• Title/Summary/Keyword: Nephrotic syndrome

Search Result 219, Processing Time 0.181 seconds

Can We Predict How Often Nephrotic Syndrome will Relapse into the Patients? (신증후군에서 스테로이드 반응성과 재발할 환자를 예측할 수 있을까?)

  • Namgoong, Mee Kyung
    • Clinical and Experimental Pediatrics
    • /
    • v.48 no.10
    • /
    • pp.1033-1037
    • /
    • 2005
  • Pediatric nephrotic syndrome is a well-known illness for its high relapsing rate. If we can predict the relapsing rate and the responses to the steroid therapy of individual patients with nephrotic syndrome, the predictability will be helpful in building a therapeutic plan. Here is my review of research articles on the risk factors for the prediction of relapsing nephrotic syndrome.

A Case of Infantile Nephrotic Syndrome with Focal Segmental Glomerulosclerosis (국소성 분절성 사구체 경화증에 의한 영아형 신증후군 1례)

  • Kim Se Eun;Han Young Sim;Song Min Seop;Chung Woo Yeong
    • Childhood Kidney Diseases
    • /
    • v.3 no.1
    • /
    • pp.100-103
    • /
    • 1999
  • Focal Segmental Glomerulosclerosis(FSGS) is one of the underlying pathology of congenital and infantile nephrotic syndrome. There is no ultimate curative treatment except renal transplantation. We have experienced a case of infantile nephrotic syndrome In a 10 month old boy who presented with proteinuria and hematuria. His elder brother also suffered from nephrotic syndrome and died at the age of 18 months due to sepsis. We have diagnosed this patient with clinical manifestations, laboratory data and pathologic findings which was done by open renal biopsy. The patient expired 54 days after admission because or progressive uremia and sepsis.

  • PDF

A Case of Peritoneal Empyema in a Patient with Nephrotic Syndrome due to Medical Neglect (신증후군에서 의료방임으로 발생한 복부농양 1례)

  • Shin Yun-Hye;Hur Joon;Lee Kang-Gyoon;Yim Hyun-Ee;Pai Ki-Soo
    • Childhood Kidney Diseases
    • /
    • v.9 no.1
    • /
    • pp.108-111
    • /
    • 2005
  • Nephrotic syndrome is a common chronic disease in childhood. Patients with nephrotic syndrome are at an increased risk of bacterial Infections due to immunological changes secondary to proteinuria. Primary bacterial peritonitis is one of the most serious and common infectious complications. The rate of peritonitis is, 2-6% and overwhelming infection still carries a mortality rate of 1.5%. We experienced a rare case of nephrotic syndrome complicated with severe peritonitis and Peritoneal empyema in a 10-year old girl after 2 months of medical neglect by parents. Here we emphasize thf: importance of early detection and treatment of peritoneal infection in nephrotic syndrome.

  • PDF

Clinical Evaluation of Nephrotic Syndrome Manifesting in the First Year of Life (1세 이하의 소아에서 발병한 신증후군의 임상적 고찰)

  • Cho, Sung-Hee;Lee, Joo-Hoon;Cho, Young-Mi;Park, Young-Seo;Cheong, Hae-Il
    • Childhood Kidney Diseases
    • /
    • v.13 no.2
    • /
    • pp.161-169
    • /
    • 2009
  • Purpose : This study was performed to report the diagnosis and treatment of nephrotic syndrome manifesting in the first year of life. Methods : We retrospectively reviewed the clinical data with chart review in 7 patients who were diagnosed as nephrotic syndrome manifesting in the first year of life from 1996 to 2007. Results : Three patients had congenital nephrotic syndrome, the other 4 patients had infantile nephrotic syndrome. Their ages ranged from birth to 11 months and male to female ratio was 1 to 6. Renal biopsies were done in 6 patients. One patient had Finnish type congenital nephrotic syndrome, 2 patients had diffuse mesangial sclerosis, 2 patients had focal segmental glomerulosclerosis and 1 patient had minimal change disease. Genetic analyses of NPHS2, PLCE1, and WT1 were done in 4 patients and 2 of them had WT1 mutation. Among 3 patients with congenital nephrotic syndrome, 1 patient was diagnosed as congenital nephrotic syndrome of Finnish type and the other 2 patients were diagnosed as Denys-Drash syndrome. All of the patients with congenital nephrotic syndrome died due to sepsis. Among 4 patients with infantile nephrotic syndrome, 2 patients died and 1 had remission, another patient progressed to end stage renal disease. Conclusion : Most of nephrotic syndrome manifesting in the first year was hereditary renal disease. Patients with nephrotic syndrome manifesting in the 3 month of life had poorer prognosis and needed more aggressive management including early dialysis and renal transplantation might be considered compared with infantile nephrotic syndrome. Further genotype-phenotype correlation studies are needed.

A Case of Nephrotic Syndrome with Swine-origin H1N1 Influenza Virus (H1N1 인플루엔자 바이러스 감염과 동반되어 발생한 신증후군 1례)

  • Kim, Sae-Yoon;Kim, Myoung-Uk;Lee, Sang-Su;Park, Yong-Hoon
    • Childhood Kidney Diseases
    • /
    • v.14 no.2
    • /
    • pp.218-222
    • /
    • 2010
  • Nephrotic syndrome is a clinical syndrome characterized by heavy proteinuria, hypoalbuminemia, edema and hyperlipidemia. Causes of idiopathic nephrotic syndrome include minimal change nephrotic syndrome (MCNS), focal segmental glomerulosclerosis (FSGS) and mesangial proliferation. Other causes of nephrotic syndrome are rare genetic disorders and secondary diseases associated with drugs, infections, or neoplasia. Since February 2009, a swine-origin H1N1 influenza virus (S-OIV) from Mexico has been spread among humans in unexpected rapidity. S-OIV is markedly different from seasonal influenza, in that many of those affected are previously healthy young people. While pulmonary complications of S-OIV infection have been frequently documented, renal complications have not been as widely recognized. We report a case of 4 year-old boy who had developed nephrotic syndrome after S-OIV infection with good response after steroid treatment.

A Case of Renal Vein Thrombosis Associated with Nephrotic Syndrome (방사선학적으로 확진된 신증후군과 동반된 신정맥 혈전증 1예)

  • Jung, Kyung-Hwa;Byun, Woo-Mok;Chang, Jae-Chun
    • Journal of Yeungnam Medical Science
    • /
    • v.4 no.2
    • /
    • pp.179-184
    • /
    • 1987
  • Renal vein thrombosis is usually a complication of multiple underlying renal disease rather than primary process. High incidence of renal vein thrombosis in patients with nephrotic syndrome, which suggest the nephrotic syndrome play a paramount role in the genesis of renal vein thrombosis or thromboembolic phenomena. But these are likely to relationship of "egg and chicken", and then we cannot determine what is primary of these. Recently authors experienced a case that was questioned renal vein thrombosis with nephrotic-syndrome clinically, laboratory and preliminary radiologically, and this case is confirmed by selective left renal venography. Here we report a case of renal vein thrombosis with nephrotic syndrome which successfully managed with oral anticoagulants and reviewed literatures.

  • PDF

A Case of Acute Acalculous Cholecystitis Superimposed on the Nephrotic Syndrome (미세 변화형 신증후군과 동반된 급성 무결석 담낭염 1례)

  • Shin Youn-Ho;Park Jee-Min;Shin Jae-Il;Kim Myung-Jun;Lee Jae-Seung
    • Childhood Kidney Diseases
    • /
    • v.7 no.1
    • /
    • pp.91-95
    • /
    • 2003
  • The nephrotic syndrome is characterized by generalized edema, hypoproteinemia(<2 g/dL), proteinuria(>40 $mg/m^2/hr$), and hypercholesterolemia(>200 mg/dL). It is reported that hypoalbuminemia, which is one of the four diagnostic criteria of the nephrotic syndrome, is associated with gallbladder wall thickening. An explanation for the thickened wall in hypoalbuminemic states is the accumulation of fluid in the subserosal layer of the gallbladder wall which contains the most areolar tissue. This report describes a patient who was initially diagnosed with the nephrotic syndrome at the age of 4 and subsequently developed acute acalculous cholecystitis at the age of 5.8 with an albumin level of 1.3 g/dL. The patient responded to fluid therapy, nasogastric suction, and broad spectrum antibiotics.

  • PDF

Genetic Basis of Steroid Resistant Nephrotic Syndrome

  • Park, Eujin
    • Childhood Kidney Diseases
    • /
    • v.23 no.2
    • /
    • pp.86-92
    • /
    • 2019
  • Steroid-resistant nephrotic syndrome (SRNS) has long been a challenge for clinicians due to its poor responsiveness to immunosuppressants, and rapid progression to end-stage renal disease. Identifying a monogenic cause for SRNS may lead to a better understanding of podocyte structure and function in the glomerular filtration barrier. This review focuses on genes associated with slit diaphragm, actin cytoskeleton, transcription factors, nucleus, glomerular basement membrane, mitochondria, and other proteins that affect podocyte biology.

A case of Galloway-Mowat syndrome with novel compound heterozygous variants in the WDR4 gene

  • Kim, Hamin;Lee, Hyunjoo;Lee, Young-Mock
    • Journal of Genetic Medicine
    • /
    • v.17 no.2
    • /
    • pp.97-101
    • /
    • 2020
  • The combination of central nervous system abnormalities and renal impairment is a notable characteristic of Galloway-Mowat syndrome (GAMOS), a disease which often accompanies microcephaly, developmental delay, and nephrotic syndrome. Many subtypes exist having various phenotypes and genotypes, and many genetic causes are still being identified. An 18-month-old boy first visited our clinic for seizure, delayed development, and microcephaly. During follow-up visits he developed proteinuria and nephrotic syndrome at the age of 6. Nephrotic syndrome became refractory to treatment. These phenotypes were suggestive of GAMOS. Next generation sequencing was performed for genetic analysis and revealed novel compound heterozygous variants in the WDR4 gene: c.494G>A (p.Arg165Gln) and c.540C>G (p.Ile180Met). This is the first case in Korea of GAMOS involving the WDR4 gene.

Acute Leukoencephalopathy During Cyclosporin A Therapy in a Pediatric Patient with Nephrotic Syndrome (신 증후군 환아에서 Cyclosporin A 치료 중 발생한 급성 백색질 뇌증 1례)

  • Jung Suk-Won;Rhee Kyung-Hwa;Kwon Young-Se;Kim, Soon-Ki;Son Byong-Kwan;Lee Ji-Eun
    • Childhood Kidney Diseases
    • /
    • v.9 no.1
    • /
    • pp.91-96
    • /
    • 2005
  • Cyclosporin A-induced central neurotoxicity has been rarely reported in patients with nephrotic syndrome. We report a pediatric patient who developed acute leukoencephalopathy diagnosed by MRI during CsA therapy for nephrotic syndrome.

  • PDF