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Sample records for acquired cystic kidney

  1. Acquired cystic kidney disease

    Choyke, P.L. [National Institutes of Health, Bethesda, MD (United States). Dept. of Diagnostic Radiology

    2000-11-01

    Acquired cystic kidney disease (ACKD), also known as acquired renal cystic disease (ARCD,) occurs in patients who are on dialysis for end-stage renal disease. It is generally accepted that ACKD develops as a consequence of sustained uremia and can first manifest even before dialysis is initiated while the patient is still in chronic renal failure. The role of immune suppression, particularly in transplant recipients, in the development of ACKD, is still under investigation. The prevalence of ACKD is directly related to the duration of dialysis and the risk of cancer is directly related to the presence of cysts. Herein we review the current understanding of the pathophysiology and imaging implications of ACKD. (orig.)

  2. Acquired cystic kidney disease

    Acquired cystic kidney disease (ACKD), also known as acquired renal cystic disease (ARCD,) occurs in patients who are on dialysis for end-stage renal disease. It is generally accepted that ACKD develops as a consequence of sustained uremia and can first manifest even before dialysis is initiated while the patient is still in chronic renal failure. The role of immune suppression, particularly in transplant recipients, in the development of ACKD, is still under investigation. The prevalence of ACKD is directly related to the duration of dialysis and the risk of cancer is directly related to the presence of cysts. Herein we review the current understanding of the pathophysiology and imaging implications of ACKD. (orig.)

  3. Acquired Cystic Kidney Disease

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  4. Metastatic malignant tumor in native kidney with acquired cystic disease after renal transplantation

    Patients on long-term hemodialysis frequently develop Acquired Cystic Renal Disease (ARCD). When hematuria or flank pain occurs, the possibility of malignant renal tumors should be investigated. The authors present an ARCD patient who received a kidney transplant and developed malignancy in a native kidney, the first manifestation being bone metastases, and discuss the role of CT in evaluating these patients. (authors). 9 refs.; 2 figs

  5. CT of acquired cystic kidney disease and renal tumors in long-term dialysis patients

    Levine, E.; Grantham, J.J.; Slusher, S.L.; Greathouse, J.L.; Krohn, B.P.

    1984-01-01

    The kidneys of long term dialysis patients frequently demonstrate multiple small acquired cysts and renal cell tumors on pathologic examination. The original kidneys of 30 long-term dialysis patients and six renal transplant patients were evaluated by computed tomography to determine the incidence of these abnormalities. Among dialysis patients, 43.3% had diffuse bilateral cysts, while 16.7% had occasional cysts (fewer than five per kidney), and 40% showed no renal cysts. Seven solid renal tumors were detected in four dialysis patients with renal cysts. Acquired cystic kidney disease tends to result in renal enlargement, is more common in patients who have been maintained on dialysis for prolonged periods, and may lead to spontaneous renal hemorrhage. The six transplant patients showed no evidence of renal cysts, and all had markedly shrunken kidneys. Acquired cystic disease and renal cell tumors in the original kidneys of dialysis patients may be due to biologically active substances that are not cleared effectively by dialysis but that are removed by normally functioning transplant kidneys.

  6. CT of acquired cystic kidney disease and renal tumors in long-term dialysis patients

    The kidneys of long term dialysis patients frequently demonstrate multiple small acquired cysts and renal cell tumors on pathologic examination. The original kidneys of 30 long-term dialysis patients and six renal transplant patients were evaluated by computed tomography to determine the incidence of these abnormalities. Among dialysis patients, 43.3% had diffuse bilateral cysts, while 16.7% had occasional cysts (fewer than five per kidney), and 40% showed no renal cysts. Seven solid renal tumors were detected in four dialysis patients with renal cysts. Acquired cystic kidney disease tends to result in renal enlargement, is more common in patients who have been maintained on dialysis for prolonged periods, and may lead to spontaneous renal hemorrhage. The six transplant patients showed no evidence of renal cysts, and all had markedly shrunken kidneys. Acquired cystic disease and renal cell tumors in the original kidneys of dialysis patients may be due to biologically active substances that are not cleared effectively by dialysis but that are removed by normally functioning transplant kidneys

  7. Long-term natural history of acquired cystic disease of the kidney.

    Ishikawa, Isao; Hayama, Satoshi; Morita, Kyoko; Nakazawa, Tetsuya; Yokoyama, Hitoshi; Honda, Ryumon; Satoh, Kyoko; Kakuma, Tatsuyuki

    2010-08-01

    Patients with acquired cystic disease of the kidney (ACDK) were followed longitudinally over an average of 21.7 +/- 5.4 years to determine the natural history of the disease; that is, how big the kidneys become, when the kidney size reaches a plateau, and when the size regresses. Twenty-seven male and 20 female patients with chronic glomerulonephritis treated at our hospital were investigated. CT scans were performed once a year and kidney volume was measured. Two different quadratic curves with a node of 5.2 years for males and 2.5 years for females after the start of hemodialysis were fitted to log-transformed kidney volume to the duration of hemodialysis using a linear mixed model. The maximum kidney volume in male patients was obtained 21.1 years after the start of hemodialysis using this model. Peak values of kidney volume were demonstrated in 19 of 26 cases during the observation period. The median peak value (interquartile range) of bilateral kidney volumes was 274 (165-849) mL/1.73 m(2) occurring 19.1 +/- 4.5 years after the start of dialysis. In one male patient who had undergone nephrectomy due to renal cell carcinoma and in two of the remaining 26 male patients, the maximum kidney volume of 782 (residual kidney), 1151, and 1129 mL regressed to 428, 616, and 847 mL (reduction rate: 45.3, 46.5, and 25.0%) at 20.6, 25.4, and 23.1 years after the start of hemodialysis, respectively. Kidney enlargement due to ACDK reached a plateau after 21.1 years of hemodialysis in the male patients. Partial regression of severe ACDK may occur naturally after long-term hemodialysis without renal transplantation. PMID:20649762

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

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

    1990-08-01

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

  9. Contrast-enhanced ultrasound assessment of complex cystic lesions in renal transplant recipients with acquired cystic kidney disease: preliminary experience.

    Paudice, N; Zanazzi, M; Agostini, S; Bertelli, E; Caroti, L; Carta, P; Moscarelli, L; Tsalouchos, A; Salvadori, M; Bertoni, E

    2012-09-01

    We prospectively studied the potential value of contrast-enhanced ultrasound (CEUS) to characterize complex acquired cystic kidney disease (ACKD) or suspected solid renal masses, avoiding the risk of inducing acute kidney injury in 138 renal transplant recipients by contrast-enhanced computed tomography (CT). Forty-three cases (31%) had ACKD; 15 ACKD patients (35%) showed suspicious or nondiagnostic ultrasound. The latter subgroup underwent CEUS and, if the suspicion was confirmed, a contrast-enhanced CT. Thirty five lesions were identified in the 15 patients studied by CEUS. According to the Bosniak classification, 27 cysts were type I (BI), four type II (BII), two type III (BIII) with enhancement at the level of thickened septa; we also identified two solid enhancing lesions (BIV). We followed the BI and BII lesions with serial CEUS, while the remaining four cases underwent contrast-enhanced CT showing two solid lesions and two complex cysts with contrast enhancement in the septea. The four patients underwent surgical resection yielding three renal cell carcinomas one papillary carcinoma as the pathological findings. This preliminary study characterized solid nodules and BIII lesions for further evaluation by CT. CEUS seems to correctly characterize BI and BII cysts that are not clearly defined by standard ultrasound. PMID:22974874

  10. Bilateral spontaneous perirenal hemorrhage in an acquired cystic kidney disease hemodialysis patient.

    Hirohama, Daigoro; Miyakawa, Hiroshi

    2012-01-01

    Acquired cystic kidney disease (ACKD) is a well-known late stage complication of chronic kidney disease. Cysts tend to grow with time on dialysis and could lead to malignant transformation, and intra- or perirenal hemorrhage is a rare complication of ACKD. Here we describe one case of bilateral spontaneous perirenal hemorrhage of ACKD in a 44-year-old man, on hemodialysis for 15 years. One was due to cyst rupture, and the other was due to aneurism rupture, both were controlled with transcatheter arterial embolization. In renal arteriography at the second rupture, we demonstrated extravasation from an aneurysm being present at the periphery of right renal artery. Several spontaneous perirenal hemorrhage cases were reported but its clinical information is limited, moreover, bilateral cases were extremely rare. Furthermore, to our knowledge, this is the first report of spontaneous perirenal hemorrhage caused by intraparenchymal renal artery aneurysm rupture in ACKD patients. We report this case because of its rarity and significance with respect to the complication of dialysis patients, review reported bilateral cases, and discuss some clinical characteristics. PMID:24533199

  11. Acquired cystic kidney disease in dialysis and renal transplant patients: ultrasonography and CT analysis

    To evaluate by US and CT the incidence and complications of acquired cystic kidney disease (ACKD) in dialysis and renal transplant patients and to compare the effectiveness of US and CT in the diagnosis of this entity. This study was prospectively performed in 70 dialysis patients and 13 renal transplant patients, and excluded any with multiple renal cysts or polycystic kidney disease, on as seen on initial films. US were obtained in all patients, and CT scans were randomly obtained in 27 who had been on dialysis for 3 years or more; all these US and CT scans were analyzed, with particular emphasis on whether or not cysts were present. In order to correlate the numbers of cysts with duration of dialysis, all patients were assigned to one of three groups, according to the number of cysts found (group 1, 0; group 2, 1-4; group 3, >4). Only group 3 was diagnosed as suffering from ACKD. In order to compare the cyst-detection capability of US with that of CT, 27 dialysis patients who had undergone US and CT were divided into four groups according to the number of cysts found (grade 1, 0; grade 2, 1-4; grade 3, 5-10; grade 4, >10). Seventy dialysis patients were divided according to the results of US, as follows : group 1, 20%; group 2, 47.1%; group 3, 32.9%. The mean duration of dialysis in group 1 (31.9 months) was statistically different from that in group 2 (50.6 months) and in group 3 (95.8 months) (p < 0.000). Thirteen renal transplant patients were divided as follows : group 1, 61.5%; group 2, 38.5%; group 3, 0%. In dialysis patients with ACKD, complications noted were renal cell carcinoma (n=1), hemorrhagic cysts (n=2), and hematomas (n=2). Among the 27 dialysis patients who underwent CT, this and US showed an equal grade of cystic change in 53.7%, while CT showed a higher grade in 46.3%. The detection rate of ACKD in these 27 patients was 46% on US and 63% on CT. A prolongation of dialysis corresponded to an increased incidence of ACKD; renal neoplasm and

  12. Acquired cystic kidney disease in dialysis and renal transplant patients: ultrasonography and CT analysis

    Jeon, Young Tae; Lee, Hae Kyung; Jung, Mi Sun; Yoon, Jong Pil; Hong, Hyun Sook; Kwon, Kui Hyang; Choi, Deuk Lin; Hwang, Seung Duk; Lee, Hi Bahl [Soonchunhyang Univ. College of Medicine, Seoul (Korea, Republic of)

    1997-04-01

    To evaluate by US and CT the incidence and complications of acquired cystic kidney disease (ACKD) in dialysis and renal transplant patients and to compare the effectiveness of US and CT in the diagnosis of this entity. This study was prospectively performed in 70 dialysis patients and 13 renal transplant patients, and excluded any with multiple renal cysts or polycystic kidney disease, on as seen on initial films. US were obtained in all patients, and CT scans were randomly obtained in 27 who had been on dialysis for 3 years or more; all these US and CT scans were analyzed, with particular emphasis on whether or not cysts were present. In order to correlate the numbers of cysts with duration of dialysis, all patients were assigned to one of three groups, according to the number of cysts found (group 1, 0; group 2, 1-4; group 3, >4). Only group 3 was diagnosed as suffering from ACKD. In order to compare the cyst-detection capability of US with that of CT, 27 dialysis patients who had undergone US and CT were divided into four groups according to the number of cysts found (grade 1, 0; grade 2, 1-4; grade 3, 5-10; grade 4, >10). Seventy dialysis patients were divided according to the results of US, as follows : group 1, 20%; group 2, 47.1%; group 3, 32.9%. The mean duration of dialysis in group 1 (31.9 months) was statistically different from that in group 2 (50.6 months) and in group 3 (95.8 months) (p < 0.000). Thirteen renal transplant patients were divided as follows : group 1, 61.5%; group 2, 38.5%; group 3, 0%. In dialysis patients with ACKD, complications noted were renal cell carcinoma (n=1), hemorrhagic cysts (n=2), and hematomas (n=2). Among the 27 dialysis patients who underwent CT, this and US showed an equal grade of cystic change in 53.7%, while CT showed a higher grade in 46.3%. The detection rate of ACKD in these 27 patients was 46% on US and 63% on CT. A prolongation of dialysis corresponded to an increased incidence of ACKD; renal neoplasm and

  13. [Renal failure and cystic kidney diseases].

    Correas, J-M; Joly, D; Chauveau, D; Richard, S; Hélénon, O

    2011-04-01

    Cystic kidney diseases often are discovered at the time of initial work-up of renal failure through ultrasound or family history, or incidentally at the time of an imaging test. Hereditary diseases include autosomal dominant or recessive polycystic kidney disease (PKD), tuberous sclerosis (TS) and medullary cystic kidney disease (MCKD). Autosomal dominant PKD is characterized by large renal cysts developing in young adults. Renal failure is progressive and becomes severe around 50-60 years of age. Atypical cysts (hemorrhagic or hyperdense) are frequent on CT and MRI examinations. Imaging plays a valuable role in the management of acute complications such as cyst hemorrhage or infection. Autosomal recessive PKD is often detected in neonates, infants or young adults. It is characterized by renal enlargement due to the presence of small cysts and liver disease (fibrosis and biliary ductal dilatation). Late manifestation or slow progression of autosomal recessive PKD may be more difficult to distinguish from autosomal dominant PKD. These cystic kidney diseases should not be confused with non-hereditary incidental multiple renal cysts. In tuberous sclerosis, renal cysts are associated with angiomyolipomas and sometimes pulmonary lymphangioleiomyomatosis. Renal failure is inconstant. Other hereditary cystic kidney diseases, including MCKD and nephronophtisis, are usually associated with renal failure. Non-hereditary cystic kidney diseases include multicystic renal dysplasia (due to complete pelvi-ureteric atresia or hydronephrosis), acquired multicystic kidney disease (chronic renal failure, chronic hemodialysis) and varied cystic kidney diseases (multicystic renal disease, glomerulocystic kidney disease, microcystic kidney disease). PMID:21549887

  14. Acquired Cystic Kidney Disease

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  15. Pilot study of non-contrast-enhanced MRI vs. ultrasound in renal transplant recipients with acquired cystic kidney disease: a prospective intra-individual comparison.

    Mühlfeld, Anja S; Lange, Christian; Kroll, Gisela; Floege, Jürgen; Krombach, Gabriele A; Kuhl, Christiane; Eitner, Frank; Schrading, Simone

    2013-01-01

    The incidence of renal cell carcinoma (RCC) after kidney transplantation is 15-fold increased. Acquired cystic kidney disease (ACKD) is one of the known risk factors. We performed a small pilot study to assess the role of non-enhanced magnetic resonance imaging (MRI) as a tool for intensified screening in renal transplant recipients with ACKD. Renal ultrasound was used to assess the native kidneys of 215 renal transplant recipients. Thirty patients with 54 kidneys, fulfilling the criteria of ACKD, underwent non-enhanced MRI at 1.5T using T2- and T1-weighed as well as diffusion-weighted sequences with a high spatial resolution. Among the 54 kidneys assessed by both methods, three RCCs were identified (6%). Of those, one RCC was detected by both imaging methods (33%), while two RCCs were diagnosed by MRI alone (67%). MRI identified an additional four proteinaceous or hemorrhagic cysts that did not fulfill the criteria for RCC but were classified as suspicious. All of these lesions were stable in size and appearance in follow-up studies. In conclusion, non-enhanced MRI was more sensitive than ultrasound in identifying RCCs and lesions suspicious for RCC and thus appears to be a useful secondary screening tool in patients with ACKD after renal transplantation. PMID:24118352

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

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

    2015-01-01

    Spontaneous retroperitoneal hemorrhage (SRH) is a rare and potentially fatal condition. Acquired cystic kidney disease (ACKD) may cause SRH in hemodialysis patients. However, presentation of retroperitoneal hematoma as hemoperitoneum in peritoneal dialysis (PD) patients is exceedingly rare. We report a 44-year-old male PD patient who presented with hemoperitoneum secondary to retroperitoneal hematoma. The reason of SRH was rupture of the cysts of ACKD. The patient underwent unilateral nephrectomy with subsequent disappearance of hemoperitoneum. The importance of this case lies in the fact that the patients who have been receiving dialysis for a long time should be under surveillance in terms of ACKD development and potential associated complications such as cyst hemorrhage and malignancy. PMID:26199480

  17. Medullary cystic kidney disease

    ... in the blood), especially with end-stage kidney disease Hypokalemia (too little potassium in the blood) Infertility Menstrual problems Miscarriage Pericarditis Peripheral neuropathy Platelet dysfunction with easy bruising Skin color ...

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

    Meola, Mario; Samoni, Sara; Petrucci, Ilaria

    2016-01-01

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

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

    Bhatnagar, Ramneesh; Alexiev, Borislav A

    2012-02-01

    Clear-cell papillary renal-cell carcinoma (CCPC) and acquired cystic kidney disease-associated carcinoma (ACDAC) are neoplasms with distinct morphological characteristics that behave less aggressively than conventional renal-cell carcinomas. End-stage kidney specimens from 61 patients (47 males and 14 females) with 109 renal-cell carcinomas were selected. Papillary renal-cell carcinoma was the most common malignancy (61/109, 56%), followed by CCPC (20/109, 18%). The CCPC showed a papillary or tubular/solid architecture, clear cytoplasm, low nuclear grade, and a distinct immunohistochemical profile (RCC-, vimentin+, CK7+, p504S-). ACDAC displayed a variety of architectural patterns, eosinophilic cytoplasm, high nuclear grade, intratumoral calcium oxalate deposits, and an immunohistochemical profile similar to type 2 papillary renal-cell carcinoma (RCC+, vimentin+, CK7-/+, p504S+). Less than 5% (3/69) of pathologically staged renal-cell carcinomas in end-stage kidneys presented with lymphogenous and/or hematogenous metastases. PMID:21791489

  20. Nephronophthisis and medullary cystic kidney disease complex

    Stanišić Marijana; Hrvačević Rajko; Paunić Zoran; Petrović Stanko

    2005-01-01

    Background. Nephronophthisis and medullary cystic kidney disease complex refers to the genetic heterogeneous group of inherited tubulointerstital nephritis. Nephronophthisis comprises at last 3 clinical manifestations, has the autosomal recessive pattern of inheritance, appears early in life and is the most frequent inherited kidney disease that causes terminal renal failure in childhood, while medullary cystic kidney disease has the autosomal dominant pattern of inheritance, is less frequent...

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

    H.M. Bezerra

    1997-03-01

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

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

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

  3. Nephronophthisis and medullary cystic kidney disease complex

    Stanišić Marijana

    2005-01-01

    Full Text Available Background. Nephronophthisis and medullary cystic kidney disease complex refers to the genetic heterogeneous group of inherited tubulointerstital nephritis. Nephronophthisis comprises at last 3 clinical manifestations, has the autosomal recessive pattern of inheritance, appears early in life and is the most frequent inherited kidney disease that causes terminal renal failure in childhood, while medullary cystic kidney disease has the autosomal dominant pattern of inheritance, is less frequent, and terminal renal failure appears later in life. These two forms have similar clinical and morphological findings but extrarenal manifestations, the median ages of occurrence of terminal renal failure, and siblings presence help us distinguish these diseases. Case report. In this article we illustrated the case of a 20- years old patient with the suspicion of having complex nephornophthisis and medullary cystic kidney disease based upon mild renal failure, seen in routinely taken laboratory findings and bilateral cysts in corticomedullary region of the kidneys verified on abdominal ultrasound examination. Conclusion. This disease should rise suspicion in children or adolescents with progressive renal failure, a typical clinical manifestation, blood and urine samples results, bilateral cysts in the corticomedullary region of the kidneys seen during ultrasound examination of the kidneys and family inheritance.

  4. Cystic gene dosage influences kidney lesions after nephron reduction.

    Le Corre, Stéphanie; Viau, Amandine; Burtin, Martine; El-Karoui, Khalil; Cnops, Yvette; Terryn, Sara; Debaix, Huguette; Bérissi, Sophie; Gubler, Marie-Claire; Devuyst, Olivier; Terzi, Fabiola

    2015-01-01

    Cystic kidney disease is characterized by the progressive development of multiple fluid-filled cysts. Cysts can be acquired, or they may appear during development or in postnatal life due to specific gene defects and lead to renal failure. The most frequent form of this disease is the inherited polycystic kidney disease (PKD). Experimental models of PKD showed that an increase of cellular proliferation and apoptosis as well as defects in apico-basal and planar cell polarity or cilia play a critical role in cyst development. However, little is known about the mechanisms and the mediators involved in acquired cystic kidney diseases (ACKD). In this study, we used the nephron reduction as a model to study the mechanisms underlying cyst development in ACKD. We found that tubular dilations after nephron reduction recapitulated most of the morphological features of ACKD. The development of tubular dilations was associated with a dramatic increase of cell proliferation. In contrast, the apico-basal polarity and cilia did not seem to be affected. Interestingly, polycystin 1 and fibrocystin were markedly increased and polycystin 2 was decreased in cells lining the dilated tubules, whereas the expression of several other cystic genes did not change. More importantly, Pkd1 haploinsufficiency accelerated the development of tubular dilations after nephron reduction, a phenotype that was associated to a further increase of cell proliferation. These data were relevant to humans ACKD, as cystic genes expression and the rate of cell proliferation were also increased. In conclusion, our study suggests that the nephron reduction can be considered a suitable model to study ACKD and that dosage of genes involved in PKD is also important in ACKD. PMID:25531116

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

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

    2010-01-01

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

  6. Clear cell papillary renal cell carcinoma and clear cell renal cell carcinoma arising in acquired cystic disease of the kidney: an immunohistochemical and genetic study.

    Kuroda, Naoto; Shiotsu, Tomoyuki; Kawada, Chiaki; Shuin, Taro; Hes, Ondrej; Michal, Michal; Ohe, Chisato; Mikami, Shuji; Pan, Chin-Chen

    2011-08-01

    Clear cell papillary renal cell carcinoma (RCC) is a recently established disease entity. However, there are few reports on genetic study of this entity. We report such a case with focus on genetic study. A 57-year-old Japanese man was found to have 3 renal tumors. Histologically, two tumors showed findings of clear cell RCC; and the other tumor showed findings of clear cell papillary RCC that was characterized by papillary growth pattern of neoplastic cells in cystic space with purely clear cell cytology. Immunohistochemically, tumor cells of clear cell papillary RCC were diffusely positive for PAX2 and cytokeratin 7, but negative for CD10, RCC Ma, and AMACR. In fluorescence in situ hybridization study for one clear cell papillary RCC, we detected polysomy for chromosome 7 and monosomy for chromosomes 17, 16, and 20. In addition, we detected mutation of VHL gene in clear cell RCC, but found no VHL gene mutation in clear cell papillary RCC. Finally, our results provide further evidence that clear cell papillary RCC may be both morphologically and genetically distinct entity from clear cell RCC and papillary RCC. PMID:20952286

  7. Acquired intrathoracic kidney in thoracic kyphosis

    Two cases of acquired intrathoracic kidney associated with thoracic kyphosis are reported, with emphasis on the radiographic manifestations. A search of the scientific literature disclosed that the acquired type of this abnormality is rare. The importance of recognizing this entity from a differential diagnostic standpoint is underscored. (author)

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

    Chijioke Adindu

    2010-01-01

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

  9. Acquired cystic disease and renal cell carcinoma in hemodialysis patients: A case report on three patients

    Mijušković Mirjana

    2015-01-01

    Full Text Available Introduction. Renal cell carcinoma (RCC is derived from renal tubular epithelial cells and represents approximately 3.8% of all malignancies in adults. The incidence of renal cell carcinoma has been growing steadily and ranging from 0.6 to 14.7 for every 100,000 inhabitants. Patients with end-stage renal disease and acquired cystic kidney disease are at increased risk of developing RCC while undergoing dialysis treatment or after renal transplantation. Case report. We presented 3 patients undergoing hemodialysis, with acquired cystic kidney disease accompanied by the development of RCC. In all the patients tumor was asymptomatic and discovered through ultrasound screening in 2 patients and in 1 of the patients by post-surgery pathohistological analysis of the tissue of the kidney excised using nephrectomy. All the three patients had organ-limited disease at the time of the diagnosis and they did not require additional therapy after surgical treatment. During the follow- up after nephrectomy from 6 months to 7 years, local recurrence or metastasis of RCC were not diagnosed. Conclusion. Acquired cystic kidney disease represents a predisposing factor for the development of renal cell carcinoma in dialysis patients and requires regular ultrasound examinations of the abdomen aimed at early diagnosis of malignancies. Prognosis for patients with endstage renal disease and RCC is mostly good because these tumors are usually of indolent course.

  10. Oligoarray comparative genomic hybridization of renal cell tumors that developed in patients with acquired cystic renal disease.

    Kuntz, Eva; Yusenko, Maria V; Nagy, Anetta; Kovacs, Gyula

    2010-09-01

    Renal cell carcinoma occurs at higher frequency in acquired cystic renal disease than in the general population. We have analyzed 4 tumors obtained from the kidneys of 2 patients with acquired cystic renal disease, including 2 conventional renal cell carcinomas and 2 acquired cystic renal disease-associated tumors, for genetic alterations. DNA changes were established by applying the 44K Agilent Oligonucleotide Array-Based CGH (Agilent Technologies, Waldbronn, Germany), and mutation of VHL gene was detected by direct sequencing of the tumor genome. DNA losses and mutation of the VHL gene, which are characteristic for conventional renal cell carcinomas, were seen in 2 of the tumors. The acquired cystic renal disease-associated eosinophilic-vacuolated cell tumor showed gain of chromosomes 3 and 16. No DNA alterations occurred in the papillary clear cell tumor. We suggest that not only the morphology but also the genetics of renal cell tumors associated with acquired cystic renal disease may differ from those occurring in the general population. PMID:20646738

  11. Outcome of Multi-Cystic Dysplastic Kidneys in Children

    Suleiman D. Mashat

    2015-10-01

    Full Text Available Background: Renal cystic diseases are important causes of chronic kidney disease (CKD. Objectives: We report the pattern of renal cystic disease in children and evaluate the outcome of children with multicystic dysplastic kidney (MCDK. Patients and Methods: Retrospective study of all children with cystic kidney diseases at King Abdulaziz University hospital from 2006 to 2014. Results: Total of 55 children (30 males; 25 MCDK, 22 polycystic kidney diseases (PKD, 4 nephronophthises and 4 renal cysts. Consanguinity was positive in 96.2%. MCDK and simple renal cyst patients had good renal function while PKD and nephronophthisis developed renal impairment. Most MCKD were diagnosed ante-natally, 16 of them were followed up for 3.4 (1.97 year. Their last creatinine was 33.9 (13.5 umol/L. MCDK was spontaneously involuted at mean age of 2.6 (1.3 years in 56%. Conclusions: MCDK is the commonest cystic renal disease and diagnosed ante-natally in the majority of cases. It has a good prognosis.

  12. [Cystic renal pathology].

    Rosi, P; Cesaroni, M; Bracarda, S; Rociola, W; Virgili, G

    1993-08-01

    Ultrasonography has a great interest in diagnosis of cystic kidney disorders for typical eco-pattern of this pathology. In this work we show the eco-pattern of the most common cystic kidney disorders. Particularly we examine simple cysts (typical, atypical, complicated), multicystic kidney dysplasia, autosomal recessive polycystic kidney disease (infantile) autosomal dominant polycystic kidney disease (adult age). The so-called neoplastic cysts (multiloculated cysts, multiloculated cysts nephroma, cystic nephroblastoma), medullar cysts (medullary sponge kidney, medullary cystic disease), parapyelic cysts, acquired cystic kidney disease in renal failure patients, parasitic cysts, epidermoid cysts. About this disorders we present the more typical and expressive ultrasonographic appearance and we define the role and the opportunity of diagnostic setting by echography, moreover ultrasonography allows us to make a differential diagnosis between cystic kidney disorders and other kidney disease. PMID:8353538

  13. Radiological diagnosis of hypernephromas in renal cysts or cystic kidneys

    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.)

  14. Acquired cystic disease-associated renal cell carcinoma: further characterization of the morphologic and immunopathologic features.

    Ahn, Soomin; Kwon, Ghee Young; Cho, Yong Mee; Jun, Sun-Young; Choi, Chan; Kim, Hyun-Jung; Park, Yong Wook; Park, Weon Seo; Shim, Jung Won

    2013-12-01

    Acquired cystic disease-associated renal cell carcinoma (ACD-RCC) is a subtype of renal cell carcinoma (RCC) with unique morphologic features found exclusively in the background of end-stage renal disease. We analyzed the clinicopathologic features and immumoreactive profiles of 12 cases of ACD-RCC to further characterize this recently recognized entity. Review of histologic slides was performed in conjunction with immunohistochemical staining directed to the contemporary diagnostic antibodies and the putative target therapy-related markers. Histologically, the tumors showed characteristic inter-or intracellular microlumens and eosinophilic tumor cells. Intratumoral hemosiderin deposition and degenerating foamy tumor cells were consistent findings which were not previously described. Immunohistochemically, all the tumors were positive for alpha-methylacyl-CoA-racemase, CD10, pan-cytokeratin, PTEN (phosphatase and tensin homolog deleted on chromosome 10) and c-met, while negative for carbonic anhydrase-9, CD57, CD68, c-kit, pax-2, platelet-derived growth factor receptor (PDGFR)-α or vascular endothelial growth factor receptor (VEGFR)-2. Heterogenous staining was found for CK7 and kidney-specific cadherin. Positive reaction to c-met suggests its utility as a plausible therapeutic target in ACD-RCC. Thus, we present the unique morphologic and immunopathologic features of ACD-RCC, which may be helpful in both diagnostic and therapeutic aspects. PMID:23471757

  15. Acquired cystic disease-associated renal cell carcinoma with sarcomatoid change and rhabdoid features.

    Kuroda, Naoto; Tamura, Masato; Hamaguchi, Nobumasa; Mikami, Shuji; Pan, Chin-Chen; Brunelli, Matteo; Martignoni, Guido; Hes, Ondrej; Michal, Michal; Lee, Gang-Hong

    2011-12-01

    Acquired cystic disease (ACD)-associated renal cell carcinoma (RCC) is a recently described entity. To the best of our knowledge, there are no reports of ACD-associated RCC with sarcomatoid and rhabdoid changes. In this article, we present the first case of such a tumor. A 56-year-old Japanese man has received long-term hemodialysis and had a history of right renal cancer. Following the discovery of metastatic cancer in the thoracic wall, detailed imaging studies revealed a mass in the left kidney. The histologic examination of the left renal tumor showed ACD-associated RCC with sarcomatoid change and rhabdoid features. Immunohistochemically, intracytoplasmic globular inclusions in rhabdoid cells were positive for vimentin and cytokeratin CAM5.2. The G-band karyotype showed the following changes: 46, X, +X. -Y[1]/43, idem, add(2)(q31), -6, -9, -14, -15, +16, -22, +mar1[6]/46, XY[2]/abnormal cell[11]. In conclusion, pathologists and urologists should be aware that rhabdoid features may occur in ACD-associated RCC and that the loss of chromosomes 9 and 14 may occur during the process of sarcomatoid change in ACD-associated RCC. PMID:21036640

  16. UNILATERAL CYSTIC RENAL DISEASE WITH DIFFUSE INVOLVEMENT OF KIDNEY: A CASE REPORT

    Manoj; De, Nabanita; Gautam

    2014-01-01

    : Unilateral renal cystic disease (URCD) is a rare, non-familial, non-progressive, unilateral cystic disorder of the kidney that is not associated with cysts or disorders in other organs and it is not related to other genetic cystic diseases. Only a few cases have been reported in the literature. We report a case of URCD in an adult male 49 years of age. Absence of a family history, benign clinical course and limitation of the disease to one kidney distinguish this condition f...

  17. Cystic renal neoplasms and renal neoplasms associated with cystic renal diseases in adults: cross-sectional imaging findings.

    Katabathina, Venkata S; Garg, Deepak; Prasad, Srinivasa R; Vikram, Raghu

    2012-01-01

    Cystic renal neoplasms in adults are a heterogeneous group of tumors with characteristic histogenesis, pathological findings, and variable biological profiles. They include disparate entities that are either biologically benign (lymphangioma, cystic nephroma, and mixed epithelial and stromal tumor) or malignant (cystic renal cell carcinoma, multilocular cystic renal cell carcinoma, and primary renal synovial sarcoma). Renal cystic diseases are characterized by cystic changes of the kidneys due to hereditary, developmental, or acquired etiology. Cystic renal diseases such as acquired cystic kidney disease, von Hippel-Lindau disease, and tuberous sclerosis are associated with the development of a wide spectrum of benign and malignant renal neoplasms. Most cystic renal tumors and cystic disease-associated renal neoplasms show characteristic cross-sectional imaging findings that permit accurate diagnosis. In addition, cross-sectional imaging is pivotal in the follow-up and surveillance of adult cystic tumors of the kidney. PMID:23192202

  18. Hypokalemic paralysis associated with cystic disease of the kidney: case report

    Jayasinghe Saroj; Rodrigo Chaturaka; Gamakaranage Champika SSSK; Rajapakse Senaka

    2011-01-01

    Abstract Background Severe hypokalemia is known to cause muscle paralysis, and renal tubular acidosis is a recognized cause. Cystic disease of the kidney is associated with severe hypokalemia. Case presentation We report a 33-year-old male patient who presented with generalized limb weakness caused by severe hypokalemia due to renal tubular acidosis, who was found to have renal medullary cysts. Conclusion The association of cystic renal disease with hypokalemia, and the possible pathophysiolo...

  19. Cystic diseases of the kidney in children: MRI, including RARE-MR-urography

    In ten children with cystic diseases of the kidney RARE-MR-urography (rapid acquisition with relaxation enhancement) and T1-weighted magnetic resonance imaging were performed. RARE-MR-urography rapidly and selectively depicts fluid by heavy T2 weighting. Thus, the entire kidney with all its cysts and the urinary tract can be displayed in one non-tomographic image, or preferably in multiple tomographic slices, should there be many small cysts. T1-weighted sequences show the renal parenchyma, signal intensity changes, and/or loss of corticomedullary differentiation. As regards morphology, ultrasound (US) will suffice for diagnostic purposes in most cystic diseases of the kidney. However, in very small cystic lesions where US shows a ''pepper and salt'' pattern due to insufficient spatial resolution, RARE-MR-urography can display the small fluid-filled spaces causing this appearance. (orig.)

  20. Enfermedad renal quística adquirida que simula una poliquistosis renal del adulto en un paciente en hemodiálisis crónica Acquired cystic kidney disease mimicking adult polycystic kidney disease in a patient undergoing chronic hemodialysis

    Yanet Parodis López

    2006-03-01

    Full Text Available Se presentó la evolución clínica de un paciente de 59 años de edad en hemodiálisis desde el año 1994, que llega a la insuficiencia renal crónica (IRC terminal por la vía aparente de la hipertensión arterial, que luego de 11 años en el proceder dialítico desarrolla un aumento de tamaño de los riñones con grandes quistes, cuyo aspecto en la ecografía y en la tomografía es indistinguible de una poliquistosis renal dominante del adulto.The clinical evolution of a 59-year-old patient on hemodialysis since 1994 that apparently reaches the end-stage chronic kidney failure (CKF by arterial hypertension is presented. After 11 years under the dialytic procedure, it is observed an increase of the size of the kidneys with large cysts, whose aspect in the echography and in the tomography is undistinguishable from an adult dominant polycystic kidney disease.

  1. Hypokalemic paralysis associated with cystic disease of the kidney: case report

    Jayasinghe Saroj

    2011-04-01

    Full Text Available Abstract Background Severe hypokalemia is known to cause muscle paralysis, and renal tubular acidosis is a recognized cause. Cystic disease of the kidney is associated with severe hypokalemia. Case presentation We report a 33-year-old male patient who presented with generalized limb weakness caused by severe hypokalemia due to renal tubular acidosis, who was found to have renal medullary cysts. Conclusion The association of cystic renal disease with hypokalemia, and the possible pathophysiological basis of the development of renal cysts in patients with severe hypokalemia, are discussed.

  2. Perinatal differential diagnosis of cystic kidney disease and urinary tract obstruction: anatomic pathologic, ultrasonographic and genetic findings.

    Friedmann, W; Vogel, M; Dimer, J S; Luttkus, A; Büscher, U; Dudenhausen, J W

    2000-04-01

    According to the classification of Osathanondh and Potter of cystic kidney diseases an antenatal differential diagnosis is presented, which is based on the anatomic pathologic, ultrasonographic and genetic findings. Since the ultrasound evaluation influences the obstetric and neonatal management, each second and third trimester sonography should consider the most common malformations in pediatric autopsies. The autosomal recessive polycystic kidney disease (ARPK), autosomal dominant polycystic kidney disease (ADPK), multicystic renal dysplasia, obstructive multicystic kidneys and cystic renal malformations found in other syndromes with genetic linkage are discussed in this review. PMID:10725570

  3. Therapeutic Approaches to Acquired Cystic Fibrosis Transmembrane Conductance Regulator Dysfunction in Chronic Bronchitis.

    Solomon, George M; Raju, S Vamsee; Dransfield, Mark T; Rowe, Steven M

    2016-04-01

    Chronic obstructive pulmonary disease is a common cause of morbidity and a rising cause of mortality worldwide. Its rising impact indicates the ongoing unmet need for novel and effective therapies. Previous work has established a pathophysiological link between the chronic bronchitis phenotype of chronic obstructive pulmonary disease and cystic fibrosis as well as phenotypic similarities between these two airways diseases. An extensive body of evidence has established that cigarette smoke and its constituents contribute to acquired dysfunction of the cystic fibrosis transmembrane conductance regulator (CFTR) protein in the airways, pointing to a mechanistic link with smoking-related and chronic bronchitis. Recent interest surrounding new drugs that target both mutant and wild-type CFTR channels has paved the way for a new treatment opportunity addressing the mucus defect in chronic bronchitis. We review the clinical and pathologic evidence for modulating CFTR to address acquired CFTR dysfunction and pragmatic issues surrounding clinical trials as well as a discussion of other ion channels that may represent alternative therapeutic targets. PMID:27115953

  4. Genetic and Non-Genetic Cystic Kidney: An Overview, Morphological Classification and Diagnostic Value of Imaging Procedures

    M. Mearadji

    2008-01-01

    Full Text Available Congenital renal cystic disease comprises a mixed group of developmental anomalies with different etiology, histology and clinical presentation. Progress in molecular genetics as well as use of prenatal and postnatal ultrasound contributed in early diagnosis and differentiation of congenital cystic diseases of the kidney. "nIn a retrospective study clinical and imaging findings of 131 infants and children with cystic diseases of kidney were collected with reviewing of clinical and imaging findings."nThis patient material is categorized in two groups: inherited renal cystic diseases and non-inherited cystic kidney diseases."nThe group of inherited renal diseases includes 28 patients with an autosomal dominant polycystic kidney disease (ADPKD. Eleven infants and children suffered from an autosomal recessive polycystic kidney disease (ARPKD. The remaining 6 of the 45 inherited cystic kidney diseases were 4 children with nephronophtisis and 2 with syndromal related cysts in kidney."nWithin the large group of patients with a non-inherited cystic kidney disease 79 infants and chil-dren were with one-sided non-functioning multicystic dysplastic kidney disease (MCKD. An ectopic ureter was found in 11 patients in this group and in 2 cases the ureter of the affected kidney was atretic. Lung hypoplasia was found in 2 cases with double-sided multicystic dysplastic kid-ney."nCystic nephroma (2 cases, renal cysts in malforma-tion syndromes (2 cases and the medullary sponge kidney (one case are the diagnosis of the remaining patients with a non-inherited renal cystic disease. "nSpecial attention should be payed to hepatic in-volvement in cases with ADPKD (hepatic cysts and ARPKD (liver fibrosis."nWithin the scope of this meeting the importance of early diagnostic and the incredible value of ultra-sound in prenatal and postnatal life will be empha-sized. The mode of inheritance in different genetic renal cystic diseases, complications and other addi

  5. Genetic spectrum of Saudi Arabian patients with antenatal cystic kidney disease and ciliopathy phenotypes using a targeted renal gene panel

    Al-Hamed, Mohamed H; Kurdi, Wesam; Alsahan, Nada; Alabdullah, Zainab; Abudraz, Rania; Tulbah, Maha; Alnemer, Maha; Khan, Rubina; Al-Jurayb, Haya; Alahmed, Ahmed; Tahir, Asma I; Khalil, Dania; Edwards, Noel; Al Abdulaziz, Basma; Binhumaid, Faisal S; Majid, Salma; Faquih, Tariq; El-Kalioby, Mohamed; Abouelhoda, Mohamed; Altassan, Nada; Monies, Dorota; Meyer, Brian; Sayer, John A; Albaqumi, Mamdouh

    2016-01-01

    Background Inherited cystic kidney disorders are a common cause of end-stage renal disease. Over 50 ciliopathy genes, which encode proteins that influence the structure and function of the primary cilia, are implicated in cystic kidney disease. Methods To define the phenotype and genotype of cystic kidney disease in fetuses and neonates, we correlated antenatal ultrasound examination and postnatal renal ultrasound examination with targeted exon sequencing, using a renal gene panel. A cohort of 44 families in whom antenatal renal ultrasound scanning findings in affected cases included bilateral cystic kidney disease, echogenic kidneys or enlarged kidneys was investigated. Results In this cohort, disease phenotypes were severe with 36 cases of stillbirth or perinatal death. Extra renal malformations, including encephalocele, polydactyly and heart malformations, consistent with ciliopathy phenotypes, were frequently detected. Renal gene panel testing identified causative mutations in 21 out of 34 families (62%), where patient and parental DNA was available. In the remaining 10 families, where only parental DNA was available, 7 inferred causative mutations were found. Together, mutations were found in 12 different genes with a total of 13 novel pathogenic variants, including an inferred novel variant in NEK8. Mutations in CC2D2A were the most common cause of an antenatal cystic kidney disease and a suspected ciliopathy in our cohort. Conclusions In families with ciliopathy phenotypes, mutational analysis using a targeted renal gene panel allows a rapid molecular diagnosis and provides important information for patients, parents and their physicians. PMID:26862157

  6. Review of acquired cystic disease-associated renal cell carcinoma with focus on pathobiological aspects.

    Kuroda, Naoto; Ohe, Chisato; Mikami, Shuji; Hes, Ondrej; Michal, Michal; Brunelli, Matteo; Martignoni, Guido; Sato, Yasuharu; Yoshino, Tadashi; Kakehi, Yoshiyuki; Shuin, Taro; Lee, Gang-Hong

    2011-09-01

    Acquired cystic disease (ACD)-associated renal cell carcinoma (RCC) is a recently established entity. In this article, we introduce the general view of this new entity. Macroscopically, the disease exclusively occurs in ACD and may arise as a dominant mass or non-dominant masses. Histologically, the tumor is characterized by a microcystic pattern, neoplastic cells with an eosinophilic or oncocytic cytoplasm and frequent intratumoral oxalate crystal deposition. Prominent nucleoli of tumor cells are often observed. Immunohistochemically, neoplastic cells are generally positive for AMACR but negative for cytokeratin 7. Ultrastructurally, neoplastic cells contain abundant mitochondria in the cytoplasm. Genetically, the gain of chromosomes 3, 7, 17 and abnormality of the sex chromosome were frequently observed in several studies. In conclusion, ACD-associated RCC may be widely recognized as a distinct entity in the near future because this tumor is morphologically and genetically different from other renal tumor entities that have been previously established. PMID:21751153

  7. Acquired cystic disease-associated renal cell carcinoma: an immunohistochemical and fluorescence in situ hybridization study.

    Kuroda, Naoto; Yamashita, Motoki; Kakehi, Yoshiyuki; Hes, Ondrej; Michal, Michal; Lee, Gang-Hong

    2011-12-01

    Acquired cystic disease (ACD)-associated renal cell carcinoma (RCC) has been recently identified. However, there are only a few genetic studies to date. In this article, we performed an immunohistochemical and fluorescence in situ hybridization (FISH) study for six cases including one case with sarcomatoid change. As a result, we observed frequent immunohistochemical expression of AMACR. FISH of chromosome 3 showed trisomy for three cases, monosomy for two cases, and disomy for one case. Additionally, FISH of chromosome 16 showed trisomy for three cases, monosomy for two cases, and both trisomy and monosomy for one case. Furthermore, both the carcinomatous area and the sarcomatoid area of one ACD-associated RCC with sarcomatoid change revealed monosomy of chromosomes 3, 9, and 16 but showed disomy of chromosome 14. In conclusion, the numerical abnormalities of chromosomes 3 and 16, irrespective of gain or loss, may be characteristic of ACD-associated RCC. PMID:22179186

  8. Localized Cystic Disease of the Kidney: A Rare Cause of Hypertension in a Young Adult

    Aynur Solak

    2013-01-01

    Full Text Available Localized cystic disease of kidney (LCDK is a rare, non-familial, non-progressive renal disorder that is not associated with cysts or disorders in other organs. Only a few cases have been reported in the literature. While this condition is morphologically identical to the autosomal dominant form of polycystic kidney disease, it is not inherited and is not associated with significant deterioration of renal function. We present a case of a 16-year-old male patient who suffered from hypertension for over two years. On imaging we found several, variable-sized cysts in the upper half of the right kidney. The left kidney and lower segment of the right kidney were normal. Selective renal vein catheterization and sampling showed markedly elevated renin level in the right upper segmental vein (92 pg/ml, normal value: 11-33 pg/ml. The patient underwent a right upper heminephrectomy and histopathology was suggestive of LCDK. After surgery, the patient′s blood pressure returned to normal levels without any need of antihypertensive medication and he is under follow-up on outpatient basis for the past two years.

  9. Bilateral multiple cystic kidney disease and renal cortical abscess in a Boerboel.

    Kitshoff, A M; McClure, V; Lim, C K; Kirberger, R M

    2011-06-01

    Cystic renal disease is rare in dogs and although infected renal cysts have been reported in humans, no report could be found in dogs. A 58 kg, 5-year-old, castrated, male Boerboel presented with weight loss, pyrexia, lethargy and vomiting, 20 months after an incident of haematuria was reported. The initial ultrasonographic diagnosis was bilateral multiple renal cysts of unknown aetiology. The cysts had significantly increased in size over the 20-month period and some contained echogenic specks which could be related to infection, normal cellular debris or haemorrhage. In both kidneys the renal contours were distorted (the left more than the right). The abnormal shape of the left kidney was largely due to multiple cysts and a large crescent-shaped septate mass on the cranial pole of the kidney. Aspirates of the septate mass were performed (left kidney) and the cytology and culture were indicative of an abscess. It is suggested that the previous incident of haematuria provided a portal of entry for bacteria into the cysts resulting in renal cortical abscess formation. PMID:22135926

  10. Bilateral multiple cystic kidney disease and renal cortical abscess in a Boerboel

    A. M. Kitshoffa

    2011-04-01

    Full Text Available Cystic renal disease is rare in dogs and although infected renal cysts have been reported in humans, no report could be found in dogs. A 58 kg, 5-year-old, castrated, male Boerboel presented with weight loss, pyrexia, lethargy and vomiting, 20 months after an incident of haematuria was reported. The initial ultrasonographic diagnosis was bilateral multiple renal cysts of unknown aetiology. The cysts had significantly increased in size over the 20-month period and some contained echogenic specks which could be related to infection, normal cellular debris or haemorrhage. In both kidneys the renal contours were distorted (the left more than the right. The abnormal shape of the left kidney was largely due to multiple cysts and a large crescent-shaped septate mass on the cranial pole of the kidney. Aspirates of the septate mass were performed (left kidney and the cytology and culture were indicative of an abscess. It is suggested that the previous incident of haematuria provided a portal of entry for bacteria into the cysts resulting in renal cortical abscess formation.

  11. CT and MR imaging for evaluation of cystic renal lesions and diseases.

    Wood, Cecil G; Stromberg, LeRoy J; Harmath, Carla B; Horowitz, Jeanne M; Feng, Chun; Hammond, Nancy A; Casalino, David D; Goodhartz, Lori A; Miller, Frank H; Nikolaidis, Paul

    2015-01-01

    Cystic renal lesions are commonly encountered in abdominal imaging. Although most cystic renal lesions are benign simple cysts, complex renal cysts, infectious cystic renal disease, and multifocal cystic renal disease are also common phenomena. The Bosniak classification system provides a useful means of categorizing cystic renal lesions but places less emphasis on their underlying pathophysiology. Cystic renal diseases can be categorized as focal, multifocal, or infectious lesions. Diseases that manifest with focal lesions, such as cystic renal cell carcinoma, mixed epithelial and stromal tumor, and cystic nephroma, are often difficult to differentiate but have differing implications for follow-up after resection. Multifocal cystic renal lesions can be categorized as acquired or heritable. Acquired entities, such as glomerulocystic kidney disease, lithium-induced nephrotoxicity, acquired cystic kidney disease, multicystic dysplastic kidney, and localized cystic renal disease, often have distinct imaging and clinical features that allow definitive diagnosis. Heritable diseases, such as autosomal dominant polycystic kidney disease, von Hippel-Lindau disease, and tuberous sclerosis, are usually easily identified and have various implications for patient management. Infectious diseases have varied imaging appearances, and the possibility of infection must not be overlooked when assessing a cystic renal lesion. A thorough understanding of the spectrum of cystic renal disease will allow the radiologist to make a more specific diagnosis and provide the clinician with optimal recommendations for further diagnostic testing and follow-up imaging. PMID:25590393

  12. Adult renal cystic disease: a genetic, biological, and developmental primer.

    Katabathina, Venkata S; Kota, Gopi; Dasyam, Anil K; Shanbhogue, Alampady K P; Prasad, Srinivasa R

    2010-10-01

    Renal cystic diseases in adults are a heterogeneous group of disorders characterized by the presence of multiple cysts in the kidneys. These diseases may be categorized as hereditary, acquired, or developmental on the basis of their pathogenesis. Hereditary conditions include autosomal dominant polycystic kidney disease, medullary cystic kidney disease, von Hippel-Lindau disease, and tuberous sclerosis. Acquired conditions include cystic kidney disease, which develops in patients with end-stage renal disease. Developmental cystic diseases of the adult kidney include localized renal cystic disease, multicystic dysplastic kidney, and medullary sponge kidney. In recent years, many molecular and cellular mechanisms involved in the pathogenesis of renal cystic diseases have been identified. Hereditary renal cystic diseases are characterized by genetic mutations that lead to defects in the structure and function of the primary cilia of renal tubular epithelial cells, abnormal proliferation of tubular epithelium, and increased fluid secretion, all of which ultimately result in the development of renal cysts. A better understanding of these pathophysiologic mechanisms is now providing the basis for the development of more targeted therapeutic drugs for some of these disorders. Cross-sectional imaging provides useful information for diagnosis, surveillance, prognostication, and evaluation of treatment response in renal cystic diseases. PMID:21071372

  13. Raloxifene improves skeletal properties in an animal model of cystic chronic kidney disease.

    Newman, Christopher L; Creecy, Amy; Granke, Mathilde; Nyman, Jeffry S; Tian, Nannan; Hammond, Max A; Wallace, Joseph M; Brown, Drew M; Chen, Neal; Moe, Sharon M; Allen, Matthew R

    2016-01-01

    Patients with chronic kidney disease (CKD) have an increased risk of fracture. Raloxifene is a mild antiresorptive agent that reduces fracture risk in the general population. Here we assessed the impact of raloxifene on the skeletal properties of animals with progressive CKD. Male Cy/+ rats that develop autosomal dominant cystic kidney disease were treated with either vehicle or raloxifene for five weeks. They were assessed for changes in mineral metabolism and skeletal parameters (microCT, histology, whole-bone mechanics, and material properties). Their normal littermates served as controls. Animals with CKD had significantly higher parathyroid hormone levels compared with normal controls, as well as inferior structural and mechanical skeletal properties. Raloxifene treatment resulted in lower bone remodeling rates and higher cancellous bone volume in the rats with CKD. Although it had little effect on cortical bone geometry, it resulted in higher energy to fracture and modulus of toughness values than vehicle-treated rats with CKD, achieving levels equivalent to normal controls. Animals treated with raloxifene had superior tissue-level mechanical properties as assessed by nanoindentation, and higher collagen D-periodic spacing as assessed by atomic force microscopy. Thus, raloxifene can positively impact whole-bone mechanical properties in CKD through its impact on skeletal material properties. PMID:26489025

  14. Hospital-acquired acute kidney injury in medical, surgical, and intensive care unit: A comparative study

    Singh, T. B.; Rathore, S. S.; Choudhury, T. A.; Shukla, V. K.; D.K. Singh; Prakash, J.

    2013-01-01

    Acute kidney injury (AKI) is a common complication in hospitalized patients. There are few comparative studies on hospital-acquired AKI (HAAKI) in medical, surgical, and ICU patients. This study was conducted to compare the epidemiological characteristics, clinical profiles, and outcomes of HAAKI among these three units. All adult patients (>18 years) of either gender who developed AKI based on RIFLE criteria (using serum creatinine), 48 h after hospitalization were included in the study. Pat...

  15. Acquired cystic disease-associated renal cell carcinoma with gain of chromosomes 3, 7, and 16, gain of chromosome X, and loss of chromosome Y.

    Kuroda, Naoto; Shiotsu, Tomoyuki; Hes, Ondrej; Michal, Michal; Shuin, Taro; Lee, Gang-Hong

    2010-12-01

    Acquired cystic disease (ACD)-associated renal cell carcinoma (RCC) has been recently described. To date, there are no reports on genetic findings of G-band karyotype of ACD-associated RCC. In this article, we report the first report of G-band karyotype of ACD-associated RCC. A 66-year-old Japanese man was found to have a left renal tumor during the follow-up of hemodialysis consequent to chronic renal failure. Left nephrectomy was performed. Histological examination of three tumors in the left kidney showed the cribriform or microcystic growth pattern of neoplastic cells with eosinophilic cytoplasm, and many oxalate crystals were observed. The G-band karyotype of ACD-associated RCC showed 49, X, +X, -Y, +3, +7, +16. These chromosomal abnormalities resemble those of sporadic papillary RCC that has been previously reported. Finally, we suggest that this tumor may show a close relationship between ACD-associated RCC and papillary RCC, but a large-scale study will be needed to clarify the relationship between ACD-associated RCC and papillary RCC. PMID:21267700

  16. Renal cystic disease: A practical overview

    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

  17. Renal cystic disease

    Hartman, D.S.

    1988-01-01

    The book begins with an overview of renal cystic disease and a presentation of simple renal cysts. Subsequent chapters cover cystic disease in association with renal neoplasms and medullary sponge kidney. The chapters addressing autosomal-dominant and autosomal-recessive polycystic kidney disease discuss and differentiate the infantile and adult forms of the disease. There are also separate discussions of medullary cystic disease, multicystic dysplastic kidney, and cysts of the renarenal sinus.

  18. Imaging of acquired cystic disease-associated renal cell carcinoma by contrast-enhanced ultrasonography with perflubutane microbubbles and positron emission tomography-computed tomography.

    Ishikawa, Isao; Morita, Kyoko; Hayama, Satoshi; Nakazawa, Tetsuya; Araki, Ichiro; Higashi, Kotaro; Miyazawa, Katsuhito; Suzuki, Koji; Nojima, Takayuki

    2011-02-01

    The preoperative assessment of renal cell carcinoma (RCC) complicated with acquired renal cystic disease in a 63-year-old male patient on long-term hemodialysis (30 years and 8 months) that was difficult because of no or poor contrast enhancement by dynamic CT scan is reported. Contrast-enhanced ultrasonography with perflubutane microbubbles and positron emission tomography-computed tomography (PET-CT) with 18F-fluorodeoxy glucose (FDG) in addition to dynamic CT were effective and useful for preoperative assessment of this patient. The pathological subtype of RCC in this patient was acquired cystic disease-associated RCC (ACD-associated RCC), which has been newly defined by Tickoo et al. (Am J Surg Pathol 30:141-153, 2006). PMID:20824295

  19. Medullary cystic disease of the kidney: report of a case diagnosed by ultrasonography and computed tomography examinations; Doenca cistica medular do rim: relato de um caso diagnosticado por ultra-sonografia e tomografia computadorizada

    Carvalho, Tarcisio Nunes; Araujo Junior, Cyrillo Rodrigues de; Fraguas Filho, Sergio Roberto; Costa, Marlos Augusto Bittencourt; Teixeira, Kim-Ir-Sen Santos [Goias Univ., Goiania, GO (Brazil). Hospital de Clinicas. Dept. de Diagnostico por Imagem]. E-mail: Tracisionunes@hotmail.com.br; Ribeiro, Flavia Aparecida de Souza [Goias Univ., Goiania, GO (Brazil). Hospital de Clinicas. Servico de Diagnostico por Imagem

    2003-12-01

    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)

  20. Kidney biopsy

    ... Goodpasture syndrome IgA nephropathy Interstitial nephritis Lupus nephritis Medullary cystic kidney disease Membranoproliferative glomerulonephritis Membranous nephropathy Minimal change disease Nephrotic ...

  1. Synchronous primary carcinoid tumor and primary adenocarcinoma arising within mature cystic teratoma of horseshoe kidney: a unique case report and review of the literature

    Perepletchikov Aleksandr M

    2009-06-01

    Full Text Available Abstract Background Malignant transformation of mature cystic teratoma is a rare complication. While any of the constituent tissues of a teratoma has the potential to undergo malignant transformation, squamous cell carcinoma is the most commonly associated malignancy. Renal carcinoid tumors are rare and frequently associated with horseshoe kidney and renal teratoma. Renal teratoma rarely presents together with carcinoid tumor or adenocarcinoma. To the best of our knowledge, there has never been a report of renal teratoma coexisting with both carcinoid tumor and adenocarcinoma. Methods Here, we present a unique and first case of synchronous primary carcinoid tumor and moderately differentiated adenocarcinoma arising within mature cystic teratoma of horseshoe kidney in a 50-year-old female. Lumbar spine X-ray, done for her complaint of progressive chronic low back pain, accidentally found a large calcification overlying the lower pole of the right kidney. Further radiologic studies revealed horseshoe kidney and a large multiseptated cystic lesion immediately anterior to the right renal pelvis with central calcification and peripheral enhancement. She underwent right partial nephrectomy. Results Macroscopically, the encapsulated complex solid and multiloculated cystic tumor with large calcification, focal thickened walls and filled with yellow-tan gelatinous material. Microscopically, the tumor showed coexistent mature cystic teratoma, moderately differentiated adenocarcinoma and carcinoid tumor. Immunohistochemically, alpha-methylacyl-coenzyme A-racemase, calretinin, CD10 and thyroid transcription factor-1 were negative in all the three components of the tumor. The teratomatous cysts lined by ciliated epithelium showed strong staining for cytokeratin 7 and pancytokeratin, and those lined by colonic-like epithelium showed strong staining for CDX2, cytokeratin 20 and pancytokeratin, but both were negative for calretinin. Additionally, the

  2. Cyst number but not the rate of cystic growth is associated with the mutated gene in autosomal dominant polycystic kidney disease.

    Harris, Peter C; Bae, Kyongtae T; Rossetti, Sandro; Torres, Vicente E; Grantham, Jared J; Chapman, Arlene B; Guay-Woodford, Lisa M; King, Bernard F; Wetzel, Louis H; Baumgarten, Deborah A; Kenney, Philip J; Consugar, Mark; Klahr, Saulo; Bennett, William M; Meyers, Catherine M; Zhang, Qin Jean; Thompson, Paul A; Zhu, Fang; Miller, J Philip

    2006-11-01

    Data from serial renal magnetic resonance imaging of the Consortium of Radiologic Imaging Study of PKD (CRISP) autosomal dominant polycystic kidney disease (PKD) population showed that cystic expansion occurs at a consistent rate per individual, although it is heterogeneous in the population, and that larger kidneys are associated with more rapid disease progression. The significance of gene type to disease progression is analyzed in this study of the CRISP cohort. Gene type was determined in 183 families (219 cases); 156 (85.2%) had PKD1, and 27 (14.8%) had PKD2. PKD1 kidneys were significantly larger, but the rate of cystic growth (PKD1 5.68%/yr; PKD2 4.82%/yr) was not different (P = 0.24). Cyst number increased with age, and more cysts were detected in PKD1 kidneys (P cysts develop earlier, not because they grow faster, implicating the disease gene in cyst initiation but not expansion. These insights will inform the development of targeted therapies in autosomal dominant PKD. PMID:17035604

  3. 肾脏多房囊性病变的影像学表现%The image features of multiocular cystic lesions of the kidney

    翁燕香; 王祖飞; 陈正华

    2015-01-01

    目的:探讨肾脏多房性囊性病变的 CT 及 MRI 表现特征,提高对其诊断的准确性。方法回顾性分析经手术病理证实的多房囊性病变共28例,26例行 CT 平扫及增强扫描,10例行 MRI 扫描。结果总共28例,均为多房囊性病变,其中囊性肾癌18例,囊壁及分隔厚薄不均,5例可见壁结节,4例囊内见絮状悬浮物,17例增强扫描囊壁及囊内分隔呈快进快出强化,囊液未见强化。另外10例 ,多房囊性肾瘤 4例 、多房性肾囊肿 5例 、肾髓质囊肿病1例,其中 7例囊壁及囊内分隔均匀,3例囊壁及分隔粗细不均,最厚处小于3mm ,增强扫描 5例囊壁及囊内分隔不同程度强化,5例囊壁及分隔未见强化,10例囊内均未见强化,未见壁结节。结论多房囊性肾癌与其它多房囊性病变的影像表现特征,有助于提高其术前诊断的准确率。%Objective To analyze the CT and MRI features of multilocular cystic lesions of the kidney in order to improve the accuracy of the diagnosis .Methods This retrospective analysis involved 28 patients with multilocular cystic lesions of the kidney .All these tumors were pathologically confirmed after surgery or biopsy .Among them ,26 patients underwent CT scan ,10 patients underwent MRI scan .Results Those tumors included cystic renal cell carcinoma (18 cases) ,multi‐locular cystic renal tumors (4 cases) ,multilocular renal cyst (5 cases) ,and renal medullary cystic disease (1 case) .Un‐regular cystic wall were found in all cystic renal cell carcinoma ,papillary projection (5 cases) and layering debris (4 cases) were in cystic renal cell carcinoma .The cyst masses (17 cases) were obvious enhancement in the arterial phase ,and de‐creased degree of enhancement in portal venous phase and delay phase .In the remaining 10 cases of multilocular cystic le‐sions ,7 cases were regular cystic wall and 3 cases were unregular cystic wall with the thickest of

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

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

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

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

    2002-03-15

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

  6. Autosomal Dominant Polycystic Kidney Disease Patient Specified Bilateral Renal Mass: A Case Report

    Ercan Ogreden

    2014-12-01

    Full Text Available Hereditary cystic lesions of the kidney cysts nonherediter lesion with a wide range. They may be the only finding in the form of extrarenal renal cysts may also be part of a clinical syndrome. For this reason, multi-disciplinary approach brings with cystic lesions in the different units. Autosomal dominant polycystic kidney disease, kidney different sizes and numbers of both systemic and hereditary disease that manifests itself in the form of cystic changes. Hereditary and acquired cystic lesions on the basis of some of the rare tumor association is determined in several studies. Here are diagnosed with autosomal dominant polycystic kidney disease, abdominal pain because of a mass in the last six months, physical examination, radiological and laboratory findings were diagnosed with bilateral renal tumors are uncommon and the right renal mass nephron-sparing surgery, the patients with left renal mass followed with active surveillance discussed in the current literature.

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

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

    2014-03-01

    Although local recurrence of renal cell carcinoma after laparoscopic radical nephrectomy is sometimes reported, cystic local recurrence of renal cell carcinoma has rarely been reported. We report the case of a 59-year-old man with hemodialysis who developed cystic local recurrence of renal cell carcinoma accompanied by acquired cystic disease of the kidney in the retroperitoneal space after laparoscopic radical nephrectomy. A cystic tumor of 5.1 cm in diameter occurred in the left retroperitoneal space 15 months after left laparoscopic radical nephrectomy, and enlarged to 7.2 cm in diameter with enhanced mass along the wall of the cyst 36 months after surgery. The cystic tumor was removed and showed local recurrence of renal cell carcinoma on pathological examination. PMID:23909823

  8. Cystic renal tumors: new entities and novel concepts.

    Moch, Holger

    2010-05-01

    Cystic renal neoplasms and renal epithelial stromal tumors are diagnostically challenging and represent some novel tumor entities. In this article, clinical and pathologic features of established and novel entities are discussed. Predominantly cystic renal tumors include cystic nephroma/mixed epithelial and stromal tumor, synovial sarcoma, and multilocular cystic renal cell carcinoma. These entities are own tumor entities of the 2004 WHO classification of renal tumors. Tubulocystic carcinoma and acquired cystic disease-associated renal cell carcinoma are neoplasms with an intrinsically cystic growth pattern. Both tumor types should be included in a future WHO classification as novel entities owing to their characteristic features. Cysts and clear cell renal cell carcinoma frequently coexist within the kidneys of patients with von Hippel-Lindau disease. Sporadic clear cell renal cell carcinomas often contain cysts, usually as a minor component. Some clear cell renal cell carcinomas have prominent cysts, and multilocular cystic renal cell carcinoma is composed almost exclusively of cysts. Recent molecular findings suggest that clear cell renal cancer may develop through cyst-dependent and cyst-independent molecular pathways. PMID:20418675

  9. Cyst infection in unilateral renal cystic disease and the role of diffusion-weighted magnetic resonance imaging.

    Takase, Yasukazu; Kodama, Koichi; Motoi, Isamu; Saito, Katsuhiko

    2012-11-01

    In multicystic renal diseases, cyst infection is a complex issue because of the absence of validated diagnostic methods. Unilateral renal cystic disease is a rare multicystic disease, believed to have an acquired maldevelopmental origin. Unilateral renal cystic disease is often confused with autosomal dominant polycystic kidney disease but has some distinguishing characteristics: unilateral localization, negative family history, and no progression to chronic renal failure. We describe a case of unilateral renal cystic disease with cyst infection that could be detected by diffusion-weighted magnetic resonance imaging, but not by conventional imaging techniques. Diffusion-weighted magnetic resonance imaging can be useful for detecting infected cysts, especially in multicystic renal diseases. PMID:22990058

  10. The ratio of CRP to prealbumin levels predict mortality in patients with hospital-acquired acute kidney injury

    Hao Chuanming

    2011-06-01

    Full Text Available Abstract Background Animal and human studies suggest that inflammation and malnutrition are common in acute kidney injury (AKI patients. However, only a few studies reported CRP, a marker of inflammation, albumin, prealbumin and cholesterol, markers of nutritional status were associated with the prognosis of AKI patients. No study examined whether the combination of inflammatory and nutritional markers could predict the mortality of AKI patients. Methods 155 patients with hospital-acquired AKI were recruited to this prospective cohort study according to RIFLE (Risk, Injury, Failure, Lost or End Stage Kidney criteria. C-reactive protein (CRP, and the nutritional markers (albumin, prealbumin and cholesterol measured at nephrology consultation were analyzed in relation to all cause mortality of these patients. In addition, CRP and prealbumin were also measured in healthy controls (n = 45, maintenance hemodialysis (n = 70 and peritoneal dialysis patients (n = 50 and then compared with AKI patients. Results Compared with healthy controls and end-stage renal disease patients on maintenance hemodialysis or peritoneal dialysis, patients with AKI had significantly higher levels of CRP/prealbumin (p 28 days. Similarly, the combined factors including the ratio of CRP to albumin (CRP/albumin, CRP/prealbumin and CRP/cholesterol were also significantly higher in the former group (p p = 0.027 while the others (CRP, albumin, prealbumin, cholesterol, CRP/albumin and CRP/cholesterol became non-significantly associated. The hazard ratio was 1.00 (reference, 1.85, 2.25 and 3.89 for CRP/prealbumin increasing according to quartiles (p = 0.01 for the trend. Conclusions Inflammation and malnutrition were common in patients with AKI. Higher level of the ratio of CRP to prealbumin was associated with mortality of AKI patients independent of the severity of illness and it may be a valuable addition to SOFA score to independent of the severity of illness and it may be a

  11. Mature Cystic Renal Teratoma

    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

  12. Clinical and pathological analyses of medullary cystic kidney disease%肾髓质囊肿病的临床病理分析

    梁或; 陈育青; 王素霞; 刘颖; 鄂洁; 张宏

    2011-01-01

    Objectives Medullary cystic kidney disease (MCKD) is a tubulointerstitial nephropathy leading to end-stage renal failure. We combine the clinical and pathological characteristics as well as laboratory examinations to discuss the diagnosis of MCKD. Methods A total of 156 individuals with tubulointerstitial nephropathy were assigned into 3 groups, acute tubulointerstitial nephropathy (ATIN), chronic tubulointerstitial nephropathy (CTIN) and MCKD. Clinical data and pathological findings were analyzed. Immunohistochemistry staining of uromodulin was performed for MCKD cases. Urinary uromodulin concentrations in MCKD and 99 healthy people were tested by ELISA. Results The age at diagnosis was much younger in MCKD than in CTIN. Serum uric acid level was significantly higher in MCKD than in CTIN patients (P=0.011). Uromodulin staining by immunohis-tochemistry showed block mass and dense stain in tubular cells, while it was diffused in cytoplasm with apical reinforce in normal controls. Urinary uromodulin concentration was much lower in MCKD (P=0.047). Conclusions MCKD as one cause of tubulointerstitial nephropathy should not be ignored. Clinical data, pathological and laboratory examinations are useful for its diagnosis.%目的 探讨肾髓质囊肿病的诊断和病理特点.方法 156例病理诊断为肾小管间质肾病患者分为急性肾小管间质肾病(acute tubulointerstitial nephropathy,ATIN)组、慢性肾小管间质肾病(chronic tubulointerstitial nephropathy,CTIN)组和肾髓质囊肿病(medullary cystic kidney disease,MCKD)组,分析临床和病理,肾组织尿调蛋白染色,测定尿中尿调蛋白(uromodulin,UMOD)水平.结果 MCKD组发病年龄小,血尿酸高于CTIN组(P=0.011),UMOD呈团块状分布在小管上皮细胞内,与正常的均质状分布不同.尿UMOD低于对照(P=0.047).结论 MCKD诊断应结合临床病理及尿调蛋白的染色及尿中水平的测定.

  13. Role of the Transient Receptor Potential Canonical 6 ion channel in genetic and acquired forms of proteinuric kidney disease

    Möller, Clemens Claudius

    2007-01-01

    Podocyte foot processes and the interposed glomerular slit diaphragm are critical components of the permeability barrier in the kidney. Mutations in several podocyte genes have been identified as the cause for progressive kidney failure and focal segmental glomerulosclerosis (FSGS). Podocyte injury is a hallmark of glomerular disease and usually involves the rearrangement of the podocyte actin cytoskeleton. Cell-specific therapies targeting podocyte injury are currently not available. In 2004...

  14. Doença cística medular do rim: relato de um caso diagnosticado por ultra-sonografia e tomografia computadorizada Medullary cystic disease of the kidney: report of a case diagnosed by ultrasonography and computed tomography examinations

    Tarcísio Nunes Carvalho

    2003-12-01

    Full Text Available Doença cística medular, nefronofitíase juvenil familiar ou complexo de doença cística medular referem-se a um grupo de doenças similares cuja lesão básica é uma progressiva atrofia tubular renal com esclerose glomerular e formação cística medular. É importante causa de insuficiência renal em pacientes adolescentes. Estudos de imagem têm papel principal no diagnóstico desta enfermidade, com cistos caracteristicamente individualizados na medula renal e junção córtico-medular e tamanho renal normal ou reduzido. Neste artigo demonstramos os achados de imagem da doença cística medular renal em uma adolescente com quadro clínico característico, por meio de ultra-sonografia e tomografia computadorizada.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 corticomedullary 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.

  15. Renal cancer in kidney transplanted patients.

    Frascà, Giovanni M; Sandrini, Silvio; Cosmai, Laura; Porta, Camillo; Asch, William; Santoni, Matteo; Salviani, Chiara; D'Errico, Antonia; Malvi, Deborah; Balestra, Emilio; Gallieni, Maurizio

    2015-12-01

    Renal cancer occurs more frequently in renal transplanted patients than in the general population, affecting native kidneys in 90% of cases and the graft in 10 %. In addition to general risk factors, malignancy susceptibility may be influenced by immunosuppressive therapy, the use of calcineurin inhibitors (CNI) as compared with mammalian target of rapamycin inhibitors, and the length of dialysis treatment. Acquired cystic kidney disease may increase the risk for renal cancer after transplantation, while autosomal dominant polycystic kidney disease does not seem to predispose to cancer development. Annual ultrasound evaluation seems appropriate in patients with congenital or acquired cystic disease or even a single cyst in native kidneys, and every 2 years in patients older than 60 years if they were on dialysis for more than 5 years before transplantation. Immunosuppression should be lowered in patients who develop renal cancer, by reduction or withdrawal of CNI. Although more evidence is still needed, it seems reasonable to shift patients from CNI to everolimus or sirolimus if not already treated with one of these drugs, with due caution in subjects with chronic allograft nephropathy. PMID:26202137

  16. [Ultrasonographic study on kidneys in patients with chronic renal failure. Part I. Ultrasonic measurement of renal size and analysis of renal ultrasonotomograms].

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

    1990-08-01

    Ultrasonograms of 546 kidneys were obtained in 280 patients undergoing chronic dialysis. Dialysed kidneys could be detected in 529 of the 546 kidneys (96.9%) by ultrasonic examination. The ultrasonic diagnoses on dialysed kidneys were contracted kidney in 313 kidneys (59.2%) and acquired cystic disease of the kidney in 107 kidneys (20.2%). Ultrasonic measurement of the size of kidney (length and thickness) revealed that the kidneys in patients with chronic renal failure were much smaller than normal ones. But the kidneys in patients undergoing dialysis for more than 8 years gradually increased in size with incidence of acquired renal cysts. The kidneys in patients with diabetic nephropathy were greater in length and thickness than those with chronic glomerulonephritis. Sonographic features of dialysed kidneys were unclear renal imaging, unidentified central echoes, cortico-medulla + border and increased parenchymal echogenicity. Irregularity of the renal contour had a tendency to increase in number with incidence of cysts in long-term dialysis patients. The ultrasonograms of the kidneys with diabetic nephropathy showed fewer changes than normal ones. No major complication of the kidney was detected in the present study. However, two retroperitoneal hematomas and one renal cell carcinoma developed within two years after this examination. We believe that regular screening of the kidneys by ultrasonic examination is mandatory in patients on chronic dialysis for early diagnosis and treatment of these complications. PMID:2232408

  17. CT of the kidney in chronic renal failure

    The transverse size of the kidneys was measured by CT, and CT findings of the kidneys were studied in 94 patients with chronic renal failure under hemodialysis (HD), 58 patients with chronic renal failure not under hemodialysis (CRF) and 100 controls. The transverse size of the kidneys decreased according to the deterioration of renal function. The ratio of the maximal renal transverse size to the minimal vertebral size, which the author proposed as a new criterion for renal atrophy, was 1.8 in controls, 1.2 in CRF and 0.8 in HD. A kidney smaller than the vertebral body indicated chronic renal failure. Characteristic CT features in CRF were mild renal atrophy and cystic changes (41.4 %). In HD, renal atrophy was more advanced, the occurrence of cystic changes was more frequent (64.9 %), and there were frequent renal (68.1 %) and aortic calcifications. Furthermore acquired cystic disease of the kidney (ACD) was observed (27.7 %) only in HD. In this study no renal neoplasm was found in ACD. However, several complications in HD, one perirenal hematoma and six hydronephroses, were observed. (author)

  18. CT of the kidney in chronic renal failure

    Kojima, Kanji

    1988-10-01

    The transverse size of the kidneys was measured by CT, and CT findings of the kidneys were studied in 94 patients with chronic renal failure under hemodialysis (HD), 58 patients with chronic renal failure not under hemodialysis (CRF) and 100 controls. The transverse size of the kidneys decreased according to the deterioration of renal function. The ratio of the maximal renal transverse size to the minimal vertebral size, which the author proposed as a new criterion for renal atrophy, was 1.8 in controls, 1.2 in CRF and 0.8 in HD. A kidney smaller than the vertebral body indicated chronic renal failure. Characteristic CT features in CRF were mild renal atrophy and cystic changes (41.4 %). In HD, renal atrophy was more advanced, the occurrence of cystic changes was more frequent (64.9 %), and there were frequent renal (68.1 %) and aortic calcifications. Furthermore acquired cystic disease of the kidney (ACD) was observed (27.7 %) only in HD. In this study no renal neoplasm was found in ACD. However, several complications in HD, one perirenal hematoma and six hydronephroses, were observed.

  19. Mature Cystic Renal Teratoma

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

    2014-01-01

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

  20. Clinicopathological manifestations of nephronophthisis-medullary cystic kidney disease%肾单位肾痨-髓质囊肿病的临床病理

    王素霞; 章友康; 周福德; 王仁贵; 屈海云; 邹万忠

    2008-01-01

    Objective To report the clinicopathological features of 2 cases of nephronophthisis-medullary cystic kidney disease (NPH-MCKD). Methods The clinical data and pathological changes of renal biopsy in two patients of NPH-MCKD from our hospital were analyzed, and associated literatures were reviewed simultanously. The clinicopathological featuresand diagnosis of NPH-MCKD were discussed. Results Two adolescent patients were admitted to our hospital for indolent renal insufficiency, polyuria accompanied by polydipsia as first signs.Urine analysis showed low specific density urine, mild proteinuria, and few formed elements in urinary sediments. The ability of urine concentration and acidification was decreased. Familial history of renal disease and extra-renal lesions were not found. Renal ultrasound presented an increased echogenicity with diminished cortico-meduUary differentiation, and multiple small cysts in renal corticomedullary border were identified in one case by computed tomography. Pathological examination of renal biopsy revealed diffuse tubular interstitial lesion which was characterized by the triad of tubular basement disintegration, tubular atrophy with cyst development, and interstitial fibrosis. Some of glomerular sclerosis occurred. Cyst development at the corticomedullary border of the kidneys was the specific feature of NPH-MCKD. Conclusions Young patients with impaired tubular function should be suspected of NPH-MCKD. Renal ultrasound or computed tomography can provide an important clue. Multiple renal cysts at the corticomedullary border identified by renal biopsy can be a diagnostic indication for NPH-MCKD.%目的 报道两例.肾单位肾痨-髓质囊肿病(NPH-MCKD)的临床病理特点.方法 分析本院诊断的两例NPH-MCKD的临床资料及肾活检组织的病理改变,并结合文献复习,探讨NPH-MCKD的临床病理特点及其诊断方法.结果 两例均为青年患者,首发症状为烦渴、多尿,低相对密度尿,轻度蛋白

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

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

  2. Is the serum vitamin D level at the time of hospital-acquired acute kidney injury diagnosis associated with prognosis?

    Lingyun Lai

    Full Text Available BACKGROUND: Low circulating vitamin D levels have been suggested to potentially contribute to acute complications in critically ill patients. However, in patients with acute kidney injury (AKI, whether vitamin D deficiency occurs and is a potential contributor to worse early outcomes at the time of AKI diagnosis remains unclear. METHODOLOGY/PRINCIPAL FINDINGS: Two hundred patients with AKI were enrolled in our study. Healthy subjects and critically ill patients without AKI served as controls. Serum vitamin D concentrations were measured in the three groups. The patients with AKI were followed up for 90 days and grouped according to median serum vitamin D concentrations. In addition, vitamin D receptor polymorphisms (BsmI and FokI were measured in these patients; they were also followed up for 90 days and grouped according to vitamin D receptor gene mutations. Low serum 1,25-dihydroxyvitamin D levels (59.56±53.00 pmol/L were detected in patients with AKI and decreased with increasing severity of AKI. There were no significant findings with respect to 25-hydroxyvitamin D. The 90-day survival curves of individuals with high vitamin D concentrations showed no significant differences compared with the curves of individuals with low concentrations. The survival curves of patients with BB/Bb or FF/Ff genotypes also showed no significant differences compared with patients with bb or ff genotypes. In Cox regression analysis, the vitamin D status in patients with AKI was not an independent prognostic factor as adjusted by age, sex, Sequential Organ Failure Assessment score, or vitamin D receptor polymorphisms. CONCLUSIONS/SIGNIFICANCE: Patients with AKI manifested a marked decrease in the 1,25-dihydroxyvitamin D level at the time of AKI diagnosis, and the degree of 1,25-dihydroxyvitamin D deficiency increased with the severity of AKI. No association between the serum vitamin D level at the time of AKI diagnosis and 90-day all-cause mortality was found in

  3. Cystic fibrosis*

    1985-01-01

    Cystic fibrosis represents a major health problem in developed countries. Although its distribution is worldwide, in most developing countries there is little awareness of its prevalence and little opportunity to provide treatment. The improvements in survival of cystic fibrosis patients in developed countries over the past two decades have brought mean life expectancy to 20 years or more, and many adult patients with cystic fibrosis at present are fully integrated in society and have an acce...

  4. Cystic diseases of the biliary tract and liver

    Nafiye Urgancı

    2008-01-01

    Cystic diseases of liver are recognized in infancy and childhood initially. Cystic diseases of liver and biliary tract are choledocal cysts, autosomal recessive and autosomal dominant polycystic kidney disease, congenital hepatic fibrosis and Caroli disease (cystic dilatation of intrahepatic bile ducts). Choledochal cysts and Caroli disease do not allow biliary flow, cause chronic or obstructive cholestasis and progressive liver disease. In congenital hepatic fibrosis and polycystic kidney di...

  5. Cystic diseases of the biliary tract and liver Invited Editor

    Urgancı, Nafiye

    2008-01-01

    Cystic diseases of liver are recognized in infancy and childhood initially Cystic diseases of liver and biliary tract are choledocal cysts autosomal recessive and autosomal dominant polycystic kidney disease congenital hepatic fibrosis and Caroli disease cystic dilatation of intrahepatic bile ducts Choledochal cysts and Caroli disease do not allow biliary flow cause chronic or obstructive cholestasis and progressive liver disease In congenital hepatic fibrosis and polycystic kidney disease th...

  6. Acquired cystic disease-associated renal cell carcinoma with a focal sarcomatoid component: Report of a case showing more pronounced polysomy of chromosomes 3 and 16 in the sarcomatoid component.

    Tajima, Shogo; Waki, Michihiko; Doi, Wataru; Hayashi, Kazumasa; Takenaka, Syunsuke; Fukaya, Yoshie; Kimura, Ryosuke

    2015-02-01

    Acquired cystic disease (ACD)-associated renal cell carcinoma (RCC) has recently been established. Herein we report the sixth case of ACD-associated RCC with a sarcomatoid change. The patient was a 77-year-old man who regularly underwent hemodialysis for 14 years due to chronic renal failure resulting from IgA nephropathy. On computed tomography, a large right RCC was observed with contrast enhancement in the arterial phase. A nodular protrusion into the perirenal fat was detected. Right nephrectomy was performed under laparoscopy. Surgically resected specimens revealed a tan-to-yellow tumor (95 × 75 × 55 mm) with a whitish nodule (20 × 15 × 15 mm) invading into the perirenal fat. Histopathologically, the large carcinoma component of the tumor displayed a cribriform or microcystic growth pattern with deposition of oxalate crystals. The whitish nodule corresponded to the sarcomatoid component, and the spindled and pleomorphic tumor cells showed diffuse positivity of p53 on immunohistochemistry. Fluorescence in situ hybridization revealed trisomy of chromosomes 3 and 16 in the carcinoma component, as was expected from the literature. In addition, increased polysomy of these chromosomes was also observed in the sarcomatoid component. This finding may be related to the development of the sarcomatoid component along with the TP53 mutation. PMID:25424516

  7. Renal (Kidney) Manifestations in TSC

    Full Text Available ... When the tumor suppressor genes are inactivated by mutations, cell growth is unchecked, leading to tumors. Cysts ... with TSC and severe cystic kidneys can have mutations (changes in the DNA) of both the TSC2 ...

  8. Renal (Kidney) Manifestations in TSC

    Full Text Available ... must be kept in mind. Diagnosis The current methods to diagnosis these renal abnormalities include renal ultrasonography, ... cells, which surround a fluid-filled cavity. Some children and adults with TSC and severe cystic kidneys ...

  9. Renal (Kidney) Manifestations in TSC

    Full Text Available ... cells, which surround a fluid-filled cavity. Some children and adults with TSC and severe cystic kidneys ... vomiting. However, bleeding or rupture rarely occurred in children; larger tumors occurred at an older age (greater ...

  10. Cystic Fibrosis

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

  11. Genetics Home Reference: uromodulin-associated kidney disease

    ... and How They Work Educational Resources (4 links) Disease InfoSearch: Medullary cystic kidney disease 2 Merck Manual Home Edition: ... Registry (3 links) Familial juvenile gout Glomerulocystic kidney disease with hyperuricemia and ... cystic kidney disease 2 Scientific articles on PubMed ( ...

  12. Cystic diseases of the biliary tract and liver

    Nafiye Urgancı

    2008-06-01

    Full Text Available Cystic diseases of liver are recognized in infancy and childhood initially. Cystic diseases of liver and biliary tract are choledocal cysts, autosomal recessive and autosomal dominant polycystic kidney disease, congenital hepatic fibrosis and Caroli disease (cystic dilatation of intrahepatic bile ducts. Choledochal cysts and Caroli disease do not allow biliary flow, cause chronic or obstructive cholestasis and progressive liver disease. In congenital hepatic fibrosis and polycystic kidney disease there is cystic formations at terminal interlobular bile ducts, but cholestasis is not seen. They don’t cause liver and biliary tract functional disturbances. (Turk Arch Ped 2008; 43: 40-5

  13. Cilium, centrosome and cell cycle regulation in polycystic kidney disease.

    Lee, Kyung; Battini, Lorenzo; Gusella, G Luca

    2011-10-01

    Polycystic kidney disease is the defining condition of a group of common life-threatening genetic disorders characterized by the bilateral formation and progressive expansion of renal cysts that lead to end stage kidney disease. Although a large body of information has been acquired in the past years about the cellular functions that characterize the cystic cells, the mechanisms triggering the cystogenic conversion are just starting to emerge. Recent findings link defects in ciliary functions, planar cell polarity pathway, and centrosome integrity in early cystic development. Many of the signals dysregulated during cystogenesis may converge on the centrosome for its central function as a structural support for cilia formation and a coordinator of protein trafficking, polarity, and cell division. Here, we will discuss the contribution of proliferation, cilium and planar cell polarity to the cystic signal and will analyze in particular the possible role that the basal bodies/centrosome may play in the cystogenetic mechanisms. This article is part of a Special Issue entitled: Polycystic Kidney Disease. PMID:21376807

  14. Cystic Lymphangioma of Pancreas.

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

    2016-03-01

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

  15. Giant cystic abdominal masses in children

    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.)

  16. Renal (Kidney) Manifestations in TSC

    Full Text Available ... cells, which surround a fluid-filled cavity. Some children and adults with TSC and severe cystic kidneys can have mutations (changes in the DNA) of both the TSC2 gene on chromosome 16 and the gene for polycystic kidney disease (PKD1), which lies right next to the TSC2 gene. Mutations in the ...

  17. Tubulocystic carcinoma of the kidney

    Podduturi, Varsha; Adair, Carol F.; Zhang, Haiying

    2015-01-01

    Tubulocystic carcinoma (TCC) of the kidney is a unique, rare, and recently recognized neoplasm. Although originally considered a low-grade collecting duct carcinoma, TCC is now considered to be a distinct entity. TCC should be considered in the differential diagnosis of cystic renal neoplasms. We report a case of TCC arising in the left kidney.

  18. [Ultrasound and color Doppler applications in chronic kidney disease].

    Meola, Mario; Petrucci, Ilaria

    2012-01-01

    Chronic kidney disease (CKD) encompasses all clinical features and complications during the progression of various kidney conditions towards end-stage renal disease. These conditions include immune and inflammatory diseases such as primary and HCV-related glomerulonephritis; infectious diseases such as pyelonephritis with or without reflux and tuberculosis; vascular diseases such as chronic ischemic nephropathy; hereditary and congenital diseases such as polycystic disease and congenital cystic dysplasia; metabolic diseases including diabetes and hyperuricemia; and systemic diseases (collagen disease, vasculitis, myeloma). During the progression of CKD, ultrasound imaging can differentiate the nature of the renal damage in only 50-70% of cases. Infact, the end-stage kidney appears shrunken, reduced in volume (Ø acquired cystic degeneration (small and multiple cysts involving the cortex and medulla) or nephrocalcinosis, but there are rare exceptions, such as polycystic kidney disease, diabetic nephropathy, and secondary inflammatory nephropathies. The main difficulties in the differential diagnosis are encountered in multifactorial CKD, which is commonly presented to the nephrologist at stage 4-5, when the kidney is shrunken, unstructured and amorphous. As in acute renal injury and despite the lack of sensitivity, ultrasonography is essential for assessing the progression of the renal damage and related complications, and for evaluating all conditions that increase the risk of CKD, such as lithiasis, recurrent urinary tract infections, vesicoureteral reflux, polycystic kidney disease and obstructive nephropathy. The timing and frequency of ultrasound scans in CKD patients should be evaluated case by case. In this review we will consider the morphofunctional features of the kidney in all nephropathies that may lead to progressive CKD. PMID:23229668

  19. Multilocular cystic renal tumor in children: radiologic-pathologic correlation.

    Agrons, G A; Wagner, B J; Davidson, A J; Suarez, E S

    1995-05-01

    Multilocular cystic renal tumor is a term that encompasses two histologically distinct but grossly indistinguishable lesions: cystic nephroma and cystic partially differentiated nephroblastoma (CPDN). Cystic nephroma is a segmental, purely cystic mass characterized by multiple septations composed entirely of differentiated tissues, without blastemal elements. CPDN is also a multiloculated lesion without nodular solid components, but its septa contain embryonal cells. Multilocular cystic tumors primarily affect boys during early childhood, with a substantial number of the lesions containing blastema (CPDN), and adult women, with lesions that more commonly lack septal blastema (cystic) nephroma). As a rule, nephrectomy is curative and the clinical course benign, but CPDN may recur locally. Although cystic nephroma and CPDN cannot be distinguished radiologically, failure to do so has no practical impact on management, since all of these tumors are surgically removed. However, the differential diagnosis includes other pediatric cystic renal masses that may require different treatment stratagems: Wilms tumor with cyst formation due to hemorrhage and necrosis, cystic clear cell sarcoma, cystic mesoblastic nephroma, cystic renal cell carcinoma, multicystic dysplastic kidney, and segmental multicystic dysplasia in a duplicated renal collecting system. PMID:7624570

  20. Clinical Scenarios in Chronic Kidney Disease: Kidneys' Structural Changes in End-Stage Renal Disease.

    Meola, Mario; Samoni, Sara; Petrucci, Ilaria

    2016-01-01

    Acquired cystic kidney disease (ACKD) and renal cell carcinoma (RCC) are the most important manifestations of end-stage kidneys' structural changes. ACKD is caused by kidney damage or scarring and it is characterized by the presence of small, multiple cortical and medullary cysts filled with a fluid similar to preurine. ACKD prevalence varies according to predialysis and dialysis age and its pathogenesis is unknown, although it is stated that progressive destruction of renal tissue induces hypertrophy/compensatory hyperplasia of residual nephrons and may trigger the degenerative process. ACKD is almost asymptomatic, but it can lead to several complications (bleeding, rupture, infections, RCC). Ultrasound (US) is the first level imaging technique in ACKD, because of its sensitivity and reliability. The most serious complication of ACKD is RCC, which is stimulated by the same growth factors and proto-oncogenes that lead to the genesis of cysts. Two different histological types of RCC have been identified: (1) RCC associated with ACKD and (2) papillary renal clear cell carcinoma. Tumors in end-stage kidneys are mainly small, multifocal and bilateral, with a papillary structure and a low degree of malignancy. At US, RCC appears as a small inhomogeneous nodule (<3 cm), clearly outlined from the renal profile and hypoechoic if compared with sclerotic parenchyma. In some cases, tumor appears as a homogeneous and hyperechoic multifocal mass. The most specific US sign of a small tumor in end-stage kidney is the important arterial vascularization, in contrast with renal parenchymal vascular sclerosis. PMID:27169876

  1. What Causes Cystic Fibrosis?

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

  2. Cystic Fibrosis Research

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

  3. Cystic Fibrosis and Pregnancy

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

  4. Cystic fibrosis

    2004-01-01

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

  5. Kidney Failure

    ... Health Information > Health Communication Programs > National Kidney Disease Education Program > Learn About Kidney Disease > Living With Kidney Disease > Kidney Failure | Share External Link Disclaimer Living With Kidney Disease ...

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

    Kenji Mimatsu

    2011-10-01

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

  7. IgG4-related tubulointerstitial nephritis accompanied with cystic formation

    Fukuhara, Hideo; Taniguchi, Yoshinori; Matsumoto, Manabu; Kuroda, Naoto; Fukata, Satoshi; Inoue, Keiji; Fujimoto, Shimpei; Terada, Yoshio; Shuin, Taro

    2014-01-01

    Background An immunoglobulin G4 (IgG4)-related disease is important disease in differential diagnosis of tumors in kidney, pancreas, lung and other organs. The imaging findings of IgG4-related kidney diseases are usually expressed as defect contrast region, while cystic formation in kidney is extremely rare. Here, we report a case of IgG4-related tubulointerstitial nephritis with renal cystic change caused by the narrowing or obstruction of collecting duct in renal medulla. Case presentation ...

  8. Computerized tomography of kidneys in long-term dialysis patients

    A cohort is presented of 21 patients with irreversible kidney failure who were examined by computerized tomography in 1986-1987. The mean period of dialysis programme was 5.7 years, chronic glomerulonephritis and interstitial nephritis were the basic conditions. According to the results of computerized tomography, the patients were divided into four groups: 1. the presence of cysts or tumors was not established, 2. multiplecysts were found in bilateral location, 3. less than 3 cysts were found, 4. kidney tumor was diagnosed. The factor causing the development of the cysts was not identified. It is presumed that the disease is caused by a biologically active substance which is not efficiently dialyzed. The examination of the kidneys is recommended in patients in the stage of chronic kidney insufficiency and particularly in patients on dialysis for a long period of time. The recommended examination uses computerized tomography, and attention should be given to the possible occurence of acquired cystic disease or kidney tumors. (author). 6 figs., 8 refs

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

    Mimatsu, Kenji; Oida, Takatsugu; Kawasaki, Atsushi; Kano, Hisao; Kida, Kazutoshi; Fukino, Nobutada; Kuboi, Youichi; Amano, Sadao

    2011-01-01

    We report a case of multiple gastric carcinomas associated with Potter type III cystic disease of the liver, mesenterium and kidney. A 65-year-old man with chronic renal failure due to polycystic kidneys and under hemodialysis treatment 3 times a week for 2 years was admitted to our hospital because of anemia. He stated that his sister had suffered from polycystic kidney disease. Gastrointestinal fiberscopy showed two lesions in the lesser curvature in the lower portion of the stomach, and hi...

  10. Fetal Kidney Anomalies: Next Generation Sequencing

    Rasmussen, Maria; Sunde, Lone; Nielsen, Marlene Louise;

    undergone postmortem examination. The approximately 110 genes included in the targeted panel were chosen on the basis of their potential involvement in embryonic kidney development, cystic kidney disease, or the renin-angiotensin system. DNA was extracted from fetal tissue samples or cultured chorion villus...... the nephronophthisis associated gene, TMEM67 and six fetuses had mutations in kidney developmental genes. For these fetuses kidney histology is presented. Conclusion and Perspectives In eight (14%) fetuses we identified a likely genetic cause of the kidney anomalies. Ten fetuses from eight families......, in which no mutations were identified, have been selected for exome sequencing in order to uncover novel genes associated to fetal kidney anomalies....

  11. Cystic fibrosis - nutritional considerations

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

  12. Cystic fibrosis - nutritional considerations

    Cystic fibrosis (CF) is a life-threatening disease that causes thick, sticky mucus to build up in the lungs and digestive tract. Persons with cystic fibrosis need to eat high-calorie and high-protein ...

  13. Cystic acoustic neuromas

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

    2002-01-01

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

  14. Cystic fibrosis: case report

    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

  15. Cystic fibrosis: case report

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

    2002-12-01

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

  16. Risk of cancer in retransplants compared to primary kidney transplants in the United States.

    Kalil, Roberto S; Lynch, Charles F; Engels, Eric A

    2015-10-01

    Recipients of kidney transplantation have elevated risk of developing cancer. There are limited data on cancer risk in recipients of kidney retransplantation. We used data from the Transplant Cancer Match Study, which links the U.S. transplant registry with 15 cancer registries. Cancer incidence in recipients of kidney retransplantation and primary kidney transplants was compared utilizing Poisson regression, adjusting for demographic and medical characteristics. We assessed 109 224 primary recipients and 6621 retransplants. Compared to primary recipients, retransplants were younger (median age 40 vs. 46 yr), had higher PRA, and more often received induction with polyclonal antibodies (43% vs. 25%). A total of 5757 cancers were observed in primary recipients and 245 in retransplants. Overall cancer risk was similar in retransplants compared with primary recipients (incidence rate ratio [IRR] 1.06, 95% CI 0.93-1.20, adjusted for age, gender, race/ethnicity, PRA, and use of polyclonal induction). However, renal cell carcinoma (RCC) occurred in excess among retransplants (adjusted IRR 2.03, 95% CI 1.45-2.77), based on 514 cases in primary recipients and 43 cases in retransplants. Overall cancer risk did not differ in retransplants compared to primary recipients. Increased risk of RCC may be explained by the presence of acquired cystic kidney disease, which is more likely to develop with additional time with kidney disease and time spent on dialysis waiting for retransplantation. PMID:26255999

  17. Tuberous sclerosis with cystic renal disease and multifocal renal cell carcinoma in a baby girl

    An infant, in whom the prenatal diagnosis of tuberous sclerosis complex was made, presented with extreme bilateral nephromegaly owing to diffuse cystic changes. Histology of the resected non-functioning left kidney revealed, in addition to the characteristic cysts, two foci of renal cell carcinoma not visible on US or MRI. This infant is exceptional given the extensive cystic transformation of both kidneys and the presence of malignant lesions at this young age. (orig.)

  18. Tuberous sclerosis with cystic renal disease and multifocal renal cell carcinoma in a baby girl

    Breysem, Luc; Nijs, Els; Smet, Maria-Helena [Department of Radiology, University Hospitals, Herestraat 49, 3000 Leuven (Belgium); Proesmans, Willem [Department of Paediatrics, University Hospitals, Leuven (Belgium)

    2002-09-01

    An infant, in whom the prenatal diagnosis of tuberous sclerosis complex was made, presented with extreme bilateral nephromegaly owing to diffuse cystic changes. Histology of the resected non-functioning left kidney revealed, in addition to the characteristic cysts, two foci of renal cell carcinoma not visible on US or MRI. This infant is exceptional given the extensive cystic transformation of both kidneys and the presence of malignant lesions at this young age. (orig.)

  19. Caroli′s syndrome with autosomal recessive polycystic kidney disease

    Prithi Shenoy; Syed Ahmed Zaki; Preeti Shanbag; Swapnil Bhongade

    2014-01-01

    Caroli′s syndrome (CS) is a rare congenital disorder characterized by multiple segmental cystic or saccular dilatations of the intrahepatic bile ducts and congenital hepatic fibrosis. We report a 9-year-old boy who was diagnosed with CS and autosomal recessive poly-cystic kidney disease. On screening, his 5-month-old asymptomatic sister had multiple dilated biliary radicals with multiple bilateral renal cystic lesions. Both the patient and the affected sibling have been advised regular follow...

  20. How Is Cystic Fibrosis Diagnosed?

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

  1. Genetics Home Reference: cystic fibrosis

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

  2. How Is Cystic Fibrosis Treated?

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

  3. CASE REPORT: Papillary Adenoma of Kidney- An Incidental Autopsy Finding: Report of Two Cases

    Bhakti D. Deshmukh

    2012-01-01

    Full Text Available Background: Renal papillary adenoma is usually an incidental finding at autopsy with an incidence of 7% to 23%. The frequency of small papillary tumours of kidney increases with age to approximately 40% of the population over the age of 65. These tumours occur morefrequently in scarred kidneys, acquired renal cystic disease and in children with von Hippel-Lindau syndrome. Case history: In this report we describe renal papillary adenoma incidentally detected during autopsies of two elderly males. Gross examination of kidneyshowed two tiny subcapsular yellowish nodules in one case and single nodule with similar morphology in the other. Microscopic examination in both the cases showed a well circumscribed tumour composed of densely packed tubules and papillae lined by small cuboidal to columnar cells with rounded uniform nuclei. However there was no nuclear atypia, mitosis or necrosis.

  4. Acquired Techniques

    Lunde Nielsen, Espen; Halse, Karianne

    2013-01-01

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

  5. Acquired blepharoptosis

    Oosterhuis, HJGH

    1996-01-01

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

  6. Pathologic spectrum of cysts in end-stage kidneys: possible precursors to renal neoplasia.

    Hosseini, Mojgan; Antic, Tatjana; Paner, Gladell P; Chang, Anthony

    2014-07-01

    Acquired cystic disease (ACD) is common in patients with end-stage renal disease. Given the significant increased risk of renal cell carcinoma (RCC) in these patients, we characterized the pathologic spectrum of cysts in end-stage kidneys to determine the possible relationship with coincidental neoplasms. Twenty-one native end-stage kidneys contained multiple cysts (0.1-4 cm), which could be categorized into 3 groups based on the cytoplasm of the predominant cell type: clear, eosinophilic, or foamy. Clear cell cysts showed strong staining with carbonic anhydrase IX (CA9) in a cup-shaped manner. Of 7 kidneys with CA9-positive clear cell cysts, 3 had at least 2 foci of RCC (0.5-8 cm), which all demonstrated the morphologic features and immunoprofile of clear cell papillary RCC. Eight kidneys contained foamy cysts, and 4 of these contained ACD-associated RCC, but 1 papillary RCC was also encountered. Six kidneys had eosinophilic cysts, which were negative for CA9, and 3 of these were associated with papillary RCC. Clear cell cysts, although few in number, are common in end-stage nephrectomy specimens. These cysts were present in all kidneys with clear cell papillary RCC and a few kidneys without an obvious mass. In specimens with ACD-associated RCC or papillary RCC, cysts lined by epithelial cells with predominantly eosinophilic or foamy cytoplasm were identified. These data support the idea that the cysts in end-stage kidneys could represent the earliest precursor lesion of renal neoplasia. PMID:24775605

  7. Renal replacement therapy for rare diseases affecting the kidney

    Wühl, Elke; van Stralen, Karlijn J; Wanner, Christoph;

    2014-01-01

    . RESULTS: From 1 January 2007 to 31 December 2011, 7194 patients started RRT for a rare disease (10.6% children). While some diseases were exclusively found in adults (e.g. Fabry disease), primary oxalosis, cystinosis, congenital anomalies of the kidney and urinary tract (CAKUT) and medullary cystic kidney...

  8. Living with Cystic Fibrosis

    ... Most of these centers have pediatric and adult programs or clinics. For more information about CF Care Centers, go to the Cystic Fibrosis Foundation's Care Center Network Web page. It's standard to have CF checkups every 3 ...

  9. Congenital Cystic Lung Diseases

    Aditi Jain

    2013-01-01

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

  10. Cystic parotid gland lesion evaluation

    We evaluated differential diagnoses of cystic parotid gland lesions and the efficacy of preoperative diagnosis. Of 191 parotid gland nodules resected between January 2003 and October 2008, 167 (87%) were benign and 24 (13%) malignant. Thirty-five parotid gland nodules whose components were almost cystic were enrolled in this study. All cystic lesions were retrospectively evaluated with respect to preoperative diagnostic examinations and histopathological confirmed diagnosis. Cystic components in surgical specimens were also evaluated histopathologically. The relationships with magnetic resonance imaging (MRI) findings and histopathological confirmed diagnosis were studied. Of 35 cystic lesions, 11 were complete cystic masses and had no mural nodules, while remaining 24 were incomplete and had mural nodules. Histopathological examinations showed that 5 were nonneoplastic, 27 were cystic degenerations of benign tumors, and 3 were cystic degenerations of malignant tumors. In the 11 complete cysts, preoperative diagnosis could not be made using any modality, whether with fine needle aspiration cytology (FNAC), salivary scintigraphy, or 67-gallium citrate scintigraphy. Of 24 incomplete cysts, only 9 cystic Warthin tumors were diagnosed correctly before surgery. Studies of cystic components in MRI and histopathology suggested that hemorrhagic degeneration of malignant tumors should be kept in mind for cystic lesions showing hemorrhagic portions. Cystic parotid gland lesions are difficult to diagnose correctly before surgery, but the evaluation of cystic components by MRI and FNAC is helpful in differentiating between benign and malignant tumors. (author)

  11. Serum nutritional markers are predictors of early mortality in hospital-acquired acute kidney injury%血清营养学指标预测住院急性肾损伤患者早期死亡

    马帅; 顾勇; 林善锬; 丁峰; 谢琼虹; 游怀舟; 周莹; 钱璟; 匡鼎伟; 刘骏峰; 何奇柳; 郝传明

    2012-01-01

    目的 评价血清营养学指标对住院期间发生急性肾损伤( AKI)的患者的预后,特别是早期死亡和晚期死亡的预测价值.方法 采用前瞻性队列研究.入选华山医院住院期间发生AKI的成人患者194例.AKI诊断标准为RIFLE分期中的Scr标准,除外肾后梗阻、原发性肾小球肾炎、间质性肾炎及血管炎引起的AKI.收集患者临床资料及实验室检测指标,用人体测量、血清营养指标及主观全面评价法(SGA)来评估患者的营养状况.根据存活时间是否超过28 d,将入选患者分为存活组129例(存活>28 d)与死亡组59例(存活≤28 d).进一步将死亡组分为早期死亡组(存活≤7d)和晚期死亡组(存活8~28 d)两个亚组.观察各项营养指标对AKI患者预后的预测价值.结果 人体测量、血清营养指标及SGA结果显示,高比例的AKI患者存在营养不良.单因素分析显示,SGA、血清前白蛋白及胆固醇、外周血总淋巴细胞计数(TLC)、Maastricht指数(MI)在早期死亡组、晚期死亡组和存活组间差异有统计学意义.早期死亡组前白蛋白及胆固醇显著低于存活组和晚期死亡组(P<0.05).多因素分析显示,在校正了年龄、性别、透析、机械通气、血红蛋白、血小板、血清胆红素和Glasgow昏迷评分(GCS)后,SGA及血清白蛋白、前白蛋白、胆固醇仍与早期死亡相关.以白蛋白、前白蛋白、胆固醇预测早期死亡的受试者工作特征( ROC)曲线下面积(AUC)分别为0.591、0.736和0.603,前白蛋白的AUC显著大于白蛋白及胆固醇(均P<0.05).结论 低水平前白蛋白、白蛋白和胆固醇可独立预测住院期间发生AKI患者的早期死亡.%Objective To evaluate the role of nutritional parameters in prognosis,especially in the early and late mortality of hospital-acquired acute kidney injury (AKI) patients.Methods This study was a prospective cohort study conducted in a hospital comprising 1500 beds in Shanghai

  12. Cystic Fibrosis: Symptoms, Diagnosis, Treatment

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

  13. Intestinal disease in cystic fibrosis.

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

    1988-01-01

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

  14. Multiple cystic lung disease

    Flavia Angélica Ferreira Francisco

    2015-12-01

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

  15. Multiple cystic lung disease.

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

    2015-12-01

    Multiple cystic lung disease represents a diverse group of uncommon disorders that can present a diagnostic challenge due to the increasing number of diseases associated with this presentation. High-resolution computed tomography of the chest helps to define the morphological aspects and distribution of lung cysts, as well as associated findings. The combination of appearance upon imaging and clinical features, together with extrapulmonary manifestations, when present, permits confident and accurate diagnosis of the majority of these diseases without recourse to open-lung biopsy. The main diseases in this group that are discussed in this review are lymphangioleiomyomatosis, pulmonary Langerhans cell histiocytosis and folliculin gene-associated syndrome (Birt-Hogg-Dubé); other rare causes of cystic lung disease, including cystic metastasis of sarcoma, are also discussed. Disease progression is unpredictable, and understanding of the complications of cystic lung disease and their appearance during evolution of the disease are essential for management. Correlation of disease evolution and clinical context with chest imaging findings provides important clues for defining the underlying nature of cystic lung disease, and guides diagnostic evaluation and management. PMID:26621970

  16. Sinonasal Manifestations in Cystic Fibrosis

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

    2012-01-01

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

  17. MRI of renal cell carcinoma in hemodialyzed kidneys

    We evaluated MRI findings of 7 cases of renal cell carcinomas developed in patients with long-term hemodialysis. There were 4 men and 3 women with their ages from 46 to 82 years (mean; 59). They had undergone hemodialysis from 4 years and 10 months, to 20 years (mean; 11 years and 10 months). On T1-weighted images the tumors showed a high intensity to renal parenchyma in 6 of 7 cases. On T2-weighted images they showed either an iso or high intensity except one case with a low intensity. On dynamic studies with Gd-DTPA 6 of 7 cases showed an early enhancement with a distinct delineation of the tumor. Acquired cystic disease of the kidneys was present in 6 of 7 cases. There was no side effect due to Gd-DTPA. We conclude that a dynamic MRI study with Gd-DTPA is useful for the diagnosis of renal cell carcinoma in hemodialyzed kidney. (J.P.N.)

  18. MRI of renal cell carcinoma in hemodialyzed kidneys

    Kawasaki, Yoshimichi; Nakamura, Katsumi; Egashira, Kanji; Nakata, Hajime; Kaizu, Kazo; Okamura, Tomohiko; Sugita, Atsuo [University of Occupational and Environmental Health, Kitakyushu, Fukuoka (Japan)

    1995-12-01

    We evaluated MRI findings of 7 cases of renal cell carcinomas developed in patients with long-term hemodialysis. There were 4 men and 3 women with their ages from 46 to 82 years (mean; 59). They had undergone hemodialysis from 4 years and 10 months, to 20 years (mean; 11 years and 10 months). On T1-weighted images the tumors showed a high intensity to renal parenchyma in 6 of 7 cases. On T2-weighted images they showed either an iso or high intensity except one case with a low intensity. On dynamic studies with Gd-DTPA 6 of 7 cases showed an early enhancement with a distinct delineation of the tumor. Acquired cystic disease of the kidneys was present in 6 of 7 cases. There was no side effect due to Gd-DTPA. We conclude that a dynamic MRI study with Gd-DTPA is useful for the diagnosis of renal cell carcinoma in hemodialyzed kidney. (J.P.N.).

  19. Kidney Disease

    ... build up in the body. Kidney Function and Aging Kidney function may be reduced with aging. As ... more sensitive to certain medicines. For example, nonsteroidal anti-inflammatory drugs (NSAIDs) and some antibiotics may harm ...

  20. Kidney Failure

    ... York Golf Classic The 11th Annual New York Golf Classic Each August, supporters in our Northeast Region hit the links in support of AKF. Kidney Action Day Kidney Action Day Learn about our signature outreach event. Free health screenings ...

  1. Kidney Disease

    ... and other mineral levels. Common Kidney Conditions in Teens Sometimes, the kidneys aren't able to do ... conditions is a renal ultrasound . Like the ultrasound pictures that pregnant women get of their fetuses, a ...

  2. Kidney School

    ... licensing agreement Kidneys: How They Work, How They Fail, What You Can Do For people at any ... Kidney School is a program of the Medical Education Institute, Inc. , a 501(c)(3) organization, © 2002– ...

  3. Kidney Problems

    ... Home & Community Home › Aging & Health A to Z › Kidney Problems Font size A A A Print Share Glossary Basic Facts & ... The kidneys also help maintain body fluids at normal levels. In addition, the kidneys play important roles in controlling blood pressure and ...

  4. Kidney Transplant

    ... You are here Home » Kidney Transplant Click to watch a video on this topic Click to watch a video on Hemodialysis When kidneys fail, there ... 05/2016 - 10:00am Philadelphia, PA Kidney Camp Sun, 07/17/2016 - 6:00pm Ingleside, IL Register ...

  5. Arthritis in cystic fibrosis.

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

    1984-01-01

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

  6. The multicystic dysplastic kidney in children.

    Chang, W T; Chen, H C; Peng, H C

    1993-05-01

    Multicystic dysplastic kidney (MCDK) is the most common renal cystic disease in infancy and the second most common neonatal abdominal mass. It represents an extreme form of renal dysplasia. The key features in MCDK include variability in size, loss of kidney shape, absence of function and obstruction of ureter. Current management is still controversial. The prognosis is largely depended upon the problems of associated anomalies and the condition of the contralateral kidney. From September '82 to September '91, twelve patients with multicystic kidney have been treated at Taichung Veterans General Hospital. Ten of them received elective nephrectomy. The postoperative condition was uneventful. However, there was a high frequency of associated anomalies (41.6%) and pathologic contralateral kidney (50%). Two patients who refused operation were put on close observation and follow-up with ultrasound. During the surgical patients' follow-up period, one suffered from repeated urinary tract infection and the other died of multiple congenital anomalies. PMID:8334562

  7. Incidentally Detected Primary Giant Renal Cystic Enchinococcosis in a Young Patient: An Underestimated Entity?

    Emre Urer; Ozer Ural Cakici; Abdullah Erdem Canda; Aylin Kilic Yazgan; Seda Marali; Erem Asil; Ali Fuat Atmaca

    2015-01-01

    Echinococcosis is a parasitic infestation caused by Echinococcus granulosus and is an endemic disease in many parts of world. The symptoms and signs depend on the location and size of the cyst. Renal cystic echinococcosis or hydatid cyst (HC) disease of the kidney is extremely rare and constitutes only 2-4% of all cases of hydatid disease (HD). We present a 39-year-old male patient who was referred to our outpatient clinic with cystic right kidney mass that was incidentally diagnosed during...

  8. Incidental finding of malignant renal cystic tumour diagnosed sonographically

    Stojanović Milan

    2008-01-01

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

  9. Analysis of Prognostic Risk Factors in Patients with Community Acquired Pneumonia Complicated with Acute Kidney Injury%社区获得性肺炎伴发急性肾损伤的预后危险因素分析

    李家瑞; 张红燕; 尚跃丰; 曹书华

    2014-01-01

    目的:探讨社区获得性肺炎(CAP)患者伴发急性肾损伤(AKI)的预后危险因素。方法456例CAP患者为无伴发AKI(N-AKI)组和伴发AKI组。AKI组又根据RIFLE的严重程度级别分为3个亚组:危险(Risk)、损伤(Injury)和衰竭(Failure)组。比较各组患者CAP的严重程度,各项临床指标和预后评估指标的差别;多因素分析采用Logistic回归模型,生存分析采用Kaplan-Meier法,分析影响CAP患者预后不良的危险因素及RIFLE标准在预后评估中作用。结果456例CAP患者中有30%(135例)伴发AKI,诊断为Risk 61例(45.2%),Injury 23例(17%), Failure 51例(37.8%)。CAP患者PSI评分为Ⅰ~Ⅲ级的患者(300例)中有23.3%(70例)伴发AKI,PSI分级≥IV的患者(156例)中有41.7%(65例)伴发AKI,差异有统计学意义(P75岁、合并肾外器官衰竭是住院CAP患者预后不良的危险因素。结论住院CAP患者伴发AKI的预后不良。RIFLE诊断及分级标准可有效评估CAP伴发AKI患者的预后。%Objective To explore clinical characteristics and prognostic risk factors in patients with community ac-quired pneumonia(CAP)complicated with acute kidney injury(AKI).Methods In total, 456 CAP patients were included based on the diagnostic guide.According to whether the patients were accompanied with AKI,the patients were divided in-to two groups(non-AKI group and AKI group). AKI group were further divided into risk group, injury group and failure group by RIFLE criteria using admission creatinine.Severity in CAP patients,clinical indexes and prognostic evaluation in-dexes were compared between different groups. Multiple factors were analyzed using Logistic regression model,survival analysis were examined by Kaplan-Meier, which analyzed the risk factors of poor prognosis in CAP patients and the role of RIFLE criteria in prognostic evaluation. Results Thirty percent(135)of the total 456 CAP patients were

  10. Cervicothoracic cystic dysraphism.

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

    2016-09-01

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

  11. Cystic Fibrosis: Prenatal Screening and Diagnosis

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

  12. Cystic pulmonary hydatidosis

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

    2016-01-01

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

  13. Multiple cystic lung disease

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

    2015-01-01

    Multiple cystic lung disease represents a diverse group of uncommon disorders that can present a diagnostic challenge due to the increasing number of diseases associated with this presentation. High-resolution computed tomography of the chest helps to define the morphological aspects and distribution of lung cysts, as well as associated findings. The combination of appearance upon imaging and clinical features, together with extrapulmonary manifestations, when present, permits confident and a...

  14. Cystic tumors of the pancreas

    Morana, Giovanni; Guarise, Alessandro

    2006-01-01

    Cystic tumors of the pancreas are less frequent than solid lesions and are often detected incidentally, as many of these lesions are small and asymptomatic. However, they may be associated with pancreatitis or have malignant potential. With advancements in diagnostic imaging, cystic lesions of the pancreas are being detected with increasing frequency. Many lesions can cause a pancreatic cyst, most being non-neoplastic while approximately 10% are cystic tumors, ranging from benign to highly ma...

  15. Eosinophilic activation in cystic fibrosis.

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

    1994-01-01

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

  16. Cystic lesions of the liver

    Rosado, E.; J Pereira; Bouchaibi, S; Bali, M

    2014-01-01

    LEARNING OBJECTIVES: To present the CT and MRI features of the cystic liver lesions, with emphasis on the differential diagnosis. BACKGROUND: Cystic liver lesions are a frequent finding in abdominal imaging and may represent a broad spectrum of entities, ranging from benign developmental cysts to malignant neoplasms. Radiological features of various cystic liver lesions frequently overlap. Therefore, it is necessary to integrate imaging with clinical and laboratorial findings. The most imp...

  17. Clinico-pathological profile of 22 cases of cystic renal dysplasia.

    Singh, Sompal; Gupta, Ruchika; Nigam, Sonu; Khurana, Nita; Aggarwal, Satish Kumar; Chaturvedi, K Uma; Mandal, Ashish Kumar

    2007-01-01

    Renal dysplasia is one of the major renal developmental anomaly characterized by abnormal structural organization and development of metanephric elements. It is usually detected antenatally or in early childhood. The kidney may be multicystic, aplastic, hypoplastic or duplex. We studied 22 cases of cystic renal dysplasia diagnosed over a period often years to identify the spectrum of morphological changes in dysplastic kidney, with special emphasis on mesenchymal changes. Clinical, radiological and gross morphologicalfeatures were noted. Microscopic features were studied in detail, including the epithelial and mesenchymal changes. Twenty-one of the 22 cases studied were children. One case was a 21-year-old adult, which is a rare age at presentation. Male to female ratio was 1.1:1. One of our patients had contra-lateral ureteric stenosis, a rare anomaly reported with renal dysplasia. Ten patients, all autopsy cases, had multi-system congenital anomalies. As cystic renal dysplasia is not a hereditary disease, it must be differentiated from polycystic kidney disease. Other differential diagnoses are cystic nephroma and cystic partially differentiated nephroblastoma. Histopathological examination is the final diagnostic tool since radiological features alone may not be sufficient to exclude other cystic renal lesions. Cartilage may not be seen in all cases of renal dysplasia. Once diagnosed, other associated anomalies should also be looked for. PMID:17474245

  18. Lithium-induced Nephrotoxicity: A Case Report of Renal Cystic Disease Presenting as a Mass Lesion

    Yvonne McCartney

    2014-11-01

    Full Text Available Lithium is an effective therapeutic agent used in the management of bipolar disorder. However, lithium is also associated with several side effects, including renal toxicity. We present a case of a symptomatic cystic mass lesion in the kidney of a patient who had a history of lithium therapy for the management of bipolar disorder.

  19. Incidentally Detected Primary Giant Renal Cystic Enchinococcosis in a Young Patient: An Underestimated Entity?

    Emre Urer

    2015-01-01

    Full Text Available Echinococcosis is a parasitic infestation caused by Echinococcus granulosus and is an endemic disease in many parts of world. The symptoms and signs depend on the location and size of the cyst. Renal cystic echinococcosis or hydatid cyst (HC disease of the kidney is extremely rare and constitutes only 2-4% of all cases of hydatid disease (HD. We present a 39-year-old male patient who was referred to our outpatient clinic with cystic right kidney mass that was incidentally diagnosed during hepatobiliary ultrasound for chronic hepatitis B evaluation. Routine blood tests were normal without eosinophilia. Indirect haemagglutination test was negative. Abdominal kidneys, ureters, and bladder X-ray showed an 83×95 mm sized curvilinear calcification in the right upper abdominal quadrant. Abdominal computed tomography scan and magnetic resonance imaging demonstrated a 10x9x10 cm sized cystic mass arising from the middle pole of the right kidney, destructing the whole upper pole and extending into the liver. Daughter vesicles were present in the cystic lesion suggesting renal HD. Right retroperitoneal exploration with flank approach and right radical nephrectomy was performed without any complications. Pathology confirmed HC lesion. Following surgery, albendazole 400 mg per os twice daily for 4 weeks was suggested.

  20. Lithium-induced Nephrotoxicity: A Case Report of Renal Cystic Disease Presenting as a Mass Lesion *

    Yvonne McCartney; Cliodhna Browne; Little, Dilly M.; Christian Gulmann

    2014-01-01

    Lithium is an effective therapeutic agent used in the management of bipolar disorder. However, lithium is also associated with several side effects, including renal toxicity. We present a case of a symptomatic cystic mass lesion in the kidney of a patient who had a history of lithium therapy for the management of bipolar disorder.

  1. Cystic Fibrosis: Diet and Nutrition

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

  2. Cystic hemangioblastoma of the brainstem

    Amit Agrawal

    2010-01-01

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

  3. Cystic hemangioblastoma of the brainstem

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

    2010-01-01

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

  4. Benign cystic peritoneal mesothelioma

    Santhosh Shetty

    2014-04-01

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

  5. Childhood abdominal cystic lymphangioma

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

    2002-02-01

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

  6. Imaging from cystic fibrosis

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

  7. Childhood abdominal cystic lymphangioma

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

  8. Cystic hemispheric medulloepithelioma

    Jacqueline du Toit

    2015-10-01

    Full Text Available Medulloepithelioma is a rare primitive embryonal tumour of the central nervous system which typically occurs in early childhood. The histopathological appearance has been confused with primitive neuroectodermal tumours (PNETs, but medulloepitheliomas have a more aggressive clinical course. Imaging typically demonstrates a well-circumscribed, poorly or non-enhancing solid mass, with cyst formation being infrequent. We report the imaging findings in a case of a cystic hemispheric medulloepithelioma. The importance of considering this diagnosis is discussed in view of the propensity to aggressive recurrence in subtotal resection.

  9. First Trimester Cystic Hygroma : Ultrasonographic Findings and Significance

    To evaluate the ultrasonographic findings and the outcome of the cystic hygroma diagnosed in the first trimester of pregnancy. Eleven fetuses with cystic hygroma were found on routine antenatal trans vaginal ultrasonography. Nine fetuses had karyotyping. All of the continued pregnancies had detailed-ultrasonographic follow-up and postnatal physical examination. The thickness of the nuchaltranslucency was 3∼8 mm. Seperation was seen in 3 of 11 cases. 10 cases showed nuchal translucency in dorsalaspect and 1 showed lateral extension. Aneuploidy was found in 2 (Trisomy 21, Triploidy XXY) of 9 cases, which was 22.2%. Among 7 euploid cases, one was spontaneously aborted and one was terminated due to cystic hygroma and bilateral polycystic kidney. Nuchal thickening in the second trimester was found in 3 of 7 cases. One of 7delivered cases showed hemangioma in the cheek and 6 case showed normal on physical examination. If fetal cystic hygroma is suspected in the first trimester, antenatal karyotyping should be done, and detailed-ultrasonographic examination and postnatal physical examination should be recommended for possible associated anomaly even if the karyotype is normal

  10. First Trimester Cystic Hygroma : Ultrasonographic Findings and Significance

    Kim, Young Hwa [Chunan Hospital, Soonchunhyang University College of Medicine, Cheonan (Korea, Republic of)

    1996-12-15

    To evaluate the ultrasonographic findings and the outcome of the cystic hygroma diagnosed in the first trimester of pregnancy. Eleven fetuses with cystic hygroma were found on routine antenatal trans vaginal ultrasonography. Nine fetuses had karyotyping. All of the continued pregnancies had detailed-ultrasonographic follow-up and postnatal physical examination. The thickness of the nuchaltranslucency was 3{approx}8 mm. Seperation was seen in 3 of 11 cases. 10 cases showed nuchal translucency in dorsalaspect and 1 showed lateral extension. Aneuploidy was found in 2 (Trisomy 21, Triploidy XXY) of 9 cases, which was 22.2%. Among 7 euploid cases, one was spontaneously aborted and one was terminated due to cystic hygroma and bilateral polycystic kidney. Nuchal thickening in the second trimester was found in 3 of 7 cases. One of 7delivered cases showed hemangioma in the cheek and 6 case showed normal on physical examination. If fetal cystic hygroma is suspected in the first trimester, antenatal karyotyping should be done, and detailed-ultrasonographic examination and postnatal physical examination should be recommended for possible associated anomaly even if the karyotype is normal

  11. Kidney (Renal) Failure

    ... How is kidney failure treated? What is kidney (renal) failure? The kidneys are designed to maintain proper fluid ... marrow and strengthen the bones. The term kidney (renal) failure describes a situation in which the kidneys have ...

  12. Why kidneys fail in autosomal dominant polycystic kidney disease.

    Grantham, Jared J; Mulamalla, Sumanth; Swenson-Fields, Katherine I

    2011-10-01

    The weight of evidence gathered from studies in humans with hereditary polycystic kidney disease (PKD)1 and PKD2 disorders, as well as from experimental animal models, indicates that cysts are primarily responsible for the decline in glomerular filtration rate that occurs fairly late in the course of the disease. The processes underlying this decline include anatomic disruption of glomerular filtration and urinary concentration mechanisms on a massive scale, coupled with compression and obstruction by cysts of adjacent nephrons in the cortex, medulla and papilla. Cysts prevent the drainage of urine from upstream tributaries, which leads to tubule atrophy and loss of functioning kidney parenchyma by mechanisms similar to those found in ureteral obstruction. Cyst-derived chemokines, cytokines and growth factors result in a progression to fibrosis that is comparable with the development of other progressive end-stage renal diseases. Treatment of renal cystic disorders early enough to prevent or reduce cyst formation or slow cyst growth, before the secondary changes become widespread, is a reasonable strategy to prolong the useful function of kidneys in patients with autosomal dominant polycystic kidney disease. PMID:21862990

  13. Deletion of ErbB4 accelerates polycystic kidney disease progression in cpk mice

    Zeng, Fenghua; Miyazawa, Tomoki; Kloepfer, Lance A.; Harris, Raymond C.

    2014-01-01

    ErbB4 is highly expressed in the cystic kidneys with polycystic kidney diseases. To investigate its potential role in cystogenesis, cpk mice carrying a heart-rescued ErbB4 deletion were generated. Accelerated cyst progression and renal function deterioration were noted as early as 10 days postnatally in cpk mice with ErbB4 deletion compared to cpk mice, as indicated by increased cystic index, higher kidney weight to body weight ratios and elevated BUN levels. No apparent defects in renal deve...

  14. Congenital giant megaureter associated with ipsilateral multicystic dysplastic kidney in newborn

    Rajendran Ramaswamy

    2016-01-01

    Full Text Available Congenital giant megaureter presents as abdominal mass and impose diagnostic difficulties. It can be associated with other upper urinary tract anomalies. A female newborn with antenatal diagnosis of polycystic kidneys was admitted at birth due to lower abdominal mass. Ultrasound and CT scans diagnosed a multiloculated cystic lesion in the mid and lower abdomen along with right side multicystic kidney. At laparotomy, an extaperitoneal, lobulated cystic swelling was found due to rightside giant megaureter. Its lower end was of normal caliber and orthotopic. End cutaneous ureterostomy was done. Intravenous urogram and isotope renograms showed nonfunctioning right kidney. She also had grade II vesicoureteral reflux on left side. Child suffered urinary infection twice. At 9m age, right nephroureterectomy was done. Histopathologic examination was consistent with cystic renal dysplasia and dilated ureter. This is the first case report of giant megaureter associated with ipsilateral multicystic dysplastic kidney in newborn.

  15. Caroli′s syndrome with autosomal recessive polycystic kidney disease

    Prithi Shenoy

    2014-01-01

    Full Text Available Caroli′s syndrome (CS is a rare congenital disorder characterized by multiple segmental cystic or saccular dilatations of the intrahepatic bile ducts and congenital hepatic fibrosis. We report a 9-year-old boy who was diagnosed with CS and autosomal recessive poly-cystic kidney disease. On screening, his 5-month-old asymptomatic sister had multiple dilated biliary radicals with multiple bilateral renal cystic lesions. Both the patient and the affected sibling have been advised regular follow-up for monitoring the progression of the disease. In conclusion, patients with CS should be screened for renal cystic lesions and vice versa even if they are asymptomatic. Also, as the disease is inherited in an autosomal recessive manner, it is important to screen family members for early diagnosis and management.

  16. Caroli's syndrome with autosomal recessive polycystic kidney disease.

    Shenoy, Prithi; Zaki, Syed Ahmed; Shanbag, Preeti; Bhongade, Swapnil

    2014-07-01

    Caroli's syndrome (CS) is a rare congenital disorder characterized by multiple segmental cystic or saccular dilatations of the intrahepatic bile ducts and congenital hepatic fibrosis. We report a 9-year-old boy who was diagnosed with CS and autosomal recessive poly-cystic kidney disease. On screening, his 5-month-old asymptomatic sister had multiple dilated biliary radicals with multiple bilateral renal cystic lesions. Both the patient and the affected sibling have been advised regular follow-up for monitoring the progression of the disease. In conclusion, patients with CS should be screened for renal cystic lesions and vice versa even if they are asymptomatic. Also, as the disease is inherited in an autosomal recessive manner, it is important to screen family members for early diagnosis and management. PMID:24969198

  17. Your Kidneys

    ... you to go to the bathroom. When you pee, the urine goes from the bladder down another tube called the urethra (say: yu-REE-thruh) and out of your body. The kidneys, the bladder, and their tubes ...

  18. Bilateral cystic dysplasia of the rete testis with renal adysplasia.

    Bouron-Dal Soglio, Dorothée; Harvey, Isabelle; Jovanovic, Mubina; Oligny, Luc L; Fournet, Jean-Christophe

    2006-01-01

    Cystic dyplasia of the rete testis (CDRT) is an uncommon, generally unilateral lesion characterized by anastomosing cystic spaces lined by a flattened simple cuboidal epithelium in the rete testis. In the literature this lesion often is associated with an ipsilateral urogenital lesion such as renal agenesia or multicystic dysplasia of the kidney, in order of frequency. The pathogenesis is explained by some authors by their common embryologic origin. We are reporting the finding of bilateral CDRT associated with ultrasound-diagnosed renal adysplasia in a 20-week gestational age fetus with oligohydramnios. Although CDRT has been referred to as being associated with multicystic renal dysplasia or renal agenesis, the present case appears to be unique in combining all the malformations together. PMID:16822083

  19. Polycystic kidney disease gene in the Lewis polycystic kidney rat is mapped to chromosome 10q21–q26

    Yengkopiong JP

    2012-08-01

    Full Text Available Jada Pasquale YengkopiongDr John Garang Memorial University of Science and Technology, Faculty of Science and Technology, Bor, Republic of South SudanBackground: Polycystic kidney disease (PKD is a life-threatening disorder that affects the kidneys of millions of people across the world. The disease is normally inherited, but it can also be acquired, and leads to development of many cysts in the renal nephrons. In this study, the aim was to characterize PKD in the Lewis polycystic kidney (LPK rat, the newest model for human PKD.Methods: Mating experiments were performed between male LPK rats with PKD and female Brown Norway and Wistar Kyoto rats without PKD to raise second filial (F2 and backcross 1 (BC1 progeny, respectively. Rats that developed PKD were identified. Histological examination of the kidneys and liver was performed. Liver tissue samples were collected from each rat and used to extract DNA. The extracted DNA was amplified, and mapping and linkage analyses were performed to identify the quantitative trait locus that controlled the disease phenotypes.Results: It was established that the disease was controlled by a recessive mutation in a single gene (F2: PKD = 42, non-PKD = 110, χ2 = 0.53; BC1: PKD = 67, non-PKD = 72, χ2 = 0.18, P > 0.05 and that the disease was inherited as autosomal recessive polycystic kidney disease (ARPKD. The rats with PKD developed larger fluid-filled cystic kidneys, higher systolic blood pressure, and anemia. However, there were no extrarenal cysts and no pup deaths. Mapping studies and linkage analyses associated the disease phenotypes in both the F2 and BC1 rats to chromosome 10q21–q26, giving a maximum LOD score of 7.9 (P = 0.00001 between peak markers D10Rat180 and D10Rat26.Conclusion: The quantitative trait locus on chromosome 10q21–q26 does not contain the Pkhd-1 gene, and it is different from quantitative trait loci that control ARPKD in other murine models. The candidate genes located in the

  20. Cystic tumors of the pancreas

    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.)

  1. Trichosporon loubieri Infection in a Patient with Adult Polycystic Kidney Disease

    Padhye, Arvind A.; Verghese, Susan; Ravichandran, P.; Balamurugan, G.; Hall, Leslie; Padmaja, P.; Fernandez, Maria C.

    2003-01-01

    A 45-year-old man from Nepal with a 13-year history of polycystic kidney disease was diagnosed as suffering from chronic renal failure with end-stage renal disease. After receiving empirical antituberculosis treatment, he was treated with broad-spectrum antibiotics. A left nephrectomy was performed, and after 4 months, he received a kidney transplant. The left kidney was grossly enlarged, with multiple cystic spaces filled with blackish material. Histologic examination of the excised left kid...

  2. Ultrasonography of Cystic mass of ovary

    Findings of gray scale ultrasound were analyzed in 106 surgically proven cystic masses of ovary. Sonographic differential diagnosis was attempted on the basis of size, definition of border and internal consistency of the mass. Cystic masses of ovary coule be subcategorized into 4 groups: homogenous cystic mass, cystic mass with internal septation, cystic mass with solid foci, and cystic mass with low level internal echo. The results obtained were as follows: 1. 62% of mucinous cyst adenoma(13 cases) were cystic masses with internal septation. 2.62% of serous cyst adenoma(21 cases) were homogenous cystic masses. 3. 14 cases of corpus luteum cyst were 13homogenous cystic masses and 1 cystic mass with septation. 4. 7 cases of endometriosis were 3 homogenous cystic masses, 3 cystic masses with internal echo and 1 cystic mass with solid foci. 5. 63% of cystic teratoma(30 cases)were cystic masses with solid foci. 6. 11 cases of incorrect diagnosis as ovarian cyst were 4 uterine myomas, 2 abscesses in iliac fossa, hematoma, hydrosalpinx, mesenteric cyst, distended urinary bladder, etc

  3. Heat shock proteins in the kidney.

    Sreedharan, Rajasree; Van Why, Scott K

    2016-10-01

    Heat shock proteins (Hsps) are essential to cell survival through their function as protein chaperones. The role they play in kidney health and disease is varied. Hsp induction may be either beneficial or detrimental to the kidney, depending on the specific Hsp, type of cell, and context. This review addresses the role of Hsps in the kidney, including during development, as osmoprotectants, and in various kidney disease models. Heat shock transcription factor, activated by a stress on renal cells, induces Hsp elaboration and separately regulates immune responses that can contribute to renal injury. Induced Hsps in the intracellular compartment are mostly beneficial in the kidney by stabilizing and restoring cell architecture and function through acting as protein chaperones. Intracellular Hsps also inhibit apoptosis and facilitate cell proliferation, preserving renal tubule viability after acute injury, but enhancing progression of cystic kidney disease and malignancy. Induced Hsps in the extracellular compartment, either circulating or located on outer cell membranes, are mainly detrimental through enhancing inflammation pathways to injury. Correctly harnessing these stress proteins promises the opportunity to alter the course of acute and chronic kidney disease. PMID:26913726

  4. Global impact of bronchiectasis and cystic fibrosis

    Margarida Redondo

    2016-09-01

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

  5. Mitochondrial dysfunction in inherited renal disease and acute kidney injury.

    Emma, Francesco; Montini, Giovanni; Parikh, Samir M; Salviati, Leonardo

    2016-05-01

    Mitochondria are increasingly recognized as key players in genetic and acquired renal diseases. Most mitochondrial cytopathies that cause renal symptoms are characterized by tubular defects, but glomerular, tubulointerstitial and cystic diseases have also been described. For example, defects in coenzyme Q10 (CoQ10) biosynthesis and the mitochondrial DNA 3243 A>G mutation are important causes of focal segmental glomerulosclerosis in children and in adults, respectively. Although they sometimes present with isolated renal findings, mitochondrial diseases are frequently associated with symptoms related to central nervous system and neuromuscular involvement. They can result from mutations in nuclear genes that are inherited according to classic Mendelian rules or from mutations in mitochondrial DNA, which are transmitted according to more complex rules of mitochondrial genetics. Diagnosis of mitochondrial disorders involves clinical characterization of patients in combination with biochemical and genetic analyses. In particular, prompt diagnosis of CoQ10 biosynthesis defects is imperative because of their potentially reversible nature. In acute kidney injury (AKI), mitochondrial dysfunction contributes to the physiopathology of tissue injury, whereas mitochondrial biogenesis has an important role in the recovery of renal function. Potential therapies that target mitochondrial dysfunction or promote mitochondrial regeneration are being developed to limit renal damage during AKI and promote repair of injured tissue. PMID:26804019

  6. [Rhinosinusitis in cystic fibrosis].

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

    2015-11-01

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

  7. Neutrophils in cystic fibrosis.

    Laval, Julie; Ralhan, Anjali; Hartl, Dominik

    2016-06-01

    Cystic fibrosis (CF) lung disease is characterized by chronic infection and inflammation. Among inflammatory cells, neutrophils represent the major cell population accumulating in the airways of CF patients. While neutrophils provide the first defensive cellular shield against bacterial and fungal pathogens, in chronic disease conditions such as CF these short-lived immune cells release their toxic granule contents that cause tissue remodeling and irreversible structural damage to the host. A variety of human and murine studies have analyzed neutrophils and their products in the context of CF, yet their precise functional role and therapeutic potential remain controversial and incompletely understood. Here, we summarize the current evidence in this field to shed light on the complex and multi-faceted role of neutrophils in CF lung disease. PMID:26854289

  8. Cystic tumors of Pancreas

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

  9. Cystic tumors of Pancreas

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

  10. Targeted Therapies for Kidney Cancer

    ... for kidney cancer Targeted therapies for kidney cancer Biologic therapy (immunotherapy) for kidney cancer Chemotherapy for kidney cancer Pain control for kidney cancer Treatment choices by stage for ...

  11. Profile of cystic fibrosis

    Mona M. El-Falaki

    2014-09-01

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

  12. Steady Advances Against Cystic Fibrosis

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

  13. Cystic fibrosis in premature infants

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

    2011-01-01

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

  14. Keep Your Kidneys Clear: Kicking Kidney Stones

    ... Your Kidneys Clear Keep Your Kidneys Clear Kicking Kidney Stones Some say that passing a kidney stone is like delivering a baby made of razor ... is that, although they can be excruciatingly painful, kidney stones rarely cause permanent damage, and you may be ...

  15. Cystic Lesions in Autoimmune Pancreatitis

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

    2015-01-01

    Autoimmune pancreatitis (AIP) can be chronic or recurrent, but frequently completely reversible after steroid treatment. A cystic lesion in AIP is a rare finding, and it can mimic a pancreatic cystic neoplasm. Difficulties in an exact diagnosis interfere with treatment, and surgery cannot be avoided in some cases. We report the history of a 63-year-old male presenting with jaundice and pruritus. AIP was confirmed by imaging and elevated IgG4 blood levels, and the patient completely recovered ...

  16. Alveolar inflammation in cystic fibrosis

    Ulrich, Martina; Worlitzsch, Dieter; Viglio, Simona;

    2010-01-01

    BACKGROUND: In infected lungs of the cystic fibrosis (CF) patients, opportunistic pathogens and mutated cystic fibrosis transmembrane conductance regulator protein (CFTR) contribute to chronic airway inflammation that is characterized by neutrophil/macrophage infiltration, cytokine release and...... accumulated in type II alveolar epithelial cells, lacking CFTR. P. aeruginosa organisms were rarely present in inflamed alveoli. CONCLUSIONS: Chronic inflammation and remodeling is present in alveolar tissues of the CF lung and needs to be addressed by anti-inflammatory therapies....

  17. Nutritional management of cystic fibrosis.

    A MacDonald

    1996-01-01

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

  18. Bilateral multiloculated cystic renal cell carcinoma (Case report)

    Gümürdülü, D; Uğuz, A; Gökdemir, A.; Soyupak, B.

    2014-01-01

    Aim: Multiloculated cystic renal cell carcinoma is a rare variant of renal cell carcinoma. Incidence and biological behaviour of the tumor are unknown and bilateral cases are very rare. Case report: Fifty four-years- old male patient was admitted to the Urology policlinic with a left flank pain which was present during one month. On ultra sonographic examination solid hypoecoic mass 37x 32 mm in size and extending to the adrenal area were found at the upperpole of right kidney. Another mass 3...

  19. Hospital-acquired pneumonia

    ... this page: //medlineplus.gov/ency/article/000146.htm Hospital-acquired pneumonia To use the sharing features on this page, please enable JavaScript. Hospital-acquired pneumonia is an infection of the lungs ...

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

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

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

    Tanima Jana

    2015-01-01

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

  2. Bilateral Supernumerary Kidney: A Very Rare Presentation

    To our knowledge, bilateral supernumerary kidney is a very rare renal abnormality and there are five cases presented in the literature. It is difficult to diagnose supernumerary kidney and clinicians have not detected most cases preoperatively. Laboratory and imaging studies were acquired and carefully examined. The normal laboratory tests were found. Emergency ultrasonography was performed and they revealed no signs of parenchymal abnormality in both kidneys. Serial imaging study including enhanced computed tomography (CT) was performed. An imaging study identified bilateral supernumerary kidney with expanded collecting systems. On each side, significant rotation anomaly was found. In addition, there were two different renal arteries originating from the aorta. This report presents radiological determinations of supernumerary kidney bilaterally in a young man. We think that CT commonly appears to be enough for the diagnosis of supernumerary kidneys

  3. Kidney Dysplasia

    ... urinary tract defects. 2 1 Swiatecka-Urban A. Multicystic renal dysplasia. Medscape website. http://emedicine.medscape.com/article/982560- ... 27, 2013. Accessed July 14, 2014. 2 Fetal multicystic dysplastic kidney. Anne & Robert H. Lurie Children’s Hospital of Chicago ...

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

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

    1996-08-01

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

  5. Cystic thymic diseases: CT manifestations

    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.

  6. Glomerulocystic Kidney Disease and its rare associations: an autopsy report of two unrelated cases

    Sachdeva Man

    2007-04-01

    Full Text Available Abstract Background Glomerulocystic kidney disease is an uncommon type of cystic renal disease. It is characterized by cortical microsysts, which are represented by cystic dilatation of Bowman's spaces. Case presentation We describe a case of glomerulocystic disease in a neonate and another in an abortus associated with tracheo-oesophageal fistula and megacystic-megaureter syndrome. The kidney on autopsy was sponge-like and revealed presence of cysts corresponding to dilatations of Bowman's space microscopically. In these two cases, the Glomerulocystic Kidney Disease in one case corresponded to a sporadic form and, in the other, to a syndromic, non-heritable form of glomerulocystic kidney disease. Conclusion The associated anomalies in Glomerulocystic Kidney disease are well described in the literature. Two more new unrelated associations are described in this article.

  7. Mucolytics in cystic fibrosis.

    Henke, Markus O; Ratjen, Felix

    2007-03-01

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

  8. Computed tomography of cystic pancreatic fibrosis

    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.)

  9. Cystic Fibrosis (CF) Respiratory Screen: Sputum

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

  10. Cystic neoplasms of the pancreas

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

  11. Serum pancreatic lipase activity in cystic fibrosis.

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

    1983-01-01

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

  12. Intrarenal mature cystic teratoma associated with renal dysplasia: case report and literature review.

    Otani, M; Tsujimoto, S; Miura, M; Nagashima, Y

    2001-07-01

    We report a case of intrarenal teratoma in a 6-year-old boy. Two years before his operation, multicystic masses had been found in the left side of his abdomen. In the operation, three main cystic masses were located in the upper and lower poles of the left kidney, which were removed in pieces. Histologically, the cyst wall was lined mainly with keratinizing squamous epithelium with hair follicles, shafts and sebaceous glands. The adjacent renal parenchyma showed atrophy, with partially dysplastic and angiomyolipoma-like lesions. Based on these findings, the lesion was diagnosed as mature cystic teratoma of dermoid cyst type. Extragonadal teratoma occurs predominantly along the median line of the body. Intrarenal teratoma is extremely rare; however, it should be distinguished from teratoid Wilms' tumor and other renal cystic lesions. PMID:11472570

  13. Anti-VEGF antibody treatment accelerates polycystic kidney disease.

    Raina, Shagun; Honer, Michael; Krämer, Stefanie D; Liu, Yang; Wang, Xueqi; Segerer, Stephan; Wüthrich, Rudolf P; Serra, Andreas L

    2011-10-01

    Polycystic kidney growth implies expansion of the vasculature, suggesting that vascular endothelial growth factor (VEGF)-dependent processes play a critical role and that VEGF is a putative therapeutic target. Whether an anti-VEGF antibody improves renal cystic disease has not been determined. We administrated 5 mg/kg B20.4.1, an anti-VEGF-A antibody, or vehicle intraperitoneally twice weekly to 4-wk-old male normal (+/+) and cystic (Cy/+) Han:SPRD rats for 6 wk. Renal function, urinary protein excretion, organ/body weight ratios, cyst volume, tubular epithelial cell (TEC) proliferation, renal VEGF, hypoxia-inducible factor (HIF)-1α and -2α expression, renal histology, and kidney hypoxia visualized by [(18)F]fluoromisonidazole positron emission tomography were assessed. The treated compared with untreated +/+ rats had lower TEC proliferation rates, whereas Cy/+ rats receiving B20.4.1 displayed an increased proximal TEC proliferation rate, causing enhanced cyst and kidney growth. The +/+ and Cy/+ rats receiving B20.4.1 had severe renal failure and extensive glomerular damage. Proteinuria, which was highest in anti-VEGF-treated Cy/+ and lowest in untreated normal littermates, was positively correlated with renal HIF-1α and negatively correlated with VEGF expression. The untreated Cy/+ vs. +/+ rats had higher overall [(18)F]fluoromisonidazole uptake. The +/+ rats receiving B20.4.1 vs. untreated had increased [(18)F]fluoromisonidazole uptake, whereas the uptake was unchanged among treated vs. untreated Cy/+ animals. In conclusion, B20.4.1 caused an exaggerated cystic response of the proximal tubules in cystic rats and severe kidney injury that was associated with low renal VEGF and high HIF-1α levels. Anti-VEGF drug therapy may therefore not be a treatment option for polycystic kidney disease. PMID:21677148

  14. Diagnostic Testing in Cystic Fibrosis.

    Brewington, John; Clancy, J P

    2016-03-01

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

  15. MRI of cystic pituitary tumors

    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)

  16. KIDNEY ANOMALIES: HORSE SHOE KIDNEY

    Hemalatha; Komarabattina; Nageshwar Rao; Kotikala Prabhakara

    2015-01-01

    INTRODUCTION : Horse Shoe Kidney was first recognized during an autopsy by De Carpi in 1521. This anomaly consists of two distinct renal masses lying vertically on either side of the midline and connected at their respective lower poles by a parenchymatous or fibrous isthmus that crosses the mid pl ane of the body. This isthmus lies at the level of 4th lumbar vertebra just beneath the origin of inferior mesenteric ...

  17. Diagnostic imaging of infantile abdominal cystic disease

    Thirty-seven patients with infantile abdominal cystic disorders were studied by ultrasound (US), computed tomography (CT), or magnetic resonance imaging (MRI). US studies are simple to conduct, and commonly show an echo-free mass, but tend to yield hyperechoic images when fat and blood are present in the cystic fluid. CT studies, which distinctly demonstrate water, fat, soft tissue density and calcification, are very helpful in the diagnosis of cystic tumor, especially cystic teratoma. MR imaging, which is capable of distinguishing the nature of the cystic fluid (e.g., serous, chylous, bloody, fatty, etc.), is useful in the diagnosis of lymphangioma, teratoma, and torsion of ovarian cystic tumor. Based on the results of the present study, US may be considered to be the modality of first choice in the examination of infantile abdominal cystic disorders, and MRI and/or CT should be employed additionally as necessary in that order. (author)

  18. Cystic ovarian lesions in SSFP diffusion imaging

    MR assessments of ovarian cystic lesions are usually based on morphological features, signal intensities and enhancement with contrast media. This study was performed to evaluate the usefulness of the steady-state free precession (SSFP) diffusion imaging of cystic ovarian lesions for analyzing cystic contents. Sixty-one ovarian cystic lesions in 37 patients were examined. The diffusion-related coefficient (DRC) and the ratio of the relative apparent diffusion coefficient of the lesion to that of subcutaneous fat tissue (rADCL/rADCF) were calculated from SSFP diffusion images. The DRCs and the rADCL /rADCF ratios in endometrial cysts and in the fatty parts of dermoid cysts were significantly lower than in other cystic tumors. SSFP diffusion imaging can be included in clinical practice to analyze ovarian cystic lesions within a short scan time; the DRC and the rADCL /rADCF ratio are useful for evaluating cystic contents. (author)

  19. [Echinococcal infection of the kidney (author's transl)].

    Rost, A; Fiedler, U

    1976-11-01

    800 echinococcal infections of the kidney have been described in the world literature, nevertheless, here this disease is very rare. As a result of increasing tourism and employment of workers coming from endemic areas, the morbidity rises and the clinical picture gains in importance. A review has been made of epidemiology, life cycle and symptomatology of the cystic echinococcus and the diagnosis, differential diagnosis and therapy of this parasitosis has been discussed with reference to two misdiagnosed cases from our own patients. PMID:825762

  20. Lactate in cystic fibrosis sputum

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

    2011-01-01

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

  1. Cystic echinococcosis of the liver

    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...

  2. Multicystic dysplastic kidney: a retrospective study with clinicopathological correlation

    N. Rukmangadha

    2013-04-01

    Full Text Available Background: Multicystic dysplastic kidney (MCDK is one of the most frequently observed congenital urinary tract abnormalities. Methods: Retrospective study of MCDK in nephrectomy specimens seen over a 20 year period at a tertiary care teaching hospital in South India. Results: MCDK was evident in 6 of the 230 nephrectomy specimens (2.6% seen during the 20 year period of study. Their median age [interquartile range (IQR] was 2.25 (1.075-7.750 years; there were 3 males. MCDK more frequently involved the right kidney (5/6; 4 cases presented with megaureter. Salient histopathological findings included cystically dilated spaces lined by flattened to cuboidal lining epithelium with intervening stroma showing entrapped, dilated, narrowed immature tubules with surrounding smooth muscle cuffing. Conclusions: Histopathological examination remains the mainstay in the diagnosis and helps in differentiating MCDK from other cystic lesions.

  3. Kidney Stones in Children

    ... 345 KB) Alternate Language URL Kidney Stones in Children Page Content On this page: What is a ... the ureters. [ Top ] Are kidney stones common in children? No exact information about the incidence of kidney ...

  4. Diabetes and Kidney Disease

    ... Disease, and Other Dental Problems Diabetic Eye Disease Diabetes and Kidney Disease What are my kidneys and ... urine until releasing it through urination. How can diabetes affect my kidneys? Too much glucose , also called ...

  5. Injury - kidney and ureter

    ... Kidney stone disease Radiation to the belly area Trauma ... Emergency symptoms may include: Abdominal pain and swelling Severe ... one kidney is affected and the other kidney is healthy, you may ...

  6. Laboratory-acquired brucellosis

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

    2008-01-01

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

  7. Primary synovial sarcoma of the kidney

    Shwetank Mishra

    2015-01-01

    Full Text Available Primary synovial sarcoma (SS of the kidney is a very rare disease as well as a diagnostic dilemma. Here, we present a case of a 60-year-old male clinically diagnosed as renal cell carcinoma. The radical nephrectomy specimen showed a well-circumscribed renal mass of approximately 13 cm × 9 cm × 7 cm. The cut-surface of the mass was tawny and firm, with cystic areas, and also showed focal hemorrhage and necrosis. Histologically, the tumor was composed of spindle cells arranged in the intersecting fascicles, alternating with hypocellular areas suggestive of monophasic synovial sarcoma. Morphological and immunohistochemical features were compatible with the diagnosis of SS of the kidney.

  8. Autosomal dominant polycystic kidney disease: new treatment options and how to test their efficacy

    Wüthrich, R.P.; Serra, A. L.; Kistler, A.D.

    2009-01-01

    Autosomal dominant polycystic kidney disease (ADPKD) represents a slowly progressing cystic kidney disorder which evolves into end-stage renal disease in the majority of patients. Currently, there are no established treatments to retard the progression of the disease, but several promising therapeutic options are being tested in ongoing clinical trials. An inherent dilemma for the investigation of therapies in ADPKD is the dissociation of the early onset and constant rate of cyst growth from ...

  9. Pharmacogenetics of cystic fibrosis treatment.

    Carter, Suzanne C; McKone, Edward F

    2016-08-01

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

  10. Posttraumatic Cranial Cystic Fibrous Dysplasia

    Arata Tomiyama

    2011-01-01

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

  11. MRI in mucoviscidosis (cystic fibrosis)

    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.)

  12. Mesenteric cystic lymphangioma mimicking malignancy.

    Hureibi, Khalid; Sunidar, Osama A

    2014-01-01

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

  13. [Cystic fibrosis and associated complications].

    Schwarz, C; Staab, D

    2015-03-01

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

  14. Breakdown in Breathing: The Complexities of Cystic Fibrosis

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

  15. Cystic Fibrosis Research | NIH MedlinePlus the Magazine

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

  16. Computed tomography of the kidneys

    The use of the CT scan in renal pathology is reviewed and updated, stating the indications and advantages of the method in relation to the other imaging techniques. The technique employed in this examination and the risks involved, including the contrast medium are discussed. The main part of the paper provides a summary of renal pathology, such as cystic renal masses, benign and malign solid tumours, differential diagnoses, pseudo-renal tumours, congenital inflammatory disease, as well as the value of CT in trauma and obstructive conditions of the urinary system. Other benefits are its use in CT-guided biopsy puncture and the drainage of collections, as well as nephrostomies. The cases described represent the authors' cumulative experience of the method acquired in various departments of radiology in Brazil and other countries. (author)

  17. Finger clubbing in cystic fibrosis.

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

    1986-01-01

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

  18. A case of cystic fibrosis

    Maria Alice Monti

    2009-09-01

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

  19. Cerebellar Cystic Hemangioblastoma: Case Report

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

    2001-01-01

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

  20. Ceramide mediates lung fibrosis in cystic fibrosis

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

    2013-01-01

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

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

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

    2016-01-01

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

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

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

    2016-01-01

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

  3. Kidney Disease Basics

    ... injury . This can occur in a person with normal kidneys or in someone who already has kidney problems. ... attack. What your kidneys do. You have two kidneys. They are bean-shaped and about the size of a fist. They are located in the ...

  4. Simple Kidney Cysts

    ... cysts do not enlarge the kidneys, replace their normal structure, or cause reduced kidney function like cysts do in people with PKD. ... the kidneys and what do they do? The kidneys are two bean-shaped organs, each about the size of a fist. They are located near the ...

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

    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...

  6. Pneumonia - adults (community acquired)

    ... breathing (respiratory) condition in which there is an infection of the lung. This article covers community-acquired pneumonia (CAP). This type of pneumonia is found in persons who have not recently been in the hospital or another health care facility such as a ...

  7. Etiopathology of acquired cholesteatoma

    Prabodh Karnik

    2011-01-01

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

  8. Evidence of Inhaled Tobramycin in Non-Cystic Fibrosis Bronchiectasis

    Vendrell, Montserrat; Muñoz, Gerard; de Gracia, Javier

    2015-01-01

    There is currently less experience with inhaled tobramycin in non-cystic fibrosis bronchiectasis than in cystic fibrosis (CF). Intravenous formulation and solution for inhalation (TSI) have been studied in non-CF bronchiectasis patients with chronic P. aeruginosa bronchial infection. An improvement in clinical parameters and a reduction in bacterial density have been shown with both inhaled solutions in these patients. However, further trials are needed to determine the most effective dose and administration protocol in these patients. Based on the current evidence, recommendations cannot be made regarding the use of TSI to treat exacerbations. Although no systemic toxicity has been reported in studies specifically investigating this treatment, patients with known kidney disease or ear disorders should be treated with caution. Adverse respiratory effects are reported to be more common in non-CF patients than in CF patients, who tend to be non-smokers and younger. Research is being conducted into the possibility of combining tobramycin with other antibiotics to increase its antibacterial activity. In this review we will present and discuss the published evidence regarding the use of inhaled tobramycin in non–CF bronchiectasis. PMID:25893022

  9. Cystic pulmonary chondroid hamartoma: a case report

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

  10. HOME CARE IN CYSTIC-FIBROSIS PATIENTS

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

    1995-01-01

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

  11. Cystic pulmonary chondroid hamartoma: a case report

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

    2008-06-15

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

  12. Laparoscopic cholecystectomy in adult cystic fibrosis.

    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.

  13. Outcome in cystic fibrosis liver disease.

    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.

  14. Sarcoidosis in an adult with cystic fibrosis.

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

    1989-01-01

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

  15. Self-management education for cystic fibrosis.

    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.

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

    Mansfeld, R. van

    2014-01-01

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

  17. Biologic Therapy (Immunotherapy) for Kidney Cancer

    ... for kidney cancer Targeted therapies for kidney cancer Biologic therapy (immunotherapy) for kidney cancer Chemotherapy for kidney cancer Pain control for kidney cancer Treatment choices by stage for ...

  18. Acquired epidermolysis bullosa

    Maricel Sucar Batista; Yanier Serrano García; Taimí Miranda Vergara

    2015-01-01

    Epidermolysis bullosa is a group of diseases or skin disorders genetically transmitted and it is characterized by the appearance of bullae, ulcers and skin wounds. It usually appears at birth or in the first months of life. This is a case of a 72-year-old female patient who comes to the dermatology department with skin lesions of 6 months of evolution. A skin biopsy was performed, taking a sample for direct and indirect immunofluorescence. Acquired epidermolysis bullosa of unknown etiology wa...

  19. Acquired hypertrichosis lanuginosa

    Kumar Pramod

    1993-01-01

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

  20. AIDS: acquired immunodeficiency syndrome

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

    2002-01-01

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

  1. Endocrine Disorders in Cystic Fibrosis.

    Blackman, Scott M; Tangpricha, Vin

    2016-08-01

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

  2. Pulmonary complications of cystic fibrosis

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

  3. Liver manifestations of cystic fibrosis

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

  4. The Irish cystic fibrosis database.

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

    1995-01-01

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

  5. Antibiotic allergy in cystic fibrosis

    Parmar, J.; Nasser, S.

    2005-01-01

    Allergic reactions to antibiotics are more common in cystic fibrosis (CF) than in the general population. This in part is due to the improving survival in adults with CF and the increased use of high dose intravenous antibiotics. While some are immediate anaphylaxis type (IgE mediated) reactions, the majority are late onset and may have non-specific features such as rash and fever. Piperacillin has consistently been found to have the highest rate of reported reactions (30–50%). There is a low...

  6. Laparoscopic resection of an adrenal pseudocyst mimicking a retroperitoneal mucinous cystic neoplasm

    Bum-Soo Kim; Sun-Hyung Joo; Sung-Il Choi; Jeong-Yoon Song

    2009-01-01

    Adrenal pseudocysts are rare cystic masses that arise within the adrenal gland and are usually non-functional and asymptomatic. Adrenal pseudocysts consist of a fibrous wall without a cellular lining. We report a patient with a 9 cm, left-sided suprarenal cystic mass who presented with abdominal discomfort of 2 years' duration. A 38-year-old woman was referred to our service for evaluation of abdominal discomfort and gastrointestinal symptoms. Routine laboratory tests were within normal limits. An abdominal computed tomography scan showed a 9 cm × 8 cm × 8 cm well-defined cystic lesion displacing the left kidney. Magnetic resonance imaging showed a cystic lesion with low signal intensity on the T1-weighted image and high signal intensity on the T2-weighted image. A laparoscopic left adrenalectomy was performed to diagnose the lesion. The final pathology showed an adrenal pseudocyst without a cellular lining. The patient had no postoperative complications and she was discharged four days after surgery.

  7. June 2014 critical care case of the month: acute exacerbation in cystic fibrosis

    Yun S

    2014-06-01

    Full Text Available No abstract available. Article truncated after 150 words. History of Present Illness: A 28 year-old woman with a history of cystic fibrosis, presented with worsening shortness of breath and cough associated with productive secretions. She was diagnosed with cystic fibrosis when she was 14 months old, and has a history of multiple inpatient admissions for acute pulmonary exacerbation of cystic fibrosis. Her most recent hospitalization was a month prior to this admission, and sputum culture demonstrated methicillin-resistant Staphylococcus aureus, multidrug-resistant Pseudomonas aeruginosa, and Achromobacter xylosoxidans. She was treated with linezolide, meropenum, colistin, and azithromycin with significant symptom improvement, then, discharged home with ciprofloxacin, linezolide and zosyn. However, she developed worsening respiratory distress again and came back to hospital. In the emergency department she required 10 L/min of oxygen to maintain an SpO2 above 90 %. PMH: cystic fibrosis, seizure, kidney stone, portacath placement, gastrostomy tube placement. Medications: azithromycin 500 mg 3 times, dornase alpha 1 mg/ml nebulizer twice ...

  8. Deletion of ErbB4 accelerates polycystic kidney disease progression in cpk mice.

    Zeng, Fenghua; Miyazawa, Tomoki; Kloepfer, Lance A; Harris, Raymond C

    2014-09-01

    ErbB4 is highly expressed in the cystic kidneys with polycystic kidney diseases. To investigate its potential role in cystogenesis, cpk mice carrying a heart-rescued ErbB4 deletion were generated. Accelerated cyst progression and renal function deterioration were noted as early as 10 days postnatally in cpk mice with ErbB4 deletion compared to cpk mice, as indicated by increased cystic index, higher kidney weight to body weight ratios, and elevated BUN levels. No apparent defects in renal development were noted with ErbB4 deletion itself. Increased cell proliferation was predominately seen in the cortex of cystic kidneys with or without ErbB4 deletion. However, there was significantly more cell proliferation in the cyst-lining epithelial cells in cpk mice with ErbB4 deletion. TUNEL staining localized apoptotic cells mainly to the renal medulla. There were significantly more apoptotic cells in the cyst-lining epithelial cells in ErbB4-deleted cpk kidneys, with decreased levels of cyclin D1, increased levels of p21, p27, and cleaved caspase 3. Thus, lack of ErbB4 may contribute to elevated cell proliferation and unbalanced cell apoptosis, resulting in accelerated cyst formation and early renal function deterioration. These studies suggest that the high level of ErbB4 expression seen in cpk mice may exert relative cytoprotective effects in renal epithelia. PMID:24670412

  9. Learning-By-Being-Acquired

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

    -categorization theories, we argue that R&D team reorganization increases the acquired inventors’ use of the prior stock of technological knowledge of the acquiring firm after the acquisition. Furthermore, this effect is enhanced if acquired inventors have higher innovation ability relative to their acquiring peers but is...

  10. Cystic Fibrosis Transmembrane Conductance Regulator Activation by Roflumilast Contributes to Therapeutic Benefit in Chronic Bronchitis

    Lambert, James A.; Raju, S. Vamsee; Tang, Li Ping; McNicholas, Carmel M.; Li, Yao; Courville, Clifford A.; Farris, Roopan F.; Coricor, George E.; Smoot, Lisa H.; Mazur, Marina M.; Dransfield, Mark T; Bolger, Graeme B.; Rowe, Steven M

    2014-01-01

    Cigarette smoking causes acquired cystic fibrosis transmembrane conductance regulator (CFTR) dysfunction and is associated with delayed mucociliary clearance and chronic bronchitis. Roflumilast is a clinically approved phosphodiesterase 4 inhibitor that improves lung function in patients with chronic bronchitis. We hypothesized that its therapeutic benefit was related in part to activation of CFTR. Primary human bronchial epithelial (HBE) cells, Calu-3, and T84 monolayers were exposed to whol...

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

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

    2012-01-01

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

  12. Two cases of cystic meningioma

    Two cases of cystic meningioma are presented. Case 1: A 60-year-old woman, who had undergone surgery for the removal of a left parasagittal meningioma 6 years before, was admitted on February 14, 1986, complaining of paraplegia. A computerized tomography (CT) scan revealed the recurrence of the tumor and a large surrounding cyst in the right parasagittal region. On February 20, 1986, bilateral parietal craniectomy was performed, and a tumor with a large cyst was totally removed. Histological examination showed the tumor to be an angioblastic meningioma; neoplastic cells were found in the cyst walls. Case 2: A 34-year-old man, who had been treated by hemodialysis because of chronic renal failure, was diagnosed as having a left frontal tumor by CT scan after becoming unconscious. He was admitted on September 8, 1986, and a CT scan revealed a contrast-enhancing mural nodule and a surrounding cyst in the left frontal region. On September 10, 1986, bilateral frontal craniotomy was performed, and the tumor with a cyst was totally resected. Histological examination showed the tumor to be a meningothelial meningioma; no evidence of neoplastic cells was found in the cyst wall. The authors discuss the mechanism of cyst formation and the CT findings of cystic meningioma, and the reports in the literature are reviewed. (author)

  13. Aspergillus infections in cystic fibrosis.

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

    2016-07-01

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

  14. Acquired von Willebrand Syndrome

    郭涛

    2005-01-01

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

  15. Acquired epidermolysis bullosa

    Maricel Sucar Batista

    2015-12-01

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

  16. [Polycystic kidney disease: from genetics to dialysis].

    Ortiz Arduan, Alberto

    2012-01-01

    Autosomal dominant polycystic kidney disease (ADPKD) is the most common genetic cause of end stage renal disease. In addition, end-stage renal disease is complicated by acquired polycystic kidney disease. Recent advances in our understanding of the pathogenesis of ADPKD have identified the primary cilium as key to cystogenesis, and have defined the molecular, cellular and tissue pathogenesis of the disease, leading to the design of clinical trials that may ultimately lead to effective therapy of the disease. In 2012 a key trial has shown that blockade of vasopressin receptors with tolvaptan slows the rate of cyst growth and may slow the loss of renal function. PMID:24298871

  17. National Kidney Foundation

    ... Sign up for our FREE magazine, Kidney Living Organ Donation & Transplantation Be an Organ Donor Living Donation Donor ... Giving Primary menu Home Prevention Kidney Disease Patients Organ Donation & Transplantation Professionals Events Advocacy Donate Search Search Header ...

  18. About Chronic Kidney Disease

    ... Sign up for our FREE magazine, Kidney Living Organ Donation & Transplantation Be an Organ Donor Living Donation Donor ... Giving Primary menu Home Prevention Kidney Disease Patients Organ Donation & Transplantation Professionals Events Advocacy Donate Search Search Header ...

  19. Pregnancy and Kidney Disease

    ... Sign up for our FREE magazine, Kidney Living Organ Donation & Transplantation Be an Organ Donor Living Donation Donor ... Giving Primary menu Home Prevention Kidney Disease Patients Organ Donation & Transplantation Professionals Events Advocacy Donate Search Search Header ...

  20. American Kidney Fund

    ... York Golf Classic The 11th Annual New York Golf Classic Each August, supporters in our Northeast Region hit the links in support of AKF. Kidney Action Day Kidney Action Day Learn about our signature outreach event. Free health screenings ...

  1. Tests for Kidney Health

    ... York Golf Classic The 11th Annual New York Golf Classic Each August, supporters in our Northeast Region hit the links in support of AKF. Kidney Action Day Kidney Action Day Learn about our signature outreach event. Free health screenings ...

  2. Chronic Kidney Disease (CKD)

    ... York Golf Classic The 11th Annual New York Golf Classic Each August, supporters in our Northeast Region hit the links in support of AKF. Kidney Action Day Kidney Action Day Learn about our signature outreach event. Free health screenings ...

  3. HIV and Kidney Disease

    ... Sheets Permission to Use Fact Sheets Sponsors and Advertising Privacy Policy Project ... Disease WHY SHOULD PEOPLE WITH HIV CARE ABOUT KIDNEY DISEASE? WHAT IS NORMAL KIDNEY FUNCTION? HOW DO I KNOW IF THERE ARE PROBLEMS ...

  4. Diet - chronic kidney disease

    ... Many foods contain extra iron (liver, beef, pork, chicken, lima and kidney beans, iron-fortified cereals). Talk to your provider or dietitian about which foods with iron you can eat because of your kidney disease.

  5. Kidney Disease of Diabetes

    ... a healthy kidney from a donor. Most U.S. citizens who develop kidney failure are eligible for federally ... Budget & Legislative Information Strategic Plans & Reports Advisory & Coordinating Committees Research Areas Jobs at NIDDK FAQs Visit Us ...

  6. Kidney transplant - series (image)

    ... functions that both kidneys perform in healthy people. Kidney transplant recipients are required to take immunosuppressive medications for the rest of the lives, to prevent immune rejection of the transplanted organ.

  7. Kidney transplant - series (image)

    ... Donor kidneys are obtained from either brain-dead organ donors, or from living relatives or friends of the ... the lower right quadrant of the abdomen. The donor kidney is transplanted into the right lower pelvis of the recipient.

  8. Fetal and Postnatal Magnetic Resonance Imaging of Unilateral Cystic Renal Dysplasia in a Neonate with Tuberous Sclerosis.

    Tyagi, Vineet; Bornstein, Eran; Schacht, Robert; Lala, Shailee; Milla, Sarah

    2014-02-01

    Tuberous sclerosis (TS) is an autosomal dominant condition associated with mutations in the TSC1 and/or TSC2 genes. Clinical manifestations are multisystemic, and they often include lesions in the brain, skin, heart, kidneys, and bones. TSC2 gene mutations can be seen concomitantly with autosomal dominant polycystic kidney disease gene mutations. We present a case of a fetus with prenatal diagnosis of TS that had unique asymmetrical distribution of renal cystic disease. We describe the extensive work up with both fetal and neonatal magnetic resonance imaging with correlating images of the unilateral polycystic renal disease in addition to typical TS brain findings. PMID:24495558

  9. Renal (Kidney) Manifestations in TSC

    Full Text Available ... Download the AfiniTRAC patient support brochure . National Kidney Foundation: www.kidney.org Kidney and Urology Foundation: www.kidneyurology.org Polycystic Kidney Disease Foundation: www. ...

  10. Living Donor Kidney Transplant Surgery

    Full Text Available ... you can see the left kidney here and right kidney here. And we generally use the left ... that the vein to the kidney of the right kidney. Here is another CT angiogram image, and ...

  11. Keeping Your Single Kidney Healthy

    ... factors for kidney problems? Certain treatments for childhood cancer can sometimes cause kidney problems. These include radiation to the kidney , chemotherapy that can affect the kidney (cisplatin, carboplatin, methotrexate and/or ifosfamide), or other medications that can ...

  12. Renal (Kidney) Manifestations in TSC

    Full Text Available ... to kidney impairment and even kidney failure, requiring dialysis or transplantation. Lastly, renal cell carcinoma, the least ... kidney failure occurs, renal replacement therapy such as dialysis or transplantation is necessary. How kidney cysts develop ...

  13. Renal (Kidney) Manifestations in TSC

    Full Text Available ... can maintain normal kidney function. Additional Resources Afinitor® www.afinitor.com | Download the AfiniTRAC patient support brochure . National Kidney Foundation: www.kidney.org Kidney and Urology Foundation: www.kidneyurology. ...

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

    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...

  15. Deficiency of FLCN in mouse kidney led to development of polycystic kidneys and renal neoplasia.

    Jindong Chen

    Full Text Available The Birt-Hogg-Dubé (BHD disease is a genetic cancer syndrome. The responsible gene, BHD, has been identified by positional cloning and thought to be a novel tumor suppressor gene. BHD mutations cause many types of diseases including renal cell carcinomas, fibrofolliculomas, spontaneous pneumothorax, lung cysts, and colonic polyps/cancers. By combining Gateway Technology with the Ksp-Cre gene knockout system, we have developed a kidney-specific BHD knockout mouse model. BHD(flox/flox/Ksp-Cre mice developed enlarged kidneys characterized by polycystic kidneys, hyperplasia, and cystic renal cell carcinoma. The affected BHD(flox/flox/Ksp-Cre mice died of renal failure at approximate three weeks of age, having blood urea nitrogen levels over tenfold higher than those of BHD (flox/+/Ksp-Cre and wild-type littermate controls. We further demonstrated that these phenotypes were caused by inactivation of BHD and subsequent activation of the mTOR pathway. Application of rapamycin, which inhibits mTOR activity, to the affected mice led to extended survival and inhibited further progression of cystogenesis. These results provide a correlation of kidney-targeted gene inactivation with renal carcinoma, and they suggest that the BHD product FLCN, functioning as a cyst and tumor suppressor, like other hamartoma syndrome-related proteins such as PTEN, LKB1, and TSC1/2, is a component of the mTOR pathway, constituting a novel FLCN-mTOR signaling branch that regulates cell growth/proliferation.

  16. The senile kidney

    Denisova Т.Р.

    2015-03-01

    Full Text Available The given work summarizes external data and self-obtained results on development and diagnostic of kidney involution modifications. Article discusses definition of "senile kidney" as a clinical and pathomorphological term. Major statements on pathophysiological causes of age-associated renal disorders and their prognosis, specifics of chronic kidney disease in elderly and senile patients have been reviewed. Phenomenon of renal "multimorbidity" in eldely maximizes worsening risk of unmodifiable kidney function.

  17. Myofibroblasts in Fibrotic Kidneys

    Nakagawa, Naoki; Duffield, Jeremy S.

    2013-01-01

    Fibrosis of the kidney glomerulus and interstitium are characteristic features of almost all chronic kidney diseases. Fibrosis is tightly associated with destruction of capillaries, inflammation, and epithelial injury which progresses to loss of nephrons, and replacement of kidney parenchyma with scar tissue. Understanding the origins and nature of the cells known as myofibroblasts that make scar tissue is central to development of new therapeutics for kidney disease. Whereas many cell lineag...

  18. CONSENSUS EXPERT RECOMMENDATIONS FOR THE DIAGNOSIS AND MANAGEMENT OF AUTOSOMAL RECESSIVE POLYCYSTIC KIDNEY DISEASE: REPORT OF AN INTERNATIONAL CONFERENCE

    Guay-Woodford, Lisa M.; Bissler, John J.; Braun, Michael C.; Bockenhauer, Detlef; Cadnapaphornchai, Melissa A.; Dell, Katherine M.; Kerecuk, Larissa; Liebau, Max C; Alonso-Peclet, Maria H.; Shneider, Benjamin; Emre, Sukru; Heller, Theo; Kamath, Binita M.; Murray, Karen F.; Moise, Kenneth

    2014-01-01

    Autosomal recessive polycystic kidney disease (ARPKD; MIM 263200) is a severe, typically early onset form of cystic disease that primarily involves the kidneys and biliary tract. Phenotypic expression and age at presentation can be quite variable1. The incidence of ARPKD is 1 in 20,000 live births2, and its pleotropic manifestations are potentially life-threatening. Optimal care requires proper surveillance to limit morbidity and mortality, knowledgeable approaches to diagnosis and treatment,...

  19. Rapidly Evoluting Congenital Cystic Neuroblastoma in a Neonate

    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.

  20. A Case of a Solid Renal Mass Together with a Cystic Pancreatic Lesion in a 50-Year-Old Patient

    Satorres Rosas J

    2005-03-01

    Full Text Available CONTEXT: Pancreatic cysts may be incidentally detected in asymptomatic patients evaluated for other clinical manifestations. Microcystic adenomas are particularly rare among pancreatic cyst neoplasms. They are benign lesions and can present as solitary pancreatic tumors or as a radiological manifestation combined with other cystic and tumoral lesions affecting different organs. CASE REPORT: A 50-year-old man presented with hematuria. A computed tomography scan of the abdomen showed a 9-centimeter renal mass in the left kidney consistent with a renal-cell carcinoma as well as a cystic lesion the head of the pancreas. The histopathological study of the cystic mass, following a computed tomography guided biopsy, showed a microcystic adenoma. Therefore, further studies were performed so as to assess the relationship between both lesions and determine the final diagnosis. CONCLUSIONS: Microcystic adenomas are exceedingly rare tumors among pancreatic cysts. The combination of a solid renal mass and a pancreatic cystic lesion should lead to a broad differential diagnosis. Pancreatic magnetic resonance imaging has been proven to be particularly useful in evaluating cystic masses. The presence of walls and internal septations in the pancreatic mass with gadolinium enhancement should raise the possibility of an underlying Von Hippel-Lindau syndrome.

  1. Chronic kidney disease

    ... enable JavaScript. Chronic kidney disease is the slow loss of kidney function over time. The main job of the kidneys is to remove wastes and excess water from the body. Causes ... over months or years. You may not notice any symptoms for some time. The loss of function may be so slow that you ...

  2. Amyloidosis and Kidney Disease

    ... a physical exam urinalysis blood tests a kidney biopsy Medical and Family History Taking a medical and family history may help ... of the kidneys with a medical and family history a physical exam urinalysis blood tests a kidney biopsy A health care provider diagnoses dialysis-related amyloidosis ...

  3. Pyrrolidine dithiocarbamate reduces the progression of total kidney volume and cyst enlargement in experimental polycystic kidney disease.

    Ta, Michelle H T; Rao, Padmashree; Korgaonkar, Mayuresh; Foster, Sheryl F; Peduto, Anthony; Harris, David C H; Rangan, Gopala K

    2014-12-01

    Heterocyclic dithiocarbamates have anti-inflammatory and anti-proliferative effects in rodent models of chronic kidney disease. In this study, we tested the hypothesis that pyrrolidine dithiocarbamate (PDTC) reduces the progression of polycystic kidney disease (PKD). Male Lewis polycystic kidney (LPK) rats (an ortholog of Nek8/NPHP9) received intraperitoneal injections of either saline vehicle or PDTC (40 mg/kg once or twice daily) from postnatal weeks 4 until 11. By serial magnetic resonance imaging at weeks 5 and 10, the relative within-rat increase in total kidney volume and cyst volume were 1.3-fold (P = 0.01) and 1.4-fold (P < 0.01) greater, respectively, in LPK + Vehicle compared to the LPK + PDTC(40 mg/kg twice daily) group. At week 11 in LPK rats, PDTC attenuated the increase in kidney weight to body weight ratio by 25% (P < 0.01) and proteinuria by 66% (P < 0.05 vs. LPK + Vehicle) but did not improve renal dysfunction. By quantitative whole-slide image analysis, PDTC did not alter interstitial CD68+ cell accumulation, interstitial fibrosis, or renal cell proliferation in LPK rats at week 11. The phosphorylated form of the nuclear factor (NF)-κB subunit, p105, was increased in cystic epithelial cells of LPK rats, but was not altered by PDTC. Moreover, PDTC did not significantly alter nuclear expression of the p50 subunit or NF-κB (p65)-DNA binding. Kidney enlargement in LPK rats was resistant to chronic treatment with a proteasome inhibitor, bortezomib. In conclusion, PDTC reduced renal cystic enlargement and proteinuria but lacked anti-inflammatory effects in LPK rats. PMID:25501440

  4. CT diagnosis of cystic ovarian lesions

    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.)

  5. Magnetic resonance imaging of cystic ovarian tumors

    We studied 48 cases of cystic ovarian tumors by magnetic resonance imaging (MRI) with 0.15 T resistive system and examined the ability of qualitative diagnosis by means of signal intensities and caluculated T1 values. MRI supposed to discriminate among various kinds of cystic ovarian tumors in most cases, especially dermoid cysts, endometrial cysts and cystadenomas. MRI has lots of merits which other imaging methods cannot offer, though it takes a long scan time and high cost at the present time. It plays an important role in the diagnosis of cystic ovarian tumors. (author)

  6. Acquired Blaschkoid dermatitis

    Mercy P

    2007-01-01

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

  7. Pulmonary changes in cystic fibrosis

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

  8. Pyelo-cystic Reflux in F-18 FDG PET Scan Due to Ureteral Obstruction

    A 72-year-old woman with a history of cervical cancer was treated with brachytherapy and chemotherapy. Combined F-18 FDG PET/CT performed for restaging demonstrated increased FDG uptake in a hypodense cystic lesion at the posterior part of the right renal cortex and a hypermetabolic soft tissue mass at the right parailiac region suggestive of a metastatic lymph node causing ureteral obstruction. There had been no FDG uptake in the cystic lesion on the FDG PET/CT study performed 1 year before. These findings suggest that the increased FDG uptake in the cystic lesion was caused by pyelocystic reflux due to ureteral obstruction secondary to parailiac lymph node metastasis (Figs. 1 and 2). Several renal lesions may have increased metabolism, such as renal cell carcinoma, lymphoma, oncocytoma, adult Wilms' tumor, angiomyolipoma, metastatic lesions, xanthogranulo-matous pyelonephritis and infected cyst. Most of these lesions are solid. Some infected renal cysts may be FDG avid, but in this situation increased FDG uptake is observed on the wall of the cyst. In our case, FDG uptake was seen in the entire cystic lesion. The patient had no symptoms or laboratory findings related to infection. Cysts are the most common space-occupying lesions of the kidney. The vast majority of these are simple cysts that are usually unilateral and solitary. Simple cysts are asymptomatic, except when complications exist such as hemorrhage, infection or rupture. There have been a few reports on spontaneous communications between renal cysts and the pyelocaliceal system, in most cases involving ruptures of the cysts into the pyelocaliceal system due to increased intracystic pressure caused by bleeding or infection of the cyst. In the present case, the cause of the connection between the cystic cavity and the pyelocaliceal system is the increased pressure in the renal pelvic cavity due to the ureteral obstruction secondary to parailiac lymph node metastasis

  9. Pyelo-cystic Reflux in F-18 FDG PET Scan Due to Ureteral Obstruction

    Reyhan, Mehmet [Baskent Univ., Adana (Turkmenistan)

    2013-09-15

    A 72-year-old woman with a history of cervical cancer was treated with brachytherapy and chemotherapy. Combined F-18 FDG PET/CT performed for restaging demonstrated increased FDG uptake in a hypodense cystic lesion at the posterior part of the right renal cortex and a hypermetabolic soft tissue mass at the right parailiac region suggestive of a metastatic lymph node causing ureteral obstruction. There had been no FDG uptake in the cystic lesion on the FDG PET/CT study performed 1 year before. These findings suggest that the increased FDG uptake in the cystic lesion was caused by pyelocystic reflux due to ureteral obstruction secondary to parailiac lymph node metastasis (Figs. 1 and 2). Several renal lesions may have increased metabolism, such as renal cell carcinoma, lymphoma, oncocytoma, adult Wilms' tumor, angiomyolipoma, metastatic lesions, xanthogranulo-matous pyelonephritis and infected cyst. Most of these lesions are solid. Some infected renal cysts may be FDG avid, but in this situation increased FDG uptake is observed on the wall of the cyst. In our case, FDG uptake was seen in the entire cystic lesion. The patient had no symptoms or laboratory findings related to infection. Cysts are the most common space-occupying lesions of the kidney. The vast majority of these are simple cysts that are usually unilateral and solitary. Simple cysts are asymptomatic, except when complications exist such as hemorrhage, infection or rupture. There have been a few reports on spontaneous communications between renal cysts and the pyelocaliceal system, in most cases involving ruptures of the cysts into the pyelocaliceal system due to increased intracystic pressure caused by bleeding or infection of the cyst. In the present case, the cause of the connection between the cystic cavity and the pyelocaliceal system is the increased pressure in the renal pelvic cavity due to the ureteral obstruction secondary to parailiac lymph node metastasis.

  10. Myofibroblasts in Fibrotic Kidneys

    Nakagawa, Naoki; Duffield, Jeremy S

    2013-01-01

    Fibrosis of the kidney glomerulus and interstitium are characteristic features of almost all chronic kidney diseases. Fibrosis is tightly associated with destruction of capillaries, inflammation, and epithelial injury which progresses to loss of nephrons, and replacement of kidney parenchyma with scar tissue. Understanding the origins and nature of the cells known as myofibroblasts that make scar tissue is central to development of new therapeutics for kidney disease. Whereas many cell lineages in the body have become defined by well-established markers, myofibroblasts have been much harder to identify with certainty. Recent insights from genetic fate mapping and the use of dynamic reporting of cells that make fibrillar collagen in mice have identified with greater clarity the major population of myofibroblasts and their precursors in the kidney. This review will explore the nature of these cells in health and disease of the kidney to underst and their central role in the pathogenesis of kidney disease. PMID:24187654

  11. Multicystic transformation of the kidneys in dialysis patients

    Frifelt, J J; Larsen, C; Elle, Birgitte;

    1989-01-01

    In a dialysis population patients who had been treated merely with haemodialysis (HD) or continuous ambulatory peritoneal dialysis (CAPD) were examined with ultrasound. The occurrence of multicystic transformation of the kidney was 4/15 in HD patients and 8/25 in CAPD patients with no significant...... difference between the two groups. There was a significant association between the occurrence of multicystic transformation and the patient age while there was no significant association to the duration of dialysis or the duration of the uraemic state. No tumour or any other complication to cystic...... transformation was found. In 582 persons without renal disease examined as controls we found 44 with cystic change. This material does not support the recommendation of regular ultrasound examination of our dialysis patients. However, until these results can be confirmed by prospective studies we must recommend...

  12. Pathohistological changes in fetuses with cystic fibrosis

    Đolai Matilda

    2012-01-01

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

  13. Cystic cervical intramedullary schwannoma with syringomyelia

    Shenoy S

    2005-01-01

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

  14. Nutrition in Cystic Fibrosis: Macro- and Micronutrients

    Oudshoorn, Johanna Hermiena

    2006-01-01

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

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

    Tünger, Özlem

    2013-01-01

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

  16. Ultrasonographic findings of cystic mass of ovary

    The sonographic findings of histologically proven 160 cystic masses of ovary were analysed to find helpful diagnostic sign. The results were as follows: 1. The bilateralities were identified in 1 serous cystadenocarcinoma (33%), 2 mucinous cystadenocarcinoma (40%), and 2 dermoid cysts (5%). 2. 76% of serous cystadenoma were homogenous cystic masses. 3. 64% of mucinous cystadenoma were cystic masses with internal septation (7 daugter cysts), and 5 mucinous cystadenomas were showed multiple homogenous low level echoes within it. 4. 64% of dermoid cyst revealed strong echogenic solid component within the upper part of the cyst. 5. 80% of corpus luteal cyst were mainly cystic lesion with several low-level internal echoes. 6. 80% of malignant ovarian mass revealed irregular, thick internal septations and irregular inhomogenous internal solid components within it

  17. Immunoreactive trypsin and neonatalscreening for cystic fibrosis

    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

  18. New approaches to the autosomal recessive polycystic kidney disease patient with dual kidney-liver complications.

    Telega, Grzegorz; Cronin, David; Avner, Ellis D

    2013-06-01

    Improved neonatal medical care and renal replacement technology have improved the long-term survival of patients with ARPKD. Ten-yr survival of those surviving the first year of life is reported to be 82% and is continuing to improve further. However, despite increases in overall survival and improved treatment of systemic hypertension and other complications of their renal disease, nearly 50% of survivors will develop ESRD within the first decade of life. In addition to renal pathology, patients with ARPKD develop ductal plate malformations with cystic dilation of intra- and extrahepatic bile ducts resulting in CHF and Caroli syndrome. Many patients with CHF will develop portal hypertension with resulting esophageal varices, splenomegaly, hypersplenism, protein losing enteropathy, and gastrointestinal bleeding. Management of portal hypertension may require EBL of esophageal varices or porto-systemic shunting. Complications of hepatic involvement can include ascending cholangitis, cholestasis with malabsorption of fat-soluble vitamins, and rarely benign or malignant liver tumors. Patients with ARPKD who eventually reach ESRD, and ultimately require kidney transplantation, present a unique set of complications related to their underlying hepato-biliary disease. In this review, we focus on new approaches to these challenging patients, including the indications for liver transplantation in ARPKD patients with severe chronic kidney disease awaiting kidney transplant. While survival in patients with ARPKD and isolated kidney transplant is comparable to that of age-matched pediatric patients who have received kidney transplants due to other primary renal diseases, 64-80% of the mortality occurring in ARPKD kidney transplant patients is attributed to cholangitis/sepsis, which is related to their hepato-biliary disease. Recent data demonstrate that surgical mortality among pediatric liver transplant recipients is decreased to <10% at one yr. The immunosuppressive regimen

  19. Axillary Schwannoma with Extensive Cystic Degeneration

    Jadhav, Chaithra R; Angeline, N R; Bipin Kumar; Ramachandra V Bhat; G Balachandran

    2013-01-01

    Schwannoma affect mainly head, neck, and flexor aspect of the limbs. Neurogenic tumors arising from the brachial plexus are rare and axillary schwannoma is extremely uncommon. Cystic degeneration is common in longstanding cases and which when aspirated may yield only macrophages or lymphocytes leading to false diagnosis of the case in spite of strong clinical suspicion. We report one such rare case of a solitary axillary schwannoma with extensive cystic degeneration, which was misdiagnosed on...

  20. Unusual Presentation of Cystic Papillary Thyroid Carcinoma

    Patil, Vijayraj S.; Abhishek Vijayakumar; Neelamma Natikar

    2012-01-01

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

  1. Communicating with young adults with cystic fibrosis.

    Webb, A. K.

    1995-01-01

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

  2. Mesenchymal Cystic Hamartoma Presenting with Spontaneous Pneumothorax

    J Glezos; Toppin, D; Cooney, T

    2003-01-01

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

  3. Adventitial Cystic Disease of the Popliteal Artery

    Kawarai, Shun-ichi; Fukasawa, Manabu; Kawahara, Yu

    2012-01-01

    We describe a patient with adventitial cystic disease of the popliteal artery with intermittent claudication involving the right calf during exercise. Magnetic resonance imaging (MRI) and computed tomography (CT) revealed a cystic lesion that encircled and compressed the popliteal artery. Resection of the cyst involving a segment of the affected popliteal artery and interposing an autologous vein graft resolved the symptoms, and the postoperative course was uneventful. The cyst was histologic...

  4. Cystic lymphangioma of breast: a case report

    Cystic lymphangioma are rare benign tumors that originate as a congenital anomaly of the lymphatic system. Their common presentation is in neck and axillary area at the birth, during infancy or early pediatric age group. Author reports an unusual case of cystic lymphangioma of breast which was diagnosed in a 32-year-old woman. The findings were very dense, sharply defined, and multilobulated masses on mammography, and multiloculated echo free lesions on ultrasonogram

  5. Cystic lymphangioma of breast: a case report

    Shin, Hyun Ja; Yun, Eun Joo; Lee, Jong Koo; Kim, Jeong Rye [Korea Veterans Hospital, Seoul (Korea, Republic of)

    1995-02-15

    Cystic lymphangioma are rare benign tumors that originate as a congenital anomaly of the lymphatic system. Their common presentation is in neck and axillary area at the birth, during infancy or early pediatric age group. Author reports an unusual case of cystic lymphangioma of breast which was diagnosed in a 32-year-old woman. The findings were very dense, sharply defined, and multilobulated masses on mammography, and multiloculated echo free lesions on ultrasonogram.

  6. Adenoid cystic carcinoma of the lacrimal gland

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

    2013-01-01

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

  7. Acquired immunologic tolerance: with particular reference to transplantation

    Starzl, Thomas E.

    2007-01-01

    The first unequivocally successful bone marrow cell transplantation in humans was recorded in 1968 by the University of Minnesota team of Robert A. Good (Gatti et al. Lancet 2: 1366–1369, 1968). This achievement was a direct extension of mouse models of acquired immunologic tolerance that were established 15 years earlier. In contrast, organ (i.e. kidney) transplantation was accomplished precociously in humans (in 1959) before demonstrating its feasibility in any experimental model and in the...

  8. Collecting duct carcinoma of the kidney: CT and pathologic correlation

    Yoon, Seong Kuk [Department of Diagnostic Radiology, Dong-A University College of Medicine, 1, 3-Ga, Dongdaesin-Dong, Seo-Ku, Busan (Korea, Republic of)]. E-mail: cerub@chollian.net; Nam, Kyung Jin [Department of Diagnostic Radiology, Dong-A University College of Medicine, 1, 3-Ga, Dongdaesin-Dong, Seo-Ku, Busan (Korea, Republic of); Rha, Seo-Hee [Department of Pathology, Dong-A University College of Medicine, Busan (Korea, Republic of); Kim, Jeong Kon [Department of Radiology, Ulsan University College of Medicine, Seoul (Korea, Republic of); Cho, Kyoung-Sik [Department of Radiology, Ulsan University College of Medicine, Seoul (Korea, Republic of); Kim, Bohyun [Department of Radiology, Sungkyunkwan University School of Medicine, Seoul (Korea, Republic of); Kim, Kie Hwan [Department of Radiology, Korea Cancer Center Hospital, Seoul (Korea, Republic of); Kim, Kyung-Ah [Department of Radiology, Korea University College of Medicine, Seoul (Korea, Republic of)

    2006-03-15

    Purpose: We characterized CT findings of collecting duct carcinoma of the kidney and correlated these with the histopathologic findings. Materials and methods: CT scans of 18 patients with pathologically proven collecting duct carcinoma of the kidney were retrospectively reviewed. We analyzed CT findings of collecting duct carcinoma and also correlated CT findings with the histopathologic findings. Results: The mean size of the tumors was 6.9 cm and all cases were solid. Seventeen (94%) tumors had a medullary location. Nine (69%) and 11 (85%) cases showed weak and heterogeneous enhancement, respectively. A cystic component (50%) was frequently seen within the tumors. Lymphadenopathy and metastasis were noted in 10 (56%) and 6 (33%) cases, respectively. Perinephric stranding and vascular invasion were present in 10 (56%) and 5 (28%) cases, respectively. In 17 (94%) of the 18 cases, involvement of the renal sinus was present. Infiltrative growth (67%) and preservation of the renal contour (61%) were more common than expansile growth (33%) and exophytic configuration (39%), respectively. These CT features were well correlated with the histopathologic findings. Conclusion: Medullary location, weak and heterogeneous enhancement, involvement of the renal sinus, infiltrative growth, preserved renal contour, and a cystic component are CT findings frequently seen in patients with collecting duct carcinoma of the kidney. CT findings are nevertheless nonspecific and do not allow collecting duct carcinoma to be easily differentiated from the other subtypes of renal cell carcinoma. However, when CT demonstrates a renal tumor with these findings, collecting duct carcinoma can be considered in the differential diagnosis.

  9. CT findings in skeletal cystic echinococcosis

    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

  10. Cystic fibrosis from the gastroenterologist's perspective.

    Ooi, Chee Y; Durie, Peter R

    2016-03-01

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

  11. Intercontrole acquiring by Framatome

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

  12. [Clinical diagnosis of Autosomal Dominant Polycystic Kidney Disease].

    Magistroni, Riccardo; Izzi, Claudia; Scolari, Francesco

    2016-01-01

    Autosomal dominant polycystic kidney disease (ADPKD) is the most common genetic disorder related to kidney. ADPKD is usually easy to diagnose in people who have a family history of ADPKDs developing typical symptoms, including flank, abdominal pain or macroscopic hematuria. In this setting, diagnosis in adults at risk for ADPKD is commonly performed by ultrasonography, which reveals two enlarged kidneys with multiple bilateral cysts. ADPKD may be more difficult to diagnose in the absence of family history or in subjects with atypical presentation, including asymmetric or focal renal imaging findings, discordant disease within family, early onset of ADPKD and development of ESRD before 30 yr of age. The presence of a total of three or more renal cysts for at-risk subjects aged 15-39 years and two cysts or more in each kidney for at-risk subjects aged 40-59 years are sufficient for the diagnosis of ADPKD. The absence of any renal cyst is sufficient for disease exclusion only for at-risk subjects aged 40 years or older. If the family history is negative, the diagnosis of ADPKD can be made in a patient with enlarged kidneys, numerous cysts, presence of liver cysts and absence of findings suggesting a different cystic disease. If the imaging diagnosis is not clear or showing atypical manifestations in subjects, molecular genetic testing should be performed. PMID:27067212

  13. Diabetes and kidney disease

    Diabetic nephropathy; Nephropathy - diabetic; Diabetic glomerulosclerosis; Kimmelstiel-Wilson disease ... Diabetic kidney disease is a major cause of sickness and death in people with diabetes. It can ...

  14. Male fertility in cystic fibrosis.

    Chotirmall, S H

    2011-04-05

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

  15. Antibiotic allergy in cystic fibrosis.

    Parmar, J S; Nasser, S

    2005-06-01

    Allergic reactions to antibiotics are more common in cystic fibrosis (CF) than in the general population. This in part is due to the improving survival in adults with CF and the increased use of high dose intravenous antibiotics. While some are immediate anaphylaxis type (IgE mediated) reactions, the majority are late onset and may have non-specific features such as rash and fever. Piperacillin has consistently been found to have the highest rate of reported reactions (30-50%). There is a low risk of cross reactions between penicillins and other non-beta-lactam classes of antibiotics in penicillin skin prick positive patients. Carbapenems should only be used with extreme caution in patients with positive skin prick tests to penicillin. However, aztreonam can be used safely in patients who are penicillin allergic with positive skin prick reactions. The aminoglycosides are a relatively uncommon cause of allergic reactions, but patients who react to one member of the family may cross react with other aminoglycosides. Desensitisation relies on the incremental introduction of small quantities of the allergen and has been used for penicillins, ceftazidime, tobramycin and ciprofloxacin and must be repeated before each course. Personalized cards should be regularly updated for patients who develop allergic reactions. Written instructions on the emergency treatment of allergic reactions should be provided to patients self-administering intravenous antibiotics at home. Further research is required to identify risk factors and predictors for antibiotic allergy. PMID:15923254

  16. Microbial ecology and adaptation in cystic fibrosis airways

    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 with...

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

    Weiss Nielsen, Martin

    Bacterial infections have a large impact on the health of the general public, however individuals with cystic fibrosis (CF) are immensely susceptible to acquire pulmonary infections with environmental bacteria, viruses and fungal species. Ultimately pulmonary infections are the major cause of...... develop an accurate tool for growing biofilms that can mimic the cystic fibrosis airways, emulating one of the most important characteristics of the human airways, the oxygen environments. Microfluidic approaches that allow biofilm formation under controllable oxygen concentrations, and furthermore enable....... Different microenvironments within the airways of the patients lead to phenotypic and genotypic diversification, sculpting the bacteria to become an efficient colonizer despite the environmental niche in the CF airways. Despite intensive antibiotic treatment and a profound immunologic response, the...

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

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

    2013-01-01

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

  19. Nitric oxide metabolites in cystic fibrosis lung disease

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

    1998-01-01

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

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

    Durie, P R

    1994-01-01

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

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

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

    2016-05-01

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

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

    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.

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

    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

  4. Living Donor Kidney Transplant Surgery

    Full Text Available ... kidneys. There is plenty of room for the new kidney. We actually put it down in part ... to both remove their kidneys and put a new one in, so that’s not necessary. We now ...

  5. Eating Right for Kidney Health

    Eating Right for Kidney Health Tips for People with Chronic Kidney Disease (CKD) National Kidney Disease Education Program hat ... eat healthier. These tips will help you eat right as you manage your CKD. The First Steps ...

  6. Renal (Kidney) Manifestations in TSC

    Full Text Available ... increased blood pressure, but usually they do not cause discomfort. However, sometimes the kidney is filled with cysts, and this can lead to kidney impairment and even kidney failure, requiring dialysis or transplantation. Lastly, renal cell carcinoma, ...

  7. Living Donor Kidney Transplant Surgery

    Full Text Available ... painful, we have really excellent visualization of the anatomy of the kidney and organs around the kidney. ... which gives us an excellent view of the anatomy of the kidneys, including the blood vessels. And ...

  8. Renal (Kidney) Manifestations in TSC

    Full Text Available ... blood pressure can accelerate the loss of kidney function when the kidneys are filled with cysts. If ... surgical removal can lead to loss of kidney function. As of April 26, 2012, adults with TSC ...

  9. Kidney and Urinary Tract

    2005-01-01

    11.1.Kidney function2005391 Increased levels of advanced oxidationproducts are associated with atherosclerosis in chron-ic kidney disease.YANG Xiaobing(杨小兵),et al.Dept Nephrol,Nanfang Hops,1st Milit Med Univ,Guangzhou.Chin J Intern Med 2005;44(5):342-346.Objective:Accumulation of advanced oxidation protein

  10. Acute kidney injury.

    Lang, Joanna; Zuber, Kim; Davis, Jane

    2016-04-01

    Acute kidney injury (AKI) complicates up to 20% of all hospital admissions. Responding to the increase in admissions, complications, mortality, morbidity, and cost of AKI, Kidney Disease: Improving Global Outcomes convened an expert panel to study the issue, review the literature, and publish guidelines to evaluate and treat patients with AKI in the acute setting. This article reviews those guidelines. PMID:27023656

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

    Machado, G.O.

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

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

    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;…

  13. A residual cystic lesion in acute disseminated encephalomyelitis

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

    2000-09-01

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

  14. Acute Scedosporium apiospermum Endobronchial Infection in Cystic Fibrosis.

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

    2016-06-01

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

  15. Endocytosis and intracellular protein degradation in cystic fibrosis fibroblasts

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

  16. Synchronous clear cell renal cell carcinoma and multilocular cystic renal cell neoplasia of low malignant potential: A clinico-pathologic and molecular study.

    Raspollini, Maria Rosaria; Castiglione, Francesca; Cheng, Liang; Montironi, Rodolfo; Lopez-Beltran, Antonio

    2016-05-01

    We report a rare case of synchronous clear cell renal cell carcinoma and multilocular cystic renal cell neoplasia of low malignant potential in the same kidney. The tumors were seen incidentally in a 45-year-old man. Pathologic study revealed that the former tumor was nucleolar grade 2, and the multilocular cystic renal cell neoplasia of low malignant potential was nucleolar grade 1. At immunohistochemistry, the clear cells in both tumors were positive for CD10 and CA IX. Interestingly, these uncommon synchronous tumors showed a different KRAS/NRAS mutation analysis that was characterized by KRAS mutation at codon p.G12C in the clear cell renal cell carcinoma, while this mutation was not present in the case of multilocular cystic renal cell neoplasia of low malignant potential. NRAS mutation was not seen in any of the tumors. PMID:26874573

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

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

    2014-01-01

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

  18. [Promoting Living Kidney Transplantation].

    Lin, Chiu-Chu

    2016-04-01

    Kidney transplantation is the best approach for treating patients with end stage renal disease, offering patients the best chance of returning to normal health. While the techniques used in kidney transplantation surgery are mature and highly successful, there is a severe shortage of donor organs. Statistics show a serious imbalance between organ donations and patients on the waiting list for organ transplantation. Moreover, evidence from empirical studies has shown a better transplantation outcome for patients who receive living donor transplantation than for those who receive organs from cadavers. Although using relatives as donors offers an effective way to reduce the problem of organ shortage, this strategy faces many challenges and many other factors affect the promotion of living donor transplantation. This article elaborates how cultural and psychological factors, kidney transplantation awareness, and ethics and laws impact upon living kidney donations and then proposes coping strategies for promoting living kidney transplantation. PMID:27026555

  19. Kidney Injury Accelerates Cystogenesis via Pathways Modulated by Heme Oxygenase and Complement

    Zhou, Juling; Ouyang, Xiaosen; Schoeb, Trenton R.; Bolisetty, Subhashini; Cui, Xiangqin; Mrug, Sylvie; Yoder, Bradley K.; Johnson, Martin R.; Alexander J. Szalai; Mrug, Michal

    2012-01-01

    AKI accelerates cystogenesis. Because cystogenic mutations induce strong transcriptional responses similar to those seen after AKI, these responses may accelerate the progression of cystic renal disease. Here, we modulated the severity of the AKI-like response in Cys1cpk/cpk mice, a model that mimics autosomal recessive polycystic kidney disease. Specifically, we induced or inhibited activity of the renoprotective enzyme heme oxygenase (HO) and determined the effects on renal cystogenesis. We...

  20. Dyspnea perception in cystic fibrosis patients

    B. Ziegler

    2013-09-01

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

  1. Cystic fibrosis transmembrane conductance regulator chloride channel blockers: Pharmacological, biophysical and physiological relevance

    Paul; Linsdell

    2014-01-01

    Dysfunction of the cystic fibrosis transmembrane con-ductance regulator(CFTR) chloride channel causes cys-tic fibrosis, while inappropriate activity of this channeloccurs in secretory diarrhea and polycystic kidney dis-ease. Drugs that interact directly with CFTR are there-fore of interest in the treatment of a number of diseasestates. This review focuses on one class of small mol-ecules that interacts directly with CFTR, namely inhibi-tors that act by directly blocking chloride movementthrough the open channel pore. In theory such com-pounds could be of use in the treatment of diarrheaand polycystic kidney disease, however in practice allknown substances acting by this mechanism to inhibitCFTR function lack either the potency or specificity forin vivo use. Nevertheless, this theoretical pharmaco-logical usefulness set the scene for the developmentof more potent, specific CFTR inhibitors. Biophysically,open channel blockers have proven most useful as ex-perimental probes of the structure and function of theCFTR chloride channel pore. Most importantly, the useof these blockers has been fundamental in developing afunctional model of the pore that includes a wide innervestibule that uses positively charged amino acid sidechains to attract both permeant and blocking anionsfrom the cell cytoplasm. CFTR channels are also subjectto this kind of blocking action by endogenous anionspresent in the cell cytoplasm, and recently this blocking effect has been suggested to play a role in the physio-logical control of CFTR channel function, in particular as a novel mechanism linking CFTR function dynamically to the composition of epithelial cell secretions. It has also been suggested that future drugs could target this same pathway as a way of pharmacologically increasing CFTR activity in cystic fibrosis. Studying open channel blockers and their mechanisms of action has resulted in significant advances in our understanding of CFTR as a pharmacological target in disease states, of

  2. Genetics of Cystic Fibrosis: Clinical Implications.

    Egan, Marie E

    2016-03-01

    Cystic fibrosis (CF) is a common life-shortening autosomal recessive genetic disorder caused by mutations in the gene that encodes for the cystic fibrosis transmembrane conductance regulator protein (CFTR). Almost 2000 variants in the CFTR gene have been identified. The mutational classes are based on the functional consequences on CFTR. New therapies are being developed to target mutant CFTR and restore CFTR function. Understanding specific CF genotypes is essential for providing state-of-the art care to patients. In addition to the variation in CFTR genotype, there are several modifier genes that contribute to the respiratory phenotype. PMID:26857764

  3. Cystic fibrosis, intravenous antibiotics, and home therapy.

    Hammond, L J; Caldwell, S; Campbell, P W

    1991-01-01

    The survival rate of patients with cystic fibrosis has improved considerably in the last 20 years. Although not all of the factors accounting for this change are understood, aggressive nutritional management and treatment of pulmonary exacerbations certainly play a role. Home intravenous (IV) antibiotic delivery for pulmonary exacerbation has proved to be as effective as hospital treatment and offers significant advantages to the patient and family. This article examines the microbiology of pulmonary infections in patients with cystic fibrosis, as well as antimicrobial therapy, methods of IV administration, home IV therapy, and the nurse practitioner's role in this home program in the future. PMID:1990112

  4. Diagnosis of Adult Patients with Cystic Fibrosis.

    Nick, Jerry A; Nichols, David P

    2016-03-01

    The diagnosis of cystic fibrosis (CF) is being made with increasing frequency in adults. Patients with CF diagnosed in adulthood typically present with respiratory complaints, and often have recurrent or chronic airway infection. At the time of initial presentation individuals may appear to have clinical manifestation limited to a single organ, but with subclinical involvement of the respiratory tract. Adult-diagnosed patients have a good response to CF center care, and newly available cystic fibrosis transmembrane receptor-modulating therapies are promising for the treatment of residual function mutation, thus increasing the importance of the diagnosis in adults with unexplained bronchiectasis. PMID:26857767

  5. Qulaity of Life in Cystic Fibrosis Children

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

    2013-01-01

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

  6. Elevated c-myc protooncogene expression in autosomal recessive polycystic kidney disease

    The polycystic kidney diseases (PKDs) are a group of disorders characterized by the growth of epithelial cysts from the nephrons and collecting ducts of kidney tubules. The diseases can be inherited or can be provoked by environmental factors. To investigate the molecular basis of the abnormal cell growth associated with PKD, c-myc protooncogene expression was studied in a mouse model for autosomal recessive PKD. Homozygous recessive C57BL/6J (cpk/cpk) mice develop massively enlarged cystic kidneys and die from renal failure shortly after 3 weeks of age. Quantitative dot blot and RNA blot hybridization experiments in which whole kidney poly(A)+ RNA was hybridized with a c-myc RNA probe showed a 2- to 6-fold increase in c-myc mRNA at 2 weeks, and a 25- to 30-fold increase in c-myc mRNA at 3 weeks of age in polycystic mice, as compared to normal littermates. c-myc expression was also examined under two conditions in which kidney cell growth was experimentally induced in normal adult mice: compensatory renal hypertrophy and tubule regeneration following folic acid-induced renal cell injury. While compensatory hypertrophy resulted in only a small increase in c-myc, folic acid treatment gave rise after 24 hr to a 12-fold increase in c-myc RNA. The induction of c-myc by folic acid is consistent with increased cellular proliferation regenerating tubules. In contrast, polycystic kidneys show only a minimal increase in cellular proliferation over that seen in normal kidneys, while c-myc levels were found to be markedly elevated. Thus, the level of c-myc expression in cystic kidneys appears to be out of proportion to the rate of cell division, suggesting that elevated and potentially abnormal c-myc expression may be involved in the pathogenesis of PKD

  7. Cystic Eccrine Spiradenoma of the Finger Mimicking a Ganglion

    Khalid F. Jaber, MBChB

    2014-01-01

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

  8. Detection of cystic structures using pulsed ultrasonically induced resonant cavitation

    Bar-Cohen, Yoseph (Inventor); Kovach, John S. (Inventor)

    2002-01-01

    Apparatus and method for early detection of cystic structures indicative of ovarian and breast cancers uses ultrasonic wave energy at a unique resonance frequency for inducing cavitation in cystic fluid characteristic of cystic structures in the ovaries associated with ovarian cancer, and in cystic structures in the breast associated with breast cancer. Induced cavitation bubbles in the cystic fluid implode, creating implosion waves which are detected by ultrasonic receiving transducers attached to the abdomen of the patient. Triangulation of the ultrasonic receiving transducers enables the received signals to be processed and analyzed to identify the location and structure of the cyst.

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

    Jea Andrew

    2011-08-01

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

  10. And the Winner is - Acquired

    Henkel, Joachim; Rønde, Thomas; Wagner, Marcus

    2015-01-01

    New entrants to a market tend to be superior to incumbents in originating radical innovations. We provide a new explanation for this phenomenon, based on markets for technology. It applies in industries where successful entrepreneurial firms, or their technologies, are acquired by incumbents that...... lower probability accompanies higher value in case of success—that is, a more radical innovation. In the second stage, successful entrants bid to be acquired by the incumbent. We assume that entrants cannot survive on their own, so being acquired amounts to a prize in a contest. We identify an...

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

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

    2000-01-01

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

  12. Zinc supplementation in children with cystic fibrosis

    Cystic fibrosis (CF) leads to malabsorption of macro- and micronutrients. Symptomatic zinc deficiency has been reported in CF but little is known about zinc homeostasis in children with CF. Zinc supplementation (Zn suppl) is increasingly common in children with CF but it is not without theoretcial r...

  13. Nutritional assessment in children with cystic fibrosis

    Optimal nutrition, including consuming 35–40% of calories (kcal) as fat, is a vital part of the management of cystic fibrosis (CF), and involves accurate assessment of dietary intake. We compared 3 methods of nutritional assessment in 8– to 14-year-old children (n=20) with CF: 1) a 24-h Dietary Reca...

  14. Pseudomonas aeruginosa biofilms in cystic fibrosis

    Høiby, Niels; Ciofu, Oana; Bjarnsholt, Thomas

    2010-01-01

    The persistence of chronic Pseudomonas aeruginosa lung infections in cystic fibrosis (CF) patients is due to biofilm-growing mucoid (alginate-producing) strains. A biofilm is a structured consortium of bacteria, embedded in a self-produced polymer matrix consisting of polysaccharide, protein and...

  15. Respiratory bacterial infections in cystic fibrosis

    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...

  16. Barriers to adherence in cystic fibrosis

    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 of...

  17. Inhalation of antibiotics in cystic fibrosis

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

    1995-01-01

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

  18. Surgical management of pneumothorax in cystic fibrosis.

    Seddon, D. J.; Hodson, M E

    1988-01-01

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

  19. Spontaneous pneumothorax in cystic adenomatoid malformation

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

  20. Spontaneous pneumothorax in cystic adenomatoid malformation

    Gaisie, G.; Sang Oh, K.

    1983-07-01

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

  1. Cystic fibrosis, are we missing in India?

    Pramila Menon

    2012-09-01

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

  2. The cystic fibrosis of exocrine pancreas

    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....

  3. [Macrolides, Pseudomonas aeruginosa and cystic fibrosis].

    Guillot, M; Amiour, M; El Hachem, C; Harchaoui, S; Ribault, V; Paris, C

    2006-10-01

    Long-term low dose azithromycin treatment in cystic fibrosis patients with chronic Pseudomonas aeruginosa infection is safe and reduces the decline in lung function, the number of acute exacerbations and improves nutritional status; underlying efficacy mechanisms are multiple and synergistic. PMID:17370396

  4. Intestinal organoids as model for cystic fibrosis

    Dekkers, J.F.

    2015-01-01

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

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

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

    2016-01-01

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

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

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

    2015-01-01

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

  7. National Kidney Disease Education Program

    ... Keeping Your Kidneys Healthy Testing for Kidney Disease Diet and Lifestyle Changes Medicines and Kidney Disease Treatment for Kidney Failure ​ Identify & M​anage Patients Managing CKD in Primary Care Promoting Patient Self Management CKD and Nutrition​​ Training for CDEs, RDs, and ...

  8. Testing for Kidney Disease

    ... statement, and the Director's Update newsletter Offices & Divisions Organizational structure and descriptions ... kidney disease usually does not have signs (a change in your body) or symptoms (a change in ...

  9. Acute arterial occlusion - kidney

    ... arterial thrombosis; Renal artery embolism; Acute renal artery occlusion; Embolism - renal artery ... often result in permanent kidney failure. Acute arterial occlusion of the renal artery can occur after injury ...

  10. Kidney Stones (For Parents)

    ... hospital emergency room. To make a diagnosis of kidney stones, the doctor will ask about the symptoms and how long they've been going on; your child's diet; factors that could be causing dehydration; any family ...

  11. Kidney Disease (Nephropathy)

    ... Glucose Testing Medication Doctors, Nurses & More Oral Health & Hygiene Women A1C Insulin Pregnancy 8 Tips for Caregivers ... Other symptoms of kidney disease include loss of sleep, poor appetite, upset stomach, weakness, and difficulty concentrating. ...

  12. Diet and Kidney Stones

    ... of kidney stone is a uric acid stone. Red meat and shellfish have high concentrations of a natural ... cut down on high-purine foods such as red meat, organ meats, and shellfish, and follow a healthy ...

  13. Kidney Disease of Diabetes

    ... Research Training & Career Development Grant programs for students, postdocs, and faculty Research at NIDDK Labs, faculty, and ... diabetes, digestive and liver diseases, kidney diseases, weight control and nutrition, urologic diseases, endocrine and metabolic diseases, ...

  14. Polycystic Kidney Disease

    ... Research Training & Career Development Grant programs for students, postdocs, and faculty Research at NIDDK Labs, faculty, and ... diabetes, digestive and liver diseases, kidney diseases, weight control and nutrition, urologic diseases, endocrine and metabolic diseases, ...

  15. Kidney-Pancreas Transplant

    ... day or so in the intensive care unit (ICU) for close watching to make sure both kidney ... worker at your center for information about the rehabilitation services provided through your state's Department of Vocational ...

  16. Diabetes and Kidney Disease

    ... NKF Newsroom Contact Us You are here Home » Diabetes - A Major Risk Factor for Kidney Disease Diabetes ... of your body. Are there different types of diabetes? The most common ones are Type 1 and ...

  17. Diet - chronic kidney disease

    ... this special diet to limit the buildup of waste products in the body. Limiting fluids between dialysis ... up when the kidneys no longer function well. Dangerous heart rhythms may result, which can lead to ...

  18. Kidney Stones in Adults

    ... may also help prevent kidney stones, such as orange juice or lemonade. Talk with your health care ... perform a physical exam and take a medical history. The health care provider may perform urine, blood, ...

  19. Kidney Disease and Diabetes

    ... Blood Pressure Tools & Resources Stroke More Kidney Disease & Diabetes Updated:Jan 26,2016 One of the more ... thereafter.) This content was last reviewed January 2016. Diabetes • Home • About Diabetes • Why Diabetes Matters Introduction Cardiovascular ...

  20. Sulfadiazine for kidney disease

    Rucker, R.R.; Bernier, A.F.; Whipple, W.J.; Burrows, R.E.

    1951-01-01

    The blueback salmon fingerlings (Oncorhynchus nerka) at the U.S. Fish-Cultural Station at Winthrop, Washington, underwent an infection that was caused by a very short, Gram-positive, nonmotile, rod-shaped bacterium. A further description is impossible at this time, as the organism has not been grown satisfactorily for proper identification. The disease was characterized by white, raised areas of dead tissue mainly in the kidney: for this reason it is referred to as kidney disease. Belding and Merrill (1935) described a disease among the brook, brown, and rainbow trout at a State hatchery in Massachusetts which, from the description, might be the same as kidney disease. J.H. Wales of the California Division of Fish and Game described (unpublished manuscript, 1941) a disease in hatchery trout in California which seems to be identical to kidney disease.

  1. Exophytic benign mixed epithelial stromal tumour of the kidney: case report of a rare tumour entity

    Küster Jens

    2010-03-01

    Full Text Available Abstract Background Mixed epithelial and stromal tumour (MEST represents a recently described benign composite neoplasm of the kidney, which predominantly affects perimenopausal females. Most tumours are benign, although rare malignant cases have been observed. Case report A 47-year-old postmenopausal female presented to the urologist with flank pain. A CT scan of the abdomen showed a 30-mm-in-diameter uniform mass adjacent to the pelvis of the left kidney. Surgical exploration showed a tumour arising from the lower anterior hilus of the left kidney. The tumour could be excised by preserving the kidney. By intraoperative frozen section the tumour showed characteristic features of MEST with epithelial-covered cysts embedded in an "ovarian-like" stroma. Additional immunohistochemistry investigations showed expression for hormone receptors by the stromal component of the tumour. Discussion MEST typically presents in perimenopausal women as a primarily cystic mass. Commonly, the tumour arises from the renal parenchyma or pelvis. The tumour is composed of an admixture of cystic and sometimes more solid areas. The stromal cells typically demonstrate an ovarian-type stroma showing expression for the estrogen and progesterone receptors. Conclusion MEST represents a distinctive benign tumour entity of the kidney, which affects perimenopausal woman. The tumour should be distinguished from other cystic renal neoplasms. By imaging studies it is difficult to distinguish between a benign or malignant nature of the tumour. Thus, intraoperative frozen section is necessary for conservative surgery, since the overall prognosis is favourable and renal function can be preserved in most cases.

  2. Role Of Various Radiologic Methods In Differentiation Of Pediatric Renal Cystic Diseases

    A. Hekmatnia

    2005-08-01

    Full Text Available Introduction & Background: Renal cystic diseases include a wide spectrum of systemic and local renal dis-eases,which may be either congenital or acquired. Although the Potter classification does not include all of these disorders, it is still commonly used. Precise diagnosis of the above diseases in the neonatal period is valu-able for preventing irreversible and permanent side effects. Ultrasonography as the first diagnostic tool can help the physicians estimate the severity of disease and helps differential diagnosis. Ultrasonographic findings also determine the best methods for patients follow up.

  3. Pediatric Kidney Transplantation 2011

    N. K. Kanzelmeyer; Lehner, F; Pape, L.

    2011-01-01

    As recently as 50 years ago, children suffering from renal insuffiency were dying due to a lack of adequate treatment. Nowadays providing different kinds a dialysis modalities, e. g. peritoneal dialysis and hemodialysis, as well as kidney transplantation have nearly become routine therapeutic options in children. Today, dialysis treatment or kidney transplantation can even be performed in infants.Patient survival of 20 years and near-normal mental and physical development is observed in more ...

  4. Cystic dysplasia of the epididymis: a disorder of mesonephric differentiation associated with renal maldevelopment.

    Nistal, Manuel; González-Peramato, Pilar; Sousa, Grevelyn; García-Cabezas, Miguel Angel; Rodríguez, José Ignacio; Cajaiba, Mariana M

    2010-06-01

    The occurrence of congenital epididymal malformations with a cystic component has not been fully characterized. Most epididymal cysts occur later in life and are likely acquired. In addition, congenital malformations of the male excretory system are extremely uncommon in fetuses and neonates, and epididymal dysplastic changes have not been reported in these cases. In this study, we report 20 cases (including 19 fetal/neonatal autopsies and one surgical specimen from an older child) showing the same spectrum of histological findings in the epididymis, characterized by cystic ductal dilation with dysplastic ducts of variable diameters and irregular shapes, with ill-defined walls. Efferent ductules also showed dysplastic features. In addition, 18 cases had either renal and/or urinary tract anomalies, including renal dysplasia (eight), pelvicaliceal dilation (eight), renal agenesis (four) and hypoplasia (one), ureteral agenesis (two) and hypoplasia (one), urethra and bladder agenesis (two), prostate agenesis (two), and autosomal recessive polycystic renal disease (two). Our observations led to the recognition of a peculiar, not previously described congenital lesion of the epididymis, and we propose the term cystic dysplasia of the epididymis for this anomaly. Similar to what is observed in other male genital system anomalies (including malformations of the rete testis, vas deferens, and seminal vesicles), most lesions occurred in association with renal and/or urinary tract malformations, suggesting a spectrum of congenital malformations. The shared embryological origin of these structures may explain their simultaneous occurrence, possibly related to disrupted mesonephric duct development. PMID:20361206

  5. Constitutive renal Rel/nuclear factor-κB expression in Lewis polycystic kidney disease rats

    Ta, Michelle H T; Schwensen, Kristina G; Liuwantara, David; Huso, David L; Watnick, Terry; Rangan, Gopala K

    2016-01-01

    AIM: To determine the temporal expression and pattern of Rel/nuclear factor (NF)-κB proteins in renal tissue in polycystic kidney disease (PKD). METHODS: The renal expression of Rel/NF-κB proteins was determined by immunohistochemistry, immunofluorescence and immunoblot analysis in Lewis polycystic kidney rats (LPK, a genetic ortholog of human nephronopthsis-9) from postnatal weeks 3 to 20. At each timepoint, renal disease progression and the mRNA expression of NF-κB-dependent genes (TNFα and CCL2) were determined. NF-κB was also histologically assessed in human PKD tissue. RESULTS: Progressive kidney enlargement in LPK rats was accompanied by increased renal cell proliferation and interstitial monocyte accumulation (peaking at weeks 3 and 10 respectively), and progressive interstitial fibrosis (with α smooth muscle actin and Sirius Red deposition significantly increased compared to Lewis kidneys from weeks 3 to 6 onwards). Rel/NF-κB proteins (phosphorylated-p105, p65, p50, c-Rel and RelB) were expressed in cystic epithelial cells (CECs) of LPK kidneys as early as postnatal week 3 and sustained until late-stage disease at week 20. From weeks 10 to 20, nuclear p65, p50, RelB and cytoplasmic IκBα protein levels, and TNFα and CCL2 expression, were upregulated in LPK compared to Lewis kidneys. NF-κB proteins were consistently expressed in CECs of human PKD. The DNA damage marker γ-H2AX was also identified in the CECs of LPK and human polycystic kidneys. CONCLUSION: Several NF-κB proteins are consistently expressed in CECs in human and experimental PKD. These data suggest that the upregulation of both the canonical and non-canonical pathways of NF-κB signaling may be a constitutive and early pathological feature of cystic renal diseases. PMID:27458563

  6. Diffusion-weighted magnetic resonance imaging in evaluation of primary solid and cystic renal masses using the Bosniak classification

    Purpose: Our purpose was to determine whether quantitative diffusion-weighted MR imaging (DWI) could be used in discrimination of benign and malignant primary solid and cystic renal tumors. Materials and methods: A total of 105 consecutive patients with renal masses and 30 healthy controls were enrolled in this prospective study. Dynamic contrast enhanced routin renal images and DWI (with b factors of 0, 500 and 1000 s/mm2) was performed at 1.5 T unit. Renal masses were divided into two groups as cystic or solid and all cystic lesions were prospectively assigned to a Bosniak classification number. The median apparent diffusion coefficient (ADC) values along with b 500 and 1000 signal intensities of normal kidneys, solid components of mixed renal masses and total of cystic lesions were calculated. Results: The mean ADC value of normal renal parenchyma in control group was 2.18 ± 0.13 × 10−3 mm2/s. Solid renal tumors had significant lower ADC values (median: 1.16 ± 0.27 × 10−3 mm2/s), in contrast to cystic tumors (median: 2.73 ± 0.44 × 10−3 mm2/s). The mean ADC value of the Bosniak Category I cysts was significantly higher (3.09 ± 0.14 × 10−3 mm2/s) than normal renal parenchyma (p −3 mm2/s) was significantly higher than that of papillary cell carcinoma (0.90 ± 0.16 × 10−3 mm2/s) and clear cell carcinoma (1.23 ± 0.13 × 10−3 mm2/s). Conclusion: Accurate assessment of renal masses is important for establishing whether tumors require surgical intervention or not. While MRI is a useful modality as an investigative tool for diagnosing, characterizing and staging renal masses, DWI contributes additional value by promising differentiation benign from malignant renal tumors, even histologically subtyping of renal cell cancer.

  7. ADAM17 promotes proliferation of collecting duct kidney epithelial cells through ERK activation and increased glycolysis in polycystic kidney disease.

    Beck Gooz, Monika; Maldonado, Eduardo N; Dang, Yujing; Amria, May Y; Higashiyama, Shigeki; Abboud, Hanna E; Lemasters, John J; Bell, P Darwin

    2014-09-01

    Polycystic kidney disease (PKD) is a common genetic disorder leading to cyst formation in the kidneys and other organs that ultimately results in kidney failure and death. Currently, there is no therapy for slowing down or stopping the progression of PKD. In this study, we identified the disintegrin metalloenzyme 17 (ADAM17) as a key regulator of cell proliferation in kidney tissues of conditional knockout Ift88(-/-) mice and collecting duct epithelial cells from Ift88°(rpk) mice, animal models of autosomal recessive polycystic kidney disease (ARPKD). Using Western blotting, an enzyme activity assay, and a growth factor-shedding assay in the presence or absence of the specific ADAM17 inhibitor TMI-005, we show that increased expression and activation of ADAM17 in the cystic kidney and in collecting duct epithelial cells originating from the Ift88°(rpk) mice (designated as PKD cells) lead to constitutive shedding of several growth factors, including heparin-binding EGF-like growth factor (HB-EGF), amphiregulin, and transforming growth factor-α (TGF-α). Increased growth factor shedding induces activation of the EGFR/MAPK/ERK pathway and maintains higher cell proliferation rate in PKD cells compared with control cells. PKD cells also displayed increased lactate formation and extracellular acidification indicative of aerobic glycolysis (Warburg effect), which was blocked by ADAM17 inhibition. We propose that ADAM17 is a key promoter of cellular proliferation in PKD cells by activating the EGFR/ERK axis and a proproliferative glycolytic phenotype. PMID:24899059

  8. Improved Structure and Function in Autosomal Recessive Polycystic Rat Kidneys with Renal Tubular Cell Therapy.

    K J Kelly

    Full Text Available Autosomal recessive polycystic kidney disease is a truly catastrophic monogenetic disease, causing death and end stage renal disease in neonates and children. Using PCK female rats, an orthologous model of autosomal recessive polycystic kidney disease harboring mutant Pkhd1, we tested the hypothesis that intravenous renal cell transplantation with normal Sprague Dawley male kidney cells would improve the polycystic kidney disease phenotype. Cytotherapy with renal cells expressing wild type Pkhd1 and tubulogenic serum amyloid A1 had powerful and sustained beneficial effects on renal function and structure in the polycystic kidney disease model. Donor cell engraftment and both mutant and wild type Pkhd1 were found in treated but not control PCK kidneys 15 weeks after the final cell infusion. To examine the mechanisms of global protection with a small number of transplanted cells, we tested the hypothesis that exosomes derived from normal Sprague Dawley cells can limit the cystic phenotype of PCK recipient cells. We found that renal exosomes originating from normal Sprague Dawley cells carried and transferred wild type Pkhd1 mRNA to PCK cells in vivo and in vitro and restricted cyst formation by cultured PCK cells. The results indicate that transplantation with renal cells containing wild type Pkhd1 improves renal structure and function in autosomal recessive polycystic kidney disease and may provide an intra-renal supply of normal Pkhd1 mRNA.

  9. Acquiring taste in home economics?

    Stenbak Larsen, Christian

    2015-01-01

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

  10. Ablation and Other Local Therapy for Kidney Cancer

    ... for kidney cancer Targeted therapies for kidney cancer Biologic therapy (immunotherapy) for kidney cancer Chemotherapy for kidney cancer Pain control for kidney cancer Treatment choices by stage for ...

  11. Multi-modality imaging review of congenital abnormalities of kidney and upper urinary tract.

    Ramanathan, Subramaniyan; Kumar, Devendra; Khanna, Maneesh; Al Heidous, Mahmoud; Sheikh, Adnan; Virmani, Vivek; Palaniappan, Yegu

    2016-02-28

    Congenital abnormalities of the kidney and urinary tract (CAKUT) include a wide range of abnormalities ranging from asymptomatic ectopic kidneys to life threatening renal agenesis (bilateral). Many of them are detected in the antenatal or immediate postnatal with a significant proportion identified in the adult population with varying degree of severity. CAKUT can be classified on embryological basis in to abnormalities in the renal parenchymal development, aberrant embryonic migration and abnormalities of the collecting system. Renal parenchymal abnormalities include multi cystic dysplastic kidneys, renal hypoplasia, number (agenesis or supernumerary), shape and cystic renal diseases. Aberrant embryonic migration encompasses abnormal location and fusion anomalies. Collecting system abnormalities include duplex kidneys and Pelvi ureteric junction obstruction. Ultrasonography (US) is typically the first imaging performed as it is easily available, non-invasive and radiation free used both antenatally and postnatally. Computed tomography (CT) and magnetic resonance imaging (MRI) are useful to confirm the ultrasound detected abnormality, detection of complex malformations, demonstration of collecting system and vascular anatomy and more importantly for early detection of complications like renal calculi, infection and malignancies. As CAKUT are one of the leading causes of end stage renal disease, it is important for the radiologists to be familiar with the varying imaging appearances of CAKUT on US, CT and MRI, thereby helping in prompt diagnosis and optimal management. PMID:26981222

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

    Kenichi Hirabayashi

    2008-03-01

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

  13. Cystic schwannoma of the pancreas: a case report

    Kim, Hee Jung; Lee, Won Jae; Kim, Sung Mok; Jang, Kee Taek [Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul (Korea, Republic of)

    2008-05-15

    A pancreatic schwannoma is an extremely rare pancreatic neoplasm. This tumor can vary in size, and can be variably cystic such that lesion mimics the common cystic tumor of the pancreas. We report a case of a 73-year-old woman with a surgically proven cystic schwannoma of the pancreas. CT images showed the presence of a well-defined, cystic mass in the pancreatic head area, which contained solid, enhancing areas. Furthermore, multi-planar reformatted images provided additional information by demonstrating the intrapancreatic location of the mass. According to our experience a cystic schwannoma of the pancreas should be considered in the differential diagnosis of a cystic tumor of the pancreas.

  14. Cystic schwannoma of the pancreas: a case report

    A pancreatic schwannoma is an extremely rare pancreatic neoplasm. This tumor can vary in size, and can be variably cystic such that lesion mimics the common cystic tumor of the pancreas. We report a case of a 73-year-old woman with a surgically proven cystic schwannoma of the pancreas. CT images showed the presence of a well-defined, cystic mass in the pancreatic head area, which contained solid, enhancing areas. Furthermore, multi-planar reformatted images provided additional information by demonstrating the intrapancreatic location of the mass. According to our experience a cystic schwannoma of the pancreas should be considered in the differential diagnosis of a cystic tumor of the pancreas

  15. The value of MRI in diagnosis of ovarian cystic teratoma

    Objective: To study the MRI manifestations and diagnostic values in ovarian cystic teratoma. Methods: MRI appearances of 52 cases of ovarian cystic teratoma confirmed by surgery and pathology were analyzed retrospectively. Results: All of 52 cases were confirmed as mature cystic teratoma by pathology. Of the 52 cases, 43 were unilateral, 9 cases bilateral. And 61 lesions were totally found. The diameter of lesions was 12.8 cm-2.7 cm. The appearance of MRI was mainly cystic. The cystic area of the lesion showed low signal intensity on T1WI and high signal intensity on T2WI. The wall of the cyst was smooth and enhanced after contrast medium administration. MRI revealed that 52 cases were mixed intensity and containing variety of fat signal intensity area. The accuracy of MRI diagnosis in ovarian cystic teratoma was 100%. Conclusion: Ovarian teratoma has characteristic appearances in MRI. Pelvic MRI examination for ovarian teratoma can make exact diagnosis. (authors)

  16. Computed tomographic evaluation of pancreatic and peripancreatic cystic mass

    Forty-four cases of pancreatic cystic mass (pancreatic pseudocyst 37, pseudocyst caused by pancreatic carcinoma 2, mucous producing caner 2, cystadenoma 1, cystadenocarcinoma 2, and 16 cases of peripancreatic cystic mass (intestinal leiomyosarcoma 10, lymphonode metastasis 3, mesenteric hematoma 1, carcinoma of Vater's papilla 1, duplicating cyst of duodenum 1) were evaluated by computed tomography during the period from April 1978 to December 1984 at Fujita-Gakuen Health University Hospital. The most common cystic masses of pancreatic and peripancreatic lesion were pseudocyst. Although there are many cystic masses mimicking a pancreatic pseudocyst in pancreatic region, sometimes it is impossible to distinguish between cystic mass arising in the pancreas and the adjuvant organ. Difficulties in the computed tomographic differential diagnosis between pancreatic and peripancreatic cystic mass are analyzed and discussed in this paper. (author)

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

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

    2014-12-01

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

  18. The cystic form of rheumatoid arthritis

    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.)

  19. Cystic sacrococcygeal teratoma-case report

    A 21 days old female was brought for abdominal distension, constipation and vomiting of 8 days duration. She was the product of vaginal delivery with an uneventfull post natal history. A plain film of the abdomen shows a soft density filling the pelvis and the lower abdomen. An ultrasound examination of the abdomen and pelvis reveals a markedly distended bladder and a bilateral hydronephrosis. Following the catheterization of the bladder, there remained a cystic lesion with septations in the lower part of the pelvis. A CT examination done with oral and intravenous contrast medium confirms the presence of the cystic lesion.The differential diagnosis of this lesion includes sacrococcygeal teratoma, ovarian cyst and meningomyelocele

  20. European Cystic Fibrosis Society Standards of Care

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

    2014-01-01

    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......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....

  1. Magnetic resonance imaging of ovarian cystic tumor

    Seventeen cases with cystic ovarian tumors were examined with both magnetic resonance imaging (MRI) and CT scanning. The contents of the cysts were analyzed as for protein, fat, and iron. The intensity patterns of the cystic lesion were not directly related to the amount of protein, fat, nor iron. But an for the cystadenomas, protein concentration are the most responsible for their intensities, and as for the dermoid cysts, fat are the most responsible for. And in endemetrial cysts, it seems that variable amount of protein, fat, and iron make the unique intensity patterns. MRI was superior to CT in characterizing cyts especially for cystadenoma and endmetorial cysts. And with much more case studies, relationship between protein concentration and intensities will be proved. (author)

  2. Mature cystic Theratome. Presentation of a case

    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

  3. Diffuse Cystic Lung Diseases: Diagnostic Considerations.

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

    2016-06-01

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

  4. Diffuse Cystic Lung Disease. Part II.

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

    2015-07-01

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

  5. Diffuse Cystic Lung Disease. Part I.

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

    2015-06-15

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

  6. Supratentorial cystic intracranial lesions: MR imaging features

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

    1997-01-01

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

  7. Exacerbations in cystic fibrosis: 2 · Prevention

    Bell, Scott C; Robinson, Philip J

    2007-01-01

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

  8. Cystic Fibrosis in a Hispanic Adolescent

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

    2013-01-01

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

  9. MR imaging of pancreas in cystic fibrosis

    The pancreatic regions of 18 patients with cystic fibrosis were analyzed with a 1.5 Tesla MR unit. Signal intensity of the pancreas was correlated with clinical data and ultrasound. A hyperintense pancreas on T1-weighted image was consistent with fatty replacement of pancreatic insufficiency. A pancreas of normal soft tissue intensity was found in two asymptomatic and one symptomatic patient. A very hypointense pancreas on any pulse sequence was considered to be an intermediate stage of pancreatic degeneration. (orig.)

  10. Reversible airway obstruction in cystic fibrosis.

    Ormerod, L P; Thomson, R A; Anderson, C. M.; Stableforth, D. E.

    1980-01-01

    Fourteen (29%) of 48 children with cystic fibrosis had a greater than 15% improvement in forced expiratory volume in one second, or in forced vital capacity after inhalation of salbutamol. All these children were atopic (one or more positive prick tests) and had a significantly higher mean serum IgE than either non-atopic subjects or those atopic subjects without airways reversibility (p less than 0.02). Half of those with airways reversibility had or subsequently developed the clinical pictu...

  11. Vitamin D Deficiency in Cystic Fibrosis

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

    2010-01-01

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

  12. Posterior midline cervical fetal cystic hygroma.

    Oak S

    1992-04-01

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

  13. Sebaceous Carcinoma Arising in Mature Cystic Teratoma of Ovary

    An, Hyo Jeong; Jung, Yong Han; Yoon, Hye Kyoung; Jung, Soo Jin

    2013-01-01

    Roughly 1% of mature cystic teratomas undergo malignant transformation. In particular, cutaneous-type adnexal neoplasms may occur in mature cystic teratomas. Sebaceous carcinomas, which arise from mature cystic teratomas, have rarely been observed, with only seven cases previously reported. Here, we present a case of a 69-year-old female who had pelvic pain for two weeks and who subsequently underwent bilateral salpingo-oophorectomy and hysterectomy. Her left ovary showed a unilocular cyst, m...

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

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

    2009-01-01

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

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

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

    2012-01-01

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

  16. Pulmonary abnormalities in obligate heterozygotes for cystic fibrosis.

    Davis, P B; Vargo, K

    1987-01-01

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

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

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

    2007-01-01

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

  18. A cystic hygroma case associated with Turner’s syndrome

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

    1997-01-01

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

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

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

    2014-01-01

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

  20. Acquired Aplastic Anemia in Children

    Hartung, Helge D.; Olson, Timothy S.; Bessler, Monica

    2013-01-01

    This article provides a practice-based and concise review of the etiology, diagnosis, and management of acquired aplastic anemia in children. Bone marrow transplantation, immunosuppressive therapy, and supportive care are discussed in detail. The aim is to provide the clinician with a better understanding of the disease and to offer guidelines for the management of children with this uncommon yet serious disorder.

  1. Acquired anhidrosis a case report

    Nair Laxmi

    1992-01-01

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

  2. Acquired anhidrosis a case report

    Nair Laxmi; Beena D; Manohar S

    1992-01-01

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

  3. [Molecular epidemiology of cystic fibrosis in Tunisia].

    Messaoud, T; Bel Haj Fredj, S; Bibi, A; Elion, J; Férec, C; Fattoum, S

    2005-01-01

    Cystic fibrosis is the most frequent autosomal recessive genetic disease in North European population. This pathology seems to not be rare in Tunisia. On another hand, development of molecular biology techniques has largely contributed to implement the study of the different mutations in the CFTR gene where over 1,300 mutations were reported. Herein, we describe the strategy used to detect molecular defects responsible of cystic fibrosis on 390 children (383 families) in Tunisian population. Several techniques were performed for genotype diagnosis: DNA extraction was from peripheral blood. Polymerase chain reaction (PCR) and polyacylamide gel electrophoresis, and reverse dot blot procedures were used to detect known point mutations. Denaturant gradient gel electrophoresis (DGGE) were used in a next step searching for the unknown point mutations that are later identified by automated sequencing on ABIprism 310. This strategy allowed us to detect 17 different mutations located on the different exons of the CFTR gene. The most frequent was the F508del (50.74%) followed by three other mutations (G542X, W1282X and N1303K) known to be common in the Mediterranean area. For mutations (T665S, 2766 del8, F1166C, L1043R) were exclusively found, up to now, in the Tunisian population. Our results permitted to establish cystic fibrosis mutations and their distribution in Tunisia and to implement an appropriate prevention program of these diseases through the genetic council and prenatal diagnosis. PMID:16330381

  4. Progress in therapies for cystic fibrosis.

    De Boeck, Kris; Amaral, Margarida D

    2016-08-01

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

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

    Braun, C; Bacchetta, J; Reix, P

    2016-08-01

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

  6. Bilateral cystic neuroblastoma: imaging features and differential diagnoses

    Neuroblastoma is one of the most common malignant tumors of childhood, with 40 % arising in the adrenal glands. Bilateral adrenal involvement from synchronous development or metastatic spread of the tumor is seen in less than 10 % of children with neuroblastoma [1[. Neuroblastoma rarely presents as a cystic suprarenal mass that is difficult to differentiate from adrenal hemorrhage, extralobar sequestration, or dilated upper-pole renal calyces. To our knowledge, bilateral cystic neuroblastoma has not been previously reported. We present a case of bilateral cystic adrenal neuroblastoma to demonstrate the imaging features of this unusual entity, and to expand the differential diagnosis of bilateral cystic suprarenal masses in an infant. (orig.). With 2 figs

  7. Occlusion of the cystic duct by electrocoagulation: A radiologic technique

    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

  8. Isolated type I pelvic cystic echinococcosis mimicking ovarian tumor

    Mehmet Vural

    2011-01-01

    Full Text Available Context : Cystic echinococcosis is an endemic infestation with unique clinical and laboratory manifestations. Isolated pelvic type 1 cystic echinococcosis is a rare form of the disease with diagnostic pitfalls mainly based on non-diagnostic imaging findings. Case Report : We present an isolated pelvic cystic echinococcosis resembling ovarian tumor which was diagnosed during operation. Conclusions : Characteristic findings of hydatid disease lacks in type 1 and cause diagnostic difficulties. This rare entity should be considered for differential diagnosis due to varied examination findings especially in type 1 cystic echinococcosis.

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

    Hays, Steven R.; John V Fahy

    2006-01-01

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

  10. Genetic modifiers of nutritional status in cystic fibrosis1234

    Bradley, Gia M; Blackman, Scott M.; Christopher P Watson; Doshi, Vishal K.; Cutting, Garry R

    2012-01-01

    Background: Improved nutrition early in life is associated with better pulmonary function for patients with cystic fibrosis (CF). However, nutritional status is poorly correlated with the CFTR genotype.

  11. Berberine slows cell growth in autosomal dominant polycystic kidney disease cells

    Bonon, Anna; Mangolini, Alessandra [Department of Biomedical and Specialty Surgical Sciences, University of Ferrara, 44121 Ferrara (Italy); Pinton, Paolo [Department of Morphology, Surgery and Experimental Medicine, Section of General Pathology, University of Ferrara, 44121 Ferrara (Italy); Senno, Laura del [Department of Biomedical and Specialty Surgical Sciences, University of Ferrara, 44121 Ferrara (Italy); Aguiari, Gianluca, E-mail: dsn@unife.it [Department of Biomedical and Specialty Surgical Sciences, University of Ferrara, 44121 Ferrara (Italy)

    2013-11-22

    Highlights: •Berberine at appropriate doses slows cell proliferation in ADPKD cystic cells. •Reduction of cell growth by berberine occurs by inhibition of ERK and p70-S6 kinase. •Higher doses of berberine cause an overall cytotoxic effect. •Berberine overdose induces apoptotic bodies formation and DNA fragmentation. •Antiproliferative properties of this drug make it a new candidate for ADPKD therapy. -- Abstract: Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary monogenic disorder characterized by development and enlargement of kidney cysts that lead to loss of renal function. It is caused by mutations in two genes (PKD1 and PKD2) encoding for polycystin-1 and polycystin-2 proteins which regulate different signals including cAMP, mTOR and EGFR pathways. Abnormal activation of these signals following PC1 or PC2 loss of function causes an increased cell proliferation which is a typical hallmark of this disease. Despite the promising findings obtained in animal models with targeted inhibitors able to reduce cystic cell growth, currently, no specific approved therapy for ADPKD is available. Therefore, the research of new more effective molecules could be crucial for the treatment of this severe pathology. In this regard, we have studied the effect of berberine, an isoquinoline quaternary alkaloid, on cell proliferation and apoptosis in human and mouse ADPKD cystic cell lines. Berberine treatment slows cell proliferation of ADPKD cystic cells in a dose-dependent manner and at high doses (100 μg/mL) it induces cell death in cystic cells as well as in normal kidney tubule cells. However, at 10 μg/mL, berberine reduces cell growth in ADPKD cystic cells only enhancing G{sub 0}/G{sub 1} phase of cell cycle and inhibiting ERK and p70-S6 kinases. Our results indicate that berberine shows a selected antiproliferative activity in cellular models for ADPKD, suggesting that this molecule and similar natural compounds could open new

  12. A mouse model for polycystic kidney disease through a somatic in-frame deletion in the 5' end of Pkd1.

    Starremans, P G; Li, X; Finnerty, P E; Guo, L; Takakura, A; Neilson, E G; Zhou, J

    2008-06-01

    Autosomal dominant polycystic kidney disease, a leading cause of end-stage renal disease in adults, is characterized by progressive focal cyst formation in the kidney. Embryonic lethality of Pkd1-targeted mice limits the use of these mice. Here we developed a floxed allele of Pkd1 exons 2-6. Global deletion mutants developed polyhydramnios, hydrops fetalis, polycystic kidney and pancreatic disease. Somatic Pkd1 inactivation in the kidney was achieved by crossing Pkd1(flox) mice with transgenic mice expressing Cre controlled by a gamma-glutamyltranspeptidase promoter. These mutants developed cysts in both proximal and distal nephron segments and survived for about 4 weeks. Somatic loss of heterozygosity was shown in a reporter mouse strain to cause cystogenesis. Some cysts in young mice are positive for multiple tubular markers and a mesenchymal marker, suggesting a delay in tubular epithelial differentiation. A higher cell proliferation rate was observed in distal nephron segments probably accounting for the faster growth rate of distal cysts. Although we observed an overall increase in apoptosis in cystic kidneys, there was no difference between proximal or distal nephron segments. We also found increased cyclic AMP, aquaporin 2 and vasopressin type 2 receptor mRNA levels, and apical membrane translocation of aquaporin 2 in cystic kidneys, all of which may contribute to the differential cyst growth rate observed. The accelerated polycystic kidney phenotype of these mice provides an excellent model for studying molecular pathways of cystogenesis and to test therapeutic strategies. PMID:18385665

  13. Kidney Stones and the Risk for Chronic Kidney Disease

    Rule, Andrew D.; Bergstralh, Eric J.; Melton, L. Joseph; Li, Xujian; Amy L. Weaver; Lieske, John C.

    2009-01-01

    Background and objectives: Kidney stones lead to chronic kidney disease (CKD) in people with rare hereditary disorders (e.g., primary hyperoxaluria, cystinuria), but it is unknown whether kidney stones are an important risk factor for CKD in the general population.

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

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

    2010-01-01

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

  15. Renal (Kidney) Manifestations in TSC

    Full Text Available ... transplantation would be indicated. Renal Cell Carcinoma (Kidney Cancer) Over the past 20 years, there have been at least 25 published reports of kidney cancer occurring in individuals with TSC. Drs. Bjornsson, Short, ...

  16. Living Donor Kidney Transplant Surgery

    Full Text Available ... here and right kidney here. And we generally use the left kidney because the vein for the ... of the renal vein. We’re going to use this ligature device, again, to basically heat this ...

  17. Renal (Kidney) Manifestations in TSC

    Full Text Available ... kidney disease can develop in infancy or early childhood and renal failure most often occurs in early ... should be performed by a team with TSC experience, many individuals with TSC can maintain normal kidney ...

  18. Flu Season and Your Kidneys

    ... Sign up for our FREE magazine, Kidney Living Organ Donation & Transplantation Be an Organ Donor Living Donation Donor ... Giving Primary menu Home Prevention Kidney Disease Patients Organ Donation & Transplantation Professionals Events Advocacy Donate Search Search Header ...

  19. Kidney Stone Treatment with Lithotripsy

    Full Text Available ... year-old white gentleman who's got at least three stones in his left kidney. He's been having ... patient with Lithotripsy. Now the stones -- there are three stones here in the pelvis of the kidney, ...

  20. National Kidney Disease Education Program

    ... campuses in Maryland and Arizona Research Resources Protocols, repositories, mouse models, plasmids, and more Technology Advancement & Transfer ... chronic kidney disease Pharmacy Working Group Increase pharmacists' knowledge of chronic kidney disease (CKD) and provide them ...

  1. Staying Fit with Kidney Disease

    ... Sign up for our FREE magazine, Kidney Living Organ Donation & Transplantation Be an Organ Donor Living Donation Donor ... Giving Primary menu Home Prevention Kidney Disease Patients Organ Donation & Transplantation Professionals Events Advocacy Donate Search Search Header ...

  2. Renal (Kidney) Manifestations in TSC

    Full Text Available ... with cysts. If kidney failure occurs, renal replacement therapy such as dialysis or transplantation is necessary. How kidney cysts develop is not known. The TSC genes are tumor suppressor genes. Normally, tumor suppressor genes ...

  3. Living Donor Kidney Transplant Surgery

    Full Text Available ... prior to putting the patient on the immunosuppressive medicines to take care of their new kidney. But ... Absolutely. All of our patients take three immunosuppressive drugs to keep them from rejecting the kidney. Even ...

  4. Screening for Chronic Kidney Disease

    Understanding Task Force Recommendations Screening for Chronic Kidney Disease The U.S. Preventive Services Task Force (Task Force) has issued a final recommendation on Screening for Chronic Kidney Disease (CKD) . This recommendation ...

  5. Renal (Kidney) Manifestations in TSC

    Full Text Available ... Get Involved RENAL (KIDNEY) MANIFESTATIONS IN TSC Download a PDF of this information. The majority of individuals ( ... the least common renal association with TSC, is a cancerous growth of the kidney. Although it is ...

  6. Living Donor Kidney Transplant Surgery

    Full Text Available ... hospitals in the America for patients with kidney disease, and only one other hospital in the State ... as diabetes, which in turn can cause kidney disease, but also because of risks perioperatively such as ...

  7. Renal (Kidney) Manifestations in TSC

    Full Text Available ... the kidneys are filled with cysts. If kidney failure occurs, renal replacement therapy such as dialysis or transplantation is ... can develop in infancy or early childhood and renal failure most often occurs in early adulthood. Renal Angiomyolipomas ...

  8. Aging changes in the kidneys

    ... this page: //medlineplus.gov/ency/article/004010.htm Aging changes in the kidneys and bladder To use ... in the reproductive system can affect bladder control. Aging Changes and Their Effects on the Kidneys and ...

  9. Living Donor Kidney Transplant Surgery

    Full Text Available ... problems such as diabetes, which in turn can cause kidney disease, but also because of risks perioperatively ... the diseased kidneys really don’t tend to cause any problem. They actually get smaller and smaller ...

  10. Living Donor Kidney Transplant Surgery

    Full Text Available ... Absolutely. All of our patients take three immunosuppressive drugs to keep them from rejecting the kidney. Even ... the recipient that the recipient could have fewer drugs to take to keep from rejecting the kidney. ...

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

    Cleveland Robert H

    2010-04-01

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

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

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

    2012-01-01

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

  13. Simple Kidney Cysts

    ... ended or closed at one end; some newer machines are designed to allow the person to lie in a more open space. Like CT scans, MRIs can show cysts and tumors. [ Top ] How are simple kidney cysts treated? Treatment is not needed for ...

  14. Kidneys and urinary system

    Nuclear medicine studies, through primarily concerned with the functional aspects of the organ, can also provide useful information about the anatomy. An understanding of the anatomy and physiology of the kidneys and urinary system greatly helps in the interpretation of data from radionuclide studies

  15. KIDNEY AND URINARY TRACT

    2003-01-01

    11.1 Kidney function2003454 Effects of protein kinase C on type 1 inosi-tol 1,4, 5-triphosphate receptor expression in smooth muscle cells of rat glomerular afferent arterioles.WANG Jingyan(王静艳), et al. Dept Infect DLs, 2nd Affili Hosp, China Med Univ, Shenyang 110004. China World J Digestol 2003; (11)6:705-707.

  16. KIDNEY AND URINARY TRACT

    2004-01-01

    11.1 Kidney function2004416 The role of adhesion molecules and dendritic cells in hepatic/renal ischemia-reperfusion injury and the effect of antiadhesive treatment in rats.ZHOU Tong(周同), et al. Dept Nephrol, Ruijing Hosp Shanghai 2nd Med Univ, Shanghai 200025. Chin J Emerg Med 2004; 13(5):315 -318.

  17. Medullary Sponge Kidney

    ... see bones, tissues, and organs inside the body. Health care providers commonly choose one or more of three imaging techniques to diagnose medullary sponge kidney: intravenous pyelogram computerized tomography (CT) scan ultrasound A radiologist—a doctor who specializes in medical imaging—interprets ...

  18. Kidneys and Urinary Tract

    ... more common kidney and urinary tract problems include: Congenital problems of the urinary tract. As a fetus develops in the womb, any part of the urinary tract can grow to an abnormal size or in an abnormal ... congenital abnormalities (meaning abnormalities that exist at birth) is ...

  19. Connexins and the kidney

    Hanner, Fiona; Sørensen, Charlotte Mehlin; Holstein-Rathlou, Niels-Henrik;

    2010-01-01

    Connexins (Cxs) are widely-expressed proteins that form gap junctions in most organs, including the kidney. In the renal vasculature, Cx37, Cx40, Cx43, and Cx45 are expressed, with predominant expression of Cx40 in the endothelial cells and Cx45 in the vascular smooth muscle cells. In the tubules...

  20. Everolimus in kidney transplantation

    Cooper JE

    2011-07-01

    Full Text Available James E Cooper¹, Uwe Christians², Alexander C Wiseman¹¹Division of Renal Diseases and Hypertension, Transplant Center, ²iC42 Integrated Solutions in Systems Biology for Clinical Research and Development, University of Colorado Denver, Aurora, CO, USAAbstract: Everolimus is a novel target of rapamycin (mTOR-I analog that has recently been approved in combination with cyclosporine A and steroids for use in the prevention of organ rejection in kidney transplant recipients. Compared with rapamycin, everolimus is characterized by a shorter half-life and improved bioavailability. Prior to US Food and Drug Administration approval, a number of Phase II and III clinical trials were undertaken to evaluate the effectiveness of everolimus in combination with calcineurin inhibitors for preventing acute rejection and promoting allograft survival in kidney transplant recipients. In this report, we review the pharmacokinetic properties of everolimus, the clinical efficacy studies that led to its approval for use in kidney transplantation, as well as reported data on patient safety and tolerability associated with its use.Keywords: mTOR inhibitors, kidney transplantation, everolimus

  1. Autosomal recessive polycystic kidney disease. A case report.

    Hernando Diocaretz V

    2015-01-01

    Full Text Available INTRODUCTION: Polycystic Kidney Disease is a genetic disorder characterized by progressive cystic dilations of the renal ducts, presenting as autosomal dominant or recessive forms with an incidence of 1 in 1.000 and 1 in 20.000 births, respectively, according to international series. The autosomal recessive variety can be lethal in the neonatal period due to respiratory failure secondary to pulmonary hypoplasia and can manifest during childhood with hypertension, short stature and complications of portal hypertension. CASE REPORT: 3 years and 11 months old preschoolar with antecedent of fetal growth restriction and oligohydramnios during prenatal period, and a history of asthenia, pallor and progressive feeding difficulty with postprandial vomiting. Physical examination shows cardiac bruit, hypertension, splenomegaly, caput medusae and short stature. Laboratory tests with peripheral pancytopenia; abdominal ultrasonography showed hepatosplenomegaly, findings consistent with autosomal recessive polycystic kidney disease and periportal fibrosis; renal scintigraphy with bilateral kidney failure; a positive fecal occult blood test; an upper endoscopy that shows small esophageal varices; a hand radiography that shows bone age delayed and an echocardiography with cardiomegaly. DISCUSSION: This infrequent disease requires a high degree of suspicion by the clinician and presents with portal hypertension, with platelet count being the best predictor of severity. This condition has no cure and will progress to end-stage renal disease in any moment, so the aim is to minimize and treat renal and hepatic complications.

  2. Occupationally Acquired American Cutaneous Leishmaniasis

    Maria Edileuza Felinto de Brito

    2012-01-01

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

  3. CNOOC Acquires Oversea Assets Successfully

    Hu Senlin

    2006-01-01

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

  4. Cogema acquires TOTAL's uranium assets

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

  5. Living Donor Kidney Transplant Surgery

    Full Text Available ... of non-steroidal medications like ibuprofen, which can affect kidney function. And then about four weeks after ... for you? “How does having only one kidney affect a patient’s life? Is kidney function still normal?” ...

  6. Chronic Kidney Disease and Medicines

    ... from our online catalog. Alternate Language URL Español Chronic Kidney Disease and Medicines: What You Need to Know Page ... What you need to know Because you have chronic kidney disease, you should take steps to protect your kidneys. ...

  7. Renal (Kidney) Manifestations in TSC

    Full Text Available ... can arise including hematuria (bloody urine) and nephrolithiasis (kidney stones). Often the best drugs to use to lower the blood pressure in this situation are either angiotensin converting enzyme ... the loss of kidney function when the kidneys are filled with cysts. ...

  8. Is Progressive Chronic Kidney Disease a Slow Acute Kidney Injury?

    Cowgill, Larry D; Polzin, David J; Elliott, Jonathan; Nabity, Mary B; Segev, Gilad; Grauer, Gregory F; Brown, Scott; Langston, Cathy; van Dongen, Astrid M

    2016-11-01

    International Renal Interest Society chronic kidney disease Stage 1 and acute kidney injury Grade I categorizations of kidney disease are often confused or ignored because patients are nonazotemic and generally asymptomatic. Recent evidence suggests these seemingly disparate conditions may be mechanistically linked and interrelated. Active kidney injury biomarkers have the potential to establish a new understanding for traditional views of chronic kidney disease, including its early identification and possible mediators of its progression, which, if validated, would establish a new and sophisticated paradigm for the understanding and approach to the diagnostic evaluation, and treatment of urinary disease in dogs and cats. PMID:27593574

  9. Association between renal cystic lesions and bilateral Wilms' tumours

    Simanovsky, Natalia; Hiller, Nurith [Hadassah-Hebrew University Medical Center at Mt Scopus, Department of Medical Imaging, POB 12000, Jerusalem (Israel); Revel-Vilk, Shoshana; Weintraub, Michael [Hadassah-Hebrew University Medical Center, Department of Pediatric Hematology/ Oncology, Jerusalem (Israel)

    2016-06-15

    Evaluate a potential association between Wilms' tumour (WT) and renal cystic lesions. Digital records and imaging files of consecutive patients diagnosed with WT between 2004 and 2014 were retrospectively reviewed under an Institutional Review Board waiver of informed consent. The locations of renal cysts seen on US, CT, and/or MRI were recorded and compared with the locations of newly developed WT. A total of 48 patients (mean age 3 years 9 months) presented with newly diagnosed WT in the study period. Mean follow-up was 4.5 (range 1-10) years. WT was unilateral in 40 children, bilateral in 8. Renal cysts were identified in only one of the forty patients (2.5 %) with unilateral disease - in the contralateral kidney. In contrast, renal cysts were found in seven of eight patients with bilateral WT (87.5 %), in two of whom, new tumours developed in the same location where cysts had been seen on previous imaging studies. Renal cystic lesions in patients with Wilms' tumour should be regarded as potential tumour precursors, and followed with frequent imaging. (orig.)

  10. Acquired causes of intestinal malabsorption.

    van der Heide, F

    2016-04-01

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

  11. Inhibitors against the coagulation factors spontaneously acquired: Acquired B Hemophilia

    Claudia Lucía Sossa Melo; Sara Inés Jiménez Sanguino; Pilar Rodríguez

    2003-01-01

    Spontaneously acquired inhibitors to factor IX, are extremely rare. A 70-year-old male, presented with major continuous post-orthopedic surgery bleeding. His initial APTT was 77.4 s (normal range, 25-36) and normal PT. Expanded APTT corrects, results in favor of deficit of factor IX, confirming the level of dose of IX factor: 52% (NR 70–125%) with normal factor VIII. It was realized with fresh frozen plasma, and by the fifth day of treatment, he presents a bruise in the surgery bed with radic...

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

    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) and...

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

    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) and...

  14. Kidney-specific inactivation of the Pkd1 gene induces rapid cyst formation in developing kidneys and a slow onset of disease in adult mice.

    Lantinga-van Leeuwen, Irma S; Leonhard, Wouter N; van der Wal, Annemieke; Breuning, Martijn H; de Heer, Emile; Peters, Dorien J M

    2007-12-15

    Autosomal dominant polycystic kidney disease, caused by mutations in the PKD1 gene, is characterized by progressive deterioration of kidney function due to the formation of thousands of cysts leading to kidney failure in mid-life or later. How cysts develop and grow is currently unknown, although extensive research revealed a plethora of cellular changes in cyst lining cells. We have constructed a tamoxifen-inducible, kidney epithelium-specific Pkd1-deletion mouse model. Upon administration of tamoxifen to these mice, a genomic fragment containing exons 2-11 of the Pkd1-gene is specifically deleted in the kidneys and cysts are formed. Interestingly, the timing of Pkd1-deletion has strong effects on the phenotype. At 1 month upon gene disruption, adult mice develop only a very mild cystic phenotype showing some small cysts and dilated tubules. Young mice, however, show massive cyst formation. In these mice, at the moment of gene disruption, cell proliferation takes place to elongate the nephron. Our data indicate that Pkd1 gene deficiency does not initiate sufficient autonomous cell proliferation leading to cyst formation and that additional stimuli are required. Furthermore, we show that one germ-line mutation of Pkd1 is already associated with increased proliferation. PMID:17932118

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

    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.

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

    Fluge, G; Ojeniyi, B; Høiby, N;

    2001-01-01

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

  17. 78 FR 26681 - Medical Criteria for Evaluating Cystic Fibrosis

    2013-05-07

    ... ADMINISTRATION RIN 0960-AF58 Medical Criteria for Evaluating Cystic Fibrosis AGENCY: Social Security... we published in the Federal Register on February 4, 2013 (78 FR 7968). We use Listings 3.04 and 103.04 to evaluate claims involving cystic fibrosis in adults and children under titles II and XVI of...

  18. LUNG TRANSPLANTATION IN PATIENTS WITH CYSTIC-FIBROSIS

    MANNES, GPM; VANDERBIJ, W

    1995-01-01

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

  19. Squamous Cell Carcinoma arising in a Cystic Teratoma

    Ruchi Khajuria, Madalsa Bargotra,Rubey Bhat, V.K.Dubey

    2000-04-01

    Full Text Available Malignant transformation in a benign cystic teratoma occurs in 1-3% of cases. A rare case of squamouscell carcinoma arising in a benign cystic teratoma ofovary in a 37 year old f~male is reported. The patientpresented with increasing abdominal girth and pain abdomen and was cl inically diagnosed having a largeovarian cyst.

  20. Rare cystic liver lesions: a diagnostic and managing challenge.

    Bakoyiannis, Andreas; Delis, Spiros; Triantopoulou, Charina; Dervenis, Christos

    2013-11-21

    Cystic formations within the liver are a frequent finding among populations. Besides the common cystic lesions, like simple liver cysts, rare cystic liver lesions like cystadenocarcinoma should also be considered in the differential diagnosis. Thorough knowledge of each entity's nature and course are key elements to successful treatment. Detailed search in PubMed, Cochrane Database, and international published literature regarding rare cystic liver lesions was carried out. In our research are included not only primary rare lesions like cystadenoma, hydatid cyst, and polycystic liver disease, but also secondary ones like metastasis from gastrointestinal stromal tumors lesions. Up-to date knowledge regarding diagnosis and management of rare cystic liver lesions is provided. A diagnostic and therapeutic algorithm is also proposed. The need for a multidisciplinary approach by a team including radiologists and surgeons familiar with liver cystic entities, diagnostic tools, and treatment modalities is stressed. Patients with cystic liver lesions must be carefully evaluated by a multidisciplinary team, in order to receive the most appropriate treatment, since many cystic liver lesions have a malignant potential and evolution. PMID:24282350