Alphonse J. Palubinskas

Incidence of Radiographically Evident Bone Disease, Nephrocalcinosis, and Nephrolithiasis in Various Types of Renal Tubular Acidosis

The syndrome of renal tubular acidosis has been categorized into three physiologic types that have different clinical findings and prognostic and therapeutic implications. We reviewed radiographs of the skeleton and kidneys in 92 patients (56 children and 36 adults) with renal tubular acidosis in order to determine whether the radiologic findings could be related to the type of syndrome. Forty-four patients had Type 1 renal tubular acidosis, 18 had Type 2, and 30 had Type 4. Evidence of skeletal abnormalities was uncommon (17 per cent) and was confined to patients who had the Type 2 disorder or azotemia. The children with Type 2 and skeletal abnormalities had rickets; the adults had osteopenia without pseudofractures. Nephrocalcinosis was evident in approximately one fourth of the group (29 per cent) and was restricted to patients with the Type 1 syndrome. In patients with Type 4, osteopenia was evident in 12 per cent, all of whom were azotemic. Our observations indicate that the radiographic manifestations of renal tubular acidosis are influenced by the physiologic type of renal tubular acidosis.

Extrarenal fibromuscular hyperplasia

Abstract Clinical, radiologic, and pathologic studies on nineteen patients with lesions in the extrarenal arteries that resembled fibromuscular hyperplasia are described. In eleven patients fibromuscular hyperplasia was present in the renal arteries. Patients with involvement of the celiac artery were the only ones who had symptoms of visceral ischemia. Fibromuscular hyperplasia of the internal carotid arteries was observed in six patients, two of whom had symptoms of cerebral ischemia. Nine patients with fibromuscular hyperplasia of the carotid or renal arteries had intracranial aneurysms, and in two others intracranial hemorrhage developed in the absence of demonstrable aneurysms. The histologic similarities between intracranial aneurysms and other types of aneurysms that appear in patients with fibromuscular hyperplasia, the frequency of intracranial aneurysms in patients with extracranial fibromuscular hyperplasia, and the similar sex and age incidence suggest a common etiologic origin.

Medullary sponge kidney

Abstract Twenty cases of medullary sponge kidney (MSK), diagnosed on the basis of urographic findings, are reported in tabular form, the pertinent literature is considered and the generally held concept of MSK as a distinct clinical and pathologic entity is critically examined. The available evidence does not support the distinctiveness of MSK. Rather, the diversity of urographic pattern, underlying renal pathologic pattern, associated clinical abnormalities, and functional status suggest that patients diagnosed as having MSK have a heterogeneous group of renal disorders. The urographic pattern of MSK is characterized by pericalyceal pyramidal opacifications. Although previously held to be indicative of cystic ectasia limited to the pyramidal collecting tubules, the pattern is markedly variable in appearance and does not always reflect tubular ectasia limited to the renal pyramids. It seems probable that this urographic pattern does not exclude the diagnosis of polycystic kidney. The occurrence in the reported cases of such diverse abnormalities as Ehlers-Danlos syndrome, hemihypertrophy and congenital pyloric stenosis suggests that MSK in some instances may be a nonspecific renal expression of a more general abnormality. The urographic pattern of MSK was demonstrated in two successive generations of a family and in two siblings of the same family, although it could not be demonstrated in successive generations or sibships of families of other patients. We interpret this as evidence of the heterogeneity of the renal abnormality reflected by such a pattern. Evidence is also presented of heterogeneity of the renal function of acidification of patients with MSK. Some but not all patients with MSK have defects of renal acidification. The defects may take the form of impaired urinary excretion of ammonia or of titratable acid. Patients diagnosed as having MSK on the basis of urographic findings constitute a population of patients selected as to function. Such a selection may account for the apparent benignity of the renal disorder or disorders diagnosed as MSK since patients with an etiologically identical disorder but marked functional impairment would be excluded.

Results and Significance of Angiography in Potential Kidney Donors

Multiple renal arteries originating from the aortoiliac vessels were identified angiographically in 44% of 444 prospective renal donors. Bilateral multiple renal arteries were identified in 12%. With good immunological donor-recipient matching, 17 kidneys with multiple renal arteries were transplanted with excellent results; therefore, presence of multiple renal arteries should not be considered a contraindication to kidney transplantation. A high proportion (17%) of the prospective donors, acceptable by all other means of evaluation, had abnormmal angiographic findings that led to reconsideration of their acceptance. In addition to multiple renal arteries, neither renovascular atherosclerosis nor fibromuscular dysplasia proved to be an absolute contraindication to transplantation.

Roentgen diagnosis of fibromuscular hyperplasia of the renal arteries.

Fibromuscular hyperplasia of the renal artery has recently been reported as a cause of renal arterial stenosis and secondary hypertension in a small number of cases. Leadbetter and Burkland (2) first described the entity in a five-and-one-half-year-old Negro boy. DeCamp and Birchall (1) reported a case in a twenty-three-year-old male. McCormack et al. (3) added 3 examples but did not mention the sex of their patients. Poutasse and Dustan (5) described “fibrous intimal proliferation” in the renal artery of 2 young persons with hypertension but they, also, failed to designate the sex. Wylie and Wellington (6) pointed out characteristic features of fibromuscular hyperplasia of the renal artery in 3 patients and made reference to an additional 5 cases. The following report emphasizes the roentgen findings in 11 patients with fibromuscular hyperplasia of the renal arteries seen within a period of two years, including the cases described by Wylie and Wellington. Ten were investigated at the University of Califo...

Hypertension Secondary to Renal Artery Occlusive Disease

Historically, the awareness of renal artery narrowing as a curable cause of hypertension has evolved gradually, highlighted especially by Goldblatt and Poutasse. The increasing use of renal arteriography over the past 8 years in this hospital has yielded a total of 70 patients with renal artery abnormalities out of 110 hypertensive patients examined. In order to select the hypertensive patients most likely to have demonstrable arterial lesions, certain indications for arteriography were used. Most useful among these were the presence of an epigastric bruit, malignant hypertension, atheroselerosis of the abdominal aorta, and recent onset of hypertension. However, no one indication was always present in patients with lesions or always absent in those without abnormalities. Of the 70 patients with renal artery abnormalities, 54 were considered to represent sufficient renal artery stenosis to be potential candidates for surgical correction, while 16 had minor renal artery abnormalities. Atherosclerotic lesions occurred in 63 per cent of the patients with significant lesions, fibromuscular hyperplasia in 28 per cent, unilateral renal artery hypoplasia or atrophy in 7 per cent, and one case had embolic renal artery occlusion. Fifty-four per cent of all patients with significant lesions had bilateral disease. The patients with atherosclerotic lesions and those with fibromuscular hyperplasia differed markedly in sex distribution, age, and severity of hypertension. It is suggested that the retrograde transfemoral catheterization technic may be associated with fewer complications in patients without extensive occlusive atherosclerotic disease of the aorta, and iliac and femoral arteries. At operation the radiologic findings were confirmed in all but one patient. Corrective surgical procedures were performed in 38 patients, including nephrectomy, endarterectomy, segmental resection with reanastomosis, and splenorenal arterial shunt. Of the 31 patients who survived, 25 (81 per cent) had a postoperative fall in blood pressure, 14 to normal, in addition to improvement in clinical status. The follow-up period, however, is not yet sufficiently long to permit definite conclusions. Seven patients died; most of these had bilateral renal artery disease and extensive atherosclerosis of the cerebral and coronary arteries. Divided renal function studies were of limited diagnostic value because of the high incidence of bilateral lesions. In all patients with significant differences in renal sodium and water excretion, a postoperative fall in blood pressure occurred, but the same number of patients with equal bilateral excretion also had a fall in blood pressure. The importance of suspecting renal artery lesions in hypertensive patients regardless of age, severity of hypertension, or renal function is stressed. The question is discussed whether all patients with sustained hypertension should undergo arteriography. Although further studies to determine the true prevalence of occlusive renal artery lesions in the hypertensive population are in order, the fact that 50 per cent of our 110 patients had occlusive lesions and 60 per cent of the operated cases had a fall in blood pressure attests not only to the prevalence of the lesion but also to its potential curability.

Renal arteriography in the evaluation of unexplained hypertension in children and adolescents

Arteriography demonstrated renal artery stenosis in 24% of 101 selected patients whose hypertension was first diagnosed before the age of 20 years. Arteriographic findings were normal in 58%. The prevalence of RAS was greater in the younger than in the older age groups. An abdominal bruit and urographic abnormalities were frequently observed in association with RAS. Adequate follow-up information was available for 16 patients who underwent vascular repair or nephrectomy for RAS. In 14 of the 16, the hypertension was eliminated (81%) or definitely reduced (6%).

Renal Pyramidal Structure Opacification in Excretory Urography and its Relation to Medullary Sponge Kidney

Medullary sponge kidney is a relatively uncommon disorder characterized by ectasia of the collecting tubules of the pyramids, frequently associated with small cysts or cyst-like structures and minute concretions (1, 4, 7, 8, 10). In a recent review of a series of 2,465 consecutive intravenous urograms 14 cases were discovered, an overall incidence of approximately 0.5 per cent (9). When the lesion is severe and pyramidal concretions are present, the differential diagnosis involves primarily the separation of medullary sponge kidney from diseases causing nephrocalcinosis, particularly renal tubular acidosis and hyperparathyroidism. To a lesser extent, papillary necrosis, unusual renal tuberculosis, and abscesses with chronic pyelonephritis have to be excluded. When the lesions are well developed, the diagnosis of medullary sponge kidney usually presents no problem (3). A mild case, on the other hand, frequently goes unrecognized. The chief diagnostic difficulty in this mild group has been the exclusion of ...

Arterial-portal venous shunting in cavernous hemangioma of the liver.

Arterial-portal venous shunting was found to occur in a focal cavernous hemangioma of the liver. This occurrence stresses the lack of specificity of this finding in differentiating benign from malignant lesions.

Calcification in Renal Masses: An Eleven-Year Survey

Since the early days of radiology, calcification in the renal area has signified the presence of disease. In 1914, Case (7) described a calcified hemorrhagic cyst replacing a kidney, and numerous accounts have since appeared reporting calcification in cysts, tumors, and cystic tumors. In an extensive review of all possible causes of renal calcification, Goldstein and Abeshouse (12) found it to be frequent in tumors but rare in cysts. Shanks et al. (21) suggested that calcification in a cyst may be the result of change from hemorrhage. Edling (9) described shell-like calcification in the walls of some older cysts, and recently Cannon, Zanon, and Karras (6) emphasized that cyst-like calcification can be seen in tumors. The considerable literature on renal calcification is confusing since many reports have emphasized the “typical” and the unusual, but not the common findings. Calcification as a diagnostic roentgen sign in renal masses seemed, therefore, to warrant further analysis. Material Records were stud...