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Signa Vitae

Journal of Intensive Care and Emergency Medicine

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Pulmonary hypertension leading to right heart failure in a patient with IgA gammapathy

Abstract

Amyloidosis is a rare disorder characterized by the deposition of amorphous, extracellular, insoluble fibrillar protein in various tissues of the body. Pulmonary hypertension usually occurs in the last stages of the disease with co-existing left ventricular failure. Amyloidosis causing pulmonary hypertension in a patient with no evidence of left ventricular failure is rarely mentioned in literature. Here, we present a patient with IgA gammopathy presenting with pulmonary hypertension leading to progressive right heart failure and death.

Key words: amyloidosis, pulmonary hypertension, IgA gammopathy

 

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Severe barium sulphate aspiration: a report of two cases and review of the literature

Abstract

Aspiration of barium sulphate is a well-recognized complication, occurring accidentally during examinations of the upper gastrointestinal system using contrast media. Rarely, large amounts of barium sulphate are inadvertently aspirated into the lung. Certain conditions affecting the anatomical and functional integrity of the oropharynx and oesophagus suggest predisposing factors. Aspiration of barium sulphate is not expected to cause severe lung injury due to its relatively non-irritant matter. On the other hand, acute inflammation or even death attributed either to high or low density preparations of barium sulphate, have been reported.
We present two patients, both with a history of schizophrenia, who developed acute respiratory failure requiring mechanical ventilation, following aspiration of large amounts of barium, during an upper gastrointestinal radiographic contrast study. One patient died following massive aspiration which led to multiple organ dysfunction syndrome, while the other, although sub- acutely complicated by pneumonia, was successfully treated.
Beside presentation of cases, several aspects of investigation, differential diagnosis, treatment and prevention are discussed. Complications of barium sulphate aspiration depend upon the density and quantity of the aspirated solution, the extent of tracheobronchial distribution and the general physical condition of the patient. In severe cases, early treatment and close follow up with high-resolution computed tomography are mandatory to prevent progression towards fibrosis. Patients with psychiatric disorders, apart from other conditions predisposing to aspiration, should be dealt with particular caution, when performing the above-mentioned procedures.

Key words: barium, barium sulphate, aspiration pneumonia, lung CT, lung scintigraphy

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Debilitating Darier’s disease

Abstract

Darier’s disease is an inherited dermatitis that is due to a mutation in the ATP2A2 gene. This mutation causes disrupted signaling within cells and leads to a skin condition that is resistant to many dermatologic treatments. Though serious complications are rare, Darier’s disease can result in bacterial sepsis and Kaposi’s varicelliform eruption. In some families seizure disorders are linked to Darier’s disease. There is also a paraneoplastic variant that could be considered in patients without a family history of Darier’s disease. This article reports a case with typical verrucous masses and waxy papules which, over twenty years, became disabling. Symptoms included pruritus and foul odor and were relieved, along with the keratotic rash, utilizing systemic treatment with acitretin though the condition recurred rapidly when treatment was interrupted. Darier’s disease must be considered when patients present with hyperkeratotic lesions that are resistant to usual forms of therapy for inflammatory dermatoses.

Key words: Darier’s Disease, ace-tretin, inherited dermatoses.

 

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