No abstract available.
Adhesives*

Chronic granulomatous disease (CGD) is one of congenital immunodeficient disease and a rare X-linked or autosomal recessive disease characterized by recurrent life- threatening infections and granuloma formation. We observed clinical features, laboratory findings and genetic subgroups of 33 children who were diagnosed with chronic granulomatous disease in the Department of Pediatrics, Seoul National University Children's Hospital. There were 23 males and 10 females. Activated NBT test of all patients revealed 0% positive cell and mothers of 15 patients had 25%- 75% normal neutrophils in the activated NBT test. According to the result of activated NBT test and family history, the ratio of X-linked and autosomal recessive inheritance was 2:3. There was a significant difference for the age at onset of the first infection in the different genetic subgroups. The X-linked group had the mean onset at 1.98 months of age and autosomal recessive group had a mean onset as late as 3.82 months (p<0.05). The most common type of the first infection was lymphadenopathies (41%) and other infections were skin pustules, fever, perianal abscess, pneumonia and chronic diarrhea. However, the age at diagnosis was not significant in the different genetic subgroups. Lymphadenitis (27%) was the most common infection, and pneumonia, gastrointestinal tract infection, skin infection were also common. The most common infectious agent was Candida sp. (5%) and other microorganisms involved were BCG, coagulase-negative staphylococcus, S. aureus, K/ebsiella pneumoniae, Aspergi/lus sp., and Enterococcus faecium. Chronic condition associated with CGD were hepatomegaly (59%), splenomegaly, and anemia of chronic disease, underweight, and lymphadenopathy. The leukocyte count of patients at diagnosis was within normal limit except in three patients and leukopenia was not observed in any of the patients. The humoral and cellular immunity and complement system were normal, but the level of Ig E in four patients was elevated. Early diagnosis of CGD can be made by suspicion if there are lymphadenitis after BCG vaccination and recurrent pyogenic infections under the first year of age. Though progression in the treatment of CGD, like gene therapy, is concerned, genetic counseling and prenatal diagnosis by carrier detection and molecular genetic analysis is thought to be necessary.
Abscess ; Anemia ; Candida ; Child ; Chronic Disease ; Complement System Proteins ; Diagnosis ; Diarrhea ; Early Diagnosis ; Enterococcus faecium ; Female ; Fever ; Gastrointestinal Tract ; Genetic Counseling ; Genetic Therapy ; Granuloma ; Granulomatous Disease, Chronic* ; Hepatomegaly ; Humans ; Immunity, Cellular ; Korea* ; Leukocyte Count ; Leukopenia ; Lymphadenitis ; Lymphatic Diseases ; Male ; Molecular Biology ; Mothers ; Mycobacterium bovis ; Neutrophils ; Pediatrics ; Pneumonia ; Prenatal Diagnosis ; Seoul ; Skin ; Splenomegaly ; Staphylococcus ; Thinness ; Vaccination ; Wills

To evaluate the typical appearance and the influence in the image interpretation of the metallic artifact which is known as one of the patient-related field artifacts, we analysed the magnetic resonance (MR) images of 40 patients (the total number of metallic materials were 45) acquired at MR 1.5T unit. All patients were screened for the presence of metal. The metallic implants were surgical wires and clips, orthopedic devices, and the other miscellaneous materials. The artifacts produced by metallic objects can be seen on MRI as the focal loss of signal and/or the local distortion of the image. Regardless of their ferromagnetic properties, metallic implants created regional artifacts in their images. Ferromagnetic materials, such as a lead fragment, showed severe artifacts and nonferromagnetic metals showed mild to moderate artifacts. The conspicuity of artifact was related tot he composition, mass, shape, orientation, and the location of the metallic objects in the body. Under high magnetic field strength, there were no significant differences between the various pulse sequences. Artifacts are particularly prominent on gradient-echo images. Our findings indicate that MR imaging of patients with standard nonferromagnetic metallic materials can be successfully performed and usefully interpretated.
Artifacts* ; Humans ; Magnetic Fields ; Magnetic Resonance Imaging* ; Magnets ; Metals ; Orthopedics

Most of the malignant tumors of the pancreas are adenocarcinomas arising from the ductal epithelium. Primary leiomyosarcoma of the pancreas, even though it is the most common sarcoma of the pancreas, is very rare. We present a case of leiomyosarcoma of the pancreas, probably primary, with metastases to the stomach, lymph nodes, and abdominal wall. A 52-year-old woman visited the hospital with vague right upper abdominal pain and weigh loss of 6 kg for 2 months. The radiological and endoscopic examination revealed that she had a large heterogeneous mass, 11 cm in size, in the pancreatic body and tail, a 4 cm-sized mass in the paraaortic area, and a 3 cm-sized polypoid mass in the stomach. Histologically, they were all similar to one another and composed of markedly pleomorphic cells. Immunohistochemical and electron microscopic studies showed definite smooth muscle differentiation of the tumor cells. Two months later, the patient underwent an excision of a new 3 cm mass in the right lower abdominal wall, showing features of well differentiated leiomyosarcoma.
Abdominal Pain ; Abdominal Wall ; Adenocarcinoma ; Epithelium ; Female ; Humans ; Leiomyosarcoma* ; Lymph Nodes ; Middle Aged ; Muscle, Smooth ; Neoplasm Metastasis ; Pancreas* ; Sarcoma ; Stomach

We analyzed the MR findings of spinal epidural mass in 23 patients retrospectively. MR images were performed at 1.5T unit. The lesions were confirmed as metastasis(12 cases), lymphoma (2 cases), leukemia(1 cases), multiple myeloma(2 cases), meningioma(3 cases), neurofibroma(1 case), dysraphism with lipoma(1 case) and lipomatosis(4 cases), Most MRI examinations consisted of T1, proton density and T2 weighted sagittal imaging of the spine, with additional pulse sequences or image planes as needed for clarification. The level of the spinal epidural mass was cervical spine level in 2 cases, thoracic in 15 cases, lumbar in 7 cases, and sacral in 2 cases. The location of epidural mass within the spinal canal was eccentric in 20 cases, multiple in 2 cases, and encircled in 4 cases. Paraspinal mass was seen in 11 cases. Signal intensity of epidural mass was variable. The marrow of spine revealed low signal intensity(SI) on TIWI and high SI on T2WI in 14 cases. In conclusion, MRI is a useful diagnostic tool for the evaluation of spinal epidural mass.
Bone Marrow ; Humans ; Lymphoma ; Magnetic Resonance Imaging ; Protons ; Retrospective Studies ; Spinal Canal ; Spine

Spinal epidural abscesses were evaluated using Gadolinium-enhanced magnetic resonance imaging (MRI) in 19 surgically and clinically confirmed cases, including 13 with tuberculous spondylitis and 6 with pyogenic spondylitis. We observed rim enhancement patterns in 10 patients and diffuse enhancement patterns in 9 patients. The spread patterns were localized to ventral or dorsal epidural space and encircled entire epidural space. We conclude that rim enhancement indicates abscess and diffuse enhancement indicates granulation tissue and fibrosis. Tuberculous epidural abscess spreads to ventral epidural space and pyogenic epidural abscess encircles entire epidural space.
Abscess ; Epidural Abscess* ; Epidural Space ; Fibrosis ; Granulation Tissue ; Humans ; Magnetic Resonance Imaging* ; Spondylitis

The magnetic resonance images (MRI) of 30 patients with surgically or biochemically confirmed pituitary adenomas (20 macroadenomas, 10 microadenomas) were retrospectively evaluated. Ten patients had hyperprolactinemia, another eight had acromegaly, another eight had nonfunctioning adenoma and four had Cashing disease. The examination were performed at a 1.5-T superconducting MR system using a multisection spin-echo technique with 3mm-thick sections and a 256×224 matrix. TI weighted sagittal and coronal images were obtained before and within 30 minutes after the administration of Gd-DTPA (0.1mmol/kg). Analysis of the MRI was focused on the signal intensity and enhamcement patterns of the pituitary adenoma before and after Gd-DTPA administration. Compared with endocrinologic diagnosis, macroadenoma showed heterogeneous enhacement in 55%, rim enhancement in 35% and homogeneous enhancement in 10%, Conclusively, the enhancement patterns of the pituitary adenoma did not correlate with the subtypes made according to hormone production.
Acromegaly ; Adenoma ; Diagnosis ; Gadolinium DTPA ; Humans ; Hyperprolactinemia ; Magnetic Resonance Imaging* ; Pituitary Neoplasms* ; Retrospective Studies

No abstract available.
Esophageal Atresia* ; Tracheoesophageal Fistula*

No abstract available.
Child* ; Humans ; Lung*

No abstract available.
Female* ; Humans ; Pregnancy*