Search results for "bone"

Expression of Matrix-Degrading Cysteine Proteinase Cathepsin K in Cholesteatoma

2001

Cholesteatoma is a nonneoplastic lesion of the middle ear space or mastoid that is histologically characterized by a progressive bone erosion of the ossicles and surrounding bone. Several matrix-degrading enzymes have been implicated as mediators of this bone erosion. Because the novel cysteine proteinase cathepsin K has been shown to play a central role in bone resorption, we examined the expression of this enzyme in tissue specimens of cholesteatoma. Tissue specimens of 9 patients with cholesteatoma were obtained during middle-ear surgery. Expression of cathepsin K mRNA was determined by RT-PCR using specific primers. Immunohistochemical analysis of cathepsin K protein expression in tissu…

Depressed indurated plaque with elastorrhexis as a distinctive lesion in Buschke‐Ollendorff syndrome

2020

Buschke-Ollendorff syndrome (BOS) is a rare autosomal dominant genodermatosis caused by heterozygous mutations in LEMD3 and characterized by connective tissue nevi and sclerotic bone lesions known as osteopoikilosis. We report a family with three individuals affected by BOS, two of whom manifested clinical and histopathological peculiarities, presenting with a depressed indurated plaque as the main cutaneous manifestation instead of the classic connective tissue nevi. Notable elastorrhexis was present in both biopsies.

HETEROGENEITY OF METATROPIC DYSPLASIA

1983

Metatropic dysplasia is a neonatally manifest entity that is characterized clinically by a rapidly progressing kyphoscoliosis leading to severe shortening of the originally long trunk ("metatropism"). Major radiographic features include flattening and defective ossification of the vertebral bodies, a narrow thorax and a marked hypoplasia of the basilar portions of the ilia with crescent-shaped iliac crests. There is some evidence of genetic heterogeneity. From five personal observations and from a review of the literature we conclude that metatropic dysplasia comprises at least three genetic entities: (1) a nonlethal type with autosomal recessive transmission; (2) a nonlethal dominant type …

Intrasinusoidal bone marrow infiltration: a possible hallmark of splenic lymphoma.

1996

A particular type of lymphomatous bone marrow infiltration defined as intrasinusoidal and occuring in seven patients with splenomegaly and mild to moderate thrombocytopenia is reported. Immunocytochemistry highlighted the intrasinusoidal pattern and showed a mature B-cell phenotype. Four patients underwent splenectomy and a diagnosis of splenic marginal cell lymphoma was made. The disease course seems to be chronic and quite indolent. All patients are alive and well ; follow-up ranges from 2 to 42 months. The value of bone marrow biopsy as a primary diagnostic tool in splenic marginal zone lymphomas is discussed.

CD27 distinguishes two phases in bone marrow infiltration of splenic marginal zone lymphoma.

2004

Aims:  To investigate CD27 expression in splenic marginal zone lymphoma (SMZL), an indolent low-grade B-cell lymphoma with constant involvement of the bone marrow, especially with an intrasinusoidal pattern. It is not clear if the neoplastic clone is composed of virgin or somatically mutated B cells. CD27 is reported to be a hallmark of memory B cells. Methods and results:  We evaluated 64 bone marrow biopsy specimens (BMBs) from 36 patients with SMZL for the expression of CD27. For comparison, splenectomy specimens of patients with traumatic splenic rupture or with SMZL were used. All BMBs showed lymphomatous infiltration. When located in the marrow sinusoids, neoplastic cells were CD27– i…

Bone marrow granulomas in hairy cell leukaemia following 2-chlorodeoxyadenosine therapy

1994

Stromal SPARC contributes to the detrimental fibrotic changes associated with myeloproliferation whereas its deficiency favors myeloid cell expansion.

2012

Abstract In myeloid malignancies, the neoplastic clone outgrows normal hematopoietic cells toward BM failure. This event is also sustained by detrimental stromal changes, such as BM fibrosis and osteosclerosis, whose occurrence is harbinger of a dismal prognosis. We show that the matricellular protein SPARC contributes to the BM stromal response to myeloproliferation. The degree of SPARC expression in BM stromal elements, including CD146+ mesenchymal stromal cells, correlates with the degree of stromal changes, and the severity of BM failure characterizing the prototypical myeloproliferative neoplasm primary myelofibrosis. Using Sparc−/− mice and BM chimeras, we demonstrate that SPARC contr…

Reconstruction of critical-size mandibular defects in immunoincompetent rats with human adipose-derived stromal cells

2013

In patients with bony defects, autologous bone grafts are the "gold standard" for reconstruction. In children, autologous bone harvesting is limited but tissue engineering offers an alternative. Next to bone marrow, adipose tissue is a source of mesenchymal stromal cells, and adipose-derived stromal cells (ADSC) can differentiate into osteocytes. The aim of this study was to evaluate the efficacy of bioactive implants (ADSC in fibrin glue) for repair of critical-size mandibular defects in athymic rats. Human adult ADSC embedded in fibrin glue were implanted into a critical-size defect in the rat mandible and their efficacy was compared to those of protected bone healing (pbh), autologous bo…

Bone Marrow-Derived Cells from Male Donors Do Not Contribute to the Endometrial Side Population of the Recipient

2012

Accumulated evidence demonstrates the existence of bone marrow-derived cells origin in the endometria of women undergoing bone marrow transplantation (BMT). In these reports, cells of a bone marrow (BM) origin are able to differentiate into endometrial cells, although their contribution to endometrial regeneration is not yet clear. We have previously demonstrated the functional relevance of side population (SP) cells as the endogenous source of somatic stem cells (SSC) in the human endometrium. The present work aims to understand the presence and contribution of bone marrow-derived cells to the endometrium and the endometrial SP population of women who received BMT from male donors. Five fe…

Maxillofacial fracture epidemiology and treatment plans in the Northeast of Iran: A retrospective study

2017

Background: The epidemiology of facial injuries varies based on lifestyle, cultural background and socioeconomic status in different countries and geographic zones. This study evaluated the epidemiology of maxillofacial fractures and treatment plans in hospitalized patients in Northeast of Iran (2015-2016). Material and Methods: In this retrospective study, the medical records of 502 hospitalized patients were evaluated in the Department of Maxillofacial Surgery in Kamyab Hospital in Mashhad, Iran. The type and cause of fractures and treatment plans were recorded in a checklist. Data were analyzed with Mann–Whitney test, chi-squared test and Fisher’s exact test, using SPSS 21. Results: The …