Biomedical subjects
A Albanese
Publications and source records attributed to A Albanese.
Postural axial tremor in a patient with cerebellar atrophy.
A patient affected by low-frequency postural tremor of the trunk and limbs is reported. Apart from mild dysarthria and gait ataxia, no other neurological abnormalities were present. Cerebellar atrophy was demonstrated by means of magnetic resonance imaging. The tremor was associated with alternating activity in antagonistic muscles; it was triggered whenever a contraction of lumbar back extensor muscles occurred. Electrical stimulation of the cerebellum did not produce the normal suppression phases of motor responses evoked by a magnetic stimulation of the cerebral cortex.
Localization of MPP+ binding sites in the brain of various mammalian species.
The distribution and density of 3H-MPP+ binding sites were studied by in vitro quantitative autoradiography in the brain of the mouse, rat and monkey. The highest levels of 3H-MPP+ specific binding were observed in rat brain. The substantia nigra in rat and monkey, and the anterior caudate-putamen formation in mouse and monkey showed the lowest density of autoradiographic grains. The presence of a relatively high density of MPP+ sites in the hippocampus of all species studied could be of interest to explain some effects of MPTP administration on convulsions caused by chemoconvulsants. The finding of a 60-70% reduction of 3H-MPP+ binding sites in the rat caudate-putamen, on the side of quinolinic acid infusion and no changes after 6-hydroxydopamine lesion of dopaminergic nigrostriatal neurons suggests the presence of these sites mainly on striatal cells. The results suggest that the distribution of MPP+ binding sites in brain would not seem to be related to MPTP toxicity.
Growth hormone deficiency throughout puberty.
Growth during puberty does not appear to be the major determinate of final height in isolated GH deficient patients. Early diagnosis and commencement of therapy are probably the most important factors, as reflected by the correlation between final height and height at the onset of puberty. The cost effectiveness of increasing the dose of GH during puberty does not appear to represent any advantage from the data presently available. Indeed, such an approach may have a deleterious effect on final height by shortening the duration of pubertal maturation. Further prospective studies are required to demonstrate the effectiveness of manipulating the onset and duration of puberty using gonadotrophin releasing hormone analogues on final height in isolated GH deficiency.
Chronic administration of MPTP to monkeys: behavioural morphological and biochemical correlates.
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Optimum method for administration of biosynthetic human growth hormone: a randomised crossover trial of an Auto Injector and a pen injection system.
The use of optimum conventional growth hormone administration, using a growth hormone vial combined with an Auto Injector, was compared with a pen injection system using a cartridge of growth hormone. In both methods of administration the concentration of growth hormone was 16 IU/ml. Thirty patients (22 boys, eight girls) who had all previously been treated with growth hormone (4 IU/ml) administered using needles and syringes (without an Auto Injector) were randomised into receiving one of either treatment for three months and then crossed over for a further three months. Fourteen patients (10 boys, four girls) initially received KabiVial 16 IU/ml combined with an Auto Injector while 16 patients (12 boys, four girls) were treated with KabiPen 16 IU/ml. Mean age in both groups was 9.6 years. The majority of patients in both groups were treated with a regimen of either 15 or 20 units/m2/week as a daily subcutaneous injection. Of the 30 patients who started in this trial, two who commenced using an Auto Injector refused to change to a pen system and were excluded from further analysis. When scored on a scale of -5 to +5 general convenience when changing from an Auto Injector to the KabiPen decreased from +4.7 to +1.0. When assessed for pain, the Auto Injector group scored +4.7, which decreased to -0.2 (more painful) for the pen. At the end of the trial 23 patients (82%) chose to continue with the KabiVial/Auto Injector combination as they found this less painful and the child did not see the needle or need to insert the needle manually. Five patients (18%) continued with the KabiPen as they considered the device smaller and easier to use. The accuracy of dosing using KabiVial was 100% compared with the range of 88% to 111% using KabiPen as the latter was available only in 0.5 unit increments. No growth hormone was wasted using KabiVial, although a mean of 0.6 units was wasted with every 16 IU cartridge in the KabiPen system. It is concluded that patients should be able to contribute to the choice of growth hormone delivery systems and that newer methods need careful assessment.
Botulinum toxin as a treatment for blepharospasm, spasmodic torticollis and hemifacial spasm.
Fifty-two patients affected by focal dystonia or hemifacial spasm were treated with repeated injections of botulinum toxin. A clinical improvement was observed in all patients with blepharospasm; clinical benefit had a mean duration of 10 weeks. Clinical results were less impressive, but also favorable in patients affected by spasmodic torticollis and by hemifacial spasm. In the latter, the incidence of drug-induced paresis was much higher than that observed in patients with blepharospasm, even though the doses of toxin injected were significantly lower.
The effects of growth hormone therapy on spontaneous sexual development.
We have carried out a prospective randomised study in 52 (46 male, 6 female) children with isolated growth hormone (GH) deficiency treated with a GH regimen of 15 IU/m2/week administered as a daily subcutaneous injection. At the onset of the pubertal growth spurt, the patients were randomised to receive either an unaltered regimen (26 males, 1 female) or 30 IU/m2/week (20 males, 5 females). There was no change in the frequency of GH administration. The number of females in this study was too small to give a meaningful result. In contrast, the boys treated with either dose regimen showed no significant alteration in growth rate, but there was a faster than normal progression in pubertal maturation. It is concluded that the optimum final height of children with isolated GH deficiency may not be achieved in patients without the therapeutic manipulation of the onset and/or duration of puberty. 16 short normal children (9 males, 7 females) were treated with GH in a regimen of 25 IU/m2/week (range: 20-30) as a daily subcutaneous injection. The mean age for the onset of GH treatment was 11.5 and 11.0 years in the boys and girls, respectively. Our data suggest that both boys and girls had a more rapid rate of pubertal maturation than normal. It may be that in terms of final height prognosis, the events of puberty related to GH treatment counterbalance the improvement in growth prognosis during prepuberty.
Unsuspected, surreptitious drug-induced parkinsonism.
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Coating of commercially available materials with a new heparinizable material.
Different commercial materials, such as polyurethane (PU), plasticized PVC (PVC), glass, Gore-tex, and Dacron, were coated with a well-characterized biomaterial (PUPA) based on polyurethane and poly(amido-amine) components. Two different classes of coating were obtained due to the different characteristics of the substrates. In the case of PVC and polyurethane which are soluble in the solvent of the PUPA-coating solution, there was penetration and blending of the coating and underlying materials. In the case of glass, Gore-tex, and Dacron, which are insoluble in the solvent of the coating solution, only a superficial layer of PUPA could be obtained. The coating stability was investigated and the interaction between coating and underlying material studied by FT-IR. All the stable coatings showed the ability to bind as much heparin as PUPA material by itself.
Transient alternating hemichorea as presenting sign of progressive supranuclear palsy.
We report the case of a man who developed unilateral alternating chorea four years before presenting a typical clinical form of Steele-Richardson-Olszewski syndrome. It is known that choreic dyskinesias may occur during the course of this syndrome but this is the first account of choreas presenting sign of the syndrome.
Heparinized polyurethane surface through ionic bonding of heparin.
Surface heparinization through an ionic bond is one of the methods used to improve polyurethane blood compatibility. Chains of poly(amido-amine), a tertiary aminic polymer capable of forming stable complexes with heparin, were either surface-grafted on polyurethane or interconnected with polyurethane chains using hexamethylenediisocyanate as cross-linking agent. In the latter case, a new material (PUPA) is formed with a heparin adsorbing capacity higher than poly (amido-amine) surface-grafted polyurethane. By changing the percentages of the components, different series of PUPA materials can be obtained with different physico-chemical properties. The ATR/FT-IR technique was used to characterize the new materials in the native and in the heparinized state. PUPA solution was used to coat commercial biomedical devices and they were also characterized physicochemically using ATR/FT-IR.
[Role of diagnostic imaging in Rubinstein-Taybi syndrome. Personal experience with 8 cases].
Both etiology and pathogenesis of Rubinstein-Taybi syndrome (RTS) are still questionable, even though a genetic factor seems to be certain. A typical face, psychomotor delay, and thumb and halluces abnormalities (big, prevalently short, and often "spoon-like" toes) are the main characteristic patterns of RTS. Eight subjects (4 male and 3 female children aged 26 days-7 years, and a 31-year-old woman, mother of 1 of the affected children) with different signs of RTS were studied over the last 3 years. The results are here reported, with a special emphasis on malformations detected with conventional radiography (Rx), Computerized Tomography (CT), and ultrasound (US). Evaluated parameters were thumbs and halluces (Rx), bone age and skeleton (Rx), cranium (Rx) and encephalon (US, CT), cryptorchidism (US, CT), and urological (Rx, US) and cardiovascular (US) systems. A typical face and psychomotor delay were found in all cases, while thumb and halluces abnormalities were observed only in 6 cases. Among several clinical signs of RTS, we found: severe (less than 3rd centile) bone maturation delay in 4 cases; skull volume reduction (less than 50th centile) in 3 subjects and microcrania in 4; skeletal abnormalities in 7 cases (5 of them positive for bilateral coxofemoral abnormalities); urinary tract (4 cases) and cardiovascular (3 cases) malformations; and cryptorchidism in 3 of 4 males. A case was diagnosed during neonatal period (within the first month of life); it was a rare case associated with a variant form of Dandy-Walker anomaly; semiologic similarities were observed between mother and daughter patients. X-rays, US and CT rarely play an important role in the diagnosis of RTS, considering the several clinical signs, mainly the face, affecting the patients. However, diagnostic imaging techniques help diagnose hidden malformations and confirm and integrate clinical signs.
Aging is associated with a diffuse impairment of forebrain cholinergic neurons.
The study evaluates whether any degenerative changes affect forebrain cholinergic systems during natural aging. The medial septal nucleus, the nuclei of the diagonal band, the neostriatum, and the basal nucleus were studied in adult and aged Wistar rats. Butcher's technique for acetylcholinesterase allowed us to identify neurons located in these forebrain nuclei, which stained intensely or moderately for the enzyme and were putatively cholinergic. The size of forebrain regions containing stained neurons, and the number and size of stained perikarya located therein, were measured. In aged rats, the size of forebrain cholinergic nuclei was reduced by an average of 26%. The density of neurons located in these regions was also significantly lower in aged rats than in controls; intensely stained neurons displayed a mean reduction of 27.81%, while intensely and moderately stained perikarya together were reduced by 25.43%. Cross-sectional area of the stained perikarya was also reduced in aged rats by 32.87%. These data show that the number of forebrain acetylcholinesterase-containing neurons is reduced in aged rats. They are consistent with the hypothesis that natural aging brings about a diffuse and homogeneous depletion of forebrain cholinergic perikarya. Neurons which are viable, and can be selectively stained, show morphological alterations, which are likely to be related to a degenerative process.
Chronic administration of MPTP to marmosets.
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Anterior speech region. Asymmetry and weight-surface correlation.
Results of this work show, in the inferior frontal gyrus and in the different portions into which it was divided, asymmetry of weight and cortical surface and left predominance in the posterior portion, which is concerned with language. In spite of the difference among the absolute individual values, the correlation between weight and surface values is excellent in all cases, and the weight and surface ratio is the same for the portions related to language and different for the remainder of the inferior frontal gyrus.
Further treatment with MPTP does not produce parkinsonism in marmosets showing behavioural recovery from motor deficits induced by an earlier exposure to the toxin.
1-Methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP), or 0.9% saline, was administered to a group of common marmosets initially treated with the toxin 12-18 months previously. Initial treatment had consisted of a cumulative dose of 6-22 mg/kg (i.p.) which caused marked parkinsonism. Subsequently, the animals gradually recovered normal motor function. Further treatment consisted of a cumulative dose of MPTP of 78-83 mg/kg (i.p.) but this produced only modest akinesia. At 12-18 months after the initial treatment with MPTP, the content of dopamine, HVA and DOPAC in the caudate and putamen was markedly reduced. However, levels of dopamine, HVA and DOPAC in the nucleus accumbens were normal. Three months after the second treatment with MPTP there was no further decrease in the content of dopamine in the caudate-putamen. However, in the nucleus accumbens the content of dopamine, HVA and DOPAC was now reduced. The initial treatment with MPTP substantially decreased the binding of [3H]mazindol in the caudate-putamen but less so in the nucleus accumbens. Only a small additional decrease occurred upon further treatment with MPTP. The density of tyrosine hydroxylase (TH) immunoreactive cells in substantia nigra was reduced after the initial treatment with MPTP. However, the cell loss was far less marked than the decrease in terminal density, assessed by the binding of [3H]mazindol. Subsequent treatment with MPTP caused a small further loss of tyrosine hydroxylase-positive cells. Initial treatment with MPTP may kill the majority of MPTP-sensitive dopamine cells in the nigra. Compensation by the remaining nigrostriatal neurones may account for the behavioural recovery observed.(ABSTRACT TRUNCATED AT 250 WORDS)
Treatment of central precocious puberty with LH-RHa (buserelin): long term effect on growth, bone maturation and predicted height.
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