Biomedical subjects

R W Simms

Publications and source records attributed to R W Simms.

28 records · Page 2Linked to original sources

PubMed1994

Orthotopic liver transplantation for familial amyloidotic polyneuropathy.

Orthotopic liver transplantation for inborn errors of metabolism has become a standard indication for transplantation in pediatric and adult patients with alpha-1 antitrypsin deficiency, Wilson's disease and tyrosinemia, amongst several less common diseases. Familial amyloidotic polyneuropathy (FAP) is a rare autosomal dominant disease whose metabolic origin lies in an abnormal protein synthesized primarily in the liver. FAP, also discussed as the autosomal dominant form of amyloidosis, is characterized as a hereditary form of amyloidosis. It is the rarest form of amyloidosis affecting kindreds of specific ethnic backgrounds. The true incidence of this disease in the United States is not known. The mutant protein, called transthyretin or prealbumin, forms amyloid fibrils which accumulate in vital tissues ultimately leading to the patient's death. Liver transplantation for this inherited disease leads to the production of normal transthyretin protein. This theoretically should arrest the disease process. The first 5 patients in the United States with FAP who have undergone transplantation are presented.

Adult↗

PubMed1993

Established fibromyalgia syndrome and parvovirus B19 infection.

OBJECTIVE: To determine the seroprevalence of prior and persistent parvovirus B19 (B19) infection in a group of patients with fibromyalgia (FS) compared with controls. METHODS: Fifteen female patients with FS who recalled a viral prodrome (+VP) preceding the onset of FS symptoms and eleven patients with FS who did not recall any such illness (-VP) were selected from a referral practice. We excluded patients with FS who described a history of trauma prior to the onset of FS symptoms. Twenty-six female medical workers served as controls. Serum IgM and IgG anti-B19 antibodies were measured by ELISA: Polymerase chain reaction (PCR) products from serum were analyzed by dot blot hybridization for B19 DNA. Fisher's 2-tailed exact test was used to compare the proportion of positive serologies in each group. RESULTS: No patient or control had positive IgM levels. For all patients with FS, the prevalence of prior B19 infection was comparable to that of healthy controls (11/26 vs 12/26, p = 1.00) and that of the general population. No significant difference was found in the prevalence of prior B19 infection in FS + VP and FS-VP patients (8/15 vs 3/11, p = 0.25). None of the patients or controls showed evidence for persistent B19 viremia, as determined by PCR analysis. CONCLUSION: Our data do not suggest that B19 plays a pathogenic role in this population of patients with FS. Testing for IgM against B19 within 2-3 months of symptom onset may prove more helpful in further defining the role of B19 in FS.

Adult↗

PubMed1992

Fibromyalgia syndrome in patients infected with human immunodeficiency virus. The Boston City Hospital Clinical AIDS Team.

PURPOSE: To prospectively assess rheumatic manifestations of human immunodeficiency virus (HIV) disease in a municipal hospital clinic population in which intravenous drug use was the most common risk factor for HIV infection. PATIENTS AND METHODS: Patients with documented HIV infection were evaluated for rheumatic disease using a standardized questionnaire and examination. Patients with fibromyalgia were compared with HIV-infected patients without fibromyalgia and with fibromyalgia patients without known risk factors for HIV infection. RESULTS: Thirty-seven of 140 patients with HIV infection had muskuloskeletal symptoms. Three of these 37 patients had arthritis, but none had Reiter's syndrome or psoriatic arthritis. Thirty (81%) of 37 patients had chronic musculoskeletal symptoms (for 3 months or longer). Twenty of 30 patients with chronic musculoskeletal symptoms had polyarthralgia, and of those, 15 (75%) were found to have either definite or probable fibromyalgia syndrome. Therefore, fibromyalgia syndrome was found in 41% of HIV-infected patients with musculoskeletal symptoms and in approximately 11% of all HIV-infected patients. Fibromyalgia patients with HIV infection had a longer duration of HIV infection (p = 0.01) and more frequently reported past depressed mood (p = 0.001) than HIV-infected patients without fibromyalgia. Compared with 301 patients with fibromyalgia syndrome and no known risk behavior for HIV, known HIV-infected patients with fibromyalgia were more commonly male (p = 0.001) and reported current depressed mood more frequently (p = 0.0001). CONCLUSION: Few patients with arthritis were noted among HIV-infected patients who had intravenous drug use as risk behavior. By comparison, fibromyalgia syndrome appeared to be a common cause of musculoskeletal symptoms in this patient population.

Acute Disease↗

PubMed1992

Excessive fibrinolysis in amyloidosis associated with elevated plasma single-chain urokinase.

Severe bleeding resulting from excessive fibrinolysis has been observed in patients with primary amyloidosis. The authors studied a patient with this hemostatic disorder before and during therapy with epsilon-aminocaproic acid. Excessive fibrinolysis was associated with depressed plasma concentrations of coagulation Factors XII, XI, high-molecular-weight kininogen, and Factors VIII and V; and plasminogen and alpha-2-plasmin inhibitor. These deficiencies were corrected with treatment. The functional and antigenic concentrations of tissue plasminogen activator and plasminogen activator inhibitor in the patient's plasma were normal. Urokinase-type activator activity and antigen were three to five times elevated in the patient's plasma. Results of immunoprecipitation showed that single-chain urokinase-type activator was the primary urokinase-type activator species in the patient's plasma. Excessive fibrinolysis in patients with amyloidosis results from increased plasma single-chain urokinase-type activator activity.

Amyloidosis↗

PubMed1991

Bowel dysfunction in fibromyalgia syndrome.

Fibromyalgia and irritable bowel syndrome frequently coexist. In this study, we utilized a previously validated self-administered questionnaire to assess the prevalence of symptoms of bowel dysfunction and irritable bowel syndrome in 123 patients with fibromyalgia as compared to 54 patients with degenerative joint disease (DJD) and 46 normal controls. Ninety (73%) of the fibromyalgia patients reported altered bowel function as compared to 20 (37%) DJD patients and none of the normal controls (P less than 0.001). Ninety-nine patients (81%) reported normal alternating with irregular bowel pattern, and 77 (63%) had alternating diarrhea and constipation. In contrast, only 24 (44%) of DJD patients and six (13%) of controls had regular alternating with irregular bowel pattern and only 12 (22%) of the DJD patients and none of the healthy controls had alternating constipation and diarrhea (P less than 0.01). Other bowel dysfunction complaints noted in the fibromyalgia group were abdominal gas (59%), nausea (21%), diarrhea (9%), and constipation (12%). Seventy-nine (64%) fibromyalgia patients reported frequent abdominal pain that was stress-related 47% of the time. Laxative use was frequent in the fibromyalgia group (19%) and absent in the other two groups. Fifty percent of fibromyalgia patients, compared to 28% of DJD patients, felt that their bowel complaints were worse during exacerbations of their joint disease (P less than 0.05). In conclusion, patients with fibromyalgia have a high prevalence of gastrointestinal complaints that should be carefully assessed. If the diagnosis of IBS is confirmed, appropriate treatment may improve patients' symptoms, although this approach requires further study.

Adult↗

PubMed1991

Extrapulmonary thoracic restriction (hidebound chest) complicating eosinophilic fasciitis.

Eosinophilic fasciitis (EF) is an unusual disorder characterized by diffuse skin thickening and induration due to inflammation within the deep fascia; visceral involvement is generally mild or absent. A patient with biopsy-proved EF developed progressive respiratory limitation. Physical examination revealed marked induration of the thoracic integument with a severely limited chest wall excursion. Total lung capacity was 62 percent of predicted with a normal corrected Dco and maximal inspiratory force; a chest computed tomogram with thin sections showed no evidence of parenchymal lung disease. Extrapulmonary thoracic restriction ("hidebound chest") has not been previously reported to complicate EF.

Aged↗

PubMed1991

Development of preliminary criteria for response to treatment in fibromyalgia syndrome.

We developed a set of preliminary response criteria for use in future clinical trials in fibromyalgia syndrome. We determined outcome measures from a previously reported clinical trial which best distinguished patients treated with effective medication from those treated with placebo or ineffective medication, using stepwise logistic regression analysis. Several combinations of outcome measures were identified and plotted in the form of receiver operating characteristic (ROC) curves. The combination of variables possessing the greatest area under the ROC curve included (1) physician global assessment score less than or equal to 4 (0 = extremely well, 10 = extremely poorly), (2) patient sleep score less than or equal to 6 (0 = sleeping extremely well, 10 = sleeping extremely poorly), and (3) tender point score less than or equal to 14 (maximum possible tender point score equalled 20). These criteria accurately distinguished those treated with effective drug from those treated with placebo when tested in an unreported therapeutic trial of cyclobenzaprine. The criteria identified 11 of 14 patients in the amitriptyline trial and 4 of 6 patients in the cyclobenzaprine trial who attained improvement measured independently. The methodology used to define these preliminary criteria may be applied to refine the criteria as additional sensitive and clinically relevant outcomes are developed.

Amitriptyline↗

PubMed1990

High frequency of fibromyalgia in patients with chronic fatigue seen in a primary care practice.

We administered a standardized history questionnaire and performed a tender point examination on 27 patients with debilitating fatigue of at least 6 months duration, seen in a primary care practice, as well as on 20 patients with fibromyalgia. Sixteen of the 27 patients with chronic fatigue met the full criteria for the working case definition of chronic fatigue syndrome (CFS). Eight patients with chronic fatigue denied having any current persistent, diffuse musculoskeletal pain, and their tender point scores were similar to those in 10 normal control subjects. In contrast, 19 patients with chronic fatigue (70%) had persistent, diffuse musculoskeletal pain. The results of their tender point examinations were similar to those of the patients with fibromyalgia. Thus, the majority of these patients with debilitating chronic fatigue, including those who met criteria for CFS, met the historical and tender point diagnostic criteria for fibromyalgia. The presence of current musculoskeletal pain will identify those CFS patients who have fibromyalgia.

Adult↗

PubMed1988

Tenderness in 75 anatomic sites. Distinguishing fibromyalgia patients from controls.

We studied tenderness at 75 unilateral anatomic locations in 10 fibromyalgia patients and 10 normal control subjects to determine which sites best identified patients with fibromyalgia. Using a dolorimeter, the mean amount of pressure required to elicit tenderness was significantly lower in patients than in controls at 19 sites (P less than 0.001). Of the previously proposed tender points, only 2 were included in those 19 best discriminating points. Fifteen of the 19 best discriminating points were clustered in regions around the anterior shoulder, anterior chest, posterior scapula, and medial knee. The 19 sites that we have identified best separate patients with fibromyalgia from controls, although the discriminating power of these sites in other chronic pain syndromes will require further study. Examination of specific regions may be more useful clinically than the exact anatomic sites within these regions.

Adult↗

PubMed1988

Symptoms mimicking neurologic disorders in fibromyalgia syndrome.

Recent reports have not emphasized the presence of paresthesias in fibromyalgia syndrome. In our retrospective review of 161 patients with fibromyalgia we found that 84% complained of numbness or tingling at initial evaluation. Most had either bilateral upper and lower extremity or bilateral upper extremity paresthesias. None had concurrent diseases commonly associated with peripheral neuropathy. Thirty-six patients with paresthesias had electromyograms performed before the diagnosis of fibromyalgia and 32 were normal. At a second assessment performed at a mean of 25 months from time of diagnosis, 56 of these 57 patients reported current paresthesias. Paresthesias are common in fibromyalgia and may mimic a neurologic disorder, although objective abnormalities are rare. Judicious use of neurodiagnostic tests are therefore indicated in the clinical setting of fibromyalgia.

Diagnosis, Differential↗