Concerns about using Zn supplementation in Down's syndrome (DS) children.
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Alzheimer Disease↗
Biomedical subjects
G Sabatino
Publications and source records attributed to G Sabatino.
Lack of mother-to-child HIV-1 transmission is associated with elevated serum levels of 90 K immune modulatory protein.
BACKGROUND: 90 K is a secreted human serum glycoprotein with immune modulatory activity. METHODS: Serum 90 K levels were determined by an enzyme-linked immunosorbent assay in 19 HIV-1-infected mothers at the time of delivery, in their new-borns (11 HIV-1 infected and eight uninfected), in 26 HIV-1-uninfected mothers and in 86 new-borns of HIV-1-uninfected mothers. RESULTS: 90 K levels in HIV-1-infected transmissive mothers (22.4 +/- 13.9 microg/ml) were similar to those of HIV-1-uninfected mothers (21.1 +/- 7.6 microg/ml; P = 0.715), but lower than those of HIV-1-infected non-transmissive mothers (45.5 +/- 24.8 microg/ml; P = 0.019). The levels were higher in HIV-1-uninfected (47.6 +/- 22.4 mg/ml) than HIV-1-infected (23.7 +/- 15.6 microg/ml; P = 0.014) new-borns of HIV-1-infected mothers. The new-borns of HIV-1-uninfected mothers had lower levels (11.7 +/- 5.3 microg/ml) than both HIV-1-infected and HIV-1-uninfected new-borns of HIV-1-infected mothers (all differences, P < 0.001). CONCLUSION: These results suggest that high 90 K protein serum levels in HIV-1-infected mothers and their new-borns are associated with lack of mother-to-child HIV-1 transmission.
Adult↗
Carotid body NO-CO interaction and chronic hypoxia.
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Animals↗
Stormy onset of benign childhood epilepsy with occipital paroxysmal discharges.
We studied six children with ages ranging from 4 to 10 years who were affected by childhood epilepsy with occipital paroxysms and presented after a stormy onset with prolonged loss of consciousness for 6-14 h. In all these patients, seizures were preceded by visual symptoms in the form of colored circular disks. A CT scan was performed immediately after the onset of symptoms and was normal in all patients. Routine laboratory and cerebrospinal fluid examinations were normal in all patients. The interictal EEG was characterized by continuous or subcontinuous occipital spike wave discharges, which disappeared after the patients' eyes opened. We carried out a 7-year follow-up of all these patients. Only two patients were treated with antiepileptic drugs. The therapy (phenobarbital, clobazam) in the two patients did not induce changes in the EEG pattern. The first did not suffer any further seizures. The second patient had two more seizures (at 8 and 18 months from the onset) with phosphenes, confusional state, and involuntary movements followed by loss of consciousness. Among the other four patients, who did not receive any treatment, only one had any other seizures. The stormy onset of the syndrome described in our six patients emphasizes the extreme variability in the presentation of this type of childhood epilepsy. Our follow-up confirms the good prognosis of this epilepsy even when it has a stormy onset.
Child↗
Childhood epilepsy with occipital paroxysms and benign nocturnal childhood occipital epilepsy.
Two types of childhood epilepsy have recently been reported: childhood epilepsy with occipital paroxysms, and benign nocturnal childhood occipital epilepsy. This article reports the clinical evolution, electroencephalographic (EEG) changes, and response to therapy of eight children with childhood epilepsy with occipital paroxysms (five boys and three girls, aged from 1 1/12 to 8 years) and eight children with benign nocturnal childhood occipital epilepsy (six boys and two girls, aged from 1 4/12 to 8 3/12 years). A careful clinical and EEG follow-up of at least 7 years was carried out for all patients. At the end of follow-up, all but one of the patients with childhood epilepsy with occipital paroxysms were seizure-free, and only two were still receiving anticonvulsant drugs. All but three children had a normal EEG, and normal mental development was observed in all but two cases. Patients with benign nocturnal childhood occipital epilepsy had a good long-term prognosis; all but two children with benign nocturnal childhood occipital epilepsy had a normal EEG. These two patients showed learning disabilities and poor school performances, and required remedial education. Therefore, although childhood epilepsy with occipital paroxysms and benign nocturnal childhood occipital epilepsy are two different types of epilepsy, the long-term prognosis seems to be similar.
Anticonvulsants↗
Down's syndrome is associated with increased 8,12-iso-iPF2alpha-VI levels: evidence for enhanced lipid peroxidation in vivo.
Postmortem and in vitro studies have shown that oxidative stress plays a role in the pathogenesis of many of the clinical features of Down's syndrome. The isoprostane 8,12-iso-iPF2alpha-VI is a specific marker of lipid peroxidation. We found elevated levels of this isoprostane in urine samples of subjects with Down's syndrome compared with those of matched controls, which correlated with the duration of the disease. These results suggest that increased in vivo lipid peroxidation is a prominent component early in the course of Down's syndrome.
Adolescent↗
Inefficient phospholipase C activation and reduced Lck expression characterize the signaling defect of umbilical cord T lymphocytes.
Adult and neonatal immunocompetent cells exhibit important functional distinctions, including differences in cytokine production and susceptibility to tolerance induction. We have investigated the molecular features that characterize the immune response of cord blood-derived T lymphocytes compared with that of adult T lymphocytes. Our findings demonstrate that phospholipase C (PLC) isozymes, which play a pivotal role in the control of protein kinase C activation and Ca2+ mobilization, are differently expressed in cord and adult T lymphocytes. PLCbeta1 and delta1 are expressed at higher levels in cord T cells, while PLCbeta2 and gamma1 expression is higher in adult T lymphocytes. PLCdelta2 and gamma2 appear to be equally expressed in both cell types. In addition, a functional defect in PLC activation via CD3 ligation or pervanadate treatment, stimuli that activate tyrosine kinases, was observed in cord blood T cells, whereas treatment with aluminum tetrafluoride (AlF4-), a G protein activator, demonstrated a similar degree of PLC activation in cord and adult T cells. The impaired PLC activation of cord blood-derived T cells was associated with a a very low expression of the Src kinase, Lck, along with a reduced level of ZAP70. No mitogenic response to CD3 ligation was observed in cord T cells. However, no signaling defect was apparent downstream of PLC activation, as demonstrated by the mitogenic response of cord T cells to the pharmacologic activation of protein kinase C and Ca2+ by treatment with PMA and ionomycin. Thus, neonatal cord blood-derived T cells show a signaling immaturity associated with inadequate PLCgamma activation and decreased Lck expression.
Adult↗
Iron-dependent human platelet activation and hydroxyl radical formation: involvement of protein kinase C.
BACKGROUND: Iron is an important modulator of lipid peroxidation, and its levels have been associated with the progression of atherosclerosis. Little is known about the possibility that this metal, when released from tissue stores, may modulate the reactivity of blood cell components, in particular platelets. Therefore, we investigated a possible link between iron, oxygen free radical formation, and platelet function. METHODS AND RESULTS: Human whole blood was stimulated with collagen 2 micrograms/mL, and an irreversible aggregation with thromboxane (Tx)B2 formation was observed (15+/-4 versus 130+/-10 ng/mL). Deferoxamine (DSF), a specific iron chelator, and catalase, an H2O2 scavenger, inhibited collagen-induced whole-blood aggregation. The aggregation was accompanied by an increase in hydroxyl radical (OH.) levels (30+/-8 versus 205+/-20 nmol/L dihydroxybenzoates), which were reduced by DSF and by 2 specific OH. scavengers, mannitol and deoxyribose. Iron (Fe2+) dose-dependently induced platelet aggregation, TxB2 formation (6+/-2 versus 135+/-8 ng/mL), and protein kinase C (PKC) translocation from the cytosol to the cell membrane when added to platelets that have been primed with a low concentration of collagen (0.2 micrograms/mL). In the same system, an increase in OH. levels was observed (37+/-12 versus 230+/-20 nmol/L dihydroxybenzoates). Mannitol and deoxyribose, but not urea, were able to reduce OH. formation, PKC activation, and platelet aggregation. Selective inhibition of PKC activity by GF 109203X prevented iron-dependent platelet aggregation without influencing OH. production. CONCLUSIONS: The present study shows that iron can directly interact with human platelets, resulting in their activation. Its action is mediated by OH. formation and involves PKC activity. Our findings provide an additional contribution to the understanding of the mechanism(s) by which iron overload might promote atherosclerosis and coronary artery disease.
Adult↗
Zinc sulfate supplementation improves thyroid function in hypozincemic Down children.
In subjects affected by trisomy 21 (Down syndrome), hypothyroidism is the most common endocrinological deficit. Plasma zinc levels, which are commonly detected below the normal range in Down patients, are related to some endocrinological and immunological functions; in fact, zinc deficiency has been shown to impair immune response and growth rate. Aims of this study were to evaluate (1) the role of zinc deficiency in subclinical hypothyroidism and (2) thyroid function changes in Down children cyclically supplemented with zinc sulfate. Inverse correlations have been observed between age and triiodotironine (T3) and between zinc and thyroid-stimulating hormone (TSH); higher TSH levels have been found in hypozincemic patients at the beginning of the study. After 6 mo of supplementation, an improvement of thyroid function (TSH levels: 3.96 +/- 1.84 vs 2.64 +/- 1.33 mUI/mL basally and after 6 mo, respectively) was observed in hypozincemic patients. In the second cycle of supplementation, a similar trend of TSH was observed. At the end of the study, TSH significantly decreased in treated hypozincemic subjects (4.48 +/- 1.93 vs 2.96 +/- 1.20 mUI/mL) and it was no longer different in comparison to normozincemic patients. We suggest zinc supplementation to the diet in hypozincemic Down children as a simple and useful therapeutic tool.
Adolescent↗
Neonatal suppurative parotitis: a study of five cases.
UNLABELLED: Suppurative parotitis is uncommon in newborns. During a 9-year study period, five cases of neonatal suppurative parotitis were detected in 3,624 hospital admissions. The relative risk of developing neonatal suppurative parotitis in admitted infants was 5.52 (0.62-49.35). Staphylococcus aureus was the causative organism most commonly detected in the hospital-acquired cases. Antimicrobial therapy was effective in all cases; surgery was not required. CONCLUSION: Although neonatal suppurative parotitis is now uncommon in the newborn, it cannot be considered a "vanishing disease".
Cross Infection↗
Headache in children with centrotemporal spikes.
The aim of the study was to evaluate the long-term evolution of headache associated with rolandic centrotemporal spikes (CTS). The patient group consisted of a group of 32 children who suffered from headache and presented CTS at electroencephalogram (EEG). As the control group, we selected 52 sex- and age-matched children with headache without any EEG abnormalities. During a follow-up of 5 years none of them showed epileptic seizures. The number of headache attacks decreased in the majority of patients, as in the controls. A good correlation could be identified between CTS and the number of headaches attacks both at baseline (r = 0.58, P < 0.001) and at follow-up (r = 0.64, P < 0.001). In four children (12.5%), the frequency of headache attacks increased and this increase was associated with a higher frequency of CTS. In two patients, a change in the EEG pattern was observed during follow-up, with a 'migration' of the epileptiform complex from central to parietooccipital leads. In conclusion, these findings confirm that CTS are not pathognomonic of centrotemporal epilepsy and that evolution of CTS and headache in children are statistically related.
Adolescent↗
Human milk 90K (Mac-2 BP): possible protective effects against acute respiratory infections.
Eighty-six children fed human milk were followed prospectively from birth to 12 months of age to assess the effect of milk 90K, a secreted glycoprotein with immune-stimulatory properties, on development of acute respiratory infections (ARI). The level of human milk 90K was inversely related to episodes of ARI (r = - 0.34; P = 0.001). The average 90K level in human milk fed to children who did not develop ARI was significantly higher than in milk fed to children in whom infection occurred on multiple occasions (156.6 +/- 144.8 microg/ml versus 70.9 +/- 92.3 microg/ml; P = 0.001). These data suggest that the protective effects of human milk against ARI may be due in part to immune maturation effects by secreted 90K.
Acute Disease↗
Centro-temporal spikes in non-epileptic children: a long-term follow up.
OBJECTIVE: This study was undertaken in order to determine the natural evolution of centro-temporal spikes (CTS) in a healthy paediatric population. METHODOLOGY: Forty children and adolescents (mean age +/- SD: 9.9 +/- 4.1 years) with non-epileptic disturbances (tics, headache, pavor nocturnus, vertigo, etc), who showed CTS during routine EEG recordings were studied. No patient suffered from seizures. A clinical and electroencephalographic follow-up of at least 5.5 years was carried out in all subjects. RESULTS: At the follow-up examination, 33 of the 40 cases (82.5%) had no CTS or other epileptic discharges in awake and sleep EEGs, while six subjects continued to show CTS discharges in sleep EEG. Among those subjects who showed persistence of CTS in sleep EEG, rolandic epilepsy developed in two cases, while all other subjects continued to be seizure free. CONCLUSIONS: This study demonstrates that CTS can be present in non-epileptic children and adolescents; in the majority of subjects this EEG abnormality disappears spontaneously and only a small percentage of subjects who have persistence of this pattern develops rolandic epilepsy.
Action Potentials↗
Hypoglycaemia in children with type 1 diabetes mellitus.
Hypoglycaemia is a frequent acute complication of IDDM and is usually defined as a blood glucose level below 3.0 mmol/l. Hypoglycaemia stimulates several neuroendocrine responses, such as secretion of glucagon, adrenaline, growth hormone and cortisol, which are generally increased during this phenomenon. The true prevalence of hypoglycaemia is not known. Studies of the epidemiology of severe hypoglycaemia give prevalences ranging from 2.7 to 85.7 episodes per 100 patients per year. The major risk factor for severe hypoglycaemia is hypoglycaemia unawareness, which occurs particularly in patients with type 1 diabetes of long duration and in those with a history of frequent episodes of hypoglycaemia. The first step in the management of hypoglycaemia is to check blood glucose and to treat hypoglycaemia on the basis of symptoms. Hypoglycaemia requires urgent treatment with a fast-acting carbohydrate or, if severe, with parenteral glucagon or intravenous glucose. Prevention measures should be instituted to prevent subsequent episodes, particularly in younger children with hypoglycaemic seizures or when seizures are recurrent.
Adolescent↗
Immunocytochemical localization of phospholipase C isozymes in cord blood and adult T-lymphocytes.
The response of T-cells to peptide antigen plus major histocompatibility complex (MHC) consists of a series of cellular events collectively called T-cell activation. An essential component of this pathway is phospholipase C (PLC)gamma1, whose hydrolytic activity increases rapidly after binding of ligands to the T-cell receptor (TCR) and consequent activation of tyrosine kinases. Recent studies also suggest a GTP binding protein-dependent activation of PLCbeta during the early steps of T-cell activation. On the basis of these findings, we first checked the expression of PLC isoforms by Western blotting and by confocal and electron microscopy techniques, and then we looked for the phosphoinositide breakdown induced by CD3 engagement in cord and adult T-lymphocytes. Our results indicated that PLCbeta1 was almost exclusively expressed in cord T-cells, whereas PLCbeta2 was more strongly represented in the adult. The amount of PLCgamma1 was found to be larger in the adult than in cord cells. No significant differences were found in PLCgamma2 and delta2 expression. PLCdelta1 was scarcely detectable. On CD3 stimulation, adult lymphocytes gave rise, as expected, to a dramatic increase in phosphoinositide breakdown, whereas in cord cells this response was scarcely detected. These results indicate that a shift in PLC expression occurs in the postnatal period and that this change is associated with induction of the capability to respond to CD3 engagement with phosphoinositide hydrolysis.
Adult↗
Epilepsy with myoclonic absences with early onset: a follow-up study.
We studied six children (four girls and two boys) suffering from cryptogenic myoclonic absence seizures with early onset. The age at onset of the seizures ranged between 6 and 27.8 months (mean age +/- SD: 18.5+/-12.4 months). The neurologic evaluation was normal in all patients at the first hospital admission. After the diagnosis, we followed up all children for at least 5 years. At the end of follow-up, two of these patients (a girl and a boy) showed severe mental retardation, a high number (from one to three per day) of seizures, and persistent pathologic electroencephalograms. The other patients showed normal electroencephalograms: all of them were seizure free and without mental retardation. The two patients with mental retardation have been treated with polytherapy. In all other children we used valproate alone successfully. Our data suggest that myoclonic absence seizures with early onset can have a good long-term prognosis. Valproate is a useful anticonvulsant drug in these patients. Mental retardation is present only in patients with poor seizure control.
Anticonvulsants↗
Novel evidence of expression and activity of ecto-phospholipase C gamma1 in human T lymphocytes.
Although much is known about the intracellular phospholipase C (PLC) specific for inositol phospholipids, few data are available about the presence of a less common PLC at the external side of the membrane bilayer of some cell types. This ectoenzyme seems to play particular roles in cellular function by hydrolyzing inositol lipids located on the outer leaflet of the plasma membrane. Here, we provide the first evidence that peripheral T lymphocytes express a discrete level of a PLCgamma1 at the outer leaflet of the plasma membrane. Flow cytometry showed that the PLCgamma1-positive (PLCgamma1(+)) cells (approximately 37%) were CD8(+) and CD45RA+. Biochemical evidence indicated that (1) this ectoenzyme displays a mass similar to the cytoplasmic form, (2) it is phosphorylated on tyrosine residues, and (3) its activity is Ca2+-dependent. In addition, this enzyme appeared to be correlated with the proliferative state of the cell, since stimulation with phytohemagglutinin (PHA) downregulated both its expression and activity, which were restored by treatment with an antiproliferative agent like natural interferon beta. Moreover, the different kinetics of formation of its hydrolytic products, inositol 1 phosphate and inositol 1:2 cyclic phosphate (Ins(1)P and Ins(1:2 cycl)P), formed upon incubation of the lymphocytes with [3H]-lyso-phosphatidylinositol (PI), allow the hypothesis of a selective involvement of the two inositol phosphates in the mechanisms regulating the metabolism of particular T-lymphocyte subsets.
Adult↗
Confirmation of the colobomatous macrophthalmia with microcornea syndrome: report of another family.
We report on the occurrence of microcornea, coloboma, and macrophthalmia in 4 generations of an Italian family. The patients had no additional physical anomalies, were of normal intelligence, and had a normal karyotype. This condition has been reported in only 1 family [Bateman and Maumenee, 1984: Ophthalmol Pediatr Genet 4:59-66]. The recurrence of this distinctive ocular pattern in our opinion confirms the existence of a new syndrome, with autosomal dominant inheritance.
Adult↗