Cilia in children with recurrent upper respiratory tract infections: ultrastructural observations.

We investigated the ultrastructure of nasal cilia in 27 children suffering from recurrent infections of the upper respiratory tract, during and after the onset of an acute respiratory infection, and after a convalescent period of 12 weeks. Our results demonstrated that in seven subjects after resolution of infection, the morphology of a large proportion of the cilia (32%) was not back to normal. These findings suggest a long-term residual effect of infection, or the inability to reestablish normal ciliary structure during the convalescent period in some subjects with recurrent upper respiratory tract infection.

Effect of oral administration of bacterial extracts on the bactericidal capacity of polymorphonuclear leucocytes in children with recurrent respiratory infections.

The effect of orally administered bacterial extracts given intermittently over 16 weeks on the bactericidal capacity of polymorphonuclear leucocytes (PMNs) in children with recurrent respiratory infections was investigated using a luminol-amplified chemiluminescence assay. Chemiluminescence of PMNs stimulated with zymosan or N-formyl-methionyl-leucyl-phenylalanine (fMLP) before and after treatment with bacterial extracts or intramuscular benzanthine penicillin was evaluated. Chemiluminescence induced by opsonized zymosan increased significantly (P less than 0.05) after treatment with bacterial extracts, whereas no significant changes were observed in the fMLP-stimulated PMNs. Long-acting penicillin treatment did not significantly affect zymosan- or fMLP-stimulated chemiluminescence. The data suggest that orally administered bacterial extracts can increase the opsonic capacity of serum and thus the bactericidal capacity of PMNs in subjects with recurrent respiratory infections.

[The therapy of malformation syndromes]. / La terapia nelle sindromi malformative.

The therapeutic possibilities in malformative syndromes are basically surgical, rehabilitative and, in a few cases, pharmacologic. The possibilities of using drugs are limited to some hormonal disorders caused by diencephalic and hypophyseal dysfunctions with clinical signs appearing at the level of the various target organs. The clinical signs most commonly found are hypogonadism, short stature and obesity. The Authors discuss on the opportunity of the use of the growth hormone in syndromic patients with short stature.

Membrane fluidity of polymorphonuclear leukocytes from children with primary ciliary dyskinesia.

Plasma membrane fluidity and heterogeneity of polymorphonuclear leukocytes (PMN) were investigated in seven children with primary ciliary dyskinesia (PCD) and 17 healthy controls. Membrane fluidity and heterogeneity were studied by measuring the steady state fluorescence anisotropy and fluorescence decay of 1-(4-trimethylammoniumphenyl)-6-phenyl-1,3,5-hexatriene (TMA-DPH) incorporated into PMN plasma membrane. Our results show an increase in membrane fluidity at the surface level of PMN from patients with PCD. Distribution analysis of TMA-DPH lifetime values indicate an increase in membrane heterogeneity in subjects with PCD. The observed changes in the physicochemical properties of the membrane could lead to alterations in the function of PMN from children with PCD.

[Cohen syndrome. Description of a new case and study of the central nervous system using nuclear magnetic resonance]. / La sindrome di Cohen. Descrizione di un nuovo caso e studio del sistema nervoso centrale mediante risonanza magnetica nucleare.

The Cohen syndrome is characterized by dysmorphic face, obesity, narrow hands and feet and mild mental retardation. So far only 42 cases have been described in literature. The Authors describe a patient who presented some cerebral anomalies at the MRI examination. In particular the MRI showed a large sellar cavity compared to the size of the hypophysis.

[Oxidative metabolism in polymorphonuclear granulocytes of children with trisomy 21]. / Metabolismo ossidativo in granulociti polimorfonucleati di bambini con trisomia 21.

In response to certain stimuli, polymorphonuclear leukocytes (PMNs) undergo an oxidative burst during which a series of reactive oxygen metabolites are generated. The importance of the release of these oxygen metabolites by polymorphonuclear leukocytes, is recognized to be a key event in the function of these cells during infection and inflammation. We have evaluated the release of reactive oxygen species during the activation of respiratory burst (RB) of PMNs obtained from children with trisomy 21 using chemiluminescence techniques. As chemiluminogenic probes we have employed lucigenin and luminon that are know to be sensitive to the superoxide anion and the H2O2-myeloperoxidase-halide system of PMNs, respectively. Activated PMNs from children with trisomy 21 exhibited a low level of superoxide and a reduced activity of H2O2-myeloperoxidase-halide system compared to the control group. No significant difference in extracellular H2O2 release was observed. It seems likely that alterations in the enzymatic activities of the Cu/Zn-Superoxide dismutase and myeloperoxidase induce imbalance in the release of reactive oxygen species in activated PMNs from children with trisomy 21. This imbalance could be on the basis of the increased oxidative injury reported in trisomy 21.

A study of the Interaction Between Cetirizine and Plasma Membrane of Eosinophils, Neutrophils, Platelets and Lymphocytes using A fluorescence Technique.

The effect of cetirizine on plasma membrane fluidity and heterogeneity of human eosinophils, neutrophils, platelets and lymphocytes was investigated using a fluorescence technique. Membrane fluidity and heterogeneity were studied by measuring the steady-state fluorescence anisotropy and fluorescence decay of 1-(4- trimethylammonium-phenyl)-6-phenyl-1, 3, 5-hexatriene (TMA-DPH) incorporated in the membrane. The results demonstrate that cetirizine (1 mug/ml) induced a significant increase in the Hpid order in the exterior part of the membrane and a decrease in membrane heterogeneity in eosinophils, neutrophils and platelets. Moreover, cetirizine blocked the PAF induced changes in membrane fluidity in these cells. Cetirizine did not influence significantly the plasma membrane of lymphocytes. These data may partially explain the effect ofcetirizine on inflammatory cell activities.

Polymorphonuclear leukocyte-generated oxygen metabolites decrease beat frequency of human respiratory cilia.

We investigated the effect of polymorphonuclear leukocyte (PMN)-generated oxygen metabolites on the ciliary beat frequency. PMNs were incubated with human respiratory cilia obtained by nasal brushing. The oxidative metabolism was stimulated by opsonized zymosan, and ciliary beat frequency was evaluated before and after activation of PMNs. Ciliary beat frequency was studied using video microscopy. Our results demonstrate a significant decrease in ciliary beat frequency after activation of PMNs. This effect was reduced by catalase. These data suggest that the PMN-generated oxygen metabolites, particularly H2O2, decrease beat frequency of human respiratory cilia.

[Protective effect of oxatomide in infantile bronchial asthma. Double-blind study vs placebo]. / Effetto protettivo dell'oxatomide sull'asma bronchiale infantile. Studio in doppio cieco verso placebo.

BACKGROUND: This study has been designed to assess the protective effect of oxatomide in allergic bronchial asthma of the seasonal type in young children. METHODS: The study was carried out in a paediatric clinic; sixteen children divided into two balanced groups took oxatomide in an oral suspension at the dosage of 1 mg/kg/day, or placebo for a period of 2 months. Eight patients (7 males, 1 female), aged 22 months +/- 2.83 (mean +/- SD) took oxatomide in an oral suspension at the dosage of 1 mg/kg/day, while the other eight (3 males, 5 females; 22.13 months +/- 3.48) took placebo. Efficacy was assessed by monitoring cough, dyspnea at rest, dyspnea following exercise, wheezing, sleep disorders at baseline and after 15, 30 and 60 days of treatment, on the basis of a semiquantitative scale. All side effects were recorded. RESULTS: Persistent coughing was significantly reduced (p < 0.05) after two weeks' treatment with oxatomide. Sleep disorders and other symptoms remarkably improved. Dyspnea at rest and following exercise disappeared after 15 days' therapy, while the intensity of wheezing decreased after 30 days' active treatment. In all parameters examined, oxatomide was significantly more active than placebo at the first examination (p < 0.05 and p < 0.01). Oxatomide was well tolerated and only 2 patients complained of drowsiness which required a reduction in dosage. CONCLUSIONS: Oxatomide, at the dose of 1 mg/kg/day, obtained a good control of respiratory symptoms.

Successful interferon gamma therapy in a patient with X-linked chronic granulomatous disease, McLeod syndrome and hyper-IgE. Case report.

Recent studies have shown clinical benefit resulting from recombinant interferon gamma (rIFN-gamma) therapy in patients affected by chronic granulomatous disease (CGD), which represents an important adjunct to conventional therapy. In order to evaluate the effect of interferon gamma therapy, we investigated clinical and haematological parameters in a child with X-linked CGD, McLeod phenotype (kell negative) and hyper-IgE, before and after 8 months of therapy. Our results show no significant effect of rIFN-gamma on the respiratory burst of peripheral polymorphonuclear leukocytes. This notwithstanding, we observed improved clinical and haematological conditions. These results support the view that interferon gamma may benefit these subjects by influencing oxygen-independent antimicrobial activity or other immunological parameters.

Steatosis associated with immotile cilia syndrome: an unrecognized relationship?

The present study deals with a case of hepatic parenchymal steatosis in a child with primary ciliary dyskinesia (immotile cilia syndrome) well documented by functional and ultrastructural evaluation of the ciliary epithelia. Hepatic steatosis was associated with ultrastructural evidence of retention of material either in the cisternae of the endoplasmic reticulum or in proximity of the Golgi apparatus of hepatocytes. It is suggested that the absence of dynein in the axoneme is probably part of a diffuse genetic defect which may extend to cytoplasmic, non axonemal, dynein and lead to a disturbance of various microtubule-dependent cell activities.

[A case of focal necrotizing granulomatous hepatitis in childhood]. / Su un caso di epatite granulomatosa necrotizzante a focolai, in età pediatrica.

Granulomatous hepatitis make up a group of conditions of various aetiologies. The diagnosis of granulomatous hepatitis is histologic, since the aspect of the granulomatous lesion is not always indicative of a specific disease. It is important to take into consideration for the aetiologic diagnosis a good response to a specific therapy or the execution of complementary examinations. The 25% of granulomatous hepatitis remain undiagnosed.

Respiratory distress in a newborn with primary ciliary dyskinesia, situs inversus and Turner syndrome.

A report is given of a newborn girl with situs inversus and Turner syndrome that presented respiratory distress. The patient had a mosaic karyotype 45,X/46,X + mar (80%/20%). Ciliary motion analysis demonstrated a total absence of ciliary motion whereas, ultrastructural studies revealed typical features of primary ciliary dyskinesia (PCD) (absence or short outer/inner dynein arms in 90% of the cilia). We regard this rare combination (PCD, situs inversus and Turner syndrome) as a coincidental occurrence.

Double-blind multicenter study on the efficacy and tolerability of cetirizine compared with oxatomide in chronic idiopathic urticaria in preschool children.

BACKGROUND: There are no studies on the use of cetirizine in children under the age of 6. OBJECTIVE: We compared the efficacy and tolerability of cetirizine in patients with idiopathic chronic urticaria to the more widely used antihistamine, oxatomide. METHODS: This double-blind study was performed on 62 patients (38 male and 24 female) with idiopathic chronic urticaria, recruited from four different medical centers of the national territory (Ancona, Cagliari, Catania, and Messina). The children's ages ranged from 2 to 6 years (mean 3.85). The patients were randomly assigned to two treatment groups: one group treated 31 children with cetirizine at a dosage of 5 mg q.d., and a second group treated 31 children for the same amount of time with oxatomide, at a dosage of 25 mg q.d. Sixty-two children began the treatment, but five did not finish the study (three in the cetirizine and two in the oxatomide group). Thus, the clinical study and the statistical evaluation were conducted on 57 children (28 cetirizine and 29 oxatomide). The Student's t test was used to compare severity of the illness and changes in the hematochemical tests. RESULTS: Overall, the effectiveness of the two medications in treating erythema, papules, edema, and itching showed comparable therapeutic activity (P < 0.001). Neither medication produced significant side effects. CONCLUSIONS: The results of the present study suggest that cetirizine may represent an effective and safe pharmacologic therapy for chronic urticaria in preschool children. There was no evidence for changes in hematochemical and urinary values, demonstrating the safety and the tolerability of the two antihistamines, even when given to young children.

Effect of inhaled nedocromil sodium therapy on blood gas changes in children challenged with ultrasonically nebulized distilled water. A comparison of 8 mg versus 16 mg daily dose.

Changes in time course of blood partial pressures of oxygen (PtcO2) and carbon dioxide (PtcCO2) before, during and after challenge with ultrasonically nebulized distilled water (UNDW) were evaluated in 22 children with mild asthma in basal conditions, and after 8 weeks of therapy with inhaled nedocromil sodium at a daily dosage of 8 or 16 mg. PtcO2 and PtcCO2 were followed using transcutaneous O2 and CO2 monitoring system. All asthmatic subjects presented a significant decrease in PtcO2 and/or PtcCO2 (> 20% basal value) during or after challenge. After therapy, the decrease in PtcO2 and PtcCO2 was normalized in the group treated with 16 mg/day, whereas only a partial yet significant reduction in the decrease of O2 and CO2 was observed in the group assuming 8 mg/-day. These data indicate that inhaled nedocromil is effective in treating bronchial hyperresponsiveness in childhood and that the dose required to achieve this effect is of 16 mg/day.

Effect of nedocromil sodium on polymorphonuclear leukocyte plasma membrane.

The effect of nedocromil sodium on the plasma membrane fluidity of polymorphonuclear leukocytes (PMNs) was investigated by measuring steady-state fluorescence anisotropy of 1-[4-trimethylammonium-phenyl]-6-phenyl- 1,3,5-hexatriene (TMA-DPH) incorporated in the membrane. Our results show that nedocromil sodium 300 muM significantly decreased membrane fluidity of PMNs. The decrease in membrane fluidity of PMNs induced by fMLP was abolished in the presence of nedocromil sodium. These data suggest that nedocromil sodium interferes with the plasma membranes of PMNs and modulates their activities.