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Clin Ter. 2002 Nov-Dec;153(6):367-72.
[Pharmacological sympathetic block in complex regional pain syndrome]

[Article in Italian]

Verre M, De Santis F, Glyronakis S, Grande AM, Renzi A, Santangelo E, Tortorella V, Varano M.

U.O. di Anestesia e Rianimazione, Ospedale Civile di Soveria Mannelli, Universita degli Studi Magna Graecia, Catanzaro, Italia. marioverriscalinet.it

PURPOSE: The Complex Regional Pain Syndrome (CRPS) is a chronic pain state provoked by lesions of the soft tissues or of the bony tissues (type CRPS-I or reflex sympathetic dystrophy-RSD) or by lesions of the nerves (type CRPS-II or causalgia) with vegetative alterations (perspiration, vasomotory alterations) and trophic alterations (bony cutaneous atrophy, alopecia, articular contractures). The pharmacological block of the sympathetic nerves through a peripheral vein is inserted in the multidisciplinary approach that characterizes the therapy of this syndrome. MATERIALS AND METHODS: A retrospective survey was carried out on a group of 185 patients affected by RDS/CRPS with block of the sympathetic nerves through a peripheral vein with guanethidine. Superior limb: Inflation of the tourniquet till disappearance of the radial wrist. Cannulation of a peripheral vein with Butterfly needle n. 23. Guanethidine 10 mg, lidocaine 20 mg, sodic heparin 500 u.i, NaCl 0.9% 20 ml. Injection in 5 minutes. Permanence of the pneumatic tourniquet inflated above systolic blood pressure for 15 minutes. Deflation slowly. Inferior limb: Inflation of the tourniquet till disappearance of the pedidium wrist. Cannulation of a peripheral vein with Butterfly needle n. 23. Guanethidine 20 mg, lidocaine 40 mg, sodic heparin 1000 u.i, NaCl 0.9% 40 ml. Injection in 5 minutes. Permanence of the pneumatic tourniquet inflated above systolic blood pressure for 15 minutes. Deflation slowly. RESULTS: The first stage (hyperemic) showed the highest incidence of remissions: (83, 33%). Even in the second stage (dystrophic) the answer to the therapy has been fundamentally positive: (53, 68%). In the third stage (atrophic) the results have been more modest: (8, 33%). CONCLUSIONS: The block of sympathetic system with guanethidine is still an important method in the therapy of the CRPS; in fact it is surely less invading than the blocks of the stellate ganglion or of the lumbar sympathetic.

online pharmacy ref. source: www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=12645391&dopt=Abstract

Hum Genet. 2003 Jan;112(1):78-83. Epub 2002 Oct 24.
Skewed X-chromosome inactivation causes intra-familial phenotypic variation of an EBP mutation in a family with X-linked dominant chondrodysplasia punctata.

Shirahama S, Miyahara A, Kitoh H, Honda A, Kawase A, Yamada K, Mabuchi A, Kura H, Yokoyama Y, Tsutsumi M, Ikeda T, Tanaka N, Nishimura G, Ohashi H, Ikegawa S.

Center for Molecular Biology and Cytogenetics, SRL Inc., Hino-shi, Tokyo, Japan.

X-linked dominant chondrodysplasia punctata (CDPX2) is a skeletal dysplasia characterized by stippled epiphyses, cataracts, alopecia and skin lesions, including ichthyosis. CDPX2 exhibits a number of perplexing clinical features, such as intra- and inter-familial variation, anticipation, incomplete penetrance and possible gonadal and somatic mosaicism. Recently, mutations in the gene encoding Delta8,Delta7 sterol isomerase/emopamil-binding protein (EBP) have been identified in CDPX2. To better understand the genetics of CDPX2, we examined the entire EBP gene by direct sequencing in four CDPX2 patients. We found EBP mutations in all four patients, including three novel mutations: IVS3+1G>A, Y165C and W82C. Surprisingly, a known mutation (R147H) was identified in a patient and her clinically unaffected mother. Expression analysis revealed the mutant allele was predominantly expressed in the patient, while both alleles were expressed in the mother. Methylation analysis revealed that the wild-type allele was predominantly inactivated in the patient, while the mutated allele was predominantly inactivated in her mother. Thus, differences in expression of the mutated allele caused by skewed X-chromosome inactivation produced the diverse phenotypes within the family. Our findings could explain some of the perplexing features of CDPX2. The possibility that an apparently normal parent is a carrier should be considered when examining seemingly sporadic cases and providing genetic counseling to CDPX2 families.

online pharmacy ref. source: www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=12483303&dopt=Abstract

Indian J Sex Transm Dis. 1990;11(2):66-7.
Syphilitic alopecia.

Abdul Gaffoor PM.

PIP: A case of alopecia as the only symptom of secondary syphilis in a 32-year old Indian man is described. The man presented with patchy hair loss on the scalp, eyebrows, chest and legs, and generalized nontender lymphadenopathy. Laboratory tests were positive for RPR (rapid plasma reagin test) at 1:64, FTA Ab (fluorescent treponema antibody absorption test). He had a history of heterosexual contact 9 months previously. He was treated with procaine penicillin 600,000 units im daily for 10 days, and hair growth resumed within 6 weeks. Hair loss described as "moth eaten alopecia" is common in secondary syphilis. Other patterns of hair loss related to treponema infection include diffuse, extensive alopecia as a penicillin reaction, and peripheral scalp[ alopecia in infants with congenital syphilis.

online pharmacy ref. source: www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=12343561&dopt=Abstract

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