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Congenital Pain Insensitivity

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A syndrome characterized by indifference to PAIN despite the ability to distinguish noxious from nonnoxious stimuli. Absent corneal reflexes and MENTAL RETARDATION may be associated. Familial forms with autosomal recessive and autosomal dominant patterns of inheritance have been described. (Adams et al., Principles of Neurology, 6th ed, p1343)

Also Known As:

Pain Insensitivity, Congenital; Congenital Analgesia; Congenital Indifference to Pain; Insensitivity, Congenital Pain; Congenital Pain Indifference; Congenital Pain Indifferences; Analgesia, Congenital; Pain Indifference, Congenital

Networked: 15 relevant articles (0 outcomes, 0 trials/studies) for this Disease, Comments

Disease Context: Research Results

Related Diseases

1.	Pain (Aches)
2.	Mental Retardation (Idiocy)
3.	Neurogenic Inflammation
4.	Self Mutilation
5.	Peripheral Nervous System Diseases (PNS Diseases)

Experts

1.	Tsou, Mei-Yung: 1 article (06/2004)
2.	Tsai, Shen-Kou: 1 article (06/2004)
3.	Ting, Chien-Kun: 1 article (06/2004)
4.	Lin, Su-Man: 1 article (06/2004)
5.	Kao, Sheng-Chin: 1 article (06/2004)
6.	Cheng, Kai-Wen: 1 article (06/2004)
7.	Chan, Kwok-Hon: 1 article (06/2004)
8.	Sánchez Mercado, M: 1 article (12/2002)
9.	Rodríguez Pérez, M V: 1 article (12/2002)
10.	Fernández Daza, P L: 1 article (12/2002)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Congenital Pain Insensitivity:

1.	Naloxone (Narcan)FDA LinkGeneric 01/01/1986 - "Moreover, patients with congenital analgesia show pronounced elevation of nociceptive reflex thresholds which can be reduced to within almost normal levels by dosing with naloxone. " 12/01/1984 - "[Changes in evoked potentials in pain and the tolerance threshold to intensive stimulation after naloxone administration in patients with congenital analgesia]" Order ALL the reference details at left...
2.	Vanilmandelic Acid (Vanillylmandelic Acid)IBA 01/01/1984 - "The congenital pain insensitivity syndrome is accompanied by: a) the presence of a yellow-brown pigment in the basal layer of the epidermis with the histochemical properties of melanin, b) decrease of urinary and blood concentration of dopamine and norepinephrine, c) excretion of melanin and of an abnormal as yet unidentified phenolic metabolite, d) increase in urinary p-hydroxyphenyllactic acid, p-hydroxyphenylacetic acid, indole acetic acid, and e) a high homovanillic acid/vanillylmandelic acid (HVA/VMA) ratio. " Order ALL the reference details at left...
3.	Norepinephrine (Noradrenaline)FDA LinkGeneric 01/01/1984 - "The congenital pain insensitivity syndrome is accompanied by: a) the presence of a yellow-brown pigment in the basal layer of the epidermis with the histochemical properties of melanin, b) decrease of urinary and blood concentration of dopamine and norepinephrine, c) excretion of melanin and of an abnormal as yet unidentified phenolic metabolite, d) increase in urinary p-hydroxyphenyllactic acid, p-hydroxyphenylacetic acid, indole acetic acid, and e) a high homovanillic acid/vanillylmandelic acid (HVA/VMA) ratio. " Order ALL the reference details at left...
4.	Melanins (Melanin)IBA 01/01/1984 - "The congenital pain insensitivity syndrome is accompanied by: a) the presence of a yellow-brown pigment in the basal layer of the epidermis with the histochemical properties of melanin, b) decrease of urinary and blood concentration of dopamine and norepinephrine, c) excretion of melanin and of an abnormal as yet unidentified phenolic metabolite, d) increase in urinary p-hydroxyphenyllactic acid, p-hydroxyphenylacetic acid, indole acetic acid, and e) a high homovanillic acid/vanillylmandelic acid (HVA/VMA) ratio. " Order ALL the reference details at left...
5.	Hydrocortisone (Cortisol)FDA LinkGeneric 03/01/1992 - "We have investigated plasma beta-endorphin (beta-E), ACTH, and cortisol in two cases of congenital indifference to pain (CIP), a rare syndrome characterized by unresponsiveness to painful stimuli. " Order ALL the reference details at left...
6.	Homovanillic AcidIBA 01/01/1984 - "The congenital pain insensitivity syndrome is accompanied by: a) the presence of a yellow-brown pigment in the basal layer of the epidermis with the histochemical properties of melanin, b) decrease of urinary and blood concentration of dopamine and norepinephrine, c) excretion of melanin and of an abnormal as yet unidentified phenolic metabolite, d) increase in urinary p-hydroxyphenyllactic acid, p-hydroxyphenylacetic acid, indole acetic acid, and e) a high homovanillic acid/vanillylmandelic acid (HVA/VMA) ratio. " Order ALL the reference details at left...
7.	Dopamine (Intropin)FDA LinkGeneric 01/01/1984 - "The congenital pain insensitivity syndrome is accompanied by: a) the presence of a yellow-brown pigment in the basal layer of the epidermis with the histochemical properties of melanin, b) decrease of urinary and blood concentration of dopamine and norepinephrine, c) excretion of melanin and of an abnormal as yet unidentified phenolic metabolite, d) increase in urinary p-hydroxyphenyllactic acid, p-hydroxyphenylacetic acid, indole acetic acid, and e) a high homovanillic acid/vanillylmandelic acid (HVA/VMA) ratio. " Order ALL the reference details at left...
8.	CatecholaminesIBA 01/01/1984 - "Catecholamines and congenital pain insensitivity." Order ALL the reference details at left...
9.	CalciumIBA 01/01/1971 - "Congenital indifference to pain associated with a defect in calcium metabolism." Order ALL the reference details at left...
10.	beta-Endorphin (Endorphin, beta)IBA 03/01/1992 - "Plasma beta-endorphin levels and natural-killer cells in two cases of congenital indifference to pain." 03/01/1992 - "We have investigated plasma beta-endorphin (beta-E), ACTH, and cortisol in two cases of congenital indifference to pain (CIP), a rare syndrome characterized by unresponsiveness to painful stimuli. " Order ALL the reference details at left...

Therapies and Procedures

1.	Analgesia 12/15/1984 - "Intracerebroventricular injection of cerebrospinal fluid (CSF) from a patient with congenital indifference to pain induces analgesia in rats." 12/15/1984 - "CSF from a patient with congenital indifference to pain was found to produce analgesia in the rat following intracerebroventricular injections. " 04/01/1986 - "Bilateral amputations were performed without analgesia on a patient with congenital insensitivity to pain (congenital analgesia). " 09/01/1998 - "Congenital analgesia can vary from simple analgesia without any systemic dysfunction to more serious conditions associated with peripheral neuropathy, self-mutilation, and mental retardation. " Order ALL the reference details at left...
2.	Rhizotomy 06/01/1981 - "Animal models of chronic pain: their possible validation from human experience with posterior rhizotomy and congenital analgesia (Part I of the second John J. " 12/01/1985 - "Doubts concerning the validity of this animal model have been allayed by reports of dysesthesias in humans with spinal posterior rhizotomies or ganglionectomies, and also those with congenital analgesia. " Order ALL the reference details at left...
3.	Amputation 09/01/1986 - "A case of a variant of congenital indifference to pain, in which the patient initially developed lower extremity neurogenic arthropathy ultimately requiring bilateral above-the-knee amputations, is presented. " 04/01/1986 - "Bilateral amputations were performed without analgesia on a patient with congenital insensitivity to pain (congenital analgesia). " Order ALL the reference details at left...
4.	Ganglionectomy 12/01/1985 - "Doubts concerning the validity of this animal model have been allayed by reports of dysesthesias in humans with spinal posterior rhizotomies or ganglionectomies, and also those with congenital analgesia. " Order ALL the reference details at left...
5.	Injections 12/15/1984 - "CSF from a patient with congenital indifference to pain was found to produce analgesia in the rat following intracerebroventricular injections. " Order ALL the reference details at left...

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