Search

Background: The prognosis for congenital brain tumors is usually poor, so that their management during pregnancy is difficult., Cases Reports: Case 1. A large cystic mass was revealed by ultrasound at the 37th week of pregnancy; it was located near the brain stem and was associated with ventricular dilation. The newborn was delivered by cesarean section because of an abnormally enlarged head. The CT scan confirmed the presence of this mass containing a fluid that was found to be normal after needle aspiration. Surgical shunting of excess fluid was ineffective, and progressive deterioration prevented further exploration and/or treatment. The child is still living, confined to his bed, at the age of 5 years. Case 2. Ultrasonography at the 30th week of pregnancy showed a tumor located near the brain stem with dilation of the entire ventricular system. Post mortem examination after abortion revealed a capillary and cavernous hemangioma. Case 3. Ultrasonography at the 34th week of pregnancy showed dilation of the ventricular system. As a result, birth was induced. CT scan and MRI of the newborn showed a mixed, solid and liquid, mass in the posterior fossa. Post mortem examination showed a papilloma of the choroid plexus., Conclusion: These rare congenital tumors are usually revealed by ultrasonography showing ventricular dilation. Advances in imaging techniques, especially MRI of fetal brain, should help in the management of such tumors.

All infants born in the Puy-de-Dôme area in 1983 and referred to a neonatal care unit were studied prospectively. Children were seen at their homes at 9 months and at 2 years of age and results were compared with those found in controls. High risk groups were defined on the basis of neonatal variables including prematurity, neurologic anomalies, respiratory distress, assisted ventilation, and growth retardation. At birth, prematurity was associated with an increased risk of respiratory distress and infections were more common in small-for-dates infants. Evaluations at 9 months and at 2 years of age showed increased prevalences of growth retardation and strabismus in the high-risk infants, and neurodevelopmental tests disclosed lower performances in these children. Conversely, infectious diseases and readmissions between birth and two years of age were not increased in the high risk groups, as compared with the control group.

We measured glucocorticoid receptor concentrations in human lung at different stages of alveolar growth. Lung tissue was obtained from 9 surgically aborted or stillborn fetuses of 15 to 28 weeks gestational age, and from 6 infants and children, aged 2 months to 9 years, after lobar resection or at autopsy. Samples were taken from macroscopically healthy areas. Lung histology was performed in all cases. The receptor assay was done by establishing saturation curves for labeled dexamethasone in the absence or presence of a hundredfold excess of unlabeled dexamethasone. Total binding capacity and the dissociation constant were calculated from the saturation curves by the method of Scatchard. The receptor concentration in the fetuses was high (182 +/- 88 fmol/mg prot.), irrespective of gestational age. The lowest concentration (35 fmol/mg prot.) was found in a fetus with pulmonary hypoplasia. In the infants and children the mean receptor concentration was significantly lower (14.6 +/- 9.9 fmol/mg prot.); these included a case of sudden death in which the parenchymal structure was normal.

A prospective cytologic study of cerebrospinal fluids from 57 full-term neonates confirmed the previously reported cellular polymorphism. The cells were classified as (1) exogenous cells, (2) blood and bone-marrow cells, (3) histiomonocytes, (4) cell aggregates and (5) "free" cells. Histiomonocytes were a constant feature in these fluids and were the cause of neonatal pleiocytosis. Various degrees of activation could be recognized. The so-called inactive forms were closely related to brain damage. Identification of erythrophages and siderophages had diagnostic significance. The presence of fat in macrophages (lipophages) suggested a serious developmental disorder. The free cells were incompletely identified, as were the cell aggregates, which probably originate in the meningeal lining. Both require further study.

The authors report five cases of premature newborns whose mothers had been treated with indomethacin. In all patients, clinical and biological symptoms were those of pulmonary hypertension with persistence of the fetal circulation. In two cases, autopsy showed an important reduction of the lumen of pulmonary arterioles due to a thickening of the tunica media. The role of indomethacin in such respiratory syndrome is very likely. Therefore, indications for that treatment in pregnant women should be reduced.

One hundred and one echocardiograms were performed in children 8 days to 15 years old. The values measured were : end-diastolic and end-systolic left ventricular diameters, aortic root diameter, left atrial diameter, end-diastolic septal and posterior wall thickness, mitral valve excursion. There is a good correlation of these values with the square root surface area. The indices of left ventricular performance are : mean velocity of circumferential fiber shortening (correlated with heart rate), ejection fraction and shortening fraction. These indices are not correlated with body surface area. These data can be used in the echocardiographic investigation of infants or children with heart diseases.

A variant of metachromatic leukodystrophy (MLD), Austin disease, is characterized by a multiple isozyme deficiency of arylsulfatase. A 3 1/2-year-old girl with progressive mental and physical deterioration had decreased activities of arylsulfatases A and B in the leukocytes, shown by acylamide gel electrophoresis. Under the electron microscope, biopsy specimens of the brain and the peripheral nerve showed lamellar structures with socalled zebra bodies in the cytoplasmic processes of glial cells, granulo-membranous inclusions with fingerprint configurations in neurons, and myelinlike material in Schwann cells. Results from our study suggest an intricate nature of this dysmetabolic disorder, which shows ultrastructural changes usually seen in classic MLD, a deficiency of arylsulfatase A only, concomitant with those seen in mucopolysaccharidoses such as Hurler and Sanfilippo syndromes.

Seventy-five children with bacterial meningitis were included in a multicentre trial for evaluation of cerebrospinal fluid (CSF) pharmacokinetics and clinical efficacy of cefotaxime. Mean age of patients was 4 years. Causative pathogens were Haemophilus influenzae in 28 patients (37%), Neisseria meningitidis in 27 patients (36%), Streptococcus pneumoniae in 10 patients (13%), group B streptococcus in 2 patients (2%) and unknown in 8 patients. All isolated pathogens were susceptible to cefotaxime. Seven ampicillin-resistant H. influenzae (9.4%) were found. Cefotaxime was 50 mg/kg intravenously, 4 times daily. The duration of treatment ranged from 5 to 22 days (mean: 13.8). Blood and CSF concentrations of cefotaxime were performed in 50 patients 3 h after infusion at day one and seven cefotaxime levels were determinated both by microbiological assay procedure and high pressure liquid chromatography. On day 1, CSF levels ranged from 0.39 to 2.0 mg/l by microbiological assay procedure (median 3.6) and from 0.0 to 17.4 mg/l (median 2.2) for cefotaxime and from 0.0 to 11.5 mg/l (median 2.2) for desacetyl-cefotaxime by HPLC. We observed a decrease in CSF levels of cefotaxime on day 7. They ranged from 0.3 to 7.0 mg/l (median 1.1) by microbiological assay and from 0.0 to 3.3 mg/l (median 0.8) for cefotaxime and from 0.0 to 6.0 mg/l (median 1.0) for desacetyl-cefotaxime by HPLC. On day 1 and day 7, CSF levels determined by microbiological assay and HPLC were correlated as follows: day 1:r = 0.59 (P less than 0.001). All children (100%) were cured and efficacy of cefotaxime was excellent in 72 cases (96%).(ABSTRACT TRUNCATED AT 250 WORDS)

In 11 neonates of diabetic mothers echocardiography was systematically performed and led to the discovery of 6 cases with hypertrophic cardiomyopathy. This abnormality, which may induce cardiorespiratory failure, is usually benign and consists of systolic murmur and transitory cardiomegaly; all these symptoms spontaneously regress within a few weeks. This cardiomyopathy may present with obstructive signs requiring treatment with beta adrenergic blocking agents; however, it is more often pure and needs no treatment. It may be observed after pregnancies that were not well supervised and might be induced by fetal hyperinsulinism.

We report the result of CRP and ORM assays performed at the admission in the unit, in 157 neonates (NN). The NN were divided into 3 groups: 122 "controls" (group I), 21 having septicemia with or without meningitis but with symptoms of infection (group II), 16 having a positive blood culture without symptom of infection (group III). In group I, the CRP level does not depend on gestational age at birth, neither on the age at the assay. The level of ORM depends on both factors. Neonatal infection leads to a very significant rise of the CRP rate (often above 100 mg/l). The rise of ORM is not so dramatic. These data suggest that the assay of these two proteins are useful in the diagnosis of neonatal infection if they are performed together and at several times.

Case report of a girl with fibrous osteitis lesions associated with clinical and biological symptoms of pseudo-hypoparathyroidism: hypocalcemia, hyperphosphoremia, absence of increase of phosphaturia (urine phosphate level) and especially of cyclic AMP after parathormone injection, high serum parathormone level. Similar cases described in literature are shortly analyzed. The genetic, pathogenic and nosologic problems of such an association are reviewed.

The occurrence of brain damage in 57 full term neonates was investigated. The infants were submitted to a prospective protocol involving clinical (neurological and electroencephalographical) and biological examinations, including the determination of the fibrin-fibrinogen degradation products in the blood and cerebrospinal fluid on D1, D3, D8 and D15. Psychomotor events were followed up for a year. Traumatic lumbar taps did not disturb the results significantly. Blood samples were not found to contain noticeable amounts of FDP. Conversely, the existence of FDP in CSF was not infrequent near to delivery and was shown to be closely related to brain damage as evaluated by the psychomotor events.

Very low plasma levels of thyroxine with a parallel decrease in the free thyroxine index were observed in 8 low birth weight infants. The tri-iodo-thyronine levels were slightly but significantly reduced and there was a concommitant increase in thyrotropin concentrations. The abnormalities, which were usually seen between the 10th--45th day, were transitory and resolved completely. The possible role of topical iodinated products is discussed.

A prospective study was made of levels of lactate and hydroxybutyrate dehydrogenase in the blood and cerebrospinal fluid of 57 term neonates, in relation to the time elapsed since delivery, neonatal examinations (Apgar score, neurological symptoms and EEG findings) and psychomotor outcome at one year of age. Serum determinations revealed no significant relationship with clinical assessments. CSF measurements showed significant differences near to the time of delivery between the normal and handicapped groups at age one, which supports the hypothesis of a causal link between neonatal brain damage and CSF enzyme levels.

The authors report a double-blind study of 57 full-term newborn infants prospectively subjected to clinical, electroencephalographical, blood and cerebrospinal fluid, and developmental examinations. Four enzymatic activities were measured in blood and CSF: aminotransferase (ASAT), creatine kinase (CK), lactate dehydrogenase (LD) and hydroxybutyrate dehydrogenase (HBD). Close relationships of enzymatic levels with psychomotor outcome are reported. In blood, ASAT and CL seemed to be the most important determinations, allowing threshold-values to be suggested. In CSF, LD and HBD were the determinations the most closely related to psychomotor events at age one. This method seems to be of theoretical as well as practical importance in evaluating neonatal brain injury.

A case of Farber's lipogranulomatosis is described in an 18 month-old girl. There was clinical evidence for diagnosis, which was confirmed by a ceramidase activity assay on cultured fibroblasts. A study of the conjunctiva by electron microscopy was performed. The authors emphasize the clinical and biological characteristics of such cases.

Two cases of spinal cord injury involving the lower cervical region are reported. In both, the delivery of a breech presentation was difficult. Severe hypotonia, areflexia and autonomic nervous system disturbances suggested a spinal cord injury. The evidence, type, and location of the cord lesion were given by nuclear magnetic resonance (NMR).

A systematic analysis was made of the autopsies of 74 newborns and fetuses (49 pathological cases and 25 controls) to detect defects in pulmonary growth. Radial alveolar count (RAC) and histological study were made in each case, as well as measurement of the ratio of pulmonary weight to total body weight (R). The ratio is of interest when lower than 0.012, but has no value if there is an intercurrent pathology. RAC decreases in pulmonary hypoplasia, but is not an entirely reliable indication since there is evolution of the values as pregnancy advances and a greater dispersion of results the earlier the gestational age. Histology disclosed an excess of bronchi, including distal bronchi, some of which exhibited delay in the differentiation of the distal aerian channel. The analysis of any two of the three above will enable diagnosis of pulmonary hypoplasia.

A new method for carrying out the nitroblue tetrazolium test (NBT) has been used on micro-specimens taken from 116 newborn infants for the purpose of carrying out a prospective enquiry into neonatal infection. 44 children aged from 1 to 6 days were selected at random from a population that was said to be normal in the maternity unit and were the control group. 72 children of less than 48 hours of age were made to object of a prospective study of neonatal infection, an enquiry that allowed them to be divided into 3 groups: non-infected, infected and suspect. The study showed a very strong correlation between raised scores (higher than 100) and the presence of an infection, whereas scores in the controls and the non-infected infants were low. This test, which is hardly invasive and easy to introduce as a routine when carried out early is a good screening test for infection and can be best used on the newborn population in a maternity unit.

Surgical exploration of a 7-week-old infant with a diagnosis of 'pulmonary artery sling' (left pulmonary artery arising from the right) revealed the true nature of the abnormality to be persistence of the ductus arteriosus which connected the right pulmonary artery to the aortic isthmus. This malformation has not been described previously. It has the same symptomatology as 'pulmonary artery sling' and it may be treated by surgical ligation and division. We suggest the term 'ductus arteriosus sling' to describe this rare congenital anomaly.

A 16 year old boy with Bruton type agammaglobulinaemia developed acute encephalitis. Echo virus type 3 was isolated on two occasions from the same sample of CSF. Clinical improvement occured after treatment with gammaglobulin with high anti-Echo virus titers given intramuscularly and intrathecally. However the C.S.F. protein is still raised so that it is not certain he has been completely cured.

Report of a case of renal papillary necrosis in an infant, following acute dehydration with cardio-vascular failure. Evolution was favourable and diagnosis was established retrospectively by I.V. urogram.

The authors report a double blind study on 57 full-term neonates prospectively subjected to clinical, electroencephalographical, cerebrospinal fluid and developmental examinations. Usual neonatal pleiocytosis depends on histiomonocytic cells which probably are a reflection of constant small brain damage during delivery. Infants suffering neurological sequelae at age one are recognizable as early as the 60th to 84th hours of life in view of persisting high histiomonocytic counts greater than 10 M. elements/l) and granulocytic peaks (greater than 2 M. elements/l) in clear samples. This method is then of interest, despite its invasive nature and limits (traumatic punctures, time-limits).

Results of a prospective study on neonatal brain damage are reported. 57 full term newborns were subjected to clinical examination with a one year follow-up. Blood and CSF were sampled for FDP determinations on days J1, J3, J8 and when necessary, J15. Traumatic punctures do not alter results. FDP measurement were negative in the whole blood samples and most CSF ones. High FDP values in early samples are linked to brain damage and values above 4 micrograms/ml are significantly correlated to neurological sequelae.