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Biomedical subjects

A L Lightsey

Publications and source records attributed to A L Lightsey.

At least 19 recordsLinked to original sources
PubMed1999

Surgical management of intractable epilepsy in children with hemophilia.

Intracranial hemorrhage occurs in 2-8% of patients with hemophilia and can result in neurologic sequelae including seizure disorders. There is a paucity of data concerning the surgical treatment of epilepsy in children with hemophilia. We review our experience with 2 children who developed medically refractory seizure disorders. Both children underwent hemispherotomy, at 18 months (case 1) and 13 years of age (case 2), respectively. Perioperative management included continuous factor replacement. Both children tolerated surgical intervention without complications or increased neurologic deficit. Case 2 showed a 90% reduction in seizure frequency, and case 1 is seizure free. Surgical management of intractable epilepsy in children with hemophilia is safe and effective.

Brain↗
PubMed1989

Emergency use of uncrossmatched red cells in neonatal intensive care infants.

In very rare situations, a newborn will be delivered with severe unrecognized life-threatening anemia. Two infants with severe anemia with differing etiologies were deemed to require emergency uncrossmatched O-negative blood. In one infant, the blood transfused was incompatible for the minor antigen that had caused the isoimmune hemolytic anemia. We report on the indications, potential risks and benefits in giving uncrossmatched O-negative blood to term infants with life-threatening anemia.

Blood Grouping and Crossmatching↗
PubMed1986

Castleman's disease in children.

A three and one-half-year-old girl and a 12-year-old boy presented with features of the two clinical presentations of Castleman's disease or giant lymph node hyperplasia. The girl presented with anemia, fever, night sweats, hypergammaglobulinemia, and a palpable abdominal mass. Her symptoms were consistent with those seen in the plasma-cell type of this disease. The boy presented with acute appendicitis and a left hilar mass was noted on his chest radiograph. His asymptomatic presentation was typical of the hyaline-vascular form. Both patients are well without evidence of recurrence four years following resection. Castleman's disease is a benign disorder of lymph nodes that occurs rarely in children. Since the original report in 1954 we could find only 18 cases in the 16-year and younger age group and our patient with the symptomatic form represents the youngest patient reported in the English literature. The management requires surgical resection of the enlarged nodes both for diagnosis and therapy since the enlarged nodes can mimic malignant tumors of the lymphoid system. No recurrences have been reported in pediatric patients.

Child↗
PubMed1985

Severe congenital neutropenia with unique features of dysgranulopoiesis.

Congenital dysgranulopietic neutropenia (CDN) is a recently proposed entity that describes a small subgroup of children with clinically severe neutropenia. We followed and studied a 3-year-old girl with neutropenia (less than 500/mm3) and recurrent severe infections in whom repeated marrow evaluations revealed large (30-50 microns) multinucleated promyelocytes to polymorphonuclear cells with as many as 4 to 16 nuclei or nuclear lobes, respectively. In addition to the nuclear endoreduplication, ultrastructural and cytochemical evaluation of these cells demonstrated abnormalities in granule genesis and centriole structure. Concomitantly, immunoperoxidase staining indicated that many of the granules were devoid of lactoferrin but not lysozyme. In vitro proliferation studies revealed normal to increased thymidine labeling, normal numbers of colony-forming cells, and normal colony-stimulating activity from blood and marrow mononuclear cells, findings consistent with ineffective myelopoiesis. However, serum folate, B12, and lysozyme levels were normal. The nuclear and cytoplasmic abnormalities in this patient result in an extreme example of CDN, distinct from previously described cases.

Agranulocytosis↗
PubMed1984

Purpura fulminans following late-onset group B beta-hemolytic streptococcal sepsis.

A 16-day-old male infant initially was in septic shock. Following intensive resuscitation, thrombohemorrhagic lesions developed over his extremities, except for the limb with an arterial line maintained by a continuous heparin sodium infusion. Blood and CSF cultures yielded group B beta-hemolytic streptococci. Results of laboratory studies and clinical appearance supported the diagnosis of purpura fulminans (PF). Systemic heparinization was therefore started, and subsequently his condition improved. Because of the distinct difference in limb sparing, we concluded heparin has a beneficial effect on the evolution of PF.

Arm↗
PubMed1983

Congenital self-healing histiocytosis-X.

Histiocytosis-X (H-X) is heterogeneous clinically, varying from localized benign forms to disseminated fatal forms, but has a relatively uniform and specific pathologic appearance, both by light and electron microscopy. H-X localized to bone or lung has long been recognized. Much less frequent are patients with H-X localized to skin. We report two infants with congenital self-healing H-X, clinically confined to the skin. Prediction of the clinical course from the histology of a H-X lesion is unreliable, especially for cutaneous lesions. We feel that patients with localized forms of H-X should be followed closely for progression of disease but should not be treated aggressively until time is allowed for spontaneous resolution.

Biopsy↗
PubMed1982

Utility of monoclonal antibodies in the immunologic phenotyping of acute lymphoblastic leukemia.

Peripheral blood and/or bone marrow lymphoblasts from 34 children and 11 adults with acute lymphoblastic leukemia (ALL) were evaluated with a monoclonal anti-Ia antibody and a monoclonal anti-pan T-cell antibody (T101) specific for a 65,000-dalton T-cell antigen (T65). Seventy-six per cent of cases were Ia+T65-, 20% were Ia-T65+ and the remaining 4% were Ia-T65-. Anti-Ia and T101 reactivity were mutually exclusive and no Ia+T65+ cases were identified. In childhood ALL, the Ia+T65- phenotype was associated with good prognostic factors and longer median disease-free survival than Ia-T65+ patients whose clinical parameters resembled those characteristic of high-risk T-cell ALL. Included in the Ia-T65+ group were three E-rosette negative cases with clinical features of T-cell disease. Our findings compare favorably with the results of other investigators utilizing polyclonal antisera and suggest that these monoclonal antibodies, which offer the advantages of monospecific standardized reagents, will prove useful in the immunologic characterization of ALL.

Adolescent↗
PubMed1980

Thrombocytopenia in children.

Thrombocytopenia in children can be classified on the basis of understanding of the production, life span, and fate of platelets. Thrombocytopenia may be familial, associated with drug-ingestion as a result of immune mediated mechanisms, direct toxicity to circulating platelets, or injury to megakaryocytes, or associated with other disease states.

Adrenal Cortex Hormones↗
PubMed1979

Platelet-associated immunoglobulin G in childhood idiopathic thrombocytopenic purpura.

Childhood ITP is an acquired hemorrhagic disorder with a heterogeneous clinical course. We measured PAIgG levels in 20 children with ITP (7 acute, 13 chronic). Both groups had significantly greater PAIgG values than age-matched normal subjects and thrombocytopenic controls (P less than 0.001). In addition, PAIgG values in chronic ITP were significantly lower than those in acute ITP (P less than 0.003). Serial PAIgG values were obtained in some patients; most returned to normal in association with clinical recovery. The measurement of PAIgG is useful in the diagnosis and follow-up of childhood ITP. PAIgG values may assist in differentiating acute and chronic disease in children.

Acute Disease↗
PubMed1979

Immune suppression of erythropoiesis in transient erythroblastopenia of childhood.

Serum and IgG from four children with transient erythroblastopenia of childhood (TEC) was tested to see what effect it would have on development of erythroid colonies from bone marrow mononuclear cells. Serum and IgG specimens obtained at the time of diagnosis uniformly suppressed erythroid colony development from CFU-E. Washed bone marrow mononuclear cells from a child with TEC failed to grow in the presence of his own serum, but grew normally in the presence of isologous serum. Serum specimens obtained from patients after recovery from TEC had no effect on erythroid colony development. The anemia of TEC appears to be due to transient immune suppression of erythroid colony development.

Anemia, Aplastic↗
PubMed1979

The role of the spleen in "autoimmune" blood disorders.

Several immune-mediated blood disorders in both children and adults have findings compatible with an autoimmune etiology, although the autoantigen in most instances is not clearly established. In the present report, we review briefly the clinical features of immune hemolytic anemia, immune thrombocytopenic purpura, and immune neutropenia in children and discuss the possible pathogenetic mechanisms based on present experimental and clinical observations. The role of the spleen in the pathophysiology of these disorders is emphasized.

Acute Disease↗
PubMed1977

Immune neutropenia.

A patient with hyperthyroidism and severe neutropenia had a clinical course and family history that suggested an immune cause. Neutrophil-binding IgG was demonstrated in serum using the Fab-anti Fab assay. The antineutrophil factor bound specifically to either homologous or autologous neutrophils and could be adsorbed by the target neutrophils. The quantity of IgG required to saturate the neutrophil-binding sites (175 000 molecules per neutrophil) and the serum concentration (1.3 mug/ml) were determined. It was estimated that at the time that blood and marrow neutrophils were markedly reduced, serum contained sufficient neutrophil-binding IgG to saturate the binding sites of 1.2 times the total blood neutrophil pool.

Agranulocytosis↗
PubMed1977

Platelet-associated IgG in immune thrombocytopenic purpura.

A method for the measurement of immunoglobulin G associated with gel-filtered platelets is described and finding in 70 control subjects and 37 patients with immune thrombocytopenic purpura (ITP) are reported. Control platelet-associated IgG (PAIgG) levels (nanograms IgG per 10(9) platelets) averaged (+/-SD) 1231+/-424; samples studied after 24 and 48 hr remained within the control range. PAIgG values of 19 adult and 12 childhood patients with chronic ITP averaged 4711+/-3025 and 4923+/-3955, respectively, and differed significantly from controls (p less than 0.001). There was an inverse correlation between PAIgG values and the chronic ITP patient's platelet count. Six patients with childhood acute ITP had PAIgG levels ranging from 5588 to 56,250 and appeared to represent a different statistical population from those with chronic ITP. In chronic ITP patients responding to splenectomy, there was an immediate normalization of PAIgG levels; however, a certain percentage of patients studied several months after splenectomy evidenced elevated PAIgG levels in association with normal platelet counts. These data showed that the direct measurement of platelet associated antibody is a useful technique in the diagnosis and follow-up of patients with chronic ITP. Preliminary studies in patients with acute ITP have suggested that this method may be useful in differentiating acute and chronic childhood ITP.

Adult↗
PubMed1977

Free erythrocyte porphyrin: hemoglobin ratios, serum ferritin, and transferrin saturation levels during treatment of infants with iron-deficiency anemia.

Free erythrocyte porphyrin:hemoglobin (FEP:Hb) ratios were determined on 20 infants with iron-deficiency anemia. FEB:Hb ratios were compared with simultaneously drawn serum ferritin and serum transferrin saturation levels. FEP:Hb ratios dropped steadily during treatment of the anemic infants, but remained elevated compared to age-matched nonanemic infants, until the anemia was corrected. FEP:Hb ratios detected iron deficiency when acute inflammatory disease was present. Serum ferritin levels and transferrin saturation failed to detect iron deficiency after iron therapy was started or when acute inflammatory disease was present. Measurement of FEP:Hb ratios is a reliable and practical way to make a prompt diagnosis of iron-deficiency anemia in infants.

Anemia, Hypochromic↗