Molecular defects in Hb H hydrops fetalis

Hydrops fetalis due to an unusual form of Hb H disease.

The occurrence of Hb H hydrops fetalis is reported for the first time. The mother has zeta-alpha thalassemia 1 (zeta zeta alpha alpha/----) and the father has non-deletion alpha thalassemia [zeta zeta alpha alpha/zeta zeta (alpha alpha)T]. The complete deletion of the zeta alpha cluster on one chromosome was confirmed by quantitation of alpha and zeta gene numbers, the normal alpha and zeta gen...

Prenatal Diagnosis of Hb H Hydrops Fetalis Caused By Haemoglobin Adana

α-Thalassaemia is the most common inherited disorder of Haemoglobin (Hb) production in southeast Asia, resulting from deficient synthesis of the α-globin chain component of the haemoglobin molecule due to deletion or inactivation of one or more of the normal four alpha-chain genes. The severity of the condition depends on the number of genes inactivated. The severest form is Hb Barts hydrops fe...

[Non-immunologic hydrops fetalis].

Non-immune hydrops fetalis

Aetiology of hydrops fetalis.

These 2 cases provide additional examples of HUS occurring in sibs after a long interval. The family resided in New York City, which is not considered as an endemic area for HUS, and the clinical course of the disease in both infants was almost identical, culminating in early death. The aetiology of HUS may indeed be multiple, and the genetic basis for a significant proportion of cases does not...