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Pregnancy rates in women on dialysis have increased in the last decades, thus making it a topic of growing interest. The rarity of this event is predominantly due to fertility problems and to the high rates of pregnancy failure including stillbirth, fetal, and neonatal deaths. We conducted a narrative review of existing literature in order to analyze the major issues about pregnancy on dialysis to give the reader a fully updated perspective about this topic which, even if not common, is becoming more and more frequent. Even if recently acquired knowledge has improved diagnosis and treatment of dialysis pregnancies focusing on several aspects, pregnancy on dialysis remains a great challenge for obstetricians and should be managed by a multidisciplinary expertise team. Dialysis in pregnancy may be necessary for women previously affected by end stage renal disease (ESRD) becoming pregnant, or in case of acute renal injury presenting for the first time during gestation or, again, in case of existent renal pathology worsening during pregnancy and requiring dialysis. Although some evidence suggests that more intensive dialysis regimens are correlated with better obstetric outcomes, the optimal therapeutic protocol still remains to be established.

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.

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.

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.

ObjectiveTo 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).DesignCross-sectional study.SettingTwo tertiary care hospitals.PatientsPatients 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 measureThe two groups were compared regarding %TST with transcutaneous CO2 (PtcCO2) >50 mm Hg. The interaction between PWS diagnosis and OSAS severity (OAHI 5 episodes/h) regarding %TST with PtcCO2 >50 mm Hg was tested using multiple linear regression.Results48 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 (p2 >50 mm Hg between children with PWS and controls for OAHI 5 episodes/h were +0.2%, +1% and +33%, respectively.ConclusionIncreasing 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.

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.

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.

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. Keywords: Deferoxamine, Iron chelation therapy, Magnetic resonance T2*, Pregnancy, Thalassemia

Corticosteroids are widely used for the treatment of allergic reactions but paradoxically themselves may induce acute, delayed, local or systemic allergic reactions and even anaphylaxis with Kounis syndrome. They can suppress the release of arachidonic acid from mast cell membranes, via phospholipase A2 and eicosanoid biosynthesis inhibition. Corticosteroids can promote cell apoptosis and mediate in annexin or lipocortin synthesis, substances that modulate inflammatory cell activation, adhesion molecule expression, transmigratory and phagocytic functions. Antigen-antibody reaction, hapten formation, and medication contaminants are some of the incriminated causes. Patients with atopic diathesis are particularly vulnerable. Complete and thorough previous history of drug reactions or allergies is necessary before administration of any particular medication including corticosteroids.

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.

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.

Although most children with Prader-Willi syndrome (PWS) have obstructive sleep apnoea syndrome (OSAS), the prevalence of sleep-related hypoventilation is unknown. We hypothesized that children with PWS have hypercapnia for higher proportion of sleep time (ST) than age- and BMI-matched control children with similar obstructive apnoea-hypopnoea index (OAHI). Methods: Children with PWS undergoing nocturnal polygraphy prior to growth hormone commencement were matched for age, BMI z-score and OAHI to otherwise healthy children with snoring, adenotonsillar hypertrophy and/or obesity. The interaction between PWS diagnosis and OSAS severity (OAHI>5/h vs. ≤5/h) regarding their effect on %ST with transcutaneous CO 2 (PtcCO 2 )>45 mmHg was explored using a general linear model. Results: 48 children with PWS and 93 controls were included (3.9±4.1 y.o. vs. 4.1±4 y.o.; BMI z-score 0.73±1.91 vs. 0.78±1.66; OAHI 3.2±6.7 vs. 2.7±6.2/h, respectively; P>0.05 for all). For OAHI>5, children with PWS and controls had greater difference in %ST with PtcCO 2 >45 (PWS: 76.6±24.7% vs. controls: 40.3±37.2%) than for OAHI≤5 (PWS: 22.6±32.1% vs. controls: 17.9±27.1%). More specifically, the interaction between PWS and OSAS severity regarding their effect on %ST with PtcCO 2 >45 was significant after adjustment for age, gender and BMI z-score (P=0.03). Results did not change when hypoventilation was defined as PtcCO 2 >50 mmHg. Conclusion: During sleep, children with PWS and OSAS hypoventilate disproportionately to the degree of upper airway obstruction. This has potential pathophysiological implications but also demonstrates that simple oximetry studies are inadequate to monitor this group of patients.

Prader- Willi Syndrome (PWS) is a multisystem genetic disorder. In infancy, patients are typically hypotonic and may fail to thrive, but as they grow older, many become obese and develop obstructive sleep apnoea (OSA). We aimed to determine the prevalence of the respiratory complications seen in our cohort of patients referred from a regional PWS clinic in London. We performed a retrospective casenote review of the 49 children with PWS seen from 2010 to 2016, highlighting conditions that could influence their respiratory status. The median age was 5.2[0.4-15.8][range]yrs, 27/49(55%)male, BMI z score 0.7[2.1] mean[SD]. Only 7/49(14%) were overweight/ obese. 75% are on growth hormone (GH). 4/49(8%) had a history of recurrent chest infections: 1 child isolated P. aeruginosa from airway secretions and received oral ciprofloxacin and nebulised colomycin in an attempt at eradication. 11/49(22%) children were at risk of aspiration on videofluoroscopy: 1 was treated with thickened fluids, 7 required nasogastric (ng) tube feeds, 3 have had gastrostomies. Of the 7/11 who needed ng tube feeds, 3 improved and resumed a normal diet within 1 year. 5/49(10%) have scoliosis for which 2 have required spinal surgery. 1 is using CPAP for OSA, 3 NIV for nocturnal hypoventilation and 1 is on nocturnal oxygen for central apnoeas. With early initiation of GH and better clinical management, obesity is not as common in PWS as previously described. Younger children can have problems with recurrent chest infections and aspiration should be considered as a possible cause. A significant number have sleep disordered breathing which may be further complicated by scoliosis. We conclude that careful respiratory follow up is indicated in PWS.

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.

Background Developmental Dysplasia of the Hip (DDH) is an abnormal growth of the hip structures, regarding both osseous and soft tissues. While different factors are strongly associated and the overall frequency reported is between 1 and 5 cases per 1000, its aetiology and prevalence are not well established. Aim Aim of our study was to assess the relationship between the presence of hip ossification core and hip dysplasia and to evaluate the overall prevalence of this disorder among our population. Methods the same examinator performed hip ultrasonography (US) to all babies, using Graf method and a questionnaire about biological data was administered to all parents. Results 947 US were performed to all patients between the 2° and the 22° week of life (493 male, 454 female) 934 US were normal, 10 showed physiological hip immaturity, only 3 demostrated pathological hip conformation (2 with IIC grade and 1 with IV grade). The presence of hip ossification core doesn’t correlate with DDH, nutritional factors and fetal presentation but had a strong relationship with birth weight and female sex. Conclusions our data about the prevalence confirmed the previous results, (3.1/1000): The presense of hip ossification core is not related with DDH.