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Biomedical subjects

J C Gartner

Publications and source records attributed to J C Gartner.

At least 19 recordsLinked to original sources

Fever of unknown origin.

FUO is an uncommon problem in pediatric patients if one uses a strict definition of at least 2 weeks of fever. Perhaps the best definition, as suggested by Lorin and Feigin, is more than 1 week of fever and a negative initial evaluation, including examination and preliminary laboratory results. Most patients, especially younger ones, will spontaneously improve or have common disorders, usually respiratory-related infections. Most patients will have uncommon manifestations of more commonly recognized disorders. Mortality rates, even in those series that include a week in the hospital without a diagnosis, are 15% to 20% at maximum--half that seen in most series of adult patients. Common mistakes are failure to document fever, failure to perform a complete history or physical examination, a shotgun approach that uses the laboratory to make diagnoses, and overuse or inappropriate use of newer imaging techniques in an undirected fashion. Patience, persistence, repeated histories and physical examinations, and continued observation offer the best chance of making a diagnosis in difficult cases. In the current decade, we continue to learn about new manifestations of old disorders--cat-scratch disease, Kawasaki disease, neonatal syphilis, and Epstein-Barr virus infection are examples. The use of older and well-established methods (history and physical examination) and the addition of newer techniques (ultrasound, CT, MRI, etc.) to pursue suggested diagnoses offer the best current approaches to the patient with prolonged and unexplained fever.

Child

Orthotopic liver transplantation, Epstein-Barr virus, cyclosporine, and lymphoproliferative disease: a growing concern.

Lymphoproliferative disease (LPD) is a well-recognized complication of both solid organ and bone marrow transplantations. The occurrence of LPD in these settings is related in part to the use of the immunosuppressive agent cyclosporine. We report 12 cases of LPD after orthotopic liver transplantations in 132 pediatric patients. Lymphoproliferative disease occurred as one of three clinical syndromes: (1) lymphadenopathic, (2) systemic, and (3) lymphomatous. Effective management of LPD with excisional therapy or reduction of immunosuppressive medications or both resulted in the survival of 7 of 12 patients. In an alarming and increasing percentage of patients after orthotopic liver transplantation, progressive LPD develops with lethal outcome (5/12 patients). Early recognition of LPD and aggressive intervention may improve outcome in this group.

Child

Fatal pulmonary aspergillosis presenting as acute eosinophilic pneumonia in a previously healthy child.

A previously healthy boy presented with cough and diffuse pulmonary interstitial infiltrates. Acute eosinophilic pneumonia was diagnosed by bronchoalveolar lavage in the absence of a demonstrable infectious etiologic agent. Corticosteroid therapy resulted in immediate improvement but was followed by respiratory distress and death from invasive aspergillosis and Pseudomonas cepacia sepsis.

Acute Disease

Varicella in pediatric orthotopic liver transplant recipients.

From May 1981 to May 1984, 90 pediatric patients underwent liver transplantation and 65 patients survived as of May 1986. Two of the nonsurvivors died with complications related to clinical varicella. Of these 67 patients (65 survivors and two nonsurvivors who died of varicella-related causes), 51 patients were determined to be varicella susceptible. Clinical disease developed in no patients with serologic evidence or clinical history of varicella prior to transplantation. Eighteen susceptible patients were exposed and received zoster immune globulin and varicella did not develop. Clinical disease developed in eight patients despite zoster immune globulin, although one patient received it 96 hours after exposure. Six patients received no zoster immune globulin and clinical varicella developed. In all, varicella developed in 14 patients. Thirteen were admitted to the hospital and treated with intravenous acyclovir. Of those treated, two died of causes related to complications of varicella. The remaining patients treated with acyclovir had mild disease. The one patient not treated with acyclovir also had mild disease. We conclude that patients contracting varicella after liver transplantation while receiving maintenance immunosuppressive agents should be treated with intravenous acyclovir. Generally, when treated with acyclovir while receiving maintenance immunosuppressive drugs, these patients have mild clinical disease. Patients recently treated with high-dose prednisone and cyclosporine may have severe clinical disease resulting in death.

Acyclovir

Acute profound dystonia in infants with glutaric acidemia.

Acute profound dystonia developed in three previously well infants who were found to have glutaryl-CoA dehydrogenase deficiency in cultured skin fibroblasts. Two patients had excessive urinary excretion of glutaric acid, but one did not. Neuroradiologic studies performed in all three patients at the onset of their illnesses revealed large CSF-containing spaces both within the sylvian fissures and anterior to the temporal lobes. Pathologic examination of the brain of one patient demonstrated cerebral and cerebellar atrophy, shrinkage of the putamen, and white matter vacuolation. Glutaric acidemia may be a common cause of acquired persistent dystonia or choreoathetosis in infancy.

Atrophy

Lumbar cutaneous hemangiomas as indicators of tethered spinal cords.

Children with cutaneous hemangiomas in the lumbar region often also have tethered spinal cords. This diagnosis can be overlooked, because children with cutaneous hemangiomas may be otherwise asymptomatic and neurologically normal. From 1983 to 1987, six consecutive children with lumbar cutaneous hemangiomas underwent magnetic resonance imaging, and a seventh had a myelogram. In all seven children, despite their normal neurologic status, tethered spinal cords were diagnosed. All children had operations, which confirmed this finding. None had postoperative neurologic deficits. Infants and children with similar lumbar cutaneous hemangiomas should have magnetic resonance scans to detect the presence of tethered spinal cords.

Child, Preschool

Kawasaki disease and perineal rash.

During the past several years, we treated seven children with Kawasaki disease who developed a distinctive but rarely described perineal rash. This rash began three to four days from onset of the illness and desquamated in all instances by days 5 to 7. The presence of this rash may facilitate early diagnosis of Kawasaki disease and, hence, may influence the initiation of early treatment.

Child

Endothelial proliferation in paroxysmal nocturnal hemoglobinuria.

Paroxysmal nocturnal hemoglobinuria (PNH) is a disorder of stem cells that is associated with venous thrombosis in multiple organ sites. We present a patient with necrotizing pharyngitis and intestinal infarction who has classic laboratory findings for PNH: positive complement-mediated acid and sucrose hemolysis and absence of decay accelerating factor (DAF) in peripheral blood leukocytes. Histopathologic examination of tissue from tonsil and large bowel demonstrated not only venous thrombosis but unusual proliferation of endothelial cells (papillary endothelial hyperplasia). This has not been described previously. We speculate that the endothelial cell in PNH may share in the defective regulation of complement activity. Venous thrombosis could precipitate or be a consequence of these vascular changes.

Adolescent

Changes in life-style after liver transplantation.

Sixty-five pediatric patients who received liver transplants between May 1981 and May 1984 were observed for as many as 5 years and examined for changes in life-style. Children were less frequently hospitalized, spent less time hospitalized, required fewer medications, and generally had excellent liver and renal function after hepatic transplantation as compared with their pretransplantation status. Most children were in age-appropriate and standard school classes or were only 1 year behind. Cognitive abilities remained unchanged. Children improved in gross motor function and patients' behavior significantly improved according to parents' perceptions. Enuresis was more prevalent, however, than in the population of children who had not received liver transplants. Parental divorce rates were no greater than those reported for other families with chronically ill children. Overall, objective changes in life-style as well as parents' perceptions of behavior of children appear to be improved after liver transplantation.

Achievement

Choosing a pediatric recipient for orthotopic liver transplantation.

Between March 3, 1981, and June 1, 1984, 216 children were evaluated for orthotopic liver transplantation. Of the 216 patients, 117 (55%) had received at least one liver transplant by June 1, 1985. Fifty-five (25%) died before transplantation. The 117 patients who received transplants were grouped according to severity of disease and degree of general decompensation at the time of transplantation. The severity of a patient's medical condition with the possible exception of deep hepatic coma, did not predict outcome following orthotopic liver transplantation. Seventy variables were assessed at the time of the evaluation. Twenty-three of the 70 variables were found to have prognostic significance with regard to death from progressive liver disease before transplantation. These 23 variables were incorporated into a multivariate model to provide a means of determining the relative risk of death among pediatric patients with end-stage liver disease. This information may allow more informed selection of candidates awaiting liver transplantation.

Ambulatory Care

Pediatric liver transplantation: patient evaluation and selection, infectious complications, and life-style after transplantation.

Liver transplantation is an increasingly accepted treatment for children with end-stage liver disease. Evaluation of the patient and appropriate patient selection for transplantation will become increasingly important issues as more and more children come to transplantation and compete for available organs. Numerous complications occur after transplantation, including infections. We have summarized our experience with bacterial, fungal, and viral infections in these patients and emphasize the need for continued improvement in immune suppressive drugs and regimens to minimize such complications. And finally, information presented on 65 pediatric patients followed 2 to 5 years suggests that, despite numerous complications and often prolonged hospitalization for transplantation, life-style after transplantation appears to be significantly improved.

Biliary Atresia

Progression of neurovisceral storage disease with supranuclear ophthalmoplegia following orthotopic liver transplantation.

A 7-year-old girl with progressive ataxia, spasticity, supranuclear ophthalmoplegia, and sea-blue histiocytes in her bone marrow underwent orthotopic liver transplantation for hepatocellular carcinoma. After an initial period of stabilization, she has shown progression of neurologic symptoms with recurrence of storage material in the transplanted liver.

Carcinoma, Hepatocellular

Acquired immunodeficiency syndrome in the child of a haemophiliac.

Oral thrush developed during the second month of life in the 5-month-old son of a patient with haemophilia A. He did not feed well, and interstitial pneumonitis, lymphadenopathy, hepatosplenomegaly, and a cellular immune defect consistent with the acquired immunodeficiency syndrome (AIDS) followed. Both parents had signs of pre-AIDS during the year before their son's illness. Transmission presumably occurred in 3 steps: parenterally, via factor VIII concentrate in the haemophiliac; heterosexually, from the haemophiliac to his wife; and vertically, from mother to infant, or via close paternal-infant or maternal-infant contact. This first report of AIDS in the child of a haemophiliac supports the theory that AIDS is caused by an infectious agent. Concentrate-treated haemophiliacs may transmit this agent to their spouses or children, resulting in pre-AIDS or AIDS.

Acquired Immunodeficiency Syndrome

Meningitis from a pneumococcus moderately resistant to penicillin.

Strains of pneumococci moderately resistant to penicillin were recovered from three patients at the Children's Hospital of Pittsburgh during 1978. One of these children had meningitis and showed signs of early relapse despite high doses of penicillin G potassium. Routine screening of pneumococci for penicillin resistance is recommended.

Chloramphenicol