Incidence of renal biopsy complications in a 5-year follow up

Perkutana biopsija bubrega (PBB) predstavlja zlatni standard u dijagnostici parenhimskih bolesti bubrega. Rutinski se obavlja pod ultrazvučnom (UZV) kontrolom, a u kompliciranijim slučajevima pod kontrolom MSCT uređaja. Kako je riječ o invazivnom postupku nad izrazito prokrvljenim organom moguć je razvoj komplikacija koje dijelimo u male (bez potrebe za transfuzijom krvi ili intervencijom), velike (potrebna transfuzija krvi ili intervencija) i katastrofalne (gubitak funkcionalne bubrežne mase, potreba za nefrektomijom, smrtni ishod). Cilj ovog istraživanja retrospektivna je analiza komplikacija svih biopsija bubrega učinjenih u Zavodu za nefrologiju KBC Zagreb u razdoblju 2015.-2019. godine. Tijekom navedenog razdoblja, učinjena je ukupno 201 biopsija nativnih (173 pod UZV i 28 uz MSCT kontrolu) i 339 presađenih bubrega (321 pod UZV i 18 uz MSCT kontrolu). U skupini bolesnika s bioptiranim nativnim bubrezima do razvoja malih postbioptičkih komplikacija došlo je u 28,4 % pacijenata, a 3,5 % je razvilo velike komplikacije. U skupini bolesnika s bioptiranim transplantiranim bubrezima male komplikacije razvilo je 10,6 % pacijenata, a velike komplikacije 0,6 % bolesnika. Katastrofalnih komplikacija nije bilo. PBB pokazala se sigurnom dijagnostičkom metodom s malom vjerojatnošću komplikacija čija je učestalost bila u skladu s podacima iz drugih velikih centara.Percutaneous renal biopsy (PRB) represents a gold standard procedure in diagnostics of parenchymal kidney diseases. Real-time ultrasound (US) serves as usual guidance method, while multislice computed tomography (MSCT) is used in more complicated cases. As every invasive procedure, kidney biopsy also poses risk for development of complications which are usually classified as minor, major and chatastrophic. The aim of this retrospective study was to analyze incidence and type of complications of PRB performed from 2015 till 2019 in Department of nephrology UHC Zagreb. Study included 540 patients who underwent PRB , (201 patients with native kidney PRB and 339 with transplanted organ PRB), under the US (173 native , 321 transplanted kidney) or MSCT guidance (28 native and 18 transplanted kidney PRB). Complications were divided into minor (no need for clinical intervention or blood transfusions), major (medical interventions or blood transfusion required) and catastrophic (functional kidney mass loss, nephrectomy, death). In the group of patients with PRB of native kidneys 28,4 % developed minor while 3,5 % major complications. In the transplanted kidney PRB group,10,6 % of patients developed minor while 0,6 % major complications. There were no catastrophic complications in either group. The rate of complications in our center was in line with the results from other experienced centers. PRB is a safe procedure with low complication risk

Incidence of renal biopsy complications in a 5-year follow up

Perkutana biopsija bubrega (PBB) predstavlja zlatni standard u dijagnostici parenhimskih bolesti bubrega. Rutinski se obavlja pod ultrazvučnom (UZV) kontrolom, a u kompliciranijim slučajevima pod kontrolom MSCT uređaja. Kako je riječ o invazivnom postupku nad izrazito prokrvljenim organom moguć je razvoj komplikacija koje dijelimo u male (bez potrebe za transfuzijom krvi ili intervencijom), velike (potrebna transfuzija krvi ili intervencija) i katastrofalne (gubitak funkcionalne bubrežne mase, potreba za nefrektomijom, smrtni ishod). Cilj ovog istraživanja retrospektivna je analiza komplikacija svih biopsija bubrega učinjenih u Zavodu za nefrologiju KBC Zagreb u razdoblju 2015.-2019. godine. Tijekom navedenog razdoblja, učinjena je ukupno 201 biopsija nativnih (173 pod UZV i 28 uz MSCT kontrolu) i 339 presađenih bubrega (321 pod UZV i 18 uz MSCT kontrolu). U skupini bolesnika s bioptiranim nativnim bubrezima do razvoja malih postbioptičkih komplikacija došlo je u 28,4 % pacijenata, a 3,5 % je razvilo velike komplikacije. U skupini bolesnika s bioptiranim transplantiranim bubrezima male komplikacije razvilo je 10,6 % pacijenata, a velike komplikacije 0,6 % bolesnika. Katastrofalnih komplikacija nije bilo. PBB pokazala se sigurnom dijagnostičkom metodom s malom vjerojatnošću komplikacija čija je učestalost bila u skladu s podacima iz drugih velikih centara.Percutaneous renal biopsy (PRB) represents a gold standard procedure in diagnostics of parenchymal kidney diseases. Real-time ultrasound (US) serves as usual guidance method, while multislice computed tomography (MSCT) is used in more complicated cases. As every invasive procedure, kidney biopsy also poses risk for development of complications which are usually classified as minor, major and chatastrophic. The aim of this retrospective study was to analyze incidence and type of complications of PRB performed from 2015 till 2019 in Department of nephrology UHC Zagreb. Study included 540 patients who underwent PRB , (201 patients with native kidney PRB and 339 with transplanted organ PRB), under the US (173 native , 321 transplanted kidney) or MSCT guidance (28 native and 18 transplanted kidney PRB). Complications were divided into minor (no need for clinical intervention or blood transfusions), major (medical interventions or blood transfusion required) and catastrophic (functional kidney mass loss, nephrectomy, death). In the group of patients with PRB of native kidneys 28,4 % developed minor while 3,5 % major complications. In the transplanted kidney PRB group,10,6 % of patients developed minor while 0,6 % major complications. There were no catastrophic complications in either group. The rate of complications in our center was in line with the results from other experienced centers. PRB is a safe procedure with low complication risk

Acute renal failure due to severe rhabdomyolysis provoked by a mild covid-19 infection in patient with LCHAD deficency- a case report

Introduction: LCHAD (long-chain 3-hydroxy-acyl-CoA dehydrogenase) deficiency is an inherited fatty acid oxidation disorder in which the body is unable to break down certain fats resulting in hypoketotic hypoglycemia, myopathy, episodic rhabdomyolysis and neuropathy. Metabolic decompensation is often precipitated by infection or fasting. Case report: A 26-year-old patient was admitted to the emergency department because of generalized myalgias. This is a patient with known congenital deficiency of long-chain 3- hydroxy-acyl- CoA dehydrogenase (LCHAD). He was diagnosed at the age of 3 years and regularly undergoes check-ups in specialized metabolic department. 10 days prior to the symptoms he had milder form of covid-19 infection with a persistent dry cough. Previously, he was vaccinated with two doses of the mRNA SARS-COV 2 vaccine. Initially laboratory findings at emergency department showed elevated levels of creatine kinase (46000 U/L) with normal renal function (egfr: 105 ml/min/1.73m2). Chest X-ray excluded pneumonia. Abundant hydration with intravenous infusions (0.9% NaCl, 5% glucose) was started, but during the observation the patient developed oliguria with urine output <10 ml/hour. Further laboratory findings showed acute kidney injury with worsening rhabdomyolysis (CK>80,000 U/L, egfr: 19 ml/min/1.73m2, creatinine: 369 umol/L). Due to the need for hemodialysis, he was hospitalized in the intensive care unit where dialysis procedures (CVVHD, CVVHDF) were continuously performed for 7 days until gradual decrease in creatinine and CK levels. In continuation he was carefully hydrated with infusions of 10% glucose and received a specially adapted diet to ensure sufficient caloric intake and to prevent catabolism. In total, he was 12 days on continuous hemodialysis and the renal function completely recovered after 3 weeks with the normalization of creatinine and CK values. Beside mild SARS-CoV-2 infection, we haven’t founded any other cause of patient’s metabolic decompensation. Conclusion: Patients with LCHAD should be educated and controlled more often in the covid-19 pandemic, as even the mild form of SARS-CoV-2 infection can lead to a rapid metabolic decompensation and a possible fatal outcome

Hereditary fructose intolerance – two case reports and literature review

Nasljedna nepodnošljivost fruktoze ili fruktozemija je autosomno recesivno nasljedni poremećaj koji nastaje zbog nedostatne aktiv- nosti enzima fruktoza-1-fosfat aldolaze (aldolaze B). Bolest se klinički, u dotad naizgled zdravog dojenčeta, očituje nakon uvođenja namirnica koje sadrže fruktozu, saharozu ili sorbitol. Najčešći simptomi bolesti su povraćanje, bljedilo, drhtavica, somnolencija, a katkad i konvulzije (većinom kao posljedica hipoglikemije, odnosno neuroglikopenije). Može se razviti i akutno zatajenje jetre, a ako se dijete pravilno ne zbrine, takva kriza može završiti smrću. Pošto se fruktoza, saharoza i sorbitol isključe iz prehrane, simptomi bolesti se brzo i spontano povlače. Ako bolest ostane neprepoznata, dugoročno može uzrokovati ponavljajuće bolove u trbuhu, povraćanje, kroničnu bolest jetre, tubulopatiju, rahitis te zaostajanje u rastu i razvoju. Anamnestički podatci o pojavi simptoma nakon uvođenja voća i povrća u dohranu i/ili odbojnost prema slatkoj hrani ključni su za postavljanje sumnje na ovu bolest. Labo- ratorijski nalazi koji podupiru dijagnozu, a česti su u akutnom pogoršanju, su hipoglikemija, hipofosfatemija, hiperuricemija, meta- bolička acidoza i povišene aktivnosti aminotransferaza. Dijagnoza se potvrđuje nalazom bialelnih patogenih mutacija gena ALDOB ili mjerenjem aktivnosti enzima u tkivu jetre ili crijeva, što se danas rjeđe primjenjuje. Pravodobno postavljanje dijagnoze ključno je za dobar ishod. Ako se spriječi unos fruktoze u organizam, prognoza je izvrsna. Cilj ovog rada je predočiti klinička obilježja nasljedne nepodnošljivosti fruktoze prikazom dvoje bolesnika, jednog s akutnim, a drugog sa subakutnim kliničkim tijekom, te tako upozoriti na ovu rijetku, a lječivu nasljednu metaboličku bolest.Hereditary fructose intolerance or fructosemia is an inborn error of fructose metabolism caused by deficiency of the enzyme fructose- -1-phosphate aldolase. Individuals with this disorder are asymptomatic until fructose, sucrose, or sorbitol is introduced to the diet. First symptoms usually appear in infancy, after solid food containing fructose is started. Common acute presentation is nausea, vomiting, and symptoms of hypoglycaemia. In some infants, acute liver failure may occur. If the disease is unrecognized, chronic exposure to fructose may cause recurrent vomiting, episodes of abdominal pain, failure to thrive, chronic liver disease, proximal renal tubular dysfunction, and growth retardation. Many patients develop strong and protective aversion to sweet-tasting food. Labora- tory findings in acute crisis include hypoglycaemia, hypophosphataemia, hyperuricaemia, metabolic acidosis, and elevated liver enzymes. Complete elimination of fructose from the diet results in dramatic recovery. The diagnosis of hereditary fructose intoler- ance is confirmed by genetic testing. The cornerstone of treatment is exclusion of fructose, sucrose, and sorbitol from the diet, which results in complete alleviation of all signs and symptoms. The aim of this paper is to raise awareness of the spectrum of clinical symptoms in patients suffering from this rare but treatable metabolic disorder, by presenting two patients with different clinical course