Transient neonatal cyanosis associated with a new Hb F variant: Hb F viseu.

نویسندگان

  • Celeste Bento
  • Tabita Magalhães Maia
  • Ines Carvalhais
  • Filipa Moita
  • Gabriela Abreu
  • Luis Relvas
  • Alexandra Pereira
  • José Farela Neves
  • Maria L Ribeiro
چکیده

Neonatal cyanosis in healthy newborns can be associated either with methemoglobin due to cytochrome b5 reductase deficiency or to M-hemoglobin, a group of hemoglobin variants resulting from mutations in the globin chain genes. We report the clinical case of a neonate with cyanosis and normal cardiac and respiratory function. At birth the hematological parameters were normal; however, the methemoglobinemia was 16%. Spontaneously, the cyanosis gradually decreased and by the fifth month of age the methemoglobin level was normal. A heterozygous Gγ-globin gene (HBG2) missense mutation 87 C-A (Leu28Met) was identified. His father, with a history of transfusion in the neonatal period, is heterozygous for the same mutation. This hemoglobin variant, not previously described, was called Hb F Viseu and is the sixth Gγ-chain variant reported in association with neonatal cyanosis.

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

Hemoglobin Kansas as a Rare Cause of Cyanosis: A Case Report and Review of the Literature

Hemoglobin (Hb) Kansas is an inherited Hb variant with a low oxygen affinity that is associated with low oxygen saturation on pulse oximetry (SpO2). It leads to asymptomatic cyanosis. Patients with Hb Kansas do not require any specific treatment and the prognosis is good. In patients with unexplained cyanosis, we should thus consider Hb variants, including Hb Kansas and avoid unnecessary invest...

متن کامل

Unexpected hemoglobin A1c results.

A 52-year-old woman with a medical history of hepatitis B, hyperlipidemia, hypertension, anemia, and depression presented to the internal medicine clinic for a routine visit. Laboratory tests 3 months previously had revealed an impaired fasting glucose concentration of 5.9 mmol/L (106 mg/dL) [reference interval, 3.9 –5.6 mmol/L (70 –100 mg/dL)]. Therefore, a hemoglobin (Hb) A1c analysis was per...

متن کامل

Hemoglobin Daneshgah-Tehran (HBA1:c.218A>G p.His72Arg): a Rare α1-Globin Variant from Iran

There are more than 400 different variations on α-globin protein, and most of them are not associated with noticeable clinical manifestation. Hemoglobin (Hb) is an oxygen-transporting protein and Hb Daneshgah- Tehran is an α-globin variant that for the first time was reported from Iran in a case with normal haematological indices. The capillary electrophoresis of an 8-year- old-girl with normal...

متن کامل

Incidence of variant hemoglobin (Hb) and increased fetal Hb concentrations and their effect on Hb A1c measurement in a Korean population.

To the Editor: Glycohemoglobin assays are important for evaluating long-term glycemic control in patients with diabetes (1 ). Ion-exchange HPLC is commonly used for measuring hemoglobin A1c (Hb A1c), but hemoglobin variants (Hb) or increased fetal Hb concentrations may affect the quantification of Hb A1c by HPLC. The presence of Hb or high Hb F concentrations can be recognized by the separate e...

متن کامل

A Case Report of Hb-Geelong in Hormozgan Province

Abstract Background and Objectives Thalassemia is a group of inherited hemoglobin disorders with defect in the synthesis of hemoglobin chains.   Case The young couple resident in Bandar Abbas,  a 23 year old woman with MCV:63fl; MCH:19; HbA2:3.9  and  a 25 year old man with MCV:94fl; MCH:32; HbA2:2.1; HbF:36, were referred to the Bandar Abbas Medical Genetic & PND Center for genetic counsell...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

عنوان ژورنال:
  • Journal of pediatric hematology/oncology

دوره 35 2  شماره 

صفحات  -

تاریخ انتشار 2013