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Biomedical subjects

R Jaffe

Publications and source records attributed to R Jaffe.

At least 55 records · Page 3Linked to original sources
PubMed1989

S100 protein positive dendritic cells in primary biliary cirrhosis and other chronic inflammatory liver diseases. Relevance to pathogenesis?

A study to determine the location of dendritic cells, in chronic inflammatory liver disease was performed. S100 protein positivity and dendritic cytoplasmic morphology were used to identify dendritic cells. S100 protein positive dendritic cells (S100 + DC) were found inside the basement membrane between biliary epithelial cells of septal bile ducts of livers affected by early stage PBC, but were not present at later stages. S100 + DC also were seen in areas of piecemeal necrosis in chronic active hepatitis of various etiologies. In contrast, intra-epithelial S100 + DC were not found with any consistency in sclerosing cholangitis, secondary biliary cirrhosis, extrahepatic biliary atresia, or chronic liver allograft rejection, all of which are characterized by inflammatory bile duct damage. The possible relevance of DC in the pathogenesis of PBC is discussed.

Dendritic Cells↗
PubMed1988

Occurrence of cytomegalovirus hepatitis in liver transplant patients.

The differential diagnosis of liver dysfunction after orthotopic liver transplantation can be difficult. Cytomegalovirus (CMV) hepatitis is one possibility. This report reviews our experience with 17 cases of pathologically proven CMV hepatitis following liver transplantation and demonstrates the need for percutaneous liver biopsies to establish the diagnosis. There were seven pediatric patients (ages 2-11 years, five males, two females) and ten adult patients (ages 17-53 years, eight males, two females). The most common symptoms were prolonged fever (15 patients, with a mean duration of 22 +/- 5.5 days), elevation in total bilirubin (14 patients), and elevation in liver enzymes (15 patients); all symptoms were also found in rejection. Leukopenia and thrombocytopenia, reported to frequently occur with CMV infection, were found in only three and five patients, respectively. Twelve patients with the above symptoms underwent percutaneous biopsy on one or more occasions to differentiate CMV hepatitis from rejection. The diagnosis was made at retransplantation in five patients. CMV hepatitis followed treatment for acute rejection in 14 patients and occurred without additional immunosuppression in three patients. All patients were maintained on cyclosporine and prednisone. Acute rejection episodes were treated with a 5-day tapering dose of steroids (17 courses in 12 patients), OKT3 monoclonal antibody [Ortho (4 patients)] antithymocyte globulin [Upjohn (2 patients)], and azathioprine (1 patient). CMV was isolated from urine (nine patients), blood (nine patients), throat (seven patients), lungs (two patients), and other organs (two patients). CMV was cultured from the liver biopsy specimens in five of the seven attempts in pediatric patients. When the diagnosis was confirmed in the absence of rejection, immunosuppression was routinely lowered. When rejection occurred concomitantly with CMV hepatitis, therapy had to be individualized. Retrospectively, three patients treated for rejection were noted at retransplantation to have only CMV hepatitis, and all three patients died. A high index of suspicion and the judicious use of liver biopsies is essential in order to differentiate CMV hepatitis from other causes of posttransplant liver dysfunction.

Adolescent↗
PubMed1988

Rhabdomyosarcoma in Roberts syndrome.

A 23-month-old child diagnosed as having Roberts syndrome, born to consanguineous parents, developed a sarcoma botryoides. Cytogenetic evaluation of peripheral blood lymphocytes and tumor cells showed premature centromere separation, which is characteristic of Roberts syndrome.

Abnormalities, Multiple↗
PubMed1988

Comparison between lateral and axial ultrasonic measurements of the fetal femur.

The necessity of adequate determination of fetal age is well recognized, and several different parameters have been used for this purpose. Lately fetal femur length has been accepted as a relatively accurate determination. It is customary to measure the femur in the lateral plane; however, because of positional changes the femur sometimes must be measured vertically. The aim of our study was to perform lateral and axial measurements of the femur at various gestational ages and to examine the relationship between them. Our results reveal a significant difference between both measurements in all age groups.

Femur↗
PubMed1988

The frequency of Epstein-Barr virus infection and associated lymphoproliferative syndrome after transplantation and its manifestations in children.

Twenty cases of Epstein-Barr virus (EBV)-associated lymphoproliferative syndrome (LPS), defined by the presence of EBV nuclear antigen and/or EBV DNA in tissues, were diagnosed in 1467 transplant recipients in Pittsburgh from 1981-1985. The frequency of occurrence in pediatric transplant recipients was 4% (10/253), while in adults it was 0.8% (10/1214) (P less than .0005). The frequency of LPS in adults declined after 1983 coincidental with the introduction of cyclosporine monitoring. However there was no apparent decline of LPS in children. We describe these ten pediatric cases and one additional case of LPS in a child who received her transplant before 1981. The frequency of EBV infection in 92 pediatric liver recipients was 63%. Of these subjects, 49% were seronegative and 77% of those acquired primary infection. Of 11 cases of pediatric EBV-associated LPS, 10 were in children who had primary infection shortly before or after transplantation. These results reinforce the importance of primary EBV infection in producing LPS, which was previously shown in adults. Children are at greater risk because they are more likely to be seronegative for EBV and to acquire primary infection. Three clinical types of LPS were recognized in children. The first (5 cases) was a self-limited mononucleosislike syndrome. The second syndrome (4 cases) began similarly, but then progressed over the next two months to widespread lymphoproliferation in internal organs and death. The third type (2 cases) was an extranodal intestinal monoclonal B cell lymphoma, occurring late after primary infection.

Adolescent↗
PubMed1988

Diagnosis and intrauterine management of enlargement of the cerebral ventricles.

Enlargement of the cerebral ventricles can be caused by one of two main mechanisms: increase in pressure within the brain because of obstruction to the flow of fluid through and out of the ventricular system or primary loss of brain substance with normal or low intraventricular pressure. Accurate diagnosis is particularly important. In late pregnancy a larger than expected biparietal diameter as measured by ultrasound renders the diagnosis relatively easy. In earlier pregnancy one has to measure the ventricule/hemisphere (V/H) ratio. According to Campbell, a ratio greater than 0.5 after 18 weeks is a sufficient criterian. After 20 weeks the normal V/H ratio should be equal to or less than 0.33. It is vital to perform serial examinations particularly in high-risk patients because early absence of ventriculomegaly does not always preclude its later development. The natural history of such an enlargement in infants or adults is usually a progressive loss of brain function and mental deterioration. In fetuses the prognosis is much less well defined. Only about 50% do not have associated problems. Etiology is very often unprecise which makes prediction of the outcome very difficult. Regarding therapy, three options are available according to the phase of pregnancy when the diagnosis is made, to the extent of the disease and naturally according to the availability of modes of therapy: termination of pregnancy, withholding of therapy or active intervention in form of fetal surgery with percutaneous intrauterine placement of a ventricular shunt. Fetal surgery is a very new alternative with very precise indications and advantages but limited justifications at the moment.(ABSTRACT TRUNCATED AT 250 WORDS)

Cerebral Ventricles↗
PubMed1988

Induction of ovulation with D-Trp6-LHRH combined with purified FSH in patients with polycystic ovarian disease.

Seventeen patients with polycystic ovarian disease (PCOD) and evidence of mild or severe ovarian hyperstimulation syndrome (OHSS) during therapy with CC/hCG, FSH/hCG or hMG/hCG were treated with D-Trp6-LHRH until medical gonadectomy was attained. Under the suppressive therapy with the GnRH agonist (GnRHa) ovulation was induced with FSH/hCG. In 15 out of 17 patients, ovulatory cycles were obtained with this new modality of treatment. Seven patients conceived (3 viable pregnancies and 4 early abortions) after the 1st treatment cycle. Fourteen of the 17 patients demonstrated symptoms of mild OHSS which did not require hospitalization. Only 1 patient developed severe OHSS after the combined treatment. Our results suggest that therapy with GnRHa, especially in its delayed release formulation, is effective for the prevention of severe ovarian hyperstimulation in PCOD patients undergoing treatment with menotropins for the induction of ovulation.

Adult↗
PubMed1988

Acute humoral rejection of an ABO-unmatched liver allograft in a pediatric recipient.

Acute humorally mediated rejection of organ allografts has long been recognized in kidney transplantation as the syndrome of hyperacute rejection. This reaction precludes renal transplantation across the major blood-group system and in many other instances in which preformed antibodies are present in the host. There is increasing evidence of the importance of humoral rejection in graft survival following liver transplantation. Although the antibodies responsible for this process have seldom been definitively identified, it is clear that the antibodies of the major blood-group system are of particular importance. Humoral rejection of a transplanted liver has not been well documented previously in a pediatric recipient. We present a case illustrating acute hepatic graft failure resulting from circulating anti-B IgM isohemagglutinins following an ABO-unmatched liver transplantation.

ABO Blood-Group System↗
PubMed1988

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↗
PubMed1988

Sonographic monitoring of ovarian volume during LHRH analogue therapy in women with polycystic ovarian syndrome.

Polycystic ovarian disease is characterized by menstrual disorders, infertility, obesity, and large ovaries. Large ovaries with multiple cysts are the direct cause of the high incidence of ovarian hyperstimulation during ovulation induction. Lately, gonadotropin-releasing hormone (GnRH) analogues have been employed to decrease ovarian steroidogenesis and thus reduce the incidence of ovarian hyperstimulation. In this study the ovarian size was ultrasonographically assessed during chronic GnRH analogue treatment, revealing a significant reduction in ovarian volume. This decrease in volume results in a reduced incidence of hyperstimulation, and we think the ultrasonic scanning can be effectively used to assess the success of GnRH treatment.

Female↗
PubMed1988

The pathology of posttransplant lymphoproliferative disorders occurring in the setting of cyclosporine A-prednisone immunosuppression.

Posttransplant lymphoproliferative disorders (PTLDs) were diagnosed in 43 patients from the Pittsburgh-Denver series between June 1980 and March 1987. This constitutes a detection rate of 1.7%. Major categories of clinical presentation included a mononucleosislike syndrome, gastrointestinal/abdominal disease, and solid organ disease. The median time of onset in patients initially immunosuppressed with cyclosporine-A (CsA)-containing regimens was 4.4 months after transplant, regardless of tumor clonality. A strong association of PTLD with Epstein-Barr virus (EBV) was observed. A histologic spectrum of lesions from polymorphic to monomorphic was observed. Whereas polymorphic lesions could be either clonal or nonclonal, monomorphic lesions appeared to be clonal in composition. The presence of large atypical cells (atypical immunoblasts) or necrosis did not appreciably worsen the prognosis. Twelve patients had clonal, 13 had nonclonal, and five had both clonal and nonclonal tumors. Clonality was indeterminate in 13 cases. Most patients were treated with a regimen based on reduced immunosuppression and supportive surgery. Almost all nonclonal and about half of the clonal lesions respond to this conservative therapy, indicating that it is an appropriate first line of treatment. This behavior suggests that a spectrum of lesions ranging from infectious mononucleosis to malignant lymphoma constitutes the entity known as PTLD. Some monoclonal tumors can undergo regression, however, apparently in response to host immune control mechanisms. Because of its short latency and strong association with EBV, PTLD is an important model for the study of virus-associated tumor progression in humans.

Adolescent↗
PubMed1988

Antibody-mediated rejection of human orthotopic liver allografts. A study of liver transplantation across ABO blood group barriers.

A clinicopathologic analysis of liver transplantation across major ABO blood group barriers was carried out 1) to determine if antibody-mediated (humoral) rejection was a cause of graft failure and if humoral rejection can be identified, 2) to propose criteria for establishing the diagnosis, and 3) to describe the clinical and pathological features of humoral rejection. A total of 51 (24 primary) ABO-incompatible (ABO-I) liver grafts were transplanted into 49 recipients. There was a 46% graft failure rate during the first 30 days for primary ABO-I grafts compared with an 11% graft failure rate for primary ABO compatible (ABO-C), crossmatch negative, age, sex and priority-matched control patients (P less than 0.02). A similarly high early graft failure rate (60%) was seen for nonprimary ABO-I grafts during the first 30 days. Clinically, the patients experienced a relentless rise in serum transaminases, hepatic failure, and coagulopathy during the first weeks after transplant. Pathologic examination of ABO-I grafts that failed early demonstrated widespread areas of geographic hemorrhagic necrosis with diffuse intraorgan coagulation. Prominent arterial deposition of antibody and complement components was demonstrated by immunoflourescent staining. Elution studies confirmed the presence of tissue-bound, donor-specific isoagglutinins within the grafts. No such deposition was seen in control cases. These studies confirm that antibody mediated rejection of the liver occurs and allows for the development of criteria for establishing the diagnosis.

ABO Blood-Group System↗
PubMed1988

Huge mesenteric mucinous cystadenoma in normal pregnancy.

A 36-year-old woman required laparotomy early in the puerperium after a normal delivery because of the presence of a large abdominal cyst. At laparotomy, a large mucinous cystadenoma was found attached to the root of the ileal mesentery; the ovaries were normal. This is the third case of mesenteric cyst published in association with pregnancy, but the first with a mucinous pattern. Pathologic aspects, possible complications, and treatment are discussed.

Adult↗