Objective The indications for endoscopic treatment in early stage cancer of the digestive tract are expanding with the emergence and technical development of endoscopic submucosal dissection (ESD). ESD requires longer term stable sedation than conventional endoscopic procedures due to the necessity of meticulous control of the devices during the procedure. Propofol has a very short half-life and can be administered continuously, which is advantageous for long-term sedation. Propofol, thus, is likely to be useful for sedation during ESD. Methods Fifty consecutive patients who underwent ESD for early gastric cancer with propofol sedation (Group P) and those with midazolam sedation (Group M) were included in this study. Cardiorespiratory suppression rate and the condition of arousal were compared between the groups. A questionnaire survey on the satisfaction of endoscopists, anesthesiologists, endoscopy nurses, and ward nurses with the use of propofol was also carried out. Results Respiratory suppression was observed in 50% in Group M and in 20% in Group P (p<0.05). Hypotension was seen in 14% and 36% in Groups M and P, respectively (p<0.05). No sedation-related complications were encountered in either of the groups. Arousal rates 1 hour and 3 hours after the procedure were 23% and 60% in group M and 86% and 100% in Group P (p<0.05). As for the questionnaire survey, most respondents, in particular the ward nurses, supported the use of propofol. Conclusion Our data suggest that propofol is safe and useful during ESD as compared with midazolam.
A 37-year-old man was diagnosed with Wilson disease at the age of 14. His first manifestations were neurological. He was treated with trientine for more than 10 years and suffered from anemia and liver dysfunction. Wilson disease is a genetic disorder characterized by accumulation of copper in the body. Excess copper is toxic, but copper is an essential trace element. Copper-binding ceruloplasmin is important for iron metabolism. Excess copper chelating treatment-induced anemia and iron deposition in the liver was suspected. Proper monitoring of copper status is important for the management of Wilson disease.
We describe a 60-year-old man with IgG4-related chronic sclerosing dacryoadenitis and sialoadenitis associated with lymphoplasmacytic and eosinophilic infiltration in erythematous nodules. Physical examination revealed left eye extrusion and small itchy nodules on the scalp and neck. The serum IgG level was 1,570 mg/dL, IgG4 463 mg/dL (29.5%), and IgE 4,554 IU/mL. Lacrimal gland biopsy disclosed prominent infiltrates of IgG4-positive plasma cells and scattered eosinophilic infiltrates with fibrosis, consistent with IgG4-related disease. A skin biopsy of a cutaneous nodule demonstrated that the infiltrated plasma cells around arterioles or venules in the deep dermis and subcutaneous fat tissue were strongly positive for IgG4. Although the swollen lacrimal and parotid gland and itchy subcutaneous erythematous nodules improved rapidly with oral prednisolone at a dose of 20 mg per day, the skin, lacrimal, and parotid lesions deteriorated simultaneously during steroid tapering and improved after increasing the dosage. As skin lesions are easy to biopsy, further study of the skin manifestations of IgG4-related disease will be important in further clarifying the clinical spectrum, pathophysiology and response to therapy of this disorder.
A 38-year-old man was admitted for evaluation of Cushing's syndrome. Physical findings showed swelling of the face, and hypertension, but not Cushingoid stigmata. Laboratory data revealed serum cortisol level of 34.1 μg/dL and plasma ACTH of 140 pg/mL. Overnight administration of 1 and 8 mg dexamethasone did not suppress plasma ACTH or serum cortisol. Chest X-ray showed a mass at the upper-anterior quadrant of the mediastinum, and chest CT scan revealed a heterogenous tumor of approximately 60 mm in diameter, which infiltrated into the superior vena cava and ascending aorta, and caused superior vena cava syndrome. The tumor was resected. Histological examination indicated large cell neuroendocrine carcinoma of the thymus and positive immunoreactivity for ACTH. Ten days after the operation, the plasma ACTH decreased as low as 13.7 pg/mL. The present study indicates that large cell neuroendocrine carcinoma of the thymus can cause superior vena cava syndrome and ectopic ACTH syndrome.
Most mantle cell lymphoma patients show remarkable disseminated disease at the initial diagnosis. We describe two cases of mantle cell lymphoma mainly involving thoracic lesions at the initial presentation of the disease. The clinical presentations were right hilar lymphadenopathy in one case and right pleural thickness in the other. The diagnosis of mantle cell lymphoma was confirmed by immunohistochemistry, including CD5, CD20, and cyclin D1, and the presence of t(11 ; 14)(q13 ; q32) by fluorescence in situ hybridization. These thoracic manifestations at the initial diagnosis should be taken into consideration for the clinical spectrum of mantle cell lymphoma.
We here report a rare case of a patient with IgD-lambda-positive multiple myeloma presenting with FDG-PET/CT negative bone marrow involvement. A 72-year-old man was admitted to our hospital for evaluation of a paravertebral tumor of the chest. Thoracotomy was performed and a histopathological evaluation of resected intrathoracic tumor demonstrated a plasmacytic neoplasma. Initially we thought that this case was a solitary plasmacytoma because there were no positive findings on postoperative FDG-PET/CT. However, bone marrow aspiration study demonstrated massive infiltration of myeloma cells (72%). It is necessary to recognize that IgD-lambda type myeloma cells may not be sufficiently metabolically active to form high uptake on FDG-PET/CT.
Cardioembolic stroke due to tumor emboli is a rare complication of neoplasm. A patient with metastatic cardiac liposarcoma who suffered from embolic stroke is reported. Autopsy confirmed that the cardiac tumor was a metastatic liposarcoma from the retroperitoneum, and the cerebral vessel was occluded by tumor cells and fibrin clot.
An 18-year-old woman with Gitelman syndrome (GS) associated with idiopathic intracranial hypertension (IIH) is described. She was obese and showed a 10 kg gain in body weight over a period of 8 months. She presented with headache, vomiting, and diplopia. She had bilateral papilledema, and right abducens palsy. CSF examination demonstrated high pressure (over 320 mmH2O) with normal cytochemistry. Brain MRI was normal. She showed mild alkalosis, hypokalemia, hypomagnesemia, increased plasma renin activity, and normal blood pressure. Two heterozygous mutations in the SLC12A3 gene were identified. Therefore, she was diagnosed as GS with IIH. We should keep in mind the possible occurrence of IIH in GS.
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