- Clinical & laboratory features of seventy-eight UK patients with Good's syndrome (thymoma & hypogammaglobulinemia). [Journal Article]
- CEClin Exp Immunol 2018 Sep 14
- Good's syndrome (thymoma and hypogammaglobulinemia) is a rare secondary immunodeficiency disease, previously reported in the published literature as mainly individual cases or small case series. We u...
Good's syndrome (thymoma and hypogammaglobulinemia) is a rare secondary immunodeficiency disease, previously reported in the published literature as mainly individual cases or small case series. We use the national UK-Primary Immune Deficiency (UKPID) registry to identify a large cohort of patients in the UK with this PID to review its clinical course, natural history and prognosis. Clinical information, laboratory data, treatment and outcome were collated and analysed. Seventy-eight patients with a median age of 64 years, 59% of whom were females were reviewed. Median age of presentation was 54 years. Absolute B cell numbers and serum immunoglobulins were very low in all patients and all received immunoglobulin replacement therapy. All patients had undergone thymectomy and 9 (12%) had thymic carcinoma (four locally invasive and five had disseminated disease) requiring adjuvant radiotherapy and/or chemotherapy. CD4 T-cells were significantly lower in these patients with malignant thymoma. Seventy-four (95%) presented with infections, 35 (45%) had bronchiectasis, seven (9%) chronic sinusitis, but only eight (10%) had serious invasive fungal or viral infections. Patients with AB-type thymomas were more likely to have bronchiectasis. Twenty (26%) suffered from autoimmune diseases (pure red cell aplasia, hypothyroidism, arthritis, myasthenia gravis, SLE, Sjogren's syndrome). There was no association between thymoma type and autoimmunity. Seven (9%) patients had died. Good's syndrome is associated with significant morbidity relating to infectious and autoimmune complications. Prospective studies are required to understand why some patients with thymoma develop persistent hypogammaglobulinemia. This article is protected by copyright. All rights reserved.
- Risk factors associated with obstructive sleep apnea-hypopnea syndrome in Chinese children: A single center retrospective case-control study. [Journal Article]
- PlosPLoS One 2018; 13(9):e0203695
- Pediatric obstructive sleep apnea-hypopnea syndrome is caused by multiple factors. The present study aimed to investigate the potential risks of pediatric obstructive sleep apnea hypopnea syndrome (O...
Pediatric obstructive sleep apnea-hypopnea syndrome is caused by multiple factors. The present study aimed to investigate the potential risks of pediatric obstructive sleep apnea hypopnea syndrome (OSAHS) and their correlation with the disease severity. A total of 338 pediatric patients with OSAHS (polysomnography (PSG) diagnosis) were enrolled between June 2008 and October 2010. These pediatric patients were divided into mild, moderate and severe subgroups according to the obstructive apnea index (OAI) and/or apnea hypoventilation index (AHI). A total of 338 pediatric patients with vocal nodules who were without obstruction of the upper respiratory tract were enrolled as the control group. The patients were analyzed retrospectively. The average number of upper respiratory tract infections each year and tonsil hypertrophy, adenoid hypertrophy, positive serum tIgE, chronic sinusitis, nasal stenosis, craniofacial features and obesity were significantly higher in OSAHS compared with controls (P<0.01). The parameters the average number of upper respiratory tract infections each year (OR: 1.395, 95% CI: 1.256-1.550), adenoid hypertrophy (OR: 8.632, 95% CI: 3.990-18.672), tonsil hypertrophy (OR: 9.138, 95% CI: 4.621-18.073), nasal stenosis (8.023, 95% CI: 3.633-17.717) and chronic sinusitis (OR: 27.186, 95% CI: 13.310-55.527) were independent factors of pediatric OSAHS (P<0.01). The distribution of chronic sinusitis, nasal stenosis, craniofacial features and obesity indicated a gradual increasing trend in the severity of OSAHS (P<0.01). Number of upper respiratory tract infections per year, adenoid hypertrophy, tonsil hypertrophy, chronic sinusitis, nasal stenosis, infections, allergic reactions, craniofacial features and obesity may be potential risk factors of pediatric OSAHS.
- Does Medical Therapy Improve SinoNasal Outcomes Test-22 Domain Scores? An Analysis of Clinically Important Differences. [Journal Article]
- LLaryngoscope 2018 Sep 12
- CONCLUSIONS: MCID values for SNOT-22 total and domain scores in patients electing CAMT are similar to previously published MCID values associated with ESS, indicating that MCID values are independent of treatment modality selection. Therefore, despite evidence of statistical significance, CAMT for CRS may not be associated with clinically discernable improvements in average SNOT-22 domain scores.
- Does Delaying Endoscopic Sinus Surgery Adversely Impact Quality-of-Life Outcomes? [Journal Article]
- LLaryngoscope 2018 Sep 12
- CONCLUSIONS: Symptom duration was not associated with mean preoperative disease severity or QOL. Patients with long-term symptom duration reported the greatest mean postoperative QOL improvement, suggesting that delayed surgical intervention may not reduce QOL improvements following ESS.
- Impact of Gastroesophageal Reflux Disease on Mucosal Immunity and Atopic Disorders. [Review]
- CRClin Rev Allergy Immunol 2018 Sep 11
- Atopic disorders and gastroesophageal reflux disease (GERD) are some of the most common medical conditions treated by primary care physicians and specialists alike. The observation that atopic disord...
Atopic disorders and gastroesophageal reflux disease (GERD) are some of the most common medical conditions treated by primary care physicians and specialists alike. The observation that atopic disorders, like asthma, allergic rhinitis and sinusitis, food allergies, atopic dermatitis, contact dermatitis, and eosinophilic esophagitis are common comorbidities in patients with GERD raises the question of the nature of the relationship that may exist between GERD and atopic disorders. In this article, we review the pathophysiology of GERD, its effect on the immune system, the effect of acid-blocking medications on allergic responses, as well as several common atopic conditions that have been associated with GERD including asthma, chronic rhinosinusitis (CRS), allergic rhinitis (AR), atopic dermatitis (AD), contact dermatitis (CD), food allergies, proton pump inhibitor (PPI)-responsive esophageal eosinophilia (PPI-REE), and eosinophilic esophagitis (EoE). In each condition, the evidence of a causal link is not definitive. Although the relationship between asthma and GERD remains controversial, evidence suggests that a subset of asthma patients with documented GERD may experience improved asthma control following appropriate treatment of GERD. The relationship of GERD to allergic rhinitis and chronic sinusitis is weak; however, studies support the concept that treatment of frequent episodes of GERD can have a positive effect on rhinitis and sinusitis overall. The relationship between allergic sensitization and GERD is likely bidirectional. GERD may induce changes in the mucosal immune system that may favor the development of food allergy and allergic sensitization to aeroallergens; however, the underlying mechanisms have not been established.
- Bacteriological analysis of isolated chronic sinusitis without polyps. [Journal Article]
- PDPostepy Dermatol Alergol 2018; 35(4):375-380
- CONCLUSIONS: There is an indication that the presence of Staphylococcus aureus is associated with isolated sinusitis, especially in women.
- Conjunctivitis as a manifestation of Wegener's Granulomatosis. [Journal Article]
- JCJ Curr Ophthalmol 2018; 30(3):268-272
- CONCLUSIONS: Because prolonged conjunctivitis occurs only rarely in association with systemic disease, ophthalmologists should be aware of this potential, particularly in patients with GPA.
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- Fungal sinusitis among patients with chronic rhinosinusitis who underwent endoscopic sinus surgery. [Journal Article]
- OPOtolaryngol Pol 2018 Jun 20; 72(4):35-41
- CONCLUSIONS: The most common form of non-invasive fungal sinusitis is the so-called fungus ball, which was also confirmed in our report (95% of the test subjects). AFRS is more likely to occur in warm, moist climates that favour the growth of fungi.