The impact of a register on the management of neonatal cooling in Switzerland.

BACKGROUND: Therapeutic hypothermia following hypoxic ischaemic encephalopathy in term infants was introduced into Switzerland in 2005. Initial documentation of perinatal and resuscitation details was poor and neuromonitoring insufficient. In 2011, a National Asphyxia and Cooling Register was introduced. AIMS: To compare management of cooled infants before and after introduction of the register concerning documentation, neuromonitoring, cooling methods and evaluation of temperature variability between cooling methods. STUDY DESIGN: Data of cooled infants before the register was in place (first time period: 2005-2010) and afterwards (second time period: 2011-2012) was collected with a case report form. RESULTS: 150 infants were cooled during the first time period and 97 during the second time period. Most infants were cooled passively or passively with gel packs during both time periods (82% in 2005-2010 vs 70% in 2011-2012), however more infants were cooled actively during the second time period (18% versus 30%). Overall there was a significant reduction in temperature variability (p < 0.001) comparing the two time periods. A significantly higher proportion of temperature measurements within target temperature range (72% versus 77%, p < 0.001), fewer temperature measurements above (24% versus 7%, p < 0.001) and more temperatures below target range (4% versus 16%, p < 0.001) were recorded during the second time period. Neuromonitoring improved after introduction of the cooling register. CONCLUSION: Management of infants with HIE improved since introducing the register. Temperature variability was reduced, more temperature measurements in the target range and fewer temperature measurements above target range were observed. Neuromonitoring has improved, however imaging should be performed more often.

Proton magnetic resonance spectroscopy of the brain of a neonate with nonketotic hyperglycinemia: in vivo-in vitro (ex vivo) correlation.

Nonketotic hyperglycinemia (NKH) is an inborn error of amino acid metabolism caused by a defect in the glycine cleavage multienzyme complex resulting in high concentrations of glycine within the brain and spinal cord. Quantitative magnetic resonance spectroscopy ((1)H-MRS) allows measurement of absolute glycine concentrations within different parts of the brain in vivo. In addition, (1)H-MRS may be useful in monitoring treatment of NKH and to differentiate this disease from other disorders of glycine metabolism.

Pulmonary artery sling associated with tracheobronchial malformations.

We describe three cases of pulmonary artery slings associated with tracheal stenoses by complete cartilaginous rings and abnormalities in the tracheobronchial branching pattern. This association implicates special problems of management that are different from the simple pulmonary artery sling. Pathologic anatomy, symptoms, diagnostic procedures, and the problems of therapy are described. Considering similar cases in the literature, we conclude that thorough diagnostic evaluation of the tracheobronchial and the cardial system should be carried out in all cases of pulmonary artery sling. Simple correction of the aberrant vessel without correcting the tracheal stenosis is of no value in these cases. In some milder cases, a conservative approach is possible and probably less harmful than an operation.

The value of peritoneal dialysis in the treatment of severe ethanol intoxication in childhood revised.

We treated a girl aged 3.5 years (15 kg) with ethanol intoxication, using peritoneal dialysis. The blood ethanol concentration was 6.4 g/l (640 mg/dl; 138.9 mmol/l). It was calculated that the child drank a total amount of 67.2 g of ethanol (4.5 g/kg). The spontaneous ethanol elimination rate before peritoneal dialysis was 0.27 g/l (5.86 mmol/l) per hour; during peritoneal dialysis we found an ethanol elimination rate of 0.32 g/l (6.94 mmol/l) per hour, which was lower than expected. In childhood the ethanol elimination rate with peritoneal dialysis is only slightly faster in comparison to the high spontaneous elimination rate. We conclude that treatment of severe ethanol intoxication should include mainly the maintenance of the vital functions and the meticulous control of blood sugar levels and acid-base disturbances, especially in children. Indications for dialysis are complications caused by ethanol and resistant to supportive therapy, such as seizures, metabolic disturbances, persistent hypoglycemia and the possibility of combined intoxication with other dialysable drugs.

[Toxic shock syndrome due to osteomyelitis]. / Toxic-Schock-Syndrom (TSS) infolge Osteomyelitis.

An 11 3/4 year old girl contracted Toxic-Shock-Syndrome following osteomyelitis. The symptoms comprised a severe fall in blood-pressure with centralisation, petechiae and haematoma particularly in the lower extremities, hyperaemia of the oral mucosa and of the throat with profuse and unstaunchable bleeding in this area within in a short period of time. In order to stabilise the blood-pressure the patient had be given approx. 15 l colloidal solution (350 ml/kg) intravenously, mainly frozen-plasma. The risk of aggravation of a pulmonary failure along the lines of ARDS particularly due to the backward flow of the interstitial deposited fluid was avoided by the use of continuous veno-venous haemofiltration (CVVH).

Dopplersonographic classification of brain edema in infants.

25 infants, 23 newborns and 2 older infants with B-mode sonographic evidence of cerebral edema (gestational age of the newborns 39.1 +/- 2.1 weeks, weight 3270 +/- 672 g) were examined by means of pulsed Doppler sonography. Pulsed Doppler recordings were obtained in the anterior cerebral, internal carotid, basilar, and middle cerebral arteries. In all measured arteries the peak systolic peak endsystolic, peak enddiastolic and the time averaged mean velocities, as well as the resistance index and the pulsatility index were determined. In addition to the flow parameters the pH, pCO2, pO2, oxygen saturation and the blood pressure were measured. The flow velocities were compared with the normal values established by our group. Three different types of flow profiles and velocities could be found: Group 1: 12 infants had normal flow velocities. Group 2: 7 infants showed increased diastolic flow velocities. Group 3: 6 infants demonstrated decreased diastolic flow velocities. There were no significant differences according to gestational age, weight, pH, pCO2, pO2, oxygen saturation and blood pressure in the three groups. The outcome of the 12 children in group 1 was favourable: normal development 10; minor retardation 2. In group 2 only 1 child showed normal development; 2 infants had minor, 1 major handicaps; 2 infants died. Patients in group 3 had the worst outcome: no patient developed normally; 4 infants died; 2 severely handicapped infants showed polycystic leucomalacia and brain atrophy; 1 infant had minor psychomotoric problems.

[Ultrasound study of congenital mesoblastic nephroma]. / Die Ultraschalluntersuchung des kongenitalen mesoblastischen Nephroms.

The mesoblastic nephroma is the most common renal tumor among newborn and young infants. The tumor is benign, and metastatic growth has only been described in individual cases. Three patients suffering from histologically proven mesoblastic nephroma were examined with an ultrasonic device. Sonographically, the mesoblastic nephroma is manifest as solid and homogeneous tumor of a fine to medium coarse basic texture and of a medium echogenity which may show individual small cystic areas. The tumor cannot be delimited sharply from the healthy renal tissue although uneventful tissue which often caps the kidney is proved. A secure differential diagnosis against a Wilms' tumor is not possible if metastases cannot be detected sonographically or by means of an excretory urogram. The echopattern and the relation of the mesoblastic nephroma to the kidney in combination with an excretory urogram should, however, make the delimitation of a neuroblastoma possible.

[Cholelithiasis in the neonatal period]. / Cholelithiasis in der Neonatalperiode.

We report about a four weeks old male infant who developed jaundice with cholangitis and fever caused by cholelithiasis which could be confirmed by sonographic examination. Familiar history revealed several relatives with gallstones.

[Fatal consequences of fructose infusion in undiagnosed fructose intolerance]. / Fatale Folgen einer Fructose-Infusion bei undiagnostizierter Fructose-Intoleranz.

A 13-year-old girl with previously undiagnosed fructose intolerance was operated on for acute appendicitis. Postoperatively she received several infusions containing fructose or sorbitol. Haematemesis occurred on the fourth postoperative day, as well as tarry stools and jaundice. Blood sugar was 2 mg/100 ml, Quick test 3%, liver enzymes were markedly elevated, serum bilirubin was over 9 mg/100 ml, and there was a metabolic acidosis. Despite intensive treatment, including haemodialysis and plasmapheresis, she died on the 11th postoperative day.

[Age-dependent blood flow velocity in the cerebral arteries of newborn infants and infants measured by Doppler sonography]. / Altersabhängigkeit dopplersonographisch ermittelter Flussgeschwindigkeiten in den Hirnarterien von Neugeborenen und Säuglingen.

In 121 prematurely born infants, newborn and older infants (age 1-30 days, weight at investigation 1070-3750 g) flow velocities in the anterior cerebral artery, the internal carotid artery and the basilar artery were measured. All investigations were performed by computerised pulsed Doppler sonography (Acuson 128), with a 5 MHz transducer. Maximal systolic, endsystolic, and end diastolic flow velocities were measured as well as the pulsatility index and the resistance index. The flow velocities showed a strong positive linear correlation with age. The pulsatility indices were independent of age. For all flow parameters normal values were estimated.

[Doppler sonographic flow parameter of the anterior cerebral artery in patent ductus arteriosus of the newborn infant compared to a healthy control sample]. / Dopplersonographische Flussparameter in der Arteria cerebri anterior beim offenen Ductus arteriosus des Frühgeborenen im Vergleich zu einem gesunden Kontrollkollektiv.

33 premature infants (age: 32 +/- 3 weeks; birth weight 1,268 +/- 535 gs) with the clinical signs of patent ductus arteriosus Botalli (PDA) and a control group of 96 healthy infants (age: 37 +/- 4 weeks; birth weight 2 348 +/- 944 gs) were investigated. Pulsed doppler recordings were obtained in the anterior cerebral arteries (ACA) and compared with the flow pattern in the truncus coeliacus (TC). In all children the maximal systolic velocity (Vs), the endsystolic (Ves) and the enddiastolic velocity (Ved) and the pulsatility-index (PI) were measured. The 96 healthy premature born infants showed the following velocities: Vs: 41 +/- 12 cm X sec-1; Ves: 19 +/- 7 cm X sec-1; Ved: 10 +/- 4 cm X sec-1. The pulsatility-index was 0.74 +/- 0.08. In children with PDA all velocities were significantly lower than in the healthy control group: Vs: 31 +/- 10 cm X sec-1; Ves: 7 +/- 6 cm X sec-1; Ved: -1 +/- 5 cm X sec-1. Ved was more decreased than Vs resulting in a significant increase in PI (1.04 +/- 0.14). 22 infants with surgically proven large PDA (age: 31 +/- 3 weeks; birth weight: 1,160 +/- 467 gs) showed significant lower velocities (Vs: 34 +/- 8 cm X sec-1; Ves: 4 +/- 4 cm X sec-1; Ved: -4 +/- 4 cm X sec-1) in comparison with the healthy control group and the 11 children with small PDA (age: 33 +/- 4 weeks; birth weight: 1,494 +/- 621 gs).(ABSTRACT TRUNCATED AT 250 WORDS)

[Neonatal tricuspid insufficiency--a contribution to the differential diagnosis of the cyanotic newborn infant]. / Die neonatale Trikuspidalinsuffizienz--ein Beitrag zur Differential-diagnose des zyanotischen Neugeborenen.

A cyanotic newborn is described to illustrate and discuss the rare transient tricuspid insufficiency together with functional pulmonary atresia. Etiologically the manifold causes of perinatal asphyxia are to be taken into consideration. The clinical appearance corresponds to type A of transient myocardial dysfunction according to the classification of Rowe together with respiratory distress syndrome and heart failure. Pathogenetically the disease is based on the different histological consequences of myocardial ischaemia of the right ventricle in connection with a delayed fall of the pulmonary arterial resistance. Fetal connections lead to a pronounced right-to-left-shunt. The diagnosis is primarily established by the way of clinical and echocardiographic investigation and pulsed doppler examination. The most important differential diagnosis are in Ebstein-anomaly, obstruction and atresia of the right ventricular outflow tract. Therapy is conservative. Its aim is to improve myocardial performance and to lower right ventricular afterload. Outcome of patients with neonatal tricuspid insufficiency is uncertain and depends on the severity of myocardial ischaemia. In our patient the course has been uneventful after initial difficulties.

[Sonographic diagnosis of hypertrophic pyloric stenosis in childhood]. / Sonographische Diagnose der hypertrophen Pylorusstenose im Kindesalter.

In most cases, diagnosis of hypertrophic pyloric stenosis is now based on real-time ultrasound examination. We employed the criteria stated in literature for sonographic diagnosis of hypertrophic pyloric stenosis, to evaluate our results. We studied 15 patients with symptoms of hypertrophic pyloric stenosis via real-time ultrasound, as well as 15 healthy infants. 10 patients were boys and 5 were girls, between 19 and 83 days of age (average age 45 days). 15 healthy infants of the same age were studied (average age 41 days) for comparison. In the healthy group, the average transverse pyloric diameter was 1.0 cm (0.6-1.5 cm); the mean wall thickness was 0.24 cm (0.2-0.3 cm) and mean pyloric length was 1.29 cm (1.0-1.6 cm). Analysis of the results in hypertrophic condition showed that the mean transverse pyloric diameter was 1.6 cm (1.2-1.9 cm). The wall thickness ranged from 0.4 to 0.8 cm with an average of 0.56 cm. The muscle length ranged from 1.7 to 2.5 cm with an average of 2.1 cm. On comparing the data of both groups we found significant differences in wall thickness and length. There was no false negative scan. Sonographic diagnosis was confirmed in all cases by surgical intervention. Our results agree with those obtained by other authors.

[Diagnosis of bilateral renal vein thrombosis in infancy by pulsed Doppler sonography of the renal vessels]. / Diagnose der beidseitigen Nierenvenenthrombose im Säuglingsalter mit der gepulsten Dopplersonographie der Nierengefässe.

In a premature infant bilateral renal venous thrombosis and associated thrombosis of the inferior vena cava were diagnosed at the age of 6 weeks, using a 2-dimensional ultrasound sector scanner with integrated pulsed doppler ultrasound of the renal vessels. In contrast to 30 healthy children, who showed continuous forward flow in the renal arteries throughout systole and diastole, diastolic flow was decreased or even retrograde in renal venous thrombosis (RVT) according to the severity of thrombosis. Doppler recordings in renal veins normally showed continuous retrograde flow patterns, whereas in RVT venous flow was decreased or absent. Pulsed doppler ultrasound is a useful and noninvasive method for recording renal flow patterns, especially in renovascular disease.