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Item Advances in pediatric acute kidney injury pathobiology: a report from the 26th Acute Disease Quality Initiative (ADQI) conference(Springer, 2024) Starr, Michelle C.; Barreto, Erin; Charlton, Jennifer; Vega, Molly; Brophy, Patrick D.; Bignall, O. N. Ray, II; Sutherland, Scott M.; Menon, Shina; Devarajan, Prasad; Arikan, Ayse Akcan; Basu, Rajit; Goldstein, Stuart; Soranno, Danielle E.; ADQI 26 workgroup; Pediatrics, School of MedicineBackground: In the past decade, there have been substantial advances in our understanding of the pathobiology of pediatric acute kidney injury (AKI). In particular, animal models and studies focused on the relationship between kidney development, nephron number, and kidney health have identified a number of heterogeneous pathophysiologies underlying AKI. Despite this progress, gaps remain in our understanding of the pathobiology of pediatric AKI. Methods: During the 26th Acute Disease Quality Initiative (ADQI) Consensus conference, a multidisciplinary group of experts discussed the evidence and used a modified Delphi process to achieve consensus on recommendations for opportunities to advance translational research in pediatric AKI. The current state of research understanding as well as gaps and opportunities for advancement in research was discussed, and recommendations were summarized. Results: Consensus was reached that to improve translational pediatric AKI advancements, diverse teams spanning pre-clinical to epidemiological scientists must work in concert together and that results must be shared with the community we serve with patient involvement. Public and private research support and meaningful partnerships with adult research efforts are required. Particular focus is warranted to investigate the pediatric nuances of AKI, including the effect of development as a biological variable on AKI incidence, severity, and outcomes. Conclusions: Although AKI is common and associated with significant morbidity, the biologic basis of the disease spectrum throughout varying nephron developmental stages remains poorly understood. An incomplete understanding of factors contributing to kidney health, the diverse pathobiologies underlying AKI in children, and the historically siloed approach to research limit advances in the field. The recommendations outlined herein identify gaps and outline a strategic approach to advance the field of pediatric AKI via multidisciplinary translational research.Item Age of Transfused Red Blood Cells and Health Outcomes in Two Surgical Cohorts(Elsevier, 2019-03) Khan, Sikandar H.; Devnani, Rohit; LaPradd, Michelle; Landrigan, Matt; Gray, Alan; Kelley, Andrea; Eckert, George J.; Li, Xiaochun; Khan, Babar A.; Medicine, School of MedicineRationale: Red blood cells (RBC) undergo morphologic and biochemical changes during storage which may lead to adverse health risks upon transfusion. In prior studies, the effect of RBC age on health outcomes has been conflicting. We designed the study to assess the effects of RBC units' storage duration on health outcomes specifically for hospitalized patients undergoing hip fracture surgery or coronary artery bypass grafting (CABG) surgery. Methods: Using International Classification of Diseases (ICD) 9 codes, hip fracture surgery and CABG surgery patients, who received RBC transfusions between 2008 and 2013, were retrospectively identified from the electronic medical records system. Hip fracture surgery and CABG cohorts were sub-divided into 3 blood age groups based upon RBC unit age at the time of transfusion: young blood (RBC units stored less than or equal to 14 days), old blood (RBC units were stored for greater than or equal to 28 days), or mixed blood for the remaining patients. Outcome variables were 30-day, 90-day, and inpatient mortality as well as hospital length of stay. Results: A total of 3,182 patients were identified: 1,121 with hip fractures and 2,061 with CABG. Transfusion of old blood was associated with higher inpatient mortality in the hip fracture surgery cohort (OR 166.8, 95% CI 1.067-26064.7, p = 0.04) and a higher 30-day mortality in the CABG cohort (OR 4.55, 95% CI 1.01-20.49, p = 0.03). Conclusions: Transfusing RBC units stored for greater than or equal to 28 days may be associated with a higher mortality for patients undergoing hip fracture or CABG.Item Arthroscopic dorsal wrist ganglion excision with color-aided visualization of the stalk: minimum 1-year follow-up(Springer, Part of Springer Science+Business Media, 2014-06) Ahsan, Zahab S.; Yao, Jeffrey; Department of Orthopaedic Surgery, School of MedicineBackground Dorsal wrist ganglia (DWG) are a common, benign soft-tissue mass of the wrist. Excision of DWG is a common procedure performed by hand surgeons and may be performed using either open or arthroscopic techniques. This study aims to evaluate the frequency of stalk visualization with intralesional injection of inert dye in the course of arthroscopic excision along with incidence of recurrence with a minimum of 1-year follow-up. Methods Upon IRB approval, a retrospective chart review was performed identifying 27 patients who had consecutively undergone arthroscopic excision of a DWG with the color-aided technique at our institution with a minimum follow-up duration of 12 months. Intraoperative findings were reviewed. Patients were contacted to investigate for incidence of recurrence. Results Of the 27-patient cohort, the ganglion stalk was identified in 100 % of the color-aided arthroscopic DWG excisions. Ganglion recurrence was identified in one patient, an incidence of 3.7 %. Conclusions The color-aided technique for arthroscopic DWG visualization was found to be a safe and valuable tool for surgeons performing arthroscopic DWG resection. The intraarticular ganglion stalk was identified in 100 % of cases and patients responded well with a low incidence of recurrence.Item Assessment of morbidity and mortality after liver transplantation for primary sclerosing cholangitis(AME, 2024) Ekser, Burcin; Mihaylov, Plamen; Mangus, Richard S.; Surgery, School of MedicineItem Building Evidence for Clinical Use of Pharmacogenomics and Reimbursement for Testing(Elsevier, 2022) Cavallari, Larisa H.; Pratt, Victoria M.; Medical and Molecular Genetics, School of MedicineItem Characteristics and outcomes of patients with sepsis who had cortisol level measurements or received hydrocortisone during their intensive care unit management: A retrospective single center study(Sage, 2023-01-03) Rivas, Marcella; Motes, Arunee; Ismail, Amr; Yang, Shengping; Sotello, David; Arevalo, Meily; Vutthikraivit, Wasawat; Suchartlikitwong, Sakolwan; Carrasco, Cynthia; Iwuji, Kenneth; Pachariyanon, Pavida; Jaroudi, Sarah; Thavaraputta, Subhanudh; Nugent, Kenneth; Medicine, School of MedicineObjectives: The current guidelines for managing patients with sepsis include the early cultures, administration of antibiotics, and fluid resuscitation. Several clinical trials have tried to determine whether or not the administration of corticosteroids improves outcomes in these patients. This study analyzed the characteristics of a large group of critically ill patients who either had cortisol levels drawn during their intensive care unit management or had hydrocortisone administered during their management. Methods: A list of patients who had cortisol levels measured or who had hydrocortisone administered empirically for the treatment of sepsis was identified by the medical record department at University Medical Center in Lubbock, Texas. The primary outcome was in-hospital mortality. Secondary outcomes included the need for mechanical ventilation, the need for renal replacement therapy, the need for vasopressors, length of stay, and the development of nosocomial infections. Results: This study included 351 patients, including 194 women (55.3%). The mean age was 62.9 ± 16.1 years. The mean admission SOFA score was 9.3 ± 3.63, the mean APACHE 2 score was 18.15 ± 7.7, and the mean lactic acid level was 3.8 ± 4.0 mmol/L. One hundred sixty-two patients required intubation, 262 required vasopressors, 215 developed acute kidney injury, and 319 had cortisol levels measured. The mean length of stay was 11.5 ± 13.7 days; the mortality rate was 32.2%. Multiple variable analysis demonstrated that higher cortisol levels were associated with increased mortality (44.1% if cortisol ⩾20 µg/dL versus 17.5% if cortisol <20 µg/dL). One hundred forty-five patients received corticosteroids, and multivariable analysis demonstrated that these patients had increased mortality (40.0% versus 26.7%). Conclusion: In this study, higher cortisol levels were associated with increased mortality. The administration of hydrocortisone was associated with increased mortality possibly reflecting the use of this medication in patients who had a higher likelihood of poor outcomes.Item Characteristics of children ≤36 months of age with DIPG: A report from the international DIPG registry(Oxford University Press, 2022) Bartlett, Allison L.; Lane, Adam; Chaney, Brooklyn; Yanez Escorza, Nancy; Black, Katie; Cochrane, Anne; Minturn, Jane; Bartels, Ute; Warren, Kathy; Hansford, Jordan; Ziegler, David; Diez, Blanca; Goldman, Stewart; Packer, Roger; Kieran, Mark; DeWire-Schottmiller, Mariko; Erker, Craig; Monje-Deisseroth, Michelle; Wagner, Lars; Koschmann, Carl; Dorris, Kathleen; Shih, Chie-Schin; Hassall, Tim; Fisher, Paul; Wang, Stacie S.; Tsui, Karen; Sevlever, Gustavo; Zhu, Xiaoting; Dexheimer, Phillip; Asher, Anthony; Fuller, Christine; Drissi, Rachid; Jones, Blaise; Leach, James; Fouladi, Maryam; Pediatrics, School of MedicineBackground: Children ≤36 months with diffuse intrinsic pontine glioma (DIPG) have increased long-term survival (LTS, overall survival (OS) ≥24 months). Understanding distinguishing characteristics in this population is critical to improving outcomes. Methods: Patients ≤36 months at diagnosis enrolled on the International DIPG Registry (IDIPGR) with central imaging confirmation were included. Presentation, clinical course, imaging, pathology and molecular findings were analyzed. Results: Among 1183 patients in IDIPGR, 40 were eligible (median age: 29 months). Median OS was 15 months. Twelve patients (30%) were LTS, 3 (7.5%) very long-term survivors ≥5 years. Among 8 untreated patients, median OS was 2 months. Patients enrolled in the registry but excluded from our study by central radiology review or tissue diagnosis had median OS of 7 months. All but 1 LTS received radiation. Among 32 treated patients, 1-, 2-, 3-, and 5-year OS rates were 68.8%, 31.2%, 15.6% and 12.5%, respectively. LTS had longer duration of presenting symptoms (P = .018). No imaging features were predictive of outcome. Tissue and genomic data were available in 18 (45%) and 10 patients, respectively. Among 9 with known H3K27M status, 6 had a mutation. Conclusions: Children ≤36 months demonstrated significantly more LTS, with an improved median OS of 15 months; 92% of LTS received radiation. Median OS in untreated children was 2 months, compared to 17 months for treated children. LTS had longer duration of symptoms. Excluded patients demonstrated a lower OS, contradicting the hypothesis that children ≤36 months with DIPG show improved outcomes due to misdiagnosis.Item Characteristics of patients ≥10 years of age with diffuse intrinsic pontine glioma: a report from the International DIPG/DMG Registry(Oxford University Press, 2022) Erker, Craig; Lane, Adam; Chaney, Brooklyn; Leary, Sarah; Minturn, Jane E.; Bartels, Ute; Packer, Roger J.; Dorris, Kathleen; Gottardo, Nicholas G.; Warren, Katherine E.; Broniscer, Alberto; Kieran, Mark W.; Zhu, Xiaoting; White, Peter; Dexheimer, Phillip J.; Black, Katie; Asher, Anthony; DeWire, Mariko; Hansford, Jordan R.; Gururangan, Sridharan; Nazarian, Javad; Ziegler, David S.; Sandler, Eric; Bartlett, Allison; Goldman, Stewart; Shih, Chie-Schin; Hassall, Tim; Dholaria, Hetal; Bandopadhayay, Pratiti; Samson, Yvan; Monje, Michelle; Fisher, Paul G.; Dodgshun, Andrew; Parkin, Sarah; Chintagumpala, Murali; Tsui, Karen; Gass, David; Larouche, Valerie; Broxson, Emmett; Garcia Lombardi, Mercedes; Shiqi Wang, Stacie; Ma, Jie; Hawkins, Cynthia; Hamideh, Dima; Wagner, Lars; Koschmann, Carl; Fuller, Christine; Drissi, Rachid; Jones, Blaise V.; Leach, James; Fouladi, Maryam; Pediatrics, School of MedicineBackground: Diffuse intrinsic pontine gliomas (DIPG) generally occur in young school-age children, although can occur in adolescents and young adults. The purpose of this study was to describe clinical, radiological, pathologic, and molecular characteristics in patients ≥10 years of age with DIPG enrolled in the International DIPG Registry (IDIPGR). Methods: Patients ≥10 years of age at diagnosis enrolled in the IDIPGR with imaging confirmed DIPG diagnosis were included. The primary outcome was overall survival (OS) categorized as long-term survivors (LTS) (≥24 months) or short-term survivors (STS) (<24 months). Results: Among 1010 patients, 208 (21%) were ≥10 years of age at diagnosis; 152 were eligible with a median age of 12 years (range 10-26.8). Median OS was 13 (2-82) months. The 1-, 3-, and 5-year OS was 59.2%, 5.3%, and 3.3%, respectively. The 18/152 (11.8%) LTS were more likely to be older (P < .01) and present with longer symptom duration (P < .01). Biopsy and/or autopsy were performed in 50 (33%) patients; 77%, 61%, 33%, and 6% of patients tested had H3K27M (H3F3A or HIST1H3B), TP53, ATRX, and ACVR1 mutations/genome alterations, respectively. Two of 18 patients with IDH1 testing were IDH1-mutant and 1 was a LTS. The presence or absence of H3 alterations did not affect survival. Conclusion: Patients ≥10 years old with DIPG have a median survival of 13 months. LTS present with longer symptom duration and are likely to be older at presentation compared to STS. ATRX mutation rates were higher in this population than the general DIPG population.Item Clinical characteristics and outcomes of autoimmune pancreatitis based on serum immunoglobulin G4 levels: A single-center, retrospective cohort study(Baishideng, 2023) Jaber, Fouad; Elfert, Khaled; Alsakarneh, Saqr; Beran, Azizullah; Jaber, Mohammed; Gangwani, Manesh Kumar; Abboud, Yazan; Medicine, School of MedicineAutoimmune pancreatitis (AIP) is a complex, poorly understood disease gaining increasing attention. "Clinical Characteristics and Outcome of AIP Based on Serum IgG4 levels," investigated AIP with a focus on serum immunoglobulin (Ig) G4 levels. The 213 patients with AIP were classified according to serum IgG4 levels: Abnormal (elevated) and normal. Patients with higher IgG4 levels exhibited a more active immune system and increased relapse rates. Beyond IgG4, the IgA levels and age independently contributed to relapse risk, guiding risk assessment and tailored treatments for better outcomes. However, limitations persist, such as no IgA correlation with IgG4 levels, absent data on autoantibody-positive AIP cases critical for Asian diagnostic criteria, and unexplored relapse rates in high serum IgG AIP by subtype. Genetic factors and family histories were not addressed. As the understanding and referral of seronegative AIPs increase, there's a growing need for commercially available, highly sensitive, and specific autoantibodies to aid in diagnosing individuals with low or absent serum IgG4 levels.Item Clinical features and prognostic factors of intensive and non-intensive 1014 COVID-19 patients: an experience cohort from Alahsa, Saudi Arabia(BioMed Central, 2021-05-24) Alhumaid, Saad; Al Mutair, Abbas; Al Alawi, Zainab; Al Salman, Khulud; Al Dossary, Nourah; Omar, Ahmed; Alismail, Mossa; Al Ghazal, Ali M.; Jubarah, Mahdi Bu; Al Shaikh, Hanan; Al Mahdi, Maher M.; Alsabati, Sarah Y.; Philip, Dayas K.; Alyousef, Mohammed Y.; Al Brahim, Abdulsatar H.; Al Athan, Maitham S.; Alomran, Salamah A.; Ahmed, Hatim S.; Al-Shammari, Haifa; Elhazmi, Alyaa; Rabaan, Ali A.; Al-Tawfiq, Jaffar A.; Al-Omari, Awad; Medicine, School of MedicineCOVID-19 is a worldwide pandemic and has placed significant demand for acute and critical care services on hospitals in many countries.