Cytoplasmatic cellular enzymes, like LDH, in the extracellular space, although of no further metabolic function in this space, are still of benefit because they serve as indicators suggestive of disturbances of the cellular integrity induced by pathological conditions. Since LDH is an enzyme present in essentially all major organ systems, serum LDH activity is abnormal in a large number of disorders.Although the increase in total serum LDH activity is rather nonspecific, it is proposed that measurement of LDH activity levels and its isoenzyme pattern in pleural effusion and, more recently, in bronchoalveolar lavage fluid may provide additional information about lung and pulmonary endothelial cell injury.

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Background: Duchenne muscular dystrophy (DMD) is a progressive muscle disease. No curative therapy is currently available, but in recent decades standards of care have improved. These improvements include the use of corticosteroids and mechanical ventilation. Objective: To present a detailed population based report of the DMD disease course in The Netherlands and evaluate the effect of changes in care by comparing it with an historical Dutch DMD cohort (1961)(1962)(1963)(1964)(1965)(1966)(1967)(1968)(1969)(1970)(1971)(1972)(1973)(1974). Methods: Information about DMD patients was gathered through the Dutch Dystrophinopathy Database using a standardized questionnaire and information from treating physicians.

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It has been suggested that alterations in bronchoalveolar lavage fluid (BALF) reflect pathologic changes in the lung. Cytoplasmatic enzymes such as lactate dehydrogenase (LDH), alkaline phosphatase (ALP), and LDH isoenzymes are recognized indicators of cell damage or death. The aim of this study was to determine whether there is a relation between the enzyme activity and the cell types present in BALF. Therefore, BALF samples obtained from patients with various pulmonary disorders were studied. Out of these samples a group with mainly polymorphonuclear neutrophils (PMNs; n = 15; Group I) and another with mainly alveolar macrophages (AMs; n = 10; Group II) were selected. Additionally, the value of analysis of lysed cells in BALF for assessment of LDH-isoenzyme patterns was examined. The cell-free fraction of BALF of Group II showed lower LDH and ALP activity compared to Group I. The LDH-isoenzyme pattern also differed, with the LDH3/LDH5 ratios being lower in all BALF samples with predominantly PMNs than in BALF samples with predominantly AMs. Lysis of the cells present in the BALF samples by sonication prior to LDH-isoenzyme analysis provided no additional information beyond that found by analysis of the cell-free BALF. In conclusion, determination of enzyme activity appears to be useful in monitoring pulmonary inflammation.

Thirty percent of ALS patients have a frontotemporal syndrome (FS), defined as behavioral changes or cognitive impairment. Despite previous studies, there are no firm conclusions on the effect of the FS on survival and the use of non-invasive ventilation (NIV) in ALS. We examined the effect of the FS on survival and the start and duration of NIV in ALS. Behavioral changes were defined as >22 points on the ALS-Frontotemporal-Dementia-Questionnaire or ≥3 points on ≥2 items of the Neuropsychiatric Inventory. Cognitive impairment was defined as below the fifth percentile on ≥2 tests of executive function, memory or language. Classic ALS was defined as ALS without the frontotemporal syndrome. We performed survival analyses from symptom onset and time from NIV initiation, respectively, to death. The impact of the explanatory variables on survival and NIV initiation were examined using Cox proportional hazards models. We included 110 ALS patients (76 men) with a mean age of 62 years. Median survival time was 4.3 years (95 % CI 3.53–5.13). Forty-seven patients (43 %) had an FS. Factors associated with shorter survival were FS, bulbar onset, older age at onset, short time to diagnosis and a C9orf72 repeat expansion. The adjusted hazard ratio (HR) for the FS was 2.29 (95 % CI 1.44–3.65, p < 0.001) in a multivariate model. Patients with an FS had a shorter survival after NIV initiation (adjusted HR 2.70, 95 % CI 1.04–4.67, p = 0.04). In conclusion, there is an association between the frontotemporal syndrome and poor survival in ALS, which remains present after initiation of NIV.

Determination of the cellular profile of bronchoalveolar lavage fluid (BALF), lactate dehydrogenase (LDH) and alkaline phosphatase (ALP) appeared to be useful in monitoring pulmonary damage. The aim of this study was to investigate whether the cellular profile, LDH, its isoenzyme pattern and/or ALP in BALF are useful in the diagnostic work-up of patients with suspected pneumonia.The BALF specimens of 80 patients were studied. Group I consisted of patients with a pulmonary infection (n=33) and group II of patients without signs of a pulmonary infection (n=47). Differentiation between these two groups was based upon the results of microscopy and quantitative cultures.The absolute as well as relative numbers of polymorphonuclear neutrophils (PMNs) was significantly higher in group I compared to group II (p<0.0001). The absolute number of PMNs showed a sensitivity of predicting the correct group of 95.7% and a specificity of 84.8%. The LDH activity in BALF was significantly higher in group I than in group II (p<0.0001). The LDH 4 /LDH 5 ratio in BALF was lower in group I compared to group II (p<0.0001) and appeared to be the best discriminator between the two groups with a sensitivity of 93.6% and a specificity of 93.9%.In conclusion, the number of polymorphonuclear neutrophils as well as the lactate dehydrogenase activity, particularly its isoenzymes, in bronchoalveolar lavage fluid appeared to be of potential practical value to distinguish between infectious and noninfectious pulmonary disorders. Eur Respir J 1999; 14: 496±502.