Effectiveness of surgical treatment of severe macrocheilia in a patient with orofacial granulomatosis.

Orofacial granulomatosis (OFG) is the term given to a group of diseases characterized by the presence of non-necrotizing granulomatous inflammation affecting the soft tissues of the orofacial region. Treatment of OFG is often challenging and unsatisfactory. We report on a 32-year-old man with a 2-year history of oedema and swelling of the upper lip without systemic symptoms. The history, clinical features and histopathological findings led to the diagnosis of cheilitis granulomatosa (CG), a disease included in the spectrum of OFG. The patient was treated with oral diaminodiphenyl sulfone (DDS) and clofazimine without success. Oral doxycycline led to a slight improvement of the disease. Because the volume of the upper lip was twice normal size, surgical reduction was performed, followed by administration of oral doxycycline for 3 months. This therapeutic approach led to complete remission, with no recurrence after 3 years.

Post-surgical lipophagic panniculitis: a specific model of traumatic panniculitis and new histopathological findings.

Aim of this work was to define the histopathological features of post-surgical panniculitis. Dermal and hypodermal changes will be analyzed in detail, to understand the cascade of events that characterize the tissue response to surgical trauma. Cutaneous re-excision specimens of cases of basal cell carcinoma, squamous cell carcinoma, and melanoma consecutively seen from January 1, 2011 to June 30, 2011 at the Department of Dermatology, University of Pavia, were included in this study. Only the cases in which the first surgical procedure included the subcutaneous fat, were considered. In addition, the time elapsed from the first surgical procedure and the re-excision had to be included in a period of time from one to three months. All the specimens were stained with hematoxylin and eosin. Thirty cutaneous re-excision specimens were studied. Histopathologic examination revealed changes of epidermis, ranging from slight atrophy to moderate hyperplasia. In two cases focal ulceration was seen, with transfollicular elimination of foreign body material. The main dermal changes observed were the: 1) scar with well defined vertical orientation along the dermal suture line; 2) rounded cicatricial areas with radial branching septa of scarring tissue; 3) foreign body granuloma formation; 4) alignment of hystiocytes at the dermo-hypodermal border; 5) traumatic neuromas. The subcutaneous fat changes included: 1) lobular panniculitis with consistent presence of foam cells; 2) striking anisocytosis with pseudocystic degeneration and necrosis of adipocytes; 3) eritrocyte extravasation, mainly at the dermo-hypodermal border; 4) deep seated phlebitis. Post-surgical panniculitis is a lobular foam cell panniculitis characterized by simultaneous dermal and hypodermal changes, expression of the multi-faceted tissue response to a surgical trauma. This type of peculiar lipophagic response puts post-surgical panniculitis into the wider chapter of lipophage tissue response seen in atherosclerosis, glomerulosclerosis and some infectious models such as Mycobacterium tuberculosis and Chlamydia pneumoniae infections. Furthermore it may be seen as a reliable and convenient model for laboratory investigation on foam cell tissue response.

The almond milk: a new approach to the management of cow-milk allergy/intolerance in infants.

AIM: Elimination of the offending food is imperative in the management of children with cow-milk allergy/intolerance (CMA/CMI). Herein we report the result of randomized clinical trial carried out to test the efficacy and safety of a new almond-based food (hereinafter named almond milk) in a group of infant with CMI/CMA. METHODS: A group of 52 infants aged 5 to 9 months and with documented CMI/CMA was enrolled and randomized to: almond milk (Group A, n=26); soy-based formula (Group B, n=13); protein hydrolysate-based formula (n=13). The main efficacy outcomes were the improvement in clinical symptoms and the decrease in serum levels of soluble CD30 (a potential marker for atopic disorders; sCD30). RESULTS: Elimination of the offending food and supplementation with a milk protein-free formula produced a considerable improvement of clinical manifestations within 5-12 days in all cases examined (at the onset of the study: 26.4+/-5.4 U/mL and 7.9+/-5.2 U/mL in IgE+ and IgE- infants respectively, after 6 months of supplementation: 16.6+/-4.8 U/mL and 7.1+/-4.5 U/mL in IgE+ and IgE- infants respectively). No difference in growth rate (increment of weight, length and head circumference) was found, during the entire study, between infants given the almond milk and babies given the soy-based formula or the protein hydrolysate-based formula. Supplementation with the soy-based and protein hydrolysate-based formulas caused the development, in some subjects, of a secondary sensitization (23% to soy-based and 15% protein hydrolysate-based formula), whereas supplementation with the almond milk did not. CONCLUSIONS: Though preliminary, the present findings seem to demonstrate that the almond milk may an efficacious substitute of cow milk in infants with CMA/CMI. One could speculate that some active principles contained in the almond milk could contribute to its beneficial effect observed in CMI/CMA-affected infants.

Serum levels of malondialdehyde and 4-hydroxy-2,3-nonenal in patients affected by familial chronic nail candidiasis.

Familial chronic nail candidiasis (FCNC.MIM 607644) is a rare disorder characterized by early onset infections caused by different species of Candida and restricted to the nails; this disorder is genetically associated with low serum concentration of intercellular adhesion molecule 1 (ICAM-1). Herein we report the evidence of high circulating levels of malondialdehyde (MDA) and 4-hydroxy-2,3-nonenal (HNE) in seven patients of a five-generation Italian family affected by FCNC.MIM 607644. The present data evidence, in these patients, an increase in circulating MDA and HNE levels. Only some merely speculative hypotheses may be suggested to explain the mechanisms subserving the oxidative stress condition observed in these genetically ICAM-1 deficient patients; however, one has to point out that a chronic oxidative stress condition could contribute to the development of concurrent pathological alterations in which an overproduction of free radicals may play a central role.

Chromosome mapping and identification of amphiphilic proteins of hexaploid wheat kernels.

Amphiphilic proteomic analysis was carried out on the ITMI (International Triticae Mapping Population) population resulting from a cross between "Synthetic", i.e.: "W7984" and "Opata". Out of a total of 446 spots, 170 were specific to either of the two parents, and 276 were common to both. Preliminary analysis, which was performed on 80 progenies (Amiour et al. 2002a), was completed here using a total of 101 selfed lines. Seventy two Loci of amphiphilic spots placed at LOD = 5 were conclusively assigned to 15 chromosomes. Some spots mapped during the first analysis were eliminated because of the significant distortion segregation observed in the second analysis. Group-1 chromosomes had by far the greatest number of mapped spots (51). Using the Quantitative Trait Loci (QTLs) approach, analysis of the quantitative variation of each spot revealed that 96 spots out of the 170 specific ones showed at least one Protein Quantity Locus (PQL). These PQLs were distributed throughout the genome. With Matrix Laser Desorption Ionisation Time Of Flight (MALDI-TOF) spectrometry and Database interrogation, a total of 93 specific and 41 common spots were identified. This enabled us to show that the majority of these proteins are associated with membranes and/or play a role in plant defence against external invasions. Using multiple-regression analysis, other amphiphilic proteins, in addition to puroindolines, were shown to be involved in variation in kernel hardness in the ITMI population.

Protein carbonyl group content in patients affected by familiar chronic nail candidiasis.

Familiar chronic nail candidiasis (FCNC) is a rare disorder characterized by early-onset infections caused by different species of Candida, restricted to the nail of the hands and feet, and associated with a low serum concentration of intercellular adhesion molecule 1. Host defense mechanisms against candidiasis require the cooperation of many immune cells through several candidacidal mechanisms, including oxygen-dependent killing mechanisms, mediated by a superoxide anion radical myeloperoxidase--H2O2--halide system, and reactive nitrogen intermediates. We analyzed protein carbonyl groups (considered a useful marker of oxidative stress) in the serum of patients belonging to a five-generation Italian family with an isolated form of FCNC. Serum protein carbonyl groups in FCNC patients were significantly lower than those measured in healthy donors. Also, if this hypothesis is merely speculative, we could suggest that the decreased circulating level of protein carbonyl groups in these patients is not a marker of a lower oxidative stress condition, but might be linked to a lower protease activity.

A gene for familial isolated chronic nail candidiasis maps to chromosome 11p12-q12.1.

Chronic mucocutaneous candidiases (CMC) are a group of rare disorders where an altered immune response against Candida leads to persistent and/or recurrent infections of the skin, nails, and mucous membranes. We analysed a five-generation Italian family with an isolated form of CMC, affecting nails only, in the presence of low serum concentration of intercellular adhesion molecule I (ICAM-1). We excluded linkage to candidate regions on chromosomes 2p (CMC with thyroid disease), 21q22.3 (APECED), and 19q13 (ICAM-1). We then carried out a genome-wide scan and assigned the CMC locus to a 19 cM pericentromeric region on chromosome 11.

Monocyte and neutrophil activity after minor surgical stress.

BACKGROUND/PURPOSE: Surgical stress produces changes in the immune status of patients. In adults, major surgery causes immunosuppression, whereas minor operations stimulate immune responses. In children, the immunologic response to surgery has not been elucidated completely. The authors investigated the effects of minor surgery on immune response by analyzing neutrophil and monocyte phagocytosis and oxidative burst activity. METHODS: Sixteen children undergoing elective minor surgery were enrolled. Blood samples were collected before the operation (at time of induction of anesthesia), at the end of operation, and 72 hours after surgery. Neutrophil and monocyte phagocytosis and oxidative burst activity were studied using a flow cytometric method. RESULTS: Phagocytosis and oxidative burst increased significantly at the end of the operation, both in neutrophils (7.4% and 14.3%, respectively) and monocytes (11.6% and 27%, respectively). The increase was only significant for monocytes (17.5%) 72 hours after surgery. White cell count did not show any significant changes. There was no significant correlation between phagocytosis, oxidative burst activity, and white cell count or neutrophil and monocyte count. CONCLUSIONS: This study shows that minor surgery in children induces immune activation by increasing neutrophil and monocyte phagocytosis and oxidative burst activity. Further studies are required to understand the molecular basis of these findings.

Asking the gatekeepers: a national survey of judges on judging expert evidence in a post-Daubert world.

Drawing on the responses provided by a survey of state court judges (N = 400), empirical evidence is presented with respect to judges' opinions about the Daubert criteria, their utility as decision-making guidelines, the level to which judges understand their scientific meaning, and how they might apply them when evaluating the admissibility of expert evidence. Proportionate stratified random sampling was used to obtain a representative sample of state court judges. Part I of the survey was a structured telephone interview (response rate of 71%) and in Part II, respondents had an option of completing the survey by telephone or receiving a questionnaire in the mail (response rate of 81%). Survey results demonstrate that judges overwhelmingly support the "gatekeeping" role as defined by Daubert, irrespective of the admissibility standard followed in their state. However, many of the judges surveyed lacked the scientific literacy seemingly necessitated by Daubert. Judges had the most difficulty operationalizing falsifiability and error rate, with only 5% of the respondents demonstrating a clear understanding of falsifiability and only 4% demonstrating a clear understanding of error rate. Although there was little consensus about the relative importance of the guidelines, judges attributed more weight to general acceptance as an admissibility criterion. Although most judges agreed that a distinction could be made between "scientific" and "technical or otherwise specialized" knowledge, the ability to apply the Daubert guidelines appeared to have little bearing on whether specific types of expert evidence were designated as "science" or "nonscience." Moreover, judges' "bench philosophy of science" seemed to reflect the rhetoric, rather than the substance, of Daubert. Implications of these results for the evolving relationship between science and law and the ongoing debates about Frye, Daubert, Joiner, and Kumho are discussed.

Factors affecting immunization status of college students in New York City (NYC).

OBJECTIVES: Although immunization is an important public health issue, there have been few studies conducted and minimal information gathered concerning the immunization status of NYC college students. In response to this void, the NYC Department of Health's Bureau of Immunization designed and implemented a study to determine the accessibility of immunization information and service. METHODS: Three hundred and twenty-one college students were asked to complete an immunization survey designed by the researchers in the immunization clinics of New York City. RESULTS: Significant correlations were found between the accessibility of immunization information and immigration status, years of residence in the U.S.A., primary language, as well as school credit completed. Results also indicated that the accessibility of immunization service correlates significantly with ethnicity, immigration status, primary language, years of residence in the U.S.A., accessibility of immunization information, insurance status, employment status, and personal and family income. CONCLUSIONS: The information gathered from this study is useful for public health administration, policy analysis, and program planning.

Serum IgD levels in children with atopic asthma. A longitudinal study.

BACKGROUND: Serum IgD and IgE levels were measured in children with atopic asthma and in control Group in order to determine their relationship with clinical status. METHODS: Samples of venous blood (of 5 cc) were drawn from 25 asthmatic children (Group A) and 25 healthy children (Group B) at the moment of first diagnosis (T0), after 6 months (T180) and after 18 months (T540). To measure IgD, an ELISA assay based on the sandwich principle was used. RESULTS: At T0, IgD were significantly higher in Group A (182.7 5+/-88.18 IU/ml) in comparison with Group B (69.58+/-4.93 IU/ml, p<0.0001); IgD levels decreased in Group A at T540. CONCLUSIONS: In conclusion, a significant increase of IgD levels observed in children at first signs of asthma and the following normalization of these same levels after 18 months, may represent a non specific response or an attempt of the organism to block asthma, favouring therefore immunologic tolerance.

Intracellular mechanisms mediating the neuronal death and astrogliosis induced by the prion protein fragment 106-126.

Prion encephalopathies include fatal diseases of the central nervous system of men and animals characterized by nerve cell loss, glial proliferation and deposition of amyloid fibrils into the brain. During these diseases a cellular glycoprotein (the prion protein, PrP(C)) is converted, through a not yet completely clear mechanism, in an altered isoform (the prion scrapie, PrP(Sc)) that accumulates within the brain tissue by virtue of its resistance to the intracellular catabolism. PrP(Sc) is believed to be responsible for the neuronal loss that is observed in the prion disease. The PrP 106-126, a synthetic peptide that has been obtained from the amyloidogenic portion of the prion protein, represents a suitable model for studying the pathogenic role of the PrP(Sc), retaining, in vitro, some characteristics of the entire protein, such as the capability to aggregate in fibrils, and the neurotoxicity. In this work we present the results we have recently obtained regarding the action of the PrP 106-126 in different cellular models. We report that the PrP 106-126 induces proliferation of cortical astrocytes, as well as degeneration of primary cultures of cortical neurons or of neuroectodermal stable cell lines (GH(3) cells). In particular, these two opposite effects are mediated by the same attitude of the peptide to interact with the L-type calcium channels: in the astrocytes, the activity of these channels seems to be activated by PrP 106-126, while, in the cortical neurons and in the GH(3) cells, the same treatment causes a blockade of these channels causing a toxic effect.

Prolonged treatment with alpha-glycerylphosphorylethanolamine facilitates the acquisition of an active avoidance behavior and selectively increases neuronal signal transduction in rats.

The effects of alpha-glycerylphosphorylethanolamine on both behavioral and neurochemical parameters were studied in adult rats. Daily administration of the drug caused a significant improvement in the behavioral performance of rats in the active avoidance conditioning test. This effect was observed after about ten days of treatment, and lasted until the end of the experiment (fifteen days). The improvement in this memory-related behavioral test correlated with a facilitation of both muscarinic and beta-adrenergic stimulation of brain adenylyl cyclase activity. Conversely, no changes were observed in basal or forskolin-induced stimulation of cAMP production, suggesting that the alpha-glycerylphosphorylethanolamine effects were not directed on the enzyme itself, but might favor the coupling between receptors, G proteins and effectors. Similar results were observed on the muscarinic stimulation of inositol phosphate accumulation although, in this case, a potentiation of the basal activity also occurred. In conclusion, our data indicate that daily treatment with alpha-glycerylphosphorylethanolamine improves the learning and memory processes in the rat, evaluated using the active avoidance conditioning test. Furthermore, the subchronic administration of this compound is able to enhance receptor-mediated neuronal signal transduction, namely cAMP and inositol phosphate production. These neurochemical modifications may represent, at least in part, the molecular mechanisms of action of the drug.

Comparisons of immunisation accessibility between non-US born and US-born children in New York City.

BACKGROUND: New York City (NYC) has the second highest immigrant population in the USA; with many migrants from countries outside the USA being inadequately immunised. To assess the immunisation status of this population, the NYC Department of Health's Family Health Services Division designed and implemented a study in order to examine how accessible the immunisation service was to immigrant families who wished to have their children immunised. METHODOLOGY: A one time cross-sectional design was used in this study and data was collected, by means of a survey, in the NYC Department of Health's Immunisation Clinics. These were the major walk-in and free of charge immunisation facilities for children in NYC. Descriptive and Chi square statistics were used to analyse the data from this survey. RESULTS: Of 270 children surveyed, 211 (78%) were born outside the US; of these, 116 (55%) reported that apart from the NYC Department of Health's Immunisation Clinics they had no other access to immunisation services. There was a significant difference in the accessibility to immunisation services between non-US-born and US-born children. The factors that influenced the accessibility by non-US-born children compared with US-born children included health insurance, family income, primary language and years of residence in the US. How they came to hear about immunisation services and general knowledge regarding immunisation were not significantly different between these two groups and these were not significant factors which affected accessibility to immunisation services among children. CONCLUSION: Major steps have been taken in NYC to help increase access to immunisation services by using free public health immunisation programs. Such programs should be expanded to target non-US-born children and their parents. Such educational and outreach programs need to be culturally sensitive.

Molecular mapping of wheat. Homoeologous group 2.

A molecular-marker map of bread wheat having many markers in common with other grasses in the Gramineae family is a prerequisite for molecular level genetic studies and breeding in this crop species. We have constructed restriction fragment length polymorphism maps of the A-, B-, and D-genome chromosomes of homoeologous group 2 of hexaploid wheat (Triticum aestivum L. em. Thell) using 114 F7 lines from a synthetic x bread wheat cross and clones from 11 libraries. Chromosomes 2A, 2B, and 2D comprise 57, 60, and 56 markers and each spans about 200 cM. Comparisons between chromosomes are facilitated by 26 sets of homoeoloci. Genes mapped include a heterologous abscisic acid responsive locus cloned as pBS128, the epidermal waxiness inhibitor W21, and two presumed leaf rust and stem rust resistance genes. Anomalies suggesting ancestral rearrangements in chromosome 2B are pointed out and features of wheat group 2 chromosomes that are common to barley (Hordeum vulgare L.), rice (Oryza spp.), and T. tauschii are discussed.

[Preoperative diagnosis of thyroid nodule. Ultrasonography-guided fine-needle aspiration]. / In tema di diagnosi di natura preoperatoria del nodulo tiroideo. L'FNA ecoguidata.

Nodular thyroid pathology weighs heavily for 4-5% of the general population, being the most common among the endocrinopathies. Because of this these diagnostic methods have gained more and more importance as FNA able to select already in the pre-operation phase the malignant nodules from the benign ones and to decide on the most suitable surgical intervention. In the period of time between January 1989 and June 1992 in the Institute of the III Surgical Clinic in Rome there have been performed 403 FNA. From the whole total we have extrapolated 221 patients. In this study we stressed how the FNA method has a sensibility of 80%, a specificity of 93% and afterwards an accurate diagnosis of 92%. Besides we have verified that sonography and radionuclide scanning have a truth worthiness respectively of 64% and 52%. The FNA in our opinion allows us to have in the pre-operation phase a much more accurate diagnosis as regards that obtained with only sonography and radionuclide scanning therefore the latter methods must be considered complementary to FNA.