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Biomedical subjects

E Gertner

Publications and source records attributed to E Gertner.

At least 19 recordsLinked to original sources

Treatment with sildenafil for the healing of refractory skin ulcerations in the antiphospholipid syndrome.

Nonhealing skin ulceration is a cutaneous manifestation of the antiphospholipid syndrome (APS) and is associated with thrombosis of small dermal vessels. Numerous therapies have been described but are either ineffective or require a prolonged course of treatment with potential complications. We describe the efficacy of sildenafil, a phosphodiesterase inhibitor, in the treatments of such ulcers.

Antiphospholipid Syndrome↗

Antibodies to beta 2-glycoprotein I and cardiolipin with symptoms suggestive of systemic lupus erythematosus in parvovirus B19 infection.

Parvovirus B 19 infection may mimic systemic lupus erythematosus (SLE) in its clinical presentation. Serological abnormalities during acute infection have been reported, including positive antinuclear antibodies and anti-doublestranded DNA antibodies. We describe a patient who, in addition to the clinical and laboratory findings suggestive of SLE, was found to have antibodies to cardiolipin and beta2 glycoprotein I. The symptoms resolved and the antibodies disappeared over a 9 month period. This report highlights the striking similarity between SLE and parvovirus B19 infection and the potential difficulty in distinguishing between the diseases.

Adult↗

Diffuse alveolar hemorrhage in the antiphospholipid syndrome: spectrum of disease and treatment.

OBJECTIVE: To describe clinical manifestations, laboratory findings, and treatment of patients with the antiphospholipid syndrome (APS) who develop diffuse alveolar hemorrhage. METHODS: Diffuse alveolar hemorrhage is an occasionally reported manifestation of the APS. The diagnosis, however, may be overlooked or manifestations attributed to another disease process. Seven episodes in 5 patients with primary APS were identified and retrospectively reviewed for presenting symptoms and signs, laboratory findings, and response to treatment. RESULTS: The severity of the condition varies, and diffuse alveolar hemorrhage may be the initial manifestation of APS. Patients may present with symptoms ranging from cough, dyspnea, and fever with or without hemoptysis, to symptoms of acute respiratory failure. Hypoxemia and anemia are usually present. Other causes need to be excluded. Bronchoscopy and bronchoalveolar lavage with or without biopsy often aid in confirming the diagnosis. The pathologic abnormality appears to be microvascular thrombosis with or without capillaritis. Treatment with corticosteroids usually leads to marked improvement. CONCLUSION: Patients with APS may present with diffuse alveolar hemorrhage resulting in mild to life threatening symptoms. Prompt and thorough evaluation to confirm the diagnosis and treatment with corticosteroids usually leads to rapid improvement. The clinical setting will dictate whether other therapies such as immunosuppressive agents or intravenous immunoglobulin are required.

Adult↗

Digital necrosis in acquired immune deficiency syndrome vasculopathy treated with recombinant tissue plasminogen activator.

Widespread digital ischemic changes and gangrene of the hands and feet is an uncommon but dramatic presentation in patients with human immunodeficiency virus (HIV) infection. We describe a patient in whom these clinical findings were associated with elevated serum endothelin levels. Because endothelin may affect the fibrinolytic system, we elected to treat with tissue plasminogen activator (tPA), which resulted in salvage of tissue of the fingers and toes. Patients with HIV infection with widespread ischemic necrosis and gangrene may require treatment with corticosteroids (in the event of possible vasculitis), thrombolytic agents (for the thrombotic component), or both, unless there are contraindications to either.

Acquired Immunodeficiency Syndrome↗

Oral administration of an easily prepared solution of injectable methotrexate diluted in water: A comparison of serum concentrations vs methotrexate tablets and clinical utility.

OBJECTIVE: To investigate whether the injectable formulation of methotrexate (MTX) given as an easily prepared oral solution of MTX diluted in water results in serum concentrations similar to those obtained with MTX tablets; to describe an easy and safe method of dispensing the drug. METHODS: Six patients (5 women, 1 man) with rheumatoid arthritis were given 10 mg of liquid MTX orally. The liquid was prepared by diluting 0.4 ml of the injectable formulation of MTX (50 mg/2 ml) in 8 ounces of water. One to 2 weeks later these patients were given 10 mg of MTX in the tablet form. MTX serum concentrations were determined using a fluorescence polarization immunoassay. The area under the concentration vs time curve (AUC), maximum concentration (Cmax) and the time to reach maximum concentration (tmax) were determined from the resulting concentration vs time curves. RESULTS: There was no statistical difference in the variables measured (AUC, Cmax, tmax), demonstrating comparable concentrations with these 2 methods of MTX administration. Patients found the medication easy to administer, potential hazards with the use of needles were avoided, and the cost of the drug was greatly decreased. CONCLUSION: The administrator of this easily prepared MTX solution is an alternative to the conventional administration of MTX tables, and may be of particular benefit in patients with financial limitations.

Administration, Oral↗

Complications resulting from the use of Chinese herbal medications containing undeclared prescription drugs.

OBJECTIVE: Many patients with chronic disease use alternative therapies. Our objective was to investigate complications resulting from the use of Chinese herbal medications containing undeclared prescription drugs, and to analyze these pills. METHODS: Medical records of 5 patients with complications were reviewed. Pills from symptomatic and asymptomatic individuals were analyzed for possible content of undeclared prescription drugs. RESULTS: All pills analyzed contained mefenamic acid and diazepam. Complications related to the presence of these substances included, among others, massive gastrointestinal bleeding. CONCLUSION: Chinese herbal medications may contain undeclared prescription drugs including nonsteroidal antiinflammatory drugs and benzodiazepines.

Adult↗

Systemic therapy with fibrinolytic agents and heparin for recalcitrant nonhealing cutaneous ulcer in the antiphospholipid syndrome.

Skin ulceration is a cutaneous manifestation of the antiphospholipid syndrome (APS) and is associated with thrombosis of small dermal vessels. Numerous therapeutic agents have been used but are often ineffective. We describe the efficacy of heparin and fibrinolytic agents [urokinase and tissue plasminogen activator (tPA)] in the treatment of longstanding nonhealing cutaneous ulcers. In one patient, heparin plus low dose tPA resulted in healing. In another patient, treatment first with urokinase and heparin, and subsequently with tPA alone, resulted in healing. When the ulcer recurred secondary to severe peripheral edema, tPA plus heparin led to complete resolution of the ulcer. This suggests that fibrinolytic therapy and/or heparin may be useful in other recurrent thrombotic manifestations of the APS as well. However, it must be emphatically stressed that since life threatening adverse reactions can occur secondary to hemorrhage, this treatment should be undertaken only after extensive evaluation and close monitoring of the coagulation status.

Antiphospholipid Syndrome↗

Pulmonary capillaritis, alveolar hemorrhage, and recurrent microvascular thrombosis in primary antiphospholipid syndrome.

Pulmonary manifestations of the antiphospholipid syndrome (APS) include pulmonary hypertension, pulmonary embolism, and in patients with the catastrophic APS, respiratory insufficiency associated with diffuse pulmonary infiltrates. There are few descriptions of pathological findings associated with these pulmonary complications. We document pulmonary capillaritis, recurrent microvascular thrombosis and alveolar hemorrhage in patients with APS. These patients have a wide spectrum of clinical presentations, ranging from recurrent episodes of fever, minimal hemoptysis, and mild dyspnea, to respiratory insufficiency requiring mechanical ventilation. Pulmonary involvement can occur alone or simultaneously with other manifestations of APS and may range from mild to severe. Pulmonary capillaritis and alveolar hemorrhage are complications of APS, and whether they are causally related to recurrent thromboembolism and pulmonary hypertension remains undetermined.

Adult↗

Intracardiac mural thrombus mimicking atrial myxoma in the antiphospholipid syndrome.

A wide spectrum of cardiac involvement including valvular lesions, myocardial infarction and myocardial dysfunction has been reported in patients with antiphospholipid antibodies (aPL), suggesting that cardiac manifestations may be part of the antiphospholipid syndrome (APS). We describe 3 patients (2 with primary APS and one with APS and SLE) who were found to have right atrial masses by echocardiography (transthoracic and/or transesophageal) and/or angiography, which were felt preoperatively to be atrial myxomata. Pathological examination of resected material showed only organized thrombus with calcification. We describe outcome 12 months to 7 years after resection of thrombus and document possible recurrence in one patient after 7 years. Intracardiac mural thrombus may be an additional cardiac manifestation of the APS, and presents considerable diagnostic confusion in its differentiation from atrial myxomata.

Adolescent↗

Massive soft tissue calcification causing complete loss of extensor tendon function in renal failure.

Extraskeletal soft tissue calcifications occur commonly in patients with uremia receiving dialysis. Rarely, a large tumoral calcinosis-like mass may develop. A patient receiving chronic ambulatory peritoneal dialysis for only 7 months developed a tumoral calcinosis-like mass that encased the extensor tendons of his wrist with loss of extensor tendon function, initially suggesting extensor tendon rupture. Surgical debridement restored tendon function. Tumoral calcinosis-like lesions are uncommon, but may cause limitation of joint movement, pain or ulceration through the skin. Measures aimed at controlling factors contributing to soft tissue calcification should be undertaken in any event whether surgery is required or not.

Calcinosis↗

Absent neutrophil alkaline phosphatase in the eosinophilia myalgia syndrome associated with L-tryptophan use.

The clinical constellation of leukocytosis, thrombocytosis, and low or absent stainable neutrophil alkaline phosphatase (NAP) is considered characteristic of chronic myelogenous leukemia (CML). CML with eosinophilic differentiation (eosinophilic leukemia) is well described, and leukemia and other clonal hematologic malignancies are associated with the syndrome of eosinophilic fasciitis. We describe leukocytosis, thrombocytosis, eosinophilia, mild basophilia, and absent stainable NAP, initially suggesting the diagnosis of CML in a patient with the eosinophilia myalgia syndrome associated with L-tryptophan use, a condition resembling eosinophilic fasciitis. Cytogenetic and molecular genetic studies failed to demonstrate a clonal proliferation of eosinophils.

Adult↗

HLA antigens and nailfold capillary microscopy studies in patients with insulin dependent and noninsulin dependent diabetes mellitus and limited joint mobility.

We investigated the HLA status of patients with diabetes associated with limited joint mobility and microvascular complications. An increased frequency of HLA-B8, DR3 and DR4 in patients with insulin dependent diabetes mellitus (IDDM) compared to controls and patients with noninsulin dependent diabetes mellitus (NIDDM) was confirmed. HLA antigen DQw1 was detected less frequently in patients with IDDM and was negatively associated with limited joint mobility and retinopathy. Limited joint mobility was significantly correlated with disease duration in IDDM, and was associated with neuropathy in both IDDM and NIDDM and with retinopathy in IDDM. No correlation was found between DR3, DR4 and limited joint mobility or diabetic complications. We also investigated the usefulness of nailfold capillary microscopy in a large group of patients with IDDM and NIDDM. Although capillary enlargement and avascular areas were noted in a few patients, nailfold capillary microscopy was not felt to be a useful tool in the evaluation of diabetes.

Adolescent↗

Eosinophilia-myalgia syndrome associated with L-tryptophan use.

The eosinophilia-myalgia syndrome associated with the use of oral preparations of the amino acid L-tryptophan was recognized in late 1989. We describe the clinical and laboratory manifestations, pathological findings and early clinical course of 20 patients with the eosinophilia-myalgia syndrome. Prominent clinical findings included severe myalgias limiting function, fatigue, rashes, edema and weight gain, weight loss, muscle weakness and shortness of breath. Laboratory findings included eosinophilia (often marked), normal erythrocyte sedimentation rate, and elevated aldolase with normal or low creatine kinase values. On biopsy fascial inflammation was always seen consisting of lymphocytes, histiocytes and eosinophils in a perivascular distribution. Invasion of the vascular wall by lymphocytes was seen in 20%. Capillary and arteriolar endothelial cell thickening was found in most cases on electron microscopy and endothelial cell necrosis or mural invasion by lymphocytes was seen in 25% of cases. Two patients improved with no therapy. Ten patients responded to therapy with prednisone alone. Three patients have had progressive disease and one of these died. The relationship of this syndrome to previously described disease entities associated with eosinophilia is discussed.

Adult↗

Microangiopathy in the eosinophilia-myalgia syndrome.

The eosinophilia-myalgia syndrome associated with the ingestion of L-tryptophan was recognized in late 1989. We describe our pathologic study of skin, fascial, and muscle biopsies from 21 patients evaluated by light microscopy, histochemistry, and electron microscopy. A perivascular, lymphocytic infiltrate with eosinophils was present in the dermis, fascia, and skeletal muscle. Lymphocytic infiltration of arteries and arterioles was seen. Ultrastructurally, capillary and arteriolar endothelial cell thickening and necrosis was present. This microangiopathy suggests that ischemia may be a contributing factor to the findings in this syndrome.

Adult↗

Pilot study of etretinate in psoriatic arthritis.

A 2-center pilot study compared clinical and laboratory outcomes in 40 patients with psoriatic arthritis before and after treatment for 8 to 24 weeks with the vitamin A derivative, etretinate. The number of tender joints fell from 22.0 +/- 8.75 before treatment to 11.44 +/- 8.50 after treatment (p = .000). The duration of morning stiffness was 101.95 +/- 62.45 min before therapy and 44.53 +/- 82.10 min after treatment (p = 0.0004). Similar highly clinically and statistically significant improvement was seen in all clinical outcome measures and in the erythrocyte sedimentation rate. Primarily mucocutaneous side effects were seen in 39/40 patients and resulted in treatment termination before 24 weeks in 9 patients.

Arthritis↗

Successive unfolding of two different collagen vascular diseases in the same patient.

Genetic factors may be important in the development of autoimmune disease. We describe the development of fulminant systemic lupus erythematosus in a patient 5 years after recovery from polymyositis. The successive unfolding of different autoimmune diseases in the same individual is suggestive of sequential gene activation.

Antibodies, Antinuclear↗

Aseptic arthritis in a man with toxic shock syndrome.

Synovitis in toxic shock syndrome (TSS) is an unusual finding. A 31-year-old man presented with pain and swelling in both knees and was found to have TSS, secondary to a septic bursitis caused by Staphylococcus aureus. Immune complexes were not detected in serum or synovial fluid, and the S aureus was not recovered from the inflamed joints. Antibodies against the TSS toxin-1 were detected in serum and synovial fluid, but in lower levels than would be seen in a normal control serum. Complement studies implicated alternative pathway activation by a marked diminution in C3 levels compared with C4 levels, and by lower levels of factor B than would be found in other inflammatory joint diseases. The diagnostic dilemma posed by TSS in a man is discussed.

Adult↗