Clinical-haematological changes and predictors of severity in acute food protein-induced enterocolitis syndrome reactions at oral food challenge: a multicentre observational study.

BACKGROUND: Oral food challenge (OFC) is the gold standard for diagnosis of acute Food Protein-Induced Enterocolitis Syndrome (FPIES). No diagnostic/prognostic biomarkers are available, and OFC assessment criteria are not validated. OBJECTIVE: To assess clinical-haematological changes and predictors of severity of FPIES reactions at OFC. METHODS: Observational multicentre prospective study. Children aged 0-18 years diagnosed with acute FPIES were recruited at follow-up OFC in 12 tertiary centres in Spain and Italy. OFC Outcomes (as positive/negative/inconclusive and mild/moderate/severe) were assessed based on published '2017 FPIES Consensus' criteria. Clinical characteristics were recorded, and full blood count was done at baseline, reaction onset and 4 hours later. Regression analysis was performed to assess predictors of severe reactions at OFC. RESULTS: 81 children had positive OFC (mild in 11% (9/81), moderate in 61% (49/81), severe in 28% (23/81)). Increase in neutrophils and reduction in eosinophils, basophils and lymphocytes was observed (P-value<0.05). OFC was inconclusive in 19 cases despite objective signs or neutrophilia. Regression analysis showed a 2-day OFC protocol where only 25% of an age-appropriate portion is given on day 1 (not gender, age, culprit food, cumulative dose and previous reaction severity) was associated with reduced odds of severe reaction compared to giving multiple doses in a single day. CONCLUSION: Distinct haematological changes may help support FPIES diagnosis. Current OFC assessment criteria may not capture the broad spectrum of acute FPIES presentations. This 2-day protocol may associate a reduced risk of severe reactions. Future work should aim to develop safer OFC and non-OFC diagnostics for FPIES.

Dopamine denervation in the functional territories of the striatum: a new MR and atlas-based 123I-FP-CIT SPECT quantification method.

Current quantification methods of 123I-FP-CIT SPECT rely on anatomical parcellation of the striatum. We propose here to implement a new method based on MRI segmentation and functional atlas of the basal ganglia (MR-ATLAS) that could provide a reliable quantification within the sensorimotor, associative, and limbic territories of the striatum. Patients with Parkinson's disease (PD), idiopathic rapid eye movement sleep behavioral disorder (iRBD), and healthy controls underwent 123I-FP-CIT SPECT, MRI, motor, and cognitive assessments. SPECT data were corrected for partial volume effects and registered to a functional atlas of the striatum to allow quantification in every functional region of the striatum (nucleus accumbens, limbic, associative, and sensorimotor parts of the striatum). The MR-ATLAS quantification method is proved to be reliable in every territory of the striatum. In addition, good correlations were found between cognitive dysexecutive tests and the binding within the functional (limbic) territories of the striatum using the MR-ATLAS method, slightly better than correlations found using the anatomical quantification method. This new MR-ATLAS method provides a robust and useful tool for studying the dopaminergic system in PD, particularly with respect to cognitive functions. It may also be relevant to further unravel the relationship between dopaminergic denervation and cognitive or behavioral symptoms.

Parkinson's disease polygenic risk score is not associated with impulse control disorders: A longitudinal study.

OBJECTIVE: To examine the relationship between a Parkinson's disease (PD) polygenic risk score (PRS) and impulse control disorders (ICDs) in PD. BACKGROUND: Genome wide association studies (GWAS) have brought forth a PRS associated with increased risk of PD and younger disease onset. ICDs are frequent adverse effects of dopaminergic drugs and are also more frequent in patients with younger disease onset. It is unknown whether ICDs and PD share genetic susceptibility. METHODS: We used data from a multicenter longitudinal cohort of PD patients with annual visits up to 6 years (DIG-PD). At each visit ICDs, defined as compulsive gambling, buying, eating, or sexual behavior were evaluated by movement disorders specialists. We genotyped DNAs using the Megachip assay (Illumina) and calculated a weighted PRS based on 90 SNPs associated with PD. We estimated the association between PRS and prevalence of ICDs at each visit using Poisson generalized estimating equations, adjusted for dopaminergic treatment and other known risk factors for ICDs. RESULTS: Of 403 patients, 185 developed ICDs. Patients with younger age at onset had a higher prevalence of ICDs (p < 0.001) as well as higher PRS values (p = 0.06). At baseline, there was no association between the PRS and ICDs (overall, p = 0.84). The prevalence of ICDs increased over time similarly across the quartiles of the PRS (overall, p = 0.88; DA users, p = 0.99). CONCLUSION: Despite younger disease onset being associated with both higher PRS and ICD prevalence, our findings are not in favor of common susceptibility genes for PD and ICDs.

Increase of natural killer cells in children with liver transplantation-acquired food allergy

BACKGROUND: Transplantation-acquired food allergies (TAFA) are frequently reported and considered to be caused by immunosuppressive therapy. The aim of this study was to investigate the allergic and immunologic responses in children who had liver or kidney transplantations. METHODS: Twelve children receiving liver transplantations and 10 children receiving kidney transplantations were investigated. All children underwent the allergy work-up and in most of them, lymphocyte screening and serum cytokine measurements were also performed. RESULTS: TAFA were found in 7/12 (58%) children with liver transplantations and in none of the 10 children with kidney transplantations. The mean age at transplantation was significantly lower in children who underwent liver transplantations (p < 0.001). The immunosuppressive therapy administered to children with liver transplantation was tacrolimus in 11 patients and cyclosporine in one patient, while all 10 children with kidney transplantation received tacrolimus plus mycophenolate. The most common antigenic food was egg. The natural killer (NK) cell numbers were significantly higher in liver-transplant children than in kidney-transplant children. No significant differences were found in the serum cytokine levels. CONCLUSIONS: This study confirms that liver-transplant children treated with tacrolimus alone have a higher risk of developing TAFA than kidney-transplant children treated with tacrolimus plus mycophenolate. NK cells might be involved in this difference

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Increase of natural killer cells in children with liver transplantation-acquired food allergy.

BACKGROUND: Transplantation-acquired food allergies (TAFA) are frequently reported and considered to be caused by immunosuppressive therapy. The aim of this study was to investigate the allergic and immunologic responses in children who had liver or kidney transplantations. METHODS: Twelve children receiving liver transplantations and 10 children receiving kidney transplantations were investigated. All children underwent the allergy work-up and in most of them, lymphocyte screening and serum cytokine measurements were also performed. RESULTS: TAFA were found in 7/12 (58%) children with liver transplantations and in none of the 10 children with kidney transplantations. The mean age at transplantation was significantly lower in children who underwent liver transplantations (p<0.001). The immunosuppressive therapy administered to children with liver transplantation was tacrolimus in 11 patients and cyclosporine in one patient, while all 10 children with kidney transplantation received tacrolimus plus mycophenolate. The most common antigenic food was egg. The natural killer (NK) cell numbers were significantly higher in liver-transplant children than in kidney-transplant children. No significant differences were found in the serum cytokine levels. CONCLUSIONS: This study confirms that liver-transplant children treated with tacrolimus alone have a higher risk of developing TAFA than kidney-transplant children treated with tacrolimus plus mycophenolate. NK cells might be involved in this difference.

Functional results and patient satisfaction after long fusion for spinal deformity in Parkinson's disease.

BACKGROUND: Patients with Parkinson's disease often present abnormal posture or severe sagittal malalignment, causing significant disability. Surgical fusion is these cases shows high rates of complications, but may nevertheless provide functional benefit; however, this remains to be assessed. HYPOTHESIS: Long fusion for patients with Parkinson's disease and postural disorder could alleviate disability despite the high risk of complications. METHODS: We retrospectively reviewed 18 Parkinson patients treated by long fusion for spinal deformity. Functional results on the Oswestry Disability Index (ODI) and patient satisfaction were assessed at a minimum 2 years' follow-up. Predictive factors for good outcome were analyzed. RESULTS: Median follow-up was 44.4 months (IQR, 36-62.4 months). ODI showed significant improvement, from 64 (IQR, 59-77) preoperatively to 49 (IQR, 40-57) at last follow-up (p=0.0014). Fifteen patients (83%) were very satisfied (n=5) or satisfied (n=10) with the procedure. On multivariate analysis, only age was significantly associated with improvement in ODI at last follow-up (estimate: -9.8; p=0.5). DISCUSSION: Although long spinal fusion involves a high risk of complications in Parkinson's patients, the improvement in autonomy and patient satisfaction should be borne in mind before rejecting surgery, especially with motivated patients. LEVEL OF EVIDENCE: IV.

Assessment of neuroactive steroids in cerebrospinal fluid comparing acute relapse and stable disease in relapsing-remitting multiple sclerosis.

Previous studies have reported an involvement of neuroactive steroids as neuroprotective and anti-inflammatory agents in neurological disorders such as multiple sclerosis (MS); an analysis of their profile during a specific clinical phase of MS is largely unknown. The pregnenolone (PREG), dehydroepiandrosterone (DHEA), and allopregnanolone (ALLO) profile was evaluated in cerebrospinal fluid (CSF) in relapsing-remitting multiple sclerosis (RR-MS) patients as well as those in patients affected by non-inflammatory neurological (control group I) and without neurological disorders (control group II). An increase of PREG and DHEA values was shown in CSF of male and female RR-MS patients compared to those observed in both control groups. The ALLO values were significantly lower in female RR-MS patients than those found in male RR-MS patients and in female without neurological disorder. During the clinical relapse, we observed female RR-MS patients showing significantly increased PREG values compared to female RR-MS patients in stable phase, while their ALLO values showed a significant decrease compared to male RR-MS patients of the same group. Male RR-MS patients with gadolinium-enhanced lesions showed PREG and DHEA values higher than those found in female RR-MS patients with gadolinium-enhanced lesions. Similary, male RR-MS patients with gadolinium-enhanced lesions showed PREG and DHEA values higher than male without gadolinium-enhanced lesions. Female RR-MS patients with gadolinium-enhanced lesions showed DHEA values higher than those found in female RR-MS patients with gadolinium-enhanced lesions. Male and female RR-MS patients with gadolinium-enhanced lesions showed ALLO values higher than those found in respective gender groups without gadolinium-enhanced lesions. ALLO values were lower in male than in female RR-MS patients without gadolinium-enhanced lesions. Considering the pharmacological properties of neuroactive steroids and the observation that neurological disorders influence their concentrations, these endogenous compounds may have an important role as prognostic factors of the disease and used as markers of MS activity such as relapses.

Altered natural killer cells subsets distribution in children with hepatitis C following vertical transmission.

BACKGROUND: Natural killer (NK) cells number, phenotypes and function have been evaluated in many studies in adults with hepatitis C as compared with healthy controls or dynamically during interferon-based and interferon-free treatments. Overall, in adults with chronic infection number of circulating NK cells has been reported to be lower when compared to spontaneous resolvers and healthy subjects. Different studies yielded inconsistent findings due to patient and virus heterogeneity. AIM: To evaluate NK cells in children according to the different outcomes of the infection. METHODS: In this cross-sectional study, we examined numbers and phenotypes of circulating NK cells from a homogenous cohort of Italian children with vertically acquired hepatitis C. RESULTS: We compared 31 children who developed chronic infection with nine who presented spontaneous clearance and 13 controls. CD56(+) CD3(-) NK cell numbers were consistently lower in the persistently infected group (P = 0.03 and 0.04). This decrease was due to depletions of CD56(dim) NK cells (P = 0.03 chronic infection vs. spontaneous clearance), while CD56(bright) NK cells were expanded (P = 0.03). No significant difference was found in the frequencies of CD56(+) CD16(+) and CD56(dim) CD16(-) cells. Perforin expression was higher in children with chronic infection (P = 0.03 vs. spontaneous clearance). CONCLUSIONS: Altered NK cells number and phenotypes could impact the outcome of HCV infection in children following vertical transmission. This study suggests for the first time that NK cells cytolytic function, featured by CD56(dim) cells, contributes to the elimination of HCV in children presenting spontaneous clearance.

Morbidity of teeth in the line of the fracture.

AIM: Management of a jaw fracture in which a tooth lies in the fracture line is controversial. In the past, teeth were generally removed because they were thought to be the cause of infections or locus minoris resistentiae. To provide a better indication in the management of teeth in the fracture line and the relate complications we retrospectively reviewed the files particularly in relation to treatment options. METHODS: From 1999 to 2009 a total of 478 patients with jaw fracture were observed at the Maxillofacial Department of the University "Federico II" of Naples. RESULTS: In 63 cases the fracture involved the angle of the mandible and in 48 a tooth lay in the fracture line. Of the 48 teeth in the fracture line, 14 were removed for various causes. Surgical treatment consisted of fracture reduction and fixation by titanium plates and screws. CONCLUSION: We suggest few guiding lines in the management of teeth in fracture lines that need to be extracted only in particular conditions. Finally there are few cases of later dental complications that can affect the teeth in the fracture line after the osseous healing process of the fractures. These cases require a follow-up of three-six months, and the treatment is the same commonly described in Literature for dental trauma.

Leiomyosarcoma of the submandibular gland. Report of a case and review of the literature.

This report describes the first case of primary leiomyosarcoma of the submandibular salivary glands and emphasizes the role of immunohistochemical study for a correct diagnosis of this tumour. In line with results of international literature, we associated surgery with radiotherapy and 2 years postoperatively there was no sign of recurrence.

Intraosseous mucoepidermoid carcinoma. Report of a long-term evolution case.

Primary central mucoepidermoid carcinoma (CMC) of the jaws accounts only for 2-3% of all mucoepidermoid carcinomas reported. Bhaskar in 1963 first analysed the criteria for his central origin, histology and pathogenesis. The authors report a long-term evolution case of CMC of the mandible with peculiar clinical features observed at the Department of Maxillo-Facial Surgery of the School of Medicine and Surgery of the "Federico II" University of Naples (Naples, Italy) examining histopathologic and clinical features and problems related to the treatment.

Surgical management of the neck in squamous cell carcinoma of the tongue.

If the nodes are involved, survival of patients with squamous cell carcinoma of the tongue is considerably reduced. Surgery remains the treatment of choice and, to define its role, we have reviewed 82 consecutive cases. Sixty-two cases (76%) were T1-2, and 46 patients (56%) had involved nodes. The cervical region II was the most often involved (n=26). Occult nodal metastases were present in 12 cases. The extent of nodal spread and prognosis varies according to whether the body or the base of the tongue is involved. Lesions of the base with involved node should be treated by a selective posterolateral neck dissection, whilst in the case of a lesion of the body of the tongue, the dissection should be selective anterolateral. In lesions of the base, when there are no nodes involved, a prophylactic selective posterolateral neck dissection is recommended, whilst in the case of the lesions of the body, selective supraomohyoid neck dissection in T2-4 lesions is recommended.

[Pathological fracture of the mandible: a report of a clinical case treated with clodronate]. / Frattura patologica della mandibola: descrizione di un caso clinico trattato con clodronato.

Oral squamous cell carcinoma often invades the mandible. However, the incidence of pathological fractures of the maxillofacial bones is low and their treatment is rarely satisfactory. A patient, too weak to undergo surgery, affected by squamous cell carcinoma of the mandibular region with bone involvement and pathological fracture of the mandible, underwent chemotherapy with carboplatin associated with diphosphonate.

Neuroendocrine (Merkel cell) carcinoma of the oral mucosa: report of a case with immunohistochemical study and review of the literature.

Merkel cell carcinoma (Mcc) is an uncommon and aggressive tumour with neuroendocrine features that occur predominantly in the head and neck region. The rarity of this tumour, especially when it arises in the oral mucosa, makes both early identification and standardisation of treatment difficult, particularly as regards complementary treatment. The availability of monoclonal antibodies with restricted specificity for some antigens thought to be related to neuroendocrine carcinomas, such as Merkel cell carcinoma, and ultrastructural studies offer some new leads to investigation. This has allowed, a greater number of these tumours to be discovered, thereby increasing the chances of effective management. A case of Mcc of the floor of the mouth is reported, together with the results of cytokeratin, neuron specific enolase and chromogranin immunohistochemistry.

Perineural invasion of the lower alveolar nerve by oral cancer: a follow-up study of 12 cases.

Twelve previously untreated cases of oral cancer with perineural infiltration were studied retrospectively. Age, sex, site, clinical stage and outcome were evaluated. Management of the neoplasm in each case involved surgical removal and six patients required adjuvant radiotherapy. The most frequent site was the lip. At the time of diagnosis, five patients had sensory complaints and palpable lymphadenopathy was observed in three patients. The 5-year crude survival rate was 16.7%. In the cases with postoperative assessment of perineural infiltration, a median survival time of 30.8 months was observed; while in the case of preoperative assessment of nerve infiltration, extensive surgery was performed with a consequent median survival time of 44.5 months. The perineural infiltration of the lower alveolar nerve is more common (6.3%) than is generally thought. This frequency is due to the relationship with the lower lip and the mandibular region. In carcinoma of the lip, spread is generally limited to 10-15 mm along the lower alveolar nerve. In carcinoma of the mandibular region, spread is entirely dependent on the location of the tumour; the absence of clinical fixation to the bone and the small size of the carcinoma does not preclude the possibility of bone involvement. Neurological symptoms should be evaluated carefully, and a radiographic investigation of the nerve canal is mandatory.

Surgical management of the neck in squamous cell carcinoma of the floor of the mouth.

Nodal involvement in squamous cell carcinoma considerably lowers survival rate. Despite its importance, neck management has still not been adequately explored. The Authors have retrospectively reviewed the records of 112 cases. Unilateral N+ were treated with a homolateral therapeutic and a controlateral prophylactic neck dissection; bilateral N+ were treated with a bilateral therapeutic neck dissection. On first observation the majority of cases (66.1%) were T1-2, N+ patients accounted for 45.5%. Among N- patients, 21.3% of occult nodal metastases were observed. The 5-year survival rate was 52.7%. With N+ lesions, a radical neck dissection should be performed; the dissection should be performed bilaterally. With N- lesions a prophylactic modified radical neck dissection is recommended in T2-4 lesions.