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BACKGROUND: Cardiac tuberculosis is rare and usually manifests as tuberculous pericarditis. Involvement of other part of the heart is unusual and descriptions in the pediatric literature are confined to few case reports regarding mainly myocardial tuberculosis. CASE PRESENTATION: We describe a case of pulmonary miliary tuberculosis associated with intracardiac left atrial tuberculoma in an immunocompetent eleven-month-old infant successfully treated with surgery and antituberculous therapy. CONCLUSION: Although unusual, involvement of endocardium in disseminated tuberculosis should be kept in mind.

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A rare case of hypoplastic left heart syndrome associated with pulmonary stenosis detached prenatally is reported.

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OBJECTIVE: To evaluate the frequency and clinical spectrum of chickenpox complications among immunocompetent hospitalized children. PATIENTS AND METHODS: Multicentre retrospective study of clinical records of all patients consecutively hospitalized for varicella during the last decade, in ten tertiary care clinical centres of Pediatrics and Infectious Diseases, throughout Italy. RESULTS: Two hundred and nineteen out of 991 patients (22.1%) hospitalized for varicella suffered from a complicated disease (247 complicating events on the whole). Central nervous system (CNS) involvement was prevalent (104 episodes), followed by skin/soft tissue infections, lower respiratory tract involvement, and thrombocytopenia. A complicated disease was significantly associated with the male gender and an elevated incidence of varicella-zoster (VZ) virus infection acquired by household contacts. The involvement of lower respiratory airways and skin/soft tissues seemed to occur at an earlier age, compared with CNS and thrombocytopenia. Moreover, lower respiratory tract and skin/soft tissue infections occurred earlier during disease course than complications interesting the CNS and coagulation system. All subjects with complicated chickenpox showed a favourable outcome within 5-40 days, except two patients developing a lethal cardiomyopathy and Reye syndrome, respectively. Anyway, chickenpox complications led to a prolonged hospitalization, and needed further pharmacologic treatment in all evaluated cases. The administration of anti-VZ virus treatments (i.v. acyclovir or VZ immunoglobulins) did not modify significantly the course of disease in treated patients, when compared with untreated ones. CONCLUSIONS: Although chickenpox is considered a self-limiting, uncomplicated disease in immunocompetent children, when assessing hospitalized patients a considerable incidence and a broad spectrum of complications are observed, requiring prolonged admission and pharmacologic and supportive care. Even though a lethal outcome remains a rare occurrence, it may be of relevant concern when considering the overall incidence of chickenpox in the general population. The role of an early antiviral treatment in reducing the incidence and severity of varicella complications deserves further evaluation.

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Kawasaki Disease (KD) is an acute systemic vasculitic disorder of childhood of unknown etiology. Initially KD was thought to be a benign disease, but later on it became obvious that cardiac manifestations are present in about 25-30% of patients and lead to death in about 0.5-2% of them. An early diagnosis and treatment are important to avoid an unfavourable prognosis. In recent years, a number of publications described patients in which the diagnosis was delayed because they did not fulfil the required criteria of KD. These forms of KD are known as atypical or incomplete KD. The aim of this work is to describe two cases of atypical KD observed at the Department of Pediatrics, University of Pisa during the year 1992.

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The Munchausen syndrome by proxy, or Polle syndrome can be considered as a form of child abuse in which parents falsify or even produce a real pathology in their children submitting them to frequent hospitalizations and pointless medical examinations. The authors discuss two cases and emphasize the importance of an early identification of these cases to avoid psychical impairments that inevitably stem from this "new" form of child abuse.

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The authors describe a case of Williams syndrome-Coeliac Disease that they have observed at the age of three years and 10/12. There are few reports in the literature. We focus on the variability of clinical and biochemical aspects of Williams Disease and the necessity for an adequate gastroenterologic follow-up (anti-gliadine antibody and anti-endomisium antibody) in these patients with little growth in terms of weight and height and intestinal alterations present in superior measure in companion with the reported standards for the same syndrome.

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The Authors report two cases of acute osteomyelitis, in a newborn and in an infant. Bone and joint sepsis in the first years of life is rare. The metaphases of long bones are the most common sites of hematogenous osteomyelitis. The hip and the knee are especially at risk. The patients were treated with pharmacologic and orthopedic therapy. The Authors describe the clinical features, radiographic changes, the diagnosis and management of this disease.

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The authors report the case of a 13-year-old girl affected with urethritis, conjunctivitis, and oligoarticular arthritis. These symptoms represent the classical triad of Fiessinger-Leroy-Reiter's syndrome. The research for HIV was negative and the antigen HLA B27 was absent. The young patients has been treated by FANS and tetracycline. The authors reviewed the literature and analyses the epidemiology-cal, etiological and clinical problems of this disease.

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Carnitine is a very important co-factor for the metabolism of fatty acids, because it is the necessary carrier for the passage of acyl groups inside the mitochondria, where beta-oxidation takes place. In the human body the total pool of carnitine is made by two fractions, one being endogenous and the second exogenous. The absorption of exogenous carnitine takes place mainly at duodenal-jejunal level by an active transport mechanism based on amino acid carrier. Plasma L-carnitine concentrations have been measured in 66 coeliac patients (mean age: 7 years and 4 months). Thirty three of them were on a free diet and the other 33 were on a gluten-free diet for at last six month. In 10 patients we studied plasma L-carnitine levels both on a free diet and on a gluten-free diet. As controls we examined 33 healthy children comparable for age. Plasma L-carnitine concentrations have been measured by a spectrophotometrical method according to Marquis and Fritz's technique and subsequently modified by Pearson and Seccombe. In the 66 coeliac patients the mean values of serum L-carnitine were significantly lower than those in the controls (p < 0.001). The levels were significantly lower in patients a free diet with respect to those on gluten-free diet (p < 0.01). The 10 subjects who were examined both on free diet and on gluten-free diet showed an increase of plasma concentrations in the latter condition.(ABSTRACT TRUNCATED AT 250 WORDS)

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Although the definitive cause of infantile hypertrophic pyloric stenosis is unknown, it's probable that several predisposing risk factors would be associated with the condition. We analysed some perinatal factors in relation to the incidence of infantile hypertrophic pyloric stenosis among children observed during the period 1970-90. We examined 61 infants with surgically confirmed hypertrophic pyloric stenosis and, as controls, 61 healty children comparable for age. In every child we studied: sex, birth rank, pregnancy and delivery, birthweight, parental age, type of feeding, familial history of hypertrophic pyloric stenosis and of atopy, seasonal variation in incidence, AB0 and Rh blood phenotypes. In the 61 infants with hypertrophic pyloric stenosis, the incidence of three factors (male sex, primogeniture and feeding with artificial milk) was significantly higher than that in the controls. We conclude that infantile hypertrophic pyloric stenosis probably has a particular genetic basis, but perinatal factors are responsible for the rising of the condition. However the true aetiology remains to be elucidated.

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The aim of the study was to assess the utility of intravenous integral molecule immunoglobulin treatment in Guillain-Barré syndrome. The etiopathogenesis of this syndrome is still unclear but it seems increasingly likely that immune phenomena are involved in the genesis of the neurological lesions. This would explain the efficacy of iv immunoglobulin treatment even if the effective mechanism of action can still only be hypothesised.

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Immune mechanisms have been invoked to explain coeliac disease and associations between this and other immunologically mediated diseases have been described. A direct relationship proposed is that coeliac disease may be associated with a range of autoimmune disease through the formation of immune complexes in the small intestine. It's more probable a common origin in an inherited predisposition to hypersensitive immuno responses to extrinsic antigens or auto-antigens. The association between coeliac disease and particular histocompatibility antigens was recognised.

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