RESUMO
BACKGROUND & AIMS: The efficacy of a low fermentable oligo-, di-, monosaccharides and polyols (FODMAP) diet in irritable bowel syndrome (IBS) is well established. After the elimination period, a reintroduction phase aims to identify triggers. We studied the impact of a blinded reintroduction using FODMAP powders to objectively identify triggers and evaluated the effect on symptoms, quality of life, and psychosocial comorbidities. METHODS: Responders to a 6-week low FODMAP diet, defined by a drop in IBS symptom severity score (IBS-SSS) compared with baseline, entered a 9-week blinded randomized reintroduction phase with 6 FODMAP powders (fructans, fructose, galacto-oligosaccharides, lactose, mannitol, sorbitol) or control (glucose). A rise in IBS-SSS (≥50 points) defined a FODMAP trigger. Patients completed daily symptom diaries and questionnaires for quality of life and psychosocial comorbidities. RESULTS: In 117 recruited patients with IBS, IBS-SSS improved significantly after the elimination period compared with baseline (150 ± 116 vs 301 ± 97, P < .0001, 80% responders). Symptom recurrence was triggered in 85% of the FODMAP powders, by an average of 2.5 ± 2 FODMAPs/patient. The most prevalent triggers were fructans (56%) and mannitol (54%), followed by galacto-oligosaccharides, lactose, fructose, sorbitol, and glucose (respectively 35%, 28%, 27%, 23%, and 26%) with a significant increase in abdominal pain at day 1 for sorbitol/mannitol, day 2 for fructans/galacto-oligosaccharides, and day 3 for lactose. CONCLUSION: We confirmed the significant benefit of the low FODMAP diet in tertiary-care IBS. A blinded reintroduction revealed a personalized pattern of symptom recurrence, with fructans and mannitol as the most prevalent, and allows the most objective identification of individual FODMAP triggers. Ethical commission University hospital of Leuven reference number: s63629; Clinicaltrials.gov number: NCT04373304.
Assuntos
Dieta com Restrição de Carboidratos , Dissacarídeos , Fermentação , Síndrome do Intestino Irritável , Lactose , Manitol , Monossacarídeos , Oligossacarídeos , Qualidade de Vida , Humanos , Síndrome do Intestino Irritável/dietoterapia , Feminino , Masculino , Adulto , Pessoa de Meia-Idade , Oligossacarídeos/administração & dosagem , Oligossacarídeos/efeitos adversos , Manitol/administração & dosagem , Manitol/efeitos adversos , Dieta com Restrição de Carboidratos/métodos , Dieta com Restrição de Carboidratos/efeitos adversos , Resultado do Tratamento , Lactose/efeitos adversos , Lactose/administração & dosagem , Monossacarídeos/administração & dosagem , Monossacarídeos/efeitos adversos , Dissacarídeos/administração & dosagem , Dissacarídeos/efeitos adversos , Polímeros/administração & dosagem , Frutose/administração & dosagem , Frutose/efeitos adversos , Sorbitol/administração & dosagem , Sorbitol/efeitos adversos , Frutanos/administração & dosagem , Frutanos/efeitos adversos , Índice de Gravidade de Doença , Método Duplo-Cego , Inquéritos e Questionários , Pós , Recidiva , Adulto Jovem , Dieta FODMAPRESUMO
OBJECTIVE: This systematic review aims to evaluate current choices in practice and outcomes of biomaterials used in patch repair of congenital diaphragmatic hernia (CDH). BACKGROUND: Multiple biomaterials, both novel and combinations of pre-existing materials are employed in patch repair of large size CDHs. METHODS: A literature search was performed across Embase, Medline, Scopus, and Web of Science. Publications that explicitly reported patch repair, material used, and recurrences following CDH repair were selected. RESULTS: Sixty-three papers were included, presenting data on 4595 patients, of which 1803 (39.2%) were managed using 19 types of patches. Goretex® (GTX) (n=1106) was the most frequently employed patch followed by Dualmesh® (n=267), Surgisis® (n=156), Marlex®/GTX® (n=56), Tutoplast dura® (n=40), Dacron® (n=34), Dacron®/GTX® (n=32), Permacol® (n=24), Teflon® (n=24), Surgisis®/GTX® (n=15), Sauvage® Filamentous Fabric (n=13), Marlex® (n=9), Alloderm® (n=8), Silastic® (n=4), Collagen coated Vicryl® mesh (CCVM) (n=1), Mersilene® (n=1), and MatriStem® (n=1) Biomaterials were further subgrouped as: synthetic nonresorbable (SNOR) (n=1458), natural resorbable (NR) (n=241), combined natural and synthetic nonresorbable (NSNOR) (n=103), and combined natural and synthetic resorbable (NSR) (n=1). The overall recurrence rate for patch repair was 16.6% (n=299). For patch types with n>20, recurrence rate was lowest in GTX/Marlex (3.6%), followed by Teflon (4.2%), Dacron (5.6%), Dualmesh (12.4%), GTX (14.8%), Permacol (16.0%), Tutoplast Dura (17.5%), SIS/GTX (26.7%), SIS (34.6%), and Dacron/GTX (37.5%).When analyzed by biomaterial groups, recurrence was highest in NSR (100%), followed by NR (31.5%), NSNOR (17.5%), and SNOR the least (14.0%). CONCLUSION: In this cohort, over one-third of CDH were closed using patches. To date, 19 patch types/variations have been employed for CDH closure. GTX is the most popular, employed in over 60% of patients; however, excluding smaller cohorts (n<20), GTX/Marlex is associated with the lowest recurrence rate (3.6%). SNOR was the material type least associated with recurrence, while NSR experienced recurrence in every instance.
Assuntos
Materiais Biocompatíveis , Hérnias Diafragmáticas Congênitas , Herniorrafia , Telas Cirúrgicas , Hérnias Diafragmáticas Congênitas/cirurgia , Humanos , Herniorrafia/métodos , Herniorrafia/instrumentação , RecidivaRESUMO
BACKGROUND: Recurrent respiratory tract infections (rRTIs) are a common reason for immunodiagnostic testing in children, which relies on serum antibody level measurements. However, because RTIs predominantly affect the respiratory mucosa, serum antibodies may inaccurately reflect local immune defences. We investigated antibody responses in saliva and their interplay with the respiratory microbiota in relation to RTI severity and burden in young children with rRTIs. METHODS: We conducted a prospective cohort study including 100 children aged <10â years with rRTIs, their family members and healthy healthcare professionals. Total and polyreactive antibody concentrations were determined in serum and saliva (ELISA); respiratory microbiota composition (16S rRNA sequencing) and respiratory viruses (quantitative PCR) were characterised in nasopharyngeal swabs. Proteomic analysis (Olink) was performed on saliva and serum samples. RTI symptoms were monitored with a daily mobile phone application and assessed using latent class analysis and negative binomial mixed models. RESULTS: Serum antibody levels were not associated with RTI severity. Strikingly, 28% of salivary antibodies and only 2% of serum antibodies displayed polyreactivity (p<0.001). Salivary polyreactive IgA was negatively associated with recurrent lower RTIs (adjusted OR 0.80, 95% CI 0.67-0.94) and detection of multiple respiratory viruses (adjusted OR 0.76, 95% CI 0.61-0.96). Haemophilus influenzae abundance was positively associated with RTI symptom burden (regression coefficient 0.05, 95% CI 0.02-0.08). CONCLUSION: These results highlight the importance of mucosal immunity in RTI severity and burden, and suggest that the level of salivary polyreactive IgA and H. influenzae abundance may serve as indicators of infection severity and burden in young children with rRTIs.
Assuntos
Haemophilus influenzae , Recidiva , Infecções Respiratórias , Saliva , Humanos , Masculino , Feminino , Haemophilus influenzae/imunologia , Estudos Prospectivos , Infecções Respiratórias/imunologia , Infecções Respiratórias/microbiologia , Infecções Respiratórias/diagnóstico , Pré-Escolar , Saliva/imunologia , Lactente , Criança , Índice de Gravidade de Doença , Anticorpos Antibacterianos/sangue , Infecções por Haemophilus/imunologia , Infecções por Haemophilus/diagnóstico , Anticorpos Antivirais/sangue , Imunoglobulina A/sangue , RNA Ribossômico 16S/genéticaRESUMO
Incontinentia pigmenti (IP, Bloch-Sulzberger syndrome) is a multisystem disorder which associates specific skin lesions that evolves in four stages, and occasionally, central nervous system, eye, hair, and teeth involvement. Familial (35%) and sporadic (65%) cases are caused by pathogenic variants in the IKBKG gene. Here we report an unusual family, where, in two half-sisters affected by typical IP, molecular genetic analysis identified a likely pathogenic non-sense variant in the IKBKG gene of one of the sisters, the other being not a carrier. The strong clinical conviction motivated further molecular genetic investigations, which led to the characterization of a second variant in this unique family. X chromosome inactivation studies demonstrated the paternal origin of these two de novo variants. For genes with frequent de novo mutations, the coexistence of different pathogenic mutations in the same family is a possibility, and constitutes a challenge for genetic counseling.
Assuntos
Quinase I-kappa B , Incontinência Pigmentar , Mutação , Linhagem , Humanos , Incontinência Pigmentar/genética , Incontinência Pigmentar/patologia , Quinase I-kappa B/genética , Feminino , Mutação/genética , Inativação do Cromossomo X/genética , Masculino , Recidiva , Fenótipo , Predisposição Genética para DoençaRESUMO
We present the case of a 58-year-old female patient who presented with an extramedullary B-ALL relapse after prior allogenic HSCT and blinatumomab therapy. The patient died from complications of a drug-induced acute liver failure after a salvage therapy combining inotuzumab ozogamicin (InO)-based induction followed by consolidation with high dose MTX and pegaspargase based on the GMALL protocol for older ALL patients. After a diagnosis of the extramedullary relapse in the form of a retro vesical chloroma, the patient received an individualized multi-agent chemotherapy based on induction chemotherapy for older patients in combination with InO. After four administrations of InO, in combination with vincristine, dexamethasone, cytarabine, and cyclophosphamide, CT-imaging showed a reduction in volume of the chloroma and response to therapy. Consolidation with high-dose methotrexate and pegaspargase was administered. The patient developed toxic liver damage manifested by hyperbilirubinemia and progressive hepatic encephalopathy. The diagnostic criteria for VOD were met, and therapy with defibrotide was initiated. Liver biopsy revealed no histological signs of VOD but instead steatohepatitis indicative of drug-induced toxicity. The patient ultimately died of hemorrhagic shock through postinterventional hemorrhage after liver biopsy. In conclusion, although InO shows promising results in the therapy of r/r ALL with and without additional chemotherapy, the combination with MTX and pegaspargase in an intensively pretreated patient with relapse after HCST may impart an increased risk for liver-related toxicity. Special caution is required when assessing fitness for further liver toxic regimens. A key takeaway is also the reminder that InO can cause liver damage not only in the form of VOD but also through direct hepatocellular toxicity.
Assuntos
Asparaginase , Falência Hepática , Polietilenoglicóis , Leucemia-Linfoma Linfoblástico de Células Precursoras , Sarcoma Mieloide , Feminino , Humanos , Pessoa de Meia-Idade , Cromossomo Filadélfia , Sarcoma Mieloide/tratamento farmacológico , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamento farmacológico , Inotuzumab Ozogamicina/uso terapêutico , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Falência Hepática/induzido quimicamente , RecidivaRESUMO
BACKGROUND: Endoscopic transmural drainage (ETD) using double-pigtail stents (DPSs) is a well-established treatment for walled-off pancreatic necrosis (WON). This study aimed to compare outcomes in patients undergoing ETD with DPSs left indwelling versus those where stents were removed or migrated. METHODS: This retrospective multicenter cohort study included patients with WON who underwent ETD using DPSs between July 2001 and December 2019. The primary outcome was recurrence of a pancreatic fluid collection (PFC). Secondary outcomes were long-term complications and recurrence-associated factors. Competing risk regression analysis considered DPS removal or migration as time-varying covariates. RESULTS: Among 320 patients (median age 58; 36% women), DPSs were removed in 153 (47.8%), migrated spontaneously in 27 (8.4%), and remained indwelling in 140 (43.8%). PFC recurrence was observed in 57 patients (17.8%): after removal (n = 39; 25.5%); after migration (n = 4; 14.8%); in patients with indwelling DPSs (n = 14; 10.0%). In 25 patients (7.8%), drainage of recurrent PFC was indicated. Risk factors for recurrence were DPS removal or migration (hazard ratio [HR] 3.45, 95%CI 1.37-8.70) and presence of a disconnected pancreatic duct (HR 5.08, 95%CI 1.84-14.0). CONCLUSIONS: Among patients who undergo ETD of WON, leaving DPSs in situ seems to lower the risk of recurrent fluid collections, without any long-term DPS-related complications. These results suggest that DPSs should not be routinely removed and can be safely left indwelling indefinitely.
Assuntos
Drenagem , Pancreatite Necrosante Aguda , Plásticos , Recidiva , Stents , Humanos , Feminino , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Stents/efeitos adversos , Drenagem/métodos , Drenagem/efeitos adversos , Pancreatite Necrosante Aguda/cirurgia , Idoso , Remoção de Dispositivo/métodos , Adulto , Migração de Corpo Estranho/etiologia , Endoscopia do Sistema Digestório/métodosRESUMO
INTRODUCTION: Transversus abdominis release (TAR) is increasingly being performed for reconstruction of complex incisional and recurrent ventral hernias, with complication rates ranging from 17.4% to 33.3% after open TAR (oTAR) or robotic TAR (rTAR). The purpose of this study was to describe the outcomes of patients undergoing TAR with macroporous polypropylene mesh (MPM) and to compare outcomes between oTAR and rTAR. METHODS: A retrospective review of 183 consecutive patients undergoing TAR with MPM performed by a single surgeon at a single institution from 2015 to 2021 was performed. Patients with less than one year of follow-up were excluded. Univariate analysis was performed to compare outcomes between oTAR and rTAR patients. RESULTS: Average patient age was 59.4 y, median body mass index was 33.2 kg/m2, and median hernia width was 12.0 cm. Forty 2 (23%) patients underwent oTAR, 127 (69%) underwent rTAR, and 14 (8%) underwent laparoscopic TAR. Patients experienced 16.4%, 10.4%, 3.8%, and 6.0% rates of overall complications, surgical site occurrences, surgical site infections, and other complications, respectively. At average follow-up of 2.3 y, a 2.7% hernia recurrence rate was observed. In comparison to patients undergoing oTAR, rTAR patients required shorter operative times and length of stay, and were less likely to experience postoperative complications overall, and other complications. Recurrence rates were similar between oTAR and rTAR. CONCLUSIONS: Patients undergoing TAR with MPM experienced complication and recurrence rates in alignment with previously published results. In comparison to oTAR, rTAR was associated with more favorable perioperative outcomes and complication rates, but similar recurrence rates.
Assuntos
Músculos Abdominais , Hérnia Ventral , Herniorrafia , Polipropilenos , Complicações Pós-Operatórias , Telas Cirúrgicas , Humanos , Telas Cirúrgicas/efeitos adversos , Pessoa de Meia-Idade , Estudos Retrospectivos , Masculino , Feminino , Idoso , Hérnia Ventral/cirurgia , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Herniorrafia/instrumentação , Herniorrafia/métodos , Herniorrafia/efeitos adversos , Resultado do Tratamento , Procedimentos Cirúrgicos Robóticos/efeitos adversos , Procedimentos Cirúrgicos Robóticos/métodos , Recidiva , Adulto , Seguimentos , Laparoscopia/efeitos adversos , Laparoscopia/métodosRESUMO
PURPOSE: To compare patency and reintervention outcomes after either plain old balloon angioplasty (POBA) or drug-coated balloon angioplasty (DCBA) for venous stenoses after percutaneous arteriovenous fistula (pAVF) creation. MATERIALS AND METHODS: One-hundred ninety-five pAVFs were successfully created during the study period, 141 using Ellipsys and 54 using Wavelinq. After pAVF creation, 95 patients (48.7%) required secondary percutaneous transluminal angioplasty (PTA) with either POBA (n = 55, 58%) or DCBA (n = 40, 42.1%). The most common site for PTA was the juxta-anastomotic segment (75.5%; 74/98). Univariate and multivariate Cox regression analyses were used to compare target lesion primary patency, access circuit primary patency, secondary patency, and reintervention rates in the POBA and DCBA cohorts. RESULTS: Thirty-four of 55 (62%) patients in the POBA cohort and 14 of 40 (35%) patients in the DCBA cohort required reinterventions for pAVF restenosis. Mean number of follow-up days among patients treated with POBA was 1,030.4 (SD ± 342.9) and among those treated with DCBA was 744.4 (SD ± 403.5). The use of POBA compared with DCBA was not associated with target lesion and access circuit primary patency loss in multivariate analysis (hazard ratio [HR], 1.81; 95% CI, 0.93-3.51; P = .080; and HR, 1.77; 95% CI, 0.73-4.28; P = .210, respectively). However, time from fistula creation to the first PTA (days) was statistically significantly associated with both outcomes (HR, 0.997; 95% CI, 0.994-0.999; P = .009; and HR, 0.997; 95% CI, 0.992-0.999; P = .021, respectively). There were no major adverse events. CONCLUSIONS: In this retrospective single-center analysis of pAVFs, considerably more patients who underwent PTA with POBA after pAVF creation required reinterventions compared with PTA using DCBA, although the follow-up time of POBA was longer. In multivariate analysis, no differences were noted in the hazard of patency loss between POBA and DCBA.
Assuntos
Angioplastia com Balão , Derivação Arteriovenosa Cirúrgica , Materiais Revestidos Biocompatíveis , Oclusão de Enxerto Vascular , Grau de Desobstrução Vascular , Humanos , Feminino , Masculino , Angioplastia com Balão/instrumentação , Angioplastia com Balão/efeitos adversos , Derivação Arteriovenosa Cirúrgica/efeitos adversos , Pessoa de Meia-Idade , Idoso , Resultado do Tratamento , Estudos Retrospectivos , Oclusão de Enxerto Vascular/etiologia , Oclusão de Enxerto Vascular/terapia , Oclusão de Enxerto Vascular/fisiopatologia , Oclusão de Enxerto Vascular/diagnóstico por imagem , Fatores de Risco , Fatores de Tempo , Diálise Renal , Desenho de Equipamento , Análise Multivariada , Recidiva , Modelos de Riscos Proporcionais , Fármacos Cardiovasculares/administração & dosagem , Fármacos Cardiovasculares/efeitos adversos , Dispositivos de Acesso Vascular , Retratamento , Estimativa de Kaplan-MeierRESUMO
PURPOSE: To assess the different adjunctive catheter techniques required to achieve complete occlusion of renal arteriovenous malformations (rAVMs) of different angioarchitectural types. MATERIALS AND METHODS: Overall, data on 18 patients with rAVM (Type 1, n = 7; Type 2, n = 2; Type 3, n = 9; mean age, 53.8 years) who underwent 25 procedures between 2011 and 2022 were reviewed. The clinical presentations, endovascular techniques, arteriovenous malformation (AVM) occlusion rate, adverse events (including the incidence of renal infarction), and clinical symptoms and outcomes (including recurrence/increase of AVM) were analyzed according to the Cho-Do angioarchitectural classification. Posttreatment renal infarction was classified as no infarction, small infarction (<12.5%), medium infarction (12.5%-25%), and large infarction (>25%) using contrast-enhanced computed tomography or magnetic resonance imaging. RESULTS: Hematuria and heart failure were presenting symptoms in 10 and 2 patients, respectively. The embolic materials used were as follows: Type 1 rAVM, coils alone or with n-butyl-2-cyanoacrylate (nBCA); Type 2 rAVM, nBCA alone or with coils; and Type 3 rAVMs, nBCA alone. Fourteen patients underwent adjunctive catheter techniques, including flow control with a balloon catheter and multiple microcatheter placement, alone or in combination. Immediate postprocedural angiography revealed complete occlusion in 15 patients (83%) and marked regression of rAVM in 3 (17%). Small asymptomatic renal infarctions were observed in 6 patients with Type 3 rAVMs without any decrease in renal function. No major adverse events were observed. All symptomatic patients experienced symptom resolution. Recurrence/increase of rAVM was not observed during the mean 32-month follow-up period (range, 2-120 months). CONCLUSIONS: Transarterial embolization using adjunctive catheter techniques according to angioarchitectural types can be an effective treatment for rAVMs.
Assuntos
Malformações Arteriovenosas , Embolização Terapêutica , Artéria Renal , Veias Renais , Humanos , Pessoa de Meia-Idade , Feminino , Masculino , Embolização Terapêutica/efeitos adversos , Malformações Arteriovenosas/terapia , Malformações Arteriovenosas/diagnóstico por imagem , Resultado do Tratamento , Adulto , Estudos Retrospectivos , Idoso , Artéria Renal/diagnóstico por imagem , Artéria Renal/anormalidades , Veias Renais/diagnóstico por imagem , Veias Renais/anormalidades , Angiografia por Tomografia Computadorizada , Valor Preditivo dos Testes , Recidiva , Fatores de Tempo , Adulto Jovem , Fatores de Risco , Embucrilato/administração & dosagem , Infarto/diagnóstico por imagem , Infarto/etiologia , Infarto/terapia , Angiografia por Ressonância MagnéticaRESUMO
BACKGROUND: Pathogenesis and relapse of steroid-sensitive nephrotic syndrome (SSNS) are primarily associated with infection. Dental caries is the most common chronic progressive oral infection in children. However, clinical studies of SSNS combined with dental caries in children are rare. METHODS: In our retrospective cohort study from January 2021 to June 2022, 145 children with SSNS were included in the baseline analysis and 105 in the follow-up analysis. The follow-up period was 1 year. The primary study endpoints were the relapse-free period and frequently relapsing nephrotic syndrome (FRNS). Secondary endpoints included the number and triggers of relapses and concomitant medications. RESULTS: The median age was 5.5 years, with a caries rate of 60.7%, the mean DMFT/dmft was 3.86, and the caries filling rate was 1.6%. Except for the lower proportion of high household income and high parental education observed in the caries group, no statistical differences were found when comparing the other baseline data with the non-caries group. The caries group had a shorter relapse-free period and a lower 1-year cumulative relapse-free survival rate (HR = 1.90, 95% CI 1.17-3.09, P = 0.009). Univariate regression analysis showed caries associated with FRNS (OR = 2.714, 95% CI 1.021-7.219, P = 0.045), but the correlation no longer remained in the multivariate analysis. Additionally, seven cases of caries-derived pulpal periapical inflammation triggered SSNS relapses. The caries group had more infection triggers and concomitant medication use. CONCLUSION: Dental caries and relapse of SSNS are potentially associated, but careful evaluation is needed.
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Cárie Dentária , Síndrome Nefrótica , Criança , Humanos , Pré-Escolar , Síndrome Nefrótica/tratamento farmacológico , Síndrome Nefrótica/epidemiologia , Estudos Retrospectivos , Cárie Dentária/epidemiologia , Inflamação , RecidivaRESUMO
AIM: Chronic graft versus host disease (cGVHD) is a major cause of morbidity postallogeneic peripheral blood stem cell transplant (PBSCT). There is paucity of literature describing incidence, risk factors, characteristics, and outcome of cGVHD in children undergoing haploidentical PBSCT with post-transplant cyclophosphamide (PTCy). Here, we describe our experience from our center regarding the same. METHODS: All children who underwent haploidentical PBSCT with PTCy between January 2016 and December 2021 at our center and survived beyond day+100 post-transplant were included in this retrospective study. Conditioning regimens used were: Thiotepa-Fludarabine-Cyclophosphamide with 2 Gy single fraction total body irradiation, Thiotepa-Busulfan-Fludarabine, Fludarabine-total body irradiation and Fludarabine-Melphalan. Peripheral blood was used as stem cell source in all patients. GVHD prophylaxis was PTCy 50 mg/kg on day +3 and +4, Mycophenolate mofetil and Calcineurin inhibitors. Clinical and laboratory data was electronically retrieved and analyzed based on National Institute of Health Consensus Criteria-2014 at regular intervals. Impact of various patient, donor, and transplant-related factors on development of cGVHD were analyzed. Incidence of relapse, event free survival (EFS) and overall survival (OS) were calculated and compared between cGVHD and no cGVHD groups. Patients with rejection were excluded from risk factor analysis for cGVHD but were considered for survival analysis. RESULTS: Fifty-one children included in this study. Median age of transplant of our cohort was 7.5 years with male:female=1.6:1. Eight patients had rejection with autologous recovery. History of acute GVHD (aGVHD) was present in 15/51 (Grade III to IV in 7/51). cGVHD developed in 19/51 patients (mild-9/51, moderate-6/51, and severe-4/51). Skin was the most common organ involved (100%) followed by gastrointestinal tract (47.4%), liver (36.8%), eyes (21%), lungs (21%), mouth (15.7%), and joints (5.2%). Advanced donor age (>30 y) and previous aGVHD were found to be significantly associated with increased risk of developing cGVHD. At last follow-up, complete response and partial response of cGVHD was seen in 6/19 and 4/19 patients, respectively. Overall mortality was 15/51 (cause of mortality was relapse of cancer 8/15, cGVHD-3/15, other 4/15). EFS and OS of full cohort was 55% and 70.6%, respectively. Compared with patients without cGVHD, patients with cGVHD demonstrated a lower relapse (18.2% vs. 40%, P =0.2333), higher EFS (68.4% vs. 53.1%, P =0.283), and higher OS (73.7% vs. 68.8%, P =0.708). CONCLUSION: Incidence of cGVHD was high in children undergoing haploidentical PBSCT with PTCy. Other than PBSC graft source; donor age and previous aGVHD were the risks factors for development of cGVHD. Patients with cGVHD had lower incidence of relapse translating into better survival but this difference was not statistically significant.
Assuntos
Síndrome de Bronquiolite Obliterante , Doença Enxerto-Hospedeiro , Transplante de Células-Tronco Hematopoéticas , Transplante de Células-Tronco de Sangue Periférico , Criança , Humanos , Masculino , Feminino , Transplante de Células-Tronco de Sangue Periférico/efeitos adversos , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Estudos Retrospectivos , Incidência , Tiotepa/uso terapêutico , Ciclofosfamida/uso terapêutico , Doença Enxerto-Hospedeiro/etiologia , Doença Enxerto-Hospedeiro/prevenção & controle , Doença Enxerto-Hospedeiro/tratamento farmacológico , Fatores de Risco , Recidiva , Condicionamento Pré-Transplante/efeitos adversosRESUMO
BACKGROUND: Pancreatic fluid collections (PFCs) may recur after resolution with endoscopic transmural drainage (ETD) and standard stent removal (SSR). Herein, we compared the efficacy and safety of leaving long-term indwelling plastic stents (LTIS) vs. standard stent removal after PFC resolution with ETD. METHODS: We performed a systematic review of MEDLINE, EMBASE, CINAHL, Scopus, and Cochrane databases from inception to September 2022. Full-text articles comparing long-term (> 6 months) outcomes of LTIS and SSR were eligible, as well as single-arm studies with ≥ 10 patients with LTIS. Two independent reviewers selected studies, extracted data, and assessed the risk of bias using the Newcastle-Ottawa Scale. Measured outcomes included the following: (A) PFC recurrence; (B) interventions for PFC recurrence; (C) technical success; and (D) adverse events (AEs). Meta-analysis was carried out using random-effects models. RESULTS: We included 16 studies, encompassing 1285 patients. Compared to SSR after PFC resolution with ETD, LTIS was associated with significantly lower risk of PFC recurrence (3% vs. 23%; OR 0.22 [95%CI 0.09-0.52]; I2 = 45%) and need for interventions (2% vs. 14%; OR 0.35 [95%CI 0.16-0.78]; I2 = 0%). The superiority of LTIS on reducing PFC recurrence was found with walled-off necrosis, with or without disconnected pancreatic duct, and with placement of ≥ 2 LTIS. When using LTIS, the pooled proportion of AEs was 8% (95%CI 4-11%) and technical success was 93% (95%CI 86-99%). CONCLUSIONS: Our results show that LTIS after PFC resolution with ETD is feasible, safe, and superior to SSR in reducing the risk of PFC recurrence and need for interventions.
Assuntos
Remoção de Dispositivo , Drenagem , Suco Pancreático , Stents , Humanos , Remoção de Dispositivo/métodos , Drenagem/métodos , Plásticos , Recidiva , Resultado do Tratamento , Suco Pancreático/metabolismoRESUMO
BACKGROUND: Rebleeding is a significant complication of endoscopic injection of cyanoacrylate in gastric varices in cirrhotic patients. AIM: This systematic review and meta-analysis aimed to evaluate the efficiency of endoscopic cyanoacrylate injection and summarized the risk factors for rebleeding. METHODS: Databases were searched for articles published between January 2012 and December 2022. Studies evaluating the efficiency of endoscopic injection of cyanoacrylate glue for gastric varices and the risk factors for rebleeding were included. RESULTS: The final analysis included data from 24 studies. The hemostatic rates ranged from 65 to 100%. The pooled rate of gastric varices recurrence was 34% [95% CI 21-46, I2 = 61.4%], early rebleeding rate was 16% [95% CI 11-20, I2 = 37.4%], late rebleeding rate was 39% [95% CI 36-42, I2 = 90.9%], mild and moderate adverse events rate were 28% [95% CI 24-31, I2 = 91.6%], 3% [95% CI - 2 to 8, I2 = 15.3%], rebleeding-related mortality rate was 6% [95% CI 2-10, I2 = 0%], all-cause mortality rate was 17% [95% CI 12-22, I2 = 63.6%]. Independent risk factors for gastric variceal rebleeding included portal venous thrombosis, ascites, cyanoacrylate volume, fever/systemic inflammatory response syndrome, red Wale sign, previous history of variceal bleeding, active bleeding and paragastric veins. The use of proton pump inhibitors could be a protective factor. CONCLUSIONS: Endoscopic cyanoacrylate glue injection is an effective and safe treatment for gastric varices. Cirrhotic patients with the above risk factors may benefit from treatment aimed at reducing portal hypertension, antibiotic prophylaxis, and anticoagulation if they meet the indications.
Assuntos
Cianoacrilatos , Varizes Esofágicas e Gástricas , Hemorragia Gastrointestinal , Recidiva , Humanos , Varizes Esofágicas e Gástricas/terapia , Varizes Esofágicas e Gástricas/etiologia , Cianoacrilatos/administração & dosagem , Cianoacrilatos/efeitos adversos , Hemorragia Gastrointestinal/etiologia , Hemorragia Gastrointestinal/terapia , Fatores de Risco , Adesivos Teciduais/administração & dosagem , Cirrose Hepática/complicações , Hemostase Endoscópica/métodosRESUMO
BACKGROUND: Currently, the type of patch used for carotid endarterectomy closure depends on the preference of the operating surgeon. Various materials are available, including autologous venous patches, bovine pericardial patches (BPP), and synthetic patches. The purpose of this study was to compare the long-term outcomes. METHODS: All patients who underwent primary carotid endarterectomy with patch angioplasty using a venous, bovine, or polyester patch between 2010 and 2020 at two high-volume medical centers were included in this retrospective analysis on largely prospectively collected data. Study endpoints included long-term ipsilateral transient ischemic attack or cerebrovascular accident, restenosis, reintervention, and all-cause mortality. Cox proportional hazard models were fitted to assess the effect of patch type to each outcome. RESULTS: In total, 1481 CEAs were performed with a follow-up of 32 (13-65) months. Venous patch was used in 309 patients (20.9%), BPP in 1000 patients (67.5%), and polyester patch in 172 patients (11.6%). A preoperative symptomatic carotid artery stenosis of >50% was observed in 91.9% (n = 284) of the patients who received a venous patch, 92.1% (n = 921) of the patients who received BPP, and 90.7% (n = 156) of the patients who received a polyester patch (p = 0.799). Only in selected patients with an asymptomatic stenosis of >70% surgery was considered. Multivariable analyses showed no significant differences between the three patch types regarding long-term outcomes after adjusting for confounders. CONCLUSIONS: In patients undergoing primary carotid endarterectomy, the use of venous, bovine pericardial, or polyester patches seems equally safe and durable in terms of comparability in long-term outcomes.
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Estenose das Carótidas , Endarterectomia das Carótidas , Acidente Vascular Cerebral , Humanos , Bovinos , Animais , Endarterectomia das Carótidas/efeitos adversos , Poliésteres , Estudos Retrospectivos , Resultado do Tratamento , Estenose das Carótidas/cirurgia , Acidente Vascular Cerebral/etiologia , RecidivaRESUMO
BACKGROUND: This Bayesian network meta-analysis (NMA) sought to evaluate the efficacy of different endovascular treatments for femoropopliteal artery in-stent restenosis (FP-ISR). METHODS: PubMed, Embase, Cochrane Central Register of Controlled Trials, and Web of science for clinical trials from database inception to March 31, 2023, with no language restrictions to retrieve randomized controlled trials or cohort studies evaluating the impact of any kind of endovascular treatments for FP-ISR. Pair-wise meta-analysis and Bayesian NMA were performed to pool the outcome estimates different endovascular treatments. The primary end points under consideration were primary patency rates at both 6-month and 12-month follow-up. RESULTS: A total of 15 studies with 1,424 patients were ultimately enrolled to be analyzed, 7 types of endovascular treatment were identified for comparison. In terms of primary patency and freedom from target lesion revascularization (TLR) at 6-month and12-month follow-up, the direct meta-analysis findings showed that drug-coated balloons (DCB) and covered stent (CS) are considerably superior to plain old balloon angioplasty (POBA), Excimer laser atherectomy (ELA) + DCB is significantly better than DCB. According to the meta-analysis based on Bayesian theory, during the 6-month and 12-months follow-up, we could not find significant difference between the different treatments in terms of the primary patency and the freedom from TLR, based on the surface values under the cumulative ranking curve (SUCRA), CS was considered the best treatment in terms of primary patency (6 months SUCRA = 85.2; 12 months SUCRA = 78.9) and freedom from TLR (6 months SUCRA = 84.9; 12 months SUCRA = 70.9); directional atherectomy + POBA may lead to higher survival rate at 12 months (SUCRA = 89.1) than others treatments; in addition, both ELA + POBA and ELA + DCB have higher limb salvage than POBA. CONCLUSIONS: The findings of this NMA suggest that CS showed positive encouraging results in primary patency and TLR in FP-ISR at 6 and 12 months. However, due to the potential influence of certain confounding factors, the long-term results necessitate validation through numerous randomized controlled trials.
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Procedimentos Endovasculares , Artéria Femoral , Doença Arterial Periférica , Artéria Poplítea , Stents , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Angioplastia com Balão/instrumentação , Angioplastia com Balão/efeitos adversos , Teorema de Bayes , Materiais Revestidos Biocompatíveis , Procedimentos Endovasculares/instrumentação , Procedimentos Endovasculares/efeitos adversos , Artéria Femoral/fisiopatologia , Artéria Femoral/diagnóstico por imagem , Metanálise em Rede , Doença Arterial Periférica/terapia , Doença Arterial Periférica/fisiopatologia , Doença Arterial Periférica/diagnóstico por imagem , Artéria Poplítea/fisiopatologia , Artéria Poplítea/diagnóstico por imagem , Recidiva , Fatores de Risco , Fatores de Tempo , Resultado do Tratamento , Grau de Desobstrução VascularRESUMO
BACKGROUND: After drug-coated balloon (DCB) treatment of the femoropopliteal artery in-stent restenosis (ISR), a certain proportion of patients also experience target lesion restenosis. The purpose of this study was to explore the efficacy and safety of rivaroxaban combined with aspirin in the treatment of ISR after DCB intervention. METHODS: Patients who underwent DCB treatment for ISR after femoropopliteal artery intervention at our center from March 2017 to February 2022 were included consecutively. According to the drug treatment after DCB intervention of ISR, the patients were divided into rivaroxaban and aspirin group (RA Group) and dual antiplatelet therapy (DAPT) group. The outcomes of 2 groups during the 12-month follow-up after DCB intervention were compared. RESULTS: A total of 92 patients were included in final analysis, with 43 in RA group and 49 in DAPT group. During 12-month follow-up, a total of 15 cases of recurrent ISR were detected, and the recurrence rate of ISR and clinically driven target lesion revascularization in the RA group were lower than those in the DAPT group (P < 0.05). The vascular patency rate in the RA group was higher than that in the DAPT group at 6 and 12 months of follow-up (P < 0.05). During the follow-up, there were no adverse events such as death, myocardial infarction, stroke, amputation, or major bleeding, and only a total of 5 cases of minor bleeding occurred. CONCLUSIONS: Compared with the standard DAPT regimen, rivaroxaban combined with aspirin can safely improve the follow-up outcome after DCB for femoropopliteal ISR.
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Angioplastia com Balão , Aspirina , Materiais Revestidos Biocompatíveis , Inibidores do Fator Xa , Artéria Femoral , Doença Arterial Periférica , Inibidores da Agregação Plaquetária , Artéria Poplítea , Recidiva , Rivaroxabana , Grau de Desobstrução Vascular , Humanos , Masculino , Feminino , Artéria Femoral/diagnóstico por imagem , Artéria Femoral/fisiopatologia , Rivaroxabana/administração & dosagem , Rivaroxabana/efeitos adversos , Estudos Retrospectivos , Idoso , Aspirina/administração & dosagem , Aspirina/efeitos adversos , Doença Arterial Periférica/terapia , Doença Arterial Periférica/fisiopatologia , Doença Arterial Periférica/diagnóstico por imagem , Angioplastia com Balão/instrumentação , Angioplastia com Balão/efeitos adversos , Artéria Poplítea/fisiopatologia , Artéria Poplítea/diagnóstico por imagem , Inibidores da Agregação Plaquetária/administração & dosagem , Inibidores da Agregação Plaquetária/efeitos adversos , Pessoa de Meia-Idade , Resultado do Tratamento , Fatores de Tempo , Inibidores do Fator Xa/administração & dosagem , Inibidores do Fator Xa/efeitos adversos , Stents , Dispositivos de Acesso Vascular , Fatores de Risco , Idoso de 80 Anos ou mais , Terapia Antiplaquetária DuplaRESUMO
PURPOSE: To report the rate of retinal redetachment after silicone oil removal following rhegmatogenous retinal detachment surgery and to determine potential risk factors. METHODS: Retrospective observational case series of 161 eyes who underwent rhegmatogenous retinal detachment surgery and subsequent silicone oil removal. Pre- and intraoperative risk factors were evaluated using univariate and multivariate logistic regression. We also evaluated the effect of tamponade duration on anatomical outcomes. RESULTS: The median tamponade duration was 5.9 [4.3;7.6] months. Seventeen (10.6%) eyes underwent silicone oil removal within 3 months of surgery, with a median delay of 2.3 [2.0;2.8] months. The rate of retinal detachment after silicone oil removal was 14.9%. A history of previous unsuccessful surgery was the only significant risk factor for retinal redetachment after silicone oil removal (OR 4.8, 95%CI [1.5;19.0], p = 0.02). The use of 360° laser retinopexy and concomitant air or gas tamponade during silicone oil removal were not found to affect the redetachment rate. Eyes with silicone oil tamponade ≤ 3 months showed an increased, albeit not significant, risk of developing recurrent rhegmatogenous retinal detachment after silicone oil removal (35.3% versus 12.5%, p = 0.06). CONCLUSION: A retinal redetachment occurred in 14.9% of eyes undergoing silicone oil removal following rhegmatogenous retinal detachment surgery. Previous failed surgery was associated with a 4.8-fold increased risk of developing recurrent rhegmatogenous retinal detachment after silicone oil removal. Eyes with silicone oil tamponade ≤ 3 months tended to have a higher redetachment rate. TRIAL REGISTRATION NUMBER: ID NCT05647928 (12th April 2022).
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Tamponamento Interno , Descolamento Retiniano , Óleos de Silicone , Vitrectomia , Humanos , Descolamento Retiniano/cirurgia , Estudos Retrospectivos , Feminino , Óleos de Silicone/efeitos adversos , Masculino , Fatores de Risco , Pessoa de Meia-Idade , Tamponamento Interno/efeitos adversos , Vitrectomia/efeitos adversos , Recidiva , Adulto , Acuidade Visual , Idoso , Complicações Pós-Operatórias , DrenagemRESUMO
BACKGROUND: A straight silicone stent can be used to treat proximal benign tracheal stenosis in non-surgical candidates. However, stent migration is a common complication when placed at a particular location and can lead to major complications. This case series of laryngotracheal stenosis reports a fixation method for straight silicone stents in the subglottic trachea (Stage 3 of the McCaffrey classification). METHODS: The medical charts of these patients scheduled for straight silicone stent placement with suture fixation between 2014 and 2020 at the CHU UCL Namur Hospital (Belgium) were retrospectively reviewed. The procedure was performed using a rigid bronchoscope. Details of the procedure were obtained from medical records. RESULTS: This case series included six patients (males: 4, females: 2). The median patient age was 59 years. Two suture fixations were placed following previous silicone stent migration episodes, whereas the others were placed proactively to avoid this risk. All fixations were performed by the device Freka® Pexact II ENFIt®, originally developed for gastropexy in endoscopic gastrostomy. The sutures were subcutaneously buried. CONCLUSIONS: During the 6-month follow-up period, complications such as fixation issues and stent migration were reported despite the off-label use of the treatment. The straight silicone stent fixation technique used in this case series was simple and effective for securing the stent in upper benign tracheal stenosis.
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Laringoestenose , Stents , Estenose Traqueal , Humanos , Feminino , Pessoa de Meia-Idade , Masculino , Estenose Traqueal/cirurgia , Laringoestenose/cirurgia , Estudos Retrospectivos , Idoso , Adulto , Técnicas de Sutura , Recidiva , Silicones , BroncoscopiaRESUMO
OBJECTIVES: The aim of this study is to investigate the neurovascular bundle (NVB) as a potential orthodontic relapse factor. The mechanical properties and the forces generated in the NVB after orthodontic extrusion are explored. MATERIALS AND METHODS: Six NVBs branching from the inferior alveolar nerve to the apices of the mandibular canines and premolars of mature pigs were harvested. Stress relaxation tests were conducted. A standard linear solid model (SLS) was utilized to simulate the orthodontic extrusion of a single rooted tooth with NVB length and cross-sectional diameter of 3.6 and 0.5 mm, respectively, so the NVB was stretched 10% and 20% of its original length. The maximum force within the NVB was then calculated. RESULTS: Based on our data, the average Young's modulus before relaxation ( E 0 ), after relaxation ( E P ) and the difference between Young's moduli before and after relaxation ( E S ) were 324 ± 123, 173 ± 73 and 151 ± 52 kPa, respectively. The theoretical force within the NVB stretched to 10% and 20% strain was 3 and 5 mN, respectively. CONCLUSION: The data from our study indicate that the NVB exhibits stress relaxation, a characteristic trait of viscoelastic materials. SLS model simulation predicted residual forces around 5 mN for elongation up to 20%. We observed strain hardening with additional elongation, which has the potential to cause forces to increase exponentially. Therefore, tensile forces in the NVB should not be ruled out as a contributor to orthodontic relapse, especially in adult patients who may have decreased adaptability of their NVB. Further preclinical and clinical models should be developed to further clarify what is the contribution of the NVB to orthodontic relapse.
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Ortodontia Corretiva , Animais , Suínos , Recidiva , Nervo Mandibular , Dente , Estresse MecânicoRESUMO
When treating posterior crossbite, the primary goal is to achieve long-term crossbite correction. The majority of studies however focus on relapse of the increase in the transverse dimension, but not relapse of the crossbite itself, which is an essential outcome. The aim of the present study was to determine long-term stability (2 years minimum post-treatment) of posterior crossbite correction, treated in mixed or early permanent dentitions of growing children. Following registration in PROSPERO (CRD42022348858), an electronic literature search including PubMed, Embase, Web of Science, the Cochrane Library, and a manual search were conducted up to January 2023, to identify longitudinal studies looking into the long-term stability of crossbite correction in growing children. Data extraction and risk of bias assessment were carried out, and subsequently, a random-effects meta-analyses models were used to calculate estimates for relapse of the crossbite and relapse at the transverse level. Twenty-two studies were included, of varying designs and quality, representing 1076 treated patients, with different expansion appliances and protocols. Meta-analysis results showed that 19.5% (95% CI: 15%; 25%) of patients present with relapse of posterior crossbite at long-term follow-up. At the transverse level, 19.3% of the total expansion (including overexpansion) relapsed (95% CI: 13%; 27%) regardless of whether there a was relapse of the crossbite itself. Data from existing studies, with a moderate level of evidence, indicate that the long-term stability of posterior crossbite correction in growing children is unfavourable in roughly 1 in 5 growing children, with crossbite relapse long-term. On average, 19% of the maxillary expansion performed (including overexpansion) relapses long-term, which may occur in cases with or without relapse of the crossbite.