No abstract available.
Embryonic Structures* ; Humans* ; Somites*

Vaginal agenesis is rare gynecologic condition, and the most common etiology is Mayer-Rokitansky-Kster-Hauser (MRKH) syndrome, characterized by the absence of uterus and vagina and presence of normal ovaries and tubes. In such patients, the evaluation for associated malformations as well as careful non-surgical and surgical approach are essential. The neovaginoplasty is an important issue for these patients in regard of functional and psychological standpoint. There are many options available for creation of neovagina. We report six cases of laparoscopic assisted neovaginoplasty using pelvic peritoneal flap.
Abnormalities, Multiple ; Female ; Humans ; Kidney ; Laparoscopy ; Mullerian Ducts ; Ovary ; Somites ; Spine ; Uterus ; Vagina

OBJECTIVE: To determine whether there is a re-closure capacity of the open neural tube defect(ONTD) and to characterize its re-closing process, the morphological changes and the re-closure rate of a surgically induced ONTD are examined chronologically in early chick embryos. METHODS: Embryos of Hamburger and Hamilton stage 18-19 were used. The posterior roof of the central canal in the closed neural tube was incised longitudinally at the wing bud level. The incision was 3 somites long, which was equivalent to approximately 0.8mm. Following surgery, the embryos were re-incubated in ovo for three or five days. The area of the incision was observed with a stereomicroscope. Some of them were examined histologically with the transverse section of the wing bud area. They were divided into two groups(POD 3 and POD 5) according to the re-incubation period at the time of sacrifice and then into two subgroups(re-closure and defect group) according to the presence of ONTD at the operative site. RESULTS: The results showed : 1) Re-closure of ONTD occurred in 58%(23/40) of POD 3 embryos and 46%(22/48) of POD 5 embryos. The difference of re-closure was not statistically significant. 2) Most of the re-closed neural tubes revealed no significant difference from the controls in the histological examination. 3) In POD 3 and 5 groups, there was a tendency of zipper-like fusion in both re-closure and defect groups. CONCLUSION: The results of study showed that the neural tube of the early chick embryo has a re-closure capacity after being surgically reopened. Seemingly, re-closure occurs mainly before POD 3 and progresses from the ventral to the dorsal part of the neural tube. The mechanism of re-closure needs to be investigated further.
Animals ; Chick Embryo* ; Embryonic Structures ; Neural Tube Defects* ; Neural Tube* ; Somites ; Wings, Animal

Müllerian duct aplasia-renal aplasia-cervicothoracic somite dysplasia (MURCS) association is a unique development disorder with four common types of malformations that include uterine aplasia or hypoplasia, renal ectopy or agenesis, vertebral anomalies, and short stature. The majority of MURCS patients are diagnosed with primary amenorrhea from late-adolescence. However, a few cases with MURCS association are not well diagnosed during childhood and long-term outcomes are not well reported. We report a case of an 8-year-old girl with MURCS association who presented with recurrent urinary tract infections and multiple congenital malformations, and who was followed for 10 years until adulthood. MURCS association should be considered as one of the differential diagnoses when evaluating prepubertal females with vertebral and renal malformations.
Amenorrhea ; Child ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Humans ; Somites ; Urinary Tract Infections

The effects of embryo number and incubation volume on the development of mouse embryos were evaluated. The growth rate of two-cell mouse embryos to attached blastocyst stage and the growth rate of blastocysts to early somite stage were assessed after culture in different incubation volumes and embryo densities. Embryos were collected from ICR female mice superovulated with pregnant mare serum gonadotropin and human chorionic gonadotropin and mated by ICR males. In experiment 1, groups of one, five, ten, twenty 2-cell embryos were cultured in 10-, 50-, 500-, 1000-microliter drops of BWW media under mineral oil at 37 degrees C in a humidified atmosphere of 5% CO2 and 95% air. As the incubation volume decreased, significantly (p<0.05) higher rates of embryos reached morular and blastocyst stage on day 3 and 4 culture, respectively In experiment 2, groups of one, five, ten, twenty blastocysts were cultured in 1- and 2-ml volumes of CMRL 1066 media under same condition as in experiment 1. However the reverse was the result. Decreasing the number of embryos incubated per volume from 1 to 20 significantly (p<0.05) increased the number of blastocysts reaching the late egg cylinde. (LEC) and early somite (ES) stage on day 6 and 8 culture, respectively, regardless of incubation volume. Blastocysts cultured in 2ml had higher (p<0.05) development rates to LEC and ES stage on day 6 and 8 culture, respectively, than embryos cultured in 1ml. Our results suggest that the effects of embryo number and incubation volume on the development of mouse embryos are stage specific and the shifting point was between hatching and EEC stage.
Animals ; Atmosphere ; Blastocyst ; Chorionic Gonadotropin ; Embryonic Structures* ; European Union ; Female ; Gonadotropins ; Humans ; Male ; Mice* ; Mineral Oil ; Ovum ; Somites

During the morphogenesis of the hindlimb and the digits of the rat, apoptosis has a crucial role to make their normal shape. In this study, apoptotic changes of the hindlimb from GD 13.5 to GD 17.5 were observed and compared with 5 -FU treated group to reveal the mechanism of teratogenic action of 5 -FU. The results were as follows; 1. The mean body weight of rat fetuses was decreased significantly in the 5 -FU treated group. 2. Congenital anomalies of the hindlimb digits were cleft foot, syndactyly and short digits, and the occurance rate of congenital anomalies was 58.1%, in which right -sided anomalies was 49.1% and left -sided anomalies was 18.9%. 3. Normal morphogenesis of the hindlimb digits was as follows; initially, hindlimb bud appeared at the lateral wall of lumbar somite on GD 10.5, grown as an elliptical mass proximodistally on GD 13.5, flattening and digital rays formation on GD 14.5, interdigital notches appeared on GD 15.5, interdigital spaces formation and interphalageal joints formation on GD 16.5, and deepening of interdigital spaces and elongation of digits on GD 17.5. 4. 5 -FU inhibited differentiation and growth of hindlimb bud initially, but its effect on development of hindlimb was disappeared after GD 15.5. According to the above results, it is considered that 5 -FU has an inhibitory effect on differentiation and growth of hindlimb of rat fetuses, and the TUNEL immunohistochemical method is much available to detect normal morphogenesis of an organ and to detect the mechanism of action of various teratogens.
Animals ; Apoptosis ; Body Weight ; Fetus ; Fluorouracil* ; Foot ; Hindlimb* ; In Situ Nick-End Labeling ; Joints ; Morphogenesis* ; Rats ; Somites ; Syndactyly ; Teratogens

Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is characterized by congenital aplasia of the uterus and the upper part of the vagina in women showing normal development of secondary sexual characteristics and a normal 46, XX karyotype. MRKH syndrome usually remains undetected until the patient presents with primary amenorrhea despite normal female sexual development. MRKH syndrome is the second frequent cause of primary amenorrhea. There have been several reports concerning gynecologic disease in MRKH syndrome, but there has been few case about MRKH syndrome with gonadal agenesis. We report an exceptional association between bilateral ovarian agenesis 46,XX and MRKH syndrome. A 27-year-old woman who presented with primary amenorrhea and absence of secondary sexual development. She had normal, 46XX karyotype, but no upper vagina, uterus and both ovary. And there was no urogenital and skeletal malformation. She was diagnosed as the atypical form of MRKH syndrome (bilateral gonadal agenesis 46 XX).
Abnormalities, Multiple ; Adult ; Amenorrhea ; Female ; Genital Diseases, Female ; Gonadal Dysgenesis ; Gonads ; Humans ; Karyotype ; Kidney ; Mullerian Ducts ; Ovary ; Sexual Development ; Somites ; Spine ; Uterus ; Vagina

Teratogenic effects of a diphenylhydantoin on the neurulation of the explanted early chick embryos were studied using the punched-out filter paper explantation technique. The 6th to 9th Hamburger and Hamilton staged chick embryos were explanted and cultured in the Ham's F-10 media treated with 15 microgram/ml, 30 microgram/ml, 60 microgram/ml, 90 microgram/ml, 120 microgram/ml of diphenylhydantoin in the CO2 incubator for 6-9 hours. The morphological chracteristics and the ultrastructural changes of the neuroepithelium of early chick embryos were compared with the control and experimental group using the stereomicroscope and the electron microscope. Of th 40 chick embryos cultured in the Ham's F-10 media without drug, 37 embryos(92.5%) developed normally and 3 embryos(7.5%) developed abnormally in 94 embryos(61.4%). The frequent anomalous features of the embryos were deformities of the neural folds in the cranial regions, failure of neural tube closure, dispersion of somites and developmental arrest. The scanning electron microscopic findings of neuropithelial cells of abnormally developed embryos were diminished surface blebs and microvilli, flattened and smooth cellular surfaces, and irregular size of cells. The transmission electron microscopic findings of neuroepithelial cells of abnormally developed embryos showed no significant changes of the development of intracellular organelles except the smooth cellular surface and mild underdevelopment of microfilaments.
Actin Cytoskeleton ; Animals ; Blister ; Chick Embryo* ; Congenital Abnormalities ; Embryonic Structures ; Incubators ; Microvilli ; Neural Crest ; Neural Tube ; Neuroepithelial Cells ; Neurulation* ; Organelles ; Phenytoin* ; Somites

Klippel-Feil syndrome(KFS) consists of short neck, low posterior hairline and restriction of motion of the neck due to fusion of cervical vertebrae. The typical disorder results from a failure of the normal segmentation of mesodermal somites during 3-8 weeks of gestation. In 1912, the first complete clinical description of this syndrome was given by Klippel and Feil. Feil reported additional cases in 1919 and distinguished between three morphologic groups. The incidence of KFS has been estimated to be approximately 1 : 40,000-42,000 births. A slight female predilection has been noted. Although the disorder is sporadic, there are examples of familial occurrence; how ever, no clear mechanism of inheritance has been accepted. Since the disturbance producing a short neck occurs early in embryogenesis, defects in other organ systems may occur at the same time. Common musculoskeletal anomalies that accompany KFS include scoliosis, as well as Sprengel's deformity in as many as one-third of cases. Neurologic, cardiovascular, and urinary tract anomalies are associated with KPS. We report a case of Klippel-Feil syndrome with associates anomalies include Sprengel's deformity.
Cervical Vertebrae ; Congenital Abnormalities ; Embryonic Development ; Female ; Humans ; Incidence ; Klippel-Feil Syndrome* ; Mesoderm ; Neck ; Parturition ; Pregnancy ; Scoliosis ; Somites ; Urinary Tract ; Wills

Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is a rare congenital disorder characterized by the congenital absence or hypoplasia of the uterus and the upper two thirds of the vagina due to mullerian duct malformation during embryogenesis. MRKH syndrome usually presents as primary amenorrhea in adolescence in females showing normal development of secondary sexual characteristics and a normal 46, XX karyotype. For this reason, MRKH syndrome usually remains undiagnosed until primary amenorrhea or difficulty in sexual intercourse occurs. In this study, a case of MRKH syndrome diagnosed in a child with idiopathic precocious puberty is reported.
Abnormalities, Multiple ; Adolescent ; Amenorrhea ; Child ; Coitus ; Congenital, Hereditary, and Neonatal Diseases and Abnormalities ; Embryonic Development ; Female ; Humans ; Karyotype ; Kidney ; Mullerian Ducts ; Pregnancy ; Puberty, Precocious ; Somites ; Spine ; Uterus ; Vagina