A community-based cohort of 201 consecutive patients with primary Sjögren's syndrome in Israel: Ashkenazi patients compared with those of Sephardic descent.

OBJECTIVE: To determine the spectrum and prevalence of the varied manifestations, associated conditions and laboratory abnormalities of patients with primary Sjögren's syndrome in Israel and compare them between individuals of Sephardic and Ashkenazi descent and with data from the literature. METHODS: A retrospective study of a cohort of 201 consecutive patients diagnosed and followed at a single academic medical center. All cases were diagnosed using stringent criteria according to the American European Concensus Group including a labial minor salivary gland biopsy in all cases. RESULTS: Patients' mean age was 57 years and 84% were women. Overall, more than 98% of patients had sicca symptoms of dry eyes and mouth. About 35% of the cohort had hematological manifestations--primarily immune cytopenias, protein immunoelectrophoresis abnormalities and lymphoma. About 20% had associated neurological conditions (not only peripheral but often central nervous system) and 15% had pulmonary involvement. In addition, thyroid disease, liver disease, vascular or cutaneous manifestations, synovitis, ocular and renal disease could be found. In fact, the presenting manifestation was extraglandular or an abnormal test result in 39% of the patients. CONCLUSION: No significant differences were found in glandular or extraglandular manifestations or laboratory test results between Ashkenazi and Sephardic patients, despite their genetic differences. A negative history of sicca symptoms effectively rules out primary Sjögren's syndrome in this cohort. These symptoms may not be volunteered by patients and the large variety of extraglandular involvement patterns and associated conditions observed may dominate the patient's presentation, and mandate physicians' awareness and a high index of suspicion for a timely diagnosis.

A simple technique for minor salivary gland biopsy appropriate for use by rheumatologists in an outpatient setting.

Minor salivary gland biopsy is a potentially simple procedure with high diagnostic value used primarily in the diagnosis of Sjögren's syndrome. We summarise our experience performing a simplified biopsy procedure, which is modified to allow its use by rheumatologists and other non-surgical physicians in an outpatient setting.

Agranulocytosis in Sjögren's syndrome: two case reports and analysis of 11 additional reported cases.

OBJECTIVES: To report 2 patients who presented with agranulocytosis that was found to be immune-mediated and associated with occult primary Sjögren's syndrome (primary SS) and to identify and study similar cases reported in the literature. METHODS: Two patients encountered in 2 large medical centers over a period of 5 years were studied in detail. All reported cases of agranulocytosis in primary SS identified through a MEDLINE search were reviewed. RESULTS: Two patients presented with marked systemic symptoms alone or associated with recurrent infections. Agranulocytosis with either a pattern of maturation arrest or a hypercellular reactive bone marrow was found and was associated with "acute phase" markers, hypergammaglobulinemia, a small paraprotein peak, and high rheumatoid factor titers. A diagnosis of immune-mediated agranulocytosis associated with an occult primary SS was established and was successfully treated with intravenous immunoglobulins or prednisone. Both patients subsequently developed skin vasculitis. This rare association of agranulocytosis and Sjögren's syndrome was identified in 11 other cases and was the presenting manifestation of primary SS in 10 of 13 (77%) patients. CONCLUSIONS: Agranulocytosis should be recognized as a rare but well-established association of primary SS. Bone marrow neutrophil production may be affected, or neutrophils may be destroyed in the circulation, by both humoral and cellular immune-mediated mechanisms. Agranulocytosis or neutropenia should be added to the varied hematologic manifestations of primary SS and may be its presenting feature and an important clue to diagnosis.

Sudden sensorineural hearing loss as a first manifestation of systemic lupus erythematosus: association with anticardiolipin antibodies.

Sudden sensorineural hearing loss is a rarely reported manifestation of systemic lupus erythematosus (SLE). This condition has been most frequently seen in individuals with concomitant anticardiolipin antibody (ACL) syndrome, although a direct causal relationship remains unconfirmed. We report an unusual case of a young male with sudden unilateral hearing loss as the first manifestation of SLE. This individual was also found to be ACL positive and subsequently presented with other thrombotic manifestations compatible with this syndrome. The literature regarding this condition is reviewed and the significance of this case in fortifying the association of anticardiolipin antibodies and sensorineural hearing loss is discussed.

Cactus thorn arthritis: case report and review of the literature.

Synovitis secondary to penetrating plant thorn injuries is an infrequently reported event. Despite its wide geographic distribution, thorns from the prickly pear cactus (Optunia ficusindica) are a rare source of this type of inflammatory arthritis. We hereby present an unusual case of an individual who developed an acute monoarthritis of the knee shortly after sustaining a penetrating cactus thorn injury. The clinical and pathophysiologic features of cactus thorn arthritis are reviewed and the unusual features present in this individual are highlighted. Treatment options, with an emphasis on rapid diagnosis and therapeutic interventions, are discussed. Increased physician awareness and recognition of this unusual but not rare entity are essential as a means of improving clinical outcome.

Patient risk factors for adverse drug events in hospitalized patients. ADE Prevention Study Group.

BACKGROUND: Adverse drug events (ADEs) are common in hospitalized patients, but few empirical data are available regarding the strength of patient risk factors for ADEs. METHODS: We performed a nested case-control study within a cohort that included 4108 admissions to a stratified random sample of 11 medical and surgical units in 2 tertiary care hospitals during a 6-month period. Analyses were conducted on 2 levels: (1) using a limited set of variables available for all patients using computerized data available from 1 hospital and (2) using a larger set of variables for the case patients and matched controls from both hospitals. Case patients were patients with an ADE, and the matched control for each case patient was the patient on the same unit as the case patient with the most similar prevent length of stay. Main outcome measures were presence of an ADE, preventable ADE, or severe ADE. RESULTS: In the cohort analysis, electrolyte concentrates (odds ratio [OR], 1.7), diuretics (OR, 1.7), and medical admission (OR, 1.6) were independent correlates of ADEs. Independent correlates of preventable ADEs in the cohort analysis were low platelet count (OR, 4.5), antidepressants (OR, 3.3), antihypertensive agents (OR, 2.9), medical admission (OR, 2.2), and electrolyte concentrates (OR, 2.1). In the case-control analysis, exposure to psychoactive drugs (OR, 2.1) was an independent correlate of an ADE, and use of cardiovascular drugs (OR, 2.4) was independently correlated with severe ADEs. For preventable ADEs, no independent predictors were retained after multivariate analysis. CONCLUSIONS: Adverse drug events occurred more frequently in sicker patients who stayed in the hospital longer. However, after controlling for level of care and preevent length of stay, few risk factors emerged. These results suggest that, rather than targeting ADE-prone individuals, prevention strategies should focus on improving medication systems.

Music therapy and chiropractic: an integrative model of tonal and rhythmic spinal adjustment.

There is a philosophical basis for the integration of treatment using music therapy and chiropractic. Perception is intimately linked to the nervous system. A relationship between spinal integrity and consciousness does exist. We can see that as spinal distortions diminish and awareness increases, there is a natural attraction toward the higher or more loving state of consciousness. Rhythms of healing and suffering are a key concept in combining music therapy with chiropractic manipulation. Donald Epstein's conceptualization of the rhythmic stages of consciousness corresponding to prescribed physiological patterns serves as a starting point for the use of rhythm in the healing process. Using interactive music, the music therapist can help facilitate a change in the patient's physical or emotional state. This occurs when the practitioner establishes an initial connection or musical validation of the patient's emotional state and assists the healing process by improvising supportive music while suggesting possibilities for resolution. We believe that the power of music can be used as a significant tool in chiropractic work to aid individuals in their healing process.

Limulus amebocyte lysate assay for detection of endotoxin in patients with sepsis syndrome. AMCC Sepsis Project Working Group.

Clinical predictions alone are insufficiently accurate to identify patients with specific types of bloodstream infection; laboratory assays might improve such predictions. Therefore, we performed a prospective cohort study of 356 episodes of sepsis syndrome and did Limulus amebocyte lysate (LAL) assays for endotoxin. The main outcome measures were bacteremia and infection due to gram-negative organisms; other types of infection were secondary outcomes. Assays were defined as positive if the result was > or = 0.4 enzyme-linked immunosorbent assay units per milliliter. There were positive assays in 119 (33%) of 356 episodes. Assay positivity correlated with the presence of fungal bloodstream infection (P < .003) but correlated negatively with the presence of gram-negative organisms in the bloodstream (P = .04). A trend toward higher rates of mortality in the LAL assay-positive episodes was no longer present after adjusting for severity. Thus, results of LAL assay did not correlate with the presence of bacteremia due to gram-negative organisms or with mortality after adjusting for severity but did correlate with the presence of fungal bloodstream infection.

Tuberculosis testing. Physician attitudes and practice.

OBJECTIVE: To assess physician agreement with and adoption of American Academy of Pediatrics' (AAP) recommendations on tuberculosis screening in children. DESIGN AND PARTICIPANTS: Survey of a random sample of 1272 community pediatricians and family physicians (excluding academic institutions) in 4 mid-Atlantic states and the District of Columbia. RESULTS: The response rate was 66%. Seventy-five percent of the respondents were aware of the 1994 AAP screening recommendations. Most (64%) test children at low risk periodically (at age 1 year, preschool age, and adolescent age), and 81% test children at high risk annually. Eighty-one percent of the respondents estimated that 10% or less of their patients were at high risk for tuberculosis. Most use patient-specific factors, geographic or community prevalence, or both as principal criteria to determine risk. Eighty-seven percent used multiple-puncture tests in 1993; this declined to 55% in 1994. Multiple-puncture tests are still used exclusively by 29% of the respondents. Ninety-one percent of those using multiple-puncture tests and 19% of those using the Mantoux test allow parents to read the test reaction. Forty-six percent of the respondents believed that if a return visit was required for reading the test reaction, 50% or less of their patients would return. Only 22% of the respondents adhere to the 1994 AAP recommendations regarding frequency, testing method, and reading the test reaction. Factors associated with adherence to AAP recommendations included physician and practice characteristics and knowledge of AAP recommendations. CONCLUSIONS: Physician practice of tuberculosis testing varies widely; most do not adhere to the 1994 AAP recommendations. Most physicians vary the frequency and testing method based on a patient's risk status. The use of multiple-puncture tests has declined, although they are still widely used. Accurate reading of screening tests remains a major concern.

Association of serum beta-hCG levels with myosalpingeal invasion and viable trophoblast mass in tubal pregnancy.

OBJECTIVE: To test the hypothesis that myosalpingeal invasion and viable trophoblast mass are associated with serum beta-hCG levels in tubal-ampullary pregnancy. METHODS: Twenty-seven salpingectomy specimens of tubal-ampullary pregnancies were assessed for the presence or absence of myosalpingeal invasion. The mass of viable trophoblast was quantified in terms of the number of high-power fields (x400) occupied. The cases were stratified into three groups: small, less than one field; medium, one or two fields; and large, more than two fields and/or embryo present. Beta-hCG was measured before the procedure (mIU/mL, Third International Standard). RESULTS: The mean (+/- standard error of the mean) beta-hCG level for the nine cases exhibiting myosalpingeal invasion was significantly higher than for the 18 cases without invasion (13,665 +/- 2986 versus 2169 +/- 870 mIU/mL; P = .0001). Beta-hCG levels greater than or equal to 5400 mIU/mL predicted myosalpingeal invasion in eight of nine cases (positive predictive value 89%). In contrast, levels less than 5400 mIU/mL were associated with lack of myosalpingeal invasion in 17 of 18 cases (negative predictive value 94%). The volume of trophoblast mass correlated with both beta-hCG levels (r = 0.647, P = .0003) and myosalpingeal invasion (r = 0.735, P = .0001). There was no invasion in the 13 cases in the group with small trophoblast mass, whereas two of five cases in the medium-mass group displayed myosalpingeal invasion. In this group, the mean beta-hCG for cases with myosalpingeal invasion was higher than in the cases without invasion (16,917 +/- 117 versus 3799 +/- 1094 mIU/mL; P = .003). In the group with large trophoblast mass, seven of nine specimens showed myosalpingeal invasion. CONCLUSION: Both myosalpingeal invasion and viable trophoblast mass correlate positively with serum levels of beta-hCG. Myosalpingeal invasion is highly likely when beta-hCG levels reach 5400 mIU/mL.

Diagnosis and treatment of rheumatoid arthritis.

Rheumatoid arthritis is a chronic, inflammatory, connective-tissue disease that has well-defined clinical and laboratory characteristics. The management of rheumatoid arthritis requires an aggressive multidisciplinary treatment combining both pharmacologic and nonpharmacologic measures. Using this approach, most patients with rheumatoid arthritis can maintain their functional capacity while having their pain and suffering relieved.

New incision for penile surgery.

We present a recent modification of the surgical incision used for patients with Peyronie's disease of the dorsal penile shaft and/or who require a concomitant complete dorsal vein ligation procedure. This approach spares the penile skin from trauma and torsion, and allows for improved access to the more proximal penile shaft. Our experience to date has demonstrated no evidence of increased morbidity with excellent cosmetic results.

Upper tract transitional cell carcinoma following treatment of superficial bladder cancer with BCG.

Eighty-two consecutive patients treated with intravesical bacillus Calmette-Guerin (BCG) for recurrent superficial bladder tumors were evaluated for development of metachronous upper tract tumors (UTT). All patients had normal upper tract studies within three months of starting BCG treatment. With a median follow-up of sixty-two months (range 25 to 124), 11 patients (13.4%) were found to have UTT. The median interval between initiation of BCG therapy and diagnosis of the UTT occurrence was thirty-eight months (range 7 to 110). All patients were asymptomatic when the UTT was diagnosed. An abnormal surveillance intravenous or retrograde pyelogram was the method of diagnosis in 8 patients. Positive cytology alone directed diagnoses in 2 patients, and 1 patient was diagnosed in the workup of hematuria. Overall upper tract cytology was positive in 7 of 11 patients. Nephroureterectomy was performed in 9 patients and 2 had ureteroscopic biopsy and fulguration. Median follow-up after treatment of UTT was thirty-two months (range 3 to 80). UTT pathologic stage was Pa in 2 patients, P1 in 1 patient, and P2 or higher in 8 patients. Distant metastasis developed in 7 patients, 2 patients have recurrent superficial bladder tumors, and 2 patients are free of disease. The reported incidence in the literature for UTT tumors in patients with previous superficial or muscle invasive tumors ranges from 1.6 percent to 8.5 percent. The 13.4 percent incidence of UTT in the present study demonstrates the increased risk for patients in this series who were selected for BCG therapy. These risk factors include high tumor grade, associated carcinoma in situ (CIS), multiple tumors, T1 tumors, and failure of prior intravesical therapy. The fact that all patients were asymptomatic at the time of diagnosis of UTT emphasizes the importance of long-term periodic surveillance with radiographic and cytologic studies of the upper tracts for patients with similar risk factors.

Reevaluation of prostate biopsy after definitive radiation therapy.

We have previously reported (1987) that a positive biopsy from a clinically normal prostate eighteen months or more after interstitial Iodine 125 or external beam irradiation predicted disease progression. In the present study, all biopsies were reexamined by the same pathologist (LEL) and correlated with long-term patient status. Of twenty-six positive biopsy specimens, twenty-two were reconfirmed as positive and four were reassigned to a negative diagnosis (false positive = 15%). Seventy-two of seventy-seven negative specimens were available for reexamination and seventy were reconfirmed as negative while two were reassigned to a positive diagnosis (false negative = 2%). A statistically higher incidence of local and/or distant failure for patients with positive biopsy specimens compared with patients with negative biopsy specimens was again confirmed (p = < 0.001). However, there is a group of patients with a positive biopsy (17%) who remain clinically free of disease at greater than ten years of follow-up. Therefore, a positive biopsy is not an absolute indication of imminent failure. Our results demonstrate the technical difficulty and potential error in interpreting prostate biopsies after radiation therapy. Therapeutic decisions should be based not only on biopsy histology but must also weigh the patient's initial tumor stage and grade, current clinical examination, PSA level, age, and health.