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1.
Pediatr Blood Cancer ; 71(10): e31220, 2024 Oct.
Article in English | MEDLINE | ID: mdl-39096194

ABSTRACT

BACKGROUND: Children treated for cancer are at risk for adverse effects of iron due to transfusions administered during prolonged marrow suppression, which may increase exposure to toxic forms of iron, extrahepatic iron accumulation, and long-term organ damage. OBJECTIVE: This study aimed to characterize the severity and organ distribution of clinically significant, multisystem iron overload (IO) in an at-risk cohort of pediatric cancer patients. METHODS: This was a retrospective, cross-sectional study of childhood cancer patients who underwent a magnetic resonance imaging (MRI) due to clinical concern for IO. Data regarding cancer type and treatment, transfusion history, MRI and laboratory results, and treatment for IO were collected. Severity of IO was analyzed by non-parametric tests with respect to clinical characteristics. RESULTS: Of the 103 patients, 98% of whom had a Cancer Intensity Treatment Rating (ITR-3) of 3 or higher, 53% (54/102) had moderate or greater hepatic siderosis, 80% (77/96) had pancreatic siderosis, 4% (3/80) had cardiac siderosis, and 45% (13/29) had pituitary siderosis and/or volume loss. Pancreatic iron was associated with both cardiac (p = .0043) and pituitary iron (p = .0101). In the 73 off-therapy patients, ferritin levels were lower (p = .0008) with higher correlation with liver iron concentration (LIC) (p = .0016) than on-therapy patients. Fifty-eight subjects were treated for IO. CONCLUSION: In this heavily treated cohort of pediatric cancer patients, more than 80% had extrahepatic iron loading, which occurs with significant exposure to toxic forms of iron related to decreased marrow activity in setting of transfusions. Further studies should examine the effects of exposure to reactive iron on long-term outcomes and potential strategies for management.


Subject(s)
Hemosiderosis , Neoplasms , Humans , Male , Child , Female , Hemosiderosis/etiology , Retrospective Studies , Neoplasms/therapy , Neoplasms/complications , Cross-Sectional Studies , Child, Preschool , Adolescent , Transfusion Reaction , Magnetic Resonance Imaging , Cancer Survivors , Infant , Iron Overload/etiology , Adult , Blood Transfusion , Follow-Up Studies
3.
EBioMedicine ; 99: 104929, 2024 Jan.
Article in English | MEDLINE | ID: mdl-38128412

ABSTRACT

BACKGROUND: Iron overload due to the excessive use of parenteral iron in haemodialysis is now an increasingly recognised clinical issue. Before erythropoiesis-stimulating agents (ESA) were introduced, a specific feature of patients treated by dialysis and having iron overload was that iron levels in the bone marrow were paradoxically low in most of them, despite severe hepatosplenic siderosis. Whether or not this paradox persists in the actual ESA era was unknown until recently, when an autopsy study in 21 patients treated by haemodialysis revealed similarities between liver and bone marrow iron content. The aim of this study was to further explore these recent findings in a cohort of alive patients on dialysis and to analyse the determinants of iron bone marrow. METHODS: Liver iron concentration (LIC) and vertebral T2∗ (a surrogate marker of bone marrow iron) were analysed retrospectively in 152 alive patients on dialysis (38.8% female) of whom 47.4% had iron overload by quantitative magnetic resonance imaging (MRI). FINDINGS: Vertebral T2∗ differed significantly between patients classified according to liver iron content at MRI: those with mild or moderate and severe liver iron overload had increased vertebral iron content at R2∗ relaxometry MRI (mild: vertebral T2∗ = 9.9 ms (4-24.8); moderate and severe: vertebral T2∗ = 8.5 ms (4.9-22.8)) when compared to patients with normal LIC (vertebral T2∗ = 13.2 ms (6.6-30.5) (p < 0.0001 Kruskal-Wallis test)). INTERPRETATION: The paradoxical discrepancy between bone marrow and liver iron-storage compartments observed in the pre-ESA era has disappeared today, as shown by a recent autopsy study and the present study in a cohort of alive patients treated by dialysis. FUNDING: None.


Subject(s)
Hemosiderosis , Iron Overload , Humans , Female , Male , Retrospective Studies , Bone Marrow/chemistry , Renal Dialysis/adverse effects , Hemosiderosis/etiology , Hemosiderosis/pathology , Iron , Iron Overload/pathology , Liver/pathology , Magnetic Resonance Imaging/methods
4.
Rev. esp. pediatr. (Ed. impr.) ; 63(6): 504-507, nov.-dic. 2007.
Article in Spanish | IBECS | ID: ibc-60213

ABSTRACT

La aplasia congénita de la serie roja es una rara causa de anemia hipoplásica congénita, generalmente secundaria a una mutación esporádica. Por su amplio espectro de presentación puede ser de difícil diagnóstico, aunque está descrito un fenotipo característico. La corticolterapia produce la remisión completa en un gran porcentaje de estos pacientes. La ausencia de repuesta a ésta plantea la realización de un trasplante alogénico de médula de dónate HLA idéntico, preferentemente familiar. Si no se dispone de donante adecuado es preciso aplicar una terapia sustitutiva con transfusiones. La hemosiderosis secundaria puede causar una gran morbimortalidad. Nuevas terapias inmunosupresoras con resultados diversos y el uso de la quelación oral podrían cambiar el pronóstico de forma importante. Presentamos, una paciente de diagnóstico precoz, en la que se evidenció una mutación esporádica, resistente a los tratamientos convencionales y no convencionales que, gracias a la quelación oral, ha mejorado radicalmente sus cifras de ferritina, evitando las complicaciones de la hemosiderosis. Actualmente está en espera para acceder a un programa de fertilización para selección embrionaria de posible donante, ya que no posee donante familiar histocompatible (AU)


Congenital aplasia of the red cell series is a rare cause of congenital hypoplastic anemia, generally secondary to sporadic mutation. Due to its wide presentation spectrum, it may be difficult to diagnose, although a characteristic phenotype is described. Corticosteroid therapy may produce complete remission in a large percentage of these patients. Lack of response to it suggests the performance of an allergenic bone marrow transplant form an identical HLA donor, preferably a relative. If no adequate donor is available, substitution treatment must be given with transfusions. Secondary hemosiderosis may cause great morbidity-mortality. New immunosuppressant therapies with different results and the use of oral chelation may change the prognosis significantly. We present the case of a patient with early diagnosis in whom sporadic mutation was observed that was resistant to conventional and non-conventional treatments. Thanks to oral chelation, her ferritin values have radically improved, thus avoiding the complications of hemosiderosis. At present, she is waiting to access a fertilization program for embrionary selection of a possible donor, since she has no histocompatible family donor (AU)


Subject(s)
Humans , Child , Anemia, Diamond-Blackfan/complications , Anemia, Hypoplastic, Congenital/etiology , Anemia, Diamond-Blackfan/diagnosis , Anemia, Diamond-Blackfan/drug therapy , Immunosuppressive Agents/therapeutic use , Adrenal Cortex Hormones/therapeutic use , Anemia, Hypoplastic, Congenital/diagnosis , Hemosiderosis/etiology
5.
Rev. méd. Chile ; 133(11): 1311-1316, nov. 2005. ilus, tab
Article in Spanish | LILACS | ID: lil-419934

ABSTRACT

Background: Twenty percent of patients with chronic hepatitis C evolve to cirrhosis in 10 to 20 years. The degree of steatosis and hepatic iron stores in liver biopsy increase the risk. Age, high body mass index, diabetes mellitus and alcohol consumption are factors associated to the severity of liver damage. Aim: To study the association of steatosis and increased iron stores in the liver biopsy and age, overweight, alcohol consumption and diabetes with the severity of liver damage in patients with hepatitis C virus infection. Patients and methods: Retrospective study of 84 liver biopsies of patients with chronic infection with hepatitis C virus were studied. The pathological appearance was classified as stage I when chronic hepatitis with mild activity without fibrosis was observed; as stage II when moderate chronic hepatitis with mild fibrosis was observed and as stage III when there was a moderate chronic hepatitis with fibrosis or cirrhosis. The amount of steatosis and iron deposition in the biopsy were also assessed. Results: Forty one percent of patients were in stage I, 32% in stage II and 27% in stage III. Patients in stage I were younger than those in stages II and III (40.7 and 52.2 years respectively, p <0,001). No association between the severity of liver damage and the degree of steatosis, hemosiderosis, body mass index or alcohol intake, was observed. The frecuency of diabetes mellitus increased along with pathological staging (3, 15 and 30% in stages I, II and III, respectively, p <0,05). Conclusions: This study confirms that severity of chronic hepatitis C is associated with age and the presence of diabetes mellitus.


Subject(s)
Adult , Aged , Female , Humans , Male , Middle Aged , Fatty Liver/pathology , Hepatitis C, Chronic/pathology , Alcohol Drinking/adverse effects , Biopsy , Body Mass Index , Diabetes Complications , Hemosiderosis/etiology , Hemosiderosis/pathology , Hepatitis C, Chronic/classification , Liver Cirrhosis/pathology , Overweight , Retrospective Studies , Severity of Illness Index
6.
Rev. chil. pediatr ; 71(3): 192-6, mayo-jun. 2000.
Article in Spanish | LILACS | ID: lil-270922

ABSTRACT

La anemia de Diamond-Blackfan es una causa poco frecuente de anemia en el recién nacido y el lactante menor. Analizamos retrospectivamente las características clínicas y de laboratorio en 20 pacientes controlados en un período de 30 años. Encontramos que en el 85 por ciento de los casos el diagnóstico se planteó antes del año de edad y la mayoría eran de sexo femenino. En 45 por ciento existía el antecedente de bajo peso de nacimiento. El principal hallazgo al examen físico, además de la palidez, fue la talla baja y no se encontraron otras malformaciones asociadas en la mayoría de los pacientes. El 100 por ciento presentaba valores de hemoglobina por debajo de los valores normales para su edad, con macrocitosis y reticulocitopenia, sin compromiso del resto de las series hematológicas. La respuesta adecuada a corticoides se observó en 85 por ciento. De los 3 pacientes refractarios, 2 fallecieron y 1 sobrevive con dependencia a transfusiones de glóbulos rojos y hemosiderosis secundaria, ya que no fue posible efectuar trasplante de médula ósea por falta de donante. El trasplante debe ser considerado precozmente como alternativa terapéutica en el grupo no respondedor a corticoides, si existe donante compatible, ya que otros tratamientos son poco exitosos


Subject(s)
Humans , Male , Female , Infant, Newborn , Infant , Fanconi Anemia/diagnosis , Infant, Low Birth Weight , Blood Transfusion , Fanconi Anemia/complications , Fanconi Anemia/therapy , Hemoglobins/deficiency , Hemosiderosis/etiology , Infant, Low Birth Weight , Prednisone/therapeutic use , Retrospective Studies , Bone Marrow Transplantation
8.
J. pediatr. (Rio J.) ; 66(10/12): 278-81, out.-dez. 1990. tab, ilus
Article in Portuguese | LILACS | ID: lil-119058

ABSTRACT

Os autores mostram um caso de Hemossiderose Pulmonar Idiopatica na forma de inicio infantil, com uma sobrevida bem maior que a relatada na literatura. A crianca tem 5 anos de evolucao da doenca, e poucos sinais de cronicidade, apesar dos episodios graves de sangramento pulmonar, o que se opoe a descricao classica do quadro. Foi sugerida a alergia a proteina do leite de vaca como etiologia da doenca, e o tratamento foi feito com corticoide durante os surtos, com rapida resposta. Observou-se tambem varios episodios de recrudescen-cia por fuga da dieta, que nao ocorreram apos orientacao de uma equipe multidis-ciplinar. Os autores discutem ainda outros aspectos da doenca e fazem uma revi-sao da literatura.


Subject(s)
Child , Humans , Female , Hemosiderosis/etiology , Lung , Adrenal Cortex Hormones/drug therapy , Breast-Milk Substitutes/adverse effects , Hemosiderosis/therapy
9.
Arq. bras. neurocir ; 7(4): 237-41, dez. 1988. ilus
Article in Portuguese | LILACS | ID: lil-73513

ABSTRACT

Os autores relatam uma modificaçäo técnica nas hemisferectomias totais, com intuito de prevenir os sangramentos tardios e distorçöes cerebrais


Subject(s)
Humans , Cerebral Decortication/methods , Cerebral Decortication/adverse effects , Cerebral Hemorrhage/prevention & control , Craniotomy/methods , Hemosiderosis/etiology , Hemosiderosis/prevention & control
10.
Rev. Hosp. Clin. Fac. Med. Univ. Säo Paulo ; 42(6): 282-9, nov.-dez. 1987.
Article in Portuguese | LILACS | ID: lil-52787

ABSTRACT

É feita breve revisäo sobre o metabolismo do ferro no qual os Autores chamam a atençäo para o equilibrio que deve existir entre as quantidades ingeridas com a alimentaçäo e as condiçöes de absorçäo desse metal. Como no homem näo há via de excreçäo do ferro as trocas que se fazem entre o organismo e o meio externo devem ser bem conhecidas para evitar processo de depósito, condiçäo muito mais grave que sua carência. Revêm o mecanismo de absorçäo do ferro ao nível das células do intestino e sua distribuiçäo no organismo. O ferro é encontrado ligado a proteínas: hemoglobina, hemo-enzimas, hemo-pigmentos, mioglobina, ferritina e hemossiderina, estas duas últimas sob a forma de material armazenado. Abordam os tipos de carência: 1 - por falta de absorçäo; 2 - por perda hemorrágica e 3 - misto. Analisam os mecanismos envolvidos nestas situaçöes assim como seu tratamento. O excesso de depósito de ferro (hemossiderose e hemocromatose) é revisto, bem como as possibilidades terapêuticas. Chamam a atençäo para as campanhas comunitárias que se propöem a combater em geral, as carências de ferro das populaçöes de nível sócio-econômico mais baixo, atentando para os perigos que daí podem advir, ou seja, o aumento de incidência de hemossiderose e da hemocromatose


Subject(s)
Humans , Hemochromatosis/etiology , Hemosiderosis/etiology , Intestinal Absorption , Iron/metabolism , Chelating Agents/therapeutic use , Diet , Food, Fortified/adverse effects , Hemochromatosis/therapy , Hemosiderosis/therapy , Iron/deficiency , Phlebotomy
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