Pregnancy in women with cystic fibrosis: 14 case reports and literature review

Osszefoglalo. A cystas fibrosisban szenvedő betegek varhato elettartama jelentősen megnőtt az utobbi evtizedben, egyre tobb beteg kepes sajat gyermeket vallalni. Celunk a cystas fibrosisban szenvedő varandos nők perinatalis es anyai torteneseinek felmerese sajat eseteink es az irodalmi adatok alapjan. 14, cystas fibrosisban szenvedő nő 16 varandossagarol szamolunk be. Rogzitettuk a varandosok eletkorat, testtomeget, testmagassagat, testtomegindexet, legzesfunkcios ertekeit a graviditas kezdeten es vegen. Az anyai atlageletkor szuleskor 21,6 (18-25) ev volt. Az anyak graviditasanak kezdeten a testmagassag atlaga 162 (150-175) cm, a testtomeg atlaga 57,6 (42-72) kg, a testtomegindex atlaga 21,4 (19,1-23,2) kg/m2 volt. A graviditas vegen a testtomeg atlaga 62 (39-76) kg, a testtomegindex atlaga 23,6 (21,3-24,1) kg/m2 volt. A graviditas alatti sulygyarapodas atlaga 8 (1,5-21,5) kg volt. A legzesfunkcios ertekek a graviditas kezdeten 2 betegnel voltak beszűkultek. A graviditas alatt meg 2 beteg legzesfunkcios ertekei csokkentek. A sikeres graviditasok szama 13 volt. 1 anya ketszer szult. A koraszulesek szama 1 volt. A varandossag atlagosan a 38. (34-40.) gestatios het utan 7 esetben csaszarmetszessel, 6 esetben huvelyi szulessel fejeződott be. A vetelesek szama 3 volt. Az Apgar-pontszam minden esetben normalis volt. 13 gyermek kozul 11-nel a verejtekteszt nem volt emelkedett. 2 gyermeknel magas verejtekertekek voltak, egyikuk c.1521_1523delCTT-heterozigota, a masiknal genmutaciot nem tudtunk igazolni. A cystas fibrosisban szenvedő nők altalaban jol toleraljak a varandossagot az esetek tobbsegeben. A korosan beszűkult tudőfunkcioval, alacsony taplaltsagi allapottal es cukorbetegseggel rendelkező nők nagyobb valoszinűseggel szamithatnak karos kovetkezmenyekre. Az ujszulottek prognozisa altalaban jo, de szamitani kell a koraszules es a kis sullyal szuletes gyakoribb előfordulasara. Idealis esetben a varandossagot előzetes tanacsadas utjan kell megtervezni, es specialis cystas fibrosis csoportnak kell a varandosok ellatasat figyelemmel kiserni, ideertve a cystas fibrosis kezeleseben jartas szuleszeket is. Kisszamu sajat adatunk retrospektiv elemzese megerősiti az irodalmi adatok tanusagait. 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.