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Biomedical subjects

F C Ryckman

Publications and source records attributed to F C Ryckman.

At least 19 recordsLinked to original sources

The use of extracorporeal life support in pediatric burn patients with respiratory failure.

Respiratory failure is the most common cause of death after thermal injury and may be caused by inhalation injury, acute respiratory distress syndrome (ARDS) or pneumonia. ARDS is usually associated with sepsis; however, it may also occur during burn shock, especially in patients that have a delayed or inadequate fluid resuscitation. During the past 24 months, five pediatric burn patients underwent extracorporeal life support (ECLS) for respiratory failure unresponsive to optimal medical management. The mean age of the patients was 26 months (range, 8.5 to 48 months), with a mean burn size of 46% TBSA (> 95% third degree). The etiology of the respiratory failure included severe bronchospasm in a 22-month-old former premature infant with bronchopulmonary dysplasia; three patients with ARDS; and one patient with a severe inhalation injury. All five patients required greater than 56 cm H2O peak pressures and 100% FIO2 at the time of beginning ECLS. The oxygenation index (OI) ranged from 45 to 180. Three (60%) of the patients survived. In the three patients who ultimately survived, significant improvements in pulmonary and hemodynamic parameters occurred within 96 hours of ECLS. The two patients who died showed no improvement and were removed from ECLS at 10 and 11 days; both expired within hours. The patients who expired developed significant hemodynamic instability, coagulopathy, and hemorrhage from their burn wounds. The extent and degree of burn injury did not seem to alter the outcome. Indications for considering ECLS in the pediatric burn patient are unmanageable, life threatening pulmonary insufficiency in patients that undergo a relative short course of pre-ECLS ventilator support.(ABSTRACT TRUNCATED AT 250 WORDS)

Burns

Traumatic delivery: a risk factor for bleeding during ECMO?

Traumatic delivery is associated with orthopedic, nervous, and soft tissue injuries. Anticoagulation for extracorporeal membrane oxygenation (ECMO) support may predispose to hemorrhage from these birth injuries. A case of delayed hemoperitoneum secondary to hepatic laceration after delivery and ECMO, not previously reported, is detailed herein.

Birth Injuries

Myocardial calcification: a predictor of poor outcome for myocarditis treated with extracorporeal life support.

Myocarditis is an inflammatory disease of the myocardium with a variety of causes. It is potentially reversible, and has been treated successfully with extracorporeal life support (ECLS). With increasing severity, myocarditis results in significant damage to myocardial cells. Dystrophic calcification of the myocardium may occur, serving as a marker of severe damage. At the authors' institution, from July 1990 to January 1992, five patients (four neonates, one 5 year old) with severe myocarditis refractory to medical management were treated with venoarterial ECLS. Three survived (60%) and two died. All patients were female, and their age range was 2 weeks to 5 years. Nonsurvivors had significant myocardial calcification, which was detected by a chest roentgenogram as well as a two-dimensional echocardiogram; the three survivors had no evidence of calcification. The nonsurvivors had minimal recovery of myocardial function and subsequently had their ECLS discontinued at 83 and 169 hours, respectively. The authors conclude that the development of progressive myocardial calcification in conjunction with a lack of recovery of cardiac function is a sign of severe myocardial damage and poor prognosis. Continuation of ECLS in this setting may not be warranted.

Calcinosis

Catheter-induced urinary bladder rupture presenting with pneumoperitoneum.

Neonatal bladder injury is rare and usually associated with umbilical artery catherization. Patients may present with apparent renal failure, abdominal distension, and respiratory distress. Treatment involves operative closure of the perforation and bladder drainage. A case of Foley catheter-induced bladder rupture in a premature infant, not previously reported in the literature, is detailed.

Female

The spectrum of bile duct complications in pediatric liver transplantation.

It has been noted that reduced-size liver transplants are associated with increased rates of biliary complications, and it has been suggested that some of these complications can be handled nonoperatively. In a 6-year period, 91 orthotopic liver transplants were performed in 77 children. The medical records were reviewed to analyze the effect of reduced-size grafts on the incidence of bile duct complications and to investigate the utility of interventional radiology techniques for treatment. Forty-two children received 47 whole-organ transplants, and 35 children received 44 reduced-size transplants. The median age and weight were greater for children receiving whole-organ transplants (age, 4.25 years; weight, 16 kg) than for those receiving reduced-size grafts (age, 1.0 year; weight, 8 kg). The overall incidence of bile duct complications was 19.5% (n = 15). The incidence was not different between the whole organ group (17%) and the reduced-size group (16%). Four of the children with bile duct complications had associated hepatic artery thrombosis, two of whom had another transplant. Complications included anastomotic stricture (n = 6), anastomotic leak (n = 5), intraparenchymal biloma (n = 3), and multiple strictures (n = 1). Twelve of 15 children presented within 3 months of transplantation. Six children had initial percutaneous drainage or placement of transanastomotic stents (external). Operative repair was eventually required for all 15 children, three of whom received a second transplant. There was a 40% incidence of cytomegalovirus infection involving the liver or extrahepatic bile ducts near the time of presentation.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent

Antenatal diagnosis of choledochal cyst.

We report five infants in whom antenatal diagnosis of choledochal cyst was established by ultrasonography, and we review the seven previously reported cases. All but one infant had cystic dilatation of the common bile duct (type 1 cysts), and all infants were diagnosed during the second or third trimester. Eight of 12 infants (67%) developed jaundice in the first few days of life, but only 25% had a palpable abdominal mass. Seven of nine infants (78%) demonstrated complete obstruction of the distal common bile duct on intraoperative cholangiography. Liver histology was available for six patients. Five of six had cirrhosis or fibrosis with bile duct proliferation. All of the infants with fibrosis or cirrhosis had distal common bile duct obstruction. Despite liver biopsy findings of extensive fibrosis plus ascites with failure to thrive in one of our patients, all five patients demonstrated clinical and biochemical improvement following surgical excision and porto- or choledochoenterostomy. All were free of symptoms by 6 months of age. Congenital choledochal cyst should be considered in the differential diagnosis of any sonolucent abdominal mass of the fetus. Neonates with distal common bile duct obstruction and fibrosis in association with presumed choledochal cyst should have prompt surgical exploration, intraoperative cholangiography, and close postoperative follow-up. The long-term outcome with prompt surgical correction is excellent.

Choledochal Cyst

Variations in serum OKT3 concentration based upon age, sex, transplanted organ, treatment regimen, and anti-OKT3 antibody status.

An essential parameter of the efficacy of OKT3 therapy is serial determinations of serum OKT3 levels. We hypothesized that precise monitoring of these levels would optimize treatment protocols. Therefore, enzyme-linked immunosorbent assay (ELISA) technology was utilized to measure OKT3 serum concentrations daily during 263 OKT3 treatment courses in recipients of solid organ grafts. Patient characteristics were: mean age 33 years (0.1-71), 147 male/116 female, 134 kidney/82 liver/47 heart, 122 prophylaxis/141 rejection, and 213 conventional dosing/50 increased dosing. Mean OKT3 levels were higher in women than in men at all time points from day 1 to day 14, reaching the greatest difference between groups on day 7 (849 versus 598 ng/ml, p = 0.004). Patients receiving OKT3 as a component of a prophylactic protocol had higher levels than those receiving the drug for treatment of rejection from day 1 to day 6, with the greatest difference between groups occurring on day 1 (678 versus 333 ng/ml, p < 0.00001). However, from day 7 to day 14 patients receiving OKT3 prophylactically had lower mean OKT3 levels than did those receiving OKT3 for rejection, with the greatest difference between groups occurring on day 11 (555 versus 784 ng/ml, p < 0.05). Liver transplant recipients had significantly higher OKT3 levels than did kidney or heart transplants at all time points. However, more liver patients required increased OKT3 doses to modulate peripheral blood CD3+ cells to < 25/mm3. Kidney recipients had higher levels than did heart recipients. Children < 10 years of age had higher OKT3 levels than did older patients at all time points.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent

The significant relationship between platelet count and haemorrhagic complications on ECMO.

Haemorrhagic complications, which occur in up to 35% of infants during extracorporeal membrane oxygenation (ECMO), often produce devastating sequelae. Although many complex factors interact to control haemostasis, platelet number and function has significant impact on the development of primary haemostasis. The optimum platelet count on ECMO, however, has not been defined. At our institution prior to August 1987, platelet counts were maintained at greater than 100,000/mm3. After August 1987, however, platelet counts of greater than 200,000/m3 were maintained. In a retrospective study, patients were randomly chosen from these two treatment periods: group 1--March 1986 to July 1987; and group 2--June 1988 to June 1989. The average platelet count, platelets administered, hours on ECMO, and bleeding complications were compared to each other and to the July 1992 ELSO Registry. There was a significant difference in average platelet counts between group 1 and group 2. However, the amount of platelets administered per kg per day was similar. There was a significant difference in overall bleeding complications between Group 2 (12%) and the ELSO Registry (35%) (p < 0.01). There was a trend towards decreased complications in all subgroups, although sample size precluded significance. We conclude that increasing platelet counts to greater than 200,000/mm3 decreases the overall bleeding complication rate. This advantage is achieved without a continuous need for increased platelet administration once the desired level is reached and without an increase in perfusion time, mechanical complications, or mortality.

Extracorporeal Membrane Oxygenation

Induction therapy with OKT3 in pediatric liver-transplant recipients.

The goal of any posttransplant immunosuppressive regimen is to prevent allograft rejection while minimizing infectious complications. We hypothesized that sequential induction immunotherapy using the monoclonal antibody ORTHOCLONE OKT3 (muromonab-CD3) would meet these objectives effectively. We have therefore used such a protocol since July 1988 for all pediatric patients undergoing liver transplantation at Children's Hospital Medical Center of Cincinnati. Initial immunotherapy consisted of OKT3, administered preoperatively and then QD, methylprednisolone, and azathioprine. Cyclosporine was begun on POD 3-5, and OKT3 was discontinued when therapeutic cyclosporine levels were achieved for 48 hours. Rejection has not occurred throughout the lifetime of the allograft in 55% of long-term survivors. In the 28 patients who experienced rejection episodes, 71% had a single episode, 21% had two episodes, and 7% had a single episode, 21% had two episodes, and 7% had more than two. Rejection occurring after more than 120 days was invariably associated with noncompliance or subtherapeutic cyclosporine levels. The use of an OKT3-based sequential induction protocol resulted in a decreased incidence of acute rejection. Renal function was preserved, and the incidence of infection was not increased. Long-term outcome analysis of this protocol shows excellent patient survival and the near absence of late or chronic rejection.

Adolescent

Soluble interleukin-2 receptor monitoring during bacterial and viral infections in liver transplant recipients: a comparative evaluation.

We evaluated the significance of serial sIL-2R serum levels as a differential marker of immune activation during bacterial versus viral infections in liver transplant recipients. A comparative evaluation of sIL-2R levels was performed in 76 liver transplant recipients (51 pediatric and 27 adult) during bacterial versus viral infections at 7 days prior to infection diagnosis (DAY-7), the day of diagnosis (DAY 0), peak sIL-2R level and at the end of therapy (END). There were no significant elevations at any time point during bacterial infections in either adult or pediatric transplant recipients. However, adult recipients demonstrated significant elevations during viral infections when comparing DAY-7 to PEAK (3840 +/- 830 vs 7225 +/- 2814 p = 0.03), with PEAK levels significantly higher during viral versus bacterial infections in this population (7225 +/- 2814 vs 4195 +/- 1819). Pediatric recipients demonstrated similar increases in sIL-2R serum levels during viral infections from DAY-7 to PEAK (4932 +/- 887 vs 11323 +/- 2794 p = 0.0012). Significant decreases from PEAK to END were noted during viral infections in both adult and pediatric recipients (7225 +/- 2814 vs 2911 +/- 1376 p = 0.01 and 11323 +/- 2794 vs 5214 +/- 2403 p = 0.006). Pediatric recipients had higher mean sIL-2R levels than adult recipients at all time points during viral infections. In conclusion, significant elevations in mean sIL-2R serum levels were observed during viral but not bacterial infections in pediatric and adult liver transplant recipients. This suggests that serial sIL-2R monitoring is a valuable immunologic marker of viral pathogenesis and may be useful in monitoring the progression of viral infections as well as response to antiviral therapy.

Adolescent

Effect of orthotopic liver transplantation on bone mineral content and serum vitamin D metabolites in infants and children with chronic cholestasis.

Almost all infants and children with chronic cholestasis have osteopenia. We evaluated the effect of orthotopic liver transplantation on bone mineral content and serum 25(OH)-vitamin D-[25(OH)D]-of nine infants and children (five girls; age, 6 to 21 mo at the time of orthotopic liver transplantation) with end-stage liver disease resulting from chronic cholestasis. We hypothesized that after orthotopic liver transplantation, decreased bone mineral content will recover and the serum 25(OH)D level will either normalize or remain normal in those who were previously vitamin D deficient or sufficient, respectively. All had subnormal bone mineral content before transplant. On long-term follow-up (> 4 mo) of seven patients, bone mineral content normalized in all between 6.5 and 19 mo after transplant, with a mean of 11.2 +/- 4.5 mo. In six patients with normal serum 25(OH)D levels before orthotopic liver transplantation, the serum 25(OH)D levels had declined markedly 1 to 2 mo after transplant, followed by return to normal by 3 to 6 mo. Low serum 25(OH)D levels (< 15 ng/ml) in three patients before orthotopic liver transplantation normalized after transplant. Although there was no correlation between bone mineral content and serum 25(OH)D level before transplant, sustained normal serum 25(OH)D and 1,25(OH)2D levels preceded or accompanied normalization of bone mineral content in the seven patients available for long-term follow-up. We conclude that (a) in infants and children younger than 2 yr with chronic cholestasis, bone mineral content normalizes approximately 11 mo after orthotopic liver transplantation. This normalization is preceded by a sustained period of normal serum 25(OH)D levels.(ABSTRACT TRUNCATED AT 250 WORDS)

Bone Density

Extracorporeal membrane oxygenation in the management of cardiac failure secondary to myocarditis.

While most patients with viral myocarditis have a relatively uncomplicated clinical course, a small number of patients will present with cardiogenic shock unresponsive to standard medical therapy. We describe the clinical course of three patients who developed profound cardiac failure secondary to a documented viral myocarditis. Each patient was managed using venoarterial (VA) extracorporeal membrane oxygenation (ECMO) support using the right common carotid artery/internal jugular vein for cannulation. While undergoing ECMO support, each patient developed elevated left-sided cardiac chamber pressures with resultant pulmonary edema. This was managed by balloon atrial septostomy in two cases and combined blade/balloon atrial septostomy in one case. Excellent decompression of the left heart was achieved in each patient. Two patients were successfully weaned from ECMO and are currently alive, with one demonstrating residual cardiac dysfunction. One patient developed global myocardial necrosis and ultimately died. This small series demonstrates a role for ECMO in the management of cardiac failure due to acute viral myocarditis unresponsive to medical therapy. Our experience also suggests that balloon atrial septostomy may be useful to decompress the left atrium and ventricle of patients with acute myocarditis while on ECMO.

Acute Disease

The predictive value of head ultrasound in the ECMO candidate.

Cranial ultrasound (US) examination is the screening technique of choice for assessing preexisting neurological damage in potential neonatal extracorporeal membrane oxygenation (ECMO) candidates. Currently, US evidence of intracranial hemorrhage greater than grade I in severity is a contraindication to ECMO at this ECMO center. In the current study, radiological findings were reviewed in 129 consecutive neonatal ECMO cases in an attempt to identify which pre-ECMO US findings were associated with the development of subsequent intracranial complications while on ECMO. Pre-ECMO head US, post-ECMO head US, and head computed tomography (CT) scans were reviewed retrospectively by one radiology team. Ventricular, parenchymal, and extraaxial fluid abnormalities were recorded for each case. Pre-ECMO US findings were then correlated with the subsequent development of significant intracranial radiological abnormalities noted on post-ECMO studies as well as with clinical data regarding ECMO course and outcome. Results showed that infants with evidence of severe edema or periventricular leukomalacia on pre-ECMO imaging had a 63% incidence of subsequent major intracranial complications. This represents a significantly higher risk than in candidates with a normal examination or evidence of grade I intracranial hemorrhage, subependymal cysts, or mild edema. These results suggest that infants with sonographic evidence of ischemic or anoxic damage on pre-ECMO US are at high risk for the development of significant intracranial complications if ECMO therapy is instituted.

Brain

A prospective, multicenter, randomized study of high versus low positive end-expiratory pressure during extracorporeal membrane oxygenation.

To test the hypothesis that increased positive end-expiratory pressure (PEEP) could prevent deterioration of pulmonary function and lead to more rapid recovery of lung function, we randomly assigned 74 patients undergoing extracorporeal membrane oxygenation (ECMO) at four centers to receive either high (12 to 14 cm H2O) or low (3 to 5 cm H2O) PEEP. The two groups were similar in terms of weight, gestational age, diagnosis, and pre-ECMO course. All other aspects of care were identical. Dynamic lung compliance was measured at baseline and every 12 hours. Radiographs of the chest were obtained daily. Survival rates were similar in the two groups: 36 of 40 for low PEEP and 34 of 34 for high PEEP. The duration of ECMO therapy was 97.4 +/- 36.3 hours in the high-PEEP group and 131.8 +/- 54.5 hours in the low-PEEP group (p less than 0.01). Dynamic lung compliance throughout the first 72 hours of ECMO was significantly higher in patients receiving high PEEP. Radiographic appearance of the lungs correlated well with lung compliance: patients receiving high PEEP had significant deterioration of the radiographic score less frequently than those receiving low PEEP. High PEEP also was associated with significantly fewer complications. We conclude that PEEP of 12 to 14 cm H2O safely prevents deterioration of pulmonary function during ECMO and results in more rapid lung recovery than traditional lung management with low PEEP.

Extracorporeal Membrane Oxygenation

Hepatobiliary scintigraphy in pediatric liver transplant recipients.

The clinical usefulness of hepatobiliary scintigraphy was evaluated in pediatric liver transplant recipients. One hundred fifteen hepatobiliary scintigraphic studies were performed in 30 patients who received 22 whole liver and 16 segmental grafts. Parameters that were useful in predicting an adverse outcome were failure to visualize excreted radiopharmaceutical at or beyond the biliary anastomosis on a study performed within 24 hours after transplant, and persistent or increasing delay in the time of visualization of excreted radiopharmaceutical. Abnormalities of liver uptake and excretion were seen in rejection, but they were also seen in patients who remained well without rejection or parenchymal disease. Significant biliary leaks were identified in the three cases in which they were known to be present. In liver transplant recipients, hepatobiliary imaging is useful in predicting graft survival and identifying biliary leaks.

Adolescent

Monoethylglycinexylide formation in assessing pediatric donor liver function.

Lidocaine metabolism to monoethylglycinexylide (MEGX) has been described as a novel method to assess liver function in adult transplant donors and recipients. While this assay appears to offer a number of advantages over existing liver function tests, limited work has been done to evaluate its potential in the pediatric population. This study evaluated MEGX formation in potential pediatric liver donors (n = 35) and a control group of children (n = 16). The mean MEGX formation was significantly higher in pediatric donors than in the control group (156 +/- 62 vs 106 +/- 33 ng/ml, p < 0.05). No correlation with age, total bilirubin, liver transaminases, or alkaline phosphatase could be made within each group. Significant differences in MEGX levels were noted when each group was compared to its adult counterpart. Both pediatric donors and controls had greater mean MEGX formation than has been reported for adult donors and controls (156 +/- 62 vs 127 +/- 61 ng/ml, p < 0.05 and 106 +/- 33 vs 72 +/- 36 ng/ml, p < 0.05, respectively). Drugs that alter lidocaine pharmacokinetics and their potential influence on MEGX formation were evaluated in the pediatric donor group. Donors exposed to hepatic enzyme-inducing drugs had a higher mean MEGX formation (187 +/- 60 vs 146 +/- 63 ng/ml). No significant differences were noted between donors receiving and not receiving vasopressors. In conclusion, the significant differences between pediatric and adult MEGX formation should be noted when establishing reference or normal ranges for this diagnostic test. Furthermore, concomitant drug therapy may significantly alter MEGX formation.

Adult

Left-lobe hepatic transplants: spectrum of normal imaging findings.

The limited availability of suitable size-matched donor whole organs has been a major cause of morbidity and mortality in infants and children requiring liver transplantation. Techniques for transplanting segments of the liver have successfully addressed this problem. The surgical anatomy of the left hepatic lobe transplant is unique in three primary respects: the cut edge, the presence of an enteric Roux loop for biliary drainage, and the alteration in the position and number of hepatic vessels. The spectrum of normal imaging findings in 17 left-lobe allografts is illustrated.

Anastomosis, Surgical