Paola Francalanci

Summary

Country: Italy

Publications

  1. pmc Gene and functional up-regulation of the BCRP/ABCG2 transporter in hepatocellular carcinoma
    Caecilia Hc Sukowati
    Centro Studi Fegato, Fondazione Italiana Fegato, Bld Q AREA Science Park Basovizza, Trieste, Italy
    BMC Gastroenterol 12:160. 2012
  2. ncbi Malignant pancreatic endocrine tumor in a child with tuberous sclerosis
    Paola Francalanci
    Department of Pathology, Children s Hospital Bambino Gesu, Piazza Sant Onofrio 4, Rome, Italy
    Am J Surg Pathol 27:1386-9. 2003
  3. ncbi CD44-v6 expression in smooth muscle cells in the postnatal remodeling process of ductus arteriosus
    Paola Francalanci
    Department of Pathology, Bambino Gesu Children s Hospital, Rome, Italy
    Am J Cardiol 97:1056-9. 2006
  4. doi Eosinophilic esophagitis and Barrett's esophagus: an occasional association or an overlap disease? Esophageal 'double trouble' in two children
    Paola Francalanci
    Department of Pathology, Children s Hospital Bambino Gesu, Rome, Italy
    Digestion 77:16-9. 2008
  5. doi Z and Mmalton-1-antitrypsin deficiency-associated hepatocellular carcinoma: a genetic study
    Paola Francalanci
    Department of Pathology, Bambino Gesu Children s Hospital, Rome, Italy
    Liver Int 29:1593-6. 2009
  6. ncbi Expression of the lipogenic enzyme fatty acid synthase (FAS) as a predictor of poor outcome in nephroblastoma: an interinstitutional study
    Francesca Diomedi Camassei
    Department of Pathology, Ospedale Pediatrico Bambino Gesù IRCCS, Rome, Italy
    Med Pediatr Oncol 40:302-8. 2003
  7. ncbi Pulmonary blastomas of childhood: histologic, immunohistochemical, ultrastructural aspects and therapeutic considerations
    Renata Boldrini
    Department of Pathology, Bambino Gesu Pediatric Hospital, IRCCS, Rome, Italy
    Ultrastruct Pathol 29:493-501. 2005
  8. doi Tyrosinemia type 1: metastatic hepatoblastoma with a favorable outcome
    Valerio Nobili
    Division of Metabolism, Bambino Gesu Children s Hospital, Rome, Italy
    Pediatrics 126:e235-8. 2010
  9. pmc Genetics and Molecular Modeling of New Mutations of Familial Intrahepatic Cholestasis in a Single Italian Center
    Isabella Giovannoni
    Dept Pathology and Molecular Histopathology, Bambino Gesu Children s Hospital, IRCCS, Rome, Italy
    PLoS ONE 10:e0145021. 2015
  10. doi Malignant pheochromocytoma in a 16-year-old patient with neurofibromatosis type 1
    Isabella Giovannoni
    1 Department of Pathology, Children s Hospital Bambino Gesu, IRCCS Rome, Italy
    Pediatr Dev Pathol 17:126-9. 2014

Collaborators

Detail Information

Publications30

  1. pmc Gene and functional up-regulation of the BCRP/ABCG2 transporter in hepatocellular carcinoma
    Caecilia Hc Sukowati
    Centro Studi Fegato, Fondazione Italiana Fegato, Bld Q AREA Science Park Basovizza, Trieste, Italy
    BMC Gastroenterol 12:160. 2012
    ..Since data on ABCG2 expression in liver malignances are scanty, here we report the expression of ABCG2 in adult human hepatocellular carcinoma (HCC) in both in vivo and in vitro models with different degree of malignancy...
  2. ncbi Malignant pancreatic endocrine tumor in a child with tuberous sclerosis
    Paola Francalanci
    Department of Pathology, Children s Hospital Bambino Gesu, Piazza Sant Onofrio 4, Rome, Italy
    Am J Surg Pathol 27:1386-9. 2003
    ..These findings suggest a role for tuberin, the TSC2 gene product, in the pathogenesis of malignant pancreatic endocrine tumor...
  3. ncbi CD44-v6 expression in smooth muscle cells in the postnatal remodeling process of ductus arteriosus
    Paola Francalanci
    Department of Pathology, Bambino Gesu Children s Hospital, Rome, Italy
    Am J Cardiol 97:1056-9. 2006
    ..In conclusion, the present data provide evidence that closure of DA involves synthetic SMCs highly positive for CD44-v6, and patent or closed DAs are populated by CD44-v6-negative SMCs...
  4. doi Eosinophilic esophagitis and Barrett's esophagus: an occasional association or an overlap disease? Esophageal 'double trouble' in two children
    Paola Francalanci
    Department of Pathology, Children s Hospital Bambino Gesu, Rome, Italy
    Digestion 77:16-9. 2008
    ..Barrett's esophagus (BE) is due to chronic gastroesophageal reflux. The pathological consequence is the replacement of normal stratified squamous epithelium by columnar mucosa with goblet cells...
  5. doi Z and Mmalton-1-antitrypsin deficiency-associated hepatocellular carcinoma: a genetic study
    Paola Francalanci
    Department of Pathology, Bambino Gesu Children s Hospital, Rome, Italy
    Liver Int 29:1593-6. 2009
    ..It is noteworthy that hepatocellular carcinoma (HCC) arises specifically from the AAT-negative areas but the reason for this remains unclear...
  6. ncbi Expression of the lipogenic enzyme fatty acid synthase (FAS) as a predictor of poor outcome in nephroblastoma: an interinstitutional study
    Francesca Diomedi Camassei
    Department of Pathology, Ospedale Pediatrico Bambino Gesù IRCCS, Rome, Italy
    Med Pediatr Oncol 40:302-8. 2003
    ..Correlation of elevated levels of the lipogenic enzyme fatty acid synthase (FAS) with aggressiveness of some cancers has drawn attention to this enzyme as a possible marker of poor prognosis...
  7. ncbi Pulmonary blastomas of childhood: histologic, immunohistochemical, ultrastructural aspects and therapeutic considerations
    Renata Boldrini
    Department of Pathology, Bambino Gesu Pediatric Hospital, IRCCS, Rome, Italy
    Ultrastruct Pathol 29:493-501. 2005
    ..The expression of the transmembrane tyrosin kinase receptor c-kit in all the solid cases of this series leads the authors to hypothesize new possible therapeutic implications for these tumors...
  8. doi Tyrosinemia type 1: metastatic hepatoblastoma with a favorable outcome
    Valerio Nobili
    Division of Metabolism, Bambino Gesu Children s Hospital, Rome, Italy
    Pediatrics 126:e235-8. 2010
    ..We encourage clinicians to perform more accurate evaluation of liver histology, because a neoplastic mass in a child with tyrosinemia type 1 is not the same as HCC...
  9. pmc Genetics and Molecular Modeling of New Mutations of Familial Intrahepatic Cholestasis in a Single Italian Center
    Isabella Giovannoni
    Dept Pathology and Molecular Histopathology, Bambino Gesu Children s Hospital, IRCCS, Rome, Italy
    PLoS ONE 10:e0145021. 2015
    ..Two cases were compound heterozygous for BRIC/PFIC2 mutations. Our results highlight the importance of the pathogenic role of novel mutations in the three genes and unusual modes of their transmission. ..
  10. doi Malignant pheochromocytoma in a 16-year-old patient with neurofibromatosis type 1
    Isabella Giovannoni
    1 Department of Pathology, Children s Hospital Bambino Gesu, IRCCS Rome, Italy
    Pediatr Dev Pathol 17:126-9. 2014
    ..On the other hand, since 30% of PHEOs had germline mutations and, more rarely, somatic mutations, patients with PHEO should be investigated for associated genetic syndromes...
  11. ncbi Severe liver disease in early childhood due to fibrinogen storage and de novo gamma375Arg-->Trp gene mutation
    Paola Francalanci
    Department of Pathology, Children s Hospital Bambino Gesu, Rome, Italy
    J Pediatr 148:396-8. 2006
    ..Fibrinogen gene analysis revealed a de novo Aguadilla (c.1201C>T; p.Arg375Trp) mutation. This mutation should be considered in childhood hypofibrinogenemia associated with chronic liver disease...
  12. doi Two novel mutations in African and Asian children with progressive familial intrahepatic cholestasis type 3
    Isabella Giovannoni
    Dep Pathology, Children s Hospital Bambino Gesu, Rome, Italy
    Dig Liver Dis 43:567-70. 2011
    ..Defects in ABCB4 have been found to cause progressive familial intrahepatic cholestasis type 3. Liver histology is important, but not specific, for diagnosis. Genotyping is conclusive...
  13. pmc The Impact of Specific Viruses on Clinical Outcome in Children Presenting with Acute Heart Failure
    Maria Giulia Gagliardi
    Medical and Surgical Department of Cardiology, Children s Hospital Bambino Gesu, Piazza S Onofrio 4, 00165 Rome, Italy
    Int J Mol Sci 17:486. 2016
    ..07). In our series, presence of a virus-positive EMB (mainly PVB19) was associated with improvement over time in cardiac function and better long-term prognosis. ..
  14. doi Bile salt export pump deficiency: A de novo mutation in a child compound heterozygous for ABCB11. Laboratory investigation to study pathogenic role and transmission of two novel ABCB11 mutations
    Paola Francalanci
    Department of Pathology Liver Unit Research Laboratories, Bambino Gesu Children s Hospital, IRCCS, Rome, Italy
    Hepatol Res 43:315-9. 2013
    ..This case stresses that clinical PFIC with an attenuated rather than absent BSEP immunostaining can still be due to ABCB11 mutations presumably encoding a functionally deficient protein...
  15. doi Pediatric adrenocortical neoplasms: immunohistochemical expression of p57 identifies loss of heterozygosity and abnormal imprinting of the 11p15.5
    Isabella Giovannoni
    Department of Pathology, Bambino Gesu Children s Hospital, IRCCS, Rome, Italy
    Pediatr Res . 2017
    ..The identification of loss of heterozygosity (LOH) of 11p15, containing the p57 gene, could be a tool for differential diagnosis of benign and malignant ACN...
  16. doi Alpha-1-antitrypsin deficiency: from genoma to liver disease. PiZ mouse as model for the development of liver pathology in human
    Isabella Giovannoni
    Department of Pathology, Children s Hospital Bambino Gesu, IRCCS, Rome, Italy
    Liver Int 35:198-206. 2015
    ..The aim of this study was to determine liver pathology in PiZ mice, focusing the attention on the distribution of AAT globules in normal liver, regenerative foci and neoplastic nodules...
  17. ncbi Paternal isodisomy of chromosome 2 in a child with bile salt export pump deficiency
    Isabella Giovannoni
    Units of Pathology Molecular Medicine and Neurosciences Hepatic Surgery, Children s Hospital Bambino Gesu, Rome Unit of Pathology, Molinette Hospital, Turin IRCCS Fondazione Stella Maris, Pisa, Italy
    Hepatol Res 42:327-31. 2012
    ..Recognizing isodisomic transmission may have a significant impact on genetic counseling helping to define the risk of recurrence in subsequent pregnancies...
  18. doi Heart transplant and 2-year follow up in a child with generalized arterial calcification of infancy
    Isabella Giovannoni
    Department of Pathology and Molecular Histopathology, Bambino Gesu Children s Hospital, IRCCS, Rome Piazza Sant Onofrio, 4 00165, Rome, Italy
    Eur J Pediatr 173:1735-40. 2014
    ..Two years after transplantation, the child shows good clinical conditions and growth with no recurrence of calcium deposits in the heart...
  19. doi Focal congenital hyperinsulinism managed by medical treatment: a diagnostic algorithm based on molecular genetic screening
    Arianna Maiorana
    Department of Pediatrics, Metabolic Unit, IRCCS Bambino Gesù Children s Hospital, Rome, Italy
    Clin Endocrinol (Oxf) 81:679-88. 2014
    ..Objective of this report is proposing a new algorithm in which early genetic screening, rather than diazoxide trial, dictates subsequent clinical decisions...
  20. pmc Genotype-phenotype relationship in human ATP6i-dependent autosomal recessive osteopetrosis
    Anna Taranta
    Istituto Dermopatico dell Immacolata, Rome, Italy
    Am J Pathol 162:57-68. 2003
    ..Bone marrow transplantation was successful in all patients, and posttransplant osteoclasts showed rescue of a3 subunit immunoreactivity...
  21. doi Eosinophilic esophagitis: is it also a surgical disease?
    Francesca Rea
    Digestive Surgery and Endoscopy Unit, Pediatric Hospital and Research Institute Bambino Gesù IRCCS, Rome, 00165, Rome, Italy
    J Pediatr Surg 48:304-8. 2013
    ..An association between EoE and gastro-esophageal reflux disease (GERD) has not been well established...
  22. doi Autoimmune hepatitis type 2 following anti-papillomavirus vaccination in a 11-year-old girl
    Claudia Della Corte
    Unit of Liver Research, Department of Pathology, Bambino Gesu Children s Hospital, IRCCS, P le S Onofrio 4, 00165 Rome, Italy
    Vaccine 29:4654-6. 2011
    ....
  23. pmc Novel large-range mitochondrial DNA deletions and fatal multisystemic disorder with prominent hepatopathy
    Marzia Bianchi
    Unit of Molecular Medicine for Neuromuscular and Neurodegenerative Diseases, Bambino Gesu Children s Hospital, Rome, Italy
    Biochem Biophys Res Commun 415:300-4. 2011
    ..Our data further expand the array of deletions in the mitochondrial genomes in association with liver failure. Thus analysis of mtDNA should be considered in the diagnosis of childhood-onset hepatopathies...
  24. doi Preemptive liver transplantation in a child with familial hypercholesterolemia
    Arianna Maiorana
    Metabolic Unit, Bambino Gesu Children Hospital, Rome, Italy
    Pediatr Transplant 15:E25-9. 2011
    ..Preemptive liver transplantation is a definitive cure of familial homozygous hypercholesterolemia and might be more effective if performed before development of vascular complications...
  25. ncbi A new ABCB11 mutation in two Italian children with familial intrahepatic cholestasis
    Valerio Nobili
    Hepatology, IRCCS Bambino Gesù Hospital, Piazza S Onofrio, 4 00165, Rome, Italy
    J Gastroenterol 41:598-603. 2006
    ..Our findings are expected to facilitate detection of the molecular cause of PFIC in affected families...
  26. ncbi Cystic dysplasia of the rete testis: report of two cases and review of the literature
    Francesca Diomedi Camassei
    Department of Pathology, Bambino Gesu Children s Hospital Research Institute, Piazza S Onofrio 4, 00165 Rome, Italy
    Pediatr Dev Pathol 5:206-10. 2002
    ..Orchiectomy was performed in both patients, for extensive gonad involvement in the older boy and for suspected gonad torsion in the newborn patient...
  27. ncbi Decreased CDKN1C Expression in Congenital Alveolar Rhabdomyosarcoma Associated with Beckwith-Wiedemann Syndrome
    Fiammetta Piersigilli
    Department of Medical and Surgical Neonatology, Bambino Gesu Children s Hospital, Piazza S Onofrio 4, 00165, Rome, Italy
    Indian J Pediatr 83:1476-1478. 2016
    ..Genetic analysis showed lack of the two characteristic translocations in the tumor tissue, supporting a different oncogenic pathway of alveolar RMS in children with BWS...
  28. doi Association of DiGeorge anomaly and caudal dysplasia sequence in a neonate born to a diabetic mother
    Maria L Dentici
    Department of Medical Genetics, Cardiology and Pathology, Bambino Gesu Pediatric Hospital, IRCCS, Rome, Italy
    Cardiol Young 23:14-7. 2013
    ..Infants with DiGeorge anomaly can display additional manifestations within the spectrum of caudal dysplasia sequence, including vertebral anomalies and renal agenesis...
  29. ncbi Nephroblastoma: multidrug-resistance P-glycoprotein expression in tumor cells and intratumoral capillary endothelial cells
    Francesca Diomedi Camassei
    Department of Pathology, Bambino Gesu Children s Hospital Research Institute, Rome, Italy
    Am J Clin Pathol 117:484-90. 2002
    ..Previous chemotherapy induced P-gp overexpression in tumor cells...
  30. ncbi Cystitis glandularis in children
    Nicola Capozza
    Division of Paediatric Urology, Bambino Gesu Children s Hospital, Research Institute, Piazza S Onofrio, 4 00165, Rome, Italy
    BJU Int 95:411-3. 2005
    ..To assess the characteristics of cystitis glandularis in children...