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Biomedical subjects

V Carnelli

Publications and source records attributed to V Carnelli.

At least 91 records · Page 5Linked to original sources

[Preliminary data on the endocrinologic evaluation of thalassemic adolescents].

Endocrine disfunction are well known in young patients with thalassaemia major and are mainly due to iron overload. Gonadotrophin releasing hormone (GnRH) was administered to 13 boys aging more than 14 years and to 13 girls aging more than 13 years to assess pituitary function. Human Chorionic gonadotrophin (HCG) was also administered to 13 boys to evaluate their gonadal function. Most of the patients had evidence of pituitary hypofunction and in 4 boys there was evidence of gonadic failure. This study is mandatory for a correct therapeutic approach.

Adolescent↗

[The prevention of liver disease in the hemophilic child].

The modern replacement therapy of inherited bleeding disorders has proved to be a major advance in the management of haemophilic children. However, the haemophiliacs, early treated with commercial clotting factor concentrates obtained from large amounts of plasma, are exposed to blood borne viruses responsible for post-transfusion hepatitis (PTH) and for their possible harmful long-term sequelae. Infact high prevalence of infection with hepatitis B virus, non-A, non-B agents, delta agent has been documented among haemophilic children. In this study we analyze the measures of surveillance at present available in order to reduce the risk of PTH in young haemophilic patients. Among these measures of prevention we point out the magnitude of administrating hepatitis B vaccine to susceptible children and of using antihaemophilic factor heat-treated to reduce infectivity in those children who have never been treated and without signs of active viral infections.

Child↗

[Clinical evaluation of a new factor VIII concentrate in hemophilic children].

Studies of in vivo recovery, longevity and dose response of factor VIII following infusions of a new factor VIII concentrate (Koate, Cutter Laboratories) yielded results similar to those reported using other sources of factor VIII. Koate has been demonstrated to be clinically effective in classic hemophilia. This preparation was produced by refinements of the methods first described by Hershgold, Pool and Pappenhagen in 1966. By these procedures a high purity factor VIII concentrate is obtained being some 65 to 170 fold purified. This factor VIII concentrate was clinically evaluated in 11 hemophilic children (one of them with 7 Bethesda Units inhibitor) who received a total of 13 separate infusions. Biologic half-life values determined in two subjects resulted 12 and 9,5 hours comparing favorably with those previously reported. Initial 50% disappearance averaged 5,4 and 4,8 hours respectively. In vivo recovery of the infused factor VIII activity averaged 98 +/- 12,75%. Dose response measurements showed that 1 unit/Kg gave an in vivo increase in circulating activity of 1,97 +/- 0,93%.

Child↗

[Chronic lead poisoning in childhood: clinical experience].

Since 1978, 818 children living near an industrial source of pollution have been screened for lead poisoning. Biochemical evidences of increased lead absorption have been found in 114 cases and in 62 of them it was of such degree to guarantee further investigations. Those 62 children received a thorough neuropsychologic evaluation revealing a significant reduction of IQ, learning difficulties, poor concentration and behavioral abnormalities. We also documented an increased rate of abnormal mathafases and breaks of chromatides in children with increased lead absorption compared with age and sex matched controls.

Brain↗

[Sylvatic rabies: a pediatric problem?].

Rabies epizooty has been recently reported from several Italian provinces: Bolzano, Udine, Belluno, Brescia, sondrio, Varese, Como and Bergamo. The rate of spread of the infection through wild animals has been estimated in 50 Km/year. We have reviewed the fundamental characters or rabies virus, the pathogenesis and clinical manifestations of disease. Furthermore the fundamental guidelines of passive and active immunization will be stressed with spread emphasis to the pre-exposition active immunization in children-living in high risk areas.

Animals↗

[Treatment of thalassemia].

The management of Cooley's anemia today is based on: a careful transfusional therapy using international schedules with standardized parameters for evaluating its efficacy; prevention of iron overload improving the compliance for chelation therapy; blood transfusion with the optimal methods to prevent reactions mainly due to leukocytes; psychosocial and clinical approach of adolescent's problems.

Adolescent↗

[Infantile angiodysplasias: diagnosis and therapy].

Three cases of angiodysplasias are reported. There are some problems still open concerning diagnostic and therapeutic approach. In this paper we try to correlate localisation, time of onset, size, aspect, histological features, evolution, side effects and hemodynamic problems with diagnosis, prognosis and therapy of childhood angiodysplasias mainly concerning cavernous, intracranial, visceral and lymphatic angiomas.

Brain Diseases↗

[Comparative study of 3 different prophylactic programs in hemophiliac children].

Prophylaxis continues to be an area for discussion in the therapy of the hemophilic child. In fact, standard parameters are still not used in the evaluation of the efficacy of this treatment. At the Milan University Pediatric Clinic we have begun three different prophylactic programs with 52 children with ages ranging from 12 months to 14 years 7 months. We have evaluated the efficacy of these regimes comparing the data obtained in the 12 months following the start of the program with those obtained in the 12 months prior to it. Our data, even though they pertain to a limited number of children, show that continuous prophylaxis is truly efficacious if it is begun early before the onset of an arthopathy. Therapeutic prophylaxis however for the stabilizing of a target joint is usually better accepted by the children and their parents. The high cost of the prophylactic programs can nevertheless be justified by the better quality of life of the patients.

Adolescent↗

[Thermographic monitoring of the evolution and therapy of chronic synovitis and cysts in hemophiliacs].

The authors have studied the evolution of chronic synovitis and of cysts in hemophilic patients, through measurement of the articular circumference or of the cyst. To obtain this, they have used a folding rule and liquid cristal strips, that have the characteristic, that they change colours from braunish to orange, from greenish to bluish according to the thermic gradient revealed. The authors have studied 21 hemophilic patients, affected from chronic synovitis for a total of 23 articulations and 5 patients with cyst. All patients have undergone several controls reporting dates revealed at 2,6 and 12 months from beginning of disease. The authors conclude, underlining the usefulness of this simple methodic that permits: - a reliable valutation of the therapeutic (profilatic) scheme applied to every single patient and gives a prognostic precision; - distinguishing, in fact, the recovery from the clinical recovery of the synovitis in order to avoid the appearance of unpleasant relapses.

Adolescent↗

[Home care for the hemophilic child: experience of 4 training courses for the parents].

Much has already been written about training courses for home care of the hemophiliac held both in Italy and abroad. The advantages of home care are unquestionable. On one hand, the disorder becomes less hospital-dependent, and on the ofter, trough greater partecipation both the infusor and family become more responsible and competent on dealing with it. Hemophilia is a disease which puts the heaving burden on the pediatrics years. A specialized pediatric center is undoubtedly ideal for providing "global" assistance to the hemophilic child and his family so as to prevent any physical consequence of the disorder. There is a natural setting for training courses for home care for parents where they receive proper instruction with respect to problems that are often solely pediatric. The purpose of the course is not just to teach how to reconstitute and infuse freeze-dried concentrate, a practice which is, in itself, fairly easy, but also to give the parents through explanation, and a chance to meet with other parents to exchange ideas and experience.

Adult↗

[Preliminary data on the study of various immunologic parameters in hemophilic children].

Treatment of coagulation deficiencies with high dosages of the missing factor is a source of continuous diffusion of homologous proteins that could modify the normal immunological profile. We have performed immunologic studies on 24 children with ages ranging from 1 - 16 years with severe classical hemophilia and 1 child with von Willebrand's and on a control of 19 age-matched healthy children. Our preliminary data show no statistically significant alterations in the basic immunological profile between normal children and those with hemophilia. Nor were there any differences among the hemophilic patients on the various transfusional regimes. Considerations are made on the reasons for the discrepancies between our results and those on adults presented in the literature.

Adolescent↗

[Intra-familial transmission of HBV infection].

We investigated 63 household contacts of 29 children positive for serologic markers of hepatitis B virus, in order to assess the intrafamiliar spread of HBV infection. 40 relatives of 15 children affected by chronic hepatitis B (Group I) and 23 relatives of 14 patients affected by hemophilia (Group II) were studied. HBV markers (HBsAg, anti-HBs, anti-HBc, HBeAg, anti-HBe) were tested in all the subjects by RIA. Positive markers were found in 39 (62%) of the subjects; the combined prevalence for all HBV markers was 75% in the Group I, and 39% in the Group II. The higher prevalence rate in Group I can be explained by the possible previous infection of another member of the family, whereas in Group II the child is almost surely the first infected subject. Besides, subjects of Group I have probably been in contact with HBV infected children a longer time, compared to subjects in Group II. Our data suggest that household contacts of children with HBV infection are at high risk and should therefore be immunized against HBV.

Child↗

[Biliary atresia: pathogenetic considerations emerging from the histopathologic analysis of the liver parenchyma].

Nine patients had been surgically treated for extrahepatic biliary atresia from 1974 to 1982. The analysis of the hepatic specimens obtained during the surgical exploration revealed a wide spectrum of histopathologic lesions. In 3 patients we found severe Giant-cell transformation, while in the other 6 it was not possible to ascribe the histopathologic picture to a univocal pathogenesis. Comparing our results to the reports of other Authors we believe biliary atresia can not be explained by a single pathologic mechanism. The obstruction of the extrahepatic bile ducts might be the common morphologic denominator of different pathologic entities, from each of them it depends a different evolution of the hepatic lesions and a different survival after Kasai's operation.

Bile Ducts↗

["Follow-up milk": general principles and evaluation of the nutritional status of 100 subjects fed a liquid transitional formula].

Many authors have pointed that precocious weaning expose infants to serious risks as hypernutrition, obesity, adverse reactions to foods, hypernatremia, dental caries, emotional problems as anorexia or bulimia, so that actually weaning is delayed after 6th month of age. Going on with the "adapted" formula is a relative nonsense because "adapted" milks have low protein and calcium contents so that they are not adequate to cover estimated and advisable intakes of 4-6 month baby unless feeding unusual higher volumes. On the other side "fresh milk" can not be considered a nutritional "chance", owing its low values of EFA, iron, vitamins, getting worse when fresh milk is diluted. So, recently was born a new milk formula "the follow up milk", on covering nutritional requirement for infants after 4 months of age. Someone is still critical about a follow up milk, also if ESPGAN in 1981 has confirmed its value in the infant feeding. Our work dealed on physical and biochemical nutritional assessment of 100 infants fed a new "liquid follow up formula" (Transilat). Nutritional assessment was performed with the following parameters: daily changes in weight according Fomon standards, plasmatic iron, cholesterol, transferrin, calcium, total proteins, hemoglobin concentration; all data are related to literature values for age. Results show that infants fed (Transilat) are growing well; nutritional data from biochemical point of view discovered any form of minimal or sporadic malnutrition. The follow up milk is a good nutritional "chance" after 4th month of age, instead of fresh cow milk; some infant with clinical problem needing a delayed introduction of cow milk can benefit of follow up milk also in older ages.

Animals↗

[Reference values of urinary delta-aminolevulinic acid in children].

Delta-aminolaevulinic acid is well known to be an intermediate in the synthesis of haeme and small amounts are normally excreted in urine (ALA-U). An increased excretion of this substance occurs in porphyria and in lead poisoning. The urine specimens of 670 children (362 boys and 308 girls), aging from less than one to fifteen years and who had no evidence of abnormal exposure to lead, have been collected in order to find out the range of normal values of ALA-U expressed in mg/l for the different age and sex group. Our data can be usefull, together with zincprotoporphyrin (ZPP), for screening of anemias and for monitoring lead exposure.

Adolescent↗

[Idiopathic pulmonary hemosiderosis: report of a case with a favorable response to cyclophophamide therapy].

A 13-years old boy who presented a severe degree of iron-deficiency anemia and diffuse parenchimal infiltrates on the chest roentgenogram is reported. The clinical picture and the presence of hemosiderin laden macrophages in bronchial washing suggest Idiopathic Pulmonary Hemosiderosis (I.P.H.): open lung biopsy confirmed the diagnosis. Immunofluorescence studies showed no deposition of IgG, IgA, IgM and B1C in the lung. The most striking abnormality observed at electron microscopy was hemosiderin deposition in the alveolar-capillary basement membrane. One year after cyclophosphamide therapy was both in complete hematologic and pulmonary remission.

Adolescent↗