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Biomedical subjects

V Carnelli

Publications and source records attributed to V Carnelli.

At least 73 records · Page 4Linked to original sources
PubMedDate not supplied

Leukocyte depletion of red cell units at the bedside by transfusion through a new filter.

Standard packed red cell (PRC) units can be depleted of leukocytes and platelets if they are transfused through a blood administration set in which the usual 170-mu filter has been replaced by a leukocyte removal filter (Sepacell R-500). During a 6-month period, 1550 PRC units were transfused through this filter in 611 transfusions to 80 multitransfused patients with thalassemia who had had a patient reaction rate (PRR) of 63 percent and a transfusion reaction rate (TRR) of 13 percent when given standard PRC or buffy-coat-depleted PRC. When given filtered PRC, PRR and TRR became 3.7 percent and 0.5 percent, respectively. The effectiveness of the filter was also evaluated in vitro. By filtering 2 standard PRC units through the same filter, median values (and ranges) for red cell recovery and for residual leukocytes and platelets were 87 percent (83-92), 6.1 X 10(6) (0-100), and 2.7 X 10(9) (0.6-9.7), respectively. Although refinements are needed to improve standardization of the filter and to increase red cell recovery (which is low when 1 unit is filtered through one filter) and blood administration rate, the ability to provide leukocyte-free red cells prepared at the bedside for virtually all recipients appears to be a realistic goal.

Blood↗
PubMedDate not supplied

Altered cytokine and acute phase response protein levels in the blood of children with Downs syndrome: relationship with dementia of Alzheimer's type.

Downs syndrome (DS) subjects are at high risk of developing Alzheimer's disease (AD). Patients with AD often show altered levels of some immune molecules in their peripheral blood which correlate with cognitive impairment. However, whether the altered peripheral immune phenotype is a late and secondary phenomenon associated with dementia or an early impairment linked to mechanisms controlling neurodegeneration of the central nervous system (CNS) is still an unanswered question. Here we studied immune molecules in the blood of non demented children with DS to investigate whether altered peripheral immune phenotype could be present in these subjects without dementia, many years before the presentation of clinical signs of cognitive deterioration. Plasma levels of interleukin-6 (IL-6) and soluble IL-6 receptor (sIL-6R) were significantly higher in DS than in control children. Plasma levels of soluble intercellular adhesion molecule-3 (sICAM-3), soluble vascular cell adhesion molecule-1 (sVCAM-1) and C reactive protein (CRP) were also increased in DS. The increase of IL-6 and CRP from DS children was similar to that found in elderly patients with clinical AD. Peripheral altered immune phenotype in healthy young subjects with DS might be an early sign of CNS alterations leading many years later to cognitive deterioration and dementia.

Acute-Phase Proteins↗
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[Cardiorespiratory monitoring in hospital and at home].

Apnoea become a medical problem when associated with a symptomatology cohort characterized by skin colour modifications, muscular tone modifications and consciousness. This syndrome named ALTE, from the acronym Apparent Life Threatening Event, well describes the near death sensation in the witnesses. Only 60% of this events may be revealed in his etiopathogenesis, also even a strong diagnostic protocol is applied; the remaining part, called idiopathic represent a open doubt and stressing factor for the physicians. ALTE may be analyzed in his rising and resolving ways, throughout a complete and accurate report of the history and throughout his association with other several symptoms eventually associated, able to leading at diagnosis. The child must be investigated about his metabolic status near the crisis, particularly for each disease life threatening as cardiac arrhythmias, electrolytes alterations and hide or beginning infections. The use of instruments for domestic surveillance is based on old observations, denied by a lot of studies, there is an high relation between ALTE and SIDS. Really the monitoring has different rules in the management of child with ALTE: it is a useful tool to evaluate in continuous child's life parameters in order to be able to make a quick intervention in case of life threatening alterations for child. It also represents a diagnostic and prognostic way because it allows to evaluate respiratory and cardiac patterns and their modifications time related. There are some side effects as anxiety elicited in parents, cause of high frequency in false alarms. This allows the need of a strict relation between a SIDS Center and the family in order to increase the parents compliance.

Apnea↗
PubMedDate not supplied

[Analysis of risk factors for SIDS: experience at a referral center].

The aim of this research is to value, with a questionnary for the families, epidemiologic aspects in 20 children died for SIDS. The risk factors we have considered regard: ambiental condition, pregnancy, neonatal and post-neonatal period, and modality of death. The result is that sleeping position, child's age and month in which the death happens, are still the most significant risk factors for SIDS.

Female↗
PubMedDate not supplied

[Characteristics of 86 infants' cluster with A.L.T.E. (Apparent Life-Threatening Event): similarities and differences versus main risk factors for SIDS].

In this study we have gathered 86 ALTE infants' characteristics about physiological, familiar, remote and near pathological case history, modality of apnoea episode's presentation, environmental and behavioural conditions of babies and we have compared these ones with main risk factors for SIDS to find possible similarities or differences. Many of the already known differences or similarities between ALTE and SIDS have been confirmed by our data and new ones have been observed. So we can say that the association between ALTE and SIDS remains contrasting because beside undiscussed factors of association there are other ones which differentiate the two Syndromes.

Female↗
PubMedDate not supplied

[CVS-cardiac thrombosis related in a child with an intracranial germinoma].

We report a case of a CVC infection with a grave cardiac thrombosis in a child afflicted with intracranial functioning germinoma. During the second chemotherapeutic cycle we found a positive blood culture and a partial CVC occlusion; echocardiography showed a 2 cm diameter interatrial thrombus. After removal of the CVC a voluminous thrombus in the right atrium persisted. The profibrinolytic factor's concentration was normal, and so we preferred to use aspirin, the most common antiplatelet agent, combined with antibiotic and antimycotis therapy. This treatment allowed us to observe the disappearance of the thrombus by 25 days.

Brain Neoplasms↗
PubMedDate not supplied

[Pulmonary hemosiderosis induced by cow's milk proteins: a discussion of a clinical case].

Pulmonary hemosiderosis (PH), a rare pathology, usually occurring during infancy and childhood, is characterized by numerous and repeated intra-alveolar bleedings, interstitial iron build-up with consequent progressive fibrosis and severe anemia. PH can be basically divided into 3 different categories: Primitive or idiopathic, involving a primitive deficit of antioxidizing enzymes in the erythrocytes in genetically predisposed subjects; Secondary, subsequent to chronic pneumopathy or cardiopathy; Associated with various pathologies including collagenopathies, glomerulonephritis, myocardiopathies, diabetes, steatorrhea, tireotossicosis. A particular type of Pulmonary Hemosiderosis, associated with allergy to cow's milk, has been described for the first time by Heiner. We present in this paper our personal experience of a young patient suffering from pulmonary hemosiderosis induced by cow's milk protein.

Child↗
PubMedDate not supplied

[New therapeutic trends in thalassemia: oral chelating agents].

The future therapeutic strategy for decreasing iron overload in poly-transfused patients will include oral chelation. The product currently undergoing the most intensive experimentation is Ll (1,2-dimethyl-3-hydroxypyrid-4-one). We report here a short-term efficacy study carried out in 10 thalassemic transfusion-dependent pediatric volunteer patients, already closely followed in our Day Hospital, after having provided their informed consent. In our study Ll, when compared to Desferrioxamine, was clearly efficacious and safe.

Administration, Oral↗
PubMedDate not supplied

[Iron deficiency in children: which is the correct therapy?].

Iron is essential for human metabolism. Under normal circumstances its homeostasis is strictly kept by absorption and excretion through genitourinary, gastrointestinal tracts and skin losses. In several systemic disorders, dietary iron is insufficient to keep such a dynamic balance: development of iron deficiency may be due to increased requirements, decreased intestinal absorption, inadequate dietary uptake. Low birth weight newborns, children and adolescents are at increased risk for developing iron deficiency. Although clinical aspects may vary, hematochemical findings show a three-step gradual progression. In a first step iron deficiency is diagnosed by serum ferritin level which will be under 10-20/micrograms/ml showing a depletion of total body iron stores. In a second step progressing iron deficiency will be assessed by lowered serum iron and increased unsaturated serum transferrin, serum iron bound to transferrin and erythrocyte protoporphyrin IX. Scanty clinical signs are still available. In a third step while clinical findings show a complete features of iron deficiency anemia (weakness, fatigue, palpitations, etc.), laboratory findings show morphologic alterations in red cells (hypochromia and microcytemia), together with the aforementioned disorders in ferrokinetics. Iron deficiency anemia responds very effectively to treatment due a correct etiological diagnosis, crucial to a through therapy tending to first eliminating the causes of it. Prophylaxis against iron deficiency anemia is required in prematurely born and low birth weight infants because of doubled iron requirements. After the second month of life diet is supplemented with 2-4 mg/kg/die of ferrous iron orally along the first year of life.

Adolescent↗
PubMedDate not supplied

[Meningococcal sepsis and DIC in childhood: a report of a clinical case].

Disseminated intravascular coagulation (DIC) is a frequent complication of meningococcal sepsis in children. The clinical course variability, the severity of manifestations and the need of an early diagnosis for appropriate treatment, guides us to report a case of meningococcal sepsis and DIC. The patient, male, prematurely born, 11 months years old, presented himself with high fever of sudden onset, malaise, diarrhea, diffuse skin rash with abdominal petechiae, and no clinical evidence of meningitidis. Initial hematochemical findings, peripheral leukocytosis, quantitative and qualitative changes in plasma coagulation factors, liquoral hypocellularity together with the development of signs of meningeal irritation (stiff neck and back) were considered diagnostic clues for meningococcal sepsis associated with DIC. A gram-negative diplococcus was cultured from liquor. Primary goals of the treatment of this life-threatening clinical picture were the elimination of the bacterial component, the correction of clotting disorders and careful control of shock and metabolic acidosis often related with DIC. The patient then received a wide spectrum Cephalosporin, fresh frozen plasma, appropriate electrolyte solutions and eventually heparin, which led to a complete control and resolution of symptomatology.

Acidosis↗
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[Children with hemorrhagic diathesis: correct diagnostic and therapeutic approach].

Bleeding defects are of great interest in pediatrics since the prevalence of congenital forms and the early appearance of acquired ones. The pathology itself and the therapy indeed can often interfere with the growing-up patients. Bleeding defects have been identified with an heterogeneous group of clinical disease that differs from one another in etiology, pathogenesis, epidemiology and incidence in population. Bleeding diathesis is the common symptom: bleeding tendency may be mild, moderate or severe, localized or generalized, cutaneous or mucosal, superficial or deep. Bleeding disorders may be classified as a) defects in the primary haemostasis, which include quantitative and qualitative abnormalities of platelets and vascular disorders and b) defects in secondary haemostasis, which include intravascular disorders (blood coagulation). Careful history and clinical examination are essential in diagnosis of bleeding disorders. History of patient should be taken a) to differentiate acquired from congenital disease and to know the way of hereditary transmission (family history); b) to know exactly the disease's start and the mutual relation with former or accompanying disease; c) mutual relation with drugs token. Subsequently a careful physical examination should be done. A specific hemorrhagic diathesis has been seen with a deficiency of primary or secondary haemostasis. A deficient or late haemostatic plug in small vessels can cause superficial, interstitial bleeding that may be intracutaneous or intramucosal and is called purpura. In coagulation factor deficiency the haemostatic plug cannot be consolidated by fibrin: spontaneous hematomas, hemarthrosis and ecchymoses often occurs. The initial laboratory work up for screening patients with bleeding disorders should include first step tests to differentiate bleeding disorders for bone-marrow malignancies; from virus infections carrying screening of major viruses and from hepatic diseases. Second step laboratory examination includes a) platelet count or estimation of platelet number on blood smear; b) bleeding time to test small vessel integrity and platelet function; c) aPTT, PT, AP to measure clotting activity; d) fibrinogen determination. With this battery of screening test it is usually possible to determine the general area of the defect (abnormalities of platelets number or function or congenital defect of one or more clotting factors activity). Acute idiopathic thrombocytopenic purpura is the most common bleeding disorders in childhood. Usually no therapy may be required no matter platelet count. Patients with a significant hemorrhagic tendency are treated either with prednisone (2 mg/kg orally in divided daily doses) for a period of 2 weeks or with a 5 days course of special polyvalent intact immunoglobulin (400 mg/kg/die) for intravenous use.(ABSTRACT TRUNCATED AT 400 WORDS)

Adolescent↗
PubMedDate not supplied

[Treatment of hemophilia in children].

Thanks to recent developments and evolution in prenatal diagnosis and early onset within the first year of life, hemophilia may now be considered a pathology of primarily pediatric interest. The treatment of hemophilia in children has furthermore undergone a number of changes that include 2 main events in therapy that have served to modify the quality of life of the hemophiliac. The first of these events regards blood products and the prevention of viral infections, hepatitis and HIV transmission. Prevention is based on various factors which include: donor selection, immunization, product testing and heat treatment of blood products. The second extremely important aspect of treatment in hemophilia is the concept of global assistance, which includes: the treatment of the bleeding episode itself, and an ongoing psycho-social support system. In this paper we suggest some practical treatment schedules for the therapy of bleeding episodes in addition to examining the severe side effects of HIV and Hepatitis viruses. The message which our paper attempts to transmit is that the hemophilic child must be ideally assisted in an exclusively pediatric environment.

Acquired Immunodeficiency Syndrome↗
PubMedDate not supplied

[Audiologic evaluation of patients with thalassemic syndromes treated with desferrioxamine B].

Following various articles in literature which have appeared in the last 3 years regarding the neurotoxicity of desferrioxamine B we studied, from the auditory functionality point of view, 95 patients affected with transfusion-dependent thalassemic syndromes, under regular chelation treatment with desferrioxamine B. Our results lead us to conclude that at standard doses, between 40 and 60 mg/Kg/day, desferrioxamine B does not demonstrate a neurotoxicity enough to cause an organic deficit in the auditory sphere, while maintaining therapeutic efficacy.

Acoustic Impedance Tests↗
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[Long-term evaluation of immunosuppressive therapy in childhood idiopathic pulmonary hemosiderosis].

Idiopathic pulmonary hemosiderosis (IPM) is a rare disease of unknown etiology, whose diagnostic, prognostic and therapeutic approach is still open to discussion. In this paper the authors report a study regarding three cases of IPH initially detected in 13, 11 and 7 year-old children. The patients were treated with cyclophosphamide and prednisone according to different cycles depending on the clinical stage in the disease. All three patients are still alive after 10, 6 and 5 years since initial diagnosis. This therapeutic protocol therefore seems to be effective in preventing the progression of IPH and in maintaining the patients in an asymptomatic condition.

Adolescent↗