Placenta Accreta Spectrum (PAS) Disorder: Ultrasound versus Magnetic Resonance Imaging.

OBJECTIVE: PAS is one of the most dangerous conditions associated with pregnancy and remains undiagnosed before delivery in from half to two-thirds of cases. Correct prenatal diagnosis is essential to reduce the burden of maternal and fetal morbidity. The purpose of our study is to evaluate the accuracy of US and MRI in the diagnosis of PAS. STUDY DESIGN: In this retrospective study, 104 patients with suspected placenta accreta were enrolled and had been investigated with US and MRI. They were divided into four groups: no PAS, accreta, increta, and percreta. RESULTS: Compared to MRI, US results were higher in the diagnosis and in the identification of PAS severity (85% US vs. 80% MRI). For both methods, in the case of posterior placenta, there is greater difficulty in identifying the presence/absence of the disease (67% in both methods) and the severity level (61% US vs. 55% MRI). CONCLUSION: US, properly implemented with the application of defined and standardized scores, can be superior to MRI and absolutely sufficient for the diagnosis of PAS, limiting the use of MRI to a few doubtful cases and to cases in which surgical planning is necessary.

Noncystic fibrosis bronchiectasis in children and adolescents: Follow-up over a decade.

BACKGROUND: Noncystic fibrosis bronchiectasis (NCFB) is still considered an "orphan disease" in pediatric age. OBJECTIVE: The study describes the clinical and functional features, the instrumental, and microbial findings of a large cohort of patients with NCFB, followed in a single tertiary level hospital. METHODS: Children and adolescents diagnosed with NCFB from January 1, 2010 to December 31, 2019 were included. Data from the diagnosis and during the years of follow-up were recorded retrospectively. RESULTS: One hundred and thirty-eight patients were enrolled. The most common cause of NCFB was postinfectious (33%), followed by primary ciliary dyskinesia (PCD) (30%), esophageal atresia (EA) (9.5%), and secondary immunodeficiency (9.5%). Chronic cough was the most frequent symptom. The median age of symptoms presentation was 3 years (interquartile age [IQR]: 12-84), with a precocious onset in PCD and EA groups. The median age of CT diagnosis was 9 years for all groups but PCD patients who were diagnosed at older age. Lingula, medium, upper, and lower lobes were more involved in PCD group, while diffuse distribution was observed in the postinfectious one. Microbial exams showed Pseudomonas aeruginosa colonization higher in PCD patients (22%). Despite microbial differences in airways colonization, no difference in respiratory exacerbation rate was recorded among groups. Lung function tests demonstrated the stability of forced expiratory volume in 1 s (FEV1) and forced vital capacity (FVC) over time, except for the secondary immunodeficiency group. CONCLUSIONS: The role of infections in developed countries should not be underestimated and a major effort to obtain an earlier identification of bronchiectasis should be taken. A prompt diagnosis of NFCB could help to reduce the frequency of exacerbations and improve the stability of lung function over time.

Effects of the COVID-19 pandemic and lockdown on symptom control in preschool children with recurrent wheezing.

INTRODUCTION: Preschool wheezers are at high risk of recurrent attacks triggered by respiratory viruses, sometimes exacerbated by exposure to allergens and pollution. Because of the COVID-19 infection, the lockdown was introduced, but the effects on preschool wheezers are unknown. We hypothesized that there would be an improvement in outcomes during the lockdown, and these would be lost when the lockdown was eased. MATERIALS AND METHODS: Patients underwent medical visits before and after the COVID-19 lockdown. We recorded the childhood Asthma Control Test (cACT) and a clinical questionnaire. Data on symptoms, the need for medications and the use of healthcare resources were recorded. We compared these data with retrospective reports from the preceding year and prospectively acquired questionnaires after lockdown. RESULTS: We studied 85 preschool wheezers, mean age 4.9 years. During the lockdown, cACT score was significantly higher (median 25 vs. 23); families reported a dramatic drop in wheezing episodes (51 vs. none), significant reductions in the day and nighttime symptoms, including episodes of shortness of breath (p < .0001); the use of salbutamol and oral corticosteroids (OCS) dropped significantly (p < .0001) and 79 (95%) patients needed no OCS bursts during the lockdown. Finally, patients had significantly fewer extra medical examinations, as well as fewer Emergency Room visits (p < .0001). All were improved compared with the same time period from the previous year, but outcomes worsened significantly again after lockdown (cACT median: 22). CONCLUSIONS: During the national lockdown, children with persistent preschool wheeze showed a significant clinical improvement with reduction of respiratory symptoms, medication use for exacerbations, and use of healthcare resources. This trend reversed when lockdown restrictions were eased.

Adnexal masses in pregnancy: an updated review on diagnosis and treatment.

Adnexal masses are not common in pregnancy. They are often discovered incidentally during routine ultrasound examinations. In general, 24%-40% of the cases are benign tumors; up to 8% are malignant tumors. Adnexal masses are usually asymptomatic, but sometimes can be responsible for abdominal or pelvic pain. Transvaginal and transabdominal ultrasound is essential to define the morphology of pelvic masses and to distinguish between benign and malignant cases. Magnetic resonance imaging can be a complementary examination when ultrasound findings are equivocal and a useful additional examination to better define tissue planes and relations with other organs. Patient counseling can be challenging because there is no clear consensus on the management of adnexal masses during pregnancy. Treatment options consist of observational management (in case of asymptomatic women with reassuring instrumental findings) or surgery (via laparoscopy or laparotomy). Surgery can be offered as a primary tool when cancer is suspected or when acute complications such as ovarian torsion occur.

A Novel, Portable MESH Nebulizer-An Alternative to Metered Dose Inhaler: Efficacy and Usability in Preschool Wheezers.

Introduction and Objectives: Wheezing episodes are the first causes of doctor's consultation in preschool age. Treatment is usually administered with a metered dose inhaler (MDI) spacer. At variance, many parents and doctors prefer to use a compressor nebulizer, which cannot be easily carried. The study is aimed at testing whether a pocket mesh nebulizer has similar efficacy and acceptability than a standard MDI device. Materials and Methods: The IPAC study was a randomized, controlled, non-inferiority trial (number: 1616/2018, Ospedale Pediatrico Bambino Gesu'-IRCCS). The study had two arms: cases, using MicroAIR U100, and controls, using MDI+spacer device. Both devices were adopted for long-term treatment and for exacerbations. Follow-up was organized with clinical visits and a daily e-diary connected to an application for mobile phone. Results: One hundred patients were enrolled. The frequency of asthmatic symptoms showed a non-inferiority for MicroAIR U100 group vs. MDI. Accordingly, no significant difference was found in the average % of days with cough, wheezing, breathlessness after exercise, days lost at school, and not-programmed visits. Considering only patients with >1 day with symptoms, no significant sdifferences were found in the number of exacerbations nor in the cumulative days with symptoms. The acceptance and usability of both devices have been favorable. However, the MDI+AeroChamber® device showed better acceptability. Conclusions: Our study shows that MicroAIR U-100, a mesh nebulizer, has similar clinical efficacy but lower acceptance and usability than an MDI plus Aerochamber® in delivering therapy in preschool wheezers. Therefore, MicroAIR U-100 might be a valuable second choice, when the delivery of medication with an MDI plus Aerochamber® is not accepted, or wrongly used by the parents.

Ultrasonographic Diagnosis of Placenta Accreta Spectrum (PAS) Disorder: Ideation of an Ultrasonographic Score and Correlation with Surgical and Neonatal Outcomes.

The objective of this study was to evaluate a novel ultrasonographic scoring system for the diagnosis of PAS and the prediction of maternal and neonatal outcomes. In this retrospective study, 138 patients with at least one previous caesarean section (CS) and placenta previa were included. They were divided into four groups ranging from Group 0 (Non PAS) to Group 3 (Placenta Percreta) according to the histological or surgical confirmation. Their ultrasound examinations during pregnancy were reviewed according to the nine different ultrasound signs reported by the European Working Group on Abnormally Invasive Placenta. For each parameter, 0 to 2 points were assigned. The sum of the points reflects the severity of PAS with a maximum score of 20. The scoring system revealed good performances in evaluation metrics, with an overall accuracy of 94%. In addition to this, patients' characteristics and surgical and neonatal outcomes were analyzed with an evidence of higher incidence of complications in severe forms. Our study suggests that antenatal ultrasonographic diagnosis of PAS is feasible with sufficient level of accuracy. This will be important in identifying high-risk patients and implementing preventive strategy.

Does hysterosalpingo-foam sonography have any therapeutic effect? A systematic review.

INTRODUCTION: Tubal patency is one of the mandatory and necessary conditions to be granted in order to guarantee a good pregnancy rate. Numerous studies have been conducted to compare the various testing techniques for tubal evaluation in order to optimize the diagnostic-therapeutic process. Aim of this review is to clarify if hysterosalpingo-foam sonography could be considered as a useful tool not only in the diagnostic procedure, but also in treatment of infertility. EVIDENCE ACQUISITION: We performed a comprehensive search of relevant studies from January 2010 to December 2019 to ensure all possible studies were captured. A systematic search of PubMed databases was conducted. EVIDENCE SYNTHESIS: Over the years, increasingly less invasive approaches have been used to test tubal patency. For many years Laparoscopic with chromopertubation (DLS) has been considered the reference standard, then less invasive procedures have been introduced, such as hysterosalpingography (HSG). Sonohysterosalpingography (HyCoSy) represents a non-invasive procedure with accuracy comparable to HSG. Several studies have been made on different contrast agents that could be used on this procedure and recent studies considered hysterosalpingo-foam sonography (HyFoSy) procedure as a new technique used for the study of tubal function performed on unfertile women. Nowadays, HyFoSy is largely used in the study of tubal patency, but it is not completely clear the role of this technique as treatment of imperviousness of Fallopian tubes, leading to an increase in pregnancy rate after its use. CONCLUSIONS: As described in the literature for other procedures, similarly with HyFoSy, the tubal flushing improves the chance of an embryo implanting and establishing a spontaneous pregnancy. More prospective studies should be taken to better analyze the singular maternal risk fators, hoping to offer more complete indications to recommend HyFoSy.

Use of deferoxamine (DFO) in transfusion-dependent ß-thalassemia during pregnancy: A retrospective study.

OBJECTIVE: To report cases of use of chelation therapy during pregnancy which resulted in favorable outcomes for the babies. MATERIALS AND METHODS: In this retrospective cohort study, we described the evolution and outcome of 9 pregnancies in Italian thalassemic women who received deferoxamine (DFO) inadvertently during early pregnancy. RESULTS: The use of deferoxamine during first trimester did not lead to adverse effects on the fetus or cause major complications for the gestation, although an increase in iron burden was observed after suspending chelation therapy. CONCLUSION: In our experience, iron-chelation therapy might be administrated in pregnancy where the benefits to the mother outweigh the potential risks to the baby.

Diagnosis & management of imported malaria in pregnant women in non-endemic countries.

Malaria in pregnancy is an important cause of maternal and foetal morbidity and is a potentially life-threatening infection. With ever-growing global exchanges, imported malaria in pregnancy is becoming an issue of concern in non-endemic countries where women, because of low immunity, have higher risk of severe diseases and death. Malaria in pregnancy is a dangerous condition which can be associated with important consequences for both mother and child such as stillbirth, low birth weight, maternal anaemia. In non-endemic-countries it is more frequent in its severe form which can lead to maternal death if not treated adequately. Specific anti-malarial interventions such as the use of repellents and insecticide treated bed nets in addition to chemoprophylaxis should be used by pregnant women if they are travelling to endemic areas. In cases of confirmed infection, specific treatment regimens vary according to gestational age and the presence of complications. Malaria should be considered a global health problem, increasingly involving western countries. Clinicians all over the world need to be prepared for this emerging disease both in terms of prevention and therapy.

Efficacy of ulipristal acetate (UPA) for the treatment of expulsion uterine myomas: report of two cases.

Expulsion leiomyoma can represent an emergency condition and may lead to severe vaginal hemorrhage with anemia and urgent hysterectomy. Among medical treatments used for leiomyoma, Selective progesterone receptor modulators (SPRM) and, in particular, Ulipristal Acetate (UPA) have been proved to be effective in the management of bleeding and myoma size reduction. However, to our knowledge, there are no cases reported in literature on the use of UPA used as 'emergency' medical therapy in patients with severe anemia and vaginal bleeding due to expulsion leiomyoma. In this paper we would report two cases of patients affected by expulsion myoma successfully treated with UPA with immediate resolution of vaginal bleeding and subsequent elective conservative treatment.

Hypoventilation disproportionate to OSAS severity in children with Prader-Willi syndrome.

OBJECTIVE: To test the hypothesis that children with Prader-Willi syndrome (PWS) and obstructive sleep apnoea syndrome (OSAS) have hypercapnia for higher proportion of total sleep time (TST) than non-syndromic children with similar obstructive apnoea-hypopnoea index (OAHI). DESIGN: Cross-sectional study. SETTING: Two tertiary care hospitals. PATIENTS: Patients with PWS and non-syndromic children with snoring who underwent polygraphy and were of similar age, body mass index (BMI) z-score and OAHI. MAIN OUTCOME MEASURE: The two groups were compared regarding %TST with transcutaneous CO2 (PtcCO2) >50 mm Hg. The interaction between PWS diagnosis and OSAS severity (OAHI <1 episode/h vs 1-5 episodes/h vs >5 episodes/h) regarding %TST with PtcCO2 >50 mm Hg was tested using multiple linear regression. RESULTS: 48 children with PWS and 92 controls were included (median age 2.3 (range 0.2-14.1) years vs 2.2 (0.3-15.1) years; BMI z-score 0.7±1.9 vs 0.8±1.7; median OAHI 0.5 (0-29.5) episodes/h vs 0.5 (0-33.9) episodes/h; p>0.05). The two groups did not differ in %TST with PtcCO2 >50 mm Hg (median 0% (0-100%) vs 0% (0-81.3%), respectively; p>0.05). However, the interaction between PWS and OSAS severity with respect to duration of hypoventilation was significant (p<0.01); the estimated mean differences of %TST with PtcCO2 >50 mm Hg between children with PWS and controls for OAHI <1 episode/h, 1-5 episodes/h and >5 episodes/h were +0.2%, +1% and +33%, respectively. CONCLUSION: Increasing severity of upper airway obstruction during sleep in children with PWS is accompanied by disproportionately longer periods of hypoventilation when compared with non-syndromic children with similar frequency of obstructive events.

Asthma: Differential Diagnosis and Comorbidities.

Childhood asthma remains a multifactorial disease with heterogeneous clinical phenotype and complex genetic inheritance. The primary aim of asthma management is to achieve control of symptoms, in order to reduce the risk of future exacerbations and progressive loss of lung function, which results especially challenging in patients with difficult asthma. When asthma does not respond to maintenance treatment, firstly, the correct diagnosis needs to be confirmed and other diagnosis, such as cystic fibrosis, primary ciliary dyskinesia, immunodeficiency conditions or airway and vascular malformations need to be excluded. If control remains poor after diagnostic confirmation, detailed assessments of the reasons for asthma being difficult-to-control are needed. Moreover, all possible risk factors or comorbidities (gastroesophageal reflux, rhinosinusitis, dysfunctional breathing and/or vocal cord dysfunction, obstructive sleep apnea and obesity) should be investigated. At the same time, the possible reasons for poor symptom control need to be find in all modifiable factors which need to be carefully assessed. Non-adherence to medication or inadequate inhalation technique, persistent environmental exposures and psychosocial factors are, currently, recognized as the more common modifiable factors. Based on these premises, investigation and management of asthma require specialist multidisciplinary expertise and a systematic approach to characterizing patients' asthma phenotypes and delivering individualized care. Moreover, since early wheezers are at higher risk of developing asthma, we speculate that precocious interventions aimed at early diagnosis and prevention of modifiable factors might affect the age at onset of wheezing, reduce the prevalence of persistent later asthma and determine long term benefits for lung health.

Age and seasons influence on at-home pulse oximetry results in children evaluated for suspected obstructive sleep apnea.

BACKGROUND: Seasonal variability on obstructive sleep apnea has already been studied by polysomnography in children. Winter and spring season emerged as critical periods. No data are currently available for pulse oximetry performed at home. The aim of our study was to evaluate the effect of seasonality and age on the results of at-home pulse oximetry performed in children referred for suspected OSA. METHODS: We retrospectively studied 781 children (64.3% Males), aged 4.9 ± 2.5 years. For all patients, we evaluated both pulse oximetry metrics and the McGill Oximetry Score. Variables for seasonal groups were assessed using Kruskal-Wallis test. A logistic regression model was performed to assess the relationship between patients' main characteristics, season period and the likelihood to have an abnormal McGill Oximetry Score. RESULTS: Patients recorded during winter were significantly younger (p < 0.02), nadir SpO2 was significantly lower (p < 0.002) and DI4 significantly higher than during others seasons (p < 0.005). Moreover, patients recorded during winter were nearly 2 times more likely to have an abnormal MOS (aOR 1.949). The logistic regression showed that also younger age (p < 0.0001) was associated with a higher risk to find an abnormal pulse oximetry. CONCLUSIONS: In our study, the winter season confirms to be a critical period for pulse-oximetry and it should be taken into account by clinicians for a correct interpretation of tests. Our data show that also younger age affects the prevalence of abnormal at-home pulse oximetry in children.

Anaphylactic shock with methylprednisolone sodium succinate in a child with short bowel syndrome and cow's milk allergy.

BACKGROUND: Medications with methyl-prednisolone sodium succinate containing lactose, which potentially contains traces of cow's milk proteins (CMP), could cause allergic reactions or compromise treatment of acute allergic reactions in sensitized patients. CASE PRESENTATION: We describe the unusual case of a one-year-old child affected by short bowel syndrome and history of severe cow's milk allergy (CMA) and anaphylactic reaction due to intravenous administration of methyl-prednisolone sodium succinate (Solu-Medrol 40 mg, Pfizer). He was admitted to our hospital for severe respiratory failure and was initially treated with methyl-prednisolone (Urbason 40 mg, Sanofi Aventis), then with methyl-prednisolone sodium succinate (Solu-Medrol 40 mg, Pfizer). After the intravenous administration of second steroid, immediate anaphylaxis was recorded and treatment was stopped. Antihistamine and epinephrine were required and symptom resolution occurred. CONCLUSION: Children who are highly sensitive to milk may have severe allergic reactions also after exposure to CMP through a different administration route than the oral one. Patients who have food allergies need to pay particular attention to the prescription of drugs and their formulation.

Intestinal permeability in children with recurrent respiratory and gastrointestinal symptoms.

AIM: Increased intestinal permeability has been reported in asthmatic subjects as well as in patients with gastrointestinal disease, thus suggesting the involvement of all the mucosal immune system. We aimed to assess intestinal permeability according to recurrent respiratory and gastrointestinal symptoms in children with asthma and children with functional gastrointestinal disorders (FGIDs). METHODS: In 108 outpatients aged 3-14 years (45 asthmatic, 63 with FGIDs), we measured the urinary lactulose/mannitol (L/M) ratio, performed allergy skin prick tests and administered questionnaires for recurrent respiratory and gastrointestinal symptoms starting from at least 2 months which persisted over the previous 4 weeks. L/M ratios were compared with previously reported normal values yielded by our chromatographic method (liquid chromatography-mass spectrometry). RESULTS: High L/M ratios (>0.030) were less frequent in asthmatic children than in children with FGIDs (9/45: 20% vs. 41/63: 65%, P < 0.001). High L/M ratios were associated with gastrointestinal symptoms in 8/9 asthmatic (P < 0.05) and 39/41 subjects with FGIDs (P < 0.005). L/M ratios were not associated with respiratory symptoms or atopy. In a regression model, a high L/M was predicted by low height, absence of asthma and presence of gastrointestinal symptoms (r = 0.72, P < 0.001). CONCLUSIONS: Increased intestinal permeability is associated with recurrent gastrointestinal symptoms rather than with recurrent respiratory symptoms in both asthmatic children and those with FGIDs. Our findings do not support the hypothesis of mucosal intestinal damage following an inflammatory stimulus in the respiratory mucosa.

Intestinal permeability is increased in children with non-alcoholic fatty liver disease, and correlates with liver disease severity.

BACKGROUND: Increased intestinal permeability seems to play a major role in non-alcoholic liver disease development and progression. AIM: To investigate the prevalence of altered intestinal permeability in children with non-alcoholic fatty liver disease, and to study its potential association with the stage of liver disease. METHODS: We performed a case-control study examining intestinal permeability in children using the lactulose-mannitol bowel permeability test. RESULTS: Overall, 39 consecutive patients (30 males, median age 12 years) and 21 controls (14 males, median age 11.8 years) were included. The lactulose/mannitol ratio resulted impaired in 12/39 patients (31%) and none of the controls. Intestinal permeability was higher in children with non-alcoholic fatty liver disease (lactulose/mannitol ratios: 0.038±0.037 vs. 0.008±0.007, p<0.05). Within the non-alcoholic fatty liver disease group, intestinal permeability was increased in children with steatohepatitis compared to those with steatosis only (0.05±0.04 vs. 0.03 vs. 0.03, p<0.05). Pathological lactulose/mannitol ratio correlated with portal inflammation (p=0.02), fibrosis (p=0.0002), and ballooning of hepatocytes (p=0.003). Blood lipopolysaccharides levels were higher in children with steatohepatitis (2.27±0.68 vs. 2.80±0.35, p<0.05). CONCLUSIONS: Intestinal permeability is increased in children with non-alcoholic fatty liver disease, and correlates with the severity of the disease.

Awareness, understanding and attitudes of Italians regarding palliative care.

There are numerous difficulties encountered in the diffusion of palliative care (PC) in developed countries. A correct and widespread awareness of PC among the general public represents an important factor for its enhanced diffusion and use. The aim of this study is to verify the level of awareness of PC among Italians and their perception of the needs of patients with incurable illness. A random sample of 1897 adult subjects, aged 18 to 74 years, representative of the Italian population, was interviewed after stratification by gender, age (6 strata), education (3 levels), geographic area of residency (4 areas) and town of residency (4 dimensions). 59.4% of those interviewed had heard of PC, but only 23.5% of them believed that they had an adequate or precise idea of what PC is; 27% of them did not know or had a mistaken idea about the nature of PC. The most accepted perception was that PC alleviates pain and improves quality-of-life. The principal concerns attributed to incurable patients were: fear of suffering and of death, and the principal needs were perceived as: relief from pain and physical suffering. The primary needs of the family were identified in: medical and nursing care at home, followed by: care provided by volunteers and psychological support. The most appropriate care-setting for these patients was indicated as their home, possibly with the support of professional carers. In the case of children, the main concern indicated was that of being "separated" from family, friends, home and toys. Only 45.2% of interviewees thought that they knew a person who had experienced PC. In conclusion, the awareness of the Italian population of PC is scarce and often incorrect. In order to achieve a greater diffusion and better use of PC in our country, this awareness needs to be improved by appropriate intervention.

Giant pilomatricoma mimicking a malignant parotid mass.

Pilomatricomas are benign neoplasms of the hair follicle occurring relatively often in the pediatric population. However, lesions attaining massive proportions are very rarely encountered. We describe such a case presenting in the parotid region and initially misdiagnosed as malignancy. Preoperative biopsy provided accurate diagnosis and allowed definitive surgical excision using a parotid-sparing approach. In children, giant pilomatricoma should be included into the differential diagnosis of noninflammatory masses of the salivary gland region.