Neonatal cutaneous disseminated aspergillosis in a preterm extremely-low-birth-weight infant with favourable outcome at 3-year follow-up: a case report.

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.

Mode of delivery and threshold retinopathy of prematurity in pre-term ELBW neonates.

BACKGROUND: Retinopathy of prematurity (ROP) is a multifactorial disease, but little is known about its relationship with perinatal risk factors. We tested the hypothesis that the mode of delivery may be associated with threshold ROP as defined by CRYO-ROP. METHODS: We conducted a prospective, cohort analysis of a database of all extremely low birth weight (ELBW) neonates (= birth weight < 1000 g) admitted over a 8-year period from 1997 to 2004 to a large tertiary neonatal intensive care unit in a urban area of northern Italy and screened for ROP. Incidence of threshold ROP was calculated for the whole studied population. The definition of threshold ROP was as defined by the CRYO-ROP study. Univariate analysis was performed to look for significant associations between threshold ROP and several possible associated factors, and among them, the mode of delivery (vaginal delivery or caesarean section). When an association was indicated by p < 0.05, multiple logistic regression was used to determine the factors significantly associated with ROP. RESULTS: Enrolled ELBW neonates were 174, and 46 of them (26.4%) displayed threshold ROP. Threshold ROP occurred in 40.9% (27 of 66) of the neonates vaginally delivered and in 17.5% (19 of 108) of those born via caesarean section (R.R. 3.35; 95% CI 1.230-4.855; p = 0.008 at univariate analysis, and = 0.04 at multivariate logistic regression after controlling for birth weight, gestational age, intraventricular haemorrhage grade 2 or more, days on supplemental oxygen, systemic fungal infection). Birth by vaginal delivery was not significantly associated with other major sequelae of prematurity (intraventricular haemorrhage, bronchopulmonary dysplasia, necrotizing enterocolitis). CONCLUSIONS: In our Institution birth by vaginal delivery is a significant and independent predictor of threshold ROP in ELBW infants. We suggest to consider closely ophthalmological surveillance for pre-term ELBW infants born this mode.

Oral supplementation with Lactobacillus casei subspecies rhamnosus prevents enteric colonization by Candida species in preterm neonates: a randomized study.

BACKGROUND: Colonization by Candida species is the most important predictor of the development of invasive fungal disease in preterm neonates, and the enteric reservoir is a major site of colonization. We evaluated the effectiveness of an orally supplemented probiotic (Lactobacillus casei subspecies rhamnosus; Dicoflor [Dicofarm spa]; 6 x 10(9) cfu/day) in the prevention of gastrointestinal colonization by Candida species in preterm, very low birth weight (i.e., < 1500-g) neonates during their stay in a neonatal intensive care unit. METHODS: Over a 12-month period, a prospective, randomized, blind, clinical trial that involved 80 preterm neonates with a very low birth weight was conducted in a large tertiary neonatal intensive care unit. During the first 3 days of life, the neonates were randomly assigned to receive either an oral probiotic added to human (maternal or pooled donors') milk (group A) or human milk alone (group B) for 6 weeks or until discharge from the NICU, if the neonate was discharged before 6 weeks. On a weekly basis, specimens obtained from various sites (i.e., oropharyngeal, stool, gastric aspirate, and rectal specimens) were collected from all patients for surveillance culture, to assess the occurrence and intensity of fungal colonization in the gastrointestinal tract. RESULTS: The incidence of fungal enteric colonization (with colonization defined as at least 1 positive culture result for specimens obtained from at least 1 site) was significantly lower in group A than in group B (23.1% vs. 48.8%; relative risk, 0.315 [95% confidence interval, 0.120-0.826]; P = .01). The numbers of fungal isolates obtained from each neonate (P = .005) and from each colonized patient (P = .005) were also lower in group A than in group B. L. casei subspecies rhamnosus was more effective in the subgroup of neonates with a birth weight of 1001-1500 g. There were no changes in the relative proportions of the different Candida strains. No adverse effects potentially associated with the probiotic were recorded. CONCLUSIONS: Orally administered L. casei subspecies rhamnosus significantly reduces the incidence and the intensity of enteric colonization by Candida species among very low birth weight neonates.

Fungal and bacterial sepsis and threshold ROP in preterm very low birth weight neonates.

OBJECTIVE: To determine whether an association exists between either fungal or bacterial sepsis and retinopathy of prematurity (ROP). STUDY DESIGN: Retrospective cohort study on all neonates with birth weight <1500 g admitted to a large Italian third Level Neonatal Intensive Care Unit in the years 1997-2001 and screened for ROP. Univariate analysis and multiple logistic regression were used to detect significant associations with ROP (all grades and threshold) in neonates with birth weight<1000 g (extremely low birth weight (ELBW)) and 1000-1500 g. RESULTS: Among 301 enrolled neonates, ROP (all grades), threshold ROP, fungal and bacterial sepsis occurred in 31.9, 12.9, 11.6 and 40.5% of the infants, respectively. At multivariate analysis, only gestational age (P=0.03), colonization by Candida non-albicans spp (P=0.03) and fungal sepsis (P=0.03) were independent predictors of threshold ROP, and only in ELBW neonates. CONCLUSIONS: Fungal (but not bacterial) sepsis is significantly and independently associated with ROP, but only in ELBW neonates and only with threshold ROP.

[Effectiveness of topical acetate tocopherol for the prevention and treatment of skin lesions in newborns: a 5 years experience in a 3rd level Italian Neonatal Intensive Care Unit]. / Efficacia del tocoferolo acetato (vitamina E) per uso topico nella prevenzione e nella terapia delle patologie cutanee nel neonato: esperienza di 5 anni in una grande unità di terapia intensiva neonatale.

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.

[G-CSF, neutropenia and candidiasis in VLBW patients]. / G-CSF, neutropenia e candidiasi nel VLBW.

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.

[Trisomy 6 with choanal atresia. The first Italian case]. / Trisomia 6 con atresia coanale. Primo caso Italiano.

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.

Poland syndrome: a case with a combination of syndromes.

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.

Prion-induced neuro-psychiatric syndromes.

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.

[An antioxidant in pediatrics]. / Un antiossidante in pediatria.

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.

[Prions and infantile pathology]. / Prioni e patologia infantile.

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

The diagnosis of so-called "constitutional" bone dysmorphias (embryonal osteochondropathies).

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.

[On the diagnosis of so-called "constitutional" bone diseases]. / Sulla diagnostica delle dismorfie ossee cosiddette "costituzionali".

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.

[Cytokines, immunostimulation and lactic enzymes]. / Citochine, immunostimolazione e lattofermenti.

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.

[Low cholesterol and pathological manifestations]. / Colesterolo basso e manifestazioni patologiche.

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.

Some objections to the international nomenclature for constitutional diseases of the bone.

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.

Criticism of the International Nomenclature of Constitutional Diseases of Bone.

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.

Dubowitz' syndrome with special characteristics.

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.