Specific heart muscle disorders

Systemic immune-mediated diseases are autoimmune and autoinflammatory diseases affecting at least two-organ systems. Autoinflammatory diseases are a family of conditions characterized by episodes of unprovoked inflammation in the absence of high autoantibody titres or autoreactive T lymphocytes, reflecting a primary dysfunction of the innate immune system. Autoimmune diseases are characterized by aberrant B, T, and dendritic cell responses with predominantly cell-mediated or autoantibody-mediated responses to self-antigens in genetically susceptible individuals. Cardiovascular involvement is systemic immune-mediated diseases may be occult and often goes undetected, but is associated with a poor prognosis. As any anatomical structure in the heart may be involved, patients can present with one or more features consistent with pericarditis, myocarditis, endocarditis, and vasculitis. There is often no correlation between the extent of systemic disease and cardiac involvement.

Do We Live In A Universe Where There Are Reliable Experiments About Conscious Experience?

A primary dysfunction in the current field of consciousness science is its lack of examination of its own methodological foundations, from a physical perspective. Mainstream approaches currently make two contradictory assumptions: (1) a standard-physics setting; and (2) the existence of reliable experiments about conscious experience, based on report. The key question for consciousness science is whether we *do* live in a Universe where reliable experiments about conscious experience exist - which would contradict standard physics. Fortunately the kind of Universe we live in is itself subject to relatively-straightforward experimental test! [Chapter 4 from 'The Science We Need - One Experiment to Change the World'.]

Etiopathogenesis of internal and external genital endometriosis (evolution of views)

The etiology and pathogenesis of endometriosis and adenomyosis, as well as their interrelations, are not fully understood and are the subject of discussions and research. The question remains whether the endometriosis and adenomyosis are different diseases, or they represent different phenotypes of the same pathology. Considerations are given about the etiopathogenesis of these diseases, which existed in different years. It is now recognized that endometrioid heterotopias in adenomyosis and endometriosis are derivatives of the basal layer of the endometrium - endometrial stem cell. There are three possible ways of spreading the stem cells of the basal layer of the endometrium, leading to the formation of foci of endometriosis and adenomyosis: retrograde cast into the abdominal cavity with menstrual blood, invasive growth in myometrium due to the mechanism of epithelial-mesenchymal transformation and disruption of the endometrium and myometrium connection zone, and neonatal bleeding. Thus, the mechanisms of the appearance of foci of endometriosis in various organs and tissues are quite diverse and are associated with the primary dysfunction of the endometrium itself, retrograde menstruation, pathological regeneration accompanied by epithelial-mesenchymal transformation, as well as pathological changes in the endometrium and myometrium connection zone, neonatal bleeding. The severity of the clinical course of endometriosis, the frequency of relapse after surgical treatment, the effectiveness of conservative therapy, and the overcoming of infertility are probably due to the mechanism of development of this pathology in each specific case.

Measurement of bone mineral density in stable COPD patients using ultrasound densitometry: prevention is better than cure

Background: COPD is a multisystem disorder and three or more comorbidities are associated with poor prognosis. Osteoporosis is one of the major comorbidity and is associated with an impact on the primary dysfunction in COPD. Bone Mineral density measures strength of the skeletal system and ultrasound based BMD measurement is a cheap, cost effective intervention which is also free from ionizing radiation. Ultrasound densitometer BMD measurement can be utilized for screening of osteoporosis in COPD patients. Methods: 50 stable COPD (diagnosed as per GOLD guideline) patients attending a post graduate institute in Mumbai were enrolled for the study and bone mineral density was measured using ultrasonography BMD scan of calcaneum. Results: Among 50 enrolled COPD patients, 27 (54%) patients had osteopenia and 14 (28%) were osteoporotic.9 (64.3%) female and 18 (50%) male COPD patients had osteopenia. 4 (28.6%) females and 10 (27.8%) males were osteoporotic. Conclusions: Considering the additional burden of osteoporosis on management of COPD, it is advisable to measure BMD to formulate strategies to prevent fractures in COPD patients.

A rare case of dysphonia in mitochondrial myopathy

<p class="abstract"><span lang="EN-US">The mitochondrial myopathy consists of diverse group of disorders which is characterized by primary dysfunction of mitochondrial respiratory chain leading to muscle disease. The involvement of larynx is very rare and only few cases have been reported in the literature. This study presents the fourth published case of dysphonia in the setting of mitochondrial myopathy. A patient with dysphonia with laryngeal involvement in mitochondrial myopathy is presented with literature review. A 43 year old man presented with progressive dysphonia and muscle weakness. Laryngeal examination showed bilateral adductor weakness of vocal cords and biopsy revealed findings typical of mitochondrial myopathy (MM). He underwent conservative trial for dysphonia with no relief and subsequently medalization thyroplasty showed some improvement in dysphonia. </span>Mitochondrial myopathy should be considered in the differential diagnosis of dysphonia for early diagnosis and management.</p><p class="p"> </p>

HEPATIC STEATOSIS ASSESSMENT:

ContextLiver transplantation is one of the last viable resources for patients with end-stage liver disease. Many strategies are been used to improve the number of available organs and overcome waiting list delay. However, hepatic steatosis is one of the mainly concerns when organs are consider to transplantation due to it is importance as a risk factor for primary dysfunction. Surgeons play an important role to decide each organ will be accept or decline and its righteous allocation.ObjectiveRetrospectively evaluate the surgeon assessment of steatosis degree and its confrontation with further histopathologic findings.MethodsWe analyzed 117 patients underwent deceased liver transplantation for end-stage liver disease in University Hospital Walter Cantideo, Fortaleza, CE, Brazil. A matrix table was organized to estimate the categorical data observed. We clustered the subjects into mild (0%–30%) and moderate (30%-60%) steatosis degree under the clinical criteria of organ suitability for transplantation. We categorized the organs as suitable organ for transplant and as non-suitable organ for transplant. Evaluations between the two first assessments, before perfusion (pre-perfusion) vs biopsy findings and after perfusion vs biopsy findings observations were analyzed and also a comparison between pre-perfusion and after perfusion data was performed.ResultsOn the first assessment, we obtained a 93% of agreement (n = 109) between the two evaluations. On the second assessment, we had an 8% (n = 9) of mistaken allocation. Comparing the observation before (pre-perfusion) and after (after perfusion), we obtained a strong agreement between the surgeons.ConclusionsAlthough our experienced surgeon team, we have wrongly evaluated feasible organs for transplantation. Nonetheless, our faulty percentage is low comparing to worldwide percentage.

Large vestibular aqueduct syndrome and endolymphatic hydrops: two presentations of a common primary inner-ear dysfunction?

Objective:To present the theory that large vestibular aqueduct syndrome (i.e. the recognised existence of an enlarged vestibular aqueduct with progressive sensorineural hearing loss) and endolymphatic hydrops are due to a common primary dysfunction of inner-ear fluid homeostasis.Method:Case report and review of the world literature concerning large vestibular aqueduct syndrome and endolymphatic hydrops.Results:We report a family in which one sibling suffered from large vestibular aqueduct syndrome while the other had classic Ménière's disease. This suggests that large vestibular aqueduct syndrome and endolymphatic hydrops, in some cases, may be due to a common primary dysfunction of inner-ear fluid homeostasis.Conclusion:To our knowledge, this is the first report in the world literature to postulate that variation in the relative compliance of inner-ear membranes could be the factor that determines the manifestation of the disorder as either endolymphatic hydrops or large vestibular aqueduct syndrome.