Prader‐Willi syndrome (PWS) is a complex genetic disorder with three molecular classes but clinical ascertainment is based on distinctive features. The prevalence of dysmorphic features was studied in 355 PWS participants (61% deletion, 36% maternal disomy [UPD], and 3% imprinting defects) from the National Institute of Health PWS Rare Diseases Clinical Research Network. The effect of growth hormone (GH) treatment on growth and dysmorphic features was compared. Among participants, upslanting palpebral fissures were seen in 23%; strabismus in 42%; abnormal dentition in 32%; small hands in 63% and small feet in 70%; hypopigmentation in 30%; striae in 32% and skin picking in 26%. Compared to those with UPD, participants with deletions were found to be heavier (p = 0.002), had smaller head circumference (HC) (p = 0.009), higher incidence of a flat occiput (p = 0.005); low‐anterior hairline (p = 0.04); abnormal dentition (p = 0.009); abdominal striae (p = 0.045), nail abnormalities (p = 0.050), and fair‐haired (p < 0.001). Participants in both genetic groups receiving GH were taller (p = 0.005), had larger HCs (p = 0.005), and longer hands (p = 0.049). This study suggested that PWS genetic subtypes and GH treatment can influence growth and dysmorphic features that may impact clinical diagnosis of PWS, such as stature, head shape and appearance of the eyes, nose, and genitalia.
Мета дослідження: провести порівняльний аналіз усклад нень позадулонної радикальної простатектомії (ПРПЕ) та малоінвазивної радикальної простатектомії (МРПЕ) у хво рих на рак передміхурової залози (РПЗ) шляхом визна чення частоти і тяжкості післяопераційних ускладнень. Матеріали та методи. Було проаналізовано історії хво роби 453 хворих, які перенесли радикальну простатек томію (РПЕ) в умовах ДУ «Інститут урології НАМН Ук раїни» у 2013-2015 рр. та ДУ «Інститут невідкладної та відновної хірургії імені В.К. Гусака НАМН України» про тягом 2006-2013 рр. Усіх хворих розподілили на дві гру пи. У І групу увійшли 210 (46,4%) пацієнтів, яким було виконано МРПЕ, у ІІ групу-243 (53,6%) хворих, що пе ренесли ПРПЕ. Ускладнення було розподілено: за кількістю; за ступенем тяжкості відповідно до шкали P.A. Clavien та співавторів (2009); за типом за G. Novara та співавторами (2010); прогностичні групи формувались за P.M. Pierorazio та співавторами (2013). Результати. У групі МРПЕ переважали генітоуринарні ус кладнення-64,8±3,3% проти 50,2±3,2% у групі ПРПЕ ( 2 =9,736, df=1; p<0,01), кількість проведених гемотранс фузій в обох групах була практично рівною-10,5±2,1% та 10,3±1,9% ( 2 =0,004, df=1; p>0,05). Кількість інфекційних ускладнень була вищою у групі ПРПЕ, ніж у групі МРПЕ-98,6±1,8% та 3,3±1,2% відповідно ( 2 =5,474, df=1; p<0,05). В одного пацієнта могло спостерігатися більше одного уск ладнення. В обох групах переважали «малі» ускладнення (Clavien I-II) 80,8±2,7% та 85,9±2,2%, «великі» ускладнен ня (Clavien III-IV) були зафіксовані у 19,2±2,7% та 14,1±2,2% у групах І та ІІ відповідно. Кількість ускладнень, які припадали на одного хворого статистично не відрізня лась між групами ( 2 =1,152, df=1; p>0,05). Відсоток відсут ності РПЗ за даними післяопераційного заключного гісто логічного дослідження у групі МРПЕ складав 2,9±1,1%, у групі ПРПЕ-3,3±1,1%. Група І ілюструє статистично зна чущо більшу кількість хворих II прогностичної групи: 30,5±3,2% проти 22,2±2,7% ( 2 =3,984, df=1; p<0,05) та мен шу кількість хворих IV прогностичної групи: 6,2±1,7% про ти 9,5±1,9% ( 2 =8,176, df=1; p<0,01). Різниця за іншими прогностичними групами статистично не значуща. Заключення. Кількість і характер зафіксованих усклад нень у хворих досліджуваних груп свідчить про не обхідність розроблення комплексу заходів, які направлені на попередження ускладнень інфекційної та кардіоцереб ропульмональної груп під час проведення ПРПЕ та геніто уринарної групи-при проведенні МРПЕ. Виникнення пе реважної кількості ускладнень у хворих генітоуринарної групи, які були зареєстровані у пацієнтів групи МРПЕ, за лежить від етапу формування везико уретрального анаста мозу та може бути суттєво знижено шляхом його удоско налення. Ключові слова: рак передміхурової залози, позадулонна ра дикальна простатектомія, малоінвазивна, лапароскопічна, ендовідеоскопічна екстраперитонеальна радикальна проста тектомія, везико уретральний анастамоз, ускладнення.
The objective: is to conduct the search, systematization and analysis of existing literature publications on the use of various integral combinations of efficacy (ICE) of radical prostatectomy (RPE) in the treatment of patients with prostate cancer (PC), as well as to evaluate the efficiency of the new method for the formation of vesico urethral anastomosis (VUA) with performing endoscopic radical prostatectomy (EPPE) using the ICE and to compare the findings with other studies.Materials and methods. Information search was conducted using the international databases PubMed, Google Scholar and electronic databases of the National Library of Ukraine named after V.I. Vernadskiy according to July 2017. Only original research was selected, literary reviews, or works published only as theses for consideration were not included. Each literary source was analyzed in a full text version, separately determined: author’s team, year of publication of the study, the number of patients, in relation to which the ICE were calculated, the number of operating surgeons, the type of RPE, the time of observation of patients, the number of patients who reached one or another ICE of RPE and its individual components. Urine continence (UC) and erectile function (EF) after the RPE were described in the focus of the method of their fixation and evaluation, followed by analysis of the obtained data, this approach is due to the problem of the lack of standardization of these states. Some author’s comments and conclusions are given at the end of the description of the source. The works, in which several ICE were defined, were described in the part of the review of the ICE, which of them is newer.The experimental group included 24 patients who had undergone a modified ERPE in the conditions of the SI «Institute of Urology of NAMS Ukraine» for 2015–2016 years. The average follow up time for patients was 17,4±5,3 months, a monitoring point of observation was 12 months. Biochemical recurrence (BCR) was detected in the case of PSA>0,2 ng/ml, positive surgical margin (PSM) was determined by the staff of the Laboratory of pathomorphology of the SI «Institute of Urology of NAMS Ukraine». UC were determined by subjective, non strict method (no pad/one safety pad). The EF was evaluated in accordance with the possibility for intercourse in patients with more than half of the cases with or without the of 5 phosphodiesterase inhibitors. Complications were classified by P.A. Clavien (2004). The evaluation of the results was carried out by determining the number of patients who achieved trifecta and pentafecta as the most commonly used ICE’s.Results. Generally, six separate ICE of RPE were identified, which were proposed by various authors from 2003 to 2012 years. The most commonly used were trifecta and pentafecta. The results obtained in the studies of other authors are summarized in tabular form. An analysis of the methods used by the authors to evaluate UC and EF are carried out. There were in the study group, a new method for the formation of VUA PSM was absent in 91,7%, the absence of BCR was established at 87,5%, the UC was noted at 91,7%, the preservation of EF was 62,5%, the complications were absent at 70,8% of patients. Thus, trifecta were reached 58,3%, and pentafecta were reached 50% of operated patients using a new method for the formation of VUA.Conclusions. At present time, various authors have developed 6 different integral combinations of the efficiency of the RPE, of which the scientific community has universally recognized and the most frequently used «trifecta» (M. Benson, 2004) and «pentafecta» (V. Patel, 2011). In this paper, there were, for the first time, integrated combinations of efficacy of endoscopic RPE in patients with localized PC, as well as detailed ways of evaluating the results of the RPE according to the criteria of objectivity and severity. The obtained performance indicators of ERPE with the proposed method for the formation of VUA correspond to the data of modern literature, the implementation of the new method does not compromise the oncological results of ERPE with the achievement of trifecta in 58,3%, and pentafecta – in 50% of patients with localized PC. The use of the «octafecta» concept regarding the surgical treatment of PC contravenes the norms of generally accepted clinical practice in Ukraine and is not recommended for implementation in clinical work.
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