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Background: Rhesus D (RhD) incompatibility is still the most important cause of hemolytic disease of the fetus and newborn (HDFN) worldwide. The aim of this study was to investigate the incidence, causes, and consequences of anti-D alloimmunizations in pregnancy in Iceland, prior to implementation of targeted routine antenatal anti-D prophylaxis (RAADP) in 2018., Study Design and Methods: This was a nation-wide cohort study of 130 pregnancies affected by RhD alloimmunization in Iceland in the period from 1996 through 2015. Data were collected from transfusion medicine databases, medical records, and the Icelandic Medical Birth Register., Results: Of 130 RhD alloimmunizations, 80 cases (61.5%) represented new RhD immunization in the current pregnancy. Sensitization was discovered in the third trimester in 41 (51.3%) and occurred in the first pregnancy in 14 cases (17.5%). The most likely causative immunization event was the index pregnancy for 45 (56.25%), a previous pregnancy/birth for 26 (32.5%), abortion for 3 (3.75%), and unknown for 6 women (7.5%). Higher anti-D titers were associated with shorter gestational length, cesarean sections, positive direct antiglobulin test (DAT), and severe HDFN. Intrauterine transfusion (IUT) was performed in five pregnancies (3.8%), and 35 of 132 (26.5%) live-born neonates received treatment for HDFN; 32 received phototherapy (24.2%), 13 exchange transfusion (9.8%), and seven simple blood transfusion (5.3%)., Conclusion: In about half of cases, RhD alloimmunization was caused by the index pregnancy and discovered in the third trimester. Thus, the newly implemented RAADP protocol should be effective in reducing the incidence of RhD immunization in Iceland in the future., (© 2019 AABB.)

Background: Red blood cell (RBC) alloimmunization during pregnancy is still a major problem. Historically, anti-D antibodies are most likely to cause severe hemolysis, but other antibodies are also important. In Iceland, postnatal RhIg prophylaxis was implemented in 1969, universal RBC antibody screening was implemented in 1978, but antenatal RhIg prophylaxis is not yet routine., Study Design and Methods: This nation-wide population study gathered data on alloimmunized pregnancies in Iceland between 1996 and 2015. Blood bank alloimmunization data were linked to Icelandic Medical Birth Registry data. RBC antibodies were classified as either clinically significant or clinically nonsignificant., Results: In total, 912 positive antibody screens from 87,437 births were identified (1.04% prevalence). The most frequent antibodies were anti-M (19.4%), anti-E (19.0%), and anti-D (12.5%). Anti-D prevalence among D-negative mothers was 1.1%. Icelandic Medical Birth Registry data were available for 881 (96.6%) pregnancies. In the clinically significant group (n = 474), anti-E (27%) and anti-D (20%) were most common, whereas anti-M was most frequent (53%) in the clinically nonsignificant group (n = 407). Mothers in the clinically significant group were older, more often multigravidae, had more abortions and stillbirths, and had shorter gestational length. Newborns in the clinically significant group were less healthy, had lower weight and Apgar scores, and required more treatment. Among specificities in the clinically significant group, anti-D antibodies were most strongly associated with severe hemolysis., Conclusion: In this study, the prevalence of alloimmunization was similar to that in previous reports. Of all clinically significant antibodies, anti-D was most strongly associated with severe hemolysis, requiring phototherapy or exchange transfusions. Our data emphasize the importance of implementing an antenatal prophylactic RhIg program in Iceland in the near future., (© 2017 AABB.)

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.

Objective: To evaluate whether N-acetylcysteine (NAC) infusion during the first week of life reduces the risk of death or bronchopulmonary dysplasia (BPD) in infants with extremely low birth weight. Study design In a Nordic multicenter, double-blind trial, infants (n=391) weighing 500 to 999 g and on ventilator or nasal continuous positive airway pressure were randomized before the age of 36 hours to receive NAC 16 to 32 mg/kg/d (n=194) or placebo (n=197) intravenously for 6 days. Primary end points were death or BPD, defined as supplementary oxygen requirement at 36 weeks' gestational age., Results: There was no difference in the combined incidence of the primary end points death or BPD, 51% vs. 49%, between the NAC group and control group. Also similar was the incidence of BPD in survivors at 36 weeks' gestational age, 40% vs. 40%, and the mean oxygen requirement at the age of 28 days, 31.2% vs. 30.7%, respectively. The severity of BPD was similar in both groups., Conclusions: A 6-day course of intravenous N-acetylcysteine at the dosage used does not prevent BPD or death in infants with extremely low birth weight.

Background: Development of protein-conjugated pneumococcal vaccines for infants has led to formulations that are immunogenic in the age group at highest risk for pneumococcal diseases. This study focuses on the search for an optimal formulation., Methods: In a randomized trial Icelandic infants (n = 160) were immunized at age 3, 4 and 6 months with one of two octavalent pneumococcal conjugate vaccines (serotypes 3, 4, 6B, 9V, 14, 18C, 19F and 23F conjugated to diphtheria toxoid (PncD) or tetanus protein (PncT) followed with a booster of either the same conjugate or 23-valent polysaccharide vaccine at 13 months. Safety data were collected after each vaccination, and IgG responses (enzyme-linked immunosorbent assay) were measured at 3, 4, 6, 7, 13 and 14 months., Results: Both conjugates were safe and caused fewer local reactions than the routine vaccines (P < 0.0001). At 7 months both groups had significant IgG response to all serotypes. The geometric mean concentration range was 0.35 to 4.09 and 0.65 to 3.38 microg/ml for PncD and PncT, respectively, with 88.2 to 100% and 92.4 to 100% of subjects reaching > or = 0.15 microg/ml. The PncD gave better primary responses to serotypes 3, 9V and 18C, whereas PncT gave better response to serotype 4. Similar responses were induced to the other serotypes. Good booster IgG responses were obtained in all vaccine groups; 97.5 to 100% of subjects reached > or = 1 microg/ml., Conclusions: Both octavalent pneumococcal conjugates were safe and immunogenic in infants. Based on the results from this and similar trials, a mixed diphtheria and tetanus pneumococcal conjugate vaccine was designed to provide the optimal immune response to each serotype.

Background: Streptococcus pneumoniae is a major cause of meningitis, bacteremia, pneumonia and otitis media. Pneumococcal polysaccharides are not immunogenic in infants, but improved immunogenicity of polysaccharide-protein conjugates has been demonstrated. Antibiotic-resistant pneumococci have increased the need for an effective vaccine., Objective: To study the safety and immunogenicity of a pneumococcal type 6B polysaccharidetetanus toxoid conjugate (Pn6B-TT) in infants and to assess the function of antibodies., Methods: Healthy infants were injected, Group A at 3, 4 and 6 months (n = 21) and Group B at 7 and 9 months (n = 19). Booster injection was given at 18 months. Antibodies were measured by enzyme-linked immunosorbent assay and radioimmunoassay, and functional activity was measured by opsonization of radiolabeled pneumococci. Nasopharyngeal cultures were obtained., Results: No significant adverse reactions were observed. Pn6B-IgG (enzyme-linked immunosorbent assay) increased to a geometric mean of 0.62 microgram/ml (P = 0.367, compared with prevaccination titers) in Group A at 7 months and 1.22 micrograms/ml (P < 0.001) in Group B at 10 months. Total Pn6B antibodies (radioimmunoassay) were 44 ng of antibody N/ml (P < 0.053) in Group A and 211 ng of antibody N/ml (P < 0.001) in Group B. A smaller increase in IgM and IgA anti-Pn6B was observed. Reinjection at 18 months elicited booster responses in total and IgG anti-Pn6B; 62% of those in Group A and 79% of those in Group B had > 300 ng of antibody N/ml. Opsonic activity, after initial and booster vaccinations, correlated with Pn6B-antibody titers. Three infants with nasopharyngeal cultures repeatedly positive for serogroup 6 had poor serum IgG responses., Conclusion: Our results demonstrate that Pn6B-TT is safe, elicits functional antibodies and memory responses in infants.

The results of 44 operations on 42 patients (nine men and 33 women) for primary hyperparathyroidism in Landakotsspitali, Reykjavik during the period 1973-1994 were studied. Only one patient needed reoperation because of persistent hypercalcemia. The operative success rate is 97.7%. One patient had two recurrent single adenomas with an interval of three years during which the patient was normocalcemic. Two patients were diagnosed having multiple endocrine neoplasia type I (MEN) before the operation. One of those had a brother with parathyroid carcinoma. Hyperparathyroidism was histologically verified in all 42 patients. Thirtyseven (88%) had adenoma, and four (9.5%) had chief cell hyperplasia. Double adenomas were diagnosed in four patients (9.5%). In two patients functioning oxyphil cell adenomas were encountered. One case (2.4%) could not be histologically sub typed. This patient had a brother who died of parathyroid carcinoma. One patient with type I MEN syndrome had adenoma, the other had chief cell hyperplasia. No parathyroid carcinoma was diagnosed. Thirtyone patients needed medical treatment for transient postoperative hypocalcemia. Permanent hypocalcemia was found in three patients. Vocal cord paralysis was recorded in one case. No other complication was found. Two patients had elevated parathyroid hormone (PTH), both nine years after the operation. One of those is normocalcemic and without symptoms, the other hypocalcemic for unexplainable reasons.

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 retrospective study was performed of all patients diagnosed as having pancreatic cancer in Iceland during the period 1974-85 (12 years). The incidence of the disease during the period according to this study was 10.7 per 100,000 males and 9.8 per 100,000 females, with age-adjusted world standard incidences of 9.0 per 100,000 males and 6.7 per 100,000 females. A total of 301 patients were identified; adequate information could be obtained for 281 patients, and 225 (74.8%) had the diagnosis histologically confirmed. Two hundred and five patients with adenocarcinoma were accepted for detailed analysis. Of the patients with adenocarcinoma 139 (67.8%) were diagnosed at laparotomy, and 33 of them had the tumour resected, with an operative mortality of 12.1%. The cancer was located in the head of the pancreas in 102 patients (49.8%), and in 159 (77.6%) metastases were found at the time of diagnosis. The median survival time for the patients with adenocarcinoma was 95.4 days (SD +/- 11.1 days), although there were two patients in this group who were alive 5 years after diagnosis. The median survival for the total group of 281 patients was 98.3 days (SD +/- 11.0 days), although 6 of these patients lived for more than 5 years. The percentage of histologically confirmed tumours in Iceland is high compared with many previously reported studies.

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.