Recommendations for the prevention and diagnosis of asthma in children: Evidence from international guidelines adapted for Mexico.

BACKGROUND: With the availability of high-quality asthma guidelines worldwide, one possible approach of developing a valid guideline, without re-working the evidence, already analysed by major guidelines, is the ADAPTE approach, as was used for the development of National Guidelines on asthma. METHODS: The guidelines development group (GDG) covered a broad range of experts from medical specialities, primary care physicians and methodologists. The core group of the GDG searched the literature for asthma guidelines 2005 onward, and analysed the 11 best guidelines with AGREE-II to select three mother guidelines. Key clinical questions were formulated covering each step of the asthma management. RESULTS: The selected mother guidelines are British Thoracic Society (BTS), GINA and GEMA 2015. Responses to the questions were formulated according to the evidence in the mother guidelines. Recommendations or suggestions were made for asthma treatment in Mexico by the core group, and adjusted during several rounds of a Delphi process, taking into account: 1. Evidence; 2. Safety; 3. Cost; 4. Patient preference - all these set against the background of the local reality. Here the detailed analysis of the evidence present in BTS/GINA/GEMA sections on prevention and diagnosis in paediatric asthma are presented for three age-groups: children with asthma ≤5 years, 6-11 years and ≥12 years. CONCLUSIONS: For the prevention and diagnosis sections, applying the AGREE-II method is useful to develop a scientifically-sustained document, adjusted to the local reality per country, as is the Mexican Guideline on Asthma.

Classification and Assessment of the Patelar Reflex Response through Biomechanical Measures.

Clinical evaluation of the patellar reflex is one of the most frequent diagnostic methods used by physicians and medical specialists. However, this test is usually elicited and diagnosed manually. In this work, we develop a device specifically designed to induce the patellar reflex and measure the angle and angular velocity of the leg during the course of the reflex test. We have recorded the response of 106 volunteers with the aim of finding a recognizable pattern in the responses that can allow us to classify each reflex according to the scale of the National Institute of Neurological Disorders and Stroke (NINDS). In order to elicit the patellar reflex, a hammer is attached to a specially designed pendulum, with a controlled impact force. All volunteer test subjects sit at a specific height, performing the Jendrassik maneuver during the test, and the medical staff evaluates the response in accordance with the NINDS scale. The data acquisition system is integrated by using a tapping sensor, an inertial measurement unit, a control unit, and a graphical user interface (GUI). The GUI displays the sensor behavior in real time. The sample rate is 5 kHz, and the control unit is configured for a continuous sample mode. The measured signals are processed and filtered to reduce high-frequency noise and digitally stored. After analyzing the signals, several domain-specific features are proposed to allow us to differentiate between various NINDS groups using machine learning classifiers. The results show that it is possible to automatically classify the patellar reflex into a NINDS scale using the proposed biomechanical measurements and features.

A modified method for the repair of esophageal atresia with tracheoesophageal fistula.

Forty patients with esophageal atresia with tracheoesophageal fistula were operated upon during a period of 2 years, 39 of them with the end-to-side anastomosis technique. According to Waterston's classification, the survival rate was 91 percent for Group A (11 patients), 60 percent for Group B (15 patients), and 14 percent for Group C (14 patients). A prospective study carried out with the first 30 patients showed that the partial removal of the mucosa of the lower esophagus, just distal to its junction with the trachea, reduced significantly the incidence of recanalization of the tracheoesophageal fistula.

Experimental antireflux intestinal valve.

A reflux-preventing valve was obtained by invaginating a 3.5 to 4.5 cm segment of small bowel into the distal lumen, after removing the seromuscular layers at both ends of the segment of bowel. Antiperistaltic pressure resistance, measured at regular intervals during 6 months, showed valves with competence equal or superior to the ileocecal valve. The blood supply to the intussuscepted segment must be carefully preserved.

Performing procrustes discriminant analysis with HOLMES.

Program HOLMES devised by target factor analysis has been updated for performing procrustes discriminant analysis (PDA). Computational details are outlined. The equivalence between PDA and partial least squares-discriminant analysis (PLS-DA) is established. Application of the PDA is illustrated by two case studies taken from literature.

Hemithyroid agenesis associated with a cervical thymic cyst.

A case is presented of a 7-yr-old boy with left hemithyroid agenesis associated with cervical thymic cyst. No left parathyroid glands were found. The diagnosis was established after surgical excision and histologic examination. Clinical and embryological implications of this condition are briefly discussed. No similar case has been found in the literature.

Bilateral multifocal Wilms' tumor.

A three-year-old boy who presented with symptoms of peritonitis was found to have four Wilms' tumors affecting both kidneys. Individual enucleation of three tumors in the right kidney plus left lower nephrectomy were performed. Chemotherapy was administered for one year. The diagnosis of Wilms' tumor was confirmed on each specimen by the histologic studies. The child remains asymptomatic and developing normally six years after the initial surgical treatment. Bilateral partial nephrectomies is the most conservative of the surgical treatments available for bilateral Wilms' tumor.

Hepatic artery aneurysm in a child.

The present report refers to a child hospitalized because of nonspecific symptomatology, who was found to have aneurysms of both hepatic arteries. A year after simple proximal ligature of both hepatic arteries, the child remains symptom-free and developing normally. Proximal ligation appears to be a simple and effective technique for treating hepatic artery aneurysm in children in preference to direct approach of the vascular pathology.

Ureteral triplication and duplicated opposite kidney with refluxing ureterocele.

A case is reported of ureteral triplication with ectopia of two of the ureters and contralateral duplication with a ureterocele. This patient is the youngest that we have found reported with this type of anomaly and the only one presenting with abdominal distention and an intact but refluxing ureterocele.

[Cognitive aspects in girls with fragile X syndrome]. / Aspectos cognitivos en niñas con sindrome X fragil.

INTRODUCTION AND AIMS: Fragile X syndrome (FXS) is the first cause of intellective dysfunction due to hereditary reasons, but above all it is a multisystemic pathology, in which the cognitive behavioural phenotype is going to mark the child's entire school and social life. An early diagnosis is fundamental for proper genetic counselling and for the pedagogical approach. In girls, this diagnosis is hindered by a poorer knowledge of the semiology and because of the variability of the symptoms. Recognising the most significant clinical signs that suggest a diagnosis during early childhood is fundamental. DEVELOPMENT: An analysis of the literature offered us very few reports of girls affected by FXS and most of them are to be found in publications about genetics. There is often no clear separation between adulthood and childhood or between permutation and complete mutation, and extreme shyness and low self esteem are the most commonly reported data. Intellective capacity is normal in 40% of those affected by complete mutation; the pragmatic aspects of language and difficulties at school that can take on symptoms of non verbal learning disorder are the most significant data at school age; the incidence that the number of CGG repetitions, the degree of methylation and the FMR protein rate can have on both the symptomatology and the intensity with which they appear do not offer any homogeneous data; the attitude of the school and familial environment is a factor that has recently been considered to be of great importance in the maintenance or improvement of behavioural aspects. CONCLUSIONS: Although the discovery of the FMR1 gene provided us with a greater understanding of the symptomatology of FXS in girls, the scarcer knowledge available about its manifestations means that we can find ourselves before the problem of possibly mistaking it for learning disorders. The greater variability of its clinical symptoms and the shortage of studies that have appeared in publications on paediatrics and neuropaediatrics may be the underlying reason behind this lack of knowledge. Spanish language publications practically ignore cases of girls with FXS.

[Aspects of cognition and language in children with fragile X syndrome]. / Aspectos cognitivos y del lenguaje de niños con síndrome X frágil.

INTRODUCTION: Fragile X syndrome, which is produced by mutation of a gene in the X chromosome, is the most frequent cause of hereditary mental retardation. The multisystemic alterations of the disorder are due to the inhibition of the expression of the FMR1 gene and to the lack or absence of FMRP protein. Mental retardation and autistic spectrum constitute the most serious manifestations of the syndrome, but there are numerous neuropsychological disorders that make up the cognitive behavioural (CB) phenotype of patients, and the number of clinical manifestations they are going to present is also high. AIMS: The aim of the study was to evaluate the parameters that can contribute to the elaboration of a set of generally agreed guidelines that include early diagnosis and the indispensable genetic counselling, as well as a multidisciplinary intervention that contemplates, in a global manner, the medical and educational needs of those affected. METHODOLOGY: The method used to conduct the study involved an analysis of the early manifestations of the disease and the neuropsychological aspects of those affected, by means of a study protocol that includes biological and pedagogical data together with batteries of standard tests. RESULTS AND CONCLUSIONS: Preliminary results confront us with the delay in diagnosis and in genetic counselling because the CB phenotype, in which language disorders were the most constant element, is not taken as being an early sign of the clinical manifestations or as a serious interference factor in the cognitive aspects in the progress of the disease.

[Pyeloureteral stenosis secondary to Schoenlein-Henoch purpura]. / Estenosis pieloureteral secundaria a púrpura de Schönlein-Henoch.

We present an 8-year-old male suffering from Schönlein-Henoch purpura with multisystemic clinical manifestations, which developed as a complication a pyeloureteral stenosis secondary to vasculitis. He was operated on and a ureteropyeloanastomosis was carried out according to the Anderson-Hynes technique with good results. We discuss the etiology, diagnostic methods and therapeutic possibilities. It is the eighth case in the literature requiring surgical correction as a result of this exceptional complication.

[Intestinal obstruction caused by milk curds in newborns]. / Obstrucción intestinal por tapón de leche en neonatos.

Intestinal obstruction due to milk curds syndrome may present with a clinical picture and radiological findings which suggest the correct diagnosis. This type of intestinal obstruction usually affects neonates, previously healthy, with concentrated formula feeding. Surgical treatment may be avoided in some cases by the administration of Gastrografin enemas. Two patients with milk curd obstruction treatment in our Hospital, are presented.

[Tendon and/or nerve lesions of the hand in childhood]. / Lesiones tendinosas y/o nerviosas de la mano en la infancia.

We have reviewed 50 consecutive children treated during the last five years of injuries of nerves and tendons of the hand, wrist and forearm. Seventy six per cent of these injuries affected the hand and/or fingers. The rest were located in the wrist or forearm.

[Modified Kasai technique for the treatment of biliary atresia]. / Una modificación de la técnica de Kasai para el tratamiento de la atresia biliar.

Kasai's procedure has changed significantly the prognosis of biliary atresia. However, ascending cholangitis is a relatively frequent postoperative complication with high morbidity and mortality rates. We have developed a modification of that technic consisting in the isolation of a segment of jejunum-with its blood supply carefully preserved, after fashioning an antireflux valve in its distal end. This segment of bowel, less than 10 cm. in length, is anastomosed to the porta hepatis proximally and to the duodenum distally. Using this technic, we have operated upon three infants with biliary atresia and a child with extended rabdosarcoma of the biliary tree. After a follow up of 10 to 17 months with an average of 12.5 months, all children remain free of cholangitis and two of them are anicteric.

[New aspects of gallbladder pathology in children]. / Nuevos aspectos de la patologia de la vesicula biliar en niños.

We have reviewed in a retrospective study 38 children treated in our Hospital, during the last seven years, because of non congenital gall-bladder disease. The diagnosis established were cholelithiasis 58 for 100, hydrops 26 for 100, acalculous cholecystitis 13 for 100 and hemobilia in one child. Cholelithiasis was more frequent in females at a rate of 2/1. Just the opposite incidence was found for acalculous gall-bladder pathology. Mean age at time of first hospitalization was 7.6 years for the cholelithiasis group and 6.6 years for the rest. Symptoms in order of frequency were abdominal pain, vomiting and fever. Abdominal sonography has been basic in establishing to correct diagnosis in practically all cases. Our most interesting finding has been the clinical manifestations of patients with "Hydrops", consisting of abdominal pain which disappears spontaneously after 24 to 48 hours. The diagnosis has been established only after performing abdominal sonographic studies in children with abdominal pain of unclear etiology.

Use of gold nanoparticles as crosslink agent to form chitosan nanocapsules: study of the direct interaction in aqueous solutions.

A systematic study of the interaction between free anionic gold nanoparticles and chitosan in a solution is presented. A spectroscopic study of the interaction between 10nm gold nanoparticles and low molecular weight chitosan is reported as a function of the concentration and pH of the polymer in a solution. Zeta potential measurements and TEM images indicate the effective aggregation of the nanoparticles in the presence of chitosan. At the same time, anionic gold nanoparticles act as crosslink agents to form chitosan nanocapsules with an average molecular size of 260nm. The changes of the surface plasmon band due to the adsorption of the polymer on the nanoparticle surface allow using of the citrate gold nanoparticles as sensors of the polymer for analytical purposes. The limit of detection for chitosan biopolymer is 69nM. The optimum pH for the interaction between the biopolymer and the nanoparticles is found at a value of 6.4, obtained from spectrophotometric measurements and applying a deconvolution analysis of the experimental data. A simple model based on molecular surface electrostatic interactions is proposed to understand the pH dependence of the investigated system.

Thermodynamic and structural study of phenanthroline derivative ruthenium complex/DNA interactions: probing partial intercalation and binding properties.

The binding of [Ru(PDTA-H(2))(phen)]Cl (PDTA = propylene-1,2-diaminetetra-acetic acid; phen = 1,10 phenanthroline) with ctDNA (=calf thymus DNA) has been investigated through intrinsic and induced circular dichroism, UV-visible absorption and fluorescence spectroscopies, steady-state fluorescence, thermal denaturation technique, viscosity and electrochemical measurements. The latter indicate that the cathodic and anodic peak potentials of the ruthenium complex shift to more positive values on increasing the DNA concentration, this behavior being a direct consequence of the interaction of both the reduced and oxidized form with DNA binding. From spectrophotometric titration experiments, the equilibrium binding constant and the number of monomer units of the polymer involved in the binding of one ruthenium molecule (site size) have been quantified. The intrinsic circular dichroism (CD) spectra show an unwinding and a conformational change of the DNA helix upon interaction of the ruthenium complex. Quenching process, thermal denaturation experiments and induced circular dichroism (ICD) are consistent with a partial intercalative binding mode.