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Biomedical subjects

K Heiss

Publications and source records attributed to K Heiss.

7 recordsLinked to original sources

MitoP2: the mitochondrial proteome database--now including mouse data.

The MitoP2 database (http://www.mitop.de) integrates information on mitochondrial proteins, their molecular functions and associated diseases. The central database features are manually annotated reference proteins localized or functionally associated with mitochondria supplied for yeast, human and mouse. MitoP2 enables (i) the identification of putative orthologous proteins between these species to study evolutionarily conserved functions and pathways; (ii) the integration of data from systematic genome-wide studies such as proteomics and deletion phenotype screening; (iii) the prediction of novel mitochondrial proteins using data integration and the assignment of evidence scores; and (iv) systematic searches that aim to find the genes that underlie common and rare mitochondrial diseases. The data and analysis files are referenced to data sources in PubMed and other online databases and can be easily downloaded. MitoP2 users can explore the relationship between mitochondrial dysfunctions and disease and utilize this information to conduct systems biology approaches on mitochondria.

Animals↗

Selective distal splenorenal shunts for intractable variceal bleeding in pediatric portal hypertension.

The treatment of portal hypertension in the pediatric population has undergone an evolution toward less invasive methods of care. With the advent of endoscopic sclerotherapy, surgery is less common in the acute care of these patients. Few reports deal with the role of portosystemic shunting in the emergent management of variceal hemorrhage in children. To address this issue, the authors studied the medical records of all pediatric patients at their institution who underwent placement of a shunt for portal hypertension during the last 10 years. Nine patients underwent a total of 10 emergent or semiurgent shunting procedures. Seven were boys and two were girls. Six patients had portal hypertension as a result of intrahepatic disease. Two had extrahepatic portal vein thrombosis. Five children had abnormal hepatic function. The median age at the time of the procedure was 9 years. The indication for surgical shunting in all cases was gastrointestinal hemorrhage not responsive to sclerotherapy. Eight patients underwent emergent distal splenorenal shunts (DSRS), and two underwent a nonselective mesocaval shunt, with one undergoing both. Postoperatively all patients had cessation of bleeding. Operative mortality was zero. Early complications included ascites (3), small bowel obstruction (1), and hepatorenal syndrome (1). The child who underwent a nonselective shunt procedure had encephalopathy. Two DSRS thrombosed, requiring reexploration; eight shunts remained patent. Three patients eventually had orthotopic liver transplantation (OLT) because of progressive hepatic failure. Two children died; neither death was shunt related.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Thrombosis of the portal venous system after splenectomy for pediatric hematologic disease.

Splenic, portal, or mesenteric venous thrombosis after splenectomy for hematologic disease has not been reported in the pediatric literature. It is a rare complication associated with significant morbidity and mortality in adult reports. Between 1981 and 1991, 3 patients (13-year-old boy with hereditary elliptocytosis [HE], 13-year-old boy with thalassemia intermedia [TI], and 18-year-old girl with idiopathic thrombocytopenic purpura [ITP]) presented with abdominal pain, nausea, with or without fever, at 4, 11, and 13 days postsplenectomy, respectively. Abdominal Doppler ultrasound (US) and/or computed tomography (CT) showed: (1) an intraluminal filling defect with partial obstruction to flow in the right branch of the portal vein with the remaining vessels patent (HE); (2) splenic vein thrombosis with complete occlusion of the main portal vein and proximal superior mesenteric vein (TI); and (3) complete thrombosis of the splenic vein, proximal superior mesenteric vein and portal vein (including central radicles), with retrogastric collateralization (ITP). Subsequent imaging showed either complete resolution of vascular obstruction on no treatment (patient 1), or portal venous cavernomatous transformation with hepatofugal flow after 6 months of systemic anticoagulation (patients 2 and 3), and all 3 patients are currently asymptomatic. Postoperative sonographic evaluation of a consecutive series of pediatric splenectomies for hematologic disease (n = 16), was performed at a median of 51 days (range, 3 to 124). This demonstrated one case of asymptomatic left portal venous thrombosis with subsequent recanalization in the absence of treatment.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Pure esophageal atresia: outlook in the 1990s.

Eleven newborns with pure esophageal atresia were treated between 1980 and 1989 inclusive; there were six girls and five boys. Their gestational age ranged from 31 to 40 weeks (average, 37 weeks) and weight from 1.1 to 3.0 kg (average, 2.2). The only associated anomalies were Down's syndrome, respiratory distress syndrome, and patent ductus arteriosus. All babies received an immediate gastrostomy. Several radiologic studies were done to see if the distance between the two esophageal pouches was decreasing. Dilatations of the upper pouch were carried out in two patients. After a wait of 1 to 7 months (average, 3 1/2) a primary anastomosis was attempted; the weight of six babies doubled during this time. Eight neonates had a primary repair (two were aided by a circular myotomy). Two had a staged gastric tube constructed, and one baby had a gastric pull-up procedure. Three of the infants with a primary anastomosis required a subsequent antireflux operation, and one needed her anastomosis resected 16 months later. Ten of these 11 newborns are alive and well; one of the gastric tube children died from an adhesive small bowel obstruction at age four years.(ABSTRACT TRUNCATED AT 250 WORDS)

Anastomosis, Surgical↗

Respiratory failure due to retained esophagus: a complication of esophageal replacement.

Recurrent fistulas occur in about 10% of infants treated for esophageal atresia with distal tracheoesophageal fistula. Failed repair of a recurrent fistula rarely requires esophageal replacement and removal or diversion of the native esophagus. We present a patient who underwent multiple operations for recurrent tracheosophageal fistula whose native esophagus was eventually replaced with a colonic interposition graft. Over the subsequent 9 years he experienced failure to thrive, respiratory distress, and repeated pulmonary infections attributed to chronic aspiration. Eventually, he developed respiratory failure and required endotracheal intubation and mechanical ventilation. He became increasingly difficult to ventilate and, in spite of aggressive efforts, suffered a cardiac arrest from which he could not be resuscitated. At postmortem, a dilated blind segment of native esophagus, which was compressing and obstructing the malacic trachea, was found in the posterior mediastinum. Death was caused by massive air embolus, which was in turn attributed to the high airway pressures needed to ventilate the patient. Tracheal compression by a remnant of native esophagus should be considered in the differential diagnosis of respiratory failure after esophageal replacement.

Child↗

Reversal of mortality for congenital diaphragmatic hernia with ECMO.

Extracorporeal Membrane Oxygenation (ECMO) has been available to neonates with respiratory failure at the University of Michigan School of Medicine since June 1981. In order to evaluate the impact of this type of pulmonary support, a retrospective analysis of 50 neonates with posterolateral congenital diaphragmatic hernia (CDH) who were symptomatic during the first hour of life and were treated between June 1974 and December 1987 was carried out. The patients were divided into two groups, those treated before June 1981 (16 patients) and those treated after June 1981 (34 patients). Overall survival improved from 50% (eight of 16 patients) during the pre-ECMO era to 76% (26 of 34 patients) during the post-ECMO period (p = 0.06). During the period after June 1981, 21 neonates were unresponsive to conventional therapy and were therefore considered for ECMO. Failure of conventional therapy was defined as acute clinical deterioration with an expected mortality of greater than 80% based on an objective formula previously reported. Six patients were excluded on the basis of specific contraindications to ECMO. Thirteen of 15 infants (87%) supported with ECMO survived. Three patients treated before 1981 met criteria for ECMO; all three died while receiving treatment using conventional therapy. These survival differences are significant (p less than 0.01). In addition, the survival of 87% for the infants treated with ECMO versus the expected mortality of greater than 80% for these same patients when treated with conventional therapy is highly significant (p less than 0.005). Based on this data, ECMO appears to be a successful, reliable, and safe method of respiratory support for selected, critically ill infants with CDH.

Evaluation Studies as Topic↗

Measuring infant metabolism: design and testing of a miniature gas exchange monitor.

A compact closed-circuit gas exchange monitor (GEM) was built for measurement of oxygen consumption (VO2) in ventilated infants. The GEM includes a ventilator-driven slave bellows, a CO2 scrubber, one-way valves to ensure unidirectional flow, and tubing to complete the small-volume low-compliance system, which fits easily between the ventilator (VENT) and the endotracheal tube (ETT). Oxygen consumption is measured by volume loss from a spirometer attached by a one-way valve. Pressure is monitored at the airway, and the VENT is adjusted to attain the desired pressure pattern. The system was leak tested by placing a 3-kg weight on the spirometer bell (continuous positive airway pressure = 20 cm H2O) and then ventilating with peak inspiratory pressures (PIP) of 60 cm H2O without leak. Bench testing for accuracy of volume loss was checked by ventilating the device into another calibrated spirometer and achieving equal volumes. First, four rabbits were studied to determine the range of ventilator backup rates (BUR = 0 to 60), inspiratory times (IT = .2 to .6 seconds), and airway pressures (up to 40/8 cm H2O) attainable by this device. Then six fasted rabbits weighing 2.2 to 4.0 kg were anesthetized with a ketamine-rompun mixture, underwent tracheostomy, and were placed on a pressure VENT. The BUR was set at 20/min and the IT at .5 seconds. The GEM was placed between the VENT and the ETT, and the PIP was adjusted to maintain PaCO2 between 30 and 40 torr, eliminating spontaneous respiration. Oxygen consumption was measured at five-minute intervals for one hour.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗