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Biomedical subjects

R Jaffe

Publications and source records attributed to R Jaffe.

At least 163 records · Page 9Linked to original sources

Lectin histochemistry and ultrastructure of kidneys from patients with I-cell disease.

The undegraded stored material in I-cell disease (ICD) includes mucopolysaccharides, lipids, and oligosaccharides. We used ten different lectins as histochemical probes to identify specific carbohydrate residues in stored materials, and electron microscopy to examine the morphology of abnormally stored material in kidneys from two patients with ICD. In both cases, all the glomerular epithelial cells (ie, podocytes), endothelial cells, and renal tubular cells were enlarged and vacuolated. Ultrastructural studies revealed both fibrillogranular material and lamellated membrane structures in the vacuoles. The cytoplasm of affected cells stained with Concanavalia ensiformis agglutinin, wheat-germ agglutinin, and succinylated-wheat-germ agglutinin, but no corresponding control cells stained with lectins. The latter findings indicate an accumulation of N-linked oligosaccharides containing alpha-mannosyl, beta-N-acetylglucosaminyl, and sialyl residues in renal cells affected by ICD.

Child↗

Pathology of Langerhans cell histiocytosis.

The spectrum of the histopathology of Langerhans cell histiocytosis was presented with special attention paid to the pathologic Langerhans cell as the diagnostic lesional cell. Features that distinguish this histiocyte from others are defined by electron microscopic demonstration of Birbeck granules and by immunohistochemical characteristics such as possession of antigenic determinant(s) that react with the monoclonal antibody T6. The relationship between Langerhans cells and the other histiocytes from which they are derived seems to be determined by events initiated by surface antigens reacting with specific antibodies. Immunohistochemical tests that are of value in diagnosing and classifying the various histiocytosis syndromes include those used to identify the S-100 antigen, T6 antigenic determinant(s), and peanut agglutinin binding sites. The use of a standard nomenclature and diagnostic criteria for the histiocytosis syndromes is encouraged as a step forward in progress to better understand and communicate about these puzzling disorders.

Cell Differentiation↗

Pregnancy with an artificial pacemaker.

Complete heart block in pregnancy is not a common encounter. The first case was reported in 1914 by Nanta and today some 100 cases are documented. Heart block may be congenital or acquired secondary to cardiac surgery, rheumatic heart disease, or infective disorders. Heart block, whether congenital or acquired, rarely creates any special obstetric problems. Today there is an increasing use of cardiac pacemakers in younger people and the first reported obstetric experience with a cardiac pacemaker implanted before pregnancy was by Shouse and Acker. This review will document the course and outcome of all reported pregnancies in women conceiving with an artificial pacemaker, and discuss complications and principles of management. We will also report our experience with a woman suffering from a complete heart block in whom an internal cardiac pacemaker was inserted before pregnancy.

Adult↗

Neurofibromatosis and childhood tumors.

Although tumors are a known complication of von Recklinghausen's neurofibromatosis (NF), they often develop after the second decade and their characteristics in the pediatric population are unclear. To define the frequency and distribution of tumor types in children with NF, the authors reviewed the experience at Children's Hospital of Pittsburgh between 1953 and 1984. During that time, 121 children younger than 18 years with documented NF were seen. Three patients (2.5%) developed locally extensive, unresectable sarcomas, two of which were clearly neural in origin. Two other children died from complications of highly cellular but not histologically malignant neurofibromas. Seventeen patients (14%) had brain tumors, of which 3 (2.5%) were malignant astrocytomas and 9 were optic gliomas. Two children had acute myelogenous leukemia. Although it has been said that patients with NF are particularly susceptible to the oncogenic effects of radiation, 12 children were treated with a median of 5000 rad to their tumor bed. Follow-up after radiation in that small cohort of patients ranged from 6 months to 12 years (mean, 6 years). Eleven of 12 patients experienced adequate local control. During the follow-up period, none developed a second tumor outside or within the radiation port that could be related to radiation. The authors conclude that clinically significant tumors in children with NF, not all of which are histologically malignant, are not rare. Although more prospective information is needed on response to and sequelae from therapy, the data in this report suggest no contraindication to aggressive therapy.

Adolescent↗

Lingual lipase: immunocytochemical localization in the rat von Ebner gland.

Rat lingual lipase was purified to homogeneity by solubilization in 1% octylglucoside, followed by centrifugation, affinity chromatography on hydroxylapatite, gel filtration, and chromatofocusing. Polyclonal antibodies to the enzyme were raised in rabbits, and monospecificity was established by immunoblotting. Tissues were embedded in either paraffin or Epon for light and transmission electron microscopy, respectively. Immunocytochemical localization of the enzyme was established using the peroxidase-antiperoxidase and streptavidin-gold techniques. Immunospecific staining was observed in all acinar cells of the serous von Ebner glands, but only in the demilunes of the lingual mucous glands. The secretory granules of the acinar cells of the von Ebner glands were stained with antilipase on electron microscopic examination. No staining was observed in the salivary glands, pancreas, esophagus, stomach, or duodenum. If a true gastric lipase exists in the rat, it is immunogenically distinct from lingual lipase.

Animals↗

Resolution of thyroid-induced schizophreniform disorder following subtotal thyroidectomy: case report.

Psychoses due to thyrotoxicosis are typically affective in nature. We report on a schizophreniform psychosis in a woman with Graves' disease whose symptoms abated following subtotal thyroidectomy. Surgical thyroidectomy should be considered in thyrotoxic patients who are unresponsive to medical therapy or who manifest unremitting or life-threatening psychotic behavior, especially if long-term compliance required for medical therapy appears unlikely.

Adult↗

Myotonic dystrophy and pregnancy: a review.

The previous reports of 20 women with myotonic dystrophy and pregnancy are reviewed, and a new case report added. Myotonic dystrophy is often unrecognized in early life due to vague symptoms, and is often first diagnosed during pregnancy which is known to worsen the main features of muscular weakness and myotonia. Complications of pregnancy and delivery are frequent and serious and should be known to all those dealing with pregnant women. A history of fetal wastage or a child suffering from symptoms of congenital myotonic dystrophy may be a clue to the diagnosis and these patients should be closely followed until safe delivery. The neonatologist should be involved as feeding and respiratory problems in the neonate may indicate that the infant is affected.

Abortion, Spontaneous↗

Single- versus multiple-dose mezlocillin prophylaxis in emergency cesarean section.

163 patients undergoing emergency cesarean section were prospectively and randomized evaluated to determine the effect of mezlocillin in reducing postoperative morbidity. We compared a single 5-gram preoperative dose and a perioperative 3-dose regimen, each of 2 g, with a placebo. Postoperative morbidity was reduced from 65% in the placebo group to 20% in the groups receiving mezlocillin (p less than 0.005). The incidences of febrile morbidity, endometritis and urinary tract infection were all significantly lower in both groups given mezlocillin. There was no difference in the reduction of morbidity between the two groups receiving mezlocillin. The main advantages of the prophylaxis included a shorter hospitalization and the absence of serious infections in the treated groups.

Adult↗

Fetal death in early pregnancy due to electric current.

Electric shock injuries in pregnant women are rare events. The uterus and amniotic fluid are thought to be excellent conductors of electric current which reaches the fetus causing cardiac arrest and fetal death. All cases except one reported in the literature occurred in the third trimester, and ended with the death of the fetus. We report a case of a woman struck by a high voltage electric current in the 13th week of her pregnancy, causing fetal death and abortion.

Abortion, Spontaneous↗

Problems of long-term informed consent.

This study addresses problems arising with informed consent for long-term maintenance pharmacotherapy. Obtaining patient consent to neuroleptic treatment, with the risk of tardive dyskinesia, has raised questions about long-term recall and the competence of psychiatric patients as a special population. The subjects were 32 adult outpatients, 16 were followed in the psychiatric clinic and 16 in the rheumatology, pulmonary, and neurology clinics. Structured interviews with these patients dealt with knowledge about relevant short-term and long-term medication side effects. Interview results were used to compare psychiatric and medical groups with respect to overall levels of comprehension. Two results were striking. 1. There was a remarkable similarity in the degree of comprehension between psychiatric and medical outpatient groups; this suggests that psychiatric patients need not be considered any less competent than medical outpatients in assimilating necessary medication information. 2. Patients in both groups were knowledgeable about short-term side effects, usually as a consequence of personal experience with them. However, their knowledge was consistently inadequate with regard to potential long-term side effects from their maintenance medication. Current informed consent doctrine may presume a degree of recall and comprehension beyond the capabilities of most patients. The development of an appropriate doctor-patient relationship that reconciles the need for consent with patient limitations remains an important challenge for clinicians.

Adult↗

Progression of neurovisceral storage disease with supranuclear ophthalmoplegia following orthotopic liver transplantation.

A 7-year-old girl with progressive ataxia, spasticity, supranuclear ophthalmoplegia, and sea-blue histiocytes in her bone marrow underwent orthotopic liver transplantation for hepatocellular carcinoma. After an initial period of stabilization, she has shown progression of neurologic symptoms with recurrence of storage material in the transplanted liver.

Carcinoma, Hepatocellular↗

Sports medicine emergencies.

With the high interest in fitness and sporting activities at present, all primary care physicians must be prepared to treat emergencies in sports medicine. Basic knowledge, reference material, equipment, and supplies are mandatory (see Tables 1 and 2). Unique situations may require additional items.

Altitude↗

Recurrent hepatitis B in liver allograft recipients. Differentiation between viral hepatitis B and rejection.

The histologic findings in the original liver obtained from 9 liver allograft patients with active B virus hepatitis were compared with 28 posttransplant pathology specimens. All specimens were studied with the use of light and immunohistochemical microscopy in conjunction with pertinent clinical data. Eight of the 9 patients had chronic active hepatitis B (HB) with cirrhosis, prior to transplant, one of which had coexistent hepatocellular carcinoma. The ninth patient had fulminant hepatic necrosis secondary to acute HB prior to transplantation. In all of the patients with chronic HB prior to transplantation who survived more than 2 months after transplantation recurrent infection of the graft developed despite perioperative HB immunoglobulin therapy. The patient with acute fulminant hepatitis B pretransplant has done well postoperatively and has evidence of HB virus immunity (positive anti-HBs) 15 months after transplantation. Examination of tissue specimens obtained during episodes of allograft dysfunction in these 9 patients indicate that pathologic alterations of active HB infection of the allograft are associated with a preferential lobular insult, whereas those occurring in rejection preferentially involve portal tract structures. Serologic data combined with biopsy histopathologic data are essential in distinguishing between the two quite different events.

Diagnosis, Differential↗

Ultrasonic real-time diagnosis of transitory fetal pleural and pericardial effusion.

Congenital fetal effusions are rare and often fatal. Prenatal ultrasonic diagnosis is an important factor in both diagnosis and management of this condition. We report a case with both pleural and pericardial effusion, the ultrasonic diagnosis and follow-up, and the birth of a healthy baby. A short review of the literature is given.

Adult↗