BIB induces a temporary sense of satiety in morbidly obese patients which is not mediated by modification of fasting or postprandial levels of plasma ghrelin.

Mitochondrial neurogastrointestinal encephalopathy (MNGIE) is an autosomal recessive multisystem disorder caused by mutations in the thymidine phosphorylase gene (TP). 1 The disease is characterized by onset between the first and fifth decades of life, ptosis, progressive external ophthalmoparesis, peripheral neuropathy, and leukoencephalopathy. 1,2 Gastrointestinal (GI) involvement is a major hallmark of the disease, and all reported patients have GI dysmotility. [2][3][4] Case report. We report a 60-year-old woman born after a normal pregnancy. She had bilateral ptosis since childhood and tremor affecting hands and head since age 2 years. She was 145 cm tall and weighed 41 kg at age 60 years. Neurologic examination at age 60 years showed bilateral ptosis with bilateral external ophthalmoplegia. Optic fundi were normal without retinitis. Strength was normal, but she had areflexia. She also had action and rest tremor. Her maternal grandfather and her daughter had tremor. Echocardiography revealed a mild thickening in the septum heart muscle. She had no GI symptoms. A radionuclide gastric emptying test showed mild gastroparesis. Laboratory data showed elevated venous lactate at rest (4.1 mmol/L; normal, 0.9 to 2.7). Brain MRI revealed diffuse hyperintense signal affecting the white matter of the cerebral hemispheres, midbrain, and pons. Abundant cytochrome c oxidase (COX)-negative ragged-red fibers and lipid droplets were found in biceps muscle biopsy (figure). The patient's sister had short stature, and the patient's daughter had tremor, but no major criteria of MNGIE were observed in either.Muscle respiratory chain enzyme activities were decreased, in particular COX activity (patient, 17; control mean, 41 IU/CS). Southern blot analysis failed to reveal mitochondrial DNA (mtDNA) deletions or mtDNA depletion (see figure). Biochemical analysis from peripheral blood showed negligible thymidine phosphorylase activity in buffy coat (patient, 4.4; control range, 495 to 719 nmol of thymine formed/h/mg protein) and high plasma levels of thymidine (patient, 9.0; control, Ͻ0.05 mol/L) and deoxyuridine (patient, 17.7; control, Ͻ0.05 mol/L). 5 Sequencing analysis of the TP gene 1 revealed the presence of four heterozygous nucleotide changes from the consensus sequence (GenBank no. NM_001953; see figure): a c.228GϾA substitution in exon 3 resulting in an M76I change; a c.847CϾG transversion in exon 7 resulting in an H283D missense mutation; a c.1311GϾA transition in Figure. 1) Consecutive cross-sections of patient's muscle biopsy stained for succinate dehydrogenase (A) and for cytochrome c oxidase (COX; B) showing the presence of ragged-red (A) and COXnegative fibers (B); magnification ϫ400. 2) Macroscopic mitochondrial DNA (mtDNA) lesion analysis. (A) Southern blot analysis showing a DNA molecularweight marker (lane 1), the patient's wild-type band corresponding to muscle mtDNA (lane 2), and the mtDNA from a muscle normal control (lane 3). (B) Equimolar two-probe Southern blot analysis to detect mtDNA depletion (top band, 16...

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The number of papers involved is high, but their accessibility is limited. Evidence is overall scarce, but high in cases such as methylene blue for Barrett s esophagus, lugol in the detection of esophageal carcinoma, and indigo carmine for colonic hyperplastic polyp differentiation. Drug compounding is rather simple, but scarcely developed.

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