scholarly journals Immunological Findings and Clinical Outcomes of Infants With Positive Newborn Screening for Severe Combined Immunodeficiency From a Tertiary Care Center in the U.S.

2021 ◽  
Vol 12 ◽  
Author(s):  
Vasudha Mantravadi ◽  
Jeffrey J. Bednarski ◽  
Michelle A. Ritter ◽  
Hongjie Gu ◽  
Ana L. Kolicheski ◽  
...  
Keyword(s):  
Newborn Screening ◽  
Clinical Outcomes ◽  
Care Center ◽  
Tertiary Care ◽  
Severe Combined Immunodeficiency ◽  
Tertiary Care Center ◽  
Combined Immunodeficiency ◽  
Perinatal Complications ◽  
Positive Correlation ◽  
Novel Variants

The implementation of severe combined immunodeficiency (SCID) newborn screening has played a pivotal role in identifying these patients early in life as well as detecting various milder forms of T cell lymphopenia (TCL). In this study we reviewed the diagnostic and clinical outcomes, and interesting immunology findings of term infants referred to a tertiary care center with abnormal newborn SCID screens over a 6-year period. Key findings included a 33% incidence of non-SCID TCL including infants with novel variants in FOXN1, TBX1, MYSM1, POLD1, and CD3E; 57% positivity rate of newborn SCID screening among infants with DiGeorge syndrome; and earlier diagnosis and improved transplant outcomes for SCID in infants diagnosed after compared to before implementation of routine screening. Our study is unique in terms of the extensive laboratory workup of abnormal SCID screens including lymphocyte subsets, measurement of thymic output (TREC and CD4TE), and lymphocyte proliferation to mitogens in nearly all infants. These data allowed us to observe a stronger positive correlation of the absolute CD3 count with CD4RTE than with TREC copies, and a weak positive correlation between CD4RTE and TREC copies. Finally, we did not observe a correlation between risk of TCL and history of prenatal or perinatal complications or low birth weight. Our study demonstrated SCID newborn screening improves disease outcomes, particularly in typical SCID, and allows early detection and discovery of novel variants of certain TCL-associated genetic conditions.

Higher Incidence Rates of Hypothyroidism and Late TSH Rise in Preterm Very-Low-Birth-Weight Infants at a Tertiary Care Center

2018 ◽  
Vol 89 (4) ◽  
pp. 224-232 ◽  
Author(s):  
Hala Tfayli ◽  
Lama Charafeddine ◽  
Hani Tamim ◽  
Joanne Saade ◽  
Rose T. Daher ◽  
...  
Keyword(s):  
Birth Weight ◽  
Low Birth Weight ◽  
Newborn Screening ◽  
Preterm Infants ◽  
Very Low Birth Weight ◽  
Care Center ◽  
Compliance Rate ◽  
Tertiary Care ◽  
Tertiary Care Center ◽  
Low Birth Weight Infants

Background/Aims: Preterm newborns with a very low birth weight (VLBW) of < 1,500 g have an atypical form of hypothyroidism with a delayed rise in TSH, necessitating a second newborn screening specimen collection. The aims of this study were to survey the compliance with second newborn screening to detect delayed TSH rise in VLBW preterm infants at a tertiary care center, and to determine the rate of atypical hypothyroidism. Methods: Retrospective review of the records of 104 preterm VLBW infants. Late TSH rise was defined as an increase in TSH concentration after 14 days of age in the presence of a normal initial screen. Results: The compliance rate was 92% for the second screening. High rates of hypothyroidism (16.3%) and of late TSH rise (4.8%) were detected. Patients with hypothyroidism had a significantly lower birth weight (p = 0.01) and longer hospital stay (p = 0.004). Patients with late versus those with early TSH rise had a significantly lower mean birth weight (851 ± 302 vs. 1,191 ± 121 g, p = 0.004). Conclusion: The rates of early and late TSH rise in this VLBW population were higher than those in the literature and could be due to the use of povidone-iodine disinfectants. The yield of a second TSH screening in this study was high indicating the need for vigilance in screening VLBW preterm infants.

scholarly journals Multivisceral resections for rectal cancers: short-term oncological and clinical outcomes from a tertiary-care center in India

2016 ◽  
Vol 7 (3) ◽  
pp. 345-353 ◽  
Author(s):  
Vishwas D. Pai ◽  
Sudhir Jatal ◽  
Vikas Ostwal ◽  
Reena Engineer ◽  
Supreeta Arya ◽  
...  
Keyword(s):  
Clinical Outcomes ◽  
Care Center ◽  
Tertiary Care ◽  
Tertiary Care Center ◽  
Short Term ◽  
Rectal Cancers

scholarly journals SARS-CoV-2 testing and clinical outcomes in a Texas tertiary care center labor and delivery unit

2021 ◽  
Vol 34 (2) ◽  
pp. 229-231
Author(s):  
Thomas Woodard ◽  
Lueke Anderson ◽  
Jessica Ehrig ◽  
Courtney Shaver ◽  
Michael Hofkamp
Keyword(s):  
Clinical Outcomes ◽  
Care Center ◽  
Tertiary Care ◽  
Labor And Delivery ◽  
Tertiary Care Center ◽  
Delivery Unit ◽  
Labor And Delivery Unit

scholarly journals Clinical Outcomes of Pediatric COVID-19 in a Tertiary Care Center in Bangkok, Thailand

IJID Regions ◽  
2021 ◽  
Author(s):  
Suvaporn Anugulruengkitt ◽  
Sirinya Teeraananchai ◽  
Napaporn Chantasrisawad ◽  
Pathariya Promsena ◽  
Watsamon Jantarabenjakul ◽  
...  
Keyword(s):  
Clinical Outcomes ◽  
Care Center ◽  
Tertiary Care ◽  
Tertiary Care Center

scholarly journals Deep Neck Space Infection: Clinical Outcomes of Patients Treated in a Tertiary Care Center

2021 ◽  
Vol 2 (1) ◽  
pp. 20-26
Author(s):  
Kerem Sami Kaya ◽  
İbrahim Yağcı ◽  
Uğur Doğan ◽  
Nurullah Seyhun ◽  
Suat Turgut
Keyword(s):  
Clinical Outcomes ◽  
Care Center ◽  
Tertiary Care ◽  
Tertiary Care Center ◽  
Space Infection ◽  
Deep Neck Space Infection

Cystic Fibrosis Newborn Screening: Five‐year Experience from a Tertiary Care Center

2021 ◽  
Author(s):  
Halime Nayir Buyuksahin ◽  
Nagehan Emiralioglu ◽  
Beste Ozsezen ◽  
Dilber Ademhan Tural ◽  
Birce Sunman ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Newborn Screening ◽  
Care Center ◽  
Tertiary Care ◽  
Tertiary Care Center

Abstract 240: Concomitant Use of Therapeutic Hypothermia with Mechanical Circulatory Support in Patients Post Cardiac Arrest in Cardiogenic Shock: A Single Center Experience

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Michael G Silverman ◽  
Molly H O’Brien ◽  
Kathleen R Avery ◽  
Annmarie Chase ◽  
Carol D Pierce ◽  
...  
Keyword(s):  
Cardiac Arrest ◽  
Cardiogenic Shock ◽  
Clinical Outcomes ◽  
Hospital Discharge ◽  
Mechanical Circulatory Support ◽  
Care Center ◽  
Tertiary Care ◽  
Circulatory Support ◽  
Tertiary Care Center ◽  
Baseline Characteristics

Background: The concurrent use of therapeutic hypothermia (TH) following cardiac arrest and mechanical circulatory support (MCS) for cardiogenic shock is becoming increasingly common. Little is known however, about the combined use of TH and MCS for patients after ROSC following a cardiac arrest who remain in cardiogenic shock. Therefore we describe the experience with concomitant use of TH and MCS from a large academic tertiary care center in Boston. Methods: Baseline characteristics and clinical outcomes at hospital discharge were reported for patients undergoing TH following cardiac arrest who also received MCS for cardiogenic shock. MCS included Intra-aortic balloon pump (IABP) two percutaneous ventricular assist devices (Impella, and TandemHeart), and extracorporeal membrane oxygenation (ECMO). Clinical outcomes included mortality as well as cerebral performance category (CPC) at hospital discharge. Results: There were a total of 14 patients who underwent concomitant TH and MCS following a cardiac arrest. Baseline characteristics and clinical outcomes are noted in the Figure. 9 patients underwent placement of IABP, 2 patients an Impella pump, 2 patients a TandemHeart, and 1 patient ECMO. All 14 cardiac arrests were due to cardiovascular etiologies; 9 of 14 had STEMI. 9 of 14 patients had an initial shockable rhythm. Mean age was 56 years (+/- 19), mean downtime was 35 minutes (+/- 24). All patients were vasopressor dependent. Bleeding events are noted in the table. 8 patients survived to hospital discharge, all with good neurologic outcome. These rates were comparable to the survival rates and neurologic outcomes among 82 patients who underwent TH post cardiac arrest (from cardiovascular etiologies) without concomitant MCS (Figure). Conclusion: Based on our experience from a large academic tertiary care center, concomitant use of TH and MCS is both safe and feasible with an encouraging rate of cardiac and neurologic recovery.

Clinical and Laboratory Characteristics of Primary Immunodeficiency Patients from a Tertiary Care Center in Pakistan

2020 ◽  
pp. 1-15
Author(s):  
Hamid Nawaz Tipu ◽  
Dawood Ahmed
Keyword(s):  
Primary Immunodeficiency ◽  
Clinical Presentation ◽  
Care Center ◽  
Tertiary Care ◽  
Severe Combined Immunodeficiency ◽  
Laboratory Data ◽  
Genetic Diagnosis ◽  
Warning Signs ◽  
Combined Immunodeficiency ◽  
Phagocytic Defects

Abstract Objective: The aim of this study was to describe and identify clinical presentation of primary immunodeficiency disorders (PIDs). Characteristic quantitative and qualitative immunological abnormalities have been described which help in establishing a definitive PID diagnosis. Methods: Cross sectional study in Immunology department, Armed Forces Institute of Pathology, Rawalpindi, Pakistan, from Jan 2016 to Dec 2018. Sixty patients of different PIDs including humoral defects, combined immunodeficiency, phagocytic defects and other miscellaneous disorders, were diagnosed over a period of 3 years in our institute. Their clinical presentation and laboratory data are presented in this study. Results: In 3 years, 40 (66%) males and 20 (33%) females were diagnosed, with 13 (21.6%) patients of humoral deficiency, 22 (36.6%) of severe combined immunodeficiency, 18 (30%) of phagocytic defects and 7 (11.6%) of other miscellaneous disorders. Maximum patients belonged to Punjab province, i.e., 23 (38.3%). Their mean age for initiation of symptoms was 7 + 12.6 months, while diagnosis was made at mean age of 26 + 39.28 months, in all groups combined. Respiratory infections were commonest presentation, in 46 (76.6%) patients. Also 46 (76.6%) patients had consanguineous parents. Presence of family history of PID in 27 (45%) patients is not associated with an earlier diagnosis (p 0.955). Each group of patients carried characteristic laboratory findings. Conclusion: PIDs should be suspected in offsprings with warning signs coming from consanguineous parents. There is a need to introduce genetic diagnosis of PIDs in order to timely diagnose less characteristic PID presentations. Continuous...

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