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[Retrospective study of anorexia nervosa: reduced mortality and stable recovery rates].

OBJECTIVES: Anorexia nervosa is an eating disorder that combines malnutrition, amenorrhea, and distorted body image. To learn more about the course of this disease we undertook a retrospective study of girls diagnosed with anorexia nervosa in the Saint Etienne Endocrinology Department between 1979 and 2004. METHODS: Patients were diagnosed according to DSMIV criteria. Data collected to complete the Morgan-Russell outcome assessment schedule included chronology of illness, patients' morphological features, anorexia type, treatment choice, patient's gynecological history, and social status. RESULTS: The study included 206 cases. The average follow-up period was 8.3 +/- 5.3 years. Defining recovery as stable BMI>17.5 kg/m2 for at least one year and recovery of normal menstruation, full recovery was observed in 55.8% and partial recovery in 25.7%, while 18.5% remained chronically ill. Early onset (i.e., during adolescence) was associated with good prognosis, and advanced emaciation and delayed or insufficient medical care with poor prognosis. CONCLUSIONS: The seriousness of this disease is due more to the incidence of cases that become chronic than to the mortality rate. Prediction of severity would be improved by taking into account underlying personality traits, such as addictive tendencies and depression.

Adolescent↗

[Anorexia nervosa and severe malnutrition: nutritional practice guidelines in pediatrics].

The nutritional care of a severely emaciated anorexic adolescent can be performed in any pediatric ward. This article is a practical guide which reminds the indications, mostly clinical, for hospitalization and the way to achieve a progressive but efficient renutrition. We wish to share our own practical experience while focusing on acute nutritional care, according to the aim of this section of the journal.

Adolescent↗

Insulin action on protein metabolism.

On the basis of the preceding observations, the following sequence of events can be postulated during insulin deficiency or excess. The main feature of insulin deficiency is the disruption of protein balance in muscle that rapidly leads to emaciation and wasting. Muscle protein degradation is greatly enhanced while increased amino acid availability maintains protein synthesis. In splanchnic tissues, both degradation and synthesis are increased but with an altered pattern, so that the levels of some proteins are increased (e.g. proteins of the acute-phase response), while those of others are decreased (e.g. albumin). As a result, intracellular protein content in liver is maintained but secretion of plasma proteins is abnormal. In healthy subjects, an acute increase in insulin concentration, as occurs after a meal, leads to a rapid suppression of protein breakdown in the splanchnic area. If hyperinsulinaemia is not supported by an exogenous amino acid supply, as might occur during a protein-free meal or experimentally during euglycaemic hyperinsulinaemic clamping, the plasma as well as muscle free amino acid concentration drops, owing to reduced splanchnic release. With reduced amino acid availability, insulin is not anabolic in muscle. If amino acid concentrations are maintained at normal or high levels, e.g. following a mixed meal, a net protein deposition in muscle may occur, primarily because of a stimulation of synthesis and possibly owing to inhibition of breakdown.

Amino Acids↗

Extended-term effects of head and neck irradiation in a rodent.

Radiotherapy to the head and neck is a common treatment for malignancies of the region. Unfortunately, exposure to irradiation often results in a variety of complications, most of which are localised and expressed in the short term following irradiation. However, prolonged and systemic effects may have greater clinical importance as the survival rate of head and neck irradiated patients is increasing yearly. Six groups of 18-20 rats were evaluated during a 1 year study. The non-irradiated control group was compared with 2.5 Gy, 5, 7.5, 10 and 15 Gy irradiated groups. We found a dose-dependent reduction in both survival and body weight in our rat models following a delayed, prolonged and chronic process. Dying animals were emaciated, dehydrated and starved, and many were blind and immunocompromised. While the exact underlying mechanism of this delayed, but devastating, phenomenon has not yet been determined, the delayed xerostomia inflicted on these animals may, at least partially, explain it. The clinical implications for head and neck patients require further evaluation, but our data should be considered, in the context of the available evidence for the long-term effects of head and neck irradiation in humans.

Animal Feed↗

Removal of cartilage rings prevents graft stenosis in extended tracheal allotransplantation with omentopexy and immunosuppression: an experimental study.

BACKGROUND: One of the serious problems in longer size tracheal transplantation is severe stenosis of the graft, probably caused by an inadequate blood supply. We have previously reported that removal of some cartilage rings of the graft and omentopexy helps to provide sufficient blood flow to the graft mucosal tissue and results in satisfactory survival and non-significant graft stenosis in extended tracheal autotransplantation. However, it is unclear whether this method can be applied to extended tracheal allotransplantation that requires immunosuppression. In this report, we describe midterm results of extended tracheal allotransplantation with the technique. METHODS: Twenty-four adult mongrel dogs were used. In 18 dogs, a nine-cartilage-ring length of the trachea was allotransplanted when five cartilage rings of the graft were removed, leaving two rings intact at both ends of the graft for simple fixing to the recipient. Two artificial tracheal rings outside the graft and a stent inside the graft were used for maintaining the lumen width. Omentopexy was done for sufficient blood supply to the graft. FK 506 (0.1 mg/kg) was given on each day after the operation in Group A (n = 10), but was not given at all in Group B (n = 8). In Group C (n = 6), a nine-cartilage-ring length of the trachea, without removal of any cartilage ring, was transplanted into the recipient dog and covered with an omental pedicle flap. The same dose of FK 506 as that used in Group A dogs was given to Group C dogs. RESULTS: In Group A, 2 dogs died of graft stenosis within 9 weeks after surgery and 1 died of emaciation without tracheal stenosis. Seven dogs (70%) survived until time of killing. Among the 8 dogs in Group B, 6 died of graft stenosis within 9 weeks after surgery, with 1 dying of pneumonia and only 1 (13%) surviving for >1 year until killing. In Group C, all 6 dogs died of graft stenosis within 6 weeks after surgery. Survival at 16 weeks after surgery was 70% in Group A, 13% in Group B and 0% in Group C (p < 0.01, A vs B and C). No significant graft stenosis was found in 6 dogs and mild stenosis was found in 2 dogs at the time of death or killing in Group A (80%), whereas mild stenosis was found in only 2 dogs in Group B (25%) (p < 0.05). Mucosal blood flow of the graft in Group A was higher than that in Group C and was the same as that in Group B within 4 weeks after surgery; however, it remained unchanged to ultimately be higher than in Group B at 6 and 8 weeks after surgery. CONCLUSIONS: Removal of some cartilage rings, omentopexy and immunosuppression improved blood supply to the graft and resulted in good survival and non-significant tracheal stenosis in extended tracheal allotransplantation.

Animals↗

Eating disorders in school-aged children.

It is widely accepted that eating disorders do occur in children. There is a growing literature on childhood-onset AN, and it seems that the core behavioral, psychologic, and physical features are similar to those in adults. The differences between children and adults also must be taken into account, however. Because children have lower levels of body fat, they tend to become emaciated and suffer the effects of starvation for more quickly than adults, which must be taken into account when considering treatment. Although cases of childhood-onset BN have been reported, they are so rare that empirical research is difficult. Clinical features reported regarding the atypical childhood-onset eating disorders generally concur, although empirical testing of these features has yet to be developed. Theories as to why children develop these disorders need further development. The general consensus is that all childhood-onset eating disorders must be considered using a multidimensional model that takes into account physical, psychologic, social, and family factors in origin, assessment, and treatment.

Anorexia Nervosa↗

Association between body condition score and live weight in pasture-based Holstein-Friesian dairy cows.

The objective was to quantify the strength of the relationship between body condition score (BCS) and live weight (LW) in pasture-based Holstein-Friesian dairy cattle, and to determine the kg LW per unit BCS. A total of 26021 test-day records with information on both BCS (1-10 scale, where 1 is emaciated and 10 is obese) and LW across 1110 lactations from one research farm were used in the analysis. Correlation and regression analyses were used to determine the degree of association between BCS and LW in different parities, stages of the inter-calving interval and years. Correlations between BCS and LW were relatively consistent, with the mean correlation between BCS and LW across all data of 0.55 implying that differences in BCS explain approximately 30% of the variation in LW. Significantly different regressions of LW on BCS were present within stage of inter-calving interval by parity subclasses. Excluding calving, LW per unit BCS varied from 17 kg (early to mid lactation in parity 1) to 36 kg (early lactation in parity 4 and 5). However, LW per unit BCS was greatest at calving varying from 44 kg in first parity animals to 62 kg in second parity animals. On average, 1 BCS unit equated to 31 kg LW across all data.

Animals↗

Clinico-pathological studies on naturally-occurring bovine fascioliasis in the Sudan.

An investigation was made of the pathological, haematological and biochemical aspects of naturally-occurring bovine fascioliasis in the Sudan. 228 animals infected with Fasciola gigantica and 25 non-infected controls were used in the study. The infected cattle revealed emaciation, typical liver pathology, and, occasionally, lesions in the lung and the pancreas. Analysis of their sera also showed reduced albumin values, increased globulin concentrations and decrease albumin/globulin ratio, in addition to increased arginase activity. The serum iron concentration, on the other hand, was decreased, while the total iron binding capacity increased and the resultant iron saturation values reduced. Haematological findings in the infected animals included reduced erythrocyte counts, decreased haematocrit values, increased mean corpuscular volumes, eosinophilia and decreased neutrophil, lymphocyte and monocyte counts. Other parameters were similar in infected and control cattle.

Animals↗

The pathology of experimental Schistosoma bovis infection in sheep.

Five desert sheep were exposed to 5000 or 10000 Schistosoma bovis cercariae each, and the parasitological clinical and pathological parameters were recorded. The pre-patent period was approximately 7 weeks and from that time onwards, the animals became progressively ill and emaciated. Following necropsy at the 12th week, a percentage schistosome recovery ranging from 36%--63.6% was found, and there were high egg densities in the intestinal tract and the liver. These organs revealed severe pathological lesions which were described in detail along with the changes occurring in other tissues.

Animals↗

The role of nematode parasites in Soay sheep (Ovis aries L.) mortality during a population crash.

In early 1989, two-thirds of the Soay sheep population on St Kilda died over 12 weeks. Post-mortem examinations revealed emaciated carcasses and considerable nematode burdens, with protein-energy malnutrition as the probable cause of death. Haematological and blood biochemical changes in the sheep, as well as fecundity of gastrointestinal nematodes, suggested the hosts were immunosuppressed. In parallel, laboratory experiments in which Soay sheep on a high plane of nutrition were artificially infected with Ostertagia circumcincta, showed no clinical signs or mortality when supporting worm burdens similar to those recorded in dead sheep on St Kilda. Anthelmintic treatment of a group of animals increased daily survival rates in ewes and male lambs, although treated animals became re-infected as the 'crash' progressed. It is suggested that parasites contribute to mortality in malnourished hosts, exacerbating the effects of food shortage.

Analysis of Variance↗

Comparative studies of Trypanosoma (Duttonella) vivax isolates from Colombia.

The characterization of four Trypanosoma vivax isolates from Colombia in South America showed that although minor phenotypic differences existed between them, these parasites are antigenically related and belong to a single serodeme. Characterization by isoenzyme assay, karyotyping and DNA probe analysis, showed the Colombian isolates to be more similar to the West African than to Kenyan T. vivax. There was, however, little serological cross-reactivity between South American and African groups of T. vivax. Although the T. vivax isolates from Colombia were pathogenic for dairy calves which showed the typical sign of progressive emaciation, these parasites failed to infect mice or tsetse and could not be cultivated as bloodstream forms in vitro. This study represents initial attempts to establish the phenotypic and serological diversity amongst T. vivax isolates from South America.

Africa, Western↗

Fat phobic and non-fat phobic anorexia nervosa: a comparative study of 70 Chinese patients in Hong Kong.

A mixed retrospective-prospective study of 70 Chinese anorexic patients in Hong Kong shows that although they were similar to Western anorexics in most other ways, 41 (58.6%) of them did not exhibit any fear of fatness throughout their course of illness. Instead, these non-fat phobic patients used epigastric bloating (31.4%), no appetite/hunger (15.7%) or simply eating less (12.9%) as legitimating rationales for food refusal and emaciation. Compared to fat phobic anorexics, they were significantly slimmer pre-morbidly (P < 0.0001) and were less likely to exhibit bulimia (P = 0.001). The possible explanations for the absence of fat phobia and the interpretive dilemma this provokes are discussed from historical, pathoplastic and cultural anthropological perspectives. It is argued that anorexia nervosa may display phenomenological plurality in a Westernizing society, and its identity may be conceptualized without invoking the explanatory construct of fat phobia exclusively. As non-fat phobic anorexia nervosa displays no culturally peculiar features, it is not strictly speaking a Western culture-bound syndrome, but may evolve into its contemporary fat phobic vogue under the permeative impact of Westernization. Its careful evaluation may help clarify the aetiology and historical transformation of eating disorder, foster the development of a cross-culturally valid taxonomy of morbid states of self-starvation, and exemplify some of the crucial issues that need to be tackled in the cross-cultural study of mental disorders.

Adult↗

Amenorrhoea, body weight and serum hormone concentrations, with particular reference to prolactin and thyroid hormones in anorexia nervosa.

Twenty women with anorexia nervosa were investigated at varying stages during weight gain. Basal prolactin and TSH and prolactin responses to TRH were normal and unrelated to body weight. LH, FSH and 17 beta oestradiol were low in emaciated patients and rose with weight gain. There was no correlation between serum gonadotrophin and prolactin concentrations. T3 and T4 concentrations were low but T3 rose with weight gain during refeeding over 4-6 weeks, whereas T4 remained low. A positive correlation was found between the TSH response to TRH and body weight. The abnormalities in the hypothalamic-pituitary-thyroid axis were similar to those seen in a variety of chronic illnesses and appear to be unrelated to the amenorrhoea. The failure of restoration of normal function at least after short-term refeeding requires further investigation. It was concluded that the amenorrhoea in anorexia nervosa is not associated with changes in prolactin secretion but is determined primarily by changes in the hypothalamic-pituitary-gonadal axis. These changes are induced largely by nutritional factors but psychological factors may also be involved.

Adolescent↗

How patients describe bulimia or binge eating.

Thirty-two patients who complained of episodes of ravenous overeating which they felt unable to control (bulimia) were asked to describe their behaviour and symptoms. There was considerable variation both between and within individuals, but a number of factors were defined which appeared to be common to all with the complaint. It is difficult to set up strict criteria for the recognition of bulimia, and those that have recently been proposed are criticized in the light of our present findings. Bulimia is usually associated with an excessive concern about body weight. It occurs in patients with anorexia nervosa, in whom it is often a relatively early feature of the illness, but it is also found in subjects of normal weight or obese subjects who have never been emaciated. Episodes of bulimia are usually preceded by dysphoric mood states. The gorging may alleviate the dysphoria temporarily, but many patients later experience negative feelings such as depression and self-depreciation. The ability to induce vomiting after a bulimic episode is a major influence determining the clinical presentation.

Adolescent↗

Ovine lentivirus is aetiologically associated with chronic respiratory disease of sheep on the Laikipia Plateau in Kenya.

A study was undertaken to investigate the occurrence of ovine lentivirus (OvLV) infection in sheep with chronic respiratory disease on the Laikipia Plateau, Kenya. All seven Merino crossbred sheep with chronic dyspnoea and emaciation examined for gross and microscopic lesions had lymphoid interstitial pneumonia (LIP), and one also had pulmonary abscesses. Two of the sheep with LIP also had lesions of ovine pulmonary carcinoma (OPC, jaagsiekte). Using in situ hybridization, OvLV DNA localized to a high proportion of pulmonary macrophages in lungs with lesions of LIP. Lung tissue samples from six of these sheep were positive for a syncytium-inducing virus in cultures of lamb testis cells. Thin-section electron microscopy of infected cells showed virions with morphogenesis typical of lentiviruses. In a western blotting assay, monoclonal antibodies to the OvLV capsid (CA, p27) and matrix (MA, p15) proteins of a North American OvLV isolate reacted with similar-sized bands of the virus, and serum from six of the sheep were reactive with CA from the Kenyan viral isolate. Using an OvLV agar gel immunodiffusion (AGID) test, all seven sheep were positive for serum antiviral antibody, as were 29% of 63 clinically normal sheep from Laikipia District. However, when sera from the healthy sheep were tested in a western blot assay, only 52% had IgG reactive to the OvLV CA, indicating a high rate of false negative reactions with the AGID test. Serum samples from 87 Red Maasai or Dorper crossbred sheep from two farms in other parts of Kenya were OvLV seronegative by both the AGID test and the western blot assay. These results document the first identification of OvLV as a cause of chronic respiratory disease in sheep in Kenya and show a high rate of infection in sheep flocks, with a high prevalence of chronic respiratory disease.

Animals↗

Beta-galactosidase-deficient mouse as an animal model for GM1-gangliosidosis.

GM1-gangliosidosis is a progressive neurological disease in humans caused by deficiency of lysosomal acid beta-galactosidase, which hydrolyses the terminal beta-galactosidic residue from ganglioside GM1 and other glycoconjugates. In this study, we generated a mouse model for GM1-gangliosidosis by gene targeting in embryonic stem cells. The mouse homozygous for the disrupted beta-galactosidase gene showed beta-galactosidase deficiency, presented with progressive spastic diplegia, and died of emaciation at 7-10 months of age. Pathologically, PAS-positive intracytoplasmic storage was observed in neuronal cells of various areas in the brain. Biochemical analysis revealed a marked accumulation of ganglioside GM1 and asialo GM1 in brain tissue. This animal model will be useful for pathogenetic analysis and therapeutic trial of human GM1-gangliosidosis.

Animals↗

Mutation analysis of the acid ceramidase gene in Japanese patients with Farber disease.

Farber disease is a rare lysosomal storage disease, characterized by the accumulation of ceramide in tissues due to acid ceramidase deficiency. Here we report the identification of three novel mutations in the acid ceramidase gene from two Japanese patients. Patient 1 showed joint problems at around 10 months of age and the patient is now emaciated, with multiple nodules and mild neurological problems at 10 years of age. Patient 2 had consanguineous parents and showed joint contractures at around 8 months of age. He showed neurological symptoms around 2 years of age and died at 6 years owing to respiratory failure. The diagnosis was made clinically and was confirmed by enzymatic assay of acid ceramidase. Molecular analysis of cultured skin fibroblasts showed normal mRNA levels expressed in both patients. By direct sequencing of cDNA, missense mutations of V97E in exon 4 and G235R in exon 9 were detected in patient 1 and 96delV in exon 4 was homozygously identified in patient 2. These mutations were also confirmed in genomic DNA. Expression of mutated acid ceramidase cDNA in COS-1 cells showed acid ceramidase activity decreased to 35%, 2% and 37% of control value, respectively. We also found a new polymorphism V3691 in exon 14 in the allele from the mother of patient 1. To date, 13 mutations, including our newly identified mutations, have been reported. All these mutations were genetically private and genotype-phenotype correlations could not be made.

Animals↗

[Swiss scrapie surveillance. I. Clinical aspects of neurological diseases in sheep and goats].

Small ruminants infected with scrapie show a large range of often unspecific clinical symptoms. The most-often described signs, locomotion, sensibility and behavioural disorders and emaciation, rarely occur together, and cases have been described in which only one of those signs was detectable.Thus, formulating a well-circumscribed definition of a clinical suspect case is difficult. Most animals with CNS-effecting diseases such as listeriosis, polioencephalomacia, cerebrospinal nematidiasis and enterotoxemia will, in a thorough neurological examination, show at least some scrapie-like symptoms. Among the 22 neurological field cases examined in this study, a goat with cerebral gliomatosis and hair lice showed the closest similarity to clinical scrapie. The unilateral deficiency of the cerebral nerves has potential as an clinical exclusion criterion for scrapie. However, the laboratory confirmation--or exclusion--of scrapie remains important. It thus needs to be realized that a consistent and thorough examination of neurologically diseased small ruminants (including fallen stock) is the backbone of a good surveillance system for these diseases. This should be a motivation for submitting adult sheep and goats for neuropathological examination.

Animals↗