Cardiac iron overload evaluation in thalassaemic patients using T2* magnetic resonance imaging following chelation therapy: a multicentre cross-sectional study

Abstract Introduction Magnetic resonance imaging (MRI) T2* technique is used to assess iron overload in the heart, liver and pancreas of thalassaemic patients. Optimal iron chelation and expected tissue iron response rates remain under investigation. The objective of this study was to analyse serum ferritin and the iron concentration in the heart, liver and pancreas measured by MRI T2*/R2* during regular chelation therapy in a real-world cohort of patients with thalassemia. Methods We evaluated thalassaemic patients ≥ 7 years old undergoing chelation/transfusion therapy by MRI and assessed serum ferritin at baseline and follow-up from 2004-2011. Results We evaluated 136 patients, 92% major thalassaemic, with a median age of 18 years, and median baseline ferritin 2.033ng/ml (range: 59-14,123). Iron overload distribution was: liver (99%), pancreas (74%) and heart (36%). After a median of 1.2 years of follow-up, the iron overload in the myocardium reduced from 2,63 Fe mg/g to 2,05 (p 0.003). The optimal R2* pancreas cut-off was 148 Hertz, achieving 78% sensitivity and 73% specificity. However, when combining the R2* pancreas cut off ≤ 50 Hertz and a ferritin ≤ 1222 ng/ml, we could reach a negative predictive value (NPV) of 98% for cardiac siderosis. Only 28% were undergoing combined chelation at baseline assessment, which increased up to 50% on follow up evaluation. Conclusions Chelation therapy significantly reduced cardiac siderosis in thalassaemic patients. In patients with moderate/severe liver iron concentration undergoing chelation therapy, ferritin levels and myocardium iron improved earlier than the liver siderosis.

Indications et résultats de la splénectomie chez le drépanocytaire au Centre Hospitalier Universitaire de Libreville / Indications and results of splenectomy in sickle cell patients at the University Hospital Center of Libreville

Introduction : la drépanocytose est une affection génétique héréditaire responsable d'une déformation des hématies en «faucilles¼. Elle peut engendrer de nombreuses complications parmi lesquelles la séquestration splénique et l'hypersplénisme. La splénectomie fait partie de l'arsenal thérapeutique, induisant un état d'hyposplénie ou d'asplénie aux conséquences parfois mortelles, surtout chez le sujet drépanocytaire. Le but de ce travail mené au service de chirurgie du centre hospitalier universitaire de Libreville au Gabon, est de déterminer les indications et les résultats à moyen terme de la splénectomie chez le drépanocytaire.Matériel et méthodes : il s'agissait d'une étude rétrospective, descriptive et monocentrique allant d'octobre 2016 à avril 2019. Tous les dossiers des patients drépanocytaires opérés d'une splénectomie seule ou associée à un autre geste chirurgical étaient retenus.Résultats : au total treize patients ont été inclus dans notre étude. L'âge moyen des patients était de 10,7 ans [3-25 ans]. L'indication opératoire était l'hypersplénisme (8 cas), la séquestration splénique (3 cas), et l'infarctus splénique (2 cas). Tous les patients étaient opérés d'une splénectomie totale par laparotomie. La vaccinothérapie et l'antibioprophylaxie post-opératoire étaient réalisées chez tous les patients. Aucune complication post-opératoire immédiate ni aucun décès n'avaient été noté chez tous les patients.Conclusion : l'indication des splénectomies chez le drépanocytaire est dominée par l'hypersplénisme. Dans notre context la splénectomie totale par laparotomie semble être la règle. La vaccination prophylactique est bien administrée. La morbi-mortalité post-opératoire immédiate est nulle.

Introduction: Sickle cell disease is a genetic inherited disorder responsible for the deformation of red blood cells into"sickles". It can lead to many complications including splenic sequestration and hypersplenism. Splenectomy is the indicated gesture. It induces a state of hyposplenia or asplenia with dreadful consequences, sometimes fatal especially in this sickle cell patient. The aim of this work, conducted at the Surgery Department of Libreville University Hospital Center in Gabon, is to determine the indications and results of splenectomy in sickle cell patients.Material and methods: this was a retrospective, descriptive and single-center study from October 2016 to April 2019.All records of sickle cell patients undergoing splenectomy alone or with other surgical procedures were included.Results: a total of thirteen patients were included in our study. The average age of the patients was 10.7 years [3-25years]. The operative indication was hypersplenism (8 cases), splenic sequestration (3 cases), and splenic infarction(2 cases). All patients underwent total splenectomy under laparotomy. Vaccine therapy and postoperative antibiotic prophylaxis were performed in all patients. No immediatepostoperativecomplications or deaths were noted in all patients. Conclusion: the indication of splenectomies in sickle cell disease is dominated by hypersplenism. In our context, total splenectomy under laparotomy was the rule. Prophylactic vaccination is well administered. Immediate postoperative morbidity and mortality is nil.

Profil clinique, biologique et traitement des drépanocytaires régulièrement suivis au Centre Hospitalier Universitaire de Libreville / Clinical, biological and treatment profile of sickle cell patients regularly followed at Libreville University Hospital Center

Introduction: La mise en place depuis Septembre 2016 au Centre Hospitalier Universitaire (CHUL) d'une consultation d'hématologie dédiée aux adultes drépanocytaires a été l'occasion de mener cette étude dont le but principal était d'établir les profils clinique et paraclinique de l'adulte drépanocytaire régulièrement suivi.Patients et méthodes : Il s'agissait d'une étude rétrospective. La population d'étude était constituée de patients drépanocytaires homozygotes de 18 ans et plus, qui avaient effectués au moins trois consultations d'hématologie sur une année. Les informations recueillies, après étude du dossier médical et entretien téléphonique avec le patient, concernaient les données socio-démographiques, l'histoire de la drépanocytose, les antécédents, les complications de la maladie, les examens biologiques et radiologiques et le traitement.Résultats : Au total 88 patients répondaient aux critères d'inclusion sur les 233 drépanocytaires vus durant la période de l'étude. L'âge moyen était de 30,4 ± 7,8 ans. L'interrogatoire révélait que la crise vaso-occlusive (CVO) était la principale complication aiguë et la lithiase vésiculaire (36,3%) la première complication chronique. L'hémoglobine moyenne était de 7,8 g/dl et pour 49,3% des patients elle se situait entre 7 et 9 g/dl. Les leucocytes étaient augmentés dans 65,7%. L'échographie cardiaque réalisée chez 35 patients retrouvait 11,1% d'hypertension artérielle pulmonaire et 22,8% d'hypertrophie ventriculaire gauche. L'intensité de la crise douloureuse motivait une consultation au service des urgences du CHU dans 81,6% des cas dont plus de 62% déploraient un retard dans l'exécution de cette prise en charge bien qu'ils en aient été satisfaits dans 54,9% des cas.Conclusion : Les drépanocytaires adultes régulièrement suivis au CHUL sont peu nombreux. La transition entre le suivi pédiatrique et adulte doit se faire avec une transmission des informations du dossier médical sur l'histoire de la drépanocytose.

Introduction: The establishment since September 2016 at the University Hospital Center (CHUL) of a hematology consultation dedicated to adults with sickle cell disease was an opportunity to conduct this study, the main purpose of which was to establish the clinical and paraclinical profiles of the adults with sickle cell disease regularly monitored. Patients and methods: This was a retrospective study. The study population consisted of homozygous sickle cell patients aged 18 and over, who had performed at least three hematology consultations over a year. The information collected, after studying the medical file and telephone interview with the patient, concerned socio-demographic data, history of sickle cell disease, history, complications of the disease, biological and radiological examinations and treatment. Results: A total of 88 patients met the inclusion criteria out of the 233 sickle cell patients seen during the study period. The mean age was 30.4 ± 7.8 years. The questioning revealed that vaso-occlusive crisis (VOC) was the main acute complication and cholelithiasis (36.3%) the first chronic complication. The average hemoglobin was 7.8 g/dl and for 49.3% of the patients it was between 7 and 9 g/dl. Leukocytes were increased in 65.7%. Cardiac ultrasound performed in 35 patients found 11.1% pulmonary arterial hypertension and 22.8% left ventricular hypertrophy. The intensity of the painful crisis motivated a consultation in the emergency department of the CHU in 81.6% of cases, of which more than 62% complained of a delay in the execution of this care although they were satisfied with it in 54 .9% of cases.Conclusion: Few adult sickle cell sufferers are regularly monitored at the CHUL. The transition between pediatric and adult follow-up must be made with a transmission of information from the medical file on the history of sickle cell disease

Elevated Iron Chelating Activity of Ilex paraguariensis Leaf Infusion: in vitro and in vivo Pilot Studies

Abstract This study presents an Ilex paraguariensis leaf infusion with important potential as natural iron-chelating. The impact of infusion time and the water volume to obtain an Ilex paraguariensis leaf infusion with high phenolic content and iron chelating activity, such as the stability of these proprieties in the storage time and temperature (immediately and after 24 h at 8 and 25 (C) were assessed. The acute consumption effect of this infusion to reduce iron absorption in vivo was also evaluated. A preliminary crossover trial with volunteers that ingested a meal containing non-haem iron (11.4 mg) with the treatments: Ilex paraguariensis leaf infusion with the highest phenolic content and iron chelating activity (200 mL) or control (200 mL water). Blood samples were withdrawn before and 1, 2, 3 and 4 h after the meal for serum iron measurement. The highest phenolic content (18.1 mg/mL) and iron chelating activity ((100%) were observed for 10 min infusion time using 30 g leaves/300 mL water. Storage at 8 or 25 (C for 24 h decreased total phenolics and di-caffeoylquinic acids by 23.5% and 25.5%, respectively (p< 0.05), without affecting the iron-chelating activity due to a saturating chelating effect at 3.34 mg/mL phenolic content. Inhibition of the iron absorption in vivo by infusion was 78% considering the iron recovery at peak maximum. The in vitro and preliminary in vivo results showed a functional property of the Ilex paraguariensis leaf infusion that may be useful for adjuvant management of iron overload diseases.

Prospecção de sideróforos do tipo hidroxamato e quinona para terapia de sobrecarga de ferro / Prospection of hydroxamate and quinone-type siderophores for iron overload chelation therapy

A sobrecarga de ferro é uma condição prejudicial para os pacientes, que apresentam uma diminuição significativa na qualidade de vida. Os fármacos quelantes são moléculas que têm capacidade de uso clínico para atuar como atenuadores da sobrecarga de metais. Neste trabalho apresentamos uma análise de sideróforos do tipo hidroxamato e quinona, com o objetivo de ampliar a gama de terapia de sobrecarga de ferro. Para cada composto foi realizado um ensaio competitivo com a sonda calce- ína para verificar a capacidade de ligação do ferro, e um ensaio antioxidante baseado na supressão da oxidação dependente de ferro da dihidrorrodamina (DHR) sob ascorbato. Foi observado que o hidroxamato cíclico piridoxatina apresentou capacidade de sequestrar ferro de substratos de alta afinidade, tanto em meio tamponado quanto em meio intracelular. Em ambas as situações também se mostrou um antioxidante eficiente. Entretanto, parece ser o mais tóxico do grupo dos hidroxamatos (que ainda continha o hidroxamato linear desferricoprogênio e o aromático desferriastercromo). Outros compostos naturais também foram estudados como possíveis candidatos a fármacos para sobrecarga de ferro. Complexos de ferro foram caracterizados por espectrofotometria para avaliar a estequiometria possível, considerando os sítios de ligação para cada composto. Ensaios de fluorescência revelaram que entre os quatro compostos em estudo (ácido clorogênico, lapachol, hemateína e hematoxilina), o complexo entre ferro e hemateína apresenta maior estabilidade relativa do que outros

Iron overload is a harmful condition for patients, who have a significant decrease in life quality. Chelating drugs are molecules that have the capacity for clinical use to act as attenuators of metal overload. In this work we present an analysis of hydroxamate and quinone-type siderophores, intending to broaden the range of iron overload therapy. For each compound it was conducted a competitive assay with the fluorescent probe calcein to verify the iron binding ability, and an antioxidant assay based on suppression of the iron-dependent oxidation of dihydrorhodamine (DHR) under ascorbate. It was observed that cyclic hydroxamate pyridoxatin displayed good ability to scavenge iron from high affinity substrates both in buffer and in intracellular medium. It was also an efficient antioxidant in both setups. However, pyridoxatin seems to be the most toxic from the hydroxamate group (composed also by the linear desferricoprogen and the aromatic desferriasterchrome). Other natural compounds have also been studied as possible candidates for iron-overload drug therapy. Iron complexes were characterized by spectrophotometry to assess the possible stoichiometry considering the binding sites for each compound. Fluorescence assays revealed that among the four compounds in study (chlorogenic acid, lapachol, hematein and hematoxylin), the complex between iron and hematein has higher relative stability than others

Relationship between liver iron concentration determined by R2-MRI, serum ferritin, and liver enzymes in patients with thalassemia intermedia

BACKGROUND: Iron overload is a risk factor affecting all patients with thalassemia intermedia (TI). We aimed to determine whether there is a relationship of serum ferritin (SF) and alanine aminotransferase (ALT) with liver iron concentration (LIC) determined by R2 magnetic resonance imaging (R2-MRI), to estimate the most relevant degree of iron overload and best time to chelate in patients with TI. METHODS: In this cross-sectional study, 119 patients with TI (mean age years) were randomly selected and compared with 120 patients who had a diagnosis of thalassemia major (TM). Correlations of LIC, as determined by R2-MRI, with SF and ALT levels, were assessed in all participants. A P-value 5 mg Fe/g dry weight (P < 0.0001). A significant relationship was also found for patients with TI who had elevated ALT level (63.5 U/L), of 3.15 times the upper normal laboratory limit, using a cut-off for LIC ≥5 mg Fe/g dry weight. CONCLUSION: We determined the cut-off values for ALT and SF indicating the best time to start iron chelation therapy in patients with TI, and found significant correlations among iron overload, SF, and ALT.

A Patient with Beta-Propeller Protein-Associated Neurodegeneration: Treatment with Iron Chelation Therapy

We present a case of beta-propeller protein-associated neurodegeneration, a form of neurodegeneration with brain iron accumulation. The patient harbored a novel mutation in the WDR45 gene. A detailed video and description of her clinical condition are provided. Her movement disorder phenomenology was characterized primarily by limb stereotypies and gait dyspraxia. The patient's disability was advanced by the time iron-chelating therapy with deferiprone was initiated, and no clinical response in terms of cognitive function, behavior, speech, or movements were observed after one year of treatment.

Profil clinique et immunologique des patients infectés par le VIH à l'initiation du traitement antirétroviral au centre hospitalier régional de Sokodé (Togo), une décennie après la gratuité de la trithérapie

Introduction : L'initiation tardive du Traitement Anti-Rétroviral (TAR) est fréquente dans les pays à ressources limitées, où la plupart des individus ne connaissent pas leur statut sérologique jusqu'à ce qu'ils soient symptomatiques.Matériel et méthodes : Il s'est agi d'une étude transversale et analytique, qui s'est déroulée du 1er janvier 2016 au 31 décembre 2016 dans le service de médecine générale du Centre Hospitalier Régional (CHR) de Sokodé, le service de référence dans la prise en charge de l'infection à VIH dans la région centrale du Togo.Résultats : Cent-neuf patients dont 15 enfants (13,8%) ont été inclus dans la file active du service de médecine générale du CHR de Sokodé par l'initiation du TAR. La moyenne d'âge des patients était de 32,38 ans [1-59 ans] avec une prédominance féminine (67,9%). La majorité des patients était classée aux stades III (60 cas ; 55%) et au stade IV (11 cas ; 10,1%). Les manifestations cliniques ou affections opportunistes étaient dominées par la diarrhée chronique (15 cas), la fièvre prolongée inexpliquée (12 cas) et la candidose oropharyngée (12 cas). Le taux moyen des CD4 des patients était de 328,54 cellules/mm3 [0-2216 cellules/mm3] et la co-infection VIH-VHB a été retrouvée dans 13,8% (n = 15). L'association Ténofovir (TDF) - Lamivudine (3TC) - Efavirenz (EFV) (87,2% ; n = 95) a été la plus prescrite pour l'initiation du TAR.Conclusion : Le profil des patients infectés par le VIH à l'initiation du TAR à Sokodé est caractérisé par un stade avancé de l'infection à VIH

Sensory Polyneuropathy and Cobalt Intoxication after Metal-on-Metal Total Hip Arthroplasty

We describe a patient with sensory polyneuropathy and cobalt intoxication. The cause of cobalt intoxication was metallosis of a metal-on-metal hip joint composed of cobalt-chrome alloy. A nerve conduction study revealed axonal sensory polyneuropathy, which improved slightly after chelation therapy and revision surgery. This case implies that a history of arthroplasty should not be neglected in the context of sensory polyneuropathy.

Cases of acute mercury poisoning by mercury vapor exposure during the demolition of a fluorescent lamp factory

BACKGROUND: In 2015, workers dismantling a fluorescent lamp factory in Korea were affected by mercury poisoning from exposure to mercury vapor. CASE PRESENTATION: Eighteen out of the 21 workers who participated in the demolition project presented with symptoms of poisoning and, of these, 10 had persistent symptoms even at 18 months after the initial exposure to mercury vapor. Early symptoms of 18 workers included a general skin rash, pruritus, myalgia, sleep disturbance, and cough and sputum production. Following alleviation of these initial symptoms, late symptoms, such as easy fatigue, insomnia, bad dreams, and anxiety disorder, began to manifest in 10 out of 18 patients. Seven workers underwent psychiatric care owing to sleep disturbance, anxiety disorder, and depression, and three workers underwent dermatologic treatment for hyperpigmentation, erythematous skin eruption, and chloracne-like skin lesions. Furthermore, three workers developed a coarse jerky movement, two had swan neck deformity of the fingers, and two received care at an anesthesiology clinic for paresthesia, such as burning sensation, cold sensation, and pain. Two workers underwent urologic treatment for dysfunction of the urologic system and impotence. However, symptomatic treatment did not result in satisfactory relief of these symptoms. CONCLUSION: Awareness of the perils of mercury and prevention of mercury exposure are critical for preventing health hazards caused by mercury vapor. Chelation therapy should be performed promptly following mercury poisoning to minimize damage.

Résultats préliminaires du traitement des fractures trochantériennes par le clou Gamma à Cotonou

L'ostéosynthèse est le traitement classique des fractures trochantériennes. Elle doit être stable et solide pour autoriser un appui précoce même sur terrain ostéoporotique.L'objectif de cette étude était d'évaluer les résultats préliminaires du traitement de ces fractures par le clou Gamma.Matériel et méthodes Cette étude prospective descriptive du 1er Janvier 2012 au 30 Juin 2014 a concerné 23 patients totalisant 24 fractures trochantériennes. Ils étaient opérés après un délai moyen de19 jours. L'âge moyen des patients était de 56 ans (28-102 ans).Il y avait 18 hommes et cinq femmes. Le score moyen de Parker en pré opératoire était de 8,5 (2-9). Toutes les fractures étaient instables selon les critères de Ender. L'évolution après traitement et les résultats anatomiques et fonctionnels ont été évalués avec un recul moyen de 28 mois.Résultats La durée moyenne de l'intervention était de 98 minutes (40-240 minutes).Le Tip apex distance moyen était de 19,58 mm (4,2-36,76 mm). Il était inférieur à 25 mm (n=16). La réduction était anatomique (n=9), acceptable (n=14), et mauvaise (n=1).La position de la vis était idéale (n=13), acceptable (n=8) et mauvaise (n=3). Le délai moyen de mobilisation des patients était de 3 jours. Au dernier recul (22 patients, 22 fractures), les résultats radiologiques étaient bons (n=21) et moyens en varus (n=1). Le score de PMA moyen au dernier recul était de 17(14-18). Seize patients avaient regagné leur autonomie initiale. Le score de Parker moyen au dernier recul était de 8,09 (3-9).Conclusion Cette étude suggère que l'ostéosynthèse des fractures du massif trochantérien par le clou Gamma donne de bons résultats cliniques, radiologiques, et fonctionnels. Cette technique doit être vulgarisée dans notre pays

Treatment and Management of Late Complications in Hereditary Hemolytic Anemia / 임상소아혈액종양

Hereditary hemolytic anemia is a very heterogeneous disorder in which abnormalities of red blood cell structural protein, globin protein, or enzyme defect lead to shortened life span. There has been much progress in revealing its pathophysiology and genetic backgrounds, but the lifelong plans for caring these patients are not well established yet. All patients with hereditary hemolytic anemic have three common problems: transfusion dependency, iron overload and iron chelation therapy. Patients with hereditary spherocytosis (HS) usually manifest severe anemia in neonatal period and infancy, but transfusion requirements may decrease in adulthood. But patients with thalassemia or sickle cell disease usually transfusion-dependent throughout life. Maintaining the optimal hemoglobin (Hb) levels in these patients is crucial because correction of anemia and dilution of abnormal Hb helps prevent certain complications that frequently occur in these patients. Frequent transfusion leads to transfusion-mediated infection and hemochromatosis. Iron chelation therapy should be started early to prevent permanent organ damage. Folate therapy can be helpful in patients with hereditary spherocytosis. Regular evaluations for cholestasis should be started at age 5, and splenectomy with concurrent cholecystectomy can be considered if the patient has cholecystitis. Hydroxyurea can be used to reduce transfusion requirements and prevent complications in patients with β-thalassemia and sickle cell disease. Consensus on long-term management of patients with hereditary hemolytic anemia is lacking, especially for adult patients. But further efforts to build guidelines for long-term follow-up and management of the patients with hereditary hemolytic anemia in the context of Korean society are needed.

Treatment and Management of Late Complications in Hereditary Hemolytic Anemia / 임상소아혈액종양

Hereditary hemolytic anemia is a very heterogeneous disorder in which abnormalities of red blood cell structural protein, globin protein, or enzyme defect lead to shortened life span. There has been much progress in revealing its pathophysiology and genetic backgrounds, but the lifelong plans for caring these patients are not well established yet. All patients with hereditary hemolytic anemic have three common problems: transfusion dependency, iron overload and iron chelation therapy. Patients with hereditary spherocytosis (HS) usually manifest severe anemia in neonatal period and infancy, but transfusion requirements may decrease in adulthood. But patients with thalassemia or sickle cell disease usually transfusion-dependent throughout life. Maintaining the optimal hemoglobin (Hb) levels in these patients is crucial because correction of anemia and dilution of abnormal Hb helps prevent certain complications that frequently occur in these patients. Frequent transfusion leads to transfusion-mediated infection and hemochromatosis. Iron chelation therapy should be started early to prevent permanent organ damage. Folate therapy can be helpful in patients with hereditary spherocytosis. Regular evaluations for cholestasis should be started at age 5, and splenectomy with concurrent cholecystectomy can be considered if the patient has cholecystitis. Hydroxyurea can be used to reduce transfusion requirements and prevent complications in patients with β-thalassemia and sickle cell disease. Consensus on long-term management of patients with hereditary hemolytic anemia is lacking, especially for adult patients. But further efforts to build guidelines for long-term follow-up and management of the patients with hereditary hemolytic anemia in the context of Korean society are needed.

Intoxicación por mercurio elemental por inyección intramuscular: presentación de caso y revisión de literatura / Poisoning with elemental mercury by intramuscular injection. Case report and review of literature

Se han reportado en la literatura pocos casos de intoxicación por mercurio por administración en tejidos blandos. No se cuenta con suficiente evidencia acerca del manejo con terapia quelante en este tipo de intoxicación. Se reporta el caso de una mujer de 34 años con antecedente psiquiátrico la cual se administró mercurio intramuscular en fosa cubital izquierda con fines autolíticos. Acudió al servicio de urgencias 24 horas posteriores a su administración, el motivo principal fue el dolor intenso en la zona y la presencia de edema, sin efectos sistémicos. La radiografía mostró depósitos metálicos en 1/3 de brazo, localizados en músculo, y que migraron a través de la fascia hacia 2/3 del antebrazo. La placa de tórax no mostró alteraciones. Fue intervenida quirúrgicamente en 3 ocasiones extrayendo mínimas cantidades de mercurio. La paciente fue manejada con antibióticos por presencia de celulitis. Un mes después presentó temblor mercurial, razón por la cual se tomaron muestras de sangre y orina para la determinación de mercurio, el cual resulto elevado en ambas muestras, por lo que se le administró terapia quelante con D-penicilamina.

There are just a few cases of mercury toxicity after administration in soft tissue, reported in the literature. There is insufficient evidence about the management with chelation therapy in this type of poisoning. We report the case of a 34 year-old woman with a psychiatric history who administered herself a mercury injection into de muscle in the left cubital fossa, referred as a suicide attempt. She came to the emergency department 24 hours after administration; the main reason was the intense pain in the area and the presence of edema, with no systemic effects. Radiography showed metallic deposits in 1/3 arm, located in muscle, which moved through the fascia to 2/3 of the forearm. Chest radiography was normal. She underwent surgery trhee times extracting trace amounts of mercury. The patient was managed with antibiotics by the presence of cellulite. One month later she had tremor mercuralis, so a blood and urine samples were sent to the laboratory in order to determinate mercury levels, which resulted high in both fluids, therefore chelation therapy with D-penicillamine was administered.

Nefropatía membranosa secundaria a exposición laboral con mercurio metálico

Las nefropatías tóxicas secundarias a la exposición ocupacional a metales han sido ampliamente estudiadas. La nefropatía membranosa por mercurio es poco frecuente.La intoxicación ocupacional con mercurio sí es frecuente, siendo las principales formas de presentación las manifestaciones clínicas neurológicas. La afectación renal secundaria a la exposición crónica a mercurio metálico puede desarrollar enfermedad glomerular por depósito de inmunocomplejos. La glomerulopatía membranosa y a cambios mínimos son las más frecuentemente comunicadas.Se presenta el caso de un paciente con exposición ocupacional a mercurio metálico, con síndrome nefrótico y biopsia renal con glomerulopatía membranosa que presentó respuesta favorable luego del tratamiento quelante e inmunosupresor.

Toxic nephrophaties secondary to occupational exposure to metals have been widely studied, including membranous nephropathy by mercury, which is rare. Occupational poisoning by mercury is frequent, neurological symptoms are the main form of clinical presentation. Secondary renal involvement in chronic exposure to metallic mercury can cause glomerular disease by deposit of immune-complexes. Membranous glomerulopathy and minimal change disease are the most frequently reported forms. Here we describe the case of a patient with occupational exposure to metallic mercury, where nephrotic syndrome due to membranous glomerulonephritis responded favorably to both chelation and immunosuppressive therapy.

Polineuropatía motora subaguda secundaria a intoxicación por plomo / Subacute motor polyneuropathy secondary to lead poisoning

Las manifestaciones clínicas de la intoxicación por plomo son variadas e inespecíficas y continúan siendo un reto diagnóstico, en especial cuando son producto de presentaciones tan infrecuentes como las secundarias a fragmentos por proyectiles de arma de fuego intraarticulares. En suamplio espectro de manifestaciones se han descrito cambios que van desde el déficit de atenciónhasta estados más avanzados como las polineuropatías. El diagnóstico se basa principalmente enla medición del metal en distintos elementos del paciente y el tratamiento en medidas generales desoporte y terapia de quelación. Describimos el caso de un hombre con el antecedente de una herida por arma de fuego en caderaderecha, con un cuadro clínico progresivo de dolor abdominal, anemia acompañada de punteadobasófilo y manifestaciones neurológicas. Con un estudio por electromiografía que reporto unapolineuropatía axonal desmielinizante motora y sensitiva. Sus niveles séricos de plomo fueron 113µg/dL (Rango normal hasta 10 µg/dL) y después de un esquema de tratamiento con edetato calciodisódico (EDTA) más D penicilamina los síntomas neurológicos mejoraron asociado a un controlsérico de plomo de 49.1 µg/dL. Conclusión: la intoxicación por plomo es causa de polineuropatía motora subaguda especialmente en pacientes con exposición prolongada y niveles séricos altos de plomo. (Acta Med Colomb2014; 39: 185-190).

The clinical manifestations of lead poisoning are varied and nonspecific and remain a diagnostic challenge, especially when they are product of infrequent presentations such as the secondary to intraarticular gun fire fragments. In its broad spectrum of manifestations, changes ranging from attention deficit disorder to more advanced states like polyneuropathies have been described. The diagnosis is based mainly on the measurement of metal in various elements of the patient, and treatment in general supportive measures and chelation therapy. The case of a man with a history of gunshot wound in right hip with a progressive clinical picture of abdominal pain, anemia accompanied by basophilic stippling and neurological manifestations is described. The electromyography reported a motor and sensory axonal demyelinating polyneuropathy. Serum levels of lead were 113 µg/dL (normal range up 10 µg/dL) and after a treatment regimen with calcium disodium edetate (EDTA ) plus D penicillamine, the neurological symptoms improved, associated with a serum lead control of 49.1 µg/dL. Conclusion: lead poisoning causes subacute motor polyneuropathy especially in patients with prolonged exposure and high serum lead levels. (Acta Med Colomb 2014; 39: 185-190).

Saturnismo por retenção de projétil de arma de fogo em articulação:relato de caso / Saturnism caused by retained intra-articular bullet: case report

A intoxicação por chumbo, também conhecida como saturnismo, que ocorre em razão do projétil retido em ferimento por arma de fogo, é uma complicação já conhecida e descrita na literatura. O risco dessa intoxicação endógena por chumbo está associado ao contato do projétil com o líquido sinovial ou líquido cefalorraquidiano. O tratamento é feito com terapia de quelação, sendo definitivo com a retirada cirúrgica do projétil. Relatamos aqui um caso clínico de um paciente que desenvolveu intoxicação por chumbo em razão de projétil retido em contato com líquido sinovial. Paciente do gênero masculino, 24 anos, internado para investigação de quadro de dor abdominal associada a náuseas, vômitos e febre há 7 dias. Diagnosticado saturnismo, que foi confirmado por meio de exames laboratoriais e de imagem. Procedeu-se, então, à retirada dos fragmentos, além do segmento clínico de suporte. Apresentou evolução favorável após tratamento

Lead poisoning, also known as saturnism, caused by a retained bullet, is a well-known complication, and has also been described in literature. This endogenous intoxication is associated with the contact of the projectile with synovial liquid or cerebrospinalfluid. Chelation therapy is advised as a course of treatment in addition to the surgical removal of the projectile, both all- together sums up to a definitive optimum outcome. We discuss here a clinical case of a patient who developed lead poisoning due to a retained projectile within synovial liquid. Male patient,24 years old, admitted with the symptoms of abdominal pain, nausea, vomiting and fever for 7 days for investigation. He was diagnosed with saturnism, that was confirmed by laboratorial and imaging exams. The following procedure was the removal of fragments in addition to the continuous clinical follow-up. The patient presented with positive response to treatment.

Iron chelation therapy and its influence on the alleviation of EPO resistance in MDS patients / 中国实验血液学杂志

This study was aimed to investigate the changes of erythropoietin (EPO), hemoglobin(Hb) and recombinant EPO (rEPO) levels in MDS patients receiving iron chelation therapy, and to explore the relationship between EPO and serum ferritin(SF). A total of 172 MDS patients and 30 healthy controls were studied. The levels of SF, EPO, serum iron (SI), total iron binding capacity (TIBC), C-reaction protein (CRP) and Hb were measured respectively, the level of SF was adjusted according to the changes of CRP. Among them, there were 34 cases of low-risk (SF>1 000 mg/L) receiving deferoxamine therapy, whose changes of SF, EPO, SI, TIBC, Hb levels were detected and compared before and after treatment. Besides, the difference in the incidence of EPO resistance in iron overload group and non-iron overload group was assessed before and after therapy, and 58 cases of low-risk and EPO<1 000 U/L MDS patients were given rEPO therapy. The results showed that the level of EPO in non-iron overload group was higher than that in the normal control group (997.44 ± 473.48 vs 467.27 ± 238.49, P < 0.05). Obviously, the level of EPO in iron overload group was higher than that in non-iron overload group and control group (3257.59 ± 697.19 vs 997.44 ± 473.48, P = 0.012, 3257.59 ± 697.19 vs 467.27 ± 238.49, P = 0.002). Otherwise, the incidence of EPO resistance in iron overload group was higher than that in non-iron overload group (18/35 vs 2/23, P = 0.001), and the level of EPO and SF was positively related to each other in iron overload group (r = 0.310,P = 0.036). After receiving iron chelation therapy, the levels of SF, SI, TIBC and EPO in iron overload group were significantly lower than that before therapy (3942.38 ± 641.82 vs 2266.35 ± 367.31, P = 0.028;48.61 ± 10.65 vs 28.52 ± 12.61, P = 0.034;59.84 ± 12.62 vs 33.76 ± 15.43, P = 0.045;3808.01 ± 750.22 vs 1954.78 ± 473.18, P = 0.042). Moreover, the level of Hb increased (35 ± 18 vs 57 ± 21, P = 0.046) and the EPO resistance in some patients was decreased. It is concluded that iron chelation therapy can improve the efficacy of EPO to alleviate EPO resistance in patients wtih anemic MDS, decrease the pathological level of EPO, enhance Hb levels and reduce the dependency on blood transfusion.

Efficacy and safety of combined oral iron chelation therapy with deferasirox and deferiprone in a patient with beta-thalassemia major and persistent iron overload

No abstract available.

Effects of iron chelating therapy and / or HBV-vaccination on beta-thalassemia major patients

Thalassemia is a genetic inherited blood disorder in which the body makes abnormal hemoglobin with excessive destruction of red blood cells, which leads to anemia. For many years, hepatitis B virus was a major problem for patients with thalassemia substantially contracted from blood transfusions. The development of effective vaccine has further reduced the magnitude of the problem of hepatitis B. Iron chelators are used to remove excess iron that accumulates due to repeated blood transfusion. To compare thalassemic patients either have or haven't HCV with healthy persons as regards biochemical indices taking in consideration effect of vaccination against HBV or not and using iron chelating therapy or not by the studied persons. A case control study in which 40 thalassemic, blood transfusion dependent patients were chosen randomly to act as a case group from thalassemic patients attending the VACSERA Company. The cases [40 patients] they were classified to patients having HCV, patients were HBV vaccinated, other non vaccinated, using iron chelating therapyor don't use it. Another 10 healthy and non thalassemic persons were chosen randomly among persons attending the same company as a control group to be matched with the case group. 50% of studied thalassemic patients had HCV seropositivity. Biochemical blood indices which were found to be significantly elevated among thalassemic patients than controls were ALT, AST, ALP, GGT and LDH enzymes in addition to serum iron, ferritin and globulin mostly in HBV non-vaccinated and iron chelating therapy non dependent patients while, other biochemical indices which were significantly decreased among thalassemic patients compared to controls included: total cholesterol, total protein, albumin and albumin/globulin ratio mostly in HBV vaccinated and iron chelating non dependent patients. This study illustrated the effectiveness of iron chelators agentsand the importance of vaccination for reduction of morbidity and mortality