Prirojene nepravilnosti sečil so najpogostejši vzrok končne ledvične odpovedi pri otrocih. Ključnega pomena sta zgodnje diagnosticiranje in ustrezno zdravljenje in pogosto vključujeta sodelovanje ...različnih specialistov. Obravnava osnovne razvojne motnje je lahko konzervativna, kirurška ali kombinirana. Vedno zdravimo tudi zaplete in morebitne pridružene zdravstvene težave. Zaradi obsežnosti tematike smo se osredotočili na obravnavo otrok in kirurško zdravljenje tistih primerov, pri katerih indikacija za kirurško zdravljenje ni povsem jasna. Ker je obravnava otrok z obstruktivno nefropatijo in motnjo v delovanju spodnjih sečil opisana v ločenih prispevkih, na tem mestu prikazujemo zdravljenje otrok z multicistično displastično ledvico (tj. displazijo ledvic) in otrok s prirojenim vezikouretelnim refluksom. Opisujemo konzervativno vodenje in način kirurškega zdravljenja ter navajamo nekaj novejših spoznanj glede indikacij za kirurško zdravljenje. Najboljšega načina zdravljenja teh otrok še vedno ne poznamo natanko. Vsekakor je potreben individualni pristop. Cilj zdravljenja je ohranitev delovanja ledvic.
Slikovno diagnosticiranje je pomembno pri odkrivanju in opredelitvi prirojenih nepravilnosti sečil, na katere posumimo že v sklopu prenatalnega diagnosticiranja, takoj po rojstvu ali kasneje v ...življenju. Slikovno diagnosticiranje pomaga tudi pri določanju tveganja kronične ledvične bolezni v otroštvu, saj je potrebno spremljanje otrok, da zmanjšamo oziroma odložimo razvoj kronične ledvične bolezni.
ZAPLETENE AKUTNE OKUŽBE SEČIL PRI OTROCIH Blejc Novak, Ana; Ključevšek, Damjana; Plankar Srovin, Tina ...
Slovenska pediatrija,
11/2023, Letnik:
30, Številka:
4
Journal Article
Recenzirano
Odprti dostop
Ker v literaturi ni enotne definicije zapletenih okužb sečil pri otrocih, v prispevku kot zapletene okužbe sečil pri otrocih obravnavamo tiste okužbe, ki potekajo s hujšo in invazivno (sistemsko ali ...lokalno) klinično sliko ali se pojavijo ob dejavnikih tveganja. Predstavljamo torej žariščni nefritis, ledvični absces, pionefros, perirenalni absces, emfizematozni pielonefritis, ksantogranulomatozni pielonefritis, okužbe sečil ob ledvičnih kamnih in okužbe sečil ob vstavljenih umetnih pripomočkih. Omenjena stanja se pogosto razvijejo ob pridruženih dejavnikih tveganja, kot so funkcionalne in anatomske nepravilnosti sečil, vstavljeni umetni pripomočki (npr. urinski kateter, perkutana drenažna cevka), nedavni poseg na sečilih, okužbe z večkratno odpornimi mikroorganizmi, bolnišnične okužbe in imunosupresivno zdravljenje. Opisujemo tudi diagnostične možnosti in pristop k zdravljenju zapletenih okužb sečil, ki lahko vključuje tudi kirurško zdravljenje.
Recurrence of primary disease is one of the major risks for allograft loss after pediatric RTx. The risk of recurrence of FSGS/SRNS after pediatric RTx in particular can be up to 86% in idiopathic ...cases. There is a need for consensus recommendations on its prevention and treatment. The CERTAIN study group has therefore performed a thorough literature search based on the PICO model of clinical questions to formulate educated statements to guide the clinician in the process of decision‐making. A set of educated statements on prevention and treatment of FSGS/SRNS after pediatric RTx has been generated after careful evaluation of available evidence and thorough panel discussion. We do not recommend routine nephrectomy prior to transplantation; neither do we recommend abstaining from living donation. Special attendance needs to be given to those patients who had already experienced graft loss due to FSGS/SRNS recurrence. Early PE or IA with or without high‐dose CsA and/or rituximab seems to be most promising to induce remission. The educated statements presented here acknowledge that FSGS/SRNS recurrence after pediatric RTx remains a major concern and is associated with shorter graft survival or even graft loss. The value of any recommendation needs to take into account that evidence is based on cohorts that differ in ethnicity, pre‐transplant history, immunosuppressive regimen, definition of recurrence (eg, clinical and/or histological diagnosis) and treatment modalities of recurrence.
Hajdu‐Cheney syndrome (HJCYS) is a rare, autosomal dominant, skeletal disorder caused by mutations in the NOTCH2 signaling pathway for which genetic testing has recently become available. Renal ...abnormalities are associated in at least 10% of cases. We present an 8‐year‐old Caucasian boy, born with multiple dysmorphic features consistent with HJCYS. Imaging of the urinary tract revealed bilateral cystic dysplastic kidneys with associated vesicoureteral reflux. Renal function has been impaired since birth and deteriorated progressively to end‐stage renal disease (ESRD) by the age of two and a half years, when peritoneal dialysis was initiated and only recently renal transplantation was performed. Additional congenital abnormalities and multisystem involvement in HJCYS further complicated management, and he developed refractory anemia. Molecular diagnosis was confirmed by identification of a truncating mutation in exon 34 of NOTCH2. Although, renal abnormalities are considered an integral part of the HJCYS, published reports on ESRD are scarce. In those few published cases, where ESRD was recognized, renal failure developed either in late adolescence or adulthood. This is the first report of early ESRD occurring in a child. Patients with HJCYS may need chronic renal replacement therapy even in early childhood. The management of these children can be challenging given the multisystemic manifestations of HJCYS.
A central venous catheter (CVC) can either be inserted “de novo” or placed by guidewire exchange (GWE). From September 1998 to September 2015, 32 children (19 boys, 13 girls) were hemodialyzed in our ...unit by using a CVC. The mean age at CVC insertion was 12.6 ± 0.5 years. A total of 121 uncuffed catheters were placed, either “de novo” or by GWE in 64 (52.9%) and 57 (47.1%) cases, respectively. The most frequent cause for line revision was catheter dysfunction in 40/121 (33.1%) patients. The overall incidence of bacteremia was 1.5/1000 catheter‐days. The incidence in newly inserted and GWE catheters was 1.4 and 1.7/1000 catheter‐days, respectively. The difference did not reach statistical significance (P = 0.939). The infection rate correlated with patient age, and was higher in younger children (P = 0.006). GWE is an effective option of line revision, and did not influence the infection rate in our study.
Chronic Hemodialysis in Small Children Novljan, Gregor; Rus, Rina R; Premru, Vladimir ...
Therapeutic apheresis and dialysis,
06/2016, Letnik:
20, Številka:
3
Journal Article
Recenzirano
When peritoneal dialysis is inapplicable, chronic hemodialysis (HD) becomes the only available treatment option in small children. Due to small patient size, central venous catheters (CVC) are mainly ...used for vascular access. Over the past 4 years, four children weighing less than 15 kg received chronic HD in our unit. A total of 848 dialysis sessions were performed. Altogether, 21 catheters were inserted. In all but one occasion, uncuffed catheters were used. Catheter revision was performed 15 times during the study period, either due to infection or catheter malfunction. The median number of catheter revisions and the median line survival was 3.0/patient‐year and 53 days (range; 6–373 days), respectively. There were 14 episodes of catheter related infections requiring 11 CVC revisions (78.6%). The median rate of line infections was 2.8/patient‐year. Chronic HD in small children is demanding and labor intensive. Issues pertain mainly to CVCs and limit its long‐term use.
Renal transplantation is the optimal renal replacement therapy (RRT) in children, but some primary diseases can recur after transplantation, and recurrence accounts for a significant proportion of ...graft losses, being second only to acute rejection. The risk of disease recurrence is highest among patients with idiopathic focal segmental glomerulosclerosis (FSGS), presumably due to a circulating permeability factor. Less is clear about the genetic forms of FSGS, where the data regarding the frequency of recurrence are rather conflicting. We present a 12‐year‐old girl with rapidly progressive FSGS and end‐stage renal disease in her native kidneys associated with heterozygous mutations in NPHS1 and in NPHS2, suffering from early post‐transplant recurrence. On the basis of reviewed literature, and until further and more conclusive evidence considering pathogenicity is provided, we propose that FSGS patients with heterozygous mutations in NPHS1 or NPHS2 should be considered as having idiopathic FSGS, and post‐transplant recurrence should be anticipated.
Renal replacement treatment (RRT) is required in severe acute kidney injury, and a functioning central venous catheter (CVC) is crucial. Twenty‐eight children younger than 16 years have been treated ...at the University Medical Centre Ljubljana between 2003 and 2012 with either acute hemodialysis (HD) and/or plasma exchange (PE), and were included in our study. The age of the patients ranged from 2 days to 14.1 years. Sixty‐six CVCs were inserted (52% de novo, 48% guide wire). The sites of insertion were the jugular vein in 20% and the femoral vein in 80%. Catheters were in function from 1 day to 27 days. The most common cause for CVC removal or exchange was catheter dysfunction (50%). CVCs were mostly inserted in the femoral vein, which is the preferred site of insertion in acute HD/PE because of the smaller number of complications.
