• Childhood Kidney Diseases
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• 제11권1호
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• pp.112-117
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• 2007

저자들은 Leber의 선청성 흑암시를 진단받은 남매에서 성장 부전, 다음, 다뇨 및 야뇨증의 병력 없이 소아기에 만성 신부전으로 진행된 사춘기 콩팥 황폐증을 경험하여 우리나라에서는 드문 $Senior-L\ddot{o}ken$ 증후군을 보고하는 바이다.

• 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.

• Childhood Kidney Diseases
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• 제22권2호
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• pp.64-66
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• 2018

Autosomal dominant polycystic kidney disease (ADPKD) is the most frequent hereditary renal disease and causes terminal chronic renal failure. ADPKD is characterized by bilateral multiple renal cysts, which are produced by mutations of the PKD1 and PKD2 genes. PKD1 is located on chromosome 16 and encodes a protein that is involved in cell cycle regulation and intracellular calcium transport in epithelial cells and is responsible for 85% of ADPKD cases. Although nine cases of unilateral ADPKD with contralateral kidney agenesis have been reported, there have been no reports of early childhood ADPKD. Here, we report the only case of unilateral ADPKD with contralateral kidney dysplasia in the world in a four year-old girl who was intrauterinely diagnosed since she was 20 weeks old and followed for four years until present.

• 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.

• 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.

• Childhood Kidney Diseases
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• 제13권2호
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• pp.256-260
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• 2009

Russell-Silver 증후군은 자궁내 성장 지연, 특징적인 얼굴 기형, 저신장을 특징으로 하는 질환이다. Russell-Silver 증후군에서 동반되는 신질환은 말굽 신장, 신세뇨관 산증, 물콩팥증, 요관 깔때기막힘, 방광 요관 역류 등이 있다. 저자들은 Russell-Silver 증후군 환자에서 말기 신부전이 발생한 예를 경험하였으며 문헌 고찰에서 유사한 증례를 찾을 수 없었기에 보고하는 바이다.

• 대한수의사회지
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• 제32권10호
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• pp.627-633
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• 1996

• Childhood Kidney Diseases
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• 제3권2호
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• pp.217-220
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• 1999

Kimura disease is a chronic benign disorder, primarily seen in asians male during the second and third decades of life, which presents itself as a tumour like lesion with a predilection for the head and neck region. There is high prevalence of associated renal disease. We report two cases of nephrotic syndrome associated with Kimura disease, and this is the first report of Kimura disease with renal involvement in Korean children.

• Childhood Kidney Diseases
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• 제2권1호
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• pp.90-94
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• 1998

Hinman syndrome is a condition representing urinary voiding dysfunction in the neurologically intact child. The syndrome is probably caused by acquired behavioral and psychosocial disorders manifested by bladder and/or bowel dysfunction mimicking neurologic disease. Clinically, the symptom complex may include day and night time enuresis, encopresis, constipation, and recurrent urinary tract infections. Cystoscopy frequently demonstrates normal vesicourethral anatomy. Voiding films usually demonstarate a carrot-shaped proximal urethra with a persistent narrowing at the external sphincter. The bladder is large and often appears trabeculated with a thickened wall and significant postvoid residual. A 13-year-old male child was admitted due to fever, urinary tract infection, enuresis and flank pain. His neurologic examination was normal. Renal sonograms showed moderate hydronephrosis. Voiding cystourethrograms showed a huge, trabeculated bladder without vesicourethral reflux and urethral valves. No abnormal findings was found in spinal MRI.

• Clinical and Experimental Pediatrics
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• 제59권sup1호
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• pp.145-148
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• 2016

Takayasu arteritis (TA) is a chronic inflammatory disease of unknown etiology that affects mainly the aorta, main aortic branches, and pulmonary arteries. Diverse neurological manifestations of TA have rarely been reported in children. Posterior reversible encephalopathy syndrome (PRES) is a neuroradiological condition that presents with headache, seizure, visual disturbances, and characteristic lesions on imaging. Inflammatory condition and severe hypertension in TA can cause PRES. We report of a 5-year-old girl with presumed TA who presented with PRES and chronic total occlusion in the renal artery. The findings on magnetic resonance imaging suggested PRES. Left nephrectomy was performed for total occlusion of the left renal artery, and the confirmatory diagnosis of TA was based on the pathologic findings of the renal artery.