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Because neonates are obligate nose breathers, neonatal nasal obstruction may have serious consequences. Prompt diagnosis and appropriate treatment are essential to avoid severe hypoxia. The most common cause of neonatal nasal obstruction is rhinitis. However, it is essential to first rule out anatomical causes such as choanal atresia and stenosis, pyriform aperture stenosis, traumatic deformation or tumors, as these conditions may be life-threatening and require prompt surgical treatment.

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BACKGROUND: To evaluate the efficiency of intraoral saccharose administration for analgesia among neonates born after at least 33 weeks of gestation. POPULATION AND METHOD: Thirty-seven neonates from two neonatal units were tested using an objective scale of infants' pain. A double blind study of the heel prick response after saccharose vs. water administration was performed. RESULTS: Prior administration of saccharose significantly reduced the pain reaction (1.24 vs. 2.24, P < 10(-5)). CONCLUSION: The analgesia obtained after an intraoral saccharose administration can be useful for repeated punctures for which common procedures of analgesia are ineffective. Beyond its use for heel prick, this procedure could be proposed for venous punctures when ELMA analgesia is not possible.

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Generalized lymphangiomatosis is an extremely rare condition with clinical features depending on the extent of involvement. We report a newborn infant with chylothorax and cutaneous lymphangiomas of unique clinical presentation. The baby required artificial ventilation, pleural drainage, low triglyceride infusions, and diet. The course was favorable, with a complete regression of the cutaneous lymphangiomas.

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BACKGROUND: Neonatal alloimmune thrombocytopenia (NAIT) in the HPA-3a system is responsible for less than 5% of all cases of NAIT. CASE REPORT: Thomas, a male infant, was born at 39 weeks of gestation after an uncomplicated pregnancy. Delivery was normal. The Apgar score was 9 at 1 minute, and 10 at 5 and 10 minutes. At 1 hour of age, he displayed extensive petechiae and purpura over the back. The platelet count was 8,000/mm3. Hematesis and extensive petechiae were noted, leading to an exchange transfusion followed by a transfusion of 0.5 U/kg of random donor platelets, 0.4 g/kg/d of intravenous immunoglobulin (IVIg) and 10 mg/kg/d of corticosteroids. IVIg were discontinued on d5 and corticosteroids on d10. There was no relapse of thrombocytopenia. A neonatal alloimmune thrombocytopenia with an HPA-3a (Baka) incompatibility was confirmed. CONCLUSION: HPA-3a incompatibility is certainly more frequent than the rare cases reported and must be searched for in all cases of neonatal thrombocytopenia.

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BACKGROUND: Only 11 cases of beta mannosidase deficiency have been reported until now. We report a new case. CASE HISTORY: J was born at full term to consanguineous parents; her weight was 2,080 g and her height was 44 cm. During the first months of life she was hypotonic and had feeding difficulties. At the age of 7 months, she was admitted to an intensive care unit because of a serious inhalation. Standard blood analysis, chest X-ray, abdominal ultrasonography, electroencephalogram, cerebral nuclear magnetic resonance and electromyography were normal. Blood and urine amino acids and urine organic acids were also normal. The only detected abnormality was a marked deficiency of beta mannosidase in her serum and leukocytes. Later on, she suffered from recurring respiratory infections, and she had abnormalities of esophageal mobility, hypotoria of the lower esophageal sphincter, and at the age of 2 years, achalasia requiring surgery. To date, her motor development is retarded. CONCLUSIONS: The main clinical manifestations of beta mannosidosis are various degrees of mental retardation, speech disorders and hearing loss. Our patient presented with abnormalities of swallowing and esophageal motility resulting in recurring respiratory infections, previously reported in some other cases.

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BACKGROUND: Blood C-reactive protein levels have been frequently found to be increased after Curosurf instillation. These variations have been compared to the values after Surfexo therapy and after absence of surfactant therapy. POPULATION AND METHODS: The files of not infected premature babies, aged 25 to 36 weeks of gestational age, under mechanical ventilation for a hyaline membrane disease (HMD), admitted in our unit between January 1990 to June 1995, have been retrospectively studied. They were separated into three groups: A: 67 infants ventilated for more than 5 days for HMD without surfactant therapy; B: 23 infants treated by Surfexo; C: 60 infants treated by Curosurf. CRP was measured daily between day 0 (DO) and D5. Means and standard deviations were calculated for each day and each group. The mean values of CRP at D1 to D5 in group C were compared to DO. The daily CRP values were compared in the three groups. For group C, the results were studied daily according to the gestational age, dosage and age of the neonate at the first instillation. The statistical results have been given according to the Student t test. RESULTS: After Curosurf, the mean CRP value rose significantly from D1 to D4 compared to D0. There was no difference of CRP between groups A and B from D0 to D5, Group C had higher values in comparison to group A (between D1 to D5) and to group B (between D1 and D3). There was no significant difference of the CRP values in group C according to the number of instillations or the amount instilled, but CRP was lower in early treated infants (< H6). DISCUSSION: Curosurf instillation is followed by a significant increase in CRP, maximum at D2. This is not seen after Surfexo. This increase seems less important at D2-D3 when Curosurf is administered early. The CRP increase after Curosurf therapy could be due to an inflammatory reaction to the heterologous proteins it contains.

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BACKGROUND: Mild methemoglobinemia is a known side-effect of one of the constituents of EMLA cream, this topical local anesthetic is used with great caution in neonates. POPULATION AND METHODS: One hundred and sixteen neonates admitted from January to July 1994 in an intensive care unit were included in the study. All required skin punctures which were performed 1 h 30-2 hours after EMLA had been applied on the skin. A reaction score (0 to 5) to skin puncture was established 157 times (120 after and 37 without local anesthesia); methemoglobin (Met Hb) concentrations were measured in 47 blood samples, 18-24 hours (40.4% of samples) or 2-3 days (36.2%) after application of EMLA. RESULTS: Ninety-four neonates were quiet before puncture (score 0-1). Among them, 57% of those who were given EMLA had a low score (2 or less) vs 18% without EMLA. A low reaction was observed in 65% when the dressings had been kept in place for at least 90 minutes vs 15% with a shorter application. A lower reaction was noted in 78.8% of cases after venopuncture (41% after arterial puncture). No Met Hb level was above 5% and 7 (15%), in five neonates, were between 3 and 5%. There was no clear relationship between methemoglobinemia and gestational age or duration of dressing. CONCLUSION: EMLA cream is effective and safe in neonates including preterms, when it is applied in a small amount once a day.

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This paper is a literature overview on the potential long-term side effects of steroid therapy in the prenatal and neonatal period. Incomplete and transient suppression of adrenal function without growth impairment has been reported in prolonged treatment with steroids for bronchopulmonary dysplasia. There seems to be no secondary chronic immune dysfunction. There is evidence for deleterious effects on lung and brain development in experimental animals. In human preterm neonates, no pulmonary and cerebral secondary effects were observed after short prenatal steroid courses, but it is as yet uncertain whether this applies also to prolonged steroid therapy which requires further long-term investigations, including school performance.

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A case of ventriculo-atrial shunt nephritis in a 3 year-old child is reported. It was induced by an infection with Staphylococcus Albus. Complete cure of kidney disease was obtained with antibiotics (Oxacilline and Rifampicine) alone without resorting to surgical removal of the valve. Attention is drawn to the fact that a long-term clinical and biological follow-up is necessary to make sure that this conservative attitude does not compromises renal function.

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A case of purulent pericarditis due to Hemophilus influenzae is reported. In spite of management by antibiotics and pericardial drainage, the disease ran a subacute course towards pericardial constriction. Fifty-two previously reported cases are reviewed. The most important clinical and paraclinical features are analyzed. Emphasis is put on the factors which promote subacute pericardial constriction.

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The indications and effects of exchange transfusion, peritoneal dialysis, osmotic diuresis and early feeding have been studied in 28 children with inborn errors of aminoacid metabolism presenting in the neonatal period. Exchange-transfusion has only a transitory and incomplete effect but it is simple and quick. Peritoneal dialysis has a remarkable and often life-saving effect because the blood levels of toxic metabolites are reduced very effectively in organic acidaemias. However the rate of removal may decline if plasma concentrations fall below a critical level (1.2 mmol/L foor leucine). If the dialysis is prolonged for more than 36 hours it may cause hypoprotidaemia. Osmotic diuresis increases the 45 ml/min. However it is of little elimination of methylmalonic acid because the renal clearance is between 15 and 45 ml/min. However it is of little value in maple syrup urine disease, propionic acidaemia or isovaleric acidaemia because the renal clearance of the toxic metabolites is so low. The early re-introduction of low protein high calorie of a low protein and high calorie diet by continuous intragastric feeding is very important. The authors propose a protocol for the treatment of babies presenting inborn errors of aminoacid metabolism in the neonatal period. Peritoneal dialysis should be started as soon as the diagnosis is considered and continued for 24 to 36 hours. An exchange of transfusion shold be undertaken before and after the dialysis, together with an osmotic diuresis if appropriate. Continuous enteral feeding should be given, the quantity being adjusted to the baby's requirements.

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