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Biomedical subjects

D Grisaru

Publications and source records attributed to D Grisaru.

43 records · Page 3Linked to original sources
PubMed1990

Pulmonary syndrome in patients with thalassemia major receiving intravenous deferoxamine infusions.

Eight patients with transfusion-dependent thalassemia major were given continuous intravenous infusions of the chelator, deferoxamine mesylate, to reduce iron overload. Within 5 to 9 days of starting the infusions, four patients developed a pulmonary syndrome of moderate to life-threatening severity characterized by tachypnea, hypoxemia, and a diffuse interstitial pattern on chest roentgenogram. Pulmonary function studies showed restrictive dysfunction. Lung biopsy showed diffuse abnormalities with alveolar damage, interstitial fibrosis, and inflammation. The inflammatory infiltrate comprised lymphocytes, eosinophils, and mast cells. Exposure of the biopsy specimen to fluorescein-conjugated anti-IgE antibody showed fixation of IgE to the mast cell surface, suggesting a hypersensitivity reaction. Detailed studies failed to identify an infectious agent. The temporal relationship between drug administration and lung disease, and the clinical similarities in the four affected patients, strongly suggested a cause and effect relationship. We recommend that therapy with continuous intravenous infusions of deferoxamine be monitored carefully with respect to pulmonary status.

Adolescent↗
PubMed1990

High incidence of cholelithiasis in older patients with homozygous beta-thalassemia.

The records of 48 patients with homozygous beta-thalassemia were reviewed for evidence of cholelithiasis by abdominal ultrasonography or plain abdominal X-ray. The presence of cholelithiasis was reported in 25 patients (52%). The incidence increased with age to 83% in patients over 31 years of age. Gallstones were more frequent among patients with beta-thalassemia intermedia and with less blood transfusion requirements. Eleven patients with cholelithiasis (44%) became symptomatic. Eight received operations on an elective or semielective basis. Another patient had incidental cholecystectomy during splenectomy. The preoperative evaluation included abdominal ultrasonography, nucleotide biliary scan and evaluation of the cardiopulmonary status. Due to the longer survival of patients with homozygous beta-thalassemia, the problem of cholelithiasis is becoming more frequent.

Adolescent↗
PubMed1990

Cardiopulmonary assessment in beta-thalassemia major.

Thalassemia patients succumb at a young age to congestive heart failure. Hitherto, attention has been focused on left ventricular function. This report emphasizes right ventricular dysfunction and abnormal pulmonary function. We performed cardiopulmonary evaluation, including echo-Doppler, spirometry, CO diffusion (DCO), and blood gas analyses in 35 patients with homozygous beta-thalassemia maintained by multiple blood transfusions. Six autopsy lung specimens were studied. Thalassemia patients exhibited pulmonary dysfunction, characterized by hypoxemia (85 percent of the patients were outside the 95 percent confidence limits), reduced lung volumes (51 percent), flow rates (63 percent) and DCO (50 percent). Right ventricular dysfunction was more prevalent than left ventricular dysfunction. Furthermore, 75 percent of the patients had evidence of pulmonary hypertension consistent with more frequent right ventricular rather than left ventricular dysfunction. Our findings suggest that in thalassemia patients, complex cardiopulmonary abnormalities precede the final outcome of congestive heart failure.

Adult↗
PubMed1989

Severe acquired aplastic anemia in children: 11-year experience with bone marrow transplantation and immunosuppressive therapy.

We reviewed the management and outcome of severe acquired aplastic anemia in 36 patients younger than 18 years old between March 1977 and June 1987. In most patients, no cause could be found. The best therapeutic results were achieved with allogeneic bone marrow transplantation from histocompatible related donors (14 patients), with a cure rate of 79%. Bone marrow transplantation from mismatched related donors was attempted in two patients and failed in both. Immunosuppression using antithymocyte globulin and/or high-dose methylprednisolone represented an alternative treatment in the absence of a suitable bone marrow donor (12 patients) and induced complete remission in 25% of the patients. Children treated with supportive care only (eight patients) had a 75% mortality with a very short survival time. These data confirm the superiority of allogeneic bone marrow transplantation over immunosuppressive therapy in children with severe aplastic anemia.

Adolescent↗
PubMed1986

Deferoxamine improves left ventricular function in beta-thalassemia.

Serial echocardiographic examinations were made to study the changes in left ventricular (LV) function and wall mass in 35 patients with thalassemia followed up for 5.5 +/- 2 years (mean +/- SD). Twenty patients received deferoxamine sulfate for 2.0 +/- 0.6 years (drug group) and 15 patients did not (nondrug group). Repeated blood transfusions were used to maintain the pretransfusion hemoglobin levels at 9 g/dL (90 g/L). Deferoxamine therapy improved LV function and decreased LV wall mass. Percentage shortening of LV diameter improved in the drug group (5.0% +/- 3.9%) and deteriorated in the nondrug group (-6.8% +/- 5.6%). Similarly, the maximum velocity of LV posterior wall motion improved in the drug group (16.1 +/- 20.1 mm/s) and deteriorated in the nondrug group (-18.3 +/- 19.0 mm/s). Left ventricular wall mass decreased in the drug group when compared with the nondrug group. In a subset of the drug group, pathologic natural deterioration in LV systolic function was reversed by treatment. Correlation studies indicated that frequent blood transfusions together with chelation therapy reduced LV dilatation and wall thickness, but blood transfusions alone did not have the same effect. Thus, treatment of patients with thalassemia with modest blood transfusions and deferoxamine can prevent deterioration and may even improve their LV systolic function, associated probably with arrest and reversal of the pathologic process that increases LV wall mass.

Adolescent↗
PubMedDate not supplied

Integrated 18F-fluorodeoxyglucose positron emission tomography and computerized tomography in endodermal sinus tumor.

Application of an in-line positron emission tomography and computerized tomography (PET-CT) in endodermal sinus tumor (EST) is described in this study. CASE 1: A young female with massive ascites postovarian mass resection had elevated alpha-fetoprotein (AFP) serum levels. Following a positive PET-CT study with increased (18)F-fluorodeoxyglucose (FDG) uptake, a CT-guided core biopsy of a peritoneal mass was performed. EST was diagnosed histologically. The patient was disease free after chemotherapy. Follow-up PET-CT was negative in keeping with no viable tumor tissue. CASE 2: A large pelvic mass diagnosed histologically as primarily EST was removed in a teenage patient with elevated AFP levels. PET-CT showed diffuse abdominal spread of FDG uptake, suggesting extensive peritoneal seeding. The patient was disease free after chemotherapy. Follow-up PET-CT was negative. EST is an FDG-avid tumor. PET-CT delineated the prechemotherapy tumor extent adequately ruled out the presence of residual tumor after a successful treatment.

Adolescent↗