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Biomedical subjects

J Pfenninger

Publications and source records attributed to J Pfenninger.

At least 19 recordsLinked to original sources
PubMed2001

Survivors with bad outcome after hypoxic-ischaemic encephalopathy: full-term neonates compare unfavourably with children.

UNLABELLED: Hypoxic-ischaemic encephalopathy (HIE) is of major importance in neonatal and paediatric intensive care with regard to mortality and long-term morbidity. Our aim was to analyse our data in full-term neonates and children with special regard to withdrawal of life support and bad outcome. PATIENTS: All patients with HIE admitted to our unit from 1992-96 were analysed. Criteria for HIE were presence of a hypoxic insult followed by coma or altered consciousness with or without convulsions. Severity of HIE was assessed in neonates using Sarnat stages, and in children the duration of coma. In the majority of cases staging was completed with electrophysiological studies. Outcome was described using the Glasgow Outcome Scale. Bad outcome was defined as death, permanent vegetative state or severe disability, good outcome as moderate disability or good recovery. RESULTS: In the neonatal group (n = 38) outcome was significantly associated with Sarnat stages, presence of convulsions, severely abnormal EEG, cardiovascular failure, and multiple organ dysfunction (MOD). A bad outcome was observed in 27 cases with 14 deaths and 13 survivors. Supportive treatment was withdrawn in 14 cases with 9 subsequent deaths. In the older age group (n = 45) outcome was related to persistent coma of 24-48 h, severely abnormal EEG, cardiovascular failure, liver dysfunction and MOD. A bad outcome was found in 36 cases with 33 deaths and 3 survivors. Supportive treatment was withdrawn in 15 instances, all followed by death. CONCLUSIONS: Overall, neonates and older patients did not differ with regard to good or bad outcome. However, in the neonatal group there were significantly more survivors with bad outcome, either overall or after withdrawal of support. Possible explanations for this difference include variability of hypoxic insult, maturational and metabolic differences, and the more compliant neonatal skull, which prevents brainstem herniation.

Child, Preschool↗
PubMed1999

Bronchodilator responsiveness in a ventilator-dependent infant with severe tracheobronchomalacia.

A neonatal case of severe, ventilator-dependent tracheobronchomalacia (TBM) is described. The extent of the malacic segment was determined by endoscopy and tracheobronchography. Additionally, relevant and ever increasing reversible peripheral airway obstruction was documented by measuring the mechanical properties of the respiratory system before and after salbutamol. With the combination of endoscopically guided aortopexy and salbutamol infusion, the infant was eventually weaned from mechanical ventilation at the age of 86 days. We speculate that in ventilator-dependent infants with severe TBM the determination of bronchodilator responsiveness may have clinical consequences.

Albuterol↗
PubMed1999

[Changes in the epidemiology of the acute respiratory distress syndrome (ARDS) in children].

OBJECTIVE: Our aim was to analyze, in a retrospective study, changes in acute respiratory distress syndrome (ARDS) within the same pediatric intensive care unit by using the same diagnostic criteria as published in 1982. PATIENTS AND METHODS: Fifteen patients (mean age 5.1 years, range 16 days-15 years) admitted between 1988 and 1994 fulfilling our former criteria for ARDS were included in the study. RESULTS: The incidence of ARDS after the age of 7 days was 0.45% of all admissions between the age of 1 week and 16 years vs 1.79% in the former series of patients (p < 0.001). Thus, the yearly rate of ARDS decreased from 5.7 to 2.1 cases per year. Six patients suffered a chronic underlying disease vs none in 1982 (p < 0.01). Triggering of ARDS by infection/inflammation was present in 14/15 patients vs 7/20 in the first series (p < 0.001). Except for the nadir PaO2/FiO2 ratio (54 mmHg vs 97 mmHg, p < 0.01), and duration of FiO2 > or = 0.5 (204 h vs 39 h, p < 0.01) there was no statistically significant difference with regard to respiratory data. Incidence of multiple organ/system failure and numbers of failing organs/systems remained unchanged. Eight of 15 patients died in the actual series vs 8/20 in 1982 (not significant). CONCLUSIONS: Compared to our former data, the incidence of ARDS has decreased. Although the number of patients with severe chronic disease has increased, mortality remains statistically unchanged. Infection/inflammation is currently the predominant event triggering ARDS. Judging by the PaO2/FiO2 ratio and duration of FiO2 > or = 0.5, pulmonary involvement is more severe. The number of failing organs/systems remains nearly twice as frequent in non-survivors compared to survivors.

Acute Disease↗
PubMed1998

Partial 3-methylcrotonyl-CoA carboxylase deficiency in an infant with fatal outcome due to progressive respiratory failure.

UNLABELLED: Isolated partial 3-methylcrotonyl-CoA carboxylase (MCC) deficiency has been described to be the cause for a distinct relatively mild clinical picture in a single patient. We describe another patient with isolated partial MCC deficiency who suffered from failure to thrive, muscular hypotonia and progressive respiratory insufficiency with fatal outcome at the age of 6.5 months. MCC deficiency was suspected at 3 months of age on the basis of mildly elevated urinary excretion of 3-hydroxyisovaleric acid and 3-methylcrotonylglycine and confirmed by enzyme analysis in lymphocyte and fibroblast homogenates. Residual MCC activity in lymphocytes was 25% of the mean normal value. Residual activity in fibroblasts was lower than in lymphocytes (3.8% of mean normal) and not significantly different from that in patients with complete MCC deficiency. However, the residual incorporation of 14C-isovalerate into macromolecules in intact fibroblasts, was clearly higher (28% of mean normal) than in fibroblasts with complete MCC deficiency (<4%). In both patients with partial deficiency the residual MCC activity was higher in lymphocytes than in fibroblasts. Clinical symptoms and signs in our patient attributable to MCC deficiency include muscular hypotonia, failure to thrive (already present at birth), progressive respiratory failure due to diaphragmatic paresis and a moderate brain atrophy. The clinical presentation was more severe than in many patients with complete MCC deficiency. Dietary therapy was biochemically effective as shown by normalization of organic acid excretion, however, had no effect on the CNS symptoms. CONCLUSION: We speculate that the severity of the disease could be related primarily to deficiency of MCC activity in the brain. Variable MCC activity among various organs may explain the peculiar clinical picture in this patient.

Brain↗
PubMed1997

Initial and follow-up MRI in a case of early diagnosed Reye's syndrome.

Early MRI in a case of clinically established Reye's syndrome confirmed CT findings of compressed ventricles and additionally demonstrated signal alterations in the thalamus, mesencephalon and pons. On follow-up MRI the pontine lesion had vanished by 1 week later, while the thalamic lesion persisted for more than 2 months. The patient, however, recovered without neurological sequelae.

Adolescent↗
PubMed1997

Cesarean deliveries: when is a pediatrician necessary?

OBJECTIVE: We evaluated the need for vigorous resuscitation (bag-and-mask ventilation, tracheal intubation, and cardiopulmonary resuscitation) in certain common cesarean deliveries at term to evaluate the need for pediatrician attendance on behalf of the fetus. METHODS: Records of singleton cesarean deliveries (repeat, nonprogressive labor, fetal malposition, fetal heart rate abnormality) at term over 2 years were reviewed for the following: need for vigorous resuscitation, Apgar scores, anesthesia used, and the need for newborn intensive care. The next consecutive, uncomplicated singleton vaginal delivery in each case was used to create a control group. Exclusion criteria included the presence of maternal disease (diabetes, pregnancy-induced hypertension, placenta previa) or suspicion of fetal abnormalities (growth restriction, congenital defect, known meconium staining of the amniotic fluid). There were 834 cesarean deliveries and 834 controls (low-risk vaginal deliveries). RESULTS: Compared with vaginal deliveries, Apgar scores of 6 or less at 1 minute were more frequent in all cesarean deliveries except for the repeat cesarean category. The incidence of needing vigorous resuscitation was as follows: vaginal 1.7%, repeat 3.0%, nonprogressive labor 4.8%, fetal malposition 11.2%, and fetal heart rate abnormality 17.7%. The use of regional anesthesia reduced the need for vigorous resuscitation in cesarean deliveries for the repeat group and the group with nonprogressive labor without fetal heart rate abnormalities to a level similar to that in uncomplicated vaginal deliveries (2.1% repeat; 1.6% nonprogressive labor without fetal heart rate abnormality). CONCLUSIONS: Both repeat cesarean deliveries and cesareans done for nonprogressive labor without signs of fetal heart rate abnormality, when performed under regional anesthesia, may not need a pediatrician in attendance because of minimal fetal risk.

Adult↗
PubMed1997

Malformation and stenosis of the cricoid cartilage in association with Larsen's syndrome.

Three patients with the typical features of Larsen's syndrome are described. All three developed severe respiratory symptoms caused by a congenital subglottic stenosis. Tracheotomy and treatment of the stenosis by means of laryngotracheoplasty resulted in complete collapse of the cricoid cartilage and the proximal tracheal skeleton. Lack of rigidity of the laryngeal and tracheal cartilages in patients with Larsen's syndrome could well be responsible for this failure. Surgical treatment consisted of resection of the stenotic and collapsed areas and end-to-end anastomosis. This therapy was eventually successful in all three patients.

Abnormalities, Multiple↗
PubMed1996

Acute respiratory distress syndrome (ARDS) in neonates and children.

ARDS remains a syndrome which despite all efforts poses problems in exact definition (cause, course and severity). Most of the existing information comes from clinical observations and uncontrolled studies and is therefore of limited value. Despite the advent of new treatment modalities mortality from ARDS has remained high and is influenced or caused by several factors like underlying disease, previous health status, presence of MOSF, complications of therapy or ultimate failure of gas exchange. Therapy is directed at elimination of the cause of ARDS if possible, but then mainly supportive, considering all organs and systems. With the introduction of gentler respiratory support techniques (small tidal volumes and pressure limitation, permissive hypercapnia and HFO) and appropriate measures to reduce oxygen toxicity (titration of PEEP, possibly NO), iatrogenic lung injury, indistinguishable from ARDS, can be reduced, and this might improve survival rates. For the future, modulation of the host's inflammatory response may hold great promises for prevention and treatment of ARDS, but such strategies need to be explored with well controlled clinical trials, respecting the complexity of the issue.

Child↗
PubMed1995

In vivo proton magnetic resonance spectroscopy in a case of Reye's syndrome.

A case of a 14-year-old boy with Reye's syndrome (RS) and complete neurologic recovery is presented. 1H magnetic resonance spectroscopy was performed on days 1 (admission to ICU), 8 and 62: During the acute phase of RS substantial cerebral metabolic imbalances were observed and their normalization monitored. The spectra from day 1 featured extremely high glutamine content (approximately 18 mmol/kg excess) and low concentrations of choline compounds pounds (approximately 1 mmol/kg deficit). Also some excess lactate was present. The subsequent spectra demonstrated the return to an almost normal brain metabolite profile.

Adolescent↗
PubMed1994

Once daily dosing of netilmicin in neonatal and pediatric intensive care.

OBJECTIVE: To examine a once daily dosing regimen of netilmicin in critically ill neonates and children. DESIGN AND SETTING: Open, prospective study on 81 antibiotic courses in 77 critically ill neonates and children, hospitalized in a multidisciplinary pediatric/neonatal intensive care unit. For combined empiric therapy (aminoglycoside and beta-lactam), netilmicin was given intravenously over 5 min once every 24 h. The dose ranged from 3.5-6 mg/kg, mainly depending upon gestational and postnatal age. Peak levels were determined by immunoassay 30 min after the second dose and trough levels 1 h before the third and fifth dose or after adaptation of dosing. RESULTS: All peak levels (n = 28) were clearly above 12 mumol/l (mean 22, range 13-41 mumol/l). Eighty-nine trough levels were within desired limits (< 4 mumol/l) and 11 (11%) above 4 mumol/l, mostly in conjunction with impaired renal function. CONCLUSIONS: Optimal peak and trough levels of netilmicin can be achieved by once daily dosing, adapted to gestational/postnatal age and renal function.

Critical Illness↗
PubMed1994

Respiratory syncytial virus triggered adult respiratory distress syndrome in infants: a report of two cases.

Two infants with severe respiratory syncytial virus infection which resulted eventually in classical adult respiratory distress syndrome (ARDS) are presented. Both infants had severe apneic spells, necessitating intubation and mechanical ventilation (MV). Chest radiographs changed after a few days after institution of MV from initial bronchopneumonia like pattern to severe ARDS. Assessment of respiratory system mechanics (single breath occlusion technique) revealed severe restrictive disease in both cases. The first patient recovered with residual restrictive changes determined during a follow-up 2.5 months later, whereas the second infant died because of ARDS, pulmonary interstitial emphysema and hypoxemic hypoxia.

Fatal Outcome↗
PubMed1994

[Acute respiratory distress].

Respiratory distress and respiratory failure are to be distinguished, although both terms are often used synonymously. The most frequent causes of respiratory distress are summarized, the emphasis being placed on the distinction of acute epiglottitis and croup syndrome (i.e. viral croup, spasmodic croup and bacterial laryngotracheitis). The importance of correct decisions for triage and first life-saving measures is underlined.

Acute Disease↗