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Complications of PI to PIII hemipelvic resections for intermediate and malignant tumours : a systematic review and meta-analysis

Smolle, Maria A; Wenzl, Florian A; Laitinen, Minna K; Jeys, Lee M; ,; Leithner, Andreas; Abdul Bari, Yunus; Abood, Ahmed; Abraham, John; Acosta, Marthelena; Agarwal, Rishi; Agarwal, Manish; Ajit Singh, Vivek; Akiyama, Toru; Al Farii, Humaid; Aldosari Omar, A; Alfaro, Patricio; Aljuhani, Wazzan; Allison, Daniel; Almashahedi, Mohammed; Alotaibi, Abdullah; Alpan, Bugra; Alshaygy Ibrahim, S; Althunayan, Turki; Andreani, Lorenzo; Andreou, Dimosthenis; Andriandi, Andriandi; Annabell, Lucas; Aponte-Tinao, Luis; Armas, Selma; Aston, William; Aycan Osman, Emre; Baad-Hansen, Thomas; Baird, Charles; Balach, Tessa; Barriga Juan, Alfonso; Barry, Janie; Basile, Georges; Bastoni, Stefano; Basuki Mohammad, Hardian; Bauer, Henrik; Bayliss, Lee; Becker, Ricardo; Bedi, Angad; Benevenia, Joseph; Bengoa, Francisco; Berger, Christina; Bernthal, Nicholas; Binitie, Odion; Bird, Justin E; Bobseit, Abdulrahman; Bodian, Caitlin; Boffano, Michele; Bonilla Huertas, Patricia; Botello, Eduardo; Boyle, Richard; Bramer, Jos; Broekhuis, Demien; Broida, Samuel E; Brown, Danielle; Budny, Tymoteusz; Burke, Zachary; Cabrolier, Jorge; Calvo Haro Jose, Antonio; Campanacci, Domenico Andrea; Cardoso, Rodrigo; Carey Smith, Richard; Casales Fresenga, Nicolas; Ceballos, Oscar; Chan Chung, Ming; Chan, Lester; Choong, Peter; Chrobok, Adam; Chung, Yang-Guk; Ciechanowicz, Dawid; Clara-Altamirano Miguel, Angel; Consuegra, Luis; Courtot, Louis; Crawford, Brooke; Cribb, Gillian; Cuervo-Lozano Carlos, Eduardo; Dammerer, Dietmar; de la Rosa, Pablo; De Paolis, Massimiliano; De Santos de la Fuente Francisco, Javier; de Vaal, Marieke; Deisenhofer, Julian; Delgado, Javier; Deo, Shaneel; Deventer, Niklas; Di Bella, Claudia; Dierselhuis, Edwin; Domson, Gregory; Donati, Davide Maria; Duran-Ciarrochi, Rodolfo; Durr Hans, Roland; Ebeid, Walid; Ehlers, Pierre; El Ghoneimy Ahmed, Mohamed; El Motassime, Alessandro; Eloi Pinto Fabio, Fernando; Endo, Makoto; Epstein, Gadi; Eralp, Levent; Etaiwi, Mahmoud; Eward, Will; Fabbri, Nicola; Faimali, Martina; Farooq, Arbab; Farooque, Khalil; Ferguson, Peter; Ferreira, Nando; Fiorenza, Fabrice; Fonseca de Freitas, Joao; Forsberg Jonathan, A; Fuchs, Bruno; Fujiwara, Tomohiro; Funovics, P T; Fuzy, Edward; Galli Serra, Marcos; Gamie, Zakareya; Garcia Carrasco, Maria; Gaston Czar, Louie; Georges, Basile; Ghert, Michelle; Ghert, Michelle; Ghosh, Kanishka; Giardina Fabio, Luca; Gomez Mier Luis, Carlos; Gomez-Mascard, Anne; Gomez-Sierra, Maria Antonia; Goodman Mthethwa, Phakamani; Gortzak, Yair; Goulding, Krista; Gracia, Isidro; Green, Natalie; Griffin, Anthony; Guedes, Alex; Gulia, Ashish; Gupta, Sanjay; Guzman, Maurice; Hardes, Jendrik; Hasan Yusuf, O; Havard, Hel; Haydon, Rex; Hegde, Prateek; Hernandez Lopez, Adriana; Herrera, David; Hesla, Asle; Hess, Matthew; Hilton, Thomas; Hirschmann, Adam; Hobusch, Gerhard; Hongsaprabhas, Chindanai; Hornicek, Francis; Hosking, Keith; Houdek, Matthew T; Hsu, Megan; Idowu, Oluwaseyi; Idulhaq, Mujaddid; Ippolito, Joseph; Iwata, Shintaro; Jagiello, Jake; Jeys, Lee; Johan, Muhammad Phetrus; Johnson, Luke; Johnston, Andy; Joo, Min Wook; Jutte, Paul; Jääskeläinen, Anna-Stina; Kaldas, Kadri; Kapanci, Bilal; Karaca Mustafa, Onur; Kawai, Akira; Kemp, Alysia; Khal Adyb-, Adrian; Khan, Zeeshan; Khan Zainab, Aqeel; Kim, Han-Soo; Klopper, Schalk; Kobayashi, Eisuke; Kobayashi, Hiroshi; Kontogeorgakos, Vasileios; Kotrych, Daniel; Krishnamurthy Anjan, Venkataraman; Kunisada, Toshiyuki; Kurisunkal, Vineet; Kyte, Richard; Laitinen, Minna; Laubscher, Maritz; Lazarides, Alexander; Le Nail, Louis-Romee; Lee Francis, Y; Legosz, Pawel; Lehner, Burkhard; Leithner, Andreas; Leone, Gianpaolo; Lewis Valerae, O; Li, Binghao; Liikanen, Hanna; Lin, Peng; Linda, Zwelithini; Lindsay, Sarah; Lozano Calderon, Santiago; Lutomia Lumbasi, Mark; MacDonald, Jonathan; Malina, Mario; Marais, Len; Mascard, Eric; Mattei, Jean-Camille; McCullough, Louise; McMahon, Sam; Medellin Rincon Manuel, Ricardo; Mediavilla Santos, Lidya; Meijer, Diederik; Meijer Johannes, Gerard; Miller, Ben; Molloy, Allan; Moriel Garcesco Diego, Jesus; Morris, Guy; Morris Carol, D; Morse-Sanyal, Ashlyn; Mottard, Sophie; Munir Hashim, Ahmad; Murcia, Miguel; Müller, Michelle; Nakayama, Robert; Narhari, Prashant; Nasar, Ali; Nayak, Prakash; Neugebauer, Johannes; Nieminen, Jyrki; Ntombela, Philani; Nystrom, Lukas; O'Toole, Gary; Ogura, Koichi; Oliveira, Vania; Olivier, André; Omar, Mohamed; Omran Hasan, Yusuf; Ortiz-Cruz, Eduardo; Ozaki, Shuhei; Ozaki, Toshifumi; Pala, Elisa; Palmerini, Emanuela; Panchwagh, Yogesh; Papagelopoulos, Panayiotis; Papagelopoulos, Dimitra; Paraliticci, Giovanni; Gibbs C, Parker; Parry, Michael; Peiró, Ana; Perera, Jonathan; Petersen, Michael Moerk; Phakathi, Oatile; Phimolsarnti, Rapin; Phiri, Tshepang; Pinto Santander, Nicolas; Ploegmakers, Joris; Pollock, Robin; Powell, Gerard; Pruthi, Manish; Puhaindran, Mark; Puri, Ajay; Quirion, Julia; Rabin, Eden; Rachbauer, Anna; Radhakrishnan, Sanjeevan; Raja, Anand; Rajalbandi, Rohit; Rajani, Rajiv; Rajasekaran Raja, Bhaskara; Rajkovic, Stanislav; Ramkumar, Dipak; Rankin, Kenneth; Ras El Abiad Mejia, Alejandro; Rasappan, Kumaran; Redl, Malena; Rose Peter, S; Rosenberg, Andrew; Ruggieri, Pietro; Russell, Michael; Salcedo, German; Saleh, Ahmad; Sambri, Andrea; Saputra Rhyan, Darma; Scanferia, Roberto; Scharschmidt, Thomas; Schubert, Thomas; Scoccianti, Guido; Segura, Florencio; Sellevold, Simen; Rajasekaran, Shanmuganthan; Shehadeh, Ahmad; Shreemal, Bhim; Shumelinsky, Félix; Siddiqi Muhammad, Ather; Silveri, Claudio; Sinnaeve, Friedl; Smolle, Maria Anna; Snyman, Franz; Solomons, Michael; Sommerville, Scott; Sood Sahil, Sood; Spense Mariel, Eliana; Spiegel, Christian; Spiguel, Andre; Staals, Eric; Stavropoulos, Nikolaos; Steadman, Peter; Stern, Sydney; Stevenson, Jonathan; Stoppiello, Pablo; Sullivan Mikaela, H; Szostakowski, Bartlomiej; Szostakowski, Bartek; Tang, Xiaodong; Thippesamy Pushpa, B; Thorkildsen, Joachim; Tootsi, Kaspar; Torner Rubies, Ferran; Tosyali, Koray; Traub, Frank; Trent Jonathan, C; Trikoupis, Ioannis; Tsagkozis, Panagiotis; Tuntarattanapong, Pakjai; Ullah, Farman; Ulrich, Marisa N; Vainio, Veli-Matti; Valencia, Juan; van de Sande, Michiel; Van Der Geest, Ingrid; van der Wal, Robert; Velez Villa, Roberto; Verbeke, Léonie; Verspoor Floortje, G M; Versteeg, Anne; Vicatos, George; Virk Jagandeep, Singh; Visgauss, Julia; Vyrva, Oleg; Wafa, Hazem; Wan Faisham Numan Wan, Ismail; Wang Patrick, Qi; Wei, Ran; Wennergren, David; Werier, Joel; Weschenfelder, Wolfram; Williams, Nadine; Wunder, Jay; Yildiz Huseyin, Yusuf; Yonamine, Eduardo; Yonezawa, Hirotaka; Yousuf, Maitham; Zainul Abidin, Suraya; Zamora, Tomas; Zuckerman, Lee; Zumarraga Juan, Pablo; Özger, Harzem; Özkan, Korhan; Triganjananun, Chanonta; Aguirre, Marcela; Alaqeel, Motaz; Alaseem, Abdulrahman; Alexander, Kate; Ardelt, Melanie; Baeza, Pablo; Banse, Xavier; Franks, Daniel; Baydar, Semay; Doshi, Arpan; Bruschi, Alessandro; Buist, Mirka; Busse, Tilmann; Carrasco, María; Castan, Ashley; Chang, Liang; Charoenlap, Chris; Chaustre, Florez; Chin, Janet; Pate, Matthew; Olson, Daniel; Cornu, Olivier; Farris, Clayton; de, Lima; Demir, Eren; Docquier, Pierre-Louis; Duivenvoorden, Myléne; Farman, Ullah; Flint, Michael; García-Huidobro, Gabriel; Gazendam, Aaron; Golovina, Yanina; Gomez M Luis, Carlos; González-Browne, Catalina; Gonzalez-Saldivar, Juan Carlos; González-Motta, Alejandro; Gosheger, Georg; Gouin, Francois; Graydon, Andrew; Hesham, Amr; Hohensteiner, Anna; Igbinoba, Bright; Joyce, David; Letson, Douglas; Tepper, Sarah; Jung, Beatrice; Jungels, Christiane; Jutte, Willem; Khan Zainab, Aqeel; Khaouam, Nader; Krebbekx, Gitte; Lacroix, Valérie; Lee, Jewoo; Lee, Minpyo; Machado, Pau; Majirija, Edgar; Malyk, Roman; Mthethwa Phakamani, G; Muñoz-Montecinos, Carlos; Quirland, Camila; Ramirez, Maria; O'Reilly-Harbidge, Sarah; Oktayana, Made Dolly; Olusunmade, Opeyemi; Pang, Grant; Stubbe, Chris; Parizzia, Walter; Pinheiro, Rafael; Prabowo, Yuni; Ramkumar Dipak, B; Rizzo, Arianna; Samy, Ahmed; Sánchez-Maldonado, Maria; Sundin, Nathalia; Suntaxi, Basantes; Tandon, Nikhil; Terrarossa, Bruno; Trullols, Laura; Tsoi, Kim; Zaghloul, Ahmed
AIMS/UNASSIGNED:Surgical management of intermediate and malignant tumours in the pelvis is complex. Complications are frequent and either related to the surgery itself or to post-surgical failure of the reconstruction technique. This systematic review and meta-analysis aims at analyzing all reported complications following PI to PIII pelvic resections for intermediate and malignant tumours. METHODS/UNASSIGNED:Based on a systematic literature search on PubMed adhering to the PRISMA guidelines, 1,683 study records were identified, of which we included 90 original studies published until 22 July 2025. Overall complication rates were assessed with random-effects meta-analysis. Differences in complication rates between reconstruction types (i.e. megaprosthetic, mostly biological, none) were evaluated with meta regression analysis. RESULTS/UNASSIGNED:Data on 2,199 patients (1,250 males (57%)) with mainly PI to PIII pelvic resections were analyzed. The most common reconstruction types were custom-made implants (21%; n = 451) and ice-cream cone prostheses (14%; n = 312). Pooled rates of infections, wound healing problems, nerve injuries, and deep vein thrombosis (DVT) amounted to 15% (95% CI 12% to 18%), 13% (95% CI 10% to 15%), 7% (95% CI 5% to 9%), and 4% (95% CI 2% to 6%), respectively. Further, pooled implant revision/removal and secondary external hemipelvectomy rates were 14% (95% CI 11% to 17%) and 4% (95% CI 3% to 5%). Mostly biological reconstructions were associated with higher rates of nerve injuries (p < 0.001), construct failures (p = 0.010), and secondary implant revision/removal (p = 0.003) compared to megaprosthetic reconstruction. Further, biological reconstructions were associated with increased secondary external hemipelvectomy rates compared to megaprosthetic reconstructions (p = 0.005) or no reconstructions (p = 0.001). CONCLUSION/UNASSIGNED:Treatment of pelvic malignancies is challenging, with technically demanding resections and complex reconstructions. Across all reconstruction techniques following sacrum-sparing pelvic resections, infections and wound healing problems are the most common complications, yet there is also a considerable proportion of patients with neurovascular complications and DVTs.
PMCID:13222729
PMID: 42219227
ISSN: 2633-1462
CID: 6043392

Racial and Ethnic Differences in Predicted Cardiovascular Disease Risk Using the PREVENT Equations in the US Population [Letter]

Khan, Sadiya S; Huang, Xiaoning; Coresh, Josef; Shah, Nilay S; Lloyd-Jones, Donald M; Echouffo-Tcheugui, Justin B; Matsushita, Kunihiro; Ndumele, Chiadi E
PMID: 42220236
ISSN: 3068-563x
CID: 6043412

Subthalamic versus Posterior Subthalamic Stimulation for Optimal Tremor Control in Parkinson's Disease

Bellini, Gabriele; Boccia, Vincenzo Daniele; Ceravolo, Roberto; Mogilner, Alon; Pourfar, Michael H
UNLABELLED:<p>Introduction: Tremor-predominant Parkinson's disease (TPPD) generally responds favorably to deep brain stimulation (DBS) targeting the subthalamic nucleus (STN). However, traditional stereotactic targeting of the STN does not universally yield the anticipated intraoperative improvement, prompting exploration of additional targets to achieve optimal results prior to permanent implantation of electrodes. The posterior subthalamic area (PSA), including the caudal zona incerta (cZI), have been associated with tremor suppression and can be easily compared to the neighboring STN intraoperatively. METHODS:We retrospectively compared intraoperative and clinical outcomes in tremor-dominant PD patients who prospectively underwent dual trajectory microelectrode monitor targeting the STN and PSA/cZI. We compared the neurophysiology and tremor response of both the central (STN) and posterior (PSA) trajectories in 22 patients and analyzed outcomes in those who ultimately received traditional STN (16) or PSA/cZI lead implantation (12). RESULTS:While both groups achieved substantial overall motor improvement under chronic stimulation, intraoperative test stimulation through the posterior path produced more consistent tremor arrest compared with STN. These findings suggest that positioning the DBS lead further posteriorly to engage the PSA can augment tremor suppression in select cases of TPPD without compromising other parkinsonian symptom relief. CONCLUSION/CONCLUSIONS:Our results emphasize the value of intraoperative physiological feedback in trajectory selection in tremor-predominant patients and are consistent with emerging literature that PSA/cZI DBS is an effective and potentially superior target for management of tremor in PD. </p>.
PMCID:12810967
PMID: 41411220
ISSN: 1423-0372
CID: 6043352

An Evaluation of Ibrutinib and Ixazomib in Patients With Relapsed/Refractory Mantle Cell Lymphoma: PrE0404

Cohen, Jonathon B; Portell, Craig A; Hamadani, Mehdi; Jegede, Opeyemi; Diefenbach, Catherine; Fletcher, Christopher; Matasar, Matthew; Landsburg, Daniel; Mantha, Suparna; Kahl, Brad
INTRODUCTION/BACKGROUND:Management of relapsed mantle cell lymphoma (MCL) has included Bruton's tyrosine kinase (BTK) inhibitors for more than 10 years, but finding an optimal combination partner that meaningfully improves outcomes while limiting toxicity has been difficult. We conducted a phase 1/2 trial of ibrutinib and the proteasome inhibitor, ixazomib, in patients with relapsed/refractory MCL. PATIENTS AND METHODS/METHODS:Patients and Methods: The primary endpoint for the phase 1 study was to determine the recommended phase 2 dose (RP2D), and for the phase 2 study was the complete response (CR) rate, compared with the previously reported single-agent CR rate of ibrutinib. After the phase 1 portion of the study identified a recommended phase 2 dose of ixazomib 4 mg days 1, 8, and 15, of a 28-day cycle combined with ibrutinib 560 mg daily until progression or unacceptable toxicity. RESULTS:We enrolled 35 BTK-naïve patients to the phase 2 portion of the study. Overall response rate was 77%, and 37% of patients achieved a CR. The 2-year progression-free survival is 44%, and the duration of response is 47%. Treatment-related adverse events were common, resulting in treatment discontinuation for 37% of patients. CONCLUSION/CONCLUSIONS:Given that the progression-free survival (PFS) was not significantly improved compared to historical reports for single-agent BTK inhibitors and the high rate of treatment-related toxicity, this combination does not merit further study in this setting. Additional trials evaluating newer BTK inhibitors and alternative combination partners are warranted.
PMID: 42209393
ISSN: 2152-2669
CID: 6042802

Long-term outcomes of congenital high airway obstruction syndrome (CHAOS) at a single comprehensive fetal center

Wagner, Monica L; Peiro, Jose L; Rymeski, Beth A; Smith, Matthew M; de Alarcon, Alessandro; McKinney, David N; Habli, Mounira A; Lim, Foong-Yen
OBJECTIVES/OBJECTIVE:Congenital high airway obstruction syndrome (CHAOS) is characterized by over-distended lungs leading to impaired cardiac return and fetal hydrops. Survivors have been reported following prenatal spontaneous fistulization, fetal procedures to decompress the airway, or ex-utero intrapartum treatment (EXIT). The long-term outcomes of survivors are unclear. METHODS:We performed a retrospective chart review on patients diagnosed with CHAOS in our center between 2005-2025. RESULTS:Of the 28 patients with CHAOS, three (10.7%) underwent a fetal procedure to decompress the airway. Three patients (10.7%) had evidence of spontaneous fistulization. Four patients (14.3%) terminated the pregnancy and four (14.3%) had in-utero fetal demise. Twenty patients (71.4%) were live-born; of these, 14 (70%) died shortly after delivery and two (10%) died in the neonatal period. Seven patients (35%) underwent EXIT-to-tracheostomy at our center, of which four (57.1%) are long-term survivors ranging in age from 4 to 19 years old. Three patients have undergone airway reconstruction between 1.6 and 5.6 years of age; one remains tracheostomy-dependent due to recurrent airway stenosis, one patient has undergone reconstruction and is likely to be decannulated soon, and one patient had successful reconstruction and was decannulated. The fourth patient has not yet undergone airway reconstruction. CONCLUSIONS:CHAOS remains a highly morbid diagnosis, but long-term survivorship and liberation from tracheostomy is possible.
PMID: 42213638
ISSN: 1421-9964
CID: 6042902

On-site exposure to clinical epilepsy practice for experimental scientists engaged in epilepsy research: A pilot study by the ILAE commission on neurobiology

de Curtis, Marco; Battaglia, Giulia; Aguado-Carrillo, Gustavo; Aronica, Eleonora; Asukile, Melody; Balestrini, Simona; Barba, Carmen; Baumgartner, Tobias; Becker, Albert J; Bisulli, Francesca; Braga, Patricia; Carcak, Nihan; Cavalheiro, Esper; Delanty, Norman; Ferri, Lorenzo; Friedman, Alon; Friedman, Daniel; Galovic, Marian; Gelinas, Jennifer N; Giagante, Brenda; Henriquez-Ch, Rodrigo; Kander, Veena; Kochen, Silvia; Krysl, David; Kudr, Martin; Ikeda, Akio; Legnani, Mariana; Lin, Yicong; Martinez-Juarez, Iris; Muccioli, Lorenzo; Mwendaweli, Naluca; Oddo, Silvia; Özkara, Çigdem; Peixoto-Santos, Jose Eduardo; Perucca, Piero; Potschka, Heidrun; Rocha, Luisa; Scharfman, Helen; Scheffer, Ingrid E; Surges, Rainer; Triki, Chanez Charfi; Uribe-San-Martin, Reinaldo; Valente, Kette; van Vliet, Erwin A; Wang, Yuping; Whatley, Benjamin; Wilmshurst, Jo M; Yacubian, Elza Marcia; De Rossi, Alessandro; de Curtis, Stefano; Jiruska, Premysl; Henshall, David C
Educational initiatives that address the gap between basic/preclinical and clinical practices are important to effectively translate basic science discoveries to benefit patients. The ILAE Neurobiology Commission conducted a pilot project aimed at exposing basic and preclinical scientists engaged in epilepsy research to general clinical issues pertaining to the diagnosis and care of people with epilepsy. This aim was addressed through a two-week-long, on-site clinical training program for 50 basic scientists in 21 epilepsy centers across 18 countries in the six ILAE regions (with a maximum of 3 basic scientists per center). The learning objectives and the training module were discussed and defined by the project organizing committee, which consisted of Neurobiology Commission members and a team of epileptologists representing different geographical regions. The training activities were conducted at each epilepsy center under the local supervision of clinical tutors. Each basic scientist was exposed to 50.3 ± 23.3 (range 16-89) hours of intensive and dedicated clinical training, coordinated by 2-3 tutors per center, assisted by 6.8 ± 3.6 colleagues. A structured test consisting of 17 general clinical epilepsy questions was completed by the trainees before and after the training activity. The learning assessment was based on the comparison between responses to the exit and entry tests. After the on-site clinical exposure, the proportion of correct answers increased to 87% compared to 61% in the entry test. Structured post-training questionnaires demonstrated very high satisfaction of trainees and all involved tutors across the different aspects of the training module. This global pilot study demonstrated that on-site attendance by basic scientists in specialized clinical settings up-scaled their knowledge of clinical epileptology and facilitated networking with clinicians. Expansion of this pilot to further centers should be considered to understand how exposure to clinical practice affects research direction and quality of translational epilepsy research. PLAIN LANGUAGE SUMMARY: Epilepsy research has long benefitted from collaboration between scientists and clinicians. Early exposure of researchers to people with epilepsy and their care teams may strengthen future impact. This pilot study tested a two-week immersive experience where small teams of basic scientists shadowed clinicians during their work at hospitals around the world. Questionnaires showed high satisfaction among both groups. Results support expanding such training, with the backing of the International League Against epilepsy and aligned centers, to build understanding, interest, and long-term commitment, ensuring bench research is informed by and translates to clinical practice and improved quality of life for patients.
PMID: 42220231
ISSN: 2470-9239
CID: 6043402

Prenatal repair of myelomeningocele is associated with lower need for long-term feeding support

Healy, Jennifer; Liu, Chunyan; Ehrlich, Shelley; Lim, Foong-Yen; Peiro, Jose L; Haberman, Beth; Stevenson, Charles B; Riddle, Stefanie
OBJECTIVE:Infants with myelomeningocele (MMC) are at risk of brainstem dysfunction secondary to symptomatic Chiari II malformation with hindbrain herniation (HH), which can manifest as feeding difficulties including aspiration and dysphagia. This study aims to investigate whether prenatal repair of MMC is associated with improved feeding outcomes compared to postnatal repair. STUDY DESIGN/METHODS:Retrospective observational study of 208 infants with MMC, 105 repaired prenatally and 103 repaired postnatally, from January 2011 to July 2022. Primary outcome was feeding tube at discharge and longitudinally through 12 months corrected gestational age (CGA). RESULTS:9.5% of infants repaired prenatally and 13.6% repaired postnatally required feeding tube at discharge (p = 0.3585). By 53 weeks CGA, the prenatal repair group had decreased odds of requiring feeding tube (0.325 [95% CI 0.121, 0.872]). CONCLUSION/CONCLUSIONS:Prenatal MMC repair was associated with decreased need for long-term feeding support, suggesting a potential functional benefit of prenatal repair related to reversal of HH.
PMCID:13008767
PMID: 40702155
ISSN: 1476-5543
CID: 6043112

In Vivo Effect of a Synthetic Amniotic Fluid on Fetal Lung and Gastrointestinal Tract: A Pre-Clinical Rodent Model

Forde, Braxton; Finoti, Stephanie; Oria, Marc; Peiro, Jose L
OBJECTIVE:Amnioinfusions in anhydramnios aim to promote fetal lung development, but currently used fluids (Normal Saline [NS], Lactated Ringer's [LR]) fail to mimic the intrauterine environment and increase reactive oxygen species (ROS). We developed a synthetic amniotic fluid (Amnio-well, AW) designed to reduce intrauterine ROS. This study evaluated the pulmonary and gastrointestinal effects of 2 formulations of AW compared with those of NS and LR in a pre-clinical model. METHOD:At gestational age E17.5, pregnant rats underwent amniotic fluid replacement with NS, LR, AW, AW plus epidermal growth factor and transforming growth factor-β (AW++), or sham control. Fetal lungs were harvested at E20.5 for histology, fractional airspace, and blinded pathological evaluation. Surfactant protein (SP-A, SP-B, SP-C) expression and inflammatory gene panels were assessed in lungs and gastrointestinal (GI) tissue. RESULTS:NS and LR lungs demonstrated edema, macrophage infiltration, and reduced airspace (p < 0.001). AW improved SP-B and SP-C relative to control, whereas AW++ suppressed SP-B and SP-C (p < 0.05). Lung gene profiling showed NS/LR induced alterations in histamines, annexins, and immune recruitment, while AW closely resembled control. GI histology was similar across groups, though NS/LR altered TNF, prostaglandin, and adhesion pathways (p < 0.05). CONCLUSION:AW reduced lung inflammation and enhanced surfactant expression compared with NS or LR, with minimal GI effects.
PMCID:13070220
PMID: 41882498
ISSN: 1097-0223
CID: 6042882

Brain Imaging Findings Show Efficacy of Fetal Endoscopic Third Ventriculostomy as Prenatal Treatment for Induced Congenital Hydrocephalus in Fetal Lambs

Duru, Soner; Oria, Marc; Fernandez-Tome, Blanca; Peiro, Lucas; Encinas, Jose L; Sanchez-Margallo, Francisco M; Peiro, Jose L
BACKGROUND AND OBJECTIVES/OBJECTIVE:Congenital obstructive hydrocephalus (HCP) causes progressive, irreversible fetal brain damage through ventricular enlargement and increasing fetal cerebral tissue compression. Postnatal treatments of choice include ventriculoperitoneal shunting or endoscopic third ventriculostomy (ETV). Intrauterine treatments, such as ventriculoamniotic shunting, were attempted unsuccessfully 4 decades ago and failed to improve postnatal outcomes, likely due to inadequate fetal patient selection. The aim of this study was to evaluate the efficacy of prenatal ETV for early ventricular decompression and potential prevention of fetal brain damage in hydrocephalic fetal lambs. METHODS:HCP was induced in 24 fetal lambs by injecting BioGlue into the cisterna magna at E85. Three weeks later (E105-110), fetal ETV was successfully performed on 8 fetuses using a small rigid cystoscope. Fetal brain lateral ventricular diameters and cerebral mantle thicknesses were monitored by prenatal and postnatal ultrasounds and fetal MRI. RESULTS:According to the Cincinnati HCP Severity Scale, moderate and severe HCP subgroups responded positively to fetal ETV with reduced cerebral ventricular diameters. Ten days post-ETV, severe HCP fetal lambs improved to moderate levels, whereas those with moderate HCP normalized by birth. A similar improvement pattern was seen for the mechanical compression threshold (ventricular diameters/biparietal diameter). Biparietal diameter values did not significantly differ among nontreated, treated, and normal control groups during pregnancy. MRI revealed a significant increase in brain mantle thickness in the prenatally treated fetuses. CONCLUSION/CONCLUSIONS:Prenatal ETV is feasible in hydrocephalic fetal lambs and effectively reverses ventriculomegaly and brain compression in cases of severe or moderate fetal HCP in this ovine model.
PMCID:12875635
PMID: 40844280
ISSN: 1524-4040
CID: 6043132

Increased risk of atrial fibrillation and flutter in patients with Cushing's syndrome: a population-based matched cohort study

Polansky, Amir; Rudman, Yaron; Kushnir, Shiri; Shochat, Tzipora; Gurevitz, Chen; Isand, Kristina; Agrawal, Nidhi; Fleseriu, Maria; Akirov, Amit
BACKGROUND:Patients with Cushing's syndrome (CS) have a high prevalence of cardiovascular disease, and other recognized risk factors for atrial fibrillation/flutter (AF/AFL); however, the prevalence of AF/AFL has not been well characterized in this population. METHODS:We conducted a retrospective matched-cohort study using the Clalit Health Services database, including patients with CS and 1:5 matched controls. We assessed the risk of new-onset AF/AFL overall and according to disease etiology and remission status. Pre-existing AF/AFL was defined as >30 days before CS, and new-onset as within 30 days or thereafter. RESULTS:The cohort included 609 patients with CS and 3018 controls. Pre-existing AF/AFL was more common among patients with CS than controls (3.6% vs. 2.1%; OR 1.70, 95% CI 1.04-2.78). During a mean follow-up of 15 years, patients with CS had a significantly higher risk of developing new-onset AF/AFL compared with controls (HR 1.55, 95% CI 1.19-2.03). This increased risk was observed in both Cushing's disease (CD) (HR 1.53, 95% CI 1.01-2.32) and adrenal CS (HR 1.70, 95% CI 1.06-2.74). AF/AFL risk did not significantly differ according to remission status, although a trend toward lower risk was observed. Multivariate analysis identified older age at diagnosis, male sex, hypertension, vascular disease, and higher BMI as predictors for new-onset AF/AFL. CONCLUSION/CONCLUSIONS:CS is associated with an increased risk of AF/AFL. This elevated risk is observed across both CD and adrenal CS and persists despite disease remission, underscoring the need for heightened awareness and close cardiovascular surveillance in this population. The increased risk of AF/AFL appears to be primarily driven by coexisting cardiovascular comorbidities rather than cortisol excess or other CS-specific features.
PMID: 42200260
ISSN: 1479-683x
CID: 6043372