A5061463245- Pediatric Urology and Nephrology Studies
- Urological Disorders and Treatments
- Renal and Vascular Pathologies
- Intestinal Malrotation and Obstruction Disorders
- Lysosomal Storage Disorders Research
- Congenital Anomalies and Fetal Surgery
- Congenital gastrointestinal and neural anomalies
- Intestinal and Peritoneal Adhesions
- Sphingolipid Metabolism and Signaling
- Ureteral procedures and complications
- Autophagy in Disease and Therapy
- Pancreatic function and diabetes
- Gastrointestinal disorders and treatments
- Abdominal Trauma and Injuries
- Lipid metabolism and biosynthesis
- Congenital Diaphragmatic Hernia Studies
- Childhood Cancer Survivors' Quality of Life
- Esophageal and GI Pathology
- Liver Diseases and Immunity
- Vascular Anomalies and Treatments
- Adrenal and Paraganglionic Tumors
- Abdominal vascular conditions and treatments
- Calcium signaling and nucleotide metabolism
- Appendicitis Diagnosis and Management
- Urinary and Genital Oncology Studies
University of Southern Denmark
2024-2025
Medical University of Graz
2021-2024
Novo Nordisk Foundation
2024
University of Copenhagen
2024
BioTechMed-Graz
2021
University of Zagreb
1989-1995
University Hospital Centre Zagreb
1975-1994
Universitätsklinik für Chirurgie Wien
1980
Marymount University
1975
Lysosomal acid lipase (LAL) is the key enzyme, which degrades neutral lipids at an acidic pH in lysosomes. The role of LAL various cellular processes has mostly been studied LAL-knockout mice, share phenotypical characteristics with humans suffering from deficiency. In vitro, cell-specific functions have commonly investigated by using inhibitors Lalistat-1 and Lalistat-2. We performed lipid hydrolase activity assays serine hydrolase-specific activity-based labeling combined quantitative...
Exercise improves metabolic health, enhances insulin sensitivity, and preserves muscle function, making it a core intervention to combat age-related decline disorders. However, large-scale genetic pharmacological screens uncover exercise-induced adaptations remain challenging in mammalian models due their complexity cost. Here, we present the ClimbMaster, fully automated, computer-controlled platform for assessing physical performance fruit fly Drosophila. The system uses repeated climbing...
Thioglycolate-elicited macrophages exhibit abundant conjugation of LC3 with PE (LC3-II). Among other autophagy-related (ATG) proteins, it is proposed that, like in yeast, both ATG5 and ATG7 are essential for conjugation. Using atg5-deficient (−/-) atg7−/-macrophages, we provide evidence that loss but not resulted LC3-II depletion. Accumulation elicited atg7−/- response to bafilomycin A1 validated these data. Furthermore, complete ATG3 demonstrated dispensable LC3–PE In contrast...
Lysosomal acid lipase (LAL) is the sole lysosomal enzyme responsible for degradation of cholesteryl esters and triacylglycerols at acidic pH. Impaired LAL activity leads to deficiency (LAL-D), a severe fatal disease characterized by ectopic lipid accumulation. Reduced also contributes development progression non-alcoholic fatty liver (NAFLD). To advance our understanding LAL-related pathologies, we performed comprehensive proteomic profiling livers from mice with systemic genetic loss...
To date, the only enzyme known to be responsible for hydrolysis of cholesteryl esters and triacylglycerols in lysosome at acidic pH is lysosomal acid lipase (LAL). Lipid malabsorption small intestine (SI), accompanied by macrophage infiltration, one most common pathological features LAL deficiency. However, exact role intestinal lipid metabolism still unknown. We collected three parts SI (duodenum, jejunum, ileum) from mice with a global (LAL KO) or intestine-specific deletion (iLAL...
Cholesterol and fatty acids are essential lipids that critical for membrane biosynthesis fetal organ development. Cholesteryl esters (CE) degraded by hormone-sensitive lipase (HSL) in the cytosol lysosomal acid (LAL) lysosome. Impaired LAL or HSL activity causes rare pathologies humans, with deficiency presenting less severe clinical manifestations. The infantile form of deficiency, a lipid storage disorder, leads to premature death. However, importance defective CE degradation its...
Cystitis cystica was found at cystoscopy in 157 children between the ages of 9 1/2 months and 14 years. Girls were affected much more often than boys, proportion being 153 to 4. Urinary infection, most commonly caused by E. coli, almost invariable. tract anomalies, particular vesico-ureteric reflux, frequently present. Histologically, cysts composed submucous aggregations lymphoid tissue. affecting ureteric orifices may contribute occurence reflux involvement bladder neck produce some degree...
Lysosomal acid lipase (LAL) is the only enzyme known to hydrolyze cholesteryl esters (CE) and triacylglycerols in lysosomes at an acidic pH. Despite importance of lysosomal hydrolysis skeletal muscle (SM), research this area limited. We hypothesized that LAL may play important role SM development, function, metabolism as a result lipid and/or carbohydrate disruptions. Mice with systemic deficiency (Lal-/-) had markedly lower mass, cross-sectional area, Feret diameter despite unchanged...
Metabolic dysfunction-associated steatotic liver disease (MASLD) is a prevalent pathology worldwide, closely associated with obesity and metabolic disorders. Increasing evidence suggests that macrophages play crucial role in the development of MASLD. Several human studies have shown an inverse correlation between circulating lysosomal acid lipase (LAL) activity LAL sole enzyme known to degrade cholesteryl esters (CE) triacylglycerols lysosomes. Consequently, these substrates accumulate when...
Advanced maternal age and obesity are the main risk factors to develop gestational diabetes mellitus (GDM). Obesity is a consequence of increased storage triacylglycerol (TG). Cytosolic lysosomal lipid hydrolases break down TG cholesteryl esters (CE) release fatty acids (FA), free cholesterol, glycerol. We have recently shown that intracellular lipases present active in mouse placenta deficiency acid lipase alters placental fetal homeostasis. To date, hydrolysis GDM has been poorly studied...
Two children (both females) aged 15 months and 4 years are described as very rare cases of central cyanosis in childhood being caused by a congenital pulmonary arteriovenous fistula. The initial diagnosis was made based on chest radiographs, with normal physical, ECG, radiological findings the heart. They had no family history Rendu-Weber-Osler syndrome. patients underwent cardiac catheterization angiography, where confirmed. After surgery, both were symptomfree, evidence disease.
This is the first report on technique and results of an antireflux ureterocystostomy in vault bladder. During last 3 years, a total 106 ureterocystostomies at vertex urinary bladder has been performed. In 93 cases without another preceding operation, only 2 (2-1%) recurrent refluxes were found, whereas 13 where operation had performed previously, 4 reflux seen, making 30% rate operative failure this particular group. all other end result was excellent; urine become negative culture...
In the Department of Pediatric Surgery, Clinical Hospital Center Rebro in Zagreb, during last 10 years a new method invasive diagnostic and at same time therapeutic procedure for treatment Wilms' tumor has been introduced. The is preoperative Percutaneous Transcatheter Intraarterial Embolization (PTIE) renal artey. aim this to reduce vascularization, decrease mass kidney affected by tumor, separate it from surrounding tissue, intraoperative spillage malignant cells into blood stream their...
Summary— A total of 618 children with primary vesicoureteric reflux (VUR) were examined preoperatively and 6, 9 24 months after an antireflux ureterocystostomy the vertex bladder (AUVB); 206 operated on bilaterally, so that 824 AUVBs performed. Most patients had grade 3 or 4 only 5% 2 reflux. Successful results noted in 792 ureteric units; 10 cases post‐operative stenosis 17 recurrent VUR a second AUVB was The following causes operative failure found: obstruction submucosal ureter tailoring...
To date, about 100 cases of a blind-ending branch bifid ureter have been reported. We present 13 clinically, radiologically and histologically evaluated congenital ureteric diverticulum. believe this anomaly is not as rare hitherto believed suggest the term diverticulum for ureter.
A case of an extremely rare type cloacal exstrophy in a male infant with normally developed subvesical part the urinary system and external genitalia but absent distal colon segment is presented. The patient also had omphalocele, upper tract anomalies sacrococcygeal teratoma.