• Childhood Kidney Diseases
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• v.2 no.1
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• pp.69-72
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• 1998

Thromboemolism is one of the severe complications of nephrotic syndrome. And arterial thromboembolism is rare than venous thromboembolism. Hypercoagulability is the main pathophysiologic factors of thromboembolism in nephrotic syndrome with severe hypoalbuminemia. We experienced one case of arterial thromboembolism which occured in right common iliac artery. It was seen in a 6 year-old male child that presented with generalized edema and rigth ankle joint pain. Emergency embolectomy and anticoagulant therapy (heparin and antithrombin III) was performed. He didn't have to be amputated and recovered to self ambulation. This is an uncommon case that successful recovery was possible by early diagnosis and invasive surgical management with proper anticoagulant therapy.

• Childhood Kidney Diseases
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• v.10 no.2
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• pp.238-243
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• 2006

Nephrotic syndrome in childhood is known to be associated with a hypercoagulable state and thromboembolic complications, among which cerebral venous thrombosis is a very rare and serious one, with only a few isolated reports in the literature. A 9-year-old boy with known nephrotic syndrome was admitted due to a relapse with massive proteinuria and generalized edema. He complained of a prolonged frontal headache. The enhanced brain magnetic resonance imaging(MRI) showed a high signal in the region of the superior sagittal sinus and right transverse sinus consistent with a thrombus. He was managed with steroids, cyclosporine and warfarin. His headache subsided 2 weeks later and proteinuria resolved 1 month later. An MRI 2 months later was normal. We describe this case and review the literature to emphasize the importance of recognizing this potentially life threatening complication and initiating anticoagulation therapy.

• Childhood Kidney Diseases
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• v.13 no.2
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• pp.271-277
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• 2009

Kimura's disease is a chronic inflammatory disorder of unknown etiology. A 14 year old boy suffering from steroid dependant nephrotic syndrome, was presented with relapsing painless subcutaneous masses on the left buccal area. Blood analysis showed increased IgE and eosinophilia. During 4 years follow up, he was been treated by low dose steroid and short term cyclosporine. Consequently, frequent relapses of subcutaneous masses and nephrotic syndrome has been relieved. Cyclosporine treatment combined with steroid may be useful for preventing frequent relapse of Kimura's disease.

• Journal of Yeungnam Medical Science
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• v.33 no.1
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• pp.25-28
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• 2016

Nephrotic syndrome is associated with a hypercoagulable state, which results in thromboembolism as one of its main complications. Various pathogenetic factors that cause the hypercoagulable state in nephrotic syndrome have been recognized. We report on a 19-year-old female with a minimal-change disease who developed pulmonary thromboembolism combined with intracardiac thrombus while on tapering steroid. Our patient showed hypoalbuminemia with an episode of shock, and was successfully treated with thrombolysis and anticoagulation therapy.

• Childhood Kidney Diseases
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• v.1 no.1
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• pp.79-81
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• 1997

Minimal change nephrotic syndrome is characterized by proteinuria, hypoproteinemia, edema, and hyperlipidemia. Children with onset of nephrotic syndrome between the age of 1 and 8 year are likely to have steroid response to minimal chage disease, but we experienced one case of minimal change disease which failed to respond to steroid therapy at beginning and subsequently developed acute renal failure. It was seen in a 5 year-old male child that presented with edema and gross hematuria. Peritoneal dialysis was performed for acute renal failure for 11 days. Patient was completely recorvered from acute renal failure and renal biopsy was done at 27th day after onset of disease which revealed typical picture of minimal change disease complicated by acute tubular necrosis. We beleive this case is very unusual and it may be the first case in the literature in terms of pediatric cases.

• Clinical and Experimental Pediatrics
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• v.59 no.sup1
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• pp.72-75
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• 2016

Eosinophilic gastroenteritis is a rare disease characterized by prominent eosinophilic tissue infiltration of the gastrointestinal tract. Here, we report a case of eosinophilic gastroenteritis in an 18-year-old patient with prolonged nephrotic syndrome who presented with abdominal pain and peripheral hypereosinophilia. During the previous 2 years, he had visited local Emergency Department several times because of epigastric pain and nausea. He had been treated with steroid-dependent nephrotic syndrome since 3 years of age. Tests ruled out allergic and parasitic disease etiologies. Gastroduodenoscopy with biopsy revealed marked eosinophilic infiltration in the duodenum. Renal biopsy findings indicated minimal change disease spectrum without eosinophilic infiltration. The oral deflazacort dosage was increased, and the patient was discharged after abdominal pain resolved. To our knowledge, this is the first report of eosinophilic gastroenteritis in a patient with minimal change disease.

• Childhood Kidney Diseases
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• v.5 no.2
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• pp.206-209
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• 2001

Clq nephropathy is an immune complex glomerulonephritis defined by the presence of mesangial Clq deposits in immunofluorescence microscopy and electron dense deposits on electron microscopy. It was described as a distinct disease entity in 1985 by Jennette and Hipp. Thirty four cases were reported in the literature but there has been no pediatric case reported in Korea yet. It commonly presents with steroid- resistent nephrotic syndrome in older children and young adults, and occasionally nephritic-nephrotic syndrome or rapidly progressive glomerulonephritis We report a case of Clq nephropathy in a 23-month-old girl with steroid-dependent nephrotic syndrome. (J. Korean Soc Pediatr Nephrol 2001;5 : 206-9)

• Childhood Kidney Diseases
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• v.10 no.2
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• pp.132-141
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• 2006

Purpose : This study was aimed to determine the predictive risk factors for the treatment response and relapse rate in children diagnosed with idiopathic nephrotic syndrome. Methods : We analyzed the medical records of children who were diagnosed and treated for childhood idiopathic nephrotic syndrome from November 1991 to May 2005. Variables selected in this study were age at onset, sex, laboratory data, concomitant bacterial infections, days to remission, and interval to first relapse. Results : There were 46 males and 11 females, giving a male:female ratio of 4.2:1. The age($mean{\pm}SD$) of patients was $5.8{\pm}4.1$ years old. Of all patients who were initially given corticosteroids, complete remission(CR) was observed in 54(94.7%). Of the 54 patients who showed CR with initial treatment, 40(70.2%) showed CR within 2 weeks and 14(24.6%) showed CR after 2 weeks. The levels of serum IgG were lower in the latter group who showed CR after 2 weeks(P=0.036). Of the 54 patients who showed CR with initial treatment, 47(82.5%) relapsed. Of these patients, 35.1% were frequent relapsers and 43.9% were infrequent relapsers. There was no significant correlation between the frequency of relapse and the following variables : sex, days to remission, and laboratory data. However, age at onset and interval to first relapse had a negative correlation with the frequency of relapse(Pearson's coefficient=-0.337, -0.433, P<0.012, P<0.01). Conclusion : The age at onset and the interval to first relapse were found to be predictive clinical parameters for the relapse rate, while the levels of serum IgG at initial presentation were a predictive laboratory factor for treatment response in childhood idiopathic nephrotic syndrome.

• The Korean Journal of Physiology and Pharmacology
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• v.13 no.1
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• pp.1-7
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• 2009

Sodium retention is a hallmark of nephrotic syndrome. We investigated whether sodium retention is associated with changes of natriuretic peptide system at different stages (i.e., a sodium retaining stage and a compensatory stage) of nephrotic syndrome. At day 7 after PAN(puromycin aminonucleoside) injection, the urinary excretion of sodium was decreased, along with the development of ascites and positive sodium balance. The plasma and urinary ANP(atrial natriuretic peptide) immunoreactivities were increased. ANP mRNA expression was increased in the heart and kidney, whereas that of NPR(natriuretic peptide receptor)-A and NPR-C mRNA was decreased in the kidney. The expression of NEP was decreased in the kidney. At day 14, urinary excretion of sodium did not differ from the control. The plasma ANP level and heart ANP mRNA expression returned to their control values. The expression of ANP mRNA in the kidney was increased in association with increased urinary ANP immunoreactivities. The expression of NPR-A in the kidney became normal, whereas that of NPR-C kept decreased. The expression of NEP(neutral endopeptidase) remained decreased. These findings suggest that the increased renal ANP synthesis in association with decreased metabolism via NEP and NPR-C may play a compensatory role against the development of sodium retention in nephrotic syndrome. The decreased of NPR-A expression in the kidney may contribute to the ANP resistance at day 7. The subsequent recovery of NPR-A expression may play a role in promoting sodium excretion in later stage(at day 14).

• Child Health Nursing Research
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• v.4 no.2
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• pp.221-230
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• 1998

This study was designed to identity the degree of burden felt by mothers of children with nephrotic syndrome. Also, relations between the subject characteristics and burden were investigated to provide basis data for their family health and nursing intervention. The study subjects were composed of 70 mothers of nephrotic syndrome patients whose children were hospitalized in 2 Pediatric wards of University Hospital in Seoul and 1 in Pusan from Mar. 2nd, 1998 to May. 30th in the same year The questionnaires were used which dealt with burden of mothers. the questionnaires for this study designed and used by researcher placed their basis in Burden Measurement Instrument developed by Montgomery et. al (1985) and the reliability of the used instrument was .78. The data analysis was done by SPSS, t-test, ANOVA and Stepwise Multiple Regression. The results of were as follows. 1. Mean score of burden of subjects was 60.82(Maximum 86, standard deviation 1.244). 2. Of the mothers characteristics, the score of burden was high in case of no religion and low income. 3. Of the patients characteristics, the score of burden ranked as high in MCNS, doing oral therapy and injection therapy at the same time, and negative perceived patients' condition. 4. The degree of burden felt by mothers of children with nephrotic syndrome was significantly predicted by the level of pt's diagnosis(11%), pt's condition(8%), economic state (6%) and pt's sex (5%), respectively. In conclusion to above study, the researcher suggests that the development of instrument for measurement of burden is in much need. Relations between burden and social support should be studied to lessen burden of mothers of children with nephrotic syndrome.