No abstract available

Reactive perforating collagenosis, first described by Mehregan et al in ]967, is a skin disorder characterized by transepidermal eliniination of altered collagen fibers. This rare disease appears as recurrent, umbilicated, crusted papules in response to minor trauma. We report here a case of reactive perforating collagenosis in a 21-year-old man. Examination of the skin revealed a number of centrally umbilicated, asymptomatic papules filled with keratotic plugs in various development and regression. The lesions were distributed on the dorsa of the hands, face, and neck. The biopsy spccimen showed a few of thin bundles of collagen which v ere extruded from the dermis through the atrophic epidermis and the turinels within the epidermis, reaching the buttom of the plug.
Biopsy ; Collagen ; Dermis ; Epidermis ; Hand ; Humans ; Neck ; Rare Diseases ; Skin ; Young Adult

Apert syndrome, or acrocephalosyndactyly is a complex of associated malformations, which are craniosynostosis and symmetric complex syndactyly of the hands and feet. The syndrome was first described by Apert in 1906. Review of the worlds literature disclosed about 200 similar cases and 9 Cases in Korea. Authors experienced one case of Apert syndrome and treated surgically for acro-syndactyly and polydactyly of both hands and feet. The case was compared with the other previously reported cases in Korea.
Acrocephalosyndactylia ; Craniosynostoses ; Foot ; Hand ; Korea ; Polydactyly ; Syndactyly

A case of pathological hip dislocation in a child, aged 2 years 6 months, who had surgical treatment, is reported. The boy has history of osteomyelitis of right ilium at age of 10 month, which ended up with the right hip dislocation. Postoperatively cephalocotyloid relation of the right hip was very well reestablished during five years of postoperative observation. Through this case, it is reconfirmed that a child hip has a natural remodelling power even in case of hip joint destruction.
Child ; Dislocations ; Hip Dislocation ; Hip Joint ; Hip ; Humans ; Ilium ; Male ; Osteomyelitis

BACKGROUND: To evaluate the influence of changes in mitral valve area (MVA) and left atrial pressure on pulmonary vein flow (PVF) we analyzed PVF with transesophageal echocardiography (TEE) before, after and one-year after percutaneous mitral valvuloplasty (PMV) in patients with mitral stenosis (MS) in sinus rhythm. METHODS: PMV was guided with TEE. Follow-up TEE was done about one year after PMV. MVA and transmitral mean gradient (TMG) were measured. Systolic velocity (S), diastolic velocity (D), atrial reversal velocity (AR), their time-velocity integral (S-TVI, D-TVI, AR-TVI) and their ratio (S/D ratio, S-TVI/D-TVI ratio were evaluated. RESULTS: The number of patients was twenty-two (F:20). The age was 39+/-9 years (range:26-64). Follow-up duration was 16+/-6 months (range:7-28). MVA increased from 0.9+/-0.2 cm2 to 1.9+/-0.3 cm2 after PMV and decreased to 1.7+/-0.3 cm2 on follow-up TEE significantly. TMG decreased from 15.4+/-4.3 mmHg to 5.5+/-1.9 mmHg after PMV and was 6.2+/-2.4 mmHg on follow-up. S increased significantly on follow-up at both pulmonary vein (PV). D increased on follow-up at left PV. S/D ratio increased on follow-up at both PV. AR increased on follow-up at both PV. S-TVI increased after PMV at left PV and increased on follow-up at both PV. D-TVI had no change. S-TVI/D-TVI ratio increased on follow-up at left PV. AR-TVI increased on follow-up at right PV. CONCLUSIONS: The main changes after PMV in patients with MS in sinus rhythm were increasing tendency in S, S-TVI, S/D ratio, S-TVI/D-TVI ratio and AR. And these changes were statistically significant on follow-up TEE rather than immediately after PMV.
Atrial Pressure ; Echocardiography* ; Echocardiography, Transesophageal ; Follow-Up Studies ; Humans ; Mitral Valve ; Mitral Valve Stenosis* ; Pulmonary Veins

The mechanism of cytolysis by complement attack of nucleated cells(NC) is of special interest in comparison to that of red blood cells. It is known that NC death by membrane attack comples, C5b-9, is caused by many factors, i.e., efficiency of complex assembly, activation of intrinsic metabolic pathway by signal transduction, cytotoxic effect of the channel itself and natural repair ability. These factors suggest that colloid osmotic lysis, known in red blood cells, does not fully explain the complement-mediated cell death of NC. In this study, the authors investigated correlation between biochemical and morphological changes to prove "Ca2+-mediated metabolic death"8~13) representing a mechanism of NC death caused by C5b-9 attack. The L1210 cells, mouse leukemic cell line carrying small complement channel(TAC5b-91) were used in the experiments. The amounts of intracellular adenine nucleotides to extracellular Ca2+, ouabain, KC1 and dextran were analyzed by bioluminescence method using luminometer. Cell viability was checked by 0.4% trypan blue dye and LDH release. Morphological observation of TAC5b-91 was done by immunocytochemical staining and electron microscope. The results were as follows: 1) The release of ATP, ADP and AMP followed by cell death was rapid and progressive along the incubation time at 37 degrees C and it was accelerated in 1.5 mM of [Ca2+]0. 2) There was no evidence of ATP repairment in the TAC5b-91. 3) Extracellular KC1(150 mM), dextran(0.66 mM) and ATP supplement(0.2 microM) could not effectively inhibit ATP depletion and cell death. Ouabain(27 and 100 microM) enhanced cell death and could not completely prevent ATP loss. 4) Most of the mitochondria showed swelling, loss of cristae and Ca2+ deposit in matrix in the electron microscopic observation. Rapid, sustained and irreversible depletion of adenine nucleotides was due to Ca2+ deposit with destruction of mitochondria and also the leakage through transmembrane channels. Moreover this energy depletion was accelerated by high extracellular Ca2+ concentration. These results indicate that Ca2+-mediated, energy exhaustion is one of the mechanisms of the metabolic cell death by C5b-9 attack of NC.
Mice ; Animals

A 14-year-old boy and his 16-year-old elder brother, who had generalized tinea corporis for 1 and 3 years respectively, developed multiple discrete, non tender soft tumors on the scalp, forehead, neck and extremities. The boys were well nourished and had no systemic diseases. Histopathologic examination of the subcutaneous nodules revealed a well encapsulated granuloma containing lobulated granules characteristic of mycetoma; these granules consisted of septated fungal hyphae with vesicles. The dermatophyte isolated from the tumors was identified as Trichophyton schoenleinii.
Adolescent ; Arthrodermataceae ; Extremities ; Forehead ; Granuloma ; Humans ; Hyphae ; Male ; Mycetoma ; Neck ; Scalp ; Siblings* ; Tinea ; Trichophyton*

No abstract available.
Animals ; Rats*

Cystadenoma of the liver is a rare neoplasm. Although many cystadenomas are asymptomatic, symptoms can include abdominal pain, postprandial epigastric discomfort, and nausea. Dramatic changes in hepatic imaging techniques have been helpful for diagnosing cystic lesions of the liver, such as simple cyst, hydatid cyst, cystadenoma, cystadenocarcinoma, and metastatic neuroendocrine tumors. However, it remains difficult to differentiate cystadenoma from cystadenocarcinoma for multiseptated cystic hepatic lesions with papillary projection on computed tomography (CT) and magnetic resonance imaging (MRI). Here we report the case of a 47-year-old woman with several months of postprandial discomfort and abdominal fullness. CT and MRI revealed multiseptated cystic lesions with papillary excrescences. A left hemihepatectomy was performed. Histology showed a benign mucinous cystic tumor with ovarian-like stroma.
Abdominal Pain ; Cystadenocarcinoma ; Cystadenoma ; Cystadenoma, Mucinous ; Cystadenoma, Papillary ; Echinococcosis ; Female ; Humans ; Liver ; Magnetic Resonance Imaging ; Middle Aged ; Mucins ; Nausea ; Neuroendocrine Tumors

No abstract available.
Animals ; Brain* ; Glutamic Acid* ; Glycine* ; Rats*