• Anatomy and Cell Biology
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• 제56권4호
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• pp.421-427
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• 2023

Bladder exstrophy is a rare congenital condition of the pelvis, bladder, and lower abdomen that opens the bladder against the abdominal wall, produces aberrant growth, short penis, upward curvature during erection, wide penis, and undescended testes. Exstrophy affects 1/30,000 newborns. The bladder opens against the abdominal wall in bladder exstrophy, a rare genitourinary condition. This study is vital to provide appropriate therapy choices as a basis to improve patient outcomes. This study may explain bladder exstrophy and provide treatment. Epispadias, secretory placenta, cloacal exstrophy, and other embryonic abnormalities comprise the exstrophy-spades complex. The mesenchymal layer does not migrate from the ectoderm and endoderm layers in the first trimester, affecting the cloacal membrane. Embryological problems define the exstrophy-aspidistra complex, which resembles epimedium, classic bladder, cloacal exstrophy, and other diseases. Urogenital ventral body wall anomalies expose the bladder mucosa, causing bladder exstrophy. Genetic mutations in the Hedgehog cascade pathway, Wnt signal, FGF, BMP4, Alx4, Gli3, and ISL1 cause ventral body wall closure and urinary bladder failure. External factors such as high maternal age, smoking moms, and high maternal body mass index have also been associated to bladder exstrophy. Valproic acid increases bladder exstrophy risk; chemicals and pollutants during pregnancy may increase bladder exstrophy risk. Bladder exstrophy has no identified cause despite these risk factors. Exstrophy reconstruction seals the bladder, improves bowel function, reconstructs the vaginal region, and restores urination.

• Journal of Radiation Protection and Research
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• 제31권2호
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• pp.91-96
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• 2006

The effects of Magnetic resonance imaging (MRI) on mouse embryos at the early stage of organogenesis were investigated. Pregnant ICR mice were exposed on day 8 of gestation to MRI at 0.5 T for 0.5 hour to 3 hours. The mortality rates of embryos or fetuses, the incidence of external malformations, fetal body weight and sex ratio were observed at day 18 of gestation. A significant increase in embryonic mortality was observed after exposure to either 0.5 T MRI for 0.5 hour or 2 hours. However, the exposure to MRI for 1 hour or 3 hours did not induce any significant increase in embryonic mortality when compared with control. External malformations such as exencephaly, cleft palate and anomalies of tail were observed in all experimental groups exposed to each MRI. A statistically significant increase of external malformations was observed in all groups treated with 0.5 T MRI for 0.5 hour and 3 hours. The incidence of external malformations in the mice group exposed to 0.5 T MRI for 0.5-hour was found to be higher than those of mice group exposed to 0.5 T MRI for 2 hours. The effects of MRI on the external malformations might not to be dose-dependent. There was no statistically significant difference in fetal body weight and sex ratio among each MRI exposure groups.

• Neonatal Medicine
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• 제17권2호
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• pp.262-264
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• 2010

요막관은 총 배설관에서 유래된 구조물로서, 태생기에 방광의 첨부와 제대 사이를 연결해주는 관이며, 임신 8-9개월에 완전히 막히게 되어 출생시에는 정중제인대로 남게된다. 드물게 요막관이 출생 후에도 요막관 게실, 요막관 낭종, 개방성 요막관 등으로 남아 있는 경우가 있는데, 그중 가장 심한 형태가 개방성 요막관으로 이를 통해 방광이 탈출되는 경우는 매우 드문 것으로 알려져 있다. 환아는 재태연령 41주, 출생체중 3,110 g, 아프가점수 1분 1점, 5분 7점, 정상 질식 분만되었으며, 산모는 35세로 임신 기간 중 약물 복용이나 다른 병력은 없었다. 재태연령 20주 2일에 시행한 산전 초음파상 낭성의 요막관 잔여물이 발견되었으며, 태아 자기공명영상에서 방광과 연결되는 낭종이 관찰되었다. 재태연령 40주 4일에는 고형종물 양상으로 관찰되어 초기의 낭종 형태에서 변화된 양상을 보였다. 출생 후 진찰 소견상, 제대 하방에 점막으로 둘러싸인 $3{\times}4cm$의 종물이 있었으며 요도로 삽입한 도관이 종물의 개구부를 통해 나오는 것이 확인되어 종물이 요도와 연결된 천공된 상태의 방광임을 추정할 수 있었다. 생후 1일에 시행한 복부 초음파상 수신증이나 다른 요로기형은 발견되지 않았으며, 생후 2일째 방광 복원술 및 요막관제거술을 시행하였다. 생후 9일째 시행한 배설성 방광 요로 조영술에서 방광 요로 역류는 없었고, 방광 기능은 좋은 것으로 확인되었다. 생후 10일째, 정상적인 배뇨를 보여 퇴원하였고 이후 특별한 문제없이 외래 추적 관찰 중이다. 저자들은 재태연령 20주 2일에 산전 초음파에서 요막관 잔여물로 추정되었던 낭종이 출생 후 요막관을 통한 방광 탈줄증으로 확진된 후 합병증 없이 수술적으로 완치되어 국내 최초로 보고하는 바이다.

• 대한소아치과학회지
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• 제23권3호
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• pp.601-608
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• 1996

저자는 전반적인 치아우식증을 주소로 개인 의원에서 경희대학교병원 소아치과에 의뢰된 10세 여아에서 Robinow syndrome 의 드문 증례의 치료를 시행하고 문헌을 고찰하여 다 음과 같은 결론을 얻었다. 1. 전신소견으로 전두부 돌출, 양안격리, 넓은 안검렬, 들창코 등을 보이는 태아모습의 특정적 안모와 작은키, 짧은 팔, 굽은 손가락, 생식기의 미발육 등이 관찰되었고 전반적인 발육지연이 있었다. 2. 구내소견으로 치아우식, 치아총생, 구개수의 미발육, 수술받은 구개열, 구호흡이 관찰되었다. 3. 이 증후군에서 드물게 보여지는 정신지체, 청각장애, 삼출성 중이염을 동반하였다. 4. 가족력은 발견할수 없었다. 5. 전신마취하에 전반척인 치과치료와 이비인후과치료를 함께 시행하였다. 6. Robinow 증후군은 여러 합병증을 수반할 수 있으므로 타과와의 협력하에 전반적인 검사를 시행하고 포괄적인 협력진료가 요구된다.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• 제49권1호
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• pp.21-29
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• 2023

Objectives: Dental or maxillofacial emergencies are uncommon during pregnancy, but if they occur, they are challenging to treat due to potential risks. The mother should not be denied necessary medical or dental care because of pregnancy. The aim of the study is to observe outcomes of pregnancy in patients requiring emergency minor oral surgical procedures during gestation and to determine the safety of the pregnant woman undergoing the procedure and the fetus. Materials and Methods: The study was conducted on 52 pregnant women requiring emergency oral surgical procedures. A standard treatment protocol for treatment of specific entities was followed. Close monitoring and observation were the primary goal of treatment. All patients were followed postoperatively until complete recovery from the surgical procedures and then until birth of the baby. A control group of 52 healthy pregnant patients who did not require oral surgical procedures was considered for statistical analysis. The measurements to calculate observation were fetal loss (spontaneous abortion), preterm birth, low-birth weight, or incidence of any congenital anomalies in the baby and its association with surgical procedures. Results: No fetal loss occurred in any of the cases. However, four patients experienced preterm birth and seven neonates exhibited low birth weights. No congenital abnormalities were discovered. In one instance, a patient who underwent surgery for a mandibular symphysis fracture under general anesthesia in the 31st week of pregnancy experienced labor pain on the fourth postoperative day, requiring an emergency Caesarean section. Conclusion: The results of our study demonstrate that, compared to the control group, minor emergency surgeries performed during pregnancy have no discernible negative effects on the fetus. These procedures can safely be performed by adhering to our described protocols.

• 한국동물번식학회:학술대회논문집
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• 한국동물번식학회 2003년도 학술발표대회 발표논문초록집
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• pp.18-18
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• 2003

In human, sudden infant death syndrome(SIDS) is synonyms for the sudden, unexpected and unexplained death of an infant. The incidence of SIDS has been estimated to be from 1 to 3%. Cloning has a relatively high rate of late abortion and early postnatal death, particularly when somatic cells are used as donors of nuclei and rates as high as 40 to 70% have been reported. However, the mechanisms for SIDS in cloned animals are not known yet. To date, few reports provide detailed information regarding phenotypic abnormality of cloned pigs. In this study, most of the cloned piglets were alive at term and readily recovered respiration. However, approximately 82% of male cloned piglets (81/22) died within a week after birth. Significant findings from histological examinations showed that 42% of somatic cloned male piglets died earlier than somatic cloned female piglets, most probably due to severe congestion of lung and liver or neutrophilic inflammation in brain, which indicates that unexpected phenotypes can appear as a result of somatic cell cloning. No anatomical defects in cloned female piglets were detected, but three of the piglets had died by diarrhea due to bacterial infection within 15 days after birth. Although most of male cloned piglets can be born normal in terms of gross anatomy, they develop phenotypic anomalies that include leydig cell hypoplasia and growth retardation post-delivery under adverse fetal environment and depigmentation of hair- and skin-color form puberty onset. This may provide a mechanism for development of multiple organ system failure in some cloned piglets. Th birth weights of male cloned pig in comparison with those of female cloned piglets are significantly reduced(0.8 vs 1.4kg) and showed longer gestational day(120 vs 114). In conclusion, brain meningitis and hepatopneumonic congestion are a major risk factor for SIDS and such pregnancy in cloned animals requires close and intensive antenatal monitoring.

• Journal of Genetic Medicine
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• 제16권2호
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• pp.76-80
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• 2019

About 15% to 20% of all clinically recognized pregnancies result in spontaneous abortion or miscarriage, and chromosomal anomalies can be identified in up to 50% of first trimester miscarriages. Chromosomal microarray analysis (CMA) is currently considered first-tier testing for detecting fetal chromosomal abnormalities and is supported by the absence of cell culture failure or erroneous results due to cell contamination in pregnancy loss. Triploidy is a lethal chromosome number abnormality characterized by an extra haploid set of chromosomes. Triploidy is one of the most common chromosomal aberrations in first trimester spontaneous abortions. Here, we report two cases of triploidy abortion that were not detected using array comparative genomic hybridization-based CMA. The aim of this report was to remind clinicians of the limitations of chromosomal testing and the misdiagnosis that can result from biased test selection.

• 한국동물생명공학회지
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• 제34권3호
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• pp.253-258
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• 2019

A female English bulldog was gave birth two neonates by cesarean section on the sixty one days after mating, but both neonates were died soon after birth. The bodies of neonates were diagnosed using radiography, ultrasonography, computed tomography and necropsy immediately after death. Both neonates had caudal regression syndrome, butterfly vertebra, hydrocephalus, umbilical hernia, cleft palate and bow-legged hind-limb. At necropsy, neonates had mild fetal anasarca, cleft lip and the skull was remained non-union. At thoracic cavity, only three ribs and thoracic spines were existed and patent ductus arteriosus was found. At abnormal cavity, the renal ectopia was found with abnormal morphology. In the present case, those English bulldog neonates with multiple congenital malformation syndromes seriously suffered vertebral column anomalies and that may induced by neural tube defects in during embryonic period. To prevent congenital malformation occurring in English bulldog, further in depth studies are needed for the breed specific genetic diversity and for the reason of behind genetic abnormality in these breed.

• Journal of Chest Surgery
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• 제28권4호
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• pp.381-386
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• 1995

In our hospital we have seen 20 cases of congenital diaphragmatic anomalies from June 1984 until December 1993. These were classified into 10 cases of diaphragmatic eventration, 8 cases of Bochdalek hernia, 1 case of Morgagni hernia, and 1 case of esophageal hiatal hernia. Diaphragmatic eventration cases were composed of 8 males and 2 females with ages varing from 3 hour to 42 year. They were discovered by symptoms: 5 cases of respiratory insufficiency; 3 cases of frequent respiratory infection; and 2 cases by chance; 6 cases involved the left side, 4 cases involved right side. Emergency operations were done to 4 patients. Among the 10 patients, only one operative mortality occurred; 3 hour old female.Bochdalek hernia cases composed 6 females and 2 males, 5 patients were less than 6 hour old. All patients were operated on an emergency status and three of them expired due to the vicious cycle of pulmonary hypertension and pulmonary vasoconstriction, persistent fetal circulation, hypoxia, and metabolic acidosis. Morgagni hernia was seen in one 69 year old female patient, she had no complaint of symptoms and was incidentally detected. Hernia was repaired through right thoracotomy. She was discharged with healthy appearence. Esophageal hiatal hernia was seen in a 10 month old male patient, his symptoms were persistent vomiting and coughing since birth. Sliding type of esophageal hiatal hernia repair was completed through left thoracotomy.

• Radiation Oncology Journal
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• 제16권3호
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• pp.233-243
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• 1998

목적 : 착상전기의 진단 및 치료 영역의 방사선 조사가 임신 ddy mouse에서 산전 사망과 외부 기형을 유발하는지, Compaction전 시기의 반응에 시기별 차이가 있는지 그리고 계통에 따르는 차이가 있는지 여부를 알아 보고자 하였다. 대상 및 방법 : 임신 ddy mouse 대조군 32 마리와 실험군 53 마리를 대상으로 연구를 시행하였으며, 실험군에 대한 방사선 조사는 착상전의 중요한 두 시기인 24 h p.c.와 48 h p.c.에 진단 영역에서도 이용되는 방사선 선량인 0.1, 0.5 Gy를 포함하여 0.75, 1.5, 3 Gy를 조사한 후, 임신 18일에 희생시켜 산전 사망 즉 착상전 사망, 배 사망 및 태아 사망과 외부 기형을 관찰하였다. 결과 : 1) 착상전 사망은 24 h p.c. 및 48 h p.c.에서 대조군에 비해 현저하게 많이 발생하였으며 선량 의존성을 나타냈고, 시기별 한계 선량은 각각 0.05 Gy 및 0.075 Gy이상으로 24 h p.c.가 48 h p.c.보다 방사선에 대한 감수성이 높은 시기임을 알 수 있었다. 2) 배 사망은 48 h p.c.의 0.1 Gy 조사군을 제외한 24 h p.c. 및 48 h p.c.의 모든 조사군에서 대조군에 비해 많이 발생하였고 선량 의존성을 보였으며, 한계 선량은 각각 0.1 Gy 및 0.25 Gy이상으로 24 h p.c.가 48 h p.c.보다 방사선 조사에 의한 배 사망의 감수성이 높았다. 3) 태아 사망은 24 h p.c. 및 48 h p.c.의 실험군 모두에서 발생하지 않았다. 4) 외부 기형은 24 h p.c. 실험군에서 뇌노출 기형 2예, 안검결손 기형 3예, 무안구증 3예, 구개열 2예, 복벽 파열 2예, 꼬리 기형 2예 및 다리 기형 1예, 국소 복벽 결손 1예 등이 발생하였는데, 그 중 진단 영역의 방사선 선량인 0.1 Gy군에서 안검결손 기형 1예, 복벽 파열 1예, 0.5 Gy군에서 뇌노출 기형 1예, 꼬리 기형 2예 및 다리 기형 1예가 발생하여 대조군에 비해 통계적으로 유의한 증가를 나타냈으며 이 시기의 한계선량은 0.2 Gy이상이었다. 48 h p.c.군에서도 안검결손 기형 1예, 꼬리 기형 2예가 발생하였으나 대조군과 통계적인 유의차가 없었다. 결론 : 이상의 결과를 통해 치료 영역뿐만 아니라 진단 영역의 방사선 조사로도 착상전기 임신 ddy mouse에서 착상전 사망 및 배 사망이 발생하고 24 h p.c.에서는 기형도 유발되어 이 시기의 방사선 영향이 "all-or-none" 반응만 일어나는 것이 아님을 알 수 있었으며, 24 h p.c.가 48 h p.c.보다 방사선 감수성이 높은 시기라는 사실과 함께 다른 연구 결과들과 비교하여 계통에 따르는 차이가 있음을 알 수 있었다. 그러므로 가임기 여성의 방사선 진단 및 치료시 Rugh의 10일 법칙을 적용하여 착상전기 방사선 조사로 인한 부작용들을 적극적으로 예방하는 것이 매우 중요하다고 생각한다.