Gastroesophageal reflux disease in children: how reliable is the gold standard?

OBJECTIVE: To investigate the causes and degree of interobserver variability in esophageal pH monitoring for the diagnosis of gastroesophageal reflux. METHODS: This retrospective study included all children (n = 72) who underwent pH monitoring during 1 year at Maxima Medical Centre in Veldhoven, the Netherlands. RESULTS: An interobserver variability of 18% was found. Variability was caused by differences in opinion about the duration of registration, doubts about probe position, artifacts and drift of baseline pH. CONCLUSIONS: Most of these problems can be eliminated by posttest calibration and assessment of the pH electrode position. However, a clear definition of monitoring artifacts is lacking. This study shows that mutual agreement in the interpretation of pH studies was fair (kappa coefficient of 0.70).

Doença do refluxo gastroesofágico em crianças: quão confiável é o padrão-ouro? / Gastroesophageal reflux disease in children: how reliable is the gold standard?

OBJETIVO: O monitoramento do pH esofágico é considerado o método mais confiável para diagnosticar o refluxo gastroesofágico. No entanto, a interpretação do mesmo estudo de pH pode diferir entre observadores. Neste estudo, investigamos as causas e o grau de variabilidade entre observadores. MÉTODOS: Este estudo retrospectivo incluiu todas as crianças (n = 72) que realizaram monitoramento de pH durante 1 ano no Maxima Medical Centre, em Veldhoven, Holanda. RESULTADOS: Foi encontrada uma variabilidade de 18 por cento entre observadores. A variabilidade foi causada por diferenças de opinião sobre a duração do registro, dúvidas sobre a posição da sonda, artefatos e flutuação do pH de base. CONCLUSÕES: A maioria desses problemas pode ser eliminada por avaliação da posição do eletrodo de pH e calibração pós-teste. No entanto, ainda falta uma definição clara dos artefatos de monitoramento. Este estudo mostra que a concordância mútua na interpretação dos estudos de pH foi regular (coeficiente kappa de 0,70).

OBJECTIVE: To investigate the causes and degree of interobserver variability in esophageal pH monitoring for the diagnosis of gastroesophageal reflux. METHODS: This retrospective study included all children (n = 72) who underwent pH monitoring during 1 year at Maxima Medical Centre in Veldhoven, the Netherlands. RESULTS: An interobserver variability of 18 percent was found. Variability was caused by differences in opinion about the duration of registration, doubts about probe position, artifacts and drift of baseline pH. CONCLUSIONS: Most of these problems can be eliminated by posttest calibration and assessment of the pH electrode position. However, a clear definition of monitoring artifacts is lacking. This study shows that mutual agreement in the interpretation of pH studies was fair (kappa coefficient of 0.70).

Complications of percutaneous endoscopic gastrostomy with or without concomitant antireflux surgery in 96 children.

BACKGROUND/PURPOSE: A study was conducted of the complications of percutaneous endoscopic gastrostomy (PEG) with or without antireflux surgery (ARS). METHODS: A retrospective review was conducted of all patients, receiving a PEG in the period January 1993 through December 1997. Patients' characteristics including underlying disease, indications, results of preoperative screening, and complications were recorded. PEG placement was performed with the Seldinger technique and, in some cases, under laparoscopic control. In the event of a pathologic pH study during preoperative screening, laparoscopic antireflux surgery (ARS) was added. RESULTS: Mean age was 5 years and 10 months. The majority of the children were mentally retarded. The main indications for PEG were vomiting, food refusal, inability to swallow, and aspiration. Fifty-nine patients had PEG without ARS. Nineteen of these patients had concomitant laparoscopy. Thirty-seven patients had PEG with ARS. One patient died postoperatively of gastric leakage. PEG-related complications occurred in 31% of the patients. There was a significant higher incidence of complications in the group of patients that underwent ARS together with PEG compared with PEG placement without ARS. Roughly half of the complications were peristomal infection related to the use of T-fasteners and the other half gastroduodenal obstruction caused by the balloon of the gastrostomy catheter, both preventable complications. Preoperative vomiting without a positive pH-study disappeared in most cases after PEG placement. Although the pH study normalized in 34 of 37 patients after concomitant ARS, vomiting persisted in 7 of 17 patients. PEG improved the nutritional status in 75% of the children. CONCLUSIONS: PEG improved the nutritional status in the majority of the children. However, PEG placement can lead to a considerable amount of complications, especially when combined with ARS. ARS together with PEG is successful in treating GER but does not necessarily cure preexistent vomiting. PEG alone cures vomiting in 80% of the patients and rarely leads to vomiting. There seems no good reason for combining PEG with ARS. Only if symptoms progress after PEG, ARS should be considered. Caretakers and patients should be well informed before placement.

A novel disorder of N-glycosylation due to phosphomannose isomerase deficiency.

Three siblings suffered from an unusual disorder of cyclic vomiting and congenital hepatic fibrosis. Serum transferrin isoelectric focusing showed increased asialo- and disialotransferrin isoforms as seen in the carbohydrate-deficient glycoprotein (CDG) syndrome type I. Phosphomannomutase, which is deficient in most patients with type I CDG syndrome, was found to be normal in all three patients. Structural analysis of serum transferrin revealed nonglycosylated, hypoglycosylated, and normoglycosylated transferrin molecules. These findings suggested a defect in the early glycosylation pathway. Phosphomannose isomerase was found to be deficient and the defect was present in leucocytes, fibroblasts, and liver tissue. Phosphomannose isomerase deficiency appears to be a novel glycosylation disorder, which is biochemically indistinguishable from CDG syndrome type I. However, the clinical presentation is entirely different.

Autosomal-recessive inheritance of benign recurrent intrahepatic cholestasis.

Benign recurrent intrahepatic cholestasis (BRIC) is a rare disorder characterized by recurrent episodes of cholestasis without permanent liver damage. Familial and sporadic cases have been described. Based on existing evidence, both autosomal-recessive and autosomal-dominant inheritance have been considered. We describe a large Dutch pedigree with 4 patients, strongly suggesting autosomal-recessive inheritance.

Failure to thrive is an early symptom of the imerslund Gräsbeck syndrome.

PURPOSE: The Imerslund-Gräsbeck syndrome (IGS) is a rare inherited disorder characterized by a megaloblastic anemia due to a selective vitamin B12 malabsorption in association with a mild proteinuria. Usually recurrent infections, gastrointestinal complaints, and pallor are presenting symptoms. We report two cases of IGS with an unusual presentation. PATIENTS AND METHODS: Two girls are described with the Imerslund-Gräsbeck syndrome who had a failure to thrive as a presenting symptom without infections or gastrointestinal complaints. The diagnosis of IGS was based on marked macrocytic anemia, very low serum vitamin B12 levels, abnormal Schilling urinary excretion test results, and mild proteinuria. When parenteral vitamin B12 was started, a rapid catch-up growth was seen in both girls. CONCLUSIONS: The absence of well-known causes of failure to thrive, such as recurrent infections and gastrointestinal complaints, favors the concept that the metabolic disturbances caused by an isolated cobalamin deficiency as seen in IGS causes a failure to thrive.

[Nutrition pumps in pediatrics: a comparative study]. / Voedingspompen in de pediatrie: een vergelijkend onderzoek.

Home enteral nutrition as the sole or as an additional way of feeding children is becoming increasingly popular. It can be used in the form of bolus-feeding, or as drip-feeding, either continuous (24 hours) or semicontinuous (nightly). For the latter method usually a feeding pump is used. In the Netherlands several feeding pumps are available, which were tested in this report. These feeding pumps (Compat, Enteroport, Flexiflo Companion, Flocare 500, Frenta Mat, Frenta-System II, Kangaroo 324, Nutromat Päd S, Nutromat S) were investigated for accuracy, available rate-settings, adequate alarming at interruption of the drip feeding, weight and ease of operation. All pumps were tested stationary, and suitable pumps weighing less than 1000 grams were subsequently tested ambulatory. The nine tested pumps were all fairly easy to operate. Three pumps were not suitable for pediatric use, either because the infusion rate was more than 30% out of range (Flexiflo Companion) or because of inadequate rate settings (Nutromat S and Frentasystem II; respectively 20 and 25 ml/hour minimally). Accuracy for the other pumps was always within 10% of the set rate. One pump, the Enteroport, has a rate setting which is suboptimal for pediatric use. In addition this pump does not alarm when the drip feeding is interrupted. The other pumps (Compat, Frenta Mat, Kangaroo 324, Flocare 500, and Nutramat Päd S) are all suitable for pediatric use, both with respect to the rate settings as to the adequate alarming when the drip feeding is interrupted. The Flocare 500 and the Nutromat Päd S, in addition to the Enteroport, have a weight below 1000 grams.(ABSTRACT TRUNCATED AT 250 WORDS)