External laryngeal nerve landmarks revisited.

BACKGROUND: Because external laryngeal nerve (ELN) iatrogenic damage is frequent during neck surgery, its precise localization has been highly recommended. This study analyzes the different surgical landmarks previously proposed and the anatomy of the collateral and terminal branches of the ELN. METHODS: The necks of 157 (77 men and 80 women) human adult embalmed cadavers were examined. The ELN origin, length, and relationship to different landmarks were recorded and results statistically compared with those previously reported. RESULTS: The ELN is located deep to the ascending pharyngeal vein in 100% of patients. In most patients, it crosses the carotid axis at the thyroid artery origin level (47% of patients), passes medial to it (89% of patients), and shows an intramuscular trajectory through the inferior constrictor of the pharynx (80% of patients). CONCLUSION: The ELN position, in relation to classical landmarks, is highly variable. The most reliable relationships are those with the ascending pharyngeal vein or with the superior thyroid artery.

Antiadenohypophysis autoantibodies in patients with nongluten-related gastroenteropathies.

AIM: To investigate the presence of antipituitary antibodies (APA) in the serum of patients undergoing gastroenteropathies (GEP) other than celiac disease (CD). METHODS: APA were analyzed in GEP patients (n = 103), CD patients (n = 94), idiopathic growth hormone (GH) deficiency patients (n = 21), and 98 age- and sex-matched controls. Indirect immunofluorescence was performed in cryostat sections of baboon pituitary gland. RESULTS: APA were detected in 30% of GEP patients, 38% of them showed failure to thrive. No significant differences were found when we compared thrive impairment in negative and positive APA GEP patients. Indeed, normal values of insulin-like growth factor 1 were found in 93% of positive APA GEP patients. APA were detected in 52% of the CD patients. Ninety-one percent of them, in contrast to GEP group, showed significant growth impairment (P < 0.05) when compared to APA negative CD individuals. GH-deficient non-CD patients did not show APA. CONCLUSIONS: We have shown the presence of APA in patients with nongluten-related enteropathies. The presence of antipituitary autoantibodies in GEP patients does not seem to be associated with failure to thrive as it occurs in CD.

Interleukin-21 overexpression dominates T cell response to Epstein-Barr virus in a fatal case of X-linked lymphoproliferative syndrome type 1.

Interleukin-21 (IL-21) is a cytokine whose actions are closely related to B cell differentiation into plasma cells as well as to CD8(+) cytolytic T cell effector and memory generation, influencing the T lymphocyte response to different viruses. X-linked lymphoproliferative syndrome type 1 (XLP-1) is a primary immunodeficiency syndrome that is characterized by a high susceptibility to Epstein-Barr virus. We observed in a pediatric patient with XLP-1 that IL-21 was expressed in nearly all peripheral blood CD4(+) and CD8(+) T cells. However, IL-21 could not be found in the lymph nodes, suggesting massive mobilization of activated cells toward the infection's target organs, where IL-21-producing cells were detected, resulting in large areas of tissue damage.

A novel phenotype variant of severe congenital neutropenia caused by G6PC3 deficiency.

Severe congenital neutropenia type 4 (SCN4) is associated with mutations in the G6PC3 gene. To date, all patients bearing the p.Gly260Arg variant of the G6PC3 gene show heart defects. Here, we present a case of the p.Gly260Arg variant in a patient who did not have structural or functional heart anomalies. Treatment with granulocyte colony-stimulating factor recovered the absolute neutrophil count and neutrophil functional competence.

IL-17-producing CD8+ T lymphocytes from psoriasis skin plaques are cytotoxic effector cells that secrete Th17-related cytokines.

IL-17-producing CD4+ T lymphocytes (Th17) are currently considered relevant participants in the pathogenesis of psoriasis skin lesions. However, little is known about the potential role of IL-17-producing CD8+ T cells, which are also present at the psoriatic plaque. We have addressed the functional characterization of this CD8+ subtype of T lymphocytes from psoriasis patients. Our results show that CD8+IL-17+ cells from psoriasis-inflamed skin tissue produce TNF-alpha and IFN-gamma (Th1-related cytokines) as well as IL-17, IL-21, and IL-22 (Th17-related cytokines) efficiently. A significant up-regulation of the RORC transcription factor is also observed. These cells are refractory to Tregs but show a proliferative response to anti-CD3/CD28 stimulation that is enhanced by IL-12 and IL-15. Blocking of TNF-alpha activity inhibits TCR-mediated activation and IL-17 production. CD8+IL-17+ T cells are cytotoxic cells that display TCR/CD3-mediated cytotoxic abilities to kill target cells. Thus, CD8+IL-17+ T cells share some key features with Th17 cells and exhibit remarkable differential abilities attributable to the CD8+ lineage of T lymphocytes, adding new insights into the functional resources of IL-17-producing cells from human epidermis that could be of potential interest to our understanding of the pathogenesis of psoriasis.

[Total idiopathic laryngotracheal stenosis. Treatment with mitomycin C]. / Obstrucción laringotraqueal completa idiopática. Tratamiento con mitomicina C.

This case report involves a 69-year-old woman who presented idiopathic laryngotracheal stenosis with total obstruction of the airway. Seven surgical procedures involving open field and endoscopy techniques had to be performed due to repeated re-obstructions. The novelty in this case is that success was finally obtained with the use of mitomycin C. The drug action is well known, but is interesting to report a case in which success was only obtained when we used mitomycin C.