• Childhood Kidney Diseases
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• v.5 no.2
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• pp.125-135
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• 2001

Purpose : Efforts to predict long-term outcome of focal segmental glomerulosclerosis(FSCS) have been made but have yielded conflicting results. Reports are rare especially in Pediatric patients. In this study, we reviewed the predictable prognostic factors in patients of FSGS Method : Fifty children who diagnosed as biopsy-proven FSGS at department of pediatrics at Yonsei university were studied retrospectively. Based on medical records, response to treatment and pathologic slides, we compared normal renal function group and decreased renal function group, assessed the factors affecting renal survival and progression to renal failure. Results : The mean age at onset was 8 1/12 years, sex ratio was 2.3 : 1, and the mean duration of follow-up was 7 1/12 years. The overall renal survival rate was $34\%$ at 5 years, $8\%$ at 10 years Five-year survival rate was $74\%$ in normal renal function group and $27\%$ in decreased renal function group. Between the two groups, there were no significant differences in age at onset, sex ratio, amount of proteinuria, incidence of hematuria and hypertension, mesangial hypercellularity. Decreased renal function group showed higher serum creatinine level, poor response to treatment, higher percent of glomeruli with sclerosis, moderate to severe tubulointerstitial change and vascular change(P<0.05). The prognostic factors of renal survival rate were same as above and incidence of hypertension also affected renal survival( P<0.05). The progression rate to renal failure did not show statistically significant factor. Conclusion : We reviewed the factors affecting long-term outcome of FSGS. Serum creatinine level, steroid responsiveness, and the degree of glomerulosclerosis were significant prognostic factors. (J Korean Soc Pediatr Nephrol 2001 ;5 : 125-35)

• Childhood Kidney Diseases
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• v.2 no.2
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• pp.118-124
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• 1998

Purpose : There is no specific treatment guidelines for Henoch-$Sch{\ddot{o}}nlein$(HS) nephritis. Therefore we performed this study to observe the effect of long term steroid therapy combined with azathioprine Methods : Treatment protocols; 1) Steroid pulse therapy: methylprednisolon 30 mg/kg/dose, maximum 1 gm, intravenolisly 6 times for alternate day. 2) Oral steroid was given 2 mg/kg/day for 1 month, 1 mg/kg/day for following 1 month and alternate day oral steroid combined with azathioprine 2 mg/kg/day for 2 years. Results : Time period from HSP to onset of HS nephritis was between 2 weeks to 5 months with mean $7.4{\pm}7.4$ weeks. Clinical remission were seen in 4 cases out of 5 ($80\%$). Mean time period with disappearance of proteinuria and microscopic hematuria were $5{\pm}2.4$ month and $13.3{\pm}2.9$ month respectively. On pathologic findings by ISKDC, 3 cases were grade IIIb, 2 cases were grade IV in first kidney biopsies and showed pathologic improvement in follow up tidneybiopsiesafterlyearstreatment. Conclusion : As there is no definitive treatment for HS nephritis so far, our study of long term oral steroid therapy with azathioprine was effective in clinical and histologic aspect. Therefore further study in HS nephritis with in a large group will be needed in the future.

• Childhood Kidney Diseases
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• v.9 no.2
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• pp.222-230
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• 2005

Purpose : ARC syndrome refers to an association of arthrogryposis, renal tubular dysfunction, and cholestasis. The VPS33B gene was recently identified as the causative gene. So far, 41 cases of ARC syndrome have been reported worldwide, and it has rarely been reported in Korea. This study was conducted to report the clinical findings of seven ARC syndrome cases in Korean children, focusing especially on renal tubular dysfunction. Methods : The hospital records of 7 cases diagnosed as ARC syndrome at Severance Hospital between Mar. 1995 and Aug. 2005 were reviewed and analyzed. Results : Of the 7 cases, 4 were boys and 3 were girls. Six patients(85$\%$) were born with normal birth weight at term, and one was born at preterm. All cases presented with cholestasis and severe jaundice. According to the type of arthrogryposis described by Brown et al, type 3 and 4 were found in 2 patients and type,6, 7, and the undistributed type in one patient respectively. Other associated clinical findings were as follows : failure to thrive in 6(85$\%$), lax skin in 5(71$\%$), and gray platelet syndrome in 4(57$\%$). Urine analysis revealed 6 cases(85$\%$) with proteinuria, 3(43$\%$) with hematuria, 5(71$\%$) with glycosuria, 2(29$\%$) with phosphaturia and 2(29$\%$) with calciuria. Serum electrolytes showed 4 cases(57$\%$) with hyponatremia, 3(43$\%$) with hypokalemia, and 1(14$\%$) with creatinine elevation. Renal tubular dysfunctions were diagnosed as renal tubular acidosis in 6 cases(85$\%$), nephrogenic diabetes insipidus in 2(29$\%$), and Fanconi syndrome in 2(29$\%$). During the follow-up period, 2(29$\%$) had no treatment, 5(85$\%$) had continuous supplementation to correct the electrolyte imbalance and acidosis, and 1(14$\%$) had dialysis. Only one patient had a family history of ARE syndrome in a sibling. Four cases(57$\%$) were diagnosed at the mean age of 8.2 months, and one case was lost during follow-up. Ages of the survived 2 cases were 13 and 25 months, respectively. Conclusion : The rare disease of ARC syndrome Is associated with severe renal dysfunction. However, this study revealed that the renal manifestation of ARC syndrome in Korean children is relatively mild and survival rate is higher than that of previous studies. Contrary to previous reports, this study showed that familial cases are rare and sporadic occurence is possible in Korea. Thus, the diagnosis of this syndrome requires a careful evaluation of the venal function in cases of congenital arthrogryposis, and a mandatory genetic counseling of affected family for prevention of familial occurance. (J Korean Soc Pediatr Nephrol 2005;9:222-230)

• Proceedings of the KOR-BRONCHOESO Conference
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• 1981.05a
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• pp.5.3-6
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• 1981

There are too many kinds of esophageal corrosive agents, such as sodium hydrochloride, acetic acid, hydrochloric acid, etc. Esophageal burn due to above chemical agents are decreasing recently, but still many patients visited to the hospital because of swallowing corrosive agents for the purpose of suicide or accidentally. Among the treatment of corrosive injury of the esophagus, prevention of esophageal stricture is the key point. Recently various methods are using as the treatment of corrosive esophagitis and prevention of esophageal stricture. 51 cases of corrosive injury of the esophagus who had been admitted and treated at the Dept. of Otolaryngology, Han Yang University Hospital during past 9 years (from May 1972 to Dec. 1980) were evaluated and report the result about age distribution, sex incidence, monthly distribution, cause of swallowing, swallowing agents, arriving time at hospital after swallowing, changes on oral and pharyngeal mucosa, laboratory findings, emergency treatment and treatment during admission, treatment follow up results and complications with review of liter ature. Following results were obtained; 1. Female patients 27 cases (52.9%) were more than male patients 24 cases (47.1%) and its ratio was 1.13 : 1. 2. Age distribution showed predilection for age of 21-30 with 20 cases(39.2%), and 11-20 with 11 cases (21.6%), 31-40 with 7 cases(13.7%), over 50 with 7 cases (13.7%) were following. 3. Monthly distribution showed predilection for March with 8 cases(15.7%), and April, July with 7 cases (13.7%), September with 6 cases(l1.8%), October 5 cases(9.8%) were following. 4. For the purpose of suicide was the most cause of swallowing with 40 cases(78.4%), and accidentally swallowing 11 cases(21.6%). 5. Acetic acid was the most swallowing agent with 24 cases (47.0%), and hydrochloric acid 11 cases (21.5%), lye 8 cases(15.7%), iodine 2 cases(3.9%) were following. 6. Arriving time at the hospital after swallowing showed predilection for within 12 hours with 42 cases (82.4%), and from 12 hours to 24 hours with 4 cases(7.8%) was next. 7. Moderate change with injection and swelling was the prevalent change on oral and pharyngeal mucosa with 20 cases(39.2%) and severe cases with ulceration 18 cases (35.3%), mild cases with injection 10 cases (19.6%) were following. 8. Leukocytosis was seen on 40 cases (78.4%), and increased Hct. was seen 31 cases (60.8%). On urine analysis, 14 cases(27.5%) showed over 1.030 S.G., and proteinuria was seen on 25 cases(49.0%), glycosuria was seen on 5 cases(9.8%) and hematuria was seen on 6 cases(11.8). 9. Gastric lavage was done on 30 cases (58.8%) as emergency treatment and on 3 cases(5.9%) tracheostomy was done for the airway keeping. 10. As methods of treatment during admission, L-tube insertion was done on 50 cases (98.0%), antibiotics was given to 49 cases (96.1%), steroid and antacid were given to 46 cases(90.2%). 11. 36 cases(70.6%) were in favorable condition after proper treatment, but 2 cases (3.9%) were expired during admission, 4 cases (7.8%) showed esophageal stricture in-spite of treatment, and 1 case(2.0%) showed pyloric stenosis. 12. Complications were observed in 8 cases (17.7%). Renal failure (4 cases), aspiration pneumonia (2 cases), upper G-I bleeding (1 cases), and diabetic coma (1 cases) were seen in order of frequency.

• Pediatric Infection and Vaccine
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• v.8 no.2
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• pp.213-221
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• 2001

Propose : Tsutsugamushi fever is a acute febrile disease, which is caused by O. tsutsugamushi. Recently, this disease is increasingly reported in children. This study was undertaken to investigate clinical features of tsutsugamushi fever in children. Methods : This study involved 17 children with tsutsugamushi fever who were admitted to Masan Samsung hospital between September 1997 and December 2000. We investigated the age, sex ratio, clinical manifestations, laboratory findings, response of therapy and prognosis. Results : The age of patients was $6.9{\pm}3.6$ years, ranging from 6 months to 12 years and male predilection(58.8%) was noted and all cases of patients occured in October or November. The most common symptoms were fever in all cases and headache in 8(47.1%). The most common signs were skin rash in all cases, eschar in 14(82.4%) and lymphadenopathy 8(47.1%). Locations of the eschars were back and inguinal area in each 3 cases, neck and chest in each 2, popliteal area in 2, scalp and thigh in each 1. Laboratory findings included anemia in 1 case, leukopenia and thrombocytopenia in each 5, hematuria and proteinuria in each 1, ESR elevation in 2 and positive CRP in 12, AST elevation in 9 and ALT elevation in 7. Serologic diagnosis was made by passive hemagglutination assay(PHA) in 8 cases(47%) on admission, 4 cases in initial negative group were performed follow-up test at 2nd or 3rd weeks of illness and then all cases of 4 were converted to positive reaction. Clinical improvement was noticed in all cases after treatment to chloramhenicol or doxycycline. Mean duration for defervescence after treatment was $1.4{\pm}0.8$ days. Complications were interstitial pneumonia in 1 case and aseptic meningitis in 3, but all cases of patients were recovered without sequelae or recurrence. Conclusions : Tsutsugamushi fever in children was similiar to adult in the clinical features except male predilection. Early diagnosis and empirical treatment based on clinical manifestations such as fever, skin rash, eschar, lymphadenopathy is important and serologic diagnosis need to perform follow-up test at 2nd or 3rd weeks of illness.

• Proceedings of the KOR-BRONCHOESO Conference
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• 1981.05a
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• pp.7-8
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• 1981

Authors observed clinically 34 cases of the corrosive esophagitis caused by various corrosive agents at Kyung Hee University Hospital from Aug. 1978 to Dec. 1980. The results obtained were as follows; 1. Among the 34 patients, male was 19 (55.9%) and female 15(44.1%). Most frequently found age was 3rd decade. 2. 18 cases(52.9%) came to the hospital within 24 hours after ingestion of the agents, and 13 cases(38.2%) within 2 to 7 days. 3. Seasonal distribution showed most frequently in spring(35.3%). 4. The moment of the accident was suicidal attempt in 27 cases(79.4%) and misdrinking in 7 cases(20.6%). 5. Acetic acid was a most commonly used agent, showing 23 cases(67.6%), lye and insecticides were next in order. 6. Common chief complaints were swallowing difficulty and sore throat. 7. The average hospital days was 14.8 days. 8. Esophagogram was performed between 3 to 7 days after ingestion in 13 cases(38.2 %), findings were constrictions on the 1st narrowing portion in 4 cases(30.8%) and within normal limits in 3 cases(23.1%). 9. Esophagoscopy was performed in 31 cases(91.2%) between 2 to 7 days after ingestion, which revealed edema and coating on entrance of the esophagus in 9 cases (29.0 %). Diffuse edema on entire length of the esophagus and within normal limits were next in order. 10. Laboratory results were as follows: Anemia was in 1 cases(2.9%), leukocytosis. in 21 cases (61.8%), increase ESR in 9 cases (26.5%), markedly increased BUN and creatinine in 3 cases (8.8%), and hypokalemia in 1 cases(2.9%). Proteinuria in 10 cases(29.4%) hematuria in 4 cases(l1.8%), and coca cola urine in 3 cases (8.8%). 11. Associated diseases were 3 cases(8.8%) of cancer, 1 cases (2.9%) of diabetes mellitus, and 1 cases(2.9%) of manic depressive illness. 12. Various treatment was given: Esophageal and gastric washing in 23 cases(67.6%) for the emergent treatment, antibiotics in 32 cases(94.1%), steroids in 30 cases(88.2%), bougienation in 5 cases(14.7%), hemodialysis in 1 case(2.9%), and partial esophagectomy with gastrostomy and gastroileal anastomosis in 1 cases(2.9%). 13. Serious complications were observed in 9 cases (26.5%), consisted of 6 cases(17.6%) of esophageal stricture, 1 cases(2.9%), of aute renal failure, 1 cases (2.9%) of pneu momediastinum with pneumonia, and 1 cases (2.9%) of pneumonia.

• Childhood Kidney Diseases
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• v.5 no.2
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• pp.78-86
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• 2001

Purpose: Rapidly progressive glomerulonephritis (RPGN) is characterized by the rapid increase in serum creatitnin and crescents formation involving more than $50\%$ of glomeruli. 10 patients who had been treated for RPGN were studied retrospectively for thier underlying diseases and clinical features Method: Cilinical review was performed on 10 children who were diagnosed with RPGN by clinical features and renal biopsy and followed up at department of pediatrics during tile last 10 years, from May 1990 to May 2000. Result: There were 6 males and 4 females between the ages of 2.1 and 14.3 years (mean $10.9{\pm}3.8$). 3 had Henoch-$Sch{\ddot{o}}nlein$ purpura nephritis; 2, idiopathic rapidly progressive glomerulonephritis; 2, lupus nephritis; 1, hemolytic uremic syndrome; 1, membranous glomerulonephritis and 1, microscopic polyangiitis. The most common chief complaints were gross hematuria and oliguria. Initial clinical features included proteinuria, edema, hypertension, nausea and arthralgia. Mean serum BUN was $74.2{\pm}39.1\;mg/dL$ mean serum creatinin, $3.2{\pm}1.8\;mg/dL$ and mean creatinin clearance, $26.5{\pm}13.2\;mL/min/1.73m^2$. Antineutrophil cytoplasmic antibody was positive only in microscopic polyangiitis. ANA and Anti-DNA antibody were positive in two lupus nephritis patients. Serum complements were decreased in 4 patients. All patients except Hemolytic uremic syndrome received steroid pulse therapy and immunosupressive agents. 3 patients were performed acute peritoneal dialysis and 2 patients were given plasmapheresis. At the last follow up, 1 patient was dead, 4 patients had elevated serum creatinin, 2 of these 4 patients were on chronic ambulatory peritoneal dialysis and 6 patients had normal renal function. Conclusion: Rapidly progressive glomerulonephritis is a medical emergency that requires very rapid diagnosis, classification, and therapy. Appropriate therapy selected on the basis of underlying disease mechanism can substantially improve renal survival. (J. Korean Soc Pediatr Nephrol 2001 ; 5 : 78-86)