• Title/Summary/Keyword: Nephrology

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Angiotensinogen M235T Polymorphism in Children with $Henoch-Sch\"{o}nlein$ Purpura Nephritis ($Henoch-Sch\"{o}nlein$ Purpura 신염에서 Angiotensinogen M235T 유전자 다형성)

  • Ha Chang-Woo;Joo Hee-Jung;Park Ji-Kyoung;Chung Woo-Yeong
    • Childhood Kidney Diseases
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    • v.8 no.1
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    • pp.10-17
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    • 2004
  • Purpose : $Henoch-Sch\"{o}nlein$ purpura(HSP) nephritis has a variable range of prevalence from 25 to 50% among HSP patients and is a common cause of chronic glomerulonephritis in children. In our study, we evaluated the distribution and the association of the angioten-sinogen(AGT) M235T polymorphism with the clinical manifestations, particularly proteinuria in children with HSP with or without nephritis. Methods : The AGT M235T polymorphism was determined in children with HSP nephritis (n=33) or HSP without nephritis(n=28) who had been diagnosed at Busan Paik hospital from January 1996 to June 2001. The M235T polymorphism of the AGT gene was determined by PCR amplification of the genomic DNA. Results : The M235T polymorphism of AGT gene frequency was MM 75%, MT : 25%, TT : 0% in HSP and MM : 64%, MT : 36%, TT : 0% in HSP nephritis, there was no significant differences in the genotype and allele frequencies between the two groups. No significant differences in clinical manifestations at onset and last follow-up were seen between the two genotypes. When statistical analysis was done according to the presence of the M allele, the amount of 24-hour urinary protein excretion and the incidence of moderate to heavy proteinuria(>500 $mg/m^2/day$) at onset and at last follow-up were higher in the MT genotype than in those of in the MM genotype but these difference were not statistically significant. Conclusion : We suggest a lack of association between M235T polymorphism of the AGT gene and clinical manifestations in children with HSP nephritis. However, further follow-up studies based on sufficient number of patients and long term follow up periods are necessary to confirm the role of M235T polymorphism of AGT gene in children with HSP nephritis.

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Therapeutic Efficacy of Alendronate for Glucocorticoid Induced Metabolic Bone Disease in Children with Nephrotic Syndrome (신증후군 환아에서 스테로이드 유발 대사성 골질환에 대한 Alendronate의 치료 효과)

  • Lee Ji-Eun;Lee Hyun-Ok;Paik Kyung-Hoon;Lee Suk-Hyang;Jin Dong-Kyu
    • Childhood Kidney Diseases
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    • v.8 no.1
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    • pp.33-42
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    • 2004
  • Purpose : Children with nephrotic syndrome(NS) are under high risk for metabolic bone disease(MBD) as a complication of long-term glucocorticoid therapy. We prospectively evaluated the effect of oral bisphosphonate(alendronate) therapy in children with NS, which has proven efficacy in adult patients with glucocorticoid induced MBD. Methods : Among 58 children with NS, aged 5 to 8 years and haying a disease duration of more than 2 years, 30(51.7%) were enrolled to meet the selection criteria, less than -1.0 Z-scores of lumbar spine bone mineral density(BMD) by dual energy X-ray absorptiometry (DEXA). These 30 children were divided into three groups and each were assigned to receive alendronate, calcitriol, and no-medication, respectively for one year. Lumbar spine BMD was followed up every 6 months and the biochemical indexes were measured before and 1 year after the treatment. There were no significant difference among groups with respect to the average age, the initial BMD, and the cumulative steroid doses. Analysis of the treatment efficacy was done by the % change of BMD and by the changes in Z-scores of lumbar spine BMD. Results : Mean age and disease duration of patients at the initial lumbar spine BMD evaluation was $7.4{\pm}1.7$ years and $2.2{\pm}1.2$ years, respectively. Twenty-three of 30 children(76%) had osteopenia, and seven(23%) had osteoporosis. There was no difference in the biochemical values among the groups, before and 1 year after the treatment(P<0.05). Twenty two children(73.3%) with frequent relapsing or steroid dependant NS had more frequent MBD, compared to the 8 children(26.6%) with infrequent relapsing NS. The one year % changes of BMD were 8.56 in alendronate group, 5.79 in calcitriol group, and 1.9 in no-medication group. The changes in Z-score of lumbar spine BMD increased in the alendronate group and the calcitriol group, but not in the no-medication group. One year % changes of BMD were different among groups(P=0.0002). Significant differences were found between the alendronate and the no-medication group, and between the calcitriol and the no-medication group(P<0.05). There was no difference between the alendronate and the calcitriol group. No serious adverse effect was observed in the alendronate group. Conclusion : Children with NS receiving high dose steroids are under the high risk of BMD and should undergo regular BMD evaluation. Z-score of lumbar spine BMD was a useful parameter in diagnosing low bone mass in children. Alendronate weekly oral therapy was effective and relatively safe in increasing the lumbar spine BMD in children with NS having steroid induced MBD.

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Guideline in the Management of Antenatally Diagnosed Unilateral Hydronephrosis (산전 초음파로 발견된 수신증의 치료 방침)

  • Hahn Hye-Won;Jun Nu-Lee;Kim Kun-Seok;Moon Dae-Hyuk;Yoon Chong-Hyun;Park Young-Seo
    • Childhood Kidney Diseases
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    • v.7 no.1
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    • pp.60-66
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    • 2003
  • Purpose : Neonatal hydronephrosis has been detected with increasing frequency with the widespread use of prenatal ultrasonography, but the consensus about its postnatal management has not yet been reached, especially about surgical intervention. We attempted to determine the guideline of follow-up study and surgical intervention of hydronephrosis by analyzing clinical outcomes of neonates with hydronephrosis. Materials and Methods : Between 1994 and 2000, 128 hydronephrotic kidneys were postnatally confirmed. Cases associated with other urologic anomalies were excluded and 90 unilateral hydronephrotic kidneys with a minimum follow-up of 12 months were enrolled in this study. We classified the patients into 6 groups according to the anterior posterior pelvic diameter(APPD) at initial ultrasonography(USG) within 1 month after birth. Renal USG and $Tc^{99m}-mercaptoacetyl$ triglycerine(MAG3) scan were done according to a set protocol, and pyeloplasty was performed when indicated according to our protocol. Results : Most cases whose APPD were below 10 mm improved or resolved. Only few cases with APPD above 20 mm showed spontaneous improvement and most(88%) had undergone operation. Those with initial APPD within 10-19 mm showed variable outcomes. When the risk factors for irreversible renal functional deterioration were analyzed, the age at pyeloplasty and pre-operative functional deficit were significant. Conclusion : We concluded that in infants with initial APPD below 10 mm, consideration of surgery is not needed, and in those with initial APPD above 20 mm, early operation is recommended. Our set protocol based on initial USG is useful, but the cut-off value of relative renal function(RRF) for operation might be increased to 40% to improve post operative RRF.

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Mutational Analysis of Mitochondria DNA in Children with IgA Nephropathy (소아 IgA 신병증 환자에서 미토콘드리아 DNA 돌연변이 분석)

  • Eom, Tae Min;Jang, Chang-Han;Kim, Hyoung Kyu;Kim, Nari;Chung, Yun Seo;Han, Jin;Chung, Woo Yeong
    • Childhood Kidney Diseases
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    • v.16 no.2
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    • pp.73-79
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    • 2012
  • Purpose: The association of mitochondrial DNA (mtDNA) mutations, deletions and copy number with progressive changes in patients with some glomerular disease and end-stage renal disease have been reported. In this study, we performed mtDNA mutation analysis in children with IgA nephropathy to investigate its role in progressive clinical course. Methods: Seven children with IgA nephropathy were involved in this study. MtDNA isolated from platelet was amplified by PCR and sequenced entirely. Results: The mean age at renal biopsy was $11.5{\pm}2.2$ year and the mean age at latest evaluation was $17.9{\pm}3.2$ year. The mean follow-up period were $7.8{\pm}3.1$ years. Patients was divided into 2 groups according to the amount of proteinuria at presenting manifestation. Group 2 patients were nephrotic syndrome. Renal function reveals within normal range in all patients. In group 2 patients, the mean serum albumin level was significantly lower than those of group 1 ($3.7{\pm}0.6g/dL$ vs. $4.7{\pm}0.2g/dL$, P=0.0241) and the mean total cholesterol level was significantly higher than those of group 1 ($222.7{\pm}35.7mg/dL$ vs. $148.3{\pm}29.1mg/dL$, P=0.0283). In Group 2 patients, total amount of protein of 24 hour collected urine also significantly higher than those of group 1 ($1,466.0{\pm}742.5mg$ vs. $122.5{\pm}48.1mg$, P=0.0135). Pr/Cr ratio in random urine sample was also higher in group 2 than those of group 1 but the statistical significance was not noted ($1.8{\pm}1.6$ vs. $0.2{\pm}0.2$, P=0.0961). Deletion of mtDNA nt 8272-8281 were observed in two patients, one patient in each groups, respectively. This is noncoding lesion. No patients demonstrated the mtDNA mutations. Conclusions: We have identified a deletion of mtDNA nt 8272-8281 in two children with IgA nephropathy. Further studies are needed to clarify the role of mitochondrial function in the progressive change of IgA nephropathy.

Analysis of Octyl-2-Cyanoacrylate as a Dressing Material after Pediatric Urological Procedures (소아비뇨기과 관혈적 수술 시 피부봉합제로 사용된 Octyl-2-Cyanoacrylate (Dermabond$^{TM}$)의 효용성)

  • Lee, Hahn-Ey;Min, Sun Ho;Kim, Kwang Myung
    • Childhood Kidney Diseases
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    • v.16 no.2
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    • pp.115-120
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    • 2012
  • Purpose: We aimed to evaluate the efficacy of Octyl-2-cyanoacrylate (Dermabond$^{TM}$) as a topical skin adhesive for pediatric urologic open surgery. Methods: From August 2010 to August 2011, we retrospectively evaluated pediatric patients who underwent urologic open surgery at our institution. A total of 128 pediatric patients with 210 incisions used Dermabond$^{TM}$ for skin closure. Results: We divided the 128 patients into 3 groups according to type of surgery. Group 1 underwent hydrocelectomy (55 cases, 41.3%), Group 2 underwent orchiopexy (43 cases, 32.3%), Group 3 underwent penoplasty (35 cases, 26.4%). One hundred and twenty eight patients who underwent 133 surgeries in total, with a total of 210 incisions visited our outpatient department postoperatively, and a total of 5 wound complications (2.3%) occurred, but were simple inflammations and no dehiscence was observed. When analyzed according to groups, no wound problems occurred in Group 1 (0/55, 0%), one occurred in Group 2 (1/43, 2.3%) and four cases occurred in Group 3 (4/35, 11.4%) respectively. When re-analyzed according to wound locations, one occurred in an inguinal wound (1/120, 0.83%), none occurred in scrotal wounds (0/55, 0%), and four occurred in penile wounds (4/35, 11.4%). In Group 3, the incidence of penile wounds was significantly increased compared to other groups (P=0.008). All 5 wound problems were inflammatory and healed at an average of 13.8 days (13-15 days) with antibiotic ointment application only. Conclusion: Dermabond$^{TM}$ is feasible and safe topical skin adhesive alternative to standard skin suture in pediatric urologic surgery. However, further research about its efficacy and safety could be valuable in the future.

The Clinical Comparison between Monomicrobial and Polymicrobial Urinary Infection in Febrile Pediatric Acute Pyelonephritis (발열성 소아 신우 신염에서 단일 세균 감염과 혼합 세균 감염의 임상적 비교)

  • Lee, In Hak;Nam, Seong Woo;Seo, Hyeon Seok;Yim, Hyung Eun;Yoo, Kee Hwan;Hong, Young Sook;Lee, Joo Won
    • Childhood Kidney Diseases
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    • v.16 no.2
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    • pp.102-108
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    • 2012
  • Purpose: We investigated the clinical presentation of febrile pediatric patients with acute pyelonephritis (APN) with a mixed urine culture from an aseptic urine sample, and compared with that of those with a single culture. Methods: We retrospectively reviewed the medical charts of 95 patients diagnosed as APN with fever between January 2008 and October 2010 at Korea University Medical Center. We classified the patients with APN into two groups with a positive single culture (S group) and a positive mixed culture (M group) from an aseptic urine sample of suprapubic bladder aspiration or urethral catheterization and compared the fever duration, laboratory markers such as serum white blood cell (WBC) counts and C-reactive protein (CRP) values in peripheral blood, and the presence of hydronephrosis, renal scar and vesicoureteral reflux (VUR) between the two groups (If presence of hydronephrosis, scar and VUR=1 and no=0). Results: Total pediatric patients with febrile APN were 95 patients, a positive S group was 89 patients and a positive M group was 6 patients. Fever duration (S vs. M, $4.7{\pm}3.1$ vs. $6{\pm}5.7$ days), serum WBC (S vs. M, $18,630{\pm}6,483$ vs. $20,153{\pm}7,660/{\mu}L$) and CRP (S vs. M, $100.6{\pm}2.46$ vs. $81.1{\pm}0.09\;mg/L$) values, and the presence of hydronephrosis, renal scar and VUR were not different between the two groups. Conclusion: Our data shows that there were no specific differences of clinical manifestation between a positive single urine culture and a positive mixed urine culture in pediatric APN. A mixed urine culture from an aseptic urine sample should be interpreted cautiously.

Acute Hyponatremia in Pneumonia and CNS Infections of Children (소아의 폐렴과 중추신경계 감염에서 급성 저나트륨혈증의 발생 양상)

  • Shin, Sung Hyun;Um, Tea Min;Lee, Yun Jin;Son, Seung Kook;Kim, Seong Heon;Kim, Su Yung
    • Childhood Kidney Diseases
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    • v.16 no.2
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    • pp.89-94
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    • 2012
  • Purpose: The option of selecting isotonic rather than hypotonic fluids for maintenance fluid in children has been advocated by some authors. Pneumonia and CNS infections are frequent clinical settings for acute hyponatremia because of nonosmotic anti-diuretic hormone stimuli in children. We conducted the present study to identify the incidence of hyponatremia in pneumonia and CNS infection of children and to determine the importance of maintenance intravenous fluid therapy regimen and other related factors. Methods: The study included 1,992 patients admitted to the Department of Pediatrics at Pusan National University Children's Hospital between November 2008 and August 2011, who were diagnosed with pneumonia or CNS infections and checked for serum sodium concentration. Their clinical data including laboratory findings were reviewed retrospectively. Results: During the study period, 218 patients were identified to have acute hyponatremia among 1,992 patients. The overall incidence of hyponatremia was 10.9%. The incidence of hyponatremia in encephalitis (37.3%) was highest and the incidence in bacterial meningitis (27.4%), viral meningitis (20.0%), bacterial pneumonia (11.1%), mycoplasma pneumonia (9.2%), and viral pneumonia (6.8%) were in descending order. The mean age was higher in hyponatremic patients than in isonatremic patients. The incidence of hyponatremia was higher in who had 0.18% NaCl in 5% dextrose (D5 0.18% NS) than 0.45% NaCl in 5% dextrose infusion (D5 1/2NS) (9.0% vs. 2.2%). SIADH was identified in 20.5% among hospital acquired hyponatremic patients after adequate evaluation for SIADH. Conclusion: We recommend D5 1/2NS rather than D5 0.18% NS as the maintenance fluid given to children with pneumonia or infectious CNS diseases.

Peritoneal Dialysis-related Peritonitis in Children: A Single Center's Experience Over 12 Years (소아 복막 투석 환자에서 발생한 복막염 : 단일기관에서 12년간의 경험)

  • Oh, Seong Hee;Lee, Yoon Jung;Lee, Jina;Lee, Joo Hoon;Park, Young Seo
    • Childhood Kidney Diseases
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    • v.16 no.2
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    • pp.80-88
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    • 2012
  • Purpose: The aim of our study was to investigate the characteristics of the peritoneal dialysis (PD) - related peritonitis and to evaluate the effectiveness of the empirical antibiotics recommended by the International Society for Peritoneal Dialysis in Korean children. Methods: We retrospectively reviewed the medical records of 72 children on peritoneal dialysis at the Department of Pediatrics, Asan Medical Center over the period from March 2000 to February 2012. Results: Seventy-nine episodes of peritonitis occurred in 32 patients. The incidence of peritonitis was 0.43 episodes/patient year. There were no significant differences in the incidence of peritonitis in terms of dialysis modality (P=0.459). Twenty-one patients experienced 51 catheter exit-site infections (0.28 episode/patient year). There were no significant differences in the incidence of peritonitis between those with and without history of exit-site infections (P=0.721). Specific pathogens were isolated from 68.4% (54/79) of the patient with peritonitis episodes, including Gram-positive bacteria (n=34), Gram-negative bacteria (n=25) and fungus (n=1). Among Gram-positive bacteria, 85.3% of the isolates were susceptible to ${\beta}$-lactam antibiotics, among Gram-negative rods, 94.7% of the isolates were susceptible to ceftazidime. Among 25 cases with unknown etiologies, 92.0% of cases demonstrated satisfactory responses to cefazolin and ceftazidime. Conclusions: The incidence of peritonitis was 0.43 episodes/patient year. Initial empirical therapy consisting of cefazolin and ceftazidime was appropriate for 91.1% of the PD-related peritonitis treatment. Continuous monitoring for the emergence of the resistant organisms is an important part of the appropriate managements of PD-related peritonitis.

The Changes of Slit Diaphragm Molecules After Using Sirolimus (Sirolimus 사용 후 사구체 기저막 세극막 관련 분자의 변화)

  • Choi, Jung-Youn;Han, Gi-Dong;Kim, Yong-Jin;Park, Yong-Hoon
    • Childhood Kidney Diseases
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    • v.14 no.2
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    • pp.143-153
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    • 2010
  • Purpose: Recently, massive proteinuria has been observed in some transplant patients after switching cyclosporine A (CsA) to sirolimus. To evaluate the pathogenesis of sirolimus-associated proteinuria, we investigated the early changes in slit diaphragm molecules by various administrative conditions of sirolimus and CsA. Methods: In vitro-Mouse podocytes were incubated with buffer (C), sirolimus ($10\;{\mu}g/mL$) after CsA ($10\;{\mu}g/mL$) (C-S), sirolimus only (S) and CsA and sirolimus simultaneously (C+S) for 12, 24, and 48 hours. In vivo- twenty four SPF female Wistar rats were divided into 4 groups buffer (C), sirolimus after 2 weeks of CsA (C-S), sirolimus only (S) and CsA and sirolimus simultaneously (C+S). All groups were treated by intraperitoneal injection every other day for 4 weeks (CsA: 25 mg/kg, sirolimus: 0.5 mg/kg). The changes in mRNA of slit diaphragm molecules were examined by RT-PCR. Results: The mRNA of nephrin was significantly decreased in group C-S and C+S in vitro. In vivo, the mRNA of nephrin in all groups using sirolimus and the mRNA of podocin in group C-S and C+S were decreased. Microscopically, group C-S and C+S showed small vacuolization and calcification in proximal tubular epithelial cells. Immunohistochemistry using nephrin and podocin antibodies did not show remarkable decrease of staining along the glomerular capillaries. Electron-microscopically, focal fusion of foot processes was seen in group C-S and C+S. Conclusion: This study suggests the decrease of slit diaphragm molecules (nephrin and podocin) in podocyte may be one of the causes of sirolimus associated proteinuria, and podocyte injury by sirolimus may need a primary hit by CsA to develop the proteinuria.

Clinical Features of Enuresis in Children with Diabetes Mellitus (소아 당뇨병 환아에서 야뇨증의 임상적 특징)

  • Kim, Myoung-Uk;Kim, Sae-Yoon;Choi, Jung-Youn;Cho, Min-Hyun;Ko, Cheol-Woo;Kim, Heung-Sik;Park, Yong-Hoon
    • Childhood Kidney Diseases
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    • v.14 no.2
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    • pp.210-217
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    • 2010
  • Purpose : Diabetes mellitus (DM) is known as one of the common causes of secondary enuresis in children. However, enuresis in diabetic children is overlooked only as a symptom of polyuria due to hyperglycemia. We evaluated the prevalence of nocturnal enuresis in children with diabetes mellitus in this paper. Methods : Among children with diabetes in three hospitals in Daegu area, 117 agreed to 'Tele research by means of a questionnaire'. Results : Diabetic patients were divided into two groups: Nocturnal enuresis and nonnocturnal enuresis group. thirty-two of 117 (27.0%) patients were in enuresis group, with more daytime urination than non-nocturnal enuresis group ($4.2{\pm}1.6/3.6{\pm}1.2$ times, P=0.016). HbA1c at diagnosis was $12.0{\pm}2.3%/12.0{\pm}2.5%$, and at follow-up $9.3{\pm}2.3%/8.3{\pm}2.3%$ (P =0.042). Insulin was administered at $1.1{\pm}0.5/1.1{\pm}0.4$ units/kg/day. Ten children of enuresis (31.2%) group were monosymptomatic (MNE) and 22 (68.8%) children were non-monosymptomatic enuresis (non-MNE). Fourteen (43.8%) of enuresis group had persistent symptoms, with 5 MNE and 9 non-MNE each. HbA1c at diagnosis was $11.1{\pm}2.5$, $12.4{\pm}2.1$, higher in non-MNE (P=0.144). Average arousal during sleep was step $3.3{\pm}1.2$, $2.5{\pm}1.0$, higher in improved enuresis group (P=0.059). Conclusion : Nocturnal enuresis among DM patients is underestimated. However, considering psychological and social effects of enuresis in children, extensive and long-term studies are needed in the future to clarify relationship between prevalence and DM control.