Browsing by Author "Rahrig, April"

Now showing 1 - 5 of 5
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    A familial SAMD9 variant present in pediatric myelodysplastic syndrome
    (Cold Spring Harbor Laboratory, 2023-05-09) Rahim, Mahvish Q.; Rahrig, April; Overholt, Kathleen; Conboy, Erin; Czader, Magdalena; Saraf, Amanda June; Pediatrics, School of Medicine
    Myelodysplastic syndrome (MDS) is a rare pediatric diagnosis characterized by ineffective hematopoiesis with potential to evolve into acute myelogenous leukemia (AML). In this report, we describe a unique case of a 17-yr-old female with an aggressive course of MDS with excess blasts who was found to have monosomy 7 and a SAMD9 germline variant, which has not previously been associated with a MDS phenotype. This case of MDS was extremely rapidly progressing, showing resistance to chemotherapy and stem cell transplant, unfortunately resulting in patient death. It is imperative to further investigate this rare variant to aid in the future care of patients with this variant.
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    Factors associated with a prolonged hospital stay during induction chemotherapy in newly diagnosed high risk pediatric acute lymphoblastic leukemia
    (Elsevier, 2018-08) Warrick, Kasper; Althouse, Sandra K.; Rahrig, April; Rupenthal, Joy; Batra, Sandeep; Pediatrics, School of Medicine
    Background High Risk (HR) or Very High Risk (VHR) acute lymphoblastic leukemia (ALL) treated with 4 drug induction chemotherapy is often associated with adverse events. The aim of this study was to identify risk factors associated with a prolonged inpatient length of stay LOS during induction chemotherapy. Procedure Data from patients (N = 73) (age<21 years) was collected through a retrospective chart review. Univariable and multivariable logistic regression was used to test for statistical significance. The overall survival and disease (leukemia)-free survival were analyzed using the Kaplan–Meier method and log-rank test. Results Of the 73 patients, 42 (57%) patients were discharged on day 4 of induction (short LOS, group A), while 31 (43%) patients (group B) experienced a prolonged LOS or an ICU stay (16 ± 27.7 days, median hospital stay = 8 days vs 4 days (group A), p = 0.02) due to organ dysfunction, infectious or metabolic complications. Group B patients were more likely to have a lower platelet count, serum bicarbonate, and a higher blood urea nitrogen (BUN) on day 4 of treatment (OR = 4.52, 8.21, and 3.02, respectively, p < 0.05). Multivariable analysis identified low serum bicarbonate (p = 0.002) and a platelet count<20,000/μL (p = 0.02) on day 4 of induction to be predictive of a prolonged LOS. Twenty six (group A (n = 16, 36%) and B (n = 11, 35%), p = 0.8) patients experienced unplanned admissions, within 30 days of discharge. Conclusions A significant proportion of newly diagnosed HR or VHR pediatric ALL patients experience a prolonged LOS and unplanned re-admissions. Aggressive discharge planning and close follow up is indicated in this cohort of patients.
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    Hypogammaglobulinemia in Adolescents and Young Adults with Acute Lymphoblastic Leukemia
    (Mary Ann Liebert, Inc., 2020-12) Lange, Cassandra S.; Rahrig, April; Althouse, Sandra K.; Nelson, Robert P.; Batra, Sandeep; Medicine, School of Medicine
    Hypogammaglobulinemia is a poorly described complication of chemotherapy in adolescents and young adults (AYAs, 15–39 years) with acute lymphoblastic leukemia (ALL). The majority of AYAs treated on a Berlin–Frankfurt–Munster-based ALL regimen experienced hypogammaglobulinemia (65.0% [13/20]). Febrile neutropenia episodes (throughout the treatment course) and infectious events during maintenance occurred more frequently in hypogammaglobulinemic patients compared with patients with normal immunoglobulin G levels (n = 7) (median 1.0 vs. 0.0, p = 0.02; 7.0 vs. 3.0, p = 0.02, respectively). Hypogammaglobulinemia did not impact overall or event-free survival. Further studies are needed to elucidate the etiology of hypogammaglobulinemia and to establish criteria for immunoglobulin replacement in these patients.
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    Interventions Performed in Children With Immunocompromised Conditions in the Pediatric Intensive Care Unit Within 48 Hours of Death
    (Mary Ann Liebert, 2024) Kegel, Anna; Espinoza, Jason; Rahrig, April; Schade-Wills, Tina; Rowan, Courtney M.; Pediatrics, School of Medicine
    Background: Understanding interventions preceding death in children with immunocompromised conditions is important to ensure a peaceful and dignified perideath experience. The aim of this study was to describe the number of interventions performed in the pediatric intensive care unit (PICU) within the 48 hours before death in this population. Methods: This was a single-center, retrospective cohort study of all children with an underlying oncologic, hematologic, or immunologic diagnosis admitted to the PICU for at least 72 hours between 2014 and 2021. Medical records were reviewed for interventions within 48 hours preceding death and for palliative care involvement. Interventions were defined as new or escalations in respiratory support, cardiopulmonary resuscitation (CPR), vascular access, drains, and radiographic studies. Associations were evaluated using simple logistic regression. Results: A total of 55 patients were included in this study. The predominant PICU admission diagnoses were respiratory (51%), followed by shock (25%), and neurologic diagnoses (9%). These predominant diagnoses were similar perideath. At PICU admission, only 1 patient had a do-not-resuscitate (DNR) order. Forty-six percent had a DNR order 48 hours preceding death, and 91% had DNR orders in place at time of death. During the 48-hour period preceding death, 80% of children received at least one intervention. Radiographic studies were the most common, used in 78% of children, followed by respiratory (20%), vascular (16%), CPR (13%), and drain placement (7%). Palliative care was involved in 38% of cases and was associated with a decrease in the number of radiologic interventions (p = 0.028) and CPR (p = 0.026). Conclusions: Children in the PICU with underlying immunocompromised conditions frequently receive interventions within the 48-hour period preceding death. Palliative care involvement was associated with fewer radiographic studies and fewer occurrences of CPR. The impact of interventions on the dying experience warrants further investigation.
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    Leukapheresis in Pediatric Acute Leukemia with Hyperleukocytosis: A Single-Center Experience
    (MDPI, 2022-04-02) Jones, Sandra Renee; Rahrig, April; Saraf, Amanda J.; Pediatrics, School of Medicine
    Hyperleukocytosis in pediatric acute leukemia is associated with increased morbidity and mortality and at present there is no consensus on the use of leukapheresis (LPH) for its management. Our aim was to review characteristics and outcomes of newly diagnosed leukemia patients with hyperleukocytosis (HL) comparing those who received LPH and those who did not. An IRB approved retrospective case control study reviewed data from a single institution over a 10 year period. At our institution, LPH was used in 8 of 62 (13%) patients with hyperleukocytosis with minimal complications. Mean leukocyte count in patients who received LPH versus those who did not was 498 k cells/mm3 and 237 k cells/mm3, respectively. Patients who had symptoms of neurologic (63 vs. 17%) or pulmonary leukostasis (75 vs. 17%) were more likely to have undergone leukapheresis. The time from presentation to the initiation of chemotherapy was not different between those who received LPH and those who did not (mean of 35 h vs. 34 h). There was one death in the LPH group, that was the result of neurologic sequelae of hyperleukocytosis and not LPH itself. The use of LPH in patients with hyperleukocytosis is safe, well tolerated and does not alter time to chemotherapy at our institution.