Retrospective analysis of clinical and pathomorphological features of lupus nephritis in children.

PURPOSE: The aim of the study was to evaluate the clinical course and pathomorphological correlations in Polish children with the diagnosis of lupus nephritis (LN). METHODS: We retrospectively analyzed the medical records of 39 children hospitalized due to LN in 7 pediatric nephrology units in Poland between 2010 and 2019. Demographic data, clinical symptoms at the onset of LN and laboratory parameters were reviewed. We analyzed Systemic Lupus Erythematosus Disease Activity Index (SLEDAI), histological LN findings with the activity (IA) and chronicity index (IC). RESULTS: We examined 32 girls and 7 boys, median age at LN onset was 14.75 (IQR 13.0-16.0) years, SLEDAI of 22.0 (IQR 18.0-27.0) points; LN histological class: IV (59.4%), III (18.9%), III/V (10.8%), IV/V (8.1%), VI (2.7%); IA 8.0 (IQR 6.0-11.0) points, IC 1.05 (IQR 0-2.0) points. Children with nephrotic (n â€‹= â€‹22) and non-nephrotic (n â€‹= â€‹17) proteinuria differed in median Hb level (9.55, IQR 8.3-11.2 vs 10.9, IQR 10.1-11.6 â€‹g/L; P â€‹< â€‹0.05), albumin level (2.5, IQR 2.1-3.19 vs 3.6, IQR 3.4-4.1 â€‹g/dL; P â€‹< â€‹0.001), proteinuria (5.76, IQR 3.0-7.5 vs 1.08, IQR 0.53-1.50 â€‹g/day; P â€‹< â€‹0.0001), eGFR (53.9, IQR 27.0-68.8 vs 96.7, IQR 73.8-106.2 â€‹mL/min/1.73 â€‹m2; P â€‹< â€‹0.01) and occurrence of hypertension (77% vs 23%; P â€‹< â€‹0.01). In multivariate analysis Hb level (ߠ​= â€‹8.0; 95%CI, 1.90-14.11) was the significant predictor of eGFR<90 â€‹mL/min/1.73 â€‹m2. CONCLUSIONS: Proliferative forms of LN in children may have a varying clinical presentation. Children with LN with nephrotic range proteinuria have lower Hb level, lower eGFR and higher occurrence of hypertension. Hb level is the significant predictor of eGFR<90 â€‹mL/min/1.73 â€‹m2 in children with LN.

The relationship between jumping to conclusions and neuropsychological functioning in schizophrenia.

Jumping to conclusions (JTC) is defined as a tendency to make decisions based on insufficient information. JTC has been reported in patients with psychosis, but the mechanisms of this cognitive bias remain unknown. The main aim of our study was to investigate the relationship between JTC and neuropsychological functioning in schizophrenia. A total of 85 schizophrenia patients were assessed with neuropsychological tests, including executive functions, verbal memory, working memory, processing speed and attention. JTC was assessed with the Fish Task (probability 80:20 and 60:40) and a self-report scale (The Davos Assessment of Cognitive Biases Scale, DACOBS). Symptom severity was assessed with the Positive and Negative Syndrome Scale (PANSS). The relationship between JTC and neuropsychological functioning was investigated with correlation and regression analyses. The regression analyses model, when controlling for duration of illness, age and symptoms, showed that verbal memory and working memory were specifically related to JTC measured by Fish Task 60:40. JTC measured using Fish Task 60:40 was correlated only with severity of symptoms of disorganization (PANSS). The results from the present study suggest that the relationship between decision making during the reasoning task and neuropsychological functioning is modulated by task demands.

Endothelin-1 (ET-1), N-terminal fragment of pro-atrial natriuretic peptide (NTpro-ANP), and tumour necrosis factor alpha (TNF-α) in children with primary hypertension and hypertension of renal origin.

INTRODUCTION: Hypertension is regarded as a condition of mild inflammation and endothelial imbalance. The aim of the study was to evaluate serum concentrations of biomarkers of inflammation and endothelial function: tumour necrosis factor alpha (TNF-α), endothelin-1 (ET-1), and N-terminal fragment of pro-atrial natriuretic peptide (NTpro-ANP) in hypertensive and normotensive children. MATERIAL AND METHODS: We studied 63 children aged 13.56 ± 3.73 years, divided into two groups: a group with primary hypertension (n = 50) and a group with renal hypertension (n = 13). The control group consisted of 34 normotensive children aged 12.76 ± 3.96 years. Biomarkers were measured with ELISA tests. RESULTS: ET-1 levels were significantly higher in primary hypertension (9.93 ± 1.73 pg/ml) and renal hypertension (10.77 ± 1.50 pg/ml) in comparison to controls (4.03 ± 0.97 pg/ml), (p < 0.001, p < 0.001, respectively). NT-pro ANP concentrations in primary hypertension (71.03 ± 10.02 pg/ml), and renal hypertension (84.78 ± 6.44 pg/ml) were significantly higher than in the control group (29.62 ± 5.56 pg/ml) (p < 0.001, p < 0.001, respectively). TNF-α concentrations in primary hypertension (8.36 ± 1.60 pg/ml) and renal hypertension (7.35 ± 0.93 pg/ml) significantly exceeded concentrations in controls (4.49 ± 0.93 pg/ml), (p < 0.001, p < 0.001, respectively). ET-1 and NT-pro ANP concentrations in renal hypertension significantly exceeded those in primary hypertension (p = 0.049, p < 0.001, respectively) while TNF-α levels in renal hypertension were significantly lower than in primary hypertension (p = 0.046). CONCLUSIONS: The results of our study show that ET-1, NT-pro ANP, and TNF-a concentrations are increased in hypertension in children. Our investigation indicates significant importance of inflammation and endothelial involvement in hypertension in youth.

Non-dipping status and selected adipokines concentration in children with primary arterial hypertension.

High blood pressure (BP) in children and adolescents is associated with increased risk of persistent hypertension (HT) in adulthood and, consequently, cardiovascular disease and target organ damage. AIM: The values of selected anthropometric parameters and laboratory test results including leptin and apelin concentration were evaluated with regard to averaged values of office BP measurements and 24-hour ABPM. MATERIAL AND METHODS: The study included 55 children: 23-with primary HT and coexisting overweight or obese (HT-OB), 16-with primary HT and normal weight (HT-NW), and 16 healthy children (C). RESULTS: The concentration of leptin and apelin in HT-OB children was significantly higher than in the C and HT-NW group. A similar finding for leptin level was demonstrated in comparison of HT-NW with C group. In children with HT-OB, the lack of decline in nocturnal DBP was associated with significantly higher BMI and the Cole's Index value. Children with HT-OB and lack of decline in nocturnal SBP showed lower leptin and insulin concentrations, and lower values of HOMA-IR as compared with dipping children. Positive correlation in the whole study group was found between adipokines levels and BP measurements. In conclusion, children with primary HT show higher concentration of adipokines compared with their healthy peers. The data on relationship of serum adipokines levels with anthropometric and BP parameters in children may be helpful to clarify the sequence of disturbances in the cardiovascular system in adults, which requires further examination.

The increasing incidence of initial steroid resistance in childhood nephrotic syndrome.

BACKGROUND: Recently, a number of reports have highlighted changes in the histopathology and response to corticosteroid treatment in childhood nephrotic syndrome; however, these involved ethnically mixed populations. For comparison, the purpose of our research was to search for changes in the characteristics of nephrotic syndrome in a homogeneous population of Caucasian children over two consecutive decades. METHODS: Chart analysis was performed to identify children with new-onset nephrotic syndrome. The children were admitted to the Division of Pediatric Nephrology, Zabrze, during two periods: 1986-1995 (76 patients) and 1996-2005 (102 patients). Specifically, a comparison of clinical characteristics and morphology of nephrotic syndrome between the two groups was performed. Steroid resistance was defined as no remission within 8 weeks of corticosteroid treatment. Histopathology was available in 36.8% and 43.1% of patients respectively. RESULTS: There was a significant increase in primary steroid resistance in the latter decade: 15.8% vs 31.4% (P = 0.017). Changes in the histopathology did not reach the level of statistical significance: minimal change nephrotic syndrome 25% vs 9% (P = 0.095), mesangial proliferative glomerulonephritis 46.4% vs 61.3% (P = 0.21), focal segmental glomerulosclerosis 17.9% vs 20.4% (P = 0.78), membranoproliferative glomerulonephritis 7.1% vs 6.8% (P = 1.0), membranous glomerulonephritis 3.6% vs 0% (P = 0.39). CONCLUSIONS: Our results show the increasing incidence of primary steroid resistance in childhood nephrotic syndrome.

Percutaneous endoscopic gastrostomy as a method of nutrition support in children with chronic kidney disease.

Chronic kidney disease (CKD) exerts a significant impact on children's growth and development. In infants and children with CKD, feeding-related problems are not infrequent, leading to protein-energy malnutrition and resulting in retarded physical development. Because spontaneous energy intake is usually inadequate in children with CKD, special dietary counseling with supportive procedures are often required. Placement of a percutaneous endoscopic gastrostomy (PEG) tube is one practical way to allow home tube feeding. Such nutrition support is valuable in children with CKD, and although it is not free from complications and technical problems, it seems to be helpful in providing required nutrition intake. In this report, the authors describe their own experience with PEG implantation and use in 3 children with CKD.

Bone metabolism and the muscle-bone relationship in children, adolescents and young adults with phenylketonuria.

The aim of the study was to assess body composition in subjects with phenylketonuria (PKU). Forty-five patients aged 13.8 ± 5.2 years were evaluated. Among them, 15 patients had not reached sexual maturity, showing normal serum values of phenylalanine (Phe) (subgroup 1), and 30 subjects were sexually mature (Tanner 5 stage), showing either normal serum Phe (18 cases; subgroup 2a) or increased serum Phe (12 cases; subgroup 2b). DXA-assessed spine and total body (TB) measurements [bone mineral density (BMD), bone mineral content (BMC), lean body mass (LBM) and the calculated ratios BMC/LBM] as well as laboratory parameters (serum carboxyterminal telopeptide of type I collagen, bone alkaline phosphatase, osteocalcin, parathormone, calcitonin, total and ionized calcium) were analyzed. Statistically significant differences were revealed between subgroup 1 versus 2a for TB BMC/LBM ratio SD scores and between subgroup 2a versus 2b for TB BMD, spine BMD, TB BMC/LBM ratio and spine BMC/LBM ratio SD scores. Stepwise multiple regression analysis revealed that serum Phe negatively affected bone status. The skeletal status in children with PKU is impaired by the disease. Applying body composition parameters instead of BMD alone may reflect the level of impairment in a new, different way.

Utility of the dobutamine stress echocardiography in the evaluation of the effects of a surgical repair of aortic coarctation in children.

BACKGROUND: Exercise-induced hypertension following repair of the coarctation of the aorta (CoA) is a well known phenomenon. The most important functional parameters in the assessment of the effects of a surgical repair of CoA are the maximal pressure gradient in the descending aorta (GRAD) and systolic blood pressure (SBP). Results of treadmill exercise test using the Bruce protocol (treadmill test) and dobutamine stress echocardiography (DSE) were compared to determine utility of the DSE in the evaluation of the effects of surgical treatment of CoA in children. METHODS: The study population comprised of 29 patients, including 20 males and 9 females (mean age 12 years) who underwent a surgical repair of CoA. Changes of the cardiovascular parameters including SBP, GRAD and heart rate (HR) during the treadmill test and DSE were compared. RESULTS: During the treadmill test, SBP at peak exercise ranged from 120 to 230 (mean 163.7) mm Hg, GRAD ranged from 29 to 109 (mean 59.8) mm Hg, and HR ranged from 140 to 188 (mean 169) bpm. At the end of DSE, SBP ranged from 123 to 215 (mean 164.7) mm Hg, GRAD ranged from 29 to 113 (mean 55.4) mm Hg, and HR ranged from 76 to 155 (mean 111) bpm. We found positive correlations of SBP (r = 0.68, p < 0.001) and GRAD (r = 0.82, p < 0.001) values during both tests but no significant correlation for HR (r = 0.42, p = NS). CONCLUSIONS: Dobutamine stress echocardiography is useful in the evaluation of the effects of surgical repair of CoA in children.

[Diagnostic value and safety of percutaneous kidney biopsy in experience of one clinical center]. / Wartosc diagnostyczna i bezpieczenstwo przeskórnej biopsji nerki w doswiadczeniach jednegos osrodka.

UNLABELLED: Percutaneous kidney biopsy is a widely applied diagnostic procedure providing with kidney tissue for microscopy. AIM OF THE STUDY: The assessment of diagnostic value of kidney tissue specimens gained by percutaneous biopsy and the assessment of that method's safety. MATERIAL: 119 percutaneous kidney biopsies performed on patients of Pediatric Clinic in Zabrze from 1998 through 2007 were analysed. RESULTS: Histopathological diagnosis was assessed in 107 cases (90%). The other 12 specimens (10%) were described by the pathomorphologist as non-diagnostic. In analysed group of patients serious complications were noted in 5 cases (4.2%): massive haematuria (in 2 cases), massive bleeding in perirenal space (in 3 cases including 1 resulting in nephrectomy). CONCLUSIONS: Percutaneous kidney biopsy is a valuable diagnostic procedure. Because of possible complications it is very important to comply with indications and contraindications established for that procedure.