Bloom Syndrome

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Bloom Syndrome


Bloom Syndrome

Bloom Syndrome is a disorder that is characterized by a significantly increased risk of cancer and various other features. Signs and symptoms of bloom Syndrome involves short stature sun-sensitive skin changes on the face, hands or arms a highpitched voice and distinctive facial features including a long, narrow face, small lower jaw, large nose and prominent ears. Some affected individuals can also have learning disabilities an increased risk of diabetes chronic obstructive pulmonary disease (COPD) and recurrent infections of the upper respiratory tract, ears, and lungs during infancy.


What are the causes of Bloom Syndrome? The exact cause of development of Bloom Syndrome is presently unknown. It is a genetic disorder involving the BLM gene that is transmitted in an autosomal recessive manner Autosomal recessive: The conditions of autosomal recessive are traits or disorders that occur when two copies of an abnormal gene have been inherited on a non-sex chromosome. If both parents have an autosomal recessive condition, there is a 100% likelihood of passing on the mutated genes to their children.


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What are the causes of Bloom Syndrome? 

The individual will be a carrier of the condition if only one mutant copy of the gene is inherited, but will not be present with any symptoms. Children born to two carriers, have a 25% chance of being homozygous dominant (unaffected), a 50% chance of being heterozygous (carrier), and a 25% chance of being homozygous recessive (affected).


What are the signs and symptoms of Bloom Syndrome?

The signs and symptoms of Bloom Syndrome may include: 

Agenesis of maxillary lateral incisor

Bronchiectasis

Chronic lung disease

Clinodactyly of the 5th finger

Cryptorchidism

Hypertrichosis

Leukemia

Prominent nose

Specific learning disability

Spotty hypopigmentation

Type II diabetes mellitus


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What are the signs and symptoms of Bloom Syndrome?

Based on the frequency of symptoms observed, the following information may be noted: Very frequently present symptoms in 80-99% of the cases: 

Abnormality of chromosome stability

Delayed skeletal maturation

Diarrhea

Dolichocephaly

Erythema

Facial telangiectasia in butterfly midface distribution

Intrauterine growth retardation

Malar flattening


What are the signs and symptoms of Bloom Syndrome?

Narrow face

Postnatal growth retardation

Recurrent respiratory infections

Sinusitis

Spotty hyperpigmentation Frequently present symptoms in 30-79% of the cases:

Cutaneous photosensitivity

High pitched voice

Hypoplasia of the zygomatic bone

Short nose

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What are the signs and symptoms of Bloom Syndrome?

Occasionally present symptoms in 5-29% of the cases: 

Acute leukemia

Azoospermia

Decreased fertility in females

Finger syndactyly

Hand polydactyly

Hyperhidrosis

Hypopigmented skin patches

Ichthyosis

IgA deficiency

IgG deficiency

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What are the signs and symptoms of Bloom Syndrome?

IgM deficiency

Intellectual disability, mild

Lymphoma

Microcephaly

Neoplasm of the gastrointestinal tract

Protruding ear

Reduced number of teeth

Sacral dimple

Squamous cell carcinoma


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