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Biomedical subjects

S S Siegelman

Publications and source records attributed to S S Siegelman.

At least 19 recordsLinked to original sources

Urologic manifestations of sickle hemoglobinopathies.

We analyzed the renal radiographic changes in the sickle hemoglobinopathies in 92 patients, in 70 of whom the specific hemoglobin type was documented by electrophoresis. The following conclusions have been drawn: (1) Approximately 50% of patients with SS hemoglobin have renal enlargement (both on roentgenograms and at autopsy), while only a small proportion of patients with the other hemoglobinopathies show renal enlargement; (2) slightly more than one half of patients with each type of hemoglobinopathy have calyceal changes; and (3) these calyceal changes do not fall into a specific pattern with respect to hemoglobin type.

Adolescent

Pulmonary interstitial changes following bone marrow transplantation.

Observations of 20 consecutive bone marrow transplantation recipients disclosed two distinctive categories of pulmonary interstitial changes. Interstitial infiltrates occurring less than 14 days after transplantation were observed in 13 of 20 patients and are believed to represent pulmonary edema. A late pulmonary interstitial process, which appears more than 30 days following transplantation (median 57 days), was encountered in 10 of 14 patients with successful grafts; the late changes represent interstitial pneumonia, a common cause of morbidity and mortality following bone marrow transplantation. It is probably attributable to a combination of factors including the preparatory chemotherapy and radiotherapy, and delayed immunologic reconstitution following transplantation.

Adult

The multiple roentgen manifestations of sclerosing mediastinitis.

Sclerosing mediastinitis is excessive production of fibrous tissue in the mediastinum. Its most common manifestations, widening of the mediastinum and superior vena caval obstruction, are the only roentgenographic findings generally recognized to be associated with this disease. Analysis of 29 cases of sclerosing mediastinitis and review of the literature reveal that compression of mediastinal organs by constricting bands of collagen may cause various roentgen manifestations including bronchial or tracheal obstruction leading to atelectasis or obstructive pneumonitis, pulmonary venous obstruction, pulmonary artery obstruction, esophageal obstruction and nerve entrapment. The most common abnormality seen on the chest roentgenogram, mediastinal widening, is usually superior and especially right paratracheal in location. Superior vena caval compression is smooth and tapered rather than irregular as in malignant disease. Pulmonary venous and arterial obstruction may cause cor pulmonale and death, although the other manifestations are usually not life-threatening. The most common causes of sclerosing mediastinitis are generally considered to be granulomatous diseases, especially histoplasmosis, although many other causes have also been suggested. The disease may be regarded as an inappropriate response to one of several stimuli.

Adolescent

Abnormalities in the sequence of filling and emptying of mesenteric arteries and veins: a guide to ischemic disease of the bowel.

Functional abnormalities in the sequence of filling and emptying of mesenteric arteries and veins may indicate significant disease of the bowel which is not associated with identifiable structural alterations in the blood vessels. In three patients, focal ischemic damage to the bowel was associated with significantly delayed arterial emptying, delayed or absent filling and emptying of mesenteric veins or a combination of both. Evaluation for sequential filling and emptying of vessels proved most helpful since an abnormal pattern may represent the only angiographic abnormality indicative of intestinal infarction.

Adult

Protean manifestations of mycotic aneurysms.

Nine patients examined by arteriography were shown to have mycotic aneurysms involving the thoracic aorta, subclavian artery, renal artery, middle cerebral artery, hepatic artery, and splenic artery. Patients presented with sepsis, chest pain, mediastinal mass, headache, hypertension, and intraperitoneal bleeding. Etiologic factors included endocarditis, septicemia, drug abuse, and poorly controlled soft-tissue infection. Most mycotic aneurysms were virulent processes with rapid progression and only three of the nine patients (33%) survived. Since mycotic aneurysms may be associated with rapid progression and poor prognosis, early recognition is mandatory.

Adolescent

Functional abnormalities, of mesenteric blood flow. A guide to organic disease of the bowel.

Analysis of functional as well as anatomic aspects of blood flow greatly increases the diagnostic accuracy of superior mesenteric artery angiography. In almost all normal individuals the major branches of the superior mesenteric artery empty within a spain of two seconds. The corresponding draining veins all first appear within a two second span. Deviations from the pattern must be explained, as they usually signal abnormality. Five pertinent cases illustrate functional abnormalities leading to the detection of tumors where definite tumor vascularity was not demonstrated, the localization of a bleeding site where extravasation was not detected, and the recognition of obstructing adhesive bands.

Adenocarcinoma

Superior mesenteric artery embolization: an angiographic emergency.

Eleven cases of superior mesenteric artery embolization are presented. The extent of bowel necrosis was found to be related to two major factors: the site of occlusion and the elapsed time between the onset of symptoms and definitive therapy. Angiography, available on an emergency basis, provides a definitive diagnosis which can only be suspected by other means. Therapy instituted within 24 hours of the onset of symptoms in all cases resulted in a mortality of only 9% attributable to intestinal infarction.

Adult

Transitional cell tumors of the kidney: how diagnostic is the angiogram?

Angiograms of 51 tests cases including 34 transitional cell carcinomas, eight inflammatory lesions, four hypernephromas, and five miscellaneous disorders were shown to six radiologists for analysis of the angiographic features and specific diagnosis. Overall accuracy was 60% with a false positive rate of 44%. The most common false positive diagnosis occurred with inflammatory lesions (seven of eight). False negative cases were variously called normal, hypernephromas, inflammatory, or other. Vessel encasement (26%), neovascularity (56%), and tumor blush (15%) were recognized far less frequently in the transitional cell tumors of this series than in previous reports in the literature. Further, when hydronephorsis was present, the angiogram was not helpful in determining the etiology. It is concluded that the "classic" angiographic findings associated with this tumor are only moderately reliable since they can be mimicked by other conditions.

Angiography

Nonmalignant lymphoid disorders of the chest.

Several nonmalignant lymphoid disorders involve the lung parenchyma or the mediastinal or hilar lymph nodes. The pulmonary parenchymal lesions include lymphocytic interstitial pneumonitis, pseudolymphoma, and lymphomatoid granulomatosis. These disorders are generally not accompanied by lymph node enlargement. Lymph nodes (e.g., in the mediastinum and hilum) are involved in lymphadenitis, giant lymph node hyperplasia, and a new and unclearly defined entity called angioimmunoblastic lymphadenopathy. An awareness of the distinction between these reactive disorders and lymphoma is important because the radiologic appearances may be similar. Histologic diagnosis is essential before treatment is initiated. With the exception of angioimmunoblastic lymphadenopathy and possibly of lymphadenitis, involvement of the pulmonary parenchyma associated with radiologic evidence of lymph node enlargement militates against the presence of any of these nonmalignant disorders.

Adult

Restoration of bronchial artery circulation after canine lung allotransplantation.

To evaluate the re-establishment of the bronchial circulation in lung transplantation, we studied 10 immunosuppressed dogs up to 14 weeks after left lung allografting. Selective in vivo bronchial arteriograms were performed repetitively via the transfemoral route. In the early postoperative period, no fillinf og vessels distal of the bronchial anastomosis could be shown. After 12 days, however, continuity of the bronchial arteries across the anastomosis was present, and dye-filled ramifications of these vessels were visualized on the secondary and tertiary bronchi. Reconstitution of the bronchial circulation was also confirmed by postmortem studies after injecting the isolated descending thorasis aorta with colored radiopaque material (microfil). The bronchial mucosa at autopsy was examined microscopically. There was no correlation between its viability and bronchial artery regeneration. Although early ischemia of the transplant bronchus may be after a factor in the bronchial complcations that follow lung transplantation, the present study indicates that this ischemia is not due to failure of bronchial artery regeneration.

Animals

Localized tumor-like lesions in ulcerative colitis and Crohn's disease of the colon.

Ulcerative colitis and Crohn's disease of the colon may manifest roentgenographically as localized tumor-like lesions of the colon. Such lesions are often of inflammatory origin but are likely to be mistaken for polypoid carcinoma or infiltrating submucosal malignancy. Four patients with localized inflammatory lesions of the colon that mimicked a neoplastic process are reported. The clinical features and the radiological-pathological correlation of such lesions are presented.

Adult

Beneficial effect of propranolol in a histologically appropriate model of postischemic acute renal failure.

Acute renal failure caused in the rabbit by clamping one renal pedicle for 1 hour and removing the opposite kidney produced a histologic picture very similar to that observed in "hypotensive" acute renal failure in man. Intravenous infusion of propranolol, a drug which prevents renin release, at 1 mg/kg for 70 minutes beginning at time of pedicle clamping resulted in significantly lower serum creatinine in this model (2.8 +/- 0.2 mg% at 48 hours with propranolol versus 5.2 +/- 0.8 mg% without). Renin stimulation by dehydration or feeding a low-salt diet enhanced the difference between treated and untreated groups (2.6 +/- 0.4 mg% with propranolol versus 6.2 +/- 1.8 mg% without, after dehydration; 3.5 +/- 1.0 mg% with propranolol versus 7.6 +/- 1.4 mg% without, after low-salt diet).Suppression of renin production by saline feeding eliminated propranolol's beneficial effect (5.6 +/- 0.9 mg% with propranolol versus 4.0 +/- 0.6 mg% without). In rabbits with a normal food and water intake, renal denervation using phenol also eliminated propranolol's effect (creatinine 8.6 +/- 1.4 mg% with propranolol versus 8.6 +/- 1.8 mg% without). In rabbits with intact kidneys, flow probe recording of renal blood flow showed a significantly higher blood flow immediately after unclamping in the propranolol-treated animals, and renal angiograms showed less vasoconstriction in this group after unclamping. In this model of acute renal failure, renal vasoconstriction plays an important role following the initial ischemic insult. Propranolol lessens the severity of this vasoconstriction and the resulting acute renal failure. Its probable action is interference with neurogenically stimulated renin release.

Acute Kidney Injury

Initial results from an agressive roentgenological and surgical approach to acute mesenteric ischemia.

The 70% to 80% mortality rate of patients with acute mesenteric ischemia (AMI) has remained unchanged over the past 40 years. We report here the initial results using an aggressive approach to this problem. This included the earlier and more liberal use of angiography in patients at risk and the intra-arterial infusion of papaverine for the relief of superior mesenteric artery (SMA) vasoconstriction in both nonocclusive and occlusive forms of AMI. Of the first 50 patients managed by this approach, 35 (70%) had AMI demonstrated by SMA angiography, Nineteen (54%) of these 35 patients survived, including nine of 15 patients with nonocclusive mesenteric ischemia, seven of 16 with SMA embolus, two of three patients with SMA thrombosis, and the one patient with mesenteric venous thrombosis. Seventeen of the 19 survivors lost no bowel or had excision of less than 3 feet of small intestine.

Acute Disease

Pneumatosis intestinalis in association with connective tissue disease.

Pneumatosis intestinalis in association with connective tissue diseases is an unusual combination whose pathogenesis is not yet understood. Furthermore, steroid medication, often used to treat these diseases, may itself cause pneumatosis. Three cases of scleroderma, systemic lupus erythematosus, and amyloidosis in association with pneumatosis and without prior steroid therapy are presented. The small vessel occlusive pathologic processes in these diseases may cause focal areas of mucosal ischemia resulting in small, perhaps transient ulcerations that allow gas to enter the gut wall from the lumen.

Adult

Diagnosis and pathophysiology of paradoxical embolism.

Three cases of paradoxical embolism are presented. Although most systemic emboli originate in the heart, detection of other sources is aided by venography of the lower extremities, indocyanine green-dye curve studies of the inferior vena cava, obtaining right heart pressures, oximetry, and pulmonary angiography. Forty per cent of 55 patients reviewed had repeat episodes of systemic embolism and 2 of the 3 proved cases of paradoxical embolism had repeat episodes. Prolonged anticoagulant therapy may prevent recurrence.

Aged