Early and Atypical Radiologic Presentations of Pulmonary Langerhans Cell Histiocytosis: A Report of Two Cases.

Pulmonary Langerhans cell histiocytosis (PLCH) is a rare, multi-systemic disease primarily affecting young male adults with a history of smoking. The two patients with PLCH in our report showed relatively early and atypical radiologic presentations at initial evaluation. On chest CT, PLCH presents variable radiologic features depending on the evolutional stage of the disease. Atypical CT features of PLCH may render precise radiologic diagnosis difficult and usually require lung biopsy for a confirmation of the diagnosis. Our case review is aimed at raising the awareness of radiologists on the atypical CT features of PLCH, to help make accurate radiologic diagnosis and prevent unnecessary and invasive diagnostic procedures.

A pancreatic hemorrhagic pseudocyst with pseudoaneurysm and the role of doppler ultrasonography: a case report.

Hemorrhagic pseudocysts with pseudoaneurysms are a rare and fatal complication of chronic pancreatitis due to the erosion of pancreatic to peripancreatic arteries. The timing of the rupture cannot be accurately predicted, but prompt diagnosis and management are essential to prevent further bleeding. We describe the case of a 68-year-old man who presented acute epigastric pain and anemia and had a history of chronic pancreatitis with a pseudocyst. A biliary and pancreas MRI showed an enlarged size of a known pancreatic pseudocyst with internal high signal intensity material. Color-Doppler ultrasonography showed pulsating signals in the pseudocyst, and our final diagnosis was a pseudoaneurysm in the pancreatic hemorrhagic pseudocyst. The pseudoaneurysm was successfully treated with coil embolization of the feeding artery. We report this case of a rare complication of chronic pancreatitis to show that color-Doppler ultrasound is a non-invasive and effective diagnostic tool for pseudoaneurysm, which enables early detection and prompt treatment without the need for invasive diagnostic modalities.

A pancreatic hemorrhagic pseudocyst with pseudoaneurysm and the role of doppler ultrasonography: a case report

SUMMARY Hemorrhagic pseudocysts with pseudoaneurysms are a rare and fatal complication of chronic pancreatitis due to the erosion of pancreatic to peripancreatic arteries. The timing of the rupture cannot be accurately predicted, but prompt diagnosis and management are essential to prevent further bleeding. We describe the case of a 68-year-old man who presented acute epigastric pain and anemia and had a history of chronic pancreatitis with a pseudocyst. A biliary and pancreas MRI showed an enlarged size of a known pancreatic pseudocyst with internal high signal intensity material. Color-Doppler ultrasonography showed pulsating signals in the pseudocyst, and our final diagnosis was a pseudoaneurysm in the pancreatic hemorrhagic pseudocyst. The pseudoaneurysm was successfully treated with coil embolization of the feeding artery. We report this case of a rare complication of chronic pancreatitis to show that color-Doppler ultrasound is a non-invasive and effective diagnostic tool for pseudoaneurysm, which enables early detection and prompt treatment without the need for invasive diagnostic modalities.

Beetle immunity.

Genetic studies have elegantly characterized the innate immune response in Drosophila melanogaster. However, these studies have a limited ability to reveal the biochemical mechanisms underlying the innate immune response. To investigate the biochemical basis of how insects recognize invading microbes and how these recognition signals activate the innate immune response, it is necessary to use insects, from which larger amounts of hemolymph can be extracted. Using the larvae from two species of beetle, Tenebrio molitor and Holotrichia diomphalia, we elucidated the mechanisms underlying pathogenic microbe recognition. In addition, we studied the mechanism of host defense molecule amplification. In particular, we identified several pattern recognition proteins, serine proteases, serpins and antimicrobial peptides and examined how these molecules affect innate immunity.

A Korean female patient with thiamine-responsive pyruvate dehydrogenase complex deficiency due to a novel point mutation (Y161C)in the PDHA1 gene.

Pyruvate dehydrogenase complex (PDHC) deficiency is mostly due to mutations in the X-linked E1alpha subunit gene (PDHA1). Some of the patients with PDHC deficiency showed clinical improvements with thiamine treatment. We report the results of biochemical and molecular analysis in a female patient with lactic acidemia. The PDHC activity was assayed at different concentrations of thiamine pyrophosphate (TPP). The PDHC activity showed null activity at low TPP concentration (1 x 10(-3) mM), but significantly increased at a high TPP concentration (1 mM). Sequencing analysis of PDHA1 gene of the patient revealed a substitution of cysteine for tyrosine at position 161 (Y161C). Thiamine treatment resulted in reduction of the patient's serum lactate concentration and dramatic clinical improvement. Biochemical, molecular, and clinical data suggest that this patient has a thiamine-responsive PDHC deficiency due to a novel mutation, Y161C. Therefore, to detect the thiamine responsiveness it is necessary to measure activities of PDHC not only at high but also at low concentration of TPP.