Use OF NBI for the assessment of clinical signs of rhino-pharyngo-laryngeal reflux in pediatric age: Preliminary results.

OBJECTIVES: Laryngopharyngeal reflux (LPR) is a complex problem in pediatric population: diagnosis and clinical presentation are still controversial. Classic white light endoscopy shows some pathognomonic signs of LPR in children, such as thickening of pharyngo-laryngeal mucus, the cobblestoning aspect of pharyngeal mucosa, arytenoid edema/hyperemia, nodular thickening/true vocal cord edema, hypertrophy of the posterior commissure, subglottic edema. The NBI (Narrow Band Imaging) technology, generally used in oncology, allows to study neoangiogenesis and hypervascularization of the mucosa, common aspects in both chronic inflammation and neoplastic transformation. The aim of our study was to evaluate the added value of this technology in identifying the main laryngopharyngeal reflux sign in a pediatric population. METHODS: We evaluated at the Otolaryngology Unit of the "Fondazione Policlinico A. Gemelli" hospital and the Airway Surgery Unit of the "Bambino Gesù" Children's Hospital 35 patients aged from 2 months to 16 years divided into two groups in the period between November 2017 and May 2018. Group A included patients with clinical suspicion of LPR after gastroenterologist evaluation and Group B included patients who underwent an endoscopic evaluation for the assessment of recurrent respiratory symptoms such as stridor, recurrent croup, wheezing and persistent cough. We performed an endoscopic evaluation by white light and NBI for each patient, comparing the results of both methods to evaluate signs of pharyngo-laryngeal reflux and to calculate the value of reflux finding score (RFS). RESULTS: The analysis of the data showed: for Group A an average value of RFS with white light of 11,84 (range 8-17, standard deviation 2,52 ±â€¯0,57) and with NBI of 13,63 (range 10-17, standard deviation 2,13 ±â€¯0,49); for Group B the analysis of the data showed an average value of RFS with white light of 10,06 (range 8-14, standard deviation 2,32 ±â€¯0,58) and with NBI of 12,50 (range 9-18, standard deviation 2,63 ±â€¯0,65). The comparison between the two methods resulted significant. Furthermore evaluation by NBI allowed to highlight other signs of pharyngo-laryngeal reflux, characteristic of pediatric age and not included in RFS, in particular cobblestone aspect of the hypopharingeal mucosa, phlogosis of the tonsillar crypts and adenoid surface, hyperemia and hypervascularization of subglottic and tracheal mucosa. CONCLUSION: Although still preliminary our results represent an interesting starting point for further studies, because they underline the potentiality of NBI endoscopy in LPR evaluation and how this technology could improve the identification of reflux signs.

Emerging and re-emerging infectious disease in otorhinolaryngology.

SUMMARY: Emerging and re-emerging infectious disease in otorhinolaryngology (ENT) are an area of growing epidemiological and clinical interest. The aim of this section is to comprehensively report on the epidemiology of key infectious disease in otorhinolaryngology, reporting on their burden at the national and international level, expanding of the need of promoting and implementing preventive interventions, and the rationale of applying evidence-based, effective and cost- effective diagnostic, curative and preventive approaches. In particular, we focus on i) ENT viral infections (HIV, Epstein-Barr virus, Human Papilloma virus), retrieving the available evidence on their oncogenic potential; ii) typical and atypical mycobacteria infections; iii) non-specific granulomatous lymphadenopathy; iv) emerging paediatric ENT infectious diseases and the prevention of their complications; v) the growing burden of antimicrobial resistance in ENT and the strategies for its control in different clinical settings. We conclude by outlining knowledge gaps and action needed in ENT infectious diseases research and clinical practice and we make references to economic analysis in the field of ENT infectious diseases prevention and care.

Laryngotracheal reconstruction in glottic-subglottic stenosis associated with DiGeorge syndrome in a four and a half-month-old infant.

Neonatal subglottic stenosis still remains a substantial challenge for paediatric ENT surgeons. Herein, we present a case of a single stage laryngotracheal reconstruction for a glottic-subglottic stenosis in an 18-week-old, 7.2 kg infant with DiGeorge syndrome. Our surgical approach was compared with those reported in the literature. Paediatric airway surgery should be tailored to individual patients according to age, weight, comorbidities and family collaboration, with the ultimate objective to minimise the invasiveness of the procedure.

Resolution of opisthotonus in respiratory distress by aortopexy.

We report a case of worsening respiratory distress associated with opisthotonus secondary to tracheomalacia, a rather unique pathophysiological phenomenon. A 2-month-old male baby was referred to our hospital for respiratory distress syndrome with a noticeable opisthotonus. Examination and investigation confirmed the presence of an aberrant innominate artery compressing the trachea. The infant underwent aortopexy and made a dramatic post-operative recovery. Of special note, the opisthotonus vanished soon after the operation. Opisthotonus is not always related to neurological impairment and may be a warning sign of mediastinal overcrowding in patients with respiratory distress syndrome secondary to vascular compression.

Parapharyngeal neuroglial heterotopia in Pierre Robin sequence: MR imaging findings.

Heterotopic neuroglial tissue is a rare lesion, occurring more frequently in the nasal cavities. Other rare locations are the orbit, the scalp, the palate, the pharynx, the parapharyngeal space and the lungs. They are usually detected occasionally because they are often asymptomatic, but sometimes they might present with dyspnoea, feeding difficulty, snorting and nasal flaring. Respiratory symptoms occur when heterotopic neuroglial tissue is located in the parapharyngeal space. We report a case of an infant affected by Pierre Robin sequence (PRS) who was admitted to our Institution for a worsening respiratory distress that was not explainable only by PRS.

In vivo exposure of carp to graded concentrations of bisphenol A.

A wide range of environmental pollutants commonly termed endocrine disrupters (ED) can mimic steroid hormones causing adverse health effects. Recent studies showed that bisphenol A (BPA) together with other estrogenic chemicals, may be responsible for the disrupting effects observed in fish of the middle River Po. The present study has investigated the potential role of BPA in inducing this kind of effects and to this aim, 1-year-old (k1) common carp were exposed for 14 days to graded concentrations of BPA (1, 10, 100, and 1000microg/L). Histological alterations of gonads were described and compared to vitellogenin (VTG) and sex steroid levels. In carp males, BPA caused severe alterations of testis structure starting from 1microg/L. Several specimens lost the typical lobular structure showing spermatogenic cysts intermingled with free spermatozoa often degenerating into the lumen. Oocyte atresia was observed starting from 1micro BPA/L, concerning 57.1% of females at the highest concentration (1000microg BPA/L). A few carp from the same highest treatment also showed intersexuality (27%). Oestradiol-17beta (E2) significantly decreased both in 1 and 10microg BPA/L exposed carp reaching again values not significantly different from control in 1000microg BPA/L. At this concentration, a significant reduction of testosterone (T) was observed in both males and females. The mean plasma concentration of VTG significantly increased in 1000microg BPA/L exposed carp, even if a growing number of responsive carp was observed starting from the environmental concentrations (1 and 10microg BPA/L).

Investigating the estrogenic risk along the river Po and its intermediate section.

Different endpoints have been used to investigate the occurrence of estrogenic risk along the Po River, particularly its middle section. An in vitro assay based on recombinant yeast could not detect estrogenic activity in bed sediments of the Italian river or in bile samples of five Cyprinid species, with the only exception being one carp (Cyprinus carpio) captured downstream of the River Lambro, a polluted tributary of the middle River Po. Chemical analyses of fish bile and water samples from the same middle section showed diffuse contamination by moderately low levels of estrogenic chemicals (estrone [E1], 17beta-estradiol, estriol [E3], 17alpha-ethinylestradiol, 4-nonylphenol [NP], 4-tert-octylphenol [tOP], 4-n-octylphenol, and bisphenol A) but they were of limited help in understanding the risk present in the downstream area where intersex barbel were previously found. In contrast, the analyses of River Lambro waters showed that this tributary is a source to the middle River Po of all eight estrogens investigated. Analyses of bed sediments and macroinvertebrates from the same area consistently showed at least two levels of contamination, with the downstream stretch showing higher concentrations of natural steroids (E1 and E3) and xenoestrogens (NP and tOP). Accordingly, new histologic examinations undertaken on young barbel (Barbus sp.) showed intersex gonads only in the individuals captured in the downstream stretch, thereby confirming previous results. Present findings confirm the occurrence of disrupting conditions in the middle River Po and provide the first suggestions of cause-effect relationships.

In vivo and in vitro exposures of carp and carp tissues to graded concentrations of endocrine disrupting chemicals.

The objective of this study was to correlate morphological and biochemical parameters of reproductive activity in fish exposed to graded concentrations of endocrine disrupting chemicals. Two-year-old carps were exposed for two weeks to graded concentrations of ethynyl-estradiol, tamoxifen, and flutamide. For each chemical, morphological alterations of the gonads and liver were described according to biomarker and sex steroid levels. In parallel, isolated follicular cells (FCs) and testis fragments were incubated in the presence or absence of carp pituitary homogenate and 25-hydroxycholesterol. Media were analyzed for sex steroid level evaluations.

Fish community characterization in two stretches upstream and downstream of the Lambro River confluence with the Po River.

This study aimed to characterize the fish community in two stretches of the Po River, upstream and downstream of the Lambro River confluence, to verify the environmental effects of the presence of endocrine disrupters (EDs). More than 5000 specimens were captured, identified at the species level, and recorded for biometric parameters. Plasma, liver, and gonad fragments were sampled for histological and biochemical analyses. Few specimens belonging to carp and barbel showed gonads with the concomitant presence of male and female tissues and altered plasma levels of vitellogenin and sex steroids; thus, these species will be considered for successive environmental studies.

Histological and histochemical study of female germ cell development in the dusky grouper Epinephelus marginatus (Lowe, 1834).

The developmental stages of female germ cells were analysed in a wild population of the protogynous teleost Epinephelus marginatus (Lowe, 1834). 321 wild dusky grouper females were collected in the South Mediterranean Sea during the spawning season and their ovaries analysed using histological and histochemical techniques. Oocyte morphology, nucleus-cytoplasm ratio (N/C) range, location and movements of cytoplasmic inclusions during primary growth, vitellogenesis and final oocyte maturation were described. The distribution of proteins, lipids and carbohydrates through oocyte development was also investigated in 50 females. Lipid vesicles appeared firstly in the mid ooplasm of oocytes larger than 130 microm, at the beginning of the secondary growth phase. Immediately afterwards, small carbohydrate granules (PAS and Alcian blue positive) appeared before the occurrence of the first yolk granules. Tyrosine-enriched proteins were especially evidenced in the zona radiata interna of late vitellogenic oocytes. Specific lectin binding patterns reflected characteristic differences in the content and distribution of specific sugar moieties expressed in the oocytes during vitellogenesis and final maturation. At the end of vitellogenesis and during final maturation, follicular cells, zona radiata, and cortical alveoli were characterised by a strong increase of specific binding for WGA.

First observation of intersex cyprinids in the Po River (Italy).

Barbel (Barbus plebejus, Cyprinidae) were captured in the Po River, upstream and downstream from the confluence of the Lambro River, a polluted tributary of the major Italian watercourse. The gonads of the two groups of barbel have been histologically examined, and only the downstream specimens showed histo-morphological alterations that can be related to the Lambro tributary as a source to the main river of endocrine disrupting chemicals, possibly with estrogenic effects. In fact, 50% of the barbel captured (8 of 16 fish) in the downstream reach showed intersex gonads.

Intravenous immune globulin in lysinuric protein intolerance.

In addition to systemic manifestations with skeletal, pulmonary, renal, and haematological signs, lysinuric protein intolerance (LPI), a membrane transport defect of cationic amino acids, is often complicated by severe life-threatening immunological manifestations. A 10-year-old boy with LPI who exhibited a severe systemic immunohaematological disease is described here. This patient showed cutaneous lesions similar to the subacute form of systemic lupus erythematosus, severe anaemia and dysproteinaemia, and a marked reduction of circulating T lymphocytes, mainly the CD4+ cells. In vitro bone marrow cell culture studies showed that addition of patient's serum induced macrophage proliferation and inhibited erythroid progenitor cell growth. Treatment with high-dose intravenous immune globulin resolved most of the clinical and laboratory abnormalities.

[A rare case of endotympanic hemangioma]. / Su di un raro caso di emangioma endotimpanico.

Hemangiomas affectioning the external ear canal or the tympanic membrane have rarely been described. Only a very few cases are reported in world literature conarn these vascular tumors involving the middle ear space. The Authors report a rare case of a right endotympanic hemangioma associated with ossicular dysmorphism, resulting in conductive unilateral hearing loss, in a 9 year-old girl with cutaneous hemangiomas of the face. The Authors review Literature and discuss the possible pathogenetic implications of the intraoperatory findings.

Acinic cell carcinoma of the parotid gland in childhood.

A 13-year-old girl underwent surgery at the Ear Nose and Throat Department of the Children's Hospital and Scientific Research Institute, Bambino Gesù, Rome, because of an acinic cell carcinoma of the parotid gland. Two years after total parotidectomy with preservation of the facial nerve, metastasis occurred in a lateral cervical lymph node. Acinic cell tumors are uncommon in childhood; in children under the age of 16 years only 35 cases have been reported. There are too few acinar cell carcinomas occurring in children to yield an idea of their natural course. The neoplasm is also referred to as 'acinar cell tumor', in contrast to acinar cell carcinoma, because the neoplasm may be benign or malignant. Unfortunately, there are no histological distinguishing features that permit the pathologist to determine which neoplasm will behave in an aggressive fashion.

[Bronchoalveolar lavage (BAL) in the diagnosis of pulmonary infections in immunocompromised children]. / Il lavaggio bronco-alveolare (BAL) nella diagnostica delle infezioni polmonari nei bambini immunocompromessi.

The authors report their personal case records of broncho-alveolar lavage in immuno-compromised children. 22 children were observed from 1991 to 1992 at the E.N.T. Department of the Bambino Gesù Hospital, National Research Institute, in Rome. Flexible bronchoscopy is used under both local and general anesthesia without muscle relaxants. The epithelial lining fluid collection is greater than that obtained when muscle relaxation is used. For each patient 3 specimens are collected: for virological, bacteriological, mycological and immunological analysis. In 90% of the cases a correct etiological diagnosis of the pulmonary disease was possible. In 14 cases the diagnosis was possible even after a week from the beginning of the antibiotic therapy.

[Hematic infiltrates and the expression of HLA-DR antigens in naso-sinusoidal polyps of the fibrous type]. / L' infiltrato ematico e la espressione di antigeni HLA-DR in polipi naso-sinusali di tipo fibroso.

In this study fibrous nasal polyps, obtained from four patients, were analyzed by means of immunocytochemical methods for the presence of interstitial hematic cell infiltrate and HLA-DR molecule expression. This histologic type accounted for 36.4% of nasal polyps studied. Our results demonstrated that cells belonging to monocyte-macrophage lineage were mainly detected within fibrous and edematous zones (greater than 50%), whereas T cells were found within the subepithelial peripheral connective tissue (greater than 60%). Surface and gland epithelial cells appeared to be more intensely stained for HLA-DR molecules than nasal epithelium of normal subjects, thus indicating that the intensity of HLA-DR molecule expression correlated with the presence of a hematic cell infiltrate. Nasal polyps are a frequent pathology whose etiology has not yet been completely clarified. The present study provides additional information about the fibrous polyp structure and can support some speculations on the nasal polyp etiology.

[Pancreatic passenger leukocytes in organs obtained from cadaver donors for allotransplantation]. / I "passenger leukocytes" pancreatici in organi prelevati da donatore cadavere per allotrapianto.

In previous studies we have demonstrated that human normal pancreata were populated by interstitial mononuclear cells constituted by dendritic cells (70%), macrophages (30%) and few B cells (less than 1%). Furthermore, the endocrine parenchyma and the centroacinar cells appeared negative for the detection of HLA-A,B,C molecules. In the present report, 15 pancreata from cadaver donors were studied by means of immunohistochemical methods. 5 out of 15 organs were found to contain a larger amount of interstitial hematic cells that were constituted mainly by macrophages and B cells, together with a small percentage of granulocytes and T cells. In such "infiltrated" pancreata both endocrine parenchyma and centroacinar cells displayed a positivity for HLA-A,B,C antigens. As HLA-class I antigens have importance in allo-recognition and rejection responses, our data suggest that pre-transplant biopsies may be useful for a better evaluation of the pancreatic tissue immunogenicity.

[Modulation of the expression of histocompatibility antigens class I (HLA-A,B,C) in the human pancreas]. / Modulazione della espressione di antigeni di istocompatibilità di classe I (HLA-A,B,C) nel pancreas dell'uomo.

In this study the expression of HLA class I molecules was analysed on caudal portions of ten pancreata from cadaver donors by means of indirect immunoperoxidase and immunophosphatase techniques. In 7 out of ten pancreata the results showed that islets tissue was almost negative for the expression of HLA Class I antigens as opposed to exocrine tissue that appeared positive. Within exocrine tissue and large sized islets strongly positive interstitial cells were also detected. Double stainings showed that the strongly positive interstitial cells expressed also Leu M1 antigens. Preliminary studies on the remaining three pancreata demonstrate an increase of hematic interstitial cells together with a parallel increase of HLA class I antigens by endocrine parenchyma. The above data suggest that an increase of interstitial cells within pancreas may influence islets antigenicity.

The pathogenesis of chronic recurrent parotitis in infants: a study of 93 cases including an analysis of the vascular and glandular changes before and after parasympathectomy.

Ninety-three cases of infantile chronic recurrent parotitis are recorded. Fifty-three cases were treated with parasympathectomy which was carried out at 2 levels. Good results were achieved in 79.1% of patients. The paper describes the use of the Doppler examination to study the effects of operation on the physiology of the parotid.