Outcome of immunosuppression in children with IgA vasculitis-related nephritis.

BACKGROUND AND HYPOTHESIS: IgA vasculitis with nephritis (IgAVN) is the most common vasculitis in children. Treatment recommendations are, due to a lack of evidence, based on expert opinion resulting in variation. The aim of this study was to describe clinical presentation, treatment and outcome of an extremely large cohort of children with biopsy proven IgAVN to identify prognostic risk factors and signals of treatment efficacy. METHODS: Retrospective data were collected on 1148 children with biopsy proven IgAVN between 2005 and 2019 from 41 international paediatric nephrology centres across 25 countries and analyzed using multivariate analysis. The primary outcome was estimated glomerular filtration rate (eGFR) and persistent proteinuria at last follow up. RESULTS: The median follow up was 3.7 years (IQR 2-6.2). At last follow up, 29% of patients had an eGFR < 90 ml/min/1.73m2, 36% had proteinuria and 3% had chronic kidney disease stage 4-5. Older age, lower eGFR at onset, hypertension and histological features of tubular atrophy and segmental sclerosis were predictors of poor outcome. There was no evidence to support any specific second line immunosuppressive regimen to be superior to others, even when further analysing subgroups of children with reduced kidney function, nephrotic syndrome or hypoalbuminemia at onset. Delayed start of immunosuppressive treatment was associated with a lower eGFR at last follow up. CONCLUSION: In this large retrospective cohort, key features associated with disease outcome are highlighted. Importantly there was no evidence to support that any specific immunosuppressive treatments were superior to others. Further discovery science and well-conducted clinical trials are needed to define accurate treatment and improve outcomes of IgAVN.

Predictors of rapidly progressive glomerulonephritis in acute poststreptococcal glomerulonephritis.

BACKGROUND: Acute post-streptococcal glomerulonephritis (APSGN) is an immune-mediated inflammatory respsonse in the kidneys caused by nephritogenic strains of group A ß-hemolytic streptococcus (GAS). The present study aimed to present a large patient cohort of APSGN patients to determine the factors that can be used for predicting the prognosis and progression to rapidly progressive glomerulonephritis (RPGN). METHODS: The study included 153 children with APSGN that were seen between January 2010 and January 2022. Inclusion criteria were age 1-18 years and follow-up of ≥ 1 years. Patients with a diagnosis that could not be clearly proven clinically or via biopsy and with prior clinical or histological evidence of underlying kidney disease or chronic kidney disease (CKD) were excluded from the study. RESULTS: Mean age was 7.36 ± 2.92 years, and 30.7% of the group was female. Among the 153 patients, 19 (12.4%) progressed to RPGN. The complement factor 3 and albumin levels were significantly low in the patients who had RPGN (P = 0.019). Inflammatory parameters, such as C-reactive protein (CRP), platelet-to-lymphocyte ratio, CRP/albumin ratio, and the erythrocyte sedimentation rate level at presentation were significantly higher in the patients with RPGN (P < 0.05). Additionally, there was a significant correlation between nephrotic range proteinuria and the course of RPGN (P = 0.024). CONCLUSIONS: We suggest the possibility that RPGN can be predicted in APSGN with clinical and laboratory findings. A higher resolution version of the Graphical abstract is available as Supplementary information.

The relevance of practical laboratory markers in predicting high-grade vesicoureteral reflux and renal scarring.

INTRODUCTION: A high vesicoureteral reflux (VUR) grade is among the specific risk factors for febrile urinary tract infection (febrile UTI) and renal scarring. The aim of this study was to examine the predictive value of some potential hematological parameters for high-grade VUR and renal scarring in children 2 to 24 months old with febrile UTI. METHODS: We retrospectively examined the clinical features, laboratory tests, and imaging studies of 163 children 2 to 24 months old with a diagnosis of febrile UTI. The hematological parameters based on the neutrophil-to-lymphocyte ratio (NLR), platelet-to-lymphocyte ratio (PLR), and white blood cell count (WBC) were calculated using a receiver operating characteristic (ROC) analysis to select which one is suitable. RESULTS: Of the 163 children with febrile UTI, 57 patients (35%) exhibited high-grade VUR. Regarding the predictive power for high-grade VUR, the median area under the curve (AUC) was 0.692 for NLR (sensitivity 61.4%, specificity 69.8%, P < 0.001) and 0.681 for PLR (sensitivity 63.2%, specificity 62.3%, P < 0.001). White blood cell count demonstrated the highest area under the ROC curve for diagnosis of high-grade VUR (0.884, 95% confidence interval 0.834-0.934) and an optimal cutoff value of 13.5 (sensitivity 80.7%, specificity 80.2%, P < 0.001). White blood cell count, with the highest AUC of 0.892 while the sensitivity and specificity were 83.3% and 82.8, was the preferred diagnostic index for renal scarring screening. CONCLUSIONS: White blood cell count, NLR, and PLR were useful biomarkers closely related to children with febrile UTI who are at risk for high-grade VUR can also act as a novel marker to accurate prediction of high-grade VUR and renal scarring. Also, NLR and PLR can serve as useful diagnostic biomarkers to distinguish high-grade VUR from low-grade VUR.

Determining the effectiveness of the immature granulocyte percentage and systemic immune-inflammation index in predicting acute pyelonephritis.

AIM: The most serious form of urinary tract infection (UTI) is acute pyelonephritis (APN), which can result in bacteremia and renal scarring. This study aims to show the roles that the systemic immune-inflammation index (SII) and the immature granulocyte (IG) percentage play in predicting APN in pediatric patients by comparing them with traditional infection markers. By illustrating a significant relationship between APN, the IG percentage, and the SII, the study's contributions to the differential diagnosis of UTI can promote a rapid and appropriate treatment of APN. MATERIAL AND METHODS: The present study included 522 pediatric patients. The patients were divided into two groups: (I) an APN group (n = 236) and (II) a lower UTI group (n = 286). RESULTS: The mean age of the sample was 5.05 ± 4.7 years. According to the most appropriate cutoff values, the findings showed that among all the parameters, C-reactive protein (CRP) and the IG percentage had the highest sensitivities, specificities, and predictive values for predicting APN. CONCLUSION: This study emphasizes the support features of the IG percentage and the SII in the diagnosis of APN in pediatric patients, a topic that has recently attracted attention. The findings indicated that among all of the parameters behind CRP, the IG percentage and the SII had the highest sensitivities, specificities, and predictive values for forecasting APN. It is worth noting that these methods can be applied without additional costs or burdens to the patient.

The importance of ambulatory blood pressure monitoring for diagnosing masked hypertension in patients with renal parenchymal scarring.

BACKGROUND: The most well-known and common long-term complication in children with renal parenchymal scarring (RPS) is hypertension (HT). The present study aimed to evaluate the presence of HT in children with RPS based on ambulatory blood pressure monitoring (ABPM) and to compare the patients' blood pressure (BP) to that in healthy controls matched for age, gender, and BMI. METHODS: The study included 55 patients aged < 18 years diagnosed with RPS who were followed up for ≥ 1 year and 48 healthy controls matched for age, gender, and BMI. RESULTS: Mean age in the RPS group was 12.8 ± 3.3 years, and 49.1% of the group were female. Among the RPS patients, 28 were diagnosed with HT based on ABPM, of which 18 (32.7%) had an office BP < 90th percentile for age, gender, and height; seven had an office BP between the 90-95th percentiles for age, gender, and height; and three had an office BP > 95 percentile for age, gender, and height. The difference in detection of HT based on ABPM between the two groups was significant (P = 0.00). CONCLUSIONS: Early diagnosis of HT via ABPM can help prevent development of kidney failure and cardiovascular disease in patients with RPS, significantly reducing the rates of morbidity and mortality. All children with RPS should be evaluated via ABPM, even if office BP measurements are normal. "A higher resolution version of the Graphical abstract is available as Supplementary information".

Clinical spectrum of immunoglobulin A vasculitis in children and determining the best timing of urine examination to predict renal involvement.

BACKGROUND: Immunoglobulin A (IgA) vasculitis (also known as Henoch-Schonlein purpura) is the most common small vessel vasculitis of childhood. The long-term prognosis depends on renal involvement. The aim of this study was to evaluate the risk factors associated with renal involvement in children with IgA vasculitis and to investigate the best timing of urine examination to predict the presence of renal involvement at the sixth month after the diagnosis. METHODS: In this prospective observational study, medical records, demographic data, clinical findings, laboratory tests, and urine microscopic examinations of pediatric patients diagnosed with IgA vasculitis were evaluated to identify potential risk factors associated with renal involvement. RESULTS: A total of 178 patients with a median age of 6 years were involved in the study. Renal involvement was found in 24 (13.5%) patients. Most of the patients (85.7%), whose urine examination was found to be abnormal at the sixth month after the diagnosis, also had abnormalities in the first month urine examination. Factors significantly associated with renal involvement were as follows: older age, presence of hematuria and or proteinuria in the first month urine examination and patients who presented the disease in the spring season. Multivariate logistic regression analysis showed age ≥8 years (p = 0.005), the season of onset (p = 0.025), serum creatinine levels (p = 0.016), and abnormal urine examinations at the first-month visits (p = 0.005) significantly increased the risk of renal involvement. CONCLUSION: This study has demonstrated that the optimal date to predict the presence of hematuria and/or proteinuria in the sixth month is the urine examination performed in the first month of the disease. Therefore, we think that patients with IgA vasculitis who show a presence of hematuria and/or proteinuria in the first-month urine examination should be followed more closely.

COVID-19 in pediatric nephrology centers in Turkey.

BACKGROUND: There is limited data on COVID-19 disease in children with kidney disease. We aimed to investigate the characteristics and prognosis of COVID-19 in pediatric nephrology patients in Turkey. METHODS: This was a national, multicenter, retrospective cohort study based on an online survey evaluating the data between 11th March 2020 and 11th March 2021 as an initial step of a detailed pediatric nephrology COVID-19 registry. RESULTS: Two hundred and three patients (89 girls and 114 boys) were diagnosed with COVID-19. One-third of these patients (36.9%) were between 10-15 years old. Half of the patients were on kidney replacement therapy: kidney transplant (KTx) recipients (n = 56, 27.5%), patients receiving chronic hemodialysis (n = 33, 16.3%) and those on peritoneal dialysis (PD) (n = 18, 8.9%). Fifty-four (26.6%) children were asymptomatic. Eighty-two (40.3%) patients were hospitalized and 23 (28%) needed intensive care unit admission. Fifty-five percent of the patients were not treated, while the remaining was given favipiravir (20.7%), steroid (16.3%), and hydroxychloroquine (11.3%). Acute kidney injury developed in 19.5% of hospitalized patients. Five (2.4%) had MIS-C. Eighty-three percent of the patients were discharged without any apparent sequelae, while 7 (3.4%) died. One hundred and eight health care staff were infected during the study period. DISCUSSION: COVID-19 was most commonly seen in patients who underwent KTx and received HD. The combined immunosuppressive therapy and frequent exposure to the hospital setting may increase these patients' susceptibility. Staff infections before vaccination era were alarming, various precautions should be taken for infection control, particularly optimal vaccination coverage.

Comparison of pediatric patients with noninfectious idiopathic uveitis and noninfectious uveitis associated with an underlying systemic disease: from a referral center in Turkey.

Aim: The aim of this study was to determine the demographic, clinical, treatment, and outcome features of pediatric noninfectious uveitis patients at a Turkish tertiary center.Materials and methods: This retrospective cohort study included 101 pediatric patients with noninfectious uveitis. Location of uveitis, laterality, age at onset of uveitis, complications of uveitis, duration of follow-up, associated systemic diseases, laboratory findings, medications used, and status of uveitis at the time of data collection were obtained from the patients' files. The patients were divided into two groups according to etiology of uveitis, as follows; group 1: idiopathic noninfectious uveitis; group 2: noninfectious uveitis in association with an underlying systemic disease. Clinical findings were compared between the two groups.Results: Mean age at diagnosis of uveitis was 9.36 ± 4.24 years and mean duration of follow-up was 48.4 ± 9.2 months. Anterior uveitis was the most common form, accounting for 69.3% (n = 70) of the patients. There were 67 (66.3%) patients in group 1 and 34 (33.7%) patients in group 2. Mean age at diagnosis of uveitis was lower in group 2 (p < 0.05). Although gender, type of inflammation, laterality of eye involvement, and mean age at initiation of biological treatment did not differ between the groups; symptomatic uveitis was more common in group 1, whereas anterior uveitis was also more common in group 2 (p < 0.05). At the time of data collection 16 (15.8%) of the patients had active uveitis. Ocular complications were observed fewer in idiopathic noninfectious uveitis group than noninfectious uveitis associated with an underlying systemic disease (p < 0.05). Biological therapy was administered in 26.8% of the patients in group 1, versus 67.6% of those in group 2. Ocular complications were observed in 22.8% of the patients at presentation or during the follow-up.Conclusion: Herein a large retrospective cohort of noninfectious pediatric uveitis has been described. The findings indicate that noninfectious idiopathic uveitis is characterized by more symptomatic, later onset, and fewer complications than noninfectious uveitis associated with an underlying systemic disease.

Retrospective evaluation of children with unilateral renal agenesis.

BACKGROUND: Children born with unilateral renal agenesis (URA) are thought to have a risk of developing hypertension, proteinuria, and progressive chronic kidney disease (CKD). The present study aimed to evaluate the long-term prognosis and clinical characteristics of children with URA. METHODS: The study included 171 patients aged < 18 years diagnosed as URA who were followed-up for ≥ 1 year and 121 healthy controls matched for age, gender, and BMI. RESULTS: Median age at diagnosis was 2 years (IQR: 1 month-16 years) and the incidence of URA in males (65.4%) was higher than in females. Among the patients, 21 (12.2%) had other urinary system anomalies. It was noted that 2.3% of the patients had proteinuria, 15.2% had hyperfiltration, and 2.9% had CKD. Hypertension based on ambulatory blood pressure monitoring (ABPM) was diagnosed in 18 (10.5%) of the patients, of whom 10 had masked hypertension. Diastolic blood pressure in the URA patients was significantly higher than in the healthy controls. The incidence of hypertension and CKD was significantly higher in the patients with other urinary system anomalies. CONCLUSIONS: Patients with a single functional kidney should be periodically evaluated throughout their lifetime for urine protein, blood pressure, and kidney functions. The most remarkable finding of this study is the importance of the use of ABPM for evaluating blood pressure in pediatric URA patients, especially for the detection of masked hypertension and the non-dipper phenomenon, which cannot be achieved with office blood pressure measurement.

Similarities between pediatric FMF patients with sacroiliitis and pediatric juvenile spondyloarthropathy patients with sacroiliitis: a preliminary study.

Objective: Familial Mediterranean fever (FMF) is an autosomal recessive disease characterized by recurrent, self-limited attacks of fever with serositis. Acute recurrent arthritis is the most common form of musculoskeletal involvement in FMF; however, ≤5% of FMF patients can develop chronic arthritis, including sacroiliitis. It is difficult to determine if sacroiliitis is a musculoskeletal finding of FMF or if they are concomitant diseases-FMF and juvenile spondyloarthropathy (JSpA). The present study aimed to compare clinical and laboratory findings in FMF patients with concomitant sacroiliitis and JSpA patients with concomitant sacroiliitis.Materials and Methods: The medical files of patients diagnosed with FMF and JSpA with concomitant sacroiliitis were retrospectively evaluated. All patients had MRI findings consistent with sacroiliitis. Patient demographic data, clinical features, and laboratory findings were compared between the patients with FMF and concomitant sacroiliitis, and those with JSpA and concomitant sacroiliitis.Results: The study included 18 patients with FMF and sacroiliitis, and 38 patients with JSpA and sacroiliitis. The median (range) age at diagnosis of FMF accompanied by sacroiliitis and JSpA accompanied by sacroiliitis was 12.0 years (3.5-18 years) and 13 years (4-18 years), respectively. There weren't any significant differences in HLA-B27 positivity, family history of ankylosing spondylitis, presenting complaints, arthritis, enthesitis, or treatment between the 2 patient groups.Conclusion: The present findings show that pediatric patients with FMF and sacroiliitis, and those with JSpA and sacroiliitis have the same clinical and laboratory findings.

Clinical features in 305 patients with juvenile idiopathic arthritis: A single center Turkish Study.

BACKGROUND: Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatologic disease of childhood. The various subtypes of JIA differ in clinical features and treatments. The aim of this study was to analyze the frequency of JIA subtypes, patient demographic and clinical features, as well as the rates of macrophage activation syndrome, uveitis, and remission in Turkish JIA patients treated at a single center, and to compare the findings to those in the literature. METHODS: The files of all JIA patients treated at our pediatric rheumatology department between January 2017 and January 2019 were retrospectively reviewed. Patient demographic, clinical, and laboratory data were obtained from the patients' files and the hospital database. RESULTS: The study included 305 patients (180 females) with a mean age at onset of 7.83 ± 4.62 years. Among all the JIA subtypes, the most frequent was oligoarthritis (41.6%), followed by enthesitis-related arthritis (29.2%), rheumatoid factor (RF)-negative polyarthritis (13.4%), systemic arthritis (9.5%), RF-positive polyarthritis (2.6%), psoriatic arthritis (2.0%), and undifferentiated arthritis (1.6%). At the time of data collection, 278 patients (91.0%) were in remission, whereas 27 patients (9.0%) had active disease. Macrophage activation syndrome developed in 12 of the 29 (41.0%) systemic arthritis. Uveitis was noted in 32 (10.0%) patients. Biological agents were administered in 142 of the patients. CONCLUSIONS: The available data indicate that JIA as a whole is a heterogeneous disease with significant variability in course and long-term outcome. As such, each patient should be evaluated according to his / her disease subtype.

Clinical characteristics of childhood acute tubulointerstitial nephritis.

BACKGROUND: Acute tubulointerstitial nephritis (ATIN) is a rare cause of acute kidney injury in children that can lead to chronic kidney disease. The aim of this study was to describe the presenting features, etiology, and clinical characteristics of childhood ATIN, and to evaluate treatment modalities and renal outcomes. METHODS: The study included 38 patients who had been diagnosed with ATIN, were younger than 18 years old, and were admitted for at least 6 months of follow up. RESULTS: The median age at diagnosis was 13.1 years (range 1.2-16.6 years). The female / male ratio was 1.37. The most common symptoms were abdominal pain (65.7%) and nausea / vomiting (55.2%). Twenty-three of the patients had a history of drug intake (60.5%), and the most active drugs in the etiology were non-steroidal anti-inflammatory drugs (56.5%). Eleven patients were given steroid therapy due to severe kidney involvement and rapid increases in serum creatinine values, and ten of those patients were drug-related ATIN cases. Other patients were given symptomatic treatment. Four patients needed several sessions of dialysis due to the severity of their acute renal failure. The follow-up creatinine and estimated glomerular filtration rate levels were not statistically different between the symptomatic and corticosteroid treated groups (P > 0.05). CONCLUSIONS: Although our study was unable to show the beneficial effect of corticosteroid therapy on the extent of renal recovery, the use of steroids may be preferable in severe nephritis because of the rapid recovery of estimated glomerular filtration rate with corticosteroid therapy.

A case of Type 1 Dent disease presenting with isolated persistent proteinuria.

Dent disease is a rare X-linked recessive tubular disorder, characterized by the triad of low molecular-weight proteinuria, hypercalciuria, nephrocalcinosis and/or nephrolithiasis. It is caused by mutations in the CLCN5 gene or OCRL gene. Thirty to 80% of affected males develop end-stage kidney disease between the ages of 30 and 50 years. Some children were reported to present with isolated persistent proteinuria and a part of these patients were diagnosed as having focal segmental glomerulosclerosis with kidney biopsy. Although there is no specific treatment, treatment of proteinuria and hypercalciuria is thought to delay the progression of the disease. For this reason, awareness of the disease findings and early diagnosis are important. In this case report, we present a boy followed-up with isolated persistent proteinuria and then diagnosed as having Dent disease with mutation analysis that showed c.328_330delT (p.Phe110Trpfs27*) in the CLCN5 gene. The importance of researching low-molecular- weight proteinuria and considering Dent disease in the differential diagnosis of children presenting with isolated persistent proteinuria has been emphasized.

Clinicopathological Assessment of Kidney Biopsies in Children with Familial Mediterranean Fever: A Single-Center Experience.

OBJECTIVES: Familial Mediterranean fever (FMF) is a monogenic auto-inflammatory disease which might rarely cause glomerulopathy in patients. The aim of this study was to determine the clinical, demographic, and genetic characteristics and type of glomerular lesions in pediatric FMF patients who underwent kidney biopsy. METHODS: The data of 30 pediatric FMF patients with biopsy-proven glomerulopathy were retrospectively reviewed. Patients were grouped into 2 categories as amyloid nephropathy (AN, n = 16) and non-amyloid nephropathy (N-AN, n = 14). RESULTS: The mean age at FMF diagnosis was 7.2 ± 3.0 years. The AN group showed higher rates of hypertension, higher levels of 24-h protein excretion and serum creatinine, and lower estimated glomerular filtration rate at the time of kidney biopsy. The rate of ESRD was found to be higher in the AN group (p = 0.011). Mesangioproliferative glomerulonephritis was the most common pathology in the N-AN group (21.4%). The frequency of amyloidosis was significantly higher in patients with homozygous p.M694V mutations than non-homozygous p.M694V mutations (p = 0.039). CONCLUSIONS: In children with FMF, nephropathy is rare. To our knowledge, this is the first study performed in pediatric FMF patients exploring amyloid and non-amyloid glomerulopathies. Patients with AN had higher rates of proteinuria, lower estimated glomerular filtration rate levels, and higher blood pressure than N-AN patients at the time of biopsy.

Effects of hemodialysis access type on right heart geometry in adolescents.

INTRODUCTION: This study aimed to investigate the complication frequency and the changes in right heart geometry with different access types in the pediatric population. METHODS: We included 32 consecutive patients aged between 10 and 19 and who underwent hemodialysis sessions via permanent hemodialysis catheter (nHC = 18) or arterio-venous fistula (nAVF = 14) between January 2013 and March 2018. We recorded and compared the complication frequency and the changes in echocardiography findings with different access types. FINDINGS: Demographic data were similar in both groups. Number of new access creation (nHC = 15 vs nAVF = 1) and all complications (nHC = 19 vs nAVF = 6) were significantly higher in hemodialysis catheter group and the statistical analysis showed the superiority of arterio-venous fistula group in comparison of event-free survival (event-free patients; nAVF = 8 (57%), nHC = 3 (16%); p = 0.02). Control echocardiography showed impressive delta-change in right atrium diameter (p = 0.04), right ventricular end-diastolic volume (p = 0.004), right ventricular end-systolic volume (p < 0.001), and right ventricular free wall thickness (p = 0.009) in arterio-venous fistula group, but no significant difference between two groups in terms of delta-change of right ventricular ejection fraction (p = 0.35), fractional area change (p = 0.21), and tricuspid annular plane systolic excursion (p = 0.13) parameters. CONCLUSION: Arterio-venous fistula has lower risk of complications, but overloading stress on right heart chambers triggers remodeling process and geometrical changes, which can be early pieces of evidence of delayed right heart dysfunction in pediatric hemodialysis patients.

Pediatric electrical injury in Turkey: Five year retrospective hospital-based study.

BACKGROUND: We aimed to evaluate children who presented with electrical injury to the emergency department. We tried to reveal the complications of these patients and the measures to be taken in this regard. METHODS: Medical records of pediatric patients who were admitted with electrical injury to emergency department between January 2007 and January 2012 were retrospectively reviewed. An information form was completed about demographic data, clinical features, exposure place, electrical current type, clinical course, duration of hospitalization, electrocardiography, echocardiography and other systems findings. RESULTS: Children who were the most exposed to electrical injury were children 4 years and under. While low-voltage current exposure was more common in younger ages, high-voltage current exposure was more common in older ages. Most of our patients (%72) were exposed to low voltage. Patients who exposed to high-voltage electricity had significantly higher levels of CPK, CK-MB, LDH, AST, and ALT compared to patients who were exposed to low voltage electricity. None of the patients have died. CONCLUSION: Young children are often exposed to electric shock at home. Increasing precautions to be taken at home reduces electrical shock injuries. Children and especially families should be educated in this regard.

A retrospective analysis of children with Henoch-Schonlein purpura and re-evaluation of renal pathologies using Oxford classification.

BACKGROUND: Henoch-Schönlein purpura (HSP) is the most common vasculitis in childhood. The long-term prognosis is variable and depends on renal involvement. The aims of this study were to investigate the clinical and laboratory characteristics of our HSP patients, to identify the risk factors for the development of Henoch-Schönlein purpura nephritis (HSPN) and to assess the efficacy of the Oxford Classification system for predicting renal outcomes. METHODS: We performed a retrospective review of HSP patients who admitted to our center between 2001 and 2016, and were < 18 years on admission. RESULTS: A total of 1120 children with HSP were analyzed. Their mean age was 7.4 ± 3.4 years. At onset, purpura was present in all cases, arthritis/arthralgia in 42.4%, abdominal involvement in 39% and renal involvement in 37%. Risk factors for the development of nephritis were age ≥ 8 years, atypical distribution of purpura, ESR > 20 mm/h and abdominal pain. Renal biopsy was performed on 75 patients before immunosuppressive treatment. The mesangial score was strongly associated with proteinuria. Segmental glomerulosclerosis, tubular atrophy/interstitial fibrosis, and crescent formation of ≥ 50% were associated with reduced eGFR at the time of biopsy. A Kaplan-Meier plot showed that segmental glomerulosclerosis and tubular atrophy/interstitial fibrosis significantly predict poor renal outcome. CONCLUSION: The long-term morbidity of HSP is predominantly attributed to renal involvement. Patients with HSP, who have a high risk to develop nephritis, could be followed for longer periods of time. The Oxford classification is useful in predicting long-term outcomes of HSPN.

MEFV gene mutations in children with Henoch-Schönlein purpura and their correlations-do mutations matter?

OBJECTIVE: To explore the frequency of MEFV gene mutations in children with Henoch-Schönlein purpura who had no prior familial Mediterranean fever diagnosis and to evaluate the association of MEFV mutations with the clinical and laboratory features of Henoch-Schönlein purpura. METHODS: Data of 1120 patients diagnosed with Henoch-Schönlein purpura were reviewed retrospectively. The spectrum and degree of organ involvement and acute phase reactant levels were documented for each patient. Blood for MEFV gene mutation analysis was obtained either at the time of the Henoch-Schönlein purpura diagnosis or during follow-up visits. Pathological specimens of patients who underwent biopsy (renal/skin) were evaluated with special consideration for immunofluorescent examinations. RESULTS: Two hundred and thirty-eight (21.3%) patients were found to have one of the MEFV mutations in which exon 10 mutations were the most common (16.7%). Abdominal pain, joint involvement, scrotal involvement, and relapse were more frequent, and acute-phase reactant levels were significantly high in patients with MEFV mutations. More severe characteristics were observed in the presence of homozygous exon 10 mutations. There was no significant association between exon 2 variants and clinical course of Henoch-Schönlein purpura. Patients carrying MEFV mutations did not have significantly higher levels of IgA deposits in the biopsy materials. CONCLUSION: Henoch-Schönlein purpura in patients with homozygous exon 10 MEFV mutations seems to be more severe than that in patients carrying other mutations. In patients with exon 10 MEFV mutations, Henoch-Schönlein purpura might be considered as an associated presentation of familial Mediterranean fever rather than a separate clinical entity. Key points • p.M694V mutation is more common in Henoch-Schönlein purpura than in the general population. • p.E148Q variants have no impact on clinical symptoms and laboratory findings in Henoch-Schönlein purpura patients. • The majority of Henoch-Schönlein purpura patients with familial Mediterranean fever have no IgA deposits. • Henoch-Schönlein purpura in familial Mediterranean fever patients may be considered as an integral clinical feature of familial Mediterranean fever.

Dynamic Thiol/Disulphide Homeostasis in Children with Nephrotic Syndrome.

BACKGROUND: There is a growing body of evidence showing that there is an association between oxidative damage and kidney injury in patients with nephrotic syndrome (NS). Dynamic thiol/disulphide homeostasis (DTDH) is an important antioxidant system. The aim of this study was to determine if DTDH could be used as a biomarker of antioxidant status in pediatric NS patients using a novel automated method. METHODS: The study included 39 patients with NS and 40 healthy controls. The DTDH level was measured using a calorimetric and automated method developed by Erel and Neselioglu. In the NS group, DTDH was measured during first-attack episode, relapse, and remission. RESULTS: The native thiol, total thiol, and disulphide levels were significantly lower, the native thiol/total thiol ratio was significantly lower, and the disulphide/native thiol and disulphide/total thiol ratios were significantly higher in the NS patients during relapse and first-attack episode than during remission and in the controls. In addition, the native thiol and total thiol levels, and the native thiol/total thiol ratio were significantly lower, and the disulphide/native thiol and disulphide/total thiol ratios were significantly higher in the NS group during remission than those in the control group. CONCLUSIONS: The present findings show that DTDH shifted toward disulphide formation in the NS patients, even during remission. A clearer understanding of the mechanism of this disruption to homeostasis in NS patients might lead to the development of novel therapeutic strategies.

Value of renal pelvic diameter and urinary tract dilation classification in the prediction of urinary tract anomaly.

BACKGROUND: The aim of this study was to identify the cut-offs of postnatal anteroposterior renal pelvic diameter (APRPD), according to the urinary tract dilation (UTD) classification system, to identify the predictors of final diagnosis of UTD and the need for surgery. METHODS: A total of 260 infants (336 renal units) with prenatally detected UTD were prospectively evaluated on serial ultrasonography by the same radiologist. Additional voiding cystourethrography and scintigraphy was done according to the clinical algorithm. RESULTS: Prenatal and postnatal APRPD in patients with transient dilation were significantly lower than in those with urinary tract anomalies (UTA). On follow up, the slope of decrease in APRPD was significantly higher in transient dilation compared with UTA. APRPD 10 mm at first-month ultrasonography, predicted UTA with a sensitivity of 83.1%, and specificity of 71.1%. On multivariate analysis the likelihood of surgical intervention and final diagnosis were predicted independently by the UTD system risk group. CONCLUSIONS: Careful ultrasonography evaluation can avoid unnecessary testing in patients with transient or clinically insignificant dilation. The UTD classification system is valid for evaluation of postnatal hydronephrosis and is reliable in predicting the need for surgical intervention.