A 71-year-old male suffering from an intraductal papillary tumor of the pancreas was admitted to our hospital for further investigation. Diagnostic trials, including endoscopic retrograde pancreatography, did not produce an adequate ductography because of a large amount of mucinous fluid. Therefore, we performed endoscopic ultrasonographic-guided punctured pancreatic ductography (EPPD). This procedure was safely performed without any complications. We report this initial and successful trial of EPPD.

Aged↗

PubMedDate not supplied

Decreased 6-keto prostaglandin F1 alpha (6-keto PGF1 alpha) in patients with Lesch-Nyhan syndrome.

The coagulation abnormality in patients with Lesch-Nyhan syndrome (LNS) prompted us to examine 6-keto prostaglandin F1 alpha (6-keto PGF1 alpha), a stable metabolite of prostacyclin (PGI2). Plasma levels of 6-keto PGF1 alpha were significantly low in 4 patients with LNS, but they were elevated after discontinuation of allopurinol. Other indicators of coagulation and fibrinolysis systems did not change after the discontinuation of allopurinol. PGI2 prevents the production of superoxide which is formed after cerebral ischemia. The potential source of superoxide is xanthine oxidase which is inhibited by allopurinol. It is assumed that plasma PGI2 increased in response to formed superoxide because xanthine oxidase inhibition was abolished after discontinuation of allopurinol.

6-Ketoprostaglandin F1 alpha↗

PubMedDate not supplied

Short latency somatosensory evoked potentials and 99mTc-HMPAO SPECT in a case of flunarizine-effective alternating hemiplegia in infancy.

Short latency somatosensory evoked potentials (SSEPs) and 99mTc-hexamethylpropylene amine oxime single photon emission computed tomography (99mTc-HMPAO SPECT) were examined in a patient with alternating hemiplegia in infancy (AHI) before and after flunarizine treatment. The low amplitude and elongation of the latency time at the C'4 level of the interictal SSEPs before treatment were ameliorated by flunarizine administration. The cerebral hypoperfusion in the right hemisphere observed on 99mTc-HMPAO SPECT before treatment improved, as seen by normal and symmetrical imaging, after flunarizine treatment. These observations suggest that AHI is caused by a functional disturbance in the cerebral area contralateral to the hemiplegia, and that SSEPs and 99mTc-HMPAO SPECT are useful for evaluating functional disturbance in the contralateral motoneurons and the effect of flunarizine treatment in AHI patients.

Evoked Potentials, Somatosensory↗

PubMedDate not supplied

Vascular involvement in benign infantile mitochondrial myopathy caused by reversible cytochrome c oxidase deficiency.

A 1-month-old Japanese girl had profound generalized weakness, hypotonia, and severe lactic acidosis. The infant improved gradually: she held her head at 9 months, learned to walk by 15 months. At the first muscle biopsy at 11 weeks of age, the specimen was characterized by numerous ragged-red fibers and decreased enzyme activity on cytochrome c oxidase (COX) staining. Electron microscopic findings were characterized by the presence of excessive abnormal mitochondria not only in skeletal muscle fibers but also in blood vessels. Vascular abnormalities consisted of an increased number of enlarged mitochondria in endothelial and smooth muscle cells of small arteries. Biochemical analysis showed an isolated defect of COX activity, which was only 16% of the mean control level. At the second biopsy at 44 months of age, the COX activity had increased to normal in the entire specimen. On electron microscopy, the abnormal mitochondria present on the first biopsy specimen had disappeared both in muscle fibers and blood vessels; nearly all mitochondria were morphologically normal at the second biopsy. Now at 5 years of age she can run and does not show muscle weakness. We report reversibility of abnormal mitochondria with age not only in skeletal muscle fibers but also in blood vessels in a patient, who had reversible COX deficiency with a benign clinical course.

Arteries↗

PubMedDate not supplied

Renovascular hypertension: a case of intimal fibroplasia with congenital anomaly of the renal artery.

We report a case of renovascular hypertension caused by fibromuscular dysplasia (intimal fibroplasia) with congenital anomaly of the renal artery. Arteriography revealed complete occlusion from the root to the middle of the right renal artery and a collateral artery from the right lumbar artery. Histological examination showed intimal fibroplasia and three arterioles in the intima of the renal artery.

Adult↗