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PubMed1983

Arthritis of hemochromatosis. Clinical spectrum, relation to histocompatibility antigens, and effectiveness of early phlebotomy.

Five patients who presented with arthritis as the sole manifestation of hereditary hemochromatosis and 51 family members were studied. Studies included clinical evaluation for the presence of arthritis and hemochromatosis, roentgenography of hands, knees, and pelvis, serum iron and serum ferritin measurements, complete HLA typing for 50 of the A and B loci, and, when indicated, liver biopsy. Arthritis occurred in 45 percent of persons with hemochromatosis. Although typical involvement of second and third metacarpophalangeal joints was observed in all five patients and some family members, two with typical arthritis did not have characteristic radiographic changes, two had constitutional symptoms without arthropathy, and one had unilateral hand changes. A specific HLA haplotype (A2/B17 in Family 1 and A29/B15 in Family 2) correlated with hereditary hemochromatosis but not with the arthropathy. Phlebotomy alleviated the early constitutional symptoms but did not help advanced arthritis. Anti-inflammatory drugs, intraarticular injections of glucocorticoids, and resection osteotomies of metacarpal heads were other treatment modalities.

Anti-Inflammatory Agents↗
PubMed1983

Echocardiographic assessment of cardiovascular abnormalities in the Marfan syndrome. Comparison with clinical findings and with roentgenographic estimation of aortic root size.

Echocardiographic abnormalities of the mitral valve and aortic root were compared with auscultatory findings and with assessment of aortic root size by chest roentgenography in 61 patients with the Marfan syndrome. Echocardiography was more sensitive than physical examination in detecting valvular and aortic root abnormalities. Although physical examination revealed findings of mitral valve disease and/or of aortic regurgitation in 52 percent of patients (mitral valve disease in 44 percent and aortic regurgitation in 23 percent), echocardiography detected abnormalities of the mitral valve and/or aortic root in 82 percent of patients (mitral valve prolapse in 57 percent and aortic root enlargement in 69 percent). Prevalence of mitral valve prolapse was approximately equal in male and female patients, whereas aortic root enlargement was more frequent in males (83 percent) than in females (50 percent). Echocardiographically detected aortic root enlargement was frequently not apparent on chest x-ray films. Indeed, five patients with markedly increased aortic root diameters (ranging from 6.0 to 7.9 cm) had no evident enlargement of the aortic root on routine chest x-ray films. In all four of those patients who had angiographic and/or pathologic correlations, confirmation of marked aortic root dilatation was obtained. There are limitations to echocardiographic evaluation of the presence and severity of underlying cardiovascular disease in patients with the Marfan syndrome. Mitral valve disease may not be detected, especially in patients with left ventricular dilatation. In addition, due to anteroposterior compression of the left atrium by the enlarged aorta, left atrial size may be underestimated in patients with aortic root enlargement.

Adolescent↗
PubMed1984

Myocardial function and perfusion in the CREST syndrome variant of progressive systemic sclerosis. Exercise radionuclide evaluation and comparison with diffuse scleroderma.

Myocardial function and perfusion were evaluated in 22 patients with progressive systemic sclerosis with the CREST syndrome using exercise and radionuclide techniques, pulmonary function testing, and chest roentgenography. The results were compared with a similar study of 26 patients with progressive systemic sclerosis with diffuse scleroderma. The prevalence of thallium perfusion abnormalities was similar in the groups with CREST syndrome and diffuse scleroderma, (64 percent versus 77 percent), but the defects were significantly smaller in the CREST syndrome (p less than 0.01). Reperfusion thallium defects in the absence of extramural coronary artery disease were seen in 38 percent of patients with diffuse scleroderma. This finding was not seen in any of the patients with the CREST syndrome. In diffuse scleroderma, abnormalities of both right and left ventricular function were related to larger thallium perfusion defects. In the CREST syndrome, abnormalities of left ventricular function were minor, were seen only during exercise, and were unrelated to thallium perfusion defects. Abnormal resting right ventricular function was seen in 36 percent of the patients with the CREST syndrome and was associated with an isolated decrease in diffusing capacity of carbon monoxide. It is concluded that the cardiac manifestations of the CREST syndrome are distinct from those found in diffuse scleroderma. Unlike diffuse scleroderma, abnormalities of left ventricular function in the CREST syndrome are minor and are unrelated to abnormalities of coronary perfusion. Right ventricular dysfunction in the CREST syndrome appears to be primarily related to pulmonary vascular disease.

Adult↗
PubMed1985

The electrocardiogram in systemic sclerosis (scleroderma). Study of 102 consecutive cases with functional correlations and review of the literature.

The electrocardiographic findings in 102 consecutive patients with scleroderma were reviewed to determine the frequency and nature of the electrocardiographic abnormalities associated with this disease. Septal infarction pattern unassociated with QRS prolongation was present in 10 percent, compared with none of 96 control subjects (p less than 0.001). Ventricular conduction abnormalities were present in 17 percent. A normal electrocardiogram was obtained in 49 percent. A subset of 48 patients underwent detailed cardiopulmonary evaluation including exercise thallium scintigraphy, rest and exercise radionuclide ventriculography, pulmonary function tests, and chest roentgenography. Functional correlations of the electrocardiographic findings were examined in this subset. Septal infarction pattern (five of 48) and ventricular conduction abnormalities (10 of 48) were both associated with septal or anteroseptal thallium perfusion abnormalities (10 of 15 versus six of 33 of the remainder, p less than 0.005), which were present despite normal coronary angiographic results. Thallium defect scores were greater in patients with septal infarction pattern or ventricular conduction abnormalities compared with the remainder (defect scores 3.0 +/- 2.6 versus 1.4 +/- 2.2, respectively, p less than 0.025). In patients with ventricular conduction abnormalities, both left bundle branch block and right bundle branch block with left anterior fascicular block were associated with abnormal left ventricular function, whereas isolated right bundle branch block or left anterior fascicular block was associated with normal left ventricular function. A normal electrocardiographic finding (19 of 48) was associated with normal left ventricular function at rest (19 of 19). However, 11 of 19 (58 percent) had thallium perfusion defects and four of 19 (21 percent) had an abnormal response to exercise, although in none was the peak ejection fraction less than 50 percent. It is concluded that both septal infarction pattern and ventricular conduction abnormalities are electrocardiographic abnormalities associated with scleroderma heart disease; they appear to be a result of myocardial fibrosis. Some degree of myocardial fibrosis may be present with a normal electrocardiographic result, but significant left ventricular dysfunction is unlikely. Septal infarction pattern and ventricular conduction abnormalities, when present, are indicators of more advanced fibrosis.

Adult↗
PubMed1985

Community-acquired lower respiratory tract infections. Prevention and cost-control strategies.

The treatment of patients with community-acquired pneumonia can be expensive, particularly if care is hospital-based. Cost control begins with prevention. Current influenza vaccines are about 80 percent protective, but grossly underused. Amantadine and rimantadine are effective chemoprophylactic agents against influenza A, but also underused. Use of pneumococcal vaccine is controversial, but patients who are thought to be at increased risk should be immunized. Management decisions in patients with pneumonia that have major cost implications include the need for hospitalization and choice of diagnostic tests and therapy. The need for hospitalization has not been well studied. In general, young patients with atypical pneumonia are treated at home, whereas older patients with complicating illnesses are admitted to hospitals. Length of hospitalization has decreased in recent years. Diagnostic tests have traditionally emphasized chest roentgenography, Gram staining of the sputum, and sputum culture. Published data suggest that a Gram staining of the sputum can be useful. Sputum cultures are frequently confusing and should be discontinued. Intermittent positive pressure breathing treatments have no value, and chest physiotherapy is unnecessary for most patients.

Amantadine↗
PubMed1985

Diagnostic strategies in osteomyelitis.

Technetium-99 (99Tc) pyrophosphate bone scanning often identifies patients with osteomyelitis before roentgenographic findings appear. However, recent studies have shown that 99Tc bone scanning often gives false-negative results, especially in neonates. The accuracy of computed tomographic scanning and indium-111 leukocyte scanning for diagnosis of early osteomyelitis has not been established. 99Tc bone scanning often gives false-positive results in patients with other conditions leading to bone injury and repair, such as trauma or recent surgery, further limiting the usefulness of this imaging procedure. Newer imaging techniques have not been adequately evaluated to establish their specificity. Because of their high cost and unproved accuracy, these new imaging procedures should not be routinely applied until their usefulness has been established. Bone biopsy remains the procedure of choice for establishing the diagnosis in patients suspected clinically to have osteomyelitis with negative findings on roentgenography and 99Tc bone scanning. Although Staphylococcus aureus is the leading cause of osteomyelitis, other pathogens cause 30 to 40 percent of cases. Aspiration or biopsy of the involved bone is usually required to choose appropriate antibiotic therapy. Bone biopsy is essential in chronic osteomyelitis, since cultures of sinus drainage are unreliable. Osteomyelitis in diabetics with foot infection and in association with decubitus ulcers presents special problems. Radionuclide scanning often give false-positive results in these patients. Proper diagnosis usually requires careful assessment of clinical and roentgenographic findings. Although bone biopsy seems useful in diagnosing osteomyelitis underlying decubitus ulcers, its role in diabetic patients with foot infections is not established. I do not recommend biopsy of foot bones in diabetic patients, since culture of bone biopsy specimens often give unreliable results in these situations because of contamination with ulcer organisms.

Acute Disease↗
PubMed1985

Acute severe mitral regurgitation. Pathophysiology, clinical recognition, and management.

Acute severe mitral regurgitation often goes unrecognized as an emergency requiring prompt, lifesaving treatment. Its causes, physical signs, natural history, echocardiographic features, and findings on chest roentgenography, electrocardiography, and nuclear scintigraphic scanning are reviewed. Acute severe mitral insufficiency can be differentiated from chronic severe mitral insufficiency by noninvasive two-dimensional echocardiography. M-mode echocardiography is a valuable tool in evaluating mitral prosthetic paravalvular regurgitation.

Acute Disease↗
PubMed1986

Mechanism of acute lower extremity pain syndrome in fluoride-treated osteoporotic patients.

Acute pain in the lower extremity, which has previously been attributed to synovitis or fasciitis, develops in about 15 percent of osteoporotic patients treated with sodium fluoride. This report describes 11 osteoporotic women in whom this syndrome developed while they were being treated with sodium fluoride (mean dose 78 mg per day; range, 60 to 90). [99mTc]Hydroxymethylene diphosphonate scintiscanning showed an increased number of foci of abnormal uptake in the lower extremities (p less than 0.05), when compared with results of scintiscanning in 12 nonsymptomatic osteoporotic women treated with sodium fluoride and 12 osteoporotic women treated with oral calcium carbonate only. The increased uptake was not restricted to the areas of pain. Roentgenography revealed stress microfractures in five of the 11 symptomatic patients. It is concluded that the acute lower extremity pain syndrome during fluoride therapy usually results from intense regional bone remodeling, which may be complicated by stress microfractures.

Aged↗
PubMed1986

Clinically silent congenital adrenal hyperplasia masquerading as ectopic adrenocorticotropic hormone syndrome.

A 64-year-old man with an asymptomatic pulmonary mass discovered on routine chest roentgenography was found to have substantial bilateral adrenal enlargement by abdominal computed tomography. Percutaneous adrenal aspiration biopsy showed cytologically normal adrenal glands. A diagnosis of subclinical 21-hydroxylase deficiency was established by stimulation testing with adrenocorticotropic hormone. The adrenal size and appearance by computed tomographic scanning in congenital adrenal hyperplasia and particularly in its subclinical form have not been well defined. This case demonstrates that marked adrenal enlargement can occur and may provide the only clue to the diagnosis in an asymptomatic patient without other clinical stigmata of adrenal hyperplasia.

17-alpha-Hydroxyprogesterone↗
PubMed1987

Lung bullae with air-fluid levels.

Fourteen patients in whom air-fluid levels developed in pre-existing emphysematous bullae were prospectively identified and followed. Although two patients were asymptomatic, 12 patients presented with one or more symptoms of fever, dyspnea, cough, pleuritic chest pain, and/or purulent sputum. Three patients had focal pulmonary abnormalities on physical examination; three patients had elevated leukocyte counts. Exudative fluid with an elevated leukocyte count was aspirated from the bullae of two patients; one aspirate grew Bacteroides melaninogenicus on culture. Bronchoscopy in seven patients influenced neither the diagnosis nor therapy. Thirteen patients received either oral penicillin (11 patients) or oral tetracycline (two patients). Complete resolution of symptoms and air-fluid levels on chest roentgenography occurred in all patients over two to 32 weeks (mean, 12).

Adult↗
PubMed1987

Spectrum of gas within the kidney. Emphysematous pyelonephritis and emphysematous pyelitis.

Renal emphysema is an important clinical entity that is not addressed frequently in the medical literature. The affected patients may have gas within the renal parenchyma, emphysematous pyelonephritis, or confined to the collecting system, emphysematous pyelitis. Two patients that illustrate the spectrum of this entity are described. The literature has been reviewed to determine the clinical features of each disorder and to provide a schema for diagnosis and management. Emphysematous pyelonephritis is seen primarily in diabetic patients, whereas emphysematous pyelitis is recognized most often in association with urinary tract obstruction. The diagnosis is made radiographically by demonstrating renal gas on plain abdominal roentgenography or intravenous pyelography. Location and extent of renal gas are best evaluated by computed tomographic scanning. Intraparenchymal gas usually requires nephrectomy, whereas successful therapy of gas limited to the collecting system involves medical management, with a drainage procedure when obstruction coexists.

Adult↗
PubMed1987

How to classify Raynaud's phenomenon. Long-term follow-up study of 73 cases.

Raynaud's phenomenon without an underlying cause was diagnosed in 96 consecutive patients in 1978 to 1979. Seventy-three patients were available for long-term follow-up. They were classified on initial evaluation as having primary Raynaud's phenomenon (49 patients) when no clinical, laboratory, or serologic abnormality was detected, and as having suspected secondary Raynaud's phenomenon when at least one finding was abnormal. Re-evaluation was performed in 1984 to 1985 after an average duration of Raynaud's phenomenon of 14.9 +/- 12 years. The average duration of the follow-up from initial to final evaluation was 4.7 +/- 1 years. On final evaluation, none of the 49 patients with an initial diagnosis of primary Raynaud's phenomenon had evidence of secondary Raynaud's phenomenon, whereas 14 of the 24 patients with suspected secondary Raynaud's phenomenon had a definite diagnosis. Among them, there were 13 connective tissue diseases. The study proved that Raynaud's phenomenon without an underlying cause must be followed up for more than two years, contrary to what was recommended previously, before it can be rightly diagnosed as primary Raynaud's phenomenon. Moreover, the results suggested that, in order to distinguish early primary Raynaud's phenomenon from suspected secondary Raynaud's phenomenon, a simple and noninvasive evaluation is sufficient. In this study, the evaluation included history and clinical examination, tests for antinuclear antibodies, radiography of hands, chest roentgenography, and nailfold capillary microscopy.

Adult↗
PubMed1994

Sustained improvement in functional capacity after removal of body fluid with isolated ultrafiltration in chronic cardiac insufficiency: failure of furosemide to provide the same result.

OBJECTIVES: This study was designed to investigate whether a subclinical accumulation of fluid in the lung interstitium associated with moderate congestive heart failure interferes with the patient's functional capacity, and whether furosemide treatment can promote reabsorption of the excessive fluid. BACKGROUND: In patients with moderate congestive heart failure, pulmonary overhydration may be detected by chest roentgenography even if therapy is optimized to keep the urinary output normal and to prevent weight gain and dependent edema formation. Removal of the overhydration may help define its significance. METHODS: Patients, whose regimens of digoxin, oral furosemide, and angiotensin-converting enzyme (ACE) inhibitor therapy were kept constant, were randomly allocated to receive ultrafiltration (8 cases) or an intravenous bolus of supplemental furosemide (mean dose: 248 mg; 8 cases). The amount of body fluid removed with each method approximated 1600 mL. Functional performance was assessed with cardiopulmonary exercise tests. RESULTS: Soon after fluid withdrawal by either method, the filling pressures of the two ventricles and body weight were reduced and plasma renin activity, norepinephrine, and aldosterone were augmented. After furosemide administration, hormone levels remained elevated for the next 4 days, and during this period, patients had positive water metabolism, recovery of the elevated ventricular filling pressures, and re-occurrence of lung congestion with no improvement in functional capacity. After ultrafiltration, levels of renin, norepinephrine, and aldosterone fell to below control values within the first 48 hours and water metabolism was equilibrated at a new set point (less fluid intake and diuresis without weight gain). The favorable circulatory and ventilatory adjustments consequent to the reabsorption of lung water improved the functional capacity of these patients. That may also have restored the lung's ability to clear norepinephrine, thus restraining its facilitation of renin release. The improvement continued 3 months after the procedure. CONCLUSIONS: In patients with congestive heart failure the set point of fluid balance is altered in spite of oral furosemide therapy; supplemental intravenous furosemide does not shift the set point, at least not when combined with ACE inhibition. Excessive, although asymptomatic, lung water limits the functional capacity of the patient.

Aged↗
PubMed1980

Upper genital tract changes and pregnancy outcome in offspring exposed in utero to diethylstilbestrol.

Hysterosalpingography (HSG) was performed on 267 diethylstilbestrol (DES)-exposed women. One hundred eighty-five (69%) of these women demonstrated an abnormality and 82 women had normal findings. A relationship between abnormal x-ray findings and structural abnormalities of the cervix and vaginal epithelial changes was noted. Ninety-three women on whom roentgenography was performed had a total of 144 pregnancies. The pregnancy outcome in this group of women was significantly worse than that found in a control population of 87 women who had 147 pregnancies. In addition, women with abnormal x-ray films were found to have a poorer pregnancy outcome than the group of women with normal findings. The women with abnormal x-ray films had more ectopic pregnancies, spontaneous abortions, and premature deliveries than women with normal x-ray films although these differences were not statistically significant. Significantly fewer women with abnormal findings than women with normal x-rays were delivered of term infants.

Abortion, Spontaneous↗
PubMed1982

The association between pituitary adenomas and chronic anovulation syndrome.

A cohort of 487 patients with chronic anovulation syndrome diagnosed between January 1, 1970, and January 1, 1980, were followed up to determine the frequency of pituitary adenomas among them. Of the 487 patients, four (0.8%) had pituitary adenoma. This compares with an expected number of 0.42, yielding a relative risk of 9.5 (95% confidence interval, 2.6 to 24.3). Within this cohort, the prevalence rate was 2.7% for galactorrhea and 3.3% for hyperprolactinemia. The prevalence rate of abnormal radiologic findings was 6.4% for roentgenography of the sella, 25.4% for sellar tomography, and 14.7% for computed tomography. In addition, 15 patients with pituitary adenomas and 60 control subjects were compared for prior anovulation syndrome. The relative risk of pituitary adenomas for patients with chronic anovulation syndrome in this case-control study was 24.3 (95% confidence interval, 4.9 to 120.6).

Adenoma↗
PubMed1986

Upper genital tract changes and infertility in diethylstilbestrol-exposed women.

A possible relationship of upper genital tract abnormalities in the diethylstilbestrol-exposed woman and infertility was studied. Six hundred thirty-two women had roentgenography performed. Three hundred sixty-seven of these women were not using contraception and were actively trying to become pregnant. One hundred thirty-four (36%) of these women reported difficulties conceiving for a period of 1 or more years. Seventy-three percent of these women had abnormal hysterosalpingograms. The frequency of abnormal x-ray films in the women who did conceive within 1 year was essentially the same (74%). Furthermore, a similar number of women with normal x-ray films (37%) had difficulty conceiving as did women with abnormal x-ray films (36%). When specific hysterosalpingographic abnormalities were related to the presence or absence of infertility, it was observed that the presence of a constriction of the upper uterine cavity resulted in a 2.26-times greater likelihood that a woman would not be able to conceive. If a T-shaped uterus was found in association with constriction of the upper uterine cavity, the odds ratio for inability to conceive was found to be 2.63.

Abnormalities, Drug-Induced↗
PubMed1976

Disease of the gallbladder in patients with normal cholecystograms.

In contrast with what has widely been assumed, disease of the gallbladder sometimes remains undertected by even repeated cholecystography or cholegraphy. Chronic cholecystitis, gallstones, and acalculous cholesterolosis can escape detection by roentgenography of the gallbladder. Of 4,000 patients subjected to cholecystectomy between 1962 and 1973, the cholecystogram had been normal in only two. The range of indications for the operation has since been widened. Patients with typical symptons of gallbladder disease or recurrent pancreatitis of unknown origin have been operated on despite a normal cholecystogram. Within as short a period as eight months, an additional six patients with a normal cholecstogram have been operated on at our hospitals. Treatment consisted of cholecystectomy also in the four patients in whom no stones could be palpated in the gallbladder. Gross and microscopic examination of the gallbladder in these patients revealed cholesterolosis. When last seen, seven of the eight patients were still symptom-free an average fifteen months after the operation.

Acute Disease↗
PubMed1977

Radiomanometric guides to common bile duct exploration.

The accuracy of operative cholangiograms can be increased by radiomanometry. This study identified 17 patients among 198 studied whose choledocholiths would have likely been missed by routine cholangiography. These are almost invariably small stones the fate of which would be conjectural if left behind. In the present study, if the cholangiogram was negative and the passage pressure within normal limits, calculi were seen only once. If the cholangiogram was positive for choledocholithiasis and the passage pressure elevated, stones were almost invariably found (95 per cent). Such accuracy cannot be expected from either roentgenography alone or determination solely of pressure relationships.

Adult↗