Aim: To present a case of Sagliker syndrome (SS), a rare syndrome caused by longterm heavy tertiary hyperparathyroidism (HPT) in end-stage renal disease (ESRD). Case report: In the year 2000, the 38-year-old man was diagnosed with ESRD. He is currently of low height, paraplegic, pigeon and barrel chested, with elongated upper extremities, deformed fingers, mandibular and maxillary asymmetric deformities with teeth malformations. Due to extremely high serum parathormone (PTH), resistant to pharmacological treatment, subtotal parathyroidectomy (PTx) was performed in 2007. However, he underwent parathyroid resurgery in 2010 for persistently high PTH of more than 2500 pg/mL (upper normal limit 69). In 2012, imaging found two suspected parathyroid glands and one of them was surgically removed. Postoperatively, the expected decrease in calcium and PTH serum concentrations did not occur. Ten years after the diagnosis of ESRD, the patient began to notice more pronounced skeletal deformities (upper, lower jaw, extremities, deformities of fingers, kyphoscoliosis) along with depressive disorder. Laboratory findings still show extremely high PTH (1994 pg/mL), low calcium, 1.89 mmol/L (normal range 2.14–2.53), and high alkaline phosphatase, 837 U/L (normal range 60–120), despite continuous pharmacological treatment. Conclusion: SS was firstly recognized by Yahya Sagliker in 2004 and it has not been described in Croatia to date. Pervasiveness and knowledge of the syndrome is still poor. The most efficient way of treating/preventing SS is early total PTx in severe secondary HPT related to ESRD. However, it can only stop progress of the disease, but cannot return skeletal deformities.
Cilj: Istražiti učestalost infekcija mokraćnog sustava (IMS) u bolesnika s bubrežnim presatkom u prvoj poslijetransplantacijskoj godini te odnos učestalosti IMS-a prema terapiji imunosupresivima (IS) i funkciji bubrežnog presatka. Ispitanici i metode: Uključeno je 114 bolesnika (60,5 % muškaraca, medijan dobi svih bolesnika je 52 godine, u rasponu od 27 do 71). Podatci su preuzeti iz medicinskih zapisa i statistički obrađeni pomoću IBM SPSS Statistic, 25.0, uz razinu statističke značajnosti P < 0,05. Rezultati: Unutar godine dana od bubrežne presadbe (BP) u 67 od 114 ispitanika (58,8 %) zabilježeno je 170 epizoda IMS-a uzrokovanih s ukupno dvadeset jednim različitim uzročnikom. Kao najčešće kliničko očitovanje javljala se asimptomatska bakterijurija (44,1 %), a najčešće izolirani uzročnik IMS-a u svim kliničkim očitovanjima bio je E. coli (38,9 %). Najčešće se IMS javljao u prva tri mjeseca od BP-a (58,2 %), kada su bolesnici dio vremena imali Foleyev kateter i/ili JJ stent. IMS je bio značajno češći u žena i starijih, sve rjeđi s vremenom, a veća učestalost nije bila povezana s lošijom funkcijom presatka koncem prve poslijetransplantacijske godine. Ispitanici koji su u IS terapiji imali kortikosteroide, krajem prve poslijetransplantacijske godine imali su značajno veći broj epizoda IMS-a (P = 0,02). Zaključak: Više od polovice ispitanika imalo je u prvoj godini poslije BP-a neki oblik IMS-a, s najčešće izoliranim uzročnikom E. coli. Veća učestalost IMS-a zabilježena je u ženskom spolu, u starijih, uz veći IS (uz kortikosteroide) te u prva tri mjeseca od BP-a. Ispitanici s češćim IMS-om nisu imali lošiju funkciju bubrežnog presatka godinu dana nakon BP-a.
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