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1.
Artigo em Inglês | MEDLINE | ID: mdl-39073002

RESUMO

Despite remarkable advances in immunotherapy, melanoma remains a significant cause of cancer mortality. Many factors concerning melanoma mortality are poorly understood, posing an obstacle to optimal care. We conducted a retrospective observational cohort study of 183 patients with metastatic melanoma who died following immunotherapy treatment to investigate sites of metastases at death, settings of death, and mechanisms of death. The median time from metastatic diagnosis to death was 16.1 months (range 0.3-135.1 months). Most patients experienced hospitalization within 3 months before death (80.3%), with 31.7% dying while hospitalized, 31.2% while in inpatient hospice, and 29.4% while in home hospice. The most common sites of metastases at death were distant lymph nodes (62.8%), lung (57.9%), liver (50.8%), brain (38.8%), and bone (37.7%). The most common causes of death were progressive failure to thrive (57.5%), respiratory failure (22.4%), and infection (21.8%); the vast majority (87.9%) of patients died from melanoma-specific causes. Overall, 10.9% of patients in our cohort had survival >5 years after metastatic diagnosis, and 76.2% of long-term survivors died due to melanoma. This study describes factors associated with melanoma mortality, highlighting an ongoing need for therapeutic advancements.

2.
J Radiosurg SBRT ; 9(1): 43-52, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-38029012

RESUMO

Background: Proton beam therapy (PBT) is a non-surgical treatment that spares adjacent tissues compared to photon radiation and useful for Hepatocellular carcinoma (HCC) and intrahepatic cholangiocarcinoma (iCCA). We present a single center experience in HCC and iCCA treated with Pencil Beam Scanning (PBS) PBT. Methods: Forty-four consecutive patients (22 patients in each group) receiving PBT were included and reviewed. PBT was delivered with hypofractionated or stereotactic body radiation therapy (SBRT) using PBS. Tumor size was approximated by clinical target volume (CTV). Outcomes were evaluated with Kaplan-Meier and liver toxicity was determined by MELD-Na and albumin-bilirubin (ALBI) grade. Results: Median follow up was 38.7 months, fourteen (35%) had multifocal disease and median CTV was 232.5cc. Four (9%) and 40 (91%) patients received SBRT and hypofractionated radiation, respectively. Two year overall survival was statistically higher for HCC (entire group: 68.9% months [95% CI: 61.3 - 76.3%]; iCCA: 49.8% [95% CI: 38.5% - 61.1%]; HCC: 89.4% [95% CI: 82.3 - 96.5%]; P <0.005). There was no statistical difference in progression-free survival or freedom from local failure. Biologically Equivalent Dose (BED) was greater than or equal to 80.5Gy in 37 (84%) patients. All iCCA patients had stable or improved ALBI grade following treatment. ALBI grade was stable in 83% of HCC patients and average MELD-Na score remained stable. Tumor size, pretreatment liver function, and total radiation dose were not associated with liver toxicity. Conclusions: PBT for unresectable HCC and iCCA is safe and effective, even for large and multifocal tumors. Liver function was preserved even in those with baseline cirrhosis in this advanced population with large tumors.

3.
J Pediatr Surg ; 58(10): 2006-2011, 2023 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-37393165

RESUMO

INTRODUCTION: Pediatric surgery applicants are increasingly pursuing research in non-traditional fields including surgical innovation. This study aims to evaluate the relative value that pediatric surgeons involved in fellow selection place on innovation experience compared to traditional research. METHODS: A cross-sectional web-based survey of American Pediatric Surgical Association members involved in the selection of pediatric surgical fellows was conducted. Respondents reported their own innovation experience and were asked to identify valuable traits of applicants who completed an innovation fellowship. They rated the value of traditional research metrics including publications, presentations, and advanced degrees compared to patents and other innovation-related metrics. Comparisons were made between those with and without innovation experience with respect to gender, years in practice, and institutional role. RESULTS: One hundred thirty respondents were involved in pediatric surgery fellow selection. Innovation work was felt to be equal to or more valuable than basic science by 75% of respondents (84% vs. clinical/outcomes, 93% vs. other non-traditional, 72% vs. other clinical fellowships). Commonly cited concerns included "fewer publications" (21%) and "preoccupation with financial reward" (19%). The most valuable innovation-related metrics were "developing a novel surgical procedure" (67%) and "developing a novel device" (58%). When asked if the respondent would advise a junior resident to pursue an innovation fellowship, 49% would, 9% would not, and 43% were unsure. Seventeen percent expressed concern for match success. CONCLUSION: Innovation experience is generally viewed positively by pediatric surgeons involved in fellow selection. However, applicants and mentors would benefit from focusing on traditional academic outputs to ensure competitiveness. TYPE OF STUDY: Cross-sectional observational study. LEVEL OF EVIDENCE: III.


Assuntos
Internato e Residência , Especialidades Cirúrgicas , Cirurgiões , Criança , Humanos , Estados Unidos , Bolsas de Estudo , Estudos Transversais , Atitude , Inquéritos e Questionários
4.
Surgery ; 174(3): 698-702, 2023 09.
Artigo em Inglês | MEDLINE | ID: mdl-37357096

RESUMO

BACKGROUND: Laparoscopic gastrostomy is commonly performed for durable enteral access in children. T-fasteners have been used intraoperatively to achieve a secure gastropexy, traditionally using external bolsters. We compare the safety profile of a modified paired T-fastener technique to standard laparoscopic-assisted suture gastropexy. METHODS: A retrospective matched case-control study was performed of pediatric patients who underwent laparoscopic gastrostomy at a single center from 2015 to 2021. In the paired T-fastener group, pairs of T-fasteners were passed into the stomach in a square configuration, allowing the suture pairs to be tied subcutaneously. This cohort was matched in a 1:2 fashion with age, sex, and body mass index or weight-matched controls who underwent laparoscopic gastrostomy with buried transabdominal gastropexy. RESULTS: Thirty patients underwent laparoscopic gastrostomy using the paired T-fastener technique and were matched to 60 controls. There was no significant difference in median operative time or 30-day complication rates between the groups, but the paired T-fastener technique significantly reduced the number of trocars required, and it was used for patients with thicker abdominal walls. CONCLUSION: We demonstrate the modified paired T-fastener technique as a safe, efficient means of gastropexy in pediatric laparoscopic gastrostomy. The paired T-fastener approach eliminates external bolsters, reduces additional trocars, and may be advantageous for thicker abdominal walls while maintaining a similar complication profile to standard laparoscopic gastrostomy.


Assuntos
Gastrostomia , Laparoscopia , Humanos , Criança , Gastrostomia/métodos , Estudos Retrospectivos , Estudos de Casos e Controles , Estômago/cirurgia , Laparoscopia/métodos
5.
Blood ; 142(1): 23-32, 2023 07 06.
Artigo em Inglês | MEDLINE | ID: mdl-36928087

RESUMO

WHIM syndrome is an autosomal dominant immunodeficiency disorder caused by gain-of-function mutations in chemokine receptor CXCR4 that promote severe panleukopenia because of retention of mature leukocytes in the bone marrow (BM). We previously reported that Cxcr4-haploinsufficient (Cxcr4+/o) hematopoietic stem cells (HSCs) have a strong selective advantage for durable hematopoietic reconstitution over wild-type (Cxcr4+/+) and WHIM (Cxcr4+/w) HSCs and that a patient with WHIM was spontaneously cured by chromothriptic deletion of the disease allele in an HSC, suggesting that WHIM allele inactivation through gene editing may be a safe genetic cure strategy for the disease. We have developed a 2-step preclinical protocol of autologous hematopoietic stem and progenitor cell (HSPC) transplantation to achieve this goal. First, 1 copy of Cxcr4 in HSPCs was inactivated in vitro by CRISPR/Cas9 editing with a single guide RNA (sgRNA) that does not discriminate between Cxcr4+/w and Cxcr4+/+ alleles. Then, through in vivo natural selection, WHIM allele-inactivated cells were enriched over wild-type allele-inactivated cells. The WHIM allele-inactivated HSCs retained long-term pluripotency and selective hematopoietic reconstitution advantages. To our knowledge, this is the first example of gene therapy for an autosomal dominant gain-of-function disease using a disease allele inactivation strategy in place of the less efficient disease allele repair approach.


Assuntos
Síndromes de Imunodeficiência , Verrugas , Camundongos , Animais , Alelos , Sistemas CRISPR-Cas , RNA Guia de Sistemas CRISPR-Cas , Síndromes de Imunodeficiência/genética , Síndromes de Imunodeficiência/terapia , Verrugas/genética , Verrugas/terapia , Terapia Genética , Receptores CXCR4/genética
6.
J Allergy Clin Immunol Pract ; 11(5): 1401-1416, 2023 05.
Artigo em Inglês | MEDLINE | ID: mdl-36646382

RESUMO

Chronic granulomatous disease (CGD) is a rare inborn error of immunity, resulting from a defect in nicotinamide adenine dinucleotide phosphate oxidation and decreased production of phagocyte reactive oxygen species. The main clinical manifestations are recurrent infections and chronic inflammatory disorders. Current approaches to management include antimicrobial prophylaxis and control of inflammatory complications. Hematopoietic stem cell transplantation or gene therapy can provide definitive treatment. Gastrointestinal and hepatic manifestations are common in CGD and include structural changes, dysmotility, CGD-associated inflammatory bowel disease, liver abscesses, and noncirrhotic portal hypertension. The findings can be heterogeneous, and the management is complex in light of the underlying immune dysfunction. This review describes the various clinical findings and the latest studies in management of gastrointestinal and hepatic manifestations in CGD, as well as the management experience at the National Institutes of Health.


Assuntos
Doença Granulomatosa Crônica , Transplante de Células-Tronco Hematopoéticas , Hipertensão Portal , Humanos , Doença Granulomatosa Crônica/complicações , Doença Granulomatosa Crônica/terapia , Doença Granulomatosa Crônica/genética , Trato Gastrointestinal , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , NADPH Oxidases/genética
8.
Prog Transplant ; 32(4): 274-282, 2022 12.
Artigo em Inglês | MEDLINE | ID: mdl-36367716

RESUMO

INTRODUCTION: The growing practice of living liver donation requires comprehensive understanding of the financial implications for living liver donors. While obtaining and maintaining insurance is important to financial health, little is known about the impact of liver donation on future insurability. RESEARCH QUESTIONS: The purpose of this study was to evaluate the donors' experiences with insurance following donation and identify the insurance provider-driven factors that contribute to donor insurability. DESIGN: A two center cohort of living donors with donation between January 2000 and December 2018 (N = 442) were surveyed about postdonation insurance experiences. To understand insurance provider practices towards liver donors, life (n = 11) and disability (n = 4) insurance underwriters were asked to provide policy quotes for a standardized living liver donor profile. RESULTS: Responses (N = 101) were received by August 2020 (response rate = 22.9%). Living liver donors reported owning life (58%), disability (35%), and medical (87%) insurance at rates comparable to the general population with low proportions reporting difficulty obtaining these insurance types (9%, 9%, 4%, respectively). Post-donation life insurance ownership was associated with post-donation employment (P = 0.01). Underwriter responses indicate life and disability insurability were adversely affected up to 12 months following donation. CONCLUSIONS: Living liver donors did not have difficulty maintaining insurance in the long-term but should be counseled to purchase insurance prior to surgery as short-term insurability may be affected. Perception of difficulty obtaining insurance following donation remains of significant concern among living donors. Further collaboration between the transplant community and insurance companies is warranted.


Assuntos
Transplante de Fígado , Doadores Vivos , Humanos , Inquéritos e Questionários , Emprego , Fígado
9.
Radiol Artif Intell ; 4(5): e210268, 2022 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-36204530

RESUMO

Purpose: To evaluate the performance of a deep learning (DL) model that measures the liver segmental volume ratio (LSVR) (ie, the volumes of Couinaud segments I-III/IV-VIII) and spleen volumes from CT scans to predict cirrhosis and advanced fibrosis. Materials and Methods: For this Health Insurance Portability and Accountability Act-compliant, retrospective study, two datasets were used. Dataset 1 consisted of patients with hepatitis C who underwent liver biopsy (METAVIR F0-F4, 2000-2016). Dataset 2 consisted of patients who had cirrhosis from other causes who underwent liver biopsy (Ishak 0-6, 2001-2021). Whole liver, LSVR, and spleen volumes were measured with contrast-enhanced CT by radiologists and the DL model. Areas under the receiver operating characteristic curve (AUCs) for diagnosing advanced fibrosis (≥METAVIR F2 or Ishak 3) and cirrhosis (≥METAVIR F4 or Ishak 5) were calculated. Multivariable models were built on dataset 1 and tested on datasets 1 (hold out) and 2. Results: Datasets 1 and 2 consisted of 406 patients (median age, 50 years [IQR, 44-56 years]; 297 men) and 207 patients (median age, 50 years [IQR, 41-57 years]; 147 men), respectively. In dataset 1, the prediction of cirrhosis was similar between the manual versus automated measurements for spleen volume (AUC, 0.86 [95% CI: 0.82, 0.9] vs 0.85 [95% CI: 0.81, 0.89]; significantly noninferior, P < .001) and LSVR (AUC, 0.83 [95% CI: 0.78, 0.87] vs 0.79 [95% CI: 0.74, 0.84]; P < .001). The best performing multivariable model achieved AUCs of 0.94 (95% CI: 0.89, 0.99) and 0.79 (95% CI: 0.71, 0.87) for cirrhosis and 0.8 (95% CI: 0.69, 0.91) and 0.71 (95% CI: 0.64, 0.78) for advanced fibrosis in datasets 1 and 2, respectively. Conclusion: The CT-based DL model performed similarly to radiologists. LSVR and splenic volume were predictive of advanced fibrosis and cirrhosis.Keywords: CT, Liver, Cirrhosis, Computer Applications-Detection/Diagnosis Supplemental material is available for this article. © RSNA, 2022.

10.
J Neurol Surg B Skull Base ; 83(Suppl 3): e610, 2022 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-36068907

RESUMO

A case of a recurrent sphenocavernous meningioma is presented. The patient is a 42-year-old male who presented with an episode of transient right-sided numbness. A magnetic resonance imaging (MRI) revealed a large left sphenocavernous meningioma. The patient underwent a frontotemporal craniotomy for tumor resection. Near total resection was achieved with minimal residual in the left cavernous sinus (CS) and orbital apex. The pathology was consistent with meningioma, World Health Organization (WHO) grade I. A follow-up MRI was done 9 months after surgery and showed a growth of the residual tumor, which was treated with intensity modulated radiotherapy. Tumor growth was detected on serial imaging over a 4-year period. Surgical resection was offered. A left frontotemporal craniotomy with pretemporal transcavernous approach was performed. The bone flap was reopened and the dura was opened in a Y-shaped fashion. The roof of the optic canal was drilled off, and the falciform ligament was opened to decompress the optic nerve. The tumor was disconnected from the anterior clinoid region (the anterior clinoid process was eroded by the tumor) and reflected off the wall of the lateral CS. Tumor was adherent to the V2 fascicles (the lateral CS wall was resected in the first surgery) and was sharply dissected off. Gross total resection was achieved. The pathology was consistent with meningioma, WHO grade I. The patient had an unremarkable postoperative course without any new neurological deficits. The link to the video can be found at: https://youtu.be/KVBVw_86JqM .

11.
Ann Rheum Dis ; 81(10): 1453-1464, 2022 10.
Artigo em Inglês | MEDLINE | ID: mdl-35868845

RESUMO

OBJECTIVES: To test the hypothesis that ROSAH (retinal dystrophy, optic nerve oedema, splenomegaly, anhidrosis and headache) syndrome, caused by dominant mutation in ALPK1, is an autoinflammatory disease. METHODS: This cohort study systematically evaluated 27 patients with ROSAH syndrome for inflammatory features and investigated the effect of ALPK1 mutations on immune signalling. Clinical, immunologic and radiographical examinations were performed, and 10 patients were empirically initiated on anticytokine therapy and monitored. Exome sequencing was used to identify a new pathogenic variant. Cytokine profiling, transcriptomics, immunoblotting and knock-in mice were used to assess the impact of ALPK1 mutations on protein function and immune signalling. RESULTS: The majority of the cohort carried the p.Thr237Met mutation but we also identified a new ROSAH-associated mutation, p.Tyr254Cys.Nearly all patients exhibited at least one feature consistent with inflammation including recurrent fever, headaches with meningeal enhancement and premature basal ganglia/brainstem mineralisation on MRI, deforming arthritis and AA amyloidosis. However, there was significant phenotypic variation, even within families and some adults lacked functional visual deficits. While anti-TNF and anti-IL-1 therapies suppressed systemic inflammation and improved quality of life, anti-IL-6 (tocilizumab) was the only anticytokine therapy that improved intraocular inflammation (two of two patients).Patients' primary samples and in vitro assays with mutated ALPK1 constructs showed immune activation with increased NF-κB signalling, STAT1 phosphorylation and interferon gene expression signature. Knock-in mice with the Alpk1 T237M mutation exhibited subclinical inflammation.Clinical features not conventionally attributed to inflammation were also common in the cohort and included short dental roots, enamel defects and decreased salivary flow. CONCLUSION: ROSAH syndrome is an autoinflammatory disease caused by gain-of-function mutations in ALPK1 and some features of disease are amenable to immunomodulatory therapy.


Assuntos
Doenças Hereditárias Autoinflamatórias , NF-kappa B , Proteínas Quinases/genética , Amiloidose , Animais , Estudos de Coortes , Mutação com Ganho de Função , Doenças Hereditárias Autoinflamatórias/genética , Humanos , Inflamação/genética , Camundongos , Mutação , NF-kappa B/genética , NF-kappa B/metabolismo , Proteínas Quinases/metabolismo , Qualidade de Vida , Proteína Amiloide A Sérica , Síndrome , Inibidores do Fator de Necrose Tumoral
12.
Oper Neurosurg (Hagerstown) ; 23(2): 125-132, 2022 08 01.
Artigo em Inglês | MEDLINE | ID: mdl-35838452

RESUMO

BACKGROUND: Optimal management of recurrent neurogenic thoracic outlet syndrome (NTOS) remains a considerable challenge. OBJECTIVE: To assess the safety and effectiveness of reoperative brachial plexus neurolysis in patients with recurrent NTOS. METHODS: From 2009 to 2019, 85 patients underwent reoperative supraclavicular brachial plexus neurolysis for recurrent NTOS after a previous anatomically complete supraclavicular decompression. Data from a prospectively maintained database were analyzed retrospectively. RESULTS: The mean patient age at reoperation was 36.9 ± 1.3 (range 15-64) years, 75% were female, and the interval after previous primary operation was 2.5 ± 0.2 years. Intervening injury had precipitated recurrent NTOS in 14 patients (16%), and the mean Disability of the Arm, Shoulder, and Hand (QuickDASH) score before reoperation was 65.2 ± 2.6, reflecting substantial disability. Operative findings consisted of dense fibrous scar tissue surrounding/encasing the brachial plexus. Compared with the previous primary operations, reoperations had a shorter operative time (198 ± 4 vs 161 ± 5 minutes, P < .01) and hospital stay (4.4 ± 0.2 vs 3.6 ± 0.1 days, P < .01), but there were no significant differences in the frequency of prolonged hospitalization (7.1% vs 4.7%), early reoperation (3.5% vs 1.2%), or 30-day hospital readmission (8.2% vs 7.1%). During a median follow-up of 4.8 years, QuickDASH scores improved by 23.3 ± 2.6 (34.2% ± 3.6%; P < .01) and patient-rated outcomes were excellent in 24%, good in 42%, fair in 26%, and poor in 8%. CONCLUSION: Reoperative supraclavicular brachial plexus neurolysis is technically challenging but safe and effective treatment for recurrent NTOS, with significant improvements in symptoms and function. Diminishing perineural scar tissue development and avoiding secondary injury would likely decrease the need for reoperations.


Assuntos
Plexo Braquial , Síndrome do Desfiladeiro Torácico , Adolescente , Adulto , Plexo Braquial/cirurgia , Cicatriz/cirurgia , Descompressão Cirúrgica , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Reoperação , Estudos Retrospectivos , Síndrome do Desfiladeiro Torácico/cirurgia , Adulto Jovem
13.
Transplant Cell Ther ; 28(11): 747.e1-747.e10, 2022 11.
Artigo em Inglês | MEDLINE | ID: mdl-35878742

RESUMO

Hepatic chronic graft-versus-host disease (cGVHD) causes morbidity and current diagnostic criteria are nonspecific. An accurate diagnosis is imperative because overdiagnosis can lead to unnecessary treatment with immunosuppressive agents and raising the risk of opportunistic infections. We aim to characterize different patterns of liver injury and cytokine profiles associated with hepatic dysfunction in cGVHD, to evaluate the accuracy of the NIH Consensus Criteria (NCC) for hepatic cGVHD and to explore predictors for hepatic cGHVD. Patients were evaluated in this prospective cross-sectional study of patients with cGVHD recruited under a natural history protocol. Laboratory tests and cytokines were measured. The cGVHD were diagnosed and scored based on NCC. Clinically indicated liver biopsy specimens or autopsies were reviewed by an expert hepatopathologist (D.E.K.). Comparisons were made between groups, and univariable and multivariable logistic regression were calculated. Of the 302 patients enrolled, 151 fulfilled hepatic cGVHD based on NCC; however, 69% had at least 1 abnormal liver test result. Abnormal alanine aminotransferase (ALT) and aspartate aminotransferase were associated with lower platelets, higher total bilirubin (TB), total cholesterol, serum amyloid A, and IL 15. Abnormal ALP and gamma-glutamyl transpeptidase were associated with higher cholesterol, and IL7. Lower platelet count was associated with higher ALT, TB, and triglycerides and lower albumin. Of the 27 with liver tissue, 16 had histologic features of GVHD, only eight met clinical criteria for hepatic GVHD. Sensitivity and specificity of NCC in identifying hepatic GVHD were 50% and 27% (Kappa = -0.23). Only 6 had only hepatic GVHD, whereas 10 had hepatic GVHD with either iron overload, nodular regenerative hyperplasia, or steatosis. Multivariable logistic regression showed that ALP and total cholesterol were associated with hepatic GVHD and total cholesterol >220 mg/dL increased the sensitivity for histologic hepatic GVHD. In conclusion, abnormal liver enzymes in cGVHD are nonspecific and have poor correlation with histologic evidence for hepatic GVHD, highlighting the importance of histology. Cytokines provide insight into the pathogenesis of hepatic cGVHD. Decreased platelet count was associated with factors associated with liver disease including portal vein diameter, which may suggest progression of liver disease. This highlights the need of incorporating these factors in natural history study and using liver biopsy to understand the development of liver dysfunction in hematopoietic stem cell transplantation and to develop better instruments to decreased hepatic cGVHD related morbidity and mortality. The study was registered with a ClinicalTrials.gov identifier NCT00092235.


Assuntos
Doença Enxerto-Hospedeiro , Hepatopatias , Humanos , Doença Enxerto-Hospedeiro/diagnóstico , Consenso , Estudos Transversais , Estudos Prospectivos , Doença Crônica , Hepatopatias/diagnóstico , Citocinas/uso terapêutico , Colesterol/uso terapêutico
14.
J Vasc Surg Cases Innov Tech ; 8(2): 287-292, 2022 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-35619944

RESUMO

Supraclavicular operations can be associated with postoperative cutaneous dysesthesia and hypersensitivity. Regenerative peripheral nerve interfaces, created by attaching the proximal end of a divided peripheral nerve into a viable muscle target, can promote neurite regrowth and neuromuscular connections to help suppress painful nerve hyperactivity. During 40 consecutive operations for neurogenic thoracic outlet syndrome, we demonstrated that division of at least one of the superficial supraclavicular cutaneous sensory nerve branches was necessary in 98% of cases. We subsequently developed a novel regenerative peripheral nerve interface for supraclavicular operations using the adjacent omohyoid muscle and have described the technical steps involved in this procedure.

15.
Hepatol Commun ; 6(8): 2210-2220, 2022 08.
Artigo em Inglês | MEDLINE | ID: mdl-35527712

RESUMO

Hepatic graft-versus-host disease (HGVHD) contributes significantly to morbidity and mortality after hematopoietic stem cell transplantation (HSCT). Clinical findings and liver biomarkers are neither sensitive nor specific. The relationship between clinical and histologic diagnoses of HGVHD was assessed premortem and at autopsy. Medical records from patients who underwent HSCT at the National Institutes of Health (NIH) Clinical Center between 2000 and 2012 and expired with autopsy were reviewed, and laboratory tests within 45 days of death were divided into 15-day periods. Clinical diagnosis of HGVHD was based on Keystone Criteria or NIH Consensus Criteria, histologic diagnosis based on bile duct injury without significant inflammation, and exclusion of other potential etiologies. We included 37 patients, 17 of whom had a cholestatic pattern of liver injury and two had a mixed pattern. Fifteen were clinically diagnosed with HGVHD, two showed HGVHD on autopsy, and 13 had histologic evidence of other processes but no HGVHD. Biopsy or clinical diagnosis of GVHD of other organs during life did not correlate with HGVHD on autopsy. The diagnostic accuracy of the current criteria was poor (κ = -0.20). A logistic regression model accounting for dynamic changes included peak bilirubin 15 days before death, and an increase from period -30 (days 30 to 16 before death) to period -15 (15 days before death) showed an area under the receiver operating characteristic curve of 0.77. Infection was the immediate cause of death in 68% of patients. In conclusion, liver biomarkers at baseline and GVHD elsewhere are poor predictors of HGVHD on autopsy, and current clinical diagnostic criteria have unsatisfactory performance. Peak bilirubin and cholestatic injury predicted HGVHD on autopsy. A predictive model was developed accounting for changes over time. Further validation is needed.


Assuntos
Colestase , Doença Enxerto-Hospedeiro , Bilirrubina , Biomarcadores , Colestase/diagnóstico , Estado Terminal , Doença Enxerto-Hospedeiro/diagnóstico , Humanos , Fígado/patologia
16.
Am J Surg ; 224(1 Pt B): 530-534, 2022 07.
Artigo em Inglês | MEDLINE | ID: mdl-35164959

RESUMO

BACKGROUND: Surgical shunts are commonly used to manage complications resulting from extrahepatic portal vein thrombosis (EHPVT) in children. We describe a single-center experience utilizing a functional Side-to-Side Splenorenal Shunt (fSRS), created using either an enlarged inferior mesenteric vein (IMV) or left adrenal vein (LAV). METHODS: Pediatric patients with isolated EHPVT who were poor candidates for a Rex shunt and who underwent a fSRS procedure at our institution between 2003 and 2020 were reviewed. The pre/post shunt portosystemic gradient change, rates of early and late complications, postoperative shunt patency, and mortality were evaluated. RESULTS: Twelve EHPVT patients (mean age of 6.1 years) underwent a fSRS procedure. The mean portosystemic gradient change for the cohort was -11.7 mmHg (±4.9). There were no cases of recurrent variceal bleeding or episodes of shunt thrombosis reported after fSRS procedures. CONCLUSIONS: Surgical shunts continue to be an important adjunct in the treatment of complications related to EHPVT. The functional Side-to-Side Splenorenal Shunt is a safe alternative that is easy to perform, involves minimal dissection and requires only a single anastomosis.


Assuntos
Varizes Esofágicas e Gástricas , Hipertensão Portal , Derivação Esplenorrenal Cirúrgica , Trombose , Trombose Venosa , Criança , Varizes Esofágicas e Gástricas/complicações , Varizes Esofágicas e Gástricas/cirurgia , Hemorragia Gastrointestinal/etiologia , Humanos , Hipertensão Portal/complicações , Hipertensão Portal/cirurgia , Veia Porta/cirurgia , Derivação Portossistêmica Cirúrgica/efeitos adversos , Derivação Portossistêmica Cirúrgica/métodos , Derivação Esplenorrenal Cirúrgica/efeitos adversos , Derivação Esplenorrenal Cirúrgica/métodos , Trombose Venosa/cirurgia
18.
Ann Surg ; 276(6): e1107-e1113, 2022 12 01.
Artigo em Inglês | MEDLINE | ID: mdl-34183509

RESUMO

OBJECTIVE: This study aims to quantify the number of patent-holding surgeons and determine their specialty demographics. SUMMARY BACKGROUND DATA: The number of intellectual property filings related to surgery has exponentially increased over the past 40 years, yet surgeon inventor status among these inventions remains poorly defined. METHODS: A query of the United States Patent and Trademark Office (USPTO) Patent Full-Text and Image Database was performed over the years 1993 to 2018. Patents related to surgery were defined as surgical devices, implantables, dressings, introducers, and sterilization equipment based on Cooperative Patent Classification (CPC) code. Inventor names were cross-indexed with names of active Fellows in the American College of Surgeons (FACS) as of 2019. Surgeon inventors were identified and differences between specialty and sex were evaluated. RESULTS: A total of 275,260 patents related to surgery were issued over the study period. The number of surgical patents has increased by 462% from 4593 per year to 21,241 per year. A total of 9008 patents were held by a total of 2164 surgeons (4% of FACS). This represents 3.3% of all surgical patents with a mean of 5 patents (range 1-346) per patent-holding surgeon. Specialties with the largest number of patent holders include neurosurgery (9%) and orthopedic surgery (8%). Ninety-seven percent of patent-holding surgeons were male. CONCLUSIONS: 3.3% of patents related to surgery involve a surgeon inventor, and although the number of surgical patents has shown an exponential increase, surgeon involvement in these inventions has grown minimally. Surgical innovation training may offer an opportunity to reduce these discrepancies and increase surgeon involvement as patent holders.


Assuntos
Neurocirurgia , Cirurgiões , Masculino , Humanos , Estados Unidos , Feminino , Invenções , Procedimentos Neurocirúrgicos , Criatividade
19.
J Laparoendosc Adv Surg Tech A ; 31(12): 1431-1435, 2021 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-34677092

RESUMO

Purpose: Gastropexy during laparoscopic gastrostomy tube (GT) insertion can be technically challenging. T-fasteners are an effective method of gastropexy. However, the use of external bolsters requires an additional procedure for removal and may cause skin complications due to pressure necrosis. We describe our experience utilizing T-fasteners in a novel way that eliminates external bolsters during laparoscopic GT placement. Methods: Pediatric patients requiring enteral access who underwent gastrostomy at a single institution using the paired T-fastener technique were reviewed. Gastropexy was achieved by passing pairs of T-fasteners, under laparoscopic and/or endoscopic guidance, through single stab incisions into the stomach in a square configuration, allowing the suture from one T-fastener to be tied subcutaneously to its paired suture. This eliminates the need for external bolsters. Operative time and 30-day postoperative complications, including local wound infection, granulation tissue formation, bleeding, and tube replacement, are reported. Results: Thirty patients underwent gastrostomy placement using the paired T-fastener technique. Mean age was 9.2 years (standard deviation [SD] 6.9) and mean weight 29.9 kg (SD 21.0). Mean tube length was 2.2 cm (SD 0.71). Eight patients underwent an additional procedure at the time of gastrostomy. Mean operative time was 74.4 minutes (SD 39.7). Five patients developed a local wound infection requiring antibiotics. Five developed granulation tissue. Seven patients underwent tube replacement within 30 days for dislodgment or stem upsize. Conclusion: The paired T-fastener technique is a safe and efficient method for primary button gastrostomy placement. This method eliminates the need for additional trocars or external bolsters and may be helpful in patients with thick abdominal walls.


Assuntos
Gastropexia , Laparoscopia , Criança , Gastrostomia , Humanos , Instrumentos Cirúrgicos , Suturas
20.
J Surg Case Rep ; 2021(4): rjab101, 2021 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-33936586

RESUMO

Although there is consensus that thromboprophylaxis is necessary for major orthopedic surgeries such a joint replacement, there is no widespread consensus on the need for thromboprophylaxis for minor arthroscopic surgery. Here, we present a case of deep vein thrombosis (DVT) and pulmonary embolism (PE) after a common arthroscopic meniscectomy in a healthy 20-year-old female collegiate athlete. The patient had no risk factors except for prior use of combined oral contraceptive pills (COCPs). Twenty hours after an uncomplicated right knee meniscectomy, patient presented to ED with right calf pain and cramping, and DVT was confirmed using ultrasound. One week later, patient presented again to ED with dyspnea and chest pain. PE was diagnosed on CT angiography. Despite the rarity of thromboembolic complications in minor arthroscopy surgery, the broadened use of thromboprophylaxis in patients with even few risk factors could prevent thromboembolic complications from occurring.

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