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Biomedical subjects

V C Canale

Publications and source records attributed to V C Canale.

16 recordsLinked to original sources

Botryoid rhabdomyosarcoma of the nasopharynx.

A case report of a Stage III botryoid rhabdomyosarcoma of the nasopharynx associated with a six-and-a-half-year survival is presented. Treatment consisted of surgery, radiotherapy (6,000 rads TCT) to the nasopharynx and maxillary sinuses bilaterally, and six cycles of polychemotherapy with Vincristine, Adriamycin, Cyclophosphamide and DTIC, without major loss of function or cosmetic deformity. The histology of the lesion is discussed with reference to recent classification and prognosis. The authors suggest that the histological type and prognosis of rhabdomyosarcoma of the nasopharynx in children may be better correlated in future studies.

Child

Echocardiographic abnormalities in patients with transfusion-dependent anemia and secondary myocardial iron deposition.

The heart was evaluated by echocardiography in 56 patients at risk for myocardial iron deposition. Fifty-four had congenital anemia for which they required repeated transfusions, and two had primary hemochromatosis. The data, plotted according to one of three functions of the body surface area, were compared to values obtained in 105 normal subjects whose age spanned a similar range. Left ventricular wall thickness, transverse dimension and mass, as well as left atrial transverse dimension, were increased in the majority of patients with chronic iron overload despite the infrequent occurrence of cardiac enlargement on routine chest films (32 per cent) or electrocardiographic abnormality (16 per cent). Left ventricular ejection fraction was normal in all but four patients. These four patients died within a six month follow-up period suggesting that deterioration in systolic function is an indicator of poor prognosis. Our findings indicate that echocardiography provides a simple noninvasive means for assessing changes in cardiac structure and function that should prove useful in the serial evaluation of patients who are at risk for the development of myocardial iron deposition.

Adolescent

Hepatocellular carcinoma, transfusion-induced hemochromatosis and congenital hypoplastic anemia (Blackfan-Diamond syndrome).

A 25 year old patient with congenital hypoplastic anemia (Black-fan-Diamond syndrome) is described. This patient was hepatitis-antigen negative, had not received androgens and had a hepatoma develop in a transfusional hemochromatotic liver. Since androgens have been associated with hepatocellular carcinoma, the use of androgenic steroids for other than life-threatening symptoms in this disease should be avoided.

Adult

Chelation studies with 2,3-dihydroxybenzoic acid in patients with beta-thalassaemia major.

2-3-Dihydroxybenzoic acid was evaluated as a potentially useful, orally effective iron-chelating drug by performing iron balance studies in patients with beta-thalassaemia major. The administration of this substance at 25 mg/kg/d to five patients for 8 d caused an average increase in iron excretion of 4.5 mg/d. When the drug was administered at 25 mg/kg q.i.d. to eight patients for 21 d, iron excretion increased to 6.5 mg/d. Chelation was highly specific for iron with changes in magnesium and calcium excretion being insignificant. The drug was well tolerated with side effects limited to gastrointestinal complaints which ameliorated when the drug was taken with food. These studies provide a rationale for further evaluation of 2,3-dihydroxybenzoic acid in patients with iron overload.

Administration, Oral

Fractures in thalassemia.

Fractures occur frequently in patients with homozygous beta-thalassemia. A study was made of the fractures noted in a group of patients who were followed at the Thalassemia Clinic at The New York Hospital-Cornell Medical Center. Results indicate that these patients often sustain fractures which are multiple and which frequently heal with resultant deformities.

Adolescent

Wilms' tumor.

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Child

Fatal pulmonary hypersensitivity reaction to HL-A incompatible blood transfusion:report of a case and review of the literature.

A girl with Thalassemia major reacted to a transfusion of packed red blood cells with increasing respiratory distress until death 12 1/2 hours later. Chills and fever were followed by dry cough, dyspnea, and pulmonary edema. The recipient had lymphocytotoxic antibodies specific for donor leukocyte antigens HL-A11 and possibly W14. At autopsy, the lungs showed pulmonary edema with extensive nonspecific acute alveolar injury. Similar cases in the literature are reviewed.

Adolescent