Pulmonary manifestations of amyloidosis.

Amyloidosis is caused by abnormal protein deposition in various tissues, including the lungs. Pulmonary manifestations of amyloidosis may be categorized by areas of involvement, such as parenchymal, large airway and pleural involvement. We describe four distinct manifestations of amyloidosis involving the lung and review their clinical, radiological and pathological features and summarize the evidence for treatment in each of these presentations. We describe alveolar-septal amyloidosis, cystic amyloid lung disease, endobronchial amyloidosis and pleural amyloidosis.

Evaluation of hemodynamic parameters among patients with myeloproliferative neoplasms and suspected pulmonary hypertension.

Myeloproliferative neoplasms (MPNs) are associated with pulmonary hypertension (PH). We studied MPN patients who underwent right-heart-catheterization (RHC) to identify hemodynamic differences between MPN-subtypes. Per RHC, hemodynamics were classified as pre, post or combined pre and post-capillary PH. One-way analysis-of-variance (ANOVA) was used to compare hemodynamic differences among MPN-subtypes. Correlation of RVSP between trans-thoracic echocardiography (TTE) and RHC was evaluated. We included 68 patients. Median age was 63. Fifty-nine percent were male and 87% Caucasian. Polycythemia vera and essential thrombocythemia were the most common subtypes. On TTE, 91.5% had PH. On RHC, only 29% met criteria for pre-capillary PH. No MPN-subtype was more likely than others to have pre-capillary PH. Bland-Altman analysis showed significant intra-person variability between TTE and RHC-derived right ventricular systolic pressures. Post-capillary involvement is more common than precapillary PH in MPN. Type of PH does not appear to differ by MPN-subtype.

Association Between Volume of Fluid Resuscitation and Intubation in High-Risk Patients With Sepsis, Heart Failure, End-Stage Renal Disease, and Cirrhosis.

BACKGROUND: Initial fluid resuscitation volume for sepsis is controversial, particularly in patients at high baseline risk for complications. This study was designed to assess the association between 30 mL/kg crystalloids and intubation in patients with sepsis or septic shock and heart failure, end-stage renal disease, or cirrhosis. METHODS: This propensity score-matched retrospective cohort study included patients with sepsis or septic shock admitted to a large medical ICU. Primary exposure was IV fluid volume in the first 6 h following sepsis diagnosis, divided into two cohorts: ≥ 30 mL/kg (standard group) and < 30 mL/kg (restricted group). The primary outcome was need for mechanical ventilation within 72 h following initiation of fluid resuscitation. Secondary outcomes were length of stay, ventilator days, and time to intubation. RESULTS: A total of 208 patients were included, with 104 (50%) in the restricted group (< 30 mL/kg) and 104 in the standard group (≥ 30 mL/kg). No difference in intubation incidence was detected between the two groups, with 36 patients (35%) in the restricted group and 33 (32%) in the standard group (adjusted OR, 0.75; 95% CI, 0.41-1.36; P = .34) intubated. There was no difference between standard and restricted groups in alive ICU-free days (17 ± 11 days vs 17 ± 10 days; P = .64), duration of mechanical ventilation (10 ± 12 days vs 11 ± 16 days; P = .96), or hours to intubation (16 ± 19 h vs 14 ± 15; P = .55). CONCLUSIONS: No differences were detected in the incidence of intubation in patients with sepsis and cirrhosis, end-stage renal disease, or heart failure who received guideline-recommended fluid resuscitation with 30 mL/kg compared with patients initially resuscitated with a lower fluid volume.