Demographic and clinical characterization of pediatric group patients with inborn errors of the immune system in a Colombian tertiary hospital

Introduction: In recent decades, there has been a growing increase in the diagnosis of patients with inborn errors of the immune system, formerly known as primary immunodeficiency dis-orders (PIDs). Timely diagnosis remains a challenge due to low clinical suspicion and poor edu-cation on the subject. It is estimated that between 70% and 90% of these pathologies remain underdiagnosed in our environment.Objective: The objective of this study is to characterize the demographic and clinical presen-tation of pediatric group patients with inborn errors of the immune system in a Colombian tertiary hospital.Methods: Retrospective descriptive study of 306 patients with a diagnosis of innate errors of the immune system who consulted the PID clinic between 2011 and 2018 in a high-complexity institution in Cali, Colombia.Results: Three-hundred and six patients were included. The median age was 4 years (IQR 2.3–7.7 years), and 59.5% of the patients were male. According to the International Union of Immunological Societies Expert Committee for Primary Immunodeficiency classification for inborn errors of the immune system, the most common group was antibody deficiency in 74.8% (n=229), especially in the age group between 1 and 5 years. The least frequent in our pop-ulation was complement deficiency. Of the warning signs stipulated for these pathologies, the most frequent were the (1) need for intravenous antibiotics (32%), (2) difficulty growing (15.7%), (3) four or more episodes of ear infection (10.8%), and (4) abscesses in organs or cuta-neous abscesses (12.7%). No patient reported two or more episodes of pneumonia or sinusitis, and only 5.8% of the patients received a bone marrow transplant (AU)

Conocimiento tradicional de las vendedoras de plantas medicinales de la ciudad de Sucre-Bolivia / Traditional knowledge of the medicines of medicinal plants of the city of Sucre-Bolivia

En Bolivia se práctica la medicina tradicional por su alta diversidad cultural y biológica. Las plantas medicinales son comercializadas en Sucre-Bolivia mediante puestos urbanos atendidos por mujeres de origen quechua y castellano el objetivo es saber los conocimientos y usos de las plantas medicinales. La recolección de información fue por medio de entrevistas, con cinco preguntas básicas en los meses de julio-agosto. Se identificó que las señoras que tienen puesto fijo tienen de 90 a 110 especies de plantas medicinales y las señoras ambulantes que en su mayoría se encuentran en el mercado campesino tienen poca variedad de plantas medicinales de 5 a 10 especies, estas ambulantes en su totalidad son de orígenes indígenas es porque ellas siembran y cosechan sus propias plantas que en este caso son plantas con características medicinales en cambio las que tienen puesto fijo, también son las que tienen más años de experiencia traen plantas de La Paz (yungas), Cochabamba ya que estas ciudades producen mayor cantidad de plantas medicinales, también son traídas de los alrededores de sucre como Yotala, Icla, Jatun Mayu, Ravelo y del valle que son exportadas para su venta. Es así que estos recursos genéticos representan oportunidades para impulsar el desarrollo económico, enmarcado dentro la sostenibilidad y equidad social.

In Bolivia, traditional medicine is practiced because of its high cultural and biological diversity. The medicinal plants are commercialized in Sucre-Bolivia through urban posts served by women of Quechua and Spanish origin. The objective is to know the knowledge and uses of the medicinal plants. The information was collected through interviews, with five basic questions in the months of July-August. It was identified that the ladies who have a fixed position have 90 to 110 species of medicinal plants and the traveling ladies who are mostly in the peasant market have little variety of medicinal plants of 5 to 10 species, these ambulants in their entirety are of indigenous origins it is because they sow and harvest their own plants that in this case are plants with medicinal characteristics instead those that have a fixed position, they are also those that have more years of experience bring plants from La Paz (yungas), Cochabamba since These cities produce more medicinal plants, they are also brought from the surroundings of Sucre such as Yotala, Icla, Jatun Mayu, Ravelo and the valley that are exported for sale. Thus, these genetic resources represent opportunities to boost economic development, framed within sustainability and social equity.

Candida isolation in patients hospitalized on a surgical ward: significance and mortality-related factors.

BACKGROUND: Infections caused by Candida are an emerging pathology on surgical wards. The aim of the present study was to evaluate the incidence, characteristics, and predictive factors of mortality in patients colonized and/or infected by Candida spp. in this setting. METHODS: A consecutive series of 105 patients hospitalized on a general surgery ward between 2000 and 2004 were included, and 118 positive cultures for Candida were identified. The variables age, sex, previous medical history, current disease, anemia, ICU stay, type and localization of the microorganism, need for parenteral nutrition, and transfusions were recorded. The primary outcome was in-hospital mortality. A univariate analysis was performed to determine which of these variables were associated with mortality. With a logistic regression model, independent prognostic factors of mortality were determined. RESULTS: The prevalence of patients colonized and/or infected by Candida on our surgical ward was 0.98% (CI 95%: 0.79-1.17), and the incidence was 49 cases per 1,000 patient-years. Of the 105 patients in this series, 56 were men (53%) and 49 women (47%); the mean age was 63.8 years (SD +/- 15.7). Twelve patients (11.4%) had candidemia. Crude mortality was 23% (24 patients), whereas the mortality attributable to candidemia was 25% (3/12 cases). Anemia (p = 0.001); transfusions (p = 0.003), and an ICU stay (p = 0.002) were associated with mortality. Candidemia was associated with neoplasms (p = 0.02) and the infection caused by Candida parapsilosis (p = 0.04). The only independent factor related to mortality was the anemia (p = 0.028; Odds Ratio: 6.43; 95% CI: 1.23-33.73). CONCLUSIONS: Colonization and/or infection by Candida spp. in non-ICU hospitalized surgical patients implies a relative high mortality. Anemia is an independent factor for mortality.

Neumotórax en el recién nacido: experiencia de un servicio de cirugía de tórax / Pneumothorax in the newborn: experience of a thoracic surgery department

Objetivos: Evaluar la experiencia del Servicio de Cirugía de Tórax del Hospital Universitario de Maracaibo en el diagnóstico y tratamiento del neumotórax en el recién nacido. Métodos: 23 recién nacidos con neumotórax fueron evaluados por el Servicio de Cirugía de Tórax en la Emergencia Pediátrica, Unidad de Cuidados Intensivos Pediátricos y en el Servicio de Neonatología entre los años 2000–2004. Se recolectaron los datos relacionados con: la duración de la gestación, complicaciones maternas durante el embarazo, examen físico, estudios diagnósticos, tratamiento yevolución de los pacientes. Resultados: 13(56.52%) delos recién nacidos eran varones y la edad gestacional promedio fue de 35.25 +/- 2.49 semanas. 12(52.17%) nacieron por cesárea y 10(43.47%) tenían antecedentes de maniobras de reanimación al nacer. El neumotórax se localizó en el hemitórax izquierdo en 8 recién nacidos (34.78%), hemitórax derecho en 14 recién nacidos (60.86%) y en ambos hemitórax en 1 recién nacido (4.47%). 22 (95.65%) presentaron dificultad respiratoria El tratamiento incluyó una conducta expectante en 2 recién nacidos con neumotórax menor de 25%, y la colocación de tubo de toracostomía en 21 recién nacidos (91.30%) con neumotórax mayor de 25% Conclusión: el neumotórax es una complicación que se observa principalmente en el recién nacido con Apgar bajo al nacer o querequieren maniobras de reanimación y se presenta con dificultad respiratoria. Los neumotorax menores de 25% pueden manejarse con observación y radiología seriada, pero aquellos mayores de 25% requerirán colocación de tubo de toracostomía.

Objectives: To evaluate the experience of Thoracic Surgery Service in the diagnosis and treatment of pneumothorax in newborn at theHospital Universitario de Maracaibo.Methods: 23 newborn with pneumothorax were evaluated by the Thoracic Surgery Service in the Pediatric Emergency Unit, PediatricIntensive Care Unit and Neonatology Service between the years 2000-2004. Data was collected regarding gestational age, complications during the pregnancy, physical exam, diagnostic studies, treatment and evolution of the patients. Results: 13 (56.52%) were males, with mean gestational age of 35 +/- 2.5 weeks. 12 (52.17%) were born by caesarean section and 11(47.82%) by vaginal delivery. 10 newborns had history of resuscitation manoeuvres (43.47%). Pneumothorax was located in lefthemithorax in 8 newborn (34.78%), right hemithorax in 14 newborn (60.86%) and both hemithorax in 1 newborn (4.47%). 22 (95.65%)presented respiratory distress. Treatment included observation in 2 newborns with pneumothorax < 25%, and tube thoracostomy in 21 (91.30%) with pneumothorax > 25%. Conclusion: Pneumothorax is a complication seen mainly in newborns with low Apgar score and those who required resuscitation manoeuvres and almost always presents with respiratory distress. Pneumothorax < 25% can be treated with observation and serialradiology but those > 25%.will require tube thoracostomy.

[Splenic artery aneurysm. Review of two surgically operated cases]. / Aneurisma de la arteria esplénica. Revisión de dos casos intervenidos quirúrgicamente.

Among arterial aneurysms, splenic artery aneurysms are very uncommon in the current practice. The etiologic factors are in relation with: angiodysplasia, portal hypertension, pregnancy and atherosclerosis. The great majority are asymptomatic. Symptomatic cases are in relation with rupture in the peritoneum or in organs or structures of the vicinity. We present two cases of splenic artery aneurysm in a 57 year old man and in a 59 year old woman. In both cases the diagnosis was made by plain abdominal x-ray, abdominal scanner and abdominal aortography. Both cases were surgically treated by transverse laparotomy and total aneurysm resection. The pathology dictamen of the two cases was consistent with atherosclerosis. After 120 and 38 months of follow-up both patients are doing well and without evidence of splenic artery aneurysm. A review of this entity is exposed, with special reference in the etiology, symptomatology, diagnosis and treatment.

[Combined treatment of embolization followed by surgical resection in carotid paraganglioma]. / Tratamiento combinado de embolización seguido de resección quirúrgica en un paraganglioma carotídeo.

Carotid body tumors represent a relatively uncommon finding in vascular surgery. We present the case of a 62 years old man with a right carotid paraganglioma diagnosed by cervical ultrasound scanner, magnetic resonance imaging and carotid arteriography. We decided to treat the patient in two times. In a first approach the tumor was percutaneously embolized, later on in a second time the paraganglioma was surgically resected. The patient is doing well without echographic evidence of tumor 3 years after the operation. This modality of treatment, embolization followed by surgical excision is a useful and valuable alternative.

Aneurisma de la arteria esplénica. Revisión de dos casos intervenidos quirúrgicamente / Splenic artery aneurysm. Review of two surgically operated cases

Entre los aneurismas arteriales, los localizados en la arteria esplénica son de infrecuente aparición en la práctica clínica. La etiología puede ser debida y está en relación con: fibrodisplasia, hipertensión portal, embarazo y arteriosclerosis. La mayor parte son asintomáticos, otras veces la clínica es por la rotura y perforación del aneurisma en peritoneo libre o en un órgano o estructura vecina. Presentamos dos casos de aneurisma silente en la arteria esplénica en dos pacientes, un varón y una hembra de 57 y 59 años respectivamente. En ambos se hizo el diagnóstico por radiología de abdomen, TAC abdominal y arteriografía. Los dos se intervinieron quirúrgicamente por laparotomía transversa, practicándose resección completa del aneurisma. En los dos casos el diagnóstico anatomopatológico fue arteriosclerosis. Los pacientes están bien y sin evidencia de aneurisma visceral a los 120 y 38 meses respectivamente de la operación. Se comentan los aspectos clínicos, diagnósticos y el tratamiento de esta entidad

Among arterial aneurysms, splenic artery aneurysms are very uncommon in the current practice. The etiologic factors are in relation with: angiodisplasia, portal hypertension, pregnancy and atherosclerosis. The great majority are asymptomatic. Symptomatic cases are in relation with rupture in the peritoneum or in organs or structures of the vecinity. We present two cases of splenic artery aneurysm in a 57 year old man and in a 59 year old woman. In both cases the diagnosis was made by plain abdominal x-ray, abdominal scanner and abdominal aortography. Both cases were surgically treated by transverse laparotomy and total aneurysm resection. The pathology dictamen of the two cases was consistent with atherosclerosis. After 120 and 38 months of follow-up both patients are doing well and without evidence of splenic artery aneurysm. A review of this entity is exposed, with special reference in the etiology, symptomatology, diagnosis and treatment

Tratamiento combinado de embolización seguido de resección quirúrgica en un paraganglioma carotídeo / Combined treatment of embolization followed by surgical resection in carotid paraganglioma

Los paragangliomas carotídeos son un diagnóstico poco frecuente en patología vascular. Presentamos el caso de un paciente varón de 62 años con un paraganglioma carotídeo derecho diagnosticado por ecografía, TAC, RMN y arteriografía cervical que fue tratado en dos tiempos. En una primera fase se efecúó embolización percutánea del tumor evidenciándose arteriograficamente la disminución en su irrigación arterial. En una segunda fase se procedió a la resección quirúrgica. El resultado ha sido excelente estando el paciente asintomático y si evidencia de tumor al tumor al estudio ecográfico cervical a los 3 años. En casos seleccionados, ésta aproximación terapéutica nos parece de utilidad e interés en el tratamiento de este tipo de lesiones

Carotid body tumors represent a relatively uncommon finding in vascular surgery. We present the case of a 62 years old man with a right carotid paraganglioma diagnosed by cervical ultrasound scanner, magnetic resonance imaging and carotid arteriography. We decided to treat the patient in two times. In a first approach the tumor was percutaneously embolized, later on in a second time the paraganglioma was surgically resected. The patient is doing well without echographic evidence of tumor 3 years after the operation. This modality of treatment, embolization followed by surgical excision is a useful and valuable alternative

Effects of ovariectomy on the neuroendocrine axes regulating reproduction and energy balance in young cynomolgus macaques.

Degeneration of the ovary in middle-aged women results in castrate levels of ovarian steroids and increased gonadotropin secretion from the anterior pituitary gland. Ageing in women is also accompanied by significant changes in energy homeostasis. We have observed alterations in hypothalamic morphology and gene expression in older women, including hypertrophy and increased gene expression of neurokinin B (NKB) neurones, elevated levels of gonadotropin releasing-hormone (GnRH) mRNA and decreased numbers of neurones expressing pro-opiomelanocortin (POMC) mRNA. To determine if loss of ovarian steroids could produce comparable changes in gene expression in young primates, we measured the effects of ovariectomy on NKB, GnRH and POMC gene expression in young cynomolgus monkeys. We also measured serum leptin and body weight to examine the consequences of ovariectomy on energy balance. NKB neurones in the infundibular nucleus of ovariectomized monkeys were larger, more numerous and displayed increased levels of NKB mRNA compared to those of intact controls. Moreover, ovariectomy increased the number of neurones expressing GnRH gene transcripts and elevated serum luteinizing hormone. By contrast, several parameters related to energy balance, including POMC gene expression, serum leptin and body weights, were unchanged by ovariectomy. Thus, the rise in NKB and GnRH gene expression in older women was simulated by ovariectomy in monkeys, but the changes in POMC gene expression and energy balance were not. This study provides strong support for the hypothesis that ovarian failure contributes to the increased NKB and GnRH gene expression observed in postmenopausal women.

Anomalías útero-vaginales: presentación de 5 pacientes / Uterine-vaginal anomalies: a five case-report

Las anomalías en el sedarrollo de los conductos de Muller tienen una baja incidencia en la población, están determinadas por la interrupción del desarrollo del defecto de fusión de los mismos. Presentamos 5 niñas con duplicidad uterina, vagina con tabique asimétrico que ocluía una de ellas y que fueron tratadas en los últimos cinco años en el Servicio de Cirugía del Hospital Pediátrico Universitario "José Luis Miranda", se analizaron variables como la edad, sintomatología, examen físico, estudio realizado, tratamiento médico quirúrgico y evolución. Dos de ellas presentaron diagnóstico de tumor quístico de ovario, una con tumor hipogastrio sin menarquia y dos con hematocolpos. La ecografía y la cirugía videoendoscópica, asociadas a abordaje perineal permitió lograr mejor diagnóstico y tratamiento adecuado(AU)

Anomalías útero-vaginales: presentación de 5 pacientes / Uterine-vaginal anomalies: a five case-report

Las anomalías en el sedarrollo de los conductos de Muller tienen una baja incidencia en la población, están determinadas por la interrupción del desarrollo del defecto de fusión de los mismos. Presentamos 5 niñas con duplicidad uterina, vagina con tabique asimétrico que ocluía una de ellas y que fueron tratadas en los últimos cinco años en el Servicio de Cirugía del Hospital Pediátrico Universitario "José Luis Miranda", se analizaron variables como la edad, sintomatología, examen físico, estudio realizado, tratamiento médico quirúrgico y evolución. Dos de ellas presentaron diagnóstico de tumor quístico de ovario, una con tumor hipogastrio sin menarquia y dos con hematocolpos. La ecografía y la cirugía videoendoscópica, asociadas a abordaje perineal permitió lograr mejor diagnóstico y tratamiento adecuado

Demandas judiciales contra médicos en Chile / Lawsuits against physicians in Chile

Lawsuits against physicians are becoming relevant in Chile. We analized 18 trials against physicians that occured between 1977 and 1985 and that were ruled by Metropolitan Region courts. The causes of demands were death of patients in 8 trials and severe lesions in the rest. Twelve trials originated from surgical procedures (6 from gynecological interventions) and 9 came from private clinics. Nine trials lasted less than 2 years and 3, more than 5 years. Sentences was pronounced by criminal courts in 12 cases and by appealing courts in six

Effects of brief starvation on brain protease activity.

Changes in the activity of proteases (cathepsin D and calpains) caused by 48-h food withdrawal were studied in the brain, liver, kidney, spleen, and heart of 3-, 12-, and 24-month-old Fischer rats. Cathepsin D activity was similar in brain, liver, and heart of control animals; in kidney it was 5-fold higher and in spleen about 10-fold higher. With age, activity increased in all organs tested except spleen. Brief starvation caused no change of cathepsin D activity in brain, but caused an increase in liver and a decrease in spleen. Neutral proteolytic activity in control was highest in the pons-medulla-cerebellum fraction of brain, and activity in liver and heart was below that in brain. Activity increased with age in brain and decreased in other organs. Brief starvation in young animals caused an increase in activity in brain, and a decrease in liver and spleen. Isolated calpain II activity was high in control brain. It increased with age in the cerebrum. Brief starvation resulted in a decrease in the brain. The results indicate that the protease content of the brain is altered with age and in malnutrition, with changes not being the same for all proteases, and changes in brain being different from those in other organs.

Observations about amyotrophic lateral sclerosis and the parkinsonism-dementia complex of Guam with regard to epidemiology and etiology.

For more than 150 years, Chamorro natives of the Mariana Islands in the Western Pacific Ocean, have developed fatal paralysis in middle and later life, which we term amyotrophic lateral sclerosis/parkinsonism-dementia (ALS/PD). The cause of the disease might be exposure to seeds of the indigenous cycad. Motor system disease is induced in cynomolgus monkeys by feeding them beta-N-methylamino-L-alanine (BMAA), an amino acid present in cycad seeds. We believe that the cycad seeds which usually cause no immediate adverse symptoms when prepared and eaten as flour, or applied topically as medicine, can give rise to widespread and severe nerve cell degeneration after a latency of many decades. Furthermore, it may be that only a single exposure to this potent but silent toxin(s) can result in fatal neurological disease years later.

A reverse enzyme immunoassay for detection of Vespula spp. sensitivity.

The Reverse Enzyme Immunoassay (REIA) method for the detection of specific IgE recently described by us has been applied to the diagnosis of Vespula spp. sensitivity. To do so, 0.5 mg of Vespula venom of commercial origin was conjugated with peroxidase according to a previously described technique. The results obtained from the study of 50 nonallergic patients have demonstrated the specificity of the method in as much as no positive value was found. In addition, only one low positive value was obtained from a group of 47 atopic patients showing no evidence of sensitivity to insect stings. On the contrary, very high ELISA values from a group of 23 patients with suspected sensitivity to insect venom were obtained. With these results, we conclude that this method can constitute an aid in the diagnosis of these patients, besides being a low-cost method free from the interference of blocking antibodies. It is our opinion however, that this method is not as reliable as the RAST.

Living-related kidney donors: a multicenter study of donor education, socioeconomic adjustment, and rehabilitation.

To determine the consequences of living-related kidney donation, a study was conducted of 536 donors whose nephrectomies had been performed at nine geographically dispersed centers during the past 12 years. The data demonstrated that greater than 84.0% of the donors thought they had been adequately informed regarding all aspects of donation, and less than 15.0% reported being pressured in their decision. Only two serious medical complications were directly attributable to the surgery, greater than 92.0% of the donors believed their health had not been adversely affected by donation, and 96.8% reaffirmed their decision regardless of the graft's success or the financial distress they experienced (P greater than .05). However, greater than 14.0% experienced direct pressure, particularly not to donate. Donation also appeared to stress previously troubled marriages, especially among donors without a religious affiliation, who were pressured to donate by their families, or who borrowed from family members (P less than .05). Substantial unreimbursed expenses (greater than or equal to $1,000) were incurred by 43 donors, and 23.2% of all donors reported that donation caused a financial hardship.

Dietary lithium during development: changes in amino acid levels, ionic content, and [3H]spiperone binding in the brain of rats.

We found that chronic lithium diet affects the sensitivity of neuroleptic receptors and the content of amino acids in the brain, and that the changes in adult animals differ from those in young rats. Pregnant rats were kept on lithium diet (pellets with 0.21% Li2CO3 and 0.21% NaCl) during the gestation period and the offspring were kept on lithium for six weeks after delivery. Control rats were kept on normal diet under identical conditions. In corpus striatum and cerebral cortex of lithium-treated young rats a reduction in apparent dissociation constant and no change in (3H)spiperone total binding sites were found, suggesting a sensitization of the neuroleptic receptor; this result was unlike that obtained with adult lithium-treated rats, where the total number of binding sites was decreased. The lithium content of brain was very high (2.32 meq/kg of wet weight), whereas in the serum only 0.75 meq/l was recorded. K+ and Na+ levels increased by 20% and 9% respectively in the brain and remained at normal levels in the serum. Analysis of free amino acids in the cerebral cortex, midbrain, and cerebellum showed increases in GABA and glycine levels in all three regions, a significant increase in taurine in midbrain, and an increase in lysine in cerebral cortex and cerebellum. The results indicate that the effect of chronic dietary lithium given during pregnancy on the neuroleptic receptor in young rats is different from that in adult animals. It produces an increase in the number of the neuroleptic receptor sites instead of the decline in the number of binding sites found in adult rats. It remains to be established whether this effect is related more to the age of the animal tested or to the stage of development of the CNS at which the lithium was administered.