Adult patients with short bowel syndrome treated with teduglutide: A descriptive cohort study.

BACKGROUND: Teduglutide is a synthetic glucagon-like peptide-2 analogue approved for the treatment of short bowel syndrome associated with chronic intestinal failure (SBS-IF) in adult patients. Clinical trials have demonstrated its ability to reduce parenteral support (PS) requirement. This study aimed to describe the effect of 18-month treatment with teduglutide, evaluating PS and factors associated with PS volume reduction of ≥20% from baseline and weaning. Two-year clinical outcomes were also assessed. METHODS: This descriptive cohort study collected data prospectively from adult patients with SBS-IF treated with teduglutide and enrolled in a national registry. Data were collected every 6 months and included demographics, clinical, biochemical, PS regimen, and hospitalizations. RESULTS: Thirty-four patients were included. After 2 years, 74% (n = 25) had a PS volume reduction of ≥20% from baseline, and 26% (n = 9) achieved PS independency. PS volume reduction was significantly associated with longer PS duration, significantly lower basal PS energy intake, and absence of narcotics. PS weaning was significantly associated with fewer infusion days, lower PS volume, longer PS duration, and lower narcotics use at baseline. Alkaline phosphatase was significantly lower in weaned patients after 6 and 18 months of treatment. During the 2-year study duration, patients who had PS volume reduction of ≥20% had significantly fewer yearly hospitalizations and hospital-days. CONCLUSIONS: Teduglutide reduces PS volume and promotes weaning in adults with SBS-IF. Lack of narcotics and longer PS duration were associated with PS volume reduction and weaning, and lower baseline PS volume and fewer infusion days were favorable in obtaining enteral autonomy.

Ophthalmoplegia and leg myorhythmia in Whipple's disease: report of a case.

Whipple's disease (WD) is a rare disorder that is more common in males than in females. Progressive supranuclear ophthalmoplegia (SNO) in conjunction with oculomasticatory myorhythmia (OMM) or oculofacioskeletal myorhythmia are characteristic movement abnormalities when WD involves the nervous system. Limb myorhythmia without facial or ocular myorhythmia has not been reported in WD. We report such a case who had SNO and leg myorhythmia but no facial or ocular myorhythmia. She had onset of WD at age 28 and 16 years later developed SNO and leg myorhythmia. The neurological manifestations did not respond to antimicrobial agents or to the drugs used for parkinsonism or essential tremor. Valproate produced a remarkable improvement in leg myorhythmia, but the efficacy declined after 3 months. Because WD may infest as a neurological disorder without gastrointestinal symptoms, all SNO cases, with or without OMM, and those with skeletal myorhythmia should be suspected of WD. These patients should be treated vigorously and followed carefully since neurological involvement is the most disabling feature and it has a propensity to relapse.

Protein-losing enteropathy in systemic lupus erythematosus associated with intestinal lymphangiectasia.

This case report concerns a young woman with systemic lupus erythematosus who presented with a protein-losing enteropathy. Intestinal biopsy revealed lymphangiectasia. Mesenteric lymph nodes showed paracortical lymphoid depletion and multiple small sinusoids. Elevated cholesterol levels and normal lymphocyte counts characterized the peripheral blood samples. Lymph nodes, though enlarged, showed no evidence of obstructive pathology. No secondary cause of lymphangiectasia could be identified. The patient exhibited antinuclear antibodies and antibodies to dsDNA at the onset, and then, 5 yr later, the classic features of systemic lupus erythematosus (SLE). The lymphocytopenia and hypolipidemia that characterizes lymphangiectasia is not a feature of the SLE cases reported to date. Furthermore, the mechanism for the dilated lymphatics and villous edema is more likely immunological than mechanical disruption of lymphatics.

Pancreatitis associated with pancreas divisum: results of surgical intervention.

The embryologic defect that results when the ventral and dorsal anlages of the pancreas do not fuse has been referred to as pancreas divisum. The patients who present with recurrent pancreatitis or pancreatitis-like pain in association with pancreas divisum should be investigated to determine the presence of minor papilla stenosis. In the present study of 11 patients, 9 became asymptomatic with surgical decompression of the dorsal pancreatic duct. Two surgical failures were related to wrong diagnosis and restenosis of the sphincteroplasty. A delay in the clearance of dye from the dorsal pancreatic duct after endoscopic retrograde cholangiopancreatography was utilized to determine minor papilla stenosis. In the future, secretin ultrasound tests should prove of value in assessing minor papilla stenosis. Once the diagnosis is made, sphincteroplasty of the minor papilla is the most logical procedure because of the good results obtained. In the present study, pancreaticojejunostomy provided good duct decompression as well.

Cytomorphologic aspects of hepatic angiosarcoma. Fine needle aspiration biopsy of a case.

A case of hepatic angiosarcoma diagnosed by fine needle aspiration biopsy (FNAB) is documented. The presence of malignant endothelial cells was diagnostic of angiosarcoma. Since hepatic angiosarcoma is an extensive lesion, the chance of getting a good cell sample for cytologic diagnosis by aspiration biopsy is very high. FNAB of the liver is a safe procedure and has proven to have a high diagnostic accuracy in hepatic cancer.

Spiral filling defect in the common bile duct.

A spiral filling defect in the common bile duct was found in an elderly woman with intermittent obstructive jaundice. A large, smooth papilla was shown on hypotonic duodenogram. At laparotomy, mucus strans were found in the DUCT, and ampullary carcinoma in the duodenum. The significance of the mucus strand and large duodenal papilla is discussed.

Periampullary tumours: advances in diagnosis and surgical treatment.

Forty-five carcinomas of the region of the ampulla of Vater were resected at the Toronto General Hospital during a 16-year period. In 26 the presenting symptom was pain and in 32 it was jaundice. The most useful means of investigation was endoscopic retrograde cholangiopancreatography. Angiography was of value in determining resectability prior to operation, and percutaneous aspiration biopsy allowed a preoperative diagnosis in the case of large pancreatic tumours. In 39 patients who had a Whipple procedure the operative mortality was 8%. Total pancreatectomy performed in two patients and local excision of ampullary carcinoma in four patients were attended by no operative deaths. Long-term survival was best in patients with ampullary carcinomas and worst in those with pancreatic cancer.

Percutaneous fine needle aspiration biopsy of the pancreas following endoscopic retrograde cholangiopancreatography.

Fine needle aspiration biopsies were performed in 9 patients with suspected pancreatic malignancies demonstrated and localized by endoscopic retrograde cholangiopancreatography (ERCP). The biopsies were positive in 7 of 8 patients with pancreatic carcinoma and there were no complications. ERCP is an accurate method for diagnosing pancreatic cancer which may be confirmed safely and easily by fine needle aspiration biopsy.