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Introduction: Hemolytic disease of the fetus and newborn (HDFN) is caused by the destruction of fetal red blood cells due to red cell antibodies produced by the mother. HDFN can cause fetal hydrops during pregnancy or neonatal jaundice after birth. Direct Antiglobulin Test (DAT) detects antibodies bound to red cells and is a valuable test aiding in the diagnosis of HDFN. In Iceland DAT is routinely performed on cord blood or newborn blood samples if the mother is Rhesus D negative or has non-A/B red cell alloantibodies. The aim of this study was to investigate the causes and consequences of positive DAT in newborns in Iceland over a period of eight years., Material and Methods: The study population was infants diagnosed with a positive DAT in the Blood Bank in Iceland in the years 2005-2012. Relevant data on the blood group and antibody status of mother and child, blood transfusion and DAT results were retrieved from the Blood Bank information system ProSang. Birth records provided information on birth weight, gestational age and phototherapy. Health records from the Children's Hospital provided information on the management and fate of the newborn., Results: Over the study period 383 newborns had a positive DAT result at the Blood Bank. In 73.6% of cases the underlying cause was ABO blood group mismatch between mother and infant, in 20.4% of cases the mother had non-A/B red cell alloantibodies, in 3.9% both of above factors were present, while in 2.1% the cause was unclear. A total of 179 (47.6%) children had neonatal jaundice that required treatment, of which 167 (93.3%) only needed phototherapy. Eight infants required exchange transfusion, five of these had Rhesus antibodies and three ABO blood group mismatch., Conclusion: ABO blood group mismatch between mother and child was the most common cause for a positive DAT in neonates in Iceland in the years 2005-2012. Almost half of the neonates required treatment but usually phototherapy was sufficient. Rarely, blood transfusion or exchange transfusion was necessary in severe cases of ABO blood group mismatch or non-A/B red cell alloantibodies., Key Words: Coombs test, Direct Antiglobulin Test (DAT), Hemolytic disease of the fetus and newborn (HDFN), ABO blood group mismatch, red cell alloantibodies, neonatal jaundice, exchange transfusion. Correspondence: Anna Margret Halldorsdottir, annamha@landspitali.is.

Ichthyosis prematurity syndrome (IPS) is a rare inherited skin disorder. Children are born prematurely with thick skin and have been found to develop neonatal asphyxia due to occlusions in the bronchial tree from debris in the amniotic fluid. At 31 weeks of gestation, separation of amniotic and chorionic membranes was identified as well as polyhydramnion. The child was born 2 weeks later, with thickened skin with a granular appearance and required immediate ventilation and intensive care. At 2 years of age, the patient has developed an atopic skin condition with severe itching, recurrent skin infections, food intolerance and periods of wheezing. Prenatal observation of separation of foetal membranes or dense amniotic fluid may be signs of IPS and severe complication immediately after birth.

Aim: Hyponatremia can potentially have serious effects in the premature infant, Therefore, it is important to recognize its causes and prevent it if possible. The aim of this study was to evaluate the causes of hyponatremia in very low birth weight (VLBW) infants cared for at the Neonatal Intensive Care Unit (NICU) of Children's Hospital Iceland., Subjects and Methods: Retrospective descriptive study of 20 VLBW infants at the NICU of Children's Hospital Iceland, born after <30 weeks gestation with birth weight of < or =1250 g. Information was obtained on fluid administration, weight loss, sodium administration and serum sodium concentrations during their first ten days of life., Results: The median gestational age was 27 weeks (24-29 weeks) and the median birth weight was 905 g (620-1250 g). A negative correlation was found between birth weight and the amount of fluids given (R2=-0.42; p=0.002). The median weight loss was 10.6% (3.1-29.5%). A positive correlation was found between weight loss and the amount of fluids the infants received (R2=0.76; p<0.001). The amount of sodium given was on the average 5.7+3.1 mmól/kg/24 hours. The median serum sodium concentration was 137 mmól/L (127-150 mmól/L). A negative correlation was found between the amount of sodium given and serum sodium concentrations (R2=-0.42; p<0.001). There was no correlation between the amount of fluids given and serum sodium concentrations (R2=0.006; p=0.7). A negative correlation was found between birth weight and serum sodium concentrations (R2=-0.24; p=0.027)., Conclusion: High sodium requirements in VLBW infants at our hospital suggests that their hyponatremia is mainly due to the immaturity of their kidneys, which is known to result in excessive loss of sodium in the urine.

Objective: To evaluate the efficacy of high frequency ventilation (HFV) in infants failing conventional ventilator therapy at our institution., Study Group and Methods: Medical records of all infants managed on HFV after having failed conventional ventilator management from 1994-2004 were reviewed. Ventilatory settings, blood gases and pH just prior to starting HFV, and two and four hours after starting HFV were recorded., Results: Sixty one infants met the study criteria. At two hours of HFV there was a significant improvement in oxygenation (Alveolar to arterial oxygen tension difference), ventilation and acid-base balance. These values were not significantly different between two and four hours of HFV. There was no significant difference in oxygenation between survivors (n=41) and non-survivors (n=20) prior to HFV, but after two hours of HFV the survivors had significant improvement in oxygenation. Thirty one of the survivors had improved oxygenation at two and four hours of HFV, but only eight of the nonsurvivors (p=0.03)., Conclusions: HFV results in significant improvements in oxygenation, ventilation and acid-base balance in most infants failing conventional ventilatory management. The immediate response to HFV may be a predictor of survival in infants with severe hypoxic respiratory failure.

In 1987 a 73 year old man was diagnosed with a malignant sarcoma of the anus. It was originally regarded as a leiomyosarcoma. Fourteen years later the original diagnoses of the specimen was reviewed and the diagnosis was changed to GIST (gastrointestinal stromal tumour). This diagnosis was confirmed with appropriate immunohistochemical staining on the tumour tissue. This is the first case of GIST in the anus diagnosed in Iceland.

The rate of evaporation from the skin (g/m2/h) was measured in 12 full-term and 16 preterm infants (gestational age 25-34 wk) both during incubator care and when nursed under a radiant heater. The method for evaporation rate measurement is noninvasive and based on determination of the water vapor pressure gradient close to the skin surface. Measurements were first made with the infant nursed in an incubator with a controlled environment with respect to humidity, temperature, and air velocity. The measurements in the term infants were performed at an ambient relative humidity (RH) of 50%, and in the preterm infants first at 50% and subsequently at 30-40%. Evaporation rate was then measured with the infant nursed under a radiant heater. In term infants, mean evaporation rate was 3.3 g/m2/h during incubator care (RH 50%) and 4.4 g/m2/h during care under the radiant heater. In preterm infants, the corresponding values were 15.5 g/m2/h in the incubator at RH 50%, 16.7 g/m2/h at RH 30-40%, and 17.9 g/m2/h under the radiant heater. It is concluded that the evaporative water loss from the skin depends on the ambient water vapor pressure, irrespective of whether the infant is nursed in an incubator or under a radiant heater. The higher rate of evaporation during care under a radiant heater is due to the lower ambient water vapor pressure and not to any direct effect of the nonionizing radiation on the skin.

Six patients with the ectrodactyly-ectodermal dysplasia-clefting (EEC) syndrome, namely five members of the same family and one sporadic case, are presented. One of the main features of the EEC syndrome, ectrodactyly, was missing in five of the patients. The diagnosis did not become clear until the youngest son of the family was born. All of our six patients had a low birth weight and some were born preterm, and four had poly- and/or syndactyly without ectrodactyly. A low birth weight and polysyndactyly have been reported previously in patients with the EEC syndrome and might be features of the syndrome. The present patients illustrate the great phenotypic variability in the EEC syndrome and the need for a careful search for microsymptoms in potential gene-carriers. In two members of the affected family, EEC syndrome was diagnosed prenatally after 16 weeks of gestation by detection of the cleft lip and palate on ultrasound examination. The mother chose to continue the pregnancies. However, prenatal diagnosis of cleft lip and palate might be of value in genetic counselling for other inherited syndromes leading to severe disability.

A study was performed to investigate whether measurements of the evaporation rate from the skin of newborn infants by the gradient method are affected by the presence of non-ionizing radiation from phototherapy equipment or a radiant heater. The evaporation rate was measured experimentally with the measuring sensors either exposed to or protected from non-ionizing radiation. Either blue light (phototherapy) or infrared light (radiant heater) was used; in the former case the evaporation rate was measured from a beaker of water covered with a semipermeable membrane, and in the latter case from the hand of an adult subject, aluminium foil or with the measuring probe in the air. No adverse effect on the determinations of the evaporation rate was found in the presence of blue light. Infrared radiation caused an error of 0.8 g/m2h when the radiant heater was set at its highest effect level or when the ambient humidity was high. At low and moderate levels the observed evaporation rate was not affected. It is concluded that when clinical measurements are made from the skin of newborn infants nursed under a radiant heater, the evaporation rate can appropriately be determined by the gradient method.

Serums from patients with dermatitis herpetiformis, herpes gestationis and pemphigus vulgaris were examined and compared with serums from normal individuals. Consistent deviations from normal were found, in that the γA globulin fraction was increased. The γM globulins appeared normal in dermatitis herpetiformis and herpes gestationis but decreased in pemphigus vulgaris. The γA and γM globulin changes in dermatitis herpetiformis and in herpes gestationis were similar.