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.

[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.

[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.

-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.

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.

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.

Changes in hepatic vitamin K1 levels after prophylactic administration to the newborn.

We undertook a study of hepatic concentrations of vitamin K (vitamin K1 or phylloquinone, vitamin K1-epoxide, and menaquinones) in 18 infants, ages 1-8 days, with or without vitamin K1 supplementation. The infants who had no supplementation had a total hepatic storage ranging between 0.1 and 0.9 micrograms. Also, hepatic storage of phylloquinone was poor (< 1 microgram) when compared with daily requirements. Moreover, we did not detect any menaquinone in the livers of these infants in our study. The prophylaxis applied to the other infants was very efficient. Hepatic vitamin K1 concentrations, obtained < 24 h after administration, were very high (62.8-93.5 micrograms/g). Vitamin K1-epoxide concentrations were high, which proved the efficiency of the vitamin K cycle. In contrast, the decrease in vitamin K1 concentrations was also very rapid, since the median value after 48 h was 8.4 micrograms/g and only 2.9 micrograms/g 5 days after administration. However, hepatic total storage after 5 days in one infant with vitamin K1 supplementation was much higher (112 micrograms) than in infants who had not received supplementation. In conclusion, hepatic phylloquinone storage at birth was poor (< 1 microgram). The newborn infant might be in a situation of potential deficiency. After prophylactic oral administration of phylloquinone, uptake by the liver was quite satisfactory, but concentrations dropped quickly. However, phylloquinone hepatic storage remained elevated (112 micrograms) after 5 days.

[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)

[Ethical considerations on live transplantation from living related donors. Le conseil d'éthique de l'hôpital Debrousse]. / Considérations éthiques sur la transplantation hépatique à partir d'un donneur vivant apparenté. Le conseil d'éthique de l'hôpital Debrousse.

This review examines the ethical problems of liver transplantation from living parental donors regarding the main ethical principles. It appears that this method of transplantation is a new possibility offered to patients that cannot be subtracted from the parents' choice without interfering with their liberty. Moreover, there is a social decisiveness to ensure the offering of this option which is limited by the conditions of realisation, timing and personal situations of the parents. The considerations of risks, benefits, advantages and prejudices between donor and recipient show a favourable balance for this type of transplantation provided that adequate medical indications are respected. However, the considerations of psychological cost and burden deserve special attention and are detailed in this paper especially regarding the motivations and the long-term effects on the donor. Futility and ethical generalisation examinations favour such a procedure. Moral justice will generally be respected. The consideration of autonomy required special attention owing to the situation pressure upon the donor's consent. Consent deserves adapted solicitation according to variable situations: whether the donor explicitly specifically wanted to donate part of his liver or only required information. Excessive solicitation of parents' participation to liver donation represents qualified "forced choice"; in some situations where the parents cannot effectively refuse their participation, paternalistic action is ethically acceptable. A general procedure is proposed to ensure the psychological, legal and ethical protection of the donor and recipient.

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?

Ultrasound diagnosis of midgut volvulus: the "whirlpool" sign.

The authors present their US findings in 24 patients with proved complicated midgut malrotation: volvulus in 18 and occlusive Ladd's bands in 6. All the 24 patients have had US examination prior to surgery. Contrast examinations were performed in only 9 patients, always after US and before surgery. The sonographic "whirlpool" pattern of the superior mesenteric vein and mesentery around the superior mesenteric artery was detected in 15 of the 18 patients with midgut volvulus, and was best seen using Doppler color. Embryological signification of midgut malrotation is discussed.

Neonatal hypoglycemia.

Neonatal hypoglycemia is particularly dangerous for the brain when it is symptomatic or/and recurrent. In order to avoid symptomatic hypoglycemia a compensatory production of ketone bodies is required since they are actively taken up by the neonatal brain. Recurrent hypoglycemia can be avoided by stimulation of neonatal gluconeogenesis which is limited by glucose infusion and/or insulin secretion. Lipid administration induces a hyperglycemic response which is explained by a stimulation of gluconeogenesis. It also results in ketogenesis which provides the compensatory factors of hypoglycemia and which seems to be linked to gluconeogenesis. We therefore suggest that the best way to prevent neonatal hypoglycemia is achieved by supplementing the neonate with lipids and especially medium chain triglycerides.