| Abstract (croatian) | Cilj: Prikazati pacijenta s porodičnom hiperkolesterolemijom (engl. familial hypercholesterolemia; FH) i panhipopituitarizmom kod kojeg je unatoč trojnoj hipolipemijskoj terapiji ciljna vrijednost LDL kolesterola gotovo postignuta tek nakon uvođenja nadomjesne terapije somatotropinom koja inače nije neophodna za odrasle osobe s panhipopituitarizmom. Prikaz slučaja: 54-godišnji pacijent upućen je na endokrinološku evalvaciju radi hipogonadizma i hiperlipidemije. Pacijentu je 2005. godine učinjena perkutana koronarna intervencija s postavljanjem stenta u desnu koronarnu arteriju te je 2016. i 2017. godine zbog ateroskleroze provedeno endovaskularno liječenje stenoza femoralnih arterija. Pacijent boluje od arterijske hipertenzije, dislipidemije, šećerne bolesti tipa 2 te debljine 3. stupnja (ITM 43,5 kg/m2). Obradom erektilne disfunkcije i poliurije 2018. godine ustanovljen je panhipopituitarizam. Pacijent je redovito uzimao preporučenu nadomjesnu hormonsku terapiju (hidrokortizon, levotiroksin, dezmopresin, testosteron undekanoat) uz kroničnu terapiju (metformin, fenofibrat, atorvastatin, ezetimib, klopidogrel, nebivolol, lerkanidipin). Unatoč maksimalnoj dozi visokopotentnog statina i ezetimiba utvrđena je izrazito povišena razina LDL kolesterola (8,0 mmol/L), ukupnog kolesterola (10,5 mmol/L), triglicerida (3,8 mmol/L) te snižena vrijednost HDL kolesterola (0,6 mmol/L). S obzirom na anamnestičke podatke i laboratorijske nalaze te prema bodovnom sustavu Duch Lipid Clinical Network koji je iznosio 12, vrlo je vjerojatna dijagnoza porodične hiperkolesterolemije te je, uz dosadašnju hipolipemijsku terapiju, preporučeno liječenje PCSK-9 inhibitorom. Tri mjeseca nakon uvođenja PCSK9i u terapiju značajno su se reducirali LDL kolesterol (2,3), ukupni kolesterol (4,0) i trigliceridi (1,8) te porasle vrijednosti HDL-a (0,9). S obzirom na pretpostavljeni povoljan učinak nadomjesne terapije hormonom rasta, preporučen je somatotropin kojega je u daljnjem tijeku liječenja pacijent, uz dosadašnju terapiju, uzimao te se na sljedećem kontrolnom pregledu vrijednost LDL-a dodatno reducirala (1,6), uz povoljan učinak i na ostale lipidne parametre (ukupni kolesterol 3,5, trigliceridi 2,5, HDL 0,8, ne-HDL 2,7). Zaključak: Istraživanja koja se bave odnosom nadomjesne terapije hormonom rasta i dislipidemije, malobrojna su te se u liječenju dislipidemije preporučuje trojna hipolipemijska terapija. U našega pacijenta, koji boluje od FH i panhipopituitarizma, primjenom kombinacije statina, ezetimiba, PCSK9i te somatotropina gotovo su postignute ciljne vrijednosti LDL-a, što može sugerirati da su potrebna dodatna istraživanja u ovom području kako bi se definirale terapijske smjernice. |
| Abstract (english) | Aim: To present a patient with FH (familial hypercholesterolemia) and panhypopituitarism in whom, despite triple hypolipidemic therapy, the target LDL cholesterol was approximately reached only after the introduction of somatotropin replacement therapy, which is not otherwise necessary for adults with panhypopituitarism. Case report: A 54-year-old patient was referred for endocrinological evaluation at the Rijeka Clinical Hospital. In 2005, the patient underwent percutaneous coronary intervention with stent placement in the right coronary artery, endovascular treatment of femoral artery stenosis, he also suffers from arterial hypertension, dyslipidemia, type 2 diabetes and grade 3 obesity (BMI 43.5 kg/m2). In the treatment of erectile dysfunction and polyuria in 2018, panhypopituitarism was established. The patient regularly took the recommended hormone replacement therapy (hydrocortisone, levothyroxine, desmopressin, testosterone undecanoate) in addition to chronic therapy (metformin, fenofibrate, atorvastatin, ezetimibe, clopidogrel, nebivolol, lercanidipine). Despite the maximum dose of high-potency statin and ezetimibe, markedly elevated levels of LDL cholesterol (8.0 mmol/L), total cholesterol (10.5 mmol/L), triglycerides (3.8 mmol/L) and decreased HDL cholesterol (0.6 mmol/L) were found. Given the anamnestic data, laboratory findings and the Duch Lipid Clinical Network scoring system, which was 12, the diagnosis of familial hypercholesterolemia was very likely and, in addition to current hypolipemia therapy, the treatment with PCSK-9 inhibitor was recommended. Three months after the introduction of PCSK9i into therapy, LDL cholesterol (2.3), total cholesterol (4.0) and triglycerides (1.8) were significantly reduced, and HDL values (0.9) increased. Given the presumed beneficial effect of growth hormone replacement therapy, somatotropin was recommended, which the patient took in the course of further treatment, and the LDL value was further reduced at the next follow-up examination (1.6), with a beneficial effect on other lipid parameters. (total cholesterol 3.5, triglycerides 2.5, HDL 0.8, non-HDL 2.7). Conclusion: There are few studies that deal with the relationship between growth hormone replacement therapy and dyslipidemia, and triple hypolipidemic therapy is recommended in the treatment of dyslipidemia. In our patient, who has FH and panhypopituitarism, the use of a combination of statins, ezetimibe, PCSK9i and somatotropin has almost reached the target values of LDL, which may suggest that further research is needed in this area to define therapeutic guidelines. |
