Case report: splenic infarction and acute splenic sequestration in adults with hemoglobin SC disease.

While acute splenic sequestration and splenic infarction are commonly observed in infants and young children with sickle cell anemia, they are rarely experienced by adult hemoglobin S homozygotes because the recurrent splenic infarction that takes place during childhood is typically followed by scarring, atrophy, and splenic fibrosis. Both acute splenic sequestration and splenic infarction do remain relatively common in adults with the other sickle hemoglobinopathies. These episodes are almost certainly a consequence of the persistently enlarged and distensible spleens that often remain present in these conditions.

Predictive validity of a house-officer selection process at one medical school.

This study explored the rank list generated by a process of evaluating applicants for a surgery residency; the process is a revision of an older process (pre-1982) used for that purpose. The study's aim was to learn whether the new process is more predictive of residents' performances in their first year than was the process it replaced and whether the rough rankings computed directly from ratings of applicants are more predictive than the final rankings determined in a meeting by the department faculty, who modify the rough rankings. The 1982-1986 rankings and performances of 32 first-year residents at the University of North Carolina at Chapel Hill School of Medicine were studied and compared with the rankings and performances of 77 applicants from 1976 and 1978-1979 under the previous system.

Colonic and esophageal transepithelial potential difference in cystic fibrosis.

To evaluate differences in the expression of cystic fibrosis (CF) transport defects in the gastrointestinal tract of subjects with CF, in vivo measurements of colonic and esophageal transepithelial electrical potential difference (PD) were performed before and during amiloride superfusion in CF and healthy subjects. Esophageal PD before (-16 +/- 2 vs. -16 +/- 3 mV) and after (-14 +/- 2 vs.

Autonomously functioning thyroid nodules in childhood and adolescence.

Autonomously functioning thyroid nodules (AFTNs) in children and adolescents (under age 18) are unusual but are not as rare as earlier reports suggested. These lesions have a significantly different biologic potential than similar lesions in older patients. In the younger age group there is a more rapid progression toward toxicity and a higher incidence of thyroid carcinoma.

Current management of the patient with autonomously functioning nodular goiter.

Autonomously functioning thyroid nodules (AFTNs) are presumably independent of TSH for growth and function and appear "hot" on scintiscan because they selectively concentrate radionuclide to a greater extent than the remaining thyroid gland, which is controlled by the normal TH-TSH feedback mechanism. Such autonomously functioning tissue may occur in "patchy" areas, as a solitary nodule, or as multiple nodules (classic Plummer's disease), with the mass of hyperfunctioning tissue and the related secretion of thyroid hormones determining whether the patient is euthyroid or hyperthyroid. Important diagnostic tests include a 99mTc thyroid scan, T4 RIA, T3 uptake, FTI, TSH RIA, and occasionally T3 RIA ("T3 thyrotoxicosis").

Oophoropexy and the management of Hodgkin's disease. A reevaluation of the risks and benefits.

Female patients with Hodgkin's disease who undergo staging laparotomy frequently have oophoropexy performed to preserve both fertility and hormone production. Because of recent changes in therapy favoring systemic chemotherapy rather than total nodal irradiation for patients with stage III Hodgkin's disease, the need for oophoropexy may be less than previously described. Thirty-nine women of childbearing age underwent laparotomy at the University of North Carolina, Chapel Hill, from 1970 to 1984.

Hirschsprung's disease in young adults.

Hirschsprung's disease is rarely seen in the young adult, and presents unique problems in management because of the massive dilatation and hypertrophy that occur proximal to the aganglionic rectum or the rectosigmoid colon. The diagnosis, which may be suspected by barium enema, is confirmed by suction or full-thickness biopsy of the rectum that may be complemented by anal manometry. Based on our experience with eight patients, a two-stage surgical reconstruction is recommended, with a preliminary sigmoid colostomy through the normally innervated colon and an associated defunctionalized stoma constituting the initial operation.

Cholelithiasis in sickle cell anemia: surgical considerations.

Gallstones are frequently found in patients with sickle cell anemia. The differentiation between acute calculous biliary tract disease and sickle cell crisis can be difficult and should be based on the clinical presentation, comparison with previous episodes of abdominal pain, and judicious use of hepatobiliary radionuclide scanning. Emergency cholecystectomy is associated with a high morbidity and should be avoided if possible.

Hereditary spherocytosis. Recent experience and current concepts of pathophysiology.

Hereditary spherocytosis is a clinically heterogeneous, genetically determined red blood cell membrane disorder resulting in hemolytic anemia. A deficiency of spectrin, the largest and most abundant structural protein of the erythrocyte membrane skeleton, results in the formation of spherocytes which lack the strength, durability, and flexibility to withstand the stresses of the circulation. Clinical manifestations of the disease are primarily dependent on the severity of hemolysis, which additionally results in an increased incidence of pigment gallstones.

Neonatal intestinal obstruction from omphalomesenteric duct remnants.

Three cases of omphalomesenteric band obstruction in early infancy with various radiographic presentations, including intermittent obstruction in one, are reported. Small-bowel volvulus was found in all three patients at surgery. One case progressed to bowel necrosis with portal venous gas.

Primary hyperparathyroidism during pregnancy.

Primary hyperparathyroidism during pregnancy is associated with significant risk of fetal loss and neonatal and maternal morbidity. Neonatal hypocalcemia probably results from transient hypoparathyroidism consequent to abnormal suppression by fetal hypercalcemia. Loss of the protective effect provided by the placental calcium transport mechanism produces significant maternal risk for development of acute hypercalcemia and possible crisis immediately postpartum.

Intestinal endometriosis.

Endometriosis involving the intestine usually takes the form of asymptomatic, small, superficial serosal implants on segments of bowel lying in the pelvis in proximity to the genital organs. Deeper and more extensive intestinal wall involvement may result in obstruction and occasionally bleeding and requires distinction from a neoplasm or other inflammatory bowel process. Intestinal endometriosis should be considered in the differential diagnosis of recurring lower abdominal pain and other episodic bowel symptoms in women of child-bearing age.

Acute jejunogastric intussusception.

Our patient represents an example of acute retrograde jejunograstric intussusception with the additional finding of a bleeding jejunal ulcer in the intussusception. The ulcer may have been etiological in precipitating the intussusception, or a consequence of mucosal sloughing in the hemorrhagic intussusception. Early diagnosis and immediate surgical therapy resulted in an uneventful recovery.

Aneurysms of the hepatic artery.

Aneurysms of the hepatic artery are rare and, when symptomatic, they may present a triad of upper abdominal pain, gastrointestinal bleeding, and obstructive jaundice. Asymptomatic and unsuspected aneurysms are demonstrated occasionally by abdominal arteriography or are encountered infrequently during abdominal operation for an unrelated disease. Hepatic artery aneurysms have great potential for rupture with bleeding into the peritioneal cavity, the common bile duct, or an adjacent hollow viscus.

Angiographic assessment of resectability in primary hepatic carcinoma.

The nature of primary hepatic malignancy and the magnitude of operative procedures for treatment dictate that hepatic resection be carried out only when there is the chance of cure. Following resection, a sufficient amount of liver with an intact afferent and efferent vascular system must remain to sustain life. Complete hepatic angiographic evaluation by arteriography, inferior vena cavography, hepatic venography and portal venography provides valuable information about extent of tumor involvement and the anticipated hepatic remnant.

Diagnosis of an enteric duplication with pertechnetate 99mTc scanning.

A case of enteric duplication is reported in which preoperative pertechnetate (99m)Tc scanning demonstrated localized uptake in the region of the anatomic abnormality. This test is recommended for use in the evaluation of lower gastrointestinal bleeding in infants and children.

Tumors of the small intestine.

An analysis of 80 cases of malignant and benign tumors of the small intestine is presented, and major clinical features of various histologic lesions are discussed. Tumors of the small intestine are rare, and the small bowel may have intrinsic protective systems against the development of neoplasms. Gastrointestinal bleeding and symptoms of intermittent obstruction are prominent findings in patients with symptomatic benign tumors, although many benign tumors are incidental findings at operation for an unrelated disease.