[Pathologic childhood aerophagia]. / L'enfant avaleur d'air: définition et prise en charge.

UNLABELLED: "Air swallowing" described as being part of functional gastrointestinal disorders in "Rome criteria" in 1999 is often misdiagnosed, particularly in non-mentally deficient children. AIMS: To recognize "air swallowing" child and to describe any progress according to the treatment. POPULATION AND METHODS: This retrospective study reports 13 cases of children without mental deficiency or neuromuscular disease. Clinical elements and precise histories are detailed and we have contacted consulting doctors or families for news. RESULTS: Ten boys and 3 girls, from 2,5 years to 10 years old, were referred for long lasting pain or abdominal distension. Numerous laboratory investigations were always normal. Diagnosis relied upon the observation of air swallowing and X-Rays views of gastric distension. Air swallowing was observed 7 times, 9 children had twitches and 3 language troubles. In 10 cases, X-rays showed gastric and colic distension. Three children have Chilaïditi syndrome. Favourable results followed in 12 cases after an average of 28 months of treatment. One case was lost for follow-up. Treatment was long, often disappointing and required the intervention of a psychiatrist, a paediatrician and (temporarily) a speech therapist. CONCLUSION: Pathological childhood aerophagia is often underdiagnosed and deserves to be better known by paediatricians, psychiatrists and surgeons. A late diagnosis leads to many negative results and causes anxiety. An early diagnosis should lead to a multidisciplinary care.

Ciprofloxacin pharmacokinetics in young cystic fibrosis patients after repeated oral doses.

Until recently, only compassionate use of ciprofloxacin in children with cystic fibrosis was possible despite limited pharmacokinetic data. We studied five subjects with cystic fibrosis and exacerbation of pulmonary infection. A 15 mg/kg b.i.d. regimen of oral ciprofloxacin was administered. On the 15th day, eight blood samples were collected and plasma concentrations were measured by HPLC. The actual dose administered ranged from 10 to 14 mg/kg b.i.d., due to the fixed-dosage formulation. Corresponding AUC0-12 ranged from 8.2 to 11.9 mg.h/L. Plasma concentrations were maintained above individual MICs for a median time of 12.7 h over 24 h. The median area under the inhibitory curve was 52.8, which is about half the proposed target value for ciprofloxacin in pulmonary infections with Gram-negative bacteria such as Pseudomonas aeruginosa. A higher dose, administered from a specific formulation to ensure precise dosing, must be given in order to obtain adequate concentrations in cystic fibrosis children.

[Children of foreign origin adopted in France. Analysis of 68 cases during l2 years at the University Hospital Center of Tours]. / L'enfant adopté d'origine étrangère en France. Analyse de 68 observations sur 12 ans au CHU de Tours.

BACKGROUND: The number of children of foreign origin adopted in France has increased in the past 15 years. The aim of this study was to analyse distinctive features and medical problems associated with international adoption. PATIENTS AND METHODS: The files of 68 foreign-origin adopted children who attended or were admitted to the Medical and Nutrition Unit of the Pediatric Hospital in Tours between January 1st 1986 and December 31st 1997, were studied retrospectively. Various parameters were analysed: age at first consultation, age at adoption, country of origin and health problems, especially nutritional status, infectious diseases, growth and development. RESULTS: In the majority of cases, children were adopted before 12 months of age (61.2%), particularly before six months of age (49.3%). The children came mainly from Africa and countries in the Indian Ocean (48.5%), and from Southeastern Asia (33.9%). Malnutrition was frequent in children from Africa. Twenty-one children (30.9%) presented with severe malnutrition characterized by weight less than the mean-2SD for height and required hospitalization. No children had congenital hypothyroidism, hyperphenylalaninemia, nor were HIV positive. Some patients had infectious diseases: 11 Hepatitis B, four congenital syphilis and six tuberculosis, with favorable outcomes after treatment. The other most frequent diseases were acute diarrhea, chronic diarrhea, intestinal parasites, and scabies, varying according to countries. Three girls adopted from southern Asia developed precocious puberty. Growth and psychomotor development were satisfactory overall, but abnormalities were observed in 15 children (22.1%), which is higher than in the general population. CONCLUSION: The expansion of international adoption with distinctive diseases proves the importance of a checkup as soon as possible after the arrival of these children in France. This checkup has to include nutritional and general evaluation and supplementary tests, especially for specific serology and vaccination status. Following this, it would be possible to propose a suitable follow-up to the adoptive parents.

Pseudomonas aeruginosa from cystic fibrosis patients: study using whole cell RAPD and antibiotic susceptibility.

Pseudomonas aeruginosa is the most important bacterial pathogen in lung disease of cystic fibrosis patients. Different morphotypes of the bacterium are frequently recovered in sputum samples of these patients. We developed a whole cell Randomly Amplified Polymorphic DNA (RAPD) technique in order to establish the relatedness between morphotype, genotype and antibiotic susceptibility. Six cystic fibrosis patients already colonized by P. aeruginosa were investigated by collecting three successive sputum samples (before and after antibiotic treatment, and one month later) and selecting 10 isolates per morphotype. 250 isolates of P. aeruginosa were recovered from 16 of 18 sputum samples. Five patients carried a single RAPD type strain four of which showed at least two morphotypes; one patient carried two RAPD types strains. No patients carried the same strain. These results confirmed other studies previously published in showing stability of the chronic colonization with a single strain. Antibiotype differences were not associated with differences of RAPD profiles and no relation was found between antibiotype and morphotype.

[Pulmonary deposition of colistin aerosols in cystic fibrosis. Comparison of an ultrasonic nebulizer and a pneumatic nebulizer]. / Déposition pulmonaire des aérosols de colistine dans la mucoviscidose. Comparaison d'un nébuliseur ultra sonique et d'un nébuliseur pneumatique.

The objective of this study was to quantify the deposition in the lung of a Colistine aerosol generated using a pneumatic nebuliser (Pari LL(R) equipped with a Pari Master, Pari, Germany) and to compare this with the results obtained with an ultrasonic nebuliser (DP100, DP Medical, France) in four subjects suffering from cystic fibrosis being colonised with Pseudomonas aeruginosa. To quantify the pulmonary deposition of the aerosols we have used an indirect isotopic method which consists in assimilating the kinetics of the molecules studied with a serum albumin tagged with Technetium 99m (Tc99mm) and added to a preparation of Colistine. We have previously verified that the addition of a radioactive tracer does not change the normal distribution or dynamics of the medication within the aerosol and the radioactive counter linked to the tracer reflects the mass of the medicament. The pulmonary deposition was expressed as a percentage of the nebuliser dose. A regional analysis of the deposition (central, peripheral, superior and inferior) was carried out and in central deposition compared to the periphery (C/P) and superior compared to inferior (S/I) were calculated. With the DP100 nebuliser the pulmonary deposition of the aerosol was very reproducible from one patient to another, varying only between 9.5 to 14 percent of the nebuliser dose. With the Pari LL the fraction deposited varied more from one patient to another from 5.6 to 27% of the nebuliser dose. In three of four patients, the pulmonary deposition was superior or equal to that obtained with the ultrasonic nebuliser. The patients whose pulmonary deposition was inferior, using the pneumatic nebuliser, was the youngest in the group and co-ordinately poorly the triggering of the nebuliser with the beginning of inspiration. With the two nebulisers, the pulmonary deposition of Colisitine was very heterogeneous throughout the pulmonary parenchyma. The mean of the ratio C/P and S/I obtained in all four patients was identical (1.35 an 0.86 respectively), indicating a deposition of the aerosol which was predominantly central and inferior but was distributed equally in the peripheral parts of the lung. Pneumatic nebulisers offer a reliable alternative notably for domiciliary treatment for Colistine aerosols in patients suffering from cystic fibrosis. In younger patients who have not yet acquired good motor co-ordination, nebulisers which function continuously or are triggered by inspiration seem to be the preferred choice.

[Congenital adrenal hyperplasia and testicular hypertrophy]. / Hyperplasie congénitale des surrénales et hypertrophie testiculaire.

BACKGROUND: Testicular tumors have been reported in boys and adolescents with congenital adrenal hyperplasia (CAH) inadequately controlled by hormonal therapy. CASE REPORTS: Two adolescents were treated for CAH due to 21-hydroxylase deficiency. They developed hyperplastic nodular testes at the age of 16 and 17 years, respectively. CAH in both was inadequately controlled as confirmed by hormonal studies. The tumors regressed after adequate steroid therapy in the first patient but persisted in the second patient despite normalization of 17-OH progesterone and plasma renin activity. CONCLUSION: Testicular ultrasonography should be systematic in all male patients with CAH since radiological findings are earlier than clinical manifestations. The ACTH-dependent tumors require intensification of hormonal therapy in order to obtain tumoral regression and to prevent infertility.

[Re-education in biofeedback: role in terminal constipation in children]. / La rééducation en biofeedback: rôle dans la constipation terminale de l'enfant.

BACKGROUND: Chronic constipation with or without encopresis is often associated with abnormal defecation dynamics evidenced by manometric study and possibly manageable with biofeedback treatment. PATIENTS AND METHODS: Twenty-six children more than 5 years of age suffering from constipation with or without encopresis for at least 6 months were treated with biofeedback plus lactulose; 15 of them were also given psychiatric attention. Pressure recordings from the internal and external sphincters in response to transient balloon distension of the rectum were obtained in all patients as did the recto-anal inhibitory reflex and the rectal sensitivity. RESULTS: Sixteen patients were considered as definitely cured or improved with this management; two others had relapses 6-12 months later and five were not improved; the three remaining patients were lost for follow-up. CONCLUSION: As already reported, biofeedback treatment represents an interesting role in management of such children.

Early cholestasis in premature infants receiving total parenteral nutrition: a possible consequence of shock and hypoxia.

We report 18 premature infants (gestational age: 31.1 weeks +/- 2.6 [mean +/- SD] (range: 28-36]) with necrotizing enterocolitis (NEC) who developed total parenteral nutrition (TPN) associated cholestasis. Liver function tests were performed at the start of TPN (D1) and repeated once a week. Considering the date of cholestasis onset (direct bilirubin > 30 mumol/l and/or serum bile salts > 10 mumol/l), the patients can be divided in two groups. The first group consisted of 9 patients who had cholestasis at D1. In these patients shock and/or hypoxia occurred prior to D1 and were the only risk factors of cholestasis identified before D1. The second group consisted of 9 patients who developed cholestasis after D1 and in whom the cause of cholestasis was multifactorial (sepsis, lack of enteral feeding, shock and/or hypoxia). These results suggest that shock and/or hypoxia can be responsible for early cholestasis in premature infants. We conclude that shock and hypoxia should be considered when discussing TPN-associated cholestasis.

[Arterial hypertension due to mercury poisoning: diagnostic value of captopril]. / Hypertension artérielle par intoxication au mercure: intérêt diagnostique du captopril.

BACKGROUND: Mercury poisoning is a rare cause of hypertension in children. Urinary excretion sometimes remains low despite severe clinical intoxication. CASE REPORT: A 32 month-old girl was admitted with hypertension, tachycardia, apathy, irritability and excessive sweating. Erythromelalgia and neurologic symptoms permitted the diagnosis of acrodynia. Urine mercury remained normal until chelation. Captopril significantly increased urine mercury concentration but failed to improve clinical manifestations. Clinical improvement required infusions of BAL for 5 days then oral dimercaptosuccinic acid for 3 months. Metal vapors originated from the mercury which spilled from a broken thermometer onto the carpet. COMMENTS: Low basal urine mercury could be associated with real mercury poisoning. Small amounts of metal mercury held in a thermometer could produce a high level of mercury vapor leading to intoxication in young children. The binding capacity of metal ions by captopril could be used to increase urine mercury output. Nevertheless, captopril therapy fails to improve acrodynia. Total elimination of mercury requires long-term therapy with BAL or dimercaptosuccinic acid. CONCLUSIONS: An unexpected mode of intoxication and low basal urine mercury are not decisive arguments against mercury poisoning, which is the only cause of acrodynia.

Combined laser-steroid therapy in blue rubber bleb nevus syndrome: case report and review of the literature.

We report the case of a 7-year-old boy with blue rubber bleb nevus syndrome and frequent digestive tract hemorrhage. Gastrointestinal hemangiomas were treated with laser therapy. Intermittent steroid therapy was administered from the age of 6 years. The results are compared to the relevant medical literature.

[Blue rubber bleb naevus syndrome. Diagnosis and management apropos of 2 cases]. / Blue Rubber Bleb Naevus Syndrome. Diagnostic et conduite à tenir à propos de deux cas.

Blue Rubber Bleb Naevus Syndrome is a rare pathology: 70 cases have been noted since the first description, by Gascoyen, in 1860. It is an acquired disease which usually appears before the age of ten. It is marked by angiomatic cutaneo-digestive tumours combined with chronic anaemia and iron deficiency. Its many and various complications, particularly cataclysmic haemorrhages, car jeopardize the vital prognosis throughout. This little known syndrome is rarely diagnosed at the time of its first manifestations. We recall, from two cases and a review of their literature, the clinical characteristics of the syndrome, its complications and its essentially symptomatic treatment. We therefore suggest an approach to its diagnosis.

[Short-duration fatty meal loading for the assessment of intestinal malabsorption syndromes in children: preliminary study]. / Epreuve courte de charge orale en graisse pour l'exploration des syndromes de malabsorption intestinale chez l'enfant: étude préliminaire.

The determination of fecal fat gives a reliable index for studying fat intestinal absorption in children, but requires the collection of whole day stools for 3 consecutive days. To avoid stool collection constraint, the authors implemented a 3-point short-duration fatty meal test with determination of subsequent increase in serum levels of triglycerides and chylomicrons which then were compared to fasting values. Normal values were determined among control healthy children. Five patients with diagnosed malabsorption syndromes showed markedly impaired results. This fatty meal test seemed simple, easy to perform during a full-day admission. Further studies are being implemented to confirm its good diagnostic value.

[Is the qualitative research of benzodiazepines by the EMIT method and diazocopulation in accidental poisoning in children relevant?]. / La recherche qualitative de benzodiazépines par la méthode EMITSt et la diazocopulation au cours des intoxications accidentelles de l'enfant est-elle pertinente?

A retrospective study was undertaken to evaluate the relevance of benzodiazepine detection in 42 children with accidental poisoning. Immunoenzymatic assay for benzodiazepine in serum and colorimetric method in urines were positive respectively in 3 and 4 patients only. Several reasons could explain these results: the high detection threshold, the different drug reactivity according to the molecular structure and the great delay between intoxication and toxicology analysis. An advised physician should not prescribe a toxicological analysis after a small quantity of benzodiazepine ingestion.

[Mucosal impaction of a gastric foreign body (a coin)]. / Inclusion muqueuse d'un corps étranger (pièce de monnaie) gastrique.

The follow up of a 4 1/2 years old boy ingesting a coin shows gastric stagnation of the foreign body. The coin does not pass through the pylorus and upper endoscopy is performed at 6 weeks. No foreign body is visualized but gastric retention is showed by X ray. The coin's extraction is performed after several "biopsy" because mucosal impaction. The patient is now no symptomatic. The anusual complication of coin's ingestion is reported to remember management of this problem.

[Rectal injury caused by a broken thermometer. Risks related to mercury]. / Plaie rectale par bris de thermomètre. Risques liés au mercure.

This is a case of local mercury absorption caused by accidental rectal perforation during monitoring of temperature. This complication is only reported in cases of subcutaneous injury by a broken thermometer. Treatment necessitates complete excision of mercury deposits. When mercury remains, a clinical and biochemical follow-up is necessary but indication for chelation therapy is exceptional.