Hereditary late-onset lymphedema with pleural effusion and laryngeal edema.

We examined two middle-aged male cousins with unexplained edema of postpubertal onset involving the upper and lower limbs, face, and larynx and, in one of them, a persistent pleural effusion. Scintilymphangiography detected an apparent paucity or absence of lymph nodes in the axillae and above the inguinal ligaments, indicating a defect in the lymphatic systems. Laryngeal edema, confirmed endoscopically, produced changes in one of them in the flow volume loop characteristic of a variable extrathoracic obstruction. A family study showed autosomal dominant transmission of the disorder. The nosology of late-onset lymphedema is briefly discussed, with particular reference to the so-called yellow nail syndrome.

Management of post-traumatic acute renal failure with peritoneal dialysis.

To evaluate the efficacy of peritoneal dialysis (PD) in the management of post-traumatic renal failure, the authors reviewed the courses of five critically injured patients treated with PD over an 18-month period. Each patient had a double-cuffed PD catheter inserted through a subcutaneous tunnel with PD initiated within 48 hours. The dialysis prescription was individualized for each patient with frequent exchanges performed using either a manual manifold system or a continuous cycling machine. Three of the five patients survived and none of the survivors required dialytic therapy at discharge. Duration of PD ranged from 10 to 57 days. Three patients required intermittent hemodialysis (HD) due to progressive azotemia and hyperkalemia. Two patients developed bacterial peritonitis and three patients developed hyperglycemia with PD continuing without interruption in each patient. When compared to HD, PD offers the advantages of better hemodynamic tolerance, no anticoagulation, no vascular access, and a reduced personnel requirement if continuous cyclic PD is used.

Right atrial thrombus formation following central venous dialysis catheter placement.

Silicone rubber central venous dialysis catheters are used to provide vascular access in acute renal failure patients and in chronic dialysis patients in whom conventional vascular access cannot be achieved or maintained. The use of these catheters is not without hazard. The first reported case of right atrial thrombus formation associated with the use of a double lumen silicone rubber central hemodialysis catheter is described. This patient's course points out another potential complication of this type of vascular access and emphasizes the importance of removing such catheters in a timely fashion when they are no longer required for dialysis.

Tear flow in cyclosporine recipients.

PURPOSE: To measure tear flow in renal allograft recipients who received oral cyclosporine as immunosuppressive therapy. PATIENTS: Control subjects were from the staff of various clinics, dialysis patients were recruited, and one group of kidney transplant recipients took oral cyclosporine, whereas another group took azathioprine. Written informed consent was obtained for an institutionally approved protocol. METHODS: A 4-minute Schirmer test with topical anesthetic was performed in both eyes of each person, and the average score was used in calculations. RESULTS: The tear flow rate was 10.6 mm/4 minutes in the control subjects, 13.6 mm/4 minutes in the dialysis patients, 10.4 mm/4 minutes in kidney transplant recipients taking azathioprine, and 19.0 mm/4 minutes in kidney transplant recipients taking cyclosporine. Kidney transplant recipients taking cyclosporine had significantly increased tear flow compared with the other three groups (P < 0.05); dialysis patients, kidney transplant recipients taking azathioprine, and control subjects showed statistically indistinguishable tear flow. Within-group comparisons (male versus female, black versus white, and all combinations) showed no statistically significant differences. CONCLUSION: Medications used by dialysis patients or the azathioprine group did not significantly increase tear flow compared with control subjects. The addition of cyclosporine (plus prednisone) enhanced tear flow in kidney transplant recipients compared with all other groups. Systemic cyclosporine appears to increase tear flow even when no lacrimal autoimmune disease exists.

Longitudinal tear study after cyclosporine in kidney transplant recipients.

PURPOSE: To determine Schirmer wetting (tear flow) in patients before and after kidney transplantation where systemic cyclosporine was used as an immunosuppressive. METHODS: Patients with end-stage renal disease who received living donor or cadaveric kidney transplants and simultaneous systemic cyclosporine were recruited. A 4-minute Schirmer test with topical anesthetic was performed in both eyes of each person before cyclosporine was initiated and three times after transplantation. The tear flow values from both eyes were averaged before analysis. RESULTS: Tear flow increased significantly from precyclosporine levels of 19.4 +/- 1.5 mm/4 minutes (mean +/- standard error of the mean, n = 7; readings at 1 to 3 days before cyclosporine was begun) to 28.4 +/- 2.4 mm/4 minutes at 1 to 2 months after cyclosporine (P < 0.01), 26.4 +/- 2.0 mm/4 minutes at 3 to 5 months (P < 0.05), and 24.4 +/- 2.1 mm/4 minutes at 9 to 18 months (P < 0.05) after initiation of cyclosporine therapy. No relation existed between tear flow and systemic cyclosporine concentration. CONCLUSION: Tear flow was significantly enhanced by systemic cyclosporine when given to renal allograft recipients as an immunosuppressive. The increased flow rate was sustained over the maximum follow-up of 18 months and indicates an unexpected, beneficial side effect of cyclosporine, even in the absence of a deficit in baseline tear production.

Hereditary central diabetes insipidus: plasma levels of antidiuretic hormone in a family with a possible osmoreceptor defect.

A large Canadian kindred of Irish extraction extending from Quebec to British Columbia with autosomal dominant diabetes insipidus responsive to exogenous antidiuretic hormone (ADH) is described. Out of 121 individuals 34 have been identified as affected in seven generations. The disorder is characterized by variability in age at onset and in severity, and by apparently spontaneous abatement in old age. The affected subjects do not appear to manifest hypertension or its sequelae. In three individuals tested the plasma ADH level was very low in spite of adequate osmotic stimulation. However, the level rose in two of them when they were given furosemide, which suggests an osmoreceptor defect and a normal ADH response to volume change.

Hypertyraminemia in cirrhotic patients.

To evaluate the role of tyramine in hepatic disorders, we used a radioimmunoassay to study plasma concentration of tyramine in eight healthy subjects, 20 hospitalized patients without liver disease, and 13 cirrhotic patients of whom seven had hepatic encephalopathy. The effect of increasing dietary protein on tyramine level of cirrhotic patients was also assessed. No significant difference in plasma tyramine concentration was seen between normal subjects, 1.3 +/- 0.1 ng per milliliter (average +/- S.E.), hospitalized patients without hepatic disease (1.4 +/- 0.1 ng per milliliter) and cirrhotic patients without encephalopathy (2.7 +/- 0.5 ng per milliliter). However, the tyramine level in cirrhotic patients with encephalopathy, 6.4 +/- 0.1 ng per milliliter, was significantly (P less than 0.001) higher than in normal subjects or in cirrhotic patients without encephalopathy. Increasing dietary protein from 40 to 80 g per day raised fasting tyramine concentration by 30 to 70 per cent within three days in both encephalopathic and non-encephalopathic cirrhotic patients. Concentration of plasma tyramine in cirrhotic subjects was significantly correlated with that of plasma tyrosine (P less than 0.001).

Transmission of human immunodeficiency virus (HIV) by transplantation: clinical aspects and time course analysis of viral antigenemia and antibody production.

The human immunodeficiency virus (HIV) was transmitted to a patient who received a cadaveric renal transplant from a donor who had received massive blood component replacement. A negative HIV antibody test was obtained on serum drawn immediately after transfusion. After transplantation, pretransfusion sera and sera obtained several hours after transfusion tested positive for HIV antibody, suggesting that transfusions had transiently diluted the patient's serum and resulted in a false-negative HIV antibody test. Immediately after transplantation, the recipient showed a transient increase in HIV antigen levels followed by a more sustained increase representing de-novo antigen synthesis. Antibodies to HIV were detected 51 days after transplant. The recipient has shown no signs or symptoms of HIV infection after 1 year. In potential cadaveric organ donors, HIV antibody testing should be performed on pretransfusion sera or on sera obtained several hours after massive transfusion of blood products.