Clinical features and neuroradiological findings of mitochondrial pathology in six neonates.

OBJECTS: We hoped to itemize the clinical and neuroradiological features of six neonates with mitochondrial disorders. METHODS: We examined a case series of six neonates. The diagnosis of mitochondrial cytopathy was made on the basis of spectrophotometric measurements of respiratory chain enzyme activities in skeletal muscle biopsy specimens. Magnetic resonance (MR) imaging was performed in all cases. CONCLUSIONS: The antenatal onset in five cases and the lack of any symptom-free interval are suggestive of fetal expression of the disease. No specific symptoms were found: arthrogryposis congenita multiplex in one, progressive hepatocellular dysfunction in three, encephalomyelopathy and cardiomyopathy in four. Complex I deficiency was found in three patients, while one patients had a defect of complex IV and the last a combined defect of complexes I and IV. Neuroradiological findings were either cerebral atrophy or white matter abnormalities of the brain stem in all cases but one and gave additional information, because clinical symptoms are not quite specific. The combination of clinical and MRI findings in neonatal cases can rule out hypoxic ischemic encephalopathy, which suggests an additional screening method to look for mitochondrial disorder.

Capgras syndrome: a clinical manifestation of watershed cerebral infarct complicating the use of extracorporeal membrane oxygenation.

Ischaemic cerebral accidents are frequent following extracorporeal membrane oxygenation (ECMO), especially after fixing the reinjection cannula in the right primitive carotid artery, which leads to an interruption in downstream flow. We describe a rare and unusual symptom of cerebral ischaemic accident that is known as Capgras syndrome. This feature is interesting because it may be documented by computed tomography (CT) scan and particular electroencephalography signals. It appears that our observation represents the first documented case of Capgras syndrome complicating ECMO. This incident emphasizes the potential hazards associated with right common artery ligature for venoarterial extracorporeal membrane oxygenation (VAECMO). In addition, it shows that this psychiatric symptom (that has been interpreted psychodynamically for many years) can have an organic basis, which should be studied.

Measurement of tracheal temperature is not a reliable index of total respiratory heat loss in mechanically ventilated patients.

BACKGROUND: Minimizing total respiratory heat loss is an important goal during mechanical ventilation. The aim of the present study was to evaluate whether changes in tracheal temperature (a clinical parameter that is easy to measure) are reliable indices of total respiratory heat loss in mechanically ventilated patients. METHOD: Total respiratory heat loss was measured, with three different methods of inspired gas conditioning, in 10 sedated patients. The study was randomized and of a crossover design. Each patient was ventilated for three consecutive 24-h periods with a heated humidifier (HH), a hydrophobic heat-moisture exchanger (HME) and a hygroscopic HME. Total respiratory heat loss and tracheal temperature were simultaneously obtained in each patient. Measurements were obtained during each 24-h study period after 45 min, and 6 and 24 h. RESULTS: Total respiratory heat loss varied from 51 to 52 cal/min with the HH, from 100 to 108 cal/min with the hydrophobic HME, and from 92 to 102 cal/min with the hygroscopic HME (P < 0.01). Simultaneous measurements of maximal tracheal temperatures revealed no significant differences between the HH (35.7-35.9 degrees C) and either HME (hydrophobic 35.3-35.4 degrees C, hygroscopic 36.2-36.3 degrees C). CONCLUSION: In intensive care unit (ICU) mechanically ventilated patients, total respiratory heat loss was twice as much with either hydrophobic or hydroscopic HME than with the HH. This suggests that a much greater amount of heat was extracted from the respiratory tract by the HMEs than by the HH. Tracheal temperature, although simple to measure in ICU patients, does not appear to be a reliable estimate of total respiratory heat loss.

[Hypoxic-ischemic encephalopathy in the full-term newborn. Contribution of electroencephalography and MRI or computed tomography to its prognostic evaluation. Apropos of 26 cases]. / Encéphalopathie hypoxo-ischémique du nouveau-né à terme. Apport de l'électroencéphalogramme et de l'IRM ou de la TDM à l'évaluation pronostique. A propos de 26 observations.

OBJECTIVES: Perinatal asphyxia complicated by hypoxic ischemic brain injury still remains the source of neurological lesions often serious and definitive. A major aim of neonatologists is to appreciate the severity of the hypoxic ischemic brain injury in the first days of life and to evaluate the forecast. The purpose of this work is to establish a relation between clinical signs, EEG, neuroimaging (MRI and CTS) and neuro-development. MATERIALS AND METHODS: 26 neonates from paediatric resuscitation unit (Hospital North, Marseille) were enrolled in a retrospective study since February 1994 to December 1997. All the newborns had at least one anamnestic criteria of perinatal asphyxia, an early electroencephalogram in the first two days of life and another between the third and the seventh day of life, and neuroimaging in the first 15 days of life: CTS in five cases and MRI in 21 cases. RESULTS: There was a good electro-clinic (P: 0.01) and prognostic (P: 0.03) correlation in patients within stage 3 of the "Sarnat classification". In the stage 2, the EEG did not provide valuable information about severity of the injury, and neuroimaging (especially MRI) allowed better prognosis. Diffuse brain injury or lesions of basal ganglia in MRI were associated, independently of clinical settings and EEG tracings, with a poor neurodevelopmental outcome (P: 0.02). The MRI was diagnostic in four cases of congenital encephalopathy complicated with neonatal suffering. CONCLUSION: Our study confirms the interest of the association of clinical settings, EEG tracings and MRI in the diagnosis and the prognostic of the hypoxic-ischemic encephalopathy in term neonate.

Preparation and use of morphine capsules in paediatric patients with burns.

Morphine is an analgesic agent used for the symptomatic relief of moderate to severe pain. The lack of oral paediatric form of morphine hydrochloride has led the pharmacy unit to develop capsules containing 1 mg of morphine. These capsules are prepared in conformity with G.M.P. and controlled by H.P.L.C. 73 burn children were treated in this study with pain suppression without adverse effects. This galenic form may be an adequate candidate for the management of paediatric patients because of its analgesic qualities and of numerous advantages.

[Hypoglossia-hypodactylia syndrome: apropos of a case with maxillo-mandibular synostosis, glossopalatine ankylosis and cleft palate]. / Syndrome hypoglossie-hypodactylie: à propos d'un cas avec synostose maxillomandibulaire, ankylose glossopalatine et fente palatine.

BACKGROUND: The hypoglossia-hypodactylia syndrome, a combination of limb hypogenesis and micrognathia is exceptionally associated with glossopalatine ankylosis and cleft palate. CASE REPORT: A twin girl from monochorionic pregnancy had hypoglossia and micrognathia with anterior maxillo-mandibular fusion, glossopalatine ankylosis and cleft palate. Her left foot was amputated distal to the talus and calcaneous; her right foot had only one phallange. The second phallange of the second finger was hypoplastic on her right hand. There was no abnormality on left hand. Her sister was unaffected. The maxillo-mandibular fusion was divided on second day. The infant was able to suckle on the tenth day. Episodes of airway obstruction occurred on cardiorespiratory recording and the patient was further monitored at home. CONCLUSION: This case suggests that the hemodynamic disorders due to multiple vascular connections from monochorionic pregnancies may have induced ischemic lesions of hypoglossia-hypodactylia. Other malformations and signs of brain stem dysfunction should be detected.

[Congenital tuberculosis. Apropos of a case disclosed after necrotizing enterocolitis]. / Tuberculose congénitale. A propos d'un cas révélé au décours d'une entérocolite nécrosante.

The authors report on the observation of a premature infant (gestational age 31 weeks, birth weight 1440 g) who experienced a necrotizing enterocolitis and a congenital tuberculosis. Congenital tuberculosis is a rare infection; transmission of Koch's bacilli is achieved through hematogenous route or more frequently through inhalation or ingestion of infected amniotic fluid. The diagnosis was done when miliary patterns were found on the mother's chest X-ray. At the same time, the newborn presented with a chronic lung disease, a poor clinical status with biological signs of inflammation. Usual antibiotics were unsuccessful. A rapid improvement was obtained with a specific antituberculous treatment.

[Neurophysiological study of propofol (2-6 diisopropylphenol): experimental data]. / Etude neurophysiologique du propofol (2-6 diisopropylphenol): données expérimentales.

Propofol was assessed in laboratory animals using an experimental technique which leads to an over all appreciation of central nervous system function. After Bimar and Naquet, (1966) a battery of neurophysiological tests in the cat investigate specific primary pathways (visual evoked potentials), non specific pathways (arousal reaction) and hemodynamic response to painful aggression (stimulation of dentine). Propofol appears to be a powerful narco-hypnotic drug. More, it have an original property which is quite rapidly effective and unconnected with narcosis: a protective hemodynamic ("sympathoplegic or neuroleptic") effect. So, propofol, a new anesthetic agent, seems to be a progress with regard to other drugs used previously.

[Fatal acute cerebral edema in a diabetic child]. / Oedème cérébral aigu mortel chez un enfant diabétique.

A case is reported of fatal acute cerebral oedema occurring in a 15-year-old child suffering diabetic ketoacidosis. He had severe gastro-enteritis, with a weight lose of 8 kg over a period of 8 days (initial weight = 50 kg). He was admitted in a stupor with pH 7.15, 129 mmol.l-1 natraemia, and 31 mmol.l-1 blood glucose concentration. Blood osmolaity was calculated to be 310 mosmol.l-1. He was rehydrated with 416 ml.h-1 normal saline and 416 ml.h-1 of 1.4% sodium bicarbonate. At the same time a total dose of 75 i.u. of ordinary insulin was given. After 2 h, the patient's condition suddenly worsened with unreactive coma, bilateral fixed mydriasis, respiratory pauses, and impairment of haemodynamic state (heart rate 150 b.min-1, blood pressure 80/50 mmHg). The diagnosis of cerebral oedema with severe intracranial hypertension was confirmed by different investigations. Despite ventilatory support and continued intensive care, the patient died a few hours later. It is concluded that some degree of subclinical brain swelling could be common occurrence during diabetic ketoacidosis, present maybe even before the start of treatment. Such cases of cerebral oedema are often reported, but the pathophysiological mechanisms remain unclear. However, unlike this case, rehydration must be moderate (less than 41.m-2.day-1), especially in case of hyponatraemia. Insulin and sodium bicarbonate must be used with care. Early rigorous clinical and biological monitoring is essential. Treatment should aim at a progressive correction of the metabolic disturbances.

[Effects of isoflurane and halothane on the auditory and somatosensory evoked potentials]. / Effets de l'isoflurane et de l'halothane sur les potentiels évoqués auditifs et somesthésiques.

The effects of isoflurane and halothane anaesthesia on brainstem auditory (BEAR) and somatosensory evoked potentials (SEP) were recorded in 15 patients; eight received isoflurane and seven halothane. Atropine alone was given as premedication. After induction of anaesthesia with thiopentone, it was maintained with 50% nitrous oxide in oxygen. Ventilation was controlled. Isoflurane and halothane concentrations were gradually increased to 3%. Recordings were made at 1%, 2.5% and 3%. There were statistically significant differences in the latencies of SEP and BEAR with increasing concentrations of anaesthetic. The BEAR waves significantly affected were wave V and the latency between peaks I to V (p less than 0.001); for the SEP, it was wave N20. SEP central conduction time was prolonged with both anaesthetics (p less than 0.001). The effects of isoflurane and halothane were similar. These results could not be explained by changes in central body temperature or end-tidal carbon dioxide concentration. The study showed a dose-related direct effect of both anaesthetic agents on the brainstem auditory and somatosensory evoked responses. It may be that the measurement of changes in evoked potentials could be a useful indicator of anaesthetic depth.

[Zinc and biotin deficiency during prolonged parenteral nutrition in the infant]. / Carence en zinc et en biotine au cours d'une alimentation parentérale prolongée chez le nourrisson.

The case of a premature hypotrophic infant who, after 4 months of parenteral nutrition, presented with deficiency in both zinc and biotin is reported. The two deficiencies had similar clinical manifestations: dermatitis, alopecia and susceptibility to infection. The diagnosis was confirmed by zinc blood level measurement on the one hand and by urinary organic acids chromatography on the other. The clinical manifestations can only be prevented by an additional intake of zinc and biotin in doses adjusted to the child's age and to the underlying pathology.

[Trial of a new technic of transcutaneous diaphragmatic electrostimulation in man]. / Essai d'une nouvelle technique d'électrostimulation diaphragmatique transcutanée chez l'homme.

A new method of diaphragmatic pacing by transcutaneous electrical stimulation, using alternative currents with frequency and amplitude modulations, was tested in 12 subjects: 3 were normal and awake (group I); 4 were studied in acute respiratory failure while under mechanical ventilation and central apnea (group II) and 5 were studied while anaesthetized for peripheral venous surgery and breathing spontaneously (group III). Stimulation was performed either during spontaneous ventilation (groups I and II) or during short periods of respiratory depression (group III). Tidal volume was measured by pneumotachography. A positive correlation was found between stimulus intensity and inspired volume. This effect was not due to voluntary control alone, since diaphragmatic stimulation could support 24% of the theoretical minute ventilation in group II subjects and 64% in group III subjects. These preliminary results demonstrate the relative efficacy of this new method of diaphragmatic pacing using computerized alternative currents.