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Biomedical subjects

J Borel

Publications and source records attributed to J Borel.

At least 19 recordsLinked to original sources
PubMed1998

[Optimizing plasma cell content in bone marrow aspirates].

Bone marrow samples used for flow cytometric analysis of plasma cells frequently provide a low plasma cell content. Regularly, samples used for flow cytometry are provided by second step aspiration while the first aspirate is used for cytologic examination. We investigated whether the use of secondary aspirates leads to a systematic underestimation of the bone marrow plasma cell content as a consequence of an increased blood contaminant. To test the hypothesis, plasma cell (CD38bright) percentages were established by flow cytometry in 13 pairs of primary/secondary aspirates. In all cases we found lower plasma cell contents in secondary as compared to primary aspirates (p = 0.0015). Median plasma cell counts in secondary aspirates were 57% lower compared to primary aspirates. We conclude that the use of secondary aspirates leads to systematic underestimation of the bone marrow plasma cell content.

Bone Marrow↗
PubMed1996

Effect of erucic acid on platelets in patients with adrenoleukodystrophy.

In a clinical trial for the management of adrenoleukodystrophy, we analyzed the effect of erucic acid (a component of Lorenzo's oil) on platelet number, fatty acid composition, and function. Analysis of variance was performed to compare platelet counts before starting treatment with Lorenzo's oil and at 6 and 12 months. We measured platelet fatty acid composition in subjects and control patients and correlated these values with their platelet counts using discriminant analysis. After 6 months, the mean platelet count decreased from 247,000/mm3 to 169,000/mm3 (+/- 1 standard deviation 58,000,n =39), P < 0.0001 compared to 18 subjects on a control diet having a mean baseline platelet count of 259,000/mm3 (+/- 1 standard deviation 67,000, n = 19) and at 6 months 267,000/mm3 (+/- 1 standard deviation 71,000). We found at P < 0.05 that the platelet counts showed a strong inverse relationship with erucic acid levels and other omega 9 fatty acids that form from the administration of the erucic acid component of Lorenzo's oil. Morphologic and platelet sizing measurements suggest that the physical properties of platelets may also be affected by erucic acid. Our studies show that the ingestion of erucic acid affects platelet biology. This indicates that platelet counts and properties are influenced by monounsaturated fatty acids, in addition to the well-known effects of polyunsaturated fatty acids. In areas of the world where erucic acid is widely ingested, the biology of platelets in these populations may be affected.

Adrenoleukodystrophy↗
PubMed1995

[Flow cytometry identification of CD34-positive cells].

In the present study three clinical applications of flow-cytometric quantification of CD34-positive cells in the field of blood and marrow transplantation are evaluated. By daily determination of CD34-positive cells in the peripheral blood after mobilization of stem cells with chemotherapy or growth-factors it is possible to set the optimal time for collection of the transplant material. Thereafter the yield of CD34-positive cells within the harvested material is determined. Finally, the efficacy of positive CD34 selection using a biotin-avidin column is evaluated. Initial experience is presented here.

Antigens, CD↗
PubMed1995

Dietary management of X-linked adrenoleukodystrophy.

Adrenoleukodystrophy (ALD) is an X-linked disorder that involves mainly the nervous system white matter and adrenal cortex. It is associated with the accumulation of saturated very-long-chain fatty acids (VLCFAs), such as hexacosanoic acid (C26:0), that occurs as a result of the impaired capacity to degrade these substances, a reaction that normally takes place in the peroxisome. The VLCFAs originate from the diet and are also synthesized endogenously. Interest in dietary therapy arose from the observation that the administration of oils containing erucic and oleic acid (Lorenzo's oil), when combined with restriction of dietary intake of VLCFAs, can normalize plasma VLCFA levels in ALD patients. Clinical results in patients who are already symptomatic have been disappointing. However, preliminary data, still in need of confirmation, suggest that dietary therapy begun in asymptomatic patients can reduce the frequency and severity of later neurological disability.

Adrenoleukodystrophy↗
PubMed1994

Brain, liver, and adipose tissue erucic and very long chain fatty acid levels in adrenoleukodystrophy patients treated with glyceryl trierucate and trioleate oils (Lorenzo's oil).

Brain, liver, and adipose lipids were studied in the postmortem tissues of four adrenoleukodystrophy patients who had been treated with a mixture of glyceryl trioleate and trierucate oils ("Lorenzo's Oil") and compared to 7 untreated ALD patients and 3 controls. The dietary therapy appeared to reduce the levels of saturated very long chain fatty acids in the plasma, adipose tissue and liver; in the brain they were reduced in only one of the four patients. While substantial amounts of erucic acid were present in some of the tissues even 12 months after therapy had been discontinued, the levels in brain did not exceed those in controls at any time. The failure of erucic acid to enter the brain in significant quantity may be a factor in the disappointing results of dietary therapy for adrenoleukodystrophy.

Adipose Tissue↗
PubMed1992

Adrenoleukodystrophy: phenotypic variability and implications for therapy.

X-linked adrenoleukodystrophy (ALD) is a relatively common disorder that shows a great deal of phenotypic variability. Approximately half of the patients have the rapidly progressive childhood cerebral form that is associated with an inflammatory response in brain and leads to total disability or death during the first decade. Twenty five per cent or more of the patients have adrenomyeloneuropathy (AMN), a form that progresses slowly, involves the spinal cord mainly, shows little or no inflammatory response, manifests in adulthood, and is compatible with a near-normal life span. The two forms of the disease occur frequently within the same kindreds and nuclear families. Segregation analysis based on 3862 individuals in 89 kindreds points to the existence of an autosomal modifier locus with a likelihood ratio of 20:1. In addition, we present preliminary results of three types of therapy. Two hundred and four patients have received a dietary regimen that combines the administration of oils containing mono-unsaturated fatty acids (oleic and erucic) with the restricted intake of very long-chain fatty acids. This regimen normalizes the levels of satured very long-chain fatty acids in plasma within 4 weeks. It appears to improve peripheral nerve function in patients with AMN, and a large-scale trial is in progress to determine whether it can prevent the onset of neurological involvement in patients who have the biochemical abnormality of ALD but are neurologically intact. We report early results of bone marrow transplantation in 14 patients. There is encouraging but still preliminary evidence that transplantation can arrest the progression of the disease in patients with mild neurological involvement. There is urgent need to develop methods to combat the rapid progression of the cerebral forms of the disease, which so far has resisted therapeutic intervention, including immunosuppression or the administration of immunoglobulin.

Adolescent↗
PubMed1992

Improvement of clinical and MRI findings in a boy with adrenoleukodystrophy by dietary erucic acid therapy.

A 5-year-old boy with adrenoleukodystrophy, with clinical symptoms of visual, mental and motor disturbances which progressed rapidly, was treated with Lorenzo's oil consisting 1 volume of glyceryl trierucate and 4 volumes of glyceryl trioleate. Five months after initiation of this therapy, ability to swallow was enhanced and T2-weighted magnetic resonance imaging of the brain revealed regression of high intensity area of the parieto-occipital white matter.

Adrenoleukodystrophy↗
PubMed1988

Selenium treatment in neuronal ceroid-lipofuscinosis.

Neuronal ceroid-lipofuscinosis (NCL) refers to a group of disorders with devastating effects on the central nervous system. The accumulation of autofluorescent lipopigments containing lipid peroxides is considered a pathogenetic mechanism of the cell damage seen in NCL. Therapy aimed at preventing further lipid peroxidation, such as the Zeman regimen, did not slow progression of the disease. Therefore, Santavuori and Westermarck [Santavuori and Westermarck 1984] introduced treatment with a combination of selenium and vitamin E and reported favorable results with few side effects. We present information on the rationale for the use of selenium, recommendations on the daily intake, and reported side effects. However, our limited experience with selenium in this disorder does not permit conclusions. Additionally, careful studies are indicated before this treatment is dispensed routinely.

Child↗
PubMed1987

A new dietary therapy for adrenoleukodystrophy: biochemical and preliminary clinical results in 36 patients.

A new dietary regimen has been administered for periods ranging from 60 days to 1 1/2 years in 34 patients with various forms of X-linked adrenoleukodystrophy (ALD), as well as in 1 patient with neonatal ALD and 1 patient with infantile Refsum's disease. The diet combines the administration of a glyceryl trioleate oil (GTO) with the dietary restriction of very-long-chain fatty acids (VLFA), particularly hexacosanoic acid (C26:0). Reductions in the levels of plasma C26:0 and other VLFA were achieved in 25 of the 36 patients. Fifteen of these 25 patients were treated for more than 100 days. The mean reduction of the plasma C26:0 level was 53% (range, 22 to 73%) in these 15 patients. While the focus of the study was on biochemical variables, comparison of pre- and post-diet studies of peripheral nerve function showed improvement in 1 patient with adrenomyeloneuropathy (AMN) and 1 heterozygote. In contrast, 2 patients with ALD onset in childhood developed new neurological deficits while on therapy. We conclude that it is possible to lower plasma VLFA levels in ALD patients. A clinical trial is indicated to test whether this approach can alter the neurological progression in patients with AMN or in symptomatic heterozygotes, and to determine whether it can prevent the onset of neurological disability in asymptomatic persons who have the biochemical defect of ALD.

Adrenoleukodystrophy↗
PubMed1986

A comparison of the effectiveness of cyclosporine A, D, and G in the treatment of experimental autoimmune uveitis in rats.

Cyclosporine A (CsA), one compound in the family of cyclosporines, has effectively modulated the course of S-antigen induced experimental autoimmune uveitis (EAU). Cyclosporines G (CsG) and D (CsD), related to CsA in structure, were evaluated in their ability to prevent or modulate EAU in Lewis rats. 10 mg/kg/day IM of CsA effectively prevented the expression of EAU when therapy began on the day of immunization, while the same dosage of CsG prevented EAU in 81% of animals, and CsD only in 33%. Higher concentrations of CsG (40 mg/kg/day) did effectively block manifestations of the disease. Topical administration of CsG did not prevent the expression of disease but local protection was seen when the 500 micrograms CsG was placed intracamerally into only one eye. The in vitro comparison of these cyclosporines' capacity to alter proliferation and IL-2 release of a rat T-cell line capable of inducing EAU showed marked differences. CsA appeared to be most effective at abrogating these cellular functions at all concentrations tested, while CsD was least effective. CsG, however, approached the effectiveness of CsA. CsG is felt to be markedly less nephrotoxic than CsA, the secondary effect that is most commonly encountered, and could potentially be useful in the treatment of human intra-ocular inflammatory disease.

Animals↗
PubMed1984

T-tube suction drainage and/or prophylactic two-dose metronidazole in abdominal hysterectomy.

The efficacy of a two-dose perioperative course of rectally administered metronidazole and/or suction drainage of the vaginal vault in preventing febrile and infectious morbidity was evaluated in a randomized study of 198 patients undergoing abdominal hysterectomy. When compared with the control values, the incidence of febrile morbidity was significantly reduced (from 49% to 10%, 24.5%, and 10% respectively) in the groups treated with metronidazole, T-tube suction drainage, and a combination of the two prophylactic measures. The incidence of infectious morbidity was significantly reduced by using prophylactic metronidazole alone or in combination with suction drainage, but the reduction was most significant in the group treated with metronidazole alone. In the group managed with suction drainage alone, no reduction in infectious morbidity was found. The rate of postoperative urinary tract infection was significantly lower in the group treated with metronidazole alone as compared with the control group. No side effects of metronidazole were encountered.

Adult↗
PubMed1984

Prophylactic metronidazole or suction drainage in abdominal hysterectomy.

The efficacy of a two-dose perioperative course of oral metronidazole or suction drainage of the vaginal vault was evaluated in a randomized study of 150 patients undergoing abdominal hysterectomy. Patients receiving metronidazole demonstrated a significant reduction in febrile morbidity (from 48 to 8%), infectious morbidity (from 46 to 16%), urinary tract infections (from 30 to 10%), and pelvic and/or abdominal wound infections (from 18 to 2%). The patients managed with suction drainage showed a marked reduction in febrile and infectious morbidity; however, this reduction was not significant. Overall, 17% of all specific postoperative infections were first diagnosed after discharge from the hospital. No adverse side effects of metronidazole were encountered.

Adult↗