scholarly journals Does Autoimmunity in Family Members of Children with Immune Thrombocytopenia Help Identify Patients at Higher Risk of Chronic Immune Thrombocytopenia?

Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 3168-3168
Author(s):  
Michell Lozano Chinga ◽  
Mark Fluchel ◽  
Jessica Meznarich

Abstract Background Immune thrombocytopenia (ITP) is the most common cause of thrombocytopenia during childhood. Approximately 10-30% of pediatric patients will develop chronic ITP (cITP), which is defined as thrombocytopenia lasting over 12 months, and constitutes a significant burden for patients and their caregivers. Patients with cITP may require ongoing medications to treat symptomatic thrombocytopenia, may have asymptomatic thrombocytopenia not requiring medical interventions, or may experience complete resolution of their ITP. There are not specific patient nor disease characteristics that can help us predict how cITP may progress, and which patients are more likely to require ongoing treatments. ITP can be a manifestation of immune dysregulation in patients with other autoimmune conditions or primary immunodeficiency disorders (PIDDs). We aimed to assess the characteristics of patients with cITP including the presence of autoimmune or allergic disorders in the patients and in first-, second-, and third-degree family members. We hypothesized that patients with cITP may have a higher incidence of immune dysregulation in family members in contrast to patients with acute aITP. Methods The study was approved by the institutional review and ethics boards at the University of Utah. We queried the Primary Children's Hospital database for cases of "immune thrombocytopenia" from January 1 st, 2001 to January 1 st,2021. Retrospective chart review was done to confirm the diagnosis. Patient demographics, clinical presentation, and family history of patients were reviewed. Data was collected in RedCap at the University of Utah. Descriptive summaries of data were done. Results Medical charts from 266 ITP patients diagnosed during the study period were reviewed; 182/266 (68.5%) had acute ITP (aITP) and 84/266 (31.5%) patients had cITP, defined as platelet count <150 K/µl for >12 months. Resolution of ITP occurred in 28/84 (33.3%) patients with cITP (resolved cITP), while 56/84 (66.7%) had ongoing thrombocytopenia (unresolved cITP). Mean duration of ITP in patients with resolved cITP was 2.9 years, and 4.6 years in patients with unresolved cITP at the time of the last known platelet count. Mean age at diagnosis was 7.4 years in the cITP group and 5.1 years in the aITP group. Concurrent allergic conditions were identified in 10/84 (12%) of patients with cITP and 5/182 (2.7%) of patients with aITP. Autoimmune conditions were identified in 3 patients (3.5%) with cITP, and 4 patients (2.2%) with aITP. First-degree family members of cITP patients were more likely to be reported with an autoimmune condition than first-degree family members of aITP patients (15.5% vs. 5.5%, p=0.007 using Chi-square test); this effect was not seen amongst second- or third-degree relatives. The most common autoimmune condition reported in family members was autoimmune thyroid disease in both cohorts (2.7% in aITP and 9.5% in cITP). Common variable immunodeficiency (CVID) was reported in second degree relatives of 3/84 (3.6%) patients with cITP; no relatives of patients with aITP had a report of PIDD. Additionally, we identified 14 patients with Evans syndrome (ES), all with chronic immune thrombocytopenia and all patients had been followed for over a year at the time of the chart review. Four ES patients were previously diagnosed with 22q11.2 deletion, and one with CVID. In patients with ES, 4/14 (28.6%) and 5/14 (35.7%) had first- and second-degree family members with a reported autoimmune condition, respectively. No PIDDs were identified in first, second-, or third-degree relatives of patients with ES. Conclusions There is increasing evidence that patients with chronic ITP may exhibit polyautoimmunity or other signs of immune dysregulation, suggesting that ITP may be the initial manifestation of another autoimmune process or PIDD. We evaluated medical histories of patients with ITP and their family members. Patients with cITP have a history of autoimmunity in their family stronger than in patients with aITP. This association was even stronger in patients with ES. Disclosures No relevant conflicts of interest to declare.

1982 ◽  
Vol 141 (5) ◽  
pp. 453-458 ◽  
Author(s):  
Bruce Pfohl ◽  
Ned Vasquez ◽  
Henry Nasrallan

SummaryPrevious studies attempting to support unipolar mania as an entity distinct from bipolar disorder, have produced conflicting results. The present study reports on a chart review of 247 patients admitted to the University of Iowa with a history of at least one manic episode; 87 of these had apparently never experienced a depression. A subgroup of 92 patients, who met DSM III diagnostic criteria and had a history of at least two episodes of affective disorder, were also examined. There were few clinically meaningful differences between patients with unipolar mania and bipolar disorder on demographic, symptomatic, or familial variables. An earlier report that unipolar manics were more likely to be male and have a family history of unipolar depression was not confirmed. Unipolar mania is not supported as a separate entity from bipolar disorder.


2021 ◽  
Vol 8 (Supplement_1) ◽  
pp. S672-S672
Author(s):  
Isabella McNamara ◽  
Nicole L Pershing ◽  
Jacob Wilkes ◽  
Anne Bonkowsky

Abstract Background Intracranial extension of sinusitis is a rare complication. Non-specific presentations are a diagnostic challenge, and complications include long-term neurologic sequelae. Early recognition is critical, although optimal management remains poorly characterized. Methods We conducted a retrospective chart review of 123 patients admitted to Primary Children’s Hospital between 2004-2019 with ICD9 and ICD10 codes for sinusitis and intracranial suppurative infection. Chart review confirmed cases in patients < 18 years with evidence of sinusitis and intracranial extension. Variables collected included: demographic data, clinical presentation, microbial profile, clinical management, and outcomes. Results We observed 84 pediatric sinogenic intracranial infections between 2004-2019. Incidence significantly increased over the interval. Median patient age was 12.8 years (IQR 10.5-14.7 years); most were male (n=52). The most common presenting symptoms were headache and fever. Most patients (n=80) required surgical management; 62 required neurosurgery. All but one patient survived. Most infections were polymicrobial (n=47). Streptococcus anginous group were most frequently identified (n=32). All patients were started on vancomycin empirically; only 10 required use for definitive management. Most patients were treated with a carbapenem (n=42) or ceftriaxone plus metronidazole (n=32). Average duration of antibiotics was 44 days (IQR 38-55 days). The most common complication was epilepsy (n=11). Patients with Streptococcus sp. infections were more likely to experience adverse outcomes (p= 0.04). We observed significantly decreased carbapenem use following introduction of an antimicrobial stewardship program in 2012, with no change in clinical outcomes. Annual incidence of Sinogenic Intracranial Infections Shown is the increasing incidence of sinogenic intracranial infections annually in the state of Utah/100,000 children. Conclusion Sinogenic intracranial infections are increasingly frequent severe pediatric infections with associated long term neurologic sequelae. Most patients require both surgical and long-term IV antibiotic treatment. Most infections are polymicrobial. Streptococcus sp. are commonly identified. Antibiotic resistant bacteria are rare. The combination ceftriaxone and metronidazole is appropriate for treatment of most infections. Disclosures Anne Bonkowsky, MD/PhD, BioFire Diagnostics (Consultant, Grant/Research Support, Other Financial or Material Support, I have intellectual property through the University of Utah in BioFire Diagnostics and the FilmArray and receive royalties through the University of Utah.)Merck (Advisor or Review Panel member)


Author(s):  
George Frison ◽  
Jane Beiswenger

The purpose of this project was to interpret the vegetational history of the Yellowstone Park area by the analysis of pollen contained in sediment cores from four locations in Yellowstone Lake. The cores, collected by Dr. Robert Smith of the University of Utah, were from the north central portion, the west thumb area, the south arm and the southeast arm of the lake. The cores were to be compared to determine pollen variations within the lake. Differences between a small pond and a large lake basin in reflecting climatic change were to be studied by comparing the results with pollen counts from Fifteen Foot Lagoon (Baker, 1976).


2004 ◽  
Vol 33 (2) ◽  
Author(s):  
Kenning Arlitsch ◽  
John Herbert

The Marriott Library at the University of Utah (U of U) has a long history of large-scale newspaper projects beginning with the National Endowment for the Humanities' United States Newspapers Program (USNP) in the 1980s, in which the Library led the effort to catalog and microfilm Utah newspapers. This involvement continues today with the Utah Digital Newspaper (UDN) program, which is digitizing historic Utah newspapers, making them searchable and available on the Internet.


Neurosurgery ◽  
2006 ◽  
Vol 58 (5) ◽  
pp. 874-880 ◽  
Author(s):  
Ganesh Rao ◽  
Paul Klimo ◽  
Randy L. Jensen ◽  
Joel D. MacDonald ◽  
William T. Couldwell

Abstract OBJECTIVE: Recurrent cranial base meningiomas are among the most difficult tumors to treat surgically. Although they are histologically benign, these tumors often invade through the cranial base into the infratemporal and pterygopalatine fossae. We reviewed our experience with these tumors to describe the natural history of these lesions as well as provide a possible treatment paradigm. METHODS: Between 2000 and 2004, seven patients with meningiomas recurring through the cranial base into facial structures were treated at the University of Utah. Five patients were treated with transcranial approaches only, and two were treated with a combination of transcranial and transfacial approaches. RESULTS: The average age of our patients (6 women, 1 man) was 55 years. The original site of tumor was the sphenoid wing in four patients, the middle fossa in two patients, and the left frontal region in one patient. The average interval between the most recent tumor resection and recurrence into the face was 9.9 years. The mean number of resections a patient underwent before invasion into the face was two. All but one patient had adjunctive therapy (including either radiation or chemotherapy) before recurrence into the face. CONCLUSION: Meningiomas that recur into facial structures present a unique treatment challenge. These lesions have a high rate of recurrence once they have invaded through the cranial base. Although combined approaches may be necessary to achieve a gross total resection, these lesions can often be reached using standard transcranial techniques.


2019 ◽  
Vol 3 (Supplement_1) ◽  
pp. S614-S614
Author(s):  
Jacqueline Eaton

Abstract The Gerontology Interdisciplinary Program at the University of Utah has a long history of offering certificates (48 years) and a Master of Science degree (25 years). Relatively new to our program is the minor in gerontology, first available in 2013. This paper will describe efforts to grow this program, barriers to enrollment, and plans for expansion. In 2018, the program developed a targeted strategy to increase student awareness of and enrollment in the minor. First, we focused on newly enrolled pre-nursing students through new student orientation presentations and registration support. The introductory course within the minor jumped in enrollment from 6 students in Fall of 2017 to 40 in Fall 2018. Students taking courses in the minor received follow-up support and instructions mid-semester to ease matriculation. Enrollment in the minor increased by 140%. Next, we plan to apply these strategies to a broader number of colleges and departments throughout campus.


2019 ◽  
Author(s):  
Allyson Mower

Descriptive bibliography that shows the history of research and writing at an institution of higher learning.


Author(s):  
Aishah Ekhzaimy ◽  
Afshan Masood ◽  
Seham Alzahrani ◽  
Waleed Al-Ghamdi ◽  
Daad Alotaibi ◽  
...  

Summary Central diabetes insipidus (CDI) and several endocrine disorders previously classified as idiopathic are now considered to be of an autoimmune etiology. Dermatomyositis (DM), a rare autoimmune condition characterized by inflammatory myopathy and skin rashes, is also known to affect the gastrointestinal, pulmonary, and rarely the cardiac systems and the joints. The association of CDI and DM is extremely rare. After an extensive literature search and to the best of our knowledge this is the first reported case in literature, we report the case of a 36-year-old male with a history of CDI, who presented to the hospital’s endocrine outpatient clinic for evaluation of a 3-week history of progressive facial rash accompanied by weakness and aching of the muscles. Learning points: Accurate biochemical diagnosis should always be followed by etiological investigation. This clinical entity usually constitutes a therapeutic challenge, often requiring a multidisciplinary approach for optimal outcome. Dermatomyositis is an important differential diagnosis in patients presenting with proximal muscle weakness. Associated autoimmune conditions should be considered while evaluating patients with dermatomyositis. Dermatomyositis can relapse at any stage, even following a very long period of remission. Maintenance immunosuppressive therapy should be carefully considered in these patients.


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