[Reversal of a right-left shunt and permanent improvement of oxygenation by inhalation of nitrogen monoxide in a premature infant with lung hypoplasia and asphyxia]. / Umkehr eines Rechts-Links-Shunts und dauerhafte Verbesserung der Oxygenierung durch Inhalation von Stickstoffmonoxyd (NO) bei einem Frühgeborenen mit Lungenhypoplasie und Asphyxie.

We report the case of a 29 weeks gestation female premature infant who suffered from severe postnatal asphyxia following spontaneous vaginal delivery. Prenatally lung hypoplasia due to prematurely ruptured membranes with subsequent oligohydramnios was suspected sonographically. Echocardiography revealed right-to-left shunting via PDA and foramen ovale, in addition to that tricuspid incompetence with a pulmonary arterial pressure gradient of 40 mmHg was demonstrated. At an oxygenation index (OI) of 34, an arterio-alveolar oxygen difference (AaDO2) of 639 mmHg, an FiO2 of 1.0 and a maximal paO2 of 37 mmHg during high frequency ventilation (HFV), we applied inhaled nitric oxide (up to 70 ppm) for a duration of approximately 30 hours. Within two hours the inspiratory oxygen concentration could be weaned to an FiO2 of 0.21, mean airway pressures were reduced markedly. Echocardiographically tricuspid incompetence had disappeared, the PDA was closed and now left-to-right shunting across the foramen ovale was demonstrated. The infant was extubated on day 5 and subsequently had oxygen requirements up to an FiO2 of 0.3 during spontaneous breathing for 20 days.

Zinc deficiency in an exclusively breast-fed preterm infant.

UNLABELLED: A formerly premature, exclusively breast-fed infant with severe zinc deficiency syndrome is presented. He showed the characteristic erosive skin changes, including alopecia, as seen in acrodermatitis enteropathica. In addition, he manifested a failure to thrive and irritability. The diagnosis was confirmed by reduced serum levels of zinc (2.3 mumol/l) and alkaline phosphatase (45 U/l). We consider the reduced zinc supply in the breast milk (5.7 mumol/l) as the most likely cause of the disease. Therapy consisted of oral zinc supplements (50 mumol/kg/day) for a period of 30 weeks. Symptoms and laboratory values normalized completely and did not recur on a normal diet. CONCLUSION: A diet of breast milk can, in rare circumstances, cause insufficient zinc intake resulting in severe zinc deficiency syndrome with characteristic dermatological features. Therapy consists of temporary oral zinc supplementation at a daily dose of 50 mumol/kg.

Serum alpha 1-microglobulin and beta 2-microglobulin for the estimation of fetal glomerular renal function.

As proteins cannot cross the placenta levels of the microproteins alpha 1-microglobulin (alpha 1MG) and beta 2-microglobulin (beta 2 MG) can be used to assess fetal glomerular renal function. alpha 1MG, beta 2MG and creatinine were routinely determined in cord and maternal blood of 133 newborns [gestational age (GA) 25-42 weeks]. Twenty-nine patients with suspected impaired maternal or fetal renal function were studied separately and two fetuses were studied in utero. The mean fetal beta 2MG concentration fell from 3.87 +/- 0.56 mg/l in the 25-31 weeks GA group to 2.60 +/- 0.50 mg/l in the mature newborn group. alpha 1MG concentration fell from 3.10 +/- 0.51 to 2.25 +/- 0.49 mg/dl. In contrast, the mean maternal beta 1MG concentration rose from 1.73 +/- 0.69 mg/l in the 25-31 weeks GA group to a mean of 1.83 +/- 0.48 mg/l in the mature newborn group; alpha 1MG rose from 3.96 +/- 0.58 to 4.33 +/- 1.6 mg/dl. Maternal and fetal creatinine levels were identical. Fetal microprotein levels fall during intra-uterine development as glomerular filtration rate (GFR) rises. There is no correlation between cord blood and maternal alpha 1MG or beta 2MG concentrations. In 13 children with urological anomalies only 1 had elevated microprotein levels and he later developed renal insufficiency. Determination of microprotein levels in fetal serum can be used to detect severe renal function disturbances and to estimate GFR independently of maternal renal function.

Diagnosis of brain death by transcranial Doppler sonography.

The blood flow velocities in the basal cerebral arteries can be recorded at any age by transcranial Doppler sonography. We examined nine children with either initial or developing clinical signs of brain death. Soon after successful resuscitation increased diastolic flow velocities indicated a probable decrease in cerebrovascular resistance; this was of no particular prognostic importance. As soon as there was a clinical deterioration, there was a reduction in flow velocities with retrograde flow during early diastole, probably due to an increase in cerebrovascular resistance; this indicated a doubtful prognosis. In eight of the nine children with clinical signs of brain death a typical reverberating flow pattern was found, which was characterised by a counterbalancing short forward flow in systole and a short retrograde flow in early diastole. This indicated arrest of cerebral blood flow. One newborn showed normal systolic and end diastolic flow velocities in the basal cerebral arteries for two days despite clinical and electroencephalographic signs of brain death. Shunting of blood through the circle of Willis without effective cerebral perfusion may explain this phenomenon. No patient had the typical reverberating flow pattern without being clinically brain dead. Transcranial Doppler sonography is a reliable technique, which can be used at the bedside for the confirmation or the exclusion of brain death in children in addition to the clinical examination.

[Primary management of newborn infants with respiratory failure due to a congenital defect of the diaphragm]. / Die Primärversorgung respiratorisch insuffizienter Neugeborener mit kongenitalem Zwerchfelldefekt.

The authors report on 36 premature and term infants with congenital defects of the diaphragm, including 17 patients who developed a severe respiratory insufficiency either immediately after birth or during the first 30 minutes of life. The overall mortality rate was 64%; the results of treatment were marked by a particularly high mortality rate among the patients with early respiratory insufficiency: out of 17 patients, 12 died. The evaluation of the courses confirmed that adequate preoperative primary care is of major prognostic importance in patients with early respiratory insufficiency. On the basis of personal experience the authors have formulated the following recommendations for improving diagnosis and therapy at this stage of care: greater use of sonography examination methods for antepartal diagnosis of congenital defects of the diaphragm, making it possible to treat the corresponding high-risk pregnancies at an early stage in clinics equipped for neonatologic and pediatric surgical treatment; early intubation respiration with a ventilation technique which does not overstrain the patient, in newborns with abnormal respiration and suspected congenital defects of the diaphragm; the great diagnostic importance of plain radiographs of the thorax and abdomen; neonatologic and intensive medical stabilization treatment in patients with severely suppressed metabolism prior to the anesthesiologic and surgical stress of closure of the diaphragm defect.

[Beta 2 microglobulin in the serum as a parameter of glomerular kidney function in the first days of life]. / beta 2-Mikroglobulin im Serum als Parameter der glomerulären Nierenfunktion in den ersten Lebenstagen.

Though serum creatinine is a very reliable parameter for predicting glomerular filtration rate in infancy, this does not apply to the first hours and days of life. As there is no placental barrier for creatinine, serum creatinine at birth reflects maternal renal function at the moment of delivery and, during the first days of life, establishment of the steady state condition between creatinine serum level and actual infantile glomerular filtration rate. Serum creatinine levels of cord blood and maternal blood in term and preterm infants of 25-42 weeks gestational age are almost identical (maternal blood 0.82 +/- 0.34 mg-%, cord blood 0.87 +/- 0.34 mg-%, n = 77, r = 0.94), whereas there is no correlation between maternal and infantile beta 2-microglobulin concentrations (maternal blood 2.1 + 1 mg/1, cord blood 3.3 +/- 0.6 mg/l, n = 78, r = 0.05). There is no free diaplacental exchange for this low molecular weight protein. The determination of cord blood beta 2-microglobulin levels therefore predicts the newborn's renal function independently of the mother's. It is possible to differentiate between prenatal and perinatal genesis of renal damage in case of renal failure in the newborn, and to study the elimination of creatinine preloading in maternal renal insufficiency. Although we are not yet able to give an exact quantitative prediction of glomerular filtration rate by determining beta 2-microglobulin we believe it to be the best parameter of glomerular renal function in this age-group.

[Intravascular monitoring by axillary artery cannulation in infants]. / Intravasales Monitoring durch Arteria axillaris-Kanülierung im Säuglingsalter.

In patients with respiratory failure and shock serial arterial blood analyses are important to calculate adequate respiratory therapy. During circulatory insufficiency punction of a radial artery can be difficult or impossible, especially in very young patients. The case report of a 9-month old female infant with septic shock and Waterhouse-Friderichsen's syndrome illustrates these problems and is helpful to describe an alternative technique. Percutaneous axillary arterial catheterization with a disposable venous cannula proved to be a simple and quick method to obtain the possibility of arterial monitoring in very young patients with severe hypotension.

Diagnostic procedures leading to successful separation of xipho-omphalopagus twins.

Xipho-omphalopagus twins with a pericardial bridge, extended liver tissue union and considerable intestinal herniation from one abdominal cavity to the other were separated successfully at the age of three months. Special diagnostic procedures including cardiac and abdominal sonography, catheterism of the umbilical vein with portal angiography, radionucleotide liver and bile duct imaging and separate oral glucose tolerance tests provided important information for perioperative and surgical patient management. Relevant items for determination of the favourable data and method of surgery are discussed.

Cerebral infarction in term neonates: diagnosis by cerebral ultrasound.

Cranial ultrasound (US) through the newborn's open fontanelle can diagnose not only intracerebral hemorrhages but also diffuse and localized hypoxic-ischemic encephalopathies. Sonographically, it was possible to distinguish between different courses of cerebral ischemia in seven neonates: ischemic infarction, usually in the area of the middle cerebral artery: borderline infarction; transient ischemia. The patients showed lateralized seizures during the first days of life with a corresponding focus in the electroencephalogram (EEG). Computed tomography showed areas of partially reduced density corresponding to the regions of increased echogenicity in ultrasound. The course was various; prognosis was good except in one patient. Etiologically, embolism, thromboses or hypoxemia were responsible for cerebral infarction. In some cases secondary bleeding ensued. The prognostic value of cerebral lesions was dependent on the involved area, gestational age, and any concurrent hypoxic cerebral damage.

[Transfusion-induced acid load caused by erythrocyte concentrates in the newborn]. / Transfusionsbedingte Säurebelastung durch Erythrozytenkonzentrate im Neugeborenenalter.

Erythrocyte concentrates are preferred increasingly to whole-blood transfusions in treatment of neonatal anemias. By means of their application even very important restorations of hematocrit are possible without the risk of fluid overload. A possible side-effect of these erythrocyte suspensions is a transfusion mediated acidosis, which depends on the used buffer solution, temperature and storage conditions before and during use. The case report of a preterm infant with Rh-Erythroblastosis and postpartal shock demonstrates the problem of an additional acidosis caused by the ACD-adenin stabilised erythrocyte concentrate. The impression of a transfusion mediated acidaemia is confirmed by 30 comparative pH-measurements in ACD-adenin and heparin stored erythrocytes. We conclude that erythrocyte transfusions in very ill neonates with severe disturbances of metabolism and reduced organ functions should be done with heparin erythrocyte preparations.

Dysplastic features, growth retardation, malrotation of the gut, and fatal ventricular septal defect in a 4-month-old girl with ring chromosome 15.

A 20-day-old female neonate was admitted with symptoms caused by a large ventricular septal defect which was subsequently confirmed angiographically. Other clinical findings were pre- and postnatal growth retardation, microcephaly, dysmorphism of ears, fingers and feet. Cytogenetic analysis revealed a ring chromosome 15. Despite a palliative banding operation of the pulmonary artery, the infant succumbed to complications of her congenital heart disease in the 4th month of life.

[Leprechaunism (Donohue's syndrome). Clinical and pathologico-anatomical findings]. / Leprechaunismus (Donohue-Syndrom). Klinische und pathologisch-anatomische Befunde.

Leprechaunism is a very rare condition of obscure aetiology. At the age of three weeks the neonate described in this report lost all subcutaneous fat in spite of additional parenteral nutrition. He acquired purulent pneumonia, and finally died of septicaemia. The typical stigmata were a prominent nose, broad mouth with putty lips, and large, hypertrophic , backwards rotated ears, cutis laxa, atrophy of adipose tissue and gynaecomastia with hirsutism. Since the first report (1948), 32 patients have been described in detail. Compared with these reports of dysendocrinism we observed an excessive proliferation of various tissues, e.g. of the epithelia of the epidermis, bronchi, collecting tubules of kidneys, bile-ducts, and pancreatic ducts. Moreover, almost complete atrophy of lymphoid tissue was remarkable.

Plasma elimination of antithrombin III (heparin cofactor activity) is accelerated in term newborn infants.

Antithrombin III (AT III) levels are markedly increased in newborn infants following exchange transfusion with adult blood, and subsequently return to pre-exchange values. This transient rise in AT III (heparin cofactor activity), was used to estimate its plasma elimination half-life. AT III activities were measured serially, before and after double-volume exchange transfusions with heparinised blood in newborn infants requiring therapy for severe hyperbilirubinaemia. The plasma elimination half-life of AT III activity was calculated to be 3.9 +/- 1.4 h (mean +/- SEM). Compared with published data on the kinetics of AT III infusions in adults, the neonate has a considerably accelerated turnover. This finding has important implications for the design of future therapeutic trials of AT III concentrates and provides further evidence that plasma proteins, including components of the coagulation system, appear to have different kinetics in the neonatal period.

[Therapeutic recommendations for the resuscitation of children]. / Therapieempfehlungen in der Reanimation von Kindern.

The general management and steps of cardiopulmonary cerebral resuscitation are the same for infants, children and adults: Airway management; Breathing, Cardiac compressions and Drugs to restart circulation and maintain cerebral and myocardial oxygen supply. However, priorities and techniques differ somewhat because of variations in size, physiology and cause of circulatory arrest. In pharmacological support there are several new aspects: The immediate correction of acidosis is not necessary in CPR and iatrogenic alkalosis has deleterious effects. Beta-receptor stimulators and calcium should not be used furthermore in CPR after cardiac arrest. Epinephrine was and is still the drug of choice in resuscitation. Drugs for cerebral resuscitation are still in a state of development.

Congenital aortic thrombosis with complete obliteration of the aortic arch and the great vessels.

Thrombosis of the great arteries rarely occurs in the neonate. We report a case of thrombosis of the aortic arch, the brachiocephalic truncus, the left carotid and subclavian arteries, seen in a one-day-old neonate. The clinical findings were at first misdiagnosed as interrupted aortic arch syndrome, though, in retrospect echocardiography was very suggestive of the correct diagnosis. On the basis of the histological examination we assume, as the most likely cause for thrombosis, an aortitis in the prenatal period. Following thrombectomy and Gore-Tex bypass from the ascending to the descending aorta, combined with ligature of a PDA, the child developed a low output state and died on the table. The postmortem examination revealed that the latter was due to multiple infarcts of the left ventricular myocardium.

Arterial occlusion in a preterm infant. Successful non-surgical treatment with urokinase and low-dose heparin.

Neonatal arterial occlusion is a rare condition of obscure aetiology, when not associated with catheterization of the umbilical artery. Embolic occlusion of the left iliac artery was diagnosed in a preterm baby weighing 1000 g, who presented with a cold, pale and pulseless left leg on the 6th day of life. Surgical embolectomy was contraindicated in this very small baby. Fibrinolytic therapy with urokinase and administration of low-dose heparin resulted in a complete recovery of skin colour, skin temperature and blood pressure. After three weeks of urokinase therapy, no pulse differences could be detected between right and left femoral and popliteal arteries by Doppler examination, and only minimal differences were present between right and left tibial arteries.

[How to diagnose intracranial haemorrhages in infants by two-dimensional ultrasound scanning (author's transl)]. / Zur Diagnostik von Hirnblutungen beim Säugling mit der zweidimensionalen Sektor-Echo-Enzephalographie.

Applying a rotatory sector-scanner, in 437 infants between the 29th gestational week and 18th month of life, a sonographic study was performed in order to look for an intracranial haemorrhage. The recording was performed real-time, using the anterior fontanelle as an acoustic window. In 42 infants we saw signs of an intracranial haemorrhage, which was confirmed 11 times anatomically and 11 times by CAT. Advantages and disadvantages of the method are discussed.

Decreased production or increased turnover of antithrombin III in severe acquired coagulopathy?

During the course of severe coagulopathy in an infant suffering from septicaemia and shock, antithrombin III levels were determined repeatedly before and during substitution therapy with human antithrombin. By mathematical analysis of these data, using a biexponential function, the plasma elimination half-life of the antithrombin III was estimated to be 7.5-10.5 h. Compared with known plasma half-lives of radioactively labelled antithrombin III in adults the increase was five-to ten-fold. This indicates that the significantly decreased levels of antithrombin III in this case of coagulopathy were at least partly due to an accelerated consumption of antithrombin III. The estimation of the plasma elimination half-life of antithrombin III helps to differentiate decreased production from increased consumption in cases of severe coagulopathy. Thus, a more precise diagnosis of disseminated intravascular coagulation can be made whilst taking advantage of substitution therapy and avoiding the hazards of radioactive tracer proteins.