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Biomedical subjects

V Biarese

Publications and source records attributed to V Biarese.

At least 55 records · Page 3Linked to original sources
PubMed1977

[Lymphoid myelofibrosis or hairy cell leukemia].

By lymphoid myelofibrosis or hairy cell leukaemia or tricholeukaemia is meant an unusual haemopathic condition known only for the past few years. It is characterized pathognomonically by the presence of lymphocyte type cells with villous extroflexions, hence the name "hairy cell". Clinically the disease presents as an involutive myelopathy associated with splenomegaly, generally without any particular lymph gland involvement. The attention of students today is concentrated on the nature of the hairy cells; while some are inclined to admit their monocyte or histiocyte derivation, others consider that they derive from B lymphocytes. Therapeutically, almost everybody agrees that splenectomy is the only valid step. A case of H.C.L., which was typical from the clinical and laboratory viewpoints is reported. It is probable that certain haemopathic pictures once classified among atypical leucoses and lymphomas, would today be more correctly classed as hairy cell leukaemia.

Alkaline Phosphatase↗
PubMed1977

[Fanconi-Zinsser disease].

Fanconi-Zinsser's disease is a serious involutive myelopathy responsible for pancytopenia, hyperpigmentation, oralplakia and onychodystrophy, together with lesser dysmorphisms in many cases. The marrow blood picture is reminiscent of the better-known Fanconi's disease, while the skin and mucosa picture is similar to that of congenital dyskeratosis - hence the combined name. A typical case, but complicated by Lewandowsky's disease for the first time in the literature, is presented. Papova-virus was noted in the typical verrucae. The modern "pathology due to genome instability" is examined. Lewandowsky's disease is regarded as a familial form and precancerous, owing to its possible Bowenoid transformation. The association is seen as particularly significant in stressing the disorder of the immunocompetent syste, this being most evident from the finding of serum anti-red-cell auto-antibodies and a deficiency of T lymphocytes. Fanconi-Zinsser's disease has only been reported 21 times in the literature, mostly in males. Incomplete forms are, however, noted in relatives, suggesting that it originates in a genetic disorder whose transmission modality is not clear, though incomplete dominance is suspected. Genome instability is probably responsible behind the onset of the disease and its neoplastic complications - these being also feature of other forms provoked by such instability, such as Bloom's syndrome and ataxia telengiectasia. Fanconi's disease also has marked neoplastic tendencies. Clinically, Fanconi-Zinsser's disease can be classified as distinct, since it has signs and an evolutive modality that distinguish it from Franconi's disease, Estren-Damesheck's syndrome and amegakaryocytic thrombocytopenia. Genetically, it can be seen that all these diseases are referable to "pathology due to genome instability".

Adult↗
PubMed1977

[Osteopetrosis (Albers-Schoenberg disease). III. Current nosographic aspects and relations to other condensing osteopathies].

A malignant paediatric variety and an adult variety of Albers-Schönberg disease are normally distinguished. On the basis of recent findings and personal observation it would appear advisable to accept two different courses of Albers-Schönberg disease in adults: one resembling the malignant infant form and the other with slow, practically asymptomatic (apart, obviously, from the skeletal lesions) course allowing for prolonged survival. This classification is of considerable practical importance for prognosis and therapeutic purposes. Other hereditary-familial and constitutional condensing osteopathy pictures exist that present radiological stigmata similar to those seen in Albers-Schönberg disease. The interest of the relations between A-S disease and certain of these condensing osteopathic conditions is obvious.

Diagnosis, Differential↗
PubMed1977

[Osteopetrosis ( Albers-Schönberg disease ). II. Personal contribution].

Five cases of marble bones in two families living near Brescia are presented. Case 1 (42-yr-old female) was a typical malignant form with deep and extensive eburnation, many pathological fractures, concomitant osteitis and the formation of many fistulae, massive spleen enlargement and infarct, and marked anaemia with clear signs of extra-medullary haemopoiesis. The patient died 5 yr after her first admission. Two of her brothers had had an identical, fatal form. Case 4 (28-yr-old male) was much the same: virtually general osteosclerosis despite the difference in age, marked spleen enlargement, a history of fractures, serious anaemia and extramedullary haemopoiesis. Benign pictures were seen in cases 2 and 3 (73- and 68-yr-old females). In case 5 (25-yr-old male), typical bone condensation was the only significant pathological sign. It is suggested that this, too, may be seen as a "benign" form.

Adult↗
PubMed1977

[Osteopetrosis (Albers-Schönberg disease). I. Clinico-radiological and pathogenetic aspects].

An account is given of recent clinical and radiological findings with respect to Albers-Schönberg disease. Reference is made to its hereditary transmission modality and clinical picture (skeletal lesions and haematological distress), and to its radiological aspects. Suggestions are made with respect to the aetiopathogenesis of disease. Stress is laid on the as yet undefined mechanism of "genotypical condensing dysplasia". The relation between osteopetrosis and thyrocalcitonin is discussed, though no definite conclusions could be drawn from the hormone levels determined in a personal series.

Adult↗
PubMed1977

[Gilbert's jaundice. Current clinico-nosographic, physiopathological and therapeutic aspects. III. Therapeutic action of enzyme inductors with reference to unconjugated bilirubin icterus].

The value of phenobarbital in the treatment of free bilirubin icterus is demonstrated by a series of clinical experiments in which the drug was administered to patients with bilirubinaemia, even at high levels, the situation being brought back to normal within about two weeks. The percentage excreted with the urine in a conjugated form of various drugs proved higher in subjects treated with phenobarbital than in controls, thus proving that the drug acts as an enzymic inductor. Moreover it is ineffective in patients genetically lacking in the capacity to synthesize glycuronyltransferase. The induction of this latter enzyme, however, does not exhaust the effects of the barbiturate for it has been shown that phenobarbital is capable of speeding up the disappearance of exogenous bilirubin from the plasma in animals, of stimulating bile flow and increasing uptake of the pigment by the liver. The increase in bile flow is of the order of 30% and takes place by way of a modification in the flow fraction independent of bile salts. It would also appear that the drug is capable of increasing the activity of 7-alpha-hydroxylase, an enzyme that represents the rate limiting step in the synthesis of biliary salts. Other drugs commonly used in the treatment of free bilirubin icterus such as ethanol, rifampicin and uridindiphosphoglucose are considered. Finally the case of a female patient who from birth had presented persistent free bilirubin icterus of about 8 mg% is reported. After 14 days treatment with phenobarbital (100 mg X 2) blood levels of the pigment had returned to normal.

Adult↗
PubMed1976

[Gilbert's jaundice. Current clinico-nosographical, physiopathological and therapeutic aspects. Note II. The phenomenon of enzyme induction at the level of the liver].

After a brief survey of the various modifications that may be encountered by drugs through the work of systems that detoxify the liver cell, attention is given to the problem of enzymic induction. The latter is the result of derepression of a gene that codes for a given enzyme; at the molecular level, derepression takes place when the substrate, by changing the tertiary structure of the repressor, brings about its detachment from DNA: in this way, RNA-polymerase is made capable of synthesising the corresponding mRNA. The inducing activity of phenobarbitone, a drug employed classically in the management of Gilbertian forms, must be substantially attributed to an increase in the synthesis of microsomial proteins, as shown by studies with labelled amino acids.

Amino Acids↗
PubMed1976

[Gilbert's jaundice. Current clinico-nosographic physiopathological and therapeutical aspects. I. Recent findings in Gilbert's syndrome].

Gilbert's icterus is a term used to cover certain forms of free bilirubin hyperbilirubinaemia which occur without any clear signs of hyperhaemolysis and are thus based on a fundamental defect in bilirubin liver cell clearance. Speculatively, this defect may be considered as being located at the level of any one of the steps along the metabolic route of the pigment, between the vascular pole of the liver cell and the microsomes. The incidence of these forms is calculated at about 4-6% of the population, while study of its familial distribution would suggest its inclusion among genetically conditioned metabolic disturbances. Investigations of various groups of patients suggest heredity of poorly penetrating, incomplete expressivity dominant autosomic type. As for pathogenesis, analysis of the formation of glycuronide bilirubin on the part of liver microsomes has shown a frequent reduction in glycuronyltransferase activity in patients with Gilbert's icterus; on the other hand, separation of the two carrier proteins y and z, and kinetic studies with free radiobilirubin, suggest that in certain of these subjects there is an alteration in the liver cell's capacity to take up and hold bilirubin removed from the blood. On the basis of such data, Gilbert's icteruses have been traditionally subdivided into two types: the first, with slight bilirubinaemia, due to an uptaking defect, the second, with higher bilirubin, due to a reduction in glycuronide conjugation. From the morphological viewpoint, the optical microscope does not reveal any outstanding elements in the livers of Gilbert patients; some workers using the electronic microscope have insisted on the not infrequent presence of damage to the vascular pole of the liver cell, which would fall in with the hypothesis of a membrane pathology as the underlying factor in one type of Gilbert's icterus. Numerous granules with lysosome characteristics have also been seen at the biliary pole of the liver cell. Whether these are the cause of the disease or, as would appear more likely, they are only an epiphenomenon secondary to the accumulation of a non-metabolized product of the liver, is still under discussion. Theoretically, therefore, two groups can be distinguished for free bilirubin icteruses of hepatic pathogenesis and thus not only for Gilbert's icterus; those due to a membrane or y and z carrier pathology, and those with microsome pathology due to partial glycuronyltransferase deficiency. The most recent tendency is thus to unify under the common label of a glycuronyltransferase deficiency the type II of Gilbert's icterus and the Crigler-Najjar disease, even though gene transmission modalities differ. Some workers thus suggest two types of Crigler-Najjar disease: type I, the classical type, due to absolute glycuronyltransferase deficiency and type II due to a relative deficiency, taking in the II form of Gilbert's icterus...

Adolescent↗
PubMed1976

[Takayasu-Onishi arteritis. II. Relations of Takayasu-Onishi arteritis with other non-specific arteritides].

Takayasu-Onishi arteritis (T.O.) is similar to Hutchison-Horton arteritis (H.H.) on histological, clinical, laboratory, and pathogenetic grounds. Both probably depend on immunitary dysreactivity, their different clinical expression being attributable to differences in the district involved and the age of the subject. Both are preceded or accompanied by rheumatism. An interesting relation can be made out between temporal arteritis and "rheumatic polymyalgia" or, more aptly, "rhizomelic polymyalgia" (Ballabio, 1975). The latter (of rheumatic origin) may accompany arteritis - Hamrin, indeed, has suggested their unification in the description "arteritic polymyalgia". It is uncertain whether vasculopathy in the course of collagen disease, rheumatic arteritis, and polyarteritis nodosa can be identified with T.O., even though a common immunological basis can be made out. The difference between T.O. and thromboangiitis obliterans, on the other hand, is quite clear at the present time.

Aortic Arch Syndromes↗
PubMed1976

[Takayasu-Onishi arteritis. Current etiopathogenetic aspects].

By Takayasu-Onishi's arteritis is meant an arteritic process with unknown aetiology which electively affects young women, seemingly of prevalently Asiatic stock. The disease concerns almost exclusively large elastic arteries and presents clinically with early preocclusive symptomatology followed, after a varying period, by a picture of obstructive angiopathy. Here, the most recent aetiopathogenetic findings are considered. The disease's predilection for the young female, together with certain clinical and experimental observations, suggest that a dysendocrine condition may have some pathogenetic responsibility, at least in a favourable sense; this responsibility is documented by the finding of high levels of oestrogenuria during the entire cycle in patients suffering from the disease. On the other hand, the angiopathy localization in the large elastic arteries and in certain segments of the aortic arch and epiaortic trunks means that the role of topographic moments whould not be underestimated. As regards infectious factors, the tubercular and streptococcic continue to be of great importance even today. Neither acts directly, however, but by way of an abnormal immunitary reaction which they seem able to trigger off. The infectious moment would thus appear to be related to the immunitary moment, and the latter would seem to play the part of perpetuating the pathological vascular involvement promoted by contact of the organism with the aetiological agent.

Adolescent↗
PubMed1975

[Adriamycin in chemotherapy of solid tumors].

From one to eight, 7 or more day spaced, three-day 0,5 mg/kg/day cycles of adriamycin (an anthracycline antiblastic very similar to daunomycin) were administered to 50 adults with various solid tumour forms. Toxic signs were constant and sometimes compelled abandonment. The main residual signs were leucopenia, alopecia and stomatitis. The drug also displayed a cardiotoxic effect, though this was less than observed with daunomycin. It is felt that the theoretical interest aroused in adriamycin as an inhibitor nucleic acid systhesis, together with its marked anti-neoplastic efficacy in the experimental animal, have been betrayed by this performance in the management of solid tumors.

Adult↗