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Sample records for acquired omental cystic

  1. Omental Lymphangioma in Adults—Rare Presentation Report of a Case

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    T. Narayana Rao

    2012-01-01

    Full Text Available Lymphangioma is an uncommon benign lesion that usually occurs during childhood. Its occurrence in adults is rare. Its presentation in the abdomen is even rare. This case report describes a case of omental lymphangioma presented as retroperitoneal lump. Subsequent imaging, operative, and histological findings revealed omental lymphangioma. Laparotomy done under general anesthesia, a 10 × 12 cm cystic swelling arising from omentum, identified complete excision of the cyst done and send the specimen for histopathological examination. Biopsy report came as omental lymphangioma. Complete surgical excision is the treatment of choice. Prognosis is excellent and recurrence rate is very low if resection is complete. During two years of followup no recurrence was detected. Omental lymphangioma is very rare presentation among abdominal lymphangiomas specifically in adults. Complete excision is the treatment of choice. Long-term followup is required to detect recurrence.

  2. [Ultrasonographic study on kidneys in patients with chronic renal failure. Part II. Acquired cystic disease of the kidneys].

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    Yamaguchi, S; Fujii, H; Kaneko, S; Yachiku, S; Anzai, T; Inada, F; Kobayashi, T; Furuta, K; Ishida, H

    1990-08-01

    Ultrasonic examination of the kidney was performed on 280 patients undergoing chronic dialysis. Acquired cystic disease of the kidney (ACDK) was detected in 107 of 529 kidneys (20.2%). This paper presents an analysis of ultrasonotomograms of ACDK. Ultrasonic measurement of the size of ACDK was 72.5 +/- 15.2 mm in length and 41.7 +/- 9.8 mm in thickness. The size of ACDK was significantly greater than that of contracted kidneys by ultrasonographic diagnosis. With regard to sex distinction the length and thickness of ACDK were significantly greater in males than in females. As for laboratory data, patients with ACDK showed significantly higher values of red blood cell count, hematocrit and serum creatinine concentration compared with contracted kidneys. Prolongation of the dialysis peirod increased the incidence of ACDK. The size of ACDK showed a tendency to increase with duration of dialysis. However, no correlation was noted statistically between the incidence of ACDK and duration of dialysis and between the size of ACDK and duration of dialysis. There was a significantly lower incidence of ACDK in patients with diabetic nephropathy than those with chronic glomerulonephritis. A sonographic feature of ACDK is irregularity of the renal contour because of cystic transformation. Renal imaging, identification of the corticomedullary border, identification of the central echoes and increased parenchymal echogenicity were similar to other dialyzed kidneys. The main complications of ACDK are hemorrhage and tumor formation. We observed two retroperitoneal hematomas and one renal cell carcinoma developed within two years after this examination. The incidence of complications of ACDK was 5.1 per cent. We believe that patients with ACDK should be watched carefully by regular ultrasonic examination for early diagnosis and treatment of these complications. PMID:2232409

  3. Mesenteric and Omental Primary Cysts: Image Findings; Quistes primarios mesentericos y omentales: hallazgos e imagen

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    Castillo Caro, S.; Vargas Serrano, B. [Hospital Universitario Virgen del Rocio. Sevilla (Spain); Rodriguez, M. L. [Hospital Morales Meseguer. Murcia (Spain)

    2004-07-01

    To describe the radiological appearance of different histological subtypes of mesenteric and omental cysts: lymph angioma, enteric duplication cysts, enteric and mesothelial, and non-pancreatic pseudocysts. The basis of this study is the clinical, radiological and pathological review os 13 cases from our departmental archives involving cystic lesions of the omentum or mesentery: 3 lympangiomas, one enteric duplication cyst, 7 mesothelial cysts and 2 non-pancreatic pseudocysts. The sex distribution was 9 women and 4 men. Most common clinical findings were pain and abdominal distension. Echography (ultra-sound scanning) was performed in 8 cases, abdominal x-ray in 10 and computerized tomography (CT in 13 cases. In the ultra-sound and CT studies, lymphangiomas appeared as large thin-walled multilocular cystic lesions. Mesonthelial cysts were observed as unilocular thin-walled (calcified in 2 cases) cystic lesions of varying size. Duplication cysts were unilocular, seemingly liquid-filled under ultrasound scan and with a thick calcified wall. the non-pancreatic pseudocysts were of varying size, heterogeneous and thick-walled. Aside from being able to determine whether abdominal masses are cystic in nature, ultrasound scan and CT can determine whether such are mesenteric or omental in origin. They can also determine the cystic nature of an abdominal mass which would be helpful in narrowing down diagnostic possibilities. It is important to remember that some malignant neoplasia can occasionally resemble cystic lesions with imaging techniques. (Author) 7 refs.

  4. Postoperative omental infarction following colonic resection

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    Kerr, S.F., E-mail: skerr44@doctors.org.uk [Department of Radiology, Leeds General Infirmary, Leeds (United Kingdom); Hyland, R.; Rowbotham, E.; Chalmers, A.G. [Department of Radiology, Leeds General Infirmary, Leeds (United Kingdom)

    2012-02-15

    Aim: To illustrate the computed tomography (CT) appearances and natural history of postoperative omental infarction following colonic resection and to highlight the important clinical implications of this radiological diagnosis. Materials and methods: Over a 3 year period, 15 patients with a history of colonic resection were identified as having a CT diagnosis of postoperative omental infarction. Relevant clinical and pathological data were retrospectively collected from the institution's electronic patient records system and all relevant imaging was reviewed, including serial CT images in 10 patients. Results: A diagnosis of postoperative omental infarction was made in symptomatic and asymptomatic patients who had undergone open or laparoscopic colonic resection for benign or malignant disease. CT appearances ranged from diffuse omental stranding to discrete masses, which typically appeared within weeks of surgery and could persist for years. In four (36%) of the patients with colorectal cancer, the CT appearances raised concern for recurrent malignancy, but percutaneous biopsy and/or serial CT allowed a confident diagnosis of omental infarction to be made. Although most cases were self-limiting, three (20%) cases were complicated by secondary infection and required radiological or surgical intervention. Conclusion: Postoperative omental infarction is an under-recognized complication of colonic resection. It has the potential to mimic recurrent malignancy and may require radiological or surgical intervention for secondary infection.

  5. Postoperative omental infarction following colonic resection

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    Aim: To illustrate the computed tomography (CT) appearances and natural history of postoperative omental infarction following colonic resection and to highlight the important clinical implications of this radiological diagnosis. Materials and methods: Over a 3 year period, 15 patients with a history of colonic resection were identified as having a CT diagnosis of postoperative omental infarction. Relevant clinical and pathological data were retrospectively collected from the institution’s electronic patient records system and all relevant imaging was reviewed, including serial CT images in 10 patients. Results: A diagnosis of postoperative omental infarction was made in symptomatic and asymptomatic patients who had undergone open or laparoscopic colonic resection for benign or malignant disease. CT appearances ranged from diffuse omental stranding to discrete masses, which typically appeared within weeks of surgery and could persist for years. In four (36%) of the patients with colorectal cancer, the CT appearances raised concern for recurrent malignancy, but percutaneous biopsy and/or serial CT allowed a confident diagnosis of omental infarction to be made. Although most cases were self-limiting, three (20%) cases were complicated by secondary infection and required radiological or surgical intervention. Conclusion: Postoperative omental infarction is an under-recognized complication of colonic resection. It has the potential to mimic recurrent malignancy and may require radiological or surgical intervention for secondary infection.

  6. Acquired Cystic Kidney Disease

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    ... including diabetes, high blood pressure, glomerulonephritis, and cys tic kidney diseases. Participants in clinical trials can play ... Life Options Rehabilitation Resource Center c/o Medical Education Institute, Inc. 414 D’Onofrio Drive, Suite 200 ...

  7. Giant omental lipoblastoma and CD56 expression

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    Go Miyano

    2013-01-01

    Full Text Available We report a case of giant omental lipoblastoma in a 13-month-old boy, which was treated successfully by total excision. Tumor cells were positive for S100, CD34 and CD56. This is the first report of lipoblastoma expressing CD56, a fact that could be used to differentiate lipoblastoma from liposarcoma.

  8. Spontaneous retroperitoneal hemorrhage presenting as hemoperitoneum secondary to renal cyst rupture in a peritoneal dialysis patient with acquired cystic kidney disease.

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    Biyik, Z; Solak, Y; Gaipov, A; Ozbek, O; Esen, H; Turk, S

    2015-01-01

    Spontaneous retroperitoneal hemorrhage (SRH) is a rare and potentially fatal condition. Acquired cystic kidney disease (ACKD) may cause SRH in hemodialysis patients. However, presentation of retroperitoneal hematoma as hemoperitoneum in peritoneal dialysis (PD) patients is exceedingly rare. We report a 44-year-old male PD patient who presented with hemoperitoneum secondary to retroperitoneal hematoma. The reason of SRH was rupture of the cysts of ACKD. The patient underwent unilateral nephrectomy with subsequent disappearance of hemoperitoneum. The importance of this case lies in the fact that the patients who have been receiving dialysis for a long time should be under surveillance in terms of ACKD development and potential associated complications such as cyst hemorrhage and malignancy.

  9. Primary omental Gastrointestinal stromal tumor (GIST

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    Hirahara Nobutsune

    2007-06-01

    Full Text Available Abstract Background We report herein a rare case of primary omental gastrointestinal stromal tumor (GIST. Case presentation A 65 year-old man was referred to our hospital with a huge abdominal mass occupying the entire left upper abdomen as shown by sonography. On computed tomography (CT, this appeared as a heterogeneous low-density mass with faint enhancement. Abdominal angiography revealed that the right gastroepiploic artery supplied the tumor. With such an indication of gastric GIST, liposarcoma, leiomyosarcoma or mesothelioma laparotomy was performed and revealed that this large mass measured 20 × 17 × 6 cm, arising from the greater omentum. It was completely resected. Histopathologically, it was composed of proliferating spindle and epithelioid cells with an interlacing bundle pattern. Immunohistochemically, the tumor was positive for myeloid stem cell antigen (CD34, weakly positive for c-KIT (CD117 and slightly positive for neuron-specific enolase (NSE, but negative for cytokeratin (CK, alpha-smooth muscle actin (SMA and S-100 protein. A mutation was identified in the platelet-derived growth factor alpha (PDGFRA juxtamembrane domain (exon 12, codon561 and the tumor was diagnosed as an omental GIST. The postoperative course was uneventful. The patient is treated by Glevec® and is alive well with no sign of relapse. Conclusion Our case demonstrated a weak immunohistochemical expression of c-kit (CD117 and a point mutation in PDGFRA exon 12 resulting in an Asp for Val561 substitution. Imatinib therapy as an adjuvant to complete resection has been carried out safely. Because of the rarity of primary omental GISTs, it is inevitable to analyze accumulating data from case reports for a better and more detailed understanding of primary omental GISTs.

  10. Calcifying fibrous tumour: an unusual omental lesion

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    Sudhakar, Sniya; Gibikote, Sridhar [Christian Medical College Hospital, Department of Radiology, Vellore, Tamil Nadu (India); Mistry, Yogesh [Christian Medical College Hospital, Department of Pathology, Vellore, Tamil Nadu (India); Dastidar, Arindam; Sen, Sudipta [Christian Medical College Hospital, Department of Pediatric Surgery, Vellore, Tamil Nadu (India)

    2008-11-15

    Calcifying fibrous tumour (CFT) is a recently described distinct clinicopathological entity characterized by calcifying lesions usually occurring in soft tissue of the extremities, trunk, axilla, pleura, mediastinum and peritoneum of children and adults. Most reported cases involving the peritoneum have been in adults. We present the imaging, surgical and pathology findings of CFT in a 7-year-old child who presented with an incidental finding of a large omental mass. (orig.)

  11. Calcifying fibrous tumour: an unusual omental lesion

    International Nuclear Information System (INIS)

    Calcifying fibrous tumour (CFT) is a recently described distinct clinicopathological entity characterized by calcifying lesions usually occurring in soft tissue of the extremities, trunk, axilla, pleura, mediastinum and peritoneum of children and adults. Most reported cases involving the peritoneum have been in adults. We present the imaging, surgical and pathology findings of CFT in a 7-year-old child who presented with an incidental finding of a large omental mass. (orig.)

  12. Ultrasound-guided omental biopsy: Review of 173 patients

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    Govindarajan Padmapriya; Keshava Shyamkumar

    2010-01-01

    Background: Omental biopsy has conventionally been performed using a surgical approach. USG-guided omental biopsy is a safe and effective alternative. The purpose of this study was to assess the utility of USG guidance for biopsy of the greater omentum. Study design: Retrospective study. Materials and Methods: We retrospectively reviewed all omental biopsies performed under USG guidance from April 2006 to March 2010 in a tertiary care hospital. Results: One hundred and seventy-three patients ...

  13. Cystic Lymphangioma as a Cause of Massive Abdominal Hemorrhage

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    E. Consuegra Llapur

    2011-01-01

    Full Text Available We report the case of two-year-old girl with hypovolemic shock caused by bleeding from an abdominal cystic lymphangioma. The whole blood was contained within a large omental bag that could be completely removed. There were no associated anomalies. The child progressed satisfactorily.

  14. Two Cases of Omental Torsion Mimicking Acute Appendicitis

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    Feeroz Alam Khan; Naeem Liaqat; Sajid Hameed Dar; Asif Iqbal Sandhu; Sajid Nayyer

    2014-01-01

    Acute appendicitis is often simulated by other entities like mesenteric adenitis, worm infestation, Meckel’s diverticulitis, urinary tract infection and rarely omental torsion. We report two cases, a 6 year old boy and an 11 year old girl, who presented with symptoms and signs of acute appendicitis but upon exploration turned out to be omental torsion.

  15. Two Cases of Omental Torsion Mimicking Acute Appendicitis

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    Feeroz Alam Khan

    2014-02-01

    Full Text Available Acute appendicitis is often simulated by other entities like mesenteric adenitis, worm infestation, Meckel’s diverticulitis, urinary tract infection and rarely omental torsion. We report two cases, a 6 year old boy and an 11 year old girl, who presented with symptoms and signs of acute appendicitis but upon exploration turned out to be omental torsion.

  16. Two Cases of Omental Torsion Mimicking Acute Appendicitis

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    Liaqat, Naeem; Dar, Sajid Hameed; Sandhu, Asif Iqbal; Nayyer, Sajid

    2014-01-01

    Acute appendicitis is often simulated by other entities like mesenteric adenitis, worm infestation, Meckel’s diverticulitis, urinary tract infection and rarely omental torsion. We report two cases, a 6 year old boy and an 11 year old girl, who presented with symptoms and signs of acute appendicitis but upon exploration turned out to be omental torsion. PMID:24834389

  17. PRIMARY OMENTAL HYDATID CYST – A RARE ENTITY

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    Prashant M

    2015-08-01

    Full Text Available Hydatid cyst is caused by the parasite Echinococcus granulosus ( L arval form in humans with lesions most frequently encountered in the liver and lungs. It can rarely involve extra - hepatic organs. Primary omental hydatid cyst is rare entity. This report presents the interesting case of a very large primary omental hydatid cyst.

  18. Omental infarction associated with right-sided heart failure

    International Nuclear Information System (INIS)

    A 31-year-old man with a known congenital heart disease presented with cardial decompensation and an acute abdomen with tenderness in the right inferior abdominal quadrant. Because infectious parameters were slightly elevated, acute appendicitis was suspected. A CT scan showed an isolated focal infiltration of the omentum, superficial to the ascending colon, small amounts of ascites, and dilated hepatic and mesenteric veins. Laparoscopic resection and histopathologic examination confirmed hemorrhagic omental infarction due to thromboses of several small omental veins. This is a report on the pathogenesis, differential diagnoses, and CT findings of omental infarction. (orig.)

  19. Omental infarction associated with right-sided heart failure

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    Wiesner, W.; Bongartz, G. [Institute of Diagnostic Radiology, University Hospital Basel, Petersgraben 4, CH-4031 Basel (Switzerland); Kaplan, V. [Department of Internal Medicine, University Hospital Zuerich, Raemistrasse 100, CH-8091 Zuerich (Switzerland)

    2000-07-01

    A 31-year-old man with a known congenital heart disease presented with cardial decompensation and an acute abdomen with tenderness in the right inferior abdominal quadrant. Because infectious parameters were slightly elevated, acute appendicitis was suspected. A CT scan showed an isolated focal infiltration of the omentum, superficial to the ascending colon, small amounts of ascites, and dilated hepatic and mesenteric veins. Laparoscopic resection and histopathologic examination confirmed hemorrhagic omental infarction due to thromboses of several small omental veins. This is a report on the pathogenesis, differential diagnoses, and CT findings of omental infarction. (orig.)

  20. Idiopathic omental infarction, diagnosed and managed laparoscopically: a case report.

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    Abdulaziz, Ahmed; El Zalabany, Tamer; Al Sayed, Abdul Rahim; Al Ansari, Ahmed

    2013-01-01

    Idiopathic omental infarction is a rare cause of acute abdomen in adults, and the clinical finding can mimic acute appendicitis. Although idiopathic omental infarction is uncommon, the incidence of its detection has become more frequent as a result of advances in radiological technologies. We reported on a 21-year-old man who presented with sudden onset of intermittent right lower quadrant abdominal pain for seven days. The pain became more localized at the right iliac fossa (RIF) at day 2 before admission. A physical examination revealed a fever (38.2°C), severe RIF tenderness, mass-like fullness, and positive rebound tenderness. A CT of the abdomen showed inflammatory changes and increased fat density mass in the right upper quadrant measuring 5 × 4 cm representing focal panniculitis. However, the appendix was visualized normally and the findings were not in favor of acute appendicitis. Diagnosis was carried on laparoscopically. Serosanguinous free fluid was found in all abdominal quadrants. A 6 × 4 cm gangrenous omental mass was noted. The omental mass was excised and an appendectomy was performed. In summary, omental infarction should be considered as a deferential diagnosis for acute right-sided abdominal pain, especially if the clinical finding does not correspond to appendicitis.

  1. Idiopathic Omental Infarction, Diagnosed and Managed Laparoscopically: A Case Report

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    Ahmed AbdulAziz

    2013-01-01

    Full Text Available Idiopathic omental infarction is a rare cause of acute abdomen in adults, and the clinical finding can mimic acute appendicitis. Although idiopathic omental infarction is uncommon, the incidence of its detection has become more frequent as a result of advances in radiological technologies. We reported on a 21-year-old man who presented with sudden onset of intermittent right lower quadrant abdominal pain for seven days. The pain became more localized at the right iliac fossa (RIF at day 2 before admission. A physical examination revealed a fever (38.2∘C, severe RIF tenderness, mass-like fullness, and positive rebound tenderness. A CT of the abdomen showed inflammatory changes and increased fat density mass in the right upper quadrant measuring 5×4 cm representing focal panniculitis. However, the appendix was visualized normally and the findings were not in favor of acute appendicitis. Diagnosis was carried on laparoscopically. Serosanguinous free fluid was found in all abdominal quadrants. A 6×4 cm gangrenous omental mass was noted. The omental mass was excised and an appendectomy was performed. In summary, omental infarction should be considered as a deferential diagnosis for acute right-sided abdominal pain, especially if the clinical finding does not correspond to appendicitis.

  2. Omental and Retroperitoneal Hydatid Cyst: A Case Report

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    "M. Sirus

    2006-07-01

    Full Text Available Hydatid disease is one of the commonest parasitic infections of the liver, rupture of which into the peritoneal cavity leads to secondary echinococcosis. Seventy percent of hydatid disease cases occur in the liver, although any organ may be involved. A case of omental and retroperitoneal hydatid disease along with the hydatid cyst of the liver is present.

  3. Gravidade da doença renal cística adquirida determina melhora da anemia da insuficiência renal crônica Improvement on anemia in acquired cystic disease of the end-stage kidney

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    H.M. Bezerra

    1997-03-01

    Full Text Available OBJETIVO. Identificar correlação entre duração do tratamento dialítico, gravidade da doença renal cística adquirida (DRCA medida pelo tamanho dos cistos e seu efeito sobre a correção espontânea da anemia. MATERIAL E MÉTODOS. Foram selecionados dez pacientes, seis do sexo masculino e quatro do feminino, com mais de cinco anos em tratamento dialítico. Nenhum paciente selecionado tinha doença renal policística bilateral como doença primária. A avaliação renal foi feita com ultra-sonografia. DRCA foi caracterizada pela presença de quatro ou mais cistos em cada rim. Os maiores cistos foram medidos para efeito de correlação. O diagnóstico da anemia foi estabelecido pelo valor do hematócrito e da hemoglobina séricos. Foram analisados, também, uréia, creatinina, albumina, ferro, capacidade total de combinação do ferro e o percentual de saturação da transferrina séricos. RESULTADOS. Os pacientes estavam adequadamente dialisados (uréia e creatinina séricas = 98,7 ± 35mg/dL e 9,7 ± 2,7mg/dL, respectivamente, com bom estado nutricional (albumina sérica = 4,5 ± 0,5g/dL, e tinham razoável reserva de ferro (ferro sérico = 80 ± 34mg/dL. A prevalência de DRCA foi de 80%. Não detectamos nenhum sinal ultra-sonográfico de malignidade nesses cistos. Houve correlação significante entre tempo de diálise e hematócrito (R = 0,70; p OBJECTIVE - To identify a correlation between dialysis treatment duration and severity of acquired cystic disease of the end-stage kidney measured by cyst sizes; and assess its effect on spontaneous improvement of anemia. MATERIALS AND METHODS - Ten patients, 6 males and 4 females were selected, who have been on hemodialytic treatment for more than 5 years. There was no patient selected with autosomal dominant polycystic kidney disease. The renal evaluation has been made through ultrasonographic studies. Acquired cystic disease of the end-stage kidney was characterized by finding four or more

  4. Omental torsion in a captive polar bear (Ursus maritimus).

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    Mendez-Angulo, Jose L; Funes, Francisco J; Trent, Ava M; Willette, Michelle; Woodhouse, Kerry; Renier, Anna C

    2014-03-01

    This is the first case report of an omental torsion in a polar bear (Ursus maritimus). A captive, 23-yr-old, 250-kg, intact female polar bear presented to the University of Minnesota Veterinary Medical Center with a 2-day history of lethargy, depression, and vomiting. Abdominal ultrasound identified large amounts of hyperechoic free peritoneal fluid. Ultrasound-guided abdominocentesis was performed and yielded thick serosanguinous fluid compatible with a hemoabdomen. An exploratory laparotomy revealed a large amount of malodorous, serosanguineous fluid and multiple necrotic blood clots associated with a torsion of the greater omentum and rupture of a branch of the omental artery. A partial omentectomy was performed to remove the necrotic tissue and the abdomen was copiously lavaged. The polar bear recovered successfully and is reported to be clinically well 6 mo later. This condition should be considered as a differential in bears with clinical signs of intestinal obstruction and hemoabdomen. PMID:24712179

  5. Use of omental pedicles in mycotic abdominal aortic aneurysm repair

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    Alibhai, M.K.; Samee, A; Ahmed, M.; Duffield, R.

    2011-01-01

    We report a case of a sixty year old man with a mycotic infra-renal abdominal aortic aneurysm complicated by a left psoas abscess. After treatment with parenteral antibiotics he underwent early aortic reconstruction with an in-situ prosthetic graft wrapped in an omental pedicle. Mycotic abdominal aortic aneurysms can be treated in this way despite the potential for graft infection from persisting retroperitoneal sepsis.

  6. UNILOCULAR OMENTAL CYST IN ADULT FEMALE PRESENTING AS HUGE ABDOMINAL LUMP: A RARE CASE REPORT

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    Himansu

    2015-05-01

    Full Text Available Omental cysts are rare entity with a prevalence of 1: 1,000,000 in adults and in 1: 20, 000 in children. We are reporting a case of a 30 year female patient with abdominal lump over epigastrium and left hypochondrium for 6 months; diagnosed on laparotomy as uniloculated omental cyst originating from lesser omentum. Omental cyst is a challenging diagnostic entity with varied presentations and a wide range of differential diagnosis has to be kept in mind.

  7. Omental infarction in the postpartum period: a case report and a review of the literature

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    Izbicki Jakob R

    2010-11-01

    Full Text Available Abstract Introduction Omental infarction is a rare and often misdiagnosed clinical event with unspecific symptoms. It affects predominantly young and middle aged women. Case presentation This is a case report of a 26-year-old Caucasian woman with spontaneous omental infarction two weeks after normal vaginal delivery. Conclusion Omental infarction is a differential diagnosis in the postpartum acute abdomen. As some cases of omental infarction, which are caused by torsion, can be adequately diagnosed via computed tomography, a conservative treatment strategy for patients without complications should be considered in order to avoid any unnecessary surgical intervention.

  8. Experience acquired over a four-year period using Vichi's scoring system in chest alterations occurring in patients with cystic fibrosis

    International Nuclear Information System (INIS)

    The classification of chest alterations in Cystic Fibrosis (CF) and related score proposed by Chrispin and Norman has been widely adopted in Europe and is still applied (although slightly modified) in most European Centres. Brasfield classification instead has been monstly used in the USA. Lately, however, to revise both classifications, the need has been felt, for a more precise correlation to anatomo-radiological data as well as for inclusion of headings which have not been taken into account so far. In 1980 one of the authors (Vichi) worked out a new scoring system for the chest alterations of CF. Results are reported from a follow-up of 15 patients with CF carried out at the FC Center of Meyer Ospedale in Florence from the late 1981 to 1985. The patients underwent periodic checking including determination of clinical scoring system-according to Shwachman and Kulczychi modified by Doershuk-respiratory function tests, chest X-rays evaluated by three radiologists separately, following both Chrispin and Norman and Vichi scoring systems. The latter system has proved to be well correlated to clinical data and to the ordinary pulmonary function tests but it mainly presents a high observer reproducibility

  9. Epiploic appendicitis and omental infarction. Findings in the ultrasonography and computerized tomography; Apendicitis epiploica e infarto omental. Hallazgos en ecografia y tomografia computarizada

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    Migule, A.; Ripolles, T.; Martinez, M. J.; Morote, V.; Ruiz, A. [Hospital Universitario Dr. Peset. Valencia (Spain)

    2001-07-01

    Describe the findings in the ultrasonography and computerized tomography (CT) of the omental infarction and epiploic appendicitis. The clinical and radiological findings of the patients diagnosed in our hospital with epiploic appendicitis or omental infarctions between August 1994 and March 2001 were assessed retrospectively. We found a high incidence (42 cases) of these two diseases: 30 patients with diagnosis of epiploic appendicitis and 12 with the diagnosis of omental infarction. Four patients were treated surgically, while the remaining 38 were conservatively, without posterior complications. The ultrasonography and CT images is characteristics. making it possible to make a diagnosis of epiploic appendicitis or omental infarction with certainty. It is not necessary to make a differential diagnosis between the two entities because their prognosis and treatment are similar. Their incidence is much more frequent than that previously published. (Author) 19 refs.

  10. Cystic Fibrosis

    Science.gov (United States)

    ... mucus and needs to spit it out. In gym class, she participates in sports but often gets ... make normal mucus, the body needs a special protein. This protein is defective in cystic fibrosis, producing ...

  11. Renal-cell carcinomas in end-stage kidneys: a clinicopathological study with emphasis on clear-cell papillary renal-cell carcinoma and acquired cystic kidney disease-associated carcinoma.

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    Bhatnagar, Ramneesh; Alexiev, Borislav A

    2012-02-01

    Clear-cell papillary renal-cell carcinoma (CCPC) and acquired cystic kidney disease-associated carcinoma (ACDAC) are neoplasms with distinct morphological characteristics that behave less aggressively than conventional renal-cell carcinomas. End-stage kidney specimens from 61 patients (47 males and 14 females) with 109 renal-cell carcinomas were selected. Papillary renal-cell carcinoma was the most common malignancy (61/109, 56%), followed by CCPC (20/109, 18%). The CCPC showed a papillary or tubular/solid architecture, clear cytoplasm, low nuclear grade, and a distinct immunohistochemical profile (RCC-, vimentin+, CK7+, p504S-). ACDAC displayed a variety of architectural patterns, eosinophilic cytoplasm, high nuclear grade, intratumoral calcium oxalate deposits, and an immunohistochemical profile similar to type 2 papillary renal-cell carcinoma (RCC+, vimentin+, CK7-/+, p504S+). Less than 5% (3/69) of pathologically staged renal-cell carcinomas in end-stage kidneys presented with lymphogenous and/or hematogenous metastases.

  12. Cystic Echinococcosis.

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    Agudelo Higuita, Nelson Iván; Brunetti, Enrico; McCloskey, Cindy

    2016-03-01

    Echinococcosis is one of the 17 neglected tropical diseases (NTDs) recognized by the World Health Organization. The two major species of medical importance are Echinococcus granulosus and Echinococcus multilocularis. E. granulosus affects over 1 million people and is responsible for over $3 billion in expenses every year. In this minireview, we discuss aspects of the epidemiology, clinical manifestations, and diagnosis of cystic echinococcosis or cystic hydatid disease caused by E. granulosus. PMID:26677245

  13. Cystic fibrosis - resources

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    Resources - cystic fibrosis ... The following organizations are good resources for information on cystic fibrosis : Cystic Fibrosis Foundation -- www.cff.org March of Dimes -- www.marchofdimes.org/baby/cystic-fibrosis-and- ...

  14. Omental torsion in acute abdomen: a case report

    Directory of Open Access Journals (Sweden)

    Alibakhshi A

    2010-01-01

    Full Text Available "n Normal 0 false false false EN-US X-NONE AR-SA MicrosoftInternetExplorer4 /* Style Definitions */ table.MsoNormalTable {mso-style-name:"Table Normal"; mso-tstyle-rowband-size:0; mso-tstyle-colband-size:0; mso-style-noshow:yes; mso-style-priority:99; mso-style-qformat:yes; mso-style-parent:""; mso-padding-alt:0in 5.4pt 0in 5.4pt; mso-para-margin:0in; mso-para-margin-bottom:.0001pt; mso-pagination:widow-orphan; font-size:11.0pt; font-family:"Calibri","sans-serif"; mso-ascii-font-family:Calibri; mso-ascii-theme-font:minor-latin; mso-fareast-font-family:"Times New Roman"; mso-fareast-theme-font:minor-fareast; mso-hansi-font-family:Calibri; mso-hansi-theme-font:minor-latin; mso-bidi-font-family:Arial; mso-bidi-theme-font:minor-bidi;} Background: Management of acute abdomen usually does not necessitate accurate pre-operative diagnosis but the surgeon should make a decision about the need for emergent laparotomy. This fact is somehow different for localized peritonitis (e.g. acute appendicitis in which the clinical presentation directs straightly to the diagnosis. However, acute appendicitis has lots of differential diagnoses, finding the normal appendix during laparotomy is just a start point to look for other diagnoses. Omental torsion is a rare cause for acute abdomen that is usually missed. Knowing about this rare condition and its frequently encountered presentation at the operating room (sero-sanguinous fluid coming out of peritoneal cavity may prevent missing the diagnosis and doing a malpractice."n"nCase report: Report a 9 year- old boy presented with acute abdomen which turned out to be an omental torsion after the operation."n"nConclusion: Knowledge about this rare condition "omental torsion" and its clinical and intra operative presentations may prevent missing the diagnosis and a malpractice.

  15. Cystic Lymphangioma of Pancreas.

    Science.gov (United States)

    Bihari, Chhagan; Rastogi, Archana; Rajesh, S; Arora, Ankur; Arora, Asit; Kumar, Nikhil

    2016-03-01

    Lymphangiomas are benign hamartomatous malformations which can arise either from congenitally sequestered lymphatic channels or due to acquired obstruction caused by fibrosis of lymph channels. They are common in the pediatric age group in the soft tissue of neck and the axilla. Abdominal lymphangiomas are rare; even rarer is the primary involvement of pancreas. It occurs more frequently in females and is often located in the distal pancreas. The authors report the case of cystic lymphangioma of pancreas in a 26-year old female presenting with recurrent episodes of upper abdominal pain that was treated with laparoscopic cyst excision. Although exceptionally rare, lymphangioma of the pancreas should be considered in the differential diagnosis of pancreatic cystic lesions, especially in young women. PMID:27065694

  16. Free greater omental flap for treatment of mandibular osteoradionecrosis

    Energy Technology Data Exchange (ETDEWEB)

    Moran, W.J.; Panje, W.R.

    1987-04-01

    Osteoradionecrosis can involve the mandible following radical irradiation for treatment of oral cavity cancer. The radionecrosis of the mandible is often associated with severe intractable pain, local or extensive deformity, including pathologic fracture, orocutaneous fistula formation, and frequent loss of function. Treatment has ranged from analgesia and antibiotics to hyperbaric oxygen treatments to local or extensive sequestrectomies with partial or total mandibulectomy and restoration of tissue losses with unirradiated tissue. To our knowledge, this is the first report of the successful use of a free greater omental flap for immediate treatment of mandibular osteoradionecrosis and concomitant reconstruction. We found the omentum to be an excellent vascular bed that rapidly resolved the osteoradionecrosis and pain, promoted healing, and restored mandibular function with minimal discomfort to the patient.

  17. Secondary omental infarction related to open and laparoscopic-assisted distal gastrectomy: report of two cases.

    Science.gov (United States)

    Park, Kyung Eun; Chung, Dong Jin; Kim, Wook; Hahn, Seong-Tae; Lee, Jae Moon

    2011-01-01

    Omental infarction occurring after open and laparoscopic-assisted distal gastrectomy with partial omentectomy for gastric cancer was a very rare disease in the past, but its incidence has increased as more partial omentectomies are now being performed. But there are few case reports or radiologic studies on its increasing incidence. It is necessary to differentiate omental infarction from carcinomatosis peritonei, since both have similar imaging findings. In this report, we describe two cases of omental infarction; each occurred after open and laparoscopic-assisted distal gastrectomy in early gastric cancer patients. Partial omentectomy was performed in both cases. Omental infarction following distal gastrectomy with partial omentectomy can be discriminated from carcinomatosis peritonei by comparing with different initial and follow up CT findings.

  18. The oncoplastic breast surgery with pedicled omental flap harvested by laparoscopy: initial experiences from China

    OpenAIRE

    Guan, Dandan; Lin, Hui; LV, ZHENYE; Xin, Ying; MENG, KEXIN; Song, Xiangyang

    2015-01-01

    Background A new technique of oncoplastic breast surgery (OBS) using laparoscopically harvested pedicled omental flap has been developed in the past 10 years. This study aimed to evaluate the feasibility of this technique. Methods Twenty-five patients underwent OBS using laparoscopically harvested omental flap. Operative time, blood loss, complications, recurrence, and cosmetic outcomes were prospectively analyzed. Results Between June 2010 and March 2014, 25 patients were recruited in our st...

  19. 99mTc-Macroaggregated Albumin SPECT/CT Perfusion Imaging of Omental Extrahepatic Vascularization.

    Science.gov (United States)

    Nguyen, Ba D; Yang, Ming; Roarke, Michael C

    2016-01-01

    The authors present a case of omental perfusion by an extrahepatic branch of the right hepatic artery depicted during pre-embolization planning with 99mTc-macroaggregated albumin SPECT/CT imaging. This omental scintigraphic finding corresponds to an extrahepatic branch of the right hepatic artery demonstrated by selective angiography and related CT angiography. The authors would like to add the omentum to the previously reported sites of extrahepatic vascularization encountered during the hepatic pre-embolization imaging. PMID:26650883

  20. Generalized peritonitis requiring re-operation after leakage of omental patch repair of perforated peptic ulcer

    Directory of Open Access Journals (Sweden)

    Hemmat Maghsoudi

    2011-01-01

    Full Text Available Background/Aim: Peptic ulcer perforations are a common emergency, but available literature is silent on the exact definition, incidence, management, and complications of peritonitis due to omental patch leakage. Patients and Methods: Retrospective data were collected on 422 patients who underwent omental patch repair of perforated peptic ulcer between March 20, 1999 and March 20, 2006. The definitive diagnosis of perforated peptic ulcer and omental patch leakage was obtained at surgery. Results: Seventeen (4% patients experienced generalized peritonitis due to omental patch leakage. Mean age was 60.6 years. Mortality rate was 29.4%, and the mean hospital stay was 23.6 days. Delay in surgical approach, shock on admission, and age were all significantly associated with increased mortality. Conclusions: Peritonitis due to omental patch leakage can result in significant morbidity and mortality. The most common causes of omental patch leakage and operative procedures were unknown and reinsertion of omentum, respectively. Factors such as shock on admission or delayed surgery, have significantly contributed to fatal outcomes and need careful attention.

  1. Computed Tomography and Ultrasound of Omental Infarction in Children: Differential Diagnoses of Right Lower Quadrant Pain

    Energy Technology Data Exchange (ETDEWEB)

    Lim, So Yeoun; Hong, Hyun Sook; Lee, Hae Kyung; Lee, Min Hee [Dept. of Radiology, Soonchunhyang University College of Medicine, Bucheon Hospital, Bucheon (Korea, Republic of); Kim, Young Tong [Dept. of Radiology, Soonchunhyang University College of Medicine, Cheonan Hospital, Cheonan (Korea, Republic of)

    2013-03-15

    Omental infarction in children occurs rarely and is often confused with other diseases that cause right lower quadrant (RLQ) pain. This study evaluates ultrasonography (US) and computed tomography (CT) findings of omental infarction in children with abdominal pain. The CT and US findings and clinical presentations of nine children diagnosed with omental infarction between 2005 and 2012 were retrospectively reviewed. Distributions of abdominal pain in the patients included RLQ (n = 6), right upper quadrant (RUQ, n = 1), periumbilical (n = 1), and the epigastric (n = 1) region. All patients underwent abdominal CT, and three underwent abdominal US. On CT scan, a typical triangular, heterogeneous fatty mass was seen between the abdominal wall and ascending colon (n = 6) or hepatic flexure (n = 1). A fatty mass with an enhanced rim that mimicked acute appendagitis was present in two patients. The other two patients had diffuse fat infiltration without mass. On US, a heterogeneously hyperechoic omental mass was seen in the RLQ (n = 2) or RUQ (n = 1). Three patients underwent appendectomy and partial omentectomy, and pathology confirmed omental infarction. Knowledge of the typical imaging features of omental infarction and application for diagnosis are important for its differentiation from other conditions that also present with RLQ pain and can avoid unnecessary surgery.

  2. What Causes Cystic Fibrosis?

    Science.gov (United States)

    ... page from the NHLBI on Twitter. What Causes Cystic Fibrosis? A defect in the CFTR gene causes cystic ... in the severity of the disease. How Is Cystic Fibrosis Inherited? Every person inherits two CFTR genes—one ...

  3. Cystic Fibrosis Research

    Science.gov (United States)

    ... please turn Javascript on. Feature: Steady Advances Against Cystic Fibrosis Cystic Fibrosis Research Past Issues / Fall 2012 Table of Contents "Remarkable strides in cystic fibrosis research over the past two decades have culminated ...

  4. 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 ...

  5. Cystic fibrosis

    OpenAIRE

    2004-01-01

    In the 1930s, when cystic fibrosis (CF) was first clearly recognised, it was a disorder that inevitably led to death in early childhood from respiratory failure and malnutrition. Since that time, antibiotic treatment and improving nutrition have brought increasing hope to sufferers from the disorder, so that increasing numbers of children have lived on into adult life. Chaired by Professor John Walker-Smith, and attended by a group of leading experts in field at the time, this transcript disc...

  6. Malignant mesothelioma of the greater omentum mimicking omental infarction: A case report

    Institute of Scientific and Technical Information of China (English)

    Min-Kee Shin; Ok-Jae Lee; Chang-Yoon Ha; Hyun-Joo Min; Tae-Hyo Kim

    2009-01-01

    Mesothelioma develops most commonly in the pleura, and less frequently in the peritoneum. Usually, it manifests as diffuse peritoneal thickening and multiple nodules, and rarely as a solitary mass. We report a rare case of primary malignant mesothelioma of the greater omentum, which mimicked omental infarct. A 54-year-old Korean man was admitted because of severe abdominal pain of sudden onset. A tender mass with indistinct margins was palpated in the upper abdomen. Abdominal ultrasound and computed tomography showed an illdefined mass in the greater omentum and little ascites in the peri-hepatic space, and neutrophil-dominant exudates were documented on paracentesis. Intravenous antibiotics and analgesics were given for omental infarction with superimposed infection, which resulted in symptomatic improvement. The imaging studies after a week revealed a growing mass and ascites. Laparoscopic surgery was performed and an 8 cm × 3.3 cm greater omental mass was found, with multiple small nodules on the peritoneum, diaphragm, and pelvic cavity wall. Histological examination showed proliferating malignant epithelioid cells that stained strongly for calretinin, which was compatible with malignant mesothelioma. We recommend that primary omental mesothelioma should be included in the differential diagnosis of patients with omental infarction, despite its rarity.

  7. Increased levels of endothelin ETB receptor mRNA in human omental arteries after organ culture

    DEFF Research Database (Denmark)

    Möller, S; Adner, M; Edvinsson, L

    1998-01-01

    1. Using competitive reverse transcription-polymerase chain reaction (RT-PCR) and in vitro pharmacology, smooth muscle endothelin ETB receptor expression was studied in segments of human omental artery, fresh and after organ culture for 1 and 5 days. 2. The competitive RT-PCR assay used in the pr...

  8. A huge 6.2 kilogram uterine myoma coinciding with omental leiomyosarcoma: case report.

    Science.gov (United States)

    Ruan, C W; Lee, C L; Yen, C F; Wang, C J; Soong, Y K

    1999-12-01

    Surgery for massive abdominal tumors is both interesting and challenging. We present a case involving a multiple uterine myoma weighing 6.2 Kg which coincided with omental leiomyosarcoma. To our knowledge, this is the first report of this type of condition in the English literature. A 44-year-old nulliparous woman had suffered from abdominal pain for a long time. A huge abdominal mass was palpated on physical examination. Computed tomography scanning revealed a huge pelvic-abdominal mass with the possibility of small bowel loops invaded by the mass. A 6-cm omental mass was incidentally found during the subsequent hysterectomy procedure. Perforation of the urinary bladder occurred during the dissection of adhesion. Resection of the omental mass, wide wedge resection of the invaded small bowel, primary repair of the bladder, and hysterectomy were performed. The final pathologic diagnosis was uterine leiomyomata with omental leiomyosarcoma. The patient returned home on postoperative day 14 and was well at the 18-month follow-up examination. The challenge of these tumors lies in their proper diagnosis and surgical management. More case reports and follow-up studies are needed to confirm the efficacy of their management.

  9. Omental transposition flap in colorectal carcinoma: adjunctive use in prevention and treatment of radiation complications

    Energy Technology Data Exchange (ETDEWEB)

    Russ, J.E. (St. Joseph Hospital, Elgin, IL); Smoron, G.L.; Gagnon, J.D.

    1984-01-01

    The versatility of the omentum has led to its use as a surgical adjunct in the total oncological management of primary and recurrent colorectal carcinoma. The omentum is used as a transposition pedicle flap, broadly based on the left gastroepiploic vascular supply. Following abdominoperineal resection or low anterior resection of the rectum, the small bowel is elevated out of the pelvis by the omental bulk. The pelvic defect is reperitonealized and the risk of pelvic small bowel adhesions is diminished. With the increasing use of postoperative radiation to the pelvis for rectal carcinoma, the tolerance to therapy may be improved and the incidence of radiation enteritis and enteropathy should be reduced. Surgical complications such as leakage from low anterior anastomoses and pelvic abscesses, which may delay or contraindicate necessary postoperative radiation, are dramatically decreased. Reconstruction of the perineum with omental flap provides adequate soft tissue bulk and contour when a radical resection has been performed. The omental flap has been used in 24 patients with colorectal carcinoma; one flap was lost as a result of distal omental infarction in a patient with recurrent rectal carcinoma and radionecrosis of the perineum. The safety and ease of this procedure has allowed increased surgical innovation, especcially in the prevention and treatment of radiation complications.

  10. Omental transposition flap in colorectal carcinoma: adjunctive use in prevention and treatment of radiation complications

    International Nuclear Information System (INIS)

    The versatility of the omentum has led to its use as a surgical adjunct in the total oncological management of primary and recurrent colorectal carcinoma. The omentum is used as a transposition pedicle flap, broadly based on the left gastroepiploic vascular supply. Following abdominoperineal resection or low anterior resection of the rectum, the small bowel is elevated out of the pelvis by the omental bulk. The pelvic defect is reperitonealized and the risk of pelvic small bowel adhesions is diminished. With the increasing use of postoperative radiation to the pelvis for rectal carcinoma, the tolerance to therapy may be improved and the incidence of radiation enteritis and enteropathy should be reduced. Surgical complications such as leakage from low anterior anastomoses and pelvic abscesses, which may delay or contraindicate necessary postoperative radiation, are dramatically decreased. Reconstruction of the perineum with omental flap provides adequate soft tissue bulk and contour when a radical resection has been performed. The omental flap has been used in 24 patients with colorectal carcinoma; one flap was lost as a result of distal omental infarction in a patient with recurrent rectal carcinoma and radionecrosis of the perineum. The safety and ease of this procedure has allowed increased surgical innovation, especcially in the prevention and treatment of radiation complications

  11. Comparison of Closure of Gastric Perforation Ulcers With Biodegradable Lactide-Glycolide-Caprolactone or Omental Patches

    NARCIS (Netherlands)

    Bertleff, Marietta J. O. E.; Stegmann, Toon; Liem, Robert S. B.; Kors, Geert; Robinson, Peter H.; Nicolai, Jean Philippe; Lange, Johan F.

    2009-01-01

    Background: The current treatment of perforated peptic ulcers is primary closure, supported by the application of an omental patch. It is difficult and time consuming to perform this procedure by laparoscopic surgery, largely because of the required suturing. It was our aim to develop and test a new

  12. [Primary Pelvic Cystic Echinococcosis].

    Science.gov (United States)

    Yaman, İsmail; İnceboz, Ümit; İnceboz, Tonay; Keyik, Bahar; Uzgören, Engin

    2015-06-01

    Cystic echinococcosis caused by Echinococcus granulosus is still an important health problem in endemic areas. Cystic echinococcosis may involve different organs or areas with the most common sites being the liver and the lungs. Pelvic involvement has previously been reported and was mainly accepted as secondary to cystic echinococcosis in other organs, isolated pelvic involvement is very rare. In this case report, we aimed to present the case with pelvic cystic mass that was finally diagnosed with isolated pelvic cystic echinococcosis in and after the operation, and we would like to draw attention to include "cystic echinococcosis" in the differential diagnosis of pelvic masses.

  13. The relationship of omental and subcutaneous adipocyte size to metabolic disease in severe obesity.

    LENUS (Irish Health Repository)

    O'Connell, Jean

    2012-02-01

    OBJECTIVE: Several studies have reported the existence of a subgroup of obese individuals with normal metabolic profiles. It remains unclear what factors are responsible for this phenomenon. We proposed that adipocyte size might be a key factor in the protection of metabolically healthy obese (MHO) individuals from the adverse effects of obesity. SUBJECTS: Thirty-five patients undergoing bariatric surgery were classified as MHO (n = 15) or metabolically unhealthy obese (MUO, n = 20) according to cut-off points adapted from the International Diabetes Federation definition of the metabolic syndrome. Median body mass index (BMI) was 48 (range 40-71). RESULTS: There was a moderate correlation between omental adipocyte size and subcutaneous adipocyte size (r = 0.59, p<0.05). The MHO group had significantly lower mean omental adipocyte size (80.9+\\/-10.9 microm) when compared with metabolically unhealthy patients (100.0+\\/-7.6 microm, p<0.0001). Mean subcutaneous adipocyte size was similar between the two groups (104.1+\\/-8.5 microm versus 107.9+\\/-7.1 microm). Omental, but not subcutaneous adipocyte size, correlated with the degree of insulin resistance as measured by HOMA-IR (r = 0.73, p<0.0005), as well as other metabolic parameters including triglyceride\\/HDL-cholesterol ratio and HbA1c. Twenty-eight patients consented to liver biopsy. Of these, 46% had steatohepatitis and fibrosis. Fifty percent (including all the MHO patients) had steatosis only. Both omental and subcutaneous adipocyte size were significantly associated with the degree of steatosis (r = 0.66, p<0.0001 and r = 0.63, p<0.005 respectively). However, only omental adipocyte size was an independent predictor of the presence or absence of fibrosis. CONCLUSION: Metabolically healthy individuals are a distinct subgroup of the severely obese. Both subcutaneous and omental adipocyte size correlated positively with the degree of fatty liver, but only omental adipocyte size was related to metabolic health

  14. Isolated omental metastasis of renal cell carcinoma after extraperitoneal open partial nephrectomy: A case report

    Science.gov (United States)

    Acar, Ömer; Mut, Tuna; Sağlıcan, Yeşim; Sag, Alan Alper; Falay, Okan; Selcukbiricik, Fatih; Tabak, Levent; Esen, Tarık

    2016-01-01

    Introduction Metachronous metastatic spread of clinically localized renal cell carcinoma (RCC) affects almost 1/3 of the patients. They occur most frequently in lung, liver, bone and brain. Isolated omental metastasis of RCC has not been reported so far. Case presentation A 62-year-old patient previously diagnosed and treated due to pulmonary sarcoidosis has developed an omental metastatic lesion 13 years after having undergone open extraperitoneal partial nephrectomy for T1 clear-cell RCC. Constitutional symptoms and imaging findings that were attributed to the presence of a sarcomatoid paraneoplastic syndrome triggered by the development this metastatic focus complicated the diagnostic work-up. Biopsy of the [18F]-fluorodeoxyglucose (+) lesions confirmed the diagnosis of metastatic RCC and the patient was managed by the resection of the omental mass via near-total omentectomy followed by targeted therapy with a tyrosine kinase inhibitor. Discussion Late recurrence of RCC has been reported to occur in 10–20% of the patients within 20 years. Therefore lifelong follow up of RCC has been advocated by some authors. Diffuse peritoneal metastases have been reported in certain RCC subtypes with adverse histopathological features. However, isolated omental metastasis without any sign of peritoneal involvement is an extremely rare condition. Conclusion To our knowledge, this is the first reported case of metachronously developed, isolated omental metastasis of an initially T1 clear-cell RCC. Constitutional symptoms, despite a long interval since nephrectomy, should raise the possibility of a paraneoplastic syndrome being associated with metastatic RCC. Morphological and molecular imaging studies together with histopathological documentation will be diagnostic. PMID:26874583

  15. CT findings of lymphoma with peritoneal, omental and mesenteric involvement: Peritoneal lymphomatosis

    Energy Technology Data Exchange (ETDEWEB)

    Karaosmanoglu, Devrim [Department of Radiology, Hacettepe University School of Medicine, Ankara (Turkey); Karcaaltincaba, Musturay [Department of Radiology, Hacettepe University School of Medicine, Ankara (Turkey)], E-mail: musturayk@yahoo.com; Oguz, Berna; Akata, Deniz; Ozmen, Mustafa; Akhan, Okan [Department of Radiology, Hacettepe University School of Medicine, Ankara (Turkey)

    2009-08-15

    Purpose: We aimed to describe computed tomography (CT) findings in patients with peritoneal, omental and mesenteric lymphoma involvement. Materials and methods: We searched our archive retrospectively to find out patients with peritoneal, omental and mesenteric lymphoma involvement. We found 16 patients with non-hodgkin lymphoma meeting these criteria. CT studies of these patients were reevaluated for the presence of peritoneal involvement, ascites, omental mass, organomegaly, retroperitoneal lymphadenopathy, bowel wall thickening and other associated findings. Results: There were 14 males and 2 females with peritoneal and/or mesenteric and omental lymphoma involvement. Mean age was 39 (range 4-76). Subgroups of non-hodgkin lymphoma were diffuse large B-cell lymphoma (n = 11), small cell lymphocytic lymphoma (n = 2), small cleaved cell lymphoma (n = 1), T-cell lymphoma (n = 1) and Burkitt's lymphoma (n = 1). Peritoneal involvement was seen in 15 patients (93.8%) in the form of linear (n = 12) and nodular (n = 3) thickening. Ascites was seen in 12 (75%) patients. Omental and mesenteric masses were present in 10 (66.6%) and 10 (66.6%) patients, respectively. Bowel wall thickening, retroperitoneal lymphadenopathy and hepatosplenomegaly were also common and observed in 10, 10 and 11 patients, respectively. Solid organ involvement in the form of liver and splenic lesions was seen in 9 (56%) patients. Conclusion: Peritoneal involvement can be seen in many subtypes of lymphoma and most frequently in diffuse large B-cell lymphoma. Peritoneal lymphomatosis can mimic peritoneal carcinomatosis and should be included in the differential diagnosis list in patients with ascites, hepatosplenic lesions and unidentified cause of peritoneal thickening on CT in a male patient.

  16. Cystic acoustic neuromas

    OpenAIRE

    Chitkara, Naveen; Chanda, Rakesh; Yadav, S. P. S.; N.K. Sharma

    2002-01-01

    Predominantly cystic acoustic neuromas are rare and they usually present with clinical and radiological features different from their more common solid counterparts. Two cases of cystic acoustic neuromas are reported here.

  17. Cystic fibrosis - nutritional considerations

    Science.gov (United States)

    ... this page: //medlineplus.gov/ency/article/002437.htm Cystic fibrosis - nutritional considerations To use the sharing features on this page, please enable JavaScript. Cystic fibrosis (CF) is a life-threatening disease that causes ...

  18. Cystic fibrosis: case report

    International Nuclear Information System (INIS)

    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

  19. Cystic fibrosis: case report

    Energy Technology Data Exchange (ETDEWEB)

    Park, Si Hyun; Lee, Hyun Ju; Kim, Ji Hye; Park, Chol Heui [Gachon Medical School, Inchon (Korea, Republic of)

    2002-12-01

    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.

  20. CT findings of peritoneal tuberculosis and peritoneal carcinomatosis: relationship between peritoneal change and omental infiltration

    International Nuclear Information System (INIS)

    To compare the CT findings of peritoneal tuberculosis (PT) and peritoneal carcinomatosis (PC) based on the morphologic features of the peritoneum and assess the relationship between the degree of peritoneal thickness and the severity of omental infiltration in PT and PC. We retrospectively reviewed the CT findings of 15 patients with PT and 14 with PC. We checked morphologic changes of the peritoneum as seen on CT, for the following points: 1) the presence of peritoneal change: 2) the pattern of any change-diffuse thickening, plaque or nodularity, combined thickening (diffuse and plaque, or nodularity) 3) the degree of thickness on the whole peritoneum - mild (grossly definite, but not more than 3mm), moderate (more than 3mm); 4) the presence of irregularity on the peritoneal surface. We also evaluated the significance of the relationship between peritoneal thickness and omental infiltration in both disease entities. The degree of omental infiltration was described as follows : grade 1 (no change or focal smudge pattern); grade 2 (diffuse smudge), grade 3 (omental cake regardless extent). Peritoneal change was seen in 12 of 15 PT patients and in 7 of 14 PC patients. In all 12 PT patients, the pattern of change was diffuse thickening, and among the seven PC patients, there was diffuse thickening in one, plaque or nodular thickening in four, and combined thickening in two. In PT patients, the degree of thickness on the whole peritoneum was mild in six and moderate in six, and in PC patients it was mild in two and moderate in one. An irregular peritoneum surface was seen in one patient with PT and in two with PC. The degree of omental infiltration in PT was grade 1 in four patients, grade 2 in six and grade 3 in five. In PC, it was grade 1 in six patients, grade 2 in two and grade 3 in six. Smooth diffuse thickening of the peritoneum was seen in 11 of 15 PT cases and in one of 14 PC (P 0.5). In PT and PC different CT findings based on peritoneal morphologic changes

  1. Role of the Use of Omental Flap in Prognosis of Cases with Induced Acute Pancreatitis in Experimental Dogs

    OpenAIRE

    Mohamed Hassan; Nader Shaaban; Abu-Seida, Ashraf M.; Mostafa Khodeir; Reem Jan; Hisham Elsharkawy; Engie Hefnawy

    2016-01-01

    Aim Acute pancreatitis frequently involves peripancreatic tissues and remote organ systems resulting in severe complications and high risk of mortality. Therefore, the present study was carried out to assess the effect of omental flap as a new treatment of acute pancreatitis. Methods Ten mongrel dogs with experimentally induced acute pancreatitis were randomly divided into two equal groups; treated and control groups. The pancreas was wrapped with omental flap in the treated group and the pan...

  2. Greater Omental Pancake Tumour due to Metastasis of Ovarian Cancer – A Cadaveric Study

    Science.gov (United States)

    Bhusari, Prashant A.; Khairnar, Karan B.

    2014-01-01

    Cancer of ovary is the one of the common of all gynecological tumors and is the leading cause of death among women. A unique attempt is made to trace masses & its causes found in an abdomen of female cadaver during routine anatomy dissection. The mass was thick, hard and somewhat nodular in the region of greater omentum, After dissecting the pelvic cavity, it was found that both the ovaries were bulky, nodular & hard. Whole abdominal cavity was dissected & found that liver also involved by metastasis. Case suggests that there is the development of metastatic omental mass from grade IV ovarian cancer. Primary human omental adipocytes promote homing, migration and invasion of ovarian cancer cells. Adipokines like interleukin-8 (IL-8) mediate these activities. PMID:24596747

  3. Greater Omental Pancake Tumour due to Metastasis of Ovarian Cancer - A Cadaveric Study.

    Science.gov (United States)

    Bhusari, Prashant A; Khairnar, Karan B

    2014-01-01

    Cancer of ovary is the one of the common of all gynecological tumors and is the leading cause of death among women. A unique attempt is made to trace masses & its causes found in an abdomen of female cadaver during routine anatomy dissection. The mass was thick, hard and somewhat nodular in the region of greater omentum, After dissecting the pelvic cavity, it was found that both the ovaries were bulky, nodular & hard. Whole abdominal cavity was dissected & found that liver also involved by metastasis. Case suggests that there is the development of metastatic omental mass from grade IV ovarian cancer. Primary human omental adipocytes promote homing, migration and invasion of ovarian cancer cells. Adipokines like interleukin-8 (IL-8) mediate these activities. PMID:24596747

  4. How Is Cystic Fibrosis Diagnosed?

    Science.gov (United States)

    ... page from the NHLBI on Twitter. How Is Cystic Fibrosis Diagnosed? Doctors diagnose cystic fibrosis (CF) based on ... tested to see whether the baby has CF. Cystic Fibrosis Carrier Testing People who have one normal CFTR ...

  5. Genetics Home Reference: cystic fibrosis

    Science.gov (United States)

    ... Me Understand Genetics Home Health Conditions cystic fibrosis cystic fibrosis Enable Javascript to view the expand/collapse boxes. Print All Open All Close All Description Cystic fibrosis is an inherited disease characterized by the buildup ...

  6. How Is Cystic Fibrosis Treated?

    Science.gov (United States)

    ... page from the NHLBI on Twitter. How Is Cystic Fibrosis Treated? Cystic fibrosis (CF) has no cure. However, ... help oral pancreatic enzymes work better. Treatments for Cystic Fibrosis Complications A common complication of CF is diabetes . ...

  7. Case Report of Multimodality Imaging in Omental Cake: Plain Radiograph, Computed Tomography, and Ultrasonography: A Care-Compliant Article.

    Science.gov (United States)

    Juan, Yu-Hsiang; Cheung, Yun-Chung; Ng, Koon-Kwan; Ng, Shu-Hang; Huang, Jen-Seng; Chang, Liang-Che; Lin, Yu-Ching

    2015-11-01

    The imaging finding of omental cake has been demonstrated in other modalities, such as computed tomography, magnetic resonance imaging, and ultrasonography. However, to the best of our knowledge, the image presentation of omental cake on a routine kidney-ureter-bladder film has not been reported before in the literature. We presented a unique case of a 61-year-old woman, with known advanced cecal colon mucinous adenocarcinoma, presented to our institution with abdominal fullness, poor appetite, and decreased stool passage for 20 days. Physical examination was unremarkable, except distended abdomen. Subsequent study revealed massive post-pigtail catheter drainage ascites with a prominent soft-tissue mass-causing centralization and tethering of focally distended small bowel gas, suggestive of omental cake on plain radiograph. The imaging finding in plain radiograph corresponds to the findings in other imaging modalities, including abdominal sonography and computed tomography. The patient underwent subtotal colectomy and ileostomy during later courses of chemotherapy due to adhesion ileus and possible intraabdominal abscess, and pathologic study confirmed the diagnosis of cecal mucinous adenocarcinoma and peritoneal carcinomatosis. Although the image finding of omental cake on plain radiograph has never been described, this image finding is unique and should be recognized, as it may suggest the presence of omental cake when first identified in the emergency department from patients with abdominal distension and warrant further evaluation to evaluate the underlying cause.

  8. Pathologic Review of Cystic and Cavitary Lung Diseases

    OpenAIRE

    Kim, Na Rae; Han, Joungho

    2012-01-01

    Pulmonary cystic and cavitary lesions caused by diverse etiologies are commonly encountered in chest imaging. The terms "cyst" and "cavity" are used to describe air-filled regions in the center of a nodule or consolidation of the lung. To date, only radiologic aspects of these lesions have been addressed. The morphologies of pulmonary cystic and cavitary lesions exhibit a broad spectrum, ranging from benign to malignant pulmonary diseases of acquired or congenital origin, including variable i...

  9. Omental Torsion after Laparoscopic Roux-en-Y Gastric Bypass Mimicking Appendicitis: A Case Report and Review of the Literature

    Science.gov (United States)

    Basilicata, Giacinto; Nocito, Antonio

    2016-01-01

    Introduction. Laparoscopic Roux-en-Y gastric bypass (LRYGBP) is a common procedure in obesity surgery. The aim of an antecolic approach is to reduce the rate of internal herniation. Our aim is to make bariatric surgeons aware of another possible complication of antecolic LRYGBP. Methods and Results. We present a case report of omental torsion 24 months after antecolic LRYGBP presenting as an acute abdomen, suggesting appendicitis. During diagnostic laparoscopy, omental infarction due to torsion was observed. Resection of the avital omentum was performed. Discussion. Omental torsion after antecolic LRYGBP is a rare complication. When appearing in the early postoperative phase, it may mimic an anastomotic leakage. It may also occur as late complication, presenting with acute abdomen as an appendicitis. PMID:27019758

  10. Human omental-derived adipose stem cells increase ovarian cancer proliferation, migration, and chemoresistance.

    Directory of Open Access Journals (Sweden)

    Aleksandra Nowicka

    Full Text Available Adipose tissue contains a population of multipotent adipose stem cells (ASCs that form tumor stroma and can promote tumor progression. Given the high rate of ovarian cancer metastasis to the omental adipose, we hypothesized that omental-derived ASC may contribute to ovarian cancer growth and dissemination.We isolated ASCs from the omentum of three patients with ovarian cancer, with (O-ASC4, O-ASC5 and without (O-ASC1 omental metastasis. BM-MSCs, SQ-ASCs, O-ASCs were characterized with gene expression arrays and metabolic analysis. Stromal cells effects on ovarian cancer cells proliferation, chemoresistance and radiation resistance was evaluated using co-culture assays with luciferase-labeled human ovarian cancer cell lines. Transwell migration assays were performed with conditioned media from O-ASCs and control cell lines. SKOV3 cells were intraperitionally injected with or without O-ASC1 to track in-vivo engraftment.O-ASCs significantly promoted in vitro proliferation, migration chemotherapy and radiation response of ovarian cancer cell lines. O-ASC4 had more marked effects on migration and chemotherapy response on OVCA 429 and OVCA 433 cells than O-ASC1. Analysis of microarray data revealed that O-ASC4 and O-ASC5 have similar gene expression profiles, in contrast to O-ASC1, which was more similar to BM-MSCs and subcutaneous ASCs in hierarchical clustering. Human O-ASCs were detected in the stroma of human ovarian cancer murine xenografts but not uninvolved ovaries.ASCs derived from the human omentum can promote ovarian cancer proliferation, migration, chemoresistance and radiation resistance in-vitro. Furthermore, clinical O-ASCs isolates demonstrate heterogenous effects on ovarian cancer in-vitro.

  11. Protection from septic peritonitis by rapid neutrophil recruitment through omental high endothelial venules.

    Science.gov (United States)

    Buscher, Konrad; Wang, Huiyu; Zhang, Xueli; Striewski, Paul; Wirth, Benedikt; Saggu, Gurpanna; Lütke-Enking, Stefan; Mayadas, Tanya N; Ley, Klaus; Sorokin, Lydia; Song, Jian

    2016-01-01

    Acute peritonitis is a frequent medical condition that can trigger severe sepsis as a life-threatening complication. Neutrophils are first-responders in infection but recruitment mechanisms to the abdominal cavity remain poorly defined. Here, we demonstrate that high endothelial venules (HEVs) of the greater omentum constitute a main entry pathway in TNFα-, Escherichia coli (E. coli)- and caecal ligation and puncture-induced models of inflammation. Neutrophil transmigration across HEVs is faster than across conventional postcapillary venules and requires a unique set of adhesion receptors including peripheral node addressin, E-, L-selectin and Mac-1 but not P-selectin or LFA-1. Omental milky spots readily concentrate intra-abdominal E. coli where macrophages and recruited neutrophils collaborate in phagocytosis and killing. Inhibition of the omental neutrophil response exacerbates septic progression of peritonitis. This data identifies HEVs as a clinically relevant vascular recruitment site for neutrophils in acute peritonitis that is indispensable for host defence against early systemic bacterial spread and sepsis.

  12. Experimental study on spinal cord injury treated by embryonic spinal cord transplantation and greater omental transposition

    Institute of Scientific and Technical Information of China (English)

    Hao Dingjun(郝定均); Zheng Yonghong(郑永宏); Yuan Fuyong(袁福镛); He Liming; Wang Rong; Yuan Yong

    2004-01-01

    Objective: To observe the clinical efficacy of the embryonic spinal cellular transplantation and greater omental transposition for treatment of the spinal cord injury in 24 mongrel dogs. Methods: 24 adult mongrel dogs, weighing 10 ~ 13kg,bryonic spinal cellular transplantation and greater omental transposition group (group D). Each group consisted of 6 dogs. SEP(somatosensory evoked potential) and MEP (motor evoked potential) of the spinal cord were examed prior to the spinal cord injury and 2 months after the treatment to observe the changes of the animals' behavior. All dogs were killed 2 months after surgery and the spinal cord sections were obtained from T12 to L1 level for pathological analysis and observation under the electron microscope.Results: There was an obvious difference in the spinal somatosensory evoked potential and the motor evoked potential between the group D and the other three groups (group A, B, and C). Recovery of the behavior was noted. The spinal cells had survived for two months following the transplantation. Conclusion: Transplantation of the embryonic spinal cell and greater omentum for treatment of the spinal cord injury in dogs can gain a better outcome than the other groups in behavior and spinal somatosensory and motor evoked potential, but the further study is still essential to confirm its clinical efficacy.

  13. Acquired Techniques

    DEFF Research Database (Denmark)

    Lunde Nielsen, Espen; Halse, Karianne

    2013-01-01

    Acquired Techniques - a Leap into the Archive, at Aarhus School of Architecture. In collaboration with Karianne Halse, James Martin and Mika K. Friis. Following the footsteps of past travelers this is a journey into tools and techniques of the architectural process. The workshop will focus upon...

  14. Acquired blepharoptosis

    NARCIS (Netherlands)

    Oosterhuis, HJGH

    1996-01-01

    A review is given of the aetiology and possible treatment of acquired (non-congenital) blepharoptosis, which is a common but not specific sign of neurological disease: The diagnostic categories of upper eyelid drooping are scheduled as (a) pseudo-ptosis due to a local process or overactivity of eye

  15. Congenital Cystic Lung Diseases

    Directory of Open Access Journals (Sweden)

    Aditi Jain

    2013-01-01

    Full Text Available Congenital cystic diseases of the lung are a rare but significant cause of morbidity in children and young adults presenting with respiratory distress and repeated chest infections. They consist of cystic adenomatoid malformation, bronchogenic cyst, pulmonary sequestration, and congenital lobar emphysema. Surgical treatment is a safe and an effective method of treatment. Chest X-ray and computed tomography are the key imaging modalities used for diagnosis.

  16. Clinical Scenarios in Chronic Kidney Disease: Cystic Renal Diseases.

    Science.gov (United States)

    Meola, Mario; Samoni, Sara; Petrucci, Ilaria

    2016-01-01

    Cysts are frequently found in chronic kidney disease (CKD) and they have a different prognostic significance depending on the clinical context. Simple solitary parenchymal cysts and peripelvic cysts are very common and they have no clinical significance. At US, simple cyst appears as a round anechoic pouch with regular and thin profiles. On the other hand, hereditary polycystic disease is a frequent cause of CKD in children and adults. Autosomal dominant polycystic kidney disease (ADPKD) and autosomal recessive polycystic kidney disease (ARPKD) are the best known cystic hereditary diseases. ADPKD and ARPKD show a diffused cystic degeneration with cysts of different diameters derived from tubular epithelium. Medullary cystic disease may be associated with tubular defects, acidosis and lithiasis and can lead to CKD. Acquired cystic kidney disease, finally, is secondary to progressive structural end-stage kidney remodelling and may be associated with renal cell carcinoma. PMID:27169740

  17. 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...

  18. Intestinal disease in cystic fibrosis.

    OpenAIRE

    Baxter, P S; Dickson, J. A.; Variend, S; Taylor, C J

    1988-01-01

    Three children with cystic fibrosis developed steatorrhoea unresponsive to changes in pancreatic supplements. The final diagnoses were chronic giardiasis, stagnant loop syndrome, and Crohn's disease. Refractory intestinal symptoms in cystic fibrosis merit further investigation.

  19. Cystic Fibrosis: Symptoms, Diagnosis, Treatment

    Science.gov (United States)

    ... please turn Javascript on. Feature: Steady Advances Against Cystic Fibrosis Symptoms, Diagnosis, Treatment Past Issues / Fall 2012 Table ... both of the baby's CFTR genes are normal. Cystic Fibrosis Carrier Testing People who have one normal and ...

  20. 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.

  1. Multiple cystic lung disease.

    Science.gov (United States)

    Ferreira Francisco, Flavia Angélica; Soares Souza, Arthur; Zanetti, Gláucia; Marchiori, Edson

    2015-12-01

    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. PMID:26621970

  2. Sinonasal Manifestations in Cystic Fibrosis

    OpenAIRE

    Oomen, Karin P. Q.; Max M. April

    2012-01-01

    Cystic fibrosis is a genetic disease, characterized by accumulation of thickened mucous secretions in exocrine glands. Although the major clinical manifestations of the disease are pancreatic and pulmonary disease, the majority of cystic fibrosis patients will develop sinonasal manifestations as well. This paper outlines the etiology, evaluation, and management of the nasal and sinus manifestations in patients with cystic fibrosis.

  3. Retroperitoneal Cystic Lymphangioma

    Directory of Open Access Journals (Sweden)

    Ş.Sevil Altunrende

    2010-03-01

    Full Text Available Abdominal cystic lymphangioma is a rare, benign, congenital tumor. It is an developmental abnormality of the lymphatic system. Males are relatively more affected and 90% of the cases are under 5 years of age. Multilocular cystic lesion with septations is seen on abdominal ultrasonography. As echogenicity can vary depending on the content of the cyst like cellular debris, hemorrhage or chylous, liquid-liquid or fat-liquid levels can be observed. Definitive treatment is total excision. Cyst aspiration is not an effective method and nearly always ends with recurrence. Computerized tomography and magnetic resonance imaging findings of sonographically detected retroperitoneal cystic lymphangioma in a newborn girl with abdominal distention are discussed in this paper. (The Medical Bulletin of Haseki 2010; 48: 47-9

  4. Arthritis in cystic fibrosis.

    OpenAIRE

    Schidlow, D V; Goldsmith, D P; Palmer, J; Huang, N N

    1984-01-01

    We have confirmed previous observations of a transient, non-disabling recurrent arthritis in patients with cystic fibrosis. This arthritis differs from classic rheumatoid arthritis, is frequently associated with skin arthritis lesions, and its occurrence is unrelated to the severity of lung disease.

  5. Pedicle omental graft created by laparoscopic surgery for filling a radiation-induced ulcer in a woman with breast cancer

    Energy Technology Data Exchange (ETDEWEB)

    Ida, Katsuya [Kosai General Hospital, Shizuoka (Japan)

    2002-06-01

    In patients with advanced or recurrent breast cancer, it is difficult to reconstruct chest wall ulcers due to postoperative irradiation, which is often infected. We present a laparoscopic technique for creating and mobilizing an omental flap. A 63-year-old woman diagnosed with parasternal lymph node metastases from left breast cancer 11 months after standard radical mastectomy underwent lymph node resection with radiation therapy. She developed ulceration of the irradiated chest wall 3 years and 10 months later. An omental flap obtained by laparoscopy was used to fill the space after the radiation-induced ulcer was resected and covered with a free skin graft. The skin graft adapted to the omentum. This laparoscopic procedure is more cosmetrically acceptable and less invasive than laparotomy in obtaining the omentum while yielding equivalent results in chest wall reconstruction. (author)

  6. Laparoscopic versus Open Omental Patch Repair for Early Presentation of Perforated Peptic Ulcer: Matched Retrospective Cohort Study

    Directory of Open Access Journals (Sweden)

    Daniel Jin Keat Lee

    2016-01-01

    Full Text Available Introduction. The aim of this study was to compare the outcomes between laparoscopic and open omental patch repair (LOPR versus OR in patients with similar presentation of perforated peptic ulcer (PPU. The secondary aim was to evaluate the outcomes according to the severity of peritonitis. Methods. All patients who underwent omental patch repair at two university-affiliated institutes between January 2010 and December 2014 were reviewed. Matched cohort between LOPR and OR groups was achieved by only including patients that had ulcer perforation 21, LOPR is also shown to benefit, particularly resulting in significant shorter LOS (4 days versus 11 days, p<0.01. Conclusion. LOPR offers improved short-term outcomes in patients who present within 48 hours and with perforation size <2 cm. LOPR also proved to be more beneficial in high MPI cases.

  7. A rare cause of bleeding after laparoscopic sleeve gastrectomy : pseudo-aneurysm of the gastro-omental artery.

    Science.gov (United States)

    Mege, D; Louis, G; Berthet, B

    2013-01-01

    A serious complication of laparoscopic sleeve gastrectomy (LSG) is bleeding that is primarily located along the staples lines. Bleeding may be due to several causes, including hematomas, trocar sites, or visceral pseudo-aneurysms. We reported here a case of bleeding related to a pseudo-aneurysm of the gastro-omental artery. An LSG was performed on a 43-year-old woman (BMI = 46 kg/m2) without apparent surgical complications. Fifteen days later, she was admitted to the emergency department for hematemesis and symptoms of hemorrhagic shock. Abdominal computed tomography angiography revealed blood in the stomach, without a digestive leak, and active bleeding from a pseudo-aneurysm of the gastro-omental artery. An arterial embolisation was performed with the sandwich technique and angiographic guide wires and the placement of several detachable coils. The patient was discharged two days later. We demonstrated for the first time that post-LSG bleeding may involve a pseudo-aneurysm of the gastro-omental artery.

  8. Role of the Use of Omental Flap in Prognosis of Cases with Induced Acute Pancreatitis in Experimental Dogs

    Directory of Open Access Journals (Sweden)

    Mohamed Hassan

    2016-03-01

    Full Text Available Aim Acute pancreatitis frequently involves peripancreatic tissues and remote organ systems resulting in severe complications and high risk of mortality. Therefore, the present study was carried out to assess the effect of omental flap as a new treatment of acute pancreatitis. Methods Ten mongrel dogs with experimentally induced acute pancreatitis were randomly divided into two equal groups; treated and control groups. The pancreas was wrapped with omental flap in the treated group and the pancreas was left without treatment in control group. Urea, Creatinine, lactate dehydrogenase, serum Amylase, Ca, and C-reactive protein were measured in all dogs before surgery and 2 hours, 3 days, 7 days, and 4 weeks post-surgery. Weekly ultrasound examination was performed post-surgery. Exploratory laparotomy was carried out 45 days post-surgery, all findings were reported and pancreatic biopsies were collected and prepared for histopathological examination using Schmidt Scoring system. Data were displayed as the mean ± SD and SPSS was used. Statistical significance (at P < 0.05 was calculated by χ2 test. Results There was a significant difference between the two groups in favor of the treated group as regards levels of lactate dehydrogenase, ultrasonographic findings, pathological findings and Schmidt scoring. Conclusion Omental flap can be used as a new minimally invasive effective technique for treatment of acute pancreatitis.

  9. Cervicothoracic cystic dysraphism.

    Science.gov (United States)

    Valeur, Natalie S; Iyer, Ramesh S; Ishak, Gisele E

    2016-09-01

    Cystic dysraphism of the cervical and upper thoracic spine is very rare. It differs from the much more common lumbosacral dysraphism in appearance and structure, and usually portends a better prognosis due to lack of functional neurological tissue in the dysraphic sac and absent or less severe intracranial anomalies. There is ambiguity in the literature regarding terminology because of the paucity of cases. We present cases of the most common type of cervicothoracic cystic dysraphism and emphasize differences from lumbosacral myelomeningocele. Patient outcome depends on the presence of associated anomalies and whether complete surgical resection is performed. Imaging plays a critical role in surgical planning, screening the central nervous system for additional anomalies, and in the postoperative setting for evaluation of retethering. PMID:27147079

  10. Cystic Fibrosis: Prenatal Screening and Diagnosis

    Science.gov (United States)

    ... Management Education & Events Advocacy For Patients About ACOG Cystic Fibrosis: Prenatal Screening and Diagnosis Home For Patients Search ... Screening and Diagnosis FAQ171, February 2016 PDF Format Cystic Fibrosis: Prenatal Screening and Diagnosis Pregnancy What is cystic ...

  11. Multiple cystic lung disease

    OpenAIRE

    Flavia Angélica Ferreira Francisco; Arthur Soares Souza; Gláucia Zanetti; Edson Marchiori

    2015-01-01

    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 a...

  12. Cystic pulmonary hydatidosis

    Science.gov (United States)

    Sarkar, Malay; Pathania, Rajnish; Jhobta, Anupam; Thakur, Babu Ram; Chopra, Rajesh

    2016-01-01

    Cystic echinococcosis (CE) is a zoonotic parasitic disease caused by the larval stages of the cestode Echinococcus granulosus. Worldwide, pulmonary hydatid cyst is a significant problem medically, socially, and economically. Surgery is the definitive therapy of pulmonary hydatidosis. Benzimidazoles may be considered in patients with a surgical contraindication. This review will focus on pathogenesis, lifecycle, clinical features, and management of pulmonary hydatid disease. PMID:27051107

  13. Eosinophilic activation in cystic fibrosis.

    OpenAIRE

    Koller, D. Y.; Götz, M.; Eichler, I; Urbanek, R

    1994-01-01

    BACKGROUND--The neutrophil is a potent contributor to pulmonary destruction in cystic fibrosis. Since eosinophils also possess destructive potential the involvement of eosinophils in cystic fibrosis has been investigated. METHODS--Eosinophil numbers and levels of eosinophil cationic protein (ECP), a marker of eosinophil activation, were determined in the serum of 42 patients with cystic fibrosis and in the sputum of 10 of them. To determine neutrophil activation levels of myeloperoxidase (MPO...

  14. Simple esophageal hiatus omental herina%单纯食管裂孔网膜疝

    Institute of Scientific and Technical Information of China (English)

    葛青松; 黄虹; 曹和涛; 吴献华; 王鸿帼

    2014-01-01

    Objective To discusses the characteristic MSCT findings of simple esophageal hiatus omental hernia (SE-HOH) .Methods Prospective study was made on 7 cases of SEHOH confirmed by surgery ,all patients were examined by plain and enhanced MSCT scan to make MPR and MIP with sub-millimeter data .Results All 7 cases showed sizes of a pure fat vascular sac above the diaphragmatic esophageal hiatus (EH) on a cross-sectional image ,among which 3 cases presented oval and 3 presented similar round in shape ,1 case showed lobulated ;Most of them located at the right front side of esophagus .On sagittal images ,the sac all displayed casting fat density shadow at postcordial ,and the morphology of the sac raged from half or triangle in the middle of the sagittal image to that of triangle or semicircle in the parasagittal planar ;In the coronal planar images ,the sac displayed oval in 3 cases ,pear-shaped upside-down in 1 case and lobulated in 1 case ,The bottom of the pure fat vascular sac that presented narrow neck shape connected with abdominal omental fat , and the diaphragmatic esophageal membrane that was shoved off in the upper polar of the pure fat vascular sac linked with the esophageal then formed an angle .The whole 7 cases of MIP images showed that the trunk of GLA was stiff on MIP , and it passed through EH with a symmetric "∩"near the esophagus branch .Gastro-esophageal connections were all loca-ted under the diaphragm .Conclusion The following findings are characteristically presents of SEHOH ,which are of great importance to imaging diagnosis of SEHOH :the expansablity ,morphological plasticity ,closed to the esophagus and con-nected to the abdominal omental of the pure fat vascular sac ,and stiff of the trunk of GLA ,and it passes through EH near the esophagus branch with a symmetric "∩".%目的:探讨单纯食管裂孔网膜疝(simple esophageal hiatus omental hernia ,SEHOH)的MSCT影像特点。方法回顾性分析7例手术证实的SEHOH ,均

  15. Characterization and comparison of adipose tissue-derived cells from human subcutaneous and omental adipose tissues.

    Science.gov (United States)

    Toyoda, Mito; Matsubara, Yoshinori; Lin, Konghua; Sugimachi, Keizou; Furue, Masutaka

    2009-10-01

    Different fat depots contribute differently to disease and function. These differences may be due to the regional variation in cell types and inherent properties of fat cell progenitors. To address the differences of cell types in the adipose tissue from different depots, the phenotypes of freshly isolated adipose tissue-derived cells (ATDCs) from subcutaneous (SC) and omental (OM) adipose tissues were compared using flow cytometry. Our results showed that CD31(-)CD34(+)CD45(-)CD90(-)CD105(-)CD146(+) population, containing vascular smooth muscle cells and pericytes, was specifically defined in the SC adipose tissue while no such population was observed in OM adipose tissue. On the other hand, CD31(-)CD34(+)CD45(-)CD90(-)CD105(-)CD146(-) population, which is an undefined cell population, were found solely in OM adipose tissue. Overall, the SC adipose tissue contained more ATDCs than OM adipose tissue, while OM adipose tissue contained more blood-derived cells. Regarding to the inherent properties of fat cell progenitors from the two depots, adipose-derived stem cells (ADSCs) from SC had higher capacity to differentiate into both adipogenic and osteogenic lineages than those from OM, regardless of that the proliferation rates of ADSCs from both depots were similar. The higher differentiation capacity of ADSCs from SC adipose tissue suggests that SC tissue is more suitable cell source for regenerative medicine than OM adipose tissue.

  16. Unique transcriptomic signature of omental adipose tissue in Ossabaw swine: a model of childhood obesity.

    Science.gov (United States)

    Toedebusch, Ryan G; Roberts, Michael D; Wells, Kevin D; Company, Joseph M; Kanosky, Kayla M; Padilla, Jaume; Jenkins, Nathan T; Perfield, James W; Ibdah, Jamal A; Booth, Frank W; Rector, R Scott

    2014-05-15

    To better understand the impact of childhood obesity on intra-abdominal adipose tissue phenotype, a complete transcriptomic analysis using deep RNA-sequencing (RNA-seq) was performed on omental adipose tissue (OMAT) obtained from lean and Western diet-induced obese juvenile Ossabaw swine. Obese animals had 88% greater body mass, 49% greater body fat content, and a 60% increase in OMAT adipocyte area (all P development, 2) cellular function and maintenance, and 3) connective tissue development and function, while transcripts associated with RNA posttranslational modification, lipid metabolism, and small molecule biochemistry were reduced. DAVID and Gene Ontology analyses showed that many of the classically recognized gene pathways associated with adipose tissue dysfunction in obese adults including hypoxia, inflammation, angiogenesis were not altered in OMAT in our model. The current study indicates that obesity in juvenile Ossabaw swine is characterized by increases in overall OMAT transcript number and provides novel data describing early transcriptomic alterations that occur in response to excess caloric intake in visceral adipose tissue in a pig model of childhood obesity.

  17. 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.

  18. Cystic local recurrence of renal cell carcinoma after laparoscopic radical nephrectomy in a hemodialysis patient.

    Science.gov (United States)

    Ito, Kazuyo; Takagi, Toshio; Kondo, Tsunenori; Yoshida, Kazuhiko; Iizuka, Junpei; Kobayashi, Hirohito; Tomita, Eri; Hashimoto, Yasunobu; Tanabe, Kazunari

    2014-03-01

    Although local recurrence of renal cell carcinoma after laparoscopic radical nephrectomy is sometimes reported, cystic local recurrence of renal cell carcinoma has rarely been reported. We report the case of a 59-year-old man with hemodialysis who developed cystic local recurrence of renal cell carcinoma accompanied by acquired cystic disease of the kidney in the retroperitoneal space after laparoscopic radical nephrectomy. A cystic tumor of 5.1 cm in diameter occurred in the left retroperitoneal space 15 months after left laparoscopic radical nephrectomy, and enlarged to 7.2 cm in diameter with enhanced mass along the wall of the cyst 36 months after surgery. The cystic tumor was removed and showed local recurrence of renal cell carcinoma on pathological examination.

  19. Next generation in vitro systems for biofilm studies - a cystic fibrosis patient airway perspective

    DEFF Research Database (Denmark)

    Weiss Nielsen, Martin

    Bacterial infections have a large impact on the health of the general public, however individuals with cystic fibrosis (CF) are immensely susceptible to acquire pulmonary infections with environmental bacteria, viruses and fungal species. Ultimately pulmonary infections are the major cause...... was to develop an accurate tool for growing biofilms that can mimic the cystic fibrosis airways, emulating one of the most important characteristics of the human airways, the oxygen environments. Microfluidic approaches that allow biofilm formation under controllable oxygen concentrations, and furthermore enable...

  20. Cystic Fibrosis: Diet and Nutrition

    Science.gov (United States)

    ... a Friend Who Cuts? Cystic Fibrosis: Diet and Nutrition KidsHealth > For Teens > Cystic Fibrosis: Diet and Nutrition Print A A A Text Size What's in ... or the flu. With the right balance of nutrition, extra fat and calories , and prescribed supplements, though, ...

  1. Cystic hemangioblastoma of the brainstem

    Directory of Open Access Journals (Sweden)

    Amit Agrawal

    2010-01-01

    Full Text Available Hemangioblastomas are very highly vascular neoplasm with benign characteristics and; in comparison to cerebellar hemangioblastoma; cases of cystic hemangioblastoma of the brain stem are rare with only a few case reports available in the literature. We report the case of a 43-year-old-female with cystic hemagioblastoma of the brainstem managed successfully and review the relevant literature.

  2. Cystic hemangioblastoma of the brainstem

    OpenAIRE

    Amit Agrawal; Anand Kakani; Vagh, Sunita J; Hiwale, Kishore M; Gaurav Kolte

    2010-01-01

    Hemangioblastomas are very highly vascular neoplasm with benign characteristics and; in comparison to cerebellar hemangioblastoma; cases of cystic hemangioblastoma of the brain stem are rare with only a few case reports available in the literature. We report the case of a 43-year-old-female with cystic hemagioblastoma of the brainstem managed successfully and review the relevant literature.

  3. Benign cystic peritoneal mesothelioma

    Directory of Open Access Journals (Sweden)

    Santhosh Shetty

    2014-04-01

    Full Text Available A well-defined but rare entity of Benign Cystic Peritoneal Mesothelioma (BCPM is reported. The aetiology of this neoplasm remains obscure. The presenting features make a precise preoperative diagnosis difficult but information provided by computed tomography and cytology may help. A firm diagnosis can only come from an electronic microscopy or immunohistological examination of the tumour. Diagnostic accuracy and diligent follow up are essential because, although the tumour is considered benign, it does tend towards local recurrence. [Int J Res Med Sci 2014; 2(2.000: 762-764

  4. Childhood abdominal cystic lymphangioma

    International Nuclear Information System (INIS)

    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. Conclusions: US

  5. 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

  6. Imaging from cystic fibrosis

    International Nuclear Information System (INIS)

    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.)

  7. Mature Cystic Renal Teratoma

    OpenAIRE

    Yavuz, Alpaslan; Ceken, Kagan; Alimoglu, Emel; Bahar AKKAYA

    2014-01-01

    Teratomas are rare germline tumors that originate from one or more embryonic germ cell layers. Teratoma of the kidney is extremely rare, and less than 30 cases of primary intrarenal teratomas have been published to date. We report the main radiologic features of an unusual case of mature cystic teratoma arising from the left kidney in a two-year-old boy. A left-sided abdominal mass was detected on physical examination and B-Mod Ultrasound (US) examination revealed a heterogeneous mass with ce...

  8. Hepatocellular Carcinoma with Blood Supply from Parasitized Omental Artery: Angiographic Appearance And Chemoembolization

    Institute of Scientific and Technical Information of China (English)

    Song Gao; Ren-jie Yang; Jia-hong Dong

    2012-01-01

    Objective:To analyze angiographic appearance of hepatocellular carcinoma (HCC) with blood supply from parasitized omental artery (POA),and evaluate the technical feasibility,safety and therapeutic efficacy of chemoembolization via the POAs.Methods:A total of 1,221 HCC patients who had undergone chemoembolization procedures were evaluated retrospectively.The evaluated indexes included the incidence rate of POAs,success rate of superselective catheterization,post-reaction after chemoembolization,and the cumulative survival rates.Results:Totally 1,221 HCC patients had undergone 3,639 chemoembolization procedures,and 32 patients with POAs were enrolled,with 97 POAs found in 76 angiography procedures,giving an incidence rate of 2.09%.POA was observed mostly at the right lobe and left medial lobe except the segment Ⅱ,and 62 POAs underwent superselective catheterization with microcatheter,giving a success rate of 63.9%.The angiographic appearance was:(1) hypertrophic POAs participating in tumor staining (n=28); (2) stiff and distorted POA (n=11),displaced due to tumor's oppression (n=8); and (3) defective tumor staining close to either gastrocolic omentum distribution or liver capsule (n=7).In 19 patients,chemoembolization via POAs was performed successfully (A group),while the remaining 13 patients failed (B group).Except 1 acute edema pancreatitis case,no serious complication was recorded.The cumulative survival rates of 6-,12-,18-and 24-month were 78.9%,47.4%,31.6% and 21.1% respectively for A group; correspondingly,61.5%,30.g%,15.4% and 7.7%% for B group,in which 2 patients died of ruptured HCC.Conclusion:Chemoembolization with microcatheter via POAs is a relatively safe,feasible and valuable method.

  9. Colgajo de omento para tratamiento de dehiscencia de herida esternal Omental flap for management of sternal wound dehiscence

    Directory of Open Access Journals (Sweden)

    A. Pérez García

    2013-03-01

    Full Text Available La mediastinitis es una de las complicaciones más graves de la cirugía cardíaca. Aparece en el 0,5-5 % de las esternotomías y esta incidencia es mucho mayor en pacientes diabéticos, obesos, inmunodeprimidos, con enfermedad pulmonar obstructiva crónica (EPOC, etc. Presentamos el caso de una paciente diabética, obesa mórbida, que sufre mediastinitis tras doble by-pass coronario. Para la cobertura del defecto se empleó un colgajo de epiplon tunelizado transdiafragmático. El colgajo omental permite cubrir grandes defectos además de que posee capacidad antimicrobiana y favorece la revascularización tisular. Las características de este colgajo hacen que actualmente deba considerarse como primera elección en pacientes con grandes defectos esternales y trastornos de la inmunidad y la cicatrización.Mediastinitis is one of the most serious complications from cardiac surgery. It is reported to occur in 0'5-5 % of sternotomy incisions, and this incidence is much higher in diabetic patients, obese, immunocompromised, COPD, etc. We report a case of a diabetic and morbid obese woman suffering mediastinitis after double coronary bypass surgery. For the reconstruction a transdiaphragmatic tunnelled omental flap was used. The omentum is useful to cover large defects. It has antiinfective properties and promotes revascularization of neighbouring tissues. Omental flap can be a highly effective treatment for patients with large sternal wounds and immune disorders and wound healing disorders.

  10. Segmental omental infarction in childhood: an unusual case of left-sided location with extension into the pelvis

    International Nuclear Information System (INIS)

    Segmental omental infarction (OI) is a rare cause of abdominal pain in children. It generally occurs in the right lower and upper quadrants of the abdomen and only a few cases of other locations have been described in adults. We report a unique paediatric case of OI with an unusual left-sided location extending into the pelvis in a 6-year-old non-obese girl. The diagnosis was suspected on US and CT based on imaging patterns previously described in adults and children. Laparoscopic resection should be balanced with nonsurgical management. (orig.)

  11. Effect of the CGRP receptor antagonist BIBN4096BS in human cerebral, coronary and omental arteries and in SK-N-MC cells

    DEFF Research Database (Denmark)

    Edvinsson, Lars; Alm, Rikard; Shaw, Duncan;

    2002-01-01

    , coronary, and omental arteries were studied with a sensitive myograph technique. CGRP induced a concentration-dependent relaxation that was antagonized by both CGRP-(8-37) and BIBN4096BS in a competitive manner. CGRP was a weaker agonist on coronary arteries as compared to intracranial arteries; however......, BIBN4096BS was an equally effective antagonist. In human omental arteries, CGRP did not induce relaxation. BIBN4096 had a pA(2) value of 10.1 in cerebral and 10.4 in coronary arteries. The results of clinical trials with BIBN4096BS for acute migraine attacks are awaited with great interest....

  12. Nutritional Issues in Cystic Fibrosis.

    Science.gov (United States)

    Solomon, Missale; Bozic, Molly; Mascarenhas, Maria R

    2016-03-01

    The importance of maintaining adequate nutrition in patients with cystic fibrosis has been well known for the past 3 decades. Achieving normal growth and maintaining optimal nutrition is associated with improved lung function. Comprehensive and consistent nutritional assessments at regular intervals can identify those at risk of nutritional failure and uncover micronutrient deficiencies contributing to malnutrition. Management of malnutrition in cystic fibrosis should follow a stepwise approach to determine the causes and comorbidities and to develop a nutritional plan. Nutritional management is crucial at every stage in a person's life with cystic fibrosis and remains a cornerstone of management.

  13. Risk analysis and outcome of mediastinal wound and deep mediastinal wound infections with specific emphasis to omental transposition

    LENUS (Irish Health Repository)

    Parissis, Haralabos

    2011-09-19

    Abstract Background To report our experience, with Deep mediastinal wound infections (DMWI). Emphasis was given to the management of deep infections with omental flaps Methods From February 2000 to October 2007, out of 3896 cardiac surgery patients (prospective data collection) 120 pts (3.02%) developed sternal wound infections. There were 104 males & 16 females; (73.7%) CABG, (13.5%) Valves & (9.32%) CABG and Valve. Results Superficial sternal wound infection detected in 68 patients (1.75%) and fifty-two patients (1.34%) developed DMWI. The incremental risk factors for development of DMWI were: Diabetes (OR = 3.62, CI = 1.2-10.98), Pre Op Creatinine > 200 μmol\\/l (OR = 3.33, CI = 1.14-9.7) and Prolong ventilation (OR = 4.16, CI = 1.73-9.98). Overall mortality for the DMWI was 9.3% and the specific mortality of the omental flap group was 8.3%. 19% of the "DMWI group", developed complications: hematoma 6%, partial flap loss 3.0%, wound dehiscence 5.3%. Mean Hospital Stay: 59 ± 21.5 days. Conclusion Post cardiac surgery sternal wound complications remain challenging. The role of multidisciplinary approach is fundamental, as is the importance of an aggressive early wound exploration especially for deep sternal infections.

  14. 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.

  15. [Rhinosinusitis in cystic fibrosis].

    Science.gov (United States)

    Mainz, J G; Gerber, A; Arnold, C; Baumann, J; Baumann, I; Koitschev, A

    2015-11-01

    In cystic fibrosis (CF) mucociliary clearance of the entire respiratory system is impaired. This allows pathogens, such as Pseudomonas aeruginosa to persist and proliferate, which by progressive pulmonary destruction causes 90 % of premature deaths due to this inherited disease. The dramatic improvement in life expectation of patients due to intensive therapy has resulted in the inevitable but variably expressed sinonasal involvement coming into the clinical and scientific focus. Thereby, almost all CF patients reveal sinonasal pathology and many suffer from chronic rhinosinusitis. Recently, the sinonasal niche has been recognized as a site of initial and persistent colonization by pathogens. This article presents the pathophysiological background of this multiorgan disease as well as general diagnostic and therapeutic standards. The focus of this article is on sinonasal involvement and conservative and surgical options for treatment. Prevention of pathogen acquisition is an essential issue in the otorhinolaryngological treatment of CF patients. PMID:26495450

  16. Cystic tumors of Pancreas

    International Nuclear Information System (INIS)

    Case material consists of five patients, four of them corresponding to cystic tumors of pancreas and one to a pseudocyst which, as literature tells us, is often difficult to differentiate from the former.There is a description of main clinical and image aspects and the conduct applied there to. This subject is in full process of development and new pathology grading is applied although the last word has yet to be said. It is very difficult to reach exact diagnosis in the pre and in the intra operative stage. As they tend to be malignant, surgical resection is advised, however without discarding enucleation.Whenever they are malignant prognosis in general is better than in the case of solid pancreatic cancer

  17. Cystic tumors of Pancreas

    International Nuclear Information System (INIS)

    Case material consist of five patients, four of them corresponding to cystic tumors of Pancreas and one to pseudocyst which, as literature tells us, is often difficult to differentiate from the former.There is a description of main clinical and image aspects and the conduct applied thereto. This subject is in full process of development and a new pathology grading is applied although the last word has yet to be said. It is very difficult to reach exact diagnosis in the pre-and in the intra-operative stage.As they tend to be malignant, surgical resection is advised, however without discarding enucleation.Whenever they are malignant prognosis in general is better than in the case of solid pancreatic cancers

  18. 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.

  19. Steady Advances Against Cystic Fibrosis

    Science.gov (United States)

    ... age 2, he grew up playing everything from football and lacrosse to ice hockey and golf. And ... Who's at Risk? Cystic fibrosis affects males and females from all racial and ethnic groups. It is ...

  20. Cystic fibrosis in premature infants

    OpenAIRE

    Lu, KD; Engmann, C.; Moya, F. (Francina); Muhlebach, M

    2011-01-01

    There are few reports of cystic fibrosis (CF) diagnosed in premature infants. We describe the clinical course of three patients, from our neonatal intensive care units, who were diagnosed with CF, and discuss the existing literature and treatment considerations.

  1. Nutritional management of cystic fibrosis.

    OpenAIRE

    A MacDonald

    1996-01-01

    Nutritional support is an integral part of the management of cystic fibrosis patients. It is arguably best provided by a qualified dietitian and nutritional care sister working in conjunction with the rest of the cystic fibrosis team. The patient's nutritional needs should be assessed, regularly reviewed, and nutritional treatment tailored to meet the changing clinical and psychosocial needs of the patient. Nutritional intervention is not without complications, and in particular attention to ...

  2. Cystic Lesions in Autoimmune Pancreatitis

    OpenAIRE

    Gompertz, Macarena; Morales, Claudia; Aldana, Hernán; Castillo, Jaime; Berger, Zoltán

    2015-01-01

    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 ...

  3. What Are the Signs and Symptoms of Cystic Fibrosis?

    Science.gov (United States)

    ... Twitter. What Are the Signs and Symptoms of Cystic Fibrosis? The signs and symptoms of cystic fibrosis (CF) ... respiratory, digestive, or reproductive systems of the body. Cystic Fibrosis Figure A shows the organs that cystic fibrosis ...

  4. Cepacia Syndrome in a Non-Cystic Fibrosis Patient.

    Science.gov (United States)

    Hauser, Naomi; Orsini, Jose

    2015-01-01

    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. PMID:26357579

  5. Pathologic review of cystic and cavitary lung diseases.

    Science.gov (United States)

    Kim, Na Rae; Han, Joungho

    2012-10-01

    Pulmonary cystic and cavitary lesions caused by diverse etiologies are commonly encountered in chest imaging. The terms "cyst" and "cavity" are used to describe air-filled regions in the center of a nodule or consolidation of the lung. To date, only radiologic aspects of these lesions have been addressed. The morphologies of pulmonary cystic and cavitary lesions exhibit a broad spectrum, ranging from benign to malignant pulmonary diseases of acquired or congenital origin, including variable infectious diseases. In this review, we summarized the differential diagnosis of pathological entities to provide pathologists and radiologists with an overview of the diseases most commonly associated with pulmonary cystic and cavitary lesions in adults and children. The results showed slightly different patterns in the distribution of the diseases in the two groups. The most common causes of cavitary lesions include malignancy and infection in adults, and congenital malformation in children. Therefore, identification of pathologic entities correlating with the radiologic findings, clinical course, and location of the lesion is important in the evaluation of cystic and cavitary lung lesions in order to avoid unnecessary surgical procedures or delayed treatment. PMID:23136566

  6. 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.

  7. Pancreatic cystic neoplasms: Review of current knowledge, diagnostic challenges, and management options

    Directory of Open Access Journals (Sweden)

    Tanima Jana

    2015-01-01

    Full Text Available Pancreatic cystic lesions are being detected with increasing frequency, largely due to advances in cross-sectional imaging. The most common neoplasms include serous cystadenomas, mucinous cystic neoplasms, intraductal papillary mucinous neoplasms, solid pseudopapillary neoplasms, and cystic pancreatic endocrine neoplasms. Computed tomography (CT, magnetic resonance imaging (MRI, and endoscopic ultrasound (EUS are currently used as imaging modalities. EUS-guided fine needle aspiration has proved to be a useful diagnostic tool, and enables an assessment of tumor markers, cytology, chemistries, and DNA analysis. Here, we review the current literature on pancreatic cystic neoplasms, including classification, diagnosis, treatment, and recommendations for surveillance. Data for this manuscript was acquired via searching the literature from inception to December 2014 on PubMed and Ovid MEDLINE.

  8. Cerebellar cystic hemangioblastoma and cystic astrocytoma : differentiation on MR imaging

    Energy Technology Data Exchange (ETDEWEB)

    Yu, In Kyu; Chang, Kee Hyun; Han, Moon Hee; Kim, In One; Yeon, Kyung Mo [Seoul National Univ. College of Medicine, Seoul (Korea, Republic of); Song, Chi Sung; Lee, Sang Hyung [Boramae Hospital, Seoul (Korea, Republic of)

    1996-08-01

    To determine differential points, if any, on MR imaging between cerebellar cystic hemangioblastoma and cystic astrocytoma. MR images of patients with sugically proven cerebellar cystic hemangioblastomas (n=12) and cystic astrocytomas (n=14) were retrospectively reviewed with regard to the following point: size, location and signal intensity of the tumor ; tumor margin; presence, size and location of the enhancing mural nodule; vascule signal voids, internal septations, enhancing fearure of the cyst wall, secondary findings (degree of peritumoral edema and presence of hydrocephalus ) and the patient's age. The significant (p<.05) differential points were vascular signal voids, which were the most important clue, as well as the presence of an enhancing mural nodule, tumor margin, enhancing featrure of the cyst wall and the patient's age. If the patient was an adult and presented an enhancing mural nodule with adjacent vascular signal voids and smooth tumor margin, then cysitc hemangioblastoma was suggested, while the presence of an irregular-margined thick enhancing cyst wall, mural nodule without adjacent vascular signal voids and pediatric age were suggestive of cystic astrocytoma. On MR imaging, there are certain significant differential points between these similar-appearing tumors and these would be useful for a more accurate diagnosis.

  9. 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.

  10. Isolated omental duplication cyst with respiratory epithelium & pancreatic glands: Case report & review of literature

    Directory of Open Access Journals (Sweden)

    Phuoc T. Nguyen

    2016-08-01

    Full Text Available Duplication cysts are uncommon congenital anomalies. They are usually in communication with or are attached to an adjacent segment of bowel. Rarely are they completely isolated from the gastrointestinal tract. To date, there have been 29 reported cases of non-communicating or isolated duplication cysts. Few contain respiratory epithelium and pancreatic glands. Patients may present with pain, an acute abdomen, bleeding or malignant degeneration. Differential diagnoses for an isolated cystic mass should include duplication cyst in the pediatric population. Recognition and awareness of these anomalies and their various presentations can aid in management. The unusual case of an isolated duplication cyst containing respiratory and pancreatic tissue, found within omentum, is presented with a review of the literature.

  11. Mucolytics in cystic fibrosis.

    Science.gov (United States)

    Henke, Markus O; Ratjen, Felix

    2007-03-01

    Mucus accumulation in the lower airways is a key feature of cystic fibrosis (CF) lung disease. The major component of mucus in CF is not mucin derived from mucus producing cells but rather pus that includes viscous material such as polymerized DNA derived from degraded neutrophils. This has important implications for mucolytic therapy aiming to improve mucus clearance from the airways, since degradation of mucin may not be a suitable treatment strategy. In addition, thinning of secretions may not always be beneficial, since it may negatively affect certain aspects of mucus transport such as cough clearance. While inhaled N-acetylcysteine has been used as a mucolytic drug in CF for decades, there is little evidence that it has any beneficial effect. Dornase alfa has been shown to reduce pulmonary exacerbations and improve lung function and is currently the only mucolytic agent with proven efficacy in CF. Newer agents targeting other components of CF mucus, such as filamentous actin, are currently in development. Ultimately, drugs that are mucokinetic, which preserve viscoelasticity, rather than mucolytic may prove to be beneficial for CF lung disease in the future. PMID:17419975

  12. Cystic Fibrosis (CF) Respiratory Screen: Sputum

    Science.gov (United States)

    ... Tropical Delight: Melon Smoothie Pregnant? Your Baby's Growth Cystic Fibrosis (CF) Respiratory Screen: Sputum KidsHealth > For Parents > Cystic Fibrosis (CF) Respiratory Screen: Sputum Print A A A ...

  13. Computed tomography of cystic pancreatic fibrosis

    International Nuclear Information System (INIS)

    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.)

  14. Mucinous Cystic Neoplasms of Pancreas

    Science.gov (United States)

    Naveed, Shah; Qari, Hasina; Banday, Tanveer; Altaf, Asma; Para, Mah

    2014-01-01

    The purpose of this study was to investigate the actual management of mucinous cystic neoplasm (MCN) of the pancreas. A systematic review was performed in December 2009 by consulting PubMed MEDLINE for publications and matching the key words “pancreatic mucinous cystic neoplasm”, “pancreatic mucinous cystic tumor”, “pancreatic mucinous cystic mass”, “pancreatic cyst” and “pancreatic cystic neoplasm” to identify English language articles describing the diagnosis and treatment of the MCN of the pancreas. In total, 16,322 references ranging from January 1969 to December 2009 were analyzed and 77 articles were identified. No articles published before 1996 were selected because MCNs were not previously considered to be a completely autonomous disease. Definition, epidemiology, anatomopathological findings, clinical presentation, preoperative evaluation, treatment and prognosis were reviewed. MCNs are pancreatic mucin-producing cysts with a distinctive ovarian-type stroma localized in the body-tail of the gland and occurring in middle-aged females. The majority of MCNs are slow growing and asymptomatic. The prevalence of invasive carcinoma varies between 6% and 55%. Preoperative diagnosis depends on a combination of clinical features, tumor markers, computed tomography (CT), magnetic resonance imaging, endoscopic ultrasound with cyst fluid analysis and positron emission tomography-CT. Surgery is indicated for all MCNs.

  15. 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.

  16. Cystic neoplasms of the pancreas

    International Nuclear Information System (INIS)

    Cystic neoplasms of pancreas are rare lesions. Following the Compagno-Oertel classification, we differenciate serous microcystic adenomas (SMA) from mucinous macrocystic adenomas/adenocarcinomas (MMA). The former are benign tumors with slow growth, composed by innumerable small and tiny cystic with centra calcifications, resulting in a ''honeycomb'' pattern. They have a mixed US structure while CT densitometric values reflect a mixture of connective tissue and proteinaceous fluid. Postcontrast enhancement is frequently seen. MMA are potential (adenoma) or frankly (adenocarcinoma) malignant tumors. They appears as moltilocular cystic masses containing septa and/or papillary bulgings, with thickened walls. Both US and CT demonstrate their predominantly cystic character, and the eventual presence of excrescences. WE report a series of 23 cases (6 SMA, 17 MMA) of cystic neoplasms of the pancreas studied during the past five years. A correct diagnosis of SMA was possible in all 6 cases, while MMA was correctly diagnosed in 17 out of 18 cases. There were no false negatives, and 1 falsa positive. All differential diagnoses are also discussed

  17. Serum pancreatic lipase activity in cystic fibrosis.

    OpenAIRE

    Junglee, D; Penketh, A; Katrak, A; Hodson, M.E.; Batten, J C; Dandona, P

    1983-01-01

    Patients with cystic fibrosis have been found to have abnormal serum concentrations of immunoreactive trypsin and abnormal activities of pancreatic isoamylase. A study was undertaken to discover whether activity of pancreatic lipase is also altered in cystic fibrosis. Serum from 23 patients with cystic fibrosis was assayed for immunoreactive trypsin and pancreatic lipase. Median serum pancreatic lipase activity was significantly lower in patients with cystic fibrosis than in controls, as was ...

  18. Using Cystic Fibrosis Therapies for Non-Cystic Fibrosis Bronchiectasis.

    Science.gov (United States)

    ElMaraachli, Wael; Conrad, Douglas J; Wang, Angela C C

    2016-03-01

    Non-cystic fibrosis bronchiectasis (NCFB) is an increasingly prevalent disease that places a significant burden on patients and health systems globally. Although many of the therapies used to treat NCFB were originally developed as cystic fibrosis (CF) therapies, not all of them have been demonstrated to be efficacious in NCFB and some may even be harmful. This article explores the evidence for which therapeutic strategies used to treat CF have been translated into the care of NCFB. The conclusion is that therapies for adult NCFB cannot be simply extrapolated from CF clinical trials, and in some instances, doing so may actually result in harm.

  19. A Case of Bilateral Cystic Partially Differentiated Nephroblastoma vs Cystic Wilms' Tumor: Highlighting a Diagnostic Dilemma.

    Science.gov (United States)

    Stout, Thomas E; Au, Jason K; Hicks, J M; Gargollo, Patricio C

    2016-06-01

    Cystic partially differentiated nephroblastoma (CPDN) is a rare multicystic renal tumor along the spectrum of cystic nephroma and cystic Wilms' tumor. There have only been two previously reported cases of bilateral CPDN in the literature. We present here a case of bilateral CPDN vs cystic Wilms' tumor treated with neoadjuvant and adjuvant chemotherapy in addition to a bilateral partial nephrectomy. We also review the relevant literature regarding CPDN in an effort to aid in diagnosis and management of these rare cystic renal tumors.

  20. Diagnostic Testing in Cystic Fibrosis.

    Science.gov (United States)

    Brewington, John; Clancy, J P

    2016-03-01

    Cystic Fibrosis (CF) is a rare, multisystem disease leading to significant morbidity and mortality. CF is caused by defects in the cystic fibrosis transmembrane conductance regulator protein (CFTR), a chloride and bicarbonate transporter. Early diagnosis and access to therapies provides benefits in nutrition, pulmonary health, and cognitive ability. Several screening and diagnostic tests are available to support a diagnosis. We discuss the characteristics of screening and diagnostic tests for CF and guideline-based algorithms using these tools to establish a diagnosis. We discuss classification and management of common "diagnostic dilemmas," including the CFTR-related metabolic syndrome and other CFTR-associated diseases. PMID:26857766

  1. 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...

  2. 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....

  3. Acquired Multiple Cysts of the Kidney in Neuroblastoma Survivors.

    Science.gov (United States)

    Moodalbail, Divya G; Apple, Leah Z; Meyers, Kevin E; Ginsberg, Jill P; Kaplan, Bernard S; Bellah, Richard

    2016-07-01

    Cystic kidney disease includes a wide range of hereditary, developmental, and acquired conditions of the kidneys. Some of the inherited causes of cystic kidney disease include autosomal dominant polycystic kidney diseases (caused by mutations in PKD1 or PKD2), autosomal recessive polycystic kidney disease, tuberous sclerosis complex, von Hippel-Lindau disease, oral-facial-digital syndrome type I, and Hadju-Cheney syndrome. Acquired cystic kidney disease has been reported in patients receiving long-term hemodialysis or peritoneal dialysis and in children after liver transplantation. Acute kidney injury can occur in patients with neuroblastoma, usually as a result of thrombotic microangiopathy associated with bone marrow transplantation. End-stage renal disease is described in long-term survivors. However, in this case report, we provide what is to our knowledge the first description of multiple kidney cysts in long-term survivors of stage IV neuroblastoma. None of the 7 patients we describe with neuroblastoma and multiple kidney cysts had a family history of autosomal dominant polycystic kidney disease. Also, all lacked stigmata of tuberous sclerosis complex, von Hippel-Lindau disease, or Hadju-Cheney syndrome. Two patients progressed to end-stage renal disease; in addition, one of them developed an oncocytoid renal cell carcinoma. PMID:27016049

  4. Cystic fibrosis from the gastroenterologist's perspective.

    Science.gov (United States)

    Ooi, Chee Y; Durie, Peter R

    2016-03-01

    Cystic fibrosis is a life-limiting, recessive disease caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. Increased survival outcomes and the multisystem nature of the disease, including the involvement of hepatobiliary and gastrointestinal tracts, now require the need for more extensive knowledge and expertise in cystic fibrosis among gastroenterologists. Manifestations are either a direct consequence of the primary defect in cystic fibrosis or a secondary complication of the disease or therapy. Adult patients with cystic fibrosis also have an increased risk of malignancy in the gastrointestinal and pancreatico-biliary tracts compared with the general population. Novel treatments that target the basic defects in the CFTR protein have emerged, but to date not much is known about their effects on the gastrointestinal and hepatobiliary systems. The introduction of such therapies has provided new opportunities for the application of intestinal endpoints in clinical trials and the understanding of underlying disease mechanisms that affect the gut in cystic fibrosis.

  5. Non-cystic fibrosis bronchiectasis.

    Science.gov (United States)

    Al Subie, Haya; Fitzgerald, Dominic A

    2012-05-01

    Non-cystic fibrosis (CF) bronchiectasis is said to be a declining problem in developed countries, although its prevalence in indigenous communities in Australia and New Zealand is among the highest reported in the world. Early childhood pneumonia and underlying conditions such as immunodeficiency and primary ciliary dyskinesia need to be considered in the aetiology. A high-resolution computerised tomography scan is the key investigation in children with a chronic wet cough in whom bronchiectasis is suspected. Regardless of the cause, the treatment of bronchiectasis is centred upon facilitating the clearance of airway secretions and the treatment of pulmonary exacerbations. This review aims to provide general paediatricians with an update on the presenting features, investigation and management of non-cystic fibrosis bronchiectasis. PMID:21040075

  6. Mesenteric cystic lymphangioma mimicking malignancy.

    Science.gov (United States)

    Hureibi, Khalid; Sunidar, Osama A

    2014-01-01

    Mesenteric cystic lymphangiomas are benign tumours arising from the mesentery, and have no known aetiology. Patients might be discovered incidentally to have asymptomatic mesenteric cysts, or they can present with symptoms such as pain, nausea and vomiting. A 27-year-old man presented with vague abdominal pain, loss of appetite, postprandial fullness and significant weight loss. There was no lymphadenopathy, and abdominal examination was unremarkable. CT showed a mesenteric mass and a diagnosis of abdominal lymphoma was suggested. There was no evidence of pulmonary tuberculosis on chest X-ray and the purified protein derivative test was negative. On laparotomy, a 5×9×7 cm sessile cyst containing thick white fluid and arising from the ileal mesentery was found and completely removed. Histopathology proved a diagnosis of mesenteric cystic lymphangioma. The patient made uneventful recovery, and was asymptomatic on clinical follow-up after 6 weeks. PMID:25178885

  7. [Cystic fibrosis and associated complications].

    Science.gov (United States)

    Schwarz, C; Staab, D

    2015-03-01

    Cystic fibrosis (CF) is an autosomal recessive inherited metabolic disease. The mutation is located on the long arm of chromosome 7. Due to a defect in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, chloride ion transport is reduced across the cell membrane. As a result, the disease can be described as an exocrinopathy. In all organs with exocrine glands, disorders occur in association with the defective chloride transport. The main impact of this defect is manifested in the lungs. Therefore, the most common cause of death is pulmonary disease with respiratory insufficiency due to recurrent infections. Unfortunately, a cure for the disease is still not available. However, new therapies that may affect the CFTR mutation more specifically give new hope for better therapeutic options in the future. The long-term goal of therapy is to develop a causal therapy for all six different mutation classes and thus for about 2000 mutations. PMID:25693903

  8. 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.

  9. MRI in mucoviscidosis (cystic fibrosis)

    International Nuclear Information System (INIS)

    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.)

  10. Pharmacogenetics of cystic fibrosis treatment.

    Science.gov (United States)

    Carter, Suzanne C; McKone, Edward F

    2016-08-01

    Cystic fibrosis (CF) is genetic autosomal recessive disease caused by reduced or absent function of CFTR protein. Treatments for patients with CF have primarily focused on the downstream end-organ consequences of defective CFTR. Since the discovery of the CFTR gene that causes CF in 1989 there have been tremendous advances in our understanding of the genetics and pathophysiology of CF. This has recently led to the development of new CFTR mutation-specific targeted therapies for select patients with CF. This review will discuss the characteristics of the CFTR gene, the CFTR mutations that cause CF and the new mutation specific pharmacological treatments including gene therapy that are contributing to the dawning of a new era in cystic fibrosis care. PMID:27490265

  11. Breakdown in Breathing: The Complexities of Cystic Fibrosis

    Science.gov (United States)

    ... Subscribe A Breakdown in Breathing The Complexities of Cystic Fibrosis Cystic fibrosis (CF) is an inherited illness that ravages the ... B. Guggino, a researcher with the Johns Hopkins Cystic Fibrosis Center. But in CF, “the mucus gets sticky, ...

  12. Association of adipocyte genes with ASP expression: a microarray analysis of subcutaneous and omental adipose tissue in morbidly obese subjects

    Directory of Open Access Journals (Sweden)

    Lu HuiLing

    2010-01-01

    Full Text Available Abstract Background Prevalence of obesity is increasing to pandemic proportions. However, obese subjects differ in insulin resistance, adipokine production and co-morbidities. Based on fasting plasma analysis, obese subjects were grouped as Low Acylation Stimulating protein (ASP and Triglyceride (TG (LAT vs High ASP and TG (HAT. Subcutaneous (SC and omental (OM adipose tissues (n = 21 were analysed by microarray, and biologic pathways in lipid metabolism and inflammation were specifically examined. Methods LAT and HAT groups were matched in age, obesity, insulin, and glucose, and had similar expression of insulin-related genes (InsR, IRS-1. ASP related genes tended to be increased in the HAT group and were correlated (factor B, adipsin, complement C3, p Results HAT adipose tissue demonstrated increased lipid related genes for storage (CD36, DGAT1, DGAT2, SCD1, FASN, and LPL, lipolysis (HSL, CES1, perilipin, fatty acid binding proteins (FABP1, FABP3 and adipocyte differentiation markers (CEBPα, CEBPβ, PPARγ. By contrast, oxidation related genes were decreased (AMPK, UCP1, CPT1, FABP7. HAT subjects had increased anti-inflammatory genes TGFB1, TIMP1, TIMP3, and TIMP4 while proinflammatory PIG7 and MMP2 were also significantly increased; all genes, p Conclusion Taken together, the profile of C5L2 receptor, ASP gene expression and metabolic factors in adipose tissue from morbidly obese HAT subjects suggests a compensatory response associated with the increased plasma ASP and TG.

  13. Cerebellar Cystic Hemangioblastoma: Case Report

    OpenAIRE

    AKDEMİR, İsmail; EROL, Fatih S.; TİFTİKÇİ, Murat; İÇKE, M. Uğur; ÇOBANOĞLU, Bengü

    2001-01-01

    Thirty-seven-year old woman was admitted to the hospital with the complaint of headache, nausea and vomiting. There were left sided nistagmus and papilledema on neurological examination. A cystic lesion with mural nodule enhancing contrast medium was found on computed tomography (CT). Acute hydrocephalia was characterized by dilatation of the forth, third, and lateral ventricles. The cyst was drained through left sided suboccipital craniectomy and the mural nodule was removed totally. The his...

  14. Finger clubbing in cystic fibrosis.

    OpenAIRE

    Pitts-Tucker, T J; Miller, M G; Littlewood, J M

    1986-01-01

    Finger clubbing was measured in 73 of 105 patients with cystic fibrosis undergoing full assessment. The sign correlated well with the chest x ray score and indices of pulmonary function and infection but not with weight, height, age, liver function, or degree of fat malabsorption. The presence of clubbing suggests appreciable pulmonary involvement. Most probably its progression indicates a deterioration in pulmonary state. In both instances increased efforts should be made to treat the infect...

  15. 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.

  16. Ceramide mediates lung fibrosis in cystic fibrosis

    OpenAIRE

    Ziobro, Regan; Henry, Brian; Edwards, Michael J.; Lentsch, Alex B.; Gulbins, Erich

    2013-01-01

    Fibrosis of the lung is one of the major clinical problems of cystic fibrosis and chronic obstructive pulmonary disease. However, the molecular mechanisms leading to pulmonary fibrosis are poorly characterized and require definition. Here, we demonstrate that chronic accumulation of ceramide in the lung contributes to the development of fibrosis in aged cystic fibrosis mice. Genetic or pharmacological normalization of ceramide in cystic fibrosis mice, which was achieved by heterozygosity of a...

  17. Sepsis Caused by Achromobacter Xylosoxidans in a Child with Cystic Fibrosis and Severe Lung Disease

    Science.gov (United States)

    Stobbelaar, Kim; Van Hoorenbeeck, Kim; Lequesne, Monique; De Dooy, Jozef; Ho, Erwin; Vlieghe, Erika; Ieven, Margaretha; Verhulst, Stijn

    2016-01-01

    Patient: Female, 10 Final Diagnosis: Sepsis Symptoms: Fever • hypotension • not tollerating enteral feeds • respiratory deterioration Medication: — Clinical Procedure: IV antibiotics • lungtransplantion Specialty: Pediatrics and Neonatology Objective: Unusual clinical course Background: Achromobacter xylosoxidans is an aerobic, motile, Gram-negative, opportunistic pathogen that can be responsible for various severe nosocomial and community-acquired infections. It has been found in immunocompromised patients and patients with several other underlying conditions, but the clinical role of this microorganism in cystic fibrosis is unclear. Case Report: We describe a case of septic shock caused by A. xylosoxidans in a 10-year-old child with cystic fibrosis and severe lung disease. Conclusions: As the prevalence of A. xylosoxidans in cystic fibrosis patients is rising and patient-to-patient transmission is highly probable, further studies are warranted to determine its role and to document the appropriate treatment strategy for eradication and long-term treatment of this organism. PMID:27498677

  18. Sepsis Caused by Achromobacter Xylosoxidans in a Child with Cystic Fibrosis and Severe Lung Disease.

    Science.gov (United States)

    Stobbelaar, Kim; Van Hoorenbeeck, Kim; Lequesne, Monique; De Dooy, Jozef; Ho, Erwin; Vlieghe, Erika; Ieven, Margaretha; Verhulst, Stijn

    2016-01-01

    BACKGROUND Achromobacter xylosoxidans is an aerobic, motile, Gram-negative, opportunistic pathogen that can be responsible for various severe nosocomial and community-acquired infections. It has been found in immunocompromised patients and patients with several other underlying conditions, but the clinical role of this microorganism in cystic fibrosis is unclear. CASE REPORT We describe a case of septic shock caused by A. xylosoxidans in a 10-year-old child with cystic fibrosis and severe lung disease. CONCLUSIONS As the prevalence of A. xylosoxidans in cystic fibrosis patients is rising and patient-to-patient transmission is highly probable, further studies are warranted to determine its role and to document the appropriate treatment strategy for eradication and long-term treatment of this organism. PMID:27498677

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

    Energy Technology Data Exchange (ETDEWEB)

    Cho, Jeong Yeon; Song, Mi Jin; Lee, Young Ho; Cho, Byung Jae; Hong, Sung Ran [Samsung Cheil Hospital, Sungkyunkwan University School of Medicine, Seoul (Korea, Republic of)

    2002-03-15

    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.

  20. Hospital-acquired pneumonia

    Science.gov (United States)

    ... tends to be more serious than other lung infections because: People in the hospital are often very sick and cannot fight off ... prevent pneumonia. Most hospitals have programs to prevent hospital-acquired infections.

  1. Acquired color vision deficiency.

    Science.gov (United States)

    Simunovic, Matthew P

    2016-01-01

    Acquired color vision deficiency occurs as the result of ocular, neurologic, or systemic disease. A wide array of conditions may affect color vision, ranging from diseases of the ocular media through to pathology of the visual cortex. Traditionally, acquired color vision deficiency is considered a separate entity from congenital color vision deficiency, although emerging clinical and molecular genetic data would suggest a degree of overlap. We review the pathophysiology of acquired color vision deficiency, the data on its prevalence, theories for the preponderance of acquired S-mechanism (or tritan) deficiency, and discuss tests of color vision. We also briefly review the types of color vision deficiencies encountered in ocular disease, with an emphasis placed on larger or more detailed clinical investigations.

  2. Laboratory-acquired brucellosis

    DEFF Research Database (Denmark)

    Fabiansen, C.; Knudsen, J.D.; Lebech, A.M.

    2008-01-01

    Brucellosis is a rare disease in Denmark. We describe one case of laboratory-acquired brucellosis from an index patient to a laboratory technician following exposure to an infected blood culture in a clinical microbiology laboratory Udgivelsesdato: 2008/6/9......Brucellosis is a rare disease in Denmark. We describe one case of laboratory-acquired brucellosis from an index patient to a laboratory technician following exposure to an infected blood culture in a clinical microbiology laboratory Udgivelsesdato: 2008/6/9...

  3. 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...

  4. Cystic pulmonary chondroid hamartoma: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Yoo, Eun Young; Lee, Kyung Soo; Han, Jeong Ho; Kim, Yoon Kyung [Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul (Korea, Republic of)

    2008-06-15

    Hamartomata is the most common benign type of tumor, occurring in the lung. However, a cystic pulmonary hamartoma is extremely rare, and is difficult to diagnose due to its nonspecific nature. We report a case of cystic pulmonary hamartoma in which a popcorn-like calcification is clearly identified, thus enabling a confident diagnosis of the disease.

  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. 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.

  7. 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.

  8. 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...

  9. Sarcoidosis in an adult with cystic fibrosis.

    OpenAIRE

    Rettinger, S D; Trulock, E. P.; MacKay, B.; Auerbach, H S

    1989-01-01

    Sarcoidosis in an adult patient with cystic fibrosis lung disease was diagnosed on the basis of pulmonary function and radiographic data. It should be considered in the differential diagnosis of new diffuse interstitial infiltrates or hilar adenopathy in a patient with cystic fibrosis; biopsy of lung, lymph node, or skin lesions and interleukin-2 receptor levels may help to obtain a diagnosis.

  10. Cystic pulmonary chondroid hamartoma: a case report

    International Nuclear Information System (INIS)

    Hamartomata is the most common benign type of tumor, occurring in the lung. However, a cystic pulmonary hamartoma is extremely rare, and is difficult to diagnose due to its nonspecific nature. We report a case of cystic pulmonary hamartoma in which a popcorn-like calcification is clearly identified, thus enabling a confident diagnosis of the disease

  11. HOME CARE IN CYSTIC-FIBROSIS PATIENTS

    NARCIS (Netherlands)

    VANAALDEREN, WMC; MANNES, GPM; BOSMA, ES; ROORDA, RJ; HEYMANS, HSA

    1995-01-01

    Intravenous antibiotics and enteral tube feeding at home for the treatment of pulmonary exacerbations and underweight condition in cystic fibrosis (CF) patients have become tools that are used in many cystic fibrosis centres, The experience with home care programmes from different countries is quite

  12. Muscular cystic hydatidosis: case report

    Directory of Open Access Journals (Sweden)

    Naspetti Riccardo

    2007-03-01

    Full Text Available Abstract Background Hydatidosis is a zoonosis caused by Echinococcus granulosus, and ingesting eggs released through the faeces from infected dogs infects humans. The location of the hydatid cysts is mostly hepatic and/or pulmonary, whereas musculoskeletal hydatidosis is very rare. Case presentation We report an unusual case of primary muscular hydatidosis in proximity of the big adductor in a young Sicilian man. The patient, 34 years old, was admitted to the Department of Infectious and Tropical Diseases for ultrasonographic detection, with successive confirmation by magnetic resonance imaging, of an ovular mass (13 × 8 cm in the big adductor of the left thigh, cyst-like, and containing several small cystic formations. Serological tests for hydatidosis gave negative results. A second drawing of blood was done 10 days after the first one and showed an increase in the antibody titer for hydatidosis. The patient was submitted to surgical excision of the lesion with perioperatory prophylaxis with albendazole. The histopathological examination of the bioptic material was not diriment in the diagnosis, therefore further tests were performed: additional serological tests for hydatidosis for the evaluation of IgE and IgG serotype (Western Blot and REAST, and molecular analysis of the excised material. These more specific serological tests gave positive results for hydatidosis, and the sequencing of the polymerase chain reaction products from the cyst evidenced E. granulosus DNA, genotype G1. Any post-surgery complications was observed during 6 following months. Conclusion Cystic hydatidosis should always be considered in the differential diagnosis of any cystic mass, regardless of its location, also in epidemiological contests less suggestive of the disease. The diagnosis should be achieved by taking into consideration the clinical aspects, the epidemiology of the disease, the imaging and immunological tests but, as demonstrated in this case, without

  13. Influence of obesity and insulin sensitivity on insulin signaling genes in human omental and subcutaneous adipose tissue.

    Science.gov (United States)

    MacLaren, R; Cui, W; Simard, S; Cianflone, K

    2008-02-01

    Obesity and insulin resistance are independent risk factors for metabolic syndrome, diabetes, and cardiovascular disease. Adipose tissue samples from nonobese (NO), insulin-sensitive obese (ISO), and insulin-resistant obese (IRO) subjects from subcutaneous (SC) and omental (OM) adipose tissue (n = 28) were analyzed by microarray and confirmed by real-time PCR. Insulin signaling gene expression changes were greater in OM than in SC tissue and were related to insulin resistance rather than to obesity; few genes correlated with body mass index. Insulin receptor and insulin receptor substrate 1 (IRS-1) increased in the IRO versus pooled insulin-sensitive (NO+ISO) subjects. In glucose transport, PI3Kalpha and PDK2 decreased in IRO subjects, whereas PI3Kgamma, Akt2, GLUT4, and GLUT1 increased. IRS-1 regulators Jnk and IKK increased in IRO (P < 0.01 and P < 0.001 respectively). In protein synthesis, most genes examined were downregulated in IRO subjects, including mTor, Rheb, and 4EBP and eIF members (all P < 0.05). In proliferation, SHC, SOS, and Raf1 (P < 0.05) were increased, whereas Ras and MEK1/2 kinase 1 (P < 0.05) were decreased, in IRO subjects. Finally, in differentiation, PPARgamma, CEBPalpha, and CEBPbeta decreased, whereas PPARdelta, CEBPgamma, and CEBPepsilon increased, in IRO subjects (P < 0.05). Together, microarray and real-time PCR data demonstrate that insulin resistance rather than obesity is associated with altered gene expression of insulin signaling genes, especially in OM adipose tissue. PMID:17986714

  14. A malignant omental extra-gastrointestinal stromal tumor on a young man: a case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Almaroof Babatunde

    2008-05-01

    Full Text Available Abstract Background Gastrointestinal stromal tumors (GIST are uncommon intra-abdominal tumors. These tumors tend to present with higher frequency in the stomach and small bowel. In fewer than 5% of cases, they originate primarily from the mesentery, omentum, or peritoneum. Furthermore, these extra-gastrointestinal tumors (EGIST tend to be more common in patients greater than 50 years of age. Rarely do EGIST tumors present in those younger than 40 years of age. Case presentation We report a case of a large EGIST in a 27-year-old male. An abdominal pelvic computerized tomography imaging demonstrated an intra-abdominal mass of 22 cm, without invasion of adjacent viscera or liver lesions. This mass was resected en bloc with its fused omentum and an adherent portion of sigmoid colon. Pathology results demonstrated a malignant gastrointestinal stromal tumor with positive CD117 (c-kit staining, and negative margins of resection, and no continuity of tumor with the sigmoid colon. Due to the malignant and aggressive nature of this patient's tumor, he was started on STI-571 as adjuvant chemotherapy. Conclusion Stromal tumors of an extra-gastrointestinal origin are rare. Of the reported omental and mesenteric EGISTs in four published series, a total of 99 tumors were studied. Of the 99 patients in these series only 8 were under 40 years of age, none were younger than 30 years old; and only 5 were younger than 35 years old. Our patient's age is at the lower end of the age spectrum for the reported EGISTs. Young patients who present with an extra-gastrointestinal stromal tumor (EGIST, who have complete resection with negative margins, have a good prognosis. There is little data to support the role of STI-571 in adjuvant or neoadjuvant therapy after curative resection. Given the lack of data, the use of STI-571 must be individualized.

  15. Opposing Effects of Omega-3 and Omega-6 Long Chain Polyunsaturated Fatty Acids on the Expression of Lipogenic Genes in Omental and Retroperitoneal Adipose Depots in the Rat

    Directory of Open Access Journals (Sweden)

    B. S. Muhlhausler

    2010-01-01

    Full Text Available This study aimed to determine the effect of varying dietary intake of the major n-3 PUFA in human diets, α-linolenic acid (ALA; 18 : 3n-3, on expression of lipogenic genes in adipose tissue. Rats were fed diets containing from 0.095%en to 6.3%en ALA and a constant n-6 PUFA level for 3 weeks. Samples from distinct adipose depots (omental and retroperitoneal were collected and mRNA expression of the pro-lipogenic transcription factors Sterol-Retinoid-Element-Binding-Protein1c (SREBP1c and Peroxisome Proliferator Activated Receptor-γ (PPARγ, lipogenic enzymes Sterol-coenzyme Desaturase1 (SCD-1, Fatty Acid Synthase (FAS, lipoprotein lipase (LPL and glycerol-3-phosphate dehydrogenase (G3PDH and adipokines leptin and adiponectin determined by qRT-PCR. Increasing dietary ALA content resulted in altered expression of SREBP1c, FAS and G3PDH mRNA in both adipose depots. SREBP1c mRNA expression was related directly to n-6 PUFA concentrations (omental, r2=.71; P<.001; Retroperitoneal, r2=.20; P<.002, and inversely to n-3 PUFA concentrations (omental, r2=.59; P<.001; Retroperitoneal, r2=.19; P<.005 independent of diet. The relationship between total n-6 PUFA and SREBP1c mRNA expression persisted when the effects of n-3 PUFA were controlled for. Altering red blood cell concentrations of n-3 PUFA is thus associated with altered expression of lipogenic genes in a depot-specific manner and this effect is modulated by prevailing n-6 PUFA concentrations.

  16. Pseudomonas aeruginosa colonization in patients with cystic fibrosis; population structure, the Dutch clone and effects of segregation

    NARCIS (Netherlands)

    Mansfeld, R. van

    2014-01-01

    More than half of the patients with cystic fibrosis (CF) are infected with Pseudomonas aeruginosa in their lungs. It was thought that patients acquired unique strains of this bacterium from the environment, however in the last decades epidemic clones have been described in other countries that are m

  17. Five cases of cystic osteoechinococcosis

    Institute of Scientific and Technical Information of China (English)

    JIANG Ci-peng

    2005-01-01

    @@ Osseous cystic echinococcosis (CE) is a rare disease caused by the larval stage of Echinococcus granulosus (E. granulosus) and CE is different from alveolar echinococcosis (AE) caused by the larva of E. multilocularis. During a period of 20 years (1983-2003), a total of 364 cases of echinococcosis, including 263 CE and 101 AE cases were diagnosed at Hydatidos Research Laboratory (HRL), Basic Medical College, Lanzhou University, China. Of them, 5 cases of osseous CE (1.37%) were encountered and now reported as follows.

  18. Pulmonary complications of cystic fibrosis

    International Nuclear Information System (INIS)

    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

  19. Liver manifestations of cystic fibrosis

    International Nuclear Information System (INIS)

    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

  20. Endocrine Disorders in Cystic Fibrosis.

    Science.gov (United States)

    Blackman, Scott M; Tangpricha, Vin

    2016-08-01

    Cystic fibrosis is frequently complicated by endocrine disorders. Diabetes can be expected to affect most with CF and pancreatic insufficiency and varies widely in age of onset, but early identification and treatment improve morbidity and mortality. Short stature can be exacerbated by relative delay of puberty and by use of inhaled corticosteroids. Bone disease in CF causes fragility fractures and should be assessed by monitoring bone mineral density and optimizing vitamin D status. Detecting and managing endocrine complications in CF can reduce morbidity and mortality in CF. These complications can be expected to become more common as the CF population ages. PMID:27469183

  1. Cystic Lesions of the Mediastinum.

    Science.gov (United States)

    Vargas, Daniel; Suby-Long, Thomas; Restrepo, Carlos S

    2016-06-01

    Cystic lesions are commonly seen in the mediastinum, and they may arise from virtually any organ. The vast majority of these lesions are benign and result in no symptoms. When large, cysts may produce symptoms related to compression of adjacent structures. The most common mediastinal cysts are pericardial and foregut duplication cysts. Both computed tomography and magnetic resonance are routinely used to evaluate these lesions. Although computed tomography offers superior spatial resolution, magnetic resonance is useful in differentiating cysts that contain proteinaceous material from solid lesions. Occasionally, cysts arise from solid lesions, such as thymoma or teratoma. Although cysts are alike in appearance, location helps narrowing the differential diagnoses.

  2. The Irish cystic fibrosis database.

    OpenAIRE

    Cashman, S M; Patino, A; Delgado, M G; Byrne, L; Denham, B; de Arce, M

    1995-01-01

    We have found records of 1014 Irish cystic fibrosis patients alive by December 1994, belonging to 883 families. Prevalence in the population is 1/3475 and incidence at birth 1/1461, with a gene frequency of 2.6%. Twenty percent of the patients are aged over 20 years, but at present survival rate falls rapidly after that age. We have identified 85% of the mutations on the CFTR gene in a sample of 29% of the families (506 CF chromosomes). Mutation delta F508 is found in 72% of Irish CF chromoso...

  3. Etiopathology of acquired cholesteatoma

    Directory of Open Access Journals (Sweden)

    Prabodh Karnik

    2011-01-01

    Full Text Available The etiopathology of acquired cholesteatoma has undergone numerous changes over the past 150 years. However, certain facts stand out with clarity. The presence of cytokeratins in acquired cholesteatoma, which are akin to those found in the tympanic membrane and external auditory canal, shows that these are probably the site of origin of acquired cholesteatoma. The cholesteatoma sac also shows its greatest growth at its tympanic membrane attachment into the middle ear. Implantations of squamous epithelium due to trauma or surgery could be another originating factor. The basic pathology is the formation of papillary cones from the tympanic membrane or external auditory canal, which progress from microcholesteatoma to frank cholesteatoma with keratin collections. There is an altered matrix metalloproteinase pathway. Tumor necrosis factor activation with altered wound healing process contributes to the collateral destruction of bone. Trisomy and aneuploidy of chromosome 8 predispose to cholesteatoma formation in affected individuals. In this article, we present the etiopathology of acquired cholesteatoma as it stands today.

  4. "Ready to Acquire"

    DEFF Research Database (Denmark)

    Yetton, Philip; Henningsson, Stefan; Bjørn-Andersen, Niels

    2013-01-01

    This article describes the experiences of Danisco (a global food ingredients company) as it followed a growth-by-acquisition business strategy, focusing on how a new CIO built the IT resources to ensure the IT organization was "ready to acquire." We illustrate how these IT capabilities expedited...

  5. Cystic lymphangioma of the pancreas

    Institute of Scientific and Technical Information of China (English)

    Radoje B Colovic; Nikica M Grubor; Marjan T Micev; Henry Dushan E Atkinson; Vitomir I Rankovic; Mihajlo M Jagodic

    2008-01-01

    Lymphangioma of the pancreas is an extremely rare benign turnout of lymphatic origin, with fewer than 60 published cases. Histologically, it is polycystic, with the cysts separated by thin septa and lined with endothelial cells. Though congenital, it can affect all age groups, and occurs more frequently in females. Patients usually present with epigastric pain and an associated palpable mass. Complete excision is curative, even though, depending on the tumour location, surgery may be simple or involve extensive pancreatic resection and anastomoses. The authors present a 49-year-old woman in whom a polycystic septated mass, 35 mm × 35 mm in size, was discovered by ultrasonography (US) in the body of the pancreas during investigations for epigastric pain and nausea. At surgery, a well circumscribed polycystic tumor was completely excised, with preservation of the pancreatic duct. The postoperative recovery was uneventful. Histology confirmed a microcystic lymphangioma of the pancreas. Immunohistochemistry showed cystic endothelial cells reactivity to factor Ⅷ -RA (++), CD31 (+++) and CD34 (-). Postoperatively, abdominal pain disappeared and the patient remained symptomfree for 12 mo until now. Although extremely rare, lymphangioma of the pancreas should be taken into consideration as a differential diagnosis of a pancreatic cystic lesion, especially in women.

  6. Aspergillus infections in cystic fibrosis.

    Science.gov (United States)

    King, Jill; Brunel, Shan F; Warris, Adilia

    2016-07-01

    Patients with cystic fibrosis (CF) suffer from chronic lung infection and airway inflammation. Respiratory failure secondary to chronic or recurrent infection remains the commonest cause of death and accounts for over 90% of mortality. Bacteria as Staphylococcus aureus, Pseudomonas aeruginosa and Burkholderia cepacia complex have been regarded the main CF pathogens and their role in progressive lung decline has been studied extensively. Little attention has been paid to the role of Aspergillus spp. and other filamentous fungi in the pathogenesis of non-ABPA (allergic bronchopulmonary aspergillosis) respiratory disease in CF, despite their frequent recovery in respiratory samples. It has become more apparent however, that Aspergillus spp. may play an important role in chronic lung disease in CF. Research delineating the underlying mechanisms of Aspergillus persistence and infection in the CF lung and its link to lung deterioration is lacking. This review summarizes the Aspergillus disease phenotypes observed in CF, discusses the role of CFTR (cystic fibrosis transmembrane conductance regulator)-protein in innate immune responses and new treatment modalities. PMID:27177733

  7. Case Studies of the Spatial Heterogeneity of DNA Viruses in the Cystic Fibrosis Lung

    OpenAIRE

    Willner, Dana; Haynes, Matthew R.; Furlan, Mike; Hanson, Nicole; Kirby, Breeann; Lim, Yan Wei; Rainey, Paul B.; Schmieder, Robert; Youle, Merry; Conrad, Douglas; Rohwer, Forest

    2012-01-01

    Microbial communities in the lungs of patients with cystic fibrosis (CF) and chronic obstructive pulmonary disease (COPD) have been shown to be spatially heterogeneous. Viral communities may also vary spatially, leading to localized viral populations and infections. Here, we characterized viral communities from multiple areas of the lungs of two patients with late-stage CF using metagenomics, that is, the explanted lungs from a transplant patient and lungs acquired postmortem. All regions har...

  8. SONOGRAPHIC PATTERNS AND DIFFERENTIAL DIAGNOSIS OF CYSTIC RENAL CARCINOMAS

    Institute of Scientific and Technical Information of China (English)

    蔡胜; 李建初; 等

    2002-01-01

    Objective:To study the sonographic features and patterns of cystic renal carcinomas.Methods:Thirteen cases of cystic renal carcinoma confirmed by operation and pathology were examined by ultrasonography,and the cystic walls,septa and solid mural nodules were studied.Results:Solid mural nodules of some cases and irregular thickening of the cystic walls and septa were characteristic findings for the ultrasonic diagnosis of cystic renal carcinomas.According to their pathologic mechanisms and sonographic features,cystic renal carcinomas were classified into 3 patterns:unilocular cystic mass,multiloculated cystic mass and cystic-solid mass.Conclusions:Typical cystic renal carcinomas can be well diagnosed,while atypical cases may be misdiagnosed as benign renal cysts by ultrasonography.Color Doppler ultrasonography and needle aspiration guided by ultrasonography are helpful in the diagnosis of these atypical cases.

  9. SONOGRAPHIC PATTERNS AND DIFFERENTIAL DIAGNOSIS OF CYSTIC RENAL CARCINOMAS

    Institute of Scientific and Technical Information of China (English)

    蔡胜; 李建初; 姜玉新; 戴晴; 谭莉; 张缙熙

    2002-01-01

    Objective. To study the sonographic features and patterns of cystic renal carcinomas. Methods. Thirteen cases of cystic renal carcinoma confirmed by operation and pathology were examined by ultrasonography, and the cystic walls, septa and solid mural nodules were studied. Results. Solid mural nodules of some cases and irregular thickening of the cystic walls and septa were characteristic findings for the ultrasonic diagnosis of cystic renal carcinomas. According to their pathologic mechanisms and sonographic features, cystic renal carcinomas were classified into 3 patterns: unilocular cystic mass, multiloculated cystic mass and cystic-solid mass. Conclusions. Typical cystic renal carcinomas can be well diagnosed, while atypical cases may be misdiagnosed as benign renal cysts by ultrasonography. Color Doppler ultrasonography and needle aspiration guided by ultrasonography are helpful in the diagnosis of these atypical cases.

  10. 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...

  11. 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.

  12. Mediastinal Cystic Lymphangioma in a Patient with Situs Inversus Totalis

    Directory of Open Access Journals (Sweden)

    Teruya Komatsu

    2014-01-01

    Full Text Available We present a case of cystic lymphangioma of the mediastinum complicated with situs inversus totalis. The 70-year-old man underwent thoracoscopic resection of a mediastinal cystic tumor, which was diagnosed as cystic lymphangioma. Cystic lymphangiomas are congenital cystic abnormalities of the lymphatic system. The head and neck area is often involved while the mediastinum is rarely affected. The rarity of this case is further attributed to the coexistence of situs inversus totalis.

  13. Artery to Cystic Duct: A Consistent Branch of Cystic Artery Seen in Laparoscopic Cholecystectomy

    Directory of Open Access Journals (Sweden)

    Arshad Rashid

    2015-01-01

    Full Text Available Uncontrolled arterial bleeding during laparoscopic cholecystectomy is a serious problem and may increase the risk of bile duct damage. Therefore, accurate identification of the anatomy of the cystic artery is very important. Cystic artery is notoriously known to have a highly variable branching pattern. We reviewed the anatomy of the cystic artery and its branch to cystic duct as seen through the video laparoscope. A single artery to cystic duct with the classical “H-configuration” was demonstrated in 161 (91.47% patients. This branch may cause troublesome bleeding during laparoscopic dissection in the hepatobiliary triangle. Careful identification of artery to cystic duct is helpful in the proper dissection of Calot’s triangle as it reduces the chances of hemorrhage and thus may also be helpful in prevention of extrahepatic biliary radical injuries.

  14. AIDS: acquired immunodeficiency syndrome

    OpenAIRE

    Gilmore, N. J.; Beaulieu, R.; Steben, M.; Laverdière, M.

    2002-01-01

    Acquired immunodeficiency syndrome, or AIDS, is a new illness that occurs in previously healthy individuals. It is characterized by immunodeficiency, opportunistic infections and unusual malignant diseases. Life-threatening single or multiple infections with viruses, mycobacteria, fungi or protozoa are common. A rare neoplasm, Kaposi's sarcoma, has developed in approximately one third of patients with AIDS. More than 800 cases of AIDS have been reported in North America, over 24 of them in Ca...

  15. Acquired hypertrichosis lanuginosa

    Directory of Open Access Journals (Sweden)

    Kumar Pramod

    1993-01-01

    Full Text Available Acquired hypertirichosis lanuginose developed rapidly in a patient with no detectable malignancy. Soft, fine, downy hair growth was noticed on the face, ears, limbs and trunk. Bilaterally symmetrical vitiliginous macules were present on the ear and preauricular region. This case is reported because of its rarity, absence of any detectable malignancy and development of vitiligo, which to our knowledge has not been reported earlier.

  16. DIFFERENTIAL EXPRESSION OF GENES IN OMENTAL FAT OF NORMAL WEIGHT AND OBESE SUBJECTS AND OBESE DIABETIC PATIENTS USING cDNA MICROARRAY

    Institute of Scientific and Technical Information of China (English)

    LUO Tian-hong; ZHENG Pei-zheng; ZHAO Chun-jun; ZHAO Yu; LI Guo; ZHANG Hong-li; LI Wen-yi; LIU You-ping; LUO Min; WANG Kan-kan; ZHANG Ji

    2007-01-01

    Objective To identify genes differentially expressed in omental fat of normal weight subjects,obese subjects and obese diabetic patients. Methods Using a high-density cDNA microarray, gene expression profile of omental fat from normal weigh subjects, obese subjects and obese diabetic patients were compared.Results Totally, 119 and 257 genes were up-regulated in obese subjects and obese diabetic patients respectively,while 46 and 58 genes were down-regulated. A total of 77 genes, including PDK4, which switched from carbohydrate to fatty acids as the primary source of fuel, were up-regulated in both obese and obese diabetic patients, while 8 genes, including key enzymes in lipid synthesis, such as HMG-CoA synthase, fatty acid synthase and stearoyl-CoA desaturase, were down-regulated in both groups. Tyrosine-3-monooxygenase/tryptophan 5-monooxygenase activation protein θ ( YWHAZ) , a negative regulator for insulin signal transduction, was up-regulated only in obese diabetic patient, but not in normal-glycemic obese subjects. Conclusion The study demonstrated that decrease of lipogenesis along with increase of fatty acids oxidation of adipose tissue could be a common cause of insulin resistance in obesity and type 2 diabetes, while block of insulin signal transduction may trigger the transition from obesity to diabetes. Further exploration of these genes will be useful in the understanding of the pathogenesis of obesity and diabetes.

  17. Association of SelS mRNA expression in omental adipose tissue with Homa-IR and serum amyloid A in patients with type 2 diabetes mellitus

    Institute of Scientific and Technical Information of China (English)

    DU Jian-ling; SUN Chang-kai; L(U) Bo; MEN Li-li; YAO Jun-jie; AN Li-jia; SONG Gui-rong

    2008-01-01

    Background Tanis was repoded as a putative receptor for serum amyloid A(SAA)involving glucose regulated protein in insulin regulated resistance.It was found to be dysregulated in diabetic rats(Psammomys obesus,Israeli sand rat)and its homologue for humans is SelS/AD-015.The present study analyzed mRNA expression of SelS in omental adipose tissue biopsies from patients with type 2 diabetes mellitus (T2DM),and age-and weight-matched nondiabetic patients,the relationship of SelS mRNA with Homa-IR and serum SAA level.Methods Human omental adipose tissues from ten cases of type 2 diabetic patients and twelve cases of nondiabetic individuals were analyzed for the expression level of SelS mRNA by semiquantitative polymerase chain reaction(PCR),Homa-IR estimated by standard formula and SM level by enzyme-linked immunosorbent assay(ELISA).Results SelS mRNA expression.Homa-IR and serum SAA were higher in T2DM sufferers than in nondiabeUc control group.SelS mRNA level was positively correlated with Homa-IR and SAA level in each group.Conclusions SelS protein may be involved in insulin resistarice;in Chinese with T2DM by acting as the SAA receptor,thus playing an important role in the development of T2DM and atherosclerosis.

  18. Expression of Genes Related to Prostaglandin Synthesis or Signaling in Human Subcutaneous and Omental Adipose Tissue: Depot Differences and Modulation by Adipogenesis

    Directory of Open Access Journals (Sweden)

    Andréanne Michaud

    2014-01-01

    Full Text Available Objectives. (1 To examine depot-specific PGE2 and PGF2α release and mRNA expression of enzymes or receptors involved in PG synthesis or signaling in human adipose tissues; (2 to identify changes in expression of these transcripts through preadipocyte differentiation; and (3 to examine associations between adipose tissue mRNA expression of these transcripts and adiposity measurements. Methods. Fat samples were obtained surgically in women. PGE2 and PGF2α release by preadipocytes and adipose tissue explants was measured. Expression levels of mRNA coding for enzymes or receptors involved in PG synthesis or signaling were measured by RT-PCR. Results. Cultured preadipocytes and explants from omental fat released more PGE2 and PGF2α than those from the subcutaneous depot and the corresponding transcripts showed consistent depot differences. Following preadipocyte differentiation, expression of PLA2G16 and PTGER3 mRNA was significantly increased whereas COX-1, COX-2, PTGIS, and PTGES mRNA abundance were decreased in both compartments (P≤0.01 for all. Transcripts that were stimulated during adipogenesis were those that correlated best with adiposity measurements. Conclusion. Cells from the omental fat compartment release more PGE2 and PGF2α than those from the subcutaneous depot. Obesity modulates expression of PG-synthesizing enzymes and PG receptors which likely occurs through adipogenesis-induced changes in expression of these transcripts.

  19. Heart involvement in cystic fibrosis: A specific cystic fibrosis-related myocardial changes?

    Science.gov (United States)

    Labombarda, Fabien; Saloux, Eric; Brouard, Jacques; Bergot, Emmanuel; Milliez, Paul

    2016-09-01

    Cystic fibrosis is a complex multi-systemic chronic disease characterized by progressive organ dysfunction with development of fibrosis, possibly affecting the heart. Over the last four decades pathological, experimental, and clinical evidence points towards the existence of a specific myocardial involvement in cystic fibrosis. Multi-modality cardiac imaging, especially recent echocardiographic techniques, evidenced diastolic and/or systolic ventricular dysfunction in cystic fibrosis leading to the concept of a cystic fibrosis-related cardiomyopathy. Hypoxemia and inflammation are among the most important factors for heart involvement in cystic fibrosis. Cystic Fibrosis Transmembrane Regulator was found to be involved in the regulation of cardiomyocyte contraction and may also account for cystic fibrosis-related myocardial dysfunction. This review, mainly focused on echocardiographic studies, seeks to synthesize the existing literature for and against the existence of heart involvement in cystic fibrosis, its mechanisms and prognostic implications. Careful investigation of the heart function may be helpful for risk stratification and therapeutic decisions in patients with cystic fibrosis.

  20. Management of refractory Pseudomonas aeruginosa infection in cystic fibrosis

    Directory of Open Access Journals (Sweden)

    Roger Sordé

    2011-01-01

    Full Text Available Roger Sordé1,2, Albert Pahissa1,2, Jordi Rello3,41Department of Infectious Diseases, Hospital Universitari Vall d'Hebron, Vall d'Hebron Research Institute (VHIR, Universitat Autònoma de Barcelona, Barcelona, Spain; 2Spanish Network for Research in Infectious Diseases (REIPI, Spain; 3Department of Critical Care, Hospital Universitari Vall d'Hebron, Vall d'Hebron Research Institute (VHIR, Universitat Autònoma de Barcelona, Barcelona, Spain; 4CIBER Enfermedades Respiratorias (CIBERES, SpainAbstract: Cystic fibrosis (CF is the most common life-limiting inherited disease in Caucasian populations. The main cause of death in CF patients is respiratory failure resulting from chronic pulmonary infection. Pseudomonas aeruginosa is the most prevalent organism in the airway colonization of CF patients, and its persistence in the airways has been related to greater morbidity with a more rapid deterioration in lung function. P. aeruginosa has enormous genetic and metabolic flexibility that allows it to adapt and persist within the airways of CF patients, and it has the ability to easily acquire antimicrobial resistance. For these reasons, the management of infections and chronic colonization by P. aeruginosa remains a challenge for physicians. This article reviews the current and future antibacterial chemotherapy options for respiratory pseudomonal infection in CF patients.Keywords: cystic fibrosis, Pseudomonas aeruginosa, respiratory infection, antimicrobial treatment

  1. Pulmonary changes in cystic fibrosis

    International Nuclear Information System (INIS)

    Pulmonary abnormalities in cystic fibrosis result from the obstruction of small bronchi by highly viscous mucus. Chronic obstructive lung disease and recurrent pulmonary infections result in a typical radiographic pattern later in the disease. Most patients can now be expected to survive into adulthood. The radiologist must make a careful comparison of serial films in order to detect complications early. By far the most important imaging modality is the conventional chest radiograph. CT is more sensitive for detection of structural abnormalities of the lung. Bronchography is a dangerous procedure and can lead to rapid deterioration of lung function. Lung scanning is a very sensitive method for demonstrating regional disturbances of ventilation and may reveal abnormalities earlier than conventional radiographs. In severe hemoptysis, selective bronchial arteriography with embolization of the bleeding vessel can be a life-saving procedure. (orig.)

  2. [Epidemiology of cystic echinococcosis in the world].

    Science.gov (United States)

    Tünger, Özlem

    2013-01-01

    The incidence and prevalence of cystic echinococcosis have fallen dramatically over the past several decades. Nonetheless, cystic echinococcosis remains a major public health issue in several countries and regions as a result of a reduction of control programmes due to economic problems. Geographic distribution differs by country and region depending on the presence of large numbers of nomadic or semi-nomadic sheep and goat flocks that represent the intermediate host of the parasite, and their close contact with the final host, the dog, which mostly provides the transmission of infection to humans. The greatest prevalence of cystic echinococcosis in human and animal hosts is found in countries of the temperate zones, including Mediterranean regions, southern and central parts of Russia, central Asia, China, Australia, South America and north and east Africa. In this article, the geographic distribution and epidemiology of cystic echinococcosis worldwide are reviewed. PMID:23619047

  3. Pathohistological changes in fetuses with cystic fibrosis

    Directory of Open Access Journals (Sweden)

    Đolai Matilda

    2012-01-01

    Full Text Available Introduction. Cystic fibrosis or mucoviscidosis is a genetically caused disease. The intensity of disease and histopathological changes grow throughout the life. According to the literature, pathological changes characteristic of cystic fibrosis become noticeable around the sixth month of life. Case Report. After amniocentesis of a 5-lunar month-old fetus had been done, which confirmed cystic fibrosis, the Ethics Commission approved the preterm labor. The autopsy and histopathological analysis demonstrated the existence of typical histopathological changes in the pancreas and intestines. Discussion. In the late fetal period or during the period around the delivery, cystic fibrosis is usually manifested as meconial cap with or without obstruction of the intestinal lumen. Morphological changes in the exocrine glands usually develop only after birth. In this case, the existence of meconial obstruction, as well as the typical acidofil content in the secretory ducts and acini of the pancreas was confirmed, which is unusual for the fetal age of five months.

  4. Cystic cervical intramedullary schwannoma with syringomyelia

    Directory of Open Access Journals (Sweden)

    Shenoy S

    2005-01-01

    Full Text Available We report a case of cervical intramedullary cystic schwannoma associated with segmental syrinx in a young adult without evidence of neurofibromatosis. The relevant literature is reviewed.

  5. 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 d

  6. Immunoreactive trypsin and neonatalscreening for cystic fibrosis

    International Nuclear Information System (INIS)

    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

  7. Subglottic adenoid cystic carcinoma mistaken for asthma

    Institute of Scientific and Technical Information of China (English)

    Hua-lin WANG; Lin XU; Fu-jun LI

    2009-01-01

    of the tumor. The diagnosis of subglottic adenoid cystic carcinoma should be considered in patients who are characterized by dyspnea, cough, and stridor, but do not respond to regular anti-asthmatic therapy.

  8. Acquired von Willebrand Syndrome

    Institute of Scientific and Technical Information of China (English)

    郭涛

    2005-01-01

    @@ Acquired von Willebrand syndrome (AvWS) is kind of bleeding disorder with laboratory findings similar to those in congenital yon Willebrand disease (vWD).AvWS doesn's have any personal or family history of bleeding, but is associated with certain diseases or abnormal conditions or drugs. Although AvWS is being stated as a rare disease, it has gained more and more attention during the past years. Not because of the severity of the disease, but it is more common than we thought and most patients don' t have a proper diagnosis.

  9. Acquired epidermolysis bullosa

    Directory of Open Access Journals (Sweden)

    Maricel Sucar Batista

    2015-12-01

    Full Text Available Epidermolysis bullosa is a group of diseases or skin disorders genetically transmitted and it is characterized by the appearance of bullae, ulcers and skin wounds. It usually appears at birth or in the first months of life. This is a case of a 72-year-old female patient who comes to the dermatology department with skin lesions of 6 months of evolution. A skin biopsy was performed, taking a sample for direct and indirect immunofluorescence. Acquired epidermolysis bullosa of unknown etiology was diagnosed. Treatment was started with low-dose colchicine to increase it later, according to the patient’s tolerance and disease progression.

  10. Unusual Presentation of Cystic Papillary Thyroid Carcinoma

    OpenAIRE

    Patil, Vijayraj S.; Abhishek Vijayakumar; Neelamma Natikar

    2012-01-01

    Papillary thyroid carcinoma is the most common thyroid malignancy, accounting for 80% of all thyroid cancers. The most common presentation of thyroid cancer is an asymptomatic thyroid mass or a nodule. Usually as thyroid enlarges, it extends in to mediastinum. Papillary thyroid carcinoma presentation as multiple true cystic swelling extending from neck to anterior chest wall in subcutaneous plane is not present in the literature. We present a rare case of cystic papillary thyroid carcinoma wh...

  11. Mesenchymal Cystic Hamartoma Presenting with Spontaneous Pneumothorax

    OpenAIRE

    J Glezos; Toppin, D; Cooney, T

    2003-01-01

    A 53-year-old woman presented with a spontaneous right-sided pneumothorax. Computed tomography chest scan demonstrated a large bulla involving the right middle lobe. Recurrence of the pneumothorax after initial closed chest tube drainage necessitated thoracotomy and wedge resection. Histopathological examination revealed features consistent with a benign mesenchymal cystic hamartoma. Pneumothorax is a recognized complication of mesenchymal cystic hamartoma, a lesion that also has malignant po...

  12. Nephronophthisis and medullary cystic kidney disease complex

    OpenAIRE

    Stanišić Marijana; Hrvačević Rajko; Paunić Zoran; Petrović Stanko

    2005-01-01

    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...

  13. Communicating with young adults with cystic fibrosis.

    OpenAIRE

    Webb, A. K.

    1995-01-01

    The care of the young adult with cystic fibrosis is complex, requiring a multidisciplinary input from different carers. Communication with and education of patients covers many areas; topics may include medical and personal problems, transplantation, survival, current scientific breakthroughs and the future. Communicating in these areas with knowledgeable young adults requires skill, tact and self-education upon the part of the cystic fibrosis team.

  14. 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....

  15. Cystic fibrosis from the gastroenterologist's perspective.

    Science.gov (United States)

    Ooi, Chee Y; Durie, Peter R

    2016-03-01

    Cystic fibrosis is a life-limiting, recessive disease caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. Increased survival outcomes and the multisystem nature of the disease, including the involvement of hepatobiliary and gastrointestinal tracts, now require the need for more extensive knowledge and expertise in cystic fibrosis among gastroenterologists. Manifestations are either a direct consequence of the primary defect in cystic fibrosis or a secondary complication of the disease or therapy. Adult patients with cystic fibrosis also have an increased risk of malignancy in the gastrointestinal and pancreatico-biliary tracts compared with the general population. Novel treatments that target the basic defects in the CFTR protein have emerged, but to date not much is known about their effects on the gastrointestinal and hepatobiliary systems. The introduction of such therapies has provided new opportunities for the application of intestinal endpoints in clinical trials and the understanding of underlying disease mechanisms that affect the gut in cystic fibrosis. PMID:26790364

  16. CT findings in skeletal cystic echinococcosis

    International Nuclear Information System (INIS)

    Purpose: To evaluate the CT findings of skeletal cystic echinococcosis. Material and Methods: CT findings of 7 patients with pathologically confirmed skeletal cystic echinococcosis were evaluated. Results: There were 4 men and 3 women, aged 36-75 years. Hydatid cysts were located in the spine (n=2), a rib (n=3), the pelvis and a vertebra (n=1), the pelvis and the left femur (n=1). The size of the lesions varied from 1 cm to 15 cm. CT showed well defined, single or multiple cystic lesions with no contrast enhancement, no calcification, no daughter cysts, and no germinal membrane detachment. The cystic lesion had a honeycomb appearance in 2 cases, there was pathologic fracture in 2 cases, bone expansion in 5 cases, cortical thinning in 6 cases, cortical destruction in 6 cases, bone sclerosis in 1 case, and soft tissue extension in 6 cases. Conclusion: Preoperative differential diagnosis of skeletal cystic lesions should include cystic echinococcosis, especially in endemic areas, since this diagnosis may easily be missed unless kept in mind

  17. Acquired Blaschkoid dermatitis

    Directory of Open Access Journals (Sweden)

    Mercy P

    2007-01-01

    Full Text Available Acquired Blaschkoid dermatitis characterised by unilateral relapsing inflammatory disease along the lines of Blaschko. A 40-year-old Indian male presented with unilateral erythematous, itchy grouped papules on the left side of the chest, abdomen, back and left arm of 15 days duration. The eruption stopped abruptly at the midline of the torso, completely sparing the right side of the body. The lesions were arranged in whorls and streaks corresponding to the lines of Blaschko. Skin biopsy showed hyperkeratosis and features suggestive of sub-acute spongiotic dermatitis with lymphocytic infiltrate around the blood vessels in the dermis. Patient was diagnosed as a case of Blaschkoid dermatitis. To the best of our knowledge, this is the first case of this condition being reported from India.

  18. Learning-By-Being-Acquired

    DEFF Research Database (Denmark)

    Colombo, Massimo G.; Moreira, Solon; Rabbiosi, Larissa

    In this paper we study post-acquisition integration in terms of R&D team reorganization—i.e., the creation of new teams with both inventors of the acquiring and acquired firms—and assess its impact on knowledge transfer in the period that follows the acquisition. Drawing on social identity and self......-categorization theories, we argue that R&D team reorganization increases the acquired inventors’ use of the prior stock of technological knowledge of the acquiring firm after the acquisition. Furthermore, this effect is enhanced if acquired inventors have higher innovation ability relative to their acquiring peers...... but is weakened for acquired inventors with high preacquisition ingroup collaborative strength. We construct a sample of 3,625 acquired inventors implementing the coarsened exact matching (CEM) technique and empirically test our arguments applying a difference-in-differences setup in a longitudinal data setting...

  19. Surgical outcome in cystic vestibular schwannomas

    Science.gov (United States)

    Nair, Suresh; Baldawa, Sachin S.; Gopalakrishnan, Chittur Viswanathan; Menon, Girish; Vikas, Vazhayil; Sudhir, Jayanand B.

    2016-01-01

    Background: Cystic vestibular schwannomas (VS) form a rare subgroup that differs from the solid variant clinically, radiologically, and histopathologically. These tumors also vary in their surgical outcome and carry a different risk of post-operative complications. We analyzed our series of 64 patients with cystic VS and discuss the technical difficulties related to total excision of these tumors and focus on complication avoidance. Materials and Methods: A retrospective review of cystic VS surgically managed over a span of 11 years. The case records were evaluated to record the clinical symptoms and signs, imaging findings, surgical procedure, complications, and follow-up data. Post-operative facial nerve palsy was analyzed with respect to tumor size and tumor type. Results: Progressive hearing impairment was the most common initial symptom (76.6%). Atypical initial symptoms were present in 15 patients (23.4%). Preoperatively, 78% patients had good facial nerve function (HB grade 1, 2) and 22% had intermediate (HB grade 3, 4) to poor (HB grade 5 and 6) function. Mean tumor size was 4.1 cm. Complete tumor removal was achieved in 53 patients (83%). The facial nerve was anatomically intact but thinned out after tumor excision in 38 patients (59.4%). Ninety percent patients had either intermediate or poor facial nerve function at follow-up. Poor facial nerve outcome was associated with giant tumors and peripherally located, thin-walled cystic tumors. Conclusion: Resection of cystic VS is complicated by peritumoral adhesions of the capsule to the nerve. Extensive manipulation of the nerve in order to dissect the tumor–nerve barrier results in worse facial nerve outcome. The outcome is worse in peripherally located, thin-walled cystic VS as compared to centrally located, thick-walled cystic tumors. Subtotal excision may be justified, especially in tumors with dense adhesion of the cyst wall to the facial nerve in order to preserve nerve integrity. PMID:27366248

  20. 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.

  1. 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.

  2. Respiratory Conditions Update: Cystic Fibrosis.

    Science.gov (United States)

    Pritchard, Lyle L

    2016-09-01

    Cystic fibrosis (CF) is an autosomal recessive genetic disease that occurs in approximately 1 in 2,500 white live births. It is less common in nonwhite individuals. A dysfunctional epithelial chloride channel leads to excessively thick mucus affecting multiple organ systems. Common issues include mucous plugging of the airway, lung inflammation, chronic pulmonary infections, intestinal malabsorption, and malnutrition. Universal screening of newborns for CF is recommended in many countries. CF can be diagnosed based on clinical evidence of disease along with genetic testing or other laboratory evidence of chloride channel dysfunction. Pulmonary system dysfunction causes the most morbidity and mortality. Pulmonary function testing is the primary modality used to monitor CF progression. Therapies include chest physiotherapy, mucolytics, antibiotics, anti-inflammatory drugs, targeted therapies, and vaccines. Dysfunction of the exocrine pancreas and gastrointestinal tract leads to malabsorption, malnutrition, and intestinal obstruction. Nutrition should be optimized with adequate calories, pancreatic enzymes, and appropriate dietary supplements. Complications, including acute pulmonary exacerbations, gastrointestinal conditions, chronic rhinosinusitis, CF-related diabetes, osteoporosis, infertility, and psychosocial issues, must be managed. At the appropriate time, lung transplantation and end-of-life issues must be addressed. PMID:27576234

  3. T-Cell lymphoproliferative disorder of hand-mirror cell morphology presenting in an eosinophilic loculated peritoneal effusion, with omental "caking"

    Directory of Open Access Journals (Sweden)

    Tufankjian Dearon

    2006-01-01

    Full Text Available Abstract Background Cells with "hand mirror" morphology have not, to the best of our knowledge, been described in a primary effusion sample. This paper describes a case of T-cell lymphoma with eosinophilia in a patient with suspected peritoneal carcinomatosis. Rarely, a T-cell lymphoproliferative process may mimic primary peritoneal carcinomatosis, clinically suggested by a presentation in CT imaging of omental caking with bilateral massive loculated effusions in a patient without lymphadenopathy or splenomegaly. Methods A 60 year old caucasian male presented with vague abdominal discomfort and increasing abdominal girth. Computed tomography showed a two centimeter thick omental cake and a small loculated effusion. The clinical presentation and imaging findings were most consistent with peritoneal carcinomatosis. Cytologic evaluation of the effusion was undertaken for diagnostic study. Results Rapid intraprocedural interpretation of the effusion sample showed a monomorphic population of cells with "hand-mirror" cell morphology exhibiting cytoplasmic extensions (uropodia with 3–5 course dark cytoplasmic granules and a rim of vacuolated cytoplasm capping the opposing "mirror head" side. These cells were seen within a background of mature eosinophils. Flow cytometric evaluation of the ascites fluid demonstrated an atypical T-cell population with the following immunophenotype: CD2-, CD3+, CD4-, CD5-, CD7-, CD8+, CD56+. T-cell receptor (TCR gene rearrangement was positive for clonal TCR-gamma gene rearrangement, supporting the diagnosis of a T-lymphoprolifereative disorder. Conclusion A T-cell lymphoproliferative process may present with "hand mirror" morphology in an effusion sample. These cells may show polar cytoplasmic vacuolization and 3–5 course granules within the "handle" of these unique cells. Cytoplasm shows peripheral constriction around the nucleus.

  4. Intercontrole acquiring by Framatome

    International Nuclear Information System (INIS)

    The Framatome group, as the worldwide leader in nuclear power plant construction, has reinforced his competences in nuclear services thanks to the acquiring of the Intercontrole company, specialized in non-destructive testing in nuclear and industrial environments. After a presentation of the functioning principle and of the safety aspects of a PWR reactor, this press dossier presents in a first part the role of nuclear services and in particular of non-destructive testing in nuclear power plants (in-service inspection, regulatory aspects, testing processes). This part is illustrated with some examples of inspection performed on some components of the primary coolant loop (steam generators, reactor vessel, pressurizer, pipes, primary pumps). A second part presents the technical centres and units of Framatome in charge of performing non-destructive inspections, while a third part describes the industrial policy and strategy of the group in this domain (market of nuclear park maintenance in France, in the USA and worldwide, creation of the 'inspection and control' centre of Framatome). A last part presents the activities of the Intercontrole company and of its daughter companies with some examples of actions realized in the nuclear and natural gas domains. (J.S.)

  5. Vitamin K and the management of patients with cystic fibrosis.

    OpenAIRE

    Durie, P R

    1994-01-01

    OBJECTIVE: To assess the advisability of routine vitamin K supplementation in patients with cystic fibrosis (CF). DATA SOURCES: Studies identified through a MEDLINE search with the use of MeSH terms vitamin K, cystic fibrosis, PIVKA-II (protein induced by vitamin K absence-II), coagulation abnormality and cystic fibrosis, and hepatic disorder and cystic fibrosis. STUDY SELECTION: Six articles published between January 1981 and December 1992 were selected: one general review of vitamin K in in...

  6. Cystic Meningioma Masquerading as a Metastatic Tumor: A Case Report.

    Science.gov (United States)

    Ramanathan, Nithya; Kamaruddin, Khairul Azmi; Othman, Aizzat; Mustafa, Fadhli; Awang, Mohamed Saufi

    2016-05-01

    Cystic meningioma is a rare form of intracranial meningioma. Meningiomas are typically solid tumors but may rarely have cystic components. The diagnosis of cystic meningioma is clinically challenging as the finding of multiple intra-axial tumors, including metastatic tumors, is relatively common. We report a case of cystic meningioma initially diagnosed as a metastatic tumor from a recurrence of acute lymphoid leukemia. However, postoperative histopathological examination demonstrated an atypical meningioma. PMID:27418876

  7. Nitric oxide metabolites in cystic fibrosis lung disease

    OpenAIRE

    Grasemann, H; Ioannidis, I.; Tomkiewicz, R; de Groot, H.; Rubin, B; Ratjen, F

    1998-01-01

    Although the activity of nitric oxide (NO) synthases are increased in lung tissue of patients with cystic fibrosis, the concentrations of nasal and exhaled NO have recently been found to be decreased in cystic fibrosis. This could either be due to reduced NO formation or metabolism of NO within airway fluids. In this study, the stable NO metabolites, nitrate and nitrite, were determined in the saliva and sputum of 18 stable cystic fibrosis patients, 21 cystic fibrosis pat...

  8. Rare cystic liver lesions: A diagnostic and managing challenge

    OpenAIRE

    Bakoyiannis, Andreas; DELIS, SPIROS; Triantopoulou, Charina; Dervenis, Christos

    2013-01-01

    Cystic formations within the liver are a frequent finding among populations. Besides the common cystic lesions, like simple liver cysts, rare cystic liver lesions like cystadenocarcinoma should also be considered in the differential diagnosis. Thorough knowledge of each entity’s nature and course are key elements to successful treatment. Detailed search in PubMed, Cochrane Database, and international published literature regarding rare cystic liver lesions was carried out. In our research are...

  9. Cystic lung disease: a comparison of cystic size, as seen on expiratory and inspiratory HRCT scans

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Ki Nam; Yoon, Seong Kuk; Nam, Kyung Jin [Donga University College of Medicine, Pusan (Korea, Republic of); Choi, Seok Jin [Inje University College of Medicine, Gimhae (Korea, Republic of); Goo, Jin Mo [Seoul National University College of Medicine, Seoul (Korea, Republic of)

    2000-06-01

    To determine the effects of respiration on the size of lung cysts by comparing inspiratory and expiratory high-resolution CT (HRCT) scans. The authors evaluated the size of cystic lesions, as seen on paired inspiratory and expiratory HRCT scans, in 54 patients with Langerhans cell histiocytosis (n = 3), pulmonary lymphangiomyomatosis (n = 4), confluent centrilobular emphysema (n = 9), paraseptal emphysema and bullae (n = 16), cystic bronchiectasis (n = 13), and honeycombing (n = 9). Using paired inspiratory and expiratory HRCT scans obtained at the corresponding anatomic level, a total of 270 cystic lesions were selected simultaneously on the basis of five lesions per lung disease. Changes in lung cyst size observed during respiration were assessed by two radiologists. In a limited number of cases (n = 11), pathologic specimens were obtained by open lung biopsy or lobectomy. All cystic lesions in patients with Langerhans cell histiocytosis, lymphangiomyomatosis, cystic bronchiectasis, honeycombing, and confluent centrilobular emphysema became smaller on expiration, but in two cases of paraseptal emphysema and bullae there was no change. In cases in which expiratory CT scans indicate that cysts have become smaller, cystic lesions may communicate with the airways. To determine whether, for cysts and cystic lesions, this connection does in fact exist, paired inspiratory and expiratory HRCT scans are necessary.

  10. Cystic lung disease: a comparison of cystic size, as seen on expiratory and inspiratory HRCT scans

    International Nuclear Information System (INIS)

    To determine the effects of respiration on the size of lung cysts by comparing inspiratory and expiratory high-resolution CT (HRCT) scans. The authors evaluated the size of cystic lesions, as seen on paired inspiratory and expiratory HRCT scans, in 54 patients with Langerhans cell histiocytosis (n = 3), pulmonary lymphangiomyomatosis (n = 4), confluent centrilobular emphysema (n = 9), paraseptal emphysema and bullae (n = 16), cystic bronchiectasis (n = 13), and honeycombing (n = 9). Using paired inspiratory and expiratory HRCT scans obtained at the corresponding anatomic level, a total of 270 cystic lesions were selected simultaneously on the basis of five lesions per lung disease. Changes in lung cyst size observed during respiration were assessed by two radiologists. In a limited number of cases (n = 11), pathologic specimens were obtained by open lung biopsy or lobectomy. All cystic lesions in patients with Langerhans cell histiocytosis, lymphangiomyomatosis, cystic bronchiectasis, honeycombing, and confluent centrilobular emphysema became smaller on expiration, but in two cases of paraseptal emphysema and bullae there was no change. In cases in which expiratory CT scans indicate that cysts have become smaller, cystic lesions may communicate with the airways. To determine whether, for cysts and cystic lesions, this connection does in fact exist, paired inspiratory and expiratory HRCT scans are necessary

  11. Study of cystic artery by arteriography. Importance of previous cholecystography

    Energy Technology Data Exchange (ETDEWEB)

    Machado, G.O.

    An oral cholecystography previously to celiac and mesenteric arteriography is performed, in order to identify the cystic artery, in 42 patients with pancreatitis, according Seldinger technique. The cystic artery was identified in all the cases, the pattern being the outlet of the cystic artery from the right hepatic artery. Infusion pump and seriography were not used.

  12. 78 FR 26681 - Medical Criteria for Evaluating Cystic Fibrosis

    Science.gov (United States)

    2013-05-07

    ... ADMINISTRATION RIN 0960-AF58 Medical Criteria for Evaluating Cystic Fibrosis AGENCY: Social Security....04 to evaluate claims involving cystic fibrosis in adults and children under titles II and XVI of the... information on the disability program. 2. Information for individuals with cystic fibrosis who apply...

  13. 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.)

  14. Acute Scedosporium apiospermum Endobronchial Infection in Cystic Fibrosis.

    Science.gov (United States)

    Padoan, Rita; Poli, Piercarlo; Colombrita, Domenico; Borghi, Elisa; Timpano, Silviana; Berlucchi, Marco

    2016-06-01

    Fungi are known pathogens in cystic fibrosis patients. A boy with cystic fibrosis boy presented with acute respiratory distress. Bronchoscopy showed airways obstruction by mucus plugs and bronchial casts. Scedosporium apiospermum was identified as the only pathogen. Bronchoalveolar lavage successfully resolved the acute obstruction. Plastic bronchitis is a new clinical picture of acute Scedosporium endobronchial colonization in cystic fibrosis patients. PMID:26967814

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

    Science.gov (United States)

    ... Understand Genetics Home Health Conditions medullary cystic kidney disease type 1 medullary cystic kidney disease type 1 Enable Javascript to ... and How They Work Educational Resources (4 links) Disease InfoSearch: Medullary cystic kidney disease 1 Merck Manual Consumer Version: ...

  16. 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;…

  17. Endocytosis and intracellular protein degradation in cystic fibrosis fibroblasts

    International Nuclear Information System (INIS)

    Normal rates of pinocytosis of [3H]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.)

  18. Premalignant cystic neoplasms of the pancreas.

    Science.gov (United States)

    Dudeja, Vikas; Allen, Peter J

    2015-02-01

    Due to increasing utilization of cross-sectional imaging, asymptomatic pancreatic cysts are frequently being diagnosed. Many of these cysts have premalignant potential and offer a unique opportunity for cancer prevention. Mucinous cystic neoplasm and intraductal papillary mucinous neoplasm are the major premalignant cystic neoplasms of pancreas. The prediction of the risk of malignancy (incidental and future risk of malignant transformation) and balancing the risks of watchful waiting with that of operative management with associated mortality and morbidity is the key to the management of these lesions. We review the literature that has contributed to the development of our approach to the management of these cystic neoplasms. We provide an overview of the key features used in diagnosis and in predicting malignancy. Particular attention is given to the natural history and management decision making.

  19. Retrorectal cystic hamartoma: a problematic 'tail'.

    Science.gov (United States)

    Suhani; Meena, Kusum; Ali, Shadan; Aggarwal, Lalit; Thomas, Shaji

    2014-01-01

    The retrorectal space is an uncommon seat for neoplastic masses. Retrorectal hamartoma or tailgut cyst (TGC) is an uncommon developmental cystic lesion occurring in this space which mostly occurs in middle-aged females. We recently cared for a 16-year-old girl who presented with vague lower abdominal pain and occasional constipation. Per rectal examination revealed an extraluminal mass bulging from posterior rectal wall. Preoperative radiological investigations revealed by suggested it to be a mature cystic teratoma. The patient underwent exploratory laprotomy with en masse excision of the cyst. Histopathological examination of the specimen showed it to be a TGC. This case highlights the possibility of a TGC as a differential for retrorectal cystic lesions and the need to completely excise them given the possibility of future malignant transformation. PMID:25598944

  20. Dyspnea perception in cystic fibrosis patients

    Directory of Open Access Journals (Sweden)

    B. Ziegler

    2013-09-01

    Full Text Available We evaluated dyspnea perception in cystic fibrosis patients compared with normal subjects, during an inspiratory resistive loading test and 6-min walk test. We also evaluated the correlation between dyspnea scores induced by resistive loads and by the 6-min walk test. In this prospective, cross-sectional study, 31 patients with cystic fibrosis (≥15 years of age and 31 age-, gender-, and ethnicity-matched healthy volunteers (20 females and 11 males per group underwent inspiratory resistive loading, spirometry, and the 6-min walk test. As the magnitude of the inspiratory loads increased, dyspnea scores increased (P<0.001, but there was no difference between groups in dyspnea score (P=0.654. Twenty-six (84% normal subjects completed all the resistive loads, compared with only 12 (39% cystic fibrosis patients (P<0.001. Dyspnea scores were higher after the 6-min walk test than at rest (P<0.001, but did not differ between groups (P=0.080. Post-6-min walk test dyspnea scores correlated significantly with dyspnea scores induced by resistive loads. We conclude that dyspnea perception induced in cystic fibrosis patients by inspiratory resistive loading and by 6-min walk test did not differ from that induced in normal subjects. However, cystic fibrosis patients discontinued inspiratory resistive loading more frequently. There were significant correlations between dyspnea perception scores induced by inspiratory resistance loading and by the 6-min walk test. This study should alert clinicians to the fact that some cystic fibrosis patients fail to discriminate dyspnea perception and could be at risk for delay in seeking medical care.

  1. 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.

  2. Genetics of Cystic Fibrosis: Clinical Implications.

    Science.gov (United States)

    Egan, Marie E

    2016-03-01

    Cystic fibrosis (CF) is a common life-shortening autosomal recessive genetic disorder caused by mutations in the gene that encodes for the cystic fibrosis transmembrane conductance regulator protein (CFTR). Almost 2000 variants in the CFTR gene have been identified. The mutational classes are based on the functional consequences on CFTR. New therapies are being developed to target mutant CFTR and restore CFTR function. Understanding specific CF genotypes is essential for providing state-of-the art care to patients. In addition to the variation in CFTR genotype, there are several modifier genes that contribute to the respiratory phenotype.

  3. 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...

  4. 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. PMID:26857767

  5. Qulaity of Life in Cystic Fibrosis Children

    OpenAIRE

    Hamid-Reza Kianifar; Banafsheh Bakhshoodeh; Paria Hebrani; Fatemeh Behdani

    2013-01-01

    Objective: Cystic fibrosis (CF) is a chronic, multisystem genetic disease with a wide variability in clinical severity. The measurement of quality of life in CF provides additional information about the impact of this disease. This article tries to assess quality of life (QoL) in children and adolescents with CF and to compare it with control group.Methods: Patients 2-18 years old with admission diagnosis of cystic fibrosis entered the study. QoL was observed in CF patients and compared with ...

  6. Cystic dilation of the ventriculus terminalis

    Science.gov (United States)

    Kawanishi, Masahiro; Tanaka, Hidekazu; Yokoyama, Kunio; Yamada, Makoto

    2016-01-01

    The ventriculus terminalis (VT) is a virtual cavity of the conus medullaris that appears during embryonic life. We presented a case with the cystic dilation of the ventriculus terminalis (CDVT) in a symptomatic patient. A 66-year-old female suffered from disturbance while walking evolving for the past 2 years. An MR image revealed a cystic dilatation of ventriculus terminalis. The patient experienced marked improvement of lower extremity strength by a fenestration of cyst and cyst-subarachnoid shunt. Treatment for asymptomatic patients seems to be the best conducted conservatively, whereas patients with focal neurological deficits seem to be best handled surgically.

  7. Cystic Eccrine Spiradenoma of the Finger Mimicking a Ganglion

    Directory of Open Access Journals (Sweden)

    Khalid F. Jaber, MBChB

    2014-01-01

    Full Text Available Summary: We report a rare case of cystic eccrine spiradenoma in the finger. A 46-year-old man presented with a cystic mass in his left index finger. Clinical assessment along with the investigation pointed toward a diagnosis of a ganglion. However, excisional biopsy of the mass revealed histopathological findings of cystic eccrine spiradenoma. Very few cases of eccrine spiradenoma have been reported in the hand and none of them were cystic in consistency. We believe that this case will draw the surgeon’s attention to the possibility of unusual differential diagnoses in the evaluation and treatment of cystic lumps in the hand.

  8. Rare association between cystic fibrosis, Chiari I malformation, and hydrocephalus in a baby: a case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Jea Andrew

    2011-08-01

    Full Text Available Abstract Introduction Cystic fibrosis, an epithelial cell transport disorder caused by mutations of the cystic fibrosis transmembrane conductance regulator gene, is not generally associated with malformations of the central nervous system. We review eight previously published reports detailing an infrequent association between cystic fibrosis and Chiari I malformation. Case presentation To the best of our knowledge, our report describes only the ninth case of a baby presenting with a new diagnosis of cystic fibrosis and Chiari I malformation, in this case in a 10-month-old, full-term Caucasian baby boy from the United States of America. Neurosurgical consultation was obtained for associated developmental delay, macrocephaly, bulging anterior fontanel, and papilledema. An MRI scan demonstrated an extensive Chiari I malformation with effacement of the fourth ventricle, obliteration of the outlets of the fourth ventricle and triventricular hydrocephalus without aqueductal stenosis. Our patient was taken to the operating room for ventriculoperitoneal shunt placement. Conclusions It is possible that the cystic fibrosis transmembrane conductance regulator gene may play a previously unrecognized role in central nervous system development; alternatively, this central nervous system abnormality may have been acquired due to constant valsalva from recurrent coughing or wheezing or metabolic and electrolyte imbalances that occur characteristically in cystic fibrosis.

  9. Anti-Biofilm and Immunomodulatory Activities of Peptides That Inhibit Biofilms Formed by Pathogens Isolated from Cystic Fibrosis Patients

    OpenAIRE

    César de la Fuente-Núñez; Sarah C. Mansour; Zhejun Wang; Lucy Jiang; Breidenstein, Elena B. M.; Melissa Elliott; Fany Reffuveille; Speert, David P.; Reckseidler-Zenteno, Shauna L.; Ya Shen; Markus Haapasalo; Robert E W Hancock

    2014-01-01

    Cystic fibrosis (CF) patients often acquire chronic respiratory tract infections due to Pseudomonas aeruginosa and Burkholderia cepacia complex (Bcc) species. In the CF lung, these bacteria grow as multicellular aggregates termed biofilms. Biofilms demonstrate increased (adaptive) resistance to conventional antibiotics, and there are currently no available biofilm-specific therapies. Using plastic adherent, hydroxyapatite and flow cell biofilm models coupled with confocal and scanning electro...

  10. Cystic Lung Disease: a Comparison of Cystic Size, as Seen on Expiratory and Inspiratory HRCT Scans

    OpenAIRE

    Lee, Ki-Nam; Yoon, Seong-Kuk; Choi, Seok Jin; Goo, Jin Mo; Nam, Kyung-Jin

    2000-01-01

    Objective To determine the effects of respiration on the size of lung cysts by comparing inspiratory and expiratory high-resolution CT (HRCT) scans. Materials and Methods The authors evaluated the size of cystic lesions, as seen on paired inspiratory and expiratory HRCT scans, in 54 patients with Langerhans cell histiocytosis (n = 3), pulmonary lymphangiomyomatosis (n = 4), confluent centrilobular emphysema (n = 9), paraseptal emphysema and bullae (n = 16), cystic bronchiectasis (n = 13), and...

  11. 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...

  12. 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...

  13. Intestinal organoids as model for cystic fibrosis

    NARCIS (Netherlands)

    Dekkers, J.F.

    2015-01-01

    Recent advances in adult stem cell culture technology have enabled long-term in vitro expansion of intestinal organoids or ‘mini-guts’. In this thesis, we used the organoid model to develop a novel assay to measure function of CFTR, the protein mutated in subjects with cystic fibrosis (CF). This met

  14. Statement on Exercise Testing in Cystic Fibrosis

    NARCIS (Netherlands)

    Hebestreit, Helge; Arets, Hubertus G M; Aurora, Paul; Boas, Steve; Cerny, Frank; Hulzebos, Erik H J; Karila, Chantal; Lands, Larry C; Lowman, John D; Swisher, Anne; Urquhart, Don S

    2015-01-01

    This statement summarizes the information available on specific exercise test protocols and outcome parameters used in patients with cystic fibrosis (CF) and provides expert consensus recommendations for protocol and performance of exercise tests and basic interpretation of results for clinicians. T

  15. 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...

  16. 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...

  17. Exercise and Immune Function in Cystic Fibrosis

    NARCIS (Netherlands)

    van Leeuwen, P.B.

    2013-01-01

    In this thesis, we investigated the association between exercise, infection and inflammation in patients with Cystic Fibrosis (CF), since they have been described to be interconnected.Patients with CF spend a lot of time in physical exercises to maintain physical fitness, since exercise capacity dec

  18. 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...... respiratory tract (nasal sampling) should be investigated and both infection sites should be treated....

  19. 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

  20. Spontaneous pneumothorax in cystic adenomatoid malformation

    International Nuclear Information System (INIS)

    Spontaneous pneumothorax as the initial manifestation of cystic adenomatoid malformation of the lung is rare. Only four cases have been reported in the English literature. We add one more case, and review the clinical and roentgenographic findings. All the patients presented later in infancy or childhood and the cyst type appear to belong to Madewell's dominant cyst type. (orig.)

  1. Spontaneous pneumothorax in cystic adenomatoid malformation

    Energy Technology Data Exchange (ETDEWEB)

    Gaisie, G.; Sang Oh, K.

    1983-07-01

    Spontaneous pneumothorax as the initial manifestation of cystic adenomatoid malformation of the lung is rare. Only four cases have been reported in the English literature. We add one more case, and review the clinical and roentgenographic findings. All the patients presented later in infancy or childhood and the cyst type appear to belong to Madewell's dominant cyst type.

  2. Surgical management of pneumothorax in cystic fibrosis.

    OpenAIRE

    Seddon, D. J.; Hodson, M E

    1988-01-01

    Twenty seven adults with cystic fibrosis who had had either a surgical pleurodesis or pleurectomy for the management of pneumothorax were studied. There were no significant differences in postoperative respiratory function, incidence of recurrent pneumothorax, or incidence of major postoperative complications between the two groups.

  3. Cystic fibrosis, are we missing in India?

    Directory of Open Access Journals (Sweden)

    Pramila Menon

    2012-09-01

    Full Text Available Introduction: This study was undertaken to evaluate and improve the availability and accessibility of CF services in Maharashtra, Pune,India. Material and methods: Tool used was a questionnaire (Annexure I which was prevalidated by the departmental faculty with 15 items which included experience regarding CF patients in relation to knowledge about diagnosis and prognosis of CF patients, availability about diagnostic and management services of CF wasadministered to the pediatricians who participated in the training workshop at Pune Regional centre, Aundh, Pune, Maharashtra, India. ( n = 40. Results: 92.5% agreed that Cystic fibrosis (CF, is now a pan ethnic disease. 100% accepted that the factors that are associated with decreased survival in CF patients from developing countries are early age of onset of symptoms, severe malnutrition at the time of diagnosis and frequent episodes of pneumonia. Severemalnutrition, not responding to nutritional therapy , neonatal meconium ileus and recurrent pneumonia , 87.5% 100% and 85% suspect cystic fibrosis respectively. 90% do not have facilities for diagnosis of cystic fibrosis like sweat chloride test and 87.5% felt absence of facilities makes the diagnosis of cystic fibrosis difficult. 90% agreed that the education of pediatricians about the disease, can improve the quality of life and survival in CF. Conclusion: Inadequacy of diagnostic services for CF may be reason for missing CF cases in India and we need to sensitize the health professionals for CF diagnosis and develop better diagnostic and patient support services for CF.

  4. Inhalation of antibiotics in cystic fibrosis

    NARCIS (Netherlands)

    Touw, D J; Brimicombe, R W; Hodson, M E; Heijerman, H G; Bakker, W

    1995-01-01

    Aerosol administration of antipseudomonal antibiotics is commonly used in cystic fibrosis. However, its contribution to the improvement of lung function, infection and quality of life is not well-established. All articles published from 1965 until the present time concerning the inhalation of antibi

  5. US Cystic Fibrosis Foundation and European Cystic Fibrosis Society consensus recommendations for the management of non-tuberculous mycobacteria in individuals with cystic fibrosis: executive summary

    OpenAIRE

    Floto, R. Andres; Olivier, Kenneth N.; Saiman, Lisa; Daley, Charles L.; Herrmann, Jean-Louis; Nick, Jerry A.; Noone, Peadar G; Bilton, Diana; Corris, Paul; Gibson, Ronald L.; Hempstead, Sarah E.; Koetz, Karsten; Sabadosa, Kathryn A; Sermet-Gaudelus, Isabelle; Smyth, Alan R

    2016-01-01

    Non-tuberculous mycobacteria (NTM) are ubiquitous environmental organisms that can cause chronic pulmonary infection, particularly in individuals with pre-existing inflammatory lung disease, such as cystic fibrosis (CF). Pulmonary disease (PD) caused by NTM has emerged as a major threat to the health of individuals with CF, but remains difficult to diagnose and problematic to treat. In response to this challenge, the US Cystic Fibrosis Foundation (CFF) and the European Cystic Fibrosis Society...

  6. US Cystic Fibrosis Foundation and European Cystic Fibrosis Society consensus recommendations for the management of non-tuberculous mycobacteria in individuals with cystic fibrosis

    OpenAIRE

    Floto, R. Andres; Olivier, Kenneth N.; Saiman, Lisa; Daley, Charles L.; Herrmann, Jean-Louis; Nick, Jerry A.; Noone, Peadar G; Bilton, Diana; Corris, Paul; Gibson, Ronald L.; Hempstead, Sarah E.; Koetz, Karsten; Sabadosa, Kathryn A; Sermet-Gaudelus, Isabelle; Smyth, Alan R

    2015-01-01

    Non-tuberculous mycobacteria (NTM) are ubiquitous environmental organisms that can cause chronic pulmonary infection, particularly in individuals with pre-existing inflammatory lung disease such as cystic fibrosis (CF). Pulmonary disease caused by NTM has emerged as a major threat to the health of individuals with CF but remains difficult to diagnose and problematic to treat. In response to this challenge, the US Cystic Fibrosis Foundation (CFF) and the European Cystic Fibrosis Society (ECFS)...

  7. 带蒂大网膜包肾防治肾小球硬化的实验研究%The Effects of Pedicled Omental Transposition on Rats with Glomerulosclerosis

    Institute of Scientific and Technical Information of China (English)

    董兴刚; 安增梅; 杨海春; 周江华; 殷祥雷; 顾建新

    2001-01-01

    To investigate the protective effects of pedicled omental transposition on the kidney with glomerulosclerosis.Uninephrectomized rats were divided into two groups.Normal rats were put in the blank control group,glomerulosclerosis rats by adriamycin were divided into glomerulosclerosis group and pedicled omental transposition group.Serum lipids,urea and urinary protein were assayed. Renal histological examination was also performed. Results showed that pedicled omental transposition could not only reduced the serum levels of total cholesterol and triglyceride, but also decrease the accumulation of extracellular matrix (P<0.05).Conclusions pedicled omental transposition has the effect of attenuating renal damage in rats with glomerulosclerosis.%为探讨带蒂大网膜包肾术防治肾小球硬化的作用。采用单侧肾切除加二次注射阿霉素制备的肾小球硬化动物模型,观察带蒂大网膜包肾对肾脏的作用。结果表明,带蒂大网膜包肾可降低血胆固醇、甘油三脂,尿蛋白排泄,肾小球球囊粘连减轻。血尿素氮有下降趋势。说明带蒂大网膜包肾术对肾小球硬化大鼠的肾脏损伤有保护作用。

  8. Value versus Use for Patients: Findings from an ICT supported Cystic Fibrosis Self-Management Project.

    Science.gov (United States)

    Roehrer, Erin; Cummings, Elizabeth; Ellis, Leonie; Turner, Paul

    2015-01-01

    This paper presents findings from the implementation of an online symptom monitoring diary that was part of a broader project aimed to support self-management of cystic fibrosis and the development of an online community of practice (CoP). The findings challenge conventional perspectives on how value is acquired by patients and their families from electronic tools designed to provide them with support. Additionally, the findings highlight complex relationships between user needs and subsequent tool use that challenge approaches to the measurement of value from ehealth projects. PMID:25676995

  9. Acquired ichthyosis with hoffman's syndrome

    Directory of Open Access Journals (Sweden)

    Sathyanarayana B

    2003-01-01

    Full Text Available A middle aged man presented with features of acquired ichthyosis with Hoffman's syndrome. Laboratory tests support hypothyodism. Myoedema and hypertrophy of muscles were present. Patient was previously treated for Pellagra.

  10. Cytological Features of the Cystic Fluid of Pancreatic Schwannoma with Cystic Degeneration. A Case Report

    Directory of Open Access Journals (Sweden)

    Kenichi Hirabayashi

    2008-03-01

    Full Text Available Context Schwannomas are benign neoplasms arising from peripheral nerve tissue. Pancreatic schwannoma is a very rare condition. We present the histological and cytological features of a pancreatic schwannoma with cystic degeneration. Case report A 51-year-old male was diagnosed with a cystic tumor measuring approximately 6 cm in the tail of the pancreas. Distal pancreatectomy and splenectomy were performed. Cystic fluid from the tumor was obtained intraoperatively by fine-needle aspiration, and it showed scattered spindle tumor cells against a background of hemosiderin-laden histiocytes. During the operation, we informed the surgeon that the tumor consisted of “atypical spindle cells”. Histologically, the tumor was diagnosed as a schwannoma with cystic degeneration which had originated in the pancreas. The diagnosis was confirmed by positive immunostaining of the tumor cells in both histological and cytological materials for S-100 protein. Conclusion Problems occasionally arise with the use of fine-needle aspiration in the diagnosis of cystic diseases of the pancreas because of the difficulty in obtaining adequate specimens. Nevertheless, it should be emphasized that intraoperative fine-needle aspiration is as informative as a frozen section diagnosis, when appropriately performed.

  11. Somatically acquired structural genetic differences

    DEFF Research Database (Denmark)

    Magaard Koldby, Kristina; Nygaard, Marianne; Christensen, Kaare;

    2016-01-01

    Structural genetic variants like copy number variants (CNVs) comprise a large part of human genetic variation and may be inherited as well as somatically acquired. Recent studies have reported the presence of somatically acquired structural variants in the human genome and it has been suggested...... with age.European Journal of Human Genetics advance online publication, 20 April 2016; doi:10.1038/ejhg.2016.34....

  12. Pregnancy and cystic fibrosis: Approach to contemporary management.

    Science.gov (United States)

    Geake, James; Tay, George; Callaway, Leonie; Bell, Scott C

    2014-12-01

    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

  13. Mature cystic Theratome. Presentation of a case

    International Nuclear Information System (INIS)

    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

  14. Diffuse Cystic Lung Disease. Part II.

    Science.gov (United States)

    Gupta, Nishant; Vassallo, Robert; Wikenheiser-Brokamp, Kathryn A; McCormack, Francis X

    2015-07-01

    The diffuse cystic lung diseases have a broad differential diagnosis. A wide variety of pathophysiological processes spanning the spectrum from airway obstruction to lung remodeling can lead to multifocal cyst development in the lung. Although lymphangioleiomyomatosis and pulmonary Langerhans cell histiocytosis are perhaps more frequently seen in the clinic, disorders such as Birt-Hogg-Dubé syndrome, lymphocytic interstitial pneumonia, follicular bronchiolitis, and light-chain deposition disease are increasingly being recognized. Obtaining an accurate diagnosis can be challenging, and management approaches are highly disease dependent. Unique imaging features, genetic tests, serum studies, and clinical features provide invaluable clues that help clinicians distinguish among the various etiologies, but biopsy is often required for definitive diagnosis. In part II of this review, we present an overview of the diffuse cystic lung diseases caused by lymphoproliferative disorders, genetic mutations, or aberrant lung development and provide an approach to aid in their diagnosis and management. PMID:25906201

  15. Diffuse Cystic Lung Disease. Part I.

    Science.gov (United States)

    Gupta, Nishant; Vassallo, Robert; Wikenheiser-Brokamp, Kathryn A; McCormack, Francis X

    2015-06-15

    The diffuse cystic lung diseases (DCLDs) are a group of pathophysiologically heterogenous processes that are characterized by the presence of multiple spherical or irregularly shaped, thin-walled, air-filled spaces within the pulmonary parenchyma. Although the mechanisms of cyst formation remain incompletely defined for all DCLDs, in most cases lung remodeling associated with inflammatory or infiltrative processes results in displacement, destruction, or replacement of alveolar septa, distal airways, and small vessels within the secondary lobules of the lung. The DCLDs can be broadly classified according to underlying etiology as those caused by low-grade or high-grade metastasizing neoplasms, polyclonal or monoclonal lymphoproliferative disorders, infections, interstitial lung diseases, smoking, and congenital or developmental defects. In the first of a two-part series, we present an overview of the cystic lung diseases caused by neoplasms, infections, smoking-related diseases, and interstitial lung diseases, with a focus on lymphangioleiomyomatosis and pulmonary Langerhans cell histiocytosis. PMID:25906089

  16. Diffuse Cystic Lung Diseases: Diagnostic Considerations.

    Science.gov (United States)

    Xu, Kai-Feng; Feng, Ruie; Cui, Han; Tian, Xinlun; Wang, Hanping; Zhao, Jing; Huang, Hui; Zhang, Weihong; Lo, Bee Hong

    2016-06-01

    Diffuse cystic lung disease (DCLD) is a group of heterogeneous diseases that present as diffuse cystic changes in the lung on computed tomography of the chest. Most DCLD diseases are rare, although they might resemble common diseases such as emphysema and bronchiectasis. Main causes of DCLD include lymphangioleiomyomatosis, Birt-Hogg-Dubé syndrome, pulmonary Langerhans cell histiocytosis, lymphoid interstitial pneumonia, amyloidosis, light-chain deposition disease, Sjögren syndrome, and primary or metastatic neoplasm. We discuss clinical factors that are helpful in the differential diagnosis of DCLDsuch as sex and age, symptoms and signs, extrapulmonary presentations, cigarette smoking, and family history. Investigations for DCLD include high-resolution computed tomography, biochemical and histopathological studies, genetic tests, pulmonary function tests, and bronchoscopic and video-assisted thoracoscopic biopsies. A proposed diagnostic algorithm would enhance ease of diagnosing most cases of DCLD. PMID:27231867

  17. Parotid Cystic Lesion in Amelanotic Malignant Melanoma.

    Science.gov (United States)

    Santos, Vitorino Modesto Dos; Gondim Neto, Manoel da Costa; de Melo, Tereza Rodrigues de Carvalho Vieira; Motta, Iara Machado

    2016-09-01

    A 60-year Brazilian woman, presented with an enlarged lymph node in the neck for one year, and a superficial nonulcerated lesion was observed in the scalp. Fine needle aspiration and biopsy of the lymph node revealed amelanocytic metastasis, and immunohistochemistry study showed Melan-A/ Mart-1 antigen (clone A103 and S-100 protein). The entire suspected area of the scalp was further resected and an amelanotic melanoma without angiolymphatic invasion was diagnosed. Ultrasonography and PET-computed tomography showed hypermetabolic cystic area in the right parotid. Furthermore, aspiration biopsy and surgical samples from parotid cyst confirmed the malignant amelanotic melanoma. Cystic metastases are scarcely reported in parotid gland, and can pose diagnostic challenges. PMID:27671185

  18. 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.

  19. Exacerbations in cystic fibrosis: 2 · Prevention

    OpenAIRE

    Bell, Scott C; Robinson, Philip J

    2007-01-01

    The life span of people with cystic fibrosis (CF) has increased dramatically over the past 50 years. Many factors have contributed to this improvement. Respiratory exacerbations of CF lung disease are associated with the need for hospitalisation and antibiotic treatment, reduction in the quality of life, fragmented sleep and mortality. A number of preventive treatment strategies have been developed to reduce the frequency and severity of respiratory exacerbations in CF including mucolytic age...

  20. Supratentorial cystic intracranial lesions: MR imaging features

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Young Joo; Son, Young Bo; Choi, Kyu Ho; Chun, Kyung Ah; Kim, Sung Hoon; Park, Seog Hee; Shinn, Kyung Sub [The Catholic University of Korea College of Medicine, Seoul (Korea, Republic of)

    1997-01-01

    To describe MR findings and differential points of supratentorial cystic intracranial lesions. We retrospectively reviewed and analyzed the MR findings of 59 patients with supratentorial cystic intracranial lesions, and classified them as follows : tumor-associated cyst, infectious cyst, ex-vacuo type cyst, and congentital/developmental cyst. Among 59 patients, 47 tumor-associated cysts were seen in 17, 42 infectious cysts in 13, 17 ex-vacuo type cysts in 10, and 19 congenital/developmental cysts in 19. In 44 of 47 tumor-associate cysts, increased or inhomogeneous internal signal intensity was seen on T1-weighted image, 37 of 47 showed thick uneven walls ; 37 of 47 had enhancing solid components and there was variable perifocal edema and mass effect. Infectious cysts were multiple (11 of 13). In cases of brain abscess, increased internal signal intensity on T1-weighted image, low signal intensity of abscess wall on T2-weighted image, thick even enhancing wall, and marked perifocal edema (4 of 4) were seen in all four cases. Cysts in cysticercosis were variable in appearance depending on the stage, but were smaller than other cystic lesions. Ex vacuo type cysts were of uniform CSF signal intensity in all pulse sequences and there was no identifiable wall or enhancement associated with enlarged adjacent ventricle and encephalomalacia (17 of 17). Congenital/developmental cysts showed a single lesion (19 of 19), a signal intensity similar to CSF in all pulse sequences (15 of 19), no identifiable wall (16 of 19), no enhancement (17 of 19), and no perifocal edema (19 of 19). MR was used to categorize supratentorial cystic intracranial lesions into four groups on the basis of their number, size, internal homogeneity of signal intensity on T1-weighted image, enhancing pattern, perifocal edema and mass effect, thereby improving diagnostic specificity and patient management.

  1. Cystic Fibrosis in a Hispanic Adolescent

    OpenAIRE

    Lin, Jenny H.; Collaco, Joseph M.; Paranjape, Shruti M.

    2013-01-01

    We describe the presentation of a Hispanic adolescent with chronic respiratory symptoms and poor growth that led to a diagnosis of cystic fibrosis (CF) based on an indeterminate sweat chloride result and DNA sequence analysis that revealed a single new frameshift mutation, Nt3878insATCAG, which results in a premature stop codon in exon 20 of the CFTR gene. This case, highlighted by the identification of a deleterious, disease-causing mutation, illustrates the importance of maintaining both a ...

  2. MR imaging of pancreas in cystic fibrosis

    International Nuclear Information System (INIS)

    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.)

  3. Vitamin D Deficiency in Cystic Fibrosis

    OpenAIRE

    Hall, William B.; Sparks, Amy A.; Aris, Robert M

    2010-01-01

    Cystic Fibrosis is the most common inherited genetic respiratory disorder in the Western World. Hypovitaminosis D is almost universal in CF patients, likely due to a combination of inadequate absorption, impaired metabolism, and lack of sun exposure. Inadequate levels are associated with the high prevalence of bone disease or osteoporosis in CF patients, which is associated with increased morbidity including fractures, kyphosis, and worsening pulmonary status. Treatment goals include regular ...

  4. Prevalence and pattern of cystic kidney diseases in Ilorin, Nigeria

    OpenAIRE

    Chijioke Adindu; Aderibigbe Ademola; Olarenwaju Timothy; Makusidi Aliyu; Oguntoyinbo Adewale

    2010-01-01

    Cystic kidney disease is an important cause of chronic renal failure. Since the utili-zation of imaging techniques in the diagnosis of diseases has become widespread, cystic kidney disease is now being increasingly diagnosed. This study is designed to determine the prevalence and pattern of cystic kidney disease at the Nephrology Unit of University of Ilorin Teaching Hospital (UITH), Ilorin. All consecutive adult patients seen in the Nephrology Unit of UITH during a ten-year period (January 1...

  5. Hypertonic Saline in Treatment of Pulmonary Disease in Cystic Fibrosis

    OpenAIRE

    Reeves, Emer P; Kevin Molloy; Kerstin Pohl; McElvaney, Noel G

    2012-01-01

    The pathogenesis of lung disease in cystic fibrosis is characterised by decreased airway surface liquid volume and subsequent failure of normal mucociliary clearance. Mucus within the cystic fibrosis airways is enriched in negatively charged matrices composed of DNA released from colonizing bacteria or inflammatory cells, as well as F-actin and elevated concentrations of anionic glycosaminoglycans. Therapies acting against airway mucus in cystic fibrosis include aerosolized hypertonic saline....

  6. Pulmonary abnormalities in obligate heterozygotes for cystic fibrosis.

    OpenAIRE

    Davis, P B; Vargo, K

    1987-01-01

    Parents of children with cystic fibrosis have been reported to have a high prevalence of increased airway reactivity, but these studies were done in a select young, healthy, symptomless population. In the present study respiratory symptoms were examined in 315 unselected parents of children with cystic fibrosis and 162 parents of children with congenital heart disease (controls). The cardinal symptom of airway reactivity, wheezing, was somewhat more prevalent in cystic fibrosis parents than i...

  7. Challenges in pulmonary fibrosis · 3: Cystic lung disease

    OpenAIRE

    Cosgrove, Gregory P.; Frankel, Stephen K.; Kevin K. Brown

    2007-01-01

    Cystic lung disease is a frequently encountered problem caused by a diverse group of diseases. Distinguishing true cystic lung disease from other entities, such as cavitary lung disease and emphysema, is important given the differing prognostic implications. In this paper the features of the cystic lung diseases are reviewed and contrasted with their mimics, and the clinical and radiographic features of both diffuse (pulmonary Langerhans' cell histiocytosis and lymphangioleiomyomatosis) and f...

  8. Management of Mucin-Producing Cystic Neoplasms of the Pancreas

    OpenAIRE

    Fritz, Stefan; Warshaw, Andrew L.; Thayer, Sarah P.

    2009-01-01

    During the last decade small lesions of the pancreas have been increasingly recognized in clinical practice. Among these lesions, mucin-producing cystic neoplasms represent a recently described and unique entity among pancreatic tumors. In 1996, the World Health Organization distinguished two different types of mucinous cystic tumors: intraductal papillary mucinous neoplasms (IPMNs) of the pancreas, which are characterized by mucin production, cystic dilation of the pancreatic ducts, and intr...

  9. Immature Teratoma after Three Laparoscopic Resections for Mature Cystic Teratomas

    OpenAIRE

    Kazuhiro Nishioka; Naoto Furukawa; Taketoshi Noguchi; Hirotaka Kajihara; Kiyoshige Horie

    2014-01-01

    We report a case in which an immature teratoma developed following three previous resections for mature cystic teratomas. The patient was a 26-year-old nulliparous woman with a regular menstrual cycle. Twelve years earlier, she had consulted a pediatrician for complaints of lower abdominal pain. Bilateral cystic teratomas were suspected and she underwent a left salpingo-oophorectomy and a right cystectomy laparoscopically, and bilateral mature cystic teratomas were diagnosed histologically. S...

  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. A cystic hygroma case associated with Turner’s syndrome

    OpenAIRE

    Balat, Dr. Özcan; Atmaca, Dr. Ruşen; Balat, Dr. Ayşe; Burak, Dr. Feza; Saraç, Dr. Kaya

    1997-01-01

    Cystic hygroma is a congenital anomaly and its incidence is 1.7-5%. It is seen together with chromosomal abnormalities and usually associated with Turner syndrome. Prenatal diagnosis is possible earlier in pregnancy with ultrasonography. We have discussed a case with cystic hygroma diagnosed prenatally. [Journal of Turgut Ozal Medical Center 1997;4(2): 206-208] Key words: Cystic hygroma, prenatal diagnosis, Turner's syndrome

  12. 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.

  13. Progress in therapies for cystic fibrosis.

    Science.gov (United States)

    De Boeck, Kris; Amaral, Margarida D

    2016-08-01

    Standard follow-up and symptomatic treatment have allowed most patients with cystic fibrosis to live to young adulthood. However, many patients still die prematurely from respiratory insufficiency. Hence, further investigations to improve these therapies are important and might have relevance for other diseases-eg, exploring how to increase airway hydration, how to safely downscale the increased inflammatory response in the lung, and how to better combat lung infections associated with cystic fibrosis. In parallel, development of modulators that target the underlying dysfunction in the cystic fibrosis transmembrane conductance regulator (CFTR) is fast moving forward. Existing treatments are specific to certain mutations, or mutation class, in CFTR. An effective, although not yet entirely corrective, treatment is available for patients with class III mutations, and a treatment with modest effectiveness is available for patients who are homozygous for Phe508del, albeit at a very high cost. Corrective treatments that are non-specific to mutation class and thus applicable to all patients-eg, gene therapy, cell-based therapies, and activation of alternative ion channels that bypass CFTR-are being explored, but they are still in early stages of development. In view of the large number of patients with very rare mutations, a plan to advance personalised biomarkers to predict treatment effect is also being investigated and validated. PMID:27053340

  14. Characterizing Mucous Cell Remodeling in Cystic Fibrosis: Relationship to Neutrophils

    OpenAIRE

    Hays, Steven R.; John V Fahy

    2006-01-01

    Rationale: Relatively few studies have characterized mucous cells or mucins in detail in cystic fibrosis (CF), and the relationship between mucous cell abnormalities and neutrophilic inflammation is uncertain.

  15. Genetic modifiers of nutritional status in cystic fibrosis1234

    OpenAIRE

    Bradley, Gia M; Blackman, Scott M.; Christopher P Watson; 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.

  16. [Insights into cystic fibrosis-related bone disease].

    Science.gov (United States)

    Braun, C; Bacchetta, J; Reix, P

    2016-08-01

    With the increasing life expectancy of patients with cystic fibrosis (CF), prevalence of late complications such as CF-related bone disease (CFBD) has increased. It was initially described in 24% of the adult population with CF and has also been reported in the pediatric population. CFBD is multifactorial and progresses in different steps. Both decreased bone formation and increased bone resorption (in different amounts) are observed. CFBD is likely primitive (directly related to the CFTR defect itself), but is also worsened by acquired secondary factors such as lung infections, chronic inflammation, denutrition, vitamin deficiency, and decreased physical activity. CFBD may be clinically apparent (i.e., mainly vertebral and costal fractures), or clinically asymptomatic (therefore corresponding to abnormalities in bone density and architecture). CFBD management mainly aims to prevent the occurrence of fractures. Prevention and regular monitoring of bone disease as early as 8 years of age is of the utmost importance, as is the control of possible secondary deleterious CFBD factors. New radiological tools, such as high-resolution peripheral quantitative computed tomography, allow an accurate evaluation of cortical and trabecular bone micro-architecture in addition to compartmental density; as such, they will likely improve the assessment of the bone fracture threat in CF patients in the near future. PMID:27345551

  17. Lack of correlation between pulmonary disease and cystic fibrosis transmembrane conductance regulator dysfunction in cystic fibrosis: a case report

    OpenAIRE

    Cleveland Robert H; García Christopher; Asher Daniel; Cannon Carolynn L; Levy Hara; Pier Gerald B; Knowles Michael R; Colin Andrew A

    2010-01-01

    Abstract Introduction Mutations in both alleles of the cystic fibrosis transmembrane conductance regulator gene result in the disease cystic fibrosis, which usually manifests as chronic sinopulmonary disease, pancreatic insufficiency, elevated sodium chloride loss in sweat, infertility among men due to agenesis of the vas deferens and other symptoms including liver disease. Case presentation We describe a pair of African-American brothers, aged 21 and 27, with cystic fibrosis. They were homoz...

  18. Minimally invasive surgery colloidal cystic thyroid nodules

    Directory of Open Access Journals (Sweden)

    V G Petrov

    2013-12-01

    Full Text Available An analysis of the use of minimally invasive interventions - laser-induced thermotherapy (LITT, ethanol destruction (ED under ultrasound guidance, a combination of these methods in the treatment of сystic-col- loidal nodes (CCN of thyroid nodules in 120 patients. ED carried out in 31 patients with cystic destruction of a site of more than 70%. Marked decrease in the average volume with 4.68 ± 2.25 cm3 to 1.15 ± 0.9 cm3. In 4 patients remained cystic cavity of small size, which required a combination of LITT (CombiLITT. Nine patients underwent LITT solid residue of more than 1 cm3. The average size of nodules within 6 months after the primary surgery was 0.9 ± 0.3 cm3. СombiLITT performed in 42 patients with cystic cavity from 20 to 70%. The volume of units decreased from 7.98 ± 4.56 to 1.87 ± 0.9 cm3. After 3 months, 11 patients with the size of a solid residue of more than 1 cm3 held LITT. All of them when viewed after 3 months observed decrease of more than 50%. LITT performed 23 patients with major nodes and cystic cavities less than 20% of the node. Marked decrease in the amount of nodes with 2.89 ± 1.25 cm3 to 1.42 ± 0.8 cm3. Retesting LITT took 2 patients. After 6 months, the average amount of units was 0.7 ± 0.2 cm3 LITT conducted in 24 patients with small cystic nodes to 1 cm3. Marked decrease in size from 0.69 ± 0.23 cm3 to 0.29 ± 0.12 cm3. Varied selection of minimally inva- sive treatment of CCN is effective and in most cases, to achieve reduction of the size of nodes.

  19. Lack of correlation between pulmonary disease and cystic fibrosis transmembrane conductance regulator dysfunction in cystic fibrosis: a case report

    Directory of Open Access Journals (Sweden)

    Cleveland Robert H

    2010-04-01

    Full Text Available Abstract Introduction Mutations in both alleles of the cystic fibrosis transmembrane conductance regulator gene result in the disease cystic fibrosis, which usually manifests as chronic sinopulmonary disease, pancreatic insufficiency, elevated sodium chloride loss in sweat, infertility among men due to agenesis of the vas deferens and other symptoms including liver disease. Case presentation We describe a pair of African-American brothers, aged 21 and 27, with cystic fibrosis. They were homozygous for a rare frameshift mutation in the cystic fibrosis transmembrane conductance regulator 3791delC, which would be expected to cause significant morbidity. Although 80% of cystic fibrosis patients are colonized with Pseudomonas aeruginosa by eight years of age, the older brother had no serum opsonic antibody titer to P. aeruginosa by age 13 and therefore would have failed to mount an effective antibody response to the alginate (mucoid polysaccharide capsule of P. aeruginosa. He was not colonized with P. aeruginosa until 24 years of age. Similarly, the younger brother was not colonized with P. aeruginosa until age 20 and had no significant lung disease. Conclusion Despite a prevailing idea in cystic fibrosis research that the amount of functional cystic fibrosis transmembrane conductance regulator predicts clinical status, our results indicated that respiratory disease severity in cystic fibrosis exhibits phenotypic heterogeneity. If this heterogeneity is, in part, genetic, it is most likely derived from genes outside the cystic fibrosis transmembrane conductance regulator locus.

  20. Acquiring taste in home economics?

    DEFF Research Database (Denmark)

    Stenbak Larsen, Christian

    2015-01-01

    that the pupils were encouraged to use their senses: listen to things frying, touch the meat to check if it was done and taste the food in the process of seasoning it. But while some children learned what the teachers expected: to produce well tasting food, others learned to cook very salty and hot food...... appreciated by the group of boys, and others again learned to stick with their idiosyncrasies when pressured by the teacher. Conclusions: Children were acquiring taste in the home economic lessons, but not only the kind of tastes that the teacher had planned for. This leads to reflections on the very complex...... process of taste acquiring and to a call for further research into taste acquiring in complex real life contexts as home economics lessons....

  1. And the Winner is - Acquired

    DEFF Research Database (Denmark)

    Henkel, Joachim; Rønde, Thomas; Wagner, Marcus

    2015-01-01

    that then commercialize the innovation. To this end we analyze an innovation game between one incumbent and a large number of entrants. In the first stage, firms compete to develop innovations of high quality. They do so by choosing, at equal cost, the success probability of their R&D approach, where a lower probability...... accompanies higher value in case of success—that is, a more radical innovation. In the second stage, successful entrants bid to be acquired by the incumbent. We assume that entrants cannot survive on their own, so being acquired amounts to a prize in a contest. We identify an equilibrium in which...

  2. Phenotypes selected during chronic lung infection in cystic fibrosis patients: implications for the treatment of Pseudomonas aeruginosa biofilm infections.

    Science.gov (United States)

    Ciofu, Oana; Mandsberg, Lotte F; Wang, Hengzhuang; Høiby, Niels

    2012-07-01

    During chronic lung infection of patients with cystic fibrosis, Pseudomonas aeruginosa can survive for long periods of time under the challenging selective pressure imposed by the immune system and antibiotic treatment as a result of its biofilm mode of growth and adaptive evolution mediated by genetic variation. Mucoidy, hypermutability and acquirement of mutational antibiotic resistance are important adaptive phenotypes that are selected during chronic P. aeruginosa infection. This review dicsusses the role played by these phenotypes for the tolerance of biofilms to antibiotics and show that mucoidy and hypermutability change the architecture of in vitro formed biofilms and lead to increase tolerance to antibiotics. Production of high levels of beta-lactamase impairs penetration of beta-lactam antibiotics due to inactivation of the antibiotic. In conclusion, these data underline the importance of biofilm prevention strategies by early aggressive antibiotic prophylaxis or therapy before phenotypic diversification during chronic lung infection of patients with cystic fibrosis.

  3. Co-morbidity of cystic fibrosis and celiac disease in Scandinavian cystic fibrosis patients

    DEFF Research Database (Denmark)

    Fluge, G; Olesen, H V; Gilljam, M;

    2009-01-01

    BACKGROUND: The co-morbidity of cystic fibrosis (CF) and celiac disease (CD) has been reported sporadically since the 1960s. To our knowledge, this is the first time a systematic screening is performed in a large cohort of CF patients. METHODS: Transglutaminase-IgA (TGA), endomysium-IgA (EMA...

  4. Co-morbidity of cystic fibrosis and celiac disease in Scandinavian cystic fibrosis patients

    DEFF Research Database (Denmark)

    Fluge, Gjermund; Olesen, Hanne Vebert; Giljam, Marita;

    2009-01-01

    Background: The co-morbidity of cystic fibrosis (CF) and celiac disease (CD) has been reported sporadically since the 1960s. To our knowledge, this is the first time a systematic screening is performed in a large cohort of CF patients. Methods: Transglutaminase-IgA (TGA), endomysium-IgA (EMA...

  5. Acquired anhidrosis a case report

    Directory of Open Access Journals (Sweden)

    Nair Laxmi

    1992-01-01

    Full Text Available A 30-year -old man was seen for acquired anhidrosis. There was no systemic disease. Vasomotor functions were normal. Biopsy showed normal sweat glands and ducts. Intravenous injection of neostigmine could produce profuse sweating on the face, trunk and arms. The disorder is likely to be due to a peripheral dysautonomia selectively affecting the sudomotor function.

  6. Acquired anhidrosis a case report

    OpenAIRE

    Nair Laxmi; Beena D; Manohar S

    1992-01-01

    A 30-year -old man was seen for acquired anhidrosis. There was no systemic disease. Vasomotor functions were normal. Biopsy showed normal sweat glands and ducts. Intravenous injection of neostigmine could produce profuse sweating on the face, trunk and arms. The disorder is likely to be due to a peripheral dysautonomia selectively affecting the sudomotor function.

  7. 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.

  8. LUNG TRANSPLANTATION IN PATIENTS WITH CYSTIC-FIBROSIS

    NARCIS (Netherlands)

    MANNES, GPM; VANDERBIJ, W

    1995-01-01

    Worldwide more than 600 heart-lung or lung transplantations have been performed in patients with cystic fibrosis and end-stage respiratory disease. At the University Hospital in Groningen 10 patients with cystic fibrosis underwent bilateral sequential lung transplantation until April 1994. The 1-yea

  9. Typing of Pseudomonas aeruginosa strains in Norwegian cystic fibrosis patients

    DEFF Research Database (Denmark)

    Fluge, G; Ojeniyi, B; Høiby, N;

    2001-01-01

    OBJECTIVES: Typing of Pseudomonas aeruginosa isolates from Norwegian cystic fibrosis (CF) patients with chronic Pseudomonas lung infection in order to see whether cross-infection might have occurred. METHODS: Isolates from 60 patients were collected during the years 1994-98, and typed by pulsed...... between cystic fibrosis patients has occurred....

  10. Radiological Evaluation of Cystic Lesions Presenting as Painful Knee

    Directory of Open Access Journals (Sweden)

    Rajpal Yadav

    2014-06-01

    Conclusion:Painful knee can be a presenting symptom of many cystic lesions of knee. Ultrasound can show the cystic abnormality, but it is only the Magnetic Resonance Imaging which can demonstrate the complete entity. [Cukurova Med J 2014; 39(3.000: 550-558

  11. Biopsy results of Bosniak 2F and 3 cystic lesions

    DEFF Research Database (Denmark)

    Rasmussen, René; Hørlyck, Arne; Nielsen, Tommy Kjærgaard;

    with a cystic change were re-evaluated and re-categorized after the Bosniak classification system. The re-evaluation and re-categorization was performed in consensus by a junior radiologist (RR) and an uro-radiological expert (OG). Results: Biopsies from eighteen Bosniak 2F cystic lesions were...

  12. Cystic duct closure by sealing with bipolar electrocoagulation

    DEFF Research Database (Denmark)

    Schulze, S; Damgaard, B; Jørgensen, Lars Nannestad;

    2010-01-01

    BACKGROUND: Cystic duct leakage after cholecystectomy is not uncommon and is a potentially serious complication. The aim of this study was to assess a bipolar sealing system (LigaSure) for closure of the cystic duct. METHODS: The records from consecutive laparoscopic cholecystectomies performed i...

  13. Infectious and nutritional mechanisms in children with cystic fibrosis

    OpenAIRE

    Diaconu Ramona; Bozomitu Laura; Anton Emil; Popovici Paula; Anton Carmen; Timofte Daniel; Alin Ciobica; Moraru Evelina

    2015-01-01

    Cystic fibrosis is a polymorphic disease characterized by severe genetic dysfunctions. Besides the complex genetic background, most patients with cystic fibrosis also have increased susceptibility to infections and and their nutritional status is affected. Chronic pulmonary infection and gastrointestinal or nutritional abnormalities are characteristics of this disorder. Of our selected 56 subjects, 21.28% presented a pulmonary condition, and 28.57% digestiv...

  14. New insights into pulmonary inflammation in cystic fibrosis

    OpenAIRE

    Rao, S; Grigg, J

    2006-01-01

    Persistent lower airway infection with inflammation is the major cause of morbidity and mortality in cystic fibrosis. This review examines the recent advances in the understanding of airway inflammation in cystic fibrosis, and focuses on the evidence that pulmonary inflammation is, under some circumstances, disassociated from infection, and the potential implications for therapeutic intervention.

  15. Differential points of mediastinal cystic lesion in chest computed tomography

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Seung Jin; Baek, Jang Mi; Song, Jang Hyeon; Seon, Hyun Ju [Dept. of Radiology, Chonnam National University Hospital, Gwangju (Korea, Republic of); Kim, Yun Hyeon [Dept. of Radiology, Chonnam National University Hwasun Hospital, Hwasun (Korea, Republic of)

    2013-10-15

    To find differential diagnostic imaging findings of mediastinal cystic lesions in chest computed tomography. We retrospectively reviewed imaging findings of 70 patients with histopathologically proven mediastinal cystic lesions. They were 33 male and 37 female patients. Among 70 cases, 49 cases were in the anterior mediastinum, 12 cases were in the middle mediastinum, and 9 cases were in the posterior mediastinum. 19 patients had symptoms. Chest discomfort was the most common symptom. When the cystic lesion was located in the anterior mediastinum, and unilocular, the possibility of thymic cyst was the most likely (p < 0.0027). When the cystic lesion was located in the anterior mediastinum and was multilocular with a relatively thick wall, the possibility of a mature cystic teratoma was the most likely (p < 0.001). When the lesion was a high attenuation cystic lesion located around the air-way, the possibility of a bronchogenic cyst was the most likely (p < 0.001). Chest CT gives information about the location, loculation, wall thickness and internal attenuation of mediastinal cystic lesions. And certain details seen on CT imaging can help with the correct diagnosis, especially in the cases of thymic cyst, mature cystic teratoma and bronchogenic cyst.

  16. Microbiological surveillance in patients with cystic fibrosis

    Directory of Open Access Journals (Sweden)

    Paola Gualdi

    2010-06-01

    Full Text Available Pulmonary infections in patients with cystic fibrosis (CF, are a major cause of morbidity and mortality. Prevention, diagnosis and therapy in cystic fibrosis, lead to the necessary collaboration between clinical and laboratory to identify effective strategies and appropriate solutions to address the problems inherent isolation micro-organisms, antibiotic strategies, overcoming of bacterial resistance and other problems management of these patients. The task of the microbiology laboratory and research in quickly and accurately, the agents responsible for these infectious processes, in order to isolate them from material, identify and determine their sensitivity antibiotics. A microbiological surveillance on 34 patients (13 males and 21 females with CF and related to the “Support Services Provincial Trento for the treatment of cystic fibrosis “in the period July 2005 - August 2008, was carried out. 180 Gram positive and 278 of Gram negative bacteria as well as 235 fungi wre collected. Staphylococcus aureus was the most frequently organism found in patients with CF with an incidence of 23% on 156 strains isolated, Pseudomonas aeruginosa was collected 19% of all microorganisms isolated corresponding to 131 strains, Candida albicans is the yeast often isolated with a frequency 22% equal to 149 isolates, Aspergillus fumigatus was isolated at a rate of 8%. From the data we collected and processed has been noted that the local epidemiology of CF patients reflects as reported in the scientific literature and national international consulting, both as a type microorganisms that frequency also isolated compared to age groups. Considering the score of Bartlett as discriminating respiratory fitness of the material, it has been observed that only 32 samples over 327 total (10% would materials insignificant. It follows therefore that the time of sample collection, followed by personnel (physiotherapists dedicated to CF patients, represents a crucial step

  17. A rare case of community acquired Burkholderia cepacia infection presenting as pyopneumothorax in an immunocompetent individual

    Institute of Scientific and Technical Information of China (English)

    Suman S Karanth; Hariharan Regunath; Kiran Chawla; Mukhyaprana Prabhu

    2012-01-01

    Burkholderia cepacia (B. cepacia) infection is rarely reported in an immunocompetent host. It is a well known occurence in patients with cystic fibrosis and chronic granulomatous disease where it increases both morbidity and mortality. It has also been included in the list of organisms causing nosocomial infections in an immunocompetent host, most of them transmitted from the immunocompromised patient in which this organism harbors. We report a rare case of isolation of B. cepacia from the bronchoalveolar lavage fluid of an immunocompetent agriculturist who presented with productive cough and fever associated with a pyopneumothorax. This is the first case of community acquired infection reported in an immunocompetent person in India.

  18. A rare case of community acquired Burkholderia cepacia infection presenting as pyopneumothorax in an immunocompetent individual

    Directory of Open Access Journals (Sweden)

    Suman S Karanth

    2012-02-01

    Full Text Available Burkholderia cepacia (B. cepacia infection is rarely reported in an immunocompetent host. It is a well known occurence in patients with cystic fibrosis and chronic granulomatous disease where it increases both morbidity and mortality. It has also been included in the list of organisms causing nosocomial infections in an immunocompetent host, most of them transmitted from the immunocompromised patient in which this organism harbors. We report a rare case of isolation of B. cepacia from the bronchoalveolar lavage fluid of an immunocompetent agriculturist who presented with productive cough and fever associated with a pyopneumothorax. This is the first case of community acquired infection reported in an immunocompetent person in India.

  19. New insights into cystic fibrosis-related diabetes in children.

    Science.gov (United States)

    Ode, Katie L; Moran, Antoinette

    2013-09-01

    Cystic fibrosis-related diabetes (CFRD) is the most common complication of cystic fibrosis. It is associated with significantly increased morbidity and mortality in adults and children. Adolescents with cystic fibrosis have a much higher prevalence of diabetes than any other similar age population. Glucose abnormalities that precede diabetes are even more common, especially in children younger than 10 years. The pathophysiology of glucose metabolic abnormalities is poorly understood, but insulin insufficiency is clearly the main component. Findings from animal studies have provided insight into the pathophysiology of CFRD, and imply that carbohydrate metabolic abnormalities might begin at much younger ages than was previously thought in patients with cystic fibrosis, and might be related to the basic cystic fibrosis chloride channel defect. In this Review we explore present knowledge of CFRD in children and adolescents, and new data that indicate that the pathophysiology of CFRD begins in very young patients.

  20. Vaccines for preventing infection with Pseudomonas aeruginosa in cystic fibrosis

    DEFF Research Database (Denmark)

    Johansen, Helle Krogh; Gøtzsche, Peter C

    2015-01-01

    BACKGROUND: Chronic pulmonary infection in cystic fibrosis results in progressive lung damage. Once colonisation of the lungs with Pseudomonas aeruginosa occurs, it is almost impossible to eradicate. Vaccines, aimed at reducing infection with Pseudomonas aeruginosa, have been developed....... This is an update of a previously published review. OBJECTIVES: To assess the effectiveness of vaccination against Pseudomonas aeruginosa in cystic fibrosis. SEARCH METHODS: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register using the terms vaccines AND pseudomonas (last search 30...... March 2015). We previously searched PubMed using the terms vaccin* AND cystic fibrosis (last search 30 May 2013). SELECTION CRITERIA: Randomised trials (published or unpublished) comparing Pseudomonas aeruginosa vaccines (oral, parenteral or intranasal) with control vaccines or no intervention in cystic...

  1. Vaccines for preventing infection with Pseudomonas aeruginosa in cystic fibrosis

    DEFF Research Database (Denmark)

    Johansen, H.K.; Gøtzsche, Peter C.; Johansen, Helle Krogh

    2008-01-01

    BACKGROUND: Chronic pulmonary infection in cystic fibrosis results in progressive lung damage. Once colonisation of the lungs with Pseudomonas aeruginosa occurs, it is almost impossible to eradicate. Vaccines, aimed at reducing infection with Pseudomonas aeruginosa, have been developed. OBJECTIVES......: To assess the effectiveness of vaccination against Pseudomonas aeruginosa in cystic fibrosis. SEARCH STRATEGY: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register using the terms vaccines AND pseudomonas (last search May 2008) and PubMed using the terms vaccin* AND cystic...... fibrosis (last search May 2008). SELECTION CRITERIA: Randomised trials (published or unpublished) comparing Pseudomonas aeruginosa vaccines (oral, parenteral or intranasal) with control vaccines or no intervention in cystic fibrosis. DATA COLLECTION AND ANALYSIS: The authors independently selected trials...

  2. Pseudomonal infection in cystic fibrosis: the battle continues.

    Science.gov (United States)

    Elkin, Sarah; Geddes, Duncan

    2003-12-01

    Pseudomonas aeruginosa lung infection is the major cause of morbidity and mortality in patients with cystic fibrosis. Infection usually begins in childhood and is responsible for respiratory failure and death in most patients with cystic fibrosis. The organism triggers an exuberant chronic inflammatory reaction which damages the airways and leads to progressive loss of lung function. Over the last decade significant advances have been made in the understanding of the pathophysiology of cystic fibrosis airways disease. These should assist the development of new and better therapies to treat this pathogen. This review provides an overview of pseudomonal infection in cystic fibrosis, including mechanisms by which the bacteria may colonize the cystic fibrosis airway, persistence of pseudomonal infection and the biofilm mode of growth. Available treatments and possible novel approaches to therapy will be discussed. PMID:15482158

  3. Cystic Fibrosis: Microbiology and Host Response.

    Science.gov (United States)

    Zemanick, Edith T; Hoffman, Lucas R

    2016-08-01

    The earliest descriptions of lung disease in people with cystic fibrosis (CF) showed the involvement of 3 interacting pathophysiologic elements in CF airways: mucus obstruction, inflammation, and infection. Over the past 7 decades, our understanding of CF respiratory microbiology and inflammation has evolved with the introduction of new treatments, increased longevity, and increasingly sophisticated laboratory techniques. This article reviews the current understanding of infection and inflammation and their roles in CF lung disease. It also discusses how this constantly evolving information is used to inform current therapeutic strategies, measures and predictors of disease severity, and research priorities. PMID:27469179

  4. Cystic Fibrosis Diagnosis and Newborn Screening.

    Science.gov (United States)

    Rosenfeld, Margaret; Sontag, Marci K; Ren, Clement L

    2016-08-01

    The diagnosis of cystic fibrosis (CF) has evolved over the past decade as newborn screening has become universal in the United States and elsewhere. The heterogeneity of phenotypes associated with CF transmembrane conductance regulator (CFTR) dysfunction and mutations in the CFTR gene has become clearer, ranging from classic pancreatic-insufficient CF to manifestations in only 1 organ system to indeterminate diagnoses identified by newborn screening. The tools available for diagnosis have also expanded. This article reviews the newest diagnostic criteria for CF, newborn screening, prenatal screening and diagnosis, and indeterminate diagnoses in newborn-screened infants and symptomatic adults. PMID:27469178

  5. Targeting the Root Cause of Cystic Fibrosis.

    Science.gov (United States)

    Trescott, Laura; Holcomb, Joshua; Spellmon, Nicholas; Mcleod, Cathy; Aljehane, Leala; Sun, Fei; Li, Chunying; Yang, Zhe

    2015-01-01

    Cystic Fibrosis (CF) is a serious genetic condition caused by CF transmembrane conductance regulator (CFTR) mutation. CF patients have shortened lifespan due to airway obstruction, infection, and end-stage lung failure. However, recent development in CF therapy suggests a brighter future for CF patients. Targeting specific CFTR mutations aims to potentiate the channel gating activity of impaired CFTR and restore protein trafficking to the plasma membrane. Gene therapy introduces correct CFTR gene into the affected airway epithelium leading to the functional expression of CFTR in CF patients. This review will sum up the current status in CF-cause targeting therapy. PMID:25316272

  6. Nontuberculous Mycobacterial Infections in Cystic Fibrosis.

    Science.gov (United States)

    Martiniano, Stacey L; Nick, Jerry A; Daley, Charles L

    2016-03-01

    Nontuberculous mycobacteria (NTM) are important emerging cystic fibrosis (CF) pathogens, with estimates of prevalence ranging from 6% to 13%. Diagnosis of NTM disease in patients with CF is challenging, as the infection may remain indolent in some, without evidence of clinical consequence, whereas other patients suffer significant morbidity and mortality. Treatment requires prolonged periods of multiple drugs and varies depending on NTM species, resistance pattern, and extent of disease. The development of a disease-specific approach to the diagnosis and treatment of NTM infection in CF patients is a research priority, as a lifelong strategy is needed for this high-risk population. PMID:26857770

  7. Nutrition and Growth in Cystic Fibrosis.

    Science.gov (United States)

    Lusman, Sarah; Sullivan, Jillian

    2016-08-01

    Close attention to nutrition and growth is essential in caring for children with cystic fibrosis (CF). Growth and nutritional status should be monitored as part of routine CF care. Children with CF should achieve growth and nutritional status comparable with that of well-nourished children without CF. Children with CF are at risk for nutritional deficiencies. Optimal nutritional and growth status may be difficult to attain in this population given risk of insufficient caloric intake and likelihood of increased caloric expenditure. Various methods to attain optimal nutritional status may be used, including oral supplementation, behavioral treatment, pharmacotherapy, and enteral nutrition. PMID:27469181

  8. Vitamin D Deficiency in Cystic Fibrosis

    Directory of Open Access Journals (Sweden)

    William B. Hall

    2010-01-01

    Full Text Available Cystic Fibrosis is the most common inherited genetic respiratory disorder in the Western World. Hypovitaminosis D is almost universal in CF patients, likely due to a combination of inadequate absorption, impaired metabolism, and lack of sun exposure. Inadequate levels are associated with the high prevalence of bone disease or osteoporosis in CF patients, which is associated with increased morbidity including fractures, kyphosis, and worsening pulmonary status. Treatment goals include regular monitoring 25 hydroxyvitamin D (25OHD levels with aggressive treatment for those with levels <75 nmol/L (<30 ng/mL. More research is needed to determine optimal supplementation goals and strategies.

  9. Cogema acquires TOTAL's uranium assets

    International Nuclear Information System (INIS)

    On April 28, France's nuclear fuel cycle conglomerate Cogema and petroleum group TOTAL announced a plan in which Cogema will assume ownership of TOTAL's uranium assets worldwide, and as part of the deal, each firm will acquire shares of the other. On June 2, the agreement will be submitted to shareholders and, assuming it is approved, will go into effect this year. The agreement calls for TOTAL to acquire a 10.8-percent share in Cogema, thus becoming its first private sector shareholder, by underwriting a reserve capital increase of FF1.5 billion (approximately US$283 million). In return, Cogema will pay FF2.52 billion for approximately 4.3 percent of TOTAL, as part of a reserve capital increase totalling FF4.07 billion

  10. Occupationally Acquired American Cutaneous Leishmaniasis

    Directory of Open Access Journals (Sweden)

    Maria Edileuza Felinto de Brito

    2012-01-01

    Full Text Available We report two occupationally acquired cases of American cutaneous leishmaniasis (ACL: one accidental laboratory autoinoculation by contaminated needlestick while handling an ACL lesion sample, and one acquired during field studies on bird biology. Polymerase chain reaction (PCR assays of patient lesions were positive for Leishmania, subgenus Viannia. One isolate was obtained by culture (from patient 2 biopsy samples and characterized as Leishmania (Viannia naiffi through an indirect immunofluorescence assay (IFA with species-specific monoclonal antibodies (mAbs and by multilocus enzyme electrophoresis (MLEE. Patients were successfully treated with N-methyl-glucamine. These two cases highlight the potential risks of laboratory and field work and the need to comply with strict biosafety procedures in daily routines. The swab collection method, coupled with PCR detection, has greatly improved ACL laboratory diagnosis.

  11. CNOOC Acquires Oversea Assets Successfully

    Institute of Scientific and Technical Information of China (English)

    Hu Senlin

    2006-01-01

    @@ After last year CNOOC's bidding for buy the US energy company Unocal Corp lost out to the Chevron Corporation, it conducted the crossing-border asset-acquirement again in the beginning of this year. On Jan. 9, 2006,CNOOC Ltd signed a definitive agreement with Nigeria South Atlantic Petroleum Limited (SAPETRO) to acquire a 45 % working interest in an offshore oil developing license OML 130 in Nigeria for US$2.268 billion cash. The purchase will be funded by the internal capital resources of CNOOC Ltd. In which, US$1.75 billion will pay for buying SAPETRO, and the remaining cash will be used to pay for the early operation cost.

  12. Management of antenatally diagnosed pulmonary sequestration associated with congenital cystic adenomatoid malformation

    OpenAIRE

    Samuel, M; BURGE, D.

    1999-01-01

    BACKGROUND—Sequestration with associated cystic adenomatoid malformation is rare. A study was undertaken to determine whether pulmonary sequestration associated with congenital cystic adenomatoid malformation has a more favourable natural history than that of sequestration without associated cystic adenomatoid malformation.
METHODS—An outline of the postnatal work up leading to the management of extralobar or intralobar pulmonary sequestration with congenital cystic ad...

  13. Acquired causes of intestinal malabsorption.

    Science.gov (United States)

    van der Heide, F

    2016-04-01

    This review focuses on the acquired causes, diagnosis, and treatment of intestinal malabsorption. Intestinal absorption is a complex process that depends on many variables, including the digestion of nutrients within the intestinal lumen, the absorptive surface of the small intestine, the membrane transport systems, and the epithelial absorptive enzymes. Acquired causes of malabsorption are classified by focussing on the three phases of digestion and absorption: 1) luminal/digestive phase, 2) mucosal/absorptive phase, and 3) transport phase. Most acquired diseases affect the luminal/digestive phase. These include short bowel syndrome, extensive small bowel inflammation, motility disorders, and deficiencies of digestive enzymes or bile salts. Diagnosis depends on symptoms, physical examination, and blood and stool tests. There is no gold standard for the diagnosis of malabsorption. Further testing should be based on the specific clinical context and the suspected underlying disease. Therapy is directed at nutritional support by enteral or parenteral feeding and screening for and supplementation of deficiencies in vitamins and minerals. Early enteral feeding is important for intestinal adaptation in short bowel syndrome. Medicinal treatment options for diarrhoea in malabsorption include loperamide, codeine, cholestyramine, or antibiotics. PMID:27086886

  14. Magnetic resonance imaging of cystic periventricular leukomalacia

    Energy Technology Data Exchange (ETDEWEB)

    Kadoi, Nobuaki; Nomura, Junko; Nowatari, Masahiko; Ohta, Takeo; Kamohara, Takashi; Yashiro, Kimio (Kitasato Univ., Sagamihara, Kanagawa (Japan). School of Medicine)

    1990-08-01

    A study was performed to assess the values of magnetic resonance (MR) imaging in evaluation and the follow up of patients with cystic periventricular leukomalacia. Ten patients selected for MR imaging were diagnosed as having periventricular cystic lesions based on US scans. The range of gestational ages was 27 to 32 weeks, and the range of birth weights was 927 to 2,046 g. Twenty MR examinations were carried out using a 0.5 T superconducting system (Resona; Yokogawa). On the first MR examinations, taken by 6 months of age, low signal intensity lesions within the periventricular white matter, moderate ventriculomegaly with irregularity of the ventricular wall and delayed myelination were observed. These were the MR findings observed in the subacute stage of PVL. On the second or the third MR examinations, taken after 12 months of age, increased signal intensity in periventricular white matter on T{sub 2} weighted images decreased volume of periventricular white matter and centrum semiovale and the ventriculomagaly with irregularity of ventricular wall were observed. However, progressions of myelination were proved to be not delayed in comparison with age matched controls. These were thought to be the MR findings of late stage of PVL. As the US findings of PVL have good correlation with pathologic changes revealed at autopsy, MR imaging can depict myelination and detect PVL lesion beyond the neonatal period. These observations demonstrate the value of the MR imaging for the follow up of the patients with PVL beyond the time of fontanel closure. (author).

  15. CFTR protein repair therapy in cystic fibrosis.

    Science.gov (United States)

    Quintana-Gallego, Esther; Delgado-Pecellín, Isabel; Calero Acuña, Carmen

    2014-04-01

    Cystic fibrosis is a single gene, autosomal recessive disorder, in which more than 1,900 mutations grouped into 6 classes have been described. It is an example a disease that could be well placed to benefit from personalised medicine. There are currently 2 very different approaches that aim to correct the basic defect: gene therapy, aimed at correcting the genetic alteration, and therapy aimed at correcting the defect in the CFTR protein. The latter is beginning to show promising results, with several molecules under development. Ataluren (PTC124) is a molecule designed to make the ribosomes become less sensitive to the premature stop codons responsible for class i mutations. Lumacaftor (VX-809) is a CFTR corrector directed at class ii mutations, among which Phe508del is the most frequent, with encouraging results. Ivacaftor (VX-770) is a potentiator, the only one marketed to date, which has shown good efficacy for the class iii mutation Gly551Asp in children over the age of 6 and adults. These drugs, or a combination of them, are currently undergoing various clinical trials for other less common genetic mutations. In the last 5 years, CFTR has been designated as a therapeutic target. Ivacaftor is the first drug to treat the basic defect in cystic fibrosis, but only provides a response in a small number of patients. New drugs capable of restoring the CFTR protein damaged by the most common mutations are required.

  16. Cystic echinococcosis in Greece. Past and present*

    Directory of Open Access Journals (Sweden)

    Sotiraki S.

    2010-09-01

    Full Text Available Cystic echinococcosis is a zoonotic disease with a wide geographical distribution, Greece included, and is considered to be a serious problem for the public health and the livestock economy. Although the disease was widespread in Greece since ancient times, cystic echinococcosis was identified as a serious problem around 1970, and since then national surveillance programmes are running, based on meat inspection and stray dogs management. Ever since, there are official records of the parasite’s prevalence in humans and livestock which show a continuous decline. More precisely, human hydatidosis, according to the official records, declines from an annual incidence of 14.8 per 100,000 inhabitants during 1967-1971 to 0.3 in 2008. Late surveys reveal that in Greece the prevalence of echinococcosis was 23-39.2 % for sheep, 7.6-14.7 % for goats, 0 % in cattle and 0.6 % in pigs, while further molecular analyses in Southern Greece showed the existence of the genotypes G1 and G3 in sheep and G7 in goats in that area. All data presented demonstrate that the parasite is still present in Greece. Surveillance is nowadays being performed under EU regulations but it is highly important to improve and adopt corrective and preventive measures to avoid animal and human infection.

  17. Cystic lung disease: Achieving a radiologic diagnosis

    Energy Technology Data Exchange (ETDEWEB)

    Trotman-Dickenson, Beatrice, E-mail: btrotmandickenson@partners.org

    2014-01-15

    Diffuse cystic lung disease represents a diverse group of uncommon disorders with characteristic appearance on high resolution CT imaging. The combination of imaging appearance with clinical features and genetic testing where appropriate permits a confident and accurate diagnosis in the majority of the diseases without recourse for open lung biopsy. The mechanism of cyst development disease is unclear but in some disorders appears to be related to small airways obstruction. These diseases are incurable, with the exception of Langerhans cell histiocytosis which may spontaneously remit or resolve on smoking cessation. Disease progression is unpredictable; in general older patients have a more benign disease, while young patients may progress rapidly to respiratory failure. An understanding of the complications of cystic lung disease and the appearance of disease progression is essential for the management of these patients. A number of these disorders are associated with malignancy, recognition of the potential tumors permits appropriate imaging surveillance. Due to the widespread use of CT, pulmonary cysts are increasingly discovered incidentally in an asymptomatic individual. The diagnostic challenge is to determine whether these cysts represent an early feature of a progressive disease or have no clinical significance. In the elderly population the cysts are unlikely to represent a progressive disease. In individuals <50 years further evaluation is recommended.

  18. Targeting ion channels in cystic fibrosis.

    Science.gov (United States)

    Mall, Marcus A; Galietta, Luis J V

    2015-09-01

    Mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene cause a characteristic defect in epithelial ion transport that plays a central role in the pathogenesis of cystic fibrosis (CF). Hence, pharmacological correction of this ion transport defect by targeting of mutant CFTR, or alternative ion channels that may compensate for CFTR dysfunction, has long been considered as an attractive approach to a causal therapy of this life-limiting disease. The recent introduction of the CFTR potentiator ivacaftor into the therapy of a subgroup of patients with specific CFTR mutations was a major milestone and enormous stimulus for seeking effective ion transport modulators for all patients with CF. In this review, we discuss recent breakthroughs and setbacks with CFTR modulators designed to rescue mutant CFTR including the common mutation F508del. Further, we examine the alternative chloride channels TMEM16A and SLC26A9, as well as the epithelial sodium channel ENaC as alternative targets in CF lung disease, which remains the major cause of morbidity and mortality in patients with CF. Finally, we will focus on the hurdles that still need to be overcome to make effective ion transport modulation therapies available for all patients with CF irrespective of their CFTR genotype. PMID:26115565

  19. Cystic lung disease: Achieving a radiologic diagnosis

    International Nuclear Information System (INIS)

    Diffuse cystic lung disease represents a diverse group of uncommon disorders with characteristic appearance on high resolution CT imaging. The combination of imaging appearance with clinical features and genetic testing where appropriate permits a confident and accurate diagnosis in the majority of the diseases without recourse for open lung biopsy. The mechanism of cyst development disease is unclear but in some disorders appears to be related to small airways obstruction. These diseases are incurable, with the exception of Langerhans cell histiocytosis which may spontaneously remit or resolve on smoking cessation. Disease progression is unpredictable; in general older patients have a more benign disease, while young patients may progress rapidly to respiratory failure. An understanding of the complications of cystic lung disease and the appearance of disease progression is essential for the management of these patients. A number of these disorders are associated with malignancy, recognition of the potential tumors permits appropriate imaging surveillance. Due to the widespread use of CT, pulmonary cysts are increasingly discovered incidentally in an asymptomatic individual. The diagnostic challenge is to determine whether these cysts represent an early feature of a progressive disease or have no clinical significance. In the elderly population the cysts are unlikely to represent a progressive disease. In individuals <50 years further evaluation is recommended

  20. [CYSTIC FIBROSIS: CARE OF THE LUNG DISEASE].

    Science.gov (United States)

    Hubert, Dominique

    2015-10-01

    (Rh-DNase) and/or hydration (hypertonic saline) nebulisations, Moreover, treatment with inhaled antibiotics is indicated (tobramycin, colistine or aztreonam lysine) for chronic lung infection with Pseudomonas aeruginosa (PA). The treatment regimen also includes bronchodilators for bronchospasms and azithromycin. Regular physical activity is recommended. A treatment potentiating the CFTR protein, ivacaftor, is now indicated for patients with a class 3 mutation. Initial bronchial infection with PA must be treated as soon as possible in order to eradicate the pathogen. Pulmonary exacerbations require antibiotic courses, either orally or intravenously for PA infection. Complications require hospitalisation, with thoracic chest tube placement for a pneumothorax or bronchial artery embolisation for massive hemoptysis. Oxygen therapy and non-invasive ventilation with a nasal mask become necessary when respiratory insufficiency progresses, justifying the initiation of the lung transplant process. Lung disease affects the prognosis of cystic fibrosis, therefore its management in cystic fibrosis centres is of utmost importance. Maintenance treatment mainly relies on daily chest physiotherapy, which can be facilitated by mucolytic PMID:26749716

  1. Pancreatic pseudocyst or a cystic tumor of the pancreas?

    Institute of Scientific and Technical Information of China (English)

    Mohammad Ezzedien Rabie; Ismail El Hakeem; Mohammad Saad Al Skaini; Ahmad El Hadad; Salim Jamil; Mian Tahir Shah; Mahmoud Obaid

    2014-01-01

    Pancreatic pseudocysts are the most common cystic lesions of the pancreas and may complicate acute pancreatitis, chronic pancreatitis, or pancreatic trauma. While the majority of acute pseudocysts resolve spontaneously, few may require drainage. On the other hand, pancreatic cystic tumors, which usualy require extirpation, may disguise as pseudocysts. Hence, the distinction between the two entities is crucial for a successful outcome. We conducted this study to highlight the fundamental differences between pancreatic pseudocysts and cystic tumors so that relevant management plans can be devised. We reviewed the data of patients with pancreatic cystic lesions that underwent intervention between June 2007 and December 2010 in our hospital. We identified 9 patients (5 males and 4 females) with a median age of 40 years (range, 30-70 years). Five patients had pseudocysts, 2 had cystic tumors, and 2 had diseases of undetermined pathology. Pancreatic pseudocysts were treated by pseudocystogastrostomy in 2 cases and percutaneous drainage in 3 cases. One case recurred after percutaneous drainage and required pseudocystogastrostomy. The true pancreatic cysts were serous cystadenoma, which was treated by distal pancreatectomy, and mucinous cystadenocarcinoma, which was initialy treated by drainage, like a pseudocyst, and then by distal pancreatectomy when its true nature was revealed. We conclude that every effort should be exerted to distinguish between pancreatic pseudocysts and cystic tumors of the pancreas to avoid the serious misjudgement of draining rather than extirpating a pancreatic cystic tumor. Additionaly, percutaneous drainage of a pancreatic pseudocyst is a useful adjunct that may substitute for surgical drainage.

  2. Prevalence and pattern of cystic kidney diseases in Ilorin, Nigeria

    Directory of Open Access Journals (Sweden)

    Chijioke Adindu

    2010-01-01

    Full Text Available Cystic kidney disease is an important cause of chronic renal failure. Since the utili-zation of imaging techniques in the diagnosis of diseases has become widespread, cystic kidney disease is now being increasingly diagnosed. This study is designed to determine the prevalence and pattern of cystic kidney disease at the Nephrology Unit of University of Ilorin Teaching Hospital (UITH, Ilorin. All consecutive adult patients seen in the Nephrology Unit of UITH during a ten-year period (January 1999-December 2008 were studied for the presence of cystic kidney disease. The results were analyzed with specific reference to age, gender, annual inci-dence, type of cystic disease, location of cyst, mode of presentation, complications and prognosis. A total of 67 out of 436 renal patients (15.4% studied had cystic kidney disease. A progressive annual increase in the number of cases was noticed. The age-range was 20-83 years with a mean of 47.4 +/- 16.2 years and the peak incidence was in the third and sixth decades with male to female ratio of 1.3:1. The types of cystic kidney disease identified in the study were: 26 simple cysts (38.8%, 35 polycystic kidney disease (53.3% and six multicystic kidney disease (8.9%. The most common mode of presentation was abdominal pain followed by hypertension, urinary tract infection, chronic renal failure and palpable abdominal mass, in decreasing order. Our study indicates that cystic kidney disease is not an uncommon problem among our renal patients and the incidence is on the increase. Although, routine screening of family members with cystic kidney disease still remains a contentious issue because the knowledge may evoke anxiety in terms of employment and insurance, screening of symptomatic cases or those that develop hypertension, hematuria and proteinuria is strongly recommended.

  3. Congenital cystic masses of the face and neck: CT evaluation

    International Nuclear Information System (INIS)

    Recognition of the congenital cystic masses of the face and neck is important because they are usually benign, and can be completely cured by surgical excision. We retrospective analyzed CT scan of 18 surgically proven congenital cystic masses of the face and neck. The cases included 5 thyroglossal duct cysts, 4 cystic hygromas, 5 dermoid cysts, 1 branchial cleft cyst, and 3 fissural cysts of the face. Of five cases of thyroglossal duct cysts, CT showed either a well-marginated, rim enhancing unilocular cystic mass (n=3), or a diffuse but heterogeneous highly enhancing soft tissue mass (n=2). The latter two cases were confirmed later as infected thyroglossal duct cysts. Four cases of cystic hygromas were seen as either an irregularly-marginated (n=3) or a well-marginated (n=1) rim enhancing multiseptated cystic mass with a fluid-fluid level. Five cases of dermoid cysts appeared as well-marginated rim enhancing unilocular ovoid masses. The content of each mass was predominantly fluid in four cases, of which additional solid components were found in two, and interspersed fat globules in one. One case was composed of a homogeneous fatty density. One case of branchial cleft cyst was in the anterior triangle near the left mandibular angle, and appeared as a well-marginated enhancing cystic mass with a thick rim. In this case displacement of the adjacent structures was noted also. All three cases of fissural cysts of the face were seen as a well-marginated, rim enhancing cystic mass, causing a smooth pressure erosion of the adjacent bones. We conclude that CT is useful for the evaluation of the congenital cystic masses of the face and neck, because it can differentiate various forms of the congenital lesions and is able to clearly reveal the relation of the mass to the adjacent structures

  4. Cystic change in primary paediatric optic nerve sheath meningioma.

    Science.gov (United States)

    Narayan, Daniel; Rajak, Saul; Patel, Sandy; Selva, Dinesh

    2016-08-01

    Primary optic nerve sheath meningiomas (PONSM) are rare in children. Cystic meningiomas are an uncommon subgroup of meningiomas. We report a case of paediatric PONSM managed using observation alone that underwent cystic change and radiological regression. A 5-year-old girl presented with visual impairment and proptosis. Magnetic resonance (MR) imaging demonstrated a PONSM. The patient was left untreated and followed up with regular MR imaging. Repeat imaging at 16 years of age showed the tumour had started to develop cystic change. Repeat imaging at 21 years of age showed the tumour had decreased in size. PMID:27310300

  5. Quantitative immunoassays for diagnosis and carrier detection in cystic fibrosis

    International Nuclear Information System (INIS)

    Quantitative immunoprecipitation and immunoradiometric assays have been developed for a protein present in the serum of cystic fibrosis homozygotes, and to a lesser extent in the serum of heterozygotes. When tested on a panel of sera from 14 cystic fibrosis patients, 29 heterozygotes and 23 controls, the immunoprecipitation assay allowed correct assignments to be made on 94% of occasions with one batch of antiserum and 95% with another. With the same panel of sera, the immunoradiometric assay allowed 94% correct assignments. It is suggested that such accuracy is the maximum that can be expected in the present state of knowledge of cystic fibrosis. (author)

  6. Pitfalls in diagnosing a small cystic insulinoma: a case report

    Directory of Open Access Journals (Sweden)

    Sumarac-Dumanovic Mirjana

    2007-12-01

    Full Text Available Abstract Insulinoma is a rare pancreatic endocrine tumour and is typically sporadic and solitary. Over 90% of all insulinomas are benign. Cystic insulinomas are also rare. It is not difficult to determine the site of such neoplasm, as cystic insulinomas are usually 4–10 cm in diameter. We present the case of a patient with a histologically confirmed cystic insulinoma diagnosed after approximately 10 years of hypoglycaemia symptoms. This case is unique because of the small size (2.2 cm of the tumour. Endoscopic ultrasound (EUS was useful for localizing this tumour.

  7. Pneumonia acquired in the Community

    Directory of Open Access Journals (Sweden)

    María Caridad Fragoso Marchante

    2007-06-01

    Full Text Available A bibliographical revision of the main aspects in the diagnosis and treatment of the patients suffering from pneumonia acquired in the community is carried out. Microorganisms responsible for this type of pneumonia are mention in this paper as well as the available diagnostic methods for germs isolation. Different guidelines for diagnosis and treatment of this disease published by several medical societies and scientific institutions are analyzed by means of a review of the stratification index of the patients used in each of them. Aspects related to the duration of the treatment and the possible causes associated with the unfavorable evolution are stated.

  8. Knowledge transfer - Acquiring implicit knowledge

    International Nuclear Information System (INIS)

    Many organisations have recognised the problem of experts taking home a huge amount of specific knowledge, which they have gathered in their department, when they leave. The successor is capable only of acquiring explicit expertise because implicit experiences are not documented and therefore no more available. That is why we have started this pilot study in order to try to conserve the above mentioned tacit and implicit knowledge and to make it available to other colleagues. Using a semi-standardised interview we elicit tacit knowledge from the expert and summarise it in a report. This interview-guideline forms the basis of in-depth investigation for implicit knowledge. (author)

  9. Channel Gating Regulation by the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) First Cytosolic Loop.

    Science.gov (United States)

    Ehrhardt, Annette; Chung, W Joon; Pyle, Louise C; Wang, Wei; Nowotarski, Krzysztof; Mulvihill, Cory M; Ramjeesingh, Mohabir; Hong, Jeong; Velu, Sadanandan E; Lewis, Hal A; Atwell, Shane; Aller, Steve; Bear, Christine E; Lukacs, Gergely L; Kirk, Kevin L; Sorscher, Eric J

    2016-01-22

    In this study, we present data indicating a robust and specific domain interaction between the cystic fibrosis transmembrane conductance regulator (CFTR) first cytosolic loop (CL1) and nucleotide binding domain 1 (NBD1) that allows ion transport to proceed in a regulated fashion. We used co-precipitation and ELISA to establish the molecular contact and showed that binding kinetics were not altered by the common clinical mutation F508del. Both intrinsic ATPase activity and CFTR channel gating were inhibited severely by CL1 peptide, suggesting that NBD1/CL1 binding is a crucial requirement for ATP hydrolysis and channel function. In addition to cystic fibrosis, CFTR dysregulation has been implicated in the pathogenesis of prevalent diseases such as chronic obstructive pulmonary disease, acquired rhinosinusitis, pancreatitis, and lethal secretory diarrhea (e.g. cholera). On the basis of clinical relevance of the CFTR as a therapeutic target, a cell-free drug screen was established to identify modulators of NBD1/CL1 channel activity independent of F508del CFTR and pharmacologic rescue. Our findings support a targetable mechanism of CFTR regulation in which conformational changes in the NBDs cause reorientation of transmembrane domains via interactions with CL1 and result in channel gating.

  10. Pruritic acquired nevus of Ota.

    Science.gov (United States)

    Quenan, S; Strueven, V; Saxer, N; Laffitte, E; Kaya, G; Krischer, J; Hafezi, F; Le Gal, F-A

    2013-01-01

    Nevus of Ota is a unilateral, asymptomatic cutaneous and mucosal hyperpigmentation of the face that is congenital or may appear during childhood. We present a case of symptomatic acquired nevus of Ota in an adult, associated with intense pruritus, not described in the literature so far. A 32-year-old woman presented with brownish mottled macules which appeared on her face progressively over 8 days, following the distribution of the first and second divisions of the left trigeminal nerve and partially covering the iris and sclera of the left eye. She reported an intense pruritus in this area. We performed a biopsy on the left forehead, which confirmed the diagnosis of nevus of Ota. Specific stains and immunohistochemistry revealed increased numbers of mast cells. Ophthalmological tests showed acute acquired melanocytosis of the left iris and sclera. The origin of the nevus is still unclear. Several hypotheses suggest a reactivation of melanocytes during their migration from the neural crest. The pruritus reported in our patient may be explained by the increased quantity of mast cells observed in the lesion and/or neuronal stimulation of the ophthalmic and maxillary divisions of the fifth cranial nerve.

  11. Poly Cystic Ovarian Syndrome: an updated overview

    Directory of Open Access Journals (Sweden)

    Samer eEl Hayek

    2016-04-01

    Full Text Available 1.AbstractPoly 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, 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.

  12. 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. PMID:27092084

  13. Diabetes mellitus in childhood cystic fibrosis.

    LENUS (Irish Health Repository)

    Rauf, F

    2012-02-03

    Since 1984, five patients in the cystic fibrosis (CF) clinic at Cork Regional Hospital have developed diabetes mellitus (DM) and were treated with Insulin. None had received systemic corticosteroids but two had high calorie naso-gastric feeding regimes. Two died from lung disease. A fifteen year old boy developed bilateral cataracts. In nine other paediatric CF clinics in the Republic of Ireland (total: 420 patients), three patients have DM, two receiving Insulin. Abnormal glucose tolerance is becoming more common in CF as patients survive longer. The possible role of corticosteroid treatment and intensive carbohydrate feeding regimes in development of glucose intolerance must be considered. DM in CF differs from the usual childhood DM. Regular screening and early Insulin supplementation may be beneficial.

  14. The diffusing capacity in adult cystic fibrosis.

    Science.gov (United States)

    Espiritu, J D; Ruppel, G; Shrestha, Y; Kleinhenz, M E

    2003-06-01

    The value of adjusting the diffusing capacity for the lung volume has been demonstrated in a large number of patients with other lung diseases but has not been validated in patients with cystic fibrosis (CF). Pulmonary function test results on a cohort of 52 adult CF patients were analyzed to determine whether the diffusing capacity of carbon monoxide by single breath method (DLCO(SB)) when adjusted for alveolar volume (V(A)%), correlated with the severity of pulmonary dysfunction. The DLCO(SB) remained within the reference range except in those with severe lung impairment (61.88 +/- 15.48%). DLCO(SB) has a significant (P V/Q relationship due to claustration in CF. PMID:12814143

  15. Paediatric nasal polyps in cystic fibrosis.

    Science.gov (United States)

    Mohd Slim, Mohd Afiq; Dick, David; Trimble, Keith; McKee, Gary

    2016-01-01

    Patients with cystic fibrosis (CF) are at increased risk of nasal polyps. We present the case of a 17-month-old Caucasian patient with CF who presented with hypertelorism causing cycloplegic astigmatism, right-sided mucoid discharge, snoring and noisy breathing. Imaging suggested bilateral mucoceles in the ethmoid sinuses. Intraoperatively, bilateral soft tissue masses were noted, and both posterior choanae were patent. Polypectomy and bilateral mega-antrostomies were performed. Histological examination revealed inflammatory nasal polyposis typical of CF. The role of early functional endoscopic sinus surgery (FESS) in children with CF nasal polyposis remains questionable as the recurrence rate is higher, and no improvement in pulmonary function has been shown. Our case, however, clearly demonstrates the beneficial upper airway symptom relief and normalisation of facial appearance following FESS in a child with this condition. PMID:27329094

  16. 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-concept...... behaviour and self-concept. Young children with CF (7-10 years) and boys with CF scored significantly higher on anxiety. Girls with CF scored significantly lower on anger than controls. BMI was not associated with any of the BYI subscales. In patients aged 11-14 years, there was a significant correlation...... between FEV(1) and self-concept as well as a significant inverse correlation between FEV(1) and anxiety. CONCLUSIONS: Younger children with CF and boys with CF were more anxious than the healthy controls, and girls with CF expressed less anger than their healthy peers. Effects sizes however were small...

  17. [Nutrition, cystic fibrosis and the digestive tract].

    Science.gov (United States)

    Olveira, Gabriel; Olveira, Casilda

    2008-05-01

    The prevalence of hyponutrition in cystic fibrosis is high although it may vary according to the different studies. Detection of hyponutrition should be done by combining different methods, depending on their availability. However, the simplest and most validated criterion is to measure at each visit the weight (and height in children) in order to calculate the body mass index and categorizing hyponutrition according to absolute criteria: in adults parenteral nutritional support are similar to those used in other pathologies. Dietary and nutritional control should be included in a multidisciplinary program allowing the improvement of the functional capacity and the quality of life and reducing, at least from a theoretical viewpoint, the morbimortality associated to malnourishment in these patients.

  18. Influenza vaccination in children with cystic fibrosis.

    Science.gov (United States)

    Patria, Maria Francesca; Longhi, Benedetta; Esposito, Susanna

    2013-04-01

    Cystic fibrosis (CF) is an inherited autosomal recessive disease characterized by progressive pulmonary damage and respiratory failure. It is known that bacterial infections play a critical role in the development of significant lung damage, whereas the role of respiratory viruses in CF pulmonary exacerbations and the relationship between viral infections and the progression of lung damage are uncertain. Health authorities throughout the world recommend influenza vaccination for CF patients. The aim of this review is to analyze the impact of seasonal and pandemic influenza on CF patients and data concerning influenza vaccination in order to assess the current situation and identify areas for future study. As data are limited, further well-constructed clinical studies of the effectiveness of influenza vaccination on the main clinical outcome measures of pulmonary function and nutritional status in patients with CF are required.

  19. Control programme for cystic echinococcosis in Uruguay

    Science.gov (United States)

    Irabedra, Pilar; Ferreira, Ciro; Sayes, Julio; Elola, Susana; Rodríguez, Miriam; Morel, Noelia; Segura, Sebastian; dos Santos, Estela; Guisantes, Jorge A

    2016-01-01

    Cystic echinococcosis is a highly endemic parasitic zoonosis that is present in the Southern Cone countries of America. For several decades, various prevention and control programmes have been implemented in different countries and regions, with varying results. In Uruguay, a new control programme was implemented in 2006 that employed new strategies for canine diagnosis and treatment, dog population control, diagnosis in humans, epidemiological surveillance, and health education, including community participation. The control programme in Uruguay addresses the control and surveillance of the disease from a holistic perspective based on Primary Health Care, which has strengthened the community’s participation in developing and coordinating activities in an interdisciplinary manner. Similarly, the control programme that is currently implemented is based on a risk-focused approach. The surveillance and control measures were focused on small villages and extremely poor urban areas. In this study, the strategies used and the results obtained from 2008-2013 are analysed and discussed. PMID:27223652

  20. Scoliosis in cystic fibrosis - an appraisal

    International Nuclear Information System (INIS)

    An unusually high prevalence (10%) of scoliosis is described in a series of 151 patients aged four years and older with cystic fibrosis. The scolioses were of the late onset (juvenile and adolescent) type, being typically thoracic with the curve convex to the right, although there was no significant preference for either sex. No direct relationship was found between the spinal curvature and the severity or distribution of the lung disease, although the worse scolioses tended to occur in patients with relatively severe pulmonary involvement. There was no evidence of metabolic bone disease as a predisposing cause. Some indication of a familial tendency towards scoliosis was apparent, and a genetic or constitutional basis is postulated with an unknown precipitating factor. (orig.)

  1. Dosimetry for radiocolloid therapy of cystic craniopharyngiomas

    CERN Document Server

    Rojas, E L; Lallena, A M; Bodineau, C; Galan, P; Al-Dweri, Feras M.O.; Lallena, Antonio M.; Bodineau, Coral; Galan, Pedro

    2003-01-01

    The dosimetry for radiocolloid therapy of cystic craniopharyngiomas is investigated. Analytical calculations based on the Loevinger and the Berger formulae for electrons and photons, respectively, are compared with Monte Carlo simulations. The role of the material of which the colloid introduced inside the craniopharyngioma is made of as well as that forming the cyst wall is analyzed. It is found that the analytical approaches provide a very good description of the simulated data in the conditions where they can be applied (i.e., in the case of a uniform and infinite homogeneous medium). However, the consideration of the different materials and interfaces produces a strong reduction of the dose delivered to the cyst wall in relation to that predicted by the Loevinger and the Berger formulae.

  2. Chronic Rhinosinusitis in Patients with Cystic Fibrosis.

    Science.gov (United States)

    Hamilos, Daniel L

    2016-01-01

    Chronic rhinosinusitis (CRS) is highly prevalent in patients with cystic fibrosis (CF) and accounts for significant morbidity and contribution to CF lung disease. Mutations of the cystic fibrosis transmembrane regulator gene occur with increased prevalence in patients with CRS without CF, suggesting some contribution to CRS pathophysiology. Nasal polyps (NPs) occur with increased prevalence in patients with CF of all ages and have a more neutrophilic appearance with fewer eosinophils and increased submucosal glandular elements in comparison to NPs from patients without CF. Mainstays of medical treatment include isotonic saline irrigations and topical intranasal glucocorticoids, with some evidence that topical intranasal glucocorticoids reduce NP size. Although inhaled hypertonic saline (7%) has been widely studied as a mucolytic agent for CF lung disease, there are no reports of its use in CF CRS. Mucolytics have also not been studied as a treatment for CRS in CF, and most evidence does not support their use for CF lung disease. Nasally nebulized dornase alfa (recombinant human deoxyribonuclease) following sinus surgery shows promise for treatment. Other unproven therapies include addition of baby shampoo to isotonic saline to potentially thin mucus and help prevent biofilm formation. There are no data to support the use of low-dose oral macrolide antibiotics or the use of prophylactic oral antibiotics for CRS in patients with CF. However, there is some support for the use of topical antibiotics, including colistimethate sodium or tobramycin, administered as a sinus irrigation or antral lavage in patients following sinus surgery when susceptible bacteria are cultured. Key components of CF sinus surgical management include extensive surgery to ensure that the maxillary, frontal, sphenoid, and ethmoid sinuses are all widely opened with smoothing of bony overhangs to prevent mucus retention and bacterial recolonization, postoperative meticulous daily nasal irrigations

  3. Human cystic echinococcosis in South Africa

    Directory of Open Access Journals (Sweden)

    Benjamin Mogoye

    2012-06-01

    Full Text Available 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 infection constituting a threat to public health, and is considered an emerging disease around the world. There are at least 10 Echinococcus strain types (G1 – G10, each exhibiting diversity of morphology, development and host range. The epidemiology of CE is poorly understood in South Africa. A retrospective data analysis of the National Health Laboratory Service (NHLS laboratory information system on echinococcosis serology, microscopy and histopathology results in eight provinces (excluding KwaZula-Natal showed an overall positivity rate in submitted diagnostic samples of 17.0% (1056/6211, with the Eastern Cape (30.4%, North West (19.0% and Northern Cape (18.0% provinces showing highest rates. The data showed considerable variability between provinces. The review also showed that most proven cases were negative on serology, implying that the actual number of patients could be underestimated. To our knowledge, no data exist about the prevalent strains of E. granulosus and this prospective study will attempt to fill that gap. The aim is to genotype strains causing the disease in South Africa. Two different polymerase chain reaction (PCR methods will be used to respectively target the 12S rRNA and nad 1 genes. To date, three samples have been genotyped as G1, G5 and G6; suggesting diversity of strains prevalent in the country, but more data is needed for a clearer picture.

  4. Cystic fibrosis Delta F508 heterozygotes, smoking, and reproduction

    DEFF Research Database (Denmark)

    Dahl, Morten; Tybjaerg-Hansen, A; Wittrup, H H;

    1998-01-01

    Cystic fibrosis is the most common fatal autosomal recessive disease affecting Caucasian populations. It remains a puzzle how this disease is maintained at such a remarkably high incidence, however, it could be due to a reproductive advantage in cystic fibrosis heterozygotes. We tested this hypot......Cystic fibrosis is the most common fatal autosomal recessive disease affecting Caucasian populations. It remains a puzzle how this disease is maintained at such a remarkably high incidence, however, it could be due to a reproductive advantage in cystic fibrosis heterozygotes. We tested...... allowing for gender and the potential confounders of age, income, and education. Finally, after allowing for these covariates, number of children in DeltaF508 heterozygotes decreased with increasing extent of smoking (trend test: P = 0.003), while the opposite was true for noncarriers (trend test: P

  5. 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.)

  6. Cystic duct remnant mucocele in a liver transplant recipient

    International Nuclear Information System (INIS)

    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.)

  7. Inflammation in Achromobacter xylosoxidans infected cystic fibrosis patients

    DEFF Research Database (Denmark)

    Hansen, C. R.; Pressler, T.; Nielsen, K. G.;

    2010-01-01

    BACKGROUND: Achromobacter xylosoxidans infection may cause conspicuous chronic pulmonary inflammation in cystic fibrosis (CF) patients similar to Pseudomonas aeruginosa and the Burkholderia cepacia complex (Bcc). Evolution in lung function was compared in chronically infected patients. Cytokine...

  8. Gastrointestinal transit times and motility in patients with cystic fibrosis

    DEFF Research Database (Denmark)

    Hedsund, Caroline; Gregersen, Tine; Jønsson, Iben;

    2012-01-01

    OBJECTIVE: Patients with cystic fibrosis (CF) often suffer from gastrointestinal (GI) dysfunction including obstructive symptoms, malabsorption and pain, but the underlying pathophysiology remains obscure. AIM: To compare GI motility and transit times in CF patients and healthy controls. MATERIAL...

  9. Modular microfluidic system as a model of cystic fibrosis airways

    DEFF Research Database (Denmark)

    Skolimowski, Maciej; Weiss Nielsen, Martin; Abeille, Fabien;

    2012-01-01

    A modular microfluidic airways model system that can simulate the changes in oxygen tension in different compartments of the cystic fibrosis (CF) airways was designed, developed, and tested. The fully reconfigurable system composed of modules with different functionalities: multichannel peristaltic...... pumps, bubble traps, gas exchange chip, and cell culture chambers. We have successfully applied this system for studying the antibiotic therapy of Pseudomonas aeruginosa, the bacteria mainly responsible for morbidity and mortality in cystic fibrosis, in different oxygen environments. Furthermore, we...... have mimicked the bacterial reinoculation of the aerobic compartments (lower respiratory tract) from the anaerobic compartments (cystic fibrosis sinuses) following an antibiotic treatment. This effect is hypothesised as the one on the main reasons for recurrent lung infections in cystic fibrosis...

  10. Immunisation in the current management of cystic fibrosis patients

    DEFF Research Database (Denmark)

    Malfroot, Anne; Adam, Georgios; Ciofu, Oana;

    2005-01-01

    Although no special recommendations exist, clearly patients with cystic fibrosis (CF) can benefit from immunisation. We reviewed the literature regarding vaccination in CF and other chronic diseases. CF subjects should follow national immunisation programmes without delay to obtain optimal...

  11. Interdisciplinary Management of Cystic Neoplasms of the Pancreas

    Directory of Open Access Journals (Sweden)

    Linda S. Lee

    2012-01-01

    Full Text Available Cystic neoplasms of the pancreas are increasingly recognized due to the frequent use of abdominal imaging. It is reported that up to 20% of abdominal cross-sectional scans identify incidental asymptomatic pancreatic cysts. Proper characterization of pancreatic cystic neoplasms is important not only to recognize premalignant lesions that will require surgical resection, but also to allow nonoperative management of many cystic lesions that will not require resection with its inherent morbidity. Though reliable biomarkers are lacking, a wide spectrum of diagnostic modalities are available to evaluate pancreatic cystic neoplasms, including radiologic, endoscopic, laboratory, and pathologic analysis. An interdisciplinary approach to management of these lesions which incorporates recent, specialty-specific advances in the medical literature is herein suggested.

  12. Benign Cystic Peritoneal Mesothelioma Revealed by Small Bowel Obstruction.

    Science.gov (United States)

    Bray Madoué, Kaimba; Boniface, Moifo; Annick Laure, Edzimbi; Pierre, Herve

    2016-01-01

    Benign cystic peritoneal mesothelioma is a rare tumor which frequently occurs in women of reproductive age. Abdominal pain associated with pelvic or abdominal mass is the common clinical presentation. We report the case of a 22-year-old woman with a pathological proved benign cystic mesothelioma of the peritoneum revealed by a small bowel obstruction and a painful left-sided pelvic mass with signs of psoitis. Contrast enhanced abdominal CT-scan demonstrated a large pelvic cystic mass with mass effect on rectosigmoid and pelvic organs. The patient underwent surgical removal of the tumor. Pathological examination revealed the diagnosis of benign cystic mesothelioma of the peritoneum. The outcome was excellent with a 12-month recoil.

  13. Changes in Cystic Fibrosis Airway Microbiota at Pulmonary Exacerbation

    OpenAIRE

    Carmody, Lisa A.; Zhao, Jiangchao; Schloss, Patrick D.; Petrosino, Joseph F; Murray, Susan; Young, Vincent B.; Li, Jun Z.; LiPuma, John J.

    2013-01-01

    Rationale: In persons with cystic fibrosis (CF), repeated exacerbations of pulmonary symptoms are associated with a progressive decline in lung function. Changes in the airway microbiota around the time of exacerbations are not well understood.

  14. Cystic fibrosis in a retro-positive child

    OpenAIRE

    Praveen Sharma; N Arthi; Sagar Bhattad; Kim Vaiphei

    2015-01-01

    We present a rare association of cystic fibrosis and retro positivity in a grossly malnutrited child. The child had pulmonary, pancreatic and colonic manifestations with superadded herpes simplex virus interstitial pneumonia and lymphocytic meningitis.

  15. Sarcoidosis in two patients with cystic fibrosis: a fortuitous association?

    OpenAIRE

    Cooper, T J; Day, A. J.; Weller, P H; Geddes, D. M.

    1987-01-01

    Two cases of cystic fibrosis complicated by sarcoidosis are described. Possible pathogenetic interactions between the two diseases are discussed. The diagnosis of sarcoidosis in patients with chronic pulmonary inflammation is difficult, and if overlooked may lead to inappropriate treatment.

  16. Association between Pulmonary Function and Sputum Biomarkers in Cystic Fibrosis

    OpenAIRE

    Mayer-Hamblett, Nicole; Aitken, Moira L.; Accurso, Frank J.; Kronmal, Richard A.; Konstan, Michael W.; Burns, Jane L.; Sagel, Scott D.; Ramsey, Bonnie W.

    2007-01-01

    Rationale: Sputum biomarkers of infection and inflammation are noninvasive measures that enable quantification of the complex pathophysiology of cystic fibrosis (CF) lung disease. Validation of these biomarkers as correlates of disease severity is a key step for their application.

  17. AEROSOL DEPOSITION AS A FUNCTION OF AIRWAY DISEASE: CYSTIC FIBROSIS

    Science.gov (United States)

    Progressive lung disease associated with cystic fibrosis (CF) is a continuous interaction of the processes of airway obstruction, infection and inflammation. ecent literature has suggested that the manifestation of CF could compromise the successful administration of pharmacologi...

  18. Cystic fibrosis with normal sweat chloride concentration: case report

    Directory of Open Access Journals (Sweden)

    Silva Filho Luiz Vicente Ferreira da

    2003-01-01

    Full Text Available Cystic fibrosis is a genetic disease usually diagnosed by abnormal sweat testing. We report a case of an 18-year-old female with bronchiectasis, chronic P. aeruginosa infection, and normal sweat chloride concentrations who experienced rapid decrease of lung function and clinical deterioration despite treatment. Given the high suspicion ofcystic fibrosis, broad genotyping testing was performed, showing a compound heterozygous with deltaF508 and 3849+10kb C->T mutations, therefore confirming cystic fibrosis diagnosis. Although the sweat chloride test remains the gold standard for the diagnosis of cystic fibrosis, alternative diagnostic tests such as genotyping and electrophysiologic measurements must be performed if there is suspicion of cystic fibrosis, despite normal or borderline sweat chloride levels.

  19. Hydrator Therapies for Chronic Bronchitis. Lessons from Cystic Fibrosis.

    Science.gov (United States)

    Bennett, William D; Henderson, Ashley G; Donaldson, Scott H

    2016-04-01

    Patients with the chronic bronchitis form of chronic obstructive pulmonary disease and cystic fibrosis share similar clinical features, including mucus obstruction of airways and the development of chronic/recurrent airways infections that often manifest as disease exacerbations. There is growing evidence that these diseases may have parallels in disease pathogenesis as well, including cystic fibrosis transmembrane conductance regulator dysfunction, mucus dehydration, and defective mucociliary clearance. As progress is made in the development of therapies that target the basic defects that lead to cystic fibrosis lung disease, it is possible that similar approaches could also benefit patients with chronic bronchitis. A deeper understanding of how tobacco smoke and other triggers of chronic bronchitis actually lead to disease, and exploration of the concept that therapies that restore cystic fibrosis transmembrane conductance regulator function, mucus hydration, and/or mucociliary clearance may benefit patients with chronic bronchitis, hold the prospect of significant progress in treating this prevalent disease. PMID:27115955

  20. Adeno-associated virus for cystic fibrosis gene therapy

    Directory of Open Access Journals (Sweden)

    S.V. Martini

    2011-11-01

    Full Text Available Gene therapy is an alternative treatment for genetic lung disease, especially monogenic disorders such as cystic fibrosis. Cystic fibrosis is a severe autosomal recessive disease affecting one in 2500 live births in the white population, caused by mutation of the cystic fibrosis transmembrane conductance regulator (CFTR. The disease is classically characterized by pancreatic enzyme insufficiency, an increased concentration of chloride in sweat, and varying severity of chronic obstructive lung disease. Currently, the greatest challenge for gene therapy is finding an ideal vector to deliver the transgene (CFTR to the affected organ (lung. Adeno-associated virus is the most promising viral vector system for the treatment of respiratory disease because it has natural tropism for airway epithelial cells and does not cause any human disease. This review focuses on the basic properties of adeno-associated virus and its use as a vector for cystic fibrosis gene therapy.

  1. [Cystic formations of ovaries in women: clinical and morphological features

    Directory of Open Access Journals (Sweden)

    Sorokina I.V.

    2015-06-01

    Full Text Available Background. Cystic formations of ovaries are an urgent problem of medicine due to the high incidence of these diseases in women, the difficulties of differential diagnosis and a high percentage of diagnostic and tactical mistakes leading to disruption of reproductive function. Objective: to identify the clinical and morphological features of cystic formations of ovaries in women of Kharkiv region on the basis of the analysis of archival material. Methods. The material of this study was archival materials of pathological anatomy department of The Municipal Health Care Institution «Regional Clinical Hospital – The Center of Emergency Medicine and Disaster Medicine» during 2013 y. 354 cases of histological examination of surgical material – deleted fragments of ovaries due to cystic formations or ovaries in complex with uterus and fallopian tubes due to leiomyoma of uterus – were analyzed. The slides, stained with hematoxylin and eosin, were studied on the microscope «Olympus BX-41». Digital data were processed using statistical methods of investigation. Results. 1. It was established that in women of Kharkiv region among all cystic formations of ovaries tumor-like processes (in order of frequency of occurrence – yellow bodies cysts, follicular cysts, simple cysts, endometrial cysts occur 5.5 times more frequently in comparison with tumor processes (in order of frequency of occurrence – dermoid cysts, cystadenomas, cystadenocarcinoma. 2. Tumor and tumor-like cystic formations of ovaries occur 4.6 times more frequently in right ovary in comparison with the left. 3. Tumor and tumor-like cystic formations of ovaries were characterized by a certain age. Tumor-like cystic formations of ovaries were typical for younger women (average age of women – 31.03±0.49 year and tumor cystic formations – for older women (average age of women – 37.70±1.53 years. Among all tumor-like cystic formations of ovaries yellow bodies cysts were typical

  2. Brainstem cysticercose simulating cystic tumor lesion: a case report

    Directory of Open Access Journals (Sweden)

    Walter O. Arruda

    1994-09-01

    Full Text Available The authors report the case of a 37 year-old man with a solitary cysticercus cyst in the brainstem (pons successfully removed through a suboccipital craniectomy. Surgery in neurocysticercosis has been indicated in patients with hydrocephalus and/or large cystic lesions. Cystic lesions in the brainstem and spinal cord may have indication for surgery for two reasons: (1 diagnosis; and (2 treatment. Aspects related to differential diagnosis and therapeutic alternatives are discussed.

  3. Pulmonar collision tumor: metastatic adenoid cystic carcinoma and lung adenocarcinoma.

    Science.gov (United States)

    Blanco, M; García-Fontán, E; Ríos, J; Rivo, J E; Fernández-Martín, R; Cañizares, M A

    2012-01-01

    We report an extraordinary case of collision tumor consisting of a lung adenocarcinoma and a metastatic adenoid cystic carcinoma in a 56 year-old man. He was diagnosed with a pulmonary nodule 11 years after treatment of an adenoid cystic carcinoma of the right maxillary sinus. A non-small cell carcinoma was observed when a transbronchial biopsy was performed. The other component of the nodule was only diagnosed with pathological examination of the resection specimen. PMID:21802893

  4. Cystic changes associated with pulmonary tuberculosis: a case report

    Institute of Scientific and Technical Information of China (English)

    CAI Hou-rong; CAO Min; MENG Fan-qing; LI Wei-chun

    2006-01-01

    @@ There are a wide range of computed tomography (CT) findings in patients with pulmonary tuberculosis, including diffuse or localized nodules,reticular opacities, ground glass attenuation, air trapping, consolidation, cavitation, fibrosis, lymph nodes enlargement, and septal thickening.1-3However, CT findings of pulmonary tuberculosis that appeared as multiple cystic lesions were very rare.3,4Herein, the CT findings appeared as multiple cystic lesions in a patient with pulmonary tuberculosis are reported.

  5. Cystic fibrosis school for 10-year-olds

    DEFF Research Database (Denmark)

    Bregnballe, Vibeke; Schiøtz, Peter Oluf

    2000-01-01

    Compliance or rather lack of compliance has always been a problem when treating patients with cystic fibrosis. The patients tend to drop the treatment more or less if the treatment gets too complicated or laboured. We have attempted a systematical education of our patients in a CF school...... give them a good basis on which they can make their own choices about how they want to live their lives with cystic fibrosis and with optimal quality of life....

  6. Vaccines for preventing infection with Pseudomonas aeruginosa in cystic fibrosis

    DEFF Research Database (Denmark)

    Johansen, Helle Krogh; Gøtzsche, Peter C

    2013-01-01

    Chronic pulmonary infection in cystic fibrosis results in progressive lung damage. Once colonisation of the lungs with Pseudomonas aeruginosa occurs, it is almost impossible to eradicate. Vaccines, aimed at reducing infection with Pseudomonas aeruginosa, have been developed.......Chronic pulmonary infection in cystic fibrosis results in progressive lung damage. Once colonisation of the lungs with Pseudomonas aeruginosa occurs, it is almost impossible to eradicate. Vaccines, aimed at reducing infection with Pseudomonas aeruginosa, have been developed....

  7. Phenotypes selected during chronic lung infection in cystic fibrosis patients

    DEFF Research Database (Denmark)

    Ciofu, Oana; Mandsberg, Lotte F; Wang, Hengzhuang;

    2012-01-01

    During chronic lung infection of patients with cystic fibrosis, Pseudomonas aeruginosa can survive for long periods of time under the challenging selective pressure imposed by the immune system and antibiotic treatment as a result of its biofilm mode of growth and adaptive evolution mediated...... the importance of biofilm prevention strategies by early aggressive antibiotic prophylaxis or therapy before phenotypic diversification during chronic lung infection of patients with cystic fibrosis....

  8. Fluoroquinolones in the treatment of bronchopulmonary disease in cystic fibrosis

    OpenAIRE

    2012-01-01

    Fluoroquinolones are commonly used to treat lung infections in those with cystic fibrosis. Patients with cystic fibrosis are susceptible to lung infection with common bacteria such as Staphylococcus aureus and Haemophilus influenzae, but also are prone to infection by opportunistic bacteria, including Pseudomonas aeruginosa. The good oral bioavailability and broad antimicrobial spectrum of activity, including anti-pseudomonal properties make this class of antimicrobial attractive. We review t...

  9. Defective fluid transport by cystic fibrosis airway epithelia.

    OpenAIRE

    Smith, J.J.; Karp, P H; Welsh, M J

    1994-01-01

    Cystic fibrosis (CF) airway epithelia exhibit defective transepithelial electrolyte transport: cAMP-stimulated Cl- secretion is abolished because of the loss of apical membrane cystic fibrosis transmembrane conductance regulator (CFTR) Cl- channels, and amiloride-sensitive Na+ absorption is increased two- to threefold because of increased amiloride-sensitive apical Na+ permeability. These abnormalities are thought to alter respiratory tract fluid, thereby contributing to airway disease, the m...

  10. Serum pancreatic lipase as a screening test for cystic fibrosis.

    OpenAIRE

    Adriaenssens, K; Van Riel, L

    1982-01-01

    Pancreatic lipase catalyses the hydrolysis of emulsified triglycerides to form a transparent solution of monoglycerides and fatty acids. Levels of serum pancreatic lipase were measured in neonates known to have cystic fibrosis and compared with levels in control infants. During the first weeks of life infants with cystic fibrosis had raised serum pancreatic lipase values in parallel with raised serum trypsin values. A simple and specific turbidimetric dried blood spot assay for serum pancreat...

  11. Hamartoma cístico retrorretal Retrorectal cystic hamartoma

    OpenAIRE

    Cleverson Winston de Liz Medeiros; William Kondo; Calixto Antonio Hakim Neto

    2002-01-01

    A 32-year-old woman was referred to our institution for a pelvic mass. She was asymptomatic and was found to have a mass on rectal examination. Magnetic resonance showed a large solid-cystic mass and the patient underwent complete surgical excision with pathologic findings suggesting retrorectal cyst-hamartoma. Tailgut cysts (or retrorectal cystic hamartomas) are rare congenital lesions settled in the retrorectal (presacral) space. They are made up of cysts lined by multiple types of epitheli...

  12. Congenital cystic polypoid rectal hamartoma in a newborn foal.

    Science.gov (United States)

    Dunkel, B; Shokek, A B; Wilkins, P A

    2004-11-01

    A neonatal foal with signs of rectal bleeding was diagnosed with an intraluminal rectal mass and intussusception on surgical exploration of the abdomen. Histologically, the mass consisted of cystic spaces lined by simple columnar epithelium with numerous goblet cells and was surrounded by thin bands of smooth muscle in a myxomatous stroma. Although the mass shared similarities with retrorectal cystic hamartoma (tailgut cyst) and juvenile polyps, described in human medicine, location and histologic findings were not entirely consistent with either condition. PMID:15557082

  13. [Retrorectal cystic hamartoma (tailgut cyst): radiologic and pathologic findings].

    Science.gov (United States)

    Cuartero, V; López, J; Carrero, J; Nevado, M

    2008-01-01

    Cystic hamartoma is a rare congenital lesion that presents as a presacral mass. It arises from remnants of the embryonic postanal gut. It is more common in women and usually presents in middle age. It is usually asymptomatic. It is difficult to distinguish the imaging appearance of cystic hamartoma from that of many other presacral cysts; therefore, histologic analysis is essential for the definitive diagnosis. Complete surgical excision is indicated to establish the diagnosis and avoid complications (infection and malignant transformation). PMID:19055922

  14. Pancreatic pseudocyst or a cystic tumor of the pancreas?

    OpenAIRE

    Mohammad Ezzedien Rabie; Ismail El Hakeem; Mohammad Saad Al Skaini; Ahmad El Hadad; SalimJamil; Mian TahirShah; MahmoudObaid

    2014-01-01

    Pancreatic pseudocysts are the most common cystic lesions of the pancreas and may complicate acute pancreatitis, chronic pancreatitis, or pancreatic trauma. While the majority of acute pseudocysts resolve spontaneously, few may require drainage. On the other hand, pancreatic cystic tumors, which usually require extirpation, may disguise as pseudocysts. Hence, the distinction between the two entities is crucial for a successful outcome. We conducted this study to highlight the fundamental diff...

  15. Assessment of hyperinflation in children with cystic fibrosis.

    OpenAIRE

    Marchant, J; Hansell, D M; Bush, A

    1994-01-01

    BACKGROUND--Radiological estimates of hyperinflation are used in several clinical and radiographic scoring systems for cystic fibrosis, but it is not known if these estimates of hyperinflation are related to measured total lung capacity. METHODS--Comparison was made of independent clinical estimates of hyperinflation from chest radiographs with objective plethysmographic and radiographic measurements of total lung capacity in 25 children with cystic fibrosis. RESULTS--There was good agreement...

  16. Chronic Low Back Pain due to Retroperitoneal Cystic Lymphangioma

    Directory of Open Access Journals (Sweden)

    Asieh Sadat Fattahi

    2014-03-01

    Full Text Available   Abdominal cystic lymphangioma is a rare benign neoplasm. Less than 1% of lymphangiomas is in the retroperitoneum. Lymphangioma is mostly asymptomatic. Chronic symptoms were reported in retroperitoneal type more than others. Acute symptoms due to complications like infection, cyst rupture or hemorrhage may occur. We report an 18-years-old girl with low back pain from 6 months ago with huge pelvic mass and diagnosis of retroperitoneal cystic lymphangioma.

  17. Chronic Low Back Pain due to Retroperitoneal Cystic Lymphangioma

    Directory of Open Access Journals (Sweden)

    Asieh Sadat Fattahi

    2014-03-01

    Full Text Available Abdominal cystic lymphangioma is a rare benign neoplasm. Less than 1% of lymphangiomas is in the retroperitoneum. Lymphangioma is mostly asymptomatic. Chronic symptoms were reported in retroperitoneal type more than others. Acute symptoms due to complications like infection, cyst rupture or hemorrhage may occur. We report an 18-years-old girl with low back pain from 6 months ago with huge pelvic mass and diagnosis of retroperitoneal cystic lymphangioma.

  18. Abdominal manifestations of cystic fibrosis in adults: a review

    International Nuclear Information System (INIS)

    Gastrointestinal manifestations of disease are present in most adults with cystic fibrosis. Radiologists are familiar with the classical imaging characteristics of end-stage pulmonary disease and the radiological findings of meconium ileus in neonates. As most patients now live into adulthood, recognition of the imaging appearances of abdominal disease is important to enable prompt diagnosis and treatment. Accordingly, this article presents typical imaging appearances of the adult gastrointestinal manifestations of cystic fibrosis. Copyright (2004) Blackwell Science Pty Ltd

  19. Allergic bronchopulmonary aspergillosis complicating cystic fibrosis in childhood.

    OpenAIRE

    Brueton, M. J.; Ormerod, L P; Shah, K J; Anderson, C. M.

    1980-01-01

    Allergic bronchopulmonary aspergillosis, known to be associated with cystic fibrosis in older patients, occurred in 7 young atopic children with cystic fibrosis. The diagnosis was suggested by the onset of, or the increase in, asthmatic symptoms accompanied by major chest x-ray changes ranging from total collapse of a lung or lobe to extensive but changing areas of consolidation. Each of the children had a blood eosinophilia, positive type I skin tests to Aspergillus fumigatus, and reversible...

  20. Increased oral bioavailability of ciprofloxacin in cystic fibrosis patients.

    OpenAIRE

    Christensson, B A; Nilsson-Ehle, I; Ljungberg, B; Lindblad, A.; Malmborg, A. S.; Hjelte, L; Strandvik, B

    1992-01-01

    The altered pharmacokinetic properties of, e.g., aminoglycosides in cystic fibrosis patients have to be considered when pulmonary exacerbations are treated. Since reported data on ciprofloxacin, a fluorinated quinolone, are conflicting, we compared intravenous and oral administration in cystic fibrosis patients when treating them for mild symptoms of pulmonary infection. All of the patients were colonized with Pseudomonas species. Ciprofloxacin was administered orally (15 mg/kg of body weight...

  1. Cepacia Syndrome in a Non-Cystic Fibrosis Patient

    OpenAIRE

    Naomi Hauser; Jose Orsini

    2015-01-01

    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 h...

  2. Fecal isolation of Pseudomonas aeruginosa from patients with cystic fibrosis.

    OpenAIRE

    Agnarsson, U; Glass, S; Govan, J R

    1989-01-01

    Fecal isolation of Pseudomonas aeruginosa was observed in 8 of 10 patients with cystic fibrosis who at the time of sampling also exhibited colonization of the respiratory tract. In contrast, P. aeruginosa cells were isolated at low frequency (9.1%) from the stools of 44 patients with cystic fibrosis with no previous history of chronic colonization. The results of this study suggest that the gastrointestinal tract is not a significant chronic reservoir of P. aeruginosa prior to pulmonary colon...

  3. Serum antibodies to Pseudomonas aeruginosa in cystic fibrosis.

    OpenAIRE

    Brett, M M; Ghoneim, A T; Littlewood, J M

    1986-01-01

    Serum IgG antibodies to Pseudomonas aeruginosa cell surface antigens were determined by enzyme linked immunosorbent assay. Titres in patients without cystic fibrosis were low (140-235). Those in patients with cystic fibrosis who were chronically infected by P. aeruginosa were very high (1100-20,500), while patients who grew the organism intermittently had lower titres (160-4400). Longitudinal studies showed that raised titres were observed at a very early stage of infection. High titres were ...

  4. Natural Compounds as Therapeutic Agents in the Treatment Cystic Fibrosis

    OpenAIRE

    Dey, Isha; Shah, Kalpit; Bradbury, Neil A.

    2016-01-01

    The recent FDA approval of two drugs to treat the basic defect in cystic fibrosis has given hope to patients and their families battling this devastating disease. Over many years, with heavy financial investment from Vertex Pharmaceuticals and the Cystic Fibrosis Foundation, pre-clinical evaluation of thousands of synthetic drugs resulted in the production of Kalydeco and Orkambi. Yet, despite the success of this endeavor, many other compounds have been proposed as therapeutic agents in the t...

  5. Celiac Disease and Cystic Fibrosis: Challenges to Differential Diagnosis

    Directory of Open Access Journals (Sweden)

    Ramos Alessandra Teixeira Pessoa

    2016-06-01

    Full Text Available Cystic fibrosis and celiac disease were considered a single clinical entity for many years. Differentiation between the diseases occurred some time in the 1930s of the 20th Century. Both diseases may present the intestinal malabsorption syndrome and similar clinical manifestations that contribute to difficulties with clinical distinction. We describe a report of two patients with initial diagnosis of cystic fibrosis, who were subsequently diagnosed with celiac disease.

  6. Increased PIVKA-II concentrations in patients with cystic fibrosis.

    OpenAIRE

    de Montalembert, M.; Lenoir, G.; Saint-Raymond, A.; Rey, J.; Lefrère, J. J.

    1992-01-01

    Serum vitamin K concentrations and prothrombin induced by absence of vitamin K (PIVK-II) concentrations were assayed in 43 patients with cystic fibrosis. Twenty nine showed a normal PIVKA-II and vitamin K concentrations; 14 showed an increased PIVKA-II concentration, in one of whom serum vitamin K was decreased. Although their vitamin K concentrations were normal, some patients with cystic fibrosis still had an increased PIVKA-II. There was a significant correlation between PIVKA-II concentra...

  7. Cystic Fibrosis-Related Oxidative Stress and Intestinal Lipid Disorders

    OpenAIRE

    Kleme, Marie-Laure; Levy, Emile

    2015-01-01

    Significance: Cystic fibrosis (CF) is the most common lethal genetic disorder in the Caucasian people. It is due to the mutation of cystic fibrosis transmembrane conductance regulator (CFTR) gene located on the long arm of the chromosome 7, which encodes for CFTR protein. The latter, an adenosine triphosphate binding cassette, is a transmembrane chloride channel that is also involved in glutathione transport. As glutathione/glutathione disulfide constitutes the most important pool of cellular...

  8. Microbial ecology and adaptation in cystic fibrosis airways

    DEFF Research Database (Denmark)

    Yang, Lei; Jelsbak, Lars; Molin, Søren

    2011-01-01

    constitute the selective forces that drive the evolution of the microbes after they migrate from the outer environment to human airways. Pseudomonas aeruginosa adapts to the new environment through genetic changes and exhibits a special lifestyle in chronic CF airways. Understanding the persistent......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...

  9. Excessive faecal losses of vitamin A (retinol) in cystic fibrosis.

    OpenAIRE

    Ahmed, F; Ellis, J.; Murphy, J.; Wootton, S.; Jackson, A A

    1990-01-01

    Vitamin A (retinol) deficiency is a recognised complication of cystic fibrosis and is presumed to be a consequence of an impairment in the digestion and absorption of dietary fats. The dietary intake of fat and retinol was assessed from a seven day weighed food intake in 11 subjects with cystic fibrosis and 12 matched controls. Faecal excretion of retinol and fat were measured from three day stool collections. There was little difference between the two groups in the intake of fat or retinol ...

  10. Chronic Low Back Pain due to Retroperitoneal Cystic Lymphangioma.

    Science.gov (United States)

    Fattahi, Asieh Sadat; Maddah, Godratollah; Motamedolshariati, Mohammad; Ghiasi-Moghadam, Taghi

    2014-03-01

    Abdominal cystic lymphangioma is a rare benign neoplasm. Less than 1% of lymphangiomas is in the retroperitoneum. Lymphangioma is mostly asymptomatic. Chronic symptoms were reported in retroperitoneal type more than others. Acute symptoms due to complications like infection, cyst rupture or hemorrhage may occur. We report an 18-years-old girl with low back pain from 6 months ago with huge pelvic mass and diagnosis of retroperitoneal cystic lymphangioma.

  11. Mucins in the diagnosis and differential diagnosis of pancreatic cystic neoplasms: report of 40 cases

    Institute of Scientific and Technical Information of China (English)

    JI Yuan; TAN Yun-shan; XU Jian-fang; QI Wei-dong; LI Xiao-ping; SU-JIE Ake-su; ZHU Xiong-zeng

    2006-01-01

    @@ Cystic neoplasms of the pancreas account for 10% to 15% of all cystic pancreatic lesions.The majority (85% to 90%) of cystic lesions of the pancreas are pseudocysts. Although cystic neoplasms of the pancreas are rare, they range from benign to malignant neoplasms. The clinical challenge is the differential diagnosis and management of the cystic neoplasms, which represent 10% to 25% of primary pancreatic neoplasms. Pancreatic neoplasms and tumour like lesions with cystic features have been recently reviewed. The incidence of pancreatic cystic neoplasms reported is variable. Because there is no large, systematic study on tne cases from China comparing the incidence and biology of cystic neoplasms of pancreas to that of Western series, we reviewed all the cases of cystic neoplasms from Zhongshan Hospital over 6 years. Most of the neoplasms in our series were classified according to the recent World Health Organization (WHO)classification.1,2

  12. Epidemiology of Pseudomonas aeruginosa in cystic fibrosis and the possible role of contamination by dental equipment

    DEFF Research Database (Denmark)

    Jensen, E T; Giwercman, B; Ojeniyi, B;

    1997-01-01

    Cystic fibrosis (CF) patients often suffer from Pseudomonas aeruginosa lung infection yet the source of this organism is not known. In order to determine whether CF patients might be contaminated with P. aeruginosa from dental equipment, a total of 103 water samples from 25 dental sessions...... in Frederiksberg Municipal Oral Health Care Service were examined. Three samples (2.9%) were positive for P. aeruginosa. Three hundred and twenty-seven water samples from 82 dental sessions from various other Municipal Oral Health Services in Denmark, attended by CF patients, were also examined. Eighteen of 327...... samples (5.5%) from nine sessions (11%) were positive for P. aeruginosa. In one case, genotypically identical (RFLP, pulsed-field gel electrophoresis) P. aeruginosa strains were found both in water from the dental equipment and in the CF patients sputum. This indicates a small risk for acquiring P...

  13. Pseudomonas aeruginosa Evolutionary Adaptation and Diversification in Cystic Fibrosis Chronic Lung Infections.

    Science.gov (United States)

    Winstanley, Craig; O'Brien, Siobhan; Brockhurst, Michael A

    2016-05-01

    Pseudomonas aeruginosa populations undergo a characteristic evolutionary adaptation during chronic infection of the cystic fibrosis (CF) lung, including reduced production of virulence factors, transition to a biofilm-associated lifestyle, and evolution of high-level antibiotic resistance. Populations of P. aeruginosa in chronic CF lung infections typically exhibit high phenotypic diversity, including for clinically important traits such as antibiotic resistance and toxin production, and this diversity is dynamic over time, making accurate diagnosis and treatment challenging. Population genomics studies reveal extensive genetic diversity within patients, including for transmissible strains the coexistence of highly divergent lineages acquired by patient-to-patient transmission. The inherent spatial structure and spatial heterogeneity of selection in the CF lung appears to play a key role in driving P. aeruginosa diversification. PMID:26946977

  14. Pseudomona pseudomallei community acquired pneumonia

    International Nuclear Information System (INIS)

    This is the first published case report en Colombia about pseudomona pseudomallei community acquired pneumonia. This uncommon pathogen is from the epidemiological standpoint a very important one and medical community should be aware to look after it in those patients where no other etiological pathogen is recovered. A brief summary about epidemiology is showed, emphasizing those regions where it can be found. Likewise, comments about the differential diagnosis are important since it should be considered in those patients where tuberculosis is suspected. This is particularly representative for countries with high tuberculosis rates. Furthermore, a microbiological review is shown, emphasizing on isolation techniques, descriptions about therapeutics and other regarding treatment issues according international standards. Finally; a description about the clinical picture, laboratory findings, treatment and evolution of the case reported are shown for discussion

  15. Lymphoma in acquired generalized lipodystrophy.

    Science.gov (United States)

    Brown, Rebecca J; Chan, Jean L; Jaffe, Elaine S; Cochran, Elaine; DePaoli, Alex M; Gautier, Jean-Francois; Goujard, Cecile; Vigouroux, Corinne; Gorden, Phillip

    2016-01-01

    Acquired generalized lipodystrophy (AGL) is a rare disease thought to result from autoimmune destruction of adipose tissue. Peripheral T-cell lymphoma (PTCL) has been reported in two AGL patients. We report five additional cases of lymphoma in AGL, and analyze the role of underlying autoimmunity and recombinant human leptin (metreleptin) replacement in lymphoma development. Three patients developed lymphoma during metreleptin treatment (two PTCL and one ALK-positive anaplastic large cell lymphoma), and two developed lymphomas (mycosis fungoides and Burkitt lymphoma) without metreleptin. AGL is associated with high risk for lymphoma, especially PTCL. Autoimmunity likely contributes to this risk. Lymphoma developed with or without metreleptin, suggesting metreleptin does not directly cause lymphoma development; a theoretical role of metreleptin in lymphoma progression remains possible. For most patients with AGL and severe metabolic complications, the proven benefits of metreleptin on metabolic disease will likely outweigh theoretical risks of metreleptin in lymphoma development or progression.

  16. Complement's participation in acquired immunity

    DEFF Research Database (Denmark)

    Nielsen, Claus Henrik; Leslie, Robert Graham Quinton

    2002-01-01

    B cell receptor for antigen (BCR), a complex composed of the iC3b/C3d fragment-binding complement type 2 receptor (CR2, CD21) and its signaling element CD19 and the IgG-binding receptor FcgammaRIIb (CD32). The positive or negative outcome of signaling through this triad is determined by the context...... in which antigen is seen, be it alone or in association with natural or induced antibodies and/or C3-complement fragments. The aim of this review is to describe the present status of our understanding of complement's participation in acquired immunity and the regulation of autoimmune responses.......The preliminary evidence for the involvement of complement in promoting primary humoral responses dates back over a quarter of a century. However, it is only in the course of the past decade or so that the detailed mechanisms underlying complement's influence have been characterized in depth. It is...

  17. Cystic neoplasms of the pancreas: A diagnostic challenge

    Institute of Scientific and Technical Information of China (English)

    Grant F Hutchins; Peter V Draganov

    2009-01-01

    Cystic neoplasms of the pancreas are increasingly recognized due to the expanding use and improved sensitivity of cross-sectional abdominal imaging. Major advances in the last decade have led to an improved understanding of the various types of cystic lesions and their biologic behavior. Despite significant improvements in imaging technology and the advent of endoscopic-ultrasound (EUS)-guided fineneedle aspiration, the diagnosis and management of pancreatic cystic lesions remains a significant clinical challenge. The first diagnostic step is to differentiate between pancreatic pseudocyst and cystic neoplasm.If a pseudocyst has been effectively excluded, the cornerstone issue is then to determine the malignant potential of the pancreatic cystic neoplasm. In the majority of cases, the correct diagnosis and successful management is based not on a single test but on incorporating data from various sources including patient history, radiologic studies, endoscopic evaluation, and cyst fluid analysis. This review will focus on describing the various types of cystic neoplasms of the pancreas, their malignant potential, and will provide the clinician with a comprehensive diagnostic approach.

  18. Diffusion-weighted imaging in characterization of cystic pancreatic lesions

    Energy Technology Data Exchange (ETDEWEB)

    Sandrasegaran, K., E-mail: ksandras@iupui.edu [Department of Radiology, Indiana University School of Medicine, Indianapolis, IN (United States); Akisik, F.M.; Patel, A.A.; Rydberg, M. [Department of Radiology, Indiana University School of Medicine, Indianapolis, IN (United States); Cramer, H.M.; Agaram, N.P. [Department of Pathology, Indiana University School of Medicine, Indianapolis, IN (United States); Schmidt, C.M. [Department of Surgery, Indiana University School of Medicine, Indianapolis, IN (United States)

    2011-09-15

    Aim: To evaluate whether apparent diffusion coefficient (ADC) measurements from diffusion-weighted imaging (DWI) can characterize or predict the malignant potential of cystic pancreatic lesions. Materials and methods: Retrospective review of the magnetic resonance imaging (MRI) database over a 2-year period revealed 136 patients with cystic pancreatic lesions. Patients with DWI studies and histological confirmation of cystic mass were included. In patients with known pancreatitis, lesions with amylase content of >1000 IU/l that resolved on subsequent scans were included as pseudocysts. ADC of cystic lesions was measured by two independent reviewers. These values were then compared to categorize these lesions as benign or malignant using conventional MRI sequences. Results: Seventy lesions were analysed: adenocarcinoma (n = 4), intraductal papillary mucinous neoplasm (IPMN; n = 28), mucinous cystic neoplasm (MCN; n = 9), serous cystadenoma (n = 16), and pseudocysts (n = 13). There was no difference between ADC values of malignant and non-malignant lesions (p = 0.06), between mucinous and serous tumours (p = 0.12), or between IPMN and MCN (p = 0.42). ADC values for low-grade IPMN were significantly higher than those for high-grade or invasive IPMN (p = 0.03). Conclusion: ADC values may be helpful in deciding the malignant potential of IPMN. However, they are not useful in differentiating malignant from benign lesions or for characterizing cystic pancreatic lesions.

  19. Partially Cystic Thyroid Nodules: Ultrasound Findings of Malignancy

    Energy Technology Data Exchange (ETDEWEB)

    Park, Jang Mi; Choi, Yoon Jung; Kwag, Hyon Joo [Dept. of Radiology, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine, Seoul (Korea, Republic of)

    2012-09-15

    To seek for the ultrasound (US) findings of partially cystic thyroid nodules that are associated with malignancy. We reviewed the US characteristics of 22 surgically confirmed partially cystic papillary carcinomas, and compared them with those of 80 benign partially cystic nodules. The review cases were selected in a random order from a total of 1029 partially cystic nodules that were diagnosed with an US-guided fine needle aspiration biopsy over a period of 8 years (June 2003 to October 2010) at our institution. In partially cystic thyroid nodules, a taller-than-wide shape (100%, p<0.001) and spiculated or microlobulated margin (58.3%, p 0.003) were significantly associated with malignancy. In terms of internal solid portion of the nodule, eccentric configuration (68.0%, p<0.001), non-smooth margin (81.3%, p<0.001), hypoechogenecity (30.0%, p<0.042), and microcalcification (89.5%, p<0.001) were more frequently demonstrated in malignant nodules than benign ones. In partially cystic thyroid nodules, understanding the characteristics of US findings is important to make a precise diagnosis of malignant nodules.

  20. Targeted therapies to improve CFTR function in cystic fibrosis.

    Science.gov (United States)

    Brodlie, Malcolm; Haq, Iram J; Roberts, Katie; Elborn, J Stuart

    2015-01-01

    Cystic fibrosis is the most common genetically determined, life-limiting disorder in populations of European ancestry. The genetic basis of cystic fibrosis is well established to be mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene that codes for an apical membrane chloride channel principally expressed by epithelial cells. Conventional approaches to cystic fibrosis care involve a heavy daily burden of supportive treatments to combat lung infection, help clear airway secretions and maintain nutritional status. In 2012, a new era of precision medicine in cystic fibrosis therapeutics began with the licensing of a small molecule, ivacaftor, which successfully targets the underlying defect and improves CFTR function in a subgroup of patients in a genotype-specific manner. Here, we review the three main targeted approaches that have been adopted to improve CFTR function: potentiators, which recover the function of CFTR at the apical surface of epithelial cells that is disrupted in class III and IV genetic mutations; correctors, which improve intracellular processing of CFTR, increasing surface expression, in class II mutations; and production correctors or read-through agents, which promote transcription of CFTR in class I mutations. The further development of such approaches offers great promise for future therapeutic strategies in cystic fibrosis. PMID:26403534

  1. Cystic Odontoma in a Patient with Hodgkin's Lymphoma.

    Science.gov (United States)

    Costa, Victor; Caris, Adriana Rocha; León, Jorge Esquiche; Ramos, Carolina Judica; Jardini, Vaneska; Kaminagakura, Estela

    2015-01-01

    Cystic odontoma is a rare entity, which is characterized by the association of a cyst with complex/compound odontoma. The aim of this study was to report the case of a 5-year-old male patient diagnosed previously with Hodgkin's lymphoma and treated successfully with chemotherapy and radiotherapy, who developed a mandibular odontogenic lesion. Physical examination revealed a swelling on the right side of the mandible. Radiographically, a well-defined radiolucent area surrounded by radiopaque material was observed. An incisional biopsy was performed and microscopic analysis showed a cystic lesion consisting of an atrophic epithelium comprising 2-3 cell layers and the absence of inflammation in the cystic capsule. The cyst was decompressed and the lesion was removed after 3 months of follow-up. Microscopic analysis of the surgical specimen showed a cystic hyperplastic epithelium surrounded by an intense chronic inflammatory cell infiltrate, which was in close contact with mineralized tissue resembling dentin and cementum. The final diagnosis was cystic odontoma. Since chemotherapy can affect the growth and development of infant teeth, a relationship between chemotherapy-associated adverse events and cystic odontoma is suggested in the present case.

  2. Cystic Odontoma in a Patient with Hodgkin’s Lymphoma

    Directory of Open Access Journals (Sweden)

    Victor Costa

    2015-01-01

    Full Text Available Cystic odontoma is a rare entity, which is characterized by the association of a cyst with complex/compound odontoma. The aim of this study was to report the case of a 5-year-old male patient diagnosed previously with Hodgkin’s lymphoma and treated successfully with chemotherapy and radiotherapy, who developed a mandibular odontogenic lesion. Physical examination revealed a swelling on the right side of the mandible. Radiographically, a well-defined radiolucent area surrounded by radiopaque material was observed. An incisional biopsy was performed and microscopic analysis showed a cystic lesion consisting of an atrophic epithelium comprising 2-3 cell layers and the absence of inflammation in the cystic capsule. The cyst was decompressed and the lesion was removed after 3 months of follow-up. Microscopic analysis of the surgical specimen showed a cystic hyperplastic epithelium surrounded by an intense chronic inflammatory cell infiltrate, which was in close contact with mineralized tissue resembling dentin and cementum. The final diagnosis was cystic odontoma. Since chemotherapy can affect the growth and development of infant teeth, a relationship between chemotherapy-associated adverse events and cystic odontoma is suggested in the present case.

  3. Computed tomography of cystic tumors of the mediastinum

    Energy Technology Data Exchange (ETDEWEB)

    Yoon, Chong Hyun; Im, Jung Gi; Han, Man Chung [Seoul National University College of Medicine, Seoul (Korea, Republic of)

    1983-12-15

    Authors retrospectively analyzed the CT findings of cystic tumors of the mediastinum in surgically confirmed 13 cases at Seoul National University Hospital during the recent 3 years from August, 1980 to August, 1983. The results are as follows; 1. Among 13 cases, bronchogenic cyst and cystic teratoma were 4 cases respectively, pericardial cyst were 2 cases, and thymic cyst and cystic hygroma was 1 case respectively. 2. CT No. of 4 bronchogenic cysts was in the range of 12-112 HU, 3 cases among them showed high density above 69 HU. CT No. of 4 cystic teratomas was in the range of -24 -20 HU. All of them had thick-walled capsules. CT No. of 2 pericardial cysts was in the range of 12-25 HU. CT No. of a thymic cyst was in the range of -10 -23 HU. CT No. of a cystic hygroma was about 24 HU. 3. With CT, cystic tumors of mediastinum are easily diagnosed by its shape, location and typical water density mass. But bronchogenic cyst, because of its mucoid contents, may often have CT No. above those usually considered characteristic of cyst. So we must consider bronchogenic cyst in differential diagnosis even if CT No. is high, if the lesion is in typical location and the shape is round or ovoid.

  4. Acquired vulval lymphangiectases mimicking genital warts

    OpenAIRE

    Sharma Rajeev; Tomar Sudarshan; Chandra Mithilesh

    2002-01-01

    Acquired lymphangiectasia can sometimes occur on the vulva and cause diagnostic difficulties especially if they have a warty appearance. We report a case of acquired vulva I lymphangiectasia which mimicked genital warts.

  5. Acquired vulval lymphangiectases mimicking genital warts

    Directory of Open Access Journals (Sweden)

    Sharma Rajeev

    2002-01-01

    Full Text Available Acquired lymphangiectasia can sometimes occur on the vulva and cause diagnostic difficulties especially if they have a warty appearance. We report a case of acquired vulva I lymphangiectasia which mimicked genital warts.

  6. US Cystic Fibrosis Foundation and European Cystic Fibrosis Society consensus recommendations for the management of non-tuberculous mycobacteria in individuals with cystic fibrosis: executive summary.

    Science.gov (United States)

    Floto, R Andres; Olivier, Kenneth N; Saiman, Lisa; Daley, Charles L; Herrmann, Jean-Louis; Nick, Jerry A; Noone, Peadar G; Bilton, Diana; Corris, Paul; Gibson, Ronald L; Hempstead, Sarah E; Koetz, Karsten; Sabadosa, Kathryn A; Sermet-Gaudelus, Isabelle; Smyth, Alan R; van Ingen, Jakko; Wallace, Richard J; Winthrop, Kevin L; Marshall, Bruce C; Haworth, Charles S

    2016-01-01

    Non-tuberculous mycobacteria (NTM) are ubiquitous environmental organisms that can cause chronic pulmonary infection, particularly in individuals with pre-existing inflammatory lung disease, such as cystic fibrosis (CF). Pulmonary disease (PD) caused by NTM has emerged as a major threat to the health of individuals with CF, but remains difficult to diagnose and problematic to treat. In response to this challenge, the US Cystic Fibrosis Foundation (CFF) and the European Cystic Fibrosis Society (ECFS) convened a panel of 19 experts to develop consensus recommendations for the screening, investigation, diagnosis and management of NTM-PD in individuals with CF. PICO (population, intervention, comparison, outcome) methodology and systematic literature reviews were employed to inform draft recommendations, which were then modified to achieve consensus and subsequently circulated for public consultation within the USA and European CF communities. We have thus generated a series of pragmatic, evidence-based recommendations as an initial step in optimising management for this challenging condition.

  7. 12 CFR 583.1 - Acquire.

    Science.gov (United States)

    2010-01-01

    ... AND LOAN HOLDING COMPANIES § 583.1 Acquire. The term acquire means to acquire, directly or indirectly, ownership or control through an acquisition of shares, an acquisition of assets or assumption of liabilities, a merger or consolidation, or any similar transaction....

  8. Primary omental yolk sac tumor

    OpenAIRE

    Lim, Seon Hwa; Kim, Yon Hee; Yim, Ga Won; Nam, Eun Ji; Kim, Young Tae; KIM, SUNGHOON

    2013-01-01

    Extra-ovarian yolk sac tumor arising in the omentum is extremely rare. As yolk sac tumor originated from the omentum has been rarely reported, its clinical information is very limited. The authors encountered a case of yolk sac tumor originated from the omentum, and reported the case herein. A 32-year-old woman was presented with developed low abdominal distension for a month. Magnetic resonance imaging findings were suggestive of ovarian malignancy with ascites and peritoneal seeding nodules...

  9. Primary omental yolk sac tumor.

    Science.gov (United States)

    Lim, Seon Hwa; Kim, Yon Hee; Yim, Ga Won; Nam, Eun Ji; Kim, Young Tae; Kim, Sunghoon

    2013-11-01

    Extra-ovarian yolk sac tumor arising in the omentum is extremely rare. As yolk sac tumor originated from the omentum has been rarely reported, its clinical information is very limited. The authors encountered a case of yolk sac tumor originated from the omentum, and reported the case herein. A 32-year-old woman was presented with developed low abdominal distension for a month. Magnetic resonance imaging findings were suggestive of ovarian malignancy with ascites and peritoneal seeding nodules. Explorative laparotomy was performed and then the findings from frozen biopsy of omentum were suggestive of poorly differentiated tumor though whether it was primary or metastatic was uncertain. Thus, staging laparotomy were performed. Histopathology confirmed that the tumor was a yolk sac tumor of omentum origin. Then, 6 cycles of postoperative adjuvant chemotherapy at intervals of 3 weeks were performed using bleomycin, etoposide, and cisplatin regimen. Four-year outpatient follow-up thereafter showed no relapse. PMID:24396822

  10. Intestinal Bicarbonate Secretion in Cystic Fibrosis Mice

    Directory of Open Access Journals (Sweden)

    Clarke LL

    2001-07-01

    Full Text Available Gene-targeted disruption of the cystic fibrosis transmembrane conductance regulator (CFTR in mice results in an intestinal disease phenotype that is remarkably similar to bowel disease in cystic fibrosis patients. In the intestinal segment downstream from the stomach (i.e., the duodenum, CFTR plays an important role in bicarbonate secretion that protects the epithelium from acidic gastric effluent. In this report, we examine the role of CFTR in cAMP-stimulated bicarbonate secretion in the murine duodenum and the mechanisms of acid-base transport that are revealed in CFTR knockout (CF mice. Ion substitution, channel blocker and pH stat studies comparing duodena from wild-type and CF mice indicate that CFTR mediates a HCO(3(- conductance across the apical membrane of the epithelium. In the presence of a favorable cell-to-lumen HCO(3(- gradient, the CFTR-mediated HCO(3(- current accounts for about 80% of stimulated HCO(3(- secretion. Exposure of the duodenal mucosa to acidic pH reveals another role of CFTR in facilitating HCO(3(- secretion via an electroneutral, 4,4'-diisothiocyanato-stilbene-2,2' disulfonic acid (DIDS sensitive Cl(-/HCO(3(- exchange process. In CF duodenum, other apical membrane acid-base transporters retain function, thereby affording limited control of transepithelial pH. Activity of a Cl(--dependent anion exchanger provides near-constant HCO(3(- secretion in CF intestine, but under basal conditions the magnitude of secretion is lessened by simultaneous activity of a Na(+/H(+ exchanger (NHE. During cAMP stimulation of CF duodenum, a small increase in net base secretion is measured but the change results from cAMP inhibition of NHE activity rather than increased HCO(3(- secretion. Interestingly, a small inward current that is sensitive to the anion channel blocker, 5-nitro-2(3-phenylpropyl amino-benzoate (NPPB, is also activated during cAMP stimulation of the CFTR-null intestine but the identity of the current is yet to be

  11. Cystic Fibrosis Heterozygote Resistance to Cholera Toxin in the Cystic Fibrosis Mouse Model

    Science.gov (United States)

    Gabriel, Sherif E.; Brigman, Kristen N.; Koller, Beverly H.; Boucher, Richard C.; Stutts, M. Jackson

    1994-10-01

    The effect of the number of cystic fibrosis (CF) alleles on cholera toxin (CT)-induced intestinal secretion was examined in the CF mouse model. CF mice that expressed no CF transmembrane conductance regulator (CFTR) protein did not secrete fluid in response to CT. Heterozygotes expressed 50 percent of the normal amount of CFTR protein in the intestinal epithelium and secreted 50 percent of the normal fluid and chloride ion in response to CT. This correlation between CFTR protein and CT-induced chloride ion and fluid secretion suggests that CF heterozygotes might possess a selective advantage of resistance to cholera.

  12. Liver and lung transplantation in cystic fibrosis: an adult cystic fibrosis centre's experience.

    Science.gov (United States)

    Sivam, S; Al-Hindawi, Y; Di Michiel, J; Moriarty, C; Spratt, P; Jansz, P; Malouf, M; Plit, M; Pleass, H; Havryk, A; Bowen, D; Haber, P; Glanville, A R; Bye, P T P

    2016-07-01

    Liver disease develops in one-third of patients with cystic fibrosis (CF). It is rare for liver disease to have its onset after 20 years of age. Lung disease, however, is usually more severe in adulthood. A retrospective analysis was performed on nine patients. Three patients required lung transplantation approximately a decade after liver transplant, and another underwent combined liver and lung transplants. Four additional patients with liver transplants are awaiting assessment for lung transplants. One patient is awaiting combined liver and lung transplants. With increased survival in CF, several patients may require more than single organ transplantation. PMID:27405894

  13. Infections Acquired in the Garden.

    Science.gov (United States)

    Cunha, Cheston B; Cunha, Burke A

    2015-10-01

    Gardening is a wonderful pastime, and the garden is a very peaceful place to enjoy one's vacation. However, the garden may be a treacherous place for very young or compromised hosts when one takes into account the infectious potential residing in the soil, as well as the insect vectors on plants and animals. Even normal hosts may acquire a variety of infections from the soil, animals, or animal-related insect bites. The location of the garden, its natural animal and insect inhabitants, and the characteristics of the soil play a part in determining its infectious potential. The most important factor making the garden an infectious and dangerous place is the number and interaction of animals, whether they are pets or wild, that temporarily use the garden for part of their daily activities. The clinician should always ask about garden exposure, which will help in eliminating the diagnostic possibilities for the patient. The diagnostic approach is to use epidemiological principles in concert with clinical clues, which together should suggest a reasonable list of diagnostic possibilities. Organ involvement and specific laboratory tests help further narrow the differential diagnosis and determine the specific tests necessary to make a definitive diagnosis. PMID:26542044

  14. Infertility Management in Men with Cystic Fibrosis

    Institute of Scientific and Technical Information of China (English)

    Nikita Rawal; Rafit Gazvani; Roger Mountford

    2009-01-01

    Cystic fibrosis (CF) is a multisystem autosomal recessive disorder. CF has a reported incidence of 1 in 2 500 and a carrier frequency of 1 in 25. It is caused by mutations in a gene located on the long arm of chromosome 7. With almost 50% of patients with CF now in the adult population, and a very high interest in future parenting (78% of men wanting children), there is an increasing need to be proactive in terms of advice about reproductive health. Discussion on reproduction should start in early adolescence and be regularly updated by medical staff. The recent advances in reproductive techniques have allowed the development of sperm aspiration coupled with intra-cytoplasmic sperm injection (ICSI). Spermatozoa can be retrieved from either the epididymis or the testes. Genetic counselling is strongly recommended for both partners when congenital bilateral aplasia of vas deferens (CBA VD) is diagnosed as there is the inevitability of transmitting a mutated CFTR gene, and an increased risk of producing an affected child with serious long-term implications. Most treatment centres, with the introduction of ICSI, routinely screen all males with azoospermia caused by obstruction (other than that caused by vasectomy) or germ cell failure. Protocols usually involve screening for 28 relatively common CFTR mutations. Further advanced testing may be necessary up to a final detection level of approximately 98.5%. Before considering any assisted reproduction treatment, it is pertinent that CF patients receive medical and psychological counselling.

  15. Asthma and cystic fibrosis: A tangled web.

    LENUS (Irish Health Repository)

    Kent, Brian D

    2014-03-01

    Successfully diagnosing concomitant asthma in people with cystic fibrosis (CF) is a challenging proposition, and the utility of conventional diagnostic criteria of asthma in CF populations remains uncertain. Nonetheless, the accurate identification of individuals with CF and asthma allows appropriate tailoring of therapy, and should reduce the unnecessary use of asthma medication in broader CF cohorts. In this review, we discuss the diagnostic challenge posed by asthma in CF, both in terms of clinical evaluation, and of interpretation of pulmonary function testing and non-invasive markers of airway inflammation. We also examine how the role of cross-sectional thoracic imaging in CF and asthma can assist in the diagnosis of asthma in these patients. Finally, we critically appraise the evidence base behind the use of asthma medications in CF populations, with a particular focus on the use of inhaled corticosteroids and bronchodilators. As shall be discussed, the gaps in the current literature make further high-quality research in this field imperative. Pediatr Pulmonol. 2014; 49:205-213. © 2014 Wiley Periodicals, Inc.

  16. The cystic fibrosis lower airways microbial metagenome

    Science.gov (United States)

    Moran Losada, Patricia; Chouvarine, Philippe; Dorda, Marie; Hedtfeld, Silke; Mielke, Samira; Schulz, Angela; Wiehlmann, Lutz

    2016-01-01

    Chronic airway infections determine most morbidity in people with cystic fibrosis (CF). Herein, we present unbiased quantitative data about the frequency and abundance of DNA viruses, archaea, bacteria, moulds and fungi in CF lower airways. Induced sputa were collected on several occasions from children, adolescents and adults with CF. Deep sputum metagenome sequencing identified, on average, approximately 10 DNA viruses or fungi and several hundred bacterial taxa. The metagenome of a CF patient was typically found to be made up of an individual signature of multiple, lowly abundant species superimposed by few disease-associated pathogens, such as Pseudomonas aeruginosa and Staphylococcus aureus, as major components. The host-associated signatures ranged from inconspicuous polymicrobial communities in healthy subjects to low-complexity microbiomes dominated by the typical CF pathogens in patients with advanced lung disease. The DNA virus community in CF lungs mainly consisted of phages and occasionally of human pathogens, such as adeno- and herpesviruses. The S. aureus and P. aeruginosa populations were composed of one major and numerous minor clone types. The rare clones constitute a low copy genetic resource that could rapidly expand as a response to habitat alterations, such as antimicrobial chemotherapy or invasion of novel microbes. PMID:27730195

  17. New and Emerging Treatments for Cystic Fibrosis.

    Science.gov (United States)

    Barry, Peter J; Jones, Andrew M

    2015-07-01

    Recently, a significant number of additional key medications have become licensed in Europe for the treatment of patients with cystic fibrosis (CF), including a number of inhaled antibiotics, such as nebulised aztreonam and dry powder versions of colistin and tobramycin for inhalation; dry powder inhaled mannitol, an agent to improve airway hydration and aid airway clearance; and ivacaftor, an oral therapy that directly acts on dysfunctional CFTR to correct the basic defect encountered in CF patients with the G551D CF gene mutation. The marked success of ivacaftor both in clinical trials and in post-licensing evaluation studies in treating patients with G551D and other gating mutations has greatly encouraged the ongoing development of similar therapies that can directly target the underlying cause of CF. Other therapies, including a number of anti-infectives, anti-inflammatories and replacement pancreatic enzymes, are currently undergoing clinical studies. This article reviews those treatments that have been recently licensed for CF and highlights some of the exciting emerging therapies presently under evaluation in clinical trials. In addition, it discusses some of the potential challenges being encountered by research and clinical teams in developing and delivering treatments for this condition. PMID:26091951

  18. The Evolution of Cystic Fibrosis Care.

    Science.gov (United States)

    Pittman, Jessica E; Ferkol, Thomas W

    2015-08-01

    Cystic fibrosis (CF) is the most common life-limiting inherited illness of whites. Most of the morbidity and mortality in CF stems from impaired mucociliary clearance leading to chronic, progressive airways obstruction and damage. Significant progress has been made in the care of patients with CF, with advances focused on improving mucociliary clearance, minimizing inflammatory damage, and managing infections; these advances include new antimicrobial therapies, mucolytic and osmotic agents, and antiinflammatory treatments. More recently, researchers have targeted disease-causing mutations using therapies to promote gene transcription and improve channel function, which has led to impressive physiologic changes in some patients. As we develop more advanced, allele-directed therapies for the management of CF, it will become increasingly important to understand the specific genetic and environmental interactions that cause the significant heterogeneity of lung disease seen in the CF population. This understanding of CF endotypes will allow for more targeted, personalized therapies for future patients. This article reviews the genetic and molecular basis of CF lung disease, the treatments currently available, and novel therapies that are in development. PMID:25764168

  19. Lentiviral Vectors and Cystic Fibrosis Gene Therapy

    Directory of Open Access Journals (Sweden)

    Massimo Conese

    2010-01-01

    Full Text Available Cystic fibrosis (CF is a chronic autosomic recessive syndrome, caused by mutations in the CF Transmembrane Conductance Regulator (CFTR gene, a chloride channel expressed on the apical side of the airway epithelial cells. The lack of CFTR activity brings a dysregulated exchange of ions and water through the airway epithelium, one of the main aspects of CF lung disease pathophysiology. Lentiviral (LV vectors, of the Retroviridae family, show interesting properties for CF gene therapy, since they integrate into the host genome and allow long-lasting gene expression. Proof-of-principle that LV vectors can transduce the airway epithelium and correct the basic electrophysiological defect in CF mice has been given. Initial data also demonstrate that LV vectors can be repeatedly administered to the lung and do not give rise to a gross inflammatory process, although they can elicit a T cell-mediated response to the transgene. Future studies will clarify the efficacy and safety profile of LV vectors in new complex animal models with CF, such as ferrets and pigs.

  20. Dermatoglyphic Patterns in Cystic Fibrosis Children

    Science.gov (United States)

    Ezzati, Atefeh; Batoei, Fereshteh; Jafari, Seyed-Ali; Kiyani, Mohammad-Ali; Mahdavi-Shahri, Naser; Ahanchian, Hamid; Tehranian, Shahrzad; Kianifar, Hamid-Reza

    2014-01-01

    Objective: It is believed that fingerprints and palm patterns may represent genetically determined congenital abnormalities in Cystic Fibrosis (CF). The main idea of this paper was to determine differences of fingerprints and palm patterns in CF and normal children. Methods: Forty-six CF children (27 males, 19 females) and 341 (113 males, 228 females) healthy individuals were recruited for this study. Fingerprint patterns, Total ridge count (TRC) of each finger, a-b ridge count, and atd angles of all participants were recorded. Asymmetry of the right and left hand for each value was determined and dissimilarity in fingerprint patterns between homologous fingers was compared using Chi-square analysis, Mann-Whitney U test and Fisher's exact test. Findings: There were significant differences in the mean TRC of the right digit IV (P=0.009), left digit III (P=0.02), left digit IV (P=0.03), and left digit V (P=0.03). Furthermore, we found significant differences in right atd angel (P=0.001), left atd angel (P=0.002), right a-b ridge (P=0.007) and left a-b ridge (P=0.001). In contrast, we found no significant differences in atd angle asymmetry, a-b ridge count asymmetry and pattern dissimilarity score between both groups (P>0.05). Conclusion: Dermatoglyphic characteristics could be used as a supplementary diagnostic method in CF children. PMID:25793070

  1. Recombinant antigens for immunodiagnosis of cystic echinococcosis

    Directory of Open Access Journals (Sweden)

    Li Jun

    2004-01-01

    Full Text Available Three cDNAs, termed EpC1, TPxEg and EgG5, were isolated by immunoscreening from an Echinococcus granulosus cDNA library. The recombinant phages exhibited strong reactivity with sera from humans with confirmed cystic echinococcosis (CE and with sera from mice infected with E. granulosus oncospheres. The cDNAs were subcloned into a pET vector, expressed as fusion proteins tagged with GST and affinity purified against the GST tag. Of the three recombinant proteins, EpC1 achieved the highest performance for serodiagnosis of CE in Western blot analysis using a panel of clinically defined human sera to initially address the sensitivity and specificity of the molecules. The protein yielded an overall sensitivity of 92.2% and specificity of 95.6%, levels unprecedented taking into account the large panel of 896 human sera that were tested. The strategy used may also prove suitable for improved immunodiagnosis of other parasitic infections.

  2. Vitamin K status in cystic fibrosis patients

    Directory of Open Access Journals (Sweden)

    Patrycja Krzyżanowska

    2010-12-01

    Full Text Available Vitamin K belongs to the family of fat-soluble vitamins and plays an important role in hemostasis, bone metabolism and may affect cerebral sphingolipid synthesis. It is a cofactor necessary for posttranslational γ-carboxylation of glutamyl residues in selected proteins such as the osteocalcin, and procoagulation factors II, VII, IX, X. Vitamin K deficient individuals appear to have more undercarboxylated proteins, which are functionally defective. The vitamin K deficiency has been frequently documented in patients with cystic fibrosis. The main possible causes of this deficiency include: fat malabsorption due to pancreatic exocrine insufficiency, cholestatic or  noncholestatic liver disease, reduced production of vitamin K by colonic flora related to chronic antibiotic treatments, bowel resections and increased mucous accumulation in the bowel. CF patients are more prone to osteopenia, caused by chronic vitamin K shortage, than to coagulopathy. Despite available evidence, which strongly suggests that all CF patients are at risk for developing vitamin K deficiency, its supplementation doses have not been established. Recent recommendations from Europe and the UK have suggested varied doses ranging from 0.3 mg/day to 10 mg/week. Further studies, both cross sectional and longitudinal interventional, are still required to determine routine and therapeutic supplementation doses.

  3. A millennial view of cystic fibrosis.

    Science.gov (United States)

    Dodge, John A

    2015-01-01

    Although only identified as a distinct disease in the 1930s, it was soon apparent that Cystic Fibrosis (CF) had been present, but unrecognised, in European populations for many years - perhaps even centuries [1] . Within a decade of the early descriptions, the autosomal recessive nature of this genetic disease had been clarified, and its clinical features had been expanded. Secondary nutritional deficiencies complicated the underlying condition: the first clear description of CF as "a new disease", which included a speculation about its genetic basis (because there were 2 pairs of sibs in the case series) was published as Vitamin A deficiency in children [2]. The diagnosis was most often made at autopsy. When it was suspected in life, the diagnostic tests used included duodenal intubation to obtain fluid which would show impaired tryptic digestion of the coating of X-Ray film in CF children, and measurement of vitamin A in the blood. Some nutritional improvement could be expected with simple, rather inefficient pancreatic enzyme preparations, but it was not until mid-century that antibiotics began to treat pulmonary infections effectively. As a young doctor in the 1950s I soon became aware that the median age at death for affected children was about one year, and most died before reaching school age. . PMID:26003065

  4. Computed Tomography in pulmonary cystic fibrosis

    International Nuclear Information System (INIS)

    This study was aimed at evaluating CT sensitivity in identifying the signs of pulmonary cystic fibrosis (CF). The chests of 39 patients (16 males and 23 females, mean age 19.1 years) were examined by CT: all patients had been given a clinical score according to Schwachman and Kulckzycki criteria. Thickened bronchial walls were observed in all cases, which are typical of peribronchitis. Bronchiectases were present in 87% of cases; their extent, pattern and localization were exactly shown on CT scans. Bronchoceles were seen on CT scans in 64% of patients; less frequent was the finding of atelectases and subpleural bullous emphysema. In a great number of patients (64% and 82%, respectively) pleural thickening and hilar adenopathy were demonstrated on CT scans. In conclusion, our results confirm CT as a more sensitive method than conventional radiography to identify and locate the signs of pulmonary CF. The early identification of the lesions of high prognostic value, since the early detection and treatment of bronchoceles may prevent permanent bronchiectasis

  5. Cystic fibrosis on the African continent.

    Science.gov (United States)

    Stewart, Cheryl; Pepper, Michael S

    2016-07-01

    Cystic fibrosis (CF; OMIM 219700) is a life-shortening and costly autosomal recessive disease that has been most extensively studied in individuals of Caucasian descent. There is ample evidence, however, that it also affects other ethnicities. In Africa there have been several reports of CF, but there has been no concerted effort toward establishing the molecular epidemiology of this disease on the continent, which is the first step toward outlining a public health strategy to effectively address the needs of these patients. A literature search revealed reports from only 12 of the 54 African states on the molecular analysis of the mutations present in suspected CF patients, resulting in the identification of 79 mutations. Based on previous functional investigations, 39 of these cause CF, 10 are of varying clinical consequence, 4 have no associated evidence regarding whether they cause CF, 4 are synonymous, 5 are novel, and 21 are unique to Africa. We propose that CF be more thoroughly investigated on the continent to ensure that the public health needs of African CF patients-both those in Africa and those of African descent living elsewhere-are met.Genet Med 18 7, 653-662. PMID:26656651

  6. Pulmonary Exacerbations in Children with Cystic Fibrosis.

    Science.gov (United States)

    Waters, Valerie; Ratjen, Felix

    2015-11-01

    Pulmonary exacerbations treated with intravenous antibiotics have significant, well-characterized negative consequences on clinical outcomes in cystic fibrosis (CF). The impact of milder exacerbations in children with CF, commonly treated with oral antibiotics, are less well defined. Pulmonary exacerbations have multiple triggers, but viral infections are particularly common in children. Children with CF and healthy control subjects have similar frequencies of viral respiratory infections, but there is evidence of greater virus-related morbidity in patients with CF, likely due to a combination of increased viral load, more pronounced inflammatory response, and more pronounced impairment in mucociliary clearance. In recent clinical trials in children, definitions have been used that are more symptom based rather than intervention based. These studies have demonstrated differences in the spectrum of symptoms between children and older patients but have also shown that, despite low threshold definitions, a considerable number of patients receive treatment for events not fulfilling the pulmonary exacerbation criteria. Additional research is needed to determine the impact of these milder exacerbations on lung function recovery and time to subsequent exacerbation as well as long-term outcomes such as mortality. PMID:26595740

  7. Cystic fibrosis: newborn screening in America.

    Science.gov (United States)

    Kleven, Daniel T; McCudden, Christopher R; Willis, Monte S

    2008-07-01

    Cystic fibrosis is the most common lethal genetic disease in Caucasians, manifesting as progressive lung dysfunction, pancreatic insufficiency, and intestinal disease. CF was traditionally diagnosed clinically, either because of a family history or occurrence of meconium ileus, or as a result of intestinal malabsorption and chronic pulmonary disease. In 1979, it was discovered that immunoreactive trypsinogen was increased in neonatal dried-blood specimens on Guthrie cards, making it possible to screen neonates. During the past decades, survival rates of patients with CF have improved significantly (see Figure 5). To continue this progress, universal newborn screening has been implemented in many states as an addition to the arsenal of therapies and strategies to improve survival. National newborn-screening programs to identify CF patients after birth have been adopted for a number of years in Europe, Australia, and Canada. As expected, many benefits have been seen due to the early identification of CF patients, including improved survival, better lung function and growth with less intensive therapy, and reduced cost of therapy. To date, 37 states in the United States have adopted similar programs, in the hopes of improving CF outcomes. This welcome trend should help improve the lives of CF patients living in America. PMID:18717498

  8. 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.)

  9. Abdominal manifestations of cystic fibrosis in children

    International Nuclear Information System (INIS)

    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.)

  10. The genus Prevotella in cystic fibrosis airways.

    Science.gov (United States)

    Field, Tyler R; Sibley, Christopher D; Parkins, Michael D; Rabin, Harvey R; Surette, Michael G

    2010-08-01

    Airway disease resulting from chronic bacterial colonization and consequential inflammation is the leading cause of morbidity and mortality in patients with Cystic Fibrosis (CF). Although traditionally considered to be due to only a few pathogens, recent re-examination of CF airway microbiology has revealed that polymicrobial communities that include many obligate anaerobes colonize lower airways. The purpose of this study was to examine Prevotella species in CF airways by quantitative culture and phenotypic characterization. Expectorated sputum was transferred to an anaerobic environment immediately following collection and examined by quantitative microbiology using a variety of culture media. Isolates were identified as facultative or obligate anaerobes and the later group was identified by 16S rRNA sequencing. Prevotella spp. represented the majority of isolates. Twelve different species of Prevotella were recovered from 16 patients with three species representing 65% of isolates. Multiple Prevotella species were often isolated from the same sputum sample. These isolates were biochemically characterized using Rapid ID 32A kits (BioMérieux), and for their ability to produce autoinducer-2 and beta-lactamases. Considerable phenotypic variability between isolates of the same species was observed. The quantity and composition of Prevotella species within a patients' airway microbiome varied over time. Our results suggest that the diversity and dynamics of Prevotella in CF airways may contribute to airway disease.

  11. Clinicopathological associations of acquired erythroblastopenia

    Science.gov (United States)

    Gunes, Gursel; Malkan, Umit Yavuz; Yasar, Hatime Arzu; Eliacik, Eylem; Haznedaroglu, Ibrahim Celalettin; Demiroglu, Haluk; Sayinalp, Nilgun; Aksu, Salih; Etgul, Sezgin; Aslan, Tuncay; Goker, Hakan; Ozcebe, Osman Ilhami; Buyukasik, Yahya

    2015-01-01

    Introduction: Acquired erythroblastopenia (AE) is a rare clinical situation. It is characterized by the reduction of erythroid precursors in the bone marrow together with the low reticulocyte counts in the peripheral blood. Background: Main secondary causes of AE are drugs, Parvovirus B19 and other infectious reasons, lymphoid and myeloid neoplasia, autoimmune diseases, thymoma and pregnancy. The aim of this study is to assess the frequencies and clinical associations of AE via analyzing 12340 bone marrow samples in a retrospective manner. Material and method: Bone marrow aspirations which were obtained from patients who applied to Hacettepe University Hematology Clinic between 2002 and 2013, were analyzed retrospectively. Results: Thirty four erythroblastopenia cases were found. Patients ranged in age from 16 to 80 years with a median of 38 years. Fifteen patients were men (44%) and nineteen were women (56%). In these patients, detected causes of erythroblastopenia were MDS, idiopathic pure red cell aplasia (PRCA), parvovirus infection, post chemotherapy aplasia, plasma proliferative diseases, copper deficiency due to secondary amyloidosis, fever of unknown origin, hemophagocytic syndrome, enteric fever and legionella pneumonia. We found that between those reasons the most common causes of erythroblastopenia are MDS (17.7%) and idiopathic PRCA (17.7%). Discussion: As a result, erythroblastopenia in the bone marrow may be an early sign of MDS. In those AE cases possibility of being MDS must be kept in mind as it can be mistaken for PRCA. Conclusion: To conclude, in adults MDS without excess blast is one of the most common causes of erythroblastopenia in clinical practice and in case of erythroblastopenia the presence of MDS should be investigated. PMID:26885236

  12. Targeting a genetic defect: cystic fibrosis transmembrane conductance regulator modulators in cystic fibrosis

    Directory of Open Access Journals (Sweden)

    Nico Derichs

    2013-03-01

    Full Text Available Cystic fibrosis (CF is caused by genetic mutations that affect the cystic fibrosis transmembrane conductance regulator (CFTR protein. These mutations can impact the synthesis and transfer of the CFTR protein to the apical membrane of epithelial cells, as well as influencing the gating or conductance of chloride and bicarbonate ions through the channel. CFTR dysfunction results in ionic imbalance of epithelial secretions in several organ systems, such as the pancreas, gastrointestinal tract, liver and the respiratory system. Since discovery of the CFTR gene in 1989, research has focussed on targeting the underlying genetic defect to identify a disease-modifying treatment for CF. Investigated management strategies have included gene therapy and the development of small molecules that target CFTR mutations, known as CFTR modulators. CFTR modulators are typically identified by high-throughput screening assays, followed by preclinical validation using cell culture systems. Recently, one such modulator, the CFTR potentiator ivacaftor, was approved as an oral therapy for CF patients with the G551D-CFTR mutation. The clinical development of ivacaftor not only represents a breakthrough in CF care but also serves as a noteworthy example of personalised medicine.

  13. Lymphoepithelial cysts and cystic lymphangiomas: Underrecognized benign cystic lesions of the pancreas

    Institute of Scientific and Technical Information of China (English)

    Ioannis; T; Konstantinidis; Avinash; Kambadakone; Onofrio; A; Catalano; Dushyant; V; Sahani; Vikram; Deshpe; David; G; Forcione; Jennifer; A; Wargo; Carlos; Fernandez-del; Castillo; Keith; D; Lillemoe; Andrew; L; Warshaw; Cristina; R; Ferrone

    2014-01-01

    AIM: To identify their diagnostic and prognostic clinical characteristics in a large series.METHODS: Retrospective review of clinicopathologic and imaging characteristics of patients diagnosed with lymphoepithelial cysts and cystic lymphangiomas of the pancreas at Massachusetts General Hospital.RESULTS: Twelve patients were identified between 1/1/1997 and 8/1/2007. Their median age was 55.5 years(range 19-78 years), and 6 were females. The le-sion was incidentally discovered in half of the patients.Contrast enhanced computed tomography demonstrat-ed that the cysts had thin walls, without calcifications, pancreatic duct dilation or pancreatic parenchyma inva-sion. Endoscopic ultrasound with fine needle aspiration(EUS/FNA) confirmed the diagnosis of a lymphoepithe-lial cyst in 3 patients, one of whom was spared an op-eration and continues to do well after 6 years. Eleven patients had a resection: 3 pancreaticoduodenecto-mies, 7 distal pancreatectomies, and 1 enucleation. The median size of the cysts was 3 cm(range 2-20 cm). At a median follow-up of 57 mo no recurrences or other pancreas-related conditions occurred.CONCLUSION: Lymphoepithelial cysts and cystic lymphangiomas of the pancreas can be diagnosed with a combination of contrast-enhanced computed tomog-raphy scans and EUS/FNA. If the lesion is asymptom-atic, an operation might be avoided.

  14. Targeting a genetic defect: cystic fibrosis transmembrane conductance regulator modulators in cystic fibrosis.

    Science.gov (United States)

    Derichs, Nico

    2013-03-01

    Cystic fibrosis (CF) is caused by genetic mutations that affect the cystic fibrosis transmembrane conductance regulator (CFTR) protein. These mutations can impact the synthesis and transfer of the CFTR protein to the apical membrane of epithelial cells, as well as influencing the gating or conductance of chloride and bicarbonate ions through the channel. CFTR dysfunction results in ionic imbalance of epithelial secretions in several organ systems, such as the pancreas, gastrointestinal tract, liver and the respiratory system. Since discovery of the CFTR gene in 1989, research has focussed on targeting the underlying genetic defect to identify a disease-modifying treatment for CF. Investigated management strategies have included gene therapy and the development of small molecules that target CFTR mutations, known as CFTR modulators. CFTR modulators are typically identified by high-throughput screening assays, followed by preclinical validation using cell culture systems. Recently, one such modulator, the CFTR potentiator ivacaftor, was approved as an oral therapy for CF patients with the G551D-CFTR mutation. The clinical development of ivacaftor not only represents a breakthrough in CF care but also serves as a noteworthy example of personalised medicine. PMID:23457166

  15. Infections with Pseudomonas aeruginosa in patients with cystic fibrosis.

    Science.gov (United States)

    Tümmler, B; Bosshammer, J; Breitenstein, S; Brockhausen, I; Gudowius, P; Herrmann, C; Herrmann, S; Heuer, T; Kubesch, P; Mekus, F; Römling, U; Schmidt, K D; Spangenberg, C; Walter, S

    1997-02-01

    The lung infection with Pseudomonas aeruginosa is regarded as one of the major causes of health decline in patients with cystic fibrosis (CF). The CF host response to the persistent bacterial antigen load in the endobronchiolar lumen is characterized by a pronounced humoral response, local production of cytokines, influx of neutrophils into the lung and a protease-protease inhibitor imbalance predominantly sustained by released neutrophil elastase. CF is an autosomal recessive disease, and we could demonstrate for our local patient population that the age-dependent risk to become chronically colonized with P. aeruginosa can be differentiated by the disease-causing CFTR mutation genotype. The age-specific colonisation rates were significantly lower in pancreas sufficient than in pancreas insufficient patients. P. aeruginosa is occasionally detected in throat swabs already in infancy or early childhood in most patients although there is a lapse of several years amenable to preventive measures such as vaccination until onset of persistent colonization. The epidemiology of the infection with P. aeruginosa was investigated by quantitative macrorestriction fragment pattern analysis. The distribution and frequency of clones found in CF patients match that found in other clinical and environmental aquatic habitats, but the over-representation of specific clones at a CF clinic indicates a significant impact of nosocomial transmission for the prevalence of P. aeruginosa-positive patients at a particular center. Most patients remain colonized with the initially acquired P. aeruginosa clone. According to direct sputum analysis the majority of patients is carrying a single clonal variant at a concentration of 10(7)-10(9) CFU. Co-colonization with other species or other clones is infrequent. Independent of the underlying genotype, the CF lung habitat triggers a uniform, genetically fixed conversion of bacterial phenotype. Most CFP, aeruginosa strains become non-motile, mucoid

  16. 17 CFR 210.8-06 - Real estate operations acquired or to be acquired.

    Science.gov (United States)

    2010-04-01

    ... 17 Commodity and Securities Exchanges 2 2010-04-01 2010-04-01 false Real estate operations acquired or to be acquired. 210.8-06 Section 210.8-06 Commodity and Securities Exchanges SECURITIES AND... Statements of Smaller Reporting Companies § 210.8-06 Real estate operations acquired or to be acquired....

  17. The value of sonography in determining cystic duct patency.

    Science.gov (United States)

    McGrath, F P; Gibney, R G; Burhenne, H J

    1992-07-01

    A prospective blinded comparison of ultrasonography (US) and oral cholecystography (OCG) was performed in 100 patients with symptomatic gall-stones to determine whether US would enable an accurate assessment of cystic duct patency to be made. Patency of the cystic duct was defined as gall-bladder opacification on OCG or a greater than 20% decrease in gall-bladder volume by US post-fatty meal. The ellipsoid method of volume measurement was used. Any patient who had a non-opacified gall-bladder on OCG but a greater than 20% volume decrease on US had cholescintigraphy performed (DISIDA). Oral cholecystography demonstrated cystic duct patency in 88 patients (88%), and fatty-meal gall-bladder US met the specified study criteria for patency in 86 patients (86%). False negative results were identified in four of the OCG and in six of the US examinations. The results of this study indicate that gall-bladder sonography with a post-fatty meal contraction of greater than 20% is a very accurate predictor of cystic duct patency. A contraction of less than 20%, however, cannot be considered a reliable predictor of cystic duct occlusion. PMID:1643780

  18. Multiple Gastric Carcinomas Associated with Potter Type III Cystic Disease

    Directory of Open Access Journals (Sweden)

    Kenji Mimatsu

    2011-10-01

    Full Text Available We report a case of multiple gastric carcinomas associated with Potter type III cystic disease of the liver, mesenterium and kidney. A 65-year-old man with chronic renal failure due to polycystic kidneys and under hemodialysis treatment 3 times a week for 2 years was admitted to our hospital because of anemia. He stated that his sister had suffered from polycystic kidney disease. Gastrointestinal fiberscopy showed two lesions in the lesser curvature in the lower portion of the stomach, and histopathological analysis of the gastric tumor biopsies revealed that one of the tumors was a papillary adenocarcinoma and the other a poorly differentiated adenocarcinoma. Helicobacter pylori infection was not detected in the stomach mucosa. Abdominal computed tomography scan revealed polycystic lesions in the liver, mesenterium and both kidneys. These imaging findings and family history suggested that the patient suffered from multiple gastric carcinomas associated with Potter type III cystic disease of the liver, mesenterium and kidney. Reports on the association of malignant neoplasm with Potter type III cystic disease are extremely rare. Especially, no case of the association of gastric carcinoma with Potter type III cystic disease of the liver and kidney has been described previously. This is a first report of the association of gastric carcinoma with Potter type III cystic disease. We also review reports of other malignant neoplasms associated with polycystic disease.

  19. European experts consensus statement on cystic tumours of the pancreas.

    Science.gov (United States)

    Del Chiaro, Marco; Verbeke, Caroline; Salvia, Roberto; Klöppel, Gunter; Werner, Jens; McKay, Colin; Friess, Helmut; Manfredi, Riccardo; Van Cutsem, Eric; Löhr, Matthias; Segersvärd, Ralf

    2013-09-01

    Cystic lesions of the pancreas are increasingly recognized. While some lesions show benign behaviour (serous cystic neoplasm), others have an unequivocal malignant potential (mucinous cystic neoplasm, branch- and main duct intraductal papillary mucinous neoplasm and solid pseudo-papillary neoplasm). European expert pancreatologists provide updated recommendations: diagnostic computerized tomography and/or magnetic resonance imaging are indicated in all patients with cystic lesion of the pancreas. Endoscopic ultrasound with cyst fluid analysis may be used but there is no evidence to suggest this as a routine diagnostic method. The role of pancreatoscopy remains to be established. Resection should be considered in all symptomatic lesions, in mucinous cystic neoplasm, main duct intraductal papillary mucinous neoplasm and solid pseudo-papillary neoplasm as well as in branch duct intraductal papillary mucinous neoplasm with mural nodules, dilated main pancreatic duct >6mm and possibly if rapidly increasing in size. An oncological partial resection should be performed in main duct intraductal papillary mucinous neoplasm and in lesions with a suspicion of malignancy, otherwise organ preserving procedures may be considered. Frozen section of the transection margin in intraductal papillary mucinous neoplasm is suggested. Follow up after resection is recommended for intraductal papillary mucinous neoplasm, solid pseudo-papillary neoplasm and invasive cancer.

  20. Cystic micropapillary neoplasm of peribiliary glands with concomitant perihilar cholangiocarcinoma.

    Science.gov (United States)

    Uchida, Tsuneyuki; Yamamoto, Yusuke; Ito, Takaaki; Okamura, Yukiyasu; Sugiura, Teiichi; Uesaka, Katsuhiko; Nakanuma, Yasuni

    2016-02-21

    We report a case of a 75-year-old man with cystic micropapillary neoplasm of peribiliary glands detected preoperatively by radiologic examination. Enhanced computed tomography showed a low-density mass 2.2 cm in diameter in the right hepatic hilum and a cystic lesion around the common hepatic duct. Under a diagnosis of perihilar cholangiocarcinoma, right hepatectomy with caudate lobectomy and bile duct resection were performed. Pathological examination revealed perihilar cholangiocarcinoma mainly involving the right hepatic duct. The cystic lesion was multilocular and covered by columnar lining epithelia exhibiting increased proliferative activity and p53 nuclear expression; it also contained foci of micropapillary and glandular proliferation. Therefore, the lesion was diagnosed as a cystic micropapillary neoplasm of peribiliary glands and resembled flat branch-type intraductal papillary mucinous neoplasm of the pancreas. Histological examination showed the lesion was discontinuous with the perihilar cholangiocarcinoma. Immunohistochemistry showed the cystic neoplasm was strongly positive for MUC6 and that the cholangiocarcinoma was strongly positive for MUC5AC and S100P. These results suggest these two lesions have different origins. This case warrants further study on whether this type of neoplasm is associated with concomitant cholangiocarcinoma as observed in pancreatic intraductal papillary mucinous neoplasm with concomitant pancreatic duct adenocarcinoma. PMID:26900302

  1. Incidental finding of malignant renal cystic tumour diagnosed sonographically

    Directory of Open Access Journals (Sweden)

    Stojanović Milan

    2008-01-01

    Full Text Available Introduction Malignant cystic renal tumor is a rare variant of renal malignancy. Cystic neoplasm results from haemorrhage, necrosis and colliquation of a solid tumour or tumour occurring within the wall of a cyst. That pathoanatomic substratum reflects characteristic sonographic features indicating its malignant nature. It is important to distinguish a simple cyst (not requiring surgery from intracystic malignant lesion because it requires surgery. Case Outline The authors present a 59-year-old woman with a sonographic finding of a simple cyst in the upper pole of the right kidney revealed during gynaecological ultrasonography. Immediately afterwards, the radiologist performed renal sonography and its finding was a cystic lesion suggestive of malignancy. Further evaluation by CT scan showed that the lesion was clearly malignant. After surgery, the histological finding verified cystic renal cancer. Conclusion Ultrasonography may reveal a complex cyst and solid mass but requires an experienced sonographer. Contrast CT scan would be performed to examine the "suspicious" lesion because it clearly shows if a cystic lesion is benign or malignant. .

  2. Phagocytosis of Pseudomonas aeruginosa by polymorphonuclear leukocytes and monocytes: effect of cystic fibrosis serum.

    OpenAIRE

    Thomassen, M J; Demko, C A; Wood, R E; Sherman, J. M.

    1982-01-01

    It has been shown previously that serum from chronically infected patients with cystic fibrosis inhibits the phagocytosis of Pseudomonas aeruginosa by both normal and cystic fibrosis alveolar macrophages. In the present study, the ability of peripheral monocytes and polymorphonuclear leukocytes from normal volunteers and cystic fibrosis patients to phagocytize P. aeruginosa was shown not to be inhibited in the presence of serum from cystic fibrosis patients.

  3. DeltaF508 heterozygosity in cystic fibrosis and susceptibility to asthma

    DEFF Research Database (Denmark)

    Dahl, Morten; Tybjaerg-Hansen, A; Lange, P;

    1998-01-01

    Cystic fibrosis is a recessive disorder mainly characterised by lung disease. We tested the hypothesis that individuals heterozygous for the common cystic fibrosis deltaF508 mutation are at risk of obstructive pulmonary disease.......Cystic fibrosis is a recessive disorder mainly characterised by lung disease. We tested the hypothesis that individuals heterozygous for the common cystic fibrosis deltaF508 mutation are at risk of obstructive pulmonary disease....

  4. Clinical and Genetic Correlates of Exercise Performance in Young Children with Cystic Fibrosis1,2

    OpenAIRE

    McBride, Michael G.; Schall, Joan I.; Zemel, Babette S.; Stallings, Virginia A.; Ittenbach, Richard F.; Paridon, Stephen M.

    2010-01-01

    Exercise performance in individuals with cystic fibrosis has been shown to be related to the degree of pulmonary dysfunction and undernutrition and genetic profile. The aim of this study was to examine these relationships in young children with cystic fibrosis. The participants were 64 children ages 8 to 11 years (M = 9.3, SD = 0.9) with cystic fibrosis and pancreatic insufficiency recruited from 13 different U.S. Cystic Fibrosis Centers. Assigned to one of three groups by Δ...

  5. Cystic coccygeal medullary vestige presenting as a sacrococcygeal mass: a case report and MRI findings

    Energy Technology Data Exchange (ETDEWEB)

    Ito, E. [Dept. of Surgery, Branch Hospital, Nagoya Univ. School of Medicine (Japan); Watanabe, Y. [Dept. of Surgery, Branch Hospital, Nagoya Univ. School of Medicine (Japan); Harada, T. [Dept. of Surgery, Branch Hospital, Nagoya Univ. School of Medicine (Japan); Ando, H. [Dept. of Surgery, Branch Hospital, Nagoya Univ. School of Medicine (Japan); Seo, T. [Dept. of Surgery, Branch Hospital, Nagoya Univ. School of Medicine (Japan); Ito, T. [Dept. of Surgery, Branch Hospital, Nagoya Univ. School of Medicine (Japan)

    1997-03-01

    The case of a 10-month-old body with a cystic coccygeal medually vestige is presented. Although the MRI findings of this lesion resemble those of sacrococcygeal teratomas, the presence of a cystic component located at the tip of the coccyx and associated sinus formation may help in diagnosing it. Cystic coccygeal medullary vestige should be entertained in the differential diagnosis of coccygeal cystic lesions. (orig.)

  6. 75 FR 45646 - Design of Clinical Trials of Aerosolized Antimicrobials for the Treatment of Cystic Fibrosis...

    Science.gov (United States)

    2010-08-03

    ... the Treatment of Cystic Fibrosis; Public Workshop AGENCY: Food and Drug Administration, HHS. ACTION... management and/or treatment of patients with cystic fibrosis. Aerosolized antimicrobials are used to treat... design of clinical trials of aerosolized antimicrobials in patients with cystic fibrosis. The input...

  7. 21 CFR 866.5900 - Cystic fibrosis transmembrane conductance regulator (CFTR) gene mutation detection system.

    Science.gov (United States)

    2010-04-01

    ... 21 Food and Drugs 8 2010-04-01 2010-04-01 false Cystic fibrosis transmembrane conductance... DEVICES Immunological Test Systems § 866.5900 Cystic fibrosis transmembrane conductance regulator (CFTR... intended as an aid in confirmatory diagnostic testing of individuals with suspected cystic fibrosis...

  8. Airway Clearance Devices for Cystic Fibrosis

    Science.gov (United States)

    2009-01-01

    Executive Summary Objective The purpose of this evidence-based analysis is to examine the safety and efficacy of airway clearance devices (ACDs) for cystic fibrosis and attempt to differentiate between devices, where possible, on grounds of clinical efficacy, quality of life, safety and/or patient preference. Background Cystic fibrosis (CF) is a common, inherited, life-limiting disease that affects multiple systems of the human body. Respiratory dysfunction is the primary complication and leading cause of death due to CF. CF causes abnormal mucus secretion in the airways, leading to airway obstruction and mucus plugging, which in turn can lead to bacterial infection and further mucous production. Over time, this almost cyclical process contributes to severe airway damage and loss of respiratory function. Removal of airway secretions, termed airway clearance, is thus an integral component of the management of CF. A variety of methods are available for airway clearance, some requiring mechanical devices, others physical manipulation of the body (e.g. physiotherapy). Conventional chest physiotherapy (CCPT), through the assistance of a caregiver, is the current standard of care for achieving airway clearance, particularly in young patients up to the ages of six or seven. CF patients are, however, living much longer now than in decades past. The median age of survival in Canada has risen to 37.0 years for the period of 1998-2002 (5-year window), up from 22.8 years for the 5-year window ending in 1977. The prevalence has also risen accordingly, last recorded as 3,453 in Canada in 2002, up from 1,630 in 1977. With individuals living longer, there is a greater need for independent methods of airway clearance. Airway Clearance Devices There are at least three classes of airway clearance devices: positive expiratory pressure devices (PEP), airway oscillating devices (AOD; either handheld or stationary) and high frequency chest compression (HFCC)/mechanical percussion (MP

  9. Intraductal papillary mucinous neoplasms and other pancreatic cystic lesions

    Institute of Scientific and Technical Information of China (English)

    Hugh James Freeman

    2008-01-01

    Pancreatic cystic neoplasms are being increasingly recognized, even in the absence of symptoms, in large part, due to markedly improved imaging modalities such as magnetic resonance imaging (MRI)/magnetic resonance cholangio pancreatography (MRCP) and computer tomography (CT) scanning. During the past 2 decades, better imaging of these cystic lesions has resulted in definition of different types, including pancreatic intraductal papillary mucinous neoplasms (IPMN). While IPMN represent only a distinct minority of all pancreatic cancers, they appear to be a relatively frequent neoplastic form of pancreatic cystic neoplasm. Moreover, IPMN have a much better outcome and prognosis compared to pancreatic ductal adenocarcinomas. Therefore, recognition of this entity is exceedingly important for the clinician involved in diagnosis and further evaluation of a potentially curable form of pancreatic cancer.

  10. Mechanisms of the noxious inflammatory cycle in cystic fibrosis

    Directory of Open Access Journals (Sweden)

    Freyssinet Jean-Marie

    2009-03-01

    Full Text Available Abstract Multiple evidences indicate that inflammation is an event occurring prior to infection in patients with cystic fibrosis. The self-perpetuating inflammatory cycle may play a pathogenic part in this disease. The role of the NF-κB pathway in enhanced production of inflammatory mediators is well documented. The pathophysiologic mechanisms through which the intrinsic inflammatory response develops remain unclear. The unfolded mutated protein cystic fibrosis transmembrane conductance regulator (CFTRΔF508, accounting for this pathology, is retained in the endoplasmic reticulum (ER, induces a stress, and modifies calcium homeostasis. Furthermore, CFTR is implicated in the transport of glutathione, the major antioxidant element in cells. CFTR mutations can alter redox homeostasis and induce an oxidative stress. The disturbance of the redox balance may evoke NF-κB activation and, in addition, promote apoptosis. In this review, we examine the hypotheses of the integrated pathogenic processes leading to the intrinsic inflammatory response in cystic fibrosis.

  11. Hypertonic saline in treatment of pulmonary disease in cystic fibrosis.

    LENUS (Irish Health Repository)

    Reeves, Emer P

    2012-01-01

    The pathogenesis of lung disease in cystic fibrosis is characterised by decreased airway surface liquid volume and subsequent failure of normal mucociliary clearance. Mucus within the cystic fibrosis airways is enriched in negatively charged matrices composed of DNA released from colonizing bacteria or inflammatory cells, as well as F-actin and elevated concentrations of anionic glycosaminoglycans. Therapies acting against airway mucus in cystic fibrosis include aerosolized hypertonic saline. It has been shown that hypertonic saline possesses mucolytic properties and aids mucociliary clearance by restoring the liquid layer lining the airways. However, recent clinical and bench-top studies are beginning to broaden our view on the beneficial effects of hypertonic saline, which now extend to include anti-infective as well as anti-inflammatory properties. This review aims to discuss the described therapeutic benefits of hypertonic saline and specifically to identify novel models of hypertonic saline action independent of airway hydration.

  12. Hypertonic Saline in Treatment of Pulmonary Disease in Cystic Fibrosis

    Directory of Open Access Journals (Sweden)

    Emer P. Reeves

    2012-01-01

    Full Text Available The pathogenesis of lung disease in cystic fibrosis is characterised by decreased airway surface liquid volume and subsequent failure of normal mucociliary clearance. Mucus within the cystic fibrosis airways is enriched in negatively charged matrices composed of DNA released from colonizing bacteria or inflammatory cells, as well as F-actin and elevated concentrations of anionic glycosaminoglycans. Therapies acting against airway mucus in cystic fibrosis include aerosolized hypertonic saline. It has been shown that hypertonic saline possesses mucolytic properties and aids mucociliary clearance by restoring the liquid layer lining the airways. However, recent clinical and bench-top studies are beginning to broaden our view on the beneficial effects of hypertonic saline, which now extend to include anti-infective as well as anti-inflammatory properties. This review aims to discuss the described therapeutic benefits of hypertonic saline and specifically to identify novel models of hypertonic saline action independent of airway hydration.

  13. Night blindness in a teenager with cystic fibrosis.

    LENUS (Irish Health Repository)

    Roddy, Marie Frances

    2011-12-01

    This article describes the case of a 16-year-old boy with cystic fibrosis who presented with difficulty seeing in the dark. He had a history of bowel surgery at birth, and he developed cystic fibrosis liver disease and osteopenia during his teenage years. He always had good lung function. When his serum vitamin A level was checked, it was undetectable in sample. He was diagnosed with night blindness and commenced on high-dose vitamin A. His symptoms resolved within 3 days. However, it took over 1 year for his vitamin A level to return to normal. This case emphasizes the importance of monitoring vitamin levels in cystic fibrosis to detect deficiency and prevent long-term consequences, and it highlights the challenges encountered during the course of night blindness treatment.

  14. An atypical presentation of cystic fibrosis: a case report

    Directory of Open Access Journals (Sweden)

    Joshi Deepak

    2008-06-01

    Full Text Available Abstract Introduction The presentation of cystic fibrosis is dependant upon which organs are affected. Common presentations include chronic respiratory infections and malabsorption. Patients with atypical disease tend to present late in childhood or as adults. Eye manifestations of cystic fibrosis are less well known. Case presentation A 14-year-old Caucasian boy presented with tiredness and difficulty seeing at night, over a period of 6 months. Good vision was only described in bright conditions. There was no history of jaundice, steatorrhea or diarrhoea. Conclusion This is the first reported case of newly diagnosed cystic fibrosis-related liver disease in a teenage boy, whose presenting symptom was night blindness secondary to vitamin A deficiency.

  15. Intrathoracic cystic hygroma with sudden respiratory distress mimicking pneumonia

    Directory of Open Access Journals (Sweden)

    Umesh Goneppanavar

    2012-01-01

    Full Text Available Benign cystic lesions such as cystic hygroma commonly manifest as progressively increasing swelling in the neck with or without compression effects. Rarely, they present with sudden respiratory distress in instances such as infection or haematoma resulting in a sudden increase in the size of the tumour. We present a seven month old child with sudden onset respiratory distress without any obvious neck swelling. The chest X ray findings correlated with the history and were suggestive of right upper lobe pneumonia that leads to a wrong diagnosis of aspiration pneumonia. However, presence of a deviated trachea in the neck raised a suspicion of possible mass. Computed tomogram showed a large cystic mass in the right upper mediastinum with tracheal collapse. We caution intensivists and paediatricians that sudden respiratory distress in infants in the absence of obvious neck swelling does not rule out possibility of intrathoracic tumour.

  16. Breakthrough therapies: Cystic fibrosis (CF) potentiators and correctors.

    Science.gov (United States)

    Solomon, George M; Marshall, Susan G; Ramsey, Bonnie W; Rowe, Steven M

    2015-10-01

    Cystic Fibrosis is caused by mutations in the Cystic Fibrosis Transmembrane conductance Regulator (CFTR) gene resulting in abnormal protein function. Recent advances of targeted molecular therapies and high throughput screening have resulted in multiple drug therapies that target many important mutations in the CFTR protein. In this review, we provide the latest results and current progress of CFTR modulators for the treatment of cystic fibrosis, focusing on potentiators of CFTR channel gating and Phe508del processing correctors for the Phe508del CFTR mutation. Special emphasis is placed on the molecular basis underlying these new therapies and emerging results from the latest clinical trials. The future directions for augmenting the rescue of Phe508del with CFTR modulators are also emphasized. PMID:26097168

  17. Computed tomography of cystic lung lesions; Computertomographie bei zystischen Lungenerkrankungen

    Energy Technology Data Exchange (ETDEWEB)

    Grgic, A.; Heinrich, M.; Girmann, M.; Kramann, B. [Inst. fuer Radiodiagnostik, Universitaetskliniken des Saarlandes, Homburg/Saar (Germany); Wilkens, H. [Innere Medizin V-Pneumonologie, Universitaetskliniken des Saarlandes, Homburg/Saar (Germany); Uder, M. [Inst. fuer Diagnostische Radiologie, Friedrich-Alexander-Univ. Erlangen Nuernberg (Germany)

    2004-07-01

    A cystic lesion in the lung is defined as a well-demarcated epithel-lined cavity, that can be mostly filled with air, water, as well as solid material content. This definition includes a wide variety of diseases such as bronchogenic cyst, abscess formation, lymphangioleiomyomatosis, Langerhans cell histiocytosis, emphysema, bronchiectasis, and pneumatoceles. Despite the difficulties in differential diagnosis, there are some diagnostic criteria for CT-scanning helping the radiologist to differentiate between these cystic entities. Moreover, clinical informations are extremely important. The most important clinical parameters include age, sex, clinical history and symptoms. Thus, a better understanding of classic CT appearance of cystic lung disease will allow more definitive diagnosis and could, in some cases, avoid biopsy. (orig.)

  18. Retrorectal Cystic Hamartoma: A Problematic ‘Tail’

    Science.gov (United States)

    Suhani; Meena, Kusum; Ali, Shadan; Aggarwal, Lalit; Thomas, Shaji

    2014-01-01

    The retrorectal space is an uncommon seat for neoplastic masses. Retrorectal hamartoma or tailgut cyst (TGC) is an uncommon developmental cystic lesion occurring in this space which mostly occurs in middle-aged females. We recently cared for a 16-year-old girl who presented with vague lower abdominal pain and occasional constipation. Per rectal examination revealed an extraluminal mass bulging from posterior rectal wall. Preoperative radiological investigations revealed by suggested it to be a mature cystic teratoma. The patient underwent exploratory laprotomy with en masse excision of the cyst. Histopathological examination of the specimen showed it to be a TGC. This case highlights the possibility of a TGC as a differential for retrorectal cystic lesions and the need to completely excise them given the possibility of future malignant transformation. PMID:25598944

  19. Large mucinous cystic neoplasm of the pancreas associated with pregnancy

    Institute of Scientific and Technical Information of China (English)

    2008-01-01

    Mucinous cystic neoplasms (MCNs) of the pancreas occur mostly in females and are potentially sex hormone-sensitive. However, a MCN occurring during pregnancy is quite rare. A 30-year-old woman in the tenth week of pregnancy was referred to us because of a rapid increase in lett hypochondrial distending pain. On ultrasound, the patient had a large intra-abdominal cystic lesion. She was thereafter diagnosed with missed abortion and a computed tomography scan showed that the lesion was a cystic tumor 18 cm in diameter originating from the pancreatic tail. The patient subsequently underwent tumor resection with distal pancreatectomy, sparing the spleen. Histopathological analysis of the specimen revealed a pancreatic MCN with moderate dysplasia. Immunohistochemically, the tumor was positive for both estrogen and progesterone receptors. To our knowledge, this is the first reported case of pancreatic MCN with moderate zysplasia in association with pregnancy. Our case strongly indicates that pancreatic MCN is female-hormone dependent.

  20. Pancreatic cystic neoplasms: a review of preoperative diagnosis and management

    Institute of Scientific and Technical Information of China (English)

    Xue-li BAI; Qi ZHANG; Noman MASOOD; Waqas MASOOD; Yun ZHANG; Ting-bo LIANG

    2013-01-01

    Pancreatic cystic neoplasms (PCNs) are a diverse group of neoplasms in the pancreas,and are more increasingly encountered with widespread abdominal screening and improved imaging techniques.The most common types of PCNs are serous cystic neoplasms (SCNs),mucinous cystic neoplasms (MCNs),and intraductal papillary mucinous neoplasms (IPMNs).Clinicians frequently feel bewildered in the differential diagnosis and subsequent management among the various types of lesions in the pancreas,which may lead to overtreatment or delayed treatment.The current review provides recent developments in the understanding of the three most common types of PCNs,the latest modalities used in preoperative diagnosis and differential diagnosis,as well as the most up to date management.Suggestions for diagnosis and differential diagnosis of SCNs,MCNs,and IPMNs are also provided for young surgeons.Better understanding of these neoplasms is essential for clinicians to make accurate diagnosis and to provide the best management for patients.

  1. Acquired Dyslexia and Dysgraphia in Chinese

    Directory of Open Access Journals (Sweden)

    Wengang Yin

    2005-01-01

    Full Text Available Understanding how the mappings between orthography and phonology in alphabetic languages are learned, represented and processed has been enhanced by the cognitive neuropsychological investigation of patients with acquired reading and writing disorders. During the past decade, this methodology has been extended to understanding reading and writing in Chinese leading to new insights about language processing, dyslexia and dysgraphia. The aim of this paper is to review reports of patients who have acquired dyslexia and acquired dysgraphia in Chinese and describe the functional architecture of the reading and writing system. Our conclusion is that the unique features of Chinese script will determine the symptoms of acquired dyslexia and dysgraphia in Chinese.

  2. The Approach to Pseudomonas aeruginosa in Cystic Fibrosis.

    Science.gov (United States)

    Talwalkar, Jaideep S; Murray, Thomas S

    2016-03-01

    There is a high prevalence of Pseudomonas aeruginosa in patients with cystic fibrosis and clear epidemiologic links between chronic infection and morbidity and mortality exist. Prevention and early identification of infection are critical, and stand to improve with the advent of new vaccines and laboratory methods. Once the organism is identified, a variety of treatment options are available. Aggressive use of antipseudomonal antibiotics is the standard of care for acute pulmonary exacerbations in cystic fibrosis, and providers must take into account specific patient characteristics when making treatment decisions related to antibiotic selection, route and duration of administration, and site of care.

  3. Case report 467: Cystic chondroblastoma left 4th rib

    Energy Technology Data Exchange (ETDEWEB)

    Sundaram, M.; McGuire, M.H.; Naunheim, K.; Schajowicz, F.

    1988-03-01

    A case of chondroblastoma in an 18-year-old female arising from a rib near the costotransverse articulation (presumably from the epiphysis of the articular process) has been described. The destructive pattern noted in the rib, associated with a large soft tissue mass, favored a malignant lesion rather than a benign one. Histologically, this tumor had large cystic and hemorrhagic components, consistent with the diagnosis of a cystic chondroblastoma of a rib. Curiously, this patient, like two others with chondroblastoma in a rib was asymptomatic, and attention to the abnormality was drawn from a routine chest radiography. The patient remains disease-free one and a half-years following surgery.

  4. Cystic dystrophy of heterotopic pancreas in duodenal wall

    Directory of Open Access Journals (Sweden)

    Gandhi Minesh B

    2014-05-01

    Full Text Available Cystic dystrophy in heterotopic pancreas is a rare, poorly understood benign disease of gastric and duodenal wall, which was described for the first time by the French authors in 1970, who reported the presence of focal pancreatic disease localized in an area comprising the C-loop of duodenum & the head of pancreas. We report a case of 33 years old male patient with complaint of recurrent abdominal pain with the probable diagnosis of groove pancreatitis on the clinoradiological findings. Whipple surgery was done. Diagnosis of cystic dystrophy in heterotopic pancreas in duodenal wall with chronic pancreatitis was given histopathologically

  5. Aerosol scintigraphy in the assessment of therapy for cystic fibrosis

    International Nuclear Information System (INIS)

    This paper evaluates respiration therapy, counselling, and antibiotic therapy in the treatment of exacerbations of cystic fibrosis. Thirteen patients with cystic fibrosis, aged 11-32 years, who were hospitalized for exacerbation and who had sputum cultures positive for Pseudomonas were treated initially for 3 days with respiration therapy and counselling followed by 14 days of therapy with antibiotics (n = 7) or placebo (n = 6). Tc-99m-DTPA aerosol scintigraphy was performed on days, 1, 4, and 17. Scintigrams were evaluated for change in number of nonventilated segments, change in number of bronchial deposits of aerosol, and subjective overall change

  6. Plasma lactoferrin levels in pregnancy and cystic fibrosis

    International Nuclear Information System (INIS)

    Plasma lactoferrin levels have been determined by radioimmunoassay for the different weeks of normal pregnancy, in normal healthy adults and in children with and without cystic fibrosis. The lactoferrin levels were higher in pregnancy than in both male and female normal adults and showed a slight progressive increase up to week 29 and thereafter remained high. Five out of seven children with cystic fibrosis had markedly raised plasma lactoferrin levels from six to 16 times higher than the mean of a control group of children. (Auth.)

  7. Computed tomography of the thorax in children with cystic fibrosis

    International Nuclear Information System (INIS)

    We studied retrospectively the value of computed tomography of the thorax in patients suffering from cystic fibrosis. Twenty-six patients were studied, which showed as the most frequency pulmonary findings bronchial wall thickening in 22 patients (84.6), followed by bronchiectasis in 16 patients (61.5%). Less frequent finding were ill-defined patch consolidation, mucoid impaction, bullaes and atelectasis. We found a predominant distribution of bronchial wall thickening and bronchiectasis in the upper lobes of the lungs. Computed tomography is the more sensitive technique for early visualization and location of the manifestations of cystic fibrosis bronchopathy. (author)

  8. Cystic angiomatous meningioma in the cerebellopontine angle mimicking hemangioblastoma

    Directory of Open Access Journals (Sweden)

    Deb Prabal

    2010-01-01

    Full Text Available We hereby report an extremely unusual case of cystic angiomatous meningioma in the CPA region in a 58-year-old male patient. He presented with complaints of headache, repeated episodes of vomiting and increasing unsteadiness of gait. Neuroimaging showed a large multicystic left-sided tentorial tumor projecting into the cerebellum and CPA with contrast-enhancing peripheral solid rim. He underwent a left retromastoid craniectomy and total excision of the tumor. Histopathology revealed an angiomatous meningioma with predominant microvascular component and extensive cystic changes. Immunopositivity for epithelial membrane antigen (EMA, vimentin and S-100 protein proved vital in excluding a hemangioblastoma.

  9. Adenocarcinoma of the lung presenting with atypical cystic brain lesions

    Science.gov (United States)

    Costa, Ricardo; Costa, Rubens B; Bacchi, Carlos; Sarinho, Filipe

    2014-01-01

    Brain metastases occur in up to 10–30% of patients with cancer. Metastatic lesions are usually diagnosed as multiple mass lesions at the junction of the grey and white matter with associated perilesional vasogenic oedema. Cysticercosis is an endemic disease in underdeveloped countries of Africa, Central and South America and is the most common parasitic infection of the central nervous system. The classical radiological finding of neurocysticercosis is cystic lesions showing the scolex in the brain parenchyma. We report a case of metastatic adenocarcinoma of the lung presenting with cystic brain lesions mimicking neurocysticercosis. PMID:24717598

  10. Antenatal diagnosis of cystic adenomatoid malformation: Effect on patient management

    Energy Technology Data Exchange (ETDEWEB)

    Claiborne, A.K.; McAlister, W.H.; Martin, C.M.; Gast, M.J.

    1985-07-01

    Congenital adenomatoid malformation (CAM) of the lung was diagnosed at 30 weeks gestation. The mother presented with preterm labor and polyhydramnios. A complex cystic mass was seen in the right lung of the fetus. Additional radiographic and sonographic investigations prior to delivery allowed differentiation of this rare lesion from other cystic thoracic pathology of the fetal period. Careful hospital obstetric management of the mother and fetus for over 3 weeks allowed the delivery of an infant with adequate pulmonary maturity to permit stabilization and surgery on the baby in the first days of life.

  11. Antenatal diagnosis of cystic adenomatoid malformation: Effect on patient management

    International Nuclear Information System (INIS)

    Congenital adenomatoid malformation (CAM) of the lung was diagnosed at 30 weeks gestation. The mother presented with preterm labor and polyhydramnios. A complex cystic mass was seen in the right lung of the fetus. Additional radiographic and sonographic investigations prior to delivery allowed differentiation of this rare lesion from other cystic thoracic pathology of the fetal period. Careful hospital obstetric management of the mother and fetus for over 3 weeks allowed the delivery of an infant with adequate pulmonary maturity to permit stabilization and surgery on the baby in the first days of life. (orig.)

  12. Hamartoma cístico retrorretal Retrorectal cystic hamartoma

    Directory of Open Access Journals (Sweden)

    Cleverson Winston de Liz Medeiros

    2002-08-01

    Full Text Available A 32-year-old woman was referred to our institution for a pelvic mass. She was asymptomatic and was found to have a mass on rectal examination. Magnetic resonance showed a large solid-cystic mass and the patient underwent complete surgical excision with pathologic findings suggesting retrorectal cyst-hamartoma. Tailgut cysts (or retrorectal cystic hamartomas are rare congenital lesions settled in the retrorectal (presacral space. They are made up of cysts lined by multiple types of epithelium, often predominantly mucin-secreting. The lesion requires complete surgical excision to prevent complications of recurrence, infection or carcinomatous degeneration.

  13. Airway inflammatory markers in individuals with cystic fibrosis and non-cystic fibrosis bronchiectasis

    Directory of Open Access Journals (Sweden)

    Bergin DA

    2013-01-01

    Full Text Available David A Bergin, Killian Hurley, Adwait Mehta, Stephen Cox, Dorothy Ryan, Shane J O’Neill, Emer P Reeves*, Noel G McElvaney*Respiratory Research Division, Department of Medicine, Royal College of Surgeons in Ireland, Education and Research Centre, Beaumont Hospital, Dublin, Ireland*These authors share joint senior authorshipAbstract: Bronchiectasis is an airway disease characterized by thickening of the bronchial wall, chronic inflammation, and destruction of affected bronchi. Underlying etiologies include severe pulmonary infection and cystic fibrosis (CF; however, in a substantial number of patients with non-CF-related bronchiectasis (NCFB, no cause is found. The increasing armamentarium of therapies now available to combat disease in CF is in stark contrast to the limited tools employed in NCFB. Our study aimed to evaluate similarities and differences in airway inflammatory markers in patients with NCFB and CF, and to suggest potential common treatment options. The results of this study show that NCFB bronchoalveolar lavage fluid samples possessed significantly increased NE activity and elevated levels of matrix metalloproteinases 2 (MMP-2 and MMP-9 compared to healthy controls (P < 0.01; however, the levels detected were lower than in CF (P < 0.01. Interleukin-8 (IL-8 concentrations were significantly elevated in NCFB and CF compared to controls (P < 0.05, but in contrast, negligible levels of IL-18 were detected in both NCFB and CF. Analogous concentrations of IL-10 and IL-4 measured in NCFB and CF were statistically elevated above the healthy control values (P < 0.05 and P < 0.01, respectively. These results indicate high levels of important proinflammatory markers in both NCFB and CF and support the use of appropriate anti-inflammatory therapies already employed in the treatment of CF bronchiectasis in NCFB.Keywords: bronchiectasis, cystic fibrosis, proteases, inflammation

  14. Diagnosis of cystic lymphangioma of the colon by endoscopic ultrasound: Biopsy is not needed!

    Science.gov (United States)

    Bhutani, Manoop S.; Annangi, Srinadh; Koduru, Pramoda; Aggarwal, Aakash; Suzuki, Rei

    2016-01-01

    Cystic lymphangioma of the colon (CLC) is a rare benign lesion that is usually asymptomatic and found incidentally during colonoscopy. Limitations in the conventional noninvasive diagnostic techniques have led to surgical resection of these lesions for diagnostic confirmation. Classic endoscopic ultrasound (EUS) findings of colonic cystic lymphangioma are submucosal anechoic cystic spaces with septations, intact muscularis propria, and no solid component. Patients who are asymptomatic with lesions having classic appearance as cystic lymphangioma with EUS can be observed without any intervention. We herein report a case of cystic lymphangioma of distal transverse colon in an asymptomatic patient diagnosed noninvasively using 20-MHz miniprobe EUS and managed conservatively without any surgical intervention.

  15. And the Winner is – Acquired

    DEFF Research Database (Denmark)

    Henkel, Joachim; Rønde, Thomas; Wagner, Marcus

    value in case of success—that is, a more radical innovation. In the second stage, successful entrants bid to be acquired by the incumbent. We assume that entrants cannot survive on their own, so being acquired amounts to a ‘prize’ in a contest. We identify an equilibrium in which the incumbent chooses...

  16. Acquired Zinc Deficiency in an Adult Female

    OpenAIRE

    Mohanan Saritha; Divya Gupta; Laxmisha Chandrashekar; Devinder M Thappa; Nachiappa G Rajesh

    2012-01-01

    Acrodermatitis enteropathica is an autosomal recessive inherited disorder of zinc absorption. Acquired cases are reported occasionally in patients with eating disorders or Crohn′s disease. We report a 24-year-old housewife with acquired isolated severe zinc deficiency with no other comorbidities to highlight the rare occurrence of isolated nutritional zinc deficiency in an otherwise normal patient.

  17. Pancreatic tuberculosis with acquired immunodeficiency syndrome: A case report and systematic review

    Institute of Scientific and Technical Information of China (English)

    Somchai Meesiri

    2012-01-01

    Pancreatic tuberculosis (TB) is a relatively rare disease that can mimic carcinoma,lymphoma,cystic neoplasia,retroperitoneal tumors,pancreatitis or pseudocysts.Here,I report the case of a 31-year-old immigrant Burmese woman who exhibited epigastralgia,fever,weight loss and an epigastric mass.The patient was diagnosed with pancreatic TB and acquired immunodeficiency syndrome,and was treated with antituberculous drugs and percutaneous catheter drainage without a laparotomy.The clinical presentation,radiographic investigation and management of pancreatic TB are summarized in this paper to emphasize the importance of considering this rare disease in the differential diagnosis of pancreatic masses concomitant with human immunodeficiency virus infection.I also emphasize the need for both histopathological and microbiological diagnosis via fineneedle aspiration.

  18. Fungal atopy in adult cystic fibrosis.

    LENUS (Irish Health Repository)

    Henry, M

    2012-02-03

    This study set out to estimate the prevalence of atopy to a variety of common ubiquitous fungi, including A. fumigatus, in cystic fibrosis (CF), and to evaluate the investigations by which the diagnosis was made. Particular attention was paid to the usefulness of skin testing and immunoassays in detecting which patients had simple fungal atopy, and which patients were at high risk of developing allergic bronchopulmonary mycoses. This cross-sectional study included 21 adult CF patients and 20 matched controls. Serum samples were taken for the measurement of total serum IgE and specific serum IgE to nine common fungi. Immediate hypersensitivity skin prick testing to each of the fungi was also performed. Simple fungal atopy was described in subjects fulfilling the following criteria: total serum IgE > 100 KU l(-1) with specific radioimmunoassay > or = grade 1 to at least one fungus and a positive skin prick test (SPT) > or = 3 mm to the same fungus. \\'High risk\\' for developing allergic bronchopulmonary mycosis (ABPM) was described in subjects fulfilling the following criteria: total serum IgE > 200 KU l(-1) with specific radioimmunoassay > or = grade 2 to at least one fungus and a positive skin prick test (SPT) > or = 6 mm to the same fungus. The adult CF group had a significantly higher total SPT score (P=0.005) and mean total serum IgE (P<0.05) than controls. Forty-three percent of CF patients fulfilled the criteria for fungal atopy to at least a single fungus. Over half this group had an atopic tendency to more than one fungus. Nineteen percent of the CF group were at least \\'high risk\\' of developing ABPM. Skin prick testing is a better marker of fungal atopy and a better predictor of those adult CF patients at higher risk of developing ABPM than specific radioimmunoassay serum testing. There is a high prevalence of fungal atopy in the adult CF population. Total serum IgE and skin prick testing are good predictors of fungal atopy and help predict those at

  19. Cystic fibrosis, molecular genetics for all life

    Directory of Open Access Journals (Sweden)

    Ausilia Elce

    2015-10-01

    Full Text Available Cystic fibrosis (CF is the most frequent lethal autosomal recessive disorder among Caucasians (incidence: 1:2,500 newborn. In the last two decades CF prognosis considerably improved and many patients well survive into their adulthood. Furthermore, milder CF with a late onset was described. CF is a challenge for laboratory of molecular genetics that greatly contributes to the natural history of the disease since fetal age. Carrier screening and prenatal diagnosis, also by non-invasive analysis of maternal blood fetal DNA, are now available, and many labs offer preimplantation diagnosis. The major criticism in prenatal medicine is the lack of an effective multidisciplinary counseling that helps the couples to plan their reasoned reproductive choice. Most countries offer newborn screening that significantly reduce CF morbidity but different protocols based on blood trypsin, molecular analysis and sweat chloride cause a variable efficiency of the screening programs. Again, laboratory is crucial for CF diagnosis in symptomatic patients: sweat chloride is the diagnostic golden standard, but different methodologies and the lack of quality control in most labs reduce its effectiveness. Molecular analysis contributes to confirm diagnosis in symptomatic subjects; furthermore, it helps to predict the disease outcome on the basis of the mutation (genotype-phenotype correlation and mutations in a myriad of genes, inherited independently by CF transmembrane conductance regulator (CFTR, which may modulate the clinical expression of the disease in each single patient (modifier genes. More recently, the search of the CFTR mutations gained a role in selecting CF patients that may benefit from biological therapy based on correctors and potentiators that are effective in patients bearing specific mutations (personalized therapy. All such applications of molecular diagnostics confirm the “uniqueness” of each CF patient, offering to laboratory medicine the

  20. Metastatic malignant melanoma representing a multiple mesenteric cystic tumor: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Jong Lim; Woo, Ji Young [Kangnam Sacred Heart, College of Medicine, Hallym University, Seoul (Korea, Republic of)

    2008-05-15

    A metastatic malignant melanoma is a malignant tumor which can involve virtually every organ system. It has variable radiographic findings which mostly indicate solid masses in the mesentery. We report here on a case of a metastatic malignant melanoma, which is made up of multiple mesenteric cystic tumors that need to differentiate from the mesenteric cystic tumor. These include the cystic spindle cell tumor, cystic teratoma, cystic mesothelioma as well as the mesenteric cystic and the solid tumor, which in turn comprises the gastrointestinal stromal tumor, lymphoma and metastatic lesion. The metastatic malignant melanoma can offer a differential diagnosis when the image findings indicate multiple mesenteric cystic masses, multiple organic metastases, and subcutaneous nodules.