• Childhood Kidney Diseases
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• v.10 no.1
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• pp.52-57
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• 2006

Kimura's disease is a rare chronic inflammatory disease of unknown etiology which appears primarily in young Asian males as non-tender subcutaneous swellings in the head and neck region. Histologic characteristics are the presence of lymphoid follicles, vascular proliferation and infiltration of eosinophils. Peripheral eosinophilia and elevated serum IgE are frequently combined. Systemic steroid therapy with surgical excision is the mainstay of treatment, though recurrence after surgery or discontinued steroid treatment is common. It has been known that about 16% of the cases are associated with renal diseases, particularly nephrotic syndrome. We present an 8-year-old boy with a past history of steroid-responsive, infrequently relapsing nephrotic syndrome who developed right buccal swelling and peripheral eosinophilia during the remission state. He has been managed with surgical resection, steroid and cyclosporine due to multiple recurrences.

• Child Health Nursing Research
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• v.7 no.3
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• pp.280-297
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• 2001

The purpose of the study was to develop and test the model for the quality of life in mothers of children with nephrotic syndrome. A hypothetical model was constructed on the basis of previous studies and a review of literature. The conceptual framework was built around ten constructs. Exogenous variables included in this model were mother's health, father's health, marital intimacy, mother's attitude on children, economic state, side effect of steroid, severity of illness and social support. Endogenous variables were mother's burden and quality of life. Empirical data for testing the hypothetical model were collected by using a self-report questionnaire from 152 mothers of children with nephrotic syndrom at the outpatient clinics and in the hospital. The data was collected from May, 1999 to August, 1999.Reliability of the seven instruments was tested with Cronbach's alpha which ranged from 0.71 - 0.92.For the data analysis, SPSS 8.0 WIN program and LISREL 8.20 WIN program were used for descriptive statistics and covariance structural analysis. The results of covariance structural analysis were as follow :1. The hypothetical model showed a good fit with the empirical data. [x2 = .56, df = 3, p = .90(p>.05 ), GFI = .99, AGFI = .99, RMSR = .005.] 2. For the parsimony of model, a modified model was constructed by deleting 1 variable and excluding 2 paths according to the criteria of statistical significance and meaning.3. The modified model also showed a good fit with the data[x2 = 2.83, df = 7, p = .90( p>.05 ), GFI = 1.00, AGFI = .97, RMSR = .011].The result of the testing of the hypothesis were as follows : 1. Mother's health(γ21 = .26, t = 4.16), father's health(γ22 = .19, t = 2.92), marital intimacy(γ23 = .26, t = 4.13) and social support(γ28 = .12, t = 2.03) had a significant direct effect on the quality of life.2. Mother's burden(β21 = -.20, t = -3.10) had a significant negative direct effect on the quality of life.3. Mother's attitude on children(γ14 = -.34, t = .-4.57), mother's health(γ11 = -.22, t = -2.96) and side effect of steroid (γ16 = -.23, t = .-2.69) had a significant direct negative effect on the burden. The result of this study showed that mother's health, marital intimacy, mother's burden, father's health, and social support had a significant direct effect on the quality of life. Mother's attitude on children, mother's health, and side effect of steroid had a significant direct effect on mother's burden. These six variables, mother's health, marital intimacy, father's health, social support, mother's attitude on children and side effect of steroid were identified as relatively important variables. The results of this study suggest, it needed to determine the nursing intervention will alleviate mother's burden and promote a greater quality of life in mothers of children with nephrotic syndrom.

• Research in Community and Public Health Nursing
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• v.17 no.1
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• pp.125-133
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• 2006

Purpose: This study was performed to identify the relationship between PMS and sex role. Method: Data were collected from 417 female college students in Seoul and Gyeonggi-do area during the period from the 6th of September to the 15th of October in 2004. Collected data were analyzed using SPSS WIN 11.0. Result: The results of this study were as follows: 1) Perceived subjective symptoms were clustered endogenous depressive feature ($2.76{\pm}.97$), anxiety ($2.76{\pm}1.09$), and impulsivity ($2.72{\pm}1.02$). 2) PMS was significantly different according to the general characteristics of menarche age (F=5.910, p=.003), pain (F=7.886. p=.000) and family history (F=5.366, p=.005), masculinity was significantly different according to menarche age (F=3.174, p=.043), and femininity was significantly different according to menarche age (F=3.742, p=.025) and pain (F=3.256. p=.040). 3) Femininity and PMS were in a positive correlation with each other (r=.623. p=.000). Conclusion: This results showed that there is a significant correlation between PMS and sex role. For future research, it is recommended to identify major factors affecting PMS and the relationships between them and various subjects.

• Journal of the Korean Academy of Child and Adolescent Psychiatry
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• v.8 no.1
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• pp.133-138
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• 1997

Prader Willi Syndrome(PWS) was first recognized and reported by Prader-Willi. The etiology of the syndrome is not fully understood, but 50-70% of the patients show small deletion in chromosome 15. Manifested symtoms vary according to developmental age. In early life, hypotonia, areflexia, feeding difficulties, hypothermia, microgenitalia, hypoplastic scrotum, cryptochordism were observed. But in several years, hypotonia disappears, and polyphagia, decreased satiety, psychomotor retardation, obesity, hypogonadism and short stature become main problems. Behavioural problems including temper and aggressive outbursts, stealing food, hoarding food, and self excoriating skin picking, trichotillomania are more prominent during adolescence and young adulthood. Also, irritable, depressed mood are described. Lots of psychological and behavioural problems explain the reason why psychiatrists have managed and reported this syndrome. However, there has been no official report of PWS in our country. So authors report the clinical characteristics and issues in management of a patient with PWS.

• Childhood Kidney Diseases
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• v.16 no.2
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• pp.121-125
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• 2012

Gitelman syndrome is an autosomal recessive renal tubular disorder characterized by hypokalemic metabolic alkalosis, and it is distinguished from Batter syndrome by hypomagnesemia and hypocalciuria. This disorder is caused by mutation in SLC12A3 gene which encodes thiazide-sensitive $Na^+-Cl^-$cotransporter (NCCT) which is expressed in the apical membrane of cells, lining distal convoluted tubule. A 8-year old boy who presented with Rolandic epilepsy, and horseshoe kidney accidentally showed clinical features of metabolic alkalosis, hypokalemia, hypocalciuria without hypomagnesemia. So we identified a heterozygote mutation and an abnormal splicing in the SLC12A3 gene, encoding NCCT. The mutation was detected in the exon 15 and 22 of SLC12A3 gene.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.13 no.1
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• pp.37-42
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• 2017

Noonan syndrome is a relatively common genetic disorder which is autosomal dominant. Prevalence of Noonan syndrome is varying from 0.04% to 0.1%. It is characterized by distinctive facial features, chest deformity, short stature and congenital heart disease. Oral findings in patient with Noonan syndrome include high arched palate, dental malocclusion, articulation difficulties, and micrognathia. The purpose of this case report is to describe dental treatment of a children with Noonan syndrome. 5 year old boy with Noonan syndrome visited to the Seoul National University Dental Hospital for dental treatment. Due to need for close monitoring, concern about seizure and poor cooperation, we planned to perform the dental treatment under general anesthesia. Under general anesthesia, caries treatment was successfully performed and there was no postoperative complications related to general anesthesia. High arched palate was observed which is characteristic in Noonan syndrome.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.14 no.1
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• pp.17-21
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• 2018

Williams syndrome (WS) is a rare congenital disorder which is caused by microdeletion of approximately 1.6 MBP from the long arm of chromosome 7 at 7q11.23. It is characterized by cardiovascular anomalies, elfin face and mental retardation. The most typical oral signs in patient with WS are hypodontia, reduced mesio-distal dimensions both in the primary and permanent teeth, macroglossia, excessive interdental spacing, enamel hypoplasia and enamel hypomineralization. The majority of children with WS have mild to moderate mental retardation, generalized anxiety disorder, hyperactivity disorder and sensitivity to sounds. The purpose of this presentation is to describe dental treatment for a child with WS. A 9-year-old boy diagnosed with WS had caries on his first permanent molars. Because of the poor cooperation, these teeth were filled temporarily with glass ionomer, and treatment under general anesthesia was planned. Under general anesthesia, caries treatment of first permanent molar and extraction of primary molar was successfully performed and there was no postoperative complications related to general anesthesia. Open bite, hypodontia, excessive dental space, enamel hypoplasia, enamel hypomineralization were observed which were characteristic in WS.

• Childhood Kidney Diseases
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• v.7 no.2
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• pp.211-216
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• 2003

An epidemic outbreak of food poisoning due to enterohemorrhagic Escherichia coli(EHEC) occurred throughout Korea from May to September, 2003. Patients infected via contaminated school lunch foods were found mainly in Seoul and Gyeonggi province. As a result of this food poisoning, 36 patients were diagnosed as hemolytic uremic syndrome(HUS) up to mid-September, 2003 and five of them visited our hospital. Before the outbreak this year, we had experienced only 23 cases of HUS, including diarrhea associated (D+) and atypical (D-) forms, for the last 14 years, and there had been no case with pancreatic enzyme elevation. Unlike the past HUS cases, those of this year presented with severe abdominal pain and spiking elevation of pancreatic enzyme levels in 2 cases. We report 2 cases of transient pancreatitis combined with HUS in children during the epidemic outbreak in 2003 with a brief review of related literatures.

• Clinical and Experimental Pediatrics
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• v.52 no.1
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• pp.119-123
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• 2009

Renal cell carcinoma (RCC) arising from epithelial cells of the renal tubules is a highly aggressive and malignant tumor in all ages; however, it rarely occurs in children. the standard treatment for RCC is radical nephrectomy with lymph node dissection when the tumor is localized and can be completely resected. Adjuvant chemotherapy, radiotherapy, and immunotherapy are used for pediatric patients with advanced RCC involving lymph nodes or metastatic lesions. Sorafenib is an oral, multikinase inhibitor that has recently been approved for use in metastatic RCC. Common toxicities that have been reported include dermatologic changes such as rash or desquamation and hand-foot skin reaction, diarrhea, fatigue, alopecia, and hypertension. In particular, hand-foot syndrome (HFS) an erythematous skin lesion of the palms and solesis most often caused by cytostatic chemotherapeutic agents. In this report, we have studied a 14-year-old female patient with hand-foot syndrome that occurred in association with sorafenib for the treatment of metastatic RCC. Furthermore, this case demonstrates that reversal of complications can be achieved by discontinuing the drug and intervention with topical steroids, vitamin E, and high-dose pyridoxine.

• Childhood Kidney Diseases
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• v.17 no.2
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• pp.127-131
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• 2013

Urinomas can occur after renal trauma or perforation of the collecting system during an endosurgical procedure. However, spontaneous urinomas are very rare. Here we report a case of a spontaneous perinephric urinoma following the removal of a Foley catheter in an 18-year-old girl with acute kidney injury caused by septic shock. The patient had been treated for septic shock, acute kidney injury, and acute respiratory distress syndrome, and had a Foley catheter in place for seven days. After Foley catheter removal, the patient complained of consistent voiding difficulty. An abdominal computed tomography scan showed a large amount of left perinephric fluid, and the aspirated fluid included urothelial cells, confirming the diagnosis of a urinoma. The urinoma was successfully treated by insertion of a double-J stent into the left ureter. This report discusses the available literature on urinomas, and their clinical features, diagnosis, and treatment.