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Biomedical subjects

A Joseph

Publications and source records attributed to A Joseph.

At least 145 records · Page 8Linked to original sources
PubMed1994

3-Methylglutaconic aciduria in the Iraqi-Jewish 'optic atrophy plus' (Costeff) syndrome.

Eleven new patients of Iraqi-Jewish origin with bilateral optic atrophy, neurological abnormalities ('optic atrophy plus' syndrome) and 3-methylglutaconic aciduria (type III) are described. Clinical abnormalities in decreasing order of frequency were bilateral optic atrophy, extrapyramidal signs, spasticity, ataxia, dysarthria and cognitive deficit. An association with age was found only for spasticity. Spasticity, extrapyramidal signs and optic atrophy frequently led to major disability, in contrast to ataxia, dysarthria and cognitive deficit. The combined excretion of 3-methylglutaconic and 3-methylglutaric acid ranged between 9 and 187 mmol/mol creatinine. The primary enzymatic defect possibly may reside in the mitochondrial respiratory chain.

Adolescent↗
PubMed1994

Ten-year course of schizophrenia--the Madras longitudinal study.

Ninety first-episode patients fulfilling ICD-9 criteria for schizophrenia were followed up prospectively for 10 years. Complete assessments were possible on 76. The pattern of illness was good in 67% of the cases, and the commonest patterns was one with recurrent episodes. Predictors of poor course and longer time spent in psychosis were identified. All positive and negative symptoms showed a steep decline at the end of 10 years. The results are discussed in the context of longitudinal research on the course of schizophrenia in developing countries.

Adult↗
PubMed1994

Spontaneous improvement in a patient with the hepatopulmonary syndrome assessed by serial exercise tests.

A 37 year old patient with chronic active hepatitis progressing to cirrhosis presented with increasing breathlessness and was found to be hypoxic with finger clubbing. A progressive exercise study with measurement of oxygen saturation (SaO2) showed abnormally high ventilation and desaturation to 81% at 100 W. Serial studies over nearly two years showed, first, deterioration, then improvement with lower ventilation and higher saturation levels at all work loads. This could not be correlated with any change in treatment with azathioprine, prednisolone, or propranolol.

Adult↗
PubMed1994

Delusions of pregnancy.

We report five cases of delusions of pregnancy of whom three were women and four had chronic schizophrenia. We draw the attention of clinicians to this symptom which is not as rare as has previously been thought.

Adult↗
PubMed1994

Splenic reimplantation does not affect outcome in chronic canine model.

The effects of septic insult were compared in a canine model of splenic reimplantation. Sequential changes in hematologic, hepatic, and immunologic function were monitored biweekly in 18 dogs during 10 months after splenectomy, splenectomy with reimplantation, or sham operation. There was no significant difference in these measures between the two groups. At the end of the 10-month period, spleen scans with technetium (99Tc) labeled, heat-damaged RBCs were obtained on the reimplanted dogs. 99Tc scanning revealed no active splenic implants at 10 months. All dogs were then infected with intravenous Type III pneumococcus for 9 consecutive days. There were no measurable hematologic, hepatic, or immunologic differences between groups before or after the septic insult. These animals were then sacrificed for histologic analysis of the splenic reimplants. Reimplant histology showed active germinal centers, but the surrounding pulp was fibrotic and lymphocyte-depleted. Splenic reimplantation in this canine model yields no apparent benefit.

Analysis of Variance↗
PubMed1993

Identification of a cDNA for a human high-molecular-weight B-cell growth factor.

Proliferation is necessary for many of the phenotypic changes that occur during B-cell maturation. Further differentiation of mature B cells into plasma cells or memory B cells requires additional rounds of proliferation. In this manuscript, we describe a cDNA for a human B-cell growth factor we call high-molecular-weight B-cell growth factor (HMW-BCGF). Purified HMW-BCGF has been shown to induce B-cell proliferation, inhibit immunoglobulin secretion, and selectively expand certain B-cell subpopulations. Studies using antibodies to HMW-BCGF and its receptor have suggested that HMW-BCGF, while produced by T cells and some malignant B cells, acts predominantly on normal and malignant B cells. The HMW-BCGF cDNA was identified by expression cloning using a monoclonal antibody and polyclonal antisera to HMW-BCGF. Protein produced from the cDNA induced B-cell proliferation, inhibited immunoglobulin secretion, and was recognized in immunoblots by anti-HMW-BCGF antibodies. The amino acid sequence of HMW-BCGF deduced from the cDNA predicts a secreted protein of 53 kDa with three potential N-linked glycosylation sites. The identification of this cDNA will allow further studies examining physiologic roles of this cytokine. We propose to call it interleukin 14.

Amino Acid Sequence↗
PubMed1993

Magnetic stimulation of the sacral roots.

Magnetic stimulation using an external surface coil induces an electrodynamic field that penetrates various tissues and stimulates peripheral nerves in a similar fashion to conventional electrical stimulation. An 83 mm magnetic surface coil was used to stimulate 11 spinal cord injury (SCI) patients, during which time detrusor activity and evoked potentials of the striated urinary sphincter motor pathways were evaluated. All patients had urodynamic studies and conventional sacral evoked potentials prior to magnetic stimulation. The mean bladder capacity was 337 ml (range 109-590), mean leak point pressure was 50 cm H2O (range 10-80), and mean sacral reflex (afferent-efferent) latency was 37.9 ms (range 25.1-49.3). Eight patients had detrusor-sphincter dyssynergia. Magnetic stimulation over the sacral spine at different bladder volumes was performed. Detrusor and striated sphincter responses were recorded during stimulation. In all patients the technique was easy and the results were reproducible. The mean sacral motor pathway (efferent) latency was 27.9 ms (range 18.7-39.6). Using maximal stimulation, no detrusor response was recorded at bladder volumes < 200 ml. However, a detrusor response was recorded in 7 patients (> 10 cm H2O in 2, < 10 cm H2O in 5) when the bladder volume was > 200 ml. No complications were seen. Sacral evoked potential measurements assess the function and integrity of the sacral arc but it does not distinguish between afferent and efferent pathways. Magnetic stimulation is a safe and effective method to assess the integrity and function of the detrusor and striated sphincter motor (efferent) pathways. When combined with sacral evoked potential studies, the sensory (afferent) pathways can be evaluated indirectly.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1993

Cloning and characterization of the human t(3;6)(p14;p11) translocation breakpoint associated with hematologic malignancies.

The t(3;6)(p14;p11) chromosome translocation was identified in a family in which three members developed hematologic malignancies. To help characterize the region on chromosome 3 surrounding this translocation breakpoint, two flanking lambda clones, MS156 and MJ1525, were linked by pulsed-field gel electrophoresis to the same 510-kb NotI fragment on chromosome 3. MS156 was localized to a region proximal to the breakpoint of a der(3) chromosome somatic cell hybrid (derived from the t(3;6) cell line), and MJ1525 localized distal to the breakpoint. MJ1525 was used to screen the CEPH yeast artificial chromosome (YAC) library, which revealed a YAC, 195F3, that spanned the breakpoint. Subcloning into Lambda DASH II and production of a contiguous array of overlapping lambda clones revealed a clone, L17, that spanned the breakpoint. A rare restriction endonuclease map for the YAC 195F3 was constructed, and multiple clusters of rare restriction sites within the YAC were identified, possibly indicating the disruption of a gene by the t(3;6) translocation breakpoint.

Animals↗
PubMed1993

Manifestations of coronary atherosclerosis in young trauma victims--an autopsy study.

OBJECTIVES: The aim of this study was to look at the prevalence of coronary atherosclerosis, its severity and site of involvement in patients < 35 years old who died from noncardiac trauma. BACKGROUND: Autopsies performed on casualties of the Korean War revealed coronary artery involvement in 77.3% of the hearts studied, and data after the Vietnam War noted the presence of atherosclerosis in 45% of casualties with severe disease in 5%, suggesting a decline in the prevalence of coronary atherosclerosis in young men. METHODS: One hundred eleven victims of noncardiac trauma (86.4% white with a mean age of 26 +/- 6 years) underwent pathologic examination of their coronary arteries to estimate the presence and severity of coronary atherosclerosis grossly, microscopically and through computerized planimetry. Identified segments of the coronary arteries were sectioned at 3-mm intervals, stained with special stains and after microscopic examination transferred to videotape and digitized to allow estimation of the percent compromise in the lumen area by atherosclerotic plaque. RESULTS: Signs of coronary atherosclerosis were seen in 78.3% of the total study group, with > 50% narrowing in 20.7% and > 75% narrowing in 9%. No demographic or anatomic features separated the groups with less or more severe involvement of their coronary arteries. Proximal involvement was more common except in the right coronary artery, which was as frequently involved distally. CONCLUSIONS: The overall prevalence of coronary atherosclerosis in a young, predominantly male study group was comparable with that noted after the Korean War. Left main or significant two- and three-vessel involvement was noted in 20% of the group studied and emphasizes the need for aggressive risk factor modification in this group.

Adolescent↗
PubMed1993

Recurrent metabolic decompensation in profound carnitine palmitoyltransferase II deficiency.

A 3-year-old boy had recurrent episodes of lethargy, encephalopathy, and hepatomegaly accompanied by hypoglycemia, elevated liver aminotransferase and creatine kinase values, and nonketotic dicarboxylic aciduria; the serum carnitine level was moderately reduced. Carnitine palmitoyltransferase II activity was decreased in lymphocytes and fibroblasts. Therapy with L-carnitine and a diet low in long-chain triglycerides did not prevent recurrent episodes.

Carnitine O-Palmitoyltransferase↗
PubMed1993

Maternal age and birth order of young IDDM patients. A study from southern India.

OBJECTIVE: To assess the effects of maternal age and birth order on the risk of IDDM in Asian Indians. RESEARCH DESIGN AND METHODS: Data from a diabetes registry in Madras, India, was used to find IDDM patients diagnosed before 20 yr of age. A questionnaire was used to elicit clinical details of subjects. RESULTS: Children with mothers < or = 24 yr of age had a higher percentage of IDDM. Children with lower birth orders had a higher prevalence of IDDM. CONCLUSIONS: The risk of IDDM in children is increased in children with lower birth orders, born of younger mothers.

Adolescent↗
PubMed1993

Rat placental hepatocyte growth factor/scatter factor: purification, characterization, and developmental regulation.

In view of significant species-specific sequence differences between human and rat placental hepatocyte growth factor (HGF)/scatter factor (SF), the rat placental HGF/SF (rpSF) was purified, and its properties compared with human placental HGF/SF (hpSF). Like hpSF, rpSF scattered Madin-Darby canine kidney cells at 1-2 ng/ml and is composed of two subunits of 60 kDa and 30 kDa. Higher amounts (> 50%) of uncleaved 90-kDa form was present in the HGF/SF preparations from both human and rat placentas. Rat placental SF reacts with antibodies raised against hpSF in rabbits and chickens. The SF activity when expressed per gram rat placental tissue rises rapidly up to 9 days and then levels off. When expressed per milligram tissue protein it also increases rapidly up to 9 days and then declines. The expression of HGF/SF mRNA during development parallels that of HGF/SF activity. The specific activity of HGF/SF receptor (c-met) mRNA also appears to peak at 6 days. These findings suggest that (i) in spite of significant (> 10%) sequence differences between rpSF and hpSF, they exhibit similar structural, biologic, and immunologic characteristics and (ii) HGF/SF and its receptor are expressed in high amounts on Day 6 and then decline in developing placenta.

Animals↗
PubMed1993

HGF-SF: effects on motility and morphology of normal and tumor cells.

HGF-SF is a recently discovered cytokine which has both mitogenic and motogenic effects on a wide variety of cells. We used a computerized digital imaging system to measure motility and morphology of isolated cells. In this chapter we will describe the effect of HGF-SF and of other growth factors on the velocity, area, circularity, and flatness of normal and tumor cells. We will then discuss the possible mechanism of HGF-SF induced motility and the possible role of this factor in biological and pathological processes.

Animals↗
PubMed1992

Scatter factor is a glycoprotein but glycosylation is not required for its activity.

Scatter factor (SF) is a protein produced by fibroblasts, smooth muscle cells, and human placenta which scatter cohesive epithelial cell colonies and increases cellular motility. SF bound to concanavalin A and other lectins with high affinity. SF could also be stained with a glycoprotein specific stain. Incubation of producer cells (N-ras-transformed 3T3), with tunicamycin homolog A1 did not have any significant effect on the secretory activity of SF. The treatment of SF with N- and O-glycanases as well as endoglycosidase H had no effect on its activity. However, treatment of target (Madin Darby canine kidney) cells with tunicamycin A1, abolished the scattering response. These studies suggest that scatter factor is a glycoprotein, but glycosylation is not required for its secretion or activity by the producer cells; however, glycosylation of proteins in the target cells is required for SF action.

3T3 Cells↗
PubMed1992

Binding of scatter factor to epithelial cell membrane protein: identification of its receptor.

Binding of scatter factor (SF) to the surface protein of Madin-Darby canine kidney (MDCK) cells was investigated. The factor has a specific affinity for membrane proteins of MDCK cells and could be purified 10-20-fold using a membrane protein-affinity chromatographic procedure. The binding was pH- and salt-dependent. The factor did not bind to columns prepared with membrane proteins from non responder cells or with bovine serum albumin. Further purification to homogeneity was achieved using reverse phase and immunoaffinity chromatography. The factor dissociated into 92, 62 and 34/32 kDa bands on SDS-PAGE under reducing conditions. A 230 kDa protein band, the receptor-SF complex, was observed when radiolabeled SF was crosslinked to surface proteins of MDCK cells and the complexes were subjected to electrophoresis. The binding of radiolabeled SF to the MDCK cells was decreased in presence of excess unlabeled SF. These observations suggest that the binding of SF to surface proteins of MDCK cells is specific and occurs predominantly to a 150 kDa protein.

Animals↗