Jae Il Shin

Effect of renal Doppler ultrasound on the detection of nutcracker syndrome in children with hematuria.

To assess the detection rate of nutcracker syndrome in children with isolated hematuria, renal Doppler ultrasound examinations were routinely performed on 216 consecutive children (176 microscopic hematuria and 40 gross hematuria). Renal Doppler ultrasound was also performed on 32 healthy normal children. The peak velocity (PV) was measured at the hilar portion of the left renal vein (LRV) and at the LRV between the aorta and the superior mesenteric artery. The PV at the aortomesenteric portion (P=0.003) and the PV ratios of the LRV (P=0.003) were significantly higher in children with hematuria than in normal children, while the PV at the hilar portion was not different. If a PV ratio of the LRV of at least 4.1 (the cut-off level set at the mean ±2 SD of the value for the normal children) was defined as abnormal, 72 cases (33.3%) in children with hematuria and no cases in normal children were diagnosed as having nutcracker syndrome. The prevalence of nutcracker syndrome is relatively high in children with isolated hematuria, and the inclusion of renal Doppler ultrasound as a screening examination has a substantial effect on the detection of nutcracker syndrome.

Atypical Hemolytic Uremic Syndrome Associated With Complement Factor H Autoantibodies and CFHR1/CFHR3 Deficiency

Although genetic defect of complement factor H (CFH) is a common cause of atypical hemolytic uremic syndrome (aHUS), development of autoantibodies to CFH (CFH-Ab) is also known to be an acquired cause of aHUS. Recently, a correlation between the development of CFH-Ab and the deficiency of the CFH-related proteins, CFHR1 and CFHR3, was identified. In this study, plasma complement profiles were measured and genetic analysis of the CFH, CFI, MCP, CFHR1, and CFHR3 genes were performed in three female patients diagnosed with aHUS with positive CFH-Ab. Acute stage plasmas of all the three patients revealed low C3, low or low-normal CFH antigenic levels, and high titers of CFH-Ab. All the patients also showed complete plasma CFHR1 deficiency and homozygous genomic deletion of CFHR1/CFHR3, but none had CFH, CFI, or MCP mutations. All the patients were treated with plasmapheresis, and two patients required additional immunosuppressive therapy. These patients had a novel subgroup of aHUS characterized by a combination of genetic (a homozygous deletion of CFHR1/CFHR3) and acquired (development of CFH-Ab) factors. Patients with this disease may need intensive immunosuppressive therapy in addition to plasmapheresis. Screening for CFH-Ab and the CFHR1/CFHR3 deficiency should be included in the diagnostic tests for patients with aHUS.

Predictive factors for nephritis, relapse, and significant proteinuria in childhood Henoch-Schonlein purpura

Objective: To identify predictive factors for nephritis, relapse, and significant proteinuria in childhood Henoch–Schönlein purpura (HSP). Methods: Two hundred and six consecutive patients with HSP (93 female, 113 male), followed up at a single centre between 1996 and 2001, were analysed retrospectively. They were regularly monitored for clinical and laboratory parameters for renal sequelae and relapse. Results: Nephritis was seen in 78 patients (38%), relapse in 52 (25%), and significant proteinuria in 39 (19%). In univariate analysis, an older age at onset (>10 years), persistent purpura, severe bowel angina, and relapse were identified as factors associated with nephritis and significant proteinuria. Relapse‐related factors were an older age, persistent purpura, severe bowel angina, and leucocytosis. Logistic regression analysis showed that nephritis was significantly associated with an older age, persistent purpura, and relapse, and significant proteinuria was closely related to severe bowel angina and relapse. Conclusion: We identified some predictors for nephritis, relapse, and significant proteinuria in childhood HSP, and close attention should be paid to those patients with the risk factors, such as an older age at onset, persistent purpura, severe bowel angina, and relapse.

Coinfection of viral agents in Korean children with acute watery diarrhea.

Currently, there are a few reports on viral coinfection that causes an acute watery diarrhea in Korean children. So, to evaluate the features of coinfectious viral agents in children with acute watery diarrhea, we enrolled 155 children with acute watery diarrhea from July 2005 to June 2006. Fecal samples were collected and evaluated for various viral infections such as rotavirus, norovirus, adenovirus and astrovirus. The mean (±standard deviation) age of the children was 2.71±2.37 yr. The detection rate of viral agents was most common in children between the ages of 1 and 3 yr. Rotavirus was detected in 63 children (41.3%), norovirus in 56 (36.2%), adenovirus in 11 (7.1%), and astrovirus in 1 (0.6%). Regarding rotavirus, there were 38 (60.3%) cases with monoinfection and 25 (39.7%) with coinfection. For norovirus, there were 33 (58.9%) cases with monoinfection and 23 (41.1%) with coinfection. Coinfection with rotavirus and norovirus was most common, and occurred in 20/155 cases (12.9%) including coinfection with adenovirus. So, rotavirus and norovirus were the most common coinfectious viral agents in our study population with acute watery diarrhea.

Smoking and Rheumatoid Arthritis

Rheumatoid arthritis (RA) is a chronic inflammatory disease caused by both genetic and environmental factors. Smoking has been implicated as one of the most important extrinsic risk factors for its development and severity. Recent developments have shed light on the pathophysiology of RA in smokers, including oxidative stress, inflammation, autoantibody formation and epigenetic changes. The association of smoking and the development of RA have been demonstrated through epidemiologic studies, as well as through in vivo and animal models of RA. With increased use of biological agents in addition to standard disease-modifying antirheumatic drugs (DMARDs), there has been interest in how smoking affects drug response in RA treatment. Recent evidence suggests the response and drug survival in people treated with anti-tumour necrosis factor (anti-TNF) therapy is poorer in heavy smokers, and possible immunological mechanisms for this effect are presented in the current paper.

IBD immunopathogenesis: A comprehensive review of inflammatory molecules

Inflammatory molecules play a crucial role in the pathogenesis of inflammatory bowel disease (IBD) such as ulcerative colitis and Crohns disease, both of which are chronic inflammatory conditions of the gastrointestinal tract. Abnormal expressions of pro- and anti-inflammatory molecules have been described to cause an imbalance to the gut innate and adaptive immunity, and recently a large portion of research in IBD has been geared towards identifying novel molecules that may be used as potential therapeutic targets. Understanding of these inflammatory molecules has suggested that although ulcerative colitis and Crohns disease share many common clinical symptoms and signs, they are in fact two separate clinical entities characterized by different immunopathogenesis. In this review, we comprehensively discuss the roles of numerous inflammatory molecules including but not limited to cytokines, chemokines, inflammasomes, microRNAs and neuropeptides and their expression status in ulcerative colitis and Crohns disease in relation to their effects on the overall intestinal inflammatory process.

Complications of nephrotic syndrome

Nephrotic syndrome (NS) is one of the most common glomerular diseases that affect children. Renal histology reveals the presence of minimal change nephrotic syndrome (MCNS) in more than 80% of these patients. Most patients with MCNS have favorable outcomes without complications. However, a few of these children have lesions of focal segmental glomerulosclerosis, suffer from severe and prolonged proteinuria, and are at high risk for complications. Complications of NS are divided into two categories: disease-associated and drug-related complications. Disease-associated complications include infections (e.g., peritonitis, sepsis, cellulitis, and chicken pox), thromboembolism (e.g., venous thromboembolism and pulmonary embolism), hypovolemic crisis (e.g., abdominal pain, tachycardia, and hypotension), cardiovascular problems (e.g., hyperlipidemia), acute renal failure, anemia, and others (e.g., hypothyroidism, hypocalcemia, bone disease, and intussusception). The main pathomechanism of disease-associated complications originates from the large loss of plasma proteins in the urine of nephrotic children. The majority of children with MCNS who respond to treatment with corticosteroids or cytotoxic agents have smaller and milder complications than those with steroid-resistant NS. Corticosteroids, alkylating agents, cyclosporin A, and mycophenolate mofetil have often been used to treat NS, and these drugs have treatment-related complications. Early detection and appropriate treatment of these complications will improve outcomes for patients with NS.

Henoch-Schönlein purpura nephritis with nephrotic-range proteinuria: histological regression possibly associated with cyclosporin A and steroid treatment.

Objective: To clarify the therapeutic role of cyclosporin A (CyA) for patients with Henoch–Schönlein purpura nephritis (HSPN) showing nephrotic‐range proteinuria. Methods: The clinical and histological findings of eight children (7.7±3.8 years), who were treated with CyA and prednisolone, were evaluated retrospectively. All underwent a renal biopsy before therapy, and six of the eight patients received a follow‐up biopsy after therapy. Results: The histological grade of the International Study of Kidney Disease in Children (ISKDC) was improved in all six patients who received a follow‐up biopsy (pre‐therapy, four grade IIIa and two grade IIIb; post‐therapy, one grade I and five grade II) and it was statistically significant (p = 0.031). The activity index was significantly decreased after therapy (8.3±1.6 vs. 3.5±1.5, p = 0.031), and the chronicity index (0.5±0.5 vs. 0.7±1.0) and tubulointerstitial (TI) scores (1.5±1.3 vs. 0.8±1.6) did not change. There was a reduction in proteinuria from 3.2±2.3 to 0.1±0.1 g/m2/day (p = 0.008) and renal function remained normal in all patients after therapy. However, one patient showed CyA‐induced nephrotoxicity at a second biopsy. After an average follow‐up period of 3.8 years, six patients showed normal urine and renal function, and two showed minor urinary abnormalities. Conclusion: This study suggests that CyA therapy is effective in reducing proteinuria, which is a known risk factor for the development of renal insufficiency in HSPN and may regress the renal pathology in patients with nephrotic‐range proteinuria.

Spot Urine Albumin to Creatinine Ratio and Serum Cystatin C are Effective for Detection of Diabetic Nephropathy in Childhood Diabetic Patients

Spot urinary albumin to creatinine ratio (ACR) measurement has been suggested as a surrogate to 24-hr urine collection for the assessment of microalbuminuria, and cystatin C (cysC) is known as an advantageous marker for renal function. The aim of this study was to evaluate the clinical values of spot urinary ACR and serum cysC for the assessment of diabetic nephropathy instead of 24-hr urine microalbumin in children and adolescents with diabetes. A total of 113 children and adolescents (age 12-19 yr, M:F = 47:66) with type 1 or 2 diabetes were enrolled. We evaluated the validity of spot urine ACR and serum cysC, and then compared them to 24-hr urine microalbumin and creatinine clearance. Spot urine ACR was correlated with 24-hr urine albumin excretion (R2 = 0.828, P = 0.001) and creatinine clearance (R2 = 0.249, P = 0.017). The ROC curve analysis of serum cysC demonstrated higher diagnostic accuracy than that of serum creatinine (AUC 0.732 vs 0.615). Both the measurements of spot urine ACR and serum cysC might better predict the presence of diabetic nephropathy than 24-hr urine microalbumin in childhood diabetic patients.

Simple diagnostic approach to childhood fecal retention using the Leech score and Bristol stool form scale in medical practice.

Background and Aim:  To assess fecal retention, plain abdominal radiography is frequently used to complement the clinical history and physical examination, and three scoring systems have been proposed by Barr, Blethyn, and Leech on a single abdominal radiography. The aim of the present study was to find simple and useful diagnostic tools for an approach to fecal retention by correlation of the three scoring systems with the clinical characteristics.