Biomedical subjects
Publications and source records attributed to R S Shapiro.
A 39-year-old woman presented with a vermiform superior conjunctival mass that progressively enlarged during 4 years of follow-up. Although magnetic resonance imaging suggested that the lesion was located in the superior forniceal conjunctiva, surgical exploration disclosed a vascular anomaly (varix) that extended deep into the orbit. The clinical features and management of orbital varices are discussed.
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Tracheal gas insufflation (TGI) improves the efficiency of CO2 elimination by reducing the CO2-laden dead space of the airways. The effect of TGI on PaCO2 diminishes in the setting of acute lung injury (ALI) because an increased alveolar component dominates the total physiologic dead space. Nevertheless, adopting a strategy of permissive hypercapnia should partially offset the decreased efficacy of TGI by increasing CO2 concentration in the proximal airways. To examine these issues we studied the CO2 removal efficacy of expiratory TGI as an adjunct to conventional mechanical ventilation (CMV) before and after oleic acid-induced lung injury (OAI). We first examined the effect of TGI before and after OAI, keeping tidal volume (VT) and frequency constant, and allowing PaCO2 to increase after OAI. We then tested TGI efficiency after matching PaCO2 after OAI to its pre-OAI level by increasing VT (post-OA/VT stage). PaCO2 was 53 +/- 3, 79 +/- 21, and 52 +/- 4 mm Hg in the pre-OAI, post-OAI, and post-OA/VT stages of CMV, respectively. The corresponding decrements in PaCO2 produced by TGI at a flow rate of 10 L/min were 16 +/- 3, 24 +/- 10, and 10 +/- 2 mm Hg, respectively. TGI decreased total physiologic dead space per breath (VD) by 56, 31, and 28 ml during the pre-OAI, post-OAI, and post-OA/VT stages, respectively. Despite a smaller reduction in VD during the post-OAI stage, the effect of TGI on PaCO2 was preserved because of the relatively high PaCO2 prior to its initiation.(ABSTRACT TRUNCATED AT 250 WORDS)
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.
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.
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.
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.
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.
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)
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.
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.
A 20-gauge Chiba needle with a stylet embedded with polyvinyldifluoride (PVDF), a polymer that acts as an acoustic-electric transducer, facilitated a variety of diagnostic and therapeutic ultrasound (US)-guided interventions in nine patients. PVDF receives acoustic energy from the US scanner and transmits an electrical signal through the stylet and a shielded cable to the scanner, which results in appearance of a bright echo on the monitor at the location of the needle tip.
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A 35-year-old woman had a 4-year history of skin depression on the right side of both the forehead and chin. A biopsy specimen showed a normal dermis and an absence of subcutaneous tissue. She also had Poland syndrome on the left side of the body, with loss of the sternocostal portion of the greater pectoral muscle. Significant cosmetic improvement was achieved with dermal fat grafts and silicone implants.
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The "comet tail" is a well-known ultrasound artifact that appears as a series of parallel bands radiating from a source. This artifact was observed in the gallbladder lumen in eight of 10 patients after extracorporeal shock wave lithotripsy of radiolucent calculi. To investigate the cause of the comet tail artifact, cholesterol crystals were studied in an in vitro model. The comet-tail appearance was reproduced in vitro and was found to be directly related to the interrogating frequency and the size of the crystal aggregates. The authors conclude that cholesterol crystals can serve as the source of the comet-tail artifact secondary to reverberation within the crystals.
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