Kalkvakaóhóf og árangur skurðaðgerða á Borgarspítalanum 1985-1989

Neðst á síðunni er hægt að nálgast greinina í heild sinni með því að smella á hlekkinn View/OpenWe did a clinical and pathological study of 34 patients (27 females and 7 males) operated on for primary hyperparathyroidism during a 5 year period 1985-1989 at the Reykjavik City Hospital. Of these 28 patients became normocalcemic immediately after operation, 2 patients needed medical- treatment for hypoparathyroidism for several months. Four patients had a persistent hypercalcemia, two of those were successfully reoperated on after localization of a parathyroid adenoma with the help of selective venous sampling and angiography in one case and thallium-technetium subtraction scintigraphy in the other. Two patients relapsed but they have only mild and symptomfree hypercalcemia. Two patients still have slight persistent hypercalcemia. Two patients suffered transient unilateral vocal cord paralysis. No other complications were noticed. The operative success rate is 88.2%. Histological diagnosis was confirmed in 31 patients, 29 patients (93.5%) had adenoma, two patients (6.5%) had hyperplasia. There was one case of double adenoma. Two patients had oxyphil cell adenoma. In one case of adenoma there was a previous history of radioiodine treatment for hyperthyroidism. One patient had MEA-I and parathyroid adenoma. Both hyperplasia cases were of the chief cell type. During the 5-year period 1985-1989 a total of 64 patients (54 females, 10 males) were operated on in Iceland and had histologically proven hyperparathyroidism, 55 patients (86%) had adenoma, 9 patients (14%) had hyperplasia. This corresponds to 5.2 operations per 100.000 inhabitants per year. No parathyroid carcinoma was diagnosed during the study period. In conclusion, as we find in our material negligible operative complications and a high surgical success rate, we recommend a surgical treatment of all hyperparathyroid patients except in cases where contraindications for surgery are to be found.Til að kanna árangur skurðlæknismeðferðar við kalkvakaóhófi (hyperparathyroidismus) á skurðdeild Borgarspítalans 1985-1989 voru kannaðar sjúkraskrár þeirra 34 sjúklinga, sem fengu þessa greiningu á tímabilinu. Kynjahlutfall var einn karl á móti fjórum konum. Meðalaldur var 61 ár (frá 26-86 ára). Geðrænar truflanir voru algengar (62%). Sjö af 10 nýrnasteinasjúklingum höfðu langa sögu um nýrnasteina, (meðaltal 8.5 ár) og eðlilegar kalkmælingar á þeim tíma. Hvorki fannst samband milli sermiskalks og stærðar æxlis, né heldur kalkvaka og stærðar æxlis. Veikt en tölfræðilega marktækt (r=0.397, p=0.022) samband fannst milli sermiskalks og kalkvaka í sermi. Fundvísi ómskoðunar á æxli á hálsi var aðeins 21%. Eftir aðgerð fengu 28 sjúklingar strax eðlilegt sermiskalk. Tveir þörfnuðust lyfjameðferðar um hríð vegna of lags kalks en jöfnuðu sig. Fjórir sjúklingar höfðu áfram sermiskalkshækkun, en tveir þeirra fengu bata eftir endurtekna aðgerð. Tveir sjúklingar hafa áfram mjög væga hækkun á sermiskalki og tveir hafa fengið vægt bakslag en allir fjórir eru einkennalausir. Tveir sjúklingar fengu tírrtabundna raddbandalömun öðrum megin en engir aðrir aukakvillar komu fram hjá hópnum. Settu marki var náð með skurðaðgerð hjá 88% sjúklinganna án nokkurra varanlegra fylgikvilla. Kalkvakaóhóf var staðfest með vefjagreiningu hjá 31 (25 konum, sex körlum) af 34 sjúklingum Borgarspítalans. Af þeim höfðu 29 (93.5%) góðkynja kirtilæxli (adenoma) og tveir (6.5%) vefjaauka (hyperplasia)

NAC blocks Cystatin C amyloid complex aggregation in a cell system and in skin of HCCAA patients

Funding Information: We thank all the patients involved in this study for their participation. Funding for this work was provided by an Institutional Development Fund to the Center for Applied Genomics from Children’s Hospital of Philadelphia and a sponsored Research agreement with Artic Therapeutics LLC. Funding was provided to Dr. Gutierrez-Uzquiza from Autonomous Community of Madrid (CAM). Spain. “2017-T1/BMD-5468” 2018-2020.-IP: Alvaro Gutierrez Uzquiza. Publisher Copyright: © 2021, The Author(s).Hereditary cystatin C amyloid angiopathy is a dominantly inherited disease caused by a leucine to glutamine variant of human cystatin C (hCC). L68Q-hCC forms amyloid deposits in brain arteries associated with micro-infarcts, leading ultimately to paralysis, dementia and death in young adults. To evaluate the ability of molecules to interfere with aggregation of hCC while informing about cellular toxicity, we generated cells that produce and secrete WT and L68Q-hCC and have detected high-molecular weight complexes formed from the mutant protein. Incubations of either lysate or supernatant containing L68Q-hCC with reducing agents glutathione or N-acetyl-cysteine (NAC) breaks oligomers into monomers. Six L68Q-hCC carriers taking NAC had skin biopsies obtained to determine if hCC deposits were reduced following NAC treatment. Remarkably, ~50–90% reduction of L68Q-hCC staining was observed in five of the treated carriers suggesting that L68Q-hCC is a clinical target for reducing agents.Peer reviewe