Perinatalna opieka paliatywna realizowana w oddziale polozniczym i neonatologicznym we wspólpracy z hospicjum dla dzieci - doswiadczenia wlasne.

INTRODUCTION: Wady letalne prowadza do wewnatrzmacicznego zgonu plodu lub dziecka bezposrednio po urodzeniu lub we wczesnym okresie niemowlecym, bez wzgledu na zastosowane leczenie. W przypadku wad letalnych nie ma mozliwosci skutecznej pomocy dziecku, mimo postepu mi zeadsytcoysnoyw ania najnowoczesniejszej aparatury lub terapii. Rodzice, którzy decyduja sie na urodzenie dziecka z wada letalna moga byc objeci perinatalna opieka hospicyjna, która ma charakter kompleksowy. Polega ona na wsparciu ciezarnej w okresie przygotowania do porodu, w czasie porodu i po porodzie oraz na wsparciu jej rodziny oraz obejmuje udzielenie rodzicom pelnej informacji o chorobie ich dziecka. Opieka nad dzieckiem po urodzeniu jest nastawiona na ochrone przed uporczywa terapia i zapewnienie dziecku opieki paliatywnej. CEL: Wykazanie znaczenia perinatalnej opieki paliatywnej dla kobiet w ciazy, u których wyniki badan prenatalnych wskazywaly na ciezkie zaburzenie rozwojowe u plodu o potencjalnie letalnym rokowaniu oraz przedstawienie schematu postepowania wedlug modelu wewnatrzszpitalnego hospicjum perinatalnego. MATERIAL I METODY: Analiza retrospektywna objeto dokumentacje 67 pacjentek skierowanych do Programu RAZEM we Wroclawiu w latach 2014-2018 z powodu nieprawidlowych wyników badan prenatalnych (ultrasonograficznych lub/i genetycznych), które wskazywaly na ciezkie zaburzenie rozwojowe u plodu o potencjalnie letalnym rokowaniu. Dokonanoanalizy danych socjodemograficznych, danych klinicznych rozpoznania choroby u plodu, przebiegu ciazy i porodu, trybu postepowania w okresie prenatalnym, podczas porodu i po urodzeniu sie dziecka. WYNIKI: Do Programu zostalo skierowanych 67 kobiet w wieku 20-43 lat (srednio 31,2), które zglaszaly sie w okresie od 15 do 39 tygodnia ciazy (srednio w 25. tygodniu ciazy). Do opieki paliatywnej zakwalifikowano 57 kobiet, czyli 85% skierowanych do programu. Opieke paliatywna kontynuowano u 51 pacjentek, poniewaz 6 kobiet w trakcie procesu diagnostycznego zdecydowalo sie na zakonczenie ciazy (10,5%). Najczestszymi zaburzeniami u plodów byly aberracje chromosomowe, wady OUN i wady nerek. W 95% przypadków doszlo do obumarcia wewnatrzmacicznego plodu lub smierci noworodka. WNIOSKI: Perinatalna opieka paliatywna jest niezbedna forma opieki dla kobiet w ciazy, u których wyniki badan prenatalnych wskazuja na ciezkie zaburzenie rozwojowe u plodu o potencjalnie letalnym rokowaniu. Model wewnatrzszpitalny hospicjum perinatalnego jest korzystna forma opieki, zapewnia jej spójnosc i dobra komunikacje w zespole, co wplywa na dobra jakosc opieki. INTRODUCTION: Lethal defects lead to the intrauterine death of the fetus or the passing away of the child immediately after birth or in early infancy, regardless of the treatment used. In the case of lethal defects, it is not possible to effectively help the child, despite using the most modern equipment or medicines in the treatment or the progress made by medicine. Parents, who decide to continue the pregnancy, although the fetus has a lethal defect that cannot be cured, may be covered by perinatal hospice care, which is comprehensive and consists in supporting the pregnant woman during the prenatal time, during delivery and after delivery and support of her family, giving full information to the parents about their child's illness. Childcare after birth is focused on protecting the infant from persistent therapy and providing him with appropriate conditions. AIM: To demonstrate the role of perinatal palliative care for pregnant women in whom the results of prenatal tests pointed to a severe developmental disorder in the fetus with a potentially lethal prognosis, and to present a pattern of behavior for their hospitalization in the perinatal hospice. MATERIALS AND METHODS: The retrospective analysis included documentation of 67 patients referred to the RAZEM (TOGETHER) Program in Wroclaw in 2014-2018 due to abnormal results of (ultrasound and / or genetic) prenatal tests, which indicated a serious developmental disorder in the fetus with potentially lethal prognosis. Analysis was conducted of sociodemographic data, clinical data on fetal diagnosis, pregnancy and delivery, the procedure for prenatal delivery and postnatal birth. RESULTS: 67 women aged 20-43 years (mean 31.2) were referred to the RAZEM Program. Out of these, 57 women were enrolled for palliative care, which accounted for 85% of those referred to the program. Palliative care was continued in 51 patients, because 6 women decided to terminate their pregnancy during the diagnostic process (10.5%). The most common abnormalities in the fetuses were chromosomal aberrations, CNS defects and kidney defects. In 95% of the cases, intrauterine fetal death or neonatal death occurred. CONCLUSIONS: Perinatal palliative care is an indispensable form of care for pregnant women in whom the results of prenatal tests indicate a serious developmental disorder in the fetus with potentially lethal prognosis. The in-hospital model of a perinatal hospice is a beneficial form of care, as it ensures consistency and good communication in the team, which favourably affects its quality.

[Perinatal palliative care performed in obstetrics and neonatology wards and hospices for children - own experience].

OBJECTIVE: Lethal defects lead to the intrauterine death of the fetus or the passing away of the child immediately after birth or in early infancy, regardless of the treatment used. In the case of lethal defects, it is not possible to effectively help the child, despite using the most modern equipment or medicines in the treatment or the progress made by medicine. Parents, who decide to continue the pregnancy, although the fetus has a lethal defect that cannot be cured, may be covered by perinatal hospice care, which is comprehensive and consists in supporting the pregnant woman during the prenatal time, during delivery and after delivery and support of her family, giving full information to the parents about their child's illness. Childcare after birth is focused on protecting the infant from persistent therapy and providing him with appropriate conditions. Aim: To demonstrate the role of perinatal palliative care for pregnant women in whom the results of prenatal tests pointed to a severe developmental disorder in the fetus with a potentially lethal prognosis, and to present a pattern of behavior for their hospitalization in the perinatal hospice. PATIENTS AND METHODS: Materials and methods: The retrospective analysis included documentation of 67 patients referred to the RAZEM (TOGETHER) Program in Wroclaw in 2014-2018 due to abnormal results of (ultrasound and / or genetic) prenatal tests, which indicated a serious developmental disorder in the fetus with potentially lethal prognosis. Analysis was conducted of sociodemographic data, clinical data on fetal diagnosis, pregnancy and delivery, the procedure for prenatal delivery and postnatal birth. RESULTS: Results: 67 women aged 20-43 years (mean 31.2) were referred to the RAZEM Program. Out of these, 57 women were enrolled for palliative care, which accounted for 85% of those referred to the program. Palliative care was continued in 51 patients, because 6 women decided to terminate their pregnancy during the diagnostic process (10.5%). The most common abnormalities in the fetuses were chromosomal aberrations, CNS defects and kidney defects. In 95% of the cases, intrauterine fetal death or neonatal death occurred. CONCLUSION: Conclusions: Perinatal palliative care is an indispensable form of care for pregnant women in whom the results of prenatal tests indicate a serious developmental disorder in the fetus with potentially lethal prognosis. The in-hospital model of a perinatal hospice is a beneficial form of care, as it ensures consistency and good communication in the team, which favourably affects its quality.

Feasibility of implementation of a "simplified, No-X-Ray, no-lead apron, two-catheter approach" for ablation of supraventricular arrhythmias in children and adults.

INTRODUCTION: Although the "near-zero-X-Ray" or "No-X-Ray" catheter ablation (CA) approach has been reported for treatment of various arrhythmias, few prospective studies have strictly used "No-X-Ray," simplified 2-catheter approaches for CA in patients with supraventricular tachycardia (SVT). We assessed the feasibility of a minimally invasive, nonfluoroscopic (MINI) CA approach in such patients. METHODS: Data were obtained from a prospective multicenter CA registry of patients with regular SVTs. After femoral access, 2 catheters were used to create simple, 3D electroanatomic maps and to perform electrophysiologic studies. Medical staff did not use lead aprons after the first 10 MINI CA cases. RESULTS: A total of 188 patients (age, 45 ± 21 years; 17% <19 years; 55% women) referred for the No-X-Ray approach were included. They were compared to 714 consecutive patients referred for a simplified approach using X-rays (age, 52 ± 18 years; 7% <19 years; 55% women). There were 9 protocol exceptions that necessitated the use of X-rays. Ultimately, 179/188 patients underwent the procedure without fluoroscopy, with an acute success rate of 98%. The procedure times (63 ± 26 vs. 63 ± 29 minutes, P > 0.05), major complications (0% vs. 0%, P > 0.05) and acute (98% vs. 98%, P > 0.05) and long-term (93% vs. 94%, P > 0.05) success rates were similar in the "No-X-Ray" and control groups. CONCLUSIONS: Implementation of a strict "No-X-Ray, simplified 2-catheter" CA approach is safe and effective in majority of the patients with SVT. This modified approach for SVTs should be prospectively validated in a multicenter study.

Less invasive percutaneous closure of persistent arterial duct in children below 10 kilos.

INTRODUCTION: There is quite high risk of vascular complications connected with arterial puncture - mainly in the group of the smallest patients weighing less than 10 kg. AIM: To evaluate a new method of percutaneous closure of persistent arterial duct (PDA) in small children. MATERIAL AND METHODS: Six patients were enrolled in this method of PDA closure at the age of 10.67 ±1.97 months. The mean weight was 8.85 ±0.66 kg. The occluders were implanted using only venous access. The position of the occluder was determined by angiography in the pulmonary artery through the delivery system and by echocardiography. RESULTS: The procedure was effective in all six cases but we managed to proceed in the planned transvenous method in five cases. There was one case of complete duct constriction after introduction of the catheter, so precise measurements were impossible. After removal of the catheter the constriction remitted completely. The duct was closed in the standard manner. During echocardiographic examination we found complete closure of all six arterial ducts directly after the procedures and in the follow-up (9.17 ±3.02 months). No stenosis within the pulmonary arteries or aorta were found. CONCLUSIONS: Transcatheter arterial duct occlusion without arterial puncture is an effective and safe method of treatment allowing one to reduce the complications connected with arterial puncture. A possible complication of this method may be a constriction of the duct walls as a result of a larger amount of manipulations.

[Right atrial thrombus following surgical closure of atrial septal defect--a case report]. / Skrzeplina w prawym przedsionku po operacyjnym zamknieciu ubytku w przegrodzie miedzyprzedsionkowej--opis przypadku.

A case of an 11-year-old girl with atrial septal defect is described. The patient underwent surgical closure of the defect, which was complicated by asymptomatic thrombus formation in the right atrium. Anticoagulant therapy was initially successful, but thrombus recurred. Surgical thrombus removal was successful.

Difficult to treat recurrent stenosis of the aorta.

The risk associated with repeated treatment of aortic stenosis is as high as 5% and increases to as much as 25% in complex heart diseases. Among the methods that are commonly accepted and used in the treatment of recurrent aortic stenosis are balloon dilatation and stent implantation. In this study we describe five patients with recurrent stenosis of the aorta treated with stent implantation. The short-term results of such treatment are promising. However, in some cases it is only palliative in character and does not completely resolve the problems arising from congenital heart disease. (Cardiol J 2007; 14: 186-192).

[Influence of age on treatment results in children and adolescents with Hodgkin's disease]. / Wplyw wieku na wyniki leczenia dzieci i mlodziezy z choroba Hodgkina.

Over the last few years, treatment failures (progression, relapse) in Hodgkin's disease (HD) occurred mainly in elder children treated in the Polish Pediatric Leukemia/Lymphoma Study Group (PPLLSG) participating centers. That is why analysis of the influence of age on the treatment outcome in children and adolescents treated with the protocol introduced in 1997 was performed. In years 1997-2001, in 10 centers of PPLLSG, 280 patients (age 2.3-18.8 years) were treated for HD. In all patients MVPP and B-DOPA chemotherapy with or without radiotherapy was introduced. Among 280 treated children the first remission was achieved in 275 patients (98.2%). Relapses occurred in 11 patients (4%). The 5-year probability of overall survival, relapse-free survival (RFS) and event-free survival (EFS) was 99%, 93% and 90%, respectively. All children with relapse were over 10 years old at the time of diagnosis (range: 10.6-17.1, median 14.6 years); mediastinal tumor mass was present in all of them. The logistic regression analysis did not reveal the border value for increasing the probability of relapse or event, thus age of 10 years (age of the youngest child with relapse) was identified as the border value. The probability of 5-year EFS and RFS for children over and under the 10th year of age was 98%, 92% and 100%, 92%, respectively. The differences were not statistically significant. Among children over the 10th year of age some features of the disease occurred more frequently: female sex, shorter history of the disease, presence of mediastinal tumor, greater stage of the disease, NS histopathological type of the disease, presence of general signs and ESR over 50 mm, greater tumor burden and higher number of involved lymphatic regions. Among the patients over the 10th year of age, the presence of the general signs and mediastinal tumor influenced the occurrence of relapses substantially. The aim of the further treatment modifications ought to comprise the need of better treatment outcome, especially in patients over the 10th years of age in which unfavorable prognostic factors are identified. child with relapse) was identified as the border value. The probability of 5-year EFS and RFS for children over and under the 10th year of age was 98%, 92% and 100%, 92%, respectively. The differences were not statistically significant. Among children over the 10th year of age some features of the disease occurred more frequently: female sex, shorter history of the disease, presence of mediastinal tumor, greater stage of the disease, NS histopathological type of the disease, presence of general signs and ESR over 50 mm, greater tumor burden and higher number of involved lymphatic regions. Among the patients over the 10th year of age, the presence of the general signs and mediastinal tumor influenced the occurrence of relapses substantially. The aim of the further treatment modifications ought to comprise the need of better treatment outcome, especially in patients over the 10th years of age in which unfavorable prognostic factors are identified.

[Over 30-year experience of Polish Pediatric Leukemia/Lymphoma Study Group for treatment of Hodgkin's disease in children and adolescents: improvement curability and decrease of serious complications]. / Ponad 30-letnie doswiadczenie Polskiej Grupy ds. Leczenia Bialaczek i Chloniaków w leczeniu choroby Hodgkina u dzieci i mlodziezy: poprawa wyleczalnosci i zmniejszenie powaznych powiklan.

Currently over 90% of children with HD can be cured thanks to use of chemotherapy (CT) combined with involved field radiotherapy (IF-RT). From 1971 to 2001, 1062 children and adolescents with HD (stage I to IV) were treated in 10 oncological centers PPLLSG. Majority of patients were treated with CT combined with IF-RT. Year by year the intensity of therapy (CT and RT) was gradually adjusted to the risk-factor groups, and invasive methods of staging were also gradually limited. Supportive care was improved at the same time. Along with the modified therapy protocol, five consecutive periods of time (I: 1971-82; II: 1983-87; IIII: 1988-93; IV: 1994-96; V: 1997-2001) were analyzed. In the first period the basic CT was MVPP (mechlorethamine, vinblastine, procarbazine, prednisone), while later B-DOPA (bleomycin, dacarbazine, vincristine, prednisone, doxorubicin) was gradually introduced alone or alternately with MVPP. In order to decrease the incidence of late complications, the dose of IF-RT from 45 Gy to 15-30 Gy was reduced in the next periods. In V period in 21 children with stage IA and IIA with favorable prognostic factors, CT alone was used. The probability of over 5-year: overall survival, relapse free survival and event free survival were in the I period: 92%, 83% and 77%, in the II period: 97%, 92% and 89%, in the III period: 97%, 93% and 91%, in the IV period: 95%, 91% and 90%, in the V period: 95%, 95% and 93%. Decreased rate of serious complications was also observed. Intensity of therapy should be tailored to the stage of disease, and to other significant prognostic factors. The current strategy of diagnosing and treatment of HD is aimed at balancing between the highest possible cure rates and risk of late complications.

In vitro activity of glufosfamide in childhood acute leukemia.

Glufosfamide is a new agent for cancer chemotherapy. The objective of the study was the comparison of the in vitro drug resistance profile of glufosfamide with other oxazaphosphorines in 106 samples of childhood acute leukemia by means of the MTT assay. The following drugs were tested: glufosfamide, 4-HOO-ifosfamide, 4-HOO-cyclophosphamide, mafosfamide cyclohexylamine salt, prednisolone, vincristine, L-asparaginase, daunorubicin and cytarabine. In the group of initial Acute Lymphoblastic Leukemia (ALL) samples, equivalent cytotoxicity values for glufosfamide, 4-HOO-ifosfamide, 4-HOO-cyclophosphamide and mafosfamide were 5.95, 9.92, 4.60 and 3.90 microg/ml, respectively. In comparison to initial ALL samples, the relative resistance for glufosfamide and 4-HOO-ifosfamide in relapsed ALL samples were 1.9 (p=0.049) and 1.3 (ns), and in initial Acute Myeloblastic Leukemia (AML) samples, respectively, 31 (p<0.001) and 5 (p=0.001). All oxazaphosphorines showed highly significant cross-resistance. In conclusion, in vitro activity of glufosfamide is comparable to ifosfamide. Glufosfamide shows high activity against lymphoblasts both on diagnosis and on relapse, however it cannot circumvent resistance to other oxazaphosphorines.