[Cytomegalovirus infection causing protracted diarrhea in an immunocompetent child]. / Infección por citomegalovirus como causa de diarrea grave rebelde en un lactante inmunocompetente.

Cytomegalovirus infection is a frequent cause of complications in immunodeficient patients. In healthy hosts, cytomegalovirus infection is usually asymptomatic. We describe a case of a 3 month old immunocompetent boy who suffered from protracted diarrhoea that required parenteral nutrition. After treatment with ganciclovir he recovered without any after effects.

[Gastrointestinal malformations: a cause of chest pain]. / Malformaciones gastrointestinales: causa de dolor torácico.

Chest pain is an uncommon cause of consultation in childhood and is even less frequent if resulting from digestive causes. We present the cases of two patients with gastrointestinal tract malformations (diverticulum and esophageal duplication cyst) diagnosed after investigation of chest pain. A potential etiology of diverticulum could be a traction effect caused by fibrous adenopathy of tuberculous primary infection. Duplication cysts are inborn defects. Although these malformations are uncommon, clinicians should take them into account in patients with chest pain.

[Intestinal failure and transplantation in microvillous inclusion disease]. / Fracaso intestinal y trasplante en la enfermedad por inclusiones microvellositarias.

INTRODUCTION: Microvillous inclusion disease is a rare autosomal recessive condition, characterized by severe secretory diarrhea that produces a permanent intestinal failure and dependency on parenteral nutrition. It usually begins in the neonatal period, and the only treatment at present is intestinal transplantation. PATIENTS AND METHODS: A retrospective review was conducted on 6 patients (three males and three females) diagnosed with microvillous inclusion disease between 1998 and 2013. RESULTS: All debuted in the first month of life, with a median age of three days (range, 3-30 days), and had secretory diarrhea dependent on parenteral nutrition, with fasting fecal volume of 150-200ml/kg/day. Light microscopy of duodenal biopsy samples showed varying degrees of villous atrophy without cryptic hyperplasia, accumulation of PAS positive material in the cytoplasm of enterocytes brush border, and anti-CD10 immunostaining was suggestive of intracytoplasmic inclusions. Diagnostic confirmation was performed with electron microscopy. Two of them had a genetic study, and showed mutations in MYO5B gene. Three died and three are alive; two of them with an intestinal transplantation and the third waiting for a multivisceral transplantation.

[Ménétriers disease associated with cytomegalovirus infection]. / Enfermedad de Ménétrier asociada a infección por citomegalovirus.

Menetrier's disease is a rare entity in children, characterized by a protein-losing gastroenteropathy with thickening of the gastric mucosa and generalized edema. The most common etiology is viral, and cytomegalovirus is the agent most frequently implicated. Unlike in the adult, it is a self-limited disorder with a good prognosis in children. Four patients (three boys and one girl) diagnosed with Ménétrier disease in the past five years were reviewed. The mean age at presentation was 28.7 months (range: 10-48 months). The most common clinical symptoms were fever, vomiting, and edema. Endoscopy showed thickened gastric folds and erosions in several stages. All patients had an associated gastric cytomegalovirus infection, and a favorable outcome, with resolution of the disorder,was observed within a few weeks.

[Diagnosis and therapeutic options in chronic idiopathic intestinal pseudo-obstruction: review of 16 cases]. / Diagnóstico y opciones terapéuticas en la pseudoobstrucción intestinal idiopática crónica: revisión de 16 casos.

Chronic idiopathic intestinal pseudo-obstruction (CIPS) is a rare condition in which there is a defective motility of the gastrointestinal tract of unknown cause leading to repeated bouts of intestinal obstruction without organic explanation. This syndrome groups several ill-defined varieties of motor disorders that can sometimes be classified according to the presence of familial incidence and to the presence of muscular or nervous lesions. Nevertheless, a considerable proportion of cases cannot be ascribed to either type. CIPS is a very difficult challenge for pediatric surgeons because our role is never curative and because when we are involved in it is usually as a result of a false diagnosis. We present herein the experience of 2 Pediatric Surgery Departments in this entity. In the last 30 years we have been involved in the management of 16 children with CIPS. Male-to-female ratio was 5:11 and all but 3 patients had symptoms before 6 months of life. Thirteen had abdominal distension, 10 maldevelopment, 9 recurrent bouts of intestinal obstruction, 8 chronic diarrhea, 7 vomiting, 2 dysphagia and 2 constipation. Seven out of the 16 had urinary tract involvement and in three prenatal diagnosis of megacysts was made. The mean delayed time for diagnosis was 3.08 years. Esophageal or antroduodenal manometry was performed in 8 patients and it was abnormal in 7. Histologic and histochemical samples were available in 8 patients, but only in 4 was enough to make a diagnosis of myopathy. Twelve patients underwent 41 surgical procedures. Three are currently included in a program of home parenteral nutrition. Only three have died, and the mean age of the survivors is 13.9 years. In most of the patients with CIPS surgery is only useful for nutritional purposes, for diversion procedures or for intestinal transplantation in extreme cases. Every effort should be made to avoid unnecessary explorations, misdiagnosis and delay in the identification of the syndrome.

[Benign transient hyperphosphatasemia: the contribution of 20 new cases]. / Hiperfosfatasemia transitoria benigna: aportación de 20 nuevos casos.

We report 20 children diagnosed with transient benign hyperphosphatasemia (THI) during a period of 2 years. The mean age was 29.3 months, ranging from 3 months to 10.2 years, with a male predominance (1.5:1). There were several associated disorders, with the most frequent being acute gastroenteritis, failure to thrive and asthma, the mean alkaline phosphatase (AP) value was 4,137 +/- 2,624 U/L (mean +/- SD). All AP values were above the 97th percentile for each age, with a mean of 3.8 times the level in the 97th percentile for every one-year period (between 0 and 14 years), 6.2 times the 50th percentile mean level and 14.8 times higher than the mean normal upper limit value for adults. The range was from 1,199 to 9,950 U/L. Their serum AP levels returned to normal in a mean period of 3.16 months (1 to 6 months) i 18 cases. The remaining 2 cases are pending on a new checkup. There was no seasonal predominance and the frequency was uniform throughout the year In 2 cases the onset age was more than 5 years and in another 2 patients follow-up serum AP levels did not return to reference values within 4 months. THI is a benign and self-limited entity. We think that the age and duration limits proposed by Kraut may be too rigorous; hence, it would be advisable to review these criteria.

[Cystic fibrosis: nutritional considerations]. / Fibrosis quística: aspectos nutricionales.

During the last few decades, improved treatment measures and nutritional support in cystic fibrosis have increased survival and quality of life in these patients. There is a clear relationship between the development of malnutrition and worsening in respiratory function and both factors have been related to poor disease outcome. Malnutrition is a very frequent complication of this disease and is found in 20% of patients, due to negative energy-proteic balance. This disequilibrium can be explained by increased energy expenditure, high nutritional requirements and decreased oral intake. Periodic monitoring of clinical, anthropometrical and functional nutritional status is mandatory. Intake must be greater than requirements and specific nutritional support should be established when required. Patients with cystic fibrosis must receive a hypercaloric and hyperproteotic diet, with a high fat content, a normal quantity of carbohydrates and with pancreatic and liposoluble vitamin supplements in case of pancreatic insufficiency.

[Current indications of esophageal pH-monitoring]. / Indicaciones actuales de la monitorización de la pHmetría esofágica.

Regurgitation is a common manifestation in infants below the age of 1 year and a frequent cause of visits to general practitioners and pediatricians. Accurate diagnosis of this condition requires a good choice of pH monitoring equipment and optimal technique when performing this procedure. The present article is a consensus statement of the Working Group on Gastroesophageal Reflux of the Spanish Society of Pediatric Gastroenterology, Hepatology and Nutrition (SSPGHN) and reflects the conclusions reached in the VII Congress of the SSPGHN (Vigo, 2000). In general, esophageal pH monitoring is indicated in the following situations: (i) when symptoms of gastroesophageal reflux are present and evolution is not favorable, despite appropriate treatment; (ii) when trying to determine the relationship between gastroesophageal reflux and non-digestive symptoms and (iii) when evaluating the results of treatment, whether medical or surgical.

[Triple-therapy treatment during 1 month versus 2 weeks in gastroduodenal disease due to Helicobacter pylori in children]. / Tratamiento con triple terapia durante un mes versus dos semanas en la enfermedad gastroduodenal por Helicobacter pylori en el niño.

The aim of this study was to assess the clinical outcome of two different durations of triple therapy in children with Helicobacter pylori infection. We established two treatment groups: 1) Short treatment, including 2 week of bismuth subcitrate, amoxycillin and metronidazole treatment (n = 21) and 2) Long treatment, which included the same therapy for two weeks plus bismuth subcitrate alone for two weeks more (n = 26). We found no difference between the short and long treatments. The infection remained in 14% and 19%, respectively. In both groups, we observed children (19%) with persistent abdominal pain in spite of Helicobacter pylori eradication and gastritis healing. We conclude that it is not necessary to prolong triple therapy more than 2 weeks in order to have therapeutic success.