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Lane-Hamilton syndrome (LHS) presents a medical emergency, with 14% mortality due to Idiopathic Pulmonary Hemosiderosis (IPH) in acute phase. Despite the clinical severity of this entity, there has been no published review in the international literature, resulting in lack of awareness and delayed diagnosis.A rigorous search of international databases yielded a total of 80 LHS cases from January 1971 to August 2020. We analyzed 44 children (8.56 ± 4.72 years, 21 boys) and 36 adults (33.61 ± 13.41 years, 12 men) to present the clinical manifestations, radiological and immunological pattern, therapeutic approaches and outcome of LHS. We also elaborated on clinical and laboratory findings' associations to propose diagnostic indexes and clarified differences based on age distribution.Celiac disease (CD) and IPH diagnosis was made concurrently in 46 patients, whereas in 21 patients, the diagnosis of LHS was delayed for 2.5y (3 months-11 years). Hemoptysis ( n = 56, 70%), dyspnea ( n = 47, 58.8%), anemia ( n = 72, 90%), and iron deficiency ( n = 54, 67.5%) were most commonly observed. Medical history revealed recurrent episodes of hemoptysis ( n = 38) and persistent iron deficiency anemia ( n = 25) in need of multiple blood transfusions or iron supplementation. Patchy infiltrate opacities to consolidation predominated in children, whereas bilateral diffuse ground-glass opacities in adults. Duodenal biopsy was performed in 66 cases (diagnostic 87.8%), BAL in 51 (diagnostic 74.5%), and surgical lung biopsy in 20. Anti-tTG titer was positive in all 24 (54.6%) children and 19 (52.8%) adults that documented this assay. Prednisone or methylprednisolone pulse therapy and GFD were initiated in the acute phase, whereas chronic therapy included GFD, along with long-term prednisone in refractory cases. Three cases with severe respiratory failure or hemodynamic instability were intubated and a further three succumbed.A thorough understanding of LHS may reveal further diagnostic indexes and a consensus on therapy guidelines. Screening for CD is essential in all IPH cases for timely recognition and favorable outcome.

Introduction: The role of comorbidities in determining health-related quality of life (HRQL) in obstructive sleep apnea (OSA) pa-tients has not been thoroughly investigated. Commonly used comorbidity tools, such as Charlson Comorbidity Index (CCI), have been designed with mortality as the outcome variable. A new tool, the Functional Comorbidity Index (FCI), has been especially developed to assess the effect of comorbidities on the "physical functioning" subscale of the Medical Outcomes Short Form-36 Health Survey (SF-36)., Aims: 1) To determine the role of FCI in the prediction of the effect of comorbidities on HRQL in OSA. 2) To determine whether FCI and CCI are equally robust in predicting the effect of comorbidities on HRQL in OSA., Material and Methods: Two hundred and fifty-five OSA patients were enrolled. Patients completed the SF-36 and the Medical Outcomes Study Sleep Scale (MOS-SS) forms, while their comorbidity status was assessed by FCI and CCI. The SF-36 physical (PCS-36) and mental component summary (MCS-36) scores were also calculated., Results: PCS-36 was predicted by FCI (p < 0.001), male gender (p = 0.001), BMI (p = 0.002) and the "awakening with "breathlessness/headache" MOS-SS subscale (p = 0.011) (R2 = 0.348). Among these predictors, FCI exerted the most important quantitative effect. MCS-36 was predicted only by the "sleep disturbance" (p = 0.005) and the "awakening with breathlessness/headache" MOS-SS subscales (p < 0.001) (R2 = 0.221)., Conclusions: In patients with OSA, FCI is an independent predictor of the physical aspect of their HRQL. FCI is more robust than CCI in assessing the effect of comorbidities on HRQL in OSA.

Background: Latest evidence suggests that periodontitis is prevalent among patients with chronic obstructive pulmonary disease (COPD), while recent studies have also reported a potential benefit of periodontal treatment on several COPD outcomes. This systematic review aims to determine the impact of periodontal treatment on exacerbation rate, lung function and quality of life of COPD patients., Methods: A systematic search of electronic databases of PubMed, Scopus, Virtual Health Library, ScienceDirect, Wiley Online Library, Web of Science, ProQuest Dissertation and Theses Global and Google Scholar was conducted. Search restricted to studies involving human subjects which were published from January 2000 to March 2020 in English language. Distiller Systematic Review software was used for data management. Risk of bias was assessed using Risk of Bias 2 (RoB2) and Risk of Bias for non-randomized studies of intervention (ROBINS-I) tools. Overall quality of evidence was judged based on Grading of Recommendations Assessment, Development and Evaluation working group methodology., Results: Out of 1442 articles retrieved, 7 full text articles were included in the review. Limited evidence suggests that periodontal treatment in patients with COPD and periodontitis is associated with reduced exacerbation frequency and a slower lung function decline rate, while its effects on quality of life remain unclear. In addition, periodontal treatment in COPD is associated with lower hospitalization rates and reduced all-cause mortality. Significant methodological differences were noted amongst included studies, while very low-to-moderate overall quality of evidence was demonstrated., Conclusions: Although it is reasonable to advise COPD patients not to neglect their dental health, further studies are warranted to determine the role of periodontal therapy on COPD clinical outcomes., Trial Registration: PROSPERO 2020 (CRD42020158481). https://www.crd.york.ac.uk/prospero/display&#95;record.php?ID=CRD42020158481.

The Stewart-Figge acid-base model has been criticized for being mathematically complex. We aimed to develop simpler formalisms, which can be used at the bedside. The following simplifications were used: (1) [Ca 2+ ] and [Mg 2+ ] are replaced by their mid-reference concentrations (2) pH is set to 7.4. In the new model [SIDa] is replaced by its adjusted form, [SIDa, adj] = [Na + ] + [K + ] - [Cl - ] + 6.5 and [SIG] is replaced by "bicarbonate gap", [BICgap] = [SIDa, adj] - (0.28⋅[Albumin]) - (1.82⋅[Phosphate i ])- [HCO 3 ̄]. The diagnostic performance of the model was tested in 210 patients with acute respiratory diseases and 17 healthy volunteers. [BICgap] was also compared to albumin-corrected anion gap ([AGc]). The concordant correlation coefficient between [SIDa, adj] and [SIDa] and between [BICgap] and [SIG] was 0.98 in both comparisons. The mean bias (limits of agreement) of [SIDa, adj] - [SIDa] and of [BICgap] - [SIG] were 0.53 meq/l (- 0.46 to 1.53) and 0.50 meq/l (- 0.70 to 1.70), respectively. A [SIDa, adj] < 50.4 meq/l had an accuracy of 0.995 (p < 0.001) for the diagnosis of strong ion (SI) acidosis, while a [SIDa, adj] > 52.5 meq/l had an accuracy of 0.997 (p < 0.001) for the diagnosis of SI alkalosis. A [BICgap] > 11.6 meq/l predicted unmeasured ion (UI) acidosis with an accuracy of 0.997 (p < 0.001), while an [AGc] > 19.88 meq/l predicted UI acidosis with an accuracy of 0.994 (p < 0.001). The "[BICgap] model" is a reliable tool for the assessment of acid-base disorders in patients with acute respiratory diseases. [BICgap] is not inferior to [AGc] in the diagnosis of UI acidosis.

Respiratory muscle weakness is a major cause of morbidity and mortality in patients with neuromuscular diseases (NMDs). Respiratory involvement in NMDs can manifest broadly, ranging from milder insufficiency that may affect only sleep initially to severe insufficiency that can be life threatening. Patients with neuromuscular diseases exhibit very often sleep-disordered breathing, which is frequently overlooked until symptoms become more severe leading to irreversible respiratory failure necessitating noninvasive ventilation (NIV) or even tracheostomy. Close monitoring of respiratory function and sleep evaluation is currently the standard of care. Early recognition of sleep disturbances and initiation of NIV can improve the quality of life and prolong survival. This review discusses the respiratory impairment during sleep in patients with NMDs, the diagnostic tools available for early recognition of sleep-disordered breathing and the therapeutic options available for overall respiratory management of patients with NMDs., Competing Interests: The authors declare that they have no conflicts of interest., (Copyright © 2019 Athanasios Voulgaris et al.)

Purpose: To suggest a simplified method for strong ion gap ([SIG]) calculation., Patients and Methods: To simplify [SIG] calculation, we used the following assumptions: (1) the major determinants of apparent strong ion difference ([SID a ]) are [Na + ], [K + ] and [Cl - ] (2) [Ca 2+ ] and [Mg 2+ ] do not contribute significantly to [SID a ] variation and can be replaced by their reference concentrations (3) physiologically relevant pH variation is at the order of 10 -2 and therefore we can assume a standard value of 7.4. In the new model, [SID a ] is replaced by its adjusted form, i.e. [SID a,adj ] = [Na + ] + [K + ] - [Cl - ] + 6.5 and [SIG] is replaced by "bicarbonate gap", i.e. [BIC gap ] = [SID a,adj ] - (0.25·[Albumin]) - (2·[Phosphate]) - [HCO 3 - ]. The model was tested in 224 postoperative cardiac surgical patients., Results: Strong correlations were observed between [SID a,adj ] and [SID a ] (r = 0.93, p < 0.0001) and between [BIC gap ] and [SIG] (r = 0.95, p < 0.0001). The mean bias (limits of agreement) of [SID a,adj ] - [SID a ] and of [BIC gap ]-[SIG] was - 0.6 meq/l (- 2.7 to 1.5) and 0.2 meq/l (- 2 to 2.4), respectively. The intraclass correlation coefficients between [SID a,adj ] and [SID a ] and between [BIC gap ] and [SIG] were 0.90 and 0.95, respectively. The sensitivities and specificities for the prediction of a [lactate - ] > 4 meq/l were 73.4 and 82.3% for a [BIC gap ] > 12.2 meq/l and 74.5 and 83.1% for a [SIG] > 12 meq/l, respectively., Conclusions: The [BIC gap ] model bears a very good agreement with the [SIG] model while being simpler and easier to apply at the bedside. [BIC gap ] could be used as an alternative tool for the diagnosis of unmeasured ion acidosis.