The Netherlands journal of medicine
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We describe three patients with multifocal osteomyelitis caused by Mycobacterium avium and a family history of one or more first degree family members diagnosed with various clinical presentations of infections with nontuberculous mycobacteria. There was a significant delay in the diagnosis and they had a protracted course of their illness, which responded only slowly to prolonged multi-drug treatment. ⋯ Genomic sequencing revealed that these patients and their affected family members were heterozygous for a previously described dominant negative mutation in the gene encoding the IFN-gamma binding receptor-1 chain. The clinical presentations of the infections with nontuberculous mycobacteria in these families, with spread limited to skin, bone and lymph nodes, is discussed in the light of the immune mechanisms that are responsible for the clearance of otherwise poorly pathogenic environmental mycobacteria.
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Human immunodeficiency virus-associated nephropathy (HIVAN) is a distinct clinico-pathological syndrome that occurs almost exclusively in black patients with an AIDS defining diagnosis. It is characterized by rapidly progressive renal failure with a severe nephrotic syndrome. ⋯ The use of ACE-inhibitors and steroids may slow down the progression to end-stage renal failure. With the introduction of highly active anti-retroviral therapy, HIVAN may now be treated effectively although clinical data are so far limited to case-reports.
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Severe acute lung injury may result from many infectious and other insults. Although the initial insult may cause overwhelming tissue damage with subsequent gas exchange impairment and risk of death, several strategies of management may also add substantial toxicity. ⋯ Liberal fluid management may be another important source of toxicity. The evidence from the literature for survival advantage of lung protective and other strategies is critically reviewed.
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Case Reports
Aerosolized prostacyclin in acute respiratory distress syndrome caused by Chlamydiaceae spp. pneumonia.
Management of respiratory failure in acute respiratory distress syndrome (ARDS) typically requires ventilatory assistance. If traditional approaches to mechanical ventilation and adjunctive measures fail to succeed in achieving adequate oxygenation, alternative measures should be considered. We describe an ARDS patient with respiratory failure caused by a severe Chlamydiaceae species community-acquired pneumonia (CAP). Aerosolized prostacyclin (PGI(2)) treatment was successfully instituted for refractory hypoxemia.
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Lately renewed attention has been given to the abdominal compartment syndrome. Despite of this there still remain a lot of controversies with regard to the pathophysiological mechanisms underlying this syndrome and the therapeutic options. ⋯ The abdominal compartment syndrome is a well-recognised disease entity related to acutely increased abdominal pressure. Urgent laparotomy can be lifesaving in some cases. However no single threshold of abdominal pressure can be applied universally. Pending further clinical trials the best therapeutic option seems to be to decompress the abdomen surgically if the intravesical pressure is 25 mmHg or higher in patients with refractory hypotension, acute renal failure or respiratory failure due to abdominal distension.