Assessment of Interleukin-15 (IL-15) Concentration in Children with Idiopathic Nephrotic Syndrome.

Idiopathic nephrotic syndrome (INS) is a chronic glomerular disease in children, characterized by severe proteinuria, hypoalbuminemia, and/or presence of edema and hyperlipidemia. The pathogenesis, however, has not been yet established. The clinical course of the disease is characterized by frequent relapses. Interleukin-15 (IL-15) is a pro-inflammatory cytokine, that apart from its involvement in the immune system, was found to be playing a vital role in various cells' functioning, including renal tissue. It is desirable to look for new predictors of INS. Our study aimed to evaluate IL-15 as a potential marker in the early diagnosis of the disease. The cohort participating in the study consisted of patients hospitalized in Clinical Hospital No. 1 in Zabrze, from December 2019 to December 2021, including study group with INS (n = 30) and control group (n = 44). Results: The concentration of IL-15 in both serum and urine was significantly elevated in patients with INS, compared to healthy controls. The cytokine might serve as a marker of the disease, however, further research on larger study groups is needed.

Assessment of Brain-Derived Neurotrophic Factor (BDNF) Concentration in Children with Idiopathic Nephrotic Syndrome.

Idiopathic nephrotic syndrome (INS) is a chronic disease affecting children in early childhood. It is characterized by proteinuria, hypoalbuminemia, edema and hyperlipidemia. To date, the diagnosis is usually established at an advanced stage of proteinuria. Therefore, new methods of early INS detection are desired. This study was designed to assess brain-derived neurotrophic factor (BDNF) as a potential marker in the early diagnosis of INS. The study group included patients with a diagnosis of idiopathic nephrotic syndrome (n = 30) hospitalized in Clinical Hospital No. 1 in Zabrze, from December 2019 to December 2021. Our study shows that serum BDNF concentration decreased and urine BDNF concentration increased in a group of patients with INS, compared with healthy controls. Such outcomes might be related to loss of the BDNF contribution in podocyte structure maintenance. Moreover, we anticipate the role of BDNF in urine protein concentration increase, which could be used as a direct predictor of urine protein fluctuations in clinical practice. Moreover, the ROC curve has also shown that serum BDNF and urine BDNF levels might be useful as an INS marker.

Atypical Hemolytic Uremic Syndrome (aHUS) and Adenosine Deaminase (ADA)-Deficient Severe Combined Immunodeficiency (SCID)-Two Diseases That Exacerbate Each Other: Case Report.

Hemolytic uremic syndrome (HUS) is defined by the triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury (AKI). Atypical HUS (aHUS), distinguished by its etiology, is caused by uncontrolled overactivation of the alternative complement pathway. The correct diagnosis of aHUS is complex and involves various gene mutations. Severe combined immunodeficiency (SCID), characterized by severe T-cell lymphocytopenia and a lack of antigen-specific T-cell and B-cell immune responses, is of seldom occurrence. In 10-15% of pediatric patients, SCID is caused by adenosine deaminase (ADA) deficiency. The authors describe the case of a boy who suffered from both aHUS and ADA-deficient SCID. At the age of 9 months, the patient presented acute kidney injury with anuria and coagulopathy. The diagnosis of aHUS was established on the basis of alternative complement pathway deregulation and disease-associated gene mutations. Further examination revealed immune system failure and, at the age of 13 months, the ADA deficiency was confirmed by genetic tests and the boy was diagnosed with ADA-SCID. ADA SCID has recently been described as a possible triggering factor of aHUS development and progression. However, more research is required in this field. Nevertheless, it is crucial in clinical practice to be aware of these two co-existing life-threatening diseases.

A rare complication of systemic lupus erythematosus in a 9-year-old girl: Questions.

Serious renal involvement in systemic diseases is common and generally constitutes a pivotal prognostic factor, making those pathology frequently seen in nephrology departments. Authors describe the case of a nine-year-old girl with lupus nephritis. After admission the patient's state deteriorated over a period of a few days, with an unremitting high-grade fever, significant weakness and drowsiness, generalized erythema, and decrease of the kidney function to eGFR nadir of 56 ml/min/1,73m 2. Treatment with pulsed methylprednisolone was started. After the first pulse the general state of the patient improved slightly, although laboratory tests showed an alarming evolution, with the exacerbation of anemia, leukopenia, neutropenia, increase of serum CRP concentration, extremely high D-dimer concentration and increase in activity of lactate dehydrogenase. The concentration of ferritin rose reaching the level of 540 µg/l, triglicerydes level was also high. Intravenous cyclophosphamide pulse therapy was added to the ongoing steroid treatment, and resulted in a radical patient improvement. Authors underline that it seems important to be aware of rare, non-renal, but potentially devastating complications of systemic diseases, like in this clinical case: the secondary hemophagocytic lymphohistiocytosis (HLH). When HLH complicates a rheumatic disease, it is also referred to as macrophage activation syndrome (MAS). Unfortunately treatment of MAS is still based on reports provided by individual centres and gathered own experiences so drawing up unambiguous diagnostic criteria will be valuable in future. The treatment should be individually tailored, and more specific evidence-based recommendations are needed.

Evaluation of liver-type fatty acid binding protein (L-FABP) and interleukin 6 in children with renal cysts.

BACKGROUND: Renal cysts, according to their etiology, can be divided into genetic and acquired cysts. This is of great importance in patients with cystic kidney disease with a possible poor prognosis to identify markers of early kidney damage. OBJECTIVES: The objective of this study was to evaluate the concentration of serum and urine liver-type fatty acid binding protein (L-FABP) and interleukin 6 (IL-6) in children with kidney cysts. MATERIAL AND METHODS: The study was conducted on a group of 39 children with kidney cysts including 20 subjects with autosomal dominant polycystic kidney disease (ADPKD). RESULTS: Serum and urine L-FABP concentration in children with renal cysts was significantly higher compared to the controls, regardless of the underlying type of cystic degeneration, number of cysts and gender. Also, serum and urinary IL-6 concentration was significantly higher than in the control group. There was a significant negative correlation between serum L-FABP concentration and standard deviation score (SDS) for diastolic blood pressure (DBP). A significant negative correlation was found between serum IL-6 concentration and systolic blood pressure (SBP), DBP and mean arterial pressure (MAP) values as well as SDS for SBP and DBP. In addition, a significant positive correlation was found between urinary IL-6 concentration and estimated glomerular filtration rate (eGFR). CONCLUSIONS: Higher concentration of L-FABP in serum and urine in children with kidney cysts indicates the early damage to the renal parenchyma, detectable before the onset of hypertension and other organ damage. Significantly higher serum and urinary IL-6 levels in children with cystic kidney disease compared to healthy children may suggest the role of this cytokine in chronic kidney disease development.

Angiotensinogen and interleukin 18 in serum and urine of children with kidney cysts.

BACKGROUND: The most common disease associated with the presence of kidney cysts in the population is autosomal dominant polycystic kidney disease (ADPKD), which finally leads to end-stage renal disease. METHOD: The study evaluated serum and urinary concentration of angiotensinogen (AGT) and interleukin 18 (IL-18) in a group of 39 children with renal cysts of different aetiology. RESULTS: Serum and urinary AGT concentration in children with renal cysts was higher compared to controls, regardless of the underlying background and gender. Serum IL-18 concentration was lower, in contrast, and the concentration of IL-18 in the urine did not differ between affected and healthy children. Negative correlation between urinary IL-18 concentration and systolic and mean arterial blood pressure was noted. CONCLUSIONS: Higher AGT levels in serum and urine in children with renal cysts may indicate the activation of the renin-angiotensin-aldosterone system, including its intrarenal part, even before the onset of hypertension. Lower serum concentration of IL-18 in children with kidney cysts may indicate the loss of the protective role of this cytokine with the occurrence of hypertension.

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.

Rare case of nephrocalcinosis in a 14-year-old girl: Questions.

A 14-year-old Caucasian girl with a history of primary hypoparathyroidism and unstable calcium and phosphorus levels and on ongoing treatment was admitted to the Department of Pediatric Nephrology because of the onset of nephrocalcinosis and difficulties achieving normocalcemia. Coexistence of hypoparathyroidism, oral candidiasis, dental enamel hypoplasia, and subclinical Hashimoto's disease was strongly suggestive for autoimmune polyglandular syndrome (APS) type I. One of the clinical implications of this diagnosis is the high probability of future occurrence of adrenal insufficiency and hence the importance of maintaining a high level of suspicion in case of the onset of symptoms like weakness, fainting, hypotonia, or hyperkaliemia. Addison's disease would, in fact, be quite challenging for the future management of this patient.This clinical quiz highlighted the importance of careful evaluation of all multiorgan symptoms occurring in a patient to prevent further complications.

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.

Polish 2010 growth references for school-aged children and adolescents.

Growth references are useful in monitoring a child's growth, which is an essential part of child care. The aim of this paper was to provide updated growth references for Polish school-aged children and adolescents and show the prevalence of overweight and obesity among them. Growth references for height, weight, and body mass index (BMI) were constructed with the lambda, mu, sigma (LMS) method using data from a recent, large, population-representative sample of school-aged children and adolescents in Poland (n = 17,573). The prevalence of overweight and obesity according to the International Obesity Taskforce definition was determined with the use of LMSGrowth software. Updated growth references for Polish school-aged children and adolescents were compared with Polish growth references from the 1980s, the Warsaw 1996-1999 reference, German, and 2000 CDC references. A positive secular trend in height was observed in children and adolescents from 7 to 15 years of age. A significant shift of the upper tail of the BMI distribution occurred, especially in Polish boys at younger ages. The prevalence of overweight or obesity was 18.7% and 14.1% in school-aged boys and girls, respectively. The presented height, weight, and BMI references are based on a current, nationally representative sample of Polish children and adolescents without known disorders affecting growth. Changes in the body size of children and adolescents over the last three decades suggest an influence of the changing economical situation on anthropometric indices.

[Carriage of Streptococcus pneumoniae in nasopharynx in children with nephrotic syndrome aged 2-5 years in Poland]. / Nosicielstwo nosogardiowe Streptococcus pneumoniae u dzieci w wieku 2-5 lat z zespolem nerczycowym w Polsce.

Children with nephrotic syndrome (NS) are at increased risk of Streptococcus pneumoniae infections. Streptococcus pneumoniae carrier state in the nasopharynx is an early stage of this infections. The aim of the study was to assess carriage rate and characterise bacterial isolates of Streptococcus pneumoniae among children with NS in a prospective multicentre study involving 10 nephrologic centres in Poland. We studied 95 children including 56 boys (59%) i 39 girls (41%) with NS in mean age 3 i 7/12 +/- 11 miesiecy. All children had throat and nasopharynx swabs taken in two epidemiological seasons (Oct-Dec 2006 and Feb-Apr 2007). Streptococcus pneumoniae isolates were serotyped with PCR. Carriage of Streptococcus pneumoniae was diagnosed in 13 children (13.7%) including 13 boys (100%). Following serotypes were found: 6B (38.5%), 9V (15.3%), 23F (15.3%), 19F (7.6%). There were 23.3% isolates without identifiable serotype. Carriage of Streptococcus pneumoniae was diagnosed mostly in boys with NS. Serotype 6B was dominant in this age group.

[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.

[Port device central venous access in children with chronic renal disease--personal experience]. / Zastosowanie centralnych dostepów zylnych typu "port" u dzieci z przewleklymi chorobami nerek--doswiadczenia wllsne.

UNLABELLED: The application of central venous lines in children has been widely accepted in the case of pediatric cancer treatment. This is of particular importance when the treatment must be continued during the long period of time. The indication to long-term application of central venous lines became significantly frequent within last years. They are necessary in the treatment of chronic pediatric patients, in whom the central venous line allows continuous access for medication, parenteral rehydration, nutrition and frequent blood sampling. MATERIAL AND METHODS: In the current study authors present their experience in subcutaneous port devices application in children with kidney disease. The case history data obtained from 8 children were retrospectively analysed. In these children subcutaneous port devices were applied for mean 26.7 months (totally 9 port devices). The mean age at the time of implantation was 2.2 years, and the mean body weight--10.6 kg. RESULTS: Peripheral venous access in all children was bad. In one child during the time of implantation the hematoma of coli and chest was present. Infectious complications connected with implanted port device were not detected. Thrombotic complications were present in 6 children with chronic renal failure--in 5 the lumen of port device has been successfully recanalysed, in 3 cases even several times. In 1 child the thrombus on the tip of central venous line was detected. In 2 children the removal of port device was necessary because of breakage of venous line and in the second case because of port device thrombosis. Two children died with functioning port device. The cause of death was not connected with implanted port device. CONCLUSIONS: The application of subcutaneous port devices definitely improved the comfort of treatment but was significantly associated with thrombotic complications. Infectious complications were not detected as compared to hematological group of patients.

[The diagnosis and treatment of ureteral enuresis in a girl aged 14 with unexpected explanation of the cause of the disorder--a case report]. / Przebieg diagnostyki i leczenia moczenia moczowodowego u 14-letniej dziewczynki z nieoczekiwanym wyjasnieniem przyczyny dolegliwosci--opis przypadku.

In our case report we present the course of diagnosis and treatment of primary urinary incontinence in a girl aged 14. In abdominal ultrasound examination we found features of double renal pelvis on the left side. Intravenous urography confirmed that anatomical variant. Moreover it gave the suggestion of duplicated left ureter. Regarding the clinical data and the results of ultrasonography and urography, we suspected that the symptoms might be connected with the ectopic orifice of one from the left ureters, probably that one associated with the upper renal pelvis. We managed to find this ectopic ureteral orifice in the vaginal vestibule. But when we performed antegrade pyelography through that orifice, we found that it constituted the ending of additional right ureter, which was not imaged in previous examinations. That diagnosis was very unexpected. The child was operated on. During the operation a small, hypoplastic additional right kidney was discovered. That kidney was excised together with the ectopic ureter. The urine incontinence stopped immediately after the operation.

[Treatment of chronic hepatitis with interferon in children with kidney diseases]. / Leczenie interferonem przewleklego zapalenia watroby u dzieci z chorobami nerek.

UNLABELLED: The study aimed at evaluation of chronic hepatitis treatment results in children previously treated from nephrological indications in 1994-2002 years. MATERIAL AND METHODS: Examination was performed in 42 children in the age 10.2 +/- 4.8 years at the onset time of interferon (INF) treatment application. In 30 children (71.4%) chronic HBV infection, in 8 (19.1%) - HCV infection, in 4 (9.5%) - mixed HBV and HCV infection in phase of replication was revealed. Among examined children in 26 (61.9%) symptoms of glomerulonephritis were previously reported, in 17 (40.5%) - symptoms of nephrotic syndrome; in 9 (21.4%) - chronic renal failure was observed. 22 children received prednisone treatment. Concentrations of albumin, gamma- globulin, bilirubin, haemoglobin, creatinine, haematocrit, leukocytosis, activity of alanine aminotransferase (ALT), chronic hepatitis markers, before, during and 6 and 12 months after treatment termination were evaluated. IFN alpha-2a, alpha-2b and human recombined IFN-alpha were applied RESULTS: In 22 (52.4%) children ALT values before treatment not exceeded 100 IU/l. Liver biopsy was performed in 39 children. In 18 (46.2%) - high activity of inflammatory process was revealed; only in 5 of them with ALT activity above 100 IU/l. Higher leukocytosis at the beginning of treatment was accompanied by diminished activity of inflammatory process. In 14/34 children seroconversion was obtained in HBe markers, in 4/12 HCV-RNA elimination occurred after the 1st course of IFN. Only in 5 (11.9%) children treatment was stopped because of side effects (not connected with urinary tract), in 1 - because of relapse of main disease. 2nd course was applied in 13 children. In 2 - seroconversion in HBe markers was obtained. CONCLUSIONS: Considering the small number of examined children full evaluation of chronic hepatitis treatment efficacy is not possible. It seems comparable as observed in population of children without the risk of nephropathies. IFN treatment in children on previous medication of kidney disease, in most cases does not create complications leading to earlier drug cessation. In the case of glomerulonephritis also does not bear an increased risk of relapse of main disease.