Personalized medicine – parenteral ketogenic diet in pyruvate dehydrogenase deficiency in the ICU; what do we need to know about it?

Manjak kompleksa piruvat dehidrogenaze (engl. Pyruvate Dehydrogenase Complex Deficiency, PDCD) rijedak je genetski neurometabolički poremećaj. Pripada u skupinu mitohondrijskih bolesti. Kliničke manifestacije se kreću od često smrtonosne, teške, novorođenačke laktacidoze do ozbiljnih neuroloških poremećaja kasnije tijekom života. Većina bolesnika ne doživi odraslu dob i rijetko je ova problematika nazočna u jedinicama intenzivnog liječenja odraslih bolesnika. Jedan od važnijih terapijskih postupaka kod ovih bolesnika je ketogena prehrana s visokim udjelom masti kojom se proizvode ketoni kao alternativno gorivo za tijelo i mozak. Ponekad može biti potrebna parenteralna ketogena prehrana koja još uvijek nije precizno definirana u postojećim smjernicama za prehranu bolesnika u jedinicama intenzivnog liječenja. Prikazano je provođenje parenteralne ketogene prehrane, te laboratorijski i klinički nadzor u 18-godišnje bolesnice s mitohondrijskom bolesti (PCDC) i to nakon abdominalnoga zahvata povezanog s upalnim komplikacijama, kada enteralna prehrana nije bila moguća. Prehrana je zahtijevala detaljni izračun osnovnih sastojaka kako bi se osigurala adekvatna opskrba energijom, volumenom i mikronutirijentima, a da bi se pri tome proizvela ketoza. Zajedno s ostalim metodama intenzivnoga liječenja, ovakav način prehrane pomogao je rješavanju komplikacija i ishodu liječenja ove epizode. U zaključku, pravilno propisana parenteralna ketogena prehrana kod bolesnika s PCDC smanjuje laktacidozu i učestalost neuroloških komplikacija. Treba je provoditi isključivo u jedinicama intenzivnoga liječenja, poglavito zbog nužnosti trajnog laboratorijskog i kliničkog nadzora. Kad god je moguće, treba ponovo što prije prijeći na enteralnu prehranu. Poželjno bi bilo imati i točno definirane smjernice za parenteralnu ketogenu prehranu.The pyruvate dehydrogenase complex deficiency (PDCD) is a rare genetic neurometabolic disorder. It belongs to the group of mitochondrial diseases. Clinical manifestations range from often fatal, severe, neonatal lactic acidosis to serious neurological disorders later in life. Most patients do not reach adulthood and are rarely present in intensive care units for adult patients. One of the most important therapeutic procedures in these patients is a ketogenic high-fat diet that produces ketones as an alternative fuel for the body and brain. Sometimes a parenteral ketogenic diet may be required; however, it has not been yet precisely defined in existing nutrition guidelines for intensive care patients. Here we described the implementation of parenteral ketogenic diet, along with the basics of laboratory and clinical monitoring in the 18-year old patient with mitochondrial disease (PCDC) after abdominal surgery associated with inflammatory complications, while enteral intake was not possible. It required a detailed calculation of the basic ingredients of the diet to ensure an adequate supply of energy, volume and micronutrients, while producing ketosis. Together with other intensive care methods, it helped resolve the complications and treatment outcome of this episode. In conclusion, properly prescribed and implemented parenteral ketogenic diet in patients with PCDC reduces the incidence of lactic acidosis and neurological complications. It should be performed exclusively in intensive care units, mainly due to continuous laboratory and clinical monitoring. Whenever possible, enteral nutrition should be resumed as soon as possible. It would also be desirable to have well-defined guidelines for parenteral ketogenic nutrition

Clostridial gas gangrene after laparoscopic cholecystectomy: the role of SARS-CoV-2 virus – a case report and review of literature

Klostridijska plinska gangrena je brzo šireća nekrotična infekcija mekog tkiva s visokim smrtnim ishodom. Rijetka je komplikacija laparoskopske kolecistektomije. Predstavljamo 68-godišnju bolesnicu koja je razvila klostridijsku plinsku gangrenu nakon laparoskopske kolecistektomije. Bolesnica je prethodno bila bez komorbiditeta osim što je 14 dana ranije imala SARS CoV-2 infekciju s blagim respiratornim simptomima. Klostridijska infekcija je nastala neposredno nakon operacije uzrokujući gangrenu trbušne stijenke, septički šok i multiorgansko zatajenje. Unatoč pravovaljanoj i pravodobnoj antibiotskoj terapiji, kirurškom debridementu i ostalim mjerama intenzivnog liječenja, ishod bolesti bio je letalan. Postavljamo si dva pitanja: je li virus SARS CoV-2 mogao biti inicijator letalne kaskade u ove bolesnice i bismo li promijenili ishod da smo odgodili operaciju?Clostridium gas gangrene is fast-spreading necrotic infection of soft tissue relevant to high mortality rates. It is a rare complication after laparoscopic cholecystectomy. We present a case of a 68-year old woman who developed clostridial gas gangrene after laparoscopic cholecystectomy. The patient didn’t have have any comorbidity except SARS CoV-2 infection with mild respiratory symptoms 14 days ago. The infection developed immediately after the operation causing gangrene of the abdominal wall, septic shock and multi-organ failure. Although the patient was treated with appropriate and prompt antibiotic therapy and thorough surgical debridement and with other supportive measures, outcome was lethal. Based on this case we ask ourselves: Could the SARS CoV-2 virus initiate lethal cascade and should we change the outcome if we postponed the operation

Compassionate mesenchymal stem cell treatment in a severe COVID-19 patient: a case report

COVID-19 presentations range from cold-like symptoms to severe symptoms with the development of acute respiratory distress syndrome (ARDS). We report on a severe COVID-19 patient who was mechanically ventilated and who developed ARDS and bacterial infection. Because of rapid clinical deterioration and the exhaustion of other treatment options, the family and attending physicians requested a compassionate use of adult allogeneic bone marrow-derived mesenchymal stem cells (MSC) in addition to commonly used immunosuppressive, antiviral, and supportive therapy. The clinical course is discussed thoroughly, with a special emphasis on the safety and effect of MSC therapy. Compassionate MSC treatment, given in three rounds, affected ARDS regression. The patient was discharged from the intensive care unit after 31 days and from hospital after 49 days in a good general condition. MSC treatment was not associated with any side effects and was well tolerated in a three-week period; therefore, it should be studied in larger trials and considered for compassionate us

Klostridijska plinska gangrena nakon laparoskopske kolecistektomije: uloga SARS-CoV-2 virusa – prikaz bolesnice s pregledom literature

Klostridijska plinska gangrena je brzo šireća nekrotična infekcija mekog tkiva s visokim smrtnim ishodom. Rijetka je komplikacija laparoskopske kolecistektomije. Predstavljamo 68-godišnju bolesnicu koja je razvila klostridijsku plinsku gangrenu nakon laparoskopske kolecistektomije. Bolesnica je prethodno bila bez komorbiditeta osim što je 14 dana ranije imala SARS CoV-2 infekciju s blagim respiratornim simptomima. Klostridijska infekcija je nastala neposredno nakon operacije uzrokujući gangrenu trbušne stijenke, septički šok i multiorgansko zatajenje. Unatoč pravovaljanoj i pravodobnoj antibiotskoj terapiji, kirurškom debridementu i ostalim mjerama intenzivnog liječenja, ishod bolesti bio je letalan. Postavljamo si dva pitanja: je li virus SARS CoV-2 mogao biti inicijator letalne kaskade u ove bolesnice i bismo li promijenili ishod da smo odgodili operaciju