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1.
An. pediatr. (2003. Ed. impr.) ; 97(3): 172-178, Sept. 2022. tab
Article in English, Spanish | IBECS | ID: ibc-207803

ABSTRACT

Objetivos: 1) Describir la prevalencia de deficiencia de IgA (DIgA), uveítis, enfermedad celiaca (EC) y alteraciones tiroideas en una cohorte multicéntrica de pacientes diagnosticados de artritis idiopática juvenil (AIJ), y 2) evaluar si los pacientes con AIJ y DIgA presentan otras enfermedades autoinmunes con más frecuencia que los pacientes con niveles normales de IgA. Métodos: Estudio retrospectivo de una cohorte de pacientes con AIJ en seguimiento en unidades de Reumatología pediátrica en 2 hospitales de Madrid (España). Resultados: Se incluyó a 193 pacientes, de los cuales 123 eran mujeres (64%). La edad media al inicio fue 5,6 años (RIC 2,5-9,7) y la mediana de seguimiento 5,1 años (RIC 2,2-8,1). Las 3 categorías ILAR más frecuentes fueron oligoarticular (53%), poliarticular con factor reumatoide negativo (20%) y artritis relacionada con entesitis (10%). Los niveles séricos de IgA estaban disponibles en 172/193 (89%); 25/172 (15%) tenían DIgA, selectiva (< 7mg/dl, n=8) o parcial (7-69mg/dl, n=17). Todos los pacientes tuvieron revisiones oftalmológicas periódicas. Tuvieron uveítis anterior 18 pacientes (9%), 15/18 crónica y 3/18 aguda. Los niveles séricos de antitransglutaminasa IgA (o IgG en pacientes con DIgA) fueron obtenidos en 135/193 (70%); 4 pacientes (3%) fueron diagnosticados de EC por biopsia (n=3) o por criterios clínicos, serológicos o genéticos (n=1); 2 de ellos tenían DIgA (p=0,12; OR=6,4; IC del 95%, 0,9-47,6). Solo 1/153 (0,7%) tuvo hipertirotropinemia con anticuerpos antitiroideos positivos y requirió tratamiento. Conclusión: Los pacientes con AIJ presentan comorbilidades autoinmunes con frecuencia. La DIgA no parece aumentar su prevalencia, con la posible excepción de la EC. (AU)


Objectives: (1) To describe the prevalence of IgA deficiency (IgAD), uveitis, coeliac disease (CD) and thyroid disorders in a multicentre cohort of patients with juvenile idiopathic arthritis (JIA), and (2) to assess whether patients with JIA and IgAD have additional autoimmune disorders more frequently than patients with JIA and normal serum levels of IgA. Methods: Retrospective chart review of a cohort of patients with JIA managed in the paediatric rheumatology units of 2hospitals in Madrid, Spain. Results: This study included 193 patients, 123 (64%) female. The median age at disease onset was 5.6 years (IQR 2.5–9.7) and the median duration of followup was 5.1 years (IQR 2.2–8.1). The 3most common categories of JIA based on the ILAR classification were oligoarticular (53%), poliartritis RF-negative (20%) and enthesitis-related arthritis (10%). Serum IgA levels were available in 172/193 (89%); 25/172 (15%) had selective (<7mg/dl, n=8) or partial (7-69mg/dl, n=17) IgAD. All patients underwent periodic ophthalmic examinations. Eighteen children (9%) had anterior uveitis, which was chronic in 15 and acute in 3. Serum levels of anti-transglutaminase IgA, or IgG in IgAD were obtained in 135/193 (70%). Four children (3%) were diagnosed with CD either by intestinal biopsy (n=3) or by the combination of characteristic clinical, serological and genetic features (n=1); 2of them had IgAD (OR=6.4; 95% CI, 0.9–47.6; p=.12). Only 1 of these 153 patients (0.7%) had hyperthyrotropinaemia with positive anti-thyroid antibodies and required replacement therapy. Conclusion: Patients with JIA frequently present autoimmune comorbidities. IgAD does not seem to increase their prevalence, with the possible exception of CD. (AU)


Subject(s)
Humans , Child, Preschool , Child , IgA Deficiency , Arthritis , Uveitis , Celiac Disease , Thyroid Diseases , Retrospective Studies , Rheumatology , Prevalence
2.
Andes Pediatr ; 93(2): 167-173, 2022 Apr.
Article in Spanish | MEDLINE | ID: mdl-35735294

ABSTRACT

INTRODUCTION: During the SARS-CoV-2 pandemic, efforts have focused on trying to identify the routes of transmis sion of the virus, characterize its symptoms and signs, and investigate the best diagnostic and thera peutic methods. There are fewer published data and series in the pediatric population than in adults. OBJECTIVE: To analyze the clinical and epidemiological characteristics in children under 16 years of age diagnosed with SARS-CoV-2. PATIENTS AND METHOD: Descriptive study carried out on children who underwent SARS-CoV-2 RNA testing due to compatible symptoms, close contact, or requiring hospitalization or surgery, in the Emergency Department of a hospital in Madrid, Spain. 30 variables were collected including epidemiological data, symptoms, and signs of infection. RESULTS: Out of 1378 patients, 12% were positive (165). There was a higher proportion of patients of North African origin in the positive group than in the negative one (p < 0.01). Of all patients, 35.6% reported close contact with a confirmed case, which was more frequent in the positive group. 75.8% of the positive patients had some symptoms, most frequently fever, runny nose, and cough, followed by digesti ve symptoms. There was one case of COVID-19 pneumonia and two patients with MIS-C, one of which had SARS-CoV-2 infection. Eight of the positive patients (4.8%) required hospitalization due to SARS-CoV-2 infection. CONCLUSION: Although SARS-CoV-2 infection is milder in the pediatric population, almost 5% will require hospitalization. No close contact was identified in a high percen tage of patients (61%). Further studies are needed at all levels of care to characterize the infection in children and adolescents.


Subject(s)
COVID-19 , Adolescent , Adult , COVID-19/complications , COVID-19/diagnosis , COVID-19/epidemiology , Child , Humans , Pandemics , RNA, Viral , SARS-CoV-2 , Systemic Inflammatory Response Syndrome
3.
An Pediatr (Engl Ed) ; 97(3): 172-178, 2022 Sep.
Article in English | MEDLINE | ID: mdl-35459637

ABSTRACT

OBJECTIVES: (1) To describe the prevalence of IgA deficiency (IgAD), uveitis, coeliac disease (CD) and thyroid disorders in a multicentric cohort of patients diagnosed with JIA and, (2) to evaluate whether patients with JIA and IgAD present other autoimmune diseases more frequently than patients with normal serum levels of IgA. METHODS: Retrospective chart review of a cohort of patients diagnosed with JIA followed at the paediatric rheumatology units of two hospitals in Madrid, Spain. RESULTS: A total of 193 patients were included. Of them, 123 were females (64%). Median age at disease onset was 5.6 years (IQR 2.5-9.7) and the median time of follow-up was 5.1 years (IQR 2.2-8.1). The three most common ILAR categories were oligoarticular (53%), polyarticular RF negative (20%) and enthesitis related arthritis (10%). Serum IgA levels were available in 172/193 (89%); 25/172 (15%) had selective (<7mg/dl, n=8) or partial (7-69mg/dl, n=17) IgAD. All the patients had periodic eye exams. Eighteen children (9%) had anterior uveitis, 15/18 chronic and 3/18 acute. Serum anti transglutaminase IgA, or IgG in IgAD were obtained in 135/193 (70%). Four children (3%) were diagnosed with CD either by intestinal biopsy (n=3) or by the combination of characteristic clinical, serological and genetic features (n=1); two of them had IgAD (p=0.12; OR=6.4; 95% CI 0.9-47.6). Only 1/153 (0.7%) patient had hyperthyrotropinemia with positive anti-thyroid antibodies and required replacement therapy. CONCLUSION: Patients with JIA frequently present autoimmune comorbidities. IgAD does not seem to increase their prevalence, with the possible exception of CD.


Subject(s)
Arthritis, Juvenile , Celiac Disease , IgA Deficiency , Arthritis, Juvenile/diagnosis , Arthritis, Juvenile/epidemiology , Celiac Disease/diagnosis , Celiac Disease/epidemiology , Child , Child, Preschool , Female , Humans , IgA Deficiency/diagnosis , IgA Deficiency/epidemiology , Immunoglobulin A , Male , Retrospective Studies , Transglutaminases
4.
Rev. colomb. reumatol ; 28(4): 309-311, Dec. 2021. graf
Article in English | LILACS | ID: biblio-1423894

ABSTRACT

ABSTRACT Osteoarticular infections due to anaerobes are very rare in children, with the Fusobacterium genus being the most frequently isolated. The course is usually subacute and, although there are predisposing factors described, most patients do not present with them. Generally, joint fluid cultures are sterile since these microorganisms are very sensitive to contact with oxygen, so they require specific culture media. All of the above causes the diagnosis to be delayed, increasing the risk of long-term sequelae. However, the prognosis improves when treatment is started early. The case is presented of a 10-year-old patient who was admitted for 30 days due septic arthritis of the right hip caused by Fusobacterium nucleatum During the admission, he required three surgical interventions, and completed 6 weeks of effective antibiotic therapy, with a good outcome and remaining asymptomatic at the current time.


RESUMEN Las infecciones osteoarticulares por anaerobios son muy raras en los niños, siendo el género Fusobacterium el que se aisla con más frecuencia. El curso suele ser subagudo y, aunque hay factores predisponentes descritos, la mayoría de los pacientes no los presenta. Generalmente, los cultivos de liquido articular son estériles ya que estos microorganismos son muy sensibles al contacto con el oxigeno, por lo que precisan medios de cultivo específicos. Todo lo anterior hace que el diagnóstico se retrase y que el riesgo de secuelas a largo plazo aumente. Sin embargo, el pronóstico mejora cuando el tratamiento se inicia de modo precoz. Por todo ello, presentamos el caso de un paciente de 10 anos con una artritis séptica de cadera derecha por Fusobacterium nucleatum que permaneció ingresado 30 dias. Durante el ingreso precisó 3 intervenciones quirúrgicas y cumplió 6 semanas de antibioterapia efectiva, con buena evolución; permanece asintomático en el momento actual.


Subject(s)
Humans , Male , Child , Osteomyelitis , Bone Diseases, Infectious , Child , Musculoskeletal Diseases , Persons
5.
Pediatr Rheumatol Online J ; 19(1): 162, 2021 Nov 27.
Article in English | MEDLINE | ID: mdl-34838054

ABSTRACT

BACKGROUND: This study aimed to assess the baseline characteristics and clinical outcomes of coronavirus disease 2019 (COVID-19) in pediatric patients with rheumatic and musculoskeletal diseases (RMD) and identify the risk factors associated with symptomatic or severe disease defined as hospital admission, intensive care admission or death. METHODS: An observational longitudinal study was conducted during the first year of the SARS-CoV-2 pandemic (March 2020-March 2021). All pediatric patients attended at the rheumatology outpatient clinics of six tertiary referral hospitals in Madrid, Spain, with a diagnosis of RMD and COVID-19 were included. Main outcomes were symptomatic disease and hospital admission. The covariates were sociodemographic and clinical characteristics and treatment regimens. We ran a multivariable logistic regression model to assess associated factors for outcomes. RESULTS: The study population included 77 pediatric patients. Mean age was 11.88 (4.04) years Of these, 30 patients (38.96%) were asymptomatic, 41 (53.25%) had a mild-moderate COVID-19 and 6 patients (7.79%) required hospital admission. The median length of hospital admission was 5 (2-20) days, one patient required intensive care and there were no deaths. Previous comorbidities increased the risk for symptomatic disease and hospital admission. Compared with outpatients, the factor independently associated with hospital admission was previous use of glucocorticoids (OR 3.51; p = 0.00). No statistically significant risk factors for symptomatic COVID-19 were found in the final model. CONCLUSION: No differences in COVID-19 outcomes according to childhood-onset rheumatic disease types were found. Results suggest that associated comorbidities and treatment with glucocorticoids increase the risk of hospital admission.


Subject(s)
Antirheumatic Agents/therapeutic use , COVID-19/physiopathology , Glucocorticoids/therapeutic use , Hospitalization/statistics & numerical data , Rheumatic Diseases/drug therapy , Adolescent , Arthritis, Juvenile/drug therapy , Arthritis, Juvenile/epidemiology , Asthma/epidemiology , COVID-19/epidemiology , Carrier State/epidemiology , Child , Cohort Studies , Comorbidity , Female , Heart Diseases/epidemiology , Hereditary Autoinflammatory Diseases/drug therapy , Hereditary Autoinflammatory Diseases/epidemiology , Humans , Intensive Care Units, Pediatric , Length of Stay , Logistic Models , Longitudinal Studies , Lupus Erythematosus, Systemic/drug therapy , Lupus Erythematosus, Systemic/epidemiology , Male , Multivariate Analysis , Obesity/epidemiology , Renal Insufficiency, Chronic/epidemiology , Rheumatic Diseases/epidemiology , Risk Factors , SARS-CoV-2 , Severity of Illness Index , Spain/epidemiology
7.
Arch. argent. pediatr ; 118(2): e183-e187, abr. 2020. ilus
Article in Spanish | BINACIS, LILACS | ID: biblio-1100433

ABSTRACT

La epilepsia refleja por agua caliente es un tipo de convulsión poco frecuente cuya fisiopatología se desconoce. Estas crisis reflejas suelen iniciarse pocos segundos tras el contacto con el agua y, comúnmente, a temperaturas de 37-48 ºC. Los automatismos y las crisis parciales complejas, con o sin generalización secundaria, son el modo de manifestación principal.La exploración neurológica y el electroencefalograma intercrítico no suelen presentar alteraciones, lo que puede condicionar un retraso en el diagnóstico, por lo que es fundamental la sospecha clínica.El tratamiento antiepiléptico se inicia cuando se asocia a otro tipo de epilepsia o cuando ciertas medidas sencillas, como el descenso de la temperatura del agua en el baño, no controlan las crisis. Es posible la desaparición espontánea. Cuando es necesaria la terapéutica farmacológica, existe, normalmente, buena respuesta.Se presenta el caso de un lactante con diagnóstico de epilepsia refleja por agua caliente.


Hot-water epilepsy is a rare type of seizure whose pathophysiology is unknown. These reflex seizures usually begin a few seconds after contact with water, commonly at temperatures between 37-48 ºC. Automations and complex partial crises, with or without secondary generalization, are the main manifestation mode of this type of reflex epilepsies.Neurological examination and intercritical electroencephalography are usually normal, which may condition a delay in diagnosis, and the clinical suspicion is fundamental.Antiepileptic treatment is initiated when associated with another type of epilepsy or when certain simple measures, such as lowering the water temperature in the bath, do not control crises. Spontaneous disappearance is possible; when pharmacological therapy is necessary, there is usually a good response.We present the case of an infant diagnosed with hot-water epilepsy.


Subject(s)
Humans , Male , Infant , Epilepsy, Reflex/diagnosis , Seizures , Baths , Epilepsy, Reflex/drug therapy , Hot Temperature
8.
Arch Argent Pediatr ; 118(2): e183-e187, 2020 04.
Article in Spanish | MEDLINE | ID: mdl-32199061

ABSTRACT

Hot-water epilepsy is a rare type of seizure whose pathophysiology is unknown. These reflex seizures usually begin a few seconds after contact with water, commonly at temperatures between 37-48 ºC. Automations and complex partial crises, with or without secondary generalization, are the main manifestation mode of this type of reflex epilepsies. Neurological examination and intercritical electroencephalography are usually normal, which may condition a delay in diagnosis, and the clinical suspicion is fundamental. Antiepileptic treatment is initiated when associated with another type of epilepsy or when certain simple measures, such as lowering the water temperature in the bath, do not control crises. Spontaneous disappearance is possible; when pharmacological therapy is necessary, there is usually a good response. We present the case of an infant diagnosed with hot-water epilepsy. Key words: seizures, reflex epilepsies, hot water-induced reflex epilepsy.


La epilepsia refleja por agua caliente es un tipo de convulsión poco frecuente cuya fisiopatología se desconoce. Estas crisis reflejas suelen iniciarse pocos segundos tras el contacto con el agua y, comúnmente, a temperaturas de 37-48 ºC. Los automatismos y las crisis parciales complejas, con o sin generalización secundaria, son el modo de manifestación principal. La exploración neurológica y el electroencefalograma intercrítico no suelen presentar alteraciones, lo que puede condicionar un retraso en el diagnóstico, por lo que es fundamental la sospecha clínica. El tratamiento antiepiléptico se inicia cuando se asocia a otro tipo de epilepsia o cuando ciertas medidas sencillas, como el descenso de la temperatura del agua en el baño, no controlan las crisis. Es posible la desaparición espontánea. Cuando es necesaria la terapéutica farmacológica, existe, normalmente, buena respuesta. Se presenta el caso de un lactante con diagnóstico de epilepsia refleja por agua caliente.


Subject(s)
Epilepsy, Reflex/diagnosis , Hot Temperature/adverse effects , Water/adverse effects , Electroencephalography , Epilepsy, Reflex/etiology , Humans , Infant , Male
9.
Arch. argent. pediatr ; 115(6): 420-423, dic. 2017. tab
Article in Spanish | LILACS, BINACIS | ID: biblio-887405

ABSTRACT

El linezolid es un antibiótico de la familia de las oxazolidinonas, que actúa inhibiendo la síntesis proteica. Se emplea en infecciones graves por cocos Gram-positivos multirresistentes. Sus principales efectos secundarios son los gastrointestinales y, con menor frecuencia, la neuropatía periférica, la acidosis láctica y la mielosupresión. Se presenta el caso clínico de un niño de 12 años con diagnóstico de osteoartritis séptica de cadera derecha con osteomielitis femoral en tratamiento con linezolid, que presentó un cuadro de intolerancia digestiva, asociado a astenia y pérdida de peso. Presentaba, además, anemia normocítica, junto con leucopenia y trombopenia leves, con datos sugestivos de alteración de la hematopoyesis en el frotis sanguíneo, sugerente de toxicidad por fármacos. El cuadro se resolvió con la interrupción de la administración del fármaco. La mielosupresión reversible asociada a linezolid se relaciona con tratamientos prolongados (> 28 días), por lo que son necesarios los controles hematológicos periódicos durante ellos.


Linezolid is an antibiotic of oxazolidinones family that inhibits proteical synthesis. It is used in several Gram-positive multirresistent infections. Its more frequent side effects are gastrointestinal, followed by peripheral neuropathy and myelosuppression. We report the case of a 12-year-old boy diagnosed with septic osteoarthritis of the hip and femoral osteomyelitis, following treatment with linezolid, who complained about digestive intolerance and weight loss. He showed severe normocytic anemia and mild leukopenia and thrombocytopenia with data of hematopoiesis disorder in the blood smear that suggested drug toxicity. These findings reverted when the treatment was discontinued. Reversible myelosuppression associated with linezolid is related to long treatments (more than 28 days). So it is necessary to check the blood count during long treatments.


Subject(s)
Humans , Male , Child , Osteomyelitis/drug therapy , Pseudomonas Infections/drug therapy , Thrombocytopenia/chemically induced , Osteoarthritis, Hip/drug therapy , Linezolid/adverse effects , Leukopenia/chemically induced , Anti-Bacterial Agents/adverse effects , Bone Marrow/drug effects , Femur
10.
Arch Argent Pediatr ; 115(6): e420-e423, 2017 12 01.
Article in Spanish | MEDLINE | ID: mdl-29087126

ABSTRACT

Linezolid is an antibiotic of oxazolidinones family that inhibits proteical synthesis. It is used in several Gram-positive multirresistent infections. Its more frequent side effects are gastrointestinal, followed by peripheral neuropathy and myelosuppression. We report the case of a 12-year-old boy diagnosed with septic osteoarthritis of the hip and femoral osteomyelitis, following treatment with linezolid, who complained about digestive intolerance and weight loss. He showed severe normocytic anemia and mild leukopenia and thrombocytopenia with data of hematopoiesis disorder in the blood smear that suggested drug toxicity. These findings reverted when the treatment was discontinued. Reversible myelosuppression associated with linezolid is related to long treatments (more than 28 days). So it is necessary to check the blood count during long treatments.


El linezolid es un antibiótico de la familia de las oxazolidinonas, que actúa inhibiendo la síntesis proteica. Se emplea en infecciones graves por cocos Gram-positivos multirresistentes. Sus principales efectos secundarios son los gastrointestinales y, con menor frecuencia, la neuropatía periférica, la acidosis láctica y la mielosupresión. Se presenta el caso clínico de un niño de 12 años con diagnóstico de osteoartritis séptica de cadera derecha con osteomielitis femoral en tratamiento con linezolid, que presentó un cuadro de intolerancia digestiva, asociado a astenia y pérdida de peso. Presentaba, además, anemia normocítica, junto con leucopenia y trombopenia leves, con datos sugestivos de alteración de la hematopoyesis en el frotis sanguíneo, sugerente de toxicidad por fármacos. El cuadro se resolvió con la interrupción de la administración del fármaco. La mielosupresión reversible asociada a linezolid se relaciona con tratamientos prolongados (> 28 días), por lo que son necesarios los controles hematológicos periódicos durante ellos.


Subject(s)
Anti-Bacterial Agents/adverse effects , Leukopenia/chemically induced , Linezolid/adverse effects , Osteoarthritis, Hip/drug therapy , Osteomyelitis/drug therapy , Pseudomonas Infections/drug therapy , Thrombocytopenia/chemically induced , Bone Marrow/drug effects , Child , Femur , Humans , Male
11.
Pediatr. aten. prim ; 18(70): 165-170, abr.-jun. 2016. ilus
Article in Spanish | IBECS | ID: ibc-153805

ABSTRACT

Se presenta el caso de un niño de ocho años que desarrolla una piomiositis de los músculos obturador interno y externo, coincidiendo con una faringoamigdalitis estreptocócica. Inicialmente presenta solo dolor en miembro inferior izquierdo, siendo diagnosticado de contractura del bíceps femoral. Pocos días más tarde comienza con odinofagia, exantema cutáneo escarlatiniforme, fiebre y exudado amigdalar. Llama la atención la persistencia de dolor intenso en la pierna izquierda, dolor lumbar bajo y signo de Lassègue positivo, por lo que se realiza una resonancia magnética en la que se observa la piomiositis. Se realiza una revisión de los casos de piomiositis descritos recientemente, observándose un aumento de la incidencia en nuestro entorno. Se compara la clínica con dichos casos para intentar identificar signos clínicos que puedan ayudar a un diagnóstico precoz del proceso (AU)


We present the case of an eight-year-old child who developed a pyomyositis of the internal and external obturator muscles and streptococcal tonsillitis simultaneously. Initially, he only presented pain in the lower left limb and he was diagnosed a contracture of the biceps femoris muscle. A few days after, he began with odynophagia, cutaneous exanthema and fever with tonsillar exudate. However, the pain in the left leg persisted, lower lumbar pain and Lassègue sign appeared, so we underwent a magnetic resonance imaging demonstrating the pyomyositis. We reviewed recently described cases of pyomyositis, and we observed an increase of their incidence in our environment. A comparison is made with other case reviews to identify the clinical symptoms that could help in diagnosing the condition early (AU)


Subject(s)
Humans , Male , Child , Pyomyositis/drug therapy , Pyomyositis , Pharyngitis/complications , Pharyngitis/drug therapy , Tonsillitis/complications , Tonsillitis/diagnosis , Tonsillitis/drug therapy , Streptococcus pyogenes , Streptococcus pyogenes/isolation & purification , Anti-Bacterial Agents/therapeutic use , Obturator Nerve , Pyomyositis/complications , Obturator Nerve/pathology , Obturator Nerve , Exanthema/complications , Primary Health Care/methods , Magnetic Resonance Imaging/methods
12.
Rev. chil. endocrinol. diabetes ; 2(2): 102-107, abr. 2009. tab
Article in Spanish | LILACS | ID: lil-612495

ABSTRACT

Psychotropic medications have a series of neurobiological effects which may be related to adverse effects. The endocrinological side effects may affect prolactin and thyroid, parathyroid and antidiuretic hormones. They may also influence the appearance of metabolic syndrome, gonadal and sexual problems. There disturbances must be borne in mind to prevent or detect them on time,since they may affect the compliance with the treatment. This revision focuses on the relationship between psychotropic drugs, antidepressants, mood stabilizers and gonadal function. As a general recommendation, patients using these medications should be monitored for menstrual and fertility disturbances, weight change, hirsutism, galactorrhea and changes in libido and sexual life.


Subject(s)
Humans , Male , Female , Gonads , Psychotropic Drugs/pharmacology , Antidepressive Agents/pharmacology , Anticonvulsants/pharmacology , Sexual Dysfunction, Physiological/chemically induced , Prolactin , Prolactin , Psychotropic Drugs/adverse effects , Sexuality , Mental Disorders/drug therapy
13.
Ann Pharmacother ; 42(5): 640-6, 2008 May.
Article in English | MEDLINE | ID: mdl-18413692

ABSTRACT

BACKGROUND: Severe forms of alpha(1)-antitrypsin (AAT) deficiency require augmentation therapy by intravenous administration of purified preparations of AAT concentrate. Although standard AAT treatment schedules are widely available, pharmacokinetic studies characterizing AAT serum decay are scarce, and data on the variability of individual patients are almost nonexistent. OBJECTIVE: To establish individual AAT pharmacokinetics and develop a predictive model based on simple pharmacokinetic characterization that can be used to optimize individual AAT dosing regimens. METHODS: Seven patients with severe hereditary AAT deficiency (PI(*)ZZ phenotype) with serum AAT levels less than 0.50 g/L initially received AAT 180 mg/kg every 3 weeks. At 7, 14, and 21 days after AAT administration, serum samples were taken for quantitative AAT analysis and further one-compartment pharmacokinetic analysis. Subsequently, patients were rescheduled (dose and dosing interval) according to their individual responses. The influence of baseline AAT level, age, sex, body weight, and commercial AAT preparation was evaluated. RESULTS: The mean +/- SD AAT pharmacokinetic profile was: volume of distribution 127.6 +/- 31.9 mL/kg, clearance 10.13 +/- 1.84 mL/kg/day, and half-life 8.7 +/- 1.0 days. Hence, the mean optimized final AAT dose was 123.1 mg/kg every 2 weeks (range 118.5-125.6). AAT concentrations differed by a mean (geometrical) value of 3.9% (range -4.2% to 6.7%) from the minimum desired AAT serum trough of 0.50 g/L. The impact of baseline AAT levels and commercial AAT preparation used was statistically significant (p = 0.033 and p = 0.035, respectively). Differences between estimated and actual values were slightly lower when baseline AAT levels were taken into consideration, with a mean value of 3.3% (range -4.2% to 6.1%). CONCLUSIONS: AAT augmentation therapy can be effectively individualized on a pharmacokinetic basis with a simple, easily executed method.


Subject(s)
alpha 1-Antitrypsin Deficiency/blood , alpha 1-Antitrypsin Deficiency/drug therapy , alpha 1-Antitrypsin/administration & dosage , alpha 1-Antitrypsin/pharmacokinetics , Adult , Dose-Response Relationship, Drug , Drug Administration Schedule , Female , Humans , Infusions, Intravenous , Male , Middle Aged , alpha 1-Antitrypsin/blood
14.
Rev Med Chil ; 134(8): 973-80, 2006 Aug.
Article in Spanish | MEDLINE | ID: mdl-17130984

ABSTRACT

BACKGROUND: Eating disorders may have serious organic consequences derived from under nutrition, specific nutrient deficiencies and electrolytic disturbances and reach a mortality as high as 12%. AIM: To describe the features and outcome after six months of treatment of patients attending the Eating Disorders Unit at the Catholic University Hospital in Chile. MATERIAL AND METHODS: Review of medical records of patients with eating disorders that received a multidisciplinary treatment by a team of nutritionists, psychiatrists and psychologists and were seen at least twice in a period of six months. RESULTS: The records of 81 patients (mean age 16.3+/-3 years, only one male) were included in the review. Forty nine patients had Anorexia Nervosa of the restricting type, five of the purging type, 22 had Bulimia Nervosa of the purging type and two of the non purging type and three patients had an eating disorder not otherwise specified. Eighty percent consulted within the first year of symptom appearance. Sixty five percent came from families with both parents living together, 57% had a rigid and agglutinated family structure and 65% had occult crises. Depression or dysthymia was found in 45% of patients and drug therapy was required at the beginning of treatment in 25%. Obsessive traits (40.4%) were significantly related to restrictive eaters. A past history of obesity or overweight was common. After six months of treatment, body mass index increasing significantly in restrictive eaters with obsessive traits and occult family crises was noticed. CONCLUSIONS: A multidisciplinary therapeutic intervention improved body mass index and family symptoms in patients with restrictive eating disorders.


Subject(s)
Family Therapy , Feeding and Eating Disorders/therapy , Personality Disorders/psychology , Adolescent , Adult , Age Distribution , Anorexia Nervosa/epidemiology , Anorexia Nervosa/psychology , Anorexia Nervosa/therapy , Body Mass Index , Bulimia/epidemiology , Bulimia/psychology , Bulimia/therapy , Child , Chile/epidemiology , Combined Modality Therapy/methods , Feeding and Eating Disorders/epidemiology , Feeding and Eating Disorders/psychology , Female , Follow-Up Studies , Humans , Male , Personality Disorders/diagnosis , Psychiatric Status Rating Scales , Sex Distribution , Treatment Outcome
16.
s.l; s.n; 13 dic. 1982. 63 p. tab.
Non-conventional in Spanish | LILACS | ID: lil-86231

ABSTRACT

El presente trabajo tiene por objetivo determinar las diferentes causas de angustia en el paciente, familia, personal medico y de enfermeria en el servicio de urgencias del Hospital San Jose de Bogota. El personal asignado al servicio de urgencias esta distribuido en tres turnos: manana, tarde y noche. Ademas del personal fijo de urgencias se cuenta con la colaboracion de los especialistas fijos de turno. El paciente que ingresa al servicio de urgencias del Hospital de San Jose, se siente angustiado principalmente por estar lejos de sus familiares, por el costo de hospitalizacion y por los cambios que debe realizar en sus actividades; ademas siente temor a dolor y la muerte. Desde el ingreso al servicio el paciente y la familia experimentan angustia, y aun antes; lo que nos hace ver la necesidad de tener una persona destinada a orientar y a informar al paciente y familia. Es necesario que el personal reconozca la necesidad del paciente y de la familia de tener informacion sobre su situacion para sentirse mas seguro e incluso colaborar mas en su recuperacion. Los pacientes consideran que el trato de los medicos es mas cordial que el dado por el personal de enfermeria. Cualquier procedimiento que se le realice al paciente le causa angustia, y mas si no sabe en que consiste el procedimiento y si este les va producir dolor. El personal medico y de enfermeria debe tener siempre presente que el paciente le teme a todo aquello que le es desconocido, por lo tanto les debe dar una explicacion de los procedimientos a realizar. Uno de los factores ambientales que mas..


Subject(s)
Adult , Humans , Male , Female , Emergency Service, Hospital , Institutionalization , Stress, Psychological , Colombia , Health Surveys , Professional-Family Relations , Professional-Patient Relations
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