Two cases of Hermansky-Pudlak syndrome with interstitial pneumonia

Two cases of Hermansky-Pudlak syndrome with interstitial pneumonia were reported. Both patients had evidence of pulmonary involvement characterized by diffuse bilateral infiltrates. They had oculocutaneous albinism. Case 1 was a 55 year-old female who had a history of easy bruising. Her two sisters were albino and had died of pulmonary fibrosis. One of them was diagnosed as Hermansky-Pudlak syndrome on autopsy. Bone marrow aspirate disclosed typical macrophages with ceroid-like pigment. Transbronchial lung biopsy showed alveolar wall thickening. Lumi-aggregometer showed a decrease of platelet aggregation and an absence of ATP release. Case 2 was a 43 year-old female and had a bleeding tendency during a surgical procedure. Lumi-aggregometer showed normal platelet aggregation but an absence of ATP release. BALF analysis did not disclose macrophages with ceroid-like pigment.     

Pancreatic exocrine secretion and plasma gut hormones response after intraduodenal infusion of elemental diet in patients with chronic pancreatitis

We studied the responses of pancreatic polypeptide (PP) and cholecystokinin (CCK) using specific RIAs, and simultaneously exocrine pancreatic secretion and gall bladder contraction were checked by using triple lumen tube to intraduodenal ingestion of 100 Kcal/hr semi-digested liquid meal: Clinimeal (Eisai, Tokyo) or Elemental Diet: ED (Morishita, Osaka) in 10 patients with chronic pancreatitis (CP). Intraduodenal infusion of Clinimeal did not result in a significant physiological rise of CCK and PP from the basal values. Pancreatic secretions (volume and bicarbonate output) were slightly increased paralleled to the gall bladder contraction in chronic pancreatitis. On the other hand, intraduodenal ED can significantly stimulate the release of CCK from the small intestine and PP from the pancreas with the near range of physiological concentration. This level of CCK can evoke a significant increase in pancreatic secretion and gall bladder contraction. These results suggest that in CP the physiological regulation was disturbed and pancreatic secretion was not observed after ordinary meal ingestion. Infusion of ED which contained similar components of digestive product partially improved the responses of gut hormones and pancreatic secretion. Therefore, impaired gut hormone release in CP primarily is due to the inappropriate stimuli because of pancreatic exocrine dysfunction, and not other factors(s).     

Marked hypervascularity and early arteriovenous shunting with portal vein filling in chronic relapsing pancreatitis

Angiography was performed in 2 patients with chronic relapsing pancreatitis. Marked hypervascularity and early arteriovenous shunting with portal vein filling were demonstrated. Increased capillary vessels in the thickened capsule due to inflammation were observed in the pathologic specimen. The angiographic findings we report here are quite similar to those of pancreatic arteriovenous malformation.     

Bilateral macular colobomas in Leber's congenital amaurosis

Two siblings with Leber's congenital amaurosis had bilateral macular colobomas, nystagmus, extinguished ERGs, and degenerative salt and pepper like changes in the fundus. They had non-recordable or non-meaningful visually evoked cortical potentials in response to both flash and pattern stimuli. The ophthalmic conditions were thought to be inherited as an autosomal recessive trait.Abbreviations ERG electroretinogram - VECP visually evoked cortical potential     

Two types of normal human breast epithelial cells derived from reduction mammoplasty: phenotypic characterization and response to SV40 transfection

A culture method to grow two morphologically distinguishablenormal human breast epithelial cell types derived from reductionmammoplasty has been developed. Type I cells were characterizedby a more variable cell shape, smooth cell colony boundaries,the expression of epithelial membrane antigen (EMA) and keratin18 and the non-expression of keratin 14 and     

Intra–Arterial Infusion Therapy Following Alteration of Pelvic Blood Flow and Concurrent Radiation Therapy for Invasive Bladder Cancer

Intra–arterial infusion therapy following alteration of pelvic blood flow and concurrent radiation therapy was performed in 13 patients with muscle invading bladder cancer (T2, 2; T3, 6; T4, 5). The internal iliac artery of the opposite side was embolized and the ipsilateral gluteal and obturator arteries were embolized by metallic coils. A catheter was placed in the ipsilateral internal iliac artery. CDDP was administered daily at a dose of 7–9 mg/body over 1 minute. Radiation was done by Microtron using 10 MV x–ray. Total dose was 4500–7060 cGy.
Evaluation was done by cystoscopy, radiography and biopsy. Eight patients achieved complete response (CR) histologically. Others had partial response (PR). All CR patients had no recurrence. The observation period was between 3 and 29 months, with a mean of 11 months. This treatment modality is effective for locally advanced bladder cancer.     

Effects of continuous infusion of nitroglycerin on pulmonary hemodynamics, lung lymph balance, and prostanoid products in the response to endotoxin in awake sheep

We examined the effects of continuous intravenous infusion of nitroglycerin (NTG) on lung dysfunction induced by endotoxemia in awake sheep chronically instrumented with lung lymph fistula. We measured the responses of hemodynamics, lung lymph balance, and thromboxane (Tx)B₂ and 6-keto-prostaglandin (PG) F1α levels in plasma and lung lymph to endotoxin administration (1 μg/kg, intravenously [IV], over 30 min) with and without continuous infusion of NTG (1 μg/kg/min). Continuous infusion of NTG alone (n=5) over 5 hr did not significantly alter systemic, pulmonary hemodynamics, and/or lung lymph fluid filtration. Infusion of endotoxin alone (n=7) caused remarkable increases in pulmonary artery pressure (Ppa) and lung lymph flow ( Qlym) in the early phase. Continuous infusion of NTG (n=6) significantly prevented the early increases in Ppa and Qlym after endotoxin. The increased values of TxB₂ and 6-keto-PGF_1α in both plasma and lung lymph after endotoxemia showed the same increases in groups with and without NTG. These findings suggest that the reduction of pulmonary artery pressure induced by NTG decreased the filtration of fluid into the lungs associated with endotoxemia in sheep, and that the mechanism of vasodilating action of NTG is not due to modifications of constrictive-dilated cyclo-oxygenase products of arachidonate, such as TxA₂ and PGI₂.