Bilateral hand transplantation: Supporting the patient's choice.

Bilateral hand transplantation, as a fairly new reconstructive option for amputees, raises major ethical questions. This article, which is based on the reflections arising from the rich experience of Lyon's team in this field, addresses the topic of supporting the patient in his choice for or against this procedure. How should autonomy be understood in this particular setting? The developing field of composite tissue allotransplantation needs to establish a common thinking on this subject. The article emphasises that, even if it is their right to decide, patients have to be carefully supported to help them make the most consolidated choice possible in this challenging procedure. We deal with the question of the choice between the uncertainty in this innovative procedure and a life-threatening treatment to alleviate a handicap. We outline that the entire process of hand allograft is a unique opportunity for the patients to strengthen and exercise their autonomy in interaction with the medical team.

Plasma renin activity in normal subjects from infancy to puberty.

Immunoassayable Plasma renin activity (PRA) was measured in normal subjects aged from 15 days to 15 years. A very progressive decrease of PRA with age could be demonstrated. Practical conditions for the study of PRA were defined.

Unusual low plasma renin hypertension in a child.

A four-year-old girl with hypertension (140/60) and chronic hypokalemic alkalosis was studied to determine the origin of this clinical feature. High exchangeable sodium (56.7 meq/kg vs. 45-55 meq/kg in controls) was associated with a low plasma renin activity (6 ng/1/min vs. 26 +/- 3.1 in controls) and reduced aldosterone secretion rate (5.56 mug/day; normal: 50-150 mug per day)). A low corticosterone secretion rate (0.228 mg/day vs. 0.50-0.65 in controls) and urinary tetrahydrodeoxycorticosterone (0.007 mg/day vs. 0.03-0.09 mg/day in controls) were found. The basal secretion rate of cortisol was also low (1.80 mg/m2/day vs. 5.4-16.7 mg/m2/day in controls) in spite of normal plasma ACTH: 78 pg/ml. The normal increase of the cortisol secretion rate (from 1.80 to 65 mg/m2/day) after synthetic ACTH stimulation ruled out a 17 alpha hydroxylase deficiency. The low sweat Na/K ratio (0.25) and the good suppressing efficacy of dexamethasone and of the spironolactones on hypertension and on the hypokalemic alkalosis agreed with the hypersecretion of a mineralocorticoid. The secretion rate of 18 hydroxydeoxycorticosterone was high (91 mug/day/1.73 m2 vs. 40-80 mug per day and per 1.73 m2). As the mineralocorticoid potency of this steroid is weak, we speculate that it might be the precursor of a more potent but unknown mineralocorticoid which could influence the ACTH secretion.

Variations of phylloquinone concentration in human milk at various stages of lactation and in cow's milk at various seasons.

An improved high-performance liquid chromatographic method was used to measure phylloquinone concentrations in human and cow's milk. Concentrations in human milk obtained serially from 10 mothers 3, 8, and 21 days after delivery were found to increase during the course of lactation. No correlation was found between phylloquinone concentration and lipids but a significant negative correlation was observed between phylloquinone and cholesterol concentrations. Determinations in cow's milk showed increased levels during the summer months. Also, levels in cow's milk were significantly higher than in human milk. Notwithstanding marked individual variations in human milk, phylloquinone concentrations in cow's milk always proved higher than in human milk. Results prompt the necessity of protecting breast-fed infants by applying relevant prophylactics measures.

Effect of oral administration of lipids with 67% medium chain triglycerides on glucose homeostasis in preterm neonates.

Since hypoglycemic responses to medium chain triglycerides (MCT) have been reported in adults we studied the effect of an acute oral load of lipids (2,8 g/kg) with 67% MCT on glucose homeostasis in 21 preterm infants in comparison to 14 age-matched control preterm infants. A hyperglycemic response from (mean +/- SEM) 57 +/- 1.1 to 74 +/- 2.5 at 30 min (p less than 0.01) and to 80.5 +/- 2.5 mg/dl at 60 min (p less than 0.01) was observed after administration of the lipids whereas no change in plasma glucose concentration was observed in the control group. After administration of the lipids there was no change in the concentration of insulin and glucagon in plasma. An intravenous glucose tolerance test (1 g/kg) was similar in the control group and 60 min after administration of the lipids. After administration of the lipids free fatty acid concentration remained unchanged while a significant decrease from 304 +/- 56 to 199 +/- 28 muEq/l was observed in 60 min in the control group. At 60 min beta-hydroxybutyrate concentration was higher after lipid administration (630 +/- 86 mumol/l) than in the control group (436 +/- 66 mumol/l) (p less than 0.05). A more rapid decrease in blood lactate concentration was found after lipid administration than in the control group while no change in plasma alanine concentration was observed in either groups. In five additional preterm infants, plasma glucose concentration increased from 56 +/- 0.6 to 75 +/- 0.9 mg/dl (p less than 0.01) 60 minutes after gastric administration of glycerol (0.3 g/kg). These data show that in preterm infants, a lipid load with 67% TCM produces a hyperglycemic response through gluconeogenesis without changing the peripheral rate of glucose disappearance.

Gamma-carboxyglutamic acid in urine of newborn infants.

Gamma-Carboxyglutamic acid (GLA) was measured in the urines obtained from 11 full-term infants, 48 pre-term infants appropriate for gestational age (AGA), and 25 small-for-gestational age (SGA) infants. Separation was performed by high resolution anion exchange chromatography. The results were similar in both AGA and SGA infants. During the first 3 days of life, urinary GLA mean (and range) was 1.66 (0.34-4.60) in the low birth weight infants versus 0.88 (0.26-1.38) in the full-term infants and 0.76 (0.62-1.15) mumol . kg-1 X 24 h-1 in the control adults. In the low birth weight infants, urinary GLA fell from 2.79 (0.61-5.75) at age 1-3 days, to 1.55 (0.26-4.04) mumol/24 h at day 8 (p less than 0.01); it then rose again slowly to 2.12 (0.65-3.93) mumol/24 h at day 45. In these infants there was no correlation between urinary GLA excretion and birth weight or gestational age, or urinary hydroxyproline or serum alkaline phosphatase. Despite the well-known reduced blood levels of vitamin K dependent coagulation factors in neonates, these results show that urinary GLA excretion is at least similar to the excretion in adults. These data suggest that these neonates can carboxylate glutamic acid and that the newborn infant has a high bone turnover.

Spontaneous otoacoustic emissions in preterm neonates: prevalence and gender effects.

A number of lines of evidence indicate that the human cochlea is fully functional as a mature sound transducer by 6 months of age. However, information about the development of the active cochlear mechanisms and notably the development of outer hair cell (OHC) activity is yet incomplete. Recording and analysis of otoacoustic emissions (OAEs), probably generated by the OHCs of the organ of Corti, have led to a better understanding, in humans, of how sounds are analysed in the cochlea by means of active mechanisms. Evoked OAEs (EOAEs) and spontaneous OAEs (SOAEs), when they can be recorded in full-term and preterm neonates, show different characteristics from those in adults, suggesting that maturation of the peripheral auditory system is incomplete at birth. To learn more about this maturation, using the best-established facts concerning SOAEs in adults, such as their greater prevalence in females and also in right ears, SOAEs were studied in more detail in 81 preterm neonates, from 30 to 40 weeks of conceptional age, all presenting bilateral EOAEs according to objective criteria. The first finding of this study was that SOAEs existed and could be recorded as of 30 weeks of conceptional age in humans. Some SOAE characteristics in preterm neonates, such as prevalence, peak number and acoustic frequencies, showed similarity with full-term neonates. Comparison of other criteria between the two populations, such as greater SOAE prevalence in right ears and higher SOAE peak number in females, suggested that these developmental factors emerge around term in humans. Comparison of SOAE characteristics between male and female preterms suggested that male preterms were less advanced in peripheral auditory development than were female preterms.

Assessment of proportional growth of very low birth weight infants fed banked human milk.

Twenty appropriate (mean +/- S.D., gestational age (AGA): 29.9 +/- 1.5 weeks) and 15 small (GA: 34.6 +/- 2.4 weeks) for gestational age (SGA) very low birth weight infants fed banked mature human milk were studied until term for anthropometric parameters: midarm (MAC), chest (CC), head (HC) circumferences, triceps (TSKF) and subscapular (SSKF) skinfold thickness recorded at 15 and 60 s, dynamic skinfold (delta % SKF), muscle (AMA) and fat (AFA) areas, weight and length. In AGA infants, all the parameters at term were significantly lower in extrauterine (EUL) that in intrauterine life (IUL). At term the relative proportion of AFA to total arm area was increased in EUL compared to IUL both in AGA (25.87 +/- 3.8 vs. 23.26 +/- 1.27% respectively, P less than 0.01) and in SGA infants (21.89 +/- 4.63 vs. 18.81 +/- 3.9 respectively, P less than 0.05). SGA infants showed a similar growth in EUL compared to IUL, and a significantly lower AMA and AFA than in AGA infants in EUL. Although HC was in both infants below the 10th centile at term, the ratio weight/HC2 suggests a relative preservation of head growth in EUL compared to IUL (AGA: 20.72 less than 0.87 vs. 22.65 +/- 1.46 respectively, P less than 0.001; SGA; 20.82 +/- 1.16 vs. 21.62 +/- 1.86 respectively, NS). Delta %SKF were negatively correlated with post-conceptional age suggesting a loss of extracellular water in AGA (delta %TSKF: r = -0.287, P less than 0.02) and in SGA infants (delta %TSKF: r = -0.301, P less than 0.02; delta %SSKF: r = -0.316, P less than 0.02). An intrauterine model of discrimination between AGA and SGA infants does not apply to EUL. An equation was established in SGA infants with the best discriminant parameters giving a predictive post-conceptional age: post-conceptual age (PCA) (weeks) = 0.276 HC (cm) + 0.723 CC (cm) - 0.122 MAC (cm) + 0.5 TSKF (mm) + 10.173, (r = 0.867, P less than 0.001) allowing a clear discrimination between AGA and SGA infants. These results suggest that infants show quite different growth patterns between IUL and EUL both for AGA and SGA infants.

Anthropometric assessment of nutritional status in newborn infants. Discriminative value of mid arm circumference and of skinfold thickness.

74 appropriate-for-gestational age (AGA) and 22 small-for-gestational age (SGA) caucasian infants were studied for anthropometric parameters: mid arm circumference (MAC), triceps and subscapular skinfold thickness (TSKF and SSKF) recorded at 15 and 60 s, chest circumference (cc), head circumference, birth weight and length. MAC is highly correlated with birth weight either in AGA (r = 0.936; P less than 0.001) or in SGA infants (r = 0.860; P less than 0.001). MAC is also correlated with gestational age in AGA (r = 0.850; P less than 0.001) and SGA infants (r = 0.76; P less than 0.001). Similar correlations were found between TSKF, SSKF and birth weight or gestational age. Arm muscle and fat areas are also positively correlated with birth weight and gestational age, in AGA and SGA infants. A multiple regression analysis of our data allowed a classification of the best discriminant anthropometric parameters between AGA and SGA infants. MAC, SSKF15, SSKF60 and chest circumference were selected. An equation was established in AGA infants with these four parameters giving a predictive gestational age: gestational age (weeks) = 1.216 MAC (cm)-3.588 SSKF15 (mm) + 0.263 CC (cm) + 17.9. The ratio of predicted gestational age to the real gestational age was 1.0 +/- 0.044 in AGA versus 0.896 +/- 0.034 in SGA infants. Our data suggest that MAC and SSKF provide a simple measure of body composition of neonates and a useful tool for determining the degree of maturity of a newborn independent of birth weight.

Non-linear patterns of growth in very low birth weight infants.

The deviations of daily weight, weekly length and head circumference from linear growth were analyzed in 87 very low birth weight infants. The deviations exhibited a highly significant polynomial oscillation not only for weight, but also for length and head circumference. The weight amplitudes were larger for males than for females. They were also larger in infants appropriate for gestational age than in small-for-date infants. The difference with trophicity may be related to the process of adjustment of catch-up in small-for-date infants. However, the mechanisms of these oscillatory deviations could also be explained by clinical events, method of feeding, or homeostatic regulation. Further studies are required to elucidate the role of the different factors. Am. J. Hum. Biol. 10:637-646, 1998. © 1998 Wiley-Liss, Inc.

Hepatic concentration of vitamin K active compounds after application of phylloquinone to chickens on a vitamin K deficient or adequate diet.

Liver and serum concentrations of vitamin K active compounds were measured in two groups of (deficient and normal) broilers after administration of phylloquinone 1 mg/kg. Assays were performed by HPLC after extraction and purification of these compounds. The only menaquinone found in the chicken was menaquinone-4. In the deficient group, the chickens exhibited hepatic concentrations of vitamin K1, vitamin K1 epoxide and menaquinone-4 markedly lower than those of the control group. After administration of phylloquinone, vitamin K and vitamin K epoxide levels fell sharply. There is no hepatic storage of vitamin K comparable to that of vitamin A. However, while menaquinone levels were found to be stable in the control group, they rose significantly in the deficient group after vitamin K injection. The question is: is there a transformation of vitamin K into menaquinone and/or is there a preferential utilization of one of the vitamin K active compounds?

[Pediatric liver transplantation and related live donor. Technical and ethical considerations]. / Transplantation hépatique pédiatrique et donneur vivant apparenté. Considérations techniques et éthiques.

With improved results of liver transplantation, the number of candidates is increasing. However the scarcity of suitable grafts from cadaveric donors remains a limitation. In spite of the use of full size or reduced size grafts or partial grafts from split livers, some children still die while waiting for liver transplantation. We describe a successful orthotopic liver transplantation in a 10 months old female using the left lateral lobe (segments II and III) from her 27 years old father. The child suffered from biliary atresia, her condition was deteriorating with intractable ascites and increasing jaundice. The father asked us to give a part of his own liver to his daughter. The concept of this innovative therapy had already been submitted to a research-ethics consultation which gave us favorable conclusions. After careful donor evaluation, the left lateral lobe was harvested on July 22, 1992, including the left hepatic artery, left portal vein and left hepatic vein; hepatic artery for segment IV, which arose from the right structures, was preserved, The graft was immediately transplanted orthotopically after recipient total hepatectomy with inferior vena cava preservation. Cold ischemia time was 1 hour and 45 minutes, revascularization of the graft was homogeneous from the very beginning and its early function was excellent. Thirteen days after the operation, the donor was discharged in good condition. The child was reoperated at day 9 for a small biliary leak originating from the cut surface of the liver. After resolution of an episode of rejection and an intra-abdominal abscess, the child was discharged in good health with normal liver function 1 month post-transplant.(ABSTRACT TRUNCATED AT 250 WORDS)

-A new role for the pediatrician: the prevention of social violence-. / Un nouveau rôle pour le pédiatre: la prévention de la violence sociale.

Social violence is a kind of violence which is applied by one individual to another. It is a stake in the field of public health because of an increasing number of children with such behaviors. In addition to environmental factors, the occurrence of abnormal social behavior before 9 years of age seems to precede social violence for the subsequent 20 years of life. One must therefore identify an inadequate attitude towards the parents and/or the teachers, or a poor child's self-esteem and self-respect. These phenomena are based on parental authority, on the ethical development of the child, on his verbalization capacity, on his socialization and his affective development. It is therefore important to stimulate ethical capacities and feelings in the child not only to provide autonomy but also to allow reflexive distinction of language, behavior and judgment, which enable him to understand, consider and respect other individuals.

[Outcome of 249 premature infants, less than 29 weeks gestational age]. / Devenir de 249 prématurés de moins de 29 semaines.

UNLABELLED: The aim of this paper was to report the vital and neurological outcome of 249 preterm infants of less than 29 weeks born between 1990 and 1996, and included in a prospective study until two years of age. RESULTS: The initial mortality rate was 19%. This was related to gestational age and severe transfontanellar ultrasonographically (TFU) detected abnormalities. The rate of follow-up at two years of age was 98%. Neurological sequelae amounted to 12.8%, including four cases of deafness. The possibility of survival without neurological sequelae increased from 52% at 24-25 weeks to 72% at 26-28 weeks of gestational age (p < 0.005). The presence of sequelae was significantly related to severe cranial ultrasonographically-detected abnormalities, to parental social level, and to early neonatal anemia. Normal TFU and/or isolated periventricular hyperechogenicity could not exclude the presence of neurological sequelae which, however, appeared to be less severe than at the onset. CONCLUSION: Gestational age, severe TFU abnormalities and neonatal anemia play a major role in the rate of mortality and in the neurological sequelae in preterm infants, and can influence the decisions concerning the treatment of this pediatric population.

[Recombinant Human Erythropoietin (r-HuEPO) therapy in a newborn with hereditary spherocytosis]. / Utilisation de l'érythropoïétine chez un nouveau-né atteint de sphérocytose héréditaire.

UNLABELLED: The newborn with hereditary spherocytosis can develop severe anemia, requiring red blood cell transfusions. Therapy with r-HuEPO has been proposed to avoid transfusions. CASE REPORT: Hereditary spherocytosis was diagnosed in a newborn who had severe and early jaundice. He was treated with r-HuEPO, and did not require red blood cells transfusion. CONCLUSION: Recombinant erythropoïetin might be an interesting alternative to red blood cells transfusions during the neonatal period in newborns with hereditary spherocytosis.

[Recent data on cerebral circulation and metabolism of the brain in newborn infants]. / Données récentes sur la circulation cérébrale et le métabolisme du cerveau du nouveau-né.

Recent advances in technology have made it possible to determine cerebral blood flow and metabolism in newborn infants. The mean cerebral blood flow rate falls from 60 to 30 ml/min/100 g during the first 3 hours of life and returns to the initial value after the first week. It increases by about 30% during rapid sleep and feeding. Cerebral blood flow is independent of gestational age and represents 20-25% of cardiac output as against 16% in adults. It is influenced by blood gases and its autoregulation is impaired by asphyxia. Infants with a cerebral blood flow rate below 20 ml/min/100 g are at high risk of leucomalacia. The high metabolic activity of the neonatal brain is reflected in its considerable consumption of oxygen (54% vs 30% in adults) and ketonic bodies which participate for 10% in cerebral energy metabolism. More than 80% of the endogenous glucose is utilized by the brain of neonates despite a transport capacity lower than in adults. In neonatal pathology, it is now possible to investigate cerebral metabolism using positron emission tomography or phosphorous magnetic resonance spectroscopy which provide for better diagnostic and prognostic evaluation of cerebral functions.