Biomedical subjects
Publications and source records attributed to T R Kinney.
We have reported the first case of isolated submucosal hematoma of the true vocal cord in a hemophilic patient. He was managed with factor VIII replacement therapy as an outpatient for five consecutive days, without complication. Similar but more serious cases usually require more aggressive intervention by the pediatrician and the otolaryngologist.
A 17-month-old boy in whom immune-mediated thrombocytopenia (ITP) was the presenting manifestation of infection with human immunodeficiency virus (HIV) is being successfully managed with intermittent high-dose intravenous gamma globulin (IVIG) allowing maintenance of hemostatic platelet counts while avoiding the immunosuppression associated with other therapeutic modalities used to treat ITP. He continues to demonstrate marked responsiveness to IVIG, and has been maintained on weekly or bimonthly infusions for 12 months. The serendipitous documentation of HIV infection prior to IVIG therapy for immune-mediated thrombocytopenia in this child documents the importance of HIV testing prior to IVIG therapy to prevent erroneous assignment of IVIG as the vehicle responsible for transmission of HIV infection. This case history also documents the importance of HIV testing in the diagnostic evaluation of immune-mediated thrombocytopenias.
Broviac and Hickman catheters facilitate the care of children with cancer but provide a source of potential infection. We describe a child with a Broviac catheter who developed left-sided bacterial endocarditis in whom right-to-left atrial shunting was documented following catheter flushing. Following removal of the catheter and administration of prolonged intravenous antibiotics, recovery was complete and cardiac function returned to normal.
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Current therapy for patients with trilateral retinoblastoma, consisting primarily of surgical intervention and radiotherapy, has resulted in no long-term survivors. The use of adjuvant chemotherapy has not improved this outcome. After observing a tumor response to cyclophosphamide in a patient with suprasellar retinoblastoma, we treated a subsequent patient with trilateral retinoblastoma with both vincristine and cyclophosphamide. Objective tumor regression resulted. Although the tumor ultimately progressed in both patients, these findings suggest that vincristine and cyclophosphamide are active in patients with intracranial (trilateral) retinoblastoma.
A 4 year old girl with sickle cell disease developed acute lymphocytic leukemia null cell type. The bone marrow karyotype was 46,XX,del(9)(p13). This girl is among the few patients with acute lymphocytic leukemia and abnormalities of #9 to have an isolated 9p-.
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To determine the value of the direct platelet associated IgG (PAIgG) level as a prognostic indicator in childhood idiopathic thrombocytopenia purpura (ITP), 18 children with ITP were studied. Ten of the 18 had PAIgG levels measured at diagnosis, before any therapy. Of these 10 patients, six (Group I) had an acute course, with a mean initial platelet count of 15 X 10(9)/liter and a mean initial PAIgG level of 330.9 fg/plt. Four patients (Group II) had a chronic course, with a mean initial platelet count of 11 X 10(9)/liter and a mean initial PAIgG level of 38.3 fg/plt. There was no significant difference between the mean initial platelet count of Groups I and II (p greater than 0.10), but the initial PAIgG levels in those patients with an acute course were significantly higher than the levels in those patients with a chronic course (p less than 0.05). Of the original 18 patients, nine were splenectomized for chronic thrombocytopenia, with normalization of the platelet count in all instances. Of these splenectomized patients, five had platelet counts and PAIgG levels measured before and after splenectomy. All five had normal PAIgG levels following splenectomy. The PAIgG level is a good prognostic indicator for the clinical course of childhood ITP. A high PAIgG level suggests an acute course while a modestly elevated level suggests a chronic course. The PAIgG level normalizes in remission after splenectomy.
Autoantibodies directed against the common red-cell antigen Vel are unusual, with only 2 previous cases reported. This report describes an infant girl with steroid-resistant autoimmune hemolytic anemia whose serum contained an antibody with apparent specificity for Vel antigen. Transfusion with Vel-positive cells resulted in a massive hemolytic transfusion reaction. Survival of labelled Vel-negative cells was normal up to 8 h following transfusion, and no transfusion reaction followed subsequent use of Vel-negative cells. There was no evidence of a collagen vascular disease, an immunodeficiency syndrome, or a malignancy. Although rare, anti-Vel should be considered in unexplained autoimmune hemolytic anemia.
Mycoplasma pneumoniae, which usually causes mild infections in normal children, has been shown to cause isolated cases of severe pneumonia in children with sickle cell disease. We recently observed an outbreak of Mycoplasma pneumonia in three sisters with homozygous sickle cell disease. Two of them required hospitalization, and one progressed to respiratory failure requiring prolonged ventilatory assistance. All eventually recovered without long-term pulmonary complications. Familial outbreaks are not uncommon in Mycoplasma infection, but they have not been described previously in siblings with sickle cell disease. It is assumed that local or systemic host defense abnormalities predispose patients with sickling syndromes to more severe courses of Mycoplasma infections.
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Although abnormalities in coagulation tests have been reported during vaso-occlusive crises in patients with sickle cell disease, objective, readily performed laboratory tests that document the occurrence of this complication have not been available. We examined the relationship between fibrin D-dimer levels and the occurrence of complications in patients with sickle cell disease, using a commercially available latex bead agglutination assay. The patients were either asymptomatic, hospitalized for vaso-occlusive crisis, or had other complications of sickle cell disease including leg ulcers, chronic cholecystitis, aseptic necrosis, joint pain and infection. Fifty-seven percent of 187 samples on 96 patients had elevated levels of fibrin D-dimer. Ninety percent of 75 samples from asymptomatic patients were negative for fibrin D-dimer (less than 1 microgram/ml) but 97% of 29 samples from patients with vaso-occlusive crisis and 85% of 83 samples from patients with other complications of sickle cell disease were positive. In serial studies, worsening or amelioration in clinical complications were reflected in increasing or decreasing levels of fibrin D-dimer, respectively. The molecular species of fibrin identified by the latex agglutination test was shown to be fragment D-dimer by successive immunoprecipitation and protein blot analysis. We conclude that the complications of sickle cell disease, including vaso-occlusive crisis, result in the production of fibrin D-dimer, and its detection may be used as a marker for the presence of the complication.
Socioeconomic data on 3538 black patients enrolled in the Cooperative Study of Sickle Cell Disease (CSSCD) from 1979 to 1981 are summarized and compared with the U.S. black population (USBP) where appropriate. The following contrasts between the patients and families with Sickle Cell Disease (SCD) and the USBP were found: family structure differed, with fewer two-parent families (40 vs 54%) and more single female heads-of-household (53 vs 42%) within the SCD population; of those employed, twice as many of the SCD patients of both sexes worked in white collar positions; a higher percentage of patients were unemployed and disabled; and male SCD patients had a lower median personal income than USBP males. Several similarities were also noted: the percentage of high school graduates was 71% for SCD patients and 75% for the USBP; female heads-of-household employed full time earned approximately the same salary as USBP females. These data will help health professionals who counsel SCD patients and their families.
Evaluations were performed in 20 patients with childhood idiopathic thrombocytopenic purpura (ITP) who remained in remission longer than 12 months. The mean duration of follow-up from diagnosis was 39 months (range 17 to 87 months). Eleven patients (four girls) in group 1 had an acute course of ITP, defined as platelet count greater than 150 X 10(9)/L within 6 months of diagnosis. Nine patients (five girls) in group 2 had a chronic course, defined as platelet count less than 150 X 10(9)/L for greater than or equal to 1 year or requiring splenectomy in an attempt to control hemorrhagic symptoms. Mean age at diagnosis and duration of follow-up were similar for both groups. Platelet count and serum (indirect) platelet-associated IgG (PAIgG) levels were normal in all 20 patients at follow-up. Both direct and indirect PAIgG levels were measured using a 125I-monoclonal anti-IgG antiglobulin assay. All had normal direct PAIgG levels, except for one patient in group 1 who had a borderline elevated value of 1209 molecules per platelet. These data suggest that the prevalence of elevated platelet antibodies is low during sustained remission without medication in patients with a history of childhood ITP. These data may be relevant for pregnant women with a history of childhood ITP, with regard to the risk of delivering an infant with thrombocytopenia secondary to transplacental passage of maternal platelet antibody.
We report two patients who developed high urinary excretion of sodium chloride after receiving cisplatinum. One patient became symptomatically hyponatremic during intravenous administration of hypotonic fluid. The second patient developed orthostatic hypotension. Urine sodium and chloride concentrations exceeded 130 mmol/liter despite hyponatremia or volume depletion. There was no evidence of adrenal cortical impairment and no improvement with fludrocortisone. We propose that cisplatinum may inhibit solute transport by the thick ascending limb of Henle, causing clinically significant, previously unreported toxicity in patients receiving chronic cisplatinum antitumor therapy.
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A 28-month-old white male, 7 months status postenucleation for retinoblastoma, presented with lethargy, irritability, anemia, and thrombocytopenia. Bone marrow examination revealed almost total replacement of marrow elements with clumps of small round basophilic cells. Electron microscopic study of the marrow revealed clumps of cohesive tumor cells with intermediate junctions, establishing a diagnosis of metastatic retinoblastoma.