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Posterior reversible encephalopathy syndrome (PRES) is a clinico-radiological condition characterized by headache, nausea, vomiting, seizures, and visual disturbances with typical radiological features of symmetrical edema mostly involving the white matter in the occipital regions (1–2). PRES can develop in association with a wide array of clinical conditions, including systemic infections, hypertension, organ transplantation, and immunosuppression (especially with calcineurin inhibitors). Children who are on prolonged steroid therapy or on calcineurin inhibitor therapy in nephrotic syndrome (NS) are at risk of developing PRES (3–4). A 6-year-old Moroccan-boy, followed in his country for nephrotic syndrome, was hospitalized in our unit of pediatrics for severe generalized body edema, proteinuria (18600 mg/day), low serum albumin (0.9 g/L), high serum cholesterol (468 mg/dl). His arterial blood pressure value was 122/77 mmHg. We treated him with steroid intravenous, albumin supplementations and we continued cyclosporine. While his general conditions were improving, on the seventh day of hospitalization he developed headache, vomiting, dizziness, temporal blindness. A non-contrast computerized tomography was performed and it showed symmetrical hypodensities in parieto-occipital regions. His blood pressure was 132/71 mmHg. Then he developed two episodes of generalized tonic clonic convulsion, followed by unconsciousness. Magnetic resonance imaging (MRI) showed hyperintense signal in the parieto-occipital regions, and revealed bilateral cortical and subcortical white matter edema in parieto-occipital lobes The child was treated with antihypertensive medications, diuretic, steroids and immunosuppressant (cyclosporine A), thereafter he got no seizure and regained full consciousness and vision. His blood pressure was kept at normal range and urinary protein excretion gradually decreased. MRI performed two weeks later revealed no abnormality of the brain, which is a usual phenomenon in case of PRES The pathophysiology of PRES remains controversial, and two main hypotheses have been suggested; impaired cerebral autoregulation resulting in increased cerebral blood flow, and endothelial dysfunction with cerebral hypoperfusion (5–6). PRES must be managed carefully and its pathogenic factors should be suspected and recognized as soon as possible in order to properly treat the patient. In hypertension-related and drug-induced PRES, in fact, effective management includes prompt withdraw of offending agent, aggressive control of blood pressure, timely anti-convulsant therapy. In our case, hypertension was undoubtedly an important cause, but we were uncertain whether cyclosporine also played a pathogenic role. PRES should be always considered in the differential diagnosis of a child with idiopathic nephrotic syndrome, headache and visual disturbance.

Anna, a term baby born by Caesarean section, firstborn, Apgar 8/9, born SGA 2.630 g weight; performed in karyotype of Down syndrome suspected ’free trisomy of chromosome 21’, echocardiography and abdominal ultrasound normal, ABR REFER, discharged in 8th day of life with diet of mother’s milk integrated with zero infant formula. Hospitalisation at one month of life due to serious clinical conditions (dehydration, hypovolemic shock, metabolic acidosis, PCR 67,4 mg/L, PCT 4,45 ng/ml, leukocytosis 63870/microL, weight 2447 g); negative blood, liquor, urine and stool cultures; Chest and Abdomen X-ray showed meteoric distension of the intestinal loops. She began antibiotic (Ampicillin+Sulbactam, Netilmicin, Ceftazidime) and immunoglobulin therapy with improvement and reduction of inflammatory markers; feeding with zero infant formula. After the fourth day of hospitalisation spontaneous emission of greenish bloody stools and then liquid stools, hypotonus, metabolic acidosis, bulbous abdomen, fever, increased inflammatory markers and leukocytosis occurred despite antibiotic therapy. New negatives cultures tested, negative parasitological faecal examination, positive faecal calprotectin (702.59 mg/kg); lymphocyte subpopulations and normal immunoglobulins. Anaemia with need of blood transfusion. Therapy replaced with Meropenem and Vancomycin and diet with hydrolyzed infant formula with slight improvements. The appearance of two similar to previous episodes in the following days suggested to treat with antibiotics and immunoglobulins. After gastroenterological consulting, in allergic enterocolitis to milk protein suspect, it replaced diet with hydrolyzed infant formula with amino acids. The following days progressive clinical improvement noted, weight increase, normal inflammatory markers, good conditions and subsequently discharge. Three weeks later, during medical check-up, she appeared in good clinical condition, increase in weight of 650 g

D.R. is born at term of physiological pregnancy by caesarean section. Birth weight was 3520 g, APGAR 7/9. At birth he presented axial hypotonia, hyporeactivity, groan, mild respiratory distress. Started Non Invasive Ventilation for 2 days with good improvement of outcome. At 4 day life, we started antimicrobial therapy and immunoglobulins ev because of elevated inflammatory markers and low platelet counts (PLT 47000/mcl) confirmed at the blood smear. After 2 weeks, on the neck and on the right cheek, finding of erythematous nodules of a few millimetres of diameter. In the days after, the lesions are also extended to the left cheek and became larger up to about 3 cm. At the ultrasound they appeared as ‘delimited echogenic areas of 19 × 14 mm at left, 17 × 12 mm at right whit intrinsic vascularisation and of irregular structure. Laboratory pannels (hepatic, renal, coltural exams, procalcitonin) were negative, ESR 24 mm/h, CRP 23.6 mg/L. About 30 days after, we see a reduction of the lesions in spite of a persistent increase of inflammatory markers. Therefore, a bacterial aetiology appeared ulikely saw that antimicrobial therapy so soon established, had no effects on them. Subcutaneous fat necrosis of the newborn is a relatively rare and transient condition that appeared in the first weeks of life in term infants with a perinatal suffering. This condition is caractherized by single or multiple subcutaneous nodules, isolated or confluent in plaques, erythematous/purplish, sometimes with little depressions, calcifications or necrosis inside; symmetrically distributed on the back, shoulders, buttocks, cheeks and at the root of the limbs; often painful at palpation. These nodules grow for some weeks and completely resolve within few months. Etiopathogenesis is probably linked to ischaemic injury, hypoxia, hypothermia and/or stress damage on the immature adipose tissue in infants with perinatal asphyxia. Maternal hypertension, gestational diabetes, family history of thrombophilia and dyslipidemia are considered risk factors. The diagnosis is clinical; the cutaneous biopsy is of support. Important for diagnosis are also the Ultrasonography and RMN. Hypercalcemia is the most dangerous complication; less frequent consequencies are thrombocytopenia, metabolic disorders (hypertriglyceridemia, hypoglycemia) and atrophic development of the nodular lesions.

Background Neurofibromatosis Type 1 (NF1), a autosomal dominant disorder, which mainly involves Skin and nervous system: 15% of children with NF1 develops optic pathway gliomas (OPG), typically Astrocytomas pilocytic low grade, usually in the first decade of life. In one third of cases OPG occur with proptosis, visual impairment, early puberty or other disorders of hypothalamus-pituitary axis. According to current guidelines, children with NF1 should be screened frequently for visual examination and in case of visual impairment, brain MRI with the orbits analysis should performed. The current SIOP-LGG 2004 recommendations, in all cases of no-symptomatic, no-evolving glioma, provide clinical observation with close monitoring; chemotherapy is indicated only in presence of clinical signs and symptoms, or neuroimaging of progression. In the optic nerve tumours, surgery is reserved for patients with blindness, severe proptosis; chiasmatic tumours need surgery in case of exophytic tumours, with cystic component causing severe hydrocephalus or brain compression. patients and methods This is an observational study of 14 cases of OPG (5 males and 9 females) in patients with NF1, diagnosed from 1999 to 2015 c/o Paediatric Oncology Unit of our Department. We evaluated the clinical and radiological course and response to treatment of OPG. Results OPG were located exclusively to the optic nerves level in 43% of patients; 38% presented hypothalamus-optical-chiasmatic localization and 19% had chiasm or optic tracts involvment. The 57% of patients needed of chemotherapy according to the Protocol for Low Grade Glioma, cause of worsening of visual acuity, exophthalmoses or rapid increase of mass-size. The average age of chemotherapy start was 4.6 years (range 1.9 to 7 years). In only two cases there was marked reduction in the size of glioma, while in other cases the radiological picture was confirmed stationary at the stop-Therapy. Chemotherapy has allowed to stabilise the visual impairment, improving in 50% of cases the visual field. Conclusions Our data, accordingly with literature, confirmed that chemotherapeutic treatment for OPG is still unsatisfactory in most cases.