Principales causas de muerte en adultos con hemoglobinopatías / Main causes of death in adults with hemoglobin disease

Se efectuó un estudio descriptivo y retrospectivo de los 22 fallecidos con diagnóstico de hemoglobinopatías en el Servicio de Hematología del Hospital General Docente "Dr. Juan Bruno Zayas Alfonso" de Santiago de Cuba, desde enero de 2008 hasta diciembre de 2012, con vistas a identificar las manifestaciones clínicas que ocasionaron complicaciones fatales. En la serie predominaron la intensificación del íctero como causa principal de ingreso y el tromboembolismo pulmonar, las infecciones y la insuficiencia hepática como causas de muerte demostradas por necropsia, aunque esta solo se realizó a 22,7 % de los fallecidos; también se observó que la curva de supervivencia comenzó a declinar a partir de los 20 años en el sexo masculino y de los 30 en el femenino. Las diferentes causas de muerte estuvieron relacionadas con el daño orgánico progresivo, de manera que fueron identificados los órganos más afectados, para poder establecer así pautas preventivas en el seguimiento de los pacientes con esta enfermedad, a fin de minimizar las crisis vasooclusivas que contribuyen a complicaciones y muerte temprana.

A descriptive and retrospective study of the 22 dead with diagnosis of hemoglobin disease in the Hematology Service of "Dr. Juan Bruno Zayas Alfonso" Teaching General Hospital in Santiago de Cuba, was carried out from January, 2008 to December, 2012, aimed at identifying the clinical manifestations that caused fatal complications. In the series the icterus intensification prevailed as main cause of admission and the pulmonary thromboembolism, the infections and the hepatic failure as causes of death demonstrated by autopsy, although this was only carried out to the 22.7% of the dead; it was also observed that the survival curve began to decline starting from the 20 years in the male sex and from the 30 years in the female sex. The different causes of death were related to the progressive organic damage, so that the most affected organs were identified, to be able to establish preventive rules in the follow up of the patients with this disease, in order to minimize the vasooclusive crises that contribute to complications and early death.

Multiple Organic Damages in 202 Cases with Kawasaki Disease / 实用儿科临床杂志

Objective To analyze the clinical features of organic damages in Kawasaki disease(KD),and to improve the diagnosis and treatment level.Methods Two hundred and two cases with KD admitted in Children's Hospital Affiliated to Capital Institute of Pediatrics from Jan.2000 to Oct.2007 were reviewed,and their blood routine,urine,stool routine,liver function,kidney function,chest X-ray,and so on,were investigated.The incidence of organic damages in KD was evaluated.Results Among the 202 KD children,137 were male and 65 were female,the male-female ratio was 2.11.0.Most of them were infants,and 71.3% of them were younger than 3 years old.The damages of hematological system were detected in 78/202 cases(38.6%),respiratory system 76/202 cases(37.6%),cardiovascalar system 68/202 cases(33.7%),urinary system 52/202 cases(25.6%),digestive system 23/202 cases(11.4%),and central nervous system 3/202 cases(1.5%).On the other hand,3 cases complicated with septemia,1 case with juvenile rheumatoid arthritis and 1 case with Henoch-Schonlein purpura.Conclusions The data indicate that the incidence of hematological system involvement in the sick-children with KD were highest,with more than 2 organs involved simaltaneously.Many organic damages were involved in KD and diverse clinical manifestation could be found.Physicians should understand not only the typical features but also the rare symptoms and organic damage so as to prevent misdiagnosis and improper treatment.J Appl Clin Pediatr,2009,24(1):31-32