No abstract available.
Child* ; Humans

No abstract available.
Flow Cytometry* ; Immunophenotyping* ; Precursor Cell Lymphoblastic Leukemia-Lymphoma*

Meckel Gruber syndrome consisting of an occipital encephalocele, polycystic kidney disease and polydactyly is a rare autosomal recessive disorder with a recurrence risk of 25%. Targeted ultrasonography in late embryonic or early fetal stages of pregnancy has great importance in diagnosis and management of affected pregnancy in high risk groups due to incomplete genetic mapping of meckel syndrome gene (MKS). We present a case of prenatal diagnosis at 14 weeks' gestational age of Meckel Gruber syndrome in a woman, who experienced same disorder in her previous pregnancy.
Diagnosis ; Encephalocele ; Female ; Gestational Age ; Humans ; Polycystic Kidney Diseases ; Polydactyly ; Pregnancy ; Prenatal Diagnosis ; Recurrence ; Ultrasonography

Malignant pericardial mesothelioma is a rare and progressive cardiac tumor. There is no established standard treatment and the prognosis is poor. Most patients were retrospectively diagnosed from surgery or autopsy due to absence of specific clinical manifestation. Most patients with pericardial mesothelioma have demonstrated constrictive physiology on echocardiography or cardiac catheterization. Therefore, pericardial mesothelioma was often misdiagnosed as other causes of constrictive pericarditis. We report a case of primary pericardial mesothelioma misdiagnosed as pericardial metastasis of unknown origin.
Autopsy ; Cardiac Catheterization ; Cardiac Catheters ; Echocardiography ; Heart Neoplasms ; Humans ; Mesothelioma ; Neoplasm Metastasis ; Pericarditis, Constrictive ; Pericardium ; Prognosis ; Retrospective Studies

BACKGROUND: Selective 2-agonists cause decrease in serum K+ concentration. Midazolam is an anxiolytic, sedative, and amnestic drug. Premedication of midazolam prevents increase of catecholamine with anxiety. Clonidine, alpha 2-adrenergic receptor agonist, supresses sympathetic outflow from central nervous system. So we can expect that premedication of clonidine or midazolam will prevent hypokalemia before induction of anesthesia. METHODS: Twenty two patients received 300 mcg clonidine per oral, 22 patients 0.05 mg/kg midazolan IM and 22 patients had no premedication. We measured serum K+ level at out-patient Department (T1), at 11:00 P.M. of the day before surgery (T2) and immediately before induction of anesthesia (T3). RESULTS: Serum K+ levels at T2 decreased compared to serum K+ level at T1 in all groups. Serum K+ levels T3 decreased compared to serum K+ level at T2 in control and midazolam groups but clonidine group did not decrease in serum K+ level. CONCLUSIONS: We can not prevent decrease of serum K+ level with premedication of midazolam but we can prevent decrease of serum K+ level with premedication of clonidine. So premedication of clonidine is more effective than midazolam in prevention of hypokalemia before induction of anesthesia.
Anesthesia* ; Anxiety ; Central Nervous System ; Clonidine* ; Humans ; Hypokalemia* ; Midazolam* ; Outpatients ; Premedication

PURPOSE: To evaluate the plain chest radiographic findings of smoke inhalation. MATERIALS AND METHODS:Our study included 72 burn patients who had suffered smoke inhalation. On admis-sion,all underwent serial portable chest AP radiography. We retrospectively reviewed the plain chest radi-ographs taken between admission and pootburn day five, evaluating the pattern, distribution, and time onset of direct injury to the respiratory system by smoke inhalation. The lesions were also assessed for change. RESULTS: In 16 of 72 patients (22%), abnormal findings of direct injury to the respiratory system by smoke in-halation were revealed by the radiographs. Abnormal findings were 15 pulmonary lesions and one subglottic tracheal narrowing. Findings of pulmonary lesions were multiple small patchy consolidations (10/15), peri-bronchial cuffing (8/15), and perivascular fuzziness (6/15). Patterns of pulmonary lesions were mixed alveolar and interstitial lesion (n=9), interstitial lesion (n=5), and alveolar lesion (n=1). No interlobular septal thicken-ing was observed. Pulmonary edema was distributed predominantly in the upper lung zone and perihilar re-gion, with asymmetricity. Its time of onset was within 24 hours in 13 cases, 24 -48 hours in two cases, and 48 -72 hours in one. Five of 16 patients progressed to ARDS. CONCLUSION: Chest radiographs showed that pulmonary lesions caused by inhalation injury were due to pul-monary edema, which the pattern of which was commonly mixed alveolar and interstitial.
Burns ; Edema ; Humans ; Inhalation* ; Lung ; Pulmonary Edema ; Radiography ; Radiography, Thoracic* ; Respiratory System ; Retrospective Studies ; Smoke* ; Thorax*

Variant angina is characterized by spontaneous episodes of angina, usually occurring in the morning and having ST segment elevation on the electrocardiogram. However, in the case presented here, vasospasm and angina was shown by ergonovine without ST elevation. The patient was a 60-year-old man who presented with a 2-year history of frequent chest pain. There were no abnormalities in coronary angiography. When ergonovine (100 microg) was injected, total occlusion of the proximal right coronary artery was seen, without ST elevation at the electrocardiogram. The cause was collateral from left anterior descending artery to distal right coronary artery at the left coronary angiography. Therefore, in a patient with variant angina without ST elevation, a transient collateral circulation during vasospasm should be considered.
Angina Pectoris, Variant ; Arteries ; Chest Pain ; Collateral Circulation ; Coronary Angiography ; Coronary Vessels ; Electrocardiography ; Ergonovine ; Humans ; Middle Aged

Endodermal sinus tumor (EST) is a rare malignant germ cell tumor arising usually in the gonads such as the testis or ovary. However, 10-15% of EST cases may arise in the extragonadal sites, such as sacrocoxygeal area, mediastinum, vagina, brain and retroperitoneum in a frequent occurring order. To our knowledge there are only 2 case reports of extragonadal EST, one originating from the omentum in 1998, and another which was localized in the pelvis in 2003. We report one case of extragonadal sinus tumor which arose in appendix and mesentery in the 19-year-old female with a review of the literature.
Appendix* ; Brain ; Endoderm* ; Endodermal Sinus Tumor* ; Female ; Gonads ; Humans ; Mediastinum ; Mesentery* ; Neoplasms, Germ Cell and Embryonal ; Omentum ; Ovary ; Pelvis ; Testis ; Vagina ; Young Adult

Bortezomib is an inhibitor of 26S proteasome, which is an effective treatment for multiple myeloma. The common adverse effects of bortezomib are asthenic conditions, gastrointestinal disturbances, and peripheral neuropathy. Here we describe a patient with dyspnea and general weakness because of complete atrioventricular block while receiving bortezomib. We immediately stopped bortezomib, and after inserting a permanent VDD pacemaker, the patients' symptoms disappeared.
Atrioventricular Block/*chemically induced ; Boronic Acids/*adverse effects/*therapeutic use ; Female ; Humans ; Middle Aged ; Multiple Myeloma/*drug therapy ; Pyrazines/*adverse effects/*therapeutic use

Bortezomib is an inhibitor of 26S proteasome, which is an effective treatment for multiple myeloma. The common adverse effects of bortezomib are asthenic conditions, gastrointestinal disturbances, and peripheral neuropathy. Here we describe a patient with dyspnea and general weakness because of complete atrioventricular block while receiving bortezomib. We immediately stopped bortezomib, and after inserting a permanent VDD pacemaker, the patients' symptoms disappeared.
Atrioventricular Block/*chemically induced ; Boronic Acids/*adverse effects/*therapeutic use ; Female ; Humans ; Middle Aged ; Multiple Myeloma/*drug therapy ; Pyrazines/*adverse effects/*therapeutic use