Autonomic and sensory nerve dysfunction in primary biliary cirrhosis

AIM: Cardiovascular autonomic and peripheral sensory neuropathy is a known complication of chronic alcoholic and non-alcoholic liver diseases. We aimed to assess the prevalence and risk factors for peripheral sensory nerve and autonomic dysfunction using sensitive methods in patients with primary biliary cirrhosis (PBC). METHODS: Twenty-four AMA M2 positive female patients with clinical, biochemical and histological evidence of PBC and 20 age matched healthy female subjects were studied. Five standard cardiovascular reflex tests and 24-h heart rate variability (HRV) analysis were performed to define autonomic function. Peripheral sensory nerve function on median and peroneal nerves was characterized by current perception threshold (CPT), measured by a neuroselective diagnostic stimulator (Neurotron, Baltimore, MD). RESULTS: Fourteen of 24 patients (58%) had at least one abnormal cardiovascular reflex test and thirteen (54%) had peripheral sensory neuropathy. Lower heart rate response to deep breathing (P = 0.001), standing (P = 0.03) and Valsalva manoeuvre (P = 0.01), and more profound decrease of blood pressure after standing (P = 0.03) was found in PBC patients than in controls. As a novel finding we proved that both time domain and frequency domain parameters of 24-h HRV were significantly reduced in PBC patients compared to controls. Each patient had at least one abnormal parameter of HRV. Lower CPT values indicated hyperaesthesia as a characteristic feature at peroneal nerve testing at three frequencies (2000 Hz: P = 0.005; 250 Hz: P = 0.002; 5 Hz: P = 0.004) in PBC compared to controls. Correlation of autonomic dysfunction with the severity and duration of the disease was observed. Lower total power of HRV correlated with lower CPT values at median nerve testing at 250 Hz (P = 0.0001) and at 5 Hz (P = 0.002), as well as with those at peroneal nerve testing at 2000 Hz (P = 0.01). CONCLUSION: Autonomic and sensory nerve dysfunctions are frequent in PBC. Twenty-four-hour HRV analysis is more sensitive than standard cardiovascular tests for detecting of both parasympathetic and sympathetic impairments. Our novel data suggest that hyperaesthesia is a characteristic feature of peripheral sensory neuropathy and might contribute to itching in PBC. Autonomic dysfunction is related to the duration and severity of PBC

Relabáló/refrakter Hodgkin-lymphoma brentuximab vedotin kezelése. Hazai tapasztalatok

Introduction: The treatment of relapsed or refractory Hodgkin lymphoma is still a major therapeutic challenge. The use of brentuximab vedotin, an anti-CD30 antibody-drug conjugate, represents a promising approach for these patients, however clinical outcomes have not yet been evaluated in Hungary. Aim: Our aim was to assess the efficacy, safety and outcome of brentuximab vedotin treatment in Hungarian Hodgkin lymphoma patients. Method: In this retrospective case note review we enrolled patients at 6 clinical sites countrywide who were diagnosed with Hodgkin lymphoma and received brentuximab vedotin between 1 January 2013 and 31 December 2016. Results: A total of 86 patients were treated with brentuximab vedotin during the examined period. Before therapy initiation 66% of our patients had advanced-stage disease. Overall response rate to brentuximab vedotin, administered before autologous hematopoietic stem cell transplantation (n = 54) was 66.6%, complete remission rate was 42.6%. Thirty patients received brentuximab vedotin after AHSCT, 46.67% responded to treatment, 30% achieved complete remission. Thirty-six patients received the drug as a single-agent therapy, 50 patients were given brentuximab vedotin in combination, 39 of them with bendamustin. Toxicity was observed only in 13.95% of our patients, most common symptom was skin rash. Based on our analysis the estimated 5-year overall survival rate was 78.7%, the estimated progression free survival rate was 23.59 months (95% CI: 19.50-27.68). Conclusion: Brentuximab vedotin carries a substantial improvement in the treatment of relapsed or refractory Hodgkin lymphoma. Our results underline prior observations published in the literature. The use of brentuximab vedotin in combination can be beneficial, however further investigation is needed on the subject

Relabáló/refrakter Hodgkin-lymphoma brentuximab vedotin kezelése. Hazai tapasztalatok | Brentuximab vedotin treatment in patients with relapsed or refractory Hodgkin lymphoma. A Hungarian retrospective study

Absztrakt: Bevezetés: A relabáló és refrakter Hodgkin-lymphoma kezelése továbbra is nagy kihívást jelent. Hatalmas előrelépést jelentett a brentuximab vedotin alkalmazása, amellyel jelenleg már jelentős hazai tapasztalatok is vannak. Célkitűzés: A brentuximab vedotinnal kezelt magyar Hodgkin-lymphomás betegek adatainak, a kezelés hatékonyságának elemzése. Módszer: Hat hazai hematológiai osztályon 2013. január 1. és 2016. december 31. között brentuximab vedotinnal kezelt Hodgkin-lymphomás betegek adatainak retrospektív elemzése. Eredmények: Összesen 86 beteg részesült brentuximab vedotin kezelésben. A kezelés előtt a betegek egyharmada korai, kétharmada előrehaladott stádiumban volt. Autológ őssejt-transzplantáció előtt alkalmazva 54 betegnél a teljes válaszarány 66,6%, ebből komplett remissziót a betegek 42,6%-a ért el. Autológ őssejt-transzplantációt követően 30 beteg kapta, a teljes válaszarány 46,67%, a komplett remisszió 30% volt. Harminchat beteg csak monoterápiában kapta a készítményt, míg 50 beteg kombinációban, ebből 39 esetben bendamustinnal kombináltan. A betegek mindössze 13,95%-ánál észleltünk mellékhatást, leggyakrabban bőrkiütést. A betegek várható ötéves teljes túlélése 78,7%, az átlagos progressziómentes túlélési idő 23,59 hónap (95% CI: 19,50–27,68). Következtetés: A relabáló vagy refrakter Hodgkin-lymphomás betegek kezelésében jelentős előrelépést jelent a brentuximab vedotin kezelés alkalmazása, eredményeink a nemzetközi adatokhoz hasonlóak. A kombinációban történő korai alkalmazása előrelépést jelenthet, ennek további vizsgálata szükséges. Orv Hetil. 2017; 158(41): 1630–1634. | Abstract: Introduction: The treatment of relapsed or refractory Hodgkin lymphoma is still a major therapeutic challenge. The use of brentuximab vedotin, an anti-CD30 antibody-drug conjugate, represents a promising approach for these patients, however clinical outcomes have not yet been evaluated in Hungary. Aim: Our aim was to assess the efficacy, safety and outcome of brentuximab vedotin treatment in Hungarian Hodgkin lymphoma patients. Method: In this retrospective case note review we enrolled patients at 6 clinical sites countrywide who were diagnosed with Hodgkin lymphoma and received brentuximab vedotin between 1 January 2013 and 31 December 2016. Results: A total of 86 patients were treated with brentuximab vedotin during the examined period. Before therapy initiation 66% of our patients had advanced-stage disease. Overall response rate to brentuximab vedotin, administered before autologous hematopoietic stem cell transplantation (n = 54) was 66.6%, complete remission rate was 42.6%. Thirty patients received brentuximab vedotin after AHSCT, 46.67% responded to treatment, 30% achieved complete remission. Thirty-six patients received the drug as a single-agent therapy, 50 patients were given brentuximab vedotin in combination, 39 of them with bendamustin. Toxicity was observed only in 13.95% of our patients, most common symptom was skin rash. Based on our analysis the estimated 5-year overall survival rate was 78.7%, the estimated progression free survival rate was 23.59 months (95% CI: 19.50–27.68). Conclusion: Brentuximab vedotin carries a substantial improvement in the treatment of relapsed or refractory Hodgkin lymphoma. Our results underline prior observations published in the literature. The use of brentuximab vedotin in combination can be beneficial, however further investigation is needed on the subject. Orv Hetil. 2017; 158(41): 1630–1634

Vitamin D in the Prevention and Treatment of Diabetic Neuropathy

Purpose: Neuropathy is one of the most important complications of diabetes. According to recent advances, vitamin D deficiency might play a role in the development and progression of diabetic neuropathy. Moreover, therapeutic vitamin D supplementation has the potential to improve this condition. The aim of the present review was to summarize new data available in this area.Methods: The PubMed database was searched for articles written in English and published through September 2021, using combinations of the following key words: vitamin D, diabetes, diabetes mellitus, diabetic neuropathy, polyneuropathy, peripheral neuropathy, cardiac autonomic neuropathy, supplementation, and therapy.Findings: A number of studies have suggested that vitamin D deficiency can play a significant role in the development of peripheral neuropathy, diabetic foot ulcers, as well as cardiovascular autonomic neuropathy in patients with type 2 diabetes. Vitamin D supplementation might serve as an effective adjuvant therapy for neuropathic pain and may slow or stop the progression of neural damage. (C) 2022 The Author(s). Published by Elsevier Inc

Malignus solid tumorhoz társuló hypereosinophil szindróma

A hypereosinophil szindróma tartós eosinophil-túltermeléssel járó, a következményes eosinophilinfiltráció és mediá- torfelszabadulás miatt többszervi károsodást okozó kórkép. Etiológia szerint megkülönböztetünk myeloproliferativ eredetű, parazitafertőzéshez, solid tumorhoz és T-sejtes lymphomához társuló, valamint idiopathiás formát. Esetis- mertetésünkben a 49 éves férfit fogyás, alszári oedema, tachycardia miatt vettük fel osztályunkra. Laborjából jelentősen emelkedett epeúti obstrukciós paraméterek, valamint extrém leukocytosis, eosinophilia volt kiemelhető. Hematológiai malignus betegség erős gyanújával kezdtük vizsgálni. Az elvégzett mellkasi, hasi és kismedencei CT hepatosplenomegaliát, multiplex intrahepaticus laesiókat és egy bizonytalan solitaer cystosus képletet írt le a pancreas farki részében, kóros nyirokcsomókkal és pleuralis folyadékgyülemmel. A leírt CT-kép a klinikum ismeretében elsősorban krónikus myeloid leukaemia manifesztációjának volt megfeleltethető, de a diagnózist a perifériás kenet, az áramlási citometria, a csontvelő-biopszia és a genetikai vizsgálat sem igazolta. Mindezek fényében solid tumorhoz társuló leukaemoid reakció irányába folytattuk a kivizsgálást, a májlaesiók pontos verifikálása érdekében vastagtűbiopszia történt. A szövettani eredmény pancreatobiliaris carcinoma áttétének megfeleltethető, alacsonyan differenciált hámtumor infiltrációját mutatta. A diagnózis felállításának másnapján kezelésünk ellenére a beteg exitált. A gastrointestinalis solid tumorokhoz kapcsolódó hypereosinophil szindróma rendkívül ritka kórkép. Tudomásunk szerint ez a magyar orvosi irodalomban közölt első ilyen eset, mely felhívja a figyelmet a magas fehérvérsejtszám és eosinophilarány differenciáldiagnosztikai kérdéseire, valamint arra, hogy nem korreláló hematológiai leletek esetén nem késlekedhetünk a solid eltérések szövettani mintavételével

A diabeteses neuropathia klinikai jelentősége és újabb kezelési lehetőségei

A Teljes szövegben nem a végleges cím és oldalszám látható