Aim To assess the association between the Urinary Tract Dilatation (UTD) Antenatal (A) and Postnatal (P) Classification System grade and the outcome in term newborns. Methods This retrospective study ...enrolled 166 term newborns (71% boys, 206 ureterorenal units) evaluated for unilateral or bilateral UTD in the Neonatology Department of Ljubljana University Medical Center from 2012 to 2018. Data on family history, sex, gestational age, birth weight, head circumference, Apgar score, possible oligohydramnios, indication for and age at first postnatal ultrasound, time of follow-up, and clinical outcome were collected. Radiology records were reviewed to grade UTD according to the Multidisciplinary Consensus on the Classification of Prenatal and Postnatal UTD. Results The majority of ureterorenal units with UTD A 2-3 had UTD P 2 or 3. Spontaneous resolution, specific uropathy, the need for surgery, and the risk of urinary tract infection were all significantly associated with the UTD P grade. No patient experienced renal dysfunction at the end of follow-up (12-48 months, median 24 months), and therefore this parameter was not associated with the UTD P grade. Conclusions The UTD grade was associated with the probability of spontaneous resolution, time to its occurrence, specific uropathies urinary tract infection, and risk for surgery. However, no association with renal dysfunction was established.
Celotno besedilo
Dostopno za:
DOBA, IZUM, KILJ, NUK, PILJ, PNG, SAZU, SIK, UILJ, UKNU, UL, UM, UPUK
The aim of our study was to establish if 48-hour ambulatory blood pressure monitoring (ABPM) is more reliable than 24-hour monitoring in order to reduce the impact of external factors on blood ...pressure (BP) in children.
Two hundred fifteen children with indications for ABPM underwent 48-hour ABPM. In the first part of the study, the values of systolic BP (SBP), diastolic BP (DBP) and heart rate between the first 24 hours and the total 48 hours were compared. In the second part, the same parameters between the first and second 24 hours were compared. In the third part, we determined whether the differences in measurements between the first and second 24 hours had clinical significance and meant a change of diagnosis.
The results of the first part of the study showed no statistically significant differences. In the second part, the average values of SBP and DBP were statistically significantly higher during the first 24 hours as compared with the second 24 hours. In the second 24 hours, more children had normal BP (NBP) and a smaller number of them had high NBP or arterial hypertension (AH) compared with the first 24 hours, suggesting that this has clinical significance.
Within certain limits, the results of our study suggest that 48-hour ABPM is more reliable than 24-hour ABPM, where especially the second 24 hours are a period of measurement that should be taken into consideration for this purpose. These results could impact the diagnostics of AH in children.
Background: Second‐generation echo‐enhanced voiding urosonography (VUS) with improved contrast‐specific ultrasound (US) techniques is increasingly being used for vesicoureteric reflux (VUR) ...detection.
Aim: To compare VUS with X‐ray voiding cystourethrography (VCUG) and to evaluate the predictive value of pelvicalyceal dilatation for detecting VUR in the first year of life.
Materials and methods: Sixty‐six children younger than one were investigated by US, VUS and VCUG. VCUG represented the reference method for VUR detection.
Results: According to VCUG, VUR was present in 16/132 (12%) renal units. VUS confirmed all these and additionally revealed VUR in 26 renal units (all together 42/132 (32%) renal units with VUR). It should be noted that all VUR detected only with VUS and missed by VCUG were of grades II and III. In 27/92 (29%) US normal and in 15/40 (37%) renal units with pelvicalyceal dilatation, VUR was detected by VUS.
Conclusion: We believe that VUS could safely replace VCUG in VUR detection in infants, with no regard to the degree of abnormality of the upper urinary tract seen on US. VCUG should be limited only to cases where bladder and/or urethra pathology are suspected.
V preglednem prispevku »Algoritem obravnave otroka s sumom na prirojene nepravilnosti sečil« predstavljamo sodobne smernice za obravnavo otrok s sumom na prirojene nepravilnosti sečil, ki slonijo na ...trenutno priporočeni novi klasifikaciji antenatalne razširitve votlega sistema sečil (angl. antenatal urinary tract dilatation, UTD-A) in postnatalne razširitve votlega sistema sečil (angl. postnatal urinary tract dilatation, UTD-P). Osredotočamo se na priporočila za obravnavo UTD-P, saj obravnavo UTD-A podrobno predstavljamo v posebnem poglavju »Prenatalno diagnosticiranje prirojenih nepravilnosti sečil«. Nazadnje se posvetimo še obravnavi posebne skupine otrok s prirojenimi napakami sečil, pri katerih predvidevamo urološki poseg.
Okužbe sečil so med najpogostejšimi bakterijskimi okužbami pri otrocih vseh starosti, zlasti pri dojenčkih in majhnih otrocih. Akutne okužbe zgornjih sečil lahko v nekaterih primerih celo ogrozijo ...otrokovo življenje. V kombinaciji s prirojenimi nepravilnostmi sečil so lahko povezane z zapleti, kot so arterijska hipertenzija, kronična ledvična bolezen in končna ledvična odpoved. Zaradi naštetega je pravilno diagnosticiranje akutne okužbe sečil izrednega pomena. Diagnoza okužba sečil temelji na prisotnosti kliničnih in laboratorijskih znakov v kombinaciji s pomembno bakteriurijo oz. pozitivno urinokulturo pravilno odvzetega vzorca seča. V preglednem prispevku predstavljamo vse pomembne faze pravilnega diagnosticiranja akutne okužbe sečil – od kliničnih znakov, laboratorijskih izvidov, izvidov slikovnih preiskav, značilnih za akutno okužbo sečil, in načinov pravilnega odvzema vzorca seča do načinov ugotavljanja bakteriurije ter definicij pomembne bakteriurije in brezsimptomne bakteriurije. V zaključku podajamo okvirna navodila, ki jih mora zdravnik upoštevati ob sumu na okužbo sečil pri otroku.
We describe a case of a post vaccine immune complex-mediated glomerulonephritis in an infant with compound heterozygous mutations of C2 complement component gene, which is the first such case in the ...literature. The three and a half months old boy presented with clinical and laboratory signs of nephritic syndrome and was successfully treated with methylprednisolone. An explanation of such a clinical picture may lie in the interaction between C2 deficiency and vaccination.
Celotno besedilo
Dostopno za:
DOBA, IZUM, KILJ, NUK, PILJ, PNG, SAZU, SIK, UILJ, UKNU, UL, UM, UPUK
Razširjen votli sistem sečil (angl. urinary tract dilatation, UTD) je ena najpogostejših najdb na ultrazvočnem pregledu med nosečnostjo. Čeprav so prirojene nepravilnosti sečil (angl. congenital ...anomalies of the kidney and urinary tact, CAKUT) pred rojstvom otroka in po njem pogosto brezsimp-tomne, imata njihovo odkrivanje in optimalna obravnava prenatalno in po rojstvu otroka zelo velik klinični pomen, saj so med najpogostejšimi vzroki končne ledvične odpovedi pri otrocih. Različne izraze za opis UTD (hidronefroza, pielektazija, kaliektazija, pelvikaliektazija, uronefroza itd.) danes opuščamo. Dosedanje različne definicije stopenj UTD prenatalno in postnatalno, ki med različnimi strokovnimi združenji niso bile poenotene, so vodile v različne klinične prakse glede sledenja in obravnav ploda in otroka. To je dodatno oteževalo uporabo UTD kot napovednega dejavnika pri plodu in pri novorojenčku. Najnovejša klasifikacija prenatalne in postnatalne UTD temelji na multidisciplinarnem dogovoru različnih združenj in različnih specialistov, ki obravnavajo otroke z UTD pred rojstvom in po njem. Sistem omogoča enotno razvrstitev UTD na skupine z natančno opredeljenimi ultrazvočnimi parametri, enotno terminologijo, vrednotenje razmerja med prenatalno UTD in postnatalno UTD, končno diagnozo in napoved tveganja za klinično pomembno nepravilnost sečil.
C1q nephropathy is an uncommon glomerular disease with characteristic features on immunofluorescence microscopy. In this report, clinicopathologic correlations and outcomes are presented for 72 ...patients with C1q nephropathy. The study comprised 82 kidney biopsies from 28 children and 54 adults with male preponderance (68%). Immunofluorescence microscopy showed dominant or co-dominant staining for C1q in the mesangium and occasional glomerular capillary walls. Electron-dense deposits were observed in 48 of 53 cases. Light microscopy revealed no lesions (n = 27), focal segmental glomerulosclerosis (FSGS; n = 11), proliferative glomerulonephritis (n = 20), or various other lesions (n = 14). Clinical presentations in the patients who had no lesions histology were normal urine examination (7%), asymptomatic hematuria and/or proteinuria (22%), and nephrotic syndrome (minimal change-like lesion; 63%), which frequently relapsed. All patients with FSGS presented with nephrotic syndrome. Those with proliferative glomerulonephritis usually presented with chronic kidney disease (75%) or asymptomatic urine abnormalities (20%). Of the patients with sufficient follow-up data, complete remission of the nephrotic syndrome occurred in 77% of those with a minimal change-like lesion, progression to end-stage renal disease occurred in 33% of those with FSGS, and renal disease remained stable in 57% of those with proliferative glomerulonephritis. In conclusion, this study identified two predominant clinicopathologic subsets of C1q nephropathy: (1) Podocytopathy with a minimal change-like lesion or FSGS, which typically presents with nephrotic syndrome, and (2) a typical immune complex-mediated glomerular disease that varies from no glomerular lesions to diverse forms of glomerular proliferation, which typically presents as chronic kidney disease. Clinical presentation, histology, outcomes, and presumably pathogenesis of C1q nephropathy are heterogeneous.
The European Rare Kidney Disease Reference Network (ERKNet) recently established ERKReg, a Web-based registry for all patients with rare kidney diseases. The main objectives of this core registry are ...to generate epidemiological information, identify current patient cohort for clinical research, explore diagnostic and therapeutic management practices, and monitor treatment performance and patient's outcomes. The registry has a modular design that allows to integrate comprehensive disease-specific registries as extensions to the core database. The diagnosis (Orphacode) and diagnostic information (clinical, imaging, histopathological, biochemical, immunological and genetic) are recorded. Anthropometric, kidney function, and disease-specific management and outcome items informing a set of 61 key performance indicators (KPIs) are obtained annually. Data quality is ensured by automated plausibility checks upon data entry and regular offline database checks prompting queries. Centre KPI statistics and benchmarking are calculated automatically. Within the first 24 months since its launch, 7607 patients were enrolled to the registry at 45 pediatric and 12 specialized adult nephrology units from 21 countries. A kidney disease diagnosis had been established in 97.1% of these patients at time of enrolment. While 199 individual disease entities were reported by Orphacode, 50% of the cohort could be classified with 11, 80% with 43 and 95% with 92 codes. Two kidney diagnoses were assigned in 6.5% of patients; 5.9% suffered from syndromic disease. Whereas glomerulopathies (54.8%) and ciliopathies including autosomal dominant polycystic kidney disease (ADPKD) (31.5%) were the predominant disease groups among adults, the pediatric disease spectrum encompassed congenital anomalies of the kidney and urinary tract (CAKUT) (33.7%), glomerulopathies (30.7%), ciliopathies (14.0%), tubulopathies (9.2%), thrombotic microangiopathies (5.6%), and metabolic nephropathies (4.1%). Genetically confirmed diagnoses were reported in 24% of all pediatric and 12% adult patients, whereas glomerulopathies had been confirmed by kidney biopsy in 80.4% adult versus 38.5% pediatric glomerulopathy cases. ERKReg is a rapidly growing source of epidemiological information and patient cohorts for clinical research, and an innovative tool to monitor management quality and patient outcomes.
Understanding the role of alternative complement pathway dysregulation in membranoproliferative glomerulonephritis (MPGN) has led to a dramatic shift in its classification into two subgroups: immune ...complex-mediated MPGN and complement-mediated MPGN, consisting of dense deposit disease and C3 glomerulonephritis (C3GN). A limited number of C3GN cases have been published to date with not yet conclusive results since the novel therapeutic approach with eculizumab was introduced.
We report the clinical follow-up of a 16-year-old patient in whom a diagnosis of C3GN was confirmed by immunofluorescence and electron microscopy in second and third kidney biopsies, while the first biopsy revealed idiopathic immune complex-mediated MPGN type III, Anders and Strife variant, which failed to improve after several attempts at conventional immunosuppression therapy. Although applied late in an already fairly advanced stage of the severe active form of MPGN, the efficacy of eculizumab on C3GN was evidenced clinically and pathohistologically. Its beneficial influence on pathomorphogenesis was demonstrated by a unique follow-up in the last three biopsies, despite the recent observation, confirmed in this study, of eculizumab binding within the kidney tissue.
Clinicians and pathologists should be aware that, in some patients, an underlying genetic or acquired complement alternative pathway abnormality can be masked by an initial immune complex-mediated mechanism, which subsequently triggers an unbalanced excessive continual driving of complement terminal pathway activation and the development of C3GN. In such a patient, supplementary steroids in addition to eculizumab appear necessary to achieve an adequate response.
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