A rare disorder characterized by recurrent infantile infections and absence of neutrophils in the peripheral blood.
Symptoms of Congenital agranulocytosis
The following features are indicative of Congenital agranulocytosis:
sudden fever
rigors
sore throat
severe neutropenia
recurrent infections
It is possible that Congenital agranulocytosis shows no physical symptoms and still is present in a patient.
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Common Causes of Congenital agranulocytosis
The following are the most common causes of Congenital agranulocytosis:
autosomal recessive inherited disorder
Prevention of Congenital agranulocytosis
No, it is not possible to prevent Congenital agranulocytosis.
autosomal recessive inherited disorder
Occurrence of Congenital agranulocytosis
Number of Cases
The following are the number of Congenital agranulocytosis cases seen each year worldwide:
Widely occurring between 500K - 1 Million cases
Common Age Group
Congenital agranulocytosis most commonly occurs in the following age group:
At birth
Common Gender
Congenital agranulocytosis can occur in any gender.
Lab Tests and Procedures for Diagnosis of Congenital agranulocytosis
The following lab tests and procedures are used to detect Congenital agranulocytosis:
Blood test: To check for neutrophils level
Genetic testing: To check for the gene mutations occuring in the condition
Complications of Congenital agranulocytosis if untreated
Yes, Congenital agranulocytosis causes complications if it is not treated. Below is the list of complications and problems that may arise if Congenital agranulocytosis is left untreated:
frequent infections of the sinuses, lungs, and liver
osteopenia
osteoporosis
Procedures for Treatment of Congenital agranulocytosis
The following procedures are used to treat Congenital agranulocytosis:
Bone marrow transplantation: To treat Kostmann's Syndrome
G-CSF treatment: Helps treating Kostmann's syndrome on a short term basis
Last updated date
This page was last updated on 2/04/2019.
This page provides information for Congenital agranulocytosis.