Craniofacial Microsomia: Orthodontic Surgical Treatment of Growing Patients.

Craniofacial microsomia covers a set of morphogenetic anomalies that affect structures arising from the first and second brachial arches. Due to the vast phenotypic variation and complexity of the malformation, a global treatment that is coordinated by a multidisciplinary team is imperative. Herein, the authors describe 3 clinical patients and discuss the different therapies used according to the type of microsomia present.

Hospitalizations for asthma exacerbation in Chilean children: A multicenter observational study.

Background: Asthma hospitalization rates in Chilean children have increased in the last 14 years, but little is known about the factors associated with this.

Objective: Describe clinical characteristics of children hospitalized for asthma exacerbation.

Methods: Observational prospective cohort study in 14 hospitals.

Organ and effective doses from paediatric interventional cardiology procedures in Chile.

The aim of this study was to present the results of organ and effective doses for paediatric patients for different types of interventional cardiology procedures for age and weight groups, derived from a patient dosimetry pilot programme carried out in Chile, under the auspices of the International Atomic Energy Agency. Over seven years, a retrospective collection of demographic and patient dose data was obtained: age, gender, weight, height, number of cine series, total number of cine frames, fluoroscopy time, dose-area product (DAP) and cumulative dose at patient entrance reference point. Monte Carlo software was used to calculate organ and effective doses.

Infection Is Associated with Decreased Expression of SLC5A8, a Cancer Suppressor Gene, in Young Children.

infects half of the world's population and causes gastric cancer in a subset of infected adults. Previous blood microarray findings showed that apparently healthy children, persistently infected with have differential gene expression compared to age-matched, non-infected children. SLC5A8, a cancer suppressor gene with decreased expression among infected children, was chosen for further study based on bioinformatics analysis.

Detection of Helicobacter pylori by Real-Time PCR for 16s rRNA in Stools of NonInfected Healthy Children, Using ELISA Antigen Stool Test as the Gold Standard.

Background: We previously detected Helicobacter pylori infection by stool antigen ELISA assay in 33-41% of asymptomatic Chilean children between 2-3 years of age, of which 11-20% had a transient infection and 21-22% a persistent infection. A total of 88% of ELISA-positive samples were also rtPCR positive, while 37/133 (33%) of ELISA-negative stool samples were rtPCR positive. The significance of a ELISA-negative/rtPCR-positive sample requires clarification.

SCATTER DOSE VALUES IN LOWER EXTREMITIES FOR STAFF DURING PAEDIATRIC INTERVENTIONAL CARDIOLOGY PROCEDURES: AN EXPERIMENTAL APPROACH.

The aim of this study was to determine experimentally the scatter dose at the cardiologist's lower extremities in 10 common types of paediatric interventional cardiology procedures and categorised in four age groups of simulated patients, on the basis of measurements taken from characterisation of X-ray systems together with average fluoroscopy time values and the number of cine frames used as references. The highest scattered dose rates recorded during the simulations were 700 and 4000 µSv h-1 for the low fluoroscopy and cine modes, respectively. Scattered dose at cardiologist's lower extremities for the four age groups of simulated patients and procedures ranged from 1 to 28 µSv (aged below 1 y), 6 to 58 µSv (below 5 y), 13 to 155 µSv (below 10 y) and 29 to 375 µSv (below 15 y).

Anteriorly Based Galeo-Pericranial Frontalis Flap: A Novel Application in Secondary Rhinoplasty With Atrophic Skin.

Background: Nasomaxillary dysplasia (Binder syndrome) and cleft are congenital malformations of the midface. Ideally, the nasal deformity is treated from childhood. This approach leads to the need for several surgeries and revisions until full growth is reached.

Patient and staff doses in paediatric interventional cardiology derived from experimental measurements with phantoms.

The aim of this paper was to determine experimentally the entrance surface air kerma (ESAK) and kerma-area product (KAP) levels to patients and scatter doses at the cardiologist's eyes during paediatric interventional cardiology (IC) procedures for Chile, on the basis of measurements taken from X-ray systems characterization for different thicknesses of polymethyl methacrylate, together with the average values of fluoroscopy time and number of cine frames for ten paediatric IC procedures. The range of cumulative ESAK values when the different clinical procedures were simulated was from 2 to 1100 mGy. KAP values ranged from 0.

Administration Time for the First Dose of Antimicrobials in Episodes of Fever and Neutropenia in Children With Cancer.

Background: Early administration of antimicrobial (AM) is relevant in children with cancer, fever and neutropenia (FN). The recommendation is to administer the first dose of AM within the first hour of hospital admission. Our aims were to determine the time from the moment that a child with FN is admitted to the hospital until they receive their first dose of AM and to determine the association with clinical outcomes.

Comparison of two angiographic systems in paediatric interventional cardiology.

The aim of this work was to analyse the radiation dose for patients and staff between X-ray systems, a new biplane with flat-panel detectors (FDs) and a conventional system equipped with image intensifier (II). Entrance surface air kerma (ESAK) and scatter doses were measured on polymethyl methacrylate (PMMA) phantoms of different thicknesses (from 4 to 16 cm). The ESAK values for the different acquisition modes and PMMA thicknesses were higher for the II in comparison with FDs.

Persistent and transient Helicobacter pylori infections in early childhood.

Background: Helicobacter pylori, the main cause of peptic ulcer disease and gastric cancer in adult populations, is generally acquired during the first years of life. Infection can be persistent or transient and bacterial and host factors determining persistence are largely unknown and may prove relevant for future disease.

Methods: Two cohorts of healthy Chilean infants (313 total) were evaluated every 3 months for 18-57 months to determine pathogen- and host-factors associated with persistent and transient infection.

Entrance surface air kerma in X-ray systems for paediatric interventional cardiology: a national survey.

The aims of this work were to report the results of a national survey on entrance surface air kerma (ESAK) values for different phantom thicknesses and operation modes in paediatric interventional cardiology (IC) systems and to compare them with previous values. The national survey also offers suggested investigation levels (ILs) for ESAK in paediatric cardiac procedures. ESAK was measured on phantoms of 4-16 cm thickness of polymethyl methacrylate slabs.

Local patient dose diagnostic reference levels in pediatric interventional cardiology in Chile using age bands and patient weight values.

Purpose: To present the results of a patient dose evaluation program in pediatric cardiology and propose local diagnostic reference levels (DRLs) for different types of procedure and age range, in addition to suggesting approaches to correlate patient dose values with patient weight. This study was the first conducted in Latin America for pediatric interventional cardiology under the auspices of the International Atomic Energy Agency.

Methods: Over three years, the following data regarding demographic and patient dose values were collected: age, gender, weight, height, number of cine series, total number of cine frames, fluoroscopy time (FT), and two dosimetric quantities, dose-area product (DAP) and cumulative dose (CD), at the patient entrance reference point.

Activated STING in a vascular and pulmonary syndrome.

Background: The study of autoinflammatory diseases has uncovered mechanisms underlying cytokine dysregulation and inflammation.

Methods: We analyzed the DNA of an index patient with early-onset systemic inflammation, cutaneous vasculopathy, and pulmonary inflammation. We sequenced a candidate gene, TMEM173, encoding the stimulator of interferon genes (STING), in this patient and in five unrelated children with similar clinical phenotypes.

Laparoscopically harvested omental flap for breast reconstruction in Poland syndrome.

Background/purpose: Poland syndrome (PS) is a congenital anomaly of the thoracic wall characterised by a variable degree of unilateral agenesis of pectoralis major and minor muscles associated with ipsilateral breast, rib and upper-arm anomalies. In female patients, breast reconstruction is particularly challenging because of cranial pectoralis muscle defects and partial or complete breast agenesis. Different techniques have been used to treat the thoracic anomaly, including the positioning of an implant, fat transfer or latissimus dorsi transposition, but in some cases these techniques are not feasible resulting in a poor reconstruction.

Salival fistulae: a rare complication after microtic ear reconstruction.

Parotid fistulae are a very infrequent complication after ear reconstruction in microtia when the local conditions are favorable. We report 2 cases of salival fistulae after microtic ear reconstruction. Timing of the diagnosis is important to decide the treatment.

Retroauricular island flap as an innovation for microtia ear reconstruction.

The use of a local island flap, designed with retroauricular skin, is an excellent, simple, and reproducible innovation to the classic microtia ear reconstruction, allowing adequate coverage of the cartilage framework, specifically of the conchal area, sparing tense skin closure, reducing skin suffering and necrosis, with a better aesthetic result achieving the naturally depressed concha aspect. The flap design and surgical technique is described. This flap was used in 13 consecutive microtia ear reconstructions in our unit, no complications were encountered, and aesthetic results were excellent.

Revisiting human IL-12Rβ1 deficiency: a survey of 141 patients from 30 countries.

Interleukin-12 receptor β1 (IL-12Rβ1) deficiency is the most common form of Mendelian susceptibility to mycobacterial disease (MSMD). We undertook an international survey of 141 patients from 102 kindreds in 30 countries. Among 102 probands, the first infection occurred at a mean age of 2.

Individualized Neoral doses in pediatric renal transplantation.

We propose a model to calculate individualized Neoral doses based on individual oral clearance (CL/F)(i) and AUC((0-12h)) values. The equation proposed by Dr. Ke-Hua Wu (2005), was employed to calculate the CL/F(i) = 28,5 - (1.

Parvovirus B19 may have a role in the pathogenesis of juvenile idiopathic arthritis.

Objective: To determine the prevalence of human parvovirus B19 infection in patients with juvenile idiopathic arthritis (JIA) by detection of specific IgM, IgG, and viral DNA.

Methods: Serum samples of 50 patients with diagnosis of JIA and 39 healthy controls were analyzed by ELISA to detect IgG and IgM anti-B19-specific antibodies. The parvovirus B19 genome was detected by nested polymerase chain reaction (PCR).

Changes in the survival of patients with systemic lupus erythematosus in childhood: 30 years experience in Chile.

The objective of this study was to analyse the survival rate and cause of death in children with systemic lupus erythematosus (SLE) during the past 30 years in Chile. A retrospective analysis was performed between 1969 and 2000 on patients attending pediatric rheumatology centres in Santiago, Chile. Survival and causes of death in 31 children followed from 1969 to 1980 fulfilling the 1982 American College of Rheumatology criteria for SLE and treated with oral steroids were compared with 50 other patients who were treated with oral steroids and an aggressive treatment of IV bolus of cyclophosphamide (38 patients) and azathioprine (12 patients).

Establishment of a pediatric HSCT program in a public hospital in Chile.

Background: In Chile, survival estimates for pediatric patients with cancer are comparable to those in the United States and Western Europe. Approximately 80% of these patients are treated at government-supported centers, and an estimated 65% are cured. We reasoned that cure rates could be further improved if transplantation with hematopoietic stem cells were available for patients with chemotherapy-resistant malignancy.

Comparison of cyclosporine concentrations 2 hours post-dose determined using 3 different methods and trough level in pediatric renal transplantation.

Immunosuppression has been one of the great challenges in pediatric recipients of kidney allografts. Cyclosporine (CsA) has evolved during the past 25 years of transplantation. It requires frequent blood level monitoring because of its narrow therapeutic window and interpatient and intrapatient variability.

Combining the Cutting and Mulliken methods for primary repair of the bilateral cleft lip nose.

Background: Since 1990, primary bilateral cleft nasal reconstruction has been focused on placing the lower lateral cartilages into normal anatomical position. Of the four major techniques in this class, the Cutting (i.e.