Nasopharyngeal angiofibroma in an adult with Proteus syndrome. First reported case.

BACKGROUND: Proteus Syndrome (PS) is a rare hamartoneoplastic disorder consisting of a disproportionate and asymmetric overgrowth of body parts, leading to severe body and face disfigurement. Individuals with PS frequently need to undergo a number of surgical procedures including Otolaryngology interventions. Their peculiar anatomy turns even the most straightforward intervention to a challenging one. CASE DESCRIPTION: A 31-year-old adult male with PS presented with recurrent epistaxis and nasal obstruction. A nasal mass was found during the physical examination, which was surgically removed under general anesthesia. Histology revealed juvenile angiofibroma. CONCLUSION: This is the first reported case of a patient with PS presenting with juvenile nasopharyngeal angiofibroma. Although vascular malformations may appear in these patients, juvenile nasopharyngeal angiofibroma should be taken under consideration when PS patients present with recurrent epistaxis. HIPPOKRATIA 2017, 21(3): 147-149.

Ultrasound-guided transoral drainage of a paediatric parapharyngeal abscess.

OBJECTIVE: To report and discuss the surgical use of ultrasonography for draining a parapharyngeal space infection in a child. CASE REPORT: The use of image-guided surgery for draining a parapharyngeal abscess has been previously reported, with computed tomography for an intra-oral approach and ultrasound for an external approach. We present the first case report of a four-year-old child with a retropharyngeal abscess and a deep parapharyngeal abscess in whom neck ultrasound was used to assist intra-oral drainage. CONCLUSION: Neck ultrasound may be used in paediatric patients to visualise access to the parapharyngeal space through the intra-oral route for abscess drainage.

Multifocal bigerminal mature teratomas of the head and neck.

Teratomas and dermoids or hairy polyps of the head and neck are rare congenital tumours, often confused and reported to appear as single masses, that may cause upper aerodigestive tract impairment. Their histological classification, origin and embryological basis of development have been a topic of debate in the past and recent literature. We report a case of an infant presenting with a multifocal bigerminal teratoma in the nasopharynx and the neck, causing a deep neck infection, and discuss the unique clinicopathological aspects of this case.

Correlation of histoenzymological studies with the response to chemotherapy and survival in breast cancer patients.

The enzyme activity of cancerous tissue and the adjacent normal epithelium of 40 patients with breast cancer was determined. This enzymatic activity was correlated with the responsiveness of those tumors to the chemotherapy. It was found that the presence of cytochrome oxidase and alkaline phosphatase and the absence of leucine aminopeptidase, beta-glucuronidase and dehydrogenases in cancerous tissues was related to good response. On the contrary, the absence of alkaline phosphatase and cytochrome oxidase and the presence of leucine aminopeptidase, beta-glucuronidase and dehydrogenases in the cancerous tissues was related to poor response and therefore to poor survival.

Anomalous behavior of glutathione reductase on dilution.

Serum glutathione reductase activity decreases on standing at room temperature. Dilution of the sample slows this decay in sera from patients with a variety of clinical disorders, and reverses the inactivation in sera from patients with liver or biliary tract disease. Prolonged contact with dithioeythritol or acetate buffer, pH 6.4, at 4 degrees C prevents spontaneous inactivation of the native serum and abolishes the effect of dilution. These hitherto unreported phenomena are a source of potential inaccuracy in assays for this enzyme. The inactivation may be the result of conformational folding with "burying" of active sites, or of molecular aggregation brought about by hydrogen bonding or disulfide bond formation.

An optimized semi-automatic rate method for serum glutathione reductase activity and its application to patients with malignant disease.

An improved and optimized method for serum glutathione reductase is described. The reference range for normal subjects is 47-79 IU/1. The method is more sensitive than conventional enzyme tests in the detection of malignant disease. It was not raised more frequently in patients with clinical evidence of metastases than in those clinically free of such metastases, and it did not seem to correlate with prognosis among those patients who failed to survive six months from the time the analysis was first conducted.