Lloyd G. Bartholomew

Intestinal polyposis associated with mucocutaneous melanin pigmentation Peutz-Jeghers syndrome; review of literature and report of six cases with special reference to pathologic findings.

Summary A review of the literature disclosed 69 cases of intestinal polyposis associated with mucocutaneous melanin pigmentation (Peutz-jeghers syndrome). Six new cases are reported herein, bringing the total to 75 cases in the world literature. The diagnosis should be entertained in any instance in which there are areas of unusual pigmentation on the lips or the buccal mucosa or both. Suitable x-ray studies will reveal the presence of polyps in the small bowel in almost 100 per cent of cases. The stomach, colon and rectum are involved less frequently. Eighty per cent of these polyps are benign adenomas and 20 per cent may show low-grade malignant changes. The evidence for establishing truly malignant transformation in the polyps is not completely convincing and warrants more careful investigation in the future. Our findings suggest that these polyps are developmental abnormalities (hamartomas) rather than neoplasms. A hereditary tendency is found in 55 per cent of the subjects. The chief symptoms are those of intussusception and gastrointestinal bleeding.

HEREDITARY HEMORRHAGIC TELANGIECTASIA AND GASTROINTESTINAL HEMORRHAGE

Summary In 1.59 patients with hereditary hemorrhagic telangiectasia, the chief manifestation of the disease was hemorrhage (89 per cent), with epistaxis being most common (81 per cent). The telangiectatic lesions were located primarily on the face, mucous membranes of the head, and hands. Local treatment of the lesions and occasional estrogen therapy were of some benefit. Systematic replacement of iron and transfusion of blood were the most helpful forms of treatment. Twenty-one of the 159 patients (13 per cent) had significant gastrointestinal bleeding; 20 of these had melena and 10 had hematemesis. The bleeding occurred primarily during and after the fourth decade of life. Only rarely could the typical lesions of hereditary hemorrhagic telangiectasia be visualized at operation on the gastrointestinal tract. Gastroscopic examination occasionally demonstrated them. Surgical resection of portions of the gastrointestinal tract for bleeding was almost universally unsuccessful, probably because of the diffuse nature of this disease.

Acute and Chronic Pancreatitis in Rochester, Minnesota, 1940 To 1969

Records of all residents of Rochester; Minnesota, who had acute and chronic pancreatitis between 1940 and 1969 were reviewed and abstracted; 151 clinical cases had been diagnosed, and 170 more were included as examples of incidental pancreatitis noted at laparotomy or autopsy. Incidence rates based only on the clinical cases revealed an increased incidence from the first to the second decade, but the rates during the last two decades remained fairly stable. These rates are thought to be minimal in view of the difficulties in diagnosing pancreatitis. Incidence rates of acute pancreatitis did not differ by sex during the last two decades, although among men there was a slightly greater role of chronic pancreatitis. Age-specific rates showed a gradual rise from 4 per 100,000, for those younger than 30, to 62 per 100,000, for those over 70 years old. Acute pancreatitis is more frequent than chronic pancreatitis in a ratio of slightly more than 3:1. Etiologically, biliary tract disease was present in 37% of the patients and alcohol abuse was identified in 19%. Nine clinical cases of pancreatitis had pre-existing diabetes mellitus, and 10 subsequently developed the disease. The association of these two diseases was appreciably greater than would be expected by chance.

Chronic disease of the liver associated with systemic scleroderma

SummaryOf 727 patients with systemic scleroderma, 8 had evidence of serious hepatic disease. This low incidence hardly suggests a relationship between the diffuse cutaneous sclerotic process and the chronic hepatic disease, although the association should always be looked for. It is not possible to exclude hepatitis and malnutrition as causative factors of the hepatic disease, although the histologic appearance did not suggest the latter. The hepatic disease usually developed insidiously after years of involvement by the sclerotic process. Similar disease of the liver previously noted in other related diffuse processes, such as lupus erythematosus, and the overlapping of these diffuse processes, particularly as noted in the first case, are further suggestive of a possible etiologic relationship. Special studies of the ascitic fluid in one instance presented some direct evidence to support the concept that hepatic involvement was primary and not secondary to other causes such as cardiac involvement in scleroderma. Finally, it should be emphasized again that postnecrotic cirrhosis is a morphologic and not an etiologic diagnosis and may result from many diverse causes. Careful study of the liver and its function in future instances of diffuse systemic disease such as scleroderma and lupus erythematosus may shed further light on this relationship.

Clinicopathologic study of nonspecific ulcers of the small intestine

Fifty-nine cases (31 men and 28 women) of nonspecific small bowel ulceration, representing the Mayo Clinic experience from 1956 to 1979, are reviewed. The average age was 51 years. Presenting symptoms or signs were those of intermittent small bowel obstruction (63%), blood loss (25%), and acute abdomen (12%). Whereas 78% of the ulcers were located in the ileum, 15% in the jejunum, and 7% in the jejunoileum, perforation was by far more common in the jejunum (78%) as compared with the ileum (11%). Diagnosis was rarely made preoperatively, although abnormalities were noted roentgenographically in 66%. Treatment was surgical and was usually curative. Operative mortality was 8.5%. No single cause was implicated.

Adenocarcinoma of the rectum arising in a hamartomatous polyp in a patient with Peutz-Jeghers syndrome

SummaryThe malignant potential of the hamartomatous polyps in Peutz-Jeghers syndrome has been debated. Although this is a very rare event, these polyps can undergo carcinomatous transformation, as demonstrated by this report of a 37-year-old man with well-documented Peutz-Jeghers syndrome who developed an invasive grade 2 adenocarcinoma on the surface of a hamartomatous polyp in the rectum.

Lupus erythematosus cell phenomenon in patients with chronic ulcerative colitis

Present knowledge of systemic lupus erythematosus, albeit scarce, has been accumulated slowly since its original description to its present recognition as a complex systemic disease responsible for a number of syndromes previously considered unrelated. The true incidence of systemic lupus erythematosus, though still unknown, evidently is much greater than was realized before the discovery of the L.E. cell phenomenon by Hargraves, Richmond, and Morton (1948). From their finding has stemmed also the concept that autoimmune mechanisms operate in systemic lupus erythematosus and probably are responsible for at least some of its clinical and laboratory manifestations. Little also is known of the pathogenesis of chronic ulcerative colitis. Studies on the role of immunity in that condition have been inconclusive (Good and Condie, 1961; Kraft, Bregman, and Kirsner, 1962) but extracolonic manifestations are being recognized with increasing frequency (Sloan, Bargen, and Gage, 1950; Hightower, Broders, Haines, McKenney, and Sommer, 1958). Particularly interesting are the associated arthritis and the hepatic damage. We have found 11 cases of systemic lupus erythematosus associated with chronic ulcerative colitis reported in the literature (Brown, Haserick, and Shirey, 1956a; Gray, Mackay, Taft, Weiden, and Wood, 1958; Bartholomew, Hagedorn, Cain, and Baggenstoss, 1958; Larson, 1961; McEwen, Lingg, Kirsner, and Spencer, 1962). Although the paucity of these reports may indicate that this association is merely coincidental, the systemic manifestations of both diseases and some peculiarities of the cases reported may indicate that the association is of some significance. During the year 1962, 475 patients with chronic

PRIMARY ATROPHY AND LIPOMATOSIS OF THE PANCREAS

Summary Primary atrophy of the pancreas is an unusual pancreatic disease characterized pathologically by almost complete disappearance of the acinar cells and by disappearance of the islets of Langerhans to a lesser extent. Fatty replacement varying from mild fatty infiltration to massive replacement of the organ by adipose tissue may accompany this atrophy. No specific causative factors have been demonstrated. The possible relationship to other systemic diseases, to malnutrition and dietary deficiencies, or to an unusual form of chronic pancreatitis has been considered. The characteristic clinical state is one of a deficiency syndrome associated with steatorrhea, loss of weight, normal or increased appetite, and occasionally edema, anasarca, and diabetes mellitus. Although the disease may possibly develop in childhood, it appears to be more frequent after the fifth decade of life. Surgical exploration of the pancreas may be necessary to establish the diagnosis. Recognition of this syndrome is important since it represents a benign pancreatic disease that responds to adequate replacement therapy.

POSTBULBAR DUODENAL ULCER

Excerpt Postbulbar duodenal ulcer, as the name implies, is one situated in the duodenum distal to the duodenal bulb. Anatomically, such an ulcer is in the postbulbar part of the first portion, or i...

Systemic syndromes associated with neoplastic disease including cancer of the colon

Any malignancy, including tumors in the colon, usually presents systemically in the traditional way of all tumors and locally according to the specific function of the organ involved. Important clues may be missed if one does not realize that, for some unknown reason, the malignancy may mimic endocrine and other specialized body cells. Any atypical feature of these systemic manifestations should be viewed with suspicion and investigated completely to establish that it is not due to an underlying malignancy.