Mucocele de las glándulas Blandin-Nuhn, aspectos patológicos y terapéuticos, a propósito de dos casos clínicos / Mucocele of the glands of Blandin-Nuhn

Las glándulas de Blandin-Nuhn son un grupo de pequeñas glándulas salivales mucoserosas, ubicadas en el espesor de los haces musculares de la zona anterior de la cara ventral de la lengua. Los mucoceles de las glándulas de Blandin-Nuhn son poco frecuentes. Se revisa la literatura y se presentan dos casos de mucocele de las glándulas de Blandin-Nuhn, ambos casos eran lesiones exofíticas, indoloras, una de ellas asociada a trauma. Los mucoceles de las glándulas salivales linguales anteriores deben ser resecados hasta el plano muscular para evitar la recidiva. Como esta lesión puede ser confundida con otras patologías, se recomienda su estudio histopatológico para evitar diagnósticos errados.

The Blandin-Nuhn glands are a small group of mucous serous salivary glands, located in the thickness of the muscle bundles of the anterior ventral side of the tongue. Mucocele of the glands of Blandin-Nuhn has been reported as unusual. We review the literature and present two cases of mucocele of the glands of Blandin-Nuhn, both cases were exophytic lesions, painless, one associated with trauma. Mucoceles of the salivary glands should be resected up to the muscular plane to avoid recurrence. Because this lesion may be confused with other diseases, it is recommended an histopathological exam to avoid misdiagnosis.

Thymic hormone dependent immunodeficiency in an infant with partial trisomy of chromosome 22.

An infant with partial trisomy 22 syndrome and recurrent infections showed severe cellular immunodeficiency and serum IgG reduction. A marked increase of E-RFCs was observed after in vitro incubation of peripheral blood lymphocytes with thymostimulin (TS), a calf thymic extract. The treatment with TS resulted in reduction of infections, correction of cellular immunodeficiency and increase of IgG levels. Such a primary thymic hormone dependent immunodeficiency may be due to an altered development of branchial arches, commonly present in trisomy 22 syndrome. The same pathogenetic mechanism may account for thymus dysplasia observed in other chromosomal syndromes.

Defective interferon-alpha production in children with recurrent respiratory tract infections. A primary or secondary deficiency?

Interferon-alpha and -gamma production was assayed in 44 children with recurrent respiratory tract infections, in 9 patients with recurrent stomatitis and in 32 control subjects. A partial, transitory deficiency in interferon-alpha production was found in 2 children with recurrent respiratory tract infections. The deficiency may be either cause or effect of the recurrent infections. Many data support the latter hypothesis.

Thymostimulin therapy in patients with measles meningoencephalitis.

Four children with measles meningoencephalitis were treated with thymostimulin, a calf thymic extract. All patients were comatose when therapy was started. In all but one a good and sometimes rapid clinical improvement was observed. In the responsive patients no neurological or EEG sequellae were noted over a follow-up period of 1-4 years. The depression of cell-mediated immunity, present in some patients before treatment, normalised at the end of therapy.

[Sweet syndrome associated with articular and renal involvement]. / Síndrome de Sweet asociado a compromiso articular y renal.

We report a 29 years old female presenting with fever and painful infiltrated erythematous and violaceous plates with pseudo vesicles in the surface, located in both arms, four days after having suffered a tonsillitis. She was admitted with the diagnosis of Sweet syndrome and the lesions disappeared spontaneously. Two months later, she presented with a similar condition, again after an upper respiratory infection. Five months later, she was admitted with arthralgias with positive rheumatoid factor and antinuclear antibodies. Three years after the first admission, she was admitted with an acute glomerulonephritis and renal failure after another upper respiratory infection. Sweet syndrome was described in 1964 and, although initially considered benign, its association with inflammatory diseases or cancer has been reported.

Relevance of anti-T monoclonal antibodies in the study of children with primary immunodeficiencies.

T-cell phenotypic analysis with anti-T monoclonal antibodies (MoAb) was performed on 37 children with immunologic disorders. Abnormalities of T-cell differentiation and/or of T-cell subset distribution were observed in many patients. In particular two infants with severe combined immunodeficiency showed immunologically incompetent common thymocytes (OKT6+) in the circulation, in one case a proportion of OKT6+ cells was OKT4-, OKT8-. A boy with a selective T-cell defect synthetized normal levels of Ig classes, despite the marked reduction of helper/inducer T-cells (OKT4+). Irregularities of T-cell subsets were also noted in children with Wiskott-Aldrich syndrome and in some patients with selective IgA defect or hypogammaglobulinaemia. In one of these, in whom the agammaglobulinaemia was caused by EBV infection, a persistently reversed OKT4+/OKT8+ ratio together with an excessive suppressor T-cell function were found more than 10 years after the onset of the disease. Such a case supports the hypothesis that a viral infection may cause, in a predisposed host, both the agammaglobulinaemia and an abnormality of the regulatory T-cell subpopulations. Such abnormalities, together with those found in the other children studied, underline the importance of MoAb against different T-cell antigens for a better characterization of primary immunodeficiencies.

Effect of circulation and discussion of cervical smears on agreement between laboratories.

The study objective was assessing whether circulation of smears and discussion of those with differing interpretation can increase reproducibility between laboratories. The study included: the blind interpretation of a first set of 194 smears among seven laboratories, the discussion of smears with discrepant diagnoses during the previous phase and the blind interpretation of a second set of smears of same size and characteristics. After discussions, the overall weighted kappa increased in five laboratories (substantially in three : +50%, +27% and +20%). However, no change was observed in one laboratory and a slight decrease (-4%) in another. The latter interpreted the second set of smears at a longer time interval from discussions. Agreement improved for all diagnostic classes except low grade intraepithelial neoplasia (LSIL). Overall, the intervention increased diagnostic agreement, but its effect varied with laboratory and by diagnostic class and could be transient. Continued programmes of smear exchange and discussion appear to be advisable.

Síndrome de Sweet asociado a compromiso articular y renal / Sweet syndrome associated with articular and renal involvement

We report a 29 years old female presenting with fever and painful infiltrated erythematous and violaceous plates with pseudo vesicles in the surface, located in both arms, four days after having suffered a tonsillitis. She was admitted with the diagnosis of Sweet syndrome and the lesions disappeared spontaneously. Two months later, she presented with a similar condition, again after an upper respiratory infection. Five months later, she was admitted with arthralgias with positive rheumatoid factor and antinuclear antibodies. Three years after the first admission, she was admitted with an acute glomerulonephritis and renal failure after another upper respiratory infection. Sweet syndrome was described in 1964 and, although initially considered benign, its association with inflammatory diseases or cancer has been reported