Bernard M. Jaffe

Living-related small bowel transplantation in adults: A report of two patients

Abstract Virtually all of the small bowel transplants performed to date have utilized the whole intestine harvested from cadaveric donors. There may be distinct potential advantages to the transplantation of segments of small intestine. Experimentally, segments undergo adaptive hyperplasia, resulting in deeper crypts and taller villi. The increased mucosal surface area augments intestinal absorption and restores function in enterectomized animals. There is a direct relationship between the mass (length) of the graft and its immunogenicity in small animals, with longer segments rejecting more rapidly and mandating the use of higher doses of immunosuppression. Also, there is substantial immunologic advantage to the use of jejunum, rather than ileum in rats, although bowel from both sites otherwise function equally well. Finally, transplantation of segments would solve the problem of availability of intestine, and might overcome size restrictions, perhaps even permitting the use of adult donors for pediatric recipients. Based on these observations, and the potential advantage afforded by immediate revascularization, we initiated a program of living-related small intestinal transplantation in adults.

Islet Cell Tumors of the Pancreas

Pancreatic endocrine neoplasms are a heterogeneous group of tumors that produce active hormones and result in distinct clinical syndromes. For the most part, they are malignant and require sophisticated diagnostic and localization techniques in order to identify their presence. Delays in diagnosis are the rule rather than the exception. Improvements in the diagnosis of gastrinomas and insulinomas appear to result in an increase in resectability rates. The widespread availability of intraoperative ultrasonography, as well as improved knowledge of the location of these tumors, has also had an impact on improved cure rates. With heightened awareness of these syndromes, increasing numbers of patients can be identified and more effective treatments developed for the refractory and recurrent tumors.

Adult nesidioblastosis : A case report and review of the literature

George F. Laidlaw first described a pancreatic abnormality now known to be the most common cause of persistent hyperinsulinemic hypoglycemia in infants in 1938 (1, 2). The term he coined, nesidioblastosis, is derived from the Greek words for “islets” (nesidia) and “germ” (blastos) (3). It accurately describes the characteristic feature of nesidioblastosis, islet cells differentiating and budding from ductal epithelium. In adults, hyperinsulinemic hypoglycemia is rarely caused by nesidioblastosis and is usually caused by insulinoma or exogenous insulin treatment (4, 5). The first case series of adult nesidioblastosis was reported by Harness et al in 1981 (6). Since this case series of six patients, there have been only sporadic literature reports of adult nesidioblastosis, documenting fewer than 20 cases of adult nesidioblastosis over the past 15 years (3, 7-10). This paper presents an adult patient with hyperinsulinemic hypoglycemia due to nesidioblastosis and provides a guide to the diagnosis and treatment of this rare disorder in the adult population.

Correction of congenital indirect hyperbilirubinemia by small intestinal transplantation

INTRODUCTION Crigler-Najjar syndrome, type I, is a disease characterized by complete absence of hepatic bilirubin glucuronidation. The congenital indirect hyperbilirubinemia is due to an autosomal recessive deficiency of the enzyme, uridine diphosphate glucuronosyl transferase (UDPGT). The inbred homozygous Gunn rat is also deficient in UDPGT, exhibits unconjugated hyperbilirubinemia, and is an excellent animal model of the Crigler-Najjar syndrome. This study was performed to test the ability of transplanted intestine from normal Wistar rat donors to correct the deficiency in hepatic bilirubin conjugation. MATERIALS AND METHODS In phase 1, Gunn rats underwent 40-cm heterotopic small-bowel transplants from either Wistar (experimental) or Gunn (control) rats. In phase 2, 15- to 20-cm Wistar-to-Gunn jejunal transplants were placed either heterotopically or orthotopically (in intestinal continuity). All rats were treated with cyclosporin A (CsA), 5 mg/kg per day. Serum bilirubin levels were determined spectrophotometrically at weekly intervals posttransplantation. In phase 2, UDPGT activity was quantitated at 0, 2, 4, and 8 weeks using known quantities of bilirubin as substrate. RESULTS Total bilirubin levels decreased significantly in the 40-cm heterotopic transplant recipient rats. From the initial values of 7.12 +/- 0.59 mg/dL, levels reached the nadir of 4.23 +/- 0.27 mg/dL. A parallel drop in serum levels of indirect bilirubin was noted (5.04 +/- 0.54 mg/dL to 2.74 +/- 0.23 mg/dL). After 6 weeks, bilirubin levels began to rise toward pretransplant values. In contrast, there was no significant change in bilirubin levels in the control Gunn-to-Gunn rats. Fifteen- to 20-cm heterotopic Wistar-to-Gunn transplants caused a qualitatively similar drop in total and indirect bilirubin levels. Orthotopic (in continuity) Wistar-to-Gunn transplants lowered serum bilirubin levels more rapidly, and the effect was sustained throughout the 8-week study period. By 1 week posttransplantation, total bilirubin levels dropped from 5.11 +/- 0.48 mg/dL to 2.41 +/- 0.16 mg/dL (P < 0.05); data at 8 weeks averaged 1.84 +/- 0.35 mg/dL. Respective data for indirect bilirubin levels were 4.81 +/- 0.45 mg/dL, 2.26 +/- 0.18 mg/dL, and 1.35 +/- 0.39 mg/dL. Wistar rat UDPGT activity in intestine and liver averaged 0.61 +/- 0.05 and 1.88 +/- 0.06 mg bilirubin conjugated/mg tissue per hour, respectively. Enzyme activity in the transplanted intestine persisted throughout the course of the study. CONCLUSION Transplants of small intestine with known UDPGT activity partially corrected the deficiency in Gunn rats and allayed the hyperbilirubinemia. Since the small intestine is known to contain small but significant amounts of a large number of predominantly hepatic enzymes, bowel transplantation may be an appropriate treatment for this and other similar genetic enzyme deficiencies.

Therapy-induced leukemias and myelodysplastic syndromes after breast cancer treatment: an underemphasized clinical problem.

With the advent of multidisciplinary oncologic therapeutic regimens, cancers are being treated with greater effectiveness, as measured by increases in both diseasefree and actuarial survival rates. The application of all of these treatment arms into a single, focused therapeutic attack has allowed patients to live far longer than was thought possible 30 years ago. However, for some patients, oncologic treatment (and resultant success) has come with a price, namely, therapy-induced malignancies.1 3 Although this is an important clinical problem, it is an underemphasized and underreported phenomenon in the literature. By using a typical case report to put this problem in perspective, this article reviews the frequency and mechanism of secondary malignancies after therapy for breast cancer.

Pancreatic polypeptide islet cell tumor: case report and review of the literature.

Pure pancreatic polypeptide-containing tumors (PPomas) are quite rare. Only 20 cases have been described. In this article we report a 75-year-old woman with such an endocrine islet cell tumor. The patient had no specific symptoms that could be ascribed to the tumor. An abdominal CT scan revealed a 3 cm soft tissue mass arising inferiorly from the tail of the pancreas. Local resection by way of a distal pancreatectomy was performed. A well-circumscribed hemorrhagic multiloculated mass, 3.7 cm in greatest dimension, was present in the tail of the pancreas. The patient has remained well and tumor free for the past 22 months. The endocrine characterization of the tumor was achieved by means of immunohistochemical analysis. Staining specific for insulin, ghicagon, somatostatin, and gastrin was negative. In contrash staining of the tumor for pancreatic polypeptide was strongly positive. A number of nonfunctioning islet cell tumors of the pancreas have been described. The lack of fimction has previously been suggested to indicate the lack of secretion of an endocrine product. This report documents that islet cell tumors may function by secreting pancreatic polypeptide but not cause symptoms.

Primary Peripheral Nerve Sheath Tumors of the Thyroid Gland

BACKGROUND Primary peripheral nerve sheath tumors (PNSTs) of the thyroid gland are exceptionally rare tumors that usually present as asymptomatic neck nodules in adults. This article presents a literature review of these tumors. SUMMARY PNSTs of the thyroid can be classified into benign and malignant. Only three cases of malignant PNSTs have been reported. Benign PNSTs of the thyroid include neurofibromas and schwannomas. Only two cases of isolated neurofibroma of the thyroid have been reported. Schwannomas are typically benign, slow-growing tumors that originate from neuronal schwann cells, with a clinical picture depending on the anatomic size and site. Pathologically, schwannomas are classified into Antoni A and Antoni B. Only 17 cases of schwannomas of the thyroid exist in literature to date. CONCLUSIONS Schwannomas of the thyroid gland are extremely rare and usually asymptomatic. Complete surgical resection is mandatory for care.

Two Unusual Cases of Adult Intussusception

Adult intussusception is very rare. We report 2 unusual cases, a 58-year-old man with a transverse colo-colonic intussusception caused by a malignant sessile polyp that also had an asymptomatic synchronous neoplasm of the kidney, and an 18-year-old female with an ileocecolic intussusception caused by acute appendicitis. This report stresses the point that intussusception in adults may represent an underlying malignancy. The age of the patient and the anatomic location of the intussusception provide significant input as to the etiology and hence the most appropriate surgical procedure.

Papillary thyroid carcinoma in black thyroids

Black thyroid is a rare condition. It has been considered to be pathognomonic of chronic minocycline ingestion for more than 30 years, although it can also occur in patients with hemochromatosis, ochronosis, mucoviscidosis, and hemorrhage. A possible association of black thyroid with thyroid cancer has been considered, but no direct causal relationship has been established. Hence, the purpose of this article was to identify the malignant potential of such glands.

Black Thyroid Associated with Thyroid Carcinoma

Objective. Black thyroid is a rare pigmented change seen almost exclusively in patients upon minocycline ingestion, and the process has previously been thought to be generally benign. There have been 61 reported cases of black thyroid. We are aware of 13 cases previously reported in association with thyroid carcinoma. This paper reports six patients with black thyroid pigmentation in association with thyroid carcinoma. Design. The medical records of six patients who were diagnosed with black thyroid syndrome, all of whom underwent thyroid surgery, were reviewed. Data on age, gender, race, preoperative fine needle aspiration biopsy (FNA), thyroid function levels, and pathology reports were collected. Main Outcome. The mean age was 60 years. There were 5 females, 4 of whom were African American. All patients were clinically and biochemically euthyroid. Black pigmentation was not diagnosed in preoperative FNA, and only one patient had a preoperative diagnosis of papillary thyroid carcinoma. The other patients underwent surgery and were found to have black pigmentation of the thyroid associated with carcinoma. Conclusions. FNA does not diagnose black thyroid, which is associated with thyroid carcinoma. Thyroid glands with black pigmentation deserve thorough pathologic examination, including several sections of each specimen.