Activity,Severity and Impact of Respiratory Disease in Primary Antibody Deficiency Syndromes

Purpose

Some patients with primary antibody deficiency (PAD) syndromes develop bronchiectasis. In immunocompetent patients with bronchiectasis, key clinico-pathophysiological relationships exist between exacerbation frequency, lung function, health-status, infection and inflammation. It is not known whether such relationships are present in PAD. It is also not known how local and systemic inflammation in PAD compares with that in immunocompetent (non-PAD) bronchiectasis patients.

Method

We assessed symptoms, exacerbation frequency, health-status, lung function, CT, airway and systemic inflammation and infection in 33 PAD patients and 20 immunocompetent controls with bronchiectasis.

Results

Despite less severe airflow obstruction, PAD patients had similar health-status impairment and greater airway (sputum log₁₀ IL-6 2.71 vs. 1.81 pg/ml, p?=?0.001) and greater systemic inflammation than immunocompetent bronchiectasis controls (serum log₁₀ CRP 0.77 vs. 0.36 mg/l, p?=?0.001). In PAD, cross-sectional markers of disease severity (CT and lung function) did not relate to inflammatory markers of disease activity, however there was a relationship between FEV₁ decline rate and systemic inflammation (IL-6; r?=?0.42, p?=?0.036) and the magnitude of the systemic inflammatory response was related to that in the airway. Correlation between generic SF36 and respiratory SGRQ questionnaires (r?=??0.79, p?<?0.001) suggests that much health-status impairment in PAD relates to respiratory involvement. Health-status was associated with dyspnoea (rho?=?0.77, p?<?0.001), respiratory infection frequency (rho?=?0.48, p?=?0.016), lung function (FEV₁: r?=??0.60, p?=?0.001) and rate of lung function decline (r?=?0.41, p?=?0.047).

Conclusion

The major findings of this analysis are that in patients with PAD, cross-sectional markers of disease severity such as lung function and CT extent of disease do not reflect disease activity as assessed by airway and systemic inflammation. In addition, there is a relationship between the rate of progression of lung disease and the severity of the systemic inflammatory response which itself is related to that in the airway. Much of the quality of life impact in PAD relates to respiratory involvement, specifically the severity of airflow obstruction, respiratory exacerbation frequency and dyspnoea. Finally, patients with PAD had greater airway and systemic inflammation than a control population with non-PAD bronchiectasis which may suggest a dysregulated airway immune response.     

Update on Chronic Diarrhea: A Run-Through for the Clinician

Chronic diarrhea is a common patient complaint, with an estimated prevalence of 5?%. Diarrhea is defined as >200?g/day of stool with decreased consistency, and chronic diarrhea is defined as lasting more than 4?weeks. The purpose of this review is to guide the clinician??s diagnostic evaluation and management of chronic diarrhea, rather than providing a textbook comprehensive review of the subject, focusing on the patient in developed countries and excluding the immune suppressed patient. While the investigation and treatment of chronic diarrhea can be challenging due to its myriad causes, when the clinician employs a practical approach, dividing chronic diarrhea into bloody, fatty, and watery causes, it simplifies and streamlines the work-up and management plan and leads to improved patient outcomes.     

Femoral artery access site closure with perclose suture mediated device in coronary interventions

ObjectiveTo compare the outcome of suture mediated vascular closure device Perclose Proglide (PP) with manual compression (MC) following transfemoral access for coronary interventions (CI).MethodsIt is a retrospective, observational, single centre study from January 2018 to September 2019. Consecutive patients undergoing interventions through transfemoral access were divided into PP and MC groups. Those with less than 3 months follow up were excluded. Two groups were compared for baseline characteristics and various complications at 24 h and at 30 days.ResultsOut of 1743 patients studied, PP group included 1343 and MC group, 400 patients. Both groups were comparable in baseline characteristics, sheath size and use of antiplatelets and anticoagulation. PP group had significantly less minor bleeding (P = .01, CI 0.34–4.03) and hematoma (P = .0007, CI 0.95–5.10) at 24 h. At 30 days, minor bleeding (P < .0001, CI 0.97–4.25), hematoma (P = .0002, CI 1.05–4.93) and pseudo-aneurysm (P = .0095, CI 0.03–1.18) were also significantly less in PP group. Obesity (OR 3.5, CI 1.29–9.49) and hypertension (OR 2.41, CI 1.12–5.19) were associated with increased minor bleeding at 24 h. Device failure rate was 2.38%.ConclusionsPP device is safe, effective and is associated with fewer complications than MC in CI. Device failure rate is low. Obesity and hypertension are associated with increased minor bleeding in both groups.     

Can peak work rate predict peak oxygen uptake in children with juvenile idiopathic arthritis?

Objective

To examine whether peak work rate (W_peak) can predict peak oxygen uptake (VO _2peak) in children with juvenile idiopathic arthritis (JIA).

Methods

Ninety‐one patients with JIA with a mean ± SD age of 11.4 ± 2.9 years underwent a cardiopulmonary exercise test in which VO _2peak and W_peak were determined. A multivariate regression model was used to formulate a regression equation to predict VO _2peak using W_peak and anthropometric and demographic details. This regression equation was subsequently cross‐validated using an unrelated data set from children with JIA (n = 17).

Results

The following linear regression equation to predict VO _2peak was established: VO _2peak (liters/minute) = 0.308 + 0.146 × sex (0 = female, 1 = male) + 0.005 × weight (kg) + 0.008 × W_peak (W) (R² = 0.91, standard error of the estimate = 0.18 liter/minute). Using this equation, the predicted VO _2peak was strongly related to the measured VO _2peak (r = 0.96, P < 0.0001). Bland and Altman analysis revealed a mean difference of 0.01 liter/minute and limits of agreement between ?0.35 and 0.35 liter/minute.

Conclusion

This study suggests that W_peak is a strong predictor of VO _2peak in children with JIA and may be used as a surrogate measure of VO _2peak in situations where it is not possible to formally assess VO _2peak.