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Biomedical subjects

J E Naschitz

Publications and source records attributed to J E Naschitz.

At least 19 recordsLinked to original sources

Colonic ulcers in a patient with hepatitis C virus-associated polyarteritis nodosa.

An elderly woman presented with abdominal discomfort, pulmonary infiltrates, acute renal failure, purpura, and massive hematochezia. Numerous colonic ulcers with underlying fibrinoid necrotizing arteritis were found in the colectomy specimen. Anti-hepatitis C virus (HCV) antibodies and HCV RNA were present in the serum. The diagnosis of HCV-associated polyarteritis nodosa was clear. This clinical presentation differs from the more commonly occurring vasculitis complicating HCV infection, which is of the leukocytoclastic type, and is associated with overt liver disease and cryoglobulinemia. In our patient, results of liver tests were normal, there was no cryoglobulinemia, and the vasculitis was of the fibrinoid necrotizing arteritis type. HCV-associated polyarteritis nodosa should be considered in the differential diagnosis of necrotizing fibrinoid arteritis even in a patient with normal liver function test results and in the absence of cryoglobulinemia.

Aged

The role of the capnography head-up tilt test in the diagnosis of syncope in children and adolescents.

OBJECTIVES: To evaluate the role of the capnography head-up tilt test (CHUTT) in the diagnosis of syncope in pediatric patients. METHODS: The CHUTT is a head-up tilt test with concomitant capnometry. Hyperventilation on CHUTT was diagnosed when the patient's end-tidal carbon dioxide pressure (ETPCO2) was </=25 mm Hg. Hyperventilation syncope was diagnosed when three criteria were met: loss of consciousness, ETPCO2 </=25 mm Hg, and no significant drop in blood pressure. The cohort included 65 consecutive children and adolescents (mean age, 14.2 years) who were assessed for syncope by routine investigations and CHUTT. RESULTS: The cause of the syncope was established in 67% of cases: cardioinhibitory reaction in 17%, vasodepressor in 20%, psychogenic in 22%, and mixed neurally mediated-psychogenic in 8% of the patients. The history indicated a cause of syncope in 40%, the CHUTT in 49%, and a combination of the history and positive CHUTT in 66% of patients. Neither the patients' clinical data nor values of the blood pressure, heart rate, respiratory rate, and ETPCO2 measured during recumbency predicted which patients would manifest hyperventilation or hyperventilation syncope on tilt. CONCLUSIONS: The CHUTT contributes substantially to the diagnosis of syncope in pediatric patients. The CHUTT advances the understanding of the pathophysiological mechanisms of syncope and enables the physician to reassure the patient regarding the essentially benign nature of the condition. Because it is not possible to predict which patients would develop a hyperventilation syncope on the standard tilt test, the modification of this procedure by measuring the ETPCO2 for the assessment of children with syncope should be considered.

Adolescent

The capnography-tilt test for the diagnosis of hyperventilation syncope.

We describe the capnography tilt test (CTT) for the diagnosis of hyperventilation syncope. The CTT is a 10-min supine, 30-min head-up tilt test with simultaneous monitoring of end-tidal PCO2 (ETPCO2). Hyperventilation (HV) was defined as ETPCO2 < or = 25 mmHg. Hyperventilation syncope (HV syncope) was defined as loss of consciousness with ETPCO2 < or = 25 mmHg and no significant drop in blood pressure. Four groups of patients had the CTT: group I (n = 14), patients presenting with syncope who during a prior tilt test had lost consciousness without concomitant fall in blood pressure; group II (n = 50), syncope, primary evaluation, no prior tilt test done; group III (n = 20), generalized anxiety disorder, no syncope; group IV (n = 80), arterial hypertension, no syncope. Hyperventilation was found in 11/14 patients in group I, 5/50 in group II, 7/20 in group III, and none in group IV; HV syncope was diagnosed in seven patients, all in group I. None of the parameters measured in the evaluation, including ETPCO2, predicted HV syncope on tilting. The mechanisms of resting HV and HV during tilt are not well understood. We confirm the existence of HV syncope. The tilt test should probably be used to screen patients presenting with syncope, with the CTT reserved for patients who lose consciousness during the tilt test without an associated fall in blood pressure, as HV is not always clinically obvious.

Adolescent

Crohn's disease associated with pellagra and increased excretion of 5-hydroxyindolacetic acid.

A 47-year-old woman with seronegative polyarthritis, diarrhea, and photosensitivity dermatitis was found to have Crohn's disease and pellagra. The presence of high values of 5-hydroxyindolacetic acid in the urine began the exhaustive investigations and finally enterotomy. No mass lesion was found. Argyrophilic cells were not increased in areas of inflamed intestinal mucosa or the normal mucosa. The disagreement between biochemical and histologic findings was attributed to sampling error. Antiinflammatory treatment for Crohn's disease was given and the gastrointestinal and articular symptoms improved, excretion of 5-hydroxyindolacetic acid returned to normal and there was no relapse of pellagra. Pellagra as a complication of Crohn's disease has been described in 4 cases; malnutrition and intestinal malabsorption were the proposed mechanisms for the niacin deficiency and pellagra of those patients. In the current case, the pathogenesis of pellagra may be accounted to wastage of tryptophan by an increased pool of intestinal argyrophilic cells, suggested by increased urinary excretion of 5-hydroxyindolacetic acid.

Anti-Inflammatory Agents

Severe reversible cardiomyopathy associated with systemic vasculitis in primary Sjögren's syndrome.

A 40y old woman with primary Sjögren's syndrome developed elevated purpura, peripheral neuropathy, muscular tenderness, abdominal pain, heart failure, and convulsive spells. The hallmarks of this disease were high titers of anti-Ro antibodies and low complement levels in the serum, leukocytoclastic small vessel vasculitis in the cutaneous biopsy specimen, and a life threatening clinical course. Echocardiography revealed left ventricular hypokinesis with low ejection fraction, which is unlike the more common features of cardiomyopathy complicating Sjögren's syndrome. The rapidly deteriorating heart failure and other systemic complications remitted on pulse corticosteroid and cyclophosphamide therapy. The pathogenesis of heart failure, which appeared concurrently with vasculitis and was reversed on immunosuppressive therapy, is explained in the context of the systemic disease. Leukocytoclastic vasculitis might be at the origin of this rare variant of acute, severe but reversible cardiomyopathy in pSS.

Adult

Diagnosis of cancer-associated vascular disorders.

BACKGROUND: Unexplained thromboembolism may be an early indicator of the presence of a malignant tumor before signs and symptoms of the tumor itself become obvious. METHODS: A survey of the MEDLINE data-base was conducted concerning cancer-associated vascular disorders and their role in the diagnosis of hidden cancer. The spectrum of vascular disorders heralding occult cancer and the associated laboratory abnormalities were scrutinized. RESULTS: Deep venous thrombosis was associated with a significantly higher frequency of malignancy during the first 6 months after diagnosis. Malignancies were found using simple clinical and diagnostic methods; additional screening was not cost-efficient. Other signs associated with deep venous thrombosis that increased the probability of an occult cancer were age older than 50 years, multiple sites of venous thrombosis, associated venous and arterial thromboembolism, thromboembolism resistant to warfarin therapy, and paraneoplastic syndrome. Among vascular syndromes, only cutaneous leukocytoclastic vasculitis presenting after the age of 50 years was consistently associated with cancer. Preliminary data with an antigen specific to tumor tissue, the cancer procoagulant, suggested its possible role as a tumor marker. The sensitivity for all samples analyzed from cancer patients was 80% and the specificity was 83%. CONCLUSIONS: Data from the literature enabled us to outline clinical clues that might distinguish patients with cancer-associated vasculopathies from those unaffected by malignancies. Preliminary data with an antigen specific to tumor tissue, the cancer procoagulant, suggested its possible role in detecting early stage cancer. However, large-scale prospective studies are not currently available to evaluate the role of these clues and laboratory assays in the diagnosis of early stage cancer.

Age Factors

The fasciitis-panniculitis syndromes. Clinical and pathologic features.

The authors propose to encompass under the designation of "fasciitis-panniculitis syndromes" (FPS) a group of disorders characterized by induration of the skin due to chronic inflammation and fibrosis of the subcutaneous septa and muscular fascia. The prototype of the FPS is eosinophilic fasciitis. Thirty-two consecutive patients with FPS were cared for at the author's hospital during a 10-year period. The association of the FPS with other diseases, clinical presentations, histologic features, and response to treatment were analyzed. Idiopathic FPS, that is, eosinophilic fasciitis, was diagnosed in 14 patients. In the remaining 18 cases, the FPS were ascribed to vascular disorders (n = 6), infections (n = 6), and neoplastic disorders (n = 3), while trauma, insect bites, and Sweet syndrome antedated the FPS in 1 patient each. The lesions had a sleeve-like distribution in 20 patients, plaque-like distribution in 7, and a combined pattern in 5. Skin biopsies revealed lesions in the deep subcutaneous layers with the pathologic triad of septal and fascial fibrosis, chronic inflammatory infiltration, and small-vessel vasculopathy. Spontaneous improvement occurred in 4 cases. Following cimetidine monotherapy, complete remission was achieved in an additional 3 of 5 patients. The concept of the FPS serves to advance our understanding on several fronts: emphasizing the clinical and etiologic diversity; recognizing a stereotypic tissue reaction pattern; highlighting the panniculitis in addition to the fasciitic component; and describing a similar response to drug therapy in different clinical settings. Based on the results of the present series, cimetidine may be recommended as first-line treatment.

Adipose Tissue

An experimental model of fasciitis-panniculitis. Induction of chronic fibrosing panniculitis in the rat by subcutaneous injections of lipase.

We describe an experimental model of the fasciitis-panniculitis syndrome, which includes eosinophilic fasciitis and its related disorders. Rats were given a single or repeated subcutaneous injections of saline or a 10% lipase solution. The injection sites were studied histologically and histomorphometrically. With few exceptions, the subcutaneous-fascial unit of the saline-injected rats was normal. An acute necrotizing panniculitis with inflammatory involvement of the adjacent fascia was found 2 days after a single injection of lipase. Three to 6 weeks after a single injection of lipase, the subcutaneous fat tissue and fascia were fibrotically thickened and chronically inflamed. Similar but more advanced alterations had developed in animals killed 3 weeks after three weekly injections of lipase. Progressive fibrotic thickening of the subcutaneous-fascial unit was observed following an increasing number of weekly lipase injections. The acute-phase reaction expresses a pattern of steatonecrotic tissue damage. In the ohronic phase, the fibrotically thickened subcutaneous layers typify a reaction pattern analogous to that of the human fasciitis-panniculitis syndrome. This experimental model permits better appreciation of the disorder's nonspecific pathogenesis and may help in the search for alternative therapeutic modalities.

Acute-Phase Reaction

Comparison of the 10-minute supine-30-minute tilt test with 24-hour ambulatory blood pressure for the diagnosis of diastolic systemic hypertension.

A variant of the protracted tilt test, the 10-minute supine -30-minute tilt test (10S-30TT), has been proposed by us for the evaluation of blood pressure (BP). The present investigation compares parameters of diastolic BP by 3 methods: office measurements, 10S-30TT, and 24-hour ambulatory monitoring; the last method served as a reference standard. Twenty patients presenting at the office with diastolic BP values ranging from 80 to 120 mm Hg (median 90) were studied. Statistically significant correlations were observed between the following parameters: diastolic office BP and average ambulatory awake diastolic BP (r = 0.64, p = 0.002); average tilt-test diastolic BP and average ambulatory awake diastolic BP (r = 0.69, p = 0.0006); frequency of elevated diastolic BP values during the tilt test and frequency of elevated ambulatory awake diastolic BP values (r = 0.69, p = 0.0007). Diastolic BP diagnosis scores exhibited strong correlation only between the ambulatory and tilt-test diagnosis (kappa = 0.70), but not between ambulatory and office BP diagnosis (kappa = 0.50). Based on the close correlation between results of the 10S-30TT and ambulatory monitoring in the evaluation of diastolic BP, we suggest that the faster and cheaper tilt test may serve as a substitute in certain categories of patients.

Adult

Rheumatic manifestations of occult cancer.

BACKGROUND: The frequency with which rheumatic disorders occur when malignant neoplasms first present is unknown, and the significance of rheumatic conditions as cancer markers is unappreciated. METHODS: Patients admitted to a medical ward of a general hospital (Bnai Zion Medical Center, Haifa, Israel) during a 10-year period were surveyed. The frequency of the diagnostic evaluation of rheumatic disorders resulting in uncovering occult neoplasia at the time of index hospitalization and during the 2-year follow-up period was assessed. RESULTS: The incidence of occult cancer among patients admitted to a general medical ward with previously unclarified rheumatic disorders was 23.1%. Seventy-two percent of the group of patients with rheumatic diseases and occult cancer were male; 69% with rheumatic disease without cancer (control group) were female. The median age in the group with occult cancer was an average of 10 years older than the group without cancer, 67 versus 57 years (P < 0.001). Weight loss or anemia occurred in 52% of the group with occult cancer and in 37.3% of the control cases (not significant). Typically, there were no distinguishing features of the rheumatic syndromes suggesting the coexistence of cancer. In 19 of 25 cases, the malignancy was uncovered by routine examinations. In four instances, neoplasia was not immediately apparent but was identified in a specific search. In two cases, no search for malignancy initially was undertaken, and Hodgkin's lymphoma was diagnosed 6 and 12 months later. In 9 of 25 cases, long term remission of neoplasia was achieved by cancer therapy with improvement of rheumatic manifestations coinciding with regression. CONCLUSIONS: A significant number of patients who are referred to a general medical ward for evaluation of previously unclassified rheumatic disorders may be found to have a previously undetected malignancy. For most patients, the symptoms of cancer are found by routine physical and laboratory examinations. The importance of the association of rheumatic disorders and malignancy requires greater awareness, appreciation, and clinical study.

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