Pregled bibliografske jedinice broj: 1253612
Xanthogranulomatous pyelonephritis in a 6 month old infant presented with nephrocalcinosis and afunctional left kidney
Xanthogranulomatous pyelonephritis in a 6 month old infant presented with nephrocalcinosis and afunctional left kidney // Pediatric Nephrology, 37 (2022), 11
Ljubljana, Slovenija, 2022. str. 2890-2890 doi:10.1007/s00467-022-05630-1 (poster, recenziran, sažetak, stručni)
CROSBI ID: 1253612 Za ispravke kontaktirajte CROSBI podršku putem web obrasca
Naslov
Xanthogranulomatous pyelonephritis in a 6 month
old infant presented with nephrocalcinosis and
afunctional left kidney
Autori
Matković, Hana ; Lamot, Lovro ; Kos, Ivanka ; Ban, Maja ; Davidović, Maša ; Jakopčić, Ivan ; Bulimbašić, Stela ; Luetić, Tomislav ; Vrljičak, Kristina
Vrsta, podvrsta i kategorija rada
Sažeci sa skupova, sažetak, stručni
Izvornik
Pediatric Nephrology, 37 (2022), 11
/ - , 2022, 2890-2890
Skup
54th ESPN Annual Meeting
Mjesto i datum
Ljubljana, Slovenija, 22.06.2022. - 25.06.2022
Vrsta sudjelovanja
Poster
Vrsta recenzije
Recenziran
Ključne riječi
xanthogranulomatous pyelonephritis ; differential diagnosis ; children ;
Sažetak
Introduction: Xanthogranulomatous pyelonephritis (XPN) is a rare presentation of severe chronic pyelonephritis characterised by renal parenchymal destruction by granulomatous tissue, inflammatory infiltration and fibrosis. The possible underlying mechanism involves urinary tract obstruction due to congenital anomalies, nephrolithiasis and recurrent urinary infection, resulting in unspecific symptoms such as abdominal pain, fever, abdominal mass, growth and weight retardation with persistent anemia, leucocytosis, elevated erythrocyte sedimentation rate (ESR), bacteriuria and pyuria. Therefore, the range of possible differential diagnosis is wide. Material and methods: Case report Results: The presented patient was born from an uneventful pregnancy and delivery. He first came to our attention at the age of 6 months due to acute cystopyelonefritis. Subsequently, left-sided hydroureteronephrosis (without vesicoureteral reflux), lymphadenopathy, severe anemia, elevated ESR and liver enzymes, as well as hypergammaglobulinemia and hypertriglyceridemia were noted. Other immune mediated, infectious, metabolic and malignant conditions were excluded by thorough diagnostic workup, including bone marrow analysis and whole exome sequencing. Abdominal ultrasound and computed tomography revealed unilateral nephrocalcinosis, and dynamic renal scintigraphy afunction of the left enlarged kidney. Despite antibiotic therapy, recurrent sterile pyuria with ongoing inflammation was present, necessitating a short course of glucocorticoids which led to gradual improvement of laboratory findings. Finally, left nephrectomy was performed with pathohistological finding suggestive of XPN. Conclusions: Regardless of diligent diagnostic workup, the precise mechanisms leading to XPN and intense immune reaction in our patient remains inconclusive. The literature data suggest that it might be a result of a macrophage defect in microbial processing, although no other signs of this process were present in the presented patient. Since this condition is very rarely present in children, especially infants, increased reporting of such challenging cases might increase the awareness, alleviate diagnostic dilemmas and steer the most appropriate treatment options.
Izvorni jezik
Engleski
Znanstvena područja
Kliničke medicinske znanosti
POVEZANOST RADA
Ustanove:
Medicinski fakultet, Zagreb,
Klinički bolnički centar Zagreb
Profili:
Kristina Vrljičak
(autor)
STELA BULIMBAŠIĆ
(autor)
Tomislav Luetić
(autor)
Maja Ban
(autor)
Lovro Lamot
(autor)
Citiraj ovu publikaciju:
Časopis indeksira:
- Current Contents Connect (CCC)
- Web of Science Core Collection (WoSCC)
- Science Citation Index Expanded (SCI-EXP)
- SCI-EXP, SSCI i/ili A&HCI
- Scopus
- MEDLINE