Oral appliance to assist non-invasive ventilation in a patient with amyotrophic lateral sclerosis.

BACKGROUND: From the moment the respiratory muscle groups are affected in amyotrophic lateral sclerosis (ALS), respiratory complications will be the major cause of morbidity and mortality. Untreated respiratory muscle impairment leads to respiratory insufficiency and additionally to difficulties in airway secretion clearance. Non-invasive ventilation (NIV) is the first choice in treating respiratory insufficiency in ALS as it improves sleep-related symptoms, quality of life and life expectancy. Nevertheless, NIV is not always effective, probably due to bulbar dysfunction or anatomical abnormalities. As a result, tracheostomy ventilation (TV) may become necessary. METHODS: In this case report, we present a 60-year-old female with ALS, for whom it was not possible to provide a sufficient tidal volume with NIV. A chin lift was performed while the patient was awake to see if a more anterior jaw position would lead to an increased tidal volume. As this was the case, a mandibular advancement device (MAD) was fabricated. RESULTS: With a combination of a MAD and NIV, the upper airway obstructions were overcome and a good ventilation and adherence to therapy were seen. CONCLUSIONS: When there is the presumption of airway obstructions in combination with an ineffective NIV, we advise to perform a chin lift to assess whether the obstructions can be overcome by a more anterior jaw position. If that is the case, NIV may be combined with MAD to establish effective ventilation and avoid the use of TV.

Volume-targeted versus pressure-targeted noninvasive ventilation in patients with chest-wall deformity: a pilot study.

BACKGROUND: Long-term noninvasive ventilation (NIV) is an effective treatment for patients with chronic respiratory failure due to chest-wall deformity, but it is unknown if the time required for the patient to adjust to long-term NIV depends on whether the NIV is volume-targeted or pressure-targeted. OBJECTIVE: To determine whether volume controlled or pressure controlled NIV is easier to implement in patients with chronic respiratory failure due to chest-wall deformity. METHODS: We randomized 16 ventilator-naïve patients to receive either volume-targeted or pressure-targeted nocturnal NIV. The primary outcome was the number of days needed to successfully establish NIV, defined as adequate adjustment and effective ventilation, as measured with overnight arterial blood gas measurement. RESULTS: Two patients did not tolerate volume NIV, and switched to pressure NIV. NIV was successfully established in both groups after a median 6.0 days. There were no significant differences between the groups at any time point in P(aCO(2)) or P(aO(2)) improvement, nor in changes over time. CONCLUSIONS: There was no significant difference in days needed to successfully establish volume NIV versus pressure NIV in patients with chest-wall deformity. However, two patients switched successfully from volume NIV to pressure NIV, which suggests that they preferred pressure NIV.

Kidney Function in Patients With Neuromuscular Disease: Creatinine Versus Cystatin C.

Background: Accurate measurement of kidney function in patients with neuromuscular disorders is challenging. Cystatin C, a marker not influenced by skeletal muscle degradation, might be of clinical value in these patients. Methods: We consecutively enrolled 39 patients with neuromuscular disorders. We investigated the association of the eGFR, based on plasma creatinine and Cystatin C, with clinical and biochemical variables associated with kidney function, namely age and galectin-3. Results: Creatinine-based eGFR was 242 (±80) and Cystatin C-based eGFR was 110 (±23) mL/min/1.73 m2. Cystatin C-based eGFR was associated with age (ß -0.63 p < 0.0001) and galectin-3 levels (ß -0.43 p < 0.01), while creatinine-based eGFR was not (ß -0.22 p = 0.20; ß -0.28 p = 0.10). Sensitivity analyses in Duchenne and Becker patients revealed the same results: Cystatin C-based eGFR was associated with age (ß -0.61 p < 0.01) and galectin-3 levels (ß -0.43 p = 0.05), while creatinine-based eGFR was not (ß -0.32 p = 0.13; ß -0.34 p = 0.14). Conclusions: These data indicate that estimation of renal function in patients with neuromuscular disorders cannot reliably be achieved with creatinine, while Cystatin C appears a reasonable alternative. Since a large proportion of patients with neuromuscular disorders develops heart failure, and requires heart failure medication, adequate monitoring of renal function is warranted.

Improvement in hypercapnia does not predict survival in COPD patients on chronic noninvasive ventilation.

PURPOSE: It has recently been shown that chronic noninvasive ventilation (NIV) improves a number of outcomes including survival, in patients with stable hypercapnic COPD. However, the mechanisms responsible for these improved outcomes are still unknown. The aim of the present study was to identify parameters associated with: 1) an improved arterial partial pressure of carbon dioxide (PaCO2) and 2) survival, in a cohort of hypercapnic COPD patients treated with chronic NIV. PATIENTS AND METHODS: Data from 240 COPD patients treated with chronic NIV were analyzed. Predictors for the change in PaCO2 and survival were investigated using multivariate linear and Cox regression models, respectively. RESULTS: A higher level of bicarbonate before NIV initiation, the use of higher inspiratory ventilator pressures, the presence of anxiety symptoms, and NIV initiated following an exacerbation compared to NIV initiated in stable disease were associated with a larger reduction in PaCO2. A higher body mass index, a higher FEV1, a lower bicarbonate before NIV initiation, and younger age and NIV initiation in stable condition were independently associated with better survival. The change in PaCO2 was not associated with survival, neither in a subgroup of patients with a PaCO2 >7.0 kPa before the initiation of NIV. CONCLUSION: Patients with anxiety symptoms and a high bicarbonate level at NIV initiation are potentially good responders in terms of an improvement in hypercapnia. Also, higher inspiratory ventilator pressures are associated with a larger reduction in PaCO2. However, the improvement in hypercapnia does not seem to be associated with an improved survival and emphasizes the need to look beyond PaCO2 when considering NIV initiation.

Automated erythrocytapheresis in severe falciparum malaria: A critical appraisal.

Imported falciparum malaria is increasing in Western countries. In patients with severe disease, exchange transfusion has been added to antimalarial and conventional supportive therapy to increase removal of parasitized erythrocytes, but hemodynamic compromise limits its use; automated erythrocytapheresis may be advantageous. We review published reports of patients with severe falciparum malaria treated by automated erythrocytapheresis combined with standard therapy and add three more cases to the literature. No studies have been conducted to evaluate its clinical efficacy, and this adjunct therapy should therefore be considered as salvage therapy. Apheresis of red cells appears feasible, safe and effective in rapidly reducing parasite count.