Deborah A. Baumgarten

Magnetic Resonance Imaging Evaluation of Hepatic Cysts in Early Autosomal-Dominant Polycystic Kidney Disease: The Consortium for Radiologic Imaging Studies of Polycystic Kidney Disease Cohort

The objective of this study was to investigate the prevalence of hepatic cysts by age and gender in patients with early autosomal-dominant polycystic kidney disease (ADPKD) and to determine whether hepatic cyst volume is related to renal and renal cyst volumes by using magnetic resonance imaging (MRI). A total of 230 patients with ADPKD (94 men and 136 women) who were aged 15 to 46 yr and had relatively preserved renal function were studied. MRI images of the kidney and liver were obtained to measure renal, renal cyst, and hepatic cyst volumes. These volume measurements and hepatic cyst prevalence were compared in all patients and in subgroups on the basis of gender and age (15 to 24, 25 to 34, and 35 to 46 yr). The overall prevalence of hepatic cysts was 83%; the prevalence was 58, 85, and 94% in the sequential age groups and 85% in women and 79% in men. The prevalence was related directly to renal volume (chi2 = 4.30, P = 0.04) and to renal cyst volume (chi2 = 5.59, P = 0.02). The total hepatic cyst volume was significantly greater in women than in men (a logarithmic transformation mean of 5.27 versus 1.94 ml; P = 0.003). The average hepatic cyst volume was 0.25, 5.75, and 22.78 ml in sequential age groups. Hepatic cysts are evident in 94% of patients who are older than 35 yr and in 55% of individuals who are younger than 25 yr. Hepatic cysts are more prevalent and larger in total cyst volume in women than in men. Hepatic cyst prevalence and aggregate total hepatic cyst volume increased with age.

Cyst Number but Not the Rate of Cystic Growth Is Associated with the Mutated Gene in Autosomal Dominant Polycystic Kidney Disease

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 < 0.0001). PKD1 is more severe because more 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.

Quality of life in autosomal dominant polycystic kidney disease patients not yet on dialysis

BACKGROUND AND OBJECTIVES Autosomal dominant polycystic kidney disease (ADPKD) is an inherited progressive disorder associated with significant pain and discomfort affecting quality of life. This study determined the impact of pain medication use and other clinical, biochemical and genetic characteristics on the physical and mental well being of predialysis ADPKD patients using the Short Form 36 (SF-36) questionnaire. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS The authors prospectively evaluated ADPKD patients in the Cohort Study, funded by the Polycystic Kidney Disease Foundation. Data on clinical, biochemical, and radiologic variables were collected in patients who were given the Short Form-36 questionnaire. Variables independently associated with the Physical Component Summary (PCS) and the Mental Component Summary (MCS) scores were identified. RESULTS One hundred fifty-two patients had a mean PCS and MCS of 46.9 +/- 11.3 and 51.0 +/- 9.0, similar to the general population and better than the ESRD population. Eleven (7%) reported pain medication intake within 1 mo of evaluation and demonstrated lower PCS than those not taking pain medications. Patients with GFR >or= 80 ml/min/1.73 m(2) had greater PCS than those with GFR < 80 ml/min/1.73 m(2). Age, BMI, pulse pressure, pain medication use, and education level independently associate with PCS and account for 32% of the variability of the measurement. Pulse pressure correlated with MCS. CONCLUSIONS Predialysis ADPKD patients assess their quality of life similar to the general population. Age, BMI, pulse pressure, pain medication intake, and education level link to their physical well-being.

Prognostic factors for gastrointestinal and bronchopulmonary carcinoid tumors

Data collected by population‐based cancer registries in Iowa and metropolitan Atlanta were evaluated to determine prognostic factors for gastrointestinal (n = 270) and bronchopulmonary (n = 151) carcinoids. The predictors considered in univariate and multivariate analyses were: age, sex, race, marital status, anatomic subsite, stage, occurrence of other malignancies, and surgery. For surgically treated gastrointestinal tumors, the cumulative percentages of survivors at five years were: appendix, 85.6%; small intestine, 66.0%; and large intestine, 37.7%. The likelihood of death from gastrointestinal carcinoids was found to be related independently to increasing age (P = 0.001), advanced stage (P < 0.0001), location within the large intestine (P < 0.0001), and occurrence of another malignancy (P = 0.02). The overall five‐year survival rate for bronchopulmonary carcinoids was 87.6%, and lack of surgical treatment (P < 0.0001) and advanced stage (P = 0.006) were associated independently with unfavorable prognosis.

IMAGING AND RADIOLOGIC MANAGEMENT OF UPPER URINARY TRACT INFECTIONS

Most infections of the upper urinary tract respond promptly to antibiotic therapy and imaging is not necessary. Patients with urinary obstruction, diabetes, or immunocompromise are more likely to develop complicated infection, abscess, or have unusual organisms. Chronic granulomatous processes involving the kidney are usually related to recurrent bacterial infections. Again, stone disease or obstruction is often an underlying problem. In those patients who do not respond promptly to treatment or have a more complicated clinical picture, imaging can assess the severity and extent of disease. CT scan is the study of choice for diagnostic evaluation in these patients and directs percutaneous intervention when appropriate. Placement of drainage catheters is often curative but also may allow the patient to stabilize until surgical treatment is accomplished. One exception is the diagnosis of pyonephrosis, which may be accomplished more easily by ultrasound. In these cases, PCN placement is generally needed and is performed under fluoroscopic guidance. Ultimately, however, definitive surgical intervention often is needed to relieve the underlying obstruction.

Contrast-Induced Nephropathy: Contrast Material Not Required?

OBJECTIVE This commentary deals with the study by Newhouse and colleagues in this issue of the AJR discussing the implications of a lack of a control group in previously published studies on contrast-induced nephropathy (CIN). CONCLUSION Until more rigorous studies including an appropriate control group address the issue of CIN, our understanding of the actual risk of CIN when administering IV contrast media is limited.

Determinants and Functional Significance of Renal Parenchymal Volume in Adults

BACKGROUND AND OBJECTIVES The significance of renal parenchymal volume and the factors that influence it are poorly understood. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS Renal parenchymal volume (RPV) was measured on contrast-enhanced CT scans after exclusion of sinus fat and vessels in 224 healthy subjects evaluated as kidney donors and in a separate cohort of 22 severely obese individuals before and after 6 months of weight loss. GFR was measured by iohexol clearance in 76 of the transplant donors. RPV was correlated with age, GFR, and various anthropometric parameters. RESULTS In potential transplant donors, RPV correlated with body surface area (BSA; r = 0.68) and was 7% larger in men but did not vary with age or race. Gender and body size were independent determinants of RPV. RPV correlated well with GFR (r = 0.62) and accounted for almost all of the variability in a model of GFR that included age, race, gender, and body surface area. GFR correlated more strongly with RPV than with creatinine-based equations. The same relationship between RPV and BSA was observed in obesity, and RPV decreased with weight loss. CONCLUSIONS In healthy adults younger than 65 years, renal parenchymal volume is governed by body size and gender but not age or race and is strongly correlated with GFR. This indicates that renal parenchymal volume varies to meet metabolic demand and is closely linked to renal function.

Incidental findings of thickening luminal gastrointestinal organs on computed tomography: an absolute indication for endoscopy

OBJECTIVE:Incidental findings of thickened luminal GI organs (LGIO) on CT are not uncommon. However, the significance of these findings is unclear. Because of the lack of scientific data, there are no clinical guidelines for the evaluation of these radiologic abnormalities. Our objective was to determine whether endoscopic evaluation of these findings revealed significant abnormalities.METHODS:This study evaluated all incidental findings of thickened LGIO in a large medical center from October, 1997 to March, 1999 that were followed by endoscopic examinations.RESULTS:Ninety-six percent of patients with incidental findings of thickening of the sigmoid colon or rectum, 81% of patients with thickening of the distal esophagus, and 13% of patients with thickening of the cecum had significant abnormalities on further endoscopic work up.CONCLUSIONS:Although positive pathologic findings are less common in thickening of the cecum than in other LGIO, all of these incidental findings on CT warrant further endoscopic examination.

Duplicated vena cava with tumor thrombus from renal cancer: use of venogram for safer operative planning.

Renal and adrenal tumors can invade into the inferior vena cava (IVC) in 4%-10% of cases. Consequently, urologists must remain well versed in the anatomy of the IVC. The IVC develops embryologically from the coordinated growth and regression of a series of veins. Occasionally, these veins can persist, creating vena caval abnormalities. A completely duplicated caval system is 1 of these variations. We present a case of renal cell carcinoma with tumor thrombus extending into a duplicated IVC and show how understanding of venous drainage and pertinent imaging can aid with a successful outcome.

MR Findings in Cystic Ovarian Tumors

Ovarian neoplasms are classified by cell of origin: epithelial tumors (serous and mucinous, endometrioid, clear cell, and Brenner tumors); germ cell tumors (mature and immature teratomas, dysgerminoma, endodermal sinus tumor, and embryonal carcinoma); sex cord–stromal tumors (fibrothecoma, granulosa cell, sclerosing stromal, and Sertoli–Leydig cell tumors); and metastatic tumors. Cystic ovarian neoplasms are present in every group and often represent a diagnostic challenge, in part, because there is overlap in the imaging appearance of the different neoplasms and because benign disease is considerably more common than malignant disease and usually cystic.1 Preoperative characterization of cystic ovarian masses is of paramount importance, enabling the surgeon to anticipate malignancy and to plan appropriate treatment. MRI is more accurate than ultrasound and CT in the characterization of ovarian tumors because of its excellent contrast resolution.1,2 The difference in signal-intensity characteristics of cystic ovarian masses allows for a methodical approach to characterization and a precise diagnosis in many cases.2 The purpose of this article is to review the most common cystic ovarian neoplasms and to explore predominant MR characteristics of both benign and malignant cystic ovarian neoplasms in order to aid in differentiation or at least significantly narrow the differential diagnosis.