Lung ultrasound evaluation in people with cystic fibrosis: A new approach in the pulmonology outpatient clinic.

BACKGROUND: Cystic fibrosis (CF) is a genetic disease that causes progressive lung disease with major impact on the quality of life. Lung ultrasound (LUS) allows to assess the lung involvement through the artefacts analysis and is increasingly used in children but is not yet used to monitor people with CF(pwCF). The main aim of this study was to describe the LUS pattern of pwCF during their routinary check-up visit. The secondary objective was to correlate the LUS findings with pulmonary function indices. METHODS: We performed a cross-sectional observational study, enrolling adolescents and young adults with CF. Each patient underwent clinical assessment, measurement of SpO2, assessment of lung function by spirometry and LUS. RESULTS: Twenty-nine subjects with CF were included. The most frequent alterations were consolidations (72.4%) located in the left apical anterior and right apical posterior regions followed by interstitial syndrome (65.5%). The 41.4% of cases presented the lingula involvement, characterized by a consolidation with static air bronchogram, and 55.2% showed pleural irregularity mainly in the posterior apical regions. A significant correlation was found between the LUS total score and spirometric indices: FEV1 (p = .003), FVC (p = .002), Tiffenau Index <80% (p = .014), and FEF 25-75 (p = .004). CONCLUSIONS: Our study describes LUS findings in pwCF. It also showed a correlation between LUS score and the patients' lung function measured by spirometric indices. We conclude that LUS may be useful in routine monitoring of pwCF in combination with clinical and spirometric assessment.

Nurses' Perceptions of the Quality of Procedural Sedation in Children Comparing Different Pharmacological Regimens.

Nurses play a pivotal role during pediatric procedural sedation and their perspective is an important indicator for the quality of care. The aim of this study is to examine nurses' satisfaction comparing four different pharmacological regimens used for pediatric sedation outside of the operating room. A prospective observational study was conducted in a third-level pediatric teaching hospital, involving all the nurses with experience in the field of pediatric procedural sedation. A 13-item survey was used to assess the level of nurses' satisfaction for the quality of sedation with four different analgesic-sedative drugs. Fifty-one questionnaires were completed by pediatric nurses, with a median length of experience of 10 years. Regarding the overall quality of the sedation, the highest median satisfaction scores were observed for propofol (8, IQR 7-9), dexmedetomidine (8, IQR 6-8) and midazolam (8, IQR 7-9). Ketamine (5, IQR 3-7) displayed the lowest score. When asked to rate their level of perceived safety, nurses gave high scores to all the four drugs studied, with no statistically significant difference between them. Non-pharmacological techniques during procedural sedation were judged as important by 38 (74.5%) nurses. According to this sample of pediatric nurses, the best quality of procedural sedation in children outside of the operating room is obtained with propofol, dexmedetomidine and midazolam. During procedural sedation, nurses feel safe overall, regardless of the pharmacological regimen used. Moreover, they highlighted the relevance on non-pharmacological approaches in the preparation of the child for the procedure.

"Shoulder pain and limitation of motion in a young girl: think different".

BACKGROUND: Primary Synovial Chondromatosis (PSC) is a rare benign tumor of the synovial membrane in which cartilage metaplasia produces calcific loose bodies within the articular space. Only a few cases are reported in the pediatric population and its etiology remains unknown. This condition typically affects large weight-bearing joints with pain, swelling and decrease range of motion. Due to its slow progressions, delayed diagnosis is frequent and differential diagnosis should consider other chronic arthritis and malignancies. While arthroscopic removal of loose bodies is the current treatment up to now, the association of partial or complete synovectomy is debated. CASE PRESENTATION: We report about a 14-year-old girl with a long-lasting right shoulder pain, especially during movements or exercise, localized tenderness and hypotonia of the glenohumeral joint. No previous trauma was mentioned. Blood exams, Mantoux test and plain radiography of the right shoulder were unremarkable. Ultrasound imaging revealed echogenic and calcified bodies stretching the glenohumeral joint and dislocating the long head of biceps tendon. Magnetic resonance showed a "rice-grain" pattern of the right shoulder. From an arthroscopic surgery, multiple loose white bodies were removed within the synovial membrane, and synovial chondromatosis was confirmed by histological analysis. At one month follow up visit, the patient completely recovered without pain. CONCLUSION: Synovial chondromatosis is a very uncommon cause of mono articular pain in children, especially when it affects shoulder. Pediatricians should keep in mind this condition to avoid delayed diagnosis and treatment, even in consideration of the low risk of malignant transformation. Through this case, we would highlight common diagnostic pitfalls and treatment of synovial chondromatosis.

Acute Acalculous Cholecystitis: Think of Hepatitis A Infection and Do Not Underestimate Pain.

ABSTRACT: A 14-year-old adolescent girl presented with severe abdominal pain, tenderness, and guarding in the right upper quadrant associated with nonbilious vomiting, scleral icterus, and fever. Laboratory tests were consistent with acute hepatitis A virus-related cholestatic hepatitis. A point-of-care ultrasound showed mild gallbladder wall thickening with increased color Doppler flow and pericholecystic fluid collection, in the absence of gallstones or biliary ducts dilatation, thus suggesting acute acalculous cholecystitis. Both the clinical symptoms and the point-of-care ultrasound findings completely resolved within 1 week after admission with conservative treatment.

Intranasal dexmedetomidine and intranasal ketamine association allows shorter induction time for pediatric sedation compared to intranasal dexmedetomidine and oral midazolam.

BACKGROUND: Non-painful diagnostic procedures require an inactive state for a prolonged time, so that sedation is often needed in younger children to perform the procedures. Our standard of care in this setting consists of the association between oral midazolam (0.5 mg/kg) and intranasal dexmedetomidine (4 mcg/kg). One of the limits of this approach is that the onset of action is quite delayed (up to 55 min) and poorly predictable. We chose to compare this association with intranasal-ketamine and intranasal-dexmedetomidine. METHODS: This is a "pre-post" study. The study population included the first forty children receiving sedation with the "new" combination intranasal ketamine (3 mg/kg) and intranasal dexmedetomidine (4 mcg/kg) compared to a historical cohort including the last forty children receiving sedation with our standard of care combination of intranasal dexmedetomidine (4mcg/kg) and oral midazolam (0,5 mg/kg). RESULTS: The association intranasal dexmedetomidine and intranasal ketamine allowed for a significantly shorter sedation induction time than the combination intranasal dexmedetomidine and oral midazolam (13,5 min versus 35 min). Both group's cumulative data showed a correlation between age and sedation effectiveness, with younger children presenting a higher success rate and shorter induction time (p 0,001). CONCLUSIONS: This study suggests that the ketamine and dexmedetomidine intranasal association may have a shorter onset of action when compared to intranasal dexmedetomidine and oral midazolam.

Recovery characteristics and parental satisfaction in pediatric procedural sedation.

BACKGROUND: Despite being a standard of care for children undergoing stressful procedures, little data exist on parental perception of pediatric sedation. AIMS: This study aimed to investigate recovery characteristics and parental satisfaction for pediatric sedations performed with four widely used sedative regimens. METHODS: A prospective observational study was conducted at the Institute for Maternal and Child Health of Trieste, Italy, enrolling children undergoing procedural sedation with one of the following pharmacological regimens: propofol, propofol + midazolam, ketamine + propofol, and dexmedetomidine + midazolam. A questionnaire was used to assess the occurrence of symptoms upon recovery from sedation and the following day, and the caregivers' satisfaction for both the recovery pattern and the overall sedation experience, according to a numerical rating scale (0-10). Answers were collected through a telephone survey. The primary outcome was the difference in the quality of the recovery as perceived by caregivers; the secondary and tertiary outcomes were the perceived quality of the overall sedation experience and the frequency of sedation-related adverse events, respectively. RESULTS: Data from 655 patients, 149 receiving propofol, 245 propofol + midazolam, 134 ketamine + propofol, and 127 dexmedetomidine + midazolam, were analyzed. The level of parents' satisfaction for both the recovery and the sedation experience was overall high and increased with the patients' age in all the pharmacological groups (Spearman's rank correlation, ρ .083, p = .033, and ρ .087, p = .026, respectively), with no statistically significant differences between groups when adjusting for age. The occurrence of irritability, prolonged sleepiness, hyperactivity, unsteadiness, hallucinations, emesis, and respiratory distress at any moment negatively affected parental satisfaction. CONCLUSIONS: In this study, caregivers' satisfaction with pediatric sedation was high, regardless of the regimen used. Lower parental satisfaction was associated with younger age, irritability after sedation, prolonged sleepiness, hyperactivity, unsteadiness, hallucinations, emesis, and respiratory distress.

Nonalcoholic fatty liver in children and adolescents: an overview.

Nonalcoholic fatty liver disease is rapidly becoming one of the most common liver diseases in the pediatric population in industrialized countries because of the growing prevalence of obesity and overweight. For this reason, there is a keen and broad interest among researchers to identify new diagnostic noninvasive tools and novel treatment modalities for this condition. Unfortunately, to date, liver biopsy remains the imperfect gold standard for diagnosis. In addition, available noninvasive markers are not fully satisfactory for the diagnosis of fatty liver. Although in recent years many pharmacological agents, on the basis of pathogenetic mechanism of the disease, have been attempted, to date, the guidelines for the management of fatty liver are still lacking. Establishing effective therapeutic strategies to treat the disease represents the challenge for pediatric hepatologists in the near future. In this article, we briefly review the current knowledge and ideas concerning pediatric nonalcoholic fatty liver disease, and discuss the new perspective therapies.

A new Caucasian case of neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD): a clinical, molecular, and functional study.

Citrin is the liver-specific isoform of the mitochondrial aspartate/glutamate carrier (AGC2). AGC2 deficiency is an autosomal recessive disorder with two age related phenotypes: neonatal intrahepatic cholestasis (NICCD, OMIM#605814) and adult-onset type II citrullinemia (CTLN2, OMIM#603471). NICCD arises within the first few weeks of life resulting in prolonged cholestasis and metabolic abnormalities including aminoacidemia and galactosuria. Usually symptoms disappear within the first year of life, thus making a diagnosis difficult after this time. In this study we report a new Caucasian case of NICCD, a seven week old Romanian boy with prolonged jaundice. Sequencing of the AGC2 gene showed a novel homozygous missense double-nucleotide (doublet) mutation, which produces the change of the glycine at position 437 into glutamate. Functional studies, carried out on the recombinant mutant protein, for the first time demonstrated, that NICCD is caused by a reduced transport activity of AGC2. The presence of AGC2 deficiency in other ethnic groups besides Asian population suggests further consideration for NICCD diagnosis of any neonate with an unexplained cholestasis; a prompt diagnosis is crucial to resolve the metabolic decompensation with an appropriate dietary treatment.

Osteonecrosis of the hip after short courses of oral and inhaled steroids in a child with an increased number of glucocorticoid receptors.

We report an unusual case of osteonecrosis of the femoral head associated with recurrent myopathy and bone abnormalities in a two-year-old girl, in whom symptoms occurred after a ten-day course of oral betamethasone for infectious wheezy bronchitis, and eventually recurred and were worsened by topical treatment; a hypersensitivity to glucocorticoids is hypothesised.