A 3-year-old-male had the appearance of red urine at birth and developed recurrent bullae in sun-exposed area of the skin, erythrodontia, alopecia, splenomegaly and hemolytic anemia, We observed coral red fluorescence of the teeth and urine under Wood's light and detected excessive excretion of the uroporphyrin in the urine and coproporphyrin in the stool wlth inreased porphyrin in the blood. Fluorescence of erythrocyte was demonstrated by:fluoreacence microscopy. Histologic findings showed subepidermal bulla with PAS-positive hyaline deposits around the blood vessels and revealed IgG deposits in the wall of blood vessels and dermo-epidermal junction by direct immunofluorescence.
Alopecia ; Anemia, Hemolytic ; Anthozoa ; Blood Vessels ; Erythrocytes ; Fluorescence ; Fluorescent Antibody Technique, Direct ; Hyalin ; Immunoglobulin G ; Microscopy ; Parturition ; Porphyria, Erythropoietic* ; Porphyrias ; Skin ; Splenomegaly ; Tooth

A 50-year-old woman with lupus erythematosus profundus of about a year's duration was reported. The patient had no history of discoid lupus erythematosus or systemic lupus erythematosus. Dermatologic examination revealed two, firm, freely movable, well demarcated, subcutaneous plaques, with mildly erythematous overlying skin, measuring 2 x 2 cm (right), 5 x 5 cm (left), was present on the both deltoid region. Histopathologically, this showed panniculitis. Diret immunofluorescence studies showed IgG and IgM deposition on the dermoepidermal junction.
Female ; Fluorescent Antibody Technique ; Humans ; Immunoglobulin G ; Immunoglobulin M ; Lupus Erythematosus, Discoid ; Lupus Erythematosus, Systemic ; Middle Aged ; Panniculitis ; Panniculitis, Lupus Erythematosus* ; Skin

The CD44 protein has a functional domain for binding hyaluronic acid, maintaining the integrity and structure of the epithelium. The purpose of this study is to evaluate the presence and comparison of distribution of CD44 in human and rabbit corneas using immunohistochemical staining method. Immunostaining for CD44 was observed in the epithelium, endothelium and human stromal keratocytes. In particular, the strong positive deposition for CD44 can be seen in basal and limbal layers of the epithelium and in the endothelium. Immunodeposits for CD44 in rabbit cornea were detected in superficial parts of the epithelium, while they were localized in deep stromal keratocytes and the endothelium. In conjunctival tissue, immunostaining for CD44 was found in the epithelium, connective tissue, and vascular endothelium in human specimen but was faintly demonstrated only in the extracellular matrix of connective tissue in the rabbit conjunctiva. No immunostaining for CD44 was seen in control case. Our results suggest that CD44 protein can be seen both in human and rabbit corneas. However, there was different distribution of CD44 between two specimens.
Conjunctiva ; Connective Tissue ; Cornea* ; Endothelium ; Endothelium, Vascular ; Epithelium ; Extracellular Matrix ; Humans* ; Hyaluronic Acid

An alkali burn of the cornea severely damages not only the epithelium but also the underlying keratocytes and endothelium, the extracellular matrix is thought to play an important role in burn wound healing of the cornea. The glycoprotein CD44 of extracellular matrix has been implicated in biological cell behavior, including lymphocyte binding to endothelial venules, embryonic development cell migration, cell differentiation, and tumor metastasis. The purpose of this study is to investigate the presence and distribution of CD44 in wound healing using immunohistochemical staining method after alkali burning of rabbit cornea. 2 hour after burn, there was no immunodeposit for CD44 at keratocytes and endothelial cells at denuded epithelial burn area. 4 hours postburn, the epithelial cell gradually migrated over the burn area from the peripheral unburn area and immunodepositive staining of the keratocytes was observed at the margin of burn site. 24 hours postburn, immunopositive reaction for CD44 at adjacent alkali burn area was more strongly labeled, but the peripheral epithelial response of unburn area was faintly labeled by them. 48 hours postburn, immunopositive reaction gradually shifted from the border portion of alkali burn to the center portion of burn site. On days 7, the epithelium was completely reepithelized over the burn area and 3 weeks postburn, immunostaining were demonstrated the entire layer of burn site. The recovering time of the nornal immunostaing for CD44 of the rabbit cornea from the alkali burn was taken about 7 weeks. Our results suggest that the CD44 protein can be seen in proliferating in the epithelial cells, the keratocytes end endothelial cells, and is thought to play an important role in wound healing of the alkali burn cornea.
Alkalies* ; Burns* ; Cell Differentiation ; Cell Movement ; Cornea ; Embryonic Development ; Endothelial Cells ; Endothelium ; Epithelial Cells ; Epithelium ; Extracellular Matrix ; Female ; Glycoproteins ; Lymphocytes ; Neoplasm Metastasis ; Pregnancy ; Venules ; Wound Healing* ; Wounds and Injuries*

PURPOSE: The purpose of this study is to help diagnosis migraine and tension-type headache in children with chronic recurrent headache by comparing clinical characteristics of migraine and tension-type headache. METHODS: We performed a clinical analysis of 89 children diagnosed as migraine and/or tension-type headache by using international headache society classification with the aid of Prenky's criteria of migraine and Jay's category of tension-type headache. RESULTS: The diagnosed group consisted of 39 children with migraine, 40 tension-type headache cases and coexisting migraine and tension-type headache in 10 cases. In the location of the headache, the incidence of temporal area was significantly higher in the migraine than in tension-type headache(P<0.001) and the incidence of occipital area was significantly higher in the tension-type headache than in migraine(P<0.01). In migraine, 27 cases(69.2%) had pulsating headache which was significantly higher than in the tension-type headache(P<0.001). In tension-type headache, 18 cases(45.0%) had dull headaches which were significantly higher than in migraine(P<0.01). Nausea, vomiting, dizziness, relief of headache after sleep, photophobia, and visual disturbance among associated symptoms with headache were significantly higher in migraine than tension-type headache(P<0.05). The incidence of family history of headache in migraine(71.8 %) was higher than in the tension-type headache(40.0%)(P<0.01). CONCLUSION: Diagnostic criteria are significant in diagnosis of migraine and tension-type headache. The characteristics of headache are overlapped in both headache types and there are differences of expression for headache in children, so there is a problem in diagnosing of headache type. The international Headache Society Classifications are useful for children, but there are problems that diagnostic criteria are too strictly applied to children.
Child* ; Classification ; Diagnosis ; Dizziness ; Headache ; Humans ; Incidence ; Migraine Disorders* ; Nausea ; Photophobia ; Tension-Type Headache* ; Vomiting

No abstract available.
Animals ; Calcaneus* ; Muscle, Skeletal* ; Rats* ; Tenotomy*

BACKGROUND: Functional dyspepsia (FD) is a commonly encountered disturbance of gut function and has been shown to be associated with psychological disturbance such as depression and anxiety. Of particular importance to clinicians are the relationship between anger, alexithymia, and depression. In this study, we investigated anger, alexithymia, and depression in patients with functional dyspepsia. METHODS: Thirty patients who visited Wonkwang University Hospital from January 2001 to June 2001, were diagnosed with functional dyspepsia by a gastroenterologist and compared with 37 healthy control group. Medical investigation of FD including gastrofiberscopy, esophageal manometry, and ambulatory 24-hours intraesophageal reflux test were negative. All subjects were evaluated for depression, anxiety, anger and anger expression, and alexithymia. The measures included Beck Depression Inventory (BDI), Spielberger State-Trait Anxiety Inventory (STAI), Spielberger State-Trait Anger Expression Scale (STAXI), and Toronto Alexithymia Scale (TAS). RESULTS: The FD patients reported significantly more symptoms of depression, more difficulty describing feeling to others in TAS, less anger-in and anger-out expression in STAXI than the control subjects. Depressive symptoms in FD were positively correlated with state anxiety, trait anxiety, alexithymia, state anger, trait anger, and anger-in expression. In multiple regression model, state anger and trait anxiety together accounted for 69.1% of the depression in FD. CONCLUSION: The FD patients reported more depressive symptoms, and the depressive symptoms were related to anxiety, anger and anger-in, and alexithymia. These finding lend support that FD is a syndrome in which biopsychosocial process and affect dysregulation may play a role in features of FD.
Affective Symptoms* ; Anger* ; Anxiety ; Depression* ; Dyspepsia* ; Humans ; Manometry

lnflammatory pseudotumor (IPT) is a rare, non-neoplastic inflammatory process. It is most commonly occurs in the orbit, but extension into brain parenchyma is uncommon. In a confirmed case of IPT, most cases show good improvement with steroid theraphy. A 50-year-old man with progressive left-eye visual disturbance and mass lesion was admitted in a hospital. A left orbital mass biopsy revealed what was highly suspected as an inflammatory pseudotumor. Steroid pulse therapy with dexamethasone, radiation therapy, and chemotherapy with amphotericin B were performed, but they were not effective in improving the condition of the patient. Revision open surgery was then performed. A follow-up brain enhancement computerized tomography showed an enlarged mass volume and hydrocephalus with periventricular enhancement. As an additional procedure, ventriculoperitoneal shunt and tuberculosis medication were administered. About 2 weeks later, clinical symptoms and radiologic findings improved. We present a case of intra-cranial IPT and discuss further treatment methods.
Amphotericin B ; Biopsy ; Brain ; Cavernous Sinus ; Central Nervous System ; Combined Modality Therapy* ; Dexamethasone ; Drug Therapy ; Follow-Up Studies ; Granuloma ; Granuloma, Plasma Cell* ; Humans ; Hydrocephalus ; Middle Aged ; Orbit ; Skull Base* ; Skull* ; Sphenoid Sinus ; Tuberculosis ; Ventriculoperitoneal Shunt

Leber's Hereditary Optic Neuropathy(LHON) is a maternally inherited disorders that occurs primarily in young males and is characterized by subacute, sequential, bilateral central visual loss, ultimately, optic atrophy. We report 2 cases of molecularly confirmed LHON which reveal 11778 and 14484 mitochondral DNA mutation, respectively but there is no family history of visual loss. So the diagnosis of LHON deserves to be considered in all crypotogenic cases of acute or subacute optic or chiasmal neuropathy. Late or early age at onset, female gender, and a negative family history should not be dissuasive.
Diagnosis ; DNA ; Female ; Humans ; Male ; Molecular Biology* ; Optic Atrophy ; Optic Nerve Diseases*

STUDY DESIGN: A literature review on the radiologic findings of pelvic parameters for treatment of spinal deformity OBJECTIVES: This review examines sagittal spine alignment, pelvic parameters, and methods for assessing alignment, and examines the relationships among all of these parameters to understand spinal deformity. SUMMARY OF LITERATURE REVIEW: Understanding the main pelvic and sagittal spinal parameters and recognizing their correlation is imperative in the diagnosis and treatment of various spinal disorders. MATERIALS AND METHODS: Review of the literature. RESULTS: As spinal and pelvic parameters tend to have a strong correlation, it is essential to measure not only spinal parameters but also pelvic parameters in analyzing sagittal balance. Degenerative changes have the potential to greatly disrupt the normal curvature of the spine, leading to sagittal malalignment. Analysis of sagittal balance is crucial to optimizing the management of spinal diseases. Improvement in surgical outcomes may be achieved through better understanding of radiographic spino-pelvic parameters and their association with deformity. CONCLUSIONS: Understanding spinal and pelvic parameters raises awareness of the relationship among alignment and balance, the soft tissue envelope, and compensatory mechanisms, which will, in turn, provide a more comprehensive understanding of the nature of spinal deformity and the modalities with which it is treated.
Congenital Abnormalities ; Diagnosis ; Spinal Diseases ; Spine