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Biomedical subjects

R S Shapiro

Publications and source records attributed to R S Shapiro.

At least 55 records · Page 3Linked to original sources
PubMed1994

Immunosuppression: preliminary results of alternative maintenance therapy for familial hemophagocytic lymphohistocytosis (FHL).

Hemophagocytic lymphohistiocytosis (HLH) describes a group of disorders with similar clinical features that are associated with a very high mortality rate. Patients with HLH, and particularly the infantile form referred to as familial hemophagocytic lymphohistiocytosis (FHL), are often treated with multiple courses of epipodophyllotoxins, such as etoposide, for prolonged periods of time. Because of the concern regarding the risk of epipodophyllotoxin-induced acute myelogenous leukemia (AML) we have explored the use of immunosuppression as maintenance therapy for patients with FHL while they await the only known definitive treatment, i.e., bone marrow transplantation (BMT). We report 2 infants with FHL who had significant central nervous system involvement at diagnosis. Both were initially treated with etoposide, methotrexate, and glucocorticosteroids. Once clinical improvement was achieved these patients were successfully maintained in clinical remission of FHL on daily cyclosporine A (CSA) and glucocorticosteroids along with intermittent intrathecal methotrexate for 5 months until appropriate unrelated donors could be identified for BMT.

Cyclosporine↗
PubMed1994

Liability issues in the management of pain.

Liability issues associated with pain management are important to health-care providers, patients, pharmaceutical companies, manufacturers of pain-management devices, health-care payors, and society at large. This article discusses five specific legal liability concerns: (a) health-care providers' liability to patients and/or exposure to professional discipline for inappropriate pain management, (b) health-care providers' liability to third parties for injury caused by patients treated for pain, (c) the legal distinction between pain management and euthanasia or physician-assisted suicide, (d) health-care payors' liability to patients for cost-containment decisions that impact on pain management, and (e) manufacturers' and health-care providers' liability for the risks and side effects of prescription drugs and pain-management devices.

Cost Control↗
PubMed1994

Legal bases for the control of analgesic drugs.

Governments throughout the world have struggled for decades to ensure the availability of narcotic analgesics for legitimate medical and scientific purposes while controlling the abuse and illegal diversion of such substances. While the international drug-control system has effectively limited illicit trafficking of opioids, concerns remain about its effectiveness in ensuring the availability of these drugs for legitimate purposes. In the United States, federal legislation accommodates the use of controlled substances for medical and scientific purposes more effectively than does state law. Many states' controlled substance laws hinder appropriate opioid prescribing through (a) the use of ill-defined terms, (b) restriction of pain prescriptions to a specific number of dosage units; and/or (c) utilization of multiple-copy prescription programs. A more efficient state approach to monitoring inappropriate schedule II prescribing and dispensing may be through an electronic, computer-based pharmacy point-of-sale system, through which pharmacists can be alerted instantaneously to patients receiving the same drug from multiple pharmacies. In addition, states should consider modifying their approaches to drug abuse by adopting the revised Uniform Controlled Substances Act and/or establishing state pain initiatives.

Analgesics↗
PubMed1994

Lymphoproliferative disorders and other tumors complicating immunodeficiencies.

Lymphoproliferative disorders and selected carcinomas which occur as complications of primary or secondary immunodeficiencies are frequently fatal. The incidence rates of these cancers vary from 1% to as high as 25% among specific groups of persons with primary (genetically-determined) immunodeficiencies as well as acquired immunodeficiencies, including immunosuppressed organ transplant recipients and individuals infected with HIV. Lymphoproliferative disorders including Epstein Barr virus (EBV) associated B cell lymphoproliferative disease (BLPD) and Hodgkin's disease represent the predominant category of tumors in both primary and acquired immunodeficiencies. EBV is an important cofactor common to many, but not all, B cell "lymphomas." Immunodeficient individuals who are at risk for developing EBV BLPD may demonstrate both inadequate immune responses to the virus as well as generalized immunoregulatory dysfunction reflected as imbalances in cytokine production favoring the proliferation of transformed B lymphocytes. Historically, the success of treatment of lymphoproliferative disorders in immunodeficiencies with conventional multi agent chemotherapies and/or radiation has been limited by unfavorable tumor response rates and high morbidity and mortality related to intercurrent opportunistic infections. With improvements in supportive care and the use of recombinant biologic response modifiers such as alpha interferon and/or other immunotherapies to treat EBV BLPD, survival of immunodeficient hosts following tumor diagnosis may improve. In addition to lymphoproliferative disorders, patients with congenital immunodeficiencies associated with IgA deficiency (including ataxia telangiectasia and Common Variable Immunodeficiency) are at increased risk for gastrointestinal carcinomas. Early detection and surgical excision of such tumors can result in prolonged survival in such patients.

Humans↗
PubMed1993

MR imaging in adults with Gaucher disease type I: evaluation of marrow involvement and disease activity.

An investigation was conducted to determine the usefulness of magnetic resonance imaging (MRI) in the evaluation of bone marrow involvement in patients with Gaucher disease type I. T1- and T2-weighted images were obtained of the lower extremities of 29 adult patients. Patients were classified into one of three groups based on marrow signal patterns on T1- and T2-weighted images as well as change in signal intensity from T1- to T2-weighted images. An increase in signal intensity from T1- to T2-weighted images was the criterion for an "active process" within the bone marrow. Classification of the 29 patients produced the following results: group A: normal, 4 patients; group B: marrow infiltration, 16 patients; group C: marrow infiltration plus active marrow process, 9 patients. Correlation with clinical findings revealed that all nine patients with evidence of an active marrow process on MRI (group C) had acute bone pain. Conversely, only one of the remaining 20 patients (groups A and B) had bone pain. There was no correlation between disease activity and findings on conventional radiographs. We conclude the MRI provides an excellent noninvasive assessment of the extent and activity of marrow involvement in type I Gaucher disease.

Adult↗
PubMed1993

Prolonged neutropenia resulting from antibodies to neutrophil-specific antigen NB1 following marrow transplantation.

Marrow graft failure is a significant cause of morbidity following bone marrow transplantation. A case is reported of marrow graft failure due to neutrophil antibodies. A 13-year-old girl with a large granular lymphocytosis and chronic neutropenia was treated with granulocyte transfusions prior to undergoing a transplant with bone marrow from a partially matched, unrelated donor. Following the transplant, a bone marrow biopsy showed engraftment of donor myeloid cells, but the recipient remained neutropenic. Testing of the serum for neutrophil antibodies found that the recipient's serum had a high-titer neutrophil antibody. Immunoprecipitation studies using the marrow recipient's serum and 125I surface-labeled neutrophils showed that the antibody reacted to the neutrophil-specific antigen NB1. Phenotyping of neutrophils from the marrow donor found that they expressed NB1 antigen, and, in a crossmatch assay, the recipient's serum reacted with donor neutrophils. Despite treatment with granulocyte-macrophage--colony-stimulating factor, the marrow transplant recipient remained neutropenic and died of polymicrobial sepsis and aspergillosis 38 days after the transplant. The presence of high-titer antibodies to neutrophil-specific antigen NB1 in this patient following transplant likely prevented the recovery of her peripheral blood neutrophils and contributed to her death.

Adolescent↗
PubMed1993

CT findings after percutaneous biliary procedures.

A prospective study was performed to determine the frequency, type, and extent of abnormalities depicted with computed tomography (CT) after percutaneous biliary procedures (PBPs). Abdominal CT scans were obtained 24-72 hours after the PBP in 31 consecutive cases in 29 patients. Fifteen abnormalities were proved with CT in 14 patients (45%), as follows: subcapsular hematoma (two patients), subcapsular or perihepatic fluid collection (three patients), intrahepatic hematoma (three patients), nonspecific intrahepatic fluid collection (three patients), subcutaneous hematoma at the puncture site (one patient), free intraperitoneal air (one patient), intraperitoneal collection of contrast material (one patient), and inadvertent transxiphoid catheter tract (one patient). Only five of these patients had clinically apparent post-PBP complications that could be explained with CT findings. The 14 patients with positive CT findings required more needle passes (mean, 8.3 vs 4.6) during the PBP, had a difficult PBP more often (five patients [36%] vs four patients [27%]), and had more frequent placement of an internal-external drain (nine patients [64%]) than those with negative findings (eight patients [53%]). Positive findings on CT scans are common after a PBP and often are not associated with clinical symptoms.

Bile Ducts↗
PubMed1993

Variability of hepatic vein Doppler tracings in normal subjects.

A prospective study was undertaken to determine the variability of hepatic vein Doppler waveforms in normal subjects. Seventy-five patients without liver or heart disease underwent Doppler examination of the middle hepatic vein. Normal triphasic tracings were observed in 68 subjects, while flattened tracings were observed in seven subjects. In addition, in four of the seven subjects with flattened tracings, an increase in pulsatility was seen during prolonged inspiration. We conclude that there is variability of hepatic vein tracings in normal subjects and that respiratory maneuvers can alter these tracings.

Adult↗
PubMed1993

Color Doppler sonography of vascular malformations of the liver.

Nine cases of intrahepatic vascular malformations diagnosed using color Doppler sonography are described. These consisted of six cases of intrahepatic portal-hepatic venous shunts and three cases of arteriovenous fistulas. Among these is a case of multiple intrahepatic portal-systemic shunts. The sonographic findings and theories explaining the formation of vascular malformations in the liver are discussed.

Adult↗
PubMed1992

Primary immunodeficiencies: genetic risk factors for lymphoma.

It has been estimated that up to 25% of patients with certain genetically determined immunodeficiencies will develop tumors, primarily B-cell lymphomas, during their lifetime. Epstein-Barr virus appears to be an important cofactor in the development of lymphoproliferative disorders in patients with primary immunodeficiencies, as well as acquired immunodeficiencies. Additionally, host defects in immunoregulation and/or gene rearrangement, which are features of certain primary immunodeficiencies, probably contribute to the risk of lymphomagenesis in patients at risk.

Adolescent↗
PubMed1992

Unrelated donor bone marrow transplantation for correction of lethal congenital immunodeficiencies.

Unrelated donor marrow transplantation was undertaken in eight infants with severe combined immunodeficiency (SCID) and two children each with Wiskott-Aldrich syndrome (WAS) and Chediak-Higashi syndrome (CHS) who did not have histocompatible siblings. Donors for three patients were phenotypically matched at all HLA-A, B, Dr, and Dw loci, whereas nine donors were mismatched from the recipients at one of the HLA-A or B loci but phenotypically identical at evaluable D loci. All but one patient received conditioning chemotherapy and/or radiotherapy before infusion of donor marrow, which was not T-cell depleted. Prophylaxis for graft-versus-host disease (GVHD) consisted of methotrexate and prednisone combined with either cyclosporine A (six patients), antithymocyte globulin (five patients), or anti-CD5 ricin A chain immunotoxin (one patient). All patients engrafted with donor cells, and only 4 of 12 experienced any GVHD (1 of 8 SCID, 1 of 2 WAS, 2 of 2 CHS). Two children who developed grade II and two who developed grade III GVHD were successfully treated and all are now alive, off immuno-suppressive therapy, with no evidence of chronic GVHD greater than 18 months after transplant. Ten patients are alive with excellent immunoreconstitution greater than or equal to 1 year to greater than or equal to 3 years after transplant; actuarial survival is predicted to be 83% with a median follow-up of 2 years. Two children with SCID succumbed to pre-existing opportunistic infection early posttransplant. We conclude that closely matched unrelated donor bone marrow transplantation can correct congenital immunodeficiencies including variants of SCID, WAS, and CHS, with an acceptably low incidence of transplant-related complications, principally GVHD.

Bone Marrow Transplantation↗
PubMed1992

Renal aspergilloma: an unusual cause of infection in a patient with the acquired immunodeficiency syndrome.

The case of a 36-year-old man with the acquired immunodeficiency syndrome (AIDS) and a renal aspergilloma is reported. Aspergillus infections are uncommon in patients with AIDS. Isolated renal aspergillomas have rarely been reported in the non-AIDS population (14 cases) and have never been reported in a patient with AIDS. The patient we describe was clinically symptomatic and initially treated medically, but he did not respond to intravenous amphotericin and oral itraconazole. He eventually required nephrectomy; however, there was local recurrence of the aspergilloma postoperatively. We comment on some issues in the spectrum of Aspergillus infections in AIDS and review the literature on the manifestations and treatment of renal aspergillomas.

Acquired Immunodeficiency Syndrome↗
PubMed1992

Junctional parenchyma: revised definition of hypertrophic column of Bertin.

The so-called hypertrophic column of Bertin is a normal variation and simply represents unresorbed polar parenchyma of one or both of the two subkidneys that fuse to form a normal kidney. It contains renal cortex, pyramids, and columns (septa) of Bertin. (Since all elements of the tissue are normal, that is, neither hypertrophic nor a displaced or embryonic rest, it is referred to as "junctional parenchyma.") When a masslike lesion is suspected at sonography or excretory urography, diagnosis of junctional parenchyma can be made with a high degree of certainty when sonography shows that the structure (a) is located between the overlapping portion of two renal sinus systems, (b) is bordered by a junctional parenchymal line and defect, (c) contains renal cortex, pyramids, and columns of Bertin, and (d) contains renal cortex that is continuous with the adjacent renal cortex of the same subkidney.

Adult↗
PubMed1992

Enzyme augmentation in moderate to life-threatening Gaucher disease.

Gaucher disease type 1 (GD type 1) is the most prevalent lysosomal storage disease and has its highest frequency in the Ashkenazi Jewish population. Deficiency of the enzyme, acid beta-glucosidase, results in the deposition of glucocerebroside primarily in macrophages. The accumulation of such "Gaucher cells" leads to visceromegaly, hepatic and bone marrow dysfunction, hypersplenism, and bony disease. Eleven GD type 1 patients, ages 4-52 y, with moderate to life-threatening manifestations, received 6-12 mo of enzyme augmentation with a macrophage-targeted acid beta-glucosidase preparation. Within 6 mo, substantial increases in Hb levels (mean = +30%) and platelet counts (mean = +39%) were observed. Hepatic and splenic volumes decreased by approximately 20% (range = 3-35%) and approximately 35% (20-52%), respectively. Hematologic and hepatic volume improvements were similar in the splenectomized (n = 4) and nonsplenectomized (n = 7) patient groups. In this patient population, no major differences were observed in the hematologic and visceral improvements with enzyme doses of 30, 50, or 60 IU/kg administered every 2 wk. Normal levels of acid beta-glucosidase activity were present in hepatic autopsy samples from one patient 11 d after enzyme infusion. In comparison, exogenous activity was absent from brain and lung specimens of the same patient. High levels (approximately 10-fold normal) were present in bone marrow samples from two patients obtained at 1 and 11 d after infusions. These studies demonstrate biochemical and clinical improvements by targeted enzyme augmentation in GD type 1, even in far advanced, life-threatening involvement.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1992

Paroxysmal sneezing in children: two new cases.

Paroxysmal sneezing is an uncommon condition primarily affecting adolescents. Most of the reported cases were thought to be psychogenic, and only two were felt to be due to nasal sensitivity. This paper reports two adolescents with paroxysmal sneezing, neither of whom had apparent psychologic or emotional problems. In one child the sneezing continued during sleep. The other child was successfully treated with topical nasal anesthesia. Both children were felt to have nasal sensitivity as the etiology of their paroxysmal sneezing. The evaluation of the patient with paroxysmal sneezing requires a thorough history and physical examination. One must not assume that every case of paroxysmal sneezing is of psychogenic origin. Topical nasal anesthesia should be tried for control of intractable paroxysmal sneezing.

Administration, Intranasal↗
PubMed1991

B-cell lymphoproliferative disorders after bone marrow transplant. An analysis of ten cases with emphasis on Epstein-Barr virus detection by in situ hybridization.

Ten patients with B-cell lymphoproliferative disorders (BLPD) after bone marrow transplant were studied in a retrospective analysis of 81 specimens available from biopsy and autopsy material. Histologic review, immunophenotyping, and in situ hybridization (ISH) for Epstein-Barr virus (EBV) sequences were done. Sixty-four specimens showed morphologic evidence of BLPD, demonstrating a heterogeneous spectrum with various degrees of plasmacytoid differentiation. Immunophenotypic evidence of clonality was found in six patients. The ISH detected EBV sequences in all ten patients, including 60 of the 64 specimens with morphologic evidence of BLPD. In addition, ISH identified EBV-infected lymphoid cells in two of 17 sites without morphologic evidence of BLPD. These data demonstrate the utility of ISH for detecting EBV genome in this setting and provide further evidence for the etiologic role of EBV in the pathogenesis of BLPD.

Adolescent↗