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Pupillary and electroretinographic abnormalities in a family with neuronal intranuclear hyaline inclusion disease.

Abnormal pupillary function and a severely depressed electroretinogram were found in four members of a family with neuronal intranuclear hyaline inclusion disease, an idiopathic degenerative disorder that involves the central and peripheral nervous systems. Symptoms were limited to the gastrointestinal system and consisted principally of abdominal pain, constipation, and severe weight loss. The discovery of light-fixed pupils in the propositus led to the first antemortem diagnosis by rectal biopsy in two generations of this family. Abnormalities of gastrointestinal motility and pupillary reactions constituted the only objective evidence of autonomic dysfunction; the abnormal electroretinogram was the only evidence of central nervous system dysfunction.

Adult↗

Gastrointestinal regulatory peptides in systemic sclerosis.

OBJECTIVE: Gastrointestinal involvement commonly occurs in systemic sclerosis (SSc), but its pathogenesis is not well understood. Since there is evidence of a defect in neurotransmitter release, we were interested in examining the relationship between gastrointestinal dysfunction and plasma concentrations of gastrointestinal regulatory peptides in patients with SSc. We studied 43 consecutive patients, 18 with diffuse and 25 with limited cutaneous disease. METHODS: Levels of corticotropin-releasing hormone (CRH), gastrin, motilin, neuropeptide Y (NPY), and peptide YY (PYY) were determined by radioimmunoassay and high-performance liquid chromatography (HPLC). RESULTS: Plasma concentrations of CRH, motilin, NPY, and PYY were significantly increased among SSc patients compared with healthy control subjects, and HPLC-characterization of motilin, NPY, and PYY showed a different pattern of fragments. No correlation was found between esophageal hypomotility and the concentration of peptide. Acid output did not correlate with gastrin levels, but was more often increased in patients with increased CRH and NPY values. Fat malabsorption, assessed by the triolein breath test, was more common among patients with increased motilin and PYY. CONCLUSION: This study shows that elevated peptide concentrations commonly occur in patients with SSc. Since regulatory peptides are involved in gastrointestinal motility, secretion, and absorption, further characterization of this neuroendocrine system may help in understanding the complex regulation of gastrointestinal dysfunction in SSc.

Adult↗

Validation of the Rockall risk scoring system in upper gastrointestinal bleeding.

BACKGROUND: Several scoring systems have been developed to predict the risk of rebleeding or death in patients with upper gastrointestinal bleeding (UGIB). These risk scoring systems have not been validated in a new patient population outside the clinical context of the original study. AIMS: To assess internal and external validity of a simple risk scoring system recently developed by Rockall and coworkers. METHODS: Calibration and discrimination were assessed as measures of validity of the scoring system. Internal validity was assessed using an independent, but similar patient sample studied by Rockall and coworkers, after developing the scoring system (Rockall's validation sample). External validity was assessed using patients admitted to several hospitals in Amsterdam (Vreeburg's validation sample). Calibration was evaluated by a chi2 goodness of fit test, and discrimination was evaluated by calculating the area under the receiver operating characteristic (ROC) curve. RESULTS: Calibration indicated a poor fit in both validation samples for the prediction of rebleeding (p<0.0001, Vreeburg; p=0.007, Rockall), but a better fit for the prediction of mortality in both validation samples (p=0.2, Vreeburg; p=0.3, Rockall). The areas under the ROC curves were rather low in both validation samples for the prediction of rebleeding (0.61, Vreeburg; 0.70, Rockall), but higher for the prediction of mortality (0.73, Vreeburg; 0.81, Rockall). CONCLUSIONS: The risk scoring system developed by Rockall and coworkers is a clinically useful scoring system for stratifying patients with acute UGIB into high and low risk categories for mortality. For the prediction of rebleeding, however, the performance of this scoring system was unsatisfactory.

Adolescent↗

[Paraneoplastic syndromes].

Paraneoplasia has come to denote those tumor activities not caused within an organism directly by invasion, obstruction, or by the tumor burden itself. PSN research has yielded best results through the examination of those hormones produced by the tumor, esp. the pro-ACTH and its functionally active subgroups, furthermore the ADH, gonadotropins, HPL, STH, prolactine etc. Frequently very special features have been found to characterize this disease which have been published in detail. In addition to this, several differentiating PNS have been described without having been sufficiently defined, esp. those concerning the nervous system, the haematopoesia , the kidneys, the skin and the gastrointestinal system. In very rare cases there has been a mention or different characteristic syndromes that have come to be assigned to special kinds of tumors.

Central Nervous System Diseases↗

Pharmaco-morbidity linkage: a feasibility study comparing morbidity in two pharmacy based exposure cohorts.

STUDY OBJECTIVES: The aims were (1) to compare discharge diagnoses and concurrent medication in a pharmacy based cohort of users of H2 receptor antagonists to those in a population of users of other drugs in the same period, who did not use H2 receptor antagonists; (2) to compare these results to those of a similar study performed with the Tayside record linkage scheme. DESIGN AND SETTING: The study was a retrospective cohort study. The morbidity data from the only hospital in one medium sized city (62,000 inhabitants) were linked to the dispensing data of all five community pharmacies on an individual basis (April 1, 1986-December 31, 1989). In the absence of a unique patient identification number, data from pharmacies and hospital were linked by the combination of date of birth, gender, and general practitioner code. For every user of H2 receptor antagonists two controls were obtained from all patients who had not used these drugs, and matched for age (within 5 years), gender, and general practitioner. All discharge diagnoses which followed this first prescription up to December 31, 1989, in a patient in the index cohort, and during the same period in his or her matched controls, were included in the study. MAIN RESULTS: In the index cohort (n = 2174) 341 persons were admitted (526 admissions) as against 398 persons (527 admissions) in the control cohort (n = 4348). There was increased morbidity in the index cohort, especially concerning the gastrointestinal system (peptic ulcers and malignancies, abdominal pain, gastrointestinal haemorrhage), but also concerning the musculoskeletal, respiratory, and circulatory systems. The morbidity in the last three groups corresponded with drugs used concomitantly by patients in the index cohort, so it was probably not causally related to the intake of H2 receptor antagonists but was rather an indicator of higher levels of morbidity in the index cohort. CONCLUSIONS: The figures were grossly comparable to those of the Tayside record linkage scheme. Probabilistic linking with the patient characteristics of gender, date of birth, and general practitioner code can facilitate the undertaking of postmarketing surveillance studies.

Chronic Disease↗

Maturity of the neonatal foal.

The immature foal frequently represents a significant management challenge to even the most experienced clinician. The clinical course typically involves complications to a range of body systems,including the musculoskeletal, respiratory, and gastrointestinal systems. Before the commencement of treatment, it is important to provide the owner with an estimation of short-term and long-term survival, expected costs, and possible complications. Formulation of an accurate prognosis can be a difficult task but is aided by knowledge not only of normal maturation but of the factors that affect this process.

Animals↗

[Physiological changes during pregnancy relevant to anesthesia].

Hormone production from the corpus luteum and the placenta as well as the mechanical effects of the growing uterus are the main reasons for the physiological changes of pregnancy. These have relevant effects particularly on the cardiovascular system, the respiratory tract and the gastrointestinal system as well as the kidneys and urinary tract. Elective procedures on women of childbearing age should only be performed when early pregnancy has been excluded as the effects of even the latest anaesthetics at the time of organogenesis may not be fully understood. The aim of close teamwork between midwives, obstetricians, neonatologists and anaesthetists should be to prevent any complications for the mother and to give the newborn the most optimal start in it's young life.

Adult↗

Pulmonary biochemical and histological alterations after repeated low-level blast overpressure exposures.

Blast overpressure (BOP), also known as high energy impulse noise, is a damaging outcome of explosive detonations and firing of weapons. Exposure to BOP shock waves alone results in injury predominantly to the hollow organ systems such as auditory, respiratory, and gastrointestinal systems. In recent years, the hazards of BOP that once were confined to military and professional settings have become a global societal problem as terrorist bombings and armed conflicts involving both military and civilian populations increased significantly. We have previously investigated the effects of single BOP exposures at different peak pressures. In this study, we examined the effects of repeated exposure to a low-level BOP and whether the number of exposures or time after exposure would alter the injury outcome. We exposed deeply anesthetized rats to simulated BOP at 62 +/- 2 kPa peak pressure. The lungs were examined immediately after one exposure (1 + 0), or 1 h after one (1 + 1), two (2 + 1), or three (3 + 1) consecutive exposures at 3-min interval. In one group of animals, we examined the effects of repeated exposure on lung weight, methemoglobin, transferrin, antioxidants, and lipid peroxidation. In a second group, the lungs were fixed inflated at 25 cm water, sectioned, and examined histologically after one to three repeated exposures, or after one exposure at 1, 6, and 24 h. We found that single BOP exposure causes notable changes after 1 h, and that repeating BOP exposure did not add markedly to the effect of the first one. However, the effects increased significantly with time from 1 to 24 h. These observations have biological and occupational implications, and emphasize the need for protection from low-level BOP, and for prompt treatment within the first hour following BOP exposure.

Air Pressure↗

Recombination aneusomy of chromosome 5 associated with multiple severe congenital malformations.

A male infant is described in whom congenital anomalies were recognized prenatally by ultrasound examination. The infant was delivered following spontaneous labor and died approximately 15 min after birth. An autopsy revealed major anomalies in the central nervous system (holoprosencephaly with premaxillary agenesis), the gastrointestinal system (esophageal atresia) and the heart (tetralogy of Fallot). Chromosomal studies revealed recombinant chromosome 5 [46,XY, rec(5), dup q, inv(5)(p15q32)], resulting in partial trisomy 5q and partial monosomy 5p. Cytogenetic investigation of the family revealed a pericentric inversion of chromosome 5 in the father and paternal grandmother, 46,XY (and XX, respectively,) inv(5)(p15q32). The congenital anomalies in this infant are more extensive and severe than previously reported in cases of recombination aneusomy involving chromosome 5.

Abnormalities, Multiple↗

Cholecystokinin receptor family. Molecular cloning, structure, and functional expression in rat, guinea pig, and human.

A review of the literature encompassing numerous pharmacological, physiological, and biochemical studies indicates the presence of at least four CCK receptor types, CCKA, CCKB, gastrin, and CG-4 receptors. Multiple subtypes of the CCKAR have been postulated to account for the differences in pharmacology or affinity cross-linking of CCKARs between pancreas and gallbladder and the presence of high and low affinity CCKARs on pancreatic acini. Multiple subtypes of the CCKBR have been postulated to explain the differences in pharmacology and physiology between gastric and gallbladder smooth muscle CCKBRs. We recently cloned and functionally expressed both the CCKAR and the CCKBR from rat, guinea pig, and human. The CCKAR and CCKBR are 48% homologous and constitute a family of receptors within the guanine nucleotide-binding regulatory protein-coupled superfamily of receptors. Each receptor is highly conserved between species. A single cDNA encoding a single protein is present in both pancreas and gallbladder and can account for both high and low affinity CCKARs found on pancreatic acini when transfected into COS-7 cells. A single cDNA encoding a single CCKBR protein is present in both the central nervous system and the periphery including the gastrointestinal system. Therefore, the gastrin receptor is actually a CCKBR present on parietal cells. Genomic and cDNA library hybridization as well as Northern and Southern hybridization studies among rat, guinea pig, and human species identifies only two members of the CCK receptor family, CCKAR and CCKBR. Although these studies do not identify other closely related members of the CCK receptor family, they do not rule out the existence of other less closely related members. Furthermore, differences in tissue and species-specific posttranslational processing, receptor coupling, and associated membrane protein and lipid heterogeneity may be among some of the other factors that may account for the phenotypic expression of more receptor subtypes than molecular studies would predict.

Amino Acid Sequence↗

Management of food allergies.

Worldwide, approximately 8 and 2% of children and adults, respectively, suffer from food allergy. Cow's milk, egg, peanut, soy, wheat, fish, shellfish and tree nuts are responsible for the majority of allergic reactions to foods. Allergic reactions to food can occur by a variety of immune mechanisms including: IgE-mediated; non-IgE-mediated (T-cell-mediated); and combined IgE- and T-cell-mediated. Food allergies can affect any organ system, but most frequently involve the gastrointestinal system, the skin and the respiratory system. Knowledge of the spectrum of food allergies is important in order to identify patients at risk for severe or life-threatening allergic reactions. This article will review the mechanisms of specific food allergy disorders. It will also summarise the diagnosis of food allergy including the history of a food reaction, skin tests and laboratory tests. The management of food allergy will also be discussed with particular emphasis on the avoidance of food allergens and the pharmacotherapy of allergic reactions. Future therapy for food allergies will also be discussed.

Adolescent↗

[The dynamics and structure of neonatal mortality in Bulgaria in the period of 1989-1998].

AIM OF THE STUDY: To follow and analyse the state and the structure of neonatal mortality rate (NMR) in the last ten years in Bulgaria. MATERIAL AND METHODS: A retrospective study is fulfilled on the annual data of the National Health Statistic Institute concerning the NMR in a period of last ten years. Along to this the changes in the perinatal infant mortality rate (PIMR) including the stillbirth and the early NMR are studied. The results are analysed by a comparative method and presented graphically. RESULTS: The unfavorable trends in the social and economic development of Bulgaria in the last decade are associated with a constant increase of PIMR--from 10.8% in 1989 to 12.9% in 1998, and of its components respectively: the stillbirth--from 5.9% to 7.4% and of the early NMR--from 4.9% to 5.8%. The NMR has increased from 7.3% since 1989 to 8.3% in 1998, the highest level being in 1991--10.4%. Besides the level of prematurely has increased constantly for the recent ten years--from 6.5% to 9.6%. The leading causes of neonatal mortality in Bulgaria are: perinatal asphyxia, congenital malformations, neonatal respiratory distress syndrome (RDS) and perinatal infections. They have remained the same for the recent five years. From them only the mortality caused by perinatal asphyxia has decreased in 1998--to 41.3% in comparison with 1994--43.7% and by congenital malformations--from 29.4% to 23.4% in 1998, the highest reduction being of the malformations of the neural system (2 folds) and of the gastrointestinal system (5 folds). The NMR of the low-birth weight infants (birth weight < 2500 g) has been 58.1% in 1997 and of the very low birth weight infants (< 1500 g)--276.3%. The NMR of both groups premature infants has decreased in 1998, respectively--to 47.6% and 191.4%. The NMR of the term-born infants has been 13.2% in 1997 and 11.1% in 1998. CONCLUSIONS: The NMR in Bulgaria remains comparatively high, the larger part being on the account of the early NMR, and it has increased for the last ten years. The main causes of NMR have remained the same for the last five years in the leading place been the perinatal asphyxia, the congenital malformations and the neonatal RDS. There is a tendency to a decrease of the perinatal asphyxia and the congenital malformations as a course of NMR in the last five tears. The NMR of the premature infants has decreased in 1998 and is relatively low, but this may be due to the fact that a certain part of the deceased extremely low-birth weight infants (< 1000 g) has been still not registered.

Birth Rate↗

[Gastroenterologic endocrinology: basic aspects].

Gastrointestinal cells produce several substances which act as messengers in the various biochemical processes of the body. These substances are released by endocrine, paracrine and neurocrine mechanisms. Both, hormonal and neuronal phenomenaregulate the absorptive, secretory and motility activity of the gastrointestinal tract. This paper goes over basic and general aspects of hormones secreted in the gastrointestinal system in order to make easier the understanding of their influence in the health and disease.

Digestive System Physiological Phenomena↗

[Clinicopathologic analysis of 141 cases of metastatic carcinoma in bone].

OBJECTIVE: To study the clinicopathologic features of metastatic carcinoma in bone and to evaluate the role of immunohistochemistry in delineation of possible primary sites. METHODS: One hundred and forty-one cases of metastatic carcinoma in bone encountered during the period from 1998 to 2004 in People's Hospital, Peking University, were reviewed retrospectively. The clinical information, radiographic features and pathologic findings were analyzed. Immunohistochemical study for antigens including cytokeratins, prostatic specific antigen, thyroglobulin, thyroid transcription factor 1 and gross cystic disease fluid protein 15, was performed in 51 cases possessing skeletal metastasis with unknown primary. RESULTS: Skeletal metastasis occurred more commonly in males (male to female ratio = 1.7:1). The age of patients ranged from 23 to 86 years (mean age = 56.5). The presenting symptoms included pain and dysfunction in the affected bones. The locations of skeletal metastasis were as follows: spine (58), pelvic bone (46), long bone (34) and others (3). Twenty-three cases harbored multiple bony lesions. Radiographically, 99 cases (70.2%) of skeletal metastasis were detected by X-rays, including 85 cases (85.9%) showing lytic changes. The primary sites of the tumor could be determined by clinicopathologic correlation in 90 cases (63.8%) and were unknown in the remaining 51 cases. Upon immunohistochemical study, the primary sites were determined in another 40 cases. Overall, the primary sites were identified in 130 cases (92.2%), which included lung (37), female genital system and breast (25), kidney (18), gastrointestinal system (17), liver (12), thyroid (11), prostate (7), bladder (2) and skin (1). CONCLUSIONS: Skeletal metastasis occurs more often in elderly males. Axial bones (spine and pelvis) are usually affected. Lung and female genital system are frequent the primary sites. Immunohistochemical study is useful in cases with occult primary.

Adult↗

[Gastroenterologic manifestations of acquired immunodeficiency syndrome].

The gastrointestinal system is a target organ for many infection in the immunosuppressed. Alterations in mucosal immunity may explain the increased incidence of enteric infections, abnormalities are most marked by a decrease in total T cells, CD4 T cells and IgA producing cells. Such involvement of the gastrointestinal tract contributes to nutritional problems and may provide a portal entry for systemic infection. Gastrointestinal manifestation of patients with AIDS has been found in 78% of patients in Perú; chronic debilitating diarrhea is commonly. These immune deficits predispone such patients to rare tumors; malignant neoplasia complicate AIDS in 12% of the cases in USA, most common was Kaposi sarcoma followed by Lymphoma. Liver abnormalities are common, those related to the opportunistic infections, chronic debilitating illness and concident exposure to hepatotropic viruses. Disinfection of the endoscopic instruments is very important; glutaraldehydes can be used in fiberoptic instruments and their accessories. This report reviews the clinical problems, diagnosis and treatment in patients with AIDS and gastrointestinal manifestation.

Acquired Immunodeficiency Syndrome↗

An immunohistochemical study on the neuroendocrine system in the alimentary canal of the brown trout, Salmo trutta, L., 1758.

Several neurohormonal peptides of the gastrointestinal system of fish have been revealed by immunohistochemical methods. Among salmonids, the rainbow trout, Oncorhynchus mykiss (Walbaum) is the most studied species, whereas the informations about other species of the taxonomic group are lacking. The regional distribution and relative densities of cells belonging to the neuroendocrine system have been in this paper demonstrated in the gut of the brown trout, Salmo trutta Linnaeus. In the gastric mucosa, endocrine cells were detected, which were immunoreactive to bombesin-, gastrin-, and secretin-antisera. Endocrine cells containing gastrin-, bombesin-, cholecystokinin-8-, glucagon-, and leptin-like immunoreactivities were present in the pyloric caeca and intestine. The pancreatic endocrine islets contained glucagon-, and, possibly, secretin-like-immunoreactive endocrine cells, as well as a contingent of galanin-like-immunoreactive nerve fibres. The exocrine pancreatic parenchyma showed bombesin-like-immunoreactive nerve fibres. Within the tested regulatory peptides, bombesin and leptin were observed in both endocrine cells and nerve cell bodies and fibres. Leptin was in addition detected in epithelial cells of the gastric glands. In the brown trout we have never observed any immunoreactivity to the VIP antiserum (either in the stomach or in the intestine). Some special structural patterns (in particular those ones related to galanin- and leptin-immunohistochemical data) have thus been detected for the first time in the brown trout, and provide further data for a better knowledge of gut morpho-functional aspects in this economically important fish.

Animals↗

Pharmacodynamic effects of Sandostatin in the gastrointestinal tract.

Somatostatin is widely distributed throughout the human gastrointestinal system. There it is found in neurons and fibers of both the submucosal and the myenteric plexus and the pancreas as well as in the D cells of the stomach, gut and pancreatic islets. Whereas in the intestinal nervous system, in the duodenum and the pancreas, somatostatin-14 appears to be the predominant molecular form, the endocrine-type D cells of the intestine primarily contain somatostatin-28. Somatostatin peptides may act very differently at different sites, as hormones, as paracrine substances or neurotransmitters. Because of this complexity of action, very little is known about the physiological effects of somatostatin in the gastrointestinal tract. In contrast, the pharmacological actions of natural synthetic somatostatin have been thoroughly studied and have given rise to many therapeutic applications. Octreotide, an analogue with a longer half-life and higher potency, has greatly facilitated the clinical application of somatostatin. This review deals with the pharmacological effects of octreotide on different gastrointestinal functions. The somatostatin analogue exerts a long-lasting inhibitory action on gastric acid, pancreatic enzyme and bicarbonate secretion as well as on bile flow. It is also able to inhibit stimulated intestinal secretion, the release of neuropeptides from the gut and the pancreas. It can also prolong orocecum transit time and prevent gallbladder contraction. It inhibits absorption of nutrients and exerts inhibitory effects on splanchnic hemodynamics. It is because of these actions that somatostatin has attracted so much attention in the treatment of different gastrointestinal disorders.

Digestive System↗

Pharmacological studies with the alpha 2-adrenoceptor antagonist midaglizole. Part III: Smooth muscle, gastrointestinal and miscellaneous systems.

Pharmacological properties of midaglizole (DG-5128, CAS 66529-17-7) on smooth muscle, gastrointestinal and miscellaneous systems were investigated in comparison with those of tolbutamide. 1. Isolated smooth muscle: Midaglizole inhibited the contractile responses of the ileum to nicotine, acetylcholine, serotonin and BaCl2, and especially the response to histamine (guinea pig). Tolbutamide also reduced the contractions. On spontaneous motility of the uterus (estrus, diestrus, pregnant), midaglizole produced an increase in tonus and frequency, and decrease in contractile amplitude (rat). Tolbutamide reduced the motility of the uterus. The contractile response of the vas deferens to norepinephrine was potentiated after midaglizole and tolbutamide (guinea pig). Midaglizole inhibited the contractile response of the trachea to histamine, whereas it had little or no effect on the response to acetylcholine (guinea pig). Tolbutamide reduced the responses to both spasmogens. 2. Gastrointestinal system: Midaglizole induced an increase in gastrointestinal transit (mouse) and potentiated the gastrointestinal motility (dog). Tolbutamide was without any effect on these parameters. Gastric emptying rate, gastric secretion and gastric mucosa were not influenced after midaglizole (rat). Tolbutamide decreased gastric secretion and produced injury of gastric mucosa. 3. Urine volume: Midaglizole and tolbutamide showed a diuretic activity (rat). 4. Anti-inflammatory activity: Midaglizole and tolbutamide inhibited carrageenin-induced paw edema (rat).

Adrenergic alpha-Antagonists↗