1/86. pastoral care in Candyland. Maintaining power and life in a world of illness.wrestling with ghosts and staging fatal accidents with Matchbox cars allow a critically ill six-year-old to come to terms with the fears prompted by his illness.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
2/86. Living donor liver transplantation in critically ill children.From December 1993, St Christopher's Hospital for Children, philadelphia, PA, USA has provided living donors the opportunity to donate a portion of their liver to children who are critically ill. This report evaluates the results of living donor liver transplants (LDLT) in critically ill children. We retrospectively reviewed the first 22 LDLT at our institution and compared the patient and graft survival of the nine critically ill children with the 13 stable children. Twenty-two LDLT have been performed at our institution between December 1993 and October 1997. Nine of 22 transplants [United Network for Organ Sharing (UNOS) Status I] were performed in children who were critically ill. Thirteen of the LDLT (UNOS Status II and III) were performed on stable children either in the hospital or admitted electively from home. The median weight and age at the time of transplant were 7 kg (range 4.6-54.5 kg) and 16 months (range 3 months-12 yr), respectively, and there was no statistical difference between the two groups. In critically ill children the 1-yr allograft and patient survival was 66% and 89%, respectively, exceeding the published results from UNOS for patients on life support (59.5% graft and 69.7% patient survival at 1 yr). One-yr allograft and patient survival in the stable children was 92.3% and 100%, respectively. All living donors are alive and well with normal liver function. In conclusion, our results show that LDLT is a viable approach for transplantation in critically ill children with liver failure and should be offered to potential donors.- - - - - - - - - - ranking = 0.25keywords = life (Clic here for more details about this article) |
3/86. adult celiac disease presented with celiac crisis: severe diarrhea, hypokalemia, and acidosis.An acute severe onset of celiac disease is very uncommon in adults. We describe a patient with adult celiac disease who presented with acute diarrhea that lead rapidly to a life threatening hypokalemia and acidosis, the so-called celiac crisis. Celiac crisis, described mainly in children younger than two years of age, has become very rare due to earlier diagnosis and effective therapy of the disease. The case described is an example of the heterogeneous clinical course of celiac disease and emphasizes the need to consider it in the differential diagnosis, even in adults suffering from acute diarrhea and acidosis.- - - - - - - - - - ranking = 0.25keywords = life (Clic here for more details about this article) |
4/86. life-threatening splenic hemorrhage in two patients with gaucher disease.Massive splenomegaly is a frequent finding in patients with gaucher disease, the most common of the sphingolipidoses. Even so, the risk for splenic rupture and intracapsular hemorrhage has not been emphasized due to the rarity of this occurrence and the fibrotic, rubbery consistency of splenic tissue in these patients. We report two adult patients with type 1 gaucher disease who suffered life-threatening splenic bleeds that were not acutely diagnosed. Both patients ultimately required emergent splenectomies. Factors complicating the diagnosis of splenic hemorrhage in patients with gaucher disease are discussed. Published 2000 Wiley-Liss, Inc.- - - - - - - - - - ranking = 0.25keywords = life (Clic here for more details about this article) |
5/86. Bedside endosonography and endosonography-guided fine-needle aspiration in critically ill patients: a way out of the deadlock?endosonography and endosonography-guided fine-needle aspiration (EUS-FNA) are now established diagnostic techniques, which are performed electively in endoscopy suites. We report here the bedside use of EUS-FNA in three critically ill patients in an intensive-care unit, with a significant impact on the outcome. A mediastinal abscess after percutaneous dilational tracheotomy was aspirated in one patient, leading to appropriate antibiotic therapy and complete recovery. A paratracheal hematoma compressing the right main bronchus was aspirated in a patient with polytrauma, relieving the pressure effects. The third patient, who had end-stage dilated cardiomyopathy and was being evaluated for cardiac transplantation, was found to have an apical lung lesion suspicious for bronchogenic carcinoma. EUS was performed to exclude mediastinal metastasis and allow simultaneous resection at the time of transplantation. Although a metastasis was excluded by EUS-FNA, the patient died while awaiting surgery. We conclude that bedside EUS-FNA is a feasible procedure, and in experienced hands it can offer an alternative in life-threatening situations.- - - - - - - - - - ranking = 0.25keywords = life (Clic here for more details about this article) |
6/86. fertility following ligation of internal iliac arteries for life-threatening obstetric haemorrhage: case report.Bilateral ligation of internal iliac (hypogastric) arteries (BIL) is a life-saving operation in cases of massive obstetric haemorrhage. This operation preserves reproductive function as opposed to the more commonly performed emergency hysterectomy in such situations. We report on effectiveness and future fertility in 12 women who had internal iliac ligation to control severe obstetric haemorrhage: in 10 out of the 12 women, BIL was successful. Of the two women who subsequently needed emergency hysterectomy, one woman died of disseminated intravascular coagulation. Of the eight women we were able to follow-up to assess reproductive performance, two did not desire future fertility. Three had subsequent pregnancies (50%), of whom two proceeded to term. We conclude that BIL is a safe and effective procedure for treating life-threatening obstetric haemorrhage with preservation of future fertility. This technique should be performed more often when indicated.- - - - - - - - - - ranking = 1.5keywords = life (Clic here for more details about this article) |
7/86. aprotinin in the management of life-threatening bleeding during extracorporeal life support.Contact with the synthetic surfaces of an extracorporeal circuit induces alterations in vascular components, derangements of the coagulation cascade and a systemic inflammatory response. aprotinin reduces intraoperative and postoperative bleeding in adults undergoing cardiopulmonary bypass; however, trials in children have not had similar favorable results. While there have been some anecdotal reports, there have been no prospective clinical trials exploring the utility of aprotinin in the prevention of or as a therapy for bleeding while on extracorporeal life support (ECLS). We present a case series on our experience utilizing aprotinin for the treatment of life-threatening bleeding during ECLS. The combination of a loading dose followed by a continuous infusion resulted in significant reduction in blood loss and blood product utilization. This suggests that aprotinin may have clinical efficacy in the management of massive blood loss while on ECLS; however, larger controlled trials will be essential to determine the efficacy and appropriate dosing regimens before widespread use in ECLS can be advocated.- - - - - - - - - - ranking = 2.5keywords = life (Clic here for more details about this article) |
8/86. Evidence for a cardiac ion channel mutation underlying drug-induced QT prolongation and life-threatening arrhythmias.The aim of this study was to test the hypothesis that some cases of drug-induced arrhythmias depend on genetic predisposition. Excessive prolongation of the QT interval and life-threatening arrhythmias (torsades de pointes or ventricular fibrillation) may occur in response to a variety of cardiac and noncardiac drugs, with detrimental effects on patient safety and the investments made by the pharmaceutical industry. Moss and Schwartz hypothesized that some drug-induced arrhythmias might represent cases of "forme fruste" of the congenital long qt syndrome (LQTS). The availability of molecular screening techniques for LQTS genes allowed us to test this hypothesis. An elderly female patient with documented cardiac arrest related to cisapride, a prokynetic drug that blocks I(Kr), and transiently prolonged QT interval underwent mutational analysis of the known LQTS-related genes performed by single-strand conformational polymorphism and dna sequencing. Double-electrode voltage clamp in xenopus oocytes as the expression system was used to study the in vitro cellular phenotype caused by the genetic defect in coexpression with the wild-type (WT) gene. Molecular analysis revealed a heterozygous mutation leading to substitution of a highly conserved amino acid in the pore region of KvLQT1. This mutation was present not only in the patient with ventricular fibrillation but also in her two adult asymptomatic sons who have a normal QT interval. in vitro expression of the mutated KvLQT1 protein showed a severe loss of current with a dominant negative effect on the WT-KvLQT1 channel. Our findings demonstrate that some cases of drug-induced QT prolongation may depend on a genetic substrate. Molecular screening may allow identification among family members of gene carriers potentially at risk if treated with I(Kr) blockers. Evolving technology may lead to rapid screening for mutations of candidate genes that cause drug-induced life-threatening arrhythmias and allow early identification of individuals at risk.- - - - - - - - - - ranking = 1.5keywords = life (Clic here for more details about this article) |
9/86. Treatment of life-threatening lithium toxicity with high-volume continuous venovenous hemofiltration.There is still debate as to the preferred extracorporeal treatment modality for severe lithium intoxication. Because lithium is readily diffusable, intermittent hemodialysis is usually performed. However, this bares the risk of a post-dialysis rebound concentration and, in the case of severe lithium poisoning collapse, aggravation of hemodynamic instability. Because of the relatively slow but continuous solute removal, continuous renal replacement therapy (CRRT) may be advantageous. We report the first case in the literature of severe lithium intoxication treated effectively with high-volume continuous venovenous hemofiltration (HV-CVVH). Results compared favorably to other forms of CRRT in terms of lithium clearance. Ease of implementation, the excellent tolerability and the superior lithium clearance without rebound phenomenon may make HV-CVVH the preferred treatment modality for severe lithium poisoning.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
10/86. Hyperhemolytic transfusion reaction in sickle cell disease.BACKGROUND: An atypical form of life-threatening hemolytic transfusion reaction (HTR) in patients with sickle cell disease (SCD) has been well described in the literature. Continuation of blood transfusion may be lethal, as it can further exacerbate hemolysis. The pathophysiologic mechanism of HTR is not well understood. case reports: Two cases of severe HTR in SCD after the transfusion of compatible RBC units are reported. hemolysis of both autologous and transfused cells was documented in Case 1 by urine Hb high-performance liquid chromotography. Multispecific HLA antibodies were identified in Case 1. Reticulocytopenia was noted in both cases during the acute hemolytic process. This was followed by a rise in reticulocyte count during receipt of IVIG and steroid therapy. bone marrow examination during reticulocytopenia in Case 2 showed erythroid hyperplasia. CONCLUSION: In SCD, both mature sickle cells and sickle reticulocytes adhere more readily to macrophages. In view of the bone marrow aspiration results, it appears that the recipients' HbS cells are destroyed by hyperactive macrophages and that the reticulocytopenia observed during HTR is likely to be due to peripheral consumption (i.e., destruction by macrophages), rather than suppression of erythropoiesis. Cessation of hemolysis during IVIG and steroid treatment may be due to IVIG's blocking of the adhesion of sickle cells and reticulocytes to macrophages, together with steroid suppression of macrophage activity.- - - - - - - - - - ranking = 0.25keywords = life (Clic here for more details about this article) |
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