Publications by authors named "Francesca Tel"

22 Publications

  • Page 1 of 1

Haploidentical Hematopoietic Stem Cell Transplant Complicated by Atypical Hemolytic Uremic Syndrome and Kidney Transplant From the Same Donor With No Immunosuppression but C5 Inhibition.

Transplantation 2019 02;103(2):e48-e51

Hematology and Bone Marrow Transplantation Unit, IRCCS S. Raffaele Scientific Institution, Milan, Italy.

Background: Atypical hemolytic uremic syndrome (aHUS) is life-threatening condition particularly when complicating allograft hematopoietic stem cell transplant (HSCT). In the past, the outcome was very poor with the majority of patients reaching end-stage renal disease or dying with little or no chances of kidney transplant (KTx) due to the high risk of relapse. The availability of C5 inhibition has opened up significant therapeutic opportunities and has improved the outcome particularly if complement dysregulation (CD) is the underlying pathogenetic mechanism.

Methods: We describe a peculiar case of a girl with aHUS complicating HSCT and her subsequent successful KTx received from the same donor thus performed without immunosuppression but anti-C5 inhibition.

Results: Soon after HSCT performed for acute lymphoblastic leukemia, the patient developed a TMA due to CD and reached end-stage renal disease. After 2 years on dialysis, the patient received a KTx from her father who was already the HSCT donor. Given the full chimerism, no immunosuppressive agent was prescribed except a short (2 days) course of steroids and eculizumab to prevent aHUS relapse. Nine months after the KTx, the patient is well with normal renal function, no immunosuppression and continues eculizumab prevention of aHUS (1 infusion every 21 days).

Conclusions: All patients with transplant-associated thrombotic microangiopathy should be screened for the causes of CD. C5 inhibition with eculizumab is an important therapeutic resource to manage this complication. When KTx is necessary, immunosuppression can be safely withhold in case of same donor for both grafts and documented full chimerism.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1097/TP.0000000000002505DOI Listing
February 2019

A simple prognostic index for Shigatoxin-related hemolytic uremic syndrome at onset: data from the ItalKid-HUS network.

Eur J Pediatr 2018 Nov 10;177(11):1667-1674. Epub 2018 Aug 10.

Center for HUS Prevention Control and Management at the Epidemiology Unit, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Milano, Italy.

Shigatoxin Escherichia coli-related hemolytic uremic syndrome (eHUS) is a severe thrombotic microangiopathy (TMA) burdened by life-threatening complications and long-term sequelae. Since hemoconcentration is associated with worse outcome, we tried to develop a reliable and easy-to-calculate index for predicting complications and sequelae based on hemoglobin (Hb) at presentation. The first laboratory examinations with signs of TMA in eHUS patients were analyzed in relation to the outcomes with the receiver operating characteristic curves and their areas under the curve (AUC) for Hb and creatinine (sCr). A total of 197 eHUS patients were identified of whom 24% did not have anemia at presentation. Hb level was the best predictor of a poor outcome (AUC 0.67) but the combination of Hb with sCr, in the formula [(Hb in g/dL + (sCr in mg/dL × 2)], showed an even better AUC of 0.75. The described scoring system was also strongly associated and predictive of all complications and health care needs (8% of patients with scoring > 13 died or entered a permanent vegetative state compared with 0% of those with ≤ 13).Conclusion: The presented score is a simple and early predictor of both short- and long-term outcomes and identifies patients who should undergo rapid volume expansion to counteract hemoconcentration, the spreading of microvascular thrombosis, and the consequent increased organ damage. What is Known: • In eHUS, hemoconcentration is associated with worse short- and long-term outcome. • A prognostic index to identify patients at higher risk for complications at presentation is not available. What is New: • We developed a simple and early prognostic index for eHUS outcome with the combination of Hb and sCr at onset, in the following formula [(Hb in g/dL + (sCr in mg/dL × 2)]. • The proposed HUS Severity Score can promptly identify patients with good outcome and those with high risk of worse short- and long-term outcome.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00431-018-3198-7DOI Listing
November 2018

Serum creatinine during physiological perinatal dehydration may estimate individual nephron endowment.

Eur J Pediatr 2018 Sep 1;177(9):1383-1388. Epub 2018 Feb 1.

Neonatal Intensive Care Unit, Fondazione IRCCS Cà Granda, Ospedale Maggiore Policlinico, University of Milan, via Commenda 12, 20122, Milan, Italy.

It is well known that the nephron endowment of healthy subjects is highly variable and that individual nephron mass has potentially important implications both in health and disease. However, nephron count is technically impossible in living subjects. Based on the observation of an increase in serum creatinine (sCr) in otherwise healthy newborns with solitary kidney during the physiological perinatal dehydration, we hypothesized that perinatal sCr might be helpful in identifying healthy subjects with a reduced nephron mass. In the framework of a study on blood pressure in babies (NeoNeph), sCr of normal Caucasian neonates was determined 48-96 h after birth and their association with a family history of arterial hypertension (AH) was analyzed. SCr was determined in 182 normal newborns (90 males) at a mean of 61 ± 8 h after birth (range 46-82). Newborns with paternal AH had a higher mean sCr (0.97 + 0.28 mg/dL) then newborns without paternal AH (0.73 + 0.28 mg/dL; p = 0.006). No differences in mean sCr were found in relation with mother or grandparent's history of AH.

Conclusion: The association between parental AH and high sCr during perinatal dehydration supports the hypothesis that the latter is a promising tool for identifying normal subjects with a reduced nephron mass with potential important implications in prevention and in understanding the individual outcome of renal and extrarenal diseases (including AH). What is Known: • Nephron endowment of healthy subjects is highly variable and individual nephron mass has potentially important implications both in health and disease however nephron count is not feasible in living subjects. What is New: • Serum creatinine during perinatal dehydration is a possible biomarker for identifying normal subjects with a reduced nephron mass.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00431-018-3087-0DOI Listing
September 2018

Complement functional tests for monitoring eculizumab treatment in patients with atypical hemolytic uremic syndrome: an update.

Pediatr Nephrol 2018 03 18;33(3):457-461. Epub 2017 Oct 18.

Center for HUS Prevention, Control, and Management at Medicina Interna, Dipartimento di Fisiopatologia Medico-Chirurgica e dei Trapianti, Università degli Studi di Milano, Milan, Italy.

Background: Atypical hemolytic uremic syndrome (aHUS) is a thrombotic microangiopathy (TMA) characterized by platelet consumption, hemolysis, and organ damage. Eculizumab (ECU), a humanized antibody that blocks complement activity, has been successfully used in aHUS, but the best treatment schedule is not yet clear.

Methods: Here, we report our experience with ECU maintenance treatment and the interval between subsequent doses being extended based on global classical complement pathway (CCP) activity aimed at <30% for maintaining aHUS into remission.

Results: We report on 38 patients with aHUS, 13 children, 21 female, with a median age of 25.0 years (range 0.5-60) at disease onset treated with ECU standard schedule for a median of 2.6 months (range 0.4-24.6). Once stable TMA remission was obtained, the interval between ECU doses was extended based on complement function, with a target CCP activity of <30%. With this approach, 22 patients regularly receive ECU infusion every 28 days and 16 every 21. During a median observation period on ECU, an extended interval of 26.9 months (range 0.8-80.9), with a cumulative observation period of 1,208 months, none of the patients relapsed.

Conclusion: Monitoring complement activity allows a safe reduction in the frequency of ECU administration in aHUS while keeping the disease in remission.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00467-017-3813-2DOI Listing
March 2018

Late Onset Cobalamin Disorder and Hemolytic Uremic Syndrome: A Rare Cause of Nephrotic Syndrome.

Case Rep Pediatr 2017 1;2017:2794060. Epub 2017 Aug 1.

Pediatric Unit, Fondazione IRCCS Ca' Granda Osp. Maggiore Policlinico, Milan, Italy.

Hemolytic uremic syndrome (HUS) is an unrare and severe thrombotic microangiopathy (TMA) caused by several pathogenetic mechanisms among which Shiga toxin-producing infections and complement dysregulation are the most common. However, very rarely and particularly in neonates and infants, disorders of cobalamin metabolism (CblC) can present with or be complicated by TMA. Herein we describe a case of atypical HUS (aHUS) related to CblC disease which first presented in a previously healthy boy at age of 13.6 years. The clinical picture was initially dominated by nephrotic range proteinuria and severe hypertension followed by renal failure. The specific treatment with high dose of hydroxycobalamin rapidly obtained the remission of TMA and the complete recovery of renal function. We conclude that plasma homocysteine and methionine determinations together with urine organic acid analysis should be included in the diagnostic work-up of any patient with TMA and/or nephrotic syndrome regardless of age.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1155/2017/2794060DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5557262PMC
August 2017

Comment to "Blood urea nitrogen to serum creatinine ratio is an accurate predictor of outcome in diarrhea-associated hemolytic uremic syndrome" by Keenswijk et al. Eur J Pediatr 2017; 176(3): 355-360.

Eur J Pediatr 2018 02 19;177(2):269-270. Epub 2017 Aug 19.

Center for HUS Prevention, Control and Management, Epidemiology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00431-017-2989-6DOI Listing
February 2018

Bioimpedance-based rather than weight-based ultrafiltration prescription for children on maintenance hemodialysis
.

Clin Nephrol 2018 Oct;90(4):286-290

The determination of dry weight (DW) in young children on hemodialysis (HD) remains challenging. Bioimpedance analysis (BIA) is a potentially helpful means of estimating the need for ultrafiltration and monitoring body fluids in patients on renal replacement therapy, but its role has not yet been clearly defined. The aim of this paper is to share our experience of prescribing ultrafiltration on the basis of BIA parameters alone. The body weight (BW), resistance (Rx), and reactance (Xc) of a 3-year-old girl on chronic HD were recorded pre- and post-HD over a period of 16 months. The BIA parameter that best correlated with actual ultrafiltration (the difference between pre- and post-HD BW) was identified, and the equivalence between actual ultrafiltration and changes in Xc was derived to obtain the following equation: 1 ohm of Xc = 27.4 g of ultrafiltration. Finally, during 21 consecutive HD sessions, ultrafiltration was exclusively prescribed on the basis of the derived equation (BIA-based prescription) after having defined a target post-HD Xc of 45 ohm. The BIA-based prescription period was compared with 21 consecutive HD sessions in which ultrafiltration was prescribed using the conventional approach based on BW (BW-based prescription). Comparison of BIA-based and BW-based ultrafiltration prescription showed significantly fewer HD sessions complicated by hypotension (19 vs. 50%) or by the need for reinfusion (5 vs. 50%), and a better overall quality of HD sessions (86 vs. 37%). No difference in blood pressure was observed, and no acute fluid overload event was detected in either period. BIA-based ultrafiltration seems to be safe, feasible, and effective. The described approach can be particularly useful in the case of patients with problems in setting or maintaining the correct DW.
.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.5414/CN109109DOI Listing
October 2018

Acquired Complement Regulatory Gene Mutations and Hematopoietic Stem Cell Transplant-Related Thrombotic Microangiopathy.

Biol Blood Marrow Transplant 2017 Sep 15;23(9):1580-1582. Epub 2017 May 15.

Laboratory of Medical Genetics, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milano, Italy.

Hematopoietic stem cell transplant-related thrombotic microangiopathy (HSCT-TMA) is a severe complication whose pathophysiology is unknown. We describe 6 patients in which the disease was associated with complement regulatory gene abnormalities received from their respective donors. It is suggested that mutated and transplanted monocyte-derived cells are responsible for production of abnormal proteins, complement dysregulation, and, ultimately, for the disease. This observation might have important drawbacks as far as HSCT-TMA pathophysiology and treatment are concerned.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.bbmt.2017.05.013DOI Listing
September 2017

Early Volume Expansion and Outcomes of Hemolytic Uremic Syndrome.

Pediatrics 2016 Jan 7;137(1). Epub 2015 Dec 7.

Unit of Microbiology, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy;

Background: Hemolytic uremic syndrome associated with Shiga toxin-producing Escherichia coli (STEC-HUS) is a severe acute illness without specific treatment except supportive care; fluid management is concentrated on preventing fluid overload for patients, who are often oligoanuric. Hemoconcentration at onset is associated with more severe disease, but the benefits of volume expansion after hemolytic uremic syndrome (HUS) onset have not been explored.

Methods: All the children with STEC-HUS referred to our center between 2012 and 2014 received intravenous infusion targeted at inducing an early volume expansion (+10% of working weight) to restore circulating volume and reduce ischemic or hypoxic tissue damage. The short- and long-term outcomes of these patients were compared with those of 38 historical patients referred to our center during the years immediately before, when fluid intake was routinely restricted.

Results: Patients undergoing fluid infusion soon after diagnosis showed a mean increase in body weight of 12.5% (vs 0%), had significantly better short-term outcomes with a lower rate of central nervous system involvement (7.9% vs 23.7%, P = .06), had less need for renal replacement therapy (26.3% vs 57.9%, P = .01) or intensive care support (2.0 vs. 8.5 days, P = .02), and needed fewer days of hospitalization (9.0 vs 12.0 days, P = .03). Long-term outcomes were also significantly better in terms of renal and extrarenal sequelae (13.2% vs 39.5%, P = .01).

Conclusions: Patients with STEC-HUS had great benefit from early volume expansion. It is speculated that early and generous fluid infusions can reduce thrombus formation and ischemic organ damage, thus having positive effects on both short- and long-term disease outcomes.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1542/peds.2015-2153DOI Listing
January 2016

Epidemiology of haemolytic uremic syndrome in children. Data from the North Italian HUS network.

Eur J Pediatr 2016 Apr 24;175(4):465-73. Epub 2015 Oct 24.

Center for Prevention, Control and Management of Hemolytic Uremic Syndrome, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milano, Italy.

Unlabelled: Despite the severity of HUS and the fact that it represents a leading cause of acute kidney injury in children, the general epidemiology of HUS is all but well documented. The present study provides updated, population-based, purely epidemiological information on HUS in childhood from a large and densely populated area of northern Italy (9.6 million inhabitants, 1.6 million children). We systematically reviewed the files concerning patients with STEC-HUS and atypical HUS (aHUS) over a 10-year observation period (January 2003-December 2012). We included all incident cases with a documented first episode of HUS before the age of 18 years. We identified 101 cases of HUS during the 10 years. The overall mean annual incidence was 6.3 cases/million children aged <18 years (range 1.9-11.9), and 15.7/million of age-related population (MARP) among subjects aged <5 years; aHUS accounted for 11.9 % of the cases (mean incidence 0.75/MARP). The overall case fatality rate was 4.0 % (3.4 % STEC-HUS, 8.3 % aHUS).

Conclusion: Given the public health impact of HUS, this study provides recent, population-based epidemiological data useful for healthcare planning and particularly for estimating the financial burden that healthcare providers might have to face in treating HUS, whose incidence rate seems to increase in Northern Italy.

What Is Known: • HUS is a rare disease, but it represents the leading cause of acute kidney injury in children worldwide. • STEC-HUS (also called typical, D + HUS) is more common compared to atypical HUS, but recent, population-based epidemiological data (incidence) are scanty. What is New: • Comprehensive, population-based epidemiological data concerning both typical and atypical HUS based on a long observational period.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00431-015-2642-1DOI Listing
April 2016

Discontinuation of eculizumab treatment in atypical hemolytic uremic syndrome: an update.

Am J Kidney Dis 2015 Jul;66(1):172-3

Center for HUS Control, Prevention and Management, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1053/j.ajkd.2015.04.010DOI Listing
July 2015

In reply to 'discontinuation of eculizumab maintenance treatment for atypical hemolytic uremic syndrome'.

Am J Kidney Dis 2015 Feb;65(2):342-3

Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1053/j.ajkd.2014.05.028DOI Listing
February 2015

Hemoconcentration: a major risk factor for neurological involvement in hemolytic uremic syndrome.

Pediatr Nephrol 2015 Feb 23;30(2):345-52. Epub 2014 Aug 23.

Center for HUS Control, Prevention and Management, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Via Commenda 9, 20122, Milan, Italy,

Background: Shigatoxin-associated hemolytic uremic syndrome (STEC-HUS) is a common thrombotic microangiopathy (TMA) in which central nervous system (CNS) involvement is responsible for the majority of deaths and for severe long-term sequelae. We have analyzed the role of hemoconcentration in disease severity.

Methods: This was a retrospective review of the records and laboratory data at presentation of all patients with STEC-HUS cases (n = 61) over a 10-year period. The patients were grouped into three severity classes: group A, comprising patients who did not require dialysis; group B, patients who were dialyzed without CNS involvement; group C, patients with CNS involvement.

Results: Patients with CNS involvement (group C) had a higher mean hemoglobin level (11.2 ± 2.3 g/dL) than those of group A or B ( 9.4 ± 2.1 and 7.5 ± 1.9 g/dL, respectively; p < 0.0001). We also observed that the higher the initial hemoglobin level, the more severe the long-term renal damage (p < 0.007).

Conclusions: In patients with STEC-HUS, hemoconcentration and hypovolemia may be responsible for more severe ischemic organ damage (both short and long term) at disease onset, and these signs should be regarded as risk factors for CNS damage and for more severe TMA. Therefore, we recommend that hydration status should be actively monitored in HUS patients and that dehydration, when diagnosed, should be promptly corrected.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00467-014-2918-0DOI Listing
February 2015

Cryptic activity of atypical hemolytic uremic syndrome and eculizumab treatment.

Pediatrics 2014 Jun 19;133(6):e1769-71. Epub 2014 May 19.

Center for Hemolitic Uremic Syndrome Control, Prevention and Management, and

Atypical hemolytic uremic syndrome (aHUS) is a rare, life-threatening disease often related to uncontrolled complement activation. The use of eculizumab has changed the management and the outcome of aHUS, becoming the frontline treatment of the acute disease and for the prevention of relapses. We report the case of a male patient with aHUS due to complement factor H gene mutation who was shifted from plasmatherapy to eculizumab for preventing disease relapses. The shift to eculizumab was associated with a significant decrease in proteinuria, revealing disease activity otherwise unsuspected, being the classic criteria of disease activity (platelet, haptoglobin, LDH, schistocytes), all in the normal range.The condition of proteinuria as the only sign of thrombotic microangiopathy activity is here designated as "cryptic activity of aHUS."
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1542/peds.2013-2921DOI Listing
June 2014

Co-infection in children with bloody diarrhea caused by Shiga toxin-producing Escherichia coli: data of the North Italian HUS Network.

J Pediatr Gastroenterol Nutr 2014 Aug;59(2):218-20

*Center for HUS Prevention, Control and Management †Unit of Microbiology, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Milan, Italy.

Hemolytic-uremic syndrome (HUS) is an important cause of acute kidney injury in children often caused by Shiga toxin-producing Escherichia coli (STEC) enterocolitis. In a screening program for STEC infection in children with bloody diarrhea in northern Italy for early diagnosis of HUS, co-infection with Salmonella or Campylobacter was documented in as many as 35.6% of Shiga toxin-positive patients. It is speculated that infection by Salmonella or Campylobacter may increase the risk of STEC enterocolitis and therefore of HUS. The isolation of microorganisms (other then STEC) in HUS should not be necessarily regarded as the etiological agent for the thrombotic microangiopathy.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1097/MPG.0000000000000420DOI Listing
August 2014

Discontinuation of eculizumab maintenance treatment for atypical hemolytic uremic syndrome: a report of 10 cases.

Am J Kidney Dis 2014 Oct 19;64(4):633-7. Epub 2014 Mar 19.

Center for HUS Prevention, Control and Management, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Milan, Italy.

Atypical hemolytic uremic syndrome (aHUS) is a life-threatening thrombotic microangiopathy, and as many as 70% of patients with aHUS have mutations in the genes encoding complement regulatory proteins. Eculizumab, a humanized recombinant monoclonal antibody targeting C5, has been used successfully in patients with aHUS since 2009. The standard maintenance treatment requires life-long eculizumab therapy, but the possibility of discontinuation has not yet been tested systematically. We report the safety of discontinuing eculizumab treatment in 10 patients who stopped treatment with the aim of minimizing the risk of adverse reactions, reducing the risk of meningitis, and improving quality of life while also reducing the considerable treatment costs. Disease activity was monitored closely at home by means of urine dipstick testing for hemoglobin. During the cumulative observation period of 95 months, 3 of the 10 patients experienced relapse within 6 weeks of discontinuation, but then immediately resumed treatment and completely recovered. Our experience supports the possibility of discontinuing eculizumab therapy with strict home monitoring for early signs of relapse in patients with aHUS who achieve stable remission.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1053/j.ajkd.2014.01.434DOI Listing
October 2014

Febrile urinary tract infections: clinical and laboratory diagnosis, imaging, and prognosis.

Semin Nucl Med 2014 Mar;44(2):123-8

Nuclear Medicine Department, Ospedale di Circolo, Varese, Italy.

According to the literature, febrile urinary tract infections (UTIs) are among the most common severe bacterial infections occurring in childhood, with potential serious long-term consequences. In recent years, there have been significant developments in our understanding of the pathophysiology and clinical and laboratory issues of febrile UTIs. Studies are focusing on the role of predisposing host factors related to genes regulating immune response, inflammation and fibrosis in the development of acute renal damage and subsequent processes leading to renal scars. All the available guidelines underline the importance of a correct diagnosis of febrile UTI to allow a more rational use of antibiotics and imaging. As a consequence, a shift from aggressive imaging studies to a more restrictive and targeted approach has been recently observed. Regarding the prognosis of febrile UTI, the introduction of prenatal ultrasound studies revealed that a great portion of the alterations at imaging (and thus of the clinical complications), previously attributed to postinfection scarring, were because of congenital kidney and urinary tract abnormalities. Although the long-term consequences of febrile UTIs are difficult to ascertain, it seems that children with febrile UTI, normal renal function and normal kidneys at start present a very low risk of developing decreased renal function or hypertension during follow-up. However, high body temperature and high procalcitonin levels during the acute phase of disease, which are indicative of severe inflammation, and the finding of renal scarring on imaging with DMSA scintigraphy 6 months after febrile UTI, together with the detection of congenital kidney and urinary tract abnormalities, indicate "kidney at risk" in UTI.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1053/j.semnuclmed.2013.10.004DOI Listing
March 2014

Time to change the definition of hemolytic uremic syndrome.

Eur J Intern Med 2014 Feb 21;25(2):e29. Epub 2013 Dec 21.

Center for HUS Prevention, Control and Management, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, via della Commenda 9, 20122 Milan, Italy.

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.ejim.2013.12.002DOI Listing
February 2014

Skin involvement in atypical hemolytic uremic syndrome.

Am J Kidney Dis 2014 Apr 27;63(4):652-5. Epub 2013 Nov 27.

Center for HUS Prevention, Control and Management, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Milan, Italy.

Skin involvement in atypical hemolytic uremic syndrome (aHUS) is very uncommon and therefore often unrecognized as a specific symptom of aHUS. We describe 3 cases of patients with aHUS who developed skin lesions that completely recovered when disease-specific treatment was established. These cases suggest that in individuals with aHUS, when skin lesions of unknown origin occur, the possibility that they are due to thrombotic microangiopathy should be considered.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1053/j.ajkd.2013.09.020DOI Listing
April 2014

Pandemic influenza A/H1N1 vaccine administered sequentially or simultaneously with seasonal influenza vaccine to HIV-infected children and adolescents.

Vaccine 2011 Feb 1;29(8):1677-82. Epub 2011 Jan 1.

Department of Maternal and Pediatric Sciences, Università degli Studi di Milano, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Via Commenda 9, 20122 Milan, Italy.

In order to evaluate the immunogenicity, safety and tolerability of the 2009 A/H1N1 MF59-adjuvanted influenza vaccine administered sequentially or simultaneously with seasonal virosomal-adjuvanted influenza vaccine to HIV-infected children and adolescents, 36 HIV-infected children and adolescents, and 36 age- and gender-matched healthy controls were randomised 1:1 to receive the pandemic vaccine upon enrollment and the seasonal vaccine one month later, or to receive the pandemic and seasonal vaccines simultaneously upon enrollment. Seroconversion and seroprotection rates against the pandemic influenza A/H1N1 virus were 100% two months after vaccine administration in both groups, regardless of the sequence of administration. Geometric mean titres against pandemic and seasonal antigens were significantly higher when the seasonal and pandemic vaccines were administered simultaneously than when the seasonal vaccine was administered alone. Local and systemic reactions were mild and not increased by simultaneous administration. In conclusion, the 2009 pandemic influenza A/H1N1 MF59-adjuvanted vaccine is as immunogenic, safe and well tolerated in HIV-infected children and adolescents as in healthy controls. Its simultaneous administration with virosomal-adjuvanted seasonal antigens seems to increase immune response to both pandemic and seasonal viruses with the same safety profile as that of the pandemic vaccine alone. However, because this finding cannot be clearly explained by an immunological viewpoint, further studies are needed to clarify the reasons of its occurrence.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.vaccine.2010.12.047DOI Listing
February 2011

Factors conditioning effectiveness of a reminder/recall system to improve influenza vaccination in asthmatic children.

Vaccine 2009 Jan 3;27(5):633-5. Epub 2008 Dec 3.

Institute of Pediatrics, University of Milan, Fondazione IRCCS Ospedale Maggiore Policlinico, Mangiagalli e Regine Elena, Milan, Italy.

In order to verify whether a telephone recall system directly managed by pediatricians who usually follow up children for their asthma is more effective than an anonymous recall system, we randomly assigned 285 asthmatic children (177 males; mean age 10.3+/-3.4 years) to one of three groups: those whose mothers were to be called by a pediatrician not previously involved in caring for their asthmatic children and who received the vaccine in our immunisation clinic (group 1); those whose mothers were to be called by a pediatrician from our asthma clinic and who received the vaccine in the immunisation clinic (group 2); and those whose mothers were to be called by a pediatrician from our asthma clinic and who received the vaccine in the same clinic (group 3). Our findings highlight that the use of a reminder/recall system increases vaccination rates in asthmatic children, and show that the best results are obtained when the mothers are contacted and the vaccine administered by the pediatricians who usually follow up the child for asthma.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.vaccine.2008.11.043DOI Listing
January 2009

Pediatrician knowledge and attitudes regarding human papillomavirus disease and its prevention.

Vaccine 2007 Aug 16;25(35):6437-46. Epub 2007 Jul 16.

Institute of Pediatrics, University of Milan, Fondazione IRCCS "Ospedale Maggiore Policlinico, Mangiagalli e Regina Elena", Via Commenda 9, 20122 Milan, Italy.

Between 1 September and 8 October 2006, we used a self-administered, anonymous questionnaire in order to evaluate knowledge and attitudes regarding HPV disease and its prevention in a nationally representative sample of Italian pediatricians. Three hundred and eleven of the 400 enrolled physicians (175 primary care pediatricians, 160 hospital pediatricians and 65 residents in pediatrics) returned completed surveys (227 females; mean age+/-standard deviation, 45.3+/-11.61 years). The results showed a lack of knowledge concerning HPV disease and its prevention, with marginal differences between the subgroups, even though the majority would recommend HPV vaccination. These findings highlight an urgent need to improve pediatrician information in order to ensure the optimal implementation of HPV vaccination, and the adequate acceptability of HPV vaccines to adolescents and their parents.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.vaccine.2007.06.053DOI Listing
August 2007