63 research outputs found
Scurvy
Scurvy is a nutritional disorder which can develop
after prolonged (>1-3 months) severe vitamin C deficiency. Vitamin C is a cofactor in several enzyme reactions involved in collagen synthesis. The defect in collagen causes blood vessel fragility, poor wound healing, mucocutaneous bleedings, hair abnormalities, bone pains, and joint contractures due to perios-teal and intraarticular bleeding (1,2). Risk factors for scurvy development are undernutrition, low socio-economic status, older age, male sex, alcoholism, tobacco smoking, and severe psychiatric illnesses (1-3). The required daily intake for vitamin C is ~60 mg, and this amount of vitamin C can be found in only one medium-sized orange. For this reason, the disease is rarely encountered in developed countries and is often underrecognized by healthcare personnel. Here-in, we present an illustrative case of scurvy in order to raise the awareness of this disorder
Scurvy
Scurvy is a nutritional disorder which can develop
after prolonged (>1-3 months) severe vitamin C deficiency. Vitamin C is a cofactor in several enzyme reactions involved in collagen synthesis. The defect in collagen causes blood vessel fragility, poor wound healing, mucocutaneous bleedings, hair abnormalities, bone pains, and joint contractures due to perios-teal and intraarticular bleeding (1,2). Risk factors for scurvy development are undernutrition, low socio-economic status, older age, male sex, alcoholism, tobacco smoking, and severe psychiatric illnesses (1-3). The required daily intake for vitamin C is ~60 mg, and this amount of vitamin C can be found in only one medium-sized orange. For this reason, the disease is rarely encountered in developed countries and is often underrecognized by healthcare personnel. Here-in, we present an illustrative case of scurvy in order to raise the awareness of this disorder
NUTRITIVNI ZBROJ (CONTROLLING NUTRITIONAL STATUS - CONUT) MOŽE PREDVIDJETI PREŽIVLJENJE BOLESNIKA NA HEMODIJALIZI
Malnutrition causes substantial morbidity in maintenance hemodialysis (HD) patients. The Controlling Nutritional Status (CONUT) has emerged as a simple and an easily obtainable tool to comprehensively assess nutrition as it consists of serum albumin levels, absolute lymphocyte counts, and total cholesterol levels. The CONUT has been shown to predict overall survival (OS) in peritoneal dialysis patients. This study investigated whether CONUT might also predict OS in maintenance HD patients. Clinical and laboratory data were retrospectively collected. Survival time was calculated from the fi rst HD until death or last follow-up; survival analyses were performed using the methods of Kaplan-Meier and Cox regression analysis. Eighty-nine patients were included; mean age was 65.76 years (Ā±14), 35 (39.3%) were female, and the mean CONUT was 3. Higher CONUT score correlated with lower low-density liproprotein, higher serum creatinine, higher serum C-reactive protein and higher neutrophil-to-lymphocyte ratio, as well as with a higher incidence of nephrotic proteinuria (p<0.050 for all analyses). Univariately, patients with higher CONUT (ā„5) had an inferior OS (median 54 vs. 112 months, HR 2.27; p=0.013). In the Cox regression analysis, higher CONUT remained independently associated with inferior OS (HR 9.50; p=0.002) when adjusted to age, sex, diabetes mellitus and nephrotic proteinuria. Therefore, the CONUT score might identify HD patients at an increased risk of death; however, future studies are needed to elucidate whether CONUT score might be able to guide nutritional support in HD patients.Pothranjenost uzrokuje znaÄajan pobol i smrtnost bolesnika na hemodijalizi (HD). Controlling Nutritional Status (CONUT) je jednostavan nutritivni zbroj koji cjelovito procjenjuje uhranjenost, a sastoji se od serumske koncentracije albumina, apsolutnog broja limfocita i koncentracije serumskog kolesterola. Ova unicentriÄna retrospektivna studija analizirala je prediktivnu sposobnost zbroja CONUT da procijeni preživljenje bolesnika na HD. Ukupno preživljenje mjereno je kao vrijeme od prve HD do smrti ili posljednjeg pregleda bolesnika, a krivulje preživljenja usporeÄene su Kaplan-Meirovom metodom, dok je Coxova regresijska metoda primijenjena u multivarijatnim analizama. UkljuÄeno je 89 bolesnika, od toga 35 (39,3 %) žena; srednja dob bila je 65,76 godina (Ā±14). Srednji zbroj CONUT bio je 3. ViÅ”i zbroj CONUT korelirao je s nižim koncentracijama serumskog lipoproteina niske gustoÄe, viÅ”im serumskim kreatininom, viÅ”im serumskim C-reaktivnim proteinom i viÅ”im omjerom neutrofi la/limfocita, kao i s veÄom uÄestaloÅ”Äu nefrotske proteinurije (p<0,050 za sve analize). U univarijatnoj analizi je viÅ”i zbroj CONUT (ā„5) bio povezan s loÅ”ijim preživljenjem (medijan 54 prema 112 mjeseci, HR 2,27; p=0,013). U multivarijatnoj Coxovoj regresijskoj analizi je viÅ”i CONUT ostao nezavisno povezan s loÅ”ijim preživljenjem (HR 9,50; p=0,002) kada je bio ispravljen za dob, spol, Å”eÄernu bolest i nefrotsku proteinuriju. ZakljuÄno, zbroj CONUT može identifi cirati bolesnike na HD s poviÅ”enim rizikom od smrti. Potrebne su dodatna istraživanja kako bi se analizirala sposobnost zbroja CONUT da usmjeri nutritivnu potporu u bolesnika na HD
Serum chitotriosidase: a circulating biomarker in polycythemia vera
Objectives: Serum chitotriosidase activity (CHIT1) is a biomarker of macrophage activation with an important role in inflammation-induced tissue remodeling and fibrosis. Macrophages have been described to play a crucial role in regulating pathological erythropoiesis in polycythemia vera (PV). The aim of this study was to evaluate CHIT1 in patients diagnosed with Philadelphia-negative myeloproliferative neoplasms (MPNs). -----
Methods: Using fluorometric assay, we measured CHIT1 in 28 PV, 27 essential thrombocythemia (ET), 17 primary myelofibrosis (PMF), 19 patients with secondary myelofibrosis and in 25 healthy controls. -----
Results: CHIT1 was significantly higher in PV (p < .001) and post-PV myelofibrosis (MF) transformation (post-PV MF) (p = .020), but not in ET (p = .080), post-ET MF transformation (p = .086), and PMF patients (p = .287), when compared to healthy controls. CHIT1 in PV was positively correlated with hemoglobin (p = .026), hematocrit (p = .012), absolute basophil count (p = .030) and the presence of reticulin fibrosis in the bone marrow (p = .023). -----
Discussion: A positive correlation between CHIT1 and these distinct laboratory PV features might imply macrophages closely related to clonal erythropoiesis as cells of CHIT1 origin. In addition, a positive association between CHIT1 and reticulin fibrosis might indicate its potential role in PV progression. -----
Conclusion: CHIT1 might be considered as a circulating biomarker in PV. Additional studies are needed to clarify the role of CHIT1 in promoting disease progression and bone marrow fibrosis in PV
Glycoprotein YKL-40: a novel biomarker of chronic graftvs- host disease activity and severity?
Aim To investigate whether increased YKL-40 levels positively
correlate with graft-vs-host disease (cGVHD) activity
and severity and if YKL-40 could serve as a disease biomarker.
Methods This case-control study was conducted at the
University Hospital Centre Zagreb from July 2013 to October
2015. 56 patients treated with hematopoietic stem
cell transplantation (HSCT) were included: 35 patients with
cGVHD and 21 without cGVHD. There was no difference
between groups in age, sex, median time from transplant
to study enrollment, intensity of conditioning, type of donor,
or source of stem cells. Blood samples were collected
at study enrollment and YKL-40 levels were measured with
ELISA. Disease activity was estimated using Clinicianās Impression
of Activity and Intensity of Immunosuppression
scales and disease severity using Global National Institutes
of Health (NIH) score.
Results YKL-40 levels were significantly higher in cGVHD
patients than in controls (P = 0.003). The difference remained
significant when patients with myelofibrosis were
excluded from the analysis (P = 0.017). YKL-40 level significantly
positively correlated with disease severity (P < 0.001;
correlation coefficient 0.455), and activity estimated using
Clinicianās Impression of Activity (P = 0.016; correlation coefficient
0.412) but not using Intensity of Immunosuppression
(P = 0.085; correlation coefficient 0.296).
Conclusion YKL-40 could be considered a biomarker of
cGVHD severity and activity. However, validation in a larger
group of patients is warranted, as well as longitudinal
testing of YKL-40 levels in patients at risk of developing
cGVHD
FLUDARABINE, CYCLOPHOSPHAMIDE AND RITUXIMAB (FCR) IN THE TREATMENT OF CHRONIC LYMPHOCYTIC LEUKEMIA (CLL): UNIVERSITY HOSPITAL CENTRE ZAGREB EXPERIENCE
U kliniÄkim je studijama kombinacija fludarabina, ciklofosfamida i rituksimaba (FCR) pokazala odliÄne rezultate u lijeÄenju bolesnika s kroniÄnom limfocitnom leukemijom (KLL) i postala zlatni standard u prvoj liniji lijeÄenja takvih bolesnika bez znatnijih komorbiditeta. Cilj rada bio je ispitati terapijsku djelotvornost, toksiÄnost i provedivost ovog protokola u svakodnevnoj kliniÄkoj praksi. Retrospektivno su analizirani tijek i ishodi lijeÄenja 43-oje bolesnika s KLL-om sa Zavoda za hematologiju Klinike za unutarnje bolesti KliniÄkoga bolniÄkog centra Zagreb. Shema primjene rituksimaba razlikovala se od one u kliniÄkim studijama; primjenjivan je infuzijski u dozi od 375 mg/m2 u svim ciklusima, u ukupno osam doza u prvoj, odnosno Å”est u kasnijim linijama lijeÄenja. Na lijeÄenje je odgovorilo 95% bolesnika, a 83% postignulo je kompletnu remisiju. TrogodiÅ”nje preživljenje i preživljenje bez progresije bolesti u prvoj liniji lijeÄenja (29 bolesnika) bilo je 90 i 80%, a u kasnijim linijama 86 i 62%. TeÅ”ke neutropenije zabilježene su u 46% bolesnika, a teÅ”ke infekcije u 9% bolesnika. Ishodi lijeÄenja i toksiÄni profil u svakodnevnome kliniÄkom radu usporedivi su s onima iz kliniÄkih studijaIn clinical trials the combination of fludarabine, cyclophosphamide and rituximab (FCR) demonstrated superior results and became the gold standard for first-line treatment of fit patients with chronic lymphocytic leukemia (CLL). The aim of this study was to evaluate the efficacy, toxicity and feasibility of this protocol in everyday clinical practice. We Āretrospectively analyzed the outcomes of 43 CLL patients treated at the Division of Haematology, Department of Internal Medicine, University Hospital Centre Zagreb. The dosing of rituximab differed from that in clinical trials, we administered 375mg/m2 of rituximab per cycle, in previously untreated patients for eight and in relapsed/refractory patients for six cycles. The response rate was 95% with 83% of complete remissions. Twentyānine patients received FCR as a frontāline therapy; three-year overall and progressionāfree survival were 90% and 80%, respectively. In relapsed/refractory disease threeāyear overall and progressionāfree survival were 86% and 62%, respectively. Severe neutropenias occurred in 46% and serious infections in 9% of patients. According to these results, the toxicity profile and treatment outcomes in everyday routine clinical practice are similar to those reported in clinical trials
Fludarabin, ciklofosfamid i rituksimab (FCR) u lijeÄenju bolesnika s kroniÄnom limfocitnom leukemijom (KLL): iskustvo KliniÄkoga bolniÄkog centra Zagreb [Fludarabine, cyclophosphamide and rituximab (FCR) in the treatment of chronic lymphocytic leukemia (CLL): University Hospital Centre Zagreb experience]
In clinical trials the combination of fludarabine, cyclophosphamide and rituximab (FCR) demonstrated superior results and became the gold standard for first-line treatment of fit patients with chronic lymphocytic leukemia (CLL). The aim of this study was to evaluate the efficacy, toxicity and feasibility of this protocol in everyday clinical practice. We Āretrospectively analyzed the outcomes of 43 CLL patients treated at the Division of Haematology, Department of Internal Medicine, University Hospital Centre Zagreb. The dosing of rituximab differed from that in clinical trials, we administered 375mg/m2 of rituximab per cycle, in previously untreated patients for eight and in relapsed/refractory patients for six cycles. The response rate was 95% with 83% of complete remissions. Twentyānine patients received FCR as a frontāline therapy; three-year overall and progressionāfree survival were 90% and 80%, respectively. In relapsed/refractory disease threeāyear overall and progressionāfree survival were 86% and 62%, respectively. Severe neutropenias occurred in 46% and serious infections in 9% of patients. According to these results, the toxicity profile and treatment outcomes in everyday routine clinical practice are similar to those reported in clinical trials
FLUDARABINE, CYCLOPHOSPHAMIDE AND RITUXIMAB (FCR) IN THE TREATMENT OF CHRONIC LYMPHOCYTIC LEUKEMIA (CLL): UNIVERSITY HOSPITAL CENTRE ZAGREB EXPERIENCE
U kliniÄkim je studijama kombinacija fludarabina, ciklofosfamida i rituksimaba (FCR) pokazala odliÄne rezultate u lijeÄenju bolesnika s kroniÄnom limfocitnom leukemijom (KLL) i postala zlatni standard u prvoj liniji lijeÄenja takvih bolesnika bez znatnijih komorbiditeta. Cilj rada bio je ispitati terapijsku djelotvornost, toksiÄnost i provedivost ovog protokola u svakodnevnoj kliniÄkoj praksi. Retrospektivno su analizirani tijek i ishodi lijeÄenja 43-oje bolesnika s KLL-om sa Zavoda za hematologiju Klinike za unutarnje bolesti KliniÄkoga bolniÄkog centra Zagreb. Shema primjene rituksimaba razlikovala se od one u kliniÄkim studijama; primjenjivan je infuzijski u dozi od 375 mg/m2 u svim ciklusima, u ukupno osam doza u prvoj, odnosno Å”est u kasnijim linijama lijeÄenja. Na lijeÄenje je odgovorilo 95% bolesnika, a 83% postignulo je kompletnu remisiju. TrogodiÅ”nje preživljenje i preživljenje bez progresije bolesti u prvoj liniji lijeÄenja (29 bolesnika) bilo je 90 i 80%, a u kasnijim linijama 86 i 62%. TeÅ”ke neutropenije zabilježene su u 46% bolesnika, a teÅ”ke infekcije u 9% bolesnika. Ishodi lijeÄenja i toksiÄni profil u svakodnevnome kliniÄkom radu usporedivi su s onima iz kliniÄkih studijaIn clinical trials the combination of fludarabine, cyclophosphamide and rituximab (FCR) demonstrated superior results and became the gold standard for first-line treatment of fit patients with chronic lymphocytic leukemia (CLL). The aim of this study was to evaluate the efficacy, toxicity and feasibility of this protocol in everyday clinical practice. We Āretrospectively analyzed the outcomes of 43 CLL patients treated at the Division of Haematology, Department of Internal Medicine, University Hospital Centre Zagreb. The dosing of rituximab differed from that in clinical trials, we administered 375mg/m2 of rituximab per cycle, in previously untreated patients for eight and in relapsed/refractory patients for six cycles. The response rate was 95% with 83% of complete remissions. Twentyānine patients received FCR as a frontāline therapy; three-year overall and progressionāfree survival were 90% and 80%, respectively. In relapsed/refractory disease threeāyear overall and progressionāfree survival were 86% and 62%, respectively. Severe neutropenias occurred in 46% and serious infections in 9% of patients. According to these results, the toxicity profile and treatment outcomes in everyday routine clinical practice are similar to those reported in clinical trials
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