Pathogenesis of febrile convulsions (FC) is still unknown, suggested causes include the role of antidiuretic hormone (ADH). ADH is an endogenous antypyretic and his excessive production of the consequent hyponatraemia may be the cause of FC in children with susceptibility to this type of seizure. Whereas, interleukin-1 (IL1) is a pyrogenic substances and is involved in the release of AVP. Helminen et al. have reported a significantly higher production of IL1 in culture of peripheral blood monocytes stimulated with lipopolysaccharide (LPS) of children with FC than in the others with fever but without convulsions. More recently Lahat et al. have compared plasma and cerebrospinal fluid ILI levels of children with FC with those of children with fever but without convulsions, but they did not find significant differences. The aims of this study were to determine the IL1 levels in vivo and in the supernatants of cultures of peripheral blood mononuclear cells (PBMC) stimulated or not with LPS in children with FC and in children with fever without FC and to evaluate the influence of ADH and diazepam (DZ) on IL1 production. Blood samples for PBMC cultures were obtained from 11 children with FC on the hospital admission, (group 1) and after 48 hours from treatment with DZ (group 2). The production of IL1 was measured by RIA in the supernatants of the PBMC stimulated with LPS, LPS + DDAVP (synthetic vasopressin), LPS + DZ and in vivo in plasma samples. The control groups were constituted by 9 children with fever and without convulsions (group 3), 4 of them were studied at the end of fever too (group 4), and finally by 9 children in good health (group 5). No significant differences were observed. These results do not support the hypothesis that increased production of IL1 is involved in the pathogenesis of FC in children.

Child, Preschool↗

PubMedDate not supplied

Interleukin 2 proliferative response by cord blood mononuclear cells of term and preterm neonates.

The results of previous studies on the proliferative response of resting cord blood mononuclear cells (CBMC) of term neonates to human recombinant interleukin 2 (hrIL-2) are contrasting. Some authors have reported a good and others a poor response. In our study we have obtained a significant reactivity, compared with the response of resting peripheral blood mononuclear cells (PBMC) of adult subjects, of resting CBMC to varying quantities of hrIL-2 in the absence of any known mitogenic or antigenic stimuli. Most responding cells was CD3 positive. Both CD4-positive and CD8-positive cells responded. However, we observed a more marked increase of the percentage of the CD4-positive T cells and a clear reduction of the percentage of the CD21-positive cells (B-lymphocytes) testing CBMC rather than PBMC of the adult subjects. The percentage of the NK cells was reduced in both the categories of subjects. Moreover, we have examined the reactivity to hrIL-2 of CBMC of preterm neonates. The results showed that this response is low. The peculiar level and kinetic of CBMC proliferative response to hrIL-2 are discussed.

Adult↗

PubMedDate not supplied

The clinical spectrum and treatment options of macrophage activation syndrome in the pediatric age.

Macrophage activation syndrome is a rare and potentially fatal complication of many childhood pathological settings, most frequently reported in systemic onset-juvenile idiopathic arthritis. The disruption of the macrophage-lymphocyte interaction leads to uncontrolled proliferation of highly activated macrophages and T lymphocytes. The syndrome comprises a heterogeneous group of disorders featuring sepsis-like characteristics typically combined with impaired function of natural killer cells and cytotoxic T-cells, haemophagocytosis and hypercytokinemia, often resulting in fatal multiple organ failure. The clinical picture shows high grade fever, hepatosplenomegaly, pancytopenia, lymphoadenopathy, central nervous system involvement and consumptive coagulopathy. Macrophage activation syndrome is associated with high mortality: even though diagnostic criteria have been proposed, definite diagnosis can be a challenge for clinicians, especially in early phases. There is no standardized therapeutic protocol for macrophage activation syndrome, but it is widely recognized that aggressive treatment strategies might strongly influence prognosis. First line-therapy is usually represented by parenteral administration of high dose-corticosteroids, whilst cyclosporine is added in the steroid-resistant cases. In this paper we provide clinical clues and summarize the most recent studies about pathophysiology and management suggestions for macrophage activation syndrome.

Adolescent↗

PubMedDate not supplied

Rheumatic heart disease in children: from clinical assessment to therapeutical management.

Rheumatic heart disease is still a relevant problem in children, adolescents and young adults. Molecular mimicry between streptococcal and human proteins has been proposed as the triggering factor leading to autoimmunity and tissue damage in rheumatic heart disease. Despite the widespread application of Jones' criteria, carditis is either underdiagnosed or overdiagnosed. Endocarditis leading to mitral and/or aortic regurgitation influences morbidity and mortality of rheumatic heart disease, whilst myocarditis and pericarditis are less significant in determining adverse outcomes in the long-term. Strategy available for disease control remains mainly secondary prophylaxis with the long-acting penicillin G-benzathine.

Adolescent↗

PubMedDate not supplied

The multi-face expression of familial Mediterranean fever in the child.

Familial Mediterranean fever (FMF) is characterized by recurrent self-limiting flares of fever in the absence of pathogens, autoantibodies or antigen specific T cells and is inherited as an autosomal recessive trait probably deriving from common ancestors of Armenian, Jew, Turk and Arab origin. The underlying pathogenetic mechanisms of FMF have not been fully interpreted, but mutations in the gene MEFV encoding pyrin, a natural repressor of proinflammatory molecules, result in uncontrolled relapsing systemic inflammation, increased leukocyte migration to serosal membranes or joints and inappropriate response to inflammatory stimuli. FMF heterogeneous phenotypic expression could originate both from allelic heterogeneity or from the existence of modulating genes. Proper diagnosis of FMF is needed to begin both specific clinical management and treatment based on continuous prophylactic administration of colchicine, preventing flares or at least the onset of amyloidosis.

Amyloidosis↗

PubMedDate not supplied

The pharmacologic basis of treatment with colchicine in children with familial Mediterranean fever.

Familial Mediterranean fever (FMF) is the prototype of auto-inflammatory disorders and is ethnically restricted to people living in the Mediterranean basin and Middle-East. Pyrin, the protein product of the FMF gene, expressed in myeloid cells and fibroblasts, interacts with the cytoskeletal machinery and may modulate leukocyte effector functions. At present colchicine, an alkaloid with antimitotic activity interfering with microtubule formation, which has been used to alleviate acute gout, is the only available drug for patients with FMF to prevent both acute attacks and long-term complications such as amyloidosis. The anti-inflammatory effect of colchicine may be mediated not only through direct interaction with microtubules, but also through changes at the transcriptional level influencing cell cycle regulation and leukocyte migration. Gastrointestinal side effects may occur early and are the most frequent manifestations of colchicine toxicity in children, whilst multiple organ failure is very rarely reported as overdosage expression.

Amyloidosis↗

PubMedDate not supplied

Focus on juvenile idiopathic arthritis according to the 2001 Edmonton revised classification from the International League of Associations for Rheumatology: an Italian experience.

OBJECTIVE: To classify a cohort of Italian patients categorized as affected with juvenile idiopathic arthritis (JIA) according to the revised 2001 Edmonton International League of Associations for Rheumatology (ILAR) criteria. METHODS: Eighty-five patients with JIA firstly framed depending on traditional criteria during the last ten years were reallocated according to the JIA revised criteria proposed in 2001 by ILAR in Edmonton. RESULTS: The revision consented to define the following distribution of patients: 28.2% systemic, 55.3% oligoarticular and 11.8% polyarticular forms; only one child was defined as having psoriatic arthritis, one child with enthesitis-associated arthritis and two with the undifferentiated form of JIA. DISCUSSION: The 97.6% of the recruited patients were strictly classified according to the Edmonton ILAR criteria, demonstrating a very low number of patients whose arthritis could not be assigned to any JIA category due to unfulfillment of the required criteria.

Arthritis, Juvenile↗

PubMedDate not supplied

[Early diagnosis of sepsis in the preterm neonate].

The usefulness of the neutrophil blood cell count, the ratio of band forms to total neutrophils, the platelet count, the quantitative determination of serum IgM, C-reactive protein, alpha-1-acid glycoprotein and haptoglobin for the early identification of the serious neonatal infections was evaluated in 70 preterm newborns: 15 with sepsis, 2 with serious infections, 53 without infections. None of these tests has proved sensitive and predictive enough to be used as a single measure. The combination of 2 or more of them had improve the sensitivity (76.4%) and the predictive value of negative test (91.6%). The authors suggest that the greatest potential value of the tests is to exclude infections, with a more than 90% probability, if they are negative.

Bacterial Infections↗

PubMedDate not supplied

[Neonatal sepsis and perinatal risk factors].

Numerous studies have identified perinatal risk factors of neonatal sepsis. Some authors have attempted to develop a score system and have shown that babies with septicemia usually had a significantly higher score than healthy newborns and infants with other diseases. The aim of our study is to verify the validity of a scoring method based on the following items: maternal disease; e.g. diabetes, severe toxemia, infection; rupture of the membranes more than 24 hours before the delivery; foul-smelling amniotic fluid; complicated delivery; Apgar score less than 7; umbilical catheterization; respiratory distress and other neonatal diagnoses leading to operative procedures. We have evaluated four groups of babies, full-term AGA and SGA and preterm AGA and SGA, 84 with septicemia, 105 with other diseases and 210 healthy newborn infants. None of the perinatal risk factors or neonatal diseases was sufficiently predictive of neonatal septicemia. Only the incidence of umbilical catheterization was significantly higher (p less than 0.01) in preterm AGA (37.1%) and SGA (64.7%) babies with septicemia than in preterm healthy AGA (2.8%) and SGA (7.7%) babies; on the contrary, no statistical differences were found between preterm AGA (37.1%) and SGA (64.7%) infants with septicemia and preterm AGA (42.8%) and SGA (66.6%) infants with other neonatal diseases. A score of 1 was assigned for each of the considered items. In the full-term infants a score of 1 or less was found in 100% of the healthy infants. A score of 2-3 was found in 26% of the septicemic infants and in 42% of the infants with other diseases.(ABSTRACT TRUNCATED AT 250 WORDS)

Birth Weight↗

PubMedDate not supplied

[Neonatal sepsis caused by coagulase-negative staphylococci].

All cases of neonatal bacteremia occurring at Neonatal Department of Pediatric Clinic, Catholic University of Rome, from January 1976 to December 1983 were examined retrospectively. Twenty-seven (30%) newborn infants with positive blood cultures for coagulase-negative staphylococcus were identified. Seven (25.9%) of the 27 infants were born at term, 4 AGA and 3 SGA; mean birth weight was 2,804 gm (range 2,280-3,670). All of these neonates had clinical evidence and laboratory signs of sepsis, and one had the cerebrospinal-fluid culture positive for coagulase-negative staphylococcus. In the remaining 20 infants (74.1%) the mean birth weight was 1,445 gm (range 810 - 2,400) and mean gestational age was 32 weeks (range 27 - 36). In 15 of the 20 preterm infants clinical signs of septicemia were associated with positive blood culture, and sixty percent of these had received an umbilical artery catheter. An half of coagulase-negative staphylococci isolated from our neonatal sepsis were DNAse-positive and/or phosphatase-positive and/or mannitol-positive. Two full-term infants, one with Down syndrome and one with cardiac malformation, died at 9 days and at 2 weeks of age, respectively. Three of 15 preterm infants with coagulas-negative staphylococcal septicemia died; deaths were among infants of very low birth weights and immature gestations who had severe respiratory syndrome. These data show that coagulase-negative staphylococcus can be important cause of septicemia in patients with compromised host defenses as newborn infants, and especially in the premature babies receiving invasive procedures.

Female↗

PubMedDate not supplied

[Rh isoimmunization: experience with and critical analysis of recent diagnostic and therapeutic criteria].

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Adult↗