Invited review: Iodine level in dairy products-A feed-to-fork overview.

The theme of iodine in the dairy sector is of particular interest due to the involvement and the interconnection of several stakeholders along the dairy food chain. Iodine plays a fundamental role in animal nutrition and physiology, and in cattle it is an essential micronutrient during lactation and for fetal development and the calf's growth. Its correct use in food supplementation is crucial to guarantee the animal's recommended daily requirement to avoid excess intake and long-term toxicity. Milk iodine is fundamental for public health, being one of the major sources of iodine in Mediterranean and Western diets. Public authorities and the scientific community have made great efforts to address how and to what extent different drivers may affect milk iodine concentration. The scientific literature concurs that the amount of iodine administered through animal feed and mineral supplements is the most important factor affecting its concentration in milk of most common dairy species. Additionally, farming practices related to milking (e.g., use of iodized teat sanitizers), herd management (e.g., pasture vs. confinement), and other environmental factors (e.g., seasonality) have been identified as sources of variation of milk iodine concentration. Overall, the aim of this review is to provide a multilevel overview on the mechanisms that contribute to the iodine concentration of milk and dairy products.

Feeding cows with hay, silage, or fresh herbage on pasture or indoors affects sensory properties and chemical composition of milk and cheese.

In European countries, silage-free feeding is an ancient tradition and has a particularly positive reputation among consumers. In the present study, we compared grass-based forages from the same plot conserved as hay or silage or fed fresh either on pasture or indoors, and we evaluated the differences in sensory properties of milk and uncooked pressed cheese. All herbage from the first cut of a grassland dominated by perennial ryegrass was harvested on the same day and preserved either as hay or silage. The first regrowth of the same plot was used for strip grazing or green feeding indoors. Balanced by breed, 24 Montbéliarde and 24 Holstein cows were allocated to the 4 treatments. Apart from the forages, the late-lactation cows received 3 kg/d of dry matter from concentrate. After 2 wk of dietary adaptation, the bulk milk of 3 subgroups, each with 4 cows, was collected. Part of the milk was pasteurized, and part was left raw and partly transformed to small-sized Cantal-type cheese ripened for 9 wk. Milk and cheese underwent descriptive sensory analysis by a trained sensory panel, as well as analyses of physicochemical traits. Volatile organic compounds of the cheeses were also analyzed. Raw and pasteurized milk from hay-fed cows had less intense odors of cooked milk, cream, and barnyard than milk from grazing cows, whereby the effect of pasteurization did not differ between herbage utilization methods. Cheeses obtained from cows fed fresh herbage (grazing and indoors) were clearly yellower than cheeses from silage- and hay-fed cows, which coincided with the color intensity perceived by the panelists. Moreover, cheeses from cows fed fresh herbage had more intense barnyard and dry fruit flavors, were perceived as creamier and having less lactic odor, and exhibited more fat exudation than those from cows fed conserved herbage. Only a few differences were observed in milk and cheeses from hay-fed compared with silage-fed cows, and those differences were far less pronounced than those of milk and cheeses from cows fed fresh herbage. In conclusion, the present study did not substantiate assumptions of clear sensory differences of milk and uncooked pressed cheese from hay-fed compared with silage-fed cows. For the first time, this study reports that the global flavor intensity of cheeses from indoor green-fed cows is similar to that of cheeses derived from cows fed conserved forages, whereas cheeses from grazing cows have the greatest global flavor intensity.

Consequences of walking or transport by truck on milk yield and quality, as well as blood metabolites, in Holstein, Montbéliarde, and Valdostana dairy cows.

In the mountains, the traditional practice of transhumance is common in dairy production systems to make use of the high-altitude summer pastures. Although the effects of highland grazing have been intensively studied with respect to cow performance and milk and cheese quality, the actual moving of the animals to the highlands and the consequences of this stressor for performance and milk quality in the days immediately following transhumance has not been investigated in detail. The aim of the present study was to compare the effects of a 6-km walk (lasting 1.5 h) to those of a truck transport of 10.5 km (lasting 1 h), simulating cow movement in transhumance systems, as well as a control treatment in which cows were not moved. The experiment included 12 late-lactating Valdostana Red Pied, 12 Montbéliarde, and 12 Holstein cows (i.e., breeds contrasting in genetic merit for milk production). Each cow was subjected to each treatment in a 3-wk Latin square design. Milk yield was measured, and milk and blood samples were taken around the transhumance simulation events. Cows of the 3 breeds responded similarly to both movement treatments. Walking decreased milk yield by 1 kg/milking, but truck transport did not affect milk yield. Both treatments led to an increase in plasma nonesterified fatty acids and milk somatic cell count compared with controls, and truck transport increased milk fat content. Milk coagulation properties were better for Valdostana Red Pied and Montbéliarde cows than for Holstein cows but were not affected by walking or truck transport. Further studies aiming to compare the 3 breeds should include a wider range of response variables over a longer term, including reproduction performance and repeated transhumance.

Technical note: Development and validation of an HPLC method for the quantification of tocopherols in different types of commercial cow milk.

In the present study, a methanol-fluorescence-based HPLC method was validated for its use to quantify α-tocopherol and γ-tocopherol in raw milk, whole UHT milk, partially skimmed UHT milk, whole pasteurized milk, and partially skimmed pasteurized milk. Repeatability and reproducibility, calculated as relative standard deviation of 10 measurements within the same day and 30 measurements across 3 d, respectively, were always below 5% for both tocopherols concentrations and retention times. Recovery was assessed through 3 spiking levels and it ranged from 89 to 107%. The method was able to detect the expected declines in tocopherols in milk exposed to UHT or skimming treatments. Vitamin E, calculated as the sum of α-tocopherol and γ-tocopherol, was similar in whole pasteurized and raw milk, averaging 1.57 and 1.56 mg/L, respectively, followed by whole UHT (1.33 mg/L), partially skimmed pasteurized (0.77 mg/L), and partially skimmed UHT milk (0.61 mg/L).

Minimum 10-year follow-up results of ALPINA cementless hydroxyapatite-coated anatomic unicompartmental knee arthroplasty.

One hundred and one unicompartmental knee arthroplasties (UKA) were done between 1996 and 2000 with ALPINA(®) UNI, a cementless hydroxyapatite-coated anatomic prosthesis. Sixty-five knees were available for the long-term follow-up at a mean of 11 years. The mean IKS improved from 119.3 ± 16.8 points preoperatively to 171.4 ± 25.3 at the latest follow-up (p < 0.0001). Eighty-nine percentage of the knees were rated good and excellent. The mean knee flexion has significantly improved from 120°5 preoperatively to 127°3 at the latest follow-up (p < 0.01). Eleven revision procedures were done: 1 for early knee degeneration on rheumatoid arthritis, 1 for degeneration of osteoarthritis in the opposite compartment of the knee, 1 for unexplained pain and 1 for late ACL rupture, all these 4 cases were replaced by total knee arthroplasties; 3 revisions by another UKA were done due to polyethylene insert fracture; and 4 partial revision were done for bearing exchange due to severe polyethylene wear. When revision for any reason was defined as the end point, the 13-year Kaplan-Meier survival rate was 88 % (95 % CI 81-95 %) and when revision due to implant mechanical failure (excluding degeneration of osteoarthritis in the opposite compartment of the knee and bearing exchange only) was defined as the end point, the 13-year survival rate was 94 % (95 % CI 89.1-99.1 %).

Effects of sensor ear tags with twin pin fixing system on health and well-being of cattle.

INTRODUCTION: A sensor ear tag (SET) containing Global Positioning System (GPS), accelerometer, Radio-Frequency Identification (RFID), and Bluetooth technologies was tested for wearing comfort and compliance with animal welfare requirements in cattle in a free stall barn and on summer pasture in Switzerland. The SET was equipped with a long-lasting battery via solar panel and used a «twin pin¼ fixing system. Right ears of 12 newborns and 26 adolescent animals were tagged with the SET. While left ears were tagged simultaneously with official ear tags in newborns, the adolescent animals already carried the official ear tags. The newborns stayed in a free stall barn during the entire experiment, while adolescent animals were housed in a free stall barn and on pasture during summer. All animals developed crusts beginning on day 7 after tagging with the SET. Pain reactions were observed occasionally in the first two weeks. Ear growth in newborns during 11 months of observation did not differ between ears with SET and official ear tags. Cortisol concentration in saliva of newborns decreased in the first week after tagging which is physiological for this age group. In older animals cortisol concentrations in saliva were not affected. We registered 19 incidences in 11 animals with the SET, that required veterinary or staff intervention. Two animals lost the SET with ear injury. Scars due to tag migration were observed in ears of all newborns after the 9th month of observation. In conclusion, SET with a weight of 32 g that need a twin pin fixation in cows do not seem to induce systemic or local inflammations more frequently compared to official ear tags; however, the higher risk of accidental injuries and migration in ear cartilage would not meet Swiss welfare standards and the attachment to the ear needs to be improved for general use.

INTRODUCTION: Une marque auriculaire à capteur (SET) contenant les technologies Global Positioning System (GPS), accéléromètre, identification par radiofréquence (RFID) et Bluetooth a été testée en termes de confort et de conformité aux exigences de bien-être animal chez des bovins dans une étable à stabulation libre et sur des pâturages d'estivage en Suisse. Le SET était équipé d'une batterie longue durée via un panneau solaire et utilisait un système de fixation «twin pin¼. Les oreilles droites de 12 veaux et de 26 génisses ont été équipées avec le SET. Les oreilles gauches ont été marquées en même temps avec les marques officielles chez les nouveau-nés alors que les génisses portaient déjà ces marques officielles. Les nouveau-nés ont été détenu dans une étable à stabulation libre, avec accès à une aire de sortie et aux paturages voisins pendant toute la durée de l'expérience tandis que les génisses ont été logés dans une étable à stabulation libre et en pâturage pendant l'été. Tous les animaux ont développé des croûtes à partir du 7e jour après le marquage avec le SET. Des réactions douloureuses ont occasionnellement été observées au cours des deux premières semaines. La croissance des oreilles des nouveau-nés au cours des 11 mois d'observation n'a pas différé entre les oreilles marquées par le SET et les oreilles marquées de manière standard. La concentration de cortisol dans la salive des nouveau-nés a diminué au cours de la première semaine successive au marquage, ce qui est physiologique pour ce groupe d'âge. Chez les animaux plus âgés, les concentrations de cortisol dans la salive n'ont pas été affectées. Nous avons enregistré 19 incidents chez 11 animaux avec le SET, qui ont nécessité l'intervention d'un vétérinaire ou d'un membre du personnel. Deux animaux ont perdu le SET avec blessure à l'oreille. Des cicatrices dues à la migration des marques ont été observées sur les oreilles de tous les nouveau-nés après le 9e mois d'observation. En conclusion, les SET d'un poids de 32 g qui nécessitent une fixation par deux tiges chez les bovins ne semblent pas induire d'inflammations systémiques ou locales plus fréquemment que les marques auriculaires officielles. Cependant le risque plus élevé de blessures accidentelles et de migration dans le cartilage de l'oreille ne correspondrait pas aux normes suisses en matière de bien-être et la fixation à l'oreille doit être améliorée pour une utilisation généralisée.

Differential gene expressions in subcutaneous adipose tissue pointed to a delayed adipocytic differentiation in small pig fetuses compared to their heavier siblings.

Intra-uterine growth retardation in piglets is associated to neonatal losses and a greater susceptibility to fat deposition in the long term. Dietary l-arginine supplementation to gilts during early gestation has been proposed as a way to enhance fetal survival. This study aims to investigate the effects of variation in fetal growth within litters and dietary l-arginine treatment during early gestation in pregnant sows on expression levels of several genes involved in early adipose tissue development and lipid deposition in the fetuses. At day 75 of pregnancy, sows fed a standard gestation diet throughout pregnancy and sows fed 26g L-arginine daily from days 14 to 28 of gestation in supplement to the standard diet were sacrificed. Six pairs of littermates in each dietary group with the smallest or the heaviest fetal weights within each litter were collected (total: 24 fetuses). Expression levels of DLK1/PREF1 and FZD7 were significantly greater in subcutaneous backfat of the smallest fetuses. Conversely, transcriptional adipogenic regulators PPARG, SREBP1, and CEBPA, and genes involved in terminal adipocytic differentiation LPL, ME1, and FABP4 were less expressed in those piglets. Fetal weight has no effect on expression levels of genes involved in cell cycle progression and DNA content in subcutaneous adipose tissue. Maternal dietary L-arginine treatment did not affect subcutaneous adipose tissue features in 75-day old fetuses. The gene expression changes observed in the smallest fetuses are likely associated to a lower body fat content at birth, and could predispose to catch-up fat growth during the postnatal period.

Herbage utilisation method affects rumen fluid and milk fatty acid profile in Holstein and Montbéliarde cows.

Compared with maize silage- and concentrate-based diets, herbage-based diets were repeatedly shown to favourably influence the milk fatty acid (FA) profile. However, it is unclear how the herbage feeding mode (grazing vs indoor green-feeding) and conservation (fresh herbage vs hay vs silage) modify the milk FA profile. Therefore, the aim of the present experiment was to investigate the effect of different herbage utilisation methods (including herbage feeding mode and herbage conservation method) on the ruminal biohydrogenation of dietary FA and the consequences on the milk FA composition in cows of two breeds (Holstein and Montbéliarde). Concomitant effects of botanical composition and phenological stage of the herbage on milk FA profile were controlled for by harvesting barn-dried hay and silage simultaneously as first cut from the same ryegrass-dominated grassland in a semi-mountainous region. Seven weeks later, the first regrowth of the same plot was used as fresh herbage, either grazed or fed indoor (indoor green-feeding). Twenty-four Montbéliarde and 24 Holstein cows were randomly allocated to four groups of 12 cows balanced by breed, parity, and milk yield. In a free-stall barn, three groups were given ad libitum access to hay, silage, or fresh herbage, respectively. The fourth group was strip-grazing. All cows were supplemented with 3 kg DM/day of the same energy-rich concentrate. After 2 weeks of adaptation to the forage, samples of forage, concentrate, milk, blood, and rumen fluid were collected. Fatty acid composition of forages, rumen fluid, and milk was analysed by gas chromatography. Haymaking reduced total FA content of the herbage, in particular that of linoleic acid (LA) and α-linolenic acid (ALA). Still, rumen fluid lipids of hay-fed cows had the highest proportion of rumenic acid, LA, ALA, and total polyunsaturated fatty acids (PUFAs). Milk fat from hay-fed cows had the highest proportion of LA, and the apparent transfer rates from feed to milk of LA and ALA were higher in hay-fed cows than in silage-fed cows. The proportion of PUFAs was highest in milk fat from grazing and indoor green-fed Montbéliarde cows and lowest in silage-fed cows of both breeds. In conclusion, the herbage utilisation method affects the ruminal biohydrogenation of LA and ALA, whereby herbage drying particularly increases their transfer from herbage to milk.

Progestogen profiling in plasma during the estrous cycle in cattle using an LC-MS based approach.

In many mammalian species, corpus luteum derived progesterone (P4) is the main functional gestagen during the estrous cycle and pregnancy. P4 can be metabolized into various metabolites, of which some are biologically active. While some metabolites target the classical nuclear progesterone receptor (PR), neurosteroids bind the receptors of type A γ-aminobutyric acid (GABAA-r) in the brain. According to the position of reduction within the molecule, metabolites of P4 can be characterized into C20-reduced progestogens (20α-dihydroprogesterone (20α-DHP) and 20ß-dihydroprogesterone (20ß-DHP)), C3-reduced progestogens (3α-dihydroprogesterone (3α-DHP) and 3ß-dihydroprogesterone (3ß-DHP)), 5α-reduced progestogens (5α-dihydroprogesterone (5α-DHP), allopregnanolone and isopregnanolone) and 5ß-reduced progestogens (5ß-dihydroprogesterone (5ß-DHP), pregnanolone and epipregnanolone). We questioned whether the reduced progestogens are present in bovine plasma during the estrous cycle and whether their profiles differed from the profile of the common precursor P4 around the time of luteolysis. The analytes were monitored in plasma samples using liquid chromatography mass spectrometry (LC-MS). While progestogens lagged behind the drop of P4 at luteolysis, they followed the profile of P4 during the estrous cycle. The abundance of P4 was predominant followed by allopregnanolone, pregnanolone, epipregnanolone and 20ß-DHP. Further studies will need to focus particularly on the period around luteolysis.

Replacing sunflower oil by rumen-protected fish oil has only minor effects on the physico-chemical and sensory quality of Angus beef and beef patties.

In the present study, the effects of feeding 450 g/day of rumen-protected fish oil (FO) compared to sunflower oil (SO) to Angus heifers (60 g/kg total intake) were quantified. Animal performance was not affected whereas the physico-chemical meat quality, assessed in three muscles, was slightly affected by diet. The oxidative shelf life of the perirenal fat declined with FO compared to SO. Despite the formerly shown increased n-3 fatty acid proportions of meat due to FO supplementation, a trained sensory panel identified an only slightly more intense fishy flavour in grilled steaks and beef patties from the FO compared to the SO group. In FO compared to SO patties, flavour intensity was more pronounced. The perception of off-flavours was negligible and differences between muscles were larger than between diets. In conclusion, supplementing ruminants with FO containing nutritionally beneficial n-3 fatty acids results in few side-effects on meat quality, restricted to quite faint off-flavours and a shorter fat shelf life.

Grazing behaviour of dairy cows on biodiverse mountain pastures is more influenced by slope than cow breed.

The aim of this study was to determine how cows with different genetic merit behave and perform when grazing biodiverse and heterogeneous mountain pastures with different slopes. Three groups of 12 cows in late lactation, each composed of four Holstein, four Montbéliarde and four Valdostana Red Pied cows, breeds of increasing presumed robustness and decreasing milk yield (MY) potential. Cows grazed without concentrate either on a low-diversity flat pasture or on two species-rich mountainous pastures having slopes of either 7° or 22°. Milk yield, BW and grazing behaviour were monitored two times in the first and once in the second grazing cycle. Cows of different breeds had similar behaviour on all pastures. The Montbéliarde cows performed close to their production potential; Holstein and Valdostana cows produced less milk than anticipated. No breed difference in terms of BW loss was found. The Valdostana cows exhibited the least selective behaviour with respect to plant species and plant growth stage. Still, all cows searched for the most palatable vegetation regardless of pasture diversity. On the steep pasture, cows optimised the trade-off between ingesting and saving energy to obtain feed. They remained longer at the lowest zone and selected forbs, whereas cows on the flatter pasture went to the upper zone to select grasses. The present study gave no evidence for a superior short-term adaptation to harsh grazing conditions through an optimised feeding behaviour of the Valdostana breed compared to Montbéliarde and Holstein cows.

Differential partitioning of rumen-protected n-3 and n-6 fatty acids into muscles with different metabolism.

Bioavailability of polyunsaturated fatty acids (PUFA) in ruminants is enhanced by their protection from ruminal biohydrogenation. Both n-3 and n-6 PUFA fulfil important physiological functions. We investigated potentially different incorporation patterns of these functional PUFA into three beef muscles with different activity characteristics. We supplemented 33 Angus heifers with rumen-protected oils characterized either by mainly C18:2 n-6 (linoleic acid (LA) in sunflower oil) or by C20:5 (eicosapentaenoic acid (EPA)) and C22:6 (docosahexaenoic acid (DHA)), both prevalent n-3 PUFA in fish oil. Contents and proportions of n-3 and n-6 PUFA of total fatty acids were elevated in the muscles of the respective diet group but they were partitioned differently into the muscles. For EPA and DHA, but not for LA, the diet effect was more distinct in the extensor carpi radialis compared to longissimus thoracis and biceps femoris. Partitioning of PUFA in metabolism could be related to muscle function. This has to be confirmed in other muscles, adipose tissues and organs.

Long-term effects of castration, chronic intermittent social stress, provision of grass silage and their interactions on performance and meat and adipose tissue properties in growing-finishing pigs.

In order to assess chronic stress in entire and castrated male pigs and to describe effects of a provision of grass silage in those pigs, a 2 × 2 × 2 factorial experiment comprising the factors castration, chronic intermittent social stress and provision of grass silage was carried out with 147 growing-finishing pigs from 25.6 to 102.1 kg body weight. The experimental design allowed investigating interactions between the three factors, but only few were statistically significant. Stress exposure consisted of repeated short-term confrontations with unfamiliar pigs and short-term separations. Carcasses of stress-exposed pigs had thicker backfat, lower lean meat percentage and a different fatty acids composition of the adipose tissue. While entire males differed strongly from castrates in performance, carcass characteristics and adipose tissue properties, we found no evidence for an increased level of chronic stress in entire males compared to castrates. Provision of grass silage increased stomach weight and reduced dressing percentage, but did not impair performance, adipose tissue properties or meat quality.

A homozygous deletion in the c-erbA beta thyroid hormone receptor gene in a patient with generalized thyroid hormone resistance: isolation and characterization of the mutant receptor.

Different point mutations have been identified in the T3-binding domain of the c-erbA beta thyroid hormone receptor gene that are associated with variant phenotypes of generalized thyroid hormone resistance (GTHR). In most cases of GTHR, heterozygotes are affected; a single mutant allele results in the inhibition of the function of normal thyroid hormone receptors. We report here a novel genetic abnormality, a 3-basepair (bp) deletion in the T3-binding domain of the beta-receptor in a kindred, S, with GTHR. One patient, S1, was the product of a consanguineous union of two heterozygotes and was homozygous for this defect. Heterozygotes from kindred S harbored a CAC deletion at nucleotides 1295-1297, which resulted in the deduced loss of amino acid residue threonine at codon 332, and they displayed elevated free T4 levels and inappropriately normal TSH levels characteristic of other kindreds with GTHR. However, patient S1, who had two mutant alleles, had markedly elevated TSH and free T4 levels and displayed profound abnormalities in brain development and linear growth. A fibroblast c-erbA beta cDNA extending from codon 175 to stop codon 457 was cloned from patient S1, sequenced, and used to create a full-length mutant cDNA. The kindred S mutant receptor was synthesized in vitro and did not bind T3. This mutant receptor did bind with similar avidity as the wild-type human beta-receptor to thyroid hormone response elements of the human TSH beta (-12 to 43 bp) and rat GH (-188 to -160 bp) genes. Kindred S showed the effect in man of heterozygous and homozygous expression of a dominant negative form of c-erbA beta.

An arginine to histidine mutation in codon 315 of the c-erbA beta thyroid hormone receptor in a kindred with generalized resistance to thyroid hormones results in a receptor with significant 3,5,3'-triiodothyronine binding activity.

Generalized resistance to thyroid hormones results from diverse mutations in the T3-binding domain of the c-erbA beta thyroid hormone receptor, and different kindreds have variable phenotypes. However, the T3-binding affinities of these mutant receptors studied in vitro have all been severely reduced compared to wild type. We report here a new kindred, CL, with a mutation further upstream than previously reported, a guanine to adenine base substitution at nucleotide 1244 in codon 315 changing an arginine to histidine. This base substitution was the only one found in codons 90-456 of genomic sequence and was formally shown to be a mutation by screening 51 random individuals. The kindred CL receptor complementary DNA was recreated, and the mutant receptor synthesized with rabbit reticulocyte lysate had a T3-binding affinity of 2.4 +/- 0.9 x 10(10) M-1 compared to the wild-type human placental receptor affinity of 5.2 +/- 1.6 x 10(10) M-1. Affected members of this kindred appeared clinically to have a relatively mild degree of resistance with mean total thyroxine of only 192 +/- 24 nmol/L and inappropriately normal TSH levels. Kindred CL is an example of mild generalized resistance to thyroid hormones correlated with a mutation in the beta-receptor that resulted in only a modest deficiency in T3-binding activity.

Oligonucleotide fingerprinting of free-ranging and captive rhesus macaques from Cayo Santiago: paternity assignment and comparison of heterozygosity.

Multilocus DNA fingerprinting with oligonucleotide probes (GTG)5, (GATA)4, and (CA)8 was applied in order to determine paternity in one birth cohort (15 infants) of social group (S) from the free-ranging colony of rhesus macaques (Macaca mulatta) on Cayo Santiago. While sires could be identified in 11 cases, all males tested (N = 19) could be excluded from paternity for the remaining four infants. Data revealed marked discrepancies between actual paternity and paternity as inferred from the observation of copulation behavior. Thus, a dominant social rank does not appear to be strongly associated with reproductive success. Furthermore, alternative reproductive strategies were found to yield comparable net benefits in reproduction. A second group of animals (M) was translocated from Cayo Santiago to the Sabana Seca Field Station in 1984. They have continuously resided together in a large outdoor enclosure since then. Here paternity assessment was seriously impeded by a reduced number of discriminating bands, i.e. offspring bands which were unequivocally derived from the sires. This was initially held to be indicative of a smaller degree of heterozygosity in Group M, and was attributed to inbreeding due to a lack of male immigration or extra-group fertilizations. However, a comparison of the DNA fingerprint patterns obtained in Group S and Group M lends only partial support to this idea.

Outcome of 27 patients with Hurler's syndrome transplanted from either related or unrelated haematopoietic stem cell sources.

Over the last 15 years, we have performed a total of 30 haematopoietic stem cell transplants on 27 children suffering from Hurler's syndrome. These children were of median age 11 months at the time of diagnosis and 25 months at the time of transplantation. The phenotype was severe in 21 cases (78%). The donor was familial in 13 cases: nine genotypically identical, one phenotypically identical father and three HLA-mismatched donors. Unrelated donors were selected in 17 cases: four phenotypically identical and 13 with 1-4 HLA mismatches. The conditioning regimen generally consisted of busulphan 600 mg/m(2) plus cyclophosphamide (Endoxan) 260 mg/kg and cyclosporin with methotrexate for GvHD prophylaxis. Rabbit anti-thymocyte globulin (Thymoglobuline) was given for all unrelated or familial mismatched transplantations. The median nucleated cell dose infused was 6.00 x 10(8) TNC/kg. No bone marrow (apart from one) was T cell depleted. For first transplants, engraftment was observed in 23/27 patients (pts) (85%). Primary graft failure was observed in 4/27 patients (16%), two were retransplanted from an unrelated donor, one with success. Four patients have died. The primary cause of death was infection in three cases (TRM : 11%) and disease progression in one case, after primary graft failure. Of the 23 living patients, two have disease progression after graft failure and 21 (78%) have functional grafts with a favourable long-term outcome after a median follow-up of 4.7 years, having either full or mixed chimaerism. Among surviving patients with functional grafts, 13 (62%) were transplanted from unrelated donors of whom 10 (77 %) had HLA disparities. There was a remarkably low incidence of GvHD. In our experience, haematopoietic stem cell transplantation using an HLA-matched familial donor or an HLA-matched or -mismatched unrelated donor without T cell depletion or irradiation can achieve a favourable outcome in Hurler's syndrome, with improved cognitive function, but with a limited effect on the corneas and skeleton.

A review of interleukin-2 receptor antagonists in solid organ transplantation.

Daclizumab and basiliximab, engineered human IgG monoclonal antibodies to the interleukin-2 (IL-2) receptor alpha-subunit, were approved to prevent acute rejection after renal transplantation. Daclizumab was studied in adult and pediatric renal allograft recipients, liver allograft recipients, and calcineurin-sparing protocols in renal transplant recipients. Basiliximab was studied in renal allograft recipients and subgroups of recipients of living-related and cadaveric transplants, and in patients with diabetes mellitus. Both agents reduced acute rejection and were associated with few adverse effects. However, information regarding their long-term effects on infection, malignancy, chronic rejection, and patient survival must be available before a final decision is made regarding their proper administration. We propose that a likely role the drugs will play in the field of solid organ transplantation is in new protocols that allow sparing of other more toxic immunosuppressive agents.

Myotonic dystrophy and pregnancy. A report of two cases and a review of the literature.

Myotonic dystrophy is a rare disease (1/8000), that is rarely associated with pregnancy, due to the fact that parents carrying the disease often encounter hypogonadism. Myotonic dystrophy is a neuro-endocrinian 'heredo-degenerative' dystrophy, with dominant autosomic transmission. Its association with pregnancy can lead to several problems. The myotony is often aggravated which leads to obstetrical complications turning into fetal loss, premature term delivery, hydrops, in-utero death, difficulties in expulsion, haemorrhage during delivery and/or anaesthetic accidents. The following signs during the pregnancy can diagnose fetal damage: presence of a hydrops, rare active fetal movements, and low fetal cardiac rhythm. They signify serious fetal damage leading to a diagnosis of myotonic dystrophy. Personal and family antecedents as well as an important hypotony and respiratory distress discovered in the new born are equally evocative elements. In congenital cases (6-30% of the time) the prognosis of the child is pessimistic. For all of the above elements, transmission is of maternal origin. The diagnosis of the congenital form is difficult because the disease is often unknown by the mother. The appearance of molecular tools permits a diagnosis to be formed much more rapidly in a new-born suspected to carry the illness of neonatal Steinert. Two observations illustrate this pathology. The occurrence of congenital myotonic dystrophy in a new-born allows us to diagnose the disease within the mother.

Fetal macrosomia: risk factors and outcome. A study of the outcome concerning 100 cases >4500 g.

OBJECTIVE: Because difficult vaginal delivery is more frequent with macrosomic fetuses, some authors recommend routine caesarean section for the delivery of fetuses >4500 g. The purpose of this study was to evaluate the appropriateness of this recommendation, in particular, to analyze maternal and fetal complications according to the mode of delivery. METHOD: Maternal and neonatal records of 100 infants with weights of at least 4500 g were identified retrospectively from January 1991 to December 1996. Outcome variables included the mode of delivery and the incidence of maternal and perinatal complications. RESULTS: The study sample consisted of 100 infant and mother pairs. Macrosomic fetuses represented 0.95% of all deliveries during this period and only ten were >5000 g. Mean birth weight was 4730 g (maximum, 5780 g). Gestational diabetes was present in nineteen patients. Diabetes was present in three patients. A trial of labour was allowed in 87 women, and elective caesarean delivery was performed in thirteen patients. The overall cesarean rate, including elective caesarean delivery and failed trial of labour, was 36%. Of those undergoing a trial of labour, 73% (64/87) delivered vaginally. Shoulder dystocia occurred fourteen times (22% of vaginal deliveries) and it was the most frequent complication in our series. There were five cases of Erb's palsy, one of which was associated with humeral fracture, and four cases of clavicular fracture. By three months of age, all affected infants were without sequelae. There was no related perinatal mortality and only two cases of birth asphyxia. Maternal complications with vaginal delivery of macrosomic infants included a high incidence of lacerations requiring repair (eleven cases). No complications were noticed in the patients who had a caesarean section. CONCLUSION: Vaginal delivery is a reasonable alternative to elective cesarean section for infants with estimated birth weights of less than 5000 g and a trial of labour can be offered. For the fetuses with estimated birth weight >5000 g, an elective caesarean section should be recommended, especially in primiparous women.