Intestinal Lipodystrophy Research Articles

Intestinal lipodystrophy (Whipple's disease); diagnosis by small-intestine biopsy tube.

In a 45-year-old man with a long history of recurrent peripheral arthritis and a more recent history of gastrointestinal symptoms, a biopsy specimen obtained from the small intestine by means of the Shiner tube here described led to the diagnosis of intestinal lipodystrophy. The diagnosis was confirmed during an exploratory laparotomy, at which no other abnormalities were found. A characteristic finding under the microscope was the enlargement of the intestinal villi with distinctive, foamy-appearing mononuclear cells seen most commonly in the mucosa but also in the submucosal and subserosal layers. The patient recovered gradually on conservative treatment, including therapy with ACTH. The most recent episode of arthritis responded to increased ACTH dosage.

Intestinal lipodystrophy (Whipple's disease) treated with ACTH and corticosteroids.

Intestinal lipodystrophy (Whipple's disease) treated with ACTH and corticosteroids.

Intestinal Lipodystrophy (Whipple's Disease) Amenable to Corticosteroid Therapy

lipodystrophy in a 36-year old physician, 54 cases have been reported in the literature.~-l1 Almost all of the cases were diagnosed at autopsy, and only a few in vivo by exploratory laparotomy and biopsy of the mesenteric lymph nodes or of the small intestine. Treatment had been uniformly unsuccessful, with fatal termination 1 to 5 years after the onset of the illness. Only recently corticotropin, cortisone and hydrocortisone have been used in the treatment of this disease with encouraging results. Mendes De Leon and Coenegracht,5 Jones et al} Plummer et al.,9 Radding and Fiese,1° and Lepore ll have each reported a case of intestinal lipodystrophy which responded favorably to corticosteroid therapy. We wish to present the study of another patient with this disease-to our knowledge, the 55th case of intestinal lipodystrophy in the literature. This patient has been subjected to three prolonged courses of corticotropin, cortisone and hydrocortisone therapy during an observation period of 4Y2 years. It led to the impression that in­ testinal lipodystrophy is a controllable or perhaps a curable disease. CASE REPORT

Extaintestinal roentgen manifestations of intestinal lipodystrophy.

• Intestinal lipodystrophy is manifested in various gastrointestinal symptoms and in characteristic roentgen findings. The barium pattern in the small intestine is one of mucosal coarseness and flocculation or clumping with discontinuity of the column. In addition, certain extraintestinal manifestations aid in making the diagnosis. These are well illustrated in the four cases described. In each instance, the intestinal manifestations were typical. The extraintestinal manifestations included enlargement of mediastinal, retroperitoneal, or peripheral lymph nodes; narrowing of the hip joints; and arthritic changes in the sacroiliac joints. A widening of the duodenal loop observed in one patient was ascribed to pressure from a mass of enlarged retroperitoneal lymph nodes. Patients in whom the symptoms and intestinal roentgen findings suggest lipodystrophy should have further roentgen studies of the duodenal loop, the chest, and the sacroiliac joints.

Whipple's disease (intestinal lipodystrophy): review of the literature and report of a case successfully treated with adrenocorticotropin (ACTH) and cortisone.

Excerpt In 1907 Whipple1reported a previously undescribed disease which occurred in a 36 year old physician and was manifested by vague abdominal distress, progressive weight loss, diarrhea, arthra...

Whipple's intestinal lipodystrophy

A case of Whipple's intestinal lipodystrophy occurring in a fifty-six year old man was diagnosed clinically, and confirmed by laparotomy and mesenteric lymph node biopsy. Approximately one year after the diagnosis was established the patient remains in remission apparently induced by hydrocortisone.

WHIPPLE'S DISEASE (INTESTINAL LIPODYSTROPHY) AND SERUM GLYCOPROTEINS

The first case of Whipple's disease (intestinal lipodystrophy) in which were found both a marked elevation of the serum glycoprotein and deposits of glycoprotein in the characteristic macrophages is reported. This association may represent a fundamental pathogenetic relationship. We also wish to emphasize certain diagnostic and therapeutic features of Whipple's disease. Whipple 1 in 1907 described a disease characterized clinically by vague abdominal distress, steatorrhea, progressive loss of weight and strength, and arthralgia. His patient was a 36-year-old physician, who died following abdominal exploration. The clinical diagnosis had been tuberculous mesenteric lymphadenitis. Histologically the lamina propria of the small intestine and the mesenteric lymph nodes showed a striking accumulation of mononuclear cells and deposits of fat and fatty acids. The foamy macrophages were found predominantly in the lamina propria and to a lesser degree in the lymph nodes, where the lesion was basically a lipogranuloma. Whipple noted that

Whipple's disease; report of a case with special reference to histochemical studies of biopsy material and therapeutic results of corticosteroid therapy.

IN 1907 Whipple1 first described intestinal lipodystrophy, an entity that has since been recorded but 37 times. Theories of the pathogenesis of the lipogranulomatosis of the small intestine and mesenteric lymph nodes have failed to suggest an effective form of therapy. The majority of reported cases have occurred in males, and most of the patients have died after a debilitating course of abdominal pain, steatorrhea, malnutrition with striking pigmentation and arthritis, despite various forms of treatment, including bile salts, pancreatic extract, choline, lecithin, folic acid, liver extract, aqueous adrenocortical extract, desoxycorticosterone, duodenal fluid, the fat-soluble vitamins and supportive measures.Whipple . . .

Whipple's disease: review of the literature and report of two cases.

WHIPPLE, in 1907, described a anatomically by deposits of fat and fatty acids in the and mesenteric lymphatic tissues. 1 The case he reported was characterized by gradual loss of weight and strength, stools consisting chiefly of neutral fat and fatty acids, indefinite abdominal signs, and a peculiar multiple arthritis. In naming this syndrome he decided upon intestinal lipodystrophy, which term has been used since; however, this name has recently been shown not to be fully appropriate. The disease is encountered predominantly in middle-aged men. The usual duration of symptoms has been one to five years. Abdominal symptoms have been indefinite, although some patients presented sharp or colicky abdominal pains. Progressive loss of weight, weakness, lassitude, and, later, a progressive diarrhea were prominent symptoms. Migratory arthritis, generally with an absence of fever, was another frequent complaint. Hypotension, with brown pigmentation of the skin and oral mucosa, often

A fatal case of intestinal lipodystrophy of Whipple investigated during life.

A fatal case of intestinal lipodystrophy of Whipple investigated during life.

Effect of an oral emulsifying agent on serum lipids in patients with chronic dermatoses.

EMULSIFYING agents administered orally are stated to improve the absorption of fat1and fat-soluble vitamins2from the gastrointestinal tract. Marked improvement of a secondary pellagra-like eruption was observed in a patient with idiopathic steatorrhea treated in this manner. This response suggested the possibility that faulty fat absorption may produce abnormalities in skin metabolism; the thick, dry, hyperpigmented skin in biliary cirrhosis and intestinal lipodystrophy are cases in point.3 Alterations of the composition of tissue fat have been observed in rats fed a fat-deficient diet,4and small doses of the C-18 unsaturated fatty acids have caused healing of the desquamated epithelial surfaces caused by this deficiency.5In patients with eczematous dermatitis the low iodine numbers of the serum fatty acids were increased by the feeding of unsaturated fatty acids, and there was concomitant improvement in the skin.6In psoriatic patients the surface fat from the uninvolved skin has been reported to have a

Intestinal lipodystrophy (Whipple's disease).

THE RARE occurrence and infrequent recognition of intestinal lipodystrophy as described by Whipple1in 1907 appears to warrant report of a case diagnosed recently during laparotomy. The case reported by Whipple was characterized anatomically by deposits of fat and fatty acids in the intestinal and mesenteric lymphatic tissues. Clinically, the patient had recurring attacks of arthritis, emaciation. enlargement and tenderness of the abdomen and fatty diarrhea. Since the original description of this disease, few reports have appeared in the literature.2To date the total number represents about 24 cases. The last case appeared in the literature in 1949, as reported by Copland and others.2bSeveral other authors have used the following terminology for this entity: lipophagia granulomatosis, lymphadenocele, mesenteric chyle adenectasis and lymphadenectasis. REPORT OF A CASE A 53 year old white man was admitted to the hospital in January 1950 with the complaint of dull aching

Intestinal lipodystrophy (Whipple's disease); report of a case.

AUTHENTIC reports of intestinal lipodystrophy, first recognized and described by Whipple1 in 1907, have appeared so infrequently since then that the disease may well be classed among the rarer entities. The original description noted, during a period of over five years, arthritis, weight loss, secondary anemia, cough, diarrhea, abdominal distention, ankle swelling, dyspnea and a palpable mass in the right lower abdominal quadrant, with autopsy findings of deposits of fat and fatty acids in the intestinal wall and mesenteric lymph nodes. The actual number of similar cases reported in the intervening years has varied because not all have conformed strictly . . .

Lipophagic intestinal granulomatosis (Whipple's disease); clinical and pathologic study of thirty-four cases, with special reference to clinical diagnosis and pathogenesis.

RECENT literature contains increasingly frequent reports of lipophagic intestinal granulomatosis following generally the descriptive pattern of Whipple's first case in 1907, which he called intestinal lipodystrophy. In this study of 34 collected cases the purpose is to establish the nature and frequency of the clinical features, in an attempt thereby to evolve a more clearcut pattern which may be of practical diagnostic value to the clinician. In addition, certain unusual pathologic characteristics, formerly considered coincidental, are now available in sufficient number to be significant. It will be indicated that these are indispensable in promoting a better understanding of pathogenesis and etiology. Also, a new case is presented, adding more data for analysis. Clinically, this disease is characterized initially by polyarthritis and later by postprandial abdominal pain, distention, diarrhea, wasting and death. The anatomic changes are represented by lipogranulomas in the mucosa of the small intestine and mesenteric lymph nodes, as

Whipple's intestinal lipodystrophy; report of four cases and discussion of possible pathogenic factors.

AMONG debilitating diseases is a rare pathologic state of unknown cause first described in 1907 by Whipple.1It is characterized clinically by gastrointestinal distress with fatty diarrhea and failure of intestinal absorption leading to death. In some respects it resembles the sprue syndrome, but macrocytic anemia is lacking. In many of the cases there are chronic arthritis and hypotension. Postmortem examination reveals a striking and unusual picture of macrophages in the wall of the small intestine and fat granulomas of the mesenteric lymph nodes. Four patients with this condition have come to autopsy in Duke Hospital: this we believe to be the largest series seen in any one clinic. One of these patients (case 1) was followed by one of us (J. P. H.) at intervals throughout his illness, and the diagnosis was made during life by means of biopsy of a mesenteric lymph node. We believe this to

Roentgen manifestations of small intestinal bleeding.

The small intestine is host to many lesions that bleed. It must alsways be thought of, therefore, in patients with melena. We propose herein to review the causes for such bleeding, placing particular emphasis on their roentgen manifestations. In preparing this report we reviewed the records of 161 patients with proved lesions of the smll bowel who were admitted to the University Hospital from 1940 to 1948. In addition we consulted the literature, the Registry of Radiologic Pathology of the Army Institute of Pathology and other radiologists. Among the causes for small small intestinal bleeding one may list: Anomalies Meckel's diverticulum Nonmeckelian diverticulum Intestinal duplication Primary ulcers Postbulbar doudenal Jejunal and ileal Enteritis Nontuberculous infections Tuberculous enteritis Sarcoid Intestinal lipodystrophy Benign tumors Myoma Leiomyoma Adenoma Vascular malformations Lipoma Fibroma Neurofibroma Fibroid adenoma

Intestinal lipodystrophy (Whipple's disease)

Intestinal lipodystrophy (Whipple's disease)