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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 ... 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 ...

  8. 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 ...

  9. 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 ...

  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

    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 ...

  12. 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.

  13. 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 ...

  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 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 ...

  20. 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 ...

  1. 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– ...

  2. 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 ...

  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 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...

  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 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...

  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. 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

  5. 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

  6. 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

  7. 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

  8. 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.)

  9. 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

  10. 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.

  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. 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

  19. 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

  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. 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

  8. 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

  9. 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

  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. 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 ...

  17. 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....

  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. 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.

  6. 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.

  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. 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 ...

  9. 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.)

  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. [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

  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. 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)

  20. 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...

  1. 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...

  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. 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.

  10. 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.)

  11. [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

  12. 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

  13. 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

  14. 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 ...

  15. 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, ...

  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. 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...

  19. 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.

  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

    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.

  10. 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.