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Sample records for cystic duct syndrome

  1. Primary tuberculosis of cystic duct lymph node.

    Science.gov (United States)

    Ghazanfar, Aamir; Asghar, Afifa; Khan, Naqeeb Ullah; Hassan, Iram

    2017-06-16

    Tuberculosis (TC) is very common and significant cause of morbidity and mortality worldwide. Isolated cystic duct lymph node TC cases without involvement of gallbladder are exceedingly rare. It is difficult to diagnose preoperatively because of lack of characteristic signs and symptoms of TC. We report a man aged 45 years who presented with right upper abdominal pain since 1week. It was associated with nausea and postprandial fullness. There was no evidence of jaundice and lymphadenopathy. Abdominal examination showed moderate right upper quadrant tenderness with positive Murphy's sign and splenomegaly but no signs of peritonism. Abdomen ultrasound revealed sludge in gallbladder, dilated pancreatic duct, coarse exotexture of liver, splenomegaly and no lymphadenopathy. He underwent laparoscopic cholecystectomy; histological report showed chronic caseating granulomatous lymphadenitis with Langhans type of giant cells in lymph node near cystic duct with chronic cholecystitis of gallbladder. Standard antituberculosis therapy was given for 12 months. © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  2. ACCESSORY CYSTIC DUCT IDENTIFIED IN LAPAROSCOPIC CHOLECYSTECTOMY: A RARE CASE

    OpenAIRE

    Kumar; Kumar; Shaleen; Wasif; Amit

    2014-01-01

    We report the case of a 53 year old lady who underwent laparoscopic cholecystectomy and was found to have an accessory cystic duct close to the fundus. Careful dissection of the liver bed was done and the duct clipped preventing a bile leak. The presence of such ducts though rare should be identified during surgery to prevent potential complications.

  3. Cystic duct remnant mucocele in a liver transplant recipient

    International Nuclear Information System (INIS)

    Ahlawat, Sushil K.; Fishbien, Thomas M.; Haddad, Nadim G.

    2008-01-01

    Cystic duct remnant mucocele is an extremely rare complication of liver transplantation in children. Surgical correction is usually required for cystic duct remnant mucocele when it causes biliary obstruction. We describe a 14-month-old liver transplant recipient who presented with biliary obstruction 1 month after orthotopic liver transplantation with an end-to-end choledochocholedocal biliary anastomosis for hepatoblastoma. US, CT and cholangiography findings were consistent with mucocele of the allograft cystic duct remnant. Surgery was not needed in our patient because the mucocele and biliary obstruction had resolved on repeat imaging most likely due to guidewire manipulation during cholangiography, resulting in opening of the cystic duct remnant orifice and drainage into the common duct. (orig.)

  4. Cystic duct remnant mucocele in a liver transplant recipient

    Energy Technology Data Exchange (ETDEWEB)

    Ahlawat, Sushil K. [Georgetown University Hospital, Department of Medicine, Division of Gastroenterology, Washington, DC (United States); University of Medicine and Dentistry of New Jersey, New Jersey Medical School, Newark, NJ (United States); Fishbien, Thomas M. [Georgetown University Hospital, Department of Medicine, Division of Gastroenterology, Washington, DC (United States); Haddad, Nadim G. [Georgetown University Hospital, Department of Surgery, Division of Transplant Surgery, Washington, DC (United States)

    2008-08-15

    Cystic duct remnant mucocele is an extremely rare complication of liver transplantation in children. Surgical correction is usually required for cystic duct remnant mucocele when it causes biliary obstruction. We describe a 14-month-old liver transplant recipient who presented with biliary obstruction 1 month after orthotopic liver transplantation with an end-to-end choledochocholedocal biliary anastomosis for hepatoblastoma. US, CT and cholangiography findings were consistent with mucocele of the allograft cystic duct remnant. Surgery was not needed in our patient because the mucocele and biliary obstruction had resolved on repeat imaging most likely due to guidewire manipulation during cholangiography, resulting in opening of the cystic duct remnant orifice and drainage into the common duct. (orig.)

  5. Ascaris lumbricoides and its invasion of the accessory cystic duct: an unusual presentation.

    Science.gov (United States)

    Majid, Zain; Masood, Irfan; Pirzada, Muhammad Taqi

    2015-04-01

    Around the world, Ascaris lumbricoides is the most common helminthic infection. We describe the case of a 25-year-old woman, known to have had Ascaris infestation, presenting with abdominal pain, constipation and jaundice together with fever and tachycardia. There was tenderness in the right hypochondrium and liver function tests confirmed cholestatic jaundice. An abdominal ultrasound showed multiple linear echogenic foci in the distal small intestine along with cholelithiasis and a thick-walled gall bladder with a single stone compressing the common bile duct (Mirizzi syndrome). The patient underwent exploratory laparotomy; more than 100 worms were found inside the small intestine and they were removed by enterotomy and manual decompression. No worm could be palpated within the common bile duct (CBD). Cholecystectomy was performed, during which an accessory cystic duct was noted opening into the common bile duct superiorly to the main cystic duct. A 10 cm live worm was found coming out of it and was removed via forceps. Later on an endoscopic retrograde cholangio-pancreatogram (ERCP) showed a widened ampulla, a mildly dilated common bile duct, but without any filling defects. The patient made an uneventful postoperative recovery being discharged on the ninth day. Worms in an accessory cystic duct have not been report in the medical literature so far. © The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

  6. Optimum cystic duct closure: a comparative study using metallic clips, ENSEAL, and ENDOLOOP in swine.

    Science.gov (United States)

    McVay, Derek; Nelson, Daniel; Porta, Christopher R; Blair, Kelly; Martin, Matthew

    2013-05-01

    Metal clips are commonly used to secure the cystic duct during cholecystectomy, although use of an ENDOLOOP (Ethicon Endo-Surgery, Blue Ash, OH) is often touted as a more secure closure when postoperative endoscopic retrograde cholangiopancreatography (ERCP) is anticipated. The objective of this study was to test the strength of 3 different cystic duct closure methods in a model simulating postoperative biliary insufflation. The extrahepatic biliary system, including common bile duct, gallbladder, and cystic duct, was harvested en bloc from 22 swine postmortem. A cholecystectomy was performed and the cystic duct was secured using 1 of 3 randomly assigned methods: metallic clips (Ethicon Endo-Surgery), an ENDOLOOP (Ethicon Endo-Surgery), or an ENSEAL tissue sealing device (Ethicon Endo-Surgery). The common bile duct was cannulated with a pressure-monitoring system and insufflated with air. The burst pressures, location of rupture, and size of the common bile duct and cystic duct were recorded and compared. There were 7 pigs each in the ENDOLOOP and ENSEAL groups and 8 in the metallic clip group, with no statistical significance between cystic and common bile duct size. Mean burst pressure was 432 mm Hg for metallic clips, 371 mm Hg for the ENDOLOOP, and 238 mm Hg for the ENSEAL device (P = .02). Post hoc analysis revealed clips to be statistically superior when compared with the ENSEAL (P= .01). There was no statistical difference between the ENDOLOOP and metal clips or between the ENDOLOOP and the ENSEAL. All 3 closure methods successfully secured the cystic duct, with mean burst pressures exceeding 195 mm Hg. Metallic clips demonstrated the highest burst pressures and no cystic duct stump leaks. This study challenges the traditional dogma of additionally securing the cystic duct with an ENDOLOOP when postoperative biliary instrumentation is expected and also suggests that an adequately secure closure may be obtained with thermal sealing devices. Published by

  7. Persistent Mullerian Duct Syndrome with Transverse Testicular ...

    African Journals Online (AJOL)

    The Mullerian duct regresses in male due to a glycoprotein secreted by the developing testes called. Mullerian inhibiting factor (MIF). Failure to regress may be due to lack of MIF or defective MIF receptor, resulting in various disorders of regression, and one such disorder is persistent Mullerian duct syndrome (PMDS).

  8. A new bipolar feedback-controlled sealing system for closure of the cystic duct and artery.

    Science.gov (United States)

    Shamiyeh, A; Schrenk, P; Tulipan, L; Vattay, P; Bogner, S; Wayand, W

    2002-05-01

    Bile leaks are serious complications after laparoscopic cholecystectomy. The aim of this study was to evaluate the feasibility of closure of the cystic duct with a new feedback-controlled bipolar sealing system (LigaSure). Ten domestic pigs underwent open cholecystectomy with the cystic duct and artery dissected and sealed with the new bipolar sealing system (LigaSure). Four and 8 days postoperatively, 5 pigs each were sacrificed and the closure of the cystic duct was evaluated. The cystic stump and the common bile duct were excised for histological examination. None of the pigs had a bile leak or a biliary peritonitis. There were no signs of postoperative bleeding or inflammation in Calot's triangle. Histology showed total necrosis of the cystic duct in the first two pigs due to too much energy used. The remaining specimens showed a regularly scaling zone without necrosis in 7 cases, and in one case a partial necrosis in the mucosa only was found. Cystic artery and cystic duct closure with the new device may be an alternative to the clip. Further trials should evaluate the feasibility and safety of the new device in the clinical setting.

  9. Oral cholecystography compared to cholescintigraphy for evaluation of cystic duct patency prior to ESWL treatment

    DEFF Research Database (Denmark)

    Monrad, H; Grønvall, S; Højgaard, L

    1994-01-01

    In a prospective, blinded study of 109 patients with cholecystolithiasis, oral cholecystography and 99Tcm-EHIDA cholescintigraphy were compared in terms of reliability for demonstrating cystic duct patency: one of the prerequisites for extracorporeal shock wave lithotripsy (ESWL) treatment...

  10. Stenting of the Cystic Duct in Benign Disease: A Definitive Treatment for the Elderly and Unwell

    Energy Technology Data Exchange (ETDEWEB)

    Hersey, N., E-mail: naomi.hersey@sth.nhs.uk [Sheffield Teaching Hospitals NHS Trust, Department of Radiology, Northern General Hospital (United Kingdom); Goode, S. D., E-mail: s.goode@sheffield.sc.uk [Sheffield Teaching Hospitals NHS Trust, Sheffield Vascular Institute (United Kingdom); Peck, R. J., E-mail: robert.peck@sth.nhs.uk; Lee, F., E-mail: fred.lee@sth.nhs.uk [Sheffield Teaching Hospitals NHS Trust, Department of Radiology, Northern General Hospital (United Kingdom)

    2015-08-15

    PurposeThere have been few case reports describing cystic duct stent insertion in the management of acute cholecystitis secondary to benign disease with no case series published to date. We present our series demonstrating the role of cystic duct stents in managing benign gallbladder disease in those patients unfit for surgery.Materials and MethodsThirty three patients unfit for surgery in our institution underwent cystic duct stent insertion for the management of acute cholecystitis in the period June 2008 to June 2013. Patients underwent a mixture of transperitoneal and transhepatic gallbladder puncture. The cystic duct was cannulated with a hydrophilic guidewire which was subsequently passed through the common bile duct and into the duodenum. An 8Fr 12-cm double-pigtail stent was placed with the distal end lying within the duodenum and the proximal end within the gallbladder.ResultsTen patients presented with gallbladder perforation, 21 patients with acute cholecystitis, 1 with acute cholangitis and 1 with necrotising pancreatitis. The technical success rate was 91 %. We experienced a 13 % complication rate with 3 % mortality rate at 30 days.ConclusionCystic duct stent insertion can be successfully used to manage acute cholecystitis, gallbladder empyema or gallbladder perforations in those unfit for surgery and should be considered alongside external gallbladder drainage as a definitive mid-term treatment option.

  11. Stenting of the Cystic Duct in Benign Disease: A Definitive Treatment for the Elderly and Unwell

    International Nuclear Information System (INIS)

    Hersey, N.; Goode, S. D.; Peck, R. J.; Lee, F.

    2015-01-01

    PurposeThere have been few case reports describing cystic duct stent insertion in the management of acute cholecystitis secondary to benign disease with no case series published to date. We present our series demonstrating the role of cystic duct stents in managing benign gallbladder disease in those patients unfit for surgery.Materials and MethodsThirty three patients unfit for surgery in our institution underwent cystic duct stent insertion for the management of acute cholecystitis in the period June 2008 to June 2013. Patients underwent a mixture of transperitoneal and transhepatic gallbladder puncture. The cystic duct was cannulated with a hydrophilic guidewire which was subsequently passed through the common bile duct and into the duodenum. An 8Fr 12-cm double-pigtail stent was placed with the distal end lying within the duodenum and the proximal end within the gallbladder.ResultsTen patients presented with gallbladder perforation, 21 patients with acute cholecystitis, 1 with acute cholangitis and 1 with necrotising pancreatitis. The technical success rate was 91 %. We experienced a 13 % complication rate with 3 % mortality rate at 30 days.ConclusionCystic duct stent insertion can be successfully used to manage acute cholecystitis, gallbladder empyema or gallbladder perforations in those unfit for surgery and should be considered alongside external gallbladder drainage as a definitive mid-term treatment option

  12. Percutaneous Placement of Permanent Metallic Stents in the Cystic Duct to Treat Obstructive Cholecystitis.

    Science.gov (United States)

    Brown, Nicholas I; Jhamb, Ashu; Brooks, Duncan M; Little, Andrew F

    2015-12-01

    This report presents a series of five patients unsuitable for surgery who had nonretrievable self-expanding metallic stents deployed along the cystic duct as treatment for benign and malignant causes of gallbladder obstruction. Techniques are described for draining cholecystitis, removing gallstones, bypassing gallbladder obstructions, and inserting metallic stents across the cystic duct to restore permanent antegrade gallbladder drainage in acute and chronic cholecystitis. Symptoms resolved in all cases, and stents remained patent for as long as 22 months. This procedure may be an effective alternative to cholecystectomy or long-term gallbladder drainage for patients in inoperable condition. Copyright © 2015 SIR. Published by Elsevier Inc. All rights reserved.

  13. Cystic fibrosis transmembrane conductance regulator protein expression in the male excretory duct system during development.

    Science.gov (United States)

    Marcorelles, Pascale; Gillet, Danièle; Friocourt, Gaëlle; Ledé, Françoise; Samaison, Laura; Huguen, Geneviève; Ferec, Claude

    2012-03-01

    Sterility due to bilateral destruction in utero or in early infancy resulting in congenital absence of the vas deferens is the rule in male patients with cystic fibrosis. To understand the developmental pattern of this anomaly, the microscopic morphology of the male excretory system was analyzed during development and the expression of the cystic fibrosis transmembrane conductance regulator protein was explored by immunohistochemistry. We observed that cystic fibrosis fetuses had no excretory ducts agenesis or obstruction until 22 weeks of gestation. However, a focal inflammatory pattern and mucinous plugs in the oldest cystic fibrosis case suggested a disruptive mechanism. Immunolabeling of cytoplasmic epithelial cystic fibrosis transmembrane conductance regulator protein was demonstrated in all cystic fibrosis and control cases with a similar pattern of expression of the protein between age-matched controls and cystic fibrosis cases. At midgestation, an apical intensification appeared in both cystic fibrosis and control cases and was stable during the remainder of fetal life. No gradient of intensity could be detected between the different segments of the excretory tract. These findings are different from those reported in adults. The absence of any morphologic anomaly until 22 weeks of gestation, the focal destruction of the epithelial structures during the second trimester, and the chronological pattern of expression of cystic fibrosis transmembrane conductance regulator are of interest for a better understanding of the pathophysiology of this disease. Copyright © 2012 Elsevier Inc. All rights reserved.

  14. Utility of Laparoscopic Subtotal Cholecystectomy with or without Cystic Duct Ligation for Severe Cholecystitis.

    Science.gov (United States)

    Hirajima, Shoji; Koh, Toshimori; Sakai, Tomohito; Imamura, Taisuke; Kato, Shunji; Nishimura, Yukihisa; Soga, Koji; Nishio, Minoru; Oguro, Atsushi; Nakagawa, Noboru

    2017-11-01

    We use open cholecystectomy (OC) to treat severe cholecystitis in cases in which we are worried that inflammation might cause anatomical changes in Calot's triangle. Furthermore, in cases of severe cholecystitis in which marked inflammation leads to fibrosis, we perform subtotal cholecystectomy (SC), i.e., incomplete gallbladder resection. Laparoscopic SC (LSC) without cystic duct dissection is considered to be effective at reducing the incidence of serious complications in patients with severe cholecystitis. The cases of 246 patients who underwent cholecystectomy for benign gallbladder disease between January 2011 and May 2015 were evaluated retrospectively. Of these patients, 14 were treated with LSC, and 19 underwent OC. Moreover, three patients in the LSC group underwent LSC without cystic duct ligation because it was considered that it would be difficult to dissect and ligate the cystic duct. The LSC group suffered significantly less intraoperative blood loss than the OC group. However, the operative times of the two groups were similar. Moreover, the duration of the postoperative hospitalization period was significantly shorter in the LSC group than in the OC group. Next, we compared the long-term outcomes of the SC and total cholecystectomy groups, regardless of the surgical method. No cases of cholecystitis or gallbladder cancer were encountered in either group. It is suggested that LSC is safe, effective, and helps to prevent serious complications in cases of severe cholecystitis that require conversion to OC, regardless of whether cystic duct ligation is performed.

  15. Persistent Mullerian Duct Syndrome (PMDS With Large Intraabdominal Seminoma

    Directory of Open Access Journals (Sweden)

    Della Harigovind

    2017-07-01

    Full Text Available Persistent Mullerian Duct Syndrome, a form of male pseudohermaphroditism is characterized by the presence of the Mullerian duct derivatives in an otherwise phenotypically as well as genotypically normal male. We report a case of large intra abdominal seminoma in a male patient with cryptorchidism, along with persistence of Mullerian duct derivatives (uterus.

  16. Primary cystic pancreatic neoplasms and tumor-like conditions. MR cholangiopancreatographic evaluation of lesions and Wirsung's duct

    International Nuclear Information System (INIS)

    Garcia, Adriana; Spina, Juan C. h; Rogondino, Jose; Chacon, Carolina; Gutierrez, Silvia

    2003-01-01

    Objective: To evaluate the contribution of single shot fast spin echo (SSFSE) in the diagnosis of cystic lesions in the pancreas, and their relation to Wirsung's duct. Material and methods: In 66 patients (33 women and 33 men; mean age: 66 years) cystic pancreatic lesions were retrospectively analyzed. The SSFSE technique was used, including an evaluation of Wirsung's duct. Conventional pancreatic sequences were added. The following lesion features were assessed: location, number, size, relation to Wirsung's duct, nature of the cystic image and signal intensity of the neoplasm or tumor-like condition. Surgical and anatomopathological correlation was obtained in 31/66 cases (47%). Results: The cystic lesions were divided in 2 groups: A) cystic lesions related to Wirsung's duct, 30 patients: all lesions measured less than 30 mm in size. Seven patients underwent surgical treatment; in 22 cases surgery was not indicated. One patient refused surgery. A mucinous papilliferous intraductal tumor was diagnosed in 3 cases, ampullar carcinoma in 1 case, pancreatic carcinoma, 1 case, autoimmune pancreatitis, 1 case, and cystic duct dilatation due to benign fibrous stenosis, 1 case. B) Cystic lesions not related to Wirsung's duct (36 patients): 7 serous cystadenomas, 7 adenocarcinomas with a cystic component, 1 mucinous cystadenoma, 1 duodenal diverticulum, 7 pseudocysts and 1 neuroendocrine tumor. In 12 patients surgery was not carried out due to clinical contraindication or patient's refusal. Conclusion: SSFSE allowed a clear differentiation between cystic lesions related (Group A) and non-related (Group B) to Wirsung's duct. The diagnosis could not be achieved by usual MRI sequences. However, benign and malignant lesions were observed in both groups. In all cases SSFSE afforded useful data either for surgical treatment or clinical follow-up. (author)

  17. Transverse testicular ectopia with persistent Mullerian duct syndrome

    African Journals Online (AJOL)

    mail: amingoh@gmail.com. Received 30 September 2011 accepted 12 February 2012. Introduction. Transverse testicular ectopia (TTE) associated with persistent Mullerian duct syndrome (PMDS) is a rare anomaly that can be encountered ...

  18. [Symptoms, diagnosis and treatment of pancreaticobiliary maljunction associated with congenital cystic dilatation of bile duct].

    Science.gov (United States)

    Funabiki, T; Matsubara, T; Ochiai, M

    1996-08-01

    The concept of congenital biliary dilatation (CBD) was established by Alonso-Lej whose classification has been widely accepted and become a standard. Todani, Komi and several investigators then added additional subclassifications and/or made a proposal of new classification. The trias, abdominal pain, abdominal mass and jaundice, had been characteristically found in these cases, but recently asymptomatic cases are often found. Many reports have shown that the cases with cystic dilation of the biliary duct frequently associated with biliary carcinoma and pancreaticobiliary maljunction (PBM), an anomalous union of pancreatic duct with biliary tree since the Rabbit's first report. Whereas extracorporeal ultrasound and CT scan are minimally invasive diagnostic procedures for these cases, a direct cholangiography, ERCP and PTC are the diagnostic procedures that make a definite diagnosis for the appropriate treatment. Recently, endoscopic ultrasound (EUS), helical CT and magnetic resonance cholangiopan-creatography (MRCP) have drawn an attention since these methods are less invasive than those ever being used. In particular, MRCP will be suitable for the screening of preoperative examination. Whereas cystoduodenostomy used to be performed in the past for these cases, the resection of dilatted bule duct along with the diversion of bile from pancreatic juice should be performed to prevent biliary carcinoma. Cholecystectomy along with the resection of bile duct and the biliary diversion from pancreatic juice should also be performed for cases of PBM without CBD because mutagenicity of bile mixed with pancreatic juice and K-ras point mutation in noncancerous bile duct epithelium in cases of PBM without CBD were demonstrated.

  19. Poly Cystic Ovarian Syndrome: An Updated Overview.

    Science.gov (United States)

    El Hayek, Samer; Bitar, Lynn; Hamdar, Layal H; Mirza, Fadi G; Daoud, Georges

    2016-01-01

    Poly Cystic Ovarian Syndrome (PCOS) is one of the most common metabolic and reproductive disorders among women of reproductive age. Women suffering from PCOS present with a constellation of symptoms associated with menstrual dysfunction and androgen excess, which significantly impacts their quality of life. They may be at increased risk of multiple morbidities, including obesity, insulin resistance, type II diabetes mellitus, cardiovascular disease (CVD), infertility, cancer, and psychological disorders. This review summarizes what the literature has so far provided from guidelines to diagnosis of PCOS. It will also present a general overview about the morbidities associated with this disease, specifically with its more severe classic form. Finally, the review will stress on the various aspects of treatment and screening recommendations currently used in the management of this condition.

  20. Persistent Mullerian duct syndrome: the hidden normal or abnormal ...

    African Journals Online (AJOL)

    normal or abnormal anatomy and the value of laparoscopy. Abdullah Alwabari, Lalit Parida and Ahmed H. Al-Salem. Persistent Mullerian duct syndrome (PMDS) is a rare disorder of male sexual development. It is characterized by the presence of a uterus, fallopian tubes, and upper vagina in an otherwise phenotypically ...

  1. Extensive comedonal and cystic acne in Patau syndrome.

    Science.gov (United States)

    Torrelo, Antonio; Fernandez-Crehuet, Pablo; Del Prado, Elena; Martes, Pilar; Hernández-Martín, Angela; De Diego, Verónica; Carapeto, Francisco

    2010-01-01

    Patau syndrome is a chromosomal disorder associated with multiple malformations caused by inheritance of an extra chromosome (trisomy 13). Some skin defects have been reported in patients with Patau syndrome, such as scalp defects, glabellar stains, deep palmar creases, rocker-bottom feet, convex soles, hyperconvextity of the nails, and multiple hemangiomas. To our knowledge, widespread comedonal and cystic acne have not been previously reported in Patau syndrome.

  2. Closure of the cystic duct during laparoscopic cholecystectomy with a new feedback-controlled bipolar sealing system in case of biliary obstruction--an experimental study in pigs.

    Science.gov (United States)

    Shamiyeh, A; Vattay, P; Tulipan, L; Schrenk, P; Bogner, S; Danis, J; Wayand, W

    2004-01-01

    The excellent results of new devices like the new bipolar feedback-controlled sealing system (LigaSure) for closure of blood vessels encouraged surgeons to use these instruments for other structures like bile ducts. The aim of this study was to evaluate the feasibility of closure of cystic duct in case of biliary obstruction. Ten domestic pigs underwent laparoscopic cholecystectomy sealing the cystic duct with LigaSure. The common bile duct was closed with an endoclip to create a biliary hypertension. On the 12th postoperative day blood samples were taken for liver enzymes. At autopsy on day 15 the pigs were investigated for bile leaks or biliary peritonitis. The cystic duct was resected for histological examination. Seven pigs survived, one pig died during introduction of anesthesia, one on the 1st and one on the 2nd postoperative day without any findings at the autopsy. One pig out of 7 had a bile leak; the other 6 were without any sign of leakage. Histologically 3 pigs had a regular coagulation zone at the cystic duct, 3 had a total necrosis, one a partial necrosis of the mucosa only. Though there was only one insufficiency, the feedback-controlled bipolar vessel sealer cannot be recommended for biliary surgery with regard to the high rate of necrosis stated in our experiment.

  3. Cepacia Syndrome in a Non-Cystic Fibrosis Patient

    Directory of Open Access Journals (Sweden)

    Naomi Hauser

    2015-01-01

    Full Text Available Burkholderia (formerly Pseudomonas cepacia complex is a known serious threat to patients with cystic fibrosis, in whom it has the potential to cause the fatal combination of necrotizing pneumonia, worsening respiratory failure, and bacteremia, known as Cepacia syndrome. The potential for this pathogen to infect non-cystic fibrosis patients is limited and its epidemiology is poorly understood. Previously reported cases of severe Burkholderia cepacia complex lung infection in immunocompetent hosts include pneumonia, bronchiectasis, pyopneumothorax, and cavitary lesions. We present a case of a 64-year-old man with Streptococcus pneumoniae community-acquired pneumonia whose hospital course was complicated by developing cavitary lung lesions, bacteremia, and acute respiratory distress syndrome. Repeated tracheal aspirate and blood cultures grew Burkholderia cepacia. Our case appears to be the first report of Cepacia syndrome in a patient without cystic fibrosis. This report raises concern regarding the potential severity of pulmonary Burkholderia cepacia complex infection and the need to broaden clinicians’ suspicion for Cepacia syndrome. A framework to help diagnose and treat infected non-cystic fibrosis individuals may be useful.

  4. Cystic fibrosis: a mucosal immunodeficiency syndrome

    Science.gov (United States)

    Cohen, Taylor Sitarik; Prince, Alice

    2013-01-01

    Cystic fibrosis transmembrane conductance regulator (CFTR) functions as a channel that regulates the transport of ions and the movement of water across the epithelial barrier. Mutations in CFTR, which form the basis for the clinical manifestations of cystic fibrosis, affect the epithelial innate immune function in the lung, resulting in exaggerated and ineffective airway inflammation that fails to eradicate pulmonary pathogens. Compounding the effects of excessive neutrophil recruitment, the mutant CFTR channel does not transport antioxidants to counteract neutrophil-associated oxidative stress. Whereas mutant CFTR expression in leukocytes outside of the lung does not markedly impair their function, the expected regulation of inflammation in the airways is clearly deficient in cystic fibrosis. The resulting bacterial infections, which are caused by organisms that have substantial genetic and metabolic flexibility, can resist multiple classes of antibiotics and evade phagocytic clearance. The development of animal models that approximate the human pulmonary phenotypes—airway inflammation and spontaneous infection—may provide the much-needed tools to establish how CFTR regulates mucosal immunity and to test directly the effect of pharmacologic potentiation and correction of mutant CFTR function on bacterial clearance. PMID:22481418

  5. Combined persistent mullerian duct syndrome, transverse testicular ectopia and mosaic klinefelter's syndrome

    International Nuclear Information System (INIS)

    Rehman, A.; Hassan, S.; Zaidi, A.

    2008-01-01

    Persistent Mullerian Duct Syndrome (PMDS), a rare form of male pseudohermaphroditism, is characterized by the persistence of Mullerian duct structures (uterus, fallopian tubes and upper two-thirds of vagina) in otherwise normallyvirilized males (Karyotype 46XY). Patients suffering from PMDS present with cryptorchidism, inguinal hernia and infertility. Diagnosis is established when Mullerian duct structures are discovered either during ultrasonography for localization of undescended testis(s), during surgical exploration for cryptorchidism or herniorrhaphy (hernii uteri inguinalis). Presence of both testes on one side of the scrotum is known as Transverse Testicular Ectopia (TTE). Co-existence of PMDS and transverse testicular ectopia in a patient of mosaic Klinefelter's syndrome (Karyotype 46XY/47XXY) is a unique genetic association. (author)

  6. Disconnected pancreatic duct syndrome: complete pancreas transection secondary to acute pancreatitis.

    Science.gov (United States)

    Gámez-del-Castillo, Juan Manuel; Garcés-Albir, Marina; Fernández-Moreno, María Carmen; Morera-Ocón, Francisco Javier; Villagrasa, Rosana; Sabater-Ortí, Luis

    2016-03-01

    Disconnected pancreatic duct syndrome is a serious complication of acute pancreatitis which is defined by a complete discontinuity of the pancreatic duct, such that a viable side of the pancreas remains isolated from the gastrointestinal tract. This pancreatic disruption is infrequently observed in the clinical practice and its diagnostic and therapeutic management are controversial. We present an extreme case of disconnected pancreatic duct syndrome with complete duct disruption and pancreatic transection following acute pancreatitis, as well as the diagnostic and therapeutic processes carried out.

  7. Persistent Mullerian duct syndrome: A case report and review of the ...

    African Journals Online (AJOL)

    Persistent Mullerian duct syndrome is a rare form of internal male pseudohermaphroditism, in which Mullerian duct derivatives (uterus and fallopian tubes) are present in a genotypic (46XY) and phenotypic male. Over 150 cases have ... tools as well as lack of finance to assess those available. Although a diagnosis was ...

  8. Hodgkin's lymphoma-related vanishing bile duct syndrome: A case report and literature review

    Directory of Open Access Journals (Sweden)

    Kiong-Ming Wong

    2013-11-01

    Full Text Available We report the case of a 38-year-old man who developed vanishing bile duct syndrome in association with Hodgkin's lymphoma. He was noted to have cervical lymphadenopathy and marked elevation of total serum bilirubin at diagnosis. He achieved complete remission with normalization of serum bilirubin after eight courses of Adriamycin, bleomycin, vinblastine, and dacarbazine chemotherapy followed with autologous hematopoietic cell transplantation. Consecutive liver biopsies performed at diagnosis and at the stage of complete remission revealed the disappearance and regeneration of interlobular bile ducts, respectively. Our case provides pathological evidence that Hodgkin's lymphoma-related vanishing bile duct syndrome is a reversible bile duct injury disease. Bilirubin is a reliable serum marker to monitor the treatment response of these cases. The mechanism to develop hyperbilirubinemia with vanishing bile duct in such a case of Hodgkin's lymphoma remains to be studied. A literature review was carried out.

  9. International prospective study of distal intestinal obstruction syndrome in cystic fibrosis: Associated factors and outcome

    NARCIS (Netherlands)

    Munck, Anne; Alberti, Corinne; Colombo, Carla; Kashirskaya, Nataliya; Ellemunter, Helmut; Fotoulaki, Maria; Houwen, Roderick; Robberecht, Eddy; Boizeau, Priscilla; Wilschanski, Michael

    Background Distal intestinal obstruction syndrome (DIOS) is a specific complication of cystic fibrosis. Methods A study was performed in 10 countries to prospectively evaluate the incidence, associated factors, and treatment modalities in children and adults. Results 102 patients presented 112

  10. Surveyor assay to diagnose persistent Müllerian duct syndrome in Miniature Schnauzers.

    Science.gov (United States)

    Kim, Young June; Kwon, Hyuk Jin; Byun, Hyuk Soo; Yeom, Donguk; Choi, Jea-Hong; Kim, Joong-Hyun; Shim, Hosup

    2017-12-31

    Persistent Müllerian duct syndrome (PMDS) is a pseudohermaphroditism in males characterized by the presence of Müllerian duct derivatives. As PMDS dogs often lack clinical symptoms, a molecular diagnosis is essential to identify the syndrome in these animals. In this study, a new molecular method using DNA mismatch-specific Surveyor nuclease was developed. The Surveyor nuclease assay identified the AMHR2 mutation that produced PMDS in a Miniature Schnauzer as accurately as that obtained by using the conventional method based on restriction digestion. As an alternative to the current molecular diagnostic method, the new method may result in increased accuracy when detecting PMDS.

  11. Clinicopathological characteristics of atypical cystic duct (ACD) of the breast: assessment of ACD as a precancerous lesion.

    Science.gov (United States)

    Kusama, R; Fujimori, M; Matsuyama, I; Fu, L; Ishii, K; Hama, Y; Asanuma, K; Shingu, K; Kobayashi, S; Tsuchiya, S

    2000-10-01

    To clarify the clinicopathological features of an atypical cystic duct (ACD) as defined by Tsuchiya's criteria as a precancerous lesion of the breast, we used 200 whole mammary gland serial sections of breast cancer. Forty-four (22%) of the 200 breast cancer patients had ACD breast lesions. The frequency of patients with ACD increased in premenopausal women (P = 0.001). There was no correlation between the ACD-present group and the ACD-absent group for immunohistochemical status of the estrogen receptor (ER), progesterone receptor (PgR), p53, or c-erbB2; Ki-67 labeling index of cancer tissues; size of tumor, or lymph node metastases. A number of ACD lesions displayed continuity to cancer lesions. In 500 serial sections of a paraffin-embedded tissue of a ACD case at 3 microm intervals, an apparent transition from ACD into ductal carcinoma in situ was observed. Immunohistochemical analysis using alpha-smooth muscle actin showed that myoepithelial cells of ACD stained strongly, and their nuclei and cytoplasm were thinning. In 16 of the 44 (36%) ACD-present patients, carcinoma cells stained positive for p53. Within those 16 cases, 12 cases (75%) were positive for p53 in ACD lesions. There was a significant correlation between the expression of p53 protein in malignant cells and ACD (P = 0.001). All 44 ACD lesions had no staining of c-erbB2, regardless of staining in malignant lesions. The mean Ki-67 labeling index of ACD lesions was low (0.3%), suggesting that ACD had a low proliferative rate. We suggest that ACD is the precancerous breast lesion because of a histologic continuum between ACD and malignancy, and because of p53 protein expression in ACD.

  12. [Clinicopathological characteristics of atypical cystic duct (ACD) of the breast: assessment of ACD as a precancerous lesion].

    Science.gov (United States)

    Fu, Li; Fu, Xiao-Ying; Kusama, Ritsu; Matsuyama, Ikuo; Liu, Tong-Hua; Tsuchiya, Shinichi

    2004-06-01

    To assess the clinicopathological features of atypical cystic duct (ACD) as a precancerous lesion of the breast. Whole mammary gland serial sections were performed on 200 cases of breast cancer without pre-operative biopsy (prior operation, fine needle aspiration or needle biopsy were routinely performed in each case). The clinicopathological findings and immunohistochemical features of ACD were investigated. Forty-four (22%) of the 200 breast cancer patients had ACD breast lesions. The frequency of patients with ACD increased in premenopausal women (P=0.001). A number of ACD lesions displayed a histological transition to adjacent ductal carcinoma in-situ. In 16 of 44 (36%) patients with ACD, carcinoma cells stained positive for p53. In 12 of these 16 cases (75%), ACD cells also stained positive for p53 protein (P=0.001). Myoepithelial cells of ACD appeared attenuated and stained strongly for alpha-smooth muscle actin. There was no correlation between the ACD-present group and the ACD-absent group in tumor size, nodal metastasis, and immunostaining patterns of estrogen receptor (ER), progesterone receptor (PR), p53, c-erbB-2 and Ki-67 labeling index of cancerous tissues. All 44 ACD lesions showed a negative staining of c-erbB-2, regardless of the staining result in their corresponding carcinomas. The mean Ki-67 labeling index of ACD lesions was low. ACD is frequently associated with breast cancer. It may represent a precancerous mammary lesion, supported by the frequent histological continuum between ACD and malignancy, and simultaneous p53 over-expression present in both ACD and its corresponding breast carcinoma.

  13. Mirizzi Syndrome: A Case Report | Muthuuri | East and Central ...

    African Journals Online (AJOL)

    The classical Mirizzi Syndrome described by P. L. Mirizzi in 1948 is characterized by a cholecystocholedochal fistula arising from a calculus in the cystic duct that erodes into the common hepatic duct. The gall bladder eventually collapses due to fibrosis while the terminal bile ducts become dilated. A calculus is usually ...

  14. Classification conundrum: Persistent mullerian duct syndrome with hypospadias

    Directory of Open Access Journals (Sweden)

    Sheetal Arora

    2016-01-01

    Full Text Available The disorders of sex development (DSD are uncommon and have wide phenotypic variation. Due to this, they often cannot be classified properly and go unreported. This case is an 18-month-old child with penoscrotal hypospadias with bilateral undescended testes. The karyotyping was 46 XY. Serum testosterone and progesterone were normal. Histopathological examination showed the right gonad with few testicular tubules containing sertoli cells and fibro-collagenization of the stroma. The left Mullerian structures showed epididymis with fallopian tube, with absent testis. Persistent Mullerian duct with hypospadias is a rare anomaly, not classifiable under any of the 46 XY DSD. The possible genesis of this unique case is due to markedly reduced sertoli cells producing low levels of anti-Mullerian hormone which led to the failure of regression of Mullerian duct structures. The associated hypospadias is probably because of impaired action of testosterone possibly due to the deficiency of 5β reductase enzyme.

  15. Persistent Mullerian Duct Syndrome presenting as irreducible inguinal hernia – A surprise surgical finding!

    OpenAIRE

    Sekhon, V.; Luthra, M.; Jevalikar, G.

    2017-01-01

    Persistent Mullerian Duct Syndrome (PMDS) is diagnosed as a discrepancy between masculine external genitalia and female internal genitalia during surgery for cryptorchidism or inguinal hernia. Approximately 200 cases have been reported in the literature so far, most of whom are adults. We discuss the management in an infant presenting with irreducible inguinal hernia with contralateral undescended testis.

  16. Sebaceous adenoma arising within an ovarian mature cystic teratoma in Muir-Torre syndrome.

    Science.gov (United States)

    Smith, Jason; Crowe, Karen; McGaughran, Julie; Robertson, Thomas

    2012-12-01

    This is the first reported case of a sebaceous adenoma arising within an ovarian mature cystic teratoma in a patient with Muir-Torre syndrome. The pathologic findings and a literature review are presented, including the importance and possible benefits of an early diagnosis of Muir-Torre syndrome. It is proposed that the presence of a sebaceous adenoma in an ovarian cystic teratoma may serve as a useful trigger to consider further history and investigations, with the goal of identifying an important genetic cancer predisposition syndrome. Crown Copyright © 2012. Published by Elsevier Inc. All rights reserved.

  17. Lung cystic disease and Sjogren's syndrome: Report of the first case in Colombia and revision of the literature

    International Nuclear Information System (INIS)

    Rojas Villarraga, Adriana; Mendez Patarroyo, Paul; Iglesias Gamarra, Antonio; Rondon Herrera, Federico; Sanchez Contreras, Alvaro; Restrepo Suarez, Jose Felix

    2002-01-01

    In this paper we present a patient with primary Sjogren's syndrome, autoimmune hypothyroidism, and diffuse cystic lung Involvement. A complete literature review about cystic and bollous long involvement in Sjogren's syndrome is presented. This is the first report in Colombia of this disease

  18. Persistent mullerian duct syndrome with teratoma in an ectopic testis: imaging features

    Energy Technology Data Exchange (ETDEWEB)

    Narlawar, R.S.; Patankar, T. [Dept. of Radiology, Seth G. S. Medical College and K. E. M. Hospital, Parel, Mumbai (India); Shah, J.R.; Parikh, V. [Dept. of CT and Ultrasound, Research Centre, Mumbai, Maharashtra (India)

    2001-06-01

    The persistent mullerian duct syndrome represents a rare form of male pseudohermaphroditism, secondary to mullerian inhibiting factor (MIF) deficiency. We describe imaging findings in a 30-year-old male (46 XY karyotype) with bilateral cryptorchidism and mullerian duct anomalies (presence of uterus and fallopian tubes). Grade-III teratoma with yolk sac tumour was detected in one of the undescended testis, lying in the pelvic cavity. The other testis was in the inguinal canal. The rest of the wolffian duct structures (e. g. prostate, seminal vesicles) were nearly normal. Very few reports of imaging findings of this entity have been published thus far, probably because of the rarity of entity, incidental detection of most of the cases at surgery and relatively asymptomatic clinical presentation. (orig.)

  19. Persistent Mullerian Duct Syndrome: a rare entity with a rare presentation in need of multidisciplinary management

    Directory of Open Access Journals (Sweden)

    Lin Da Aw

    Full Text Available ABSTRACT Main findings: A typical male looking adolescent with a legal female gender assignment presented with haematuria. Investigations led to the diagnosis of Persistent Mullerian Duct Syndrome. The condition is indeed a rare entity that needs a multidisciplinary team management. Case hypothesis: A case of Persistent Mullerian Duct Syndrome undiagnosed at birth because karyotyping was defaulted, thus resulting in a significant impact on the legal gender assignment and psychosocial aspects. Promising future implications: The reporting of this case is important to create awareness due to its rarity coupled with the rare presentation with hematuria as a possible masquerade to menstruation. There were not only medical implications, but also psychosocial and legal connotations requiring a holistic multidisciplinary management.

  20. Management of post cholecystectomy Mirizzi′s syndrome

    Directory of Open Access Journals (Sweden)

    Janes Simon

    2005-01-01

    Full Text Available Various strategies have been proposed for the management of retained calculi within the biliary tree following cholecystectomy. We present a unique case of a cystic duct remnant calculus causing Mirizzi syndrome, only the fourth such case of its kind. An open procedure was planned, however the calculus was eventually extracted endoscopically. The pathophysiology and management of Mirizzi syndrome and retained calculi within the cystic duct remnant are discussed along with the merits of a minimally invasive approach.

  1. Cystic Fibrosis Transmembrane Conductance Regulator-Related Metabolic Syndrome and Cystic Fibrosis Screen Positive, Inconclusive Diagnosis.

    Science.gov (United States)

    Ren, Clement L; Borowitz, Drucy S; Gonska, Tanja; Howenstine, Michelle S; Levy, Hara; Massie, John; Milla, Carlos; Munck, Anne; Southern, Kevin W

    2017-02-01

    An unintended consequence of cystic fibrosis (CF) newborn screening (NBS) is the identification of infants with a positive NBS test but inconclusive diagnostic testing. These infants are classified as CF transmembrane conductance regulator-related metabolic syndrome (CRMS) in the US and CF screen positive, inconclusive diagnosis (CFSPID) in other countries. Diagnostic and management decisions of these infants are challenges for CF healthcare professionals and stressful situations for families. As CF NBS has become more widespread across the world, increased information about the epidemiology and outcomes of these infants is becoming available. These data were reviewed at the 2015 CF Foundation Diagnosis Consensus Conference, and a harmonized definition of CRMS and CFSPID was developed. At the consensus conference, participants reviewed published and unpublished studies of CRMS/CFSPID and used a modified Delphi methodology to develop a harmonized approach to the definition of CRMS/CFSPID. Several studies of CRMS/CFSPID from populations around the world have been published in the past year. Although the studies vary in the number of infants studied, study design, and outcome measures, there have been some consistent findings. CRMS/CFSPID occurs relatively frequently, with CF:CRMS that ranges from 3 to 5 cases of CF for every 1 case of CRMS/CFSPID in regions where gene sequencing is not used. The incidence varies by NBS protocol used, and in some regions more cases of CRMS/CFSPID are detected than cases of CF. The majority of individuals with CRMS/CFSPID do not develop CF disease or progress to a diagnosis of CF. However, between 10% and 20% of asymptomatic infants can develop clinical features concerning for CF, such as a respiratory culture positive for Pseudomonas aeruginosa. Most studies have only reported short-term outcomes in the first 1-3 years of life; the long-term outcomes of CRMS/CFSPID remain unknown. The European CF Society definition of CFSPID and the

  2. Coincidence of Persistent M?llerian duct syndrome and testicular tumors in dogs

    OpenAIRE

    Park, Eun Jung; Lee, Seok-Hee; Jo, Young-Kwang; Hahn, Sang-Eun; Go, Do-Min; Lee, Su-Hyung; Lee, Byeong-Chun; Jang, Goo

    2017-01-01

    Background Persistent M?llerian duct syndrome (PMDS), a rare form of male pseudohermaphroditism in dogs, is an abnormal sexual phenotype in males that is characterized by the existence of a hypoplastic oviduct, uterus, and cranial part of the vagina. Dogs suffering from PMDS are often accompanied by cryptorchidism. To date, it has been mainly found in the Miniature Schnauzer breed. Case presentation In this report, two cases of PMDS with a malignant testicular tumor originating from cryptorch...

  3. First report of small cell lung cancer with PTHrP-induced hypercalcemic pancreatitis causing disconnected duct syndrome.

    Science.gov (United States)

    Montminy, Eric M; Landreneau, Stephen W; Karlitz, Jordan J

    2017-10-10

    Here we report a patient diagnosed with small cell lung cancer after first presenting with parathyroid hormone-related peptide-induced hypercalcemic pancreatitis and developed walled-off necrosis that resulted in disruption of the main pancreatic duct. Disconnected duct syndrome (DDS) is a rare syndrome that occurs when the main pancreatic duct exocrine flow is disrupted resulting in leakage of pancreatic enzymes and further inflammatory sequela. To date, no prior reports have described DDS occurring with paraneoplastic reactions. Diagnostic imaging techniques and therapeutic interventions are reviewed to provide insight into current approaches to DDS.

  4. Primary Sjogrens syndrome, with lymphocytic interticial pneumonia and cystic pulmonary disease

    International Nuclear Information System (INIS)

    Vallejo, Jimi Mejia; Bayona, Jorge Carrillo; Iglesias Gamarra, Antonio; Rondon Federico Sanchez Contreras Alvaro, Restrepo Jose Felix

    2007-01-01

    The primary Sjogrens syndrome (PSS) is a chronic autoimmune exocrinopathy characterized by lymphocytic infiltration of glandular and extraglanduIar tissue, the lung involvement is heterogeneous; the aim of this study is report the first case in Colombia of PSS with Iymphocytic intersticiaI pneumonia and cystic pulmonary disease discuss differential diagnosis and the initial diagnostic and therapeutic work up

  5. Preoperative evaluation of the cystic duct for laparoscopic cholecystectomy: comparison of navigator-gated prospective acquisition correction- and conventional respiratory-triggered techniques at free-breathing 3D MR cholangiopancreatography

    Energy Technology Data Exchange (ETDEWEB)

    Itatani, Ryo [Kumamoto Chuo Hospital, Department of Radiology, Kumamoto (Japan); Kumamoto University, Department of Diagnostic Radiology, Graduate School of Medical Sciences, Kumamoto (Japan); Namimoto, Tomohiro; Sakamoto, Fumi; Yamashita, Yasuyuki [Kumamoto University, Department of Diagnostic Radiology, Graduate School of Medical Sciences, Kumamoto (Japan); Kajihara, Hiroo; Yoshimura, Akira; Katahira, Kazuhiro [Kumamoto Chuo Hospital, Department of Radiology, Kumamoto (Japan); Nasu, Jiro [Kumamoto Chuo Hospital, Department of Digestive Surgery, Kumamoto (Japan); Matsushita, Ikuo [Kumamoto Chuo Hospital, Department of Gastroenterological Medicine, Kumamoto (Japan); Kidoh, Masafumi [Amakusa Medical Center, Department of Diagnostic Radiology, Amakusa, Kumamoto (Japan)

    2013-07-15

    To evaluate the quality of magnetic resonance cholangiopancreatography (MRCP) images obtained with a three-dimensional navigator-gated (NG) technique and compare findings with conventional respiratory-triggered (RT) images in pre-laparoscopic cholecystectomy patients. Turbo-spin-echo (TSE) RT-MRCP (average 242 s) and balanced turbo-field-echo (bTFE) NG-MRCP (average 263 s) were acquired at 1.5-T MRI for 49 pre-laparoscopic cholecystectomy patients. Two radiologists independently assessed image quality, visibility of anatomical structures, common bile duct (CBD) stones, and signal-to-noise ratios (SNRs). Interobserver agreement was also evaluated. The anatomical details of the cystic duct were clearly demonstrated in 33 (67.3 %, reader A) and 35 (71.4 %, reader B) patients on RT-MRCP, and in 45 (91.8 %) and 44 (89.7 %) patients on NG-MRCP. On NG-MRCP, visualisation of the cystic duct (3.22/3.12), its origin (3.57/3.55), and the gallbladder(3.61/3.59) was statistically better than on RT-MRCP (2.90/2.78, 3.29/3.12, 2.98/2.88, respectively). The overall image quality was statistically better on NG-MRCP than RT-MRCP. Each technique identified the presence of CBD stones in all affected patients. The SNR was significantly higher on NG-MRCP (CHD 22.40, gallbladder 17.13) than RT-MRCP (CHD 17.05, gallbladder 9.30). Interobserver agreement was fair to perfect. Navigator-gated MRCP is more useful than respiratory-triggered MRCP for evaluating the gallbladder and cystic duct in patients scheduled for laparoscopic cholecystectomy. circle Magnetic resonance cholangiopancreatography (MRCP) provides important cystic duct information before laparoscopic cholecystectomy. (orig.)

  6. Endopancreatic Bile Duct Cholangiocarcinoma in a Patient with Peutz-Jeghers Syndrome

    Directory of Open Access Journals (Sweden)

    Alexandros K. Charalabopoulos

    2011-01-01

    Full Text Available Peutz-Jeghers syndrome is a rare autosomal dominant inherited disease characterized by a special type of hamartomatous gastrointestinal polyps combined with mucocutaneous melanin pigmentations. Patients with the syndrome have a high risk of developing neoplasia, with colon, small bowel, and stomach being the most common gastrointestinal sites. Herein, we present the occurrence of a rare tumor in patients with Peutz-Jeghers syndrome; a cholangiocarcinoma of the endopancreatic bile duct. A minireview is also presented. It can be concluded that cholangiocarcinoma remains a possible diagnosis in PJS patients, as in others that present with biliary obstruction. PJS patients may be at higher risk than others in view of their propensity for malignancy.

  7. Atypical presentation of cystic fibrosis: Obese adolescent with hypertension and pseudo-Bartter’s syndrome

    Directory of Open Access Journals (Sweden)

    Sovtić Aleksandar

    2012-01-01

    Full Text Available Introduction. Infants with cystic fibrosis may fail to thrive despite recommended caloric intake because of electrolyte disurbances caused by salt depletion resulting in hypochloremic metabolic alkalosis or pseudo-Bartter's syndrome. In most patients reported symptoms began in infancy, but it may be an initial presentation of disease in a previously healthy adolescent. Case report. A 15-year-old boy was admitted for evaluation of recurrent episodes of malaise associated with dehydration and acute renal insufficiency. Laboratory analysis showed hypochloremic metabolic alkalosis with hyponatremia and hypokalemia. On admission the boy was obese, with body weight of 95.5 kg (> P97, height 174 cm (> P75, and body mass index of 31.2 kg/m2 (> P95. Physical examination was inconclusive. Blood pressure holter monitoring proved significant systolic hypertension. Routine urinalysis, protein and electrolyte levels in urine were normal. Plasma renin and aldosteron were normal. Sweat chloride concentration was 63 mmol/L. Genetic testing confirmed the diagnosis of cystic fibrosis. Conclusion. To our knowledge, this is the first reported case of atypical presentation of cystic fibrosis in an adolescent presented with pseudo-Bartter's syndrome and signs of obesity and hypertension. We suggest that every patient with hypochloremic metabolic alkalosis should be evaluated for cystic fibrosis.

  8. Persistent Mullerian duct syndrome presenting as an inguinal hernia : A case report

    Directory of Open Access Journals (Sweden)

    Amit Dangi

    2016-10-01

    Full Text Available A brief report of persistent mullerian duct syndrome (PMDS with 46XY karyotype which is one of the rarest variety of disorders of sexual differentiation (DSD accounting only 5% cases of all is being presented. A 21 years old male with left inguinal hernia and absent right testis presented in surgical outdoor and was operated. On exploration female genital organs like uterus and fallopian tubes along with contralateral testis were present in left inguinal canal as a content of sliding left inguinal hernia.

  9. Vanishing bile duct syndrome and immunodeficiency preceding the diagnosis of Hodgkin lymphoma.

    Science.gov (United States)

    Yeh, P; Lokan, J; Anantharajah, A; Grigg, A

    2014-12-01

    Vanishing bile duct syndrome (VBDS) in association with Hodgkin lymphoma (HL) is well described but not well understood. We report an unusual case of a 75-year-old patient presenting with biopsy-proven VBDS and immunodeficiency, without identifiable cause, which showed a waxing and waning course, culminating in the development of HL 18 months later. To our knowledge, this is the first adult case in which VBDS preceded the diagnosis of HL by such a long period. © 2014 The Authors; Internal Medicine Journal © 2014 Royal Australasian College of Physicians.

  10. Are there any association between polycistic ovary syndrome and congenital abnormalities of Müllerian ducts.

    Science.gov (United States)

    Tubić-Pavlović, Aleksandra; Radović-Janosević, Dragana; Petrić, Aleksandra; Stefanović, Milan

    2014-06-01

    There are many specificities of merital infertility and sometimes surprising connections between some thinks with no connections at first sight. Examinations of these patients imply diagnostic actions such as the blood basal hormone sample, doing hysterosalpingography, ultrahysterosonography, ultrasound examinations, and sometimes laparoscopy and hysteroscopy if there are necessary. The aim of the study was to determine the characteristics of the connection between policystic ovary (PCO) syndrome (Sy) and congenital Müllerian ducts abnormalities. This study included 356 patients treated in the period from January 1, to December 31, 2009, in the Department of Infertility of the Clinic for Obstetrics and Gynecology in Nis, Serbia. Exclusion criteria were no myoma, ovary cysts, tubal and male factors of infertility. A total of 180 patients were divided into 3 groups: the group I with PCO sy, the group II with uterine congenital malformation and the group III with a combination of these disorders. The middle age of patients was 29.6 +/- 4.8, body mass index (BMI) was 26.1 +/- 4,8 kg/m2 the middle thicknes of endometrium was 5.2 + 2.7 mm, and there were no significant differences between the examined groups. There were no significant among in a number of miscarriages in the examined groups. We found that PCO Sy and congenital abnormalities of Müllerian ducts were conjoint in 30% of examined patients. Conjoined PCO Sy and congenital abnormalities of Müllerian ducts do not result in a higher number of miscarriages than only either PCO Sy or abnormalities of Müllerian ducts. It is important to check BMI, basal level of follicle stimulating hormone and number of antral follicles because the induction protocol and concentracion of inductors depends on these characteristics, thus, the succsessful cycles and pregnancy.

  11. Collecting duct renal cell carcinoma with the syndrome of inappropriate antidiuresis: An autopsy case report

    Directory of Open Access Journals (Sweden)

    Emi Yasuda

    2013-01-01

    Full Text Available A 57-year-old Japanese man visited our hospital with a moist cough. Chest radiographic imaging showed a left hilar shadow. Adenocarcinoma cells were found on cytologic screening of fresh sputum. Although multiple metastases including brain were detected, no tumor was observed in the kidneys. The patient underwent whole-brain irradiation and chemotherapy for advanced-stage lung cancer. One month before his death, carcinomatous meningitis was detected. Hyponatremia, hypo-osmolality, and hypertonic urine suggested the syndrome of inappropriate antidiuresis. Restricting water intake improved the hyponatremia; however, he developed fever and hematuria. Despite systemic administration of an antibacterial drug, he died. Primary tumor in the lung was absent, but adenocarcinoma of the right kidney was evident on autopsy. Lectin histochemical analysis of the carcinoma revealed its distal nephron origin, confirming collecting duct carcinoma. Severe carcinomatous meningitis, which is possibly caused the syndrome of inappropriate antidiuresis, was observed, with no cancer involvement of the pituitary gland and hypothalamus.

  12. Typical radiological findings in Mirizzi's syndrome

    International Nuclear Information System (INIS)

    Strunk, H.; Teifke, A.; Menke, H.

    1988-01-01

    In extrahepatic cholestasis it is necessary to include Mirizzi's syndrome in the differential diagnostic considerations. Characteristic findings in sonography and computed tomography ae: a stone incorporated in the neck of the gallbladder or cystic duct with an extension of the common hepatic duct above the stone and normal width of the bile duct below the sone; in endoscopic retrograde cholangiography (ERC, direkt cholangiography), a characteristic finding is a smooth-walled, segmental stenosis through external compression or an incorporated stone. (orig.) [de

  13. Cystic fibrosis, common variable immunodeficiency and Aspergers syndrome: an immunological and behavioural challenge.

    LENUS (Irish Health Repository)

    Chotirmall, S H

    2009-08-07

    INTRODUCTION: Cystic fibrosis (CF) is of particular importance in Ireland as the Irish population has both the highest incidence (2.98\\/10,000) and the highest carrier rate (1 in 19) in the world. Primary immunodeficiency has not been previously reported as co-existing with CF. CASE REPORT: We report a unique case of CF associated with a primary immunodeficiency syndrome-common variable immunodeficiency (CVID). DISCUSSION: Our patient has CF, CVID and the additional comorbidity of Aspergers syndrome. The challenges inherent in diagnosing and treating such a case are outlined herein and the successful management of this case is evidenced by the well-preserved lung function of our patient.

  14. Cystic fibrosis, common variable immunodeficiency and Aspergers syndrome: an immunological and behavioural challenge.

    LENUS (Irish Health Repository)

    Chotirmall, S H

    2012-02-01

    INTRODUCTION: Cystic fibrosis (CF) is of particular importance in Ireland as the Irish population has both the highest incidence (2.98\\/10,000) and the highest carrier rate (1 in 19) in the world. Primary immunodeficiency has not been previously reported as co-existing with CF. CASE REPORT: We report a unique case of CF associated with a primary immunodeficiency syndrome--common variable immunodeficiency (CVID). DISCUSSION: Our patient has CF, CVID and the additional comorbidity of Aspergers syndrome. The challenges inherent in diagnosing and treating such a case are outlined herein and the successful management of this case is evidenced by the well-preserved lung function of our patient.

  15. Cystic fibrosis, common variable immunodeficiency and Aspergers syndrome: an immunological and behavioural challenge.

    Science.gov (United States)

    Chotirmall, S H; Low, T B; Hassan, T; Branagan, P; Kernekamp, C; Flynn, M G; Gunaratnam, C; McElvaney, N G

    2011-06-01

    Cystic fibrosis (CF) is of particular importance in Ireland as the Irish population has both the highest incidence (2.98/10,000) and the highest carrier rate (1 in 19) in the world. Primary immunodeficiency has not been previously reported as co-existing with CF. We report a unique case of CF associated with a primary immunodeficiency syndrome--common variable immunodeficiency (CVID). Our patient has CF, CVID and the additional comorbidity of Aspergers syndrome. The challenges inherent in diagnosing and treating such a case are outlined herein and the successful management of this case is evidenced by the well-preserved lung function of our patient.

  16. Coincidence of Persistent Müllerian duct syndrome and testicular tumors in dogs.

    Science.gov (United States)

    Park, Eun Jung; Lee, Seok-Hee; Jo, Young-Kwang; Hahn, Sang-Eun; Go, Do-Min; Lee, Su-Hyung; Lee, Byeong-Chun; Jang, Goo

    2017-06-02

    Persistent Müllerian duct syndrome (PMDS), a rare form of male pseudohermaphroditism in dogs, is an abnormal sexual phenotype in males that is characterized by the existence of a hypoplastic oviduct, uterus, and cranial part of the vagina. Dogs suffering from PMDS are often accompanied by cryptorchidism. To date, it has been mainly found in the Miniature Schnauzer breed. In this report, two cases of PMDS with a malignant testicular tumor originating from cryptorchidism in breeds other than the Miniature Schnauzer breed are described. The patients were a seven-year-old male Maltese dog and a 17-year-old male mixed-breed dog weighing 3.8 kg. They also exhibited an enlarged prostate with or without abscess and an elevated serum estradiol level and were surgically treated to remove the testicular tumor and Müllerian duct derivatives. It is recommended that PMDS should be differentially diagnosed by ultrasonography and that orchiectomy be performed at an early age in patients suspected to have cryptorchidism to prevent the ectopic testes from becoming tumorous.

  17. Cirrose biliar em felinos associada à ectasia do ducto cístico e desvios portossistêmicos extra-hepáticos Biliary cirrhosis in cats associated with cystic duct ectasia and extra-hepatic portosystemic Shunts

    Directory of Open Access Journals (Sweden)

    Marcia Regina da Silva Ilha

    2004-08-01

    (ectasia, extrahepatic portosystemic venous shunts, cavitary effusions and thin carcasses. Microscopic lesions included severe periportal, dissecting fibrosis with lymphoplasmacytic inflammation, biliary proliferation and cholestasis. In one case, Brown-Hopps’ stained slides revealed gram-positive cocci with associated inflammation inside the ductal lumen. The histological finding of intralesional bacteria in cases of feline cholangitis/cholangiohepatis complex is reported only on rare occasions and has not been described for biliary cirrhosis. Dilation of the cystic duct and formation of portosystemic shunts are also unusual complications of this syndrome. Biliary cirrhosis is an uncommon condition since most cats die or are euthanatized before reaching the final stage of this progressive inflammatory hepatic disease. The prevalence of this entity in local feline populations remains to be determined in Brazil.

  18. Occlusive vascular Ehlers-Danlos syndrome accompanying a congenital cystic adenomatoid malformation of the lung: report of a case.

    Science.gov (United States)

    Sa, Young Jo; Kim, Young Du; Moon, Seok-Whan; Kim, Chi-Kyung; Ki, Chang Seok

    2013-12-01

    An 8-year-old male presented with a cystic lung lesion in the left lower lobe, which was initially detected during surgery for a spontaneous rupture of the sigmoid colon at the age of 6 years. Tissue fragility and a tendency to bleed easily were noted during the surgery, which strongly suggested vascular Ehlers-Danlos syndrome. Although there was no abnormality in the hemostasis screening test, or any suspicious hereditary problem in his pedigree, genetic gene testing for vascular Ehlers-Danlos syndrome was recommended, and showed a de novo mutation in the COL3A1 gene. This report presents the case of patient with occlusive vascular Ehlers-Danlos syndrome accompanying a congenital cystic adenomatoid malformation of lung, in addition to a duplicated infrarenal vena cava.

  19. Cushing's syndrome due to pharmacological interaction in a cystic fibrosis patient.

    Science.gov (United States)

    Main, K M; Skov, M; Sillesen, I B; Dige-Petersen, H; Müller, J; Koch, C; Lanng, S

    2002-01-01

    Treatment of allergic bronchopulmonary aspergillosis with itraconazole is becoming more widespread in chronic lung diseases. A considerable number of patients is concomitantly treated with topical or systemic glucocorticoids for anti-inflammatory effect. As azole compounds inhibit cytochrome P450 enzymes such as CYP3A isoforms, they may compromise the metabolic clearance of glucocorticoids, thereby causing serious adverse effects. A patient with cystic fibrosis is reported who developed iatrogenic Cushing's syndrome after long-term treatment with daily doses of 800 mg itraconazole and 1,600 microg budesonide. The patient experienced symptoms of striae, moon-face, increased facial hair growth, mood swings, headaches, weight gain, irregular menstruation despite oral contraceptives and increasing insulin requirement for diabetes mellitus. Endocrine investigations revealed total suppression of spontaneous and stimulated plasma cortisol and adrenocorticotropin. Discontinuation of both drugs led to an improvement in clinical symptoms and recovery of the pituitary-adrenal axis after 3 mo. This observation suggests that the metabolic clearance of buDesonide was compromised by itraconazole's inhibition of cytochrome P450 enzymes, especially the CYP3A isoforms, causing an elevation in systemic budesonide concentration. This provoked a complete suppression of the endogenous adrenal function, as well as iatrogenic Cushing's syndrome. Patients on combination therapy of itraconazole and budesonide inhalation should be monitored regularly for adrenal insufficiency. This may be the first indicator of increased systemic exogenous steroid concentration, before clinical signs of Cushing's syndrome emerge.

  20. Imaging and clinical findings in large endolymphatic duct and sac syndrome.

    Science.gov (United States)

    Koesling, Sabrina; Rasinski, Christine; Amaya, Beatrice

    2006-01-01

    Large endolymphatic duct and sac syndrome (LEDS) is known as the most common kind of inner ear malformations, which is radiologically detectable. Nevertheless, nowadays many questions are not fully cleared and LEDS is relatively unknown among general radiologists. The aim of this study was to evaluate the incidence of LEDS in the own patient population and to present our experiences regarding imaging findings, clinical presentation and follow up. Based on a complete recording of all patients, sent from ENT department to radiology, we identified all radiological diagnosed cases of inner ear malformations including LEDS and all patients in whom an inner ear malformation was clinically suspected. The retrospective study included clinical records, HR-CT and MRI performed between 1994 and 2002. Among 169 patients (338 ear), 17 of patients (median age: 12 years) and 28 ears, respectively, had enlarged endolymphatic structures. In 10 patients - 6% - (15 ears), no other abnormalities were detected, called isolated LEDS, seven patients showed additional inner ear abnormalities. One patient showed a labyrinthine hemorrhage after sudden hearing loss. Audiometric data revealed sensorineural hearing loss in 22 ears, deafness in 5 ears and normal hearing in 1 case of 28 ears. In 10 (67%) of 15 ears with isolated LEDS, the hearing loss was downward-fluctuating progressive. Twelve patients (eight with isolated LEDS) had partly repeated sudden hearing losses. A trigger for worsening of hearing was found in five patients. A correlation between the severity of morphological changes on imaging and the degree of hearing disturbances could not be detected. Only four young patients underwent a radiological examination within the first or second year after onset of hearing loss. Three patients received a cochlear implant. LEDS might be the cause of progressive hearing loss and repeated acute hearing losses in children and young adults. Imaging plays an important role in making the

  1. Imaging and clinical findings in large endolymphatic duct and sac syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Koesling, Sabrina [University of Halle, Department of Radiology, E-Grube-Street 40, D-06097 Halle (Germany)]. E-mail: sabrina.koesling@medizin.uni-halle.de; Rasinski, Christine [University of Halle, Department of Otorhinolaryngology (Germany); Amaya, Beatrice [University of Halle, Department of Radiology, E-Grube-Street 40, D-06097 Halle (Germany)

    2006-01-15

    Objective: Large endolymphatic duct and sac syndrome (LEDS) is known as the most common kind of inner ear malformations, which is radiologically detectable. Nevertheless, nowadays many questions are not fully cleared and LEDS is relatively unknown among general radiologists. The aim of this study was to evaluate the incidence of LEDS in the own patient population and to present our experiences regarding imaging findings, clinical presentation and follow up. Materials and methods: Based on a complete recording of all patients, sent from ENT department to radiology, we identified all radiological diagnosed cases of inner ear malformations including LEDS and all patients in whom an inner ear malformation was clinically suspected. The retrospective study included clinical records, HR-CT and MRI performed between 1994 and 2002. Results: Among 169 patients (338 ear), 17 of patients (median age: 12 years) and 28 ears, respectively, had enlarged endolymphatic structures. In 10 patients - 6% - (15 ears), no other abnormalities were detected, called isolated LEDS, seven patients showed additional inner ear abnormalities. One patient showed a labyrinthine hemorrhage after sudden hearing loss. Audiometric data revealed sensorineural hearing loss in 22 ears, deafness in 5 ears and normal hearing in 1 case of 28 ears. In 10 (67%) of 15 ears with isolated LEDS, the hearing loss was downward-fluctuating progressive. Twelve patients (eight with isolated LEDS) had partly repeated sudden hearing losses. A trigger for worsening of hearing was found in five patients. A correlation between the severity of morphological changes on imaging and the degree of hearing disturbances could not be detected. Only four young patients underwent a radiological examination within the first or second year after onset of hearing loss. Three patients received a cochlear implant. Conclusions: LEDS might be the cause of progressive hearing loss and repeated acute hearing losses in children and young

  2. Imaging and clinical findings in large endolymphatic duct and sac syndrome

    International Nuclear Information System (INIS)

    Koesling, Sabrina; Rasinski, Christine; Amaya, Beatrice

    2006-01-01

    Objective: Large endolymphatic duct and sac syndrome (LEDS) is known as the most common kind of inner ear malformations, which is radiologically detectable. Nevertheless, nowadays many questions are not fully cleared and LEDS is relatively unknown among general radiologists. The aim of this study was to evaluate the incidence of LEDS in the own patient population and to present our experiences regarding imaging findings, clinical presentation and follow up. Materials and methods: Based on a complete recording of all patients, sent from ENT department to radiology, we identified all radiological diagnosed cases of inner ear malformations including LEDS and all patients in whom an inner ear malformation was clinically suspected. The retrospective study included clinical records, HR-CT and MRI performed between 1994 and 2002. Results: Among 169 patients (338 ear), 17 of patients (median age: 12 years) and 28 ears, respectively, had enlarged endolymphatic structures. In 10 patients - 6% - (15 ears), no other abnormalities were detected, called isolated LEDS, seven patients showed additional inner ear abnormalities. One patient showed a labyrinthine hemorrhage after sudden hearing loss. Audiometric data revealed sensorineural hearing loss in 22 ears, deafness in 5 ears and normal hearing in 1 case of 28 ears. In 10 (67%) of 15 ears with isolated LEDS, the hearing loss was downward-fluctuating progressive. Twelve patients (eight with isolated LEDS) had partly repeated sudden hearing losses. A trigger for worsening of hearing was found in five patients. A correlation between the severity of morphological changes on imaging and the degree of hearing disturbances could not be detected. Only four young patients underwent a radiological examination within the first or second year after onset of hearing loss. Three patients received a cochlear implant. Conclusions: LEDS might be the cause of progressive hearing loss and repeated acute hearing losses in children and young

  3. Cystic endometrial hyperplasia-pyometra syndrome in bitches: identification of hemodynamic, inflammatory, and cell proliferation changes.

    Science.gov (United States)

    Veiga, Gisele Almeida Lima; Miziara, Ricardo Henrique; Angrimani, Daniel Souza Ramos; Papa, Paula Carvalho; Cogliati, Bruno; Vannucchi, Camila Infantosi

    2017-01-01

    Cystic endometrial hyperplasia (CEH)-pyometra syndrome is one of the most common diseases of noncastrated female dogs. However, determination of etiological mechanisms and differential diagnosis of CEH-pyometra syndrome are undefined. The aim of this study is to compare immunohistochemically the expression of cyclooxygenase-2 (COX-2) inflammatory mediator, Ki-67 antigen proliferation marker, vascular endothelial growth factor (VEGF-A) angiogenesis mediator and its FLT-1 and KDR receptors, and correlate with Doppler velocimetry of uterine artery and endometrial vascularization in bitches with CEH-pyometra syndrome. Bitches were allocated into CEH-mucometra Group (n = 13), Pyometra Group (n = 11), and Control Group (n = 8). Pyometra Group presented cytoplasmatic staining intensity for COX-2, VEGF-A, and FLT-1 and KDR receptors in luminal epithelium cells significantly higher compared to CEH-mucometra and Control groups. For the glandular epithelium, Pyometra Group had higher immunostaining score for VEGF-A and its receptors (FLT-1 and KDR). Hemodynamic indexes showed negative correlation with VEGF-A and its receptors as well as with COX-2. On the other hand, uterine vascularization score showed positive correlation in relation to immunostaining of COX-2, VEGF-A, and receptors in the endometrium luminal epithelium. In conclusion, uterus of bitches with CEH-pyometra syndrome show inflammatory process characterized by COX-2 expression, resulting in greater expression of proliferative Ki-67 marker as tissue response against the infectious agent. Furthermore, the increased VEGF-A expression and its receptors in CEH-pyometra reflect the increased blood flow and lower vascular resistance. Therefore, canine pyometra is characterized by an inflammatory, proliferative, and vascular disorder. © The Authors 2016. Published by Oxford University Press on behalf of Society for the Study of Reproduction. All rights reserved. For permissions, please journals.permissions@oup.com.

  4. Birt-Hogg-Dubé syndrome: characteristic CT findings differentiating it from other diffuse cystic lung diseases.

    Science.gov (United States)

    Lee, Jung Eun; Cha, Yoon Ki; Kim, Jeung Sook; Choi, Jin Ho

    2017-01-01

    Birt-Hogg-Dubé (BHD) syndrome is an uncommon, autosomal dominant, multiorgan systemic disorder manifesting as cutaneous fibrofolliculomas, lung cysts with or without spontaneous pneumothorax, and renal tumors. Spontaneous pneumothorax and lung cysts on chest computed tomography (CT) should lead to the inclusion of BHD syndrome in the differential diagnosis, because these findings may develop earlier than other clinical manifestations. Here, we review and describe the characteristic findings of BHD syndrome. The number, shape, size, and distribution of the lung cysts can help to differentiate BHD syndrome from other diffuse cystic lung diseases. Knowledge of the chest CT findings of BHD syndrome may lead to a correct diagnosis and the initiation of an appropriate work-up in order to prevent pneumothorax and for the early detection of renal tumors.

  5. [Pseudo-Bartter syndrome as manifestation of cystic fibrosis with DF508 mutation].

    Science.gov (United States)

    Galaviz-Ballesteros, María de Jesús; Acosta-Rodríguez-Bueno, Carlos Patricio; Consuelo-Sánchez, Alejandra; Franco-Álvarez, Isidro; Olalla-Mora, Odilo Iván; Vázquez-Frias, Rodrigo

    Pseudo Bartter syndrome (PBS) is defined as hypokalaemic hypochloraemic metabolic alkalosis in the absence of renal tubular pathology. Children with cystic fibrosis (CF) are at risk of developing electrolyte abnormalities and even PBS may occur. 5 months old female infant with a history of two events of dehydration with vomit, refusal to eat, chronic cough, polyuria, malnutrition, metabolic alkalosis, hypokalemia, hyponatremia, hypochloremia and acute renal failure. Chronic cough study was performed, discarding pulmonary tuberculosis, gastroesophageal reflux disease and impaired swallowing. PBS was diagnosed due to hypokalaemic hypochloraemic metabolic alkalosis in the absence of renal tubular pathology. CF was corroborated by electrolytes in sweat and through molecular analysis of the delta F508 mutation. This is one of the few reported cases linking PBS and this mutation. In patients with hyponatremic dehydration episodes with hypokalaemic hypochloraemic metabolic alkalosis, PBS should be considered as differential diagnosis. CF could be presented as PBS, mainly in patients younger than 2 years. Copyright © 2016 Hospital Infantil de México Federico Gómez. Publicado por Masson Doyma México S.A. All rights reserved.

  6. Unilateral right pulmonary artery agenesis and congenital cystic adenomatoid malformation of the right lung with Ortner′s syndrome

    Directory of Open Access Journals (Sweden)

    Jane Jackie David

    2016-01-01

    Full Text Available We report a 2.5-year-old girl who presented with hoarseness of voice since 3 months of age and failure to thrive. Chest X-ray showed cardiomegaly with a deviation of the trachea and mediastinum to the right side. Two-dimensional echocardiography showed decreased flow across the right pulmonary artery, a small atrial septal defect (ASD with a right-to-left shunt, and a dilated right atrium and right ventricle with severe tricuspid regurgitation suggestive of severe pulmonary hypertension. A silent large patent ductus arteriosus was also seen. Multiple detector computerized tomography aortogram confirmed the findings of absent right pulmonary artery and hypoplastic right lung with small cystic lesions suggestive of congenital cystic adenomatoid malformation in the right lower lobe. Hoarseness of voice was due to the left vocal cord palsy probably secondary to severe pulmonary hypertension (Ortner′s syndrome.

  7. Long-term follow-up of distal intestinal obstruction syndrome in cystic fibrosis

    Science.gov (United States)

    Lavie, Moran; Manovitz, Tzipora; Vilozni, Daphna; Levy-Mendelovich, Sarina; Sarouk, Ifat; Weintraubv, Ilana; Shoseyov, David; Cohen-Cymberknoh, Malena; Rivlin, Joseph; Efrati, Ori

    2015-01-01

    AIM: To investigate the long-term follow-up of distal intestinal obstruction syndrome (DIOS) in Israeli cystic fibrosis (CF) patients. METHODS: This is a multi-center, comparative, retrospective study in which we reviewed the medical records of all CF patients from three major CF centers in Israel who were treated in the period from 1980 to 2012. Patients diagnosed with DIOS were defined as the study group. The patients were diagnosed with DIOS based on their clinical presentation and typical findings on either abdominal X-ray or computerized tomography scan. For the control group, CF patients with no DIOS were matched to the patients in the study group for age, sex, and cystic fibrosis transmembrane conductance regulator (CFTR) mutations. For both groups, the collected data included age, sex, CFTR genotype, weight, height, and body mass index. Clinical data included respiratory function tests in the last five years prior to the study, respiratory function test immediately before and after the DIOS event, number of hospitalizations, sputum culture results, and CF-related conditions diagnosed according to the CF clinical practice guidelines. In the study group, data on the DIOS treatment and tendency for DIOS recurrence were also analyzed. RESULTS: The medical charts for a total of 350 CF patients were reviewed. Of the 350 CF patients, 26 (7.4%) were diagnosed with DIOS. The control group included 31 CF patients with no DIOS diagnosis. The mean follow-up period was 21.6 ± 8.2 years. The total of DIOS episodes in the follow-up period was 60. The distribution of DIOS episodes was as follows: 6/26 (23.1%) study patients had one episode of DIOS in their lifetime, 7/26 (26.9%) had two episodes, 7/26 (26.9%) had three episodes, and 6/26 (23.1%) had four or more episodes. Compared to the control group, DIOS patients had a significantly higher incidence of meconium ileus in the past (65.4% vs 0%, respectively, P DIOS, which mainly includes hydration and stool softeners

  8. Roux-en-Y drainage of a pancreatic fistula for disconnected pancreatic duct syndrome after acute necrotizing pancreatitis.

    Science.gov (United States)

    Pearson, Erik G; Scaife, Courtney L; Mulvihill, Sean J; Glasgow, Robert E

    2012-01-01

    After acute necrotizing pancreatitis (ANP), a pancreatic fistula may occur from disconnected pancreatic duct syndrome (DPDS) where a segment of the pancreas is no longer in continuity with the main pancreatic duct. To study the outcome of patients treated using Roux-Y pancreatic fistula tract-jejunostomy for DPDS after ANP. Between 2002 and 2011, patients treated for DPDS in the setting of endoscopic retrograde cholangiopancreatography (ERCP) or magnetic resonance cholangiopanreatography (MRCP) documented main pancreatic duct disruption with Roux-Y pancreatic fistula tract-jejunostomy. In all, seven patients with DPDS were treated. The median age was 62 years (range 49-78) and five were men. The cause of ANP was gallstones (2), alcohol (1), ERCP (1) and idiopathic (3). Pancreatic necrosectomy was done in six patients. Time from onset of pancreatitis to fistula drainage was 270 days (164-365). Pancreatic fistulae arose from DPDS in the head/neck (4) and body/tail (3). Patients had a median fistula output of 140 ml (100-200) per day before surgery. The median operative time was 142 min (75-367) and estimated blood loss was 150 ml (25 to 500). Patients began an oral diet on post-operative day 4 (3-6) and were hospitalized for a median of 7 days (5-12). The median follow-up was 264 days (29-740). Subsequently, one patient required a distal pancreatectomy. After surgery, three patients required oral hypoglycaemics. No patient developed pancreatic exocrine insufficiency. Internal surgical drainage using Roux-en-Y pancreatic fistula tract-jejunostomy is a safe and definitive treatment for patients with DPDS. © 2011 International Hepato-Pancreato-Biliary Association.

  9. Laparoscopic correction of transverse testicular ectopia with persistent Müllerian duct syndrome

    Directory of Open Access Journals (Sweden)

    Ji-Won Han

    2017-03-01

    An 11-month old male patient visited our hospital with swelling of the right inguinal area. The right testis was palpable in the right scrotum while left testis was not in the left scrotum. During laparoscopic exploration, the left testis was found around the right inguinal area, with the left spermatic cord across the pelvic cavity from left side. When the left testis was pulled, a tubular structure, Müllerian duct remnant, and the right spermatic cord came into the abdominal cavity. The Müllerian duct seemed like a uterus was located between both vas deferens. We divided it because the length of the bilateral vas deferens was not sufficient for orchiopexy. After laparoscopic right orchiopexy and left trans-scrotal approach orchiopexy, bilateral inguinal herniorrhaphy was performed laparoscopically.

  10. Relationship of the area measurement of the large endolymphatic duct and sac syndrome as well as the clinical symptoms with CT and MR imaging results

    International Nuclear Information System (INIS)

    Park, Ji Sang; Hong, Hyun Sook; Lee, Jong Sea; Kim, Dae Ho; Lee, Hae Kyung; Yi, Beom Ha; Cha, Jang Gyu; Park, Seong Jin; Kim, Shi Chan

    2008-01-01

    To evaluate the CT and MRI findings of the large endolymphatic duct or sac syndrome (LEDS) and its associated anomalies, with clinical features. We retrospectively reviewed the MR and CT images of 52 ears obtained from 26 patients with LEDS. We reviewed the clinical findings, audiology testing, and treatment results. The degree of hearing loss was classified from normal to profound, based on pure tone audiometry. The largest areas were measured at each endolymphatic duct and analyzed to determine whether a correlation exists with the degree of hearing loss. We also analyzed the differences in measurements between CT and MRI findings. All 26 patients had some degree of sensorineural hearing loss, which resulted in 18 ears to undergo a cochlear implantation. One patient was diagnosed with Cornelia de Lange syndrome. Five patients had a sudden hearing loss onset. Ten ears had incomplete cochlear partitions, whereas 28 ears had enlarged vestibules. All patients had severe to profound hearing loss. We found no statistical correlation between the size of the largest area of the endolymphatic duct and the degree of hearing loss. The mean area of the endolymphatic ducts, as per an MRI examination, revealed slightly greater areas than the CT findings, although the differences were not significant. Enlarged vestibules and incomplete partitions of the cochlea were common anomalies associated with LEDS. We found no statistical correlation between the largest area of the endolymphatic duct or sac with the degree of hearing loss

  11. Relationship of the area measurement of the large endolymphatic duct and sac syndrome as well as the clinical symptoms with CT and MR imaging results

    Energy Technology Data Exchange (ETDEWEB)

    Park, Ji Sang; Hong, Hyun Sook; Lee, Jong Sea; Kim, Dae Ho; Lee, Hae Kyung; Yi, Beom Ha; Cha, Jang Gyu; Park, Seong Jin; Kim, Shi Chan [Soonchunhyang University Bucheon Hospital, Bucheon (Korea, Republic of)

    2008-08-15

    To evaluate the CT and MRI findings of the large endolymphatic duct or sac syndrome (LEDS) and its associated anomalies, with clinical features. We retrospectively reviewed the MR and CT images of 52 ears obtained from 26 patients with LEDS. We reviewed the clinical findings, audiology testing, and treatment results. The degree of hearing loss was classified from normal to profound, based on pure tone audiometry. The largest areas were measured at each endolymphatic duct and analyzed to determine whether a correlation exists with the degree of hearing loss. We also analyzed the differences in measurements between CT and MRI findings. All 26 patients had some degree of sensorineural hearing loss, which resulted in 18 ears to undergo a cochlear implantation. One patient was diagnosed with Cornelia de Lange syndrome. Five patients had a sudden hearing loss onset. Ten ears had incomplete cochlear partitions, whereas 28 ears had enlarged vestibules. All patients had severe to profound hearing loss. We found no statistical correlation between the size of the largest area of the endolymphatic duct and the degree of hearing loss. The mean area of the endolymphatic ducts, as per an MRI examination, revealed slightly greater areas than the CT findings, although the differences were not significant. Enlarged vestibules and incomplete partitions of the cochlea were common anomalies associated with LEDS. We found no statistical correlation between the largest area of the endolymphatic duct or sac with the degree of hearing loss.

  12. A case of persistent Müllerian duct syndrome with sertoli cell tumor and hydrometra in a dog.

    Science.gov (United States)

    Matsuu, Aya; Hashizume, Takuya; Kanda, Teppei; Nagano, Masashi; Sugiyama, Akihiko; Okamoto, Yoshiharu; Hikasa, Yoshiaki

    2009-03-01

    A 10-year-old Miniature Schnauzer with bilateral cryptorchidism and male external genitalia was referred with a history of abdominal enlargement. Upon exploratory laparotomy, two tumors and a connecting structure similar to fluid-filled uterus were recognized. After cytological and bacterial examinations of the fluid and histological examination, this dog was diagnosed with bilateral Sertoli cell tumor with hydrometra. The karyotype of this dog was 78, XY and the sry gene was detected positive by PCR. We diagnosed this dog as a case of persistent Müllerian duct syndrome (PMDS), which is male pseudohermaphroditism. This is the first report regarding the incidence of PMDS in Miniature Schnauzers in Japan, and it suggests the involvement of a gene carrier.

  13. Distal intestinal obstruction syndrome (DIOS) in patients with cystic fibrosis after lung transplantation.

    Science.gov (United States)

    Morton, Jonathan R; Ansari, Nabila; Glanville, Allan R; Meagher, Alan P; Lord, Reginald V N

    2009-08-01

    Cystic fibrosis (CF) is the commonest inherited life-threatening disease in Caucasians. With increased longevity, more patients with CF are developing gastrointestinal complications including the distal intestinal obstruction syndrome (DIOS), in which ileocecal obstruction is caused by viscid mucofeculent material. The optimal management of DIOS is uncertain. The medical records of all patients with CF who underwent lung transplantation at this institution during a 15-year period were reviewed. The definition of DIOS required the presence of both clinical and radiological features of ileocecal obstruction. One hundred twenty-one patients with CF underwent lung transplantation during the study period. During a minimum 2-year follow-up, there were 17 episodes of DIOS in 13 (10.7%) patients. The development of DIOS was significantly associated with a past history of meconium ileus (odds ratio 20.7, 95% C.I. 5.09-83.9) or previous laparotomy (odds ratio 4.93, 95% C.I. 1.47-16.6). All six patients who developed DIOS during the transplantation admission had meconium ileus during infancy, and five had undergone pretransplant laparotomy for CF complications. First-line treatment for all patients was a combination of medication (laxatives, stool softeners, and bowel preparation formulas). This was successful in 14 of the 17 DIOS but needed to be given for up to 14 days. The other three patients required laparotomy with enterotomy and fecal disimpaction. This provided definitive resolution of DIOS except in one patient who presented late and died despite ileal decompression and ileostomy. DIOS occurred in approximately 10% of CF patients after lung transplantation. Patients with a history of meconium ileus or previous laparotomy are at high risk of developing DIOS. Patients with DIOS require early aggressive management with timely laparotomy with enterotomy and possible stoma formation when non-operative therapy is unsuccessful.

  14. [Syndrome of persisting mullerian duct with first manifestation in an adult].

    Science.gov (United States)

    Zastrow, S; Nippgen, J; Manseck, A; Wirth, M P

    2004-11-01

    We report on a 22-year-old male patient who presented with an intrapelvic tumor. CT and MRI showed a left pelvic abscess, which was drained. After regression of the abscess, we removed the causative tubular structure surgically, revealing a rudimentary genital structure with parts of the Mullerian and Wolffian ducts. This case report demonstrates an abscess formation as complication of a previously asymptomatic rudimentary genital structure, which was associated with further abnormalities, such as left testicular agenesia, perineoscrotal hypospadias and transverse testicular ectopia.

  15. A rare case of Cystic artery arising from Gastroduodenal artery ...

    African Journals Online (AJOL)

    The tortuous cystic artery arose outside hepatobiliary triangle, crossed the common bile duct anteriorly and was lying anterior to the cystic duct hiding it from view. On reaching the neck of gall bladder, it again travelled for short distance before its termination. The non-peritonealised surface of the gall bladder was receiving ...

  16. Laparoscopic management of transverse testicular ectopia with persistent mullerian duct syndrome

    Directory of Open Access Journals (Sweden)

    R S Kamble

    2015-01-01

    Full Text Available A 4-month-old male child presented with right undescended testis and left inguinal hernia with funiculitis. Ultrasonography showed funiculitis on the left side testis along with presence of 1.5 × 1 cm testis like structure just above left testis and empty right scrotal sac without any evidence of mullerian structures. On diagnostic laparoscopy, right testicular vessels were crossing from right to left and had uterus with both testes in left hernia sac. Mobilization of vessels, division of uterus, and hernia repair was done laparoscopically. On the review of literature, there is only one case report of total laparoscopic repair of transversetesticular ectopia (TTE with hernia without persistent mullerian duct (PMDS. The uniqueness of our case is that it had TTE with hernia and PMDS, which were totally managed by laparoscopy. On 6 months of follow-up, both the testes are palpable in scrotum.

  17. Solitary intrahepatic bile-duct cyst presenting with Jaundice

    International Nuclear Information System (INIS)

    Park, Jeong Mi; Chun, Ki Sung; Ha, Hyun Kwon; Shinn, Kyung Sub; Bahk, Yong Whee; Kim, Jun Gi

    1989-01-01

    Caroli's disease is an uncommon condition, and characterized by congenital segmental saccular dilatation of intrahepatic bile ducts. A case of Caroli's disease, manifested by only a large communicating cystic dilatation of left intrahepatic bile duct and causing extrinsic pressure over the extrahepatic bile duct, is presented. The patient was 43-year-old housewife, hospitalized because of abdominal distension and severe jaundice. To relieve jaundice and alleviate surgical intervention, percutaneous drainage of the bile-duct cyst preceded surgery

  18. Serial MR imaging studies in enlarged endolymphatic duct and sac syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Naganawa, Shinji; Koshikawa, Tokiko; Fukatsu, Hiroshi; Ishigaki, Takeo [Department of Radiology, Nagoya University School of Medicine, 65 Tsurumai-cho, Shouwa-ku, Nagoya 466-8550 (Japan); Nakashima, Tsutomu [Department of Otolaryngology, Nagoya University School of Medicine, 65 Tsurumai-cho, Shouwa-ku, Nagoya 466-8550 (Japan)

    2002-07-01

    Large vestibular aqueduct syndrome (LVAS) is a congenital disorder characterized by progressive or fluctuating sensorineural hearing loss of unknown etiology. Serial MR examinations were performed before and after the development of hearing loss in two patients with LVAS. The signal and volume of the enlarged endolymphatic sac (EES) vary even in ears with stable hearing. In ears with fluctuating hearing, changes in EES signals were observed in only one of two patients. The finding that the EES volume and signal intensity vary dynamically independently of hearing is important for future research into the pathophysiology of hearing loss in this syndrome. (orig.)

  19. Endourologic Intervention for Management of Infertility in a Man with Zinner Syndrome Resulting in a Natural Pregnancy

    OpenAIRE

    Aghaways, Ismaeel; Ahmed, Shyaw M.

    2016-01-01

    Abstract Background: Ipsilateral renal agenesis associated with seminal vesicular cysts is an uncommon finding. Zinner syndrome is a rare variant of wolffian duct anomalies with a triad of seminal vesicle cyst, ipsilateral renal agenesis, and male fertility problems due to ejaculatory duct obstruction (EDO). Case Presentation: A 28-year-old man with 6 years history of primary infertility presented with left-side lower abdominal pain. A palpable cystic mass was found on digital rectal examinat...

  20. Male form of persistent Mullerian duct syndrome type I (hernia uteri inguinalis presenting as an obstructed inguinal hernia: a case report

    Directory of Open Access Journals (Sweden)

    Gujar Nishikant N

    2011-12-01

    Full Text Available Abstract Introduction Persistent Mullerian duct syndrome is a rare form of male pseudo-hermaphroditism characterized by the presence of Mullerian duct structures in an otherwise phenotypically, as well as genotypically, normal man; only a few cases have been reported in the worldwide literature. We report the case of a 30-year-old man with unilateral cryptorchidism on the right side and a left-sided obstructed inguinal hernia containing a uterus and fallopian tube (that is, hernia uteri inguinalis; type I male form of persistent Mullerian duct syndrome coincidentally detected during an operation for an obstructed left inguinal hernia. Case presentation A 30-year-old South Indian man was admitted to our facility with a left-sided obstructed inguinal hernia of one day's duration. He had a 12-year history of inguinal swelling and an absence of the right testis since birth. Our patient had well developed masculine features. Local physical examination revealed a left-sided obstructed inguinal hernia with an absence of the right testis in the scrotum. Exploration of the inguinal canal revealed an indirect inguinal hernia containing omentum, the left corner of the uterus and a left fallopian tube. Extension of the incision revealed a well formed uterus, cervix and upper part of the vagina attached to the prostate by a thick fibrosed band. Total excision of the uterus, bilateral fallopian tubes and right testis was performed. A biopsy was taken from the left testis. The operation was completed by left inguinal herniorraphy. Histopathological examination of the hernial contents was consistent with that of a uterus and fallopian tubes without ovaries. Both testes were atrophied, with complete arrest of spermatogenesis. Post-operative karyotype analyses were negative for 46,XY and Barr bodies on buccal smear. A semen examination revealed azoospermia with a low serum testosterone level. Conclusions In cases of unilateral or bilateral cryptorchidism

  1. Prenatal ultrasonographic findings of renal cystic diseases of the fetus

    International Nuclear Information System (INIS)

    Cho, Jeong Yeon; Song, Mi Jin; Lee, Young Ho; Cho, Byung Jae; Hong, Sung Ran

    2002-01-01

    The renal cystic diseases of the fetus consist of variable types of multicystic dysplastic kidney, autosomal recessive polycystic kidney, autosomal dominant polycystic kidney, obstructive cystic renal dysplasia, simple renal cyst, medullary cystic disease, and various syndromes with associated cystic renal diseases. Prenatal US findings of renal cystic diseases were compared and analyzed with the pathologic and postnatal US findings to establish the differential points of renal cystic diseases.

  2. Identification of Bile Duct Paucity in Alagille Syndrome: Using CK7 and EMA Immunohistochemistry as a Reliable Panel for Accurate Diagnosis.

    Science.gov (United States)

    Herman, Haley K; Abramowsky, Carlos R; Caltharp, Shelley; Metry, Diana; Cundiff, Caitlin A; Romero, Rene; Gillespie, Scott E; Shehata, Bahig M

    2016-01-01

    Bile duct paucity is the absence or marked reduction in the number of interlobular bile ducts (ILBD) within portal tracts. Its syndromic variant, Alagille syndrome (ALGS), is a multisystem disorder with effects on the liver, cardiovascular system, skeleton, face, and eyes. It is inherited as an autosomal dominant trait due to defects in NOTCH signaling pathway. ALGS is characterized by vanishing ILBD with subsequent chronic obstructive cholestasis in approximately 89% of cases. Cholestasis stimulates formation of new bile ductules through a process of neoductular reaction, making it difficult to evaluate the presence or absence of ILBD. Therefore, finding a method to differentiate clearly between ILBD and the ductular proliferation is essential for accurate diagnosis. A database search identified 28 patients with confirmed diagnosis of ALGS between 1992 and 2014. Additionally, 7 controls were used. A panel of two immunostains, cytokeratin 7 (CK7) and epithelial membrane antigen (EMA), was performed. CK7 highlighted the bile duct epithelium of ILBD and ductular proliferation, while EMA stained only the brush border of ILBD. In our ALGS group, the ratio of EMA-positive ILBD to identified portal tracts was 12.6% (range, 0%-41%). However, this same ratio was 95.0% (range, 90%-100%) among control cases (P < 0.001). We propose a panel of two immunostains, CK7 and EMA, to differentiate ILBD from ductular proliferation in patients with cholestasis. With this panel, identification of bile duct paucity can be achieved. Additional studies, including molecular confirmation and clinical correlation, would provide a definitive diagnosis of ALGS.

  3. Disconnected pancreatic duct syndrome in severe acute pancreatitis: clinical and imaging characteristics and outcomes in a cohort of 31 cases.

    Science.gov (United States)

    Pelaez-Luna, Mario; Vege, Santhi Swaroop; Petersen, Bret T; Chari, Suresh T; Clain, Jonathan E; Levy, Michael J; Pearson, Randal K; Topazian, Mark D; Farnell, Michael B; Kendrick, Michael L; Baron, Todd H

    2008-07-01

    Information regarding the natural history, clinical characteristics, and outcomes of disconnected pancreatic duct syndrome (DPDS) is limited. To describe clinical characteristics and outcomes of DPDS. A retrospective review of the Mayo Clinic endoscopy and hospital service database. Tertiary-referral center. We identified 31 DPDS cases from 1999 to 2006. Endoscopic drainage of pancreatic-fluid collections. The relationship between demographic and clinical data with endoscopic treatment and clinical outcomes in DPDS cases. The median patient age was 53 years (range 20-83 years); 48% were men. The most common etiology of acute pancreatitis (AP) was biliary (55%) followed by idiopathic (27%). The median interval between the diagnoses of AP and DPDS was 56 days (range 3-251 days); the median follow-up after the last ERCP or surgical procedure was 7 months (range 0-90 months). The DPDS location included the following: pancreas head 6%, neck 58%, body 26%, and tail 10%. Twenty-six patients had initial endoscopic treatment (19 had long-term improvement; 7 failed treatment and required surgery) and 5 underwent immediate surgery. Mortality was 0%; 26% developed chronic pancreatitis (CP) and 16% diabetes mellitus (DM); 10% resolved completely, 45% had smaller fluid collections, and 26% patients were lost to follow-up. No relationship between demographic and clinical data with endoscopic and clinical outcomes was found. Endoscopic treatment temporarily improved DPDS, with a failure rate of 23%. Immediate surgery was not required in all cases. CP and/or pancreatic atrophy occurred relatively shortly after the DPDS diagnosis in 26% and DM in 16% of cases. DPDS did not lead to mortality. Early surgery may be considered after initially stabilizing the fluid collection with endoscopic therapy.

  4. Fibrosis quística que simula un síndrome de Bartter Cystic fibrosis mimicking Bartter syndrome

    Directory of Open Access Journals (Sweden)

    Neri G Campañá Cobas

    2008-12-01

    Full Text Available La fibrosis quística es una enfermedad que se hereda como trastorno autosómico recesivo. La presentación clásica está caracterizada por enfermedad pulmonar crónica, deficiencia pancreática y concentraciones altas de electrolitos en sudor. En algunos pacientes la presentación puede ser monosíntomatica, por ejemplo, la depleción de electrolitos en sangre. El propósito de este informe es comunicar el caso de una lactante de 2 meses de edad diagnosticada de fibrosis quística, que inicialmente pareció ser un síndrome de Bartter. El motivo de ingreso fue un vómito, decaimiento y signos de deshidratación. Se realizó gasometría, estudio de electrolitos en sangre, determinación de concentración de electrolitos en la orina, prueba de electrolitos en sudor y estudio genético para fibrosis quística. La concentración de potasio (28 mEeq/L hizo pensar en un síndrome de Bartter y se comenzó tratamiento con indometacina y cloruro de potasio; se normalizaron todos los parámetros. Dos meses después reingresó con deshidratación ligera por un vómito, trastornos mixtos del equilibrio ácido-base, hiponatremia, hipocloremia y ligera hiperpotasemia. Se realizaron electrolitos en sudor en 3 ocasiones y fueron positivos, y el estudio genético para fibrosis quística demostró una mutación delta F508.Cystic fibrosis is a disease that is inherited as a recessive autosomal disorder. The classical presentation is characterized by chronic lung disease, pancreatic deficiency and high concentrations of electrolytes in sweat. In some patients, the presentation may be monosymptomatic as, for example, the depletion of electrolytes in blood. The objective of this paper is to report the case of a 2-months-old female infant with diagnosis of cystic fibrosis that initially seemed to be a Bartter syndrome. The reason to be admitted was vomit, dwindles and dehydration signs. Gasometry, study of electrolytes in blood, determination of concentration of

  5. Patent arterial duct

    Directory of Open Access Journals (Sweden)

    Martin Robin P

    2009-07-01

    Full Text Available Abstract Patent arterial duct (PAD is a congenital heart abnormality defined as persistent patency in term infants older than three months. Isolated PAD is found in around 1 in 2000 full term infants. A higher prevalence is found in preterm infants, especially those with low birth weight. The female to male ratio is 2:1. Most patients are asymptomatic when the duct is small. With a moderate-to-large duct, a characteristic continuous heart murmur (loudest in the left upper chest or infraclavicular area is typical. The precordium may be hyperactive and peripheral pulses are bounding with a wide pulse pressure. Tachycardia, exertional dyspnoea, laboured breathing, fatigue or poor growth are common. Large shunts may lead to failure to thrive, recurrent infection of the upper respiratory tract and congestive heart failure. In the majority of cases of PAD there is no identifiable cause. Persistence of the duct is associated with chromosomal aberrations, asphyxia at birth, birth at high altitude and congenital rubella. Occasional cases are associated with specific genetic defects (trisomy 21 and 18, and the Rubinstein-Taybi and CHARGE syndromes. Familial occurrence of PAD is uncommon and the usual mechanism of inheritance is considered to be polygenic with a recurrence risk of 3%. Rare families with isolated PAD have been described in which the mode of inheritance appears to be dominant or recessive. Familial incidence of PAD has also been linked to Char syndrome, familial thoracic aortic aneurysm/dissection associated with patent arterial duct, and familial patent arterial duct and bicuspid aortic valve associated with hand abnormalities. Diagnosis is based on clinical examination and confirmed with transthoracic echocardiography. Assessment of ductal blood flow can be made using colour flow mapping and pulsed wave Doppler. Antenatal diagnosis is not possible, as PAD is a normal structure during antenatal life. Conditions with signs and symptoms of

  6. Comparison of Unstimulated In Vitro Maturation and Stimulated: In Vitro Fertilization in Women with Poly Cystic Ovarian Syndrome

    Directory of Open Access Journals (Sweden)

    Z Khashavi

    2004-12-01

    Full Text Available Background: In regard to complications of controlled ovarian hyperstimulation (COH such as multiple pregnancy and ovarian hyper stimulation syndrome (OHSS and other short and long term complications, the interest to In Vitro Maturation (IVM of oocytes from unstimulated ovaries is increasing. This clinical trial study was designed to establish the relative success of treatment with IVM in comparison with In Vitro Fertilization (IVF in Poly Cystic Ovarian Syndrome (PCOS women. Methods: This prospective clinical trial included 35 IVM and 35 IVF cycles matched for age, and duration and cause of infertility. IVM patients underwent transvaginal retrieve of immature oocytes during unstimulated cycles, IVM and ICSI. Those in IVF group underwent ovarian stimulation after pituitary supression. Main outcome measures included number of retrieved oocytes, number of produced embryos, and pregnancy rate and complications. Results: In the IVM group, 1.39 mature oocytes, 0.27 fertilized oocyte, and 0.24 embryos were obtained per retrieval, and in IVF group, 8.45 mature oocytes, 3.8 fertilized oocytes, and 2.7 embryos were obtained. Conclusion: This significant differences between two groups (P~ 0 indicate that this method maybe used as a research setting until further study. Keywords: In Vitro Maturation, ART, unstimulated IVM, PCOS.

  7. Primary mediastinal synovial sarcoma with subsequent development of primary adenoid cystic carcinoma of lung presenting as superior vena cava syndrome.

    Science.gov (United States)

    Madabhavi, Irappa; Patel, Apurva; Anand, Asha; Panchal, Harsha; Parikh, Sonia

    2018-01-01

    Primary mediastinal sarcomas are aggressive tumors with a very rare incidence. This report describes the case of a 35 year old male patient who presented with acute symptoms of dyspnoea, facial puffiness, engorged neck veins and hoarseness of voice. With the clinical picture consistent with the superior vena caval (SVC) syndrome, the patient was investigated with computed tomography of the chest. This revealed a large soft tissue density mass lesion compressing the SVC along with other critical superior mediastinal structures. Histopathological evaluation of the mass revealed features consistent with a soft tissue sarcoma and positive staining was observed for vimentin and S-100. Cytogenetic analysis by fluorescent in-situ hybridization (FISH) demonstrated the t(X: 18) translocation. Thus diagnosis was established as primary mediastinal synovial sarcoma. Patient was treated with three-cycles of neo-adjuvant (ifosfamide 2400mg/m2 on days 1-5 and doxorubicin 37.5 mg/m2 on days 1 & 2) chemotherapy, to which there was a partial response as per the RECIST criteria. Surgical excision of the mediastinal mass was performed, and further post-operative treatment with adjuvant chemo-radiotherapy was provided. Patient was under regular surveillance at our clinic and remains free of symptoms one-year after treatment completion. But after 14 months of treatment completion patient again had symptoms of progressive dyspnea, hoareness of voice and mild facial puffiness over a period of 2 months. On further investigating he was found to have right-sided centrally located mass with cystic and necrotic changes with extension and compression of trachea, SVC, right upper lobe bronchus and its branches. Histopathological examination of the biopsy from the lesion revealed adenoid cystic carcinoma of the lung. Rest of the metastatic work up was within normal. Immunohistochemistry of the specimen revealed c-Kit positivity. In view of the morbid second surgery he was put on Imatinib 400mg

  8. Cystic lung disease in birt-hogg-dubé syndrome: a case series of three patients.

    Science.gov (United States)

    Kilincer, Abidin; Ariyurek, Orhan Macit; Karabulut, Nevzat

    2014-06-01

    Birt-Hogg-Dubé syndrome is characterized by clinical manifestations such as hamartomas of the skin, renal tumors and lung cysts with spontaneous pneumothoraces. Patients with Birt-Hogg-Dubé syndrome may present with only multiple lung cysts. We report the chest computerized tomography (CT) features of three patients with Birt-Hogg-Dubé syndrome. Each patient had multiple lung cysts of various sizes according to chest CT evaluation, most of which were located in lower lobes and related to pleura. The identification of unique characteristics in the chest CT of patients with Birt-Hogg-Dubé syndrome may provide an efficient mechanism for diagnosis.

  9. MR findings in thyroglossal duct cysts

    International Nuclear Information System (INIS)

    Blandino, A.; Salvi, L.; Chirico, G.; Scribano, E.; Longo, M.; Pandolfo, I.

    1990-01-01

    Two patients with thyroglossal duct cysts have been studied with CT and MR. The typical CT feature of these cystic upper-neck lesions are depicted in literature, conversely MR findings are not well known. The homogeneous high intensity on T1-weighted images, higher than simple cyst or fluid, is the most typical feature of the thyroglossal cyst. (author). 12 refs.; 5 figs

  10. [Cervical thoracic duct cyst: An uncommon entity].

    Science.gov (United States)

    Carreira-Delgado, Manuel; Fernández-Rodríguez, Elvira; Martínez-Míguez, Marta; Álvarez-Martín, María Jesús; Nuño Vázquez-Garza, José Manuel

    2017-12-01

    Cervical thoracic duct cysts are a rare anomaly. To report a case of cervical thoracic duct cyst, and perform a literature review. A 78-year-old female, with a one-year history of a left-sided asymptomatic supraclavicular cystic mass. Computerized tomography revealed a cystic mass 42mm in diameter. We performed a fine needle aspiration puncture, obtaining a thick, milky, whitish liquid. The patient underwent surgery; finding a left-sided supraclavicular cystic mass, with some lymph vessels heading towards the jugulo subclavian venous junction. We performed a ligation of these lymph vessels and resection of the mass. The histopathologic study confirmed the diagnosis of thoracic duct cyst. Diagnosis of cervical thoracic duct cyst should be suspected with a cystic lesion in the left supraclavicular region, which when perforated exudes a very distinctive thick milky, whitish liquid with a high content of lymphocytes and triglycerides. Treatment should be complete removal with ligation of the lymphatic afferent vessels. Copyright © 2016 Academia Mexicana de Cirugía A.C. Publicado por Masson Doyma México S.A. All rights reserved.

  11. Cystic medulloblastoma

    International Nuclear Information System (INIS)

    Mahapatra, A.K.; Paul, H.K.; Sarkar, C.

    1989-01-01

    In children medulloblastoma is a commonly encountered posterior fossa midline tumour in which cystic degeneration is not uncommon. A cystic medulloblastoma without solid component has, however, not been described. We report a 12-year-old boy with a posterior fossa midline cystic lesion on CT with surgical and histological confirmation of the diagnosis. (orig.)

  12. Pseudo-Bartter’s syndrome in patients with cystic fibrosis: A case series and review of the literature

    Directory of Open Access Journals (Sweden)

    Vilotijević-Dautović Gordana

    2015-01-01

    Full Text Available Introduction. Pseudo-Bartter syndrome (PBS is characterized by hyponatremic, hypochloremic metabolic alkalosis that mimics Bartter syndrome but with no pathology in the renal tubules. We present five patients with cystic fibrosis (CF and PBS. Cases Outline. Four children aged between three and five-and-one-half months with previously diagnosed CF and one aged 17 months with previously undiagnosed disease, were hospitalized during the summer season, with severe dehydration, oliguria, apathy and adynamia. Additionally, one of them had an ileostomy due to meconium ileus after birth. All children were on a diet without additional salt intake. Laboratory analysis on admission showed hyponatremia (115-133 mmol/L, mean 122.4 mmol/L, high plasma renin activity (229-500 pg/ml, mean 324 pg/ml and metabolic alkalosis (pH 7.5-7.6, mean 7.56 in all the patients, and in four of them high blood level of aldosterone (74-560 pg/ml, mean 295.9 pg/ml, hypokalemia (2.3-2.8 mmol/L, mean 2.6 mmol/L, hypochloremia (59-71 mmol/L, mean 66 mmol/L and low urinary sodium (5-12 mmol/L, mean 9 mmol/L. After intravenous rehydration followed by additional use of sodium and chloride in mean dosis of 1.78 mmol/kg per day, all the patients made a complete recovery. With advice for additional use of salt in the mentioned amount, the patients were discharged from the hospital. Conclusion. PBS is one of CF complications, especially in infants and young children in situations accompanied by increased sweating and/or other causes of additional loss of sodium and chlorine. Sometimes, as was the case with one of our patients, PBS may be the initial presentation form of the disease.

  13. A rare cause of cystic lung disease - Birt-Hogg-Dubé syndrome.

    Science.gov (United States)

    Minnis, P; Riddell, P; Keane, M P

    2016-01-01

    Birt-Hogg-Dubé syndrome, initially described in 1977, is an autosomal dominant inherited condition characterised by basal pulmonary cysts often resulting in pneumothorax, renal tumours and cutaneous involvement. Lung cysts have been described in up to 90% of patients with a corresponding risk of pneumothorax of 50 times greater than the normal population. We describe here a case of Birt-Hogg-Dubé diagnosed in the 9th decade of life and discuss the radiological findings and clinical implications.

  14. Cushing's syndrome due to pharmacological interaction in a cystic fibrosis patient

    DEFF Research Database (Denmark)

    Main, K M; Skov, M; Sillesen, Ida Blok

    2002-01-01

    and 1,600 microg budesonide. The patient experienced symptoms of striae, moon-face, increased facial hair growth, mood swings, headaches, weight gain, irregular menstruation despite oral contraceptives and increasing insulin requirement for diabetes mellitus. Endocrine investigations revealed total...... and budesonide inhalation should be monitored regularly for adrenal insufficiency. This may be the first indicator of increased systemic exogenous steroid concentration, before clinical signs of Cushing's syndrome emerge....

  15. Molecular Mechanisms of Bile Duct Development

    OpenAIRE

    Zong, Yiwei; Stanger, Ben Z.

    2010-01-01

    The mammalian biliary system, consisting of the intrahepatic and extrahepatic bile ducts, is responsible for transporting bile from the liver to the intestine. Bile duct dysfunction, as is seen in some congenital biliary diseases such as Alagille syndrome and biliary atresia, can lead to the accumulation of bile in the liver, preventing the excretion of detoxification products and ultimately leading to liver damage. Bile duct formation requires coordinated cell-cell interactions, resulting in...

  16. Invasive Cystic Hypersecretory Carcinoma of the Breast: A Case Report

    OpenAIRE

    Lee, Ji Shin; Lee, Young Jik

    2004-01-01

    Cystic hypersecretory lesions of the breast are rare. These breast lesions include cystic hypersecretory hyperplasia (CHH), atypical CHH, and cystic hypersecretory carcinoma (CHC). The characteristic features are dilated ducts and cysts filled with thyroid colloid-like eosinophilic secretion. Only seven cases of invasive CHC have been reported in the literature. Here, we report an additional case of invasive CHC. The histologic features of the tumor showed both micropapillary intraductal carc...

  17. Radiologic features of cystic, endocrine and other pancreatic neoplasms

    International Nuclear Information System (INIS)

    Balci, N. Cem; Semelka, Richard C.

    2001-01-01

    This article presents imaging features of cystic, endocrine and other pancreatic neoplasms. Microcystic adenoma which is composed of small cysts ( 2 cm) are accounted for mucinous cystic neoplasms, its variant along pancreatic duct is ductectatic mucinous cystic neoplasm. Endocrine tumors of pancreas are hypervascular and can be depicted on early dynamic enhanced crosssectional imaging modalities or on angiography when they are <1 cm. Pancreatic metastases and lymphomas are rare neoplasms which should also be included in differential diagnosis for pancreatic masses

  18. Congenital cystic disease of the liver in seven dogs

    NARCIS (Netherlands)

    Ingh, T.S.G.A.M. van den; Rothuizen, J.

    1985-01-01

    Seven canine cases of cystic disease of the liver are described. They included 3 cases with solitary cysts, 3 with the adult type of polycystic disease of the liver and one with congenital dilatation of the bile ducts type V, i.e. fusiform dilatation of the intrahepatic and extrahepatic bile ducts.

  19. [Mirizzi syndrome--a contraindication for laparoscopic surgery].

    Science.gov (United States)

    Moser, J J; Baer, H U; Glättli, A; Schweizer, W; Blumgart, L H; Czerniak, A

    1993-03-01

    The Mirizzi syndrome refers to a benign obstruction of the common hepatic duct by a stone impacted within the cystic duct or the neck of the gallbladder causing an obstructive jaundice. According to McSherry's subclassification based on endoscopic retrograde cholangio-pancreatography we distinguish a type I, involving an external compression of the common hepatic duct by a large stone impacted in the cystic duct or Hartmann's pouch without lesion of the gallbladder or the common hepatic duct wall. In type II a cholecysto-choledochal fistula is present, caused by a calculous which has already eroded partly or completely into the common bile duct. The type I is better treated with a partial cholecystectomy, the type II with a partial cholecystectomy and a bilio-digestive anastomosis. Due to a severe inflammatory process with dense adhesions and odematous changes of the hepato-duodenal ligament, it is dangerous to prepare the Calot's triangle, in case of conventional cholecystectomy and/or in case of laparoscopic cholecystectomy. From 1986-1991 we have successfully treated 10 patients according to this concept (7 patients with type II, 3 patients with type I). We have no mortality and no lesion of the biliary system. One patient has been reoperated because of a postoperative hemorrhage and biliary fistula. One patient developed a benign stricture which could be dilated two months after the procedure. Two patients were found with an incidental carcinoma of the gallbladder.

  20. Distinguishing the histological and radiological features of cystic lung disease in Birt-Hogg-Dubé syndrome from those of tobacco-related spontaneous pneumothorax.

    Science.gov (United States)

    Fabre, Aurelie; Borie, Raphael; Debray, Marie Pierre; Crestani, Bruno; Danel, Claire

    2014-04-01

    Birt-Hogg-Dubé syndrome (BHD) is a rare autosomal dominantly inherited genodermatosis that predisposes to cystic lung disease, leading to spontaneous pneumothoraces. This retrospective analysis of five BHD cases (two men, three women) compared lung histology and computed tomography (CT) imaging to a matched cohort of non-BHD patients with spontaneous pneumothoraces (SPN). Lung was sampled during pleurodesis to resect bullae. Recurrent pneumothoraces was seen in two patients. Fourteen sets of histological slides (seven in each group) and 10 CT scans (five in each group) were reviewed. CT scans in BHD showed multiple cysts with a basal predominance and intraparenchymal/peribronchial distribution. On histological examination, BHD lungs showed punch-out cysts with no inflammation, and lacked subpleural fibroelastotic scars and smoking changes. In contrast, all SPN cases showed respiratory bronchiolitis and subpleural fibroelastotic scars. This study emphasizes the importance of smoking history and topography of the lesions in assessing cystic lung disease. Pathologists need to remain alert to the possibility of BHD in the setting of recurrent pneumothoraces in a non-smoker, in particular in a woman, at any age, and should take part in a multidisciplinary approach to the diagnosis of cystic lung disease to obtain clinical and CT scan details. © 2013 John Wiley & Sons Ltd.

  1. Retrovesical cystic lesions in female patients with unilateral renal agenesis or dysplasia

    International Nuclear Information System (INIS)

    Shimada, Kenji; Matsumoto, Fumi; Matsui, Futosi; Obara, Takasi

    2010-01-01

    The objective of this study was to review our experience with female children who have unilateral renal agenesis or renal dysplasia associated with cystic lesions in the reproductive system. Between 1991 and 2007, we treated 26 patients with unilateral renal agenesis or renal dysplasia associated with pelvic, retrovesical or interlabial cystic lesions. In 16 patients, an abnormality either of the kidney or a cystic lesion was detected during the perinatal period. Another 10 patients presented clinical symptoms, including urinary incontinence in five, urinary tract infection in one, and vaginal discharge in four. Based on clinical features and imaging findings, the patients were divided into four groups: those with Gartner's duct cyst (Group 1, n=9); those with uterovaginal duplication with obstructed hemivagina (Herlyn-Werner-Wunderlich syndrome [HWW]; Group 2, n=3); those with both Gartner's duct cyst and HWW syndrome (Group 3, n=3); and those without definitive diagnosis (Group 4, n=11). In Group 1, leakage completely stopped after nephrectomy in three patients, whereas six patients continued to be incontinent despite the removal of dysgenetic kidneys. In Group 2, after the excision of a vaginal septum, no patient presented urinary incontinence. In patients in Group 3, both the mesonephric remnant and mullerian structures were confirmed on imaging or through endoscopy. Because of the high coincidence of genital and renal anomalies, it is recommended that genital systems in female patients with renal anomalies associated with cystic lesions behind the lower urinary tract be investigated. The best imaging modality to use remains under scrutiny. It is important to follow the patients until the age of puberty. The importance of a long-term follow-up in these patients needs to be emphasized. (author)

  2. Multiple cystic lung disease

    Directory of Open Access Journals (Sweden)

    Flavia Angélica Ferreira Francisco

    2015-12-01

    Full Text Available Multiple cystic lung disease represents a diverse group of uncommon disorders that can present a diagnostic challenge due to the increasing number of diseases associated with this presentation. High-resolution computed tomography of the chest helps to define the morphological aspects and distribution of lung cysts, as well as associated findings. The combination of appearance upon imaging and clinical features, together with extrapulmonary manifestations, when present, permits confident and accurate diagnosis of the majority of these diseases without recourse to open-lung biopsy. The main diseases in this group that are discussed in this review are lymphangioleiomyomatosis, pulmonary Langerhans cell histiocytosis and folliculin gene-associated syndrome (Birt–Hogg–Dubé; other rare causes of cystic lung disease, including cystic metastasis of sarcoma, are also discussed. Disease progression is unpredictable, and understanding of the complications of cystic lung disease and their appearance during evolution of the disease are essential for management. Correlation of disease evolution and clinical context with chest imaging findings provides important clues for defining the underlying nature of cystic lung disease, and guides diagnostic evaluation and management.

  3. Cystic Fibrosis

    Science.gov (United States)

    ... fixing the CFTR protein. Learn more Living with Cystic Fibrosis A diagnosis of CF is life changing for a family. However, in the last 2 to 3 decades, significant strides have been made so that children born ... Doctor about Cystic Fibrosis Making notes before your visit, as well as ...

  4. Prevalence of the AMHR2 mutation in Miniature Schnauzers and genetic investigation of a Belgian Malinois with persistent Müllerian duct syndrome.

    Science.gov (United States)

    Smit, M M; Ekenstedt, K J; Minor, K M; Lim, C K; Leegwater, Paj; Furrow, E

    2018-04-01

    Persistent Müllerian duct syndrome (PMDS) is a sex-limited disorder in which males develop portions of the female reproductive tract. Important consequences of PMDS are cryptorchidism and its sequelae of infertility and increased risk of testicular cancer. Anti-Müllerian hormone (AMH) and its receptor (AMHR2) induce the regression of the Müllerian ducts in male embryos. In Miniature Schnauzer dogs, the genetic basis has been identified as an autosomal recessive nonsense mutation in AMHR2, but the allele frequency of the mutation is unknown. Thus, the primary objective of this study was to estimate the prevalence of the AMHR2 mutation in North American Miniature Schnauzers, in order to ascertain the value of genetic testing in this breed. An additional objective was to determine whether mutations in AMH or AMHR2 were responsible for PMDS in a Belgian Malinois; this would aid development of a genetic test for the Belgian Malinois breed. Genomic DNA from 216 Miniature Schnauzers (including one known PMDS case) was genotyped for the AMHR2 mutation, and DNA from a single PMDS-affected Belgian Malinois was sequenced for all coding exons of AMH and AMHR2. The Miniature Schnauzer cohort had an AMHR2 mutation allele frequency of 0.16 and a carrier genotypic frequency of 0.27. The genetic basis for PMDS in the Belgian Malinois was not determined, as no coding or splicing mutations were identified in either AMH or AMHR2. These findings support a benefit to AMHR2 mutation testing Miniature Schnauzers used for breeding or with cryptorchidism. © 2017 Blackwell Verlag GmbH.

  5. The Effect of Home based Exercise on Treatment of Women with Poly Cystic Ovary Syndrome; a single-Blind Randomized Controlled Trial

    Directory of Open Access Journals (Sweden)

    Farzaneh Vasheghani-Farahani

    2017-01-01

    Full Text Available Background: The most common reproductive endocrine disorder of reproductive age women is a Poly cystic ovary syndrome (PCOS Metabolic syndrome has been more reported in patients with PCOS in comparison to general population. Few investigations have been performed to evaluate the independent effect of exercise on biochemical and clinical symptoms of patients with PCOS. The aim of the study was to find the effect of home base aerobic-strengthening exercises on anthropometric and hormonal variables of patients with PCOS.MaterialsandMethods:In this randomized controlled trial twenty women in the exercise group performed aerobic, strengthening exercises; the other 20 participants in the control group were advised to continue their previous physical activity pattern. Blood pressure, Waist to Hip ratio (WHR, BMI along with hormonal variables(including insulin related factors, sexual hormones and inflammatory factors were assessed at baselineand after the 12 week intervention.Results:16patients in the exercise group and 14 patients in control group finished the study. TheWHR (p<0.001 along with the blood level of insulin (p=0.016, FBS (p=0.044, Prolactine (p=0.022 and hsCRP (p=0.035 and HOMA index (p=0.009 were decreased significantly in the exercise group compared with the control group. No significant differences were found in lipid profile and sexual hormones between groups at the end of the study.Conclusion:We can conclude that 12 weeks combined aerobic-strengthening exercise program in women with poly cystic ovary syndrome can lead to a reduction of waist to hip ratio (WHR and some cardiovascular risk factors (including insulin, FBS, HOMA index and HsCRP along with an increase of prolactine level in these patients.

  6. Learning about Cystic Fibrosis

    Science.gov (United States)

    ... What do we know about heredity and cystic fibrosis? Cystic fibrosis (CF) is the most common, fatal genetic ... FAQ Top of page Additional Resources for Cystic Fibrosis Information Cystic Fibrosis [ghr.nlm.nih.gov] Summary FAQ from ...

  7. Pseudo-Bartter syndrome as the sole manifestation of cystic fibrosis in a child with 711+G>T/IVS8-5T mutation: a new face of an old disease.

    Science.gov (United States)

    Tinsa, Faten; Hadj Fredj, Sondes; Bel Hadj, Imen; Khalsi, Fatma; Abdelhak, Sonia; Boussetta, Khadija; Messaoud, Taieb

    2017-08-01

    Pseudo-Bartter syndrome (PBS) describes an uncommon complication of cystic fibrosis leading to hypochloraemic, hypokalaemic metabolic alkalosis. PBS as the sole manifestation of cystic fibrosis in children is extremely rare and has never been described in patients carrying 5T variant. We report a clinical, biochemical and genetic study of a four year-old boy presenting a pseudo-Bartter syndrome as the sole manifestation of cystic fibrosis. All 27 exons and the flanking intron regions of the CFTR gene were analysed by PCR and direct sequencing. Direct sequencing was also used to analyse TG m T n and M470V polymorphisms in the patient and his parents. Two sweat tests were abnormal with elevated chloride levels at 78 and 88 mmol/L. DNA sequencing revealed a heterozygous mutation 711+1 G>T and an IVS8-T5 allele. The mutation 711+1 G>T is in trans with the IVS8-T5-TG11 allele and the child carried M470/V470 genotype. To the best of our knowledge, the genotype 711+1 G>T /IVS8-5T found in our patient is described for the first time. The role of TG11-5T-V470 allele in cases of cystic fibrosis with PB syndrome remains to be determined.

  8. The Mirizzi syndrome: an uncommon cause of obstructive jaundice

    International Nuclear Information System (INIS)

    Marco, S.F.; Piqueras, R.M.; Jornet, J.; Gil, S.; Ambit, S.; Cervera, J.

    1997-01-01

    The Mirizzi syndromes involves obstruction of the common hepatic duct by a gallstone impacted in the bladder neck or in the cystic duct. It is accompanied by and inflammatory reaction that usually produces a biliary fistula. On rare occasions, this disorder can lead to obstructive jaundice. We reviewed nine cases of Mirizzi syndrome, comparing the findings according to different imaging techniques. All the patients underwent ultrasound study, three were assessed by computerized tomography (CT) and three by transhepatic cholangiography (THC). In eight patients, the diagnosis was confirmed intraoperatively: the remaining patient was diagnosed on the basis of the clinical evidence of obstructive jaundice, ultrasound and THC. In eight patients, ultrasound disclosed dilation of the intrahepatic bile duct. Scleroatrophic bladder with a large stone in the infundibular zone was observed in five cases, and numerous small calculi were found in the remaining four patients, two of whom presented thickening of the bladder walls: the walls were normal in the other two. Pneumobilia was observed one case. CT revealed dilation of the intrahepatic bile duct in all three cases in which it was performed. A cavity containing a calculus was observed in one case and pneumobilia in another. THC disclosed dilation of the intrahepatic bile duct and a lateral filling defect in the common hepatic duct in all three patients in whom it was performed. Two patients were found to have cholecystocholedochal fistulas. The Mirizzi syndrome should be suspected when a scleroatrophic gallbladder or gallstones compress the common hepatic duct, causing proximal, but not distal, bile duct dilation. In these case, THC should be carried out to confirm the diagnosis and determine whether or not a fistula is present since the surgical treatment varies depending on the existence of fistulas and their location. (Author) 15 refs

  9. The cystic fibrosis of exocrine pancreas

    DEFF Research Database (Denmark)

    Wilschanski, Michael; Novak, Ivana

    2013-01-01

    The cystic fibrosis transmembrane conductance regulator (CFTR) protein is highly expressed in the pancreatic duct epithelia and permits anions and water to enter the ductal lumen. This results in an increased volume of alkaline fluid allowing the highly concentrated proteins secreted by the acinar...... cells to remain in a soluble state. This work will expound on the pathophysiology and pathology caused by the malfunctioning CFTR protein with special reference to ion transport and acid-base abnormalities both in humans and animal models. We will also discuss the relationship between cystic fibrosis...

  10. Mucinous cystic neoplasm of the pancreas in a male patient

    Directory of Open Access Journals (Sweden)

    Kazuhiro Yoshida

    2011-04-01

    Full Text Available Mucinous cystic neoplasms (MCNs make up a morphologic family of similar appearing tumors arising in the ovary and various extraovarian organs such as pancreas, hepatobiliary tract and mesentery. MCNs of the pancreas occur almost exclusively in women. Here, we report a rare case of MCN in a male patient. A 39-year-old man was admitted to our hospital with the chief complaint of back pain. Abdominal computed tomography revealed a multilocular cyctic mass 6.3 cm in diameter in the pancreatic tail. In addition, the outer wall and septae with calcification were demonstrated in the cystic lesion. On magnetic resonance imaging , the cystic fluid had low intensity on T1-weighted imaging and high intensity on T2-weighted imaging. Endoscopic retrograde cholangio-pancreatography (ERCP showed neither communication between the cystic lesion and the main pancreatic duct nor encasement of the main pancreatic duct. Endoscopic ultrasonography revealed neither solid component nor thickness of the septae in the cystic lesion. Consequently, we performed distal pancreatectomy with splenectomy under the diagnosis of cystic neoplasia of the pancreas. Histopathologically, the cystic lesion showed two distinct component: an inner epithelial layer and an outer densely cellular ovarian-type stromal layer. Based on these findings, the cystic lesion was diagnosed as MCN.

  11. Postoperative follow-up studies in congenital dilatation of the common bile duct using hepatobiliary scintigraphy

    International Nuclear Information System (INIS)

    Hirobe, Seiichi; Ishida, Haruo; Hayashi, Akira; Kamagata, Shoichiro; Fuchimoto, Yasufumi; Mizuno, Dai; Yano, Tsunehiro; Ishii, Katsumi.

    1995-01-01

    Follow-up studies in congenital dilatation of the common bile duct were done in 20 cases ranging 3 to 20 years after operation. Operative cholangiography had shown dilatation of the intrahepatic bile ducts in 15 patients (75%), of these, 7 patients had cystic dilatation. Five cases of these 7 patients showed prolonged stasis of the nuclide in the cystic dilated intrahepatic bile ducts by 99m Tc-PMT hepatobiliary scintigraphy in the follow-up studies even at 10 years after operation. But none of 20 cases had complication such as intrahepatic lithiasis, cholangitis, and liver dysfunction. Hepatobiliary scintigraphy could provide moderate anatomic and excellent functional information which were useful for these follow-up studies and careful follow-up should be done in the case of cystic dilatation of the intrahepatic bile ducts. (author)

  12. Postoperative follow-up studies in congenital dilatation of the common bile duct using hepatobiliary scintigraphy

    Energy Technology Data Exchange (ETDEWEB)

    Hirobe, Seiichi; Ishida, Haruo; Hayashi, Akira; Kamagata, Shoichiro; Fuchimoto, Yasufumi; Mizuno, Dai; Yano, Tsunehiro [Tokyo Metropolitan Kiyose Children`s Hospital (Japan); Ishii, Katsumi

    1995-03-01

    Follow-up studies in congenital dilatation of the common bile duct were done in 20 cases ranging 3 to 20 years after operation. Operative cholangiography had shown dilatation of the intrahepatic bile ducts in 15 patients (75%), of these, 7 patients had cystic dilatation. Five cases of these 7 patients showed prolonged stasis of the nuclide in the cystic dilated intrahepatic bile ducts by {sup 99m}Tc-PMT hepatobiliary scintigraphy in the follow-up studies even at 10 years after operation. But none of 20 cases had complication such as intrahepatic lithiasis, cholangitis, and liver dysfunction. Hepatobiliary scintigraphy could provide moderate anatomic and excellent functional information which were useful for these follow-up studies and careful follow-up should be done in the case of cystic dilatation of the intrahepatic bile ducts. (author).

  13. Cystic Fibrosis Chest X-Ray Findings: A Teaching Analog

    Science.gov (United States)

    2008-07-01

    Congenital Cystic Fibrosis Kartagener’s Syndrome Alpha-1 Antitrypsin Deficiency Bronchomalacia Yellow- Nail Syndrome Severe Inflammation...Mycobacterium (i.e. Tuberculosis) MRSA TORCHES Fungal Infection Obstructive Foreign Body Aspiration Bronchial Stricture Airway Mass/Tumor

  14. CT findings of thyroglossal duct cyst

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Dong Oh; Kim, Hong Soo; So, Hyun Soon; Nam, Mee Young; Choi, Jae Ho; Rhee, Hak Song [Presbyterian Medical Center, Jeonju (Korea, Republic of)

    1995-05-15

    The purpose of this study was to evaluate the CT findings of thyroglossal duct cysts. Sixteen patients with pathologically proved thyroglossal duct cysts were included in the study. CT scans were assessed retrospectively for shape, size, location, density of the central portions, septations, rim enhancement, changes in the adjacent fascial planes and investment within the strap muscles in the infrahyoid cysts. Thirteen cases of thyroglossal duct cysts were seen as round or oval cystic masses, two cases of them were seen as irregular-shaped lobulated cystic masses, and one case was seen as ovoid soft tissue mass. The cysts were from 1.4 to 5.7 cm in diameter (mean, 2.6 cm). The cyst was infrahyoid in 15 cases and suprahyoid in one case. The cyst was located in midline in eight cases, off midline in four cases, and both midline and off midline in four cases. The density of the central portions ranged from 15 to 82HU (mean, 32HU). Septations were noted in four cases. Rim enhancement was seen in 14 cases (93%), and heterogeneously enhancing soft tissue mass was seen in one case. In four cases, abnormal fascial planes were observed. All but one of the infrahyoid cysts (14/15) were embedded within the strap muscles, and one case of them was located anteriorly to strap muscles. CT permits one to make the diagnosis a thyroglossal duct cyst with a high degree of accuracy, as it can differentiate thyroglossal duct cysts from the other anterior neck masses by their typical location, characteristic morphology, and investment within the strap muscles.

  15. CT findings of thyroglossal duct cyst

    International Nuclear Information System (INIS)

    Kim, Dong Oh; Kim, Hong Soo; So, Hyun Soon; Nam, Mee Young; Choi, Jae Ho; Rhee, Hak Song

    1995-01-01

    The purpose of this study was to evaluate the CT findings of thyroglossal duct cysts. Sixteen patients with pathologically proved thyroglossal duct cysts were included in the study. CT scans were assessed retrospectively for shape, size, location, density of the central portions, septations, rim enhancement, changes in the adjacent fascial planes and investment within the strap muscles in the infrahyoid cysts. Thirteen cases of thyroglossal duct cysts were seen as round or oval cystic masses, two cases of them were seen as irregular-shaped lobulated cystic masses, and one case was seen as ovoid soft tissue mass. The cysts were from 1.4 to 5.7 cm in diameter (mean, 2.6 cm). The cyst was infrahyoid in 15 cases and suprahyoid in one case. The cyst was located in midline in eight cases, off midline in four cases, and both midline and off midline in four cases. The density of the central portions ranged from 15 to 82HU (mean, 32HU). Septations were noted in four cases. Rim enhancement was seen in 14 cases (93%), and heterogeneously enhancing soft tissue mass was seen in one case. In four cases, abnormal fascial planes were observed. All but one of the infrahyoid cysts (14/15) were embedded within the strap muscles, and one case of them was located anteriorly to strap muscles. CT permits one to make the diagnosis a thyroglossal duct cyst with a high degree of accuracy, as it can differentiate thyroglossal duct cysts from the other anterior neck masses by their typical location, characteristic morphology, and investment within the strap muscles

  16. Reproductive genetic carrier screening for cystic fibrosis, fragile X syndrome, and spinal muscular atrophy in Australia: outcomes of 12,000 tests.

    Science.gov (United States)

    Archibald, Alison Dalton; Smith, Melanie Jane; Burgess, Trent; Scarff, Katrina Louise; Elliott, Justine; Hunt, Clare Elizabeth; Barns-Jenkins, Caitlin; Holt, Chelsea; Sandoval, Karina; Siva Kumar, Vanessa; Ward, Lisa; Allen, Emily Caroline; Collis, Sarah Valerie; Cowie, Shannon; Francis, David; Delatycki, Martin B; Yiu, Eppie Mildred; Massie, R John; Pertile, Mark Domenic; du Sart, Desirée; Bruno, Damien; Amor, David J

    2017-10-26

    PurposeTo describe our experience of offering simultaneous genetic carrier screening for cystic fibrosis (CF), fragile X syndrome (FXS), and spinal muscular atrophy (SMA).MethodsCarrier screening is offered through general practice, obstetrics, fertility, and genetics settings before or in early pregnancy. Carriers are offered genetic counseling with prenatal/preimplantation genetic diagnosis available to those at increased risk.ResultsScreening of 12,000 individuals revealed 610 carriers (5.08%; 1 in 20): 342 CF, 35 FXS, 241 SMA (8 carriers of 2 conditions), approximately 88% of whom had no family history. At least 94% of CF and SMA carriers' partners were tested. Fifty couples (0.42%; 1 in 240) were at increased risk of having a child with one of the conditions (14 CF, 35 FXS, and 1 SMA) with 32 pregnant at the time of testing. Of these, 26 opted for prenatal diagnosis revealing 7 pregnancies affected (4 CF, 2 FXS, 1 SMA).ConclusionThe combined affected pregnancy rate is comparable to the population risk for Down syndrome, emphasizing the need to routinely offer carrier screening. The availability of appropriate genetic counseling support and a collaborative approach between laboratory teams, genetics services, health professionals offering screening, and support organizations is essential.Genet Med advance online publication, 26 October 2017; doi:10.1038/gim.2017.134.

  17. Endourologic Intervention for Management of Infertility in a Man with Zinner Syndrome Resulting in a Natural Pregnancy.

    Science.gov (United States)

    Aghaways, Ismaeel; Ahmed, Shyaw M

    2016-01-01

    Ipsilateral renal agenesis associated with seminal vesicular cysts is an uncommon finding. Zinner syndrome is a rare variant of wolffian duct anomalies with a triad of seminal vesicle cyst, ipsilateral renal agenesis, and male fertility problems due to ejaculatory duct obstruction (EDO). A 28-year-old man with 6 years history of primary infertility presented with left-side lower abdominal pain. A palpable cystic mass was found on digital rectal examination. Semen analysis revealed low volume ejaculate azoospermia. Abdominal ultrasonography revealed a single right kidney and transrectal ultrasonography showed an evidence of left EDO. Transurethral resection of the ejaculatory duct was performed. Semen analysis after 2 weeks showed normal sperm count (23M) and acceptable progressive motility (24%). Eight weeks later, his wife was pregnant with a 7-week viable fetus. Although not a common disease, a careful physical examination and thorough semen analysis interpretation should guide clinicians to diagnose a surgically treatable syndromic cause of male infertility.

  18. Mammary Duct Ectasia

    Science.gov (United States)

    ... may worry that it's a sign of breast cancer, especially if you develop a hard lump around a milk duct affected by mammary duct ectasia. While it's important to get signs and symptoms checked out promptly, remember that mammary duct ectasia does not increase your risk of breast cancer. By Mayo ... . Mayo Clinic Footer Legal ...

  19. About Cystic Fibrosis

    Science.gov (United States)

    ... Research Share Back to top What Is Cystic Fibrosis? Cystic fibrosis is a progressive, genetic disease that causes ... carrier. Read more about diagnosis. According to the Cystic Fibrosis Foundation Patient Registry, in the United States: More ...

  20. Therapies for Cystic Fibrosis

    Science.gov (United States)

    ... Search What Is CF? X close ABOUT CYSTIC FIBROSIS Learn about cystic fibrosis, a genetic disorder that affects the lungs, pancreas, ... or your child has just been diagnosed with cystic fibrosis, or your doctor has recommended testing for CF, ...

  1. Cystic Fibrosis and Pregnancy

    Science.gov (United States)

    ... Complications & Loss > Pregnancy complications > Cystic fibrosis and pregnancy Cystic fibrosis and pregnancy E-mail to a friend Please ... this page It's been added to your dashboard . Cystic fibrosis (CF) is a condition that affects breathing and ...

  2. Sclerotherapy for Benign Cystic Diseases in the Neck

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Ji Hoon; Sohn, Chul Ho; Choi, Seung Hong; Yun, Tae Jin [Dept. of Radiology, Seoul National University Hospital, Seoul National University College of Medicine, Seoul (Korea, Republic of)

    2012-08-15

    Surgery has been the classic treatment of choice for benign cystic diseases, including lymphatic malformation, ranula, branchial cleft cyst, thyroglossal duct cyst, thyroid cyst, parathyroid cyst, and lymphocele. However, surgery is associated with a tendency toward recurrence and may be accompanied by various complications, such as nerve injuries, vascular injuries, and scar formation. Therefore, sclerotherapy using various agents has been applied successfully to treatment of benign cystic diseases in the neck. This editorial reviews the use of various sclerotic agents and application of sclerotherapy to benign cystic diseases in the neck.

  3. An Unusual Cause of Thoracic Outlet Syndrome.

    Science.gov (United States)

    Zampieri, Davide; Marulli, Giuseppe; Mammana, Marco; Calabrese, Francesca; Schiavon, Marco; Rea, Federico

    2016-12-01

    Thoracic outlet syndrome (TOS) is a condition arising from compression of the subclavian vessels and/or brachial plexus. Many factors or diseases may cause compression of the neurovascular bundle at the thoracic outlet. We describe the case of a 41-year-old woman with TOS who presented with vascular venous symptoms. Chest computed tomography (CT) scan showed a cystic mass at the level of cervico-thoracic junction, located between the left subclavian artery and vein, which appeared compressed. The cystic mass was removed through a cervical approach and it was found to be a cyst arising from the thoracic duct compressing and anteriorly dislocating the left subclavian vein. After surgery symptoms promptly disappeared. Copyright © 2016 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier B.V. All rights reserved.

  4. Fluorescent Imaging With Indocyanine Green During Laparoscopic Cholecystectomy in Patients at Increased Risk of Bile Duct Injury

    NARCIS (Netherlands)

    Ankersmit, M.; Dam, D.A. van; Rijswijk, A.S. van; Tuynman, J.B.; Meijerink, W.J.H.J.

    2017-01-01

    BACKGROUND: Although rare, injury to the common bile duct (CBD) during laparoscopic cholecystectomy (LC) can be reduced by better intraoperative visualization of the cystic duct (CD) and CBD. The aim of this study was to establish the efficacy of early visualization of the CD and the added value of

  5. Main-duct intraductal papillary mucinous neoplasm of the pancreas: a case report

    Directory of Open Access Journals (Sweden)

    Natalia Manetti

    2012-12-01

    Full Text Available Three distinct entities among non-inflammatory cystic lesions of the pancreas have been defined: intraductal papillary mucinous neoplasm (IPMN, serous cystic neoplasm (SCN and mucinous cystic neoplasm (MCN. IPMN is characterized by intraductal papillary growth and thick mucus secretion: its incidence has dramatically increased since its initial description. These lesions probably can progress towards invasive carcinoma. IPMNs are symptomatic in most cases: the typical presentation is a recurrent acute pancreatitis, without evident cause, of low or moderate severity. The diagnosis is usually based upon the imaging (CT/cholangio-MRI demonstrating a pancreatic cystic mass, involving a dilated main duct, eventually associated to some filling defects, or a normal Wirsung duct communicating with the cyst lesion. Surgical treatment is generally indicated for main duct IPMN and branch duct IPMN with suspected malignancy (tumour size ≥ 30 mm, mural nodules, dilated main pancreatic duct, or positive cytology or prominent symptoms. Herein we present a case of IPMN of the main duct which occurred with abdominal and back pain associated with weight loss. After the diagnosis, she successfully underwent surgery and is now in a follow-up program.

  6. Cystic fibrosis and renal disease: a case report

    Directory of Open Access Journals (Sweden)

    Al-Shawwa Baha A

    2007-06-01

    Full Text Available Abstract Background Cystic fibrosis (CF is an autosomal recessive disease that is predominantly seen in the Caucasian population and involves multiple organs. Traditionally it has been thought that the kidney is the only organ which does not seem to be generally affected by the disease although the cystic fibrosis transmembrane conductance regulator (CFTR gene is expressed in the kidney. Case presentation We report the case of an 11 year old boy with cystic fibrosis and nephrotic syndrome and review the literature that describes nephrotic syndrome and renal involvement in cystic fibrosis. Conclusion With continued advances in the management of cystic fibrosis and improvement in life expectancy, several unrecognized co-morbidities are expected to emerge. It is important to screen patients for possible co-morbidities. Urine analysis may be helpful in this group of patients and any proteinuria should raise the suspicion of cystic fibrosis-related renal disease.

  7. Hepaticocystic duct and a rare extra-hepatic "cruciate" arterial anastomosis: a case report

    Directory of Open Access Journals (Sweden)

    Abeysuriya Vasitha

    2008-02-01

    Full Text Available Abstract Introduction The variations in the morphological characteristics of the extra-hepatic biliary system are interesting. Case presentation During the dissection of cadavers to study the morphological characteristics of the extra-hepatic biliary system, a 46-year-old male cadaver was found to have drainage of the common hepatic duct drains directly into the gall bladder neck. The right and left hepatic ducts were not seen extra-hepatically. Further drainage of the bile away from the gallbladder and into the duodenum was provided by the cystic duct. Formation of the common bile duct by the union of the common hepatic duct and cystic duct was absent. Further more the right hepatic artery was found to be communicating with the left hepatic artery by a "bridging artery" after giving rise to the cystic artery. An accessory hepatic artery originated from the "bridging artery" forming a "cruciate" hepatic arterial anastomosis. Conclusion Combination of a Hepaticocystic duct and an aberrant variation in the extra-hepatic arterial system is extremely rare.

  8. Simultaneous Papillary Carcinoma in Thyroglossal Duct Cyst and Thyroid

    Directory of Open Access Journals (Sweden)

    Gustavo Cancela e Penna

    2017-01-01

    Full Text Available Thyroglossal duct cyst (TDC is a cystic expansion of a remnant of the thyroglossal duct tract. Carcinomas in the TDC are extremely rare and are usually an incidental finding after the Sistrunk procedure. In this report, an unusual case of a 36-year-old woman with concurrent papillary thyroid carcinoma arising in the TDC and on the thyroid gland is presented, followed by a discussion of the controversies surrounding the possible origins of a papillary carcinoma in the TDC, as well as the current management options.

  9. Endoscopic treatment for complex biliary and pancreatic duct injuries

    Directory of Open Access Journals (Sweden)

    Simon Bouchard

    2014-01-01

    Full Text Available Severe injuries of biliary or pancreatic ducts are associated with significant morbidity and mortality. Severe bile duct injuries such as major biliary leaks, complete transection, or complete occlusion of bile ducts can be grouped under the term complex bile duct injuries (CBDI. In the spectrum of pancreatic duct injuries, disconnected pancreatic duct syndrome (DPDS represents the most severe form and most often occurs after a severe episode of acute pancreatitis. Treatment of these complex injuries is quite challenging and for many years surgical management has been considered the treatment of choice. However, in the past few years, some studies have reported the successful management of CBDI or DPDS using endoscopic procedures alone or in combination with a percutaneous approach. In this review, we detail the endoscopic or combined endoscopic/percutaneous treatment possibilities for CBDI and DPDS.

  10. Ectopic drainage of the common bile duct into the lesser curvature of the gastric antrum in a newborn with pyloric atresia, annular pancreas and congenital short bowel syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Scheida, Nicola; Amaral, Joao G. [Hospital for Sick Children, Department of Diagnostic Imaging, Division of Image Guided Therapy, Toronto (Canada); Wales, Paul W. [Hospital for Sick Children, Department of Surgery, Toronto (Canada); Krishnamurthy, Ganesh [Hospital for Sick Children, Department of Diagnostic Imaging, Division of Image Guided Therapy, Toronto (Canada); Children' s Hospital of Philadelphia, Department of Diagnostic Imaging, Philadelphia, PA (United States); Chait, Peter G. [Ellesmere X-Ray and Associates, Scarborough (Canada)

    2009-01-15

    We report a newborn with bilious vomiting and the rare combination of pyloric atresia, annular pancreas and ectopic drainage of the common bile duct into the lesser curvature of the gastric antrum. Radiologic, sonographic and percutaneous transhepatic transcholecystic cholangiographic (PTTC) findings, with surgical correlation, are presented. (orig.)

  11. CT of Mirizzi syndrome

    International Nuclear Information System (INIS)

    Yamamoto, Shinichiro; Fukushima, Keisuke; Ishihara, Kenji; Hirano, Yutaka; Sano, Kaizo

    1983-01-01

    PTC or ERCP findings of four cases of Mirizzi syndrome were demonstrated. They consisted of a smooth stricture of the common hepatic duct, curved impressions of the duct and dilatation of proximal biliary radicles. CT could visualize the impacted stone in the neck of the gallbladder, dilatation of proximal common hepatic and intrahepatic duct. Absence of the dilatation of distal common bile duct could also be confirmed by CT, thus the diagnosis of Mirizzi syndrome might be possible by CT. (author)

  12. A novel duct-lobular segmentectomy for breast tumors with nipple discharge using near-infrared indocyanine green fluorescence imaging

    Directory of Open Access Journals (Sweden)

    Tsuyoshi Ohno

    2013-10-01

    Full Text Available A 44-year-old woman was referred to our hospital with pathological nipple discharge from her left breast. Ultrasonography revealed a solid tumor beneath her left areola that measured 17 mm in diameter with a dilated mammary duct. Contrast-enhanced magnetic resonance imaging showed an early-enhanced cystic tumor and a dilated mammary duct. We performed a duct-lobular segmentectomy using near-infrared indocyanine green (ICG-fluorescence imaging. Under general anesthesia, a silicone tube was inserted into an orifice of a fluid-discharging mammary duct, and 1 mL dye-fluorescence liquid containing ICG and indigo carmine was injected into the mammary duct. A periareolar incision was made, and the fluorescence image of the demarcated mammary duct segment was obtained. The mammary duct segment was dissected, along with the demarcation line. The cystic lesion and dilated mammary duct were fully resected, and the pathological diagnosis was intraductal papilloma of the breast. We report that near-infrared ICG fluorescence could be applied for imaging of the mammary duct segment, and the fluorescence image allowed for easier duct-lobular segmentectomy for nipple discharge.

  13. Cystic Fibrosis (CF): Chloride Sweat Test

    Science.gov (United States)

    ... on this topic for: Parents Kids Teens Cystic Fibrosis Cystic Fibrosis and Nutrition Cystic Fibrosis (CF) Respiratory Screen: Sputum Cystic Fibrosis: Diet and Nutrition Cystic Fibrosis Cystic Fibrosis: Diet and Nutrition View more Partner Message ...

  14. Cystic fibrosis - nutrition

    Science.gov (United States)

    Cystic fibrosis (CF) is a life-threatening disease that causes thick, sticky mucus to build up in the ... Egan ME, Green DM, Voynow JA. Cystic fibrosis. In: Kliegman RM, ... of Pediatrics . 20th ed. Philadelphia, PA: Elsevier; 2016:chap ...

  15. Cystic Meningioma

    International Nuclear Information System (INIS)

    Takeda, Naoya; Fujita, Shigekiyo; Yasuda, Muneaki; Nakamura, Mitsugu; Kondo, Takeshi; Furumoto, Masaru

    1987-01-01

    Cystic meningiomas are rare. A 60-year-old housewife was admitted because of headache and left hemiparesis. An enhanced CT scan revealed a high-density area in the right frontotemporal region, while several well-circumscribed low-density areas were seen in the high-density area. Angiography showed a sun-burst appearance fed by the middle meningeal artery. At surgery, xanthochromic fluid was aspirated and the tumor was removed completely. The pathohistological finding was meningothelial meningioma with an intratumoral cyst. There were variable-sized vacuoles among the tumor cells, and there were some tumor cells with intracytoplasmic fat-stain-positive granules. We speculated that the mechanism of cyst formation is as follows: in the first step, far droplets are stored in the tumor cells; the numbers and/or sizes of the fats gradually increase, and then the intracytoplasmic fats disappear by an unknown mechanism and these cells become vacuoles. In the second step, these vacuoles fuse to microcysts, and the microcysts fuse to macrocysts. (author)

  16. Cystic fibrosis: case report

    International Nuclear Information System (INIS)

    Park, Si Hyun; Lee, Hyun Ju; Kim, Ji Hye; Park, Chol Heui

    2002-01-01

    Cystic fibrosis is an autosomal recessive genetic disease. Among Caucasians, it is the most common cause of pulmonary insufficiency during the first three decades of life. The prevalence of cystic fibrosis varies according to ethnic origin: it is common among Caucasians but rare among Asians. We report a case in which cystic fibrosis with bronchiectasis and hyperaeration was revealed by high-resolution CT, and mutation of the cystic fibrosis conductance transmembrane regulator gene (CFTR) by DNA analysis

  17. Cystic fibrosis: case report

    International Nuclear Information System (INIS)

    Park, Si Hyun; Lee, Hyun Ju; Kim, Ji Hye; Park, Chol Heui

    2002-01-01

    Cystic fibrosis is a autosomal recessive genetic disease. Among caucasians, it is the most common cause of pulmonary insufficiency during the first three decades of life. The prevalence of cystic fibrosis varies according to ethnic origin: it is common among caucasians but rare among Asians. We report a case in which cystic fibrosis with bronchiectasis and hyperaeration was revealed by high-resolution CT, and mutation of the cystic fibrosis conductance transmembrane regulator gene (CFTR) by DNA analysis

  18. Mammary Duct Ectasia

    Science.gov (United States)

    ... lumps Mammary duct ectasia Symptoms & causes Diagnosis & treatment Advertisement Mayo Clinic does not endorse companies or products. ... a Job Site Map About This Site Twitter Facebook Google YouTube Pinterest Mayo Clinic is a not- ...

  19. Ducted fuel injection

    Energy Technology Data Exchange (ETDEWEB)

    Mueller, Charles J.

    2018-03-06

    Various technologies presented herein relate to enhancing mixing inside a combustion chamber to form one or more locally premixed mixtures comprising fuel and charge-gas with low peak fuel to charge-gas ratios to enable minimal, or no, generation of soot and other undesired emissions during ignition and subsequent combustion of the locally premixed mixtures. To enable sufficient mixing of the fuel and charge-gas, a jet of fuel can be directed to pass through a bore of a duct causing charge-gas to be drawn into the bore creating turbulence to mix the fuel and the drawn charge-gas. The duct can be located proximate to an opening in a tip of a fuel injector. The duct can comprise of one or more holes along its length to enable charge-gas to be drawn into the bore, and further, the duct can cool the fuel and/or charge-gas prior to combustion.

  20. Modified nasolacrimal duct stenting

    International Nuclear Information System (INIS)

    Tian Min; Jin Mei; Chen Huanjun; Li Yi

    2008-01-01

    Objective: Traditional nasolacrimal duct stenting possesses some shortcoming including difficulty of pulling ball head guide wire from the nasal cavity with turbinate hypertrophy and nasal septal deviation. The new method of nose-oral tube track establishment can overcome the forementioned and increase the successful rate. Methods: 5 F catheter and arterial sheath were modified to be nasolacrimal duct stent delivery device respectively. Antegrade dacryocystography was taken firstly to display the obstructed site and followed by the modified protocol of inserting the guide wire through nasolacrimal duct and nasal cavity, and establishing the stent delivery track for retrograde stent placement. Results: 5 epiphora patients with failure implantation by traditional method were all succeeded through the modified stenting (100%). During 6-mouth follow-up, no serious complications and reocclusion occurred. Conclusion: The establishment of eye-nose-mouth-nose of external nasal guide wire track can improve the successful rate of nasolacrimal duct stenting. (authors)

  1. Effects of Metformin on Ovulation and Pregnancy Rate in Women with Clomiphene Resistant Poly Cystic Ovary Syndrome

    Directory of Open Access Journals (Sweden)

    Mahnaz Ashrafi

    2007-01-01

    Full Text Available Background: To evaluate the effect of metformin on ovulation and pregnancy rate in clomiphene citrateresistant women with polycystic ovary syndrome (PCOS.Material & Methods: In this clinical trial each patient, regarding her previous resistance to Clomiphene,served as her own control. A total of 35 clomiphene citrate resistant PCOS patients, referring to Royan institutewere studied. Clomiphene citrate resistance was defined as having failure of ovulation during at least threecycles using clomiphene citrate doses up to 200 mg/day on cycle days 3-7 after a withdrawal bleeding withprogesterone. Metformin was used alone or in combination with clomiphene citrate. First, the patients receivedmetformin up to 1500 mg/day for 8 weeks. During the next 2-3 cycle if the patients did not become pregnant,clomiphene was added with increments of 100 mg (up to 150 mg/day. Follicular development and ovulationwere monitored by ultrasound scans and mid-luteal progesterone level. Menstrual pattern, ovulation, andpregnancy rate were evaluated during the two stages of treatment.Results: After 8 weeks of meformin monotherapy, ovulation occurred in 23 cases (65.7% and 7 patients (20%became pregnant. Among other patients (28/35 who were treated with Clomiphene Cirate and metformin for64 cycles, 19 patients (67.8% had proper ovulation and five of them (17.8% became pregnant. Totally,metformin induced ovulation in 31 of 35 patients (88.6% and twelve (34.3% of them achieved pregnancy.Conclusion: Metformin alone or in combination with clomiphene is a very effective treatment in inducingovulation and pregnancy in clomiphene resistant women with PCOS.

  2. A case report of an unusual type of choledochal cyst with choledocholithiasis: Saccular dilatation of the confluent portion of both intrahepatic ducts

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Jin Young; Kim, Hee Jin; Han, Hyun Young [Dept. of Radiology, Eulji University Hospital, Daejeon (Korea, Republic of)

    2015-10-15

    A choledochal cyst is a rare congenital anomaly of the biliary system manifested as the cystic dilatation of bile ducts, usually occurring in the common bile duct. Here, we describe an unusual type of choledochal cyst in a 45-year-old male that did not fit into the most widely accepted Todani classification of these cysts. The lesion mimicked duplication anomalies of the gallbladder and was finally diagnosed as a choledochal cyst involving the confluent portion of both intrahepatic ducts.

  3. Cystic breast lymphangioma in adult female: An unusual presentation

    Directory of Open Access Journals (Sweden)

    Chhanda Das

    2016-01-01

    Full Text Available Cystic lymphangiomas are rare benign tumor of the breast in adults. They are usually located in head and neck, axilla, and mediastinum. They are most commonly diagnosed in young children. Nearly 90% are apparent by the age of 2 years. Here, we reporting a case of 20-year-old female presented with gradually increasing painless swelling in the upper outer quadrant of her left breast for 1½ years. Mammography of the breast showed multiple irregular hypoechoic lesions associated with irregular duct dilatation. Lumpectomy was performed and diagnosed as cystic lymphangioma. Complete excision is the treatment of choice.

  4. Curved-Duct

    Directory of Open Access Journals (Sweden)

    Je Hyun Baekt

    2000-01-01

    Full Text Available A numerical study is conducted on the fully-developed laminar flow of an incompressible viscous fluid in a square duct rotating about a perpendicular axis to the axial direction of the duct. At the straight duct, the rotation produces vortices due to the Coriolis force. Generally two vortex cells are formed and the axial velocity distribution is distorted by the effect of this Coriolis force. When a convective force is weak, two counter-rotating vortices are shown with a quasi-parabolic axial velocity profile for weak rotation rates. As the rotation rate increases, the axial velocity on the vertical centreline of the duct begins to flatten and the location of vorticity center is moved near to wall by the effect of the Coriolis force. When the convective inertia force is strong, a double-vortex secondary flow appears in the transverse planes of the duct for weak rotation rates but as the speed of rotation increases the secondary flow is shown to split into an asymmetric configuration of four counter-rotating vortices. If the rotation rates are increased further, the secondary flow restabilizes to a slightly asymmetric double-vortex configuration. Also, a numerical study is conducted on the laminar flow of an incompressible viscous fluid in a 90°-bend square duct that rotates about axis parallel to the axial direction of the inlet. At a 90°-bend square duct, the feature of flow by the effect of a Coriolis force and a centrifugal force, namely a secondary flow by the centrifugal force in the curved region and the Coriolis force in the downstream region, is shown since the centrifugal force in curved region and the Coriolis force in downstream region are dominant respectively.

  5. Study of biliary dynamics in the common bile duct using 99mTc-EHIDA

    International Nuclear Information System (INIS)

    Thirumurthi, K.; Rehani, M.M.; Das, B.K.

    1986-01-01

    The hepatobiliary imaging has been used for study of gallbladder and cystic duct disorders but its utility in evaluating dynamics of flow through common bile duct has not been explored. The pattern of curves over CBD and liver in patients with or without patent cystic duct has been studied. Normally the CBD curve shows a steady rise till peak (in 13.7 ± 3.6 min) in 99m Tc-EHIDA study followed by the steady fall. The time to peak for CBD curve, transit time from liver to CBD and the pattern of falling portion of the curve provide information about biliary dyskinesia. Moreover, comparison of the rate of fall of the liver curve with pattern of CBD curve further gives diagnostic information about dynamics in CBD. (author)

  6. Particle deposition in ventilation ducts

    Energy Technology Data Exchange (ETDEWEB)

    Sippola, Mark Raymond [Univ. of California, Berkeley, CA (United States)

    2002-09-01

    Exposure to airborne particles is detrimental to human health and indoor exposures dominate total exposures for most people. The accidental or intentional release of aerosolized chemical and biological agents within or near a building can lead to exposures of building occupants to hazardous agents and costly building remediation. Particle deposition in heating, ventilation and air-conditioning (HVAC) systems may significantly influence exposures to particles indoors, diminish HVAC performance and lead to secondary pollutant release within buildings. This dissertation advances the understanding of particle behavior in HVAC systems and the fates of indoor particles by means of experiments and modeling. Laboratory experiments were conducted to quantify particle deposition rates in horizontal ventilation ducts using real HVAC materials. Particle deposition experiments were conducted in steel and internally insulated ducts at air speeds typically found in ventilation ducts, 2-9 m/s. Behaviors of monodisperse particles with diameters in the size range 1-16 μm were investigated. Deposition rates were measured in straight ducts with a fully developed turbulent flow profile, straight ducts with a developing turbulent flow profile, in duct bends and at S-connector pieces located at duct junctions. In straight ducts with fully developed turbulence, experiments showed deposition rates to be highest at duct floors, intermediate at duct walls, and lowest at duct ceilings. Deposition rates to a given surface increased with an increase in particle size or air speed. Deposition was much higher in internally insulated ducts than in uninsulated steel ducts. In most cases, deposition in straight ducts with developing turbulence, in duct bends and at S-connectors at duct junctions was higher than in straight ducts with fully developed turbulence. Measured deposition rates were generally higher than predicted by published models. A model incorporating empirical equations based on the

  7. Genetics Home Reference: cystic fibrosis

    Science.gov (United States)

    ... Share: Email Facebook Twitter Home Health Conditions Cystic fibrosis Cystic fibrosis Printable PDF Open All Close All Enable Javascript ... lung damage, including the formation of scar tissue (fibrosis) and cysts in the lungs. Most people with cystic fibrosis ...

  8. A case of branch duct type intraductal papillary neoplasm of the bile duct treated by open surgery after 11 years of follow-up.

    Science.gov (United States)

    Fujita, Mitsuru; Wakui, Noritaka; Yamauchi, Yoshiya; Takeda, Yuki; Sato, Takemasa; Ueki, Nobuo; Otsuka, Takafumi; Oba, Nobuyuki; Nishinakagawa, Shuta; Minagawa, Masami; Takeda, Yasushi; Shiono, Saori; Kojima, Tatsuya

    2013-11-01

    The intraductal papillary neoplasm of the bile duct (IPNB) is a novel disease concept that was recently classified as a biliary cystic tumor by the revised World Health Organization classification. This is the case report of a 70-year-old female patient who experienced repeated episodes of obstructive jaundice and cholangitis since 2000, attributed to a mucus-producing hepatic tumor. Surgery was advised due to the repeated episodes; however, the patient refused. In May, 2011, the patient developed jaundice and fever and was treated with antibiotics. Since there was no improvement, the patient was admitted to the Tokyo Rosai Hospital. Abdominal computed tomography (CT) revealed a 50-mm cystic mass with an internal septum in the left hepatic lobe. Although the tumor size had remained almost unchanged compared to the initial CT scan performed in 2000, intra- and extra-hepatic bile duct dilation was more prominent on the second CT scan. Following admission, endoscopic retrograde cholangiopancreatography was performed and revealed an expanded papilla of Vater due to a mucous plug. A balloon catheter was inserted into the bile duct to remove the mucous plug, resulting in the drainage of copious amounts of mucus and infected bile. The patient finally consented to surgery and left hepatic lobectomy was performed. Consequently, the diagnosis of low-grade IPNB was made. Branch duct type IPNB, which is characterized by imaging appearance of a cystic mass and slow progression, is attracting increasing attention. In the present case, a cystic mass was identified in the left hepatic lobe, with no significant change in size after 11 years of follow-up, leading to the diagnosis of branch duct type IPNB. Considering the fact that IPNB is usually treated surgically at the time of diagnosis, the present case, due to the long-term follow-up, provides valuable insight into the natural history of the tumor.

  9. Diagnosis of cystic fibrosis in the kindred of an infant with CFTR-related metabolic syndrome: importance of follow-up that includes monitoring sweat chloride concentrations over time.

    Science.gov (United States)

    Williams, Sophia N; Nussbaum, Eliezer; Chin, Terry W; Do, Paul C M; Singh, Kathryn E; Randhawa, Inderpal

    2014-03-01

    Newly implemented newborn screening (NBS) programs in California have resulted in a large subset of patients in whom at least two cystic fibrosis transmembrane conductance regulator (CFTR) mutations are identified, but subsequent sweat chloride analysis reveals normal or indeterminate values. These patients are diagnosed with CFTR-Related Metabolic Syndrome (CRMS). However, the natural progression and management of these patients are not clearly understood and frequently after the age of 1-year these patients are lost to follow-up with Cystic Fibrosis (CF) Centers. We present the first case of an infant who was referred to Miller Children's Hospital for a NBS positive for CF and subsequent discovery of identical mutations in six of his seven older brothers. Several siblings had positive sweat chloride results on repeat testing after the age of 3 years. We suggest the need for continued follow-up of CRMS in a CF center with diagnostic evaluation including repeat sweat chloride testing, beyond the currently recommended period. © 2013 Wiley Periodicals, Inc.

  10. Ultrasound-guided sclerotherapy for benign non-thyroid cystic mass in the neck

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Ji Hoon [Dept. of Radiology, Seoul National University Hospital, Seoul National University College of Medicine, Seoul (Korea, Republic of)

    2014-04-15

    Surgical excision has traditionally been the treatment of choice for benign non-thyroid cystic neck masses, including lymphatic malformation, ranula, branchial cleft cyst, thyroglossal duct cyst, and parathyroid cyst. However, there is a tendency toward recurrence after surgery, and surgery may be accompanied by complications, including nerve injuries, vascular injuries, and scar formation. Ultrasound-guided sclerotherapy using various agents has been challenged and successfully applied as an alternative treatment for benign non-thyroid cystic neck masses. This report reviews the available sclerosing agents and describes the applications of sclerotherapy to the treatment of benign cystic masses in the neck.

  11. Neonatal cystic fibrosis screening test

    Science.gov (United States)

    Cystic fibrosis screening - neonatal; Immunoreactive trypsinogen; IRT test; CF - screening ... Cystic fibrosis is a disease passed down through families. CF causes thick, sticky mucus to build up in ...

  12. Normal sweat chloride test does not rule out cystic fibrosis.

    Science.gov (United States)

    Başaran, Abdurrahman Erdem; Karataş-Torun, Nimet; Maslak, İbrahim Cemal; Bingöl, Ayşen; Alper, Özgül M

    2017-01-01

    Başaran AE, Karataş-Torun N, Maslak İC, Bingöl A, Alper ÖM. Normal sweat chloride test does not rule out cystic fibrosis. Turk J Pediatr 2017; 59: 68-70. A 5-month-old patient presented with complaints of fever and cough. He was hospitalized with the diagnosis of bronchopneumonia and pseudo-Bartter's syndrome. Patient was further investigated for diagnosis of cystic fibrosis. The chloride (Cl) level in sweat was determined within the normal range (25.1 mmol/L, 20.3 mmol/L). CFTR (Cystic Fibrosis Transmembrane Regulator gene; NM_000492.2) genotyping results were positive for p.E92K; p.F1052V mutations. The patient was diagnosed with cystic fibrosis. In our patient, with features of CF and normal sweat test, mutation analysis was helpful for the diagnosis of cystic fibrosis.

  13. Bile Duct Exploration

    Science.gov (United States)

    ... 223.2273 Request an Appointment Contact Us Share Facebook Twitter Linkedin Email Bile Duct Exploration Menu Print Full Article Overview Test Details Results and Follow-Up Cleveland Clinic is a non-profit academic medical center. Advertising on our site helps support our mission. We ...

  14. Duct Leakage Repeatability Testing

    Energy Technology Data Exchange (ETDEWEB)

    Walker, Iain [Lawrence Berkeley National Lab. (LBNL), Berkeley, CA (United States); Sherman, Max [Lawrence Berkeley National Lab. (LBNL), Berkeley, CA (United States)

    2014-08-01

    The purpose of this report is to evaluate the repeatability of the three most significant measurement techniques for duct leakage using data from the literature and recently obtained field data. We will also briefly discuss the first two factors. The main question to be answered by this study is to determine if differences in the repeatability of these tests methods is sufficient to indicate that any of these methods is so poor that it should be excluded from consideration as an allowed procedure in codes and standards. The three duct leak measurement methods assessed in this report are the two duct pressurization methods that are commonly used by many practitioners and the DeltaQ technique. These are methods B, C and A, respectively of the ASTM E1554 standard. Although it would be useful to evaluate other duct leak test methods, this study focused on those test methods that are commonly used and are required in various test standards, such as BPI (2010), RESNET (2014), ASHRAE 62.2 (2013), California Title 24 (CEC 2012), DOE Weatherization and many other energy efficiency programs.

  15. Cystic Granular Cell Ameloblastoma

    OpenAIRE

    Thillaikarasi, Rathnavel; Balaji, Jayaram; Gupta, Bhawna; Ilayarja, Vadivel; Vani, Nandimandalam Venkata; Vidula, Balachander; Saravanan, Balasubramaniam; Ponniah, Irulandy

    2010-01-01

    Ameloblastoma is a locally aggressive benign epithelial odontogenic tumor, while unicystic ameloblastoma is a relatively less aggressive variant. Although rare in unicystic or cystic ameloblastoma, granular cell change in ameloblastoma is a recognized phenomenon. The purpose of the present article is to report a case of cystic granular cell ameloblastoma in 34-year old female.

  16. Diagnosis of cystic fibrosis

    NARCIS (Netherlands)

    H.J. Veeze

    1995-01-01

    textabstractApplying the sweat-test as the first choice of test when a diagnosis of cystic fibrosis is suspected is still common practice and advisable. Since the cloning of the CFTR gene more than 400 different cystic fibrosis (CF) mutations have already been identified. The use of CF mutation

  17. 14 CFR 25.1103 - Induction system ducts and air duct systems.

    Science.gov (United States)

    2010-01-01

    ... 14 Aeronautics and Space 1 2010-01-01 2010-01-01 false Induction system ducts and air duct systems....1103 Induction system ducts and air duct systems. (a) Each induction system duct upstream of the first... auxiliary power unit bleed air duct systems, no hazard may result if a duct failure occurs at any point...

  18. A Study of Technical Approach Methods to Transabdominal Ultrasonography of the Extrahepatic Bile Ducts and of Following Effects from the Scan Training

    International Nuclear Information System (INIS)

    Lee, In Ja; Kang, Dae Hyun; Kim, Bo Young

    2008-01-01

    The purposes of this study are to analyze abnormal dilatation of the extrahepatic bile ducts by using transabdominal ultrasound, to confirm the existence of bile ducts diseases and their interrelationship, and for it to give a new theoretical basis for the technical access to extrahepatic bile ducts, upon which to analyze the ripple effects of the scan training. After teaching technical access process based on the new theory about extrahepatic bile duct to the thirty students who are studying ultrasonography, we allocated three hours per one student (30 mins 6 times) to focus on the training of scanning skill. Training has been performed by one-to-one method. For evaluation, all the students have to perform the scans on (1) confluence of the right and left hepatic ducts (extrahepatic bile ducts and cystic duct), (2) the suprapancreatic bile duct, (3) the intrapancreatic bile duct, (4) intrapapilla Duct, based on the clearly divided concept. The existing training and methods have had low confidency about transabdominal ultrasonography of the extrahepatic bile duct and had limitation with which they could image only the suprapancreatic bile duct. The evaluation after finishing the train based on the new theory, however, all the students (30 students) can access to (1) confluence of the right and left hepatic ducts(extrahepatic bile ducts and cystic duct), (2) the suprapancreatic bile duct objectively. 24 students can access to (3) the intrapancreatic bile duct and only one student can even make an image for (4) the intrapapilla Duct Though the evaluation on extrahepatic bile duct has to be performed with multi-sided method considering intrahepatic cause, bile duct cause and pathophysiological cause, only if we can image the extrahepatic bile duct to ampular of Vater objectively and confidently, we can greatly reduce invasive procedure such as ERCP, which is for the purpose of simple differential diagnosis and painful to the patients. Therefore if we concentrate on

  19. Noise suppression in duct

    International Nuclear Information System (INIS)

    Ahmed, A.; Barfeh, M.A.G.

    2001-01-01

    In air-conditioning system the noise generated by supply fan is carried by conditioned air through the ductwork. The noise created in ductwork run may be transmission, regenerative and ductborne. Transmission noise is fan noise, regenerative noise is due to turbulence in flow and ductborne noise is the noise radiating from duct to surroundings. Some noise is attenuated in ducts also but if noise level is high then it needs to be attenuated. A simple mitre bend can attenuate-noise. This principle is extended to V and M-shape ducts with inside lining of fibreglass, which gave maximum attenuation of 77 dB and 62 dB respectively corresponding to 8 kHz frequency as compared to mitre, bend giving maximum 18 dB attenuation. Sound level meter measured sound levels with octave band filter and tests were conducted in anechoic room. A V-shape attenuator can be used at fan outlet and high frequency noise can be minimized greatly. (author)

  20. A case of branch duct type intraductal papillary neoplasm of the bile duct treated by open surgery after 11 years of follow-up

    OpenAIRE

    FUJITA, MITSURU; WAKUI, NORITAKA; YAMAUCHI, YOSHIYA; TAKEDA, YUKI; SATO, TAKEMASA; UEKI, NOBUO; OTSUKA, TAKAFUMI; OBA, NOBUYUKI; NISHINAKAGAWA, SHUTA; MINAGAWA, MASAMI; TAKEDA, YASUSHI; SHIONO, SAORI; KOJIMA, TATSUYA

    2013-01-01

    The intraductal papillary neoplasm of the bile duct (IPNB) is a novel disease concept that was recently classified as a biliary cystic tumor by the revised World Health Organization classification. This is the case report of a 70-year-old female patient who experienced repeated episodes of obstructive jaundice and cholangitis since 2000, attributed to a mucus-producing hepatic tumor. Surgery was advised due to the repeated episodes; however, the patient refused. In May, 2011, the patient deve...

  1. Posterior midline cervical fetal cystic hygroma.

    Directory of Open Access Journals (Sweden)

    Oak S

    1992-04-01

    Full Text Available Posterior midline cervical cystic hygromas (PMC are frequently found associated with chromosomal aberrations and usually do not survive. The present report illustrates diagnosis of this condition by sonography in an 18 weeks old fetus and an amniocentesis revealed 45 x0 karyotype and increased concentration of alpha-fetoproteins. Pregnancy was terminated in view of Turner′s syndrome. The etiology and natural history of the condition is reviewed.

  2. Benign obstruction of the common hepatic duct (Mirizzi syndrome: diagnosis and operative management Obstrução benigna do ducto hepático comum (síndrome de Mirizzi: diagnóstico e tratamento operatório

    Directory of Open Access Journals (Sweden)

    Jaques Waisberg

    2005-03-01

    Full Text Available BACKGROUND: Mirizzi syndrome is a rare complication of prolonged cholelithiasis, characterized by narrowing of the common hepatic duct due to mechanical compression and/or inflammation due to biliary calculus impacted in the infundibula of the gallbladder or in the cystic duct. OBJECTIVES: To describe a series of eight consecutive patients with Mirizzi syndrome, at a single institution, submitted to surgical treatment and to comment on their aspects with emphasis on the diagnosis and treatment. METHODS: Four women and four men, with a mean age of 61.6 years (42 to 82 years, presenting Mirizzi syndrome were operated between 1997 and 2003. The following items were evaluated: clinical presentation, laboratory results, preoperative evaluation, operative findings, presence of choledocholithiasis, type of Mirizzi syndrome according to the classification by Csendes, choice of operative procedures, and complications. RESULTS: The most frequent symptoms were abdominal pain (87.5% and jaundice (87.5%. All the patients presented altered hepatic function tests. The diagnosis of Mirizzi syndrome was intra-operative in seven (87.5% patients, and preoperative in one (12.5%. Cholecystocholedochal fistula associated with choledocholithiasis was observed in three (37.5% cases. Mirizzi syndrome was classified as Csendes type I in five (62.5% patients, type II in one (12.5%, type III in one (12,5% and type IV in another (12.5%. Cholecystectomy, as an isolated surgical procedure, was performed in four (50.0% patients. One (12.5% patient was submitted to partial cholecystectomy and closure of the fistulous orifice with the central part of the infundibula. Two (25.0% patients were submitted to cholecystectomy and side-to-side choledochoduodenostomy and another (12.5% to side-to-side choledochoduodenostomy remaining the gallbladder in situ. Seven (87.5% patients had an uneventful recovery and were discharged in good conditions. One (12.5% patient presented a

  3. Duct having oscillatory side wall

    Energy Technology Data Exchange (ETDEWEB)

    Sprouse, Kenneth M.

    2018-04-03

    A pump system includes a particulate consolidator pump that has a pump outlet. A duct is coupled to the pump outlet. The duct has a wall that is coupled with an oscillator. The oscillator is operable to oscillate the wall at a controlled frequency. The controlled frequency is selected with respect to breaking static bridging of particulate in the duct due, at least in part, to consolidation of the particulate from a downstream check valve.

  4. 46 CFR 116.610 - Ventilation ducts.

    Science.gov (United States)

    2010-10-01

    ... served by the ventilation duct for shutting off the passage of air through the ventilation duct in the... 46 Shipping 4 2010-10-01 2010-10-01 false Ventilation ducts. 116.610 Section 116.610 Shipping... Ventilation § 116.610 Ventilation ducts. (a) For the purposes of this section, a ventilation duct includes any...

  5. Acquired Cystic Kidney Disease

    Science.gov (United States)

    ... Eating, Diet, & Nutrition for PKD Race, Ethnicity, & Kidney Disease Renal Artery Stenosis Renal Tubular Acidosis Simple Kidney Cysts ... kidneys to develop multiple cysts. Acquired cystic kidney disease occurs in children and adults who have chronic kidney disease (CKD) — ...

  6. Cystic Pleural Synovial Sarcoma.

    Science.gov (United States)

    Sharif, Atif; Akhtar, Tasleem; Akhtar, Mumtaz; Zia, Naeem

    2016-11-01

    Fewer than 40 cases of primary pleural synovial sarcoma have been reported so far with only 3 cases of cystic synovial sarcoma including cases originating from sites other than the pleura. Here, we present an exceedingly rare case of cystic synovial sarcoma originating from the mediastinal side of the visceral pleura in a 25-year man presenting with hemoptysis. On contrast-enhanced computed tomography (CT), cystic synovial sarcoma and cystic thymoma were difficult to be distinguished due to mediastinal location. Histopathological examination showed spindled morphology of tumor cells with hypercellularity and nuclear atypia. As these features are associated with both monophasic fibrous synovial sarcoma and type Athymoma, immunohistochemistry was performed. Adiagnosis of synovial sarcoma was confirmed by detection of CD99 and EMAand negativity of other markers. Fluorescence in situhybridization (FISH) was not done. Surgical excision was done and followed by oncology referral.

  7. Congenital dilatation of the large and segmental intrahepatic bile ducts (Caroli's disease in two Golden retriever littermates : clinical communication

    Directory of Open Access Journals (Sweden)

    R.D. Last

    2006-06-01

    Full Text Available Two, sibling, male Golden retriever puppies, 13 weeks of age, were presented with congenital biliary cysts of the liver involving both hepatic and segmental bile ducts, as well as bilateral polycystic kidney disease. Ultrasonography of the livers of both pups demonstrated segmental cystic lesions that were contiguous with the bile ducts. Histopathology revealed cystic ectatic bile duct hyperplasia and dysplasia with variable portal fibrosis in the liver, while in the kidneys there were radially arranged, cylindrically dilated cysts of the collecting ducts, which extended through the medulla and cortex. This pathology was compatible with that of congenital dilatation of the large and segmental bile ducts (Caroli's disease described in humans, dogs and rats. In humans Caroli's disease has an autosomal recessive inheritance pattern, while in rats activation of the MEK5/ERK cascade initiates the biliary dysgenesis of Caroli's disease in this species. However, the exact mode of inheritance and pathogenesis of Caroli's disease in dogs is as yet unknown. Previous reports on congenital hepatic cystic diseases of the dog have described Caroli's disease like lesions in various breeds, but these are believed to be the 1st reported cases in the Golden retriever breed.

  8. Cystic hygroma: An overview

    Directory of Open Access Journals (Sweden)

    Bilal Mirza

    2010-01-01

    Full Text Available Cystic hygromas are the cystic variety of lymphangioma, common locations being cervico-facial regions and axilla. Respiratory distress, recurrent infections or cosmetic reasons are the main indications of the treatment. The ideal treatment is complete surgical excision; however, there is a gradual conversion towards sclerosant therapy. This article reviews the current literature and discusses the various problems encountered during the management of these lesions.

  9. Ligadura de ducto biliar como modelo de estudo da síndrome hepatopulmonar e estresse oxidativo Common bile duct ligation as a model of hepatopulmonary syndrome and oxidative stress

    Directory of Open Access Journals (Sweden)

    Juliana Tieppo

    2005-12-01

    hepatopulmonary syndrome is characterized by hepatic dysfunction and presence of dilated pulmonary vessels, with alterations in air diffusion that can be demonstrated in the experimental model of common bile duct ligation. AIM: To evaluate the oxidative stress in pulmonary tissue of cirrhotic rats with common bile duct ligation. MATERIAL/METHODS: We used 12 male Wistar rats weighing between 200-300 g divided in two groups: control (Co = 6 and cirrhotic (Ci = 6. We evaluated aminotransferases, arterial gasometry, lipoperoxidation and chemoluminescence, and antioxidant enzymatic activity with superoxide dismutase. The tissues analyzed for hepatopulmonary syndrome were cirrhotic liver and lung. RESULTS: The animals with common bile duct ligation showed alterations in the following aminotransferases: aspartate aminotransferase, Co = 105.3 ± 43/Ci = 500.5 ± 90.3, alanine aminotransferase, Co = 78.75 ± 37.7/Ci = 162.75 ± 35.4, and alkaline phosphatase, Co = 160 ± 20.45/Ci = 373 ± 45.44. The lipoperoxidation and the antioxidant response had significant differences between the groups when evaluated in lung (lipoperoxidation Co = 0.87 ± 0.3/Ci = 2.01 ± 0.9, chemoluminescence Co = 16008.41 ± 1171.45/Ci = 20250.36 ± 827.82 superoxide dismutase Co = 6.66 ± 1.34/Ci = 16.06 ± 2.67. CONCLUSIONS: Our results suggest that in this experimental model of cirrhosis using common bile duct ligation, there is an increase in lipoperoxidation in pulmonary tissue as well as an increase in superoxide dismutase's antioxidant activity, suggesting a pulmonary injury caused by secondary biliary cirrhosis.

  10. 14 CFR 29.1103 - Induction systems ducts and air duct systems.

    Science.gov (United States)

    2010-01-01

    ... 14 Aeronautics and Space 1 2010-01-01 2010-01-01 false Induction systems ducts and air duct....1103 Induction systems ducts and air duct systems. (a) Each induction system duct upstream of the first... it might cause a fire hazard. (b) Each duct must be strong enough to prevent induction system failure...

  11. Restoration of CFTR Activity in Ducts Rescues Acinar Cell Function and Reduces Inflammation in Pancreatic and Salivary Glands of Mice.

    Science.gov (United States)

    Zeng, Mei; Szymczak, Mitchell; Ahuja, Malini; Zheng, Changyu; Yin, Hongen; Swaim, William; Chiorini, John A; Bridges, Robert J; Muallem, Shmuel

    2017-10-01

    Sjögren's syndrome and autoimmune pancreatitis are disorders with decreased function of salivary, lacrimal glands, and the exocrine pancreas. Nonobese diabetic/ShiLTJ mice and mice transduced with the cytokine BMP6 develop Sjögren's syndrome and chronic pancreatitis and MRL/Mp mice are models of autoimmune pancreatitis. Cystic fibrosis transmembrane conductance regulator (CFTR) is a ductal Cl -  channel essential for ductal fluid and HCO 3 - secretion. We used these models to ask the following questions: is CFTR expression altered in these diseases, does correction of CFTR correct gland function, and most notably, does correcting ductal function correct acinar function? We treated the mice models with the CFTR corrector C18 and the potentiator VX770. Glandular, ductal, and acinar cells damage, infiltration, immune cells and function were measured in vivo and in isolated duct/acini. In the disease models, CFTR expression is markedly reduced. The salivary glands and pancreas are inflamed with increased fibrosis and tissue damage. Treatment with VX770 and, in particular, C18 restored salivation, rescued CFTR expression and localization, and nearly eliminated the inflammation and tissue damage. Transgenic overexpression of CFTR exclusively in the duct had similar effects. Most notably, the markedly reduced acinar cell Ca 2+ signaling, Orai1, inositol triphosphate receptors, Aquaporin 5 expression, and fluid secretion were restored by rescuing ductal CFTR. Our findings reveal that correcting ductal function is sufficient to rescue acinar cell function and suggests that CFTR correctors are strong candidates for the treatment of Sjögren's syndrome and pancreatitis. Copyright © 2017 AGA Institute. Published by Elsevier Inc. All rights reserved.

  12. Intrahepatic transposition of bile ducts.

    Science.gov (United States)

    Delić, Jasmin; Savković, Admedina; Isaković, Eldar; Marković, Sergije; Bajtarevic, Alma; Denjalić, Amir

    2012-01-01

    Objective. To describe the intrahepatic bile duct transposition (anatomical variation occurring in intrahepatic ducts) and to determine the frequency of this variation. Material and Methods. The researches were performed randomly on 100 livers of adults, both sexes. Main research methods were anatomical macrodissection. As a criterion for determination of variations in some parts of bile tree, we used the classification of Segmentatio hepatis according to Couinaud (1957) according to Terminologia Anatomica, Thieme Stuugart: Federative Committee on Anatomical Terminology, 1988. Results. Intrahepatic transposition of bile ducts was found in two cases (2%), out of total examined cases (100): right-left transposition (right segmental bile duct, originating from the segment VIII, joins the left liver duct-ductus hepaticus sinister) and left-right intrahepatic transposition (left segmental bile duct originating from the segment IV ends in right liver duct-ductus hepaticus dexter). Conclusion. Safety and success in liver transplantation to great extent depends on knowledge of anatomy and some common embryological anomalies in bile tree. Variations in bile tree were found in 24-43% of cases, out of which 1-22% are the variations of intrahepatic bile ducts. Therefore, good knowledge on ductal anatomy enables good planning, safe performance of therapeutic and operative procedures, and decreases the risk of intraoperative and postoperative complications.

  13. Propagation of sound waves in ducts

    DEFF Research Database (Denmark)

    Jacobsen, Finn

    2000-01-01

    Plane wave propagation in ducts with rigid walls, radiation from ducts, classical four-pole theory for composite duct systems, and three-dimentional waves in wave guides of various cross-sectional shape are described.......Plane wave propagation in ducts with rigid walls, radiation from ducts, classical four-pole theory for composite duct systems, and three-dimentional waves in wave guides of various cross-sectional shape are described....

  14. Alveolar inflammation in cystic fibrosis

    DEFF Research Database (Denmark)

    Ulrich, Martina; Worlitzsch, Dieter; Viglio, Simona

    2010-01-01

    BACKGROUND: In infected lungs of the cystic fibrosis (CF) patients, opportunistic pathogens and mutated cystic fibrosis transmembrane conductance regulator protein (CFTR) contribute to chronic airway inflammation that is characterized by neutrophil/macrophage infiltration, cytokine release...

  15. Ultrasound Imaging of Cystic Nephroma

    Directory of Open Access Journals (Sweden)

    Federico Greco

    2017-07-01

    Full Text Available Cystic nephroma is a rare, benign multicystic lesion of the kidney. This tumor occurs both in children and in adults. In children, it is highly prevalent in males; in adults, it is more frequent in women. The term “cystic nephroma” represents two apparently different entities: pediatric cystic nephroma, a benign form thought to originate from metanephric tissue, and adult cystic nephroma, considered as a lesion of mixed epithelial stromal tumor. The clinical presentation may be a palpable mass or nonspecific symptoms such as abdominal pain, hematuria, and urinary tract infections. In this review, we summarize the ultrasound imaging features of cystic nephroma and describe the characteristics of the most common renal cystic lesions and the differential diagnosis of cystic nephroma with other renal cystic lesions.

  16. Cystic Fibrosis: Diet and Nutrition

    Science.gov (United States)

    ... Staying Safe Videos for Educators Search English Español Cystic Fibrosis: Diet and Nutrition KidsHealth / For Teens / Cystic Fibrosis: ... Enzyme Supplements Beating the Frustration Print What Is Cystic Fibrosis? At lunch, Lindsay often gets bored with having ...

  17. Surprisingly low infertility rate in married type 2 diabetic women: A rather curious paradox to the current opinion of insulin resistance as the joint pathogenesis of poly cystic ovary syndrome and type 2 diabetes mellitus.

    Science.gov (United States)

    Tavakolian Arjmand, Abbas; Nouri, Mahnaz; Tavakolian Arjmand, Shima

    2015-01-01

    Sharing the same pathophysiologic principle which is insulin resistance, type 2 diabetes mellitus (T2DM) and poly cystic ovary syndrome (PCOS) are usually considered closely related and easily interchangeable medical entities. Numerous attempts have been made to document this illusory perspective. Based on a delicate pathophysiologic notion, we believe that fully developed T2DM is infrequently observed with fully featured PCOS. In an observational descriptive study 257 married T2DM women were consecutively included and meticulously investigated for fertility history and, albeit, clinical and biochemical features of PCOS. Of 257 married diabetic women only six (2.3%) had no children. In one case a male problem (azoospermia) and in the second case, late marriage (aged 45 at wedding ceremony) was the cause of infertility. Thus, only four (1.6%) might have been labeled as true female factor infertility. Astounding to report was the average pregnancies for each participant which was 5.1±2.5, ranging from zero to fifteen. we would suggest that, despite the well-established fact of insulin resistance as the common pathophysiologic process for T2DM and PCOS, they are definitely separate medical entities. As a matter of fact T2DM and PCOS are the two opposite aspect of the insulin resistance coin. Copyright © 2015 Diabetes India. Published by Elsevier Ltd. All rights reserved.

  18. Causes and Prevention of Laparoscopic Bile Duct Injuries

    Science.gov (United States)

    Way, Lawrence W.; Stewart, Lygia; Gantert, Walter; Liu, Kingsway; Lee, Crystine M.; Whang, Karen; Hunter, John G.

    2003-01-01

    Objective To apply human performance concepts in an attempt to understand the causes of and prevent laparoscopic bile duct injury. Summary Background Data Powerful conceptual advances have been made in understanding the nature and limits of human performance. Applying these findings in high-risk activities, such as commercial aviation, has allowed the work environment to be restructured to substantially reduce human error. Methods The authors analyzed 252 laparoscopic bile duct injuries according to the principles of the cognitive science of visual perception, judgment, and human error. The injury distribution was class I, 7%; class II, 22%; class III, 61%; and class IV, 10%. The data included operative radiographs, clinical records, and 22 videotapes of original operations. Results The primary cause of error in 97% of cases was a visual perceptual illusion. Faults in technical skill were present in only 3% of injuries. Knowledge and judgment errors were contributory but not primary. Sixty-four injuries (25%) were recognized at the index operation; the surgeon identified the problem early enough to limit the injury in only 15 (6%). In class III injuries the common duct, erroneously believed to be the cystic duct, was deliberately cut. This stemmed from an illusion of object form due to a specific uncommon configuration of the structures and the heuristic nature (unconscious assumptions) of human visual perception. The videotapes showed the persuasiveness of the illusion, and many operative reports described the operation as routine. Class II injuries resulted from a dissection too close to the common hepatic duct. Fundamentally an illusion, it was contributed to in some instances by working too deep in the triangle of Calot. Conclusions These data show that errors leading to laparoscopic bile duct injuries stem principally from misperception, not errors of skill, knowledge, or judgment. The misperception was so compelling that in most cases the surgeon did not

  19. CT findings and differential diagnosis of cystic neck masses

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Ji Yeon; Lee, Kil Jun; Jeong, Seong Ki; Han, Seong Nim; Tae, Seok; Shin, Kyoung Ja; Lee, Sang Chun [Seoul Red Cross Hospital, Seoul (Korea, Republic of)

    1995-10-15

    The purpose of this study is to analyze the CT features of the cystic masses in the neck and to review differential diagnosis. We retrospectively reviewed and analyzed the CT findings of 22 histopathologically proved, cystic neck masses in regard to the location in fascial plane and relationship with adjacent organ. Of 22 cases, ten congenital cysts two ranulas, seven inflammatory lesions, and three solid tumors were included. Ten congenital cystic masses were located in typical locations as branchial cleft cyst (5) in mandibular angle, thyroglossal duct cyst (3) in visceral space embeded within the strap muscles, cystic hygroma (1) and cavernous hemangioma (1) in posterior cervical space with insinuating appearance. Two cases of ranula included one simple ranula localized in sublingual space and a plunging ranula extending to adjacent submandibular space. Seven cases of inflammatory lesions were characterized by multispatial locations and good contrast-enhancement of walls and adjacent tissue. Solid masses of low density mimicking cyst were two pleomorphic adenomas of submandibular gland and one neurilemmoma. It is considered that thorough analysis of the CT findings with attention to typical location, CT appearance, and the relationship with the adjacent structures usually leads to the correct diagnosis.

  20. The role of pancreatic ducts in the pathogenesis of acute pancreatitis.

    Science.gov (United States)

    Hegyi, Peter; Rakonczay, Zoltan

    2015-07-01

    Pancreatic ducts secrete 2.5 l of alkaline, HCO3(-)-rich fluid daily which greatly contributes to the homeostasis of the pancreas. Ducts are also important in the pathophysiology of the pancreas; alteration of ductal function can lead to severe diseases such as cystic fibrosis and chronic pancreatitis. The role of pancreatic ducts in the development of acute pancreatitis has only been uncovered recently. Pancreatitis inducing agents like bile acids and ethanol dose-dependently affect pancreatic ductal secretion; low concentrations stimulate, whereas high concentrations inhibit secretion. The majority of the review will focus on the central role of cystic fibrosis transmembrane conductance regulator (CFTR), a critical protein in the regulation of ductal secretion, in the pathogenesis of acute pancreatitis which is highlighted by numerous investigations. Downregulation of CFTR expression results in increased severity of acute pancreatitis in mice. Furthermore, human genetic studies have demonstrated statistically significant association of CFTR mutations with acute recurrent pancreatitis. Overall, the data support the involvement of pancreatic ducts in the pathogenesis of acute pancreatitis. Copyright © 2015 IAP and EPC. Published by Elsevier B.V. All rights reserved.

  1. Prevalence of amblyopia risk factors in congenital nasolacrimal duct obstruction.

    Science.gov (United States)

    Matta, Noelle S; Singman, Eric L; Silbert, David I

    2010-10-01

    To determine whether children who present to an ophthalmology practice for nasolacrimal duct obstruction are more likely to have risk factors for amblyopia. A retrospective chart review was conducted on all patients presenting to a pediatric oculoplastic specialist between January 1, 2001, and August 8, 2009. The review yielded 402 pediatric patients diagnosed with nasolacrimal duct obstruction. Children were classified as having amblyopia risk factors on the basis of the American Association for Pediatric Ophthalmology and Strabismus referral criteria. Of 402 patients with nasolacrimal duct obstruction, 100% received a cyclopegic refraction. Of these, 88 patients (22%) were found to have amblyogenic risk factors; 65 of these patients had amblyogenic refractive errors, 9 had strabismus, and 14 had a combination of the two. Of the 88 patients with amblyopia risk factors, 11% had a family history of strabismus compared with 9% of patients without amblyopia risk factors, which was not a statistically significant difference. There was no other significant pathology found on the dilated fundus examinations except for one child known to have persistent hyperplastic primary vitreous and a congenital cataract and one child with previously known CHARGE syndrome before the examination for nasolacrimal duct obstruction. Children who present with nasolacromal duct obstructions seem to be more likely to have amblyopia risk factors and therefore should have a full comprehensive pediatric ophthalmological examination, including cycloplegic refraction. Copyright © 2010 American Association for Pediatric Ophthalmology and Strabismus. Published by Mosby, Inc. All rights reserved.

  2. Salivary duct carcinoma.

    Science.gov (United States)

    D'heygere, Emmanuel; Meulemans, Jeroen; Vander Poorten, Vincent

    2018-01-25

    The review puts new information on geno- and phenotype of salivary duct carcinoma (SDC) in the perspective of the updated 2017 WHO classification. The proportion of SDC is increasing. This may be because of a true rise in incidence, but certainly to better diagnostic tests and changed WHO definitions. In this light, a substantial proportion of carcinoma expleomorphic adenoma is now attributed to the category of SDC. 'Low-grade SDC' and 'SDC in-situ' of the former WHO classification, are now named low-grade and high-grade intraductal carcinoma (IDC), respectively. Recent series quantify biologic aggressiveness: perineural growth, vascular invasion, and extracapsular extension in lymph node metastasis are each observed in two out of three patients with SDC. Most patients die within 3 years, but once 5-year disease-free survival is reached, further disease activity is exceptional. The typical molecular biological profile with high human epidermal growth factor receptor 2 and androgen receptor expression is increasingly successfully exploited in clinical trials for advanced SDC. The aggressive SDC is increasingly diagnosed. Despite intensive combined surgery and radiation therapy, many patients recur, for whom new bullets, targeting the molecular biological mechanisms, are the subject of ongoing clinical trials.

  3. Duct Leakage Repeatability Testing

    Energy Technology Data Exchange (ETDEWEB)

    Walker, Iain [Lawrence Berkeley National Lab. (LBNL), Berkeley, CA (United States); Sherman, Max [Lawrence Berkeley National Lab. (LBNL), Berkeley, CA (United States)

    2014-01-01

    Duct leakage often needs to be measured to demonstrate compliance with requirements or to determine energy or Indoor Air Quality (IAQ) impacts. Testing is often done using standards such as ASTM E1554 (ASTM 2013) or California Title 24 (California Energy Commission 2013 & 2013b), but there are several choices of methods available within the accepted standards. Determining which method to use or not use requires an evaluation of those methods in the context of the particular needs. Three factors that are important considerations are the cost of the measurement, the accuracy of the measurement and the repeatability of the measurement. The purpose of this report is to evaluate the repeatability of the three most significant measurement techniques using data from the literature and recently obtained field data. We will also briefly discuss the first two factors. The main question to be answered by this study is to determine if differences in the repeatability of these tests methods is sufficient to indicate that any of these methods is so poor that it should be excluded from consideration as an allowed procedure in codes and standards.

  4. Mirizzi syndrome: A sonographic diagnosis

    International Nuclear Information System (INIS)

    Tscholakoff, D.; Salomonowitz, E.; Czembirek, H.; Leitner, H.; Haller, J.; Wittich, G.; Vienna Univ.

    1984-01-01

    The ultrasound appearances of 11 patients with operatively confirmed Mirizzi syndrome have been analysed. The trio 'dilated intrahepatic bile ducts, concretions in the neighbourhood of the dilated common hepatic duct with a normal distal duct' permit the diagnosis of the Mirizzi syndrome with considerable certainty. In five patients these features were found by sonography and no other diagnostic procedure was necessary. In six patients, ERC was carried out in order to evaluate the distal common bile duct. In one case PTC was carried out, since the liver hilum could not be seen on sonography. (orig.) [de

  5. Intrapleural rupture of a cystic thymoma.

    Science.gov (United States)

    Haniuda, M; Numanami, H; Kondo, R; Kurai, M; Takashima, S; Amano, J

    2000-10-01

    Although cystic degeneration of a thymoma is not uncommon, rupture of a cystic thymoma is rare. The authors report a patient with sudden chest pain and dyspnea due to rupture of a cystic thymoma into the right pleural space.

  6. Lithotripsy of Pancreatic Stones in a Patient with Cystic Fibrosis: Successful Treatment of Abdominal Pain

    Directory of Open Access Journals (Sweden)

    AA Weiss

    1992-01-01

    Full Text Available Endoscopic pancreatic sphincteroromy and removal of pancreatic stones has been helpful in selected cases of patients with chronic pancreatitis. This article reports the case of an 18-year-old native Indian woman with cystic fibrosis who was experiencing pain related to pancreatitis, complicated by pancreatic duct stricture and lithiasis. Subsequent dilation of the pancreatic stricture and lithotripsy of the pancreatic ductal stones successfully eliminated the abdominal pain

  7. Lithotripsy of Pancreatic Stones in a Patient with Cystic Fibrosis: Successful Treatment of Abdominal Pain

    OpenAIRE

    Weiss, AA; Greig, JM; Fache, S

    1992-01-01

    Endoscopic pancreatic sphincteroromy and removal of pancreatic stones has been helpful in selected cases of patients with chronic pancreatitis. This article reports the case of an 18-year-old native Indian woman with cystic fibrosis who was experiencing pain related to pancreatitis, complicated by pancreatic duct stricture and lithiasis. Subsequent dilation of the pancreatic stricture and lithotripsy of the pancreatic ductal stones successfully eliminated the abdominal pain

  8. [News in cystic fibrosis].

    Science.gov (United States)

    Delaisi, B

    2013-08-01

    The improvement over the last two decades in the treatment of cystic fibrosis led to an increase in life expectancy approaching 40 years at birth. Logically, the population of adult patients has been increasing and is currently 50% of patients followed in France. These therapeutic advances have justified the establishment in 2003 of a generalized neonatal screening for cystic fibrosis. The latest data of this screening show an incidence of CF of 1/5359 live births, far below the incidence of 1/2500 which was widely accepted twenty years ago. The performance of this screening is currently based on the dosage of trypsin immuno reactive, followed in case of exceeding the threshold of a search of the 30 most common mutations, can detect around 96% of 150 to 200 CF cases every year. Therefore, the possibility of a false negative of the screening cannot be excluded and evocative symptoms of cystic fibrosis, even for children born after 2003, will lead to prescribe a sweat test. While treatments available so far goal consequences of cystic fibrosis, a new therapeutic class to correct the functional defect of the mutated protein, called CFTR modulators, is emerging. Ivacaftor, leader of this new class, belonging to the category of "CFTR potentiator" got its access on the market in September 2012 for patients carrying the G551D mutation. New other molecules, named "CFTR correctors" which can have synergistic effect with ivacaftor and concern patients carrying the most common mutation--DF 508--are under development. Copyright © 2013. Published by Elsevier Masson SAS.

  9. The Effects of Hydro-alcoholic Extract of Raspberry Fruit on Ovarian follicles and serum parameters in Poly Cystic Ovary Syndrome-Induced Rat

    Directory of Open Access Journals (Sweden)

    mohamad Nabiuni

    2015-02-01

    Full Text Available Background & aim: Polycystic Ovary Syndrome is an endocrine disorder leading to lack of ovulation and has inflammatory aspects. Raspberry extract, with possession of bioactive compounds such as Anthocyanins and polyphenols, have anti-inflammatory properties. In this study, the anti-inflammatory effects of raspberry extract on the development process of ovarian follicles and hormones level of pituitary, ovarian, and liver in rats suffering from PCOS were examined. Methods: In the present experimental study, 120 adult Wistar rats were divided into three groups of control, PCOS, and treated with raspberry. The induction of syndrome was done by 2mg Estradiol valerate. After a period of 60 days, 100, 150 and 200 mg/kg BW raspberry extract was injected intraperitoneally. Control group received no injection. Ten days after the last injection, all groups were anesthetized by chloroform and the serum and ovary of all groups were collected in order to investigate the histological and serologic changes. Data obtained were analyzed by using one-way ANOVA. Results: Raspberry-treated group showed a significant decrease in the hormones rate of testosterone, estradiol, LH, and CRP compared with PCOS (p<0.05, whereas a significant increase was observed in the serum level of Progesterone and FSH (p<0.05. A significant increase was observed in the number of corpus luteum and granulosa layer thickness in the raspberry-treated group compared with PCOS (p<0.05. Conclusion: The results demonstrated that the antioxidant and anti-inflammatory effects of raspberry extract was effective on regulating the hormones, CRPand improving of cysts which led to the initiation of ovulation.

  10. Prevalence, Diagnosis and Management of Pancreatic Cystic Neoplasms: Current Status and Future Directions

    Science.gov (United States)

    Farrell, James J.

    2015-01-01

    Cystic neoplasms of the pancreas are found with increasing prevalence, especially in elderly asymptomatic individuals. Although the overall risk of malignancy is very low, the presence of these pancreatic cysts is associated with a large degree of anxiety and further medical investigation due to concerns about malignancy. This review discusses the different cystic neoplasms of the pancreas and reports diagnostic strategies based on clinical features and imaging data. Surgical and nonsurgical management of the most common cystic neoplasms, based on the recently revised Sendai guidelines, is also discussed, with special reference to intraductal papillary mucinous neoplasm (IPMN; particularly the branch duct variant), which is the lesion most frequently identified incidentally. IPMN pathology, its risk for development into pancreatic ductal adenocarcinoma, the pros and cons of current guidelines for management, and the potential role of endoscopic ultrasound in determining cancer risk are discussed. Finally, surgical treatment, strategies for surveillance of pancreatic cysts, and possible future directions are discussed. PMID:26343068

  11. Defining DIOS and Constipation in Cystic Fibrosis With a Multicentre Study on the Incidence, Characteristics, and Treatment of DIOS

    NARCIS (Netherlands)

    Houwen, Roderick H.; van der Doef, Hubert P.; Sermet, Isabelle; Munck, Anne; Hauser, Bruno; Walkowiak, Jaroslaw; Robberecht, Eddy; Colombo, Carla; Sinaasappel, Maarten; Wilschanski, Michael

    Objectives: Various definitions for distal intestinal obstruction syndrome (DIOS), meconium ileus equivalent, and constipation in patients with Cystic fibrosis (CF) are used. However, an unequivocal definition for DIOS, meconium ileus equivalent, and constipation is preferred. The aims of this study

  12. Percutaneous transhepatic biliary drainage through the normal duct in patients with post-operative bile leakage

    International Nuclear Information System (INIS)

    Lee, Hyun; Kim, Young Hwan; Kim, Yong Joo

    2004-01-01

    To evaluate the technical feasibility and clinical efficacy of percutaneous transhepatic biliary drainage (PTBD) through the normal duct in patients with post-operative bile leakage. From January 1998 to December 2003, fourteen patients (male: 12, female: 2, mean age: 56) with biliary leak after laparoscopic cholecystectomy (n = 5), T-tube removal (n = 5), choledochojejunostomy due to small bowel perforation (n = 1), right lobectomy (n = 1), laparoscopic adrenalectomy (n = 1), and subtotal gastrectomy (n = 1) were treated by means of PTBD; this was performed with the two-step approach. The central bile duct was cannulated using a 21-G Chiba needle to map the intrahepatic biliary tree. An 8.5-F drainage catheter tip was positioned at the CBD after puncturing peripheral bile duct with an additional Chiba needle. We evaluated the technical feasibility, the procedure-related complications, clinical efficacy and the duration of catheter placement. PTBD of the normal duct with the two-step approach was successful in all but two cases. In these two cases, the two-step approach was failed due to the rapid disappearance of the targeted peripheral duct, and this was the result caused by biloportal fistula. PTBD was performed through the central bile duct in one patient, and through the remnant cystic duct in one patient. There were no procedure-related complications except for mild abdominal pain in seven patients. Bile leakage was demonstrated on cholangiogram in 10 of 14 patients; this occurred at the T-tube exit site (n = 4), cystic duct stump (n = 2), choledochojejunostomy site (n = 1), resection margin of liver (n = 1), caudate lobe (n = 1), and GB bed (n = 1). In 13 patients, the biliary leak stopped after drainage (mean duration: 32.1 days). In one patient, surgical management was performed one day after PTBD due to the excessive amount of bile leakage. PTBD is a technically feasible and clinically efficacious treatment for post-operative bile leakage, and it can

  13. Global impact of bronchiectasis and cystic fibrosis

    Directory of Open Access Journals (Sweden)

    Margarida Redondo

    2016-09-01

    To understand variation in the aetiology, microbiology and burden of bronchiectasis and cystic fibrosis across different global healthcare systems.; Bronchiectasis is the term used to refer to dilatation of the bronchi that is usually permanent and is associated with a clinical syndrome of cough, sputum production and recurrent respiratory infections. It can be caused by a range of inherited and acquired disorders, or may be idiopathic in nature. The most well recognised inherited disorder in Western countries is cystic fibrosis (CF, an autosomal recessive condition that leads to progressive bronchiectasis, bacterial infection and premature mortality. Both bronchiectasis due to CF and bronchiectasis due to other conditions are placing an increasing burden on healthcare systems internationally. Treatments for CF are becoming more effective leading to more adult patients with complex healthcare needs. Bronchiectasis not due to CF is becoming increasingly recognised, particularly in the elderly population. Recognition is important and can lead to identification of the underlying cause, appropriate treatment and improved quality of life. The disease is highly diverse in its presentation, requiring all respiratory physicians to have knowledge of the different “bronchiectasis syndromes”. The most common aetiologies and presenting syndromes vary depending on geography, with nontuberculous mycobacterial disease predominating in some parts of North America, post-infectious and idiopathic disease predominating in Western Europe, and post-tuberculosis bronchiectasis dominating in South Asia and Eastern Europe. Ongoing global collaborative studies will greatly advance our understanding of the international impact of bronchiectasis and CF.

  14. MicroRNAs in the pathogenesis of cystic kidney disease.

    Science.gov (United States)

    Phua, Yu Leng; Ho, Jacqueline

    2015-04-01

    Cystic kidney diseases are common renal disorders characterized by the formation of fluid-filled epithelial cysts in the kidneys. The progressive growth and expansion of the renal cysts replace existing renal tissue within the renal parenchyma, leading to reduced renal function. While several genes have been identified in association with inherited causes of cystic kidney disease, the molecular mechanisms that regulate these genes in the context of post-transcriptional regulation are still poorly understood. There is increasing evidence that microRNA (miRNA) dysregulation is associated with the pathogenesis of cystic kidney disease. In this review, recent studies that implicate dysregulation of miRNA expression in cystogenesis will be discussed. The relationship of specific miRNAs, such as the miR-17∼92 cluster and cystic kidney disease, miR-92a and von Hippel-Lindau syndrome, and alterations in LIN28-LET7 expression in Wilms tumor will be explored. At present, there are no specific treatments available for patients with cystic kidney disease. Understanding and identifying specific miRNAs involved in the pathogenesis of these disorders may have the potential to lead to the development of novel therapies and biomarkers.

  15. [Papillary Carcinoma Arising in Thyroglossal Duct Cyst in the Right Lateral Neck].

    Science.gov (United States)

    Wakasa, Yusuke; Oishi, Susumu; Nara, Masaki; Nozaki, Tsuyoshi; Yoshihara, Shuichi; Tateoka, Hiroshi

    2017-11-01

    A 47-year-old woman was admitted to our institution with the chief complaint of a right cervical mass. Imaging examination findings showed a cystic mass of 25mm with a nodular lesion in the right cervical region. Therefore, we performed extirpation of the right cervical cystic mass to allow diagnosis of the lesion. The histopathological findings showed a partial thyroid tissue on the cyst wall covered with glandular epithelium or metaplastic squamous epithelium, and tumor cells proliferating in the papillary form. Considering the histopathological evidence of the characteristic epithelium of the thyroglossal duct cyst, the potential carcinogenesis from the remnant thyroid tissues, and the absence of primary tumor in the thyroid gland, the patient was diagnosed with thyroid papillary carcinoma arising from the thyroglossal duct cyst in the right lateral cervical region. We found recurrence of the right cervical lymph node at 1 year and 5 months after the initial operation. Thus, we performed dissection of the right cervical lymph nodes. Two years and 10 months after the operation, neither recurrence nor metastasis have been observed. It was suggested that, thyroid papillary carcinoma arising from the thyroglossal duct cyst should be taken into consideration when a lateral cervical mass lesion is found.

  16. Noninvasive study of anatomic variations of the bile and pancreatic duct using magnetic resonance cholangiopancreatography; Estudio no invasivo de variantes anatomicas de la via biliar y pancreatica mediante colangiopancreatografia por resonancia magnetica (CPRM)

    Energy Technology Data Exchange (ETDEWEB)

    Fernandez, E.; Falco, J.; Campo, R.; Martin, J.; Brullet, E. [SDI-UDIAT Corporacio Sanitaria Parc Tauli. Sabadell (Spain); Espinos, J. [Hospital Mutua de Tarrasa (Spain)

    1999-07-01

    To identify anatomic variations of the bile duct and pancreatic duct and papillary anomalies by means of magnetic resonance cholangiopancreatography (MRCP) and determine their correlation with endoscopic retrograde cholangiopancreatography (ERCP) findings. Eighty-five patients were selected by means of a prospective study comparing MRCP and ERCP. Coronal and axial HASTE images and coronal and oblique coronal RARE images were acquired in all the patients. Four of the studies (6%) were excluded because of poor technical quality. Anatomic variations were observed in 26 cases (30.5%), including trifurcation (n=7; 27%), right hepatic duct draining into left hepatic duct (n=2, 7.7%), right hepatic duct draining into common bile duct (n=4; 15.4%), extrahepatic confluence (n=2; 7.7%), medial cystic duct (n=2; 7.7%), parallel cystic duct (n=3; 11.5%), juxtapapillary duodenal diverticulum (n=3; 11.5%) and pancreas divisum (n=3; 11.5%). A good correlation was observed between the MRCP and ERCP findings. The introduction of MRCP into the noninvasive study of biliary disease may be useful in the detection of anatomic variations relevant to laparoscopic surgery and other endoscopic and interventional techniques. (Author) 11 refs.

  17. Clinical significance of magnetic resonance cholangiopancreatography for the diagnosis of cystic tumor of the pancreas compared with endoscopic retrograde cholangiopancreatography and computed tomography

    Energy Technology Data Exchange (ETDEWEB)

    Mera, Kiyomi; Tajiri, Hisao; Muto, Manabu [National Cancer Center, Kashiwa, Chiba (Japan). Hospital East] [and others

    1999-06-01

    Cystic tumor of the pancreas has been investigated by a variety of imaging techniques. Magnetic resonance cholangiopancreatography (MRCP) is being widely used as a non-invasive diagnostic modality for investigation of the biliary tree and pancreatic duct system. The purpose of this study was to compare MRCP images with those of endoscopic retrograde cholangiopancreatography (ERCP) and computed tomography (CT) in order to clarify the diagnostic efficacy of MRCP for cystic tumor of the pancreas. We retrospectively studied 15 patients with cystic tumor of the pancreas that had been surgically resected and histopathologically confirmed. There were five cases of intraductal papillary adenocarcinoma, five of intraductal papillary adenoma, two of serous cyst adenoma, two of retention cyst associated with invasive ductal adenocarcinoma and one of solid cystic tumor. In all cases MRCP correctly identified the main pancreatic duct (MPD) and showed the entire cystic tumor and the communication between the tumor and the MPD. On the other hand, the detection rate by ERCP of the cystic tumor and the communication between the cystic tumor and the MPD was only 60%. Although the detection rates by CT for the septum and solid components inside the cystic tumor were 100 and 90.0%, respectively, those of MRCP for each were 58.3 and 20.0%. MRCP is capable of providing diagnostic information superior to ERCP for the diagnosis of cystic tumor of the pancreas. Although MRCP may provide complementary information about the whole lesion of interest, the characteristic internal features of cystic tumor of the pancreas should be carefully diagnosed in combination with CT. (author)

  18. Thoracic duct lymphography by subcutaneous contrast agent ...

    African Journals Online (AJOL)

    A second lymphography revealed a collateral thoracic duct that was not detected during the first lymphography. The collateral duct was ligated and chylothorax was resolved after the second surgery. The lymphography applied in this study was minimally-invasive and easily provided images of the thoracic duct in a dog with ...

  19. Network for Early Onset Cystic Kidney Diseases-A Comprehensive Multidisciplinary Approach to Hereditary Cystic Kidney Diseases in Childhood.

    Science.gov (United States)

    König, Jens Christian; Titieni, Andrea; Konrad, Martin

    2018-01-01

    Hereditary cystic kidney diseases comprise a complex group of genetic disorders representing one of the most common causes of end-stage renal failure in childhood. The main representatives are autosomal recessive polycystic kidney disease, nephronophthisis, Bardet-Biedl syndrome, and hepatocyte nuclear factor-1beta nephropathy. Within the last years, genetic efforts have brought tremendous progress for the molecular understanding of hereditary cystic kidney diseases identifying more than 70 genes. Yet, genetic heterogeneity, phenotypic variability, a lack of reliable genotype-phenotype correlations and the absence of disease-specific biomarkers remain major challenges for physicians treating children with cystic kidney diseases. To tackle these challenges comprehensive scientific approaches are urgently needed that match the ongoing "revolution" in genetics and molecular biology with an improved efficacy of clinical data collection. Network for early onset cystic kidney diseases (NEOCYST) is a multidisciplinary, multicenter collaborative combining a detailed collection of clinical data with translational scientific approaches addressing the genetic, molecular, and functional background of hereditary cystic kidney diseases. Consisting of seven work packages, including an international registry as well as a biobank, NEOCYST is not only dedicated to current scientific questions, but also provides a platform for longitudinal clinical surveillance and provides precious sources for high-quality research projects and future clinical trials. Funded by the German Federal Government, the NEOCYST collaborative started in February 2016. Here, we would like to introduce the rationale, design, and objectives of the network followed by a short overview on the current state of progress.

  20. Network for Early Onset Cystic Kidney Diseases—A Comprehensive Multidisciplinary Approach to Hereditary Cystic Kidney Diseases in Childhood

    Directory of Open Access Journals (Sweden)

    Jens Christian König

    2018-02-01

    Full Text Available Hereditary cystic kidney diseases comprise a complex group of genetic disorders representing one of the most common causes of end-stage renal failure in childhood. The main representatives are autosomal recessive polycystic kidney disease, nephronophthisis, Bardet–Biedl syndrome, and hepatocyte nuclear factor-1beta nephropathy. Within the last years, genetic efforts have brought tremendous progress for the molecular understanding of hereditary cystic kidney diseases identifying more than 70 genes. Yet, genetic heterogeneity, phenotypic variability, a lack of reliable genotype–phenotype correlations and the absence of disease-specific biomarkers remain major challenges for physicians treating children with cystic kidney diseases. To tackle these challenges comprehensive scientific approaches are urgently needed that match the ongoing “revolution” in genetics and molecular biology with an improved efficacy of clinical data collection. Network for early onset cystic kidney diseases (NEOCYST is a multidisciplinary, multicenter collaborative combining a detailed collection of clinical data with translational scientific approaches addressing the genetic, molecular, and functional background of hereditary cystic kidney diseases. Consisting of seven work packages, including an international registry as well as a biobank, NEOCYST is not only dedicated to current scientific questions, but also provides a platform for longitudinal clinical surveillance and provides precious sources for high-quality research projects and future clinical trials. Funded by the German Federal Government, the NEOCYST collaborative started in February 2016. Here, we would like to introduce the rationale, design, and objectives of the network followed by a short overview on the current state of progress.

  1. [Cystic fibrosis in 2008].

    Science.gov (United States)

    Durieu, I; Josserand, R Nove

    2008-11-01

    To describe the epidemiological, physiopathological, clinical and therapeutic knowledge concerning cystic fibrosis (CF). Important modifications in the health organization of the care concerning this orphan disease have been implemented in France. The life expectancy has dramatically increased, as well as the knowledge concerning the pathological structure and function of the CFTR gene and protein. This will lead to the development of emerging drug treatments for this lethal disease. The life expectancy is predicted to exceed 40 years for children born in the 2000s. As a result, there has been a tremendous growth of the adult population that reached 40% of the overall approximately 5000 patients included in the CF French registry (Observatoire National de la Mucoviscidose). Lung disease remains the primary cause of morbidity and mortality. The characteristic phenotypic presentation associates bronchial and rhinosinusal symptoms, pancreatic insufficiency and liver disease. Bronchial damage leads to progressive chronic respiratory insufficiency. Diabetes mellitus and osteoporosis frequently appears in adulthood. Neonatal screening has been implemented in France since 2002. It will prevent delayed diagnosis and its deleterious consequences. Some atypical cases of CF presenting only with one or two organ system involvement can be diagnosed in adulthood. Isolated chronic rhinosinusitis, bronchiectasis, congenital bilateral absence of vas deferens, recurrent pancreatitis, allergic bronchopulmonary aspergillosis, and some case of cholangitis may so revealed late form of cystic fibrosis. The health care is organized in cystic fibrosis centres. Despite gene discovery, treatment still remains symptomatic, based on intensive pulmonary and nutritional treatments. Challenges for new treatments are to correct the basic defect, either by gene therapy or by pharmacological modulation of the abnormal physiological processes.

  2. Childhood abdominal cystic lymphangioma

    Energy Technology Data Exchange (ETDEWEB)

    Konen, Osnat; Rathaus, Valeria; Shapiro, Myra [Department of Diagnostic Imaging, Meir General Hospital, Sapir Medical Centre, Kfar Saba (Israel); Dlugy, Elena [Department of Paediatric Surgery, Schneider Medical Centre, Sackler School of Medicine, Tel-Aviv University (Israel); Freud, Enrique [Department of Paediatric Surgery, Sapir Medical Centre, Sackler School of Medicine, Tel-Aviv University (Israel); Kessler, Ada [Department of Diagnostic Imaging, Sourasky Medical Centre, Tel-Aviv (Israel); Horev, Gadi [Department of Diagnostic Imaging, Schneider Medical Centre, Tel-Aviv (Israel)

    2002-02-01

    Background: Abdominal lymphangioma is a rare benign congenital malformation of the mesenteric and/or retroperitoneal lymphatics. Clinical presentation is variable and may be misleading; therefore, complex imaging studies are necessary in the evaluation of this condition. US and CT have a major role in the correct preoperative diagnosis and provide important information regarding location, size, adjacent organ involvement, and expected complications. Objective: To evaluate the clinical and imaging findings of seven children with proven abdominal cystic lymphangioma. Materials and methods: Clinical and imaging files of seven children with pathologically proven abdominal lymphangioma, from three university hospitals, were retrospectively evaluated. Patient's ages ranged from 1 day to 6 years (mean, 2.2 years). Symptoms and signs included evidence of inflammation, abnormal prenatal US findings, chronic abdominal pain, haemorrhage following trauma, clinical signs of intestinal obstruction, and abdominal distension with lower extremities lymphoedema. Plain films of five patients, US of six patients and CT of five patients were reviewed. Sequential imaging examinations were available in two cases. Results: Abdominal plain films showed displacement of bowel loops by a soft tissue mass in five of six patients, two of them with dilatation of small bowel loops. US revealed an abdominal multiloculated septated cystic mass in five of six cases and a single pelvic cyst in one which changed in appearance over 2 months. Ascites was present in three cases. CT demonstrated a septated cystic mass of variable sizes in all available five cases. Sequential US and CT examinations in two patients showed progressive enlargement of the masses, increase of fluid echogenicity, and thickening of walls or septa in both cases, with multiplication of septa in one case. At surgery, mesenteric lymphangioma was found in five patients and retroperitoneal lymphangioma in the other two

  3. Imaging from cystic fibrosis

    International Nuclear Information System (INIS)

    Schmidt, H.; Posselt, H.G.

    2008-01-01

    Cystic fibrosis (CF) is the most frequent metabolic disorder with autosomal recessive inheritance in the Caucasian population. The gene defect is located on the long arm of chromosome 7. In Germany today, the actual median survival is 37 years. The genetic defect caused by chloride anion disturbances affects multiple body systems but the morbidity and mortality is due to lung disease. The secretion of highly viscous mucus promotes viral and bacterial pulmonary infections leading to airway obstruction and consecutive destruction of the lung parenchyma. This article will review and discuss both the clinical aspects of the disease and the diagnostic methods, referring in particular to new imaging strategies. (orig.)

  4. Calprotectin in cystic fibrosis.

    Science.gov (United States)

    Rumman, Nisreen; Sultan, Mutaz; El-Chammas, Khalil; Goh, Vi; Salzman, Nita; Quintero, Diana; Werlin, Steven

    2014-05-29

    There is increasing evidence that intestinal inflammation plays a major role in gastrointestinal symptoms in cystic fibrosis (CF). Fecal calprotectin is a marker that is elevated in several gastrointestinal inflammatory diseases, but little is known about its value in CF. We aimed to look for associations of elevated fecal calprotectin among CF patients and whether its level correlates with the clinical manifestations of CF. A single stool specimen was collected from 62 patients with CF. Fecal calprotectin was measured using the commercially available ELISA kits (PhiCal™ test). Clinical data were collected from patients' records and CF registry. There were no significant differences between CF patients with normal and abnormal fecal calprotectin levels. However, patients who were not receiving inhaled antibiotics had higher fecal calprotectin levels than those who were. Elevated fecal calprotectin may not accurately predict intestinal inflammation in CF. However, the fact that it was elevated in both pancreatic sufficient and insufficient groups supports the concept of "cystic fibrosis enteropathy" regardless of the pancreatic status.

  5. Plasma transport in magnetic duct filter

    International Nuclear Information System (INIS)

    Zhang Tao; Chu, Paul K; Fu, Ricky K Y; Brown, Ian G

    2002-01-01

    A cathodic arc plasma source equipped with a curved magnetic duct to filter macro-particles was used to study plasma transport through the duct. The optimal duct bias, at which the magnetic duct produces the maximum plasma output, for titanium cathodic arc plasma at 50, 100 and 150 A arc current was determined and the parametric effects of the arc current and guiding magnetic field on the optimal duct bias were investigated. The optimal bias decreased as the guiding magnetic field increased from 100 to 400 G and was almost independent of the guiding magnetic field when it was between 400 and 600 G, the upper limit for our equipment. The optimal duct bias at 400 G guiding magnetic field decreased with increasing arc current. Our results indicate that the optimal duct bias is related not only to the structure of the plasma source, but is also influenced by many other factors including cathode material and other plasma properties

  6. Early bladder outlet obstruction in fetal lambs induces renal dysplasia and the prune-belly syndrome.

    Science.gov (United States)

    Gonzalez, R; Reinberg, Y; Burke, B; Wells, T; Vernier, R L

    1990-03-01

    A model of posterior urethral valves in fetal lambs was developed in order to evaluate the effect of intrauterine urinary obstruction on the developing kidney. Complete urethral obstruction was induced in five fetal lambs at 43 to 45 days of gestation. Two control fetal lambs underwent sham operations. At full term (140 days), two of the five experimental lambs and both control lambs were available for postmortem examination. Results of gross and histological examination of the control lambs were normal. In contrast, the kidneys of the experimental lambs were markedly asymmetrical in size. Histological examination of the kidneys in experimental lambs showed cystic dilatation of the collecting ducts and occasional cystic dilatation of Bowman's spaces, features compatible with obstruction. Also noted were peripheral cortical cysts and primitive tubules lined with cuboidal epithelium and surrounded by fibromuscular collarettes, characteristic of renal dysplasia. One of the infant lambs had many characteristics of the prune-belly syndrome, including a wrinkled, markedly distended abdomen, deficient abdominal wall musculature, flared chest wall, limb deformities, and undescended testes. These results suggest that early in utero urethral obstruction (at the beginning of the second third of gestation) causes renal dysplasia. The results also support the hypothesis that the prune-belly syndrome results from abdominal distention that occurs early in gestation.

  7. Cholangiocarcinoma of intrahepatic bile ducts with disseminated metastases in an African lion (Panthera leo).

    Science.gov (United States)

    Lepri, Elvio; Sforna, Monica; Brachelente, Chiara; Chiara, Brachelente; Vitellozzi, Giovanni; Giovanni, Vitellozzi

    2013-06-01

    A cholangiocarcinoma is reported in an 18-yr-old, female African lion (Panthera leo). The primary tumor consisted of multifocal to coalescing, hepatic, white-yellow masses distributed throughout the liver lobes. Metastases were present in regional lymph nodes, peritoneal surface, and lungs. Histologically, the tumor was characterized by a tubular pattern with alcian- and periodic acid-Schiff-positive secretory material in cystic spaces. The neoplastic cells were positive to broad-spectrum cytokeratins. Histochemical and immunohistochemical stains were consistent with bile duct carcinoma. Biliary tumors arising from the gallbladder have been reported in lions. However, to the authors' knowledge, this is the first case of intrahepatic bile duct carcinoma reported in an African lion.

  8. Gastrointestinal Manifestations of Cystic Fibrosis

    Science.gov (United States)

    2016-01-01

    Cystic fibrosis has historically been considered a pulmonary disease, but with the increasing life expectancy of these patients, gastrointestinal manifestations are becoming more important. Furthermore, nutritional status is closely linked to pulmonary function and, thus, overall mortality. This article discusses gastrointestinal manifestations (which involve nutritional, pancreatic, hepatobiliary, and, in particular, gastrointestinal tract issues) of cystic fibrosis as well as management of the disease. In addition, the article discusses studies that have been critical to our understanding of gastrointestinal manifestations of cystic fibrosis. PMID:27330503

  9. Deletion of hepatocyte nuclear factor-1-beta in an infant with prune belly syndrome.

    Science.gov (United States)

    Haeri, Sina; Devers, Patricia L; Kaiser-Rogers, Kathleen A; Moylan, Vincent J; Torchia, Beth S; Horton, Amanda L; Wolfe, Honor M; Aylsworth, Arthur S

    2010-08-01

    Prune belly syndrome is a rare congenital disorder characterized by deficiency of abdominal wall muscles, cryptorchidism, and urinary tract anomalies. We have had the opportunity to study a baby with prune belly syndrome associated with an apparently de novo 1.3-megabase interstitial 17q12 microdeletion that includes the hepatocyte nuclear factor-1-beta gene at 17q12. One previous patient, an adult, has been reported with prune belly syndrome and a hepatocyte nuclear factor-1-beta microdeletion. Hepatocyte nuclear factor-1-beta is a widely expressed transcription factor that regulates tissue-specific gene expression and is expressed in numerous tissues including mesonephric duct derivatives, the renal tubule of the metanephros, and the developing prostate of the mouse. Mutations in hepatocyte nuclear factor-1-beta cause the "renal cysts and diabetes syndrome," isolated renal cystic dysplasia, and a variety of other malformations. Based on its expression pattern and the observation of two affected cases, we propose that haploinsufficiency of hepatocyte nuclear factor-1-beta may be causally related to the production of the prune belly syndrome phenotype through a mechanism of prostatic and ureteral hypoplasia that results in severe obstructive uropathy with urinary tract and abdominal distension. Copyright Thieme Medical Publishers.

  10. Molecular Diagnosis of Cystic Fibrosis.

    Science.gov (United States)

    Deignan, Joshua L; Grody, Wayne W

    2016-01-01

    This unit describes a recommended approach to identifying causal genetic variants in an individual suspected of having cystic fibrosis. An introduction to the genetics and clinical presentation of cystic fibrosis is initially presented, followed by a description of the two main strategies used in the molecular diagnosis of cystic fibrosis: (1) an initial targeted variant panel used to detect only the most common cystic fibrosis-causing variants in the CFTR gene, and (2) sequencing of the entire coding region of the CFTR gene to detect additional rare causal CFTR variants. Finally, the unit concludes with a discussion regarding the analytic and clinical validity of these approaches. Copyright © 2016 John Wiley & Sons, Inc.

  11. [Cystic lymphangioma of the scrotum].

    Science.gov (United States)

    Oliver Llinares, F; Roques Serradilla, J L; Ruíz Jiménez, J I; Desus Ruíz, J; Segarra Llido, V

    1980-01-01

    The authors present a case of cystic lymphangioma of the scrotum. They stress the rarity of this location. They also comment upon the embriology, clinical picture and treatment of these benign tumours.

  12. Profile of cystic fibrosis

    Directory of Open Access Journals (Sweden)

    Mona M. El-Falaki

    2014-09-01

    Full Text Available It was generally believed that Cystic fibrosis (CF is rare among Arabs; however, the few studies available from Egypt and other Arabic countries suggested the presence of many undiagnosed patients. The aim of the present study was to determine the frequency of CF patients out of the referred cases in a single referral hospital in Egypt. A total of 100 patients clinically suspected of having CF were recruited from the CF clinic of the Allergy and Pulmonology Unit, Children’s Hospital, Cairo University, Egypt, throughout a 2 year period. Sweat chloride testing was done for all patients using the Wescor macroduct system for collection of sweat. Quantitative analysis for chloride was then done by the thiocyanate colorimetric method. Patients positive for sweat chloride (⩾60 mmol/L were tested for the ΔF508 mutation using primer specific PCR for cystic fibrosis transmembrane conductance regulator (CFTR gene. Thirty-six patients (36% had a positive sweat chloride test. The main clinical presentations in patients were chronic cough in 32 (88.9%, failure to thrive in 27 (75%, steatorrhea in 24 (66.7%, and hepatobiliary involvement in 5 (13.9%. Positive consanguinity was reported in 50% of CF patients. Thirty-two patients were screened for ΔF508 mutation. Positive ΔF508 mutation was detected in 22 (68.8% patients, 8 (25% were homozygous, 14 (43.8% were heterozygous, and 10 (31.3% tested were negative. CF was diagnosed in more than third of patients suspected of having the disease on clinical grounds. This high frequency of CF among referred patients indicates that a high index of suspicion and an increasing availability of diagnostic tests lead to the identification of a higher number of affected individuals.

  13. Breakdown in Breathing: The Complexities of Cystic Fibrosis

    Science.gov (United States)

    ... Healthier Lungs in Kids Wise Choices Living with Cystic Fibrosis In between checkups, practice good self-care and ... Links What Is Cystic Fibrosis? Learning About Cystic Fibrosis NIH Cystic Fibrosis Fact Sheet Genetic and Rare Diseases Information ...

  14. Quadcopter thrust optimization with ducted-propeller

    Directory of Open Access Journals (Sweden)

    Kuantama Endrowednes

    2017-01-01

    Full Text Available In relation to quadcopter body frame model, propeller can be categorized into propeller with ducted and without ducted. This study present differences between those two using CFD (Computational Fluid Dynamics method. Both categories utilize two blade-propeller with diameter of 406 (mm. Propeller rotation generates acceleration per time unit on the volume of air. Based on the behavior of generated air velocity, ducted propeller can be modeled into three versions. The generated thrust and performance on each model were calculated to determine the best model. The use of ducted propeller increases the total weight of quadcopter and also total thrust. The influence of this modeling were analyzed in detail with variation of angular velocity propeller from 1000 (rpm to 9000 (rpm. Besides the distance between propeller tip and ducted barrier, the size of ducted is also an important part in thrust optimization and total weight minimization of quadcopter.

  15. Fibrose cística: uma abordagem clínica e nutricional Cystic fibrosis: a clinical and nutritional approach

    Directory of Open Access Journals (Sweden)

    Fernanda Ribeiro Rosa

    2008-12-01

    produce thick and viscous mucus secretions that obstruct the lungs, pancreas and bile duct. Many patients have pancreatic insufficiency which leads to malabsorption of nutrients, especially proteins and fats and to gastrointestinal complications such as rectal prolapse, intestinal obstruction syndrome, constipation and hepatic cirrhosis. Cystic fibrosis is usually diagnosed during childhood by neonatal screening programs or sweat test. Because of the multiple systems involved and the variability and chronicity of the disease, a multidisciplinary team is essential to help patients and their families understand the disease and adhere to treatment. Current cystic fibrosis therapy includes maintaining the nutritional status, clearing the airways with physiotherapy and mucolytics, preventing and treating infections with antibiotics and prescribing energy supplements, high-fat and high-protein diets, as well as minerals and fat-soluble vitamins. The purpose of this study was to present a brief literature review of the clinical and nutritional aspects of cystic fibrosis.

  16. Metabolic alkalosis with multiple salt unbalance: an atypical onset of cystic fibrosis in a child

    Directory of Open Access Journals (Sweden)

    Dimitri Poddighe

    2017-12-01

    Full Text Available Dehydration with multiple salt abnormalities is frequently encountered in the paediatric emergency department, during acute illnesses complicated by loss of body fluids. Metabolic alkalosis is not a common finding in dehydrated children. The presence of unusual electrolyte unbalance, such as metabolic alkalosis, hyponatremia, hypochloremia and hypokalemia, without evidence of renal tubular defects, is named as pseudo-Bartter syndrome. It can occur in several clinical settings and, in infancy, it is described as a potential complication of cystic fibrosis. We report a case of pseudo-Bartter syndrome representing the onset of cystic fibrosis in childhood.

  17. Cystic Leukoencephalopathy with Normocephaly

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2005-05-01

    Full Text Available A new syndrome of nonprogressive encephalopathy with normo- or microcephaly and early onset of psychomotor impairment is described in 15 children, in a report from the University of Gottingen and other centers in Europe and the US.

  18. Imaging features of unusual intracranial cystic meningiomas

    International Nuclear Information System (INIS)

    Demir, M.K.; Musluman, M.; Kilicoglu, G.; Hakan, T.; Aker, F.V.

    2007-01-01

    To describe the imaging features of unusual intracranial cystic meningiomas in infants and adults. We retrospectively reviewed the magnetic resonance and computed tomography findings for 2 female patients and 3 male patients, ranging in age from 1 to 73 years (median 41 years), with histopathologically proven cystic meningioma. Although cystic meningiomas usually appear as solid and cystic masses, they may present as a mainly multicystic lesion. The wall of a cystic part of the meningioma may include both enhancing and unenhancing areas at imaging. The cystic portion of a meningioma is hypointense on diffusion-weighted images and markedly hyperintense on corresponding apparent diffusion coefficient maps. (author)

  19. Thermal neutrons streaming in straight duct

    International Nuclear Information System (INIS)

    Jehouani, A.; Boulkheir, M.; Ichaoui, R.

    2000-01-01

    The neutron streaming in duct is due to two phenomena: a) direct propagation and b) reflection on duct wall. We have used the Monte Carlo method to evaluate the ratio of the reflected neutrons flux by the duct wall to the total flux at the exit of the duct for iron and aluminium. Ten neutrons energy groups are considered between 10 -5 eV and 10 eV. A Fortran program is developed to evaluate the neutron double differential albedo. It is shown that the two following approximations are largely justified: i) Three collisions in the duct wall are sufficient to attain the asymptotic limit of the multiscattered neutron double differential albedo ii) The points of entry and exit of the neutron in the duct wall may be considered the same for the multiscattered neutrons. For a punctual source at the mouth of the duct, we have determined the direct and the reflected part of the total thermal neutron flux at the exit of the duct for different lengths and different radius of the duct. For a punctual source, we have found that the major contribution to the total flux of neutrons at the exit is due to the neutron reflection by walls and the reflection contribution decreases when the neutron energy decreases. For a constant length of the duct, the reflected part decreases when the duct radius increases while for the disk shaped source we have found the opposite phenomena. The transmitted neutron flux distribution at the exit of the duct are determined for disk shaped source for different neutron energy and for different distance from the exit center. (author)

  20. Cystic synovial sarcomas: imaging features with clinical and histopathologic correlation

    Energy Technology Data Exchange (ETDEWEB)

    Nakanishi, Hirofumi; Araki, Nobuhito [Department of Orthopedic Surgery, Osaka Medical Center for Cancer and Cardiovascular Diseases, 1-3-3, Nakamichi, Higashinari-Ku, 537-8511, Osaka (Japan); Sawai, Yuka [Department of Radiology, Osaka Medical Center for Cancer and Cardiovascular Diseases, Osaka (Japan); Kudawara, Ikuo [Department of Orthopedic Surgery, Osaka National Hospital, Osaka (Japan); Mano, Masayuki; Ishiguro, Shingo [Department of Pathology, Osaka Medical Center for Cancer and Cardiovascular Diseases, Osaka (Japan); Ueda, Takafumi; Yoshikawa, Hideki [Department of Orthopedic Surgery, Osaka University Graduate School of Medicine, Suita, Osaka (Japan)

    2003-12-01

    To characterize the radiological and clinicopathologic features of cystic synovial sarcoma. Seven patients with primary cystic synovial sarcoma were evaluated. Computed tomography (CT) and magnetic resonance (MR) imaging were undertaken at the first presentation. The diagnosis of synovial sarcoma was made on the basis of histological examinations followed by molecular analysis. Radiological and clinicopathologic findings were reviewed. CT showed well-defined soft tissue mass without cortical bone erosion and invasion. Calcification was seen at the periphery of the mass in three cases. T2-weighted MR images showed multilocular inhomogeneous intensity mass in all cases, five of which showed fluid-fluid levels. On gross appearance, old and/or fresh hematomas were detected in six cases. In the one remaining case, microscopic hemorrhage in the cystic lumen was proven. Four cases had poorly differentiated areas. In five cases prominent hemangiopericytomatous vasculature was observed. Histologic grade was intermediate in one tumor and high in six. One case had a history of misdiagnosis for tarsal tunnel syndrome, one for lymphadenopathy, two for sciatica and two for hematoma. All cystic synovial sarcomas demonstrated multilocularity with well-circumscribed walls and internal septae. Synovial sarcoma should be taken into consideration in patients with deeply situated multicystic mass with triple signal intensity on T2-weighted MR imaging. (orig.)

  1. Intraoperative cholangiographic findings of intrahepatic duct stones

    International Nuclear Information System (INIS)

    Kim, Mi Young; Suh, Chang Hae; Choi, Hye Ran; Ahn, Byeong Yeob; Chung, Woun Kyun; Lee, Yong

    1989-01-01

    Operative cholangiography is considered essential during biliary operation in order to minimise the chance of negative exploration and to demonstrate the anatomy and pathology of intra and extrahepatic biliary tree. Although many authors have evaluated and accepted various techniques for operative cholangiography, the image of operative cholangiography is affected mainly by intrahepatic duct stones and anatomic characteristic of biliary tree. In case of intrahepatic duct stones accurate evaluation of the operative cholangiographic findings is important for a plan of further treatment including interventional procedures. We reviewed 45 operative cholangiograms of 30 patients with intrahepatic duct stones from January,1986 to September, 1988, and analysed operative cholangiographic findings with a special attention to the correlation between cholangiographic findings and the location of intrahepatic duct stones. The results were as follows: 1. Common bile duct stones were found in 72%, and generalized cholangitis was combined in 43% of patient with intrahepatic duct stones. 2. Incomplete ductal filling of contrast media was the most frequent findings of left intrahepatic duct stones in operative cholangiograms with four different cholangiogram catheters. 3. Nonfilling and partial filling were the most frequent findings; nonfilling was prevalent in right anterior and left medial ducts, and partial filling was prevalent in left medial and left lateral ducts

  2. Cystic form of paraduodenal pancreatitis (cystic dystrophy in heterotopic pancreas (CDHP)): a potential link with minor papilla abnormalities? A study in a large series

    Energy Technology Data Exchange (ETDEWEB)

    Wagner, M.; Ronot, M.; Vilgrain, V. [University Hospitals Paris Nord Val de Seine, Radiology Department, Beaujon Hospital, Assistance Publique-Hopitaux de Paris, APHP, Clichy (France); Paris Diderot University, INSERM, UMR 1149, Paris (France); Vullierme, M.P. [University Hospitals Paris Nord Val de Seine, Radiology Department, Beaujon Hospital, Assistance Publique-Hopitaux de Paris, APHP, Clichy (France); Rebours, V.; Ruszniewski, P. [Paris Diderot University, INSERM, UMR 1149, Paris (France); University Hospitals Paris Nord Val de Seine, Gastroenterology Department, Beaujon Hospital, Assistance Publique-Hopitaux de Paris, APHP, Clichy (France)

    2016-01-15

    To analyze the association between cystic dystrophy in the heterotopic pancreas (CDHP) and minor papilla abnormalities. Seventy-six patients with CDHP were retrospectively included over 14 years. Two radiologists searched for MDCT signs of CDHP (cysts and thickened intestinal wall, inflammatory changes), and minor papilla abnormalities (Santorini duct dilatation, luminal calcifications of the minor papilla). Other pancreatic abnormalities (parenchymal calcifications, main pancreatic duct dilatation) or bile duct dilatation were also analysed. CDHP was mostly located in the second part of the duodenum (71/76, 93.5 %). Median duodenal wall thickness was 20 mm (range 10-46). There were multiple cysts in 86 % (65/76, median = 3), measuring 2-60 mm. No cysts were identified in four patients (5 %). Inflammatory changes were found in 87 % (66/76). Minor papilla abnormalities were found in 37 % (28/76) and calcifications in the minor papilla without calcifications in the major papilla were only observed in three patients (4 %). Abnormalities of the pancreas and main bile duct dilatation were identified in 78 % (59/76) and 38 % (29/76). Previously described CT features were seen in most patients with CDHP. However, minor papilla abnormalities were seen in a minority of patients and, therefore, do not seem to be a predisposing factor for CDHP. (orig.)

  3. Cystic thymic diseases: CT manifestations

    Energy Technology Data Exchange (ETDEWEB)

    Song, Soon Young; Choi, Yo Won; Jeon, Eui Yong; Jeon, Seok Chol; Seo, Heung Suk; Hahm, Chang Kok [School of Medicine, Hanyang University, Seoul (Korea, Republic of)

    1995-09-15

    To describe CT findings and differential points of cystic thymic lesions. We evaluated retrospectively total 19 masses with well marginated cystic lesions at thymic area on CT scans. They were 10 teratomas, 3 congenital thymic cysts, 2 multilocular thymic cysts(associated with thymoma and myasthenia gravis in each), 2 cysts Assciated with thymic Hodgkin's lymphomas an ectopic parathyroid cyst, and an infected thymic cyst. The radiological abnormalities evaluated were thickness of the wall, presence or abscene of septa, mural nodule, solid component, calcification and fat component. All three cases of congenital thymic cysts and an ectopic parathyroid cyst appeared as thin-walled unilocular cyst with homogeneous internal density and without identifiable solid component. In multilocular thymic cyst, there were thick wall and solid components(n =2), thick internal septa and calcifications(n = 1). The cysts of teratomas manifested thick walls(n = 9), internal septa(n = 4), calcifications(n = 6), fat components(n = 4), and solid components(n = 4). Cysts in Hodgkin's diseases appeared as multilocular or unilocular and had thick wall and septa without calcification. Infected thymic cyst presented with multilocular cystic mass with identifiable wall and septa, calcification, and solid components. The thymic diseases with cystic lesion include teratomas, congenital thymic cysts, multilocular thymic cysts, parathyroid cyst, and Hodgkin's disease. Congenital thymic cyst and ectopic parathyroid cyst are thin-walled unilocular cystic lesions. Cystic lesions associated with teratoma, Hodgkin's disease, and multilocular thymic cyst are thick-walled cystic lesions with or without solid component.

  4. Computed tomography of cystic pancreatic fibrosis

    International Nuclear Information System (INIS)

    Brachlow, M.; Zaunbauer, W.; Haertel, M.

    1984-01-01

    The computer tomographic appearances of atrophic and lipomatous degeneration of the pancreas in cystic pancreatic fibrosis are described. CT exploration of the pancreas in recommended, particularly in differential diagnostic aspects of cystic fibrosis. (orig.) [de

  5. Cystic Fibrosis (CF) Respiratory Screen: Sputum

    Science.gov (United States)

    ... Staying Safe Videos for Educators Search English Español Cystic Fibrosis (CF) Respiratory Screen: Sputum KidsHealth / For Parents / Cystic Fibrosis (CF) Respiratory Screen: Sputum What's in this article? ...

  6. Human cystic echinococcosis in South Africa

    NARCIS (Netherlands)

    Mogoye, Benjamin; Menezes, Colin N.; Grobusch, Martin P.; Wahlers, Kerstin; Frean, John

    2012-01-01

    Cystic echinococcosis (CE) is caused by the tapeworm, Echinococcus granulosus. The tapeworms resides in the small intestines of canids and the lifecycle involves both intermediate and definitive hosts. Humans are accidental intermediate hosts. Cystic echinococcosis is an economically important

  7. Laparoscopic common bile duct exploration.

    Science.gov (United States)

    Shuchleib, S; Chousleb, A; Mondragon, A; Torices, E; Licona, A; Cervantes, J

    1999-07-01

    Since the introduction of laparoscopic cholecystectomy, the management of common bile duct (CBD) stones has undergone significant change. Preoperative endoscopic retrograde cholangiopancreatography (ERCP) with sphincterotomy is now routinely done in cases where the diagnosis of choledocholithiasis is suspected preoperatively, with clearance of the bile ducts before laparoscopic cholecystectomy. Intraoperative discovery of CBD stones by cholangiography represents a challenge to the surgeon, who must make a decision about when to perform laparoscopic CBD exploration, convert to open surgery, or send the patient for ERCP during the postoperative period. Because ERCP has a definite failure rate, laparoscopic CBD exploration can be a treatment option. Among 2500 laparoscopic cholecystectomies done by our group from January 1991 to June 1997, 50 patients (2%) underwent laparoscopic CBD exploration, 13 by the transcystic technique and 37 by choledocotomy, with a conversion rate of 8% and a hospital stay of 4.3 days. One patient died from complicated pancreatitis following ERCP and unsuccessful extraction of a CBD stone. We obtained our goal of a CBD free of stones in 92% of the cases. We conclude that laparoscopic CBD exploration is an effective method for treating choledocolithiasis that allows management of this pathology in one stage, although it requires advanced laparoscopic skills and adequate equipment.

  8. Measure Guideline. Sealing and Insulating Ducts in Existing Homes

    Energy Technology Data Exchange (ETDEWEB)

    Aldrich, R. [Consortium for Advanced Residential Buildings (CARB), Norwalk, CT (United States); Puttagunta, S. [Consortium for Advanced Residential Buildings (CARB), Norwalk, CT (United States)

    2011-12-01

    This document begins with a discussion on potential cost and performance benefits of duct sealing and insulating. It continues with a review of typical duct materials and components and the overall procedures for assessing and improving the duct system.

  9. Measure Guideline: Sealing and Insulating of Ducts in Existing Homes

    Energy Technology Data Exchange (ETDEWEB)

    Aldrich, R.; Puttagunta, S.

    2011-12-01

    This document begins with a discussion on potential cost and performance benefits of duct sealing and insulating. It continues with a review of typical duct materials and components and the overall procedures for assessing and improving the duct system.

  10. Cystic Lesions in Autoimmune Pancreatitis

    Directory of Open Access Journals (Sweden)

    Macarena Gompertz

    2015-11-01

    Full Text Available Autoimmune pancreatitis (AIP can be chronic or recurrent, but frequently completely reversible after steroid treatment. A cystic lesion in AIP is a rare finding, and it can mimic a pancreatic cystic neoplasm. Difficulties in an exact diagnosis interfere with treatment, and surgery cannot be avoided in some cases. We report the history of a 63-year-old male presenting with jaundice and pruritus. AIP was confirmed by imaging and elevated IgG4 blood levels, and the patient completely recovered after corticosteroid therapy. One year later, he presented with a recurrent episode of AIP with elevated IgG4 levels, accompanied by the appearance of multiple intrapancreatic cystic lesions. All but 1 of these cysts disappeared after steroid treatment, but the remaining cyst in the pancreatic head was even somewhat larger 1 year later. Pancreatoduodenectomy was finally performed. Histology showed the wall of the cystic lesion to be fibrotic; the surrounding pancreatic tissue presented fibrosis, atrophy and lymphoplasmacytic infiltration by IgG4-positive cells, without malignant elements. Our case illustrates the rare possibility that cystic lesions can be part of AIP. These pseudocysts appear in the pancreatic segments involved in the autoimmune disease and can be a consequence of the local inflammation or related to ductal strictures. Steroid treatment should be initiated, after which these cysts can completely disappear with recovery from AIP. Surgical intervention may be necessary in some exceptional cases.

  11. Prostatic Duct Adenocarcinoma with Endometrioid Features: Report ...

    African Journals Online (AJOL)

    mn

    4, 2009. 257. 257-258. Case Report. Prostatic Duct Adenocarcinoma with Endometrioid. Features: Report of a Rare Case and Brief Review of the Literature ... 3. adenocarcinoma of the prostate: A distinctive tumor of probable prostatic duct origin. Cancer. 1976;. May;37(5):2255-62. Cohen RJ, Wheeler TM, Bonkhoff H, ...

  12. Calcium influx pathways in rat pancreatic ducts

    DEFF Research Database (Denmark)

    Hug, M J; Pahl, C; Novak, I

    1996-01-01

    A number of agonists increase intracellular Ca2+ activity, [Ca2+]i, in pancreatic ducts, but the influx/efflux pathways and intracellular Ca2+ stores in this epithelium are unknown. The aim of the present study was to characterise the Ca2+ influx pathways, especially their pH sensitivity, in native...... pathways in pancreatic ducts cells....

  13. Neurofibromas as bilateral cystic chest wall swellings.

    African Journals Online (AJOL)

    clinical entity in our centre. This rare entity should be borne in mind when considering the differential diagnosis of benign cystic chest wall tumours. Key words: neurofibromatosis, cystic swelling, posterior ... We are reporting a single case of bilat- eral cystic degenerative changes in neurofibromas that presented clinically like ...

  14. Cholangiographic evaluation of bile duct carcinoma

    International Nuclear Information System (INIS)

    Nichols, D.A.; MacCarty, R.L.; Gaffey, T.A.

    1983-01-01

    Cholangiograms and clinical histories of 82 patients with biopsy-proved bile duct carcinoma were reviewed. The carcinomas were classified according to morphologic findings and clinical outcome. Ulcerative colitis and antecedent inflammatory disease of the biliary tree, particularly primary sclerosing cholangitis, seem to predispose to the development of bile duct carcinoma. Focal stenotic lesions were the most common morphologic type (62/82). Polypoid carcinomas and diffuse sclerosing carcinomas were less common and of about equal frequency. Prognosis was best for patients with polypoid carcinomas and worst for those with diffuse sclerosing carcinomas. In 69 cases (84%), the tumors involved the intrahepatic or proximal extrahepatic ducts, makin curative resection difficult or impossible. Patients with carcinomas limited to the more distal extrahepatic bile ducts had a longer average survival and a higher probability of surgical cure. Proper management of patients with bile duct carcinoma requires a complete and accurate cholangiographic evaluation of the morphology, location, and extent of the disease

  15. Salivary Duct Cyst: Histo-pathologic Correlation

    Directory of Open Access Journals (Sweden)

    Divya Vinayachandran

    2013-01-01

    Full Text Available Non-neoplastic cysts of the salivary glands are uncommon and represent 2-5% of all salivary gland lesions. They are mainly mucoceles or salivary duct cysts. Unlike a mucocele, which is surrounded by granulation tissue, the salivary duct cyst is lined by epithelium. Salivary duct cysts are more common in the oral minor salivary glands and rarely occur in the major salivary glands, show a marked predilection for the superficial lobe of the parotid, and represent 10% of all salivary gland cysts. Neoplastic differentiation of the lining of the salivary duct cyst has been reported. We report a case of a salivary duct cyst of the left parotid gland, with a review of radiographic and histopathologic features.

  16. Mirizzi Syndrome Type 2: A Case Report

    Directory of Open Access Journals (Sweden)

    Aydemir Ölmez

    2009-01-01

    Full Text Available Mirizzi syndrome is an unusual complication of gallstone disease and occurs in approximately 1% of these pa-tients. Some cases can not be identified preoperatively; despite modern imaging techniques. Today; treatment of Mirizzi syndrome is surgical. If Mirizzi syndrome is pre-sent; the risk of bile duct injury increases; particularly during laparoscopic surgery. Therefore; preoperative or intraoperative diagnosis is important. Here; we pre-sented a 29 year-old woman with obstructive jaundice who diagnosed as cholelithiasis and choledocholithiasis. Preoperative endoscopic retrograde cholangiography re-lieved the common bile duct stone but cound not diag-nosed the Mirizzi syndrome preoperatively. During lapa-roscopy; the diagnosis of Mirizzi syndrome was sus-pected early and the procedure was converted to open cholecystectomy and T-tube to common bile duct. There was no bile duct injury and postoperative course was uneventful.

  17. Intra and extra hepathic biliary ducts of ducks (Anas boscas Vias biliares intra e extra-hepáticas de marreco-de-pequim (Anas boscas

    Directory of Open Access Journals (Sweden)

    Juarez Cezar Borges de Aquino

    2011-12-01

    Full Text Available A study in 38 liver of adults ducks from Jundiaí, state of São Paulo, was developed. After injection of Neoprene latex 650 colored by green and fixation in aqueous 10% formalin, the material was dissected under dental loupe, photographed and sketched. The intra hepatic bile ducts of the left lobe showed up in 76.3% the samples consisting of three collecting ducts bile; and 23.7% of the cases, there was the presence of one collector originated from caudal segment, draining to the hepatic duodenal duct. In the right lobe, in 100% of samples, were four collecting duct segments: cranium medial superficial, cranium medial deep, lateral and caudal (ventral caudal. With regard to biliary extra hepatic in 36 samples(94.7% it was composed of three ducts visible in hepatic hilum: hepatic duodenal, hepatic cystic and cystic duodenal. In two samples (5.3% was not observed hepatic cystic duct. The hepatic duodenal ductand cystic duodenal duct in 100% of the samples, they reached the duodenum. In six samples (15.8 %, besides the hepatic cystic duct, the gallbladder was achieved directly by bile collecting ducts of the right lobe. The gallbladder was not achieved by hepatic cystic duct in two samples (5.3 %. As vias biliares intra e extra-hepáticas de 38 marrecos-de-pequim adultos, provenientes de Jundiaí,estado de São Paulo, após injeção de Neoprene Látex 650 corado de verde e fixação em solução aquosa de formol a 10%, foram dissecados sob lupa odontológica, fotografados e desenhados esquematicamente. A via biliar intra-hepática no lobo esquerdo mostrou-se constituída por três ductos coletores biliares em 76,3% das amostras e, em 23,7% dos casos, está presente um quarto coletor, originado no segmentocaudal, drenando para o ducto hepatoduodenal. No lobo direito, em 100% das amostras ocorreram quatro ductos coletores dos segmentos craniomedial superficial, craniomedial profundo, lateral e caudal (ventrocaudal. No que se refere à via biliar

  18. Non-cystic fibrosis bronchiectasis.

    Science.gov (United States)

    Neves, Paulo C; Guerra, Miguel; Ponce, Paulo; Miranda, José; Vouga, Luís

    2011-12-01

    Bronchiectasis is characterized by irreversible widening of the medium-sized airways, with inflammation, chronic bacterial infection and destruction of the bronchial walls. Exercise or inspiratory muscle training may improve quality of life and exercise endurance in people with non-cystic fibrosis bronchiectasis. Prolonged-use antibiotics improve clinical response rates, but may not reduce exacerbation rates or lung function. Surgery is often considered for people with extreme damage to one or two lobes of the lung who are at risk for severe infection or bleeding. In this review, the authors will focus on non-cystic fibrosis bronchiectasis, pointing out the differences in management when compared with the cystic fibrosis context, with special emphasis on surgical management.

  19. Sphenoid sinus adenoid cystic carcinoma

    Directory of Open Access Journals (Sweden)

    Marambaia, Otavio

    2008-12-01

    Full Text Available Introduction: The sphenoid adenoid cystic carcinoma is a rare malign neoplasm, in the head and neck and when located in the paranasal sinuses, it is formed in the minor salivary glands. It grows slowly and is characterized by a large invasion of the adjacent tissues, and also has a large capacity of metastasis. The surgery associated with post-operative radiotherapy is used as treatment. Objective: To describe a case of sphenoid sinus adenoid cystic carcinoma in a male, black, 62 year patient. Case Report: N.L.B., 62 years of age, male, had bloody rhinorrhea for 6 months associated with bilateral nasal obstruction. The nasofibroscopy showed lesion of polypoid aspect in the left nasal cavity. He was submitted to biopsy and the anatomopathological exam showed adenoid cystic carcinoma and the patient was forwarded to oncology. Conclusions: The importance of conducting the differential diagnosis between chronic nasosinusal infection and nasosinusal tumors.

  20. Cystic fibrosis in adults

    Directory of Open Access Journals (Sweden)

    C. Damas

    2007-05-01

    Full Text Available The authors reviewed adult cystic fibrosis patients followed in the Pulmonology Unit from 1994-2004 (n = 8, five female and three male, aged 20-34 years old (median = 27 years. Patients were diagnosed at 18 months - 31 years old by sweat testing (positive in six patients and genotyping (four patients homozygous for ΔF508 mutation.Respiratory involvement was characterised by sinusitis and bronchiectasis. Pulmonary involvement was accompanied by functional abnormalities and gas exchange impairment in the majority of the patients. Bronchial tree was colonised permanently in five patients: Pseudomonas aeruginosa in four and Staphilococcus aureus in four (three patients affected by both agents simultaneously.The main causes of exacerbation were respiratory infections and haemoptysis.Non-respiratory involvement was variable. Four patients had digestive involvement (one with hepatic cirrhosis, one had renal failure and only one had a sperm count to document infertility. Four patients had osteopaenia.Treatment included chest physiotherapy, bronchodilators, dornase alfa, mucolytics, digestive enzymes, vitamins, antibiotics and oxygen therapy.At review, one had left follow-up, one had died, one was awaiting lung transplant and the others evidenced no difference in clinical characteristics.In this group of patients the severity of the pulmonary disease was not related to a late diagnosis. It can be explained by the diversity of cystic fibrosis presentation in adults Resumo: Os autores efectuaram uma revisão de doentes adultos com fibrose quística (FQ, seguidos na consulta de Pneumologia no período de 1994-2004 (n = 8: cinco mulheres e três homens, com idades compreendidas entre 20 e 34 anos (mediana  =  27 anos, cuja idade de diagnóstico variou entre os 18 meses e os 31 anos.O diagnóstico foi obtido por prova de suor (positiva em seis doentes e estudo genético (homozigotia para a mutação ΔF508 em

  1. Bile Duct Adenoma with Oncocytic Features

    Directory of Open Access Journals (Sweden)

    E. J. Johannesen

    2014-01-01

    Full Text Available Bile duct adenomas are benign bile duct proliferations usually encountered as an incidental finding. Oncocytic bile duct neoplasms are rare and the majority are malignant. A 61-year-old male with a diagnosis of colorectal adenocarcinoma was undergoing surgery when a small white nodule was discovered on the surface of the right lobe of his liver. This lesion was composed of cytologically bland cells arranged in tightly packed glands. These cells were immunopositive for cytokeratin 7, negative for Hep Par 1, contained mucin, and had a Ki67 proliferation index of 8%. The morphology, immunophenotype, presence of mucin, and normal appearing bile ducts, as well as the increased Ki67 proliferation rate, were consistent with a bile duct adenoma with oxyphilic (oncocytic change. Oncocytic tumors in the liver are rare; the first described in 1992. Only two bile duct adenomas with oncocytic change have been reported and neither of them had reported mucin production or the presence of normal appearing bile ducts within the lesion.

  2. Diagnostic value to depict branch pancreatic ducts dilatation by MR cholangiopancreatography

    International Nuclear Information System (INIS)

    Li Liang; Yuan Jiachang; Jin Erhu

    2009-01-01

    Objective: To evaluate the diagnostic value of depicting dilatation of branch pancreatic ducts (BPD) in patients with chronic pancreatitis and pancreatic carcinomas in MR cholangiopancreatography. Methods: Forty-five patients with chronic pancreatitis and thirty patients with pancreatic carcinomas were enrolled in the study. All the patients underwent MRI using axial T 1 weighted imaging, T 2 weighted imaging and MR cholangiopancreatography in a 3.0 T MR unit. and dilatation of BPD was analyse by which their morphology, location and extent of dilatation. Results: Morphologically, dilatation of BPD in cystic, dendroid manner or both of them were seen in 19, 42 and 16 cases in chronic pancreatitis, and 6, 29 and 5 cases in pancreatic carcinomas, respectively. In location, dilatation of BPD in proximal, distal or full coverage of main pancreatic duct were seen in 0, 10 and 35 cases in chronic pancreatitis, and 1, 12 and 17 cases in pancreatic carcinomas, respectively. Conclusion: Dilatation of BPD and main pancreatic duct could be well visualized in MR cholangiopancreatography at 3.0 T. It is helpful in differentiating chronic pancreatitis and pancreatic cancinoma to be familiar with features of dilatation of BPD. (authors)

  3. Cystic echinococcosis of the liver

    DEFF Research Database (Denmark)

    Branci, Sonia; Ewertsen, Caroline; Thybo, Søren

    2012-01-01

    Cystic echinococcosis (CE) of the liver can be treated with ultrasound-guided puncture, aspiration, injection, and re-aspiration (PAIR), with surgery and with benzimidazole derivatives. The aim of this study was to review available data concerning treatment modality and outcome for patients treated...... for CE of the liver in a Danish tertiary reference center....

  4. Lactate in cystic fibrosis sputum

    DEFF Research Database (Denmark)

    Bensel, Tobias; Stotz, Martin; Borneff-Lipp, Marianne

    2011-01-01

    Antibiotic therapy is thought to improve lung function in patients with cystic fibrosis (CF) by decreasing neutrophil-derived inflammation. We investigated the origin and clinical significance of lactate in the chronically inflamed CF lung. Methods Lactate was measured in sputa of 18 exacerbated...

  5. Meconium ileus in Cystic Fibrosis

    NARCIS (Netherlands)

    Sathe, Meghana; Houwen, Roderick

    2017-01-01

    Meconium ileus (MI) is often the first manifestation of cystic fibrosis (CF) and occurs in approximately 20% of patients diagnosed with CF. This article reviews the pathophysiology of MI and its clinical presentation. It focuses on the medical and surgical management emphasizing the importance of

  6. Lung transplantation for cystic fibrosis

    NARCIS (Netherlands)

    Adler, Frederick R; Aurora, Paul; Barker, David H; Barr, Mark L; Blackwell, Laura S; Bosma, Otto H; Brown, Samuel; Cox, D R; Jensen, Judy L; Kurland, Geoffrey; Nossent, George D; Quittner, Alexandra L; Robinson, Walter M; Romero, Sandy L; Spencer, Helen; Sweet, Stuart C; van der Bij, Wim; Vermeulen, J; Verschuuren, Erik A M; Vrijlandt, Elianne J L E; Walsh, William; Woo, Marlyn S; Liou, Theodore G

    2009-01-01

    Lung transplantation is a complex, high-risk, potentially life-saving therapy for the end-stage lung disease of cystic fibrosis (CF). The decision to pursue transplantation involves comparing the likelihood of survival with and without transplantation as well as assessing the effect of wait-listing

  7. Multiple arterial thromboses due to cystic medial degeneration Erdheim-Gsell: A case report.

    Science.gov (United States)

    Jud, Philipp; Gary, Thomas; Hafner, Franz; Tiesenhausen, Kurt; Ott, Thomas; Oswald, Wolfgang Kurt; Brodmann, Marianne

    2017-11-01

    Cystic medial degeneration Erdheim-Gsell is a vascular pathology mainly of the large vessels, which is mostly associated with Marfan syndrome or Ehlers-Danlos syndrome. The clinical findings of this entity are aneurysms of the aorta or large peripheral arteries which usually present in an acute setting due to rupture of an aneurysm. We present a case of a 43-year-old Caucasian male with histologically proven cystic medial degeneration of the lower limb vessels mimicking peripheral artery occlusive disease. Despite antiplatelet and anticoagulant treatment, the patient suffered multiple vascular stenosis and occlusions. Multiple arterial stenoses and thromboses leading to peripheral artery occlusive disease caused by cystic medial degeneration Erdheim-Gsell. Multiple surgical and endovascular interventions including bypass graft and intra-arterial thrombolysis as well as oral antiplatelet and anticoagulant therapy. Despite dual antiplatelet therapy, anticoagulant therapy with rivaroxaban and multiple surgical and endovascular interventions, the patient developed recurrent arterial thromboses. The patient did not suffer further thrombotic events since clopidogrel and phenprocoumon were administered. Clinical presentation of cystic medial degeneration Erdheim-Gsell mimicking peripheral artery occlusive disease is very unusual. Due to the fragile vessel wall, patients with cystic medial degeneration might have a higher risk to develop arterial thromboses, even under antiplatelet therapy or anticoagulant treatment. Copyright © 2017 The Authors. Published by Wolters Kluwer Health, Inc. All rights reserved.

  8. Multiple arterial thromboses due to cystic medial degeneration Erdheim-Gsell

    Science.gov (United States)

    Jud, Philipp; Gary, Thomas; Hafner, Franz; Tiesenhausen, Kurt; Ott, Thomas; Oswald, Wolfgang Kurt; Brodmann, Marianne

    2017-01-01

    Abstract Rationale: Cystic medial degeneration Erdheim-Gsell is a vascular pathology mainly of the large vessels, which is mostly associated with Marfan syndrome or Ehlers-Danlos syndrome. The clinical findings of this entity are aneurysms of the aorta or large peripheral arteries which usually present in an acute setting due to rupture of an aneurysm. Patient concerns: We present a case of a 43-year-old Caucasian male with histologically proven cystic medial degeneration of the lower limb vessels mimicking peripheral artery occlusive disease. Despite antiplatelet and anticoagulant treatment, the patient suffered multiple vascular stenosis and occlusions. Diagnoses: Multiple arterial stenoses and thromboses leading to peripheral artery occlusive disease caused by cystic medial degeneration Erdheim-Gsell. Interventions: Multiple surgical and endovascular interventions including bypass graft and intra-arterial thrombolysis as well as oral antiplatelet and anticoagulant therapy. Outcome: Despite dual antiplatelet therapy, anticoagulant therapy with rivaroxaban and multiple surgical and endovascular interventions, the patient developed recurrent arterial thromboses. The patient did not suffer further thrombotic events since clopidogrel and phenprocoumon were administered. Lessons: Clinical presentation of cystic medial degeneration Erdheim-Gsell mimicking peripheral artery occlusive disease is very unusual. Due to the fragile vessel wall, patients with cystic medial degeneration might have a higher risk to develop arterial thromboses, even under antiplatelet therapy or anticoagulant treatment. PMID:29381979

  9. [Salivary gland drainage into the thyroglossal duct].

    Science.gov (United States)

    Siem, G; Natvig, K; Kolbenstvedt, A; Lømo, J

    2001-01-20

    Failure in regression of the thyroglossal duct is one of the most common reasons for midline swellings in the neck. Several authors have described recurrent thyroglossal duct remnants with persisting draining sinuses. However, few have described accessory salivary glands that drain into the thyroglossal duct. In this article we report two such cases with midline salivary glands in the floor of the mouth. These two patients were subsequently successfully treated with radical tissue resection in the area between the hyoid bone and foramen cecum. Preoperative fistulography or sinography was useful to demonstrate the ductal ramification of the salivary glands, and use of methylene blue during surgery proved of significant value for the result.

  10. Huge nasopalatine duct cyst treatment with the help of cystectomy and bilateral fenestration surgery of the nasal cavity: A case report

    Directory of Open Access Journals (Sweden)

    H. Kobashi

    2017-12-01

    Full Text Available The nasopalatine duct cyst (NPDC is the most common non-odontogenic, epithelial cyst occurring in the maxilla, and it arises from the epithelial remnants of the nasopalatine duct. It accounts for approximately 2% of all maxillary cysts. A 44-year-old man presented with an asymptomatic swelling in the upper vestibular gingiva and hard palate. Computed tomography confirmed these findings, revealing a cystic mass in the midline of the maxilla that superiorly extended along the nasal septum and inferiorly along the floor of the nasal cavity. It had a maximum diameter of 42 mm. The cyst was treated by cystectomy and bilateral fenestration surgery of the nasal cavity under general anesthesia. The patient had no postoperative complications and showed no evidence of stenosis of the opening or infection in the cavity 6 months after surgery. Keywords: Nasopalatine duct cyst, Non-odontogenic epithelial cyst, Cystectomy, Fenestration surgery

  11. [Therapeutic update in cystic fibrosis].

    Science.gov (United States)

    Durupt, S; Nove Josserand, R; Durieu, I

    2014-06-01

    We present the recent therapeutic advances in the cystic fibrosis care. It concerns improvements in symptomatic treatment with the development of dry powder inhaled antibiotics that improved quality of life, and innovative treatments namely the modulators of the cystic fibrosis transmembrane protein conductance regulator (CFTR), molecules which act specifically at the level of the defective mechanisms implied in the disease. The life expectancy of cystic fibrosis patients born after 2000, is estimated now to be about 50 years. This improvement of survival was obtained with the organization of the care within the specialized centers for cystic fibrosis (Centre de ressource et de compétences de la mucoviscidose) and remains still based on heavy symptomatic treatments. Dry powder inhaled antibiotics constitute a significant time saving for patients to whom all the care can achieve two hours daily. Since 2012, the modulators of CFTR, molecules allowing a pharmacological approach targeted according to the type of the mutations, allows a more specific approach of the disease. Ivacaftor (Kalydeco(®)) which potentialises the function of the CFTR protein expressed on the cellular surface is now available for patients with the G551D mutation. Lumacaftor is going to be tested in association with ivacaftor in patients with the F508del mutation, that is present in at least 75% of the patients. The ataluren which allows the production of a functional protein CFTR in patients with a no sense mutation is the third representing of this new therapeutic class. We presently have numerous symptomatic treatments for the cystic fibrosis care. The development of CFTR modulators, today available to a restricted number of patients treated with ivacaftor represents a very promising therapeutic avenue. It will represent probably the first step to a personalized treatment according to CFTR genotype. Copyright © 2013 Société nationale française de médecine interne (SNFMI). Published by

  12. Cystic swellings of scrotum: Management

    Directory of Open Access Journals (Sweden)

    Subith Kumar K, Sasikumar J, Seetharamaiah T, Ajay Kumar B, Venugopalacharyulu N CH

    2014-04-01

    Full Text Available The cystic swellings of scrotum are one among the commonest clinical entities faced by a surgeon in day to day practice and a cause of concern to the patient more with his fertility. Presenting with varied etiology they can represent a wide range of medical issues. Gold standard for the management of such cystic swellings of scrotum continues to be surgical extirpation of the lesion. Objective: To identify the cause, mode of presentation, various modalities of treatment and outcome of these with their complications. Method: A total of 170 cases of cystic swellings of scrotum fulfilling the methodology criteria were subjected to the preformatted study. Diagnosis was mostly by clinical examination and supported by ultrasonography. All cases were treated surgically with the appropriate surgical procedure. Results: Maximum patients were in the age group of 31-50 years and presented with scrotal swelling (59% and more commonly affecting the right side. The commonest cause for cystic swelling of the scrotum was primary vaginal hydrocele (55%. Surgical procedure using Lords Plication was found to be simple, effective and associated with minimum post operative complications; the other conventional techniques like Partial/sub-total excision of sac, everson of sac were associated with more complications like haematoma, scrotal edema and infection. Majority of patients were discharged on 7th Post-operative day. Conclusion: The present study, primary vaginal hydrocele was the commonest cystic swelling of scrotum and treated surgically showed good results. Lord’s procedure was associated with the less post-operative complications, minimal tissue handling and good haemostatic control.

  13. Cystic Hygroma in an Adult; a Case Report | Veeraraghavan ...

    African Journals Online (AJOL)

    Lymphangioma is a benign infiltrative malformation of the lymphatic channels. Cystic lymphangioma or cystic hygroma is a subtype of lymphangioma which exhibits large macroscopic cystic space histologically. The cause of cystic hygroma is believed to be developmental defect or primary Multilocular cystic malformation of ...

  14. Traumatic resin ducts as indicators of bark beetle outbreaks

    Science.gov (United States)

    R. Justin DeRose; Matthew F. Bekker; James N. Long

    2017-01-01

    The formation of traumatic resin ducts (TRDs) represents an important induced defense in woody plants that enhances oleoresin production and flow in response to environmental perturbations. In some genera (Pinus), resin ducts are copious and conspicuous; however, in others (Picea), resin ducts are relatively rare. The occurrence and strength of resin ducts, in...

  15. Primary hepatocellular carcinoma in extrahepatic bile duct

    International Nuclear Information System (INIS)

    Jeong, Seok Tae; Ham, Soo Youn; Park, Cheol Min; Kim, Jung Hyuk; Cha, In Ho; Chung, Kyoo Byung; Suh, Woon Hyuck; Lee, Chang Hong

    1991-01-01

    Obstructive jaundice due to hepatocellular carcinoma in an extrahepatic bile duct, without a mass lesion in the liver parenchyma, is extremely rare. We experienced two cases of primary hepatocellular carcinoma arising from an extrahepatic bile duct: one in a 53-year-old man whose α -fetoprotein value was 800 ng/ml, and another in a 39-year-old woman, in whom the mass lesion was found to be attached to an extrahepatic bile duct. These tumors had a well-marginated sausage-like shape on CT and US, and the contrast media passed freely along their margins on both PTC and ERCP. Recurrences of these tumors were observed in the extrahepatic bile duct 6 and 2 months after surgery, respectively

  16. Unusually large sialolith of Wharton's duct

    Science.gov (United States)

    Iqbal, Ali; Gupta, Anup K.; Natu, Subodh S.; Gupta, Atul K.

    2012-01-01

    The formation of calcific concretions in the salivary duct or glands is a common disorder, especially in the submandibular glands. Most of the salivary calculi are small in size, in contrast to those that reach several centimeters, which are reported as megaliths or giant calculi in the literature. They may occur in any of the salivary gland ducts but are most common in Wharton's duct and the submandibular gland. This report presents clinical and radiographical sign of an unusually large sialolith. There was painless swelling on the floor of the edentulous mouth and patient was unaware of it. Radiographical examination revealed large irregular radio-opaque mass superimposed on right canine and premolar areas. This case report describes a patient presenting with an unusually large submandibular gland duct sialolith, the subsequent patient management, the aetiology, diagnosis and its treatment. PMID:23483770

  17. Reference values of MRI measurements of the common bile duct and pancreatic duct in children

    International Nuclear Information System (INIS)

    Gwal, Kriti; Bedoya, Maria A.; Patel, Neal; Darge, Kassa; Anupindi, Sudha A.; Rambhatla, Siri J.; Sreedharan, Ram R.

    2015-01-01

    Magnetic resonance imaging/cholangiopancreatography (MRI/MRCP) is now an essential imaging modality for the evaluation of biliary and pancreatic pathology in children, but there are no data depicting the normal diameters of the common bile duct (CBD) and pancreatic duct. Recognition of abnormal duct size is important and the increasing use of MRCP necessitates normal MRI measurements. To present normal MRI measurements for the common bile duct and pancreatic duct in children. In this retrospective study we searched all children ages birth to 10 years in our MR urography (MRU) database from 2006 until 2013. We excluded children with a history of hepatobiliary or pancreatic surgery. We stratified 204 children into five age groups and retrospectively measured the CBD and the pancreatic duct on 2-D axial and 3-D coronal T2-weighted sequences. We performed statistical analysis, using logistic and linear regressions to detect the age association of the visibility and size of the duct measurements. We used non-parametric tests to detect gender and imaging plane differences. Our study included 204 children, 106 (52%) boys and 98 (48%) girls, with a median age of 33 months (range 0-119 months). The children were distributed into five age groups. The common bile duct was visible in all children in all age groups. The pancreatic duct was significantly less visible in the youngest children, group 1 (54/67, 80.5%; P = 0.003) than in the oldest children, group 5 (22/22, 100%). In group 2 the pancreatic duct was seen in 19/21 (90.4%), in group 3 52/55 (94.5%), and in group 4 39/39 (100%). All duct measurements increased with age (P < 0.001; r-value > 0.423), and the incremental differences between ages were significant. The measurement variations between the axial and coronal planes were statistically significant (P < 0.001); however these differences were fractions of millimeters. For example, in group 1 the mean coronal measurement of the CBD was 2.1 mm and the axial

  18. Reference values of MRI measurements of the common bile duct and pancreatic duct in children

    Energy Technology Data Exchange (ETDEWEB)

    Gwal, Kriti; Bedoya, Maria A.; Patel, Neal; Darge, Kassa; Anupindi, Sudha A. [University of Pennsylvania Perelman School of Medicine, Department of Radiology, The Children' s Hospital of Philadelphia, Philadelphia, PA (United States); Rambhatla, Siri J. [Beth Israel Medical Center, Department of Pediatrics, Newark, NJ (United States); Sreedharan, Ram R. [University of Pennsylvania, Departments of Gastroenterology, Hepatology and Nutrition, The Children' s Hospital of Philadelphia, Perelman School of Medicine, Philadelphia, PA (United States)

    2015-08-15

    Magnetic resonance imaging/cholangiopancreatography (MRI/MRCP) is now an essential imaging modality for the evaluation of biliary and pancreatic pathology in children, but there are no data depicting the normal diameters of the common bile duct (CBD) and pancreatic duct. Recognition of abnormal duct size is important and the increasing use of MRCP necessitates normal MRI measurements. To present normal MRI measurements for the common bile duct and pancreatic duct in children. In this retrospective study we searched all children ages birth to 10 years in our MR urography (MRU) database from 2006 until 2013. We excluded children with a history of hepatobiliary or pancreatic surgery. We stratified 204 children into five age groups and retrospectively measured the CBD and the pancreatic duct on 2-D axial and 3-D coronal T2-weighted sequences. We performed statistical analysis, using logistic and linear regressions to detect the age association of the visibility and size of the duct measurements. We used non-parametric tests to detect gender and imaging plane differences. Our study included 204 children, 106 (52%) boys and 98 (48%) girls, with a median age of 33 months (range 0-119 months). The children were distributed into five age groups. The common bile duct was visible in all children in all age groups. The pancreatic duct was significantly less visible in the youngest children, group 1 (54/67, 80.5%; P = 0.003) than in the oldest children, group 5 (22/22, 100%). In group 2 the pancreatic duct was seen in 19/21 (90.4%), in group 3 52/55 (94.5%), and in group 4 39/39 (100%). All duct measurements increased with age (P < 0.001; r-value > 0.423), and the incremental differences between ages were significant. The measurement variations between the axial and coronal planes were statistically significant (P < 0.001); however these differences were fractions of millimeters. For example, in group 1 the mean coronal measurement of the CBD was 2.1 mm and the axial

  19. INTERIOR DUCT SYSTEM DESIGN, CONSTRUCTION, AND PERFORMANCE

    Energy Technology Data Exchange (ETDEWEB)

    Janet E.R. Mcllvaine; David Beal; Philip Fairey

    2001-10-10

    By removing air distribution and conditioning equipment from unconditioned spaces, homeowners stand to benefit substantially with respect to both energy savings and indoor air quality. Duct leakage introduces: Greater heating and cooling loads from air at extreme temperatures and humidity levels; Outside air and air from unconditioned spaces that may contain air borne contaminants, combustion gases, pollen, mold spores, and/or particles of building materials; and Higher whole-house infiltration/exfiltration rates. Exemplary studies conducted since 1990 have demonstrated the prevalence of duct leakage throughout the United States and measured energy savings of approximately 20% during both heating and cooling seasons from leakage reduction. These all dealt with duct leakage to and/or from unconditioned spaces. In the building science community, leakage within the conditioned space is generally presumed to eliminate the negative consequences of duct leakage with the exception of possibly creating pressure imbalances in the house which relates to higher infiltration and/or exfiltration. The practical challenges of isolating ducts and air handlers from unconditioned spaces require builders to construct an air-tight environment for the ducts. Florida Solar Energy Center researchers worked with four builders in Texas, North Carolina, and Florida who build a furred-down chase located either in a central hallway or at the edges of rooms as an architectural detail. Some comparison homes with duct systems in attics and crawl spaces were included in the test group of more than 20 homes. Test data reveals that all of the duct/AHU systems built inside the conditioned space had lower duct leakage to unconditioned spaces than their conventional counterparts; however, none of the homes was completely free of duct leakage to unconditioned spaces. Common problems included wiring and plumbing penetrations of the chase, failure to treat the chase as an air tight space, and misguided

  20. Cleanliness of ventilation ducts, indoor space and AHU

    OpenAIRE

    Rubina, Aleš; Rubinová, Olga; Blasinski, Petr

    2015-01-01

    Paper deals with analysis of cleanliness air conditioning duct for various stages of construction. There were taken samples and these were analyzed for different type of storage duct on construction. They are compared differences between the "right" stored duct and unprotected duct including the actual interior space and air handling units serving. In the conclusion of this article is summarized the impact of carryover dirt from the duct and an air handling unit to the ventilation s...

  1. A Comparison of Simulation Capabilities for Ducts

    Energy Technology Data Exchange (ETDEWEB)

    Miller, William A [ORNL; Smith, Matt K [ORNL; Gu, Lixing [Florida Solar Energy Center (FSEC); New, Joshua Ryan [ORNL

    2014-11-01

    Typically, the cheapest way to install a central air conditioning system in residential buildings is to place the ductwork in the attic. Energy losses due to duct-attic interactions can be great, but current whole-house models are unable to capture the dynamic multi-mode physics of the interactions. The building industry is notoriously fragmented and unable to devote adequate research resources to solve this problem. Builders are going to continue to put ducts in the attic because floor space is too expensive to closet them within living space, and there are both construction and aesthetic issues with other approaches such as dropped ceilings. Thus, there is a substantial need to publicly document duct losses and the cost of energy used by ducts in attics so that practitioners, builders, homeowners and state and federal code officials can make informed decisions leading to changes in new construction and additional retrofit actions. Thus, the goal of this study is to conduct a comparison of AtticSim and EnergyPlus simulation algorithms to identify specific features for potential inclusion in EnergyPlus that would allow higher-fidelity modeling of HVAC operation and duct transport of conditioned air. It is anticipated that the resulting analysis from these simulation tools will inform energy decisions relating to the role of ducts in future building energy codes and standards.

  2. Recommendations for quality improvement in genetic testing for cystic fibrosis European Concerted Action on Cystic Fibrosis

    NARCIS (Netherlands)

    Dequeker, E; Cuppens, H; Dodge, J; Estivill, [No Value; Goossens, M; Pignatti, PF; Scheffer, H; Schwartz, M; Schwarz, M; Tummler, B; Cassiman, JJ

    These recommendations for quality improvement of cystic fibrosis genetic diagnostic testing provide general guidelines for the molecular genetic testing of cystic fibrosis in patients/individuals. General strategies for testing as well as guidelines for laboratory procedures, internal and external

  3. Ectopic Phosphorylated Creb Marks Dedifferentiated Proximal Tubules in Cystic Kidney Disease.

    Science.gov (United States)

    Puri, Pawan; Schaefer, Caitlin M; Bushnell, Daniel; Taglienti, Mary E; Kreidberg, Jordan A; Yoder, Bradley K; Bates, Carlton M

    2018-01-01

    Ectopic cAMP signaling is pathologic in polycystic kidney disease; however, its spatiotemporal actions are unclear. We characterized the expression of phosphorylated Creb (p-Creb), a target and mediator of cAMP signaling, in developing and cystic kidney models. We also examined tubule-specific effects of cAMP analogs in cystogenesis in embryonic kidney explants. In wild-type mice, p-Creb marked nephron progenitors (NP), early epithelial NP derivatives, ureteric bud, and cortical stroma; p-Creb was present in differentiated thick ascending limb of Henle, collecting duct, and stroma; however, it disappeared in mature NP-derived proximal tubules. In Six2cre;Frs2α Fl/Fl mice, a renal cystic model, ectopic p-Creb stained proximal tubule-derived cystic segments that lost the differentiation marker lotus tetragonolobus lectin. Furthermore, lotus tetragonolobus lectin-negative/p-Creb-positive cyst segments (re)-expressed Ncam1, Pax2, and Sox9 markers of immature nephron structures and dedifferentiated proximal tubules after acute kidney injury. These dedifferentiation markers were co-expressed with p-Creb in renal cysts in Itf88 knockout mice subjected to ischemia and Six2cre;Pkd1 Fl/Fl mice, other renal cystogenesis models. 8-Br-cAMP addition to wild-type embryonic kidney explants induced proximal tubular cystogenesis and p-Creb expression; these effects were blocked by co-addition of protein kinase A inhibitor. Thus p-Creb/cAMP signaling is appropriate in NP and early nephron derivatives, but disappears in mature proximal tubules. Moreover, ectopic p-Creb expression/cAMP signaling marks dedifferentiated proximal tubular cystic segments. Furthermore, proximal tubules are predisposed to become cystic after cAMP stimulation. Copyright © 2018 American Society for Investigative Pathology. Published by Elsevier Inc. All rights reserved.

  4. MRI in mucoviscidosis (cystic fibrosis)

    International Nuclear Information System (INIS)

    Eichinger, M.; Puderbach, M.; Kauczor, H.-U.; Heussel, C.-P.

    2006-01-01

    Cystic fibrosis (CF) is a multi-systemic disease with major impact on the lungs. Pulmonary manifestation is crucial for the prognosis and life expectancy of patients. Imaging modalities and lung function tests reflect the pulmonary status in these patients. The standard imaging modality for diagnosis and follow-up of pulmonary changes is chest x-ray. The gold standard for the detection of parenchymal lung changes remains high resolution computed tomography (HRCT), but this is not used routinely for CF-patients due to radiation exposure. Magnetic resonance imaging (MRI) used to be of no importance in monitoring cystic fibrosis lung disease, as shown in studies from the 1980s and early 1990s. The continuing improvement of MRI techniques, however, has allowed for an adequate application of this non-radiation method in diagnosing the major pulmonary findings in CF, in addition to the assessment of lung function. (orig.) [de

  5. Posttraumatic Cranial Cystic Fibrous Dysplasia

    Directory of Open Access Journals (Sweden)

    Arata Tomiyama

    2011-01-01

    Full Text Available A 14-year-old was girl admitted to our hospital with a subcutaneous mass of the occipital head. The mass had grown for 6 years, after she had sustained a head injury at the age of 6, and was located directly under a previous wound. Skull X-ray Photograph (xp, computed tomography (CT, and magnetic resonance imaging (MRI showed a bony defect and cystic changes in the skull corresponding to a subcutaneous mass. Bone scintigraphy revealed partial accumulation. The patient underwent total removal of the skull mass, and the diagnosis from the pathological findings of the cyst wall was fibrous dysplasia (FD. The radiographic findings for cystic cranial FD can be various. Progressive skull disease has been reported to be associated with head trauma, but the relationship between cranial FD and head trauma has not been previously reported. Previous studies have suggested that c-fos gene expression is a key mechanism in injury-induced FD.

  6. A case of cystic fibrosis

    Directory of Open Access Journals (Sweden)

    Maria Alice Monti

    2009-09-01

    Full Text Available As the expected survival improves for patients with cystic fibrosis (CF, there is a growing population of adults with this disease. We describe a case of a 33-year-old woman with CF presenting with recurrent pancreatitis, malnutrition, borderline sweat test and respiratory diseases. The case report underlines the importance of diagnosis and management of CF in adults, and the important role played by the Family Physician in developing an adult care program.

  7. Enucleation or exenteration in two dogs with previous parotid duct transposition: parotid duct ligation versus reverse parotid duct transposition.

    Science.gov (United States)

    Young, Whitney M; Betbeze, Caroline M; Fisher, Stephen C; Boulay, James P

    2017-08-27

    Two dogs with previous parotid duct transpositions presented with unrelated ocular disease. In both cases, ophthalmic examination revealed the need for enucleation or exenteration. In case 1, systemic coccidioidomycosis was diagnosed with panuveitis and secondary glaucoma of the left eye. In this case, the parotid duct was ligated at the time of enucleation to stop salivary secretions. This dog encountered morbidity in the form of a sialocele that did not resolve for 11 months. In case 2, ultrasound and computed tomography revealed a discrete mass within the left medial orbit that was suspected to arise from the nictitating membrane. A combination of exenteration and parotid duct transposition reversal was performed to avoid morbidity associated with ligation of the parotid duct. The dog encountered no complications from this novel procedure. This case report represents the first report of re-routing a transposed parotid duct from the ventral conjunctival sac back to the mouth at the time of enucleation or exenteration in the dog. © 2017 American College of Veterinary Ophthalmologists.

  8. Ultrasound appearance of chronic mammary duct ectasia

    Energy Technology Data Exchange (ETDEWEB)

    Duchesne, N. [Ottawa Hospital, Dept. of Radiology, Ottawa, Ontario (Canada)]. E-mail: nathalie_duchesne_22@yahoo.ca; Skolnik, S. [Univ. of Toronto, Dept. of Family Medicine, Toronto, Ontario (Canada); Bilmer, S. [Ottawa Hospital, Dept. of Radiology, Ottawa, Ontario (Canada)

    2005-12-15

    Mammary duct ectasia (MDE), also called periductal mastitis, mammary dysplasia, or plasma cell mastitis, is a benign condition of the mammary gland first described by Haagensen in 1951. The etiology of MDE is unknown and its pathogenesis still controversial; the periductal inflammation could be either the cause or the result of dilated damaged ducts. The process is usually bilateral and asymptomatic, with only a small percentage of patients presenting with symptoms that may include long course of tumour formation, usually subareolar breast lumps, nipple discharge, nipple retraction, mastalgia, and mammary abscess or fistulas. Mammographic presentation of MDE is well known; its features include periductal calcification, benign intraductal calcification, and retroareolar duct dilatation. The periductal calcification results from dystrophic calcification and forms calcified rings or very dense, oval, elongated calcifications, each with a central lucency representing the dilated duct. Intraductal calcifications of duct ectasia represent inspissated intraductal material and are typically of uniform high density, often needle-like, and occasionally branching. Occasionally, there are no mammographic findings, and the diagnosis must rely on sonographic features. Appearance of MDE on ultrasonography (US) depends on the stage of the disease and the contents of the dilated ducts. The acute presentation has been demonstrated in the literature more often than has its chronic counterpart. In the former, duct content can vary from anechoic to isoechoic with surrounding fatty tissue. In chronic MDE, episodes of inflammation are longer. This tends to result in secretions that have a more solid, cheesy texture, partly due to cholesterol crystals, foam cells, and inflammatory cells. For both types of MDE, the appearance can mimic high-grade ductal carcinoma in situ (DCIS) on US. In this essay, 2 chronic MDE cases are presented and their US appearance discussed. Our goal is to explore

  9. The Sociology and Entrenchment. A Cystic Fibrosis Test for Everyone?

    DEFF Research Database (Denmark)

    Koch, Lene; Stemerding, Dirk

    1994-01-01

    Socialmedicine, genetic screening, cystic fibrosis, ethics, political regulation, sociology of technology......Socialmedicine, genetic screening, cystic fibrosis, ethics, political regulation, sociology of technology...

  10. Cystic Fibrosis & disorders of the large intestine: DIOS, constipation, and colorectal cancer.

    Science.gov (United States)

    Abraham, James M; Taylor, Christopher J

    2017-11-01

    Since 1966 when the Cystic Fibrosis Foundation Patient Registry (CFFPR) was founded, clinicians have witnessed significant advances in both the quality and quantity of life for patients living with Cystic Fibrosis (CF). As patients with CF live longer and fuller lives, increasing encumbrances from gastrointestinal manifestations of CF will be observed. This article serves to discuss "below the diaphragm" concerns involving the large intestine (Distal Intestinal Obstruction Syndrome, Constipation, and Colorectal Cancer). Avenues for development and implementation of clinical care protocols, particularly regarding proactive management of known associated conditions and cancer screening, will continue to be refined in the coming years. It falls to the multidisciplinary CF care team to be actively engaged in addressing these concerns effectively as priority shifts from relative acuity (typically related to early nutrition and lung function) to the travails of longevity as the CF population continues to age. Copyright © 2017 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.

  11. The "flying" bile duct: avulsion of the common bile duct in a plane crash survivor.

    LENUS (Irish Health Repository)

    Mohan, H

    2012-02-01

    Blunt trauma is an unusual cause of extrahepatic bile duct injury. This is a case of a 51-year-old gentleman who sustained a significant seatbelt injury in a plane crash. Laparotomy, performed due to persistent abdominal pain, revealed that the common bile duct (CBD) was completely avulsed from the duodenum. Following insertion of drains and transfer to a hepatobiliary centre, the devascularised CBD was excised and replaced with a roux-en-y hepaticojejunostomy. Necrotic tissue was debrided from the pancreatic head. A persistent bile leak developed from the sub-hepatic drain. Repeat laparotomy revealed a bile leak from small ducts on the liver surface. Ligation of the ducts and bioglue sealing of the area were successfully performed. Subsequent to this a pancreatic fistula developed from the main pancreatic duct, which has since resolved. This unusual case illustrates the need for prompt recognition and early repair to optimise outcomes in traumatic CBD injury.

  12. ITER L 6 equatorial maintenance duct remote handling study

    International Nuclear Information System (INIS)

    Millard, J.

    1996-09-01

    The status and conclusions of a preliminary study of equatorial maintenance duct remote handling is reported. Due to issues with the original duct design a significant portion of the study had to be refocused on equatorial duct layout studies. The study gives an overview of some of the options for design of these ducts and the impact of the design on the equipment to work in the duct. To develop a remote handling concept for creating access through the ducts the following design tasks should be performed: define the operations sequences for equatorial maintenance duct opening and closing; review the remote handling requirements for equatorial maintenance duct opening and closing; design concept for door and pipe handling equipment and to propose preliminary procedures for material handling outsides the duct. 35 figs

  13. New insights into the pathogenesis of cystic fibrosis: pivotal role of glutathione system dysfunction and implications for therapy.

    Science.gov (United States)

    Hudson, Valerie M

    2004-01-01

    findings in this area. Exogenous augmentation of GSH in the lung epithelial lining fluid is possible, and therapeutic approaches include administration of aerosolized buffered GSH, intravenous GSH, and oral GSH. However, it is important to remember that the pathophysiology of cystic fibrosis is multifactorial, and rectification of GSH system dysfunction in patients with cystic fibrosis will not eliminate all harmful effects of the disease. The promising results of two clinical trials of aerosolized buffered GSH in cystic fibrosis patients have been published or accepted for publication at the time of this writing. GSH depletion in lung epithelial lining fluid has also been noted in other respiratory diseases such as COPD, idiopathic pulmonary fibrosis, and adult respiratory distress syndrome, and therapies to augment GSH may also be contemplated in these diseases.

  14. Abdominal manifestations of cystic fibrosis in children

    International Nuclear Information System (INIS)

    Chaudry, Gulraiz; Navarro, Oscar M.; Levine, Daniel S.; Oudjhane, Kamaldine

    2006-01-01

    Pulmonary complications remain the main cause of mortality in cystic fibrosis, but the presenting symptoms in children are often related to gastrointestinal or pancreaticobiliary disease. Furthermore, abdominal manifestations are now seen throughout childhood, from infancy to adolescence. The child might present in the neonatal period with meconium ileus or its attendant complications. The older child might present with distal intestinal obstruction syndrome or colonic stricture secondary to high doses of pancreatic enzyme replacement. Less-common gastrointestinal manifestations include intussusception, duodenitis and fecal impaction of the appendix. Most children also show evidence of exocrine pancreatic deficiency. Radiologically, the combination of fat deposition and pancreatic fibrosis leads to varying CT and MR appearances. A higher than normal incidence of pancreatic cysts and calcification is also seen. Decreased transport of water and chloride also increases the viscosity of bile, with subsequent obstruction of the biliary ductules. If extensive, this can progress to obstructive cirrhosis, portal hypertension and esophageal varices. Diffuse fatty infiltration, hypersplenism and gallstones are also commonly seen in these patients. We present a pictorial review of the radiological appearance of these abdominal manifestations. The conditions are dealt with individually, together with typical appearances in various imaging modalities. (orig.)

  15. Abdominal manifestations of cystic fibrosis in children

    Energy Technology Data Exchange (ETDEWEB)

    Chaudry, Gulraiz; Navarro, Oscar M.; Levine, Daniel S.; Oudjhane, Kamaldine [University of Toronto, Department of Diagnostic Imaging, Hospital for Sick Children, Toronto, ON (Canada)

    2006-03-15

    Pulmonary complications remain the main cause of mortality in cystic fibrosis, but the presenting symptoms in children are often related to gastrointestinal or pancreaticobiliary disease. Furthermore, abdominal manifestations are now seen throughout childhood, from infancy to adolescence. The child might present in the neonatal period with meconium ileus or its attendant complications. The older child might present with distal intestinal obstruction syndrome or colonic stricture secondary to high doses of pancreatic enzyme replacement. Less-common gastrointestinal manifestations include intussusception, duodenitis and fecal impaction of the appendix. Most children also show evidence of exocrine pancreatic deficiency. Radiologically, the combination of fat deposition and pancreatic fibrosis leads to varying CT and MR appearances. A higher than normal incidence of pancreatic cysts and calcification is also seen. Decreased transport of water and chloride also increases the viscosity of bile, with subsequent obstruction of the biliary ductules. If extensive, this can progress to obstructive cirrhosis, portal hypertension and esophageal varices. Diffuse fatty infiltration, hypersplenism and gallstones are also commonly seen in these patients. We present a pictorial review of the radiological appearance of these abdominal manifestations. The conditions are dealt with individually, together with typical appearances in various imaging modalities. (orig.)

  16. Inhaled corticosteroids for cystic fibrosis.

    Science.gov (United States)

    Balfour-Lynn, Ian M; Welch, Karen

    2016-08-23

    Reduction of lung inflammation is one of the goals of cystic fibrosis therapy. Inhaled corticosteroids are often used to treat children and adults with cystic fibrosis. The rationale for this is their potential to reduce lung damage arising from inflammation, as well as their effect on symptomatic wheezing. It is important to establish the current level of evidence for the risks and benefits of inhaled corticosteroids, especially in the light of their known adverse effects on growth. This is an update of a previously published review. To assess the effectiveness of taking regular inhaled corticosteroids, compared to not taking them, in children and adults with cystic fibrosis. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register, comprising references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings. We requested information from pharmaceutical companies manufacturing inhaled corticosteroids and authors of identified trials.Date of most recent search of the Group's Trials Register: 15 August 2016. Randomised or quasi-randomised trials, published and unpublished, comparing inhaled corticosteroids to placebo or standard treatment in individuals with cystic fibrosis. Two independent authors assessed methodological quality and risk of bias in trials using established criteria and extracted data using standard pro formas. The searches identified 34 citations, of which 26 (representing 13 trials) were eligible for inclusion. These 13 trials reported the use of inhaled corticosteroids in 506 people with cystic fibrosis aged between six and 55 years. One was a withdrawal trial in individuals who were already taking inhaled corticosteroids. Methodological quality and risk of bias were difficult to assess from published information. Many of the risk of bias judgements were unclear due to a lack of available information. Only two trials specified how

  17. Defects in hepatic Notch signaling result in disruption of the communicating intrahepatic bile duct network in mice

    Directory of Open Access Journals (Sweden)

    Erin E. Sparks

    2011-05-01

    Abnormal Notch signaling in humans results in Alagille syndrome, a pleiotropic disease characterized by a paucity of intrahepatic bile ducts (IHBDs. It is not clear how IHBD paucity develops as a consequence of atypical Notch signaling, whether by a developmental lack of bile duct formation, a post-natal lack of branching and elongation or an inability to maintain formed ducts. Previous studies have focused on the role of Notch in IHBD development, and demonstrated a dosage requirement of Notch signaling for proper IHBD formation. In this study, we use resin casting and X-ray microtomography (microCT analysis to address the role of Notch signaling in the maintenance of formed IHBDs upon chronic loss or gain of Notch function. Our data show that constitutive expression of the Notch1 intracellular domain in bi-potential hepatoblast progenitor cells (BHPCs results in increased IHBD branches at post-natal day 60 (P60, which are maintained at P90 and P120. By contrast, loss of Notch signaling via BHPC-specific deletion of RBP-J (RBP KO, the DNA-binding partner for all Notch receptors, results in progressive loss of intact IHBD branches with age. Interestingly, in RBP KO mice, we observed a reduction in bile ducts per portal vein at P60; no further reduction had occurred at P120. Thus, bile duct structures are not lost with age; instead, we propose a model in which BHPC-specific loss of Notch signaling results in an initial developmental defect resulting in fewer bile ducts being formed, and in an acquired post-natal defect in the maintenance of intact IHBD architecture as a result of irresolvable cholestasis. Our studies reveal a previously unappreciated role for Notch signaling in the post-natal maintenance of an intact communicating IHBD structure, and suggest that liver defects observed in Alagille syndrome patients might be more complex than bile duct paucity.

  18. CFTR expression and organ damage in cystic fibrosis

    Energy Technology Data Exchange (ETDEWEB)

    Tizzano, E. [Hospital de la Santa Creu I Sant Pau, Barcelona (Spain); Chitayat, D.; Buchwald, M. [Hospital for Sick Children, Ontario (Canada)

    1994-09-01

    To assist our understanding of the origin of organ damage caused by cystic fibrosis (CF) disease, we have analyzed the pattern of expression of the CF gene (CFTR). mRNA in situ hybridization analysis was carried out in human fetal, newborn, infant and adult tissues and the abundance of the mRNA was correlated with the known pathology at the various stages of human development. Analysis of the pattern of expression indicates a constitutive level of mRNA in gastrointestinal tissues starting during early development and maintained throughout life. Prenatal respiratory tissues show qualitative and quantitative major differences in comparison to postnatal lung samples. Male reproductive tissues show high levels of expression in the head of the epididymis compared with the rest of the male ducts. Female reproductive tissues show a variable pattern of expression at different stages during fetal development and during puberty probably due to changes in hormonal levels. Gastrointestinal and male reproductive tissues have a consistent pathology at birth, whereas no lung abnormalities have been described in newborns affected by CF. Our results show that there is no exact correlations between organ damage present at birth and the degree of CFTR expression. To explain these observations, we hypothesize that the pathogenesis of organ damage in CF depend on at least three factors: the rate of CFTR-mediated fluid secretion, differences in genotype and environmental factors, such as the amount of macromolecules in the lumen of the ducts. This last element predicts that damage will occur in tissues with high protein loads and low flow rates (e.g. pancreas, epididymis), where the absence of CFTR function leads to obstruction and pathology. Organs that express CFTR but with no significant damage (e.g. prenatal lung, female reproductive tissues), will have a low protein load and a high flow rates.

  19. Percutaneous electrohydraulic lithotripsy of bile duct stones

    International Nuclear Information System (INIS)

    Juliani, E.; Righi, D.; Cesarani, F.; Regge, D.; Gandini, G.

    1988-01-01

    The results are reported of percutaneous transhepatic tratment with an electrohydraulic stone disintegrator in 4 cases of bile duct lithiasis. An electri discharge, generated by a bipolar electrode, gives origin to high-amplitude and low-frequency shoc waves in the fluid medium-which cause the stone to fragment. Complete resolution of lithiasis was obtained in 2 patients with intrahepatic calculosis. In the other 2 cases of massive lithiasis of intra and extrahepatic bile ducts the treatment, however incomplete, proved to be useful, as the biliary flux was reabilitated and clinical symptoms disappeared. No relevant immediate side-effects were observed, except for well-tolerated pain during the discharges, and transitory hemobilia which solved spontaneously. No complications were observed in this series of patients. Electrohydraulic lithotripsy proves thus to be useful for supporting standard interventional radiology techniques in very complex cases of intra and extrahepatic bile duct stones

  20. Dynamic MR cholangiography after fatty meal loading. Cystic contractility and dynamic evaluation of biliary stasis

    International Nuclear Information System (INIS)

    Omata, Takayuki; Saito, Kazuhiro; Kotake, Fumio; Mizokami, Yuji; Matsuoka, Takeshi; Abe, Kimihiko

    2002-01-01

    Dynamic MR cholangiography was conducted on patients with cholelithiasis or choledocholithiasis who had consumed a fatty test meal (Molyork) and the cystic contractility and dynamics of biliary stasis was evaluated. The subjects were 25 with intracystic cholelithiasis, 10 with choledocholithiasis and 10 normal controls. For an imaging sequence, the rapid acquisition with relaxation enhancement (RARE) method was employed and imaging was conducted for 40 min (every 30 s following Molyork administration) without breath-holding. The gallbladder contraction ratio was computed and the contractile ratio for the common bile duct was calculated. To determine the bile flow to the duodenum, the high-intensity signal, indicating the flow from the lower common bile duct, and perfusion of the duodenum were observed in dynamic mode on the monitor with the naked eye and interpreted as positive bile flow. The frequency of this flow was visually monitored. The gallbladder contractile ratio was significantly reduced in patients with cholelithiasis or choledocholithiasis compared with the controls. In a comparison with the normal controls, no sequential changes were noted in the mean contractile ratio of the common bile duct of the patients with cholelithiasis or choledocholithiasis. The mean frequency of bile flow observed for each 40 min period was 13±2.4, 6±2.2, and 4±1.3 times for the controls, those with intracystic cholelithiasis, and those with choledocholithiasis, respectively. Compared with the controls, the latter two patient groups showed evident reductions in the frequency of bile flow to the duodenum (p<0.001). Dynamic MRC combined with Molyork loading makes it possible to compute cystic contractile ratios and perform a dynamic examination of bile flow under non-invasive, near-physiological conditions. (author)

  1. Dynamic MR cholangiography after fatty meal loading. Cystic contractility and dynamic evaluation of biliary stasis

    Energy Technology Data Exchange (ETDEWEB)

    Omata, Takayuki; Saito, Kazuhiro; Kotake, Fumio; Mizokami, Yuji; Matsuoka, Takeshi; Abe, Kimihiko [Tokyo Medical Coll., Ami, Ibaraki (Japan). Kasumigaura Hospital

    2002-07-01

    Dynamic MR cholangiography was conducted on patients with cholelithiasis or choledocholithiasis who had consumed a fatty test meal (Molyork) and the cystic contractility and dynamics of biliary stasis was evaluated. The subjects were 25 with intracystic cholelithiasis, 10 with choledocholithiasis and 10 normal controls. For an imaging sequence, the rapid acquisition with relaxation enhancement (RARE) method was employed and imaging was conducted for 40 min (every 30 s following Molyork administration) without breath-holding. The gallbladder contraction ratio was computed and the contractile ratio for the common bile duct was calculated. To determine the bile flow to the duodenum, the high-intensity signal, indicating the flow from the lower common bile duct, and perfusion of the duodenum were observed in dynamic mode on the monitor with the naked eye and interpreted as positive bile flow. The frequency of this flow was visually monitored. The gallbladder contractile ratio was significantly reduced in patients with cholelithiasis or choledocholithiasis compared with the controls. In a comparison with the normal controls, no sequential changes were noted in the mean contractile ratio of the common bile duct of the patients with cholelithiasis or choledocholithiasis. The mean frequency of bile flow observed for each 40 min period was 13{+-}2.4, 6{+-}2.2, and 4{+-}1.3 times for the controls, those with intracystic cholelithiasis, and those with choledocholithiasis, respectively. Compared with the controls, the latter two patient groups showed evident reductions in the frequency of bile flow to the duodenum (p<0.001). Dynamic MRC combined with Molyork loading makes it possible to compute cystic contractile ratios and perform a dynamic examination of bile flow under non-invasive, near-physiological conditions. (author)

  2. Unusual growth rate during cystic echinococcosis.

    Science.gov (United States)

    Valour, Florent; Khenifer, Safia; Della-Schiava, Nellie; Cotte, Eddy; Guibert, Benoit; Wallon, Martine; Durupt, Stéphane; Durieu, Isabelle

    2014-04-01

    Cystic echinococcosis is a world wild zoonosis caused by Echinococcus granulosus, leading to hepatic and lung cysts with a usually slight growth rate. We report the case of an 82year-old Algerian woman with hepatic and lung cystic echinococcosis with a 10-fold size increase in 6months. Copyright © 2013. Published by Elsevier Ireland Ltd.

  3. Outcome in cystic fibrosis liver disease.

    LENUS (Irish Health Repository)

    Rowland, Marion

    2011-01-01

    Evidence suggests that cystic fibrosis liver disease (CFLD) does not affect mortality or morbidity in patients with cystic fibrosis (CF). The importance of gender and age in outcome in CF makes selection of an appropriate comparison group central to the interpretation of any differences in mortality and morbidity in patients with CFLD.

  4. Laparoscopic cholecystectomy in adult cystic fibrosis.

    LENUS (Irish Health Repository)

    McGrath, D S

    2012-02-03

    Two female patients with Cystic Fibrosis, attending the Adult Regional Cystic Fibrosis centre at the Cork University Hospital, were investigated for upper abdominal pain and found to have gallstones at ultrasonography. Laparoscopic cholecystectomy was performed successfully and, without complication, in both patients.

  5. Self-management education for cystic fibrosis.

    LENUS (Irish Health Repository)

    Savage, Eileen

    2011-01-01

    Self-management education may help patients with cystic fibrosis and their families to choose, monitor and adjust treatment requirements for their illness, and also to manage the effects of illness on their lives. Although self-management education interventions have been developed for cystic fibrosis, no previous systematic review of the evidence of effectiveness of these interventions has been conducted.

  6. European Cystic Fibrosis Society Standards of Care

    DEFF Research Database (Denmark)

    Stern, Martin; Bertrand, Dominique Pougheon; Bignamini, Elisabetta

    2014-01-01

    Since the earliest days of cystic fibrosis (CF) treatment, patient data have been recorded and reviewed in order to identify the factors that lead to more favourable outcomes. Large data repositories, such as the US Cystic Fibrosis Registry, which was established in the 1960s, enabled successful...

  7. Significance of surgical management for cystic prolactinoma.

    Science.gov (United States)

    Ogiwara, Toshihiro; Horiuchi, Tetsuyoshi; Nagm, Alhusain; Goto, Tetsuya; Hongo, Kazuhiro

    2017-04-01

    It is generally accepted that dopamine agonists (DA) represent the first-line treatment for most patients with prolactinoma, and patients become candidates for surgical intervention when DA is contraindicated. Surgical indication for cystic prolactinoma remains controversial. This study was performed to investigate the significance of surgery for cystic prolactinoma. A total of 28 patients that underwent transsphenoidal resection of prolactinoma between February 2004 and May 2016 were reviewed. Five consecutive patients with cystic prolactinoma were included in this study. Our surgical strategy for cystic prolactinoma was categorized as follows: first, when the purpose of surgical resection was normalization of the prolactin level, aggressive resection was performed; second, when volume reduction was essential to relieve the visual symptoms and headache, internal decompression was performed followed by DA therapy. The clinical outcomes were analyzed accordingly. All cystic prolactinoma were resected via the transsphenoidal approach without any complications, and all symptoms including visual impairment and hypogonadal activity were finally relieved combined with medication. Surgery for cystic prolactinoma could be a better option. Transsphenoidal surgery is relatively safe to remove the cystic prolactinoma, additionally it can normalize the prolactine level and achieve adequate and rapid decompression of optic chiasm. The risk of transsphenoidal surgery is highly dependent on the skill of the surgeon and treatment decision for cystic prolactinoma needs to be individualized for each patient.

  8. [Diagnosis and surgical treatment of cystic granuloma].

    Science.gov (United States)

    Piazza Roxas, M; Piazza Roxas, I

    1989-05-01

    The survival therapy of cystic lesion with concomitant cystotomy and apicectomy permits the healing of the inflammatory process and the total restitution of the affected tissues. The diagnostic phase is fundamental, with identification of the cystic lesion for which removal is elective.

  9. Cystic Hydatid Disease in Southern Sudan

    African Journals Online (AJOL)

    Objective: To document the experience with cystic hydatid disease at a teaching hospital in. South Sudan. Design: A prospective analysis of clinical data collected from patients admitted and treated for cystic hydatid disease between August 2004 and August 2007. setting: Juba Teaching Hospital. Patients: One hundred and ...

  10. Xylometazoline hydrochloride nasal spray combined with laser artificial nasolacrimal duct implantation for nasolacrimal duct obstruction

    Directory of Open Access Journals (Sweden)

    Xiao-Zhao Yang

    2017-02-01

    Full Text Available AIM: To study the role of xylometazoline hydrochloride nasal spray in combination therapy of nasolacrimal duct obstruction and to investigate the effect of nasal inflammation on nasolacrimal duct obstruction. METHODS: Totally 279 patients with nasolacrimal duct obstruction were collected, who received lacrimal passage irrigation, CT angiography for lacrimal passage and nasal endoscope before treated by lacrimal laser forming and artificial nasolacrimal duct implantation combined with xylometazoline hydrochloride nasal spray. In group A, 137 patients were treated with antibiotic eye drop and non-steroidal anti-inflammatory drugs after operations. In group B, 142 patients were treated with xylometazoline hydrochloride nasal spray besides the same treatment for group A. RESULTS:In the 279 patients 217(77.8%, in which 105 cases(76.6%were in group A and 112 cases(78.9%were in group B, were suffered with nasal inflammation, including nasal mucosal hyperemia, inferior turbinate hypertrophy, middle turbinate hypertrophy. At 3mo after the ducts were drawn, efficacy of group B was 95.8%, which was significant better than that of group A(86.1%, PCONCLUSION: Nasal inflammation was an important factor in the incidence of nasolacrimal duct obstruction, which shoud pay more attention in the process of diagnosis and treatment. Combination therapy could improve the cure rate of nasolacrimal duct obstruction.

  11. Percutaneous treatment of benign bile duct strictures

    Energy Technology Data Exchange (ETDEWEB)

    Koecher, Martin [Department of Radiology, University Hospital, I.P.Pavlova 6, 775 20 Olomouc (Czech Republic)]. E-mail: martin.kocher@seznam.cz; Cerna, Marie [Department of Radiology, University Hospital, I.P.Pavlova 6, 775 20 Olomouc (Czech Republic); Havlik, Roman [Department of Surgery, University Hospital, I.P.Pavlova 6, 775 20 Olomouc (Czech Republic); Kral, Vladimir [Department of Surgery, University Hospital, I.P.Pavlova 6, 775 20 Olomouc (Czech Republic); Gryga, Adolf [Department of Surgery, University Hospital, I.P.Pavlova 6, 775 20 Olomouc (Czech Republic); Duda, Miloslav [Department of Surgery, University Hospital, I.P.Pavlova 6, 775 20 Olomouc (Czech Republic)

    2007-05-15

    Purpose: To evaluate long-term results of treatment of benign bile duct strictures. Materials and methods: From February 1994 to November 2005, 21 patients (9 men, 12 women) with median age of 50.6 years (range 27-77 years) were indicated to percutaneous treatment of benign bile duct stricture. Stricture of hepatic ducts junction resulting from thermic injury during laparoscopic cholecystectomy was indication for treatment in one patient, stricture of hepaticojejunostomy was indication for treatment in all other patients. Clinical symptoms (obstructive jaundice, anicteric cholestasis, cholangitis or biliary cirrhosis) have appeared from 3 months to 12 years after surgery. Results: Initial internal/external biliary drainage was successful in 20 patients out of 21. These 20 patients after successful initial drainage were treated by balloon dilatation and long-term internal/external drainage. Sixteen patients were symptoms free during the follow-up. The relapse of clinical symptoms has appeared in four patients 9, 12, 14 and 24 months after treatment. One year primary clinical success rate of treatment for benign bile duct stricture was 94%. Additional two patients are symptoms free after redilatation (15 and 45 months). One patient is still in treatment, one patient died during secondary treatment period without interrelation with biliary intervention. The secondary clinical success rate is 100%. Conclusion: Benign bile duct strictures of hepatic ducts junction or biliary-enteric anastomosis are difficult to treat surgically and endoscopically inaccessible. Percutaneous treatment by balloon dilatation and long-term internal/external drainage is feasible in the majority of these patients. It is minimally invasive, safe and effective.

  12. Percutaneous treatment of benign bile duct strictures

    International Nuclear Information System (INIS)

    Koecher, Martin; Cerna, Marie; Havlik, Roman; Kral, Vladimir; Gryga, Adolf; Duda, Miloslav

    2007-01-01

    Purpose: To evaluate long-term results of treatment of benign bile duct strictures. Materials and methods: From February 1994 to November 2005, 21 patients (9 men, 12 women) with median age of 50.6 years (range 27-77 years) were indicated to percutaneous treatment of benign bile duct stricture. Stricture of hepatic ducts junction resulting from thermic injury during laparoscopic cholecystectomy was indication for treatment in one patient, stricture of hepaticojejunostomy was indication for treatment in all other patients. Clinical symptoms (obstructive jaundice, anicteric cholestasis, cholangitis or biliary cirrhosis) have appeared from 3 months to 12 years after surgery. Results: Initial internal/external biliary drainage was successful in 20 patients out of 21. These 20 patients after successful initial drainage were treated by balloon dilatation and long-term internal/external drainage. Sixteen patients were symptoms free during the follow-up. The relapse of clinical symptoms has appeared in four patients 9, 12, 14 and 24 months after treatment. One year primary clinical success rate of treatment for benign bile duct stricture was 94%. Additional two patients are symptoms free after redilatation (15 and 45 months). One patient is still in treatment, one patient died during secondary treatment period without interrelation with biliary intervention. The secondary clinical success rate is 100%. Conclusion: Benign bile duct strictures of hepatic ducts junction or biliary-enteric anastomosis are difficult to treat surgically and endoscopically inaccessible. Percutaneous treatment by balloon dilatation and long-term internal/external drainage is feasible in the majority of these patients. It is minimally invasive, safe and effective

  13. Evaluation of PEGIT duct connection system

    Energy Technology Data Exchange (ETDEWEB)

    Walker, Iain S.; Brenner, Douglas E.; Sherman, Max H.; Dickerhoff, Darryl J.

    2003-08-01

    Most air duct system components are assembled in the field and are mechanically fastened by sheet metal screws (for sheet metal-to-sheet metal) or by drawbands (for flex duct-to-sheet metal). Air sealing is separate from this mechanical fastening and is usually achieved using tape or mastic products after mechanical fastening. Field observations have shown that mechanical fastening rarely meets code or manufacturers requirements and that sealing procedures are similarly inconsistent. To address these problems, Proctor Engineering Group (PEG) is developing a system of joining ducts (called PEGIT) that combines the mechanical fastening and sealing into a single self-contained procedure. The PEGIT system uses a shaped flexible seal between specially designed sheet metal duct fittings to both seal and fasten duct sections together. Figure 1 shows the inner duct fitting complete with rubber seal. This seal provides the air seal for the completed fitting and is shaped to allow the inner and outer fittings to slide together, and then to lock the fittings in place. The illustration in Figure 2 shows the approximate cross section of the rubber seal that shows how the seal has a lip that is angled backwards. This angled lip allows the joint to be pushed together by folding flat but then its long axis makes it stiff in the pulling apart direction. This study was undertaken to assist PEG in some of the design aspects of this system and to test the performance of the PEGIT system. This study was carried out in three phases. The initial phase evaluated the performance of a preliminary seal design for the PEGIT system. After the first phase, the seal was redesigned and this new seal was evaluated in the second phase of testing. The third phase performed more detailed testing of the second seal design to optimize the production tolerances of the sheet metal fittings. This report summarizes our findings from the first two phases and provides details about the third phase of testing.

  14. Salivary duct carcinoma of the palate

    Directory of Open Access Journals (Sweden)

    Ponniah I

    2005-01-01

    Full Text Available Salivary duct carcinoma is a rare high-grade neoplasm that more frequently affects the parotid gland. Though neoplasms of this type are infrequent in minor salivary glands, they are less aggressive and may lead to early diagnosis before distant metastases could occur. Salivary duct carcinoma is also the most frequent epithelial component of carcinosarcoma. The present article reports a case of SDC of the palate in a 26-year-old male and discusses SDC as a malignant epithelial component in carcinosarcoma.

  15. Pulmonary complications of cystic fibrosis

    International Nuclear Information System (INIS)

    Ng, M.Y.; Flight, W.; Smith, E.

    2014-01-01

    The life expectancy of patients with cystic fibrosis (CF) has steadily increased over recent decades with a corresponding increase in the frequency of complications of the disease. Radiologists are increasingly involved with managing and identifying the pulmonary complications of CF. This article reviews the common manifestations of CF lung disease as well as updating radiologists with a number of less well-known complications of the condition. Early and accurate detection of the pulmonary effects of CF are increasingly important to prevent irreversible lung damage and give patients the greatest possibility of benefiting from the new therapies becoming available, which correct the underlying defect causing CF

  16. Liver manifestations of cystic fibrosis

    International Nuclear Information System (INIS)

    Akata, Deniz; Akhan, Okan

    2007-01-01

    Chronic liver disease is one of the major complications of cystic fibrosis (CF). Significant liver disease is seen in 13-25% of children with CF. Improved life expectancy and prolonged follow-up have favored better characterization of the hepatic manifestations of CF and allowed direct observation of an increasing number of liver-related events. Liver disease typically develops in the first decade of life, with the incidence dropping rapidly after the age of 10 years. The wide spectrum of liver disease ranging from asymptomatic gallbladder abnormalities to biliary cirrhosis will be reviewed in this article

  17. Rectal prolapse and cystic fibrosis.

    Science.gov (United States)

    El-Chammas, Khalil I; Rumman, Nisreen; Goh, Vi Lier; Quintero, Diana; Goday, Praveen S

    2015-01-01

    Screening for cystic fibrosis (CF) is suggested in patients with rectal prolapse (RP). Little is known about the association between CF and RP in the era of newborn screening for CF. Our retrospective review showed that 3.6% of patients with RP had CF, and 3.5% of patients with CF had RP. No demographic or clinical factors appear to predict the likelihood of RP in patients with CF. Sweat chloride testing for patients with RP has a low yield in the era of newborn screening but may still need to be considered in children with RP to avoid missing the rare child with CF.

  18. Fan Duct Heat Exchanger for Turbine Cooling Air, Phase I

    Data.gov (United States)

    National Aeronautics and Space Administration — The proposal is for the design of a fan duct heat exchanger in order to improve aircraft fuel burn. The fan duct heat exchanger decreases the temperature of the 15%...

  19. HVAC; Heating, Ventilation, Air Conditioning - Aerosol Duct Sealant

    Science.gov (United States)

    2016-09-01

    climatic conditions and different air distribution systems types. Data on thermal energy and fan power was collected before and after the duct sealant...Expeditionary Warfare Center. This technology internally seals leaks in air distribution ducts by injecting a fog of aerosolized sealant particles...into a pressurized duct system. The product keeps the particles suspended within the air stream. As the duct work is pressurized, the particles

  20. Mechanisms of Lower Bile Duct Stricture in Autoimmune Pancreatitis

    OpenAIRE

    Watanabe, Takayuki; Maruyama, Masahiro; Ito, Tetsuya; Maruyama, Masafumi; Muraki, Takashi; Hamano, Hideaki; Arakura, Norikazu; Hasebe, Osamu; Kawa, Shigeyuki

    2014-01-01

    Objectives We attempted to clarify the mechanism underlying lower bile duct stricture in autoimmune pancreatitis. Methods Imaging and histologic finding of the bile duct were assessed for 73 patients with autoimmune pancreatitis to clarify whether IgG4-related biliary inflammation or pancreatic head swelling is associated with lower bile duct stricture. Results Lower bile duct stricture was found in 59 (81%) patients. Pancreatic head swelling was significantly more frequent among patients wit...

  1. Novel endoscopic management for pancreatic pseudocyst with fistula to the common bile duct.

    Science.gov (United States)

    Crinò, Stefano Francesco; Scalisi, Giuseppe; Consolo, Pierluigi; Varvara, Doriana; Bottari, Antonio; Pantè, Sebastiano; Pallio, Socrate

    2014-12-16

    Pancreatic pseudocyst formation is a well-known complication of pancreatitis. It represents about 75% of the cystic lesions of the pancreas and might be located within or surrounding the pancreatic tissue. Sixty percent of the occurrences resolve spontaneously and only persistent, symptomatic or complicated cysts need to be treated. Complications include infection, hemorrhage, gastric outlet obstruction, splenic infarction and rupture. The formation of fistulas to other viscera is rare and most commonly occurs within the stomach, duodenum or colon. We report a case of a patient with a pancreatic pseudocyst in communication with the common bile duct. There have been only few cases reported in the literature. We successfully managed our case by performing an endoscopic ultrasound-guided drainage of the pancreatic collection and a contemporaneous stenting of the common bile duct. Performed independently, both drainages are effective, safe and well-coded and the expertise on these procedures is widespread. By our knowledge this therapeutic approach was never reported in literature but we retain this is the most correct treatment for this very rare condition.

  2. A case of fascioliasis in common bile duct

    International Nuclear Information System (INIS)

    Ham, Soo Youn; Park, Cheol Min; Chung, Kyu Byung; Lee, Chang Hong; Park, Seung Chul; Choi, Sang Yong; Lim, Han Jong

    1989-01-01

    A case of Fascioliasis of common bile duct is confirmed by visualization of adult fluke. Fascioliasis caused by Fasciola hepatica, is common parasitic disease in cattle and sheep. Human is an accidental host. ERCP demonstrated irregular linear conglomerated filling defects in common bile duct. Through surgical intervention, we found adult flukes of F. hepatica and adenomatous hyperplasia of common bile duct

  3. A case of fascioliasis in common bile duct

    Energy Technology Data Exchange (ETDEWEB)

    Ham, Soo Youn; Park, Cheol Min; Chung, Kyu Byung; Lee, Chang Hong; Park, Seung Chul; Choi, Sang Yong; Lim, Han Jong [Korea University College of Medicine, Seoul (Korea, Republic of)

    1989-10-15

    A case of Fascioliasis of common bile duct is confirmed by visualization of adult fluke. Fascioliasis caused by Fasciola hepatica, is common parasitic disease in cattle and sheep. Human is an accidental host. ERCP demonstrated irregular linear conglomerated filling defects in common bile duct. Through surgical intervention, we found adult flukes of F. hepatica and adenomatous hyperplasia of common bile duct.

  4. Iatrogenic injury of an aberrant right posterior sectoral bile duct

    African Journals Online (AJOL)

    (Figs 1 and 2). A week later, an endoscopic retrograde cholangiopancreatography. (ERCP) examination was performed. This showed no filling of the right posterior sectoral ducts but normal opacification of the other ducts. (Figs 3a and b). These findings led to the diagnosis of an aberrant right posterior sectoral bile duct that ...

  5. Surgical management of Stenson's duct injury using epidural ...

    African Journals Online (AJOL)

    Surgical management of Stenson's duct injury using epidural catheter: A novel technique. ... It is in the form of ductal exposure, laceration, total severing, or crushing of the duct. These conditions are difficult to ... Many techniques have been proposed for diagnosis and management of parotid duct injuries. This article ...

  6. Termination pattern of the main and accessory pancreatic ducts ...

    African Journals Online (AJOL)

    The pancreas has the main and occasionally a patent accessory pancreatic ducts that carry secretions to the duodenum. Anatomical studies on these ducts are limited. The present study observed the anatomy of the two ducts in eighty six Tanzanians' autopsy subjects to find out if the pattern is similar to those reported from ...

  7. Patent nasopalatine duct: a diagnostic pitfall

    NARCIS (Netherlands)

    Valstar, M.H.; van den Akker, H.P.

    2008-01-01

    We describe a patient in whom the combination of excessive air in the maxillary sinus and the presence of a patent nasopalatine duct led to misdiagnosis and subsequent treatment of a non-existent oroantral perforation. When a radiograph indicates that antral involvement during removal of a maxillary

  8. Whistler instability in a magnetospheric duct

    Energy Technology Data Exchange (ETDEWEB)

    Talukdar, I.; Tripathi, V.K. (Indian Inst. of Tech., New Delhi (India). Dept. of Physics); Jain, V.K. (Jawaharlal Nehru Univ., New Delhi (India). School of Environmental Sciences)

    1989-04-01

    A whistler wave propagating through a preformed magnetospheric duct is susceptible to growth/amplification by an electron beam. The interaction is non-local and could be of Cerenkov or slow-cyclotron type. First-order perturbation theory is employed to obtain the growth rate for flat and Gaussian beam densities. (author).

  9. Ducted wind turbines : A potential energy shaper

    NARCIS (Netherlands)

    Dighe, V.V.

    2016-01-01

    In order to harvest wind resources more efficiently and to the greatest extent possible, unconventional wind turbine designs have been proposed, but never gained any acceptance in the marketplace. A team of researchers from TU Delft plans to revisit the concept of ducted wind turbines, which have

  10. Gene therapy for cystic fibrosis.

    Science.gov (United States)

    Mueller, Christian; Flotte, Terence R

    2008-12-01

    Cystic Fibrosis (CF) is an autosomal recessive disorder due to mutations in the CF transmembrane conductance regulator (CFTR) gene that lead to defective ion transport in the conducting pulmonary airways and exocrine glands. Through a process that is not fully understood, CFTR defects predispose affected patients to chronic endobronchial infections with organisms such as Pseudomonas aeruginosa and Staphylococcus aureus. Following the discovery of the CFTR gene in 1989, CF became one of the primary targets for gene therapy research. Early enthusiasm surrounded the new field of gene therapy during most of the 1990s and it led academics and clinicians on a big effort to apply gene therapy for cystic fibrosis. Clinical studies have been pursued using recombinant adenovirus, recombinant adeno-associated virus, cationic liposomes, and cationic polymer vectors. Although to this date no dramatic therapeutic benefits have been observed, a lot of information has been gained from the pre-clinical and clinical studies that were performed. This learning curve has led to the optimization of vector technology and an appreciation of immune and mechanical barriers that have to be overcome for successful delivery.

  11. Parenting adolescents with cystic fibrosis

    DEFF Research Database (Denmark)

    Bregnballe, Vibeke; Schiøtz, Peter Oluf; Lomborg, Kirsten

    2011-01-01

    Background: When suffering from cystic fibrosis (CF), a number of problems may arise during adolescence; for example, poor adherence. The problems may be attributed to the adolescent being insufficiently prepared for adult life. Research on different ways of parenting adolescents with CF and the ......Background: When suffering from cystic fibrosis (CF), a number of problems may arise during adolescence; for example, poor adherence. The problems may be attributed to the adolescent being insufficiently prepared for adult life. Research on different ways of parenting adolescents with CF...... and the influence of different parenting styles on the adolescents’ adherence to treatment is still limited. Aim: The aim of this study was to identify the types of parental support that adolescents and young adults with CF want and find helpful in terms of preparing them for adult life. Methods: Sixteen Danish...... was conducted. Results: The adolescents and young adults wanted their parents educated about the adolescent experience. They wanted their parents to learn a pedagogical parenting style, to learn to trust them, and to learn to gradually transfer responsibility for their medical treatment. Additionally...

  12. Vitamin D deficiency as a risk factor for cystic fibrosis-related diabetes in the Scandinavian Cystic Fibrosis Nutritional Study

    DEFF Research Database (Denmark)

    Pincikova, T; Nilsson, Kristine Kahr; Moen, I E

    2011-01-01

    Many cystic fibrosis patients are vitamin D-insufficient. Cystic fibrosis-related diabetes is a major complication of cystic fibrosis. The literature suggests that vitamin D might possess certain glucose-lowering properties. We aimed to assess the relationship between vitamin D and cystic fibrosis...

  13. Rapidly Evoluting Congenital Cystic Neuroblastoma in a Neonate

    Energy Technology Data Exchange (ETDEWEB)

    Yun, Tae Jun; Kim, Myung Jun; Han, Seok Joo; Lee, Mi Jung [Severance Children' s Hospital, Yonsei University, College of Medicine, Seoul(Korea, Republic of)

    2012-08-15

    Perinatal detection of neonatal suprarenal masses has increased. Here, we report an unusual case of an adrenal cystic neuroblastoma that presented as a purely cystic lesion upon initial postnatal ultrasonography (US) and showed rapid evolution to a mixed cystic and solid mass during follow-up US and MRI. We suggest a short-term (two weeks) follow-up US for neonatal adrenal cystic lesions, even if they appear as purely cystic.

  14. Research advances in sump syndrome

    Directory of Open Access Journals (Sweden)

    XU Yu

    2017-08-01

    Full Text Available Sump syndrome is a rare complication of side-to-side choledochoduodenostomy (CDD and occasionally occurs after spontaneous gallbladder-bile duct-digestive tract fistula or end-to-side choledochojejunostomy. Before the development of minimally invasive surgery, conventional surgical operation used to be the most important treatment method. This article reviews the research advances in sump syndrome in recent years and points out that endoscopic retrograde cholangiopancreatography is the major diagnostic method for this disease, and endoscopic sphincterotomy combined with bile duct debridement is the most simple and effective measure for the treatment of sump syndrome. Meanwhile, this article briefly reviews sump syndrome with reference to related literature and clinical practice, in order to raise the awareness for sump syndrome.

  15. Are Ducted Mini-Splits Worth It?

    Energy Technology Data Exchange (ETDEWEB)

    Winkler, Jonathan M [National Renewable Energy Laboratory (NREL), Golden, CO (United States); Maguire, Jeffrey B [National Renewable Energy Laboratory (NREL), Golden, CO (United States); Metzger, Cheryn E. [Pacific Northwest National Laboratory; Zhang, Jason [Pacific Northwest National Laboratory

    2018-02-01

    Ducted mini-split heat pumps are gaining popularity in some regions of the country due to their energy-efficient specifications and their ability to be hidden from sight. Although product and install costs are typically higher than the ductless mini-split heat pumps, this technology is well worth the premium for some homeowners who do not like to see an indoor unit in their living area. Due to the interest in this technology by local utilities and homeowners, the Bonneville Power Administration (BPA) has funded the Pacific Northwest National Laboratory (PNNL) and the National Renewable Energy Laboratory (NREL) to develop capabilities within the Building Energy Optimization (BEopt) tool to model ducted mini-split heat pumps. After the fundamental capabilities were added, energy-use results could be compared to other technologies that were already in BEopt, such as zonal electric resistance heat, central air source heat pumps, and ductless mini-split heat pumps. Each of these technologies was then compared using five prototype configurations in three different BPA heating zones to determine how the ducted mini-split technology would perform under different scenarios. The result of this project was a set of EnergyPlus models representing the various prototype configurations in each climate zone. Overall, the ducted mini-split heat pumps saved about 33-60% compared to zonal electric resistance heat (with window AC systems modeled in the summer). The results also showed that the ducted mini-split systems used about 4% more energy than the ductless mini-split systems, which saved about 37-64% compared to electric zonal heat (depending on the prototype and climate).

  16. Tolerance of bile duct to intraoperative irradiation

    International Nuclear Information System (INIS)

    Sindelar, W.F.; Tepper, J.; Travis, E.L.

    1982-01-01

    In order to determine the effects of intraoperative radiation therapy of the bile duct and surrounding tissues, seven adult dogs were subjected to laparotomy and intraoperative irradiation with 11 MeV electrons. Two animals were treated at each dose level of 2000, 3000, and 4500 rads. A single dog which received a laparotomy and sham irradiation served as a control. The irradiation field consisted of a 5 cm diameter circle encompassing the extrahepatic bile duct, portal vein, hepatic artery, and lateral duodenal wall. The animals were followed clinically for mor than 18 months after treatment, and autopsies were performed on dogs that died to assess radiation-induced complications or tissue damage. All dogs developed fibrosis and mural thickening of the common duct, which appeared by 6 weeks following irradiation and which was dose-related, being mild at low doses and more severe at high doses. Hepatic changes were seen as early as 6 weeks after irradiation, consisting of periportal inflammation and fibrosis. The hepatic changes appeared earliest at the highest doses. Frank biliary cirrhosis eventually developed at all dose levels. Duodenal fibrosis appeared in the irradiation portal, being most severe at the highest doses and in some animals resulting in duodenal obstruction. No changes were observed in irradiated portions of portal vein and hepatic artery at any dose level. It was concluded that intraoperative radiation therapy delivered to the region of the common duct leads to ductal fibrosis, partial biliary obstruction with secondary hepatic changes, and duodenal fibrosis if bowel wall is included in the field. Clinical use of intraoperative radiation therapy to the bile duct in humans may require routine use of biliary and duodenal bypass to prevent obstructive complications

  17. Structure and function of the cystic fibrosis transmembrane conductance regulator

    Directory of Open Access Journals (Sweden)

    M.M. Morales

    1999-08-01

    Full Text Available Cystic fibrosis (CF is a lethal autosomal recessive genetic disease caused by mutations in the CF transmembrane conductance regulator (CFTR. Mutations in the CFTR gene may result in a defective processing of its protein and alter the function and regulation of this channel. Mutations are associated with different symptoms, including pancreatic insufficiency, bile duct obstruction, infertility in males, high sweat Cl-, intestinal obstruction, nasal polyp formation, chronic sinusitis, mucus dehydration, and chronic Pseudomonas aeruginosa and Staphylococcus aureus lung infection, responsible for 90% of the mortality of CF patients. The gene responsible for the cellular defect in CF was cloned in 1989 and its protein product CFTR is activated by an increase of intracellular cAMP. The CFTR contains two membrane domains, each with six transmembrane domain segments, two nucleotide-binding domains (NBDs, and a cytoplasmic domain. In this review we discuss the studies that have correlated the role of each CFTR domain in the protein function as a chloride channel and as a regulator of the outwardly rectifying Cl- channels (ORCCs.

  18. The Study for Results of Complex Cystic Breast Masses by Biopsy on Ultrasound

    Energy Technology Data Exchange (ETDEWEB)

    Kang, Hye Kyoung [Dept. of Radiology, Yangji General Hospital, Kwangju (Korea, Republic of); Dong, Kyung Rae [Dept. of Radiological Technology, Gwangju Health College, Kwangju (Korea, Republic of)

    2008-06-15

    We examined the roles of Ultrasonography conductors by analyzing the results of tissue biopsy of complex cystic masse under the guidance of breast US. This study was performed to a group of 178 who showed breast US indicating complex cystic masses among 342 patients who were definitely diagnosed by tissue biopsies and operations in our hospital from June 30th, 2003 to June 30th, 2007. The evaluation of tissues around, calcification, the distribution state of blood flow were excluded from the analysis subjects and logic 200 made by GE corporation and gun for core biopsy(Kimal corp., K7/MBD23) were used in this study. The biopsy results of 178 subjects showed FCC (fibrocystic change)(n=56 : 31.4%), Fibrosis (n=41 : 23.0%), Fibroadenoma (n=20 : 11.2%), Epithelial hyperplasia (n=17 : 9.6%), Carcinoma (n=15 : 8.4%), Fibroadipose (n=8 : 4.5%), Sclerosing adenosis (n=7 : 3.9%), Duct ectasia (n=5 : 2.8%), Papiloma (n=5 : 2.8%), and Fat necrosis (n=1 : 0.6%), Hemangioma (n=1 : 0.6%), Abscess (n=1 : 0.6%), Dystrophic calcification(n=1 : 0.6%). The US showed that the results of the tissue biopsy of complex cystic masses were mostly carcinoma(8.4%). Most of them were benign and only 9.6% of epithelial hyperplasia which has high progression rate into malignant tumors epidemically showed malignancy. Most of them were included in the spectrum of fibrous cystic nodule. Even though these results are confirmed, further studies are required. As a result, a nodule which is not certified by US should be right to take the tissue biopsy, but if it's difficult due to patients or another reasons, re-check tests in three months are required. And systemic ultrasonography evaluation should be well recognized to conduct more careful and specific tests.

  19. Patterns of failure and prognostic factors in resected extrahepatic bile duct cancer: implication for adjuvant radiotherapy

    Energy Technology Data Exchange (ETDEWEB)

    Koo, Tae Ryool; Eom, Keun Yong; Kim, In Ah; Cho, Jai Young; Yoon, Yoo Seok; Hwang, Dae Wook; Han, Ho Seong; Kim, Jae Sung [Seoul National University College of Medicine, Seoul (Korea, Republic of)

    2014-06-15

    To find the applicability of adjuvant radiotherapy for extrahepatic bile duct cancer (EBDC), we analyzed the pattern of failure and evaluate prognostic factors of locoregional failure after curative resection without adjuvant treatment. In 97 patients with resected EBDC, the location of tumor was classified as proximal (n = 26) and distal (n = 71), using the junction of the cystic duct and common hepatic duct as the dividing point. Locoregional failure sites were categorized as follows: the hepatoduodenal ligament and tumor bed, the celiac artery and superior mesenteric artery, and other sites. The median follow-up time was 29 months for surviving patients. Three-year locoregional progression-free survival, progression-free survival, and overall survival rates were 50%, 42%, and 52%, respectively. Regarding initial failures, 79% and 81% were locoregional failures in proximal and distal EBDC patients, respectively. The most common site was the hepatoduodenal ligament and tumor bed. In the multivariate analysis, perineural invasion was associated with poor locoregional progression-free survival (p = 0.023) and progression-free survival (p = 0.012); and elevated postoperative CA19-9 (> or =37 U/mL) did with poor locoregional progression-free survival (p = 0.002), progression-free survival (p < 0.001) and overall survival (p < 0.001). Both proximal and distal EBDC showed remarkable proportion of locoregional failure. Perineural invasion and elevated postoperative CA19-9 were risk factors of locoregional failure. In these patients with high risk of locoregional failure, adjuvant radiotherapy could be considered to improve locoregional control.

  20. CT diagnosis of cystic ovarian lesions

    International Nuclear Information System (INIS)

    Iio, Kazuto; Shinmura, Ryoji; Arima, Naomi; Yamada, Eiichiro; Ohkubo, Koichi; Nagata, Yukihiro

    1985-01-01

    CT was undertaken and CT numbers were measured in 47 patients with cystic ovarian lesions. CT features particularly for chocolate cyst revealed the uniform thickness of the whole cystic wall, findings suggesting adhesion to the surrounding organs, circular or oval shape, and higher CT numbers within the cyst than those in the other cystic ovarian lesions. However, because these features are not always observed in cases of chocolate cyst, one should not rely solely on CT findings in the diagnosis of chocolate cyst. (Namekawa, K.)

  1. Inhaled mannitol for cystic fibrosis.

    Science.gov (United States)

    Nevitt, Sarah J; Thornton, Judith; Murray, Clare S; Dwyer, Tiffany

    2018-02-09

    Several agents are used to clear secretions from the airways of people with cystic fibrosis. Mannitol increases mucociliary clearance, but its exact mechanism of action is unknown. The dry powder formulation of mannitol may be more convenient and easier to use compared with established agents which require delivery via a nebuliser. Phase III trials of inhaled dry powder mannitol for the treatment of cystic fibrosis have been completed and it is now available in Australia and some countries in Europe. This is an update of a previous review. To assess whether inhaled dry powder mannitol is well tolerated, whether it improves the quality of life and respiratory function in people with cystic fibrosis and which adverse events are associated with the treatment. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register which comprises references identified from comprehensive electronic databases, handsearching relevant journals and abstracts from conferences.Date of last search: 28 September 2017. All randomised controlled studies comparing mannitol with placebo, active inhaled comparators (for example, hypertonic saline or dornase alfa) or with no treatment. Authors independently assessed studies for inclusion, carried out data extraction and assessed the risk of bias in included studies. The quality of the evidence was assessed using GRADE. Six studies (reported in 50 publications) were included with a total of 784 participants.Duration of treatment in the included studies ranged from 12 days to six months, with open-label treatment for an additional six months in two of the studies. Five studies compared mannitol with control (a very low dose of mannitol or non-respirable mannitol) and the final study compared mannitol to dornase alfa alone and to mannitol plus dornase alfa. Two large studies had a similar parallel design and provided data for 600 participants, which could be pooled where data for a particular outcome and time point were

  2. Transition duct with divided upstream and downstream portions

    Energy Technology Data Exchange (ETDEWEB)

    McMahan, Kevin Weston; LeBegue, Jeffrey Scott; Maldonado, Jaime Javier; Dillard, Daniel Jackson; Flanagan, James Scott

    2015-07-14

    Turbine systems are provided. In one embodiment, a turbine system includes a transition duct comprising an inlet, an outlet, and a duct passage extending between the inlet and the outlet and defining a longitudinal axis, a radial axis, and a tangential axis. The outlet of the transition duct is offset from the inlet along the longitudinal axis and the tangential axis. The duct passage includes an upstream portion extending from the inlet and a downstream portion extending from the outlet. The turbine system further includes a rib extending from an outer surface of the duct passage, the rib dividing the upstream portion and the downstream portion.

  3. Parotid Duct Repair with Intubation Tube: Technical Note

    Science.gov (United States)

    Öztürk, Muhammed Beşir; Barutca, Seda Asrufoğlu; Keskin, Elif Seda; Atik, Bekir

    2017-01-01

    The parotid duct can be damaged in traumatic injuries and surgical interventions. Early diagnosis and treatment of a duct injury is of great importance because complications such as sialocele and salivary gland fistula may develop if the duct is not surgically repaired. We think the cuff of an intubation tube is an ideal material in parotid duct repair, because of its technical characteristics, easiness of availability, and low-cost. In this paper, we described the use of the cuff cannula of an intubation tube for the diagnosis and treatment of parotid duct laceration, as a low-cost and easy to access material readily available in every operating room. PMID:28713751

  4. Causes and prevention of laparoscopic bile duct injuries: analysis of 252 cases from a human factors and cognitive psychology perspective.

    Science.gov (United States)

    Way, Lawrence W; Stewart, Lygia; Gantert, Walter; Liu, Kingsway; Lee, Crystine M; Whang, Karen; Hunter, John G

    2003-04-01

    To apply human performance concepts in an attempt to understand the causes of and prevent laparoscopic bile duct injury. Powerful conceptual advances have been made in understanding the nature and limits of human performance. Applying these findings in high-risk activities, such as commercial aviation, has allowed the work environment to be restructured to substantially reduce human error. The authors analyzed 252 laparoscopic bile duct injuries according to the principles of the cognitive science of visual perception, judgment, and human error. The injury distribution was class I, 7%; class II, 22%; class III, 61%; and class IV, 10%. The data included operative radiographs, clinical records, and 22 videotapes of original operations. The primary cause of error in 97% of cases was a visual perceptual illusion. Faults in technical skill were present in only 3% of injuries. Knowledge and judgment errors were contributory but not primary. Sixty-four injuries (25%) were recognized at the index operation; the surgeon identified the problem early enough to limit the injury in only 15 (6%). In class III injuries the common duct, erroneously believed to be the cystic duct, was deliberately cut. This stemmed from an illusion of object form due to a specific uncommon configuration of the structures and the heuristic nature (unconscious assumptions) of human visual perception. The videotapes showed the persuasiveness of the illusion, and many operative reports described the operation as routine. Class II injuries resulted from a dissection too close to the common hepatic duct. Fundamentally an illusion, it was contributed to in some instances by working too deep in the triangle of Calot. These data show that errors leading to laparoscopic bile duct injuries stem principally from misperception, not errors of skill, knowledge, or judgment. The misperception was so compelling that in most cases the surgeon did not recognize a problem. Even when irregularities were identified

  5. Parotid Gland Biopsy, the Alternative Way to Diagnose Sjogren Syndrome

    NARCIS (Netherlands)

    Spijkervet, Fred. K.L.; Haacke, Erlin; Kroese, Frans G. M.; Bootsma, Hendrika; Vissink, Arjan

    Salivary gland biopsy is a technique broadly applied for the diagnosis of Sjogren syndrome (SS), lymphoma in SS, and connective tissue disorders (sarcoidosis, amyloidosis). In SS characteristic histology findings are found, including lymphocytic infiltration surrounding the excretory ducts in

  6. A study on CT features of intrahepatic bile duct abscess

    International Nuclear Information System (INIS)

    Min Pengqiu; Li Peng; He Zhiyan; Chen Weixia; Liu Yan

    2001-01-01

    Objective: To evaluate CT features of intrahepatic bile duct abscess (IBDA) and its pathologic basis. Methods: The CT imaging data of 31 consecutive cases of intrahepatic bile duct abscess proved by surgery or clinical treatments from October 1989 to February 1999 were retrospectively studied. The causes included acute obstructive suppurative cholangitis and retrograde infection due to different etiologies. For all the cases, the CT manifestations of liver abscess, bile duct abnormalities, and their relationship were observed respectively. Results: Manifestations of liver abscess were revealed in all cases (31/31, 100%). The CT manifestations of bile duct abnormalities included signs of etiologies caused bile duct obstruction and other signs including cholangiectasis (29/31, 93.5%), the dilated bile ducts communicated with (5/31, 16.1%) or abut on (8/31, 25.8%) the abscesses, and gas collection in bile ducts (10/31, 32.2%). The signs showing the relationship between liver abscess and bile duct abnormalities were that the abscesses complied with the obstructive site and the dilated bile ducts (15/31, 48.4%), and the liver abscesses located in different (7/31, 22.6%) or same (4/31, 12.9%) liver lobes or segments with gas collection in the dilated bile ducts. Conclusion: The CT manifestations of IBDA included signs of liver abscess, abnormalities of bile ducts, and signs showing their relationship. CT scanning was helpful in making comprehensive and accurate diagnosis of IBDA

  7. Dispersion properties of ducted whistlers, generated by lightning discharge

    Directory of Open Access Journals (Sweden)

    D. L. Pasmanik

    2005-06-01

    Full Text Available Whistler-mode wave propagation in magnetospheric ducts of enhanced cold plasma density is studied. The case of the arbitrary ratio of the duct radius to the whistler wavelength is considered, where the ray-tracing method is not applicable. The set of duct eigenmodes and their spatial structure are analysed and dependencies of eigenmode propagation properties on the duct characteristics are studied. Special attention is paid to the analysis of the group delay time of one-hop propagation of the whistler wave packet along the duct. We found that, in contrast to the case of a wide duct, the group delay time in a rather narrow duct decreases as the eigenmode number increases. The results obtained are suggested for an explanation of some types of multi-component whistler signals.

  8. Coupled parametric design of flow control and duct shape

    Science.gov (United States)

    Florea, Razvan (Inventor); Bertuccioli, Luca (Inventor)

    2009-01-01

    A method for designing gas turbine engine components using a coupled parametric analysis of part geometry and flow control is disclosed. Included are the steps of parametrically defining the geometry of the duct wall shape, parametrically defining one or more flow control actuators in the duct wall, measuring a plurality of performance parameters or metrics (e.g., flow characteristics) of the duct and comparing the results of the measurement with desired or target parameters, and selecting the optimal duct geometry and flow control for at least a portion of the duct, the selection process including evaluating the plurality of performance metrics in a pareto analysis. The use of this method in the design of inter-turbine transition ducts, serpentine ducts, inlets, diffusers, and similar components provides a design which reduces pressure losses and flow profile distortions.

  9. Cystic fibrosis-related diabetes

    DEFF Research Database (Denmark)

    Andersen, Henrik Ullits; Lanng, Susanne; Pressler, Tania

    2006-01-01

    OBJECTIVE: Cystic fibrosis (CF)-related diabetes has been regarded as a mild form of diabetes with a low risk of severe diabetes complications. The prevalence of CF-related diabetes increases with age, resulting in a 50% prevalence of diabetes at age 30 years. We sought to investigate whether...... microvascular complications in CF-related diabetes appear with a relevant frequency. RESEARCH DESIGN AND METHODS: Thirty-eight patients aged 30 (range 18-55) years with CF-related diabetes for 20 (0-31) years were screened for diabetes complications. Because of chronic pulmonary infections, the majority...... of diabetic retinopathy was found in patients with insulin-treated CF-related diabetes, stressing the need for a regular screening program as in type 1 diabetes. Severely impaired kidney function was common in lung transplant patients, probably secondary to cyclosporine treatment....

  10. SNM holdup assessment of Los Alamos exhaust ducts

    International Nuclear Information System (INIS)

    Marshall, R.S.

    1994-02-01

    Fissile material holdup in glovebox and fume hood exhaust ducting has been quantified for all Los Alamos duct systems. Gamma-based, nondestructive measurements were used to quantify holdup. The measurements were performed during three measurement campaigns. The first campaign, Phase I, provided foot-by-foot, semiquantitative measurement data on all ducting. These data were used to identify ducting that required more accurate (quantitative) measurement. Of the 280 duct systems receiving Phase I measurements, 262 indicated less than 50 g of fissile holdup and 19 indicated fissile holdup of 50 or more grams. Seven duct systems were measured in a second campaign, called Series 1, Phase II. Holdup estimates on these ducts ranged from 421 g of 235 U in a duct servicing a shut-down uranium-machining facility to 39 g of 239 Pu in a duct servicing an active plutonium-processing facility. Measurements performed in the second campaign proved excessively laborious, so a third campaign was initiated that used more efficient instrumentation at some sacrifice in measurement quality. Holdup estimates for the 12 duct systems measured during this third campaign ranged from 70 g of 235 U in a duct servicing analytical laboratories to 1 g of 235 U and 1 g of 239 Pu in a duct carrying exhaust air to a remote filter building. These quantitative holdup estimates support the conclusion made at the completion of the Phase I measurements that only ducts servicing shut-down uranium operations contain about 400 g of fissile holdup. No ventilation ducts at Los Alamos contain sufficient fissile material holdup to present a criticality safety concern

  11. Nutrition in Cystic Fibrosis: Macro- and Micronutrients

    NARCIS (Netherlands)

    Oudshoorn, Johanna Hermiena

    2006-01-01

    Cystic fibrosis (CF) is the most common life-threatening autosomal recessive inherited disease in Caucasians, and is characterized by progressive lung disease, pancreatic insufficiency, malnutrition, hepatobiliary disease and elevated sweat electrolyte levels. The increased survival of CF patients

  12. Cystic fibrosis : a hereditary inflammatory process

    NARCIS (Netherlands)

    Terheggen-Lagro, S.W.J.

    2006-01-01

    Cystic fibrosis (CF) lung disease is characterized by an exaggerated, and extended inflammation, with a massive influx of neutrophils and high concentrations of interleukin (IL)-8 in bronchoalveolar lavage fluid (BALF). Furthermore, patients with CF have chronic bacterial airway infection,

  13. Preterm twin gestation and cystic periventricular leucomalacia

    NARCIS (Netherlands)

    Resch, B; Jammernegg, A; Vollaard, E; Maurer, U; Mueller, WD; Pertl, B

    Objective: To identify risk factors for the development of cystic periventricular leucomalacia (PVL) in twin gestation. Design: Retrospective case-control study. Setting: Tertiary care university hospital, Department of Paediatrics, Division of Neonatology, Graz, Austria. Patients: Preterm twin

  14. Liver Transplantation for Cirrhosis in Cystic Fibrosis

    Directory of Open Access Journals (Sweden)

    T Lamireau

    2006-01-01

    Full Text Available BACKGROUND: Liver disease is the third most common cause of death in children with cystic fibrosis (CF. Liver transplantation is an effective treatment in children with hepatic failure.

  15. Two Qatari siblings with cystic fibrosis and apparent mineralocorticoid excess

    Directory of Open Access Journals (Sweden)

    Khalid Zahraldin

    2015-01-01

    Full Text Available Cystic fibrosis (CF and apparent mineralocorticoid excess (AME syndrome are both autosomal recessive disorders that result from mutations of specific identified genes for each condition. CF is caused by defects in the Cystic fibrosis trans membrane conductance regulator (CFTR gene which encodes for a protein that functions as a chloride channel and regulates the flow of other ions across the apical surface of epithelial cells. AME is due to the deficiency of 11β-hydroxysteroid dehydrogenase type 2 enzyme (11βHSD2, which is responsible for the peripheral inactivation of cortisol to cortisone. Cortisol excess stimulates the mineralocoritoid receptors (MR resulting in intense sodium retention, hypokalemia and hypertension. We report on a consanguineous Arab family, in which two sibs inherited both CF and AME. Gene testing for AME revealed previously unreported mutation in the 11βHSD2 gene. This report draws attention to the importance of recognizing the possibility of two recessive disorders in the same child in complex consanguineous families. Moreover, it provides a unique opportunity to highlight the implications of the coexistence of two genetic disorders on patient care and genetic counseling of the family.

  16. Adenoid cystic carcinoma of the lacrimal gland

    DEFF Research Database (Denmark)

    von Holstein, Sarah L; Fehr, André; Persson, Marta

    2013-01-01

    To investigate genetic alterations in lacrimal gland adenoid cystic carcinomas (ACCs) with emphasis on the MYB-NFIB fusion oncogene and its downstream targets, MYB rearrangements, and copy number alterations in relation to clinical data and survival.......To investigate genetic alterations in lacrimal gland adenoid cystic carcinomas (ACCs) with emphasis on the MYB-NFIB fusion oncogene and its downstream targets, MYB rearrangements, and copy number alterations in relation to clinical data and survival....

  17. Cystic Fibrosis and gastroesophageal reflux disease.

    Science.gov (United States)

    Maqbool, Asim; Pauwels, Ans

    2017-11-01

    Gastroesophageal reflux is common in children and adults with cystic fibrosis (CF). Pathological gastroesophageal reflux disease (GERD) is also frequent in patients of all ages with CF. This article reviews the pathophysiology, diagnostic work-up, management options, complications, and future directions in the evaluation and management of GERD - unique to and pertinent for - patients with CF in particular. Copyright © 2017 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.

  18. Congenital cystic adenomatoid lung malformation of newborn

    International Nuclear Information System (INIS)

    Reither, M.; Peltner, H.U.; Weigel, W.; Braune, M.; Heiming, E.

    1980-01-01

    The congenital cystic adenomatoid malformation (CCAM) of the newborn is a particular form among the cystic disorders of the lung. The clinical findings, illustrated by four cases, and especially the roentgenographic symptoms are typical. Different radiologic examinations, including the computertomography, are discussed. The differential diagnosis of the disease is various, and therefore a correct and on time diagnosis is necessary, because the prognosis of the patient depends on an adequate therapy. (orig.) [de

  19. Festival food coma in cystic fibrosis.

    Science.gov (United States)

    Pandit, Chetan; Graham, Christie; Selvadurai, Hiran; Gaskin, Kevin; Cooper, Peter; van Asperen, Peter

    2013-07-01

    Children with cystic fibrosis liver disease and portal hypertension are at risk of developing acute hepatic encephalopathy. Even in the presence of normal synthetic liver function these children may have porto-systemic shunting. We report a case of an adolosecent who had cystic fibrosis liver disease and presented with life threatening hepatinc encephalopathy. This case illustrates that it is necessary to consider an appropriate dietary regimen in adolosecents with liver disease to prevent hepatic decompensation. Copyright © 2012 Wiley Periodicals, Inc.

  20. The adenosine A2B receptor is involved in anion secretion in human pancreatic duct Capan-1 epithelial cells

    DEFF Research Database (Denmark)

    Hayashi, M.; Inagaki, A.; Novak, Ivana

    2016-01-01

    Adenosine modulates a wide variety of biological processes via adenosine receptors. In the exocrine pancreas, adenosine regulates transepithelial anion secretion in duct cells and is considered to play a role in acini-to-duct signaling. To identify the functional adenosine receptors and Cl...... by CFTRinh-172, a cystic fibrosis transmembrane conductance regulator (CFTR) Cl− channel inhibitor. The adenosine A2B receptor agonist, BAY 60-6583, increased Isc and whole-cell Cl− currents through CFTR Cl− channels, whereas the A2A receptor agonist, CGS 21680, had negligible effects. The A2B receptor....... These results demonstrate that luminal adenosine regulates anion secretion by activating CFTR Cl− channels via adenosine A2B receptors on the luminal membranes of Capan-1 cells. The present study endorses that purinergic signaling is important in the regulation of pancreatic secretion....

  1. Isolated chyle duct injury in blunt trauma.

    Science.gov (United States)

    Dissanaike, Sharmila; Griswold, John A; Halldorsson, Ari; Frezza, Eldo E

    2006-02-01

    We present a case of a patient sustaining an isolated injury to the right main branch of the cysterna chyli due to a high-speed motor vehicle accident. A 42-year-old man presented after a high-speed collision. CT revealed a collection of hypodense fluid in the gallbladder fossa, which was the clue to take him to the OR. We proceeded to laparoscopic exploration, and based on the milky white color of the fluid, identified a chyle leak. In an open fashion, the retroperitoneum was explored and the injury was identified as disruption of the right lumbar branch entering the cisterna chyli, and this was ligated with silk ties. Chyle duct injury secondary to blunt trauma is a rare finding. The use of CT imaging can identify this injury. Laparoscopy can confirm the injury. Open ligation of the injured duct is the best treatment.

  2. Acoustic Power Transmission Through a Ducted Fan

    Science.gov (United States)

    Envia, Ed

    2016-01-01

    For high-speed ducted fans, when the rotor flowfield is shock-free, the main contribution to the inlet radiated acoustic power comes from the portion of the rotor stator interaction sound field that is transmitted upstream through the rotor. As such, inclusion of the acoustic transmission is an essential ingredient in the prediction of the fan inlet noise when the fan tip relative speed is subsonic. This paper describes a linearized Euler based approach to computing the acoustic transmission of fan tones through the rotor. The approach is embodied in a code called LINFLUX was applied to a candidate subsonic fan called the Advanced Ducted Propulsor (ADP). The results from this study suggest that it is possible to make such prediction with sufficient fidelity to provide an indication of the acoustic transmission trends with the fan tip speed.

  3. Renal collecting duct carcinoma in a dog.

    Science.gov (United States)

    Kobayashi, N; Suzuki, K; Shibuya, H; Sato, T; Aoki, I; Nagashima, Y

    2008-07-01

    An 8-year-old, male, mongrel dog developed severe cough and anorexia and died within 3 months. Autopsy revealed an invasive grayish-white mass in the right kidney and multiple nodules in the lungs, thoracic wall, and spleen. Histologically, the renal mass and the other nodules were mainly composed of papillotubular structures lined by oval-to-polygonal pleomorphic cells. The cells were reactive with DBA, PNA, and UEA-1 lectins and positive for vimentin but negative for CD10 and high molecular weight cytokeratin. Because of its histological, histochemical, and immunohistochemical similarities with human collecting duct carcinoma (CDC), a diagnosis of renal collecting duct carcinoma with pulmonary, thoracic, and splenic metastases was established. To our knowledge, this is the first case report of CDC in animals.

  4. CT findings of common bile duct stones

    Energy Technology Data Exchange (ETDEWEB)

    Koh, Byung Hee; Lee, Sang Kil; Kim, Jong Sung; Joo, Kyung Bin; Cho, On Koo; Hahm, Chang Kok; Kim, Yong Il [College of Medicine, Hanyang University, Seoul (Korea, Republic of)

    1992-11-15

    A retrospective study on computed tomographic (CT) findings in 35 surgically confirmed cases of common bile duct stones was performed to analyze the characteristic CT findings. Choledocholithiasis was correctly diagnosed by CT in 33 out of 35 cases (sensitivity, 94.3%) and among these CT demonstrated calculi of high attenuation in 32 and of soft tissue density in one case. The thirty two included 23 totally calcified. 5 rim calcified and 4 laminated stones. The target sign was observed in 30 out of 33 CT positive cases (90.1%). We concluded that CT is an accurate modality in the diagnosis of common bile duct stones with its' reliable display of the calcified or the laminated stone itself and its' characteristic target sign.

  5. CT findings of common bile duct stones

    International Nuclear Information System (INIS)

    Koh, Byung Hee; Lee, Sang Kil; Kim, Jong Sung; Joo, Kyung Bin; Cho, On Koo; Hahm, Chang Kok; Kim, Yong Il

    1992-01-01

    A retrospective study on computed tomographic (CT) findings in 35 surgically confirmed cases of common bile duct stones was performed to analyze the characteristic CT findings. Choledocholithiasis was correctly diagnosed by CT in 33 out of 35 cases (sensitivity, 94.3%) and among these CT demonstrated calculi of high attenuation in 32 and of soft tissue density in one case. The thirty two included 23 totally calcified. 5 rim calcified and 4 laminated stones. The target sign was observed in 30 out of 33 CT positive cases (90.1%). We concluded that CT is an accurate modality in the diagnosis of common bile duct stones with its' reliable display of the calcified or the laminated stone itself and its' characteristic target sign

  6. Renal cystic disease: A practical overview

    International Nuclear Information System (INIS)

    Hartman, D.S.

    1987-01-01

    Renal cystic disease includes a group of lesions with extremely diverse clinical, radiographic, and pathologic findings. The recent development of multiple imaging systems to study renal cystic disease has resulted in considerable interest in correlating the images obtained by different modalities with each other and with the underlying gross pathology. A thorough knowledge of the disturbed morphology and natural history of these diseases will lead to a better understanding of their appearance on radiologic imaging. This refresher course correlates disturbed morphology with appearances on diagnostic imaging, urography, US, angiography, CT, and MR imaging. The advantages and limitations of each imaging method are detailed. A practical classification emphasizing differential features is presented. The presentation is divided into two parts. In the first part typical and atypical cystic masses, including acquired cystic disease (from dialysis), Von Hippel-Lindau disease, and the cystic disease of tuberous sclerosis are discussed. In the second part, polycystic kidney disease (dominant and recessive), medullary cystic disease, medullary sponge kidney, multicycle-dysplastic kidney, renal sinus cysts (peripelvic), and pluricystic kidney disease are discussed

  7. Iatrogenic injury to the common bile duct.

    Science.gov (United States)

    Buturovic, Sead

    2014-08-01

    The formation of gallstones in the gall bladder is very common. It is now considered that approximately 10% to 15% of the adult population has gallstones. It is more frequent in women than men. age, sex, diet, sudden weight loss, etc. According to the report of the US National Institute of Health, it is estimated that 6.3 million men and 14.2 million women, aged 20-74 years in the United States has calculosis of the gallbladder, due to which every year is performed about 700,000 cholecystectomy. Male patient, aged about 40 years, admitted at the Clinic and laparoscopic surgery was performed in our department for chronic, as multiple, symptomatic gallbladder calculi. Intraoperative findings showed chronically inflamed, curled gall bladder, and wall thickening. After the first postoperative day there is no content in the drain bag and it is taken out, and the patient leaves the hospital without any problems. The seventh post-operative day occurred abdominal pain, weakness and fatigue, with striking yellow skin and visible mucous membranes. Clinical, laboratory and echo determined abdomen full of fluids, so it was suspected lesion of the bile duct. Urgent revision was performed. Intraoperative was found a lesion of the common bile duct in the form of a complete interruption. Created is anastomosis through transhepatic drain according to Pradera. Early and late postoperative flow was entirely normal with normal laboratory and echofindings. Control, contrast imaging through a drain showed the orderly flow of extrahepatic bile ducts, with minimal extravasation of contrast. At the Department of Surgery of General Hospital in Konjic laparoscopic cholecystectomy is performed since 1999. In the beginning it was done by three trocars (European style), and later, in order to prevent complications or injury of the bile duct is performed surgery with four trocars (American style). The number of complicated procedures in our hospital does not differ from similar indicators in

  8. Transverse testicular ectopia with persistent Mullerian duct syndrome

    African Journals Online (AJOL)

    anomaly and understanding of the embryological process of testicular descent and its associated aberration are prerequisites for the proper management of this rare anomaly. These findings highlight the importance of diagnostic laparoscopy in all patients presented with nonpalpable testes. Ann Pediatr Surg 8:62–63.

  9. Decision-making in pediatric persistent Mullerian duct syndrome

    African Journals Online (AJOL)

    Biopsy of the gonads confirmed testicular tissue. Genetic analysis demonstrated a 46, XY male. ... operation was concluded. Pathologic evaluation of the gonadal tissue was consistent with testicular tissue with .... Testicular cancer incidence to rise by 25% by 2025 in Europe? Model- based predictions in 40 countries using ...

  10. Decision-making in pediatric persistent Mullerian duct syndrome | Al ...

    African Journals Online (AJOL)

    We are reporting a case of an 18-month old male who presented with bilateral cryptorchidism. The patient underwent an explorative laparoscopy in which two gonads were identified in close proximity to the uterus and fallopian tubes. Biopsy of the gonads confirmed testicular tissue. Genetic analysis demonstrated a 46, XY ...

  11. Heat Transfer Enhancement In a Ribbed Duct

    Directory of Open Access Journals (Sweden)

    J.M. Jalil

    2006-12-01

    Full Text Available The rib enhancement of heat transfer in a duct is studied numerically and experimentally, where hot air passes through a duct (0.04 x 0.16 x 1.15 m3 with different rib arrangement. The arranments are lower 12-rib arrangement; upper 12 rib arrangement and 24 rib staggered arrangement. The staggered arrangement gives better performance than the others. Also, the angle of attack was studied for lower arrangement, three different values were tested (45°, 60° and 90°. Angle of 60° gives better performance. Numerically, the three-dimension continuity, Navier-Stokes and energy   by finite volume method of flow of air through (0.04 x 0.16 x 0.6 m3. Validation of the code was performed by comparing the numerical result with the results obtained experimentally for staggered arrangement only. The agreement seems acceptable. The numerical studies were extended to study the case of cold air passing through hot ribbed duct.

  12. Revisiting sweat chloride test results based on recent guidelines for diagnosis of cystic fibrosis.

    Science.gov (United States)

    Pagaduan, Jayson V; Ali, Mahesheema; Dowlin, Michael; Suo, Liye; Ward, Tabitha; Ruiz, Fadel; Devaraj, Sridevi

    2018-03-01

    Recent sweat chloride guidelines published by the Cystic Fibrosis Foundation changed the intermediate sweat chloride concentration range from 40-59 mmol/L to 30-59 mmol/L for age > 6 months. We wanted to know how this new guideline would impact detection of cystic fibrosis among patients who previously had sweat tests done at Texas Children's Hospital. We revisited sweat chloride test results (n = 3012) in the last 5 years at Texas Children's Hospital based on the new guidelines on diagnosis of cystic fibrosis from the Cystic Fibrosis Foundation. We identified 125 patients that would be reclassified in the intermediate sweat chloride value with the new guidelines that were classified as "unlikely to have CF" in the previous guidelines. 8 (32%) patients with CFTR gene testing were positive for CFTR gene mutation(s). 4 (50%) of these patients were identified to have 2 CFTR mutations. One had variant combination that was reported to cause CF but all were diagnosed with CFTR-related metabolic syndrome. Our findings concur with the new CF diagnosis guidelines that changing the intermediate cut-off to 30-59 mmol/L sweat chloride concentration in combination with CFTR genetic analysis enhances the probability of identifying individuals that have risk of developing CF or have CF and enables for earlier therapeutic intervention.

  13. Optimization of air ducts for nuclear reactor power generation station

    International Nuclear Information System (INIS)

    Hirao, Katsumi; Yoshino, Hirokazu; Sonoda, Takayuki

    1991-01-01

    In the optimization study on the heating, ventilating and air conditions system in Nuclear Reactor Power Generation Station, proper arrangement of air ducts has been studied using the experimental and analytical investigation from a viewpoint of duct arrangement optimization. This study consists of two parts. Part I is optimization of air ducts in the corridors and Part II is optimization of air duct in each room. In part I, from viewpoints of confinement of radioactive materials in facilities having possible radioactive contamination and improvement of thermal environment for workers, the authors have studied air ducts system in which fresh air is supplied to corridors and heat removal and ventilation for each room are performed by transferring air from the corridors, instead of current ducts system with supply duct to each room. In part II, the condenser room with complex configuration and large space, and the electrical equipment room with simple space are selected for model areas. Based on these studies, experimental and analytical investigation (using a three-dimensional thermal hydraulic analysis) technique has been established, and the effective design method for duct arrangement of HVAC design has been verified for Boiling Water Reactor Power Station. The air-duct arrangements optimized in this study are applied to an Advanced Boiling Water Reactor Power Station in trial and reduction of the air-duct quantity is confirmed

  14. Compact Buried Ducts in a Hot-Humid Climate House

    Energy Technology Data Exchange (ETDEWEB)

    Mallay, Dave [Home Innovation Research Labs, Upper Marlboro, MD (United States)

    2016-01-07

    "9A system of compact, buried ducts provides a high-performance and cost-effective solution for delivering conditioned air throughout the building. This report outlines research activities that are expected to facilitate adoption of compact buried duct systems by builders. The results of this research would be scalable to many new house designs in most climates and markets, leading to wider industry acceptance and building code and energy program approval. The primary research question with buried ducts is potential condensation at the outer jacket of the duct insulation in humid climates during the cooling season. Current best practices for buried ducts rely on encapsulating the insulated ducts with closed-cell spray polyurethane foam insulation to control condensation and improve air sealing. The encapsulated buried duct concept has been analyzed and shown to be effective in hot-humid climates. The purpose of this project is to develop an alternative buried duct system that performs effectively as ducts in conditioned space - durable, energy efficient, and cost-effective - in a hot-humid climate (IECC warm-humid climate zone 3A) with three goals that distinguish this project: 1) Evaluation of design criteria for buried ducts that use common materials and do not rely on encapsulation using spray foam or disrupt traditional work sequences; 2) Establishing design criteria for compact ducts and incorporate those with the buried duct criteria to further reduce energy losses and control installed costs; 3) Developing HVAC design guidance for performing accurate heating and cooling load calculations for compact buried ducts.

  15. Unilateral Duplication Of Parotid Duct. A Rare Anatomical Variation

    Directory of Open Access Journals (Sweden)

    Humberto Ferreira Arquez

    2017-11-01

    Full Text Available Background: The paired parotid glands are the largest of the major salivary glands and produces mainly serous secretions. The secretion of this gland reaches the oral cavity through single parotid duct (Stensen’s duct. The parotid duct begins at the anterior border of the gland, crosses the masseter muscle, and then pierces the buccinator muscle to reach the mucosa lining the mouth at the level of the cheek. The purpose of this study is determine the morphologic features of the parotid duct and describe an anatomical variation until now unreported. Methods and Findings: A total of 17 cadavers were used for this study in the Morphology Laboratory at the University of Pamplona. In a cadaver were findings: The main parotid duct originated two conducts: Left superior parotid duct and Left inferior parotid duct, is observed the criss-cross of the ducts, and then perforated the buccinator muscle and entered the oral cavity at a double parotid papilla containing a double opening, separated from each other in 0,98 mm. In the remaining  33 parotid regions (97.06% the parotid duct is conformed to the classical descriptions given in anatomical textbooks. Conclusions: The parotid duct anatomy is important for duct endoscopy, lithotripsy, sialography and trans-ductal facial nerve stimulation in the early stage of facial palsy in some cases. The anatomical variations also has clinical importance for parotid gland surgery and facial cosmetic surgery. To keep in mind the parotid duct variation will reduce iatrogenic injury risks and improve diagnosis of parotid duct injury.

  16. Effect of duct geometry on Wells turbine performance

    International Nuclear Information System (INIS)

    Shaaban, S.; Abdel Hafiz, A.

    2012-01-01

    Highlights: ► A Wells turbine duct design in the form of venturi duct is proposed and investigated. ► Optimum duct geometry is identified. ► Up to 14% increase of the turbine power can be achieved using the optimized duct geometry. ► Up to 9% improve of the turbine efficiency is attained by optimizing the turbine duct geometry. ► The optimized duct geometry results in tangible delay of the turbine stalling point. - Abstract: Wells turbines can represent important source of renewable energy for many countries. An essential disadvantage of Wells turbines is their low aerodynamic efficiency and consequently low power produced. In order to enhance the Wells turbine performance, the present research work proposes the use of a symmetrical duct in the form of a venturi tube with turbine rotor located at throat. The effects of duct area ratio and duct angle are investigated in order to optimize Wells turbine performance. The turbine performance is numerically investigated by solving the steady 3D incompressible Reynolds Averaged Navier–Stocks equation (RANS). A substantial improve of the turbine performance is achieved by optimizing the duct geometry. Increasing both the duct area ratio and duct angle increase the acceleration and deceleration upstream and downstream the rotor respectively. The accelerating flow with thinner boundary layer thickness upstream the rotor reduces the flow separation on the rotor suction side. The downstream diffuser reduces the interaction between tip leakage flow and blade suction side. Up to 14% increase in turbine power and 9% increase in turbine efficiency are achieved by optimizing the duct geometry. On other hand, a tangible delay of the turbine stall point is also detected.

  17. Papillary carcinoma of a thyroglossal duct cyst in a patient with thyroid hemiagenesis: effectiveness of conservative surgical treatment.

    Science.gov (United States)

    Berni Canani, Francesco; Dall'Olio, Danilo; Chiarini, Valerio; Casadei, Gian Piero; Papini, Enrico

    2008-01-01

    To describe a case of thyroglossal duct cyst carcinoma that arose in a patient with right thyroid lobe hemiagenesis. We present the imaging, physical examination findings, treatment, and clinical course of the study patient. A 35-year-old woman was evaluated for a neck mass that had been present for 6 months and was slowly growing. She reported a previous diagnosis of right hemithyroid agenesis. The patient's preoperative workup included ultrasonography of the neck and head and neck T1- and T2-weighted magnetic resonance imaging, which showed right hemithyroid agenesis and a cystic lesion in the median region of the neck below the hyoid bone. Findings from chest x-rays and thyroid function tests were normal. The patient underwent a modified Sistrunk procedure that included removal of the median portion of the hyoid bone. Histologic findings showed a 2.5-cm thyroglossal duct cyst with a 0.6-cm focus of follicular variant of papillary carcinoma with invasion of the cyst wall. Total thyroidectomy was not performed because of the absence of tumoral invasion of the parenchyma around the thyroglossal duct cyst and because the patient was at low risk for aggressive disease. Cervical ultrasonography examinations were performed 6, 12, and 24 months after treatment, and all findings were normal. Presently, the patient is symptom-free after 4 years of follow-up and has no evidence of disease. Incidentally discovered, well-differentiated thyroid cancer that is confined to a thyroglossal duct cyst in a patient at low risk for aggressive disease can be adequately treated by a modified Sistrunk procedure that includes the median portion of the hyoid bone.

  18. Idiopathic chylopericardium treated by percutaneous thoracic duct embolization after failed surgical thoracic duct ligation

    Energy Technology Data Exchange (ETDEWEB)

    Courtney, Malachi; Ayyagari, Raj R. [Yale School of Medicine, Yale New Haven Hospital, New Haven, CT (United States); Division of Interventional Radiology, Department of Radiology, 789 Howard Avenue, P.O. Box 208042, New Haven, CT (United States)

    2015-06-15

    Chylopericardium rarely occurs in pediatric patients, but when it does it is most often a result of lymphatic injury during cardiothoracic surgery. Primary idiopathic chylopericardium is especially rare, with few cases in the pediatric literature. We report a 10-year-old boy who presented with primary idiopathic chylopericardium after unsuccessful initial treatment with surgical lymphatic ligation and creation of a pericardial window. Following readmission to the hospital for a right-side chylothorax resulting from the effluent from the pericardial window, he had successful treatment by interventional radiology with percutaneous thoracic duct embolization. This case illustrates the utility of thoracic duct embolization as a less-invasive alternative to surgical thoracic duct ligation, or as a salvage procedure when surgical ligation fails. (orig.)

  19. Adenoid cystic carcinoma of the parotid gland associated with salivary calculi: An unusual presentation.

    Science.gov (United States)

    Shenoy, Vijendra S; Kamath, M Panduranga; Sreedharan, Suja; Suhas, S S

    2015-01-01

    Adenoid cystic carcinomas (ACC) of the head and neck are relatively rare tumors, consisting of approximately 10-15% of all salivary gland neoplasms. ACC, a slow-growing aggressive malignant tumor of salivary gland commonly seen in the submandibular, sublingual, minor salivary glands is seldom found in the parotid. Calculus, the common cause of salivary gland dysfunction is usually identified in submandibular salivary gland because of its duct anatomy and physiochemical characteristic serous secretion. We report an unusual case of co-existent presentation of ACC with salivary calculi in the parotid gland which is never been reported in the literature. Co-existence of ductal calculi and ACC is rare. Presence of parotid calculus could be due to long standing ductal obstruction by the slow-growing ACC of the parotid or other possibility is that the malignancy could have developed because of chronic irritation by parotid calculi. Confirmatory studies are required to understand its mutual pathological association.

  20. Adenoid cystic carcinoma of the parotid gland associated with salivary calculi: An unusual presentation

    Directory of Open Access Journals (Sweden)

    Vijendra S Shenoy

    2015-01-01

    Full Text Available Adenoid cystic carcinomas (ACC of the head and neck are relatively rare tumors, consisting of approximately 10-15% of all salivary gland neoplasms. ACC, a slow-growing aggressive malignant tumor of salivary gland commonly seen in the submandibular, sublingual, minor salivary glands is seldom found in the parotid. Calculus, the common cause of salivary gland dysfunction is usually identified in submandibular salivary gland because of its duct anatomy and physiochemical characteristic serous secretion. We report an unusual case of co-existent presentation of ACC with salivary calculi in the parotid gland which is never been reported in the literature. Co-existence of ductal calculi and ACC is rare. Presence of parotid calculus could be due to long standing ductal obstruction by the slow-growing ACC of the parotid or other possibility is that the malignancy could have developed because of chronic irritation by parotid calculi. Confirmatory studies are required to understand its mutual pathological association.

  1. Comparative prospective randomized trial: laparoscopic versus open common bile duct exploration

    Directory of Open Access Journals (Sweden)

    Vladimir Grubnik

    2011-06-01

    Full Text Available Introduction: Single-stage laparoscopic procedures for common bile duct (CBD stones are an alternative treatmentoption to two-stage endo-laparoscopic treatment and to open choledocholithotomy. Several reports have demonstratedthe feasibility, safety, efficiency and cost-effectiveness of laparoscopic techniques.Aim: To analyse the safety and benefits of laparoscopic compared to open common bile duct (CBD exploration.Material and methods: The prospective randomized trial included a total of 256 patients with CBD stones operated from2005 to 2009 in a single centre. The male/female ratio was 82/174, with a median age 62.3 ±5.8 years (range 27 to 87years. There were two groups of patients. Group I: laparoscopic CBD exploration (138 patients. Group II: open CBD exploration(118 patients. Patient comorbidity was assessed by means of the American Society of Anesthesiologists (ASA classification;ASA II – 109 patients, ASA III – 59 patients. Bile duct stones were visualized preoperatively by means of US examinationin 129 patients, by means of ERCP in 26 patients, and by magnetic resonance cholangiopancreatography (MRCPin 72 patients. Preoperative evaluation was done through medical history, biochemical tests and ultrasonography.Results: The mean duration of laparoscopic procedures was 82 min (range 40-160 min. The mean duration of openprocedures was 90 min (range 60-150 min. Mean blood loss was much lower in the laparoscopic group than in theopen group (20 ±2 v.s 285 ±27, p < 0.01. Postoperative complications were observed in 7 patients of the laparoscopicgroup and in 15 patients in the open group (p < 0.01. Laparoscopic common bile duct exploration was performedthrough a trans-cystic approach in 76 patients and via choledochotomy in 62 patients. The transcystic approach wassuccessful in 76 patients (74.5%. External drainage was used in 25 (32.8% patients with the transcystic approach.Conclusions: Laparoscopic CBD exploration can be performed with

  2. Oxygen therapy for cystic fibrosis.

    Science.gov (United States)

    Elphick, Heather E; Mallory, George

    2013-07-25

    The most serious complications of cystic fibrosis (CF) relate to respiratory insufficiency. Oxygen supplementation therapy has long been a standard of care for individuals with chronic lung diseases associated with hypoxemia. Physicians commonly prescribe oxygen therapy for people with CF when hypoxemia occurs. However, it is unclear if empiric evidence is available to provide indications for this therapy with its financial costs and often profound impact on lifestyle. To assess whether oxygen therapy improves the longevity or quality of life of individuals with CF. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register, comprising references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings.Latest search of Group's Trials Register: 15 May 2013. Randomized or quasi-randomized controlled trials comparing oxygen, administered at any concentration, by any route, in people with documented CF for any time period. Authors independently assessed the risk of bias for included studies and extracted data. This review includes 11 published studies (172 participants); only one examined long-term oxygen therapy (28 participants). There was no statistically significant improvement in survival, lung, or cardiac health. There was an improvement in regular attendance at school or work in those receiving oxygen therapy at 6 and 12 months. Four studies examined the effect of oxygen supplementation during sleep by polysomnography. Although oxygenation improved, mild hypercapnia was noted. Participants fell asleep quicker and spent a reduced percentage of total sleep time in rapid eye movement sleep, but there were no demonstrable improvements in qualitative sleep parameters. Six studies evaluated oxygen supplementation during exercise. Again, oxygenation improved, but mild hypercapnia resulted. Participants receiving oxygen therapy were able to exercise for a

  3. Gene therapy in cystic fibrosis.

    Science.gov (United States)

    Flotte, T R; Laube, B L

    2001-09-01

    Theoretically, cystic fibrosis transmembrane conductance regulator (CFTR) gene replacement during the neonatal period can decrease morbidity and mortality from cystic fibrosis (CF). In vivo gene transfers have been accomplished in CF patients. Choice of vector, mode of delivery to airways, translocation of genetic information, and sufficient expression level of the normalized CFTR gene are issues that currently are being addressed in the field. The advantages and limitations of viral vectors are a function of the parent virus. Viral vectors used in this setting include adenovirus (Ad) and adeno-associated virus (AAV). Initial studies with Ad vectors resulted in a vector that was efficient for gene transfer with dose-limiting inflammatory effects due to the large amount of viral protein delivered. The next generation of Ad vectors, with more viral coding sequence deletions, has a longer duration of activity and elicits a lesser degree of cell-mediated immunity in mice. A more recent generation of Ad vectors has no viral genes remaining. Despite these changes, the problem of humoral immunity remains with Ad vectors. A variety of strategies such as vector systems requiring single, or widely spaced, administrations, pharmacologic immunosuppression at administration, creation of a stealth vector, modification of immunogenic epitopes, or tolerance induction are being considered to circumvent humoral immunity. AAV vectors have been studied in animal and human models. They do not appear to induce inflammatory changes over a wide range of doses. The level of CFTR messenger RNA expression is difficult to ascertain with AAV vectors since the small size of the vector relative to the CFTR gene leaves no space for vector-specific sequences on which to base assays to distinguish endogenous from vector-expressed messenger RNA. In general, AAV vectors appear to be safe and have superior duration profiles. Cationic liposomes are lipid-DNA complexes. These vectors generally have been

  4. Flow regime analysis of non-Newtonian duct flows

    Science.gov (United States)

    Speetjens, Michel; Rudman, Murray; Metcalfe, Guy

    2006-01-01

    Reoriented duct flows of generalized Newtonian fluids are an idealization of non-Newtonian fluid flow in industrial in-line mixers. Based on scaling analysis and computation we find that non-Newtonian duct flows have several limit behaviors, in the sense that such flows can become (nearly) independent of one or more of the rheological and dynamical control parameters, simplifying the general flow and mixing problem. These limit flows give several levels of modeling complexity to the full problem of non-Newtonian duct flow. We describe the sets of simplified flow models and their corresponding regions of validity. This flow-model decomposition captures the essential rheological and dynamical characteristics of the reoriented duct flows and enables a more efficient and systematic study and design of flow and mixing of non-Newtonian fluids in ducts. Key aspects of the flow-model decomposition are demonstrated via a specific, but representative, duct flow.

  5. Adenofibroma of Skene's Duct: A Case Report

    Directory of Open Access Journals (Sweden)

    Yosep Chong

    2010-01-01

    Full Text Available Skene's glands, also known as paraurethral glands, are homologues of the male prostate, in which painless cystic masses and inflammation due to obstruction have been rarely found and reported. In addition, there have been rare reported cases of adenocarcinoma of Skene's glands. Recently, the authors experienced the first case of adenofibroma arising in Skene's glands of a 62-year-old woman with coital pain. Hereby, we present the case with pathologic and immunohistochemical findings and a short review of literature.

  6. Clearance of refractory bile duct stones with extracorporeal shockwave lithotripsy

    OpenAIRE

    Ellis, R; Jenkins, A; Thompson, R; Ede, R

    2000-01-01

    BACKGROUND—Extracorporeal shockwave lithotripsy (ESWL) has been used since the mid-1980s to fragment bile duct stones which cannot be removed endoscopically. Early machines required general anaesthesia and immersion in a waterbath.
AIMS—To investigate the effectiveness of the third generation Storz Modulith SL20 lithotriptor in fragmenting bile duct stones that could not be cleared by mechanical lithotripsy.
METHODS—Eighty three patients with retained bile duct stones were treated. All patien...

  7. Active noise control in a duct to cancel broadband noise

    Science.gov (United States)

    Chen, Kuan-Chun; Chang, Cheng-Yuan; Kuo, Sen M.

    2017-09-01

    The paper presents cancelling duct noises by using the active noise control (ANC) techniques. We use the single channel feed forward algorithm with feedback neutralization to realize ANC. Several kinds of ducts noises including tonal noises, sweep tonal signals, and white noise had investigated. Experimental results show that the proposed ANC system can cancel these noises in a PVC duct very well. The noise reduction of white noise can be up to 20 dB.

  8. Squamous Cell Carcinoma of the Distal Common Bile Duct

    OpenAIRE

    Jain A; Juneja M; Naik S; Sharma S; Kapoor S; Sewkani A; Varshney S

    2005-01-01

    CONTEXT: Squamous cell carcinoma of the biliary tree is rare. Although few cases of squamous cell carcinoma of the intrahepatic bile-duct and gallbladder have been reported, until today, only four cases of squamous cell carcinoma of the extrahepatic bile duct have been reported in the literature. CASE REPORT: We present a case of squamous cell carcinoma of the distal common bile duct presenting with obstructive jaundice in a 60-year-old male which was successfully managed by a Whipple's pancr...

  9. Cystic Fibrosis-Related Diabetes (CFRD): Daily Management

    Science.gov (United States)

    Cystic Fibrosis-Related Diabetes (CFRD): Daily Management September 20, 2011 This Web cast is supported by an unrestricted ... Moran, MD Professor, Pediatric Endocrinology University of Minnesota Cystic Fibrosis-Related Diabetes (CFRD): Daily Management September 20, 2011 ...

  10. Microbial ecology and adaptation in cystic fibrosis airways

    DEFF Research Database (Denmark)

    Yang, Lei; Jelsbak, Lars; Molin, Søren

    2011-01-01

    Chronic infections in the respiratory tracts of cystic fibrosis (CF) patients are important to investigate, both from medical and from fundamental ecological points of view. Cystic fibrosis respiratory tracts can be described as natural environments harbouring persisting microbial communities...

  11. Rehabilitation with Cystic Fibrosis: From Utopia to Reality.

    Science.gov (United States)

    Goldberg, Richard T.; And Others

    1980-01-01

    The paper dispels some of the myths regarding cystic fibrosis (a genetic metabolism disorder), provides information on the latest developments in rehabilitation, summarizes research in the field, and projects future needs of the patient with cystic fibrosis. (SBH)

  12. Genetics Home Reference: medullary cystic kidney disease type 1

    Science.gov (United States)

    ... Medullary cystic kidney disease type 1 Medullary cystic kidney disease type 1 Printable PDF Open All Close All ... is direct-to-consumer genetic testing? What is precision medicine? What is newborn screening? New Pages Pelizaeus-Merzbacher- ...

  13. Male fertility in cystic fibrosis.

    LENUS (Irish Health Repository)

    Chotirmall, S H

    2011-04-05

    Infertility rates among males with cystic fibrosis (CF) approximate 97%. No information is currently available within Ireland determining an understanding of fertility issues and the best methods of information provision to this specialized group. This study aimed to determine understanding and preferred approaches to information provision on fertility issues to Irish CF males. A Descriptive Study utilizing prospective coded questionnaires was mailed to a male CF cohort (n=50). Sections included demographics, fertility knowledge & investigation. Response rate was 16\\/50 (32%). All were aware that CF affected their fertility. More than two-thirds (n=11) were able to provide explanations whilst only one-third (n=5) provided the correct explanation. Significant numbers stated thoughts of marriage and a future family. Half have discussed fertility with a healthcare professional (HCP). Mean age of discussion was 21.9 years. One third preferred an earlier discussion. The commonest first source for information was written material which was also the preferred source. Three-quarters requested further information preferring again, written material. Significant gaps in sex education of Irish CF males exist. Discussion should be initiated by HCPs and centre-directed written material devised to address deficiencies.

  14. Cystic fibrosis: a clinical view.

    Science.gov (United States)

    Castellani, Carlo; Assael, Baroukh M

    2017-01-01

    Cystic fibrosis (CF), a monogenic disease caused by mutations in the CFTR gene on chromosome 7, is complex and greatly variable in clinical expression. Airways, pancreas, male genital system, intestine, liver, bone, and kidney are involved. The lack of CFTR or its impaired function causes fat malabsorption and chronic pulmonary infections leading to bronchiectasis and progressive lung damage. Previously considered lethal in infancy and childhood, CF has now attained median survivals of 50 years of age, mainly thanks to the early diagnosis through neonatal screening, recognition of mild forms, and an aggressive therapeutic attitude. Classical treatment includes pancreatic enzyme replacement, respiratory physiotherapy, mucolitics, and aggressive antibiotic therapy. A significant proportion of patients with severe symptoms still requires lung or, less frequently, liver transplantation. The great number of mutations and their diverse effects on the CFTR protein account only partially for CF clinical variability, and modifier genes have a role in modulating the clinical expression of the disease. Despite the increasing understanding of CFTR functioning, several aspects of CF need still to be clarified, e.g., the worse outcome in females, the risk of malignancies, the pathophysiology, and best treatment of comorbidities, such as CF-related diabetes or CF-related bone disorder. Research is focusing on new drugs restoring CFTR function, some already available and with good clinical impact, others showing promising preliminary results that need to be confirmed in phase III clinical trials.

  15. Evaluation of hepatic cystic lesions

    Science.gov (United States)

    Lantinga, Marten A; Gevers, Tom JG; Drenth, Joost PH

    2013-01-01

    Hepatic cysts are increasingly found as a mere coincidence on abdominal imaging techniques, such as ultrasonography (USG), computed tomography (CT) and magnetic resonance imaging (MRI). These cysts often present a diagnostic challenge. Therefore, we performed a review of the recent literature and developed an evidence-based diagnostic algorithm to guide clinicians in characterising these lesions. Simple cysts are the most common cystic liver disease, and diagnosis is based on typical USG characteristics. Serodiagnostic tests and microbubble contrast-enhanced ultrasound (CEUS) are invaluable in differentiating complicated cysts, echinococcosis and cystadenoma/cystadenocarcinoma when USG, CT and MRI show ambiguous findings. Therefore, serodiagnostic tests and CEUS reduce the need for invasive procedures. Polycystic liver disease (PLD) is arbitrarily defined as the presence of > 20 liver cysts and can present as two distinct genetic disorders: autosomal dominant polycystic kidney disease (ADPKD) and autosomal dominant polycystic liver disease (PCLD). Although genetic testing for ADPKD and PCLD is possible, it is rarely performed because it does not affect the therapeutic management of PLD. USG screening of the liver and both kidneys combined with extensive family history taking are the cornerstone of diagnostic decision making in PLD. In conclusion, an amalgamation of these recent advances results in a diagnostic algorithm that facilitates evidence-based clinical decision making. PMID:23801855

  16. Growth factors in cystic fibrosis

    Directory of Open Access Journals (Sweden)

    Negar Khalighi

    2016-01-01

    Full Text Available Introduction: Cystic fibrosis is one of the most common autosomal recessive diseases that affects sweat glands and mucosa. CF is a hereditary disease with annual incidence of about 2500 new cases in United Kingdom. Insulin-like growth factor-1 (IGF-1 and insulin-like growth factor binding protein-3 levels decrease in CF. The aim of this study was to assess the role of growth peptides in patients with CF. Method: We searched PubMed, Google scholar, IranMedex, and Scientific Information Database (SID in September 2012 to April 2014. We included clinical studies with available abstracts and full texts that were in English or Persian languages. Manual searching was conducted within the reference lists of articles. Two reviewers independently applied eligibility criteria, assessed quality, and extracted data.Result: The earliest study was published in 1997 and the most recent one was in 2014. Study participants were adults in 3 studies (20% and 12 studies (80% were conducted in children. Patients with CF have lower levels of IGF-1 and there is a significant correlation between IGF-1 levels and growth index in patients with CF.Conclusions: IGF-1 decreases in children with CF and might be the cause of poor growth and low body mass index in these children.

  17. Liquid films flowing concurrently with air in horizontal duct, 4

    International Nuclear Information System (INIS)

    Fukano, Tohru; Takamatsu, Yasuo; Akenaga, Hiroshi; Ikeda, Masayoshi; Itoh, Akihiko; Kuriwaki, Tessho.

    1984-01-01

    The effect of the aspect ratio of the duct cross-section on a liquid film flowing concurrently with an air stream was investigated using three horizontal ducts. The dimensions of the duct are 10 mm x 40 mm (height x width), 40 mm x 40 mm and 10 mm x 80 mm. The results are summarized as follows: The boundary of flow patterns between the pebble wave flow and the disturbance wave flow strongly depends on the duct height. On the other hand, the boundary between a smooth surface flow and a two-dimensional wave flow as well as the nonwetting regime depends mainly on the duct width. The reason is that the volumetric flux of the liquid flow in the vicinity of the side walls of the duct is considerably larger than that in the central part of the duct width due to the formation of meniscus. Therefore, only the experimental values for the film parameters obtained at the central part of duct width are useful for comparison with the theoretical results obtained by the assumption that the flow is two-dimensional, even if the aspect ratio of duct seems to be sufficiently small. (author)

  18. Flexible metallic seal for transition duct in turbine system

    Science.gov (United States)

    Flanagan, James Scott; LeBegue, Jeffrey Scott; McMahan, Kevin Weston; Dillard, Daniel Jackson; Pentecost, Ronnie Ray

    2014-04-22

    A turbine system is disclosed. In one embodiment, the turbine system includes a transition duct. The transition duct includes an inlet, an outlet, and a passage extending between the inlet and the outlet and defining a longitudinal axis, a radial axis, and a tangential axis. The outlet of the transition duct is offset from the inlet along the longitudinal axis and the tangential axis. The transition duct further includes an interface member for interfacing with a turbine section. The turbine system further includes a flexible metallic seal contacting the interface member to provide a seal between the interface member and the turbine section.

  19. Ovarian cystic lesions: a current approach to diagnosis and management.

    Science.gov (United States)

    Ackerman, Susan; Irshad, Abid; Lewis, Madelene; Anis, Munazza

    2013-11-01

    The primary imaging modality for evaluation of ovarian cystic lesions is pelvic ultrasonography. Most ovarian cysts are benign and demonstrate typical sonographic features that support benignity. However, some ovarian cystic lesions have indeterminate imaging features, and the approach to management varies. This article discusses how to recognize and diagnose different types of ovarian cystic lesions, including an approach to management. The learning objective is to recognize imaging features of ovarian cystic lesions. Copyright © 2013 Elsevier Inc. All rights reserved.

  20. Cystic astrocytomas in children. The contribution of MRI

    International Nuclear Information System (INIS)

    Vilgrain, V.; Sellier, N.; Lalande, G.; Demange, P.; Kalifa, G.

    1988-01-01

    Three cases of cystic astrocytomas are reported in children. Two are supratentorial and one is a cerebellar tumor. The authors insist on the difficulties of the diagnosis. They emphasize the role of NMR which enables distinction between cystic astrocytomas and other cysts. In agreement with Kjos, the 3 cystic astrocytomas demonstrate an increased T1 and T2 and belong to the group of cystic tumors (type II) [fr

  1. Interposition of the gallbladder in the common hepatic duct: a rare dangerous anomaly. Case report.

    Science.gov (United States)

    Rosato, L; Ginardi, A; Mondini, G

    2011-01-01

    Anomalies of the gallbladder position in the biliary tract are rare, but they could be very dangerous during cholecystectomy. A 48-year-old man presented with a 2-week history of intermittent epigastric pain, scleral jaundice and elevation of liver function tests. After a magnetic resonance cholangiogram and an endoscopic retrograde cholangiogram with sphincterotomy, he was submitted to laparoscopic cholecystectomy, the conversion to laparotomy was decided for the suspect of gallbladder interposition. The anatomical anomaly was confirmed and a Roux-en-Y hepaticojejunostomy was executed, with end-to-side anastomosis between the confluence of the hepatic ducts and the fourth loop of jejunum, on a biliary stent. This catheter was removed in the tenth postoperative day; after cholangiography and CT abdominal scan the patient was discharged, without complications. The gallbladder interposition is a rare malformation which seems to arise from an embryonic anomaly occurring between the 4th and the 5th week and whose potential causes have not been detected. A similar outcome could be also determined by a Mirizzi syndrome, but in our case it is excluded because intra-operatively there was no inflammatory reaction that could justify the presence of a fistula between the gallbladder and the common hepatic duct. Once the gallbladder interposition is found, the surgical treatment consists in removing the gallbladder itself and the corresponding part of the common hepatic duct. The reconstruction is carried out by a Roux-en-Y hepaticojejunostomy with anastomosis at the hepatic hilum, positioning a biliary stent.

  2. Levitated Duct Fan (LDF) Aircraft Auxiliary Generator

    Science.gov (United States)

    Eichenberg, Dennis J.; Emerson, Dawn C.; Gallo, Christopher A.; Thompson, William K.

    2011-01-01

    This generator concept includes a novel stator and rotor architecture made from composite material with blades attached to the outer rotating shell of a ducted fan drum rotor, a non-contact support system between the stator and rotor using magnetic fields to provide levitation, and an integrated electromagnetic generation system. The magnetic suspension between the rotor and the stator suspends and supports the rotor within the stator housing using permanent magnets attached to the outer circumference of the drum rotor and passive levitation coils in the stator shell. The magnets are arranged in a Halbach array configuration.

  3. Fluorescent Method for Observing Intravascular Bonghan Duct

    Directory of Open Access Journals (Sweden)

    Byung-Cheon Lee

    2005-12-01

    Full Text Available Observation of intra-vascular threadlike structures in the blood vessels of rats is reported with the images by differential interference contrast microscope, and fluorescence inverted microscope of the acridine-orange stained samples. The confocal microscope image and the hematoxylin-eosin staining revealed the distinctive pattern of nuclei distribution that clearly discerned the threadlike structure from fibrin, capillary, small venule, arteriole, or lymph vessel. Physiological function of the intra-vascular thread in connection with acupuncture is discussed. Especially, this threadlike duct can be a circulation path for herb-liquid flow, which may provide the scientific mechanism for therapeutic effect of herbal acupuncture.

  4. Live 3D image overlay for arterial duct closure with Amplatzer Duct Occluder II additional size.

    Science.gov (United States)

    Goreczny, Sebstian; Morgan, Gareth J; Dryzek, Pawel

    2016-03-01

    Despite several reports describing echocardiography for the guidance of ductal closure, two-dimensional angiography remains the mainstay imaging tool; three-dimensional rotational angiography has the potential to overcome some of the drawbacks of standard angiography, and reconstructed image overlay provides reliable guidance for device placement. We describe arterial duct closure solely from venous approach guided by live three-dimensional image overlay.

  5. Effectiveness of duct sealing and duct insulation in multi-family buildings. Final report

    Energy Technology Data Exchange (ETDEWEB)

    Karins, N.H.; Tuluca, A.; Modera, M.

    1997-07-01

    This research investigated the cost-effectiveness of sealing and insulating the accessible portions of duct systems exposed to unconditioned areas in multifamily housing. Airflow and temperature measurements were performed in 25 apartments served by 10 systems a 9 multi-family properties. The measurements were performed before and after each retrofit, and included apartment airflow (supply and return), duct system temperatures, system fan flow and duct leakage area. The costs for each retrofit were recorded. The data were analyzed and used to develop a prototypical multifamily house. This prototype was used in energy simulations (DOE-2.1E) and air infiltration simulations (COMIS 2.1). The simulations were performed for two climates: New York City and Albany. In each climate, one simulation was performed assuming the basement was tight, and another assuming the basement was leaky. Simulation results and average retrofit costs were used to calculate cost-effectiveness. The results of the analysis indicate that sealing leaks of the accessible ductwork is cost-effective under all conditions simulated (simple payback was between 3 and 4 years). Insulating the accessible ductwork, however, is only cost-effective for buildings with leaky basement, in both climates (simple paybacks were less than 5 years). The simple payback period for insulating the ducts in buildings with tight basements was greater than 10 years, the threshold of cost-effectiveness for this research. 13 refs., 5 figs., 27 tabs.

  6. Endocytosis and intracellular protein degradation in cystic fibrosis fibroblasts

    International Nuclear Information System (INIS)

    Jessup, W.; Dean, R.T.

    1983-01-01

    Normal rates of pinocytosis of [ 3 H]sucrose were measured in cystic fibrosis fibroblasts, and were not affected by the addition of cystic fibrosis serum. Bulk protein degradation (a significant proportion of which occurs intralysosomally following autophagy) and its regulation by growth state were apparently identical in normal and cystic fibrosis cultures. (Auth.)

  7. Living with Cystic Fibrosis: A Guide for the Young Adult.

    Science.gov (United States)

    Cystic Fibrosis Foundation, Atlanta, GA.

    Intended for the young adult with cystic fibrosis, the booklet provides information on dealing with problems and on advances in treatment and detection related to the disease. Addressed are the following topics: description of cystic fibrosis; inheritance of cystic fibrosis; early diagnosis; friends, careers, and other matters; treatment;…

  8. 78 FR 26681 - Medical Criteria for Evaluating Cystic Fibrosis

    Science.gov (United States)

    2013-05-07

    ... Evaluating Cystic Fibrosis AGENCY: Social Security Administration. ACTION: Notice of teleconference. SUMMARY... FR 7968). We use Listings 3.04 and 103.04 to evaluate claims involving cystic fibrosis in adults and... individuals with cystic fibrosis who apply for Social Security disability benefits, and for individuals with...

  9. A residual cystic lesion in acute disseminated encephalomyelitis

    Energy Technology Data Exchange (ETDEWEB)

    Go, T. [Otsu Red Cross Hospital, Nagara (Japan). Dept. of Paediatrics; Imai, T. [Kyoto Univ. School of Medicine, Kyoto (Japan). Dept. of Paediatrics

    2000-09-01

    We report a case of acute disseminated encephalomyelitis (ADEM) with a residual cystic lesion on MRI. This seemed to be induced by Japanese encephalitis vaccination. Despite complete clinical improvement with high-dose steroid therapy, the cystic lesion has persisted for 3 years on MRI. There have been no previous reports of residual cystic lesions in ADEM. (orig.)

  10. Congenital Cystic Adenomatoid Malformation of Lung-Rare Case Report

    Directory of Open Access Journals (Sweden)

    N. S. Kamakeri

    2016-10-01

    Full Text Available Congenital cystic adenomatoid malformation of lung associated with Cystic dysplasia of kidney, cystic disease of liver with mixed gonadal dysgenesis is rare and is not reported in literature so far. Hence an attempt is made to present this rarest entity.

  11. Large congenital cystic asdenomatous malformation of the lung in a newborn

    Directory of Open Access Journals (Sweden)

    İlyas Yolbaş

    2013-12-01

    Full Text Available Congenital cystic adenomatous malformation (CCAM oflung is a rare form of congenital hamartomatous lesionsof the lung consisting of cysts filled with air. The generalclinic presentation of CCAM is dyspnea in newborns.CCAM may mimic congenital pneumonia or respiratorydistress syndrome. After the delivery, the newborn malewho had low Apgar score and severe respiratory distresswas intubated and admitted to neonatal intensive careunit. Patient was ventilated for 50 days and weaned fromthe mechanical ventilator at 50th day. Type II CCAM of thelung was diagnosed according to the chest radiographsand computed tomography scan signs. Although the surgeonssuggested lobectomy considering the patient’s notcompletely asymptomatic, family did not accept this operationdue to the risk of death. The patient was dischargedfrom the hospital until the next control.Key word: Congenital cystic adenomatous malformation of lunch, newborn, conservative treatment

  12. Oblique bile duct predisposes to the recurrence of bile duct stones.

    Directory of Open Access Journals (Sweden)

    Pavel Strnad

    Full Text Available BACKGROUND AND STUDY AIMS: Bile stones represent a highly prevalent condition and abnormalities of the biliary tree predispose to stone recurrence due to development of biliary stasis. In our study, we assessed the importance of an altered bile duct course for stone formation. PATIENTS AND METHODS: 1,307 patients with choledocholithiasis in the absence of any associated hepatobiliary disease who underwent endoscopic retrograde cholangiopancreatography (ERCP between 2002 and 2009 were analysed. The angle enclosed between the horizontal portion of the common bile duct (CBD and the horizontal plane was measured (angle α. Oblique common bile duct (OCBD was defined as a CBD with angle α < 45°. RESULTS: 103 patients (7.9% were found to harbour OCBD and these were compared to 104 randomly selected control subjects. Compared to controls, OCBD patients were (i significantly older (72 ± 13 vs. 67 ± 13, p<0.00001; (ii more frequently underwent a cholecystectomy (p = 0.02 and biliary surgery (p = 0.003 prior to the diagnosis and (iii more often developed chronic pancreatitis (p = 0.04 as well as biliary fistulae (p = 0.03. Prior to and after ERCP, OCBD subjects displayed significantly elevated cholestatic parameters and angle α negatively correlated with common bile duct diameter (r = -0.29, p = 0.003. OCBD subjects more often required multiple back-to-back ERCP sessions to remove bile stones (p = 0.005 as well as more ERCPs later on due to recurrent stone formation (p<0.05. CONCLUSION: OCBD defines a novel variant of the biliary tree, which is associated with chronic cholestasis, hampers an efficient stone removal and predisposes to recurrence of bile duct stones.

  13. Pharmacological management of cystic fibrosis related diabetes.

    Science.gov (United States)

    Moheet, Amir; Moran, Antoinette

    2018-02-01

    There are limited data from randomized clinical trials to guide optimal options for pharmacological treatment of cystic fibrosis related diabetes (CFRD). Current guidelines recommend insulin as the only treatment option for CFRD. Areas covered: Current guidelines for screening, diagnosis and pharmacological agents for treatment of impaired glucose tolerance and CFRD in patients with cystic fibrosis are reviewed. Insights from clinical studies examining the role of insulin therapy in CFRD are presented. Expert commentary: CFRD is the most common extra pulmonary complication of cystic fibrosis, and is primarily related to insulin insufficiency. Insulin is the treatment of choice for CFRD. Insulin treatment is associated with improvement in glycemic control, nutritional status and lung function. Current data does not support use of oral hypoglycemic agents for treatment of CFRD.

  14. Different manifestations of calcifying cystic odontogenic tumor

    Directory of Open Access Journals (Sweden)

    Estevam Rubens Utumi

    2012-09-01

    Full Text Available The calcifying cystic odontogenic tumor normally presents as apainless, slow-growing mass, involving both maxilla and mandible,primarily the anterior segment (incisor/canine area. It generallyaffects young adults in the third to fourth decades, with no genderpredilection. Computerized tomography images revealed importantcharacteristics that were not detected by panoramic radiography,such as fenestration, calcification and tooth-like structures. Thetypical microscopic feature of this lesion is the presence of variableamounts of aberrant epithelial cells, without nuclei, which arenamed “ghost cells”. In addition, dysplastic dentine can be foundand occasionally the cyst can be associated with an area of dentalhard tissue formation resembling an odontoma. The treatment forcalcifying cystic odontogenic tumor involves simple enucleationand curettage. The purpose of this article is to present two differentmanifestation of calcifying cystic odontogenic tumor in whichcomputerized tomography, associated to clinical features, servedas an important tool for diagnosis, adequate surgical planning andfollow-up of patients.

  15. Renal cystic diseases in children: new concepts

    International Nuclear Information System (INIS)

    Avni, Fred E.; Hall, Michelle

    2010-01-01

    This review highlights the changes that have occurred in the general approach to cystic renal diseases in children. For instance, genetic mutations at the level of the primary cilia are considered as the origin of many renal cystic diseases. Furthermore, these diseases are now included in the spectrum of the hepato-renal fibrocystic diseases. Imaging plays an important role as it helps to detect and characterize many of the cystic diseases based on a detailed sonographic analysis. The diagnosis can be achieved during fetal life or after birth. Hyperechoic kidneys and/or renal cysts are the main sonographic signs leading to such diagnosis. US is able to differentiate between recessive and dominant polycystic kidney diseases, hepatocyte nuclear factor 1 Beta mutation, glomerulocystic kidneys and nephronophtisis. MR imaging can, in selected cases, provide additional information including the progressive associated hepatic changes. (orig.)

  16. Enteral tube feeding for cystic fibrosis.

    Science.gov (United States)

    Morton, Alison; Wolfe, Susan

    2015-04-09

    Enteral tube feeding is routinely used in many cystic fibrosis centres when oral dietary and supplement intake has failed to achieve an adequate nutritional status. The use of this method of feeding is assessed on an individual basis taking into consideration the patients age and clinical status. To examine the evidence that in people with cystic fibrosis, supplemental enteral tube feeding improves nutritional status, respiratory function, and quality of life without significant adverse effects. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register which comprises references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings. We also contacted the companies that market enteral feeds and reviewed their databases.Date of the most recent search of the Group's Cystic Fibrosis Trials Register: 13 February 2015.Date of the most recent hand search of PubMed and conference abstract books: 13 February 2015. All randomised controlled trials comparing supplemental enteral tube feeding for one month or longer with no specific intervention in people with cystic fibrosis. The searches identified 38 trials; however, none were eligible for inclusion in this review. There are no trials included in this review. Supplemental enteral tube feeding is widely used throughout the world to improve nutritional status in people with cystic fibrosis. The methods mostly used, nasogastric or gastrostomy feeding, are expensive and may have a negative effect on self-esteem and body image. Reported use of enteral tube feeding suggests that it results in nutritional and respiratory improvement; but, efficacy has not been fully assessed by randomised controlled trials. It is acknowledged, however, that performing a randomised controlled trial would be difficult due to the ethics of withholding an intervention in a group of patients whose nutritional status necessitates it.

  17. Liver Disease in Cystic Fibrosis: an Update

    Science.gov (United States)

    Parisi, Giuseppe Fabio; Di Dio, Giovanna; Franzonello, Chiara; Gennaro, Alessia; Rotolo, Novella; Lionetti, Elena; Leonardi, Salvatore

    2013-01-01

    Context Cystic fibrosis (CF) is the most widespread autosomal recessive genetic disorder that limits life expectation amongst the Caucasian population. As the median survival has increased related to early multidisciplinary intervention, other manifestations of CF have emergedespecially for the broad spectrum of hepatobiliary involvement. The present study reviews the existing literature on liver disease in cystic fibrosis and describes the key issues for an adequate clinical evaluation and management of patients, with a focus on the pathogenetic, clinical and diagnostic-therapeutic aspects of liver disease in CF. Evidence Acquisition A literature search of electronic databases was undertaken for relevant studies published from 1990 about liver disease in cystic fibrosis. The databases searched were: EMBASE, PubMed and Cochrane Library. Results CF is due to mutations in the gene on chromosome 7 that encodes an amino acidic polypeptide named CFTR (cystic fibrosis transmembrane regulator). The hepatic manifestations include particular changes referring to the basic CFTR defect, iatrogenic lesions or consequences of the multisystem disease. Even though hepatobiliary disease is the most common non-pulmonary cause ofmortalityin CF (the third after pulmonary disease and transplant complications), only about the 33%ofCF patients presents clinically significant hepatobiliary disease. Conclusions Liver disease will have a growing impact on survival and quality of life of cystic fibrosis patients because a longer life expectancy and for this it is important its early recognition and a correct clinical management aimed atdelaying the onset of complications. This review could represent an opportunity to encourage researchers to better investigate genotype-phenotype correlation associated with the development of cystic fibrosis liver disease, especially for non-CFTR genetic polymorphisms, and detect predisposed individuals. Therapeutic trials are needed to find strategies of

  18. Diagnosis of Adult Patients with Cystic Fibrosis.

    Science.gov (United States)

    Nick, Jerry A; Nichols, David P

    2016-03-01

    The diagnosis of cystic fibrosis (CF) is being made with increasing frequency in adults. Patients with CF diagnosed in adulthood typically present with respiratory complaints, and often have recurrent or chronic airway infection. At the time of initial presentation individuals may appear to have clinical manifestation limited to a single organ, but with subclinical involvement of the respiratory tract. Adult-diagnosed patients have a good response to CF center care, and newly available cystic fibrosis transmembrane receptor-modulating therapies are promising for the treatment of residual function mutation, thus increasing the importance of the diagnosis in adults with unexplained bronchiectasis. Copyright © 2016 Elsevier Inc. All rights reserved.

  19. Post-Tensioning Duct Air Pressure Testing Effects on Web Cracking

    Science.gov (United States)

    2015-01-01

    Nevada Department of Transportation (NDOT) post-tensioned concrete bridges have experienced web cracking near the post-tensioning ducts during the construction process. The ducts were air pressure tested to ensure the duct can successfully be grouted...

  20. Clinical features of pancreatic cystic neoplasms and its therapeutic strategy

    Directory of Open Access Journals (Sweden)

    ZHANG Jiali

    2016-05-01

    Full Text Available The detection rate of pancreatic cystic neoplasms obviously increases with the development of imaging technology. However, it is still difficult to make the differential diagnosis between different types of pancreatic cystic neoplasms. For pancreatic cystic neoplasms, the physical condition of the patient, tumor size, location, patient preference, and other potential factors should be considered to develop an individualized treatment. The incidence rate of complications is high, although the operative mortality of pancreatic cystic neoplasms is very low. Therefore, it is necessary to strictly make the treatment decision for pancreatic cystic neoplasms.

  1. Morphological study of pancreatic duct in red jungle fowl

    African Journals Online (AJOL)

    Jane

    2010-10-18

    Oct 18, 2010 ... the birds is considered to have four lobes: ventral, dorsal, third and splenic; with three ducts: ventral, dorsal and ... morphology of the pancreatic duct of birds in general and fowl in particular. Therefore, this study .... border of these cells were stained with purple color. (Figure 12). However, after being treated ...

  2. Chloride and potassium conductances of cultured human sweat ducts

    DEFF Research Database (Denmark)

    Novak, I; Pedersen, P S; Larsen, Erik Hviid

    1992-01-01

    The purpose of this study was to characterize the ion conductances, in particular those for Cl- and K+, of human sweat duct cells grown in primary culture. Sweat duct cells from healthy individuals were grown to confluence on a dialysis membrane, which was then mounted in a mini-Ussing chamber an...

  3. Sound waves in two-dimensional ducts with sinusoidal walls

    Science.gov (United States)

    Nayfeh, A. H.

    1974-01-01

    The method of multiple scales is used to analyze the wave propagation in two-dimensional hard-walled ducts with sinusoidal walls. For traveling waves, resonance occurs whenever the wall wavenumber is equal to the difference of the wavenumbers of any two duct acoustic modes. The results show that neither of these resonating modes could occur without strongly generating the other.

  4. Intracellular pH in rat pancreatic ducts

    DEFF Research Database (Denmark)

    Novak, I; Hug, M; Greger, R

    1997-01-01

    In order to study the mechanism of H+ and HCO3- transport in a HCO3- secreting epithelium, pancreatic ducts, we have measured the intracellular pH (pHi) in this tissue using the pH sensitive probe BCECF. We found that exposures of ducts to solutions containing acetate/acetic acid or NH4+/NH3 buff...

  5. Seismic assessment of the Pickering pressure relief duct

    International Nuclear Information System (INIS)

    Ghobarah, A.

    1995-05-01

    The objectives of the study are to examine the structural response of the Pickering pressure relief duct when subjected to earthquake ground motion and to estimate the seismic withstand capacity of various components of the structural system on the basis of performance criteria consistent with the safety function of the duct. (author). 24 refs., 16 tabs., 31 figs

  6. 14 CFR 23.1103 - Induction system ducts.

    Science.gov (United States)

    2010-01-01

    ... engine installations, each induction system duct must be— (1) Strong enough to prevent induction system... 14 Aeronautics and Space 1 2010-01-01 2010-01-01 false Induction system ducts. 23.1103 Section 23... AIRWORTHINESS STANDARDS: NORMAL, UTILITY, ACROBATIC, AND COMMUTER CATEGORY AIRPLANES Powerplant Induction System...

  7. Morphological study of pancreatic duct in red jungle fowl | Kadhim ...

    African Journals Online (AJOL)

    Morphological and histochemical study of the pancreas and pancreatic ducts of ten adult red jungle fowl (Gallus gallus spadiceus) were carried out by means of light microscopy. The bulk of the pancreas consists of a dorsal, ventral, third and small splenic lobe. Three pancreatic ducts were recognized as they join the ...

  8. Long duct nacelle aerodynamic development for DC-10 derivatives

    Science.gov (United States)

    Patel, S. P.; Donelson, J. E.

    1980-01-01

    The results are presented of a wind tunnel test utilizing a 4.7-percent-scale semispan model of the DC-10 in the Calspan 8-foot transonic wind tunnel. The effect of a revised long-duct nacelle shape on the channel velocities, the incremental drag relative to the baseline long-duct nacelle, and channel velocities for the baseline long-duct nacelle were determined and compared with data obtained at Ames. The baseline and the revised long-duct nacelles are representative of a CF6-50 mixed-flow configuration and were evaluated on a model of a proposed DC-10 stretched-fuselage configuration. The results showed that the revised long-duct nacelle has an appreciable effect on the inboard channel velocities, resulting in an increased channel Mach number. However, the pressure recovery on the nacelle afterbody was about the same for both nacelles. The lift curves for both long-duct nacelle configurations were the same. The channel pressures measured at Calspan were in good agreement with those measured at Ames for the baseline long-duct nacelle. The incremental drag for the revised nacelle was measured as two to four counts (three counts is approximately equal to one percent of the airplane drag) higher than that of the baseline long-duct nacelle.

  9. Radiopaque intrahepatic duct stones in plain radiograph: case report

    International Nuclear Information System (INIS)

    Kim, Mi Young; Suh, Chang Hae; Park, Chan Sup; Chung, Won Kyun

    1994-01-01

    We experienced 3 cases of intrahepatic duct stones detected on plain radiographs. The patients had history of multiple episodes of recurrent cholangitis. Radiographic characteristics of these stones included multiple, round or rectangular radiopaque densities surrounded by calcified rim; these densities showed a branching pattern along the intrahepatic ducts

  10. Surgical management of Stenson's duct injury using epidural catheter

    African Journals Online (AJOL)

    2012-07-05

    Jul 5, 2012 ... Successful management of parotid duct injury depends on early diagnosis and appropriate intervention, failing of ... to diagnose and manage the parotid duct injuries using an “epidural catheter” which is often used for inducing spinal anesthesia. .... Water-soluble contrast media have a definite advantage.

  11. Magnetic resonance imaging of Muellerian duct anomalies in children

    International Nuclear Information System (INIS)

    Li, Yi; Phelps, Andrew; Zapala, Matthew A.; MacKenzie, John D.; MacKenzie, Tippi C.; Courtier, Jesse

    2016-01-01

    Muellerian duct anomalies encompass a wide variety of disorders resulting from abnormalities in the embryological development of the Muellerian ducts. In the prepubertal pediatric population, Muellerian duct anomalies are often incidental findings on studies obtained for other reasons. The onset of menses can prompt more clinical symptoms. Proper characterization of Muellerian duct anomalies is important because these anomalies can affect the development of gynecological disorders as well as fertility. Muellerian duct anomalies also carry a high association with other congenital anomalies, particularly renal abnormalities. MRI is widely considered the best modality for assessing Muellerian duct anomalies; it provides multiplanar capability, clear anatomical detail and tissue characterization without ionizing radiation. MRI allows for careful description of Muellerian duct anomalies, often leading to classification into the most widely accepted classification system for Muellerian duct anomalies. This system, developed by the American Society of Reproductive Medicine, includes seven subtypes: uterine agenesis/hypoplasia, unicornuate, didelphys, bicornuate, septate, arcuate, and diethylstilbestrol (DES) drug-related uterus. In cases of complex anomalies that defy classification, MRI allows detailed depiction of all components of the anatomical abnormality, allowing for proper management and surgical planning. (orig.)

  12. Consistent approach to air-cleaning system duct design

    International Nuclear Information System (INIS)

    Miller, W.H.; Ornberg, S.C.; Rooney, K.L.

    1981-01-01

    Nuclear power plant air-cleaning system effectiveness is dependent on the capability of a duct system to safely convey contaminated gas to a filtration unit and subsequently to a point of discharge. This paper presents a logical and consistent design approach for selecting sheet metal ductwork construction to meet applicable criteria. The differences in design engineers' duct construction specifications are acknowledged. Typical duct construction details and suggestions for their effective use are presented. Improvements in duct design sections of ANSI/ASME N509-80 are highlighted. A detailed leakage analysis of a control room HVAC system is undertaken to illustrate the effects of conceptual design variations on duct construction requirements. Shortcomings of previously published analyses and interpretations of a current standard are included

  13. Topological mixing study of non-Newtonian duct flows

    Science.gov (United States)

    Speetjens, Michel; Metcalfe, Guy; Rudman, Murray

    2006-10-01

    Tracer advection of non-Newtonian fluids in reoriented duct flows is investigated in terms of coherent structures in the web of tracer paths that determine transport properties geometrically. Reoriented duct flows are an idealization of in-line mixers, encompassing many micro and industrial continuous mixers. The topology of the tracer dynamics of reoriented duct flows is Hamiltonian. As the stretching per reorientation increases from zero, we show that the qualitative route from the integrable state to global chaos and good mixing does not depend on fluid rheology. This is due to a universal symmetry of reoriented duct flows, which we derive, controlling the topology of the tracer web. Symmetry determines where in parameter space global chaos first occurs, while increasing non-Newtonian effects delays the quantitative value of onset. Theory is demonstrated computationally for a representative duct flow, the rotated arc mixing flow.

  14. Transhepatic cholangiography in patients with nondilated bile ducts

    International Nuclear Information System (INIS)

    Teplick, S.K.; Flick, P.; Brandon, J.C.; Haskin, P.H.; Goldstein, R.C.; Brennan, C.

    1988-01-01

    Transhepatic cholangiography was performed in 90 patients with suspected biliary tract disease and nondilated ducts. A21- or 22-gauge needle was used, and the number of passes were not limited. The bile ducts were visualized in 62 of 90 patients, and the study was diagnostic in 58 of 90. In 13 patients, the ducts were not adequately opacified, and in one, a left hepatic duct stone was missed. Seventeen of 62 cholangiograms were abnormal (eight strictures, six calculi three cases of poor emptying); eight of the 17 patients underwent percutaneous transhepatic biliary drainage. Twenty-one of 90 patients had complications. Two deaths resulted from sepsis and hemorrhage. Sixteen of 90 patients had significant abdominal pain lasting several hours to several days. One patient had bile peritonitis, and one each developed increasing pancreatitis and transient bacteremia. The authors conclude that transhepatic cholangiography should not be the initial invasive procedure of choice in patients with nondilated ducts

  15. The Role of Spiral Multidetector Dynamic CT in the Study of Williams-Campbell Syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Scioscio, V. di; Zompatori, M.; Mistura, I.; Montanari, P.; Santilli, L.; Luccaroni, R.; Sverzellati, N. [Medical Univ. of Bologna, S. Orsola-Malpighi Policlinic (Italy). Dept. of Radiology

    2006-10-15

    Williams-Campbell syndrome is a cystic bronchiectatic disease secondary to deficiency or defect of cartilaginous plates in the wall of the airways. In the literature, two main forms are suggested: congenital and acquired (post-infectious). The most frequent symptoms are represented by recurrent pulmonary infections from childhood. Multislice spiral dynamic CT has a major role in the study of cystic pulmonary disease and in differentiating Williams-Campbell syndrome from the other causes of cystic bronchiectasis, in which even lung function tests can give deceptive results.

  16. A Plug-and-Play Duct System Evaluation

    Energy Technology Data Exchange (ETDEWEB)

    Beach, Robert [IBACOS, Inc., Pittsburgh, PA (United States); Dickson, Bruce [IBACOS, Inc., Pittsburgh, PA (United States); Grisolia, Anthony [IBACOS, Inc., Pittsburgh, PA (United States); Poerschke, Andrew [IBACOS, Inc., Pittsburgh, PA (United States); Rapport, Ari [IBACOS, Inc., Pittsburgh, PA (United States)

    2017-07-10

    This report describes an air distribution system composed of a series of uniformly-sized ducts that terminate in rooms throughout the home and return to a central manifold, similar in fashion to a “home-run” cross-linked polyethylene plumbing system. With a well-designed manifold, each duct receives an equal static pressure potential for airflow from the air handling unit, and the number of needed ducts for each room are simply attached to fittings located on the manifold; in this sense, the system is plug-and-play (PnP). As indicated, all ducts in the PnP system are identical in size and small enough to fit in the ceiling and wall cavities of a house (i.e., less than 3.5-in. outer diameter). These ducts are also more appropriately sized for the lower airflow requirements of modern, energy-efficient homes; therefore, the velocity of the air moving through the duct is between that of conventional duct systems (approximately 700 ft/min) and high-velocity systems (more than 1,500 ft/min) on the market today. The PnP duct system uses semi-rigid plastic pipes, which have a smooth inner wall and are straightforward to install correctly, resulting in a system that has minimal air leakage. However, plastic ducts are currently not accepted by code for use in residential buildings; therefore, the project team considered other duct materials for the system that are currently accepted by code, such as small-diameter, wirehelix, flexible ductwork.

  17. A Plug-and-Play Duct System Evaluation

    Energy Technology Data Exchange (ETDEWEB)

    Beach, R. [IBACOS, Inc., Pittsburgh, PA (United States); Dickson, B. [IBACOS, Inc., Pittsburgh, PA (United States); Grisolia, A. [IBACOS, Inc., Pittsburgh, PA (United States); Poerschke, A. [IBACOS, Inc., Pittsburgh, PA (United States); Rapport, A. [IBACOS, Inc., Pittsburgh, PA (United States)

    2017-07-01

    This report describes an air distribution system composed of a series of uniformly-sized ducts that terminate in rooms throughout the home and return to a central manifold, similar in fashion to a “home-run” cross-linked polyethylene plumbing system. With a well-designed manifold, each duct receives an equal static pressure potential for airflow from the air handling unit, and the number of needed ducts for each room are simply attached to fittings located on the manifold; in this sense, the system is plug-and-play (PnP). As indicated, all ducts in the PnP system are identical in size and small enough to fit in the ceiling and wall cavities of a house (i.e., less than 3.5-in. outer diameter). These ducts are also more appropriately sized for the lower airflow requirements of modern, energy-efficient homes; therefore, the velocity of the air moving through the duct is between that of conventional duct systems (approximately 700 ft/min) and high-velocity systems (more than 1,500 ft/min) on the market today. The PnP duct system uses semi-rigid plastic pipes, which have a smooth inner wall and are straightforward to install correctly, resulting in a system that has minimal air leakage. However, plastic ducts are currently not accepted by code for use in residential buildings; therefore, the project team considered other duct materials for the system that are currently accepted by code, such as small-diameter, wirehelix, flexible ductwork.

  18. The diagnosis of cystic fibrosis.

    Science.gov (United States)

    De Boeck, Kris; Vermeulen, Francois; Dupont, Lieven

    2017-06-01

    Establishing the diagnosis of cystic fibrosis (CF) is straight forward in the majority of patients: they present with a clear clinical picture (most frequently chronic respiratory symptoms plus malabsorption), the sweat chloride value is>60mmol/L and two known disease causing CFTR mutations are identified. In less than 5% of subjects, mainly those with a milder or limited phenotype, the diagnostic process is more complex, because initial diagnostic test results are inconclusive: sweat chloride concentration in the intermediate range, less than 2 CF causing mutations identified or both. These patients should be referred to expert centers where bioassays of CFTR function like nasal potential difference measurement or intestinal current measurement can be done. Still, in some patients, despite symptoms compatible with CF and some indication of CFTR dysfunction (e.g. only intermediate sweat chloride value), diagnostic criteria are not met (e.g. only 1 CFTR mutation identified). For these subjects, the term CFTR related disorder (CFTR-RD) is used. Patients with disseminated bronchiectasis, congenital bilateral absence of the vas deferens and acute or recurrent pancreatitis may fall in this category. CF has a very wide disease spectrum and increasingly the diagnosis is being made during adult life, mainly in subjects with milder phenotypes. In many countries, nationwide CF newborn screening (NBS) has been introduced. In screen positive babies, the diagnosis of CF must be confirmed by a sweat test demonstrating a sweat chloride concentration above 60mmol/L. To achieve the benefit of NBS, every baby in whom the diagnosis of CF is confirmed must receive immediate follow-up and treatment in a CF reference center. CF NBS is not full proof: some diagnoses will be missed and in some babies the diagnosis cannot be confirmed nor ruled out with certainty. Screening algorithms that include gene sequencing will detect a high number of such babies that are screen positive with an

  19. Laryngeal adenoid cystic carcinoma: case report

    Directory of Open Access Journals (Sweden)

    André Del Negro

    Full Text Available CONTEXT: Adenoid cystic carcinomas are malignant tumors that occur in both the major and the minor salivary glands. A laryngeal location is rare because of the paucity of accessory salivary glands in this area. Adenoid cystic carcinomas account for less than 1% of all malignant tumors in the larynx, and only about 120 cases have been reported in the literature. These tumors have a slight female predisposition, and their peak incidence is in the fifth and sixth decades of life. In this article, we describe a case of laryngeal adenoid cystic carcinoma and discuss its clinical characteristics and treatment. CASE REPORT: We report on a case of laryngeal adenoid cystic carcinoma in a 55 year-old female patient who presented with dyspnea and hoarseness. Features of the diagnostic and therapeutic evaluation are described and the clinical management of such cases is outlined. The clinical course, definitive treatment strategy and surgical procedure, and also adjuvant treatment with irradiation are discussed. Although the tumor is radiosensitive, it is not radiocurable.

  20. Retrorectal Cystic Hamartoma: A Problematic 'Tail'

    African Journals Online (AJOL)

    had attained menarche at 14 years of age and had normal menstrual cycle. General physical and per abdominal ... uncommon developmental cystic lesion occurring in this space which mostly occurs in middle-aged females. We ... Singh Marg, New Delhi, India. Address for correspondence: Dr. Suhani, Senior Resident,.

  1. Prognosis in Cystic Fibrosis: Trends and Predictors

    NARCIS (Netherlands)

    Slieker, M.G.

    2008-01-01

    Cystic fibrosis (CF) is a multisystem disease affecting the digestive system, sweat glands, and the reproductive tract, but progressive lung disease continues to be the major cause of morbidity and mortality. Patients develop chronic infection of the respiratory tract with a characteristic array of

  2. Cystic echinococcosis in sub-Saharan Africa

    NARCIS (Netherlands)

    Wahlers, Kerstin; Menezes, Colin N.; Wong, Michelle L.; Zeyhle, Eberhard; Ahmed, Mohammed E.; Ocaido, Michael; Stijnis, Cornelis; Romig, Thomas; Kern, Peter; Grobusch, Martin P.

    2012-01-01

    Cystic echinococcosis is regarded as endemic in sub-Saharan Africa; however, for most countries only scarce data, if any, exist. For most of the continent, information about burden of disease is not available; neither are data for the animal hosts involved in the lifecycle of the parasite, thus

  3. Barriers to adherence in cystic fibrosis

    DEFF Research Database (Denmark)

    Bregnballe, Vibeke; Schiøtz, Peter Oluf

    2012-01-01

    Danish patients with cystic fibrosis aged 14 to 25 years and their parents. Conclusions: The present study showed that the majority of adolescents with CF and their parents experienced barriers to treatment adherence. Patients and parents agreed that the three most common barriers encountered lack...

  4. Cystic fibrosis year in review 2016.

    Science.gov (United States)

    Savant, Adrienne P; McColley, Susanna A

    2017-08-01

    In this article, we highlight cystic fibrosis (CF) research and case reports published in Pediatric Pulmonology during 2016. We also include articles from a variety of journals that are thematically related to these articles, or are of special interest to clinicians. © 2017 Wiley Periodicals, Inc.

  5. Pseudomonas aeruginosa biofilms in cystic fibrosis

    DEFF Research Database (Denmark)

    Høiby, Niels; Ciofu, Oana; Bjarnsholt, Thomas

    2010-01-01

    The persistence of chronic Pseudomonas aeruginosa lung infections in cystic fibrosis (CF) patients is due to biofilm-growing mucoid (alginate-producing) strains. A biofilm is a structured consortium of bacteria, embedded in a self-produced polymer matrix consisting of polysaccharide, protein...

  6. Psychosocial problems in children with cystic fibrosis

    DEFF Research Database (Denmark)

    Bregnballe, V; Thastum, M; Schiøtz, P O

    2007-01-01

    AIM: To compare the well-being of children (7-14 years) with cystic fibrosis (CF) (n = 43) with the well-being of healthy controls (n = 1121). METHODS: The self-report questionnaire Beck Youth Inventories (BYI) was used to study depression, anxiety, anger, disruptive behaviour and self...

  7. Immunoreactive trypsin and neonatalscreening for cystic fibrosis

    International Nuclear Information System (INIS)

    Travert, G.; Laroche, D.; Blandin, C.; Pasquet, C.

    1988-01-01

    Immunoreactive trypsin (IRT) was measured in dried blood spots from 160.822 five-day-old babies as a part of a regionwide neonatal screening program for cystic fibrosis. A second test was performed for 492 babies in whom blood IRT levels were found greater than 900 μg/l; retesting revealed persistent elevation in 55. Sweat testing confirmed cystic fibrosis in 43 babies, but results were normal in 12. During the course of this study, a total of 51 cystic fibrosis babies were identified: 43 by newborn screening, 6 because they had meconium ileus; so, early diagnosis was achieved in 49 cases out of 51. Two newborn babies did not have elevated IRT and they were missed by the screening test. Our results confirm that elevated blood IRT is characteristic of newborn babies with cystic fibrosis and show that this test has an excellent specificity (99.7%) and a good sensitivity (95%) when used as a neonatal screening test [fr

  8. Respiratory bacterial infections in cystic fibrosis

    DEFF Research Database (Denmark)

    Ciofu, Oana; Hansen, Christine R; Høiby, Niels

    2013-01-01

    PURPOSE OF REVIEW: Bacterial respiratory infections are the main cause of morbidity and mortality in patients with cystic fibrosis (CF). Pseudomonas aeruginosa remains the main pathogen in adults, but other Gram-negative bacteria such as Achromobacter xylosoxidans and Stenotrophomonas maltophilia...

  9. Mature cystic teratomas: Relationship between histopathological ...

    African Journals Online (AJOL)

    ... tumor size, symptoms related to MCT and laterality of the tumor did not differ among the patients according to the MCT contents. Conclusions: Our findings suggest no relationship between the clinical features and histopathological contents of MCTs. Key words: Histopathological contents, mature cystic teratoma, ovarian, ...

  10. Nutritional assessment in children with cystic fibrosis

    Science.gov (United States)

    Optimal nutrition, including consuming 35–40% of calories (kcal) as fat, is a vital part of the management of cystic fibrosis (CF), and involves accurate assessment of dietary intake. We compared 3 methods of nutritional assessment in 8– to 14-year-old children (n=20) with CF: 1) a 24-h Dietary Reca...

  11. Zinc supplementation in children with cystic fibrosis

    Science.gov (United States)

    Cystic fibrosis (CF) leads to malabsorption of macro- and micronutrients. Symptomatic zinc deficiency has been reported in CF but little is known about zinc homeostasis in children with CF. Zinc supplementation (Zn suppl) is increasingly common in children with CF but it is not without theoretcial r...

  12. Congenital cystic adenomatoid malformation: case report | Wafula ...

    African Journals Online (AJOL)

    Congenital cystic adenomatoid malformation of the lung is a rare lesion that typically manifests as severe progressive respiratory distress in the neonate secondary to expansion of the affected lung. We present a neonate in whom this condition was diagnosed and managed at the Aga Khan University Hospital. In presenting ...

  13. [Intra-cystic renal calcium milk].

    Science.gov (United States)

    Meunier, B; Médart, L; Massart, J P; Collignon, L

    2015-02-01

    Intra-cystic renal calcium milk is a rare entity. The authors report a clinical case, and describe the radiographic and tomodensitometric appearances. This 50 year old patient has been followed up for more than ten years for urinary lithiasis with recurrent pain.

  14. CYSTIC AMELOBLASTOMA: A CLINICO-PATHOLOGIC REVIEW

    African Journals Online (AJOL)

    a tertiary health care centre. Materials: All cases diagnosed as cystic ameloblastoma in the ..... Unicystic amelobla- stoma. A prognostically distinct entity. Cancer. 1977;40: 2278-2285. 4. Ackermann GL, Altini M, Shear M: The unicystic ameloblastoma: A clinicopathologic study of 57 cases. J Oral Pathol. 1988;17: 541-546. 5.

  15. Cystic echinococcosis: prevalence and economic significance in ...

    African Journals Online (AJOL)

    A cross-sectional study was conducted from November 2015 to April 2016 at ELFORA export abattoir to determine the prevalence, cyst viability, organ distribution and economic significance of small ruminant cystic echinococcosis. A total of 850 small ruminants (400 sheep and 450 goats), were examined for the presence of ...

  16. Cystic echinococcosis: Future perspectives of molecular epidemiology

    Science.gov (United States)

    Human cystic echinococcosis (CE) has been conceived to be caused predominantly by Echinococcus granulosus sensu stricto (the dog-sheep strain). Recent molecular approaches on CE, however, have revealed that human cases are also commonly caused by another species, Echinococcus canadensis. All indices...

  17. Common bile duct pathologies at nawabshah

    International Nuclear Information System (INIS)

    Talpur, A.A.; Memon, J.M.; Ansari, A.G.

    2007-01-01

    To determine the causes, presentation, management and outcome of Common Bile Duct (CBD) pathologies. All patients who presented with CBD pathologies. Data of all the patients with CBD pathologies was collected and entered on a proforma, including their complaints, positive examination findings, investigations, diagnosis, procedure performed and its outcome. During the study period 45 patients presented with CBD pathology. Amongst them 14 were males and the rest females (31), with a mean age of 36.7 years. Around 67% patients had choledocholithiasis as the commonest cause. Exploration of the CBD with T-tube insertion was the commonest procedure, performed in 69% patients. About 4% patients had retained stones and 20% developed wound infection. Mean hospital stay was 13 days. Most common pathology involving the CBD was secondary stones; 95% patients had associated gall stones also. (author)

  18. Granular Cell Tumour of the Bile Duct in Association with Intrahepatic Bile Duct Adenomas

    OpenAIRE

    Schweiger, F; Radhi, J; Coop, FW; Murphy, RW

    1994-01-01

    Granular cell tumour of the extrahepatic biliary tract is a rare benign lesion likely of neurogenic origin. Review of the previously reported cases indicates that almost all patients are female, and the majority is Black. Symptoms usually are those of biliary obstruction or cholecystitis. Surgical resection of the tumour is curative. Intrahepatic bile duct adenoma is another rare benign biliary neoplasm that does not manifest clinically but can be confused with metastatic carcinoma, cholangio...

  19. Nutrient Status of Adults with Cystic Fibrosis

    Science.gov (United States)

    GORDON, CATHERINE M.; ANDERSON, ELLEN J.; HERLYN, KAREN; HUBBARD, JANE L.; PIZZO, ANGELA; GELBARD, RONDI; LAPEY, ALLEN; MERKEL, PETER A.

    2011-01-01

    Nutrition is thought to influence disease status in patients with cystic fibrosis (CF). This cross-sectional study sought to evaluate nutrient intake and anthropometric data from 64 adult outpatients with cystic fibrosis. Nutrient intake from food and supplements was compared with the Dietary Reference Intakes for 16 nutrients and outcomes influenced by nutritional status. Attention was given to vitamin D and calcium given potential skeletal implications due to cystic fibrosis. Measurements included weight, height, body composition, pulmonary function, and serum metabolic parameters. Participants were interviewed about dietary intake, supplement use, pulmonary function, sunlight exposure, and pain. The participants’ mean body mass index (±standard deviation) was 21.8±4.9 and pulmonary function tests were normal. Seventy-eight percent used pancreatic enzyme replacement for malabsorption. Vitamin D deficiency [25-hydroxyvitamin D (25OHD)<37.5 nmol/L] was common: 25 (39%) were deficient despite adequate vitamin D intake. Lipid profiles were normal in the majority, even though total and saturated fat consumption represented 33.0% and 16.8% of energy intake, respectively. Reported protein intake represented 16.9% of total energy intake (range 10%–25%). For several nutrients, including vitamin D and calcium, intake from food and supplements in many participants exceeded recommended Tolerable Upper Intake Levels. Among adults with cystic fibrosis, vitamin D deficiency was common despite reported adequate intake, and lipid profiles were normal despite a relatively high fat intake. Mean protein consumption was adequate, but the range of intake was concerning, as both inadequate or excessive intake may have deleterious skeletal effects. These findings call into question the applicability of established nutrient thresholds for patients with cystic fibrosis. PMID:18060897

  20. Transcatheter stenting of arterial duct in duct-dependent congenital heart disease

    Directory of Open Access Journals (Sweden)

    Đukić Milan

    2013-01-01

    Full Text Available Introduction. Critical congenital heart diseases (CHD are mostly duct-dependent and require stable systemic-pulmonary communication. In order to maintain patency of the ductus arteriosus (DA, the first line treatment is Prostaglandin E1 and the second step is the surgical creation of aortic-pulmonary shunt. To reduce surgical risk in neonates with the critical CHD, transcatheter stenting of DA can be performed in selected cases. Case Outline. A four-month old infant was diagnosed with the pulmonary artery atresia with ventricular septal defect (PAA/VSD. The left pulmonary artery was perfused from DA, and the right lung through three major aortopulmonary collaterals (MAPCAs. A coronary stent was placed in the long and critically stenotic DA, with final arterial duct diameter of 3.5 mm, and significantly increased blood supply to the left lung. After the procedure, the infant’s status was improved with regard to arterial oxygen saturation, feeding and weight gain. During the follow-up, one year later, aortography revealed in-stent stenosis. The left pulmonary artery, as well as the branches, was well-developed and the decision was made to proceed with further surgical correction. Conclusion. Stenting of DA can be an effective alternative to primary surgical correction in selected patients with duct-dependent CHD.

  1. Impaired regulatory volume decrease in freshly isolated cholangiocytes from cystic fibrosis mice: implications for cystic fibrosis transmembrane conductance regulator effect on potassium conductance.

    Science.gov (United States)

    Cho, Won Kyoo; Siegrist, Vicki J; Zinzow, Wendy

    2004-04-09

    Various K(+) and Cl(-) channels are important in cell volume regulation and biliary secretion, but the specific role of cystic fibrosis transmembrane conductance regulator in cholangiocyte cell volume regulation is not known. The goal of this research was to study regulatory volume decrease (RVD) in bile duct cell clusters (BDCCs) from normal and cystic fibrosis (CF) mouse livers. Mouse BDCCs without an enclosed lumen were prepared as described (Cho, W. K. (2002) Am. J. Physiol. 283, G1320-G1327). The isotonic solution consisted of HEPES buffer with 40% of the NaCl replaced with isomolar amounts of sucrose, whereas hypotonic solution was the same as isotonic solution without sucrose. The cell volume changes were indirectly assessed by measuring cross-sectional area (CSA) changes of the BDCCs using quantitative videomicroscopy. Exposure to hypotonic solutions increased relative CSAs of normal BDCCs to 1.20 +/- 0.01 (mean +/- S.E., n = 50) in 10 min, followed by RVD to 1.07 +/- 0.01 by 40 min. Hypotonic challenge in CF mouse BDCCs also increased relative CSA to 1.20 +/- 0.01 (n = 53) in 10 min but without significant recovery. Coadministration of the K(+)-selective ionophore valinomycin restored RVD in CF mouse BDCCs, suggesting that the impaired RVD was likely from a defect in K(+) conductance. Moreover, this valinomycin-induced RVD in CF mice was inhibited by 5-nitro-2'-(3-phenylpropylamino)-benzoate, indicating that it is not from nonspecific effects. Neither cAMP nor calcium agonists could reverse the impaired RVD seen in CF cholangiocytes. Our conclusion is that CF mouse cholangiocytes have defective RVD from an impaired K(+) efflux pathway, which could not be reversed by cAMP nor calcium agonists.

  2. Treatment of infants with congenital nasolacrimal duct obstruction

    Directory of Open Access Journals (Sweden)

    Zhuo Chen

    2014-07-01

    Full Text Available AIM: To explore the different ages of congenital nasolacrimal duct obstruction in infants, take different treatment methods at different times. METHODS:The 87 cases of 102 children were divided into three different age groups: the first group of 25d-3mo of age 21 cases 26 eyes; The second group >3mo-7mo 31 cases 36 eyes; The third group >7-24mo of age 35 cases 40 eyes. For the first group of infants, the implementation of the lacrimal sac nasolacrimal duct massage + eye drops; for the second group of infants, carry lacrimal pressure washing treatment; for the third group of infants, the implementation of the nasolacrimal duct probing treatment. RESULTS:The first group of children through the nasolacrimal duct sac massage + drops tobramycin eye drops treatment unobstructed 12, the cure rate was 46.2%; The second group of children through pressurized irrigation treatment lacrimal patency by 33, the cure rate was 91.7%; The third group of children through the nasolacrimal duct probing unobstructed 36 treatment, the cure rate was 90.0%. The second and third group were better than the first group(χ2=15.71, Pχ2=15.27, Pχ2=0.02, P>0.05.CONCLUSION:Infants with congenital nasolacrimal duct obstruction should distinguish between ages, taking different treatments, in order to obtain a better therapeutic effect, and lacrimal pressure washing is the preferred way of treating infants with congenital nasolacrimal duct obstruction.

  3. A case of prenatally detected hepatic cyst communicating with the hepatic duct

    Directory of Open Access Journals (Sweden)

    Shinsuke Katsuno

    2014-10-01

    Full Text Available Here, we describe the case of a 9-year-old girl. During the patient's prenatal period, her mother had suffered domestic violence perpetrated by the patient's father. On maternal ultrasonography and magnetic resonance imaging, an intraabdominal cystic lesion was detected in the fetus at around the 30th prenatal week. The patient was delivered normally, and there were no evident anomalies on her body. Computed tomography with drip infusion cholangiography and percutaneous retrograde transhepatic cholangiography demonstrated an intrahepatic cyst of approximately 3 cm in diameter, which was located at S5–S8 and communicated with the confluence of the bilateral hepatic ducts. The cyst is clinically conjectured to be a solitary intrahepatic biliary cyst. However, it remains possible that the cyst is a ciliated hepatic foregut cyst or indicates hepatic injury that may have occurred as a result of domestic violence to the mother. Careful, long-term observation of the patient will be continued to ensure that any malignant transformation is not missed.

  4. Physical exercise training for cystic fibrosis.

    Science.gov (United States)

    Radtke, Thomas; Nevitt, Sarah J; Hebestreit, Helge; Kriemler, Susi

    2017-11-01

    Physical exercise training may form an important part of regular care for people with cystic fibrosis. This is an update of a previously published review. To assess the effects of physical exercise training on exercise capacity by peak oxygen consumption, pulmonary function by forced expiratory volume in one second, health-related quality of life and further important patient-relevant outcomes in people with cystic fibrosis. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register which comprises references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings.Date of the most recent search: 04 May 2017.We searched ongoing trials registers (clinicaltrials.gov and the WHO ICTRP). Date of most recent search: 10 August 2017. All randomised and quasi-randomised controlled clinical trials comparing exercise training of any type and a minimum duration of two weeks with conventional care (no training) in people with cystic fibrosis. Two authors independently selected studies for inclusion, assessed methodological quality and extracted data. The quality of the evidence was assessed using the GRADE system. Of the 83 studies identified, 15 studies which included 487 participants, met the inclusion criteria. The numbers in each study ranged from nine up to 72 participants; two studies were in adults, seven were in children and adolescents and six studies included all age ranges. Four studies of hospitalised participants lasted less than one month and 11 studies were outpatient-based, lasting between two months and three years. The studies included participants with a wide range of disease severity and employed differing levels of supervision with a mixture of types of training. There was also wide variation in the quality of the included studies.This systematic review shows very low- to low-quality evidence from both short- and long-term studies that in people

  5. [Ophthalmological manifestations of Cornelia de Lange syndrome: Case report and review of the literature].

    Science.gov (United States)

    Avgitidou, G; Cursiefen, C; Heindl, L M

    2015-05-01

    A 2-year-old boy suffering from Cornelia de Lange syndrome, presented with mucopurulent ocular discharge and epiphora since birth. Irrigation and probing of the nasolacrimal system revealed and successfully treated bilateral nasolacrimal duct obstructions. Cornelia de Lange syndrome is characterized not only by typical facial features, visceral and urogenital anomalies but also by ophthalmological manifestations in 99% of cases. The most common ophthalmological disorders are synophrys, blepharitis, epiphora, hypertrichosis of the eyebrows and eyelashes, myopia, ptosis and nasolacrimal duct obstruction.

  6. Primary adenocarcinoid tumor of the ovary arising in mature cystic teratoma. A case report.

    Science.gov (United States)

    Gungor, T; Altinkaya, O; Ozat, M; Sirvan, L; Yalcin, H; Mollamahmutoglu, L

    2009-01-01

    Primary carcinoid tumors of the ovary are rare, forming 0.3 % of all carcinoid tumors and less than 0.1 % of ovarian malignancies. We present a case of a 47-year-old woman with abnormal vaginal bleeding and abdominal discomfort. Pelvic ultrasonography revealed a right-sided, 4 cm solid ovarian mass. At laparoscopy, a smooth surfaced, firm and mobile right adnexal mass with solid and cystic portions was detected and the tumor was excised. Pathologic and immunohistochemical examination revealed carcinoid tumor. After the evaluation of gastrointestinal and respiratory systems by imaging and endoscopic studies, no evidence of tumor tissue or metastases were found. Based on this finding, total abdominal hysterectomy, bilateral salpingo-oophorectomy, omentectomy, and appendectomy were performed and pelvic-paraaortic lymph nodes were also removed. All histological findings were similar to the features of mature cystic teratoma and carcinoid tumor. Examination of the resected lymphatic, omental and appendiceal tissue indicated no tumoral invasion. Thus, the diagnosis was primary ovarian carcinoid arising from a mature cystic teratoma. This case, in the setting of a normal appendix and negative workup for an extraovarian origin, is one of a primary ovarian adenocarcinoid tumor. The primary carcinoid tumor should be differentiated from a metastatic carcinoid inside the ovary, which is always bilateral. It is important to be aware of this unusual entity in diagnoses of ovarian tumors, even in the absence of any clinical symptoms of carcinoid syndrome.

  7. Cystic adventitial degeneration of the popliteal artery - the diagnostic value of duplex sonography

    Energy Technology Data Exchange (ETDEWEB)

    Brodmann, Marianne E-mail: marianne.brodmann@kfunigraz.ac.at; Stark, Gerhard; Pabst, Edmund; Seinost, Gerald; Schweiger, Wolfgang; Szolar, Dieter; Pilger, Ernst

    2001-06-01

    Cystical adventitial degeneration of the popliteal artery is a disorder which is difficult to diagnose, due to the similarity of the symptoms of people presenting with peripheral arterial occlusive disease (PAOD) or popliteal entrapment syndrome. The only thing that differs from patients suffering from PAOD is the lack of typical risk factors for arteriosclerosis. Typical diagnostic procedures like conventional angiography or magnetic resonance imaging angiography can be negative, too and therefore misleading. The only which is crucial in the diagnosis of cystic adventitial degeneration of the popliteal artery is to know the morphological background of this disorder, namely that it is a cyst of the adventitia of the artery which leads to a dynamic exercise-dependent flow inhibition. We present a 57-year old white male who had a week's history of intermittent claudication in his left calf. He was lacking of typical risk factors for arteriosclerosis and on first examination all pulses in both lower extremities were palpable and Doppler index on both legs was >1. Only duplexsonography revealed a cystic formation impressing the left popliteal artery in the height of the rift in the popliteal joint.

  8. Postoperative outcomes and quality of life in patients with cystic fibrosis undergoing laparoscopic cholecystectomy: a retrospective study.

    Science.gov (United States)

    Cogliandolo, Andrea; Patania, Mariangela; Currò, Giuseppe; Chillè, Giovanni; Magazzù, Giuseppe; Navarra, Giuseppe

    2011-06-01

    Approximately 28% of the patients with cystic fibrosis are affected by cholelythiasis. More than 40% of them have a symptomatic disease, which would mandate cholecystectomy. The aim of this study was to review surgical and respiratory outcomes and quality of life scores of cystic fibrosis patients undergoing laparoscopic cholecystectomy for symptomatic cholelythiasis to verify the hypothesis that cholecystectomy is a low-risk operation by laparoscopy, not affecting unfavorably respiratory function and quality of life. Study group was consisted of 9 patients with a mean age of 24.8±8.1 years (range, 15 to 38 y), 2 male and 7 female patients, with cystic fibrosis and symptomatic cholelithiasis. Three patients also presented common bile duct stones. All the patients underwent perioperative Positive End-Expiratory Pressure mask sessions and aggressive antibiotic regimens. At the middle of the antibiotics regimen period, a standard laparoscopic cholecystectomy was performed. In the 3 cases with common duct lithiasis, the so-called "rendezvous" technique was carried out. Preoperatively, intraoperatively, and postoperatively, respiratory function was strictly monitored by the evaluation of SO2 and of the forced expiratory volume in 1 second (FEV1). Preoperatively and 6 months after laparoscopic cholecystectomy the Gastro Intestinal Quality of Life Index was evaluated on all patients. All the operations were completed laparoscopically. No mortality was observed. The intraoperative mean SO2 was 89.0%±5.6% (range, 80% to 95%), versus 82.8%±8.5% (range, 66% to 91%) at the extubation (P=0.006). Intraoperative respiratory functions were stable in 6 patients. In 3 patients, a severe bronchospasm occurred determining marked desaturation. Preoperative mean FEV1 was 70.5%±7.0% (range, 55% to 75%) versus 61.8%±13.2% (range, 39% to 80%) 48 hours after the operation (P=0.132). The 3 patients, who experienced intraoperatively severe bronchospasm, reported a 48 hours postoperative

  9. BACTERIOLOGICAL INVESTIGATION ON Fasciola hepatica AND CATTLE BILIARY DUCTS

    Directory of Open Access Journals (Sweden)

    A. Panebianco

    2009-03-01

    Full Text Available The aim of this work was to investigate the occurrence of bacteria in Fasciola hepatica and into cattle biliary ducts containing the parasite. A total of 24 liver and 58 F. hepatica samples were analysed. In all biliary ducts and in 62,06% of parasite Enterobacteriaceae were isolated. The bacterial specie more frequently isolated from parasite were Citrobacter freundii (34%, Proteus mirabilis (18%, Providencia rettgeri (12%, Staphylococcus spp. (18%, Enterobacter spp. (12%. There doesn’t appear to be a correlation between bacterial specie from parasite and ducts. The Authors conclude with some related inspective consideration.

  10. Effects of explosion-generated shock waves in ducts

    International Nuclear Information System (INIS)

    Busby, M.R.; Kahn, J.E.; Belk, J.P.

    1976-01-01

    An explosion in a space causes an increase in temperature and pressure. To quantify the challenge that will be presented to essential components in a ventilation system, it is necessary to analyze the dynamics of a shock wave generated by an explosion, with attention directed to the propagation of such a wave in a duct. Using the equations of unsteady flow and shock tube theory, a theoretical model has been formulated to provide flow properties behind moving shock waves that have interacted with various changes in duct geometry. Empirical equations have been derived to calculate air pressure, temperature, Mach number, and velocity in a duct following an explosion

  11. A remote joint system for large vacuum ducts

    International Nuclear Information System (INIS)

    Hagmann, D.B.; Coughlan, J.B.

    1983-01-01

    A large remote vacuum duct joining system has been developed for fusion machines that uses several two-jaw screwdriven clamps. The preferred location for clamp installation is inside the vacuum duct where access space is available for the actuating device. It also decreases space needed for handling operations exterior to the duct. The clamp system is unique in that it is low cost, applies force directly over the seal, permits leak testing to the seal annulus, is highly reliable, can be remotely replaced, and is usable on a variety of other applications

  12. Massive Cervico-Lingual Cystic Hygroma

    African Journals Online (AJOL)

    alcohol consumption. Karyotype abnormalities are found in 25 -70% of children with CH. The Genetic syndromes reported with CH include; Turner's syndrome, Down's syndrome, Noonan syndrome and chromosomal abnormalities such as trisomy. 13, 18, and 21. Isolated CH can also be inherited as an autosomal recessive ...

  13. [Prevention, diagnosis and treatment of iatrogennic lesions of biliary tract during laparoscopic cholecystectomy. Management of papila injury after invasive endoscopy. Part 1. Prevention and diagnosis of bile duct injuries].

    Science.gov (United States)

    Sváb, J; Pesková, M; Krska, Z; Gürlich, R; Kasalický, M

    2005-04-01

    Endoscopic invasive procedures in 70th and 80th years leaded to decrease reoperations on biliary tree. Iatrogenic injury of the biliary tract have increased in incidence in the first decade with the introduction of laparoscopic cholecystectomy. Athough a number of factors have been identified with a high risk of injury ( and number of technical steps have been emphasized to avoid these injury, the incidence of the bile duct injury has reached at least double the rate observed with open cholecystectomy. Cholecystectomy is most frequently performed abdominal operation and the most serious complication associated with this procedure is accidental injury to the common bile duct (0.3-0.4%). This preventable technical error has tradicionally been thought to occur in one or more of three situations: 1. When the operator attempts to clip or ligate a bleeding cystic artery and also clips the common hepatic duct (Fig. 3a). 2. When too much traction has been exerted on the gallbladder so that the common bile duct has tented up into an albow, which was either tied off with ligature or clipped (Fig. 3b). 3. When anatomic anomalies were not recognized and the wrong structure is divided, for example, when the cystic duct winds anterior to the common bile duct and enters on the left side, or when the cystic duct joins the right hepatic duct rather than the junction of the common hepatic and the common bile ducts (Fig. 1, 2, 3cd). In anatomical incertain cases is discussed about cholangiography and cholecystocholangiography during laparoscopy cholecystectomy. Most patients sustained a bile duct injury are recognized in the weeks folloving laparoscopic cholecystectomy. Careful preoperative preparation should include control of sepsis by draining any bile collections or fistulas and komplete cholangiography. Long-term results are best achieved in specialized hepatobiliary centres performing biliary reconstruction with a Roux-Y hepaticojejunostomy. Success rates over 90% have been

  14. The transmission of thermal neutrons along air filled ducts in water

    International Nuclear Information System (INIS)

    Piercey, D.C.

    1962-06-01

    Predictions and measurements of thermal neutron transmission along air filled ducts in water have been made. To aid the analysis, the ducts were shielded in various ways using cadmium sheet. The predictions were, in general, in agreement to better than a factor 4 for ducts up to 200 duct radii in length. (author)

  15. Pregnancy and cystic fibrosis: Approach to contemporary management

    Science.gov (United States)

    Tay, George; Callaway, Leonie; Bell, Scott C

    2014-01-01

    Over the previous 50 years survival of patients with cystic fibrosis has progressively increased. As a result of improvements in health care, increasing numbers of patients with cystic fibrosis are now considering starting families of their own. For the health care professionals who look after these patients, the assessment of the potential risks, and the process of guiding prospective parents through pregnancy and beyond can be both challenging and rewarding. To facilitate appropriate discussions about pregnancy, health care workers must have a detailed understanding of the various important issues that will ultimately need to be considered for any patient with cystic fibrosis considering parenthood. This review will address these issues. In particular, it will outline pregnancy outcomes for mothers with cystic fibrosis, issues that need to be taken into account when planning a pregnancy and the management of pregnancy for mothers with cystic fibrosis or mothers who have undergone organ transplantation as a result of cystic fibrosis. PMID:27512443

  16. Radiation of sound in a semi-infinite hard duct inserted axially into a larger infinite lined duct

    Science.gov (United States)

    Tiwana, Mazhar Hussain; Nawaz, Rab; Mann, Amer Bilal

    2017-12-01

    This article examines sound radiation from a hard semi-infinite duct placed symmetrically inside an acoustically lined duct. We introduce a wake on right handed region of the duct configuration to analyze sound radiation process for the trailing edge situation. The integral transforms together with Wiener-Hopf techniques render the solution of underlying problem. However expressions for field intensity involve infinite sums/products that enable solution using truncation approach. The sound radiation analysis is then observed graphically while using different choice of some pertinent parameters. It is worth mentioning that results of leading edge situation can be recovered as a limiting case.

  17. EFFECT OF NIGELLA SATIVA ON NUMBER OF CYSTIC FOLLICLES IN LETROZOLE INDUCED POLYCYSTIC OVARIES IN MICE

    Directory of Open Access Journals (Sweden)

    Noreen Anwar

    2016-06-01

    Full Text Available Objective: To observe the protective effect of Nigella sativa on number of cystic follicles in Letrozole induced polycystic ovaries in mice. Study Design: Laboratory based randomized control trial. Place and Duration of Study: Department of Anatomy, Army Medical College in collaboration with National Institute of Health from Nov 2014 to Nov 2015. Material and Methods: Forty female BALB/c mice were selected and divided in four groups, each having 10 animals. Group A served as control and was given normal diet. Group B was given Letrozole at a dose of 1milligram/kilogram body weight. Group C was treated with Letrozole for eight weeks at a dose of 1milligram/kilogram body weight and Nigella sativa seeds powder at a dose of 10grams/kilogram body weight once daily starting at 22 day and continued up to eight weeks. Group D was treated with Letrozole for eight weeks at a dose of 1milligram/kilogram body weight and Nigella sativa oil at a dose of 4milliliter/kilogram body weight once daily starting at 22 day and continued up to eight weeks. Animals were dissected a day after last dose. Size, shape, color and consistency of ovary was observed. Right ovary was processed, embedded and stained for histological study. Number of cystic follicles were counted and noted. Results: Significant number of cystic follicles was observed in ovaries of animals of group B as compared to group A. While their number decreased significantly in group C and D as compared to group B. Conclusion: Nigella sativa seeds powder and its oil, both have a similar protective effect on histomorphology of ovary of polycystic ovarian syndrome (PCOS in mice by decreasing the number of cystic follicles.

  18. Radiologic features of preteus syndrome: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Ok Hwa [Dept. of Radiology, Haeundae Paik Hospital, Inje University College of Medicine, Busan (Korea, Republic of)

    2014-04-15

    Proteus syndrome is a rare congenital hamartomatous condition that is characterized by a wide range of malformations with overgrowth of various tissues. The author reports the case of a Proteus syndrome in a 14-year-old girl who had the unique features of this syndrome including megaspondylodysplasia with resultant scoliosis, leg discrepancy, macrodactyly, clinodactyly, hyperostosis in external auditory meatus, asymmetric megalencephaly, splenomegaly, cystic lung changes, asymmetric soft tissue fat infiltrations and a long, asymmetric face, with descriptions of the radiological features.

  19. Method and apparatus for duct sealing using a clog-resistant insertable injector

    Science.gov (United States)

    Wang, Duo; Modera, Mark P.

    2010-12-14

    A method for forming a duct access region through one side of a previously installed air duct, wherein the air duct has an air flow with an air flow direction by inserting an aerosol injector into a previously installed air duct through the access region. The aerosol injector includes a liquid tube having a liquid tube orifice for ejecting a liquid to be atomized; and a propellant cap. The method is accomplished by aligning the aerosol injector with the direction of air flow in the duct; activating an air flow within the duct; and spraying a sealant through the aerosol injector to seal the duct in the direction of the air flow.

  20. Noise generation by ducted combustion systems. [resonant oscillations

    Science.gov (United States)

    Chiu, H. H.; Plett, E. G.; Summerfield, M.

    1973-01-01

    Analysis of the interaction between a zone of nonsteady combustion and its confining duct shows that resonant type oscillations occur with resulting noise intensities far greater than from corresponding unconfined flame zones. The blading action on the flow through the compressor and turbine of an engine generates discrete frequency noise which, it is found, is enhanced by the response of the combustion zone in between. Ducted combustor experiments verify the predicted resonant type noise, the amplitude and frequency of which are strongly influenced by the duct dimensions and end impedance. A convergent exit nozzle results in stronger internal resonant noise oscillations but also generates a higher jet velocity, with the attendant jet noise which exceeds noise from inside the duct at jet Mach numbers near unity.-

  1. Design requirement on KALIMER control rod assembly duct

    Energy Technology Data Exchange (ETDEWEB)

    Hwang, W.; Kang, H. Y.; Nam, C.; Kim, J. O.; Kim, Y. J

    1998-03-01

    This document establishes the design guidelines which are needs for designing the control rod assembly duct of the KALIMER as design requirements. it describes control rod assembly duct of the KALIMER and its requirements that includes functional requirements, performance requirements, interfacing systems, design limits and strength requirements, seismic requirements, structural requirements, environmental requirements, reliability and safety requirements, standard and codes, QA programs, and other requirements. The control rod system consists of three parts, which are drive mechanism, drive-line, and absorber bundle. This report deals with the absorber bundle and its outer duct only because the others are beyond the scope of fuel system design. The guidelines for design requirements intend to be used for an improved design of the control rod assembly duct of the KALIMER. (author). 19 refs.

  2. Lacrimal gland duct stones: misdiagnosed as chalazion in 3 cases.

    Science.gov (United States)

    Kim, Sung Chul; Lee, Kook; Lee, Sang Un

    2014-02-01

    To report 3 cases of lacrimal gland duct stones misdiagnosed as chalazion. Retrospective case series. Three patients with lacrimal gland duct stones misdiagnosed as chalazion at a local clinic between 2010 and 2012. A thorough review of clinical, imaging, and histopathologic manifestations. Clinical manifestations of lacrimal gland duct stones included conjunctival injection, lid swelling, tenderness, and ocular discharge, which are similar to chalazion symptoms. Computed tomography revealed a relatively well-defined, high-density mass near the lacrimal gland. Histopathologic examination of excised material revealed calcified amorphous stones. Intractable chalazion-like lesions at the lateral canthal area near the lacrimal gland should be carefully examined; imaging studies are required to confirm the presence of lacrimal gland duct stones, which require surgical removal. Crown Copyright © 2014. Published by Elsevier Inc. All rights reserved.

  3. Surgical versus endoscopic treatment of bile duct stones

    DEFF Research Database (Denmark)

    Martin, D J; Vernon, D R; Toouli, J

    2006-01-01

    10% to 18% of patients undergoing cholecystectomy for gallstones have common bile duct (CBD) stones. Treatment options for these stones include pre- or post-operative endoscopic retrograde cholangiopancreatography (ERCP) or open or laparoscopic surgery....

  4. Status of the development of hot gas ducts for HTRs

    International Nuclear Information System (INIS)

    Stehle, H.; Klas, E.

    1984-01-01

    In the PNP nuclear process heat system the heat generated in the helium cooled core is transferred to the steam reformer and to the successive steam generator or to the intermediate heat exchanger by the primary helium via suitable hot gas ducts. The heat is carried over to the steam gasifier by the intermediate heat exchanger and a secondary helium loop. In both the primary and the secondary loop, the hot gas ducts are internally insulated by a ceramic fibre insulation to protect the support tube and the pressure housing from the high helium temperatures. A graphite hot gas liner will be used for the coaxial primary duct with an annular gap between support tube and pressure shell for the cold gas counterflow. A metallic hot gas liner will be installed in the secondary duct

  5. Design requirement on KALIMER control rod assembly duct

    International Nuclear Information System (INIS)

    Hwang, W.; Kang, H. Y.; Nam, C.; Kim, J. O.; Kim, Y. J.

    1998-03-01

    This document establishes the design guidelines which are needs for designing the control rod assembly duct of the KALIMER as design requirements. it describes control rod assembly duct of the KALIMER and its requirements that includes functional requirements, performance requirements, interfacing systems, design limits and strength requirements, seismic requirements, structural requirements, environmental requirements, reliability and safety requirements, standard and codes, QA programs, and other requirements. The control rod system consists of three parts, which are drive mechanism, drive-line, and absorber bundle. This report deals with the absorber bundle and its outer duct only because the others are beyond the scope of fuel system design. The guidelines for design requirements intend to be used for an improved design of the control rod assembly duct of the KALIMER. (author). 19 refs

  6. Sound transmission through ducts and aircraft noise prediction, volume 1

    Science.gov (United States)

    Schauer, J. J.; Datko, J. T.; Guyton, R. W.

    1982-01-01

    Aircraft engine acoustical lining impedance models, ray acoustics, hydrodynamic modes, and transient analysis of sound propagation in variable area duct studies were applied to aircraft noise prediction. The effects of several duct lining configurations in a TF33 P5 and a CFM56 engined KC-135B aircraft were predicted. The prediction was based on a model corrected to fit flight noise data and modified by including theoretical duct noise attenuation predictions. The transient solution of variable area ducts permitted the prediction of sound propgation in bullet nose inlets for no low and was moderately successful when a potential flow was included with low Mach numbers. Volume 1 contains the technical report and analysis. Volume 2 contains the user's manuals and listings of the computer codes developed.

  7. Measure Guideline: Buried and/or Encapsulated Ducts

    Energy Technology Data Exchange (ETDEWEB)

    Shapiro, C. [Consortium for Advanced Residential Buildings (CARB), Norwalk, CT (United States); Zoeller, W. [Consortium for Advanced Residential Buildings (CARB), Norwalk, CT (United States); Mantha, P. [Consortium for Advanced Residential Buildings (CARB), Norwalk, CT (United States)

    2013-08-01

    Buried and/or encapsulated ducts (BEDs) are a class of advanced, energy-efficiency strategies intended to address the significant ductwork thermal losses associated with ducts installed in unconditioned attics. BEDs are ducts installed in unconditioned attics that are covered in loose-fill insulation and/or encapsulated in closed cell polyurethane spray foam insulation. This Measure Guideline covers the technical aspects of BEDs as well as the advantages, disadvantages, and risks of BEDs compared to other alternative strategies. This guideline also provides detailed guidance on installation of BEDs strategies in new and existing homes through step-by-step installation procedures. Some of the procedures presented here, however, require specialized equipment or expertise. In addition, some alterations to duct systems may require a specialized license.

  8. Surgical management of Stenson's duct injury using epidural catheter

    African Journals Online (AJOL)

    2012-07-05

    rediffmail.com. Introduction. Deep penetrating wounds over the buccal area are common in maxillofacial injuries. Vital structures like parotid duct, facial nerve branches, and transverse facial artery are vulnerable to injury in ...

  9. A case report of corgenotal cystic adenomatoid malformation

    International Nuclear Information System (INIS)

    Jun, Soon Ae; Cha, Kyung Sub; Chi, Je Geun

    1987-01-01

    Congenital cystic adnomatoid malformation (CCAM) is rare pulmonary cystic disease. CCAM has been detected on prematurity, stillborn and respiratory distress infant or child by chest X-ray film and CT scan. One case of CCAM diagnosed in utero at gestational age 22 weeks is reported with sonographic findings and autopsy findings. Ultrasonographic findings are large cystic lesion in fetal thorax and fetal hydrops without hydramnios. The survival of these infants is very poor despite accurate prenatal diagnosis and maximal postnatal care

  10. A case report of corgenotal cystic adenomatoid malformation

    Energy Technology Data Exchange (ETDEWEB)

    Jun, Soon Ae; Cha, Kyung Sub; Chi, Je Geun [Cha Women' s Hospital, Seoul (Korea, Republic of)

    1987-08-15

    Congenital cystic adnomatoid malformation (CCAM) is rare pulmonary cystic disease. CCAM has been detected on prematurity, stillborn and respiratory distress infant or child by chest X-ray film and CT scan. One case of CCAM diagnosed in utero at gestational age 22 weeks is reported with sonographic findings and autopsy findings. Ultrasonographic findings are large cystic lesion in fetal thorax and fetal hydrops without hydramnios. The survival of these infants is very poor despite accurate prenatal diagnosis and maximal postnatal care.

  11. Laryngeal adenoid cystic carcinoma in an adolescent.

    Science.gov (United States)

    Aydin, Omer; Ustündağ, Emre; Işeri, Mete; Erçin, Cengiz

    2008-01-01

    Malignant tumors of the larynx are extremely rare in children and adolescents. Adenoid cystic carcinoma accounts for less than 1% of all malignant tumors in the larynx. We presented a 16-year-old girl with subglottic adenoid cystic carcinoma, whose symptoms of prolonged and exacerbating dyspnea had been attributed to asthma at another medical center. Indirect flexible and rigid laryngoscopy revealed a smooth mass in the subglottic region occupying 80% of the airway passage. The lesion was also confirmed by both CT and MRI. There was no lymphadenopathy in the neck. The patient was successfully treated by surgery and postoperative radiotherapy. Pathological staging was T4N0M0 according to the AJCC, 2003. During six years of follow-up, there was no evidence for local recurrence or regional and distant metastasis.

  12. Mature cystic Theratome. Presentation of a case

    International Nuclear Information System (INIS)

    Rivera B, Aura Lucia; Carrillo B, Jorge Alberto; Ojeda L, Paulina

    2004-01-01

    The case of a patient of five months of age is presented, to which was diagnosed cystic theratome, initially was assisted in another institution to present consistent square of four days in dry cough and sialorrea. It was managed initially with pneumonia diagnosis and spill paraneumonic. The x-ray of initial thorax demonstrated an opacity committing the two inferior thirds of the left hemithorax, with obliteration of the costofrenic angle and contralateral deviation of the cardio mediastinum, later on another thorax x-ray to the entrance with diagnostic impression of sepsis of lung origin and pneumonia suspicion with spill associate pleural, was practiced closed thoracotomy, obtaining 60 cc of sallow liquid. For the persistence of the opacity basal left in the control x-ray, he was practiced thorax tomography. For the presence of multiple densities and the localization of the lesion it outlines the possibility of cystic theratome

  13. The cystic form of rheumatoid arthritis

    International Nuclear Information System (INIS)

    Dijkstra, P.F.; Gubler, F.M.; Maas, A.

    1988-01-01

    A nonerosive form of rheumatoid arthritis (R.A.) was found in 62 patients out of 660 patients with R.A.. These 62 patients exhibit slowly progressive cystic changes in about the same joints in which usually erosions develop in classic R.A.. The E.S.R. is often low, half of the patients remained seronegative and there are 35 males and 27 females in the group. A smaller group of 15 out of these patients could be followed from a stage wherein the radiographs were normal to a stage of extensive cystic changes, over a period of at least 6 years. An attempt is made to delineate this group within the rheumatoid arthritis disease entity. (orig.) [de

  14. Inspiratory muscle training for cystic fibrosis.

    Science.gov (United States)

    Houston, Brian W; Mills, Nicola; Solis-Moya, Arturo

    2013-11-21

    Cystic fibrosis is the most common life-limiting genetic condition in Caucasians and the life-expectancy of those newly diagnosed is increasing. Inspiratory muscle training may be a way of improving the lung function and quality of life of people with cystic fibrosis. Hence there is a need to establish whether this intervention is beneficial. To determine the effect of inspiratory muscle training on health-related quality of life, pulmonary function and exercise tolerance. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials register comprising of references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings.Date of most recent search: 08 July 2013. Randomised or quasi-randomised clinical controlled trials comparing different inspiratory muscle training regimens with each other or a control in people with cystic fibrosis. Three review authors independently applied the inclusion and exclusion criteria to publications and assessed the quality of the included studies. Fourteen studies were identified. Of these eight studies with 180 participants met the review inclusion criteria. There was wide variation in the quality of the included studies. Data were not published in sufficient detail or with sufficiently similar outcome measures in these studies to perform meta-analyses. We have not found any evidence to suggest that this treatment is either beneficial or not. We would advise that practitioners evaluate on a case-by-case basis whether or not to employ this therapy. We recommend that future studies make more use of health-related quality of life and exercise tolerance measures; and that there is an agreement upon a single standard measure of classifying the clinical status of the participants.

  15. MR imaging of pancreas in cystic fibrosis

    International Nuclear Information System (INIS)

    Murayama, S.; Robinson, A.E.; Mulvihill, D.M.; Stallworth, J.M.; Goyco, P.G.; Beckerman, R.C.; Hines, M.R.

    1990-01-01

    The pancreatic regions of 18 patients with cystic fibrosis were analyzed with a 1.5 Tesla MR unit. Signal intensity of the pancreas was correlated with clinical data and ultrasound. A hyperintense pancreas on T1-weighted image was consistent with fatty replacement of pancreatic insufficiency. A pancreas of normal soft tissue intensity was found in two asymptomatic and one symptomatic patient. A very hypointense pancreas on any pulse sequence was considered to be an intermediate stage of pancreatic degeneration. (orig.)

  16. Impending Airway Compromise due to Cystic Hygroma

    Directory of Open Access Journals (Sweden)

    Itai Shavit

    2011-05-01

    Full Text Available We report on a 3-month-old infant, who arrived in the pediatric emergency department (ED with a cervical cystic hygroma causing an impending compromise of the airway. We recognize that such a lesion can rapidly progress, and the judicious use of imaging in the ED may help to avoid airway compromise and possibly fatal complications. [West J Emerg Med. 2011;12(4:368–369.

  17. Mature cystic teratoma involving adrenal gland.

    Science.gov (United States)

    Bedri, Shahinaz; Erfanian, Kamil; Schwaitzberg, Steven; Tischler, Arthur S

    2002-01-01

    A mature cystic teratoma presented as an adrenal mass in a 57-yr-old woman. The tumor was found to be predominantly paraadrenal but focally interrupted the adrenal cortex so that an intraadrenal origin could not be ruled out. Similar lesions have been reported extremely rarely and should be considered in the differential diagnosis of hormonally silent adrenal tumors. The findings of rimlike calcification and fatty density on computed tomography may be helpful diagnostically.

  18. Flame acceleration and transition to detonation in ducts

    Energy Technology Data Exchange (ETDEWEB)

    Ciccarelli, G. [Queen' s University, 130 Stuart Street, Kingston, Ontario (Canada); Dorofeev, S. [FM Global, 1151 Boston-Providence Turnpike, P.O. Box 910, Norwood, MA 02062 (United States)

    2008-08-15

    This paper reviews the state of knowledge on flame acceleration and deflagration-to-detonation transition (DDT) in smooth ducts and ducts equipped with turbulence-producing obstacles. The objective is to bring to light the basic understanding of the phenomenon and its application to explosion safety. The scope of the review is restricted to homogeneous gas-phase combustion with emphasis placed on experimental investigation. (author)

  19. Molecular basis of potassium channels in pancreatic duct epithelial cells

    DEFF Research Database (Denmark)

    Hayashi, M.; Novak, Ivana

    2013-01-01

    Potassium channels regulate excitability, epithelial ion transport, proliferation, and apoptosis. In pancreatic ducts, K channels hyperpolarize the membrane potential and provide the driving force for anion secretion. This review focuses on the molecular candidates of functional K channels...... other cell types, preferably in epithelia, and, where known, their identification and functions in pancreatic ducts and in adenocarcinoma cells. We conclude by pointing out some outstanding questions and future directions in pancreatic K channel research with respect to the physiology of secretion...

  20. Bile Duct Obstruction Secondary to Chronic Pancreatitis in Seven Dogs

    OpenAIRE

    Cribb, Alastair E.; Burgener, David C.; Reimann, Keith A.

    1988-01-01

    Seven icteric dogs were determined to have bile duct obstruction secondary to chronic pancreatitis. All dogs had histories of intermittent vomiting and diarrhea. Alkaline phosphatase and alanine aminotransferase activities and total bilirubin concentrations were markedly elevated. Diagnosis was based on exploratory laparotomy and histological examination. Each dog had a 3 to 10 cm mass in the body of the pancreas and obstruction of the common bile duct. Three dogs treated with pancreatectomy,...

  1. Compact Buried Ducts in a Hot-Humid Climate House

    Energy Technology Data Exchange (ETDEWEB)

    Mallay, D. [Home Innovation Research Labs, Upper Marlboro, MD (United States)

    2016-01-01

    A system of compact, buried ducts provides a high-performance and cost-effective solution for delivering conditioned air throughout the building. This report outlines research activities that are expected to facilitate adoption of compact buried duct systems by builders. The results of this research would be scalable to many new house designs in most climates and markets, leading to wider industry acceptance and building code and energy program approval.

  2. BETTER DUCT SYSTEMS FOR HOME HEATING AND COOLING.

    Energy Technology Data Exchange (ETDEWEB)

    ANDREWS,J.

    2001-01-01

    This is a series of six guides intended to provide a working knowledge of residential heating and cooling duct systems, an understanding of the major issues concerning efficiency, comfort, health, and safety, and practical tips on installation and repair of duct systems. These guides are intended for use by contractors, system designers, advanced technicians, and other HVAC professionals. The first two guides are also intended to be accessible to the general reader.

  3. Genetic modifiers of nutritional status in cystic fibrosis1234

    OpenAIRE

    Bradley, Gia M; Blackman, Scott M; Watson, Christopher P; Doshi, Vishal K; Cutting, Garry R

    2012-01-01

    Background: Improved nutrition early in life is associated with better pulmonary function for patients with cystic fibrosis (CF). However, nutritional status is poorly correlated with the CFTR genotype.

  4. Bilateral cystic neuroblastoma: imaging features and differential diagnoses

    International Nuclear Information System (INIS)

    Cassady, C.; Winters, W.D.

    1997-01-01

    Neuroblastoma is one of the most common malignant tumors of childhood, with 40 % arising in the adrenal glands. Bilateral adrenal involvement from synchronous development or metastatic spread of the tumor is seen in less than 10 % of children with neuroblastoma [1[. Neuroblastoma rarely presents as a cystic suprarenal mass that is difficult to differentiate from adrenal hemorrhage, extralobar sequestration, or dilated upper-pole renal calyces. To our knowledge, bilateral cystic neuroblastoma has not been previously reported. We present a case of bilateral cystic adrenal neuroblastoma to demonstrate the imaging features of this unusual entity, and to expand the differential diagnosis of bilateral cystic suprarenal masses in an infant. (orig.). With 2 figs

  5. Giant cystic thymoma with haemorrhage and necrosis: an unusual case.

    Science.gov (United States)

    Pai, Radha R; Sahu, Kausalya; Shetty, A B; Goel, Garima; Prasad, H V Krishna

    2009-01-01

    Extreme degree of cystic, haemorrhagic and necrotic changes in a thymoma is rare. A 22-year-old male presented with cough, grade 2 dyspnoea, and occasional chest pain for the past six months. Radiological investigations revealed a large cystic lesion in the anterior mediastinum. A benign cystic tumour was suspected. Surgical resection of the tumour was done. Grossly, the almost entirely cystic and haemorrhagic dumb-bell shaped encapsulated tumour showed a subcapsular residual nodule. Histopathological examination was suggestive diagnosis of benign thymoma (World Health Organization [WHO] Type A, medullary type) associated with the rare features of cells with dendritic processes containing melanin pigment seen singly scattered throughout the tumour.

  6. Giant cystic lymphangioma of the mesentery: varied clinical ...

    African Journals Online (AJOL)

    Giant cystic lymphangioma of the mesentery: varied clinical presentation of 3 cases. Mohamed Rami, Abdelhalim Mahmoudi, Aziz El Madi, Khalid Khattala, Moulay Abderrahmane Afifi, Youssef Bouabdallah ...

  7. Cystic variant of calcifying epithelial odontogenic tumor.

    Science.gov (United States)

    Gopalakrishnan, Rajaram; Simonton, Susan; Rohrer, Michael D; Koutlas, Ioannis G

    2006-12-01

    Calcifying epithelial odontogenic tumor (CEOT) is a benign, locally aggressive odontogenic neoplasm characterized by sheets and nests of epithelial cells with deeply eosinophilic or occasionally clear cytoplasm, calcifications, and eosinophilic amorphous material that stains positive for amyloid. Although many cases of CEOT are associated with impacted teeth and occasionally appear radiographically as dentigerous cysts, a true cystic variant has not been previously reported. We report a 15-year-old white male with a large cystic maxillary lesion that filled most of the left maxillary sinus. It deformed the medial wall, the inferior orbital floor, and caused narrowing of the left inferior meatus. Histologically, the cystic lining showed characteristics of CEOT. An intraluminal component that featured histologic characteristics of CEOT was identified during surgery. The lesion was enucleated and the postsurgical course of the patient was uneventful. Because follow-up has been for less than 1 year, a meaningful long-term prognosis cannot be determined at present. However, the patient has not reported any symptoms or signs of recurrence during the follow-up period.

  8. Congenital cystic lung malformations; Konnatale zystische Lungenfehlbildungen

    Energy Technology Data Exchange (ETDEWEB)

    Stoever, B.; Scheer, I.; Bassir, C. [Klinik fuer Strahlenheilkunde, Berlin (Germany). Abt. Paediatrische Radiologie, Charite; Mau, H. [Campus Virchow-Klinikum, Klinik fuer Kinderchirurgie, Berlin (Germany); Chaoui, R. [Campus Mitte, Klinik fuer Geburtsmedizin, Berlin (Germany); Henrich, W. [Campus Virchow-Klinikum, Klinik fuer Geburtsmedizin, Berlin (Germany); Schwabe, M. [Campus Mitte, Inst. fuer Pathologie, Berlin (Germany); Wauer, R. [Campus Mitte, Klinik fuer Neonatologie, Berlin (Germany)

    2006-04-15

    Purpose: The aim of the study concerning congenital cystic lung malformations was to evaluate prenatal diagnoses postnatally to determine prognostic factors as well as to define optimized perinatal management. Materials and Methods: The study is based on 45 prenatal ultrasound examinations depicting fetal cystic lung lesions. 32 of the mothers had follow-up examinations. 5 pregnancies were terminated due to CCAM and additional malformations. Complete regression of the lesions was seen prenatally in 8 cases and postnatally in 5 children. Results: Surgical intervention due to respiratory insufficiency was necessary in 4 neonates. According to the imaging results, CCAM was present in 4 cases and sequestration in 7 patients. No correlation between the imaging findings and the surgical results was found in 3 children: One child suffered from rhadomyoid dysplasia, and in the case of the second child, a left-sided hernia of the diaphragm and additional sequestration were detected. The third child showed AV malformation. The cystic lesions of the 14 children operated upon were proven histologically. The degree of accuracy in the present study was high. Conclusion: Precise perinatal management is warranted in order to determine according to the clinical relevance surgical intervention and to prevent complications after the first year of life. This is performed during the neonatal period for respiratory insufficient neonates and within the first year of life for clinically stable children. (orig.)

  9. Nephronophthisis and medullary cystic kidney disease complex

    Directory of Open Access Journals (Sweden)

    Stanišić Marijana

    2005-01-01

    Full Text Available Background. Nephronophthisis and medullary cystic kidney disease complex refers to the genetic heterogeneous group of inherited tubulointerstital nephritis. Nephronophthisis comprises at last 3 clinical manifestations, has the autosomal recessive pattern of inheritance, appears early in life and is the most frequent inherited kidney disease that causes terminal renal failure in childhood, while medullary cystic kidney disease has the autosomal dominant pattern of inheritance, is less frequent, and terminal renal failure appears later in life. These two forms have similar clinical and morphological findings but extrarenal manifestations, the median ages of occurrence of terminal renal failure, and siblings presence help us distinguish these diseases. Case report. In this article we illustrated the case of a 20- years old patient with the suspicion of having complex nephornophthisis and medullary cystic kidney disease based upon mild renal failure, seen in routinely taken laboratory findings and bilateral cysts in corticomedullary region of the kidneys verified on abdominal ultrasound examination. Conclusion. This disease should rise suspicion in children or adolescents with progressive renal failure, a typical clinical manifestation, blood and urine samples results, bilateral cysts in the corticomedullary region of the kidneys seen during ultrasound examination of the kidneys and family inheritance.

  10. WAVE PROPAGATION in the HOT DUCT of VHTR

    Energy Technology Data Exchange (ETDEWEB)

    Richard Schultz; Jim C. P. Liou

    2013-07-01

    In VHTR, helium from the reactor vessel is conveyed to a power conversion unit through a hot duct. In a hypothesized Depressurized Conduction Cooldown event where a rupture of the hot duct occurs, pressure waves will be initiated and reverberate in the hot duct. A numerical model is developed to quantify the transients and the helium mass flux through the rupture for such events. The flow path of the helium forms a closed loop but only the hot duct is modeled in this study. The lower plum of the reactor vessel and the steam generator are treated as specified pressure and/or temperature boundary to the hot duct. The model is based on the conservation principles of mass, momentum and energy, and on the equations of state for helium. The numerical solution is based on the method of characteristics with specified time intervals with a predictor and corrector algorithm. The rupture sub-model gives reasonable results. Transients induced by ruptures with break area equaling 20%, 10%, and 5% of the duct cross-sectional area are described.

  11. Amblyopia risk factors in infants with congenital nasolacrimal duct obstruction.

    Science.gov (United States)

    Kim, Jung Wan; Lee, Hwa; Chang, Minwook; Park, Minsoo; Lee, Tae Soo; Baek, Sehyun

    2013-07-01

    This study aims to report the prevalence of amblyopia risk factors in patients with congenital nasolacrimal duct obstructions. We recruited patients who were treated for congenital nasolacrimal duct obstruction from April 2007 to December 2011 at Korea University Ansan Hospital. We evaluated visual acuity and refraction, performed strabismus test and slit-lamp examination, and assessed marginal reflex distance 1 (MRD1) to rule out amblyopia risk factors. We examined 26 children among 115 patients. Ten patients underwent probing procedure and 16 underwent Monoka stent intubation. Nine (35%) patients showed amblyopia risk factors, which occurred in the same eye as the congenital nasolacrimal duct obstruction in 8 (89%) patients. Seven out of 9 patients had refractive error alone, and 2 patients had both refractive error and strabismus. One (11%) had anisometropia, 2 (22%) had hyperopia, and 8 (89%) had astigmatism. Children with congenital nasolacrimal duct obstruction had a higher prevalence of amblyopia risk factors than children in the general population. Children with congenital nasolacrimal duct obstruction require special attention and treatments for refractive error. We recommend early treatment, such as probing or intubation, for congenital nasolacrimal duct obstruction.

  12. Thermal performance test of the hot gas ducts of HENDEL

    International Nuclear Information System (INIS)

    Hishida, M.; Kunitomi, K.; Ioka, I.; Umenishi, K.; Tanaka, T.; Shimomura, H.; Sanokawa, K.

    1984-01-01

    A hot gas duct provided with internal thermal insulation is to be used for high-temperature gas-cooled reactors (HTGR). This type of hot gas duct has not been used so far in industrial facilities, and only a couple of tests on such a large-scale model of a hot gas duct have been conducted. The present report deals with the results of the thermal performance of the single tube type hot gas ducts which are installed as parts of a helium engineering demonstration loop (HENDEL). Uniform temperature and heat flux distribution at the surface of the duct were observed, the experimental correlations being obtained for the effective thermal conductivity of the internal thermal insulation layer. The measured temperature distribution of the pressure tube was in good agreement with the calculation by a TRUMP heat transfer computer code. The temperature distribution of the inner tube of the co-axial hot gas duct was evaluated and no hot spot was detected. These results would be very valuable for the design and development of HTGR. (orig.)

  13. Role of Collecting Duct Renin in the Pathogenesis of Hypertension.

    Science.gov (United States)

    Gonzalez, Alexis A; Lara, Lucienne S; Prieto, Minolfa C

    2017-08-01

    The presence of renin production by the principal cells of the collecting duct has opened new perspectives for the regulation of intrarenal angiotensin II (Ang II). Angiotensinogen (AGT) and angiotensin-converting enzyme (ACE) are present in the tubular fluid coming from the proximal tubule and collecting duct. All the components needed for Ang II formation are present along the nephron, and much is known about the mechanisms regulating renin in juxtaglomerular cells (JG); however, those in the collecting duct remain unclear. Ang II suppresses renin via protein kinase C (PKC) and calcium (Ca 2+ ) in JG cells, but in the principal cells, Ang II increases renin synthesis and release through a pathophysiological mechanism that increases further intratubular Ang II de novo formation to enhance distal Na + reabsorption. Transgenic mice overexpressing renin in the collecting duct demonstrate the role of collecting duct renin in the development of hypertension. The story became even more interesting after the discovery of a specific receptor for renin and prorenin: the prorenin receptor ((P)RR), which enhances renin activity and fully activates prorenin. The interactions between (P)RR and prorenin/renin may further increase intratubular Ang II levels. In addition to Ang II, other mechanisms have been described in the regulation of renin in the collecting duct, including vasopressin (AVP), bradykinin (BK), and prostaglandins. Current active investigations are aimed at elucidating the mechanisms regulating renin in the distal nephron segments and understand its role in the pathogenesis of hypertension.

  14. Measure Guideline: Buried and/or Encapsulated Ducts

    Energy Technology Data Exchange (ETDEWEB)

    Shapiro, C.; Zoeller, W.; Mantha, P.

    2013-08-01

    Buried and/or encapsulated ducts (BEDs) are a class of advanced, energy-efficiency strategies intended to address the significant ductwork thermal losses associated with ducts installed in unconditioned attics. BEDs are ducts installed in unconditioned attics that are covered in loose-fill insulation and/or encapsulated in closed cell polyurethane spray foam insulation. This Measure Guideline covers the technical aspects of BEDs as well as the advantages, disadvantages, and risks of BEDs compared to other alternative strategies. This guideline also provides detailed guidance on installation of BEDs strategies in new and existing homes through step-by-step installation procedures. This Building America Measure Guideline synthesizes previously published research on BEDs and provides practical information to builders, contractors, homeowners, policy analysts, building professions, and building scientists. Some of the procedures presented here, however, require specialized equipment or expertise. In addition, some alterations to duct systems may require a specialized license. Persons implementing duct system improvements should not go beyond their expertise or qualifications. This guideline provides valuable information for a building industry that has struggled to address ductwork thermal losses in new and existing homes. As building codes strengthen requirements for duct air sealing and insulation, flexibility is needed to address energy efficiency goals. While ductwork in conditioned spaces has been promoted as the panacea for addressing ductwork thermal losses, BEDs installations approach - and sometimes exceed - the performance of ductwork in conditioned spaces.

  15. Parotid duct laceration repair in two horses : case report

    Directory of Open Access Journals (Sweden)

    A. Olivier

    1998-07-01

    Full Text Available Repair of parotid duct lacerations in 2 horses is described using intraluminal silastic tubing as a stent. The duct was lacerated traumatically at the facial vessel notch (incisura vasorum facialium in the 1st horse, and iatrogenically after removal of an intraluminal sialolith after development of infection within the duct in the 2nd horse. In both cases, a silastic tube was passed retrograde into the duct via the salivary papilla, past the wound until the end lay rostroventral to the parotid salivary gland. The severed salivary ducts and the wounds were sutured. The external portion of the silastic tube was sutured to the skin and the tube left in place. Recovery in the 1st case was uneventful. In the 2nd case a salivary duct/cutaneous fistula formed at a wound distant from the sutured wound, which healed spontaneously. This technique differs from a similar described technique in that the stent tube exits the oral cavity and is attached to the outer skin surface.

  16. [Physiopathology and treatment of nephrotic syndrome complications].

    Science.gov (United States)

    Ducloux, Didier

    2011-02-01

    The nephrotic syndrome is defined by a urinary protein excretion exceeding 3g per day, associated with hypoalbuminaemia (syndrome are multiple, essentially dominated by sodium retention and oedema formation. The oedema physiopathology is related to both increased capillary permeability and primary activation of the Na/K pump in the collect duct. Other complications of the nephrotic syndrome include thromboembolic complications, dyslipidaemia, and infections. The treatment of these complications represents an important part of the general management of the nephritic syndrome. Copyright © 2010 Association Société de néphrologie. Published by Elsevier SAS. All rights reserved.

  17. Measure Guideline: Summary of Interior Ducts in New Construction, Including an Efficient, Affordable Method to Install Fur-Down Interior Ducts

    Energy Technology Data Exchange (ETDEWEB)

    Beal, D. [BA-PIRC, Cocoa, FL (United States); McIlvaine, J. [BA-PIRC, Cocoa, FL (United States); Fonorow, K. [BA-PIRC, Cocoa, FL (United States); Martin, E. [BA-PIRC, Cocoa, FL (United States)

    2011-11-01

    This document illustrates guidelines for the efficient installation of interior duct systems in new housing, including the fur-up chase method, the fur-down chase method, and interior ducts positioned in sealed attics or sealed crawl spaces.

  18. Transarterial Chemoembolization for Hepatocellular Carcinomas with Central Bile Duct Invasion: Safety, Prognosis, and Predictive Factors

    Energy Technology Data Exchange (ETDEWEB)

    Choi, Jin Woo; Chung, Jin Wook, E-mail: chungjw@snu.ac.kr [Seoul National University Hospital, Department of Radiology (Korea, Republic of); Cho, Yun Ku [VHS Medical Center, Department of Radiology (Korea, Republic of); Kim, Yoon Jun; Yoon, Jung-Hwan [Seoul National University Hospital, Department of Internal Medicine (Korea, Republic of); Kim, Hyo-Cheol; Jae, Hwan Jun [Seoul National University Hospital, Department of Radiology (Korea, Republic of)

    2015-08-15

    PurposeTo assess the safety and effectiveness of transarterial chemoembolization (TACE) of patients who have hepatocellular carcinomas (HCCs) with central bile duct invasion.Materials and MethodsThe institutional review board approved this retrospective study and waived informed consent. Fifty-three patients, initially treated with TACE for HCCs with central bile duct invasion from January 1999 to September 2012, were included. Clinical, laboratory, and survival data were reviewed. Complications and hospitalization length were evaluated using the χ{sup 2} test, Fisher’s exact test, and logistic regression analysis. Survival was analyzed using the Kaplan–Meier method with log-rank test and Cox proportional hazard model.ResultsSeven patients experienced TACE-related major complications (severe post-embolization syndrome in 3, non-fatal sepsis in 3, and secondary bacterial peritonitis in 1). The overall major complication rate was 13.2 %, but there were no permanent adverse sequelae or deaths within 30 days. Serum total bilirubin ≥3.0 mg/dL was the only significant risk factor for long hospitalization [hazard ratio (HR) = 4.341, p = .022]. The median survival was 12.2 months. Extrahepatic metastasis (HR = 6.145, p < .001), international normalized ratio (PT-INR) ≥1.20 (HR = 4.564, p < .001), vascular invasion (HR = 3.484, p = .001), and intermediate tumor enhancement (HR = 2.417, p = .019) were significantly associated with shorter survival.ConclusionTACE can be a safe and effective treatment for patients who have HCCs with central bile duct invasion. In particular, long-term survival can be expected if patients have strongly enhancing tumors without poor prognostic factors such as extrahepatic metastasis, PT-INR prolongation, and vascular invasion.

  19. Oral calorie supplements for cystic fibrosis.

    Science.gov (United States)

    Smyth, Rosalind L; Rayner, Oli

    2017-05-04

    Poor nutrition occurs frequently in people with cystic fibrosis and is associated with other adverse outcomes. Oral calorie supplements are used to increase total daily calorie intake and improve weight gain. However, they are expensive and there are concerns they may reduce the amount of food eaten and not improve overall energy intake. This is an update of a previously published review. To establish whether in people with cystic fibrosis, oral calorie supplements: increase daily calorie intake; and improve overall nutritional intake, nutritional indices, lung function, survival and quality of life. To assess adverse effects associated with using these supplements. We searched the Cochrane Cystic Fibrosis Trials Register comprising references from comprehensive electronic database searches, handsearches of relevant journals and abstract books of conference proceedings. We contacted companies marketing oral calorie supplements.Last search: 18 October 2016. Randomised or quasi-randomised controlled trials comparing use of oral calorie supplements for at least one month to increase calorie intake with no specific intervention or additional nutritional advice in people with cystic fibrosis. We independently selected the included trials, assessed risk of bias and extracted data. We contacted the authors of included trials and obtained additional information for two trials. We identified 21 trials and included three, reporting results from 131 participants lasting between three months and one year. Two trials compared supplements to additional nutritional advice and one to no intervention. Two of the included trials recruited only children. In one trial the risk of bias was low across all domains, in a second trial the risk of bias was largely unclear and in the third mainly low. Blinding of participants was unclear in two of the trials. Also, in one trial the clinical condition of groups appeared to be unevenly balanced at baseline and in another trial there were

  20. Diagnosis of common bile duct stones by MR imaging. Mainly MR cholangiopancreatography

    Energy Technology Data Exchange (ETDEWEB)

    Isomoto, Ichiro; Koshiishi, Takeshi [Saga National Hospital (Japan); Fukuda, Toshio; Hayashi, Kuniaki

    1998-09-01

    We evaluated the accuracy of MR imaging (MRI), mainly MR cholangiopancreatography (MRCP), in the diagnosis of choledocholithiasis in comparison with ultrasound (US), computed tomography (CT), direct cholangiography, and intravenous cholangiography (DIC). Thirty-seven patients with biliary disease diagnosed by surgery and direct cholangiography underwent MRI with T1-weighted images (T1-WI), T2-weighted images (T2-WI), and MRCP (source images and projection images). The rate of stone detection was evaluated for each MRI image and modality. Fifteen of 37 patients were found to have common bile duct stones at surgery. The depiction rate of T1-WI, T2-WI, source images, and projection images were 7%, 67%, 93%, and 53%, respectively. The depiction rate of MR, US, CT, direct cholangiography, and DIC were 100% (15/15), 25% (3/12), 64% (9/14), 71% (5/7), and 57% (4/7), respectively. In addition, there were two false-positive cases, one of duodenal diverticulum (Lemmel`s syndrome) and the other of hemobilia. MRI had a sensitivity of 100%, specificity of 91%, and accuracy of 94% in the diagnosis of common bile duct stones. In conclusion, MRI is useful for evaluating suspected choledocholithiasis. In spite of the high depiction rate of the source images, other images should also be taken into consideration in the diagnosis of choledocholithiasis. (author)