Hypoxic Ischemic Encephalopathy in Units Reporting to the Ibero-American Society of Neonatology Network: Prevalence and Mortality.

Introduction: Hypoxic ischemic encephalopathy is a neurological condition occurring immediately after birth following a perinatal asphytic episode. Therapeutic hypothermia is a safe and effective intervention to reduce mortality and major disability in survivors. In Latin America, perinatal asphyxia is a major problem, but no data are available characterizing its current situation in the region or the impact of hypoxic ischemic encephalopathy on its management.

Abdominal complications in patients with a ventriculoperitoneal shunt: proposal for management recommendations from a single pediatric tertiary center.

Purpose: The aim of this study was to describe our experience in the management of patients with ventriculoperitoneal shunt-related complications (abdominal pseudocyst, bowel-related complications, and ascites) and to propose treatment recommendations.

Material And Methods: A retrospective descriptive study was conducted in patients with ventriculoperitoneal shunt-related abdominal complications seen between 2009 and 2019 at a tertiary-care pediatric center. Patients were classified according to their diagnosis into the following: group A (abdominal pseudocyst), group B (bowel-related complications), and group C (ascites).

Outcomes of event-free survival in patients with Wilms tumor undergoing preoperative chemotherapy. Analysis of lymph-node yield in a single-center cohort.

Introduction: The clinical value of lymph node sampling in Wilms tumor (WT) lies in its ability to accurately determine lymph node (LN) involvement. LN yield (LNY) is used as a valuable tool to measure LN retrieval, and a minimum of 6 LNs is one of the current recommendations. In patients who are managed with the SIOP strategy, preoperative chemotherapy decreases the retrieval of LN during surgery resulting in lower LNY values.

Skeletal dysplasias in Latin America.

Skeletal dysplasias (SD) are disturbances in growth due to defects intrinsic to the bone and/or cartilage, usually affecting multiple bones and having a progressive character. In this article, we review the state of clinical and research SD resources available in Latin America, including three specific countries (Brazil, Argentina, and Chile), that have established multidisciplinary clinics for the care of these patients. From the epidemiological point of view, the SD prevalence of 3.

Growth in achondroplasia, from birth to adulthood, analysed by the JPA-2 model.

Objectives: In general population, there are three phases in the human growth curve: infancy, childhood and puberty, with different main factors involved in their regulation and mathematical models to fit them. Achondroplasia children experience a fast decreasing growth during infancy and an "adolescent growth spurt"; however, there are no longitudinal studies that cover the analysis of the whole post-natal growth. Here we analyse the whole growth curve from infancy to adulthood applying the JPA-2 mathematical model.

Frequency of CYP3A5 Genetic Polymorphisms and Tacrolimus Pharmacokinetics in Pediatric Liver Transplantation.

The evidence available in the pediatric population is limited for making clinical decisions regarding the optimization of tacrolimus (TAC) in pharmacotherapy. The objective of this study was to estimate the frequency of CYP3A5 genetic polymorphisms and their relationship with tacrolimus requirements in the pediatric population. This was a longitudinal cohort study with a two-year follow-up of 77 patients under 18 years old who underwent a liver transplant during the period 2009-2012 at the J.

Long-term urological assessment and management of cloaca patients: A single tertiary institution experience.

Purpose: Preserving renal function and achieving urinary continence are the most important urological goals in the management of cloaca patients. Many prognostic factors have been described, such as the length of the common channel (CC) and urethra, the presence of spinal dysraphism, and associated urological anomalies. The aim of this study was to analyze urinary continence status and need for reconstructive procedures and their correlation with urological risk factors in a series with a long-term follow-up.

Variable pulmonary manifestations in Chitayat syndrome: Six additional affected individuals.

Hand hyperphalangism leading to shortened index fingers with ulnar deviation, hallux valgus, mild facial dysmorphism and respiratory compromise requiring assisted ventilation are the key features of Chitayat syndrome. This condition results from the recurrent heterozygous missense variant NM_006494.2:c.

Rufinamide as add-on therapy in children with epileptic encephalopathies other than Lennox-Gastaut syndrome: A study of 34 patients.

Objective: Here, we present a multicenter series of patients with developmental and epileptic encephalopathies (DEE) and related electroclinical patterns (REP) other than Lennox-Gastaut syndrome (LGS) who were treated with rufinamide as add-on therapy.

Methods: Medical records of 34 patients with DEE and REP other than LGS treated with add-on rufinamide seen at four pediatric neurology centers in Argentina between May 2014 and March 2019 were retrospectively analyzed.

Results: We evaluated 34 patients (18 males, 16 females), aged between 2 and 15 years with a mean and median age of 6 and 8 years, respectively.

Dravet syndrome: Treatment options and management of prolonged seizures.

Over time, with careful delineation of Dravet syndrome, we have gained experience in treatments most likely to lead to improvement in seizures, as well as those that should be avoided. Sodium valproate, clobazam, stiripentol, and topiramate are all medications that may lead to benefit, as well as the ketogenic diet. Bromides may be utilized in resistant cases.

Online Learning and Residents' Acquisition of Mechanical Ventilation Knowledge: Sequencing Matters.

Objective: Rapid advancements in medicine and changing standards in medical education require new, efficient educational strategies. We investigated whether an online intervention could increase residents' knowledge and improve knowledge retention in mechanical ventilation when compared with a clinical rotation and whether the timing of intervention had an impact on overall knowledge gains.

Design: A prospective, interventional crossover study conducted from October 2015 to December 2017.

Height growth velocity during infancy and childhood in achondroplasia.

There is a lack of knowledge about longitudinal growth during childhood in achondroplasia. We report patterns of linear growth and height growth velocity references. The sample consisted of 84 children, 41 girls and 43 boys.

FGFR3-related hypochondroplasia: longitudinal growth in 57 children with the p.Asn540Lys mutation.

Background Children with hypochondroplasia (HCH), who have FGFR3 mutations c.1620C>A or c.1620C>G (p.

Laparoscopic total nephrectomy for Wilms tumor: Towards new standards of care.

Introduction: Laparoscopic total nephrectomy (LN) in malignant pediatric tumors remains controversial. For selected patients undergoing pre-operative chemotherapy in referral centers, LN has so far shown comparable results to the standard open technique.

Purpose: To describe the inclusion criteria and preliminary results of laparoscopic nephrectomies (LN) for the treatment of unilateral Wilms tumors (WT).

Growth velocity and biological variables during puberty in achondroplasia.

Background: Achondroplasia is the most common form of inherited disproportionate short stature. Cross-sectional design studies of height show that, during childhood, height standard deviation scores (SDS) declines steadily and reaches a mean adult height at -6.42 and -6.

Leg length, sitting height, and body proportions references for achondroplasia: New tools for monitoring growth.

Achondroplasia is the most common form of inherited disproportionate short stature. We report leg length, sitting height, and body proportion curves for achondroplasia. Seven centile format of sitting height, leg length, sitting height/leg length ratio, sitting height/height ratio, and head circumference/height ratio were estimated by the LMS method.

Intraneural perineuriomas: diagnostic value of magnetic resonance neurography.

Intraneural perineurioma (IP) is an under-recognized hypertrophic peripheral nerve tumor. It affects young patients involving frequently the sciatic nerve and its branches and presents with a progressive, painless and predominantly motor deficit. Magnetic resonance neurography (MRN) is a useful tool to localize the lesion, evaluate its extension, and discriminate between different etiologies.

Growth references for height, weight, and head circumference for Argentine children with achondroplasia.

Unlabelled: In order to prepare growth references for height, weight, and head circumference for Argentine children with Achondroplasia, 228 children (114 boys) aged 0-18 years attending the Growth Clinic at Hospital Garrahan were measured between 1992 and 2009. Centiles were calculated by LMS, a method for summarizing growth data which adjusts for skewness. Curves for centiles are obtained using the formula: [Formula: see text], where Z (α) is the normal equivalent deviate for tail area α; C100α is the weight or height centile corresponding to Z (α), t is age in years, and L(t) is (skewness)(t), M(t) is median, S (t) is coefficient variation and C(100α) (t) indicates the corresponding values of each curve at age t.

Delayed diagnosis and management of injuries involving the distal radioulnar joint and distal ulna in the pediatric population: recognition and conduct.

Introduction: Distal radius fractures associated with ligament or distal epiphyseal injuries of the ulna occur less frequently in skeletally immature patients. The underestimation of the mechanisms of injury leads to conservative management of this type of fracture, resulting in unsatisfactory reductions and loss of range of motion.

Materials And Methods: Between January 2000 and March 2008, ten cases that were referred to our institution from other centers were studied.

Growth in post-viral chronic lung disease.

Introduction: Acute lower respiratory infection due to adenovirus is an important cause of chronic lung disease (CLD) in infants. The objectives of this paper is to describe growth in the height and weight of children with post-viral chronic lung disease (PVCLD) and to relate it with associated variables (invasive mechanical ventilation, chronic hypercapnia, oxygen therapy at home and steroid treatment).

Materials And Methods: Ninety-nine patients with diagnosis of PVCLD with one or more years of follow-up were included in the study.

Problems associated with the apnea test in the diagnosis of brain death.

Background: Brain death is the absence of all cortical functions, including the brainstem. The apnea test (AT) is a necessary requisite to complete this diagnosis. Anecdotal reports describing hypotension and acidosis due to apnea test have been reported.