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

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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 phenylketonuria.In the last 50 years,many criticisms have been focused on the opportunity of its inclusion.Consequently,long-term single center experiences with this issue are generally lacking.Methods:We reviewed the outcome of newborn screening for hypergalactosemia performed at our department since 1982 and the correspondent long-term clinical outcome.Results:Among 1123909 newborns screened for hypergalactosemia,33 showed abnormal results confirmed at second tier test.Thirteen 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 deficiency.Acute 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 3.Despite early treatment and longterm steadily normal peripheral blood galactose,77%of patients with severe GALT deficiency present mild to severe intellectual disabilities.All patients with partial GALT deficiency showed normal intellectual development on a regular diet,as well as patients with galactokinase deficiency under treatment.Conclusions: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

Gastric duplication associated with pancreas divisum diagnosed by a multidisciplinary approach before surgery

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World Journal of Gastroenterology 2010年第8期16卷 1031-1033页

作者： Marta Di Pisa Gabriele Curcio Gianluca Marrone Mariapina Milazzo marco spada Mario Traina Mario Traina Gastroenterology Service Department of Radiology IsMeTT-UPMC Department of Surgery IsMeTT-UPMC

We report a unique case of communicating gastric duplication associated with pancreas divisum,diagnosed with a multidisciplinary approach,including X-rays,computed tomography,magnetic resonance imaging,esophagogastro duodenoscopy,ultrasound endoscopy and histology. We believe that this approach constitutes a fuller diagnostic evaluation,resulting in better and safer surgery.

关键词： Gastric duplication Pancreas divisum Computed tomography Magnetic resonance imaging Ultra-sonic endoscopy

Completely obstructed colorectal anastomosis: A new non-electrosurgical endoscopic approach before balloon dilatation

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World Journal of Gastroenterology 2010年第37期16卷 4751-4754页

作者： Gabriele Curcio marco spada Fabrizio di Francesco Ilaria Tarantino Luca Barresi Gaetano Burgio Mario Traina Department of Gastroenterology IsMeTT UPMC

Benign stricture is a relatively common complication of colorectal anastomosis after low anterior resection. On occasion, the anastomosis may close completely. A variety of endoscopic techniques have been described, but there is a lack of data from controlled prospective trials as to the optimal approach. Through-the-scope balloon dilatation is well known and easy to perform. Some case reports describe different endoscopic approaches, including endoscopic electrocision with a papillotomy knife or hook knife. We report a case of a colorectal anastomosis web occlusion, treated without electrocision. Gastrografin enema and sigmoidoscopy showed complete obstruction at the anastomotic site due to the presence of an anastomotic occlusive web. In order to avoid thermal injuries, we decided to use a suprapapillary biliary puncture catheter. The Artifon catheter was inserted into the center of the circular staple line at the level of the anastomosis, and fluoroscopic identification of the proximal bowel was obtained with dye injection. A 0.025-inch guidewire was then passed through the catheter into the colon and progressive pneumatic dilatation was performed. The successful destruction of the occlusive web facilitated passage of the colonoscope, allowing evaluation of the entire colon and stoma closure after three months of follow-up. The patient tolerated the procedure well, with no complications. This report highlights an alternative non-electrosurgical approach that uses a new device that proved to be safe and useful.

关键词： Anastomosis Dilation Balloon Obstructed Artifon

Tulip bundle technique and fibrin glue injection:Unusual treatment of colonic perforation

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World Journal of Gastroenterology 2011年第8期17卷 1088-1090页

作者： Filippo Mocciaro Gabriele Curcio Ilaria Tarantino Luca Barresi marco spada Sergio Li Petri Mario Traina Department of Gastroenterology IsMeTT UPMC Surgery Unit IsMeTT UPMC

We report a case of a 63-year-old male who experienced an iatrogenic sigmoid perforation repaired combining three endoscopic techniques.The lesion was large and irregular with three discrete perforations,therefore,we decided to close it by placing one clip per perforation,and then connecting all the clips with two endoloops.Finally we chose to use a fibrin glue injection to obtain a complete sealing.Four days after the colonoscopy the patient underwent a laparoscopic right hemicolectomy due to evidence of a large polyp of the caecum with high grade dysplasia and focal carcinoma in situ.Inspection of the sigma showed complete repair of the perforation.This report underlines how a conservative approach,together with a combination of various endoscopic techniques,can resolve complicated iatrogenic perforations of the colon.

关键词：纤维蛋白胶结肠镜穿孔技术郁金香治疗 Sigma 检查表

Different modalities of arterial reconstruction in hepatic retransplantation using right partial graft

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World Journal of Gastroenterology 2009年第26期15卷 3322-3323页

作者： Salvatore Gruttadauria Fabrizio di Francesco marco spada Mariapina Milazzo Bruno Gridelli Mediterranean Institute for Transplant and Advanced Specialized Therapies University of Pittsburgh Medical Center in Italy

Though split-liver and living-related transplantation are routinely performed,they are done almost exclusively for primary liver transplantation because of potential surgical difficulties.These difficulties are generally related to arterial revascularization,particularly if there is hepatic artery thrombosis.According to UNOS data,of the hepatic retransplantations performed between 1996 and 2007,only 8.7% were done using right or extended right grafts from deceased donors,and 14.3% using right grafts from live donors.Here we report our experience with 5 hepatic retransplantations in which right partial grafts resulting from conventional in situ splits,and one right lobe resulting from an adult-toadult living-related transplant,were successfully used with different modalities of graft arterialization.

关键词：动脉血管移植肝脏使用权原发性肝癌血栓形成肝动脉权利

Long-term outcome of pancreatic function following oncological surgery in children:Institutional experience and review of the literature

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World Journal of Clinical Cases 2021年第25期9卷 7340-7349页

作者： Giulia Bolasco Teresa Capriati Chiara Grimaldi Lidia Monti Maria Debora De Pasquale Ippolita Patrizia Patera marco spada Giuseppe Maggiore Antonella Diamanti Hepatology Gastroenterology and Nutrition Unit“Bambino Gesù”Children’s HospitalIRCCSRome 00165Italy Division of Hepatobiliopancreatic Surgery Liver and Kidney Transplantation"Bambino Gesù"Children's HospitalIRCCSRome 00165Italy Radiology Department "Bambino Gesù"Children’s HospitalIRCCSRome 00165Italy Hematology/Oncology Department "Bambino Gesu"Children's HospitalIRCCSRome 00165Italy Endocrinology and Diabetes Unit "Bambino Gesù"Children’s HospitalIRCCSRome 00165Italy

BACKGROUND Pancreatic neoplasms are uncommon in children and in most cases they are benign or have low malignant potential.Pancreatoblastoma and solid pseudopapillary tumor are the most frequent types in early and late childhood,respectively.Complete resection,although burdened by severe complications,is the only curative treatment for these diseases.Pancreatic surgery may result in impaired exocrine and endocrine pancreatic function.However,limited data are available on the long-term pediatric pancreatic function following surgical resection.AIM To investigate endocrine and exocrine pancreatic function and growth after oncological pancreatic surgery in a pediatric series.METHODS A retrospective analysis of all pediatric patients who underwent surgery for pancreatic neoplasm in our Institution from January 31,2002 to the present was performed.Endocrine and exocrine insufficiency,auxological and fat-soluble vitamin status(A,D,E and clotting tests)were assessed at diagnosis and at every follow-up visit.Exocrine insufficiency was defined as steatorrhea with fecal elastase-1<200μg/g stool,while endocrine insufficiency was identified as onset of Diabetes or Impaired Glucose Tolerance.Growth was evaluated based on body mass index(BMI)z-score trend.RESULTS Sixteen patients(12 girls and 4 boys,mean age 10.7±5.3 years),were included.Nine patients(56%)had a neoplasm in the pancreatic head,4 in the body/tail,2 in the tail and 1 in the body.Histological findings were as follows:Solid pseudopapillary tumor in 10 patients(62.5%),insulinoma in 2 patients,neuroendocrine tumor in 2 patients and acinar cell carcinoma in 2 patients.The most frequent surgery was pancreaticoduodenectomy(50%).Exocrine failure occurred in 4 patients(25%)and endocrine failure in 2 patients(12.5%).Exocrine insufficiency occurred early(within 6 mo after surgery)and endocrine insufficiency later(8 and 10 years after surgery).Mean BMI z-score was 0.36±1.1 at diagnosis and 0.27±0.95 at the last assessment.Vitamin D was insufficient(<30 ng/mL)in 8 of the 16 p

关键词： Pancreatic neoplasms Pancreatic function Children Malabsorption Pancreatic failure Growth

Combined endovascular-surgical treatment for complex congenital intrahepatic arterioportal fistula: A case report and review of the literature

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World Journal of Hepatology 2020年第4期12卷 160-169页

作者： Roberta Angelico Guglielmo Paolantonio Monica Paoletti Chiara Grimaldi Maria Cristina Saffioti Lidia Monti Manila Candusso Massimo Rollo marco spada Department of Abdominal Transplantation and Hepatobiliary and Pancreatic Surgery Bambino GesùChildren’s Hospital IRCCSRome 00165Italy Department of Surgery Hepato-bilio-pancreatic Surgery and Transplant UnitUniversity of Rome Tor VergataFondazione PVTRome 00133Italy Interventional Radiology Unit Department of ImagingBambino GesùChildren’s Hospital IRCCSRome 00165Italy Department of Imaging Bambino GesùChildren’s Hospital IRCCSRome 00165Italy Division of Hepatogastroenterology Bambino GesùChildren’s Hospital IRCCSRome 00165Italy

BACKGROUND Congenital intrahepatic arterioportal fistula(IAPF) is a rare vascular malformation in infants that causes severe portal hypertension(PH) with poor prognosis if untreated. Currently, radiological embolisation is considered the first-line therapy for simple IAPF; however, it might be not resolutive for complex hepatic vascular lesions. When endovascular embolization is not sufficient to completely obliterate the IAPF, surgical intervention is needed, but it has been associated with severe morbidity and mortality in small children.Furthermore, indications are not defined.CASE SUMMARY We present the first case of a 6-month-old girl with trisomy 21 affected by a complex congenital IAFP, which caused severe PH, successfully treated with an endovascular-surgical hybrid procedure. The novel technique comprised a multistep endovascular embolisation, including a superselective transarterial embolisation of the afferent vessels and a direct transhepatic embolisation of the dilated portal vein segment, combined with selective surgical ligation of the arterial branches that supply the fistula, which were too small to be embolised.The complex IAPF was also associated with severe cholestasis and intra/extrahepatic biliary tree dilatation, which was successfully treated by a temporary biliary drainage. At 24-mo follow-up, the hybrid endovascularsurgical procedure achieved complete occlusion of the complex IAPF and resolution of the PH. A comprehensive review of the literature on congenital IAPF management, focussed on alternative treatment strategies, is also reported.CONCLUSION The combined radiological-surgical approach is a safe and effective treatment option for complex IAPF and avoids major invasive surgery.

关键词： Liver Intrahepatic arterioportal fistula Congenital malformation Portal hypertension Radiological embolization Hepatic surgery Case report

Early graft dysfunction following adult-to-adult livingrelated liver transplantation:Predictive factors and outcomes

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World Journal of Gastroenterology 2009年第36期15卷 4556-4560页

作者： Salvatore Gruttadauria Fabrizio di Francesco Giovanni Battista Vizzini Angelo Luca marco spada Davide Cintorino Sergio Li Petri Giada Pietrosi Duilio Pagano Bruno Gridelli Department of Surgery University of PittsburghCoordinator Abdominal Adult Transplant.ISMETT (Mediterranean Institute for Transplant and Advanced Specialized TherapiesUniversity of Pittsburgh Medical Center in Italy)Via E. Tricomi N.190127 PalermoItaly Thomas Starzl Transplantation Institute University of Pittsburgh Medical CenterPittsburgh15213 PAUnited States

AIM:To describe a condition that we define as early graft dysfunction(EGD)which can be identified preoperatively. METHODS:Small-for-size graft dysfunction following living-related liver transplantation(LRLT)is characterized by EGD when the graft-to-recipient body weight ratio(GRBWR)is below 0.8%.However, patients transplanted with GRBWR above 0.8%can develop dysfunction of the graft.In 73 recipients of LRLT(GRBWR>0.8%),we identified 10 patients who developed EGD.The main measures of outcomes analyzed were overall mortality,number of re-transplants and length of stay in days(LOS).Furthermore we analyzed other clinical pre-transplant variables,intraoperative parameters and post transplant data.RESULTS:A trend in favor of the non-EGD group(3-mo actuarial survival 98%vs 88%,P=0.09;3-mo graft mortality 4.7%vs 20%,P=0.07)was observed as well as shorter LOS(13 d vs 41.5 d;P=0.001)and smaller requirement of peri-operative Units of Plasma (4 vs 14;P=0.036).Univariate analysis of pre- transplant variables identified platelet count,serum bilirubin,INR and Meld-Na score as predictors of EGD. In the multivariate analysis transplant Meld-Na score (P=0.025,OR:1.175)and pretransplant platelet count(P=0.043,OR:0.956)were independently associated with EGD. CONCLUSION:EGD can be identified preoperatively and is associated with increased morbidity after LRLT. A prompt recognition of EGD can trigger a timely treatment.

关键词：功能障碍肝移植成年人预测早期血小板计数十二指肠多因素分析

De novo malignancies after liver transplantation: The effect of immunosuppression-personal data and review of literature

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World Journal of Gastroenterology 2019年第35期25卷 5356-5375页

作者： Tommaso Maria Manzia Roberta Angelico Carlo Gazia Ilaria Lenci Martina Milana Oludamilola T Ademoyero Domiziana Pedini Luca Toti marco spada Giuseppe Tisone Leonardo Baiocchi HPB and Transplant Unit Department of Surgery University of Rome Tor Vergata Rome 00133 Italy Division of Abdominal Transplantation and HPB Surgery Bambino Gesù Children's Hospital IRCCS Rome 00165 Italy Wake Forest Institute for Regenerative Medicine Winston-Salem NC 27101 United States Hepatology and Liver Transplant Unit University of Tor Vergata Rome 00133 Italy

BACKGROUND Immunosuppression has undoubtedly raised the overall positive outcomes in the post-operative management of solid organ transplantation. However, long-term exposure to immunosuppression is associated with critical systemic morbidities. De novo malignancies following orthotopic liver transplants (OLTs) are a serious threat in pediatric and adult transplant individuals. Data from different experiences were reported and compared to assess the connection between immunosuppression and de novo malignancies in liver transplant patients. AIM To study the role of immunosuppression on the incidence of de novo malignancies in liver transplant recipients. METHODS A systematic literature examination about de novo malignancies and immunosuppression weaning in adult and pediatric OLT recipients was described in the present review. Worldwide data were collected from highly qualified institutions performing OLTs. Patient follow-up, immunosuppression discontinuation and incidence of de novo malignancies were reported. Likewise, the review assesses the differences in adult and pediatric recipients by describing the adopted immunosuppression regimens and the different type of diagnosed solid and blood malignancy.RESULTS Emerging evidence suggests that the liver is an immunologically privileged organ able to support immunosuppression discontinuation in carefully selected recipients. Malignancies are often detected in liver transplant patients undergoing daily immunosuppression regimens. Post-transplant lymphoproliferative diseases and skin tumors are the most detected de novo malignancies in the pediatric and adult OLT population, respectively. To date, immunosuppression withdrawal has been achieved in up to 40% and 60% of well-selected adult and pediatric recipients, respectively. In both populations, a clear benefit of immunosuppression weaning protocols on de novo malignancies is difficult to ascertain because data have not been specified in most of the clinical experiences. CONCLUSION The selected populations of toleran

关键词： Pediatric liver transplant Immunosuppression weaning Clinical operational tolerance Adult liver transplant Graft rejection Immune system De novo malignancies Immunosuppression minimization Cancer