Association between scleroderma, renal cell carcinoma and membranous nephropathy.

We report a patient with scleroderma, renal cell carcinoma (RCC) and membranous nephropathy (MN). Certain clinical and laboratory features suggested that RCC caused or enhanced the other two conditions. A 55-year-old man developed scleroderma which progressed rapidly during its first 2 years with development of hypertension and acute renal failure, peak serum creatinine (SCr) 327 micromol/l (3.7 mg/dl) and partial improvement of the renal function (SCr 239 micromol/l or 2.7 mg/dl) after initiation of an angiotensin converting enzyme inhibitor. He subsequently developed nephrotic syndrome (urine protein excretion 9 gm/24-h) and progressive renal failure, with SCr 469 +/- 18 micromol/l (5.3 +/- 0.2 mg/dl). An anti-nuclear mitotic apparatus protein (NUMA) antibody, which is uncommon in scleroderma but has been linked to certain malignancies, was found in his serum. A left upper pole RCC was removed by heminephrectomy. MN was found in the renal parenchyma adjacent to the excised tumor. In the 3.5 years following surgery, the clinical manifestations of scleroderma have been arrested while the medications prescribed for this condition have been greatly reduced. Proteinuria is consistently less than 1 gm/24-h and 42 months after surgery serum creatinine was 256 micromol/l (2.9 mg/dl). Nutrition has also improved. Although this case may represent chance occurrence of three uncommon diseases (scleroderma, RCC, MN) in the same individual, the sustained improvement of the manifestations of scleroderma and MN after resection of the RCC contrasted to the rapid course of these conditions until the surgery, and the presence in the patient's serum of an autoantibody which is uncommon in patients with scleroderma, but has been linked to malignancy, suggest a pathogenetic relationship between the three conditions.

Sneddon's syndrome: a cause of cognitive decline in young adults.

OBJECTIVE: To review the clinical, psychometric, laboratory, and radiologic findings of 6 patients with Sneddon's syndrome (SS) who presented with cognitive dysfunction rather than stroke. DESIGN AND METHODS: Case series. All patients fulfilled were diagnosed as SS based on the co-occurrence of livedo racemosa and neurologic disease. Patients presenting with clinical stroke were excluded. RESULTS: Patients presented with cognitive complaints at an early age and all noted skin lesions from 6 months to 10 years before onset of cognitive symptoms. Associated systemic disorders included hypertension and seizures. Laboratory evidence of a hypercoagulable condition was identified in 4 of 6 cases. Brain MRI scans demonstrated atrophy, especially in parieto-occipital regions and cerebral blood flow on brain SPECT scan was reduced in a similar distribution. CONCLUSION: Patients with SS can develop dementia without antecedent clinical stroke. While the specific pathogenic mechanism of dementia in SS remains speculative, the disease predominantly injures brain tissue in vascular "watershed" territories.

Equal aspiration rates in gastrically and transpylorically fed critically ill patients.

OBJECTIVES: To determine the difference in aspiration rates between gastrically and transpylorically fed patients in the intensive care unit. DESIGN: A prospective controlled study of critically ill patients randomized to receive either a gastrically placed feeding tube or a transpylorically placed feeding tube. SETTING: University teaching hospital's medical intensive care unit. The study was conducted over 14 months. PATIENTS: Fifty-four critically ill subjects (with an overall 40% mortality) with similar baseline age, severity of illness, and nutritional needs requiring enteral nutrition, with 51 completing the study. INTERVENTIONS: All feeds were tagged with technetium-99m radiolabeled sulfur colloid, and the pulmonary secretions or lungs of each patient were scanned on a daily basis to determine whether aspiration had occurred. Patients were fed according to their assigned tube placement which was verified daily by continuous electromyography. MEASUREMENTS AND RESULTS: Of 27 gastrically fed patients 2 (7%) had evidence of scanned feed in pulmonary secretions or the lung, compared to 3 of 24 (13%) transpylorically fed patients (n.s.). Clinical suspicion of aspiration was insensitive and detected only 60% of isotopically documented aspirations with a positive predictive value of 27%. CONCLUSION: There was no difference in aspiration rates between gastrically and transpylorically fed critically ill patients.

Evaluation of coronary subclavian steal syndrome using sestamibi imaging and duplex scanning with observed vertebral subclavian steal.

Coronary subclavian steal is defined as retrograde blood flow from the myocardium through the internal mammary artery graft, secondary to a proximal subclavian artery stenosis. The incidence of this syndrome in patients undergoing internal mammary artery grafts for coronary artery bypass is estimated to be 0.44%. Angiography remains the definitive diagnostic test for confirming this condition. We describe a noninvasive method for evaluating coronary subclavian steal syndrome in a 57-year-old man, with a 50-55% subclavian stenosis confirmed by angiography. Noninvasive evaluation using duplex scanning demonstrated normal vertebral artery blood flow. Technetium 99m-sestamibi (99mTc) imaging confirmed a fixed anterolateral defect. When left-arm isometric exercise was employed, retrograde vertebral artery blood flow was observed by Doppler imaging. A repeat 99mTc-sestamibi study documented an increase in tracer distribution in the anterolateral defect confirming reperfusion of the myocardium through the left internal mammary artery graft. The use of duplex scanning and 99mTc-sestamibi may serve as an adjunct in evaluating coronary subclavian steal syndrome as well as documenting transient vertebral subclavian steal in this patient population.

Thyroid palpation versus high-resolution thyroid ultrasonography in the detection of nodules.

Detection of thyroid nodules by physical examination and high-resolution ultrasonography was compared using small groups of blinded, experienced physician examiners working with a sample of 2441 persons from Estonia, most of whom were Chernobyl nuclear reactor clean-up workers. A random subsample of 113 (5%) persons was subjected to triple control examinations with both physical examination and high-resolution ultrasonography. Positive high-resolution ultrasonographic findings were considerably more reproducible among different observers than were positive physical examination findings. Agreement between methods was poor. Nodules were found in 169 (6.9%) subjects by physical examination and in 249 (10.2%) subjects by high-resolution ultrasonography. Physical examination found only 53 (21%) of the 249 nodules found by high-resolution ultrasonography. High-resolution ultrasonography did not confirm the existence of 115 (68%) of the 169 nodules found by physical examination. Only 6.4% of nodules less than 0.5 cm in diameter, as based on high-resolution ultrasonographic results, were detected by physical examination. Physical examination detection improved with increasing nodule size but was still only 48.2% for nodules larger than 2 cm. Physical examination was relatively effective in detecting nodules in the isthmus of the thyroid gland but much less so for nodules in the upper pole of the gland. Clinical evaluation and epidemiologic studies of nodular thyroid disease stand to benefit from the greater sensitivity and specificity of ultrasonographic examinations.

192IrCl acid skin burn: case report and review of the literature.

A worker was contaminated following a chemical explosion that splashed an HNO3 radioactive solution containing approximately 180 MBq (5 mCi) 192Ir onto the left side of his face. Initial efforts reduced the contamination at least fivefold. Removal of a patch of contaminated hair was necessary. Most of the contamination was fixed to the skin; only a small amount of contamination was absorbed.

Thyroid nodularity and cancer among Chernobyl cleanup workers from Estonia.

Thyroid examinations, including palpation, ultrasound and, selectively, fine-needle aspiration biopsy, were conducted on nearly 2,000 Chernobyl cleanup workers from Estonia to evaluate the occurrence of thyroid cancer and nodular thyroid disease among men with protracted exposure to ionizing radiation. The examinations were conducted in four cities in Estonia during March-April 1995, 9 years after the reactor accident. The study population was selected from a predefined cohort of 4,833 cleanup workers from Estonia under surveillance for cancer incidence. These men had been sent to Chernobyl between 1986 and 1991 to entomb the damaged reactor, remove radioactive debris and perform related cleanup activities. A total of 2,997 men were invited for thyroid screening and 1,984 (66%) were examined. Estimates of radiation dose from external sources were obtained from military or other institutional records, and details about service dates and types of work performed while at Chernobyl were obtained from a self-administered questionnaire. Blood samples were collected for assay of chromosomal translocations in circulating lymphocytes and loss of expression of the glycophorin A (GPA) gene in erythrocytes. The primary outcome measure was the presence or absence of thyroid nodules as determined by the ultrasound examination. Of the screened workers, 1,247 (63%) were sent to Chernobyl in 1986, including 603 (30%) sent in April or May, soon after the accident. Workers served at Chernobyl for an average of 3 months. The average age was 32 years at the time of arrival at Chernobyl and 40 years at the time of thyroid examination. The mean documented radiation dose from external sources was 10.8 cGy. Biological indicators of exposure showed low correlations with documented dose, but did not indicate that the mean dose for the population was higher than the average documented dose. Ultrasound examinations revealed thyroid nodules in 201 individuals (10.2%). The prevalence of nodules increased with age at examination, but no significant associations were observed with recorded dose, date of first duty at Chernobyl, duration of service at Chernobyl, building the sarcophagus or working on the roof of neighboring buildings or close to the damaged reactor. Nodularity showed a nonsignificant (p(1) = 0.10) positive association with the proportion of lymphocytes with chromosome translocations, but associations with the frequency of variant erythrocytes in the GPA assay were weak and unstable (p(1) > or = 0.46). The majority of fine-needle biopsies taken on 77 study participants indicated benign nodular disease. However, two cases of papillary carcinoma and three benign follicular neoplasms were identified and referred for treatment. Both men with thyroid cancer had been sent to Chernobyl in May of 1986, when the potential for exposure to radioactive iodines was greatest. Chernobyl cleanup workers from Estonia did not experience a markedly increased risk of nodular thyroid disease associated with exposure to external radiation. Possible reasons for the apparent absence of effect include low radiation doses, the protracted nature of the exposure, errors in dose measurement, low sensitivity of the adult thyroid gland or the insufficient passage of time for a radiation effect to be expressed.

Fourier phase analysis of first-pass data: noninvasive detection of pulmonary sequestration.

Nuclear medicine Fourier phase analysis of first-pass data was used to evaluate blood flow to cystic lung masses in two children suspected of having pulmonary sequestrations. In both cases, the Fourier images provided a rapid, noninvasive, inexpensive analysis of the blood supply to the masses and permitted identification of the masses as pulmonary sequestrations. The analysis does not depend on the location of the mass and demonstrates the location of systemic rather than pulmonic arterial blood supply to the mass. In one case, Fourier analysis identified a second source of systemic blood supply not visible with other imaging modalities. Preoperative assessment of a cystic lung mass using Fourier analysis enables noninvasive classification of the mass as a pulmonary sequestration with systemic blood supply and aids the surgeon in resection.

MR 'hot nose sign' and 'intravascular enhancement sign' in brain death.

Three cases of MR with gadopentetate dimeglumine in patients diagnosed with cerebral death are presented. Observation of an MR "hot nose sign" and an "intravascular enhancement sign" provided additional imaging support in the clinical diagnosis of brain death. The MR findings in brain death include: 1) transtentorial and foramen magnum herniation, 2) absent intracranial vascular flow void, 3) poor gray matter/white matter differentiation, 4) no intracranial contrast enhancement, 5) carotid artery enhancement (intravascular enhancement sign), and 6) prominent nasal and scalp enhancement (MR hot nose sign). Additional modalities for confirming brain death are discussed.

Persistence of positive dialysate cultures after apparent cure of CAPD peritonitis.

Clinical features, diagnosis and outcomes of persistently positive dialysate culture (PPDC) after apparent cure of continuous ambulatory peritoneal dialysis (CAPD) peritonitis were investigated in 16 PPDC episodes observed in 16 elderly (age 62 +/- 8 years) men who had been on CAPD for 14 +/- 9 months. Seven patients (46.7%) were diabetic. Peritonitis was caused by S. aureus in 14 cases and S. epidermidis in 2 cases. Preexisting or simultaneous infectious foci were present in 15 cases, exit-site infection in 5, tunnel infection in 13, and intra-abdominal abscess in 2 cases. Indium scans were positive in 6/9 cases (67%). Two patients died with the peritoneal catheter in situ, one from intercurrent myocardial infarction and one from S. aureus sepsis with pneumonia. In another 14 cases the peritoneal catheters were removed because of either tunnel abscess (8 cases) or peritonitis recurrence (6 cases). PPDC following apparent cure of CAPD peritonitis is almost always associated with exit-site, tunnel, or intra-abdominal abscess and leads invariably to catheter loss. Associated mortality is substantial.

The two-view radionuclide ventriculogram. Advantages of the LPO view.

This report is a prospective study of 33 male patients who underwent both contrast ventriculography (CVG) and radionuclide ventriculography (RVG) within a 24-hour period. Expert, blinded observers graded the left ventricle's regional wall motion (RWM) in the left anterior descending (LAD), left circumflex (LCx), and posterior descending arterial (PDA) distributions on right anterior oblique (RAO), and left anterior oblique (LAO) CVGs, and on anterior (ANT), LAO, 70 degrees left anterior oblique (LAO70), and left posterior oblique (LPO) RVGs. When statistically compared with CVG RWM standard data, RVG studies composed of LAO and LPO views were equal to the RVG studies composed of ANT, LAO, and LAO70 views in assessment of the LAD and LCx distributions. The RVG with LAO and LPO views was superior to the RVG with ANT, LAO, LAO70 in the detection of the posterior descending artery RWM. The authors conclude that accurate assessment of RWM is efficiently performed with the RVG composed of LAO and LPO views.

Osteomyelitis: sensitivity of 0.064 T MRI, three-phase bone scanning and indium scanning with biopsy proof.

We compared the ability of magnetic resonance imaging (MRI) using a 0.064 T permanent magnet, three-phase bone scanning, and indium-labeled white blood cell (111In-WBC) scanning, to diagnose osteomyelitis. Twenty-three patients underwent biopsy. All patients were examined at presentation with all three modalities. Sensitivities for each modality were calculated using biopsy as a gold standard. The results were 72% for MRI, 68% for bone scan, and 45% for 111In-WBC. Specificities were not calculated because of lack of negative biopsies. MRI was as sensitive as bone scanning in the diagnosis of osteomyelitis. All modalities had lower than previously reported sensitivities for imaging osteomyelitis.

Periarticular tumoral calcinosis and hypercalcemia in a hemodialysis patient without hyperparathyroidism: a case report.

We present a case of a 58-yr-old male to illustrate the scintigraphic, roentgenographic, clinical, and pathologic features of periarticular tumoral calcinosis that occurred in a hemodialysis patient. Soft-tissue calcifications developed 3 yr after onset of hemodialysis, became progressively larger during the ensuing five years, and culminated in voluntary withdrawal from dialysis because of the extreme discomfort and lack of mobility that resulted from the calcinosis. Histologically, an aplastic disorder was present with very low bone formation. On bone scintigraphy, intense calcium uptake in soft tissues implied that it was metabolically active. We hypothesize that this high metabolic activity contributed to the persistent hypercalcemia observed during the patient's last year of life.

Paraprosthetic cecal fistula: CT demonstration--a case report.

A case in which repeated infection defied diagnosis after aortobifemoral bypass graft is reported. The key investigation proved to be computed tomography (CT), which detected the right limb of the graft penetrating the cecum. This is a complication of graft surgery that has not been previously reported.

SPECT gallium-67 scanning in early human immunodeficiency virus (HIV) infection. Failure of scanning abnormalities to correlate with immunologic staging.

The use of gallium scanning in the treatment of patients with AIDS has been well described. In this study, 27 HIV-infected patients (Walter Reed Staging Classification I-V) with normal chest roentgenograms were evaluated to determine the occurrence of thoracic gallium abnormalities in early HIV infection. SPECT was used for gallium scanning. Patients received gallium injection on day 1 and bronchoalveolar lavage on day 2, and scanning was performed on day 3. Twenty-eight scans in 27 patients were performed. Mean nodal SPECT activity was compared with corresponding values for lung parenchyma, bronchoalveolar lavage cell counts, and peripheral blood T4 lymphocyte counts. No relationship between nodal and parenchymal activity and cell counts was observed. Although visual scan interpretation was unaffected, gallium activity was significantly increased in the region of bronchoalveolar lavage compared with uptake in other lung regions. It is concluded that SPECT gallium scanning demonstrates significant gallium avidity in recently lavaged lung areas, although no impact on visual scan interpretation was seen. Further, no correlation was seen between the degree of nodal uptake and the immunologic status of these patients with early HIV infection.