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Biomedical subjects

L Quaranta

Publications and source records attributed to L Quaranta.

At least 19 recordsLinked to original sources
PubMed2004

Arthropathies and thyroid diseases.

AIM: Improvement of articular symptoms following thyroidectomy has often been observed in patients with an association of thyroid and joint diseases. An assessment has therefore been made of the types of arthropathy thus benefited and the anatomopathological features of the thyroid in patients with concomitant joint diseases. An account is given of the arthropathies associated with nontoxic nodular goitre (NTG). METHODS: Three cell markers are examined to identify immunocytokine elements differentiating thyroid diseases. RESULTS: Immunohistochemical examination shows extravasal lymphocyte infiltrates; thyrocytes were negative for HLA-Cl II, CD38 and IL-6R, and only dim-positive for HLA-Cl I. Endothelial cells were positive for HLA-Cl I and II and CD38, and negative for IL-6R. The lymphocyte were positive for HLA-Cl I, HLA-Cl II and CD38, but negative for IL-6R. The follow-up of 6 thyroidectomised patients disclosed improvement in joint pain and remission of rheumatoid arthritis and spondylarthritis. Association of nodular goitre with arthro-pathies is demonstrated. CONCLUSION: Arthritis and arthralgia are frequent in patients with thyroid diseases, we particularly found the association with MHNG and Hurthle cell adenoma. Arthritis and arthralgia quickly improve after thyroidectomy. Immunohistochemical NTG thyrocytes are still normal cells (HLA-Cl II negative) by contrast with their HLA-Cl II positivity in autoimmune thyroiditis.

ADP-ribosyl Cyclase↗
PubMed2002

Peripheral neuropathy with hypomyelinating features in adult-onset Krabbe's disease.

We describe three brothers suffering from Krabbe's disease with onset in the fifth decade. The proband showed a complete deficiency of leukocyte enzyme galactocerebrosidase and was found to be heterozygous for two previously described mutations: G > A809 and 502T/del consisting of a 30 kb deletion. In all three brothers the neurological examination showed features of asymmetrical peripheral neuropathy associated with pyramidal signs and the electrophysiological examination showed a generalized slowing of nerve conduction velocities. Two patients died at 59 and 61 years of age due to respiratory failure. Both the proband and his brother underwent a sural nerve biopsy. In the former the most striking finding was the presence of uniformly thin myelin sheaths without evidence of demyelination; a complete absence of fibers was found in the latter. Our findings confirm that peripheral neuropathy may be the presenting feature of late-onset Krabbe's disease. Hypomyelination rather than demyelination may represent the distinguishing pathological finding of this condition.

Adult↗
PubMed2001

Small ring constrained peptidomimetics. Synthesis of epoxy peptidomimetics, inhibitors of cysteine proteases.

Different dipeptide analogues containing an oxirane ring in the place of the peptidic bond were prepared starting from naturally occurring amino acids. N-Fmoc-amino aldehydes were transformed into the corresponding methoxyvinyl derivatives through a Wittig reaction, and the addition of PhSeCl gave a series of different alpha-phenylselenyl aldehydes. Mukajiama reaction with silylketene acetals gave an intermediate product that was finally transformed into the desired oxiranyl peptidomimetics. Following this strategy we were able to control three new contiguous stereocenters starting from the enantiomerically pure amino acid. The dipeptide analogues could be used in SPPS on a SASRIN resin as the final epoxides were relatively unstable under acidic conditions. Moreover the synthesis of the single dipeptide mimetics was carried out on solid phase to generate a small library of epoxy peptidomimetics. Some of the products prepared in this work resulted as time-dependent reversible inhibitors of cysteine protease.

Cysteine Proteinase Inhibitors↗
PubMed2001

Administration of glutathione in patients with type 2 diabetes mellitus increases the platelet constitutive nitric oxide synthase activity and reduces PAI-1.

Several studies suggest that nitric oxide (NO) production is impaired in diabetes mellitus. Reduced levels of NO could contribute to cardiovascular mortality. Furthermore, NO synthesis is impaired in glutathione (GSH)-depleted human umbilical vein endothelial cells and GSH is reduced in patients with type 2 diabetes mellitus (T2DM). We tested the hypothesis that treatment with GSH may improve platelet constitutive NO sinthase (cNOS) activity in patients with T2DM. Fifteen patients with T2DM underwent a treatment with GSH 600 mg/day i.m. for 10 days. With respect to the basal values on the 10th day of treatment, the red blood cell GSH concentration and platelets cNOS increased (1.4+/-0.1 vs 1.9+/-0.1 micromol/10(10) RBC, p<0.001 and 0.7+/-0.1 vs 2.9+/-0.2 fmol x min(-1) x 10(-9) PLTs, p<0.001, respectively) and the plasma PAI-1 levels diminished (81.4+/-3.7 vs 68.7+/-4.0 ng/ml, p<0.002). A negative correlation between the cNOS and the PAI-1 was found on the basal values. After a wash-out of 30 days the values of red blood cell GSH concentration, platelet cNOS activity and PAI-1 Ag returned to the basal levels. These data suggest that the administration of GSH, in patients with T2DM, is able to improve platelet cNOS activity together with a reduction of PAI-1.

Aged↗
PubMed2001

Pure motor chronic inflammatory demyelinating polyneuropathy.

We describe four patients affected by chronic inflammatory demyelinating polyneuropathy (CIDP) in a pure motor form. Selective involvement of motor fibers was suggested by the absence of sensory symptoms, normal sensation at neurological examination and normal findings on electrophysiological testing of sensory fibres and sural nerve biopsy. The onset of the disease occurred at a young age (3-29 years) and the clinical course was relapsing-remitting. Over a follow-up period of 1.5-14 years, periodical clinical and electrophysiological examinations showed that selective involvement of motor fibers remained a constant feature. Electromyography and nerve conduction studies continued to show a purely demyelinating neuropathy without signs of axonal impairment. All patients were steroid-unresponsive, whereas they considerably improved after being treated with immunoglobulins. Two patients were treated with interferon alpha and showed a good response. In conclusion, the occurrence in our four patients of pure motor involvement over a long period of time during which several relapses occurred, suggests that pure motor CIDP may represent the result of a specific immunological process rather than of a random distribution of inflammation throughout peripheral nerves.

Adult↗
PubMed2001

Growth hormone-independent cardiotropic activities of growth hormone-releasing peptides in normal subjects, in patients with growth hormone deficiency, and in patients with idiopathic or ischemic dilated cardiomyopathy.

Growth hormone releasing peptides (GHRPs) are synthetic molecules endowed with potent neuroendocrine activities mediated by specific receptors in the pituitary and in the central nervous system. GHRPs receptors have been reported even in perpheral tissues, particularly in the myocardium, where they probably mediate growth hormone (GH)-independent activities. We studied in humans the cardiac effects of hexarelin administration in 7 normal adults, in 7 severe GH-deficient patients, and in 12 patients with severe dilated cardiomyopathy. Left ventricular ejection fraction (LVEF), mean blood pressure (MBP), heart rate (HR), and GH levels were evaluated at baseline and every 15 min up to 60 min after acute 2.0 microg/kg iv hexarelin administration. Basal LVEF in dilated cardiomyopathy was impaired and lower (p < 0.001) than in GH deficiency, in turn lower (p< 0.001) than in normal subjects. Hexarelin signficantly (p < 0.05) increased LVEF in normal and in GH-deficient subjects, but not in dilated cardiomyopathy, without significant variations in MBP and HR. Hexeralin significantly (p < 0.05) increased GH levels in normal subjects and in dilated cardiomyopathy but not in GH deficiency. These findings suggest that, in humans, the acute administration of hexarelin exerts a GH-independent positive inotropic effect likely mediated by specific GHRPs myocardial receptors.

Adult↗
PubMed1999

Ab-interno goniotrabeculotomy versus mitomycin C trabeculectomy for adult open-angle glaucoma: a 2-year randomized clinical trial.

OBJECTIVE: To evaluate the effect of ab-interno goniotrabeculotomy (AIGT) on the intraocular pressure (IOP) in adult patients with primary open-angle glaucoma (POAG), compared with the effects of mitomycin C trabeculectomy (MT). DESIGN: Prospective, randomized, clinical trial. PARTICIPANTS: Thirty-two eyes of 32 patients with medically uncontrolled POAG. INTERVENTION: Standard limbus-based trabeculectomy with adjunct mitomycin C (0.3 mg/mL for 3 minutes) in 16 eyes of 16 patients; AIGT was performed in 16 eyes of 16 patients. The groups were matched for age, preoperative IOP, duration of preoperative antiglaucoma treatment, use of preoperative beta-blockers and parasympathomimetics, and use of beta-blockers in the fellow eye. The IOP (average of the two highest values measured in the diurnal curve, from 8 AM to 6 PM, every 2 hours) and complications were recorded 1, 3, 6, 12, 18, and 24 months after surgery. MAIN OUTCOME MEASURES: Identification of complications and IOP. RESULTS: All patients were followed up for 24 months. More postoperative complications occurred in the MT group during the 2-year follow-up. One month after surgery, IOP was 10 +/- 1.46 mmHg (range, 8-13) in the MT group and 12.12 +/- 1.63 mmHg (range, 8-14) in the AIGT group (Student's t test, P = 0.001). Three months after surgery, IOP was 11.5 +/- 1.59 mmHg (range, 8-14) and 12.75 +/- 1.57 mmHg (range, 10-16) in the MT and AIGT groups, respectively (Student's t test, P = 0.033). From the 6th to the 24th postoperative month, no statistically significant difference in IOP was found between the two groups. At the end of follow-up, 14 of 16 eyes (87.5%) of the AIGT group and 13 of the 16 eyes (81.25%) of the MT group showed an IOP < or = 14 mmHg. CONCLUSION: Ab-interno goniotrabeculotomy appears to be a viable and safe surgical treatment for adult POAG. More extended follow-up, however, and a larger series of patients are needed to ascertain the actual effectiveness of this procedure in adult POAG.

Anterior Chamber↗
PubMed1998

Congenital glaucoma associated with Rubinstein-Taybi syndrome.

PURPOSE/METHODS: Rubinstein-Taybi syndrome is a constellation of clinical findings characterized by mental and motor retardation, broad thumbs and broad first toes, marked growth retardation, microcrania, typical facies, high-arched palate, and cryptorchidism in males. Ocular and adnexal abnormalities are quite common and include antimongoloid slant of the palpebral fissures, epicanthal folds, congenital obstruction of the lacrimal excretory system, ptosis, strabismus, and severe ametropia. Macrocornea, microophthalmos, colobomas of the iris and of the optic nerve head, congenital cataract, and optic nerve atrophy have also been described. Congenital glaucoma is a rare complication. We examined a patient with Rubinstein-Taybi syndrome with bilateral congenital glaucoma. RESULTS/CONCLUSIONS: Examination of this patient revealed bilateral antimongoloid slants of the palpebral fissures, and bilateral congenital glaucoma. Gonioscopic examination revealed the iris to be inserted flatly into the trabecular meshwork. This case emphasizes the importance of detailed, complete ocular examinations in patients with Rubinstein-Taybi syndrome, and also highlights the occurrence of ocular abnormalities rarely associated with this disease.

Female↗
PubMed1997

Color Doppler imaging of ophthalmic artery blood flow velocity: a study of repeatability and agreement.

PURPOSE: Color Doppler imaging (CDI) is a relatively new technique that allows quantification of blood flow velocity in orbital and ocular vasculature. Despite the numerous clinical studies that have used CDI, repeatability of this technique and agreement between observers have not been documented. METHODS: The authors performed a prospective investigation of the repeatability and agreement between observers on ophthalmic artery blood flow velocity measurements in 35 patients (35 eyes). RESULTS: Results on the estimated error of measurement (variability between repeated readings on the same subject) indicate good repeatability of the measurements; in fact, the measurement variances were only 5.6% for the peak systolic velocity, 11.4% for the end diastolic velocity, and 6.2% for the mean envelope velocity. The statistical analysis of repeatability showed a very narrow 95% confidence interval for both observers. The measurement of agreement between the two observers demonstrated the existence of a good concordance of the measurements taken by each observer on each subject. CONCLUSIONS: Results suggest that CDI is a reliable tool for quantitative assessment of ophthalmic artery blood flow velocity.

Aged↗
PubMed1996

[Laparoscopy: access with open versus closed technique].

Pneumoperitoneum in diagnostic or therapeutic laparoscopy can be achieved by an open or a closed technique. The authors, on the basis of their experience, compare both techniques, concluding that open method is safer, because of the lack of major complications observed in the closed method and with only few minor complications.

Cholecystectomy, Laparoscopic↗