Delayed Presentation of Respiratory Symptoms and Prolonged Survival in Homozygous α3 Integrin Deficiency

2020 ◽  
Vol 57 (3) ◽  
pp. 268-269
Author(s):  
Sumitha Udayashankar Tarur ◽  
S. Srinivasan ◽  
Arasar Seeralar
2021 ◽  
Vol 4 (Supplement_1) ◽  
pp. 152-154
Author(s):  
E Carrión-Jaramillo ◽  
M Chilvers ◽  
C Barker

Abstract Background Infliximab is used to induce and maintain remission of inflammatory bowel disease (IBD). Pulmonary abnormalities in IBD are rare, but comprise airway, parenchymal and interstitial lung complications. Pulmonary adverse effects reported after Infliximab include tuberculosis reactivation, invasive aspergillosis, interstitial pneumonitis, pulmonary oedema and alveolar hemorrhage. Aims Four cases previously reported associate Infliximab to eosinophilic pneumonitis. However, this is the first in pediatrics. Methods Case report of an adolescent female with IBD and a rare adverse reaction to Infliximab Results Our patient was diagnosed with ulcerative pancolitis, Mayo 2, initially treated with 5-ASA. Five months into therapy she was switched to azathioprine and Infliximab (10mg/kg every 8 weeks) due to worsening disease. She attained and maintained clinical and biochemical remission for the next 1.5 years. Azathioprine was stopped at the patient’s request. One month later, she presented with cough and nasal congestion but was afebrile. Peripheral eosinophilia was noted. Salbutamol and steroid inhalers were started, without improvement. She was admitted to hospital due to progressive cough, shortness of breath, and wheezing. Physical examination was otherwise unremarkable. Pulmonary function test showed FVC of 69%, FEV1 of 45% with bronchodilator effect of 12% and a FEV1/FVC of 59. Her blood work showed eosinophilia (4.42 x109/L) and an elevated IgE (4596 ug/L). Serum-specific IgE for Aspergillus was positive; but skin testing was negative. An X-ray showed marked bilateral interstitial thickening in the mid and lower zones. Chest CT showed extensive bronchiectasis with bronchial mucous plugging. A bronchoscopy with bronchoalveolar lavage (BAL) reported marked eosinophilia, negative for mycobacterium, bacterial and fungal growth. Other infectious and autoimmune etiologies were ruled out. She responded successfully to high-dose prednisone (50 mg/day). At discharge, symptoms had improved, and her pulmonary function tests were normal (FEV1 of 95% predicted and FEV1/FVC 84). Upon steroid taper, she relapsed in pulmonary symptoms, eosinophilia, as well as a flare of her IBD. Reinduction with steroids resolved her symptoms and eosinophilia. Due to her loss of response to Infliximab and pulmonary, she was switched to Vedolizumab with good response. After stopping Infliximab her lung function improved significantly (FVC of 96% predicted and FEV1 of 98% predicted) and has remained so. Peripheral eosinophilia dropped to 0.2 x109/L. Her IBD is currently in remission without any respiratory symptoms. Conclusions Eosinophilic pneumonitis can be a complication of Infliximab. Our patient had a delayed presentation of respiratory symptoms, 16 months after a satisfactory response to Infliximab. Although responsive to steroids, she ultimately needed to be switched to another immunosuppressive agent. Funding Agencies None


2017 ◽  
Vol 190 ◽  
pp. 268-270.e1 ◽  
Author(s):  
Juan A. López-Andreu ◽  
Antonio D. Hidalgo-Santos ◽  
Miguel A. Fuentes-Castelló ◽  
Nuria Mancheño-Franch ◽  
Juan A. Cerón-Pérez ◽  
...  

2021 ◽  
Vol 14 (9) ◽  
pp. e244483
Author(s):  
Corne De Vos ◽  
Charlie Kohler ◽  
Natash Fourie ◽  
Pierre Goussard

Delayed presentation of oesophageal atresia (OA) with a tracheo-oesophageal fistula (TOF) is rare. Only a few case reports and two larger case series have been published. We present a neonate who was referred to our unit on day 14 of life with a missed OA and a TOF, having survived without any feeds or total parenteral nutrition up until referral.We concluded that although such a delayed presentation is rare and avoidable, it does occur. This case highlights the necessity of good feedback to the referral hospitals with education on how to prevent this from recurring again. It also emphasises the necessity of a comprehensive clinical examination of all newborn babies. A high index of suspicion for OA with or without a TOF is essential in all babies with clinical drooling, feeding problems (from the first feed) and/or respiratory symptoms especially if combined with antenatal polyhydramnios.


2005 ◽  
Vol 2 (1) ◽  
pp. 41-45
Author(s):  
AR Maqsood Ahmed ◽  
DI Bhat ◽  
B Indira Devi ◽  
BA Chandramouli
Keyword(s):  

2018 ◽  
Author(s):  
Maria Dehmel ◽  
Gabriele Hahn ◽  
Sebastian Brenner ◽  
Sonja Walsh ◽  
Nataliya Didonato ◽  
...  

2011 ◽  
Vol 3 (11) ◽  
pp. 355-357
Author(s):  
Dr. Pradeep Pakalapati ◽  
◽  
Dr Spurthi Kalva ◽  
Dr Vivekanand Undrakonda ◽  
Dr GudimetlaSandeep Reddy
Keyword(s):  

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