[Arterial hypertension with renal disease revealed by heart failure in infants: two case reports]. / Hypertension artérielle d'origine rénale révélée par une défaillance cardiaque chez le nourrisson : à propos de 2 cas.

While blood pressure measurement methods in infants are well established, hypertension, a rare disease in this population, may still be revealed by heart failure. Kidney diseases are the most common causes of hypertension, prompting the search for a renovascular cause to start appropriate treatment. We report on 2 cases of late diagnosis of hypertension in infants, with hypertensive cardiomyopathy, one in the context of autosomal recessive polycystic kidney disease and the other in the context of renal artery stenosis with hemodynamic disorder, hypertensive encephalopathy and neurological sequelae. In both cases, the equilibrium of blood pressure was difficult to achieve in the acute phase. Renal ultrasound is fundamental for diagnosis. The potential complications related to hypertension require early diagnosis, emphasizing the importance of measuring blood pressure during a routine consultation in infants.

[Malignant pertussis: 3 case reports]. / La coqueluche maligne : à propos de 3 observations.

Three infants aged less than 2 months were hospitalized for malignant pertussis. Echocardiography showed pulmonary hypertension. High-frequency oscillations and nitric oxide were ineffective. Respiratory and hemodynamic conditions deteriorated secondarily. The third case received an exchange transfusion without success. All three infants died following multiorgan failure. Malignant pertussis is the leading cause of infectious death in infants less than 2 months of age, treatment is often ineffective, and prevention, targeting the population of young adults, is particularly important.

[Botulism in a neonate]. / Toxi-infection botulique chez un nouveau-né.

Botulism was suspected in a 17-day-old breastfed infant who developed over 2 days progressive muscular weakness and hypoventilation. The patient also presented with pupil dilation and light unresponsiveness. The electroencephalogram was normal. Full recovery was obtained after 85 days of artificial ventilation. Diagnosis was confirmed by the presence of the botulin toxin B in the patient serum. The source of the infection was not identified.

[Flexible bronchoscopy in children. Experience at French centers of pediatric pneumology]. / La fibroscopie bronchique chez l'enfant. Expertise des centres français de pneumologie pédiatrique.

INTRODUCTION: Fibreoptic bronchoscopy (FB) is an important diagnostic examination in paediatric pulmonology. In 2002 the Paediatric Pulmonology and Allergy Club undertook a retrospective study to establish the current status of fibreoptic bronchoscopy among its members. METHODS: In 2001 sixty five paediatric pulmonologists carried out an average of 116 examinations (+/- 111) in 35 paediatric centres. FB was performed either in an operating theatre (15 centres), a dedicated bronchoscopy suite (6 centres) or an endoscopy suite shared with gastro-enterologists (7 centres). Other examinations were performed in areas dedicated to, or associated with intensive care. General anaesthesia was routinely used in 18 centres. The others used sedation including an equimolar mixture of oxygen and nitrous oxide in 14 centres. Ten centres performed less than 50 examinations, 12 between 51 and 100, 4 between 101 and 200 and 8 centres more than 200 in the year. Seventy two per cent of the children were less than 6 years old. The washing and disinfection procedures were manual in 20 centres and automatic in 15. RESULTS: Three principal indications were reported: persistent wheezing, suspicion of a foreign body and ventilatory difficulties. Cough, desaturation and fever were the most frequently reported side effects. CONCLUSIONS: This is the first survey in paediatric pulmonology in France. It shows a wide variation in the practice of fibreoptic bronchoscopy in children.

[Intestinal volvulus in extremely premature infants]. / Volvulus du grêle et grande prématurité.

UNLABELLED: Volvulus with or without malrotation are infrequent in the extremely premature newborn. CASE REPORTS: Intestinal volvulus in seven premature newborns are reported with abdominal distention, bright and tense skin without visible bowel loops and spiraled bowel loops on the abdominal X-ray. Intestinal resection was avoided due to early diagnosis. We identified abdominal wall massages as a risk factor, because no new cases have occurred since interdiction of these practices. CONCLUSIONS: Symptoms and radiologic findings are relatively specific for excluding the diagnosis of necrotizing enterocolitis in premature newborns. Abdominal nursing could be the incriminating factor.

[Zinc deficiency in mucoviscidosis]. / Carence en zinc au cours de la mucoviscidose.

UNLABELLED: Zinc deficiencies can induce dermatitis in subjects presenting cystic fibrosis. CASE REPORT: A patient, Clement, presented with a digestive form of cystic fibrosis. At four months of age, he presented a dermatitis similar to acrodermatitis enteropathica. Early clinical diagnosis and treatment led to a rapid response to zinc sulfate therapy. DISCUSSION: Less intestinal absorption, malnutrition, and diet are just some of the numerous reasons for the zinc deficiency in this case. Biological support is not necessary to begin the treatment.

[Heterozygous protein C deficiency: apropos of 2 cases with cerebral venous thrombosis in the neonatal period]. / Déficit hétérozygote en protéine C: à propos de deux cas avec thromboses veineuses cérébrales en période néonatale.

UNLABELLED: Thrombotic accidents in the newborn, particularly cerebrovascular accidents, are reported in case of abnormalities in the coagulation system and rarely in heterozygous protein C deficiency; a low protein C level could be either physiological or acquired. CASE REPORT: Two cases of heterozygous protein C deficiency are reported in neonates. Severe neurologic distress was associated with bloody cerebrospinal fluid, and hemorrhagic lesions due to cerebral sinovenous occlusion were visualised by cerebral imaging. The course was severe. One case was associated with renal thrombosis. Mutation in the 168 proline/leucine was detected by molecular biology in the neonates and their mothers. In one case a treatment with protein C had no beneficial effect. CONCLUSION: Cerebral sinus venous thrombosis has to be sought by magnetic resonance imaging in the case of neurologic distress with profound cerebral hemorrhage in the newborn. A low level of protein C has to be interpreted with caution. The diagnosis of a heterozygous deficiency status can only be made through molecular biology. The effect of treatment with protein C concentrate is questionable.

[Thrombocytopenia due to materno-fetal allo-immunization. Report of a familial case]. / Thrombocytopénie par allo-immunisation materno-foetale. A propos d'une observation familiale.

The diagnosis of fetomaternal alloimmune thrombocytopenia (FMAT) was made in a newborn with thrombocytopenia and intracranial hemorrhage. The first child of the family was severely affected with neurodevelopmental sequelae secondary to intracranial hemorrhage. According to the maternal HPA phenotype, close to 100% of subsequent pregnancies could be expected to be affected as the homozygous state was observed in both platelet systems. Another infant was born after a poorly followed pregnancy and was affected as was his elder brother. Prednisolone was given during another pregnancy. A thrombocytic newborn without intracranial hemorrhage was delivered by prudent cesarian section. The infant received platelet transfusion (maternal platelets). We present case histories of FMAT, and stress the conditions for prenatal diagnosis and management.

Arbitrarily primed PCR, ribotyping, and plasmid pattern analysis applied to investigation of a nosocomial outbreak due to Enterobacter cloacae in a neonatal intensive care unit.

In December 1992, Enterobacter cloacae was isolated from the oropharynx and respiratory tract of six ventilated neonates hospitalized in the intensive care unit (ICU) of our hospital. To establish the spread of the outbreak, 41 strains of E. cloacae were analyzed for genotypic markers by three methods: plasmid profile analysis, ribotyping with EcoRI or PvuII endonuclease, and arbitrarily primed (AP) PCR. The tested strains included 12 isolates from the 6 epidemic cases, 4 isolates from the respiratory tract of 4 children hospitalized in other wards during the same period, 13 isolates from 12 children hospitalized in pediatric units before or after the outbreak, and 12 epidemiologically unrelated isolates. Ribotyping and AP PCR demonstrated that each of the last 12 strains exhibited distinct genomic patterns, as did each of the strains isolated from neonates hospitalized before or after the epidemic peak. Conversely, two clones of strains were found among the isolates recovered in December, with concordant results being obtained by the three typing methods: the first clone included seven strains from five ventilated children in the ICU and two children from another ward; another clone was shared by one neonate in the ICU and an infant from another ward. These results indicate that ribotyping and AP PCR-the latter applied, to our knowledge, for the first time to the genotypic analysis of E. cloacae--represent very discriminatory tools for the investigation of nosocomial outbreaks caused by this species.

[Value of aortopexy in infants in the treatment of segmental tracheomalacia]. / Intérêt de l'aortopexie chez le nourrisson dans le traitement des trachéomalacies segmentaires.

We report two cases of localized tracheomalacia, one associated with esophageal atresia, and one isolated. The pathophysiology explains that the symptoms are more important during or shortly after eating, the alimentary bowl crushing the trachea against the aorta, or the innominate artery. The exact cause of tracheomalacia is unknown. Esophageal atresia is frequently associated. The tracheal compression is more often due to the innominate artery, because its origin is located on the left side of the trachea in infants. The aorta or a vascular anomaly are rarely implicated. The symptoms of tracheomalacia are largely due to airway obstruction during expiration: stridor, baking cough, and the life-threatening "dying spell". For diagnosis, the endoscopy is the most important investigation. Among the many methods of treatment which have been proposed, the aortopexy appears to be the technique giving the best results. A single acute apneic attack is an absolute indication for surgery. It is also important to rule out severe gastroesophageal reflux, which can produce the same symptoms.

[Endoscopic extraction of a foreign body from the distal bronchus in the middle lobe, inaccessible by usual techniques, in a 3-year old child]. / Extraction par voie endoscopique d'un corps étranger distal du lobe moyen inaccessible par les techniques habituelles chez une enfant de 3 ans.

The authors report on the extraction of a piece of peanut impacted into the antero-internal bronchus of the right middle lobe, inaccesible to rigid bronchoscopy. After 4 months, this foreign body was visualised by flexible endoscope and removed by this technique under general anesthesia. We initially used a brush to pull the foreign body out of the distal bronchus and then used a biopsy forceps to remove it. Three months later, the chest-X-ray is normal and the child is asymptomatic.

Nosocomial infection with Legionella pneumophila serogroup 1 and 8 in a neonate.

A case of pneumonia related to 2 serogroups (1 and 8) of Legionella pneumophila (Lp) in a 10-day-old boy is described together with the epidemiological survey in the maternity ward which made it possible to establish its nosocomial origin. Rodshaped bacteria reacting with an Lp genus-specific monoclonal antibody and serogroup 1 and 8 polyclonal sera were detected in bronchoalveolar lavages (BAL) collected on day 13. Serogroups 1 and 8 were recovered from cultures of BAL collected on days 12 and 13. Fourfold or more antibody rises to serogroups 1, 5, 8 and 10 of Lp were observed in sequential serum specimens. Water samples collected from the tank and mixer of the maternity ward grew serogroups 1 and 8 of Lp. Serogroup 1 was detected in large amounts in water samples taken at several points of the hot water supply system and from the oxygen nebulizers and the feeding-bottle heater. Analysis of the Lp serogroup 1 strains isolated from the water by subgroup-specific monoclonal antibodies revealed the presence of 4 different subgroups, one of which was identical to the Lp 1 subgroup isolated from the neonate's BAL. This latter subgroup, reactive with McKinney monoclonal antibody Mab 2, has been described as highly virulent. No other case of legionellosis was recorded in the maternity ward.

[Hemolytic uremic syndrome and febrile urinary Escherichia coli infection]. / Syndrome hémolytique et urémique et infection urinaire fébrile à Escherichia coli.

The authors report on 2 cases of hemolytic uremic syndrome in 2 infants during a febrile Escherichia coli urinary tract infection. They discuss the role of urinary tract infection in the occurrence of hemolytic uremic syndrome and the possible role of specific Escherichia coli strains.