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ABSTRACT: Methylmalonic acidemia (MMA) is an inborn error of organic acid metabolism. Patients with severe disease develop many complications
despite treatment; often, the disease progresses to severe damage of the central nervous system or to end-stage renal disease
(ESRD). When medical treatment is ineffective, liver, kidney, or combined liver and kidney transplantation is advocated. At
present, there are no definite guidelines as for the organ to be transplanted, and results are inconsistent. We report on
a 27-year-old woman with MMA MUT0. The clinical symptoms developed at age 4months. She progressed to ESRD and received a kidney transplant in November 1996
at age 17years. One hundred and twenty months after transplant, renal function is normal; although urinary levels of methylmalonic
acid are above normal limits, no episodes of metabolic decompensation have been observed after transplantation. Although liver
is the major site of methylmalonyl-CoA mutase activity, this case and similar ones in the literature suggest that the smaller
mutase activity present in the transplanted kidney may be sufficient to ensure partial correction of the metabolism of organic
acids sufficient to prevent the onset of episodes of metabolic decompensation. It is worth investigating whether kidney transplant
can be a safer and more satisfactory alternative to liver transplantation in cases of MMA unresponsive to medical treatment
although urine MMA excretion remains significantly elevated.
Pediatric Nephrology 04/2012; 22(8):1209-1214. · 2.52 Impact Factor
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The American journal of emergency medicine 02/2012; 30(2):371-2. · 1.54 Impact Factor
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European journal of emergency medicine: official journal of the European Society for Emergency Medicine 09/2011; 18(6):364-5. · 0.73 Impact Factor
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ABSTRACT: Cardiorespiratory fitness is significantly reduced in children with end-stage renal disease. The role of renal transplantation in improving cardiorespiratory fitness has not been thoroughly investigated.
In this work, we wanted to assess whether, in children after a successful renal transplant, the amount of weekly physical exercise affects cardiorespiratory fitness and left ventricular mass (LVM). The study was conducted on 16 children after renal transplantation and 36 matching healthy controls. Four groups were formed according to the weekly amount of physical exercise; all children received an echocardiogram and underwent a treadmill exercise test according to the Bruce protocol.
Cardiorespiratory fitness is worst in renal transplant children with a weekly physical exercise<3 h; renal transplant children with a physical exercise of 3-5 h per week attain a cardiorespiratory fitness comparable to controls with a sedentary lifestyle (<3-h exercise per week); healthy controls with a weekly physical exercise of 3-5 h per week show the highest levels of cardiorespiratory fitness; the LVM assessed via echocardiography follows the same pattern.
In children with a successful renal transplant, a weekly physical exercise of 3-5 h significantly improves the cardiorespiratory fitness and the LVM, up to the level of matching healthy controls with a sedentary lifestyle (<3 h exercise per week).
Nephrology Dialysis Transplantation 08/2011; 27(4):1677-81. · 3.40 Impact Factor
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Riccardo Lubrano,
Corrado Cecchetti, Marco Elli,
Caterina Tomasello,
Giuliana Guido,
Matteo Di Nardo,
Raffaele Masciangelo,
Elisabetta Pasotti,
Maria Antonietta Barbieri,
Elena Bellelli,
Nicola Pirozzi
[show abstract]
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ABSTRACT: In critically ill adults, a reduction in the extravascular lung water index (EVLWi) decreases time on mechanical ventilation and improves survival. The purpose of this study is to assess the prognostic value of EVLWi in critically ill children with acute respiratory failure and investigate its relationships with PaO(2), PaO(2)/FiO(2) ratio, A-aDO(2), oxygenation index (OI), mean airway pressure, cardiac index, pulmonary permeability, and percent fluid overload.
Twenty-seven children admitted to PICU with acute respiratory failure received volumetric hemodynamic and blood gas monitoring following initial stabilization and every 4 h thereafter, until discharge from PICU or death. All patients are grouped in two categories: nonsurvivors and survivors.
Children with a fatal outcome had higher values of EVLWi on admission to PICU, as well as higher A-aDO(2) and OI, and lower PaO(2) and PaO(2)/FIO(2) ratio. After 24 h EVLWi decreased significantly only in survivors. As a survival indicator, EVLWi has good sensitivity and good specificity. Changes in EVLWi, OI, and mean airway pressure had a time-dependent influence on survival that proved significant according to the Cox test. Survivors spent fewer hours on mechanical ventilation. We detected a correlation of EVLWi with percent fluid overload and pulmonary permeability.
Like OI and mean airway pressure, EVLWi on admission to PICU is predictive of survival and of time needed on mechanical ventilation.
European Journal of Intensive Care Medicine 09/2010; 37(1):124-31. · 5.17 Impact Factor
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ABSTRACT: We report on a paediatric case of autosomal dominant polycystic kidney disease, where myocardial hypertrophy proved a consequence of noncompaction of the ventricular myocardium. Deletion of PKD1 and PKD2, the genes responsible for polycystic renal disease, has been linked also to disorganized myocardial arrangement in experimental animals. Two adults with polycystic kidney disease and myocardial hypertrophy in whom a careful diagnostic workup led to a diagnosis of non-compaction of the ventricular myocardium have been reported in the literature. Nephrologists must be aware of the possible association between the two diseases because early recognition of the disease may help in preventing the onset of complications.
Nephrology Dialysis Transplantation 09/2009; 24(12):3884-6. · 3.40 Impact Factor
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ABSTRACT: It is as yet unclear whether blood pressure load (BPL) can affect renal function in pre-hypertensive children. We have studied 250 children, with a mean age of 9.12 +/- 3.28 years, with the aim of assessing if pre-hypertension in children can indeed affect renal function. The study cohort consisted of 146 children with pre-hypertension (group P) and a control group of 104 children with normal blood pressure (group C). All children were tested for orthostatic proteinuria, an exclusion criterion, glomerular filtration rate (GFR), and proteinuria, and ambulatory blood pressure monitoring was performed. Based on the BPL, group P was further subdivided into group P1 (BPL <or= 40%, low BPL) and group P2 (BPL > 40%, high BPL). We found that GFR was reduced in pre-hypertensive children (90.74 +/- 48.69 vs. 110.32 +/- 20.30 ml/min per 1.73 m(2), p < 0.0001) and that proteinuria was increased (145.36 +/- 110.91 vs. 66.84 +/- 42.94 mg/m(2) per 24 h; p < 0.0001). However, mean values were still within normal limits. A comparison of the group with high BPL and that with low BPL revealed that the former had relatively reduced GFR (79.15 +/- 42.04 vs. 96.78 +/- 51.20 ml/min per 1.73 m(2); p < 0.006) and increased proteinuria (198.29 +/- 142.17 vs. 118.31 +/- 80.07 mg/m(2) per 24 h; p < 0.036). In comparison to the reference values of the normal population, the GFR was reduced and proteinuria was increased in the group with high BPL. Based on our results, pre-hypertension in children with high BPL seems to be associated with reduced GFR and increased proteinuria. A reasonable doubt remains that the patients with higher proteinuria and larger reduction of GFR may harbor an as yet unknown subclinical renal condition responsible for the onset of pre-hypertension. Therefore, children with even mildly elevated BP are at risk of developing renal damage and should change their lifestyle to prevent further increases in BP.
Pediatric Nephrology 12/2008; 24(4):823-31. · 2.52 Impact Factor
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ABSTRACT: To determine the effect of alpha-tocopherol in patients receiving hypotensive anesthesia with propofol-remifentanil.
Prospective, randomized study.
University hospital.
66 ASA physical status I and II patients, aged 32 to 56 years, scheduled for nasal polypectomy.
Patients were allocated into two groups, the treatment and the control groups (T group and C group). T group received alpha-tocopherol 300 mg orally 5 to 6 hours before surgery.
Sampling times and measurements were done before hypotension (t0), 45 minutes after starting hypotension (t1), 90 minutes after starting hypotension (t2), 45 minutes after recovery of normotension (t3), and 24 hours after surgery (t4). Renal function was assessed by testing glomerular and tubular functions: glomerular filtration rate, fractional excretion of sodium (FENA); fractional excretion of urea (FEUN); and urinary N-acetyl-1-beta-D-glucosoaminidase (NAG) index (NAGi).
Glomerular filtration rate values remained unchanged in all patient populations. Fractional excretion of sodium was within reference ranges in both groups at times t0, t1, and t2. At time t3, a significant FE(NA) peak was observed. At this time, FENA was significantly higher in C group than T group (P < 0.001). FEUN time course was similar to the FENA trend. At time t4, FENA and FEUN returned to basal values. At time t3, NAGi was also increased without significant intergroup differences (P < 0.01, P < 0.001, and P < 0.01 vs times t0, t1, t2 in C group, respectively; P < 0.01, P < 0.01, and P < 0.001 vs times t0, t1, and t2 in T group, respectively).
In patients without any renal disease, hypotensive anesthesia with propofol and remifentanil results in a transient tubular dysfunction, which appears to be minimized by the preoperative administration of alpha-tocopherol.
Journal of Clinical Anesthesia 05/2008; 20(3):164-9. · 1.21 Impact Factor
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Corrado Cecchetti,
Riccardo Lubrano,
Sebastian Cristaldi,
Francesca Stoppa,
Maria Antonietta Barbieri, Marco Elli,
Raffaele Masciangelo,
Daniela Perrotta,
Elisabetta Travasso,
Claudia Raggi,
Marco Marano,
Nicola Pirozzi
[show abstract]
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ABSTRACT: The objective of this study was to investigate possible correlations between the preload index global end-diastolic volume (GEDV) and the indexes of cardiac function, cardiac index, and stroke volume index in critically ill pediatric patients. The aim was to evaluate whether GEDV may help in the decision-making process concerning volume loading.
Prospective clinical study.
Pediatric intensive care unit of the Bambino Gesù Children's Research Hospital.
Seventy patients, 40 male and 30 female, mean age 62 +/- 41 months (range 5-156 months), divided into six groups: group A, hemorrhagic shock, ten cases; group B, head injury, 21 cases; group C, septic shock, ten cases; group D, encephalitis, ten cases; group E, respiratory failure, nine cases; group F, cardiogenic shock, ten cases.
All patients received volumetric hemodynamic monitoring following initial resuscitation and every 4 hrs thereafter or whenever a hemodynamic deterioration was suspected. During the cumulative in-hospital stay, a total 1,184 sets of measurements were done.
Findings are consistent with a statistically significant linear correlation of GEDV with cardiac index and stroke volume index in hemorrhagic shock (group A) (R2 = .647, p < .0001; R2 = .738, p < .0001) and cardiogenic shock (group F) (R2 = .645, p < .0001; R2 = .841, p < .0001).
GEDV may potentially be a useful guide to treatment in preload-dependent conditions, such as hemorrhagic and cardiogenic shock. In the other groups where there is little relationship between preload and cardiac function indexes, the influence of non-preload-dependent mechanisms on cardiac output is certainly more significant.
Critical care medicine 03/2008; 36(3):928-32. · 6.37 Impact Factor
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Corrado Cecchetti,
Riccardo Lubrano,
Sebastian Cristaldi,
Francesca Stoppa,
Maria Antonietta Barbieri, Marco Elli,
Raffaele Masciangelo,
Daniela Perrotta,
Elisabetta Travasso,
Claudia Raggi,
Marco Marano,
Nicola Pirozzi
[show abstract]
[hide abstract]
ABSTRACT: Objective: The objective of this study was to investigate possible correlations between the preload index global end-diastolic volume (GEDV) and the indexes of cardiac function, cardiac index, and stroke volume index in critically ill pediatric patients. The aim was to evaluate whether GEDV may help in the decision-making process concerning volume loading.
Design: Prospective clinical study.
Setting: Pediatric intensive care unit of the Bambino Gesù Children’s Research Hospital.
Patients: Seventy patients, 40 male and 30 female, mean age 62 ± 41 months (range 5–156 months), divided into six groups: group A, hemorrhagic shock, ten cases; group B, head injury, 21 cases; group C, septic shock, ten cases; group D, encephalitis, ten cases; group E, respiratory failure, nine cases; group F, cardiogenic shock, ten cases.
Interventions: All patients received volumetric hemodynamic monitoring following initial resuscitation and every 4 hrs thereafter or whenever a hemodynamic deterioration was suspected. During the cumulative in-hospital stay, a total 1,184 sets of measurements were done.
Measurements and Main Results: Findings are consistent with a statistically significant linear correlation of GEDV with cardiac index and stroke volume index in hemorrhagic shock (group A) (R2 = .647, p < .0001; R2 = .738, p < .0001) and cardiogenic shock (group F) (R2 = .645, p < .0001; R2 = .841, p < .0001).
Conclusions: GEDV may potentially be a useful guide to treatment in preload-dependent conditions, such as hemorrhagic and cardiogenic shock. In the other groups where there is little relationship between preload and cardiac function indexes, the influence of non-preload-dependent mechanisms on cardiac output is certainly more significant.
Critical Care Medicine 02/2008; 36(3):928-932. · 6.33 Impact Factor
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[show abstract]
[hide abstract]
ABSTRACT: Methylmalonic acidemia (MMA) is an inborn error of organic acid metabolism. Patients with severe disease develop many complications despite treatment; often, the disease progresses to severe damage of the central nervous system or to end-stage renal disease (ESRD). When medical treatment is ineffective, liver, kidney, or combined liver and kidney transplantation is advocated. At present, there are no definite guidelines as for the organ to be transplanted, and results are inconsistent. We report on a 27-year-old woman with MMA MUT0. The clinical symptoms developed at age 4 months. She progressed to ESRD and received a kidney transplant in November 1996 at age 17 years. One hundred and twenty months after transplant, renal function is normal; although urinary levels of methylmalonic acid are above normal limits, no episodes of metabolic decompensation have been observed after transplantation. Although liver is the major site of methylmalonyl-CoA mutase activity, this case and similar ones in the literature suggest that the smaller mutase activity present in the transplanted kidney may be sufficient to ensure partial correction of the metabolism of organic acids sufficient to prevent the onset of episodes of metabolic decompensation. It is worth investigating whether kidney transplant can be a safer and more satisfactory alternative to liver transplantation in cases of MMA unresponsive to medical treatment although urine MMA excretion remains significantly elevated.
Pediatric Nephrology 08/2007; 22(8):1209-14. · 2.52 Impact Factor
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Riccardo Lubrano,
Francesca Soscia, Marco Elli,
Flavia Ventriglia,
Claudia Raggi,
Elisabetta Travasso,
Simona Scateni,
Valeria Di Maio,
Paolo Versacci,
Raffaele Masciangelo,
Stefano Romero
[show abstract]
[hide abstract]
ABSTRACT: We investigated whether the combination of an angiotensin-converting enzyme inhibitor and an angiotensin II type 1 receptor antagonist offers better control of proteinuria and cardiovascular parameters without causing adverse side effects.
We enrolled 10 children (mean age: 12.3 +/- 4.06 years) with proteinuria resulting from chronic renal diseases of various causes. The study consisted of 2 phases, 3 months each, for an overall 6-month observation time. During phase 1 (3 months), each child was assigned randomly to treatment with either an angiotensin-converting enzyme inhibitor or an angiotensin II type 1 receptor antagonist alone. During phase 2, each child was advanced to combination therapy with the addition of an angiotensin II type 1 receptor antagonist or an angiotensin-converting enzyme inhibitor, respectively. Renal function tests, echocardiography, and 24-hour ambulatory blood pressure monitoring were performed at the beginning of the study (time 0), at 3 months (time 1), and at 6 months (time 2).
At time 2, proteinuria (change: -80.21 +/- 10.75%), interventricular septum index (change: -13.63 +/- 18.64%), posterior wall of the left ventricle index (change: -30.71 +/- 20.32%), and left ventricular mass index (change: -28.33 +/- 24.44%) were reduced significantly, compared with time 0 and time 1. No untoward side effects were detected during the study.
In the short term, the combination of angiotensin-converting enzyme inhibitors and angiotensin II type 1 receptor antagonists for children with proteinuria of renal origin reduced proteinuria significantly, compared with baseline or either drug alone. Furthermore, echocardiographic studies gave evidence of reduction of left ventricular hypertrophy. Additional studies are needed to evaluate long-term results.
PEDIATRICS 10/2006; 118(3):e833-8. · 4.47 Impact Factor
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Riccardo Lubrano,
Stefano Romero,
Pietro Scoppi,
Guido Cocchi,
Simonetta Baroncini, Marco Elli,
Manuela Turbacci,
Simona Scateni,
Elisabetta Travasso,
Rossella Benedetti,
Sebastian Cristaldi,
Rossella Moscatelli
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ABSTRACT: It has been calculated that, on average, 20% of the population should be trained to provide first aid, if a significant reduction of mortality is to be achieved. However, wide dissemination of the principles of emergency care poses a series of difficulties. As a partial solution, we have designed a first aid training course for children aged 8-11 years in their last three courses at primary school. According to the Italian school system, classes in primary school are indicated as I through V, from start to ending. The course addresses three issues: the broken tooth, nose bleeding and paediatric basic life support (PBLS). The course is divided into 17 didactic modules: each module contains a theoretical lecture, a practical demonstration by the trainer and a session for the trainees to practice under supervision. The aim of the study was to evaluate the benefit of teaching emergency procedures including practical sessions for pupils in primary schools. Four hundred and sixty-nine children were enrolled: the evaluation consisted of a 13 question multiple-choice written test taken at the end of the theoretical session and a semi-structured test at one month. Two hundred and seventy-one children attended to the theoretical lesson only, without going through the practical session (Group A), while the remaining 189 children completed the practical training (Group B). The outcome of the evaluation demonstrates that older children (in their V school class) score better than those in their IV and III class (p < 0.001). However, when comparing Group A and Group B in each class, the children that had also been exposed to the practical training (Group B) scored significantly better (V(B) versus V(A) p < 0.001; IV(B) versus IV(A) p < 0.001; III(B) versus III(A) p < 0.01). In conclusion, this proposed method of teaching emergency first aid could be successful in training primary school children. The permanent integration of the subject into the core curriculum of primary schools, and extended to higher school levels, could help in disseminating the culture of emergency care in the general population.
Resuscitation 04/2005; 64(3):303-7. · 3.60 Impact Factor
