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Aims: Retrospective cohort study to assess if different patterns of Candida colonization determine different risks of progression to invasive fungal infection (IFI) in preterm neonates in NICU. Methods: Weekly surveillance cultures from all neonates weighing at birth <1500 g admitted over a 6-year period were reviewed. Infants with available results from at least 3 cultures/week and from at least 4 different sites were enrolled and identified by the number of sites involved [1-2 (low-grade), 3 or more (high-grade)] and type (low-risk, if colonization was recovered from skin, stool, ear canal swab, gastric aspirate, nasopharynx secretions, endotracheal tube; high-risk, from urine, catheter tip, drains, surgical devices). Progression rates from colonization to IFI were calculated for each subgroup. Univariate analysis was performed looking for significant associations between IFI and a number of risk factors, including the different subgroups of colonization. Multiple logistic regression assessed all significantly (P<0.05) associated risk factors. Main Results: In the 405 eligible infants, overall colonization rate was 42.9%, IFI rate 9.9%, overall progression rate to IFI 0.23, the latter being significantly higher in high-grade or high-risk than in low-grade or low-risk colonized infants (0.59 vs. 0.18, P=0.001; 0.44 vs. 0.11, P<0.001, respectively). Infants with concomitant high-grade + high-risk colonization had 4-fold higher risk of progression than any other colonized infant, and 7-fold higher risk than infants concomitantly low-grade + low-risk colonized (P<0.001). At multivariate analysis, high-grade and high-risk colonization (P=0.001 for both), birth weight (P=0.02) and presence of central venous line (P=0.04) remained independent predictors of IFI. Conclusions: Density and severity of fungal colonization condition the progression to IFI in preterm infants in NICU, and certain patterns of colonization are independent predictors of IFI. Increased culture surveillance and prophylactic measures should be addressed to preterm colonized infants in NICU featuring the most risky colonization patterns. [ABSTRACT FROM AUTHOR]

Summary: Invasive disseminated neonatal aspergillosis is an uncommon disease, with only scattered reports in literature in the last few years. Here we report on a 25-week gestational age, 730 g at birth preterm female infant who developed on day-of-life 10 multiple cutaneous exhulcerative lesions in her right arm, trunk and abdomen. Early recognition and diagnosis of these lesions as a due to cutaneous initial symptom of cutaneous disseminated aspergillosis, as well as prompt treatment with Liposomal amphotericin B + Itraconazole, secured successful recovery from the systemic infection. Skin lesions healed without any surgical treatment. The infant was discharged in good health. Long-term follow-up at three years of age revealed normality of all neurodevelopmental and cognitive parameters. To our knowledge, this is one of the very few cases of survival, free from sequelae, for a preterm infant affected by neonatal cutaneous disseminated aspergillosis. [ABSTRACT FROM AUTHOR]

Neutropenia is a major risk factor for bacterial colonization and sepsis in preterm neonates in the neonatal intensive care unit (NICU), but little is known about its relationships with candidal colonization (CC) in these settings. We performed a case-control study on neonates with birth weight of <1500 g admitted to our NICU during a 7-year period (1996-2003, N = 585). Through database search, infants with early-onset neutropenia (EON) (n = 68, group A) were identified and 1:1 matched with controls without EON (n = 68, group B). Microbiologic data from weekly surveillance cultures were examined to determine the presence and intensity of CC. Groups A and B were similar clinically and demographically. All group A neonates recovered from EON before the 8th day of life. Incidence of CC in the 1st month of life (at least 1 site) was significantly higher in group A (61.8% versus 35.3%, P = 0.002) and was not modified by treatment with recombinant granulocyte colony-stimulating factor. The same was true of CC intensity, expressed as the number of sites affected (P = 0.002). Incidence of candidal sepsis, mortality rates, and relative frequencies of the various subspecies of Candida among the isolates did not significantly differ between the 2 groups. In conclusion, EON in preterm neonates is a significant, independent risk factor for CC. Larger, prospective, adequately powered studies should verify whether increased CC related to neutropenia may translate into a similar increased occurrence of candidal sepsis in these settings.

Objective: Colonization by Candida spp is a major risk factor for development of fungal sepsis, but little is known about the variables associated with progression to invasive disease in already colonized neonates. We investigated such variables in a large number of colonized preterm neonates in an NICU., Setting: This study was conducted in the Department of Neonatology and the NICU at Sant'Anna Hospital in Torino, Italy., Design and Patients: A database search of clinical charts and weekly surveillance cultures was used to identify all neonates with birth weights < 1500 g (very low birth weight) who were admitted to our NICU during 1998-2005 and were colonized (> or = 1 site) by Candida spp during their stay, as well as infants with invasive fungal infection. The association between a number of factors with progression to invasive fungal infection was evaluated. Those shown to be significantly associated by univariate analysis were cross-checked by logistic regression., Results: Colonization occurred in 201 infants (32.1% of very low birth weight admitted neonates), and invasive fungal infection occurred in 51 (8.1%) of them, with an overall progression rate of 0.25. At univariate analysis, 10 factors (namely low birth weight, low gestational age, use of third-generation cephalosporins, endotracheal intubation, duration of stay in the NICU, bacterial sepsis, colonization of central venous catheter, of endotracheal tube, of gastric aspirate, or in > or = 3 [multiple] sites) were associated with an increased risk of progression, whereas prophylaxis with fluconazole was associated to a decreased risk. After logistic regression, only colonization of central venous catheter and colonization in multiple sites remained significantly associated with invasive fungal infection. Fluconazole prophylaxis remained an independent protective factor., Conclusions: Central venous catheter colonization and multiple-site colonization are independent risk factors and predictors of progression to fungal sepsis in preterm very low birth weight neonates colonized by Candida spp during their stay in the NICU. Fluconazole prophylaxis is an independent protective factor. These findings can be used to improve the surveillance, prophylaxis, or preemptive measures in neonates at high risk.

Aim: The incidence of invasive fungal infection in preterm newborns is rising steadily. Early recognition and treatment are imperative, but diagnosis is difficult as data from microbiological investigations are often poor, and clinical and laboratory signs do not help in differentiating bacterial from fungal infections. We evaluated whether glucose intolerance could represent a possible surrogate marker predictor of invasive fungal infection in preterm neonates., Methods: We performed a case-control study on neonates with birthweight less than 1250 g admitted to our tertiary-level unit during the years 1998-2004 (n = 383), comparing those with invasive fungal infection (n = 45, group A) to matched controls with late-onset sepsis caused by bacterial agents (n = 46, group B). We investigated in both groups the occurrence of hyperglycaemia (serum glycaemia > 215 mg/dl, i.e. 12 mmol/l) in the first month of life, and its temporal relationship with the episodes of sepsis., Results: Hyperglycaemia occurred significantly more often in group A (21/45, 46.6%) than in group B neonates (11/46, 23.9%) (OR 1.95, 95% CI 1.235-4.432, p = 0.008). Moreover, in 19 of 21 (90.4%) neonates with hyperglycaemia in group A, the carbohydrate intolerance episode typically occurred 72 h prior to the onset of invasive fungal infection; in contrast, no temporal relationship was found in neonates with bacterial sepsis (p = 0.002). Correction of hyperglycaemia was successfully achieved in all neonates of both groups, with no significant differences in the number of days of insulin treatment needed to normalize glycaemia (p = 0.15)., Conclusions: Hyperglycaemia is significantly more frequent in neonates who subsequently develop fungal rather than bacterial late-onset sepsis, with a typical 3-d interval. We suggest that a preterm neonate whose birthweight is less than 1250 g in its first month of life should be carefully evaluated for systemic fungal infection whenever signs of carbohydrate intolerance occur.

Aim: Neonates in NICU (especially when premature) are particularly prone to skin damage by action of external aggressive conditions such as chemical, physical, infectious, radiant, mechanical and iatrogenic factors. Strategies for avoiding disruption of the skin barrier are thus highly needed in such patients., Methods: We evaluated the effectiveness of a acetate tocopherol (AT) ointment for topical use in 21 neonates admitted to our NICU and affected by neonatal abstinence syndrome with severe diaper exulcerative and erosive erythema with ulcer and granulation tissue at the bottom of the lesion (group A), and compared them to 19 matched neonates affected by the same condition and treated with a commonly used skin ointment (emollient type, water-in-oil category) (group B). For all newborns we calculated: the dermatological severity score (using a clinical score from 0 to 9 points according to the increasing severity of the lesions) at time 0, 4 and 7 days; the mean days for achieving complete recovery; the rate of therapeutical failures., Results: Mean score at day 0 was 7.8 in group A vs 7.9 in group B (P=0.35 NS). At day 4 it was 4.6 in group A vs 6.5 in group B (P=0.03), at day in 7 it was 3.1 in group A vs 5.2 in group B (P=0.04). A complete recovery with restitutio ad integrum occurred after 9.1 mean days in group A vs 12.2 mean days in group B (P=0.04). The rate of therapeutical failures was significantly lower in group A (4.2% vs 30.6%; OR 0.235; P<0.01) than in group B. No adverse effects related to AT use were reported., Conclusions: AT in our experience proved to be safe and more effective than the commonly used skin ointments in the topical treatment of exulcerative skin lesions in NICU neonates.

Objectives: Relationships among Early Onset Neutropenia (EON), i.e. neutropenia in the first week of life, treated with Filgrastim, and subsequent colonization by Candida spp. with eventual systemic fungal infection in ELBW patients are not clear. We tried to analyze these features in a retrospective study on a selected population of a large tertiary NICU., Methods: By a database search, we identified a group of VLBW patients who were diagnosed a systemic fungal infection (SFI) during their stay in NICU (n=52), and divided them in two subgroups: those who had presented Early Onset Neutropenia (EON) and thus had been treated with a 3-day course of Filgrastim (n=14)(group A), and those who had not presented EON and thus had not undergone Filgrastim therapy (n=38) (group B). We investigated in both subgroups the following variables: neutrophil count monitoring during the first 2 weeks of life, colonization by Candida spp, day of onset of SFI, outcome. Statistical analysis was performed by Chi-square test, ANOVA and T-test using SPSS 8.0 for Windows., Results: Absolute neutrophil number was obviously lower in group A at recruitment (354/mmc vs 2910\mmc, Chi-square = 9.776, p <0.005), but became normal at the end of G-CSF treatment, thus detecting no significant differences between the two groups at day 8 (p<0.12) and 14 (p<0.34). The onset of SFI occurred significantly earlier ( 9.6 dol vs 14.6 dol., p<0.004) in group A neonates. Fungal Colonization rate in the 2nd week of life was significantly higher in previously neutropenic patients (71% vs. 37%, p< 0.005), who had also a significantly higher number of sites involved (p<0.003)., Conclusions: Neutropenia in the first days of life in VLBW neonates, even if adequately and succesfully treated, heavily influences rates and severity of colonization by fungal spp., and is associated with an earlier onset of a SFI.

The case of Poland syndrome reported incorporates a number of previously unrecorded features: hypoplasia of the left lung and agenesia of the epiglottis in addition to the standard symptoms. The case also presents certain features typical of the Moebius Syndrome.

The authors report the first Italian case (second in the world) of partial trisomy syndrome of chromosome 6. All the essential signs were present, in addition to choanal atresia, reported only once before in Italy. The phenomenological picture of this extremely rare anomaly is in the process of being completed by successive reports.

Although further research is needed, the most recent thinking is that prions are tiny protein particles without DNA or RNA which have the ability to infect humans or animals. Prions cause slow infections which are fatal, experimentally transmissible, evoke no immune resistance from the infected host and are more resistant to disinfectants and chemical or physical agents than the other micro-organisms that proliferate in the infected host. Update no evidence of possible passage by mouth from cows to men with consequent illness.

The authors review the state of the art regarding vitamin E in relation to its use during childhood. The report comments on a number of aspects which are still not entirely clear and stresses the prophylactic and therapeutic importance of antioxidants, even in pediatrics, and the severe damage that may result from free radicals even during this period of life.

The authors briefly review the problem of prions pathology (with particular emphasis on children). The outstanding interest of the subject justifies this review.

The authors review the diagnosis of so-called "consitutional" bone diseases. They criticise the international nomenclature and classification of the latter and propose a diagnostic classification for embryonal osteochondropathies, which is virtually independent of the structural and metabolic criteria, based on the criterion of site and the extent of pathological manifestations. They also examine the term "dysmorphia" given that it is the shape of the bone segment which first attracts attention and guides the clinical diagnosis of embryonal osteochrondropathies.

The authors review the diagnosis process of so-called "constitutional" bone diseases. They criticize the nomenclature and international classification of them and suggest a diagnostic classification which is virtually independent of structural and metabolic criteria: based on the site and extent of pathological manifestations. The paper also examines the term "dysmorphia", given that it is the shape of the bone segment which initially attracts and guides the clinical diagnosis of embryonal osteochondropathies and suggest it.

On the basis of numerous experimental studies the authors advance the hypothesis of the use of lactoferments for immunomodulating purposes. This entails using a traditional and innocuous method for infantile prophylaxis and therapy, (adapted for immunodepressive and even iatrogenic syndromes) which might be assigned a new role through modern, large-scale experimental research.

The paper reviews the literature on the association between hypocholesterolemia and various pathologies. There are few studies on this topic and these are often fragmented and uncertain, in particular with regard to children. However, studies with a wide statistical basis have focused on the different stages of adulthood. There does not appear to be a genuine link between "low cholesterol" and disease in childhood, although the contrary would appear to be true. The authors invite pediatricians to examine this aspect further in view of the major repercussions that it may have on a wide range of factors. For this reason the authors consider it worthwhile presenting this innovative topic and field of research.

The International Nomenclature of Constitutional Diseases of Bone is criticised and the concept of embryonic osteochondropathy is proposed as an alternative approach. It is claimed for example that the term "constitutional" has no real scientific or, above all, medical meaning. The defects of this "official" nomenclature are highlighted and a slimmer classification system of use in the diagnosis of osteochondroblastic mesenchymopathies is recommended.

The International Nomenclature of Constitutional Diseases of Bone is criticised and the concept of embryonic osteochondropathy is proposed as an alternative approach. It is claimed for example that the term "constitutional" has no real scientific or, above all, medical meaning. The defects of this "official" nomenclature are highlighted and a slimmer classification system of use in the diagnosis of osteochondroblastic mesenchymopathies is recommended.

The Authors describe the main characteristics of Dubowitz' syndrome: retarded growth, microcephaly, cranio-facial deformations and dysmorphia of the extremities, psycho-motor development varying between normal and retarded, thin hair, cryptorchism, hyperactivity and others. They report a case which was brought to their attention in which, in addition to numerous anomalies and dysmorphia which justify its inclusion in Dubowitz' syndrome, there was marked bilateral metacarpal hypoplasia with strongly and permanently adducted thumbs. The aim of this report was to add to the mosaic of data relating to this highly polymorphous syndrome.

The problem of some aspects of infantile atherosclerosis is examined synthetically (period of onset, classification of hyperdyslipidemia, lipidic fractions, threshold values, variations with age) and prevention examined at length. In particular, stress is laid on a "mixed" mass screening method to prevent many subjects being untouched by targeted screening investigation.

The author makes a few diagnostic comments regarding the so-called constitutional bone diseases, quoting the international nomenclature and a classification which is thought to be useful for diagnosis, based on clinical and radiological criteria rather than purely structural and metabolic findings.

The paper reports a case of a 6-year-old boy affected by visceral leishmaniasis. In addition to clinical and anamnestic findings, diagnosis was made on the basis of the rapid increase in the titer of anti-Leishmania antibodies (16-fold), while the marrow smear was negative. A complete recovery was obtained using antimonial drugs.

The paper describes a case of bilateral polycystic kidney in a 14-year-old whose father and 3 brothers are also affected by bilateral renal cystic dysplasia (as is a paternal aunt), while a paternal uncle is affected by constant hematuria with no ultrasound signs of renal cystic dysplasia. The case in question is an adult type of dominant autosomal polycystic renal dysplasia, affecting all the males in the family and also a female within the family nucleus. The case is described in the light of the most recent reports on the subject and the problem of prevention is also discussed.

The authors report a case of "harlequin fetus" which was brought to their attention. The report was justified not only by the rarity of the disease, but above all because, after having examined the literature on the topic, this appears to be the third case treated with etretinate. The case in question was a female affected by congenital ichthyosis in its total form and by initial necrosis of the extremities. Etretinate treatment gave good results in terms of cutaneous modifications: reduction of hyperkeratosis, disappearance of ectropion and eclabium. Therefore, although the patient died when 16 days old due to sudden renal insufficiency, the case confirms the two previous reports on the value of this therapy.

Hospitals in communist China perfected a new acupuncture technique about 3 yrs ago, whereby needles are placed in the scalp to stimulate the cortical centres below. This method is particularly indicated in subjects with neurological damage. Results observed in 45 subjects with cerebral vasculopathy at the neurological clinic of Pisa University and the reflexotherapy service of the University of Turin were encouraging and suggest that clinical experimentation should be attempted on a wider scale.

A preliminary undiagnosed case of partial carbamyl-phosphate-synthetase deficiency in a 14 year old patient is described. This extremely rare metabolic disorder is unlikely to produce clinical symptoms at such an advanced age. Details are given of the clinical picture, the diagnosis (by liver biopsy and post mortem liver examination) and the attempts at treatment.

The evaluation of serum ferritin level in a population of 30 three months old babies is made by the Authors following a radioimmunological method. The reckoned average values reflect exactly the wellknown elements of iron kinetics during the first months of life. The Authors underline that those values show how complete is breast-feeding; moreover they emphasize the importance of a referring value which, together with those already known in subjects of different ages, allows on early diagnosis of syndromes due to iron decrease and subsequent check-up after therapeutic trial.

Unlabelled: 20% of unweaned children aged below three months suffer from so-called gaseous colic. The effectiveness and tolerance of an antimuscarinic drug with high spasmolytic activity on the smooth visceral musculature, cimetropium bromide, has been evaluated, excluding cases of intolerance to cow's milk and other pathologies. 40 random patients of both sexes of average age 4.4 weeks (symptoms lasting for one week with crying fits lasting for more than 90 minutes consecutively, on at least 3 days a week) split into two groups of 20 patients each were studied., Treatment: A) 1.2 mg/kg 1 hour before bottle; B) 2.0 mg/kg 1 hour before bottle. Speedy reduction in the number of crying episodes (Group A: from 2.8 +/- 0.3 to 0.1 +/- 0.1; Group B: from 2.8 +/- 0.4 to 0.6 +/- 0.4; differences n.s.) and in their duration (Group A: 99 min +/- 10 min to 5 min +/- 3 min; Group B: from 121 min +/- 11 min to 15 min +/- 9 min; differences n.s.). The pharmacological treatment was considered: Group A: very good in 75% of cases; Group B: very good in 70% of cases. In Group B 4 episodes of stypsis occurred and these resolved immediately upon suspension of the drug. Given the equal effectiveness and better tolerance, the use of cimetropium bromide is recommended at the lower dosage.

On the basis of a study of the innervation of the cutaneous areas stimulated in cranial acupuncture, a neurophysiological classification of the technique is proposed, considering the bulbar level as the first site of integration of the afferent vessels activated by cranial acupuncture.