[Relation, in Raynaud's phenomenon, of clinical severity and severity as disclosed by 2 methods of non-invasive vascular diagnosis].
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Diagnosis-Related Groups↗
Biomedical subjects
E Bianchi
Publications and source records attributed to E Bianchi.
Effect of dimethylsulfoxide pretreatment on valproic acid pharmacokinetics in rat.
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Animals↗
Plasma and urine pharmacokinetics of free and of short-chain carnitine after administration of carnitine in man.
To 6 healthy volunteers 30 mg/kg of L-carnitine (1,3-hydroxy-4-N-trimethylamino-butyrate) were injected intravenously and plasma levels (mumol/l) of free and short-chain carnitine were determined at different times between 0.033 and 24 h. The urinary excretion of L-carnitine and short-chain carnitine in 24 h was also measured. After a period of wash-out the subjects received 100 mg/kg of L-carnitine orally and plasma levels were determined between 0.5 and 24 h. The urinary excretion of L-carnitine was measured for a period of 18.5-33 h after treatment. 3 of the volunteers also received 30 mg/kg of L-carnitine orally. Carnitine plasma levels were determined at different times between 0.5 and 18 h, while the urinary excretion of L-carnitine was measured for 48 h following the treatment. The results could indicate the presence of saturation phenomena in the absorption process for the oral doses used; specific research is required to ascertain this phenomena. The transfer of carnitine from central to extravascular volume is relatively rapid, as is its urinary excretion. The short half-life of carnitine and acetyl-carnitine can suggest the use of new forms of administration (slow-release).
Administration, Oral↗
Serum and urine kinetics of rosaprostol in volunteers.
The kinetics of rosaprostol (9-hydroxy-19,20-bis-norprostanoic acid, Rosal) and of its metabolite (3-(2-n-hexyl-5-hydroxy-cyclopentyl)propionic acid) has been determined in plasma and in urine of 10 healthy volunteers after oral administration of 500 mg of rosaprostol. The peak of rosaprostol (of 524 ng/ml) appears at 4 h, whereas that of the metabolite (of 503 ng/ml) appears earlier (2 h); therefore the relationship between the two substances does not follow the precursor-successor relationship in plasma and a compartmental model has been used to fit the data. In this model the biotransformation process occurs before entering the central compartment (first-pass effect). The mean half-life of rosaprostol is equal to about 5 h and that of the metabolite is equal to 3 h. All of rosaprostol is biotransformed and only the metabolite is partially eliminated by the urine. The urinary excretion of the metabolite represents only a small fraction of the administered dose. The urinary clearance of the metabolite is equal to 5.3 l/h. The volume of distribution of both substances is equal to 21.2 l.
Anti-Ulcer Agents↗
Unusual association of glycogen storage disease type I, nesidioblastosis and possible congenital hypopituitarism.
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Consanguinity↗
Pharmacokinetic profile of fosfomycin trometamol (Monuril).
The kinetics of fosfomycin in serum and in urine was studied in 5 healthy volunteers after intravenous administration of disodium fosfomycin and after oral administration of trometamol fosfomycin at 50 mg/kg. The presence of secondary peaks in serum kinetics, more evident after oral administration, requires the use of a compartmental model with enterohepatic recirculation to which all the data of each subject were simultaneously fitted. The following values of the various pharmacokinetic parameters were calculated: central volume 10.6 +/- 0.92 liters; bioavailability 0.58 +/- 0.04; delay time in the recirculation 2.00 +/- 0.92 h; half-life 2.43 +/- 0.31 h; total clearance 8.3 +/- 1.6 l/h; urinary clearance 7.0 +/- 0.9 l/h; peak level 32.1 +/- 3.0 micrograms/ml; time of the peak 2.2 +/- 0.44 h. The fosfomycin concentration in urine remains above 1,000 micrograms/ml for 12 h and above 100 micrograms/ml for 48 h. The oral kinetics of fosfomycin is dose-dependent, as shown by serum and urine kinetics in other 4 volunteers after oral administration of 2, 3, 4 and 5 g; the fraction of the dose excreted by urine goes from 50% for 2 g to 22% for 5 g.
Administration, Oral↗
Localization and properties of the ATPase in the inner ear of rat.
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Adenosine Triphosphatases↗
Flunoxaprofen pharmacokinetics in elderly subjects.
Plasma kinetics and 24 h urinary elimination of flunoxaprofen, a nonsteroidal antiinflammatory drug, were studied in 23 elderly patients (mean age 69.9 years) and compared with the data obtained in four young volunteers. The drug was administered as a single oral 100 mg tablet and its plasma and urine concentrations were assayed by a high performance liquid chromatography method. Plasma kinetics fitted a 3-exponential equation with a mean half-life of 7.9 +/- 2.17 hours and a mean peak plasma of 8.5 +/- 2.97 micrograms/ml, which was observed at about the second hour. The values of the areas under the curves (AUC) and the values of total clearance (multiplied by the bioavailability) showed great variability, due to the large differences in the patients body weights; in fact the value of AUC was linearly correlated to the dose divided by the body weight. The mean residence time (MRT) of the drug in plasma was equal to 12.81 h. Low amounts of unmodified drug (about 10%) were found in 24 h urine sample, indicating a high degree of biotransformation. Small differences only were found in plasma kinetics of flunoxaprofen among the present group of elderly patients and the group of four young volunteers; the main difference corresponded to a slower rate of gastrointestinal absorption and to a longer mean residence time.
Aged↗
The lymphatic route--II. Pharmacokinetics of human recombinant interferon-alpha 2 injected with albumin as a retarder in rabbits.
The aim of the present investigation was to define whether multisite subcutaneous (s.c.) administration in unanesthetized, unrestrained rabbits of human recombinant interferon-alpha 2 (rec. IFN-alpha 2) either in saline, human albumin (ALB) solution (4, 7 and 10% final concentrations), or in a solution containing 75 U of hyaluronidase, modified the pharmacokinetic parameters calculated from the IFN plasma levels. Plasma disappearance rates of rec. IFN-alpha 2 were measured in rabbits after intravenous (i.v.) administration and the kinetic was adequately represented by a three-pools mammillary model. This model was the basis for evaluating the absorption and distribution of rec. IFN-alpha 2 after s.c. administration. The increase of ALB concentration (from 4 to 10%) caused a significant reduction of the plasma IFN Cmax while both the mean residence time and the release time of IFN increased linearly with the ALB concentration. The data support the postulation that s.c. administration of albumin acts as an interstitial fluid expander and may favour absorption of IFN via lymphatics rather than blood capillaries. Improvement of therapeutic index of IFN by using this route remains to be shown in clinical trials.
Animals↗
Dup(3)(p2----pter) in two families, including one infant with cyclopia.
We report on 2 unrelated cases of duplication of distal 3p due to balanced maternal translocation t(3;6)(p23;q27) and t(2;3)(p25;p23) respectively. One family was ascertained through the unbalanced offspring and the other through echographic examination of the balanced carrier mother. These cases confirm that dup(3)(p2----pter) results in a characteristic syndrome with distinctive facial appearance. In family 2 inspection of a photograph of a deceased sib was sufficient to conclude that he was affected. The patient in family 2 had cyclopia. Since holoprosencephaly was also reported by Martin and Steinberg [1983], we conclude that this anomaly appears to be a sign of the syndrome. The duplication usually derives from a maternal balanced translocation, in most cases from adjacent-1 segregation. However, family 2 was ascertained through a balanced female carrier who inherited the translocation from the father. We have noted that the second chromosome (which varies without apparent preferences) involved in these translocations is broken consistently at a distal band.
Abnormalities, Multiple↗
A family with the Saethre-Chotzen syndrome.
Acrocephalosyndactyly (ACS) is an inherited syndrome of premature synostosis of the cranial sutures and abnormalities in the distal segments of the limbs. Several forms of ACS have been described. ACS type III (or Saethre-Chotzen syndrome) is characterized by microcephaly, skull asymmetry, mild syndactyly, and facial abnormalities. We describe an Italian family with ACS III in which two sibs are clearly affected; the mother and the maternal grandmother show some features of the syndrome.
Acrocephalosyndactylia↗
Rectal extrusion of the catheter and air ventriculography following bowel perforation in ventriculo-peritoneal shunt.
Ventriculo-peritoneal shunt is frequently carried out in infantile hydrocephalus. The peritoneal shunt has a lower morbidity than ventriculo-atrial shunts and severe complications are uncommon. Abdominal complications include intestinal perforation, shunt migration, inguinal hernia, cerebrospinal fluid pseudocysts and hollow viscus perforation. A few cases of catheter extrusion from the rectum, vagina, umbilicus and urethra have been described. We report a new case of intestinal perforation with rectal extrusion of the catheter associated with a ventriculogram.
Cerebrospinal Fluid Shunts↗
Efficacy of minimal doses of an American hepatitis B vaccine in neonates.
Eight neonates and three young children at risk for hepatitis B infection were vaccinated with minimal doses of inactivated hepatitis B vaccine. The purpose of the study was to evaluate the efficacy of low doses with a view toward reducing the cost of immunization and therefore extending the practice to a larger number of subjects and minimizing the incidence of adverse reactions to the vaccine. The patients received two intramuscular injections (0.1 ml) of vaccine at a two-month interval and a booster six months after the first dose. Anti-HBs titers were determined simultaneously by radioimmunoassay on all serum samples collected. Five of the 11 patients achieved presumably protective (greater than 10 mU/ml) antibody levels within six months after the first dose (titers between 10 and 74 mU/ml), and all but one patient achieved titers between 27 and 2,500 mU/ml within two months after the booster. No adverse reaction was observed.
Child, Preschool↗
[Clinical course of brain tumors in childhood].
Brain tumors are, after leukemias, the most frequent fatal neoplasms of infancy. The clinical features and symptoms are often markedly different from those observed in the adult forms, according to the peculiar anatomy and behaviour of the child. Persistent headache, vomiting, astenia , behavioral alterations may be the precocious findings. Later, some more specific and suggestive signs such as strabismus, dyplopia , fast head size increase, funduscopic alterations, ataxia, paresis and nystagmus may be observed. On their appearance a prompt diagnostic work-up should be performed. The tumors of the posterior fossa (cerebellar astrocytoma and medulloblastoma, brainstem glyoma , hependimoma in decreasing order of frequency) generally cause precocious symptoms because of the small dimension of the subtentorial space; the presence in this region of several fundamental nuclei and pathways may explain how also small tumors may cause severe deficits. Supratentorial tumors (astrocytoma, malignant glyoma , hependimoma , craniofaringioma ) often show a more prolonged latency and may begin with signs of endocranial hypertension, seizures, or sometimes with hormonal troubles according to the involved anatomic structures. Hypothalamic astrocytoma is responsible of an extreme weight loss as far as to a cachetic status, due to the hyperincretion of GH. Finally, plexus papilloma, dermoid, optic nerve glyoma , oligodendroglyoma , germinoma, teratoma are responsible of a small number of child brain tumors, with different localization and symptomatology.
Astrocytoma↗
[Supportive therapy in brain tumors in children].
Recent advances in brain tumors therapy in infancy may be attributed not only to surgical, chemical and physical therapies improvement, but also to a more complete and accurated assistance to the neoplastic child. The constant support of an up-to-date and specialized laboratory and nursing staff may reduce the most common compliances of the neoplastic disease course and therapy. Neoplastic child with his family is facing a terrible stress, often with incumbent death risks. So a psychologic help is advisable for the whole family, and the best feeling is requested for the physicians. Supportive therapy must provide prevention and correction of the most common compliances of tumoral conditions, i.e. infections and hemorrhages . A correct and specific antibiotic therapy is absolutely necessary, but sometime is not sufficient. The introduction of hemoderivatives let us to spare the side effects of excessive whole blood transfusions, while the defective component may be selectively provided. Granulocyte transfusion recently introduced, may be the clue to the resolution of an overcoming infection.
Adolescent↗
Ruvalcaba syndrome: a case report.
An 11-year-old child with mental retardation and short stature was examined and found to be affected with some skeletal malformations. The clinical and radiological pattern of limb alterations was particularly suggestive of the features of Ruvalcaba syndrome. A complete examination confirmed the diagnosis and showed ocular involvement. To the best of our knowledge this is first published confirmation of Ruvalcaba syndrome.
Adolescent↗
Non-progressive psychomotor retardation in a child with severe deficiency of arylsulphatase A activity.
Severe deficiency of arylsulphatase A (ARSA) activity was detected in a boy with delayed psychomotor development, coarse face and liver enlargement when he was aged 2. The case does not fit into the description of the Metachromatic leukodystrophy (MLD) proposed by Hagberg (1963) mainly because he did not deteriorate. Twelve years of follow up did not show any signs of decerebration or further intellectual decline; only speech was, and still is, absent. The reduced ARSA activity in leukocytes and fibroblasts of the father of the propositus suggests that he is heterozygote and that the ARSA deficiency of the propositus is inherited. The ARSA activity of the mother overlaps with the lowest control. The assumption that the mother is a carrier of different mutations could explain the discrepancy between the clinical and biochemical findings of the patient. This is a further family in which mutations other than the typical one can be postulated to explain variable clinical or biochemical pictures of MLD.
Cerebroside-Sulfatase↗
RIA evaluation of antibody levels in neonates from HBsAg-positive mothers after active, passive, and combined immunoprophylaxis.
We evaluated the anti-HBs titers in 20 babies born to HBsAg-positive mothers and receiving different programs designed to prevent hepatitis B. Passive prophylaxis by HBIG for 6 months assured detectable antibody levels in the same period. 3 months after the last injection we could detect antibodies in 5 out of 9 cases. In no case could we detect antibodies 6 months after the last dose. Active prophylaxis by hepatitis B vaccine was able to raise anti-HBs response in 8 out of 12 babies after two or three very small doses (1.25 micrograms of antigen). 2 of these babies were given the first dose at birth. Small doses of vaccine were immunogenic in the presence of passive antibodies.
Antibodies, Viral↗