scholarly journals Cystás fibrosisban szenvedő nők várandóssága.

2021 ◽  
Vol 162 (28) ◽  
pp. 1129-1136
Author(s):  
László Ságodi ◽  
Enikő Sólyom ◽  
Andrea Almási ◽  
Eszter Csiszér ◽  
Gábor Nagy

Összefoglaló. A cystás fibrosisban szenvedő betegek várható élettartama jelentősen megnőtt az utóbbi évtizedben, egyre több beteg képes saját gyermeket vállalni. Célunk a cystás fibrosisban szenvedő várandós nők perinatalis és anyai történéseinek felmérése saját eseteink és az irodalmi adatok alapján. 14, cystás fibrosisban szenvedő nő 16 várandósságáról számolunk be. Rögzítettük a várandósok életkorát, testtömegét, testmagasságát, testtömegindexét, légzésfunkciós értékeit a graviditás kezdetén és végén. Az anyai átlagéletkor szüléskor 21,6 (18–25) év volt. Az anyák graviditásának kezdetén a testmagasság átlaga 162 (150–175) cm, a testtömeg átlaga 57,6 (42–72) kg, a testtömegindex átlaga 21,4 (19,1–23,2) kg/m2 volt. A graviditás végén a testtömeg átlaga 62 (39–76) kg, a testtömegindex átlaga 23,6 (21,3–24,1) kg/m2 volt. A graviditás alatti súlygyarapodás átlaga 8 (1,5–21,5) kg volt. A légzésfunkciós értékek a graviditás kezdetén 2 betegnél voltak beszűkültek. A graviditás alatt még 2 beteg légzésfunkciós értékei csökkentek. A sikeres graviditások száma 13 volt. 1 anya kétszer szült. A koraszülések száma 1 volt. A várandósság átlagosan a 38. (34–40.) gestatiós hét után 7 esetben császármetszéssel, 6 esetben hüvelyi szüléssel fejeződött be. A vetélések száma 3 volt. Az Apgar-pontszám minden esetben normális volt. 13 gyermek közül 11-nél a verejtékteszt nem volt emelkedett. 2 gyermeknél magas verejtékértékek voltak, egyikük c.1521_1523delCTT-heterozigóta, a másiknál génmutációt nem tudtunk igazolni. A cystás fibrosisban szenvedő nők általában jól tolerálják a várandósságot az esetek többségében. A kórosan beszűkült tüdőfunkcióval, alacsony tápláltsági állapottal és cukorbetegséggel rendelkező nők nagyobb valószínűséggel számíthatnak káros következményekre. Az újszülöttek prognózisa általában jó, de számítani kell a koraszülés és a kis súllyal születés gyakoribb előfordulására. Ideális esetben a várandósságot előzetes tanácsadás útján kell megtervezni, és speciális cystás fibrosis csoportnak kell a várandósok ellátását figyelemmel kísérni, ideértve a cystás fibrosis kezelésében jártas szülészeket is. Kisszámú saját adatunk retrospektív elemzése megerősíti az irodalmi adatok tanúságait. Orv Hetil. 2021; 162(28): 1129–1136. Summary. The life expectancy of patients with cystic fibrosis has increased significantly in the last decade, with more and more patients being able to have their own children. The aim of our study was to assess the perinatal and maternal outcome of pregnant women with cystic fibrosis based on our own cases and literature data. We report 16 pregnancies in 14 women with cystic fibrosis. We recorded the age, body weight, height, body mass index, and respiratory function values of pregnant women at the beginning and end of pregnancy. The mean maternal age at childbirth was 21.6 (18–25) years. At the beginning of maternal pregnancy, the mean height was 162 (150–175) cm, the mean body weight was 57.6 (42–72) kg, and the mean body mass index was 21.4 (19.1–23.2) kg/m2. At the end of pregnancy, the mean body weight was 62 (39–76) kg and the mean body mass index was 23.6 (21.3–24.1) kg/m2. The weight gain under pregnancy was mean 8 (1.5–21.5) kg. The respiratory function values at the onset of pregnancy were narrowed in 2 patients. During pregnancy, the respiratory function values of 2 more patients decreased. The number of successful gestations was 13. A mother gave birth twice. The number of premature births was one. The pregnancy after the mean 38. (34–40.) gestational week was completed in 7 cases by cesarean section and in 6 cases by vaginal delivery. The number of miscarriages was 3. The Apgar score was normal in all cases. In 11 of 13 children, the sweat test was not elevated. 2 children had high sweat values, one of them is heterozygous with c.1521_1523delCTT, the other could not prove a gene mutation. Women with cystic fibrosis generally tolerate pregnancy well, in most cases. Women with poor lung function, low nutritional status, and diabetes are more likely to expect adverse consequences. The outcome of the newborns is good in general, but a common occurrence of premature birth and low birth weight is to be expected. Ideally, pregnancy should be planned through prior counseling and the care of pregnant women should be monitored by a specialized cystic fibrosis team, including obstetricians experienced in the treatment of cystic fibrosis. A retrospective analysis of our own small-number data confirms the evidence from the literature data. Orv Hetil. 2021; 162(28): 1129–1136.

2017 ◽  
Vol 117 (11) ◽  
pp. 1808-1815 ◽  
Author(s):  
Francis M. Hollander ◽  
Nicole M. de Roos ◽  
Gerdien Belle van Meerkerk ◽  
Ferdinand Teding van Berkhout ◽  
Harry G.M. Heijerman ◽  
...  

Children ◽  
2022 ◽  
Vol 9 (1) ◽  
pp. 110
Author(s):  
Sun-Jin Lee ◽  
Sun-Young Kim ◽  
Minsun Kim

Gonadotropin-releasing hormone agonist (GnRHa) therapy is used to control puberty progression and it preserves height potential in patients with idiopathic central precocious puberty (ICPP). This study evaluated the correlation between weight and height gain at menarche following GnRHa treatment among girls with ICPP and relatively central early puberty (EP). We investigated height/weight trends and changes in height from diagnosis to menarche in girls with ICPP and EP treated with GnRHa. The mean difference in height (Δheight) from treatment cessation to menarche was 9.79 ± 3.53 cm. Girls were divided into girls with Δheight ≥ 9.79 cm (Group 1) and girls with Δheight < 9.79 cm (Group 2). Although near adult height was significantly higher in Group 1, the mean body mass index (BMI) and weight were significantly lower at diagnosis, treatment discontinuation, and menarche. The BMI and weight at the three time points were negatively correlated with height. Girls with higher BMI at all three time points had slower growth rates during the study period. Considering that BMI and body weight were closely related to Δheight, proper management of BMI and body weight of girls receiving early puberty treatment might contribute to growth during and after GnRHa treatment.


2015 ◽  
Vol 9 (1) ◽  
pp. 46-51 ◽  
Author(s):  
J Minov ◽  
J Karadzinska-Bislimovska ◽  
K Vasilevska ◽  
S Stoleski ◽  
D Mijakoski

Introduction : Non-cystic fibrosis bronchiectasis (NCFB) is a multidimensional disease, and no single isolated parameter is proved to have sufficient power for any overall determination of its severity and prognosis. Objective : To compare the results of the assessment of the NCFB severity with respect to its prognosis in the same patients by two different validated scores, i.e. the FACED score and the Bronchiectasis Severity Index (BSI). Methods : An observational study including 37 patients with NCFB (16 males and 21 female aged 46 to 76 years) was performed. All patients underwent evaluation of the variables incorporated in the FACED score (FEV1 % predicted, age, chronic colonization by Pseudomaonas aeruginosa, radiological extent of the disease, and dyspnea) and in the BSI (age, body mass index, FEV1 % predicted, hospitalization and exacerbations in previous year, dyspnea, chronic colonization by Pseudomaonas aeruginosa and other microrganisms, and radiological extent of the disease). Results : According to the value of the derived overall FACED score we found 17 patients (45.9%) with mild bronchiectasis, 14 patients (37.8%) with moderate bronchiectasis and 6 patients (16.2%) with severe bronchiectasis. The mean derived FACED score was 3.4 ± 1.3. In addition, according to the value of the derived overall BSI score, the frequency of patients with low, intermediate and high BSI score was 16 patients (43,2%), 14 patients (37.8%) and 7 patients (18.9%), respectively. The mean derived BSI score was 6.4 ± 2.5. Conclusion : We found similar results by the assessment of the NCFB severity in regard to its prognosis by both the FACED score and the BSI. Further studies determining how these scores may impact clinical practice are needed.


2018 ◽  
Vol 28 (2) ◽  
pp. 29566
Author(s):  
Magali Santos Lumertz ◽  
Ângela De Moura ◽  
Leonardo Araújo Pinto ◽  
Paulo Augusto Moreira Camargos ◽  
Paulo José Cauduro Marostica

AIMS: To compare clinical, laboratory and spirometric parameters of preschoolers (ages from three to six years old) with cystic fibrosis identified by abnormal newborn screening test (NS group), with data of patients whose diagnostic suspicion arose from characteristic clinical signs and symptoms (CS group).METHODS:  Retrospective cohort study, with a sample obtained from the medical charts of patients who received specialized and multidisciplinary outpatient follow-up. All the included children had a confirmed diagnosis of cystic fibrosis by sweat electrolyte testing and/or genetic sequencing. Variables included sex, weight, height, body mass index, number of hospitalizations per year, Pseudomonas aeruginosa colonization, presence of pancreatic insufficiency and Shwachman score, which covers four domains: general activity, nutrition, radiological examination and physical evaluation, each one scored between 5 and 25 points. A total score ≤40 points indicates severe state, and ≥86 points indicates excellent state. For comparison of pulmonary function, data from the last spirometry performed before the age of six years were used. Outcomes were compared by chi-square or Student's t test, with a significance limit of 0.05.RESULTS: Twenty-four patients were included in the study, seven children in the NS group and 17 children in the CS group. The children of the NS group started follow-up with a mean age of 1.51±2.04 months, and those of the CS group started with 3.77±1.80 months (p=0.014). The mean Shwachman score was 94.40±2.19 in the NS vs. 87.67±8.00 in the CS group (p=0.018). The mean Z score for body mass index was 0.95±1.02 in the NS group vs. 0.51±1.05 in the CS group (p=0.051). Spirometric variables had slightly higher values in the NS group, with no statistically significant difference between groups.CONCLUSIONS: The results suggest that those patients diagnosed with cystic fibrosis from the neonatal screening test suspicion benefited from an early intervention, being able to initiate pulmonary function tests and receive treatment and counseling earlier. Evaluation of clinical parameters with the Shwachman score showed that benefits could be already observed at the preschool age.


2019 ◽  
Vol 35 (5) ◽  
Author(s):  
Resmiye Özdilek ◽  
Yılda Arzu Aba ◽  
Sena Dilek Aksoy ◽  
Bulat Aytek Şık ◽  
Yaşam Kemal Akpak

Objective: To investigate the adaptation of pregnant women to the recommended weight gain range according to body mass index (BMI) and to determine the factors affecting them. Methods: This cross-sectional study was performed in a university hospital’s obstetrics and gynecology unit (tertiary center) in Turkey. This study was conducted between March 2018 and August 2018 (6 months) in pregnant women. Pregnant women with chronic disease and receiving treatment during antenatal follow-up, with twin pregnancy, with a fetus with a congenital abnormality, and nutritional disturbance were excluded from the study. Eight hundred twelve pregnant women with normal antenatal follow-up and who volunteered to participate were included in the study. Results: The mean age of the participants was 27.66±5.05 years. The mean weight and BMI before pregnancy were near standard in all participants. The group with the highest rate of recommended weight gain according to BMI before pregnancy was the group with low weight pregnant women. The ideal weight gain rate in all groups was 32%. Conclusions: The groups with overweight and obese pregnant women according to BMI before pregnancy had the highest rates of weight gain, above the recommended limits. BMI before pregnancy directly affects weight gain during pregnancy and the importance of pre-pregnancy counseling and weight loss is emphasized once again. doi: https://doi.org/10.12669/pjms.35.5.133 How to cite this:Ozdilek R, Aba YA, Aksoy SD, Sik BA, Akpak YK. The relationship between body mass index before pregnancy and the amount of weight that should be gained during pregnancy: A cross-sectional study. Pak J Med Sci. 2019;35(5):---------.  doi: https://doi.org/10.12669/pjms.35.5.133 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


2021 ◽  
Vol 2 (1) ◽  
pp. 43-47
Author(s):  
Javier Sánchez-Romero

The global epidemic of obesity also affects pregnant women. External cephalic version is a procedure for achieving a cephalic presentation. Obesity has been associated with lower rates of ECV success. The main objective of this article is to analyze the trends in BMI in pregnant women who underwent ECV. A descriptive retrospective analysis of ECV performed in Hospital Clinico Universitario Virgen de la Arrixaca in Murcia (Spain) between 1st of January of 2014 and 31st of December 2018 has been carried out. 417 patients were recruited. The mean maternal BMI was 25.5 kg/m2 at 36 week’s gestation. If BMI is categorized, 2.6% (N=11) of the patients had a BMI below 18.5 kg/m2, 51.1% (N=213) had a BMI between 18.5-24.9 kg/m2, 30.9% (N=129) of the patients had a BMI between 25-29.9 kg/m2 and 14.0% (N=64) had a BMI above 30 kg/ m2. Mean maternal BMI had statistically significant changes between 2014 and 2018 (p<0.001). Maternal BMI has increased over the years in patients who undergo ECV. In patients who undergo ECV, maternal BMI should be related to socio-economic inequalities and social behavior in future researches. It should be also associated with maternal BMI of pregnant women who undergo ECV with ECV success rate and with the type of delivery of those pregnancies. Keywords: External Cephalic Version; Body Mass Index; Pregnant; Obstetrics; Obesity; Overweight. Abbreviations ECV: External Cephalic Version; BMI: Body Mass Index.


2004 ◽  
Vol 101 (3) ◽  
pp. 603-613 ◽  
Author(s):  
Kinichi Shibutani ◽  
Mario A. Inchiosa ◽  
Keisuke Sawada ◽  
Mosses Bairamian

Background The currently available pharmacokinetic models for fentanyl were derived from normal weight patients and were not scaled to body weight. Their application to obese patients may cause overprediction of the plasma concentration of fentanyl. This study examined the influence of body weight on the predictive accuracy of two models (Anesthesiology 1990; 73:1091-102 and J Pharmacol Exp Ther 1987; 240:159-66). Further, we attempted to derive suggested dosing mass weights for fentanyl that improved predicted accuracy. Method Seventy patients undergoing major elective surgery with total body weight (TBW) &lt;85 kg and body mass index &lt;30 (Group L) and 39 patients with TBW &gt;/=85 kg and body mass index &gt;30 (Group O) were studied. In Group L and Group O, the mean TBW was 69 kg, and 125 kg, respectively and the mean body mass index in Group L and Group O was 24 and 44, respectively. Fentanyl infusion was used during surgery and postoperatively for analgesia. Plasma fentanyl concentrations were measured and predicted concentrations were obtained by computer simulation; 465 pairs of measured and predicted values were obtained. Results The influence of TBW on the performance errors of the original two models was examined with nonlinear regression analysis. Shafer error versus TBW showed a highly significant negative relationship (R squared = 0.689, P &lt; 0.001); i.e., the Shafer model systematically overestimated fentanyl concentration as weight increased. The Scott and Stanski model showed greater variation (R squared = 0.303). We used the exponential equation for Shafer performance error versus TBW to derive suggested dosing weights ("pharmacokinetic mass") for obese patients. The pharmacokinetic mass versus TBW curve was essentially linear below 100 kg (with slope of 0.65) and approached a plateau above 140 kg. For patients weighing 140 to 200 kg, dosing weights of 100-108 kg are projected. Total body clearance (ml/min) showed a strong linear correlation with pharmacokinetic mass (r = 0.793; P &lt; 0.001), whereas the relationship with TBW was nonlinear. Conclusion Actual body weight overestimates fentanyl dose requirements in obese patients. Dosing weight (pharmacokinetic mass) derived from the nonlinear relationship between prediction error and TBW proved to have a linear relationship with clearance.


2017 ◽  
Vol 16 ◽  
pp. S149 ◽  
Author(s):  
F.M. Hollander ◽  
N.M. De Roos ◽  
G. Belle-van Meerkerk ◽  
F. Teding van Berkhout ◽  
E.A. van de Graaf

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