• Journal of Korean Academy of Nursing
• /
• v.29 no.1
• /
• pp.84-96
• /
• 1999

This study was conducted to identity the degree of burden and social support perceived by mothers of nephrotic syndrome patients. Also, relations between burden and social support were investigated to provide basis data for their family health and nursing intervention. The study subjects were mothers of nephrotic syndrome patients whose children were hospitalized in 2 Pediatric wards of University Hospital in Seoul and 1 in Pusan from Jun. 1, 1998 to Jun. 30, 1998. Burden measurement Instrument for this study was designed by the researcher and its basis in one developed by Montgomery et al.(1985) and the reliability was .78. Also. P.R.Q. Part I, II by Brandt and Weinert(1981) was used as social support measurement instrument and the reliability .71. The data analysis was done by SPSS, t-test, ANOVA Pearson correlation. The result were as follows. 1. Burden felt by mothers shows an average value of 60.82 (Maximum 86, Standard deviation 1.244). 2. Of the mother characteristics, the score of burden was high in case of no religion and low income. Of the patient characteristics, the score burden ranked as high MCNS, doing oral therapy, injection therapy at the same time and negative perceived patients condition. 3. The mean score of support was 77.54(Maximum 96, Standard deviation 1.096). 4. The main supporters were husband (the highest), brother, sister, health speciality and the subject expressed the highest satisfaction toward supporters in chronic disease. 5. Of the patient characteristics, the higher age group and the elder group showed high support. Also, positive perceived patient's condition, high support. 6. The relationship between burden and social support is not significant. In conclusion to the above study, the researcher suggests. 1. The Qualitative research to investigate influential factors on burden of family of nephrotic syndrome patients is needed.

• Korean Journal of Clinical Pharmacy
• /
• v.10 no.2
• /
• pp.51-56
• /
• 2000

Deflazacort, an oxazoline derivative of prednisolone, has been claimed to have anti-inflammatory effects with fewer side effects compared to prednisone. The objectives of the study were to evaluate efficacy and safety of deflazacort in children with nephrotic syndrome. Eligible Patients were the children with nephrotic syndrome who were treated with deflazacort from October. 1994 to April. 1999. Nephrotic syndrome was defined as having albumin level of less than 2.5 mg/dL and 24-hour urinary protein excretion of greater than $40\;mg/m^2/hr$. The primary parameters evaluating the efficacy of deflazacort were response rate, time to respond and relapse frequency. The safety profiles were the impact on children's growth, calcium sparing effect, glucose metabolism, lipid profile and adverse drug reactions. As results, total of 60 children were evaluated (47 boys, 13 girls). Response rate was $95\%$ (57/60) for initial and late responders. Median time to respond was 12 days (range 7-110 days) and median relapse frequency was one time (range 0-6). Weight/height ratio increased from $22.05\pm3.47\;to\23.20\pm3.44\;kg/m$ (p<0.001) and plasma calcium level, from $7.55\pm3.86\;to\;9.98\pm3.77\;mg/dL$ after treatment (p<0.001). Change of fasting glucose level was not statistically significant $(91.92\pm3.53\;vs.\;98.19\pm4.78\;mg/dL,\;p=0.072)$, while change of total cholesterol was significant $(362.3\pm12.0\;vs\;251.4\pm11.5\;mg/dL$, p<0.001). In conclusion, patients on deflazacort showed similar efficacy in treatment of nephrotic syndrome as reported for prednisone with less impact on growth inhibition and metabolic side effects of hyperglycemia and hyperlipidemia.

• Childhood Kidney Diseases
• /
• v.14 no.1
• /
• pp.42-50
• /
• 2010

Purpose : The clinical characteristics and associated anomalies in children with solitary kidney (SK) were analyzed retrospectively. Methods : Total 38 children diagnosed to have SK at our hospital between December 1989 and December 2009 were recruited, and the clinical records including imaging studies were retrospectively reviewed. SK was defined as unilateral renal agenesis by imaging studies only, and patients with regression of unilateral dysplastic kidney were excluded. Results : Among total 38 patients, 12 were male. The median age at the diagnosis of SK was 6.5 months (at birth-13 years). SK was detected by prenatal ultrasonography in 14 patients and during work-up for renal or urinary tract diseases in 13 (including urinary tract infection in 7). In 10 patients, SK was detected incidentally. Anomalies in the SK were noted in 17 patients including vesicoureteral reflux in 11. Other anomalies in the genitourinary tract were present in 16 patients, and multi-organ-involving syndromes or chromosomal anomalies were detected in 9. The mean duration of follow-up was 9 years (9 months-20 years). Two patients developed chronic renal failure during follow-up, and the median serum creatinine concentration of the remaining 36 at their last follow-up was 0.6 mg/dL. Conclusion : SK may be isolated and clinically asymptomatic; it is frequently accompanied by other anomalies in genitourinary tract and other organs, some of which can induce progressive renal dysfunction. Early recognition of associated anomalies with SK and regular follow-up is recommended to reduce long-term risk.

• Pediatric Gastroenterology, Hepatology & Nutrition
• /
• v.7 no.2
• /
• pp.228-238
• /
• 2004

Purpose: Obesity is rapidly increasing in Korean children. Obesity is a risk factor for cardiovascular morbidity and is frequently associated with hypertension, diabetes mellitus and coronary artery disease. This study was designed to evaluate risk factors of the metabolic syndrome in obese children. Methods: From February 2000 to June 2004, eighty eight obese (body mass index ${\geq}95th$ percentile) children aged 4 to 15 years were included. We measured serum lipid levels (total cholesterol, triglyceride, HDL cholesterol, LDL cholesterol), fasting sugar levels and insulin levels. Insulin resistance was determined by homeostasis model assessment, fasting insulin/glucose ratio and quantitative insulin sensitivity check index. Results: Clustering of risk factors for the metabolic syndrome in obese children demonstrated that 60.2% had more than one risk factors. Hypertension (14.8%), hypertriglyceridemia (14.8%), HDL-hypocholesterolemia (14.8%), LDL-hypercholesterolemia (12.5%) and hyperinsulinemia (12.5%) were observed. As BMI increased, there was statistically significant increase in systolic blood pressure, insulin and insulin resistance values. Insulin resistance was correlated to systolic blood pressure, serum lipid and insulin levels. The more risk factors for the metabolic syndrome obese children had, the higher was their insulin resistance. Conclusion: The increase in insulin resistance and clustering of risk factors for the metabolic syndrome are already apparent in obese children. Monitoring these risk factors for the metabolic syndrome should become a part of routine medical care for obese children.

• Clinical and Experimental Pediatrics
• /
• v.53 no.4
• /
• pp.592-597
• /
• 2010

Hyperimmunoglobulin E syndrome (HIES) is a rare immunodeficiency disease which is characterized by high serum IgE levels, eczema, and recurrent infections. Herein we present the case of a patient with HIES associated with STAT3 gene ($stat3$) mutation. A 16 year-old girl was admitted to our hospital due to hemoptysis caused by pneumonia with bronchiectasis. She had a history of recurrent skin and respiratory tract infections, such as pneumonia caused by MRSA (methicillin-resistant $Staphylococcus$ $aureus$) and $Pseudomonas$ $aeruginosa$. On physical examination, a broad round shaped nose, oral thrush, and chronic eczematous skin rash over her whole body were found. Laboratory data showed an elevated eosinophil count ($750/{\mu}L$) and total IgE level (5,001 U/mL). The patient's National Institutes of Health (NIH) score for HIES was 44. Direct sequencing of the STAT3 gene revealed that the patient was heterozygous for a missense mutation in the DNA binding domain of the STAT3 protein (c.1144C>T, p. Arg382Trp). HIES should be suspected in patients with recurrent infections and can be confirmed by clinical scoring and genetic analysis.

• Journal of the korean academy of Pediatric Dentistry
• /
• v.47 no.3
• /
• pp.344-351
• /
• 2020

Amelogenesis imperfecta (AI) occurs either in isolation or in association with other dental abnormalities and systemic disorder. A rare syndrome associating AI with nephrocalcinosis was named as Enamel Renal Syndrome (ERS; OMIM #204690). This syndrome is characterized by severe enamel hypoplasia, failed tooth eruption, intra pulpal calcifications, enlarged gingiva, and nephrocalcinosis. Nephrocalcinosis is a condition where calcium salts are deposited in renal tissue, and this may lead to critical kidney complications. This rare syndrome shows pathognomonic oral characteristics that are easily detectable at an early age, which proceeds the onset of renal involvement. Pediatric dentists are the first oral health practitioners whom ERS patients will meet at early age. The role of pediatric dentists is critically important for early diagnosis and referral of patients to both nephrologists for renal assessment and geneticists for identification of causative mutation and diagnosis. Early detection of renal involvement may provide chances to prevent further undesired renal complications.

• Childhood Kidney Diseases
• /
• v.6 no.2
• /
• pp.142-154
• /
• 2002

Purpose : One of the most important adverse effects of long-term cyclosporine therapy is nephrotoxicity, the morphologic changes of which include interstitial fibrosis and arteriolar hyalinization. Recently, several authors have shown that osteopontin plays an important role in the development of interstitial fibrosis by acting as a macrophage chemoattractant and stimulating the production of $TGF-{\beta}$ in experimental cyclosporine nephrotoxicity. However, the relationship between osteopontin and $TGF-{\beta}$ in humans has not been clearly documented so far. We studied the expression of osteopontin and $TGF-{\beta}$ in children with minimal change nephrotic syndrome treated with cyclosporine to demonstrate whether there is a relationship between cyclosporine toxicity and osteopontin expression as previously shown in animal models. Materials and methods : Nineteen children (15 males and 4 females) were the subject of this study. Renal biopsies had been performed before and after the cyclosporine therapy (mean duration: 15.9 months). In 5 patients, additional biopsies were performed after completing the cyclosporine treatment (mean; 26 months). The expressions of osteopontin and $TGF-{\beta}$ were evaluated by immunohistochemistry in the glomeruli and tubulointerstitium. Results : Osteopontin expression was significantly increased in the glomerular mesangium and tubules after cyclosporine treatment. But there was no statistically significant increase of $TGF-{\beta}$ in the interstitium. There was no significant increase in tubular osteopontin and interstitial $TGF-{\beta}$ expression in those cases developing interstitial fibrosis after cyclosporine treatment compared with cases those not developing interstitial fibrosis. No significant changes in osteopontin or $TGF-{\beta}$ expression were observed in subsequent 5 biopsy samples after discontinuation of cyclosporine compared with the first follow up biopsies. Conclusion : These results suggest that osteopontin is a nonspecific marker of renal injury rather than a mediator of interstitial fibrosis in cyclosporine nephrotoxicity of human.

• Korean Journal of Biological Psychiatry
• /
• v.8 no.1
• /
• pp.167-174
• /
• 2001

A 54-year old male patient who was suffering from bipolar I disorder for 19 years and was admitted to the National Bugok Mental Hospital due to a depressive episode, was referred to the Kosin University Gospel Hospital. On arrival at the emergency room, he had confused mentality with disorientation, memory impairment, hypomania, marked anxiety and hyperirritability. The change of neuromuscular activity such as ataxia, gait disturbance, tremor, shivering, myoclonus and epileptic seizures was also shown. In addition, the symptoms and signs of autonomic instability including diaphoresis, tachycardia, hypotension, fever and facial flushing were noticed. The above symptoms developed after the administration of sertraline successive to the discontinuation of fluoxetine without any washout period. The degree of severity seemed to be severe because he had epileptic seizures, fever and hypotension. He was recovered from the severe serotonin syndrome by the supportive symptomatic treatment with sodium valproate, clonazepam, lorazepam and cyproheptadine after cessation of the selective serotonin reuptake inhibitors during hospitalization. Therefore, this rare case of severe serotonin syndrome was reported and related literatures were also reviewed.

• Childhood Kidney Diseases
• /
• v.16 no.1
• /
• pp.46-50
• /
• 2012

C1q nephropathy is a distinct clinicopathologic entity, characterized by mesangial immunoglobulin and complement deposits, predominantly C1q, with no evidence for systemic lupus erythematosus. Clinically it may present as nephrotic syndrome and non-nephrotic proteinuria per se or associated with microscopic hematuria, gross hematuria, hypertension, or renal insufficiency. So far there is only one report about a familial case of C1q nephropathy (in two sisters). We present two cases of familial C1q nephropathy with nephrotic syndrome which was steroid resistant, but partially remitted with cyclosporine.

• Childhood Kidney Diseases
• /
• v.5 no.2
• /
• pp.210-212
• /
• 2001

Retinal detachment is a rare disease in children but cases of serous retinal detachment due to use of systemic corticosteroids have been reported in and out country Rhematogenous retinal detachment has developed in a child with oral and steroid pulse therapy due to nephrotic syndrome. He was treated with laser and buckling in right and left eye respectively. Currently he is under observation through the out patient department. (J. Korean Soc Pediatr Nephrol 2001 ; 5 : 210-12)