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A 7-year-old boy was admitted to the Emergency Unit for a one-month history of low back pain, which got worse and resulted in a refusal to walk. On physical examination, the patient refused to stand, and palpation of the spinous processes of the lumbosacral spine evoked pain. Blood tests were normal, except for raised erythrocyte sedimentation rate (51 mm/h, ULN 20 mm/h). An X-ray showed fecaloma and MRI of the spine was performed. Different diagnostic hypotheses and diagnostic exams are discussed.

Key words: Churg-strauss syndrome, Eosinophilic granulomatosis with polyangiitis in childhood, Vasculitis, Asthma, Hypereosinophilia Background - Eosinophilic granulomatosis with polyangiitis, formerly known as Churg-Strauss syndrome, is an extremely rare systemic vasculitis in the paediatric population. The hallmarks of eosinophilic granulomatosis with polyangiitis are a long history of asthma and peripheral eosinophilia with eosinophilic inflammation that may involve several organs. Findings - The paper reports the clinical characteristics, courses, and outcomes of the four patients diagnosed with eosinophilic granulomatosis with polyangiitis at IRCCS Burlo Garofolo (Trieste, Italy) from 1996 to 2015. The mean age at diagnosis was 11.5 years. All the patients presented a history of asthma and peripheral eosinophilia at diagnosis. 3/4 of the children presented upper airway and pulmonary disease. Skin and heart involvement was present in half of the patients, whereas gastrointestinal and neurological symptoms were reported in 25% of the cases. When performed, tissue biopsy revealed eosinophilic inflammation in all the cases. Anti-neutrophil cytoplasmic antibodies were negative in 66% patients. One young child died shortly after presentation, one remitted after immunosuppressive treatment and two patients needed low-dose corticosteroid therapy to maintain the remission. Conclusion - Comparison with an updated review of the series and cases of childhood-onset eosinophilic granulomatosis with polyangiitis reported in the literature showed similar demographic characteristics, clinical features and outcomes. Cardiac disease represents the poorer prognostic factor, leading to the 60% of the deaths reported.

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A diagnosis of arteriovenous fistula was finally made in a 17-year-old girl presenting with “quivering” headache. A history of neonatal scalp catheterization was present.

To investigate the coping strategies of a group of adolescents with somatic symptom disorder compared to non-somatic symptom disorder peers during the COVID-19 related lockdown.This cross-sectional study is the second part of a previously published study showing an improved trend in depression and anxiety in a group of patients with somatic symptom disorder compared to non-somatic symptom disorder peers. An anonymous semi-structured survey was distributed to two groups of Italian adolescents to measure the impact of quarantine on their daily life and coping strategies.We recruited 115 adolescents, 58 (50.4%) mean age 15.3, with a recent diagnosis of somatic symptom disorder and 57 (49.6%) mean age 15.8, control peers.The aim of this study was to detect differences in coping strategies and relationships with parents and peers, during the lockdown period in a group of adolescents with somatic symptom disorder and low disease burden when compared with a non-somatic symptom disorder group.The relationship with parents significantly worsened in 4 (6.9%) of adolescents with somatic symptom disorder compared to 12 (21.1%) adolescents in the non-somatic symptom disorder group (p = 0.048). The relationship with peers significantly improved in 13 (22.4%) of adolescents with symptom disorder versus 3 (5.3%) of peers of the non- somatic symptom disorder group (p = 0.013).Adolescents with somatic symptom disorder with a low burden of physical symptoms experienced less deterioration in their relationships with parents and peers than the non-somatic symptom disorder group.

There is growing literature about the SARS-CoV-2 pathogenetic effects exerted during pregnancy and whether vertical transmission or premature birth is possible. It is not well known whether changes in the immune system of pregnant women may lead to a marked susceptibility to infectious processes and the risk of adverse maternal and neonatal complications such as preterm birth, spontaneous abortion, hospitalization in an intensive care unit, transmission to the fetus or newborns, and fetal mortality are poorly understood. Along with this ongoing debate, it is not well defined whether, during pregnancy, the role of host susceptibility in producing a specific inflammatory response to SARS-CoV-2 may represent distinctive markers of risk of vertical transmission. Furthermore, SARS-CoV-2 impact on the vaginal microbiome has not yet been described, despite mounting evidence on its possible effect on the gastrointestinal microbiome and its influence on infectious diseases and preterm labor. This report describes the impact of SARS-CoV-2 on a twin pregnancy diagnosed with infection at the third trimester of gestation including tissue infections, inflammatory response, antibody production, cytokine concentration, and vaginal microbiome composition. We identified a pattern of cytokines including IL1-Ra, IL-9 G-CSF, IL-12, and IL-8 differently expressed, already associated with previously infected patients. We detected a similar concentration of almost all the cytokines tested in both twins, suggesting that the SARS-CoV-2-induced cytokine storm is not substantially impaired during the placental passage. The analysis of the vaginal microbiome did not show relevant signs of dysbiosis, similar to other healthy pregnant women and twin healthy pregnancies. The aim of this report was to analyze the immunological response against SARS-CoV-2 infection and virus tissue tropism in a twin pregnancy.

Adolescents with mental health disorders are a high-risk population and problems during COVID-19 lockdowns have included increasing, widespread anxiety, fear, anger and uncertainty. Somatic symptom disorder (SSD) is characterised by disproportionate thoughts, feelings and behaviours about physical symptoms associated with the distress and disruption of everyday functioning. SSD accounts for 15-25% of adolescent mental health cases in primary care paediatric settings,3 and 8.6% of non-traumatic adolescent pain in emergency departments.4.

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It is 04:00. The phone rings: the delivery room announces that 25-week preterm is about to be born from unstoppable caesarean labour; we will probably have to intubate him. They do not have time to say anything else; I start running. It is a recurring nightmare of a neonatologist’s nights, being alone to manage a preterm delivery. Imagine then that he is a 29-year-old resident who works in a neonatal intensive care unit, the place where he always wanted to be, and who is about to manage a problematic pregnancy of two little preterm twins, sons of a gynaecologist resident. Imagine there is a father outside the delivery room. Immerse yourself in this father, who, on the other side of the receiver, with a heart beating and quivering with expectation, in the touching attempt to hold back the fleeting moment a little longer, wishes that everything goes well. The dream fades, and the only audible sound is the low hum of the intensive care unit alarms, the place I love. It is the unconquerable temple and, at the same time, the arena where everyday silent battles are fought in the name of life, where the soul is broken down into thousand doubtful arabesques, where hesitation is not allowed. The day I learnt that I would be a father, it was spring and there was a light breeze: my partner took me to the Molo Audace: literally the pier of the …

Background Children with severe cognitive impairments (CI) from any cause, such as cerebral palsy (CP), dysmorphic or chromosomal syndromes, traumatic brain injury, neurodegenerative disorders, and epileptic syndromes, experience pain more frequently than healthy peers. Although it is a common problem, pain is many times unrecognized because these patients are often unable to self- verbalize it or exhibit uncommon behaviors when they experience it, leading to a delay in the recognition and management of pain by the physicians. Untreated chronic or recurrent pain profoundly affects the quality of life of these children and interferes with their performance and adaptive function. Recognition, measurement, and treatment of pain in this population is challenging, and a clinical diagnosis of urinary tract infection or lithiasis may not be immediate. Case presentation: We report the case of an eight-year-old child with cerebral palsy who was admitted for recurrent abdominal pain lasting for one month. While blood laboratory tests were all in the normal range, the diaper examination revealed multiple stones. Urine culture tested positive for Providencia stuartii, a Gram-negative bacteria, and an antibiotic treatment with ceftibuten was started for two weeks. The patient fully recovered in three days without any recurrence. Conclusions urine analysis, to detect an infection, and renal and bladder US, to rule out stones, should be systematically considered in patients with cognitive impairment with unexplained pain. In a pragmatic, concise approach, the basic principles of diagnosis and treatment of pain in children with cognitive impairment are reviewed.

Background Hereditary multiple osteochondromas (HMO) is a genetic condition characterized by the presence of multiple osteochondromas, usually at the lateral side of the most active growth plate of a long bone. These lesions may persist, be asymptomatic during childhood, and may increase in number and size until growth plates close. Therefore, diagnosis of HMO in children and young people can be challenging; while short stature can be more evident at the onset of puberty, asymptomatic ostheocondromas can progress into different degrees of orthopedic deformity. Moreover, multiple complications may arise due to the presence of osteochondromas, including tendon and compression muscle pain, neurovascular disorders, obstetric problems, scoliosis and malignant transformation into secondary peripheral chondrosarcoma in adulthood. Case presentation We report the case of a girl admitted to our Institute for growth delay. While laboratory tests, including growth hormone stimulation test, were normal, left hand X-ray revealed multiple osteochondromas, suggestive for HMO. The genetic test for EXT1 and EXT2 genes confirmed the radiological diagnosis, with a mutation inherited from the mother who displayed the same radiological abnormalities along with recurrent limb pain episodes. Conclusions HMO is a genetic condition whose diagnosis can be challenging, especially in females. Every pediatricians should consider a skeletal dysplasia in case of unexplained growth delay and a skeletal survey might be fundamental in reaching a diagnosis.

A 13-year-old boy was admitted for a 4-day history of fever, malaise, sore throat and cough, treated with amoxicillin for 2 days. Physical examination revealed sparse targetoid cutaneous lesions, stomatitis with vesicles, blisters and mild conjunctival hyperaemia (figure 1). Blood tests showed an increase in erythrocyte sedimentation rate (92 mm/hour; normal value

An 8‐year‐old girl presented with a 2‐week history of cutaneous lesions localised to the distal upper and lower extremities.

A 14-year-old girl was admitted to our institute with a history of intermittent bilateral ankle swelling, and moderate but progressively worsening pain which has lasted for 2 years. The patient’s history was unremarkable. She did not take medications and was not involved in any sports activity. She reported no fever, gastrointestinal symptoms, fatigue, weight loss, travels abroad or previous infections. She reported moderate pain at night, associated with a sense of heaviness, tightness and general discomfort, and with no response to ibuprofen. Physical examination was remarkable only for bilateral ankle non-pitting oedema, more evident on the left leg, with a thickened skinfold at the base of the second toe, and without redness, swelling or skin warming. The patient had been previously examined, and her foot and ankle X-rays, ultrasound (US) and MRI were all negative. Blood tests (white cell count, C reactive protein, erythrocyte sedimentation rate, albumin, antinuclear antibodies, creatinine, transaminase, creatine kinase, lactate dehydrogenase, thyroid function and glucose) and urinalysis were in the normal range. Her ocular assessment and echocardiogram were also normal. Question 1 Based on the clinical picture and laboratory tests, what is the most likely diagnosis? Deep venous thrombosis. Osteochondritis. Lymphoedema. Juvenile idiopathic arthritis. Question 2 Based on what you see in figure 1, what is the underlying cause? Recurrent bacterial lymphangitis. Primary lymphoedema. Tumour. Filariasis. Figure 1 Lymphoscintigraphy of the lower extremities showing insufficient deep lymphatic circulation in the left leg (red arrow, A) replaced by superficial drainage (B). Question 3 Which is the best diagnostic test to confirm the diagnosis? US scan. MRI. Lymphoscintigraphy. Reassurance and clinical follow-up. Question 4 What is the mainstay of management of this condition? Wait and see. Antibiotic course. Supportive therapy (ie, physical activity, elevation of extremities, pneumatic compression). Surgical intervention. Answers can be found on page 2.

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ObjectiveThis study aims to explore the attitude of adolescents with chronic diseases toward social media exposure, focusing in particular on Facebook.DesignCross-sectional study.SettingAn anonymous semistructured survey was distributed to an Italian hospital-based cohort of adolescents with chronic disease to explore the role of Facebook in their daily life.PatientsWe recruited 212 adolescents (aged between 13 and 24 years) with a diagnosis of inflammatory bowel disease, coeliac disease, diabetes mellitus type 1 and cystic fibrosis.ResultsTwo hundred and seven of the 212 (97.6%) expressed the need of sharing their illness experience with friends, 201 out of 212 (94.8%) usually searched information on the internet to find new therapies and to discover their prognosis. One hundred and forty-nine out of 212 adolescents (70.3%) perceived dependence on their parents as the most negative aspect of having a chronic disease, and 200 out of 212 (94.3%) were looking for friends with the same disease on Facebook. Two hundred and ten out of 212 (99.1%) did not want their doctors or nurse on their social media platforms. During the active disease periods, the time spent with social media increased from an average of 5 to 11 hours.ConclusionsThis descriptive analysis focused on the Facebook impact on chronic disease perception among affected adolescents. It showed that they used to spend an increased amount of time on this platform during disease flare-up and highlighted their wish of keeping doctors and nurses away from their social dimension.

The paper describes the case of streptococcal vulvovaginitis with apparently rectal bleeding.

The term “severe neurologic impairment” (SNI) is used to describe a group of disorders of the central nervous system which arise in childhood, resulting in motor impairment, cognitive impairment and medical complexity. As a result, much assistance is required with activities of daily living. Since these patients are often unable to self-report pain, or they may exhibit uncommon behaviors when suffering, pain manifestations may go unrecognized. In this article, the basic principles of how to approach pain in children with SNI are discussed.

A case of a short girl affected by multiple familial osteochondrosis (MFO) is described. The Author underlines the need of a strict, long term clinical monitoring in all MFO children and in their affected parents because of a significantly increased risk of malignancy.

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Background: Pulmonary arterial hypertension consists in an increase of mean pulmonary arterial pressure (PAPm ≥ 25 mmHg), and may lead to right ventricular failure. Pulmonary arterial hypertension can arise in several disorders, encompassing inflammatory conditions and connective tissue diseases. The occurrence of pulmonary arterial hypertension has recently been reported in monogenic interferonopathies and in systemic lupus erythematosus, highlighting the pathogenic role of type I interferons and paving the way to therapies aimed at inhibiting interferon signaling. Case: We describe a 17-year-old boy with DNase II deficiency, presenting a clinical picture with significant overlap with systemic lupus erythematosus. During treatment with the Janus kinase inhibitor ruxolitinib, he developed pulmonary arterial hypertension, raising the question whether it could represent a sign of insufficient disease control or a drug-related adverse event. The disease even worsened after drug withdrawal, but rapidly improved after starting the drug again at higher dosage. Summary and conclusion: Pulmonary arterial hypertension can complicate type I interferonopathies. We propose that ruxolitinib was beneficial in this case, but the wider role of Janus kinase inhibitors for the treatment of pulmonary arterial hypertension is not clear. For this reason, a strict cardiologic evaluation must be part of the standard care of subjects with interferonopathies, especially when Janus kinase inhibitors are prescribed.

­ A 3-year-old toddler was admitted for a 5-day history of worsening painful limping on his left leg. History was remarkable only for a minor trauma 2 days before the onset of symptoms; the boy fell on his buttocks but was walking normally in the following days. No fever was reported. Pain was also present at night, with no response to oral ibuprofen. On physical examination, the patient refused to stand on his left leg, palpation of the left buttock evoked pain, and exorotation and abduction of the left hip were only moderately limited, without local signs of inflammation such as redness, swelling or skin warming. Blood tests showed elevated erythrocyte sedimentation rate (ESR) (98 mm/hour, normal value 9/L, haemoglobin was 127 g/L and PLT was 430 x 109/L. Creatine kinase values were within the normal range. An X-ray of the pelvis was unremarkable. An ultrasound of the left hip showed a 2 mm articular effusion. Questions Based on the clinical picture and laboratory tests, what is the most likely diagnosis? Perthes disease. Pyomyositis. Septic arthritis. Bone fracture. Leukaemia. What test could confirm the diagnosis? Bone scintigraphy. CT. Bone marrow aspirate. MRI. Intra-articular puncture. What is the mainstay of management of this condition? Wait and see. Surgical excision. Antibiotic course. Antineoplastic treatment. Answers can be found on page 2.

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In semi-arid environments vegetation density and distribution is of considerable importance for the hydrological water balance. A number of hydrological models exploit Leaf Area Index (LAI) maps retrieved by remote sensing as a measure of the vegetation cover, in order to enhance the evaluation of evapotranspiration and interception losses. On the other hand, actual evapotranspiration and vegetation development can be derived through crop growth models, such as AquaCrop, developed by FAO (Food and Agricultural Organization), which allows the simulation of the canopy development of the main field crops. We used MODIS LAI images to calibrate AquaCrop according to the canopy cover development of winter wheat. With this aim we exploited an empirical relationship between LAI and canopy cover. In detail Aquacrop was calibrated with MODIS LAI maps collected between 2008 and 2011, and validated with reference to MODIS LAI maps of 2013–2014 in Rocchetta Sant’Antonio and Sant’Agata, two test sites in the Carapelle watershed, Southern Italy. Results, in terms of evaluation of canopy cover, provided improvements. For example, for Rocchetta Sant’Antonio, the statistical indexes varyfrom r = 0.40, ER = 0.22, RMSE = 17.28 and KGE = 0.31 (using the model without calibration), to r = 0.86, ER = 0.08, RMSE = 6.01 and KGE 0.85 (after calibration).

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A 10-year-old child was referred with bilateral curving and bending of the index fingers noted from birth. His previous history included astigmatism and left knee retraction resulting in flexion deformity since he was 3 years old. Paediatricians should consider distal arthrogryposis type 5D (DA5D) in children with a family history of clino-camptodactyly and articular retraction. His physical examination revealed clubfoot hypoplasia of the index fingers, adducted thumbs and …

(+)-2-Deoxyoryzalexin S (1), the nominal enantiomer of a diterpenoid isolated in Chile from Calceolaria species, was regio- and diastereoselectively synthesized from (+)-podocarpic acid. (+)-2-Deoxyoryzalexin S (1) was characterized also as its acetyl derivative, (+)-2, whose structure was confirmed by X-ray crystallographic analysis. Surprisingly, comparison of the data recorded for (+)-1 and (+)-2 and those reported in the literature for the Calceolaria isolated diterpenoid 1 and its derivative (-)-2 showed some differences, suggesting that the latter do not possess the proposed structures.

The problem of constructing diastereoselectively the C/D ring system of stemarane diterpenes from a bicyclo[2.2.2]octane intermediate was solved resulting in very simple synthesis of (+)-13-stemarene 1. The obtaining of the latter represents also a formal synthesis of (+)-18-deoxystemarin 2. In the key step, the epimeric mixture 10, dissolved in toluene, was converted by the action of TsOH into (+)-stemar-13-en-15-one 28.