The clinical and pathological features of a paraganglioma arising in the cauda equina is described and compared with previous reports. The right microscopic fetures were similar to those of paragangliomas from other sites, with a 'Zellballen' pattern of cells containing arzyrophil granules. Immunohistocytochemical stains for neurone specific enolase, S-100 protein, cytokeratin were positive, but stains for glial fibrillary acidic protein were negative. Electron microscopy showed densely staining membrane-bound granules, cilia like structures and fibros bodies in the cytoplasm. The last two features only occur in paragangliomas from this site. The pathological findings suggest that paragangliomas in this site arise from pre-existing paraganglia, possibly of the visceral autonomic group.

Endodermal sinus tumors are an uncommon malignancy in children and occur most often in the gonads and sacrococcygeal region. In these locations, thy are found as primary tumors ; in teratoma they form part of the malignant component. We present CT and MR imaging findings in two cases of endodermal sinus tumor occurring after the resection of a mature benign cystic sacrococcygeal teratoma during the neonatal period. Awareness of the transformation of a benign cystic teratoma to a different type of malignant germ cell tumor may be helpful in the evaluation of sacrococcygeal masses.
Child ; Endoderm* ; Endodermal Sinus Tumor* ; Gonads ; Humans ; Magnetic Resonance Imaging ; Neoplasms, Germ Cell and Embryonal ; Sacrococcygeal Region ; Teratoma*

Endodermal sinus tumors are an uncommon malignancy in children and occur most often in the gonads and sacrococcygeal region. In these locations, thy are found as primary tumors ; in teratoma they form part of the malignant component. We present CT and MR imaging findings in two cases of endodermal sinus tumor occurring after the resection of a mature benign cystic sacrococcygeal teratoma during the neonatal period. Awareness of the transformation of a benign cystic teratoma to a different type of malignant germ cell tumor may be helpful in the evaluation of sacrococcygeal masses.
Child ; Endoderm* ; Endodermal Sinus Tumor* ; Gonads ; Humans ; Magnetic Resonance Imaging ; Neoplasms, Germ Cell and Embryonal ; Sacrococcygeal Region ; Teratoma*

OBJECTIVE: Peripartum emergency cesarean hysterectomy of 28 cases at our hospital for 10years were reviewed that the clinical indication, history, and annual incidence change. METHODS: A retrospective descriptive analysis from January, 1988 to December, 1997 was carried out. RESULTS: There were 28 cases of emergency cesarean hysterectomy identified during this period among total 24, 689 deliveries. The annual incidence of emergency cesarean hysterectomy was significantly declined statistically(p<0.005). Cesarean hysterectomy was performed in 20 of 6, 671 cesarean section(0.30%) and in 8 of 18, 018 vaginal deliveries(0.04%), so more frequently after cesarean section than vaginal delivery. The cesarean hysterectomy rate was more frequent in multiparous women(0, 22%) than in nulliparous women(0, 02%). The most common indication of cesarean hysterectomy was 13 cases of uterine atony(46%), followed by 5 cases uterine rupture(18%), 5 cases of placenta previa with placenta accreta(18%), 3 cases of placenta previa(11%), 2 cases of uterine myoma(7%). No significant difference in length of operating time, amount of blood loss and operative complications were found between total abdominal hysterectomy and subtotal hysterectomy. The all patient who had cesarean hysterectomy recieved from 1200ml to 15840ml of blood transfusion with a mean of 3673ml. In aspect of fetal outcome, 3 cases of FDIU(Fetal death in utero) in uterine rupture and 1 case of stillbirth in preterm were found. The maternal complications were wound disruption, DIC, wound hematoma, ureter injury, vaginal stump bleeding, UTI(Urinary tract infection) and retroperitoneal bleeding, CONCLUSION: We conclude that the incidence of emergency cesarean hysterectomy declined with prediction of antenatal risk factor, preparing of sufficient fresh blood, prophylactic antibiotics, vigorous uterotonics and close observation after delivery.
Anti-Bacterial Agents ; Blood Transfusion ; Cesarean Section ; Dacarbazine ; Emergencies* ; Female ; Hematoma ; Hemorrhage ; Humans ; Hysterectomy* ; Incidence ; Peripartum Period* ; Placenta ; Placenta Previa ; Pregnancy ; Retrospective Studies ; Risk Factors ; Stillbirth ; Ureter ; Uterine Rupture ; Wounds and Injuries

BACKGROUND: Reactive oxygen species (ROS) induce lipid peroxidation and tissue damage in the isolated rabbit thoracic aorta. The aim of this study was to explore the influence of the propofol and midazolam against ROS in the isolated rabbit thoracic aortic endothelium. METHODS: Eighteen white male rabbits (weighing 2.0-2.5 kg) were used. The thoracic aorta was dissected free and cut into rings (3-4 mm) and then suspended in a organ bath filled with 10 ml Krebs solution bubbled with 5% CO2 95% O2 at 37 degrees C. Aortic rings were then equilibrated for 90 min, and a resting tension of 1.5 g was applied. The Krebs solution was changed every 15 min. Isometric tension was recorded with transducer coupled to a data acqusition system (Biopac Inc. USA) on a PC. After precontraction with norepinephrine (NE, 10(-6)M), changes in tension were measured following the cumulative administration of acetylcholine (ACh 3x10(-7), 10(-6) and 3x10(-6)M) and nitroglycerin (NTG, 10(-5)M). Data are expressed as percentage of the 10 5 M NTG-induced relaxation (ACh/NTG). The ACh/NTG, before and after electrolysis were defined as the control and the experimental groups. The aortic rings were pretreated with propofol (3x10(-5), 10(-4), 3x10(-4) and 5.7x10(-4) M, n = 8, 10, 15, 13), midazolam (10(-4)M, n = 7), catalase (1,000 U/ml, n = 12), mannitol (3x10(-4)M, n = 5) or not pretreated group (Free, n = 6). After 30 minutes, the aortic rings were exposed to ROS generated by electrolysis (DC 9 V, 20 mA, aortic rings 1 cm away from electrode) in Krebs solution for 2 minutes, which was then changed for physiologic buffered salt solution. The aortic rings were precontracted with NE and vasorelaxation was induced with ACh and NTG at the above mentioned concentrations. RESULTS: Propofol produced vasorelaxation of NE-precontracted thoracic aorta in a dose-dependent fashion in all groups of propofol (3x10(-5), 10(-4), 3x10(-4) and 5.7x10(-4)M) even after ROS attack (P < 0.05 vs control value). Catalase produced vasorelaxation after ROS attack (P < 0.05 vs control value).On the other hand, ACh-induced significant endothelium-dependent vasorelaxation were not observed in the midazolam or mannitol pretreated group or the non-pretreated group (P <0.05 vs control group). CONCLUSIONS: These findings suggest that propofol and catalase preserve ACh induced endothelium-dependent vasorelaxation and that propofol has a concentration dependent ROS scavenging effect like catalase.
Acetylcholine ; Aorta, Thoracic ; Baths ; Catalase ; Electrolysis ; Endothelium ; Hand ; Humans ; Lipid Peroxidation ; Male ; Mannitol ; Midazolam ; Nitroglycerin ; Norepinephrine ; Propofol* ; Rabbits ; Reactive Oxygen Species* ; Relaxation ; Transducers ; Vasodilation

Joubert syndrome and Joubert syndrome-related disorders (JSRDs) are rare autosomal recessive or X-linked disorders characterized by cerebellar vermis hypoplasia and a brain stem malformation, which presents as the “molar tooth sign” in magnetic resonance imaging (MRI). JSRDs are a group of clinically heterogeneous conditions that exhibit neurological manifestations and multiple organ involvement. JSRDs are also genetically heterogeneous, and approximately 20 causative genes that account for 45% of JSRDs have been identified. A 7-yr-old boy visited Wonkwang University Sanbon Hospital with the following presentations: no ocular fixation, ataxia, growth retardation, and hypotonia. Physical examination revealed facial dysmorphism, spindle shaped fingers, and height (99 cm) and weight (13 kg) below the third percentile. Ophthalmic examination revealed retinal dystrophy. A diagnosis of JSRDs was made based on clinical and brain MRI findings. We found two heterozygous variants c.2945 G>T; p.Arg982Met (G>T) and c.2216dupA; p.Phe740Valfs*2 (dupA) in AHI1, and a heterozygous c.3973C>T; p.Arg1325Trp (C>T) variant in KIF7 by whole exome sequencing (WES). Genetic analysis on the proband's father revealed that he had both AHI1 variants, but did not have the KIF7 variant, which was inconsistent with autosomal recessive inheritance. Therefore, the G>T variant and C>T variant were presumed to be of “uncertain significance.” Furthermore, one novel dupA variant was interpreted as “pathogenic,” while the second allele was not detected. Caution should be exercised while interpreting the significance of variants detected by WES. In addition, the involvement of genes other than the 20 known ones will require further investigation to elucidate the pathogenesis of JSRDs.
Alleles ; Ataxia ; Brain ; Brain Stem ; Cerebellar Vermis ; Child* ; Diagnosis ; Exome* ; Fathers ; Fingers ; Fixation, Ocular ; Humans ; Magnetic Resonance Imaging ; Male ; Muscle Hypotonia ; Neurologic Manifestations ; Physical Examination ; Retinal Dystrophies ; Tooth ; Wills

PURPOSE: The study was performed to investigate the IgG subclasses in acute febrile phase of Kawasaki disease(KD) to find correlation between selective IgG4 deficiency and incidence of KD patients. METHODS: The patients group consisted of 64 cases in acute phase of KD hospitalized from Jan. 1996 to Jul. 1999, and control group consisted of age matched 36 cases who admitted without KD. The obtained sera were measured the level of total IgG, IgM, IgA and IgG subclasses(IgG1, IgG2, IgG3, IgG4) by using Enzyme Immuno Assay and Single Radial Immuno-Diffusion method. RESULTS: The sex ratio of male to female was 1.4:1.0, and febrile days before admission was 4.6+/-2.4 days. Total levels of IgG, IgM and IgA were normal range in both groups. The levels of IgG1, IgG2 and IgG3 were not specific changes but significantly decreased the level of IgG4 in patients group as compared with control group(P<0.05). CONCLUSION: Selective decreased the levels of serum IgG4 can be one of factors to increasing the incidence of KD.
Female ; Humans ; Immunoglobulin A ; Immunoglobulin G* ; Immunoglobulin M ; Incidence ; Male ; Mucocutaneous Lymph Node Syndrome* ; Reference Values ; Sex Ratio

OBJECTIVES: Previously, we sought to compile a list of genes expressed during early folliculogenesis by using cDNA microarray to investigate follicular gene expression and changes during primordialprimary follicle transition and development of secondary follicles (Yoon et al., 2005). Among those genes, a group of genes related to the cell size growth was characterized during the ovarian development in the present study. METHODS: We determined ovarian expression pattern of six genes related to the cell size growth (cyr61, emp1, fhl1, socs2, wig1 and wisp1) and extended into CCN family (connective tissue growth factor/cysteine-rich 61/nephroblastoma-overexpressed), ctgf, nov, wisp2, wisp3, including cyr61 and wisp1 genes. Expression of mRNA and protein according to the ovarian developmental stage was evaluated by in situ hybridization, and/or semiquantitative reverse transcriptase polymerase chain reaction (RT-PCR), and immunohistochemistry, respectively. RESULTS: Among 6 genes related to the cell size growth, cyr61 and wisp1 mRNA was detected only in oocytes in the postnatal day5 mouse ovaries. cyr61 mRNA expression was limited to the nucleolus of oocytes, while wisp1 was expressed in the cytoplasm and nucleolus of oocytes, except nucleus. cyr61 mRNA expression, however, was found in granulosa cells from secondary follicles. The rest 4 genes in the cell size growth group were detected in oocytes, granulosa and theca cells. Cyr61 and Wisp1 proteins were expressed in the oocyte cytoplasm from primordial follicle stage. Especially, Cyr61 protein was detected in pre-granulosa cells, Wisp1 protein was not. By using RT-PCR, we evaluated and decided that Cyr61 protein is produced by their own mRNA in pre-granulosa cells that was not detected by in situ hybridization. cyr61 and wisp1 genes are happen to be the CCN family members. The other members of CCN family were also studied, but their expression was detected in oocytes, granulose and theca cells. CONCLUSIONS: We firstly characterized the ovarian expression of genes related to the cell size growth and CCN family according to the early folliculogenesis. Cyr61 protein expression in the pre-granulosa cells is profound in meaning. Further functional analysis for cyr61 in early folliculogenesis is under investigation.
Animals ; Cell Enlargement* ; Cell Size* ; Cysteine-Rich Protein 61 ; Cytoplasm ; Female ; Gene Expression ; Genes, vif ; Granulosa Cells ; Humans ; Immunohistochemistry ; In Situ Hybridization ; Mice* ; Oligonucleotide Array Sequence Analysis ; Oocytes ; Ovary ; Reverse Transcriptase Polymerase Chain Reaction ; RNA, Messenger ; Theca Cells

No abstract available.

Prurigo pigmentosa(PP) is a rare dermatosis with an unknown cause characterized by intensely pruritic papules that resolve with reticulate pigmentation. It is a disease entitity commonly reported in Japan but seemingly rare in other parts of the world. We report 5 cases that clinically and histopathologically fulfilled the characterisitics of PP and were treated with dapsone resulting in significant clinical improvement.
Dapsone ; Japan ; Pigmentation ; Prurigo* ; Skin Diseases