Primary intestinal lymphangiectasia is a congenital lymphatic disorder characterized by dilated intestinal lacteals resulting in lymph leakage into the small bowel lumen and responsible for protein losing enteropathy. As a result, generalized edema, hypoalbuminemia, and lymphocytopenia are clinically manifested. We could not find the reason by several examinations. Therefore, we performed double balloon enteroscopy (DBE), and intestnal lymphangiectasia was diagnosed histologically by a biopsy. DBE is a safe and effective method to diagnose small bowel lymphangiectasia. We report a case of primary intestinal lymphangiectasia, which occurred in a 54-year-old male patient with generalized edema and ascites.
Ascites ; Biopsy ; Double-Balloon Enteroscopy ; Edema ; Humans ; Hypoalbuminemia ; Lymphopenia ; Male ; Protein-Losing Enteropathies

BACKGROUND: Angioimmunoblastic T-cell lymphoma (AITL) is a complex lymphoproliferative disorder and often mimics a viral infection with frequent skin involvement. Epstein-Barr virus (EBV) and human herpes virus (HHV)-6 are reported to be associated with AITL, but there are conflicting results. OBJECTIVE: We evaluated the association of EBV and HHV-6 with AITL. METHODS: We reviewed the clinical, histological and immunophenotypical features of 19 cases of AITL. Among them, 11 lymph node biopsies of AITL were examined for HHV-6, -7, and -8 by polymerase chain reaction (PCR) using virus-specific primers. In situ hybridization of EBV early region RNA (EBER) was performed and T cell receptor (TCR) gene rearrangement was also investigated in some cases. RESULTS: Among these 19 cases, maculopapular, plaque or nodular skin lesions accompanied AITL in 12 cases. Clonal TCR gene rearrangement was seen in 8/9 cases tested. EBER in situ hybridization was positive in 8 cases (57.1%). Among 7 cases with skin biopsies, five cases were consistent with cutaneous involvement of AITL, 1 case was a drug eruption, and the other case was Kaposi's sarcoma. Except a HHV-8 (+) case who also had Kaposi's sarcoma, all of these cases were negative for HHV-6, -7 and -8. CONCLUSION: Skin manifestation seems to be a cardinal component of AITL, be it in the context of presentation, progression or recurrent disease. Recognition of clinicopathological features of skin lesions in AITL as diagnostic clues should be stressed among dermatologists. The lack of HHV-6, -7 and -8 in lymph node biopsy of AITL argues against a pathogenic role for HHVs in AITL.
Biopsy ; Drug Eruptions ; Gene Rearrangement ; Genes, T-Cell Receptor ; Herpesvirus 4, Human ; Herpesvirus 6, Human ; Herpesvirus 8, Human ; Humans ; In Situ Hybridization ; Lymph Nodes ; Lymphoma, T-Cell ; Lymphoproliferative Disorders ; Polymerase Chain Reaction ; Receptors, Antigen, T-Cell ; RNA ; Sarcoma, Kaposi ; Skin ; Skin Manifestations ; Viruses

Backgrounds/Aims: This study aimed to estimate the seroepidemiology of hepatitis B virus (HBV) infection with emphasis on the transmission of HBV infection between mothers and their children. METHODS: For 452 patients with HBV associated chronic liver disease, and 1,098 of their offspring, who visited Korea University Kuro Hospital from February, 2000 to February, 2001, HBsAg was tested by radioimmunoassay. RESULTS: Among siblings whose mothers were HBsAg-positive, the overall prevalence rate of HBV infection was 44.9% (140/312) and decreased with decreasing age (54.7% in > or =20 years old age group, 33.3% in 10-19 years old age group, 7.6% in <10 years old age group). The estimated proportion of perinatal infection out of modes of HBV transmissions in the general population was 38% in <10 years and 63.4% in 10-19 years. CONCLUSION: The present hepatitis B vaccination strategies -especially against perinatal infection- of Korea have performed to their utmost. More effective methods for the prevention of HBV transmission are now needed.
Child ; Epidemiology ; Hepatitis B Surface Antigens ; Hepatitis B virus ; Hepatitis B* ; Hepatitis* ; Humans ; Korea ; Liver Diseases* ; Liver* ; Mothers ; Prevalence ; Radioimmunoassay ; Siblings* ; Vaccination

No abstract available.

Interests in the microanatomy of the proximal segment of the internal carotid artery(ICA) have been increased due to the anatomical complexity of this region and the potential sites of the aneurysmal formation. In this study, microanatomical dissections were performed in 70 specimens obtained from 35 formalin-fixed human cadaveric heads to examine the superior hypophyseal arteries(SHAs), ophthalmic artery, and the carotid cave. In our study, 54(77%) carotid caves were presented in 70 specimens. Twenty-five(71%, 50 caves) cadavers harbored the carotid caves on both sides, 4(11%, 4 caves) cadavers only on the one side and the remaining 6(17%) didn't have the caval structure. Total of 162 SHAs which have showen to arise from the medial or posterior aspect of the ophthalmic(C6) segment of the ICA in all cases were identified: 108(67%) arose from the proximal half of the C6 segment and 54(33%) from the distal half. Interestingly, in 54 specimens which had the carotid caves, one to four SHAs arose from the part of the C6 segment within the cave in the 43(80%) specimens. Total number of the SHAs originated within the carotid cave were 51. Sixty-four(91%) of ophthalmic arteries have shown to arise just distal to the distal dural ring on C6 segment, whereas 4(6%) from the clinoid(C5) segment and in 2(3%) cases, the ophthalmic artery arose just at the insertion of the distal dural ring level. The authors describe the microanatomical relationships between the SHA and the carotid cave, and the origin of the SHA and the ophthalmic artery. We hope to get better knowledge of the vascular relationship and the possible mechanism of the SHA aneurysm.
Aneurysm ; Arteries* ; Cadaver ; Caves ; Head ; Hope ; Humans ; Ophthalmic Artery

To improve the surgical results of the removal of the pituitary tumors extending beyond the limit of the sella turcica, several modified transsphenoidal approachs have been proposed by many surgeons. A microanatomy of the sphenoid sinus, sellar, suprasellar and parasellar regions were studied in 50 formalin-fixed human cadaveric heads. Among them, microanatomical cadaveric dissections were performed in 5 specimens which were injected with colored silicone rubber. We aim to present a detailed microsurgical anatomy of structures surrounding the sella turcica. And also, the requisite microanatomical details necessary to extend the TSA to the medial compartment of the cavernous sinus and the supradiaphragmatic intradural space.
Cadaver ; Cavernous Sinus ; Head ; Humans ; Pituitary Neoplasms ; Sella Turcica ; Silicone Elastomers ; Sphenoid Sinus

Authors report a ganglion cyst located in spinal canal. The ganglion cyst is soft and movable mass in connective tissue around peripheral joints or tendon sheath. It has been rarely reported in lumbar spine. Symptoms and signs are usually difficult to distinguish it from other etiologies of radicular pain. The extradurally located ganglion cyst at L4-5 level which caused back pain and sciatica was removed with partial hemilaminectomy. Radiological finding of cyst in MRI may be variable, according to the composition of the cyst. Based on our experience and pertinent literatue, a ganglion cyst should be considered in the differential diagnosis of radicular pain in the presence of degenerative changes of the lumbar spine.
Back Pain ; Connective Tissue ; Diagnosis, Differential ; Ganglion Cysts* ; Joints ; Magnetic Resonance Imaging ; Sciatica ; Spinal Canal ; Spine* ; Tendons

The demyelinating diseases of the central nervous system(CNS), of which multiple sclerosis is the most common, have the characteristics which are selective loss of myelin with relative axonal preservation on the histopathologic findings, variable clinical findings and unknown pathophysiology. Occasionally, the clinical features, radiological and histopathological findings of patients with demyelinating disorders of the CNS mimic the brain tumor. Therefore, it may lead to erroneous surgical intervention as well as radiotherapy. The authors report a 26-year-old woman with demyelinating pseudotumor who presented with a generalized tonicclonic type seizure and left hemiparesis. Computerized tomography and magnetic resonance imaging of the patient revealed about 3X3X2cm sized enhanced mass lesion in the right frontal lobe with severe perifocal brain edema. This space-occupying lesion was resected subtotally and confirmed as the demyelinating pseudotumor via various neurohistochemical stains. Uncommonly, large focal cerebral demyelinating lesions presented as brain tumors clinically and radiologically. Therefore, it is mandatory to make a more careful diagnosis to differentiate the demyelinating pseudotumor from other true brain tumors before undergoing the surgery.
Adult ; Axons ; Brain Edema ; Brain Neoplasms ; Coloring Agents ; Demyelinating Diseases ; Diagnosis ; Female ; Frontal Lobe ; Humans ; Magnetic Resonance Imaging ; Multiple Sclerosis ; Myelin Sheath ; Paresis ; Radiotherapy ; Seizures

OBJECTIVE: Various lesions including tumors occupying in the presellar and suprasellar regions have been traditionally removed by the transcranial approach. The new modified transsphenoidal approaches(TSAs) have been proposed to avoid the craniotomy and to get better surgical view. MATERIALS AND PATIENTS: The sellar floor and presellar anterior cranial fossa were removed through the sublabial transseptal transsphenoidal technique in the "transsphenoidal supradiaphragmatic intradural approach". One tuberculum sella meningioma and a suprasellar Rathke's cleft cyst confined to the pituitary stalk were removed via this approach. RESULTS: The dissection of the anterior intercavernous sinus, diaphragma sella, and arachnoid membrane allowed a wide surgical field of pre- and suprasellar areas and facilitated a safe removal of lesions without significant surgical complications in our cases. CONCLUSION: From the authors' limited experience, the advantages of this technique are as follows: 1) it can be easily applicable through a minor modification of the standard TSA, 2) excellent anatomical exposure of the structures located in the supradiaphragmatic suprasellar cistern, and 3) might be suitable to remove small lesions located in the presellar and adjacent to the pituitary stalk region.
Arachnoid ; Cranial Fossa, Anterior ; Craniotomy ; Humans ; Membranes ; Meningioma ; Pituitary Gland

Glomus jugulare tumors are usually slow growing, but highly vascularized tumor. The authors report the case of huge glomus jugulare tumor treated by surgical resection with preoperative embolization. A 32-year old male patient presented with a history of right-sided facial palsy and hearing disturbance. Magnetic resonance imaging demonstrated an irregular large mass with multiple signal voids in the right temporal area. Prior to surgical excision of tumor, the patient underwent superselective embolization. Embolization of the tumor and its dominant feeding arteries were achieved. Six days later, the patient underwent an infratemporal fossa approach to remove the tumor. The patient did well postoperatively except CSF leakage that was treated successfully by dura repair. We discuss the usefulness of preoperative embolization and infratemporal approach for the huge glomus jugulare tumor.
Adult ; Arteries ; Facial Paralysis ; Glomus Jugulare Tumor* ; Glomus Jugulare* ; Hearing ; Humans ; Magnetic Resonance Imaging ; Male