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Biomedical subjects

J Sharp

Publications and source records attributed to J Sharp.

At least 19 recordsLinked to original sources
PubMed1997

Implementation of total quality management after reconfiguration of services on a general hospital unit.

In 1992 the New York State Office of Mental Health issued a statewide plan for mental health services to reduce the number of inpatient beds in state-run facilities from approximately 11,000 to between 6,000 and 8,000 by the year 2000. This reduction resulted in at least a 25 percent increase in psychiatric beds at local general hospitals. In 1992 Albany Medical Center Hospital's department of inpatient psychiatry established an interdisciplinary committee to address changes resulting from the reconfiguration of services to chronic mentally ill persons. The committee established procedures to use the principles of total quality management to respond to problems and to continuously improve the therapeutic milieu. The authors describe how these principles were used to create a patient satisfaction survey, to examine and improve part of the hospital admissions procedure, and to review and revise treatment planning documentation. A concurrent review committee reviews patients' records to ensure accuracy of documentation and quality of care.

Chronic Disease↗
PubMed1996

Multiple recurrent branch retinal artery occlusions associated with varicella zoster virus.

PURPOSE: The authors describe an immunocompetent patient who developed multiple recurrent branch retinal artery occlusions (BRAOs) associated with the varicella zoster virus (VZV). METHODS: A 69-year-old woman with mild bilateral vitritis developed superior and inferior BRAOs in her right eye with decreased visual acuity to 20/40, and a peripheral BRAO inferotemporally in her left eye. One month later, the inferotemporal BRAO progressed proximally in her left eye with a decrease of visual acuity to 20/40. After an extensive negative systemic evaluation, she underwent a diagnostic pars plana vitrectomy of her right eye. RESULTS: Vitreous fluid was positive for VZV DNA by polymerase chain reaction (PCR). The patient was treated with intravenous acyclovir and systemic oral steroids. After remaining disease free for 3 months, the patient had two recurrences: 1) a mild vitritis and 2) development of a new superior temporal artery occlusion in the left eye. Both recurrences were treated with oral acyclovir and systemic steroids. The patient remained recurrence free for 12 months on a maintenance dose of oral acyclovir, and for 4 additional months without acyclovir. CONCLUSIONS: Varicella zoster virus can be associated with the syndrome of multiple recurrent BRAOs. The diagnosis of VZV-associated BRAO can be established by PCR of intraocular fluid.

Acyclovir↗
PubMed1995

Linkage analysis of late-infantile neuronal ceroid-lipofuscinosis.

The neuronal ceroid-lipofuscinoses (NCL) are a group of neurodegenerative disorders with an autosomal-recessive pattern of inheritance. There are 3 main categories of childhood NCL, namely, infantile, late-infantile, and juvenile NCL. These can be distinguished on the basis of age of onset, clinical course, and histopathology. A number of variant forms of NCL have also been described, and these show symptoms intermediary between the main classical forms. The genes for both the infantile and juvenile forms of NCL have previously been mapped to chromosome areas 1p32 and 16p12, respectively. The gene for late-infantile NCL (LINCL), CLN2, has been excluded from both these loci, but its location is as yet unknown. Recently, CLN5, the gene for the Finnish variant form of LINCL, was mapped to 13q21.1-32. Using the 3 microsatellite markers which were most tightly linked to CLN5, we have excluded CLN2 from this region using a subset of 17 families. Thus, CLN2 represents a fourth distinct genetic locus involved in the pathogenesis of NCL.

Age of Onset↗
PubMed1995

Haemodialysis and ultrafiltration in babies weighing under 1000 g.

Until now it has not be feasible to haemodialyse babies with renal failure who weigh less than 1000 g. Three babies weighing 630, 808, and 1140 g are described. They had multiorgan failure and could not be peritoneally dialysed. They were treated with veno-venous ultrafiltration and haemodialysis using a manual syringe driven technique that required only simple equipment. This method is highly labour intensive, but can provide control of fluid volume and plasma biochemistry in these very sick infants. Their prognosis, however, is determined by the underlying cause of their renal failure, and remains poor.

Acute Kidney Injury↗
PubMed1994

Rapid and stable gene expression in hippocampal slice cultures from a defective HSV-1 vector.

Stable transfer of genetic information into neurons is a powerful strategy to elucidate specific mechanisms of neurophysiology and to develop therapies for neurological disorders. To evaluate the optimal parameters for efficient gene delivery of defective herpes simplex virus type one (HSV-1) vectors into a specific brain region, an HSV-1 vector expressing E. coli beta-galactosidase was used to infect organotypic cultures of hippocampal slices. beta-Galactosidase was expressed as early as 2 h after infection in a dose-dependent manner as measured on immunoblots, and reached a maximum level after approximately 35 h. Expression of the RNA and the antigen was still evident after the longest time sampled (11-12 days), whereas no beta-galactosidase was ever detected in cultured slices infected with a control virus lacking the reporter gene. Hippocampal cells expressing the reporter gene outlined the contour of the neuronal cell body layers in fields CA3 and dentate gyrus; such correspondence was less evident in field CA1. Anatomical, morphological, and immunohistochemical criteria also confirmed that the majority of these infected cells were neurons. beta-Galactosidase was also detected in the somata and processes of infected interneurons. Tests for synaptic pathology associated with virus infection showed no changes in pre- and postsynaptic markers.

Animals↗
PubMed1994

Defined chromosomal assignment of CLN5 demonstrates that at least four genetic loci are involved in the pathogenesis of human ceroid lipofuscinoses.

We demonstrate here that at least four genetically separate loci are involved in the pathogenesis of human neuronal ceroid lipofuscinoses (NCLs), fatal brain disorders of children. Earlier the assignments of the infantile and juvenile subtypes of NCL to 1p32 and 16p12 had revealed two loci; and here a variant subtype of the late-infantile form of NCL is mapped to a well-defined region on 13q21.1-q32, whereas the clinically similar, classical form of late-infantile NCL was found to represent the fourth, yet-unidentified NCL locus. The linkage disequilibrium was crucial for locus assignment in our highly limited family material, and the data exemplify the significance of this phenomenon in the random mapping of rare human diseases.

Child↗
PubMed1993

Non-cardiac benefits of human recombinant erythropoietin in end stage renal failure and anaemia.

Recombinant human erythropoietin (r-HuEpo) is now available to correct the anaemia of end stage renal failure. The clinical consequences of increasing the haemoglobin concentration in children on dialysis are incompletely documented; a placebo controlled study is essential when assessing subjective changes, for example in appetite or other aspects of quality of life. A single blind, placebo controlled crossover study in 11 children with end stage renal failure was performed to assess the clinical benefits resulting from correction of anaemia. Ten of the 11 children completed 36 weeks of the study and seven completed both 24 week limbs. Subcutaneous administration of r-HuEpo twice a week resulted in an increase in haemoglobin concentration, from 73 to 112 g/l. This was associated with an objective improvement in exercise tolerance, and a subjective improvement in physical performance and health, and better school attendance. No consistent effect was seen on appetite, growth, psychosocial functioning, biochemical control, or peritoneal dialysis efficiency. A small but clinically unimportant increase in systolic and diastolic blood pressure was seen in five children. One child on antihypertensive treatment required an increase in dosage during r-HuEpo while another child required a reduction in treatment. These findings, together with the important cardiac benefits previously described during r-HuEpo treatment, support the use of r-HuEpo in all children with end stage renal failure and anaemia.

Anemia↗
PubMed1993

Bioethics: social work's response and training needs.

Hospital social workers (N = 255) from ten urban teaching hospitals in seven states were surveyed regarding their practice responses and training needs in bioethics. Responses to twenty-one practice situations in bioethics were analyzed in terms of levels of encountering ethical concerns, clinical participation and preparedness to handle ethical concerns. Whereas worker responses were greater in practice situations appearing more psychosocial than biomedical in nature, training needs were greater in those situations appearing more biomedical than psychosocial in nature. Rationing of health care, the number one training need across service specialties, stands out as the practice situation in which ethical concerns were encountered with the least preparation and participation. Prior training in ethical principles and analysis and service on a hospital ethics committee associated significantly with higher levels of worker responses. Implications for social work education and practice in today's health care environment are presented.

Adult↗
PubMed1992

Surgical aspects of chronic peritoneal dialysis in the neonate and infant under 1 year of age.

Since 1982 eight patients under 1 year of age with end-stage renal failure have been treated by chronic peritoneal dialysis (CPD) following insertion of an abdominal Tenckhoff catheter. We routinely perform a partial omentectomy now, and in males undertake bilateral exploration of the groins at the time of catheter insertion, with herniotomy or ligation of the patent processus vaginalis as required. Up to January 1990, 19 straight double-cuff catheters had been inserted with a total follow-up of 244.5 patient months. The median age at the initial catheter insertion was 14.6 weeks (range, 2 days to 11 months) and the median weight was 3.89 kg (range, 2.2 to 5.5). Peritonitis was the most common complication, with 46 episodes, representing one episode of peritonitis per 5.3 patient months on dialysis. The frequency of peritonitis has decreased in the last 6 months since all patients have been dialysed by two caregivers. The present rate of peritonitis is 1 episode per 10 patient months on dialysis. One patient has died of septicemia secondary to associated congenital abnormalities, one patient has regained renal function, and two patients have been transplanted, one successfully. Five patients are currently dialysing via their abdominal Tenckhoff catheters and awaiting transplantation. We conclude that neonates and infants under 1 year of age can be treated satisfactorily by CPD to enable successful preparation for transplantation later in childhood.

Catheters, Indwelling↗
PubMed1990

Ionic modulation of triethyllead neurotoxicity in cerebellar granule cell culture.

While the molecular mechanism underlying triethyllead (TEL) neurotoxicity is unknown, we hypothesize that triethyllead mediates an accelerated Cl-/OH exchange across neuronal membranes leading to prolonged depolarization and neuronal cell injury. As a test of this hypothesis we have investigated the effect of external ion modulation on triethyllead neurotoxicity in cerebellar granule cell culture. Cultures were prepared from neonatal rats and used 10-20 days in vitro. Cytotoxicity was assessed by lactate dehydrogenase (LDH) release and trypan blue exclusion. A slow, dose-dependent (1-30 microM TEL) release of LDH occurred after a variable latent period dependent upon [TEL]. External replacement of [Cl-]e by Na isothionate dramatically shifted the dose response curve to the left reflecting an accelerated stimulation of LDH release, while replacement of extracellular [Na+]e with equimolar choline chloride had a minimal protective effect. Similarly, high [Mg2+]e or low [Ca2+]e did not protect or potentiate TEL cytotoxicity. The low [Cl-]e accelerated TEL cytotoxicity was dependent on medium pH: alkaline pH potentiated the cytotoxicity. Low [Cl-]e had no significant effect on culture ATP over 5 hrs. ATP reduction was markedly stimulated by TEL in low Cl- medium in contrast to the minimal decline in [ATP] in the control medium. The reduction of ATP in the low [Cl-]e medium occurred prior to LDH or trypan blue staining release confirming that such reduction in [ATP] was not secondary to cell damage. Substituting K sulfate or Na sulfate for the Cl(-)-free medium revealed marked loss of ATP without LDH release in control and TEL supplemented cultures. These observations provide supporting evidence for the role of an abnormal Cl- flux in mediating TEL-induced neurotoxic injury. Specifically, the membrane depolarization is proportional to the gradient imposed by Cl- efflux/OH influx, stimulated by low [Cl-]e. The rapid loss in ATP appeared early, was not a secondary reflection of neuronal damage but a result of a combination of increased ion flux at the plasma membrane, stimulation of Na+/K+ ATPase and direct TEL-induced inhibition of mitochondrial oxidative phosphorylation.

Adenosine Triphosphate↗
PubMed1989

Biosynthesis of [14C]arachidonic acid from [14C]linoleate in primary cultures of rat Sertoli cells.

The conversion of [14C]linoleate to [14C]arachidonate by rat Sertoli cells was established by use of primary cultures. Most of the 14C from [1-14C]linoleate was located in C-3 of the synthesized arachidonate, indicating that the labeled tetraene had originated largely by elongation and desaturation of the intact labeled substrate rather than by mere addition of 14C-acetate generated by bio-oxidation of the radioactive substrate to an already existing 18-carbon precursor. Although a relatively small amount of 14C was present in 18:3 omega 6 and a relatively large amount of 14C was present in 20:2, it was not possible from these data to establish the relative importance of 20:2 in the biosynthesis of arachidonic acid in rat Sertoli cells.

Animals↗
PubMed1989

Interleukin 1-induced pathophysiology: induction of cytokines, development of histopathologic changes, and immunopharmacologic intervention.

In this report, we describe a murine system in which treatment with recombinant human interleukin 1 (IL-1) induced an acute lethal state with pathologic changes similar to septic shock at high doses and development of arthritic and other tissue changes following more prolonged treatment with lower doses. We have demonstrated that both recombinant human interleukin 1 alpha and recombinant human interleukin 1 beta could be administered to an endotoxin hyporesponsive strain, C3H/HeJ, and produce these pathologic changes. Induction of tumor necrosis factor (TNF) and colony-stimulating factor activity was noted. The ability to induce these changes was dose and time dependent. Histopathologic changes included lesions in the lung, heart, liver, adrenal glands, intestines, and joints. Neutrophil infiltration was a prominent feature in many organs. Drugs, immunotherapy, or other treatments which have been effective in delaying or preventing a lethal syndrome induced following high dose interleukin 2 therapy were not effective in preventing the interleukin 1-induced lethal syndrome. Interestingly, pretreatment with low nonlethal doses of IL-1 (but not lipopolysaccharides or TNF) could prevent deaths from an LD100 challenge dose of IL-1.

Adrenal Cortex↗
PubMed1989

Antibiotic therapy for Pseudomonas aeruginosa bacteremia: outcome correlations in a prospective study of 200 patients.

PURPOSE AND PATIENTS AND METHODS: We performed a prospective clinical study of 200 consecutive patients with Pseudomonas aeruginosa bacteremias to analyze in vitro susceptibility and synergistic testing of antibiotics the patients received and clinical parameters to assess their relationship to survival. RESULTS: No significant correlation between in vitro susceptibility testing (minimal inhibitory concentrations/minimal bactericidal concentrations) and outcome could be demonstrated. Similarly, improved outcome could not be demonstrated for patients receiving antibiotic combinations that were synergistic in vitro (either time-kill or checker-board) versus those combinations that were not. There was also no correlation between results obtained by time-kill curve and checkerboard synergistic testing, i.e., combinations found to be synergistic by one method were not necessarily synergistic by the other method. Clinical parameters associated with improved survival were a urinary portal of entry and absence of neutropenia. Conversely, survival was significantly decreased when the portal was the respiratory tract. The mortality rate between patients receiving combination therapy (27%) and monotherapy (47%) was significant (p less than 0.02); this significant relationship held true for most subgroups including malignancy, nosocomial infection, and infection site. CONCLUSION: Increasing effort should be placed on ensuring timely administration of combination therapy to patients with P. aeruginosa bacteremia since the use of combination therapy was even more important in determining outcome than was underlying disease.

Adolescent↗