Joseph H. Holmes

Polycystic Kidney Disease: Prospective Analysis of Nonazotemic Patients and Family Members

To develop a profile of nonazotemic polycystic kidney disease as it occurs in families, we identified and studied 164 persons with autosomal-dominant polycystic kidney disease, 81 persons suspected of having the disease, and 250 family members without the disease. Because symptoms were absent in 32% of patients with the disease but present in 30% of persons without the disease, symptoms are not reliable in screening for the disease. Hypertension and palpable kidneys and liver were significantly commoner in patients with the disease, but systolic murmur unrelated to hypertension (10.5%) and peripheral edema (9.3%) also were common. Normal laboratory values do not exclude the diagnosis of polycystic kidney disease. Ultrasonography appears to be more sensitive than excretory urography in detecting the disease and also can detect hepatic cysts. Berry aneurysms can occur and are an important cause of mortality and morbidity. The relation of renal cysts to signs, symptoms and renal function is discussed.

Cardiac calcification in uremia: A clinical, biochemical and pathologic study☆

Abstract Clinical, electrocardiographic and biochemical data are correlated with pathologic findings in six patients receiving chronic dialysis who demonstrated moderate to severe metastatic calcification of the myocardium at postmortem. Electrocardiographic changes or congestive heart failure were the only clue to metastatic calcification since x-ray signs of osteitis fibrosa or soft tissue metastatic calcification were absent. First degree heart block, the initial electrocardiographic finding in five cases, occurred within thirteen months of death and was subsequently associated with intraventricular conduction defect in four patients. This progressed to complete heart block in two patients. Sudden death occurred in two patients and intractable congestive heart failure was observed in two patients. Calcium deposition and fibrosis of the atrioventricular (A-V) node were seen in four patients with partial or complete heart block. Marked calcium deposition in the interventricular septum was noted in four patients with intraventricular conduction defect. Two patients with congestive heart failure showed moderate to severe metastatic calcification of the ventricular myocardium. One patient who died suddenly showed nearly complete calcific obliteration of the sino-atrial node. Calcium deposition with luminal narrowing of small myocardial vessels was found in all cases. The artery to the A-V node was narrowed in four patients and to the sino-atrial node in one patient. Parathyroid hyperplasia was noted in all patients.

Ultrasonic diagnosis of abdominal disease

SummaryAs currently used, ultrasonic diagnostic technics employ two types of presentation: the A-scope, or amplitude-depth, technic, and the B-scope, or compound scan and focused crystal, technic. The A-scope technic appears to have definite application in echoencephalography, echocardiography, bitemporal fetal-cephalography, and detection of tumors in the breast and gallstones in a distended gallbladder. Work on the B-scope technic for ultrasonic diagnoses over the past 12 years has demonstrated the feasibility of obtaining pictorial representation of anatomic and pathologic processes. In the abdomen good pictures have been obtained of the kidney, liver, and splenic areas, and also of fluid-filled structures such as the stomach and bladder. Under good operating conditions, this technic can clearly show such pathologic processes as cysts, abscesses, local infections, tumors, fibrosis, foreign bodies, and accumulations of fluid. At present, it can be particularly useful in precisely localizing a liver lesion for biopsy. It is necessary to acquire greater experience in interpreting pictures and in correlating these findings with those of biopsy, surgery, and autopsy. Certain improvements are discussed which may improve picture detail and equipment operation.

Changes in glucose and insulin metabolism induced by dialysis in patients with chronic uremia

Abstract The effect of repeated hemodialysis on glucose metabolism was studied in 10 patients with severe uremia. Carbohydrate metabolism was evaluated during the first dialysis and again in a subsequent dialysis (second, third or fourth dialysis). Dialysis was performed using a high bath glucose concentration ( 1500 mg. 100 cc. ) in both study periods. Improvement in carbohydrate metabolism was demonstrated in the second dialysis study period by a lower mean glucose during dialysis and by more rapid disappearance rate of glucose in the immediate postdialysis period. This improvement in carbohydrate metabolism was associated with an increase in both mean and maximum insulin levels during the second study period in 8 of the 10 patients. It is concluded that the carbohydrate intolerance associated with uremia is improved by hemodialysis. In the majority of the patients studied, this improvement was associated with a significant increase in circulating insulin levels. The increase in insulin levels in response to hyperglycemic stimulation after only one or two dialyses suggests the removal of a dialyzable substance that either inhibits insulin release or increases insulin degradation.

CHANGES IN SALIVARY FLOW PRODUCED BY CHANGES IN FLUID AND ELECTROLYTE BALANCE.

ABSTRACT Significant changes in salivary flow are shown to occur in association with disturbances of solute and fluid balance. For example, significant reductions in salivary flow were observed in dehydration and untreated diabetes insipidus. In the patients with diabetes insipidus after either pitressin or the forcing of fluids, the salivary flow returned to the normal range, which confirmed the hypothesis that water load was the primary cause for the salivary flow changes. The reductions in salivary flow observed in patients with cardiac failure, uremia and edema present a practical problem in relation to mouth care and control of fluid intake. From the data presented it is suggested that salivary flow can be altered by: (1) changes in intracellular hydration, (2) changes in salivary gland blood flow, (3) changes in metabolic composition of intra-and extra-cellular fluid, and (4) gastric and oral factors. Further study is required to clarify the physiological mechanisms causing these alterations in salivary flow and to relate them to the associated patho-physiological changes present in a particular clinical problem. In all patients complaining of thirst a reduction in salivary flow was observed.

Function of the renal homograft in man immediately after transplantation

Abstract Six patients subjected to renal homotransplantation and simultaneous bilateral nephrectomy have been studied to characterize the diuresis observed after transplant and to seek evidence of specific tubular functions in the period immediately after transplantation. A diuresis of 5,825 to 14,855 ml. per 24 hours, lasting less than 72 hours was observed in five of six cases. The magnitude of the diuresis was found to relate directly to initial creatinine clearance. Investigation of urinary composition revealed the diuresis to be osmotic. The ratio of electrolyte osmolal clearance to osmolal clearance exceeded 50 per cent in five of the six patients. Urinary urea constituted a significant but lesser portion of urinary solute. In five of six cases, mean creatinine clearances 6 and 18 hours after the transplant were 65.4 and 66.5 ml. a minute, respectively. The mean para-aminohippurate (PAH) clearance was 247 ml. a minute at 6 hours and 358 ml. a minute at 18 hours in the four patients studied at both times. Activity of specific tubular mechanisms for glucose, sodium and solute-free water reabsorption and for potassium secretion was demonstrated in the first five days following renal homotransplantation.

Analysis of 5 year experience of home dialysis as a treatment modality for patients with end-stage renal failure

Abstract We are reporting a detailed analysis of our 5 year experience with 78 home hemodialysis patients whose mean age was 39 years. All but 4 of 46 patients who had hypertension during the course of their dialysis were controlled by fluid removal and antihypertensive medications. Only two of eight patients who had positive hepatitis B antigen had clinical evidence of hepatitis. Persistent ascites was found in eight patients; four of these patients responded to either transplantation or intensive hemodialysis. Peripheral neuropathy was progressive in four diabetic patients but remained stable in nine other patients with neuropathy. Roentgenographic evidence of bone disease was found in 67.1 per cent of patients, and 21 per cent of them had fractures. The cumulative survival for the 78 patients was 95.9, 87.6, 68.7 and 53.4 per cent at 0.5, 1, 2, and 3 years, respectively. The major causes of death were cardiovascular, infectious complications and voluntary termination of dialysis. This analysis of our experience in home dialysis has allowed prospective planning in an attempt to improve both the survival and quality of life of home hemodialysis patients. The need for intensive and continuous socioeconomic and psychologic evaluation is discussed.

Urinary solute excretion as an index of renal homograft rejection.

Excerpt Initial technical success is usually achieved in human renal transplantation. Subsequent survival of the homograft and of the patient requires successful control of graft rejection. Early r...

Hyponatremia and its treatment in the hospitalized patient

Abstract Analysis of 136 cases of hyponatremia observed in this hospital shows that 50 per cent occurred in patients over 60 years old. Fluid evaluation showed that 40 per cent had excess fluids, 48 per cent normal hydration, and 12 per cent were dehydrated. Approximately 58 per cent were asymptomatic. In about half of the symptomatic group, a loss of salt had occurred and in half an excess of water. In approximately 10 per cent of the cases, the low serum sodium value proved to be a laboratory error. Effective therapy included hypertonic salt solution, 0.9% salt solution, oral sodium chloride and dialysis. Emphasis was placed on the need for complete evaluation of the patient with suspected hyponatremia and restriction of therapy to those patients with definite symptoms and with demonstrated loss of salt or water excess.

Early Polycystic Kidney Disease

Over 700 persons either having polycystic kidney disease (PKD) or related to persons having that disease were studied at the University of Colorado Health Science Center for more than 20 years.(1–4) This study was begun in 1960 when it was observed that the ultrasound image of the adult form of PKD was quite distinctive.(4) This distinctive pattern offered a potential for earlier diagnosis of PKD and for more effective family screening without the radiation exposure accompanying the excretory urogram.