Administration of steroids in pediatric cardiac surgery: impact on clinical outcome and systemic inflammatory response.

Cardiopulmonary bypass (CPB) is associated with a systemic inflammatory response. Pre-bypass steroid administration may modulate the inflammatory response, resulting in improved postoperative recovery. We performed a prospective study in the departments of cardiovascular surgery and pediatric intensive care medicine of two university hospitals that included 50 infants who underwent heart surgery. Patients received either prednisolone (30 mg/kg) added to the priming solution of the cardiopulmonary bypass circuit (steroid group) or no steroids (nonsteroid group). Clinical outcome parameters include therapy with inotropic drugs, oxygenation, blood lactate, glucose, and creatinine, and laboratory parameters of inflammation include leukocytes, C-reactive protein, and interleukin-8. Postoperative recovery (e.g., the number, dosage, and duration of inotropic drugs as well as oxygenation) was similar in patients treated with or without steroids when corrected for the type of cardiac surgery performed. After CPB, there was an inflammatory reaction, especially in patients with a long CPB time. Postoperative plasma levels of interleukin-8 were correlated with the duration of CPB time (r = 0.62, p < 0.001). Administration of steroids had no significant impact on the laboratory parameters of inflammation. Administration of prednisolone into the priming solution of the CPB circuit had no measurable influence on postoperative recovery and did not suppress the inflammatory response.

Racial differences in newborn intensive care morbidity in Alaska.

Birthweight-specific neonatal mortality for Alaska Natives is higher than for non-natives for the years 1987-1996. We investigated the reasons for this based on Level III Neonatal Intensive Care Unit information available from 1991-1996. We also investigated whether differences in mortality extended to measures of morbidity. There were less Native patients born at the tertiary care center for babies with birthweight < 1500 grams and 1500-2499 grams (64% for Natives and 87% for non-natives, p = .000). Differences in antenatal referral were only apparent for the population residing within the Anchorage/Mat-Su area. There were also less cesarean deliveries for Native infants that were born outside of the tertiary care center for both birthweight categories (25% for Native vs. 53% for non-native infants < 1500 grams, p = .01; 27% for Native vs. 48% for non-native infants 1500-2499 grams, p = .01). For Alaska Native babies < 1500 grams there was more necrotizing enterocolitis (13% in Native vs. 4.9% in non-native, p = .01), more severe retinopathy of prematurity (12% in Native vs. 4.6% in non-native, p = .01), and more bronchopulmonary dysplasia (49% in Native vs. 34% in non-native, p = .04). For Alaska Native babies 1500-2499 grams that needed ventilatory assistance there was more intraventricular hemorrhage (19% in Native vs. 7.4% in non-native, p = .003), more severe (grade 3-4) intraventricular hemorrhage (9.5% in Native vs. 0.9% in nonnative, p = .001), and more acquired sepsis (7.1% in Native vs. 1.7% in non-native, p = .02). Differences in access to Level III perinatal care and intrapartum care (cesarean delivery rates) are likely factors that contribute to the worse outcomes in the Alaska Native population.

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.

Common anorectal conditions: Part II. Lesions.

Patients with a wide variety of anorectal lesions present to family physicians. Most can be successfully managed in the office setting. A high index of suspicion for cancer should be maintained and all patients should be questioned about relevant family history or other indications for cancer screening. Patients with condylomata acuminata must be examined for human papillomavirus infection elsewhere after treatment of the presenting lesions. Their sexual partners should also be counseled and screened. Both surgical and nonsurgical treatments are available for the pain of anal fissure. Infection in the anorectal area may present as different types of abscesses, cryptitis, fistulae or perineal sepsis. Fistulae may result from localized infection or indicate inflammatory bowel disease. Protrusion of tissue through the anus may be due to hemorrhoids, mucosal prolapse, polyps or other lesions.

Common anorectal conditions: Part I. Symptoms and complaints.

Anorectal symptoms and complaints are common and may be caused by a wide spectrum of conditions. Although most conditions are benign and may be successfully treated by primary care practitioners, a high index of suspicion for colorectal cancer should be maintained, and all patients should be appropriately investigated. Inspection, palpation and anoscopic examination using an Ive's slotted anoscope provide adequate initial assessment. Pruritus ani usually represents a self-perpetuating itch-scratch cycle and is uncommonly due to infection. The history, as well as the physical examination, can distinguish anal pain due to hemorrhoids, fissure, abscess, cancer or proctalgia fugax. The most frequent causes of rectal bleeding are hemorrhoids, fissures and polyps. Diagnoses associated with difficulty in passing stool can range from constipation to fecal incontinence.

Lobar pulmonary interstitial emphysema in a premature infant on continuous positive airway pressure using nasal prongs.

Unilobar pulmonary interstitial emphysema may emerge in extremely low birth weight infants without mechanical ventilation but on continuous positive airway pressure using nasal prongs.

Common anorectal conditions.

Patients with a wide variety of anorectal lesions present to family physicians. Most can be successfully managed in the office setting. A high index of suspicion for cancer should be maintained and all patients should be questioned about relevant family history or other indications for cancer screening. Patients with condylomata acuminata must be examined for human papillomavirus infection elsewhere after treatment of the presenting lesions. Their sexual partners should also be counseled and screened. Both surgical and nonsurgical treatments are available for the pain of anal fissure. Infection in the anorectal area may present as different types of abscesses, cryptitis, fistulae or perineal sepsis. Fistulae may result from localized infection or indicate inflammatory bowel disease. Protrusion of tissue through the anus may be due to hemorrhoids, mucosal prolapse, polyps or other lesions.

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.

[Changes in the epidemiology of the acute respiratory distress syndrome (ARDS) in children]. / Cambios en la epidemiología del síndrome de dificultad respiratoria aguda (SDRA) en niños.

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.

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.

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.

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.

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.

The next challenge for newborn intensive care in Alaska: improving the survival of the larger neonate.

Using information from our database, a review of mortality for the Newborn Intensive Care Unit at Providence Alaska Medical Center was conducted for 1987-1996. There has been a significant decline in mortality over the last decade (p = 0.003). An analysis of mortality by birthweight and gestational age groups demonstrated a decline in mortality (p = 0.005) for infants with birthweight < 2 kg and infants < or = 34 weeks gestation, but no change for infants > or = 2 kg and > or = 35 weeks gestation. As a result, larger and more mature babies now account for an increasing proportion of NICU deaths. For 1995 and 1996 the major contributors to mortality for the smaller neonates were respiratory distress syndrome and congenital and nosocomial sepsis/pneumonia. The major contributors to mortality for larger neonates were persistent pulmonary hypertension of the newborn, congenital heart disease, congenital diaphragmatic hernia, and primary birth asphyxia. A majority of deaths in the larger neonates were due to non-lethal causes. We contend that improved survival in the larger neonate is an important and achievable goal. The introduction of ECMO (Extracorporeal Membrane Oxygenation) for the NICU and a focused review of the neonatal cardiac program offers the best possible potential for achieving this goal.