Reversal of cardiomyopathy in propionic acidemia after liver transplantation: a 10‐year follow‐up

Cardiomyopathy is a frequent complication in propionic acidemia. It is mostly rapidly fatal and independent of the metabolic control or medical intervention. Here, we present the reversal of a severe cardiomyopathy after liver transplantation in a patient with propionic acidemia and the long‐term stability after ten years. Liver transplantation in patients with propionic acidemia may be considered a valid and long‐lasting treatment when cardiomyopathy is progressive and unresponsive to medical therapy.     

Natural history of vertically acquired HCV infection and associated autoimmune phenomena

The natural history of vertically acquired HCV infection is ill defined. The aim of this study was to outline the natural course of vertical HCV infection in a cohort of untreated children, including rate of spontaneous viral clearance, frequency and features of HCV-related autoimmune disorders. Children with vertical HCV infection were prospectively followed from the first month of life with regular clinical and laboratory assessments. Statistical analysis was performed using Prism 5.0. Forty-five children (median age 12 years, interquartile range 6.9–15.5) were studied. Genotype 1 was predominant (53.3 %). Spontaneous viral clearance was achieved by 12 patients (26.7 %) and associated with genotype 3. Alanine-amino-transferase levels were increased in most children in the first 2 years of life with higher values in those who later cleared the infection. All children were asymptomatic for liver disease. Transient elastography (32 patients) showed mild or moderate fibrosis in nine and two cases, respectively. Non-organ-specific autoantibodies were detected in 24 children (53.3 %) independently of viremia; of these, one developed type-1 diabetes. Cryoglobulinemia was associated with genotype 1 infection and found in 15 subjects (33.3 %): two had low C4 levels and persistent proteinuria. Conclusions: Vertically acquired HCV infection may result in spontaneous clearance in up to 27 % of children. Resolution of infection is higher with genotype 3, usually occurs in preschool age and persists over time. Chronic infection is generally asymptomatic, although hepatomegaly and mild fibrosis may develop. Autoantibodies and cryoglobulins are frequent, whereas the associated clinical manifestations are rare.     

Association between nocturnal blood pressure dipping and insulin resistance in children affected by NAFLD

The aim of this study was to analyse the relationship between insulin–glucose metabolism, nocturnal blood pressure dipping and nonalcoholic fatty liver disease (NAFLD) in obese adolescents without diabetes. One hundred one consecutive children, with biopsy-proven NAFLD, were included in this study. Blood samples were drawn for the analyses of liver function tests, insulin–glucose metabolism and lipid profile appraisal. An ambulatory blood pressure measurement (ABPM) was performed. Seventy-six children (75.3 %) were systolic nondippers, and 23 of them were diastolic nondippers (30.3 %). No differences were found in the anthropometric parameters between the two groups. When compared to the systolic dippers, the systolic nondippers had higher medians of mean nocturnal blood pressure, glucose at 0, 60 and 120 min in the oral glucose tolerance test (OGTT), OGTT insulin at all time points and insulin-resistance values. No correlation of histopathological features with dipping/nondipping statuses was found. Conclusions: We found an association between a nocturnal blood pressure fall and measures of insulin levels, independent of obesity, or daytime blood pressure levels, among the obese patients with NAFLD. Although no association between nondipping profiles and NAFLD was observed in our study, further studies with a longer term follow-up are needed, to better elucidate the complex link between these particular entities.     

Ventral stream sensitivity in “healthy” preterm-born adolescents: Psychophysical and neuropsychological evaluation

Background

Deficits of motion processing have been reported in premature and very low birth-weight subjects during infancy, childhood and adolescence. Less is known about ventral stream functioning in preterms.

Aim

The aim of this study is to investigate ventral stream functioning in a sample of “healthy” adolescents born preterm with normal outcome and without brain damage.

Study design

We enrolled thirty preterm-born adolescents (mean age: 14.2 years, mean gestational age 28.9 weeks, mean birth weight 1097 g), and 34 age-matched term-born controls (mean age: 14.5 years). All subjects were administered a psychophysical test known as “Form Coherence Task” and a comprehensive standardized battery of neuropsychological tests suitable for investigating ventral stream functioning including Street Completion Test, Poppelreuter–Ghent Test and the first part of the Visual Object and Space Perception (VOSP) battery. Dorsal stream visual functioning was investigated by the second part of the VOSP.

Results

Preterm (PT) subjects showed the same results in all “ventral” tasks with respect to full-term controls without any correlation to gestational age or birth weight. We found a significant negative correlation between Form Coherence Task and Letters Task (p = .014) and between Form Coherence and Silhouette Tasks (p = .017). No correlation was observed between Form Coherence Task and Street and Ghent Tests. A statistical difference was instead found between PTs and controls in two tasks of the VOSP battery that mostly involve the dorsal stream.

Conclusions

Preterm birth per se (in absence of evident brain lesions) is not sufficient to compromise the development of ventral pathway.     

Impaired healing response of periodontal furcation defects following flap debridement surgery in smokers. A controlled clinical trial

OBJECTIVES: The purpose of the present parallel-design, controlled clinical trial was to evaluate the treatment outcome of periodontal furcation defects following flap debridement surgery (FDS) procedure in cigarette smokers compared to non-smokers. MATERIALS AND METHODS: After initial therapy, 31 systemically healthy subjects with moderate to advanced periodontitis, who presented at least one Class I or II molar furcation defect, were selected. Nineteen patients (mean age: 40.3 years, 15 males) were smokers (>or=10 cigarettes/day) and 12 patients (mean age: 44.8 years, 3 males) were non-smokers. Full-mouth plaque score (FMPS) and full-mouth bleeding score (FMBS), probing pocket depth (PPD), vertical clinical attachment level (v-CAL), and horizontal clinical attachment level (h-CAL) were assessed immediately before and 6 months following surgery. RESULTS: Overall, statistically significant v-CAL gain was observed in smokers (1.0 +/- 1.3 mm) and non-smokers (1.3+/-1.1 mm), the difference between groups being statistically significant (p=0.0003). In proximal furcation defects, v-CAL gain amounted to 2.3+/-0.7 mm in non-smokers as compared to 1.0+/-1.1 mm in smokers (p=0.0013). At 6 months postsurgery, non-smokers presented a greater h-CAL gain (1.3+/-1.1 mm) than smokers (0.6+/-1.0 mm), with a statistically significant difference between groups (p=0.0089). This trend was confirmed in both facial/lingual (1.4+/-1.0 versus 0.8+/-0.8 mm) and proximal furcation defects (1.2+/-1.3 versus 0.5+/-1.2 mm). The proportion of Class II furcations showing improvement to postsurgery Class I was 27.6% in smokers and 38.5% in non-smokers. After 6 months, 3.4% of presurgery Class I furcation defects in smokers showed complete closure, as compared to 27.8% in non-smokers. CONCLUSIONS: The results of the present study indicated that (1) FDS produced clinically and statistically significant PPD reduction, v-CAL gain, and h-CAL gain in Class I/II molar furcation defects, and (2) cigarette smokers exhibited a less favorable healing outcome following surgery in terms of both v-CAL and h-CAL gain.     

Peptides of human gingival crevicular fluid determined by HPLC-ESI-MS

The acidic-soluble protein content of human gingival crevicular fluid was analyzed by reverse-phase high-performance liquid chromatography (RP-HPLC), and the eluent deriving from the chromatography separation was directly introduced into an ion-trap mass spectrometer through electrospray ionization (ESI-IT MS). By this technique the molecular weight of peptides/proteins was determined with a precision of approximately 1/10,000 amu. On the basis of the chromatographic behavior and the knowledge of the molecular mass value, some peptides and proteins soluble in acidic solution were unambiguously recognized. Besides high quantities of human serum albumin, alpha-defensins 1-4 and minor amounts of cystatin A, statherin, basic PB salivary peptide and other unidentified components were detected. The presence of alpha-defensins in gingival crevicular fluid is in agreement with their relevant contribution to protein composition deriving from granulocyte secretions. Other peptides and proteins abundant in human saliva, such as proline-rich proteins (PRPs) and histatins, were not detected in gingival crevicular fluid. Further investigations will be necessary to establish the origin of statherin and PB salivary peptide in gingival crevicular fluid.     

Modulation of clinical expression of plaque-induced gingivitis: response in aggressive periodontitis subjects

AIM: The aim of this study was to characterize the gingival inflammatory response to de novo plaque accumulation in subjects treated for aggressive periodontitis (AP). The gingival inflammatory response of the AP subjects was retrospectively compared with that of periodontally healthy individuals (PH) matched for exposure to plaque and of periodontally healthy subjects previously identified as "high responders" (HR) and "low responders" (LR). MATERIALS AND METHODS: 13 AP subjects and 26 matched PH subjects participated in a 21-day experimental gingivitis trial. Plaque index (PlI), Gingival index (GI), gingival crevicular fluid volume (GCF) and angulated bleeding score (AngBS) were recorded at days 0, 7, 14 and 21. Cumulative plaque exposure (CPE), i.e. PlI over time, was also calculated. RESULTS: GCF was significantly higher in AP compared with PH group at each observation interval (p< or =0.001). In addition, GCF was significantly higher in AP group compared with either LR or HR groups at each observation interval (p<0.001). CONCLUSIONS: These results suggest that susceptibility to gingival inflammation in response to de novo plaque accumulation may be related to susceptibility to periodontitis.