The optimum urine collections for the detection and monitoring of Bence Jones proteinuria.

Twenty patients with malignant disease and Bence Jones (BJ) proteins were studied to determine the optimum urine collections for the detection and monitoring of light chain proteinuria. A 24-hour urine protein collection was followed by individual collections of each sequentially voided specimen over the same time interval. Samples were analyzed quantitatively for protein, and protein electrophoresis was performed on each specimen. Only one patient had BJ protein nondetectable by protein electrophoresis in the early morning specimen. Six patients had one or more random specimens (excluding the early morning specimens) absent for BJ protein on protein electrophoresis. Three patients had nondetectable protein on electrophoresis of the 24-hour specimen despite having some random specimens positive. All random specimens with protein values exceeding 0.20 g/L had BJ protein visibly detectable on electrophoresis. Thirteen specimens with protein less than 0.05 g/L still had BJ protein detected by electrophoresis. There was a linear relationship between the early morning protein concentration and the total 24-hour urinary protein production. The authors conclude that early morning specimens or 24-hour urine collections are preferable for the detection and monitoring of light chain proteinuria. These collection methods are not mutually exclusive because there are individual patients who will be negative in one collection but positive in the other.

Renal cell carcinoma and vasculitis: report of two cases.

A 63-year-old caucasian male with systemic leukocytoclastic vasculitis involving the skin, lungs, and gastrointestinal tract was found to have a renal cell carcinoma at autopsy. A 77-year-old-woman with a history of hypertension was felt to have temporal arteritis that was confirmed by biopsy. The autopsy revealed a renal cell carcinoma in association with widespread giant cell arteritis. The possible association of renal cell carcinoma with vasculitis and an assessment of the possible clinical usefulness are presented and cases of vasculitis with renal neoplasms are briefly reviewed.

Ossified skeletal muscle angioma: report of two cases.

Ossifying skeletal muscle angiomas are relatively rare occurrences. The preoperative diagnosis is exceedingly difficult, although ossification may be reflected radiologically as a "Swiss cheese" appearance. Two patients are described whose skeletal muscle angiomas contained osseous tissue. The osseus tissue varied from microscopic foci to an abundant amount which was readily recognizable radiologically. The pathologic and radiological features are discussed and these appearances to hemangioma and lymphangioma are presented.

Mucinous cystadenoma of the appendix: a case report of the diagnoses and management.

Cystadenoma of the appendix as a factor in the formation of mucous cysts (mucocele) of the appendix is well recognized. The diagnosis can be made in patients prior to surgery if appropriate investigations are utilized. A 66-year-old white woman with a complaint of increased frequency of bowel movements was investigated and a primary diagnosis of mucocele of the appendix was made preoperatively. As cecal involvement was suggested by these studies, a limited cecal resection was planned. These findings were confirmed at surgery. The appendectomy and limited cecal resection was curative for mucinous cystadenoma of the appendix.

Hepatic infarction: biochemical study of a case.

Hepatic infarction was observed post mortem in a 27-year-old man who died of aortic dissection. Blood had been sampled at admission and 12 and 19 hours later. Values for aspartate aminotransferase and alanine aminotransferase in serum were markedly above normal, whereas those for alkaline phosphatase and gamma-glutamyltransferase were only marginally increased. A threefold-increased creatine kinase was ascribable solely to isoenzyme CK-3, suggesting muscle breakdown. Moreover, total lactate dehydrogenase activity was increased threefold, accounted for by a ninefold increase in LD-5 isoenzyme. Those enzyme activities in serum that evidently are associated with acute hepatocellular necrosis increase quickly in hepatic infarction, and CK isoenzyme assay is a useful adjunct if LD-5 increases are significant.

Creatine kinase isoenzyme patterns in neoplasms of peripheral nerve.

Normal peripheral nerve and neoplastic lesions of peripheral nerve varied in their creatine kinase (CK; EC 2.7.3.2) isoenzyme pattern, as assessed both with electrophoresis and with column chromatography. All three isoenzymes were seen in normal peripheral nerve, but the peripheral nerve tumors, neurofibroma and neurilemmoma, demonstrated predominantly CK-1 isoenzyme activity, with a trace amount of CK-3. No CK-2 activity was demonstrated in these tumors. In contrast, malignant schwannoma tissue contained all three isoenzymes, but in a different proportion than in normal peripheral nerve.

Creatine kinase isoenzyme patterns in normal smooth muscle and smooth muscle neoplasms.

The CK isoenzyme composition of leiomyoma tissue is predominantly CK-BB and similar to adjacent myometrium tissue, while the leiomyosarcoma revealed a lesser quantity of CK-BB, but a greater quantity of CK-MM. The reasons for the discrepancy between the two types of neoplasms is not clear, but may reflect the changes which occur when smooth muscle becomes malignant.

Alaskan Malamute chondrodysplasia. V. Decreased gut zinc absorption.

Experiements were conducted to determine if zinc absorption was impaired in Alaskan Malamutes which had a genetic chondrodysplasia or dwarfism. When the uptake of an oral dose of 65Zn from the gut in blood was measured in whole dogs it was found that the chondrodysplastic animals absorbed only 25 per cent the amount absorbed by their controls. In vitro studies suggested that 65Zn was initially bound to a protein fraction in all cases but it was later released to a non-protein fraction in the case of normal dogs. This transfer did not occur in the case of the chondrodysplastic dog and it suggests that the transfer of zinc from a protein to a non-protein fraction in the mucosal cell may be the missing step in the dog with chondrodysplasia.

Urinary mandelic acid: identified in normal individuals following a single oral load of phenylethylamine.

1. An increased urinary mandelic acid was identified by gas-liquid chromatography in the urine of 3 healthy subjects following the oral ingestion of phenylethylamine. 2. The pathway for the formation of mandelic acid from phenylethylamine was suggested to involve the intermediate phenylethanolamine. The basic similarity of the metabolism of phenylethylamine and tyramine was suggested by the excretion of 4.5% of phenulethlamine as mandelic acid which correlated well to a reported 7.2% of p-tyramine excreted as p-hydroxy mandelic acid.

Alaskan Malamute chondrodysplasia III. Connective tissue of bone.

The dwarf Alaskan Malamute was compared in these studies with normal Alaskan Malamutes of the same age with regard to collagen and mucopolysaccharide components of the bone. The hydroxyproline concentration of bone segments was normal in most instances, whereas, the hexosamine concentration was increased (P less than .05) in distal segments. Alterations in amounts of collagen soluble in neutral salt, dilute acid and 5 M guanidine hydrochloride were observed and these were related to changes in subunits found following chromatography on carboxymethyl (CM) cellulose. The presence of an increased keratosulfate fraction in dwarf Alaskan Malamutes was consistent with an apparent delayed bone ossification process. These changes in the connective tissue moieties may account for the gross morphology with respect to the bowed legs. The primary cause for these changes has not yet been determined but alterations in factors related to processes of normal development in bone are suggested. The present observations tend to support the hypothesis that the changes in the bone are secondary to some other primary metabolic defect.

Alaskan malamute chondrodysplasia IV. Concentrations of zinc, copper and iron in various tissues.

Trace mineral concentrations in various tissues of the chondrodysplastic (dwarf) Alaskan Malamute are remarkably different as compared to normal. The zinc level in heart tissue was depressed in dwarf animals (26 weeks). Copper concentration in the liver is elevated two to four fold in 26 week old dwarf animals and iron levels are significantly elevated in kidney, liver and pancreas of these animals. These observations suggest that the dwarf Alaskan Malamutes suffer from a genetic defect in trace mineral metabolism. If this is the case, then many of the skeletal lesions reported for these animals may be attributed to disorders in either zinc or copper metabolism.

Colorimetric determination of creatine phosphokinase isoenzymes: detection in tissues and serum, and usefulness as an aid to diagnosis of myocardial infarction.

1. A colorimetric procedure for creatine phosphokinase (E.C.2.7.3.2.) isoenzymes suitable for routine laboratory use its described. The method utilizes a commercial product for visualization of CK isoenzymes in both serum and tissues. 2. The technic has been applied as an aid to the diagnosis of acute myocardial infarction. An intermediate migrating isoenzyme band (CK-MB) was detected in sera of 19 patients with clinical evidence of myocardial infarction and two patients with myocardial ischemia. The CK(MB) was absent in three patients presenting with symptoms suspect of, but not clinically confirmed as having myocardial infarction.

Urinary p-tyramine in hereditary tyrosinemia: I. Levels as compared to normal individuals, effect of diet, and relationship to urinary tyrosine.

1. A 40-fold increase in urinary p-tyramine was observed in a patient with hereditary tyrosinemia as compared to a control population. 2. The excretion of urinary-free p-tyramine was decreased with the restriction of oral phenylalanine and tyrosine in this patient. The pattern of urinary tyrosine and urinary-free p-tyramine was similar during the period of normal protein diet and restricted diet of phenylalanin and tyrosine. 3. The pattern of urinary-free p-tyramine and tyrosine following oral loads of tyrosine and phenylalanine was similar except for a lag period before a tyramine response was observed. 4. The possibility of the patient with hereditary tyrosinemia providing a model to study the origin of urinary p-tyramine is discussed.

Urinary p-tyramine in hereditary tyrosinemia: II. Origin of urinary p-tyramine.

1. A patient with hereditary tyrosinemia (tyrosinosis) was given oral loads of p-tyramine and tyrosine with and without medication (neomycin) to investigate the respective roles of intestinal bacteria and tissues in accounting for the origin of urinary p-tyramine. 2. The excretion of a high circulating level of p-tyramine following an oral load of p-tyramine in a patient with hereditary tyrosinemia (tyrosinosis) was as conjugated p-hydroxyphenylacetic acid (p-HPAA) and conjugated p-tyramine. 3. Both intestinal bacterial activity and tissue decarboxylation appeared to account for urinary p-tyramine in this patient following an oral load of tyrosine. 4. Sterilization of the gut by oral neomycin and a second load of oral tyrosine further supported a predominate role for tissue decarboxylation in the origin of urinary p-tyramine. 5. The data suggested that a major route of tyrosine metabolism in man may be via tissue decarboxylation of tyrosine.

Alaskan malamute chondrodysplasia. II. Urinary excretion of hydroxyproline, uronic acid and acid mucopolysaccharides.

The urinary excretion of free, total and non-dialyzable hydroxyproline appeared to be similar in both chondrodysplastic and non-chondrodysplastic Alaskan Malamutes of ages six and twenty-six weeks suggesting the metabolic defect was probably not related to a gross disturbance in collagen metabolism. Urinary hexuronic acids also appeared to be similar in levels for both populations. A four-fold increase in urinary mucopolysaccharide levels observed at age twenty-six weeks in the chondrodysplastic Alaskan Malamute suggested a deviation from normal. The magnitude and variability of deviation were not sufficient to indicate that this condition could serve as a model for the mucopolysaccharidosis of man but probably indicated a delayed maturation process.