The value of 2019 EULAR/ACR classification criteria in predicting lupus nephritis in childhood-onset systemic lupus erythematosus.

BACKGROUND: We investigated the role of European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) classification criteria for the prediction of LN among children with SLE. METHODS: The data of the patients with childhood-onset SLE diagnosed based on 2012 Systemic Lupus International Collaborating Clinics (SLICC) criteria were retrospectively evaluated. Based on 2019 EULAR/ACR classification criteria, the scoring was done at the time of renal biopsy. RESULTS: Fifty-two patients (12 with LN, 40 without LN) were included. The mean score was higher in patients with LN than those without (30.8±6.14, 19.8±7.76, respectively, p=0.000). The score value had indicative value for LN (area under curve [AUC]:0.863±0.055, cut-off value:22.5, p=0.000). Lymphocyte counts had a predictive value for LN (cut-off value:905/mm3, AUC:0.688±0.087, p=0.042). The score was positively associated with SLE disease activity index (SLEDAI) and activity index (r=0.879, p=0.000; r=0.811, p=0.001, respectively). There were significant negative associations between score value and GFR (r=-0.582, p=0.047). The patients with renal flare had higher the mean score than those of without renal flare (35±2/25.4±5.57, respectively, p=0.019). CONCLUSIONS: The EULAR/ACR criteria score could reflect the activity of disease and severity of nephritis in childhood-onset SLE. A point of 22.5 as score value might be an indicator for LN. During scoring, it should be taken into account that lymphopenia might guide the prediction of LN.

Diagnostic Values of Immature Granulocytes Detected by the Sysmex XN 9000 Hematology Analyzer in Children with Urinary Tract Infections.

Immature granulocytes (IGs) are used as markers of infection and systemic inflammation. We aimed to investigate the diagnostic value of IGs in children with urinary tract infections (UTIs). Children with their first UTIs were included in this observational study. Blood samples were obtained before antibiotic therapy. The blood analysis was repeated 2 weeks after the treatment ended. In total, 194 children (95 with febrile UTI, 58 with cystitis, and 41 controls) were included. The percentage of IGs (IG%) and IG count (IGC) measured at the time of admission were higher in the patients with febrile UTI than in the patients with cystitis and the controls (P = 0.000). The IGC and IG% after treatment were higher in patients with renal scarring than in those without scarring (P = 0.012 and P = 0.021, respectively). Cox's regression analysis showed the significant associations of renal scarring with both IGC and IG% (hazard ratio: 8.181, P = 0.002; hazard ratio: 5.106, P = 0.033, respectively). Both IGC and IG% were positively associated with severe vesicoureteral reflux (VUR) [odds ratio (OR): 22.235, P = 0.025; OR: 15.597, P = 0.038, respectively]. In conclusion, the IG% and IGC, which can be easily measured in a routine complete blood count without the need for additional effort, could be used as biomarkers for predicting febrile UTI, renal scarring, and severe VUR in children.

A case of familial recurrent 17q12 microdeletion syndrome presenting with severe diabetic ketoacidosis.

BACKGROUND: Heterozygous intragenic mutations of the hepatocyte nuclear factor 1 homeobox b gene (HNF1B) located on chromosome 17 and microdeletion of 17q12 region (17q12MD) leads to the complete loss of this gene, which causes renal cystic disease, diabetes mellitus (MODY5), hypomagnesemia, hyperuricemia, liver enzyme abnormalities, genital tract abnormalities and exocrine pancreatic insufficiency. In addition, patients with 17q12MD also have facial dysmorphism, neuro-developmental and neuropsychiatric disorders. CASE: A 16-year-old girl with obesity and mild facial dysmorphism was admitted to the hospital with symptoms of diabetes that started two days prior to her admission. She was diagnosed with severe diabetic ketoacidosis and treated accordingly. She had been followed up with the diagnoses of multicystic renal disease, hydronephrosis, hepatosteatosis, hypomagnesemia and hyperuricemia since the age of six. She had mild intellectual disability. Her menarche started two months ago. Cranial magnetic resonance imaging revealed mild diffuse cerebral and cerebellar atrophy and a partial empty sella. Her mother had diabetes, hypomagnesemia and mild intellectual disability and her maternal grandfather and uncle had diabetes. Her grandfather also had renal cystic disease. All of them are on oral antidiabetic medication. The genetic analysis of the patient and her mother revealed a loss of 1.6 megabases in chromosome 17q12. CONCLUSIONS: MODY5 should be kept in mind in patients with diabetes who present with extra pancreatic findings, especially with renal cystic disease, more over, a genetic analysis including the study of 17q12MD should be carried out in patients who present with additional neuropsychiatric findings. Ketoacidosis can be seen in patients with MODY5. Ketoacidosis and renal anomalies and dysfunction are factors that increase and affect the severity of each other in these patients.

Urine hepcidin, netrin-1, neutrophil gelatinase-associated lipocalin and C-C motif chemokine ligand 2 levels in multicystic dysplastic kidney / Níveis de hepcidina, netrina-1, lipocalina associada a gelatinase neutrofílica e ligante de quimiocina C-C motif na urina do rim displásico multicístico

ABSTRACT Introduction: Glomerular hyperfiltration may lead to proteinuria and chronic kidney disease in unilateral multicystic dysplastic kidney (MCDK). We aimed to investigate the urine neutrophil-gelatinase-associated lipocalin (NGAL), netrin-1, hepcidin, and C-C motif chemokine ligand-2 (MCP-1/CCL-2) levels in patients with MCDK. Methods: Thirty-two patients and 25 controls were included. The urine hepcidin, netrin-1, NGAL, and MCP-1/CCL-2 levels were determined by ELISA. Results: The patients had higher serum creatinine (Cr) levels, urine albumin, and netrin-1/Cr ratio with lower GFR. There were positive correlations between urine protein/Cr, MCP-1/CCL-2/Cr, and netrin-1 with NGAL (r = 0.397, p = 0.031; r = 0.437, p = 0.041, r = 0.323, p = 0.042, respectively). Urine netrin-1/Cr was positively correlated with MCP-1/CCL-2/Cr (r = 0.356, p = 0.045). There were positive associations between the presence of proteinuria and netrin-1/Cr, MCP-1/CCL-2/Cr, and NGAL/Cr [Odds ratio (OR): 1.423, p = 0.037, OR: 1.553, p = 0.033, OR: 2.112, p = 0.027, respectively)]. ROC curve analysis showed that netrin-1/Cr, MCP-1/CCL-2/Cr, and NGAL/Cr had high predictive values for determining proteinuria p = 0.027, p = 0.041, p = 0.035, respectively). Urine hepcidin/Cr was negatively correlated with tubular phosphorus reabsorption and was positively correlated with urine NGAL/Cr (r = -0.418, p = 0.019; r = 0.682, p = 0.000; respectively). Conclusions: MCP-1/CCL-2 may play a role in the development of proteinuria in MCDK. Netrin-1 may be a protective factor against proteinuria-induced renal injury. Urine hepcidin/Cr may reflect proximal tubule damage in MCDK. Urine NGAL/Cr may be a predictor of tubule damage by proteinuria.

Resumo Introdução: A hiperfiltração glomerular pode causar proteinúria e doença renal crônica no rim displásico multicístico unilateral (RDM). Nosso objetivo foi investigar os níveis de lipocalina associada à gelatinase neutrofílica na urina (NGAL), netrina-1, hepcidina e quimiocina C-C com ligante-2 (MCP-1/CCL-2) em pacientes com RDM. Métodos: Trinta e dois pacientes e 25 controles foram incluídos. Os níveis urinários de hepcidina, netrin-1, NGAL e MCP-1/CCL-2 foram determinados por ELISA. Resultados: Os pacientes apresentaram níveis séricos mais elevados de creatinina (Cr), albumina na urina e relação netrina-1/Cr com menor TFG. Houve correlação positiva entre proteína na urina/Cr, MCP-1/CCL-2/Cr e netrina-1 com NGAL (r = 0,397, p = 0,031; r = 0,437, p = 0,041, r = 0,323, p = 0,042, respectivamente). A netrina-1/Cr na urina foi correlacionada positivamente com MCP-1/CCL-2/Cr (r = 0,356, p = 0,045). Houve associações positivas entre a presença de proteinúria e netrina-1/Cr, MCP-1/CCL-2/Cr e NGAL/Cr [Odds ratio (OR): 1,423, p = 0,037, OR: 1,553, p = 0,033, OR: 2,112, p = 0,027, respectivamente) ]. A análise da curva ROC mostrou que netrina-1/Cr, MCP-1/CCL-2/Cr e NGAL/Cr apresentaram altos valores preditivos para determinar a proteinúria p = 0,027, p = 0,041, p = 0,035, respectivamente). A hepcidina/Cr na urina foi correlacionada negativamente com a reabsorção tubular de fósforo e positivamente com a NGAL/Cr na urina (r = -0,418, p = 0,019; r = 0,682, p = 0,000; respectivamente). Conclusões: MCP-1/CCL-2 pode ter participação no desenvolvimento de proteinúria no RDM. A Netrina-1 pode ser um fator protetor contra lesão renal induzida por proteinúria. Hepcidina/Cr na urina pode refletir danos em túbulos proximais no RDM. O valor de NGAL/Cr urinário pode ser um preditor de danos nos túbulos por proteinúria.

Urine hepcidin, netrin-1, neutrophil gelatinase-associated lipocalin and C-C motif chemokine ligand 2 levels in multicystic dysplastic kidney.

INTRODUCTION: Glomerular hyperfiltration may lead to proteinuria and chronic kidney disease in unilateral multicystic dysplastic kidney (MCDK). We aimed to investigate the urine neutrophil-gelatinase-associated lipocalin (NGAL), netrin-1, hepcidin, and C-C motif chemokine ligand-2 (MCP-1/CCL-2) levels in patients with MCDK. METHODS: Thirty-two patients and 25 controls were included. The urine hepcidin, netrin-1, NGAL, and MCP-1/CCL-2 levels were determined by ELISA. RESULTS: The patients had higher serum creatinine (Cr) levels, urine albumin, and netrin-1/Cr ratio with lower GFR. There were positive correlations between urine protein/Cr, MCP-1/CCL-2/Cr, and netrin-1 with NGAL (r = 0.397, p = 0.031; r = 0.437, p = 0.041, r = 0.323, p = 0.042, respectively). Urine netrin-1/Cr was positively correlated with MCP-1/CCL-2/Cr (r = 0.356, p = 0.045). There were positive associations between the presence of proteinuria and netrin-1/Cr, MCP-1/CCL-2/Cr, and NGAL/Cr [Odds ratio (OR): 1.423, p = 0.037, OR: 1.553, p = 0.033, OR: 2.112, p = 0.027, respectively)]. ROC curve analysis showed that netrin-1/Cr, MCP-1/CCL-2/Cr, and NGAL/Cr had high predictive values for determining proteinuria p = 0.027, p = 0.041, p = 0.035, respectively). Urine hepcidin/Cr was negatively correlated with tubular phosphorus reabsorption and was positively correlated with urine NGAL/Cr (r = -0.418, p = 0.019; r = 0.682, p = 0.000; respectively). CONCLUSIONS: MCP-1/CCL-2 may play a role in the development of proteinuria in MCDK. Netrin-1 may be a protective factor against proteinuria-induced renal injury. Urine hepcidin/Cr may reflect proximal tubule damage in MCDK. Urine NGAL/Cr may be a predictor of tubule damage by proteinuria.

A severe course of serogroup W meningococcemia in a patient with infantile nephropathic cystinosis.

We present a 9-month old boy with cystinosis admitted to our hospital with the complaints of vomiting, diarrhea and seizure. While he was hospitalized in a pediatric intensive care unit due to worsening of his signs related to cystinosis, within hours, he suffered complications of septic shock, acute renal failure, and disseminated intravascular coagulation, due to invasive Neisseria meningitidis serogroup W disease. Our patient is the first reported case of invasive meningococcal disease with cystinosis. Clinicians should consider that the unexpected and serious clinical findings of invasive meningococcal disease can mimic and/or masquerade as other metabolic diseases. Vaccination strategies, according to serogroup epidemiology and age distribution, should be implemented for the prevention of meningococcal infections.

Hemotologic Indices for Predicting Internal Organ Involvement in Henoch-Schönlein Purpura (IgA vasculitis).

Henoch-Schönlein purpura is the most common vasculitis of childhood. This study investigated the values of hematologic indices that can help predict internal organ involvement. The study included 112 patients followed up between January 2007 and May 2017 and 81 healthy children. Leukocyte, neutrophil, monocyte, lymphocyte and platelet counts, neutrophil-to-lymphocyte ratio (NLR), platelet-to-lymphocyte ratio (PLR), and C-reactive protein (CRP) levels were compared between patients with and without internal organ involvement. Overall, 57 (50.8%) patients had internal organ involvement. Leukocyte, neutrophil, and monocyte counts, NLR, and CRP levels were significantly higher in patients with internal organ involvement than in patients without internal organ involvement. There was no difference between the groups in terms of lymphocyte count, platelet count, and PLR. The cutoff values were found to be ≥10.8×10/L [area under the curve (AUC), 0.734] for leukocyte, ≥6.0×10/L (AUC, 0.665) for neutrophil, ≥0.710×10/L (AUC, 0.681) for monocyte, ≥3.95×10/L (AUC, 0.609) for NLR, and 2.41 mg/dL (AUC, 0.635) for CRP. Logistic regression analysis revealed that leukocyte count is a risk factor for internal organ involvement. Leukocyte, neutrophil, monocyte counts, NLR, and CRP levels are useful in predicting internal organ involvement in the acute phase of Henoch-Schönlein purpura. Leukocyte count is an important risk factor for internal organ involvement and its predictive value is more reliable than the other hematologic indices.

Serum vasohibin-1 and suppression of tumorigenicity-2 levels in children with predialysis chronic kidney disease

Background/aim: Chronic kidney disease (CKD), which is one of the most important health problems worldwide, could be considered as an immune inflammatory disease. A prognostic biomarker may be helpful in determining the progression of CKD in children. We aimed to investigate the serum vasohibin-1 and soluble suppression of tumorigenicity-2 (sST2) levels as potential biomarkers in children with predialysis CKD. Materials and methods: Forty-seven children with stage 2-4 CKD and 20 healthy controls were included in this cross-sectional study. Glomerular filtration rate (GFR) and urinary excretion of protein were measured in 24-h urine samples. Serum vasohibin-1 levels and sST2 were measured. The results were expressed as pg/mL and ng/mL, respectively. Results: Serum vasohibin-1 levels were similar between the patients and the control group (P > 0.05), but serum vasohibin-1 levels were higher in patients with proteinuria than in nonproteinuric patients (2574.5 ± 701.60 vs. 1822.4 ± 300.32 pg/mL, P = 0.001). A positive correlation was found between serum vasohibin-1 levels and 24-h urine protein values in patients (P = 0.036). Serum sST2 levels were higher in patients than the control group (P = 0.013). The patients with hypertension had higher sST2 levels than normotensive patients (P = 0.015). Serum vasohibin-1 and sST2 levels were not correlated with age, GFR, albumin, hemoglobin, or PTH levels. Conclusion: Serum vasohibin-1 and sST2 levels were not associated with decline in renal function. Elevated serum vasohibin levels may be a compensatory response to proteinuria in patients with predialysis CKD. The measurement of serum sST2 levels might contribute to early detection of hypertension in patients.

Foreign Body Reaction to Dialysis Chatheter and Peritoneal Fluid Eosinophilia in a Child on Continuous Ambulatory Peritoneal Dialysis.

Foreign body reaction is a tissue response against implanted materials. We described for the first time the eosinophilic peritonitis and foreign body giant cell reaction to dialysis catheter in a nonatopic child on continuous ambulatory peritoneal dialysis. We found tenderness, redness, and swelling without purulent discharge around the peritoneal catheter; increased eosinophil count in cloudy dialysis fluid; and blood and hyperechoic granulomatous formation appearance surrounding the peritoneal catheter on ultrasonography and foreign body giant cell reaction to dialysis catheter in pathologic examination of granulomatous lesionin in our patient. The peritoneal dialysis catheter was removed due to resistance to antibiotic and antihistamine treatments for suspected peritonitis and tunnel infection. Foreign body reaction and eosinophilic peritonitis with eosinophilic cloudy dialysis effluent can exist simultaneously. Foreign body reaction should be considered in the differential diagnosis of exit site and/or tunnel infection. Ultrasonography helps distinguish between foreign body reaction and exit-site or tunnel infection.