What is the best method for calculating the optimal position of an esophageal pH probe in children?

In assessment of distal esophageal pH, the sensor of the probe should be placed above the upper border of the lower esophageal sphincter. There are several methods to estimate the distance from the nose where the probe should be fixed according to the patient's height. We studied the accuracy of these methods. Data of patients who underwent esophageal monitoring were collected prospectively. The esophageal pH electrode was set with the aid of fluoroscopy in all cases, considering the location recommended by the current guideline. Esophageal probe position and anthropometric data of each patient were recorded. We compared the actual esophageal pH electrode distance from the nose with that estimated by Nowak's, Strobel's, Staiano-Clouse's, and Moreau's formulae and the Great Ormond Street Hospital (GOSH) table. A total of 98 patients were included, with ages ranging from 2 months to 19 years old. The highest success rate (67%) for all age groups was achieved by Nowak's formula (3.2 + 0.2 × height in cm). Considering only children under 3 years old, the GOSH table reached the highest-yet probably overestimated-fraction of adequate predictions. A corrected Staiano and Clouse's formula (4.28 + 0.191 × height in cm) had a slightly lower success rate than Nowak's due to a poorer performance in younger children. In conclusion, Nowak's formula is the most accurate regardless of age. It can help reduce radiation due to systematic fluoroscopy, as well as the subsequent manipulation of the esophageal probe. However, it still leads to pH sensor misplacements in more than one-third of children. In consequence, a confirmatory X-ray is advisable even after using the formula.

A systematic review with meta-analysis of the prevalence of gastroesophageal reflux in congenital diaphragmatic hernia pediatric survivors.

Congenital diaphragmatic hernia survivors are a well-known group at risk for developing gastroesophageal reflux disease that may be particularly long-term severe. The aim of this study is to provide a systematic review of the prevalence of gastroesophageal reflux in infant and children survivors treated for congenital diaphragmatic hernia.Electronic and manual searches were performed with keywords related to congenital diaphragmatic hernia, gastroesophageal reflux disease, and epidemiology terms. Summary estimates of the prevalence were calculated. Effect model was chosen depending on heterogeneity (I2). Factors potentially related with the prevalence, including study quality or the diagnostic strategy followed, were assessed by subgroup and meta-regression analyses. Risk of publication bias was studied by funnel plot analysis and the Egger test.The search yielded 140 articles, 26 of which were included in the analyses and provided 34 estimates of prevalence: 21 in patients aged 12 months or younger, and 13 in older children. The overall prevalence of gastroesophageal reflux disease in infants was 52.7% (95% confidence interval [CI]: 43.2% to 62.1%, I2 = 88.7%) and, in children over 1 year old, 35.1% (95% CI: 25.4% to 45.3%, I2 = 73.5%). Significant clinical and statistical heterogeneity was found. The strategy chosen for gastroesophageal reflux diagnosis influenced the reported prevalence. The only estimate obtained with a systematic use of multichannel intraluminal impedance provided a higher prevalence in both age groups: 83.3% (95% CI: 67.2% to 93.6%) and 61.1% (95% CI: 43.5% to 76.9%) respectively. This last prevalence did not significantly differ from that obtained using only low risk of bias estimates.As a conclusion, gastroesophageal reflux disease is commonly observed after congenital diaphragmatic hernia repair and is almost constantly present in the first months of life. It may be underdiagnosed if systematically esophageal monitoring is not performed. This should be considered when proposing follow-up and management protocols for congenital diaphragmatic hernia survivors.

[Description and study of risk factors for the diagnostic delay of pediatric inflammatory bowel disease]. / Retraso diagnóstico en la enfermedad inflamatoria intestinal pediátrica. Descripción y estudio de los factores de riesgo.

INTRODUCTION: Diagnostic delay of inflammatory bowel disease in children might be responsible for complications and a poor response to treatment. The study of diagnostic delay and its determining factors may help implement corrective measures and improve the prognosis of the disease. PATIENTS AND METHODS: A retrospective study of the information collected from primary care medical records and that from the pediatric gastroenterology service at a tertiary hospital between 2000 and 2012 was carried out on 53 patients: 31 with Crohn's disease, 19 with ulcerative colitis, and 3 with unclassified pediatric inflammatory bowel disease. The main response variable was the interval from the first physician-patient contact to diagnosis. RESULTS: The median time to diagnosis was 12 weeks (interquartile range 5-24). However for 26.3% of the ulcerative colitis cases and 25.8% of the Crohn's disease cases, the interval was longer than 1 year. There was a more marked delay trend in Crohn's disease cases, but it was not statistically significant. None of the evaluated risk factors was associated with a relevant diagnostic delay, although it tended to be longer in younger children. CONCLUSIONS: Whereas the median delay for pediatric inflammatory bowel disease seems to be acceptable, the diagnostic time spans are considerable for a large proportion of children with heterogeneous clinical characteristics. Further research into lost diagnostic opportunities needs to be carried out.

Hemofilia A en un recién nacido pretérmino. Riesgo de sangrado frente a riesgo de desarrollo de inhibidor / Hemophilia A in a preterm infant. Bleeding risk versus inhibitor development risk

La hemofilia A es la coagulopatía hereditaria más importante. Constituye una condición facilitadora de sangrados profundos por un fallo en la hemostasia secundaria. El principal abordaje terapéutico consiste en la terapia sustitutiva con factor VIII, aunque en algunos casos la formación de anticuerpos inhibidores puede dificultar su utilidad a largo plazo. Cada vez se conocen mejor los factores que condicionan el desarrollo de inhibidores, pero todavía no se puede predecir con seguridad la probabilidad que tiene un paciente de desarrollar esta complicación, aunque en algunos trabajos ya se han propuesto fórmulas a tal efecto. Son pocas las referencias que se encuentran en la bibliografía sobre el manejo de la hemofilia en el recién nacido, y todavía menos si se trata de neonatos prematuros. No existe ninguna recomendación o guía al respecto, pero quizás un planteamiento individualizado sea el idóneo, dado que el pronóstico puede cambiar en función del grado de prematuridad, el tipo de mutación, los antecedentes familiares de formación de inhibidores, la exposición a traumatismos y la madurez del resto de la cascada de la coagulación, entre otros factores. Se presenta un caso de un recién nacido de 34 semanas de edad gestacional con diagnóstico de hemofilia A grave, que fue tratado con medidas conservadoras, evitando la administración profiláctica de factor VIII por considerarse de alto riesgo para la aparición de inhibidores y, por tanto, para el fallo de la terapia sustitutiva a largo plazo(AU)

Hemophilia A is the most important inherited coagulation disease. It is a condition predisposing deep bleeding due to a failure in secondary hemostasis. Among the possibilities of therapeutic approach, factor VIII replacement therapy is considered as the mean one. Nevertheless in some cases the formation of inhibitory antibodies may hinder its long-term usefulness. There is increasing knowledge of the factors that influence the development of inhibitors but we are still not able to predict exactly the probability of a patient developing this complication, although some research groups are working on it. There are few references in the literature on the management of hemophilia in the newborn, and even less regarding preterm infants. There is no recommendation or guideline about what attitude is to be taken with preterm infants with hemophilia but perhaps an individualized approach fits the best, since the outcome can change depending on prematurity degree, mutation type, family history of inhibitors formation, trauma exposure and maturity of the rest of the clotting cascade, among others. We present a case of a 34-gestational-week newborn with severe hemophilia A who was managed with conservative steps avoiding prophylactic factor VIII administration, considering a high risk for inhibitor development and therefore, a long term failure of replacement therapy(AU)