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Biomedical subjects

R S Shapiro

Publications and source records attributed to R S Shapiro.

At least 91 records · Page 5Linked to original sources
PubMed1988

Epstein-Barr virus associated B cell lymphoproliferative disorders following bone marrow transplantation.

B cell lymphoproliferative disorders (BLPD) developed in eight patients following bone marrow transplantation (BMT) for leukemia (five patients) or immunodeficiency (three patients). Recipients of T depleted marrow from a mismatched donor were at particularly high risk of this complication. Six of 25 (24%) recipients of mismatched T depleted bone marrow developed BLPD. In contrast, none of 47 matched T depleted transplants, one of ten (10%) who received non-depleted marrow from an unrelated donor, and only one of 424 matched non-depleted transplants were associated with BLPD. Epstein-Barr virus (EBV) specific serology and DNA hybridization studies demonstrating five to 50 copies of EBV genome/cell in involved tissues implicate this virus as an associated etiologic agent. Restriction fragment length polymorphism (RFLP) and cytogenetic analysis of involved tissue demonstrated donor origin (five of seven) or host origin (two of seven). Histologic appearance was similar to EBV-induced polymorphic B cell proliferations described following solid organ transplantation, or which occur de novo in primary immunodeficiency. Six of seven patients with adequate tissue available for study were found to have monoclonal proliferations by: in situ immunofluorescence (six of seven), and/or immunoglobulin gene rearrangement, (four of six). Cytogenetic analysis of involved tissues from four patients showed a normal karyotype, whereas two had multiple clonal chromosomal abnormalities. Seven patients died despite aggressive attempts at therapy with combinations of antiviral, immunologic, and chemotherapeutic agents.

Antibodies, Viral↗
PubMed1988

Relationship of immunodeficiency to lymphoid malignancy.

Individuals with either primary or secondary immunodeficiencies are at high risk to develop not only infections but also malignancy (especially of the lymphoid system). The major focus of this paper is on malignancies that develop in immunodeficiency syndromes, particularly malignancies in naturally occurring immunodeficiencies and following bone marrow transplantation (BMT). As of August, 1986, 514 cases of naturally occurring immunodeficiencies have been registered at the Immunodeficiency Cancer Registry. Overall non-Hodgkin's lymphomas predominate in these patients, accounting for 48.6% of all cases. Non-Hodgkin's lymphoma is the predominant malignancy in ataxia-telangiectasia, common variable immunodeficiency, Wiskott-Aldrich syndrome (WAS) and severe combined immunodeficiency (SCID). The histopathology of the lymphomas differs somewhat in each of the disorders. In WAS, large cell "histiocytic" lymphoma predominates, with most cases having the features of B lymphocytes, including pleomorphic immunocytoma and immunoblastic lymphoma. Non-Hodgkin's lymphoma in SCID also generally has B cell features and in some cases multiple copies of Epstein-Barr virus (EBV) genomic DNA have been found in tumor tissue. In contrast to ataxia-telangiectasia, in which non-Hodgkin's lymphoma is also the predominant neoplasm, the morphology and cell marker characteristics are more similar to those seen in nonimmunodeficient children. The lymphomas in ataxia-telangiectasia are very heterogeneous with representation from all the major histologic subtypes. We have found no relationship between the degree of immunodeficiency and the development of malignancy. Immunodeficiency following BMT, as in naturally occurring immunodeficiencies, appears to predispose patients to the development of lymphoid malignancy, especially for recipients of partially mismatched bone marrow. In Minnesota 8 patients have developed B cell lympho-proliferative disorders (BLPD) following BMT.(ABSTRACT TRUNCATED AT 250 WORDS)

Ataxia Telangiectasia↗
PubMed1988

Otologic findings in an Inuit population of cleft palate children.

Otitis media with effusion is almost universal in infants with cleft palate. Inuit children have a very high incidence of otitis media. The otologic problems of Inuit infants and children with cleft palate have not previously been reported. The current study presents 14 Inuit children with cleft palate. All show significant otitis media with effusion or chronic otitis media with tympanic membrane perforation. The management of otitis media in this special population of cleft palate children is discussed.

Adolescent↗
PubMed1987

Cutaneous manifestations of Corynebacterium group JK sepsis.

A 14-year-old boy developed group JK corynebacteria sepsis and a generalized erythematous macular and papular skin eruption following chemotherapy for relapse of acute lymphocytic leukemia. Lesional skin biopsy demonstrated effacement of eccrine glands by numerous pleomorphic gram-positive bacilli, morphologically consistent with Corynebacterium and confirmed by culture. This is the first known report documenting the generalized skin manifestations and histopathologic features associated with Corynebacterium sepsis.

Adolescent↗
PubMed1987

Pruritus as a presenting sign of acquired immunodeficiency syndrome.

A 65-year-old man with insulin-dependent diabetes developed intractable pruritus preceding weight loss and increasing fatiguability. Esophagogastroduodenoscopy revealed infection with Candida, cytomegalovirus, and Cryptosporidium. His T cell helper/suppressor ratio was inverted, and the serum human immunodeficiency virus (HIV) antibody was positive. Results of an extensive evaluation for internal malignancy were negative. Despite optimal care, the patient died 12 weeks after his initial hospitalization. We believe that HIV infection should be added to the list of underlying disorders that may present with pruritus.

Acquired Immunodeficiency Syndrome↗
PubMed1987

B-cell lymphoproliferative disorders in children after bone marrow transplantation: radiologic manifestations.

The radiographic findings in five pediatric patients in whom unregulated B-cell lymphoproliferative disorders developed following bone marrow transplantation are described. Four patients received T-cell-depleted bone marrow from mismatched donors and one received nondepleted marrow from a matched sibling donor. These disorders are similar to B-cell lymphoproliferative disorders that have been described in other immunosuppressed hosts. They are associated with Epstein-Barr virus and range from polyclonal proliferation without cytogenetic abnormalities to monoclonal lymphoma with clonal cytogenetic changes. Unlike other postallograft lymphoproliferative processes, B-cell lymphoproliferative disorders in these patients have not responded to antiviral therapy, immunologic therapy, or chemotherapy. The radiographic patterns of disease include diffuse or focal hepatic involvement; gallbladder wall thickening; and pulmonary, soft-tissue, and basal-ganglion masses. These radiologic findings are not specific and evaluation of tissue histology is required for diagnosis.

Adolescent↗
PubMed1987

Molecular genetic rearrangements distinguish pre- and post-bone marrow transplantation lymphoproliferative processes.

Chronic myelocytic leukemia (CML) may display a lymphoproliferative phase (lymphoid blast crisis) that is generally of B cell phenotype. Since lymphoproliferative disorders may occur following bone marrow transplantation (BMT), it may be difficult to distinguish posttransplant relapse of CML lymphoid blast crisis from de novo lymphoproliferation. Lymphoid blast crisis cells from a patient with CML displayed immunoglobulin heavy chain gene (C mu) rearrangement before BMT. Following BMT the patient developed a lymphoproliferative disorder involving multiple organs. Clonal rearrangement of C mu was demonstrated in several involved tissues. The rearranged C mu restriction fragment was distinct from that displayed before BMT. Additionally, rearrangement of the breakpoint cluster region (bcr) was demonstrated in the pretransplant blast crisis sample, but not in the posttransplant lymphoproliferation samples, thus confirming that these lymphoproliferative disorders were distinct. Molecular genetic techniques offer powerful diagnostic tools for monitoring the course of patients with CML undergoing BMT.

Blast Crisis↗
PubMed1987

Button battery ingestion: a case report and review of the literature.

Disc batteries represent a distinct type of pediatric foreign body because of their potential for severe morbidity and mortality. We present the case of an 18-month-old boy who ingested a lithium battery and, despite a delay in diagnosis, had a favorable outcome. Button batteries are being used with increasing frequency in a variety of devices including hearing aids, watches and calculators. Most contain a heavy metal such as mercury, and an alkaline electrolyte. Esophageal impaction results in a corrosive esophagitis leading to perforation with the attendant sequelae. Injury may occur by four mechanisms: (1) electrolyte leakage from batteries; (2) alkali produced from external currents; (3) mercury toxicity, and (4) pressure necrosis. Early diagnosis and urgent removal are required for esophageal impaction. More distal sites can be managed conservatively with spontaneous passage occurring in the majority of cases. Prevention should be directed at placing the cells out of reach and child-proofing battery-powered appliances.

Child, Preschool↗
PubMed1984

Epignathus: a report of two cases.

Two cases of epignathus are presented. The classification and clinical and radiological diagnoses are reviewed. Epignathi are rare embryological tumors, but with better preoperative assessment and awareness, safe surgical excision can be carried out. A careful long-term follow-up with regular examinations is essential to detect any residual lesions or extensions of the original embryological malformation.

Angiography↗
PubMed1984

Lorcainide kinetics and protein binding in patients with end-stage renal disease.

Lorcainide is a new antiarrhythmic drug undergoing clinical investigation for management of patients with ventricular arrhythmias. In this study we investigated the kinetic profile of lorcainide in nine patients with end-stage renal disease. A single intravenous bolus of 100 mg of the drug was injected while the patients were undergoing hemodialysis or during the off-dialysis period. Renal disease did not alter the kinetic properties of lorcainide; the elimination t1/2 beta during hemodialysis was 8.61 +/- 6.35 h, not significantly different from 7.04 +/- 4.12 h off-dialysis. Serum protein binding of lorcainide was investigated in vitro, and the percent binding of lorcainide to serum proteins of normal volunteers and renal or cardiac patients was not significantly different. These data suggest that renal disease should not alter either the dose or the dosing interval of lorcainide.

Adult↗
PubMed1982

Partial adenoidectomy.

Adenoidectomy in the presence of an inadequate palate may result in velopharyngeal insufficiency. When an adenoidectomy is indicated in a child whose palate is not fully adequate, a partial adenoidectomy may be considered. Partial adenoidectomy involves removing the upper part of the adenoid for relief of nasal obstruction while leaving the lower portion of the adenoid intact to ensure velopharyngeal competence. Fifty-eight children underwent partial adenoidectomy over a 4-year period; 55 of these children had preoperative nasal obstruction; 49 had a tonsillectomy done at the same time; and 2 had had a previous T&A. The rationale for retaining the lower portion of the adenoid includes a short soft palate, decreased palatal mobility, a mild submucous cleft palate, huge tonsils pushing the palate anteriorly, and a short hard palate. The relief of nasal obstruction was excellent. There were no primary postoperative hemorrhages, and none of the 58 children developed velopharyngeal insufficiency following partial adenoidectomy.

Adenoidectomy↗
PubMed1982

Unidirectional absorption of gentamicin from the peritoneum during continuous ambulatory peritoneal dialysis.

Gentamicin kinetics were determined after intravenous or intraperitoneal injection in five patients undergoing continuous ambulatory peritoneal dialysis (CAPD). Our objective was to determine rate of absorption of gentamicin from the peritoneum into the systemic circulation and vice versa. After intraperitoneal instillation of 1 mg/kg in the CAPD fluid during a 6-hr dwell time, the antibiotic appeared in the serum within 15 min in four of five patients. Peak serum concentrations ranged between 1.6 and 7.2 mg/l(mean +/- SD = 3.52 +/- 2.22) in all five patients and the time to reach peak concentration was 3.8 +/- 1.5 hr. Peritoneal gentamicin clearance was 13 ml/min. Percent extraction of gentamicin from the PD fluid within the 6 hr of intraperitoneal exposure ranged from 65% to 100% (mean +/- SD = 86.8 +/- 13.2). The fraction of the intraperitoneal dose absorbed into systemic circulation was found to be 0.84 independently by calculating the ratio of AUCip and AUCiv. When the same dose of gentamicin was injected intravenously (1 mg/kg), no gentamicin could be detected in the peritoneal fluid in three of five patients and only a very small amount of the drug was present for a brief period of time in the remaining two. The kinetic parameters of intravenous gentamicin were: volume of distribution, 0.3 l/kg; elimination rate constant, 0.028 hr(-1), plasma clearance 0.009 l/kg . min(-1), and half-life 27.4 hr. In two patients with acute peritonitis treated with intraperitoneal gentamicin, peak serum concentrations were found to range between 3.5 and 4.5 mg/l. These data suggest that gentamicin is rapidly absorbed from the peritoneal fluid into the blood compartment, but that occurrence of the reverse exchange is negligible. Thus, CAPD would not be expected to alter the elimination characteristics of intravenous gentamicin. Instillation of gentamicin in CAPD fluid may allow rapid absorption to reach therapeutic serum concentrations.

Adult↗
PubMed1982

HLA-A1 and steroid-induced cataracts in renal transplant patients.

Posterior subcapsular (PSC) lens changes were found in 32 of 33 steroid-treated renal transplant patients examined with the slit lamp techniques of direct and retroillumination. Only one of 33 age-matched controls had any PSC changes. Six (18%) of the renal transplant patients were bilaterally surgically aphakic because of PSC cataracts that developed while the patient was receiving steroid therapy after transplant surgery. Steroid-induced PSC cataracts were associated with the presence of human leukocyte antigen (HLA) A1, but not with the patient's age, cause of renal failure, total dose of steroids, or duration of steroid therapy.

Adolescent↗