Tuberous sclerosis is a systemic disorder of hamartoma format on includes facial angiofibromas, subungal fibromas, hypomelanic maciles Shagreens patches. The earliest skin manifestation of tuberous sileiule, but it may appear later. We present a case of fibrous plaqu a recognized skin signs. Forehead plaque can be the earliest skin near in many organs. Skin involvement less forehead fibrous plaques, and vsis is usually hypornelanotic inacipearing before any of these more festation of tuberous sclerosis. Its presence may lead to early diagnosis and appropriate genetic couise hng hut may be a poor prognostic sign.
Angiofibroma ; Early Diagnosis ; Fibroma ; Forehead* ; Hamartoma ; Skin ; Skin Manifestations ; Tuberous Sclerosis*

Oil granuloma is a foreignbody reaction which occurs after injection of lipid usually for cosmetic or therapeutic reasons. I present a case of eyelid oil granuloma caused by accidental high pressure injury in a 39-year-old man. The patient had a 2 x 0.5cm sized, firm, normardear subcutaneous nodule on the Rt. upper eyelid. Histopathologic examination showed a characteris swiss-cheese appearance. Eyelid oil granuloma caused by high pressure injury is very rare and my case is the first described in Korean medical literatures.
Adult ; Eyelids* ; Granuloma* ; Humans

Neurilemmomas of the trachea are extremely rare. The most common site of them is the distal third of the trachea and the age of the patients at presentation varied from 6 to 78 years old. They usually have a freqeuntly very long natural history, causing symptoms only after they have attained a considerable size. We experienced a case of near-total obstruction of the trachea by an intraluminal sessile neurilemmoma. The patient was a 66-year-old man with 2-year history of progressive exertional dyspnea and had several episodes of pneumonitis associated with productive cough. Grossly, the tumor was a well-circumscribed mass. Microscopically, typical cellular Antoni A and myxoid Antoni B areas were revealed.
Aged ; Cough ; Dyspnea ; Humans ; Natural History ; Neurilemmoma* ; Pneumonia ; Trachea

BACKGROUND: Soluble IL-2R, soluble CD8 and TNF-alpha are elevated in sera of some patients with hematological malignancies, and a marked elevation of these cytokines could be used to assess disease activity and prognosis in this malignancy group. METHODS: The serum levels of sIL-2R, sCD8 and TNF-alpha were assessed in 28 patients with myelodysplastic syndrome (MDS) and 32 patients with acute myeloid leukemia (AML), and 39 cases of healthy control subjects to define clinical usefulness as prognostic markers by sandwich enzyme immunoassay. RESULTS: In MDS patients, serum sIL-2R levels were significantly higher as compared with controls, and a more pronounced increase of serum sIL-2R levels was found in patients with RAEB RAEB-t and CMML as compared with RA and RARS. Serum sCD8 levels were higher as compared with controls, but not related with FAB classification. In patients with leukemic conversion. sCD8 levels tended to be higher as compared with patients with non-conversion. The sIL-2R levels of AML patients were significantly higher than controls, and a significant correlation was detected between the levels of sIL-2R and WBC counts. Higher sIL-2R levels( >2000 U/ml) tended to affect both complete remission rate and survival. Serum sCD8 levels were higher than controls, but not related to FAB classification. No differences of serum TNF-alpha levels were detected as compared with healthy controls. CONCLUSIONS: From these results, this study indicates that serum sIL-2R and sCD8 are significantly increased in some patients with MDS and AML, and increased levels of serum sIL-2R and sCD8 may be useful for predicting prognosis of these patients.
Anemia, Refractory, with Excess of Blasts ; Classification ; Cytokines ; Hematologic Neoplasms ; Humans ; Immunoenzyme Techniques ; Leukemia, Myeloid, Acute* ; Myelodysplastic Syndromes* ; Prognosis ; Tumor Necrosis Factor-alpha*

PURPOSE: Henoch-Schonlein purpura(HSP) nephritis has been reported to vary from 25 to 50% among HSP patients and is a common cause of chronic glomerulonephritis in children. In our study, we evaluated the distribution and the association of the Insertion/Deletion(I/D) polymorphism of angiotensin converting enzyme(ACE) gene with clinical manifestations, particularly proteinuria in children with HSP nephritis, compared with that in HSP. METHODS: ACE gene polymorphism was determined in children with HSP nephritis(n=33) and HSP(n=28) who were diagnosed in Busan Paik hospital from January 1996 to June 2001. The I/D polymorphism of ACE gene was determined by PCR amplication of genomic DNA. RESULTS: The ACE I/D genotype frequency was DD : 25%, ID : 50%, II : 25% in HSP and DD : 24 %, ID : 46%, II : 30% in HSP nephritis, there was no significant difference in the genotype and allele frequencies between two groups. When statistical analysis was done according to the presence of D allele, the amount of 24-hour urinary protein excretion and the incidence of moderate to heavy proteinuria(>500 mg/m2/day) at onset and last follow-up were higher in DD/ID genotype than in those in II genotype, but these differences were not statistically significant. CONCLUSION: We suggest a lack of association between I/D polymorphism of ACE gene and clinical manifestations in children with HSP nephritis. However, further follow-up studies based on a sufficient number of patients and long term follow up periods are necessary to confirm the role of I/D polymorphism of ACE gene in children with HSP nephritis.
Alleles ; Angiotensins* ; Busan ; Child* ; DNA ; Follow-Up Studies ; Gene Frequency ; Genotype ; Glomerulonephritis ; Humans ; Incidence ; Nephritis* ; Peptidyl-Dipeptidase A* ; Polymerase Chain Reaction ; Proteinuria ; Purpura, Schoenlein-Henoch*

3-Deazaadenosine (DZA), one of the potent inhibitors of S-adenosylhomocysteine hydrolase, is known to possess several biological properties including an induction of apoptosis. To evaluate a possibility that DZA may be utilized for the treatment of human leukemia, we studied molecular events of cell death induced by DZA in human leukemia HL-60 and U-937 cells. DZA induced a specific cleavage of poly ADP-ribose polymerase (PARP) and an activation of the cysteine protease caspase-3/CPP32 which is known to cleave PARP. DZA-mediated nuclear DNA-fragmentation was completely blocked in the presence of a universal inhibitor of caspases (z-VAD-fmk) or the specific inhibitor of caspase-3 (z-DEVD-fmk) unlike of cycloheximide (CHX). DNA fragmentation was preceded by the lowering of c-myc mRNA in the DZA treated cells. In addition, DZA-induced apoptosis was blocked by pretreatment with adenosine transporter inhibitors such as nitrobenzylthioinosine (NBTI) and dipyridamole (DPD). Taken together, these results demonstrate that DZA-induced apoptosis initiated through an active transport of DZA into human leukemia cells, is dependent on the caspase-3-like activity without de novo synthesis of proteins and possibly involves c-myc down-regulation.
Adenosine/metabolism ; *Apoptosis ; Biological Transport, Active ; Carrier Proteins/metabolism ; Caspases/*metabolism ; Down-Regulation ; Enzyme Activation ; Genes, myc ; HL-60 Cells ; Human ; Leukemia, Promyelocytic, Acute/*drug therapy ; Thioinosine/*analogs & derivatives/pharmacology ; Transcription Factors/genetics ; Tubercidin/*pharmacology ; U937 Cells

OBJECTIVE: To determine the incidence of embryo retention (ER) in the transfer catheter following embryo transfer (ET) in blastocyst transfer and investigate whether retransferring retained embryos has an impact on reproductive outcomes in patients undergoing in vitro fertilization-ET. METHODS: We retrospectively analyzed the records of 1,131 blastocyst transfers, which comprised 223 single blastocyst transfer (SBT) and 908 double blastocyst transfer (DBT) cycles. Each SBT and DBT group was classified depending on whether ET was performed without retained embryos in the catheter during the first attempt (without-ER group) or whether any retained embryos were found following ET (ER group) for the purpose of comparing reproductive outcomes in a homogenous population. RESULTS: The overall incidence of finding retained embryos was 2.8% (32/1,131). There were no retained embryos in SBT cycles. In DBT cycles, implantation rates (30.0% vs. 26.6%), positive β-hCG rates (57.2% vs. 56.2%), clinical pregnancy rates (45.3% vs. 46.9%), and live birth rates (38.9% vs. 43.8%) were not significantly different between the without-ER and ER groups. There were no significant differences in the mean birth weight (g) 2,928.4±631.8 vs. 2,948.7±497.8 and the mean gestational age at birth (269.3±17.2 days vs. 264.2±25.7 days). A total of nine cases of congenital birth defects were found in this study population. Eight were observed in the without-ER group and one in the ER group. CONCLUSION: Our results suggest that retransfer of retained embryos does not have any adverse impact on reproductive outcomes in blastocyst transfer cycles. Furthermore, our results support finding that SBT might be advantageous for decreasing the incidence of retained embryos in catheters.
Birth Weight ; Blastocyst* ; Catheters ; Congenital Abnormalities ; Embryo Transfer* ; Embryonic Structures* ; Fertilization in Vitro ; Gestational Age ; Humans ; In Vitro Techniques ; Incidence ; Live Birth ; Parturition ; Pregnancy Rate ; Retrospective Studies

BACKGROUND: The current study aimed at elucidating the anatomical basis for a supraclavicular approach for central venous catheterization using three-dimensional computed tomography (3D-CT). METHODS: Retrospective review of CT images from 60 adult patients with normal body build was performed using 3D-CT reconstruction. Right-sided approach was assumed, and the skin entry point was decided as a point above the subclavian vein at the supraclavicular fossa. Measured parameters were; angles of the clavicle (Aclv) and the subclavian vein (Ascv) to the coronal plane, the distance from the clavisternomastoid angle to the skin entry point (Dse), the optimal angle of needle insertion (Ains) targeting the confluence between the internal jugular vein and the subclavian vein, the distance from the skin entry point to the confluence (Dconf), and the diameter of the confluence (Dia). Descriptive statistics were performed for the measured values. Correlation test was performed between Ascv and Aclv. RESULTS: Measured values were; Aclv = 8.0 degrees, Ascv = 5.7 degrees, Dse = 12.5 mm, Ains = 40 degrees, Dconf = 20.5 mm, and Dia = 18.1 mm. Ascv demonstrated positive correlation with corresponding Aclv (r = 0.494, P < 0.001). CONCLUSIONS: Optimal guideline for the supraclavicular approach can be provided via 3D-CT investigation. Forty degrees of needle direction to the sagittal plane and parallel to the posterior surface of the clavicle, at 1.3 cm posterior to the clavisternomastoid angle insures proper puncture of the confluence without anticipated complications.
Adult ; Catheterization ; Catheterization, Central Venous* ; Central Venous Catheters* ; Clavicle ; Humans ; Jugular Veins ; Needles ; Punctures ; Retrospective Studies ; Skin ; Somatotypes ; Subclavian Vein

We report a case of elephantiasis neurofibromatosa in a 50-year-old female patient treated by in walking excision. She had difficulty in walking due to a huge mass on the abdomen. After surgical excision, she is leading a normal life and is very satisfied.
Abdomen ; Elephantiasis* ; Female ; Humans ; Middle Aged ; Neurofibromatoses* ; Walking

No Abstract available.
Academic Medical Centers* ; Infection Control*