Trastornos hereditarios de la fibra colágena / Inherited disorders of collagen fiber

A propósito de la publicación en esta revista del artículo titulado Evaluación de la hemostasia en niños con síndrome de Ehlers-Danlos tipo III de los autores Mirta C. Campo Díaz y col1, tenemos a bien comentar algunos de los tópicos que en este se abordan.Los trastornos hereditarios del tejido conectivo incluyen al síndrome Ehlers Danlos como una de las entidades con signos clínicos de hipermovilidad articular, que no es privativa de este síndrome genético, pero como condición genética constituye un espectro con sobrelapamiento clínico y molecular difícil de diferenciar...

Análise dos efeitos da eletrolipólise no tratamento do fibro edema gelóide por meio da biofotogrametria computadorizada / Analysis of the effects of electrolipolysis in the treatment of fiber edema geloid by means of computer-aided biophotogrammetry

O Fibro Edema Gelóide (FEG) é uma infiltração edematosa do tecido conjuntivo subcutâneo e considerada uma patologia multifatorial, classificando-se em quatro graus: Grau I; Grau II, Grau IIIe Grau IV. Dentre as inúmeras técnicas terapêuticas a eletrolipólise apresenta-se como um método novo e promissor, pois o estímulo circulatório produzido pela corrente elétrica tem grande importância na drenagem da área tratada. Esse efeito, particularmente, é um dos que justifica o uso da eletrolipólise no tratamento do FEG. Como forma de avaliação quantitativa para verificar a evolução do tratamento,foi utilizada a Biofotogrametria Computadorizada, através da qual foi realizada a avaliação da área e do perímetro das lesões no prée no pós-tratamento. Assim, este estudo teve como objetivo analisar os efeitos da eletrolipólise no tratamento do FEG grau III por meio da biofotogrametria computadorizada. Realizou-se 10 sessões coma eletrolipólise na região glútea. A variável área apresentou maior redução percentual média, se comparada à variável perímetro, tanto para as condições de com contração (CC) como sem contração (SC). Após a análise dos resultados, concluiu-se que a eletrolipólise pode ser utilizada com eficácia em pacientes que apresentem FEG grau III, tendo em vista o biotipo tratado.

The Fiber Edema Geloid (FEG) is an infiltration of the edematous subcutaneous tissue and is considered a multifactorial disease, classified into four grades: Grade I, Grade II, Grade III and Grade IV. Among the many therapeutic techniques, electrolipolysis appears to be a new and promising method, since the circulatory stimulation generated by electrical current is of great importancein the drainage of the treated area. This effect, in particular, is one that justifies the use of eletrolipolysis in the treatment of FEG. In order to check treatment progress as a quantitative assessment, we used a computerized biophotogrammetry. The lesion perimeter and surface area in the pre and post-treatment were evaluated. This study aimed at analyzing the effects of electrolipolysis in the treatment of FEG grade III by means of computerized photogrammetry. We conducted 10 sessions with electrolipolysis in the gluteal region. The variable area showed greater mean percent reduction when compared to the perimeter variable for both conditions with contraction and without contraction. After analyzing the results, we concluded thatthe electrolipolysis can be used successfully in patients with gradeIII FEG, in view of the biotype treated.

Tumor desmoide: reporte de un caso / Desmoide tumor: case report

El tumor desmoide es una neoplasia rara de tejidos blandos que se desarrolla a partir de músculo, tejido conectivo, fascia y aponeurois. Se presenta esporádicamente y más frecuentemente en mujeres. El caso que se presenta concierne a paciente femenina de 27 años que fue sometida a escisión radical de una gran masa que infiltraba músculo recto abdominal, diagnosticada como tumor desmoide. Aunque tiene características benignas, es de naturaleza infiltrativa y se comporta como una masa localmente agresiva, la cual puede invadir estructuras adyacentes haciendo que la resección quirúrgica sea difícil. El único tratamiento viable es la cirugía amplia dejando bordes sanos, esto causa gran defecto de pared y por tanto serios problemas en la reconstrucción. Además, la tasa de recurrencias locales varía y depende de la edad del paciente, localización y los márgenes de resección.

Desmoid tumor is quite rare soft tissues neoplasm that develops from muscle connective tissue, fasciae and aponeuroses. This neoplasm occurs in sporadic and more frequent in women than men. The presented case report refers to young female (27 years old), who underwent the radical excision of a large desmoid tumour infiltrating the right rectus muscle of the abdomen. Although desmoid is classified pathologically as a benign tumour, its infiltrative nature leads to a locally aggressive mass, which can invade surrounding structures and organs making surgical resection difficult. The only radical treatment for her was the surgical resection carried out far from the tumour borders into the healthy tissues. This resection causes wide muscle-fascial defects determining serious reconstructive problems. Futhermore, overall local recurrence rates vary and depend on patient’s age, tumour location and margins at resection.

Fibroblastic reticular cells and fibroblast-like cells determined by monoclonal antibodies B-F45 and B-D46 in humans

Identification of stromal microenvironmental components of lymphoid organs is relatively harder at light microscopic level as few markers, which are mostly not very specific, are available to be used for such a purpose. We screened a large panel to determine monoclonal antibodies [mAbs] those reactive with fibroblasts/fibroblast-like cells aiming to obtain further evidence for the organization and function of this cell group. Tissue samples of forty patients undergoing surgery in Otorhinolaryngology, Obstetrics and Gynecology, Orthopedics and Traumatology, Cardiovascular Surgery and General Surgery Departments, Hacettepe University Medical Faculty Hospital, Ankara, Turkey, due to different pathologies obtained as partial specimens of surgery which were apart from pathological examination were immunostained by indirect immunoperoxidase method in Histology and Embryology department in 2003. Among the screened monoclonal antibodies, monoclonal antibodies B-F45 and B-D46 reacted with the members of the family, therefore examined in detail in available human organs. Among the unique staining patterns of these mAbs, reactivity on fibroblastic reticular cells, perineural sheet cells pericryptal/perivillous fibroblasts were striking. Both mAbs will provide useful tools for further studies on stromal network of peripheral lymphoid organs and peripheral nerves