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Severe Respiratory Insufficiency (severe + respiratory_insufficiency)

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Medical Sciences	100%

Selected Abstracts

Chronic inflammatory demyelinating polyneuropathy, phrenic nerve and respiratory symptoms

EUROPEAN JOURNAL OF NEUROLOGY, Issue 1 2005
J. Costa
Respiratory involvement in chronic inflammatory demyelinating polyneuropathy (CIDP) has been very recently described. Phrenic nerve conduction studies have been described as useful to detect respiratory impairment in these patients. This study describes two patients with CIDP, in whom neurophysiological studies of the respiratory muscles were performed. The first patient had severe respiratory insufficiency, and phrenic nerve studies disclosed no motor responses and electromyography (EMG) of the diaphragm confirmed severe loss of motor units, bilaterally. On treatment, we documented clinical and neurophysiological improvement. In the second patient, phrenic nerve studies showed abnormal results; however, EMG of the diaphragm ruled out loss of motor units. The first case represents the risk of phrenic nerve involvement in this disorder, and the potential recovery on treatment. The second case illustrates that the temporal dispersion of the motor responses can be misleading, and EMG of diaphragm should be performed to confirm the loss of motor units. [source]

Pulmonary embolism in a patient with severe congenital deficiency for factor V during treatment with fresh frozen plasma

HAEMOPHILIA, Issue 3 2005
A. García-Noblejas
Summary., Thrombosis is a rare complication in patients with congenital clotting factor deficiencies. In most cases, it is related to inherited procoagulant factors, use of central venous catheters or administration of coagulation factor concentrates. There are only a few case reports about thrombotic events during treatment with fresh frozen plasma (FFP). We report the case of a patient with homozygous inherited factor V deficiency, who developed a pulmonary embolism at a time of treatment with methylene blue treated FFP (MBFFP). The patient had only two other factors predisposing to thrombosis and both were acquired: obesity and bed rest. He started anticoagulant treatment with low molecular weight heparin (LMWH) while the deficient factors were replaced with MBFFP. After 8 days of treatment the patient developed a severe respiratory insufficiency. Pulmonary haemorrhage was considered among the differential diagnosis and LMWH was stopped. An inferior vena cava filter was placed without any further thrombotic complications. To our knowledge, there are no reports about patients with clotting factor deficiencies who developed a thrombotic event during treatment with MBFFP. [source]

Oral dantrolene and severe respiratory failure in a patient with chronic spinal cord injury

ANAESTHESIA, Issue 8 2010
M. Javed
Summary Oral dantrolene is used widely for the treatment of spasticity in patients with spinal cord injury. A 60-year-old patient in the rehabilitation phase following cervical spine injury presented with generalised weakness and deteriorating respiratory function, requiring intensive care admission, tracheal intubation and ventilation. He had bilateral basal lung collapse and a raised diaphragm and was on high-dose oral dantrolene. The cessation of dantrolene resulted in a dramatic recovery of respiratory function within two days. High-dose oral dantrolene can cause severe respiratory insufficiency and may present difficulties in the differential diagnosis of respiratory failure in patients with high cervical spinal cord injuries. [source]

Mutation in BAG3 causes severe dominant childhood muscular dystrophy,

ANNALS OF NEUROLOGY, Issue 1 2009
Duygu Selcen MD
Objective Myofibrillar myopathies (MFMs) are morphologically distinct but genetically heterogeneous muscular dystrophies in which disintegration of Z disks and then of myofibrils is followed by ectopic accumulation of multiple proteins. Cardiomyopathy, neuropathy, and dominant inheritance are frequent associated features. Mutations in ,B-crystallin, desmin, myotilin, Zasp, or filamin-C can cause MFMs and were detected in 32 of 85 patients of the Mayo MFM cohort. Bag3, another Z-disk,associated protein, has antiapoptotic properties, and its targeted deletion in mice causes fulminant myopathy with early lethality. We therefore searched for mutations in BAG3 in 53 unrelated MFM patients. Methods We searched for mutations in BAG3 by direct sequencing. We analyzed structural changes in muscle by histochemistry, immunocytochemistry, and electron microscopy, examined mobility of the mutant Bag3 by nondenaturing electrophoresis, and searched for abnormal aggregation of the mutant protein in COS-7 (SV-40 transformed monkey kidney fibroblast-7) cells. Results We identified a heterozygous p.Pro209Leu mutation in three patients. All presented in childhood, had progressive limb and axial muscle weakness, and experienced development of cardiomyopathy and severe respiratory insufficiency in their teens; two had rigid spines, and one a peripheral neuropathy. Electron microscopy showed disintegration of Z disks, extensive accumulation of granular debris and larger inclusions, and apoptosis of 8% of the nuclei. On nondenaturing electrophoresis of muscle extracts, the Bag3 complex migrated faster in patient than control extracts, and expression of FLAG-labeled mutant and wild-type Bag3 in COS cells showed abnormal aggregation of the mutant protein. Interpretation We conclude mutation in Bag3 defines a novel severe autosomal dominant childhood muscular dystrophy. Ann Neurol 2008 [source]