Anti-MDA5 positive clinically amyopathic dermatomyositis presenting with severe cardiomyopathy.

BACKGROUND: Anti-MDA5 (Melanoma differentiation-associated gene 5) positive dermatomyositis is a new variant of clinically amyopathic dermatomyositis that presents with characteristic mucocutaneous findings and is associated with a higher risk of developing rapidly progressive interstitial lung disease. Because its presentation differs from that of classical dermatomyositis, this entity can be a diagnostic challenge for the clinician. METHODS & RESULTS: We present the case of a 55-year-old male with a 7-month history of chill sensation, constitutional symptoms and polyarthralgia. Within 3 months, the patient developed progressive heart failure with dyspnoea and orthopnoea, together with characteristic cutaneous lesions. Skin biopsies demonstrated thrombosis of small and medium-sized arteries in the reticular dermis, together with an evolved lobular panniculitis and prominent mucin deposits. CONCLUSIONS: Clinicians should be aware of the characteristic clinical and histopathologic presentation of this variant of dermatomyositis to establish an early diagnosis. Further evidence is needed to clarify the risk of cardiac involvement in this subset of patients.

Statin-associated autoimmune myopathy: A distinct new IFL pattern can increase the rate of HMGCR antibody detection by clinical laboratories.

BACKGROUND AND OBJECTIVE: Statin-associated autoimmune myopathy (SAAM) with anti-HMGCR antibodies has recently been described. Several specific immunoassays are in use to detect HMGCR antibodies. In the course of systematic autoantibody screening we recognized a new distinct IFL staining pattern on rat liver sections that regularly coincided with anti-HMGCR antibodies. In this study we investigated whether this new IFL pattern is specifically associated to statin-associated autoimmune myopathy and corresponds to anti-HMGCR antibodies. PATIENTS AND METHODS: Twenty-three patients positive for anti-HMGCR antibodies (14 diagnosed with SAAM) were investigated for anti-HMGCR antibodies by two ELISA assays and confirmed by immmunoblot. HMGCR associated liver IFL pattern (HALIP) was detected by indirect IFL and the reactivity against HMGCR was confirmed by immunoabsorption using purified human HMGCR antigen. 90 patients with other autoimmune diseases and 45 non-autoimmune statin treated patients were studied as controls. RESULTS: 21 out of 23 (91%) anti-HMGCR positive patients were HALIP positive. The staining was completely and specifically removed by immunoabsorption with human purified HMGCR. None of the control sera from autoimmune patients or non-autoimmune statin treated subjects was positive for HALIP. Statistical concordance between HALIP and anti-HMGCR antibody specific tests was 98.7%, kappa 0.95. CONCLUSIONS: A new and distinct IFL staining pattern (HALIP) is associated to HMGCR associated myopathy. Absorption and concordance studies indicate that the antigen recognized in the liver by HALIP is HMGCR or a closely related protein. Awareness of this new pattern can help to detect HMGCR autoantibodies in statin treated patients tested for autoimmune serology.

Seasonal variations in the regiodistribution of oil extracted from small-spotted catshark and bogue.

The aim of this work was to seasonally characterize the nutritional quality of oil extracted from small-spotted catshark (Scyliorhinus canicula) and bogue (Boops boops). The proximate composition, lipid profile and regiodistribution of the fatty acid in the glycerol backbone were analyzed. In addition, three nutritional indexes were calculated (atherogenicity and thrombogenicity indexes and the hypocholesterolaemic/hypercholesterolaemic ratio). Both species presented PUFA as the predominant fraction, the most abundant being DHA. Healthy values of the aforementioned indexes were maintained throughout the year. Moreover, the relative composition of omega 3 fatty acids at the sn-2 position ranged from 47.3 to 66.8 mol%, attracting interest in the employment of these oils as the raw source for the production of 2-monoacylglycerols. Regarding the individual behavior of each fatty acid, DHA presented a high tendency to occupy the sn-2 bond, whereas EPA presented the opposite behavior.

Radical or partial maxillary sinus surgery: a dilemma today? An experimental study.

To evaluate partial or radical surgical removal of the maxillary sinus mucosa, 20 New-Zealand albino rabbits were used. After three months, specimens were taken for examination. Bacteriological cultures, light- and electron microscopy were performed, and mucociliary transport was studied. These experimental findings add further support to the concept of performing a conservative sinus procedure rather than a radical removal as a first procedure.

Submucosal glands after maxillary sinus surgery. An experimental study in rabbits.

Thirty New Zealand White rabbits underwent unilateral partial or complete removal of maxillary sinus mucosa in order to evaluate submucosal maxillary sinus glands. After three months, specimens were taken for examination from all operated on and control sinuses. Bacteriological cultures, light and electron microscopy were performed. Histopathological findings showed a decrease in the number of serous glands and significant inflammation was present in the sinus in which there was complete surgical removal. Electron microscopy revealed changes in the secretory cells of the serous glands in the regenerated post-surgical mucosa.

[Pleomorphic adenoma as a parapharyngeal tumor. The role of the CT in differential diagnosis]. / El adenoma pleomorfo como tumor parafaríngeo. Papel de la TAC en el diagnóstico diferencial.

We described 2 cases of pleomorphic adenoma as parapharyngeal masses, one of the deep lobule of the parotid gland and the other one in palatine accessory salivary glands. After computed tomography (CAT) we made the anatomopathological diagnosis of pleomorphic adenoma with fine needle aspiration. We discuss about the role of CAT in diagnosis of pleomorphic adenoma occupying the parapharyngeal space.

[Subepithelial glands in the hamster's larynx]. / Estudio de las glándulas subepiteliales en la laringe del hamster.

We have performed, with light microscope, a study about the distribution and morphology of the subepithelial glands of the hamster's larynx. The results are: at the base of the tongue the great deal of the glands are mucous, at the epiglottic base seromucous and at the subglottic space (at the level of cricoid cartilage) serous.

[Histological study of the rat's nasal sinus cavity. Its use in investigation]. / Estudio histológico de la cavidad nasosinusal de la rata. Su utilización en investigación.

An histologic study was performed by light microscopy of the rat nasal cavity. A neuroepithelial lining is found in the backside of the cavity and the medial portion of vomeronasal organ. A respiratory seudostratified epithel with a great deal of submucous glandulae cover the remainder of the cavity.

[Complete regression of a Warthin's tumor following the aspiration cytopuncture]. / Regresión total de un tumor de Warthin tras citopunciòn aspirativa.

This paper reports a rare case of a 69-year-old man bearing a right parotid lump, measuring near 5 cm diameter, which diagnosis resulted a Warthin's tumor. The used procedure to reach the diagnosis was aspiration through a fine needle. Two months after the laboratory diagnosis--so within the preoperative term--we can corroborate the clinical and radiological missing of the growth. Review and analysis of etiologic and pathogenic mechanisms.

[The hamster's larynx as an experimental model]. / La laringe del hamster como modelo experimental.

A macroscopic study and with light microscopy as well is performed in the larynx of 4 hamsters. Morphology of hamster's larynx is described. Transitional epithelium is found out on the epiglottis base and caudally to the vocal cords. Hamster's larynx may be a good model for experimental study of changes during carcinogenesis.

[Cavernous hemangioma of the larynx in an adult. Endolaryngeal excision]. / Hemangioma cavernoso de laringe en el adulto. Exéresis por vía endolaríngea.

We present a case of cavernous laryngeal hemangioma in an elderly lady. The limited surface of the lesion and its location and implantation basis as well allowed an endolaryngeal surgery.

[Eosinophilic ulcers of the tongue as an adverse reaction to carbamazepine]. / Ulcera eosinofílica de la lengua como reacción adversa a la carbamazepina.

We describe a typical case of eosinophilic ulcer of the oral mucosa (EUOM) after an adverse reaction to carbamazepine, in a manic-depressive patient. This case may be suggestive of other etiologic factors for the EUOM, different to traumas.

[Differences between excretory ducts of normal submucous glands and those of nasal polyps. A histochemical study with lectins]. / Diferencias entre los conductos excretores de las glándulas submucosas de la mucosa normal y la de los pólipos nasales. Estudio histoquímico con lectinas.

We have performed an histochemical study with lectins of normal nasal mucosa in 6 patients admitted at Hospital because an abdominal pathology, and other 6 with nasal polyps. Results showed a similar reactivity to lectins of excretory duct cells of seromucous glandulae of basement membrane of normal nasal mucosa and the analogous cells of the nasal polyps mucosa.

[Lectin-mediated histochemical study of submucous glands of the normal nasal mucosa]. / Estudio histoquímico mediante lectinas de las glándulas submucosas de la mucosa nasal normal.

We have performed an histochemical study with lectins of the normal nasal mucosa of 6 patients. These patients were admitted at the Hospital for abdominal surgery. A wide biopsy was taken of the inferior nasal turbinate mucosa. The results show a different reactivity between the serous and mucous cells of the submucosal glands and with the goblet cells. We have not found reactivity to DBA, SBA and LTA lectins.

[Secondary hearing impairment due to Paget's disease]. / Hipoacusia secundaria a enfermedad de Paget.

Paget's disease presents frequently in skull bones with incidence in temporal bone, causing vertigo, hearing loss and tinnitus. The AA. present one case in a woman, 60-year-old, diagnosed as Paget's disease, complaining of left ear deafness and hearing impairment of the right ear resulting from a conductive disorder.

[The maxillary sinus of the rabbit: an experimental model]. / El seno maxilar del conejo: un modelo experimental.

The present work describes the anatomy of the rabbit nasal cavity and maxillary sinus. Studies of mucociliary clearance in the maxillary sinus and of the sinus mucosa by light and electronic microscopy are also carried out.

[Lower antrostomy in surgery of the maxillary sinus: experimental study]. / La antrostomía inferior en la cirugía del seno maxilar: estudio experimental.

Inferior nasoantral windows, with or without radical surgical removal of the maxillary sinus mucosa, were evaluated in 15 New Zealand white rabbits. After three months, specimens were obtained for examination. Bacteriological cultures and light and electron microscopic studies were made. Mucociliary clearance was studied. Differences were found in the antrostomy patency rate between radical antrostomy and inferior nasoantral windows. After three months, the creation of a temporary inferior nasoantral window did not change the maxillary sinus mucosa and did not increase the rate of sinus infections in rabbits.