Biomaterial Database

A case of a hereditary, late progressing sensory autonomic neuropathy. 1998

Y Cinar, and F Hekimoglu, and B Ince, and U Ince, and E Onganer

Internal Medicine Department, Haydarpasa Numune Hospital, Istanbul, Turkey.

A case of a hereditary sensory autonomic neuropathy (HSAN) with severe medical complications that had been reported as nonprogressive HSAN type II has been followed and treated for a period of 10 years, and is now considered to be progressive HSAN type III, is presented. Current difficulties in the present form of the classification of HSAN, the necessity to observe these cases for longer periods and possible prolongation of survival with appropriate supportive treatment are emphasized.

UI	MeSH Term	Description	Entries
D009477	Hereditary Sensory and Autonomic Neuropathies	A group of inherited disorders characterized by degeneration of dorsal root and autonomic ganglion cells, and clinically by loss of sensation and autonomic dysfunction. There are five subtypes. Type I features autosomal dominant inheritance and distal sensory involvement. Type II is characterized by autosomal inheritance and distal and proximal sensory loss. Type III is DYSAUTONOMIA, FAMILIAL. Type IV features insensitivity to pain, heat intolerance, and mental deficiency. Type V is characterized by a selective loss of pain with intact light touch and vibratory sensation. (From Joynt, Clinical Neurology, 1995, Ch51, pp142-4)	HSAN,HSAN Type I,HSAN Type II,HSAN Type IV,HSAN Type V,HSN Type I,HSN Type II,Insensitivity to Pain with Anhidrosis, Congenital,Neuropathies, Hereditary Sensory and Autonomic,Pain Insensitivity with Anhidrosis, Congenital,Sensory Neuropathy, Hereditary,Sensory and Autonomic Neuropathies, Hereditary,Acroosteolysis, Giaccai Type,Acroosteolysis, Neurogenic,Congenital Insensitivity to Pain with Anhidrosis,Familial Dysautonomia, Type 2,Familial Dysautonomia, Type II,Giaccai Type Acroosteolysis,HSAN (Hereditary Sensory Autonomic Neuropathy),HSAN 1,HSAN 4,HSAN 5,HSAN I,HSAN IV,HSAN V,HSAN2,HSAN5,HSANII,Hereditary Sensory And Autonomic Neuropathy IV,Hereditary Sensory Autonomic Neuropathy, Type 1,Hereditary Sensory Autonomic Neuropathy, Type 2,Hereditary Sensory Autonomic Neuropathy, Type 4,Hereditary Sensory Autonomic Neuropathy, Type 5,Hereditary Sensory Neuropathy Type 1,Hereditary Sensory Neuropathy Type I,Hereditary Sensory Neuropathy Type Ia,Hereditary Sensory Radicular Neuropathy,Hereditary Sensory Radicular Neuropathy, Recessive Form,Hereditary Sensory and Autonomic Neuropathy 4,Hereditary Sensory and Autonomic Neuropathy Type 1,Hereditary Sensory and Autonomic Neuropathy Type 2,Hereditary Sensory and Autonomic Neuropathy Type I,Hereditary Sensory and Autonomic Neuropathy Type II,Hereditary Sensory and Autonomic Neuropathy Type IV,Hereditary Sensory and Autonomic Neuropathy Type V,Hereditary Sensory and Autonomic Neuropathy, Type 4,Hereditary Sensory and Autonomic Neuropathy, Type 5,Insensitivity to Pain, Congenital, with Anhidrosis,Neurogenic Acroosteolysis,Neuropathy Hereditary Sensory Radicular, Autosomal Dominant,Neuropathy Hereditary Sensory and Autonomic Type 1,Neuropathy, Congenital Sensory,Neuropathy, Congenital Sensory, with Anhidrosis,Neuropathy, Hereditary Sensory And Autonomic, Type I,Neuropathy, Hereditary Sensory And Autonomic, Type V,Neuropathy, Hereditary Sensory Radicular, Autosomal Dominant,Neuropathy, Hereditary Sensory Radicular, Autosomal Recessive,Neuropathy, Hereditary Sensory, Type I,Neuropathy, Progressive Sensory, Of Children,Acroosteolyses, Neurogenic,Congenital Sensory Neuropathies,Congenital Sensory Neuropathy,HSANs (Hereditary Sensory Autonomic Neuropathy),HSN Type IIs,Hereditary Sensory Neuropathies,Hereditary Sensory Neuropathy,Neurogenic Acroosteolyses,Neuropathies, Congenital Sensory,Neuropathies, Hereditary Sensory,Neuropathy, Hereditary Sensory,Sensory Neuropathies, Congenital,Sensory Neuropathies, Hereditary,Sensory Neuropathy, Congenital,Type I, HSAN,Type I, HSN,Type IV, HSAN
D003131	Combined Modality Therapy	The treatment of a disease or condition by several different means simultaneously or sequentially. Chemoimmunotherapy, RADIOIMMUNOTHERAPY, chemoradiotherapy, cryochemotherapy, and SALVAGE THERAPY are seen most frequently, but their combinations with each other and surgery are also used.	Multimodal Treatment,Therapy, Combined Modality,Combined Modality Therapies,Modality Therapies, Combined,Modality Therapy, Combined,Multimodal Treatments,Therapies, Combined Modality,Treatment, Multimodal,Treatments, Multimodal
D005260	Female		Females
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328	Adult	A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available.	Adults
D018450	Disease Progression	The worsening and general progression of a disease over time. This concept is most often used for chronic and incurable diseases where the stage of the disease is an important determinant of therapy and prognosis.	Clinical Course,Clinical Progression,Disease Exacerbation,Exacerbation, Disease,Progression, Clinical,Progression, Disease