• Childhood Kidney Diseases
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• v.5 no.1
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• pp.9-14
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• 2001

Purpose : This study was undertaken to investigate clinical aspects of nutcracker syndrome in children including sexual distribution, age of onset, the ratio of peak flow velocity between the narrowed and dilated portions in the left renal vein and prevalence of hematuria or proteinuria. Materials and Method : The subjects included 30 patients diagnosed with nutcracker syndrome using Doppler sonography from September 1999 to January 2001 in Severance Hospital, Department of Pediatric Nephrology. The diagnostic criteria for nutcracker syndrome was a peak flow velocity of ratio is more than 5.0. Results : Patients consisted of 14 males and 16 females. The mean age was 9.19${\pm}$2.31 years old and the ratio of peak flow velocity was 8.52${\pm}$2.24. Age of onset us higher in males 9.68${\pm}$2.82 year old than in females, 8.76${\pm}$1.72 year old respectively(P<0.05). But there was no difference in the ratio of peak flow velocities ; 8.45${\pm}$2.31 in the male group , 8.58${\pm}$2.25 in the female group(P>0.05). There was no difference in the age of onset and the ratio of peak flow velocity between patients with hematuria and without hematuria. Among patients with nutcracker syndrome, 24 cases of hematuria, 3 cases of proteinuria only, and 3 cases of hematuria with proteinuria. Conclusion : In children of school age, early diagnosis of nutcracker syndrome and consistent follow up is worthy of consideration, especially ill the prevention of sequelae. (J. Korean Soc Pediatr Nephrol 5 : 9-14, 2001)

• Childhood Kidney Diseases
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• v.7 no.1
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• pp.52-59
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• 2003

Purpose : The renin-angiotensin system(RAS) plays an important role in renal growth and development. We have studied the prevalence of renal anomalies and documented the association between karyotype and renal anomalies using IVP and ultrasonography. Furthermore, to investigate the impact of RAS gene polymorphism on renal anomaly in Turner syndrome, we examined the ACE I/D genotype, angiotensinogen(AGT) gene M235T, angiotensin receptor type 1(ATR) gene A1166C. Methods : Cytogenetic analysis was performed in 33 Turner syndrome patients on peripheral blood lymphocytes. Ultrasonography(US) of the kidneys and collecting system and intravenous pyelography(IVP) were perfomed in all patients. Nuclear scintigraphy{Tc 99m dimercaptosuccinic acid(DMSA) scan} was also performed for the definite renal diagnosis if indicated. And, ACE I/D genotype, angiotensinogen(AGT) gene M235T, angiotensin receptor type 1(ATR) gene A1166C were examined by PCR amplification of genomic DNA samples. Results : The prevalence of renal anolmalies in Turner syndrome was 36.4%(12/33). The Karyotype 45, X was observed in 18 of the 33 girls(54.5%), of whom 8(44.4%) had renal anomalies. Mosaic karyotypes were observed in 11(33.3%) and four(12.2%) had a non-mosaic structural aberration of the X chromosome. In this group 4(25.7%) had renal anomalies. More renal anomalies were associated with the 45, X karyotype than those with mosaic/structural abnormalities of X chromosome, but the difference was not statistically significant(P>0.05). And, there was no significant differences in the RAS gene polymorphism and allele frequencies between renal anomaly group and normal group in Turner syndrome. Conclusion : The prevalence of renal anolmalies in Turner syndrome was 36.4%. There is no significant differences in the RAS gene polymorphism and allele frequencies between the renal anomaly group and the normal group in Turner syndrome.

• Clinical and Experimental Pediatrics
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• v.50 no.11
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• pp.1104-1109
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• 2007

Purpose : Since 1998, school urinary screening tests have been performed on Korean school children. We could detect and treat so many asymptomatic chronic renal disease in early stage. We investigated the efficacy of school urinary screening tests from children with membranoproliferative glomerulonephritis (MPGN) type I. Methods : We analyzed the characteristics and prognosis of 18 patients with MPGN type I who admitted after 1996 and received steroid therapy with or without cyclosporine. These patients were divided into two groups. Group A (asymptomatic patients detected by school urinary screening tests) consisted of 7 patients; Group S (symptomatic patients) consisted 11 patients. Results : Mean follow-up duration was 6.3 years (from 2 to 11 years). Urinary protein excretion was 1.1 g/day in group A and 6.6 g/day in group S. 24 hour creatinine clearance (mL/min/$1.73m^2$) was 134.3 in group A and 82.3 in group S. No patients in group A had renal insufficiency, but three patients in group S had renal insufficiency and one patient required peritoneal dialysis. Conclusion : Early detection by school urinary screening tests improves prognosis of MPGN type I.

• Clinical and Experimental Pediatrics
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• v.48 no.1
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• pp.68-74
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• 2005

Purpose : There is growing use of continuous renal replacement therapy(CRRT) for pediatric patients, but reports about the use and outcome of CRRT in children is rare in Korea. We report our experiences of CRRT in critically ill pediatric patients. Methods : We reviewed the medical records of 23 pediatric patients who underwent CRRT at Asan Medical Center between May 2001 and May 2004. We evaluated underlying diseases, clinical features, treatment courses, CRRT modalities and outcomes. Results : Ages ranged from three days to 16 years with a median of five years. Patients weighed 2.4 to 63.9 kg(median 23.0 kg; 10 patients ${\leq}20kg$). The underlying diseases were malignancy(nine cases), multiple organ dysfunction syndrome(five cases), hyperammonemia(four cases), acute renal failure associated with liver failure(three cases), dilated cardiomyopathy(one case) and congenital nephrotic syndrome(one case). Pediatric Risk of Mortality(PRISM) III score was $17.6{\pm}7.6$ and the mean number of failing organs was $3.0{\pm}1.7$. Duration of CRRT was one to 27 days(median : nine days). Eleven patients(47.8%) survived. Chronic renal failure developed in two cases, intracranial hemorrhage in one case, and chylothorax in one case among the survivors. PRISM III score and the number of vasopressor before the start of CRRT was significantly lower in the survivors($12.7{\pm}4.2$ and $0.9{\pm}1.1$) compared with nonsurvivors($22.1{\pm}7.8$ and $2.4{\pm}1.4$)(P<0.05). Conclusion : CRRT driven in venovenous mode is an effective and safe method of renal support for critically-ill infants and children to control fluid balance and metabolic derangement. Survival is affected by PRISM III score and the number of vasopressors at the initiation of CRRT.

• Childhood Kidney Diseases
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• v.9 no.2
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• pp.149-158
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• 2005

Purpose : To determine the histological findings and treatment outcome in cases of child hood nephrotic syndrome which required renal biopsy. Methods : We retrospectively reviewed the clinical, laboratory, pathologic findings and therapeutic outcomes of 159 nephrotic children who received a renal biopsy at the Department of Pediatrics, Kyunghee Medical University Hospital, Seoul from 1984 to 2004 over a period of 21 years. The renal biopsy was performed in nephrotic children who showed atypical features at presentation, or needed cytotoxic therapy because of frequent-relapsing, steroid-dependent, or steroid-resistant nephrotic syndrome(SRNS). Results : Minimal change disease(MCD) was found in 52.1$\%$ of the patients, followed by diffuse mesangial proliferation(33.1$\%$), focal segmental gomerulosclerosis(5.3$\%$), membranoproliferative glomerulonephritis(2.4$\%$), membranous nephropathy(2.4$\%$), and IgA nephropathy(1.8$\%$). In MCD children, 14.8$\%$ had hematuria, 22.7$\%$ had hypertension, 5.7$\%$ showed decreased renal function, and no patient was found to have an abnormal complement level. Among patients diagnosed with diseases other than MCD, 43.2$\%$ had hematuria, 21.0$\%$ was found to be hypertensive, 7.4$\%$ of children showed decreased renal function and only 3(3.7$\%$) had decreased complement level; the rates of hematuria and SRNS were found to be significantly higher than MCD patients. Among 37 SRNS patients, 30(81.0$\%$) showed a final remission state with long-term steroid therapy, including methylprednisolone pulse therapy, over 4 months, with or without cytotoxic therapy. Conclusion : Almost half of the cases of childhood nephrotic syndrome requiring renal biopsy were not diagnosed with MCD. Among atypical features, hematuria and steroid-resistance would be the most probable indicators for a diagnosis other than MCD. Even in patients with SRNS, long-term methylprednisolone pulse therapy may result in a good remission rate. (J Korean Soc Pediatr Nephrol 2005;9:149-158)

• Childhood Kidney Diseases
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• v.7 no.2
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• pp.125-132
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• 2003

Purpose : Lipoprotein(a) is a genetically determined risk factor for atherosclerotic vascular disease and is elevated in patients with renal disease. Especially the patients with nephrotic syndrome exhibit excessively high Lp(a) plasma concentrations. Also the patients with end-stage renal disease have elevated Lp(a) levels. But the mechanism underlying this elevation is unclear. Thus, in this study, by measuring the level of serum Lp(a) in common renal diseases in children, we hoped to see whether there would be a change in Lp(a) in renal diseases other than nephrotic syndrome. Then, we figured out its implications, and looked for the factors that affect the Lp(a) concentrations. Methods : A total of 75 patients(34 patients with hematuria of unknown etiology, 10 with hematuria and hypercalciuria, 8 with IgA nephropathy, 8 with poststreptococcal glomerulone phritis, 3 with $Henoch-Sch\"{o}nlein$ nephritis, 7 with urinary tract infection, and 5 with or- thostatic proteinuria) were studied. The control group included 20 patients without renal and liver disease. Serum Lp(a), total protein, and albumin levels, 24-hour urine protein and calcium excretions, creatinine clearance and the number of RBCs and WBCs in the urinary sediment were evaluated. Data analysis was peformed using the Student t-test and a P-value less than 0.05 was considered to be statistically significant. Results : LP(a) was not correlated with 24-hour urine calcium and creatinine. Lp(a) level had a positive correlation with proteinuria and negative correlation with serum albumin and serum protein. Among the common renal diseases in children, Lp(a) was elevated only in orthostatic proteinuria (P<0.05). Conclusion : Lp(a) is correlated with proteinuria, serum protein, and serum albumin, but not with any kind of specific renal disease. Afterward, Lp(a) needs to be assessed in patients with orthostatic proteinuria and its possible role as a prognostic factor could be confirmed.

• Annals of Clinical Neurophysiology
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• v.13 no.1
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• pp.31-37
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• 2011

Background: Carpal tunnel syndrome (CTS) and tarsal tunnel syndrome (TTS) are thought to share a similar pathophysiology, compression of the median and plantar nerve by the carpal tunnel and flexor retinaculum. A few reports introduced the relationship between idiopathic CTS and TTS without definite evidence of coexistence. The current study was designed to analyze the electrophysiologic characteristics of combined idiopathic CTS and TTS by comparing with each idiopathic CTS or TTS. Methods: We retrospectively collected patients with combined idiopathic CTS and TTS (CTS-TTS group) from June 2001 to February 2009. Patients with each idiopathic CTS or TTS were collected as controls. Electrophysiologic data of median and plantar nerves were compared between CTS-TTS group and controls. Results: CTS-TTS group was composed of 31 patients. Control group of each CTS or TTS were 50 CTS and 49 TTS patients. In comparison of median nerve conduction study between CTS-TTS group and CTS control group, decreased compound muscle action potential amplitude (p<0.001), decreased median sensory nerve action potential amplitude (p<0.001) and sensory nerve conduction velocity at finger stimulation (p=0.013) were prominent in CTS-TTS group. Decreased medial plantar sensory nerve action potential amplitude (p=0.034) was indicated when CTS-TTS groups and TTS control group were compared. Conclusions: If the electrophysiology study of patients with CTS or TTS was suggestive of severe degree of nerve injury, concerns about the possibility of combined CTS and TTS would be helpful.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.9 no.1
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• pp.42-45
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• 2013

De Grouchy syndrome or Distal 18q- is a genetic condition caused by a deletion of genetic material within chromosome 18, and the deletion involves the distal section of 18q. It causes a wide range of medical and developmental concerns. Congenital orthopedic anomalies, cleft lip and palate are relatively common. People with distal 18q- are often small for their age. Most individuals with distal 18q- fall in the mild to moderate range of intellectual disability. Strabismus and nystagmus, changes in the optic nerve as well as colobomas are also fairly common. People with distal 18q- frequently have conductive and/or sensorineural hearing loss. At present, treatment for distal 18q- is only symptomatic. This article presents a case report: Caries treatment of a 4-year-old female patient with de Grouchy syndrome under general anesthesia. The special considerations of dental care, especially caries treatment for the patient with de Grouchy syndrome are discussed.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.13 no.2
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• pp.80-85
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• 2017

Angelman syndrome is a rare disorder caused by deletion or inactivation of genes on the maternally inherited chromosome 15. This neurodevelopmental disorder is characterized by developmental and intellectual delay, speech impairment, sleep disturbance, seizures, motor dysfunction, and frequent laughing or smiling. Orofacial characteristics include a prominent mandible, large mouth, prominent cheeks, a tendency to rest the tongue between the dental arches, excessive drooling, and excessive chewing behavior. Patients with this syndrome usually require general anesthesia even in a simple operation, because of risk of perioperative seizure during dental procedure. This is a case report about dental treatment of a 3-year-old female patient with Angelman syndrome under general anesthesia. This case suggests that the dental treatment under general anesthesia can be considered a safe component for the uncooperative, delayed developmental patients with underlying disease. Also, periodic dental exam appointment should be made to provide the patients with preventive treatments and to make them remain familiar with the dental environment.

• Sleep Medicine and Psychophysiology
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• v.17 no.1
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• pp.16-20
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• 2010

Objectives: We review the sleep problems of the alcohol dependence patients. Especially we are interested in the prevalence, the severity of symptoms, anxiety, depression, and sleep quality of restless legs syndrome (RLS) in the alcohol dependence patients. Methods: We recruit 86 alcohol dependence patients who were admitted from October 6th, 2008 to October 17th, 2008. We interviewed each patient and evaluated sleep questionnaires such as the Sleep Disorder Questionnaire (SDQ), the Pittsburgh Sleep Quality Index (PSQI) and the International Restless Legs Syndrome Study Group (IRLSSG) rating scale. The presence of RLS and its severity were assessed using the IRLSSG diagnostic criteria and the IRLSSG severity scale, respectively. Depression and anxiety were evaluated by the Beck Depression Inventory (BDI) and the Beck anxiety inventory (BAI). Results: Of all 86 patients, 59 patients have insomnia, 33 patients have RLS, 30 patients have Periodic limb movement disorder (PLMD), 29 patients have nightmare. RLS patients have more high score in the BAI ($21.70{\pm}10.36$ vs $14.67{\pm}11.98$), and their sleep quality was poor in the PSQI ($11.09{\pm}4.08$ vs $7.92{\pm}3.91$) than non-RLS patients. Conclusion: This study shows that alcohol dependence patients show many sleep problems such as insomnia and RLS. So we should notice that the sleep problems of alcohol dependence patients are important in clinical approach and treatment.