Okužbe sečil se pri novorojenčkih pojavljajo pogosteje kot pri starejših otrocih. V reviji Slovenska Pediatrija III/2023 je dr. Kavčič s sodelavkami objavila izsledke retrospektivne raziskave, v ...katero so vključili 274 novorojenčkov, ki so bili zaradi okužbe sečil hospitalizirani na Kliničnem oddelku za neonatologijo Pediatrične klinike med letoma 2013 in 2021. V prispevku so natančno navedeni splošni podatki o akutni okužbi sečil pri novorojenčku, podatki o kliničnih znakih, najpogostejših povzročiteljih in antibiotičnem zdravljenju ter podatki o deležu otrok s prirojenimi nepravilnostmi sečil in odpornostjo na antibiotike v slovenski skupini novorojenčkov v 8-letnem obdobju. Zato v tokratni posebni številki, ki je posvečena prispevkom 6. Slovenskega simpozija pediatrične nefrologije, vsebine ne ponavljamo.
Prenatalno diagnosticiranje in pravočasno odkrivanje razvojnih nepravilnosti sečil pri plodu sta ključni za preprečevanje nenadomestljivih zamud pri zdravljenju novorojenčkov in otrok s prirojenimi ...nepravilnostmi sečil, ki bi lahko vodile v slabšanje ali celo izgubo ledvičnega delovanja. Pri diagnosticiranju uporabljamo metode, ki nam dajo največ informacij in so hkrati najmanj škodljive za preiskovanca. Poznavanje osnovne embriologije sečil je ključnega pomena za prepoznavanje prirojenih nepravilnosti. V morfološkem prenatalnem diagnosticiranju sečil pri plodu uporabljamo ultrazvočno preiskavo (UZ) kot dobro standardizirano in uveljavljeno metodo. Če z UZ ne moremo razjasniti patoloških najdb ali če je potrebna nadaljnja razjasnitev nepravilnosti v predelu genitalno-urinarnega sistema, se odločimo za magnetnoresonančno slikanje (MRI) ploda
BACKGROUND.Improved management of growth impairment might have resulted in less growth retardation after pediatric kidney transplantation (KT) over time. We aimed to analyze recent longitudinal ...growth data after KT in comparison to previous eras, its determinants, and the association with transplant outcome in a large cohort of transplanted children using data from the European Society for Paediatric Nephrology/European Renal Association and European Dialysis and Transplant Association Registry.
METHODS.A total of 3492 patients transplanted before 18 years from 1990 to 2012 were included. Height SD scores (SDS) were calculated using recent national or European growth charts. We used generalized equation models to estimate the prevalence of growth deficit and linear mixed models to calculate adjusted mean height SDS.
RESULTS.Mean adjusted height post-KT was −1.77 SDS. Height SDS was within normal range in 55%, whereas 28% showed moderate, and 17% severe growth deficit. Girls were significantly shorter than boys, but catch-up growth by 5 years post-KT was observed in both boys and girls. Children <6 years were shortest at KT and showed the greatest increase in height, whereas there was no catch-up growth in children transplanted >12.
CONCLUSIONS.Catch-up growth post-KT remains limited, height SDS did not improve over time, resulting in short stature in nearly half of transplanted children in Europe.
Obstrukcija odtoka urina je pri otroku lahko posledica različnih vzrokov, ki ovirajo nemoteno odtekanje urina. Če obstrukcije ne razrešimo, lahko nastanejo zapleti ali celo ledvična okvara. Glede na ...raven obstrukcije razlikujemo obstrukcijo zgornjih sečil (obstrukcija pieloureternega spoja (PUS), obstrukcija vezikoureternega spoja (VUS), posledica ureterokele ali ektopičnega sečevoda) in obstrukcijo spodnjih sečil, kot je zaklopka zadnje sečnice (angl. posterior urethral valve, PUV). Pomembno je, da se za zdravljenje odločimo po posvetovanju specialistov različnih strok ter se nato pogovorimo tudi z otrokovimi starši in jim natančno predstavimo možnosti zdravljenja. Odločimo se za interventno radiološko ali kirurško zdravljenje, najpogosteje pa za kombinacijo obeh. Izidi so večinoma dobri, pri hujših prirojenih napakah pa napredovanje kronične ledvične bolezni z zdravljenjem zgolj odložimo. Po zdravljenju je potrebno redno spremljati ledvično funkcijo.
Growth and final height are of major concern in children with ESRD. This study sought to describe the distribution of adult height of patients who started renal replacement therapy (RRT) during ...childhood and to identify determinants of final height in a large cohort of RRT children.
A total of 1612 patients from 20 European countries who started RRT before 19 years of age and reached final height between 1990 and 2011 were included. Linear regression analyses were performed to calculate adjusted mean final height SD score (SDS) and to investigate its potential determinants.
The median final height SDS was -1.65 (median of 168 cm in boys and 155 cm in girls). Fifty-five percent of patients attained an adult height within the normal range. Adjusted for age at start of RRT and primary renal diseases, final height increased significantly over time from -2.06 SDS in children who reached adulthood in 1990-1995 to -1.33 SDS among those reaching adulthood in 2006-2011. Older age at start of RRT, more recent period of start of RRT, cumulative percentage time on a functioning graft, and greater height SDS at initiation of RRT were independently associated with a higher final height SDS. Patients with congenital anomalies of the kidney and urinary tract and metabolic disorders had a lower final height than those with other primary renal diseases.
Although final height remains suboptimal in children with ESRD, it has consistently improved over time.
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.
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