No abstract available.
Animals ; Embryonic Structures* ; Mice*

OBJECT: Pre-ALL is a very rare preteukemic state, which percedes acute lymphocytic leukemia, while MDS(pre-ANLL), usually the well-known type of preleukemic state, precedes acute non-lymphocytic leukemia. Initially it shows transient pancytopenia without any evidence of leukemia in bone marrow findings, followed by acute lymphocytic leukemia after recovery from pancytopenia of a short period within weeks or months. We report a case with pre-ALL in childhood. CASE: A 15-month-old male baby was admitted with the complaints of fever and cough for 5 days and pallor for 2 weeks prior to admission. On admission, CBC showed pancytopenia without any evidence of leukemia, which was recovered spontaneously in a short period, and then was followed by acute lymphocytic leukemia of CALLA negative, early pre-B cell type. During antileukemic chemotherapy, he had suffered from severe bacterial infections and was finally died of sepsis 8 months after first admission. CONCLUSION: We report a case of pre-ALL in childhood, which was preceded by CALLA negative, early pre-B cell ALL, with a review of the literatures, briefly.
Bacterial Infections ; Bone Marrow ; Cough ; Drug Therapy ; Fever ; Humans ; Infant ; Leukemia ; Male ; Pallor ; Pancytopenia ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Precursor Cells, B-Lymphoid ; Sepsis

We treated a case of extramammary Paget's disease that affected the scalp of a 45-year-old female. It is extremely rare that the disease arises in areas other than the anogenital region and the axillae. The lesion was a round erythematous oozing crusted hairless patch. Histopathologically, many pagetoid cells were found within the epidermis and dermis. The cytoplasms of these cells stained with alcian blue at pH 2.5, CEA, EMA and low-molecular-weight-cytokeratin. The patient underwent a wide local excision. We used a mapping technique to reveal the distribution of microscopically involved lesions.
Alcian Blue ; Axilla ; Cytoplasm ; Dermis ; Epidermis ; Female ; Humans ; Hydrogen-Ion Concentration ; Middle Aged ; Paget Disease, Extramammary* ; Scalp*

No abstract available.

OBJECTIVE: Linkage analysis is a very useful method for prenatal diagnosis of Hemophilia B, especially when a mutation was not identified. Seven polymorphic markers were studied in Korean populations to evaluate the efficiency for prenatal and carrier diagnosis. METHODS: Subjects of this study was 100 healthy Korean women (200 X-chromosomes). Polymerase chain reacton-restriction fragment length polymorphism (PCR-RFLP) method was used to detect SalI, MseI, NruI, DdeI, XmnI, TaqI and HhaI polymorphisms. RESULTS: SalI (-) allele showed the frequency of 0.355 and SalI(+) allele 0.645. MseI(-) allele was 0.645 in frequency and MseI(+) allele was 0.355. SalI and MseI polymorphisms were in complete linkage disequilibrium. And no increase was expected in overall heterozygosity with these two polymorphisms. NruI(-) allele frequency was 0.855 and NruI(+) was 0.145. There was no polymorphism of DdeI, XmnI and TaqI marker systems in Korean population. In HhaI polymorphism, allele frequencies were estimated that HhaI(-) is 0.82 and HhaI(+) is 0.18. CONCLUSION: Only SalI, NruI and HhaI polymorphisms are useful for the diagnosis of hemophilia B in Korean population. Expected heterozygosity for above 3 poylmorphic markers was estimated to be 0.723, and 71 of 100 female subjects were heterozygous for at least one marker system. Korean population showed relatively low extent of polymorphisms compared to Caucasians, Blacks and Japanese. For the effective prenatal diagnosis of hemophilia B with linkage analysis, other polymorphic markers should be evaluated.
African Continental Ancestry Group ; Alleles ; Asian Continental Ancestry Group ; Diagnosis ; Factor IX* ; Female ; Gene Frequency ; Hemophilia B ; Humans ; Linkage Disequilibrium ; Prenatal Diagnosis

No abstract available.
Breast Neoplasms* ; Breast*

Sterilization has received much attention in orthodontic practices over the past several years. The present study was undertaken to investigate the effects of sterilization on the physical properties of orthodontic pliers-AEZ, Unitek, and Dentronix ligature cutters. This study was designed to examine the tips of ligature cutters before and after 200 and 400 sterilization cycles using the Bowmar RHT-1000, the Dentronix DDS-5000, and the Eschmann SES-2000. The tip surface and the fracture surface were observed with a scanning electron microscope. The microstructure was observed with an optical microscope. The hardness test was carried out with the micro-Vickers hardness tester and the Rockwell C Scale hardness tester. The chemical composition was analyzed_ by energy dispersive X-ray spectrometer. The results of this study were as follows: 1. The number and the size of corrosion products on the tip surface and the proportion of cleavage planes in fractured specimen increased, but the hardness of the tip decreased in proportion to sterilization cycles. From these observations, it was considered that mechanical properities decreased in proportion to sterilization cycles. 2. The number and the size of chromium carbides increased in proportion to sterilization cycles. Coarse microstructure decreased mechanical properities. 3. The AEZ and Unitek ligature cutters were Fe-Cr stainless steels, but the Dentronix ligature cutter was Co-Cr alloy. There were many differences among manufactures, but the chemical composition was not changed after sterilization cycles. 4. The tip edge of ligature cutter used in a clinic revealed microcracks with the SEM observation. Clinical experience confirmed that ligature cutters were gradually degraded by sterilization.
Alloys ; Chromium ; Corrosion ; Hardness ; Hardness Tests ; Ligation ; Stainless Steel ; Sterilization*

No abstract available.
Joints*

Bartter's syndrome is characterized by hyperreninemia, hyperaldosteronism, hypokalemic hypochlorenlic alkalosis, normal blood pressure, juxtaglomerular apparatus hyperplasia, general weakness, and muscle weakness. We experienced a case of hypokalemic myopathy associated with Bartter's syndrome in 15 years old male. He had experienced paroxysmal muscle weakness without sensory change and myalgia since 10 years old. Subsequently, he had complaints of progressive muscle weakness, especially proximal muscles. Prominent juxtaglomerular apparatus with cellular proliferation biopsy was seen in the kidney. And there were mild perivascular inflammatory cell infiltration, small degenerating and/or regenerating muscles fibers, and normal muscle fiber distribution without evidence of chronic myopathy in the muscle biopsy. The patient was sucessfully managed with indomethacin and oral potassium chloride.
Adolescent ; Alkalosis ; Bartter Syndrome* ; Biopsy ; Blood Pressure ; Cell Proliferation ; Child ; Humans ; Hyperaldosteronism ; Hyperplasia ; Indomethacin ; Juxtaglomerular Apparatus ; Kidney ; Male ; Muscle Weakness ; Muscles ; Muscular Diseases* ; Myalgia ; Potassium Chloride

Hereditory Sensory Autonomic Neuropathy(HSAN) is variable rare disorder. So the classification of HSAN could be somewhat unsettled. There are intermingled overlap variants of HSAN in view of clinical manifestations and pathologic findings. Five types of HSAN have been described by Dyck(1993). Type I is dominantly inherited and affects both myelinated(MFs) and unmyetinated fibers(Ufs). Type II is recessively inherited and nerve biopsies show total absence of MFs but presence of Ufs. Type III is a recessive inherited dysautonomia. Type IV is characterized by insensitive to pain anhidrosis, and mild mental retardation with virtually absence of Ufs. Type V affects small MFs. We report a 5 year-old girl who presented with congenital insensitivity to pain, anhidrosis with mild mental retardation. In sural nerve biopsy, Ufs were virtually absent by electron microscopy. We reconfirmed previous pathologic findings in sural nerve of HSAN IV.
Biopsy ; Child, Preschool ; Classification ; Female ; Hereditary Sensory and Autonomic Neuropathies ; Humans ; Hypohidrosis* ; Intellectual Disability ; Microscopy, Electron ; Pain Insensitivity, Congenital* ; Primary Dysautonomias ; Sural Nerve