• Title/Summary/Keyword: Malformations

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Radiation Protective Effect of Selenium and Folic Acid Mixtures in the Development of Congenital Anomalies Following Radiation Exposure to the Fetus of Perinatal Female White Rats (주산기 암컷 백서의 태아에 방사선피폭에 따른 선천성기형 발생 시 셀레늄과 엽산 혼합물의 방사선 방호효과)

  • Jung, Do-Young;Choi, Hyung-Seok;Kim, Jang-Oh;Shin, Ji-Hye;Kim, Joo-Hee;Park, Gyeong-Jin;Min, Byung-In
    • Journal of radiological science and technology
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    • v.41 no.2
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    • pp.157-162
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    • 2018
  • This study is a search for radiation protection effects of radiation exposure on the organogenic period during the prenatal period, which is known to be the most likely to have congenital malformations by radiation exposure. To study the radiation protection for the mixture of selenium that is strong antioxidant and folic acid that is essential vitamin for DNA synthesis, 2 Gy of radiation was irradiated to pregnant female rats. then, after 14 days of fetal birth, observing blood components, SOD(Superoxide Dismutase), histological changes and external malformations. There was a significant protective effect to reduce blood cell damage(p<0.05) in the irradiation group after selenium and folic acid mixture were administered than irradiation group, and the activation of SOD which is antioxidant enzymes was increased. In addition, confirmed the effect of suppressing the expression of apoptosis of small intestinal cells and the reduction of cerebral cortex layer reduction by radiation. thus, it was confirmed that the congenital malformations were reduced as a result of these protective effects. Based on these results, selenium and folic acid mixture may reduce the incidence of congenital malformations, and it will reduce the damage of the fetus caused by the exposure of the organogenic period due to accidents.

Anorectal Malformations Associated with Esophageal Atresia in Neonates

  • Byun, Shin Yun;Lim, Ryoung Kyoung;Park, Kyung Hee;Cho, Yong Hoon;Kim, Hae Young
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.16 no.1
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    • pp.28-33
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    • 2013
  • Purpose: Anorectal malformations are often associated with other anomalies, reporting frequency with 40-70%. Gastrointestinal anomalies have been known to be relatively less common than associated anomalies of other organ system. This study was performed to assess a distinctive feature of cases associated with esophageal atresia. Methods: Clinical data (from January 2000 through December 2011) on the 196 subjects with anorectal malformations, managed in our Hospital, were reviewed. Total 14 neonates were identified with accompanying esophageal atresia and retrospective analysis was conducted. Results: The incidence was 7.1% and there were 8 male and 6 female subjects. Only 2 cases were associated with esophageal atresia without tracheoesophageal fistula. Although variable cases of anorectal malformation in female subjects, almost cases were anorectal malformations with rectourethral fistula in male. Other associated anomalies were identified in all cases, with more than 3 anomalies in 10 cases. There were 4 VACTERL (Vertebral abnormalities, Anal atresia, Cardiac anomalies, Tracheoesophageal fistula, Esophageal atresia, Renal and Limb anomalies) associations accounting for 28.6%, but could not identify chromosomal anomaly. Most cases were managed with staged procedure, usually primary repair of esophageal atresia and diverting colostomy. Overall mortality rate was 21.4%, mainly caused by heart problems. Conclusion: This study shows that early diagnosis and rational surgical approach with multidisciplinary plan are mandatory in managing anorectal malformations with esophageal atresia, when considering a high frequency of associated anomaly and a relative high mortality.

Transvaginal Direct Puncture and Ethanol Sclerotherapy for Cervicovaginal Venous Malformations: A Case Report and Literature Review (자궁경부 및 질에 발생한 정맥기형에 대한 질 경유 직접 천자 및 에탄올 경화요법: 증례 보고 및 문헌 고찰)

  • Gu Seong Jeong;Suk Hyun Bae;Young Soo Do;Hyoung Nam Lee;Sang Joon Lee
    • Journal of the Korean Society of Radiology
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    • v.82 no.3
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    • pp.688-692
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    • 2021
  • Cervicovaginal venous malformations are extremely rare. Sclerotherapy is proven to be effective for superficial venous malformations but not for venous malformations in the lower genital tract of female. A 52-year-old female presented with intermittent vaginal bleeding. The amount of vaginal bleeding gradually increased over 3 months. Contrast-enhanced pelvis CT showed several phleboliths and dilated vessels, but pelvic angiography showed no early draining veins, nidus, or feeding artery. We performed transvaginal direct puncture and ethanol sclerotherapy rather than surgical treatment because she wanted to preserve the uterus. After four sessions of sclerotherapy, she had significantly decreased vaginal bleeding without complications. Here, we report the first case of cervicovaginal venous malformations successfully treated with transvaginal direct puncture and ethanol sclerotherapy.

Anorectal Malformations:Diagnosis and Management in Neonatal Period (항문직장기형 - 진단과 신생아기 처치 -)

  • Lee, Nam-Hyuk
    • Advances in pediatric surgery
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    • v.12 no.1
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    • pp.99-106
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    • 2006
  • Anorectal malformations comprise a spectrum of disease and the majority of patients have one or more abnormalities that affect other systems. In evaluating a newborn with anorectal malformation, the decision regarding the need for a colostomy and detection and management of any life threatening associated anomalies are thetwo most important considerations. Perineal inspection provides the clue to the surgical approach in about 80-90 % of male and 90 % of female newborn baby. The remaining patients who do not show any clinical evidence need radiologic evaluation to decide whether a colostomy should be performed. In most cases the decision to make a colostomy should not be made until the baby is 20 to 24 hours old and evaluation to rule out the presence of associated anomalies completed. A divided colostomy at the junction of the descending and sigmoid colon is recommended for anorectal malformations.

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Intraneural Venous Malformations of the Median Nerve

  • Porto, Sara Alicia Gonzalez;Rodriguez, Alba Gonzalez;Miguez, Jose Midon
    • Archives of Plastic Surgery
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    • v.43 no.4
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    • pp.371-373
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    • 2016
  • Venous malformations arising from the peripheral nerve are a rare type of vascular malformation. We present the first case of an intraneural venous malformation of the median nerve to be reported in a child and review the previous two cases of median nerve compression due to a venous malformation that have been reported. These cases presented with painless masses in the volar aspect of the wrist or with symptoms suggestive of carpal tunnel syndrome. Clinical suspicion should lead to the use of Doppler ultrasonography as the first-line diagnostic tool. Magnetic resonance imaging and histopathology can confirm the diagnosis, as phleboliths are pathognomonic of venous malformations. Surgical treatment appears to be the only modality capable of successfully controlling the growth of an intraneural malformation. Sclerotherapy and radiotherapy have never been used to treat this type of malformation.

Amniotic constriction band: a report of two cases with unique clinical presentations

  • Richardson, Sunil;Khandeparker, Rakshit Vijay;Pellerin, Philippe
    • Journal of the Korean Association of Oral and Maxillofacial Surgeons
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    • v.43 no.3
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    • pp.171-177
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    • 2017
  • Amniotic constriction band is a rare clinical entity with varied manifestations that range from a combination of congenital malformations to isolated malformations that are unique to each patient. The etiology of this entity remains unknown. Herein, we highlight two cases of amniotic constriction band that presented to our unit with unique clinical characteristics. To the best of our knowledge, an isolated circumferential band of scarring on the face with ocular involvement, as demonstrated by the first case, and a combination of bilateral complete cleft lip and palate with bilateral microphthalmia, auto-amputation of the right thumb, and a constriction band on the left thumb, as demonstrated by the second case, are extremely rare presentations of amniotic constriction band that were not previously reported in the literature and therefore necessitate a special mention. We discuss potential etiologies for these cases and review the existing literature on this entity.

Sclerotherapy using 1% sodium tetradecyl sulfate to treat a vascular malformation: a report of two cases

  • Min, Hong-Gi;Kim, Su-Gwan;Oh, Ji-Su;You, Jae-Seek
    • Journal of the Korean Association of Oral and Maxillofacial Surgeons
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    • v.41 no.6
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    • pp.322-326
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    • 2015
  • Vascular malformations are the most common congenital and neonatal vascular anomalies in the head and neck region. The demand for simple and esthetic vascular malformation treatments have increased more recently. In this study, two patients that were diagnosed with venous malformations were treated with sodium tetradecyl sulfate as a sclerosing agent. Recurrence was not found one year after the surgery. This article gives a brief case report of sclerotherapy as an effective approach to treat vascular malformations in the oral cavity.

Natural history and clinical manifestation of Pediatric Brain Arteriovenous Malformations

  • Adikarige Haritha Dulanka Silva;Greg James
    • Journal of Korean Neurosurgical Society
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    • v.67 no.3
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    • pp.280-288
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    • 2024
  • Brain arteriovenous malformations (bAVMs) are aberrant arteriovenous shunts through a vascular nidus with no intervening capillary beds. They are one of the commonest causes of spontaneous intracranial haemorrhage in children and may be associated with significant morbidity and mortality in cases of rupture. Treatment strategies include microsurgical resection, endovascular embolisation, stereotactic radiosurgery, multimodality treatment with a combination thereof, and particularly in high-grade bAVMs, conservative management. Clinicians involved in treating bAVMs need to have familiarity with the natural history pertaining to bAVMs in terms of risk of rupture, risk factors elevating rupture risk as well as understanding the clinical manifestations of bAVMs. This invited review serves to provide a synthesis on natural history and clinical presentation of bAVMs with particular focus in children to inform decision-making pertaining to management.

Investigation of Actual Culture Conditions of King Oyster Mushroom (Pleurotus eryngii) and Methods for Reduction of Fruit-body Malformations II. Culture Methods for Reduction of Fruit-body Malformations of King Oyster Mushroom(Pleurotus eryngii) (큰느타리버섯 재배실태조사와 기형버섯 발생경감에 관한 연구 II. 기형버섯 발생경감 재배기술)

  • Ha, Tai-Moon;Ju, Young-Cheuol;Shin, Pyung Gyun
    • Journal of Mushroom
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    • v.9 no.2
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    • pp.69-73
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    • 2011
  • We have researched on methods which can reduce fruit-body malformations of king oyster mushroom(Pleurotus eryngii). We collected many pathogens from diseased fruit-body or malformated fruit-body and identified with chemicobiological test and microscope. The factors of fruit-body malformations and increment of contamination during the pin-heading induction time were researched with ventilation amounts in growing room. When the pathogens having high pathogenicity were inoculated in spawn running bottle and at pin-heading induction time, symptom appeared or not appeared in according to air ventilation amounts in growing room. During the pin-heading induction time, humidity degree in growing room have kept of high level and air ventilation amounts were so little that fruit-body malformations ratio was high. But, even though pathogens were inoculated at the surface of bottle factitiously, if air ventilation amounts were enough, fruit-body malformation ratio was low.

Surgical Treatment of Cavernous Malformation of Pineal Region

  • Kang, Jeong-Han;Kim, Dong-Seok;Park, Yong-Gou;Choi, Joong-Uhn
    • Journal of Korean Neurosurgical Society
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    • v.38 no.3
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    • pp.238-241
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    • 2005
  • The main causes of pineal apoplexy are hemorrhage associated with pineal region tumors, vascular malformations, and pineal cysts. Cavernous malformations rarely occur in the pineal region, with only fifteen cases reported previously. Hemorrhage associated with cavernous malformation causes apoplectic event in the pineal region. We report two surgically treated cases of pineal hemorrhage associated with cavernous malformation and discuss the consideration in management of the pineal apoplexy.