Noble Bass Sherman syndrome


Noble-Bass-Sherman syndrome: A very rare syndrome characterized by various eye anomalies


The list of signs and symptoms mentioned in various sources for Noble-Bass-Sherman syndrome includes the 8 symptoms listed below: * Ectopic lens * Cataract * Retinal detachment * Tapetoretinal dystrophy * Myopia * Impaired vision * Abnormal pupils * Night blindness


* Cataract * Impaired vision * Myopia * Night blindness * Retinal detachment


Untreated patients of Marfan's die at age 30: Marfan's syndrome is an autosomal disorder of the connective tissues. It affects the connective tissue of the skeletal, ocular and cardiovascular system. The patient is usually tall and thin with long digits, high arched palate and loose joints. He may also present with ocular and cardiac deformities. The diagnosis is based on the family history and co-relating all the findings. Of concern is aortic root dilatation which occurs in these patients and hence an annual echocardiogram is required. It must be discriminated from acromegaly, gigantism, fragile X syndrome and thyrotoxicosis by means of blood investigations. If untreated the patient dies at the age of 30 or 40 years