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Background: Children with medical complexity (CMC) have been defined (Cohen et al., Pediatrics 127: 529–538, 2011.) as an emerging population potentially eligible for PPC. The current study investigated the prevalence of children with medical complexities eligible for a local palliative care network, including a paediatric hospice. Methods: A retrospective cross-sectional survey has been conducted using children clinical charts from 14 local health authorities of our region (Emilia Romagna, Italy). Results: The total number of children with life-limiting conditions was 601, with a mean age of 7.4 ± 4.8 years, a prevalence of 8.4/10.000 residents < 19 years of age and a heterogeneous presentation among the provinces in the region. Neurological diseases affect 51% of patients, followed by congenital diseases (21%) and pathologies originating in the perinatal period (6%), while only 4% of the patients had a cancer diagnosis. Patients are dependent from many devices and supports: 32% had a gastrostomy, 22% a respiratory support and 15% of patients had both of them. Conclusions: Observed regional prevalence of complex needs is lower than that published from other European countries. More research is needed to raise awareness of palliative care for children with medical complexities in order to address specific needs. [ABSTRACT FROM AUTHOR]

Our report covers two cases of severe hypoxic-ischemic encephalopathy in newborns whose birth was complicated by shoulder dystocia. In both cases, there were inconsistencies observed among cardiotocographic traces, baby's clinical conditions at birth, and umbilical cord blood gases. Namely, normal cardiotocographic monitoring and cord pH > 7, in spite of the fact that the newborns were severely depressed at birth and their blood gases evaluated within 1 h from birth showed a severe metabolic acidosis. Moreover, one of the two newborns displayed moderately low hemoglobin levels. Metabolic and infectious causes were ruled out. Both newborns developed severe hypoxic-ischemic encephalopathy and received therapeutic hypothermia for 72 h. Both survived, one with a severe dystonic cerebral palsy whereas the other developed only a mild developmental delay in language. Cardiac asystole theory could explain these two cases, reinforcing the need for specific resuscitation guidelines for infants experiencing a birth complicated by shoulder dystocia., (Copyright © 2020 Ancora, Meloni, Soffritti, Sandri and Ferretti.)

Introduction: The survival of preterm babies has increased worldwide, but the risk of neuro-developmental disabilities remains high, which is of concern to both the public and professionals. The early identification of children at risk of neuro-developmental disabilities may increase access to intervention, potentially influencing the outcome., Aims: Neuroprem is an area-based prospective cohort study on the neuro-developmental outcome of very low birth weight (VLBW) infants that aims to define severe functional disability at 2 years of age., Methods: Surviving VLBW infants from an Italian network of 7 neonatal intensive care units (NICUs) were assessed for 24 months through the Griffiths Mental Developmental Scales (GMDS-R) or the Bayley Scales of Infant and Toddler Development (BSDI III) and neuro-functional evaluation according to the International Classification of Disability and Health (ICF-CY). The primary outcome measure was severe functional disability at 2 years of age, defined as cerebral palsy, a BSDI III cognitive composite score < 2 standard deviation (SD) or a GMDS-R global quotients score < 2 SD, bilateral blindness or deafness., Results: Among 211 surviving VLBW infants, 153 completed follow-up at 24 months (72.5%). Thirteen patients (8.5%) developed a severe functional disability, of whom 7 presented with cerebral palsy (overall rate of 4.5%). Patients with cerebral palsy were all classified with ICF-CY scores of 3 or 4. BSDI III composite scores and GMDS-R subscales were significantly correlated with ICF-CY scores (p < 0.01)., Conclusion: Neuroprem represents an Italian network of NICUs aiming to work together to ensure preterm neuro-developmental assessment. This study updates information on VLBW outcomes in an Italian region, showing a rate of cerebral palsy and major developmental disabilities in line with or even lower than those of similar international studies. Therefore, Neuroprem provides encouraging data on VLBW neurological outcomes and supports the implementation of a preterm follow-up programme from a national network perspective.

OBJECTIVE Hydrocephalus treatment in extremely low-birth-weight (ELBW) infants still represents a challenge for the pediatric neurosurgeon, particularly when the patient weighs far less than 1000 g. In such cases, the benefits in terms of neurological outcome following early treatment do not always outweigh the surgical risks, especially considering the great difference in the surgical risk before patient weight increases. To assess the efficacy and reliability of a percutaneous-tunneled, transfontanellar external ventricular drain (PTTEVD) in ELBW infants, the authors started a new protocol for the early surgical treatment of hydrocephalus. METHODS Ten cases of posthemorrhagic hydrocephalus (PHH) in ELBW infants (5 cases < 700 g, range for all cases 550-1000 g) were treated with a PTTEVD that was implanted at bedside as the first measure in a stepwise approach. RESULTS The average duration of the procedure was 7 minutes, and there was no blood loss. The drain remained in place for an average of 24 days (range 8-45 days). In all cases early control of the hydrocephalus was achieved. One patient had a single episode of CSF leakage (due to insufficient CSF removal). In another patient Enterococcus in the CSF sample was detected the day after abdominal surgery with ileostomy (infection resolved with intrathecal vancomycin). One patient died of Streptococcus sepsis, a systemic infection existing prior to drain placement that never resolved. One patient had Pseudomonas aeruginosa sepsis prior to drain insertion; a PTTEVD was implanted, the infection resolved, and the hydrocephalus was treated in the same way as with a traditional EVD, while the advantages of a quick, minimally invasive, bedside procedure were maintained. Once a patient reached 1 kg in weight, when necessary, a ventriculoperitoneal shunt was implanted and the PTTEVD was removed. CONCLUSIONS The introduction of PTTEVD placement in our standard protocol for the management of PHH has proved to be a wise option for small patients.

Background: Sucralose is an organochlorine artificial sweetener approximately 600 times sweeter than sucrose and used in over 4,500 products. Long-term carcinogenicity bioassays on rats and mice conducted on behalf of the manufacturer have failed to show the evidence of carcinogenic effects., Objective: The aim of this study was to evaluate the carcinogenic effect of sucralose in mice, using a sensitive experimental design., Methods: Five groups of male (total n = 457) and five groups female (total n = 396) Swiss mice were treated from 12 days of gestation through the lifespan with sucralose in their feed at concentrations of 0, 500, 2,000, 8,000, and 16,000 ppm., Results: We found a significant dose-related increased incidence of males bearing malignant tumors (p < 0.05) and a significant dose-related increased incidence (p < 0.01) of hematopoietic neoplasias in males, in particular at the dose levels of 2,000 ppm (p < 0.01) and 16,000 ppm (p < 0.01)., Conclusions: These findings do not support previous data that sucralose is biologically inert. More studies are necessary to show the safety of sucralose, including new and more adequate carcinogenic bioassay on rats. Considering that millions of people are likely exposed, follow-up studies are urgent.

Objectives: Brain damage following a perinatal hypoxic-ischemic (HI) insult has been documented by different diagnostic techniques. The aim of the present study was to relate a-EEG time course during the first 24h of life to brain metabolic changes detected by proton MR spectroscopy ((1)H-MRS) at 7-10days of life and to evaluate their correlation with outcome., Methods: Thirty-two patients with any grade HI encephalopathy were studied. Thirty-one out of 32 patients survived and underwent (1)H-MRS examination at 7-10days of life; a-EEG was recorded during the first 24h of life in 27/32 newborns; 26 patients underwent both examinations. Griffiths test, evaluation of motor skills, visual and hearing function were performed at regular intervals until the age of 2years., Results: a-EEG at 6, 12 and 24h of life showed a significant correlation with outcome. N-acetyl-aspartate/creatine (Cr), Lactate/Cr and myo-inositol differed significantly between patients with normal or poor outcome. a-EEG time course during the first 24h of life showed improvement in newborns with normal (1)H-MRS and good outcome and a deterioration in those with abnormal (1)H-MRS and poor outcome., Conclusions: a-EEG time course may be able to document the severity and the evolution of the cerebral damage following an HI event. a-EEG is related to the severity of cerebral injury as defined by (1)H-MRS and both examinations showed a good correlation with outcome. These data, obtained in non-cooled infants, may represent reference data for future investigations in cooled infants., (Copyright © 2009 Elsevier B.V. All rights reserved.)

Background: The Italian and European Best Practice Guidelines (EBPG) recommend a target haemoglobin value greater than 11 g/dl in most patients with Chronic Kidney Diseases. However, it is still difficult to maintain these values at a steady rate. Thus, the main aim of the study was to evaluate, throughout 2005, how many patients steadily maintained the performance targets related to anaemia treatment., Methods: The survey was conducted on 3283 patients on haemodialysis (HD) and peritoneal dialysis (PD) at 20 Italian dialysis centres. 540 patients were randomly selected; each centre provided a statistically significant sample proportional to its total number of patients. Maintenance of the following target levels was assessed over time: Haemoglobin (HB) 11-12 gr/dl; Iron: 60-160 mcg/dl; Ferritin: 30-400 mcg/l; Transferrin: 200-360 mg/dl; Transferrin saturation percentage (TSAT %):> 25 <50; Dialysis doses (KT/V): >1.2 <2.0 for non-diabetic HD patients; >1.5 <2.2 for diabetic HD patients; DP: >1.8 <2.5.Outcome included:1- Percentage of target maintenance for each parameter.2- Erythropoietin dose in relation to dialysis techniques, presence of cancer or myeloma, diabetic status, Vitamin B therapy.3- Erythropoietin dose (International Units/kg/week) (IU/kg/wk) depending on: haemoglobin values, hospitalization of more than 3 days., Results: Mean age was 65.1; mean haemoglobin concentration over the whole population was 11.3 gr/dl (Standard Deviation (SD): 0.91). The clinical performance targets were maintained over time as follows: HB: 4.3% (Mean 11.43 gr/dl) (SD: 0.42); Ferritin: 71.1% (Mean: 250.23 mcg/L (SD:104.07); Iron: 95.0% (Mean 59.79 mcg/dl)(SD:16.76); Transferrin: 44.8% (Mean 216.83 mg/dl) (SD: 19,50); TSAT %: in 8.4% (Mean: 34.33% (SD: 6.56); HD KT/V: 61.0% (Mean:1.46) (SD: 0.7); PD KT/V:31.4% (Mean: 2.10) (SD: 0.02). The average weekly dose of Erythropoietin (IU/Kg/Wk) was significantly lower for the peritoneal dialysis technique; the higher haemoglobin values, the lower the Erythropoietin dose (IU/Kg/Wk)., Conclusion: A very low percentage of patients maintained haemoglobin target values over time. We need to identify precise criteria to evaluate the stability over time of clinical performance targets proposed by the guidelines.

Background: Exercise has positive psychophysical effects on dialysis patients, thus effective programs should be identified. We evaluated the effects of an original 6-month walking program on physical capacity, health-related quality of life (HRQL) and postdialysis fatigue (PDF)., Methods: Thirty-one dialysis patients (19 male, mean age 65 -/+ 11 years) were divided into exercise (group E; n=17) and control (group C; n=14) groups, and evaluated upon entry, after the 6-month program and 19 -/+ 3 months later. Outcome measures were 6-minute walking distance (6MWD), SF-36 scale scores, self-reported PDF and recovery time. E group was assigned 2 daily 10-minute home walking sessions on the nondialysis day at a speed 50% below maximal treadmill speed as determined and updated monthly at the hospital. C group: no exercise., Results: Twenty patients (13 from E, 7 from C) completed the study. The E group, unlike the C group, increased 6MWD (308 -/+ 105 m, to 351 -/+ 118 m, p=0.0007), and HRQL, significantly for bodily pain, physical role and mental health (p<0.05), decreased PDF and recovery time (p<0.05). At the follow-up, 15 patients were reevaluated (9 from E, 6 from C). The E group was still active and showed 6MWD similar to baseline, with a decline of 0.13 -/+ 1.72 m/mo. The C group decreased 6MWD (p=0.026) with a decline of 3.43 -/+ 3.2 m/mo. For both groups, HRQL, PDF and recovery time showed slight variations from baseline., Conclusions: In dialysis patients, a 6-month exercise program prescribed at the hospital and performed at home improved physical capacity, HRQL and PDF symptoms. Patients maintained an active lifestyle after discharge and showed a slow functional decline over a 2-year period.

Background: Peripheral arterial disease (PAD) is frequently diagnosed in subjects with chronic kidney disease. Hemodialysis (HD) patients with PAD show increased morbidity and mortality and health care costs increase. Management of this complication requires time and skill by nephrologists, although negative results are frequent., Case Report: A 59-year-old Caucasian man on HD with advanced lower extremities peripheral disease and massive calcification of a plaque in the abdominal aorta has been enrolled in a home-based exercise training program. His compliance was high and claudication improved. Pain threshold speed (PTS) and maximal walking speed rose from 2.8 and 3.3 to 3.6 and 4.6 Km/h respectively. The increasing functional capability improved his quality of life and changed positively his life-style., Conclusions: Physical exercise confirms its effectiveness in reducing symptoms due to PAD. A rehabilitation program performed at home at a specific velocity, just below the PTS, and maintained by a metronome appears to be well suited for HD patients because it induces functional improvements and vascular adaptations with low costs.

In 12 hemodialysis (HD) patients with persistently raised serum alanine amino-transferase concentrations, 6 of which with anti-HCV antibodies, a liver biopsy was performed. The histological examination showed chronic persistent hepatitis or less significant changes in 11 patients and mild chronic active hepatitis in only 1. Non-A, non-B hepatitis seems to demonstrate in HD patients a low tendency to induce an active and progressive liver disease.

Microangiopathy is one of the most frequent diseases in diabetic patients. A diabetic man with a kidney transplant and with severe and progressive vasculopathy with early necrosis of the fingers and toes was submitted to three prostacyclin (PGI2) intravenous infusions (5 ng/kg/min for forty-eight hours) during a period of two months. Three months after the last infusion, radiographs of the hands and feet showed a marked reduction in the extent of the vascular calcifications and healing of the ischemic-necrotic areas. The authors discuss the immediate and later effects of PGI2 infusion in a case of diabetic vascular disease.