Assessment of Right Heart Functions in Children with Mild Cystic Fibrosis

Abstract Background Cystic fibrosis (CF) is a multisystemic disease that prevalently involves the lungs. Hypoxemia occurs due to the existing of progressive damage to the pulmonary parenchyma and pulmonary vessels. The condition may cause systolic and diastolic dysfunction to the right ventricle due to the effects of high pulmonary artery systolic pressure (PASP). The study aimed to determine echocardiographic alterations in PASP, right ventricle (RV) anatomy, and functions in mild CF children. Materials and Methods RV anatomy, systolic, and diastolic functions were evaluated with conventional echocardiographic measurements. Estimated PASP was used measured with new echocardiographic modalities, including pulmonary artery acceleration time (PAAT), right ventricular ejection time (RVET), and their ratio (PAAT/RVET). The obtained echocardiographic data were statistically compared between the patient group and the control group. Results The study consisted of 30 pediatric patients with mild CF and 30 healthy children with similar demographics. In patient group, conventional parameters disclosed differences in RV anatomy, both systolic and diastolic functions of RV compared with the healthy group. We did not compare the patient group with published standard data because of the wide range variability. However, new echocardiographic parameters showed notable increase in pulmonary artery pressure compared with values of control group and published standard data (p<0.001). Conclusion Elevated PASP, RV failure, and Cor pulmonale usually begin early in children with mild CF. In addition to routine echocardiographic measurements to evaluate RV, we recommend the use of new echocardiographic modalities for routine examinations and in the follow up of children with mild CF.

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Echocardiographic evaluation of ventricular septal defect haemodynamics

Srpski arhiv za celokupno lekarstvo ◽

10.2298/sarh0710541m ◽

2007 ◽

Vol 135 (9-10) ◽

pp. 541-546

Author(s):

Vesna Miranovic

Keyword(s):

Ventricular Septal Defect ◽

Pulmonary Artery ◽

Body Surface ◽

Septal Defect ◽

Left Ventricular ◽

The Body ◽

Control Group ◽

Healthy Children ◽

And Control ◽

The Relationship

Introduction Ventricular septal defect (VSD) is an opening in the interventricular septum. 30-50% of patients with congenital heart disease have VSD. Objective The aim of the study was to determine the dependence of the left ventricular diastolic dimension (LVD), left ventricular systolic dimension (LVS), shortening fraction (SF), left atrium (LA), pulmonary artery truncus (TPA) on the body surface and compare their values among experimental, control and a group of healthy children. Values of maximal systolic gradient pressure (Pvsd) of VSD were compared with children from one experimental and control group. Method Children were divided into three groups: experimental (32 children with VSD that were to go to surgery), control (20 children with VSD who did not require surgery) and 40 healthy children. Measurements of LVD, LVS, SF, LA, TPA were performed in accordance to recommendations of the American Echocardiographic Association. The value of Pvsd was calculated from the maximal flow velocity (V) in VSD using the following formula: Pvsd=4xV? (mm Hg). Results For children from the experimental group, the relationship between the body surface and the variability of the LVD was explained with 56.85%, LVS with 66.15%, SF with 4.9%, TPA with 58.92%. For children from the control group, the relationship between the body surface and the variability of LVD was explained with 88.8%, LVS with 72.5%, SF with 0.42%, PA with 58.92%. For healthy children, the relationship between the body surface and the variabilitiy of the LVD was explained with 88.8%, LVS with 88.78%, SF with 5.25% and PA with 84.75%. There was a significant statistical difference between average values of Pvsd in the experimental and control group (p<0.02). Conclusion The presence of the large VSD has an influence on the enlargement of LVD, LVS, SF, TPA. The enlargement of the size of the pulmonary artery depends on the presence of VSD and there is a direct variation in the magnitude of the shunt. There is a relationship and significant dependence of the LVS and LVD on the body surface. There is no statistically significant dependence between SF and body surface.

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Evaluation of the quality-of-life (QOL) and socio-demographic characteristics of patients with leukemia and lymphoma: Comparison with sibling and control group.

Journal of Clinical Oncology ◽

10.1200/jco.2020.38.15_suppl.e22524 ◽

2020 ◽

Vol 38 (15_suppl) ◽

pp. e22524-e22524

Author(s):

Nurdan Tacyildiz ◽

Tugba Karakose ◽

Emel Cabi Unal ◽

Handan Dincaslan ◽

Gulsah Tanyildiz ◽

...

Keyword(s):

Quality Of Life ◽

Patient Group ◽

Active Treatment ◽

Healthy Control Group ◽

Control Group ◽

Healthy Children ◽

Healthy Control ◽

And Control ◽

Sibling Group

e22524 Background: Pediatric cancer patients have increased survival rates with intensive and effective treatment methods which causing some chronic health problems and lower quality of life in long term follow up. In our study, patients in their active treatment period compared to patients who have completed their treatment in recent 5 years and more than 5 years groups, besides compared with their own siblings and healthy control group,evaluated in terms of quality of life ( QOL). Methods: This study has been daone in the Department of Pediatric Hematology-Oncology, Ankara University School of Medicine. A total of 191 children were included in the study, including 36 sibling, 76 children with leukemia (n:31) and lymphoma (n:45) besides 79 healthy children. The Turkish translation of the PedsQL questionnaire was used to evaluate the patients' quality of life. IBM SPSS-25 package program was used for statistical analysis. Results: When the sociodemographic characteristics of the patient, sibling and control group were examined; we determined that the patients, siblings and control groups were similar in age, age and gender. However, we found that the education level for the patient group was statisticaly signicantly lower then the siblings and the control group. In addition, physical and mental functionning and friendship capasity scores were lower in patient group than the other two groups. According to timing of the treatment; patients and parents quality of life scores were significantly lower than the patients that finished their treatment in recent 5 years or more than 5 years. QOL scores were higest in survivors and parents group that were in more than 5 years after completed the treatment. The risk of lower QOL in patients under treatment was 7.48 times higher than the children who had ≥5 years of treatment (OR = 7,48; p < 0,05), and 2.64 times higher than those who were treated < 5 years (OR = 2,64; p > 0,05). In this study, there were no independent variables that had an impact on QOL of the sibling group. Conclusions: The QOLof patients diagnosed with leukemia and lymphoma is significantly lower than siblings and healthy group. No statistically significant difference was found between the sibling group and the healthy control group. After the end of active treatment, the QOL of the patients was reflecting important increase by the time .

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Pulmonary hemodynamics and diastolic function of right ventricle at isometric exercises in children with chronic bronchopulmonary pathology

Bulletin of Siberian Medicine ◽

10.20538/1682-0363-2011-3-105-110 ◽

2011 ◽

Vol 10 (3) ◽

pp. 105-110

Author(s):

L. I. Agapitov ◽

Yu. M. Belozerov ◽

Yu. L. Mizernitsky ◽

S. E. Tsyplenkova

Keyword(s):

Pulmonary Artery ◽

Right Ventricle ◽

Diastolic Function ◽

Pulmonary Artery Pressure ◽

Isometric Exercise ◽

Healthy Children ◽

Pulmonary Hemodynamics ◽

Artery Pressure ◽

Mean Pulmonary Artery Pressure ◽

The Right

We studied pulmonary hemodynamics and right ventricular diastolic function in 300 healthy children and 392 children with chronic bronchopulmonary pathology in an isometric exercise. Using echocardiography were determined systolic, diastolic, mean, pulmonary artery pressure, total pulmonary resistance, diastolic function of the right ventricle. Defined normal values of reactivity of pulmonary hemodynamics and diastolic function of the right ventricle in an isometric load. In patients with chronic bronchopulmonary pathology set higher increase of systolic, diastolic, mean, pulmonary artery pressure, decrease diastolic reserve the right ventricle compared to the control. The worst violations of pulmonary hemodynamics and diastolic function of the right ventricle during isometric load observed in patients with chronic Infectious-inflammatory and interstitial lung disease.

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Analysis of Bone Metabolism in Children with Cystic Fibrosis

PRILOZI ◽

10.2478/prilozi-2018-0035 ◽

2018 ◽

Vol 39 (1) ◽

pp. 151-155

Author(s):

Tatjana Jakovska

Keyword(s):

Cystic Fibrosis ◽

Bone Formation ◽

Bone Markers ◽

Formation Rate ◽

Serum Markers ◽

Control Group ◽

Healthy Children ◽

Pediatric Clinic ◽

Significant Difference ◽

The University

Abstract One of the most important CF-related conditions is the bone disease, which is nowadays acknowledged as a significant clinical complication of CF. Imbalance between bone formation and degradation in cystic fibrosis (CF) has become an important issue for developing osteopenia. The aim of the study was to assess bone formation and resorption process with bone markers in children with cystic fibrosis (CF). Materials and methods: The study included 35 clinically stable children with CF who regularly attended the Cystic fibrosis center at the University Pediatric Clinic in Skopje, R. Macedonia. The control group was presented with 21 healthy children at the same age. Serum osteocalcin (OC), β cross laps, 25OHD and PTH were determined by ELISA assays in the CF group (mean age 8.25±SD1.9 y.) and in age-match controls (7.5±1.9 y.). Results: Vitamin D in the CF group was (23.83±10.9 ng/ml versus 25.6±11.53 in the control group, p=0.57), OC (70.88±34.24 ng/ml v.100.02±47.98, p=0.01) βcrosslaps (1.35±0.72 ng/ml v.1.54±0.73, p=0.37) and PTH (37.39±25.5 pg/ml v. 36.76±25.73, p=0.92). In the study, we did not find a significant difference for 25OHD between CF and healthy controls. OC in children with CF correlates significantly with the control and indicates a decreased formation rate whereas resorption rate is normal. Conclusion: Our results suggest that bone turnover in CF is impaired in childhood. Serum markers for bone formation can be used for predicting osteopenia in children with CF.

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EVALUATION OF THE RELATIONSHIP BETWEEN DENTAL CARIES AND OXIDATIVE STRESS AND ANTIOXIDANT CAPACITY

10.22541/au.161717895.53907771/v1 ◽

2021 ◽

Author(s):

Ahmet Güzelçiçek ◽

Mahmut Demir ◽

Adnan Kirmit ◽

MEHMET DOGAN

Keyword(s):

Oxidative Stress ◽

Dental Caries ◽

Patient Group ◽

Total Antioxidant Status ◽

Stress Index ◽

Control Group ◽

Healthy Children ◽

Markers Of Oxidative Stress ◽

The Relationship ◽

And Oxidative Stress

Background Dental caries can be affected or altered by the body’s oxidant / antioxidant balance and oxidative stress. Aim/Hypothesis To examine the relationship between dental caries and Total Oxidant Status (TOS), Total Antioxidant Status (TAS), and Oxidative Stress Index (OSI). Design The study group consisted of 52 children with dental caries (DMFS/dfs ≥ 5), and the control group consisted of 42 healthy children (DMFS/dfs = 0). The saliva samples, unstimulated and mid-morning, were collected from all children. Participants’ saliva samples were acquired by spitting for 5 minutes, after which the samples were frozen and stored at 4 ° C and kept at -80 ° C until analysis. NCSS (Number Cruncher Statistical System) 2007 (Kaysville, Utah, USA) was used for the statistical analysis. Results TOS of the patient group cases were statistically significantly higher than those of the control group (p=0,004; p<0,01). OSI values of the patient group cases were statistically significantly higher than those of the control group (p=0,008; p<0,01). Conclusion OSI and TOS as markers of oxidative stress levels were found to be significantly higher in the group of patients with dental caries than in the healthy group.

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The Eveluation of Pulmonary Functions in Children with Sickle Cell Anemia.

Blood ◽

10.1182/blood.v114.22.4628.4628 ◽

2009 ◽

Vol 114 (22) ◽

pp. 4628-4628

Author(s):

Cem Kurt ◽

Ilgen Sasmaz ◽

Bulent Antmen ◽

Yurdanur Kilinc ◽

Sadi Kurdak ◽

...

Keyword(s):

Patient Group ◽

Sickle Cell ◽

Sickle Cell Anemia ◽

Exercise Test ◽

Acute Chest Syndrome ◽

Control Group ◽

Healthy Children ◽

Diffusion Test ◽

Pulmonary Functions

Abstract Abstract 4628 Aim In this study we evaluated to pulmonary functions and determined relations of these findings with clinical parameters in children with sickle cell anemia (SCA) who were at follow up in our pediatric heamatology clinic. Materials and Methods 24 children with sickle cell anemia and 9 healthy children as control group include to the study. Complete blood count, hemoglobin electrophoresis and biochemical values were eveluated for both groups. At pulmonology department, the carbonmonoxide diffusion test performing for both groups. At the same day spirometric respiratory function evaluation and exercise test performed both groups at department of sports physiology. The data recieved are compared statistics. Results HbS, HbF, SGPT, ferritin, total bilirubine, direkt bilirubine and Fe++ values were high at patient group (p<0.05). Hemoglobin and hematocrit values were low at patient group according to control group as expected (p<0.05). The number of SCA patient who had one-three venoocclusive crises (VOC) were 14 (58.3%), patient who had three or more VOC were 7 (29.2%) and patient who had no VOC were 3 (12.5%). The number of patient who had acute chest syndrome (ACS) were 5 (20.9%) and 19 patients had no ACS (79.1%). Ýmpaired isole carbonmonoxide diffusion test was established at the 62.5% of the patient's. At patient group, spirometric FEV1 and MEF25 measurement were found lower than the control group (p<0.05). At exercise test VO2/HR rate were lower for patient group (p<0.05). VE/VO2 rate (p=0.023) and R (p=0.016) measurement were found higher. Conclusion Pulmonary gas transfer was found difficult in patients with SCA. Respiratory airways established obstructed in spirometric evaluation. Obstructive defficiensies have to be follow up. Oxygen pulse and respiratory exchange rates were determined low and more oxygen usage was observed for aerobic metabolic activity. With these results, ýt can be say that chronic inflamation process at lung due to oxygen radicals and hipoksemia in sickle cell patients, the aerobic respiratory load was increased. Disclosures: No relevant conflicts of interest to declare.

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An Investigation of Hearing (250–20,000 Hz) in Children with Endocrine Diseases and Evaluation of Tinnitus and Vertigo Symptoms

International Archives of Otorhinolaryngology ◽

10.1055/s-0039-1698775 ◽

2020 ◽

Vol 24 (02) ◽

pp. e198-e205

Author(s):

Murat Kocyigit ◽

Selin Ustun Bezgin ◽

Taliye Cakabay ◽

Safiye Giran Ortekin ◽

Melek Yıldız ◽

...

Keyword(s):

Hearing Loss ◽

Patient Group ◽

Metabolic Diseases ◽

Hormone Deficiency ◽

Control Group ◽

Healthy Children ◽

Endocrine Disease ◽

High Frequencies ◽

Endocrine Diseases

Abstract Introduction Despite much advancement in medicine, endocrine and metabolic diseases remain an important cause of morbidity and even mortality in children. Objective The present study was planned to investigate the evaluation of hearing that also includes high frequencies, and the presence and degree of vertigo and tinnitus symptoms in pediatric patients diagnosed with endocrine diseases such as type 1 diabetes mellitus (DM), growth hormone deficiency (GHD), obesity, idiopathic short stature, and precocious puberty Methods The present study included a patient group of 207 children patients diagnosed with endocrine disease (95 males, 112 females; mean age 9.71 years old [range 6–16 years old]) and a control group including 55 healthy children who do not have any kind of chronic disease (26 males, 29 females; mean age 9.33 years old [range 6–16 years old]). The subjects underwent a hearing test with frequencies between 250 and 20,000 Hz. The vestibular and tinnitus symptoms were evaluated with the Pediatric Vestibular Symptom Questionnaire. Results Out of 207 patients in the patient group, 5 (2.4%) had hearing loss in pure tones, 10 (4.8%) had it in high frequencies, 40 (19.3%) had tinnitus symptoms, and 18 (8.7%) had vertigo symptoms. A total of 4 out of 207 patients in the study group (1.9%), 2 out of 59 with type 1 DM patients (3.4%), 1 out of 46 with GHD (2.2%), and 1 out of 43 obesity patients (2.3%) had hearing loss, vertigo, and tinnitus symptoms. Conclusions Our results suggest that some childhood endocrine diseases can cause some changes in the inner ear, although the exact cause is unknown. Perhaps, a detailed hearing and balance examination should be a routine in a child diagnosed with an endocrine disease. We think it is necessary to work on more comprehensive patient groups and tests in the future.

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Cystic Fibrosis and Family Stress: Effects of Age and Severity of Illness

PEDIATRICS ◽

10.1542/peds.79.2.239 ◽

1987 ◽

Vol 79 (2) ◽

pp. 239-246

Author(s):

Lynn S. Walker ◽

Melissa B. Ford ◽

William D. Donald

Keyword(s):

Cystic Fibrosis ◽

Early Adolescence ◽

Middle Childhood ◽

Age Groups ◽

Severity Of Illness ◽

Illness Severity ◽

Subjective Rating ◽

Control Group ◽

Healthy Children ◽

Relationship Of

Stress and adjustment in mothers of children with cystic fibrosis was compared with that in a control group of mothers of healthy children. Mothers of children in four age groups were included: preschool, middle childhood, early adolescence, and late adolescence. Mothers of children with cystic fibrosis did not report significantly higher levels of stress than did the control group mothers; nor did they report greater feelings of inadequacy as parents. However, mothers of children with cystic fibrosis in two age groups, preschool and early adolescence, scored higher on a measure of depression than did mothers of healthy children in the same age groups. The relationship of illness severity to maternal stress and adjustment was examined in the cystic fibrosis group. The mother's subjective rating of the child's illness severity was a better indicator of her reported stress than was the Shwachman clinical rating. It appears that many mothers are able to adapt to the presence of cystic fibrosis in the family, although certain periods in the child's life and perceived increases in illness severity are associated with increased maternal distress.

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Abstract 17062: A Novel Non-Invasive Index to Characterize Right Ventricle-Pulmonary Arterial Vascular Coupling in Children

Circulation ◽

10.1161/circ.138.suppl_1.17062 ◽

2018 ◽

Vol 138 (Suppl_1) ◽

Author(s):

Philip Levy ◽

Kel Vin Woo ◽

Amanda T Hauck ◽

Aaron Hamvas ◽

Gautam K Singh

Keyword(s):

Pulmonary Artery ◽

Right Ventricle ◽

Pulmonary Artery Pressure ◽

Reliable Estimate ◽

Artery Pressure ◽

Non Invasive ◽

Mean Pulmonary Artery Pressure ◽

Wide Range ◽

Pulmonary Arterial ◽

Force Relationship

Introduction: The right ventricle (RV) is a pulsatile pump, the efficiency or work of which depends on proper hemodynamic coupling with the compliant pulmonary arterial vasculature (PV). RV-PV coupling derived by right heart catheritization (RHC) measures the ratio of ventricular elastance (Ees, contractility) to arterial elastance (Ea, afterload) and is based on the work-energy principle that states a change in energy is equal to the work expended by a force through displacement. Non-invasive echocardiography derived surrogates to the Ees/Ea ratio that account for the length-force relationship have been proposed, but their clinical correlation as estimates of RV-PV coupling remain to be established. Hypothesis: We hypothesize that the non-invasive index of RV length-force relationship is a reliable measure of invasive RV-PV coupling (Ees/Ea) in children. Methods: Prospectively acquired simultaneous echocardiography-derived ratio of tricuspid annular plane systolic excursion to pulmonary artery acceleration time (TAPSE/PAAT) and RHC-derived Ees/Ea were compared using regression analysis in a cohort of 125 children (median age, 5 years; IQR, 1-13 years) with a wide range of pulmonary hemodynamics. Ees/Ea was calculated using the single beat method from RHC measures with the following equation: (RV systolic pressure - mean pulmonary artery pressure) / mean pulmonary artery pressure. TAPSE (mm), as a measure of RV contractile reserve, was substituted for length, and PAAT (msec), a reliable estimate of RV afterload, was exchanged for force. PAAT was adjusted for RV ejection time (PAATi=PAAT/RVET) to account for heart rate variability. Results: TAPSE/PAAT and TAPSE/PAATi both correlated with invasive Ees/Ea (r = 0.81, p<0.01 and r=0.76, p<0.01, respectively, Figure). TAPSE/PAAT and TAPSE/PAATi were both decreased (p<0.001) in children with pulmonary hypertension by RHC (mPAP>25 mmHg and PVRi>3 WU.m2). With ROC analysis, TAPSE/PAAT < 0.13 m/sec and TAPSE/PAATi < 38 mm detected Ees/Ea < 1.0 with sensitivity of 95% and specificity of 91% with an AUC of 0.90 (95% CI, 0.85-0.96). Conclusions: TAPSE/PAAT and TAPSE/PAATi , non-invasive indices of the length-force relationship, provide reliable estimates of invasive RV-PV coupling in children.

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Physical-Capacity-Related Genetic Polymorphisms in Children with Cystic Fibrosis

Pediatric Exercise Science ◽

10.1123/pes.2014-0050 ◽

2015 ◽

Vol 27 (1) ◽

pp. 102-112 ◽

Cited By ~ 4

Author(s):

Thomas Yvert ◽

Catalina Santiago ◽

Elena Santana-Sosa ◽

Zoraida Verde ◽

Felix Gómez-Gallego ◽

...

Keyword(s):

Cystic Fibrosis ◽

Energy Metabolism ◽

Muscle Function ◽

Muscular Strength ◽

Genetic Factors ◽

Physical Capacity ◽

Control Group ◽

Healthy Children ◽

Secondary Purpose ◽

Significant Polymorphism

In patients with cystic fibrosis (CF), physical capacity (PC) has been correlated with mortality risk. In turn, PC is dependent on genetic factors. This study examines several polymorphisms associated with PC and healthrelated phenotype traits (VO2peak, FEV1, FVC, PImax and muscular strength) in a group of children with CF (n = 66, primary purpose). The same analyses were also performed in a control group of healthy children (n = 113, secondary purpose). The polymorphisms determined were classified as muscle function polymorphisms (ACE rs1799752; AGT rs699; ACTN3 rs1815739; PTK2 rs7843014 and rs7460; MSTN rs1805086; TRHR rs7832552; NOS3 rs2070744) or energy metabolism polymorphisms (PPARGC1A rs8192678; NRF1 rs6949152; NRF2 rs12594956; TFAM rs1937; PPARD rs2267668; ACSL1 rs6552828). No significant polymorphism/phenotype correlations were detected in children with CF, with marginal associations being observed between NOS3 rs2070744 and VO2peak and FEV1, as well as between PPARGC1A rs8192678 and FEV1. Overall, similar findings were observed in the control group, i.e., no major associations. The PC-related polymorphisms examined seem to have no effects on the PC or health of children with CF.

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