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Familial amyloid polyneuropathy: new developments in genetics and treatment.

Familial amyloid polyneuropathy is an hereditary amyloidosis related to several different genetic errors. It usually presents as a severe peripheral neuropathy. The protein most frequently involved in the disease is transthyretin, a serum transport protein synthesized primarily in the liver. Variable penetrance and variable clinical expression are widely described but the factors that influence such variability are largely unknown. Liver transplantation has been suggested as an effective treatment for this fatal condition. More than 146 patients have undergone this procedure and progression of the disease is halted after surgery. Therefore, it is thought that liver transplantation is an effective treatment for severe forms of familial amyloid polyneuropathy.

Amyloid Neuropathies↗

Advances in rectal cancer surgery in Japan.

A review of advancement of rectal cancer surgery in Japan is presented. The standard operation for rectal cancer was altered in the 1960s from abdominoperineal resection to the pull-through technique and the handsewn anterior resection in the 1970s, and it became the stapled anterior resection in the 1980s. Today, more than 75 percent of rectal cancers are treated with sphincter-preserving anterior resections, and the remaining 20 percent by abdominoperineal resections. Colonic J-pouch is used with anastomoses involving very low anterior rectal resection for cancers. In the late 1970s, a method of dissecting extended pelvic nodes was adopted to decrease local recurrence. However, extended dissection has been applied to only T3 and T4 cancers of the lower rectum because of postoperative dysfunction of pelvic organs. This was caused by injury to the pelvic nerve plexus, thus lowering the quality of life of the patients. Since the middle of the 1980s, the autonomic nerve-preserving operation attracted surgeons' attention because it prevented these dysfunctions from occurring as a result of the treatment of cancer in the upper rectum and for T1 or T2 cancers in the lower rectum. In this article, recent advances in rectal cancer surgery in Japan are reviewed.

Colorectal Surgery↗

[Prognostic factors in ruptured intracranial aneurysm. Significance of the temperature curve].

This report concerns 130 consecutive patients admitted as emergencies for ruptured intracranial aneurysms. The poorer the patient's clinical condition, the longer the time elapsed before surgery (14 days on average). While awaiting surgery, 4.6 per cent of the patients rebled and 5.4 per cent deteriorated due to vasospasm. The 14 patients who had shown no symptom since the rupture remained asymptomatic until surgery, while the clinical status of the other 116 patients improved in 36.3 per cent, remained unchanged in 53.4 per cent and deteriorated in 10.3 per cent (3 per cent of whom died). After a one year follow-up, 58 per cent of the 126 patients operated upon had resumed all their previous activities; 10.3 per cent were autonomous but had been unable to resume their professional activities normally; 8.7 per cent were dependent and 20.5 per cent had died of neurological complications and/or sequelae (2.4 per cent had died of intercurrent diseases). An analysis of prognostic factors showed that the outcome was not significantly influenced by the site of the aneurysms, the patient's sex or age, or a past history of systemic arterial hypertension, but it was narrowly dependent (P less than 0.001) upon the clinical status at the time of surgery, the amount of extravasated blood at CT or the degree of vasospasm at angiography. The greater the size of the haematoma and the degree of vasosplasm, the more the clinical status was affected (P less than 0.01). In addition, the clinical status was found to be particularly poor when hyperthermia was present (P less than 0.01), and the outcome was worse in patients who were operated upon during the ascending phase of fever (P less than 0.05). Thus, a careful examination of the temperature curve might be of help in the choice of the optimal time for surgery.

Adolescent↗

[Urologic complications in rectal surgery (author's transl)].

Urinary disorders after amputation or resection of the rectum are much more frequent than commonly supposed. Out of 239 patients they were noticed by 56.6%, undergoing amputation, and 36.6% after resection. Longterm disturbances were observed by 26.4% respect. 15.1%. The most important reason is injury of autonomic pelvic nerves and ganglia. Urinary bladder infection was verified in 22% respect. 8%. Furthermore urinary disorders may be caused by changing of bladder localization (bladder hernia), wound infection (especially of the sacral cavity), edema of the urethra, and exacerbation or manifestation of pre-existing urologic diseases. Compared to these, lesions of the urinary tract are of little importance. To prevent such complications, we postulate: 1. careful pre-operative urologic examination, 2. if possible mobilisation of the rectum along its borderlines (so-called Grenzlamellen of Pernkopf), 3. critical analysis of urologic disorders, removal of the urinary catheter as soon as possible, regular systemic prophylaxis of urinary infection, and therapy controlled by antibiogram, 4. adequate follow-up, and 5. urologic examination of all disturbances lasting longer than three months.

Age Factors↗

Bladder and male sexual functions after autonomic nerve-sparing TME with or without lateral node dissection for rectal cancer.

BACKGROUND: We evaluated to what extent lateral lymph node dissection (LND) interferes with bladder and male sexual functions after radical rectal excision with adoption of careful total autonomic nerve preservation. METHODS: The study comprised 77 patients resected for mid-rectal or lower rectal cancer. Bladder and male sexual functions were studied by means of a questionnaire more than one year after surgery. Outcomes were compared between patients who received lateral LND (group 1, 65 patients) and those who did not (group 2, 12 patients). RESULTS: Only minor disturbances of bladder function were reported in 10 patients (15%) of group 1, and in 3 patients (25%) of group 2. Ten out of 37 preoperatively sexually active patients (27%) in group 1 males and one of 5 patients (20%) in group 2 males had partial or total impotency after surgery and retrograde ejaculation occurred in 3 of 27 patients (11%) and one of 4 patients (25%), respectively. Erectile impotency occurred less frequently when patients were operated during the period 1993-1996 than during 1988-1992 (11% vs. 42%, p<0.05). The age was significantly greater among patients who had loss of ejaculation. CONCLUSIONS: If lateral lymph node dissection should be used with the aim of improving radicality in rectal excision for cancer, it should be combined with careful nerve-preserving technique--which may reduce the risk of bladder and male sexual dysfunctions.

Adult↗

Influence of hypophysectomy on the lifespan of the corpus luteum in the cyclic dog.

Five dogs were hypophysectomized on Day 4 and 9 on Day 18. Prolactin and LH stimulation tests showed that hypophysectomy was complete in 6 dogs only. In these dogs, the progesterone concentration was measured in the peripheral blood; it decreased sharply immediately after surgery. It regained normal values in 3 of the 4 dogs hypophysectomized on Day 4, and remained low in the 2 dogs hypophysectomized on Day 18. This indicates that, in the dog, luteal function is autonomous during a certain period. The luteal period of the 3 dogs hypophysectomized on Day 4 was shorter than that of control animals, although the time of onset of luteal regression appeared to be similar. This indicates that pituitary luteotrophic support is required during the second part of the oestrous cycle of the dog.

Animals↗

[The heart and diabetes mellitus].

The atherosclerotic process in the diabetic patient, is more common, it is noticed at early ages, advances more rapidly and almost equally affects males and females. This data, can not be explained on the basis of the association with other coronary heart disease risk factors, there is an intrinsic atherogenic factor attributed to diabetes, and can be related, to the early hyperinsulinemia, coagulation and lipid disorders, hyperglycemia or diabetic microangiopathy. Coronary heart disease has an increased prevalence in diabetic patients, that is not related with diabetes duration or the type of treatment. Early and late morbimortality after acute myocardial infarction and/or revascularization surgery is twice as common in the diabetic patient. Diabetic cardiomyopathy related to small vessels disease is still a matter of controversy and many authors doubt about its relevance in clinical practice. The presence of autonomic neuropathy with the cardiovascular denervation syndrome carries a poor prognosis. Early cardiovascular changes in asymptomatic patients, are detected with non-invasive test, and can help to introduce measures to protect individuals at a greater risk.

Cardiomyopathies↗

[Pseudotumoral form of hepatic tuberculosis. Apropos of a case report].

Concerning one case of autonomous hepatic tuberculosis in a pseudo-tumoral aspect, the difficulties of making the diagnosis are brought to light. Liver biopsy is the best examination to clarify things, either performed under ultrasonic control or during surgery. This biopsy permits pathologic examination and culture of a liver fragment in Lowenstein medium. But in fact, only isolation of the tubercle bacillus at the liver level confirms presence of tuberculosis.

Aged↗

The effects of reversal of neuromuscular blockade on autonomic control in the perioperative period.

Impaired parasympathetic control of heart rate is associated with increased incidence of cardiac dysrhythmias and ischemia. Anticholinergic drugs suppress parasympathetic control and could be detrimental in the early postoperative period in high-risk patients. In this double-blind randomized trial, 30 ASA physical status I and II patients undergoing minor surgery received either atropine 20 micrograms/kg and neostigmine 50 micrograms/kg (Group A), glycopyrrolate 8 micrograms/kg and neostigmine 50 micrograms/kg (Group G), or placebo (Group P) for reversal of neuromuscular blockade. Two indices of parasympathetic modulation of heart rate, spontaneous baroreflex sensitivity, and high-frequency heart rate variability, were assessed. At 2 h after reversal, Group A showed persisting impairment of baroreflex sensitivity with respect to Group P (7.12 +/- 0.86 vs 12.71 +/- 1.38 ms/mm Hg, P = 0.022) as well as decreased high-frequency heart rate variability (280.8 +/- 30.1 vs 569.2 +/- 115.2 ms2/Hz, P = 0.015). Groups A and G showed a borderline decrease in normalized high-frequency variability at 2 h (P = 0.05 for Groups A and G versus Group P). Anticholinergic drugs with neostigmine cause impairment of parasympathetic control of heart rate which persists into the early postoperative period. The effects of glycopyrrolate appear to be of shorter duration; this drug may thus be preferable in patients at risk of cardiovascular complications.

Adolescent↗

Changes in cardiac autonomic control during nocturnal repetitive oxygen desaturation episodes in patients with coronary artery disease.

BACKGROUND: Periodic fluctuations in respiration such as Cheyne-Stokes respiration or the sleep apnoea syndrome are known to be associated with very-low-frequency oscillations in heart rate (cycle times about 30-120s), which may be related to periodic changes in sympathetic and vagal tone. Since disturbances in cardiac sympathovagal balance have been associated with increased cardiovascular morbidity and mortality, especially in patients with coronary artery disease (CAD), we investigated changes in cardiac autonomic control in patients with CAD during periodic breathing episodes. METHODS: Thirty-five patients with a left ventricular ejection fraction (LVEF) of > 50% and 13 patients with an LVEF of < or = 35%, who were scheduled for coronary bypass surgery, were studied using a nocturnal Holter electrocardiograph (ECG) and pulse oximetric recordings. Heart rate variability was assessed by spectral analysis during cyclic oxygen desaturation episodes, indicating periodic breathing patterns, and during control episodes with normal oxygen saturation. RESULTS: Cyclic oxygen desaturation episodes with a duration of at least 20 min were observed in 17 patients with an LVEF of > 50% and in nine patients with an LVEF of < or = 35% (minimal oxygen saturation 83.9 +/- 3.1 versus 80.4 +/- 5.6%; cycle frequency between 0.0068 and 0.0294 Hz, no significant differences between groups), all of which were associated with very-low-frequency (0.0033-0.04 Hz) oscillations in the heart rate. During cyclic desaturation episodes patients with normal, as well as those with imparied left ventricular function, had marked increases (P < 0.01) in very low frequency power, low frequency (0.04-0.15 Hz) power and the ratio of low-to-high frequency (0.15-0.4 Hz) power. The low:high frequency ratio was significantly higher in patients with an LVEF of > 50% than in patients with an LVEF of < or = 35% during cyclic desaturation episodes, but not during control episodes. CONCLUSIONS: Repetitive oxygen desaturation episodes are associated with marked changes in heart rate variability which indicate cardiac sympathetic predominance in patients with CAD. Our results suggest differences in the modulation of cardiac sympathovagal balance during periodic breathing episodes between patients with normal and imparied left ventricular function.

Adult↗

The need for halothane supplementation of N2O-O2-relaxant anaesthesia in chronic alcoholics.

The demand for intermittant halothane supplementation during N2O-O2-relaxant anaesthesia was studied in 25 alcohlics (annual consumption over 15 1 pure alcohol) scheduled for biliary or gastric surgery. The controls were 45 non-alcoholics and 43 patients with an annual consumption of between 1 to 15 1. Thiopental (3 mg/kg/min) was given for induction. After intubation, halothane supplementation was given in 0.5% concentration for 10-min periods. Standardized criteria for halothane supplementation were various motor and autonomic responses to painful stimuli. Muscular relaxation was kept fairly constant (roughly 90%), as assessed visually with the aid of a peripheral nerve stimulator. The total time for which halothane supplementation was given, expressed as a percentage of the total anaesthesia time, was used as an indication of the need for halothane supplementation. The need for thiopental for induction was not increased to a statistically significant extent in alcoholics, but signs of excitation did occur in 40% as compared with 11% in non-alcoholics (P less than 0.01). The demand for halothane supplementation was higher in alcoholics (47 +/- 4.8%, s.e. mean) than in non-alcoholics (33 +/- 2.3%). This difference, however, was partly due to the higher incidence of gastric surgery, which required more supplementation than biliary surgery. Analysis of the different criteria indicating the need for halothane supplementation revealed that an increase in blood pressure or heart rate was more common in non-alcoholics, whereas motor irritability, sweating and lacrimation were more frequent in alcoholics. Management of the anaesthetic posed no special difficulties in the alcoholics with an estimated mean annual consumption of 32 +/- 4 (s.e. mean) litres of absolute alcohol. Three patients (5% of the alcohol consumers) reported dreams or recollections, suggesting that this mode of halothane supplementation does not guarantee an adequate anaesthetic depth. The difficulties and biases associated with this type of analysis are discussed.

Alcoholism↗

Coronary artery bypass grafting is associated with a significant worsening of QT dynamicity and heart rate variability.

BACKGROUND: Imbalance in autonomic nervous system and impaired myocardial repolarization has been shown to increase the risk for arrhythmias in patients with coronary artery disease. This study evaluated the effects of coronary artery bypass grafting (CABG) on heart rate variability and QT interval dynamicity in subjects with coronary artery disease undergoing elective CABG surgery. METHODS: The study group consisted of 68 consecutive patients (mean age +/-SD: 61 +/- 9 years) with coronary artery disease who underwent elective CABG. Twenty-four-hour Holter monitoring was performed 2-5 days before cardiac surgery and was repeated 10 days after CABG. ELATEC holter software was used to calculate heart rate variability and QT dynamicity parameters. All subjects had a complete history, laboratory examination and transthoracic echocardiography. RESULTS: All patients had beta-blocking agent medication pre- and postoperatively. Standard deviation of all NN intervals for a selected time period, square root of the mean of the sum of the squares of differences between adjacent RR intervals, the proportion of differences in successive NN intervals greater than 50 ms, normalized low-frequency power, and normalized high-frequency power were significantly decreased after CABG surgery, whereas low-frequency/high-frequency ratio was significantly increased after CABG. QT/RR slopes over 24 h were significantly increased after CABG surgery for QT end and QT apex (QTapex/RR: 0.16 +/- 0.13 vs. 0.28 +/- 0.19, p < 0.001; QTend/RR: 0.18 +/- 0.13 vs. 0.36 +/- 0.23, p < 0.001). CONCLUSION: This prospective study showed for the first time that CABG was associated with a significant worsening of heart rate variability and QT dynamicity parameters in the postoperative period.

Adrenergic beta-Antagonists↗

[Effect of general and epidural anesthesia on autonomic nervous system].

OBJECTIVE: To evaluate autonomic nervous tone during general and epidural anesthesia. METHODS: Thirty-two female patients(ASA grade I-II), scheduled for elective hysterectomy, were randomized to receive epidural anesthesia (group EA, n = 16) or general anesthesia (group GA, n = 16). The changes of HRV were observed with power spectral analysis of heart rate variability (HRV-PSA) at the following time intervals: immediately pre-anesthesia (T1), 15 min after induction (T2), 1 min after incision (T3), dragging uterus (T4), after removing uterus (T5) and end of surgery (T6). RESULTS: Patients in group EA demonstrated a significant advance in LF/HF at T4 and descending in LF and HF at T5, T6 to compare with T1, LF, HF and LF/HF remained unchanged at the rest time intervals. In group GA, compared with T1, LF, HF and LF/HF dropped remarkably except for an increase in LF/HF at T6. CONCLUSION: The autonomic nervous tone is inhibited by both epidural and general anesthesia. EA reveals a shift in the sympathovagal balance toward sympathetic predominance. GA is associated with a shift toward parasympathetic predominance intraoperatively, while postoperatively appears a dramatic increase in LF/HF. Relative to EA, there is a greater effect on autonomic nervous system with GA.

Anesthesia, Epidural↗

Spinal traumas: some postoperative complications in experimental animals.

Animals with severe spinal traumas show paraplegic syndrome and various somatic and autonomic dysfunctions. Of the various dysfunctions those related to hypothermia, bladder problems, and autophagia are of serious nature. The condition of animals with these complications deteriorates rapidly, and the animals are sacrificed for histological and pathological analyses. The findings show that the postoperative complications are related to the degree of severity of the trauma, and that 50-80% animals are lost due to these complications. Most of these animals are lost during the first two weeks after surgery, and the remaining at later stages. Transplantation of neural tissue at the site of lesion does not ameliorate these postoperative complications and improve the survival rate of the animals.

Animals↗

Is lumbar spondylosis a cause of urinary retention in elderly women?

OBJECTIVES: Lumbar spondylosis (LS) is a common spinal degenerative disorder which causes various types of lower urinary tract dysfunction (LUTD). However, it is not certain whether LS may cause urinary retention in elderly women. METHODS: In a period covering the past 3 years, we retrospectively reviewed: a) urodynamic case records of women with urinary retention (post-void residuals, PVR > 100 ml), b) the records of women with LUTD due to LS (cauda equina syndrome and spinal canal narrowing by MRI), and c) uro-neurological features of women who belonged to both a) and b). RESULTS: a) One-hundred women with a mean age of 58 years had urinary retention. The most common underlying disease was multiple system atrophy [19], followed by multiple sclerosis [13] and cervical/thoracic tumours [8]. LS was the fourth most common [5], with the highest age (71 years) of all diseases. b) Nineteen women with LUTD had LS (12, canal narrowing of 50-70%; 7 > 70 %), with a mean PVR volume of 60 ml. A fourth [5] of them had urinary retention, with severe spinal canal narrowing (all 5 > 70%). c) Thus, 5 women belonged to both a) and b). In 4 of these women, LUTD followed or occurred together with typical cauda equina syndrome symptoms such as sciatica and saddle anesthesia. However, one elderly woman presented with painless urinary retention, and absent ankle reflexes were the sole neurological abnormality. The urodynamic abnormalities underlying urinary retention included an underactive detrusor in all 5, bladder sensory impairment in 3, an unrelaxing sphincter in 2, a low compliance detrusor in one, neurogenic sphincter motor unit potentials in 2 of 4 studied, and cholinergic supersensitivity of the detrusor in one of 3 studied. Surgical decompression ameliorated urinary retention in 1 of 2 women who had surgery. CONCLUSIONS: In our series, only 5 percent of the women with urinary retention had LS, but LS poses a potential risk for retention, particularly in elderly women with severe spinal canal narrowing. Preganglionic somato-autonomic dysfunctions underlie this condition. It may appear as the sole initial complaint in cases in which no other obvious neurological abnormalities are found.

Aged↗

ASGE guideline: complications of EUS.

This is one of a series of statements discussing the utilization of GI endoscopy in common clinical situations. The Standards of Practice Committee of the American Society for Gastrointestinal Endoscopy prepared this text. In preparing this guideline, a MEDLINE literature search was performed, and additional references were obtained from the bibliographies of the identified articles and from recommendations of experts. When little or no data exist from well-designed prospective trials, emphasis is given to results from large series and reports from recognized experts. Guidelines for appropriate utilization of endoscopy are based on a critical review of the available data and expert consensus. Further controlled clinical studies are needed to clarify aspects of this statement and revision needed to clarify aspects of this statement and revision may be necessary as new data appear. Clinical consideration may justify a course of action at variance to the recommendations.

Autonomic Nerve Block↗

Robotic devices in surgery.

Robotic devices are defined wich can be used as an aid to surgery. A classification system is proposed that reflects the manner of use and the safety of the systems. Typical benefits and problems of using robots are discussed, and a number of applications are reviewed. These cover "autonomous" systems, that involve no intervention from the surgeon; "hands-on" systems, that require the direct involvement of the surgeon; and "Master/Slave" (or Telemanipulator) systems, that are somewhere between these two and involve some degree of indirect surgeon activity. A number of predictions for the future of medical robotics are provided.

Journal Article↗

Anaesthetic implications of long term diabetic complications.

A case history in which a patient suffering from long-term diabetes mellitus underwent vascular surgery of a lower extremity is presented. Anaesthesia was commenced with an epidural anaesthesia, but due to insufficient analgesia combined with a high thoracal block general anaesthesia was added. The course was complicated because of cardiomyopathy, autonomic neuropathy, difficult laryngoscopy, aspiration of gastric content, and acute renal failure. Anaesthetic implications of long-term diabetes mellitus are discussed.

Acute Kidney Injury↗