Michael Schroth

Very early extubation after open-heart surgery in children does not influence cardiac function.

The objective of our study was to evaluate hemodynamic effects and the cardiac function after very early extubation within the first 6 hours after open-heart surgery in children. During a 12-month period, we performed a retrospective study of 50 children (ages 3 months to 7 years) admitted to the pediatric intensive care unit immediately after minor cardiac surgery. All children were extubated within the first 6 hours after their arrival. Arterial blood and central venous pressure were monitored, and arterial blood gas analysis was performed. Cardiac index, stroke volume index, systemic vascular resistance index, and extravascular lung water index were measured by thermodilution. Early extubation of children after minor open-heart surgery with cardiopulmonary bypass is safe and does not affect cardiac functions. A slight decrease of arterial oxygen tension not resulting in respiratory or metabolic acidosis or reintubation was noted. Very early extubation in children after open-heart surgery does not promote cardiodepressive effects. It is a safe procedure that helps to reduce the unnecessary and prolonged mechanical ventilation of children after cardiopulmonary bypass surgery.

Recombinant Factor XIII Reduces Severe Pleural Effusion in Children after Open-Heart Surgery

Chylous effusions frequently occur after cardiac surgery due to severe damage to the lymphatic system, thus indicating that the insertion of a chest tube may be necessary. Factor XIII (FXIII) is discussed as being essential for wound healing. The aim of this retrospective study was to evaluate whether the application of a single dose of FXIII results in a reduced amount of pleural effusion, leading to an earlier release of patients from the hospital. The cases of 40 children with severe chylous effusions after open-heart surgery were examined. Twenty patients received FXIII and were compared to 20 age- and weight-matched patients who did not receive FXIII. Major parameters included the amount of effusion before and 1 and 3 days after the application of FXIII; the duration of chest tubes; the total amount of fluid loss via drainage; and the period of hospitalization. FXIII levels in plasma showed an inverse correlation with fluid loss. After application of a single dose of FXIII, a significant reduction of pleural effusion within the first 24 hours was detected. However, no difference was observed between the two groups when comparing the total amount of pleural effusions within the first 72 hours. Finally, the duration of hospitalization did not differ between the FXIII-treated and the control group. A single application of FXIII rapidly reduces the amount of chylous effusions in the early period after open-heart surgery. This effect is detectable only for 24 hours after the treatment and does not alter the further clinical outcome. Prospective clinical trials are warranted to determine if repeated application or a higher dose of FXIII may improve the clinical outcome of chylous leakages in children after open-heart surgery.

Anesthetic management of patients with ornithine transcarbamylase deficiency

Ornithine transcarbamylase deficiency (OTCD) is the most common inborn error of the urea cycle. Several specific factors require care during anesthesia in patients with this condition to avoid metabolic decompensation with acute hyperammonemia and encephalopathy. We report monozygous twins with severe neonatal‐onset OTCD undergoing general anesthesia twice each, with midazolam, s‐ketamine, fentanyl and isoflurane in combination with surgical field infiltration with ropivacaine. Alternative pathway medication and high‐caloric diet with 10% glucose solutions were continuously administered during the perioperative course. Both children were extubated within 10 min of the final suture, and their neurological state remained unchanged. Perioperatively, blood ammonia levels remained within the normal range.

Alterations of leptin and ghrelin serum concentrations in renal disease: simple epiphenomena?

The hypothesis that alterations of serum concentrations of the anorexigenic adipose tissue-derived hormone leptin or the orexigenic peptide ghrelin might help to regain appetite and fight malnutrition in patients with chronic renal failure cannot be confirmed at present. For the future, however, strategies interfering with signal transduction of these peptides in the hypothalamus might be more promising and should be investigated and further developed.

Der Patient mit Ornithintranscarbamylasemangel

ZusammenfassungDer Ornithintranscarbamylase- (OTC-)Mangel ist der häufigste angeborene Defekt des Harnstoffzyklus. Patienten mit dieser Erkrankung sind zeitlebens durch eine metabolische Dekompensation mit Hyperammonämie und konsekutiver Entwicklung einer akuten Enzephalopathie mit Hirnödem bedroht. Auslösende Faktoren für die Erstmanifestation bzw. eine Dekompensation bei bekanntem OTC-Mangel stellen katabole Stoffwechsellagen mit gesteigertem Proteinmetabolismus, etwa durch Fasten oder Nahrungsumstellungen, Proteinexzesse, Infektionen, bestimmte Medikamente sowie Stress infolge Geburt, Trauma, Operation und Narkose dar. Hieraus ergibt sich eine besondere Bedeutung dieser Erkrankung für die perioperative Medizin. Im Folgenden soll daher anhand der Kasuistik einer Erstmanifestation und der Kasuistik einer problemlosen Narkose bei bekanntem OTC-Mangel das anästhesiologische bzw. intensivmedizinische Management von Patienten mit OTC-Mangel besprochen werden.AbstractOrnithine transcarbamylase deficiency (OTCD) is the most common inborn urea cycle disorder. Patients with OTCD are at risk of acute metabolic decompensation with hyperammonemia and subsequent encephalopathy, coma and death. Symptoms may be triggered by infections, drugs and stress, evoked by trauma, pain, fear, surgery and anaesthesia or by episodes of protein catabolism, i.e. fasting-induced, post partum or during gastrointestinal bleeding. Several specific considerations must be made for anaesthetic and intensive care management in patients with this disease in order to avoid metabolic decompensation. We report the intensive care management of the first manifestation of late-onset OTCD in a 16-year-old girl and a course of inconspicuous general anaesthesia with midazolam, s-ketamine, fentanyl and isoflurane in a 22-year-old girl with known OTCD.

Patients with ornithine transcarbamylase deficiency. Anaesthesiological and intensive care management

ZusammenfassungDer Ornithintranscarbamylase- (OTC-)Mangel ist der häufigste angeborene Defekt des Harnstoffzyklus. Patienten mit dieser Erkrankung sind zeitlebens durch eine metabolische Dekompensation mit Hyperammonämie und konsekutiver Entwicklung einer akuten Enzephalopathie mit Hirnödem bedroht. Auslösende Faktoren für die Erstmanifestation bzw. eine Dekompensation bei bekanntem OTC-Mangel stellen katabole Stoffwechsellagen mit gesteigertem Proteinmetabolismus, etwa durch Fasten oder Nahrungsumstellungen, Proteinexzesse, Infektionen, bestimmte Medikamente sowie Stress infolge Geburt, Trauma, Operation und Narkose dar. Hieraus ergibt sich eine besondere Bedeutung dieser Erkrankung für die perioperative Medizin. Im Folgenden soll daher anhand der Kasuistik einer Erstmanifestation und der Kasuistik einer problemlosen Narkose bei bekanntem OTC-Mangel das anästhesiologische bzw. intensivmedizinische Management von Patienten mit OTC-Mangel besprochen werden.AbstractOrnithine transcarbamylase deficiency (OTCD) is the most common inborn urea cycle disorder. Patients with OTCD are at risk of acute metabolic decompensation with hyperammonemia and subsequent encephalopathy, coma and death. Symptoms may be triggered by infections, drugs and stress, evoked by trauma, pain, fear, surgery and anaesthesia or by episodes of protein catabolism, i.e. fasting-induced, post partum or during gastrointestinal bleeding. Several specific considerations must be made for anaesthetic and intensive care management in patients with this disease in order to avoid metabolic decompensation. We report the intensive care management of the first manifestation of late-onset OTCD in a 16-year-old girl and a course of inconspicuous general anaesthesia with midazolam, s-ketamine, fentanyl and isoflurane in a 22-year-old girl with known OTCD.

Hypercalcemia and idiopathic hypoparathyroidism

Idiopathic primary hypoparathyroidism (prHP) is a rare disorder and clinical experience of its management is limited. Prolonged immobilization of such patients can cause hypercalcemia and hypercalciuria. We report on a boy with prHP who developed hypercalcemia and renal failure as a result of calcium and calcitriol substitution not being stopped while he was immobilized for 2 months. Any substitution in patients with prHP must be stopped during prolonged immobilization. Laboratory monitoring is mandatory during this period.

Epstein-Barr virus triggered rejection after kidney allograft transplantation.

Pediatric allograft recipients are at increased risk for Epstein-Barr virus (EBV)-associated disorders. We report on a 4-year-old boy who received a cadaver kidney transplant. The donors viral status was not known. Twenty-one days after transplantation, serum creatinine and urea rose, giving evidence of transplant rejection. At this stage, EBV DNA in the recipient tested positive. The results of histological examination of the allograft kidney were interpreted as being EBV associated. For treatment of acute rejection immunosuppressive therapy was intensified. Finally, the renal transplant had to be explanted 46 days after implantation and peritoneal dialysis had to be restarted to compensate renal failure. In conclusion, EBV infection can lead to rapid rejection, and modification of immunosuppression does not necessarily improve allograft survival.

Influence of factor XIII activity on post-operative transfusion in congenital cardiac surgery—A retrospective analysis

Objectives Coagulation factor XIII (FXIII) plays a key role in fibrin clot stabilization—an essential process for wound healing following cardiothoracic surgery. However, FXIII deficiency as a risk for post-operative bleeding in pediatric cardiac surgery involving cardiopulmonary bypass (CPB) for congenital heart disease (CHD) is controversially discussed. Thus, as primary outcome measures, we analyzed the association of pre-operative FXIII activity and post-operative chest tube drainage (CTD) loss with transfusion requirements post-operatively. Secondary outcomes included the influence of cyanosis and sex on transfusion. Methods Our retrospective analysis (2009–2010) encompassed a single center series of 76 cardio-surgical cases with CPB (0–17 years, mean age 5.61 years) that were post-operatively admitted to our pediatric intensive care unit (PICU). The observational period was 48 hours after cardiac surgery. Blood cell counts and coagulation status, including FXIII activity were routinely performed pre- and post-operatively. The administered amount of blood products and volume expanders was recorded electronically, along with the amount of CTD loss. Uni- and multivariate logistic regression analysis was performed to calculate the associations (odds ratios) of variables with post-operative transfusion needs. Results FXIII activities remained stable following CPB surgery. There was no association of pre- and post-operative FXIII activities and transfusion of blood products or volume expanders in the first 48 hours after surgery. Similarly, FXIII showed no association with CTD loss. Cyanosis and female sex were associated with transfusion rates. Conclusions Although essentially involved in wound healing and clotting after surgery, FXIII activity does not serve as a valid predictor of post-operative transfusion need.

Erratum to Recombinant Factor XIII Reduces Severe Pleural Effusion in Children after Open-Heart Surgery

RE: Pediatr Cardiol 27:56–60 (2006)—In this article we retrospectively evaluated data demonstrating that severe pleural effusions due to open heart surgery in children correlated with the postoperative plasma level of human factor XIII. Substitution of factor XIII leads to a significant reduction of fluid loss in these children. Unfortunately, in the title and in the text it was stated that ‘‘recombinant’’ factor XIII was used; this is incorrect. In this study we used human ‘‘plasmatic’’ factor XIII. We apologize for this error and the misleading information it may have conveyed.