[Prenatal treatment of neonatal hemochromatosis with maternal administration of intravenous immunoglobulins (about four cases)]. / Traitement anténatal préventif de l'hémochromatose néonatale par administration maternelle d'immunoglobulines intraveineuses (à propos de quatre observations).

OBJECTIVES: Neonatal hemochromatosis is a very bad prognosis disease; liver transplantation was the only way to avoid an unfavourable evolution. Nowadays, hypothesis of an alloimmune mechanism for this disease has purposed to administrate high doses of immunoglobulins. PATIENTS AND METHODS: In this study, we report four cases of women whose previous child had neonatal hemochromatosis and who received such a treatment during the next pregnancy from 18 weeks to the term. RESULTS: This treatment allowed to lead their pregnancy to success. At birth, all four neonates were alive. Two of them presented transitory biologic symptoms of liver deficiency. All had a favourable evolution later. DISCUSSION AND CONCLUSION: Maternal treatment with high doses of immunoglobulins during pregnancy seems to improve dramatically the prognosis of neonatal hemochromatosis as it has been already reported. It could also apply to other diseases, which proceed from the same mechanism.

[Correct procedure in the presence of a fetal goiter associated with hypothyroidism. Observations from three cases]. / Conduite à tenir en présence d'un goitre foetal hypothyroïdien. A propos de trois observations.

Fetal goiter is a rare occurrence of which neonatal consequences are not always predictable. Concerning three cases of goiters associated with hypothyroidism discovered in utero, the authors describe the way to take care of in this bad codified situation. They insist upon the major role of ultrasound for goiter diagnosis and its impacts and for control of treatment efficiency. They also discuss intra amniotic L-Thyroxine injection and insist upon the necessity to obtain quick and definite thyroid evaluation after birth before decision to abstain from neonatal therapy.

[Peritoneal tuberculosis in children: report of two cases]. / Tuberculose péritonéale de l'enfant: à propos de deux cas.

UNLABELLED: Peritoneal tuberculosis is an uncommon presentation of extra-pulmonary tuberculosis in children. It usually presents as ascites, abdominal pain, anorexia and weight loss. CASES REPORT: We report two adolescent patients who presented with ascites, fever, weight loss and abdominal distension. In one case, the diagnosis was late, and confirmed by ascites culture. In the second case, a laparoscopy was performed and showed whitish nodules involving the entire abdominal cavity, compatible with peritoneal tuberculosis, later confirmed bacteriologically. CONCLUSION: Peritoneal tuberculosis presents with nonspecific symptoms. Because laboratory investigations may not be helpful, diagnosis may be difficult. Peritoneal-fluid adenosine deaminase (ADA) determination and coelioscopy seem to be the best way to make a rapid diagnosis.

Periventricular leukomalacia and mode of delivery in twins under 1500 g.

BACKGROUND: The optimal mode of delivery in twin gestations remains undefined, particularly for twins weighing less than 1500 g. OBJECTIVE: To evaluate the impact of the mode of delivery on neonatal outcome in twins below 1500 g. MATERIALS AND METHODS: In this multicenter cohort study during 1999, 66 sets of twins born in hospital and weighing below 1500 g formed our study group. Antenatal and neonatal parameters and their relationship to mode of delivery were studied, based on a factor analysis. Analysis of covariance was used to assess the effect of the mode of delivery on postnatal factors, with antenatal parameters used as covariates. RESULTS: Statistical analysis showed that infants delivered vaginally had significantly more periventricular leukomalacia than those children delivered by Cesarean section (p = 0.03). The estimated odds for leukomalacia were higher in the vaginal than in the Cesarean group when adjusted for covariates (OR = 4.7; 95% CI = 1.0, 25.15). CONCLUSION: Routine Cesarean section should be recommended in twin gestations with infants weighing less than 1500 g, regardless of gestational age or fetal presentation.

Prenatal diagnosis of hypoplasia of the corpus callosum in association with non-ketotic hyperglycinemia.

Abnormalities of the corpus callosum are often associated with a poor prognosis due to the anatomical defect itself and associated anomalies that include malformations and inherited metabolic disorders. We report a case of the prenatal diagnosis of hypoplasia of the corpus callosum that was associated with non-ketotic hyperglycinemia. Metabolic disorders are a known association with corpus callosum abnormalities and carry a dismal prognosis. A diagnosis of non-ketotic hyperglycinemia should be considered when a fetus presents with an abnormality of the corpus callosum. A literature search reviews other inherited diseases associated with hypoplasia of the corpus callosum.

Subependymal pseudocysts in the fetal brain: prenatal diagnosis of two cases and review of the literature.

Subependymal pseudocysts are cerebral cysts found in 5% of all neonates. When they are isolated and typical, they result from persistence of the germinal matrix, have a good prognosis and regress spontaneously within a few months. However, associated anomalies are frequent and in such cases the prognosis is poor. They can be of infectious, vascular, metabolic or chromosomal origin. Subependymal pseudocysts have rarely been described in the antenatal period. We report the prenatal diagnosis of two cases of isolated pseudocysts at 32 and 23 weeks. Both ultrasonography and magnetic resonance imaging assisted in the diagnosis and there was normal postnatal outcome in both cases.

[Use of a polyethylene bag: a way to improve the thermal environment of the premature newborn at the delivery room]. / Utilisation d'un sac en polyéthylène: un moyen d'améliorer l'environnement thermique du prématuré en salle de naissance.

BACKGROUND: Early interventions, such as occlusive wrapping of very low birth weight infants at delivery reduce postnatal temperature fall. This new intervention was implemented in our hospital on January 2000. The aim of this study was to investigate retrospectively the effect of polyethylene wrap, applied immediately at birth, on thermoregulation. PATIENTS AND METHODS: Matched pair analysis was conducted for 60 infants delivered inborn at less than 33 weeks' gestation and 60 premature infants who were born during the second half of 1999 fulfilling the same criteria. The only difference in the management (medical and environmental) was wrapping with a polyethylene bag in the delivery room. Rectal temperature and other vital parameters were taken, after removal of wraps, on admission to NICU. RESULTS: The perinatal characteristics of both groups were comparable. Use of wrapping resulted in a significantly higher admission rectal temperature (difference in means = 0.8 degree C, p < 0.0001), this difference was also significant in infants < 30 weeks. The incidence of hypothermia (< 35.5 degrees C) was less frequent in infants enclosed in plastic bags (8.3% vs 55%). No side effects (skin burns, infection or hyperthermia) were attributable to the intervention. The heart rate was higher in the wrapping group (163 +/- 16 vs 150 +/- 17 b/min, p < 0.01), as well as the capillary glycemia (62 +/- 26 vs 45 +/- 30 mg/dl, p < 0.01). There was no significant difference on arterial pressure. CONCLUSION: Occlusive wrapping with a polyethylene bag at birth prevented low rectal temperature in premature infants in the immediate postnatal period. This method is easy, practical and effective, and does not interfere with current practice for resuscitation.

[Early cardiovascular effects of corticotherapy for bronchopulmonary dysplasia]. / Effets cardiovasculaires précoces de la corticothérapie pour dysplasie bronchopulmonaire.

OBJECTIVES: The aim of this study was to analyze the time course of cardiovascular effects in glucocorticoid-treated premature infants with bronchopulmonary dysplasia (BPD). METHODS: In a retrospective case study, 63 ventilator-dependent very-low-birth-weight neonates (mean gestational age = 27.9 +/- 2 weeks and mean birth weight = 920 +/- 275 g) treated with dexamethasone (52%) or betamethasone (48%) were studied. The average value for each study day was calculated for systolic arterial blood pressure and heart rate. RESULTS: At initiation of treatment, blood pressures increased significantly from pre-treatment to day 1 and continued to increase during the first week: as a percentage of pre-treatment baseline the mean increase for systolic arterial blood pressure was 19% (95% confidence interval [CI] = 16, 22) on day 2 (P < 0.001). The maximum amplitude of variation was observed before day 2 for 75% of the study group. As a group as a whole, the heart rate value significantly decreased on day 1 (mean difference = -14.6 beats/min; 95% CI = -16.5, -12.6; P < 0.001), and then reached pre-treatment value within one week. Cardiovascular response was independent of gestational age, birth weight and postnatal age at the beginning of treatment. CONCLUSION: During postnatal steroid therapy a rise in blood pressure is a common side effect, but bradycardia is mentioned very occasionally. The present study shows a marked increase in blood pressure during the first 48 hours concomitant with a decrease in heart rate. The inverse relationship between systolic arterial blood pressure and heart rate suggests a baroreflex response.

[Jargon of the neonatal intensive care unit]. / Le jargon de la réanimation néonatale.

Jargon, the specialized vocabulary and idioms, is frequently used by people of the same work or profession. The neonatal intensive care unit (NICU) makes no exception to this. As a matter of fact, NICU is one place where jargon is constantly developing in parallel with the evolution of techniques and treatments. The use of jargon within the NICU is very practical for those who work in these units. However, this jargon is frequently used by neonatologists in medical reports or other kinds of communication with unspecialized physicians. Even if part of the specialized vocabulary can be decoded by physicians not working in the NICU, they do not always know the exact place that these techniques or treatments have in the management of their patients. The aim of this article is to describe the most frequent jargon terms used in the French NICU and to give up-to-date information on the importance of the techniques or treatments that they describe.

[APN: evaluation behavioral scale of acute pain in newborn infants]. / DAN: une échelle comportementale d'évaluation de la douleur aiguë du nouveau-né.

AIM: This study was designed to validate a behavioral acute pain rating scale for term and preterm neonates (APN). METHODS: From January through June 1996, neonates requiring a heel lance or a venous puncture for blood sampling at the intensive care unit and the nursery of Poissy Hospital were recruited into the study. After a pilot study, a pain rating scale was developed. The scale score pain ranged from 0 to 10 and it evaluated three items: facial expression, limb movements, and vocal expression with ratings per item ranging from 0 to 4, 0 to 3 and 0 to 3, respectively. Two observers evaluated independently each infant during a painful procedure (puncture for a blood sample) and during a dummy procedure (rubbing the thigh softly). RESULTS: Forty-two neonates born between 25 and 41 weeks gestational age were included in the study. Medians (quartiles) of gestational age, birth weight, and corrected postmenstrual age at time of investigation were 34 (29-39) weeks, 1,850 (1,055-3,093) g, and 35.5 (31-39) weeks, respectively. Ten infants were intubated. The scale showed to be sensitive because all possible scores were obtained; during painful procedures scores ranged from 1 to 10, with 95% of scores > or = 3 while during dummy procedures they ranged from 0 to 5, with 88% of scores < or = 2. The medians (95% confidence interval) of scores were for painful procedures 5 (5 to 7) and for dummy procedures 1 (0 to 1). This indicates a good specificity of the scale. High intercorrelation of items (internal consistency) was confirmed by a Cronbach's coefficient alpha of 0.88. Inter-rater agreement was high since the Krippendorff R test was 91.2. CONCLUSION: This behavioral acute pain rating scale for newborns demonstrated a good specificity and sensitivity, internal consistency and inter-rater reliability. This scale could be used to test the analgesic effects of different therapies during painful procedures.

Features of DiGeorge syndrome and CHARGE association in five patients.

We report on five patients presenting with features of two congenital disorders, DiGeorge syndrome (DGS) and CHARGE association. CHARGE association is usually sporadic and its origin is as yet unknown. Conversely, more than 90% of DGS patients are monosomic for the 22q11.2 chromosomal region. In each of the five patients, both cytogenetic and molecular analysis for the 22q11.2 region were normal. In view of the broad clinical spectrum and the likely genetic heterogeneity of both disorders, these cases are consistent with the extended phenotype of either DGS without 22q11.2 deletion or CHARGE association, especially as several features of CHARGE association have been reported in rare patients with 22q11.2 deletion association phenotypes. On the other hand, these could be novel cases of an independent association involving a complex defect of neural crest cells originating from the pharyngeal pouches.

[Telephonic advice by an emergency department given in a simulated pediatric case]. / Conseils téléphoniques donnés aux urgences pour un cas pédiatrique simulé.

BACKGROUND: Emergency departments (ED) are requested everyday to dispense medical telephone advice for children. To evaluate the quality of telephone management, a mock scenario simulating a febrile 4 month-old-girl with signs compatible with septicemia was used. METHODS: One hundred randomly selected French emergency departments were called on. Half of the hospitals had a pediatric department with more than 20 beds; the other half did not have a pediatric department. A research technician called and said: "My baby has got fever and I do not know what to do". Additional information was given only on request. RESULTS: Ninety-four ED gave medical advice by telephone: 65% of the cases by a physician, 24.5% by a nurse, 9.5% by a nurse technician and 1% by a secretary. In hospitals without a pediatric department, physicians took calls in 76.5% of the cases, whereas in hospitals with a pediatric department they only took calls in 53% of the cases. The mean number of questions asked per ED was 3.1. The age of the child was requested by 87.2% of the respondents. Advice was given by 36.1% of the ED without asking either the age of the patient or grade of the fever. The advice given by the respondents was: come to the ED immediately (30.9%), see a community physician immediately (51%), come to the ED tomorrow (2.1%), see a community physician tomorrow (8.5%), and manage at home (7.5%). CONCLUSIONS: This study has shown important inadequacies in pediatric telephone advice given by some ED. It suggests that the respondents do not use a protocol to handle the calls; development of such protocols to guide the histories taken and advice given for the most common telephone queries is urged.

[Inaugural acute cholestasis of Kawasaki disease]. / Cholestase aiguë inaugurale d'une maladie de Kawasaki.

BACKGROUND: Hepatic dysfunction with mild obstructive jaundice occurs occasionally in Kawasaki disease. Acute episode of cholestasis as a presenting symptom has never been reported. CASE REPORT: A 14 year-old-boy was admitted with fever and cholestasis. He subsequently developed the classical manifestations of Kawasaki disease. No signs of liver cell injury or hepatic failure were present. Bacteriological cultures and seroimmunologic markers for viral infection remained negative. There was no ultrasonic abnormality of bile ducts. The child was given intravenous gamma globulins and salicylate. The outcome was favourable without any cardiovascular complications. CONCLUSION: A persistent febrile cholestasis of unknown etiology should evoke the diagnosis of Kawasaki disease.

[Water intoxication following preparation for barium enema]. / Intoxication par l'eau après une préparation pour un lavement baryté.

BACKGROUND: Induction of water intoxication from tap water enemas was reported a few years ago. Its treatment is still debated. CASE REPORT: A 4 1/2 year-old boy was admitted because he suffered from coma grade I. A barium enema had been prescribed for fecal incontinence and the patient had been given orally about 4 liters of water during the 24 hours preceding this investigation. Blood examination showed;: Na 122 mEq/l; K 3 mEq/l; Cl 87 mEq/l. Brain CT scan was normal. The patient was placed under restriction of fluid and was given i.v. 5.8% NaCl solution (2 mM/kg) for 3 hours. Convulsions appeared despite this treatment requiring intubation and ventilation plus increasing doses of NaCl: 20% solution (2 mM/kg) for 30 minutes followed by 2 mM/kg for 3 hours, associated with mannitol and furosemide infusion. CONCLUSION: Use of hypertonic saline solutions in the treatment of water intoxication is discussed. Acute hyponatremia must be rapidly corrected using hypertonic saline solution plus restriction of fluid and diuretic.