ABSTRACT
Ehlers-Danlos syndrome type VIIc is characterized by altered tensile strength of connective tissue. Several severe complications exist but skin fragility is the origin of perioperative morbidity during routine procedures. We describe the difficulties encountered during the anaesthetic management of a child suffering from the disease, and suggest special care advices to avoid any skin injury.
Subject(s)
Anesthesia, Inhalation , Ehlers-Danlos Syndrome/complications , Anesthesia, Inhalation/adverse effects , Child , Female , Hemorrhoids/surgery , Hernia, Umbilical/surgery , Humans , Intubation, Intratracheal , Postoperative Complications/etiology , Postoperative Complications/pathology , Thrombosis/surgeryABSTRACT
The Brussels series of living related liver transplantation (LRLT) in 77 children (< 15 years) is reviewed. Median (range) recipient age at liver transplantation was 1.1 year (0.4-13.1). The main indication for LT was biliary atresia in 55/77 cases (71%). The living-related donor was one of the parents in 74 instances. Hepatic segments 2-3 (n = 67) or 2-3-4 (n = 10) were implanted orthotopically, with a median (range) graft weight to recipient body weight ratio of 3.17% (0.91-8.08). No severe complications or significant long-term sequelae were encountered in the living donors. One and five year survival rates were 92% and 89% for the patients, and 90% and 86% for the grafts, respectively. The retransplantation rate was 2/77 (2.6%), the indication being chronic rejection in both instances. In conclusion, LRLT is now a validated procedure in the living donors as well as in pediatric recipients with chronic or acute liver diseases. In the current context of organ shortage, it provides a valuable alternative to cadaveric LT.
Subject(s)
Liver Transplantation , Living Donors , Tissue Donors/supply & distribution , Adolescent , Belgium , Child , Child, Preschool , Female , Follow-Up Studies , Hepatectomy , Humans , Infant , Male , Postoperative Complications/etiology , Postoperative Complications/mortality , Reoperation , Survival RateABSTRACT
Previous studies have shown that the attenuated hypoxic pulmonary vasoconstriction (HPV) of young newborn lamb lungs was enhanced by cyclooxygenase inhibition. We sought to determine whether this reflected greater synthesis of and (or) responsiveness to dilator prostaglandins (PG). Protocol 1 measured responses to graded hypoxia and perfusate concentrations of 6-keto-PGF1alpha (the stable metabolite of PGI2) and PGE2 in isolated lungs from 1-day- and 1-month-old lambs. Protocol 2 compared dose responses and segmental vascular resistances during infusion of PGI2 and PGE2 in hypoxic, cyclooxygenase-inhibited, lungs from 1- to 2-day-old and 1- to 3-month-old lambs. Lungs of 1-day-old lambs with attenuated responses to 4% O2 had significantly higher perfusate concentrations of 6-keto-PGF1alpha and PGE2, but responses to both PGE2 and the more potent vasodilator, PGI2 did not differ with age. These data support the hypothesis that attenuated HPV in young newborn lamb lungs is due to increased synthesis of dilator PG, particularly PGI2.
Subject(s)
Dinoprostone/biosynthesis , Epoprostenol/biosynthesis , Hypoxia/metabolism , Lung/metabolism , 6-Ketoprostaglandin F1 alpha/metabolism , Age Factors , Animals , Animals, Newborn , Blood Pressure , Dinoprostone/metabolism , Dinoprostone/pharmacology , Dose-Response Relationship, Drug , Epoprostenol/metabolism , Epoprostenol/pharmacology , Hypoxia/physiopathology , In Vitro Techniques , Lung/growth & development , Perfusion , Pulmonary Artery/drug effects , Pulmonary Artery/physiopathology , Sheep , Vascular Resistance/drug effects , Vasoconstrictor Agents/pharmacology , Vasodilator Agents/pharmacologyABSTRACT
Hypoxemia in cirrhotic patients is well documented. One of the possible causes of this association seems to be the presence of functional intrapulmonary shunts. The extent of the ventilation/perfusion ratio (VA/Q) abnormalities and their regression after orthotopic liver transplantation has been previously studied in adults by the multiple inert gas elimination technique. We report here a similar study in three children where the hypoxemia was the main indication for early liver grafting, although the liver function was still preserved at that time. Their hypoxemia was almost exclusively caused by a right to left shunt (VA/Q = 0) with a minimal amount of poorly ventilated but well perfused areas (Low VA/Q). This association may explain the poor response of the arterial oxygen pressure to an increased inspired oxygen concentration. Despite these very large VA/Q mismatches, the children underwent successful liver transplantations, resulting in a regression of the intrapulmonary shunt, as demonstrated by multiple inert gas elimination technique, and compatible with a normal life.
