Browsing by Author "Rahrig, April L."

Now showing 1 - 4 of 4
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    A Second Look: Retrospective Identification of Thrombotic Microangiopathy in Pediatric Stem Cell Transplant Patients With Veno‐Occlusive Disease
    (Wiley, 2025) Rahim, Mahvish Q.; Nichols, Cydney; Althouse, Sandra; Rahrig, April L.; Pediatrics, School of Medicine
    Background: Veno-occlusive disease (VOD) and transplant-associated thrombotic microangiopathy (TA-TMA) remain a diagnostic and therapeutic challenge for patients undergoing hematopoietic stem cell transplant (HSCT). Both VOD and TA-TMA share an underlying etiology of microvascular endothelial damage. Potential under-recognition of TA-TMA in the context of VOD leaves HSCT recipients vulnerable to additional endothelial damage, and risk of end-organ failure. Methods: A cohort of 44 pediatric HSCT recipients diagnosed with VOD between 2010and 2019 were retrospectively evaluated for the development of TA-TMA within 1 week before and 2 months after VOD diagnosis. Patients were classified into three categories: sole diagnosis of VOD (VOD), concurrent clinical diagnosis of TA-TMA during the VOD course (VOD+TA-TMA), and patients with VOD who on retrospective review satisfied criteria for diagnosis of TA-TMA (VOD+rTA-TMA). Results: A total of 42 patients were evaluated and 50% of the patients were diagnosed clinically with TA-TMA (5) or where retrospectively identified to have TA-TMA (16). There was no difference in the severity of the course of VOD between the three groups based on need for intubation, dialysis, and pediatric intensive care unit (PICU) care. Patients in the VOD only group had the highest survival at 1 year (66.7%, n = 14) compared with patients in the VOD+TA-TMA group (60%, n = 3) and VOD+rTA-TMA group (62.5%, n = 10), p = 0.9582. Conclusion: Better understanding of the association between these two endotheliopathies is essential to improve diagnosis, treatment, and prevention of potentially fatal adverse outcomes in transplant recipients.
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    Case report: Toxic epidermal necrolysis as a unique presentation of acute graft versus host disease in a pediatric patient
    (Frontiers Media, 2025-01-23) Marlowe, Elizabeth; Palmer, Rachel; Rahrig, April L.; Dinora, Devin; Harrison, Jessica; Skiles, Jodi; Rahim, Mahvish Q.; Pediatrics, School of Medicine
    Introduction: Acute graft versus host disease (aGVHD) is a common complication of stem cell transplant (SCT), with skin involvement being most common. Severe presentations of skin aGVHD involving rapid progression of rash to bullae formation and mucosal involvement are rare. There are reports of patients with skin aGVHD that present with clinical characteristics mimicking toxic epidermal necrolysis (TEN), suggesting a possible overlap between the two. Management and outcomes of pediatric patients with this overlapping, severe presentation have rarely been described. Case presentation: This report describes an 11-year-old boy with refractory T-cell acute lymphoblastic leukemia who received peripheral blood SCT from a matched unrelated donor. Day 26 post-SCT, he developed a maculopapular facial rash, which progressed to the development of vesicles coalescing into bullae involving his conjunctiva, face, oral mucosa, and genital mucosa. Initially, systemic steroid monotherapy was initiated, but with rapid rash progression and mucosal involvement, intravenous immunoglobulin (IVIg) 2 g/kg divided over 5 days was added as management for suspected TEN-like aGVHD based on clinical findings. Ruxolitinib was subsequently started as adjunctive management for aGVHD. His skin findings continued to improve with near total resolution by day 49 post-SCT. Conclusion: We report a unique case of TEN-like aGVHD with rapid progression to >30% body surface area involvement including bullae formation and detachment of epidermis. There have been few case reports of similar presentations, most with poor outcomes. We aim to supplement the literature available by reporting our successful management with steroids, IVIg, and ruxolitinib, which resulted in early resolution of symptoms in a pediatric patient.
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    Diagnostic Yield of Bronchoscopy in Children with Leukemia or Post Hematopoietic Stem Cell Transplant
    (Wiley, 2024) Georgescu, Livia; Rahrig, April L.; Montgomery, Gregory; Rowan, Courtney M.; Pediatrics, School of Medicine
    Background: The utility of bronchoscopy with bronchoalveolar lavage (BAL) in immunocompromised children is not well understood. We aim to describe the bronchoscopy diagnostic yield and complications and to investigate factors associated with diagnostic yield. Methods: This is a single-center, retrospective cohort study of 60 children with leukemia or post-hematopoietic stem cell transplant who had a bronchoscopy with BAL between 2017 and 2021. Comparisons were done with regression analysis. Results: Of the 60 bronchoscopies performed, 46 (77%) revealed diagnostic information: 39 (65%) identified a pathogen, 14 (23.3%) found secretions/mucus plugging, and 6 (10%) found pulmonary hemorrhage. BAL results changed antimicrobial therapy in 27 (45%) cases. Bronchoscopies were performed in the intensive care unit (27/60) or operating room (33/60), with the former having a higher diagnostic yield (96% vs. 60%, p = 0.001). Half (50%) of bronchoscopies found a new infectious diagnosis. Respiratory symptoms (n = 58, 97%), supplemental oxygen use (n = 39, 65%), and antibiotic use (n = 56, 93%) before bronchoscopy were all common. The median volume of fluid instilled during bronchoscopy was 1.3 mL/kg (interquatile range [IQR]: 0.7, 2.6). None of these factors were associated with the diagnostic yield. Complications were rare and minor with only one child having self-resolved bleeding and four children, previously in room air requiring a nasal cannula. For the 27 (45%) children on mechanical ventilation when the bronchoscopy was performed, there was no difference in ventilator settings pre- and post-bronchoscopy. Conclusion: Bronchoscopies with BAL are useful, safe, and important in the diagnostic management of pulmonary complications in this cohort of children.
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    Multiple Organ Dysfunction and Critically Ill Children with Acute Myeloid Leukemia: single-center retrospective cohort study
    (Wolters Kluwer, 2023) Gaugler, Mary; Swinger, Nathan; Rahrig, April L.; Skiles, Jodi; Rowan, Courtney M.; Pediatrics, School of Medicine
    Objectives: To describe the prevalence of multiple organ dysfunction syndrome (MODS) and critical care utilization in children and young adults with acute myeloid leukemia (AML) who have not undergone hematopoietic cell transplantation (HCT). Design: Retrospective cohort study of MODS (defined as dysfunction of two or more organ systems) occurring any day within the first 72 hours of PICU admission. Setting: Large, quaternary-care children's hospital. Patients: Patients 1 month through 26 years old who were treated for AML from 2011-2019. Interventions: None. Measurements and main results: Eighty patients with AML were included. These 80 patients had a total of 409 total non-HCT-related hospital and 71 PICU admissions. The majority 53 of 71 of PICU admissions (75%) were associated with MODS within the first 72 hours. MODS was present in 49 of 71 of PICU admissions (69%) on day 1, 29 of 52 (56%) on day 2, and 25 of 32 (78%) on day 3. The organ systems most often involved were hematologic, respiratory, and cardiovascular. There was an increasing proportion of renal failure (8/71 [11%] on day 1 to 8/32 [25%] on day 3; p = 0.02) and respiratory failure (33/71 [47%] to 24/32 [75%]; p = 0.001) as PICU stay progressed. The presence of MODS on day 1 was associated with a longer PICU length of stay (LOS) (β = 5.4 [95% CI, 0.7-10.2]; p = 0.024) and over a six-fold increased risk of an LOS over 2 days (odds ratio, 6.08 [95% CI, 1.59-23.23]; p = 0.008). Respiratory failure on admission was associated with higher risk of increased LOS. Conclusions: AML patients frequently require intensive care. In this cohort, MODS occurred in over half of PICU admissions and was associated with longer PICU LOS. Respiratory failure was associated with the development of MODS and progressive MODS, as well as prolonged LOS.