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Biomedical subjects

J R Priest

Publications and source records attributed to J R Priest.

34 records · Page 2Linked to original sources
PubMed1988

Intrathoracic extravasation of sclerosing agents associated with central venous catheters.

Two children receiving continuous infusions of vesicant chemotherapy through central venous catheters (CVCs) developed venous thrombosis, and intrathoracic extravasations ensued. One child receiving a continuous vincristine infusion presented with signs of thoracic venous obstruction, fever, and respiratory distress and had pleural effusions and pulmonary infiltrates on his chest roentgenogram. The other child was receiving a continuous doxorubicin infusion and developed superior vena cava thrombosis and retrograde extravasation along the catheter tunnel site. Both children improved after chemotherapy was discontinued and the CVCs removed. Catheter placement and the continuous infusion of sclerosing agents are discussed.

Antineoplastic Agents↗
PubMed1987

Results of a pilot study of hyperfractionated radiation therapy for children with brain stem gliomas.

The majority of children with brain stem gliomas develop progressive disease within 18 months of diagnosis and treatment. Radiotherapy (RT) is of transient benefit in most patients and higher total doses of RT have been related to improved survival. The amount of RT which can be given is limited by the tolerance of the surrounding brain. Hyperfractionated RT theoretically allows higher doses of RT to be tolerated by the brain. Sixteen children with brain stem gliomas were treated on a hyperfractionated RT schedule, receiving 120 cGy of RT twice daily, to a total dose of 6480 cGy. All patients tolerated treatment well. Eleven of 15 (73%) evaluable patients had a response to treatment and two (13%) others had stable disease. One patient developed progressive disease during treatment. All patients were tapered off steroids by the completion of treatment. Thirteen of 16 (81%) patients developed progressive disease at a median of 7 months after diagnosis and three remain in remission 8, 12, and 15 months following diagnosis. These results were similar to those of historical controls. Two patients were surgically explored at time of relapse and 5 have had an autopsy. No acute or subacute neurologic toxicity was seen; but long-term detrimental effects on brain could not be assessed. The implications of this study are that escalations of the dose of hyperfractionated RT can be entertained for children with brain stem gliomas.

Adolescent↗
PubMed1982

A syndrome of thrombosis and hemorrhage complicating L-asparaginase therapy for childhood acute lymphoblastic leukemia.

L-Asparaginase therapy for childhood acute lymphoblastic leukemia causes deficiencies of plasma hemostatic proteins, especially antithrombin, plasminogen, and fibrinogen. Severe thromboses and hemorrhages occurred in 18 children receiving vincristine, prednisone, and asparaginase therapy for ALL. Thirteen children had intracranial thrombosis or hemorrhage, four had extremity thrombosis, and one had both an intracranial hemorrhage and an extremity thrombosis. These events occur characteristically in the third and fourth weeks of therapy during or just after a three-week course of L-asparaginase. Symptoms of headache, obtundation, hemiparesis, and seizure were common for the intracranial events: local pain, swelling, and discoloration were common for the extremity thromboses. These complications have been recognized in 1 to 2% of children undergoing induction therapy which includes asparaginase.

Adolescent↗
PubMed1982

The effect of L-asparaginase on antithrombin, plasminogen, and plasma coagulation during therapy for acute lymphoblastic leukemia.

Hemostatic function was studied sequentially in 12 children receiving L-asparaginase, vincristine, and prednisone as remission induction chemotherapy for acute lymphoblastic leukemia. The three-week period of L-asparaginase therapy was characterized by progressive decreases in plasma antithrombin, plasminogen, and fibrinogen concentrations, and by progressive increases in plasma clotting times (prothrombin time, partial thromboplastin time, thrombin time). Platelet counts rose rapidly during the third and fourth weeks of therapy as bone marrow remission was achieved. Factor V levels increased steadily during a five-week period, perhaps related to vincristine or prednisone therapy. Recent reports of thrombosis and hemorrhage in children and adults receiving L-asparaginase may be explained by this complex set of abnormalities in coagulation and coagulation control.

Adolescent↗
PubMed1980

Thrombotic and hemorrhagic strokes complicating early therapy for childhood acute lymphoblastic leukemia.

Sudden cerebrovascular insults occurred during or immediately following remission induction therapy in 4 children with acute lymphoblastic leukemia. In 3, cerebral infarction was due to thrombosis. In the fourth, an intracerebral hematoma developed representing either frank hemorrhaging or a hemorrhagic infarction. None of the patients had central nervous system leukemia or extreme leukocytosis at the time of diagnosis. Symptoms were obtundation, hemiparesis, seizures, and headache. The induction chemotherapy included L-asparaginase which causes deficiencies of antithrombin, plasminogen, fibrinogen, and factors IX and XI. These hemostatic abnormalities may explain the thromboses and bleeding observed in these children.

Adolescent↗
PubMed1980

Philadelphia chromosome positive childhood acute lymphoblastic leukemia.

In a 3-yr period, the Philadelphia chromosome (Ph1) was found in 4 of 43 children with acute lymphoblastic leukemia (ALL) in whom chromosomes were studied at diagnosis. The clinical, morphological, cytochemical, and immunologic findings in the Ph1-positive (PH1+) CASES WERE CONsistent with typical childhood ALL, indicating that identification of cases requires chromosome studies. A review of all reported cases of Ph1 + childhood ALL shows that Ph1 + patients are older and have higher initial platelet and white blood cell counts (WBC) than most children with ALL. However, a life table comparison between the reported cases of Ph1 + ALL in children and randomly selected age-, sex-, and WBC-matched controls with ALL shows the duration of first marrow remission to be significantly shorter (p less than 0.02) for the Ph1 + cases Ph1 + ALL is a distinct subtype of childhood ALL that is not rare and can be identified only by cytogenetic studies. The prognosis is poor. Cytogenetic studies should be done prospectively in a large group of children with ALL to define further the subgroup of patients and to confirm the findings of this retrospective analysis.

Adolescent↗
PubMed1980

Dural venous sinus thrombosis in acute lymphoblastic leukemia.

Three children with acute lymphoblastic leukemia developed sagittal sinus thrombosis. One patient was in peripheral remission. One patient survived. In neither patient who died were the walls of the dural sinuses infiltrated with leukemic cells. Attention is drawn to this potentially treatable cause of central nervous system symptoms in childhood leukemia. Angiography is the diagnostic test of choice and can also demonstrate intracerebral hematoma and subdural hematoma, if present. Sinus thrombosis can occur either during exacerbation or remission of the basic leukemic process. The possibility that chemotherapeutic techniques predispose toward this complication is raised.

Adolescent↗
PubMed1980

Transient autoimmune neutropenia due to anti-NA1 antibody.

A 6-month-old girl developed severe, but transient neutropenia due to an antibody against a neutrophil-specific antigen, NA1. The mean absolute neutrophil count was 380/microliter for 2 months and spontaneously returned to normal as the anti-NA1 antibody disappeared. After recovery, the NA1 antigen was demonstrated on the patient's neutrophils. No etiology was found. There was no therapy directed at suppressing the antibody or increasing the neutrophil count. Life-threatening infection did not occur during the neutropenia.

Agglutination↗
PubMedDate not supplied

A comparison of methods for detecting leukocyte antibodies in autoimmune neutropenia.

A six-month-old girl and an 18-month-old boy with autoimmune neutropenia due to anti-NA1 are described. The antibodies were detected by granulocyte microagglutination, and their disappearance in the girl coincided with a return of a normal neutrophil count. The autoantibodies in both patients also reacted in the granulocyte cytotoxicity (GC) assay, and in one patient, in the staphylococcal protein A (SPA) assay. However, neither the GC nor the SPA assays showed the anti-NA1 specificity found by agglutination, and the presence of GC and SPA antibodies did not coincide with neutropenia. These three leukocyte antibody techniques may detect different antibodies and have different clinical significances. This report provides additional evidence of the existence of autoimmune neutropenia and indicates that the clinical role of neutrophil antibodies detected by different serologic techniques is not yet established. Antibodies detected by granulocyte agglutination were clinically significant in these two patients with autoimmune neutropenia, while the results of testing with GC and SPA were not.

Absorption↗
PubMedDate not supplied

Autoimmune neutropenia of infancy and early childhood.

Forty-one children were identified with autoimmune neutropenia of infancy and early childhood (absolute neutrophil count [ANC] less than 500/microliters and demonstrable serum antineutrophil antibodies). There were 21 boys and 20 girls; the median age at diagnosis was 11 months (range 5-38 months). No life-threatening infections occurred. There was a gradual upward trend in ANC in all patients over many months, with 87% having an ANC > 1000/microliters by 24 months from diagnosis. Among various clinical and laboratory parameters analyzed statistically, only younger age at diagnosis was associated with earlier neutrophil recovery. There was no association between degree or duration of neutropenia and sex, race, antibody reactivity, or presence of serious illness at diagnosis.

Age Factors↗
PubMedDate not supplied

Neuroblastoma presenting as central nervous system disease.

Neuroblastoma may be extremely difficult to recognize, particularly when the tumor presents as a primary central nervous system disease. Central nervous system involvement may be considered as primary intracerebral neuroblastoma, metastases to the cranium from an occult primary, primary intraorbital neuroblastoma originating in the ciliary ganglion, metastatic intraorbital neuroblastoma from an occult primary, primary intraspinal neuroblastoma originating in dorsal root ganglia, intraspinal metastatic disease, and distant effects such as myoclonic encephalopathy. Primary neuroblastoma within the ciliary ganglion and primary intraspinal neuroblastoma are extremely rare entities. Illustrative cases the demonstrate the broad spectrum of neurologic presentations are offered. The second known report of neuroblastoma in association with primary pulmonary hypoventilation (Ondine curse) is included.

Brain Neoplasms↗
PubMedDate not supplied

Cystic renal lymphangiectasia: a distinctive clinicopathologic entity in the pediatric age group.

This report describes the features of unilateral cystic renal lymphangiectasia in a 2-year-old child who presented with hypertension, massive ascites, a left flank mass, and no evidence of familial renal cystic disease. The child became normotensive and is now asymptomatic more than 3 years after surgery. The clinical presentation and diffuse pathologic involvement are similar to findings for the few pediatric patients with cystic lymphangiectasia described in the literature and appear distinct from the more localized form of the disease seen in adults.

Child, Preschool↗