Wilsons Disease – A Varied Histological Spectrum

Anesthetic Management of a Pediatric Patient With Wilsons Disease

UNLABELLED Wilsons disease, characterized by cirrhosis, extrapyramidal symptoms and Kayser-Fleischer corneal rings, is a rare hereditary disease of human copper metabolism. Clinical findings in Wilsons disease are complex and neurological symptoms such as tremor, dysarthria, rigid dystonia, seizures, psychiatric disorders, acute liver failure, chronic hepatitis or cirrhosis may develop. A 4-yea...

Copper storage diseases: Menkes, Wilsons, and cancer.

The trace element copper is vital to the healthy functioning of organisms. Copper is used in a multitude of cellular activities including respiration, angiogenesis, and immune responses. Like other metals, copper homeostasis is a tightly regulated process. Copper is transported from dietary intake through the serum and into cells via a variety of transporters. There are a variety of copper chap...

Cervical Necrotizing Fasciitis: A Potentially Fatal Disease with Varied Etiology

Necrotizing fasciitis was recognized centuries ago by physicians. It is a rapidly progressive and potentially fatal soft-tissue infection that is typified by soft-tissue necrosis, especially affecting the subcutaneous tissues and fascia. Cervico-facial necrotizing fasciitis is said to be uncommon, but when it occurs, it is often of odontogenic origin and has severe consequences if not promptly ...

Gastrointestinal stromal tumour: varied presentation of a rare disease.

Hypertrophic pachymeningitis: varied manifestations of a single disease entity.

Hypertrophic pachymeningitis is a unique clinical entity characterised by fibrosis and thickening of the duramater with resulting neurological dysfunction. Three cases of this entity are described. Presenting features were headaches and cranial neuropathies in two patients and predominantly cerebellar dysfunction in the third. One of the patients also had evidence of spinal involvement. Lower c...