The antiphospholipid syndrome is characterized by arterial thrombosis, venous thrombosis, pregnancy wastage, and thrombocytopenia associated with a persis tently positive lupus anticoagulant and/or moderate to high positive anticardiolipin antibodies(IgG or IgM). The antiphospholipid antibodies have been detected in many medical conditions, but the antiphospholipid syndrome (APS) has mainly been restricted to the primary antiphospholipid syndrome and APS associated with systemic lupus erythematosus. Rarely, the APS has been reported in other autoimmune disorders in the literature. We describe a woman with a limited form of scleroderma and the APS manifested by complete occlusion of left axillary artery with probable thrombotic occlusive nature, thrombocytope nia, prolonged aPTT, and persistently positive lupus anticoagulant.
Antibodies, Antiphospholipid ; Antiphospholipid Syndrome* ; Axillary Artery ; Female ; Humans ; Lupus Coagulation Inhibitor ; Lupus Erythematosus, Systemic ; Pregnancy ; Scleroderma, Limited* ; Thrombocytopenia ; Thrombosis ; Venous Thrombosis

The May-Hegglin anomaly is a rare autosomal dominant trait characterized by platelet abnormalities in the peripheral blood and large (up to 5mu) pale blue staining inclusions in the cytoplasm of neutrophils, eosinophils, basophils, and monocytes. We experienced a case of May-Hegglin anomaly in a 7 year old male and another case out of his family. His initial complaints at the admission were petechiae and intermittent epistaxis. Diagnosis was confirmed by peripheral blood smear and family study. We report the case with brief review of related literature.
Basophils ; Blood Platelets ; Child ; Cytoplasm ; Diagnosis ; Eosinophils ; Epistaxis ; Humans ; Male ; Monocytes ; Neutrophils ; Purpura

Sialosyl Tn mucin antigen (STn) is a carbohydrate antigen of tumor associated mucin formed by the premature 2~6 sialation of N-acetylgalactosamine. STn has been expressed in several tumor types and showed prognostic significance in colonic carcinoma. The authors evaluated the expression of STn immunohistochemically and correlated its expression with clinicopathologic variables in 100 gastric cancers. In early gastric cancer, STn was expressed in 24 cases out of 50 cases (48%). In advanced gastric cancer, STn was expressed in 48 of 50 (96%). The difference in STn expression between advanced gastric cancer and early gastric cancer was statistically significant. The difference in STn expression between tumors with lymph node metastasis and those without lymph node metastasis, between tubular adenocarcinoma and signet ring cell carcinoma, and between intestinal type and diffuse type adenocarcinoma was statistically insignificant in early or advanced gastric adenocarcinoma. These results suggest that the STn expression plays a role in the tumor progression in both early and advanced gastric adenocarcinomas.
Adenocarcinoma* ; Carcinoma, Signet Ring Cell ; Colon ; Lymph Nodes ; Mucins* ; Neoplasm Metastasis ; Stomach ; Stomach Neoplasms

Pancreatic pseudocyst occurs as a complication of pancreatitis or trauma, which results in the escape of pancreatic enzymes from the parenchyma or ductal system of pancreas. At that time, serum amylase may have returned to normal level, and the patient may be subjectively asymptomatic. In this phase, the radiologic findings are relatively greater significance than laboratory data. In the conventional radiologic study, pancreatic pseudocyst have been frequently confused with other retroperitoneal mass, but recently with clinical application of ultrasound and CT scan, more accurate diagnosis can be obtained. The brief results are as follows; 1. Male to female ratio was 3:2 in 15 patients. Incidence was most common in young adult age. Most frequent symptom was epigastic pain, and which was noted in 11 cases of patients. 7 cases of patients had past history of abdominal trauma and past history of pancreatitis was only in 1 case. Most common laboratory finding was leukocytosis in 8cases of patients and elevated serum amylase was also noted in 7 cases. 2. In each 5 cases of patients, plain chest roentgenologic evidence of left side pleural effusion and hemidiaphragm elevation were observed. 3. On flat abdomen film, soft mass shadow was visualized in 8 cases of patients. On UGI series, evidence of retrogastric space widening was observed in 11 cases of patients. 4. The location of pseudocyst is mainly in body and tail of pancrease in 11 cases of patients. 5. More accruable diagnosis can be obtained through application of ultrasound and CT scan.
Abdomen ; Amylases ; Diagnosis ; Female ; Humans ; Incidence ; Leukocytosis ; Male ; Pancreas ; Pancreatic Pseudocyst ; Pancreatitis ; Pancrelipase ; Pleural Effusion ; Tail ; Thorax ; Tomography, X-Ray Computed ; Ultrasonography ; United Nations ; Young Adult

PURPOSE: To describe the early experience of analyzing variations and time trends in bladder volume of the rectal cancer patients who received bladder ultrasound scan. MATERIALS AND METHODS: We identified 20 consecutive rectal cancer patients who received whole pelvic radiotherapy (RT) and bladder ultrasound scan between February and April 2012. Before simulation and during the entire course of treatment, patients were scanned with portable automated ultrasonic bladder scanner, 5 times consecutively, and the median value was reported. Then a radiation oncologist contoured the bladder inner wall shown on simulation computed tomography (CT) and calculated its volume. RESULTS: Before simulation, the median bladder volume measured using simulation CT and bladder ultrasound scan was 427 mL (range, 74 to 1,172 mL) and 417 mL (range, 147 to 1,245 mL), respectively. There was strong linear correlation (R = 0.93, p < 0.001) between the two results. During the course of treatment, there were wide variations in the bladder volume and every time, measurements were below the baseline with statistical significance (12/16). At 6 weeks after RT, the median volume was reduced by 59.3% to 175 mL. Compared to the baseline, bladder volume was reduced by 38% or 161 mL on average every week for 6 weeks. CONCLUSION: To our knowledge, this study is the first to prove that there are bladder volume variations and a reduction in bladder volume in rectal cancer patients. Moreover, our results will serve as the basis for implementation of bladder training to patients receiving RT with full bladder.
Humans ; Observer Variation ; Rectal Neoplasms ; Ultrasonics ; Urinary Bladder

PURPOSE: There have been some efforts to diagnose intracranial aneurysm through a non-invasive method using MRA, although the process may be difficult when the lesion is less than 3mm. The present study prospectively compare the results of high resolution, fast speed slice interpolation MRA and DSA therapy examing the potentiality of primary non-invasive screening test. MATERIALS AND METHODS: A total of 26 cerebral aneurysm lesions from 14 patients with subarachnoid hemorrhage from ruptured aneurysm (RA) and 5 patients with unruptured aneurysm(UA). In all subjects, MRA was taken to confirm the vessel of origin, definition of aneurysm neck and the relationship of the aneurysm to nearby small vessels, and the results were compared with the results of DSA. The images were obtained with 1.5T superconductive machine(Vision, Siemens, Erlangen, Germany) on 4 slabs of MRA using slice interpolation. the settings include TR/TE/FA=30/6.4/25, matrix 160x512, FOV 150x200, 7minutes 42seconds of scan time, effective thickness of 0.7mm and an entire thickness of 102.2mm. The images included structures from foramen magnum to A3 portion of anterior cerebral artery. MIP was used for the image analysis, and multiplanar reconstruction(MPR) technique was used in cases of intracranial aneurysm. RESULTS: A total of 26 intracranial aneurysm lesions from 19 patients with 2 patients having 3 lesion, 3 patients having 2 lesions and the rest of 14 patients having 1 lesion each were examined. Among those, 14 were RA and 12 were UA. Eight lesions were less than 2mm in size, 9 lesions were 3-5mm, 7 were 6-9mm and 2 were larger than 10mm. On initial exams, 25 out of 26 aneurysm lesions were detected in either MRA or DSA showing 96% sensitively. Specificity cannot be estimated since there was no true negative of false positive findings. When MRA and MPR were used concurrently for the confirmation of size and shape, the results were equivalent to those of DSA, while in the confirmation of aneurysm neck and parent vessels, the concurrent use of MRA and MPR was far superior to the sole use of either MRA or DSA. CONCLUSION: High resolution MRA using slice interpolation technique showed equal results as those of DSA for the detection of intracranial aneurysm, and may be used as a primary nin-invasive screening test in the future.
Aneurysm ; Aneurysm, Ruptured ; Angiography* ; Angiography, Digital Subtraction* ; Anterior Cerebral Artery ; Foramen Magnum ; Humans ; Intracranial Aneurysm* ; Mass Screening ; Neck ; Parents ; Prospective Studies ; Sensitivity and Specificity ; Subarachnoid Hemorrhage

We report a case of alopecia mucinosa in a 9-year-old girl. She presented with several papules on an erythematous patch on the right side of the ala nasi, a hairless nodular lesion on the same side of the nasal canal. Histopathologic findings obtained from the nasal canal revealed typical features of alopecia mucinosa, such as reticular degeneration, cystic space formation and mucin deposition in the pilosebaceous follicle. The case displayed no evidence or clinical features of any malignant changes, and we suspect the case to be an acute form of alopecia mucinosa.
Alopecia* ; Child* ; Female ; Humans ; Mucinosis, Follicular* ; Mucins

Familial juvenile polyposis is a rare intestinal polyposis characterized by the occurrence of multiple juvenile polyps in the gastrointestinal tract. We report a case of familial juvenile polyposis in a 17-year-old man with a review of the literature. This patient underwent total colectomy due to a 6 years history of rectal bleeding. Grossly, the colon showed 36 variable sized pedunculated polyps, measuring 2.5cm x 2cm from the largest size and 0.2cm x 0.2cm to the smallest size. Histologically, the polyps consisted of cystically dilated glands, lined by normal colonic epithelial cells, scattered in loose, edematous stroma containing inflammatory cell infiltration. There were no areas of tubular adenoma or malignancy in any of the polyp.
Adenoma ; Adolescent ; Colectomy ; Colon ; Epithelial Cells ; Gastrointestinal Tract ; Hemorrhage ; Humans ; Intestinal Polyposis ; Polyps

A 29-year-old male patient was admitted due to subacute onset of right chest pain. He had no history of trauma, chest surgery or other medical disease. Chest roentgenogram showed an expansile, radiolucent lesion on the posterior segment of right seventh rib. Computed tomogram of the chest also showed lytic expansile mass with septation. He took an en-bloc resection of the involved rib. Pathologically there were multiple cystic spaces, filled with blood and collagenofibrous tissue proliferation and locally areas of new bone formation and giant cells of osteoclastic type, which was compatible with aneurysmal bone cyst. We report a case of aneurysmal bone cyst of the rib with a brief review of literature.
Adult ; Aneurysm ; Bone Cysts ; Chest Pain ; Giant Cells ; Humans ; Male ; Osteoclasts ; Osteogenesis ; Ribs* ; Thorax

PURPOSE: To analyze angiearchitecture of arteriovenous malformations(AV malformation) in order to clarify the angiegraphic risk factors for intracerebral hemorrhage and other nonhemorrhagic symptoms. MATERIALS AND METHODS: Eighty-five patients with angiegraphically-proved brain arteriovenous malformation were included in this study. Retrospective review of clinical history and angiography was done. Topographic analysis and evaluation of 17 angiearchitectural characteristics were conducted. RESULTS: Deep-seated and cortico-callosal type, small nidus size, intranidal pouch, one draining vein, deep venous drainage only venous stenosis and venous aneurysm were the most discriminating or predictive characteristics of hemorrhage in brain arteriovenous malformation. And those with large nidus size, dural supply and venous hypertension were correlated with nonhemorrhagic symptoms such as seizure, headache and neurologic deficit. CONCLUSION: Detailed analysis of the angiearchitecture of brain arteriovenous malformations is needed to identify the features that are correlated with prognostic implications for the treatment of patients with ^V malformations.
Aneurysm ; Angiography ; Arteriovenous Malformations* ; Brain* ; Cerebral Hemorrhage ; Constriction, Pathologic ; Drainage ; Headache ; Hemorrhage ; Humans ; Hypertension ; Neurologic Manifestations ; Retrospective Studies ; Risk Factors ; Seizures ; Veins