Critical illness neuromuscular disease: clinical, electrophysiological, and prognostic aspects.

BACKGROUND: Critical illness neuromuscular disease, which has been recognised as a distinct clinical entity in adults, remains poorly described in children. AIMS: To assess retrospectively the clinical, electrophysiological, and prognostic features of the disease. METHODS: Retrospective study in a children's university hospital. RESULTS: Five critically ill patients presented with generalised paralysis, associated with long lasting failure to breathe in three. The cause of the generalised paralysis was critical illness neuropathy in two, acute myopathy in two, and mixed neuromyopathy in one. CONCLUSIONS: Neuromuscular disease should be suspected in critically ill children with muscle weakness. Because corticosteroids and muscle relaxants appear to trigger some types of intensive care unit neuromuscular disease in children, their use should be restricted or administered at the lowest doses possible.

[Development of intensive care for pediatric infectious diseases over the last 20 years]. / Evolution de la réanimation des maladies infectieuses pédiatriques pendant les 20 dernières années.

Intensive care and especially pediatric intensive care originated in connection with fatal forms of infectious diseases, some forty years ago. It has come a long way during the last quarter of the past century. Several infectious diseases have disappeared in France during this period such as poliomyelitis, dipteria and tetanus. Many other are receding. Nevertheless problems remain: serious infections by pneumococcus or staphylococcus, maternofoetal infections, whooping cough in very young infant. Now, thanks to liver transplantations and intensive care, the fatal forms of infectious hepatitis can be overcome in more than 50% of the cases. Meanwhile HIV appeared involving intensivists participation. Toxic shock, and especially the purpura fulminans, remains a major difficulty. Great advances in understanding its mechanisms have been made and it seems today like the result of excessive and unsuitable defence reactions. In addition, intensive care takes charge of more and more vulnerable ill persons: immunosuppressed, extreme premature babies, children who had to undergo heavy operations. In those cases, intensive care faces superinfections, nosocomial infections, multi-resistant germs, related in part to the particular working and environmental conditions and an unsuitable use of antibiotics. To conclude, it is necessary to optimize the technical and working conditions in intensive care units, to strictly observe the well established regulations of hygienics and to develop vaccinations.

Peliosis hepatis with initial presentation as acute hepatic failure and intraperitoneal hemorrhage in children.

Peliosis hepatis, a condition characterized by the presence of blood-filled lacunar spaces in the liver, usually has a chronic presentation pattern and is mainly reported in adult patients in association with chronic wasting disorders and after administration of various drugs. The present report concerns two previously healthy young children in whom peliosis hepatis initially presented as acute hepatic failure and who had Escherichia coli pyelonephritis. Both patients had active intraperitoneal hemorrhage from the peliotic liver lesions, and liver ultrasonography showed multiple hypoechoic areas of different sizes, which in this context should suggest the diagnosis. One child died from hypovolemic shock and the other recovered. This study indicates that acute peliosis hepatis can be a serious life-threatening disease in children.

Haemorrhagic shock and encephalopathy syndrome: neurological course and predictors of outcome.

UNLABELLED: The haemorrhagic shock and encephalopathy syndrome (HSES) is a devastating disease. The aetiology of this syndrome is unknown, and, despite intensive treatment, the outcome is often fatal or associated with severe neurological sequelae. OBJECTIVE: To assess the neurological features and potential prognostic markers of the disease. DESIGN: Retrospective study. SETTING: Division of Neuropaediatrics in a children's university hospital. PATIENTS AND METHODS: Fourteen patients fulfilling the HSES criteria out of 42 children admitted with fever and shock to the Paediatric Intensive Care Unit between 1986 and 1994, were analysed for clinical, biological, neuroradiological, EEG and neuropathological findings. RESULTS: The patients (age range from 2 to 33 months) were found at night or in the morning either comatous (n = 3) or convulsing (n = 11). All but one were healthy before admission, although eight had had a brief prodromal infectious disease. All were febrile (mean body temperature 39.9 degrees C +/-0.9 degrees). Seasonal clustering during the winter months was observed. Coma and seizures with frequent status epilepticus were the main neurological manifestations. All children recovered from their multiple organ failure within a few days. Seven died (50%); four survivors had neurological sequelae (29%) with a developmental quotient (DQ) of 50% or less in three and a DQ of 75% in one and three infants (21%) had normal outcomes. Computed tomography (CT) displayed a diffuse area of low density mainly in the cerebral cortex and intraventricular and parenchymal haemorrhages. Magnetic resonance imaging (MRI) showed haemorrhagic cortical lesions. Postmortem examination of the brain conducted in three patients showed necrotic and haemorrhagic lesions, mainly in cortical areas. Comparison of the children with adverse outcome (death or neurological sequelae) with those with normal outcome revealed that predictors of poor outcome were status epilepticus (p = 0.003) and coma for more than 24 h (p = 0.01). Infants without disseminated intravascular coagulation, without a biphasic course and without brain hypodensities or haemorrhages on CT scans performed at least 4 days after onset had a normal neurodevelopmental outcome. CONCLUSION: The central nervous system appeared to be the main target of the HSES lesions. The most common outcome was brain death or severe brain damage. Further studies with a larger sample are necessary to determine whether the prognostic indicators we identified are reliable.

[Organ procurement from cardiac arrest donors: outcome of kidney grafts and recommendations]. / Prélèvements d'organes sur donneurs décédés à coeur arrêté: devenir des greffons rénaux, propositions.

OBJECTIVES: To compare literature data with results obtained with organs procured from donors who died from cardiac arrest and to make proposals for this mode of organ procurement in France. METHODS: Over the last 10 years, 10 organ donors (2%) among a series of 486 donors in a state of brain death, had died of cardiac arrest. The arrest were perfused with double-balloon catheters. The outcome of the subsequent kidney grafts was compared with data in the literature. RESULTS: Fifteen of the 18 kidneys from cardiac arrest donors were functioning 1 month after implantation compared with 17 of the 20 kidneys from braindeath donors with beating hearts. The rate of acute tubular necrosis was 55% in the cardiac arrest kidneys and 40% in the beating-heart kidneys. Serum creatinine at 1 yeart was 145 +/- 69 mumol/l 17 +/- 29 mumol/l respectively. DISCUSSION: These results and those reported in the literature demonstrate that kidney procurement from cardiac arrest donors is feasible. If intensive care and surgery units are well organized, this type of organ procurement could provide a larger number of organs for transplantation. Emergency teams must be available for preparing and transferring the organs.

A control programme for MRSA (methicillin-resistant Staphylococcus aureus) containment in a paediatric intensive care unit: evaluation and impact on infections caused by other micro-organisms.

Methicillin-resistant Staphylococcus aureus (MRSA) is increasingly reported as a hospital-acquired pathogen in intensive care units (ICUs). The inconsistent application of hygiene measures by healthcare workers accounts largely for the epidemic dissemination of such resistant strains. The efficacy of a control programme to prevent spread of MRSA was assessed in our paediatric ICU (PICU) from April 1992 to December 1995. Patients initially had weekly MRSA cultures taken from samples of anterior nares and perineum, but from January 1994, cultures were also obtained upon admission. Immediately after notification, all MRSA carriers were isolated. Education of hospital staff was an essential component of our programme. Nosocomial infection rates were recorded retrospectively in 1992 and 1993, and prospectively in 1994 and 1995. Incidence rates between 'pre-programme' and 'programme' periods were compared. The rate of MRSA infection decreased from 5.9-0.8/1000 Patient-Days (PD), (P < 10(-7). MRSA carriage also decreased from 34-2% (P < 10(-9) and the ratio of MRSA to all S. aureus fell from 71-11% (P < 10(-4). The decrease in the global incidence of infection from 20.1-13.9/1000 PD (P = 0.002) was due only to the decrease in MRSA infection. However, between 1994 and 1995, there was a significant increase in the number of transplant patients despite a constant patient/nurse ratio. The nosocomial infection rates caused by other micro-organisms decreased among the transplant patients from 64.8-33.2/1000 transplanted PD (P = 0.009) between 1994 and 1995. At the same time, we observed a slight increase of infections in non-transplanted patients, which may have been due to the effect of increased overall workload on those patients who were supposed to have fewer nosocomial risk factors. We conclude that implementation of infection control measures directed towards limiting person-to-person spread was effective in controlling high MRSA infection rates in a PICU, but it is important to allow enough time for staff to carry out hygiene practices thoroughly.

Acute inflammatory demyelinating polyradiculopathy in children: clinical and electrodiagnostic studies.

Clinical and electrophysiological features in 43 children with acute inflammatory demyelinating polyradiculoneuropathy (AIDP) were retrospectively studied. More than one-third of these children were less than 3 years old. Some distinctive clinical features specific to adults or to children were identified. Initial symptoms such as ataxia and severe limb or back pain were more frequent in children. By using the criteria suggested here, according to our neurophysiological findings, the diagnosis of AIDP could be proposed as early of the first days of illness in 90% of the children and is confirmed during the second week. The neurophysiological evolution was very similar in children and adults except that recovery occurred sooner in children. Prognosis was better in childhood (complete recovery in all but 2 patients with minor disabilities).

High dose methylprednisolone in severe acute transverse myelopathy.

No effective treatment has been shown for patients with acute transverse myelopathy. In an open study five children with severe acute transverse myelopathy were treated with intravenous methylprednisolone and compared with a historical group of 10 patients. The results show that in the methylprednisolone treatment group compared with the historical group of 10 patients: the median time to walk independently was significantly reduced (23 v 97 days); the proportion of patients with a full recovery within 12 months was significantly higher (80 v 10%); all patients had complete motor recovery within one year in contrast with only two of 10 patients in the historical group; and serious adverse effects did not occur. This pilot study suggests that high dose methylprednisolone is effective in the treatment of acute transverse myelopathy.

[Intraventricular hemorrhage and parenchymatous ischemia in the newborn at term. Report of five cases]. / Hémorragie intraventriculaire et ischémie parenchymateuse chez le nouveau-né à terme à propos de cinq cas.

Although intraventricular hemorrhage associated with cerebral ischemia without severe perinatal asphyxia is rare in full-term newborns, it can be severe, have early or late onset depending on the etiology and be of poor prognosis. Five full-term neonates (37 to 41 weeks of gestational age) without criteria of severe perinatal asphyxia were admitted to the intensive care unit for seizures: four were between seven and 11 days of age and one was only 12 h old. Clinical or electroclinical seizures recorded by continuous EEG monitoring were numerous, leading to status epilepticus in three babies. They were unilateral (at the level of the left hemisphere) in one infant and have not been recorded in the fourth case. Past-ictal EEG abnormalities were numerous rolandic or temporal slow or fast sharp waves of variable polarity. Cranial CT scans showed uni- or bilateral intraventricular hemorrhage with dilatation and subcortical or periventricular ischemic lesions with hemorrhage. Four out of the five infants died during the neonatal period.

Determinants of life span after Kasai operation at the era of liver transplantation.

The aim of this work is to determine the influence of age, extrahepatic biliary lesions pattern (EHBP) and association to polysplenia syndrome (PS) on 10 years outcome of 164 patients with biliary atresia (BA) treated from 1984 to 1992 by initial Kasai operation (KO) and secondary liver transplantation (LT) when necessary. Actuarial crude survival without or after LT (CS), actuarial survival with native liver (NLS) and jaundice-free actuarial survival with native liver (JFS) were calculated from 1 to 10 years versus age (under/over 45 days), EHBP (favorable/ unfavorable) and PS (no/yes). Overall 10-year CS is 70%, overall 10-year NLS and JFS are 14%. In univariate analysis, age at KO under 46 days, favorable EHBP (BA with patent gallbladder, and/or cystic dilatation of extrahepatic bile duct, or BA restricted to choledocus), and absence of PS are significant determinants of a better outcome regarding CS, NLS and JFS. EHBP is more discriminant than age. Influence of PS in this series is redundant with that of EHBP since 11/11 patients with PS had unfavorable EHBP.

[Current aspects of acute respiratory distress syndrome in children]. / Aspects actuels du syndrome de détresse respiratoire aiguë de l'enfant.

Acute respiratory distress syndrome (ARDS) is a frequent condition in pediatric intensive care units. The mortality remains high despite advances in conventional mechanical ventilation and aetiological treatment. Several animal studies have documented lung injury during mechanical ventilation with high tidal volume, and clinical investigations have shown that in human ARDS, most ventilation is distributed to the small areas of remaining aerated lung resulting in overdistension of these areas and lung injury ("baby lung" theory). Nevertheless the usefulness of extrapulmonary gas exchange remains much debated. New ventilatory strategies have been developed in order to reduce ventilator-induced lung injury and to improve systemic oxygenation but multicentric randomized clinical trials are needed before these strategies can be validated.

[Agranulocytosis after application of silver sulfadiazine in a 2-month old infant]. / Agranulocytose après application de sulfadiazine argentique chez un nourrisson de 2 mois.

BACKGROUND: A granulocytosis associated with the use of silver sulfadiazine has been described in burn patients. This agranulocytosis may be due to either an allergic reaction or a bone marrow toxicity by silver sulfadiazine. CASE REPORT: A 1-month old baby was hospitalized in pediatric intensive care unit after a stage one repair of a tracheoesophegeal fistula. Gastroesophageal reflux and tracheomalacia appeared in the post operative period. When she was 2-months old, she developed a perineal erythema. A topical therapy was begun, with daily applications of silver sulfadiazine and ketoconazole. Five days later, agranulocytosis (granulocytes = 0.21 x 10(9).L-1) occurred. Silver sulfadiazine therapy was immediately stopped. Granulocyte count returned to normal over a few days. No other etiology than silver sulfadiazine could be found to explain agranulocytosis. The small surface of administration, the chronology of the events and the rapid correction of the disorders after silver sulfadiazine interruption argued for an immuno-allergic reaction to this drug. Medical problems were easily controlled and the patient recovered well. CONCLUSION: This event occurred when sulfadiazine was applied in a small cutaneous area in a young baby. This may be an argument for an allergic reaction; alternatively, it could be explained by an increased cutaneous absorption of a cytotoxic drug in younger people.

[Significance of organ donor characteristics in renal function at 1 month: reflection on the allocation of renal transplant]. / Importance des caractéristiques des donneurs d'organes sur le devenir de la fonction rénale à 1 mois: réflexion sur la répartition des greffons rénaux.

In a series of 731 kidneys taken from brain-dead donors for renal transplantation, 652 were grafted, and the renal function at 1 month is known for 490 of them. The results show that some donor-related factors influence the outcome of the transplant. In particular, haemodynamic instability refractory to medical treatment is responsible for a 50 mumol increase of serum creatinine one month after transplantation and the use of UW solution during organ collection is responsible for an improvement of serum creatinine compared to other preservation solutions. Haemodynamic instability is a criterion which must be added to those already demonstrated in donors, such as the cause of death, age and sex. The authors propose that donor-related factors be taken into account in the allocation of organs in the same way as immunological factors which, at the present time, constitute the only criterion for kidney allocation.

[Homozygote CB deficiency revealed by recurrent Neisseria meningitidis infections in an adolescent]. / Déficit homozygote en C8 révélé par des infections récidivantes à Neisseria meningitidis chez un adolescent.

BACKGROUND: Meningococcal infections associated with late complement component deficiency are rarely severe and usually occur during adolescence and adulthood. We report severe manifestations in a boy in whom the first episode appeared early. CASE REPORT: A 14 year-old gypsy boy was admitted because of a febrile meningococcal meningitis that was complicated by a rapidly extensive and necrotic purpura, obnubilation and clotting abnormalities without hemodynamic anomalies. The patient was given symptomatic therapy and a 12-day course of antibiotics that resulted in rapid and complete recovery. Medical history of this patient showed that he had been admitted at the age of 3 years for a severe febrile purpura with septic shock and clotting abnormalities followed by rapid and complete recovery after symptomatic and antibiotic therapy. No germ had been then isolated. The complement system was studied 3 weeks after the second hospitalization: total hemolytic complement activity could not be detected and C2, C3 and C4 were normal. Examination of the terminal pathway-revealed total C8 deficiency. The patient received meningococcal vaccine and was discharged on oral penicillin prophylaxis. He remained healthy during the ensuing 4 years. CONCLUSIONS: Meningococcal infections associated with late complement component deficiency are generally uncomplicated but they remain potentially severe. Early screening for this late complement component deficiency should be considered after severe clinical manifestations.