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.

A broad clinical spectrum of PLCε1-related kidney disease and intrafamilial variability.

BACKGROUND: The phenotypic and genotypic spectrum and kidney outcome of PLCε1-related kidney disease are not well known. We attempted to study 25 genetically confirmed cases of PLCε1-related kidney disease from 11 centers to expand the clinical spectrum and to determine the relationship between phenotypic and genotypic features, kidney outcome, and the impact of treatment on outcome. METHODS: Data regarding demographics, clinical and laboratory characteristics, histopathological and genetic test results, and treatments were evaluated retrospectively. RESULTS: Of 25 patients, 36% presented with isolated proteinuria, 28% with nephrotic syndrome, and 36% with chronic kidney disease stage 5. Twenty patients underwent kidney biopsy, 13 (65%) showed focal segmental glomerulosclerosis (FSGS), and 7 (35%) showed diffuse mesangial sclerosis (DMS). Of the mutations identified, 80% had non-missense, and 20% had missense; ten were novel. No clear genotype-phenotype correlation was observed; however, significant intrafamilial variations were observed in three families. Patients with isolated proteinuria had significantly better kidney survival than patients with nephrotic syndrome at onset (p = 0.0004). Patients with FSGS had significantly better kidney survival than patients with DMS (p = 0.007). Patients who presented with nephrotic syndrome did not respond to any immunosuppressive therapy; however, 4/9 children who presented with isolated proteinuria showed a decrease in proteinuria with steroids and/or calcineurin inhibitors. CONCLUSION: PLCε1-related kidney disease may occur in a wide clinical spectrum, and genetic variations are not associated with clinical presentation or disease course. However, clinical presentation and histopathology appear to be important determinants for prognosis. Immunosuppressive medications in addition to angiotensin-converting enzyme inhibitors may be beneficial for selected patients. "A higher resolution version of the Graphical abstract is available as Supplementary information".

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.

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.