The use of extracorporeal membrane oxygenation in critically ill neonates with Ebstein's anomaly.
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Critical Care↗
Biomedical subjects
F C Ryckman
Publications and source records attributed to F C Ryckman.
Cardiac arrest in the ECMO candidate.
Extracorporeal membrane oxygenation (ECMO) improves survival in appropriately selected full-term neonates with severe respiratory failure. The clinical course and outcome of infants placed on ECMO after sustaining a cardiac arrest is not known. This study reviews the characteristics and outcome of 10 neonates, identified by retrospective review, placed on ECMO at Children's Hospital Medical Center (CHMC), Cincinnati, OH, after sustaining a cardiac arrest. Long-term survival in this group was 60%, significantly less than the 87% overall ECMO survival in infants at CHMC (P less than .01). Survivors and nonsurvivors in the cardiac arrest group were similar with regard to gestational age, birth weight, Apgar scores, and arterial PO2 prior to cannulation. Nonsurvivors had an ECMO course complicated by progressive multisystem organ failure. Head computed tomography obtained at the time of discharge demonstrated right-sided brain lesions in three of six survivors. Despite these radiographic findings, early clinical follow-up suggests adequate growth and development with no individuals demonstrating a severe neurological deficit. Thus, ECMO can play a role in the resuscitation of neonatal ECMO candidates sustaining cardiac arrest prior to or at the time of cannulation. Early clinical follow-up suggests adequate preservation of neurological function in this extremely high-risk group.
Brain Diseases↗
Segmental orthotopic hepatic transplantation as a means to improve patient survival and diminish waiting-list mortality.
Pediatric liver transplantation continues to be limited by the availability of suitable liver donors, a factor that restricts programmatic development and ultimately contributes to death on the recipient waiting list. We report the application of segmental liver transplantation as a technique to address both these problems as well as improving the outcome of the child undergoing the transplant procedure. Since 1986, 37 children have undergone orthotopic liver transplantation. Twenty-three children have received whole-organ grafts; 81% survived. Of those receiving whole-organ grafts, 15% had arterial thrombotic complications and 23% required retransplantation. More importantly, 29% of those children listed for transplantation died while waiting for a donor organ to become available, with a mean interval of 1.7 months (range, 2 days to 4.5 months). Since July 1988, segmental liver transplantation has been a component of our therapeutic armamentarium, and of the past 20 liver recipients, 16 have received a left lobe segmental graft. The results of the segmental transplant series have shown striking improvements. First, no child has died while awaiting donor organ availability. Second, segmental liver recipient survival is equivalent to that of whole-organ graft recipients (81%). Third, hepatic arterial thrombosis, especially a problem in high-risk infant transplants, was reduced by this technique (5%). Retransplantation due to graft complications has not increased (21%). These data suggest a vital role for segmental liver transplantation not only as a remedial salvage procedure for the critically ill child, but also as a primary transplant option.
Adolescent↗
Changes in cardiac function during extracorporeal membrane oxygenation for persistent pulmonary hypertension in the newborn infant.
The effects of extracorporeal membrane oxygenation (ECMO) on cardiac function and its determinants (preload, afterload, contractility, and heart rate) are largely unknown, although some evidence exists that function may decrease. To determine whether cardiac function decreases and what changes in the determinants take place during and after ECMO, we observed 26 newborn infants with persistent pulmonary hypertension. Serial echocardiograms were performed before ECMO, during maximum cardiopulmonary bypass, and after ECMO. Cardiac function was assessed by using standard echographic ejection phase indices (shortening fraction and cardiac output). Heart rate, preload (left ventricular end-diastolic dimension and area), afterload (left ventricular end-systolic wall stress), and contractility (relationship between velocity of circumferential fiber shortening and wall stress) were also measured. Ejection phase indices significantly decreased during ECMO (shortening fraction 33% to 25%, cardiac output 205 to 113 ml/kg/min; p less than 0.05) and returned to normal after ECMO (shortening fraction 26% to 34%, cardiac output 107 to 240 ml/kg/per minute; p less than 0.05). Heart rate also significantly decreased during ECMO (158 to 118 beats/min; p less than 0.05). Preload significantly increased after ECMO (left ventricular end-diastolic dimension 1.4 to 1.6 cm, left ventricular end-diastolic area 1.9 to 2.2 cm2; p less than 0.05). There were no significant changes in contractility and afterload during any study period. We conclude that, although left ventricular ejection phase indices and heart rate decreased during ECMO, these changes were transient and resolved when bypass was terminated. Contractility and afterload did not appear affected by bypass.
Bacterial Infections↗
Liver transplantation in the pediatric population: indications and monitoring.
Advances in immunosuppressive therapy and surgical technique have brought liver transplantation to an effective therapeutic option for children with end-stage liver disease. The indications and contraindications for transplantation, recipient selection, ethical and financial considerations, and quality of life after transplantation are outlined in this review.
Child↗
Multiple endocrine neoplasia type II B with symptoms suggesting Hirschsprung's disease: a case report.
A 3-year-old child was referred with a tentative diagnosis of Hirschsprung's disease because of life-long constipation and "megacolon" demonstrated radiographically. Our rectal biopsy revealed hyperganglionosis suggestive of multiple endocrine neoplasia (MEN) type II B. This, in addition to an elevated serum calcitonin level, prompted surgical removal of her thyroid, which appeared grossly normal but on sectioning, contained a medullary carcinoma in each lobe. She remains disease-free 5 years later. Gastrointestinal symptoms are a significant component of the MEN type II B syndrome, and often antedate the full phenotypic expression of the syndrome and the development of potentially lethal endocrine neoplasms. On the basis of this experience, it is recommended that MEN II B be included in the differential diagnosis of chronic constipation.
Child, Preschool↗
Antimurine antibody formation following OKT3 therapy.
OKT3 is an IgG2a murine monoclonal antibody directed against the CD3 antigen receptor of human T lymphocytes. A major concern with OKT3 treatment in solid organ transplant recipients is the development of antimouse antibody, which may preclude retreatment with this agent. We have administered OKT3 on 215 occasions (150 renal, 34 hepatic, 26 cardiac, 5 pancreatic) in 179 patients between April 1982 and December 1988. The mean duration of treatment was 10.5 days (range, 2-22 days). Antimouse antibody data were analyzed on the most recent 133 treatment courses where the antibody status was available pretreatment. Determination of antimouse antibody production was elicited by ELISA technology at days 0, 7, 14, and 28 of OKT3 treatment. Patients were categorized according to the antibody response as follows: (a) absence of antibody; (b) low titer (1:100); or (c) high titer (greater than or equal to 1:1000). Our earlier experience has demonstrated that retreatment with OKT3 is successful in groups a and b. The development of antimurine antibodies was analyzed with regard to the following parameters: (1) The duration of OKT3 treatment; (2) treatment type (prophylactic, primary, or secondary); (3) primary treatment or retreatment; (4) concomitant immunosuppressive regimen (double or triple therapy); (5) dosage of concomitant immunosuppressive drugs; and (6) transplant organ type. The following results were obtained. (1) Duration of treatment had no effect on antibody production (11.0 days in antibody negative and 10.0 days in antibody positive). (2) There was no difference in antibody formation rates for the first treatment of OKT3 when it was used as prophylaxis (26%), primary (19%), or secondary (27%) therapy. (3) Antibody formation rate with first treatment was 29%; with retreatment, patients who were antibody negative following first treatment became positive in 28% of cases, and retreated patients who were low titer positive following first treatment converted to high titer in 57% of cases. (4) Antibody formation was higher in patients receiving double immunosuppressive therapy (36%) than in those receiving triple immunosuppressive therapy (21%) during OKT3 treatment. (5) Concomitant immunosuppression was lower in the antibody-positive group during OKT3 therapy: steroids, 61 mg/day vs. 52 mg/day; azathioprine, 89 mg/day vs. 66 mg/day; CsA, 317 mg/day vs. 186 mg/day. (6) Antibody formation rates were lower in non-renal transplants following first treatment with OKT3 (liver 17%, heart 17%, kidney 28%); this reflects the higher doses of concomitant immunosuppressive therapy used in nonrenal transplants.(ABSTRACT TRUNCATED AT 400 WORDS)
Animals↗
Continuous hemodiafiltration in children.
Continuous arteriovenous hemofiltration is a form of renal replacement therapy whereby small molecular weight solutes and water are removed from the blood via convection, alleviating fluid overload and, to a degree, azotemia. It has been used in many adults and several children. However, in patients with multisystem organ dysfunction and acute renal failure, continuous arteriovenous hemofiltration alone may not be sufficient for control of azotemia; intermittent hemodialysis or peritoneal dialysis may be undesirable in such unstable patients. Recently, the technique of continuous arteriovenous hemodiafiltration has been used in many severely ill adults. We have used continuous arteriovenous hemodiafiltration in four patients at Children's Hospital Medical Center. Patient 1 suffered perinatal asphyxia and oliguria while on extracorporeal membrane oxygenation. Patients 2 and 4 both had Burkitt lymphoma and tumor lysis syndrome. Patient 3 had septic shock several months after a bone marrow transplant. All had acute renal failure and contraindications to hemodialysis or peritoneal dialysis. A blood pump was used in three of the four patients, while spontaneous arterial flow was adequate in one. Continuous arteriovenous hemodiafiltration was performed for varying lengths of time, from 11 hours to 7 days. No patient had worsening of cardiovascular status or required increased pressor support during continuous arteriovenous hemodiafiltration. The two survivors (patients 2 and 4) eventually recovered normal renal function. Continuous arteriovenous hemodiafiltration is a safe and effective means of renal replacement therapy in the critically ill child. It may be ideal for control of the metabolic and electrolyte abnormalities of the tumor lysis syndrome.
Acute Kidney Injury↗
Taurocholate transport by basolateral plasma membrane vesicles isolated from human liver.
Transport of taurocholate into the hepatocyte against unfavorable chemical and electrical gradients occurs via a sodium-dependent, carrier-mediated transport system. Although this cotransporter has been characterized in the rodent, it has not been demonstrated in man. Therefore, we utilized human liver, obtained via multiorgan donation but not used for transplantation, to prepare basolateral (sinusoidal) liver plasma membrane vesicles by a Percoll gradient method. Na+,K+-ATPase, a marker enzyme for the basolateral domain, was enriched 28.9-fold in the final membrane fraction compared with homogenate, whereas the bile canalicular membrane enzymes Mg++-ATPase and alkaline phosphatase were enriched only 3.4- and 6.4-fold, respectively. Marker enzyme activities for endoplasmic reticulum, lysosomes and mitochondria were not enriched compared with homogenate. Integrity of the membrane vesicles was confirmed by the demonstration of Na+-dependent concentrative uptake of the amino acid L-alanine (estimated intravesicular volume of 0.59 microliter per mg protein). An inwardly directed 100 mM Na+ gradient stimulated the initial rate of 2.5 microM taurocholate uptake and energized a transient 2-fold accumulation of the bile acid above equilibrium ("overshoot"). In contrast, uptake was slower and no overshoot occurred with a K+ gradient. A negative intravesicular potential, created by altering accompanying anions or by valinomycin-induced K+ diffusion potentials, did not enhance taurocholate uptake, suggesting an electroneutral cotransport mechanism. Chloride as the accompanying anion stimulated the initial rate of uptake compared with anions of lesser or greater lipid permeability. Na+-dependent taurocholate (4 microM) uptake was significantly inhibited by 250 microM cholate, taurocholate, glycocholate, taurochenodeoxycholate and bromsulfophthalein.(ABSTRACT TRUNCATED AT 250 WORDS)
Adult↗
Mediastinal hemorrhage during extracorporeal membrane oxygenation.
A case of mediastinal hemorrhage along with hemorrhage into a pneumatocele while on extracorporeal membrane oxygenation (ECMO) is presented. Computerized tomography of the chest was utilized to support the diagnosis. Barotrauma to the lungs best explains the inciting event that allowed the hemorrhage to occur once the patient was heparinized for ECMO. This complication serves to point out the importance of commencing early ECMO support before widespread pulmonary and mediastinal barotrauma develops.
Barotrauma↗
Factors affecting urea clearance during continuous hemodiafiltration in the canine model.
Continuous hemofiltration (CH) for the treatment of hypervolemia and electrolyte abnormalities in critically ill patients with acute renal failure has been shown to be an effective mode of therapy. This technique offers several advantages over peritoneal dialysis or hemodialysis: it is technically less complex, provides efficient ultrafiltration, and produces fewer hemodynamic disturbances. Recently, continuous hemodiafiltration (CHD) using a flow of dialysate fluid into the ultrafiltration chamber has been reported to augment urea clearance. The purpose of this study was to determine the blood flow and dialysate flow characteristics for optimal clearance of urea and creatinine using this technique. Six mongrel dogs (mean weight, 8.0 +/- 1.1 kg) underwent bilateral nephrectomy to induce anuric renal failure. Postoperatively, the animals were fluid resuscitated and fed ad libitum. Twenty-four hours following nephrectomy, venovenous hemofiltration was instituted. Blood flow was regulated via a roller pump, while dialysate flow was regulated using an infusion controller. An Amicon-30S hemofilter was used in the circuit. Blood flow rates of 5, 10, 15, 20, 25, and 30 mL/kg/min were used. Hemodiafiltration using Dianeal 1.5% solution was used in each animal. Net fluid losses via ultrafiltration were replaced using lactated Ringer's solution. Three of six animals survived for the complete five-hour hemofiltration period. No marked disturbances in electrolyte serum concentrations, including hyperkalemia, were observed. BUN concentrations were reduced by 35% and creatinine by 26%. Variation of the dialysate flow rate did not influence clearance of either urea or creatinine. Instead, clearance appeared to be flow dependent, and it was markedly increased at flow rates greater than 15 mL/kg/min.(ABSTRACT TRUNCATED AT 250 WORDS)
Acute Kidney Injury↗
Color flow mapping to document normal pulmonary venous return in neonates with persistent pulmonary hypertension being considered for extracorporeal membrane oxygenation.
This study investigated the value of color flow mapping in documenting normal pulmonary venous return in neonates with persistent pulmonary hypertension who were candidates for extracorporeal membrane oxygenation (ECMO). Forty newborn infants with persistent pulmonary hypertension underwent conventional (two-dimensional and Doppler) echocardiography and color flow mapping. Of 25 candidates for ECMO therapy, 18 subsequently received it. Conventional echocardiography demonstrated normal pulmonary venous return in only 21 of the 40 patients. In all 40, however, color flow mapping demonstrated normal right and left pulmonary venous drainage entering the left atrium. In three other patients with total anomalous pulmonary venous return, conventional echocardiography demonstrated the anomalous pulmonary venous pathways, and color flow mapping did not show jets emanating from the left atrial wall; the left atrium was shown to fill exclusively from right to left shunting through the foramen ovale. We conclude that color flow mapping is superior to conventional echocardiography for verifying normal pulmonary venous return in neonates with persistent pulmonary hypertension.
Color↗
Brain abnormalities in infants on extracorporeal membrane oxygenation: sonographic and CT findings.
The findings of cranial sonography performed before and during extracorporeal membrane oxygenation (ECMO) in 50 near-term infants and CT findings after ECMO in 18 of those patients are reported. Hemorrhage is uncommon in patients being considered for ECMO: subependymal hemorrhage was seen in one case and subependymal cyst possibly due to in utero hemorrhage was seen in five cases. Hypoxic ischemic ischemic injury is more common: severe cerebral edema was seen in two cases, occipital hemorrhagic infarct in one case, and mild cerebral edema in 17 cases. During ECMO, sonograms showed that the hemorrhage in patients with small subependymal hemorrhage or cyst before ECMO did not extend while on ECMO. Typical germinal matrix/intraventricular hemorrhage was seen uncommonly (three cases). Unusual parenchymal hemorrhage did occur. Hypoxic ischemic brain injury was more common: parenchymal hemorrhage was seen in three cases, severe cerebral edema in three cases, and infarction in three cases. Hypoxic ischemic brain injury probably occurs before ECMO, with a delay in visualization. CT after ECMO detected additional abnormalities, particularly peripheral areas of hemorrhage and infarction not visible on sonograms, and is now being performed on all patients. More emphasis should be placed on better screening of infants being considered for ECMO treatment to identify irreversible anoxic brain injury, not just hemorrhage.
Brain Diseases↗
Lidocaine metabolism as an index of liver function in hepatic transplant donors and recipients.
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Biopsy↗
Use of continuous arteriovenous hemofiltration (CAVH) for renal failure in a neonate.
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Acute Kidney Injury↗
Childhood tracheobronchial mucoepidermoid carcinoma: a case report and review of the literature.
Mucoepidermoid carcinoma of the lung presenting in childhood is an uncommon neoplasm. Symptoms of progressive bronchial obstruction proceed the recognition of this lesion. The well-confined local growth and minimal metastatic potential of childhood mucoepidermoid carcinoma make local resection with maximum conservation of pulmonary parenchyma the recommended operative treatment. Adequate surgical resection precludes the need for further radiation therapy or chemotherapy. This report details the presentation and treatment of a child with this rare tumor and reviews the present literature experience with childhood mucoepidermoid carcinoma.
Bronchial Neoplasms↗
Clinical aspects of abdominal masses in children.
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Abdomen↗
Neonatal cholestatic conditions requiring surgical reconstruction.
Obstructive jaundice in infancy and childhood can arise from a variety of causes. Recognition of the importance of persistent jaundice beyond 2 weeks of age in the infant or elevation of the conjugated bilirubin more than 2 mg/dl will allow the timely evaluation of neonates with possible biliary atresia prior to the development of irreversible hepatic damage. Episodes of intermittent jaundice or persistent conjugated hyperbilirubinemia should be investigated at any age to exclude obstructive lesions of the extrahepatic biliary tract. In all cases, a rapid but carefully coordinated evaluation can be undertaken by a pediatric gastroenterology and surgical team, allowing optimum economy of effort and preservation of hepatic function. In nearly all cases with obstructive lesions of the extrahepatic biliary tree, satisfactory hepatic function can be preserved through appropriate surgical intervention. Results with biliary atresia continue to improve but are dependent on early diagnosis and treatment. In cases in which hepatic function deteriorates and hepatic failure becomes inevitable, orthotopic hepatic transplantation can be lifesaving and should be undertaken prior to the onset of irreversible hepatic decompensation. Although the terminology may be somewhat confusing and its etiology uncertain, the entity of choledochal cysts, taken within the context of its modern classification, accurately describes a group of cystic anomalies of the extrahepatic (and intrahepatic) biliary tree, which have many common features. The vast majority can be expeditiously dealt with by surgical excision and hepaticoenteral reconstruction. Two particular associations with choledochal cysts are important to note. When it occurs with infancy, choledochal cysts can be difficult to diagnose, but very important to define and treat expeditiously in order to prevent serious impairment of hepatic function. In all patients, but particularly in adults, the risk of associated carcinoma necessitates extremely careful definition of the biliary tree and urges one to advocate complete excision of the cyst at initial operation.
Bile Ducts↗