scholarly journals Retrospective Analysis of a Longitudinal Single Institution 219 Common Variable Immunodeficiency Patient Cohort

2021 ◽  
Author(s):  
Melissa D Gans ◽  
Brian Goodman ◽  
Larry Bernstein ◽  
Rachel Eisenberg ◽  
Arye Rubinstein
Keyword(s):  
Common Variable Immunodeficiency ◽  
Chart Review ◽  
Retrospective Chart Review ◽  
Lymphoid Hyperplasia ◽  
The Novel ◽  
Limited Data ◽  
Single Institution ◽  
Cvid Patient ◽  
Patient Cohort ◽  
Common Variable

Abstract Common variable immunodeficiency is a heterogenous disorder of the immune system associated with immunodeficiency, lymphoproliferation, autoimmunity, and malignancy. Certain laboratory characteristics of common variable immunodeficiency patients can be associated with an increase of related clinical sequelae, though there is limited data on predictive characteristics for clinical sequelae in CVID patients. The purpose of this study was to analyze a unique large CVID patient cohort for predictive laboratory characteristics of clinical sequelae. A retrospective chart review was performed of a longitudinal cohort of CVID patients treated at a single institution, largely by a single provider with higher dose replacement immunoglobulin, that has not been previously described. 219 CVID patients were followed for 1,990 patient-years. 86% of the patients were on immunoglobulin with an average IgG trough of 1260 mg/dL. Low IgG at time of diagnosis, low CD19 absolute cell count, and poor mitogen induced lymphocyte proliferation were associated with increase of CVID clinical sequelae. In particular, this cohort demonstrates the novel finding that low IgG at time of diagnosis prior to immunoglobulin replacement is associated with a higher incidence of lymphoma, bronchiectasis, granulomatous disease, lymphoid hyperplasia, splenomegaly, and hepatic disease.

scholarly journals Characterizing Posterior Neck Masses: A Single-Institution Retrospective and Systematic Review

2019 ◽  
pp. 014556131988184
Author(s):  
William J. Moss ◽  
Andrey Finegersh ◽  
Ajay Narayanan ◽  
Danielle Gillard ◽  
Joseph Califano ◽  
...  
Keyword(s):  
Systematic Review ◽  
Chart Review ◽  
Case Reports ◽  
Retrospective Chart Review ◽  
Neck Mass ◽  
Single Institution ◽  
Neck Masses ◽  
Risk Of Malignancy ◽  
Posterior Neck ◽  
The Masses

Objective: Posterior neck masses are a relatively poorly characterized entity. The authors attempt to further characterize the anatomy and pathology of the posterior neck by way of a combined single-institution retrospective chart review and systematic review of the literature. Methods: A single-institution retrospective chart review was undertaken for all patients undergoing excision of a posterior neck mass between January 1, 2012, and January 1, 2017. A systematic review of the Medline, Embase, Web of Science, and Cochrane database was undertaken in accordance with Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines in search of case reports and series describing posterior neck masses. Results: A total of 28 patients who underwent excision of a posterior neck mass were encountered during the retrospective chart review. All pathologies were benign, the most prevalent of which was lipoma (22/28, 79%). A total of 19 articles describing a collective 36 posterior neck masses were encountered during the systematic review. Lipomas were the most common pathology (15/36, 42%). All but one of the masses reported were benign (35/36, 97%). Conclusions: Patients presenting with posterior neck masses can be reassured of a low risk of malignancy. The majority of posterior neck masses can be appropriately evaluated via physical examination and ultrasound.

Safety and Efficacy of Brivaracetam in Pediatric Refractory Epilepsy: A Single-Center Clinical Experience

2019 ◽  
Vol 35 (2) ◽  
pp. 102-105 ◽  
Author(s):  
Sara McGuire ◽  
Gustavo Silva ◽  
Darshan Lal ◽  
Divya S. Khurana ◽  
Agustin Legido ◽  
...  
Keyword(s):  
Seizure Frequency ◽  
Chart Review ◽  
Refractory Epilepsy ◽  
Focal Epilepsy ◽  
Retrospective Chart Review ◽  
Average Dose ◽  
Limited Data ◽  
Duration Of Treatment ◽  
Seizure Reduction ◽  
The Mean

Brivaracetam is a new antiepileptic drug with limited data in children. The objective of this study was to assess the efficacy/tolerability of brivaracetam. This is a retrospective chart review of children/adolescents with refractory epilepsy treated with brivaracetam from 2016 to 2018. The primary outcome was seizure reduction (decrease in seizure frequency >50%). Twenty-three patients were identified. Mean age at initiation was 12.5 years. Fourteen were females. Epilepsy was focal in 11, generalized in 6, and mixed in 3. Average dose was 3.9 mg/kg/d. The mean duration of treatment was 8.2 months. Eight had greater than 50% decrease in seizure frequency, of which 7 had focal epilepsy, and 1 had Lennox-Gastaut/mixed epilepsy. Two had drowsiness and 3 behavioral complaints. One experienced tingling and dizziness. Our retrospective review suggests that brivaracetam is an effective therapy for refractory focal epilepsy in children older than 4 years of age.

Intestinal nodular lymphoid hyperplasia in patients with common variable immunodeficiency: Local accumulation of B and CD8(+) lymphocytes

1988 ◽  
Vol 8 (4) ◽  
pp. 296-306 ◽  
Author(s):  
Paul van den Brande ◽  
Karel Geboes ◽  
Gaston Vantrappen ◽  
André van den Eeckhout ◽  
Simonne Vertessen ◽  
...  
Keyword(s):  
Common Variable Immunodeficiency ◽  
Lymphoid Hyperplasia ◽  
Nodular Lymphoid Hyperplasia ◽  
Local Accumulation ◽  
Common Variable

scholarly journals Fatal liver mass rupture in a common-variable-immunodeficiency patient with probable nodular regenerating hyperplasia

2022 ◽  
Vol 18 (1) ◽  
Author(s):  
Mongkhon Sompornrattanaphan ◽  
Ranista Tongdee ◽  
Chamard Wongsa ◽  
Anupop Jitmuang ◽  
Torpong Thongngarm
Keyword(s):  
Portal Hypertension ◽  
Common Variable Immunodeficiency ◽  
Multiple Organ ◽  
Late Complications ◽  
Liver Mass ◽  
Cvid Patient ◽  
Hepatic Rupture ◽  
Fatal Liver ◽  
First Case ◽  
Common Variable

Abstract Background Nodular regenerating hyperplasia (NRH) is the most common liver involvement in common variable immunodeficiency (CVID). Most patients are asymptomatic with gradually increasing alkaline phosphatase (ALP) and mildly elevated transaminase enzymes over the years. We report the first case of fatal liver mass rupture in a CVID patient with probable NRH. Case presentation A 24-year-old man was diagnosed with CVID at the age of 1.25 years. Genetic testing revealed a transmembrane activator and calcium-modulator and cyclophilin-ligand interactor (TACI) mutation. He had been receiving intravenous immunoglobulin (IVIg) replacement therapy ever since then. The trough level of serum IgG ranged between 750–1200 mg/dL. However, he still had occasional episodes of lower respiratory tract infection until bronchiectasis developed. At 22 years old, computed tomography (CT) chest and abdomen as an investigation for lung infection revealed incidental findings of numerous nodular arterial-enhancing lesions in the liver and mild splenomegaly suggestive of NRH with portal hypertension. Seven months later, he developed sudden hypotension and tense bloody ascites. Emergency CT angiography of the abdomen showed NRH with intrahepatic hemorrhage and hemoperitoneum. Despite successful gel foam embolization, the patient died from prolonged shock and multiple organ failure. Conclusions Although CVID patients with NRH are generally asymptomatic, late complications including portal hypertension, hepatic failure, and hepatic rupture could occur. Therefore, an evaluation of liver function should be included in the regular follow-up of CVID patients.

Characterizing dupilumab facial redness in children and adolescents: A single-institution retrospective chart review

2020 ◽  
Vol 83 (5) ◽  
pp. 1520-1521 ◽  
Author(s):  
Sonal Muzumdar ◽  
Micaella Zubkov ◽  
Reid Waldman ◽  
Madeline E. DeWane ◽  
Rong Wu ◽  
...  
Keyword(s):  
Children And Adolescents ◽  
Chart Review ◽  
Retrospective Chart Review ◽  
Single Institution

scholarly journals Similar Long-Term Outcomes in Children Presenting With Abscess vs Phlegmon at Diagnosis of Crohn Disease

2020 ◽  
Vol 2 (3) ◽  
Author(s):  
Andrew W Fondell ◽  
Maua H Mosha ◽  
Ross M Maltz ◽  
Brendan M Boyle ◽  
Barbara Joanna Niklinska-Schirtz ◽  
...  
Keyword(s):  
Crohn Disease ◽  
Chart Review ◽  
Pediatric Patients ◽  
Retrospective Chart Review ◽  
Limited Data ◽  
Long Term Outcomes ◽  
Bowel Diseases ◽  
Inflammatory Bowel ◽  
Disease Present

Abstract Background Limited data are available for long-term outcomes of pediatric patients with abdominal abscess or phlegmon at diagnosis of Crohn disease. Methods We performed a retrospective chart review of such children over a recent 6-year period at 5 pediatric inflammatory bowel diseases. Results Fifty-two patients (mean age 15.9 ± 1.8 years) were reviewed. Thirty-six had an abscess and 27 (75%) required resectional therapy compared to 16 with phlegmon which 10 (63%) requiring surgery. Overall (37/52) 71% had surgery which was performed within 6 months in 32 (86%). Conclusions A similar high surgical rate exists whether pediatric patients with Crohn disease present with abscess or phlegmon.

scholarly journals Laparoscopy, dorsal lumbotomy and flank incision live donor nephrectomy: comparison of donor outcomes

2013 ◽  
Vol 7 (1-2) ◽  
pp. 69 ◽  
Author(s):  
Dinesh Samarasekera ◽  
David S.H. Kim ◽  
Rachel Wang ◽  
Gordon Yip ◽  
Steven S. Tang ◽  
...  
Keyword(s):  
Chart Review ◽  
Retrospective Chart Review ◽  
Donor Nephrectomy ◽  
Single Institution ◽  
Laparoscopic Donor Nephrectomy ◽  
Pain Experience ◽  
Live Donor Nephrectomy ◽  
Estimated Blood Loss ◽  
Lower Pain ◽  
Post Operation

Background: Flank incision (FL), dorsal lumbotomy (DL) and laparoscopic surgery have been effective approaches to donor nephrectomy. While laparoscopic donor nephrectomy (LDN) has becomeincreasingly popular, there has yet to be a direct comparison of the three modalities.Methods: We performed a retrospective chart review of FL, DL and LDN operations between 2002 and 2010 within a single institution. Donor and recipient characteristics, as well as surgical outcomes, were assessed.Results: There were 496 donor nephrectomy operations available for analyses. Patients in the LDN group had the lowest estimated blood loss, compared to the DL and FL groups (p < 0.001), lowest rate of complications (p < 0.01), and shortest hospital stay (p < 0.0001). Donors who underwent DL used an average of 60.12 ± 5.0 mg of morphine, which was significantly less thanthat used by patients in the LDN (93.2 mg, p < 0.0001) and FL (111.82 mg, p < 0.001) groups. Mean serum creatinine of recipientsat day 1 post-op was the highest in the FL group (p < 0.0001 FL vs. LDN, p < 0.001 FL vs. DL), but there were no significant differences between the three groups at 2 weeks, 6, 12, 18, and 24 months post-operation (p > 0.45).Conclusions: Although a lower pain experience of LDN was not indicated, the use of LDN should be favoured over DL and FL as it is associated with fewer complications, and shorter length of stay.Of note, DL appears to be associated with higher complications and is likely not a preferred option for donor nephrectomy.

Sign in / Sign up

Export Citation Format

Share Document