Mitochondria are involved in crucial homeostatic processes in the cell: the production of adenosine triphosphate and reactive oxygen species, and the release of pro-apoptotic molecules. Thus, cell ...survival depends on the maintenance of proper mitochondrial function by mitochondrial quality control. The most important mitochondrial quality control mechanisms are mitochondrial unfolded protein response, mitophagy, biogenesis, and fusion-fission dynamics. This review deals with mitochondrial quality control in heart diseases, especially myocardial infarction and heart failure. Some previous studies have demonstrated that the activation of mitochondrial quality control mechanisms may be beneficial for the heart, while others have shown that it may lead to heart damage. Our aim was to describe the mechanisms by which mitochondrial quality control contributes to heart protection or damage and to provide evidence that may resolve the seemingly contradictory results from the previous studies.
Pediatric cystic kidney disease (CyKD) includes conditions characterized by renal cysts. Despite extensive research in this field, there are no reliable genetics or other biomarkers to estimate the ...phenotypic consequences. Therefore, CyKD in children heavily relies on clinical and diagnostic testing to predict the long-term outcomes.
A retrospective study aimed to provide a concise overview of this condition and analyze real-life data from a single-center pediatric CyKD cohort followed during a 12-year period.
Medical records were reviewed for extensive clinical, laboratory, and radiological data, treatment approaches, and long-term outcomes.
During the study period, 112 patients received a diagnosis of pediatric CyKD. Male patients were more involved than female (1:0.93). Fifty-six patients had a multicystic dysplastic kidney; twenty-one of them had an autosomal dominant disorder; fifteen had an isolated renal cyst; ten had been diagnosed with autosomal recessive polycystic kidney disease; three had the tuberous sclerosis complex; two patients each had Bardet-Biedl, Joubert syndrome, and nephronophthisis; and one had been diagnosed with the trisomy 13 condition. Genetic testing was performed in 17.9% of the patients, revealing disease-causing mutations in three-quarters (75.0%) of the tested patients. The most commonly presenting symptoms were abdominal distension (21.4%), abdominal pain (15.2%), and oligohydramnios (12.5%). Recurrent urinary tract infections (UTI) were documented in one-quarter of the patients, while 20.5% of them developed hypertension during the long-term follow-up. Antibiotic prophylaxis and antihypertensive treatment were the most employed therapeutic modalities. Seventeen patients progressed to chronic kidney disease (CKD), with thirteen of them eventually reaching end-stage renal disease (ESRD). The time from the initial detection of cysts on an ultrasound (US) to the onset of CKD across the entire cohort was 59.0 (7.0-31124.0) months, whereas the duration from the detection of cysts on an US to the onset of ESRD across the whole cohort was 127.0 (33.0-141.0) months. The median follow-up duration in the cohort was 3.0 (1.0-7.0) years. The patients who progressed to ESRD had clinical symptoms at the time of initial clinical presentation.
This study is the first large cohort of patients reported from Croatia. The most common CyKD was the multicystic dysplastic kidney disease. The most common clinical presentation was abdominal distention, abdominal pain, and oliguria. The most common long-term complications were recurrent UTIs, hypertension, CKD, and ESRD.
Ozljede su u Hrvatskoj vodeći uzrok smrti djece nakon navršene prve godine života. Stoga je cilj ovog rada prikazati kretanje smrtnosti djece u dobi 0-19 godina zbog ukupnih ozljeda i ozljeda ...zadobivenih u prometu u Hrvatskoj u razdoblju od 1995. do 2014. godine te provesti standardizaciju koristeći se podatcima Eurostata za Europsku Standardnu Populaciju za 2013. godinu. U istraživanju su korišteni podatci Državnog zavoda za statistiku o vanjskim uzrocima smrti od ozljeda te podatci o procjeni broja stanovnika prema petogodišnjim dobnim skupinama i spolu. Šifriranje uzroka smrti prema Međunarodnoj
klasifi kaciji bolesti i srodnih zdravstvenih problema, 10. revizija (MKB-10), provedeno je u Hrvatskom zavodu za javno zdravstvo temeljem podataka iz liječničkih potvrda o smrti. Razdoblje od 1995. do 2014. podijeljeno je u četiri petogodišnja razdoblja. Sve statističke analize provedene su statističkim paketom Statistica, 12. verzija. Rezultati pokazuju statistički značajan pad smrtnosti zbog ukupnih ozljeda i prometnih ozljeda u dobnim skupinama 0-19: od 10,9 na 100.000 na 5,7 na 100.000 za dječake te od 5,2 na 100.000 na 2,1 na 100.000 za djevojčice u razdoblju od 1995. do 2014. godine. Iako je napredak postignut, potrebno je nastaviti provoditi postojeće programe izobrazbe djece i roditelja te programe prevencije nesreća i unaprjeđenja sigurnosti u prometu.
Mitochondria are involved in crucial homeostatic process-
es in the cell: the production of adenosine triphosphate
and reactive oxygen species, and the release of pro-apop-
totic molecules. Thus, ...cell survival depends on the mainte-
nance of proper mitochondrial function by mitochondrial
quality control. The most important mitochondrial quality
control mechanisms are mitochondrial unfolded protein
response, mitophagy, biogenesis, and fusion-fission dy-
namics. This review deals with mitochondrial quality con-
trol in heart diseases, especially myocardial infarction and
heart failure. Some previous studies have demonstrated
that the activation of mitochondrial quality control mech-
anisms may be beneficial for the heart, while others have
shown that it may lead to heart damage. Our aim was to
describe the mechanisms by which mitochondrial quality
control contributes to heart protection or damage and to
provide evidence that may resolve the seemingly contra-
dictory results from the previous studies.
PDF
