Double blind, placebo controlled study of nedocromil sodium in asthma.

After a two week baseline, 209 asthmatic children (mean age 10 years, range 6-17) were randomly allocated to receive 4 mg nedocromil sodium (n = 110) or placebo (n = 99) four times daily for 12 weeks in addition to their current treatment. The children completed daily diary cards and visited the clinic at four week intervals. Statistically significant differences in favour of nedocromil sodium were seen for clinician assessment of asthma severity and diary card symptom scores, pulmonary function and inhaled beta 2 bronchodilator use. Total symptom score decreased by 50% from baseline in the nedocromil sodium group and by 9% in the placebo group during the final four weeks. Nedocromil sodium was considered very or moderately effective by 78% of children/parents (placebo 59%) and 73% of clinicians (placebo 50%). Nausea, headache and sleepiness, and dyspnoea led to withdrawal of one child from nedocromil sodium and placebo treatments, respectively. Reports of sore throat and headache were marginally greater with the nedocromil sodium treatment. It is concluded that nedocromil sodium was both effective and safe in the treatment of asthma in children.

CD5 positive B lymphocytes in seronegative juvenile arthritis.

We investigated the levels of circulating CD5+ B cells in 43 patients with seronegative, HLA-B27 negative juvenile arthritis, 7 patients with juvenile dermatomyositis (DM) and 16 children with systemic lupus erythematosus (SLE). We found that CD5+ B cell levels were high in juvenile arthritis (p less than 0.01), normal in juvenile DM and decreased in SLE (p less than 0.01) compared to 33 age matched controls. In juvenile arthritis, the increase of CD5+ B cells appeared to be independent of discuss activity and was present in all the onset types except in a subset of patient with late onset pauciarthritis.

Unilateral microfibrillar abnormalities in a case of asymmetric Marfan syndrome.

The Marfan syndrome is a dominantly inherited connective-tissue disorder characterized by ocular, cardiovascular, and musculoskeletal abnormalities. Although the underlying biochemical and molecular defect(s) of this pleiotropic disease is currently unknown, we have consistently observed apparent diminished content of elastin-associated microfibrillar fibers accumulating in skin, or produced by cultured fibroblasts, from patients with the Marfan syndrome and have documented the cosegregation of these immunofluorescent abnormalities of microfibrillar fibers with the Marfan syndrome phenotype in family studies. Recently, an unusual patient has been described with unilateral phenotypic features of the Marfan syndrome, providing an unique opportunity to compare microfibrillar fibers and other connective-tissue components between the affected and nonaffected sides. In the present report, we demonstrate striking differences in apparent content of microfibrillar fibers, as determined by indirect immunofluorescence of skin and fibroblast cultures, that are revealed when multiple homologous samples derived from different sides of the patient's body are compared. In contrast, no differences in apparent content of type III collagen or in the biosynthesis and apparent structure of types I and III (pro)collagens were found. HLA types and chromosome heteromorphisms were identical in fibroblasts from both sides of the body, eliminating the formal possibility of chimerism and suggesting that a postzygotic mutation accounts for the asymmetric manifestation of the Marfan syndrome in this patient. The observation of striking decreases in microfibrillar fibers on the affected side of the body provides further evidence that abnormalities of this component of the elastic fiber system may be central to the pathogenesis and possibly the etiology of the Marfan syndrome.

Variation of serum IgG subclass concentrations with disease activity in juvenile chronic arthritis.

Nineteen patients with juvenile chronic arthritis were followed up and serum IgG subclass concentrations measured at different stages of disease activity. Patients were divided into three groups according to clinical activity of the disease: active disease, partial remission, and remission. The results were compared with normal values obtained in 448 healthy children aged 6 months to 18 years with a homogeneous distribution for each year of age. Serum IgG subclass concentrations of each child were first log transformed and then age corrected, taking the deviation of the log transformed value from that expected for a child of the same age. It was found that patients with partial remission had increased concentrations of IgG2 and decreased concentrations of IgG1 compared with patients with active disease. This suggests that the remission inducing process, at least in juvenile chronic arthritis, is accompanied by a switch of IgG subclass production.

Frequency of autoantibodies in normal children.

Very few data have been reported on the frequency of autoantibodies (AAs) in normal children. In the present study we investigated the frequency of 14 AAs in a total of 268 apparently normal children (151 boys and 117 girls; age range, 1 month to 14 years). Forty-one children (22 boys and 19 girls) were positive for at least one AA, usually in a low titer; two children were positive for two AAs. None of these children had a personal or family history of autoimmune diseases. The percentage of children positive for each AA was as follows: antinuclear, 3%; anti-smooth muscle, 2.6%; antireticulin, 2.6%; antimitochondrial, 1.1%; rheumatoid factor, 0.6%; antiribosomal, 0.4%; anti-gastric parietal cells, 5.2%; and anti-thyroid microsomal, 1.3%. Anti-double-stranded DNA, anti-intestinal epithelial cells, antiliver and antikidney microsomal, antithyroglobulin, anti-islet cells, and complement-fixing anti-islet cell antibodies were not detected in any serum. Fifteen of the 41 positive children were checked for the presence of AAs two years later; six (40%) were still positive, always for the same AA, without major differences in titer. Our results suggest that the overall frequency of AAs in apparently healthy children is quite similar to that reported in young adults; this AA positivity seems most often to represent a transient phenomenon.

Clinical evaluation of sulbactam plus ampicillin in the treatment of general pediatric infections.

Sixty pediatric patients (27 males and 33 females) between the ages of 7 months and 11.7 years (mean age = 4.3 yr) were treated with parenteral sulbactam plus ampicillin (1:2 ratio) for lower respiratory tract infections (29 cases), upper respiratory tract infections (4 cases), urinary tract infections (25 cases) or skin/soft tissue infections (2 cases). The infection was mild in 6 cases, moderate in 44 and severe in 10. The infection was acute in 57 patients, recurrent in 1 (cystitis) and was a flare-up of a chronic infection in 2 (pyelonephritis and cystitis). The children received an average dose of 48 mg/kg/d of sulbactam plus 96 mg/kg/d of ampicillin by the i.m. route (43 cases) or by i.v. drip (17 cases) in 3-4 divided doses. The length of treatment ranged between 3 and 10 d (mean duration = 6 d). At the end of therapy, clinical cure was achieved in 53 patients (88.3%), while 6 (10%) had a marked improvement. Only 1 patient, with a lower respiratory tract infection, did not respond to therapy. All 25 patients with urinary tract infection experienced bacteriological cure at the end of treatment. No side effects were reported. Mild and transient changes in laboratory parameters from baseline values were observed in 10 patients (eosinophilia, elevation of SGOT or SGPT) without clinical consequence. Sulbactam plus ampicillin was effective and safe in the treatment of bacterial infections in children and appears to be useful in the treatment of those infections in which beta-lactamase-producing organisms are involved.

Asymmetric Marfan syndrome.

We describe a girl with Marfan syndrome in whom the clinical expression of the disease was much more evident on the left side of the body.

Systemic lupus erythematosus with Jaccoud's arthropathy mimicking juvenile rheumatoid arthritis.

We describe a girl who presented at age 5 with objective signs of arthritis and was initially diagnosed as having juvenile rheumatoid arthritis. Over the following years, she developed slowly progressive joint deformities indicative of Jaccoud's arthropathy. When she was 11 years old, symptoms typical of systemic lupus erythematosus appeared, accompanied by anti-DNA antibodies. This case illustrates that in children also, Jaccoud's arthropathy may be a precocious manifestation of systemic lupus erythematosus.

Enhanced interleukin 1 and depressed interleukin 2 production in juvenile arthritis.

Blood mononuclear cells from a total of 23 children with juvenile arthritis were stimulated in vitro to produce interleukin 1 (IL-1) and interleukin 2 (IL-2) and compared with age matched healthy controls. Peripheral blood monocytes from patients with juvenile arthritis produced a higher amount of IL-1 than controls, whereas peripheral blood lymphocytes from the same patients produced lower amount of IL-2 than controls. These findings could not be explained by concurrent therapy. The increase of IL-1 production was more marked in patients with active disease and therefore may have been secondary to the pathological process. However, the decrease of IL-2 production did not depend on disease activity, thus suggesting an immunoregulatory abnormality.

Role of IgE in the pathogenesis of milk allergy in infancy: reassessment by a new ELISA technique.

An ELISA technique using labelled antigen for the determination of cow's milk specific IgE in serum is described. The use of labelled antigen, rather than labelled antibody as in the RAST, permits avoidance of interference by antibodies other than IgE, such as IgG, at times responsible for a negative RAST. The results obtained with the 2 techniques in 43 infants with a positive cow's milk challenge showed a positive RAST in 28%, a positive ELISA in 35% and a positive RAST or ELISA in 42%. These findings suggest that the use of both ELISA and RAST permits in vitro diagnosis of cow's milk allergy in more patients than either test alone.

IgG subclass serum levels in juvenile chronic arthritis.

IgG subclass levels of sera from 26 patients with juvenile chronic arthritis (JCA) were determined by means of mouse monoclonal antibodies. Patients were divided into three groups according to clinical activity of the disease: active disease, partial remission, and remission. One hundred and sixty four age matched, healthy children served as controls. IgG subclass concentrations were log transformed, and a robust regression method was applied to obtain expected values for the different ages. We found a significant increase of IgG3 (p less than 0.0001), IgG1 (p less than 0.002), and IgG2 (p less than 0.035) in JCA sera, while IgG4 values did not differ significantly from those of controls. When patients were divided according to clinical activity significant increases of IgG2 and IgG4 were observed in the patients in partial remission. Our data suggest that differential increase of IgG subclasses during the courses of JCA may be of relevance to the pathogenesis of the disease.

Italian registry of patients off therapy after childhood acute lymphoblastic leukemia. Results after first phase of data collection.

The Italian Registry of Off-Therapy patients after childhood tumors now includes 760 subjects with acute lymphoblastic leukemia. These patients were all removed from treatment by December 31, 1981, and were followed in 35 different institutions. All the children have received multiple-drug treatment, combined, in 79.7% of the cases, with cranial irradiation. Thirty-nine (5%) experienced a relapse before treatment suspension. Total duration of antileukemic therapy ranges between 18 and 131 months (median, 38). At the last updating (December 31, 1981), 699 subjects were alive, 6 were lost to follow-up, and 55 had died. Life-table analysis shows that 90.8% were alive and 77% were alive in continuous complete remission at 36 months, whereas at 66 months, the cumulative proportions were 88% and 75.5%, respectively. One hundred thirty-six of 760 relapses after therapy suspension were reported: 83 in male patients and 53 in female patients (P less than 0.01). The longest interval between relapse and treatment suspension was 64 months. Six of 55 died in continuous complete remission 3 to 44 months after treatment suspension. Five births of apparently normal babies to female patients have been reported. A general outline of the project and the future program are given.

[Pediatric preventive medicine in the school of milieu: statistical results in the 1980-81 scholastic year in the commune of Giarratana]. / Medicina pediatrica preventiva nel campo scolastico: resultati statistici ottenuti nell'anno scolastico 1980-81 nel Comune di Giarratana.

414 children of the nursery, primary and secondary schools, of Giarratana's borough were visited by the pediatrician. From these data one can see an high rate of children with dental caries (37,19%), weight and height deficiency (13%). The final thoughts emphasize the need of sanitary measures because of the checked pathologies and polyspecialistic preventive controls to continue in the next years.