$-globulin, ${\alpha}2$-globulin, ${\beta}$-globulin, and ${\gamma}$-globulin fractions were increased in the disease group. But, in the quantified level, ${\alpha}2$-globulin was increased and ${\beta}$-globulin and ${\gamma}$-globulin were decreased. ESRD patients showed an increased secretion of high molecular proteins in urine protein EP. A decreased level in serum total protein correlated with the decreased level of serum albumin and the total amount of urine total protein. This study revealed the variety in the level of serum and urine proteins and their subgroups by EP.