Catecholamine Metabolites in Congenital Sensory Neuropathy with Anhydrosis

PEDIATRICS ◽  
1980 ◽  
Vol 65 (1) ◽  
pp. 154-157
Author(s):  
Walid O. Shekim ◽  
Anasseril E. Daniel ◽  
Richard L. Koresko ◽  
Harutoune Dekirmenjian

Congenital sensory neuropathy with anhydrosis is a rare syndrome in childhood, characterized by disturbed thermoregulation and by absence of pain and sweating.1,2 The etiology is unknown, but researchers postulate the presence of a disorder in the function of the autonomic sympathetic nervous system.3 From among the numberous etiologies considered to play a role in the pathophysiology of the syndrome, we list a few that are relevant to this report: (1) disturbance of catecholamine metabolism,3 (2) an inborn error of metabolism involving a minor pathway of tyrosine metabolism,4 and (3) low biological activity of nerve growth factor (NGF).5 Clinicians have used urinary catecholamine metabolites in the diagnosis of the syndrome.3

2012 ◽  
Vol 2012 ◽  
pp. 1-5 ◽  
Author(s):  
Jianguo Cheng ◽  
Anuj Daftari ◽  
Lan Zhou

The sympathetic nervous system has been implicated in pain associated with painful diabetic neuropathy. However, therapeutic intervention targeted at the sympathetic nervous system has not been established. We thus tested the hypothesis that sympathetic nerve blocks significantly reduce pain in a patient with painful diabetic neuropathy who has failed multiple pharmacological treatments. The diagnosis of small fiber sensory neuropathy was based on clinical presentations and confirmed by skin biopsies. A series of 9 lumbar sympathetic blocks over a 26-month period provided sustained pain relief in his legs. Additional thoracic paravertebral blocks further provided control of the pain in the trunk which can occasionally be seen in severe diabetic neuropathy cases, consequent to extensive involvement of the intercostal nerves. These blocks provided sustained and significant pain relief and improvement of quality of life over a period of more than two years. We thus provided the first clinical evidence supporting the notion that sympathetic nervous system plays a critical role in painful diabetic neuropathy and sympathetic blocks can be an effective management modality of painful diabetic neuropathy. We concluded that the sympathetic nervous system is a valuable therapeutic target of pharmacological and interventional modalities of treatments in painful diabetic neuropathy patients.


1990 ◽  
Vol 258 (1) ◽  
pp. H107-H112
Author(s):  
K. G. Cornish ◽  
M. W. Barazanji ◽  
R. Iaffaldano

The contribution of the autonomic nervous system, angiotensin II (ANG II), and arginine vasopressin (AVP) to the control of blood pressure (BP) was examined in 12 chronically instrumented tethered monkeys. The vasopressin antagonist, [d(CH2)5AVP] (Manning Compound, MC), the ANG II antagonist, saralasin (SAR), and the ganglionic blocking drug, hexamethonium (Hx), were injected in a random sequence into the left atrium (LA) while BP and heart rate (HR) were monitored. When given as the first antagonist, MC caused a slight decrease in BP; SAR did not significantly decrease BP regardless of the sequence of administration, whereas Hx caused a consistent decrease in blood pressure of 35–50 mmHg. Seven (4 intact and 3 with renal denervation) additional animals were involved in hemorrhage experiments. Blood pressure was reduced to 50–60 mmHg by hemorrhage and then allowed to return spontaneously. Ten to 15 min after the end of the hemorrhage, MC was given. When blood pressure had stabilized, SAR was given. Blood pressure returned to 80–90 mmHg after the hemorrhage. MC did not affect the blood pressure recovery; however, saralasin reduced it to the post-hemorrhage levels. We would conclude that the sympathetic nervous system is the primary controlling mechanism for BP in the conscious primate, with AVP making a minor contribution. The release of renin would appear to be primarily under the control of the sympathetic nervous system.


PEDIATRICS ◽  
1978 ◽  
Vol 62 (4) ◽  
pp. 588-590
Author(s):  
F. Bruder Stapleton ◽  
R. R. Skoglund ◽  
Robert B. Daggett

Guillain-Barré syndrome (GBS) is frequently complicated by hypertension, which has been ascribed to sympathetic nervous system hyperactivity. We report the case of a child with GBS and hypertension that appeared to be related to increased renin-angiotensin activity. Urinary catecholamine excretion was normal. Propranolol therapy successfully controlled the hypertension. Evaluation of the renin-angiotensin system and catecholamine excretion is indicated in patients with GBS and hypertension.


Author(s):  
E. A. Skorniakova ◽  
L. G. Zaslavskii ◽  
N. A. Neofidov ◽  
S. L. Vorobev ◽  
A. A. Gotovchikov ◽  
...  

Neuroblastoma is a malignant tumor derived from the neuroblasts of the sympathetic nervous system, which develop in any region of the nervous system. Usually, neuroblastoma is detected in children aged 1–2 years. About 90% of cases are diagnosed before the age of 5 years. The incidence of adult neuroblastoma is only 0.3 cases per million people per year. The clinical course and biological activity of adult neuroblastoma is different than children neuroblastoma. Early diagnosis of this disease in adults is necessary for timely start of treatment and increasing life expectancy. In this clinical observation, we present a detailed description of the course of this rare disease in the 34-year-old male and literature review on adult neuroblastoma.


1991 ◽  
Vol 261 (4) ◽  
pp. R1045-R1051 ◽  
Author(s):  
M. Lo ◽  
C. Julien ◽  
C. Barres ◽  
F. Boomsma ◽  
C. Cerutti ◽  
...  

To assess the role of the sympathetic nervous system in the development of genetic hypertension, blood pressure (BP) was recorded in conscious adult Lyon hypertensive (LH) and normotensive (LN) rats that had received daily injections of saline or guanethidine at 1-13 wk of age. Guanethidine abolished the pressor response to tyramine, decreased plasma norepinephrine by greater than 70% and plasma 3,4-dihydroxyphenylglycol by approximately 90%, and did not change plasma epinephrine. Bilateral adrenalectomy further reduced plasma norepinephrine to 8 and 5% of control levels in LH and LN rats, respectively. BP was lowered (-7%) in sympathectomized rats, but the mean absolute BP difference between LH and LN rats was unaltered. Despite marked supersensitivity to alpha-adrenoreceptor stimulation, phentolamine induced only a small transient depressor response, which was abolished by adrenalectomy in sympathectomized rats. It is concluded that the sympathetic nervous system is not necessary for the development of hypertension in LH rats. After sympathectomy, circulating catecholamines, which mostly derive from the adrenal medulla, play only a minor role in BP maintenance.


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