skal prognostycznych należą: Międzynarodowy Wskaźnik Prognostyczny ( International Prognostic Scoring System – IPSS) [ 3 ] oraz dynamiczny IPSS ( Dynamic IPSS – DIPSS) ( Tab. I ) [ 4 ]. Pierwszy z nich stosowany jest u chorych na PMF w chwili rozpoznania, jego odmiana dynamiczna natomiast przeznaczona jest do oceny ryzyka u chorych w dowolnej chwili trwania choroby. Każdy z tych modeli prognozuje całkowity czas przeżycia pacjentów w oparciu o parametry kliniczne, których analiza pozwala na przydzielenie chorych, zgodnie z rekomendacjami IWG-MRT ( International
Dorota Link-Lenczowska and Tomasz Sacha
Shigeaki Kobayashi and Akio Morita
, Aoki N, Fukuhara S, et al. The natural course of unruptured cerebral aneurysms in a Japanese cohort. N Engl J Med. 2012;366(26):2474-82. 31. Greving JP, Wermer MJ, Brown RJ, Morita A, Juvela S, Yonekura M, et al. Development of the PHASES score for prediction of risk of rupture of intracranial aneurysms: a pooled analysis of six prospective cohort studies. Lancet Neurol. 2014;13(1):59-66. 32. Shojima M, Oshima M, Takagi K, Torii R, Hayakawa M, Katada K, et al. Magnitude and role of wall shear stress on cerebral aneurysm: computational
Tetsuya Goto, Chika Ndubuisi, Tatsuya Kobayashi, Takafumi Kiuchi, Kunihiko Kodama, Keiichi Sakai and Kazuhiro Hongo
lesion (upper). Functional MRI by moving foot joint showing that hot spot of lower left and lower right looks almost as a mirror image. Four months later, the tumor rapidly extended to the pyramidal tract, basal ganglia and corpus callosum four months after the fourth surgery ( Figure 3 , upper). She had mild motor aphasia and right hemiparesis: proximal U/E MMT 2, distal U/E MMT 4, lower extremity (L/E) MMT 2. Her Karnofsky performance state score was 70%. Figure 3 MRI before fifth operation revealing that the enhanced lesion invades to the basal
Norbert Grząśko, Krzysztof Jamroziak, Anna Dmoszyńska and Krzysztof Giannopoulos
Przeżycie pacjentów ze szpiczakiem plazmocytowym (ang. multiple myeloma – MM) poprawiło się znacznie w ostatnich kilkunastu latach dzięki wprowadzeniu do terapii nowych leków, takich jak inhibitory proteasomu czy leki immunomodulujące. Pomimo tego MM pozostaje nieuleczalną chorobą o bardzo heterogennym przebiegu i zróżnicowanym rokowaniu. Istnieje grupa pacjentów, których przeżycie sięga ponad 10 lat, a równocześnie wielu chorych nie osiąga nigdy dobrej odpowiedzi na leczenie i żyje krócej niż 3 lata. Aktualnie stosowana klasyfikacja R-ISS (ang. Revised International Scoring System) uwzględnia del(17p), t(4;14) i t(14;16) jako negatywne czynniki ryzyka. Według ostatnich zaleceń Międzynarodowej Grupy Szpiczakowej (ang. International Myeloma Working Group – IMWG) do zmian wysokiego ryzyka należą t(4;14), t(14;16), t(14;20), del(17p), amp(1q) i równoznaczna z nią del(1p) oraz hipodiploidia. W poniższym artykule przedstawiono definicję wysokiego ryzyka cytogenetycznego w MM oraz wpływ nowych terapii na rokowanie i wyniki leczenia u chorych z tej grupy.
Małgorzata Soroka, Maciej Słodki, Ludmiła Bartoszewicz, Izabela Krukiewicz- Ruta, Tadeusz Żurawik and Anna Dobrzańska
Spontaneous premature closure of the human fetal ductus arteriosus is an uncommon event that often results in significant morbidity and mortality. We present a case of a fetus with prenatal previously not detected bone defect that presented with idiopathic intrauterine closure of the ductus arteriosus. A 23-year-old mother at 39 weeks of gestation was admitted to the hospital because of an abnormal findings in four chamber screening view of the fetal heart on routine ultrasonography. The fetal echocardiography showed no detectable flow through the ductus arteriosus. Cesarean section was performed 1 hour later. A female newborn weighing 2640g with Apgar scores of 0, 2, 4 and 6 at 1, 3, 5, and 10 minutes, respectively, was delivered.
Łukasz Sokołowski, Maria Respondek-Liberska, Michał Krekora, Joanna Płużańska and Maciej Słodki
Introduction: The aim of this study was to evaluate the following parameters of fetuses and neonates with omphalocele: the prevalence of coexisting congenital heart defects (CHD), abnormalities in heart function and the impact of coexisting CHD on fetal and neonatal survival. Material and methods: The study group consisted of 69 fetuses with omphalocele diagnosed and monitored at the Department of Prenatal Cardiology in our Institute in the years 2007-2017. The retrospective analisis of patients' data was performed. For statistical analysis we used Chi-square test, t-Student test and U Mann-Whitney test.. Results: In the studied group omphalocele was an isolated defect in 31.9% of the cases (22/69), in 68.1% (47/69) coexisting defects were present, in 49.3% (34/69) the coexisting defect was CHD. The most common CHD coexisting with omphalocele were ventricular septal defect (VSD), double outlet right ventricle (DORV) and atrio-ventricular septal defect (AVSD). Abnormalities of heart function were present in 43.5% (30/69) of fetuses with omphalocele: 23.5% (8/34) with normal heart anatomy and in 62.9% (22/35) with CHD. Statistically significant differences between the group with normal heart anatomy and the group with CHD regarded: Cardiovascular Profile Score (CVPS) (median 10 points vs median 9 points, U Mann-Whitney test p=0.034), neonatal birth weight(mean 3253 g vs median 2700 g, U Mann-Whitney test p=0.003), Apgar score (median 8 vs median 7, U Mann-Whitney test p=0.038) and survival rate until discharge from hospital (85% vs 52.9%, Chi-square test p=0.034). The comparison of data from 2007-2017 with data obtained from similar analysis performed in our center in 1999-2006, revealed significant improvement in the early detection of omphalocele (median 14.5 weeks of gestation vs mean 25.4 weeks of gestation), gestational age of delivery (mean 38 weeks of gestation vs mean 34 weeks of gestation) and survival rate until discharge both in neonates with normal heart anatomy and coexisting CHD (85% and 52.9% vs 70% and 23% respectively) . Conclusions: 1. The presence of coexisting CHD is an important prognostic factor in fetuses and neonates with omphalocele, so early fetal echocardiography should be performed in every case of omphalocele. 2. During the last decade (2007-2017), in contrast to years 1999-2006, we observed significant improvement in early and complete prenatal diagnosis of omphalocele. 3. We observed improvement in strategy of obstetrical management resulting in delivering neonates in a more advanced gestational age both in the group with normal heart anatomy and the group with coexisting CHD.
, Schmidt KG. Cardiac dimensions determined by cross-sectional echocardiography in the normal human fetus from 18 weeks to term. Am J Cardiol 1992;70:1459-1467 28. DeVore GR. Assessing fetal cardiac ventricular function. Semin Fetal Neonatal Med 2005;10:515-41 29. Pasquini L, Mellander M, Seale A, Matsui H, Roughton M, Ho SY, Gardiner HM. Z-scores of the fetal aortic isthmus and duct: an aid to assessing arch hypoplasia. Ultrasound Obstet Gynecol. 2007;29:628-33. Erratum in: Ultrasound Obstet Gynecol 2007;30:366 30
Iwona Strzelecka, Maciej Słodki, Katarzyna Zych-Krekora, Michał Krekora, Mariusz Grzesiak, Iwona Maroszyńska and Maria Respondek-Liberska
S, Neki R, Kurosaki KI, Shiraishi I, Nakai M, Nishimura K, Yoshimatsu J, Ikeda T. Cardiovascular profile score as a predictor of acute intrapartum non-reassuring fetal status in infants with congenital heart defects. J Matern Fetal Neonatal Med. 2016; 14: 1-7. 7. Story L, Pasupathy D, Sankaran S, Sharland G, Kyle P. Influence of birth weight on perinatal outcome in fetuses with antenatal diagnosis of congenital heart disease. J Obstet Gynaecol Res. 2015; 41: 896-903. 8. Anagnostou K, Messenger L, Yates R, Kelsall W. Outcome of
endovascular management. Journal of the Chinese Medical Association . 2011, 74: 134-137. 9. Statile CJ Cnota JF, Gomien S, Divanovic A, Crombleholme T, Michelfelder E. Estimated cardiac output and cardiovascular profile score in fetuses with high cardiac output lesions. Ultrasound Obstet Gynecol. 2013 Jan, 41(1):54-8. 10. Godfrey ME Tworetzky W, Morash D, Friedman KG. Cardiac Findings in the Fetus with Cerebral Arteriovenous Malformation Are Associated with Adverse Outcome. Fetal Diagn Ther. 2017, 41(2):108-114.
Katarzyna Więckowska, Katarzyna Piątek and Maria Respondek-Liberska
INTRODUCTION: Cardiac tumors (CT) have been diagnosed since the eighties of the last century. The three most commonly diagnosed types of CT are rhabdomyomas, teratomas and fibromas. Severe fetal disturbances, such as arrythmias, fetal hydrops, ventricular obstructions, may be associated with the heart tumor.
AIM OF THE STUDY: To present the key role of the echocardiographic examination in the perinatal care and to present some demographic changes in this population of the youngest patients.
MATERIAL AND METHODS: From the Filemaker database of the Department for Diagnosis and Prevention of Congenital Malformations, Polish Mother’s Memorial Hospital & Medical University of Lodz, all cases with prenatal diagnoses of cardiac tumor in singleton pregnancy were compiled over a 22-year period (1993–2015). 33 cases of heart tumors in fetuses had 72 fetal echo examinations. The average number of cases per year was 1,5 (range 1 – 5). The retrospective analysis of the material was performed using the Statistica package. The analysis included the assessment of the cardiovascular condition defined by Cardiovascular Profile Score, location and number of tumors; age, past obstetric history, place of residence of the mother, type of delivery and neonatal follow-up.
RESULTS: The number of fetuses with cardiac tumor cases did not increase during this period. The maternal age was 28,1 years (± 5,2 years) and was decreasing over time. The primigravidae constituted 60% of examined population. The history of past miscarriages was observed in 26% of women. The majority of tumors were detected and diagnosed in the third trimester – at mean 29,6 hbd, but the diagnosis was made earlier in the past decades (regression analysis, p=0,042). The average duration of pregnancy was 36,6 hbd (range: 27-42 hbd). There was no statistical difference between decades in term on pregnancy duration.
The cardiac tumors were more frequent in the female fetuses (71,4% to 28,6%; X2 test; p=0,006). The proportions between the groups of single and multiple tumors changed in last five years – currently the multiple tumors are more frequent (87,5%). The mortality in the group of single tumors was twice as high as in the group of multiple tumors. The percentage of cesarean sections between 1993 and 2005 was 67% and between 2006 and 2015 was 75% (p=0,63). The mortality of neonates with cardiac tumors declined over the period taken into account.
CONCLUSIONS: The perinatal care of the fetuses with cardiac tumors improved over last 20 years. The characteristics of analyzed population had shown that fetal CTs affected healthy, young mothers, more often primigravidae, more often female fetuses. The prevalence of CTs did not increase over time. The gestational age at the time of the diagnosis decreased, thus the period of fetal echo monitoring increased. However, the rate of cesarean sections also increased, which requires further studies and explanations.