Changes of fluorescence anisotropy in plasma membrane of human polymorphonuclear leukocytes during the respiratory burst phenomenon.

Steady state fluorescence anisotropy (rs) of TMA-DPH was measured to study the effect of respiratory burst activation with PMA, FMLP, and PAF on the physico-chemical structure of PMNs plasma membrane. Our results show a significant increase in rs during the respiratory burst activation. In the presence of NADPH-oxidase inhibitor DPI, only PAF induces changes in rs values. This suggests a non-specific effect of PAF on plasma membrane. Azide, which induces a supranormal release of H2O2, fails to increase the basal rs value after activation. Moreover, the catalase does not abolish the increase in rs induced upon activation. This rules out the possibility that changes of rs during the respiratory burst activation are attributed mainly to H2O2 release. We conclude that multiple processes accompanying the respiratory burst activation are responsible for the changes in the physico-chemical properties of PMNs plasma membrane.

Changes in carbohydrate composition in human milk over 4 months of lactation.

This study aimed to examine the carbohydrate content (monosaccharides, lactose, and oligosaccharides) of human milk over 4 months of lactation to determine whether any changes occurred over time. Milk samples from 46 mothers, who delivered at term, were collected at 4th, 10th, 30th, 60th, 90th, and 120th days after delivery. Carbohydrates were measured by high-pressure liquid chromatography. Mean lactose concentration (+/- SD) increased from 56 +/- 6.06 g/L on day 4 to 68.9 +/- 8.16 g/L on day 120. Oligosaccharide level decreased from 20.9 +/- 4.81 g/L to 12.9 +/- 3.30 g/L, respectively. Monosaccharides represented only 1.2% of total carbohydrates. The changes in carbohydrate composition found indicate that carbohydrate synthesis by the mammary gland is a dynamic process. The physiological and biological relevance of human milk oligosaccharides is also discussed.

Mild form of Jeune syndrome in two sisters.

Jeune syndrome is characterized by respiratory distress, osseous dysplasia, and short stature. Patients generally die during the first months of life. However, some cases with milder clinical manifestations have been described; these cases show characteristic renal involvement of different severity. The authors report on two cases of the mild form of Jeune syndrome in sisters.

Use of Laurdan fluorescence in studying plasma membrane organization of polymorphonuclear leukocytes during the respiratory burst.

The changes in plasma membrane polarity of polymorphonuclear leukocytes (PMN) during the activation of the respiratory burst were investigated by measuring the steady-state fluorescence emission spectra of 2-dimethylamino(6-lauroyl) naphthalene (Laurdan), which is known to be incorporated at the hydrophobic-hydrophilic interface of the bilayer, displaying spectral sensitivity to the polarity of its surroundings. Laurdan shows a marked steady-state emission blue shift in nonpolar solvents, with respect to polar solvents. Our results show a blue shift of the fluorescence emission spectra of Laurdan during activation of PMN with phorbol myristate acetate or N-formyl-methionyl-leucyl-phenylalanine. These results suggest that the activation of the respiratory burst of PMN is accompanied by a decrease in polarity in the hydrophobic-hydrophilic interface of the plasma membrane.

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.

Evaluation of the efficacy of oral cromolyn sodium or an oligoantigenic diet in children with atopic dermatitis: a multicenter study of 1085 patients.

One thousand eighty-five children with atopic dermatitis were enrolled in a multicenter study to evaluate the efficacy of 4 weeks of oral sodium cromoglycate or 4 weeks of a restricted diet. One thousand-eleven children (93%) concluded the study. At the end of the trial there was a significant improvement in skin lesions in the two groups: 61% of the patients in the sodium cromoglycate group and 69% in the restricted diet showed a significant improvement in atopic dermatitis. We concluded that, at least in our experimental design, both sodium cromoglycate and a restricted diet are equally effective in atopic dermatitis.

[Percutaneous renal biopsy in children: use of a fine needle]. / Biopsia renale percutanea in età pediatrica: uso di un ago sottile.

Percutaneous renal biopsies were performed on native kidneys in 40 children and adolescent, aged 1.7-13 (mean 6.7) years. Bleeding diatheses were excluded by the determination of Hb, blood platelets, PT, PTI and fibrinogen. Biopsies were performed under ultrasound imaging, using a semiautomated and thin needle (20 gauge in children with age under 5 years and 18 gauge for those over 5 years). All the patients were lightly sedated, except for 3 ones who received a general anesthesia. Diagnostically adequate tissue was retrieved in 38 of 40 biopsy procedures (95%). A macro-haematuria was observed with elimination of haematic coagula in 3 children (7.5%) and 24-h post-biopsy ultrasonography disciosed a small haematoma of the biopsied kidney in 2 one (5%). No mayor complications occurred. We conclude that the use of ultrasound imaging and a semiautomated needle is a safe and efficient method for performing renal biopsies in paediatric patients. The use of smaller (18 or 20-gauge) cutting needles would reduce the complications rate while allowing retrieval of sufficient tissue for histologic diagnosis.

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.

Dietary compliance in screening-detected coeliac disease adolescents.

In 1992-94 we screened 6315 students for coeliac disease (CD) by testing antigliadin antibodies (AGA) as the first-level investigation. We found 28 biopsy-proven coeliac patients who were invited to start the gluten-free diet (GFD). The aim of this study was a clinical and laboratory follow-up in these screening-detected coeliac adolescents. Patients were 17 females and 11 males with a mean age at diagnosis of 12.8 +/- 1 years (range 11-4). Mean follow-up duration time was 23 +/- 7 months (range 9-37). Twenty-three of the 28 screening-detected coeliac patients came to the control visit, 3 refused the follow-up and 2 subjects were not found. Twelve patients (52.2%) stated that they never ate any gluten-containing food, while 11 of them (47.8%) reported occasional transgressions to the diet. GFD acceptance was reported as good (n = 6), moderate (n = 11) or low (n = 6). After starting the GFD, signs of improvement were seen in most patients, such as weight gain, increased height velocity and increased feeling of well-being. AGA (both IgG and IgA classes) and antiendomysium antibodies (AEA) were normal in 19 subjects, 2 cases had IgG-AGA and AEA positivity, 1 patient showed abnormal AGA and AEA levels, while isolated IgA-AGA positivity persisted in 1 case. This study shows that even silent CD cases can clinically benefit from the GFD. The consequences of occasional transgressions to the GFD remain unclear.

The coeliac iceberg in Italy. A multicentre antigliadin antibodies screening for coeliac disease in school-age subjects.

BACKGROUND: Recent studies suggest that coeliac disease (CD) is one of the commonest, life-long disorders in Italy. The aims of this multicentre work were: (a) to establish the prevalence of CD on a nationwide basis; and (b) to characterize the CD clinical spectrum in Italy. PATIENTS AND METHODS: Fifteen centres screened 17,201 students aged 6-15 years (68.6% of the eligible population) by the combined determination of serum IgG- and IgA-antigliadin antibody (AGA) test; 1289 (7.5%) were IgG and/or IgA-AGA positive and were recalled for the second-level investigation; 111 of them met the criteria for the intestinal biopsy: IgA-AGA positivity and/or AEA positivity or IgG-AGA positivity plus serum IgA deficiency. RESULTS: Intestinal biopsy was performed on 98 of the 111 subjects. CD was diagnosed in 82 subjects (75 biopsy proven, 7 not biopsied but with associated AGA and AEA positivity). Most of the screening-detected coeliac patients showed low-grade intensity illness often associated with decreased psychophysical well-being. There were two AEA negative cases with associated CD and IgA deficiency. The prevalence of undiagnosed CD was 4.77 x 1000 (95% CI 3.79-5.91), 1 in 210 subjects. The overall prevalence of CD, including known CD cases, was 5.44 x 1000 (95% CI 4.57-6.44), 1 in 184 subjects. The ratio of known to undiagnosed CD cases was 1 in 7. CONCLUSIONS: These findings confirm that, in Italy, CD is one of the most common chronic disorders showing a wide and heterogeneous clinical spectrum. Most CD cases remain undiagnosed unless actively searched.

Human posture in microgravity: an experiment on EUROMIR '95 to verify and improve a simulation tool.

An anthropometric mannequin implemented in robotic modelling software has proved very useful in the simulation of static and semi-dynamic reachability envelopes. Its prediction of working postures has been verified to some extent during neutral buoyancy trials. While a robotic solution is useful for static analyses or rough estimates of simple movements, more realistic movement strategies need to be identified directly measuring astronauts' in-orbit behaviour. A set of experiments is to be performed as part of the EUROMIR '95 mission to the MIR orbiting station in which dynamic posture (i.e. posture and movement) measurements will be taken using the ELITE system. The data and analyses of the data will be used to animate the Alenia anthropometric mannequin and to develop movement algorithms more similar to those of a person in microgravity than the robotic solutions currently employed. This paper presents the experiments to be performed and the changes to Alenia's mannequin that will allow the model to effect movements according to the experimental results. It is aimed at expanding the dialog between the biomechanical and human factors disciplines started in this experiment to other potential end-users of the experimental results.

[Zinc and chronic enteropathies]. / Zinco ed enteropatie croniche.

In recent years the nutritional importance of zinc has been well established; its deficiency and its symptoms have also been recognized in humans. Furthermore, Acrodermatitis Enteropathica has been isolated, a rare but severe disease, of which skin lesions, chronic diarrhoea and recurring infections are the main symptoms. The disease is related to the malfunctioning of intestinal absorption of zinc and can be treated by administering pharmacological doses of zinc orally. Good dietary sources of zinc are meat, fish and, to a less extent, human milk. The amount of zinc absorbed in the small intestine is influenced by other nutrients: some compounds inhibit this process (dietary fiber, phytate) while others (picolinic acid, citric acid), referred to as Zn-binding ligands (ZnBL) facilitate it. Citric acid is thought to be the ligand which accounts for the high level of bioavailability of zinc in human milk. zinc absorption occurs throughout the small intestine, not only in the prossimal tract (duodenum and jejunum) but also in the distal tract (ileum). Diarrhoea is one of the clinical manifestations of zinc deficiency, thus many illnesses distinguished by chronic diarrhoea entail a bad absorption of zinc. In fact, in some cases of chronic enteropathies in infants, like coeliac disease and seldom cystic fibrosis, a deficiency of zinc has been isolated. Some of the symptoms of Crohn's disease, like retarded growth and hypogonadism, have been related to hypozinchemia which is present in this illness. Finally, it is possible that some of the dietary treatments frequently used for persistent post-enteritis diarrhoea (i.e. cow's milk exclusion, abuse and misuse of dietary fiber like carrot and carub powder, use of soy formula) can constitute a scarce supply of zinc and therefore could promote the persistency of diarrhoea itself.

[The importance of the clinical record in the follow-up of malformation syndromes]. / L'importanza della cartella clinica nel follow-up delle sindromi malformative.

In view of the pediatrician's need to know the natural history of these patients so as to guarantee appropriate clinical and instrumental follow-up it is necessary to collect information, in a clinical file and in as much detail as possible, on the classic phenotype and the different clinical manifestations of the various syndromes. The aim of this is to identify the most suitable times for applying possible therapy, also bearing in mind the increase in the mean age of these patients.

[Malformation syndromes and deafness]. / Sindromi malformative e sordità.

Deafness is one of the most common clinical findings in patients affected by malformation syndromes. It may be congenital, neurosensory or transmissive in nature, or acquired as the consequence of phlogistic processes due to craniofacial malformations. It is important to identify and classify the hypoacusis in order to start therapy as early as possible, using surgery or prosthesis according to the indications, to avoid the child suffering both verbal and intellectual impairment.

[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.

[Protein-losing gastroenteropathy (Ménétrier's disease) in childhood: a report of 3 cases]. / Gastroenteropatia protido-disperdente (malattia di Ménétrier) nell'età pediatrica: descrizione di tre casi.

So far, Ménétrier's disease has been thought to be an uncommon disorder in children. It is characterized by hypertrophic gastritis, protein-losing enteropathy, hypoproteinemia and edema. During childhood, the main features of this condition include an abrupt onset and a spontaneous recovery. In this paper we describe three children, aging between 3 months and 3 years, who presented with protracted vomiting, generalized edema, colitis (one case) and elevated serum aminotransferases (one case). The diagnosis of Ménétrier's disease was made by finding the typical endoscopic and histological picture of the gastric mucosa (two cases) or by the radiological findings (one case). The fecal alpha-1-antitrypsin excretion, which is a marker of the protein-losing enteropathy, was high in all patients. Two cases showed evidences for a primary CMV infection as the possible cause of Ménétrier's disease, due to the presence of cytomegalic inclusions in the gastric mucosa and the IgM class anti-CMV antibodies positivity. All 3 cases, who received only a support treatment (plasma and albumin intravenous infusions), completely recovered in a 2-3 weeks time. In conclusion, it is confirmed that in children a protein-losing gastroenteropathy may be caused by a primary infection with CMV.

Bone marrow transplantation in Hunter syndrome (mucopolysaccharidosis type II): two-year follow-up of the first Italian patient and review of the literature.

A patient with Hunter syndrome, or mucopolysaccharidosis type II (MPS-osis II), was subjected to bone marrow transplantation (BMT), at the age of 2 9/12 years. A two-year follow-up ensued to the purpose of comparing clinical, biochemical, neuropsychologic status pre- and post-BMT. From the clinical standpoint, a complete normalization of hepatosplenomegaly was observed. In addition the skin decreased in thickness and joint mobility improved. The echocardiography showed normalization of left ventricle size. With the exception of verbal capabilities, there was no further deterioration of the neuropsychologic profile. The ultrastructural examination of the liver showed an almost total disappearance of storage material. Normal iduronate sulfatase levels in leukocytes and lymphoblasts were constantly found after BMT. A qualitative and quantitative improvement in urinary glycosaminoglycan (GAG) excretion was also found. The effectiveness of the BMT in our patient is also assessed in the context of the few cases of MPS-osis II that have been reported to date. A final evaluation of the efficacy of BMT in MPS-osis II will be possible only when a higher number of patients, diagnosed as early as possible and transplanted within the first months of life, can be followed-up for more extended periods of time.