[Diagnosis of familial Holt-Oram syndrome]. / Diagnose eines familiären Holt-Oram Syndroms.

Presented is one rare case in a family affected by a Holt- Oram-Syndrome. This syndrome is associated with an upper limb malformation and a congenital heart disease. In our case we found radiusaplasia on both sides, thenaraplasia on the left hand, a hypoplastic thumb on the right hand. The heart was malformed as a Fallot tetralogy, the left kidney was absent. Four additional affected members of the family are described. By routine ultrasound examination we could not find this malformation syndrome. In families with affected history ultrasound screening examination should be done on a center for prenatal diagnosis.

Study of pertussis vaccines in infants: comparison of response to acellular pertussis DTP vaccines containing 25 micrograms of FHA and either 25 or 8 micrograms of PT with response to whole-cell pertussis DTP vaccine.

Clinical and serological responses of infants to primary vaccination with diphtheria-tetanus-acellular pertussis (DTPa) vaccines containing 25 micrograms of filamentous haemagglutinin (FHA) and either 25 or 8 micrograms of pertussis toxoid (PT) were compared in a double-blind randomized study with responses of infants receiving whole-cell pertussis DTP vaccine (DTPw). In total, 308 healthy infants were enrolled to receive three vaccine doses at 3, 4 and 5 months of age. DTPa recipients had significantly lower incidences of local reactions and fever than the DTPw recipients. No differences in reactogenicity were observed between DTPa groups receiving 8 and 25 micrograms doses of PT. After vaccination, an immune response to PT was seen in 96% of 25 micrograms PT DTPa recipients, 94% of DTPw recipients and 86% of 8 micrograms PT DTPa recipients. The geometric mean anti-PT neutralizing antibody titre was significantly higher for 25 micrograms PT dose recipients (34.9 Chinese hamster ovary (CHO)) as compared with 8 micrograms PT dose recipients (21.0 CHO). The results support the use of the higher dose of PT in acellular pertussis vaccine preparations.

Vitamin K in breast milk: no influence of maternal dietary intake.

There is limited information available on the vitamin K intake of lactating mothers, concentration of vitamin K1 in breast milk, and the effect of long-term vitamin K1 supplementation of lactating mothers on the vitamin K1 concentration in breast milk. In a randomized study, we followed 20 mothers who received a daily oral vitamin K1 supplement (average 88 micrograms, supplemented group) and 16 mothers receiving no supplement (control group) from 4 throughout 91 days postpartum. Maternal vitamin K intakes (weighed dietary intake) at 4-6, 25-29 and 87-91 days postpartum ranged between 73 and 1735 micrograms/day. Differences between the groups were statistically not significant. Average intake exceeded the recommended dietary intake for lactating women of 55 micrograms/day by 670%. In the supplemented group, mean breast-milk vitamin K1 concentrations (HPLC) at 5, 26 and 88 days postpartum were 1.73 (SD 0.74), 1.36 (SD 0.81) and 1.67 (SD 2.01) ng/ml, respectively. Corresponding values in the control group were 1.44 (SD 0.57), 1.68 (SD 0.70) and 1.78 (SD 1.05) ng/ml. The latter were not statistically different from values in the supplemented group. Mean daily vitamin K1 intakes of infants breast-fed by supplemented mothers were 0.69 (SD 0.42), 0.93 (SD 0.51) and 1.25 (1.53) micrograms, respectively on days 5, 26 and 88. Corresponding values in the control group were 0.69 (SD 0.30), 1.07 (SD 0.58) and 1.31 (SD 0.95) micrograms and were statistically not different from values in the supplemented group. Average vitamin K1 intakes corresponded to 7-13% of the recommended dietary intake of 10 micrograms/d for infants.(ABSTRACT TRUNCATED AT 250 WORDS)

Iron nutrition and growth of breast- and formula-fed infants during the first 9 months of life.

Feeding of iron (Fe)-fortified (12-15 mg/L) infant formulas is an effective and convenient means to protect infants from Fe deficiency. To study lower levels of Fe fortification of infant formulas (3 or 6 mg/L) compared with those currently in use, we compared Fe intake and Fe nutritional status of three groups of healthy, term infants between 90 and 274 days of age. One group received an Fe-fortified whey-predominant formula (3 mg/L) and the second group received the same formula with a higher Fe level (6 mg/L). A comparison group was breast-fed at least until 274 days of age. All infants received infant foods and cereals according to European Community recommendations. Mean Fe intake of infants fed formula fortified with 3 mg/L was significantly lower at 183 and 274 days of age (p < 0.05) than that of infants fed formula fortified with 6 mg/L. None of the infants fed the formula fortified with 3 mg/L met the recommended daily allowance value (10 mg) for infants between 6 and 12 months of age. Hemoglobin, hematocrit, mean corpuscular volume, free erythrocyte protoporphyrin, and serum ferritin levels were similar in the formula-fed groups; none of the infants had depleted Fe stores (ferritin < 10 micrograms/L) at 183 and 274 days of age. Thirteen percent of breast-fed infants had depleted Fe stores at 183 days of age, but only 3% were depleted at 273 days of age, when Fe-fortified beikost was already part of the diet. No influence of Fe nutritional status was found on zinc and copper nutritional status or on growth.(ABSTRACT TRUNCATED AT 250 WORDS)

[Acellular pertussis vaccine]. / Azellulärer Pertussisimpfstoff.

Acellular pertussis vaccines contain antigen components of B. pertussis, usually lymphocytosis promoting factor (LPF) which is also called pertussis-toxin (PT) or pertussigen, filamentous hemagglutinin (FH) and, eventually, agglutinogens 2 and 3. Acellular vaccines are being used in Japan since 1981. Several Phase II studies have been performed and showed a better tolerability than whole cell vaccines and antibody responses of at least equal magnitude. In some of these studies good protection rates were observed. The latter were not satisfactory in a Phase III study in Sweden. This study, however, might cause some criticism in as much as only 2 vaccine doses were given and a control group with whole cell vaccine was missing. Moreover, the significance of bacterial infections in vaccinated children is doubtful, similar observations have not been made in other trials nor in Japan since introduction of the vaccine in 1981. Preliminary results of our own study with a newly developed acellular vaccine which has been permitted by the Austrian Drug Commission have shown good tolerability.

[Does breast feeding protect from atopic diseases?]. / Schützt Stillen vor atopischen Erkrankungen?

It is well established that food antigens can pass from mothers to infants via the breast milk. Bovine-beta-lactoglobulin has been detected in several breast milk samples from mothers with regular intake of cow's milk. Healthy breastfed infants can produce IgG antibodies against cow's milk protein and in infants at risk for atopic disease specific IgE antibodies were found before cow's milk based infant formula was introduced into the diet. However, several clinical studies in infants at risk for atopic disease indicate that exclusive breastfeeding decreases the incidence of atopic disease. The protective effect of breastfeeding is only relative and it is uncertain, how long protection lasts. Sensitization to food antigens may occur already in utero, because infants whose mothers avoid common allergenic foods during the whole pregnancy and then during the lactation period have a lower incidence of atopic eczema than infants whose mothers are on an unrestricted diet. Avoidance of common allergenic foods only during the last trimester of pregnancy had no effect, because the fetus is capable of forming IgE immune response.

[Cerebrospinal fluid cytologic findings in children with borrelia infections (neuroborreliosis)]. / Lipuorzytologische Befunde bei Kindern mit Borrelia-Infektionen (Neuroborreliose).

The infection of the central nervous system by Borrelia burgdorferi induces usually a mononuclear (lympho-mono-plasmocellular) cytogram of the cerebrospinal fluid in humans. The initial granulocytosis of the liquor cerebrospinalis typical for bacterial infections is transistory only. Such cytologic findings may be misleading to a diagnosis as virus infection. In case of clinical signs (Bannwarth syndrome), typical anamnesis or chronic lymphocytic meningitis the estimation of specific IgG/IgM borrelia antibodies is indicated and the antibiotic therapy should be performed.

Iron intake and iron nutritional status of infants fed iron-fortified beikost with meat.

We compared iron intake and iron nutritional status of two groups of healthy term infants who received meat-containing baby foods fortified with ferrous sulphate (2 mg Fe/100 g). One group received an Fe-fortified formula (1.6 mg Fe/100 kcal) and the other a nonfortified formula. Fe intake of the group fed the nonfortified formula was significantly lower (p less than 0.0001). These infants received Fe mainly from fortification Fe with beikost (75-86%) and less than 10% met the recommended intake of 1 mg.kg-1.d-1; whereas 80-85% of the infants fed the Fe-fortified formula did. Hb, Hct, FEP, and ferritin were similar in both groups with the exception of lower ferritin values at age 365 d (p less than 0.05) in the group fed the nonfortified formula. No infant had hemoglobin less than 100 g/L. We conclude that regular consumption of commercially prepared Fe-fortified beikost with meat prevents most healthy term infants from Fe deficiency even if Fe intake is substantially below the recommended intake.

[Nutritional iron status of infants fed according to current recommendations]. / Der Eisenversorgungsstatus von Säuglingen, die entsprechend den gültigen Empfehlungen ernährt werden.

We examined the iron nutritional status of healthy term infants in a longitudinal study from 15 through 365 days of age. All infants were fed according to the present austrian recommendations. Serum hemoglobin (Hb) decreased from 15 through 122 days of age and remained constant thereafter. At 365 days of age, only 4.7% of the infants had hemoglobin levels below 11 g/dl, which is considered the borderline value for anemia. Mean corpuscular volume (MCV) of erythrocytes was changing during infancy. Free erythrocyte protoporphyrin (FEP) was constant from 122 days through 365 days of age. The upper normal value of 3 micrograms/gHb for infants older than 122 days of age corresponded to that for children older than one year and adults. Serum ferritin (SF) decreased from 15 through 183 days of age and remained constant thereafter. At 365 days of age, only 9.3% of the infants had SF below 10 micrograms/l, which is considered the borderline concentration for depletion of iron stores. We found no differences of iron nutritional status between infants who were breastfed longer than 122 days and infants who were breastfed shorter than 122 days or were fed formula. Our findings indicate that the prevalence of iron deficiency anemia and depletion of the iron stores is lower than in previous studies. Changes in infant nutrition during the last years resulted in higher iron intake and lower prevalence of iron deficiency.

A controlled trial for evaluating two live attenuated mumps-measles vaccines (Urabe Am 9-Schwarz and Jeryl Lynn-Moraten) in young children.

A prospective, randomised clinical trial was conducted to evaluate the efficacy of two live attenuated mumps-measles vaccines, the Urabe Am 9-Schwarz and the Jeryl Lynn-Moraten vaccine, in 400 young children aged 9 months-4.5 years (median 13.4 months). Antibody responses to both vaccine components were measured by the enzyme-linked immunosorbent assay (ELISA); 96.9% of the seronegative children who received the Urabe Am 9-Schwarz vaccine showed satisfactory mumps antibody responses compared to 90% of the Jeryl Lynn-Moraten vaccine recipients (P less than .01). Similar proportions of both groups, 98.5% and 96.8%, respectively, developed measles virus specific antibodies. Both vaccines were equally well tolerated and clinically acceptable.