• Childhood Kidney Diseases
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• 제21권2호
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• pp.41-46
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• 2017

Purpose: Thin glomerular basement membrane nephropathy (TBMN) is, along with the IgA nephropathy, the most common cause of asymptomatic hematuria in Korean children. TBMN is usually a benign renal disease not requiring treatment and is associated with a good prognosis, but some cases hematuria is indicative of a state of progressive renal insufficiency. We aimed to retrospectively evaluate clinical manifestations and renal prognosis of patients with TBMN. Methods: Among the 428 renal biopsies performed on children at Yeungnam University Hospital between January 2000 and February 2017, 167 patients were diagnosed as having TBMN. We retrospectively investigated 167 pediatric patients and identified 59 children with follow-up duration >3 years. Results: Among 59 patients, there were 33 boys and 26 girls. Mean age of onset of hematuria was $7.18{\pm}2.64$ years, and mean time from onset of disease until a renal biopsy was performed was $2.48{\pm}2.10$ years. There were no clinical features or laboratory findings among studied children to indicate decreased renal function during follow-up; however, one case progressed to chronic kidney disease (CKD) due to an unknown cause. There were seven patients among these related a positive family history of hematuria or renal insufficiency. Concluson: Although almost all patients had normal renal functions during follow-up, there were one patient who progressed to CKD and seven patients with family history of hematuria or renal insufficiency. Moreover, four among the 428 patients over 17 years underwent repeat renal biopsies, which showed results different from their earlier biopsies.Thus, large-scales studies may be required to determine long-term prognosis of TBMN in children, and further evaluation for Alport syndrome in TBMN cases is essential.

• 대한임상독성학회지
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• 제16권2호
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• pp.157-160
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• 2018

Chronic silica nephropathy has been associated with tubulointerstitial disease, immune-mediated multisystem disease, chronic kidney disease, and end-stage renal disease. On the other hand, acute intentional exposure is extremely rare. The authors' experienced a 44-year-old man who took rapid cement hardener (sodium silicate) in a suicide attempt whilst in a drunken state. He visited the emergency department approximately 1 hour after ingestion. Information on the material was obtained after 3 L gastric lavage. The patient complained of a sore throat, epigastric pain, and swollen to blood tinged vomitus. Proton pump inhibitors, hemostats, steroid, and fluids were administered. Nine hours after ingestion, he was administered 200 mL hematochezia. Immediately after, a gas-troenterologist performed an endoscopic procedure that revealed diffuse hyperemic mucosa with a color change and variable sized ulceration in the esophagus, whole stomach, and duodenal $2^{nd}$ portion. Approximately 35 hours later, persistent oligouria and progressive worsening of the renal function parameters (BUN/Cr from 12.2/1.2 to 67.5/6.6 mg/dL) occurred requiring hemodialysis. The patient underwent 8 sessions of hemodialysis for 1 month and the BUN/Cr level increased to 143.2/11.2 mg/dL and decreased to 7.6/1.5 mg/dL. He was discharged safely from the hospital. Follow up endoscopy revealed a severe esophageal stricture and he underwent endoscopic bougie dilatation. Acute cement hardener (sodium silicate) intoxication can cause renal failure and strong caustic mucosal injury. Therefore, it is important to consider early hemodialysis and treatment to prevent gastrointestinal injury and remote esophageal stricture.

• 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.

• Childhood Kidney Diseases
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• 제27권2호
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• pp.121-126
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• 2023

The administration of angiotensin type 2 receptor blockers (ARBs) during pregnancy is known to cause ARB fetopathy, including renal insufficiency. We aimed to analyze the outcomes of two patients who survived ARB fetopathy and perform an accompanying literature review. Case 1 was exposed antenatally from a gestational age of 30 weeks to valsartan because of maternal pregnancy-induced hypertension. The patient presented with oliguria immediately after birth, and renal replacement therapy was administered for 24 days. Seven years after birth, renal function was indicative of stage 2 chronic kidney disease (CKD) with impaired urinary concentration. Case 2 had a maternal history of hypertension and transient ischemic attack and was treated with olmesartan until 30 weeks of pregnancy. Renal replacement therapy was performed for 4 days since birth. After 8 years, the patient is with CKD stage 2, with intact tubular function. Recent reports suggest that ARB fetopathy might manifest as renal tubular dysgenesis and nephrogenic diabetes insipidus, in contrast to mild alterations of glomerular filtration. Tubular dysfunction may induce CKD progression and growth retardation. Patients with ARB fetopathy should be monitored until adulthood. The ARB exposure period might be a critical factor in determining the severity and manifestations of fetopathy.

• Journal of Medicine and Life Science
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• 제19권2호
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• pp.66-69
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• 2022

Patients with vesicoureteral reflux (VUR), the retrograde flow of urine from the bladder to the kidney, are known to experience renal scarring; this results in the worsening of renal function. Reflux nephropathy is a cause of chronic kidney disease, and VUR has also been observed in dialysis patients. VUR is a major underlying precursor condition of urinary tract infection (UTI) and is sometimes accompanied by hydronephrosis. However, there are no guidelines for the management of UTI due to VUR-associated hydronephrosis in patients with end-stage kidney disease. Herein, we report a case of UTI caused by VUR-associated hydronephrosis in a dialysis patient treated with percutaneous nephrostomy.

• Journal of Yeungnam Medical Science
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• 제29권2호
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• pp.102-105
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• 2012

Collapsing glomerulopathy (CG) has become an important cause of end-stage renal disease (ESRD). First delineated from other proteinuric glomerular lesions in the 1980s, CG is now recognized as a common, distinct pattern of proliferative parenchymal injury that portends a rapid loss of renal function and poor responses to empirical therapy. The first cases in the literature trace back to human-immunodeficiency-virus(HIV)-negative patients who underwent biopsy in 1979. A 45-year-old male patient complained of hematuria and proteinuria eight years ago. He showed an abrupt serum creatinine increase from 1.75 to 2.65 mg/dL in the last preceding months. Afterwards, his serum creatinine progressively increased up to 6.82 mg/dL. Moreover, his 24 h urine protein level was determined to have reached 6,171 mg/day, as opposed to 670 mg/day a year earlier. Consequently, renal biopsy was performed, and its result showed collapsing glomerulopathy, compatible with the diagnosis. He has undergone continuous ambulatory peritoneal dialysis as renal replacement therapy. Thus, it is reported herein that a patient clinically diagnosed with chronic kidney disease eight years ago showed a sudden renal-function decrease and was clinicopathologically diagnosed with collapsing glomerulopathy based on the results of his renal biopsy.

• Journal of Digestive Cancer Research
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• 제4권1호
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• pp.36-38
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• 2016

Biliary tract cancer, a relatively rare disease, is usually found in an unresectable stage. Weekly cisplatin plus gemcitabine has been applied as a standard first-line therapy for advanced biliary tract cancer, but almost up to 3-5% patients experience drug induced renal impairment. Many anticancer medication guidelines recommend drug adjustment when kidneys are damaged, but weekly cisplatin is somewhat low dose so that there is a controversy on reducing the dose. And it is known that the cumulative dose of cisplatin is the most important factor contributing to renal impairment. Therefore, clinicians face troubles whether or not to maintain the chemotherapy. Here, we reported a patient whose renal function (eGFR) had been decreased as the number of chemotherapy increased, so her chemotherapy should be stopped. Since we held the chemotherapy on her, the disease progressed aggressively. Weekly cisplatin regimen is just 25 mg/m², so it may be meaningless to reduce this dose, and it is well known that cumulative dose of cisplatin is the most important factor contributing to renal impairment, it is better not to use cisplatin anymore. Therefore, we recommend that if the patient responds well to weekly cisplatin plus gemcitabine regimen, it would be beneficial to use gemcitabine alone.

• 한국임상수의학회지
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• 제17권1호
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• pp.270-274
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• 2000

A seven-year-old female Jindo-dog was presented with a history of progressive abdominal distension. Except for severe bilateral abdominal swelling, other abnormal signs were not detected. The patient showed normal appetite and defecation. In the radiographic examination, the abdomen was filled with large masses. Suspected a certain neoplastic disease, laparotomy was taken through the cranial abdominal midline. Large pale-yellow masses were proliferated to fill the abdomen. In the masses, grey-brown or black portion presumed hemorrhagic or necrotic spots were found. Even though neoplastic tissues were not detected in the right kidney, they were infiltrated in the left kidney except for a part of the cortex. Obtaining the owner's consent, the patient was euthanized and samples were collecte for further study. In microscopic examination, the parenchyma of the medulla was substituted with tumor cells and the cortex was impressed by the expansive proliferation of the neoplastic tissues. This neoplasm was estimated as renal carcinoma originated from tubular epithelium, being based upon that tumor cells were largely cuboidal cells and they had obscure tubular forms.

• 동아시아식생활학회지
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• 제13권5호
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• pp.408-424
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• 2003

The purpose of this study was to analyze the nutritional status and dietary habits in predialysis patients of chronic renal failure(CRF). The patients group was composed of total 35 persons with chronic renal failure(diabetes exclusion), male 20, female 15 who were treated in the kidney internal department and the control group also composed of 35 persons, male 18, female 17 who were classified as normal by the medical examination. Their dietary habits, nutritional status and nutritional knowledge were investigated from two general hospitals in Inchon, middle of this year 2002. There were 31.4% of low weight patients (BMI below 20), 77.1% of anemia patients (serum hemoglobin below 12g/㎗), 6S.6% of hypertension patients with diastolic blood pressure over 90mmHg, 80% with systolic blood pressure over 140mmHg, 20% of hypercholesterolemia patients (serum cholesterol over 230mg/㎗), and 22.9% of hyperlipemia patients (serum triglyceride over 200mg/㎗). The cardiovascular disease seemed to be caused by the abnormality of lipid metabolism. The possibility of the bone disease was shown from patients of hyperphosphatemia (serum phosphorus over 4.7mg/㎗, 22.9%) and hypocalcemia (serum calcium below 8.4mg/㎗, 25.7%). Intake of insufficient calories which was caused by the lack of appetite affected on the nutritional status. The intake of most nutrients was not significantly different from the RDA for Koreans. Consequently, the patient groups took a lot of salt even after the diagnosis of CRF. But patients ate 6.lg of salt which were more than the recommended amount 2∼4g for patients with CRF. The patient groups, who had the experiences of nutritional counselling, had significantly higher nutritional knowledge related to CRF than control group. Unfortunately, patients could not have enough chances for nutritional counselling by the nutritionist even though they needed the nutritional informations and dietetic treatments. The continuous research is expected with regard to the detail plan for the improvement of nutritional support and the nutritional counselling because it is important to decide the requirements of nutrients for patients with kidney disease, considering the kidney function and status of nutrition.

• 대한유전성대사질환학회지
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• 제1권1호
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• pp.23-27
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• 2001

Fanconi-Bickel Syndrome (FBS) is a rare autosomal recessive disorder of carbohydrate metabolism recently demonstrated to be caused by mutations in the GLUT 2 gene for the glucose transporter protein 2 expressed in liver, pancreas, intestine, and kidney. This disease is characterized by hepatorenal glycogen accumulation, both fasting hypoglycemia as well as postprandial hyperglycemia and hyperglactosemia, and generalized proximal renal tubular dysfunctions. We report the first Korean patient with FBS diagnosed based on clinical manifestations and identification of a novel mutation in the GLUT 2 gene. She was initially diagnosed having a neonatal diabetes mellitus due to hyperglycemia and glycosuria at 3 days after birth. In addition, newborn screening for galactosemia revealed hypergalactosemia. Thereafter, she has been managed with lactose free milk, insulin therapy. However, she failed to grow and her liver has been progressively enlarging. Her liver functions were progressively deteriorated with increased prothrombin time. Liver biopsy done at age 9 months indicated micronodular cirrhosis with marked fatty changes. She succubmed to hepatic failiure with pneumonia at 10 months of age. Laboratory tests indicated she had generalized proximal renal tubular dysfuctions; renal tubular acidosis, hypophosphatemic rickets, and generalized aminoaciduria. Given aforementioned findings, the diagnosis of FBS was appreciated at age of 2 months. The DNA sequencing analysis of the GLUT 2 gene using her genomic DNA showed a novel mutation at 5th codon; Lysine5 Stop (K5X).