Debilitating Darier's disease (Views : 12421 times)
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.
Erythropoietin in post-resuscitation neurological recovery: is there light at the end of the tunnel? (Views : 5305 times)
Studies show that erythropoietin, besides its critical role in hematopoiesis, provides neuroprotection in hypoxic-ischemic cerebral injury. Antiapoptotic, anti-inflammatory, angiogenetic, and neurotrophic properties of erythropoietin could increase indications, currently restricted to anemia in chronic renal failure and cancer, to hypoxic-ischemic cerebral insult.
In the adult and neonatal animal model of hypoxic-ischemic cerebral injury, erythropoietin significantly reduces infarct size with attenuation of brain damage, and preservation of cortical integrity. The first human study on the impact of erythropoietin in stroke victims showed that erythropoietin is safe and well tolerated at high doses, and associated with improved neurological outcome. Even with intravenous application, concentrations of erythropoietin in cerebrospinal fluid of these patients were many-fold higher than in non-treated patients.
In successfully resuscitated cardiac arrest victims overall neurological recovery remains poor despite improved cardiopulmonary resuscitation strategies. Post-resuscitation care needs further advances in order to improve final outcome. Through promotion of neuroangiogenesis, inhibition of hypoxia-induced apoptosis in neurons, and thus support of the survival of neurons in the ischemic brain, erythropoietin could be used to improve functional recovery of these patients. Nevertheless, optimal molecular forms of EPO, therapeutic doses, and treatment time window have to be determined in order to lower the incidence of adverse effects and still preserve neuroprotective properties.
Key words: cardiopulmonary resuscitation, erythropoietin, post-resuscitation period, hypoxia.
First measured intrathoracic blood volume in icu patients indicates the appropriateness of circulatory volume management (Views : 6686 times)
Hemodynamic monitoring in Intensive Care Unit (ICU) settings is usually introduced when a patient becomes hemodynamically unstable. We analyzed how empirically guided volume management relates to first measured intrathoracic blood volume (ITBV), at the moment of the beginning of Puls Contour Cardiac Output (PiCCO) hemodynamic monitoring.
Data and measurements from 37 ICU patients, divided into four groups according to diagnosis of primary condition, were retrospectively studied. The first group consisted of polytrauma patients, second group of patients with pancreatitis and/or peritonitis, third group were postoperative patients, and fourth group were patients with various medical diagnosis: sepsis, acute respiratory distress syndrome (ARDS), acute lung failure (ALF), and acute heart failure (AHF). PiCCO monitor was introduced when the signs of hemodynamic instability were observed. First measured ITBV was recorded and analyzed according to deviation from reference values.
First measured ITBV was in reference range in 14 (37.8%) patients. Volume overloading was observed in 16 (43.2%) and hypovolemia in 7 (18.9%) patients.
The observed inappropriate blood volume in patients of all studied groups suggests that there is the need for defining indications and earlier application of hemodynamic monitoring, as well as reassessment of usual empirically guided infusion therapy in ICU setting.
Key words: hemodynamic process, intensive care, hypovolemia, monitoring
Induced hypothermia after cardiopulmonary resuscitation: possible adverse effects (Views : 14891 times)
The last several years have seen an increased interest in the use of induced hypothermia after witnessed cardiopulmonary resuscitation (CPR). The main reason for its use is protection of the brain and hence, better neurological outcome in these patients. Therefore, induced hypothermia after CPR has become a part of standard recommendations in the 2005 Resuscitation Guidelines. At the same time, hypothermia can have many adverse effects. In the event of pre-hospital and/or in-hospital induction of hypothermia, without adequate monitoring and controlled cooling, hypothermia can cause serious complications, without beneficial effects on the brain. This article explains the most frequent adverse effects of hypothermia and possible hazardous outcomes for patients.
Key words: cardiopulmonary resuscitation, hypothermia, hemodynamics
Pulmonary hypertension leading to right heart failure in a patient with IgA gammapathy (Views : 20582 times)
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