Large Omental Cystic Lymphangioma Masquerading as Mucinous Ovarian Neoplasia in an 8-Year-Old Premenarchal Girl: The Findings from Diagnostic Imaging and Laparoscopic-Assisted Excision.

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Takeda, Akihiro; Ito, Hiroaki; Nakamura, Hiromi

2017-12-01

Omental cystic lymphangioma is an extremely rare abdominal mass caused by congenital malformation. An 8-year-old premenarchal girl reported abdominal pain. Diagnostic imaging revealed a large multicystic mass measuring 22 cm in diameter, which occupied the entire abdominal cavity with ascites. Emergency laparoscopy revealed a ruptured large cystic mass originating from the greater omentum; this was followed by successful laparoscopic-assisted excision. The pathological diagnosis was omental cystic lymphangioma. The present findings show that omental cystic lymphangioma masquerading as mucinous ovarian neoplasia was a rare cause of acute abdominal events in a young girl. The present case shows that minimally invasive surgery can be a feasible option, which might achieve a favorable outcome in a young patient with an omental cystic lymphangioma. Copyright © 2017 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. All rights reserved.

Mesenteric Cystic Lymphangioma of Mesocolic Origin in a Three-Month-Old Infant: A Rare Differential Diagnosis of Abdominal Distension

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Shabbir Hussain

2015-09-01

Full Text Available Cystic lymphangioma is a rare benign tumour occurring during childhood. This tumour, caused by lymphatic system malformations, commonly occurs in head and neck regions. Herein, we report the case of a three-month-old male infant, diagnosed with a large cystic mass in the abdominal cavity and groin. The infant presented with low-grade fever and significant abdominal distension. Abdominal CT scan (with a contrast agent revealed a large cystic mass in the abdominal cavity. During surgery, the mass was found to originate from the mesentery and was inferiorly connected to the sigmoid colon. Also, a small cystic mass was detected in the left scrotal region. The mass was removed along with a portion of the sigmoid colon and the cyst in the groin. Later on, histopathological examination of the mass confirmed the diagnosis of mesenteric cystic lymphangioma.

Sonographic Finding of Scrotal Cystic Lymphangioma with Hemorrhage Caused by Percutaneous Needle Aspiration: A Case Report

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Kim, Jae Woon; Cho, Jae Ho [Yonsei University College of Medicine, Seoul (Korea, Republic of)

2011-03-15

Lymphangioma is a hamartoma, which is a benign tumor caused by congenital malformation of the lymphatic system. These tumors usually occur in the neck and axilla, and occasionally in the mediastinum, mesenterium, retroperitoneum and thigh, The scrotum and perineum are the least frequent sites. We report here on an uncommon case of cystic lymphangioma that presented as focal hemorrhage caused by percutaneous needle aspiration, and we briefly review the radiologic finding and the relevant literature

Emergency presentation of cystic lymphangioma of the colon: A case report and literature review

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Luca Lepre

2016-01-01

Conclusion: The interest in our case lies in the relatively rare diagnosis of colon lymphangioma and how the cardinal sign was anemia, which may be due to the serosanguineous cystic contents of lymphangiomatosis from the internal bleeding and can cause fatal complications that require emergency surgery.

A huge cystic craniopharyngioma

International Nuclear Information System (INIS)

Takamura, Seishi; Fukumura, Akinobu; Ito, Yoshihiro; Itoyama, Yoichi; Matsukado, Yasuhiko.

1986-01-01

The findings of computed tomography (CT) of a huge cystic craniopharyngioma in a 57-year-old woman are described. Cyst density varied from low to high levels in a short duration. Follow-up CT scans were regarded as important to diagnose craniopharyngioma. The mechanism of increment of cyst density was discussed. (author)

Scrotal and retroperitoneal lymphangioma of a 12-year-old boy: case report

International Nuclear Information System (INIS)

Zaleska-Dorobisz, U.; Koltowska, A.; Moron, K.; Jaworski, W.

2005-01-01

Lymphangiomas occurs in children quite frequently. They are most commonly localized in the head, neck, axilla, chest, abdomen, and retroperitoneum. Lymphangiomas of the scrotum are very rare and are usually clinically silent, so they are a challenge for radiologists performing US examination in children. They are obliged to differentiate the following lesions: torsion of the testicle, scrotal hernia, hydrocoele, and epididymal cyst, in addition to the masses of primary extra-testicular localization such as hemagioma, congenital vascular malformations, fibromas, and malignant tumors. Additional difficulties appear in boys who present acute scrotum syndrome. The paper reports the diagnostics and treatment of a huge multicystic lymphangioma situated in he scrotum and retroperitoneum of a 12-year-old boy who had fallen from a bicycle and presented acute scrotum. The lesion of the scrotum was surgically excised. Retroperitoneal lymphangioma was detected during this operation. Subsequent diagnostics was based on ultrasonography, computerized tomography, and magnetic resonance imaging of the abdominal cavity. The lymphangioma of the retroperitoneum was resected. The patient shows no evidence of recurrence after 7 years. Cystic lymphangiomas are rare but benign intra-scrotal, extra-testicular, painless masses in children. They are sonographically multicystic or multiloculated abnormalities and may show infiltrative extension to the perineum, retroperitoneum, or abdomen. Recognition of this entity and its extent is important for correct clinical management and may be for planning treatment. (author)

Variable ultrasonography findings of extremity lymphangioma: Pathologic correlation

International Nuclear Information System (INIS)

Oh, Jong Young; Nam, Kyung Jin; Lee, Ki Nam; Kim, Chan Sung; Lee, Jin Hwa; Kim, Dae Chul

2002-01-01

The great majority of lymphangiomas occur in the neck (75%) and axilla (20%), but extremity lymphangioma is rare. We correlate variable sonographic features of extremity lymphangioma with pathologic findings. We reviewed the sonographic findings of extremity lymphangioma in 14 patients (M:F=8:6). The all cases were histologically confirmed by operation. The variable sonographic features of extremity lymphangioma were compared to pathologic findings. The multilocular cystic mass with ill defined boundaries was distinctive sonographic appearance of extremity lymphangioma. But there were variable sonographic findings such as heterogeneous echogenic mass or homogeneous echogenic portion. The histologic section of echogenic lesion reveals clusters of abnormal

Lymphangioma causing duodenal obstruction in adult, rare presentation

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Prashant W Khade

2016-01-01

Full Text Available A submucosal lymphangioma is a rare pathology in the alimentary tract. It is a benign entity of the lymphatic system. A duodenal lymphangioma is extremely rare and has an unknown etiology. Clinical and laboratory findings are nonspecific, and they are incidentally found by endoscopy and their treatment is surgical excision. Small lesions are often asymptomatic. Recently, the authors experienced a 62-year-old man, who had a duodenal lymphangioma, showing gastric outlet obstruction symptoms. A multidetector computed tomography abdomen study revealed a single submucosal cystic lesion in second part of duodenum. The lesion was successfully excised after Whipple′s procedure. Histopathology confirmed the diagnosis of lymphangioma.

Lymphangiomas in children: correlation of sonographic and pathologic findings

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Lee, Sun Wha; Ryu, Kyung Nam; Yoon, Yup; Yu, Pil Mun

1992-01-01

The sonographic features of 23 lymphangiomas (19 pediatric patients) were compared with the pathologic findings. Nineteen lymphangiomas appeared as unicameral (n = 2) and multiloculated (n = 17) cystic masses. Remaining lesions were inhomogeneously echogenic mass with small cystic portions (n 3) and a mixed pattern (n = 1). Fourteen of the multiloculated tumors had thin septa and 6 had solid echogenic foci. The fluid within the majority of the cyst was anechoic in 8 cases and echogenic in 11 cases. Correlation of the sonographic features with the pathologic findings demonstrated that the cystic spaces corresponded to the dilated lymphatic spaces lined with endothelium, separated by septa. Echogenic fluid represented hemorrhage. The echogenic components corresponded to clusters of very smaller dilated lymphatic channels, thick fibro-fatty septa, or blood clot. The author's experience suggests that the most characteristic sonographic appearance of lymphangioma is a multiloculated cystic mass with thin septa, reflecting the preponderance of fluid-filled spaces. An atypical appearance usually reflects the presence of blood or dominancy of cavernous type. The information obtained with US imaging can help in providing a preoperative diagnosis and in planning surgical resection

Lymphangiomas in children: correlation of sonographic and pathologic findings

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Lee, Sun Wha; Ryu, Kyung Nam; Yoon, Yup [Kyung Hee University Hospital, Seoul (Korea, Republic of); Yu, Pil Mun [Dankuk University College of Medicine, Cheonan (Korea, Republic of)

1992-11-15

The sonographic features of 23 lymphangiomas (19 pediatric patients) were compared with the pathologic findings. Nineteen lymphangiomas appeared as unicameral (n = 2) and multiloculated (n = 17) cystic masses. Remaining lesions were inhomogeneously echogenic mass with small cystic portions (n 3) and a mixed pattern (n = 1). Fourteen of the multiloculated tumors had thin septa and 6 had solid echogenic foci. The fluid within the majority of the cyst was anechoic in 8 cases and echogenic in 11 cases. Correlation of the sonographic features with the pathologic findings demonstrated that the cystic spaces corresponded to the dilated lymphatic spaces lined with endothelium, separated by septa. Echogenic fluid represented hemorrhage. The echogenic components corresponded to clusters of very smaller dilated lymphatic channels, thick fibro-fatty septa, or blood clot. The author's experience suggests that the most characteristic sonographic appearance of lymphangioma is a multiloculated cystic mass with thin septa, reflecting the preponderance of fluid-filled spaces. An atypical appearance usually reflects the presence of blood or dominancy of cavernous type. The information obtained with US imaging can help in providing a preoperative diagnosis and in planning surgical resection.

Microbubbles in macrocysts - Contrast-enhanced ultrasound assisted sclerosant therapy of a congenital macrocystic lymphangioma: a case report.

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Menendez-Castro, Carlos; Zapke, Maren; Fahlbusch, Fabian; von Goessel, Heiko; Rascher, Wolfgang; Jüngert, Jörg

2017-07-06

Congenital cystic lymphangiomas are benign malformations due to a developmental disorder of lymphatic vessels. Besides surgical excision, sclerosant therapy of these lesions by intracavitary injection of OK-432 (Picibanil®), a lyophilized mixture of group A Streptococcus pyogenes, is a common therapeutical option. For an appropriate application of OK-432, a detailed knowledge about the structure and composition of the congenital cystic lymphangioma is essential. SonoVue® is a commercially available contrast agent commonly used in sonography by intravenous and intracavitary application. Here we report the case of 2 month old male patient with a large thoracic congenital cystic lymphangioma. Preinterventional imaging of the malformation was performed by contrast-enhanced ultrasound after intracavitary application of SonoVue® immediately followed by a successful sclerotherapy with OK-432. Contrast agent-enhanced ultrasound imaging offers a valuable option to preinterventionally clarify the anatomic specifications of a congenital cystic lymphangioma in more detail than by single conventional sonography. By the exact knowledge about the composition and especially about the intercystic communications of the lymphangioma sclerosant therapy becomes safer and more efficient.

Huge cystic craniopharyngioma with unusual extensions

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Kitano, I.; Yoneda, K.; Yamakawa, Y.; Fukui, M.; Kinoshita, K.

1981-09-01

The findings on computed tomography (CT) of a huge cystic craniopharyngioma in a 3-year-old girl are described. The cyst occupied both anterior cranial fossae and a part of it extended to the region of the third ventricle which was displaced posteriorly. The tumor showed no contrast enhancement after the intravenous administration of contrast medium.

Lymphangioma in pancreas: a case and literature revision

International Nuclear Information System (INIS)

Moreno-Flores, A.; Alos Company, M.J.; Solera Beltran, M.C.; Ricart Rodigro, M.; Lazaro Ventura, A.; Selfa Moreno, S.

1993-01-01

While lymphangioma is a relatively common benign tumor, it is not often located in pancreas, with less than 30 such cases published in the literature. The laboratory clinical and radiological findings are nonspecific, for which reason the definitive diagnosis is based on the pathological findings. We present the case of a patient with cystic lymphangioma of the pancreas and review the radiological findings reported in the literature. (Author)

Lymphangioma involving the urogenital system in childhood

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Michael Yap

2015-10-01

Full Text Available Lymphangioma of the urogenital system is extremely rare and we present two cases treated at our institution over a one year period. The first case is a 3 year-old boy who presented with scrotal swelling and was initially thought to have a complex hydrocele. On surgical exploration, an extratesticular multiloculated cystic mass was discovered and testis-sparing excision of the mass was performed. Pathology revealed lymphangioma. The second case is a 5 year-old male who initially presented with gross hematuria. Ultrasound was unremarkable but cystoscopy revealed varicosities extending from the bladder wall. On transurethral resection, histology showed non-specific benign vascular malformation. Magnetic resonance imaging (MRI was obtained because of persistent hematuria and showed multiple bladder lesions suggestive of lymphatic malformation. Partial cystectomy was ultimately performed and histology confirmed lymphangioma. To the author's knowledge, this represents the fifth reported case of lymphangioma of bladder.

Adult cystic hygroma: successful use of OK-432 (Picibanil).

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Woolley, S L; Smith, D R K; Quine, S

2008-11-01

We report an adult case of cystic lymphangioma treated with OK-432 (Picibanil). A case report and review of the literature concerning the use of OK-432 to treat cystic lymphangioma is presented. A 31-year-old woman developed a cystic lymphangioma four weeks post-partum. This was treated initially by aspiration, for diagnostic purposes. Investigation suggested that surgery would be challenging. A review of the literature demonstrated success with OK-432 in the treatment of this condition, although primarily in the paediatric population. This patient was successfully treated thus, and at the time of writing remained symptom free. A suggested management plan is outlined. Treatment with OK-432 is useful in the management of cystic lymphangiomas in adults and should be considered as first line treatment.

Lymphangioma of the head and neck: Four case reports

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Cho, Bong Hae; Nah, Kyung Soo [Dept. of Oral and Maxillofacial Radiology, College of Dentistry, Pusan National University, Pusan (Korea, Republic of); Jeong, Yeon Hwa [Dept. of Dental Hygiene, Yeojoo Institute of Technology, Yeojoo (Korea, Republic of)

2000-03-15

Lymphangiomas are uncommon benign congenital tumors. Most occur in the head and neck and most lesions present by the age of 2 years. We present our experience with four patients who have lymphangiomas of the head and neck with tongue involvement. First case is a 7-year-old male who has the cystic lymphangioma of left submandibular area. Second a 22-year-old female has a lesion involving the border of right tongue. Third case is the lymphangioma which occur in the right upper lip of a 6-year old male. The last patient is a 28-year old male who fell down and whose right face was swollen up. He had undergone an operation and treated with steroid before. The characteristic appearances of imaging methods were described and all lesions best depicted on T2-weighted images. Our experience indicates that MRI is useful in the diagnosis and treatment planning of lymphangioma.

Lymphangioma of the head and neck: Four case reports

International Nuclear Information System (INIS)

Cho, Bong Hae; Nah, Kyung Soo; Jeong, Yeon Hwa

2000-01-01

Lymphangiomas are uncommon benign congenital tumors. Most occur in the head and neck and most lesions present by the age of 2 years. We present our experience with four patients who have lymphangiomas of the head and neck with tongue involvement. First case is a 7-year-old male who has the cystic lymphangioma of left submandibular area. Second a 22-year-old female has a lesion involving the border of right tongue. Third case is the lymphangioma which occur in the right upper lip of a 6-year old male. The last patient is a 28-year old male who fell down and whose right face was swollen up. He had undergone an operation and treated with steroid before. The characteristic appearances of imaging methods were described and all lesions best depicted on T2-weighted images. Our experience indicates that MRI is useful in the diagnosis and treatment planning of lymphangioma.

Retiform hemangioendothelioma developed on the site of an earlier cystic lymphangioma in a six-year-old girl.

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Albertini, Anne-Fore; Brousse, Nicole; Bodemer, Christine; Calonje, Eduardo; Fraitag, Sylvie

2011-10-01

Retiform hemangioendothelioma (RH) is a rare low-grade malignancy angiosarcoma, with a high rate of local recurrence and a low metastatic risk. A 6 year-old girl with a large cervical cystic lymphangioma diagnosed by ultrasound and Doppler ultrasound, which showed a large multiloculated anechoic cyst with no flow. The lymphangioma was treated with injections of Picibanil (OK-432). The tumor regressed, but after a year, she developed a poorly limited infiltrated plaque spreading out regularly over her chest, back, and shoulder. The biopsy showed a poorly limited dermal and subcutaneous vascular proliferation composed of elongated arborising vessels lined with ovoid endothelial cells in a hobnail pattern. In addition, the deep part of the lesion showed typical features of a papillary intralymphatic angioendothelioma pattern (PILA) or Dabska tumor. The endothelial cells strongly expressed podoplanin (D2-40). A diagnosis of RH with focal areas of PILA was reached. The girl died 8 months after surgery of hypovolemic shock in a context of diffuse lymphangiomatosis with pulmonary localization. To our knowledge, RH has hardly ever been described in children. This entity exhibits a continuum with the PILA, sharing not only morphological and immunohistochemical similarities but also its ability to develop in a context of a vascular anomaly, particularly a lymphangioma. The role of Picibanil in the development of this tumor can be discussed.

Small Bowel Volvulus Induced by Mesenteric Lymphangioma in an Adult: a Case Report

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Jang, Jin Hee; Lee, Su Lim; Ku, Young Mi; An, Chang Hyeok; Chang, Eun Deok [Uijeongbu St. Mary' s Hospital, College of Medicine, The Catholic University of Korea, Uijeongbu (Korea, Republic of)

2009-06-15

Mesenteric lymphangiomas are rare abdominal masses that are seldom associated with small bowel volvulus, and especially in adult patients. We report here on an unusual case of small bowel volvulus that was induced by a mesenteric lymphangioma in a 43-year-old man who suffered from repeated bouts of abdominal pain. At multidetector CT, we noticed whirling of the cystic mesenteric mass and the adjacent small bowel around the superior mesenteric artery. Small bowel volvulus induced by the rotation of the mesenteric lymphangioma was found on exploratory laparotomy. Lymphangioma should be considered as a rare cause of small bowel volvulus in adult patients.

A Rare Diagnosis in an Infant with Upper Airway Obstruction: Lymphangioma

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Nagehan Aslan

2017-08-01

Full Text Available Lymphangiomas, or cystic hygromas, are relatively uncommon congenital malformations of the lymphatic system that usually diagnosed during the neonatal period and usually localized in the head and neck. Surgery is the first-line treatment of lymphangiomas, however, surgery can be challenging due to the close relationship between the cysts and important neurovascular structures at anatomic neighborhood and high recurrence potential. Among the alternative therapies, sclerotherapy has been at the forefront in recent years. In this paper, we report a case of a patient, who was referred with sudden respiratory distress and underwent emergency tracheostomy, and discuss lymphangiomas and treatment options in the light of the literature.

[Surgical treatment of diffuse adult orbital lymphangioma: two case studies].

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Berthout, A; Jacomet, P V; Putterman, M; Galatoire, O; Morax, S

2008-12-01

Orbital lymphangioma is a rare vascular malformation; it is a benign but severe anomaly because of its infiltrative, diffuse, and hemorrhagic nature, and its high morbidity rate. Surgical resection is a real challenge on account of the intricate architecture of the lesion. The authors report their surgical experience concerning two cases of diffuse orbital lymphangioma whose diagnosis was established in adulthood and whose surgical treatment was successful. Two patients presented with adult orbital lymphangioma. Progression was slow during the first decade and then was quickly followed by complications: major exorbitism, compressive optic neuropathy, and corneal exposure. Neuroimaging showed a diffuse and cystic orbital malformation. Surgical resection was performed as completely as possible, in one case with a Krönlein orbitotomy and in the other case only via a conjunctive route. An aspirate drain was put in the orbit for 48 h so as to prevent dead spaces forming after resection, an essential risk factor of hemorrhagic or cystic recurrence. Systemic corticotherapy was administered for the 5 days following surgery. The resection was total in one case and subtotal in the other. The surgical follow-up was uneventful with an excellent aesthetic result and an improvement in visual acuity. After 12 months, no tumoral or hemorrhagic recurrence was noted. The surgical treatment of orbital lymphangiomas is challenging because of their infiltrative nature. In diffuse forms, a complete resection is rarely possible because of the risk of sacrificing visual function. In the two cases reported herein, the resection of the extraconal portion was complete, but the intraconal portion was completely removed only in one case. Using the aspirate drain, negative pressure was maintained in the orbital cavity, preventing the formation of chocolate cysts induced by surgery. Although the clinical result was very satisfying, long-term follow-up is necessary to evaluate recurrence

[Severe iatrogenic airway obstruction due to lingual lymphangioma].

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Segado Arenas, A; Flores González, J-C; Rubio Quiñones, F; Quintero Otero, S; Hernández González, A; Pantoja Rosso, S

2011-09-01

Lymphangioma of the tongue is a rare and benign tumour involving congenital and cystic abnormalities derived from lymphatic vessels. Treatment modalities include surgery and a large number of different intralesional injections of sclerosing agents. Presently, OK-432 (Picibanil(®)) is the preferred sclerosant and when administered intralesionally will result in inflammation, sclerosis, and cicatricial contraction of the lesion. We report a case of microcystic lymphangioma of the tongue in a 5-year-old boy treated with an intralesional injection of OK-432. In the immediate postoperative period, the patient suffered severe diffuse swelling, progressive upper airway obstruction with inspiratory stridor, and respiratory distress requiring emergency fiberoptic nasotracheal intubation. Although OK-432 injections are found to be safe and effective as a first line of treatment for lymphangiomas, local swelling with potentially life-threatening airway compromise should be anticipated, especially when treating lesions near the upper airway. Copyright © 2011 Elsevier Masson SAS. All rights reserved.

Huge cystic craniopharyngioma. Changes of cyst density on computed tomography

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Takamura, Seishi; Fukumura, Akinobu; Ito, Yoshihiro; Itoyama, Yoichi; Matsukado, Yasuhiko

1986-06-01

The findings of computed tomography (CT) of a huge cystic craniopharyngioma in a 57-year-old woman are described. Cyst density varied from low to high levels in a short duration. Follow-up CT scans were regarded as important to diagnose craniopharyngioma. The mechanism of increment of cyst density was discussed.

Lymphangioma of the jejunal mesentery and jejunal polyps presenting as an acute abdomen in a teenager.

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Jayasundara, Jasb; Perera, E; Chandu de Silva, M V; Pathirana, A A

2017-03-01

Cystic lymphangioma of the small bowel mesentery is a rare clinical entity, especially after childhood. Medical literature reveals a limited number of such cases presenting as acute abdomen due to bowel obstruction, small bowel volvulus and bleeding into the tumour. We present the management experience of an 18-year-old woman who presented with rapid onset diffuse peritonism and raised inflammatory markers. Computed tomography showed a mass in the small bowel mesentery with suspicion of segmental bowel ischaemia. Emergency laparotomy revealed a mass in the mid-jejunal mesentery close to the bowel wall with no bowel ischaemia. The patient made an uncomplicated recovery after segmental bowel resection and end-to-end anastomosis. Histology confirmed the mass as a cystic lymphangioma involving the jejunal mesentery and two small jejunal polyps. Lymphangioma could be considered in the differential diagnosis of an acute abdomen in a young adult when the presentation is atypical.

OK-432 sclerotherapy for benign cystic head and neck lesions

International Nuclear Information System (INIS)

Kim, Chang Hyun; Rho, Myung Ho; Lee, Sang Wook

2003-01-01

To evaluate the efficacy of OK-432 solution for slerotheraphy of cystic lesions of the head and neck. Nineteen cystic lesions comprising ten plunging ranulas, three simple ranulas, three cystic lymphangiomas, one first branchial cleft cyst and two unknown supraclavicular cysts considered to be lymphangiomas were treated by sucking out as much liquid content as possible and then injecting the same volume of OK-432 solution under ultrasound guidance. Patients were followed up clinically and radiologically. Follow-up sonography or CT- performed after a mean interval of nine months showed total or near-total shrinkage of four plunging ranulas. However, six such lesions recurred in spite of more than one (mean, two) sclerotherapy sessions. In cases involving two simple ranulas at the floor of the mouth, failure resulted from extracystic leakage of OK-432 solution via the puncture site. Two unilocular cystic lymphangiomas completely regressed during the follow-up period (mean, seven months), but the multiocular type showed a 65% volume reduction after 12 months. A first branchial cleft cyst was markedly reduced in size, with only a small cystic portion remaining after eight months, follow-up. Two supraclavicular cysts with straw-color fluid did not respond to sclerotherapy. OK-432 sclerotherapy of macrocystic lymphangiomas is an effective and promising alternative to surgery. For other cysts, however, including plunging ranula, efficacy, varied, and 64% of such lesions recurred

OK-432 sclerotherapy for benign cystic head and neck lesions

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Kim, Chang Hyun; Rho, Myung Ho; Lee, Sang Wook [Masan Samsung Hospital, Masan (Korea, Republic of)

2003-12-01

To evaluate the efficacy of OK-432 solution for slerotheraphy of cystic lesions of the head and neck. Nineteen cystic lesions comprising ten plunging ranulas, three simple ranulas, three cystic lymphangiomas, one first branchial cleft cyst and two unknown supraclavicular cysts considered to be lymphangiomas were treated by sucking out as much liquid content as possible and then injecting the same volume of OK-432 solution under ultrasound guidance. Patients were followed up clinically and radiologically. Follow-up sonography or CT- performed after a mean interval of nine months showed total or near-total shrinkage of four plunging ranulas. However, six such lesions recurred in spite of more than one (mean, two) sclerotherapy sessions. In cases involving two simple ranulas at the floor of the mouth, failure resulted from extracystic leakage of OK-432 solution via the puncture site. Two unilocular cystic lymphangiomas completely regressed during the follow-up period (mean, seven months), but the multiocular type showed a 65% volume reduction after 12 months. A first branchial cleft cyst was markedly reduced in size, with only a small cystic portion remaining after eight months, follow-up. Two supraclavicular cysts with straw-color fluid did not respond to sclerotherapy. OK-432 sclerotherapy of macrocystic lymphangiomas is an effective and promising alternative to surgery. For other cysts, however, including plunging ranula, efficacy, varied, and 64% of such lesions recurred.

Cystic lymphangioma of the breast in an infant successfully ...

African Journals Online (AJOL)

breast lymphangioma in a 3-month-old male child, which was managed successfully by intralesional ... Annals of Pediatric Surgery 2017, 13:163–165. Keywords: breast, bleomycin, intralesional sclerosing agent, macrocystic .... histology, they are characterized by a single layer of endothelium containing, usually, a clear ...

Fatal Airway Obstruction in a Man With a Cystic Hygroma.

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Wygant, Cassandra Maria; Cohle, Stephen D

2018-05-03

We describe a 24-year-old man with a cystic hygroma of the left side of the lower neck that led to sudden death. Cystic hygroma (cystic lymphangioma) is a congenital malformation of the lymphatic system. The patient, who had a tracheostomy because of airway obstruction from the cystic hygroma, was found dead with his tracheostomy tube on the floor next to him. Complications of cystic hygroma include infiltration of the neck causing airway obstruction, dysphagia, pain, and obstructive sleep apnea.

Conjunctival lymphangioma in a 4-year-old girl revealed tuberous sclerosis complex

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Freiberg, Florentina Joyce

2016-09-01

Full Text Available Background: To present a case of conjunctival lymphangioma in a girl with tuberous sclerosis complex.Methods/results: A 4-year-old girl presented with a relapsing cystic lesion of the bulbar conjunctiva in the right eye with string-of-pearl-like dilation of lymphatic vessels and right-sided facial swelling with mild pain. Best-corrected vision was not impaired. Examination of the skin revealed three hypomelanotic macules and a lumbal Shagreen patch. Magnetic resonance imaging (MRI findings displayed minimal enhancement of buccal fat on the right side. Cranial and orbital MRI showed signal enhancement in the right cortical and subcortical areas. Genetic analysis revealed a heterozygous deletion encompassing exon 1 and 2 of the gene (tuberous sclerosis complex 1 gene, confirming the diagnosis of tuberous sclerosis complex.Conclusion: In conjunctival lymphangioma, tuberous sclerosis complex should be considered as the primary disease.

Cases of Atypical Lymphangiomas in Children

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Prashant K. Minocha

2014-01-01

Full Text Available Background. Lymphatic malformations or lymphangiomas are rare benign hamartomas that result from maldevelopment of primitive lymphatic sacs. They are most frequently found in the neck and axilla, while intra-abdominal and mediastinal lymphangiomas are uncommon. These are primarily tumours of infancy and childhood and are successfully treated with surgical excision. Summary of Cases. Five cases of lymphangioma comprising three intra-abdominal lymphangiomas and two unilateral axillary lymphangiomas presenting at one institution in Trinidad W.I. between 2005 and 2012 were examined. The presentations, location, workup, treatment, and outcome of these patients were studied. Conclusion. This paper discusses a range of extracervical lymphangioma cases seen at San Fernando General Hospital, Trinidad W.I. We report three intra-abdominal cases and the most common clinical presentations were abdominal pain and distension. Also two axillary cases were reported, which presented as painless axillary masses. The major concerns for excision of axillary lymphangioma by parents and surgeons were cosmesis and feasibility of complete resection without disruption of developing breast tissue and axillary vessels. We believe that ultrasound scan is very good at detection of the lesion, while CT is better at determining tumour content and planning for the operation. It is our opinion that complete surgical excision can be achieved.

A Supernumerary Nipple-Like Clinical Presentation of Lymphangioma Circumscriptum.

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Taylor, Dustin; Kash, Natalie; Silapunt, Sirunya

2018-01-01

Lymphangioma circumscriptum is a superficially localized variant of lymphangioma. The characteristic clinical presentation is a "frogspawn" grouping of vesicles or papulovesicles on the proximal limb or limb girdle areas. Though most lymphangiomas develop congenitally, the lymphangioma circumscriptum subtype is known to present in adults. We report a case of lymphangioma circumscriptum on the left inframammary area of an African American female with an unusual supernumerary nipple-like clinical presentation. Our patient presented with a firm, smooth, hypopigmented papule, and the clinical diagnosis of keloid was made initially. However, she returned reporting growth of the lesion and was noted to have a firm, exophytic, lobulated, pink to skin-colored nodule. Histopathological examination demonstrated dilated lymphatic vessels, consistent with the diagnosis of lymphangioma. The presentation as a firm, hypopigmented papule and later exophytic, lobulated, skin-colored nodule in our case represents a clinical presentation of lymphangioma circumscriptum not previously described in the literature. Correct diagnosis in lymphangioma circumscriptum is vital, as recurrence following surgical resection and secondary development of lymphangiosarcoma and squamous cell carcinoma following treatment with radiation have been reported. Thus, it is important to consider lymphangioma circumscriptum in the differential of similar lesions in the future to allow appropriate diagnosis, treatment, and monitoring.

Oral lymphangioma: A rare case report

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Harsha Bhayya

2015-01-01

Full Text Available Lymphangiomas are benign hamartomatous tumors of the lymphatic channels which present as developmental malformations arising from sequestration of lymphatic tissue that do not communicate with the rest of the lymphatic channels. Lymphatic vessels are filled with a clear protein-rich fluid containing few lymph cells. It can also occur in association with hemangioma. The onset of lymphangiomas are either at birth (60% to 70% or up to two years of age (90% and rare in adults. Lymphangiomas have marked predilection for the head and neck region (50-70%. The most common location in the mouth is the dorsum of tongue, followed by lips, buccal mucosa, soft palate, and floor of the mouth. On tongue, they may present as a localized or a diffused growth which may enlarge to cause macroglossia, impaired speech and difficulty in mastication. Herewith, we present a rare case of lymphangioma of tongue leading to macroglossia in a 8-year-old boy.

Unusual cystic pancreatic neoplasms -image-pathological correlations

International Nuclear Information System (INIS)

Hilendarov, A.; Simova, E.; Petrova, A.; Traikova, N.; Deenichin, G.

2013-01-01

The aim is to present the variety of signs and symptoms from the diagnostic imaging methods of atypical neoplasms of the pancreas, presented as a type of cystic lesions. This often leads to unnecessary surgery or inappropriate tracking. In 115 patients (85 men and 30 women) with cystic lesions of the pancreas ultrasonic (US),computer tomography (CT) and magnetic resonance imaging (MRI) were performed and verified through histological and macroscopic pathology preparations. The ultrasound machines equipped with linear and convex transducers, MDCT and MRI imaging systems were used. In 14 of 115 patients atypical neoplasms of the pancreas were diagnosed: two cases with macroscopic serous cystic neoplasms, two nonmucinous cystic neoplasms, two hemorrhagic mucinous neoplasms, two ductal adenocarcinomas with cystic changes, one islet cell cystic tumor, two lymphoepithetial cysts, one lymphangioma, one solid papillary epithelial neoplasm and one mucinous adenocarcinoma. The authors take into consideration and overlapping of clinical symptoms and laboratory tests. Although much of the imaging features and morphological characteristics of cystic neoplasms of the pancreas are well known, should be known about the atypical unusual images in so-called 'typical' cystic neoplasms, cystic images in solid neoplasms and various atypical tumors with cystic lesions. (authors)

Mediastinal cystic lymphangioma Linfangioma cístico do mediastino

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Sandra Saleiro

2006-11-01

Full Text Available Mediastinal lymphangioma is a rare vascular neoplasm, accounting for 0.7 to 4.5% of all the mediastinum tumors. It is a benign tumor, generally occurring as a mediastinal mass of slow growth. Most patients are asymptomatic, being incidentally diagnosed after a routine chest radiograph. The best treatment consists of complete surgical resection. Prognosis depends on the degree of resecability degree, and recurrence is not expected if the lesion is totally removed.O linfangioma mediastínico é uma neoplasia vascular rara, correspondendo a 0,7 a 4,5% de todos os tumores do mediastino. É um tumor benigno, que ocorre geralmente como uma massa mediastínica de crescimento lento. A maioria dos doentes está assintomática, sendo diagnosticado incidentalmente, após a realização de uma radiografia torácica de rotina. O tratamento de eleição consiste na ressecção cirúrgica completa. O prognóstico depende do grau de ressecabilidade e, se a lesão for totalmente removida, não se esperam recidivas.

Bilateral peri-renal lymphangioma - a case report and review of literature; Linfangioma perirrenal bilateral - relato de um caso e revisao bibliografica

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Teixeira, Sonia Marcelino; Borges, Aurea Valeria Rosa Mohana; Dinoa, Vanessa de Albuquerque [Hospital Universitario Antonio Pedro, Niteroi, RJ (Brazil). Dept. de Radiologia; Marchiori, Edson; Mello, Walter Assis de; Teixeira, Graca Helena Maia do Canto [Universidade Federal Fluminense, Niteroi, RJ (Brazil). Inst. Biomedico. Dept. de Radiologia

1996-11-01

Lymphangioma are rare benign lesions and it is difficult to state whether they are true neoplasm, hamartomas or lymphangiectasias. They are most commonly seen in children and occur in regions of the neck and axilla. They increase in size both by proliferation and by the collection of fluid, thus forming cysts. The authors report a case of a 70-year-old man, who presented abdominal swelling, a palpable mass in both flanks and polycythemia. Abdominal sonography, kidney arteriograpy, CT scan and MRI have shown multiple cysts bilaterally surrounding the kidneys without either parenchymal or functional involvement. During surgery several intercommunicating cysts were found surrounding both kidneys. Biopsies were taken from the perirenal fat, the wall of the cysts and the kidney. The histologic diagnosis was cystic lymphangioma. (author) 11 refs., 4 figs.

[Treatment of lymphangioma with OK-432 infiltration].

Science.gov (United States)

Rodríguez, J; Cáceres, F; Vargas, P

2012-10-01

The management of lymphangioma using sclerotherapy has proven to be an effective therapeutic. Our aim was to evaluate the therapeutic efficacy of OK-432 (Picibanil) in patients with lymphagioma. The study was performed from November 2010 to July 2011. Fifteen patients of both genders were diagnosed with lymphangioma, 12 days to 12 years old. All patients were infiltrated with OK-432. The studied variables were: previous surgery, localization, type of lymphangioma, number of effective injections, reduction of mass valued as excellent (100% reduction), good (reduction > 50%) and bad (reduction < 50%), presence of recurrence and complications. 40% of pacients had prior surgery and 53.3% were located in the cervical-face region. The type of macrocystic lymphangioma was present in 40% of the series, mixed type in 46.6% and microcystic type in 13.4%. The number of effective infiltrations were 3. In 6 cases (40%) the result was excellent in 5 cases (33.4%) the result was good and in 4 cases (26.6%). We had 1 recurrence (6.6%) and we haven't had complications. Injection of OK-432 in macrocystic lymphangioma and mixed had a safe therapeutic modality with satisfactory results. So it is a valid alternative to conventional surgery.

OK-432 (Picibanil) therapy for lymphangiomas in children.

Science.gov (United States)

Laranne, J; Keski-Nisula, L; Rautio, Riitta; Rautiainen, Markus; Airaksinen, Mari

2002-05-01

Lymphangiomas are benign, soft tumors that most often affect the head and neck area, usually causing marked cosmetic and functional problems. Treatment options include surgery and a large number of different sclerotherapy agents. Surgical treatment is challenging because of the need for complete excision. The risk of damage to surrounding structures or poor cosmetic results is high. Various sclerotherapy agents have been shown to have minimal effects on lymphangiomas. Their use has been associated with severe systemic, local and cosmetic side effects. OK-432 (Picibanil) is a new and promising form of sclerotherapy. An intracystic injection of OK-432 produces a local inflammatory reaction, which leads to resolution of the lesion. We have treated 11 pediatric lymphangioma patients with OK-432 with excellent results: complete regression in six, marked regression in four and no response in one case. Local swelling should be anticipated, especially when treating lesions near the upper airway. We found OK-432 injections to be safe and effective as a first line of treatment for lymphangiomas.

CAVERNOUS LYMPHANGIOMA OF THE TONGUE IN AN ADULT: A CASE REPORT

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Selin EREN

2017-04-01

Full Text Available Lymphangioma is a benign hamartomatous lesion caused by congenital malformation of the lymphatic system. This benign tumor is detected most commonly at birth or in early childhood but rarely in adults. On clinical examination, most lymphangiomas contain clear lymph fluid, but some may present as transparent vesicles containing red blood cells due to hemorrhage. In addition, lymphangioma may occur in association with hemangioma. This tumor occurs most commonly in the head and neck area, but rarely in the oral cavity. The dorsum of the tongue is the most common location in the mouth, followed by the lips, buccal mucosa, soft palate, and floor of the mouth. There are various treatment approaches for lymphangioma, but surgical excision is the preferred method. We present a case of a 26-year-old man with lymphangioma on the anterior dorsal part of the tongue, not associated with any dysfunction in mastication or speech disorders.

CT and sonographic characteristics of lymphangioma of the retoperitoneum

International Nuclear Information System (INIS)

Davidson, A.J.; Hartman, D.S.

1989-01-01

This paper reports on CT (N = 14) used to localize lymphangioma as perirenal (N = 6), pararenal (N = 7), or pelvic (N = 1). Two arose in viscera, the adrenal, and bladder. Eight were unicameral, and six were multiloculated. Septa and walls were thick in 12. Attenuation varied from chyle to soft tissue. Sonography (N = 18) was used to characterize lymphangioma as unicameral (N = 7) or multilocular (N = 11). Septa were thick in 10 of 11 multiloculated lesions. Fluid was uncomplicated, contained dependent debris, or formed elements. Six extended across several boundaries. The authors' results show distinctive CT and sonographic features that helped to differentiate lymphangioma from other retroperitoneal masses

Vacuum-assisted closure in the treatment of extensive lymphangiomas in children.

Science.gov (United States)

Katz, Michael S; Finck, Christine M; Schwartz, Marshall Z; Moront, Matthew L; Prasad, Rajeev; Timmapuri, Shaheen J; Arthur, L Grier

2012-02-01

The management of lymphangiomas in children is a complex problem with frequent recurrence and infection. Vacuum-assisted closure (VAC) devices have been shown to accelerate the healing of open wounds. We hypothesized that VAC therapy might decrease complications after resection of lymphangiomas. A retrospective review was performed on 13 children (August 2005 to April 2010) who were patients undergoing lymphangioma resection with postoperative VAC therapy. Patient demographics, size and location of the lymphangioma, VAC duration and number of changes, hospital stay, complications, need for further surgery, and length of follow-up were recorded. Thirteen children (mean age, 8 years; mean weight, 34 kg) underwent 15 operations for lymphangiomas followed by postoperative VAC therapy. Locations included the head and neck, thorax and abdomen, and lower extremity. The mean VAC duration was 19 days, and they underwent a mean of 2.6 VAC changes. Six children had operative closure of the wound at a mean of 15 days postoperative. The remaining patients underwent closure by secondary intention. There were no recurrences. Complications included VAC device malfunctions requiring intervention and wound infections. Mean follow-up was 289 days. Postoperative VAC therapy for the treatment of lymphangiomas can be an effective adjunct to surgical treatment by decreasing risks of recurrence and infection. Copyright © 2012 Elsevier Inc. All rights reserved.

Lymphangioma circumscriptum, angiokeratoma, or superficial vascular ectasia with epithelial hyperplasia?

Science.gov (United States)

Katsoulas, Nikolaos; Tosios, Konstantinos I; Argyris, Prokopios; Koutlas, Ioannis G; Sklavounou, Alexandra

2014-08-01

We report a case of lymphangioma circumscriptum (cavernous lymphangioma with epithelial hyperplasia) in a 12-year-old girl, presenting as a papillary tumor on the right dorsal side of her tongue. Microscopic examination found cavernous vascular channels lined by a single layer of CD31(+), podoplanin-positive, CD34(-) endothelial cells that occupied the papillary lamina propria and were accompanied by epithelial hyperplasia. A review of the literature on oral vascular tumors with epithelial hyperplasia, namely, lymphangioma circumscriptum and angiokeratoma, provided information that draws into question the use of these terms. Copyright © 2014 Elsevier Inc. All rights reserved.

Antenatal sonographic appearance of a large orbital encephalocele: a case report and differential diagnosis of orbital cystic mass.

Science.gov (United States)

Ahmed, Ahmed; Noureldin, Rehab; Gendy, Mohamed; Sakr, Sharif; Abdel Naby, Mahmoud

2013-06-01

Orbital meningoceles and encephaloceles are rare extracranial extensions of the brain and meninges with or without direct communication between the central nervous system and the abnormal mass. We reported a rare case of large fetal orbital encephalocele; the diagnosis was suspected initially by prenatal ultrasound and confirmed by postnatal MRI and CT scans. The differential diagnosis of an intrauterine fetal cystic orbital mass includes orbital teratoma, epidermoid inclusion cysts, hemangioma or lymphangioma, congenital cystic eye, dacryocystocele, and orbital cephalocele. Copyright © 2012 Wiley Periodicals, Inc.

Alveolar lymphangioma in infants: report of two cases.

LENUS (Irish Health Repository)

FitzGerald, Kirsten

2012-02-01

The alveolar lymphangioma is a benign but relatively rare condition found only in the oral cavities of black infants. Dentists practising in Ireland may be unaware of this condition due to its racial specificity. This paper presents two case reports of multiple alveolar lymphangiomas found in black infants in a children\\'s hospital in Ireland. The epidemiology, aetiology, clinical presentation, histology, and management options are discussed. The photographs should aid the practitioner in recognising these lesions.

Alveolar lymphangioma in infants: report of two cases.

LENUS (Irish Health Repository)

FitzGerald, Kirsten

2009-06-01

The alveolar lymphangioma is a benign but relatively rare condition found only in the oral cavities of black infants. Dentists practising in Ireland may be unaware of this condition due to its racial specificity. This paper presents two case reports of multiple alveolar lymphangiomas found in black infants in a children\\'s hospital in Ireland. The epidemiology, aetiology, clinical presentation, histology, and management options are discussed. The photographs should aid the practitioner in recognising these lesions.

A rare case of acquired lymphangioma circumscriptum of the penis.

Science.gov (United States)

Adikari, S; Philippidou, M; Samuel, M

2017-02-01

Acquired lymphangioma circumscriptum is a rare occurrence on the penis. We report a case of a 47-year-old man who presented with a single lesion of acquired lymphangioma circumscriptum on the penis resembling genital warts. We report the case to increase awareness of this rare condition which may mimic sexually transmitted infections such as genital warts.

Treatment of lymphangiomas with OK-432 (Picibanil).

Science.gov (United States)

Rautio, Riitta; Keski-Nisula, Leo; Laranne, Jussi; Laasonen, Erkki

2003-01-01

To determine the efficacy of OK-432 sclerotherapy in the treatment of lymphangiomas. The treatment was begun for 14 patients with lymphangioma. The age range of the patients at the time of the first injection was from 10 months to 42 years. Eleven of the lesions involved the head and neck region, two the thorax and one was localized in the extremity. Prior to treatment all patients were investigated with either magnetic resonance imaging, computed tomography, ultrasound or a combination of these modalities. The injections were performed with ultrasound and/or fluoroscopic guidance. Eight patients received OK-432 as first-line treatment; five were treated after surgery and one after medical therapy. On average, 2.2 intracystic injections were performed per patient. Nine of the lesions were macrocystic and five were mixed lesions. Eleven patients showed complete or marked response to the OK-432 sclerotherapy, two patients had moderate shrinkage of their lesions and only one patient showed no response to therapy. Macrocystic lesions showed the best response to therapy. Those patients who received OK-432 as first-line treatment showed complete or marked response. It was found that treatment of lymphangiomas with OK-432 was safe and effective.

[Clinical aspects and therapy of lymphangiomas, hemangiomas and nevi in the area of the head and neck].

Science.gov (United States)

Drepper, H

1985-07-01

The superficial angiomas and nevi arise from the endothelial cells, the pigment-cell-system or the epidermal cells. Hemangiomas are benign tumours of the endothelial cells appearing predominantly in female newborn infants. Depending on localisation, growth and proliferation there is a tendency for spontaneous involution. For irreversible dysfunction or anatomical deformities surgical resection during the growth phase of the hemangioma is indicated. Surgery may be necessary to improve the functional and esthetic appearance after spontaneous involution causing loose residual skin. Radiotherapy of the lesion is rarely indicated. Systemic steroid therapy in cooperation with the pediatrician should be reserved only for desperate cases such as Kasabach-Merrit-Syndrome. Port wine nevi without scars can be covered with skin tanning cosmetics. Argon-Laser-Therapy is not yet so selective that healing can be achieved with certainty and without scars. Clinical progression requires surgery, especially for racemose angiectasia. Arterial embolization should only be used under special conditions, and then only as pretreatment. Lymphangiomas are mostly angiectatic processes, especially of lymphatic vascular tissue and vessels. Even large cystic lymphangiomas can be treated quite well by surgery, but operations on large diffuse invasive lymphangiomas often cause lymphedema and infection similar to erysipelas leading to pseudorecurrence. The benign malformations of the pigment cell system require clear differentiation from malignant melanoma and its precursors. Malignant melanoma develops more frequently from congenital nevi of the deep type than from other pigmented lesions. Malignant melanomas arising from giant nevi are usually diagnosed too late so that almost all patients die. Removal of giant nevi as early as possible is recommended. The epidermal malformations, too, need accurate diagnosis. Multisymptomatic syndromes such as the Basal-Cell-Nevus-Syndrome, and vascular and

Treatment of lymphangiomas with OK-432 (Picibanil) sclerotherapy: a prospective multi-institutional trial.

Science.gov (United States)

Giguère, Chantal M; Bauman, Nancy M; Sato, Yutaka; Burke, Diane K; Greinwald, John H; Pransky, Seth; Kelley, Peggy; Georgeson, Keith; Smith, Richard J H

2002-10-01

To describe and to determine the robustness of our study evaluating the efficacy of OK-432 (Picibanil) as a therapeutic modality for lymphangiomas. Prospective, randomized trial and parallel-case series at 13 US tertiary care referral centers. Thirty patients diagnosed as having lymphangioma. Ages in 25 ranged from 6 months to 18 years. Twenty-nine had lesions located in the head-and-neck area. Every patient received a 4-dose injection series of OK-432 scheduled 6 to 8 weeks apart unless a contraindication existed or a complete response was observed before completion of all injections. A control group was observed for 6 months. Successful outcome of therapy was defined as a complete or a substantial (>60%) reduction in lymphangioma size as determined by calculated lesion volumes on computed tomographic or magnetic resonance imaging scans. Overall, 19 (86%) of the 22 patients with predominantly macrocystic lymphangiomas had a successful outcome. OK-432 should be efficacious in the treatment of lymphangiomas. Our study design is well structured to clearly define the role of this treatment agent.

Treatment of Lymphangiomas with OK-432 (Picibanil)

International Nuclear Information System (INIS)

Rautio, Riitta; Keski-Nisula, Leo; Laranne, Jussi; Laasonen, Erkki

2003-01-01

Purpose: To determine the efficacy of OK-432 sclerotherapy in the treatment of lymphangiomas. Methods: The treatment was begun for 14 patients with lymphangioma. The age range of the patients at the time of the first injection was from 10 months to 42 years. Eleven of the lesions involved the head and neck region, two the thorax and one was localized in the extremity. Prior to treatment all patients were investigated with either magnetic resonance imaging, computed tomography, ultrasound or a combination of these modalities. The injections were performed with ultrasound and/or fluoroscopic guidance. Eight patients received OK-432 as first-line treatment; five were treated after surgery and one after medical therapy. On average, 2.2 intracystic injections were performed per patient. Nine of the lesions were macrocystic and five were mixed lesions. Results: Eleven patients showed complete or marked response to the OK-432 sclerotherapy, two patients had moderate shrinkage of their lesions and only one patient showed no response to therapy. Macrocystic lesions showed the best response to therapy. Those patients who received OK-432 as first-line treatment showed complete or marked response. Conclusion: It was found that treatment of lymphangiomas with OK-432 was safe and effective

Systemic Cystic Angiomatosis Mimicking Metastatic Cancer: A Case Report and Review of the Literature

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Vivek Kumar

2017-01-01

Full Text Available Systemic cystic angiomatosis is a rare benign disorder due to the maldeveloped vascular and lymphatic system with less than 50 cases reported in literature so far. We report here a case of systemic cystic angiomatosis (SCA with multisystem involvement affecting the neck, thyroid, thoracic cavity, and skeletal system. The patient initially presented in her 4th decade of life with isolated lymphangioma in the neck requiring surgery. However, she experienced full-blown manifestations of SCA in her 6th decade which closely mimicked metastatic cancer. The diagnosis of SCA could only be established after multiple biopsies. The radiological and histological features of SCA with its course over 31 years in this patient have been described.

[Unsuccessful treatment with OK-432 picibanil for orbital lymphangioma].

Science.gov (United States)

Lanuza García, A; Bañón Navarro, R; Llorca Cardeñosa, A; Delgado Navarro, C

2012-01-01

Lymphangioma is a malformation of the lymphatic system. The classic approach is surgery. We report a case of orbital lymphangioma in a girl who was given OK-432 to avoid surgery and its complications. OK-432 is a lyophilized mixture of group A Streptococcus pyogenes which produces a fibrosis limited to the lesion with a high cure rate. The main advantages are the easy intra-lesional application. with no scars and or damage of closed areas. Its main disadvantage is a significant local inflammatory reaction. Copyright © 2011 Sociedad Española de Oftalmología. Published by Elsevier Espana. All rights reserved.

Huge mucinous cystadenoma of the pancreas mistaken for a ...

African Journals Online (AJOL)

Cystic tumors of the pancreas are rare and can be confused with pseudocysts.We present a 50 year old woman with a huge mucinous cystadenoma of the pancreas initially diagnosed and managed with a cystojejunostomy and cyst wall biopsy. She required another laparotomy and tumor excision after histological ...

A Huge Ovarian Cyst in a Middle-Aged Iranian Female

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Mohammad Kazem Moslemi

2010-05-01

Full Text Available A 38-year-old Iranian woman was found to have a huge ovarian cystic mass. Her presenting symptom was vague abdominal pain and severe abdominal distention. She underwent laparotomy and after surgical removal, the mass was found to be mucinous cystadenoma on histology.

The use of the carbon dioxide laser in head and neck lymphangioma.

Science.gov (United States)

White, B; Adkins, W Y

1986-01-01

The carbon dioxide laser has been used to treat various lesions of the head and neck, ranging from carcinomas to hemangiomas, and even including tatoos. A search of the literature does not reveal any reports of the carbon dioxide laser being used to treat lymphangioma. This report discusses the efficacy of treating lymphangioma of the air and food passages with the carbon dioxide laser, and presents three patients who have been treated in this fashion-two for palliation and one for cure.

Treatment of lymphangiomas in children: an update of Picibanil (OK-432) sclerotherapy.

Science.gov (United States)

Greinwald, J H; Burke, D K; Sato, Y; Poust, R I; Kimura, K; Bauman, N M; Smith, R J

1999-10-01

Picibanil (OK-432) is a sclerosing agent derived from a low-virulence strain of Streptococcus pyogenes that induces regression of macrocystic lymphangiomas. This report describes a prospective, nonrandomized trial to evaluate the efficacy of Picibanil in the treatment of 13 affected children ranging in age from 1 to 94 months. On average, 4.1 fluoroscopically guided intracystic injections were performed per child, with an average total dose of 0.56 mg of Picibanil. As judged by physical examination and radiographic studies, 5 children (42%) showed a complete or substantial response, and 2 children (16%) showed an intermediate response. No response was seen in 5 children (42%), 2 of whom had massive craniofacial lymphangioma. Factors that contribute to failure with Picibanil sclerotherapy are the presence of a significant microcystic component to the lesion, massive craniofacial involvement, and previous surgical resection. Macrocystic lymphangiomas of the infratemporal fossa or cervical area have the best response to therapy.

Mature cystic teratoma of the pancreas in a child

International Nuclear Information System (INIS)

Yu, C.W.; Liu, K.L.; Li, Y.W.; Lin, W.C.

2003-01-01

A cystic pancreatic tumour is rare in a child and a mature cystic teratoma of the pancreas is even rarer. This is the first demonstration of the CT appearance of such a tumour in a child. We present a 2-year-old boy who presented with a palpable abdominal mass. Abdominal CT revealed a huge cystic mass in the upper abdomen. Pathology disclosed a mature cystic teratoma originating from the pancreas. (orig.)

A Huge Ovarian Dermoid Cyst: Successful Laparoscopic Total Excision

OpenAIRE

Uyanikoglu, Hacer; Dusak, Abdurrahim

2017-01-01

Giant ovarian cysts, ≥15 cm in diameter, are quite rare in women of reproductive age. Here, we present a case of ovarian cyst with unusual presentation treated by laparoscopic surgery. On histology, mass was found to be mature cystic teratoma. The diagnostic and management challenges posed by this huge ovarian cyst were discussed in the light of the literature.

Lymphangioma circumscriptum of the penis: a case report.

Science.gov (United States)

Kokcam, Ibrahim

2007-06-01

We report a case of lymphangioma circumscriptum of the penis in a 19-year-old male. The lesions developed during puberty and resembled molluscum contagiosum and genital herpes. The case is presented because of its rarity and to increase diagnostic awareness and treatment with non-surgical intervention.

Lymphangioma of the spermatic cord

OpenAIRE

Campos, Antônio Carlos Ligocki; Costa, Marco Aurélio Raeder da; Salvalaggio, Paolo Rogério de Oliveira; Torres, Luiz Fernando Bleggi; Coelho, Júlio Cézar Uili

1998-01-01

We describe a case of a 22-year-old man that had been submitted to a left herniorraphy 11 years previously to the present admission. He returned to our hospital with another mass in the same side of the groin. At operation, several small cysts linked to the spermatic cord were demonstrated. At this time, an histological exam demonstrated the presence of conective tissue. The final histology report confirmed the diagnosis of lymphangioma of the spermatic cord in the groin region. The patient w...

Successful Vaginal Delivery despite a Huge Ovarian Mucinous Cystadenoma Complicating Pregnancy: A Case Report

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Dipak Mandi

2013-12-01

Full Text Available A 22-year-old patient with 9 months of amenorrhea and a huge abdominal swelling was admitted to our institution with an ultrasonography report of a multiloculated cystic space-occupying lesion, almost taking up the whole abdomen (probably of ovarian origin, along with a single live intrauterine fetus. She delivered vaginally a boy baby within 4 hours of admission without any maternal complication, but the baby had features of intrauterine growth restriction along with low birth weight. On the 8th postpartum day, the multiloculated cystic mass, which arose from the right ovary and weighed about 11 kg, was removed via laparotomy. A mucinous cystadenoma with no malignant cells in peritoneal washing was detected in histopathology examination. This report describes a rare case of a successful vaginal delivery despite a huge cystadenoma of the right ovary complicating the pregnancy.

OK-432 sclerotherapy in head and neck lymphangiomas: long-term follow-up result.

Science.gov (United States)

Yoo, Jae Chul; Ahn, Youngjin; Lim, Yune Syung; Hah, J Hun; Kwon, Tack-Kyun; Sung, Myung-Whun; Kim, Kwang Hyun

2009-01-01

Nonsurgical treatments, such as sclerotherapy have been attempted for head and neck lymphagiomas. Of the available sclerosing agents, picibanil has shown satisfactory short-term treatment results in many studies, but no study has presented long-term treatment results. Accordingly, in the present study, the authors retrospectively reviewed the long-term treatment results of picibanil sclerotherapy. Fifty-five lymphangioma patients who underwent picibanil sclerotherapy were enrolled. Data about initial and long-term response, recurrence, and excision rate were collected. Initial response rates were 83.5 percent and long-term response rates were 76.3 percent. Initial and the long-term response rate were equally good for lymphangioma.

Treatment of lymphangiomas of the head and neck in children by intralesional injection of OK-432 (Picibanil).

Science.gov (United States)

Brewis, C; Pracy, J P; Albert, D M

2000-04-01

The treatments previously used for lymphangiomas of the head and neck in children-surgery and intralesional injection of sclerosants-are associated with significant morbidity. A new treatment-intralesional injection of OK-432-was used for lymphangiomas of the head and neck in 11 children. The results were total shrinkage in two, marked shrinkage in two, slight shrinkage in five and no response in two. The results were not affected by previous surgery nor by whether aspiration prior to injection was possible. There were no recurrences in those children in whom shrinkage occurred and no child had subsequent surgery following injection. The results of this series support those of previous series showing that OK-432 injection is an effective and safe treatment for lymphangiomas of the head and neck in children.

Torsion of the Greater Omentum Secondary to Omental Lymphangioma in a Child: A Case Report

International Nuclear Information System (INIS)

Mistry, Kewal Arunkumar; Iyer, Dayashankar

2015-01-01

Omental cyst and omental torsion both are uncommon but important causes of acute abdomen with a difficult clinical diagnosis due to nonspecific features. Here we report a case of an eight year old child with acute abdominal pain referred for USG and CT scan which revealed two cysts in greater omentum leading to secondary omental torsion. An eight year old male child presented to casualty with severe pain abdomen since 1 day. There was no history of vomiting or altered bowel habits. The patient was febrile with tachycardia on arrival. On examination rigidity and tenderness all over abdomen were present. Serum amylase was within normal range. USG and CECT abdomen were done subsequently. USG showed two well defined cystic lesions in lower abdomen with presence of some internal echogenic debris and calcified foci in their dependent part. There was also presence of omentum with a whirl of blood vessels seen along anterior abdominal wall leading to these lesions suggesting torsion. On colour Doppler the presence of blood flow within the whirl of vessels was seen. Mild amount of free fluid was also seen in the peritoneal cavity. On CECT abdomen the findings of omental cysts and torsion of greater omentum with free fluid in abdomen were confirmed. The cysts measured 60×55 and 65×55mm on CT. The patient was taken for an emergency laparotomy for indication of acute generalized peritonitis. Two large omental cysts were found in the pelvic cavity along with torsed greater omentum along with 150 ml of hemorrhagic fluid in peritoneal cavity. The cysts and twisted necrotic part of the greater omentum were excised at surgery. No postoperative complications were observed. Histopathologic examination was suggestive of lymphangioma of omentum. Lymphangioma of the omentum is an not very uncommon however acute presentation with omental torsion and infarction is an unusual entity. Optimal utilization of preoperative imaging with USG, Doppler and contrast enhanced CT scan can provide

Lymphangioma circumscriptum: clinicopathological spectrum of 29 cases

International Nuclear Information System (INIS)

Fatima, S.; Uddin, N.; Idrees, R.; Minhas, K.; Ahmad, Z.; Ahmad, R.; Kayani, N.; Arif, M.

2015-01-01

To describe the clinicopathological spectrum of Lymphangioma Circumscriptum (LC). Study Design: Observational case series. Place and Duration of Study: Department of Pathology and Microbiology, AKUH, Karachi, from 2002 to 2012. Methodology: All reported cases of LC were retrieved from medical record. Clinical and pathological features were noted. Frequency percentages were determined. Results: There were 29 cases of LC predominantly males (62%). The mean age was 27.17 ± 15.5 years. The commonest sites was anal/perianal region (24%) followed by extremities (17%) and tongue, (14%). Vulval LC was seen in 3 patients. Two cases were described on scrotum. The lesions were most commonly suspected as viral warts, mole or polyp (in anal region). Vesicles with erosions and bleeding and localized growth were the usual clinical presentations. Four of the patients presented with swelling since birth. All were treated with surgical excision. Microscopic examination revealed acanthotic squamous epithelium with papillomatosis. The subepithelial region had collections of lymphatic channels composed of ectatic dilated vessels with serum and inflammatory cells in their lumina. The lymphatic channels were seen in deeper layers along with lymphocytic aggregates. Conclusion: Lymphangioma circumscriptum is a malformation of abnormal lymphatic channels with feeding cisterns in subcutaneous tissue. It is a benign lesion usually occurring in anal/perianal region and confused with warts. Surgical excision is preferred mode of treatment. (author)

Chylous pleural effusion associated with primary lymphedema and lymphangioma-like malformations

NARCIS (Netherlands)

Bresser, P.; Kromhout, J. G.; Reekers, J. A.; Verhage, T. L.

1993-01-01

We describe a patient with a chylous pleural effusion associated with primary lymphedema of his right leg and abdominal wall. On evaluation a generalized, severe hypoplasia of the lymphatic system turned out to be associated with hyperplastic, lymphangioma-like malformations

A young woman with a huge paratubal cyst

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Ceren Golbasi

2016-09-01

Full Text Available Paratubal cysts are asymptomatic embryological remnants. These cysts are usually diagnosed during adolescence and reproductive age. In general, their sizes are small but can be complicated by rupture, torsion, or hemorrhage. Paratubal cysts are often discovered fortuitously on routine ultrasound examination. We report a 19-year-old female patient who presented with irregular menses and abdominal pain. Ultrasound examination revealed a huge cystic mass at the right adnexial area. The diagnosis was confirmed as paratubal cyst during laporotomy and, hence, cystectomy and right salpingectomy were performed. [Cukurova Med J 2016; 41(3.000: 573-576

Full text

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Figure 2 axial CT section with contrast media showing extension of lesion. Figure 3 Photomicrograph revealing many dilated cavernous lymphatic channels filled with eosinophilic coagulum. (Haematoxylin and Eosin section Orginal magnification 40 X). Discussion. Cystic hygroma, known as cystic lymphangioma is a.

Lymphangioma circumscriptum of the penis mimicking venereal lesions.

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Gupta, S; Radotra, B D; Javaheri, S M; Kumar, B

2003-09-01

Lymphangioma circumscriptum (LC) involving the penis is rare. We report two patients with penile LC. The lesions developed in early infancy in one patient, and during puberty in the other. The lesions resembled molluscum contagiosum in one and genital warts in the other. The first patient was previously treated with a diagnosis of venereal disease. A literature search found only 4 LC patients with penile lesions reported in the English literature. These cases are presented for their rarity, and to increase diagnostic vigilance and desirability of non-intervention.

Highly selective electrocoagulation therapy: an innovative treatment for lymphangioma circumscriptum.

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Yang, Xi; Jin, Yunbo; Chen, Hui; Li, Suolan; Ma, Gang; Hu, Xiaojie; Qiu, Yajing; Yu, Wenxin; Chang, Lei; Wang, Tianyou; Lin, Xiaoxi

2014-08-01

Lymphangioma circumscriptum (LC) is a type of microcystic lymphatic malformation involving the skin and mucosa that presents as translucent vesicles of varying size with a pink, red, or black hue. Lymphangioma circumscriptum causes not only cosmetic problems but also refractory rupture, infection, lymphorrhea, and bleeding. Various invasive methods, such as surgical excision, lasers, and sclerotherapy, have been used in the past to treat LC with varying success. Herein, we report a new treatment for the management of LC. This study reports the outcomes of 12 patients (aged 4-31 years) with LC treated by electrocoagulation using a special isolated needle. Patient demographics, lesion characteristics, radiologic findings, treatment course, and clinical responses are recorded. All 12 patients who were treated with the highly selective electrocoagulation therapy achieved near-complete clearance. Minimal intra- and postoperative sequelae were observed. The local complications included mild pain (n = 9), proliferous scarring (n = 1), and ulceration (n = 1) with no systemic side effects. The mean follow-up period was 8.25 months (3-14 months). Highly selective electrocoagulation therapy is an innovative, minimally invasive technique that seems to be safe and effective for the treatment of LC; the results from our limited study population seem promising, and the observed complications are acceptable.

Cystic Fibrosis-Related Diabetes

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Kayani Kayani

2018-02-01

Full Text Available Cystic fibrosis (CF is the most common autosomal recessive disorder in Caucasian populations. Individuals with CF have seen significant increases in life expectancy in the last 60 years. As a result, previously rare complications are now coming to light. The most common of these is cystic fibrosis-related diabetes (CFRD, which affects 40–50% of CF adults. CFRD significantly impacts the pulmonary function and longevity of CF patients, yet a lack of consensus on the best methods to diagnose and treat CFRD remains. We begin by reviewing our understanding of the pathogenesis of CFRD, as emerging evidence shows the cystic fibrosis transmembrane conductance regulator (CFTR also has important roles in the release of insulin and glucagon and in the protection of β cells from oxidative stress. We then discuss how current recommended methods of CFRD diagnosis are not appropriate, as continuous glucose monitoring becomes more effective, practical, and cost-effective. Finally, we evaluate emerging treatments which have narrowed the mortality gap within the CF patient group. In the future, pharmacological potentiators and correctors directly targeting CFTR show huge promise for both CFRD and the wider CF patient groups.

Ultrasonographic findings of benign soft tissue tumors

International Nuclear Information System (INIS)

Kim, Ki Sung; Oh, Dong Heon; Jung, Tae Gun; Kim, Yong Kil; Kwon, Jung Hyeok

1994-01-01

To clarify the characteristic sonographic features of benign soft tissue tumors and to evaluate the usefulness of sonographic imaging. We retrospectively reviewed ultrasonographic images of 70 cases in 68 patients with histologically proved benign soft tissue tumors. The tumors include 33 lipomas, 11 hemangiomas, 11 lymphangiomas, 7 neurilemmomas, 4 epidermoid cysts, 2 fibromas, 1 mesenchymoma, and 1 myxoma. The sonographic appearances of the lesions were mainly solid in 53 cases(33 lipomas, 8 hemangiomas, 2 lymphangiomas, 7 neurilemmomas, 2 fibromas and 1 mesenchymoma), mainly cystic in 14 cases(1 hemangioma, 8 lymphangiomas, 4 epidermoid cysts, and 1 myxomal), and mixed in 3 cases(2 hemangiomas and 1 lymphangioma). Although an accurate histologic prediction could not be made in most cases, certain patterns appeared to be characteristic of specific tumor types. 26 cases(78%) of lipoma were seen as lentiform, iso- or hyperechoic, solid mass. Hemangioma had variable appearance and characteristic calcifications were seen in 3 cases. Unicameral or multiseptated cystic mass with variable thickness of echogenic septa and solid portion was the characteristic finding of lymhangioma. Neurilemmoma showed lobulated, oval to round , relatively hypoechoic mass or with without internal cystic portion. Sonographic evaluation of benign soft tissue tumors is useful in demonstrating the location, size, extent, and internal characteristic of the mass. A relatively confident diagnosis can made when the characteristic features of the benign soft tissue tumor are present on sonographic imaging

Ultrasonographic findings of benign soft tissue tumors

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Kim, Ki Sung; Oh, Dong Heon; Jung, Tae Gun; Kim, Yong Kil; Kwon, Jung Hyeok [Dongkang Genernal Hospital, Ulsan (Korea, Republic of)

1994-05-15

To clarify the characteristic sonographic features of benign soft tissue tumors and to evaluate the usefulness of sonographic imaging. We retrospectively reviewed ultrasonographic images of 70 cases in 68 patients with histologically proved benign soft tissue tumors. The tumors include 33 lipomas, 11 hemangiomas, 11 lymphangiomas, 7 neurilemmomas, 4 epidermoid cysts, 2 fibromas, 1 mesenchymoma, and 1 myxoma. The sonographic appearances of the lesions were mainly solid in 53 cases(33 lipomas, 8 hemangiomas, 2 lymphangiomas, 7 neurilemmomas, 2 fibromas and 1 mesenchymoma), mainly cystic in 14 cases(1 hemangioma, 8 lymphangiomas, 4 epidermoid cysts, and 1 myxomal), and mixed in 3 cases(2 hemangiomas and 1 lymphangioma). Although an accurate histologic prediction could not be made in most cases, certain patterns appeared to be characteristic of specific tumor types. 26 cases(78%) of lipoma were seen as lentiform, iso- or hyperechoic, solid mass. Hemangioma had variable appearance and characteristic calcifications were seen in 3 cases. Unicameral or multiseptated cystic mass with variable thickness of echogenic septa and solid portion was the characteristic finding of lymhangioma. Neurilemmoma showed lobulated, oval to round , relatively hypoechoic mass or with without internal cystic portion. Sonographic evaluation of benign soft tissue tumors is useful in demonstrating the location, size, extent, and internal characteristic of the mass. A relatively confident diagnosis can made when the characteristic features of the benign soft tissue tumor are present on sonographic imaging.

Spontaneous Regression of Lymphangiomas in a Single Center Over 34 Years

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Motoi Kato, MD

2017-09-01

Conclusions:. We concluded that elderly patients with macrocystic or mixed type lymphangioma may have to wait for treatment for over 3 months from the initial onset. Conversely, microcystic type could not be expected to show regression in a short period, and prompt initiation of the treatments may be required. The difference of the regression or not may depend on the characteristics of the lymph flow.

Cryotherapy for massive vulvar lymphatic leakage complicated with lymphangiomas following gynecological cancer treatment.

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Yanazume, Shintaro; Douzono, Haruhiko; Kubo, Hidemichi; Nagata, Tomomi; Douchi, Tsutomu; Kobayashi, Hiroaki

2014-11-01

Vulvar lymphatic leakage is a severe complication associated with gynecological cancer treatments. However, standard treatment strategies have not yet been determined. We encountered a rare case of a 76-year-old multiparous woman suffering from massive lymphatic fluid leakage from the entire vulva, and papules developed and were identified as lymphangiomas. A large amount of straw-colored discharge continued from all vulvar papules, which extended over the mons pubis. Nine years ago, the patient had undergone a radical hysterectomy with concurrent chemoradiation for uterine cervical cancer treatment. Her serum albumin level was 1.9 mg/dl, which was attributed to the loss of a large amount of lymph fluid due to leakage from the vulva. Her quality of life gradually decreased because of general fatigue and the need for frequent diaper exchanges every 2 h. The patient received a less-invasive treatment with cryotherapy using liquid nitrogen. She also received a multimodality treatment consisting of the intravenous administration of albumin, massage of the lower limbs and intensive rehabilitation. Cryotherapy was administered once a week for 3 months. Her discharge almost stopped and vulvar lymphangiomas decreased without any major complications. To the best of our knowledge, this is the first case report of massive lymphatic leakage complicated with vulvar lymphangiomas. Additionally, this case may represent the first successful treatment of vulva lymph leakage by cryotherapy without recurrence. Cryotherapy may have the potential to improve the quality of life as a less-invasive treatment for gynecological cancer survivors without serious complications. © The Author 2014. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

[Radical Resection of Huge Gastrointestinal Stromal Tumor of the Stomach Following Neoadjuvant Chemotherapy with lmatinib - ACase Report].

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Hiraki, Yoko; Kato, Hiroaki; Shiraishi, Osamu; Tanaka, Yumiko; Iwama, Mitsuru; Yasuda, Atsushi; Shinkai, Masayuki; Kimura, Yutaka; Imano, Motohiro; Imamoto, Haruhiko; Yasuda, Takushi

2017-11-01

The usefulness and safety of imatinibfor neoadjuvant chemotherapy for resectable gastrointestinal stromal tumor(GIST) has not been established. We reported a case of a huge GIST of the stomach that was safely resected following preoperative imatinibtherapy. A 69-year-old man was hospitalized with abdominal fullness which increased rapidly from a month ago. A CT scan showed a huge tumor containing solid and cystic component which was accompanied by an extra-wall nodule. The tumor was strongly suspected to be originated from the stomach and EUS-FNA revealed GIST. We diagnosed GIST of the stomach and initiated preoperative adjuvant chemotherapy with imatinib because there was a risk for the break of tumor capsule and composite resection of the other organs without prior chemotherapy. After the administration of imatinib4 00 mg/day for 6months, the solid component was decreased in size and its' activity by PET-CT had declined, but the size of the cystic component was not changed and the patient's complaint of fullness was not reduced. Then, after a week cessation of imatinib, we performed surgical removal of the tumor with partial gastrectomy without surgical complication during and after the operation. Imatinibwas resumed 2 weeks later postoperatively and 1 year and 8 months has passed since the operation without recurrence. Neoadjuvant chemotherapy with imatinibhas the potential to become an important therapeutic option for the treatment of huge GISTs.

Linfangioma cervical: manejo terapéutico con OK-432 (Picibanil Cervical lymphangioma: therapeutic management with OK-432 (Picibanil

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E. Valle Rodríguez

2007-12-01

Full Text Available Introducción: El linfangioma es una malformación del sistema linfático. El abordaje clásico ha sido la cirugía. El OK-432 (Picibanil tiene acción esclerosante y se está utilizando cómo primer escalón terapéutico. El objetivo es aportar un nuevo caso de linfangioma tratado con OK-432 y hacer una revisión de la literatura. Material y método: Aportamos un varón de 16 años con un linfangioma cervical macroquístico de 10 x 6 cm tratado con una dosis de OK-432. Resultados: A las 16 semanas del tratamiento, el tamaño del linfangioma era de 6 x 2 cm, siendo clínicamente inapreciable. Discusión: El tratamiento con OK-432 tiene una alta tasa de curación, con una baja tasa de recidiva y una fibrosis circunscrita a la lesión. En relación con la cirugía, se evitan cicatrices y posibles lesiones de estructuras vitales.Introduction: Lymphangioma is a malformation of the lymphatic system. The classic approach is surgery. OK-432 (Picibanil has sclerosing action and is being used as the first therapeutic step. The objective was to report a new case of lymphangioma treated with OK-432 and to review the literature. Material and method: We report the case of a 16-year-old man with a 10x6-cm macrocystic cervical lymphangioma treated with a dose of OK-432. Results: At 16 weeks of treatment, the size of the lymphangioma was 6x2 cm and it was clinically unappreciable. Discussion: OK-432 treatment has a high cure rate, low recurrence rate, and fibrosis circumscribed to the lesion. Compared to surgery, scars and possible harm to vital structures are avoided.

Cystic rectal duplication: a rare cause of neonatal bladder-outlet obstruction and hydronephrosis.

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Park, W H; Choi, S O; Park, K K

2001-03-01

A case of cystic rectal duplication (RD) is presented. A 7-day-old female was admitted with acute urinary retention, voiding difficulty, and abdominal distention since she was 4 days of age. Ultrasound and abdominal computed tomography (CT) demonstrated a huge, cystic mass in the pelvis and abdomen that resulted in acute urinary retention and bilateral hydronephrosis. CT-guided drainage of the lesion followed by transabdominal surgical excision resulted in a cure. Pathologic examination demonstrated a RD lined by respiratory epithelium.

Airway management of a rare huge-size supraglottic mass

International Nuclear Information System (INIS)

Abou-Zeid, Haitham A.; Al-Ghamdi, Abdel Mohsin A.; Al-Qurain, Abdel-Aziz A.; Mokhazy, Khalid M.

2006-01-01

Laser excision of a huge-sized supraglottic mass nearly obstructing the airway passage is a real challenge to anesthesiologists. Upper airway obstruction due to neoplasm in supraglottic region, is traditionally managed by preoperative tracheostomy, however, such a common procedure can potentially have an impact on long-term outcome. A 26-year-old patient presented with dysphagia caused by left cystic vallecular synovial sarcoma. The airway was successfully secured via fiberoptic bronchoscopy, followed by excision of the supraglottic tumor with CO2 laser surgery. Tracheostomy was not required. The patient was discharged from the hospital on the 4th day of surgery. This case, highlights the possibility to secure the airway passage without performing preoperative tracheostomy resulting in good outcome and short hospital stay. (author)

Huge endometrioma mimicking mucinous cystadenoma on MR : A case report

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Hwang, Im Kyung; Kim, Bong Soo; Nam, Kung Sook; Kim, Heung Cheol; Yoo, Yun Sik; Lee, Mee Ran; Hwang, Woo Chul [Hallym University, Chunchon (Korea, Republic of)

2001-12-01

Endometriosis is a relatively common gynecologic disease affecting women during their reproductive years. For its diagnosis, magnetic resonance imaging has been shown to have greater specificity than other modalities. Although lesions may show variable signal intensity due to numerous stages of bleeding, the characteristic finding of endometrioma which distinguishes it from other ovarian cystic masses is relatively high signal intensity on T1-weighted images and heterogeneous signal intensity with prominent shading on 72-weighted images. We report an atypical case involving a huge endometrioma. Because of varying signal intensity on T1- and T2-weighted images and scanty shading on T2-weighted images, the findings were misinterpreted and mucinous cystadenoma was diagnosed.

A huge ovarian mucinous cystadenoma associated with contralateral teratoma and polycystic ovary syndrome in an obese adolescent girl.

Science.gov (United States)

Thaweekul, Patcharapa; Thaweekul, Yuthadej; Mairiang, Karicha

2016-12-01

A 13-year-old, obese girl presented with acute abdominal pain with abdominal distension for a year. The physical examination revealed marked abdominal distension with a large well-circumscribed mass sized 13×20 cm. Her body mass index (BMI) was 37.8 kg/m2. An abdominal CT scan revealed a huge multiloculated cystic mass and a left adnexal mass. She had an abnormal fasting plasma glucose and low HDL-C. Laparotomy, right salpingooophorectomy, left cystectomy, lymph node biopsies and partial omentectomy were performed. The left ovary demonstrated multiple cystic follicles over the cortex. The histologic diagnosis was a mucinous cystadenoma of the right ovary and a matured cystic teratoma of the left ovary. Both obesity and polycystic ovary syndrome (PCOS) are associated with a greater risk of ovarian tumours, where PCOS could be either the cause or as a consequence of an ovarian tumour. We report an obese, perimenarchal girl with bilateral ovarian tumours coexistent with a polycystic ovary and the metabolic syndrome.

Intestinal Duplication Cyst Mimicking as Mesenteric Cyst with Asso- ciated Ileal Atresia Type III A

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Surekha Arakeri; Anilkumar Sirasagi

2013-01-01

Intestinal duplication cysts (IDC) are uncom-mon congenital malformations that couldpresent diagnostic and therapeutic challenge.They may be often mistaken as mesentericcysts, omental cyst, cystic lymphangioma etc.However, IDC are differentiated from otherintra-abdominal cystic lesions by presence ofgastrointestinal mucosal lining and smoothmuscles in their wall. We report a case of IDCmimicking as mesenteric cyst associated withatresia of ileum in a neonate presented withacute surgical emerg...

Picibanil (OK-432) in the treatment of head and neck lymphangiomas in children

OpenAIRE

Rebuffini, Elena; Zuccarino, Luca; Grecchi, Emma; Carinci, Francesco; Merulla, Vittorio Emanuele

2012-01-01

Background: Picibanil (OK-432) is a lyophilized mixture of group A Streptococcus pyogenes with antineoplastic activity. Because of its capacity to produce a selective fibrosis of lymphangiomas (LMs), it has been approved by Japanese administration in 1995 for the treatment of LMs. Materials and Methods: We treated 15 children (age range: 6-60 months) affected by head and neck macrocystic LMs with intracystic injections (single dose of 0.2 mL) of Picibanil (1-3 injections). Results: Co...

Linfangioma bucal del tipo capilar: Reporte de un caso en un paciente adulto Capillary buccal lymphangioma: Case report in adult patient

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Fabiano Gonzaga Rodrigues

2007-03-01

Full Text Available Se describe un caso clínico de linfangioma localizado en la mucosa bucal de un paciente de 68 años de edad, tratado con eletrocauterización. La presentación del caso comprende una breve revisión de la literatura; se aborda el diagnóstico y las características clínico-patológicas en el tratamiento del linfagioma bucal.In this paper, the authors describe a case report of a buccal lymphangioma localizeted in jugal mucosa of a sixty eight years old patient, treated by the electrocauterization. Also, they present a literature revision approaching the diagnosis, the clinical and histopathological characteristics, and the different types of treatment of the buccal lymphangioma.

Lymphatic endothelial cell line (CH3) from a recurrent retroperitoneal lymphangioma.

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Way, D; Hendrix, M; Witte, M; Witte, C; Nagle, R; Davis, J

1987-09-01

An endothelial cell line derived from a massive recurrent chyle-containing retroperitoneal lymphangioma was isolated in monolayer culture. Scanning and transmission electron microscopy and immunohistochemistry confirmed a close resemblance to blood vascular endothelium with typical cobblestone morphology, positive immunofluorescence staining for endothelial marker Factor VIII-associated antigen and fibronectin, and prominent Weibel-Palade bodies. The endothelial cells also exhibited other ultrastructural features characteristic of lymphatic endothelium, including sparse microvillous surface projections, overlapping intercellular junctions, and abundant intermediate filaments. This endothelial cell line represents a new source of proliferating lymphatic endothelium for future study, including structural and functional comparison to blood vascular endothelium.

Intestinal Duplication Cyst Mimicking as Mesenteric Cyst with Asso- ciated Ileal Atresia Type III A

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Surekha Arakeri

2013-07-01

Full Text Available Intestinal duplication cysts (IDC are uncom-mon congenital malformations that couldpresent diagnostic and therapeutic challenge.They may be often mistaken as mesentericcysts, omental cyst, cystic lymphangioma etc.However, IDC are differentiated from otherintra-abdominal cystic lesions by presence ofgastrointestinal mucosal lining and smoothmuscles in their wall. We report a case of IDCmimicking as mesenteric cyst associated withatresia of ileum in a neonate presented withacute surgical emergency.

Uso de OK-432 em crianças com linfangioma OK-432 therapy for lymphangioma in children

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Everaldo Ruiz Jr.

2004-04-01

Full Text Available OBJETIVO: Relatar a experiência no uso do OK-432 para tratamento de linfangiomas em crianças. MÉTODOS: Estudo retrospectivo de 19 crianças com linfangioma tratadas com OK-432 no Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto - USP, durante o período de 1999 a 2003. RESULTADOS: Todos os pacientes apresentaram alguma resposta ao OK-432, 12 pacientes apresentaram regressão total, sete apresentaram regressão parcial variando de 50% a 80%. Os pacientes apresentaram febre após a aplicação da droga com duração de 2 a 10 dias. Não se observaram cicatrizes após a aplicação do OK-432. CONCLUSÕES: A droga OK-432 é segura, eficaz e pode ser utilizada como primeira escolha no tratamento de pacientes com linfangiomas devido à excelente resposta, podendo tornar desnecessária a realização de cirurgia. Em pacientes com resposta parcial, podem ser realizadas novas aplicações de OK-432 ou cirurgia menos mutilante, devido à redução das dimensões da lesão.OBJECTIVE: To report the experience with OK-432 therapy for lymphangioma in children. METHODS: Retrospective study of 19 children with lymphangioma treated with OK-432 in Ribeirão Preto, state of São Paulo, Brazil, between 1999 and 2003. RESULTS: All patients presented response to OK-432, 12 had total shrinkage and seven had partial shrinkage varying from 50% to 80%. Patients had fever after injections of OK-432 for 2 to 10 days, no damage to the overlying skin was observed. CONCLUSION: OK-432 is safe, effective and can be used as primary choice of treatment of patients with lymphangiomas because of the excellent response. In these cases surgery should not be necessary. In patients with partial regression new injections of OK-432 must be used to shrink the lesion. Thereby safely surgery could be made.

Multiple lymphatic cervical cysts

International Nuclear Information System (INIS)

Jordan, J.; Piotrowski, S.; Zalewska-Rzezniczak, I.

1994-01-01

Authors described a case of 60 year-old woman with multiple lateral neck cysts. 4 cysts were located in the supraclavicular region of lateral neck triangle. During histopathological studies of postoperative specimens a cystic hygroma diagnosis was established. The fact, that cystic lymphangioma occurred in an adult woman, was interesting. The authors stress the necessity of preoperative evaluation of tumor size in view of the possibility of its penetration into the thorax. The CT examination may be useful in these cases. (author)

Non-neoplastic cystic and cystic-like lesions of the pancreas: may mimic pancreatic cystic neoplasms.

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Goh, Brian K P; Tan, Yu-Meng; Chung, Yaw-Fui A; Chow, Pierce K H; Ong, Hock-Soo; Lim, Dennis T H; Wong, Wai-Keong; Ooi, London L P J

2006-05-01

Cystic lesions of the pancreas consist of a broad range of pathological entities. With the exception of the pancreatic pseudocyst, these are usually caused by pancreatic cystic neoplasms. Non-neoplastic pancreatic cystic and cystic-like lesions are extremely rare. In the present article, the surgical experience with these unusual entities over a 14-year period is reported. Between 1991 and 2004, all patients who underwent surgical exploration for a cystic lesion of the pancreas were retrospectively reviewed. Patients with a pancreatic pseudocyst were excluded. There were 106 patients of whom 8 (7.5%) had a final pathological diagnosis consistent with a non-neoplastic pancreatic cystic or cystic-like lesion, including 3 patients with a benign epithelial cyst, 2 with a pancreatic abscess (one tuberculous and one foreign body), 2 with mucous retention cysts and 1 with a mucinous non-neoplastic cyst. These eight patients are the focus of this study. There were six female and two male patients with a median age of 61.5 years (range, 41-71 years). All the patients were of Asian origin including seven Chinese and one Indian. Four of the patients were asymptomatic and their pancreatic cysts were discovered incidentally on radiological imaging for other indications. All the patients underwent preoperative radiological investigations, including ultrasonography, computed tomography or magnetic resonance imaging, which showed a cystic lesion of the pancreas. Three patients, all of whom were symptomatic, were diagnosed preoperatively with a malignant cystic neoplasm on the basis of radiological imaging. Two patients were eventually found to have a pancreatic abscess, one tuberculous and the other, secondary to foreign body perforation. The third patient was found on final histology to have chronic pancreatitis with retention cysts. The remaining five patients had a preoperative diagnosis of an indeterminate cyst; on pathological examination, they were found to have a benign

Cystic medulloblastoma

International Nuclear Information System (INIS)

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

1989-01-01

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

CT findings of palpable neck masses in children

International Nuclear Information System (INIS)

Park, Chan Sup; Chung, Won Mo; Seok, Eul Hye; Suh, Chang Hae; Chung, Won Kyun

1994-01-01

We performed this study to assess the value of CT in the differential diagnosis of palpable neck masses in children. We retrospectively reviewed the CT scans of the palpable neck masses in 30 children. The masses were proved histopathologically and classified into cystic, solid, and inflammatory mass and their CT findings were analyzed. Twelve cases were cystic masses, 4 were solid masses, and 14 were inflammatory lesions. Cystic masses included cystic lymphangiomas (n=6), branchial cleft cysts (n=3), thyroglossal duct cysts (n=2), and ranula (n=1). Cystic lymphangiomas showed insinuating appearances into adjacent structures and 4 cases occurred in the posterior cervical space. All branchial cleft cysts were round cystic masses with smooth wall and displaced the submandibular gland anteriorly and the sternocleidomastoid muscle posteriorly. Two thyroglossal duct cysts occurred centrally adjacent to the hyoid bone and 1 ranula in the submental area. Solid masses were juvenile hemangioma, pleomorphic adenoma in submandibular gland, neurilemmoma, and fibromatosis colli. Juvenile hemangioma showed well-enhancing mass with indistinct margin and the other solid masses had well-defined margin with their characteristic location. Inflammatory lesions were abscess (n=4), deep neck infections with lymphadenopathy (n=4), submandibular gland inflammation (n=3), and tuberculous lymphadenitis (n=3) and they showed strand-like enhancement in adjacent subcutaneous tissues. Tuberculous lymphadenitis had multiple lymph node enlargement with internal low attenuation areas and showed less surrounding strand-like enhancement than suppurative lymphadenopathies. Most neck masses in infants and children were of congenital or inflammatory origin. CT is useful for the evaluation of the child presenting with a neck mass, because it can differentiate various forms of neck masses and is able to reveal the relationship of the masses to the adjacent structures with their characteristic location

CT findings of palpable neck masses in children

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Park, Chan Sup; Chung, Won Mo; Seok, Eul Hye; Suh, Chang Hae; Chung, Won Kyun [College of Medicine, Inha University, Incheon (Korea, Republic of)

1994-12-15

We performed this study to assess the value of CT in the differential diagnosis of palpable neck masses in children. We retrospectively reviewed the CT scans of the palpable neck masses in 30 children. The masses were proved histopathologically and classified into cystic, solid, and inflammatory mass and their CT findings were analyzed. Twelve cases were cystic masses, 4 were solid masses, and 14 were inflammatory lesions. Cystic masses included cystic lymphangiomas (n=6), branchial cleft cysts (n=3), thyroglossal duct cysts (n=2), and ranula (n=1). Cystic lymphangiomas showed insinuating appearances into adjacent structures and 4 cases occurred in the posterior cervical space. All branchial cleft cysts were round cystic masses with smooth wall and displaced the submandibular gland anteriorly and the sternocleidomastoid muscle posteriorly. Two thyroglossal duct cysts occurred centrally adjacent to the hyoid bone and 1 ranula in the submental area. Solid masses were juvenile hemangioma, pleomorphic adenoma in submandibular gland, neurilemmoma, and fibromatosis colli. Juvenile hemangioma showed well-enhancing mass with indistinct margin and the other solid masses had well-defined margin with their characteristic location. Inflammatory lesions were abscess (n=4), deep neck infections with lymphadenopathy (n=4), submandibular gland inflammation (n=3), and tuberculous lymphadenitis (n=3) and they showed strand-like enhancement in adjacent subcutaneous tissues. Tuberculous lymphadenitis had multiple lymph node enlargement with internal low attenuation areas and showed less surrounding strand-like enhancement than suppurative lymphadenopathies. Most neck masses in infants and children were of congenital or inflammatory origin. CT is useful for the evaluation of the child presenting with a neck mass, because it can differentiate various forms of neck masses and is able to reveal the relationship of the masses to the adjacent structures with their characteristic location.

Nonfunctional Cystic Hepatic Paraganglioma Mimicking Hydatid Cyst

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Preeti Balkisanji Agrawal

2017-11-01

Full Text Available An extra-adrenal pheochromocytoma is also known as a paraganglioma. We are reporting the case of a 68-year-old female patient with an extremely rare primary nonfunctioning hepatic paraganglioma without any clinical signs and symptoms. A CECT scan of whole abdomen was done which showed a huge well defined peripherally enhancing fluid density cystic lesion measuring 14 cm × 14 cm × 12 cm with internal enhancing septations and few foci of calcification involving V to VIII segments of right lobe of liver which was compressing and displacing portal vein, its right branch, common bile duct, gall bladder. Mass was also compressing and displacing right kidney inferiorly. Laparotomy was done and sample of cyst wall sent for histopathology. After immunohistochemistry a diagnosis of paraganglioma was confirmed.

Cystic Fibrosis (CF): Chloride Sweat Test

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

Ultrasound Imaging of Cystic Nephroma

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Federico Greco

2017-07-01

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

Congenital cystic eyeball

Directory of Open Access Journals (Sweden)

Gupta V

1990-01-01

Full Text Available A rare case of histopathologically proved case of congenital cystic eye in a one day old girl is described. It was an unusually large cystic mass bulging forwards stretching the upperlid. There was no rudimentary eyeball in the orbit. The cystic eye′s predilection for the left eye has been pointed out for the first time in this article.

Safety of huge systems

International Nuclear Information System (INIS)

Kondo, Jiro.

1995-01-01

Recently accompanying the development of engineering technology, huge systems tend to be constructed. The disaster countermeasures of huge cities become large problems as the concentration of population into cities is conspicuous. To make the expected value of loss small, the knowledge of reliability engineering is applied. In reliability engineering, even if a part of structures fails, the safety as a whole system must be ensured, therefore, the design having margin is carried out. The degree of margin is called redundancy. However, such design concept makes the structure of a system complex, and as the structure is complex, the possibility of causing human errors becomes high. At the time of huge system design, the concept of fail-safe is effective, but simple design must be kept in mind. The accident in Mihama No. 2 plant of Kansai Electric Power Co. and the accident in Chernobyl nuclear power station, and the accident of Boeing B737 airliner and the fatigue breakdown are described. The importance of safety culture was emphasized as the method of preventing human errors. Man-system interface and management system are discussed. (K.I.)

Cystic fibrosis: case report

International Nuclear Information System (INIS)

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

2002-01-01

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

Cystic fibrosis: case report

International Nuclear Information System (INIS)

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

2002-01-01

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

[Treatment of lymphangiomas with picibanil in the first year of life].

Science.gov (United States)

Subotic, U; Hosie, S; Waag, K L; Reinshagen, K

2008-01-01

The therapeutic gold standard of cystic hygroma is its complete resection. Because of its growth pattern and its main location in the head and neck region complete resection is not always possible. An alternative is the local injection of Picibanil, but only few cases have been published about its use in infants. We retrospectively analyzed the data of 8 infants (age: 2 weeks-12 months) who got Picibanil therapy because of cystic hygroma in the time period 2002 until 2006. Follow up ranged from 3 months up to 3 years. During the postoperative period all patients had local swelling, in 4 cases accompanied with local inflammation and fever. Tumor reduction of >50% was obtained in 7 of 8 patients. Local injection of Picibanil in infants with cystic hygroma seems to be a safe alternative to surgical therapy, especially when complete tumor resection means damage of important neighbouring structures. Prospective trials are necessary to confirm the better outcome after therapy with Picibanil compare to primary surgery.

Cystic tumours of the pancreas

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Itai, Y. [Dept. of Radiology, Inst. of Clinical Medicine, Tsukuba Univ. (Japan); Ohtomo, K. [Univ. of Tokyo Hospital, Tokyo (Japan)

1996-12-01

In this pictorial essay we present the typical appearances of cystic pancreatic tumours, the wide spectrum of their features, and differential features among cystic pancreatic masses with an emphasis on CT. Pseudocysts are the most common cystic lesion in the pancreas and can be induced by pancreatitis, trauma or surgery. Pseudocysts appear as a round cystic mass with a definite wall. However, they can mimic cystic tumours associated with internal septation and/or necrotic mass of various shapes. Conversely, cystic tumours can appear as a simple cyst lacking any thickening of wall, septation or mural nodule. Pancreatic carcinoma not infrequently induces secondary cysts upstream of the obstructed pancreatic duct. The cysts are pseudocysts or retention cysts in nature. When cysts are formed in the pancreatic parenchyma or adjacent to pancreatic carcinoma they may mimic cystic tumour. (orig./VHE)

Cystic tumours of the pancreas

International Nuclear Information System (INIS)

Itai, Y.; Ohtomo, K.

1996-01-01

In this pictorial essay we present the typical appearances of cystic pancreatic tumours, the wide spectrum of their features, and differential features among cystic pancreatic masses with an emphasis on CT. Pseudocysts are the most common cystic lesion in the pancreas and can be induced by pancreatitis, trauma or surgery. Pseudocysts appear as a round cystic mass with a definite wall. However, they can mimic cystic tumours associated with internal septation and/or necrotic mass of various shapes. Conversely, cystic tumours can appear as a simple cyst lacking any thickening of wall, septation or mural nodule. Pancreatic carcinoma not infrequently induces secondary cysts upstream of the obstructed pancreatic duct. The cysts are pseudocysts or retention cysts in nature. When cysts are formed in the pancreatic parenchyma or adjacent to pancreatic carcinoma they may mimic cystic tumour. (orig./VHE)

Picibanil (OK-432) in the treatment of head and neck lymphangiomas in children.

Science.gov (United States)

Rebuffini, Elena; Zuccarino, Luca; Grecchi, Emma; Carinci, Francesco; Merulla, Vittorio Emanuele

2012-12-01

Picibanil (OK-432) is a lyophilized mixture of group A Streptococcus pyogenes with antineoplastic activity. Because of its capacity to produce a selective fibrosis of lymphangiomas (LMs), it has been approved by Japanese administration in 1995 for the treatment of LMs. We treated 15 children (age range: 6-60 months) affected by head and neck macrocystic LMs with intracystic injections (single dose of 0.2 mL) of Picibanil (1-3 injections). Complete disappearance of the lesion was noticed in eight (53.33%) cases, a marked (>50%) reduction of LMs was found five (33.33%) cases, while a moderate (Picibanil side effects included fever, local inflammation, and transitory increase of blood platelets' concentration; a single case of anemia was resolved with concentrated red blood cells transfusion. Intracystic injection of Picibanil is an effective and safe treatment for macrocystic LMs in pediatric patients and may represent the treatment of choice in such cases, especially where surgical excision is associated with the risk of functional/cosmetic side effects.

Connecting slow earthquakes to huge earthquakes.

Science.gov (United States)

Obara, Kazushige; Kato, Aitaro

2016-07-15

Slow earthquakes are characterized by a wide spectrum of fault slip behaviors and seismic radiation patterns that differ from those of traditional earthquakes. However, slow earthquakes and huge megathrust earthquakes can have common slip mechanisms and are located in neighboring regions of the seismogenic zone. The frequent occurrence of slow earthquakes may help to reveal the physics underlying megathrust events as useful analogs. Slow earthquakes may function as stress meters because of their high sensitivity to stress changes in the seismogenic zone. Episodic stress transfer to megathrust source faults leads to an increased probability of triggering huge earthquakes if the adjacent locked region is critically loaded. Careful and precise monitoring of slow earthquakes may provide new information on the likelihood of impending huge earthquakes. Copyright © 2016, American Association for the Advancement of Science.

Management of cystic lymphangioma: experience of two referral ...

African Journals Online (AJOL)

Journal Home > Vol 8, No 4 (2012) > ... Conventional surgical excision remains the most popular method of treatment in many ... sex, site of the pathology, clinical presentation, investigations, management modality, and outcome of treatment.

[Cysts in the posterior triangle of the neck in adults].

Science.gov (United States)

Brea-Álvarez, Beatriz; Roldán-Hidalgo, Amaya

2015-01-01

Cystic lesions of the posterior triangle are a pathologic entity whose diagnosis is made in the first two years of life. Its presentation in adulthood is an incidental finding and the differential diagnosis includes cystic lymphangioma, lymphatic metastasis of thyroid cancer and branchial cyst. Often with the finding of a cervical lump, FNA is made before diagnostic imaging is performed, however, this procedure is not always advisable. We reviewed the cases of patients who came last year to our department with a cystic mass in this location and correlating the imaging findings with pathologic specimen. We show characteristic findings of these lesions in order to make an early diagnosis and thus to get the approach and treatment appropriate of adult patients with a cystic lesion in the posterior cervical triangle. Copyright © 2013 Elsevier España, S.L.U. y Sociedad Española de Otorrinolaringología y Patología Cérvico-Facial. All rights reserved.

MRI findings of intracranial cystic meningiomas

International Nuclear Information System (INIS)

Zhang, D.; Hu, L.-B.; Zhen, J.W.; Zou, L.-G.; Feng, X.-Y.; Wang, W.-X.; Wen, L.

2009-01-01

Aim: To report the magnetic resonance imaging (MRI) features of intracranial cystic meningiomas and compare these features in intra- and peritumoural cyst groups. Materials and methods: Fourteen cases of peritumoural cystic meningiomas were compared with 18 cases of intratumoural cystic meningiomas. All patients were examined using non-enhanced and contrast-enhanced MRI. Tumour location, tumour size, signal intensity, enhancement characteristics, and cystic changes were assessed. The MRI features were compared between the intra- and peritumoural cyst groups. Results: Most cystic meningiomas comprised two or more cysts. The solid parts of the tumours showed moderate or marked enhancement after the injection of contrast material. An enhanced cyst wall was found in six out of 14 cases in the peritumoural cyst group, but not in the intratumoural cyst group. Peritumoural cystic meningiomas were predominately located in the cerebral falx, whereas the intratumoural cystic meningiomas were predominantly found in frontal convexity (X 2 = 7.434, p = 0.024). The cysts were larger in the peritumoural cyst group than in the intratumoural cyst group (t = 5.274, p = 0.0258). Peritumoural oedema was more commonly found in the intratumoural cyst group (X 2 = 6.863, p = 0.008). Cystic meningiomas with solid parts located inside the cyst are reported for the first time. Conclusion: Cystic meningiomas, although uncommon, should be differentiated from other cystic intracranial lesions. Peri- and intratumoural cystic meningiomas have distinct MRI features. The present study provides the first report of two lesions with solid parts located inside the cyst, as well as one lesion with a calcified solid nodule and haemorrhage within the cyst.

Cystic tumors of the pancreas

International Nuclear Information System (INIS)

Brambs, H.J.; Juchems, M.

2008-01-01

Cystic lesions of the pancreas encompass a broad spectrum of benign, premalignant, and malignant tumors which are primarily cystic or result from cystic necroses of solid neoplasms. Because of the wide use of cross-sectional imaging techniques they are increasingly being identified in asymptomatic patients as well as in patients presenting with abdominal pain, jaundice or pancreatitis. Among these lesions, intraductal papillary mucinous neoplasms, serous cystic neoplasms and mucinous cystic neoplasms represent the majority of cases. With increasing experience with these tumors, a refinement of our understanding of their morphology and of their natural course has emerged. It is important to be familiar with the CT and MR imaging features of these lesions to differentiate these tumors and to orient the diagnosis towards benign or malignant forms. Because characterization of cystic tumors of the pancreas can sometimes be difficult due to overlapping imaging features, additional criteria such as clinical symptoms, localization, age and gender have to be taken into account. If appropriately treated, these tumors can usually be cured by resection and the decreasing risk of pancreatic surgery has led to an increasing number of resections of pancreatic tumors. The management of cystic tumors of the pancreas has not yet been standardized and the correct evaluation and subsequent management of the disease in asymptomatic patients have not been fully defined. (orig.) [de

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...

Sonographic-pathologic correlation of complex cystic breast lesions

Directory of Open Access Journals (Sweden)

Saravech Pongrattanaman

2013-02-01

Full Text Available Objective: To understand the pathologic basis for sonographic features of complex cystic lesions. Methods: From 2 646 female patients underwent breast sonography at King Chulalongkorn Memorial Hospital from January 2005 through December 2010, 103 cystic lesions were included. Pathologic confirmation was performed by fine-needle aspiration (n=42, core needle biopsy (n=6, excision (n=54 and mastectomy (n=1. Complex cystic breast masses were classified into 3 types as followings; thick outer wall and/or thick internal septa (type I; thick septation and thick wall were defined as equal or more than 0.5 cm, masses containing mixed cystic and solid components (at least 50% of cystic component (type II, predominantly solid with eccentric cystic foci (at least 50% of solid component (type III. Results: In 103 complex cystic masses, there are 27 lesions (26% classified as type I cystic breast masses, 37 lesions (36% as type II cystic breast masses and 39 lesions (38% type III cystic breast masses, 26 lesions (25.2% are proved to be malignant. All of type I cystic breast masses in our study are benign, and 14 (38% of type II cystic breast masses and 12 lesions (31% of type III cystic breast lesions are proved to be malignant. Conclusions: Type II and III lesions should suggest possibility of malignancy and biopsy should be performed in all lesions. All type I lesion in this study are benign. None of other parameters we included in this study (size or margin can effectively differentiate between benign or malignant cystic breast lesions. Also, grading of the malignant lesions by using type of cystic breast mass cannot be applied.

Cystic thymic diseases: CT manifestations

International Nuclear Information System (INIS)

Song, Soon Young; Choi, Yo Won; Jeon, Eui Yong; Jeon, Seok Chol; Seo, Heung Suk; Hahm, Chang Kok

1995-01-01

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

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

International Nuclear Information System (INIS)

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

2000-01-01

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

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.

Huge maternal hydronephrosis: a rare complication in pregnancy.

Science.gov (United States)

Peng, Hsiu-Huei; Wang, Chin-Jung; Yen, Chih-Feng; Chou, Chien-Chung; Lee, Chyi-Long

2003-06-10

A huge maternal hydronephrosis is uncommon in pregnancy and might be mistaken as a pelvic mass. A 21-year-old primigravida was noted at 25th week of gestation to have a visible bulging mass on her left flank. The mass was originally mistaken as a large ovarian cyst but later proved to be a huge hydronephrosis. Retrograde insertion of ureteroscope and a ureteric stent failed, so we performed repeated ultrasound-guided needle aspiration to decompress the huge hydronephrosis, which enabled the patient to proceed to a successful term vaginal delivery. Nephrectomy was performed after delivery and proved the diagnosis of congenital ureteropelvic junction obstruction.

Neonatal cystic fibrosis screening test

Science.gov (United States)

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

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.

Breakdown in Breathing: The Complexities of Cystic Fibrosis

Science.gov (United States)

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

CT diagnosis of cystic echinococcosis in pelvis

International Nuclear Information System (INIS)

Liu Wenya; Li Li; Xing Yan; Xie Jingxia

2003-01-01

Objective: To analyze the CT findings of pelvic cystic echinococcosis, and to assess the value of CT examination. Methods: Twenty-one cases of pelvic cystic echinococcosis, confirmed by clinical and pathological results, underwent CT scanning. CT characters were analyzed and compared with pathology. Results: Simple cystic echinococcosis appeared as single or multiple cystic lesions with round or oval shape, smooth boundary, and no enhancement after contrast medium administration in 3 cases; Different number and size of daughter cysts were detected inside the lesion in 17 cases; Ruptured lesions showed 'double wall', 'water snake', or 'flow ribbon' signs in 4 cases; Increase density of contents and enhanced cystic wall were demonstrated in 2 cases with companied infections. In 8 cases, calcification occurred on the cystic wall or extended inside the content. Conclusion: CT could accurately demonstrate the location, appearance, internal structure, and adjacent situation of the cystic echinococcosis, providing valuable information for correct diagnosis and treatment

Diagnosis of cystic fibrosis

NARCIS (Netherlands)

H.J. Veeze

1995-01-01

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

Vitamin A supplementation for cystic fibrosis.

Science.gov (United States)

Bonifant, Catherine M; Shevill, Elizabeth; Chang, Anne B

2014-05-14

People with cystic fibrosis and pancreatic insufficiency are at risk of fat soluble vitamin deficiency as these vitamins (A, D, E and K) are co-absorbed with fat. Thus, some cystic fibrosis centres routinely administer these vitamins as supplements but the centres vary in their approach of addressing the possible development of deficiencies in these vitamins. Vitamin A deficiency causes predominantly eye and skin problems while supplementation of vitamin A to excessive levels may cause harm to the respiratory and skeletal systems in children. Thus a systematic review on vitamin A supplementation in people with cystic fibrosis would help guide clinical practice. To determine if vitamin A supplementation in children and adults with cystic fibrosis:1. reduces the frequency of vitamin A deficiency disorders;2. improves general and respiratory health;3. increases the frequency of vitamin A toxicity. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register which comprises of references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings.Date of the most recent search of the Group's Cystic Fibrosis Trials Register: 07 April 2014. All randomised or quasi-randomised controlled trials comparing all preparations of oral vitamin A used as a supplement compared to either no supplementation (or placebo) at any dose and for any duration, in children or adults with cystic fibrosis (defined by sweat tests or genetic testing) with and without pancreatic insufficiency. No relevant studies for inclusion were identified in the search. No studies were included in this review. As there were no randomised or quasi-randomised controlled trials identified, we cannot draw any conclusions on the benefits (or otherwise) of regular administration of vitamin A in people with cystic fibrosis. Until further data are available, country or region specific guidelines on the use of

Voice Disorder in Cystic Fibrosis Patients

Science.gov (United States)

Lourenço, Bruna Mendes; Costa, Kauê Machado; da Silva Filho, Manoel

2014-01-01

Cystic fibrosis is a common autosomal recessive disorder with drastic respiratory symptoms, including shortness of breath and chronic cough. While most of cystic fibrosis treatment is dedicated to mitigating the effects of respiratory dysfunction, the potential effects of this disease on vocal parameters have not been systematically studied. We hypothesized that cystic fibrosis patients, given their characteristic respiratory disorders, would also present dysphonic symptoms. Given that voice disorders can severely impair quality of life, the identification of a potential cystic fibrosis-related dysphonia could be of great value for the clinical evaluation and treatment of this disease. We tested our hypothesis by measuring vocal parameters, using both objective physical measures and the GRBAS subjective evaluation method, in male and female cystic fibrosis patients undergoing conventional treatment and compared them to age and sex matched controls. We found that cystic fibrosis patients had a significantly lower vocal intensity and harmonic to noise ratio, as well as increased levels of jitter and shimmer. In addition, cystic fibrosis patients also showed higher scores of roughness, breathiness and asthenia, as well as a significantly altered general grade of dysphonia. When we segregated the results according to sex, we observed that, as a group, only female cystic fibrosis patients had significantly lower values of harmonic to noise ratio and an abnormal general grade of dysphonia in relation to matched controls, suggesting that cystic fibrosis exerts a more pronounced effect on vocal parameters of women in relation to men. Overall, the dysphonic characteristics of CF patients can be explained by dysfunctions in vocal fold movement and partial upper airway obstruction, potentially caused by the accumulation of mucus and chronic cough characteristic of CF symptomatology. Our results show that CF patients exhibit significant dysphonia and suggest they may

Renal cystic disease: A practical overview

International Nuclear Information System (INIS)

Hartman, D.S.

1987-01-01

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

CT findings in skeletal cystic echinococcosis

Energy Technology Data Exchange (ETDEWEB)

Tuezuen, M.; Hekimoglu, B. [Social Security Hospital, Ankara (Turkey). Dept. of Radiology

2002-09-01

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.

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.

Intra-cystic concentrations of albendazole-sulphoxide in human cystic echinococcosis: a systematic review and analysis of individual patient data.

Science.gov (United States)

Lötsch, Felix; Naderer, Judith; Skuhala, Tomislava; Groger, Mirjam; Auer, Herbert; Kaczirek, Klaus; Waneck, Fredrik; Ramharter, Michael

2016-08-01

Cystic echinococcosis (CE) is a widespread zoonosis caused by the species complex Echinococcus granulosus. Albendazole (ABZ)-the first-line anthelminthic drug for medical treatment of CE-is metabolized in vivo to the active derivative ABZ-sulphoxide (ABZ-SO). Target-site ABZ-SO concentrations in the hydatid cyst mediate the anthelminthic effect in CE. Primary outcome of this systematic review of individual patient data was the intra-cystic ABZ-SO concentration stratified by cyst size, location, calcification status and use of praziquantel. Studies reporting intra-cystic ABZ-SO concentrations in humans were identified by a systematic search. A pooled analysis of individual patient data was performed to assess intra-cystic concentrations. Pharmacokinetic data of 121 individual cysts were analysed. There was no correlation between plasma and intra-cystic ABZ-SO concentrations (rho = -0.03, p = 0.76). Intra-cystic drug concentrations were also not associated with sex and treatment duration. Use of praziquantel in combination with ABZ was associated with higher plasma (median 540 vs. 240 μg/L; p = 0.04) but not intra-cystic ABZ-SO concentrations (median 220 vs. 199 μg/L; p = 0.36). Relative drug concentrations in hepatic cysts were higher than in other cysts (0.8 vs. 0.4; p = 0.05). Intra-cystic concentrations were higher in calcified than non-calcified cysts (median 897 vs. 245 μg/L; p = 0.03). There was a trend towards higher intra-cystic concentrations in smaller sized cysts (β = -17.2 μg/L/cm; 95th CI, -35.9 to 1.6; p = 0.07). This study demonstrates that mean intra-cystic drug concentrations are similar to plasma concentrations on a population level. However, in individual patients plasma concentrations are not directly predictive for intra-cystic concentrations. The use of booster drugs was not associated with higher intra-cystic ABZ-SO concentrations in this analysis.

Efficacy of electrocoagulation in sealing the cystic artery and cystic duct occluded with only one absorbable clip during laparoscopic cholecystectomy.

Science.gov (United States)

Yang, Chang-Ping; Cao, Jin-Lin; Yang, Ren-Rong; Guo, Hong-Rong; Li, Zhao-Hui; Guo, Hai-Ying; Shao, Yin-Can; Liu, Gui-Bao

2014-02-01

Even though laparoscopic cholecystectomy (LC) emerged over 20 years ago, controversies persist with regard to the best method to ligate the cystic duct and artery. We proposed to assess the effectiveness and safety of electrocoagulation to seal the cystic artery and cystic duct after their occlusion with only one absorbable clip. We retrospectively compared the clinical data for 635 patients undergoing LC using electrocoagulation to seal the cystic artery and cystic duct that were occluded with only one absorbable clip (Group 1) and 728 patients undergoing LC using titanium clips (Group 2). In parallel, 30 rabbits randomized into six groups underwent cholecystectomy. After cystic duct ligation with absorbable or titanium clips, the animals were sacrificed 1, 3, or 6 months later, and intraabdominal adhesions were assessed after celiotomy. The mean operative time was significantly shorter (41.6 versus 58.9 minutes, PElectrocoagulation of the cystic artery and cystic duct that were occluded with only one absorbable clip is safe and effective during LC. This approach is associated with shortened operative times and reduced leakage, compared with the standard method using metal clips.

Gastrointestinal Manifestations of Cystic Fibrosis

Science.gov (United States)

2016-01-01

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

Induction of ovarian cystic follicles in sheep.

Science.gov (United States)

Christman, S A; Bailey, M T; Head, W A; Wheaton, J E

2000-10-01

Cystic follicles are a significant cause of infertility in women, dairy cattle and sheep. Sheep were used as a model to identify factors that may elicit formation of cystic follicles. Insulin resistance and elevated LH activity were tested in overweight ewes because of associations among these factors and the formation of cystic follicles. Sheep were synchronized using a progesterone-releasing pessary and insulin resistance was induced during the synchronization period through administration of bovine somatotropin. Following removal of pessaries follicular growth was stimulated by treatment with eCG or eCG and hCG (PG-600). Follicular growth was monitored via daily transrectal ultrasonography and blood samples were collected for hormonal analyses. Six of 18 ewes had a subnormal or absent preovulatory gonadotropin surge and developed cystic follicles. Neither insulin resistance nor elevated LH activity were associated with formation of cystic follicles. Ewes that developed cystic follicles were heavier (93 +/- 4 kg) than ewes that ovulated (81 +/- 3 kg; P = 0.02). Furthermore, following pessary removal and initiation of daily ultrasonography, ewes that developed cystic follicles lost body weight (-3 +/- 1%), while ovulatory ewes continued to gain body weight (1 +/- 1%; P = 0.005). It is speculated that in heavy ewes metabolic factors associated with acute body weight loss inhibit the positive feedback of estradiol and thereby suppress the preovulatory gonadotropin surge leading to formation of cystic follicles.

Imaging diagnosis of meningiomas of ethmoid sinuses

International Nuclear Information System (INIS)

Lu Bingfeng; Liang Shuming; Li Mao

2001-01-01

Objective: To study the imaging features of meningiomas of ethmoid sinuses. Methods: Six cases of meningiomas of ethmoid sinuses verified pathologically were analyzed retrospectively. Results: CT scans of 6 cases exhibited huge cystic masses (n = 3), huge cystic-solid masses (n = 2), huge solid mass (n = 1). The cystic walls were remarkable osteosclerosis. The density of solid masses were homogeneous, or heterogeneous with calcifications and cystic changes, and prominent contrast-enhancement. MR images of 1 case (1/6) showed a cystic-solid mass, the cystic portion was high signal intensity on T 2 WI and low signal intensity on T 1 WI, while the solid mass was iso-signal intensity on T 1 WI and T 2 WI. The solid portion was enhanced. X-ray plain films of 3 cases (3/6) displayed ethmoid sinuses enlargement and high density. Conclusion: For the meningiomas of ethmoid sinuses, CT finding was specific, MRI was helpful in differential diagnosis, and X-ray plain films was of no qualitative value

Cystic fibroepithelioma of Pinkus: two new cases and cystic changes in classical fibroepithelioma of Pinkus

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Zlatko Marusic

2014-09-01

Full Text Available We report two new cases of cystic fibroepithelioma of Pinkus together with immunohistochemical features and analyze the presence of cystic changes in a series of 16 classical fibroepitheliomas of Pinkus. Our findings show that the formation of cystic spaces is most probably caused by ischemic degeneration of stromal fenestrations, rather than by central tumor cell necrosis. This finding is supported by lack of CD34 positive blood vessels in edematous and hyalinized stromal fenestrations undergoing transformation into cystic spaces, as opposed to the uninvolved stromal fenestrations. Therefore, it is probably more accurate to refer to this process as pseudocystic stromal degeneration rather than true cyst formation. Also, two out of 16 classical Pinkus fibroepitheliomas exhibited focal pseudocystic changes in 50% and 10% of the tumor, respectively, demonstrating that this degenerative process can be found, rarely and focally, in classical cases as well. 

Otorhinolaryngologic manifestations of cystic fibrosis: literature review

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Carvalho, Carolina Pimenta

2008-12-01

Full Text Available Introduction: Cystic Fibrosis is the most common recessive autosomic genetic disease among Caucasians. It's caused by mutations in the gene that decodes regulatory protein for transmembrane conductance, resulting in defective transport of chlorine. Objective: Review the literature about Cystic Fibrosis, with emphasis on otorhinolaryngologic manifestations. Method: The online Pub Med databases were researched and we applied the following search terms Fibrosis Cystic and Sinusitis, and Mucoviscidosis and Sinusitis. Conclusions: Although it is not the main cause of death, the otorhinolaryngologic manifestations of the Cystic Fibrosis bring important morbidity to these patients.

Huge Thornwaldt's Cyst: A Case Report

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Jia-Hau Lin

2006-10-01

Full Text Available Thornwaldt's bursa, also known as nasopharyngeal bursa, is a recess in the midline of the nasopharynx that is produced by persistent notochord remnants. If its opening becomes obstructed, possibly due to infection or a complication from adenoidectomy, a Thornwaldt's cyst might develop. Here, we present a 53-year-old man who complained of nasal obstruction that had progressed for 1 year. Nasopharyngoscopy showed a huge nasopharyngeal mass. Thornwaldt's cyst was suspected. Magnetic resonance imaging showed a lesion measuring 3.6 × 3.4 cm, intermediate on T1-weighted and high signal intensity on T2-weighted imaging, neither bony destruction nor connection to the brain. The patient underwent endoscopic surgery for this huge mass. Afterwards, his symptoms improved significantly. We present the treatment and differential diagnosis of a nasopharyngeal cyst.

Mature Cystic Renal Teratoma

International Nuclear Information System (INIS)

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

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 central cystic component. Computed tomography (CT) demonstrated a lobulated, heterogeneous, hypodense mass extending craniocaudally from the splenic hilum to the level of the left iliac fossa. Nephrectomy was performed and a large, fatty mass arising from the left kidney was excised. The final pathologic diagnosis was confirmed as cystic renal teratoma

Cystic astrocytomas in children. The contribution of MRI

International Nuclear Information System (INIS)

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

1988-01-01

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

Huge magnetoresistance effect of highly oriented pyrolytic graphite

International Nuclear Information System (INIS)

Du Youwei; Wang Zhiming; Ni Gang; Xing Dingyu; Xu Qingyu

2004-01-01

Graphite is a quasi-two-dimensional semimetal. However, for usual graphite the magnetoresistance is not so high due to its small crystal size and no preferred orientation. Huge positive magnetoresistance up to 85300% at 4.2 K and 4950% at 300 K under 8.15 T magnetic field was found in highly oriented pyrolytic graphite. The mechanism of huge positive magnetoresistance is not only due to ordinary magnetoresistance but also due to magnetic-field-driven semimetal-insulator transition

Modern diagnostics of cystic liver lesions and hemangiomas; Moderne Diagnostik zystischer Leberlaesionen und Haemangiome

Energy Technology Data Exchange (ETDEWEB)

Poetter-Lang, S.; Bastati-Huber, N.; Ba-Ssalamah, A. [Medical University of Vienna, Department of Biomedical Imaging and Image-guided Therapy, Wien (Austria); Brancatelli, G. [Universitaetsklinikum Palermo, Abteilung Radiologie, Palermo (Italy)

2015-01-01

Cystic liver lesions incorporate a broad heterogeneous group of mostly benign but also malignant abnormalities. The radiological aim is the non-invasive diagnosis with the use of different imaging modalities to determine the type of lesion. The common generally asymptomatic incidental findings of cystic lesions on ultrasound, computed tomography (CT) and magnetic resonance imaging (MRI) must be classified on the basis of specific imaging features. Such a differentiation is essential because the clinical consequences and the appropriate therapy can vary depending on the underlying pathology. Due to the morphological overlap of many cystic lesions, conventional radiological methods are often insufficient. The huge advances in cross-sectional imaging (multidetector CT, MRI with special sequences and different contrast agents and MR cholangiopancreatography) in combination with the clinical history usually enable a non-invasive diagnosis. Pathognomonic morphological and hemodynamic lesion features, as well as a knowledge of the pathomechanisms, help to differentiate this broad spectrum of entities. In this article the different entities of cystic liver lesions, together with the appropriate diagnostic method for detection and distinction and including their strengths and limitations, are demonstrated. A well-founded knowledge about the development of various cystic liver lesions and the suitable choice of imaging method facilitate a non-invasive diagnosis. (orig.) [German] Die zystischen Leberlaesionen umfassen eine grosse heterogene Gruppe meist benigner, jedoch auch maligner Veraenderungen. Das radiologische Ziel ist die nichtinvasive Artdiagnose mithilfe verschiedener bildgebender Verfahren. Die haeufigen, meist asymptomatischen Zufallsbefunde in Ultraschall, CT und MRT muessen anhand spezieller bildgebender Kriterien klassifiziert werden. Eine Differenzierung ist hierbei essenziell, da die klinischen Konsequenzen und weiterfuehrenden Therapien je nach zugrunde

Diffuse cystic peritoneal lymphangiomatosis. Long-term clinical, ultrasonographic and CT scan study in one case

International Nuclear Information System (INIS)

Berry, M.; Paillot, B.; Monneins, F.; Delafolie, A.; Guyet-Rousset, P.

1987-01-01

An adult male with episodic manifestations of abdominal lymphangiomatosis has been followed up over a 5 year period. This prolonged surveillance of clinical features combined with ultrasound and CT scan imaging has no doubt contributed to improved knowledge of diagnostic and physiopathologic factors of these rare lesions. The particular behavior of the case reported evokes discussion on the conventional surgical treatment of lymphangioma [fr

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

NARCIS (Netherlands)

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

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

Computed tomography of cystic pancreatic fibrosis

International Nuclear Information System (INIS)

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

1984-01-01

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

CT diagnosis of cystic ovarian lesions

International Nuclear Information System (INIS)

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

1985-01-01

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

Pregnancy and cystic fibrosis: Approach to contemporary management

Science.gov (United States)

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

2014-01-01

Over the previous 50 years survival of patients with cystic fibrosis has progressively increased. As a result of improvements in health care, increasing numbers of patients with cystic fibrosis are now considering starting families of their own. For the health care professionals who look after these patients, the assessment of the potential risks, and the process of guiding prospective parents through pregnancy and beyond can be both challenging and rewarding. To facilitate appropriate discussions about pregnancy, health care workers must have a detailed understanding of the various important issues that will ultimately need to be considered for any patient with cystic fibrosis considering parenthood. This review will address these issues. In particular, it will outline pregnancy outcomes for mothers with cystic fibrosis, issues that need to be taken into account when planning a pregnancy and the management of pregnancy for mothers with cystic fibrosis or mothers who have undergone organ transplantation as a result of cystic fibrosis. PMID:27512443

Cystic Duct Closure by Sealing With Bipolar Electrocoagulation

Science.gov (United States)

Damgaard, B.; Jorgensen, L. N.; Larsen, S. S.; Kristiansen, V. B.

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 in 2 hospitals with closure of the cystic duct with LigaSure after informed consent were recorded and complications and morbidity registered. The records were compared with those of patients undergoing laparoscopic cholecystectomy with closure of the cystic duct with clips during the same period. Results: During the study period, 218 laparoscopic cholecystectomies were performed; 102 of these were performed with the LigaSure. One patient was excluded due to violation of the protocol. We experienced no cases of cystic duct leakage, but in one patient, bile leakage from the gallbladder bed was observed probably due to a small aberrant duct. Conclusion: The LigaSure system was safe and effective for closure and division of the cystic duct in laparoscopic cholecystectomy. PMID:20412641

Giant cystic abdominal masses in children

International Nuclear Information System (INIS)

Wootton-Gorges, Sandra L.; Thomas, Kristen B.; Harned, Roger K.; Wu, Sarah R.; Stein-Wexler, Rebecca; Strain, John D.

2005-01-01

In this pictorial essay the common and uncommon causes of large cystic and cyst-like abdominal masses in children are reviewed. We discuss and illustrate the following: mesenchymal hamartoma, choledochal cyst, hydrops of the gallbladder, congenital splenic cyst, pancreatic pseudocyst, pancreatic cystadenoma, hydronephrosis, multicystic dysplastic kidney, multilocular cystic nephroma, adrenal hemorrhage, mesenteric and omental cysts, gastrointestinal duplication cyst, meconium pseudocyst, ovarian cysts and cystic neoplasms, hematocolpos, urachal cysts, appendiceal abscess, abdominal and sacrococcygeal teratoma, and CSF pseudocyst. We also describe imaging features and clues to the diagnosis. (orig.)

Giant cystic abdominal masses in children

Energy Technology Data Exchange (ETDEWEB)

Wootton-Gorges, Sandra L.; Thomas, Kristen B.; Harned, Roger K.; Wu, Sarah R.; Stein-Wexler, Rebecca; Strain, John D. [University of California, Davis Health Center, Sacramento, CA (United States); Davis Children' s Hospital, Department of Radiology, Sacramento, CA (United States)

2005-12-01

In this pictorial essay the common and uncommon causes of large cystic and cyst-like abdominal masses in children are reviewed. We discuss and illustrate the following: mesenchymal hamartoma, choledochal cyst, hydrops of the gallbladder, congenital splenic cyst, pancreatic pseudocyst, pancreatic cystadenoma, hydronephrosis, multicystic dysplastic kidney, multilocular cystic nephroma, adrenal hemorrhage, mesenteric and omental cysts, gastrointestinal duplication cyst, meconium pseudocyst, ovarian cysts and cystic neoplasms, hematocolpos, urachal cysts, appendiceal abscess, abdominal and sacrococcygeal teratoma, and CSF pseudocyst. We also describe imaging features and clues to the diagnosis. (orig.)

Human cystic echinococcosis in South Africa

NARCIS (Netherlands)

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

2012-01-01

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

Cystic Lesions in Autoimmune Pancreatitis

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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.

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.

Combined lymphangioma and hemangioma of the spleen in a patient with Klippel–Trénaunay syndrome

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Spasić Marko

2012-01-01

Full Text Available Introduction. Klippel–Trénaunay syndrome (KTS is a very rare congenital anomaly of blood vessels, characterized by the following clinical triad: varicose superficial veins, port-wine stain and usually bony and soft tissue hypertophy of extremities, most often located in the lower extremities. It is often accompanied by visceral manifestations, and rarely combined with splenomegaly. Case Outline. A 30-year-old female patient came to the Surgery Clinic because of occasional left hypochondrial pain. After she was diagnosed with KTS combined with splenomegaly, splenectomy was performed. Macroscopic and microscopic spleen examination indicated the presence of tumor of vascular origin, presenting a combination of lymphangioma and hemangioma. Conclusion. Diagnosed KTS demands a thorough clinical examination of the patient because of the potential presence of visceral manifestations. When splenomegaly is present, even though being often benign, splenectomy is usually performed to alleviate accompanying symptoms which occur as a result of organ enlargement and compression, to prevent rupture and consequential bleeding when the vascular spleen tumor is large, and finally to avoid a possibility of malignant transformation.

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.

Huge interparietal posterior fontanel meningohydroencephalocele

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Jorge Félix Companioni Rosildo

2015-03-01

Full Text Available Congenital encephalocele is a neural tube defect characterized by a sac-like protrusion of the brain, meninges, and other intracranial structures through the skull, which is caused by an embryonic development abnormality. The most common location is at the occipital bone, and its incidence varies according to different world regions. We report a case of an 1-month and 7-day-old male child with a huge interparietal-posterior fontanel meningohydroencephalocele, a rare occurrence. Physical examination and volumetric computed tomography were diagnostic. The encephalocele was surgically resected. Intradural and extradural approaches were performed; the bone defect was not primarily closed. Two days after surgery, the patient developed hydrocephaly requiring ventriculoperitoneal shunting. The surgical treatment of the meningohydroencephalocele of the interparietal-posterior fontanel may be accompanied by technical challenges and followed by complications due to the presence of large blood vessels under the overlying skin. In these cases, huge sacs herniate through large bone defects including meninges, brain, and blood vessels. The latter present communication with the superior sagittal sinus and ventricular system. A favorable surgical outcome generally follows an accurate strategy taking into account individual features of the lesion.

Congenital Cystic Adenomatoid Malformation of Lung-Rare Case Report

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N. S. Kamakeri

2016-10-01

Full Text Available Congenital cystic adenomatoid malformation of lung associated with Cystic dysplasia of kidney, cystic disease of liver with mixed gonadal dysgenesis is rare and is not reported in literature so far. Hence an attempt is made to present this rarest entity.

Cystic fibrosis-related diabetes: a distinct condition.

Science.gov (United States)

Cano Megías, Marta; González Albarrán, Olga

2015-01-01

Cystic fibrosis is the most common fatal inherited autosomal recessive disease in Caucasians, affecting approximately one out of every 2,000 births. Survival of patients with cystic fibrosis has significantly improved due to advances in respiratory and nutritional care, and their current average life expectancy is 30-40 years. Development of cystic fibrosis-related diabetes is a comorbidity that increases with age and may reach a prevalence up to 50% in adults. Its development is associated to impaired lung function and nutritional status, and early diagnosis and treatment are therefore essential to improve quality of life and performance status. Insulin therapy for diabetes and other early carbohydrate metabolism disorders may improve lung function and nutritional status of patients with cystic fibrosis. Copyright © 2014 SEEN. Published by Elsevier Espana. All rights reserved.

Management of the Upper Airway in Cystic Fibrosis

Science.gov (United States)

Illing, Elisa A.; Woodworth, Bradford A.

2015-01-01

Purpose of Review Upper airway disease engenders significant morbidity for patients with cystic fibrosis and is increasingly recognized as having a much greater role in pulmonary outcomes and quality of life than originally believed. Widespread disparate therapeutic strategies for cystic fibrosis chronic rhinosinusitis underscore the absence of a standardized treatment paradigm. This review outlines the most recent evidence-based trends in the management of upper airway disease in cystic fibrosis. Recent Findings The unified airway theory proposes that the sinuses are a focus of initial bacterial colonization which seeds the lower airway and may play a large role in maintaining lung infections. Mounting evidence suggests more aggressive treatment of the sinuses may confer significant improvement in pulmonary disease and quality of life outcomes in cystic fibrosis patients. However, there is a lack of high-level evidence regarding medical and surgical management of cystic fibrosis chronic rhinosinusitis that makes generalizations difficult. Summary Well designed clinical trials with long-term follow-up concerning medical and surgical interventions for cystic fibrosis sinus disease are required to establish standardized treatment protocols, but increased interest in the sinuses as a bacterial reservoir for pulmonary infections has generated considerable attention. PMID:25250804

Cystic Fibrosis-Related Diabetes (CFRD): Daily Management

Science.gov (United States)

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

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.

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.......001). In conclusion, overall these results do not support a reproductive advantage for cystic fibrosis DeltaF508 heterozygotes. However, the data cannot totally exclude the possibility that nonsmoking DeltaF508 heterozygotes experience a reproductive advantage while smoking DeltaF508 heterozygotes experience...... the opposite, a reproductive disadvantage. Accordingly, the data suggest a previously undocumented role of smoking on fecundity among cystic fibrosis heterozygotes....

[Genetic counseling in cystic fibrosis].

Science.gov (United States)

Julia, S; Bieth, E

2000-08-01

Genetic counseling is an important part of health care in patients with cystic fibrosis or respiratory diseases associated with the CFTR (cystic fibrosis transmembrane conductance regulator) gene, including certain types of allergic bronchopulmonary aspergilloses or bronchial diseases (diffuse bronchiectasia). The basic goal is to provide patients with information on the transmission of cystic fibrosis and to asses the risk of recurrence. This risk is determined from molecular biology analyses examining the CFTR gene. Genotyping is the only means of screening for the heterozygous state, frequent in the French population (about 1/30). Because of the large number of mutated alleles not covered entirely by the genetic tests, there remains a question of probability expressed as a residual risk of a heterozygous state. A prenatal genotype diagnosis should be proposed to heterozygous couples who have a 25% risk of having a diseased child. Technically, this is almost always possible and the results are highly reliable. Nevertheless, there remains the risks related to sample taking and the ethical issue about which the patients must be informed. Management of these at risk couples who desire a child must be based on a multidisciplinary approach, particularly important when one of the parents has overt cystic fibrosis.

CT evaluation of cystic brain disease

International Nuclear Information System (INIS)

Kim, Joon Woo; Lee, Jin Woo; Joo, Yang Goo; Kim, Hong; Zeon, Seok Kil; Suh, Soo Jhi

1987-01-01

We retrospectively analysed CT findings of 47 cystic brain lesions of 44 patients, in which operation, biopsy or follow-up study was needed for their final diagnosis. The results were as follows: 1. The etiologic diseases of cystic brain lesions were 15 cases of brain abscess, 9 cases of astrocytoma, 5 cases of glioblastoma multiforme, 3 cases of meningioma, 5 cases of craniopharyngioma, 1 case of hemangioblastoma, 2 cases of dermoid cyst and 4 cases of metastasis. 2. We analyses the cystic lesions in view of their number, location, shape, perifocal edema, mass effect, wall and its thickness, evenness and characteristics of their inner and outer surfaces, mural nodule, calcification and contrast enhancement. a. 13.3% of brain abscess and 75% of metastases were multiple in number, but the remainder showed single lesion. b. The shape of cystic lesions were round or ovoid in 68%, lobulated in 8.5% and irregular in 23.5%, and no demonstrable difference of shape were noticed in different disease. c. In brain abscess, the wall of cystic lesions tend to be thin, even and smooth in inner surface, but the outer surfaces were equally smooth or irregular. d. Mural nodules were found in nearly half of the cases of astrocytoma, glioblastoma multiforme, metastasis and hemangioblastoma, but the brain abscess and dermoid cyst contained no mural nodule. e. Meningiomas were found to be attached to dura mater and showed thickening of the inner table of adjacent skull or of the falx. f. The presence of preceding infectious disease may be helpful in the diagnosis of brain abscess, but in 20% there were no demonstrable preceding infection. g. Lung cancer was confirmed as primary site in two of the cystic metastatic disease, but other 2 cases showed no demonstrable primary malignancy

CT evaluation of cystic brain disease

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Kim, Joon Woo; Lee, Jin Woo; Joo, Yang Goo; Kim, Hong; Zeon, Seok Kil; Suh, Soo Jhi [Keimyung University, School of Medicine, Daegu (Korea, Republic of)

1987-10-15

We retrospectively analysed CT findings of 47 cystic brain lesions of 44 patients, in which operation, biopsy or follow-up study was needed for their final diagnosis. The results were as follows: 1. The etiologic diseases of cystic brain lesions were 15 cases of brain abscess, 9 cases of astrocytoma, 5 cases of glioblastoma multiforme, 3 cases of meningioma, 5 cases of craniopharyngioma, 1 case of hemangioblastoma, 2 cases of dermoid cyst and 4 cases of metastasis. 2. We analyses the cystic lesions in view of their number, location, shape, perifocal edema, mass effect, wall and its thickness, evenness and characteristics of their inner and outer surfaces, mural nodule, calcification and contrast enhancement. a. 13.3% of brain abscess and 75% of metastases were multiple in number, but the remainder showed single lesion. b. The shape of cystic lesions were round or ovoid in 68%, lobulated in 8.5% and irregular in 23.5%, and no demonstrable difference of shape were noticed in different disease. c. In brain abscess, the wall of cystic lesions tend to be thin, even and smooth in inner surface, but the outer surfaces were equally smooth or irregular. d. Mural nodules were found in nearly half of the cases of astrocytoma, glioblastoma multiforme, metastasis and hemangioblastoma, but the brain abscess and dermoid cyst contained no mural nodule. e. Meningiomas were found to be attached to dura mater and showed thickening of the inner table of adjacent skull or of the falx. f. The presence of preceding infectious disease may be helpful in the diagnosis of brain abscess, but in 20% there were no demonstrable preceding infection. g. Lung cancer was confirmed as primary site in two of the cystic metastatic disease, but other 2 cases showed no demonstrable primary malignancy.

Cystic lesion around the hip joint

Science.gov (United States)

Yukata, Kiminori; Nakai, Sho; Goto, Tomohiro; Ikeda, Yuichi; Shimaoka, Yasunori; Yamanaka, Issei; Sairyo, Koichi; Hamawaki, Jun-ichi

2015-01-01

This article presents a narrative review of cystic lesions around the hip and primarily consists of 5 sections: Radiological examination, prevalence, pathogenesis, symptoms, and treatment. Cystic lesions around the hip are usually asymptomatic but may be observed incidentally on imaging examinations, such as computed tomography and magnetic resonance imaging. Some cysts may enlarge because of various pathological factors, such as trauma, osteoarthritis, rheumatoid arthritis, or total hip arthroplasty (THA), and may become symptomatic because of compression of surrounding structures, including the femoral, obturator, or sciatic nerves, external iliac or common femoral artery, femoral or external iliac vein, sigmoid colon, cecum, small bowel, ureters, and bladder. Treatment for symptomatic cystic lesions around the hip joint includes rest, nonsteroidal anti-inflammatory drug administration, needle aspiration, and surgical excision. Furthermore, when these cysts are associated with osteoarthritis, rheumatoid arthritis, and THA, primary or revision THA surgery will be necessary concurrent with cyst excision. Knowledge of the characteristic clinical appearance of cystic masses around the hip will be useful for determining specific diagnoses and treatments. PMID:26495246

Laryngeal adenoid cystic carcinoma: case report

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André Del Negro

Full Text Available CONTEXT: Adenoid cystic carcinomas are malignant tumors that occur in both the major and the minor salivary glands. A laryngeal location is rare because of the paucity of accessory salivary glands in this area. Adenoid cystic carcinomas account for less than 1% of all malignant tumors in the larynx, and only about 120 cases have been reported in the literature. These tumors have a slight female predisposition, and their peak incidence is in the fifth and sixth decades of life. In this article, we describe a case of laryngeal adenoid cystic carcinoma and discuss its clinical characteristics and treatment. CASE REPORT: We report on a case of laryngeal adenoid cystic carcinoma in a 55 year-old female patient who presented with dyspnea and hoarseness. Features of the diagnostic and therapeutic evaluation are described and the clinical management of such cases is outlined. The clinical course, definitive treatment strategy and surgical procedure, and also adjuvant treatment with irradiation are discussed. Although the tumor is radiosensitive, it is not radiocurable.

Ovarian Mature Cystic Teratoma Containing Multiple Mobile

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Cho, Hyun Sun; Yoon, Seong Eon; Lee, Young Hwan; Kim, Hye Won; Yoon, Kwon Ha [Wonkwang University Hospital, Iksan (Korea, Republic of); Park, Seong Hoon [Asan Medical Center, Seoul (Korea, Republic of)

2006-12-15

A 48-year-old woman was admitted to our hospital with a palpable mass in her lower abdomen. A left ovarian, cystic mass containing multiple mobile globules was seen on CT and MR images. The outer portion of the globules showed fat components on CT and fat-saturated T1-weighted MR images. Ultrasonography showed multiple echogenic, mobile globules with some sound attenuation and hyper echoic lines and dots within the cystic mass, which corresponded with the presence of lipid globules and hair shafts of ovarian mature cystic teratoma, respectively

Ovarian Mature Cystic Teratoma Containing Multiple Mobile

International Nuclear Information System (INIS)

Cho, Hyun Sun; Yoon, Seong Eon; Lee, Young Hwan; Kim, Hye Won; Yoon, Kwon Ha; Park, Seong Hoon

2006-01-01

A 48-year-old woman was admitted to our hospital with a palpable mass in her lower abdomen. A left ovarian, cystic mass containing multiple mobile globules was seen on CT and MR images. The outer portion of the globules showed fat components on CT and fat-saturated T1-weighted MR images. Ultrasonography showed multiple echogenic, mobile globules with some sound attenuation and hyper echoic lines and dots within the cystic mass, which corresponded with the presence of lipid globules and hair shafts of ovarian mature cystic teratoma, respectively

Computed tomography of cystic lung lesions

International Nuclear Information System (INIS)

Grgic, A.; Heinrich, M.; Girmann, M.; Kramann, B.; Wilkens, H.; Uder, M.

2004-01-01

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.)

Unusual growth rate during cystic echinococcosis.

Science.gov (United States)

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

2014-04-01

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

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.

Endocytosis and intracellular protein degradation in cystic fibrosis fibroblasts

International Nuclear Information System (INIS)

Jessup, W.; Dean, R.T.

1983-01-01

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

Pathological and clinical features of cystic and noncystic glioblastomas

International Nuclear Information System (INIS)

Utsuki, Satoshi; Oka, Hidehiro; Suzuki, Sachio; Shimizu, Satoru; Tanizaki, Yoshinori; Kondo, Koji; Fujii, Kiyotaka; Tanaka, Satoshi; Kawano, Nobuyuki

2006-01-01

The aim of this study is to review the different histological and clinical characteristics of glioblastoma multiforme (GBM) with and without cysts (cystic and noncystic GBM, respectively). Thirty-seven GBM were collected; these were tumors for which more than 80% of the volume was surgically resected, including a portion of the peripheral parenchyma of the brain. Based on preoperative magnetic resonance (MR) imaging studies, tumors were tentatively classified as cystic GBM if more than 50% of their volume appeared to be liquid; otherwise, they were considered to be noncystic GBM. Tumor volumes were estimated from contrast-enhanced T 1 -weighted MR images. Edema was deduced from the maximum width of contrast-enhanced edges. Peritumoral pathological analysis showed distinct margins, indicating little or no infiltration of tumor cells into white matter. Five cases were classified as cystic and 32 were noncystic GBMs. There was a statistically significant difference in age (Mann-Whitney U test; P<0.05) between the patients with cystic tumors (median, 44 years; range, 26-59 years) and those with noncystic tumors (median, 54 years; range, 26-81 years). Four of the cystic tumors and eight of the noncystic tumors were more than 5 cm in maximum diameter. Cystic GBMs had a well-defined tumor interface and less than 2-cm-thick peritumoral edema compared to the noncystic GBMs (Fisher's exact test; P<0.05). For patients with cystic GBMs, median survival time after surgery was 19.8 months and the 2-year survival rate was 50%. Patients with noncystic GBMs had a median survival time of 12.8 months and a 2-year survival rate of only 17%. Median time to tumor recurrence was 13.3 months for patients harboring cystic GBMs and 8.5 months for those with noncystic GBMs (log-rank test; P<0.05). Thus, the prognosis for cystic GBM was significantly better than that for noncystic GBM, possibly because cystic GBMs showed comparatively little infiltration of the peritumoral brain parenchyma

Mucinous cystic neoplasm of the pancreas in a male patient

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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.

Huge bilateral ovarian cysts in adulthood as the presenting feature of Van Wyk Grumbach syndrome due to chronic uncontrolled juvenile hypothyroidism

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K S Shivaprasad

2013-01-01

Full Text Available Juvenile primary hypothyroidism causing cystic ovaries and pseudoprecocious puberty (Van-Wyk Grumbach syndrome (VWGS is well documented in literature. There are only a few reports of primary hypothyroidism presenting as ovarian cysts in adults. Here we present a case of huge bilateral ovarian cysts in adulthood as the presenting feature of VWGS due to chronic uncontrolled juvenile hypothyroidism. Large uniloculor right ovarian cyst (119 × 81 × 90 mm and a multicystic left ovary (55 × 45 × 49 mm were detected in a 24 year lady with secondary amenorrhea, galactorrhea, and palpable abdominal mass with history of neonatal jaundice, delayed milestones, short stature, and precocious menarche at age of 7.5 years age. She had elevated levels of cancer antigen (CA-125 which normalized post levothyroxine supplementation. Elevated CA-125 may lead to misdiagnosis of ovarian carcinoma and inadvertent treatment. Bilateral ovarian cysts in adults are a rare presentation of juvenile hypothyroidism. It is necessary to screen for primary hypothyroidism in patients presenting with bilateral ovarian cysts to prevent unnecessary evaluation and treatment.

Oral foregut cyst in a neonate.

Science.gov (United States)

Rosa, Ana Cláudia Garcia; Hiramatsu, Daniel Martins; de Moraes, Fábio Roberto Ruiz; Passador-Santos, Fabrício; de Araújo, Vera Cavalcanti; Soares, Andresa Borges

2013-11-01

Oral foregut cysts are congenital choristomas that arise in the oral cavity during embryonic development from remnants of foregut-derived epithelium. This is an unusual report of a neonate with a large congenital sublingual cystic lesion, extending superficially from the left ventral tongue to the anterior floor of the mouth, impeding breast-feeding. The differential diagnosis included dermoid cyst, epidermoid cyst, mucous retention cyst, and oral lymphangioma. The treatment of choice was enucleation under general anesthesia. Histology showed a cystic lesion with a ciliated pseudostratified columnar epithelium with numerous goblet cells. Immunohistochemistry was positive for cytokeratin 7 and thyroid transcription factor 1 and negative for cytokeratin 20, resulting in a final diagnosis of an oral foregut cyst. Three weeks after surgery, the tongue had healed with good mobility, and breast-feeding could be established. No recurrence was present at 6 months of follow-up.

Incidence of cystic changes in impacted lower third molar

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Shridevi R Adaki

2013-01-01

Full Text Available Objective: To assess the incidence of cystic changes in the impacted lower third molar (ILTM in which the pericoronal (follicular space is less than 2.5 mm as measured from the radiograph. The relationship between the cystic changes and patient′s age, sex, and angular position and contact of ILTM with adjacent tooth was also evaluated. Materials and Methods: Follicular space less than 2.5 mm as measured from the panoramic radiograph was included in the study. A total of 73 tissue samples collected during the extraction ILTM were examined histopathologically. Then the data were analyzed for associations with age, sex, angular position, and contact of the ILTM with an adjacent tooth. Results: There were 37 male and 36 female patients, age ranging from 17 to 35 years (mean 23.95 years. Out of 73 specimens, 17 (23.3% showed cystic changes; among them 16 (22.1% showed dentigerous cysts and 1 (1.2% showed odontogenic keratocysts. Most of the cystic changes occurred in the 26-30 year age range. The cystic changes showed male predominance but could not gain statistical significance. The relationship between cystic changes and angular position was statistically significant (P < 0.05. Higher probability was found in distoangular positioned ILTM. The relationship between cystic changes and communication of ILTM with the second molar was not statistically significant. Conclusion: Incidence of cystic changes in ILTM justifies extraction of the impacted tooth associated with symptoms. The decision to extract or not to extract impacted third molar should be individualized, rather than generalized.

Interdisciplinary Management of Cystic Neoplasms of the Pancreas

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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.

Newtonian self-gravitating system in a relativistic huge void universe model

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Nishikawa, Ryusuke; Nakao, Ken-ichi [Department of Mathematics and Physics, Graduate School of Science, Osaka City University, 3-3-138 Sugimoto, Sumiyoshi, Osaka 558-8585 (Japan); Yoo, Chul-Moon, E-mail: ryusuke@sci.osaka-cu.ac.jp, E-mail: knakao@sci.osaka-cu.ac.jp, E-mail: yoo@gravity.phys.nagoya-u.ac.jp [Division of Particle and Astrophysical Science, Graduate School of Science, Nagoya University, Furo-cho, Chikusa-ku, Nagoya 464-8602 (Japan)

2016-12-01

We consider a test of the Copernican Principle through observations of the large-scale structures, and for this purpose we study the self-gravitating system in a relativistic huge void universe model which does not invoke the Copernican Principle. If we focus on the the weakly self-gravitating and slowly evolving system whose spatial extent is much smaller than the scale of the cosmological horizon in the homogeneous and isotropic background universe model, the cosmological Newtonian approximation is available. Also in the huge void universe model, the same kind of approximation as the cosmological Newtonian approximation is available for the analysis of the perturbations contained in a region whose spatial size is much smaller than the scale of the huge void: the effects of the huge void are taken into account in a perturbative manner by using the Fermi-normal coordinates. By using this approximation, we derive the equations of motion for the weakly self-gravitating perturbations whose elements have relative velocities much smaller than the speed of light, and show the derived equations can be significantly different from those in the homogeneous and isotropic universe model, due to the anisotropic volume expansion in the huge void. We linearize the derived equations of motion and solve them. The solutions show that the behaviors of linear density perturbations are very different from those in the homogeneous and isotropic universe model.

Ivacaftor: A Novel Gene-Based Therapeutic Approach for Cystic Fibrosis

OpenAIRE

Condren, Michelle E.; Bradshaw, Marquita D.

2013-01-01

Ivacaftor is a new therapeutic agent that acts at the cystic fibrosis transmembrane conductance regulator (CFTR) channel to alter activity. It is approved for use in patients 6 years and older with cystic fibrosis who have at least 1 G551D mutation in the CFTR gene. It is unlike any other current pharmacologic agent for cystic fibrosis in that it specifically targets the gene defect associated with cystic fibrosis as opposed to treating resulting symptomology. Mucoactive agents, antibiotics, ...

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 ...... to indicators of health, the role of CF Centres, regional networks, national health policy, and international data registration and comparisons.......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...... therapies, approaches to care and indeed data recording. The quality of care for individuals with CF has become a focus at several levels: patient, centre, regional, national and international. This paper reviews the quality management and improvement issues at each of these levels with particular reference...

Immunoreactive trypsin and neonatalscreening for cystic fibrosis

International Nuclear Information System (INIS)

Travert, G.; Laroche, D.; Blandin, C.; Pasquet, C.

1988-01-01

Immunoreactive trypsin (IRT) was measured in dried blood spots from 160.822 five-day-old babies as a part of a regionwide neonatal screening program for cystic fibrosis. A second test was performed for 492 babies in whom blood IRT levels were found greater than 900 μg/l; retesting revealed persistent elevation in 55. Sweat testing confirmed cystic fibrosis in 43 babies, but results were normal in 12. During the course of this study, a total of 51 cystic fibrosis babies were identified: 43 by newborn screening, 6 because they had meconium ileus; so, early diagnosis was achieved in 49 cases out of 51. Two newborn babies did not have elevated IRT and they were missed by the screening test. Our results confirm that elevated blood IRT is characteristic of newborn babies with cystic fibrosis and show that this test has an excellent specificity (99.7%) and a good sensitivity (95%) when used as a neonatal screening test [fr

A case report of corgenotal cystic adenomatoid malformation

International Nuclear Information System (INIS)

Jun, Soon Ae; Cha, Kyung Sub; Chi, Je Geun

1987-01-01

Congenital cystic adnomatoid malformation (CCAM) is rare pulmonary cystic disease. CCAM has been detected on prematurity, stillborn and respiratory distress infant or child by chest X-ray film and CT scan. One case of CCAM diagnosed in utero at gestational age 22 weeks is reported with sonographic findings and autopsy findings. Ultrasonographic findings are large cystic lesion in fetal thorax and fetal hydrops without hydramnios. The survival of these infants is very poor despite accurate prenatal diagnosis and maximal postnatal care

Cystic fibroadenoma of the breast: a case report.

Science.gov (United States)

Durak, Merih Güray; Karaman, Ilgın; Canda, Tülay; Balci, Pınar; Harmancioğlu, Omer

2011-01-01

Fibroadenoma is the most common breast tumor in adolescent and young women. Fibroadenomas that consist of sclerosing adenosis, papillary apocrine metaplasia, epithelial calcifications, and/or cysts greater than 3 mm are considered as complex fibroadenoma. The relative risk of developing breast cancer in patients with complex fibroadenoma is increased, compared to women with noncomplex fibroadenoma. Extensive cystic degeneration in a fibroadenoma, so called "cystic fibroadenoma" is very rare. Herein, we present a case of such a lesion in a 43-year-old female who has been on follow-up for fibrocystic changes of the breast, and discuss both radiological and histopathologic differential diagnosis of this lesion with other cystic lesions of the breast, including cystic papilloma. The patient is free of disease after 17 months of clinical follow-up.

PECULIARITIES OF ENT-DAMAGE IN CHILDREN WITH CYSTIC FIBROSIS

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I.V. Martynova

2011-01-01

Full Text Available Traditional approach to cystic fibrosis patients treatment doesn’t involve upper respiratory tract assessment, though abnormal changes — consequences of the cystic fibrosis transmembrane conductivity regulator gene mutation- do affect nasal and paranasal mucosa to the same extent. Approximately half of cystic fibrosis patients suffer from chronic rhinosinusitis and/or nasal polyposis that worsens the clinical course of already severe disease. Chronic hyperplasia in paranasal cavities can be quite extensive, recurrent and can lead to destruction of osseous walls of the cavity and of nasal septum. Thus increasing the amount of hospital admissions and and their duration. Low awareness of ENT-specialists working in polyclinics and in hospitals of ENT-pathology in cystic fibrosis patients leads to belated diagnostics, excessive manipulations, ineffective treatment, including surgery. All these lays grounds to implication of the early screening diagnostic program and development of proper treatment methods of ENT-complications of cystic fibrosis — therapeutic as well as surgical, with strict specification of indications and contraindications. Key words: cystic fibrosis, chronic rhino sinusitis, nasal polyposis. (Voprosy sovremennoi pediatrii — Current Pediatrics. — 2011; 10 (5: 49–53.

Cystic Odontoma in a Patient with Hodgkin’s Lymphoma

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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.

Cystic neuroblastoma: a case report

International Nuclear Information System (INIS)

Duran, A.; Lorente, M.L.; Fernandez, C.

1997-01-01

Neuroblastoma is the most common neonatal malignant tumor. Hemorrhage and necrosis are usual features of this lesion, but it rarely presents a totally cyst form. We report a case of cystic neuroblastoma detected on prenatal ultrasound and stress the need to include it in the differential diagnosis of cystic abdominal masses in the newborn. Ultrasound is the method of choice for assessing abdominal masses in children. However, magnetic resonance has been shown to be more advantageous for the study and follow-up of neuroblastomas. (Author) 16 refs

Thyroid nodules with minimal cystic changes have a low risk of malignancy

International Nuclear Information System (INIS)

Na, Dong Gyu; Kim, Dae Sik; Kim, Soo Jin; Kim, Ji Hoon

2016-01-01

The goal of this study was to determine the risk of malignancy of thyroid nodules with minimal cystic changes. A total of consecutive 1,000 thyroid nodules (≥1 cm) with final diagnoses from two institutions were included in this study. The risk of malignancy of thyroid nodules was analyzed according to the internal content, which was categorized as purely solid, minimally cystic (cystic changes ≤10%), and partially cystic (cystic changes >10%). We also assessed the risk of malignancy of nodules with minimal cystic changes depending on echogenicity and presence of any suspicious ultrasonografic (US) features. The overall frequency of purely solid, minimally cystic, and partially cystic nodules was 730/1,000 (73%), 61/1,000 (6.1%), and 209/1,000 (20.9%), respectively, with risks of malignancy of 14.8% (108/730), 3.3% (2/61), and 3.3% (7/209), respectively. The risk of malignancy of nodules with minimal cystic changes was significantly lower than that of purely solid nodules (P=0.013). The risk of malignancy of nodules with minimal cystic changes was also lower than that of purely solid nodules in the group of hypoechoic nodules (P=0.063) and in the group of nodules with suspicious US features (P=0.028), but was not significantly different from that of partially cystic nodules regardless of echogenicity or the presence of suspicious US features (P≥0.652). Thyroid nodules with minimal cystic changes have a low risk of malignancy, similar to that of partially cystic nodules regardless of echogenicity or the presence of suspicious US features. The US lexicon could define solid nodules as nodules with purely solid internal content in order to enhance the accuracy of estimated risks of malignancy

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;…

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...

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...

MRI of cystic pituitary tumors

Energy Technology Data Exchange (ETDEWEB)

Tokunaga, Hitoshi; Hoshi, Seiichiro; Sunada, Souichi; Sunami, Kenro [Kawatetsu Chiba Hospital (Japan); Saeki, Naokatsu; Yamaura, Akira

1998-11-01

We retrospectively reviewed MRI findings of 17 patients with 3 histologically proven cystic pituitary tumors. They consisted of 10 cystic pituitary adenomas, 4 craniopharyngiomas and 3 Rathke`s cleft cysts. We analyzed the following MRI parameters such as cyst wall appearance, enhancement pattern of cyst wall, location of residual pituitary gland and location of tumor. They were clinically significant parameters for histological differentiation. Even though combinations of such MRI parameters helped for more accurate preoperative diagnosis, the differentiation between craniopharyngioma and Rathke`s cleft cyst was difficult in some cases. (author)

MRI of cystic pituitary tumors

International Nuclear Information System (INIS)

Tokunaga, Hitoshi; Hoshi, Seiichiro; Sunada, Souichi; Sunami, Kenro; Saeki, Naokatsu; Yamaura, Akira

1998-01-01

We retrospectively reviewed MRI findings of 17 patients with 3 histologically proven cystic pituitary tumors. They consisted of 10 cystic pituitary adenomas, 4 craniopharyngiomas and 3 Rathke's cleft cysts. We analyzed the following MRI parameters such as cyst wall appearance, enhancement pattern of cyst wall, location of residual pituitary gland and location of tumor. They were clinically significant parameters for histological differentiation. Even though combinations of such MRI parameters helped for more accurate preoperative diagnosis, the differentiation between craniopharyngioma and Rathke's cleft cyst was difficult in some cases. (author)

Huge uterine-cervical diverticulum mimicking as a cyst

Directory of Open Access Journals (Sweden)

S Chufal

2012-01-01

Full Text Available Here we report an incidental huge uterine-cervical diverticulum from a total abdominal hysterectomy specimen in a perimenopausal woman who presented with acute abdominal pain. The diverticulum was mimicking with various cysts present in the lateral side of the female genital tract. Histopathological examination confirmed this to be a cervical diverticulum with communication to uterine cavity through two different openings. They can attain huge size if left ignored for long duration and present a diagnostic challenge to clinicians, radiologists, as well as pathologists because of its extreme rarity. Therefore, diverticula should also be included as a differential diagnosis. Its histopathological confirmation also highlights that diverticula can present as an acute abdomen, requiring early diagnosis with appropriate timely intervention. Immunohistochemistry CD 10 has also been used to differentiate it from a mesonephric cyst.

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....

Sclerotherapy for Benign Cystic Diseases in the Neck

Energy Technology Data Exchange (ETDEWEB)

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

2012-08-15

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

Sclerotherapy for Benign Cystic Diseases in the Neck

International Nuclear Information System (INIS)

Kim, Ji Hoon; Sohn, Chul Ho; Choi, Seung Hong; Yun, Tae Jin

2012-01-01

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

Gastroenterological endpoints in drug trials for cystic fibrosis

NARCIS (Netherlands)

Bodewes, Frank A. J. A.; Verkade, Henkjan J.; Wilschanski, Micheal

2016-01-01

The phenotype of cystic fibrosis includes a wide variety of clinical and biochemical gastrointestinal presentations. These gastrointestinal characteristics of the disease have come under renewed interest as potential outcome measures and clinical endpoints for therapeutic trials in cystic fibrosis.

Cystic duct remnant mucocele in a liver transplant recipient

International Nuclear Information System (INIS)

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

2008-01-01

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

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.)

Liver Disease in Cystic Fibrosis: an Update

Science.gov (United States)

Parisi, Giuseppe Fabio; Di Dio, Giovanna; Franzonello, Chiara; Gennaro, Alessia; Rotolo, Novella; Lionetti, Elena; Leonardi, Salvatore

2013-01-01

Context Cystic fibrosis (CF) is the most widespread autosomal recessive genetic disorder that limits life expectation amongst the Caucasian population. As the median survival has increased related to early multidisciplinary intervention, other manifestations of CF have emergedespecially for the broad spectrum of hepatobiliary involvement. The present study reviews the existing literature on liver disease in cystic fibrosis and describes the key issues for an adequate clinical evaluation and management of patients, with a focus on the pathogenetic, clinical and diagnostic-therapeutic aspects of liver disease in CF. Evidence Acquisition A literature search of electronic databases was undertaken for relevant studies published from 1990 about liver disease in cystic fibrosis. The databases searched were: EMBASE, PubMed and Cochrane Library. Results CF is due to mutations in the gene on chromosome 7 that encodes an amino acidic polypeptide named CFTR (cystic fibrosis transmembrane regulator). The hepatic manifestations include particular changes referring to the basic CFTR defect, iatrogenic lesions or consequences of the multisystem disease. Even though hepatobiliary disease is the most common non-pulmonary cause ofmortalityin CF (the third after pulmonary disease and transplant complications), only about the 33%ofCF patients presents clinically significant hepatobiliary disease. Conclusions Liver disease will have a growing impact on survival and quality of life of cystic fibrosis patients because a longer life expectancy and for this it is important its early recognition and a correct clinical management aimed atdelaying the onset of complications. This review could represent an opportunity to encourage researchers to better investigate genotype-phenotype correlation associated with the development of cystic fibrosis liver disease, especially for non-CFTR genetic polymorphisms, and detect predisposed individuals. Therapeutic trials are needed to find strategies of

Neurofibromas as bilateral cystic chest wall swellings.

African Journals Online (AJOL)

secondary to an infection, usually parasitic infections. [6,7]. However, cystic tumours of the chest wall result- ing from degenerative changes in peripheral nerves of its layers are rare, and we did not see any in the pub- lished literature. We are reporting a single case of bilat- eral cystic degenerative changes in neurofibromas ...

Congenital cystic masses of the face and neck: CT evaluation

International Nuclear Information System (INIS)

Chung, Hae Gyeong; Kim, Hyung Jin; Kim, Jae Hyoung; Hwang, Eui Gee; Jeon, Sea Young; Kim, Sun Young; Chung, Sung Hoon

1991-01-01

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

Congenital cystic masses of the face and neck: CT evaluation

Energy Technology Data Exchange (ETDEWEB)

Chung, Hae Gyeong; Kim, Hyung Jin; Kim, Jae Hyoung; Hwang, Eui Gee; Jeon, Sea Young; Kim, Sun Young; Chung, Sung Hoon [Gyeongsang National University, Jinju (Korea, Republic of)

1991-09-15

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.

[Recurrent benign cystic peritoneal mesothelioma].

Science.gov (United States)

Stroescu, C; Negulescu, Raluca; Herlea, V; David, L; Ivanov, B; Nitipir, Cornelia; Popescu, I

2008-01-01

The benign cystic peritoneal mesothelioma (BCPM) is a rare neoplasm affecting mainly females at reproductive age. The natural history and physiopathology of the BCPM are not entirely known. It is mainly characterized by the lack of malignant elements, no tendency to metastasis and by a pervasive tendency to generate local recurrences after surgical removal. The clinical manifestations are insidious, uncharacteristic; the benign cystic peritoneal mesothelioma is often discovered during a surgical procedure addressing another condition. Imaging tests can raise the suspicion of BCPM but the diagnostic can only be confirmed by histopathological examination corroborated with an immunohistochemical analysis. There are no long term studies dictating a single therapeutic attitude but a high risk of local recurrences and the possibility of transformation into malignant mesothelioma have lead to the current tendency towards an aggressive treatment of the tumor. We present the case of a recurrent benign cystic peritoneal mesothelioma in a 40 years old female patient, emphasizing the therapeutic approach and the role of radical surgery in the treatment of BPCM.

Physical exercise training for cystic fibrosis.

Science.gov (United States)

Radtke, Thomas; Nevitt, Sarah J; Hebestreit, Helge; Kriemler, Susi

2017-11-01

Physical exercise training may form an important part of regular care for people with cystic fibrosis. This is an update of a previously published review. To assess the effects of physical exercise training on exercise capacity by peak oxygen consumption, pulmonary function by forced expiratory volume in one second, health-related quality of life and further important patient-relevant outcomes in people with cystic fibrosis. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register which comprises references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings.Date of the most recent search: 04 May 2017.We searched ongoing trials registers (clinicaltrials.gov and the WHO ICTRP). Date of most recent search: 10 August 2017. All randomised and quasi-randomised controlled clinical trials comparing exercise training of any type and a minimum duration of two weeks with conventional care (no training) in people with cystic fibrosis. Two authors independently selected studies for inclusion, assessed methodological quality and extracted data. The quality of the evidence was assessed using the GRADE system. Of the 83 studies identified, 15 studies which included 487 participants, met the inclusion criteria. The numbers in each study ranged from nine up to 72 participants; two studies were in adults, seven were in children and adolescents and six studies included all age ranges. Four studies of hospitalised participants lasted less than one month and 11 studies were outpatient-based, lasting between two months and three years. The studies included participants with a wide range of disease severity and employed differing levels of supervision with a mixture of types of training. There was also wide variation in the quality of the included studies.This systematic review shows very low- to low-quality evidence from both short- and long-term studies that in people

Connecting slow earthquakes to huge earthquakes

OpenAIRE

Obara, Kazushige; Kato, Aitaro

2016-01-01

Slow earthquakes are characterized by a wide spectrum of fault slip behaviors and seismic radiation patterns that differ from those of traditional earthquakes. However, slow earthquakes and huge megathrust earthquakes can have common slip mechanisms and are located in neighboring regions of the seismogenic zone. The frequent occurrence of slow earthquakes may help to reveal the physics underlying megathrust events as useful analogs. Slow earthquakes may function as stress meters because of th...

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.

Cystic hemispheric medulloepithelioma

African Journals Online (AJOL)

2015-12-09

Dec 9, 2015 ... A right parieto-occipital craniotomy with subtotal resection was performed. Histological sections. (Figure 3) showed a primitive neuro-ectodermal tumour with a heterogeneous appearance and cystic change. Areas of primitive embryonal cells alternated with areas showing trabecular, papillary and.

Appetite stimulants for people with cystic fibrosis.

Science.gov (United States)

Chinuck, Ruth; Dewar, Jane; Baldwin, David R; Hendron, Elizabeth

2014-07-27

Chronic loss of appetite in cystic fibrosis concerns both individuals and families. Appetite stimulants have been used to help cystic fibrosis patients with chronic anorexia attain optimal body mass index and nutritional status. However, these may have adverse effects on clinical status. The aim of this review is to systematically search for and evaluate evidence on the beneficial effects of appetite stimulants in the management of CF-related anorexia and synthesize reports of any side-effects. Trials were identified by searching the Cochrane Cystic Fibrosis and Genetic Disorders Group's Cystic Fibrosis Trials Register, MEDLINE, Embase, CINAHL, handsearching reference lists and contacting local and international experts.Last search of online databases: 01 April 2014.Last search of the Cystic Fibrosis Trials Register: 08 April 2014. Randomised and quasi-randomised controlled trials of appetite stimulants, compared to placebo or no treatment for at least one month in adults and children with cystic fibrosis. Authors independently extracted data and assessed the risk of bias within eligible trials. Meta-analyses were performed. Three trials (total of 47 recruited patients) comparing appetite stimulants (cyproheptadine hydrochloride and megesterol acetate) to placebo were included; the numbers of adults or children within each trial were not always reported. The risk of bias of the included trials was graded as moderate.A meta-analysis of all three trials showed appetite stimulants produced a larger increase in weight z score at three months compared to placebo, mean difference 0.61 (95% confidence interval 0.29 to 0.93) (P children, appetite stimulants improved only two of the outcomes in this review - weight (or weight z score) and appetite; and side effects were insufficiently reported to determine the full extent of their impact. Whilst the data may suggest the potential use of appetite stimulants in treating anorexia in adults and children with cystic fibrosis

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.

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...

Cystic rheumatoid arthritis: description of a nonerosive form

NARCIS (Netherlands)

Gubler, F. M.; Maas, M.; Dijkstra, P. F.; de Jongh, H. R.

1990-01-01

In a study of patients with rheumatoid arthritis (RA), 9% (n = 70) were found to have a cystic form. At radiologic examination of these patients with cystic RA, the first abnormality seen consisted of periarticular intraosseous cysts without erosions. The cysts were distributed symmetrically, most

Laparoscopic management of cystic disease of the liver.

Science.gov (United States)

Albrink, M H; McAllister, E W; Rosemurgy, A S; Karl, R C; Carey, L C

1994-04-01

Laparoscopic surgical procedures are increasing in scope and in variety. The benefits of decreased wound morbidity and pain have been well documented for multiple procedures that have traditionally required laparotomy. Although there are few controlled studies to document them, these benefits may be evident from simple clinical observation. Cystic disease of the liver is a condition that is treated largely for symptomatic reasons. The so-called noninvasive or radiographic guided methods of treatment for cystic disease of the liver are fraught with high recurrence rates. We present four cases of cystic disease of the liver treated laparoscopically, followed with pertinent discussion.

Cystic meningiomas in 2 dogs

International Nuclear Information System (INIS)

Bagley, R.S.; Kornegay, J.N.; Lane, S.B.; Thrall, D.L.; Page, R.L.

1996-01-01

Two dogs with signs of forebrain disease had hypodense lesions on computed tomography evaluation. Magnetic resonance imaging of the first dog showed a hypointense lesion on the T1-weighted scan and a hyperintense lesion on T2-weighted scanning. At surgery, both dogs had a primary cystic intracranial lesion, and the abnormal tissue adjacent to the cyst had histological features of meningioma. Each dog underwent whole brain irradiation after surgery, and 1 dog lived for 3 years after treatment. While uncommon, meningioma should be considered as a differential diagnosis in dogs with cystic intracranial lesions

Subclinical anaemia of chronic disease in adult patients with cystic fibrosis.

LENUS (Irish Health Repository)

O'connor, T M

2012-02-03

Patients with chronic hypoxaemia develop secondary polycythaemia that improves oxygen-carrying capacity. Therefore, normal haemoglobin and haematocrit values in the presence of chronic arterial hypoxaemia in cystic fibrosis constitute \\'relative anaemia\\'. We sought to determine the cause of this relative anaemia in patients with cystic fibrosis. We studied haematological indices and oxygen saturation in healthy volunteers (n=17) and in adult patients with cystic fibrosis (n=15). Patients with cystic fibrosis had lower resting arterial oxygen saturation when compared with normal volunteers (P<0.0001), and exercise led to a greater reduction in arterial oxygen saturation (P<0.0001). However, haemoglobin and haematocrit values in patients with cystic fibrosis did not significantly differ from normal volunteers. Serum iron (P=0.002), transferrin (P=0.02), and total iron-binding capacity (P=0.01) were lower in patients with cystic fibrosis. There were no significant differences in serum ferritin, percentage iron saturation, serum erythropoietin or red cell volume between the groups. The data presented demonstrate a characteristic picture of anaemia of chronic disease in adult patients with cystic fibrosis, except for normal haemoglobin and haematocrit values. Normal haemoglobin and haematocrit values in patients with cystic fibrosis appear to represent a combination of the effects of arterial hypoxaemia promoting polycythaemia, counterbalanced by chronic inflammation promoting anaemia of chronic disease.

Ovarian cystic teratoma containing balls of fat. A case report

International Nuclear Information System (INIS)

Salinas, A.; Rebolledo, M.; Escribano, M.; Alejo, J. P.; Morenom, J.

1998-01-01

We present the case of a ovarian cystic teratoma characterized predominantly by the mobile balls floating in the intra cystic fluid. Ultrasonography demonstrated their marked echo reflectivity and computed tomography revealed that they had the density of fat. We establish a relationship among the ultrasound, computed tomography and histological findings in this uncommon type of ovarian cystic teratoma. (Author) 6 refs

Cystic malformations of the neck in children

Energy Technology Data Exchange (ETDEWEB)

Koch, Bernadette L. [Cincinnati Children' s Hospital Medical Center, Department of Radiology, Cincinnati, OH (United States)

2005-05-01

The most common cystic malformations of the neck are the result of abnormal embryogenesis involving the thyroglossal duct (TGD), lymphatic primordia and the branchial apparatus. When the basic embryology of these structures is considered, a reasonable differential diagnosis - and in some cases a definitive diagnosis - can be achieved based on the location and the imaging characteristics of the cystic mass. (orig.)

Cystic malformations of the neck in children

International Nuclear Information System (INIS)

Koch, Bernadette L.

2005-01-01

The most common cystic malformations of the neck are the result of abnormal embryogenesis involving the thyroglossal duct (TGD), lymphatic primordia and the branchial apparatus. When the basic embryology of these structures is considered, a reasonable differential diagnosis - and in some cases a definitive diagnosis - can be achieved based on the location and the imaging characteristics of the cystic mass. (orig.)

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.

The role of anaerobic bacteria in the cystic fibrosis airway.

Science.gov (United States)

Sherrard, Laura J; Bell, Scott C; Tunney, Michael M

2016-11-01

Anaerobic bacteria are not only normal commensals, but are also considered opportunistic pathogens and have been identified as persistent members of the lower airway community in people with cystic fibrosis of all ages and stages of disease. Currently, the role of anaerobic bacteria in cystic fibrosis lower airway disease is not well understood. Therefore, this review describes the recent studies relating to the potential pathophysiological role(s) of anaerobes within the cystic fibrosis lungs. The most frequently identified anaerobic bacteria in the lower airways are common to both cystic fibrosis and healthy lungs. Studies have shown that in cystic fibrosis, the relative abundance of anaerobes fluctuates in the lower airways with reduced lung function and increased inflammation associated with a decreased anaerobic load. However, anaerobes found within the lower airways also produce virulence factors, may cause a host inflammatory response and interact synergistically with recognized pathogens. Anaerobic bacteria are potentially members of the airway microbiota in health but could also contribute to the pathogenesis of lower airway disease in cystic fibrosis via both direct and indirect mechanisms. A personalized treatment strategy that maintains a normal microbial community may be possible in the future.

Radiologic features of cystic, endocrine and other pancreatic neoplasms

International Nuclear Information System (INIS)

Balci, N. Cem; Semelka, Richard C.

2001-01-01

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

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...

Constructing Optimal Coarse-Grained Sites of Huge Biomolecules by Fluctuation Maximization.

Science.gov (United States)

Li, Min; Zhang, John Zenghui; Xia, Fei

2016-04-12

Coarse-grained (CG) models are valuable tools for the study of functions of large biomolecules on large length and time scales. The definition of CG representations for huge biomolecules is always a formidable challenge. In this work, we propose a new method called fluctuation maximization coarse-graining (FM-CG) to construct the CG sites of biomolecules. The defined residual in FM-CG converges to a maximal value as the number of CG sites increases, allowing an optimal CG model to be rigorously defined on the basis of the maximum. More importantly, we developed a robust algorithm called stepwise local iterative optimization (SLIO) to accelerate the process of coarse-graining large biomolecules. By means of the efficient SLIO algorithm, the computational cost of coarse-graining large biomolecules is reduced to within the time scale of seconds, which is far lower than that of conventional simulated annealing. The coarse-graining of two huge systems, chaperonin GroEL and lengsin, indicates that our new methods can coarse-grain huge biomolecular systems with up to 10,000 residues within the time scale of minutes. The further parametrization of CG sites derived from FM-CG allows us to construct the corresponding CG models for studies of the functions of huge biomolecular systems.

Occlusion of the cystic duct by electrocoagulation: A radiologic technique

International Nuclear Information System (INIS)

Becker, C.D.; Quenville, W.F.; Burhenne, H.J.

1987-01-01

Chemical dissolution and extracorporeal shock wave lithotripsy are promising new methods for the treatment of cholelithiasis without cholecystectomy. Nonsurgical defunctionalization of the gallbladder is now required to prevent recurrent stone formation. The authors consider cystic duct occlusion to be the first step. Ten domestic pigs underwent transcatheter electrocoagulation of the cystic duct via a cholecystostomy under fluoroscopic control. Stricture formation was followed by complete cystic duct occlusion in all ten cases. After a follow-up period ranging from 2 to 17 weeks (mean, 13 weeks), the animals were killed. Histologic studies demonstrated that complete obliteration of the cystic duct lumen was due to fibrous scar formation

Festival food coma in cystic fibrosis.

Science.gov (United States)

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

2013-07-01

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

Congenital cystic lung malformations

International Nuclear Information System (INIS)

Stoever, B.; Scheer, I.; Bassir, C.; Chaoui, R.; Henrich, W.; Schwabe, M.; Wauer, R.

2006-01-01

Purpose: The aim of the study concerning congenital cystic lung malformations was to evaluate prenatal diagnoses postnatally to determine prognostic factors as well as to define optimized perinatal management. Materials and Methods: The study is based on 45 prenatal ultrasound examinations depicting fetal cystic lung lesions. 32 of the mothers had follow-up examinations. 5 pregnancies were terminated due to CCAM and additional malformations. Complete regression of the lesions was seen prenatally in 8 cases and postnatally in 5 children. Results: Surgical intervention due to respiratory insufficiency was necessary in 4 neonates. According to the imaging results, CCAM was present in 4 cases and sequestration in 7 patients. No correlation between the imaging findings and the surgical results was found in 3 children: One child suffered from rhadomyoid dysplasia, and in the case of the second child, a left-sided hernia of the diaphragm and additional sequestration were detected. The third child showed AV malformation. The cystic lesions of the 14 children operated upon were proven histologically. The degree of accuracy in the present study was high. Conclusion: Precise perinatal management is warranted in order to determine according to the clinical relevance surgical intervention and to prevent complications after the first year of life. This is performed during the neonatal period for respiratory insufficient neonates and within the first year of life for clinically stable children. (orig.)

Vorinostat in Treating Patients With Locally Advanced, Recurrent, or Metastatic Adenoid Cystic Carcinoma

Science.gov (United States)

2018-03-22

Recurrent Oral Cavity Adenoid Cystic Carcinoma; Recurrent Salivary Gland Carcinoma; Salivary Gland Adenoid Cystic Carcinoma; Stage III Major Salivary Gland Cancer AJCC v7; Stage III Oral Cavity Adenoid Cystic Carcinoma AJCC v6 and v7; Stage IVA Major Salivary Gland Cancer AJCC v7; Stage IVA Oral Cavity Adenoid Cystic Carcinoma AJCC v6 and v7; Stage IVB Major Salivary Gland Cancer AJCC v7; Stage IVB Oral Cavity Adenoid Cystic Carcinoma AJCC v6 and v7; Stage IVC Major Salivary Gland Cancer AJCC v7; Stage IVC Oral Cavity Adenoid Cystic Carcinoma AJCC v6 and v7; Tongue Carcinoma

Cystic meningioma: unusual entity with review of literature

Directory of Open Access Journals (Sweden)

Maheshwari Vikas

2017-12-01

Full Text Available Cystic meningioma is a relatively rare condition, radiological appearance of the cystic-solid components of the mass may create a diagnostic dilemma. The presence of a cyst is not a common imaging feature and this makes it difficult to differentiate it from hemangioblastoma, craniopharyngioma, metastasis and gliomas. Cystic meningiomas are present more commonly in children. We present a 60 year old male who presented with seizures and frontal lobe signs. The lesion was suspected as glioma however, postoperative histopathological examination demonstrated as meningioma. Patient showed remarkable recovery after surgery. Complete cyst resection should be considered if it is technically feasible and safe.

From tiny microalgae to huge biorefineries

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Gouveia, L.

2014-01-01

Microalgae are an emerging research field due to their high potential as a source of several biofuels in addition to the fact that they have a high-nutritional value and contain compounds that have health benefits. They are also highly used for water stream bioremediation and carbon dioxide mitigation. Therefore, the tiny microalgae could lead to a huge source of compounds and products, giving a good example of a real biorefinery approach. This work shows and presents examples of experimental...

Intrinsic pro-angiogenic status of cystic fibrosis airway epithelial cells

International Nuclear Information System (INIS)

Verhaeghe, Catherine; Tabruyn, Sebastien P.; Oury, Cecile; Bours, Vincent; Griffioen, Arjan W.

2007-01-01

Cystic fibrosis is a common genetic disorder characterized by a severe lung inflammation and fibrosis leading to the patient's death. Enhanced angiogenesis in cystic fibrosis (CF) tissue has been suggested, probably caused by the process of inflammation, as similarly described in asthma and chronic bronchitis. The present study demonstrates an intrinsic pro-angiogenic status of cystic fibrosis airway epithelial cells. Microarray experiments showed that CF airway epithelial cells expressed several angiogenic factors such as VEGF-A, VEGF-C, bFGF, and PLGF at higher levels than control cells. These data were confirmed by real-time quantitative PCR and, at the protein level, by ELISA. Conditioned media of these cystic fibrosis cells were able to induce proliferation, migration and sprouting of cultured primary endothelial cells. This report describes for the first time that cystic fibrosis epithelial cells have an intrinsic angiogenic activity. Since excess of angiogenesis is correlated with more severe pulmonary disease, our results could lead to the development of new therapeutic applications

MicroRNAs in the pathogenesis of cystic kidney disease.

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Phua, Yu Leng; Ho, Jacqueline

2015-04-01

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

MRI of the cystic mass lesions of the pancreas

International Nuclear Information System (INIS)

Ohtomo, Kuni; Itai, Yuji; Yoshikawa, Koki; Kokubo, Takashi; Yashiro, Naofumi; Iio, Masahiro

1987-01-01

Five cystic mass lesions of the pancreas were exemined by MRI. Multiplocular fluid components were demonstrated as areas of various signal intensity in mucinous cystadenoma and cystadenocarcinoma. Gas within the cystic mass was noted in ductectatic mucinous cystadenocarcinoma. Honeycomb pattern and classification were not depicted in serous cystadenoma. Necrotic matter was demonstrated as area of lower signal than liver in pseudocyst. These results were then compared with CT and ultrasound and at present enhanced CT combined with ultrasound is more diagnostic than MRI for cystic mass lesions of the pancreas. (author)

Phosphorus-32 therapy for cystic craniopharyngiomas

International Nuclear Information System (INIS)

Barriger, Robert Bryan; Chang, Andrew; Lo, Simon S.; Timmerman, Robert D.; DesRosiers, Colleen; Boaz, Joel C.; Fakiris, Achilles J.

2011-01-01

Background and purpose: To examine control rates for predominantly cystic craniopharyngiomas treated with intracavitary phosphorus-32 (P-32). Material and methods: 22 patients with predominantly cystic craniopharyngiomas were treated at Indiana University between October 1997 and December 2006. Nineteen patients with follow-up of at least 6 months were evaluated. The median patient age was 11 years, median cyst volume was 9 ml, a median dose of 300 Gy was prescribed to the cyst wall, and median follow-up was 62 months. Results: Overall cyst control rate after the initial P-32 treatment was 67%. Complete tumor control after P-32 was 42%. Kaplan-Meier 1-, 3-, and 5-year initial freedom-from-progression rates were 68%, 49%, and 31%, respectively. Following salvage therapy, the Kaplan-Meier 1-, 3-, and 5-year ultimate freedom-from-progression rates were 95%, 95%, and 86%, respectively. All patients were alive at the last follow-up. Visual function was stable or improved in 81% when compared prior to P-32 therapy. Pituitary function remained stable in 74% of patients following P-32 therapy. Conclusions: Intracystic P-32 can be an effective and tolerable treatment for controlling cystic components of craniopharyngiomas as a primary treatment or after prior therapies, but frequently allows for progression of solid tumor components. Disease progression in the form of solid tumor progression, re-accumulation of cystic fluid, or development of new cysts may require further radiotherapy or surgical intervention for optimal long-term disease control.

Nutrient Status of Adults with Cystic Fibrosis

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GORDON, CATHERINE M.; ANDERSON, ELLEN J.; HERLYN, KAREN; HUBBARD, JANE L.; PIZZO, ANGELA; GELBARD, RONDI; LAPEY, ALLEN; MERKEL, PETER A.

2011-01-01

Nutrition is thought to influence disease status in patients with cystic fibrosis (CF). This cross-sectional study sought to evaluate nutrient intake and anthropometric data from 64 adult outpatients with cystic fibrosis. Nutrient intake from food and supplements was compared with the Dietary Reference Intakes for 16 nutrients and outcomes influenced by nutritional status. Attention was given to vitamin D and calcium given potential skeletal implications due to cystic fibrosis. Measurements included weight, height, body composition, pulmonary function, and serum metabolic parameters. Participants were interviewed about dietary intake, supplement use, pulmonary function, sunlight exposure, and pain. The participants’ mean body mass index (±standard deviation) was 21.8±4.9 and pulmonary function tests were normal. Seventy-eight percent used pancreatic enzyme replacement for malabsorption. Vitamin D deficiency [25-hydroxyvitamin D (25OHD)<37.5 nmol/L] was common: 25 (39%) were deficient despite adequate vitamin D intake. Lipid profiles were normal in the majority, even though total and saturated fat consumption represented 33.0% and 16.8% of energy intake, respectively. Reported protein intake represented 16.9% of total energy intake (range 10%–25%). For several nutrients, including vitamin D and calcium, intake from food and supplements in many participants exceeded recommended Tolerable Upper Intake Levels. Among adults with cystic fibrosis, vitamin D deficiency was common despite reported adequate intake, and lipid profiles were normal despite a relatively high fat intake. Mean protein consumption was adequate, but the range of intake was concerning, as both inadequate or excessive intake may have deleterious skeletal effects. These findings call into question the applicability of established nutrient thresholds for patients with cystic fibrosis. PMID:18060897

Mucocele of the cystic duct remnant after orthotopic liver transplant: a problem revisited.

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Chatterjee, Suvadip; Das, Debasish; Hudson, Mark; Bassendine, Margaret Fiona; Scott, John; Oppong, Kofi Ernest; Sen, Gourab; French, Jeremy J

2011-06-01

Mucocele of the cystic duct remnant is an uncommon hepatobiliary complication of a liver transplant. Current practice usually involves either excising the cystic duct, or incorporating the distal end of the transected cystic duct into the suture line of the biliary anastomosis to ensure drainage. We report a patient who developed cystic duct remnant mucocele after the latter approach was adopted. We believe that this is likely related to delayed anastomotic stricturing, which prevented draining from the remnant cystic duct. We also discuss the incidence, pathology, investigations, and treatment of this condition.

Episodic seasonal Pseudo-Bartter syndrome in cystic fibrosis.

Science.gov (United States)

Kintu, Brett; Brightwell, Alex

2014-06-01

Pseudo-Bartter syndrome (PBS) describes an uncommon but well recognised complication of cystic fibrosis leading to hypochloraemic, hypokalaemic metabolic alkalosis. Pseudo-Bartter syndrome is usually seen at initial presentation or within the first two years of life in children with cystic fibrosis. Risk factors for development of PBS include warm weather conditions, severe respiratory or pancreatic disease and gastrointestinal losses (e.g. vomiting and diarrhoea). PBS is rare in older children and adolescents although epidemics have been associated with heat wave conditions in warmer climates. In this era of climate change, it is crucial that clinicians consider Pseudo-Bartter syndrome when patients with cystic fibrosis present unwell during summer. Copyright © 2014 Elsevier Ltd. All rights reserved.

Congenital cystic adenomatoid lung malformation of newborn

International Nuclear Information System (INIS)

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

1980-01-01

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

Cystic change in pulmonary tuberculosis in an immunocompetent adult: a case report

International Nuclear Information System (INIS)

Ko, Sung Min; Seo, Soo Ji; Choi, Won Il; Jeon, Young June

2008-01-01

Cystic change associated with pulmonary tuberculosis is rarely encountered, and few reports are available on the radiologic findings of pulmonary tuberculosis presenting as multiple cystic lesions associated with consolidation or bronchohematogenous nodules. The cystic lesions in our pulmonary tuberculosis patient occurred during steroid treatment without antituberculous chemotherapy and progressively increased in size, but subsequently became smaller after the initiation of antituberculous chemotherapy. Herein, we report the chest radiographic and computed tomographic findings of cystic change in pulmonary tuberculosis in an immunocompetent adult

Cystic form of rheumatoid arthritis

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Dijkstra, P.F.; Gubler, F.M.; Maas, A.

1988-10-01

A nonerosive form of rheumatoid arthritis (R.A.) was found in 62 patients out of 660 patients with R.A.. These 62 patients exhibit slowly progressive cystic changes in about the same joints in which usually erosions develop in classic R.A.. The E.S.R. is often low, half of the patients remained seronegative and there are 35 males and 27 females in the group. A smaller group of 15 out of these patients could be followed from a stage wherein the radiographs were normal to a stage of extensive cystic changes, over a period of at least 6 years. An attempt is made to delineate this group within the rheumatoid arthritis disease entity.

[Historical compilation of cystic fibrosis].

Science.gov (United States)

Navarro, Salvador

2016-01-01

Cystic fibrosis is the most common life-shortening recessively inherited disorder in the Caucasian population. The genetic mutation that most frequently provokes cystic fibrosis (ΔF508) appeared at least 53,000years ago. For many centuries, the disease was thought to be related to witchcraft and the "evil eye" and it was only in 1938 that Dorothy H. Andersen characterized this disorder and suspected its genetic origin. The present article reviews the pathological discoveries and diagnostic and therapeutic advances made in the last 75 years. The review ends with some considerations for the future. Copyright © 2015 Elsevier España, S.L.U. and AEEH y AEG. All rights reserved.

Cystic adventitial degeneration: ectopic ganglia from adjacent joint capsules.

Science.gov (United States)

Ortmann, J; Widmer, M K; Gretener, S; Do, D D; Willenberg, T; Daliri, A; Baumgartner, I

2009-11-01

Cystic adventitial degeneration is a rare non-atherosclerotic cause of peripheral arterial occlusive disease, mainly seen in young men without other evidence of vascular disease. Diagnosis will be established by clinical findings and by ultrasound or angiography and can be treated by excision or enucleation of the affected arterial segment or by percutaneous ultrasound-guided aspiration. However, the etiology of adventitial cysts remains unknown. We report a case of cystic adventitial degeneration showing a connection between the joint capsule and the adventitial cyst, supporting the theory that cystic adventitial degeneration may represent ectopic ganglia from adjacent joint capsules.

Predicting the intrauterine fetal death of fetuses with cystic hygroma in early pregnancy.

Science.gov (United States)

Shimura, Mai; Ishikawa, Hiroshi; Nagase, Hiromi; Mochizuki, Akihiko; Sekiguchi, Futoshi; Koshimizu, Naho; Itai, Toshiyuki; Odagami, Mizuha

2018-01-11

We investigated whether it was possible to predict the prognosis of fetuses with cystic hygroma in early pregnancy based on the degree of neck thickening. We retrospectively analyzed 57 singleton pregnancies with fetuses with cystic hygroma who were examined before the 22nd week of pregnancy. The fetuses were categorized according to the outcome, structural abnormalities at birth, and chromosomal abnormalities. Here, we proposed a new sonographic predictor with which we assessed neck thickening by dividing the width of the neck thickening by the biparietal diameter, which is expressed as the cystic hygroma width/biparietal diameter ratio. The median cystic hygroma width/biparietal diameter ratio in the intrauterine fetal death group (0.51) was significantly higher than that in the live birth group (0.27). No significant difference in the median cystic hygroma width/biparietal diameter ratio was found between the structural abnormalities group at birth and the no structural abnormalities group, and no significant difference in the median cystic hygroma width/biparietal diameter ratio was found between the chromosomal abnormality group and the no chromosomal abnormality group. We used receiver operating characteristic analysis to evaluate the cystic hygroma width/biparietal diameter ratio to predict intrauterine fetal death. When the cystic hygroma width/biparietal diameter ratio cut-off value was 0.5, intrauterine fetal death could be predicted with a sensitivity of 52.9% and a specificity of 100%. It is possible to predict intrauterine fetal death in fetuses with cystic hygroma in early pregnancy if cystic hygroma width/biparietal diameter ratio is measured. However, even if cystic hygroma width/biparietal diameter ratio is measured, predicting the presence or absence of a structural abnormality at birth or a chromosomal abnormality is difficult. © 2018 Japanese Teratology Society.

"Cystic fibrotics could survive cholera, choleraics could survive cystic fibrosis"; hypothesis that explores new horizons in treatment of cystic fibrosis.

Science.gov (United States)

Azimi, Arsalan

2015-12-01

Cystic fibrosis, the most common inherited disease of white population, is a disease of CFTR channels, in which mucosal function of many organs especially respiratory tract is impaired. Decreased mucociliary clearance and accumulation of mucus in airways facilitates colonization of infectious microorganisms, followed by infection. Following chronic infection, persistent inflammation ensues, which results in airway remodeling and deterioration of mucociliary clearance and result in a vicious cycle. Here, it is hypothesized that cholera toxin (CT) could ameliorate symptoms of cystic fibrosis as CT could dilute the thickened mucus, improve mucociliary clearance and alleviate airway obstruction. CT strengthens immunity of airway mucosa and it could attenuates bacterial growth and reduce persistency of infection. CT also modulates cellular immune response and it could decrease airway inflammation, hinder airway remodeling and prevent respiratory deterioration. Thereby it is hypothesized that CT could target and ameliorate many of pathophysiologic steps of the disease and it explores new horizons in treatment of CF. Copyright © 2015 Elsevier Ltd. All rights reserved.

Gamma Knife Radiosurgery for the Treatment of Cystic Cerebral Metastases

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Ebinu, Julius O.; Lwu, Shelly; Monsalves, Eric; Arayee, Mandana [Division of Neurosurgery, Department of Surgery, Toronto Western Hospital, Toronto, Ontario (Canada); Chung, Caroline; Laperriere, Normand J. [Department of Radiation Oncology, Princess Margaret Hospital, University Health Network, University of Toronto, Toronto, Ontario (Canada); Kulkarni, Abhaya V.; Goetz, Pablo [Division of Neurosurgery, Department of Surgery, The Hospital for Sick Children, University of Toronto, Toronto, Ontario (Canada); Zadeh, Gelareh, E-mail: gelareh.zadeh@uhn.on.ca [Division of Neurosurgery, Department of Surgery, Toronto Western Hospital, Toronto, Ontario (Canada)

2013-03-01

Purpose: To assess the role of Gamma Knife radiosurgery (GKRS) in the treatment of nonsurgical cystic brain metastasis, and to determine predictors of response to GKRS. Methods: We reviewed a prospectively maintained database of brain metastases patients treated at our institution between 2006 and 2010. All lesions with a cystic component were identified, and volumetric analysis was done to measure percentage of cystic volume on day of treatment and consecutive follow-up MRI scans. Clinical, radiologic, and dosimetry parameters were reviewed to establish the overall response of cystic metastases to GKRS as well as identify potential predictive factors of response. Results: A total of 111 lesions in 73 patients were analyzed; 57% of lesions received prior whole-brain radiation therapy (WBRT). Lung carcinoma was the primary cancer in 51% of patients, 10% breast, 10% colorectal, 4% melanoma, and 26% other. Fifty-seven percent of the patients were recursive partitioning analysis class 1, the remainder class 2. Mean target volume was 3.3 mL (range, 0.1-23 mL). Median prescription dose was 21 Gy (range, 15-24 Gy). Local control rates were 91%, 63%, and 37% at 6, 12, and 18 months, respectively. Local control was improved in lung primary and worse in patients with prior WBRT (univariate). Only lung primary predicted local control in multivariate analysis, whereas age and tumor volume did not. Lesions with a large cystic component did not show a poorer response compared with those with a small cystic component. Conclusions: This study supports the use of GKRS in the management of nonsurgical cystic metastases, despite a traditionally perceived poorer response. Our local control rates are comparable to a matched cohort of noncystic brain metastases, and therefore the presence of a large cystic component should not deter the use of GKRS. Predictors of response included tumor subtype. Prior WBRT decreased effectiveness of SRS for local control rates.

Gamma Knife Radiosurgery for the Treatment of Cystic Cerebral Metastases

International Nuclear Information System (INIS)

Ebinu, Julius O.; Lwu, Shelly; Monsalves, Eric; Arayee, Mandana; Chung, Caroline; Laperriere, Normand J.; Kulkarni, Abhaya V.; Goetz, Pablo; Zadeh, Gelareh

2013-01-01

Purpose: To assess the role of Gamma Knife radiosurgery (GKRS) in the treatment of nonsurgical cystic brain metastasis, and to determine predictors of response to GKRS. Methods: We reviewed a prospectively maintained database of brain metastases patients treated at our institution between 2006 and 2010. All lesions with a cystic component were identified, and volumetric analysis was done to measure percentage of cystic volume on day of treatment and consecutive follow-up MRI scans. Clinical, radiologic, and dosimetry parameters were reviewed to establish the overall response of cystic metastases to GKRS as well as identify potential predictive factors of response. Results: A total of 111 lesions in 73 patients were analyzed; 57% of lesions received prior whole-brain radiation therapy (WBRT). Lung carcinoma was the primary cancer in 51% of patients, 10% breast, 10% colorectal, 4% melanoma, and 26% other. Fifty-seven percent of the patients were recursive partitioning analysis class 1, the remainder class 2. Mean target volume was 3.3 mL (range, 0.1-23 mL). Median prescription dose was 21 Gy (range, 15-24 Gy). Local control rates were 91%, 63%, and 37% at 6, 12, and 18 months, respectively. Local control was improved in lung primary and worse in patients with prior WBRT (univariate). Only lung primary predicted local control in multivariate analysis, whereas age and tumor volume did not. Lesions with a large cystic component did not show a poorer response compared with those with a small cystic component. Conclusions: This study supports the use of GKRS in the management of nonsurgical cystic metastases, despite a traditionally perceived poorer response. Our local control rates are comparable to a matched cohort of noncystic brain metastases, and therefore the presence of a large cystic component should not deter the use of GKRS. Predictors of response included tumor subtype. Prior WBRT decreased effectiveness of SRS for local control rates

Cystic adventitial disease of popliteal artery with significant stenosis

International Nuclear Information System (INIS)

Gupta, Ranjana; Mittal, Puneet; Gupta, Praveen; Jindal, Nancy

2013-01-01

Cystic adventitial disease of popliteal artery is a rare condition of unknown etiology which usually presents in middle-aged men. We present Doppler and computed tomography angiography findings in a case of cystic adventitial disease with significant obstruction of popliteal artery, with secondary narrowing of popliteal vein

Breakthrough Therapies: Cystic Fibrosis (CF) Potentiators and Correctors

Science.gov (United States)

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

2015-01-01

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 is also emphasized. PMID:26097168

MR differentiation of cystic lesions in the maxillomandibular region

International Nuclear Information System (INIS)

Minami, M.; Kaneda, T.; Ozawa, K.; Ozawa, M.; Itaz, Y.; Sasaki, Y.

1991-01-01

This paper examines the capability of MR imaging in differentiating cystic lesions in the maxillomandibular region. MR imaging of 34 patients with cystic lesions in the maxillomandibular region was performed prospectively. After T1-weighted axial imaging, T1-weighted axial and sagittal/coronal imaging with Gd-DTPA and T2-weighted axial imaging was performed. The pathologic examinations of the cases showed 9 ameloblastomas, 13 odontogenic keratocysts, and 11 other types of cysts. MR differentiation between ameloblastoma and cysts was possible in all cases but one. MR images of ameloblastoma showed solid and cystic components, multilocularity, irregularly thickened walls, mural nodules, marked Gd-DTPA enhancement of the wall, and low T1-weighted signal fluids. MR differentiation between various kinds of cysts was difficult without information from conventional radiography, except odontogenic keratocysts. MR images of 17 keratocysts showed cystic patterns, regularly thin walls monolocular cysts, weak Gd-DTPA enhancement of the walls, and inhomogeneous intensity of the contents

Different manifestations of calcifying cystic odontogenic tumor

Directory of Open Access Journals (Sweden)

Estevam Rubens Utumi

2012-09-01

Full Text Available The calcifying cystic odontogenic tumor normally presents as apainless, slow-growing mass, involving both maxilla and mandible,primarily the anterior segment (incisor/canine area. It generallyaffects young adults in the third to fourth decades, with no genderpredilection. Computerized tomography images revealed importantcharacteristics that were not detected by panoramic radiography,such as fenestration, calcification and tooth-like structures. Thetypical microscopic feature of this lesion is the presence of variableamounts of aberrant epithelial cells, without nuclei, which arenamed “ghost cells”. In addition, dysplastic dentine can be foundand occasionally the cyst can be associated with an area of dentalhard tissue formation resembling an odontoma. The treatment forcalcifying cystic odontogenic tumor involves simple enucleationand curettage. The purpose of this article is to present two differentmanifestation of calcifying cystic odontogenic tumor in whichcomputerized tomography, associated to clinical features, servedas an important tool for diagnosis, adequate surgical planning andfollow-up of patients.

[Cystic fibrosis--initial diagnosis in a 39-year-old patient].

Science.gov (United States)

Bargon, J; Rickmann, J; Jacobi, V; Straub, R; Arnemann, J; Wagner, T O

2000-12-15

Cystic fibrosis is the most common hereditary disorder among Caucasians. Most of the patients are diagnosed as children. However, some cases are going undiagnosed into adulthood and are then often misdiagnosed because the non-pediatricians do not know cystic fibrosis very well and do not consider this diagnosis in adult patients. We present the medical history of a woman, who was diagnosed with cystic fibrosis at the age of 39 years, although she had suffered from bronchiectasis, pancreatic insufficiency and liver cirrhosis since many years. Her medical history was long with some diagnosis, but because of her age nobody considered the final diagnosis. In adult patients with bronchiectasis, liver cirrhosis and pancreatic insufficiency in combination or with only one of these symptoms, cystic fibrosis should be included into the differential diagnosis.

Recent progress in translational cystic fibrosis research using precision medicine strategies.

Science.gov (United States)

Cholon, Deborah M; Gentzsch, Martina

2018-03-01

Significant progress has been achieved in developing precision therapies for cystic fibrosis; however, highly effective treatments that target the ion channel, CFTR, are not yet available for many patients. As numerous CFTR therapeutics are currently in the clinical pipeline, reliable screening tools capable of predicting drug efficacy to support individualized treatment plans and translational research are essential. The utilization of bronchial, nasal, and rectal tissues from individual cystic fibrosis patients for drug testing using in vitro assays such as electrophysiological measurements of CFTR activity and evaluation of fluid movement in spheroid cultures, has advanced the prediction of patient-specific responses. However, for precise prediction of drug effects, in vitro models of CFTR rescue should incorporate the inflamed cystic fibrosis airway environment and mimic the complex tissue structures of airway epithelia. Furthermore, novel assays that monitor other aspects of successful CFTR rescue such as restoration of mucus characteristics, which is important for predicting mucociliary clearance, will allow for better prognoses of successful therapies in vivo. Additional cystic fibrosis treatment strategies are being intensively explored, such as development of drugs that target other ion channels, and novel technologies including pluripotent stem cells, gene therapy, and gene editing. The multiple therapeutic approaches available to treat the basic defect in cystic fibrosis combined with relevant precision medicine models provide a framework for identifying optimal and sustained treatments that will benefit all cystic fibrosis patients. Copyright © 2017 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.

Cystic angiomatosis with splenic involvement: unusual MRI findings

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Vanhoenacker, F.M. [Dept. of Radiology, Univ. Hospital Antwerp, Edegem (Belgium); Dept. of Radiology, AZ St-Maarten, Campus Duffel, Duffel (Belgium); Schepper, A.M. [Dept. of Radiology, Univ. Hospital Antwerp, Edegem (Belgium); Raeve, H. [Dept. of Pathology, Univ. Hospital Antwerp, Edegem (Belgium); Berneman, Z. [Dept. of Hematology, Univ. Hospital Antwerp, Edegem (Belgium)

2003-12-01

Cystic angiomatosis is a rare disorder with a poor prognosis. We describe a case of a 33-year-old woman who presented with longstanding bone pain, hemolytic anemia, and an enlarged spleen. Radiologically, multiple osseous lesions with a mixed pattern of lytic and sclerotic areas were seen within the shoulders, spine, and pelvis. On CT and MRI of the abdomen, the spleen was markedly enlarged, with internal hyperdense foci on non-contrast CT scan, corresponding to low signal intensity areas on all MR pulse sequences. After administration of contrast, a mottled enhancement pattern throughout the entire spleen was seen both on CT and MRI. Cystic angiomatosis was proven by histological analysis of a biopsy specimen of an involved vertebra and histopathological examination of the spleen after subsequent splenectomy. This is the first report of a patient with disseminated cystic angiomatosis with splenic involvement in which the MRI features differ from the previous reports. Instead of the usual pattern consisting of multiple well-defined cystic lesions, a diffuse involvement replacing the entire spleen, with heterogeneous signal intensities on T2-weighted images and heterogeneous enhancement pattern, was seen in our patient. (orig.)

Cystic angiomatosis with splenic involvement: unusual MRI findings

International Nuclear Information System (INIS)

Vanhoenacker, F.M.; Schepper, A.M.; Raeve, H.; Berneman, Z.

2003-01-01

Cystic angiomatosis is a rare disorder with a poor prognosis. We describe a case of a 33-year-old woman who presented with longstanding bone pain, hemolytic anemia, and an enlarged spleen. Radiologically, multiple osseous lesions with a mixed pattern of lytic and sclerotic areas were seen within the shoulders, spine, and pelvis. On CT and MRI of the abdomen, the spleen was markedly enlarged, with internal hyperdense foci on non-contrast CT scan, corresponding to low signal intensity areas on all MR pulse sequences. After administration of contrast, a mottled enhancement pattern throughout the entire spleen was seen both on CT and MRI. Cystic angiomatosis was proven by histological analysis of a biopsy specimen of an involved vertebra and histopathological examination of the spleen after subsequent splenectomy. This is the first report of a patient with disseminated cystic angiomatosis with splenic involvement in which the MRI features differ from the previous reports. Instead of the usual pattern consisting of multiple well-defined cystic lesions, a diffuse involvement replacing the entire spleen, with heterogeneous signal intensities on T2-weighted images and heterogeneous enhancement pattern, was seen in our patient. (orig.)

The Role of Computed Tomography in Monitoring Patients with Cystic Fibrosis

International Nuclear Information System (INIS)

Rybacka, Anna; Karmelita-Katulska, Katarzyna

2016-01-01

Cystic fibrosis is the most common lethal autosomal recessive disorder in the Caucasian population. Although the survival rate in patients constantly improves, lung damage is still the major cause of morbidity and mortality in patients with cystic fibrosis. In clinical practice, evaluation of patients’ pulmonary state is made by combination of monitoring of lung function and more directly by assessing the lung structure in imaging studies. Studies showed that computed tomography findings are more sensitive as compared to the pulmonary function tests. Computed tomography can identify a wide range of morphological abnormalities in patients with cystic fibrosis, such as bronchiectasis (which is progressive, irreversible and probably the most relevant structural change in cystic fibrosis) peribronchial thickening, mucous plugging and many other disorders that occur in the course of the disease. Computed tomography has a crucial role in the assessment of pulmonary damage over time, detecting complications and monitoring treatment effects in patients with cystic fibrosis

Diagnostic and radiological management of cystic pancreatic lesions: Important features for radiologists

International Nuclear Information System (INIS)

Buerke, B.; Domagk, D.; Heindel, W.; Wessling, J.

2012-01-01

Cystic pancreatic neoplasms are often an incidental finding, the frequency of which is increasing. The understanding of such lesions has increased in recent years, but the numerous types of lesions involved can hinder differential diagnosis. They include, in particular, intraductal papillary mucinous neoplasms (IPMN), serous cystic neoplasms (SCN), and mucinous cystic neoplasms (MCN). Knowledge of their histological and radiological structure, as well as distribution in terms of localization, age, and sex, helps to differentiate such tumours from common pancreatic pseudocysts. Several types of cystic pancreatic neoplasms can undergo malignant transformation and, therefore, require differentiated radiological management. This review aims to develop a broader understanding of the pathological and radiological characteristics of cystic pancreatic neoplasms, and provide a guideline for everyday practice based on current concepts in the radiological management of the given lesions.

Conservative Treatment for Cystic Duct Stenosis in a Child

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Marco Gasparetto

2013-01-01

Full Text Available Introduction. Few cases of common bile duct stenosis have been reported in the literature, and observations of strictures in the cystic duct are even more rare. Surgical cholecystectomy is the treatment needed in most cases of gallbladder hydrops. This paper describes the diagnosis and successful medical treatment of a rare pediatric case of cystic duct stenosis and gallbladder hydrops. Case Report. A formerly healthy one-year-old girl was admitted with colicky abdominal pain. Blood tests were normal, except for an increase in transaminases. Abdominal ultrasound excluded intestinal intussusception and identified a distended gallbladder with biliary sludge. MR cholangiography revealed a dilated gallbladder containing bile sediment and no detectable cystic duct, while the rest of the intra- and extrahepatic biliary tree and hepatic parenchyma were normal. This evidence was consistent with gallbladder hydrops associated with cystic duct stenosis. The baby was treated with i.v. hydration, corticosteroids, antibiotics, and ursodeoxycholic acid. Her general condition rapidly improved, with no further episodes of abdominal pain and normalization of liver enzymes. This allowed to avoid cholecystectomy, and the child is well 1.5 years after diagnosis. Conclusions. Although cholecystectomy is usually necessary in case of gallbladder hydrops, our experience suggests that surgical procedures can be avoided when the distension is caused by a cystic duct stenosis.

Lung function imaging methods in Cystic Fibrosis pulmonary disease.

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Kołodziej, Magdalena; de Veer, Michael J; Cholewa, Marian; Egan, Gary F; Thompson, Bruce R

2017-05-17

Monitoring of pulmonary physiology is fundamental to the clinical management of patients with Cystic Fibrosis. The current standard clinical practise uses spirometry to assess lung function which delivers a clinically relevant functional readout of total lung function, however does not supply any visible or localised information. High Resolution Computed Tomography (HRCT) is a well-established current 'gold standard' method for monitoring lung anatomical changes in Cystic Fibrosis patients. HRCT provides excellent morphological information, however, the X-ray radiation dose can become significant if multiple scans are required to monitor chronic diseases such as cystic fibrosis. X-ray phase-contrast imaging is another emerging X-ray based methodology for Cystic Fibrosis lung assessment which provides dynamic morphological and functional information, albeit with even higher X-ray doses than HRCT. Magnetic Resonance Imaging (MRI) is a non-ionising radiation imaging method that is garnering growing interest among researchers and clinicians working with Cystic Fibrosis patients. Recent advances in MRI have opened up the possibilities to observe lung function in real time to potentially allow sensitive and accurate assessment of disease progression. The use of hyperpolarized gas or non-contrast enhanced MRI can be tailored to clinical needs. While MRI offers significant promise it still suffers from poor spatial resolution and the development of an objective scoring system especially for ventilation assessment.

Biopsy results of Bosniak 2F and 3 cystic lesions

DEFF Research Database (Denmark)

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

be helpful in clinical decisions. Material and Methods: From March 2013 - December 2014 a total of 295 percutaneous ultrasound guided biopsies from 287 patients with a suspected malignant renal lesion were performed at our institution. All cases were reviewed in PACS by (RR) and lesions presenting...... 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 pathologically...... analyzed and three (17%) proved to be malignant. Biopsies from seventeen Bosniak 3 cystic lesions were pathologically analyzed and five (29%) were found to be malignant. Conclusion: Our results reveal a considerable malignancy rate among both Bosniak 2F and 3 cystic renal lesions. Biopsy seems...

[Treatment of autonomous and cystic thyroid nodules with intranodular ethanol injection].

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Braga-Basaria, Milena; Trippia, Marcus Adriano; Stolf, Anderson Ravy; Mesa, Cléo; Graf, Hans

2002-01-01

Intranodular ethanol injection has been used for the past 10 years as an efficient modality for treating patients with thyroid nodules. Several studies have reported the success of this therapy in autonomous and cystic nodules and, more recently, in cold benign nodules. To evaluate the efficacy of this therapeutic modality on the treatment of autonomous and cystic thyroid nodules. 42 patients (26 with cystic and 16 with autonomous nodules) were treated with ultrasound guided intranodular 99% ethanol injection and followed for 6 months. No major complications were observed during or after treatment, however, most of the patients reported slight to moderate pain and/or discomfort after the injection. Most of the nodules showed reduction after the treatment. Autonomous nodules had a mean reduction of 50.3% and cystic nodules of 69.3%. No significant differences in pretreatment serum total T3, total T4 or TSH were observed among the patients in the cystic group. Patients in the autonomous group with hyperfunctioning nodules showed a decrease in serum total T3, total T4 and an increase in serum TSH levels, hence, proving the effectiveness of this therapy. Intranodular ethanol injection is a safe and efficient treatment for autonomous and cystic nodules of the thyroid.

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.

Round Ligament Varicosities During Pregnancy: Case Report

International Nuclear Information System (INIS)

Baek, Chang Kyu; Oh, Young Taik; Jung, Dae Chul

2011-01-01

There are various causes of a painful palpable mass in the groin during pregnancy. The differential diagnoses of an inguinal mass include hernia, lymphadenopathy, mesothelial cyst, cystic lymphangioma, neoplasms (lipoma, leiomyoma and sarcoma), endometriosis, embryonic remnants and round ligament varicosities. Among them, round ligament varicosities can be easily misdiagnosed as an inguinal hernia in a pregnant woman. These lesions should be managed conservatively because they resolve spontaneously during the postpartum period. Ultrasonography can help make the diagnosis of round ligament varicosities and so prevent unnecessary surgical intervention and the associated morbidity. Herein we report on a case of round ligament varicosities that presented during pregnancy and this condition was readily diagnosed via Doppler sonography

Round Ligament Varicosities During Pregnancy: Case Report

Energy Technology Data Exchange (ETDEWEB)

Baek, Chang Kyu; Oh, Young Taik; Jung, Dae Chul [Yonsei University Health System, Seoul (Korea, Republic of)

2011-12-15

There are various causes of a painful palpable mass in the groin during pregnancy. The differential diagnoses of an inguinal mass include hernia, lymphadenopathy, mesothelial cyst, cystic lymphangioma, neoplasms (lipoma, leiomyoma and sarcoma), endometriosis, embryonic remnants and round ligament varicosities. Among them, round ligament varicosities can be easily misdiagnosed as an inguinal hernia in a pregnant woman. These lesions should be managed conservatively because they resolve spontaneously during the postpartum period. Ultrasonography can help make the diagnosis of round ligament varicosities and so prevent unnecessary surgical intervention and the associated morbidity. Herein we report on a case of round ligament varicosities that presented during pregnancy and this condition was readily diagnosed via Doppler sonography

Cystic fibrosis: a mucosal immunodeficiency syndrome

Science.gov (United States)

Cohen, Taylor Sitarik; Prince, Alice

2013-01-01

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

An atypical presentation of cystic fibrosis: a case report

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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.

Adult-onset cystic hygroma: A case report of rare entity.

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Bahl, Sumit; Shah, Vandana; Anchlia, Sonal; Vyas, Siddharth

2016-01-01

Cystic hygroma is a benign congenital malformation of the lymphatic system that occurs in infant or children younger than 2 years of age. Although cystic hygroma is well recognized in pediatric practice, it seldom presents de novo in adulthood. These are commonly present in head and neck but can be present anywhere. Cystic hygroma is very rare in adults, but it should be considered in the differential diagnosis of adult neck swellings. Patients presenting with a painless, soft, fluctuant, and enlarging neck mass should have a careful history and physical examination along with radiological imaging to assist with diagnosis. Surgical intervention is the treatment of choice for this rare condition. Here, we are reporting a case of cystic hygroma in a 32-year-old male patient in the neck region. The objectives of this case report are to discuss the clinical presentation, diagnosis, histopathological findings and management of this malformation.

Cystic degeneration of liver malignancies. Study by US and CT

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Kumada, Takashi; Nakano, Satoshi; Kitamura, Kimio; Watahiki, Hajime; Takeda, Isao

1983-03-01

CT and US were carried out on 81 patients with hepatocellular carcinoma, 20 patients with cholangiocellular carcinoma and 94 patients with metastatic liver cancer. 1) Cystic degeneration was observed in one with hepatocellular carcinoma (1.2%), one with cholangiocellular carcinoma (5.0%) and 12 with metastatic liver cancer (12.8%) by US, but this change was observed in only 5 by CT (1,0,4, respectively). Metastatic liver cancer showed the highest incidence among these tumors. 2) The characteristics of cystic degeneration of the liver tumors were thickened wall and irregularity of the inner surface of the wall. 3) Judging from macroscopic and histopathological findings, liquefactive necrosis in the tumors was shown as ''echoluent'' area. We concluded that cystic degeneration was one of the important findings in metastatic liver cancer and that careful observation by US and CT avoided the confusion with other hepatic cystic diseases.

Respiratory muscle training for cystic fibrosis.

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Hilton, Nathan; Solis-Moya, Arturo

2018-05-24

Cystic fibrosis is the most common autosomal recessive disease in white populations, and causes respiratory dysfunction in the majority of individuals. Numerous types of respiratory muscle training to improve respiratory function and health-related quality of life in people with cystic fibrosis have been reported in the literature. Hence a systematic review of the literature is needed to establish the effectiveness of respiratory muscle training (either inspiratory or expiratory muscle training) on clinical outcomes in cystic fibrosis. This is an update of a previously published review. To determine the effectiveness of respiratory muscle training on clinical outcomes in people with cystic fibrosis. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials register comprising of references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings.Date of most recent search: 17 April 2018.A hand search of the Journal of Cystic Fibrosis and Pediatric Pulmonology was performed, along with an electronic search of online trial databases up until 07 May 2018. Randomised controlled studies comparing respiratory muscle training with a control group in people with cystic fibrosis. Review authors independently selected articles for inclusion, evaluated the methodological quality of the studies, and extracted data. Additional information was sought from trial authors where necessary. The quality of the evidence was assessed using the GRADE system MAIN RESULTS: Authors identified 19 studies, of which nine studies with 202 participants met the review's inclusion criteria. There was wide variation in the methodological and written quality of the included studies. Four of the nine included studies were published as abstracts only and lacking concise details, thus limiting the information available. Seven studies were parallel studies and two of a cross-over design. Respiratory

Abdominal Cystic Echinococcosis Treated with Albendazole. A Pediatric Cohort Study.

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Samanta Moroni

Full Text Available Cystic echinococcosis is endemic in Argentina. The standard pharmacological treatment for the disease is albendazole, but surgery is a common alternative. Even though primary infection occurs mainly in the pediatric population, the optimal therapeutic option in pediatrics is not clearly defined and few pediatric cohorts with cystic echinococcosis treated with albendazole have been described to date.To describe therapeutic response to albendazole in a cohort of pediatric patients with abdominal cystic echinococcosis.Patients (0-18 years old with abdominal cystic echinococcosis who were treated with albendazole between January 1998 and August 2013. Diagnosis of abdominal cystic echinococcosis was made by ultrasound. All patients received albendazole, 10-15 mg/kg/day. Epidemiological data, symptoms, number, location and outcome of the cysts, serology and treatment received were analyzed. The parameter used to assess treatment response was cyst changes evaluated by ultrasound follow up using the WHO-IWGE classification.A total of 28 patients (with 46 abdominal cysts were included in the cohort. Mean age at enrolment was 9.4 years and mean duration of follow-up, 23.8 months. All patients resided in rural areas and had had contact with dogs. The asymptomatic form of the disease was the most common presentation. All patients received albendazole (mean duration: 142.5 days, with low incidence of adverse events. Albendazole had a positive effect on most of the cysts. Surgery was performed in 13 patients.Treatment with albendazole for uncomplicated cystic echinococcosis cysts is safe and effective, and can potentially reduce the need for surgical intervention.

Abdominal Cystic Echinococcosis Treated with Albendazole. A Pediatric Cohort Study.

Science.gov (United States)

Moroni, Samanta; Moscatelli, Guillermo; Bournissen, Facundo García; González, Nicolás; Ballering, Griselda; Freilij, Héctor; Salgueiro, Fabián; Altcheh, Jaime

2016-01-01

Cystic echinococcosis is endemic in Argentina. The standard pharmacological treatment for the disease is albendazole, but surgery is a common alternative. Even though primary infection occurs mainly in the pediatric population, the optimal therapeutic option in pediatrics is not clearly defined and few pediatric cohorts with cystic echinococcosis treated with albendazole have been described to date. To describe therapeutic response to albendazole in a cohort of pediatric patients with abdominal cystic echinococcosis. Patients (0-18 years old) with abdominal cystic echinococcosis who were treated with albendazole between January 1998 and August 2013. Diagnosis of abdominal cystic echinococcosis was made by ultrasound. All patients received albendazole, 10-15 mg/kg/day. Epidemiological data, symptoms, number, location and outcome of the cysts, serology and treatment received were analyzed. The parameter used to assess treatment response was cyst changes evaluated by ultrasound follow up using the WHO-IWGE classification. A total of 28 patients (with 46 abdominal cysts) were included in the cohort. Mean age at enrolment was 9.4 years and mean duration of follow-up, 23.8 months. All patients resided in rural areas and had had contact with dogs. The asymptomatic form of the disease was the most common presentation. All patients received albendazole (mean duration: 142.5 days), with low incidence of adverse events. Albendazole had a positive effect on most of the cysts. Surgery was performed in 13 patients. Treatment with albendazole for uncomplicated cystic echinococcosis cysts is safe and effective, and can potentially reduce the need for surgical intervention.

Na and K dependence of the Na/K pump in cystic fibrosis fibroblasts.

OpenAIRE

Reznik, V M; Schneider, J A; Mendoza, S A

1981-01-01

The Na and K dependence of the Na/K pump was measured in skin fibroblasts from patients with cystic fibrosis and age/sex-matched controls. Under basal conditions, there was no difference between control and cystic fibrosis cells in protein per cell, intracellular Na and K content, or Na/K pump activity (measured as ouabain-sensitive 86Rb uptake). There was no difference in the Na dependence of the Na/K pump between cystic fibrosis cells and control cells. In cells from patients with cystic fi...

Cystic pulmonary hydatidosis

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Malay Sarkar

2016-01-01

Full Text Available 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.

Cost-Effectiveness of Screening Individuals With Cystic Fibrosis for Colorectal Cancer.

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Gini, Andrea; Zauber, Ann G; Cenin, Dayna R; Omidvari, Amir-Houshang; Hempstead, Sarah E; Fink, Aliza K; Lowenfels, Albert B; Lansdorp-Vogelaar, Iris

2017-12-27

Individuals with cystic fibrosis are at increased risk of colorectal cancer (CRC) compared to the general population, and risk is higher among those who received an organ transplant. We performed a cost-effectiveness analysis to determine optimal CRC screening strategies for patients with cystic fibrosis. We adjusted the existing Microsimulation Screening Analysis-Colon microsimulation model to reflect increased CRC risk and lower life expectancy in patients with cystic fibrosis. Modeling was performed separately for individuals who never received an organ transplant and patients who had received an organ transplant. We modeled 76 colonoscopy screening strategies that varied the age range and screening interval. The optimal screening strategy was determined based on a willingness to pay threshold of $100,000 per life-year gained. Sensitivity and supplementary analyses were performed, including fecal immunochemical test (FIT) as an alternative test, earlier ages of transplantation, and increased rates of colonoscopy complications, to assess whether optimal screening strategies would change. Colonoscopy every 5 years, starting at age 40 years, was the optimal colonoscopy strategy for patients with cystic fibrosis who never received an organ transplant; this strategy prevented 79% of deaths from CRC. Among patients with cystic fibrosis who had received an organ transplant, optimal colonoscopy screening should start at an age of 30 or 35 years, depending on the patient's age at time of transplantation. Annual FIT screening was predicted to be cost-effective for patients with cystic fibrosis. However, the level of accuracy of the FIT in population is not clear. Using a Microsimulation Screening Analysis-Colon microsimulation model, we found screening of patients with cystic fibrosis for CRC to be cost-effective. Due to the higher risk in these patients for CRC, screening should start at an earlier age with a shorter screening interval. The findings of this study

Variation in Cilia Protein Genes and Progression of Lung Disease in Cystic Fibrosis.

Science.gov (United States)

Blue, Elizabeth; Louie, Tin L; Chong, Jessica X; Hebbring, Scott J; Barnes, Kathleen C; Rafaels, Nicholas M; Knowles, Michael R; Gibson, Ronald L; Bamshad, Michael J; Emond, Mary J

2018-04-01

Cystic fibrosis, like primary ciliary dyskinesia, is an autosomal recessive disorder characterized by abnormal mucociliary clearance and obstructive lung disease. We hypothesized that genes underlying the development or function of cilia may modify lung disease severity in persons with cystic fibrosis. To test this hypothesis, we compared variants in 93 candidate genes in both upper and lower tertiles of lung function in a large cohort of children and adults with cystic fibrosis with those of a population control dataset. Variants within candidate genes were tested for association using the SKAT-O test, comparing cystic fibrosis cases defined by poor (n = 127) or preserved (n = 127) lung function with population controls (n = 3,269 or 3,148, respectively). Associated variants were then tested for association with related phenotypes in independent datasets. Variants in DNAH14 and DNAAF3 were associated with poor lung function in cystic fibrosis, whereas variants in DNAH14 and DNAH6 were associated with preserved lung function in cystic fibrosis. Associations between DNAH14 and lung function were replicated in disease-related phenotypes characterized by obstructive lung disease in adults. Genetic variants within DNAH6, DNAH14, and DNAAF3 are associated with variation in lung function among persons with cystic fibrosis.

The Radiologic Features of Cystic versus Noncystic Glioblastoma Multiforme as Significant Prognostic Factors

International Nuclear Information System (INIS)

Choi, Seung Joon; Hwang, Hee Young; Kim, Na Rae; Lee, Sheen Woo; Kim, Jeong Ho; Choi, Hye Young; Kim, Hyung Sik

2010-01-01

The purpose of this study was to determine the preoperative radiological characteristic and survival differences of glioblastoma multiforme (GBM) with and without cysts. Twenty-one GBMs were collected retrospectively; these tumors were pathologic confirmed as GBM. Based on the preoperative MR imaging, we compared the cystic GBMs with the noncystic GBMs according to the the tumor size, the tumor interface, the tumor wall thickness and peritumoral edema. Seven cases were classified as cystic GBMs and fourteen were noncystic GBMs. The cystic GBMs had a well-defined tumor interface, a less than 2 cm thickness of the tumor wall and less than 40 cm 3 thick peritumoral edema as compared to that of the noncystic GBMs. There was a statistically significant difference in age between the patients with cystic tumors and those with noncystic tumors. For the patients with cystic GBMs and noncystic GBMs, median survival time after surgery was 43.8 months and 12.5 months, respectively. The cystic GBMs had a well-defined tumor interface, a thin wall and minimal edema, as compared with that of the noncystic GBMs. The patients with cystic GBMs were significantly younger and they had more favorable survival outcomes than did the patients with noncystic GBMs

Complementary and alternative medicine use in children with cystic fibrosis.

Science.gov (United States)

Giangioppo, Sandra; Kalaci, Odion; Radhakrishnan, Arun; Fleischer, Erin; Itterman, Jennifer; Lyttle, Brian; Price, April; Radhakrishnan, Dhenuka

2016-11-01

To estimate the overall prevalence of complementary and alternative medicine use among children with cystic fibrosis, determine specific modalities used, predictors of use and subjective helpfulness or harm from individual modalities. Of 53 children attending the cystic fibrosis clinic in London, Ontario (100% recruitment), 79% had used complementary and alternative medicine. The most commonly used modalities were air purifiers, humidifiers, probiotics, and omega-3 fatty acids. Family complementary and alternative medicine use was the only independent predictor of overall use. The majority of patients perceived benefit from specific modalities for cystic fibrosis symptoms. Given the high frequency and number of modalities used and lack of patient and disease characteristics predicting use, we recommend that health care providers should routinely ask about complementary and alternative medicine among all pediatric cystic fibrosis patients and assist patients in understanding the potential benefits and risks to make informed decisions about its use. Copyright © 2016 Elsevier Ltd. All rights reserved.

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...

Oral calorie supplements for cystic fibrosis.

Science.gov (United States)

Smyth, Rosalind L; Rayner, Oli

2017-05-04

Poor nutrition occurs frequently in people with cystic fibrosis and is associated with other adverse outcomes. Oral calorie supplements are used to increase total daily calorie intake and improve weight gain. However, they are expensive and there are concerns they may reduce the amount of food eaten and not improve overall energy intake. This is an update of a previously published review. To establish whether in people with cystic fibrosis, oral calorie supplements: increase daily calorie intake; and improve overall nutritional intake, nutritional indices, lung function, survival and quality of life. To assess adverse effects associated with using these supplements. We searched the Cochrane Cystic Fibrosis Trials Register comprising references from comprehensive electronic database searches, handsearches of relevant journals and abstract books of conference proceedings. We contacted companies marketing oral calorie supplements.Last search: 18 October 2016. Randomised or quasi-randomised controlled trials comparing use of oral calorie supplements for at least one month to increase calorie intake with no specific intervention or additional nutritional advice in people with cystic fibrosis. We independently selected the included trials, assessed risk of bias and extracted data. We contacted the authors of included trials and obtained additional information for two trials. We identified 21 trials and included three, reporting results from 131 participants lasting between three months and one year. Two trials compared supplements to additional nutritional advice and one to no intervention. Two of the included trials recruited only children. In one trial the risk of bias was low across all domains, in a second trial the risk of bias was largely unclear and in the third mainly low. Blinding of participants was unclear in two of the trials. Also, in one trial the clinical condition of groups appeared to be unevenly balanced at baseline and in another trial there were

Preimplantation genetic diagnosis for cystic fibrosis: a case report

Science.gov (United States)

Biazotti, Maria Cristina Santoro; Pinto, Walter; de Albuquerque, Maria Cecília Romano Maciel; Fujihara, Litsuko Shimabukuro; Suganuma, Cláudia Haru; Reigota, Renata Bednar; Bertuzzo, Carmen Sílvia

2015-01-01

Cystic fibrosis is an autosomal recessive disorder caused by mutations in the cystic fibrosis transmembrane conductance regulator gene. This disorder produces a variable phenotype including lung disease, pancreatic insufficiency, and meconium ileus plus bilateral agenesis of the vas deferens causing obstructive azoospermia and male infertility. Preimplantation genetic diagnosis is an alternative that allows identification of embryos affected by this or other genetic diseases. We report a case of couple with cystic fibrosis; the woman had the I148 T mutation and the man had the Delta F508 gene mutation. The couple underwent in vitro fertilization, associated with preimplantation genetic diagnosis, and with subsequent selection of healthy embryos for uterine transfer. The result was an uneventful pregnancy and delivery of a healthy male baby. PMID:25993078

Parental care and overprotection of children with cystic fibrosis.

Science.gov (United States)

Cappelli, M; McGrath, P J; MacDonald, N E; Katsanis, J; Lascelles, M

1989-09-01

Parental overprotection has often been clinically associated with the psychological maladjustment of children with a chronic disease. The purpose of this study was to examine parental care and overprotection in children with cystic fibrosis compared to healthy controls. Results indicated no differences in the level of parental care or overprotection between controls and children with cystic fibrosis. However, a number of significant correlations were found between parental care and overprotection and children's psychosocial functioning. In particular, positive correlations were found between parental overprotection and poor psychosocial functioning in children with cystic fibrosis, whereas, poor psychosocial functioning in healthy children was associated with lack of parental care. Parental overprotection and care appear to play important roles in the emotional and psychological functioning of healthy and chronically ill children.

Young patients with cystic fibrosis demonstrate subtle alterations of the cardiovascular system.

Science.gov (United States)

Eising, Jacobien B; van der Ent, Cornelis K; Teske, Arco J; Vanderschuren, Maaike M; Uiterwaal, Cuno S P M; Meijboom, Folkert J

2018-02-02

As life expectancy increases in patients with cystic fibrosis, it is important to pay attention to extra-pulmonary comorbidities. Several studies have shown signs of myocardial dysfunction in adult patients, but little is known about onset and development of these changes over time. In this prospective study, cardiac function in children with cystic fibrosis was compared to that of healthy children. 33 children, aged 3-12years, with cystic fibrosis were recruited from the Wilhelmina Children's hospital and 33 age-matched healthy children were selected from the WHISTLER study, a population-based cohort study. Measurements of lung function, arterial stiffness, and echocardiography (conventional measures and myocardial deformation imaging) were performed. There were no differences in anthropometrics, lung function and blood pressure between the two groups. The cystic fibrosis children had a higher arterial stiffness compared to the healthy children (pulse wave velocity respectively 5.76±0.57m/s versus 5.43±0.61m/s, p-value 0.049). Using conventional echocardiographic parameters for right ventricular function, Tricuspid Annular Plane Systolic Excursion) and Tissue Doppler Imaging, cystic fibrosis children had a reduced right ventricular systolic function when compared to the healthy children. After adjustment for lung function, global strains of both right and left ventricles were significantly lower in the cystic fibrosis group than in healthy children (linear regression coefficient 1.45% left ventricle, p-value 0.022 and 4.42% right ventricle, p-value cystic fibrosis children than in healthy controls. Our study suggests that already at a very young age, children with cystic fibrosis show an increased arterial stiffness and some signs of diminished both right and left ventricular function. Copyright © 2018. Published by Elsevier B.V.

The cystic form of rheumatoid arthritis

International Nuclear Information System (INIS)

Dijkstra, P.F.; Gubler, F.M.; Maas, A.

1988-01-01

A nonerosive form of rheumatoid arthritis (R.A.) was found in 62 patients out of 660 patients with R.A.. These 62 patients exhibit slowly progressive cystic changes in about the same joints in which usually erosions develop in classic R.A.. The E.S.R. is often low, half of the patients remained seronegative and there are 35 males and 27 females in the group. A smaller group of 15 out of these patients could be followed from a stage wherein the radiographs were normal to a stage of extensive cystic changes, over a period of at least 6 years. An attempt is made to delineate this group within the rheumatoid arthritis disease entity. (orig.) [de

Laboratory confirmation of the diagnosis of cystic fibrosis.

Science.gov (United States)

Tocci, P M; McKey, R M

1976-11-01

The recent commercial introduction of a method for detecting albumin in meconium makes screening for cystic fibrosis feasible for many hospitals. If the tests is adopted, confirmatory tests should be available. Quantitative analyses of sweat for sodium by flame photometry and for chloride by silver titration and ion-sleective electrodes are now used as confirmatory tests. We compare results of these confirmatory methods applied to presons with cystic fibrosis, respiratory disorders, or digestive disorders, and to control subjects.

Cystic Pulmonary Metastasis in a Patient with Scalp Angiosarcoma: A Case Report

Energy Technology Data Exchange (ETDEWEB)

Kim, Ah Yeong; Lee, Kyung Soo; Han, Jong Ho; Kim, Ho Joong; Kim, Kwhan Mien; Baek, Chung Hwan [Samsugn Medical Center, Sungkyunkwan University School of Medicine, Seoul (Korea, Republic of)

2011-08-15

It has been well known that angiosarcoma (AS), particularly scalp AS, metastasizes to the lungs with multiple air-filled cystic lesions on chest computed tomography scans. Pneumothorax, due to cystic lesion rupture into the pleural space, is frequent;however, we do not exactly know how rapidly the metastatic lesions spread to the lungs or what the exact pathogenetic mechanism for cystic metastasis is. According to our experience, the speed of disease progression in pulmonary metastasis is relatively fast and the entire lungs may be involved within two or three months. The infiltrating spindle cell tumors in the alveolar walls are tethering the adjacent alveolar spaces in order to form a dilated air-filled cystic lesion.

Not All Children with Cystic Fibrosis Have Abnormal Esophageal Neutralization during Chemical Clearance of Acid Reflux.

Science.gov (United States)

Woodley, Frederick W; Moore-Clingenpeel, Melissa; Machado, Rodrigo Strehl; Nemastil, Christopher J; Jadcherla, Sudarshan R; Hayes, Don; Kopp, Benjamin T; Kaul, Ajay; Di Lorenzo, Carlo; Mousa, Hayat

2017-09-01

Acid neutralization during chemical clearance is significantly prolonged in children with cystic fibrosis, compared to symptomatic children without cystic fibrosis. The absence of available reference values impeded identification of abnormal findings within individual patients with and without cystic fibrosis. The present study aimed to test the hypothesis that significantly more children with cystic fibrosis have acid neutralization durations during chemical clearance that fall outside the physiological range. Published reference value for acid neutralization duration during chemical clearance (determined using combined impedance/pH monitoring) was used to assess esophageal acid neutralization efficiency during chemical clearance in 16 children with cystic fibrosis (3 to chemical clearance exceeded the upper end of the physiological range in 9 of 16 (56.3%) children with and in 3 of 16 (18.8%) children without cystic fibrosis ( p =0.0412). The likelihood ratio for duration indicated that children with cystic fibrosis are 2.1-times more likely to have abnormal acid neutralization during chemical clearance, and children with abnormal acid neutralization during chemical clearance are 1.5-times more likely to have cystic fibrosis. Significantly more (but not all) children with cystic fibrosis have abnormally prolonged esophageal clearance of acid. Children with cystic fibrosis are more likely to have abnormal acid neutralization during chemical clearance. Additional studies involving larger sample sizes are needed to address the importance of genotype, esophageal motility, composition and volume of saliva, and gastric acidity on acid neutralization efficiency in cystic fibrosis children.

CT findings and differential diagnosis of cystic neck masses

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

1995-10-15

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

Treatment of cystic craniopharyngioma with phosphorus-32 intracavitary irradiation

Science.gov (United States)

Zhao, Rong; Deng, Jinglan; Liang, Xiaoyan; Zeng, Jin; Chen, Xiaoyuan

2013-01-01

Purpose The aim of the study was to evaluate the effect of phosphorus-32 colloid ([32P]) intracavitary irradiation on the treatment of patients with cystic craniopharyngiomas. Methods Twenty patients with predominantly cystic craniopharyngiomas were admitted from 1981 to 2006. Eleven patients had [32P] intracavitary irradiation by stereotactic injection or Ommaya cyst instillation as the primary treatment, and the remaining nine had the same internal irradiation as an adjuvant treatment after tumor resection. A calculated irradiation dose of 400~500 Gy per once was delivered to the cyst wall. Conclusion The patients were followed up ranging from 36 to 336 months; no operative morbidity or mortality was found from [32P] intracavitary irradiation. Fourteen patients (70%) had tumor progression and required further two to four times intracavitary irradiation. All 20 cases achieved tumor shrinkage or stabilization with effective outcome 3–6 months after the last [32P] therapy. For patients with cystic craniopharyngioma, [32P] administration by stereo-tactic injection or Ommaya cyst instillation is a safe and helpful option, which could improve the life quality, prolong the life span, and enhance the survival rate of cystic craniopharyngioma patients. PMID:19904543

Comparison of Clinico-Radiological Features between Congenital Cystic Neuroblastoma and Neonatal Adrenal Hemorrhagic Pseudocyst

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Eo, Hong; Kim, Ji Hye; Jang, Kyung Mi; Yoo, So Young [Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul (Korea, Republic of); Lim, Gye Yeon [St. Mary' s Hospital Catholic University, Seoul (Korea, Republic of); Kim, Myung Joon [Severance Hospital Yonsei University, Seoul (Korea, Republic of); Kim, Ok Hwa [Ajou University Hospital, Suwon (Korea, Republic of)

2011-02-15

To evaluate the radiological and clinical findings of congenital cystic neuroblastomas as compared with those of the cystic presentation of neonatal adrenal hemorrhage. We analyzed the US (n = 52), CT (n = 24), and MR (n = 4) images as well as the medical records of 28 patients harboring congenital cystic neuroblastomas (n = 16) and neonatal adrenal hemorrhagic pseudocysts (n = 14). The history of prenatal detection, location, size, presence of outer wall enhancement, internal septations, solid portion, calcification, turbidity, vascular flow on a Doppler examination, and evolution patterns were compared in two groups of cystic lesions, by Fischer's exact test. All (100%) neuroblastomas and three (21%) of the 14 hemorrhagic pseudocysts were detected prenatally. Both groups of cystic lesions occurred more frequently on the right side; 11 of 16 (69%) for neuroblastomas and 11 of 14 (79%) for hemorrhagic pseudocysts. The size, presence of solid portion, septum, enhancement, and turbidity did not differ significantly (p > 0.05) between the two groups of cystic lesions. However, tiny calcifications (n = 3) and vascular flow on color Doppler US (n = 3) were noted in only neuroblastomas. The cystic neuroblastomas became complex solid and cystic masses, and did not disappear for up to 90 days in the three following cases, whereas 11 of the 14 (79%) hemorrhagic pseudocysts disappeared completely and the three remaining (27%) evolved to calcifications only. Although the imaging findings of two groups of cystic lesions were similar, prenatal detection, the presence of calcification on initial images, vascularity on color Doppler US, and evolution to a more complex mass may all favor neuroblastomas

Comparison of Clinico-Radiological Features between Congenital Cystic Neuroblastoma and Neonatal Adrenal Hemorrhagic Pseudocyst

International Nuclear Information System (INIS)

Eo, Hong; Kim, Ji Hye; Jang, Kyung Mi; Yoo, So Young; Lim, Gye Yeon; Kim, Myung Joon; Kim, Ok Hwa

2011-01-01

To evaluate the radiological and clinical findings of congenital cystic neuroblastomas as compared with those of the cystic presentation of neonatal adrenal hemorrhage. We analyzed the US (n = 52), CT (n = 24), and MR (n = 4) images as well as the medical records of 28 patients harboring congenital cystic neuroblastomas (n = 16) and neonatal adrenal hemorrhagic pseudocysts (n = 14). The history of prenatal detection, location, size, presence of outer wall enhancement, internal septations, solid portion, calcification, turbidity, vascular flow on a Doppler examination, and evolution patterns were compared in two groups of cystic lesions, by Fischer's exact test. All (100%) neuroblastomas and three (21%) of the 14 hemorrhagic pseudocysts were detected prenatally. Both groups of cystic lesions occurred more frequently on the right side; 11 of 16 (69%) for neuroblastomas and 11 of 14 (79%) for hemorrhagic pseudocysts. The size, presence of solid portion, septum, enhancement, and turbidity did not differ significantly (p > 0.05) between the two groups of cystic lesions. However, tiny calcifications (n = 3) and vascular flow on color Doppler US (n = 3) were noted in only neuroblastomas. The cystic neuroblastomas became complex solid and cystic masses, and did not disappear for up to 90 days in the three following cases, whereas 11 of the 14 (79%) hemorrhagic pseudocysts disappeared completely and the three remaining (27%) evolved to calcifications only. Although the imaging findings of two groups of cystic lesions were similar, prenatal detection, the presence of calcification on initial images, vascularity on color Doppler US, and evolution to a more complex mass may all favor neuroblastomas

MR diffusion-weighted imaging in differential diagnosis of intracranial cystic lesions

International Nuclear Information System (INIS)

Ji Xueman; Lu Guangming; Wang Zhongqiu; Zhang Zongjun; Zhang Zhiqiang; Wang Junpeng

2007-01-01

Objective: To evaluate the value of diffusion-weighted imaging (DWI) on differential diagnosis of intracranial cystic lesions. Methods: Seventy-six patients with surgically and pathologically confirmed intracranial cystic lesions undergone conventional MRI, DWI and contrast enhanced MRI examination. The signal characteristics of intracranial cystic lesions on DWI were analysed retrospectively, the apparent diffusion coefficient (ADC) values of cystic areas were measured quantitatively. Results: Nineteen brain abscesses showed hyperintense signal on DWI. Among 34 brain tumors, 3 brain gliomas were hyperintense signal, 1 brain glioma was isointense signal and 1 metastasis was hyperintense signal; the other 29 brain tumors showed hypointense signal on DWI. The ADC values of all lesions were: (0.62 ± 0.15) x 10 -3 mm 2 /s in brain abscesses, (2.39 ± 0.78) x 10 -3 mm 2 /s in brain gliomas, (2.68 ± 0.40) x 10 -3 mm 2 /s in brain hemangioblastomas, (2.79 ± 0.79) x 10 -3 mm 2 /s in brain metastases, respectively. There were significant differences between the ADC values of brain abscess and the cystic or necrotic portions of brain glioma, hemangioblastoma, metastasis (P 0.05). Seven intracranial arachnoid cysts showed hypointense signal and 16 epidermoid cysts strikingly hyperintense signal on DWI. The ADC values of arachnoid cysts and epidermoid cysts were (2.96 ± 0.36) x 10 -3 mm 2 /s and (0.94 ± 0.13) x 10 -3 mm 2 /s respectively. There was significant difference between the ADC values of arachnoid cysts and epidermoid cysts (P<0.01). Conclusion: DWI and ADC values have important contribution to the differentiation of brain abscesses from cystic or necrotic tumors, intracranial cystic lesions showing hypointense signal on DWI can exclude brain abscess. (authors)

Stereotactic intracavitary brachytherapy with P-32 for cystic craniopharyngiomas in children

International Nuclear Information System (INIS)

Maarouf, Mohammad; El Majdoub, Faycal; Fuetsch, Manuel; Hoevels, Mauritius; Lehrke, Ralph; Berthold, Frank; Voges, Juergen; Sturm, Volker

2016-01-01

Although microsurgery remains the first-line treatment, gross total resection of cystic craniopharyngeomas (CP) is associated with significant morbidity and mortality and the addition of external irradiation to subtotal resection proves to achieve similar tumor control. However, concern regarding long-term morbidity associated with external irradiation in children still remains. With this retrospective analysis, the authors emphasize intracavitary brachytherapy using phosphorus-32 (P-32) as a treatment option for children with cystic CP. Between 1992 and 2009, 17 children (median age 15.4 years; range 7-18 years) with cystic CP underwent intracavitary brachytherapy using P-32. Eleven patients were treated for recurrent tumor cysts; 6 patients were treated primarily. MR imaging revealed solitary cysts in 7 patients; 10 patients had mixed solid-cystic lesions (median tumor volume 11.1 ml; range 0.5-78.9 ml). The median follow-up time was 61.9 months (range 16.9-196.6 months). Local cyst control could be achieved in 14 patients (82 %). Three patients showed progression of the treated cystic formation (in-field progression) after a median time of 8.3 months (range 5.3-10.3 months), which led to subsequent interventions. The development of new, defined cysts and progression of solid tumor parts (out-of-field progression) occurred in 5 patients and led to additional interventions in 4 cases. There was neither surgery-related permanent morbidity nor mortality in this study. The overall progression-free survival was 75, 63, and 52 % after 1, 3, and 5 years, respectively. Intracavitary brachytherapy using P-32 represents a safe and effective treatment option for children harboring cystic CP, even as primary treatment. However, P-32 does not clearly affect growth of solid tumor parts or the development of new cystic formations. (orig.) [de

18F-fluorodeoxyglucose positron emission tomography in management of pancreatic cystic tumors

International Nuclear Information System (INIS)

Zhang Yaojun; Frampton, Adam E.; Martin, Jack L.; Kyriakides, Charis; Bong, Jan Jin; Habib, Nagy A.; Vlavianos, Panagiotis; Jiao, Long R.

2012-01-01

Objectives: To evaluate the effectiveness of PET in differentiating malignant from benign pancreatic cystic tumors. Methods: Between 2009 and 2010, all patients with pancreatic cystic tumors who had PET, triple phase contrast computed tomography (CT) and endoscopic ultrasound (EUS) were reviewed. Clinicopathological characteristics and final histology were correlated with preoperative PET, CT and EUS to assess the value of each modality in detecting malignant from benign lesions for clinical decision-making. Results: Twenty of a total of 116 patients with pancreatic cystic tumors had 18F-FDG PET because of diagnostic difficulties after evaluation with conventional modalities. Sensitivity and specificity of PET in differentiating malignant from benign pancreatic cystic tumors were 100% and 93.75%, with an accuracy of 95%. PET had the best sensitivity, specificity and accuracy for detecting malignant cystic tumors compared with CT and EUS. In 5 cases, the PET results altered the treatment options completely to follow-up instead of surgery (n = 2), limited resection instead of Whipple's resection (n = 1), and surgery instead of follow-up (n = 2). Conclusions: PET is an accurate, non-invasive method to distinguish malignant from benign pancreatic cystic tumors and can be used as an adjunct to facilitate clinical decision making.

An adult cystic fibrosis patient presenting with persistent dyspnea: case report

Directory of Open Access Journals (Sweden)

Farinet Catherine L

2006-05-01

Full Text Available Abstract Background Persistent dyspnea is a common finding in the cystic fibrosis patient that typically leads to further work up of an alternative pulmonary etiology. Adult cystic fibrosis patients; however, are growing in numbers and they are living into the ages in which coronary artery disease becomes prevalent. Coronary disease should be included in the consideration of diagnostic possibilities. Case presentation A 52-year-old white male with cystic fibrosis was evaluated for exertional dyspnea associated with vague chest discomfort. Diagnostic testing revealed normal white blood cell, hemoglobin and platelet count, basic metabolic panel, fasting lipid profile, HbA1c, with chest radiograph confirming chronic cystic findings unchanged from prior radiographs and an electrocardiogram that revealed sinus rhythm with left anterior fascicular block. Stress thallium testing demonstrated a reversible anteroseptal perfusion defect with a 55% left ventricular ejection fraction. Heart catheterization found a 99% occlusion of the left anterior descending artery extending into the two diagonal branches, with 100% obstruction of the left anterior descending artery at the trifurcation and 70% lesion affecting the first posterior lateral branch of the circumflex artery. Conclusion This case report represents the first description in the medical literature of a cystic fibrosis patient diagnosed with symptomatic coronary artery disease. Applying a standard clinical practice guide proved useful toward evaluating a differential diagnosis for a cystic fibrosis patient presenting with dyspnea and chest discomfort.

Incidence of Radiographic Cystic Lesions Associated With Unerupted Teeth in Dogs.

Science.gov (United States)

Babbitt, Sam G; Krakowski Volker, Mary; Luskin, Ira R

2016-12-01

Medical records and radiographs were retrospectively reviewed over a 3-year period (2012-2015) from dogs presented to a private dental referral practice. Medical records were evaluated for the diagnosis of impacted or embedded teeth. The identified dogs' radiographs were reviewed for the presence of radiographically significant cystic lesions that were associated with the impacted or embedded teeth. Radiographic criteria were established by the authors for the purposes of identifying cystic lesions in dogs of different breeds and sizes, using established indices from human dental pathology as a basis. When histopathology results were available, they were reviewed and reported. In this study, 136 dogs ranging in age from 3 months to 17 years were diagnosed with 213 unerupted teeth during the study period. There were 62 (29.1%) of the 213 radiographically apparent cystic lesions identified based on the criteria proposed in this study. In this study, 146 (68.5%) of the 213 unerupted teeth were identified as mandibular first premolar teeth. Histopathology was obtained on 28 (45.1%) of the 62 diagnosed cystic lesions. Dentigerous cysts accounted for 20 (71.4%) of the 28 cystic lesions. Brachycephalic breeds were overrepresented in this study. Boxer, pug, Shih Tzu, and Boston terrier dogs were most likely to present with cystic lesions associated with unerupted teeth. Seventeen (85%) of the 20 cases with a histopathologic diagnosis of dentigerous cyst were found within these 4 brachycephalic breeds.

Quantitative immunoassays for diagnosis and carrier detection in cystic fibrosis

International Nuclear Information System (INIS)

Bullock, S.; Hayward, C.; Manson, J.; Brock, D.J.H.; Raeburn, J.A.

1982-01-01

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)

A unique case of a huge mixed squamous cell and glandular papilloma of non-endobronchial origin with a peripheral growth

Directory of Open Access Journals (Sweden)

Kei Yabuki

Full Text Available We report a case of a huge solitary non-endobronchial pulmonary tumor in a 76-year-old male smoker. The tumor measured 11 × 10 × 8 cm. It was ill-defined, and it was located periphery of the right lower lobe with the subpleural cystic spaces. He underwent right lower lobectomy with mediastinal lymph node dissection and is free from tumor 30 months after surgery. Microscopically, it was composed of a proliferation of squamous and ciliated columnar epithelial cells with a few mucous cells. These cells were arranged in a papillary growth fashion extending along the fibrously thickened alveolar septa together with metaplastic bronchiolar and squamous epithelia displaying an usual interstitial pneumonia-pattern. Although the histologic features of the tumor were that of a mixed squamous cell and glandular papilloma (MSCGP, it was peripherally located and showed a lepidic growth, and it was much larger than previously reported MSCGPs. It is possible that the tumor developed in association with bronchial metaplasia in the periphery of the lung, and then extended along the surface of the reconstructed air spaces, which resulted in its unique histologic appearance. Further investigations of respiratory papilloma are needed to clarify the pathogenesis of these lesions. Keywords: Mixed squamous cell and glandular papilloma, Non-endobronchial, Cytology, Interstitial pneumonia, Pulmonary tumor

Hyponatremia-associated rhabdomyolysis following exercise in an adolescent with cystic fibrosis.

Science.gov (United States)

Kaskavage, Jillian; Sklansky, Daniel

2012-07-01

Adolescents with well-controlled cystic fibrosis, including good lung function and appropriate growth, commonly participate in competitive athletic activities. We present the case of an adolescent male with cystic fibrosis, hyponatremia, dehydration, and rhabdomyolysis after participating in football practice on a summer morning. The patient presented with severe myalgia and serum sodium of 129 mmol/L, chloride 90 mmol/L, and creatine phosphokinase 1146 U/L. Aggressive hydration with intravenous 0.9% saline resulted in clinical improvement with no renal or muscular sequelae. Health care providers need to educate patients with cystic fibrosis about maintaining adequate hydration and sodium repletion during exercise. Research is needed regarding the appropriate amount and composition of oral rehydration fluids in exercising individuals with cystic fibrosis, as the physiology encountered in these patients provides a unique challenge to maintaining electrolyte balance and stimulation of thirst.

Blunted perception of neural respiratory drive and breathlessness in patients with cystic fibrosis

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Charles C. Reilly

2016-03-01

Full Text Available The electromyogram recorded from the diaphragm (EMGdi and parasternal intercostal muscle using surface electrodes (sEMGpara provides a measure of neural respiratory drive (NRD, the magnitude of which reflects lung disease severity in stable cystic fibrosis. The aim of this study was to explore perception of NRD and breathlessness in both healthy individuals and patients with cystic fibrosis. Given chronic respiratory loading and increased NRD in cystic fibrosis, often in the absence of breathlessness at rest, we hypothesised that patients with cystic fibrosis would be able to tolerate higher levels of NRD for a given level of breathlessness compared to healthy individuals during exercise. 15 cystic fibrosis patients (mean forced expiratory volume in 1 s (FEV1 53.5% predicted and 15 age-matched, healthy controls were studied. Spirometry was measured in all subjects and lung volumes measured in the cystic fibrosis patients. EMGdi and sEMGpara were recorded at rest and during incremental cycle exercise to exhaustion and expressed as a percentage of maximum (% max obtained from maximum respiratory manoeuvres. Borg breathlessness scores were recorded at rest and during each minute of exercise. EMGdi % max and sEMGpara % max and associated Borg breathlessness scores differed significantly between healthy subjects and cystic fibrosis patients at rest and during exercise. The relationship between EMGdi % max and sEMGpara % max and Borg score was shifted to the right in the cystic fibrosis patients, such that at comparable levels of EMGdi % max and sEMGpara % max the cystic fibrosis patients reported significantly lower Borg breathlessness scores compared to the healthy individuals. At Borg score 1 (clinically significant increase in breathlessness from baseline corresponding levels of EMGdi % max (20.2±12% versus 32.15±15%, p=0.02 and sEMGpara % max (18.9±8% versus 29.2±15%, p=0.04 were lower in the healthy individuals compared to the cystic

Uneven Distribution of Regional Blood Supply Prompts the Cystic Change of Pituitary Adenoma.

Science.gov (United States)

Zhang, Jianhe; Gu, Jianjun; Ma, Yiming; Huang, Yinxing; Wang, Jiaxing; Wu, Zhifeng; Zhong, Qun; Wang, Shousen

2017-07-01

Previous studies have suggested that the cystic change of pituitary adenoma might be related to the blood supply and metabolism of the tumor; however, the exact pathologic mechanism underlying the cystic change remains unknown. We aimed to assess the features of regional blood supply of pituitary adenoma and examine its relationship with the cystic change of pituitary adenoma. Patients (N = 79) with pituitary adenoma admitted to our hospital were divided into the parenchyma group (n = 40) or the cystic change group (n = 39). Dynamic contrast-enhanced magnetic resonance imaging of the pituitary adenoma was conducted for the parenchyma group and the steepest slopes (SS max , reflecting regional blood supply) at different areas were calculated. The location of cystic change of the pituitary adenoma was recorded and analyzed for the cystic change group. The parenchyma group showed an upper SS max of 2.52 ± 1.18, a lower SS max of 2.89 ± 1.46, a left SS max of 2.71 ± 1.31, and a right SS max of 2.66 ± 1.29. The difference between the upper and lower SS max was statistically significant (P supply is unevenly distributed in the parenchymal pituitary adenoma, with reduced blood supply in the upper than the lower region. Cystic change mainly occurs in the upper region of pituitary adenoma. Copyright © 2017 Elsevier Inc. All rights reserved.

Radiological findings in congenital cystic disease of the lung in infancy

International Nuclear Information System (INIS)

Heymer, R.; Benz-Bohm, G.; Arnold, G.; Koeln Univ.

1982-01-01

Three infants with congenital cystic disease of the lung are described. They consisted of a solitary bronchial cyst, an adenomatous cystic malformation and congenital lobar emphysema. The radiological appearance and differential diagnosis of these three conditions are discussed. (orig.) [de

Cystic tumors of the pancreas; Zystische Tumoren des Pankreas

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Brambs, H.J.; Juchems, M. [Universitaetsklinikum Ulm, Abteilung fuer Diagnostische und Interventionelle Radiologie, Ulm (Germany)

2008-08-15

Cystic lesions of the pancreas encompass a broad spectrum of benign, premalignant, and malignant tumors which are primarily cystic or result from cystic necroses of solid neoplasms. Because of the wide use of cross-sectional imaging techniques they are increasingly being identified in asymptomatic patients as well as in patients presenting with abdominal pain, jaundice or pancreatitis. Among these lesions, intraductal papillary mucinous neoplasms, serous cystic neoplasms and mucinous cystic neoplasms represent the majority of cases. With increasing experience with these tumors, a refinement of our understanding of their morphology and of their natural course has emerged. It is important to be familiar with the CT and MR imaging features of these lesions to differentiate these tumors and to orient the diagnosis towards benign or malignant forms. Because characterization of cystic tumors of the pancreas can sometimes be difficult due to overlapping imaging features, additional criteria such as clinical symptoms, localization, age and gender have to be taken into account. If appropriately treated, these tumors can usually be cured by resection and the decreasing risk of pancreatic surgery has led to an increasing number of resections of pancreatic tumors. The management of cystic tumors of the pancreas has not yet been standardized and the correct evaluation and subsequent management of the disease in asymptomatic patients have not been fully defined. (orig.) [German] Zystische Pankreastumoren umfassen ein breites Spektrum gutartiger, praemaligner und maligner Veraenderungen, die primaer zystisch sind oder durch eine zystische Degeneration solider Tumoren entstehen. Wegen des breiten Einsatzes von Schnittbildtechniken werden sie zunehmend bei asymptomatischen Patienten und bei Patienten mit Bauchschmerzen, Pankreatitis und Ikterus entdeckt. Unter diesen Tumoren stellen die intraduktalen papillaeren muzinoesen Neoplasien, die seroesen zystischen Neoplasien und die

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

African Journals Online (AJOL)

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

Cystic lesion of pancreas - Intraductal papillary mucinous neoplasm

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Rajiv Baijal

2013-01-01

Full Text Available Intraductal papillary mucinous neoplasm (IPMN of the pancreas is an intraductal mucin-producing epithelial neoplasm that arises from the main and/or branched pancreatic duct. It usually presents as cystic lesion of pancreas. There are well known differential diagnosis of cystic pancreatic lesion. Pancreatic cystic neoplasms are detected at an increasing frequency due to an increased use of abdominal imaging. The diagnosis and treatment of intraductal papillary mucinous tumors (IPMN of the pancreas has evolved over the past decade. IPMN represents a spectrum of disease, ranging from benign to malignant lesions, making the early detection and characterization of these lesions important. Definitive management is surgical resection for appropriate candidates, as benign lesions harbor malignant potential. IPMN has a prognosis, which is different from adenocarcinoma of the pancreas. We report a case of a 58-year-old male with intraductal papillary neoplasm involving main duct and side branches presenting to us with clinical symptoms of chronic pancreatitis with obstructive jaundice and cholangitis treated surgically.

Adenoid cystic carcinoma associated with mucous retention cyst of the parotid gland.

Science.gov (United States)

Hebbale, Manjula Advisha; Halli, Rajshekhar C; Kini, Yogesh K; Kharkar, Viraj R; Metgud, Rashmi

2011-09-01

Mucous retention cysts of the parotid gland are rare, and a coexistent adenoid cystic carcinoma is even an unusual occurrence. Such coexistent adenoid cystic carcinomas with mucous retention cyst of the parotid gland are difficult to diagnose clinically and, at times, stage difficulty in their management. We report a rare case of adenoid cystic carcinoma associated with mucous retention cyst of the parotid gland with its diagnostic and management dilemma in a 14-year-old adolescent girl.

Benign cystic mesothelioma of the appendix presenting in a woman: a case report

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Beddy David

2010-12-01

Full Text Available Abstract Introduction Benign cystic mesothelioma or peritoneal inclusion cysts are rare benign abdominal tumors usually occurring in females of reproductive age. These cysts present as abdominopelvic pain or masses but are often found on imaging or incidentally at surgery. They are commonly associated with pelvic inflammatory disease, endometriosis, or ovarian cysts. We report what is, to the best of our knowledge, the first case of a benign cystic mesothelioma complicating a presentation of acute appendicitis. Case Presentation A 19-year-old Irish Caucasian woman presented with abdominal pain. Imaging suggested appendicitis with abscess formation. She was treated with antibiotics and scheduled for interval appendicectomy. At laparoscopy, an unusual cystic mass was found arising from the appendix. Histology revealed benign cystic mesothelioma. Conclusion We report what is, to the best of our knowledge, the first case of a benign cystic mesothelioma arising from the appendix and complicating a presentation of acute appendicitis. This is a benign pathology, but recurrences are not uncommon. Benign cystic mesothelioma should be included in the differential when investigating pelvic masses or abscesses associated with either appendicitis or pelvic inflammatory disease in women.

Benign cystic mesothelioma of the appendix presenting in a woman: a case report

LENUS (Irish Health Repository)

O' Connor, Donal B

2010-12-03

Abstract Introduction Benign cystic mesothelioma or peritoneal inclusion cysts are rare benign abdominal tumors usually occurring in females of reproductive age. These cysts present as abdominopelvic pain or masses but are often found on imaging or incidentally at surgery. They are commonly associated with pelvic inflammatory disease, endometriosis, or ovarian cysts. We report what is, to the best of our knowledge, the first case of a benign cystic mesothelioma complicating a presentation of acute appendicitis. Case Presentation A 19-year-old Irish Caucasian woman presented with abdominal pain. Imaging suggested appendicitis with abscess formation. She was treated with antibiotics and scheduled for interval appendicectomy. At laparoscopy, an unusual cystic mass was found arising from the appendix. Histology revealed benign cystic mesothelioma. Conclusion We report what is, to the best of our knowledge, the first case of a benign cystic mesothelioma arising from the appendix and complicating a presentation of acute appendicitis. This is a benign pathology, but recurrences are not uncommon. Benign cystic mesothelioma should be included in the differential when investigating pelvic masses or abscesses associated with either appendicitis or pelvic inflammatory disease in women.

Aggressive angiomyxoma presenting with huge abdominal lump: A case report

Science.gov (United States)

Kumar, Sanjeev; Agrawal, Nikhil; Khanna, Rahul; Khanna, AK

2008-01-01

Agressive angiomyxoma is a rare mesenchymal neoplasm. It mainly presents in females. We here present a case of angiomyxoma presenting as huge abdominal lump along with gluteal swelling. Case note is described along with brief review of literature. PMID:18755035

Umbilicoplasty in children with huge umbilical hernia | Komlatsè ...

African Journals Online (AJOL)

With a mean follow-up of 10 months, we had 10 excellent results and two fair results according to our criteria. Conclusion: Our two lateral fl aps umbilicoplasty is well-adapted to HUH in children. Itis simple and assures a satisfactory anatomical and cosmetic result. Key words: Children, huge umbilical hernia, Togo, umbilical ...

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...

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.)

MRI in mucoviscidosis (cystic fibrosis)

International Nuclear Information System (INIS)

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

2006-01-01

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

Fine-needle aspiration study of cystic papillary thyroid carcinoma: Rare cytological findings

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Maral Mokhtari

2016-01-01

Full Text Available Background: Cystic papillary thyroid carcinoma (CPTC is a variant of papillary carcinoma that has many mimickers in cytological grounds. Aim: To study the cytomorphologic features of CPTC and compare them to those of other cystic thyroid lesions using fine-needle aspiration cytology (FNAC. We also aimed to identify the cytomorphologic features that distinguish CPTC from other cystic thyroid lesions. Materials and Methods: Seventy-three cases of CPTC were included in the study. The cytomorphologic features of these cases were analyzed. The FNA smears of other thyroid lesions with cystic changes (300 colloid goiters, 290 adenomatoid nodules, 11 follicular neoplasms, and 9 hurtle cell neoplasm were also studied. Results: The smears in CPTC revealed isolated follicular cells, small groups of cells with scalloped margins, cell swirls, small clusters with a cartwheel pattern, papillary clusters, intranuclear inclusions, nuclear grooves, sticky colloid, intracellular colloids, psammoma bodies, multinucleated giant cells, and foamy and hemosiderin laden macrophages. Small groups of cells with scalloped borders, cellular swirls, and small clusters with a cartwheel pattern were seen in CPTC, but not in other cystic lesions. Interestingly, mesothelial-like cells and hemophagocytic cells were seen in five and three cases of CPTC, respectively, but not in other cystic lesions. Conclusion: Mesothelial-like cells and hemophagocytic cells were observed in five and three cases of CPTC, respectively. Similar finding have not been previously reported in the literature.

Fine-needle aspiration study of cystic papillary thyroid carcinoma: Rare cytological findings

Science.gov (United States)

Mokhtari, Maral; Kumar, Perikala Vijayananda; Hayati, Kamran

2016-01-01

Background: Cystic papillary thyroid carcinoma (CPTC) is a variant of papillary carcinoma that has many mimickers in cytological grounds. Aim: To study the cytomorphologic features of CPTC and compare them to those of other cystic thyroid lesions using fine-needle aspiration cytology (FNAC). We also aimed to identify the cytomorphologic features that distinguish CPTC from other cystic thyroid lesions. Materials and Methods: Seventy-three cases of CPTC were included in the study. The cytomorphologic features of these cases were analyzed. The FNA smears of other thyroid lesions with cystic changes (300 colloid goiters, 290 adenomatoid nodules, 11 follicular neoplasms, and 9 hurtle cell neoplasm) were also studied. Results: The smears in CPTC revealed isolated follicular cells, small groups of cells with scalloped margins, cell swirls, small clusters with a cartwheel pattern, papillary clusters, intranuclear inclusions, nuclear grooves, sticky colloid, intracellular colloids, psammoma bodies, multinucleated giant cells, and foamy and hemosiderin laden macrophages. Small groups of cells with scalloped borders, cellular swirls, and small clusters with a cartwheel pattern were seen in CPTC, but not in other cystic lesions. Interestingly, mesothelial-like cells and hemophagocytic cells were seen in five and three cases of CPTC, respectively, but not in other cystic lesions. Conclusion: Mesothelial-like cells and hemophagocytic cells were observed in five and three cases of CPTC, respectively. Similar finding have not been previously reported in the literature. PMID:27756982

Plasma lactoferrin levels in pregnancy and cystic fibrosis

International Nuclear Information System (INIS)

Sykes, J.A.C.; Thomas, M.J.; Goldie, D.J.; Turner, G.M.

1982-01-01

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.)

The evaluation of selected insomnia predictors in adolescents and young adults with cystic fibrosis.

Science.gov (United States)

Tomaszek, Lucyna; Cepuch, Grazyna; Pawlik, Lidia

2018-03-21

The purpose of the study was to assess the incidence of insomnia in adolescents and young adults with cystic fibrosis and its impact on the quality of life, and to examine whether demographic and clinical factors and negative emotional states are predictors of insomnia in these patients. The study was conducted among 95 cystic fibrosis patients aged 14-25 years. The study used a personal questionnaire survey, the Athens Insomnia Scale, the Cystic Fibrosis Quality of Life Questionnaire, the Hospital Anxiety and Depression Scale, and the Numeric Rating Scale. Insomnia was diagnosed in 38% of cystic fibrosis patients. In patients with insomnia, the level of anxiety (Me: 10 vs. 4; P=0.000) and depression (Me: 6.5 vs. 2; P=0.000) was significantly higher than in the good sleep quality group. The risk of insomnia increases as anxiety (OR: 4.31; 95% CI: 2.20 to 8.41) and depressive symptoms exacerbate (OR: 4.98; 95% CI: 1.84 to 13.43). Insomnia significantly worsens the quality of life in cystic fibrosis patients (ß =-0.5, P=0.000). Insomnia affects a large percentage of cystic fibrosis patients, and anxiety and depression are factors that increase the risk of insomnia. Insomnia decreases the quality of life in cystic fibrosis patients.

Cost Effectiveness of Screening Individuals With Cystic Fibrosis for Colorectal Cancer.

Science.gov (United States)

Gini, Andrea; Zauber, Ann G; Cenin, Dayna R; Omidvari, Amir-Houshang; Hempstead, Sarah E; Fink, Aliza K; Lowenfels, Albert B; Lansdorp-Vogelaar, Iris

2018-02-01

Individuals with cystic fibrosis are at increased risk of colorectal cancer (CRC) compared with the general population, and risk is higher among those who received an organ transplant. We performed a cost-effectiveness analysis to determine optimal CRC screening strategies for patients with cystic fibrosis. We adjusted the existing Microsimulation Screening Analysis-Colon model to reflect increased CRC risk and lower life expectancy in patients with cystic fibrosis. Modeling was performed separately for individuals who never received an organ transplant and patients who had received an organ transplant. We modeled 76 colonoscopy screening strategies that varied the age range and screening interval. The optimal screening strategy was determined based on a willingness to pay threshold of $100,000 per life-year gained. Sensitivity and supplementary analyses were performed, including fecal immunochemical test (FIT) as an alternative test, earlier ages of transplantation, and increased rates of colonoscopy complications, to assess if optimal screening strategies would change. Colonoscopy every 5 years, starting at an age of 40 years, was the optimal colonoscopy strategy for patients with cystic fibrosis who never received an organ transplant; this strategy prevented 79% of deaths from CRC. Among patients with cystic fibrosis who had received an organ transplant, optimal colonoscopy screening should start at an age of 30 or 35 years, depending on the patient's age at time of transplantation. Annual FIT screening was predicted to be cost-effective for patients with cystic fibrosis. However, the level of accuracy of the FIT in this population is not clear. Using a Microsimulation Screening Analysis-Colon model, we found screening of patients with cystic fibrosis for CRC to be cost effective. Because of the higher risk of CRC in these patients, screening should start at an earlier age with a shorter screening interval. The findings of this study (especially those on FIT

Acoustic radiation force impulse (ARFI) ultrasound imaging of pancreatic cystic lesions

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D' Onofrio, M., E-mail: mirko.donofrio@univr.it [Department of Radiology, University Hospital G.B. Rossi, Piazzale L.A. Scuro 10, University of Verona, 37134 Verona (Italy); Gallotti, A. [Department of Radiology, University Hospital G.B. Rossi, Piazzale L.A. Scuro 10, University of Verona, 37134 Verona (Italy); Salvia, R. [Department of Surgery, University Hospital G.B. Rossi, Piazzale L.A. Scuro 10, University of Verona, 37134 Verona (Italy); Capelli, P. [Department of Pathology, University Hospital G.B. Rossi, Piazzale L.A. Scuro 10, University of Verona, 37134 Verona (Italy); Mucelli, R. Pozzi [Department of Radiology, University Hospital G.B. Rossi, Piazzale L.A. Scuro 10, University of Verona, 37134 Verona (Italy)

2011-11-15

Purpose: To evaluate the ARFI ultrasound imaging with Virtual Touch tissue quantification in studying pancreatic cystic lesions, compared with phantom fluid models. Materials and methods: Different phantom fluids at different viscosity or density (water, iodinate contrast agent, and oil) were evaluated by two independent operators. From September to December 2008, 23 pancreatic cystic lesions were prospectively studied. All lesions were pathologically confirmed. Results: Non-numerical values on water and numerical values on other phantoms were obtained. Inter-observer evaluation revealed a perfect correlation (rs = 1.00; p < 0.0001) between all measurements achieved by both operators per each balloon and fluid. Among the pancreatic cystic lesions, 14 mucinous cystadenomas, 4 pseudocysts, 3 intraductal papillary-mucinous neoplasms and 2 serous cystadenomas were studied. The values obtained ranged from XXXX/0-4,85 m/s in mucinous cystadenomas, from XXXX/0-3,11 m/s in pseudocysts, from XXXX/0-4,57 m/s in intraductal papillary-mucinous neoplasms. In serous cystadenomas all values measured were XXXX/0 m/s. Diagnostic accuracy in benign and non-benign differentiation of pancreatic cystic lesions was 78%. Conclusions: Virtual Touch tissue quantification can be applied in the analysis of fluids and is potentially able to differentiate more complex (mucinous) from simple (serous) content in studying pancreatic cystic lesions.

Acoustic radiation force impulse (ARFI) ultrasound imaging of pancreatic cystic lesions

International Nuclear Information System (INIS)

D'Onofrio, M.; Gallotti, A.; Salvia, R.; Capelli, P.; Mucelli, R. Pozzi

2011-01-01

Purpose: To evaluate the ARFI ultrasound imaging with Virtual Touch tissue quantification in studying pancreatic cystic lesions, compared with phantom fluid models. Materials and methods: Different phantom fluids at different viscosity or density (water, iodinate contrast agent, and oil) were evaluated by two independent operators. From September to December 2008, 23 pancreatic cystic lesions were prospectively studied. All lesions were pathologically confirmed. Results: Non-numerical values on water and numerical values on other phantoms were obtained. Inter-observer evaluation revealed a perfect correlation (rs = 1.00; p < 0.0001) between all measurements achieved by both operators per each balloon and fluid. Among the pancreatic cystic lesions, 14 mucinous cystadenomas, 4 pseudocysts, 3 intraductal papillary-mucinous neoplasms and 2 serous cystadenomas were studied. The values obtained ranged from XXXX/0-4,85 m/s in mucinous cystadenomas, from XXXX/0-3,11 m/s in pseudocysts, from XXXX/0-4,57 m/s in intraductal papillary-mucinous neoplasms. In serous cystadenomas all values measured were XXXX/0 m/s. Diagnostic accuracy in benign and non-benign differentiation of pancreatic cystic lesions was 78%. Conclusions: Virtual Touch tissue quantification can be applied in the analysis of fluids and is potentially able to differentiate more complex (mucinous) from simple (serous) content in studying pancreatic cystic lesions.

Pregnancy outcome in women with cystic fibrosis-related diabetes.

Science.gov (United States)

Reynaud, Quitterie; Poupon-Bourdy, Stéphanie; Rabilloud, Muriel; Al Mufti, Lina; Rousset Jablonski, Christine; Lemonnier, Lydie; Nove-Josserand, Raphaële; Touzet, Sandrine; Durieu, Isabelle

2017-10-01

With increasing life expectancy, more women with cystic fibrosis and diabetes mellitus become pregnant. We investigated how pre-gestational diabetes (cystic fibrosis-related diabetes) influenced pregnancy outcome and the clinical status of these women. We analyzed all pregnancies reported to the French cystic fibrosis registry between 2001 and 2012, and compared forced expiratory volume (FEV 1 ) and body mass index before and after pregnancy in women with and without pre-gestational diabetes having a first delivery. A total 249 women delivered 314 infants. Among these, 189 women had a first delivery and 29 of these had pre-gestational diabetes. There was a trend towards a higher rate of assisted conception among diabetic women (53.8%) than non-diabetic women (34.5%, p = 0.06), and the rate of cesarean section was significantly higher in diabetic women (48% vs. 21.4%, p = 0.005). The rate of preterm birth and mean infant birthweight did not differ significantly between diabetic and non-diabetic women. Forced expiratory volume before pregnancy was significantly lower in the diabetic group. The decline in forced expiratory volume and body mass index following pregnancy did not differ between the women with and those without pre-gestational diabetes. Pre-gestational diabetes in women with cystic fibrosis is associated with a higher rate of cesarean section but does not seem to have a clinically significant impact on fetal growth or preterm delivery. The changes in maternal pulmonary and nutritional status following pregnancy in women with cystic fibrosis were not influenced by pre-gestational diabetes. © 2017 Nordic Federation of Societies of Obstetrics and Gynecology.

Huge mucinous cystadenoma of ovary, describing a young patient: case report

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Soheila Aminimoghaddam

2017-08-01

Conclusion: Ovarian cysts in young women who are associated with elevated levels of tumor markers and ascites require careful evaluation. Management of ovarian cysts depends on patient's age, size of the cyst, and its histopathological nature. Conservative surgery such as ovarian cystectomy or salpingo-oophorectomy is adequate in mucinous tumors of ovary. Multiple frozen sections are very important to know the malignant variation of this tumor and helps accurate patient management. Surgical expertise is required to prevent complications in huge tumors has distorted the anatomy, so gynecologic oncologist plays a prominent role in management. In this case, beside of the huge tumor and massive ascites uterine and ovaries were preserved by gynecologist oncologist and patient is well up to now.

Maintenance of nutritional status in patients with cystic fibrosis: new and emerging therapies

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Kalnins D

2012-06-01

Full Text Available Daina Kalnins,1 Michael Wilschanski21Clinical Dietetics, Respiratory Medicine, The Hospital for Sick Children, Toronto, ON, Canada; 2Pediatric Gastroenterology Unit, Hadassah University Hospitals, Jerusalem, IsraelAbstract: Poor clinical outcomes in cystic fibrosis are often associated with undernutrition. Normal growth and development should be achieved in cystic fibrosis, and nutritional counseling is paramount at all ages. Prevention and early detection of growth failure is the key to successful nutritional intervention. The advance in nutritional management is certainly one factor that has contributed to the improved survival in recent decades. This review outlines the major nutritional parameters in the management of the patient with cystic fibrosis, including recent advances in pancreatic enzyme replacement therapy and fat-soluble vitamin therapy. There are sections on complicated clinical situations which directly affect nutrition, for example, before and after lung transplantation, cystic fibrosis-related diabetes, and bone health.Keywords: cystic fibrosis, nutrition, fat-soluble vitamins, pancreatic enzymes

Multilocular Cystic Nephroma of the Kidney in a Young Adult: Case ...

African Journals Online (AJOL)

mn

Histological examination of the resected specimen confirmed the diagnosis of multilocular cystic nephroma. This is a very ... diagnosis can be suggested by imaging studies, definitive diag- nosis is usually made histologically. ... the lesion, its origin and relationship with other predominantly cystic lesions of the kidney, such ...

Recombinant human DNase I reduces the viscosity of cystic fibrosis sputum.

Science.gov (United States)

Shak, S; Capon, D J; Hellmiss, R; Marsters, S A; Baker, C L

1990-12-01

Respiratory distress and progressive lung destruction in cystic fibrosis can be attributed to bacterial persistence and the accumulation of viscous purulent secretions in the airways. More than 30 yr ago it was suggested that the large amounts of DNA in purulent secretions contribute to its viscosity and that bovine pancreatic DNase I could reduce the viscosity. To evaluate the potential clinical utility of recombinant human DNase I (rhDNase) in the treatment of cystic fibrosis, we have cloned, sequenced, and expressed rhDNase. Catalytic amounts of rhDNase greatly reduce the viscosity of purulent cystic fibrosis sputum, transforming it within minutes from a nonflowing viscous gel to a flowing liquid. The reduction in viscosity is associated with a decrease in size of DNA in the sputum. Inhalation of a rhDNase aerosol may be a simple direct approach that will help individuals with cystic fibrosis and other patients with pneumonia or bronchitis to clear their airways of purulent secretions.

Rapid increase in cystic volume of an anaplastic astrocytoma misdiagnosed as neurocysticercosis: A case report

Science.gov (United States)

Li, Hong-Jiang; Han, Hong-Xiu; Feng, Dong-Fu

2016-01-01

Reports describing a rapid increase in the cystic volume of anaplastic astrocytoma (AA) in a short time frame are rare. The present study reports the case of a 68-year-old male who was admitted to the No. 9 People's Hospital, Shanghai Jiaotong University School of Medicine (Shanghai, China), with a small cystic brain lesion and positive immunological testing for cysticercosis. Head magnetic resonance imaging (MRI) showed a cystic lesion, 6 mm in diameter, in the left frontal lobe. Neurocysticercosis was suspected and the patient was treated with a clinical trial of albendazole and steroids. A period of 25 days later, the patient's condition had deteriorated, and MRI revealed a cystic lesion in the left frontal lobe; thereafter, the cystic lesion was removed and a diagnosis of AA was established. The tumor was soft, ivory white and gelatinous due to myxoid degeneration. In this case, tumor-related angiogenesis and microvascular extravasation (blood-brain barrier disruption) may have been the main cause of the rapid increase in the cystic volume in such a short time frame. The similarity of the glioma and cysticercus antigens may have been the cause of the positive reactions in the cystic fluid. The present study reports the rare occurrence of a rapid increase of cystic volume and potential diagnostic difficulties. PMID:27698865

Intracerebral abscess: A complication of severe cystic fibrosis lung disease

OpenAIRE

Fenton, Mark E; Cockcroft, Donald W; Gjevre, John A

2008-01-01

Intracerebral abscess is an uncommon complication of severe cystic fibrosis lung disease. The present report describes a case of fatal multiple intracerebral abscesses in a patient with a severely bronchiectatic, nonfunctioning right lung and chronic low-grade infection. The patient was previously turned down for pneumonectomy. Intracerebral abscess in cystic fibrosis and the potential role of pneumonectomy in the present patient are discussed.

Asymptomatic cystic changes in the brain of children after cranial irradiation: frequency, latency, and relationship to age

International Nuclear Information System (INIS)

Kitajima, Mika; Hirai, Toshinori; Maruyama, Natsuki; Yamura, Masayuki; Hayashida, Yoshiko; Baba, Yuji; Yamashita, Yasuyuki; Murakami, Ryuji; Korogi, Yukunori; Nakamura, Hideo; Kuratsu, Jun-ichi

2007-01-01

Although radiation therapy plays an important role in the management of children with brain tumors, radiation-induced brain damage sometimes occurs after radiation therapy. In some pediatric patients who had undergone cranial radiation therapy, we noticed cystic changes in the brain on follow-up MRI. The purpose of this study was to determine the frequency, latency, relationship with patient age, radiation dose, and serial change in the cystic changes in children after cranial irradiation. We retrospectively studied MRI in 33 children who had undergone cranial irradiation for their primary brain tumors. Postirradiation cystic change in the brain on follow-up MRI was defined as a well-demarcated, oval-shaped, CSF-like signal intensity area, and no contrast enhancement. Of the 33 patients, 6 (18.2%) had one or more cystic lesions. The latency of the cystic changes ranged from 1.5 to 7 years (mean 2.6 years). Cystic changes were found in the subcortical, periventricular and other deep white matter and the basal ganglia. The size of the lesions ranged from 1 to 10 mm at their first appearance; eight cystic lesions increased in size. None the cystic lesions reduced in size or resolved with time and none required intervention. All patients with cystic changes had received irradiation when they were 6 years of age or younger. The cystic changes occurred within the radiation field in patients treated with a radiation dose of 36 Gy or more. Asymptomatic brain parenchymal cystic changes appear to occur in children who have undergone cranial irradiation at 6 years of age or younger. (orig.)

Transcatheter Arterial Embolization of Cystic Artery Pseudoaneurysm in Acalculous Cholecystitis: A Case Report

Energy Technology Data Exchange (ETDEWEB)

Lee, Hyung Ook; Lee, Young Hwan [Dept. of Radiology, Daegu Catholic University College of Medicine, Daegu (Korea, Republic of); Kim, Young Hwan [Dept. of Diagnostic Radiology, Dongsan Medical Center, Keimyung University College of Medicine, Daegu (Korea, Republic of)

2011-04-15

A pseudoaneurysm of the cystic artery is a rare complication of cholecystitis, and is manifested by hemobilia or hematemesis. An early diagnosis is required for the successful treatment by cholecystectomy and ligation of the cystic artery. Herein, we report a case of a pseudoaneurysm of the cystic artery diagnosed by color Doppler ultrasonography and CT, and successfully treated by transcatheter arterial embolization with N-butyl cyanoacrylate in a high-risk surgical patient.

An evidence-based review for the management of cystic pancreatic lesions

International Nuclear Information System (INIS)

Planner, A.C.; Anderson, E.M.; Slater, A.; Phillips-Hughes, J.; Bungay, H.K.; Betts, M.

2007-01-01

The incidental finding of pancreatic cysts is becoming more common because of the increased use of cross-sectional imaging. As a result, the perspective from historical series of symptomatic patients is not always applicable to the current cohort of patients with cystic lesions in their pancreas. In this review, the characteristic radiological features that aid diagnosis are highlighted, and the complementary role of different imaging methods and the appropriate use of tissue sampling are identified. Based on the literature regarding the diagnostic role of imaging in characterizing cystic pancreatic lesions, it is possible to recommend a practical imaging algorithm for the diagnosis of cystic pancreatic lesions

Intestinal permeability to [51Cr]EDTA in children with cystic fibrosis

International Nuclear Information System (INIS)

Leclercq-Foucart, J.; Forget, P.; Sodoyez-Goffaux, F.; Zappitelli, A.

1986-01-01

Intestinal permeability was investigated in 14 children with cystic fibrosis making use of [ 51 Cr]EDTA as probe molecule. Ten normal young adults and 11 children served as controls. After oral administration of [ 51 Cr]EDTA, 24 h urine was collected. Urinary radioactivity was calculated and results expressed as percentage of oral dose excreted in 24 h urine. Mean and SEM were as follows: 2.51 +/- 0.21, 2.35 +/- 0.24, and 13.19 +/- 1.72 for control children, normal adults, and cystic fibrosis patients, respectively. The permeability differences between cystic fibrosis patients and either control children or control adults are significant (p less than 0.001)

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

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 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 an expert panel of specialists to develop consensus recommendations for the screening, investigation, diagnosis and management of NTM pulmonary disease in individuals with CF. Nineteen experts were invited to participate in the recommendation development process. Population, Intervention, Comparison, Outcome (PICO) methodology and systematic literature reviews were employed to inform draft recommendations. An anonymous voting process was used by the committee to reach consensus. All committee members were asked to rate each statement on a scale of: 0, completely disagree, to 9, completely agree; with 80% or more of scores between 7 and 9 being considered 'good' agreement. Additionally, the committee solicited feedback from the CF communities in the USA and Europe and considered the feedback in the development of the final recommendation statements. Three rounds of voting were conducted to achieve 80% consensus for each recommendation statement. Through this process, we have generated a series of pragmatic, evidence-based recommendations for the screening, investigation, diagnosis and treatment of NTM infection in individuals with CF as an initial step in optimising management for this challenging condition. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/

Abdominal manifestations of cystic fibrosis in adults: a review

International Nuclear Information System (INIS)

Constantine, S.; Au, V.W.K.; Slavotinek, J.P.

2004-01-01

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

Multilocular cystic renal cell carcinoma: imaging and clinical correlation

International Nuclear Information System (INIS)

Xu Yong; Zhang Sheng

2013-01-01

Multilocular cystic renal cell carcinoma (MCRCC) is a subtype of clear cell renal cell carcinoma and has mild clinical symptoms and a favorable prognosis. Accordingly, nephron-sparing surgery is recommended as a therapeutic strategy. If histologic subtype of MCRCC can be predicted preoperatively with an acceptable level of accuracy, it may be important in predicting prognosis and make clinical management. Most MCRCCs show characteristic cross-sectional imaging findings and permit accurate diagnosis before the treatment. Cross -sectional imaging of MCRCC reveals a well -defined multilocular cystic mass with irregularly enhanced thickened septa and without enhanced intracystic solid nodule. It is often classified as Bosniak classification Ⅲ , which is significantly different from that of other renal cystic masses. The clinical, pathologic, and radiologic features of MCRCC were discussed and illustrated in this article. The role of the imaging preoperative evaluation for MCRCC, and management implications were emphasized. (authors)

Concurrent Multilocular Cystic Renal Cell Carcinoma and Leiomyoma in the Same Kidney: Previously Unreported Association

Directory of Open Access Journals (Sweden)

Min Su Cheong

2010-07-01

Full Text Available We present an unusual case of concurrent occurrence of a multilocular cystic renal cell carcinoma and a leiomyoma in the same kidney of a patient with no evident clinical symptoms. A 38-year-old man was found incidentally to have a cystic right renal mass on computed tomography. Laparoscopic radical nephrectomy was performed under a preoperative diagnosis of cystic renal cell carcinoma. Histology revealed a multilocular cystic renal cell carcinoma and a leiomyoma. This is the first report of this kind of presentation.

Cystic Lesions in the Greater Tuberosity of the Humerus: The Relation to Rotator Cuff Tears and Age

International Nuclear Information System (INIS)

Kim, Gang Deuk; Oh, Jung Taek

2008-01-01

This study was designed to investigate the location of cystic lesions in the greater tuberosity of the humerus and the relationship to rotator cuff tears and age. A total of 78 patients (age range, 19-82 years; mean age, 51 years) who underwent arthroscopy or open surgery after MR arthrography (MRA) for a painful shoulder were enrolled in the study. The location of the cystic lesions were classified as 'A' for a supraspinatus insertion site, as 'C' for an infraspinatus insertion site, as 'B' for both a supraspinatus and infraspinatus insertion site, as 'BG' for a site posterior to the bicipital groove and as 'P' for a site at the bare area of the humeral head. The location of cystic lesions and supraspinatus and infraspinatus tears were evaluated on MRA. Statistical analyses used the chi-squared test and logistic regression. 'BG' and 'A' cystic lesions were related to the presence of a supraspinatus tear, 'C' cystic lesions were related to the presence of an infraspinatus tear and 'B' cystic lesions were related to the presence of both supraspinatus and infraspinatus tears (p < 0.05). 'P' cystic lesions were not related to the presence of rotator cuff tears. The incidence of cystic lesions increased with age, but with no statistical correlation. Cystic lesions at the supraspinatus and infraspinatus insertion sites are useful to predict the presence of a rotator cuff tear, but cystic lesions were not age related

Dynamic multislice helical CT of maxillomandibular lesions. Distinction of ameloblastomas from other cystic lesions

Energy Technology Data Exchange (ETDEWEB)

Tozaki, Mitsuhiro; Hayashi, Katsuhiko; Fukuda, Kunihiko [Jikei Univ., Tokyo (Japan). School of Medicine

2001-10-01

The purpose of this study was to evaluate the clinical usefulness of dynamic multislice helical CT in differentiating ameloblastoma from other cystic lesions in cases of maxillomandibular cystic lesions. The study included 32 patients with maxillomandibular cystic lesions (ameloblastoma [n=6], myxofibroma [n=1], odontogenic keratocyst [n=3], dentigerous cyst [n=11], radicular cyst [n=11], and paradental cyst [n=2]). Dynamic study was performed before and 30 sec, and 90 sec after intravenous contrast medium administration. CT density values and percentage of density increase were calculated at 30 and 90 sec. In five cases of ameloblastoma, a rapidly enhancing area was detected within the cystic lesions at 30 sec, while no apparent rapid enhancement was seen in the other cystic lesions. Three cysts showed gradual enhancement in the marginal area at 90 sec. Comparing ameloblastoma and other kinds of cysts, we found significant differences in the percentage of density increase at 30 sec (p<0.01) and 90 sec (p<0.05). Dynamic multislice helical CT is useful in the diagnosis of cystic lesions of the maxillomandibular region, especially in the detection of neovascularities in ameloblastoma. (author)

Dynamic multislice helical CT of maxillomandibular lesions. Distinction of ameloblastomas from other cystic lesions

International Nuclear Information System (INIS)

Tozaki, Mitsuhiro; Hayashi, Katsuhiko; Fukuda, Kunihiko

2001-01-01

The purpose of this study was to evaluate the clinical usefulness of dynamic multislice helical CT in differentiating ameloblastoma from other cystic lesions in cases of maxillomandibular cystic lesions. The study included 32 patients with maxillomandibular cystic lesions (ameloblastoma [n=6], myxofibroma [n=1], odontogenic keratocyst [n=3], dentigerous cyst [n=11], radicular cyst [n=11], and paradental cyst [n=2]). Dynamic study was performed before and 30 sec, and 90 sec after intravenous contrast medium administration. CT density values and percentage of density increase were calculated at 30 and 90 sec. In five cases of ameloblastoma, a rapidly enhancing area was detected within the cystic lesions at 30 sec, while no apparent rapid enhancement was seen in the other cystic lesions. Three cysts showed gradual enhancement in the marginal area at 90 sec. Comparing ameloblastoma and other kinds of cysts, we found significant differences in the percentage of density increase at 30 sec (p<0.01) and 90 sec (p<0.05). Dynamic multislice helical CT is useful in the diagnosis of cystic lesions of the maxillomandibular region, especially in the detection of neovascularities in ameloblastoma. (author)

Epidemiological evaluation regarding the role of cystic fibrosis as a risk factor for child malnutrition.

Science.gov (United States)

Florescu, Laura; Paduraru, Dana Teodora Anton; Mîndru, Dana Elena; Temneanu, Oana Raluea; Petrariu, F D; Matei, Mioara Calipsoana

2014-01-01

Cystic fibrosis (CF) is the most common monogenic autosomal recessive disorder with progressive chronic evolution which is potentially lethal. Poor growth is a characteristic of children suffering from cystic fibrosis. A poor nutritional status is an independent risk factor for inadequate survival in cystic fibrosis and is associated with disease complications. The appropriate nutritional management is an important part of the treatment so that the patient with cystic fibrosis can achieve normal growth and development and maintain the best possible health status. A balanced diet supplemented with snacks high in fat and calories is necessary to increase the caloric intake in children with cystic fibrosis. Children with cystic fibrosis have higher caloric needs than healthy children of the same age and sex. Malnutrition in CF is multifactorial. Cystic fibrosis is a complex multisystem disorder affecting mainly the gastrointestinal tract and respiratory system. In the past, malnutrition was an inevitable consequence of disease progression, leading to poor growth, impaired respiratory muscle function, decreased exercise tolerance and immunological impairment. A positive association between body weight and height and survival has been widely reported. The energy requirements of patients with CF vary widely and generally increase with age and disease severity. Cystic fibrosis remains a paediatric disorder which is often underdiagnosed but which, if therapeutically managed properly (by means of drug therapy as well as by appropriate physiotherapy techniques), can lead to improved quality of life and, thus, to a bigger life expectancy.

Huge music archives on mobile devices

DEFF Research Database (Denmark)

Blume, H.; Bischl, B.; Botteck, M.

2011-01-01

The availability of huge nonvolatile storage capacities such as flash memory allows large music archives to be maintained even in mobile devices. With the increase in size, manual organization of these archives and manual search for specific music becomes very inconvenient. Automated dynamic...... organization enables an attractive new class of applications for managing ever-increasing music databases. For these types of applications, extraction of music features as well as subsequent feature processing and music classification have to be performed. However, these are computationally intensive tasks...... and difficult to tackle on mobile platforms. Against this background, we provided an overview of algorithms for music classification as well as their computation times and other hardware-related aspects, such as power consumption on various hardware architectures. For mobile platforms such as smartphones...

Adenoid cystic carcinoma of uterine cervix in a young patient

Directory of Open Access Journals (Sweden)

Seth Ankit

2009-10-01

Full Text Available Adenoid cystic carcinoma of uterine cervix is a rare tumor. Its origin is debatable. It has a high incidence in postmenopausal women but rarely can develop in patients under 40. An association with squamous cell carcinoma has been described. We report a case of adenoid cystic carcinoma of the endocervical canal with foci of squamous cell carcinoma in a 34-year-old suffering from menorrhagia associated with blood-stained vaginal discharge. Per vaginum and per speculum examination revealed a growth. Cervical biopsy showed bits of tissue, suggesting adenoid cystic carcinoma. Patient was operated upon and uterus with cervix sent for histopathological examination. We report this case because of its rarity, particularly in young patients, with description of illustrative pathology and discussion on the histological diagnosis.

Decreased coenzyme Q10 concentration in plasma of children with cystic fibrosis

NARCIS (Netherlands)

Oudshoorn, J.H.; Lecluse, A.L.Y.; Berg, R. van den; Vaes, W.H.J.; Laag, J. van der; Houwen, R.H.J.

2006-01-01

OBJECTIVES: Coenzyme Q10 (CoQ10) is an effective lipophilic antioxidant and protects against lipid peroxidation by scavenging radicals. Patients with cystic fibrosis generally have fat malabsorption; thus, we hypothesized that overall plasma CoQ10 concentration in pediatric patients with cystic

Adenoid cystic carcinoma of child: a rare case.

Science.gov (United States)

Mathai, Meera; Sherubin, J Eugenia; Agnihotri, Pg; Sangeetha, Gs

2014-01-01

Adenoid cystic carcinoma (ACC) is the second most common malignant tumor affecting both major and minor salivary glands. Clinically, it is a slowly growing tumor with high propensity for local invasion, recurrence and distant metastasis. It is predominantly seen in the ffith and sixth decades of life. Here, we report a rare case of ACC affecting the right maxilla of a 12-year-old girl. How to cite this article: Mathai M, Sherubin JE, Agnihotri PG, Sangeetha GS. Adenoid Cystic Carcinoma of Child: A Rare Case. Int J Clin Pediatr Dent 2014;7(3):206-208.

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.

Innovating cystic fibrosis clinical trial designs in an era of successful standard of care therapies.

Science.gov (United States)

VanDevanter, Donald R; Mayer-Hamblett, Nicole

2017-11-01

Evolving cystic fibrosis 'standards of care' have influenced recent cystic fibrosis clinical trial designs for new therapies; care additions/improvements will require innovative trial designs to maximize feasibility and efficacy detection. Three cystic fibrosis therapeutic areas (pulmonary exacerbations, Pseudomonas aeruginosa airway infections, and reduced cystic fibrosis transmembrane conductance regulator [CFTR] protein function) differ with respect to the duration for which recognized 'standards of care' have been available. However, developers of new therapies in all the three areas are affected by similar challenges: standards of care have become so strongly entrenched that traditional placebo-controlled studies in cystic fibrosis populations likely to benefit from newer therapies have become less and less feasible. Today, patients/clinicians are more likely to entertain participation in active-comparator trial designs, that have substantial challenges of their own. Foremost among these are the selection of 'valid' active comparator(s), estimation of a comparator's current clinical efficacy (required for testing noninferiority hypotheses), and effective blinding of commercially available comparators. Recent and future cystic fibrosis clinical trial designs will have to creatively address this collateral result of successful past development of effective cystic fibrosis therapies: patients and clinicians are much less likely to accept simple, placebo-controlled studies to evaluate future therapies.

Duodenum-preserving total pancreatic head resection for benign cystic neoplastic lesions.

Science.gov (United States)

Beger, Hans G; Schwarz, Michael; Poch, Bertram

2012-11-01

Cystic neoplasms of the pancreas are diagnosed frequently due to early use of abdominal imaging techniques. Intraductal papillary mucinous neoplasm, mucinous cystic neoplasm, and serous pseudopapillary neoplasia are considered pre-cancerous lesions because of frequent transformation to cancer. Complete surgical resection of the benign lesion is a pancreatic cancer preventive treatment. The application for a limited surgical resection for the benign lesions is increasingly used to reduce the surgical trauma with a short- and long-term benefit compared to major surgical procedures. Duodenum-preserving total pancreatic head resection introduced for inflammatory tumors in the pancreatic head transfers to the patient with a benign cystic lesion located in the pancreatic head, the advantages of a minimalized surgical treatment. Based on the experience of 17 patients treated for cystic neoplastic lesions with duodenum-preserving total pancreatic head resection, the surgical technique of total pancreatic head resection for adenoma, borderline tumors, and carcinoma in situ of cystic neoplasm is presented. A segmental resection of the peripapillary duodenum is recommended in case of suspected tissue ischemia of the peripapillary duodenum. In 305 patients, collected from the literature by PubMed search, in about 40% of the patients a segmental resection of the duodenum and 60% a duodenum and common bile duct-preserving total pancreatic head resection has been performed. Hospital mortality of the 17 patients was 0%. In 305 patients collected, the hospital mortality was 0.65%, 13.2% experienced a delay of gastric emptying and a pancreatic fistula in 18.2%. Recurrence of the disease was 1.5%. Thirty-two of 175 patients had carcinoma in situ. Duodenum-preserving total pancreatic head resection for benign cystic neoplastic lesions is a safe surgical procedure with low post-operative morbidity and mortality.

Restoring Cystic Fibrosis Transmembrane Conductance Regulator Function Reduces Airway Bacteria and Inflammation in People with Cystic Fibrosis and Chronic Lung Infections.

Science.gov (United States)

Hisert, Katherine B; Heltshe, Sonya L; Pope, Christopher; Jorth, Peter; Wu, Xia; Edwards, Rachael M; Radey, Matthew; Accurso, Frank J; Wolter, Daniel J; Cooke, Gordon; Adam, Ryan J; Carter, Suzanne; Grogan, Brenda; Launspach, Janice L; Donnelly, Seamas C; Gallagher, Charles G; Bruce, James E; Stoltz, David A; Welsh, Michael J; Hoffman, Lucas R; McKone, Edward F; Singh, Pradeep K

2017-06-15

Previous work indicates that ivacaftor improves cystic fibrosis transmembrane conductance regulator (CFTR) activity and lung function in people with cystic fibrosis and G551D-CFTR mutations but does not reduce density of bacteria or markers of inflammation in the airway. These findings raise the possibility that infection and inflammation may progress independently of CFTR activity once cystic fibrosis lung disease is established. To better understand the relationship between CFTR activity, airway microbiology and inflammation, and lung function in subjects with cystic fibrosis and chronic airway infections. We studied 12 subjects with G551D-CFTR mutations and chronic airway infections before and after ivacaftor. We measured lung function, sputum bacterial content, and inflammation, and obtained chest computed tomography scans. Ivacaftor produced rapid decreases in sputum Pseudomonas aeruginosa density that began within 48 hours and continued in the first year of treatment. However, no subject eradicated their infecting P. aeruginosa strain, and after the first year P. aeruginosa densities rebounded. Sputum total bacterial concentrations also decreased, but less than P. aeruginosa. Sputum inflammatory measures decreased significantly in the first week of treatment and continued to decline over 2 years. Computed tomography scans obtained before and 1 year after ivacaftor treatment revealed that ivacaftor decreased airway mucous plugging. Ivacaftor caused marked reductions in sputum P. aeruginosa density and airway inflammation and produced modest improvements in radiographic lung disease in subjects with G551D-CFTR mutations. However, P. aeruginosa airway infection persisted. Thus, measures that control infection may be required to realize the full benefits of CFTR-targeting treatments.

Agenesis of the gallbladder with hypoplastic cystic duct diagnosed at laparoscopy.

Science.gov (United States)

Kwon, A-Hon; Yanagimoto, Hiroaki; Matsui, Yoichi; Imamura, Atsushi

2006-08-01

An 86-year-old man was admitted to our department with complaints of intermittent upper abdominal pain. Ultrasonography of the abdomen showed dilated extrahepatic bile ducts containing stones; however, the gallbladder was not clearly identified. Magnetic resonance cholangiopancreatography showed dilated extrahepatic ducts and choledocholithiasis without gallbladder visualization. The stone extraction was performed with endoscopic sphincterotomy. Three-dimensional images using spiral-computed tomography after intravenous-infusion cholangiography clearly demonstrated an obstruction of the cystic duct. The patient was scheduled for laparoscopic cholecystectomy. At laparoscopy, the gallbladder fossa was not identified on the undersurface of the liver. Despite a thorough examination of the intrahepatic (left-sided within the lesser omentum), retroperitoneal, retrohepatic (within the falciform ligament), retroduodenal, and retropancreatic areas using laparoscopic ultrasonography, the gallbladder was not found. After careful dissection of the hepatoduodenal ligament, the dilated extrahepatic bile duct and a 1-cm length of hypoplastic cystic duct were found. Gallbladder agenesis is usually accompanied by the lack of the cystic duct. The present case is the third report of gallbladder agenesis with a patent or hypoplastic cystic duct.

Solid and cystic pseudopapillary tumor of the pancreas: A case report

Directory of Open Access Journals (Sweden)

Milošević Bojan Z.

2013-01-01

Full Text Available Introduction. Solid and cystic pseudopapillary tumor of the pancreas is a rare tumor of the pancreas, for the first time described by Frantz et al. in 1959. The majority of patients are young females and most of them are asymptomatic. Case Outline. We report a case of 25-year old woman who was admitted to our institution with abdominal pain and a palpable mass in the left hypochondrial area. US and CT scan revealed a solid and cystic pseudopapillary tumor in the head of the pancreas. The patient was treated by Whipple procedure, modification Longmire-Traverso. There was no metastatic disease either in the liver or peritoneum. Histologically the tumor was diagnosed as a solid and cystic pseudopapillary tumor of the pancreas. Conclusion. The unclear pre-operative diagnoses, together with incidence of potential malignancy as well as good outcome with resection, suggest that all suspected cystic tumors of the pancreas should be resected. The exact diagnosis is based on histological findings. [Projekat Ministarstva nauke Republike Srbije, br. III41007 i br. III41010

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.

Metastatic adenoid cystic carcinoma to the kidney in a young woman.

Science.gov (United States)

Manoharan, M; Gomez, Pablo; Reyes, Martha A; Soloway, Mark S

2006-12-01

The incidence of an adenoid cystic carcinoma on major salivary glands is low, constituting only about 10% to 15% of all parotid malignancies. Cases of delayed metastasis have been reported, but reports of specific renal involvement have not been extensively found. We present the case of a 21-year-old woman with a left renal metastasis from an adenoid cystic carcinoma of the parotid gland that had been surgically treated 7 years previously. The patient underwent successful left radical nephrectomy. During follow-up, the patient developed pulmonary nodules that were metastatic adenoid cystic carcinoma and were surgically excised. At last follow-up, the patient was doing well with no evidence of disease.

Cystic fibroadenoma: report of a rare case with review of literature.

Science.gov (United States)

Bhat, Amoolya; Vijaya, C; Gowda, V S Shankare

2015-01-01

Fibroadenomas with a predominant cystic change are called cystic fibroadenomas. These are extremely rare forms of fibroadenomas and only one case has been reported so far. They are classified under the category of complex fibroadenomas. Complex fibroadenomas are a rare variant of fibroadenomas occurring in elderly females. They are characterized by presence of one of the complex features along with the usual patterns of fibroadenoma such as cysts more than 3 mm, papillary apocrine metaplasia, or sclerosing adenosis. Patients with these lesions have higher chances of developing carcinoma of breast. We present a case of 35 years old lady with a freely mobile mass in the left breast diagnosed as cystic fibroadenoma after thorough histopathological examination of the lesion.

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 by g...... 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....

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

Energy Technology Data Exchange (ETDEWEB)

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

2014-04-15

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

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

Directory of Open Access Journals (Sweden)

Ji-hoon Kim

2014-04-01

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

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

International Nuclear Information System (INIS)

Kim, Ji Hoon

2014-01-01

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

Cystic Lesions in the Greater Tuberosity of the Humerus: The Relation to Rotator Cuff Tears and Age

Energy Technology Data Exchange (ETDEWEB)

Kim, Gang Deuk; Oh, Jung Taek [Wonkwang University Hospital, Iksan (Korea, Republic of)

2008-12-15

This study was designed to investigate the location of cystic lesions in the greater tuberosity of the humerus and the relationship to rotator cuff tears and age. A total of 78 patients (age range, 19-82 years; mean age, 51 years) who underwent arthroscopy or open surgery after MR arthrography (MRA) for a painful shoulder were enrolled in the study. The location of the cystic lesions were classified as 'A' for a supraspinatus insertion site, as 'C' for an infraspinatus insertion site, as 'B' for both a supraspinatus and infraspinatus insertion site, as 'BG' for a site posterior to the bicipital groove and as 'P' for a site at the bare area of the humeral head. The location of cystic lesions and supraspinatus and infraspinatus tears were evaluated on MRA. Statistical analyses used the chi-squared test and logistic regression. 'BG' and 'A' cystic lesions were related to the presence of a supraspinatus tear, 'C' cystic lesions were related to the presence of an infraspinatus tear and 'B' cystic lesions were related to the presence of both supraspinatus and infraspinatus tears (p < 0.05). 'P' cystic lesions were not related to the presence of rotator cuff tears. The incidence of cystic lesions increased with age, but with no statistical correlation. Cystic lesions at the supraspinatus and infraspinatus insertion sites are useful to predict the presence of a rotator cuff tear, but cystic lesions were not age related

CT differentiation of mucin-producing cystic neoplasms of the liver from solitary bile duct cysts.

Science.gov (United States)

Kim, Hyoung Jung; Yu, Eun Sil; Byun, Jae Ho; Hong, Seung-Mo; Kim, Kyoung Won; Lee, Jong Seok; Kim, So Yeon

2014-01-01

The purpose of this study was to identify the CT features required for differentiating mucin-producing cystic neoplasms of the liver (mucinous cystic neoplasms and cyst-forming intraductal papillary neoplasms of the bile duct) from solitary bile duct cysts. CT images of pathologically confirmed mucinous cystic neoplasms (n = 15), cyst-forming intraductal papillary neoplasms of the bile duct (n = 16), and solitary bile duct cysts (n = 31) were reviewed. Analysis of the CT findings included shape, presence of septa, location of septa (peripheral vs central), thickness of septa (thin vs thick), mosaic pattern, mural nodules, intracystic debris, calcification, upstream bile duct dilatation, downstream bile duct dilatation, and communication between a cystic lesion and the bile duct. The maximum size of a cystic lesion and the maximum size of the largest mural nodule were measured. The presence of septa, central septa, mural nodules, upstream bile duct dilatation, and downstream bile duct dilatation were found to be significant CT findings for differentiating mucinous cystic neoplasms and cyst-forming intraductal papillary neoplasms of the bile duct from solitary bile duct cysts (p bile duct were 87% (27 of 31) and 87% (27 of 31), respectively. When two of these five criteria were used in combination, the sensitivity and specificity for diagnosing mucinous cystic neoplasms and cyst-forming intraductal papillary neoplasms of the bile duct were 87% (27 of 31) and 87% (27 of 31), respectively [corrected]. With the use of specific CT criteria, mucin-producing cystic neoplasms of the liver can be differentiated from solitary bile duct cysts with a high degree of accuracy.

Prevalence of cystic macular lesions in patients with Usher II syndrome.

Science.gov (United States)

Walia, S; Fishman, G A; Hajali, M

2009-05-01

To evaluate the prevalence of cystic macular lesions in patients with Usher II syndrome. All Usher type II patients seen in the inherited eye disease clinic at the University of Illinois at Chicago between January 2002 and December 2007 were included (n=76). Each participating patient underwent a detailed clinical examination, including best-corrected visual acuity, slit-lamp biomicroscopy and dilated fundus examination. The presence of cystoid lesions was determined by optical coherence tomography (OCT), fundus fluorescein angiogram (FFA), fundus photographs and/or clinical examination. A cystic-appearing macular change was observed in at least one eye in 19 out of the 76 patients (25%), 13 on the basis of OCT, five using FFA (two solely with the use of FFA and three based on clinical notes and FFA findings) and one based solely on clinical notes. Of the 18 patients with CME, determined by OCT or FFA, five (27.8%) showed either a funduscopically normal-appearing macula (n=4) or an atrophic appearing macular change (n=1). One-fourth of our total cohort of Usher II patients had cystic macular lesions. Moreover, a funduscopically normal-appearing macula was observed in 22% (n=4) of our 18 patients with cystic-appearing macular lesions on OCT and/or FFA testing. On the basis of the reasonably high prevalence of cystic macular lesions in our cohort, it would seem prudent to evaluate Usher II patients for the presence of cystoid macular oedema.

Cystic synovial sarcomas: imaging features with clinical and histopathologic correlation

Energy Technology Data Exchange (ETDEWEB)

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

2003-12-01

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

Cystic synovial sarcomas: imaging features with clinical and histopathologic correlation

International Nuclear Information System (INIS)

Nakanishi, Hirofumi; Araki, Nobuhito; Sawai, Yuka; Kudawara, Ikuo; Mano, Masayuki; Ishiguro, Shingo; Ueda, Takafumi; Yoshikawa, Hideki

2003-01-01

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

Internal irradiation for cystic craniopharyngioma

International Nuclear Information System (INIS)

Kobayashi, Tatsuya; Kageyama, Naoki

1979-01-01

Internal irradiation with P-32 chromic phosphate and Au-198 colloid was used to treat cystic craniopharyngioma. A newly developed dosimetric formula, by which the radiation dose can be calculated simultaneously at the cyst wall and at a point far from the radioactive source and the untoward effect of irradiation on surrounding brain tissue can be eliminated, especially in cases in which the wall is thin and can be penetrated by beta emission, was used. Radioactive phosphate or gold was injected into eight craniopharyngioma cysts throught the Ommaya reservoir and a tube inserted at the first craniotomy. All cysts were effectively treated for 3 to 33 months, to eliminate fluid retention or collapse. A collapsed cyst was removed at the second craniotomy and irradiation was histologically shown to be effective. Oculomotor palsy, a side effect of irradiation, occurred 10 days after the injection of 5 mc of P-32 chromic phosphate only in a case of small cysts (5.0 ml) in the supra- and intracellular regions. The thickness of the cyst wall was less than 0.5 mm and the oculomotor nerves were thought to adhere to the wall. Not only the amount of wall dose but also the thickness of the wall and localization of the cyst are important factors in internal irradiation. Sufficient and safer doses which kill tumor cells in the wall and have no side effects, are 9,000 to 30,000 rad. Internal irradiation can be used to treat large cysts of more than 10 ml which are supposedly difficult to remove radically and or multiple cysts. It is effective not only for cystic craniopharyngioma but also for intracrania cystic tumors other than craniopharyngioma, if dosimetry is accurate. (J.P.N.)

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....

61 HUGE BENIGN GRANULOSA CELL TUMOUR IN A 61 YEAR ...

African Journals Online (AJOL)

Dr. E. P. Gharoro

peritoneal cavity, huge right ovarian cyst measuring 37cm/29cm as in figure 1a, weighing 8.3 kg with a thick smooth wall without excrescences on surface. ... is released in the blood during pregnancy and is produced in other conditions such as endometriosis, fibroids and diverticulitis. It is useful in monitoring therapy.

Cholecystokinin cholescintigraphic findings in the cystic duct syndrome

International Nuclear Information System (INIS)

Fink-Bennett, D.; DeRidder, P.; Kolozsi, W.; Gordon, R.; Rapp, J.

1985-01-01

Fourteen patients with a cystic duct syndrome (CDS) underwent cholecystokinin (CCK) cholescintigraphy. All patients presented with persistent postprandial right upper quadrant pain and biliary colic. None of the patients had an abnormal oral cholecystography, gallbladder (GB) ultrasound exam or upper GI series. Each patient received 5 mCi of technetium-99m disofenin. When the GB maximally filled, 0.02 microgram/kg CCK was administered (3 min) intravenously. Background corrected gallbladder ejection fractions (GBEFs) were determined every 5 min X 4 by rationing the pre-CCK GB counts minus post-CCK GB counts to pre-CCK GB counts. GBEFs were: 12% (3 patients), 17% (2), 0%, 1.3%, 3%, 4%, 6%, 11%, 14%, 18.5%, and 22% (1 each). All patients underwent a surgical exploration and all had macro- or microscopically abnormal cystic ducts with (12 patients) or without (2 patients) concomitant chronic cholecystitis. No patient with a partially occluded cystic duct with or without concomitant chronic cholecystitis had an ejection fraction that exceeded 22%. In an appropriate clinical setting, a low EF response to CCK should alert the physician to the presence of either chronic acalculous cholecystitis, CDS, or the combination of both

The lower airway microbiota in early cystic fibrosis lung disease: a longitudinal analysis.

Science.gov (United States)

Frayman, Katherine B; Armstrong, David S; Carzino, Rosemary; Ferkol, Thomas W; Grimwood, Keith; Storch, Gregory A; Teo, Shu Mei; Wylie, Kristine M; Ranganathan, Sarath C

2017-12-01

In infants and young children with cystic fibrosis, lower airway infection and inflammation are associated with adverse respiratory outcomes. However, the role of lower airway microbiota in the pathogenesis of early cystic fibrosis lung disease remains uncertain. To assess the development of the lower airway microbiota over time in infants and young children with cystic fibrosis, and to explore its association with airway inflammation and pulmonary function at age 6 years. Serial, semi-annual bronchoscopies and bronchoalveolar lavage (BAL) procedures were performed in infants newly diagnosed with cystic fibrosis following newborn screening. Quantitative microbiological cultures and inflammatory marker (interleukin 8 and neutrophil elastase) measurements were undertaken contemporaneously. 16S ribosomal RNA gene sequencing was conducted on stored BAL samples. Spirometry results recorded at 6 years of age were extracted from medical records. Ninety-five BAL samples provided 16S ribosomal RNA gene data. These were collected from 48 subjects aged 1.2-78.3 months, including longitudinal samples from 27 subjects and 13 before age 6 months. The lower airway microbiota varied, but diversity decreased with advancing age. Detection of recognised cystic fibrosis bacterial pathogens was associated with reduced microbial diversity and greater lower airway inflammation. There was no association between the lower airway microbiota and pulmonary function at age 6 years. In infants with cystic fibrosis, the lower airway microbiota is dynamic. Dominance of the microbiota by recognised cystic fibrosis bacterial pathogens is associated with increased lower airway inflammation, however early microbial diversity is not associated with pulmonary function at 6 years of age. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.

Inhalation of antibiotics in cystic fibrosis

NARCIS (Netherlands)

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

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

Ursodeoxycholic acid treatment in patients with cystic fibrosis at risk for liver disease.

Science.gov (United States)

Siano, Maria; De Gregorio, Fabiola; Boggia, Bartolo; Sepe, Angela; Ferri, Pasqualina; Buonpensiero, Paolo; Di Pasqua, Antonio; Raia, Valeria

2010-06-01

Meconium ileus has been detected as a risk factor for development of liver disease in cystic fibrosis, with influence on morbidity and mortality. To evaluate the effect of early treatment with ursodeoxycholic acid in patients with cystic fibrosis and meconium ileus to prevent chronic hepatic involvement and to explore the potential role of therapy on clinical outcomes. 26 cystic fibrosis patients with meconium ileus (16 M, mean age 8,4 years, range 3,5-9) were assigned to two groups: group 1 (14 patients) treated early with ursodeoxycholic acid (UDCAe); group 2 (12 patients) treated with ursodeoxycholic acid at the onset of cystic fibrosis liver disease (UDCAd). Anthropometric data, pulmonary function tests, pancreatic status, complications such as diabetes, hepatic involvement and Pseudomonas aeruginosa colonisation were compared among groups. A higher prevalence of cystic fibrosis chronic liver disease was observed in the UDCAd group with a statistically significant difference at 9 years of age (p<0.05). Chronic infection by P. aeruginosa was found in 7% of UDCAe and 33% of UDCAd (p<0.05). No differences were observed in nutritional status and other complications. Early treatment with ursodeoxycholic acid may be beneficial in patients at risk of developing cystic fibrosis chronic liver disease such as those with meconium ileus. Multicentre studies should be encouraged to confirm these data. Copyright 2009 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.

Description and validation of a scoring system for tomosynthesis in pulmonary cystic fibrosis.

Science.gov (United States)

Vult von Steyern, Kristina; Björkman-Burtscher, Isabella M; Höglund, Peter; Bozovic, Gracijela; Wiklund, Marie; Geijer, Mats

2012-12-01

To design and validate a scoring system for tomosynthesis (digital tomography) in pulmonary cystic fibrosis. A scoring system dedicated to tomosynthesis in pulmonary cystic fibrosis was designed. Three radiologists independently scored 88 pairs of radiographs and tomosynthesis examinations of the chest in 60 patients with cystic fibrosis and 7 oncology patients. Radiographs were scored according to the Brasfield scoring system and tomosynthesis examinations were scored using the new scoring system. Observer agreements for the tomosynthesis score were almost perfect for the total score with square-weighted kappa >0.90, and generally substantial to almost perfect for subscores. Correlation between the tomosynthesis score and the Brasfield score was good for the three observers (Kendall's rank correlation tau 0.68, 0.77 and 0.78). Tomosynthesis was generally scored higher as a percentage of the maximum score. Observer agreements for the total score for Brasfield score were almost perfect (square-weighted kappa 0.80, 0.81 and 0.85). The tomosynthesis scoring system seems robust and correlates well with the Brasfield score. Compared with radiography, tomosynthesis is more sensitive to cystic fibrosis changes, especially bronchiectasis and mucus plugging, and the new tomosynthesis scoring system offers the possibility of more detailed and accurate scoring of disease severity. Tomosynthesis is more sensitive than conventional radiography for pulmonary cystic fibrosis changes. The radiation dose from chest tomosynthesis is low compared with computed tomography. Tomosynthesis may become useful in the regular follow-up of patients with cystic fibrosis.

Ultrasonographic findings of low-grade endometrial stromal sarcoma of the uterus with a focus on cystic degeneration

Energy Technology Data Exchange (ETDEWEB)

Park, Ga Eun; Rha, Sung Eun; Oh, Soon Nam; Lee, Ah Won; Lee, Keun Ho; Kim, Mee Ran [Seoul St. Mary' s Hospital, College of Medicine, The Catholic University of Korea, Seoul (Korea, Republic of)

2016-03-15

The goal of this study was to perform a retrospective analysis of the ultrasonographic findings associated with low-grade endometrial stromal sarcoma. Ten pathologically confirmed cases of low-grade endometrial stromal sarcoma at our institution from January 2007 to April 2014 were retrospectively reviewed. All patients underwent a preoperative transvaginal ultrasound. Two radiologists came to a consensus regarding the location, size, margin, and echogenicity of the tumor, as well as the presence of intratumoral cystic degeneration and its extent and configuration. Low-grade endometrial stromal sarcoma manifested as an intramural mass protruding into the endometrial cavity (n=6) or as a purely intramural mass (n=4). The maximal diameter of the lesion ranged from 4 to 9.1 cm (mean, 6.2 cm). The imaging features of low-grade endometrial stromal sarcoma were variable: six cases involved predominantly solid masses containing cystic degeneration, one was a predominantly unilocular cystic mass, two were ill-defined infiltrative solid masses, and one was a well-defined solid mass. Among the seven cases with internal cystic degeneration, five patients showed a multiseptated cystic area or a cystic area with multiple small clusters, while a unilocular cystic area within the tumor was found in two patients. Low-grade endometrial stromal sarcoma is associated with variable ultrasonographic findings with regard to the location, margin, and configuration of the lesion. Multiseptated cystic areas and multiple small areas of cystic degeneration are common.

Congenital cystic adenomatoid malformation of the lung: hazards of delayed diagnosis.

LENUS (Irish Health Repository)

Collins, Anne M

2012-02-01

Congenital cystic adenomatoid malformation is a rare pulmonary developmental anomaly, which typically manifests in neonates and infants. Presentation in adulthood is uncommon, with <60 cases reported in the literature. The majority of cases involve one lobe only. We report a case of type 1 congenital cystic adenomatoid malformation in an adult presenting with a respiratory tract infection and haemoptysis. At thoracotomy, complex cystic masses were noted in the right upper and lower lobes. Lung-sparing surgery, in the form of two segmentectomies and a non-anatomical resection, was performed in order to avoid pneumonectomy. Such presentations may be problematic as potentially incomplete resections may increase the risk of complications and malignant transformation. This suggests the importance of appropriate clinical and radiological follow up.

Microbial ecology and adaptation in cystic fibrosis airways

DEFF Research Database (Denmark)

Yang, Lei; Jelsbak, Lars; Molin, Søren

2011-01-01

Chronic infections in the respiratory tracts of cystic fibrosis (CF) patients are important to investigate, both from medical and from fundamental ecological points of view. Cystic fibrosis respiratory tracts can be described as natural environments harbouring persisting microbial communities...... 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...... colonization of microbial pathogens in CF patients in the context of ecology and evolution will expand our knowledge of the pathogenesis of chronic infections and improve therapeutic strategies....

Rehabilitation of an Advanced Case of Adenoid Cystic Carcinoma

Science.gov (United States)

Volpato, Luiz Evaristo Ricci; Caldas, Lorena Frange; Castro, Paulo Henrique de Souza; de Carvalhosa, Artur Aburad; Volpato, Maria Carmen Palma Faria; Bandéca, Matheus Coelho; Borges, Álvaro Henrique

2015-01-01

Adenoid cystic carcinoma is a cancer of the salivary gland that primarily affects the parotid, submandibular, and accessory salivary glands. Its growth is slow and it has infiltrative nature. A 46-year-old female patient coming from the rural area presented a lesion on the palate and reported pain in the region for three years. After incisional biopsy, and histopathological diagnosis of adenoid cystic carcinoma of the cribriform type of minor salivary gland, superior hemimaxillectomy and adjuvant treatment with radiotherapy and maxillofacial prosthetic rehabilitation were performed. PMID:25709844

Rehabilitation of an Advanced Case of Adenoid Cystic Carcinoma

Directory of Open Access Journals (Sweden)

Luiz Evaristo Ricci Volpato

2015-01-01

Full Text Available Adenoid cystic carcinoma is a cancer of the salivary gland that primarily affects the parotid, submandibular, and accessory salivary glands. Its growth is slow and it has infiltrative nature. A 46-year-old female patient coming from the rural area presented a lesion on the palate and reported pain in the region for three years. After incisional biopsy, and histopathological diagnosis of adenoid cystic carcinoma of the cribriform type of minor salivary gland, superior hemimaxillectomy and adjuvant treatment with radiotherapy and maxillofacial prosthetic rehabilitation were performed.

Anesthesia for Lobectomy in a Pediatric Patient` with Cystic Fibrosis: A Case Report

Directory of Open Access Journals (Sweden)

Seyedhejazi Mahin

2014-07-01

Full Text Available Cystic Fibrosis is the most common autosomal recessive disease which causes a lot of complications and there are some needed operations in lifelong of such patients. Anesthesia in these patients requires particular attention and a full cooperation of surgeon, anesthetist and pediatrician .We reported a case of lobectomy with lung separation in a 4.5 years old child with cystic fibrosis which was performed with minor complications in the hope that an awareness of such situations may turn up similar cases of study. Keywords: Cystic Fibrosis, Anesthesia, Pulmonic Lobectomy

Medullary cystic disease of the kidney: report of a case diagnosed by ultrasonography and computed tomography examinations

International Nuclear Information System (INIS)

Carvalho, Tarcisio Nunes; Araujo Junior, Cyrillo Rodrigues de; Fraguas Filho, Sergio Roberto; Costa, Marlos Augusto Bittencourt; Teixeira, Kim-Ir-Sen Santos; Ribeiro, Flavia Aparecida de Souza

2003-01-01

The terms medullary cystic disease, juvenile nephronophthisis or medullary cystic disease complex refer to a group of similar diseases in which the basic pathological abnormality is progressive renal tubular atrophy with secondary glomerular sclerosis and medullary cystic formation. Medullary cystic disease is an important cause of renal failure in adolescent patients. Imaging methods play a primary role in the diagnosis of these diseases. Cysts are characteristically seen in the renal medulla and cortico medullary junction whereas kidneys may be of normal to small size. In this article we present the ultrasonography and computed tomography findings of a female adolescent patient with characteristic clinical picture of medullary cystic disease. (author)

Churn prediction on huge telecom data using hybrid firefly based classification

Directory of Open Access Journals (Sweden)

Ammar A.Q. Ahmed

2017-11-01

Full Text Available Churn prediction in telecom has become a major requirement due to the increase in the number of telecom providers. However due to the hugeness, sparsity and imbalanced nature of the data, churn prediction in telecom has always been a complex task. This paper presents a metaheuristic based churn prediction technique that performs churn prediction on huge telecom data. A hybridized form of Firefly algorithm is used as the classifier. It has been identified that the compute intensive component of the Firefly algorithm is the comparison block, where every firefly is compared with every other firefly to identify the one with the highest light intensity. This component is replaced by Simulated Annealing and the classification process is carried out. Experiments were conducted on the Orange dataset. It was observed that Firefly algorithm works best on churn data and the hybridized Firefly algorithm provides effective and faster results.

A parallel solver for huge dense linear systems

Science.gov (United States)

Badia, J. M.; Movilla, J. L.; Climente, J. I.; Castillo, M.; Marqués, M.; Mayo, R.; Quintana-Ortí, E. S.; Planelles, J.

2011-11-01

HDSS (Huge Dense Linear System Solver) is a Fortran Application Programming Interface (API) to facilitate the parallel solution of very large dense systems to scientists and engineers. The API makes use of parallelism to yield an efficient solution of the systems on a wide range of parallel platforms, from clusters of processors to massively parallel multiprocessors. It exploits out-of-core strategies to leverage the secondary memory in order to solve huge linear systems O(100.000). The API is based on the parallel linear algebra library PLAPACK, and on its Out-Of-Core (OOC) extension POOCLAPACK. Both PLAPACK and POOCLAPACK use the Message Passing Interface (MPI) as the communication layer and BLAS to perform the local matrix operations. The API provides a friendly interface to the users, hiding almost all the technical aspects related to the parallel execution of the code and the use of the secondary memory to solve the systems. In particular, the API can automatically select the best way to store and solve the systems, depending of the dimension of the system, the number of processes and the main memory of the platform. Experimental results on several parallel platforms report high performance, reaching more than 1 TFLOP with 64 cores to solve a system with more than 200 000 equations and more than 10 000 right-hand side vectors. New version program summaryProgram title: Huge Dense System Solver (HDSS) Catalogue identifier: AEHU_v1_1 Program summary URL:http://cpc.cs.qub.ac.uk/summaries/AEHU_v1_1.html Program obtainable from: CPC Program Library, Queen's University, Belfast, N. Ireland Licensing provisions: Standard CPC licence, http://cpc.cs.qub.ac.uk/licence/licence.html No. of lines in distributed program, including test data, etc.: 87 062 No. of bytes in distributed program, including test data, etc.: 1 069 110 Distribution format: tar.gz Programming language: Fortran90, C Computer: Parallel architectures: multiprocessors, computer clusters Operating system

Fractionated stereotactic radiotherapy for vestibular schwannoma (VS): Comparison between cystic-type and solid-type VS

International Nuclear Information System (INIS)

Shirato, Hiroki; Sakamoto, Touru; Takeichi, Norihito; Aoyama, Hidefumi; Suzuki, Keishiro; Kagei, Kenji; Nishioka, Takashi; Fukuda, Satoshi; Sawamura, Yutaka; Miyasaka, Kazuo

2000-01-01

Purpose: To compare the effectiveness and complications of fractionated stereotactic radiotherapy (SRT) for cystic-type vestibular schwannoma (VS) with those of solid-type VS. Methods and Materials: In 65 patients treated with fractionated SRT between 1991 and 1999, 20 were diagnosed with cystic VS, in which at least one-third of the tumor volume was a cystic component on magnetic resonance imaging (MRI), and 45 were diagnosed with solid VS. Thirty-six Gy to 50 Gy in 20-25 fractions was administered to the isocenter and approximately 80% of the periphery of the tumor. All cystic and solid components were included in the gross tumor volume. The mean follow-up period was 37 months, ranging from 6 to 97 months. Results: The actuarial 3-year rate of no episode of enlargement greater than 2.0 mm was 55% for cystic-type and 75% for solid-type VS; the difference was statistically significant (p 0.023). The actuarial 3-year tumor-reduction (reduction in tumor size greater than 2.0 mm) rates were 93% and 31%, respectively (p = 0.0006). The overall actuarial tumor control rate (no tumor growth greater than 2.0 mm after 2 years or no requirement of salvage surgery) was 92% at 5 years in 44 patients with a follow-up period of 2 or more years. There was no difference in the class hearing preservation rate between cystic VS and solid VS. No permanent trigeminal or facial nerve palsy was observed in either group. Conclusion: Transient tumor enlargement occurs in cystic VS more frequently than in solid-type VS, but the subsequent tumor-reduction rate in cystic VS is better.

Bone cement in the management of cystic tumour defects of bone at ...

African Journals Online (AJOL)

Cystic bony defects are characteristics of bone tumours especially benign ones e.g. Giant cell tumours of bone [GCT] and some metastatic tumours to bone. These patients present late with significant cystic cavities at a time the cost and availability of prosthetic implants to replace these defects sometimes precludes ...

[Endocrine complications of cystic fibrosis in childhood].

Science.gov (United States)

Castanet, M; Wieliczko, M-C

2012-05-01

Since the 20 last years, the median age of survival has dramatically improved in children suffering from cystic fibrosis and complications such as growth retardation, pubertal delay and low bone mineral density are now more often than not observed in affected adolescents. The severity of the disease and the poor nutritional status due to pancreatic insufficiency and malabsorption are commonly implicated but recent data suggest that the disease could also play a role though the alteration of the chlore chanel (CFTR). Furthermore an increase prevalence of glucose intolerance and diabetes due to the progressive β cells destruction is observed in these children that make the life sometimes difficult for these adolescents already affected by an heavy chronic disease. The monitoring of the children should thus now become pluridisciplinary and include regular clinical evaluation of height and pubertal status, mineral bone density by DEXA and OGTT every two years since 10 years of age. Therefore, in addition to the standard treatment of cystic fibrosis is now added the vitamin D supplementation, the subcutaneous insulin therapy and may be the growth hormone that could be a new therapeutic demonstrating beneficial effects in these chronic disease. However further studies need to be performed to improve the management of these new endocrine complications more and more frequent in children and adolescents suffering from cystic fibrosis. Copyright © 2012 Elsevier Masson SAS. All rights reserved.

Congenital cystic lung malformations; Konnatale zystische Lungenfehlbildungen

Energy Technology Data Exchange (ETDEWEB)

Stoever, B.; Scheer, I.; Bassir, C. [Klinik fuer Strahlenheilkunde, Berlin (Germany). Abt. Paediatrische Radiologie, Charite; Mau, H. [Campus Virchow-Klinikum, Klinik fuer Kinderchirurgie, Berlin (Germany); Chaoui, R. [Campus Mitte, Klinik fuer Geburtsmedizin, Berlin (Germany); Henrich, W. [Campus Virchow-Klinikum, Klinik fuer Geburtsmedizin, Berlin (Germany); Schwabe, M. [Campus Mitte, Inst. fuer Pathologie, Berlin (Germany); Wauer, R. [Campus Mitte, Klinik fuer Neonatologie, Berlin (Germany)

2006-04-15

Purpose: The aim of the study concerning congenital cystic lung malformations was to evaluate prenatal diagnoses postnatally to determine prognostic factors as well as to define optimized perinatal management. Materials and Methods: The study is based on 45 prenatal ultrasound examinations depicting fetal cystic lung lesions. 32 of the mothers had follow-up examinations. 5 pregnancies were terminated due to CCAM and additional malformations. Complete regression of the lesions was seen prenatally in 8 cases and postnatally in 5 children. Results: Surgical intervention due to respiratory insufficiency was necessary in 4 neonates. According to the imaging results, CCAM was present in 4 cases and sequestration in 7 patients. No correlation between the imaging findings and the surgical results was found in 3 children: One child suffered from rhadomyoid dysplasia, and in the case of the second child, a left-sided hernia of the diaphragm and additional sequestration were detected. The third child showed AV malformation. The cystic lesions of the 14 children operated upon were proven histologically. The degree of accuracy in the present study was high. Conclusion: Precise perinatal management is warranted in order to determine according to the clinical relevance surgical intervention and to prevent complications after the first year of life. This is performed during the neonatal period for respiratory insufficient neonates and within the first year of life for clinically stable children. (orig.)

Sodium and chlorine levels in hair and nails of healthy and cystic fibrosis children

International Nuclear Information System (INIS)

Olguin, M.T.; Jimenez-Reyes, M.; Pen-Aguilar, M.T.; Sanchez-Aguirre, F.J.

1994-01-01

Sodium and chlorine values are reported in μ/g, as determined by neutron activation analysis in washed hair and nails from healthy and cystic fibrosis children. The values thus determined in cystic fibrosis tended to be higher than those in controls, however statistical differences were not significant (p>0.01). Additional experiments were carried out for comparison between washed and unwashed samples of the cystic fibrosis and control group and only the differences between washed and unwashed cystic fibrosis nails were significant (p<0.01) in both sodium and chlorine values. Analysis of a standard reference milk sample, A-11 from IAEA, for the elements mentioned above gave a good agreement with the certified values. (author) 24 refs.; 6 figs.; 2 tabs

The cystic fibrosis gene: Medical and social implications for heterozygote detection

Energy Technology Data Exchange (ETDEWEB)

Wilfond, B.S.; Fost, N. (Univ. of Wisconsin School of Medicine, Madison (USA))

1990-05-23

The primary goal of mass screening programs for cystic fibrosis carriers should be to allow people to make more informed reproductive decisions. However, previous experience with genetic screening programs, including those for phenylketonuria and sickle cell disease, have revealed complex problems including error, confusion, and stigmatization. These problems could be greater with cystic fibrosis, since more than 8 million Americans may be carriers and entrepreneurial interests can be expected to promote screening in what could become a billion-dollar industry. The present frequency of the detectable mutation ({Delta}F{sub 508}), 75%, will complicate the counseling process. The sensitivity of the test to detect at-risk couples would be 56%. The cost of screening could be as much as $2.2 million for each cystic fibrosis birth avoided. Regardless of improvements in the detection rate, implementation of population screening should be delayed until pilot studies that demonstrate its safety and effectiveness are completed. While studies are in progress, preconception testing should be offered to adult relatives of cystic fibrosis patients as part of a comprehensive program following institutional review board approval for compassionate use.

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...

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.

Multiple-Breath Washout Outcomes Are Sensitive to Inflammation and Infection in Children with Cystic Fibrosis.

Science.gov (United States)

Ramsey, Kathryn A; Foong, Rachel E; Grdosic, Jasmine; Harper, Alana; Skoric, Billy; Clem, Charles; Davis, Miriam; Turkovic, Lidija; Stick, Stephen M; Davis, Stephanie D; Ranganathan, Sarath C; Hall, Graham L

2017-09-01

The lung clearance index is a measure of ventilation distribution derived from the multiple-breath washout technique. The lung clearance index is increased in the presence of lower respiratory tract inflammation and infection in infants with cystic fibrosis; however, the associations during the preschool years are unknown. We assessed the ability of the lung clearance index to detect the presence and extent of lower respiratory tract inflammation and infection in preschool children with cystic fibrosis. Ventilation distribution outcomes were assessed at 82 visits with 58 children with cystic fibrosis and at 38 visits with 31 healthy children aged 3-6 years. Children with cystic fibrosis also underwent bronchoalveolar lavage fluid collection for detection of lower respiratory tract inflammation and infection. Associations between multiple-breath washout indices and the presence and extent of airway inflammation and infection were assessed using linear mixed effects models. Lung clearance index was elevated in children with cystic fibrosis (mean [SD], 8.00 [1.45]) compared with healthy control subjects (6.67 [0.56]). In cystic fibrosis, the lung clearance index was elevated in individuals with lower respiratory tract infections (difference compared with uninfected [95% confidence interval], 0.62 [0.06, 1.18]) and correlated with the extent of airway inflammation. These data suggest that the lung clearance index may be a useful surveillance tool for monitoring the presence and extent of lower airway inflammation and infection in preschool children with cystic fibrosis.

Sonography and computed tomography of splenic nonsystemic lesions

International Nuclear Information System (INIS)

Garretti, L.; Cassinis, M.C.; Gandini, G.

1987-01-01

Differential diagnosis problems are caused by the increased, at time occasional detection of benignant and malignant spleen focal lesions in routine superior abdomen ultrasound (US). The following pathologic conditions were reported in 29 patients with focal lesions (excepted systemic diseases): 6 plain cysts, 1 cystic lymphangioma, 3 echinococcus cysts, 3 abscesses, 7 hematomas and 9 metastases. US examination and clinical data together have permitted the differentiation of cysts from solid nodules but has limits in the characterzation of lesions and in the diagnosis of abscesses. Metastases did non present specific US or CT aspects and have been correctly charaterized only with aid of anamnestical data and for the coexhistence of hepatic lesions. CT has proven superior specificity, compared to US, only in the diagnosis of abscesses and should thus be utilized only in selected cases

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

International Nuclear Information System (INIS)

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

2003-01-01

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

The MRI appearance of cystic lesions around the knee

International Nuclear Information System (INIS)

McCarthy, Catherine L.; McNally, Eugene G.

2004-01-01

This review presents a comprehensive illustrated overview of the wide variety of cystic lesions around the knee. The aetiology, clinical presentation, MRI appearances and differential diagnosis are discussed. Bursae include those related to the patella as well as pes anserine, tibial collateral ligament, semimembranosus-tibial collateral ligament, iliotibial and fibular collateral ligament-biceps femoris. The anatomical extension, imaging features and clinical significance of meniscal cysts are illustrated. Review of ganglia includes intra-articular, extra-articular, intraosseous and periosteal ganglia, highlighting imaging findings and differential diagnoses. The relationship between proximal tibiofibular joint cysts and intraneural peroneal nerve ganglia is discussed. Intraosseous cystic lesions, including insertional and degenerative cysts, as well as lesions mimicking cysts of the knee are described and illustrated. Knowledge of the location, characteristic appearance and distinguishing features of cystic masses around the knee as well as potential imaging pitfalls such as normal anatomical recesses and atypical cyst contents on MR imaging aids in allowing a specific diagnosis to be made. This will prevent unnecessary additional investigations and determine whether intra-articular surgery or conservative management is appropriate. (orig.)

Mullerian papilloma-like proliferation arising in cystic pelvic endosalpingiosis.

Science.gov (United States)

McCluggage, W Glenn; O'Rourke, Declan; McElhenney, Clodagh; Crooks, Michael

2002-09-01

This report describes an unusual epithelial proliferation occurring in pelvic cystic endosalpingiosis. A cyst mass lined by a layer of ciliated epithelial cells involved the posterior surface of the cervix and vagina. The epithelial proliferation within the wall resembled a mullerian papilloma with fibrous and fibrovascular cores lined by bland cuboidal epithelial cells. Other areas had a microglandular growth pattern resembling cervical microglandular hyperplasia, and focally there was a solid growth pattern. Foci of typical endosalpingiosis involved the surface of both ovaries and pelvic soft tissues. The cystic lesion recurred after partial cystectomy and drainage and was followed up radiologically and with periodic fine-needle aspiration. Part of the wall of the cyst removed 11 years after the original surgery showed an identical epithelial proliferation. MIB1 staining showed a proliferation index of less than 5%, contrasting with the higher proliferation index of a typical serous borderline tumor. The differential diagnosis is discussed. As far as we are aware, this is the first report of such a benign epithelial proliferation involving cystic endosalpingiosis. Copyright 2002, Elsevier Science (USA). All rights reserved.

Laryngeal adenoid cystic carcinoma in an adolescent.

Science.gov (United States)

Aydin, Omer; Ustündağ, Emre; Işeri, Mete; Erçin, Cengiz

2008-01-01

Malignant tumors of the larynx are extremely rare in children and adolescents. Adenoid cystic carcinoma accounts for less than 1% of all malignant tumors in the larynx. We presented a 16-year-old girl with subglottic adenoid cystic carcinoma, whose symptoms of prolonged and exacerbating dyspnea had been attributed to asthma at another medical center. Indirect flexible and rigid laryngoscopy revealed a smooth mass in the subglottic region occupying 80% of the airway passage. The lesion was also confirmed by both CT and MRI. There was no lymphadenopathy in the neck. The patient was successfully treated by surgery and postoperative radiotherapy. Pathological staging was T4N0M0 according to the AJCC, 2003. During six years of follow-up, there was no evidence for local recurrence or regional and distant metastasis.

Cystic fibrosis year in review 2016.

Science.gov (United States)

Savant, Adrienne P; McColley, Susanna A

2017-08-01

In this article, we highlight cystic fibrosis (CF) research and case reports published in Pediatric Pulmonology during 2016. We also include articles from a variety of journals that are thematically related to these articles, or are of special interest to clinicians. © 2017 Wiley Periodicals, Inc.

Mature cystic teratomas: Relationship between histopathological ...

African Journals Online (AJOL)

... tumor size, symptoms related to MCT and laterality of the tumor did not differ among the patients according to the MCT contents. Conclusions: Our findings suggest no relationship between the clinical features and histopathological contents of MCTs. Key words: Histopathological contents, mature cystic teratoma, ovarian, ...

Point shear wave elastography of the pancreas in patients with cystic fibrosis: a comparison with healthy controls.

Science.gov (United States)

Pfahler, Matthias Hermann Christian; Kratzer, Wolfgang; Leichsenring, Michael; Graeter, Tilmann; Schmidt, Stefan Andreas; Wendlik, Inka; Lormes, Elisabeth; Schmidberger, Julian; Fabricius, Dorit

2018-02-19

Manifestations of cystic fibrosis in the pancreas are gaining in clinical importance as patients live longer. Conventional ultrasonography and point shear wave elastography (pSWE) imaging are non-invasive and readily available diagnostic methods that are easy to perform. The aim of this study was to perform conventional ultrasonography and obtain pSWE values in the pancreases of patients with cystic fibrosis and to compare the findings with those of healthy controls. 27 patients with cystic fibrosis (13 women/14 men; mean age 27.7 ± 13.7 years; range 9-58 years) and 60 healthy control subjects (30 women/30 men; mean age 30.3 ± 10.0 years; range 22-55 years) underwent examinations of the pancreas with conventional ultrasound and pSWE imaging. Patients with cystic fibrosis have an echogenic pancreatic parenchyma. We found cystic lesions of the pancreas in six patients. pSWE imaging of the pancreatic parenchyma gave significantly lower shear wave velocities in patients with cystic fibrosis than in the control group (1.01 m/s vs 1.30 m/s; p cystic fibrosis than in a healthy control population.

Adenoid cystic carcinoma of the breast

International Nuclear Information System (INIS)

Kallel, R.; Bahri Zouari, I.; Gouiaa, N.; Charfi, S.; Ayadi, L.; Makni, S.; Sellami Boudawara, T.; Daoud, E.; Daoud, J.

2009-01-01

Adenoid cystic carcinoma of the breast is a rare neoplasm, accounting for only 0.1% of all malignant breast tumours. It is more common in women in the sixth decade of their lives and often in the sub areolar area. The clinical criteria is not specific and the radiographic examination showed a benign-appearing tumour. The preoperative diagnosis is possible with fine-needle aspiration cytology. The diagnosis is made by histological examination, presented a difficult differential diagnosis with cribriform carcinoma; so it is necessary to use histochemical or immunohistochemical techniques. The treatment is not well established. It consists of lumpectomy with radiation or mastectomy. Compared to other locations, adenoid cystic carcinoma of the breast has a favorable prognosis. Lymph node involvement or distant metastases seldom occur. The aim of our study is to describe the epidemiological, clinico pathological characteristics, the treatment and the prognosis of this rare type of breast tumour. (authors)

Care for Amish and Mennonite children with cystic fibrosis: a case series

Directory of Open Access Journals (Sweden)

Anbar Ran D

2009-01-01

Full Text Available Abstract Background Published articles have described a lack of willingness to allow preventative measures, as well as other types of modern therapies, as an obstacle to providing medical care for Amish and Mennonite populations. Methods We present data regarding the 12 Amish and Mennonite patients at the SUNY Upstate Medical University Pediatric Cystic Fibrosis Center and three representative case reports. Results Families of patients from these communities receiving care at our Center have accepted preventive therapy, acute medical interventions including home intravenous antibiotic administration, and some immunizations for their children with cystic fibrosis, which have improved the health of our patients. Some have even participated in clinical research trials. Health care education for both the child and family is warranted and extensive. Significant Cystic Fibrosis Center personnel time and fundraising are needed in order to address medical bills incurred by uninsured Amish and Mennonite patients. Conclusion Amish and Mennonite families seeking care for cystic fibrosis may choose to utilize modern medical therapies for their children, with resultant significant improvement in outcome.

Canine Choroid Plexus Tumor with Intracranial Dissemination Presenting as Multiple Cystic Lesions

Directory of Open Access Journals (Sweden)

Trisha J. Oura

2013-01-01

Full Text Available A Miniature Pinscher developed acute blindness and behavioral changes. On magnetic resonance imaging (MRI, there were multiple small intra-axial cystic lesions, and primary differential diagnoses included primary or metastatic neoplasia and neurocysticercosis. These cystic lesions were subsequently diagnosed histopathologically as disseminated choroid plexus carcinoma. This is only the second documented description of this diagnosis in a dog, but both patients had very similar MRI findings. This patient adds to the literature about the MRI characteristics of choroid plexus tumors and indicates that choroid plexus tumor should be considered as a possible cause of small multifocal intra-axial cystic brain lesions in dogs, regardless of whether a primary intraventricular lesion is visible.

A rare case of cystic subepithelial tumor in the stomach: Gastric adenomyoma

Energy Technology Data Exchange (ETDEWEB)

Lee, Ho Seok; Jang, Yun Jin; Heo, Jun [Kyungpook National University Hospital, Daegu (Korea, Republic of)

2015-12-15

Gastric adenomyoma is a rare benign subepithelial tumor, characteristically composed of mucosal structures and a prominent smooth muscle stroma. Because of rarity and the nonspecific computed tomography (CT) features, it is difficult to diagnose gastric adenomyoma before operation. In our case, gastric adenomyoma showed a well-circumscribed cystic subepithelial mass with uneven wall thickness on a CT scan, similar to the findings of former reports. The radiologic differential diagnosis can be narrowed down to several diseases, including duplication cysts, gastritis cystica profunda, brunner's gland hyperplasia and solid tumors with cystic degeneration. Also, adenomyoma could be included in the differential diagnosis of gastric cystic subepithelial masses, especially in the distal part of the stomach.

Retreatment of Recurrent Cystic Craniopharyngioma With Chromic Phosphorus P 32

Science.gov (United States)

Kumar, P.P.; Good, R.R.; Skultety, F.M.; Jones, E.O.; Chu, W.K.

1986-01-01

A cystic craniopharyngioma in a two-year-old boy recurred six months after surgery and postoperative external-beam radiotherapy. Successful retreatment was accomplished with radioisotope injection of 0.5 mCi of chromic phosphorus P 32 into the intracranial cyst, which delivered approximately 300.00 Gy to the cyst wall. The patient's symptoms were relieved, and he is without evidence of disease or cystic fluid accumulation four years after intracavitary 32P irradiation. ImagesFigure 1Figure 2Figure 3 PMID:3735454

A huge renal capsular leiomyoma mimicking retroperitoneal sarcoma

Directory of Open Access Journals (Sweden)

Lal Anupam

2009-01-01

Full Text Available A huge left renal capsular leiomyoma mimicking retroperitoneal sarcoma presented in a patient as an abdominal mass. Computed tomography displayed a large heterogeneous retro-peritoneal mass in the left side of the abdomen with inferior and medial displacement as well as loss of fat plane with the left kidney. Surgical exploration revealed a capsulated mass that was tightly adherent to the left kidney; therefore, total tumor resection with radical left nephrectomy was performed. Histopathology ultimately confirmed the benign nature of the mass. This is the largest leiomyoma reported in literature to the best of our knowledge.

Distinctly visible right upper lobe bronchus on the lateral chest: A clue to adolescent cystic fibrosis

Energy Technology Data Exchange (ETDEWEB)

Reinig, J.W.; Sanchez, F.W.; Thomason, D.M.; Gobien, R.P.

1985-05-01

Radiographic differentiation between cystic fibrosis and asthma presenting in teenagers and young adults can be difficult. Many patients with a late presentation of cystic fibrosis display minimal changes on a chest radiograph. However, a large majority (90%) of cystic fibrosis patients with an essentially normal PA chest radiograph will have a distinctly outlined orifice of right upper lobe bronchus on a lateral chest film as opposed to a small number of asthmatics (25%) or normal patients (18%). This observation correlates well with the pathologic finding that the initial pulmonary involvement in cystic fibrosis is typically in the right upper lobe in adolescents. Teenager or young adult patients presenting with a history of repeated respiratory infections, asthma-like symptoms and a distinctly visible right upper lobe bronchus on a lateral chest film should be sweat-tested to exclude cystic fibrosis.

Clinical presentation of metabolic alkalosis in an adult patient with cystic fibrosis.

Science.gov (United States)

Sweetser, Lisel J; Douglas, James A; Riha, Renata L; Bell, Scott C

2005-03-01

In subtropical and tropical climates, dehydration is common in cystic fibrosis patients with respiratory exacerbations. This may lead to a clinical presentation of metabolic alkalosis with associated hyponatraemia and hypochloraemia. An adult cystic fibrosis patient who presented with a severe respiratory exacerbation accompanied by metabolic alkalosis is presented and the effects of volume correction are reported.

Congenital cystic adenomatoid malformation: case presentation in a two months old infant

International Nuclear Information System (INIS)

Aqrabawi, H.E.; Shabatat, M.; Abbadi, B.M.

2015-01-01

Congenital cystic adenomatoid malformation (CCAM) is a rare abnormality of lung development; it is increasingly detected by the routine ultrasound scan during pregnancy. The severity of the abnormality is very variable. Herein, we present a case of congenital cystic adenomatoid malformation that presented in a two months old infant who had normal initial chest X rays. (author)

Perinatal assessment of hereditary cystic renal diseases: the contribution of sonography

Energy Technology Data Exchange (ETDEWEB)

Avni, Fred E.; Cassart, Marie; Massez, Anne [Erasme Hospital, Department of Medical Imaging, Brussels (Belgium); Garel, Laurent [Sainte Justine Hospital, Department of Paediatric Imaging, Montreal, Quebec (Canada); Eurin, Daniele [Charles Nicolle Hospital, Department of Paediatric Imaging, Rouen (France); Didier, Francois [A. Pinard Regional Maternity Hospital, Department of Paediatric Imaging, Nancy (France); Hall, Michelle [Erasme Hospital, Department of Pediatric Nephrology, Brussels (Belgium); Teele, Rita L. [Starship Children' s Hospital, Department of Radiology, Auckland (New Zealand)

2006-05-15

The aims of this review article were to clarify the steps that may lead to a proper diagnosis of fetal and neonatal renal cystic diseases. All the hereditary cystic diseases are reviewed and a classification is proposed. The various sonographic patterns that can be used to ascertain the diagnosis are also reviewed. Finally, tables with differential diagnoses are presented to help the reader in the work-up of such pathologies. (orig.)

Cost Effectiveness of Screening Individuals With Cystic Fibrosis for Colorectal Cancer

OpenAIRE

Gini, A. (Andrea); Zauber, Ann; Cenin, Dayna R.; Omidvari, A.-H. (Amir-Houshang); Hempstead, S.E. (Sarah E.); Fink, A.K. (Aliza K.); Lowenfels, A.B. (Albert B.); Lansdorp-Vogelaar, Iris

2018-01-01

textabstractBackground & Aims: Individuals with cystic fibrosis are at increased risk of colorectal cancer (CRC) compared with the general population, and risk is higher among those who received an organ transplant. We performed a cost-effectiveness analysis to determine optimal CRC screening strategies for patients with cystic fibrosis. Methods: We adjusted the existing Microsimulation Screening Analysis-Colon model to reflect increased CRC risk and lower life expectancy in patients with cys...

A correlation study of diagnostic fine-needle aspiration with histologic diagnosis in cystic neck lesions.

Science.gov (United States)

Moatamed, Neda A; Naini, Bita V; Fathizadeh, Payman; Estrella, Julie; Apple, Sophia K

2009-10-01

The clinical diagnosis of a mass in the neck region encompasses a wide spectrum of differential diagnosis. Fine-needle aspiration is a quick and safe technique, which can provide useful information for initial assessment and further therapeutic measures. The aim of this retrospective study was to evaluate the performance characteristics of the fine-needle aspiration (FNA) in cystic neck lesions. Of 142 patients with FNA for cystic neck masses during 2002-2007, 92 cases were selected with a follow-up histologic diagnosis, excluding the cystic colloid nodule of the thyroid. The cases were divided into salivary gland cystic neck (37 patients) and non-salivary cystic neck (55 patients) mass groups. False-positive and false-negative diagnoses were applied only to the malignant lesions after confirmation by histopathology. In the first group, nine malignant and 28 benign diagnoses were made by FNA; of which three were false-negative. In the second group, there were nine malignant and 46 benign diagnoses with three false negatives. The overall performance of the FNA showed 76% sensitivity and 100% specificity. In conclusion, FNA of the cystic neck lesions offers an invaluable and highly specific initial diagnostic approach for the management of the patients. (c) 2009 Wiley-Liss, Inc.

Anaesthesia for parturients with severe cystic fibrosis: a case series.

LENUS (Irish Health Repository)

Deighan, M

2014-02-01

Cystic fibrosis affects 1 in 1600-2500 live births and is inherited in an autosomal recessive manner. It primarily involves the respiratory, gastrointestinal and reproductive tracts, with impaired clearance of, and obstruction by, increasingly viscous secretions. Severe respiratory disease, diabetes and gastro-oesophageal reflux may result. Improvements in medical management and survival of cystic fibrosis patients means more are committing to pregnancies. Although guidance for anaesthesia in this patient group is available, management and outcome data associated with more severe cases are sparse. Patients with severe cystic fibrosis require multidisciplinary input and should be managed in a tertiary referral centre. Close monitoring of respiratory function and preoperative optimisation during pregnancy are mandatory. The risk of preterm labour and delivery is increased. Pregnancy and delivery can be managed successfully, even in patients with FEV1 <40% predicted. Neuraxial anaesthesia and analgesia should be the technique of choice for delivery. Postoperative care should be carried out in a critical care setting with the provision of postoperative ventilation if necessary.

Radiological diagnosis of hypernephromas in renal cysts or cystic kidneys

International Nuclear Information System (INIS)

Crone-Muenzebrock, W.; Brassow, F.

1981-01-01

The article presents the roentgenological results obtained in 12 patients with hypernephroma in a renal cyst or cystic kidneys, the hypernephroma having been identified surgically and histologically. The patients had been examined either vial IV pyelogram, sonography, computerized tomography and angiography, or with several of these methods. The renal tumor was identified with the help of sonography, computerized tomography and angiography in all cases. The IV pyelogram failed to produce a conclusive results in 2 cases. The space-occupying growth was wrongly assessed in respect of dignity because of the absence of solid tumor parts in 3 out of 5 cystic space-occupying growths via IV pyelogram, in 2 out of 4 cases via sonography and in 1 out of 4 cases via computerized tomography; these methods yielded the erroneous finding that the hypernephroma was a purely cystic space-occupying growth, whereas angiography yielded the correct diagnosis of the type of hypernephroma in 11 out of 12 patients. (orig.) [de

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.

Cystic echinococcosis in sub-Saharan Africa

NARCIS (Netherlands)

Wahlers, Kerstin; Menezes, Colin N.; Wong, Michelle L.; Zeyhle, Eberhard; Ahmed, Mohammed E.; Ocaido, Michael; Stijnis, Cornelis; Romig, Thomas; Kern, Peter; Grobusch, Martin P.

2012-01-01

Cystic echinococcosis is regarded as endemic in sub-Saharan Africa; however, for most countries only scarce data, if any, exist. For most of the continent, information about burden of disease is not available; neither are data for the animal hosts involved in the lifecycle of the parasite, thus

Nanocellulose, a tiny fiber with huge applications.

Science.gov (United States)

Abitbol, Tiffany; Rivkin, Amit; Cao, Yifeng; Nevo, Yuval; Abraham, Eldho; Ben-Shalom, Tal; Lapidot, Shaul; Shoseyov, Oded

2016-06-01

Nanocellulose is of increasing interest for a range of applications relevant to the fields of material science and biomedical engineering due to its renewable nature, anisotropic shape, excellent mechanical properties, good biocompatibility, tailorable surface chemistry, and interesting optical properties. We discuss the main areas of nanocellulose research: photonics, films and foams, surface modifications, nanocomposites, and medical devices. These tiny nanocellulose fibers have huge potential in many applications, from flexible optoelectronics to scaffolds for tissue regeneration. We hope to impart the readers with some of the excitement that currently surrounds nanocellulose research, which arises from the green nature of the particles, their fascinating physical and chemical properties, and the diversity of applications that can be impacted by this material. Copyright © 2016 Elsevier Ltd. All rights reserved.

Ursodeoxycholic acid for cystic fibrosis-related liver disease.

Science.gov (United States)

Cheng, Katharine; Ashby, Deborah; Smyth, Rosalind L

2017-09-11

Abnormal biliary secretion leads to the thickening of bile and the formation of plugs within the bile ducts; the consequent obstruction and abnormal bile flow ultimately results in the development of cystic fibrosis-related liver disease. This condition peaks in adolescence with up to 20% of adolescents with cystic fibrosis developing chronic liver disease. Early changes in the liver may ultimately result in end-stage liver disease with people needing transplantation. One therapeutic option currently used is ursodeoxycholic acid. This is an update of a previous review. To analyse evidence that ursodeoxycholic acid improves indices of liver function, reduces the risk of developing chronic liver disease and improves outcomes in general in cystic fibrosis. We searched the Cochrane CF and Genetic Disorders Group Trials Register comprising references identified from comprehensive electronic database searches, handsearches of relevant journals and abstract books of conference proceedings. We also contacted drug companies and searched online trial registries.Date of the most recent search of the Group's trials register: 09 April 2017. Randomised controlled trials of the use of ursodeoxycholic acid for at least three months compared with placebo or no additional treatment in people with cystic fibrosis. Two authors independently assessed trial eligibility and quality. The authors used GRADE to assess the quality of the evidence. Twelve trials have been identified, of which four trials involving 137 participants were included; data were only available from three of the trials (118 participants) since one cross-over trial did not report appropriate data. The dose of ursodeoxycholic acid ranged from 10 to 20 mg/kg/day for up to 12 months. The complex design used in two trials meant that data could only be analysed for subsets of participants. There was no significant difference in weight change, mean difference -0.90 kg (95% confidence interval -1.94 to 0.14) based on 30

The therapy for huge goiter together with hyperthyroidism through 131I case studies

International Nuclear Information System (INIS)

He Jianhua; Yu Wencai; Zeng Qingwen; Wu Congjun

2001-01-01

Objective: 214 cases of the treatment of huge goiter with hyperthyroidism are revised to collect clinic material for the improvement of therapy to hyperthyroidism indications through 131 I. Methods: In all of these cases, patients take a full dose of 131 I based on MC Garack's formula for one time. Results: Among them, 154 resolved, accounting for 72%, 139 of the cases were reduced to normal size, which accounted for 64.9% of the patients. Only 114 cases of patients had side-effect, and during one year 12.1% of them have symptoms of hypothyroidism. Conclusion: The statistics shows that 131 I is convenient, safe, well and with reduces suffering from treating huge goiter with hyperthyroidism

In silico search for modifier genes associated with pancreatic and liver disease in Cystic Fibrosis.

Directory of Open Access Journals (Sweden)

Pascal Trouvé

Full Text Available Cystic Fibrosis is the most common lethal autosomal recessive disorder in the white population, affecting among other organs, the lung, the pancreas and the liver. Whereas Cystic Fibrosis is a monogenic disease, many studies reveal a very complex relationship between genotype and clinical phenotype. Indeed, the broad phenotypic spectrum observed in Cystic Fibrosis is far from being explained by obvious genotype-phenotype correlations and it is admitted that Cystic Fibrosis disease is the result of multiple factors, including effects of the environment as well as modifier genes. Our objective was to highlight new modifier genes with potential implications in the lung, pancreatic and liver outcomes of the disease. For this purpose we performed a system biology approach which combined, database mining, literature mining, gene expression study and network analysis as well as pathway enrichment analysis and protein-protein interactions. We found that IFI16, CCNE2 and IGFBP2 are potential modifiers in the altered lung function in Cystic Fibrosis. We also found that EPHX1, HLA-DQA1, HLA-DQB1, DSP and SLC33A1, GPNMB, NCF2, RASGRP1, LGALS3 and PTPN13, are potential modifiers in pancreas and liver, respectively. Associated pathways indicate that immune system is likely involved and that Ubiquitin C is probably a central node, linking Cystic Fibrosis to liver and pancreatic disease. We highlight here new modifier genes with potential implications in Cystic Fibrosis. Nevertheless, our in silico analysis requires functional analysis to give our results a physiological relevance.

Associations of diet and breed with recurrence of calcium oxalate cystic calculi in dogs.

Science.gov (United States)

Allen, Heidi S; Swecker, William S; Becvarova, Iveta; Weeth, Lisa P; Werre, Stephen R

2015-05-15

To evaluate the long-term risk of recurrence of calcium oxalate (CaOx) cystic calculi in dogs of various breeds fed 1 of 2 therapeutic diets. Retrospective cohort study. Animals-135 dogs with a history of CaOx cystic calculi. Medical records for 4 referral hospitals were searched to identify dogs that had had CaOx cystic calculi removed. Owners were contacted and medical records evaluated to obtain information on postoperative diet, recurrence of signs of lower urinary tract disease, and recurrence of cystic calculi. Dogs were grouped on the basis of breed (high-risk breeds, low-risk breeds, and Miniature Schnauzers) and diet fed after removal of cystic calculi (diet A, diet B, and any other diet [diet C], with diets A and B being therapeutic diets formulated to prevent recurrence of CaOx calculi). Breed group was a significant predictor of calculi recurrence (as determined by abdominal radiography or ultrasonography), with Miniature Schnauzers having 3 times the risk of recurrence as did dogs of other breeds. Dogs in diet group A had a lower prevalence of recurrence than did dogs in diet group C, but this difference was not significant in multivariable analysis. Results indicated that Miniature Schnauzers had a higher risk of CaOx cystic calculi recurrence than did dogs of other breeds. In addition, findings suggested that diet may play a role in decreasing recurrence, but future prospective studies are needed to validate these observations.

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

Intralesional saline injection for effective ultrasound-guided aspiration of benign viscous cystic thyroid nodules

International Nuclear Information System (INIS)

Ko, Eun Sook; Shin, Jung Hee; Sung, Jin Yong

2014-01-01

We aimed to evaluate the efficacy and safety of vigorous saline injection for viscous cystic thyroid nodules. Eighteen patients who underwent ultrasound-guided aspiration for viscous cystic thyroid nodules using a saline injection were included in our study. After failing to aspirate the cyst by the usual method, we vigorously injected saline into the cyst in multiple directions to break up and liquefy the viscous cystic contents to enable aspiration. The initial and the residual volume of the nodule were calculated, and the volume reduction rate and the time taken to perform the aspiration were recorded. The mean volume of the cystic nodules before aspiration was 11.0 mL (range, 1.2 to 26.0 mL), while the postaspiration volume was 4.2 mL (range, 0.2 to 14.5 mL). The mean aspirated volume was 63.7% of the initial volume. The mean procedure time was 12.4 minutes (range, 5 to 26 minutes). There were no significant complications related to the procedure. A vigorous saline injection followed by aspiration can be a useful method to aspirate viscous cystic thyroid nodules as a prestep for further intervention or simple management.

Vitamin K supplementation for cystic fibrosis.

Science.gov (United States)

Jagannath, Vanitha A; Thaker, Vidhu; Chang, Anne B; Price, Amy I

2017-08-22

Cystic fibrosis is a genetic disorder which can lead to multiorgan dysfunction. Malabsorption of fat and fat-soluble vitamins (A, D, E, K) may occur and can cause subclinical deficiencies of some of these vitamins. Vitamin K is known to play an important role in both blood coagulation and bone formation. Supplementation with vitamin K appears to be one way of addressing the deficiency, but there is very limited agreement on the appropriate dose and frequency of use of these supplements. This is an updated version of the review. To assess the effects of vitamin K supplementation in people with cystic fibrosis and to determine the optimal dose and route of administration of vitamin K for both routine and therapeutic use. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group's Trials Register comprising references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings.Most recent search: 30 January 2017. Randomised and quasi-randomised controlled trials of all preparations of vitamin K used as a supplement compared to either no supplementation (or placebo) at any dose or route and for any duration, in children or adults diagnosed with cystic fibrosis (by sweat test or genetic testing). Two authors independently screened papers, extracted trial details and assessed their risk of bias. Two trials (total of 32 participants) each lasting one month were included in the review and were assessed as having a moderate risk of bias. One was a dose-ranging parallel group trial in children (aged 8 to 18 years); and the other (with an older cohort) had a cross-over design comparing supplements to no treatment, but no separate data were reported for the first intervention period. Neither of the trials addressed any of the primary outcomes (coagulation, bone formation and quality of life). Both trials reported the restoration of serum vitamin K and undercarboxylated osteocalcin

Huge Tongue Lipoma: A Case Report

Directory of Open Access Journals (Sweden)

Mohammad Ali Damghani

2015-03-01

Full Text Available Introduction: Lipomas are among the most common tumors of the human body. However, they are uncommon in the oral cavity and are observed as slow growing, painless, and asymptomatic yellowish submucosal masses. Surgical excision is the treatment of choice and recurrence is not expected.    Case Report: The case of a 30-year-old woman with a huge lipoma on the tip of her tongue since 3 years, is presented. She had difficulty with speech and mastication because the tongue tumor was filling the oral cavity. Clinical examination revealed a yellowish lesion, measuring 8 cm in maximum diameter, protruding from the lingual surface. The tumor was surgically excised with restoration of normal tongue function and histopathological examination of the tumor confirmed that it was a lipoma.   Conclusion:  Tongue lipoma is rarely seen and can be a cause of macroglossia. Surgical excision for lipoma is indicated for symptomatic relief and exclusion of associated malignancy.

CYSTIC AMELOBLASTOMA: A CLINICO-PATHOLOGIC REVIEW

African Journals Online (AJOL)

a tertiary health care centre. Materials: All cases diagnosed as cystic ameloblastoma in the ..... Unicystic amelobla- stoma. A prognostically distinct entity. Cancer. 1977;40: 2278-2285. 4. Ackermann GL, Altini M, Shear M: The unicystic ameloblastoma: A clinicopathologic study of 57 cases. J Oral Pathol. 1988;17: 541-546. 5.

Unusual fast-growing ovarian cystic teratoma during pregnancy presenting with intracystic fat "floating balls" appearance.

Science.gov (United States)

Donnadieu, Anne Claire; Deffieux, Xavier; Le Ray, Camille; Mordefroid, Marie; Frydman, René; Fernandez, Hervé

2006-12-01

A large ovarian cyst was diagnosed at 22 weeks' of gestation in a 32-year-old woman. The ultrasonographic appearance of the ovarian cyst was unusual with multiple mobile, spherical echogenic structures floating in the cystic mass, called intracystic "fat balls." Right adnexectomy was performed by laparotomy at 28 weeks' of gestation, because of rapid growth and overall size exceeding 20 cm. Pathological examination confirmed ovarian cystic teratoma. Usually, dermoid cysts are slow-growing, even in premenopausal women. The exact mechanism related to the fast growth during pregnancy is unknown. It could be related to an unusual pattern of estrogen (E)/P receptors expression in the cystic teratoma. This case shows that a fast-growing, mature ovarian cystic teratoma may occur during pregnancy.

Diagnosis and Treatment of Endocrine Co-Morbidities in Patients with Cystic Fibrosis

Science.gov (United States)

Siwamogsatham, Oranan; Alvarez, Jessica

2015-01-01

Purpose of review The aim of this review is to provide an update on various relevant endocrine aspects of care in adolescents and adults with cystic fibrosis (CF). Recent findings As life expectancy in CF has continuously improved, endocrine complications have become more apparent. The common endocrine complications include cystic fibrosis related diabetes (CFRD), cystic fibrosis related bone disease, vitamin D deficiency and poor growth and pubertal development. Thyroid and adrenal disorders have also been reported, although the prevalence appears to be less common. Summary Endocrine diseases are an increasingly recognized complication that has a significant impact on the overall health of individuals with CF. This review summarizes the updated screening and management of endocrine diseases in the CF population. PMID:25105995

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

Cystic echinococcosis: prevalence and economic sig- nificance in ...

African Journals Online (AJOL)

tribution and economic significance of small ruminant cystic echinococcosis. A total of 850 small ruminants ..... Echinococcus granulosus infection in Spain. Zoonoses Pub. ... Biological, epidemiological, and clinical aspects of echinococcosis ...

A case report of hepatoma with cystic calcification

Energy Technology Data Exchange (ETDEWEB)

Jeon, Byung Hee; Choi, Sung Wook; Kim, Byung So [Busan National University College of Medicine, Busan (Korea, Republic of)

1974-10-15

A case of hepatoma with cystic calcification radiographically which confirmed by pathological examination, was reported. The patients was 19 years old boy who had abdominal mass and pain in left upper quadrant for 1 month. His family history was not contributary. The upper G-I series revealed slight posterior displacement of the fundus with a cyst like calcification, about 4.5 X 5 cm, in diameter at the left upper quadrant. Liver scanning showed normal concentration of 198{sup A}u on the right lobe but nonvisualization of the left lobe area. Biopsy specimen showed hepatoma cells invading the portal vein and intrahepatic blood vessels, and the cystic structure which was a blood vessel invaded by the tumor consisting of the organized thrombi.

Improving care at cystic fibrosis centers through quality improvement.

Science.gov (United States)

Kraynack, Nathan C; McBride, John T

2009-10-01

Quality improvement (QI) using a clinical microsystems approach provides cystic fibrosis (CF) centers the opportunity to make a significant positive impact on the health of their patients. The availability of center-specific outcomes data and the support of the Cystic Fibrosis Foundation are important advantages for these quality improvement efforts. This article illustrates how the clinical microsystems methodology can improve care delivery and outcomes by describing the gradual application of quality improvement principles over the past 5 years by the CF team at the Lewis Walker Cystic Fibrosis Center at Akron Children's Hospital in Akron, Ohio. Using the example of a project to improve the pulmonary function of the pediatric patients at our center as a framework, we describe the QI process from the initial team-building phase, through the assessment of care processes, standardization of care, and developing a culture of continuous improvement. We outline how enthusiastic commitment from physician leadership, clinical managers and central administration, the availability of coaches, and an appreciation of the importance of measurement, patient involvement, communication, and standardization are critical components for successful process improvement. Copyright Thieme Medical Publishers.

Exhaled nitric oxide in paediatric asthma and cystic fibrosis.

Science.gov (United States)

Lundberg, J O; Nordvall, S L; Weitzberg, E; Kollberg, H; Alving, K

1996-01-01

Nitric oxide (NO) is present in exhaled air of humans. This NO is mostly produced in the upper airways, whereas basal NO excretion in the lower airways is low. Children with Kartagener's syndrome have an almost total lack of NO in nasally derived air, whereas adult asthmatics have increased NO in orally exhaled air. NO excretion was measured in the nasal cavity and in orally exhaled air in 19 healthy children, in 36 age matched subjects with asthma, and in eight children with cystic fibrosis. NO levels in orally exhaled air were similar in controls and in children with cystic fibrosis, at 4.8 (SD 1.2) v 5.8 (0.8) parts per billion (ppb), but were increased in asthmatic children who were untreated or were being treated only with low doses of inhaled steroids (13.8 (2.5) ppb). Nasal NO levels were reduced by about 70% in children with cystic fibrosis compared to controls and asthmatics. Measurements of airway NO release in different parts of the airways may be useful in non-invasive diagnosis and monitoring of inflammatory airway diseases. PMID:8984919

Personalized medicine for cystic fibrosis: establishing human model systems.

Science.gov (United States)

Mou, Hongmei; Brazauskas, Karissa; Rajagopal, Jayaraj

2015-10-01

With over 1,500 identifiable mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene that result in distinct functional and phenotypical abnormalities, it is virtually impossible to perform randomized clinical trials to identify the best therapeutics for all patients. Therefore, a personalized medicine approach is essential. The only way to realistically accomplish this is through the development of improved in vitro human model systems. The lack of a readily available and infinite supply of human CFTR-expressing airway epithelial cells is a key bottleneck. We propose that a concerted two-pronged approach is necessary for patient-specific cystic fibrosis research to continue to prosper and realize its potential: (1) more effective culture and differentiation conditions for growing primary human airway and nasal epithelial cells and (2) the development of collective protocols for efficiently differentiating disease- and patient-specific induced pluripotent stem cells (iPSC) into pure populations of adult epithelial cells. Ultimately, we need a personalized human model system for cystic fibrosis with the capacity for uncomplicated bankability, widespread availability, and universal applicability for patient-specific disease modeling, novel pharmacotherapy investigation and screening, and readily executable genetic modification. © 2015 Wiley Periodicals, Inc.

Cystic pancreatic neuroendocrine tumors (cPNETs: a systematic review and meta-analysis of case series

Directory of Open Access Journals (Sweden)

Luis Hurtado-Pardo

Full Text Available Cystic pancreatic neuroendocrine tumors represent 13% of all neuroendocrine tumors. The aim of this study is to analyze the phenotype and biologic behavior of resected cystic neuroendocrine tumors. A systematic review and meta-analysis were conducted until September 2016 using a search in Medline, Scopus, and EMBASE with the terms "cystic pancreatic endocrine neoplasm", "cystic islets tumors" and "cystic islets neoplasms". From the 795 citations recovered 80 studies reporting on 431 patients were selected. 87.1% (n = 387 were sporadic tumors and 10.3% (n = 40 corresponded to multiple endocrine neoplasia type 1. Were diagnosed incidentally 44.6% (n = 135. Cytology was found to have a sensitivity of 78.5%. Were non-functional tumors 85% (n = 338, and among the functional tumors, insulinoma was the most frequent. According to the European Neuroendocrine Tumor Society staging, 87.8% were limited to the pancreas (I-IIb, and 12.2% were advanced (III-IV. Disease-free survival at 5 years in stages (I-IIIa and (IIIb-IV was 91.5% and 54.2%, respectively; and was significantly lower (p = 0.0001 in functional tumors. In patients with multiple endocrine neoplasia there was a higher incidence of functional (62.5% and multifocal (28.1% tumors. Disease-free survival at 5 and 10 years was 60%. Cystic pancreatic neuroendocrine tumors exhibit phenotypical characteristics which are different to those of solid neuroendocrine tumors.

Antenatal diagnosis of cystic adenomatoid malformation: Effect on patient management

International Nuclear Information System (INIS)

Claiborne, A.K.; McAlister, W.H.; Martin, C.M.; Gast, M.J.

1985-01-01

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. (orig.)

Systematic review of studies on cost-effectiveness of cystic fibrosis carrier testing

Directory of Open Access Journals (Sweden)

Ernesto Andrade-Cerquera

2016-10-01

Full Text Available Introduction: Cystic fibrosis is considered the most common autosomal disease with multisystem complications in non-Hispanic white population. Objective: To review the available evidence on cost-effectiveness of the cystic fibrosis carrier testing compared to no intervention. Materials and methods: The databases of MEDLINE, Embase, NHS, EBM Reviews - Cochrane Database of Systematic Reviews, LILACS, Health Technology Assessment, Genetests.org, Genetsickkids.org and Web of Science were used to conduct a systematic review of the cost-effectiveness of performing the genetic test in cystic fibrosis patients. Cost-effectiveness studies were included without language or date of publication restrictions. Results: Only 13 studies were relevant for full review. Prenatal, preconception and mixed screening strategies were found. Health perspective was the most used; the discount rate applied was heterogeneous between 3.5% and 5%; the main analysis unit was the cost per detected carrier couple, followed by cost per averted birth with cystic fibrosis. It was evident that the most cost-effective strategy was preconception screening associated with prenatal test. Conclusions: A marked heterogeneity in the methodology was found, which led to incomparable results and to conclude that there are different approaches to this genetic test.

False negative newborn screen and neonatal cholestasis in a premature child with cystic fibrosis

NARCIS (Netherlands)

Heidendael, J. F.; Tabbers, M. M.; de Vreede, I.

2014-01-01

Newborn screening for cystic fibrosis enables early diagnosis and treatment, leading to better outcomes for patients with cystic fibrosis. Although the sensitivity of several screening protocols is high, false negative screening results of the newborn patient still occur, which can lead to a

Cystic fibrosis genetics: from molecular understanding to clinical application

Science.gov (United States)

Cutting, Garry R.

2015-01-01

The availability of the human genome sequence and tools for interrogating individual genomes provide an unprecedented opportunity to apply genetics to medicine. Mendelian conditions, which are caused by dysfunction of a single gene, offer powerful examples that illustrate how genetics can provide insights into disease. Cystic fibrosis, one of the more common lethalautosomal recessive Mendelian disorders, is presented here as an example. Recent progress in elucidating disease mechanism and causes of phenotypic variation, as well as in the development of treatments, demonstrates that genetics continues to play an important part in cystic fibrosis research 25 years after the d iscove1y of the disease-causing gene. PMID:25404111

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.

How a huge HEP experiment is designed course

CERN Multimedia

CERN. Geneva HR-FAS

2007-01-01

More than twenty years after the idea of building the LHC machine was discussed in a workshop in Lausanne in 1984 for the first time, it is instructive to look back on the historical process which has led the community to where we are today with four huge detectors being commissioned and eagerly awaiting first beam collisions in 2008. The main design principles, detector features and performance characteristics of the ATLAS and CMS detectors will be briefly covered in these two lectures with, as an interlude, a wonderful DVD from ATLAS outreach depicting how particles interact and are detected in the various components of the experiments.

Vaccines for preventing infection with Pseudomonas aeruginosa in cystic fibrosis.

Science.gov (United States)

Johansen, Helle Krogh; Gøtzsche, Peter C

2015-08-23

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. To assess the effectiveness of vaccination against Pseudomonas aeruginosa in cystic fibrosis. 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). Randomised trials (published or unpublished) comparing Pseudomonas aeruginosa vaccines (oral, parenteral or intranasal) with control vaccines or no intervention in cystic fibrosis. The authors independently selected trials, assessed them and extracted data. Six trials were identified. Two trials were excluded since they were not randomised and one old, small trial because it was not possible to assess whether is was randomised. The three included trials comprised 483, 476 and 37 patients, respectively. No data have been published from one of the large trials, but the company stated in a press release that the trial failed to confirm the results from an earlier study and that further clinical development was suspended. In the other large trial, relative risk for chronic infection was 0.91 (95% confidence interval 0.55 to 1.49), and in the small trial, the risk was also close to one. In the large trial, one patient was reported to have died in the observation period. In that trial, 227 adverse events (4 severe) were registered in the vaccine group and 91 (1 severe) in the control group. In this large trial of a vaccine developed against flagella antigens, antibody titres against the epitopes contained in the vaccine were higher in the vaccine group compared to the placebo group (P Vaccines against

Diagnosis and treatment of pancreatic pseudocysts and cystic tumors based on own material and quoted literature

Directory of Open Access Journals (Sweden)

Grzegorz Ćwik

2013-09-01

Full Text Available Pseudocysts constitute the most basic cystic lesions of the pancreas. Symptomatic cysts may be treated by means of both minimally invasive methods and surgery. Currently, it is believed that approximately 5% of cystic lesions in the pancreas may in fact, be neoplastic cystic tumors. Their presence is manifested by generally irregular multilocular structures, solid nodules inside the cyst or in the pancreatic duct, frequently vascularized, as well as fragmentary thickening of the cystic wall or septation. Aim: The aim of this paper was to present current management, both diagnostic and therapeutic, in patients with pancreatic pseudocysts and cystic tumors. The article has been written based on the material collected and prepared in the author’s Department as well as on the basis of current reports found in the quoted literature. Material and methods, results: In 2000–2012, the Second Department of General, Gastrointestinal and Oncological Surgery of the Alimentary Tract treated 179 patients with cystic lesions in the region of the pancreas. This group comprised 12 cases of cystic tumors and 167 pseudocysts. Twenty-three patients (13.8% were monitored only and 144 received procedural treatment. Out of the latter group, 75 patients underwent drainage procedures and 48 were qualified to endoscopic cystogastrostomy or cystoduodenostomy. The endoscopic procedure was unsuccessful in 11 cases (23%. In a group of patients with a pancreatic cystic tumor (12 patients, 6 of them (50% underwent therapeutic resection of the tumor with adequate fragment of the gland. Conclusions: Endoscopic drainage is an effective and safe method of minimally invasive treatment of pancreatic cysts. The patients who do not qualify to endoscopic procedures require surgical treatment. The differentiation of a neoplasm from a typical cyst is of fundamental significance for the selection of the treatment method.

Psychological interventions for individuals with cystic fibrosis and their families.

Science.gov (United States)

Goldbeck, Lutz; Fidika, Astrid; Herle, Marion; Quittner, Alexandra L

2014-06-18

With increasing survival estimates for individuals with cystic fibrosis, long-term management has become an important focus. Psychological interventions are largely concerned with adherence to treatment, emotional and social adaptation and health-related quality of life. We are unaware of any relevant systematic reviews. To determine whether psychological interventions for people with cystic fibrosis provide significant psychosocial and physical benefits in addition to standard medical care. Studies were identified from two Cochrane trials registers (Cystic Fibrosis and Genetic Disorders Group; Depression, Anxiety and Neurosis Group), Ovid MEDLINE and PsychINFO; unpublished trials were located through professional networks and Listserves. Most recent search of the Cystic Fibrosis and Genetic Disorders Group's register: 19 December 2013.Most recent search of the Depression, Anxiety and Neurosis Group's register: 12 November 2013. Randomised controlled studies of a broad range of psychological interventions evaluating subjective and objective health outcomes, such as quality of life or pulmonary function, in individuals of all ages with cystic fibrosis and their immediate family. We were interested in psychological interventions, including psychological methods within the scope of psychotherapeutic or psychosomatic mechanism of action (e.g. cognitive behavioural, cognitive, family systems or systemic, psycho-dynamic, or other, e.g. supportive, relaxation, or biofeedback), which were aimed at improving psychological and psychosocial outcomes (e.g. quality of life, levels of stress or distress, psychopathology, etc.), adaptation to disease management and physiological outcomes. Three authors were involved in selecting the eligible studies and two of these authors assessed their risk of bias. The review includes 16 studies (eight new studies included in this update) representing data from 556 participants. Studies are diverse in their design and their methods. They

Lung Cancers Associated with Cystic Airspaces: Underrecognized Features of Early Disease.

Science.gov (United States)

Sheard, Sarah; Moser, Joanna; Sayer, Charlie; Stefanidis, Konstantinos; Devaraj, Anand; Vlahos, Ioannis

2018-01-01

Early lung cancers associated with cystic airspaces are increasingly being recognized as a cause of delayed diagnoses-owing to data gathered from screening trials and encounters in routine clinical practice as more patients undergo serial imaging. Several morphologic subtypes of cancers associated with cystic airspaces exist and can exhibit variable patterns of progression as the solid elements of the tumor grow. Current understanding of the pathogenesis of these malignancies is limited, and the numbers of cases reported in the literature are small. However, several tumor cell types are represented in these lesions, with adenocarcinoma predominating. The features of cystic airspaces differ among cases and include emphysematous bullae, congenital or fibrotic cysts, subpleural blebs, bronchiectatic airways, and distended distal airspaces. Once identified, these cystic lesions pose management challenges to radiologists in terms of distinguishing them from benign mimics of cancer that are commonly seen in patients who also are at increased risk of lung cancer. Rendering a definitive tissue-based diagnosis can be difficult when the lesions are small, and affected patients tend to be in groups that are at higher risk of requiring biopsy or resection. In addition, the decision to monitor these cases can add to patient anxiety and cause the additional burden of strained departmental resources. The authors have drawn from their experience, emerging evidence from international lung cancer screening trials, and large databases of lung cancer cases from other groups to analyze the prevalence and evolution of lung cancers associated with cystic airspaces and provide guidance for managing these lesions. Although there are insufficient data to support specific management guidelines similar to those for managing small solid and ground-glass lung nodules, these data and guidelines should be the direction for ongoing research on early detection of lung cancer. © RSNA, 2018.

Cystic echinococcosis: Future perspectives of molecular epidemiology

Science.gov (United States)

Human cystic echinococcosis (CE) has been conceived to be caused predominantly by Echinococcus granulosus sensu stricto (the dog-sheep strain). Recent molecular approaches on CE, however, have revealed that human cases are also commonly caused by another species, Echinococcus canadensis. All indices...

The distinctly visible right upper lobe bronchus on the lateral chest: A clue to adolescent cystic fibrosis

International Nuclear Information System (INIS)

Reinig, J.W.; Sanchez, F.W.; Thomason, D.M.; Gobien, R.P.

1985-01-01

Radiographic differentiation between cystic fibrosis and asthma presenting in teenagers and young adults can be difficult. Many patients with a late presentation of cystic fibrosis display minimal changes on a chest radiograph. However, a large majority (90%) of cystic fibrosis patients with an essentially normal PA chest radiograph will have a distinctly outlined orifice of right upper lobe bronchus on a lateral chest film as opposed to a small number of asthmatics (25%) or normal patients (18%). This observation correlates well with the pathologic finding that the initial pulmonary involvement in cystic fibrosis is typically in the right upper lobe in adolescents. Teenager or young adult patients presenting with a history of repeated respiratory infections, asthma-like symptoms and a distinctly visible right upper lobe bronchus on a lateral chest film should be sweat-tested to exclude cystic fibrosis. (orig.)

Anesthetic concerns in a huge congenital sublingual swelling obscuring airway access

Directory of Open Access Journals (Sweden)

Nilesh Kumar

2015-01-01

Full Text Available Presence of intraoral pathology poses a great challenge during management of pediatric airway. We report management of big intraoral cystic swelling physically occupying the entire oral cavity restricting access to airway. Preintubation aspiration of swelling was done to decrease its size and make room for airway manipulation, followed by laryngoscopy and intubation in lateral position. Airway patency is at risk in postoperative period also, in this case, though the swelling decreased in size postoperatively but presence of significant edema required placement of tongue stitch and modified nasopharyngeal airway. Case report highlights simple maneuvers to manage a difficult case.

Mature cystic Theratome. Presentation of a case

International Nuclear Information System (INIS)

Rivera B, Aura Lucia; Carrillo B, Jorge Alberto; Ojeda L, Paulina

2004-01-01

The case of a patient of five months of age is presented, to which was diagnosed cystic theratome, initially was assisted in another institution to present consistent square of four days in dry cough and sialorrea. It was managed initially with pneumonia diagnosis and spill paraneumonic. The x-ray of initial thorax demonstrated an opacity committing the two inferior thirds of the left hemithorax, with obliteration of the costofrenic angle and contralateral deviation of the cardio mediastinum, later on another thorax x-ray to the entrance with diagnostic impression of sepsis of lung origin and pneumonia suspicion with spill associate pleural, was practiced closed thoracotomy, obtaining 60 cc of sallow liquid. For the persistence of the opacity basal left in the control x-ray, he was practiced thorax tomography. For the presence of multiple densities and the localization of the lesion it outlines the possibility of cystic theratome

Sodium and chlorine concentrations in mixed saliva of healthy and cystic fibrosis children

Energy Technology Data Exchange (ETDEWEB)

Jimenez-Reyes, M. [Instituto Nacional de Investigaciones Nucleares, Mexico City (Mexico); Sanchez-Aguirre, F.J. [Instituto Mexicano del Seguro Social (Mexico). Dept. de Genetica

1996-03-01

Sodium and chlorine concentrations in mixed saliva were simultaneously measured by neutron activation analysis in nine normal children and in nine patients with cystic fibrosis. Sodium levels showed a significant difference (P < 0.01) between patients and controls. The concentration of chlorine was similar in both the control and the cystic fibrosis groups. (author).

Sodium and chlorine concentrations in mixed saliva of healthy and cystic fibrosis children

International Nuclear Information System (INIS)

Jimenez-Reyes, M.; Sanchez-Aguirre, F.J.

1996-01-01

Sodium and chlorine concentrations in mixed saliva were simultaneously measured by neutron activation analysis in nine normal children and in nine patients with cystic fibrosis. Sodium levels showed a significant difference (P < 0.01) between patients and controls. The concentration of chlorine was similar in both the control and the cystic fibrosis groups. (author)

THE CYSTIC FORM OF RHEUMATOID-ARTHRITIS

NARCIS (Netherlands)

Dijkstra, P. F.; Gubler, F. M.; Maas, M.

1988-01-01

A non-erosive form of rheumatoid arthritis (R.A.) was found in 62 patients out of 660 patients with R.A. These 62 patients exhibit slowly progressive cystic changes in about the same joints in which usually erosions develop in classic R.A. The E.S.R. is often low, half of the patients remained

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....

Trypsin level in gallbladder bile and ductitis and width of the cystic duct.

Science.gov (United States)

Vracko, J; Wiechel, K L

2000-01-01

The change from laparotomy to laparoscopy for cholecystectomy has raised the question of how to manage concomitant bile duct stones. The present-day interest--and controversy--has focused on a transcystic approach reported to be feasible in 66-96% of cases, but without explaining the necessary prerequisite: the widening of the cystic duct. The cystic duct, wide mainly in patients with bile duct stones, has been reported to be highly variable: from strictured to very wide. The present study aims at comparing the trypsin level in the gallbladder bile and the cystic duct morphology and width in patients with and without bile duct stones. A prospective series of 63 gallstone patients, 30 with and 33 without bile duct stones (controls), underwent cholecystectomy and bile duct clearance. The study includes the trypsin level in the gallbladder bile, the width and morphology of the cystic duct, and the size of the gallstones. The patients with bile duct stones had, in contrast to the controls, higher trypsin levels in the gallbladder bile (P extraction feasible.

CFTR-dependent defect in alternatively-activated macrophages in cystic fibrosis.

Science.gov (United States)

Tarique, Abdullah A; Sly, Peter D; Holt, Patrick G; Bosco, Anthony; Ware, Robert S; Logan, Jayden; Bell, Scott C; Wainwright, Claire E; Fantino, Emmanuelle

2017-07-01

The role of the macrophages in cystic fibrosis (CF) lung disease has been poorly studied. We hypothesized that alternatively activated M2 macrophages are abnormal in CF lung disease. Blood samples were collected from adults (n=13) children (n=27) with CF on admission for acute pulmonary exacerbation and when clinically stable. Monocytes were differentiated into macrophages and polarized into classical (M1) and alternatively-activated (M2) phenotypes, function determined ex-vivo and compared with healthy controls. In the absence of functional cystic fibrosis trans-membrane conductance regulator (CFTR), either naturally in patients with CF or induced with CFTR inhibitors, monocyte-derived macrophages do not respond to IL-13/IL-4, fail to polarize into M2s associated with a post-transcriptional failure to produce and express IL-13Rα1 on the macrophage surface Polarization to the M1 phenotype was unaffected. CFTR-dependent imbalance of macrophage phenotypes and functions could contribute to the exaggerated inflammatory response seen in CF lung disease. Copyright © 2017 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.

Cystic Fibrosis-Related Diabetes in Adults: Where Can We Go From Here?

OpenAIRE

de Valk, Harold W.; van der Graaf, Eduard A.

2007-01-01

Cystic fibrosis (CF), a dysfunction of the exocrine glands, is one of the most frequently diagnosed genetic diseases. It is characterized by chronic pulmonary disease and pancreatic deficiency. Cystic fibrosis-related diabetes (CFRD) is a complication of CF and develops from impaired glucose tolerance via postprandial hyperglycemia with fasting normoglycemia to full-blown diabetes with fasting and postprandial hyperglycemia. CFRD is related to decreased life expectancy, most notably in female...

Inadvertent chest tube insertion in congenital cystic adenomatoid malformation and congenital lobar emphysema-highlighting an important problem

International Nuclear Information System (INIS)

Prabhu, Shailesh M; Choudhury, Subhasis Roy; Solanki, Ravi S; Shetty, Gurucharan S; Agarwala, Surenderkumar

2013-01-01

Chest tube insertion in congenital cystic lung lesions is an important problem in children with acute respiratory distress having a cystic lucent lesion on chest radiograph. To evaluate the imaging findings and complications in cases of congenital cystic lung lesions with chest tube insertion and suggest the role of appropriate imaging for management of these patients. Chest radiographs and CT scans of children with congenital cystic lung lesions who had inadvertent chest tube insertion preoperatively were retrospectively reviewed for imaging appearances and complications. Fifteen patients comprising 10 cases of congenital cystic adenomatoid malformation (CCAM) and 5 cases of congenital lobar emphysema (CLE) were included. Majority of the cases were infants. CCAM was misdiagnosed as complicated pneumatocele (n = 5) and pneumothorax (n = 5), while CLE was misdiagnosed as tension pneumothorax (n = 5) on the chest radiograph findings. Final diagnosis was made on CT and operative findings with histopathology. Complications noted were pneumothorax, hydropneumothorax, and infection in cases of CCAM, and change in imaging appearance and pneumothorax in cases of CLE. Chest tube insertion in congenital cystic lesions increases the rate of associated complications. Chest CT has a definite role in early diagnosis and deciding appropriate management in these cases

"Bong lung" in cystic fibrosis: a case report

Directory of Open Access Journals (Sweden)

Hauser Jenny

2010-11-01

Full Text Available Abstract Introduction Marijuana or "bong" lung has been recently described. Subjects typically develop large peripheral paraseptal lung bullae and are predisposed to spontaneous pneumothoraces. The underlying mechanism for bullae formation is uncertain, but probably relates to direct lung toxicity and repeated barotrauma as the smoker performs frequent valsalva manoeuvres in an attempt to derive a greater drug effect. Case presentation We describe a case of probable "bong lung" occurring in a 23-year-old Caucasian man with cystic fibrosis who had a history of recurrent pneumothoraces and unusual findings on sputum cytology. Conclusion Our case highlights the importance of questioning young adult cystic fibrosis patients about illicit drug use and the utility of sputum cytology and computed tomography scanning when patients present with pneumothoraces and deteriorations in clinical status.

Antioxidant supplementation for lung disease in cystic fibrosis

DEFF Research Database (Denmark)

Ciofu, Oana; Lykkesfeldt, Jens

2014-01-01

BACKGROUND: Airway infection leads to progressive damage of the lungs in cystic fibrosis and oxidative stress has been implicated in the etiology. Supplementation of antioxidant micronutrients (vitamin E, vitamin C, ß-carotene and selenium) or glutathione may therefore potentially help maintain...... COLLECTION AND ANALYSIS: Two authors independently selected studies, extracted data and assessed the risk of bias in the included studies. We contacted trial investigators to obtain missing information. Primary outcomes are lung function and quality of life; secondary outcomes are oxidative stress...... or by inhalation) appears to improve lung function in some cases and decrease oxidative stress; however, due to the very intensive antibiotic treatment and other treatments that cystic fibrosis patients receive, the beneficial effect of antioxidants is very difficult to assess in patients with chronic infection...

Internal irradiation for cystic craniopharyngioma

International Nuclear Information System (INIS)

Kobayashi, T.; Kageyama, N.; Ohara, K.

1981-01-01

The authors report the results of internal irradiation with labeled chromic phosphate (32P) and gold-198 (198Au) colloid in eight cases of cystic craniopharyngiomas. They used a newly developed dosimetric formula, by which the radiation dose at the cyst wall and at any point far from the radioactive source can be calculated. Ten courses of irradiation in eight patients were carried out by injection of either 32P or 198Au colloid into the cyst through an Ommaya drainage system that had been placed at craniotomy. Follow-up studies ranging from 13 to 156 months revealed that all cysts were effectively treated, with elimination of fluid or collapse of the cyst. This was confirmed by Conray cystography and/or computerized tomography. Not only the dose delivered to the wall but also the thickness of the cyst wall and the location of the cyst are important factors in planning internal irradiation. A safe and adequate dose to the cyst wall could range between 9000 to 30,000 rads for craniopharyngioma. This treatment is suitable for large cysts that are thought to be difficult to remove radically, recurrent cysts resistant to previous treatment, or multiple cysts. Internal irradiation may also be applicable in other cystic intracranial tumors if dosimetry is calculated accurately

Longevity of Patients With Cystic Fibrosis in 2000 to 2010 and Beyond: Survival Analysis of the Cystic Fibrosis Foundation Patient Registry

Science.gov (United States)

MacKenzie, Todd; Gifford, Alex H.; Sabadosa, Kathryn A.; Quinton, Hebe B.; Knapp, Emily A.; Goss, Christopher H.; Marshall, Bruce C.

2015-01-01

Background Advances in treatments for cystic fibrosis (CF) continue to extend survival. An updated estimate of survival is needed for better prognostication and to anticipate evolving adult care needs. Objective To characterize trends in CF survival between 2000 and 2010 and to project survival for children born and diagnosed with the disease in 2010. Design Registry-based study. Setting 110 Cystic Fibrosis Foundation–accredited care centers in the United States. Patients All patients represented in the Cystic Fibrosis Foundation Patient Registry (CFFPR) between 2000 and 2010. Measurements Survival was modeled with respect to age, age at diagnosis, gender, race or ethnicity, F508del mutation status, and symptoms at diagnosis. Results Between 2000 and 2010, the number of patients in the CFFPR increased from 21 000 to 26 000, median age increased from 14.3 to 16.7 years, and adjusted mortality decreased by 1.8% per year (95% CI, 0.5% to 2.7%). Males had a 19% (CI, 13% to 24%) lower adjusted risk for death than females. Median survival of children born and diagnosed with CF in 2010 is projected to be 37 years (CI, 35 to 39 years) for females and 40 years (CI, 39 to 42 years) for males if mortality remains at 2010 levels and more than 50 years if mortality continues to decrease at the rate observed between 2000 and 2010. Limitations The CFFPR does not include all patients with CF in the United States, and loss to follow-up and missing data were observed. Additional analyses to address these limitations suggest that the survival projections are conservative. Conclusion Children born and diagnosed with CF in the United States in 2010 are expected to live longer than those born earlier. This has important implications for prognostic discussions and suggests that the health care system should anticipate greater numbers of adults with CF. Primary Funding Source Cystic Fibrosis Foundation. PMID:25133359

Autogenic drainage for airway clearance in cystic fibrosis.

Science.gov (United States)

McCormack, Pamela; Burnham, Paul; Southern, Kevin W

2017-10-06

Autogenic drainage is an airway clearance technique that was developed by Jean Chevaillier in 1967. The technique is characterised by breathing control using expiratory airflow to mobilise secretions from smaller to larger airways. Secretions are cleared independently by adjusting the depth and speed of respiration in a sequence of controlled breathing techniques during exhalation. The technique requires training, concentration and effort from the individual. It is important to systematically review the evidence demonstrating that autogenic drainage is an effective intervention for people with cystic fibrosis. To compare the clinical effectiveness of autogenic drainage in people with cystic fibrosis with other physiotherapy airway clearance techniques. We searched the Cochrane Cystic Fibrosis Trials Register, compiled from electronic database searches and handsearching of journals and conference abstract books. We also searched the reference lists of relevant articles and reviews, as well as two trials registers (31 August 2017).Dtae of most recent search of the Cochrane Cystic Fibrosis Trials Register: 25 September 2017. We identified randomised and quasi-randomised controlled studies comparing autogenic drainage to another airway clearance technique or no therapy in people with cystic fibrosis for at least two treatment sessions. Data extraction and assessments of risk of bias were independently performed by two authors. The authors assessed the quality of the evidence using the GRADE system. The authors contacted two investigators for further information pertinent to their published studies. Searches retrieved 35 references to 21 individual studies, of which seven (n = 208) were eligible for inclusion. One study was of parallel design with the remaining six being cross-over in design; participant numbers ranged from 17 to 75. The total study duration varied between four days and two years. The age of participants ranged between seven and 63 years with a wide

Computed tomography of the thorax in children with cystic fibrosis

International Nuclear Information System (INIS)

Parente Filho, Livio William Sales; Marchiori, Edson; Daltro, Pedro; Santos, Eloa Nunes

1998-01-01

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)

The Role of Vitamin A in Patients with Cystic Fibrosis

Directory of Open Access Journals (Sweden)

N.V. Rogovyk

2013-10-01

Full Text Available Cystic fibrosis is hereditary monogenic exocrine disease, which manifests itself most often by disorders in bronchopulmonary and digestive systems. The affection of the latter leads to deficiency of liposoluble vitamins A, D, E and K in the body of the patient. The lack and excess of vitamin A in this disease may contribute to a number of pathological states and aggravate the disease. Therefore, in the records of all European centers of cystic fibrosis it is recommended to carry out annual determination of the levels of vitamin A, followed by individual dose adjustment.

Cystic Fibrosis Related Liver Disease—Another Black Box in Hepatology

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Katharina Staufer

2014-08-01

Full Text Available Due to improved medical care, life expectancy in patients with cystic fibrosis (CF has veritably improved over the last decades. Importantly, cystic fibrosis related liver disease (CFLD has become one of the leading causes of morbidity and mortality in CF patients. However, CFLD might be largely underdiagnosed and diagnostic criteria need to be refined. The underlying pathomechanisms are largely unknown, and treatment strategies with proven efficacy are lacking. This review focuses on current invasive and non-invasive diagnostic standards, the current knowledge on the pathophysiology of CFLD, treatment strategies, and possible future developments.

Aerosol scintigraphy in the assessment of therapy for cystic fibrosis

International Nuclear Information System (INIS)

Kuni, C.C.; Regelmann, W.E.; Budd, J.R.; Cret, R.P. du; Boudreau, R.J.

1990-01-01

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

Cyanide levels found in infected cystic fibrosis sputum inhibit airway ciliary function.

Science.gov (United States)

Nair, Chandrika; Shoemark, Amelia; Chan, Mario; Ollosson, Sarah; Dixon, Mellissa; Hogg, Claire; Alton, Eric W F W; Davies, Jane C; Williams, Huw D

2014-11-01

We have previously reported cyanide at concentrations of up to 150 μM in the sputum of cystic fibrosis patients infected with Pseudomonas aeruginosa and a negative correlation with lung function. Our aim was to investigate possible mechanisms for this association, focusing on the effect of pathophysiologically relevant cyanide levels on human respiratory cell function. Ciliary beat frequency measurements were performed on nasal brushings and nasal air-liquid interface (ALI) cultures obtained from healthy volunteers and cystic fibrosis patients. Potassium cyanide decreased ciliary beat frequency in healthy nasal brushings (n = 6) after 60 min (150 μM: 47% fall, pcyanide as a key component inhibiting the ciliary beat frequency. If cyanide production similarly impairs mucocilliary clearance in vivo, it could explain the link with increased disease severity observed in cystic fibrosis patients with detectable cyanide in their airway. ©ERS 2014.

Cystic Fibrosis Gene Therapy in the UK and Elsewhere

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Pytel, Kamila M.; Alton, Eric W.F.W.

2015-01-01

Abstract The cystic fibrosis transmembrane conductance regulator (CFTR) gene was identified in 1989. This opened the door for the development of cystic fibrosis (CF) gene therapy, which has been actively pursued for the last 20 years. Although 26 clinical trials involving approximately 450 patients have been carried out, the vast majority of these trials were short and included small numbers of patients; they were not designed to assess clinical benefit, but to establish safety and proof-of-concept for gene transfer using molecular end points such as the detection of recombinant mRNA or correction of the ion transport defect. The only currently published trial designed and powered to assess clinical efficacy (defined as improvement in lung function) administered AAV2-CFTR to the lungs of patients with CF. The U.K. Cystic Fibrosis Gene Therapy Consortium completed, in the autumn of 2014, the first nonviral gene therapy trial designed to answer whether repeated nonviral gene transfer (12 doses over 12 months) can lead to clinical benefit. The demonstration that the molecular defect in CFTR can be corrected with small-molecule drugs, and the success of gene therapy in other monogenic diseases, is boosting interest in CF gene therapy. Developments are discussed here. PMID:25838137

Prenatal diagnosis of congenital cystic adenomatoid malformation of the lung: A case report

International Nuclear Information System (INIS)

Shin, Hyun Ja; Shin, M. J.; Yoo, Y. J.; Park, J. M.; Kim, J. R.

1990-01-01

Congenital cystic adenomatoid malformation is one of a rare congenital malformation usually unilateal in volving a part of lobe or a whole lobe of the fetal lung, characterized by excessive growing of terminal respiratory element. We made a prenatal diagnosis in a case of congenital cystic adenomdtoid malformation with diffuse bilateral involvement, Stocker Type III which is associated with fetal hydrops

Choroidal metastasis of a minor salivary gland adenoid cystic carcinoma: A case report.

Science.gov (United States)

Portilla Blanco, R R; Roberts Martínez-Aguirre, I; Pontón Méndez, P; Zarzosa Martín, M E; Pérez-Salvador García, E

2018-03-21

A 61-year-old man with a lower lip minor salivary gland adenoid cystic carcinoma, suffered from a unilateral progressive visual acuity loss due to choroidal metastasis. Adenoid cystic carcinoma is a rare primary tumour with significant metastatic potential. Our patient presented with a unilateral choroidal metastasis. According to the current literature, 8 cases of choroidal metastasis of salivary gland adenoid cystic carcinoma have been reported. This is the second case reported of choroidal metastasis with origin in a minor salivary gland, and the first one with origin in the minor salivary glands of the lower lip. Copyright © 2018 Sociedad Española de Oftalmología. Publicado por Elsevier España, S.L.U. All rights reserved.

Huge gastric diospyrobezoars successfully treated by oral intake and endoscopic injection of Coca-Cola.

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Chung, Y W; Han, D S; Park, Y K; Son, B K; Paik, C H; Jeon, Y C; Sohn, J H

2006-07-01

A diospyrobezoar is a type of phytobezoar that is considered to be harder than any other types of phytobezoars. Here, we describe a new treatment modality, which effectively and easily disrupted huge gastric diospyrobezoars. A 41-year-old man with a history of diabetes mellitus was admitted with lower abdominal pain and vomiting. Upper gastrointestinal endoscopy revealed three huge, round diospyrobezoars in the stomach. He was made to drink two cans of Coca-Cola every 6 h. At endoscopy the next day, the bezoars were partially dissolved and turned to be softened. We performed direct endoscopic injection of Coca-Cola into each bezoar. At repeated endoscopy the next day, the bezoars were completely dissolved.

South African adolescents with cystic fibrosis: a qualitative ...

African Journals Online (AJOL)

South African adolescents with cystic fibrosis: a qualitative exploration of their ... years) who had the defining characteristics of CF and were living in Gauteng province. ... The fundamental human need to be understood and to understand was ...

Genetic modifiers of nutritional status in cystic fibrosis1234

OpenAIRE

Bradley, Gia M; Blackman, Scott M; Watson, Christopher P; Doshi, Vishal K; Cutting, Garry R

2012-01-01

Background: Improved nutrition early in life is associated with better pulmonary function for patients with cystic fibrosis (CF). However, nutritional status is poorly correlated with the CFTR genotype.

The diagnostic analysis using the CT scan, of cystic lesions in the cranial basis in pediatric cases

International Nuclear Information System (INIS)

Oshida, Kinya; Ishimori, Shoji; Okada, Ryoho.

1978-01-01

It is the purpose of this paper to evaluate the low-density lesions of those pediatric cases which were treated at this hospital in the six months beginning July, 1977, using a CT scan, EMI-1010 Type. Among 1500 consecutive cases diagnosed with the CT scan, 300 pediatric cases were included. We discuss particularly eight patients, two with arachnoidal cysts, two with cystic craniopharyngiomas, two with cystic cerebellar astrocytomas and two with Dandy-Walker syndromes, one of which was accompanied by corpus callosum agenesis. The distribution of ages was from 2 to 12 years. We calculated the matrix of each slice of these patients, CT scan in the cystic lesions, the area including the cystic lesion, ventricles and brain tissue, predominantly the white matter or interhemispheric cortex including white matter, without dye enhancement. The x-ray absorption values of the low-density lesions were definitely different according to the nature of the cystic contents. The mean values of x-ray absorption, the standard deviations, and the sample sizes of each area in the matrix were compared statistically with those of cystic lesions. The mean values of the arachnoidal cysts, the contents of which were almost like CSF, were 17 - 21; that of cystic craniopharyngiomas, the contents of which were a thick yellowic fluid with cholesterin crystals, were 23 - 28; those of cerebellar astrocytomas, with Froin positive fluid, were 22 - 24; the cystic lesions of the posterior fossa in the Dandy-Walker syndrome were 8 - 13. The difference in the x-ray absorption values (Hounsfield number) were compared statistically for analyzing the differential diagnosis of these low-density lesions by calculating the P-values. All of these lesions were verified by surgical explorations. (author)

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...... patients were detected in the Danish CF cohort. Patients diagnosed with untreated CD reported symptoms typical of both CF and CD (poor weight gain, loose and/or fatty stools, fatigue, irritability, abdominal pain). They improved after introduction of a gluten-free diet. CONCLUSIONS: Systematic screening...

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 acina...... (CF) and pancreatitis, and outline present and potential therapeutic approaches in CF treatment relevant to the pancreas....

Differential diagnostic value of diffusion weighted imaging on brain abscess and necrotic or cystic brain tumors

International Nuclear Information System (INIS)

Zhang Xiaoya; Yin Jie; Wang Kunpeng; Zhang Jiandang; Liang Biling

2009-01-01

Objective: To investigate the value of diffusion weighted imaging (DWI)on brain abscess and necrotic or cystic brain tumors. Methods: 27 cases with brain abscesses and 33 cases with necrotic or cystic brain tumors (gliomas or metastases) were performed conventional MRI and DWI. Apparent diffusion coefficient (ADC) of region of interest (ROI) was measured and statistically tested. Sensitivity and specificity were calculated and compared with conventional MR and DWI. Results: Hyperintensity signal was seen on most brain abscesses. All necrotic or cystic brain tumors showed hypointensity signal on DWI. There was statistical significance on ADC of them. The sensitivity and specificity of conventional MRI was lower than that of DWI. Conclusion: DWI and ADC were useful in distinguishing brain abscessed from necrotic or cystic brain tumors, which was important in addition to conventional MRI. (authors)

Cystic craniopharyngioma: intratumoral chemotherapy with alpha interferon

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Patrícia Alessandra Dastoli

2011-02-01

Full Text Available OBJECTIVE: To assess whether the cystic craniopharyngiomas can be controlled with the use of intratumoral applications of interferon alpha. METHOD: Nineteen patients with the diagnosis of cystic craniopharyngioma were treated with intratumoral chemotherapy with interferon alpha from January 2002 to April 2006. All patients underwent placement of an intracystic catheter connected to an Ommaya reservoir. Through this reservoir were made applications during chemotherapy cycles. Each cycle corresponded to application of 3,000,000 units of interferon alpha three times per week on alternate days totalizing 36,000,000 units. Response to treatment was evaluated by calculating the tumor volume on MRI control after one, three and six months after the end of each cycle. Patients who developed worsening of symptoms or who had insignificant reduction in tumor volume during follow-up underwent repeat cycle chemotherapy. RESULTS: Four patients received four cycles of chemotherapy, three patients received three cycles, six patients received two cycles and six patients received one. The lower percentage of reduction in tumor volume was 60% and the bigger reduction was 98.37%. Eleven patients had a reduction greater than 90%. Five patients had a tumor reduction between 75 and 90% and in three patients the tumors were reduced by less than 75%. No deaths occurred during treatment and side effects of interferon alpha were well tolerated. No treatment was discontinued. Follow-up after the last application ranged from one year and five months to three years and nine months. CONCLUSION: The intratumoral chemotherapy with interferon alpha decreases the volume of cystic craniopharyngiomas and so far can be considered a new therapeutic alternative.

Association of High-Dose Ibuprofen Use, Lung Function Decline, and Long-Term Survival in Children with Cystic Fibrosis.

Science.gov (United States)

Konstan, Michael W; VanDevanter, Donald R; Sawicki, Gregory S; Pasta, David J; Foreman, Aimee J; Neiman, Evgueni A; Morgan, Wayne J

2018-04-01

Cystic fibrosis deaths result primarily from lung function loss, so chronic respiratory therapies, intended to preserve lung function, are cornerstones of cystic fibrosis care. Although treatment-associated reduction in rate of lung function loss should ultimately improve cystic fibrosis survival, no such relationship has been described for any chronic cystic fibrosis therapy. In part, this is because the ages of most rapid lung function decline-early adolescence-precede the median age of cystic fibrosis deaths by more than a decade. To study associations of high-dose ibuprofen treatment with the rate of forced expiratory volume in 1 second decline and mortality among children followed in the Epidemiologic Study of Cystic Fibrosis and subsequently in the U.S. Cystic Fibrosis Foundation Patient Registry. We performed a matched cohort study using data from Epidemiologic Study of Cystic Fibrosis. Exposure was defined as high-dose ibuprofen use reported at ≥80% of encounters over 2 years. Unexposed children were matched to exposed children 5:1 using propensity scores on the basis of demographic, clinical, and treatment covariates. The rate of decline of percent predicted forced expiratory volume in 1 second during the 2-year follow-up period was estimated by mixed-effects modeling with random slopes and intercepts. Survival over 16 follow-up years in the U.S. Cystic Fibrosis Foundation Patient Registry was compared between treatment groups by using proportional hazards modeling controlling for matching and covariates. We included 775 high-dose ibuprofen users and 3,665 nonusers who were well matched on demographic, clinical, and treatment variables. High-dose ibuprofen users declined on average 1.10 percent predicted forced expiratory volume in 1 second/yr (95% confidence interval; 0.51, 1.69) during the 2-year treatment period, whereas nonusers declined at a rate of 1.76% percent predicted forced expiratory volume in 1 second/yr (95% confidence interval; 1.48, 2

The experience of men and women with cystic fibrosis who have become a parent: A qualitative study.

Science.gov (United States)

Jessup, Melanie; Li, Anne; Fulbrook, Paul; Bell, Scott C

2018-04-01

To explore the experiences of men and women with cystic fibrosis in becoming parents. As lifespan for people with cystic fibrosis increases, and reproductive technology advances, having a child of their own becomes a possibility. This study used a phenomenological framework. Seven Australian adults with cystic fibrosis were invited to describe their experiences of becoming parents in the context of a semi-structured interview. Analysis of the data involved highlighting recurrent phrases and isolating emergent themes. Two overarching themes characterised the participants' experience: Counting the cost, as they recalled Concentric communication and Pathways to pregnancy; and Living the dream, as they cast a retrospective view over this, their major achievement, in the light of their Reaction: a dream comes true, Coping: a question of balance, Conjecture: the future redefined and Confidence: recalibrating. While advances in cystic fibrosis care and reproductive technology have increased the possibility of individuals with cystic fibrosis becoming parents, the passage to becoming a parent is a complex process. These findings can inform health professionals to support the adaptive work necessary for families that include members with cystic fibrosis. A contemporary understanding of this phenomenon is necessary for facilitating clinically relevant communication. © 2017 John Wiley & Sons Ltd.

New advances in the therapy of non-cystic fibrosis bronchiectasis

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A. Amorim

2013-11-01

Full Text Available Non-cystic fibrosis bronchiectasis remains a common and important respiratory disease to date. It is a chronic pathology and consequently the patients usually require continuous treatment.In recent decades therapies that do not have scientific evidence of their benefits have been commonly used in non-cystic fibrosis bronchiectasis. Cystic fibrosis has provided the experience to extrapolate therapeutic approaches to other bronchiectasis patients. Finally, in the last few years some trials have been carried out specifically in non-cystic fibrosis bronchiectasis which aim to assess the efficacy of some of the treatments which are commonly used but sometimes without clear indication.This review will discuss the recent results from these trials, namely mucoactive, anti-inflammatory and antibiotic therapy. Several trials are ongoing and we hope they will be able to add clarification to the management of these patients. Resumo: As bronquiectasias não-fibrose quística continuam a ser uma doença respiratória comum e importante. Trata-se de uma patologia crónica e, consequentemente, os doentes geralmente precisam de um tratamento contínuo.Nas últimas décadas, tratamentos sem evidência científica dos seus benefícios foram comumente usadas nas bronquiectasias não-fibrose quística. A fibrose quística serviu de experiência para extrapolar a abordagem terapêutica para outros doentes com bronquiectasias. Finalmente, nos últimos anos, foram realizados alguns ensaios bronquiectasias não-fibrose quística que visam avaliar a eficácia de alguns dos tratamentos que são comummente usados mas por vezes sem uma clara indicação.Nesta revisão serão apresentados os resultados recentes destes ensaios, nomeadamente sobre o tratamento mucoactivo, anti-inflamatório e antibiótico. Diversos estudos estão a decorrer e esperamos que estes venham a esclarecer a abordagem mais adequada destes doentes. Keywords: Non-cystic fibrosis bronchiectasis, Advances

Customer satisfaction survey to improve the European cystic fibrosis external quality assessment scheme.

Science.gov (United States)

Berwouts, Sarah; Dequeker, Elisabeth

2011-08-01

The Cystic Fibrosis European Network, coordinated from within the Katholieke Universiteit Leuven, is the provider of the European cystic fibrosis external quality assessment (EQA) scheme. The network aimed to seek feedback from laboratories that participated in the cystic fibrosis scheme in order to improve services offered. In this study we analysed responses to an on-line customer satisfaction survey conducted between September and November 2009. The survey was sent to 213 laboratories that participated in the cystic fibrosis EQA scheme of 2008; 69 laboratories (32%) responded. Scores for importance and satisfaction were obtained from a five-point Likert scale for 24 attributes. A score of one corresponded to very dissatisfied/very unimportant and five corresponded to very satisfied/very important. Means were calculated and placed in a two-dimensional grid (importance-satisfaction analysis). Means were subtracted from each other to obtain gap values (gap-analysis). No attribute had a mean score below 3.63. The overall mean of satisfaction was 4.35. Opportunities for improvement enclosed clarity, usefulness and completeness of the general report and individual comments, and user-friendliness of the electronic datasheet. This type of customer satisfaction survey was a valuable instrument to identify opportunities to improve the cystic fibrosis EQA scheme. It should be conducted on a regular basis to reveal new opportunities in the future and to assess effectiveness of actions taken. Moreover, it could be a model for other EQA providers seeking feedback from participants. Overall, the customer satisfaction survey provided a powerful quality of care improvement tool.

Barriers to adherence in adolescents and young adults with cystic fibrosis

DEFF Research Database (Denmark)

Bregnballe, Vibeke; Schiøtz, Peter Oluf; Boisen, Kirsten A

2011-01-01

Treatment adherence is crucial in patients with cystic fibrosis, but poor adherence is a problem, especially during adolescence. Identification of barriers to treatment adherence and a better understanding of how context shapes barriers is of great importance in the disease. Adolescent reports of...... of barriers to adherence have been studied, but studies of their parents' experience of such barriers have not yet been carried out. The aim of the present study was to explore barriers to treatment adherence identified by young patients with cystic fibrosis and by their parents....

Adventitial cystic disease of the common femoral vein presenting as deep vein thrombosis

Directory of Open Access Journals (Sweden)

Young-Kyun Kim

2016-07-01

Full Text Available Adventitial cystic disease of the common femoral vein is a rare condition. We herein report the case of a 50-year-old woman who presented with painless swelling in her left lower leg that resembled deep vein thrombosis. She underwent femoral exploration and excision of the cystic wall. The presentation, investigation, treatment, and pathology of this condition are discussed with a literature review.

Bilateral adrenal cystic neuroblastoma with superior vena cava syndrome and massive intracystic haemorrhage

International Nuclear Information System (INIS)

Pinarli, Faruk Guclu; Danaci, Murat; Diren, Baris; Tander, Burak; Rizalar, Riza; Dagdemir, Ayhan; Acar, Sabri

2004-01-01

Bilateral cystic adrenal tumours are a rare presentation of neuroblastoma. Intratumoural haemorrhage is a frequent finding in neuroblastoma, but is rarely symptomatic. We present an 11-month-old girl with predominantly cystic bilateral neuroblastomas and distant lymph-node metastasis. Massive intracystic haemorrhage and superior vena cava (SVC) syndrome were ominous prognostic factors, leading to death. Large tumours with intracystic haemorrhage might require a conservative approach. (orig.)

Radiologic findings of cystic fibrosis in a Korean child at follow up study: case report

International Nuclear Information System (INIS)

Jang, Il Soo; Park, Choong Ki; Jeon, Seok Chol; Choi, Yo Won; Park, Dong Woo; Kim, Yong Soo; Kim, Young Sun; Ahn, You Hern

2003-01-01

Cystic fibrosis is a hereditary disease involving recessive transmission. The fundamental abnormality consists of the production of abnormal secretion from a variety of exocrine glands such as salivary and sweat glands and those of the pancreas, colon, and tracheobronchial trees. Cystic fibrosis is the most common lethal genetically transmitted disease among whites, but is uncommon in Asians, including Koreans. Although a case involving a Korean was reported in 1988, the focus was diagnosis rather than the radiological findings. In the case of cystic fibrosis we now describe, the focus is inverted: we emphasise the reported in 1988, focusing on radiologic findings

Dramatic Tumor Shrinkage of Locally Advanced and Inoperable Adenoid Cystic Carcinoma after Intra-arterial Chemotherapy

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Fu-Jen Hsueh

2015-06-01

Full Text Available Adenoid cystic carcinoma is rare and usually arises in the salivary glands. It grows slowly, but is characterized by easy perineural invasion with local infiltration and distant metastasis. In metastatic setting, the efficacy of intravenous chemotherapy is limited. Herein, we report one male patient who had a advanced, inoperable adenoid cystic carcinoma with lung metastasis, presenting with right buccal unhealed ulcer, pain and poor intake, whose loco-regional tumors responded dramatically after intra-arterial chemotherapy and his symptoms were almost completely relieved. We also make a literature review for treatment of adenoid cystic carcinoma.

Glucose 6-phosphate dehydrogenase deficiency and cystic fibrosis

OpenAIRE

Congdon, P. J.; Aggarwal, R. K.; Littlewood, J. M.; Shapiro, H.

1981-01-01

A child born to Pakistani parents is described. He had both cystic fibrosis and G-6PD-deficiency. So far as can be ascertained, the occurrence of both these conditions in the same individual has not previously been reported.

Anorexia nervosa in cystic fibrosis.

Science.gov (United States)

Linkson, Lynette; Macedo, Patricia; Perrin, Felicity M R; Elston, Caroline M

2018-03-01

This article explores the challenges associated with diagnosing and managing eating disorders such as anorexia nervosa amongst adolescents and adults with cystic fibrosis. It reviews the known risk factors, generic verses disease specific eating disorder risk screening tools and considers the ethical dilemmas associated with critically low body mass indices. A case review is included to illustrate the complexities of managing both conditions in the context of declining respiratory function. Copyright © 2017. Published by Elsevier Ltd.

[Italian Cystic Fibrosis Registry. Report 2011-2014].

Science.gov (United States)

Giordani, Barbara; Amato, Annalisa; Majo, Fabio; Ferrari, Gianluca; Quattrucci, Serena; Minicucci, Laura; Padoan, Rita; Floridia, Giovanna; Puppo Fornaro, Gianna; Taruscio, Domenica; Salvatore, Marco

2018-01-01

The Italian Cystic Fibrosis Registry (ICFR) is based on a new agreement about the data flow towards the Registry signed on October, 4th 2016 by the Centre for Rare Diseases of the Italian National Institute of Health (NIH), the clinicians of the Italian National Referral and Support Centres for Cystic Fibrosis, the Paediatric Hospital "Bambino Gesù" (Rome), the Italian Cystic Fibrosis Society, and the Italian League for Cystic Fibrosis. The aim of the present Report is to improve the knowledge on cystic fibrosis (CF) through the epidemiological description of Italian patients. The members of the Scientific and Technical Committee have to write a report on data collected by ICFR, in order to contribute to achieve the aims of ICFR itself, i.e., to improve the care of CF patients. In particular, the Report should contribute to the following objectives: - to analyze the medium and long-term clinical and epidemiological trends of the disease; - to identify the main healthcare needs at regional and national level in order to contribute to the healthcare programmes and to the distribution of resources; - to compare Italian data with the international ones. Analyses and results described in the present Report are referred to patients in charge to the Italian National Referral and Support Centres for Cystic Fibrosis in the period 2011-2014. Data were sent by Centres by means of a specific software (Camilla, Ibis Informatica) and has undergone a double quality control (QC): the first by NIH and the second at a European level (before the inclusion of the Italian data within the European Cystic Fibrosis Registry). These QCs assure the completeness and accuracy of data as well as their consistency with European core data. A total of 29 different CF centres (referral, support, and Paediatric Hospital "Bambino Gesù") sent their data to ICFR; data referred to the period 2011-2014. Data regarding Sardinia Region (Southern Italy) are missing; data from Molise (Southern Italy) CF

[Autosomal-recessive renal cystic disease and congenital hepatic fibrosis: clinico-anatomic case].

Science.gov (United States)

Rostol'tsev, K V; Burenkov, R A; Kuz'micheva, I A

2012-01-01

Clinico-anatomic observation of autosomal-recessive renal cystic disease and congenital hepatic fibrosis at two fetuses from the same family was done. Mutation of His3124Tyr in 58 exon of PKHD1 gene in heterozygous state was found out. The same pathomorphological changes in the epithelium of cystic renal tubules and bile ducts of the liver were noted. We suggest that the autopsy research of fetuses with congenital abnormalities, detected after prenatal ultrasonic screening, has high diagnostic importance.