[Image of the month: Severe hydrocephalus in a child withcongenitalmyotonic dystrophy X]. / L'image du mois : Hydrocéphalie sévère chez un enfant avec dystrophie myotonique congénitale.

Severe hydrocephalus in a child with congenital myotonic dystrophy X A young patient with congenital myotonic dystrophy, or Steinert's disease, presented at the age of 4.5 months with an increase of his head circumference and signs of intracranial hypertension. The results of the radiological exams reveal a major hydrocephalus. The patient condition evolved favourably after ventriculoperitoneal bypass. While ventriculomegaly is common in congenital myotonic dystrophy, hydrocephalus with signs of intracranial hypertension is rare, hence the need of regular monitoring of head circumference.

Un jeune patient atteint de dystrophie myotonique congénitale, ou maladie de Steinert, présente, à l'âge de 4 mois et demi, un décrochage de son périmètre crânien et des signes d'hypertension intracrânienne. Le bilan radiologique révèle une hydrocéphalie majeure. L'enfant évolue favorablement après dérivation ventriculopéritonéale. Alors que la ventriculomégalie est fréquente lors d'une dystrophie myotonique congénitale, l'hydrocéphalie avec signes d'hypertension intracrânienne est rare, d'où la nécessité d'un suivi régulier du périmètre crânien.

[Acute neonatal osteomyelitis, an incidental life-changing diagnosis]. / Cas clinique. Une ostéomyélite aiguë néonatale, un diagnostic parfois fortuit qui peut changer une vie.

Acute neonatal osteomyelitis is a challenging disease and its diagnostic is important to avoid comorbidities. Staphylococcus aureus is the most often involved germ. The diagnostic challenge lies in its pauci-symptomatology in the premature infant in contrast to a more obvious clinical presentation in the term infant or child. The risk factors inherent to prematurity are invasive monitoring, repeated blood sampling, prolonged central catheterization, immature immune response and length of hospital stay. We report the case of an osteomyelitis secondary to staphylococcal sepsis in a preterm infant born at 25 weeks and 3 days of gestational age. The diagnosis was made incidentally on an abdominal x-ray. The low parental compliance for the child's follow-up does not allow us to affirm a future without sequelae even if the elements at our disposal at 8 months suggest a favorable outcome. Acute neonatal osteomyelitis remains a difficult but crucial diagnosis for the future development of the child.

L'ostéomyélite aiguë néonatale est un diagnostic rare, mais qui doit être posé pour en diminuer les comorbidités. Le staphylocoque doré est le germe le plus souvent en cause. La difficulté diagnostique réside dans sa pauci-symptomatologie, en particulier chez le prématuré. La voie de contamination la plus fréquente est hématogène. Les facteurs de risque inhérents à la prématurité sont le monitoring invasif, les prélèvements à répétition, le cathétérisme central prolongé, une réponse immunitaire immature et la durée d'hospitalisation. Nous rapportons le cas d'une ostéomyélite secondaire à un sepsis à staphylocoque doré chez un prématuré né à 25 semaines et 3 jours d'aménorrhée. Le diagnostic a été réalisé fortuitement sur une radiographie d'abdomen à blanc. La faible compliance parentale pour le suivi de l'enfant ne nous permet pas d'affirmer un futur sans séquelle, même si les éléments à notre disposition lors d'une consultation à 8 mois laissent penser une évolution favorable. L'ostéomyélite aiguë néonatale est un diagnostic difficile à poser, mais crucial pour le développement futur de l'enfant.

Rigid catheters reduced duration of less invasive surfactant therapy procedures in manikins.

AIM: Different catheters can be used for less invasive surfactant therapy (LIST): feeding tubes inserted with or without Magill forceps, different angiocatheters and centre specific devices, such as umbilical catheters affixed to a stylet. This study compared the effectiveness of LIST devices and endotracheal tubes (ETT). METHODS: Video recordings of 20 neonatologists simulating different LIST techniques on two manikin heads were analysed. Procedural effectiveness was evaluated by the duration of procedures and failure rates. Ease of use was scored. RESULTS: The median procedure time for the Neonatal Intubation Trainer was significantly longer with feeding tubes without Magill forceps. For the more difficult ALS Baby Trainer, successful procedures lasted a median of 24 (17-32) seconds with ETT, 24 (15-36) seconds with stylet-guided catheters and 34 (27-46) seconds and 37 (29-42) seconds with 13-cm and 30-cm angiocatheters, respectively. Both methods using feeding tubes were statistically slower than ETT intubation, lasting 32 (25-44) seconds and 39 (27-95) seconds with or without Magill forceps. Failure rates (7-20%) were no different between the LIST methods. Techniques using feeding tubes were rated as more difficult. CONCLUSION: Only rigid or stylet-guided catheters required tracheal catheterisation times similar to those of endotracheal intubation and neonatologists found them easier.

Diagnostic pitfall in antenatal manifestations of CPT II deficiency.

Carnitine palmitoyltransferase II (CPT2) deficiency is a rare inborn error of mitochondrial fatty acid metabolism associated with various phenotypes. Whereas most patients present with postnatal signs of energetic failure affecting muscle and liver, a small subset of patients presents antenatal malformations including brain dysgenesis and neuronal migration defects. Here, we report recurrence of severe cerebral dysgenesis with Dandy-Walker malformation in three successive pregnancies and review previously reported antenatal cases. Interestingly, we also report that acylcarnitines profile, tested retrospectively on the amniotic fluid of last pregnancy, was not sensitive enough to allow reliable prenatal diagnosis of CPT2 deficiency. Finally, because fetuses affected by severe cerebral malformations are frequently aborted, CPT2 deficiency may be underestimated and fatty acid oxidation disorders should be considered when faced with a fetus with Dandy-Walker anomaly or another brain dysgenesis.

[Prostaglandins and the immune response at the intestinal submucosal level. A potential site for interference with the repeated use of paracetamol and ibuprofen at a young age?]. / Les prostaglandines et l'immunité du chorion sous-muqueux intestinal. Questions à propos de l'usage répété du paracétamol et de l'ibuprofène en bas âge.

Immune deviations have been shown to exponentially increase in young children. As a consequence, research investigating possible environmental reasons for this increase is considered a public health priority. An improved understanding of the immunity of the intestinal submucosal lamina propria has demonstrated the importance of prostaglandins (PGE2s) on its local development with general immune consequences further on. PGE2s appear at this intestinal submucosal level from the metabolism of arachidonic acid mediated by type-2 cyclooxygenases (COX2s) situated in the membranes of many immune cells. The potential risk of repeated inhibition of PGE2 synthesis at a young age has been demonstrated in experiments with animals systemically exposed to a non-steroidal anti-inflammatory drug (NSAID). The repeatedly exposed animal cannot develop tolerance to food antigens and exhibits autoimmune deviations. Acetaminophen (paracetamol) and ibuprofen are analgesic and antipyretic medications given to children either alone or in combination, most often without medical prescription. Recently, it has been demonstrated that paracetamol, like ibuprofen, also carries, besides its central action, a non-selective inhibitory action on peripheral COXs. However, this inhibitory action only relates to physiological concentrations of arachidonic acid and explains the difference in their respective anti-inflammatory effects. Since recently published data have repeatedly reported an increase of immune deviations associated with paracetamol exposure at a young age, it appears important to better understand the possible negative impact of excessive and repetitive inhibitions of the physiological synthesis of prostaglandins by COX2s in childhood during which all immune mechanisms are built up at the intestinal submucosal level. Therefore, a well-designed prospective strategy for pharmacovigilance of these COX inhibitors repeatedly given during childhood is urgently needed.

[Use of therapeutic hypothermia in sudden unexpected postnatal collapse]. / Malaise grave inopiné du nouveau-né à terme : une place possible pour l'hypothermie thérapeutique.

Sudden postnatal collapse of a full-term newborn is uncommon but may result in severe consequences: these include death; epilepsy; and motor, cognitive, or sensory impairment. Most authors suggest applying a therapeutic hypothermia approach when a previously healthy newborn develops moderate or severe encephalopathy after a sudden postnatal collapse occurring within the first hours or days after birth. However, this technique has still not been validated by randomized trials. Only a few cases have been reported in the literature. This article describes five apparently healthy newborns, born between 2007 and 2012, who suffered moderate to severe encephalopathy following a postnatal collapse on their first day of life. It describes their clinical history as well as their treatment and follow-up. The article focuses on the implementation of hypothermia in this indication and its limitations. Two newborns underwent classic therapeutic hypothermia, two others underwent temperature regulation (one at 34.5 °C, the other one for only 15 h because she quickly improved). One newborn, with severe pulmonary arterial hypertension, did not receive therapeutic hypothermia. Two newborns died (one had classic hypothermia and the other hypothermia at 34.5 °C), the outcome of the three survivors at three years, 18 months, and 15 months is good with only transient postural anomalies. Follow-up must be continued to assess their cognitive development and particularly their memorization processes. Additional research and centralization of the cases is required to evaluate the feasibility, safety, and benefits of therapeutic hypothermia in this situation.

Cost analysis of neonatal and pediatric parenteral nutrition in Europe: a multi-country study.

OBJECTIVES: Parenteral nutrition (PN) is critical in neonatal and pediatric care for patients unable to tolerate enteral feeding. This study assessed the total costs of compounding PN therapy for neonates, infants and children. METHODS: Face-to-face and telephone interviews were conducted in 12 hospitals across four European countries (Belgium, France, Germany and UK) to collect information on resources utilized to compound PN, including nutrients, staff time, equipment cost and supplies. A bottom-up cost model was constructed to assess total costs of PN therapy by assigning monetary values to the resource utilization using published list prices and interview data. RESULTS: A total of 49,922 PN bags per year were used to treat 4295 neonatal and pediatric patients among these hospitals. The daily total costs of one compounded PN bag for neonates in the 12 hospitals across the four countries equalled euro 55.16 (Belgium euro 53.26, France euro 46.23, Germany euro 64.05, UK L 37.43/\[euro]42.86). Overall, nutrients accounted for 25% of total costs, supplies 18%, wages 54% and equipment 3%. Average costs per bag for infants <2 year were euro 84.52 (euro 74.65 in Belgium, euro 83.84 in France, euro 92.70 in Germany and L 52.63/euro 60.26 in the UK), and for children 2-18 years euro 118.02 (euro 93.85 in Belgium, euro 121.35 in France, euro 124.54 in Germany and L 69.49/euro 79.56 in the UK), of which 63% is attributable to nutrients and 28% to wages. CONCLUSION: The data indicated that PN costs differ among countries and a major proportion was due to staff time (L 1=euro 1.144959).

[Implementation of the intestinal micro flora in the early stage and adequate immunity later on]. / Etablissement du microbiote intestinal en bas âge et qualité de l'immunité ultérieure.

The pre and postnatal development of human immunity are remarkably continuous. The feto-placental unit builds up to promote a climate of immune tolerance specifically driven in this way by the maternal immunity. The process of birth triggers the development of the infant's postnatal immunity, in first place through the bacterial colonisation of a sterile intestinal mucosa. The progressive immune response stabilisation at the sub-mucosa level during the first year of life will arise from the interface between the host and its microflora. It will take place progressively and will occur thanks to a variety of successive and complementary very complex immune mechanisms, under the influence of a rich and diversified intestinal microbiotia. Solid scientific arguments allow hypothesising that immune deviances later in life could be the consequence of an inadequate bacterial pressure on the intestinal mucosa at the early stage. A variety of epigenetic modifications taking place in this early stage could account for the deviant programming of later immunity. Each health care provider should acknowledge that some therapeutic and nutritional interventions during the first year of life may interfere with this complex immune development, giving rise to a risk of increasing immune deviancies later on.

Review article: prevention of stress-related mucosal bleeding with proton-pump inhibitors.

Stress-related gastric mucosal bleeding occurs in a substantial number of critically ill patients, with clinically important gastrointestinal bleeding prolonging intensive care stay and increasing mortality. This paper reviews the role of proton-pump inhibitors in the prevention of stress-related mucosal bleeding. Bleeding prophylaxis appears to be warranted in patients in intensive care units on mechanical ventilation or those who have coagulopathy. Intravenous histamine H2 receptor antagonists, particularly cimetidine, have demonstrated efficacy for the prevention of bleeding in critically ill patients. Standard delayed-release proton-pump inhibitors have not been extensively studied in this patient group, but there are some data to support their efficacy in increasing intragastric pH, and in the case of intravenous pantoprazole in preventing gastrointestinal bleeding. In a large, randomized controlled trial, immediate-release omeprazole [(IR-OME) Zegerid powder for oral suspension; Santarus Inc., San Diego, CA, USA] administered via gastric tube, was as effective as intravenous cimetidine in the prevention of clinically significant bleeding, and more effective in increasing gastric pH. Effective antisecretory therapy does not appear to increase the risk of nosocomial pneumonia. In conclusion, immediate-release omeprazole provides a safe and effective alternative to intravenous cimetidine for the prevention of stress-related mucosal bleeding in critically ill patients.

Safety and efficacy of long term esomeprazole therapy in patients with healed erosive oesophagitis.

OBJECTIVE: To evaluate the safety and tolerability of long term treatment with esomeprazole in patients with healed erosive oesophagitis, and to describe its efficacy in the maintenance of healing. DESIGN AND SETTING: US multicentre, noncomparative, nonblind study. PATIENTS AND PARTICIPANTS: 807 patients with endoscopically confirmed healed erosive oesophagitis. METHODS: Patients received esomeprazole 40 mg once daily for up to 12 months. Adverse events and clinical laboratory tests were assessed over the study period. Endoscopy was performed at the final visit of the antecedent healing trials and at months 6 and 12 of the current safety trial; gastric biopsies were obtained at the initial visit of the healing trials and at the end of the safety trial. RESULTS: 80.9% of patients completed 6 months of treatment; 76.6% completed 12 months of treatment. There were no serious drug-related adverse events. Diarrhoea, abdominal pain, flatulence, and headache were the only treatment-related adverse events reported by >3% of patients. Mean changes in laboratory measures were generally small and not clinically meaningful. Plasma gastrin levels increased, as expected, and reached a plateau after 3 months. No changes in gastric histological scores were noted in the majority of patients. Evaluation of gastric biopsies revealed an overall decline in chronic inflammation and atrophy. Intestinal metaplasia findings remained essentially unchanged. Life table estimates of maintenance of healing were 93.7% [95% confidence interval (CI) 92.0 to 95.5%] at 6 months and 89.4% (95% CI 87.0 to 91.7%) at 12 months. CONCLUSIONS: Daily treatment with esomeprazole 40 mg for up to 1 year in patients with healed erosive oesophagitis was generally well tolerated and effective. No safety concerns arose.

Efficacy and safety of esomeprazole compared with omeprazole in GERD patients with erosive esophagitis: a randomized controlled trial.

OBJECTIVE: In patients with gastroesophageal reflux disease (GERD), esomeprazole, the S-isomer of omeprazole, has demonstrated pharmacological and clinical benefits beyond those seen with the racemic parent compound. This study was designed to further evaluate the efficacy and tolerability of esomeprazole relative to that of omeprazole in healing erosive esophagitis and resolving accompanying symptoms of GERD. METHODS: Esomeprazole 40 mg was compared with omeprazole 20 mg once daily in 2425 patients with erosive esophagitis (Helicobacter pylori negative by serology) in an 8-wk, multicenter, randomized, double-blind, parallel-group study conducted in 163 centers throughout the US. The primary efficacy endpoint was the proportion of patients with healed esophagitis at wk 8. Secondary endpoints were the proportion of patients healed at wk 4, resolution of heartburn at wk 4, time to first resolution and sustained resolution of heartburn, and proportion of heartburn-free days and nights. Safety and tolerability were also assessed. RESULTS: Significantly more patients were healed with esomeprazole versus omeprazole at wk 8 (93.7% vs 84.2%, p < 0.001; life table estimates, intention-to-treat analysis). Healing rates at wk 4 were 81.7% and 68.7%, respectively. Esomeprazole was superior to omeprazole for all secondary measures and had a similar safety profile. The most common adverse events in both treatment groups were headache, diarrhea, and nausea. CONCLUSIONS: Esomeprazole demonstrates significantly greater efficacy than omeprazole in the treatment of GERD patients with erosive esophagitis. The tolerability and safety of esomeprazole are comparable to that of omeprazole. (Am

Salivary and gastric epidermal growth factor in patients with Zollinger-Ellison syndrome: its protective potential.

OBJECTIVE: Evidence is accumulating that epidermal growth factor (EGF) is a major molecule contributing to the maintenance of the integrity of the upper alimentary tract mucosa before and after injury by acid and pepsin. Patients with Zollinger-Ellison Syndrome (ZES) typically have hypersecretion of acid and pepsin; however, the concentration and rate of secretion of salivary and gastric EGF that could counteract these potentially aggressive factors are unknown. Accordingly, this study was conducted to determine whether EGF affords mucosal protection in ZES patients. METHODS: The concentration and output of salivary (sEGF) and gastric epidermal growth factor (gEGF) were measured in eight patients with ZES and the results compared to those in 17 patients with nonulcer dyspepsia (NUD), serving as a control group. All patients had normal esophageal and gastric mucosa as determined by endoscopy. Total saliva was collected during 1-h parafilm- and 1-h pentagastrin/parafilm-stimulated conditions, as well as basal and pentagastrin-stimulated gastric juice. The concentration and output of EGF were determined by radioimmunoassay. RESULTS: The concentration of EGF in saliva collected from ZES patients after parafilm chewing was significantly higher compared to that in NUD patients (4.61 +/- 0.59 vs 2.75 +/- 0.50 ng/ml, p < 0.05). The concentration of EGF in saliva collected after pentagastrin stimulation in ZES patients was also significantly higher than in NUD patients (4.37 +/- 0.73 vs 2.22 +/- 0.37 ng/ml, p < 0.05). Salivary EGF output during parafilm chewing in ZES and NUD were similar (68 +/- 6.4 vs 109 +/- 25.2 ng/h). Salivary EGF output after administration of pentagastrin in ZES and NUD was also similar (66 +/- 6.1 vs 132 +/- 45.4 ng/h). Basal EGF output in the gastric juice of patients with ZES was 3-fold higher than in patients with NUD (801 +/- 73 vs 271 +/- 32 ng/h, p < 0.01). Pentagastrin-stimulated EGF output was similar in both groups (705 +/- 92 vs 675 +/- 168 ng/h). CONCLUSIONS: Patients with ZES have a significantly higher EGF concentration in saliva and EGF output in basal gastric juice. This elevated content of salivary and gastric EGF in ZES patients may play a protective role in preventing the development of reflux esophagitis and gastric ulcer under the impact of gastric acid and pepsin hypersecretion.

Oral and intravenous dosage forms of pantoprazole are equivalent in their ability to suppress gastric acid secretion in patients with gastroesophageal reflux disease.

OBJECTIVE: The aim of this study was to assess the ability of pantoprazole to maintain gastric acid suppression in patients with gastroesophageal reflux disease who are switched from an oral (p.o.) to an intravenous (i.v.) dosage form. METHODS: A total of 65 patients with gastroesophageal reflux disease were administered either 40 or 20 mg of p.o. pantoprazole daily for 10 days, then were switched to either a matching dose of i.v. pantoprazole or to placebo for 7 days. Acid output (basal and maximal) was measured at the end of the p.o. treatment period and on the first and last days of i.v. therapy. In the primary efficacy analysis, the acid output values at the end of the p.o. pantoprazole treatment were compared with those at the end of the i.v. treatment. Safety was monitored by periodic vital sign measurements, clinical laboratory evaluations, ophthalmic examinations, electrocardiograms, and reports of adverse events. The data were tested by an analysis of covariance and by Wilcoxon signed rank and t tests. RESULTS: Maximal acid output (mean +/- SD) in the 40 mg and 20 mg pantoprazole group after p.o. treatment was 6.5 +/- 5.6 mEq/h and 14.5 +/- 15.5 mEq/h, respectively; whereas, at the end of the i.v. treatment period, the values were 6.6 +/- 6.3 mEq/h and 11.1 +/- 10.2 mEq/h, respectively. In patients given i.v. placebo, acid output was significantly (p < 0.05) increased to 29.2 +/- 13.0 mEq/h by day 7. Both p.o. and i.v. pantoprazole dosage forms had similar favorable safety and tolerability profiles. CONCLUSIONS: The p.o. and i.v. formulations of pantoprazole (40 and 20 mg) are equivalent in their ability to suppress gastric acid output. The i.v. form of pantoprazole offers an alternative for gastroesophageal reflux disease patients who are unable to take the p.o. formulation.

Antacids revisited: a review of their clinical pharmacology and recommended therapeutic use.

Antacids are commonly used self-prescribed medications. They consist of calcium carbonate and magnesium and aluminum salts in various compounds or combinations. The effect of antacids on the stomach is due to partial neutralisation of gastric hydrochloric acid and inhibition of the proteolytic enzyme, pepsin. Each cation salt has its own pharmacological characteristics that are important for determination of which product can be used for certain indications. Antacids have been used for duodenal and gastric ulcers, stress gastritis, gastro-oesophageal reflux disease, pancreatic insufficiency, non-ulcer dyspepsia, bile acid mediated diarrhoea, biliary reflux, constipation, osteoporosis, urinary alkalinisation and chronic renal failure as a dietary phosphate binder. The development of histamine H2-receptor antagonists and proton pump inhibitors has significantly reduced usage for duodenal and gastric ulcers and gastro-oesophageal reflux disease. However, antacids can still be useful for stress gastritis and non-ulcer dyspepsia. The recent release of proprietary H2 antagonists has likely further reduced antacid use for non-ulcer dyspepsia. Other indications are still valid but represent minor uses. Antacid drug interactions are well noted, but can be avoided by rescheduling medication administration times. This can be inconvenient and discourage compliance with other medications. All antacids can produce drug interactions by changing gastric pH, thus altering drug dissolution of dosage forms, reduction of gastric acid hydrolysis of drugs, or alter drug elimination by changing urinary pH. Most antacids, except sodium bicarbonate, may decrease drug absorption by adsorption or chelation of other drugs. Most adverse effects from antacids are minor with periodic use of small amounts. However, when large doses are taken for long periods of time, significant adverse effects may occur especially patients with underlying diseases such as chronic renal failure. These adverse effects can be reduced by monitoring of electrolyte status and avoiding aluminum-containing antacids to bind dietary phosphate in chronic renal failure. Antacids, although effective for discussed indications of duodenal and gastric ulcer and gastro-oesophageal reflux disease, have been replaced by newer, more effective agents that are more palatable to patients. Antacids are likely to continue to be used for non-ulcer dyspepsia, minor episodes of heartburn (gastro-oesophageal reflux disease) and other clear indications. Although their wide-spread use may decline, these drugs will still be used, and clinicians should be aware of their potential drug interactions and adverse effects.

Efficacy of omeprazole versus ranitidine for symptomatic treatment of poorly responsive acid reflux disease-a prospective, controlled trial.

BACKGROUND: H2-receptor antagonists are widely used in patients with gastro-oesophageal reflux disease (GERD) and are frequently continued when symptoms persist. AIM: To compare the efficacy of omeprazole 20 mg once daily with that of ranitidine 150 mg twice daily in relieving GERD symptoms, in patients who remained symptomatic following a 6-week course of ranitidine therapy. METHODS: Patients with heartburn on at least 4 days/week but who did not have endoscopy to assess oesophageal mucosa could participate. This two-phase, prospective trial included a 6-week open-label phase (phase I), followed by an 8-week double-blind phase (phase II). Patients still symptomatic following treatment with ranitidine 150 mg twice daily (phase I) were randomized to double-blind treatment (phase II) with either omeprazole 20 mg once daily or ranitidine 150 mg twice daily. The primary efficacy variable was the proportion of patients with heartburn resolution during weeks 4 and 8 of phase II. RESULTS: Of the 533 patients with GERD who received ranitidine in phase I, 348 patients (65%) were still symptomatic. A total of 317 patients (59%) were randomized to double-blind treatment (phase II). At week 8, a significantly (P < 0.0004) greater proportion of omeprazole-treated patients (70%) experienced no more than mild heartburn compared with ranitidine-treated patients (49%). Complete resolution of heartburn also occurred in a significantly (P < 0. 00001) greater proportion of omeprazole-treated patients (46% vs. 16% of the ranitidine group at week 8). CONCLUSIONS: After 6 weeks of ranitidine treatment, the majority of patients with GERD were still experiencing moderate to severe heartburn. Omeprazole was significantly more effective than ranitidine in resolving heartburn in this group of patients.

Limp as unusual presentation of Langerhans' cell histiocytosis.

UNLABELLED: An isolated eosinophilic granuloma involving the posterior elements of a lumbar vertebra is reported in a 3-year-old boy presenting with progressive limp. Radiological investigations revealed osteolysis of the L5 right pedicle. MRI showed a well-defined homogeneous mass with nonspecific signal intensity. An unusual feature was the paravertebral muscular location of the largest part of the tumour indicating a possible soft tissue origin. Immunohistochemical studies were typical for Langerhans' cell histiocytosis. CONCLUSION: A limp can be due to lumbar and paravertebral muscular location of Langerhans' cell histiocytosis.

Use of granulocyte macrophage colony stimulating factor in children after orthotopic liver transplantation.

BACKGROUND/AIMS: Bacterial infections complicate the course of up to 80% of pediatric liver transplant recipients, and in some cases, neutropenia, surgical complications and/or antibiotic resistance prevent successful control of sepsis. The aim of the present study was to evaluate the safety and efficacy of granulocyte macrophage colony stimulating factors (GM-CSF) in treating neutropenia following pediatric orthotopic liver transplantation. METHODS: Among a cohort of 430 pediatric orthotopic liver transplantation recipients, 13 children (12 months to 15 years, median 2 years, 10 males) received 15 courses of GM-CSF, 5 microg x kg(-1) x d(-1) subcutaneously, during their post-transplant course. In nine cases, the initial neutrophil count was below 1000/mm3. Ten patients were infected. Three received GM-CSF for severe sepsis without neutropenia. The mean duration of treatment was 16.3 days (range 4-49). RESULTS: In all but one neutropenic patient the neutrophil count increased above 1500/mm3 and the mean neutrophil count increased from 1392+/-1912/mm3 (range 130-7170, median 640) to 4508+/-2459/mm3 (range 350-9630, median 4390) (p<0.01). Only one neutropenic patient (FK506 related) failed to respond to treatment. No rejection episode was induced by treatment, no side effects were noted, and patients with sepsis were cured. CONCLUSION: In these patients, GM-CSF was safe, it achieved a significant increase in neutrophilic count, and was beneficial in patients with severe bacterial infections. This compound may prevent infectious complications in neutropenic patients and may benefit patients with severe sepsis with or without neutropenia.

The impact of gastroesophageal reflux disease on health-related quality of life.

BACKGROUND: Gastroesophageal reflux disease (GERD) affects health-related quality of life. METHODS: We enrolled 533 adults with a history of heartburn symptoms for at least 6 months of moderate to severe heartburn in 4 of the 7 days before study entry. Patients were treated with ranitidine 150 mg twice a day for 6 weeks and Gelusil antacid tablets as needed. We measured physician-rated symptoms and the Medical Outcomes Study short-form 36 (SF-36) Health Survey at baseline and after 6 weeks of treatment. Baseline results were compared with normative data for the US population and for patients with selected chronic diseases. Treatment response was defined as no episode of moderate to severe heartburn for 7 days. Statistical significance was set at P <0.001. RESULTS: GERD patients reported significantly worse scores on all 8 SF-36 scales, physical function and well-being, and emotional well-being compared with the general population. Patients with GERD reported worse emotional well-being than patients with diabetes or hypertension. Treatment responders demonstrated significantly less pain and better physical function, social function, vitality, and emotional well-being compared with nonresponders. CONCLUSIONS: Patients with GERD experience decrements in health-related quality of life compared with the general population. The impact of GERD is most striking on measures of pain, mental health, and social function. Successful treatment for GERD results in improvements in health-related quality of life.

Meal type affects heartburn severity.

This study compared heartburn severity, number of episodes, and changes in esophageal pH induced by three meals. Symptomatic volunteers consumed the following on different occasions: McDonald's Quarter Pounder, french fries, and chocolate shake; McDonald's Sausage Biscuit with Egg, cheese, raw onion, and chocolate milk; and Wendy's Chili and red wine. Increases in reflux episodes over baseline for the hamburger, sausage biscuit, and chili meals were 28.8 +/- 5.7, 36 +/- 5.5 and 43.7 +/- 8.8, respectively. The sausage biscuit and chili increased reflux compared to the hamburger (P < 0.05), but the chili did not differ statistically from the sausage biscuit meal. Onset and peak heartburn for the hamburger, sausage biscuit, and chili meals were 45 and 90, 30 and 120, and 15 and 150 min, respectively. Despite lower fat content, chili and red wine promoted more reflux and heartburn pain than the other meals, demonstrating the importance of meal selection in provocative meal studies.