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[The significance of adrenergic receptors for the development of nevus flammeus and nevus anemicus (author's transl)].

Examination of patients with nevus flammeus or nevus anemicus showed disturbed sensibility in the area of the nevus in the majority of cases. Histologically and with special technique of histochemistry and fluorescencemicroscopy there was no evidence for neurogenic lesions. However, signs of vegetative dysfunction were present: hyperhidrosis and absent reactivity of vasculature in the nevus area to vasoconstrictive and vasodilatatory stimuli. Based on these findings a disturbed regulation of vascular intramural adrenergic receptors seemed possible and really could be demonstrated by means of autoradiography. In both types of nevi only one of the adrenergic receptors could be marked with specific antagonists. Therefore the persistent vascular dilatation and constriction can be accounted for by the absence of one of these receptors. This abnormal distribution of receptors could be due to a developmental defect influenced by the "nerve growth factor".

Adolescent↗

The genetics of follicular atrophoderma.

Follicular atrophoderma apparently does not occur alone but is associated with other abnormalities. It is present in three groups: (1) If it is associated with chondrodystrophia calcificans congenita, it occurs only in girls and is due either to an X-chromosomal gene causing the changes in the heterozygote (ie, in the female sex) and penatal death in the hemizygous state (ie, in the male sex), or to an autosomal dominant gene with expression almost wholly limited to the female sex; (2) If it is associated with Bazex's syndrome, it occurs in male and female persons in successive generations and is due either to an autosomal dominant gene or to an X-linked dominant gene; and (3) If it occurs in association with keratosis palmoplantaris dissipata, keratosis follicularis, and hyperhidrosis palmoplantaris, it may be due to a recessive gene or a mutation.

Atrophy↗

[Ullrich syndrome: a hypotonic disorder of early infancy, difficult to define as an entity].

Three cases, a female and two males, with the diagnosis of Ullrich syndrome are presented. Major clinical findings included congenital hypotonia, joint contractures, high-arched palate, prominent calcaneus, scoliosis, hyperhidrosis and normal intelligence. The course was not progressive or even underwent slow improvement. Our cases followed the autosomal recessive pattern of inheritance. Muscle enzymes were all within the normal ranges. EMG showed findings that were consistent with a chronic myogenic pattern. Muscle biopsy revealed variation in the size of muscle fiber diameters and large increase of the connective tissue without evidence of dystrophic changes. We consider Ullrich syndrome as a nonspecific muscle disturbance of unexplained nature, related, but not identical, to both congenital muscular dystrophy and myogenic type of arthrogryposis multiplex congenita.

Calcaneus↗

[Pachydermoperiostosis (Touraine-Solente-Golé syndrome). Clinical and radiological differential diagnosis (author's transl)].

This report describes a patient with pachydermoperiostosis (Touraine-Solente-Golé-syndrome). This disorder is characterized by clubbing of the terminal phalanges with watchglass-like nails, enlargement of hands and feet, pachydermia and periostotic skeletal changes. The signs of pachydermia are cutis verticis et frontis gyrata, ptosis and increased skin-thickness of the palms of the hand and soles of the feet. Frequently, a hyperhidrosis of the hands and feet can be found. The differential diagnosis of clinical symptoms and changes in the bones is discussed in detail.

Adult↗

[Isaacs and Mertens syndrome: report of one case (author's transl)].

The author reports a clinical case of Isaacs-Mertens syndrome (syndrome of continuous muscle fibre activity, neuromyotonia, pseudomyotonia). A 55 years old man had increasing stiffness, painful spasms and myokymias of the peripheral muscle, associated with hyperhidrosis and absent tendon reflexes. Electromyography showed continuous electrical activity while at rest, general anaesthesia, after intravenous injection of diazepam and after peripheral nerve block. The activity disappeared after muscle infiltration of procaine and after intravenous injection of succinylcholine. After two years treatment with carbamazepine, 600 mg daily, the patient showed a normal clinical and neurophysiological picture. The etiology of the disease is unknown. The defect probably lies in the terminal motor network. A review of previous literature shows a typical uniformity of the electromyographic findings and of improvement on phenytoin and carbamazepine therapy, while there is a relative polymorphism of the clinical features.

Carbamazepine↗

[Dyskeratosis congenita Zinsser-Cole-Engman form. Report of two affected brothers (author's transl)].

Cutaneous pigmentation, lingual leukoplasia and dystrophic changes of nails are present in the two cases. The other clinical manifestations are dental alterations, epiphora, loss of dermal ridges of the pulp with hyperhidrosis, atrophic skin of the dorsum of the hands. Dysphagia and bone marrow hypoplasia are present in one case. The proband (case 1) has normal values for the following: hemoglobin electrophoresis, pyruvate kinase, marrow and blood chromosome analysis. Biopsy of pigmented skin showed an atrophic epidermis with orthokeratotic-hyperkeratosis; in the higher dermis there were several melanophores. Multiple layers of vasal lamina are seen under electron microscopy. The parents and the two daughters are free of clinical or hematologic manifestations. The mother and her two affected sons have A1-BW 27 HLA haplotype. X-linked transmission is discussed.

Adolescent↗

[Thermal state of the body following exposure to UHF radiation].

A mathematical model of thermal heating of animal exposed to UHF-irradiation is suggested. Besides physical heat transfer between the organism and its surroundings, the model takes into account physiological mechanism of thermal regulation-hypernea and hyperhidrosis. Possible evaluation of exposure limits for microwave irradiation is considered on the basis of the model. Prediction for the model are well consistent with GOST (USSR Government Standard) requirements under the assumption that utmost safety increase of the body temperature in UHF-field is 0.01 degrees C.

Body Temperature↗

[Choice of anaesthesia technique in thoracic endoscopic sympathectomy (author's transl)].

Thoracic endoscopic sympathectomy (TES) is a short surgical procedure used for the treatment of palmar and axillary hyperhidrosis. It involves pneumothorax, the lateral and head up position, and necessitates minimal chest excursions during breathing, so that a special type of anaesthesia is required. In 13 patients two different methods were compared and blood gas analysis as well as circulatory parameters were studied. We therefore propose an anaesthetic method using controlled mechanical ventilation; relaxation; intravenous anaesthesia; at least 1 1 fluid replacement; and careful monitoring.

Adolescent↗

Anaesthetic implications for transthoracic endoscopic sympathectomy.

Transthoracic endoscopic sympathectomy is now considered the treatment of choice for patients with upper limb hyperhidrosis requiring sympathetic ablation. This procedure requires the use of an endobronchial double lumen tube and subsequent one-lung anaesthesia, a technique that is associated with a number of potential problems. Full patient monitoring is thus required and includes pulse, ECG, non-invasive blood pressure measurement, pulse oximetry, end-tidal carbon dioxide concentration and peak inspiratory airway pressure. We reviewed our anaesthetic technique and peri-operative complications in 26 patients, to assess patient safety. In our study hypoxaemia occurred commonly but was transient in all bar one case where re-expansion of the lung was required. Hypotension occurred at two stages of the procedure, but active intervention was not required, and two patients required underwater drainage of the pleural cavity for treatment of pneumothorax. With skilled anaesthetic personnel and adequate monitoring this procedure may be carried out safely.

Adolescent↗

The punch and purse-string suture technique.

Palmar hyperhidrosis can be successfully treated by thoracoscopic sympathicotomy, the anterior approach being the most common one. Unfortunately the resulting scars are blemishing. Incision on the upper frontside of thorax leave conspicuous scars and not uncommonly keloids. Likewise the traditional method used for radical excision of naevi leaves large scars in comparison with the size of the mark removed. With the increased awareness of malignant melanoma excision of birthmarks and naevi is increasingly being demanded. There is also a desire for more cosmetically acceptable scars. Is it possible, then, to reduce the size of these scars and make them less conspicuous? In two separate studies a new method has been designed and tested--the punch and purse-string suture technique by which the size of the scars is reduced and of a different shape. In one of the studies, the traditional method and the new technique were compared in the same patient.

Cicatrix↗

The history of cervicothoracic sympathectomy.

As early as in 1889 surgery on the cervical sympathetic nervous system was performed. During the following decades this operation was tried for a variety of diseases. In the early 1920s it was clarified that patients with hyperhidrosis, vasospastic conditions, and angina pectoris would benefit from stellectomy. It was, however, soon discovered that removal of the upper thoracic ganglia was required in order to obtain complete sympathetic denervation of the upper extremity. Several open surgical techniques for upper thoracic sympathectomy were described. During the 1940s a few pioneers started to excise sympathetic ganglia by thoracoscopy which had originally been described as a diagnostic tool by Jacobaeus in 1910. The endoscopic approach, amply documented by Kux in 1954, did not, however, gain widespread popularity until the 1980s. Like the general upsurge of interest in endoscopic surgery, thoracoscopic ablation of the upper thoracic sympathetic ganglia is now rapidly being adopted by surgeons.

Ganglia, Sympathetic↗

Iontophoresis in drug delivery: basic principles and applications.

Passive delivery of most compounds across different epithelia is limited due to the barrier properties afforded by these epithelia. Iontophoresis is a novel drug delivery system designed to improve the delivery rate of compounds. The technique generates an electrical potential gradient that facilitates the movement of solute ions across the membrane. Iontophoresis has been used has been used greatest success in therapy of hyperhidrosis. The potential of iontophoresis for systemic delivery is being rediscovered, and the technique has been observed to be particularly effective for ionic drugs. It also enhances transdermal permeation of neutral compounds by the process of electroosmosis. The recent enthusiasm for iontophoresis may also be attributed to successful production of therapeutically active protein and peptide drugs by employing recombinant DNA technology. Because of their charged nature and relatively large molecular size, iontophoresis may provide means for their effective delivery. This review discusses the basic principles and applications of iontophoresis in dermatology, ophthalmology, otolaryngology, and dentistry. In addition, the systemic applications of iontophoresis with emphasis on protein and peptide delivery are reviewed.

Animals↗

[Changes of arterial oxygen tension in supine position during one-lung anesthesia].

BACKGROUND: One-lung ventilation during anesthesia (one-lung anesthesia) in patients under lateral decubitus position to help performing intra-thoracic surgical procedures was well known to have larger alveolar-to-arterial oxygen tension difference and lower arterial oxygen tension (PaO2) as compared to two-lung ventilation. In the present study, we investigate the changes of arterial oxygen tension in the supine position during one-lung anesthesia. METHODS: Forty-two patients of palmar hyperhidrosis, ASA class I-II, scheduled to receive bilateral transthoracic endoscopic sympathectomy were studied. After anesthetic induction (fentanyl, thiopental, and succinylcholine), a 35 (for female) or 37 (for male) French left-sided Robertshaw double-lumen endobronchial tube was intubated. Anesthesia was maintained with isoflurane 1.0-1.5% and 50% O2-N2O. They were changed to isoflurane 1.5-2.0% and 100% O2 during one-lung ventilation. Once the surgical operation is completed, they were changed to 100% O2 and two-lung ventilation. Arterial blood gases were measured at 4 phases: 5 min after endobronchial intubation (two-lung ventilation), 5 min after left one-lung ventilation, 5 min after right one-lung ventilation, and 5 min after accomplishing operation (two-lung ventilation). RESULTS: The results showed PaO2 were significantly lower in left and right one-lung ventilation with 100% O2 as compared with that obtained from two-lung ventilation with 50% O2 5 min after endobronchial intubation (p < 0.05). Furthermore, right one-lung ventilation had a lower PaO2 than left one-lung ventilation (p < 0.05). CONCLUSIONS: We conclude that arterial oxygen tension can be safely maintained during one-lung anesthesia with pure oxygen in healthy patients lying in a supine position.

Adolescent↗

Intravenous tenoxicam reduces dose and side effects of PCA morphine in patients after thoracic endoscopic sympathectomy.

BACKGROUND: Among surgical modalities for treatment of palmar hyperhidrosis, endoscopic sympathectomy is the most popular choice in recent years. After surgery, the major complaint was anterior chest pain. This study was conducted to evaluate the analgesic efficacy and side effects of tenoxicam (a thienothiazine derivative) in combination with patient-controlled analgesia (PCA) using morphine in patients who received thoracic endoscopic sympathectomy. METHODS: Forty-one ASA class I patients who underwent endoscopic sympathectomy (T2 and T3 ganglia) were randomly divided into two groups. Operation was conducted under general anesthesia with single lumen endotracheal intubation. No narcotic was given during the operation except for fentanyl (3 micrograms/kg) during induction. After surgery, patients in group I received PCA morphine only and patients in group II received PCA morphine plus tenoxicam (20 mg, i.v.) immediately for pain relief. In addition, rescue analgesia with intramuscular meperidine (1 mg/kg) was available to each patient every 4 h prn. The intensity of pain was assessed with VAS pain score every 4 h for 24 h. The frequency of demand and doses of delivered PCA morphine were recorded. RESULTS: Results showed no statistically significant difference between groups in respect of age, body height, body weight and pain scores. However, based upon similar qualitative pain relief, patients in group II revealed less demand for analgesic, less doses of morphine requirement and less side effects. CONCLUSIONS: Tenoxicam may be an effective adjuvant to PCA morphine for postoperative pain control. This combination reduces the total consumption of PCA morphine with less side effects.

Adult↗

[Thoracic lymphadenectomy in video-assisted mini-invasive thoracic surgery. First experience].

The Authors report their experience in the thoracoscopic surgical treatment of neurovascular abnormalities of the upper extremities. The experience acquired in the last two years with mini-invasive video-assisted thoracic surgery of the pleura and lung forced the Authors to perform thoracic sympathectomy following the same approach. In this way 4 thoracic sympathectomies were performed in 3 patients. Two of them presented Raynaud's phenomenon, and one was affected by bilateral idiophatic upper limb hyperhidrosis. The operation is performed thanks to 3 12mm Thoracoports that allow the introduction of the telescope and operative instruments. It is easy to recognize and remove the sympathetic gangliar chain from T2 to T3. The short operative time required, the absence of mortality and morbidity, the regression of the symptomatology, the rapid return to work authorize to consider endoscopic transthoracic sympathectomy the gold standard in the treatment of neurovascular abnormalities of the upper extremities.

Female↗

Use of a single lumen endotracheal tube and continuous CO2 insufflation in transthoracic endoscopic sympathectomy.

BACKGROUND: Transthoracic endoscopic sympathectomy (TES) is an accepted standard surgical treatment for palmar hyperhidrosis. For anesthetic management, a double lumen endobronchial tube is usually used to deflate the lung on the operative side. Recently we have applied continuous insufflation of carbon dioxide (CO2) into the pleural cavity to merely compress one lung while ventilating both lungs with a conventional single lumen endotracheal tube. METHODS: We have studied 45 patients (ASA I and II) who underwent bilateral TES, Thirty-three were ventilated by single lumen tube and the other 12 by double lumen endobronchial tube with one lung ventilation. In both groups I and II, CO2 was insufflated slowly through the scope to a intrapleural pressure of 20 cm H2O. Both lungs were ventilated with peak pressure of less than 20 cmH2O at tidal volume of 5-12 ml/kg at 10-16 beats/min. RESULTS: In these patients, no CO2 retention was noted. Oxygenation and cardiovascular stability were maintained and there were no complications. In the double lumen series, we found that oxygen saturation was less uniform and less stable than those in the single lumen series. CONCLUSIONS: It is concluded that the use of single lumen endotracheal tube with continuous insufflation of CO2 in TES is easy, simple and safe.

Adolescent↗

[Laparoscopic and thoracoscopic surgery in children and adolescents].

Initial experience with laparoscopic and thoracoscopic surgery in children during the year Sept. 1992-Sept. 1993 is presented. The 36 procedures included 20 appendectomies, 3 cholecystectomies, 10 upper thoracic sympathectomies, 1 bilateral oophorectomy and 2 diagnostic laparoscopies. Indications were appendicitis, symptomatic gallstones, idiopathic primary palmar hyperhidrosis, and gonadal dysgenesis (xo/xy mosaicism) in a 13-year-old girl. 2 diagnostic laparoscopies were performed for chronic abdominal pain and for a possible retroperitoneal lesion, respectively. All procedures were successfully managed without complications. In 2 cases of acute appendicitis it was necessary to change to the open conventional technique because of technical difficulties. Duration of hospitalization for cholecystectomy and sympathectomy was significantly shorter than with the conventional surgical approach and convalescence was excellent, with less postoperative pain. As for the surgical approach in acute appendicitis, we cannot as yet decide whether or not laparoscopy is superior to the conventional technique.

Abdominal Pain↗

[The neurological aspects of chromaffinoma].

Vegetative dysfunction with episodes of tachycardia, hypertension, paleness, general hyperhidrosis, subfebrile condition, hyperglycemia manifest first among clinical symptoms of chromaffinoma. In respect to these symptoms severity the crises observed in chromaffinoma are classified as minor or major. Major crises may be cerebral, epileptiform, cardial and abdominal. The crises are attributed to hypercatecholaminergic arterial hypertension. A reliable laboratory diagnosis of the disease involves urinary measurements of catecholamines and their metabolites. Manifold increase of the latter concentrations may serve the diagnostic indication. The only effective treatment is the removal of the tumor from the adrenals.

Adolescent↗