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Newborn screening for galactosemia:a 30-year single center experience

World Journal of Pediatrics 2015年第2期11卷 160-164页

作者： francesco porta Severo Pagliardini Veronica Pagliardini Alberto Ponzone Marco Spada Department of Pediatrics University of TorinoTorinoItaly

Background:Galactosemia due to complete or near-complete galactose-l-phosphate uridyltransferase(GALT)deficiency was the first disorder added to the pioneering newborn screening panel besides *** the last 50 years,many criticisms have been focused on the opportunity of its ***,long-term single center experiences with this issue are generally ***:We reviewed the outcome of newborn screening for hypergalactosemia performed at our department since 1982 and the correspondent long-term clinical ***:Among 1123909 newborns screened for hypergalactosemia,33 showed abnormal results confirmed at second tier *** patients were affected with classic galactosemia,8 partial GALT deficiency,3 severe galactokinase deficiency,7 transient galactosemia,one congenital porto-systemic shunt,and one glucose transporter 2 *** neonatal liver failure in the late first week of life(5.8±1.1 days)unavoidably complicated the clinical course of classic galactosemia,unless in three second-born siblings treated on the basis of presumptive diagnosis immediately after newborn screening sample collection on day *** early treatment and longterm steadily normal peripheral blood galactose,77%of patients with severe GALT deficiency present mild to severe intellectual *** patients with partial GALT deficiency showed normal intellectual development on a regular diet,as well as patients with galactokinase deficiency under ***:Availability of screening results within the fifth day after birth would allow the prevention of acute decompensation in classic galactosemia.A systematic diagnostic work-up in all positive newborns is essential to unravel the etiology of hypergalactosemia.

关键词： galactose galactosemia liver failure mental retardation newborn screening

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Small diameter H-graft porta-caval shunt performed at different stages of liver disease

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Hepatobiliary & Pancreatic Diseases International 2004年第4期3卷 516-521页

作者： Giacomo Batignani francesco Vizzutti Luigi Rega Michele Zuckermann Geri Fratini Massimo Pinzani francesco Tonelli Dipartimento di Fisiopatologia Clinica-Unità di Chirurgia Firenze Italy Diparti- mento di Medicina Interna Sezione Clinica Medica ed Epatologia Laboratono di Emodinamica Epatica Firenze Italy Dipartimento di Fisiopatologia Clinica Unità di Radiodiagnostica Firenze Italy

BACKGROUND: Partial porto-systemic shunts have been popularized because of reported low rate of mortality and morbidity (especially encephalopathy, liver failure and oc- clusion). To further investigate these assumptions, we ret- rospectively reviewed the results of partial porta-caval shunts performed at different stages of liver disease. METHODS: Twenty-nine cirrhotic patients underwent a partial porta-caval shunt with a ringed polytetrafluoroethy- lene interposition prosthesis of 8-mm ( 20 patients) or 10- mm (9 patients) in diameter. Pre- and post-shunt porta- caval pressure was measured in all patients. Twelve patients (41.4%) belonged to Child A, 11 Child B (37.9%), and 6 Child C (20.7%). Eleven patients (37.9%) suffered from hepatic encephalopathy preoperatively. Twelve patients (41%) were operated on in emergency/urgency. RESULTS: porta-caval pressure gradient, reduced signifi- cantly using either 8- or 10-mm prosthesis. The overall ear- ly mortality and morbidity were 13. 8% and 48% respec- tively. The early mortality and morbidity were different be- tween patients of Child A and B when compared to those of Child C (0 vs 66.6% and 34.8% vs 66.6% respectively). No patient re-bled early from varices. The overall late mor- tality and morbidity were 40% and 64% respectively. Shunt thrombosis and stenosis took place in 16% and 8% of the two groups of patients respectively; variceal re-bleeding oc- curred in 4 patients (16%). Encephalopathy occurred post- operatively in 5 patients (20%), acute in 3 patients (12%), and chronic in 2 (8%). The actuarial survival rate at 3 and 5 years was 92% and 75% for patients of Child A, 70% and 60% for patients of Child B, and 0% for patients of Child C. CONCLUSIONS: Our results indicate that partial porta-ca- val shunt with a small diameter interposition H-graft is an effective procedure for the treatment of variceal bleeding, as well as for the prevention of re-bleeding in patients of Child A and those of Child B, as an electi

关键词： partial porto-systemic shunts portal hypertension cirrhosis variceal bleeding encephalopathy.

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First-line pazopanib in patients with advanced non-clear cell renal carcinoma:An Italian case series

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World Journal of Clinical Oncology 2021年第11期12卷 1037-1046页

作者： Sebastiano Buti Melissa Bersanelli francesco Massari Ugo De Giorgi Orazio Caffo Gaetano Aurilio Umberto Basso Giacomo Carteni Claudia Caserta Luca Galli francesco Boccardo Giuseppe Procopio Gaetano Facchini Giuseppe Fornarini Alfredo Berruti Elena Fea Emanuele Naglieri Fausto Petrelli Roberto Iacovelli Camillo porta Alessandra Mosca Medical Oncology Unit University Hospital of ParmaParma 43126Italy Medicine and Surgery Department University of ParmaParma 43126Italy Division of Oncology Policlinico Sant’Orsola-Malpighi HospitalBologna 40138Italy Department of Oncology IRCCS Istituto Scientifico Romagnolo per lo Studio e la Cura dei Tumori(I.R.S.T.)Meldola 47014Italy Department of Medical Oncology Santa Chiara HospitalTrento 38122Italy Department of Medical Oncology Division of Urogenital and Head and Neck TumoursEuropean Institute of Oncology IRCCSMilan 20141Italy Medical Oncology Unit 3 Istituto Oncologico Veneto IOV IRCCSCastelfranco VenetoPadova 31033Italy Division of Oncology Azienda Ospedaliera di Rilievo Nazionale A.CardarelliNapoli 80131Italy Medical Oncology Unit Azienda Ospedaliera S.MariaTerni 05100Italy Oncology Unit 2 University Hospital of PisaPisa 56126Italy Academic Unit of Medical Oncology IRCCS San Martino Polyclinic HospitalGenova 16132Italy Medical Oncology Genitourinary Section Fondazione IRCCS Istituto Nazionale Dei TumoriMilan 20133Italy Departmental Unit of Clinical and Experimental Uro-Andrologic Oncology Istituto Nazionale Tumori IRCCS Fondazione G.PascaleNapoli 80131Italy Medical Oncology Unit Istituto di Ricovero e Cura a Carattere Scientifico(IRCCS)Ospedale Policlinico San MartinoGenova 16132Italy University of Brescia ASST-Spedali CiviliBresciaBrescia 25123Italy Medical Oncology Unit S Croce and Carle Teaching HospitalCuneo 12100Italy Division of Medical Oncology Istituto Tumori G Paolo IIIRCCSBari 70124Italy Medical Oncology Unit ASST Bergamo OvestTreviglioBergamo 24047Italy Medical Oncology Unit Fondazione Policlinico Universitario Agostino Gemelli IRCCSRome 00161Italy Division of Oncology AOU Consorziale Policlinico di BariBari 70124Italy Department of Biomedical Sciences and Human Oncology University of Bari"A.Moro"Bari 70124Italy Multidisciplinary Outpatient Oncology Clinic Candiolo Cancer InstituteFPO-IRCCSCandioloTurin 10060Italy

BACKGROUND Non-clear cell(ncc)metastatic renal-cell carcinoma(RCC)has dismal results with standard systemic therapies and a generally worse prognosis when compared to its clear-cell *** systemic combination therapies have emerged for metastatic RCC(mRCC),but the pivotal phase III trials excluded patients with nccRCC,which constitute about 30%of metastatic RCC *** To provide a piece of real-life evidence on the use of pazopanib in this patient *** The present study is a multicenter retrospective observational analysis aiming to assess the activity,efficacy,and safety of pazopanib as first-line therapy for advanced nccRCC patients treated in a real-life *** Overall,48 patients were *** the median follow-up of 40.6 mo,the objective response rate was 27.1%,the disease control rate was 83.3%,and the median progression-free survival and overall survival were 12.3(95%confidence interval[CI]:3.6-20.9)and 27.7(95%CI:18.2-37.1)mo,*** 3 adverse events occurred in 20%of patients,and no grade 4 or 5 toxicities were *** Pazopanib should be considered as a good first-line option for metastatic RCC with variant histology.

关键词： Pazopanib Non-clear cell Kidney cancer Renal-cell carcinoma Variant histology Tyrosine kinase inhibitors

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儿童早期空气污染与呼吸道感染:ESCAPE项目中10个欧洲出生队列的分析

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环境与职业医学 2014年第3期31卷 202-202页

作者： Elaina A.MacIntyre Ulrike Gehring Anna Molter Elaine Fuertes Claudia Klümper Ursula Kramer Ulrich Quass Barbara Hoffmann Mireia Gascon Bert Brunekreef Gerard H.Koppelman Rob Beelen Gerard Hoek Matthias Birk Johan C.de Jongste H.A.Smit Josef Cyrys Olena Gruzieva Michal Korek Anna Bergstrom Raymond M.Agius Frank de Vocht Angela Simpson Daniela porta francesco Forastiere Chiara Badaloni Giulia Cesaroni Ana Esplugues Ana Fernández-Somoano Aitana Lerxundi Jordi Sunyer Marta Cirach Mark J.Nieuwenhuijsen Goran Pershagen Joachim Heinrich

[背景]将交通源空气污染作为幼儿期呼吸道感染的危险因素的研究很少。[目的]在10个欧洲出生队列——BAMSE（瑞典）、GASPⅡ（意大利）、GINIplus和LISAplus（德国）、MAAS（英国）、PIAMA（荷兰）和4个INMA队列（西班牙）——中研究空... 详细信息

[背景]将交通源空气污染作为幼儿期呼吸道感染的危险因素的研究很少。[目的]在10个欧洲出生队列——BAMSE（瑞典）、GASPⅡ（意大利）、GINIplus和LISAplus（德国）、MAAS（英国）、PIAMA（荷兰）和4个INMA队列（西班牙）——中研究空气污染与肺炎、哮喘及中耳炎之间的相关性，并使用meta分析得出综合效应估值。[方法]评价家长报告的医师诊断的儿童早期肺炎、中耳炎以及哮喘与全年平均污染物水平[二氧化氮（NO2）、氮氧化物（NOx）、≤2．5gm的颗粒物（PM2.5）、PM2.5吸光度、PM10、PM2.5-10（粗PM）]间的关联，污染物浓度的估计采用土地利用回归模型并根据孩子的出生住址分配。作为ESCAPE项目的一部分，每个研究区域都采用相同的方案建立回归模型。用logistic回归来计算每个研究校正后的效应估值，用随机效应meta分析来计算合并的估计值。[结果]除了PM2.5外的所有污染物，肺炎的合并校正优势比（OR）均升高（例如，N02每增加10μg／m3，OR=1．30，95％CI：1．02～1．65；PM10每增加10μg／m3的OR=1．76，95％CI：1．00—3．09）。对于中耳炎和哮喘，除了NO2与中耳炎问的关联（每10μg／m3对应的OR=1．09，95％CI：1．02～1．16），所有分析的结果均无显著性。[结论]对ESCAPE项目中10个欧洲出生队列进行的meta分析为空气污染和儿童早期肺炎之间的相关性找到一致性的证据，也为与中耳炎间的相关性找到了一些证据。

关键词：早期肺炎呼吸道感染出生队列空气污染欧洲儿童 meta分析 logistic

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