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Biomedical subjects

R Jaffe

Publications and source records attributed to R Jaffe.

At least 127 records · Page 7Linked to original sources

Otologic manifestations of Langerhans' cell histiocytosis.

Eighteen of 62 children diagnosed with Langerhans' cell histiocytosis at the Children's Hospital of Pittsburgh (Pa) between 1970 and 1986 demonstrated ear and temporal bone involvement. In six children, such otologic disease was their sole presenting manifestation. Common signs and symptoms included aural discharge, postauricular swelling, and conductive hearing loss. The otologic findings in these children, if not investigated properly, could easily be attributed to acute or chronic infectious ear disease. Computed tomography with contrast enhancement proved to be particularly valuable as a diagnostic study because of its clear delineation of both osseous and soft-tissue temporal bone involvement. Computed tomographic findings could also be used to enhance local treatment by guiding surgical biopsy and curettage procedures or defining low-dose radiation therapy portals. Eleven of these 18 children with otologic Langerhans' cell histiocytosis additionally required chemotherapy due to the systemic nature of their disease.

Child↗

Ultrasonographic measurement of fetal femur length in growth disturbances.

Ultrasonographic measurement of fetal femur length is a recognized technique for determination of gestational age and fetal growth. A total of 280 pregnant women were studied, each of whom had pathologies with the potential to either accelerate or delay fetal growth. There were 1000 measurements of the fetal femur length performed on these 280 fetuses. A total of 125 fetuses were found to have a growth disturbance-91 with asymmetrical intrauterine growth retardation and 34 with macrosomia. Comparison of fetuses with either intrauterine growth retardation or macrosomia with appropriate-for-gestational-age fetuses showed that the femur length is not statistically affected by intrauterine growth abnormalities.

Female↗

Control of prostaglandin induced uterine hyperactivity with intravenous ritodrine.

The prostaglandins, F2 alpha and E2, are in extensive local use for the induction of labor. The main concern with oral and vaginal administration is the difficulty in controlling rapidly progressing labor and uterine hyperactivity. Herein we present a case where intravenous ritodrine was given as soon as hyperactivity and fetal heart rate decelerations were detected. With this treatment, the hyperactivity was controlled, fetal heart rate returned to normal and labor progressed normally to the birth of a healthy infant.

Adult↗

Perforated adenocarcinoma of the colon during pregnancy.

Performated carcinoma of the colon in pregnant women is a rare event. Only six cases have been reported in the literature, all except one in the recto-sigmoid. We present a case of perforated adenocarcinoma of the colon located above the peritoneal reflection and discuss the problem of diagnosis and management.

Adenocarcinoma↗

Egg donation in an in vitro fertilization program: an alternative approach to cycle synchronization and timing of embryo transfer.

A new flexible protocol for the induction of recipient endometrial cycles is presented. For stimulation of endometrial growth, a fixed dose of conjugated estrogens, 3.75 mg/d was employed. The duration of the proliferative phase varied from 9 to 14 days, thus being adjusted to match the length of the follicular phase of the donor. Embryo transfer was performed on the fifth day of progesterone administration. Four term pregnancies resulted from 12 treatment cycles. In the conception cycles, the hormonal support was continued until the luteal placental shift occurred, regardless of gestational age.

Embryo Transfer↗

Lymphoma and hypercalcemia in a pediatric orthotopic liver transplant patient.

We present a case report of a pediatric orthotopic liver transplant recipient who developed lymphoma with hypercalcemia on cyclosporine and prednisone immunosuppression. This is the first reported posttransplant lymphoproliferative disorder complicated by hypercalcemia, with a finding of an elevated 1,25 dihydroxyl vitamin D state, suggesting that it has a role in the pathophysiology of this B cell lymphoma hypercalcemia. The clinical course and management of this disorder with a 31-month follow-up are described.

Calcitriol↗

Multivisceral intestinal transplantation: surgical pathology.

We report the diagnostic surgical pathology of two children who underwent multivisceral abdominal transplantation and survived for 1 month and 6 months. There is little relevant literature, and diagnostic criteria for the various clinical possibilities are not established; this is made more complicated by the simultaneous occurrence of more than one process. We based our interpretations on conventional histology, augmented with immunohistology, including HLA staining that distinguished graft from host cells in situ. In some instances functional analysis of T cells propagated from the same biopsies was available and was used to corroborate morphological interpretations. A wide spectrum of changes was encountered. Graft-versus-host disease, a prime concern before surgery, was not seen. Rejection was severe in 1 patient, not present in the other, and both had evidence of lymphoproliferative disease, which was related to Epstein-Barr virus. Bacterial translocation through the gut wall was also a feature in both children. This paper documents and illustrates the various diagnostic possibilities.

Antigens, Viral↗

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↗

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↗

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↗

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↗

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↗

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↗

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↗