Predictors of Progression in IgA Nephropathy in Childhood.

The aim of this retrospective study was to assess the usefulness of potential predictors of poor prognosis in IgA nephropathy in children. The study population consisted of 55 children aged 11 ± 4 years, diagnosed on the basis of the Oxford classification and MEST score of kidney biopsy findings. Proteinuria, glomerular filtration rate (GFR), and the IgA/C3 serum ratio were assessed in all patients twice: at onset and at follow-up. The patients were treated with steroids, immunosuppressive drugs, and/or angiotensin-converting enzyme inhibitors. Follow-up was at 3.9 ± 2.9 (median 2.7) years. The patients were subdivided into two groups: with GFR <90 and ≥90 mL/min at follow-up. ROC AUC curves and logistic regression were used to evaluate the power of prognostic factors. The two groups did not differ regarding the level of proteinuria, MEST score, and the IgA/C3 ratio at onset of disease. There was a significant association between GFR reductions at onset and follow-up (AUC = 0.660; p < 0.05). In patients with nephrotic range proteinuria at onset, proteinuria at follow-up was more frequent compared with other patients (AUC = 0.760; p < 0.05), MEST score ≥3 tended to be associated with reduced GFR (AUC = 0.650; p = 0.07) but not with proteinuria (AUC = 0.608; p = 0.47), and the IgA/C3 ratio was higher (p < 0.05) at follow-up. No significant associations were found between the IgA/C3 ratio at onset and reduced GFR (AUC = 0.565; p = 0.46) or proteinuria at follow-up (AUC = 0.263; p = 0.20). We conclude that predictors of poor outcome in childhood IgAN include the following: GFR reduction, nephrotic range proteinuria at onset of disease, and high MEST score in Oxford classification of kidney biopsy. Despite a higher serum IgA/C3 ratio in children with impaired renal function in long-term follow-up, we failed to demonstrate a significant association between this ratio at onset of disease and reduced GFR or persistent proteinuria at follow-up. Thus, IgA/C3 ratio is not a good foreteller of progression of IgA nephropathy in childhood.

IgA Nephropathy in Children: A Multicenter Study in Poland.

IgA nephropathy (IgAN) is the most common form of glomerulonephritis in pediatric population. The clinical presentation of the disease in children ranges from microscopic hematuria to end-stage kidney disease. The aim of the study was to retrospectively assess clinical and kidney biopsy features in children with IgAN. We assessed a cohort of 140 children, 88 boys, 52 girls with the diagnosis of IgAN in the period of 2000-2015, entered into the national Polish pediatric IgAN registry. The assessment included the following: proteinuria, hematuria, glomerular filtration rate (GFR), arterial blood pressure, and the renal pathological changes according to the Oxford classification and crescents formation, as modifiable and unmodifiable risk factors. The incidence of IgAN in Poland was set at 9.3 new cases per year. The mean age at onset of IgAN was 11.9 ± 4.3 years, and the most common presentation of the disease was the nephritic syndrome, recognized in 52 % of patients. Kidney biopsy was performed, on average, 1.3 ± 2.0 years after onset of disease. Based on the ROC analysis, a cut-off age at onset of disease for GFR <90 mL/min/1.73 m2 (risk factor of progression) was calculated as 13.9 years. Unmodifiable lesions: segmental sclerosis, tubular atrophy/interstitial fibrosis (S1, T1-2) in the Oxford classification and crescents in kidney biopsy were significantly more common in Gr 1 (>13.9 years) compared with Gr 2 (<13.9 years), despite a significantly shorter time to kidney biopsy in the former. We conclude that IgAN in children may be an insidious disease. A regular urine analysis, especially after respiratory tract infections, seems the best way for an early detection of the disease.

Increased Serum IgA in Children with IgA Nephropathy, Severity of Kidney Biopsy Findings and Long-Term Outcomes.

The aim of the study was to determine whether an elevated IgA level at the time of the diagnosis of IgA nephropathy has an effect on the severity of kidney biopsy findings and long-term outcomes in children. We retrospectively studied 89 children with IgA nephropathy who were stratified into Group 1- elevated serum IgA and Group 2 - normal serum IgA at baseline. The level of IgA, proteinuria, hematuria, glomerular filtration rate (GFR) and hypertension (HTN) were compared at baseline and after the end of the follow-up period of 4.0 ± 3.1 years. Kidney biopsy findings were evaluated using the Oxford classification. The evaluation of treatment included immunosuppressive therapy and renoprotection with angiotensin converting-enzyme inhibitor (ACEI) or angiotensin II receptor blocker (ARB), or no treatment. The elevated serum IgA was found in 46 (52 %) patients and normal serum IgA level was found in 43 (48 %) patients. No differences were found between the two groups regarding the mean age of patients, proteinuria, and the number of patients with reduced GFR or HTN at baseline. In kidney biopsy, mesangial proliferation and segmental sclerosis were significantly more common in Group 1 compared with Group 2 (p < 0.05). Immunosuppressive therapy was used in 67 % children in Group 1 and 75 % children in Group 2. The Kaplan-Meier survival curves for renal function (with normal GFR) and persistent proteinuria did not differ significantly depending on the serum IgA level at baseline. We conclude that in IgA nephropathy the elevated serum IgA at baseline may be associated with mesangial proliferation and segmental sclerosis contribute to glomerulosclerosis, but has no effect on the presence of proteinuria or on the worsening of kidney function during several years of disease course.

[13C2]oxalate absorption in children with idiopathic calcium oxalate urolithiasis or primary hyperoxaluria.

Intestinal oxalate absorption is an important part of oxalate metabolism influencing its urinary excretion and its measurement can be a valuable diagnostic tool in hyperoxaluric disorders. In this study, we use [(13)C(2)]oxalate absorption under standardized dietary conditions to assess intestinal oxalate absorption and its impact on urinary oxalate excretion. Tests were conducted in age-matched pediatric patients that included 60 with idiopathic calcium oxalate urolithiasis, 13 with primary hyperoxaluria, and 35 healthy children. In the idiopathic stone formers, median oxalate absorption was significantly higher than that in the controls or in patients with primary disease. From standardized values obtained in control patients, oxalate hyperabsorption was detected in 23 patients with idiopathic disease but not in any patients with primary hyperoxaluria; therefore, a significant correlation between intestinal absorption and urinary excretion was found only in those with the idiopathic disease. We have shown that increased intestinal oxalate absorption is an important risk factor of idiopathic calcium oxalate urolithiasis. In contrast, low intestinal oxalate absorption in patients with primary hyperoxaluria indicates that only foods with excessive oxalate content be restricted from their diet.

Cytoplasmic streaming direction reverses in dividing Paramecium bursaria.

Using the interference-contrast videomicroscopy the speed of cytoplasmic streaming was measured during the sequence of division stages in thigmotactically settled specimens of Paramecium bursaria. The speed of cytoplasmic flow gradually decreased during the first stages of binary fission and movement became indistinguishable at stage D(3). Almost at the same time cytoplasm started to move in the opposite direction, pushing or pulling the dividing micronucleus into the prospective posterior daughter cell and eventually stopped at stage D(5)-D(6). Further cell division events proceeded without detectable movement of cytoplasmic components. Cytoplasmic streaming in the normal interphase route was gradually restored in daughter cells about 30-40 min after cell separation. During the whole period of binary fission phagocytosis was arrested. Transportation and participation in the positioning of prospective micronuclei in daughter cells seems to be the main function of cytoplasmic streaming activity in cell division of Paramecium bursaria. The possible relationship between the stages of cytoskeleton transitions and the kinetics of cytoplasmic streaming associated with cell divison is discussed.

[The assessment of Helicobacter pylori eradication with the urea breathing test (13C UBT)]. / Ocena skutecznosci eadykacji zakazenia Helicobacter pylori u dzieci z zespolem nerczycowym przy zastosowaniu mocznikowego testu oddechowego (13C UBT).

UNLABELLED: The purpose of the study was to assess the efficacy of Helicobacter pylori (HP) infection treatment in children with the nephrotic syndrome, using non-invasive urea breathing test (13C UBT). The study comprised 20 children with nephrotic syndrome treated with prednisone. Children were divided into two groups: those who recurred prednisone for a short time (1-3 months) and those treated longer than 6 months. In all children gastroscopic examination, histopathologic assessment of gastric mucous membrane specimens and 13C UBT were performed. 65% of nephrotic children was HP (+). In those treated for 1-3 months incidence of HP infection was 40%, whereas in those were treated longer than 6 months it was 90%. In all children treatment of HP infection was instituted. Seven weeks after cessation of treatment its efficacy was assessed by 13C UBT. The treatment was successful in all nephrotic children. In the nephrotic controls with negative histopathologic assessment for HP infection the results of UBT were normal. CONCLUSION: 1. Children with the nephrotic syndrome are more susceptible to HP infection as compared with healthy ones. 2. In those children treatment of HP infection is efficient. 3. 13C UBT allows to estimate HP infection treatment efficacy in a non-invasive way.

[A comparison of blood pressure load in groups of children with high and low levels of neuroticism]. / Porównanie ladunku cisnienia tetniczego w grupach dzieci o wysokim i niskim poziomie neurotyzmu.

The significance of mental stress in the etiology and pathogenesis of essential hypertension is still a matter of disputes and controversies. The authors wondered if normotensive children prone to stress reactions show significant abnormalities in their ABPM patterns. The aim of this study was comparison of blood pressure loads in children of high and low neuroticism levels. 121 healthy children underwent the psychometric test evaluating the level of neuroticism. From this group 43 children with the lowest and 33 children with the highest levels of neuroticism were selected. The groups were comparable in respect of age, gender, overweight, obesity and low birth weight. 24-hour ambulatory blood pressure monitoring was performed. Average systolic and diastolic blood pressure loads were calculated. In neurotic boys and girls considered together and girls analysed separately both systolic and diastolic blood pressure loads were significantly higher than in their low-neurotic counterparts. In boys only systolic blood pressure load difference between high and low-neurotic group was statistically significant. Diastolic blood pressure load only tended to be higher in neurotic boys, without statistical significance. In our study neurotic children demonstrated in general significantly higher blood pressure loads than low-neurotic children. The prognostic value of this difference has not yet been determined. Perhaps further investigation would prove risk of developing hypertension in neurotic children with levels of blood pressure loads as observed in our study. If so, introducing of hypertension preventive measures could be profitable in neurotic children.

[Health status and physical development of children delivered by mothers who suffered from nephrotic syndrome in their infancy]. / Ocena stanu zdrowia i rozwoju fizycznego dzieci matek, które w dziecinstwie przebyly zespól nerczycowy.

Numerous complications of nephrotic syndrome due to either the illness itself or repeated courses of high-dose corticosteroids have been well recognised for several score years. However, investigations concerning children of ex-patients are lacking. The purpose of this study was to assess physical development and health state in the group of children whose mothers had suffered from the idiopathic nephrotic syndrome during their childhood. 34 such children were studied. Thorough medical history was taken from children and their mothers and available medical documents data were obtained. Physical examination was performed and centile position of weight, height and their proportion was stated. The authors found that stillborn foetus, premature or low birth weight newborn incidence is much higher than in general population and the percentage of overweight and obesity is higher in the studied group than in general population. To confirm these findings further investigations with greater number of patients are necessary.

[Evaluation of oxalate/creatinine ratio in the second morning urine sample of health school children]. / Ocena wskaznika szczawianowo-kreatyninowego w II rannej porcji moczu u zdrowych dzieci w wieku szkolnym.

The evaluation of urinary oxalate excretion is one of the most important diagnostic methods in patients with urolithiasis and/or nephrocalcinosis. Since reliable 24-h urine collections are difficult to obtain in children, excretion ratios of oxalate over creatinine are increasingly being used from single urine specimens. The aim of the study was to determine the normal values of oxalate/creatinine ratios in the second morning urine sample in healthy school children. The study involved 109 children between 6 and 16 years of age. The results showed that the values of Ox/Cr ratios are decreased in older children and there was significant difference between children under and above 12 years of age (values of the 95th percentile--0.076 and 0.051 mmol/mmol respectively). The significant correlation between 24-hours urinary oxalic acid excretions and Ox/Cr ratios (r-0.756) was found. We conclude, that Ox/Cr ratio is valuable parameter for screening purposes in children.

[A nineteen year observation of a boy with branchio-oto-renal syndrome and chronic renal failure]. / Dziewietnastoletnia obserwacja chlopca z zespolem skrzelowo-uszno-nerkowym i przewlekla niewydolnoscia nerek.

Branchio-oto-renal syndrome is a rare autosomal dominant disorder of the first and second embryonic branchial arches and the urinary tract. It is characterized in its full expression by branchial fistulas or cysts, preauricular pits, outer, middle and inner ear defects, hearing loss, lachrymal duct stenosis, facial paralysis and mental retardation. Renal anomalies may range from mild hypoplasia to complete absence. Our report demonstrates the patient with classical BOR syndrome and severe renal insufficiency since infancy up to end stage renal failure at 18 years of age caused by bilateral renal hypoplasia. Although no definitive histological diagnosis was made, the clinical findings in our patients, like mild proteinuria, normal blood pressure, polyuria, polydypsia, hyperchloremic acidosis and typical course of renal failure support the diagnosis of oligomeganephronia in this case.

[Evaluation of respiratory tract functions in children with chronic renal failure treated with hemodialysis]. / Ocena czynnosci ukladu oddechowego u dzieci z przewlekla niewydolnoscia nerek leczonych hemodializami.

The respiratory tract functions were evaluated in 9 children with severe renal failure treated with hemodialysis. In the most of observed children the values of spirometric parameters were improved after hemodialysis.

[Urinary calcium/creatinine, phosphate/creatinine, magnesium/creatinine and Sulkowicz test in the second morning urine sample in neonates and infants]. / Wskaznik wapniowo-kreatyninowy, fosforanowo-kreatyninowy, magnezowo-kreatyninowy i próba sulkowicza w drugiej rannej porcji moczu noworodków i zdrowych niemowlat.

The aim of the paper was to determine the values of the 5th and 95th percentiles of Ca/Cr, P/Cr and Mg/Cr, ratios in the second-morning urine samples in healthy neonates and infants. The collected Ca/Cr ratios were correlated with the results of Sulkowicz test performed on the same urine sample. The tests were done on the group of 30 neonates and 43 infants. The comparison of the 95th percentiles in the individual ratios in tested groups showed low values in the early newborn, distinctive increase in the first half followed by the decrease in the second half of the first year of live. There was no correlation found between the Ca/Cr ratios and the results of Sulkowicz tests.

[Anatomic and functional disorders of the urinary tract in children with urinary tract infections]. / Zaburzenia anatomiczne i czynnosciowe dróg moczowych u dzieci z zakazeniem ukladu moczowego.

The study is based on clinical analysis of 207 case recordings of children with urinary tract infection (UTI) hospitalized in The Department of Pediatric Nephrology, University Hospital, Lublin in 1995-1999. The study comprised children aged 1-18 years, average 8.8 +/- 0.4 (77.8% girls). Recurrent UTI was observed in 76.8% of children. Acute UTI was diagnosed in 20.8% of children. In 2.4% of children UTI was detected during hospitalization because of other diseases. In all children urinary X-ray examination was performed. Anatomical abnormalities were detected in 38% of children. Vesico-ureteral reflux was diagnosed in 61 children (29.5%). In 16/25 (64%) of children with vesico-ureteral reflux renal scarring was detected by radionuclide examination. In some children urodynamic examination was performed and in all of them functional abnormalities were detected.

[Hypothyroidism as the cause of lowered blood pressure during hemodialysis]. / Niedoczynnosc tarczycy przyczyna znacznych obnizen cisnienia tetniczego podczas hemodializy.

We present a case of a 19.5 years old, hemodialyzed, girl with frequent and dramatic episodes of hypotension. She presented slightly expressed symptoms of hypothyroidism, while the serum levels of hTSH, T3, T4 and fT4 were close to the bottom border of norm. The therapy with L-thyroxin (1.75 mg/kg b. w./24h) normalized the arterial blood-pressure and led to subside coexisting symptoms of hypothyroidism.

[Influence of glucocorticoid steroid therapy on gastric and duodenal mucosa and Helicobacter pylori infection in children with nephrotic syndrome]. / Wplyw glikokortykosteroidoterapii na blone sluzowa zoladka i dwunastnicy oraz zakazenie Helicobacter pylori u dzieci z zespolem nerczycowym.

UNLABELLED: The purpose of the study was to evaluate the influence of steroid therapy on endoscopic picture of gastric mucosa. The study comprised 22 children (17 boys and 5 girls) with the nephrotic syndrome aged 4-12 years (the mean 9 years) treated with prednisone. Children were divided into two groups taking into account duration of therapy. The group I (10 children) comprised patients with therapy duration not exceeding 3 months. The group II (12 children) comprised children treated 5 years in average. All patients underwent gastroscopic examination. Macro and microscopic lesions of gastric mucosa, their intensity and coexistence of Helicobacter pylori (HP) infection were estimated. CONCLUSIONS: 1. In nephrotic children during prednisone therapy different types of gastric and duodenal mucosal inflammatory changes occur. 2. Duration of prednisone therapy does not influence significantly on location, type and severity of mucosal inflammatory changes. 3. Frequency of coexistent HP infection increases with prednisone therapy duration. 4. Prednisone therapy of nephrotic children is indication for gastric and duodenal endoscopic examination.