Quality of life in adults with cystic fibrosis: the Greek experience

INTRODUCTION: Recent developments in treatment have steadily raised the median predicted age of survival for people with Cystic Fibrosis (CF). We report the health-related quality of life (HRQoL) in CF adult patients and correlate our findings with the patients’ demographic characteristics. MATERIAL AND METHODS: The Cystic Fibrosis Quality of Life (CFQoL) questionnaire was answered by 77 CF adult patients. The questionnaire included questions pertaining to age, sex and level of education and covered eight sections of functioning. RESULTS: The highest score was reported in the “Social Functioning” section, while the lowest in the “Concerns for the Future” section. When different age groups were compared, statistical significances were reported in “Physical Functioning”, “Interpersonal Relationships”, and the “Career Concerns” section, with older patients reporting statistically higher HRQoL scores than younger ones (p < 0.005). No statistically significant difference was reported amongst the scoring between male and female CF patients. When different educational levels were compared, patients that had received a higher educational training scored statistically higher in all but one sections of the questionnaire when compared with patients of a lower educational level (p < 0.005). CONCLUSION: More than half Greek adult CF patients report that they are capable to participate in social activities but most of them are worried about the outcome of their disease and its effect on their lives.INTRODUCTION: Recent developments in treatment have steadily raised the median predicted age of survival for people with Cystic Fibrosis (CF). We report the health-related quality of life (HRQoL) in CF adult patients and correlate our findings with the patients’ demographic characteristics. MATERIAL AND METHODS: The Cystic Fibrosis Quality of Life (CFQoL) questionnaire was answered by 77 CF adult patients. The questionnaire included questions pertaining to age, sex and level of education and covered eight sections of functioning. RESULTS: The highest score was reported in the “Social Functioning” section, while the lowest in the “Concerns for the Future” section. When different age groups were compared, statistical significances were reported in “Physical Functioning”, “Interpersonal Relationships”, and the “Career Concerns” section, with older patients reporting statistically higher HRQoL scores than younger ones (p < 0.005). No statistically significant difference was reported amongst the scoring between male and female CF patients. When different educational levels were compared, patients that had received a higher educational training scored statistically higher in all but one sections of the questionnaire when compared with patients of a lower educational level (p < 0.005). CONCLUSION: More than half Greek adult CF patients report that they are capable to participate in social activities but most of them are worried about the outcome of their disease and its effect on their lives

Cord Blood Ischemia-Modified Albumin Levels in Normal and Intrauterine Growth Restricted Pregnancies

Ischemia-modified albumin (IMA) is a sensitive biomarker of cardiac ischemia. Intrauterine growth restriction (IUGR) may imply fetal hypoxia, resulting in blood flow centralization in favour of vital organs (brain, heart, adrenals—“brain sparing effect”). Based on the latter, we hypothesized that cord blood IMA levels should not differ between IUGR and appropriate-for-gestational-age (AGA) full-term pregnancies. IMA was measured in blood samples from doubly-clamped umbilical cords of 110 AGA and 57 asymmetric IUGR pregnancies. No significant differences in IMA levels were documented between AGA and IUGR groups. IMA levels were elevated in cases of elective cesarean section (P = .035), and offspring of multigravidas (P = .021). In conclusion, “brain sparing effect” is possibly responsible for the lack of differences in cord blood IMA levels at term, between IUGR and AGA groups. Furthermore, higher oxidative stress could account for the elevated IMA levels in cases of elective cesarean section, and offspring of multigravidas

Hypoxia/reoxygenation-induced myocardial lesions in newborn piglets are related to interindividual variability and not to oxygen concentration

OBJECTIVE: Evaluation of myocardial histological changes in an experimental animal model of neonatal hypoxiareoxygenation. METHODS: Normocapnic hypoxia was induced in 40 male Landrace/Large White piglets. Reoxygenation was initiated when the animals developed bradycardia (HR <60 beats/min) or severe hypotension (MAP <15 mmHg). The animals were divided into four groups based on the oxygen (O2) concentration used for reoxygenation; groups 1, 2, 3, and 4 received 18%, 21%, 40%, and 100% O2, respectively. The animals were further classified into five groups based on the time required for reoxygenation: A: fast recovery (<15 min); B: medium recovery (15-45 min); C: slow recovery (45-90 min); D: very slow recovery (>90 min), and E: nine deceased piglets. RESULTS: Histology revealed changes in all heart specimens. Interstitial edema, a wavy arrangement, hypereosinophilia and coagulative necrosis of cardiomyocytes were observed frequently. No differences in the incidence of changes were observed among groups 1-4, whereas marked differences regarding the frequency and the degree of changes were found among groups A-E. Coagulative necrosis was correlated with increased recovery time: this condition was detected post-asphyxia in 14%, 57%, and 100% of piglets with fast, medium, and slow or very slow recovery rates, respectively. CONCLUSIONS: The significant myocardial histological changes observed suggest that this experimental model might be a reliable model for investigating human neonatal cardiac hypoxia-related injury. No correlation was observed between the severity of histological changes and the fiO2 used during reoxygenation. Severe myocardial changes correlated strictly with recovery time, suggesting an unreported individual susceptibility of myocardiocytes to hypoxia, possibly leading to death after the typical time-sequence of events

CD44 immunoreactivity in the developing human kidney: a marker of renal progenitor stem cells?

CD44 is a transmembrane adhesion glycoprotein, functioning as a hyaluronan receptor and participating in the uptake and degradation of hyaluronan. Recently, CD44 has been proposed in the adult kidney as a marker of activated glomerular parietal epithelial cells, the putative niche stem cells that, in case of damage to podocytes, might migrate inside the glomerular tuft and undergo transition to podocytes. Here, immunoreactivity for CD44 was tested in 18 human fetuses and newborns with a gestational age ranging from 11 to 39 weeks. CD44 immunoreactivity was observed in all but one developing kidneys, being localized in several renal cell types including intraglomerular, capsular, cortical and medullary interstitial cells and nerve cells. In some cases, CD44 marked scattered cells in nephrogenic subcapsular zone. Our data indicate that CD44 is involved in human nephrogenesis, probably marking a subset of progenitor/stem cells involved in early phases of kidney development and, putatively, in podocyte and/or interstitial cell differentiation

Perinatal Changes of Cardiac Troponin-I in Normal and Intrauterine Growth-Restricted Pregnancies

Intrauterine growth restriction (IUGR) implies fetal hypoxia, resulting in blood flow redistribution and sparing of vital organs (brain, heart). Serum cardiac Troponin-I (cTnI), a well-established marker of myocardial ischaemia, was measured in 40 mothers prior to delivery, the doubly clamped umbilical cords (representing fetal state), and their 20 IUGR and 20 appropriate-forgestational-age (AGA) neonates on day 1 and 4 postpartum. At all time points, no differences in cTnI levels were observed between the AGA and IUGR groups. Strong positive correlations were documented between maternal and fetal/neonatal values (r ≥ .498, P ≤ .025 in all cases in the AGA and r ≥ .615, P ≤ .009 in all cases in the IUGR group). These results may indicate (a) normal heart function, due to heart sparing, in the IUGR group (b) potential crossing of the placental barrier by cTnI in both groups

Reply to Ghirardello et al Letter to the Editor

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“Physiological” renal regenerating medicine in VLBW preterm infants: could a dream come true?

An emerging hypothesis from the recent literature explain how specific adverse factors related with growth retardation as well as of low birth weight (LBW) might influence renal development during fetal life and then the insurgence of hypertension and renal disease in adulthood. In this article, after introducing a brief overview of human nephrogenesis, the most important factors influencing nephron number at birth will be reviewed, focusing on the "in utero" experiences that lead to an increased risk of developing hypertension and/or kidney disease in adult. Since nephrogenesis in preterm human newborns does not stop at birth, but it continues for 4-6 weeks postnatally, a better knowledge of the mechanisms able to accelerate nephrogenesis in the perinatal period, could represent a powerful tool in the hands of neonatologists. We suggest to define this approach to a possible therapy of a deficient nephrogenesis at birth "physiological renal regenerating medicine". Our goal in preterm infants, especially VLBW, could be to prolong the nephrogenesis not only for 6 weeks after birth but until 36 weeks of post conceptual age, allowing newborn kidneys to restore their nephron endowment, escaping susceptibility to hypertension and to renal disease later in life

Breastfeeding reduces the risk of obesity in childhood and adolescence

Childhood obesity has increased dramatically over the last decades, representing one of the most serious public health hazards of the 21st century. Efforts must be made by healthcare professionals to prevent it, as it is associated with short- and long-term risks for physical and mental health and because of the increased possibility to persist during adulthood. From antiquity human breast milk was considered the ideal nourishment for the newborn. Breastfeeding is beneficial for the mother-child dyad. Among others, existing data suggest that it reduces the risk for childhood and adolescence obesity. The mechanisms for this are numerous and include the feeding behavior breastfeeding infants acquire, their growth rate, the ‘early protein hypothesis’, the role of leptin that is found in increased levels in human milk, the dietary choices the breastfed infants make during childhood and adolescence and finally the differences in their bowel flora. Meta-analyses provide sufficient evidence for this protective effect, with a dose-response effect as to the duration of breastfeeding. Healthcare professionals involved in the care of the mother-infant dyad must encourage and support mothers to breastfeed their infants for a long period of time, if obesity were to be prevented. Aim of this review is to provide an account of existing data on the association of breastfeeding and the reduced risk of obesity in childhood and adulthood

To clamp or not to clamp late?

When resuscitating a neonate, one encounters controversial issues and a great deal of confusion. These issues include the question as to when should the umbilical cord be clamped. Clamping and cutting the umbilical cord at birth is the oldest intervention in humans. Even though many aspects of the timing for cord clamping have been clarified, some questions still remain unanswered. If delayed cord clamping is to become a standard approach which timing is the optimal? And depending on the mode of delivery should timing be the same or different for normal vaginal delivery or for caesarean section? What about  the compromised neonate, or the very preterm one? We have more questions than answers. More randomized controlled studies are needed in normal and pathologic neonates, in order to address these questions, before we practice delayed cord clamping with safety for the babies and the mothers