We report an autopsy case of neonatal giant cell hepatitis that was presumed to be related to bacterial sepsis, endotoxemia and to the subsequent parenteral alimentation and antibiotics treatment. The patient died of candidal endocarditis and multiple brain infarcts. This female baby was born by a normal full term spontaneous delivery. Six days after delivery she developed fever and lethargy as she suffered from Cheyne-Stokes respiration with severe grunting. Blood culture grew Enterobacter and Acinetobacter. After management of the sepsis her general condition improved. On the 23rd day of admission she was found to have deep jaundice and hepatosplenomegaly. The liver became larger progressively and the edge was palpable at the umbilical level. Grade II systolic murmur was heard along the left lower sternal border. She died on the 31st day of hospitalization. Postmortem examination showed severe jaundice, hepatosplenomegaly, a large vegetation on the mitral valve and multiple petechial hemorrhages of the viscera. Microscopically the liver showed features of massive giant cell transformation, mild fibrosis and inflammatory cells, suggestive of giant cell hepatitis. Numerous yeasts and candidal pseudohyphae were seen in the cardiac vegetation, focally extending into the myocardium. There was a focus of candidal vasculitis in the bowel wall. In addition there were multiple bilateral organizing infarcts in the cerebral hemisphere as well as diffuse white matter damage associated with septicemia.
Female ; Infant, Newborn ; Humans

A female newborn had the following characteristics; a congenital localized absence of skin over the lower extremities; blistering of the skin or mucous membrane, incited by trauma, which heals without scarring; and congenital absence or deformity of the nails. In respect to the characteristic manifestation, clinical course and electron microscopic features, our patient seems to fit well into Bart's syndrome. The inheritance pattern appears to be autosomal dominant but, as in this report, isolated cases have been recognized. As the review of the literatures, congenital localized absence of skin has been observed in various subsets of inherited E.B. But, we believe that the term Bart's syndrome should be used to identify patients with good prognosis as the Bart's initial description To the best of our knowedge, this is the first reported case of Bart's syndrome in the korean literature.
Blister ; Cicatrix ; Congenital Abnormalities ; Female ; Humans ; Infant, Newborn ; Inheritance Patterns ; Lower Extremity ; Mucous Membrane ; Prognosis ; Skin

No abstract available.
Neck Dissection* ; Neck*

Callotasis has been widely used to treat brachymetatarsia. But various complications have been reported. Avascular necrosis of the 4th brachymetatarsia treated by callotasis has not been frequently addressed in the literature. We report 1 cases of avascular necrosis of the 4th brachymetatarsia treated by callotasis with a review of the literature.
Head ; Metatarsal Bones ; Necrosis ; Osteogenesis, Distraction

No abstract available.
Barium* ; Child* ; Humans ; Intussusception* ; Ultrasonography*

BACKGROUND: Pruritus is one of the most common complaints of patients with chronic renal failure undergoing hemodialysis. But its etiology is not clear, and there is no universally effective treatment. Recently we have experienced eosinophilia in hemodialysis patients suffering from pruritus. OBJECTIVE: The purpose of the this study was to evaluate the relationship between pruritus and eosinophilia in patients on hemodialysis. METHODS: We examined degree of pruritus and eosinophilia in 65 patients with end stage renal disease who were on hemodialysis from September 1996 to August 1997 in artificial kidney center of Chonbuk National University Hospital. The study was conducted by means of personal interview, physical examination, and review of medical records. The degree of pruritus was measured by scores from minimal 0 point to maximal 48 points and more than 5% or 500/mm3 eosinophils in peripheral blood was regarded as eosinophilia. RESULTS: 1)Incidence of each cutaneous symptoms were pruritus(61.5%), xerosis(35.4%), hyperpigmentation (30.8%), easy bruising(16.9%), nail change(12.3%), decreasing in sweating(10.8%), hypotrichosis(7.7%), and acne(4.6%). 2)Forty(61.5%) of 65 patients undergoing hemodialysis had pruritus, 32.3% in mild, 20% in moderate, and 9.2% in severe degree by pruritus score. 3)Nineteen(29.2%) of the 65 patients had eosinophilia:23.8%(5/21) of patients with mild pruritus, 30.7%(4/13) of patients with moderate pruritus, and 66.7%(4/6) of severe pruritus. It could be said that prevalence of eosinophilia was related to the severity of pruritus. 4)With repeated hemodialysis the severity of pruritus increased in 65%(26/40), decreased in 22.5% (9/40), did not change in 12.5%(5/40) of patients with pruritus. 5)Pruritus was not related to the serum calcium (Ca), inorganic phosphorus(PO4), CaxPO4 product, BUN, or creatinine. There was, however, significant correlations with the serum alkaline phohphatase. CONCLUSION: There was a positive correlation bet- ween the prevalence of eosinophilia and severity of pruritus in patients on hemodialysis.
Calcium ; Creatinine ; Eosinophilia* ; Eosinophils ; Humans ; Hyperpigmentation ; Jeollabuk-do ; Kidney Failure, Chronic ; Kidneys, Artificial ; Medical Records ; Physical Examination ; Prevalence ; Pruritus* ; Renal Dialysis*

We report herein a case of meningitis due to Streptococcus viridans that occurred after epidural nerve block. The low virulence of S. viridans resulted in milder clinical symptoms and signs than are usually observed for bacterial meningitis, thus mimicking viral meningitis. The infection may have originated from the oral cavity of the medical personnel, and so S. viridans infection should be included in the differential diagnosis of meningitis subsequent to spinal manipulation.
Diagnosis, Differential ; Manipulation, Spinal ; Meningitis ; Meningitis, Bacterial ; Meningitis, Viral ; Mouth ; Nerve Block ; Streptococcus ; Viridans Streptococci

BACKGROUND: Carotid cavernous fistula (CCF) is an abnormal communication between the carotid artery and the cavernous sinus. The pathogenesis of spontaneous CCF remains unclear, although sinus thrombosis is known to be a predisposing factor for dural arteriovenous fistula. Because spontaneous CCFs are mainly of the dural type, we considered that thrombogenic conditions, such as, protein S deficiency might be associated with CCF. CASE REPORT: A 42-year-old woman complained of conjunctival injection and retro-orbital pain that first appeared 1-month before visiting our hospital. She had no history of head trauma or intracranial surgery. Exophthalmos and chemosis were observed in her left eye, which also had lower visual acuity and higher intraocular pressure than the right eye. Magnetic resonance images and cerebral angiography revealed a left dural CCF. Her protein S was low, at 41% (normal range: 70-140%), but other hematologic values related to coagulation were normal. Her symptoms were relieved after initial transvenous coil embolization. However, a newly developed sixth-nerve palsy was detected 4 days after initial embolization. Follow-up angiography revealed a minimal shunt, and thus transvenous coil embolization was repeated. Two days later, the ophthalmoplegia started reducing, and 1-month later it had almost disappeared. CONCLUSIONS: To the best of our knowledge, this is the first report of spontaneous dural CCF in a Korean patient with concurrent protein S deficiency. Interestingly, transient sixth-nerve palsy developed after transvenous coil embolization in this patient. This additional symptom caused by the residual fistula was relieved after additional transarterial embolization.
Adult ; Angiography ; Carotid Arteries ; Cavernous Sinus ; Caves ; Central Nervous System Vascular Malformations ; Cerebral Angiography ; Craniocerebral Trauma ; Exophthalmos ; Eye ; Female ; Fistula ; Follow-Up Studies ; Humans ; Intraocular Pressure ; Magnetic Resonance Spectroscopy ; Ophthalmoplegia ; Paralysis ; Protein S ; Protein S Deficiency ; Sinus Thrombosis, Intracranial ; Visual Acuity

PURPOSE: Primary cutaneous anaplastic large cell lymphoma is rarely encountered in the lower eyelids. We report a patient with primary cutaneous anaplastic large cell lymphoma arising from the lower eyelid. METHODS: A 39-year-old man presented with a relatively fast growing mass on the center of his left lower eyelid for one month. The mass did not respond to local injection of triamcinolone at a local clinic. The lesion appeared as a solitary reddish nodule with ulceration, was non-tender, round, crusted, and measured 13 mm x 11 mm x 5 mm. Well- developed superficial vessels were found on the surface of the nodule. An incisional biopsy was performed. RESULTS: Histologic examination revealed that the bulk of the infiltrate was in the papillary and reticular dermis. Tumor cells had abundant, well-defined cytoplasm and pleomorphic nuclei with multiple nucleoli. The majority of the neoplastic cells showed immunoreactivity for CD 30 (Ki-1) along the cell membrane. A histopathological diagnosis of primary cutaneous anaplastic large cell lymphoma was made. CONCLUSIONS: Most cases of primary cutaneous anaplastic large cell lymphoma arise from the body and extremities. However, since primary cutaneous anaplastic large cell lymphoma may occur in the eyelid, it should be differentiated from nodular and relatively fast growing inflammatory tumors despite local steroid treatment.
Adult ; Biopsy ; Cell Membrane ; Cytoplasm ; Dermis ; Diagnosis ; Extremities ; Eyelids ; Humans ; Lymphoma, Primary Cutaneous Anaplastic Large Cell* ; Triamcinolone ; Ulcer

INTRODUCTION: Middle ear cholesteatoma is notorious for its capacity to absorb surrounding bone, but the exact mechanism of bone destruction is not fully understood until now. Recently, a group of matrix metalloproteinases (MMPs) were found to dissolve extracellular matrices. Among them, collagenase-3 (MMP-13), a newly found collagenase, is thought to be a strong enzyme to dissolve bone and cartilage. So we tried to find out whether Collagenase-3 is found in cholesteatoma and plays some role in bone destruction by cholesteatoma. MATERIALS AND METHOD: We performed immunohistochemical stains on 5 cholesteatomas, 5 middle ear granulations, 5 normal middle ear mucosa and 5 deep meatal skin specimens with anticollagenase-3 antibody and analysed the staining patterns. RESULTS: All the cholesteatomas showed strong immunoreactivity with collagenase-3. Two out of 5 granulations showed somewhat weaker and more disseminated patterns of immunoreactivity, but the rest 3 granulations showed no immunoreactivity. Normal middle ear mucosa and deep meatal skin specimens showed no immunoreactivity at all. CONCLUSIONS: Collagenase-3 may play an active role in the process of bone destruction by cholesteatoma; however, a further study using zymography or blotting method is needed to make this clear.
Cartilage ; Cholesteatoma ; Cholesteatoma, Middle Ear* ; Collagenases ; Coloring Agents ; Ear, Middle* ; Extracellular Matrix ; Humans* ; Matrix Metalloproteinase 13* ; Matrix Metalloproteinases ; Mucous Membrane ; Skin