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1.
Poult Sci ; 103(9): 104002, 2024 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-39053371

RESUMEN

Aflatoxin B1 (AFB1) is a significant pollutant found in food and feed, posing a threat to public health. The objective of this study was to assess the effect of Lactiplantibacillus plantarum (LACP) against AFB1 in growing rabbits by investigating growth, serum metabolites, immunity, antioxidant capacity, and inflammatory response. A total of 60 growing male rabbits (721.5 ± 2.68g) were allocated to 4 experimental groups. The control group receiving only a basal diet, the AFB1 group (0.3 mg AFB1/kg diet), the LACP group (1 × 109 cfu/g /kg diet), and the combination group (1 × 109 cfu/g + 0.3 mg AFB1/kg diet; AFB1+ LACP) for 8 wk. The administration of AFB1 alone significantly decreased the final body weight, body gain, and feed intake, while significantly increasing the feed conversion ratio (P < 0.05). A significant decline in total proteins and globulins, along with elevated levels of hepatic enzymes (AST, ALP, ALT, and GGT) and renal function markers (creatinine and uric acid), were observed in the AFB1-contaminated group (P < 0.05). Immunoglobulins (IgG and IgM) were significantly decreased, alongside a significant elevation of triglycerides, direct bilirubin, and indirect bilirubin in growing rabbits fed diets with AFB1 (P < 0.05). Supplementing the AFB1 diet with LACP restored the growth reduction, improved liver (AST, ALP, ALT, and GGT) and kidney (creatinine and uric acid) functions, and enhanced immune markers in rabbit serum (P < 0.05). Antioxidant indices (SOD, GSH, and CAT) were significantly decreased in the AFB1 group (P < 0.05). However, the addition of LACP to the AFB1-contaminated diets improved antioxidant capacity and malondialdehyde (MDA) and protein carbonylation (PC) in hepatic tissues of rabbits (P < 0.05). Serum interlukin-4 (IL-4) and interferon gamma (IFN-γ) levels were significantly increased in the AFB1 group (P < 0.05), but the addition of LACP significantly reversed this elevation. AFB1 downregulated the expression of immune-inflammatory genes such Nrf2, IL-10, and BCL-2 genes, while up-regulating the caspase-3 (CASP3) gene (P < 0.05). Supplementing AFB1 diet with LACP significantly decreased the expression of immune-inflammatory genes (Nrf2, IL-10, and BCL-2) and reduced the expression of the apoptotic-related gene CASP3. This study highlights the potential of L. plantarum (1 × 109 cfu/g /kg diet) as a protective agent against AFB1 in growing rabbits by enhancing antioxidant and immune function and reducing apoptosis and inflammation pathways.


Asunto(s)
Aflatoxina B1 , Alimentación Animal , Dieta , Inflamación , Estrés Oxidativo , Probióticos , Animales , Conejos , Aflatoxina B1/toxicidad , Masculino , Estrés Oxidativo/efectos de los fármacos , Alimentación Animal/análisis , Probióticos/administración & dosificación , Probióticos/farmacología , Inflamación/veterinaria , Inflamación/inducido químicamente , Dieta/veterinaria , Apoptosis/efectos de los fármacos , Distribución Aleatoria
3.
Heliyon ; 8(10): e10967, 2022 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-36237979

RESUMEN

Monosodium glutamate (MSG) is commonly used worldwide as a food flavour enhancer by the food industry. The current study investigated the in vivo toxic effects of MSG on the uterus in adult female Sprague Dawley rats and in vitro using MCF-7 and MDA-MB-231 cells, computational toxicity and molecular docking. The average levels of progesterone and oestrogen in the MSG-treated animals significantly altered. Besides, the average uterine lumen area (µm2) was smaller than the control group. MSG showed high-affinity binding to acetylcholine receptors and disrupted the normal nerve signal with a predicted LD50 of 4500 mg/kg. MSG also demonstrated good binding affinity to human oestrogen receptors beta and some other proteins that have an oxidative stress role in the female reproductive organs. Therefore, a precaution should be taken when utilising this compound, especially for females under the risk factor of hormonal abnormality.

4.
Environ Sci Pollut Res Int ; 29(60): 90058-90069, 2022 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-35864392

RESUMEN

Averrhoa carambola is a species of tree native to tropical Southeast Asia. It possesses antioxidant and anti-hyperlipidemia effects and has traditionally been used to treat a variety of ailments. However, the presence of oxalic acid in its fruits might restrict its consumption by individuals suffering from kidney disease, and caramboxin can cause neurotoxicity. In this study, we evaluated the acute and sub-chronic toxicity of the methanolic extract of A. carambola leaves (MEAC) in male and female rats. In the acute study, female rats were given a single oral dose of 5000 mg/kg of MEAC and closely examined for distinct indications of toxic effects during the first 4 h, periodically for 48 h, and daily thereafter for 14 days. Rats of both sexes were employed in the sub-chronic investigation for the 28-day repeated dose oral toxicity study. Results of the acute study revealed the safety of MEAC up to a dose of 5000 mg/kg where the rats did not show changes or signs of toxicity. In the sub-chronic toxicity study, MEAC (250, 500, and 1000 mg/kg) administration did not affect the body weight, food, and water consumption, motor coordination, behavior, or mental alertness in the treated rats. In addition, no variations in hematological or biochemical markers were found in MEAC-treated rats. In conclusion, these findings pinpoint the safety of MEAC at doses up to 5000 mg/kg. The leaves of A. carambola could be safely consumed by people with kidney disease to treat other ailments.


Asunto(s)
Averrhoa , Síndromes de Neurotoxicidad , Ratas , Animales , Ratas Sprague-Dawley
5.
Cancers (Basel) ; 14(5)2022 Feb 22.
Artículo en Inglés | MEDLINE | ID: mdl-35267408

RESUMEN

Cancer is a life-threatening disease and is considered to be among the leading causes of death worldwide. Chemoresistance, severe toxicity, relapse and metastasis are the major obstacles in cancer therapy. Therefore, introducing new therapeutic agents for cancer remains a priority to increase the range of effective treatments. Terpenoids, a large group of secondary metabolites, are derived from plant sources and are composed of several isoprene units. The high diversity of terpenoids has drawn attention to their potential anticancer and pharmacological activities. Some terpenoids exhibit an anticancer effect by triggering various stages of cancer progression, for example, suppressing the early stage of tumorigenesis via induction of cell cycle arrest, inhibiting cancer cell differentiation and activating apoptosis. At the late stage of cancer development, certain terpenoids are able to inhibit angiogenesis and metastasis via modulation of different intracellular signaling pathways. Significant progress in the identification of the mechanism of action and signaling pathways through which terpenoids exert their anticancer effects has been highlighted. Hence, in this review, the anticancer activities of twenty-five terpenoids are discussed in detail. In addition, this review provides insights on the current clinical trials and future directions towards the development of certain terpenoids as potential anticancer agents.

6.
Oxid Med Cell Longev ; 2021: 5511523, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-34136063

RESUMEN

Thymoquinone (TQ), the active constituent of Nigella sativa, possesses several benefits in traditional and modern medicines. This study examined the effect of a single dose of Nano-TQ on chronic lung injury induced by repetitive intratracheal installation of lipopolysaccharide (LPS). Rats received LPS twice weekly for 8 weeks via intratracheal installation and a single dose of TQ-PLGA NPs on the day after the last dose of LPS. Six rats from each group were sacrificed after 8 and 10 weeks, and samples were collected for analysis. Repetitive intratracheal installation of LPS caused histopathological alterations, including partial or complete obstruction of the alveoli, interstitial edema, mild fibroblastic proliferation, fibrous strands besides lymphocytes and plasma infiltrations, suffered fetalization, bronchiectasis, hypertrophied arterioles, and others. Investigation of the ultrastructure revealed prominent necrotic pneumocytes with destructed chromatin and remnant of necrotic debris in the narrowing alveolar lumen in LPS-induced rats. TQ-PLGA NPs effectively ameliorated LPS-induced histopathological and ultrastructural alterations in the lung of rats. In addition, TQ-PLGA NPs significantly alleviated serum levels of IL-10 and TGF-ß1 in LPS-induced rats. In conclusion, TQ-PLGA NPs prevented inflammation and tissue injury in the lungs of rats challenged with repetitive intratracheal installation of LPS. Therefore, TQ-PLGA NPs represent a promising candidate for the prevention of lung injury induced by LPS, pending further studies to determine its safety and exact protective mechanism.


Asunto(s)
Benzoquinonas/uso terapéutico , Intubación Intratraqueal/métodos , Lipopolisacáridos/efectos adversos , Lesión Pulmonar/tratamiento farmacológico , Nanopartículas/uso terapéutico , Animales , Benzoquinonas/farmacología , Enfermedad Crónica , Humanos , Lesión Pulmonar/patología , Masculino , Ratas
7.
Diabetes Metab Syndr Obes ; 13: 4809-4822, 2020.
Artículo en Inglés | MEDLINE | ID: mdl-33335409

RESUMEN

BACKGROUND: Ganoderma lucidum spores (GLS) exhibit disease prevention properties, but no study has been carried out on the anti-diabetic cardiomyopathy property of GLS. The aim of this study was to evaluate the hyperglycemia-mediated cardiomyopathy protection and mechanisms of GLS in streptozotocin (STZ)induced diabetic rats. METHODS: Male SD rats were randomly divided into three groups. Two groups were given STZ (50 mg/kg, i.p.) treatment and when their fasting plasma glucose was above 16.7 mmol/L, among them, one group was given placebo, as diabetic group, and another group was given GLS (300 mg/kg) treatment. The group without STZ treatment was given placebo as a control group. The experiment lasted 70 days. The histology of myocardium and biomarkers of antioxidants, myocardial injury, pro-inflammatory cytokines, pro-apoptotic proteins and phosphorylation of key proteins in PI3K/AKT pathway were assessed. RESULTS: Biochemical analysis showed that GLS treatment significantly reduced the blood glucose (-20.3%) and triglyceride (-20.4%) levels compared to diabetic group without treatment. GLS treatment decreased the content of MDA (-25.6%) and activity of lactate dehydrogenase (-18.9%) but increased the activity of GSH-Px (65.4%). Western blot analysis showed that GLS treatment reduced the expression of both alpha-smooth muscle actin and brain natriuretic peptide. Histological analysis on the cardiac tissue micrographs showed that GLS treatment reduced collagen fibrosis and glycogen reactivity in myocardium. Both Western blot and immunohistochemistry analyses showed that GLS treatment decreased the expression levels of pro-inflammatory factors (cytokines IL-1ß, and TNF-α) as well as apoptosis regulatory proteins (Bax, caspase-3 and -9), but increased Bcl-2. Moreover, GLS treatment significantly increased the phosphorylation of key proteins involved in PI3K/AKT pathway, eg, p-AKT p-PI3K and mTOR. CONCLUSION: The results indicated that GLS treatment alleviates diabetic cardiomyopathy by reducing hyperglycemia, oxidative stress, inflammation, apoptosis and further attenuating the fibrosis and myocardial dysfunction induced by STZ through stimulation of the PI3K/Akt/mTOR signaling pathway.

8.
Biomed Res Int ; 2020: 7136075, 2020.
Artículo en Inglés | MEDLINE | ID: mdl-32775437

RESUMEN

Even with substantial advances in cardiovascular therapy, the morbidity and mortality rates of diabetic cardiomyopathy (DCM) continually increase. Hence, a feasible therapeutic approach is urgently needed. Objectives. This work is aimed at systemically reviewing literature and addressing cell targets in DCM through the possible cardioprotection of G. lucidum through its antioxidant effects by using the Open Targets Platform (OTP) website. Methods. The OTP website version of 19.11 was accessed in December 2019 to identify the studies in DCM involving G. lucidum. Results. Among the 157 cell targets associated with DCM, the mammalian target of rapamycin (mTOR) was shared by all evidence, drug, and text mining data with 0.08 score association. mTOR also had the highest score association 0.1 with autophagy in DCM. Among the 1731 studies of indexed PubMed articles on G. lucidum published between 1985 and 2019, 33 addressed the antioxidant effects of G. lucidum and its molecular signal pathways involving oxidative stress and therefore were included in the current work. Conclusion. mTOR is one of the targets by DCM and can be inhibited by the antioxidative properties of G. lucidum directly via scavenging radicals and indirectly via modulating mTOR signal pathways such as Wnt signaling pathway, Erk1/2 signaling, and NF-κB pathways.


Asunto(s)
Antioxidantes , Cardiotónicos , Cardiomiopatías Diabéticas , Extractos Vegetales , Reishi/química , Antioxidantes/química , Antioxidantes/uso terapéutico , Cardiotónicos/química , Cardiotónicos/uso terapéutico , Cardiomiopatías Diabéticas/tratamiento farmacológico , Cardiomiopatías Diabéticas/metabolismo , Cardiomiopatías Diabéticas/patología , Humanos , Sistema de Señalización de MAP Quinasas/efectos de los fármacos , Extractos Vegetales/química , Extractos Vegetales/uso terapéutico , Serina-Treonina Quinasas TOR/metabolismo , Vía de Señalización Wnt/efectos de los fármacos
9.
Transfus Clin Biol ; 27(2): 61-64, 2020 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-32280062

RESUMEN

BACKGROUND AND AIM: The aim of this study is to evaluate the clinical, biological and hematological profiles of autoimmune hemolytic anemia (AIHA) in children and to specify its etiologies, therapeutic modalities, and treatment responses. METHODS: This is a 14-year retrospective study of AIHA cases collected at the department of pediatric emergency and reanimation of Hedi Chaker University Hospital in Sfax. We included patients under 14 years old with clinical and biological features of hemolysis and a positive direct antiglobulin test (DAT). The selected patients' demographic characteristics, physical signs, laboratory findings, and treatment responses were recorded. RESULTS: Thirteen cases of AIHA were collected, including 8 girls and 5 boys. The median age at diagnosis was 4 years and 6 months (range: 8 months to 13 years). Consanguinity was reported in 6 cases and 4 patients had a previous infection history. The onset of AIHA was progressive in 9 cases, marked by an anemic syndrome and hemolysis symptoms in 6 and 8 cases, respectively. The clinical triad (pallor, jaundice and splenomegaly) was found in only 4 cases. At the time of diagnosis, the median hemoglobin (Hb) level was 6g/dL (range: 4.2 to 9.2g/dL), anemia was non-regenerative in 2 patients. Thrombocytopenia and neutropenia were noted in 5 and 1 patient, respectively. Peripheral smear examination showed spherocytosis in 2 cases. All the patients had a positive DAT. Of these, 10 were positive with IgG and 3 with both IgG and C3d. AIHA was secondary to other conditions in 9 patients: infection (3 cases), autoimmune disease (4 cases), and immunodeficiency (2 cases). All the patients received first-line corticosteroid therapy but only 8 of them required blood transfusions due to severe anemia. Complete remission was obtained in 7 cases. Corticosteroid resistance and dependence were noted in 1 and 2 cases, respectively. During evolution, additional therapy was indicated in 4 patients and it included cyclosporine A, azathioprine, and mycophenolate mofetil (MMF). After a median follow-up of 4.5 years, the cure rate was 80% and only 1 patient (a boy) died due to his underlying pathology. CONCLUSION: Our study highlights the rarity, severity, and heterogeneity of etiological contexts of AIHA in children. The therapeutic difficulties justify specific expertise in pediatric hematology.


Asunto(s)
Anemia Hemolítica Autoinmune , Leucopenia , Trombocitopenia , Adolescente , Anemia Hemolítica Autoinmune/diagnóstico , Anemia Hemolítica Autoinmune/terapia , Niño , Femenino , Hemólisis , Humanos , Masculino , Estudios Retrospectivos
10.
Pak J Biol Sci ; 23(3): 231-239, 2020 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-31944083

RESUMEN

BACKGROUND AND OBJECTIVES: The occurrence of multi-drug resistance (MDR) Escherichia coli is one responsible for raised mortality and morbidity and was reported as major health problem. Class 1 integrons has crucial role in distributing antibiotic resistance genes among bacteria. Present work was aimed to determine the prevalence of class 1 integrons and its association with antibiotic resistance in MDR E. coli isolated from patient's body fluid and tissues from 6 health centers in Sana'a, Yemen. MATERIALS AND METHODS: A cross-sectional study a total of 198 E. coli from patients diagnosed with infection that had been referred to 6 hospitals and medical diagnostic from July, 2017 to August, 2017 in Sana'a, Yemen. Susceptibility of E. coli isolates to 15 antibiotics using the disc diffusion method. Conventional polymerase chain reaction was used for detection of class 1 of integrons in 100 randomly selected MDR E. coli. RESULTS: Overall 174 (87.9%) of 198 E. coli isolates were MDR. Class 1 integrons were detected in 67% of the randomly selected 100 of 198 MDR E. coli. A significant range (p<0.05-p<0.0001) was identified between presence of class 1 integrons and resistance to ceftriaxone, aztreonam, cefepime, amoxicillin-clavulanic acid, cefotoxime, cefepime-clavulanic acid, ceftazidime-clavulanic acid, ciprofloxacin, ceftazidime, norfloxacin and trimethoprim-sulfamethoxazole, while no significant difference were identified between integron class 1 and resistance to gentamicin, amikacin, nitrofurantoin and imipenem. CONCLUSION: High MDR E. coli isolates were detected in this study, among them the prevalence of class 1 integrons is the most common. The significant association between class 1 integrons and resistance to common prescribed antibiotics in hospitals in Sana'a, Yemen.


Asunto(s)
Infección Hospitalaria/microbiología , Farmacorresistencia Bacteriana Múltiple , Infecciones por Escherichia coli/microbiología , Escherichia coli/efectos de los fármacos , Escherichia coli/genética , Integrones , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Antibacterianos/farmacología , Niño , Preescolar , Infección Hospitalaria/tratamiento farmacológico , Estudios Transversales , Infecciones por Escherichia coli/tratamiento farmacológico , Hospitales , Humanos , Lactante , Recién Nacido , Pruebas de Sensibilidad Microbiana , Persona de Mediana Edad , Yemen , Adulto Joven
11.
Reumatismo ; 70(2): 92-99, 2018 Jul 06.
Artículo en Inglés | MEDLINE | ID: mdl-29976043

RESUMEN

The aim was to describe the profile of workers with occupational multi-site musculoskeletal disorders (MSMSD) and study the relationship between these lesions and socio-professional factors. This is a cross-sectional study involving 254 subjects with occupational musculoskeletal disorders (MSD), identified in the Department of Occupational Medicine at the University Hospital of Mahdia, in Tunisia, over a period of 10 years from 2005 to 2014. The study population was subdivided into two groups; mono-site MSD and multi-site (≥2 sites) groups. Data collection was based on a questionnaire prepared beforehand and covered the description of sociodemographic and professional characteristics. To study psychosocial constraints at work, we have used the Karasek questionnaire. MS-MSD was correlated to the number of dependent children (p=0.02), job/place of work (p=0.00), qualification (p=0.02), taking a rest period (p=0.03), decision latitude (p=0.00), mental demands (p=0.002), social support (p=0.00) and job stress (p=0.04). After binary logistic regression, MS-MSD depended significantly on the number of dependent children (p=0.013; OR=0,33; IC=0,17-0,83), working spouse (p=0.05; OR=0.35; IC=0.12-0.99), job/place of work (p=0.00; OR=4.16; IC=1.95-8.88), qualification (p=0.008; OR=0.28; IC=0.11-0.72), taking a break during work (p=0.04; OR=3.10; IC=1.04-9.22) and social support (p=0.00; OR=7,1; IC=1,9-25,3). When individual risk factors are fixed, the prevention of MS-MSD must target modifiable levers, related to the professional environment of the employees.


Asunto(s)
Enfermedades Musculoesqueléticas/epidemiología , Enfermedades Profesionales/epidemiología , Adulto , Estudios Transversales , Femenino , Humanos , Actividades Recreativas , Masculino , Industria Manufacturera , Persona de Mediana Edad , Enfermedades Musculoesqueléticas/etiología , Enfermedades Profesionales/etiología , Ocupaciones , Estudios Retrospectivos , Factores de Riesgo , Determinantes Sociales de la Salud , Estrés Psicológico/epidemiología , Encuestas y Cuestionarios , Túnez/epidemiología
12.
Arch Pediatr ; 24(12): 1249-1252, 2017 Dec.
Artículo en Francés | MEDLINE | ID: mdl-29158045

RESUMEN

Sjögren syndrome is uncommon in children and occurs most often in association with autoimmune diseases (secondary Sjögren syndrome). We describe the clinical and biological features of a 7-year-old girl with primary Sjögren syndrome revealed by recurrent parotiditis. CASE REPORT: A 7-year-old girl was referred for investigation of multiple episodes of parotid swelling since age 4 years, without systemic symptoms. The examination was unremarkable except for enlarged and painless parotid glands. Laboratory investigations and labial salivary gland biopsy revealed Sjögren syndrome without associated disease. Hydroxychloroquine was prescribed with clinical improvement. CONCLUSION: Recurrent parotiditis in children is an uncommon condition. The onset of parotid swelling at 5 years or over deserves screening for disimmune disorders, sarcoidosis, or Sjögren syndrome. Diagnosis of Sjögren syndrome is based on diagnostic criteria.


Asunto(s)
Síndrome de Sjögren/diagnóstico , Niño , Femenino , Humanos , Parotiditis/etiología , Síndrome de Sjögren/complicaciones
13.
J Assist Reprod Genet ; 34(11): 1567-1569, 2017 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-28840413

RESUMEN

PURPOSE: The majority of milk in industrialized countries is obtained from pregnant cows, which contains increased levels of estrogen and progesterone compared to non-pregnant cows. The aim of this study was to quantify the amount of hormones present in milk with different fat content because previous studies on humans have shown potential effects of increased milk consumption on serum and urine hormone levels as well as on sperm parameters. However, it is unclear whether consumption of milk at the currently recommended levels would lead to systemic effects. METHODS: Samples of cow's milk of varying fat concentrations (0, 1, 2, 3.25, 10, and 35%) were analyzed via competitive ELISA assays. RESULTS: Progesterone concentrations were significantly correlated to increasing fat content of milk (r = 0.8251, p = 0.04). CONCLUSIONS: Research on conditions in which additional progesterone may have an effect on human health should consider inclusion of limitation of milk intake and its effects. Further studies are needed to determine the concentration of progesterone in milk of different fat content in other regions and countries and to quantify the potential pathophysiologic role.


Asunto(s)
Gonadotropina Coriónica/química , Estradiol/química , Leche/química , Progesterona/química , Animales , Bovinos , Gonadotropina Coriónica/aislamiento & purificación , Estradiol/aislamiento & purificación , Femenino , Humanos , Leche/metabolismo , Embarazo , Progesterona/aislamiento & purificación , Quebec
14.
Arch Pediatr ; 22(6): 616-20, 2015 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-25911058

RESUMEN

OBJECTIVES: Pseudomonas aeruginosa sepsis usually carries a high mortality rate in immunocompromised children. Ecthyma gangrenosum is a known cutaneous manifestation due mainly to Pseudomonas infection with or without septicemia. We describe clinical, biological, and therapeutic data. PATIENTS AND METHODS: Of those children admitted to the pediatric intensive care unit, three pediatric cases of community-acquired P. aeruginosa septicemia associated with ecthyma gangrenosum were retrospectively reviewed. RESULTS: The three patients were aged 5 months, 9 months, and 1 year. Underlying hypogammaglobulinemia was detected in the oldest patient. Pseudomonas aeruginosa was isolated in all patients in blood and once in cultures of skin lesions and endotracheal aspirate. Two deaths occurred due to septic shock and multisystem organ failure despite numerous aggressive resuscitation attempts. CONCLUSIONS: P. aeruginosa sepsis should be treated as early as possible. Recognition of ecthyma gangrenosum allows early diagnosis and prescription of adequate antibiotic therapy without awaiting blood culture reports.


Asunto(s)
Ectima/etiología , Infecciones por Pseudomonas/complicaciones , Pseudomonas aeruginosa , Sepsis/complicaciones , Infecciones Comunitarias Adquiridas/complicaciones , Ectima/patología , Femenino , Gangrena , Humanos , Lactante , Masculino , Estudios Retrospectivos , Sepsis/microbiología
15.
Arch Pediatr ; 22(5): 518-22, 2015 May.
Artículo en Francés | MEDLINE | ID: mdl-25800633

RESUMEN

BACKGROUND: Chronic granulomatous disease is a rare inherited primary immune deficiency disease characterized by recurrent infection and an increased susceptibility to autoimmunity disorders. We report on the case of a girl with autoimmune hepatitis in chronic granulomatous disease to describe the clinical and biological features and treatment implications for patients with chronic granulomatous disease associated with autoimmune disorders. CASE REPORT: An 18-month-old girl was referred to our department for investigation of hepatomegaly. She was the third child of non-consanguineous parents. Her two elder sisters had died from infectious diseases at an early age. She had elevated liver transaminase levels with a normal gamma globulin concentration. Negative results were found for all autoimmune markers (antinuclear antibody, anti-smooth muscle, anti-liver-kidney microsomal, anti-liver cytosol and anti-soluble liver antigen). Her liver biopsy showed features of interface hepatitis with portal fibrosis. The diagnosis of seronegative autoimmune hepatitis was established. Treatment with corticosteroids and azathioprine led to clinical improvement with normalization of transaminases. Six months after initial presentation, at the age of 2 years, she was readmitted for fever. Staphylococcus aureus bacteremia was identified with multiple foci of infection (skin infection, arthritis of the right elbow, pneumonia, buttock abscess). The immunological workup revealed chronic granulomatous disease. The course was marked by a fatal outcome despite appropriate antibiotics and intensive care. CONCLUSION: Early diagnosis of the association between chronic granulomatous disease and autoimmune disorders allows for appropriate treatments, improves the quality of life for affected patients, and reduces the risk of mortality.


Asunto(s)
Enfermedad Granulomatosa Crónica/diagnóstico , Hepatitis Autoinmune/diagnóstico , Antibacterianos/uso terapéutico , Bacteriemia/diagnóstico , Preescolar , Cuidados Críticos , Resultado Fatal , Femenino , Estudios de Seguimiento , Enfermedad Granulomatosa Crónica/genética , Enfermedad Granulomatosa Crónica/terapia , Hepatitis Autoinmune/genética , Hepatitis Autoinmune/terapia , Humanos , Lactante , Infecciones Estafilocócicas/diagnóstico
16.
Arch Pediatr ; 22(4): 380-2, 2015 Apr.
Artículo en Francés | MEDLINE | ID: mdl-25725973

RESUMEN

Disseminated lymphangiomatosis is a congenital lymphovenous vascular malformation. It can occur in different regions, some of which are unusual. The treatment of this vascular malformation is based on surgical excision, sclerotherapy, or recombinant interferon therapy. We report the case of disseminated lymphangiomatosis in a 13-year-old girl who presented with anemia.


Asunto(s)
Anemia/etiología , Vasos Linfáticos/anomalías , Malformaciones Vasculares/complicaciones , Adolescente , Femenino , Humanos
17.
Arch Pediatr ; 21(12): 1364-6, 2014 Dec.
Artículo en Francés | MEDLINE | ID: mdl-25445129

RESUMEN

The association of chronic granulomatosis disease (CGD) with autoimmune diseases such as lupus has been described but remains rare. K… is a boy born of a consanguineous marriage. In the family history, two brothers had died at a young age. He had been followed up since the age of 6 months for CGD. At 11 years of age, he developed malar rash, cheilitis, oral ulceration, and photosensitivity. Systemic lupus erythematosus (SLE) was confirmed by the presence of high levels of antinuclear antibodies. This observation demonstrates that with the clinical association of recurrent infections and skin lesions the diagnosis of CGD with SLE must be considered.


Asunto(s)
Enfermedad Granulomatosa Crónica/complicaciones , Lupus Eritematoso Sistémico/etiología , Niño , Humanos , Masculino
18.
Arch Pediatr ; 21(10): 1123-6, 2014 Oct.
Artículo en Francés | MEDLINE | ID: mdl-25169807

RESUMEN

Distinguishing intestinal tuberculosis from Crohn disease is difficult and can result in misdiagnosis, especially when active pulmonary infection is absent. A 13-year-old girl was admitted to our hospital with a 2-month history of watery diarrhea, abdominal pain, and 12-kg weight loss. Based on clinical, radiological, endoscopic, and histological findings, she was initially misdiagnosed as having Crohn disease and treated with glucocorticosteroids, with a poor response after 4 weeks. Intestinal tuberculosis was then suspected. Improvement was observed during the 1st week of antituberculous treatment. The differentiation of intestinal tuberculosis from Crohn disease may be very difficult in some patients. A positive response to antituberculous treatment associated with clinical, endoscopic, and histological features argue in favor of the diagnosis of intestinal tuberculosis.


Asunto(s)
Enfermedades del Colon/diagnóstico , Enfermedades del Íleon/diagnóstico , Tuberculosis Gastrointestinal/diagnóstico , Dolor Abdominal/etiología , Adolescente , Enfermedad de Crohn/diagnóstico , Errores Diagnósticos , Diarrea/etiología , Femenino , Humanos , Pérdida de Peso
19.
Arch Pediatr ; 21(7): 754-6, 2014 Jul.
Artículo en Francés | MEDLINE | ID: mdl-24935451

RESUMEN

Chronic granulomatous disease is a rare, primary immunodeficiency disorder characterized by a defect in oxidative metabolism in phagocytes and recurrent bacterial and fungal infections. We report a case in a 2-month-old infant admitted with metacarpic osteomyelitis due to Serratia marcescens. Chronic granulomatous disease is rare but must be considered in cases of uncommon or atypical infection.


Asunto(s)
Enfermedad Granulomatosa Crónica/complicaciones , Osteomielitis/microbiología , Enfermedades Raras/complicaciones , Infecciones por Serratia/etiología , Serratia marcescens , Humanos , Lactante , Masculino
20.
Arch Pediatr ; 21(3): 291-5, 2014 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-24462295

RESUMEN

Childhood multiple sclerosis is a rare demyelinating autoimmune disease with particular features. Onset of multiple sclerosis is extremely uncommon in early childhood, particularly before 6 years of age. We report the case of a 5-year-old girl admitted to the hospital for altered consciousness and rapid onset of right hemiparaplegia. Magnetic resonance imaging (MRI) of the brain showed multifocal white matter disease with T2 hyperintense oval lesions in subcortical, periventricular, and cerebellar hemispheres. Treatment with high dose intravenous methylprednisolone (30 mg/kg/day for 3 days) improved symptoms. Intravenous corticosteroid therapy was followed by 1mg/kg/day of oral prednisone. A second MRI, 40 days later, revealed new disseminated T2 hyperintense lesions in the frontal periventricular white matter, corpus callosum, left middle cerebellar peduncle, and dorsal spinal cord, leading to the diagnosis of multiple sclerosis. Azathioprine (2.5 mg/kg/day) was started and the steroid dose was tapered before being stopped after 3 months. After 2 years of follow-up, the patient has remained asymptomatic with a normal neurological exam and with no relapse or side effects of azathioprine. This work shows the particularities in clinical and radiological features of multiple sclerosis in a child aged less than 6 years.


Asunto(s)
Esclerosis Múltiple/diagnóstico , Factores de Edad , Preescolar , Femenino , Humanos , Imagen por Resonancia Magnética
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