Double-blind comparative trial with 2 antiregurgitation formulae.

BACKGROUND AND AIM: Many mothers consult physicians because of frequent infant regurgitation. Guidelines recommend reassurance and dietary treatment as first approaches. The aim of the present study was to test and compare the efficacy of 2 antiregurgitation formulae (ARF). METHODS: A prospective, double-blind, randomized cross-over trial was performed for a 1-month period in 115 formula-fed infants (ages 2 weeks-5 months) comparing 2 ARF (ARF-1: nonhydrolyzed protein, locust bean gum; ARF-2: specific whey hydrolysate, locust bean gum, specially treated starch). The primary endpoint was the incidence of regurgitation. RESULTS: At inclusion, mean age was 9.1 weeks; anthropometric parameters did not differ between the groups. According to the intention-to-treat analysis, the mean number of episodes of regurgitation decreased from 8.25 to 2.32 with ARF-1 and to 1.89 with ARF-2 (statistically significant difference between both ARF, P = 0.0091). The mean score of regurgitated volume decreased significantly more with ARF-2 than with ARF-1 (P = 0.0265). There was no significant difference in stool frequency and consistency between both groups. CONCLUSIONS: The efficacy of both ARF was demonstrated by the decreased number and volume of regurgitations. ARF-2 was statistically more effective than ARF-1. Comparative trials enable the selection of the best therapeutic option.

Solitary bone plasmocytoma of the spine in an adolescent.

Solitary plasmocytoma (SP) represent only about 5% of plasma cell neoplasia. Most patients have generalized disease, that is, multiple myeloma (MM). Solitary bone plasmocytoma (SBP) is a localized plasma cell tumor and is a very rare disease in young patients. We reported here, a case of SPB in a 14-year-old girl with a 10-year disease-free survival after an aggressive treatment. The relationship of SBP to MM continues to be controversial. Recommendations on the diagnosis and management of SBP in adults, based on a literature search and consensus of expert opinion, were recently published on behalf of the Guidelines Working Group of the United Kingdom Myeloma Forum 1. MRI of the spine is necessary to assess local disease. Radiotherapy with doses of 45-50 Gy is the recommended treatment and gives a high rate of local control (83-96%). Chemotherapy remains controversial in contrast to MM, in which intensive chemotherapy with autologous bone marrow transplantation (ABMT) is widely accepted. At the present time, considering the good prognosis of patients with a normal MRI at diagnosis and a complete disappearance of the M protein after radiotherapy, we believe that ABMT should be reserved for relapse or primary therapeutic failure.

[Sleeping position, prevention of sudden death syndrome and gastroesophageal reflux]. / Position de sommeil, prévention de la mort subite du nourrisson et reflux gastrooesophagien.

Based on results of epidemiological studies, dorsal or lateral sleeping positions are now recommanded in the prevention of sudden infant death syndrome (SIDS). This raises an ethical question about the attitude towards the ventral positioning therapy for gastroesophageal reflux (GOR). The consensus conference considers that the ventral position should only be recommanded in GOR when the benefit appears to outweigh the risk of SIDS that it induces. The conference proposes that for infants with simple uncomplicated reflux, sleeping in the prone position should not be introduced in the first line treatment. Prone positioning should be restricted to complicated cases resistant to dietary and medical measures.

Description of two different patients with abetalipoproteinemia: synthesis of a normal-sized apolipoprotein B-48 in intestinal organ culture.

We describe here two patients, M. P. and S. L., with recessive abetalipoproteinemia. Analysis of restriction fragments of DNA from both patients using cDNA probes spanning the entire apolipoprotein B gene revealed no major insertions or deletions. Further, as defined by restriction fragment length polymorphism, abetalipoproteinemia, in these patients, did not appear associated with particular alleles of apolipoprotein B. Northern and dot blot analysis of intestinal mRNA of one patient (M. P.) revealed a normal-sized apolipoprotein B mRNA which was present in slightly reduced amounts. At the cellular level apolipoprotein B was detected in both intestinal and hepatic biopsies, of one patient (S. L.), by immunoenzymatic techniques using polyclonal and monoclonal antibodies to apolipoprotein B-48 and/or B-100. The level of apolipoprotein B-48 appeared to increase in the intestine after a fatty meal. In the other patient (M. P.), although no apolipoprotein B was detected in the enterocytes using similar immunoenzymatic techniques, organ culture experiments using [35S]methionine demonstrated the synthesis of a normal-sized apolipoprotein B-48 which appeared to be normally glycosylated. The glycosylation and processing of two intestinal membrane enzymes, sucrase-isomaltase and aminopeptidase N, were also normal. Although lipids and apolipoprotein B-48 were present intracellularly, no lipoprotein-like particles were observed by electron microscopy in the endoplasmic reticulum, the Golgi apparatus, or in the intercellular spaces of intestinal biopsies obtained in the fasted (M. P. and S. L.) or fed state (S. L.). The defect in these cases of abetalipoproteinemia, therefore, does not appear to involve the apolipoprotein B gene nor the synthesis or the glycosylation of the apolipoprotein but instead appears to involve some aspect of lipoprotein assembly or secretion.

Medium-chain fatty acids increase glucose production in normal and low birth weight newborns.

To study the pathogenesis of hypoglycemia in low birth weight infants, glucose production was measured in five hypotrophic and four premature newborns with glycemia of 45 +/- 6 and 59 +/- 10 mg/dl, respectively. Hepatic glucose output averaged 5.7 +/- 0.4 and 5.3 +/- 0.5 mg.kg-1.min-1 in these neonates vs. 8.2 +/- 0.5 mg.kg-1.min-1 in five normal at term newborns and was correlated with glycemia (P less than 0.02). Despite normal plasma free fatty acids, the low birth weight infants had low ketone levels of 163 +/- 72 and 126 +/- 65 vs. 263 +/- 60 microM in normals. Oral administration of medium-chain triglycerides to the neonates increased their circulating ketones by two- to threefold and restored near-normal glycemia (51 +/- 9 and 76 +/- 8 mg/dl) and production of glucose (6.7 +/- 0.7 and 6.6 +/- 0.8 mg.kg-1.min-1) in the hypotrophic and premature vs. normals (8.7 +/- 0.7 mg.kg-1.min-1). Individual rates of glucose production correlated with ketone concentrations (P less than 0.02). We conclude that the hypoglycemia characterizing low birth weight neonates is primarily due to impaired glucose production. That exogenous lipids were able to increase glucose production indicates that fatty acid oxidation plays an important glucoregulatory role in the human newborn.

[Recto-colic polyps in the child. Analysis of 183 cases]. / Polypes recto-coliques de l'enfant. Analyse de 183 cas.

Between January 1974 and April 1988, 1,533 colonoscopy were carried out in children less than 15 years old. Two hundred and seventy-four polyps were demonstrated in 183 children (106 boys, 77 girls, mean age: 6 years). The main symptom in most cases was rectal bleeding during defecation. A family history of polyps or digestive cancer was found in 6% of all patients. Two hundred and thirty-nine polyps were removed by endoscopic resection, 232 of them by the diathermic snare and 7 by William's hot biopsy technique. Histological examination of 129 polyps revealed a juvenile polyp in 125 cases, an hyperplastic polyp in 2 cases, a lymphoid polyp in 1 case and an adenoma in 1 case. No complications were observed except for one case each of hemorrhage and perforation following endoscopic polypectomy.

[Fatal congenital cutis laxa associated with bone fragility and ruptured arterial dissections]. / Cutis laxa congénitale létale associée à une fragilité osseuse et à des dissections artérielles rompues.

A male newborn exhibited congenital cutis laxa, bone disease and multiple fractures, and lethal ruptured arterial dissections. At post-mortem examination, the histologic elastic tissue of skin and arteries was dysplastic. The bone fragility is unexplained.

[Home treatment of type I glycogenosis by continuous nocturnal gastric feeding]. / Traitement en milieu familial de la glycogénose de type I par alimentation gastrique continue nocturne.

Two children with type I glycogen-storage disease were treated at home with continuous nocturnal intragastric feeding, using a high glucose formula. The children were 6.5 years and 32 months old respectively when initiating treatment, and they have now been treated for 24 and 18 months. A high carbohydrate meal was given soon after stopping the nocturnal infusion, and this was followed by frequent daytime feeding. In this way, the children were rapidly discharged from hospital and soon returned to school. This regimen stabilized blood glucose levels, avoiding hypoglycemic complications, and improved tolerance to fasting and exercise. Moreover it decreased serum triglyceride, cholesterol, uric acid and lactate levels as well as liver size. The increase in linear growth rate was remarkable and was associated with an increase in insulin-glucagon ratio. No complications resulted from the gastric tube. The method proved to be effective, simple, practical and acceptable by children and their parents. In addition, it is relatively inexpensive and represents a reliable long-term alternative therapy to portocaval shunting for patients with type I glycogen-storage disease. The nocturnal infusions should be continued until after adolescence.

[Esophageal pH-metry and gastroesophageal reflux in infants and children]. / pHmétrie oesophagienne et reflux gastro-oesophagien du nourrisson et de l'enfant.

Twenty five infants, all under 3-year-old, 16 with symptoms of gastroesophageal reflux and 9 controls, were studied by continuous pH esophageal monitoring during 18 to 24 h. The diagnostic value of several pH parameters was calculated in reference to the upper limits of normal ranges previously determined in other studies performed in similarly aged children. Sensitivity and specificity of these parameters were respectively 69 and 89 p. 100 for the time elapsed below pH 4, 44 and 100 p. 100 for the number of reflux episodes per hour, 56 and 89 p. 100 for the duration of the longest episode of reflux, and 31 and 100 p. 100 for the number of reflux episodes lasting more than 5 min. These results confirm that a percentage Of reflux time (less than pH 4) longer than 5.2 p. 100 of the total duration of pH recording has the best discriminative value. However in clinical practice 2 or 3 h postprandial pH recording have a good diagnostic value when the results are expressed using a reflux score, as in adult patients. In further studies concerning gastroesophageal reflux in infants, it is concluded that diagnostic criteria must include evidence of reflux using a discriminant pH parameter.