SEA BLUE HISTIOCYTE SYNDROME

Three children with congenital form of sea blue histiocyte syndrome are reported. Two of them had liver involvement and the third one was mentally retarded. In all these cases, marked growth retardation was documented and the two had frequent epistaxis. The diagnosis was made by the demonstration of sea blue histiocytes in the bone marrow. These cells were also shown in the liver and spleen needle biopsy specimens, whichever was obtained.     

Metastatic liver tumors

The liver is a common site of metastasis from a variety of tumors. In many cases, liver resection for metastatic cancer provides the only chance for a cure and can be performed with less than 5% mortality and acceptable morbidity. The 5-year survival following liver resection for colorectal metastasis is reported in many large series to be 25% to 37%. The data regarding liver resection for other metastatic tumor types are less clear. However, resection for selected tumors, such as neuroendocrine and renal cell, can provide durable palliation and/or cure. We will review important prognostic factors used to guide the selection of patients for resection of metastatic disease and make recommendations for imaging studies and follow-up routines. The role of adjuvant regional and systemic chemotherapy for resectable metastatic disease is also discussed. Methods for ablating unresectable metastatic tumors may prove to be useful adjuncts to current therapies.     

Epinephrine produces a beta-adrenergic receptor-mediated mechanical hyperalgesia and in vitro sensitization of rat nociceptors

Hyperalgesic and nociceptor sensitizing effects mediated by the beta-adrenergic receptor were evaluated in the rat. Intradermal injection of epinephrine, the major endogenous ligand for the beta-adrenergic receptor, into the dorsum of the hindpaw of the rat produced a dose-dependent mechanical hyperalgesia, quantified by the Randall-Selitto paw-withdrawal test. Epinephrine-induced hyperalgesia was attenuated significantly by intradermal pretreatment with propranolol, a beta-adrenergic receptor antagonist, but not by phentolamine, an alpha-adrenergic receptor antagonist. Epinephrine-induced hyperalgesia developed rapidly; it was statistically significant by 2 min after injection, reached a maximum effect within 5 min, and lasted 2 h. Injection of a more beta-adrenergic receptor-selective agonist, isoproterenol, also produced dose-dependent hyperalgesia, which was attenuated by propranolol but not phentolamine. Epinephrine-induced hyperalgesia was not affected by indomethacin, an inhibitor of cyclo-oxygenase, or by surgical sympathectomy. It was attenuated significantly by inhibitors of the adenosine 3',5'-cyclic monophosphate signaling pathway (the adenylyl cyclase inhibitor, SQ 22536, and the protein kinase A inhibitors, Rp-adenosine 3',5'-cyclic monophosphate and WIPTIDE), inhibitors of the protein kinase C signaling pathway (chelerythrine and bisindolylmaleimide) and a mu-opioid receptor agonist DAMGO ([D-Ala2,N-Me-Phe4,Gly5-ol]-enkephalin). Consistent with the hypothesis that epinephrine produces hyperalgesia by a direct action on primary afferent nociceptors, it was found to sensitize small-diameter dorsal root ganglion neurons in culture, i. e., to produce an increase in number of spikes and a decrease in latency to firing during a ramped depolarizing stimulus. These effects were blocked by propranolol. Furthermore epinephrine, like several other direct-acting hyperalgesic agents, caused a potentiation of tetrodotoxin-resistant sodium current, an effect that was abolished by Rp-adenosine 3',5'-cyclic monophosphate and significantly attenuated by bisindolylmaleimide. Isoproterenol also potentiated tetrodotoxin-resistant sodium current. In conclusion, epinephrine produces cutaneous mechanical hyperalgesia and sensitizes cultured dorsal root ganglion neurons in the absence of nerve injury via an action at a beta-adrenergic receptor. These effects of epinephrine are mediated by both the protein kinase A and protein kinase C second-messenger pathways.     

Comparison of Infasurf (calf lung surfactant extract) to Survanta (Beractant) in the treatment and prevention of respiratory distress syndrome

OBJECTIVE: To compare the relative safety and efficacy of Infasurf (calf lung surfactant extract; ONY, Inc, Amherst, NY, IND #27169) versus Survanta (Beractant, Ross Laboratories, Columbus, OH) in reducing the acute severity of respiratory distress syndrome (RDS) when given at birth and to infants with established RDS. DESIGN: A prospective, randomized, double-blind, multicenter clinical trial. SETTING: Thirteen neonatal intensive care units participated in the treatment arm: seven of these concurrently participated in the prevention arm. PATIENTS: The treatment arm enrolled infants of 相似文献   

Extrahepatic portal venous obstruction in childhood: Etiology,clinical and laboratory findings and prognosis of 34 patients

Extrahepatic obstruction of the portal vein is a well known cause of portal hypertension in childhood, that causes severe morbidity. We evaluated 34 children (24 boys, 10 girls, age 4.5 months to 12 years, mean 5.5 ± 3.8 years) with this diagnosis, to define the clinical picture, laboratory changes, diagnostic tools and therapeutic modalities. Gastrointestinal bleeding was the commonest mode of presentation (64.7%), with the second being splenomegaly. The cause of the obstruction could be determined in 38.2% (13/34) of the subjects. At the beginning of the study the main diagnostic procedure was splenoportography although in more recent years pulsed duplex Doppler ultrasonography has been used. The follow up period was a median of 5 years (range 1–11 years). The mean number of bleeding episodes was 4.7 ± 5.9 (range 1–26), while nine patients never bled. There was no mortality. Ten patients underwent surgery, while sclerotherapy was performed on 10. Twenty-one patients received beta-blocker drugs. No difference was found among these therapeutic modalities. It is well established that the major risk for children with extrahepatic portal vein obstruction is gastrointestinal bleeding which is tolerated quite well. Surgery should be indicated only in children where bleeding cannot be controlled by medical means including sclerotherapy.     

MECKEL'S DIVERTICULUM – REVISITED

Twenty five cases of Meckel''s diverticulum were studied between 1985-1995. Eight of these were symptomatic and in the remaining 17 it was an incidental finding. The symptomatic patients presented with intestinal obstruction (5 cases), perforated peritonitis (2 cases) and intussusception (1 case). All cases of acute appendicitis were also subjected to a search for Meckel''s diverticulum. Of the 25 Meckel''s diverticuli encountered, 22 were resected and in 3 patients it was left in situ. Both the patients with perforated Meckel''s diverticulum showed ectopic gastric mucosa. Complications occurred only after surgery for symptomatic Meckel''s diverticulum. All patients undergoing incidental diverticulectomy had a smooth and uncomplicated recovery.KEYWORDS: Incidental diverticulectomy, Meckel''s diverticulum     

The NHLBI workshop on Hypertension in Hispanic Americans,Native Americans,and Asian/Pacific Islander Americans.

In June 1994, the National Heart, Lung, and Blood Institute held a workshop entitled "Epidemiology of Hypertension in Hispanic Americans, Native Americans, and Asian/Pacific Islander Americans." The studies that served as the basis for the workshop along with a summary of two workshop panel discussions are being published as a supplement by Public Health Reports. In this article, the authors present graphs that compare results across these studies with data for non-Hispanic whites, blacks, and Hispanics from the Third National Health and Nutrition Examination Survey. The graphs indicate differences in mean blood pressure levels within and among these three population groups; such differences are also apparent in comparisons of these groups with the U.S. white and black populations. Although they appear modest, these differences are sufficient to result in increased mortality rates in populations with higher levels of hypertension. Environmental influences appear to underlie most of these differences. In all of these populations, blood pressure control rates are poor. Based on these studies, hypertension prevention and control programs should be undertaken. Special emphasis should be placed on the underserved minority populations that were the focus of the workshop.     

Genetic studies of temperature-sensitive mutants of Moloney-murine leukemia virus

Genetic studies were carried out on three spontaneous temperature-sensitive (ts) mutants of Moloney murine leukemia virus (Mol-MuLV). These three ts mutants were assigned to two complementation groups with ts 1 and ts 2 in group A and ts 3 in group B. A high frequency of wild-type recombinants (about 8%) was obtained from cells mixedly infected with ts mutants of different complementation groups.     

Prospective investigation of complementary and alternative medicine use and subsequent hospitalizations

Background  

The prevalence of complementary and alternative medicine (CAM) use has been estimated to be as high as 65% in some populations. However, there has been little objective research into the possible risks or benefits of unmanaged CAM therapies.