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Biomedical subjects

J Schaub

Publications and source records attributed to J Schaub.

At least 19 recordsLinked to original sources

Longitudinal zinc balances in breast-fed and formula-fed infants.

Longitudinal zinc (Zn) balance studies were performed under domestic conditions in term breast-fed (n = 10), term formula-fed (n = 5; Zn concentration in the formula: 4 mg/l) and preterm formula-fed (n = 3) infants during the first 17 weeks of life. Samples of milk, urine and feces were analyzed by atomic absorption spectrometry. The median daily Zn intake in breast-fed infants decreased from 0.592 (0.457-0.829) mg Zn/kg body weight to 0.151 (0.095-0.304) mg Zn/kg body weight in the first 17 weeks of life; comparable values for bottle-fed term infants were 0.58 (0.511-0.701) and 0.674 (0.529-0.731) mg Zn/kg body weight. The median percent retention of Zn intake was 27 (-60 to 81.4)% in breast-fed infants and 21.5 (-42 to 64)% in formula-fed infants. In view of the urinary and fecal Zn losses measured, a daily intake of 0.3-0.5 mg Zn/kg body weight is considered to be sufficient to ensure a Zn retention equivalent to breast-fed infants. This requires a Zn concentration of 2-3 mg/l of Zn depending on milk volume intake.

Bottle Feeding

[Sodium and potassium metabolism in infancy].

BACKGROUND: Recommendations for the concentration of most nutrients in infant formulas are based on their concentration in human milk. Industry succeeded in adapting sodium and potassium content in infant formulas to concentration found in human milk. Whether this adaptation affects on infants' mineral balances was studied in breast-fed and artificially-fed infants. METHODS: Sodium and potassium balances were performed in 16 term male infants from their 3rd until their 17th week of life. The balances were performed at home and comprised up to five periods in intervals of three to four weeks. Each balance period consisted of subsequent three 24 h collections of milk, stool and urine samples. Ten infants were breast-fed, six received an adapted infant formula supplemented with copper, zinc and iron. RESULTS: The breast-fed infants got a mean intake of 1 mmol Na/kg b w x day and a mean intake of 1.8 mmol K/kg b w x day. Man retention was 0.4 mmol Na and 0.7 mmol K/kg b w x day. The formula-fed infants received 1.9 mmol Na/kg b w x day and 2.1 mmol K/kg b w x day. Na- and K-retention in this group was 0.5 and 0.6 mmol/kg b w x day respectively. Although sodium intake in the formula-fed infants was nearly twice as much as in the breast-fed infants the difference in sodium retention was only small (0.4 vs 0.5 mmol/kg b w x day). The formula-fed infants got more potassium than the breast-fed infants, but potassium retention was the same in both groups. CONCLUSIONS: With the adapted infant formula of this study the artificially fed infant was as well supplied with sodium and potassium as the breast-fed infant. A further reduction of the sodium concentration seems not to be useful.

Animals

Iron availability from an infant formula supplemented with bovine lactoferrin.

Iron balance studies were performed in 16 term infants from their 3rd until their 17th week of life. The balance studies were performed at home and comprised five periods with an interval of 3 to 4 weeks, each consisting of three 24-hour collections of milk and stool samples. Seven infants were fed an adapted infant formula supplemented with bovine lactoferrin (100 mg/100 ml) and nine received the same formula without lactoferrin. The lactoferrin supplemented group received 169 micrograms iron/kg b.w. x day and retained 63 micrograms/kg b.w. x day. The mean iron intake of infants fed with the adapted formula without supplementation of lactoferrin was 118 micrograms/kg b.w. x day. The retention of iron was 43 micrograms/kg b.w. x day. Mean percentage retention of iron in the supplemented group was 36%, in the non-supplemented group 28%.

Biological Availability

Successful stimulation of autologous bone marrow recovery by GM-CSF and IL-3 after an unrelated donor BMT for juvenile CML complicated by graft failure.

We report the case of a boy with juvenile chronic myeloid leukemia (jCML), who after initial treatment with interferon alpha-2 (IFN) and hydroxyurea underwent bone marrow transplantation (BMT) from a matched unrelated donor complicated by graft failure. Subsequent stimulation of hematopoiesis by GM-CSF and IL-3 promoted autologous recovery. In contrast to the course of disease pre-BMT, the boy is now off any therapy remaining in complete remission more than 500 days after BMT.

Bone Marrow

Remission of juvenile chronic myeloid leukemia following graft failure of an unrelated marrow transplant and autologous recovery of marrow function promoted by GM-CSF and IL-3.

The unusual course of a boy with juvenile chronic myeloid leukaemia is presented. After bone marrow transplantation (BMT) from an unrelated donor followed by graft failure and subsequent stimulation by rhu GM-CFC and II-3, this patient experienced complete recovery of autologous haematopoiesis. At 17 months post-BMT the patient was off any therapy with completely normal blood counts and no signs of disease.

Bone Marrow Transplantation

Trace element excess in PKU diets?

Knowledge of trace element requirements of infants with phenylketonuria (PKU) fed a semisynthetic diet is limited. Three infants with PKU detected early were studied longitudinally in classical balance studies for 72 h, under domestic conditions, at the ages of 2, 5, 8, 12 and 16 weeks. Iron, copper and manganese concentrations in the diet and faeces were determined by atomic absorption spectroscopy. The median concentrations in the diet (4.8 mg Fe/L, 1.7 mg Cu/L, 0.43 mg Mn/L) exceed those in human milk. This is mainly due to supplementation of the amino acid preparation used. The increased intake led to a significantly higher daily retention of Cu and Mn from the PKU-diet fed, with a median of 0.17 mg Cu/kg and 6.4 micrograms Mn/kg body weight; the median retention of Fe was 0.24 mg Fe/kg. Our results confirmed the doubts about the suitability of the present trace element supplementation in formula for infants with PKU during the first four months of life.

Body Burden

[Anamnesis of brain-originated vision disorders].

A questionnaire for the subjective assessment of cerebral visual disorders was administered to 269 brain damaged patients. The patients' subjective complaints were correlated with the outcome of objective diagnostic procedures (visual perimetry, visual search, spatial contrast sensitivity, visual evoked potentials, depth perception, light- and dark-adaptation, reading, colour vision). A significant correlation was found between subjective complaints and objective diagnostic results for all fields. The prediction of visual impairments on the basis of subjective complaints and verified by objective tests was correct in 80 to 98% of the brain damaged patients tested. Interrater reliability was about 0.84. Thus, the questionnaire is suitable for the assessment of cerebral visual disorders because of its simplicity, short administration time (10-15 min) and high predictive value.

Accommodation, Ocular

[Arterial thrombosis in a premature infant].

A preterm infant of 30 weeks gestation developed thrombosis of the left arteria femoralis on his fourth day of life. Continuous intravenous heparinization with 350 to 750 U/kg bw/d was performed during the course of several weeks. Although arterial thrombosis in term and preterm infants is not a rare event, there are no generally accepted guidelines for treatment. In this article alternatives of treatment--namely surgical management, thrombolytic therapy, heparinization and supportive therapy are discussed.

Blood Coagulation Tests

Urinary zinc excretion in infancy.

In view of the conflicting data on urinary Zn excretion in infancy we investigated the possible influence of contamination, collecting methods, nutrition (human milk versus formula) and longitudinal changes during the first 16 weeks of life. Methodical investigation showed that special attention is necessary to avoid contamination due to the use of Zn-containing baby creams in the genital region. The sampling device for collection should include the smallest area of skin possible and the use of Zn-containing baby creams has to be avoided both during the collection and at least 24 hours prior to urine collection. Previous fractional urine sampling of the collecting method to be evaluated proves that erroneously high values are not obtained at the beginning of collection. Midstream urinary samples reduce the possibility of contamination. Increased urinary excretion was shown in pre-term infants under theophylline or coffeine medication. The median daily urinary Zn excretion in healthy breast-fed term infants declined significantly from 0.063 (0.027-0.111) mg per kg body weight at the age of 2 weeks to 0.018 (0.004-0.059) mg per kg body weight at the age of 16 weeks. Comparable values for formula-fed term infants were 0.029 (0.025-0.063) mg per kg body weight initially and 0.025 (0.007-0.059) mg per kg body weight at the end of the study. These values can be used as reference values for the urinary Zn excretion of healthy infants.

Aging

Selenium balances in young infants fed on breast milk and adapted cow's milk formula.

Selenium intake, renal and fecal excretion, and retention were determined in 11 breast-fed term infants and in 9 term infants fed with an adapted cow's milk formula. In most cases three-day-balances were determined five times at the age of 2, 5, 8, 12, and 16 weeks. Analytical determination of selenium was by atomic absorption spectroscopy with a selenium hybrid system after wet ashing of samples. The mean breast milk concentrations of selenium show a significant decrease in the course of lactation from 31.0 micrograms/L in the first collecting period to 17.6 micrograms/L in the last one. The two formula milks contained 11.8 and 7.7 micrograms/L. Intake in the breast milk group was higher than in the formula group, whereas absolute renal and fecal excretion were essentially the same. This leads to a higher selenium retention in the breast-fed infants than in the formula-fed infants, where most of the balances are negative.

Adult

Lissencephaly, abnormal lymph nodes, and T-cell deficiency in one patient.

We report on a child with lissencephaly type I, abnormal lymph nodes, and immunodeficiency, associated with recurrent infections, autoimmune disease, spastic tetraplegia, and psychomotor retardation. Diagnostic measures included cranial computer tomography (CT) and magnetic resonance imaging (MRI) scanning, several in vivo and in vitro immunological tests, and histology of skin, lymph nodes, and liver including electron microscopy and immunohistology. Despite medical supervision, the child died at age 4 years. A common pathogenetic mechanism of defective migration of neurons and the dysmaturation of lymph nodes is most probable. The T-cell deficiency may represent a common defect of the development of both neuronal and lymphatic tissue, as the six-layered cerebral cortex and the B-cell areas in lymph nodes develop at about the same gestational age. A common defect could also be assumed involving genetically determined cell surface proteins.

Brain

Longitudinal manganese and copper balances in young infants and preterm infants fed on breast-milk and adapted cow's milk formulas.

1. Mn and Cu intake and retention in twenty full-term infants and six preterm infants were studied on the basis of 72 h balances. The age of the infants was 2-16 weeks and the gestational age of the preterm infants (triplets) 34 and 36 weeks. Three nutrition schemes were pursued: breast-fed, formula-fed with unsupplemented adapted formula and formula-fed with trace element supplementation. 2. The mean Mn concentration of all breast-milk samples (n 2339) was 6.2 micrograms/l. The two formulas had similar Mn concentrations (77 and 99 micrograms/l) but had different Fe, Cu (121 and 619 micrograms/l), Zn and I contents. The mean Cu concentration in mother's milk was 833 micrograms/l. 3. The following mean daily Mn intakes and retentions (micrograms/kg) respectively were measured: breast-fed full-term 1.06 (SD 0.43) and 0.43 (SD 0.65), formula-fed full-term 14.2 (SD 3.1) and 2.8 (SD 4.8), formula-fed preterm 15.0 (SD 2.2) and 0.06 (SD 5.87). The results for Cu were 114.5 (SD 22.3) and 88.0 (SD 46.5) micrograms/kg in breast-fed, 19.8 (SD 4.2) and 4.6 (-11.5-9.6) in the unsupplemented formula-fed and 106.4 (SD 18.9) and 55.5 (SD 20.3) in the supplemented formula full-term infant group. No significant influence of the trace element contents of the formulas on the relative retention of Mn or Cu was found. 4. Young preterm infants, and to some degree young full-term infants, often had negative Mn balances caused by a high faecal excretion. The formulas with a Mn concentration below 100 micrograms/l gave a sufficient supply of Mn. Preterm infants fed on the unsupplemented formula had a marginal Cu supply and their first balances were negative (-3.8 (SD 1.8) micrograms/kg). 5. In accordance with the estimated safe and adequate daily dietary intakes (recommended dietary allowances), formula-fed infants receive much more Mn than breast-fed infants and their absolute retention is higher. 6. Cu from breast-milk had a significantly better biological availability than that from cow's milk formula. If retentions similar to those in breast-fed infants are intended, we conclude, therefore, that cow's milk formula should be fortified with Cu up to a level of at least 600 micrograms/l.

Animals

Fractionated total body irradiation plus high-dose VP-16 prior to allogeneic bone marrow transplantation in children with poor risk acute leukaemias.

Nineteen children (median age, 13 years; range 4 to 18 years) with acute lymphoblastic leukemia/lymphoma (ALL) (10 patients) or acute nonlymphoblastic leukemia (ANNL) (9 patients) received allogeneic bone marrow transplants (BMT). Marrow was taken from HLA-identical sibling donors (16 patients) (pts), HLA-identical unrelated donor (1 pt), or one-antigen-missmatched sibling donor (1 pt). Preparatory regimen consisted of fractionated total body irradiation and high-dose VP-16 (50-70 mg/kg body weight). At the time of BMT nine of the pts were not in complete remission (CR): seven pts were refractory to aggressive multiagent chemotherapy and two pts were in first relapse. Six pts were in second CR, one pt in third CR; three pts grafted in first CR carried additional risk factors; e.g. induction failure. Ten out of the nineteen pts are alive and free of disease between one and 53 months (median, 28 months) after BMT. The actuarial disease-free survival rate is 37% for pts with ANLL and 54% for pts with ALL. Six pts have died from BMT-related complications. Only three pts (1 pt with ALL, 2 pts with ANLL) have relapsed between day +106 and day +134 after BMT and subsequently died. The four-year actuarial relapse rates of 29% for ANLL and 14% for ALL, respectively, demonstrate that the combination of fractionated total body irradiation and high-dose VP-16 is an effective antileucemic regimen for children with advanced leukemias.

Adolescent

Glycogen storage disease: recommendations for treatment.

A workshop was held on "Aspects of treatment of patients with glycogen storage disease" within the framework of the Concerted Action "Inborn errors of metabolism" of the European Communities. Consensus was reached on the main issues of treatment of patients with deficiency of glucose-6-phosphatase, glucose-6-phosphate translocase, debranching enzyme, liver phosphorylase and phosphorylase-b-kinase. The resulting recommendations are reported.

Adolescent

Fractionated total body irradiation and high-dose VP 16-213 followed by allogeneic bone marrow transplantation in advanced leukemias.

Thirty-eight patients (median age, 21 years) with acute nonlymphoblastic leukemia (ANLL) (17 patients), acute lymphoblastic leukemia/lymphoma (ALL) (18 patients), chronic myelogenous leukemia (two patients), and refractory anemia received allogeneic bone marrow transplants from HLA-identical sibling donors or a one-antigen-mismatched brother (one patient) after a preparatory regimen consisting of fractionated total body irradiation and high-dose VP 16-213 (60 to 70 mg/kg body weight). Of the 33 patients with acute leukemia who received grafts from HLA-identical donors, three patients with ANLL received transplants in first remission and one patient with standard-risk ALL received a graft while in second remission. All other patients were in more advanced stages of their disease or exhibited other high-risk features. At the time of analysis, 20 of the 33 patients were alive, with 19 of them remaining in continued complete remission for 6 to 35 months (median, 18 months). The 3-year actuarial disease-free survival rate of 56.6% +/- 9.7% (SE) and the actuarial relapse rate of 11.9% +/- 6.8% (SE) demonstrate that the combination of fractionated total body irradiation and high-dose VP 16 is an effective mode of therapy in patients with advanced leukemias. Preliminary experience cautions against the use of VP 16 instead of cyclophosphamide in any clinical situation carrying an increased risk of graft rejection because the immunosuppressive potency of VP 16 is largely untested but may be inferior to that of cyclophosphamide.

Adolescent

Agarose gel isoelectrofocusing of UDP-galactose pyrophosphorylase and galactose-1-phosphate uridyltransferase. Developmental aspect of UDP-galactose pyrophosphorylase.

The uridine diphosphogalactose pyrophosphorylase activity has been determined in human adult and fetal tissues as well as blood of various ages by measurement of UDP-galactose production from gal-1-p and UTP. The highest activity was found from adult liver in which the specific activity was about 5% of the gal-1-p uridyltransferase activity. In general adult tissues had a somewhat higher activity than the corresponding fetal tissues except erythrocytes in which fetuses had a 5-10 times higher activity than adults. From normal blood the pyrophosphorylase activity in erythrocytes decreased with age, but in the case of galactosemia the decrease with age was not distinct. According to agarose gel isoelectrofocusing studies, at least two isozyme forms for UDP-galactose pyrophosphorylase exist with the activity bands between pH 6.0-6.15. The patterns of AGIF bands of pyrophosphorylase varied according to the age of the samples, suggesting the development of the isozyme forms of pyrophosphorylase to be age-dependent. Uridyltransferase, on the other hand, resolved into multiple bands between pH 5.1-5.6 on agarose gels and the patterns varied according to the variants but not to the age. Significance of the decrease in the pyrophosphorylase activity in erythrocytes with age as well as of the difference in AGIF bands between normal and the galactosemic were discussed with regard to the pathology of classical galactosemia.

Adolescent