Biomedical subjects
J A Church
Publications and source records attributed to J A Church.
Chronic arthritis in a 3-year-old boy.
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An inherited defect of neutrophil motility and microfilamentous cytoskeleton associated with abnormalities in 47-Kd and 89-Kd proteins.
A 2-month-old male Tongan infant presented with fever, severe skin and mucosal infections, hepatosplenomegaly, thrombocytopenia, and normal neutrophil counts. While polymorphonuclear neutrophil (PMN) morphology was normal, several neutrophil motile functions were found to be altered in the patient. Furthermore, two siblings had died in infancy with a similar clinical picture, raising the possibility of an inherited neutrophil defect. Random migration and chemotaxis, assessed by the under agarose method, were profoundly impaired. Actin polymerization, as measured by flow cytometry of N-(7-nitrobenz-2-oxa-1,3-diazol-4-yl)phallacidin (NBD-phallacidin)-stained PMNs, showed lower basal F-actin and a 1.75-fold increase in response to 10(-7) mol/L formyl-methionyl-leucyl-phenylalanine (FMLP) compared with a 4.51-fold increase in control. Microscopic examination of NBD-phallacidin-stained PMN spread on glass showed decreased area of spreading and F-actin-rich filamentous projections distinct from control. The early phase of FMLP-induced right angle light scattering was absent, similar to the effect caused by cytochalasin-B (CB), an inhibitor of actin polymerization. Accordingly, FMLP induced secretion of elastase without the addition of CB. Staphylococcus aureus killing was 50% of control whereas superoxide production response to FMLP and surface expression of CD11b were greater than twice normal. Partial defects in actin polymerization and scatter were seen in the parents and release of elastase, in the absence of CB, was also increased in both parents. Sodium dodecyl sulfate-polyacrylamide electrophoresis of whole cell proteins from the patient showed a marked decrease in an 89-Kd protein (8% of control) and a marked increase in a 47-Kd protein (4.2-fold). Both mother and father had decreased 89-Kd (77% and 42% of control) and increased 47-Kd proteins (2- and 3.4-fold), although neither had recurrent infections or chemotactic defects. These studies describe a new inherited actin dysfunction syndrome associated with severe propensity to fungal infection and draw attention to the proteins of apparent molecular weights of 89 Kd and 47 Kd, which may be of great importance in the regulation of actin polymerization in human PMNs.
Complications of central venous catheters in pediatric patients with acquired immunodeficiency syndrome.
Medical records of 18 pediatric acquired immunodeficiency syndrome patients with 24 central venous catheters (CVCs) were reviewed to determine the rates and types of CVC complications and to evaluate the influence of selected social factors, absolute granulocyte counts and CD4+ T cell counts on the rate of CVC infections. CVCs were in place for a total of 4233 days. CVCs were used for blood sampling, administration of blood products and infusions of intravenous immune globulin, parenteral nutrition and medications. Complications included catheter-related infections (8 episodes; with a rate of 1.9/1000 CVC days), occlusions (15 episodes) and unplanned catheter removals (9 episodes). Reduced CD4+ T cell counts were not predictive of CVC infection. The CVC infection rate in our pediatric acquired immunodeficiency syndrome patients was similar to rates reported in children with cancer and adults with cancer and acquired immunodeficiency syndrome.
Comparison of two techniques for sedation in dental surgery.
Forty-eight patients were randomised to receive sedation of outpatient dental surgery with midazolam. Sedation was given using the Verrill technique (24 patients) and the Glasgow Dental Hospital technique (24 patients). The differences in recovery and patient acceptability were assessed. There was no statistical difference in mean recovery times between the two groups. Memory function was examined using the Warrington memory test. Fewer patients in the Verrill group recalled the injection of local anaesthetic but they demonstrated memory defects 4 hours after sedation for words and 3 hours for faces. The Glasgow Dental Hospital group demonstrated memory defects for words up to 2 hours following sedation, but not for faces at any time. Thirty-eight patients would have dental surgery again with similar sedation. The dental surgeon found conditions for surgery inadequate in two patients. In view of the shorter duration of amnesia we recommend the Glasgow Dental Hospital technique.
Underdiagnosis and undertreatment of chronic sinusitis in children.
A review of outpatients diagnosed with sinusitis during the previous year by the allergy services of Childrens Hospital of Los Angeles and Kaiser Permanente, Orange County, revealed 34 patients who had protracted discomforting and debilitating symptoms. The mean duration of symptoms was approximately six months (range 1-14 months). None had been previously diagnosed as having sinusitis or received appropriate treatment. Although all were referred for evaluation of allergic rhinitis and/or asthma, approximately 1/3 were found to have neither of these conditions. Among patients who had asthma, their asthmatic symptoms were aggravated by the sinusitis. All patients eventually responded favorably to appropriate antibiotic therapy. Earlier diagnosis and appropriate therapy would have foreshortened the course of this very discomforting condition. In recent years, we have observed a marked increase in the number of patients referred with the chief complaint of "worsening allergy" and who were subsequently found to have sinusitis. Most of the patients experienced significant incapacitation for protracted periods before a correct diagnosis was made and treatment instituted. In all but a few cases, prompt and sustained improvement followed appropriate therapy. The purpose of this report is to present a retrospective review of 34 illustrative cases.
Human immunodeficiency virus infection in children who received transfusions in Mexico.
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Intestinal cytomegalovirus ganglioneuronitis in children with human immunodeficiency virus infection.
Cytomegalovirus in children with human immunodeficiency virus (HIV) infection can result in widespread involvement of enteric ganglion cells. Intestinal ganglioneuronitis, as noted in the two infants cited, expands the spectrum of neurologic lesions in children with acquired immunodeficiency syndrome (AIDS).
Immune functions in children treated with biosynthetic growth hormone.
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Lack of functional asplenia in HIV infection.
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Lymphomatoid granulomatosis in a 13-month-old infant.
Lymphomatoid granulomatosis is an infrequent, progressive, and frequently fatal vasculitis which typically occurs in middle aged males. We describe a 13-month-old infant with lymphomatoid granulomatosis who presented with chronic otitis media and "failure to thrive." Although it is well recognized that rheumatic conditions may result in failure to thrive, pediatricians often fail to consider this possibility and the proper diagnosis and treatment are correspondingly delayed. Lymphomatoid granulomatosis occurs with increased frequency in immunocompromised patients. In our case both hypogammaglobulinemia and persistent evidence of Epstein-Barr virus infection suggest that the infant was immunocompromised. Whether these factors predisposed this infant to lymphomatoid granulomatosis is uncertain. No patient with the onset of lymphomatoid granulomatosis prior to 7 years of age has been reported.
Recurrent fever and abdominal pain in a 4-year-old boy.
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OKT4 epitope deficiency as a cause of reduced "helper" T cells in children at risk for human immunodeficiency virus infection.
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Sedation following cardiac surgery: evaluation of alfentanil and morphine in the presence of a computerized closed loop arterial pressure controller.
Forty patients who had undergone coronary artery graft surgery and who required vasodilator therapy for postoperative hypertension were given infusions of either alfentanil or morphine together with bolus doses of midazolam for sedation and analgesia while ventilation was controlled artificially. Sodium nitroprusside (SNP) was administered to both groups using a computer-controlled closed loop system which adjusted the infusion rate to maintain a preset target arterial pressure. Target pressure +/- 5, 10, 15 and 20 mm Hg was maintained longer in the group receiving alfentanil. This group also required less SNP per hour. No difference was noted between the groups in the time taken to regain spontaneous ventilation and to extubation of the trachea, although the alfentanil group tended to be sedated more deeply during the infusion. The main advantage of alfentanil over morphine at doses used in this study was its superior ability to attenuate hypertensive responses to noxious stimuli, providing improved haemodynamic stability.
Evaluation of a new, shorter method of administration of adrenergic aerosols in the treatment of asthma.
Approximately 15 to 20 minutes are usually required to administer adrenergic agents by nebulization in the treatment of asthma. But young children often do not tolerate these prolonged treatment periods. A "5-breath" method was found to provide comparable improvements in pulmonary function and result in minimal and comparable side effects, but required only one minute to administer. The "5-breath" method may, therefore, be the method of choice in the young or otherwise uncooperative patients.
Cardiac arrest associated with halothane anesthesia in a patient receiving theophylline.
A case of a 10-year-old asthmatic patient who had cardiac arrest during induction for elective ear surgery is presented. The patient's serum theophylline level was 21.6 micrograms/mL one hour after surgery. It is likely that, since other factors that can contribute to cardiac arrest during the perioperative period were not present, the combined use of aminophylline and halothane was responsible.
Human immunodeficiency virus-associated Kaposi's sarcoma in a pediatric renal transplant recipient.
An 11-year-old boy developed Kaposi's sarcoma and progressive T lymphocyte deficiency 5 years after cadaveric kidney transplantation for end-stage renal disease. He had received 17 individual red blood cell transfusions prior to and during transplantation in 1980. Human immunodeficiency virus (HIV) was cultured from blood in cerebrospinal fluid and HIV antibodies were detected with enzyme immunoassay and immunoblot techniques. The recipient of the donor's other kidney was well and HIV antibody-negative. The patient was treated with etoposide with excellent although transient regression of tumor. Allograft function has remained stable despite minimal immunosuppressive therapy and the need for high-dose anticonvulsant therapy. This case represents the first pediatric patient with acquired immune deficiency syndrome (AIDS) and Kaposi's sarcoma following kidney transplantation.
Severe recurrent pneumonia and wheezing in a young infant.
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