Q1: What is the diagnosis? The diagnosis is pycnodysostosis, which is a rare form of skeletal dysplasia characterised by moderate and generalised osteosclerosis. The diagnosis is based on clinical features and radiological findings. The cardinal features are short stature, large open fontanelles (the fontanelles may remain open even in adulthood), an obtuse mandibular angle, dysplastic clavicle...

A 28-year-old man with Philadelphia chromosome positive chronic granulocytic leukaemia developed extensive bone marrow and bone infarction which was associated with anaemia and thrombocytopenia. He survived 20 months from the first symptoms of bone marrow infarction; during this time he developed myelofibrosis and osteosclerosis followed by blastic transformation. Extensive bone marrow infarcti...

Condensing osteitis of the clavicle is a benign disorder leading to osteosclerosis of the medial end of the clavicle. The differential diagnosis between condensing osteitis of the clavicle and ischaemic necrosis of the medial clavicular epiphysis (Friedrich's disease), osteoid osteoma, and low grade osteomyelitis can be difficult. In the case history reported here, magnetic resonance imaging wa...

Abstract Background Bisphosphonates‎ inhibit osteoclasts, prevent bone resorption and decrease bone turnover. This study examined radiography finding in bisphosphonate-associated osteonecrosis of jaws. Materials and Methods This is a retrospective series of 12 clinically diagnosed patients between 7 to 21 year old (average 13 years). They required emergency dental conditions requiring mana...

OBJECTIVE Osteoporosis, osteosclerosis, and lytic bone lesions have been observed in patients with systemic mastocytosis (SM). We examined bone mineral density (BMD) biochemical turnover markers and serum tryptase levels in SM, which is considered a rare disease. MATERIALS AND METHODS Seventeen adult patients (5 females, 12 males; median age: 33 years, range: 20-64) with mastocytosis were inc...

Splenic enlargement (splenomegaly) develops in numerous disease states, although a specific pathogenic role for the spleen has rarely been described. In polycythemia vera (PV), an activating mutation in Janus kinase 2 (JAK2(V617)) induces splenomegaly and an increase in hematocrit. Splenectomy is sparingly performed in patients with PV, however, due to surgical complications. Thus, the role of ...

Primary myelofibrosis (PMF) is the most serious myeloproliferative disorder, characterized by clonal myeloproliferation associated with cytokine-mediated bone marrow stromal reaction including fibrosis and osteosclerosis. Current drug therapy remains mainly palliative. Because the NF-kappaB pathway is implicated in the abnormal release of cytokines in PMF, the proteasome inhibitor bortezomib mi...

The objectives of this study were to investigate the time-course of influence of compression of bone tunnel wall in ligament reconstruction on tissue around the bone tunnel and to histologically examine the mechanism of preventing the complication of bone tunnel dilation, using rabbit tibia. A model in which the femoral origin of the extensor digitorum longus tendon was cut and inserted into a ...