• Childhood Kidney Diseases
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• v.11 no.2
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• pp.288-293
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• 2007

Thrombotic thrombocytopenic purpura(TTP) is a rare but life-threatening multi-system disorder characterized by the classic pentad of clinical features that includes fever, microangiopathic hemolytic anemia, thrombocytopenia, neurologic abnormalities and renal dysfunction. TTP has been rarely reported to simultaneously present with systemic lupus erythematosus (SLE). While it is important to distinguish between the two diseases of therapeutic implication, cases of concurrent TTP and SLE help to elucidate the pathophysiology that underlies each condition. We describe two adolescents with synchronous TTP and SLE, and review the literature.

• Childhood Kidney Diseases
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• v.12 no.1
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• pp.11-22
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• 2008

Telomeres consist of tandem guanine-thymine(G-T) repeats in most eukaryotic chromosomes. Human telomeres are predominantly linear, double stranded DNA as they ended in 30-200 nucleotides(bases,b) 3'-overhangs. In DNA replication, removal of the terminal RNA primer from the lagging strand results in a 3'-overhang of uncopied DNA. This is because of bidirectional DNA replication and specificity of unidirectional DNA polymerase. After the replication, parental and daughter DNA strands have unequal lengths due to a combination of the end-replication problem and end-processing events. The gradual chromosome shortening is observed in most somatic cells and eventually leads to cellular senescence. Telomere shortening could be a molecular clock that signals the replicative senescence. The shortening of telomeric ends of human chromosomes, leading to sudden growth arrest, triggers DNA instability as biological switches. In addition, telomere dysfunction may cause chronic allograft nephropathy or kidney cancers. The renal cell carcinoma(RCC) in women may be less aggressive and have less genomic instability than in man. Younger patients with telomere dysfunction are at a higher risk for RCC than older patients. Thus, telomeres maintain the integrity of the genome and are involved in cellular aging and cancer. By studying the telomeric DNA, we may characterize the genetic determinants in diseases and discover the tools in molecular medicine.

• Childhood Kidney Diseases
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• v.22 no.2
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• pp.75-80
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• 2018

Nutcracker syndrome is a phenomenon that the left renal vein (LRV) is pressed between the superior mesenteric artery (SMA) and the aorta. Clinical characteristics include gross or microscopic hematuria, orthostatic proteinuria, abdominal pain, and back pain. It occurs due to LRV squeezing caused by narrowed aortomesenteric angle. SMA syndrome is a disease that the third part of the duodenum is prone to intestinal obstruction by narrowed angle between the SMA and the abdominal aorta. Clinical symptoms include postprandial abdominal distension, epigastric pain, nausea, and vomiting. SMA syndrome and nutcracker syndrome have common features that result from narrowed aortomesenteric angle. However, it is very rare for both syndromes to occur simultaneously, so the two syndromes are regarded as separate diseases. This is a report on a case of nutcracker syndrome with SMA syndrome in a child who presented gross hematuria, recurrent abdominal pain and vomiting. To our knowledge, nutcracker syndrome simultaneous with SMA syndrome has not been previously reported in pediatric patient, especially with an exhibition of gross hematuria. This case suggests that the simultaneous presence of SMA syndrome with the same pathogenesis needs to be considered when nutcracker syndrome is suspected in pediatric patients with hematuria.

• Tuberculosis and Respiratory Diseases
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• v.78 no.1
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• pp.27-30
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• 2015

The drug reaction with eosinophilia and systemic symptom (DRESS) syndrome is a severe adverse drug-induced reaction which includes a severe skin eruption, fever, hematologic abnormalities (eosinophilia or atypical lymphocytes) and internal organ involvement. The most frequently reported drug was anticonvulsants. The diagnosis of DRESS syndrome is challenging because the pattern of cutaneous eruption and the types of organs involved are various. The treatments for DRESS syndrome are culprit drug withdrawal and corticosteroids. Here we report a 71-year-old man with skin eruption with eosinophilia and hepatic and renal involvement that appeared 4 weeks after he had taken anti-tuberculosis drugs (isoniazid, ethambutol, rifampicin, and pyrazinamide), and resolved after stopping anti-tuberculosis drugs and the administration of systemic corticosteroids. DRESS recurred after re-challenging isoniazid, we identified isoniazid was causative drug.

• Tuberculosis and Respiratory Diseases
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• v.67 no.3
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• pp.226-228
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• 2009

Henoch-$Sch\ddot{o}nlein$ purpura (HSP) is an immunologically mediated systemic vasculitis of small blood vessels that primarily involves the skin, gastrointestinal tract, joints and kidneys. HSP is a common vasculitic syndrome in children who, in most cases, achieve complete recovery. Pulmonary hemorrhage is a very rare manifestation of HSP. The authors present a case of a 46-year-old male presenting with pulmonary hemorrhage and renal involvement and the diagnosis of HSP. The patient responded to prednisolone therapy.

• YAKHAK HOEJI
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• v.56 no.1
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• pp.42-47
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• 2012

Soluble epoxide hydrolase (sEH) is a metabolic regulator of epoxyeicosatrienoic acids (EETs). EETs have many beneficial effects, vasodilation, anti-diabetes, anti-inflammation, cardiovascular protection, renal protection. Therefore, selective sEH inhibitors have a potential for treating these diseases. In the present study, screening methods for sEH inhibitors using PHOME ((3-phenyl-oxiranyl)-acetic acid cyano-(6-methoxynaphthalen-2-yl)-methyl ester) and 14-15-EET as substrates were established. To determine selectivity, microsomal epoxide hydrolase (mEH) inhibition assay was also developed using styrene oxide as a substrate of microsomal epoxide hydrolase. Our results obtained from 12-[[(tricyclo[3.3.1.13,7]dec-1-ylamino)carbonyl]amino]-dodecanoic acid (AUDA) used as a positive sEH inhibitor and valpromide used as a positive mEH inhibitor showed that these methods are useful for discovery of drug candidates.

• Journal of Veterinary Clinics
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• v.29 no.6
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• pp.435-440
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• 2012

This study was designed to evaluate the clinical efficacy and safety of Rhubarb extracts ($Rubenal^{(R)}$) in dogs with chronic renal failure (CRF). Client-owned 40 dogs with CRF graded International renal interest Society (IRIS) II-III were enrolled in this study. The dogs were equally allocated and blindly administered with $Rubenal^{(R)}$ or placebo. The following items were evaluated at day 0, 30, 90 and 180: body condition score (BCS), clinical score (appetite, polydipsia/polyuria, quality of life score), hemogram (WBC, RBC, PCV), serum biochemistry (ALT/AST, ALP, Creatinine/BUN, total protein, albumin), serum electrolyte (Na, K, Cl, Ca, P), systolic blood pressure, urinalysis (UPC, USG) and IRIS stage. In this study, we found that the $Rubenal^{(R)}$ preparation was well tolerated by dogs and induced no adverse effects. Statistically significant improvements were observed in clinical score (quality of life score by vet and clients), serum BUN and creatinine levels, serum phosphorus concentration, level of proteinuria, and the IRIS score of CRF in dogs after 6 month of treatment of $Rubenal^{(R)}$. Those findings suggested that the Rhubarb extracts can improve the clinical signs of CRF (i.e. azotemia, hypertension, proteinuria, hyperphosphoremia) and the quality of life (i.e. BCS, clinical score) and can retard the progression of CRF in dogs. Therefore the Rhubarb extracts can be a good supplementary drug for treating dogs with subclinical and clinical renal diseases. However, care should be taken for interpreting our result, because this study is not double-blinded controlled study but pilot study.

• Childhood Kidney Diseases
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• v.13 no.1
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• pp.26-32
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• 2009

Purpose : The increasing use of ultrasonography has allowed for an increase in the of the detection of congenital hydronephrosis, and the clinical outcomes of congenital hydronephrosis are widely varied. In this study, the necessity of voiding cystourethrography in neonate with hydronephrosis to rule out vesicoureteral reflux (VUR) was evaluated. Methods : Between January 2004 and December 2007, we reviewed the medical record of 157 childrens with congenital hydronephrosis detected within 1 month of age. The severity of hydronephrosis was graded by SFU (Society of Fetal Urology) system, and anterior posterior pelvic diameter (APPD). We evaluated the relationship between severity of hydronephrosis and incidence of VUR by using SPSS windows version 16.0. A P-value<0.05 is considered to be statistically significant. Results : Total renal unit number was 254, and 20(7.8%) renal units had VUR. We did not find any relationship between hydronephrosis grade the presence of VUR grade (P>0.05). In addition, there was no statistical significance between APPD, laterality of hydronephrosis and VUR incidence. However, renal units with VUR had lower spontaneous resolution rate (P<0.05), compared to renal units without VUR. Conclusion : In this study, there was no statistical significance between the severity of hydronephrosis and presence of VUR. Therefore, voiding cystourethrogram is recommended for all children with hydronephrosis to rule out VUR, regardless of the severity of hydronephrosis.

• Childhood Kidney Diseases
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• v.5 no.2
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• pp.182-187
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• 2001

Purpose : To evaluate the clinical significance of antenatally detected mild fetal pelviectasia and the role of voiding cystourethrography (VCUG) Methods : From January 1999 to September 1999, 28 neonates (44 pelviectatic kidneys) with mild fetal pelviectasia (anteroposterial diameter <10 mm) were evaluated by postnatal renal sonography and voiding cystourethrography. Results : Among 44 mild pelviectatic kidneys, 29 ($65.9\%$) kidneys had no pelviectasia and 15 ($34.1\%$) kidneys had persistent mild pelviectasia on postnatal renal sonography. Four ($9.1\%$) kidneys of 3 males ($10.7\%$) without pelviectasia on postnatal renal sonography had vesicoureteral reflux (VUR) on voiding cystourethrography. Vesicoureteral reflux was not correlated with prenatal progression or regression of pelviectasia and was not correlated with postnatal pelviectasia. Conclusion : All neonate with mild fetal pelviectasia need to undergo voiding cystourethrography for early diagnosis of vesicoureteral reflux even though postnatal renal sonography shows no pelviectasia. (J Korean Soc Pediatr Nephrol 2001 ;5 : 182-87)

• Clinical and Experimental Pediatrics
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• v.52 no.9
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• pp.999-1004
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• 2009

Purpose : Glomerular filtration rate (GFR) is a fundamental parameter in assessing renal function and predicting the progression of chronic renal disease. Because the use of serum creatinine has several disadvantages, many studies have investigated the use of cystatin C for estimating GFR. We compared creatinine clearance and GFR with formulas using serum creatinine and cystatin C. Methods : We retrospectively analyzed 211 patients with various renal diseases and classified them into two groups according to creatinine clearance (Group 1: CrCl >$90mL/min/1.73m^2$, Group 2: CrCl <$90mL/min/1.73m^2$). We measured serum creatinine, cystatin C, and creatinine clearance. We calculated GFR using the Schwartz, Counahan, Filler and Lepage, Bokencamp et al, and Grubb et al formulas. Results : GFR determined by the Schwartz formula had the highest correlation to creatinine clearance (r=0.415, P=0.00). GFR determined by various formulas using cystatin C had lower correlation to creatinine clearance (r=0.187, r=0.187, r=0.291). The Schwartz and Counahan formulas showed greater diagnostic accuracy in detecting decreased GFR than cystatin C in group 2 (areas under the curve: Schwartz, 0.596; Counahan, 0.572; Filler, 0.512; Bokencamp, 0.508; and Grubb, 0.514). Conclusion : GFR determined by the Schwartz and Counahan formulas using serum creatinine showed higher correlation coefficient than that determined by formulas using cystatin C. The formulas using cystatin C were not superior to those using serum creatinine in detecting decreased GFR. Cystatin C measurement was not satisfactory for assessing GFR in patients whose renal function was not severely decreased.