Pneumococcal carriage and serotype distribution in children with nephrotic syndrome.

BACKGROUND: Patients with nephrotic syndrome (NS) are at a higher risk of developing invasive pneumococcal disease (IPD). Pneumococcal carriage studies are helpful tools for detecting potentially infectious serotypes and guiding immunization efforts. Pneumococcal nasopharyngeal colonization is common, and IPD can easily occur in an immunosuppressed state. Limited information is available regarding the frequency of pneumococcal carriage in individuals with NS. The aim of this study was to evaluate pneumococcal carriage and serotype distribution in children with NS. METHODS: Pneumococcal carriage was detected by real-time PCR assays from nasopharyngeal swab samples from 98 children with NS, and 100 healthy controls. Isolates were serotyped by real-time PCR. RESULTS: The pneumococcal carriage rate was 44.9% in children with NS. Regarding the recommendation about pneumococcal immunization in children with NS, the vaccination rate was low. Also, non-PCV13 serotypes have been detected in at least 25% of PCV13-vaccinated children. There is no statistically significant difference in total pneumococcal carriage rate, PCV13 serotype carriage rate, or non-PCV13 serotype carriage rate between children with NS and healthy controls (p > 0.05 for all). CONCLUSIONS: The pneumococcal carriage rate was similar between children with NS and healthy controls. However, because children with NS have an increased risk for IPD, the serotype distribution of children with NS can demonstrate the improved protection offered by new pneumococcal vaccines. Regular monitoring for IPD is crucial for assessing the evolving sero-epidemiology of pneumococcal infections and evaluating the effectiveness of vaccines for children with NS.

Immature granulocytes as biomarkers of inflammation in children with predialysis chronic kidney disease.

BACKGROUND: Chronic inflammation in patients with predialysis chronic kidney disease (CKD) is quite common. We aimed to investigate the relationship of the percentage of immature granulocytes (IG%) and immature granulocyte count (IGC) with inflammation in children with predialysis CKD. METHODS: The data from children with stage 2-4 CKD and a control group of healthy children were evaluated retrospectively. A highly-sensitive C-reactive protein (hs-CRP) level above 5 mg/dL was considered the presence of inflammation. The IGCs were calculated in the white cell differential channel of the Sysmex XN-9000 using the fluorescent flow cytometry method. The IG% was expressed as percentage of total leucocyte concentration. RESULTS: The data from 57 patients (30 stage 2 CKD, 15 stage 3 CKD, 12 stage 4 CKD) and 46 controls were analyzed. hs-CRP levels, IG%, IGC, white blood cell (WBC) and neutrophil counts, and neutrophil-to-lymphocyte ratio (NLR) were higher in patients than the control group (p < 0.000, p < 0.000, p < 0.000, p = 0.001, p = 0.002, p < 0.000, respectively). Both IG% and IGC were positively correlated with hs-CRP, WBC and neutrophil counts, and NLR (r = 0.485, p < 0.000; r = 0.379, p = 0.004; r = 0.543, p < 0.000; r = 0.628, p < 0.000 for IG%; r = 0.379, p = 0.004; r = 0.351, p = 0.007; r = 0.525, p < 0.000; r = 0.601, p < 0.000 for IGC, respectively). A ROC analysis of the relationship between IGC, IG%, and inflammation showed IGC and IG% had predictive value for the presence of inflammation (cut-off value: 0.035 × 106/mL, AUC: 0.799 ± 0.061, sensitivity: 74.2%, specifity: 63%, p < 0.001 for IGC; cut-off value: 0.45%, AUC: 0.838 ± 0.056, sensitivity: 70.8%, specifity: 67.3%, p = 0.001 for IG%). CONCLUSIONS: Immature granulocytes may be used as a biomarker of inflammation in children with predialysis CKD. A higher resolution version of the Graphical abstract is available as Supplementary information.

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.

Evaluation of Arterial Stiffness and Carotid Intima-Media Thickness in Children with Primary and Renal Hypertension.

Hypertension is an increasing disease in children and the risk of endothelial damage and target organ damage increases in the presence of additional risk factors such as obesity. In our study, the effect of hypertension on early atherosclerotic changes and target organ damage in children was investigated. Twenty four-hour ambulatory pulse wave analysis was performed by oscillometric method in 71 children aged 8-18 years, 17 of whom were diagnosed with primary hypertension without obesity, 18 had both primary hypertension and obesity, and 16 had renal hypertension. Twenty healthy normotensive children were included as the control group. Carotid intima-media thickness (CIMT) and Left Ventricular Mass Index were measured. Central systolic blood pressure (cSBP), central diastolic blood pressure (cDBP), systolic blood pressure (SBP) and diastolic blood pressure (DBP) were higher in the primary hypertension group compared to controls (p = 0.001, p = 0.005, p = 0.001, p = 0.009, respectively), cSBP was higher in the renal hypertension group than the control group (p = 0.018). There was no difference between the groups in terms of pulse wave analysis parameters, CIMT, or left ventricular mass index (p > 0.05). Pulse wave velocity was positively correlated with SBP, DBP, cSBP, cDBP (p < 0.001). Augmentation index was positively correlated with DBP and cDBP (p = 0.01, p = 0.002, respectively). Our findings show that high blood pressure is associated with arterial stiffness and target organ damage beginning in childhood. The detection of early atherosclerotic vascular changes using pulse wave analysis allows to take necessary precautions such as lifestyle changes to prevent target organ damage in hypertensive children.

Evaluation of cardiovascular risk in children with solitary functioning kidney.

BACKGROUND: The present study investigates cardiovascular risk and kidney damage in patients with solitary kidneys. METHODS: Included in the study were 40 children with a unilateral functioning kidney and 60 healthy controls, all of whom were evaluated for carotid intima-media thickness, ischemia-modified albumin and oxidative stress parameters, and 24-h ambulatory blood pressure monitoring. RESULTS: Serum creatinine and urine microalbumin levels were higher and creatinine clearance was lower in the patient group than in the control group, and serum ischemia-modified albumin, carotid intima-media thickness, aldosterone, plasma renin activity and blood pressure were all higher in the patient group than in the control group. In addition, the patient group was showed a non-dipper pattern. CONCLUSION: Children with a normal functioning solitary kidney are likely at higher risk of developing cardiovascular disease and such patients should be followed closely before marked kidney impairment occurs.

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.

Risk factors for development of urinary tract infection in children with nephrolithiasis.

AIM: Nephrolithiasis is one of the causes of urinary tract infection (UTI). In this study, we investigated risk factors for UTI in children with nephrolithiasis. METHODS: The data from the patients with nephrolithiasis were evaluated in this retrospective follow-up study. Patients with a history of UTI before admission and congenital anomaly of kidney or urinary tract were excluded. The patients were divided into two groups: patients with UTI (recurrent UTI and single UTI) and patients without UTI. RESULTS: A total of 599 patients were included in this study. UTI occurred in 181 (30.2%) patients. There was a positive association between the size of stone and UTI (odds ratio (OR): 1.355, P = 0.007). Receiver operating characteristic curve analysis showed that the cut-off value of the size of stone for UTI was 5.3 mm, with a sensitivity of 74.9% and a specificity of 66.1% (area under the curve: 0.572 ± 0.028, P = 0.013). The presence of a metabolic risk factor and age at diagnosis under 2 years were significantly associated with both UTI and recurrence of UTI (OR: 2.272, P = 0.021, OR: 1.809, P = 0.028, respectively, for metabolic risk factor; OR: 1.212, P = 0.041, OR: 1.122, P = 0.046, respectively, for age at diagnosis under 2 years). Hypercalciuria was significantly associated with the recurrence of UTI (OR: 1.854, P = 0.017). CONCLUSIONS: The age at diagnosis, the presence of a metabolic risk factor and size of stone are significant risk factors for UTI in children with nephrolithiasis. The patients with idiopathic hypercalciuria, metabolic risk factor and age at diagnosis under 2 years have increased risk of recurrence of UTI.

Serum albumin and von Willebrand factor: possible markers for early detection of vascular damage in children undergoing peritoneal dialysis.

PURPOSE: Cardiovascular diseases are the main causes of morbidity and mortality in children with end-stage renal disease and the relationships among several relevant potential biomarkers were investigated in pediatric peritoneal dialysis patients. METHODS: Serum homocysteine, von Willebrand factor (vWF), apolipoproteins A and B, lipoprotein-a, high sensitive-CRP, hemoglobin, phosphorus and parathyroid hormone (PTH) levels, systolic (SBP) and diastolic (DBP) blood pressure, carotid intima-media thickness (cIMT) and left ventricular mass index (LVMI) were measured in 21 pediatric peritoneal dialysis patients and control subjects. RESULTS: All risk factors were higher in patients than controls. LVMI values were negatively correlated with hemoglobin and positively correlated with PTH and phosphorus levels (p=0.007, r= - 0.573; p=0.013, r= 0.532 and p=0.035, r= 0.461, respectively). cIMT was negatively associated with serum albumin and positively correlated with vWF levels and with SBP and DBP (p=0.006, r= - 0.578; p=0.039, r= 0.453; p=0.02, r= 0.503; p=0.024, r= 0.491, respectively). Robust regression analyses showed that hemoglobin was an independent predictor of LVMI and serum albumin was an independent predictor of cIMT. CONCLUSION: Only uremia-related factors were independent risk factors for predicting LVMI and cIMT. Hemoglobin level may be a critical factor in the development of left ventricular hypertrophy; therefore, effective treatment of anemia is crucial. Low serum albumin and high hsCRP and vWF levels, and their correlations with cIMT, indicate these patients could be at risk of developing malnutrition-inflammation-atherosclerosis syndrome and suggest that serum albumin and vWF levels may be useful markers for early detection of vascular damage.

Co-existence of renovascular hypertension, polyarteritis nodosa, antiphospholipid syndrome and methylenetetrahydrofolate reductase mutation.

We present a hypertensive child with a co-existence of polyarteritis nodosa, anti-phospholipid antibodies (aPL), methylenetetrahydrofolate reductase (MTHFR) mutation and increased lipoprotein a level. Elevated renin, aldosterone and aPL levels, micro-aneurysms, occlusion and thrombosis at left and right renal artery were found. Anti-hypertensive agents, prednisolone and pulse cyclophosphamide therapy were started and a stent was inserted in the left renal artery. Two months later, brain magnetic resonance imaging/magnetic resonance imaging angiography showed acute infarct area of the left parietofrontal lobe and middle cerebral artery stenosis. We found bilateral peripheral neuropathy, persistent aPL and elevated Lp(a) level and heterozygous A1298C/MTHFR mutation. Intravenous immunoglobulin and low-molecular-weight heparin treatment was added. In conclusion, our observation suggests that in patients with systemic vasculitis, such as polyarteritis nodosa, aPL are probably associated with greater thrombotic risks. The investigation of the LP(a) levels and MTHFR mutations as a synergic pro-coagulant effect might also be considered for determining patients with vasculitis at risk for severe thrombotic events.

Serum and urinary leptin and ghrelin in children with nephrotic syndrome.

OBJECTIVE: The aim of the present study was to evaluate the serum and urinary levels of leptin and ghrelin in children with primary idiopathic nephrotic syndrome (NS), to compare these results between patients during the relapse and remission phase and to evaluate the possible role of leptin and ghrelin in the pathogenesis of NS. PATIENTS AND METHODS: Forty-nine children with primary idiopathic NS (25 children with relapse and 24 children in remission), who were followed up at the Pediatric Nephrology Unit, enrolled. Twenty-eight age- and sex-matched healthy children served as controls. Serum and urinary leptin levels were determined by immunoenzymatic ELISA, and serum and urinary ghrelin levels were determined by the RIA method. RESULTS: The serum leptin levels were significantly lower in the children with NS during the relapse phase than in the children with NS during remission or in the controls (1.42±0.34 ng/dl and 3.60±0.70 ng/ml; p<0.01, 1.42±0.34 ng/ml and 5.27±4.67 ng/ml; p<0.001, respectively). The urinary leptin excretion levels were significantly higher in the relapse group than in the controls (0.40±0.11 ng/ml and 0.12±0.06 ng/ml, p<0.01, respectively). The serum ghrelin levels were similar between the study groups (p>0.05). The urinary ghrelin excretion levels were significantly higher in the relapse group than in the remission group and the controls (965.0 pg/ml [93-3711] and 679.7 pg/ml [93-3783], p<0.05; 965.0 pg/ml [93-3711] and 387.7 pg/ml [114-1214], p<0.001, respectively). The urinary ghrelin levels were also significantly higher in the remission group than in the controls (679.7 pg/ml [93-3783] and 387.7 pg/ml [114-1214]), p<0.01, respectively). The serum leptin levels were positively correlated with the serum albumin levels (r=0.440, p<0.05) and were negatively correlated with the serum triglyceride levels during the relapse phase. The urinary leptin and ghrelin levels were positively correlated with proteinuria in the relapse group. CONCLUSIONS: We propose that leptin plays a role in the pathophysiology of NS and is associated with proteinuria, hypoproteinemia and hyperlipidemia. The significant urinary excretion of ghrelin in children with NS is possibly due to underlying pathophysiological changes, and normal serum ghrelin levels might be associated with an unknown compensatory mechanism.

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.

Neurological Face of Familial Mediterranean Fever.

OBJECTIVE: Familial Mediterranean fever is a systemic inflammatory disease characterized by recurrent attacks in the form of fever and inflammation of serous membranes. We aimed to search for neurological signs and symptoms of children with familial Mediterranean fever. MATERIALS AND METHODS: Medical records database from 2010 to 2020 was screened retrospectively. In total, 625 children with familial Mediterranean fever were included in the study. Neurological symptoms and associated factors were searched. RESULTS: The mean age at onset of familial Mediterranean fever symptoms and time to diagnosis was calculated as 5.12 ± 3.51 years and 7.27 ± 3.9 years, respectively. The neurological symptoms were present in 142 (23.5%) patients. Headache was the most common symptom. During follow-up, different neurologic diseases were diagnosed in 40 familial Mediterranean fever patients and epilepsy was the most frequent disease. The coexistent disease was present in 49.9% of children with familial Mediterranean fever. Juvenile idiopathic arthritis was found to be a risk factor for the neurologic symptom (P < .05). The frequency of neurological symptoms was higher in patients with E148Q mutation (P < .012). CONCLUSION: The results of the present study revealed that patients with familial Mediterranean fever can present with various central nervous system manifestations. A multidisciplinary approach must be considered in the treatment of these 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.

The risk factors for recurrence of febrile urinary tract infection and renal scarring in children with functional urinary incontinence.

OBJECTIVES: Functional urinary incontinence is often associated with recurrent urinary tract infection (UTI), vesicoureteral reflux (VUR), and renal scarring. This study aims to evaluate the correlations between urodynamic findings and recurrence of UTI, VUR, and renal scarring in children with functional incontinence. METHODS: In this retrospective observational study, data on the presence of VUR, urodynamics and 99Tc-dimercaptosuccinic acid scintigraphy findings, and episodes of febrile UTI were obtained from patients' records. The patients had at least 3 years of follow-up. RESULTS: There were significant associations between recurrence of UTI and decreased bladder capacity (hazard ratio: 1.321, P = .028). The receiver operator characteristic curve analysis showed a cutoff value for compliance of 13.25 mL/cmH2 O for renal scarring (P = .000). There was a significant association between bladder wall thickening and VUR (odds ratio: 2.311, P = .008). The compliance had a cutoff value of 14.7 mL/cm H2 O (P = .023) for severe VUR. The frequency of renal scarring was higher in patients with severe VUR and dysfunctional voiding (P = .001 and P = .041, respectively). The independent risk factors for renal scarring were low compliance, severe VUR, and dysfunctional voiding in children with functional incontinence, but recurrence of febrile UTI was not a risk factor for renal scarring. Decreased bladder capacity was a risk factor for recurrence of febrile UTI. CONCLUSIONS: The present study suggests that low compliance, severe VUR, and dysfunctional voiding, but not the recurrence of febrile UTI, are the independent risk factors for renal scarring in children with functional incontinence, and decreased bladder capacity is the risk factor for the recurrence of febrile UTI.

Serum Presepsin, Proadrenomedullin andTriggering Receptor Expressed on Myeloid Cells-1 (TREM-1) as Biomarkers for the Diagnosis of Acute Pyelonephritis.

OBJECTIVE: To investigate the diagnostic values of serum presepsin, proadrenomedullin (proADM) and triggering receptor expressed on myeloid cells-1 (TREM-1) levels in children with acute pyelonephritis and lower urinary tract infection. METHODS: Peripheral venous blood and urine samples were obtained before starting antibiotic therapy at the time of admission in all patients. Serum TREM-1, presepsin and proADM concentrations were determined by the enzyme-linked immunosorbent assay method. RESULTS: 82 children (38 acute pyelonephritis, 24 lower urinary tract infection, 20 controls) were enrolled. Serum proADM and TREM-1 levels were higher in patients with acute pyelonephritis than those of lower urinary tract infection and controls (P=0.001 and P<0.001, respectively). Both serum proADM and TREM-1 levels had predictive value for diagnosis of acute pyelonephritis (P=0.006 and P<0.001, respectively). ROC analysis showed that proADM and TREM-1 had positive predictive values for diagnosis of acute pyelonephritis (AUC=0.830, P=0.003; and AUC=0.843, P<0.001, respectively). CONCLUSIONS: Serum proADM and TREM-1 levels could serve as early biomarkers for the diagnosis of acute pyelonephritis in children.

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.

Transforming Growth Factor-Beta 1 and Endoglin Levels in Congenital Solitary Functioning Kidney.

INTRODUCTION: Glomerular hyperfiltration leads to hypertension, microalbuminuria, and impaired renal function in children with congenital solitary functioning kidney (cSFK). The purpose of this study was to investigate the associations between serum transforming growth factor ß-1 (TGF) and endoglin levels and hypertension, renal function or microalbuminuria in children with cSFK. MATERIALS AND METHODS: 63 patients and 36 controls were included in the study. Serum endoglin and TGF-ß1 level was measured using ELISA commercial kits. RESULTS: Serum TGF-ß1 and endoglin levels were higher in patients than those of controls (P = 0.04 and P < 0.001, respectively). The prevalence of hypertension was found to be 45.6%. There was a positive association between endoglin levels and the presence of masked hypertension (odds ratio: 1.121, P = 0.04). TGF-ß1 and endoglin levels were positively associated with microalbuminuria (OR: 1.17, P = 0.04; OR: 1.836, P = 0.01). ROC curve analysis showed that serum endoglin and TGF-ß1 levels had predictive value for microalbuminuria (cut-off value: 4.86 ng/mL, sensitivity: 94.7%, specificity: 54.5%, area under the curve ± standard error [AUC ± SE]: 0.888 ± 0.025, P = 0.01 for endoglin; cut-off value 561.24 pg/mL, sensitivity: 89.5%, specificity: 73%, AUC ± SE: 0.995 ± 0.334, P = 0.02 for TGF-ß1). There were no significant relationships between glomerular filtration rate and serum TGF-ß1 or endoglin levels. CONCLUSIONS: Endoglin and TGF-ß1 may play an important role in the pathophysiology of microalbuminuria in cSFK. Endoglin may have a role in the development of hypertension in children with cSFK.

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.

Platelet Activation and Inflammation in Hypertensive Children with Non-dipper and Dipper Status.

INTRODUCTION: The patients with non-dipper hypertension have an increased risk for target organ damage because of inflammation and platelet activation. In this study, we aimed to investigate the association between ambulatory blood pressure monitoring (ABPM) values and inflammation with platelet indices in children with dipper and non-dipper hypertension. MATERIALS AND METHODS: A total of 153 patients who underwent ABPM were included in this retrospective study. The participants were divided into three groups (61 non-dipper hypertensive, 28 dipper hypertensive, 64 normotensive). Neutrophil and platelet count, neutrophil to lymphocyte ratio (NLR), platelet to lymphocyte ratio (PLR), mean platelet volume (MPV), plateletcrit (PCT), and platelet distribution width (PDW) were matched data among groups. RESULTS: The neutrophil counts were higher in the non-dipper and dipper groups compared with the normotensive group (P < .05, P < .05, respectively). Also, MPV levels were significantly higher in the non-dipper and dipper groups than in normotensive group (P < .05, P < .05, respectively). Logistic regression analysis showed significant association between non-dipper status and MPV with platelet count (P < .05, P < .05, respectively). The abilities of MPV and platelet count to predict the non-dipper status were determined by receiver operating characteristic curve analysis (areas under the curve were 0.709 and 0.604, respectively). CONCLUSIONS: The higher MPV and neutrophil count may be potential indicators of increased risk for the development of hypertension in children. In addition, MPV and platelet count may help to determine the presence of non-dipper status in children with hypertension.

Bone mineral density and vitamin D status in children with remission phase of steroid-sensitive nephrotic syndrome.

Children with idiopathic nephrotic syndrome are primarily treated with glucocorticoids (GCs), but long-term GC use can lead to undesired side effects. We investigated the bone mineral density (BMD) and 25-hydroxyvitamin D (25-OH D) levels in children with the remission phase of steroid-sensitive nephrotic syndrome (SSNS). This study included 32 patients with SSNS who had not received GC treatment in the last 6 months and a control group of 20 healthy children. Serum levels of calcium, phosphate, alkaline phosphatase, 25-(OH)D, and parathyroid hormone (PTH) were measured. BMD was determined in the lumbar spinal region using dual-energy X-ray absorptiometry (DEXA). Serum 25-(OH)D levels were lower in the SSNS patients than in the healthy children (P <0.05), with 22 patients (68.8%) having Z-scores <-1. The Z-scores were positively correlated with 25-(OH)D levels (r = 0.424, P <0.05). PTH levels were higher in patients with osteoporosis than in patients with Z-scores ≥-1 (P <0.05). Bone mineral content and BMD were positively correlated with the age of diagnosis (P <0.01). Receiver-operating characteristic curve analysis showed that the cutoff value of 25-(OH)D levels for predicting low BMD was 14.67 ng/mL with a sensitivity of 90% and a specificity of 64%. The area under the curve (AUC ± standard error) was 0.868 ± 0.064 (95% confidence interval: 0.742-0.994, P = 0.001). Decreased 25-(OH)D levels and the negative effects of long-term GC treatment on BMD persist in SSNS remission phase. Levels of 25-(OH)D <14.67 ng/mL could predict abnormal DEXA scans in children with SSNS remission phase.