Chronic, infantile, neurological, cutaneous, and articular syndrome in a neonate: a case report.
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Biomedical subjects
Publications and source records attributed to J Ramet.
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Orphenadrine is an anticholinergic drug used mainly in the treatment of Parkinson's disease. It has a peripheral and central effect and a known cardiotoxic effect when taken in large doses. We report the successful outcome of the treatment of a 2 1/2-year-old girl who accidentally ingested 400 mg of orphenadrine hydrochloride (Disipal). One hour after ingestion she presented neurological symptoms: confusion, ataxic walking, and periods of severe agitation. Generalized tonic-clonic seizures appeared resistant to the administration of multiple antiepileptics. They ceased after a supplementary dose of intravenous diazepam, endotracheal intubation, and mechanical ventilation. An episode of ventricular tachycardia responded well to i. v. lidocaine. Physostigmine was administered in three successive doses. The initial orphenadrine plasma level (3,55 microg/ml) was in the toxic range, associated with high mortality. The calculated elimination half-life was 10.2 h and the molecule and/or its metabolites were found up to 90 h after ingestion.
A case of invasive sino-nasal aspergillosis in an immunocompromised child is reported. Prognosis of this fulminant disease is usually poor. This paper reviews the early symptoms, clinical manifestations, risk factors, diagnosis and treatment. Awareness of the disease and early diagnosis by histological examination is essential. Treatment should consist of early administration of i.v. Amphotericin B and extensive surgical debridement.
An intravascular access line for the administration of life support drugs and volume expanders may be particularly difficult, especially in very small premature babies. We report on the successful use of an intraosseous accessline in an 800 grams preterm infant for the administration of drugs and fluid. The use and technique of an intraosseous access is an important emergency alternative which may be lifesaving, even in very preterm babies, when other methods fail.
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Patients expected to develop life-threatening complications in acute meningococcal infections require early recognition and appropriate monitoring. Different prognostic scoring systems have been developed. Three of them, chosen according to their bedside availability, were compared with our clinical observations. Twenty consecutive cases of proven meningococcal infection were admitted to the paediatric intensive care unit (PICU) of the Free University of Brussels (AZ-VUB). Biological and clinical features required for prognostic scoring were evaluated as soon as possible after admission. Glasgow meningococcal sepsis prognostic score (GMSPS), Neisseria sepsis index (NESI) and Algren criteria were retrospectively calculated and evaluated for their prognostic significance. Neisseria meningitidis was cultured from blood and cerebrospinal fluid in 11 patients and from blood in only nine patients. The age of the patients was between 1 and 15 years (mean 4.1 years). All patients received the same therapy on admission. Four patients died with a multiorgan failure within 18 hours. The three scoring systems in these four patients predicted death. Overall, the GMSPS score, the NESI score and the Algren criteria predicted death in respectively 10, nine and five patients. Death was falsely predicted in six patients by the GMSPS score, in five patients by the NESI score and in one patient by the Algren criteria. The Algren criteria predicted the severity of the clinical process more accurately than did the GMSPS and NESI scores. However, such predictability should be cautiously used even when 100% mortality is predicted. It might be used in decision-making in regard to the following issues: patient transfer to tertiary centres and mode of transportation, monitoring of patients in intensive care units, early insertion of invasive cardiovascular monitoring catheters and consideration of new or even experimental therapy. However, one should be extremely cautious of taking any therapeutically or ethical decision on the basis of one or more of the described scoring system, since we showed the lack of precision concerning the outcome of paediatric patients with meningococcaemia.
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Gitaloxin is a digitalis glycoside used for the same indications as digoxin and digitoxin. The successful outcome for a 2 1/2-year-old boy who accidentally ingested 3 mg of gitaloxin (100 times the normal therapeutic dose) is reported. At admission the child presented with irregular heart rhythm. He subsequently started vomiting, even after continuous gastric feeding. Only 48 h after ingestion of gitaloxin he became somnolent and developed bradyarrhythmia. The symptoms disappeared 96 h later; the bradyarrhythmia, however, (second-degree atrioventricular block) decreased progressively only after 120 h. The initial clinical presentation of gitaloxin poisoning may be misleading and careful observation in a pediatric intensive care unit is mandatory. A cross-reaction between the fluorescence polarization immunoassay for digitoxin and the radioimmunoassay for gitaloxin was found and was used as a helpful, but rough, estimate of the severity of gitaloxin poisoning, in the absence of a specific measurement of gitaloxin.
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UNLABELLED: Babies born after in vitro fertilisation (IVF) are increasing in number, and, although these babies are considered as very precious, no data are available regarding their risk for sudden infant death or apnoea. To evaluate the respiratory maturity of IVF babies, we evaluated the incidence of apnoea during an 8-h polysomnography in 50 consecutively presented IVF babies and in a group of 50 unselected naturally conceived babies. All infants were in good health and matched for term (born > 38 weeks of gestation), birth weight, sex and age at the time of investigation (6-11 weeks post term, median 8.0). There were 24 twins in the IVF and 6 twins in the control group. The incidence of obstructive and isolated central apnoea was comparable in the IVF and control group. However, IVF babies had significantly more periodic breathing episodes than control babies (median 2.30 (range 0-15.30) in IVF, and 1.02 (range 0-11.2) in control babies; P < 0.01). This difference was not related to the higher number of twins in the IVF group. Single IVF babies had significantly more short central apnoeas (5-10 s) than IVF twins (5-10 s) (mean 38.80 +/- 18.63 and 22.33 +/- 13.35; P < 0.001). This difference between single and twin babies was not found in the control group. CONCLUSION: IVF babies have more periodic breathing episodes indicating an immature respiratory pattern than normally conceived babies.
We describe the case of an adolescent who developed a severe but fully reversible cardiac dysfunction with low blood levels of carboxy haemoglobin (COHb = 10%) after a prolonged exposure to carbon monoxide. A 15-year-old male was admitted with a Glasgow Coma Scale of 8/15 with suspected postictal state and postanoxic encephalopathy. The cardiorespiratory failure which he developed soon after admission mandated mechanical ventilation, inotropic support and ultimately left ventricular support by intra-aortic balloon counterpulsation. The cardiac dysfunction was documented by radionuclide imaging and echocardiography. The patient fully recovered without neurological deficit. A low blood COHb concentration is a poor safety indicator since high tissue levels of accumulated carbon monoxide can be associated with coma and fulminant cardiorespiratory failure requiring advanced life support facilities.
Because the state of alertness exerts a profound influence on autonomic cardiac control, we hypothesized, that the heart rate response to a vagal stimulus, i.e., ocular compression, may differ during different states of alertness. We studied 8 healthy infants with a postconceptional age of 35-41 weeks (mean +/- SD 37.9 +/- 2.1 weeks). They underwent a standardized ocular compression test during polygraphically controlled wakefulness, rapid eye movement (REM) sleep, and non-REM (NREM) sleep. The R-R intervals were measured (1) during the 60 s preceding the ocular compression test, to determine the mean control R-R interval, and (2) during compression. Percent R-R interval was defined as the longest R-R interval in milliseconds during the test divided by the mean control R-R interval and multiplied by 100. The longest R-R interval during the test was significantly greater in REM sleep than in wakefulness (p < 0.05) and in NREM sleep (p < 0.01):939 +/- 360, 623 +/- 355, and 538 +/- 60 ms, respectively. The percent R-R interval was significantly greater in REM sleep than in NREM sleep (p < 0.01):236 +/- 91 and 129 +/- 16, respectively. The time from the longest R-R interval to return to mean control R-R interval, i.e., vagal escape, was significantly shorter in REM sleep than in NREM sleep and in wakefulness (p < 0.01): 843 +/- 168, 2,131 +/- 712, and 2,078 +/- 913 ms, respectively. This study indicates that the state of alertness should be defined when performing tests on autonomic reflexes in infants.
Nosocomial pansinusitis (N P) is most often described in nasotracheally intubated patients with craniocerebral or facial trauma. We retrospectively reviewed its occurrence and complications in the course of prolonged mechanical ventilation in orotracheally intubated patients without maxillofacial or cranial injuries. N P was deemed to be present when (1) CT scan showed opacification and/or air-fluid levels in the maxillary, ethmoid and sphenoid sinuses and (2) aspiration of both maxillary sinuses yielded pus, cultures of which revealed a high concentration of micro organisms. Nosocomial pneumonia and bacteremia were considered related to the N P if the organisms found in the sinus were identical to those recovered from the blood and/or the bronchi. During an 18-month study period, 38 cases of sinusitis were diagnosed. N P was present in 13 patients: 18 organisms (12 Gram-negative, 5 Gram-positive and 1 Candida albicans) were isolated. Pneumonia occurred in 8 patients, 6 with multi-resistant Pseudominas aeruginosa and 2 with methicillin resistant Staphylococcus aureus. Pseudomonas aeruginosa was isolated from the blood, lung and sinus in two patients. This study demonstrates that N P is relatively frequent in patients requiring long-term mechanical ventilation, even in the absence of predisposing factors. N P in these patients is mostly monomicrobial with multi-resistant Pseudomonas aeruginosa and Staphylococcus aureus as the main causative agents.
Cimetidine is commonly used for stress ulcer prophylaxis in intensive care patients. Cimetidine contains an imidazole structure. Similar drugs have been shown to inhibit steroid synthesis by blocking cytochrome P450-dependent reactions in the adrenal cortex. It is suggested that bolus injections of cimetidine suppress the normal corticosteroid production. This might be deleterious since a decreased cortisol response seems to be associated with increased mortality during chronic severe stress. We therefore performed a prospective, randomized, and controlled study to assess the effect of a short-term continuous infusion of either cimetidine or ranitidine, a non-imidazole H2-pantagonist, upon cortisol secretion in a cohort of hemodynamically stable intensive care patients. Twenty patients were consecutively enrolled following determined inclusion criteria and divided in three treatment groups: 6 controls, 7 cimetidine- and 7 ranitidinetreated subjects. Both cimetidine (1200 mg) and ranitidine (200 mg) were administered by infusion pump over 24 hrs. A short corticotropin test was done within 24 hrs after admission (d0) and repeated 7 days thereafter (d7). On both occasions, plasma cortisol was measured immediately before the test and 30 min afterwards. The three treatment groups presented a normal cortisol response at d0 and d7. Peak cortisol levels after stimulation did not show any significant difference for both the cimetidine and the ranitidine group, either at d0 or at d7. Moreover, this response at d0 and d7 was also not significantly different from the one observed in the controls. From this study we can conclude that one week treatment with conventional intravenous doses of cimetidine does not induce significant alterations of the cortisol response in hemodynamically stable ICU patients.
Cutaneous lesions resembling acrodermatitis enteropathica were present in two infants with methylmalonic acidemia and in one infant with propionic acidemia. All three infants were being fed a low-protein diet limited in branched-chain amino acids when the skin lesions developed. A deficiency in plasma levels of essential amino acids, particularly isoleucine, was confirmed. Supplementation of the diet with isoleucine in one of the patients led to a prompt improvement of the skin lesions. We conclude that dietary deficiencies associated with the treatment of organic aciduria should be added to the causes of acrodermatitis enteropathica-like cutaneous lesions.
Gastroesophageal reflux associated with apnea and/or bradycardia induces both dilatation of the esophagus and abrupt intrusion of acid content in it. To evaluate the relative importance of these two types of stimulation on cardiac and respiratory responses, a series of experimental protocols were designed, in which we evaluated the influence of esophageal dilatation alone, acid infusion alone and both stimulations simultaneously. The passage of a volume in the lower third of the esophagus (n = 14) during reflux was simulated by balloon dilation; the passage of acid gastric juice during reflux was simulated by an esophageal acid infusion test, within the physiological range (n = 8; duration = 5 min; pH = 2.2). Cardiac, respiratory and arousal responses were compared during a control period and during the distal balloon dilatation and esophageal saline and acid infusion periods. Distal esophageal balloon dilatation and acid infusion induced significant prolongation of the RR interval and of the duration of the respiratory cycle. The presence of the esophageal catheter did not lead to continuous vagal stimulation. Simultaneous stimulation by balloon dilatation and acid infusion provoked cardiac responses that were above the level reached by each stimulation separately. Behavioral and electroencephalographic observations demonstrate significantly more frequent and longer arousal responses during the acid infusion than during control and saline infusion periods. We conclude that distal esophageal balloon dilatation and acid infusion elicit significant cardiac and respiratory responses in newborns during active sleep.(ABSTRACT TRUNCATED AT 250 WORDS)