Opportunities for digitally-enabled personalization and decision support for pediatric growth hormone therapy.

Smart technologies and connected health are providing opportunities for improved healthcare for chronic conditions. Acceptance by healthcare professionals (HCPs) and patients is crucial for successful implementation. Evidence-based standards, technological infrastructure and regulatory processes are needed to integrate digital tools into clinical practice.

Experience of an interdisciplinary management for pediatric Moyamoya disease: application of a novel Hemispheric Surgical Score.

Purpose: The primary purpose of this study was to develop and implement a novel Hemispheric Surgical Score to guide the treatment of pediatric patients with Moyamoya disease (MMD). Additionally, we aimed to describe a comprehensive flowchart for the evaluation, treatment, and follow-up of these patients and to share our experience with the interdisciplinary management of a large pediatric cohort at a referral pediatric hospital.

Methods: We conducted a retrospective observational study using medical records of patients diagnosed with MMD at the Pediatric Hospital "Prof.

Surviving Sepsis After Burn Campaign.

Introduction: The Surviving Sepsis Campaign was developed to improve outcomes for all patients with sepsis. Despite sepsis being the primary cause of death after thermal injury, burns have always been excluded from the Surviving Sepsis efforts. To improve sepsis outcomes in burn patients, an international group of burn experts developed the Surviving Sepsis After Burn Campaign (SSABC) as a testable guideline to improve burn sepsis outcomes.

"Ping-pong" fracture: An exclusive entity of neonates and infants? A case analysis and literature review.

Background: "Ping-pong" fractures are a type of depressed fracture in which there is no rupture of the inner or outer table of the skull. It is produced by incomplete bone mineralization. Its appearance is frequent during neonatal and infant ages and is extremely rare outside of these age periods.

Giant Supratentorial Brain Tumors in Children: Functional Outcome and Progression-Free Survival Analysis.

Introduction: This study aimed to identify factors affecting progression-free survival (PFS) in pediatric patients with giant supratentorial brain tumors (GSBTs) treated with surgical excision. The secondary aim was to analyze how these same factors affected the functional outcome in the long term.

Methods: We performed a retrospective, analytical, single-center cohort study.

RETINAL ISCHEMIA AFTER FOCAL CEREBRAL ARTERIOPATHY IN A YOUNG GIRL.

Purpose: We present a case of an ischemic retinopathy with severe vision loss secondary to a childhood stroke.

Methods: Case report.

Results: An otherwise healthy 9-year-old girl presented with a 1-day history of impaired gait and speech.

Melanotic neuroectodermal tumor of infancy arising from the skull: report of an unusual case, review of the literature, and a diagnostic approach.

Introduction: Melanotic neuroectodermal tumor of infancy is a rare neoplasm mainly seen in children under 1 year of life. The most common location of the tumor is the maxilla followed by the cranial vault. Surgery is the treatment of choice and outcome mainly depends on extent of resection.

New Simulator for Neuroendoscopy: A Realistic and Attainable Model.

Objective: To present an attainable and realistic model for neuroendoscopic simulation which replicates exercises of tissue biopsy and coagulation and membrane fenestration.

Methods: We presented a stepwise method to create a neuroendoscopic simulation model using bovine brain and membrane units made by a soda cup covered by an amniotic membrane inside an expanded polystyrene spherical container. We used face validation for preliminary evaluation.

Encephalopathy with continuous spike-waves during slow-wave sleep: evolution and prognosis.

Encephalopathy with continuous spike-waves during slow-wave sleep (CSWS) evolves over time, and three stages can be recognized: before the onset of CSWS, during CSWS, and after the CSWS period. Clinical seizures tend to remit spontaneously around puberty. This pattern is independent of the etiological lesion.

Encephalopathy related to Status Epilepticus during slow Sleep: from concepts to terminology.

Five pediatric and adult neurologists with clinical and research interests in Encephalopathy related to Status Epilepticus during slow Sleep (ESES) express their opinions on definition, diagnostic assessment and terminology that may be considered for this condition. The aim of this "debate" is to identify aspects in which there is a shared opinion and areas where there are still controversies in the classification and suggest areas which demand further studies and research.

Aberrant O-glycosylation modulates aggressiveness in neuroblastoma.

Neuroblastoma (NB) is the most common pediatric malignancy diagnosed before the first birthday in which MYCN oncogene amplification is associated with poor prognosis. Although aberrant glycosylation is an important actor in cell biology, little is known about its role in pediatric cancers such as NB. In this work we characterized the glycophenotype and the enzyme expression involved in glycans biosynthesis in five established human NB cell lines and in patient-derived primary tumors with different MYCN status.

Treatment of electrical status epilepticus in sleep: A pooled analysis of 575 cases.

Objective: Epileptic encephalopathy with electrical status epilepticus in sleep (ESES) is a pediatric epilepsy syndrome with sleep-induced epileptic discharges and acquired impairment of cognition or behavior. Treatment of ESES is assumed to improve cognitive outcome. The aim of this study is to create an overview of the current evidence for different treatment regimens in children with ESES syndrome.

Epilepsy of infancy with migrating focal seizures: three patients treated with the ketogenic diet.

We present three patients with epilepsy of infancy with migrating focal seizures treated with the ketogenic diet. Between February 1, 2012 and January 31, 2014, three patients who met the diagnostic criteria for migrating focal seizures in infancy at our department were placed on the ketogenic diet and followed for a minimum of seven months. Two of the three children responded well to the ketogenic diet.

Immunoreactivity of the 14F7 Mab raised against N-Glycolyl GM3 Ganglioside in retinoblastoma tumours.

Introduction: The identification of molecules expressed selectively on the surface of retinoblastoma cells would allow applying targeted therapies. The Ganglioside, N-Glycolyl-GM3 (NeuGc-GM3), is an attractive candidate, as it has been detected in other paediatric neuroectodermic tumours, and it is not expressed in human normal tissues. The 14F7 antibody recognizes specifically the ganglioside NeuGc-GM3.

Epilepsy of infancy with migrating focal seizures: six patients treated with bromide.

Purpose: We present six patients with epilepsy of infancy with migrating focal seizures (EIMFS) and provide a comprehensive evaluation of potassium bromide therapy.

Method: Between February 1, 2007 and July 31, 2012, six patients who met the diagnostic criteria of EIMFS were treated with potassium bromide. Potassium bromide was added to other antiepileptic drugs (AEDs) in doses ranging from 30 to 80 mg/kg/day.

Myoclonic epilepsy in infancy: an electroclinical study and long-term follow-up of 38 patients.

Purpose: Myoclonic epilepsy in infancy (MEI) is characterized by brief generalized myoclonic seizures associated with generalized spike-wave paroxysms without other seizure types occurring in the first 3 years of life in developmentally normal children. In this study we analyze the electroclinical features, treatment, and outcome of 38 patients with MEI.

Methods: A retrospective chart review was conducted in 38 patients followed at the Neurology Department of the Pediatric Hospital Juan P.

Immune response to racotumomab in a child with relapsed neuroblastoma.

Immunotherapy targeting ganglioside antigens is a powerful tool for the treatment of high risk neuroblastoma. However, only treatment with anti-GD2 antibodies has been used in clinical practice and other options may be pursued. We report the use of racotumomab, an anti-idiotype vaccine against N-glycolyl neuraminic acid (NeuGc)- containing gangliosides, eliciting an immune response in a child with relapsed neuroblastoma expressing the NeuGcGM3 ganglioside.

Detection of N-glycolyl GM3 ganglioside in neuroectodermal tumors by immunohistochemistry: an attractive vaccine target for aggressive pediatric cancer.

The N-glycolylated ganglioside NeuGc-GM3 has been described in solid tumors such as breast carcinoma, nonsmall cell lung cancer, and melanoma, but is usually not detected in normal human cells. Our aim was to evaluate the presence of NeuGc-GM3 in pediatric neuroectodermal tumors by immunohistochemistry. Twenty-seven archival cases of neuroblastoma and Ewing sarcoma family of tumors (ESFT) were analyzed.

Childhood absence epilepsy and electroencephalographic focal abnormalities with or without clinical manifestations.

Purpose: We studied the electroclinical features and evolution in patients with childhood absence epilepsy (CAE) associated with electroencephalographic findings similar to those of benign focal epilepsies (BFE) with or without clinical manifestations compatible with these focal idiopathic syndromes.

Methods: Between June 1994 and June 2002, we found 203 (3.6%) patients with typical electroclinical features of CAE among 8285 children with epilepsy.