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Két myeloproliferativ betegség egy betegben – klónok együttélése és vetélkedése

Two myeloproliferative diseases in one patient – co-occurence of clones

Orvosi Hetilap
Authors:
Sándor Fekete
,
Judit Reichardt
,
András Kozma
,
Katalin Kapócs
, and
Nóra Meggyesi

A myeloproliferativ betegségeket genetikai hátterük alapján Philadelphia-kromoszóma-pozitív krónikus myeloid leukaemia, illetve Philadelphia-kromoszóma-negatív klasszikus myeloproliferativ neoplasia csoportokra bonthatjuk. Az utóbbiak hátterében a Janus-kináz-2 vagy a calreticulin gének mutációi állnak. Egy betegben a Philadelphia-kromoszóma-negatív és -pozitív myeloproliferativ neoplasiák együttes előfordulása irodalmi ritkaságnak számít. Munkánk során az elmúlt 20 év alatt mintegy 120, krónikus myeloid leukaemiás beteg hosszú távú követése kapcsán azon betegek klinikumát vizsgáltuk, akikben Philadelphia-kromoszóma-negatív myeloproliferativ neoplasiát is igazoltunk. A Philadelphia-kromoszóma jelenlétét klasszikus citogenetikai és/vagy fluoreszcens in situ hibridizációval mutattuk ki, majd a képződött BCRABL1 fúziós RNS mennyiségét kvantitatív, valós idejű polimeráz-láncreakcióval követtük. A Janus-kináz-2 és a kalretikulin gének mutációit szintén molekuláris genetikai módszerekkel, mennyiségi allélspecifikus polimeráz-láncreakcióval, illetve fragmensanalízissel mutattuk ki. A mutáns klónok mennyiségének időbeli változásának, valamint a klinikai adatoknak a segítségével következtettünk a betegségek kialakulásának dinamikájára. Munkánk során négy, Philadelphia-kromoszómát és a Janus-kináz-2/calreticulin gén mutációját is hordozó esetet azonosítottunk. Egyes esetekben a klónok vetélkedése, más esetben közös klónban együttes előfordulásuk volt megfigyelhető. Az izolált thrombocytosis a diagnóziskor, valamint a célzott kezelés hatására javuló genetikai eltérés mellett a perzisztáló vagy a fokozódó thrombocytosis hívhatja fel a figyelmet a két ritka hematológiai betegség társulásának lehetőségére. Eseteink felhívják a figyelmet arra, hogy a myeloproliferativ neoplasiák társulása kevésbé ritka, mint ahogy az irodalmi adatok sugallják. Ha a betegség szokatlan megjelenésű, vagy viselkedése ezt indokolttá teszi, felmerülhet a Philadelphia-kromoszóma-pozitív és -negatív myeloproliferativ neoplasiák társulásának gyanúja. Orv Hetil. 2022; 163(28): 1123–1129.

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Broiler chicken and rabbit experiments were carried out to study the effects of nickel (Ni) supplementation on growth performance and Ni metabolism. ROSS cockerels and New Zealand White female rabbits were fed a diet containing Ni in concentrations of 0, 50 and 500 mg/kg in dry matter (DM). Dietary supplementation of 50 mg Ni/kg slightly improved the body weight gain (BWG) and had a beneficial effect on the feed conversion efficiency (FCE) in broiler chickens. However, Ni added at a level of 500 mg/kg significantly (P < 0.05) reduced the BWG by 10% and resulted in significantly (P < 0.05) worse (2.3 ± 0.2 kg/kg) FCE. The relative weight of the liver in cockerels was significantly (P < 0.05) decreased by Ni as compared to the control group (1.7 and 2.1% vs. 2.6%). The activity of AST and CHE enzymes was increased insignificantly by dietary supplementation of 500 mg Ni/kg, indicating damage of the liver parenchyma. The results of serum biochemistry were confirmed by a mild or moderate form of pathological focal fatty infiltration of the liver in broilers. Supplemental Ni of 50 mg/kg concentration resulted in non-significantly increased BWG in rabbits. Ni added to the diet at a level of 500 mg/kg reduced the digestibility of crude protein by 3-4% and that of crude fibre by 20-25% in rabbits. Approx. 98% of the ingested Ni was lost from the body via the faeces, 0.5-1.5% via the urine and approx. 1% was incorporated into the organs of rabbits. As a result of dietary supplementation of 50 and 500 mg Ni/kg, Ni accumulated in the kidneys (4.9 ± 0.5 and 17.1 ± 3.1 vs. 1.9 ± 0.3 mg/kg DM), ribs (10.3 ± 0.4 and 10.4 ± 0.6 vs. 9.1 ± 0.6 mg/kg DM), heart (1.4 ± 0.2 and 2.5 ± 0.4 vs. 1.0 ± 0.1 mg/kg DM) and liver (1.3 ± 0.1 and 2.2 ± 0.2 vs. 0.9 ± 0.05 mg/kg DM), as compared to the control animals. It can be stated that supplementation of the diet with 50 mg Ni/kg had slight but non-significant beneficial effects on the growth performance of broiler chickens and rabbits.

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The effects of dietary levels of manganese (Mn) in inorganic (MnO) and organic (Mn fumarate) forms were evaluated on cockerel chicks. A basal corn-soybean diet with 23 mg/kg Mn was supplemented with levels of 0, 30, 60 and 240 ppm Mn from both Mn sources. Each treatment was replicated in five pens of 10 chicks. The chicks were fed diets ad libitum from 14 to 49 days of age, after which five birds per treatment were sacrificed for pathomorphological examinations and analysis. The treatments did not exert significant effects on the body weight (BW), the feed/gain (F/G) ratio or the mortality rate. According to the necropsy findings, no growth retardation or emaciation occurred in either of the groups and the differences in the average absolute and relative organ weights were not significant (P ? 0.05). Tissue analysis indicated that the tibia showed the greatest response to Mn, followed by the liver and kidney. Accumulation in the tibia was higher (P < 0.05) with supplements of 30, 60 and 240 mg/kg from both Mn sources (3.71, 3.78, 4.44, and 3.68, 4.00, 4.36 mg/kg DM, MnO and Mn fumarate, respectively) compared to the control group (3.21 mg/kg). Accumulation in the liver increased significantly (P < 0.05) only with supplements of 60 and 240 ppm independently of the Mn source (12.7, 14.2, and 14.0, 14.9 mg/kg, respectively) compared to the control (9.8 mg/kg). Similarly, kidney tissue Mn was higher (P < 0.05) only with supplements of 60 and 240 ppm (12.8, 12.8, and 13.1, 12.5 mg/kg, respectively) compared to the control (10.2 mg/kg). At the same level of supplementation of the two Mn sources there were no significant differences (P ? 0.05) between the Mn concentrations of organs and tissues. Droppings sensitively reflected the intake, whereas blood plasma and feathers showed only the extreme Mn loading.

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Potato and beetroot were grown on soils previously treated with heavy metal salts. Each particular microelement had a high concentration in both potato and beetroot [cadmium (Cd) 3.7 and 55.4, lead (Pb) 8.1 and 3.0, and mercury (Hg) 5.8 and 6.8 mg/kg dry matter, respectively]. In a metabolic balance trial 16 New Zealand White rabbits were fed 50 grams of basal diet and potato or beetroot ad libitum. The apparent digestibility of major nutrients and the accumulation of the microelements in different organs were investigated. Both potato and beetroot samples of high Pb and Hg content had the significantly (p < 0.05) lowest digestibility of organic matter and nitrogen-free extract. The Cd ingested from both potato and beetroot accumulated in the kidneys and liver (2.85 and 1.48 as well as 0.459 and 0.265 mg/kg, respectively). All the microelements (Cd, Pb and Hg) accumulated in the testicles (0.196, 0.32 and 0.199 mg/kg, respectively), reducing the rate of spermatogenesis. The tissue retention ofheavy metals depends not only on the element itself, but also upon the ‘carrier’ feedstuff.

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Acta Veterinaria Hungarica
Authors:
S. Fekete
,
I. Szakáll
,
Emma Kósa
,
Emese Andrásofszky
,
Kinga Fodor
,
A. Hidas
, and
J. Tőzsér

A growth trial was carried out to test the effect of organic, trivalent chromium and L-carnitine on the body composition of growing rats. At the same time, an evaluation of different measurement methods (weight of epididymal fat pad, adipocyte morphometry, total body electrical conductivity) was performed. Outbred Wistar rats of 30 days of age were fed diets of different (0, 10 and 20%) protein level. The diets were supplemented with 4 mg/kg Cr as chromium nicotinate, and 100 mg/kg L-carnitine. The experimental feeding lasted 15 days, after a 5-day-long adjustment period. It was found that Cr addition increased feed intake. Both treatments caused changes in body composition, increasing fat and protein deposition. Organic chromium had no effect at either protein level, while L-carnitine improved the protein retention only at an optimum (20%) protein supply. No statistically significant correlation was found between total body electrical conductivity (TOBEC) and body composition, which could be attributed to the great individual differences. A close correlation was found among total body fat percentage, weight of epididymal fat pad and the adipocyte surface. The data suggest that there is an interaction between dietary protein supply and the effect of repartitioning agents.

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Acta Veterinaria Hungarica
Authors:
A. Bersényi
,
S. Fekete
,
I. Hullár
,
I. Kádár
,
M. Szilágyi
,
R. Glávits
,
Margit Kulcsár
,
M. Mézes
, and
L. Zöldág

Carrots were grown on soils polluted by heavy metal salts. Each particular microelement reached a high concentration [molybdenum (Mo) 39.00, cadmium (Cd) 2.30, lead (Pb) 4.01, mercury (Hg) 30.00, and selenium (Se) 36.20 mg/kg dry matter] in the carrot. In a metabolic balance trial conducted with 15 male and 15 female New Zealand White rabbits, the control animals (n = 5) were fed ad libitum with concentrate as basal diet, while the other rabbits received the basal diet and carrots containing the particular microelement. Blood samples were taken to determine the activity of serum enzymes. To investigate the metabolism of Mo, Cd, Pb, Hg and Se, samples were taken from the heart, liver, lungs, kidneys, spleen, ovaries/testicles, entire digestive tract, adipose tissue, femur, hair, faeces and urine. Carrot had significantly higher digestibility for all nutrients than the rabbit concentrate. Carrot samples of high Pb content had the lowest digestibility of crude protein. The microelements differed in their rate of accumulation in the organs examined: Mo and Cd accumulated in the kidneys, Pb in the kidneys, liver, bones and lungs, Hg in the kidneys and liver, while Se in the liver, kidneys and heart. The proportions of microelements eliminated from the body either via the faeces and urine (Mo 80.18% and Se 47.41%) or via the faeces (Cd 37.86%, Pb 66.39%, Hg 64.65%) were determined. Pathohistological examination revealed that the rate of spermatogenesis was reduced in the Mo, Cd, Pb and Hg groups compared to the control. Lead, Cd and Hg intake resulted in a considerable decrease in gamma-glutamyltransferase (GGT) and in an increase of alkaline phosphatase (ALP) activity because of damages to the kidneys and bones. All experimental treatments decreased the activity of cholinesterase (CHE) because of lesions in the liver.

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Acta Veterinaria Hungarica
Authors:
Kinga Fodor
,
L. Zöldág
,
S. Gy. Fekete
,
A. Bersényi
,
A. Gáspárdy
,
Emese Andrásofszky
,
Margit Kulcsár
,
F. Eszes
, and
M. Shani

An experiment was carried out with young male New Zealand White (NZW) rabbits to establish live body weight changes, body measurements, body composition and sexual maturity as a function of feeding intensity. Animals in Group 1 ('AL', n = 10) were fed ad libitum, while those in Group 2 ('RS', n = 10) received restricted feeding corresponding to 70% of the ad libitum level. The starting liveweights were practically the same (0.907 ± 0.146 and 0.911 ± 0.147 kg in Group AL and Group RS, respectively). The feeding trial lasted from 6 to 22 weeks of age. The average body weight was significantly higher in Group AL from 7 to 22 weeks of age. At 22 weeks of age the body weight of RS rabbits was 85.64% of the weight of AL animals (3.22 ± 0.52 kg and 3.76 ± 0.33 kg, respectively). Average body weights of RS males at 8, 9, 11, 19 and 21 weeks of age were similar to those of ad libitum fed (AL) animals at 7, 8, 10, 15 and 16 weeks of age, respectively. The growth of bucks fed restricted tended to be allometric. The most significant difference was found at 16 and 18 weeks of age, while the lowest difference occurred at 6, 12, 15 and 19 weeks of age. It can be stated that low-intensity feeding up to slaughtering weight causes backwardness in rear cannon length and this backwardness remains also after the 15th week, which is well over the optimal slaughtering age. Based on the present data, the 70% restricted feeding cannot be recommended either for the future breeding bucks or for broiler males reared for slaughter. To determine the major chemical components of the body, rabbits were euthanised. Original dry matter and crude fat content of the body significantly (P < 0.05) decreased under restricted feeding (41.42%; 32.48% and 16.73%; 7.35%) while the percentage of protein within the dry matter increased (49.6%; 65.0%) and fat decreased (40.17%; 22.1%) significantly. Libido unambiguously decreases as a consequence of feed deprivation. The most conspicuous difference was found in the level of blood testosterone. Although a few RS bucks produced semen but only much later than the rabbits fed ad libitum. On the other hand, there was no difference in the motility of spermatozoa and ejaculate volume in comparison with AL animals. There was no relationship between the body fat content and the reproductive status of bucks in the present trial.

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Acta Veterinaria Hungarica
Authors:
Kinga Fodor
,
S. Gy. Fekete
,
L. Zöldág
,
A. Bersényi
,
A. Gáspárdy
,
Emese Andrásofszky
,
Margit Kulcsár
, and
F. Eszes

Twenty-six 6-week old female New Zealand White rabbits were divided into two groups: ad libitum (AL) and 70% restricted (RS) feeding. At the beginning of the experiment the liveweights were practically the same: 0.99 ± 0.08 vs. 1.01 ± 0.08 kg in group AL and RS, respectively. At 18 weeks of age the body weight of Group RS rabbits was 84.7% (3.14 ± 0.24 kg) of the group AL (3.71 ± 0.31 kg). The apparent digestibility of crude protein was significantly (p < 0.001) higher in Group RS than in Group AL (76.5 ± 1.4 vs. 73.0 ± 2.7%). The daily water consumption was significantly (p < 0.05) higher (3.5 ml/g DM intake) in Group RS as compared to Group AL (1.9 ml/g DM). Since the average body weight in Group RS at 18 weeks was the same as that of Group AL at 14 weeks of age (3.14 kg), the comparison of the live body measures and indices was based on these ages. Animals fed ad libitum or restricted show no differences at the defined age in most live body measurements except in heart girth and rump width, which were significantly (p < 0.05) shorter in Group AL than in Group RS (29.3 ± 0.8, 5.7 ± 0.5 and 30.7 ± 1.0, 6.2 ± 0.3 cm, respectively). Body capacity was significantly (p < 0.05) smaller in Group AL, as the head capacity-body capacity proportion was significantly (p < 0.05) higher in Group AL than in Group RS (1653.1 ± 134.5, 22.0 ± 2.5 and 1913.7 ± 168.7, 17.7 ± 1.9, respectively). Due to restricted feeding the growth of the head proved to be less intensive than that of the body at the same body weight. The body in these does tended to be wider. Since the head in comparison to length or capacity of the body was smaller in does fed 70% of ad libitum, it could be concluded that the development of body parts of restricted-fed does was unequal (allometric growth). The effect of feed restriction reflected in lower dry matter and fat, and a higher ash and protein content both in total body and in dry matter composition of rabbits at 18 weeks of age. Restricted feeding delayed sexual maturity (69 vs. 92% of rabbits) with later starting ovarian activity, weaker ovarian responsiveness, and a smaller number of tertiary follicles on the ovarian surface.

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Physiology International
Authors:
V. Fazekas-Pongor
,
M. Fekete
,
P. Balazs
,
D. Árva
,
M. Pénzes
,
S. Tarantini
,
R. Urbán
, and
J.T. Varga
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Physiology International
Authors:
M. Fekete
,
V. Fazekas-Pongor
,
P. Balazs
,
S. Tarantini
,
G. Szollosi
,
J. Pako
,
A.N. Nemeth
, and
J.T. Varga

Abstract

Background

Pathological alterations in nutritional status may develop in Chronic Obstructive Pulmonary Disease (COPD) patients through production of inflammatory cytokines and inadequate diet.

Objective

The aim of our study was to determine the correlation between nutritional status and quality of life of COPD patients.

Methods

We evaluated the nutritional status of COPD patients of Hungarian National Koranyi Institute for Pulmonology using the Malnutrition Universal Screening Tool (MUST) and bioelectrical impedance analysis (BIA) between January 1 and June 1, 2019. Lung function, physical fitness, and respiratory muscle strength were included in the assessment.

Results

Fifty patients (mean age was 66.3 ± 9.6 years) participated in our study. Mean body mass index (BMI) was 26.2 ± 6.1 kg/m2 and mean fat-free mass index (FFMI) was 16.8 ± 2.4 kg/m2. Overweight patients had better lung function values (FEV1ref%: 46.3 ± 15.2) than normal (FEV1ref%: 45.1 ± 20.9) and underweight patients (FEV1ref%: 43.8 ± 16.0). The Modified Medical Research Council Dyspnea Scale (mMRC) was significantly associated with various parameters; strongest correlation was found with FFMI (r = −0.537, P < 0.001), skeletal muscle mass index (SMMI) (r = −0.530, P < 0.001), and 6-minute walking distance (6MWD) (r = −0.481, P < 0.001).

Conclusions

Our results indicate that malnourished COPD patients may have reduced lung function and lower quality of life compared to normal weight patients. Thus, our findings suggest that nutritional therapy be included in the treatment of COPD patients combined with nutritional risk screening and BIA during the follow-up.

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