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Optimal treatment for Langerhans Cell Histiocytosis (LCH) has not yet been established. Preliminary reports suggest some effect of cyclosporine (CSA), both alone or in combination with steroids and/or vinblastine, in untreated cases. Twelve children (6 females and 6 males, age at diagnosis 3 months to 4 years) with biopsy proven, systemic LCH received oral CSA (12 mg/kg/day in two divided doses given daily) as a second-line therapy following chemotherapy including vinblastine and/or etoposide (10 cases) or steroid alone (one case); one child was not pretreated. A total of 16 CSA courses were administered to the 12 patients: 8 were completed, 4 were interrupted as unsuccessful, and 4 are still ongoing. CSA related toxicity consisted of hypertrichosis and transient hypertension and was never limiting. Treatment was associated with a clinical response in 8/12 patients: 3 had a complete response and are off therapy, and 5 had a partial response; disease reactivation following first favorable response required additional CSA courses in 3 patients. Four patients failed to respond to CSA: two died of progressive disease, while two had a favorable response to CSA + VP16. Favorable response to CSA was not related to CSA trough and peak levels and was usually observed during the first 2 weeks of CSA therapy. CSA is effective for treatment of LCH also in pretreated children with progressive disease including life-threatening organ dysfunction. Long-lasting complete remission may be achieved after 6 to 12 months of CSA therapy. When disease reactivation occurs after treatment withdrawal, a second course may be followed by favorable response. As minimal or no adverse effects are observed during or after prolonged CSA therapy, this may also be safely used in young patients.

An adult patient had a syndrome associating the features of juvenile dermatomyositis and cutaneous polyarteritis nodosa that followed a cyclic course from childhood; recurrences were always associated with a rise of serum antistreptococcal antibodies. Regions of homology between streptococcal type 5 M protein and skeletal myosin were found. These findings suggest that streptococcal infection, possibly through a molecular mimicry mechanism, played a role in the pathogenesis of the disease in our patient.

A case of Kaposi's sarcoma in a child with no serologic evidence of human immunodeficiency virus (HIV) infection is reported. A 7-year-old boy with Stage IV non-Hodgkin's lymphoma, after conventional chemotherapy, underwent autologous bone marrow transplantation (ABMT). Five months later he presented with supraclavicular mass and mediastinal enlargement. A bone marrow biopsy showed hypoplasia with no signs of the underlying disease, whereas the excised mass revealed a typical histologic pattern of Kaposi's sarcoma. The child is currently being treated with recombinant alpha-interferon (alpha-IFN) and regression of the disease has been achieved.

The social revolution of Enlightment in XVIII century re-built a new image of children as human beings well--cared--for, needy of consideration, love and respect. The first books devoted to children illnesses were printed in the same century. In XIX century the first hospitals devoted to the therapy of children diseases were organised as medical centers. The first one was founded in Paris in 1802, followed by the Great Ormond Street Hospital in London in 1852. The first italian paediatric hospital was the Ospedale Bambino Gesù in Rome, founded in 1869. A long way has been covered in the last years, both in hospitals and Universities, to adequate the medical paediatric assistance to children needs and to families' requirements. Following technical advancements in diagnosis and therapy, the hospitals have been re-tought according a style better fitting to children world. This is also the inspiration of the Study Group of the Italian Society of Paediatrics (Quality Improvement Group).

Starting from the conceptual premises of Garrod, who as long ago as 1902 spoke of “chemical individuality”, and of Burnet (1949), who recognized as self one's own molecular antigenic structures (as opposed to the antigenic “alien”: the non- self), the discovery and understanding of HLA antigens and of their extraordinarily individual and differentiated polymorphisms have gained universal recognition. Transplant medicine has now dramatically stressed, within man's knowledge of himself, the characteristic of his “biological uniqueness”. Today man, having become aware of being a biological antigenic-molecular individuality which is unique and different from that of all of his fellow men (except for monozygotic twins), can therefore easily consider himself a true “biological Ego”.

In a female with acute lymphoblastic leukemia, inadvertent doxorubicin administration intrathecally caused severe, life-threatening, acute encephalopathy with high-pressure hydrocephalus. Ventriculo-peritoneal shunting led to complete reversal of hydrocephalus with progressive disappearance of the acute encephalopathy.

We describe here an immunocompetent boy with fever, regional adenopathy, multifocal hepatosplenic granulomas, and high and increasing serum antibody titers for Bartonella henselae in whom diffuse bilateral reticulonodular pulmonary infiltrates developed in the absence of respiratory symptoms.

Detection of specific IgE by the radioallergosorbent test (RAST) which uses labelled antibody can be hampered by the presence of antibodies other than IgE but with the same specificity and may limit usefulness of the RAST for diagnosis of IgE-mediated milk allergy in infancy when high titres of cow's milk protein-specific IgG antibodies are known to be present. This can be avoided by using a system employing labelled antigen, such as the enzyme-linked immunosorbent assay (ELISA) described here, where IgE in the test serum is immunoadsorbed to anti-human IgE coated to microtitre plates. Biotinylated antigen, in this case cow's milk proteins, binds to specific IgE and the reaction is revealed colorimetrically by adding horseradish peroxidase (HRP)-avidin conjugate.

80 children with selective immunoglobulin A (IgA) deficiency--40 with severe deficiency (serum IgA less than 5 mg/dl) and 40 with partial deficiency (serum IgA greater than 5 mg/dl but less than minus 2 SD of the age-normal mean)--were followed up for 1.5 to 9 years; during which their serum and salivary IgA levels were measured periodically and the number and type of infections they had were recorded. In the partial deficiency group serum IgA rose to normal levels in half the group at a median age of 14 years and at a median time of 4 years after diagnosis, but they did not reach the normal range in the severe deficiency group. Pneumonia occurred more frequently in the severe than in the partial deficiency group. In addition, 11 of the 12 severely IgA deficient patients who had pneumonia had levels of both serum and salivary IgA of less than 0.5 mg/dl, and only 1 had detectable serum IgA levels. These data indicate that in childhood severe IgA deficiency is persistent and predisposed to pneumonia, whereas partial IgA deficiency is often transient and only occasionally associated with pneumonia.

A 7-month-old child with normal male external genitalia and normal, descended testicles had a 45,X karyotype in blood, skin, and testis cultures. He was mentally retarded and had a variety of bone alterations indicating disturbances of growth and ossifications. Dermatoglyphic patterns have several of the characteristics found in the 45,X female subjects with Turner's syndrome. This seems to be the first case of an "XO male" with normal external genitalia. We postulate the involvement of a "sex reversal" gene as in the origin of the XX males, with a secondary, coincidental loss of one X chromosome.

Sir .—The article by Dr Steele and colleagues ( Am J Dis Child 130:1218,1976) rightly underlines the need for reliable and simple tests of cellular immune competence to permit early detection of immunodeficiency diseases in infancy and childhood. In a series of 608 individuals, ranging in age from newborn to more than 70 years, whose skin was tested with phytohemagglutinin (PHA), we previously reported 1,2 an overall positivity of 98%; the only negative subjects were ten of 32 newborns. On the other In a series of 608 individuals, ranging in age from newborn to more than 70 years, whose skin was tested with phytohemagglutinin (PHA), we previously reported 1,2 an overall positivity of 98%; the only negative subjects were ten of 32 newborns. On the other hand, skin reactivity to streptokinase assessed in 584 individuals (age range, birth to more than 70 years) was remarkably age-depen dent. Some of these data (concerning subjects of