Propranolol and infantile hemangiomas: Outcome from a tricentric study.

INTRODUCTION: The introduction of propranolol as systemic therapy for infantile hemangiomas (IH) has changed the natural history of these tumors. Cases presenting with ulceration, functional limitation, and visceral or life-threatening localization are excellent indications to medical therapy. The aim was to report the medium term follow-up after the introduction of propranolol in 3 referral centers with particular attention to outcome and surgical treatments. MATERIALS AND METHODS: In the period 2011-2018 348 patients underwent systemic therapy with propanolol at a mean age of 3.5 months (range 1-10). The indications to begin the treatment were: visceral localization (10,6%), ulceration (20%), risk of severe esthetic impairment (27%), and peri­orifices localization (42.4%). RESULTS: The minimum follow-up was 1 year. Propranolol was administered for a mean of 11 months (range: 6-19) and in 22 cases it was resumed for extra 4.5 months due to rebound. Overall, 97.4% responded to therapy without severe side effects. Seven patients underwent surgery and 9 laser therapy . Twenty-four cases are scheduled for surgery for evident inesthetisms and 4 will undergo laser therapy to complete the treatment. CONCLUSIONS: The use of propranolol has considerably decreased the surgical indications for IH, improved the esthetic and functional outcome, and simplified the definitive surgical treatment. The response rate to therapy is very high and in case of failure both laser and surgery are still very effective. The correct diagnosis, early starting, and adequate duration of therapy are key factors for the success of treatment.

Syndecan-2 selectively regulates VEGF-induced vascular permeability.

Vascular endothelial growth factor (VEGF)- driven increase in vascular permeability is a key feature of many disease states associated with inflammation and ischemic injury, contributing significantly to morbidity and mortality in these settings. Despite its importance, no specific regulators that preferentially control VEGF-dependent increase in permeability versus its other biological activities, have been identified. Here we report that a proteoglycan Syndecan-2 (Sdc2) regulates the interaction between a transmembrane phosphatase DEP1 and VEGFR2 by controlling cell surface levels of DEP1. In the absence of Sdc2 or the presence of an antibody that blocks Sdc2-DEP1 interaction, increased plasma membrane DEP1 levels promote selective dephosphorylation of the VEGFR2 Y951 site that is involved in permeability control. Either an endothelial-specific Sdc2 deletion or a treatment with an anti-Sdc2 antibody result in a highly significant reduction in stroke size due to a decrease in intracerebral edema.

In transit metastases in children, adolescents and young adults with localized rhabdomyosarcoma of the distal extremities: Analysis of the EpSSG RMS 2005 study.

In-transit metastases (ITM) are defined as metastatic lymph nodes or deposits occurring between the primary tumor and proximal draining lymph node basin. In extremity rhabdomyosarcoma (RMS), they have rarely been reported. This study evaluates the frequency, staging and survival of patients with ITM in distal extremity RMS. METHODS: Patients with extremity RMS distal to the elbow or knee, enrolled in the EpSSG RMS 2005 trial between 2005 and 2016 were eligible for this study. RESULTS: One hundred and nine distal extremity RMS patients, with a median age of 6.2 years (range 0-21 years) were included. Thirty seven of 109 (34%) had lymph node metastases at diagnosis, 19 of them (51%) had ITM, especially in lower extremity RMS. 18F-FDG-PET/CT detected involved lymph nodes in 47% of patients. In patients not undergoing 18F-FDG-PET/CT lymph node involvement was detected in 22%. The 5-yr EFS of patients with ITM vs proximal lymph nodes vs combined proximal and ITM was 88.9% vs 21.4% vs 20%, respectively (p = 0.01) and 5-yr OS was 100% vs 25.2% vs 15%, respectively (p = 0.003). CONCLUSION: Our study showed that in-transit metastases constituted more than 50% of all lymph node metastases in distal extremity RMS. 18F-FDG-PET/CT improved nodal staging by detecting more regional and in-transit metastases. Popliteal and epitrochlear nodes should be considered as true (distal) regional nodes, instead of in-transit metastases. Biopsy of these nodes is recommended especially in distal extremity RMS of the lower limb. Patients with proximal (axillary or inguinal) lymph node involvement have a worse prognosis.

Second primary neoplasms in patients with lung and gastroenteropancreatic neuroendocrine neoplasms: Data from a retrospective multi-centric study.

BACKGROUND: Patients with sporadic neuroendocrine neoplasms may exhibit a higher risk of a second primary tumor than the general population. AIM: This study aimed to analyze the occurrence of second primary malignancies. METHODS: A retrospective cohort of 2757 patients with sporadic lung and gastro-entero-pancreatic neuroendocrine neoplasms, managed at eight Italian tertiary referral Centers, was included. RESULTS: Between 2000 and 2019, a second primary malignancy was observed in 271 (9.8%) neuroendocrine neoplasms patients with 32 developing a third tumor. There were 135 (49.8%) females and the median age was 64 years. The most frequent locations of the second tumors were breast (18.8%), prostate (12.5%), colon (9.6%), blood tumors (8.5%), and lung (7.7%). The second primary tumor was synchronous in 19.2% of cases, metachronous in 43.2%, and previous in 37.6%. As concerned the neuroendocrine neoplasms, the 5- and 10-year survival rates were 87.8% and 74.4%, respectively. PFS for patients with a second primary malignancy was shorter than for patients without a second primary malignancy. Death was mainly related to neuroendocrine neoplasms. CONCLUSION: In NEN patients the prevalence of second primary malignancies was not negligible, suggesting a possible neoplastic susceptibility. Overall survival was not affected by the occurrence of a second primary malignancy.

Transfer of innovation on allergic rhinitis and asthma multimorbidity in the elderly (MACVIA-ARIA) - EIP on AHA Twinning Reference Site (GARD research demonstration project).

The overarching goals of the European Innovation Partnership on Active and Healthy Ageing (EIP on AHA) are to enable European citizens to lead healthy, active and independent lives whilst ageing. The EIP on AHA includes 74 Reference Sites. The aim of this study was to transfer innovation from an app developed by the MACVIA-France EIP on AHA reference site (Allergy Diary) to other reference sites. The phenotypic characteristics of rhinitis and asthma multimorbidity in adults and the elderly will be compared using validated information and communication technology (ICT) tools (i.e. the Allergy Diary and CARAT: Control of Allergic Rhinitis and Asthma Test) in 22 Reference Sites or regions across Europe. This will improve the understanding, assessment of burden, diagnosis and management of rhinitis in the elderly by comparison with an adult population. Specific objectives will be: (i) to assess the percentage of adults and elderly who are able to use the Allergy Diary, (ii) to study the phenotypic characteristics and treatment over a 1-year period of rhinitis and asthma multimorbidity at baseline (cross-sectional study) and (iii) to follow-up using visual analogue scale (VAS). This part of the study may provide some insight into the differences between the elderly and adults in terms of response to treatment and practice. Finally (iv) work productivity will be examined in adults.

Fine-needle aspiration cytology in children with superficial lymphadenopathy.

INTRODUCTION: In pediatric population Fine-Needle Aspiration Citology (FNAC) is slowly gaining acceptance in clinical management of Superficial Lymphadenopathy (SL). Our experience adds some data about the usefulness of this technique in diagnosing the cause of a SL and therefore guiding further treatment. PATIENTS AND METHODS: 238 FNAC were performed in 217 patients with SL, observed at our Institution from 2002 to 2006. The neck was the most frequent localization. The results were available within few hours. In cases of granulomatous findings, the samples were processed for microbiological and PCR test, in order to identify Mycobacteria. RESULTS: 174 were reactive lesions, 38 granulomatous lymphadenopathies, 24 malignant lesions, 2 specimens inadequate for diagnosis. Among the 174 reactive SL, 22 required an incisional biopsy after 1 month follow-up. Among the granulomatous lymphadenopathies, 13 children with Cat-Scratch Disease recovered, 25 with Mycobacteria infection underwent surgical excision. For 24 malignant lesions, the diagnosis was confirmed by further biopsy. Two false negative and no false positive were detected (sensitivity 92%, specificity 100%). No complications were encountered. CONCLUSIONS: In our experience FNAC, performed by experienced cytopathologist, has revealed to be a fast, safe, non invasive and inexpensive method to achieve diagnosis in persistent SL. The use of FNAC gave us the possibility to select patients for further investigation and/or surgical treatment. Incisional biopsy remains necessary to confirm the diagnosis in case of malignancy or doubtful lesions.

Seroconversion of HBsAg in HBeAg positive and HBeAg negative patients with chronic HBV treated with entecavir: a case series.

We report a case series of three HBeAg positive and five HBeAg negative patients (7 males, mean age 50.6 +/- 14.6 years) with chronic HBV infection experiencing seroconversion after treatment with entecavir (0.5 mg/day or 1 mg/day), initiated in 2007. Overall, the mean time to HBsAg clearance was 9.4 +/- 4.5 months. Seroconversion occurred in all patients, after a mean time of 8.0 +/- 3.7 months. In HBeAg negative patients, mean time to HBsAg clearance and to seroconversion were 9.2 +/- 5.9 and 6.8 +/- 4.0 months, respectively. In HBeAg positive patients, mean time to HBsAg clearance and to seroconversion were 9.7 +/- 0.6 months and 10.0 +/- 2.6 months, respectively. In this case series, seroconversion was maintained and was observed both in HBeAg positive patients and in HBeAg negative patients. Therefore, it may be preliminarily suggested that treatment with entecavir could be associated to HBsAg seroconversion in a short period of time, in both HBeAg positive and HBeAg negative HBV patients.

Role of atypical bacteria in children undergoing tonsillectomy because of severely recurrent acute tonsillopharyngitis.

The aim of this study was to verify the frequency of atypical bacterial infections in children undergoing tonsillectomy because of severely recurrent acute tonsillopharyngitis (AT) and the possible benefit of surgery in cases in which Mycoplasma pneumoniae and Chlamydophila pneumoniae seem to play a role in causing the recurrences. A total of 118 patients (76 males; mean age +/- standard deviation, 6.67 +/- 3.31 years) were enrolled: 59 underwent tonsillectomy because of severely recurrent AT and 59 underwent adenotonsillectomy because of obstructive sleep apnea syndrome (OSAS). The results show, for the first time, that the great majority of children with a history of severely recurrent AT (and, therefore, considered to be eligible for elective tonsillectomy) are infected by atypical bacteria, mainly M. pneumoniae, and that tonsillectomy seems to be effective in reducing the recurrence of both AT and acute respiratory disease during 12 months follow-up postsurgery.

Hematometra in uterus didelphis: a pitfall in teenagers with lower abdominal mass. Report of two cases.

Müllerian malformations represent a wide spectrum of anomaly of the female reproductive tract, usually detected at birth or at puberty. The presence of uterus didelphys and obstructed hemivagina, associated with ipsilateral renal agenesis, is known as Herlyn-Werner-Wunderlich syndrome, a rare congenital anomaly. Two cases of uterus didelphic and hematometra caused by vaginal septum are reported; the girls had a delayed diagnosis due to absence of a specific clinical feature. Clinicians must be aware of the existence of these malformations. An adequate radiological study is mandatory to assess the anatomic conformation, and to optimize the therapeutic procedures.

Endoscopic thoracic sympathectomy by clamping in the treatment of social phobia: the Monza experience.

AIM: Facial blushing represents the peculiar symptom in social phobia and is defined as the ''hallmark of embarrassment''. At present, endoscopic thoracic sympathectomy (ETS) is the technique only able to guarantee long-term results in the treatment of sympathetic disorders. The aim of our study was to evaluate results, complications and the true value of 52 ETSs by two-port approach with the use of endoclips in patients with isolated facial blushing or in association with hyperhidrosis. METHODS: A retrospective review of 52 subjects who underwent standard ETS by clamping between September 2002 and April 2005 was carried out. This surgical practice was performed in 26 cases (50%) for isolated facial blushing only and in other 26 cases (50%) for facial blushing associated to hyperhidrosis with changeable localizations. Under endoscopic guidance we operated an endoscopic sympathetic block (ESB) with interruption of the inter-ganglion trunk at the level of T2-T3 in 51 patients (98.07%). In 1 patient (1.92%) we performed a sympathetic block with the complete exclusion of T3 thoracic ganglion. RESULTS: There were no intra and postoperative deaths. The effect rate was excellent in all patients with early complications in one patient (1.92%) only. CONCLUSIONS: ETS by clamping is safe and effective. Patients referred an high satisfaction in terms of complications and quality of life; specific early and late diseases are negligible.

[Pleural empyema: considerations about experience of the Thoracic Surgery Division in Monza and proposal of therapeutic protocol]. / Gli empiemi pleurici: considerazioni sull'esperienza della Divisione di Chirurgia Toracica di Monza e proposta di un protocollo terapeutico.

AIM: Optima l treatment of thoracic empyema depends on its phase of development. Thin, free-flowing exudative effusions can be adequately managed by thoracenthesis or simple closed tube thoracostomy and antibiotics. At the other end of the spectrum are well-organized chronic empyemas which are best treated with thoracothomy and decortication. In-between this two extremes are fibrino-purulent empyemas for which much controversies exist among pneumologists and thoracic surgeons over the most effective treatment. The authors present their experience about treatment of pleural empyema. METHODS: This retrospective study reviews the Thoracic Surgery Department of Monza's S. Gerardo Hospital experience between 1982-2004 in the treatment of thoracic empyema and tries to formulate our own guidelines for the management of the fibrino-purulent forms. RESULTS: In our experience we used a personal technique of thoracic drainage consisting in continuous wash-out of pleural cavity associated with intrapleural fibrinolytic if is it necessary. Since 1994 we have used the continuous positive alveolar pressure to mend lungs expansion and improve the CONCLUSIONS: Recent reports in the medical and surgical literature have suggested that empyemas can be managed effectively with less aggressive treatment than has been advocated in the past. In spite of the recent development of mini-invasive surgical techniques for the treatment of fibrino-purulent empyemas we believe that first approach should be always conservative. Intrapleural fibrinolytics seem to get improve the efficacy of closed tube thoracostomy.

[Thoracic sympathetic block by clamping in the treatment of primary hyperhidrosis: indications and results in 281 patients]. / Interruzione del simpatico toracico mediante neurocompressione nel trattamento dell'iperidrosi primitiva. Indicazioni e risultati ottenuti in 281 pazienti.

AIM: Hyperhidrosis is a disabling troublesome characterized by excessive perspiration that produces a significant social and professional deficit for patients. It main involves the palmar sides of hands and feet, axillae, face and, with a lesser incidence, trunk and scalp. Thoracoscopic sympathectomy represents the best approach for this disorder. The aim of our prospective study is to assess the outcome, the safety and efficacy after thoracoscopic sympathetic block by clipping in the treatment of primary hyperhidrosis. METHODS: Between september 2002 and april 2005, 281 patients (117 female and 164 male, with a mean age of 28 years) underwent Endoscopic Thoracic Sympathectomy (ETS) to treat hyperhidrosis with palmar, axillar, facial and widespread localization. RESULTS: There were no deaths or major intra-operative problems. There was no conversion to open surgery. During intermediate and long-term follow up we obtained excellent results and few complications. In 3 patients (1.06%) we noted a major complication (Horner's Syndrome), but one patient only had the surgical operation reversed; compensatory sweating occurred in 61 individuals (21.7%). Gustatory sweating occurred in no patient. CONCLUSIONS: The clipping of the thoracic sympathetic chain is a reversible surgical technique considered safe and effective. Despite of an incidence of complications and/or compensatory sweating and according to our operatory results, we conclude that the sympathetic block by clamping is the elective treatment for hyperhidrosis.

Intracolonic release of nitric oxide during trinitrobenzene sulfonic acid rat colitis.

Nitric oxide is thought to play an important role in modulating the inflammatory process. Recently an increase in the inducible form of nitric oxide synthase (iNOS) has been found in the rat trinitrobenzene sulfonic acid model of experimental colitis, and inhibition of nitric oxide synthase activity resulted in an amelioration of tissue injury. The aim of our study was to evaluate in vivo intracolonic release of nitric oxide in this model of colitis. Experimental colitis was induced in male Sprague-Dawley rats by a single intracolonic administration of trinitrobenzene sulfonic acid. Nitrite levels were determined in rectal dialysates by HPLC. The tissue myeloperoxidase and iNOS and the luminal leukotriene B4 were also measured. Nitrite levels were significantly increased in rectal dialysates during colitis and correlated significantly with tissue myeloperoxidase and iNOS activity. The correlation between nitrite dialysate levels and wall iNOS activity confirms that nitrite in dialysates is produced by inflammatory cells and not by colonic bacterial flora. Determination of nitrite levels in rectal dialysates seems a valuable method to monitor colonic inflammation in rat trinitrobenzene sulfonic acid colitis.

[Multifocal Langerhans cell granulomatosis. Report of a clinical case]. / Granulomatosi a cellule di Langerhans multifocale. Presentazione di un caso clinico.

Langerhans' cell granulomatosis is a rare disease whose incidence on adults is valued about one case out of 3.000.000. The multifocal form is rarer than the single eosinophilic granuloma of the bone. It mainly affects males during childhood and it is usually localized in the long bones, hands, feet, and in the skull. We present the case of a young woman affected by multifocal Langerhans cell granulomatosis with bones and skin involved. Unusual in the presented clinical case is the age in which the disease come out. There is the highest incidence of multifocal lesions during childhood, while the monofocal kind is more typical of the years of adult age. Bone localization in the sternum is also quite unusual. In fact it is impossible to find reports of it in recent literature. As the patient refused to undergo chemotherapy she was treated with radiant therapy as for as bone localization concerned and with mecloretamin for local administration on skin lesion. Eleven months after the diagnosis we have not noticed the appearance of new lesions.

[Gastric and hematologic changes in patients with gastric parietal cell autoantibodies and non-insulin-dependent diabetes mellitus]. / Alterazioni gastriche ed ematologiche in pazienti con autoanticorpi anticellule parietali gastriche affetti da diabete mellito non insulino dipendente.

Gastric abnormalities are more common in diabetics than in the normal population. They seem to be related to gastric parietal cell autoantibodies (GPCA). We have studied 168 patients affected with non-insulin-dependent diabetes mellitus (NIDDM), and assessed the GPCA in the serum and haematologic disorders. We have also assessed the endoscopic and histological findings of atrophic gastritis in patients with GPCA. GPCA were found in 15.74% of diabetic patients and in 2% of a control group of blood donors. 80% of GPCA positive patients showed signs of atrophic gastritis. Such presence of GPCA seems to be a good marker to identify patients affected by atrophic gastritis and its complications.

[Prevalence of hepatitis C virus infection in a population of drug addicts and their heterosexual partners]. / Prevalenza della infezione da virus C della epatite in una popolazione di tossicodipendenti e dei loro partner eterosessuali.

This study reports data concerning the prevalence of anti HCV in a population of 166 drug addicts and 25 partners (14 drug addicts and 11 with no history of substance abuse) of subjects proven anti HCV positive. The study was carried out in order to evaluate the importance of the haematic and sexual modes of transmission of HCV. The results obtained showed a prevalence of HCV infection in drug addicts with substantial statistic significance when compared with the prevalence in a population of donors. There is no evidence of statistically significant differences in prevalence between non-addicted partners and blood donors or between addicted partners and the general population of drug addicts. From these comparisons it is clear that haematic contact is an important source of contagion, whereas sexual contact appears to be of minor importance.