Menkes disease is an X-linked recessively inherited neurodegenerative disorder of copper metabolism leading to death in early childhood. This disease is characterized by low serum, liver and brain Cu levels, whereas the Cu content in nearly all other organs is increased. The main clinical features are irregular kinky hair, characteristic face, progresssive degeneration of the central nervous system, bone changes, arterial rupture and thrombosis, and instability of temperature. We have experienced a case of Menkes disease in a eight months old male patient, who manifested by seizure, developmental delay, hypotonia, characteristic hair and face along with low levels of serum copper and ceruloplasmin. We report the characteristic findings of cranial MRI and MR angiography in this Menke's patient. In cranial MRI, he showed progressive brain atrophy with subdural hemorrhage and effusion. MR angiography revealed tortuosity of vessels clearly obviating necessity of more invasive conventional arteriography in diagnosis of this disease. This study suggested that cranial MRI and especially MR angiography might be helpful in making early diagnosis of this disease.
Angiography ; Atrophy ; Brain ; Central Nervous System ; Ceruloplasmin ; Copper ; Diagnosis ; Early Diagnosis ; Hair ; Hematoma, Subdural ; Humans ; Liver ; Magnetic Resonance Imaging ; Male ; Menkes Kinky Hair Syndrome ; Metabolism ; Muscle Hypotonia ; Neurodegenerative Diseases ; Rupture ; Seizures ; Thrombosis

Repeat cesarean section is one of the leading causes to make increasing a rate of cesarean section. Trial of labor TOL after cesarean section is attempt to reduce the rate of repeat cesarean section. Trial of labor has been well established as a safe alternative in carefully selected women with transverse scars in the lower uterine segment. This study was based on 104 cases of delivery with prior cesarean birth at the Chonbuk National University Hospital from January, 1991 to June, 1997. The results were as follows: 1) Among 1400 cases with previous cesarean delivery, trial of labor was done in 104 cases (7.4%). Among 104 cases, vaginal birth was successfully completed in 96 cases (92.3%). 2) Among 28 cases with PGE2, vaginal tablet, vaginal delivery was done in 23 cases (82.1%). 3) Among 22 cases with more than 4 of Bishop score, vaginal delivery was done in 21 cases (95.5%), and among 6 cases with less than 3 of Bishop score, vaginal delivery was done only 2 cases (33.3%). 4) There was no matemal death or uterine rupture in the cases of trial of labor, But in 2 cases, uterine dehiscence was observed in 4 cases and postpartal bleeding (more than 400ml) was developed. 5) In the cases of cesarean section, mean of hospital day was 7 days and medical fee was about 400,000 won. In the cases of vaginal birth after cesarean section, mean of hospital day was 2.6 days and medical fee was about 100,000 won. In condusion, vaginal birth after cesarean section is safe and effective alternative to elective repeat cesarean section and also the use of PGE, vaginal tablet is so effective to increase success rate of vaginal delivery. After all, positive these trials might decrease cesarean rate and increase maternal health and quality of medical care.
Cesarean Section ; Cesarean Section, Repeat ; Cicatrix ; Dinoprostone ; Fees, Medical ; Female ; Hemorrhage ; Humans ; Jeollabuk-do ; Maternal Health ; Parturition ; Pregnancy ; Prostaglandins E ; Trial of Labor ; Uterine Rupture ; Vaginal Birth after Cesarean* ; Vaginal Creams, Foams, and Jellies

PURPOSE: The purpose of this study was to evaluate the pattern and the magnitude of stress distribution in the supporting tissues surrounding three different types of implants(ITI, 3i, and Bicon implant system). MATERIAL AND METHOD: Photoelastic models were made with PL-2 resin(Measurements Group, Raleigh, USA) and three implants of each kind were placed in the mandibular posterior edentulous area distal to the canine . For non-splinted restorations, individual crowns were fabricated on three titanium abutments. For splinted restorations, 3-unit fixed partial dentures were fabricated. Photoelastic stress analyses were carried out to measure the fringe order around the implant supporting structure under simulated loaded conditions(15 lb, 30 lb). CONCLUSION: The results were as follows; 1. Regardless of the implant design, stresses were increased in the apex region of loaded implant when non-splinted restorations were loaded. While relatively even stress distribution occurred with splinted restorations. Splinting was effective in the second implant. 2. Strain around Bicon implant were lower than those of other implants, which confirmed the splinting effect. The higher the load, the more the stress occurred in supporting tissue, which was most obvious in the Bicon system. 3. Stress distribution in the supporting tissue was favorable in the ITI system, while the other side of 3i system tended to concentrate the stress in some parts.
Crowns ; Denture, Partial, Fixed ; Splints ; Titanium

PURPOSE: The purpose of this study was to evaluate the pattern and the magnitude of stress distribution in the supporting tissues surrounding three different types of implants(ITI, 3i, and Bicon implant system). MATERIAL AND METHOD: Photoelastic models were made with PL-2 resin(Measurements Group, Raleigh, USA) and three implants of each kind were placed in the mandibular posterior edentulous area distal to the canine . For non-splinted restorations, individual crowns were fabricated on three titanium abutments. For splinted restorations, 3-unit fixed partial dentures were fabricated. Photoelastic stress analyses were carried out to measure the fringe order around the implant supporting structure under simulated loaded conditions(15 lb, 30 lb). CONCLUSION: The results were as follows; 1. Regardless of the implant design, stresses were increased in the apex region of loaded implant when non-splinted restorations were loaded. While relatively even stress distribution occurred with splinted restorations. Splinting was effective in the second implant. 2. Strain around Bicon implant were lower than those of other implants, which confirmed the splinting effect. The higher the load, the more the stress occurred in supporting tissue, which was most obvious in the Bicon system. 3. Stress distribution in the supporting tissue was favorable in the ITI system, while the other side of 3i system tended to concentrate the stress in some parts.
Crowns ; Denture, Partial, Fixed ; Splints ; Titanium

Although neutrophilia can manifest from various causes, it is important to be able to distinguish chronic neutrophilic leukemia (CNL) from neutrophilic leukemoid reactions (NLR). In this paper, we describe four cases of leukocytosis with neutrophilia, including one case of CNL with a T618I mutation in colony stimulating factor 3 receptor (CSF3R) and three cases of NLR associated with malignancy or sepsis, which were initially suspected as CNL. Of the three NLR cases, one was associated with ovarian cancer, one with monoclonal gammopathy of undetermined significance and one with multiple myeloma with sepsis. This study demonstrated that confirming the clonality of myeloid cells with CSF3R T618I could contribute to making an accurate differential diagnosis between CNL and NLR in patients with solid cancers or plasma cell neoplasms caused by paraneoplastic syndromes and/or infection.
Colony-Stimulating Factors ; Diagnosis, Differential ; Humans ; Leukemia, Neutrophilic, Chronic* ; Leukemoid Reaction* ; Leukocytosis ; Monoclonal Gammopathy of Undetermined Significance ; Multiple Myeloma ; Myeloid Cells ; Neoplasms, Plasma Cell ; Neutrophils* ; Ovarian Neoplasms ; Paraneoplastic Syndromes ; Sepsis

We describe two cases of multiple lymphomatous polyposis in the gastrointestinal tract from the esophagus to the rectum. Clinical findings, histopathologic and immunohistochemical findings in paraffin embedded tissue are discussed. It is important to recognize this rare form of gastrointestinal lymphoma because of the prognostic and therapeutic implications.
Antigens, CD/analysis ; Gastrointestinal Neoplasms/immunology/*pathology ; Humans ; Immunohistochemistry ; Intestinal Polyps/immunology/*pathology ; Lymphoma/immunology/*pathology ; Male ; Middle Aged ; Polyps/immunology/*pathology

Heterotopic pregnancy, simultaneous intrauterine and extrauterine gestations, is a relatively rare conditon with an estimated incidence of 1 in 30,000 pregnancies in a natural cycle. But the incidence of heterotopic pregnancies has been increased because of rising incidence of pelvic inflammatory disease, pelvic surgery, intrauterine device, the use of various ovulation induction and assisted reproductive technologies, such as in vitro fertilization and embryo transfer, gamate intrafallopian insemination. The early diagnosis of heterotopic pregnancy is very difficult. So there is a higher maternal morbidity, mortality and fetal loss. Thus careful pelvic examination, combined with serial beta hCG determinations and transvaginal sonography to evaluate the adnexal region are important. We experienced a case of 34-years-old multiparous women with heterotopic pregnancy in natual cycle confirmed by surgical removal of ruptured right tubal pregnancy and sonographic finding of the intrauterine pregnancy, which carried the intrauterine pregnancy to term delivery without complication at 39+5 weeks of gestation, and report this case with a brief review of the literature.
Early Diagnosis ; Embryo Transfer ; Female ; Fertilization in Vitro ; Fertilization* ; Gynecological Examination ; Humans ; Incidence ; Insemination ; Intrauterine Devices ; Mortality ; Ovulation Induction ; Pelvic Inflammatory Disease ; Pregnancy ; Pregnancy, Heterotopic* ; Pregnancy, Tubal ; Reproductive Techniques, Assisted ; Ultrasonography

No abstract available.
Sertoli-Leydig Cell Tumor*

PURPOSE: There were a lot of reports regarding associations of polymorphisms in the estrogen receptor alpha (ESR1). with many disorders. But, those with constitutional delay of growth and puberty (CDGP) are not known. Our aim is to find out any association between CDGP and ESR1. METHODS: In a total of 27 subjects, we compared 7 CDGP patients with 20 healthy controls with their heights and sexual maturity rates were within normal range. We selected three single nucleotide polymorphisms from intron 1 of ESR1 (rs3778609, rs12665044, and rs827421) as candidates, respectively. RESULTS: In genotype analyses, the frequency of G/G genotype at rs827421 in intron 1 of ESR1 was increased in CDGP boys (P=0.03). CONCLUSION: The genetic variation of ESR1 can be a contributing factor of tempo of growth and puberty.
Cyclic N-Oxides ; Estrogen Receptor alpha ; Estrogens ; Genetic Variation ; Genotype ; Humans ; Introns ; Polymorphism, Single Nucleotide ; Puberty ; Reference Values

PURPOSE: There were a lot of reports regarding associations of polymorphisms in the estrogen receptor alpha (ESR1). with many disorders. But, those with constitutional delay of growth and puberty (CDGP) are not known. Our aim is to find out any association between CDGP and ESR1. METHODS: In a total of 27 subjects, we compared 7 CDGP patients with 20 healthy controls with their heights and sexual maturity rates were within normal range. We selected three single nucleotide polymorphisms from intron 1 of ESR1 (rs3778609, rs12665044, and rs827421) as candidates, respectively. RESULTS: In genotype analyses, the frequency of G/G genotype at rs827421 in intron 1 of ESR1 was increased in CDGP boys (P=0.03). CONCLUSION: The genetic variation of ESR1 can be a contributing factor of tempo of growth and puberty.
Cyclic N-Oxides ; Estrogen Receptor alpha ; Estrogens ; Genetic Variation ; Genotype ; Humans ; Introns ; Polymorphism, Single Nucleotide ; Puberty ; Reference Values