선천성 신증후군(Congenital Nephrotic Syndrome) 의 임상적 고찰

김병길,이연경,이재승,정현주,권민중,차응석 대한신장학회 1997 Kidney Research and Clinical Practice Vol.16 No.1

Congenital nephrotic syndrome(CNS) is a rare disease defined by nephrotic syndrome at birth or within the 1st year of life. This study is performed to investigate a classification based on clinicopathology and to evaluate the clinical course and prognosis according to types of CNS. We performed retrospective clinical study with chart review in 8 patients who were diagnosed as CNS from 1980 to 1995. The results were as follows; Their ages at the onset of illness ranged from birth to 7 months (median 2.2 months) and there were 7 males and 1 female. There were proteinuria, hypoalbuminemia and edema in all cases, accompanied with ascites(7cases), hematuria(5cases), hepatosplenomegaly(2cases), umbilical hernia(lcase) and inguinal hernia(lcase). A classification of these based on clinicopathology showed CNS of Finnish type in 2 patients, congenital syphilitic nephrotic syndrome in 2, mesangial glomerulosclerosis, minimal change disease, Drash syndrome and undefined CNS in 1 each. Of the 8 patients with CNS, 3 died of sepsis and renal failure, 1 responded to steroid and cyclosporin therapy and is alive at 20 months, 1 responded to penicillin, 1 discharged voluntarily, and 2 were lost to follow-up. In conclusion, it had been considered that all forms of CNS except the secondary ones have a very poor prognosis. But if the appropriate management including early renal transplantation is established under the definite diagnosis which is based on clinicopathology, we can expect long term sur- vival, normal growth and development for the child of CNS.

소아 급성 신부전의 임상적 고찰

권민중,정소정,이재승,김병길 대한신장학회 1995 Kidney Research and Clinical Practice Vol.14 No.1

Cardiovascular disease is one of the most comon cause of death in patients undergoing hemodialysis. Long standing hypertension, volume overload, anemia and hyperliprlipidemia lead to dys- function of heart in these patients. Recently, lipo- protein(a) (Lp(a) ) reported as a major risk factor of ischemic heart disease and peripheral vascular disease. So we reviewed hyperlipidemic feature and serum Lp(a) concentrations of patients undergoing hemodialysis to evalualuate their functional and structural features of heart and the effects of hyperlipidemia and Lp(a) to the heart. The results are as followings'. 1) Serum Lp(a) concentrations are significantly higher in patients undergoing hemodialysis with 26.5?18.6 mg/dl than normal control subjects with 10.9?5.2 mg/dl (median value.' 22.05 mg/dl ' vs 10 mg/dl, p$lt;0.01). 2) Serum Lp(a) concentrations are significantly higher in patients with ischemic heart disease with 36.0?18.5 mg/dl than other patients with 14.9? 10.6 mg/dl (median value. 37 mg/dl vs 9.75 dl, p$lt;0.01) and show positive correlations with thickness of posterior wall of left ventricle and ejection fraction (r=0.61, r=0.49). Serum triglyc- eride concentrations show positive correlations with ejection fraction (r=0.52), serum cholesterol concentrations show positive correlatior with thickness of posterior wall of left ventricle and ejection fraction (r = 0.50, r = 0.48) and apolipo- protein A I concentrations show negative correla- tions with internal dimension of left atrium during diastole(r = 0.54) 3) Serum Lp(a) concentrations are significantly higher in patients with occlusion of arteriovenous fistula with 39.9?17.1 mg/dl than other patients with 21.5?16.8 mg/dl (median value'. 37.5 mg/dl vs 15 mg/dl, p$lt;0.01) and show positive correla- tions with thickenss of posterior wall of left ventri- cle and ejection fraction (r=0.51, r=0.65) and negative correlations with internal dimension of left atrium during diastole(r= 0.67). Triglyceride and cholesterol correlate positively with ejection fraion (r=0.49, r=0.65), and LDL cholesterol shows positive correlations with thickness of pos- terior wall of left ventricle and ejection fraction (r =0.57, r=0.70). 4) Serum Lp(a) concentrations are significantly higher in patients with arrhythmia with 30.2?20.2 mg/dl than other patients with 17.9?10.0 mg/dl (median value'. 33 mg/dl vs 15.8 mg/dl, p$lt;0.01), serum cholesterol (172.3?67.5 mg/dl vs 130.3? 22.3 mg/dl, p$lt;0.01) and LDL cholesterol(79.5? 34.6 mg/dl vs 59.1?17.6 mg/dl, p$lt;0.05) concen- trations are higher in patients with arrhythmia than other patients. Concentrations of triglyceride and cholesterol show positive correlation ehection fraction (r =0.48, r = 0.70) in patients with arrhythmia. From above results, we suggest that hyperlipidemia may acclerate the cardiovascular disease and Lp(a) may influence to the development of cardiovascular complications and survival of arteriovenous fistula in the patients undergoing hemodialysis.

신증을 동반한 HSP 신염에서 Cyclosporin A의 치료효과

김병길,정현주,최인준,권민중 대한신장학회 1995 Kidney Research and Clinical Practice Vol.14 No.4

Treatment of Henoch-Schonlein purpura nephritis (HSPN) accompanied by nephrotic syndrorne(NS) is still controversial, even though both corticosteroids and immunosuppressants have been used for therapy. Cyclosporin A(CsA) is a neutral lipophilic endecapeptide that inhibits the production and/or release of interleukins, and is used in steroid-resistant nephrotic syndrome. We studied the effects of CsA in HSPN accompanied by NS. The study was done by evaluating the clinical status and histopathological changes by sequential biopsies following the treatment. All patients developed nephrotic syndrome either initially or during the course of HSPN. They were treated with CsA (5mg/kg/day) and prednisolone (1mg/kg/q.o.d.) for 6 to 8 months. The clinical status of the patients on admission were C(16 cases) and D(1 case), but changed to B(11 cases) and A(6 cases) following treatment. All cases displayed no proteinuria 3.3±1.7 months after treatment. During 16.6?8.1 months of follow-up, 10 cases presented complete ression, one recurred NS, and one progressed to ESRD and was done transplantation. The remaining cases had persistant mild proteinuria and hernaturia. Histopathologically, 5 cases improved, 2 aggravated, and the remaining not changed in the grading system by ISKDC. But irnmunopathologically, 16 out of 17 cases decreased intensity and extent of immune deposits. This study suggests that, although control studies are needed, CsA could be used in the treatment of HSPN accompanied by NS.

소아에서의 지속성 외래 복막투석(CAPD) 의 임상적 고찰

김병길,이재승,최원규,권민중 대한신장학회 1996 Kidney Research and Clinical Practice Vol.15 No.4

CAPD has established itself as an altemative therapeutic modality to hemodialysis in the treatment of patients with e@nd-stage renal disease. Especially in children, many of the advantages originally reported with the use of CAPD have been reported with large population studies. This includes ease of training and management, no requirement of repetitive venopuncture, less restriction of diet, stable salt and water balance, ease of blood pressure control. Clinical studies carried out on the 29 cases of CAPD who had been treated during 10 years in Severance hospital. The following results were obtained: Nine- teen cases were males and ten cases were females, with male to female ratio of 1.9: 1. The mean age of the patients was 12.9?3.1 years old. The original renal diseases of ESRD were proven in 22 cases. The comon renal disesaes were reflux nephropathy, chronic glomerulonephritis and focal segmental glo- merulosclerosis each in 4 cases. Other diseases were Henoch-Schonlein purpura nephritis in 2 cases, hemlytic uremic syndrome in 2 cases, and Alport syndrome, Ig A nephropathy and Lupus nephritis were one case respectively. The mean duraton of CAPD was 16.1?19.9 months. Peritonitis was the most common complication whose incidence was 1.3 episode/patient-year. Other complications were exit site infection in 3 cases, obstruction in 3 cases, and leakage of dialysate in 3 cases, peritoneal adhesion, malposition and scrotal swelling were also found. The most common etiologic organism of peritonitis was staphyococcus coagulase negative and staphylococcus aureus was the next. Others were Pseudomonas, Enterococcus species and E. coli. As increa- sing renal transplantation, CAPD in children has been more important as renal replacement therapy.

신동맥 침범이 있는 소아의 Takayasu 동맥염

김병길,이재승,이승규,최원규,설준희,권민중 대한신장학회 1996 Kidney Research and Clinical Practice Vol.15 No.4

Takayasu arteritis frequently involves the aorta and its major branches resulting in arterial obstruction or aneurysm and is a common cause of renovascular hypertension. We selected of 12 patients showing renal involvement among 17 patients had diagnosed with Takayasu arteritis by angiogram since 1985. We performed retrospective analysis of clinical manifestations and therapeutic results. Male to female ratio was 1 to 5 and mean age was 14 years. Previous tuberculosis medication history was noted in 4 cases. Initial clinical presentations were headache, nausea, vomiting and palpitation in order of frequency. Hypertension was noted in all patients. The mean ESR at admission was 56.0±32.0mm/hr and peripheral renin activity was 47.8±46.4ng/ml/ hr. Mean duration of follow-up was 29.6±21.8 months and showed improved therapeutic result with operation and angioplasty. Takayasu arteritis is a common cause of renovascular hypertension in children. Intervention with operation or angioplasty showed improved ress compared to conservative management alone.

Glucocorticoid 투여를 요하는 소아 신증후군 환아에 대한 Prednisolone 과 Deflazacort 의 치료효과 및 골대사에 미치는 효과 비교 ( 예비 보고 )

권민중,김병길 대한신장학회 1994 학술대회및초록집 Vol.13 No.2

산전 초음파 검사로 진단된 선천성 신요로 기형에 대한 추적 관찰

김병길,양영호,박용원,권민중,박임재 대한신장학회 1995 Kidney Research and Clinical Practice Vol.14 No.2

Prenatal ultrasonography provides accurate detection and classification of urinary tract anomalies which make up the majority of prenatal anomalies. Reviewed 39 infants with urinary tract anomalies diagnosed by prenatal ultrasonography in the period 1990 to 1994, and correlative analysis was done between pre and post ultrasonography. Fifteen thousand three hundred and fifty cases of prenatal ultrasonography were performed and among them, 39 cases(0.25%) were diagnosed with urinary tract anomalies. The gestational ages on detection were between 21 to 40 weeks (mean 32 weeks). Mostly, prenatal ultrasonography were perforrned between 31 and 35 weeks. The sex ratio was 4.5:1(M:F). On prenatal ultrasonographic findings, 29 cases(74%) had pelvic dilatation, 9 cases(23%) had cystic diseases and 1 case(3%) had renal mass. Postnatal follow up studies revealed 18 cases(46%) with ureteropelvic junction obstruction, 9 cases(23@Yo) with multicystic dysplastic kidney and 7 cases(18%) had normal kidney. Among the 29 cases with pelvic dilatation on prenatal ultrasonography, 22 cases had AP diameter greater than 10mm. Postnatal ultrasonography was abnormal for these pelvic dilatation of AP diameter greater than 10mm. Other associated anomalies included Turner syndrome, Dandy-Walker syn- drome, cryptorchidism and congenital heart diseases. According to these findings, as non-invasive procedure, prenatal ultrasonography is very available for screening of fetal urinary tract anomalies. It is necessary to follow-up persistently after birth when pelvic dilatation of AP diameter greater than 10mm is present prenatally. Therefore, early diagnosis of urinary tract anomalies by prenatal ultrasonography may be able to prevent progression of renal damage and provides immediate management of these infants after delivery.

Glucocorticoid 투여를 요하는 일차성 신증후군 환아에 대한 Prednisolone 과 Deflazacort(Calcort R) 의 치료 효과 및 골 대사에 미치는 효과 비교

김병길,권민중 대한신장학회 1995 Kidney Research and Clinical Practice Vol.14 No.2

The rate of remission induction with initial steroid therapy in children with minimal change disease is known to be above 90%, of which 40% is frequently relapsing type. In long-term steroid treatment, significant side effects occur during the course of therapy such as osteoprosis, growth retardation, obesity, Cushing syndrome and diabetes mellitus. By binding to steroid receptor, deflazacort(Calcort), an oxazoline derivative of prednisolone, exhibit antiinflammatory and immune suppressive effects, but decreases incidence of osteoporosis by lesser effect on calcium homeostasis and osteoclast activation than prednisolone. Thus the authors investingated 38 patients who need long term steroid therapy because of nephrotic syndrome to see the efficacy and the effect of deflazacort on bone metabolisrn. The 38 patients were randomly divided in two groups with classic drug, prednisolone and new drug, deflazacort. Beside laboratory parameters to ensure the effect of treatment of the nephrotic syndrome, all had measu rements of the bone densitometry, serum levels of parathyroid hormone and osteocalcin at 0 and 3 months of treatrnent. The therapeutic effects on the nephrotic syndrome were not different between the two drugs. The bone loss in the prednisolne-treated group was significantly higher than that of the deflazacort-treated group(Bone mineral content, 0 month vs. 3 month: 0.644±0.133 vs 0.615±0.159 g/cm in prednisolone-treated group, 0.581±0.127 vs 0.599±0.144 g/cm in deflazacort-treated group). There was a more marked fall in osteocalcin in prednisolone-treated group(0 month vs. 3 month: 60.45±47.19 vs 28.69±25.61 ng/ml) compared to deflazacort-treated group(0 month vs. 3 month: 44.20± 33.74 vs 39.33±24.83 ng/rnl). No significant difference was demonstrated between the two groups concerning serum PTH and cortisol. Thus, the detrimental effect of long-term steroid treatment on the bone may not be abolished, but it is significantly reduced by using deflazacort instead of prednisolone.

국소성 분질성 사구체경화증에서 Cyclosporin A의 치료효과

김병길,이재승,최인준,권민중,차응석 대한신장학회 1996 Kidney Research and Clinical Practice Vol.15 No.4

It is difficult to differentiate focal segmental glomerulosclerosis(FSGS) from MCNS clinically. However, FSGS is characterized by higher incidence of hematuria, poor selective protein index(SPI), resistance to steroid and progression to end stage renal failure. This study was designed to evaluated the clinical manifestation and the possible better management in FSGS. 31 cases of children who were diagnosed as FSGS on renal biopsies were studied retrospectively. Male to female ratio was 2.4 to l. The mean age at onset was 6 6/12 years(range 1 1/12-13 11/12 years). Proteinuria was detected in all cases and edema(87%), hematuria(45%), hypertension (35%), renal failure(26%) were also found. SPI was 0.31(?0.16). As management, steroid alone was used in 6 cases, cyclosporin A with steroid in 17 cases and cyclophosphamide with steroid in 2 cases. Patients were followed up for average 18 months and 7 cases progressed to chronic renal failure. There was no significant difference in the clinical and laboratoy findings between remission and no-remission group, and between CRF and non-CRF group at diagnosis. Remission rate was 33.4% with the regimen steroid alone and 58.8% with cydospo- rin A with steroid. In condusion, the renal biopsy should be performed for the patients with nephrotic syndrome resistant to steroid as soon as possible for the detection of FSGS. Cyclosporin A with steroid was more effective than steroid alone, even though no statistical difference between the two groups.

추적 신생검을 시행한 Lupus 신염 5례의 좆기병리학적 변화

권민중,김병길,최인준,임현이 대한신장학회 1995 춘계학술대회 초록집 Vol.1995 No.-