Biomedical subjects
Publications and source records attributed to T E Lobe.
Two patients with failed drainage procedures for biliary atresia are presented in whom major hemorrhage from esophageal varices followed shortly after ostomy closure for recurrent stomal bleeding. Before ostomy closure is contemplated in these patients, evaluation for esophageal varices and their prophylactic obliteration with sclerotherapy is recommended.
Two patients with rhabdomyosarcoma presented clinically with dermal nodules on the face. The first patient was a 12-month-old girl with a 2 X 3-cm-diameter red nodule on her left cheek that had begun as a small red papule when she was 3 months old and had progressively increased in size. The second patient was a 19-year-old girl who presented with an enlarging mass on her right cheek that had begun as a pea-sized nodule three months previously. In both cases, biopsies revealed a rhabdomyosarcoma in the underlying soft tissue with extension into the overlying dermis. Rhabdomyosarcoma presenting as a dermal nodule is rare. It usually presents as an asymptomatic papule without distinctive clinical features and therefore may result in delayed diagnosis unless a biopsy is performed.
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Liquid perfluorocarbons are biologically inert compounds capable of dissolving up to 40 percent oxygen by volume. This remarkable and reversible oxygen solubility has encouraged investigations into therapeutic application in situations where tissue oxygen delivery is impaired. One such setting is intestinal ischemia. Identically prepared devascularized segments of rat intestine were treated with either intraluminal oxygenated perfluorocarbon (perfluorotributylamine) or physiologic saline solution. After timed sacrifice, blinded quantitative histologic evaluation for ischemic injury was performed. The perfluorotributylamine treatment groups had histologic scores indicative of less severe injury between 1 and 4 hours. These scores achieved statistical significance (p less than 0.05). We conclude that intraluminal oxygenated perfluorocarbons have a significant protective effect in this model of intestinal ischemia. This quantitative analysis is unique and is an important aspect of the preclinical evaluation of the perfluorocarbon preparations.
The benefits of dopamine resuscitation in neonatal shock are in doubt. To evaluate dopamine's effectiveness on regional perfusion and survival, neonatal pigs were subjected to fecal Escherichia coli peritonitis-induced septic shock and were randomly divided into equal groups. Control animals (Group I) were not resuscitated with dopamine. Dopamine resuscitation, as a continuous infusion of 5 micrograms/kg/min (Group II), 50 micrograms/kg/min (Group III), or 200 micrograms/kg/min (Group IV), began when cardiac output dropped 20% below baseline. Radiolabeled microspheres were used to assess regional perfusion. Survival was defined as no hemodynamic compromise at 24 hr. Increased survival time correlated with sustained increases in cardiac output and blood pressure in Group III, which were not observed in the other groups. Significant changes in multiorgan regional perfusion were also observed in Group III and correlate with the improved survival. These data suggest that higher than usual doses of dopamine may be beneficial in neonates with peritonitis-induced septic shock.
To evaluate the changing patterns of complications of surgery for intersex, the records of 58 patients operated on for ambiguous genitalia over the last two decades were reviewed. Patients with 21-hydroxylase deficiency constitute the largest group. These patients required the fewest operations and had the fewest complications. Surgery for all other diagnoses was accompanied by a high incidence of multistaged procedures and a high complication rate. The operations associated with the highest complication rate were those for vaginal reconstruction and severe hypospadias. Stenosis of the vagina and urinary tract problems accounted for most of the problems. Before 1975, the management of intersex was associated with a later age at diagnosis and initial operation, and a higher incidence of multistaged procedures performed by multiple surgeons from different specialties. These factors contributed to a higher complication rate than that observed after 1975, when patients were diagnosed and operated on earlier and had fewer complications.
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This report describes the pitfalls in the diagnosis of extrahepatic biliary atresia in an infant with paucity of the interlobular bile ducts.
Seven infants with congenital tracheal stenosis were evaluated and treated to assess the efficacy of current techniques of management. All had multiple congenital anomalies in addition to tracheal stenosis. Notably, three infants had imperforate anus and three had vascular rings. Each infant presented early with respiratory distress. While bronchoscopy and/or bronchography were performed in some, the diagnosis could be made from high contrast x-rays of the chest in each instance. The complexity of the tracheal lesion(s) determined the operative approach. Complicating factors included tracheomalacia, multiple stenoses, pulmonary hypoplasia, and carinal involvement. The trachea was approached through an anterolateral thoracotomy in five cases and through the neck in one. Cardiopulmonary bypass was never required. Simple resection was possible only once. Four patients received costal cartilage grafts, and one graft was created from dura. Grafts varied from 2 1/2 to 6 cm in length and extended onto the bronchi in two cases. All patients with grafts were treated with postoperative endotracheal stents and ventilatory assistance and all were extubated successfully. One patient had trisomy 16p+ detected after successful extubation and died later. One patient was treated expectantly and died. While each case must be individualized, repair of complex tracheal stenosis often results in dramatic immediate improvement and long-term success.
Two patients with nesidioblastosis, one with persistent hypoglycemia and another who was asymptomatic, underwent pancreatic resection. Dispersed pancreatic islets were prepared from each patient. Insulin secretion from cultured islet cells was increased and somatostatin decreased in the symptomatic patient compared with the asymptomatic patient. Immunocytochemistry showed increased somatostatin-containing cells in the asymptomatic patient. We hypothesize that this may be the mechanism by which some patients with nesidioblastosis maintain normal serum glucose levels.
We have used percutaneous transhepatic drainage of pyogenic hepatic abscesses with good result. This technique has been used effectively in adults by several authors. We believe this report represents the first instance of successful percutaneous drainage of a pyogenic liver abscess complicated by a bronchobiliary fistula.
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Serum hexosaminidase activity (HEX) is elevated with ischemic gut injury. To determine if subsequent decreases in HEX correlate with gut healing, 97 weanling rats were subjected to laparotomy at which alternate vascular bundles were ligated along the base of the entire anterior mesenteric artery arcade. Fifteen rats served as preoperative controls. After recovery, rats were allowed ad lib food and water. Groups were then killed at intervals, blood was drawn for HEX determination, and samples of small bowel were taken for histological evaluation. Microscopically, focal ischemic necrosis began at 6 hr. Between 12 and 48 hr, cellular changes were consistent with progressive ischemic injury. Evidence of healing was apparent beginning at 5 days and these histological changes correlated with changes in HEX. Thus HEX proves useful as a marker for gut healing in this model.
To explain the high neonatal mortality from peritonitis-induced septic shock despite current resuscitation practices, the efficacy of dopamine, naloxone, and prostacyclin was evaluated in an experimental neonatal model. Hemodynamics were monitored and survival was measured in anesthetized neonatal swine, which were subjected to fatal fecal-Escherichia coli peritonitis-induced septic shock. All the animals received fluid resuscitation, antibiotics, and bicarbonate to correct acidosis. Pharmacologic resuscitation began when cardiac output dropped below baseline in the experimental groups. Although significant differences were observed between groups in cardiac output, mean arterial and mean pulmonary arterial pressures, left ventricular stroke work, stroke volume, and pulmonary vascular resistance indices (P less than 0.02), and each animal exhibited favorable hemodynamic responses during the first several hours of dopamine and naloxone infusion, these drugs failed to prolong survival. Also, 5 of the 9 naloxone-treated pigs (56%), died with histologically proven intestinal ischemia (P less than 0.02). Thus, dopamine, naloxone, and prostacyclin (at doses commonly recommended for the treatment of septic shock) fail to positively influence the fatal course of this condition, and the use of naloxone in this model is associated with profound intestinal ischemia.
This report describes the diagnosis and management of a "giant cystic malformation of the gallbladder," in a child with multiple congenital anomalies.
Pyogenic liver abscesses are rare in previously healthy children. This review reports ten such cases of pyogenic abscess seen between 1963 and 1984. The mean age in these children was 6.3 years. One child died. Of the nine survivors, five had successful open surgical drainage. Two abscesses were drained percutaneously. Another abscess drained spontaneously into the biliary system, and one abscess was treated with antibiotics alone. The single most helpful diagnostic test was abdominal ultrasonography. Our experience is evaluated in the context of other reports of pyogenic abscesses in children.
Serum activity of hexosaminidases A and B has been measured to identify patients with Tay-Sachs and Sandhoff diseases, and may also be influenced by other clinical conditions including neonatal necrotizing enterocolitis. We have characterized the normal developmental pattern of hexosaminidase activity in serum by measuring this enzyme activity in 61 neonates of 27-40 weeks gestation who were followed from birth to age 4-8 weeks. Total serum hexosaminidase activity significantly increased with postnatal and gestational age, and with initiation of enteral feeding. We conclude that serum hexosaminidase activity in infants with clinical conditions that may influence serum activity of this enzyme must be compared to that in normal infants of similar gestational and postnatal ages.
Spontaneous extrahepatic biliary duct perforation in infancy is exceedingly rare. We report a patient in whom the diagnosis was established by ultrasonography and hepatobiliary scintigraphy.