Thomas G. Rudd

Liver cysts in patients with autosomal dominant polycystic kidney disease

Liver cysts were found in 46 (29 per cent) of 158 patients over 10 years of age with documented autosomal dominant-type polycystic kidney disease (PKD) from 62 unrelated families. Hepatic cysts were not found in any patient at risk for PKD in whom renal cysts were not detected. The prevalence of liver cysts increased with advancing age and with declining rate of glomerular filtration. Results of clinical and laboratory studies indicate that polycystic liver disease in patients with autosomal dominant-type PKD is a benign condition, rarely, if ever, causing impaired liver function or portal hypertension.

Effect of Percutaneous Nephrostolithotomy on Renal Function

In 15 patients with 17 stone-containing renal units renal function studies were performed before and 2 to 3 months after percutaneous nephrostolithotomy. The creatinine clearance of the operated renal units was calculated from the total creatinine clearance and differential renal function was obtained with radionuclides. Changes in the cortical images of the operated kidney also could be determined from the radionuclide scans. There was no significant change in the creatinine clearances when all of the operated kidneys were considered. However, there was significant improvement in the function of 12 units containing infectious stones in which preoperative function was reduced. Percutaneous nephrostolithotomy preserves or improves renal function and can be used for all types of renal stones.

Demonstration of gastroesophageal reflux in children by radionuclide gastroesophagography

Twenty-five infants and children with proved gastroesophageal reflux were studied by radionuclide gastroesophagography to determine its sensitivity in detecting reflux. Patients swallowed an inert radiotracer (Tc-99m sulfur colloid) and gamma camera images of the stomach and esophagus were made with and without abdominal pressure. The test was easy to perform and was well tolerated. Reflux was demonstrated in 20 patients (80%); this compared favorably with barium gastroesophagography.

Intrafamilial Phenotypic Expression of Autosomal Dominant Polycystic Kidney Disease

It has been suggested that the clinical expression of autosomal dominant polycystic kidney disease (ADPKD) is uniform among individuals of a given family. To test this hypothesis, intrafamilial variations in ages at onset of first symptoms, types of first symptoms, serum creatinine concentrations, and renal sizes were evaluated in 131 patients with ADPKD from 36 unrelated families. These parameters were compared in younger and older affected relatives in the same family at a single time, due to difficulties of following them longitudinally. Because the natural course of the disease is to progress with age, it was presumed that disease progression in a given family was nonuniform if older individuals had lower serum creatinine concentrations, and/or smaller kidneys than their affected younger relatives, or if relatives of similar ages had different serum creatinine concentrations and/or kidney sizes. Nonuniform progression was suggested in 38% of affected relatives by serum creatinine concentrations and in 53% by kidney sizes. Ages at onset of first symptoms and types of first symptoms were also different in patients from the same families. These data indicate that phenotypic expression of ADPKD may differ considerably among patients who belong to the same families.

Clinical Manifestations of Autosomal Dominant Polycystic Kidney Disease in Patients Older Than 50 Years

The purpose of this study was to define manifestations of autosomal dominant polycystic kidney disease (ADPKD) in older patients with the disease. Fifty-seven subjects age 50 years or more, who were at risk for having inherited the gene for ADPKD, were evaluated for renal size, hypertension, back and abdominal pain, symptoms consistent with urinary tract infection (UTI), hematuria, end-stage renal failure, and liver cysts. The diagnosis of ADPKD was made in 32 of the 57 at-risk subjects (56%). At the time of study, only one patient with the disease was asymptomatic and normotensive and denied any previous symptoms suggestive of the disease. Clinical manifestations of ADPKD in the 31 symptomatic patients were hypertension (69%), a history of back and abdominal pain (47%), symptoms consistent with UTI (41%), hematuria (31%), and end-stage renal failure (47%). Liver cysts were found in 44% of patients. No statistically significant differences in the frequency of any manifestations of ADPKD between men and women were found, although the frequency of symptoms consistent with UTI tended to be higher in women (53%) than in men (27%). Most patients developed symptoms after the age of 40 years. Notably, 31% of the older patients with ADPKD had normal serum creatinine levels. Thus, older subjects with kidney cysts who are at risk to have inherited the gene for ADPKD, should be considered to have the disease even in the presence of well-preserved kidney function. This observation may play an important role in assessing the prognosis of older subjects at risk who have bilateral renal cysts and in genetic counseling of their relatives.

The normal 99mTc-DMSA renal image.

Renal images of 194 normal subjects given 99mTc-dimer captosuccinic acid (DMSA) were reviewed to establish normal values an optimal imaging techniques for this new renal agent. Images were consistently of high quality. Normal renal size (posterior length) averaged 11.5 +/- 1 cm, and renal radioactivity (functioning tubular mass) was equally divided between the left and right kidney. 99mTc-DMSA appears to be an excellent renal parenchymal imaging agent.

Ureteropelvic Junction Obstruction in Infants and Children: Functional Evaluation of the Obstructed Kidney Preoperatively and Postoperatively

We evaluated 14 children with primary ureteropelvic junction obstruction, using preoperative excretory urography and renal imaging with the renal cortical labeling agent 99mtechnetium dimercaptosuccinic acid. All children with a reduction in function of 10 per cent or more in the obstructed kidney had severe calicectasis; others with severe calicectasis had minimal functional loss. Renal scanning with dimercaptosuccinic acid discourages surgery in the questionably obstructed kidney and encourages repair in the severely obstructed kidney. In 10 children who had followup renal imaging after repair relative function was not significantly different.

Diagnosis of right hemidiaphragmatic rupture by liver scintigraphy.

We report two cases of traumatic rupture of the right hemidiaphragm with liver herniation following blunt abdominal trauma. In both, the diagnosis was not immediately suspected, and liver scintigraphy was later diagnostic. Focal elevation of the central portion of the right lobe of the liver was present in both cases. In one case the herniated liver was surrounded by a band-like photon-deficient collar. Liver scintigraphy can be quickly performed by portable technique and may allow for early diagnosis.

Assessment of cobalt 57 tagged bleomycin as a clinical aid in staging of head and neck carcinoma

Critical assessment of head and neck cancer with respect to staging has, on occasion, been disappointingly ineffective. We have attempted to correlate the incidence of measureable uptake of cobalt 57 tagged bleomycin by primary squamous cell carcinoma and metastatic cervical lymph nodes. Forty‐six cases have been evaluated with respect to histopathological confirmation of the suspected metastatic disease. We have found that this diagnostic measure increases our acumen in staging of head and neck cancer.

Evaluation of traumatic mesenteric hemorrhage in a hemophiliac with Tc-99m labeled red blood cell scintigraphy

A hemophiliac with a self-inflicted abdominal stab wound was evaluated with computerized tomography, ultrasound, and technetium-99m labeled red blood cell (Tc-99m RBC) imaging. A large intra-abdominal hematoma was best defined by CT and ultrasound; however, continued bleeding, indicating the need for emergency surgery, could only be demonstrated by radiolabeled red blood cell scintigraphy.