PAPULAR MUCINOSIS

Most patients previously reported to have papular mucinosis had the generalized lichenoid papular form (scleromyxedema) with abnormal gamma globulin. Microscopic examination of affected skin showed increased acid mucopolysaccharides in the dermis and proliferation of fibroblasts. Our patient had the discrete form of papular mucinosis without abnormal serum gamma globulin but with increased amounts of acid mucopolysaccharides in the dermis and no proliferation of fibroblasts.     

MULTIPLE MYELOMA WITH SYNOVIAL AMYLOID DEPOSITION

A 56-year-old man developed multiple myeloma with severe jointstiffness and arthralgia without definite joint swelling. Atpost-mortem examination large amounts of amyloid were foundby light and electron microscopy in the synovial fluid and onthe surface of the synovial membrane. Smaller amounts of amyloidwere identified beneath the lining cells and in vessel walls.No inflammatory reaction was found in the synovial membraneor fluid. Synovial amyloid may occasionally be responsible forjoint stiffness and arthralgia, as well as for more marked jointchanges mimicking rheumatoid arthritis. The diagnosis can beestablished by synovial biopsy. ^*Supported in part by funds from the Veterans Administration,and grants from the Arthritis Foundation, Eastern PennsylvaniaChapter, and Smith, Kline and French Laboratories.     

Cholecystokinin Cholecystography in the Diagnosis of Acalculous Extrahepatic Biliary Tract Disorders

Cholecystokinin cholecystography represents a study designed to identify patients with acalculous e.xtrahepatic biliary tract disorders. In this study, a positive cholecystokinin cliolecystognini (CCK-GB) was defined as both reproduction of the patient's biliary tract-type pain phis one or more of various roentgen abnormalities. Using these criteria, 20 patients luid a positive CCK-GB. After failure of medical management, 19 of these patients came to surgery. Seventeen of 18 available for follow-up were cured of their biliary tract pain by surgery. FoIIow-up of this group of patients has ranged from one montli to 60 months. In view of our findings plus those in other reported series, we conclude that CCK-CB provides a reliable study for the diagnosis of acalculous extrahepatic biliary tract disorders.     

The evaluation of cardiovascular drugs in the anaesthetized,unrestrained rat

A method is reported which allows continuous long-term drug administration and simultaneous blood pressure measurement in the unanaesthetized unrestrained rat. The external jugular vein and abdominal aorta were cannulated and the opposite ends of the cannulae were passed subcutaneously and exteriorized at the back of the head. They were then passed through a spring attached at the lower end to the skull and, at the upper end, to a counter-weighted cantilever. In rats so prepared, infusion of angiotensin amide 200 ng kg^?1 min^?1 caused a rise of blood pressure which lasted the 48 h infusion period. Heart rate decreased initially but recovered within 6 h. Angiotensin amide 30 ng kg^?1 min^?1, infused up to seven days, was without effect on blood pressure or heart rate, and both doses of angiotensin amide failed to alter cardiac catecholamine turnover. Hydralazine, mecamylamine and clonidine reduced blood pressure to 63, 62 and 84% of control respectively while clonidine induced a transient increase before its depressor effect. Heart rate was increased by hydralazine to 138%, and decreased by clonidine to 74% of control, and was unaffected by mecamylamine. The magnitude of pressor response to noradrenaline, tyramine and angiotensin was reduced by hydralazine and increased by mecamylamine. Clonidine increased the pressor response to angiotensin but had no effect on that to noradrenaline or tyramine.     

FIVE YEARS‘ EXPERIENCE WITH PROXIMAL GASTRIC (HIGHLY SELECTIVE) VAGOTOMY

In the five-year period 1972 to 1976 the authors' preferred treatment for patients with chronic duodenal or prepyloric peptic ulcer requiring surgery was proximal gastric vagotomy. In spite of this preference, only two-thirds of such patients were so treated. Most patients with bleeding and stenosis were treated by bilateral truncal vagotomy and drainage, and a few by Polya gastrectomy. Proximal gastric vagotomy proved to be a safe elective operation without mortality and with a proven ulcer recurrence rate so far of 6%. Compared with those who had bilateral truncal vagotomy and drainage, the proximal gastric vagotomy patients complained less often of diarrhoea but more often of weight loss and reflux. Two patients have had persistent postprandial non-peptic pain, thought possibly due to upper gastric ischaemia.     

The Effect of Psychological Intervention on Patients' Long-Term Adjustment to the ICD: A Prospective Study

This study prospectively examined the role of cognitive behavioral therapy (CBT) in (1) alleviating psychological and somatic distress, and (2) lowering arrhythmic events requiring shocks. Forty-nine of 61 consecutive patients were randomized into therapy (CBT, n = 25) or no therapy (NT. n = 24) and completed a battery of self-report questionnaires at baseline and at 9-month follow-up. CBT was administered at preimplant, predischarge, and at seven routine follow-up visits. Patients were 65 ± 10 years old. 65% were men, and 92% Caucasian. Eighteen (72%) CBT patients and 18 (75%) NT patients were retained at follow-up. Compared to CBT patients, NT patients reported higher levels of depression (P = 0.046), more anxiety (P = 0.013), more psychological distress (P = 0.015), poorer overall adjustment (P = 0.009), and poorer sexual functioning (P = 0.003). Mean number of shocks did not differ between the CBT and NT groups (2.85 vs 2.30. respectively); however, more patients in the CBT group (61%) than the NT group (33%) received shocks (P = 0.070). At follow-up, a subgroup analysis revealed that the significant differences observed between the CBT and NT groups were attributable to the patients who received shocks in both groups. In conclusion, CBT was associated with decreased depression, decreased anxiety, and increased adjustment for ICD recipients, particularly among those patients receiving shocks. CBT can be administered effectively at routine follow-up visits or transtelephonically with little added inconvenience to the ICD recipient.     

LAPAROSCOPIC BILATERAL HAND ASSISTED NEPHRECTOMY FOR AUTOSOMAL DOMINANT POLYCYSTIC KIDNEY DISEASE: INITIAL EXPERIENCE

PURPOSE: The laparoscopic technique for bilateral nephrectomy in patients with autosomal dominant polycystic kidney disease is technically difficult. The procedure may be more acceptable if alterations to the technique made it safer and easier to perform. We describe our initial experience with, and the feasibility and potential benefits of hand assisted laparoscopic nephrectomy for approaching these large kidneys in patients with autosomal dominant polycystic kidney disease. MATERIALS AND METHODS: This approach was successfully applied in 3 patients with end stage renal disease due to autosomal dominant polycystic kidney disease. After obtaining transumbilical pneumoperitoneum ports were placed in the umbilicus (12 mm.), sub-xiphoid in the midline (12 mm.) and subcostal in the midclavicular line on each side (12 mm.). The table was tilted 40 degrees away from the planned side of initial nephrectomy with the patient in the half lateral position. A 7 cm. midline incision was made that incorporated the umbilical port and a commercially available hand assistance device was positioned. One surgeon hand was inserted into the abdomen to serve as a retractor/blunt dissector, while the other operated the electrosurgical instruments. The right hand was inserted for left nephrectomy and the left hand was inserted for right nephrectomy. The laparoscope was passed via the sub-xiphoid port and the instruments were placed through the ipsilateral subcostal laparoscopic port. Nephrectomy was completed and the specimen was removed through the hand port incision by draining the cysts as they were exposed to view via the midline incision. When dissection was difficult, an additional port was placed in the anterior axillary line at the umbilical level. Some cysts were ruptured or aspirated to decrease overall kidney size and make extraction possible via the 6 to 7 cm. midline incision. RESULTS: All procedures were successfully completed. Mean operative time for bilateral hand assisted laparoscopic nephrectomy was 5.5 hours (range 4.5 to 6.6). Estimated blood loss was 200 cc or less. Patients resumed oral intake on postoperative day 1. The mean amount of parenteral analgesics required postoperatively was decreased. Mean hospital stay was 4.3 days but it was 3 days when considering nephrectomy only. Patients returned to normal activity after an average of 2 weeks. There was sustained resolution of preoperative discomfort based on pain analog scales. At 1 month or less all patients recorded absent pain. They uniformly noticed improved preoperative pulmonary and gastrointestinal symptoms CONCLUSIONS: Hand assisted laparoscopic nephrectomy in patients with autosomal dominant polycystic kidney disease makes bilateral nephrectomy a reasonable option. The bilateral procedure may be performed as rapidly as laparoscopic only, unilateral nephrectomy in these cases. The advantages of the hand assisted approach include using tactile sensation to facilitate dissection, rapid blunt finger dissection, hand retraction and the application of immediate tamponade when needed. This procedure provides the benefits of minimal intraoperative blood loss, minimal postoperative pain, brief hospital stay and rapid convalescence in this group of patients at high risk.     

THE VARIETIES OF SELECTIONAL EXPERIENCE IN BEHAVIORAL GENETICS

“There appears to be no character-morphogenetic, behavioral, physiological, or cytological-that cannot be selected in Drosophila.” R. Lewontin (1974)