Cardiac catheterization in children with pulmonary hypertensive vascular disease: consensus statement from the Pulmonary Vascular Research Institute, Pediatric and Congenital Heart Disease Task Forces.

Cardiac catheterization is important in the diagnosis and risk stratification of pulmonary hypertensive vascular disease (PHVD) in children. Acute vasoreactivity testing provides key information about management, prognosis, therapeutic strategies, and efficacy. Data obtained at cardiac catheterization continue to play an important role in determining the surgical options for children with congenital heart disease and clinical evidence of increased pulmonary vascular resistance. The Pediatric and Congenital Heart Disease Task Forces of the Pulmonary Vascular Research Institute met to develop a consensus statement regarding indications for, conduct of, acute vasoreactivity testing with, and pitfalls and risks of cardiac catheterization in children with PHVD. This document contains the essentials of those discussions to provide a rationale for the hemodynamic assessment by cardiac catheterization of children with PHVD.

Saudi Guidelines on the Diagnosis and Treatment of Pulmonary Hypertension: Pulmonary hypertension in children.

Pulmonary hypertension (PH) is relatively uncommon in children. Pulmonary arterial hypertension (PAH) in pediatric comprises a wide spectrum of diseases, from a transient neonatal condition to a progressive disease associated with morbidity and mortality. Most common PAH in pediatric are idiopathic (IPAH) or PAH associated with congenital heart disease (PAH-CHD), while other associated conditions, such as connective tissue disease (CTD), are less common in pediatrics. Despite better understanding of PH and the availability of new medications during recent decades; the diagnosis, investigation and choice of therapy remain a challenge in children, as evidence-based recommendations depend mainly on adult studies. In this review, we provide a detailed discussion about the distinctive features of PAH in pediatric, mainly emphacysing on classification and diagnostic algorithm.

Repair of congenital heart disease with associated pulmonary hypertension in children: what are the minimal investigative procedures? Consensus statement from the Congenital Heart Disease and Pediatric Task Forces, Pulmonary Vascular Research Institute (PVRI).

Standardization of the diagnostic routine for children with congenital heart disease associated with pulmonary arterial hypertension (PAH-CHD) is crucial, in particular since inappropriate assignment to repair of the cardiac lesions (e.g., surgical repair in patients with elevated pulmonary vascular resistance) may be detrimental and associated with poor outcomes. Thus, members of the Congenital Heart Disease and Pediatric Task Forces of the Pulmonary Vascular Research Institute decided to conduct a survey aimed at collecting expert opinion from different institutions in several countries, covering many aspects of the management of PAH-CHD, from clinical recognition to noninvasive and invasive diagnostic procedures and immediate postoperative support. In privileged communities, the vast majority of children with congenital cardiac shunts are now treated early in life, on the basis of noninvasive diagnostic evaluation, and have an uneventful postoperative course, with no residual PAH. However, a small percentage of patients (older at presentation, with extracardiac syndromes or absence of clinical features of increased pulmonary blood flow, thus suggesting elevated pulmonary vascular resistance) remain at a higher risk of complications and unfavorable outcomes. These patients need a more sophisticated diagnostic approach, including invasive procedures. The authors emphasize that decision making regarding operability is based not only on cardiac catheterization data but also on the complete diagnostic picture, which includes the clinical history, physical examination, and all aspects of noninvasive evaluation.

Repair of congenital heart disease with associated pulmonaryhypertension in children: what are the minimal investigativeprocedures? Consensus statement from the Congenital HeartDisease and Pediatric Task Forces, Pulmonary VascularResearch Institute (PVRI)

Standardization of the diagnostic routine for children with congenital heart disease associatedwith pulmonary arterial hypertension (PAH-CHD) is crucial, in particular since inappropriate assignmentto repair of the cardiac lesions (e.g., surgical repair in patients with elevated pulmonary vascular resistance)may be detrimental and associated with poor outcomes. Thus, members of the Congenital HeartDisease and Pediatric Task Forces of the Pulmonary Vascular Research Institute decided to conduct asurvey aimed at collecting expert opinion from different institutions in several countries, covering manyaspects of the management of PAH-CHD, from clinical recognition to noninvasive and invasive diagnosticprocedures and immediate postoperative support. In privileged communities, the vast majority of childrenwith congenital cardiac shunts are now treated early in life, on the basis of noninvasive diagnostic evaluation,and have an uneventful postoperative course, with no residual PAH. However, a small percentageof patients (older at presentation, with extracardiac syndromes or absence of clinical features of increasedpulmonary blood flow, thus suggesting elevated pulmonary vascular resistance) remain at a higher risk ofcomplications and unfavorable outcomes. These patients need a more sophisticated diagnostic approach,including invasive procedures. The authors emphasize that decision making regarding operability is basednot only on cardiac catheterization data but also on the complete diagnostic picture, which includes theclinical history, physical examination, and all aspects of noninvasive evaluation.

The Saudi initiative for asthma - 2012 update: Guidelines for the diagnosis and management of asthma in adults and children.

This an updated guidelines for the diagnosis and management of asthma, developed by the Saudi Initiative for Asthma (SINA) group, a subsidiary of the Saudi Thoracic Society. The main objective of SINA is to have updated guidelines, which are simple to understand and easy to use by non-asthma specialists, including primary care and general practice physicians. This new version includes updates of acute and chronic asthma management, with more emphasis on the use of Asthma Control Test in the management of asthma, and a new section on "difficult-to-treat asthma." Further, the section on asthma in children was re-written to cover different aspects in this age group. The SINA panel is a group of Saudi experts with well-respected academic backgrounds and experience in the field of asthma. The guidelines are formatted based on the available evidence, local literature, and the current situation in Saudi Arabia. There was an emphasis on patient-doctor partnership in the management that also includes a self-management plan. The approach adopted by the SINA group is mainly based on disease control as it is the ultimate goal of treatment.

Mutations in axonemal dynein assembly factor DNAAF3 cause primary ciliary dyskinesia.

Primary ciliary dyskinesia most often arises from loss of the dynein motors that power ciliary beating. Here we show that DNAAF3 (also known as PF22), a previously uncharacterized protein, is essential for the preassembly of dyneins into complexes before their transport into cilia. We identified loss-of-function mutations in the human DNAAF3 gene in individuals from families with situs inversus and defects in the assembly of inner and outer dynein arms. Knockdown of dnaaf3 in zebrafish likewise disrupts dynein arm assembly and ciliary motility, causing primary ciliary dyskinesia phenotypes that include hydrocephalus and laterality malformations. Chlamydomonas reinhardtii PF22 is exclusively cytoplasmic, and a PF22-null mutant cannot assemble any outer and some inner dynein arms. Altered abundance of dynein subunits in mutant cytoplasm suggests that DNAAF3 (PF22) acts at a similar stage as other preassembly proteins, for example, DNAAF2 (also known as PF13 or KTU) and DNAAF1 (also known as ODA7 or LRRC50), in the dynein preassembly pathway. These results support the existence of a conserved, multistep pathway for the cytoplasmic formation of assembly competent ciliary dynein complexes.

The Saudi Initiative for Asthma.

The Saudi Initiative for Asthma (SINA) provides up-to-date guidelines for healthcare workers managing patients with asthma. SINA was developed by a panel of Saudi experts with respectable academic backgrounds and long-standing experience in the field. SINA is founded on the latest available evidence, local literature, and knowledge of the current setting in Saudi Arabia. Emphasis is placed on understanding the epidemiology, pathophysiology, medications, and clinical presentation. SINA elaborates on the development of patient-doctor partnership, self-management, and control of precipitating factors. Approaches to asthma treatment in SINA are based on disease control by the utilization of Asthma Control Test for the initiation and adjustment of asthma treatment. This guideline is established for the treatment of asthma in both children and adults, with special attention to children 5 years and younger. It is expected that the implementation of these guidelines for treating asthma will lead to better asthma control and decrease patient utilization of the health care system.

Overview of bronchiolitis.

Respiratory syncytial virus RSV, a nonsegmented, single stranded ribonucleic acid virus, infects one-half of all infants within the first year of life. Respiratory syncytial virus possesses pathogenetic qualities that may be attributed to the interplay of viral and host-specific factors including virus strains of different virulence, size of the inoculum, family history of asthma or airway hyper-reactivity and immunologic anomalies of the host. Inflammatory cell recruitment and activation occur in response to RSV infection of epithelial cells. Epithelial cells initiate the inflammatory response to RSV by elaborating a wide variety of cytokines and chemokines that trigger further inflammatory responses. Treatment of RSV in infants with bronchiolitis is complicated due to the multifactorial nature of this infection. Treatment of RSV bronchiolitis rests primarily on supportive care with oxygen and fluid management. Other therapies commonly used include bronchodilators, corticosteroids and ribavirin, where considered appropriate. Although oxygen administration and judicious fluid replacement are the only interventions proved to be of reliable benefit to infants with bronchiolitis, newer studies support a role for adjunctive therapies aimed at relieving airway obstruction, especially when administered very early in the course of the illness or given to infants with more severe disease.