Respiratory syncytial virus infections requiring hospitalization in patients with primary immunodeficiency.

Introduction: The aim of the study was to assess the incidence of hospital admission due to severe acute respiratory infection by respiratory syncytial virus (RSV-ARI) in children with primary immunodeficiencies (PIDs) and the severity of RSV-ARI in these patients.

Methods: We conducted a nationwide cross-sectional retrospective and prospective multicentre study in the 2011-2017 period. The study was performed in 15 Spanish hospitals and included children with PID who required hospital admission due to RSV-ARI.

Hypophosphataemic Rickets: Similar Phenotype of Different Diseases.

Hypophosphataemic rickets (HR) is a group of rare disorders caused by excessive renal phosphate wasting in which the participation of fibroblast growth factor 23 (FGF23) can be prominent. These diseases pose therapeutic challenges with important consequences for growth and bone development in childhood, with higher risk of fractures and poorer bone healing, dental problems, and nephrolithiasis or nephrocalcinosis. In some cases, the diagnostic delay can be very long; laboratory findings and an exhaustive anamnesis could help distinguish between various pathologies, and FGF23 values-although currently not routinely measured-have implications for the differential diagnosis.

Off-label use of maraviroc in HIV-1-infected paediatric patients in clinical practice.

Maraviroc (MVC) is not approved for HIV-1-infected paediatric patients. This is the first assessment of the use of MVC-based salvage therapy in vertically HIV-1-infected paediatric patients in clinical settings. The results suggest that MVC-based salvage therapy is useful in children and adolescents with extensive resistance profile leading to maintained virological suppression in up to 88% of the patients with CCR5-tropic virus.

[PHACES syndrome].

Introduction: PHACES syndrome associates a segmental facial hemangioma with cerebral malformations, aortic branches/cranial arteries anomalies, cardiac defects, eye anomalies or ventral wall defects. The aim of this study is to analyze our experience with this syndrome.

Material And Methods: Retrospective study of the cases seen at our unit in the last year.

[Relationship between the radiological length of the opaque enema and the aganglionic length of the segment in Hirschsprung's disease].

Introduction: Barium enema was the first method used for the diagnosis of Hirschsprung's disease, with the appearance of anorectal manometry and its combination with rectal suction biopsy, barium enema has lost value as a diagnosis method but it has also gain importance to decide the surgical technique that will be used for the correction of the disease.

Aim: To determine the correlation between the length of the affected segment showed by barium enema valued and the length of the removed piece.

Materials And Methods: We have studied all Hirschsprung disease's cases diagnosed and treated in our center since 1998, 127 patients underwent Soave-Boley's technique and 51 De La Torre's descent technique.

[Surgical management of bilateral Wilms' tumor: our experience with 18 cases].

Aim: To show our experience in the surgical management of bilateral Wilms' tumor.

Methods: We have reviewed the medical records of 18 patients diagnosed of bilateral Wilms' tumor between 1971 and 2007, evaluating age, sex, clinical situation, imaging studies, histology, treatment, complications and follow-up.

Results: 65% of patients with synchronous Wilms' tumor was stage I-II, 30% stage III and 5% stage IV.

[Ureteral meatotomy associated with vesicostomy, a possible urinary derivation?].

Aim: To show our experience in urinary derivation by ureteral meatotomy associated with vesicostomy.

Methods: We reviewed uni or bilateral ureteral meatotomy associated with vesicostomy carried out in our institution between 1989 and 2006. We report 20 patients (15 boys and 5 girls) with ages ranging from 1 month to 13-years-old (median 4,4-years-olds).

[Biofeedback and electrostimulation in the treatment of non monosymptomatic enuresis].

Aim: To show our experience with biofeedback and electrostimulation techniques in the treatment of non monosymptomatic enuresis.

Methods: We reviewed biofeedback and electrostimulation techniques in the treatment of complicated enuresis carried out in our institution between 1995 and 2000. We report 99 patients (65 girls and 34 boys) with ages ranging from 5 to 14 years old.

[Meckel's diverticulum as a cause of massive rectal bleeding].

A fit three months old male baby was admitted in our hospital with a history of massive painless fresh rectal bleeding. Blood transfusion, hemodynamic stabilization and emergency laparotomy were necessary because of the high suspect of Meckel's diverticulum as the cause of bleeding. It is an uncommon case due to the low age of the patient and the severity of clinical presentation.

[Characteristics and indications of topiramate].

Aim: To evaluate the efficiency of topiramate (TPM), an antiepileptic medication (AEM) which possesses multiple mechanisms of action and good pharmacokinetics, in the different types of childhood epilepsy and to make an appraisal of its value in migraines, bipolar disorder, eating disorders and neuropathic pain, according to studies that have been published. To do so, we have made use of an analysis of the literature, together with a multi centre study conducted in Spain and personal casuistry.

Method: We consider the percentage of seizure free patients and of patients who responded (reduction of 50% or above in the frequency of the seizures) in childhood epilepsy, partial epilepsy, generalized tonic clonic seizures, absence seizures, tonic seizures, patients with diverse types of seizures, juvenile myoclonic epilepsy, Lennox Gastaut syndrome, falling sickness and GTCS, West s syndrome and Dravet s syndrome.

[Progressive subaortic stenosis caused by accessory mitral valve tissue].

Accessory mitral valve tissue is a rare cause of subaortic obstruction. The reported case correspond to a two days old patient diagnosed by 2D-Echocardiography. Serial doppler flow analysis showed progressive left ventricular outflow obstruction.

[The surgical treatment of critical aortic stenosis in a child by the Ross-Konno technic].

A twenty-nine-day old male infant suffering from critical aortic stenosis underwent aortic valvotomy by cardiopulmonary bypass. At three years of age the aortic stenosis recurred and the child underwent a balloon aortic valvuloplasty, but developed severe aortic insufficiency after the procedure. The critical condition of the patient made aortic valve replacement mandatory.

[Monotherapy with vigabatrin in the treatment of West's syndrome].

Introduction: Vigabatrin (VGB) was specifically synthesized to enhance inhibitory GABAergic transmission by elevating GABA levels via irreversible inhibition of GABA transaminase.

Material And Methods: This study was conducted to determine the efficacy of VGB introduced as monotherapy in 26 children fulfilled the criteria for diagnosis of West syndrome. Duration of follow-up was 24 months.

[Portal blood flow studied in children with portal hypertension using Doppler duplex echocardiography].

Unlabelled: The study of portal morphology and hemodynamics is due the fact that the principle symptoms of portal hypertension are due primarily to alterations in the circulation of the venous portal system. The Doppler duplex echography (bidimensional echography in real time plus pulsatile Doppler) allows one to study these circulatory alterations in a non-invasive manner.

Material And Method: For these studies, an echograph (Toshiba, model Sonolayer SSH-60A) was used to study the morphology and the blood flow of the portal vein and the hepatic artery in 83 children.

[Evaluation of the portal blood flow in healthy children by Doppler duplex echography].

We have measured the dimensions and blood flow of the portal vein, as well as the resistance rate of the hepatic artery in eighty-two healthy children by using Duplex Doppler Echography. From the data obtained, we have observed that the parameters of size and blood flow of the portal vein increase with the age, weight and height of the child, with a greater correlation with weight and height than with age. We did not observed any difference between males and females nor any variation in the resistance rate of the hepatic artery with age, weight or height.

[Doppler echocardiographic evaluation of pulmonary hypertension in children].

Introduction: We analysed the usefulness of Doppler echocardiography to determine the presence and severity of pulmonary hypertension (PH) in children.

Method: The whole group consisted of 63 patients, 42 with congenital heart disease that underwent cardiac catheterization (32 of whom had PH = study group) and 21 healthy children. These 21 patients and the remaining 10 without PH at cardiac catheterization made up the control group.

[Surgical cryoablation in a child with malignant ventricular tachycardia refractory to drugs and radiofrequency ablation].

A ten-months-old child with incessant monomorphic ventricular tachycardia is presented. Tachyarrhythmia is refractory to antiarrhythmic drugs and refractory to endocardial radiofrequency current ablation. Epicardial cryoablation of arrhythmogenic area without artificial circulation resulted in the abrupt termination of tachycardia.

[Fever and petechial exanthema in children].

In an attempt to determine clinical and analytical predictive parameters of a possible grave disease, we have carried out a retrospective study of 172 children admitted to our hospital with fever and petechiae as initial symptoms. The ages ranged between 1 month and 10 years. Even though we have not found a clinical symptom or analysis sufficiently sensitive as to predict all grave diseases, the general clinical state of the child associated with either a high or low white cell count and an abnormal coagulation study should be alert signals for a serious infectious disease.

[Real time and pulsed Doppler echography in the diagnosis of hydrocephaly in the preterm newborn infant].

An evaluation of the cerebral blood flow by means of a Toshiba model Sonolayers-S SSH-60 A ultrasonograph was carried out in 44 children preterm born through the major fontanella and was divided in two groups: 1.) twenty-nine children neurologically normal in which the resistance index always achieves higher marks than the children born at term at the expense of a lesser diastolic flow, and a which a physiologic diastolic modification is described. 2.

[A study of cerebral blood flow in preterm newborns. The physiologic modification of diastole].

An evaluation of cerebral blood flow (CBF) was carried out with a Toshiba Sonolayers-S SSH-60 A echography in 127 infants with open major fontanelle which were divided in 3 groups: a) 69 non premature newborn, in which a physiological variation of all CBF parameters was found; b) 29 neurologically normal preterm infants, where the resistance index always was higher than in the preceding group, at the expense of a lower diastolic flow, in which for the first time the "physiological variation of diastole" independent of the gestational age is reported. This variation results in changes in the cerebral flow in the situations of exercise, rest or stress; c) 29 preterm infants with some abnormality, who also showed the physiologic variation of diastole with a poor flow quality were found.

[Usefulness of the determination of free erythrocyte protoporphyrin in relation to other hematologic parameters in iron deficiency].

Hematological parameters and free eythrocyte protoporphyrin (FEP) on a capillary blood sample were measured in 175 apparently healthy children ranging from 6 months to six years of age. Thirty eight children had hematological parameters descended and/or FEP elevated were asked to return for blood counts, FEP, serum ferritin, serum iron, total iron binding, capacity, transferrin saturation and ALA-D activity, on a venous blood sample. Only 34 children returned.