Rare kidney and congenital urogenital defects competence centre
Who are we?
The Competence Center for Rare Kidney Diseases and Congenital Anomalies of Kidney and Urinary Tract (CAKUT) was established in 2015 as an effort to unite healthcare professionals with experience in rare kidney diseases and CAKUT and to improve the care delivered to patients affected by these rare conditions.
The multidisciplinary team of the Center consists of highly specialized healthcare professionals with longstanding experience in managing patients with rare kidney disorders: pediatric nephrologists, pediatric urologists, adult nephrologists. Due to the complete coverage of all pediatric and adult subspecialties by the hospital the team can be extended as needed. Close collaboration with adult nephrologists ensures proper transition of pediatric patients to the adult care.
The Center provides healthcare services to the largest number of pediatric patients with rare kidney diseases and CAKUT in Lithuania.
Information about provided services
The dedicated team of the Center provides care to patients suffering from most of the rare kidney and urological disorders:
- Hereditary and immune glomerulopathies (idiopathic and congenital nephrotic syndrome, Alport syndrome, etc.);
- Ciliopathies (ARPKD, ADPKD, juvenille nephronoftysis, etc.);
- Phakomatoses with renal involvement (tuberous sclerosis, von-Hippel Lindau);
- Tubulopathies (renal tubular acidosis, electrolyte disorders, etc.);
- Thrombotic microangiopathies (HUS, aHUS, TTP);
- Metabolic and stone forming disorders (cystinosis, hyperoxaluria, etc.);
- CAKUT (renal hypo/dysplasia, obstructive nephropathies, etc.);
- Pediatric CKD, dialysis and renal transplantation;
- Renovascular hypertension.
Every year, more than 2000 children with kidney diseases and more than 6000 children with urogenital diseases are managed in Children Hospital of VUHSK. As the spectrum of rare kidney and urogenital diseases is heterogenous and covers a lot of different entities (including those with systemic involvement), some of the required diagnostic and therapeutic services are provided in collaboration with other centers of VUHSK, National Pathology Center and international laboratories. Moreover, multidisciplinary care is delivered to the patients in cooperation with other healthcare specialists of VUHSK (dietitians, psychologists, endocrinologists, rheumatologists, gastroenterologists, ophthalmologists, surgeons, etc.).
VUHSK offers a wide spectrum of diagnostic and treatment services. These include modern genetic diagnostics, advanced radiological investigations, immunological testing, dialysis (hemo- and peritoneal), CRRT, plasma exchange, immunoadsorption, pediatric renal transplantation (incl. ABO incompatible and highly sensitized patients), pediatric liver transplantation, state-of-the-art pharmacological therapies (rituximab, eculizumab), etc. Gaps in diagnostic or treatment services are constantly improved by implementing new technologies/treatments or collaboration with external (international) centers.
The services provided in the Center are summarized in the following table:
Team members are in close scientific and expertise-sharing collaboration with various international hospitals and research institutions. In cooperation with international colleagues, healthcare professionals of the Center are performing research studies and clinical trials aiming to improve the care of children with rare kidney diseases. Our pediatric nephrologists are longstanding members of the ESCAPE consortium – biggest pediatric nephrology research consortium in Europe (established by Heidelberg University Hospital, Germany) – and participate in numerous studies initiated by the consortium.
The current international network of the Center includes healthcare professionals and researchers from the following international institutions: Heidelberg University Hospital (Germany), Cologne University Hospital (Germany), Great Ormond Street Hospital (London, UK), Columbia University (New York, USA), University of Bologna (Italy), Manchester University (UK) and Cincinnati Children‘s Hospital (USA).
The Center is formed of a multidisciplinary team consisting of 3 pediatric nephrologists, 2 pediatric urologists, adult nephrologists and resident doctors that serve as administrators of the center. All healthcare professionals working in the center have a 10+ years experience in managing patients with various rare kidney diseases and CAKUT.
Composition of the core team:
Prof. Augustina Jankauskienė – pediatric nephrologist, head of the Center;
Prof. Rimantė Čerkauskienė – pediatric nephrologist;
Assoc. prof. Gilvydas Verkauskas – pediatric urologist;
Dr. Vytautas Bilius – pediatric urologist;
Renata Vitkevič – pediatric nephrologist;
Karolis Ažukaitis – pediatric nephrology fellow and PhD student, administrator of the Center.
In order to ensure appropriate multidisciplinary care and proper patient transition the team is in close cooperation with the adult subunit lead by adult nephrologist prof. Marius Miglinas.
The center has a dedicated extended team of consultants experienced in the diagnostics of rare kidney disorders:
Prof. Arvydas Laurinavičius – nephropathologist;
Dr. Donatas Vajauskas – radiologist (nuclear medicine);
Dr. Andrius Čekuolis – ultrasound specialist.
The consultant team can be extended as needed and may include: dietitians, psychologists, endocrinologists, rheumatologists, gastroenterologists, ophtalmologists, surgeons, ENT doctors and other.
Head of the Center
Prof. Augustina Jankauskienė
Research is one of the major priorities in the Center and the team currently includes 5 PhD`s also giving lectures for pediatric nephrology and urology resident doctors. In total, the team members of the Center have published more than 70 research articles in international peer-reviewed journals and more than 100 research papers in other Lithuanian and international journals, they also co-authored 4 textbooks. Team members regularly organize teaching courses and conferences dedicated to the topics of pediatric nephrology and urology.
VUH SK pediatric nephrologists have long-term experience in participating in rare kidney disease research and participate in numerous international and national research activities along with initiating local research projects and regularly commencing clinical trials for rare kidney diseases. Members of the multidisciplinary team are national or regional coordinators of several research studies for rare kidney disease.
Ongoing research studies:
- PodoNet – web-based clinical database for primary steroid resistant (SRNS) and congenital nephrotic syndrome (CNS). www.podonet.org
- PREDICT – Antibiotic Prophylaxis and Renal Damage In Congenital Abnormalities of the Kidney and Urinary Tract (PREDICT); (prof. A. Jankauskienė – national coordinator);
- Cardiovascular Comorbidity in Children with Chronic Kidney Disease (4C Study); (K. Ažukaitis – regional coordinator). www.4c-study.org
- International Registry of Autosomal Polycystic Recessive Kidney Disease (ARegPKD) (A. Jankauskienė – regional coordinator). www.aregpkd.org
- ESPN/ERA-EDTA European Registry for Pediatric Nephrology. https://www.espn-reg.org/
- International Pediatric Peritoneal Biopsy Study in Children
- „Prevalence, clinical course and prognosis of HUS in Lithuanian chilren“
- International Registry of Autosomal Dominant Polycystic Kidney Diseases (ADPedKD). www.adpedkd.org
- Genomics of IgA-related disorders in kids Study (GIGA-KIDS). gigakids.org
- Calcium balance study in pediatric dialysis patients (initiated by Great Ormond Street Hospital)
- European Registry of Dialysis Treatment of Pediatric Acute Kidney Injury (EurAKID)
Since 2004 team members are regularly commencing industry sponsored clinical trials. Three clinical trials for pediatric rare kidney diseases are currently ongoing.
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