Early experience with FK 506 induction immunosuppression--suggestion for using oral FK 506.
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Administration, Oral↗
Biomedical subjects
W Berquist
Publications and source records attributed to W Berquist.
Liver transplantation in infants weighing less than 10 kilograms.
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ABO Blood-Group System↗
Operative treatment for the gastroesophageal reflux syndrome in children.
Surgical treatment for symptomatic gastroesophageal reflux (GER) was performed on 420 infants and children over a 19-year period. Esophageal motility disorders were present in more than 35% of patients and delayed gastric emptying (DGE) was present in approximately 50% of patients studied. Gastroesophageal fundoplication (GEF) alone was performed for 357 patients, whereas 51 patients underwent GEF and pyloroplasty; 12 patients underwent pyloroplasty alone. When there is more than 60% gastric retention of technetium-99m sulphur colloid in semisolid feedings at 90 minutes, pyloroplasty appears to be useful. Twenty-two percent of the last 275 refluxing children underwent pyloroplasty combined with GEF. Most infants and young children undergoing evaluation for severe GER, particularly those with neurologic disorders, may benefit from having a gastric emptying study. Children with severe reflux and esophageal dysmotility should have a loose GEF performed. The absence of persistent dumping, and the very low incidence of complications suggests that pyloroplasty should be used more frequently when significant DGE is present.
Adolescent↗
Budd-Chiari syndrome: treatment with percutaneous transhepatic recanalization and dilation.
The authors describe a child with Budd-Chiari syndrome secondary to membranous obstruction of the hepatic veins and a web in the inferior vena cava. Transhepatic catheter venography demonstrated the occlusion, which was recanalized and dilated percutaneously via a transhepatic approach. Hepatomegaly and ascites decreased promptly and the prothrombin time became normal.
Angioplasty, Balloon↗
Gastric emptying of mixed solid-liquid meal in patients with intestinal pseudoobstruction.
Gastric emptying of solids and liquids was evaluated simultaneously in 11 patients with intestinal pseudoobstruction. Despite a normal upper gastrointestinal series in most patients, over two thirds of the patients had abnormal gastric emptying of solids and/or liquids. Half these patients exhibited delayed emptying of solids and accelerated emptying of liquids. Alterations in motility patterns were observed in the four patients tested including decreased frequency or absence of migrating motor complexes originating in the stomach, shortening or absence of phase I, and loss of distinct fasted and fed patterns. Both basal values and mean postprandial increments in serum gastrin concentrations were lower in the patients, whereas no significant difference was found for pancreatic polypeptide concentrations when compared to normals. Our findings emphasize the functional heterogeneity of patients with intestinal pseudoobstruction. In addition, in contrast to previous reports which used barium meals to access gastric function, we observed a high prevalence of gastric emptying disturbances in these patients, suggesting that the motility disturbance is not restricted to the small intestine.
Adult↗
Advances in ulcerative colitis.
Ulcerative colitis is one of the two common chronic inflammatory bowel diseases which affect the colon of children. The disease can occur at any time during infancy and childhood and is far commoner than Crohn's disease of the colon in children less than 6 years old. The Jewish population outside of Israel is at far greater risk of developing the condition than any other ethnic group. The reason for this is unknown. The chances of a family member developing the condition is 2-3 times as great as in the general population. The etiology of the condition remains unknown; however, recent advances in the understanding of the immune mechanisms in the bowel and circulation indicate there are major immunological differences between ulcerative colitis and Crohn's disease. Intestinal B cells secrete enormously increased amounts of IgG1 and a lesser increase in IgG3 in ulcerative colitis whereas in Crohn's disease, all IgG subclasses are increased, but especially IgG2. Failure of the gut immune system to control antigen crossing the colonic mucosa may be the basis for the condition. The disease is classified as moderate to severe two thirds of children as opposed to less than one third of adults. Diagnostic testing must include 3 stool cultures negative for bacterial and viral pathogens, 3 stools negative for amebiasis, trichuriasis and other intestinal parasites and absence of clostridium difficile and its toxin in the stool. Flexible proctosigmoidoscopy and/or colonoscopy should be done in every case with biopsies. Barium enema is contraindicated in the severely ill patient. Major improvements in medical treatment being tested involve the development of nonabsorbable corticosteroid enemas and sulfapyridene-free forms of salicylazosulfapyridene for use in enema and oral form. Surgery for ulcerative colitis has made major advances with the development of the Koch pouch (internal ileostomy) and ileoproctostomy. Both procedures although associated with relatively high complication rates, are esthetically and psychologically better than standard ileostomy because in neither procedure must the patient wear an ileostomy appliance. However these advanced surgical procedures are typically not done until adolescence is reached.
Adolescent↗
Surgical treatment of the gastroesophageal reflux syndrome in infants and children.
During an 18 year period, 352 infants and children under 18 years of age underwent surgical treatment for symptomatic gastroesophageal reflux. Delayed gastric emptying was present in more than 50 percent as evidenced by more than 50 percent retention of technetium-99m sulfur colloid in semisolid feedings at 90 minutes. Esophageal motility disorders occurred in over 35 percent of the patients with symptomatic reflux, militating against performing a tight antireflux operation. Of 352 patients who had operation for symptomatic gastroesophageal reflux, 308 underwent gastroesophageal fundoplication alone, 26 underwent gastroesophageal fundoplication and pyloroplasty, 12 had pyloroplasty alone, and 6 had gastroesophageal fundoplication followed by pyloroplasty as a second operation because of residual delay in gastric emptying. An abnormal result of esophageal pH monitoring and decreased lower esophageal sphincter pressure with normal gastric emptying suggest use of the gastroesophageal fundoplication alone. Delay in gastric emptying combined with decreased lower esophageal sphincter pressure and abnormal results of esophageal pH monitoring indicate the use of gastroesophageal fundoplication plus pyloroplasty. A mildly abnormal esophageal pH value in combination with a high-normal lower esophageal sphincter pressure and marked delay in gastric emptying (over 60 percent retention at 90 minutes) suggest the use of pyloroplasty alone. The excellent clinical results achieved with gastroesophageal fundoplication, with or without pyloroplasty, and the low morbidity and mortality rates indicate that these procedures should be used early in the management of infants and children with symptomatic gastroesophageal reflux syndrome.
Adolescent↗
Surgical management of the gastroesophageal reflux syndrome in childhood.
Gastroesophageal reflux (GER) is a common cause of repeated emesis, failure to thrive, repeated pulmonary infection, and asthma in infants and children. During a 14-year period 270 children underwent gastroesophageal fundoplication for symptomatic reflux. The 24-hour esophageal pH monitoring is the most accurate test available to verify the presence of GER and is also helpful in evaluating the results of fundoplication. Transabdominal fundoplication may be performed with a low risk of complications. The most frequent complication requiring reoperation is paraesophageal hiatus hernia (6/270 patients), which should be repaired in almost all instances when symptoms develop. Closure of the crura posterior to the esophagus greatly reduces the incidence of this problem. Esophageal motility disorders occur in more than 35% of patients with symptomatic reflux and militate against performing a tight antireflux operation. Approximately 50% of patients with symptomatic reflux have associated gastric motility disorders. Radionuclide studies with 99mTC sulfur colloid in semisolid feedings have determined the magnitude of gastric retention after a feeding and have been helpful in identifying children who require a pyloroplasty with or without fundoplication. Pyloroplasty is performed simultaneously with fundoplication in approximately 10% of patients with symptomatic reflux when the lower esophageal sphincter pressure is low and the esophageal pH monitor shows reflux. The excellent clinical results achieved by fundoplication with or without pyloroplasty and the low morbidity and mortality rates indicate that these procedures should be used early in the management of infants and children who suffer symptomatic GER.
Adolescent↗
Somatic growth and developmental functioning in children receiving prolonged home total parenteral nutrition.
Growth and psychomotor development in nine infants receiving prolonged home total parenteral nutrition (TPN) were studied longitudinally from infancy to 3 years of age. Although these children had received TPN for, on average, 79% of their lives, normalization of somatic growth occurred by 2 years of age in all of them. Three children maintained average or above average levels of developmental performance over the 3-year study period. Another four children had initially delayed development but average or above average developmental scores by 18 months. In two children the rate of development gradually slowed without obvious cause, so that by 3 years they functioned in the below average to mildly retarded range.
Body Weight↗
Long-term hyperalimentation in children through saphenous central venous catheterization.
During a 3.5 year period, 151 Silastic central venous catheters were inserted into the inferior vena cava through the saphenous vein in 132 children younger than age 19 years at UCLA Hospital. The major indications for catheter insertion included inflammatory bowel disease, cancer or bone marrow transplantation, and short bowel syndrome. The 151 catheters were used for a total of 13,288 days of fluid administration (mean 88 days). Complications requiring removal of the catheter occurred in 31 patients, but there were no deaths attributable to complications from the catheters. The total incidence of complications was one per 225 days of catheter use, less than that occurring in catheters placed in the external jugular and cephalic veins in young children in our hospital. The ease of insertion, the low complication rate and the simplicity of patient management when catheters are placed into the vena cava through the saphenous vein appear to make this an optimal site for administering parenteral nutrition in infants and children.
Catheterization↗
"Antral dysmotility". An unrecognized cause of chronic vomiting during infancy.
Ten infants who were less than nine months of age and had chronic nonbilious vomiting were evaluated for gastroesophageal reflux. Upper gastrointestinal series in all showed delayed gastric emptying, a funnel-shaped antrum, absent antral peristalsis, and gastroesophageal reflux. None had evidence of anatomic obstruction in the stomach or duodenum. Manometric studies of the esophagus revealed either normal (4 patients) or elevated (6 patients) lower esophageal sphincter pressures. One-hour esophageal pH probe tests were negative in seven of the ten infants. This obstruction to gastric emptying appears to represent a disorder of antral motility. Medical management, which consisted of small frequent feedings and postural therapy, was successful in six of the infants. The remaining four patients required pyloroplasty. All of the infants are now asymptomatic and gaining weight at either a normal or accelerated rate for their ages. Familiarity with this previously undescribed disorder should prevent the incorrect diagnosis of gastroesophageal reflux secondary to lower esophageal sphincter incompetence and the resulting inappropriate surgical reconstruction.
Chronic Disease↗
Gastroesophageal fundoplication for the management of chronic pulmonary disease in children.
Gastroesophageal reflux is a common cause of chronic pulmonary disease in children. Forty-two children with recurrent pneumonia or severe asthma were evaluated and shown to have signicant reflux. Esophagography and esophageal pH testing proved the best diagnostic tests for determining reflux. Although the pulmonary symptoms were often due to repeated aspiration, they appeared in several cases to be related to bronchospasm caused by acid in the upper esophagus. All of the children underwent Nissen fundoplication and gastrostomy an average of 30 months after the onset of pulmonary symptoms. Of the children who had preoperative pneumonia, 87 percent had no recurrence after operation. In 13 of the 14 asthmatic children who underwent operation, symptoms improved and less bronchodilator medication was required. Morbidity and mortality were closely related to the duration and severity of pulmonary disease.
Adolescent↗
Multiple interactions of a DNA-binding protein in vivo. III. Phage T4 gene-32 mutations differentially affect insertion-type recombination and membrane properties.
We have investigated in in vivo roles of T4 gene-32 protein in recombination. We have studied the effects of gene-32 mutations under conditions that allow normal DNA replication and are permissive for progeny production. Under these conditions, certain gene-32 mutations specifically reduce insertion-type (short-interval) recombination but none affect crossover-type (long-interval) recombination (see Figure 5). Heterozygote frequencies in all gene-32 mutants are similar to or higher than in a gene-32+ background and are not correlated with recombination deficiencies. "Recombination-deficient" alleles are dominant or codominant over the "recombination-proficient" gene 32 mutation tsL171. This explains apparent discrepancies between a gene-32 map deduced from two-factor crosses and the map derived from three-factor crosses. We have also found that the "recombination proficient" mutation tsL171 and it homdoalleles suppress the characteristic plaque morphology of rII mutants. Under restrictive conditions, tsL171 is partially suppressed by rII mutations, which allow the use of host ligase in recombination. Our present and previous results are discussed in terms of current recombination models. We conclude that gene-32 protein functions in recombination by forming a complex with DNA, with recombination enzymes and with membrane components. Since gene-32 protein interacts with many components of this recombination complex, gene-32 mutations may differentially affect various recombination steps.
Alleles↗
Pediatric intestinal transplantation at Packard Children's Hospital/Stanford University Medical Center: report of a four-year experience.
We report a 4-year experience of a new program in pediatric intestinal transplantation. Among 50 children referred for evaluation, 27 were listed for transplantation. Two children originally listed for combined liver/small bowel transplant were changed to isolated intestinal transplant as rehabilitation efforts resulted in full recovery of hepatic function. Eighteen children received 18 grafts: 12 liver/intestine, 5 isolated intestine, and 1 multivisceral. Mean age at transplant was 3.6 year with 75% of patients aged 0 to 2 years. Five listed children died while waiting and four were still on the list. Immunotherapy included antithymocyte globulin induction and tacrolimus, sirolimus, and prednisone maintenance. At 1 year, patient and graft survivals were 75% and 67%, respectively. For isolated intestine, 1 year survivals were 100% and 75%, while for combined liver/intestine, they were 71% for both. Enteral autonomy is 100% with total parenteral nutrition stopping by 35.8 days (mean). We had two patients develop posttransplant lymphoproliferative disorder and three, exfoliative rejection, one of whom recovered completely. In conclusion, our program in pediatric intestinal transplantation has become well established with a high proportion of smaller/younger children receiving grafts. Outcomes achieved levels expected based on The Intestinal Transplant Registry and UNOS criteria, which were better than expected for isolated intestinal transplants and achievement of enteral autonomy.
ABO Blood-Group System↗
Removal of a colonic foreign body by enteral lavage.
A 14-year-old boy was evaluated for the presence of a foreign body in the descending colon. It successfully passed after oral colonic lavage solution (Golytely), obviating endoscopic intervention. The approaches to colonic foreign body removal are reviewed.
Adolescent↗