Life-threatening cardiovascular manifestations of Marfan's syndrome are rare in young children. The authors report the case of a 4 1/2-year-old girl with Marfan's syndrome who had a large aneurysm of the ascending aortic valve replaced and both coronary arteries implanted into a prosthetic graft (Bentall's procedure). Other published reports of ...

The use of ketamine in patients with carcinoid syndrome is controversial because of the catecholamine response to this agent. Prior administration of diazepam modifies the catecholamine response to ketamine and may make its use in such patients safe. The successful use of a diazepam-ketamine technique for anaesthesia of a patient ...

The classic clinical features of Marfan syndrome include ectopic lens, valvular heart disease and dissecting aneurysm, and long, narrow extremeties associated with tall stature. When the classic features are incomplete, diagnosis is uncertain. This study presents some radiographic measurements of the skull in patients with the Marfan syndrome, in an ...

Gastroesophageal reflux has rarely been reported in the Zollinger-Ellison syndrome, presumably due to elevation in the lower esophageal sphincter pressure. We have evaluated 15 patients with the Zollinger-Ellison syndrome for evidence of esophageal disease. Five presented initially with esophageal disease: one, reflux symptoms; two, severe esophagitis; and two, strictures. Six ...

Twenty typical mid-gut carcinoid tumours were examined for the presence of gastrin immuno-reactivity to antisera specific to the C-terminal pentapeptide or to the mid-portion of gastrin-17. Pentagastrin immuno-reactivity was found in seven cases, and in one of these reactivity to antiserum against the mid-portion of gastrin-17 was also observed. The ...

A 2-year-old girl with Marfan's syndrome also had recurrent episodes of upper respiratory infection, otitis media, tonsillitis, and asthma. Chromosomal study revealed the karyotype 47,XXX. Immunologic evaluation showed lack of delayed hypersensitivity skin test response despite previous exposure. The coincidence of Marfan's syndrome and either XXX or immunologic dysfunction has ...

3H-thymidine uptake responses by synovial mononuclear cells to stimulation with sexually transmitted, enteric and mumps antigens were studied in 12 patients with "sexually transmitted Reiter's syndrome", 5 with "enteric Reiter's syndrome", 5 with rheumatoid arthritis, 4 with ankylosing spondylitis and 10 with "indolent arthritis of one knee." The "sexually transmitted" ...

The analysis of the clinical and HLA profiles of 99 patients with Reiter's syndrome is reported. Antigen HLA-B27, which has previously been firmly associated with Reiter's syndrome, predisposes patients to develop disease features which reflect articular involvement. The HLA haplotype A2, B27 was found to be at an elevated frequency ...

Desmosines from 24 h human urine samples were isolated, characterized and quantified. The desmosines are in peptidyl form (1000--1500 molecular weight), and their amount is decreased by two-thirds between 7 and 25 years of age. Patients with Marfan's syndrome have significantly lower urinary amounts of desmosines than do comparable controls ...

A case of Marfan's syndrome is described in which progressive neurological signs and symptoms were produced by flexion of the head. Radiological examinations revealed hypermotility between the atlas and axis, as well as compression of the medulla oblongata by the odontoid process on flexion, since low positioned cerebellar tonsils prevented ...

A 70-year-old man manifested during four years a progressive clinical picture consisting in palsy of gaze, axial rigidity, disorders of standing and gait, dysarthria, dysphagia. Neuroradiological investigations demonstrated proximal thrombosis of the left subclavian artery with subclavian steal. At necropsy, degenerative changes in several areas of the basal ganglia and ...

"Navajo arthritis" was described in 1971 as an acute, self-limited, asymmetric polyarthritis of unknown etiology seen in Navajo Indian patients. This description was before published accounts relating HLA-B27 to certain seronegative arthropathies. Review of 92 cases of arthritis seen between 1977 through 1979 in adult Navajo Indians revealed 16 cases ...

If one were to characterize the clinical presentation of Reiter's syndrome in the feet and ankles, it would be that of pain associated with mild to moderate swelling and tenderness without significant erythema. These changes would occur most commonly at the posterior calcaneus, metatarsophalangeal joint, and phalangeal regions. The ankles ...

Eighteen cases of Reiter's syndrome were found in a population of approximately 6,000 Navajo Indians. The phenotype frequency of HLA-B27 in this population was found to be 36%. The area in which these people live is endemic for shigellosis, Shigella flexneri being the most common species isolated. Presumably the high ...

Marfan's syndrome is a rare hereditary disorder characterized by skeletal, cardiovascular and ocular abnormalities. Pulmonary abnormalities occur in approximately 10% of patients the commonest being spontaneous pneumothorax and emphysema. A patient is described who had Marfan's syndrome and bronchiectasis, an association only described on 2 previous occasions in the literature.

The Zollinger-Ellison syndrome (ZES) is characterized by gastrin-producing tumors, gastric acid hypersecretion, peptic ulcers, and diarrhea. ZES may be associated with nonpancreatic endocrine tumors as part of the multiple endocrine adenomatosis-type 1 syndrome. The diagnosis of ZES can be confirmed in patients with a suggestive clinical picture and excessive gastric ...

We determined the relative concentrations of gastrin molecular species in serum samples from 21 patients with Zollinger-Ellison syndrome with localized gastrinoma (n = 11) or gastrinoma with hepatic metastases (n = 10). Gastrin molecular species were separated by gel-filtration chromatography and quantitated by radioimmunoassay with a gastrin antiserum produced in ...

The immunoreactive form of gastrin released by the islets and some of the characteristics of this release have been studied. This gastrin released by the islets in the present experiments corresponds to what has been named "Big Big" gastrin in serum of patients with the Zollinger-Ellison syndrome, in normal human ...

For the past 15 years there has been an increasing interest in the prolapsed mitral (PMV) syndrome as evidenced by its increasing attention in the literature. Although the syndrome in many of its aspects is still ill-defined, enough is known about it and its possible sequelae--arrhythmias, endocarditis, and sudden death--to ...

In an unselected series of 14 patients with Marfan's syndrome, protrusio acetabuli was documented by both presence of a center edge (CE) angle of 40 degrees or more and crossing of the teardrop by the femoral head. The disorder was unilateral in 8 patients, bilateral in 6. Since most of ...

The authors present results of histological and electron-microscopical investigations of muscular tissue obtained by biopsy from a 20-year-old man, who showed typical features of Marfan's syndrome associated with slowly progressive muscular weakness. The muscle showed a peculiar combination of centronuclear myopathy with hypotrophy of type I fibres and the so ...

Some authors have stated that nail pitting never occurs in Reiter's syndrome. The case of a patient with incomplete Reiter's syndrome with nail pitting and HLA-A2, 28; B18, 27 is presented. Patients who have both psoriasis and Reiter's syndrome have demonstrated increased frequency of HLA-A2 and B27 and markedly decreased ...

A family with Multiple Endocrine Adenomatosis Type 1 (MEA 1) is described. Of the 59 members, 15 were affected. Zollinger-Ellison syndrome was present in six individuals of whom the five available for study had hyperparathyroidism. The youngest of these patients with Zollinger-Ellison Syndrome was 25 years old. Hyperparathyroidism alone was ...

The mitral valve prolapse syndrome (MVPS) is an infrequent outcome of mitral valve prolapse, a common and usually benign structural abnormality. Psychiatric patients often present with symptoms indistinguishable from MVPS, knowledge of which is thus essential for differential diagnosis. Even when prolapse of the mitral valve is detected, the symptoms ...

To determine if patients with chronic hyperserotoninaemia from the carcinoid syndrome have increased basal adrenocortical function, I have determined the urinary free cortisol excretion of seventeen patients with carcinoid tumours and the carcinoid syndrome, twelve patients with carcinoid tumours without the carcinoid syndrome and seventeen normal subjects. There was no ...

A case of the Zollinger-Ellison syndrome, presented with watery diarrhoea, malabsorption and multiple duodenal ulcers. Resection of a gastrinoma from the head of the pancreas was ineffective. Cimetidine, administered for more than 30 months produced an immediate and sustained relief of symptoms with a gain in weight of 19 kg ...

Ultrastruct of cartilage were examined in Marfan syndrome, Menkes kinky hair syndrome, achondroplasia, asphyxiating thoracic dysplasia, mild diastrophic dysplasia and mucopolysaccharidoses I and III. Ruthenium red staining revealed decrease of proteoglycans in cases with Marfan syndrome and kinky hair syndrome, and increase in cases with osteochondrodysplasia and mucopolysaccharidosis III. This ...

A patient with Zollinger-Ellison syndrome appeared initially to respond to cimetidine with a reduction in gastric acid secretion. Symptoms immediately improved but after three days recurred with increasing severity. Intravenous cimetidine had only a short-lived and partial inhibitory effect on the rate of acid production and because of continuing pain ...

Occlusion of mesenteric vessels due to fibrosis and adventitial elastosis complicated ileal carcinoids and resulted in infarction of bowel in two patients. Synovial sarcoma antedated the carcinoid syndrome in one patient who died; carcinoma of the breast was discovered one year after hemicolectomy in the other. This rare mesenteric occlusive ...

A case of adrenocortical hyperfunction due to ectopic production of ACTH by a gastrin-producing tumor of the pancreas is described. Cushing's syndrome preceded the appearance of the overt Zollinger-Ellison syndrome by 2 years and was treated by bilateral adrenalectomy. The Zollinger-Ellison syndrome was initially treated with cimetidine, which successfully reduced ...

A previously unreported case of congenital contractural arachnodactyly (CCA) is described. This hereditary connective tissue abnormality resembles Marfan's syndrome in certain respects, but is characterized by camptodactyly rather than joint laxity, as well as by congenital contractural deformities of the knees and elbows. In addition, there is a peculiar, fairly ...

Pancreas and gut hormones are involved in many endocrine and gastrointestinal diseases. Radioimmunoassays for these hormones have proved particularly valuable in diagnosis, localisation and control of treatment of endocrine tumours, of which many are mixed. An estimate based on ten years experience in a homogenous population of 5 million inhabitants ...

Quantitative distribution of gastrin cells was evaluated in three normal human stomachs and in four stomachs from patients with Zollinger-Ellison syndrome. Cells identified by the immunoperoxidase method were counted along the entire length of five mucosal strips parallel to the axis of the lesser curvature and sampled from the posterior ...

The electrocardiogram, phonocardiogram, carotid pulse tracing and apexcardiogram were simultaneously obtained in 25 patients with mitral valve prolapse syndrome and in 62 normal subjects. This allowed us to measure systolic and diastolic time intervals and to construct the apexcarotis diagram (ACD), a new mechanocardiographic method which integrated the catotid pulse ...

To evalute the frequency of associated and hereditary endocrinopathies in the Zollinger-Ellison syndrome, 10 patients with the syndrome were studied. In seven of them, coexisting endocrine disease was found. In six, the Zollinger-Ellison syndrome was probably a feature of multiple endocrine adenomatosis type I, whereas Cushing's syndrome in the remaining ...

Seven patients with remarkably similar progressing pulmonary apical fibrocystic disease are presented. Active tuberculosis was excluded, antituberculous chemotherapy had no effect on the pulmonary lesions. One of the patients had the typical clinical picture of Reiter's syndrome, the other that of ankylosing spondylitis. HLA-antigens were determined in all seven cases, ...

The thesis is advanced that Nicolo Paganini of Genoa (1782 to 1840), the greatest violin virtuoso of all time, owed his incomparable violin virtuosity to a fortuitous and fortunate coincidence of three factors: a soaring musical genius, a flair for the dramatic and ostentatious, and manual dexterity conferred by being ...

The effects of calcium and secretin were studied in 8 patients with the Zollinger-Ellison syndrome and 18 patients with duodenal ulcer disease. Intravenous infusion of calcium gluconate produced marked increases in serum gastrin levels in the patients with Zollinger-Ellison syndrome (4,350 +/- 1,625 pg/mg) and very slight increases in the ...

A patient is presented with Zollinger-Ellison syndrome, in whom spontaneous disappearance of gastric hypersecretion and peptic ulcer disease occurred subsequent to an intercurrent illness causing acute nonspecific inflammation of the gastric mucosal lining. The dramatic clinical improvement after subsiding of the intercurrent illness was obviously linked to pronounced failure of ...

A Chinese girl with Marfan's syndrome had spontaneous pneumothorax and bronchiectasis. Spontaneous pneumothorax is not an infrequent pulmonary manifestation of Marfan's syndrome, but bronchiectasis is rarely reported to occur. So far, it has not been reported that both bronchiectasis and spontaneous pneumothorax can occur in the same patient with Marfan's ...

This study presents the frequency of severe and mild talalgias in unselected, consecutive patients with rheumatoid arthritis, ankylosing spondylitis, Reiter's syndrome, and generalized osteoarthosis. Achilles tendinitis and plantar fasciitis caused a severe talalgia and they were observed mainly in males with Reiter's syndrome or ankylosing spondylitis. On the other hand, ...

The unusual finding of peptic esophagitis and duodenal ulceration in a dog was associated with a malignant pancreatic islet cell tumor producing gastrin and ACTH. The finding of a gastrinoma in a non-human species introduces the potential for developing an animal model for the study of the protean genetic biochemical, ...

When "sausage-like" swelling of the toes occurs in the absence of clinical Reiter's disease or psoriasis, definite classification is hardly possible. Nine patients with isolated "sausage toes" (dactylitis) and minor involvement of other joints are described. The relationship between this syndrome and HLA B27 permits better classification and more rational ...

Two cases of the Marfan syndrome presented with spontaneous pneumothorax. Both had chest radiographs showing bilateral bullae in the upper lung zones and pulmonary function tests consistent with mild emphysema. There were dereases in forced expiratory flow rates at low lung volumes, carbon monoxide transfer factor, and lung elastic recoil. ...

A 44 year old white male presented with sterile urethritis, anterior uveitis, painless oral ulcers, low back pain, and synovitis of the knees and left ankle. Unilateral sacroiliitis and limited spondylitis further supported a diagnosis of Reiter's syndrome. Serum rheumatoid factor was present. The patient subsequently developed morning stiffness, symmetrical ...

Two patients are described who had the body habitus of the Marfan syndrome, cutaneous hyperextensibility, and atrophic "cigarette paper" scars. One had mild joint hypermobility and the other had generalized ligamentous laxity. Both individuals are considered to be examples of the Marfanoid hypermobility syndrome, a generalized heritable connective tissue disease ...

The relation between a specific infective event (shigellosis), a specific disease entity (Reiter's syndrome), and a specific histocompatibility antigen (HL-A B27) is documented by follow-up study of an epidemic of post-Shigella Reiter's syndrome. Five of the original 10 patients have been traced, HL-A typed, and clinically assessed 13 years after ...

To evaluate the pathogenesis of the diarrhea of carcinoid syndrome, triple-lumen jejunal-perfusion studies were performed in three patients with carcinoid syndrome and diarrhea and eleven control subjects. In contrast to the net absorption observed in the control subjects during perfusion with an isotonic mannitol-saline solution, net secretion occurred in two ...

The histamine H2-receptor antagonist metiamide is an inhibitor of endogenous and stimulated gastric-acid secretion. It appears to have therapeutic possibilities in duodenal-ulcer disease. Three patients exhibiting the Zollinger-Ellison syndrome have been treated with this drug for six months or more. Rapid symptomatic improvement occurred in each case, followed by ulcer ...

We have studied elastic tissue changes in the lungs from patients with Marfan's syndrome, generalized elastolysis, patients with emphysema of other cause, and those dying from unrelated causes. Degenerative changes were seen in the elastic tissue fibers in patient's with Marfan's syndrome; they varied from mild to severe. Elastic fibers ...