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The hepatopulmonary syndrome is an infrequent disease in pediatric population, with an unknown incidence in Mexico. The diagnosis is challenge for the clinician due to the presence of many other causes of hypoxemia in children with chronic liver disease. Two patients with Hepatopulmonary Syndrome are presented, showing the variability in both cases and outcome of the disease. First a patient with chronic liver disease secondary to an autoimmune disease (Systemic Lupus Erythematosus) with a torpid evolution, then a patient in which the diagnosis of chronic liver disease was made after the onset of the respiratory symptoms. In both cases the ideal treatment should be hepatic transplantation, however it could not be done because of the low accessibility to this surgical procedure in our country. [ABSTRACT FROM AUTHOR]

Background: There are evidence gaps in the management of pediatric cough, particularly for acute pediatric cough. This study had two aims: to identify therapeutic principles and unmet needs in the treatment of cough in pediatric patients (internationally), and to consider the evidence required to address these unmet needs., Methods: A MEDLINE/PubMed database search was performed to identify articles describing therapeutic principles in the treatment of pediatric cough. An online survey of international pediatric cough experts was conducted, with questions on the definitions, diagnosis, treatment, and unmet needs in pediatric cough management., Results: Cough guidelines have differing definitions of pediatric patients (≤12-18 years), acute pediatric cough (< 2-3 weeks), and chronic pediatric cough (> 4-8 weeks). Similarly, among 18 experts surveyed, definitions varied for pediatric patients (≤10-21 years), acute pediatric cough (< 3-5 days to < 6 weeks), and chronic pediatric cough (> 2-8 weeks). Guidelines generally do not recommend over-the-counter or prescription cough medicines in acute pediatric cough, due to lack of evidence. In the expert survey, participants had differing opinions on which medicines were most suitable for treating acute pediatric cough, and noted that effective treatments are lacking for cough-related pain and sleep disruption. Overall, guidelines and experts agreed that chronic pediatric cough requires diagnostic investigations to identify the underlying cough-causing disease and thereby to guide treatment. There are unmet needs for new effective and safe treatments for acute pediatric cough, and for randomized controlled trials of existing treatments. Safety is a particular concern in this vulnerable patient population. There is also a need for better understanding of the causes, phenotypes, and prevalence of pediatric cough, and how this relates to its diagnosis and treatment., Conclusions: Whereas pediatric cough guidelines largely align with regard to the diagnosis and treatment of chronic cough, there is limited evidence-based guidance for the management of acute cough. There is a need for harmonization of pediatric cough management, and the development of standard guidelines suitable for all regions and patient circumstances., (© 2023. The Author(s).)

Pseudomonas aeruginosa has a high adaptive capacity, favoring the selection of antibiotic-resistant strains, which are currently considered a global health problem. The purpose of this work was to investigate the rate and distribution of extensively drug-resistant (XDR) P. aeruginosa in pediatric patients with cystic fibrosis (CF) with recurrent infections and to distinguish the current efficacy of antibiotics commonly used in eradication therapy at a Mexican institute focused on children. A total of 118 P. aeruginosa isolates from 25 children with CF (2015-2019) underwent molecular identification, antimicrobial sensitivity tests, and Random Amplified Polymorphic DNA genotyping (RAPD-PCR). The bacterial isolates were grouped in 84 RAPD profiles, revealing a cross-infection between two sisters, whose resistance profile remained unchanged for more than 2 years. Furthermore, 77.1% (91/118) and 51.7% (61/118) of isolates showed in vitro susceptibility to ceftazidime and amikacin, respectively, antibiotics often used in eradication therapy at our institution. As well, 42.4% (50/118) were categorized as multi-drug resistant (MDR) and 12.7% (15/118) were XDR. Of these resistant isolates, 84.6% (55/65) were identified from patients with recurrent infections. The high frequency of XDR strains in children with CF should be considered a caution mark, as such resistance patterns are more commonly found in adult patients. Additionally, amikacin may soon prove ineffective. Careful use of available antibiotics is crucial before therapeutic possibilities are reduced and "antibiotic resistance crisis" worsens., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Background: There was a need for a solid asthma guideline in Mexico to update and unify asthma management. Because high-quality asthma guidelines exist worldwide, in which the latest evidence on asthma management is summarized, the ADAPTE approach allows for the development of a national asthma guideline based on evidence from already existing guidelines, adapted to national needs., Objective: To fuse evidence from the best asthma guidelines and adapt it to local needs with the ADAPTE approach., Methods: The Appraisal of Guidelines for Research and Evaluation (AGREE) II asthma guidelines were evaluated by a core group to select 3 primary guidelines. For each step of asthma management, clinical questions were formulated and replied according to (1) evidence in the primary guidelines, (2) safety, (3) Cost, and (4) patient preference. The Guidelines Development Group, composed of a broad range of experts from medical specialties, primary care physicians, and methodologists, adjusted the draft questions and replies in several rounds of a Delphi process and 3 face-to-face meetings, taking into account the reality of the situation in Mexico. We present the results of the pediatric asthma treatment part., Results: Selected primary guidelines are from the British Thoracic Society and Scottish Intercollegiate Guidelines Network (BTS/SIGN), Global Initiative for Asthma (GINA), and Spanish Guidelines on the Management of Asthma (GEMA) 2015, with 2016 updates. Recommendations or suggestions were made for asthma treatment in Mexico. In this article, the detailed analysis of the evidence present in the BTS/SIGN, GINA, and GEMA sections on the (non) pharmacologic treatment of pediatric asthma, education, and devices are presented for 2 age groups: children 5 years or younger and children 6 to 11 years old with asthma., Conclusion: For the pediatric treatment and patient education sections, applying the AGREE II and Delphi methods is useful to develop a scientifically sustained document, adjusted to the Mexican situation, as is the Mexican Guideline on Asthma., (Copyright © 2018 American College of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.)

Background: The need for a national guideline, with a broad basis among specialists and primary care physicians was felt in Mexico, to try unifying asthma management. As several high-quality asthma guidelines exist worldwide, it was decided to select the best three for transculturation., Methods: Following the internationally recommended methodology for guideline transculturation, ADAPTE, a literature search for asthma guidelines, published 1-1-2007 through 31-12-2015 was conducted. AGREE-II evaluations yielded 3/40 most suitable for transculturation. Their compound evidence was fused with local reality, patient preference, cost and safety considerations to draft the guideline document. Subsequently, this was adjusted by physicians from 12 national medical societies in several rounds of a Delphi process and 3 face-to-face meetings to reach the final version., Results: Evidence was fused from British Thoracic Society Asthma Guideline 2014, Global Initiative on Asthma 2015, and Guía Española del Manejo del Asma 2015 (2016 updates included). After 3 Delphi-rounds we developed an evidence-based document taking into account patient characteristics, including age, treatment costs and safety and best locally available medication., Conclusions: In cooperation pulmonologists, allergists, ENT physicians, paediatricians and GPs were able to develop an evidence-based document for the prevention, diagnosis and treatment of asthma and its exacerbations in Mexico.

Objective: To characterize P. aeruginosa strains isolated from bronchoalveolar lavage fluid of cystic fibrosis (CF) patients over a 3 year period., Material and Methods: A prospective follow-up study was carried out in a population of cystic fibrosis patients. The random amplified polymorphic DNA (RAP.D) technique was used to amplify DNA of P. aeruginosa strains isolated from bronchoalveolar lavage fluid samples of five CF patients from the Servicio de Neumología y Cirugía del Tórax del Instituto Nacional de Pediatría (Mexico City Chest Clinic of the National Pediatrics Institute) in Mexico City, between June 1996 and June 2002. Amplification patterns were established for each isolate to accurately identify all strains and to carry out an epidemiological analysis of P. aeruginosa among the selected CF patients., Results: Eighteen different DNA amplification patterns were defined and used to identify each P. aeruginosa strain isolated from the different bronchoalveolar lavage samples. No correlation was observed between the different P. aeruginosa strain genotypes and mucoid or non-mucoid phenotypes, as strains with different phenotypes showed similar amplification patterns. Several strains with different amplification patterns were identified in samples obtained from the same patient, suggesting coinfection with ore than one P. aeruginosa strain. Two siblings with CF shared similargenotypes, suggesting the occurrence of cross- contamination. Similar genotypes of P. aeruginosa strains were isolated throughout the study period., Conclusion: Genotypic characterization of P. aeruginosa strains in CF patients allows more accurate epidemiological analyses of this important host-agent relationship.

Background: Juvenile polyposis (JP) is a frequent cause of lower gastrointestinal bleeding. It is present in 3-4% of the population < 21 years of age and represents 90% of all polyps in childhood. The most common complaints are hematoquezia, abdominal pain, and polyp prolapse., Methods: A descriptive, observational, transversal study of 225 children with histopatologic diagnosis of JP seen at the Department of Gastroenterology and Nutrition of the Instituto Nacional de Pediatría, between January 1985 and December 2000. Variables studied included age of presentation, gender, frequency, clinical manifestations, type, location, and diagnostic and therapeutic methods., Results: Frequency was one polyp per 162 patient seen during the same period (0.61%) and age 2 to 8 years (82%), a total of 235 patients, 120 females and 273 total polyps were found rectum the most common site 82% (224 polyps) followed by sigmoid. Mean distance from anal margin was 6.5 cm. Of all polyps, 82.9% were in the first 10 cm of anal margin. Of all patients, 92.3% had a single polyp and 7.7% had multiple polyps. A baritated study was made in 39 patients (16.6%) with diagnostic efficacy of 74%. No additional baritated studies were needed because of high diagnostic and therapeutic efficacy of the rectosigmoidoscopy., Conclusions: Rectosigmoidoscopy and colonoscopy remain as the diagnosis and therapeutic method in children with JP.

Objectives: To study intra and interobserver variability of two pediatric neumologists in X-ray readings of interstitial lung disease (ILD), to determine the effect of clinical data on the readings, and to evaluate their interpretation in terms of the histopathology diagnosis., Design: Prospective, cross sectional, comparative, blinded., Setting: The Instituto Nacional de Pediatria of Mexico City., Material and Methods: Chest X-rays of 45 ILD pediatric patients with diagnosis confirmed by open lung biopsy were read by the two observers three times at two weeks intervals and in a randomized fashion. The observers were blinded to previous readings of both observers as well as to the diagnosis. The first two readings were done with no clinical information given to the observers, but some was given for the third reading (age of inception, length of evolution, and main symptoms at time of the X-rays). A classification in one of 5 patterns (lineal, reticular, reticulonodular, grounded glass, honeycomb) was established by the observers. Associations of patterns with anatomopathological diagnosis was explored. Weighted kappa was used in the statistical analysis., Results: With one exception, good agreement (Kw 0.57-0.88) was found intra and interobservers., Conclusion: We believe the low variability is the result of the 15 years of shared experience of the two observers participating in this study.

Objective: To assess the frequency and clinical picture of Hepatoportal Sclerosis in a population of Mexican children of the Instituto Nacional de Pediatría, México City., Background: Hepatoportal Sclerosis is a disease of unknown etiology. It's diagnosis is difficult. The main clinical presentation is splenomegaly with or without hematemesis (portal hypertension). Splenoportography and liver histology study are the best procedures for diagnosis and must be performed by experts., Methods: We studied 7/106 children with portal hypertension during a period of 10 years, who were seen at the Instituto Nacional de Pediatría, México city. Inclusion criteria were specific findings of splenoportography and histologic changes in liver biopsy., Results: We found 7/106 children. The main clinical manifestation were splenomegaly and hematemesis. We did not find any previous history of contact with arsenic, vinyl chloride or copper sulfate. In 6/7 children a porto-systemic shunt was performed. Only one received propranolol and sclerotherapy. At the time of this report all children have shown a good clinical course.

Background: Different antibiotics, antagonist H2 and others have been used for elimination and/or eradication of Helicobacter pylori., Aims: Evaluate elimination of Helicobacter pylori with amoxicillin, bismuth subsalicylate and ranitidine; and the improvement of recurrent abdominal pain., Method: 20 children with recurrent abdominal pain associated to gastritis and histologic identification of Helicobacter pylori were studied under a period of 18 months (January 1992 to June 1993), at Instituto Nacional de Pediatría, México, D.F. All children were treated simultaneously with: Amoxicillin, 15 days, plus ranitidine and bismuth subsalicylate for one month., Results: Helicobacter pylori was eliminated in 14 of 20 children studied. All these children had an important improvement of recurrent abdominal pain., Conclusion: Elimination of Helicobacter pylori and clinical improvement was present in 14 of 20 children studied (70%).

The "prune belly" syndrome classically described in males, has also been described in female patients with a variable incidence. Associations with multiple respiratory tract malformations have been reported which together with urinary tract anomalies and renal failure are often the cause of early mortality. We report the case of a female patient with prune belly in association with cystic adenomatoid malformation of the lung and pulmonary sequestration with a favorable clinical evolution following medical and surgical treatment.

We present our experience in the endoscopic management of 421 children with upper gastrointestinal foreign bodies. Only in the 1% of the cases (5 patients) we had complications who needs surgical intervention. We found a 1.5:1 ratio in male/female and the highest prevalence in the 2 to 5 years group. Most common foreign bodies were coins and small metallic objects (59%) followed by small plastic by objects (35%) and seeds, fish bones and meat in 6%, meat in 6%.

A 23-month-old girl with Osler-Weber-Rendu (OWR) disease manifested by hemoptysis died of massive pulmonary hemorrhage. Autopsy showed predominant respiratory tract involvement, but telangiectatic vessels were also present in other sites. Skin lesions were absent. A grandfather had died after bleeding by mouth following physical exertion. This disease seldom appears in children and hemorrhage usually manifests after 30 years of age.