• Title/Summary/Keyword: Nephrology

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A Case of Hinman Syndrome Complicated by Chronic Renal Failure (만성 신부전을 초래한 Hinman 증후군 1례)

  • Lee Gyeong-Hoon;Lee Eun-Sil;Park Yong-Hoon
    • Childhood Kidney Diseases
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    • v.2 no.1
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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.

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A Case of Primary Fanconi Syndrome (일차성 Fanconi 증후군 1례)

  • Kang Hyeon-Ho;Cha Seong-Ho;Cho Byung-Soo
    • Childhood Kidney Diseases
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    • v.2 no.1
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    • pp.82-85
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    • 1998
  • Fanconi syndrome is a renal disorder characterized by a generalized dysfuntion of the proximal tubule leading to excessive urinary losses of amino acids, glucose, phosphate, and bicarbonate. It is often associated with hypokalemia, hypophosphatemia, rickets, and osteomalacia. We have experienced one case of primary Fanconi syndrome. The patient was a 10 year old boy and his chief complaints were short stature, glycosuria, and genu valgum. There were aminoaciduria, hypokalemia, glycosuria, decreased TRP, and hypophosphaturia. We report a case of primary Fanconi syndrome with brief review of the literature.

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A Case of Nephrotic Syndrome in Behcet Syndrome (신 증후군을 동반한 베체트 증후군(Behcet syndrome) 1례)

  • Juhng Woo-Suk;Kim Hye-Young;Lee Dae-Yeol
    • Childhood Kidney Diseases
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    • v.2 no.1
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    • pp.73-76
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    • 1998
  • Behcet syndrome is a multisystem disease complex of unknown cause. It is usually manifested by aphthous oral and genital ulcers, uveitis and skin eruption. Less frequently, CNS involvement, colitis, large vessel vasculitis, and myocarditis occur. Recently, several studies have reported renal manifestations of amyloidosis and focal necrotizing glomerulonephritis in Behcet syndrome. We describe a patient with Behcet syndrome who experienced nephrotic syndrome with focal effacement of epithelial foot process in glomeruli. A brief review of literature ensues.

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Two Cases Suggesting the Relationship of IgA Nephropathy and Henoch-$Sch{\ddot{o}}nlein$ Purpura (IgA Nephropathy와 Henoch-$Sch{\ddot{o}}nlein$ Purpura가 동일 병인임을 시사하는 2례)

  • Jeong Dong-Ho;Song Chang-Ju;Kim Deok-Su;Ha Tae-Sun
    • Childhood Kidney Diseases
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    • v.5 no.1
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    • pp.59-63
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    • 2001
  • There are a considerable number of reports suggesting a common pathogenesis of IgA nephritis(IgANn) Henoch-$Sch{\ddot{o}}nlein$ Purpura(HSP). In previous reports, a patient develops IgAN after kidney transplantation for HSP nephritis, one of Identical twin boys, developed IgAN and the other HSP, and a boy with IgAN later developed HSP. We report two cases, one with IgAN who later developed HSP and the other with HSP who later developed IgAN, suggesting that IgAN and HSP have a common pathogenesis. (J. Korean Soc Pediatr Nephrol 5 : 59- 63, 2001)

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A Case of McCune-Albright Syndrome with Vitamin D Resistant Rickets (비타민 D 저항성 구루병을 동반한 McCune-Albright 증후군 1례)

  • Kang Hee;Choe Jeong-Hoon;Hong Young-Sook;Lee Joo-Won;Kim Soon-Kyum;Yoo Kee-Hwan
    • Childhood Kidney Diseases
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    • v.3 no.2
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    • pp.237-240
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    • 1999
  • The presence of polyostotic fibrous dysplasia of bone, hyperpigmented skin macules, and precocious sexual development in children is known as the McCune-Albright syndrome. In addition to the described in McCune-Albright syndrome, other endocrinopathies have been reported including hyperthyroidism, acromegaly, Cushing syndrome and vitamin D resistant rickets. The case describes a 6-year-old boy showing bony deformities due to polyostotic fibrous dysplasia, hyperpigmented skin macules, hyperthyroidism and vitamin D resistant rickets. The purpose of this report is to describe a patient of McCune-Albright syndrome with vitamin D resistant rickets which is very rare.

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Two Cases of Hemolytic Uremic Syndrome Associated with Pneumococcal Infection (폐렴구균 감염에 동반된 비전형적 용혈성 요독 증후군 2례)

  • Jo Seung-Heui;Park Kyung-Mi;Ha Il-Soo;Cheong Hae-Il;Choi Yong
    • Childhood Kidney Diseases
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    • v.3 no.2
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    • pp.227-231
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    • 1999
  • Hemolytic uremic syndrome is a clinical syndrome with various etiology and pathogenesis. And pneumococcal neuraminidase has been known to play a pathogenetic role in some cases with this syndrome. We experienced two children with hemolytic uremic syndrome complicated by pneumococcal infection. One was 21-month-old girl with pneumococcal pneumonia, and the other was 7-month-old girl with pneumococcal meningitis and sepsis. Both of them showed typical clinical manifestations of hemolytic uremic syndrome with prolonged anuria during the course of pneumococcal infection. The renal functions of both cases did not recovered after resolution of acute hemolytic episode and chronic renal failure developed.

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Two Cases of Nephrotic Syndrome Associated with Kimura Disease (Kimura 병에 병발한 신증후군 2례)

  • Hahn Hye-Won;Park Kyung-Mi;Ha Il-Soo;Choi Yong;Cheong Hae-Il
    • Childhood Kidney Diseases
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    • v.3 no.2
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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.

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Neutrophil Gelatinase-Associated Lipocalin and Kidney Diseases

  • Yim, Hyung Eun
    • Childhood Kidney Diseases
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    • v.19 no.2
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    • pp.79-88
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    • 2015
  • Neutrophil gelatinase-associated lipocalin (NGAL) has emerged as one of the most promising biomarkers of renal epithelial injury. Numerous studies have presented the diagnostic and prognostic utility of urinary and plasma NGAL in patients with acute kidney injury, chronic kidney disease, renal injury after kidney transplantation, and other renal diseases. NGAL is a member of the lipocalin family that is abundantly expressed in neutrophils and monocytes/macrophages and is a mediator of the innate immune response. The biological significance of NGAL to hamper bacterial growth by sequestering iron-binding siderophores has been studied in a knock-out mouse model. Besides neutrophils, NGAL is detectable in most tissues normally encountered by microorganisms, and its expression is upregulated in epithelial cells during inflammation. A growing number of studies have supported the clinical utility of NAGL for detecting invasive bacterial infections. Several investigators including our group have reported that measuring NGAL can be used to help predict and manage urinary tract infections and acute pyelonephritis. This article summarizes the biology and pathophysiology of NGAL and reviews studies on the implications of NGAL in various renal diseases from acute kidney injury to acute pyelonephritis.

Urotherapy in Non-neurogenic Pediatric Voiding Dysfunction (소아 비신경인성 배뇨장애의 배뇨치료)

  • Baek, Min-Ki
    • Childhood Kidney Diseases
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    • v.16 no.1
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    • pp.15-20
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    • 2012
  • Non-neurogenic pediatric voiding dysfunction is defined as a problem during bladder filling or emptying without any neurogenic abnormality in children. The appropriate treatment of non-neurogenic pediatric voiding dysfunction is important because the disorder is frequently seen in clinical practice and might result in damages of the child's bladder or kidney. Urotherapy can be defined as nonsurgical nonpharmacological treatment for voiding dysfunction, categorized into standard urotherapy or specific intervention. Understanding of the underlying pathophysiology of non-neurogenic pediatric voiding dysfunction will lead to a change in management, from expensive and potentially harmful medications and invasive procedures to effective, noninvasive treatment of urotherapy.