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Neurocristopathy Combined with Congenital Central Hypoventilation Syndrome, Hirschsprung's Disease and Ganglioneuroblastoma in a Neonate (신경능선 성장장애 (Neurocristopathy)로 생긴 선천성 중추성 저환기 증후군, 허쉬슈프렁병, 신경절모세포종 동반 1예)

  • Jung, Sung-Eun;Kim, Dae-Yeon;Kim, Ki-Hong;Lee, Seong-Cheol;Park, Kwi-Won;Kim, Woo-Ki
    • Advances in pediatric surgery
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    • v.5 no.2
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    • pp.146-151
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    • 1999
  • Neurocristopathy originates from aberrant development of the neural crest by genetic abnormality. Dysgenetic or neoplastic neurocristopathy mayor may not combine at one or more organs. Congenital central hypoventilation syndrome (Ondine's curse) is characterized by the respiratory depression during sleep, although showing normal ventilation while awake, because the baby does not responde to hypercapnea or hypoxia. One newborn girl, full-term, 3,020 g of birth weight with neurocristopathy is reported. It showed poor respiration at birth, and temporary Improvement with oxygen and respiratory stimulations. Abdomen was distended. Abdominal x-ray revealed small bowel obstruction and calcified opacity at the right lower quadrant. Because transitional zone was noticed at the distal jejunum during laparotomy, jejunostomy was performed. Several times trial of extubation have failed becaused of the repeated apneas. Brain sonography and echocardiogram were normal. The patient died of sepsis at 37 days of age. Para-aortic ganglioneuroblastoma was found at autopsy. In this case, congenital central hypoventilation syndrome, Hirschsprung's disease and congenital ganglioneuroblastoma are combined as a neurocristopathy.

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Surgical treatment of congenital cystic lung disease -Report of 47 cases- (선천성 낭성 폐질환의 외과적 치료)

  • 문석환
    • Journal of Chest Surgery
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    • v.23 no.4
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    • pp.698-706
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    • 1990
  • Congenital Cystic Lung Disease is a spectrum of closed related anomalies that arise during an early stage of embryonic lung bud maturation-namely bronchogenic cyst, congenital lobar emphysema, pulmonary sequestration and congenital cystic adenomatoid malformation. And they show similar surgical strategies. So they are called as the term bronchopulmonary-foregut malformations, firstly proposed by Gerle[1968]. From Aug. 1979 to Aug 1989, 47 patients were operated upon on Dept. of Thoracic & Cardiovascular Surgery at the CUMC. There were 21 females and 26 males ranging in age from age of 21 day to age of 56 year [15 cases under 15 years old]. 30 patients had bronchogenic cysts - 23 of intrapulmonary type, 7 of mediastinal type in location. Affected lobes and locations were as follows: 11 in upper lobe, 3 in middle lobe, 11 in lower lobe and anterosuperior, middle, and posterior mediastinal type were 3, 2, 2 respectively. There were 9 pulmonary sequestrations[all intralobar type] with the distribution of 5 in right lower lobe and 4 in left lower lobe. And associated anomalies were presented with arterial supply originating from thoracic aorta[8 cases], abdominal aorta[1 case] and with venous drainage into azygos vein[1 case]. They all were operated upon lower lobectomy [8 case], pneumonectomy[1 case] in case of pulmonary hypoplasia Congenital lobar emphysema and congenital cystic adenomatoid malformation had 4 cases respectively. Their affected lobes were as follows: the former were 3 in upper lobes, 1 in middle lobe and the latter were 3 in upper lobe, 1 in lower lobe. They were treated with lobectomy and segmentectomy. Diagnosis was aided by chest X - ray, bronchography, aortography, DSA and CT scan, They all were confirmed by pathologic exams. There were no hospital death but few minor morbidities such as, atelectasis-pneumonia[2], wound infection[2], prolonged chest tube placement[2]. We experienced surgical treatments of 47 cases for 10 years and reported them with literature review.

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Clinical Analysis of Traumatic Diaphragmatic injuries Report of 28 cases (외상성 횡경막 손상 28례 분석보고)

  • 장진우;이연재
    • Journal of Chest Surgery
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    • v.30 no.4
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    • pp.402-407
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    • 1997
  • The records of 28 patients with traumatic diaphragmatic injuries seen at Masan Samsung Hospital from march 1986 o March 1995 were reviewed. We treated 21 male and 7 female patients ranging in ages from 5 to 68 years. Thc diaphragimatic injuries were due to blunt trauma in'20 cases(Trawc accident 18, compression injury 1, Human trauma 1) and penetrating injuries 8 cases (all stab wound). Most common symptoms were dyspnea 27 (96%), chest pain 26 (93%), abdomianl pain 8 (29%), comatose mentality 36 (11 %). Chest X-ray were elevated diaphragm in 20 cases(71%) find hemothorax in 18 cases (64%) and 25 cascs (89%) were diagnosed or suspected as diaphragmatic inju,rims prcopcrativcly. The repair of 28 cases were performed with thoracic approach in 20 cases, abdominal approach 7, thoracoabdominal approach in 1 cases. There were 5 death (18%) and all death related to the severity of associated injury. (hypovolcmic shock 3, combined head injury 1, renal failure 1).

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A Case of Traumatic Pancreatic Transection with Main Duct Disruption and Pleural Effusion in a Child (소아에서 늑막 삼출액을 동반한 외상성 췌장 절단 및 췌장 주관 손상 1예)

  • Lee, Ga-Yeun;Yoo, Hye-Soo;Lee, Jee-Hyun;Choe, Yon-Ho;Heo, Jin-Seok
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.10 no.1
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    • pp.98-103
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    • 2007
  • An 8-year-old boy presented with abdominal pain and poor oral intake for two months. Serum amylase and lipase levels were elevated. CT of the abdomen and chest X-ray showed two pseudocysts at the pancreatic uncinate process, pancreatitis with a parenchymal defect, a large amount of ascites, and a right pleural effusion. MR cholangiography and endoscopic retrograde cholangiopanreaticography revealed a pancreatic duct disruption. The patient was successfully treated with a chest tube placement and percutaneous drainage. After surgery, his general condition improved; the serum level of amylase normalized and the pleural effusion resolved. Pancreatic injuries are rare in pediatric blunt trauma; however, diagnostic difficulty is common with isolated blunt trauma. Therefore, a high index of suspicion should follow such an injury. We report the case of an 8-year-old boy with pancreas transection, ductal disruption, ascites, and pleural effusion who was successfully treated.

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Relationship Between Non-alcoholic Fatty Liver Disease and Decreased Bone Mineral Density: A Retrospective Cohort Study in Korea

  • Sung, Jisun;Ryu, Seungho;Song, Yun-Mi;Cheong, Hae-Kwan
    • Journal of Preventive Medicine and Public Health
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    • v.53 no.5
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    • pp.342-352
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    • 2020
  • Objectives: The aim of this retrospective cohort study was to investigate whether non-alcoholic fatty liver disease (NAFLD) was associated with incident bone mineral density (BMD) decrease. Methods: This study included 4536 subjects with normal BMD at baseline. NAFLD was defined as the presence of fatty liver on abdominal ultrasonography without significant alcohol consumption or other causes. Decreased BMD was defined as a diagnosis of osteopenia, osteoporosis, or BMD below the expected range for the patient's age based on dual-energy X-ray absorptiometry. Cox proportional hazards models were used to estimate the hazard ratio of incident BMD decrease in subjects with or without NAFLD. Subgroup analyses were conducted according to the relevant factors. Results: Across 13 354 person-years of total follow-up, decreased BMD was observed in 606 subjects, corresponding to an incidence of 45.4 cases per 1000 person-years (median follow-up duration, 2.1 years). In the model adjusted for age and sex, the hazard ratio was 0.65 (95% confidence interval, 0.51 to 0.82), and statistical significance disappeared after adjustment for body mass index (BMI) and cardiometabolic factors. In the subgroup analyses, NAFLD was associated with a lower risk of incident BMD decrease in females even after adjustment for confounders. The direction of the effect of NAFLD on the risk of BMD decrease changed depending on BMI category and body fat percentage, although the impact was statistically insignificant. Conclusions: NAFLD had a significant protective effect on BMD in females. However, the effects may vary depending on BMI category or body fat percentage.

Surgical Treatment of Aortoiliac Arterial Occlusion: Report of 2 Cases (대동맥하단부-장골동맥의 급,만성 폐쇄성 동맥질환 2례)

  • 마중성
    • Journal of Chest Surgery
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    • v.5 no.1
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    • pp.19-24
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    • 1972
  • The recent development of cardiovascular surgery as well as aortoarteriogaphy has been established excellent operative result with great aid of limb-salvage. However, less consideration or less experience still exists on the regard of vascular accident and vascular disease, as well as vascular surgery in Korea. During the last 13 years, we experienced only two cases of aorto-iliac occlusion,acute and chronic, regardless of having had more than 300 cases of mitral valvotomy and gradual increasing tendency of arteriosclerosis and hypertension in Korea. Therefore it is noteworthy to report the cases in order to promote the consideration for vascular surgery. Case 1; 52 year old female who had 20 years history of mitral stenosis with uricular fibrillation and received medical treatment for recent 1 year in the medical department. 10 days before admission, acute saddle emboli developed and 15 days after the onset, embolectomy through both common femoral arteries on the groin and abdominal approach was made. The progression of emboll to the right popliteal bifurcation was found by arteriography on operating table and retrograde flushing with heparin solution by the polyethylene catheter inserted through posterior tibial artery. The operation was successful, but 9 hours after operation sudden death occurred. Considering this case, first, mitral valvotomy already before might prevent peripheral embolizatlon, secondarily, the more early detection and surgery might also prevent the progression of emboli. Thirdly, although preoperative or postoperatlve heparinization is controversial for mitraI stenosis, heparinization might prevent additional emboli to vital organs in this case Cases 2; 66 year old female who had 4 years history of left hip and calf intermittent claudication and has had rest pain, inability to walk and ischemic necrosis on the the left leg since last 3 months prior to admission to the orthopedic department under the suspicion of herniated disc. Absence of pulsation on the groin and aortography evidenced aortoillac occlusion predominantly on the left side. Thromboendarterectomy was made and the operative result was successful with absence of claudication, healing of ulcer and aortographic patency of occlusive site. This chronic occlusion is considered to result from arteriosclerosis in origin with the evidence of moderate hypertension, x-ray evidence of calcified plaque on the aortic knob and operative finding of palpable plaques.

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A Study on the Correlation between of Bone Mineral Density and Cold Hypersensitivity of Hands (골밀도와 수냉증과의 상관성 연구)

  • Lee, Dong-Nyung;Lee, Jeong-Im;Go, Ho-Yeon;Kim, Hyung-Jun
    • The Journal of Korean Obstetrics and Gynecology
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    • v.28 no.4
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    • pp.11-20
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    • 2015
  • Objectives The purpose of this study was to examine the relationship between cold hypersensitivity and bone mineral density. Methods 28 outpatients who visited OO University Oriental Hospital from July 11th, 2013 to August 28th, 2013 were analyzed. Patients were subjected to thermometer, and those with thermal difference greater than 0.3 ℃ between upper arm and palm and also with more than VAS 4 of cold hypersensitivity were diagnosed with cold sensitivity. Lumba spine (L2-4) and heel bone measurement were obtained on dual energy X-ray absorptiometry (DEXA) and Quantitative Computed Tomography (QCT). Results 1. There was statistically significant correlation between T-score of DEXA and body weight (0.522), BMI (0.643), OSTA (Osteoporosis elf-assesment Tool for Asians, 0.579), BMI, T-score and Z-score of QCT. OSTA and T-score of QCT (0.521) also had statistically significance. 2. There was no statistically significant correlation between body fat, abdominal obesity rate, degree of cold hypersensitivity, upper arm and palm's temperature difference, palm temperature, height and Z-score, T-score of QCT or T-sore of DEXA. Conclusions Bone mineral density was lower in women who had cold hypersensitivity of hand and feet. But there was no statistically significant correlation between bone mineral density and degree of cold hypersensitivity, upper arm and palm’s temperature difference.

A case of diffuse alveolar hemorrhage associated with Henoch-Schoenlein purpura (헤노호-쉔라인 자반증 (Henoch-Schoenlein purpura)에 합병된 미만성 폐포출혈 1예)

  • Cho, Won-Kyoung;Lim, Chae-Man;Lee, Sang-Do;Koh, Youn-Suck;Kim, Woo-Sung;Yoo, Eun-Sil;Kim, Dong-Soon;Kim, Won-Dong
    • Tuberculosis and Respiratory Diseases
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    • v.43 no.3
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    • pp.461-466
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    • 1996
  • Diffuse alveolar hemorrhage is a very rare manifestation in Henoch-Schoenlein purpura. Recently we experience a case of diffuse alveolar hemorrhage associated with Henoch-Schoenlein purpura which was diagnosed by typical clinical manifestation and renal biopsy. A 25 year old male was admitted due to hemoptysis and dyspnea. Chest X-ray, HRCT and BAL revealed diffuse alveolar hemorrhage. He also had a history of skin rash, polyarthralgia, and hematochezia with abdominal pain. Renal biopsy which was taken for the evaluation of microscopic hematuria showed IgA nephropathy. Under the diagnosis of Henoch-Schoenlein purpura, we treated him with solumedrol pulse therapy, plasma-pheresis and prednisolone with cytoxan. After then he showed marked improvement in clinical manifestation and was discharged with prednisolone and cytoxan.

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A Case of Huge Pulmonary Blastoma With Multiorgan Invasion (다기관 침범을 동반한 거대 폐 모세포종)

  • Lee, Tae Hwan;Lee, Ka Young;Kim, So Ri;Min, Kyung Hun;Park, Seoung Ju;Lee, Heung Bum;Rhee, Yang Keun;Lee, Yong Chul
    • Tuberculosis and Respiratory Diseases
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    • v.62 no.2
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    • pp.149-153
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    • 2007
  • A pulmonary blastoma is a rare malignant tumor of the lung that is composed of epithelial and mesenchymal elements and resembles the structure of an embryonic lung. Pulmonary blastomas have a very poor prognosis and make up 0.25 to 0.5 percent of all primary malignant lung tumors. A pulmonary blastoma usually manifests as a solitary parenchymal mass or nodule and multiple subpleural mass with effusion on chest X-ray and computed tomography. We encountered a very rare case of pulmonary blastoma in a 52 years old male. He complained of abdominal pain, fullness, and dyspnea. The radiology examination revealed a huge lung mass invading the mediastinum, heart, diaphragm, and liver. The percutaneous needle biopsies were performed, and this tumor was diagnosed as a pulmonary blastoma. We report a biopsy confirmed case of a huge pulmonary blastoma invading multiple organs.

A Case of Acute Lung Injury Complicated by Transcatheter Arterial Chemoembolization for Hepatocellular Carcinoma (간세포암의 간동맥 화학색전술 후 발생한 급성 폐손상 1예)

  • Cho, Se-Haeng;Kim, Joo-Hang;Kim, Byung-Soo;Jang, Joon
    • Tuberculosis and Respiratory Diseases
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    • v.42 no.5
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    • pp.781-786
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    • 1995
  • Transcatheter arterial chemoembolization(TACE) was performed in a 61 year old male patient with hepatocellular carcinoma with 10 cc of Lipiodol and 50 mg of doxorubicin. Three days later, he complained of dyspnea and dry cough. The arterial blood gas study revealed moderate hypoxemia and hypocarbia. The chest PA showed acute pulmonary edema with bilateral pleural effusion. To rule out the possibilities of acute respiratory failure caused by infection, pulmonary embolism or congestive heart failure, we performed several laboratory studies. The blood and sputum culture studies revealed negative results for bacterial growth. The echocardiogram was normal. The abdominal CT scan and MR imaging revealed no thrombus or mass lesion in the inferior vena cava. So we concluded pulmonary oil embolism induced by lipiodol as the cause of acute lung injury. Four weeks later, clinical symptoms and chest x-ray were markedly improved with conservative care. We report a case of acute lung injury after TACE with lipiodol and doxorubicin, with review of literatures.

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