[Chronic lead poisoning in childhood: clinical experience]. / L'intossicazione cronica da piombo in età pediatrica: esperienza clinica.

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.

SCE analysis in children exposed to lead emission from a smelting plant.

The frequency and location of SCE were evaluated in lymphocytes from peripheral blood cultures of 19 children living in a widely contaminated area and showing an increased absorption of lead. In the comparison between the exposed children and a control group living in an uncontaminated area, no significant differences were found in the mean values of SCE frequencies. The distributions of the SCE between chromosomes were proportional to chromosome length in both groups.

[The prevention of liver disease in the hemophilic child]. / La prevenzione dell'epatopatia nel bambino emofilico.

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.

[Average number of bleeding episodes in hemophilic children of different ages in "on demand" therapy]. / Incidenza media di episodi emorragici nelle varie età del bambino emofilico in terapia "on demand".

We have studied the number of important bleeds in our patients with respect to the age group in order to find criteria for putting the hemophilic child on an effective continuous prophylactic regime as early as possible. The bleeding sites are shown in the Tables. 48 children with severe hemophilia were studied. We observed an increase in bleeds with an increase in age. This increase in bleeds and their recurrence in preferential sites in a high percentage (48%) of these children indicate a higher exposure to trauma in part, and a gradual incipience of an arthro-miopathy as well. To avoid this occurring we propose beginning prophylactic treatment as early as possible.

[Home care for the hemophilic child: experience of 4 training courses for the parents]. / Trattamento domiciliare del bambino emofilico: esperienza di quattro corsi di addestramento per i genitori.

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.

[Comparative study of 3 different prophylactic programs in hemophiliac children]. / Studio comparativo su 3 differenti programmi di profilassi in bambini emofilici.

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.

[Preliminary data on the study of various immunologic parameters in hemophilic children]. / Dati preliminari sullo studio di alcuni parametri immunologici in bambini emofilici.

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.

[Hemophilia: evaluation of the nutritional status and growth in childhood]. / Emofilia: valutazione dello stato nutrizionale e della crescita in età pediatrica.

Haemophilia is a bleeding disorder characterized by decreased activity of the circulating antihemophilic factor, with different degrees of severity. The prognosis of the disease for an useful normal life is good, providing an adeguate follow-up. We think that the auxological determinants of growth and nutritional status must enter as points of a multicentre follow-up sheet of haemophilia in the pediatric age. We have tried such an approach in 13 patients; preliminary results have shown that all patients are growing well with some degrees of discrete malnutrition (arm circumference less than -1.6 SD). From costitutional point of view, haemophilic boys show a discrete "lean" body pattern (W/H2 less than or equal to O SD(with their trunk longer than legs. Data from other studies are wellcome for confirming our first impressions.

[Thermographic monitoring of the evolution and therapy of chronic synovitis and cysts in hemophiliacs]. / Monitoraggio termografico dell'evoluzione e della terapia nella sinovite cronica e nella ciste degli emofili.

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.

[Evaluation of the nutritional status and growth of children with chronic HBsAg-positive hepatitis]. / Valutazione dello stato nutrizionale e dell'accrescimento in bambini con epatite cronica HBsAg positiva.

Chronic hepatitis in children seems to have a better prognosis than in adults, with a discrete trend to spontaneous remission. Actually biopsy and liver enzymes are mandatory for a correct follow up of the disease, while few authors are interested in the assessment of growth and nutritional status. We think that an auxological approach is one of the main points in every chronic disease of the pediatric age, chiefly when some anabolic step might be affected. Our first result in a cross sectional study of growth and nutritional status in a selected group of untreated HBsAg+ chronic hepatitis children are as follows. According to enzyme values in the range of 16-171 UI/1 SGOT all patients are growing very well with a good-discrete nutritional status. Though anthropometric proteic nutritional status (muscle circumference) was in the range of normal distribution, we were able to show a moderate correlation between SGOT and muscle circumference (r = -0,50). Our impression is that some patients with a worse hepatic damage can be exposed at risk of proteic malnutrition which is a negative element in the prognosis of every chronic disease. Anthropometric auxology can detect degrees of proteic malnutrition, and can follow its development. So we recommend routine auxological assessment in all pediatric patients with chronic hepatitis.

["Follow-up milk": general principles and evaluation of the nutritional status of 100 subjects fed a liquid transitional formula]. / Latti di seguito: principi generali e valutazione dello stato nutrizionale in 100 soggetti alimentati con una formula di transizione liquida.

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.

[Idiopathic pulmonary hemosiderosis: report of a case with a favorable response to cyclophophamide therapy]. / Emosiderosi polmonare idiopatica: descrizione di un caso clinico con favorevole risposta alla terapia con ciclofosfamide.

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.