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Biomedical subjects

L Parvey

Publications and source records attributed to L Parvey.

8 recordsLinked to original sources

Thoracic aortic calcification in 3 children with candidiasis-endocrinopathy syndrome.

Three cases are reported of the association of childhood onset of thoracic aortic calcification with mucocutaneous candidiasis, endocrine dysfunction and recurrent non-fungal pulmonary disease. The aortic calcification affects the thoracic aorta and the low lumbar aorta and common iliac arteries, sparing the mid-lumbar aorta and its major branches. Ischemic signs and symptoms of the head and neck and lower limbs are absent. This peculiar, slowly progressive vascular calcification, although unexplained to date, appears to be a non-random part of the more common candidiasis-endocrinopathy syndrome.

Adolescent↗

Preoperative embolization of the spleen in children with hypersplenism.

Splenomegaly associated with myelodysplastic disorders in children may be massive and can result in pancytopenia, abdominal discomfort, and respiratory distress. When these symptoms cannot be relieved by nonsurgical means, splenectomy may be indicated. Under such conditions, surgical splenectomy carries increased risks, as the thrombocytopenia is difficult to correct secondary to splenic sequestration. Additionally, the surgical anatomy is often distorted secondary to the massive spleen and dissection can be difficult. These factors can lead to uncontrollable hemorrhage. In an attempt to decrease intraoperative blood loss, the authors successfully performed preoperative splenic artery embolization in 11 of 12 children (age range, 1-11 years) with pancytopenia due to hypersplenism. Hypersplenism requiring surgical splenectomy was due to leukemia (n = 9), myelodysplastic syndrome (n = 1), immune thrombocytopenia (n = 1), and osteopetrosis (n = 1). Embolization was performed under general anesthesia, prior to surgery, with gelatin sponge particles alone, Gianturco coils alone, or a combination of polyvinyl alcohol sponge particles and Gianturco coils. Embolization allowed for safe surgical splenectomy.

Child↗

Comparison of neuropsychologic functioning and clinical indicators of neurotoxicity in long-term survivors of childhood leukemia given cranial radiation or parenteral methotrexate: a prospective study.

We prospectively compared neuropsychologic functioning and clinical indicators of neurotoxicity in 49 consecutive childhood leukemia patients in long-term continuous complete remission (CR) who had received two different regimens of CNS prophylaxis by random assignment. Twenty-three patients were treated with 1,800 cGy cranial radiation and intrathecal methotrexate (RT group) and 26 with parenteral methotrexate only (MTX group). Over half of the RT group had somnolence syndrome, and four developed cerebral calcifications late in their clinical course. Abnormal electroencephalograms (EEGs) were seen in 15 patients in the MTX group, and six had early, transient white-matter hypodensities apparent on computed tomographic (CT) scans. Mean scores on standard tests of intelligence and academic achievement, administered after remission induction and again at a median of 6 years after treatment cessation, did not differ significantly between the two groups. However, statistically significant decreases in overall and verbal intelligence quotients (IQs) and in arithmetic achievement were found within both treatment groups. Sixteen of 26 in the MTX group and 14 of the 23 in the RT group had clinically important decreases (greater than or equal to 15 points) on one or more neuropsychologic measures. These changes did not correlate with findings on CT scans, EEGs, or other clinical signs of neurotoxicity. We conclude that 1,800 cGy cranial radiation and parenteral methotrexate, as used in this study, are associated with comparable decreases in neuropsychologic function.

Adolescent↗

Therapy for localized Ewing's sarcoma of bone.

Fifty-two previously untreated patients with localized Ewing's sarcoma of bone were treated with nonintensive chemotherapy in combination with surgery or radiation therapy (RT). RT was delivered to limited volumes in a dose dependent on the initial response to induction chemotherapy (30 to 35 Gy v 50 to 55 Gy). Fifty of the 52 patients achieved complete or partial responses with induction chemotherapy, with one nonresponding patient rendered free of tumor with surgery. Fifty patients were evaluable for local control of tumor and overall response to protocol therapy. Seventeen relapses have occurred; three metastatic, four local plus metastatic, and ten local. Two factors predicted worse disease-free survival: high WBC count (P = .03) and size of primary tumor (P = .05). Of the 14 local recurrences, 12 occurred in 28 patients who presented with primary tumors greater than 8 cm in size while only two of 22 patients with lesions less than 8 cm had local recurrence. The Kaplan-Meier estimate of disease-free survival at 3 years is 82% for those with small lesions and 64% for those with larger lesions. Site of primary was of no prognostic value (P = .27). The 5-year survival estimate for all patients is 80% (median time on study, 3.3 years).

Adolescent↗

Metastatic Ewing's sarcoma: remission induction and survival.

Eighteen patients with previously untreated metastatic Ewing's sarcoma (ES) entered a protocol designed to evaluate the response rate to cyclophosphamide and doxorubicin induction therapy delivered before delayed surgery and delayed lower dose, limited-field radiation therapy, (RT), and maintenance chemotherapy. With chemotherapy and delayed surgery, 14 of 18 were rendered free of gross tumor. RT was delivered to the primary site of 11 of these responding patients, plus four of those not free of gross disease. Following RT, two more attained complete clinical remission. Site of primary or metastases did not influence outcome; however, the size of the primary at diagnosis did appear to do so. Ten patients remain disease-free 16 to 82 months (median, 47 months) from diagnosis.

Adolescent↗

The value of computerized tomography as a routine follow-up procedure for patients with Wilms' tumor.

We performed routine computerized tomography (CT) of the chest and abdomen on patients treated for Wilms' tumor and reviewed the records of all patients who relapsed (10 patients) and 43 additional patients followed for at least 1 year. Routine chest radiographs and abdominal ultrasounds were compared with 210 CT examinations (107 chest and 103 abdomen). CT examinations were obtained at 6 months for stage I (13 patients) and at 3, 8, and 15 months for stages II, III, and IV (40 patients). Of the 10 patients who relapsed, only 2 were documented by CT scan in the absence of clinical symptomatology and presence of normal radiographs and ultrasound. Eight relapses were diagnosed by physical examination, chest radiograph, or ultrasound and confirmed by CT scan. Questionably abnormal CT scans were obtained in 7 patients, only 1 of whom eventually relapsed. Since only 2 of 210 routine CT examinations were the the only indicator of relapse, we conclude that the routine use of CT for follow-up in Wilms' tumor is not warranted.

Evaluation Studies as Topic↗

Differences between right and left lateral chest radiographs.

Differences between 150 normal right lateral chest teleroentgenographs and 150 left laterals (infants, children, and adults) were evaluated and determined to be greater than has been generally appreciated. On the left lateral view the left leaf is distinguished from the right leaf by the following generalizations: anteriorly the left leaf is lower; posteriorly it is higher as is its costrophrenic sulcus; it intersects the relatively flattened right leaf near the posterior heart border; the stomach bubble and/or colon are immediately subjacent; there is no segmental obliteration by the inferior vena cava as there often is with the right leaf; in adults the outline of the left leaf is often sharper. When both major fissures can be identified, the left almost always intersects its diaphragmatic leaf posterior to and more vertically than the right fissure.

Adult↗

The Ask-Upmark kidney. Roentgenographic and pathological features.

The Ask-Upmark kidney represents an unusual manifestation of a segmental hypoplastic kidney in which the lesion may be local or diffuse, and may involve one or both kidneys. It is almost always associated with hypertension and is, therefore, potentially curable when localized to a single kidney. It is imperative to recognize this disease in order to distinguish it from other entities that cause hypertension.

Biopsy↗