ENWEndNote BIBJabRef, Mendeley RISPapers, Reference Manager, RefWorks, Zotero AMA Leszczyńska B, Deja A, Daniel M, et al. Twenty-five years of growth hormone treatment in non-dialyzed children with chronic kidney disease in Poland – efficacy, safety, metabolic effects, and factors influencing response. Pediatria Polska - Polish Journal of Paediatrics. 2022. doi:10.5114/polp.2022.120131. APA Leszczyńska, B., Deja, A., Daniel, M., Majcher, A., Skrzypczyk, P., & Turczyn, A. et al. (2022). Twenty-five years of growth hormone treatment in non-dialyzed children with chronic kidney disease in Poland – efficacy, safety, metabolic effects, and factors influencing response. Pediatria Polska - Polish Journal of Paediatrics. https://doi.org/10.5114/polp.2022.120131 Chicago Leszczyńska, Beata, Anna Deja, Maria Daniel, Anna Majcher, Piotr Skrzypczyk, Agnieszka Turczyn, and Joanna Groszek et al. 2022. "Twenty-five years of growth hormone treatment in non-dialyzed children with chronic kidney disease in Poland – efficacy, safety, metabolic effects, and factors influencing response". Pediatria Polska - Polish Journal of Paediatrics. doi:10.5114/polp.2022.120131. Harvard Leszczyńska, B., Deja, A., Daniel, M., Majcher, A., Skrzypczyk, P., Turczyn, A., Groszek, J., Antonowicz-Zawiślak, A., and Pańczyk-Tomaszewska, M. (2022). Twenty-five years of growth hormone treatment in non-dialyzed children with chronic kidney disease in Poland – efficacy, safety, metabolic effects, and factors influencing response. Pediatria Polska - Polish Journal of Paediatrics. https://doi.org/10.5114/polp.2022.120131 MLA Leszczyńska, Beata et al. "Twenty-five years of growth hormone treatment in non-dialyzed children with chronic kidney disease in Poland – efficacy, safety, metabolic effects, and factors influencing response." Pediatria Polska - Polish Journal of Paediatrics, 2022. doi:10.5114/polp.2022.120131. Vancouver Leszczyńska B, Deja A, Daniel M, Majcher A, Skrzypczyk P, Turczyn A et al. Twenty-five years of growth hormone treatment in non-dialyzed children with chronic kidney disease in Poland – efficacy, safety, metabolic effects, and factors influencing response. Pediatria Polska - Polish Journal of Paediatrics. 2022. doi:10.5114/polp.2022.120131.
Our aim was to assess common carotid artery intima-media thickness (cIMT) in children with idiopathic nephrotic syndrome (INS) and to find relation between cIMT and clinical and biochemical parameters in these patients.In 50 children with INS we retrospectively evaluated: cIMT ((mm) and Z-score) and selected clinical and biochemical parameters. The control group consisted of 20 healthy children aged 9.46 ± 2.29 years.Children with INS had higher cIMT (0.45 ± 0.05 vs. 0.40 ± 0.05 (mm), p = 0.0002) and cIMT Z-score (1.72 ± 1.01 vs. 0.43 ± 1.01, p < 0.0001) than the control group. In the INS group, children with arterial hypertension had significantly higher cIMT (p = 0.0148) than normotensive children. In 50 children, with INS we found correlations between cIMT and disease duration (r = 0.40, p = 0.0040), number of INS relapses (r = 0.51, p< 0.0001), cumulative prednisone dose (r = 0.45, p = 0.0010), and BMI (r = 0.35, p = 0.0120); whereas, cIMT Z-score correlated only with the number of INS relapses (r = 0.41, p = 0.0160) and cumulative prednisone dose (r = 0.36, p = 0.0362). We found no relation between cIMT and response to corticosteroids, treatment used, and biochemical parameters.1. Idiopathic nephrotic syndrome predisposes to atherosclerotic lesions in affected children. 2. The severity of atherosclerotic lesions is dependent mainly on the number of INS relapses, but disease vintage, cumulative steroid dose, body mass index, and presence of arterial hypertension may also be predisposing factors. .
Studies in adults have demonstrated the essential role of microRNAs in developing hypertension and their effect on hypertension sequelae. In this preliminary study, we aimed to investigate the expression of five miRNA particles, miRNA-21, miRNA-27a, miRNA-27b, miRNA-133a, and miRNA-145, in school-aged children with primary hypertension and to examine their correlations with blood pressure and arterial and heart properties.
Objective: Paragangliomas and pheochromocytoma (PPGL) are rare causes of arterial hypertension (AH) in children. This study aimed to present the clinical course in two adolescents with PPGL tumors due to succinate dehydrogenase mutations. Design and method: We analyzed age, gender, clinical manifestations, laboratory findings, including plasma methoxycatecholamines, genetic and imaging findings, pharmacological and surgical treatment, and outcome. Results: Case 1: A 14-year-old boy presented with tinnitus, dizziness, and balance disorders. A computed tomography (CT) revealed a paraganglioma in the area of the left internal jugular vein bulb, causing ear and bone destruction. The boy was diagnosed with AH; his plasma normetanephrine was 343pg/mL (normal range: 18-138), metanephrine and 3-methoxytramine were normal. The patient was started on doxazosin, and the tumor was removed. A genetic study showed a pathogenic variant in the SDHD gene (c.33C>A). PET/CT showed another tumor between the inferior vena cava and diaphragm. A successful tumor resection was performed. Currently, the boy is 17 years old, methoxycatecholamine levels are normal. The patient has AH (primary hypertension) and is treated with rampiril and amlodipine. Case 2: A 17.5-year-old girl was admitted to the hospital due to headaches and AH lasting about six months. Laboratory tests revealed a markedly elevated plasma normetanephrine - 8734.59pg/mL (normal range: 16-142) and a slightly elevated 3-methoxytramine - 25.52pg/mL (normal range < 14). CT showed a large tumor (11x7x7cm) at the level of the diaphragm, modeling the heart, liver, and inferior vena cava. Whole-body PET/CT and MIBG scintigraphy did not detect other tumors. A genetic test showed a pathogenic variant in the SDHB gene (c.181dupT). The girl was treated with doxazosin. Surgical treatment included two transarterial embolizations, followed by surgical removal of the tumor. Currently, the patient is 18 years and 2 months old, has normal methoxycatecholamines, and does not require antihypertensive drugs. The same variant of the SDHB gene was found in the patient's father and older brother. Conclusions: 1.PPGL can have an unusual clinical course in children, and patients may present with a variety of symptoms 2.Pediatric patients with PPGL require genetic tests and individualized, multidisciplinary medical care.
Acute kidney injury (AKI) is a common consequence of perinatal asphyxia reported in 30 to 70% cases. We present 4 full-term neonates with oliguric/anuric AKI caused by perinatal asphyxia requiring renal replacement therapy (RRT) and their long-term outcomes. Patient No. 1 was dialyzed for 12 days (continuous ambulatory peritoneal dialysis (CAPD)/continuous venovenous hemodiafiltration (CWHDF)), then was treated conservatively, and received pre-emptive kidney transplantation (KTx) at the age of 3 3/12 years. Patient No. 2 was treated with CAPD/automated peritoneal dialysis (APD) for 15 months, due to recovery of renal function, dialysis was withdrawn. He is now 8 5/12 years old and has chronic kidney disease (CKD) stage III. Patient No. 3 after 5 days of continuous arteriovenous hemofiltration (CAVH) required CAPD for 17 days. The child is now 8 4/12 years old and has CKD stage III. Patient No. 4, dialyzed from 3rd day of life for 51 months (CAVH followed by CAPD/APD), was given cadaver KTx at the age of 4 3/12 years. Psychomotor development is good in 2 patients, whereas patients No. 3 and 4 have tetraplegic spastic infantile cerebral palsy, severe mental retardation, and epilepsy.Severe perinatal asphyxia with oliguric/anuric AKI is a risk factor for chronic kidney disease sometimes end-stage renal disease.
Acute poststreptococcal glomerulonephritis (APSGN) is a complication of infection with group A beta-hemolytic streptococcus. The disease manifests as microscopic/gross hematuria, arterial hypertension, edema, and acute kidney injury and has most commonly self-limiting course.The aim of study was the analysis of clinical course of APSGN in period of increased incidence in the first half of 2018.We analyzed following parameters in children hospitalized due to APSGN in January-June 2018: age, sex, anthropometric parameters, preceding infection, clinical signs, renal function, biochemical and immunological tests (including antristreptolysins (ASO) and complement), urinalysis, renal ultrasonography, and treatment. The incidence of APSGN in years 2007-2018 was analyzed.We found 11 children (6 boys, 5 girls) aged 5.01±2.44 years. The disease was preceded by pharyngitis in 8, skin infection in 1 with latent period 16.40±5.77 days. Clinical symptoms were: gross hematuria in 8, edema in 6, hypertension in 5, renal function impairment 6, and hyperkalemia in 5; all patients had lowered C3 complement factor; ASO was elevated in all patients except for a boy with skin infection. During hospitalization clinical symptoms resolved in all children; significant elevation in GFR (p=0.018) and C3 (p=0.034), and decrease in proteinuria (p=0.039) were observed. Four patients with abnormal ultrasonographic kidney image were characterized by worse kidney function (p=0.018), higher potassium concentration (p=0.052), higher proteinuria (p=0.073) and erythrocyturia (p=0.015) than remaining children. In follow-up (after 142,00±89,20days) all children had normal renal function and blood pressure, 1 patient had proteinuria, and 4 had erythrocyturia.In most cases APSGN is characterized by rapid resolution of symptoms and good prognosis, but patients require periodic follow-up visits. Abnormal initial ultrasonographic kidney image may be a marker of worse clinical course of APSGN.
INTRODUCTION AND AIMS: Inherited Nephrogenic diabetes insipidus (NDI) is a rare disorder characterized by impaired urinary concentrating ability with consequent polyuria and risk of dehydration.There is paucity of clinical data on long-term outcome to help inform prognosis and management.METHODS: We performed a single center retrospective medical record review of patients with diagnosis of NDI patients followed between 1985 and 2017.We collected available data on growth, weight, school performance, complications and comorbidities.RESULTS: We identified 36 patients with available data and a clinical diagnosis of NDI, which was genetically confirmed in 33.Patients presented at median age of 0.6 years (range 0.01-9) and median length of follow-up was 9.5 years (0.8-16.9).The chief symptoms at presentation were failure to thrive, vomiting /feeding concerns, polyuria / polydipsia, febrile illness and hypernatraemic dehydration.Four were investigated before symptoms based on family history.Median weight standard deviation scores (SDS) improved from-2.1 at presentation to 0.2 at last follow-up.In contrast height SDS remained essentially unchanged at -1.1 at presentation and -0.9 at last follow-up.Most patients were treated with NSAID and thiazides, yet weaned off during school age without apparent change in urine output.No apparent long-term toxicity was noted.Median estimated glomerular filtration rate (eGFR) at last follow up was 81 (56-129) ml/min/1.73m 2 .Urological complications were noted in fifteen patients.Constipation was recorded in eleven and learning difficulty in five patients.Median age of resolution of nocturnal enuresis was 11.3 (range: 4-16, N¼19) years.Estimated median daily fluid intake at median age of 13y (N¼28) was 3800 ml /m 2 (1600-9200).CONCLUSIONS: The overall prognosis in inherited NDI is favorable with regular treatment.Thiazide and NSIAD treatment appears to lose efficacy with age.As expected, most complications were related to polyuria.Our data inform the prognosis and management of patients with NDI.
Background: Elevated blood pressure and proteinuria are well-established risk factors for chronic kidney disease (CKD) progression in children. This study aimed to analyze risk factors for CKD progress, emphasizing detailed ambulatory blood pressure (ABPM) data. Methods: In 55 children with CKD II−V, observed for ≥1 year or until initiation of kidney replacement therapy, we analyzed ABPM, clinical, and biochemical parameters. Results: At the beginning, the glomerular filtration rate (eGFR) was 66 (interquartile range—IQR: 42.8−75.3) mL/min/1.73 m2, and the observation period was 27 (16−36) months. The mean eGFR decline was 2.9 ± 5.7 mL/min/1.73 m2/year. eGFR decline correlated (p < 0.05) with age (r = 0.30), initial proteinuria (r = 0.31), nighttime systolic and mean blood pressure (r = 0.27, r = 0.29), and systolic and diastolic blood pressure dipping (r = −0.37, r = −0.29). There was no relation between mean arterial pressure during 24 h (MAP 24 h Z-score) and eGFR decline and no difference in eGFR decline between those with MAP 24 h < and ≥50 th percentile. In multivariate analysis, systolic blood pressure dipping (beta = −0.43), presence of proteinuria (beta = −0.35), and age (beta = 0.25) were predictors of eGFR decline. Conclusions: Systolic blood pressure dipping may be a valuable indicator of CKD progression in children.
ENWEndNote BIBJabRef, Mendeley RISPapers, Reference Manager, RefWorks, Zotero AMA Szymanik-Grzelak H, Kuźma-Mroczkowska E, Skrzypczyk P, Bielecka T, Kotula I, Pańczyk-Tomaszewska M. Tuberculosis infection in children with proteinuria/nephrotic syndrome. Central European Journal of Immunology. 2017;42(3):318-323. doi:10.5114/ceji.2017.70977. APA Szymanik-Grzelak, H., Kuźma-Mroczkowska, E., Skrzypczyk, P., Bielecka, T., Kotula, I., & Pańczyk-Tomaszewska, M. (2017). Tuberculosis infection in children with proteinuria/nephrotic syndrome. Central European Journal of Immunology, 42(3), 318-323. https://doi.org/10.5114/ceji.2017.70977 Chicago Szymanik-Grzelak, Hanna, Elżbieta Kuźma-Mroczkowska, Piotr Skrzypczyk, Teresa Bielecka, Iwona Kotula, and Małgorzata Pańczyk-Tomaszewska. 2017. "Tuberculosis infection in children with proteinuria/nephrotic syndrome". Central European Journal of Immunology 42 (3): 318-323. doi:10.5114/ceji.2017.70977. Harvard Szymanik-Grzelak, H., Kuźma-Mroczkowska, E., Skrzypczyk, P., Bielecka, T., Kotula, I., and Pańczyk-Tomaszewska, M. (2017). Tuberculosis infection in children with proteinuria/nephrotic syndrome. Central European Journal of Immunology, 42(3), pp.318-323. https://doi.org/10.5114/ceji.2017.70977 MLA Szymanik-Grzelak, Hanna et al. "Tuberculosis infection in children with proteinuria/nephrotic syndrome." Central European Journal of Immunology, vol. 42, no. 3, 2017, pp. 318-323. doi:10.5114/ceji.2017.70977. Vancouver Szymanik-Grzelak H, Kuźma-Mroczkowska E, Skrzypczyk P, Bielecka T, Kotula I, Pańczyk-Tomaszewska M. Tuberculosis infection in children with proteinuria/nephrotic syndrome. Central European Journal of Immunology. 2017;42(3):318-323. doi:10.5114/ceji.2017.70977.
Introduction and objective: Evaluation of subclinical inflammation in patients with primary hypertension (PH) and white coat hypertension (WCH). Materials and methods: In 56 untreated paediatric patients with PH, 40 with WCH, and 30 healthy individuals (control group, CG), we evaluated high sensitivity C-reactive protein (hsCRP), interleukin 18 (IL-18) levels, complete blood count-derived markers of inflammation, office and ambulatory blood pressure, and selected clinical and biochemical parameters. Results: hsCRP was significantly higher in PH patients compared to CG, and neutrophil and monocyte counts were significantly higher in PH and WCH patients compared to CG. Receiver operating characteristic analysis revealed good prognostic profiles for hsCRP, neutrophil, lymphocyte, monocyte, and platelet counts, as well as neutrophil-to-lymphocyte ratio (NLR), monocyte-to-neutrophil ratio, and platelet-to-mean platelet volume ratio as predictors of the presence of PH. In multivariate analysis, monocyte-to-lymphocyte ratio (MLR) and platelet count (β = 0.217, β = 0.191) were significant predictors of office diastolic blood pressure Z-score, while neutrophil count predicted 24 h systolic blood pressure Z-score (β = 0.365), MLR, lymphocyte count, IL-18, and NLR predicted 24 h diastolic blood pressure Z-score (β = 0.305, β = 0.253, β = −0.197, β = −0.189), and neutrophil count together with IL-18 predicted 24 h mean arterial pressure Z-score (β = 0.210, β = −0.209). Conclusions: 1. Patients with PH and WCH are characterised by similar levels of subclinical inflammation, which are significantly higher compared to healthy peers. 2. Complete blood count-derived indices, especially neutrophil count and MLR, can serve as important adjuncts to the clinical evaluation of paediatric patients with PH.
