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Biomedical subjects

R L Telander

Publications and source records attributed to R L Telander.

At least 37 records · Page 2Linked to original sources
PubMed1989

Clinical outcome in children with acute cerebellar encephalopathy and neuroblastoma.

Acute cerebellar encephalopathy (ACE)--ataxia often associated with opsoclonus, polymyoclonus, and irritability--may be associated with neuroblastoma and should be suspected in a child who presents with ACE. The survival in ten children with ACE associated with neuroblastoma was 100%. Most of the tumors were ganglioneuroblastomas. The abdomen was the most common location for the tumor in this study although it also can be found in the mediastinum. All patients with this syndrome had a localized tumor. With ACTH therapy, ACE may resolve early postoperatively, but it tends to recur in most patients, continuing for as long as 3 years after the initial operation. Treatment with ACTH or prednisone modifies and rapidly clears the symptoms during the acute episode of recurrent ACE as well as helps to resolve subsequent neurologic sequelae. Significant neurologic sequelae persisted in seven of ten patients and included deficits in cognition or intellect, hyperactivity, impulsivity, emotional lability, and mild motor deficits. Seizures were seen in one patient.

Abdominal Neoplasms↗
PubMed1988

Distribution, quantitation, and origin of immunoreactive neuropeptide Y in the human gastrointestinal tract.

A radioimmunoassay for measurement of immunoreactive neuropeptide Y has been developed using antiserum from a rabbit (221) immunized with porcine neuropeptide Y. Antibody 221 has been characterized for both sensitivity and specificity. To determine the distribution of neuropeptide Y in the human gastrointestinal tract, fresh tissue specimens were separated by microdissection into the muscularis externa and the mucosa-submucosa. To examine the origin of neuropeptide Y in human colon, specimens of aganglionic and ganglionic colon were obtained from patients with Hirschsprung's disease. Immunoreactive neuropeptide Y in human gut was present in highest concentrations in the muscularis externa of the stomach and in lowest concentrations in the muscularis externa of the ileum and descending colon. Neuropeptide Y in the stomach was present in higher concentrations in the muscularis externa than in the mucosa-submucosa, but in the descending colon there were lower concentrations of neuropeptide Y in the muscularis externa than in the mucosa-submucosa. In Hirschsprung's disease, concentrations of neuropeptide Y were increased in aganglionic colon in both the muscularis externa and the mucosa-submucosa, compared to corresponding layers from proximal ganglionic colon. Extracts of the gastric muscularis externa and the colonic mucosa-submucosa were separated by C18 reverse-phase high-performance liquid chromatography. One major immunoreactive species was identified by radioimmunoassay which eluted in a position similar to synthetic human neuropeptide Y. These results demonstrated both regional and layer differences in concentrations of neuropeptide Y in human gut. Increased concentrations of neuropeptide Y in aganglionic colon from Hirschsprung's disease most likely result from enlargement of neuropeptide Y-containing extrinsic nerve fibers in both the mucosa-submucosa and the muscularis externa.

Animals↗
PubMed1988

Localization of pancreatic insulinoma: comparison of pre- and intraoperative US with CT and angiography.

Methods of preoperative radiologic localization of insulinoma were compared in 52 patients, 44 of whom had solitary tumors. Examinations performed in these 44 patients were preoperative ultrasonography (US) in 28, angiography in 26, and computed tomography in 23. Prospective sensitivities were 61%, 54%, and 30%, respectively. Imaging sensitivities were lower for the eight patients with multiple insulinomas. In 28 of the 44 patients, intraoperative US was performed without the examiner being aware of the surgical findings. The sensitivity was 84%. Four insulinomas were not palpable but were visualized sonographically. The combined sensitivity of intraoperative US and surgical palpation for detecting solitary insulinomas was 100%. High-frequency intraoperative US is valuable for detecting occult solitary insulinomas and considerably useful for determining the proximity of insulinomas to the pancreatic and bile ducts.

Adenoma, Islet Cell↗
PubMed1987

Does rectal mucosa regenerate after ileoanal anastomosis?

Regeneration of rectal mucosa after rectal mucosectomy and ileoanal anastomosis (IAA) could jeopardize the long-term safety of the procedure. The aim of this study was to determine if rectal mucosal regeneration occurred after IAA. Pathologic specimens of the IAA and surrounding rectal muscular cuff were obtained from 29 patients who had required IAA excision 17 +/- 2 months (mean +/- SEM, range, 2 to 48 months) following construction. Multiple (greater than or equal to 6) coronal and sagittal sections of each specimen were made and examined histologically. The rectal muscle cuff was bound to ileal serosa by dense fibrous tissue. Small islets of residual rectal mucosa were identified between the denuded rectal cuff and the ileal pull-through in four patients (14 percent) and at the ileoanal anastomosis in two patients (7 percent). Active rectal mucosal disease, dysplasia, or reepithelialization of the denuded rectal muscle were not seen. It is concluded that small islets of rectal mucosa may remain after IAA. Up to four years after IAA, however, no evidence of rectal mucosal regeneration could be documented.

Adolescent↗
PubMed1986

Results of early thyroidectomy for medullary thyroid carcinoma in children with multiple endocrine neoplasia type 2.

Children with multiple endocrine neoplasia type 2 (MEN2) often develop medullary carcinoma of the thyroid (MCT) or its precursor, C-cell hyperplasia. Survival results are improved if malignancy is diagnosed early from the results of plasma immunoreactive calcitonin (iCT) measurement. The effect of early detection and thyroidectomy in children with MEN2 syndrome was determined by reviewing the experience between 1975 and 1985. Seventeen children with MEN2 who were 12 years old or younger underwent a total thyroidectomy for MCT or C-cell hyperplasia. iCT was measured in all patients preoperatively and postoperatively. Of the 17 children, 14 (82%) had MEN2a and 3 (18%) had MEN2b. There were 14 (82%) female and three (18%) male patients; their mean age was 6.97 years (range 1.5 to 12 years). In all patients, the diagnosis of MCT was made from initial elevated levels of iCT after stimulation with pentagastrin. Three patients had clinical evidence of disease preoperatively. All patients underwent a total thyroidectomy and lymph nodes were removed from the central zone; a neck dissection was performed in the three with clinically obvious disease. MCT with C-cell hyperplasia was found in 11 children and C-cell hyperplasia alone in six. Of the 11 with carcinoma, eight had bilateral disease and three unilateral. Six children had bilateral C-cell hyperplasia. All 17 children were alive and feeling well at the time of this report; however, three had evidence of metastatic disease according to iCT measurements. None of the children had recurrent nerve injuries; one had evidence of hypoparathyroidism.(ABSTRACT TRUNCATED AT 250 WORDS)

Calcitonin↗
PubMed1986

Intraoperative ultrasonography of the pancreas in children.

In children, lesions of the pancreas often are small, and precise localization is required for optimal surgical management. We have used newer-generation real-time ultrasonography of the pancreas intraoperatively in seven children. Five of these children had hypoglycemia, hyperinsulinemia, and insulinomas; one had a persistent small pancreatic pseudocyst with a disrupted secondary duct, and one had familial pancreatitis with a remarkably enlarged duct and a stone. Two of the five with adenomas had multiple endocrine neoplasia syndrome I (MEN I syndrome); in them, ultrasonography localized several adenomas preoperatively and several additional adenomas intraoperatively. This allowed a 90% pancreatectomy with enucleation of small adenomas in the remaining head. One child had a nonpalpable insulinoma deep in the head of the pancreas; intraoperative ultrasonography localized the lesion and permitted successful enucleation. Another child with a small pseudocyst and a disrupted secondary pancreatic duct ultimately required surgical drainage; intraoperative ultrasonography of the inflammatory mass immediately localized the small pseudocyst and thus decreased the operative time. The child with familial pancreatitis appeared to require a surgical drainage procedure; however, intraoperative ultrasonography demonstrated that the stone had passed spontaneously just prior to operation and the duct size had returned to normal, eliminating the need for the drainage procedure at that time.

Adenoma, Islet Cell↗
PubMed1986

Endocrine disorders of the pancreas and adrenal cortex in pediatric patients.

Hyperinsulinemia, a common cause of persistent hypoglycemia in infants and children, can result in permanent damage to the central nervous system. Thus, early diagnosis and treatment are important. The typical clinical manifestation of hyperinsulinemic hypoglycemia is symptomatic hypoglycemia that responds poorly to medical therapy. Affected infants may have tremors, jitteriness, apnea, cyanosis, or seizures. If initial medical therapy (frequent feedings, large amounts of intravenously administered glucose, diazoxide, and glucocorticoids) fails to stabilize plasma glucose levels, subtotal pancreatectomy is indicated. This surgical intervention does not completely correct the hypoglycemia in all patients, but it effectively reduces the severity of the condition. Surgical treatment of pediatric patients with Cushing's syndrome or aldosteronoma has also been effective. In our experience, survival of patients with adrenocortical adenomas has been 100%, whereas only five of nine children with adrenocortical carcinomas survived, and four of the five were younger than 10 years of age.

Adrenal Cortex Neoplasms↗
PubMed1986

Benign pancreatic insulinoma: preoperative and intraoperative sonographic localization.

Twenty-nine patients with surgically proved benign pancreatic insulinoma were studied by preoperative or intraoperative sonography. Twenty-five patients had solitary pancreatic tumors; four had multiple tumors. Six of the patients with solitary insulinomas and one of the patients with multiple insulinomas had undergone previous unsuccessful exploration. Preoperative sonography was performed in 24 patients with solitary insulinomas, and 15 (63%) were localized. Intraoperative sonography was performed in 22 patients with solitary insulinomas, and 19 (86%) were visualized without having been previously located by palpation. Four of these visible solitary tumors (18%) were not detected by palpation at surgery. All the solitary insulinomas were detected with the combination of palpation and intraoperative sonography. In each of the six patients with solitary insulinoma who had undergone previous surgery, the tumor was visible with intraoperative sonography, which also demonstrated nonpalpable insulinomas in two of the four patients with multiple tumors. Preoperative real-time sonography is a sensitive, noninvasive, inexpensive method for localization of insulinoma. Intraoperative high-frequency sonography is a highly sensitive method for the detection of insulinoma. Intraoperative sonography is also valuable to determine the relationship of the insulinoma to pancreatic and bile ducts and thereby facilitate safe enucleation.

Adenoma, Islet Cell↗
PubMed1985

Surgical management of hyperinsulinism in the multiple endocrine neoplasia, type 1 syndrome.

We retrospectively reviewed the records of 12 patients from one institution (from 1970 through 1983) who had hyperinsulinism as a part of the multiple endocrine neoplasia, type 1 syndrome. All of the patients underwent surgical exploration: ten had subtotal pancreatectomies, one had enucleation, and one underwent a total pancreatectomy. There was no operative mortality. Preoperative and intraoperative localization studies (angiography and ultrasonography) tended to underestimate the number of pancreatic tumors. Ten patients had multiple B-cell adenomas (mean, 7.4), while two patients had single tumors. This study confirms that hyperinsulinism, when it occurs as part of the multiple endocrine neoplasia, type 1 syndrome, is usually due to multiple islet cell tumors, and that the islet cell tissue is probably diffusely dysplastic. Subtotal (85%) pancreatectomy is suggested as the procedure of choice for this subgroup of patients with hyperinsulinism.

Adenoma↗
PubMed1985

Anal sphincter electromyography after colectomy, mucosal rectectomy, and ileoanal anastomosis.

Electromyography (EMG) was used to evaluate the external anal sphincter in 27 patients following colectomy, distal mucosal rectectomy, and ileoanal anastomosis. The studies were conducted four months to 58 months (mean, 20 months) following the restoration of intestinal continuity. Nine patients underwent endoanal rectal mucosal stripping, while in 18 patients the rectum was everted to facilitate the stripping. Postoperative continence varied widely, from perfect to frequent and severe mucous of fecal leak. Abnormal motor-unit potentials were identified by EMG in nine patients and this finding was usually associated with poor continence. The sex of the patient, technique of mucosal stripping, and type of anastomosis did not influence the EMG result, but patients at least 40 years old all had abnormal EMGs. We conclude that poor continence after ileoanal anastomosis correlates with an abnormal EMG of the external anal sphincter. The cause of the EMG abnormality is unclear.

Adolescent↗
PubMed1985

Anoneorectal evaluation after colectomy and endorectal ileoanal anastomosis in children and young adults.

Recent improvements in the technique of colectomy, rectal mucosectomy, and endorectal ileoanal anastomosis allow a satisfactory result in most patients. However, the clinical outcome is not entirely satisfactory in about 5% to 10% of patients because of excessive stool frequency or episodic fecal incontinence or both. We evaluated anoneorectal function postoperatively to help explain the mechanisms of the difficulties. Six patients with imperfect functional results (group 1) and 6 with good functional results (group 2) after ileoanal anastomosis and closure of the loop ileostomy were compared with 12 healthy volunteers who had not had operation, through a series of tests designed to evaluate anal sphincter and neorectal function. All patients were instructed in balloon dilation of the neorectum to develop a reservoir while awaiting closure of the ileostomy. Anal sphincter manometric measurements of resting and squeeze pressures were obtained with a 4-channel probe attached to a noncompliant pneumohydraulic perfusion system. Incremental inflation of an intraluminal bag while pressures were simultaneously recorded allowed determinations of neorectal capacity and distensibility. The efficiency of neorectal evacuation was assessed by instilling a labeled synthetic viscous load into the distal bowel. Patients in group 1 had lower resting anal pressures (P less than 0.05), lower squeeze pressures (P less than 0.05), smaller neorectal capacities (P = 0.13), and less neorectal distensibility (P = 0.27) than patients in group 2. Furthermore, the values for patients in group 2 closely approximated those found in healthy volunteers.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1985

The effect of intra-abdominal resection or fecal diversion on perianal disease in pediatric Crohn's disease.

To determine whether perianal Crohn's disease responds to resection of active intra-abdominal disease or to diversion, 273 cases of Crohn's disease in children (ages 4 to 18 years) were reviewed (all had been seen between 1970 and 1983). Of these, 104 patients underwent an intestinal operation, and 42 of the 104 (40%) had documented perianal disease at the time of their operation. Follow-up averaged 8 3/4 years from time of diagnosis. Three of the 42 died, at 1, 3, and 5 months postoperatively (mortality, 7%) and one was unavailable for follow-up. In these 42 patients with active perianal disease, an intestinal resection with primary anastomosis was performed in 19; one died postoperatively and one was unavailable for follow-up. Of the remaining 17 patients, five (29%) improved and 12 (71%) had continued perianal disease, either without change or worse. At least one further intestinal resection was needed in five of the 17 and two have needed proctocolectomy. A defunctionalizing procedure was done as part of the initial operation in 11 of the 42 patients. Two showed improvement; however, both needed proctocolectomy for rectal disease, and none of the 11 with fecal diversion have had reestablishment of intestinal continuity. In 12 of the 42 patients proctocolectomy was the initial procedure, usually for severe rectosigmoid disease. Of 38 children followed, 20 had proctocolectomy, 11 initially and nine after lesser procedures; overall rate of proctocolectomy was 53%. Resection of active intra-abdominal disease is not necessarily followed by resolution of perianal lesions. Defunctionalizing the rectum with fecal diversion does not appear to alter perianal disease.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1985

Pediatric endocrine surgery.

The pediatric surgeon is in a unique position to understand endocrine surgery and, therefore, is expected to develop considerable expertise in this area. In recent years numerous advances and changes have occurred in pediatric endocrine surgery that have led to greater understanding of the disease processes and syndromes and the development of new diagnostic techniques and surgical approaches.

Adolescent↗
PubMed1984

Surgical management of hyperinsulinemic hypoglycemia in children.

Nineteen children underwent subtotal pancreatectomy for hyperinsulinemic hypoglycemia. Of the four who were older than 10 years of age at onset of symptoms, three had islet adenomas, and one had endocrine cell dysplasia (ECD). The two patients with multiple adenomas had a family history of multiple endocrine neoplasia, type 1 (MEN 1). Of the 15 who were younger than 1 year of age at onset of symptoms, 12 had ECD alone, and three had ECD with adenomatosis. Four children required a second surgical procedure and near-total pancreatectomy because of persistent hypoglycemia. All 19 patients' conditions improved, with no postsurgical complications. After near-total pancreatectomy, all four patients were treated for fat malabsorption, but only two required insulin because of secondary diabetes mellitus. We concluded that subtotal pancreatectomy is a safe, effective adjunct to the treatment of children with hyperinsulinemic hypoglycemia.

Adenoma↗
PubMed1984

Prognosis and management of lesions of the trunk in children with Klippel-Trenaunay syndrome.

The Klippel-Trenaunay syndrome is a congenital vascular anomaly consisting of the triad of soft-tissue and bony hypertrophy of the extremities, hemangiomas and/or lymphangiomas, and varicosities. From 1956 to 1983, 42 patients with the Klippel-Trenaunay syndrome were seen at the Mayo Clinic. Of the 42 patients, 16 had involvement of the trunk (thorax, abdomen and/or pelvis). Twelve of the 16 had evidence of the malformation at birth and 4 had evidence shortly thereafter. Lesions involving the trunk included hemangiomas (75%) and lymphangiomas (50%). Complications included rectal bleeding, hematuria, colonic obstruction, hemothorax, paraparesis secondary to clotting disorder, and compression of the external urethral meatus. One child died of enlargement of diffuse lymphangiomas and hemangiomas of the abdomen and chest. Eight of the 16 patients underwent 13 operative procedures. These included excision of the superficial lymphangiomas, resection of the rectosigmoid colon, and resection of an extremely large retroperitoneal mass of hemangiomatous-lymphangiomatous tissue. While progression requiring major surgery was seen in several patients, most patients had supportive therapy, with minimal surgery being necessary.

Abdomen↗
PubMed1983

Fate of the rectal mucosa after rectal mucosectomy and ileoanal anastomosis.

The aim of our study was to determine if the rectal mucosa regenerates after rectal mucosectomy and endorectal ileoanal anastomosis for chronic ulcerative colitis. Such regenerated rectal mucosa could be the site of recurrent disease, leading to complications of the operation, and potential malignant degeneration. Pathologic specimens of the ileoanal anastomosis, surrounded by rectal muscular cuff, were obtained from eight patients who required takedown of their ileoanal anastomosis between one and 18 months after construction. Reepithelialization of the rectal cuff was not observed. In two patients, small islets of rectal mucosa and anal glands were identified. In all patients, the rectal muscularis propria was adherent to the serosa of the ileum by fibrous reaction. Three patients were diagnosed, both clinically and pathologically, as having chronic ulcerative colitis at the original ileoanal operation, but features suggestive of Crohn's disease were noted in the subsequently resected neo-rectum. Our observations suggest that, although isolated rectal mucosal cells may remain after mucosectomy, extensive rectal mucosal regeneration does not occur, thus minimizing the risk of recurrent disease and potential malignant change. Failure of an ileoanal anastomosis is therefore most likely related either to technical factors or to the presence of unsuspected Crohn's disease.

Adolescent↗