Erdheim-Chester disease with multisystem involvement in a 4-year-old.

Erdheim-Chester disease is a rare form of non-Langerhans histiocytosis of unknown origin occurring mainly in adults. It is extremely rare in children. We report a case of a 4-year-old boy with Erdheim-Chester disease that initially presented as hemifacial palsy and bone pain with multisystem involvement. We describe radiographic findings of bones that show characteristic bilateral symmetrical osteosclerosis with atypical osteolytic lesions in addition to CT findings for pulmonary involvement and MR findings for intracranial lesions.

An infected urachal cyst containing an appendicolith: a case report.

We present a case of an 8-year-old girl with an infected urachal cyst containing an appendicolith found secondary to an appendico-urachal cyst fistula caused by perforated appendicitis. The clinical features and computed tomographic manifestations of this unusual case are discussed.

A chenodeoxycholic derivative, HS-1200, induces apoptosis and cell cycle modulation via Egr-1 gene expression control on human hepatoma cells.

We previously reported that HS-1200, a synthetic chenodeoxycholic acid derivative, has apoptosis-inducing activity in various human cancer cells. The present study was undertaken to examine whether HS-1200 had an anticancer effect on HepG2 (wild-type p53) and Hep3B (p53 deleted) human hepatoma cells. Treatment of both cells with HS-1200 resulted in growth inhibition and induction of apoptosis as measured by MTT assay, nuclear staining, DNA fragmentation and flow cytometry analysis. The increase in apoptosis was associated with the alteration in the ratio of Bcl-2/Bax protein expression. In addition, flow cytometry analysis indicated that HS-1200 induced G1 phase arrest in both cells. When analyzing the expression of cell cycle-related proteins, we found that HS-1200 reduced the expression levels of cyclin D1, cyclin A, and Cdk2. HS-1200 treatment also caused an increase in the expression levels of p21 WAF1/CIP1 in HepG2 cells in a p53-dependent manner and in Hep3B cells in a p53-independent manner. Moreover, the expression level of p27 KIP1 was increased in both cell lines. We also observed that HS-1200 decreased the levels of cyclooxygenase (COX)-2 mRNA and protein expression. Furthermore, HS-1200 treatment markedly induced the Egr-1 expression at an early time point, and the increased expression levels of p53, p21 WAF1/CIP1, p27 KIP1, and COX-2 after treatment with HS-1200 were completely inhibited in HepG2 cells and partially inhibited in Hep3B cells by silencing of Egr-1, respectively. Taken together, these findings provide important new insights into the possible molecular mechanisms of the anticancer activity of the synthetic bile acid derivative, HS-1200, through Egr-1 regulation.

The common ultrasonographic features of pilomatricoma.

OBJECTIVES: The purpose of this series was to describe typical ultrasonographic features of 20 cases of pilomatricoma and to improve its diagnostic rate with the use of an ultrasonographic approach. METHODS: For 20 pilomatricomas in 19 patients with preoperative ultrasonography from 1995 to 2004, we reviewed age, sex, symptoms, duration, referring clinician, and tumor sites. The ultrasonographic findings were retrospectively analyzed for tumor location, shape, size, margin, echo texture, echogenicity, presence, amount, and shape of calcification, presence of a hypoechoic rim, and Doppler flow pattern. RESULTS: The mean age of the 19 patients was 6.9 years (range, 1-21 years), and the female-male ratio was 1.1:1. Patients had a painful palpable mass in 10 cases (50%). Nine lesions occurred in the neck, 5 in the cheek, 2 in the preauricular region, and 4 in the extremity. All tumors were located in the subcutaneous layer. The mean size of the tumors was 13.4 mm. Fourteen pilomatricomas (70%) appeared as well-defined oval masses. Tumors were heterogeneously hyperechoic in 80% of cases. All tumors had internal echogenic foci. A hypoechoic rim was seen in 17 cases (85%). Doppler flow signals were observed in the peripheral region in 14 cases (70%). A correct preoperative diagnosis was made in 33% on the basis of clinical findings and in 76% by ultrasonography. CONCLUSIONS: Diagnosis of pilomatricoma should be considered when a well-defined mass with inner echogenic foci and a peripheral hypoechoic rim or a completely echogenic mass with strong posterior acoustic shadowing in the subcutaneous layer of the head, neck, or extremity is found on ultrasonography.

A peculiar multiple polypoid mucosal prolapse causing jejunal intussusception.

A case of jejunal intussusception caused by a multiple polypoid mucosal prolapse of jejunum in a 10-year-old girl is reported. The rarity of the lesion, its location, symptoms, and diagnosis are discussed.

A case of pyothorax-associated lymphoma simulating empyema necessitatis.

We describe a case of a diabetic man with a 40-year history of chronic tuberculous empyema presented with fever, chest pain and bulging soft tissue of the right chest wall. CT scan revealed a huge chest wall mass showing extensive necrosis with air-bubbles and destruction of the ribs. Decortication and extirpation of the chest wall mass were performed, and histopathologic examination confirmed diffused large cell type non-Hodgkin's lymphoma.