Subhash Popli

Demonstration of Fabry's disease deposits in placenta

Involvement of the placenta by glycosphingolipid deposits in Fabrys disease has not been previously reported. We describe the presence of such deposits in the maternal half of a placenta obtained from a heterozygous carrier of the disease.

Contaminated product water as the source of Phialemonium curvatum bloodstream infection among patients undergoing hemodialysis.

OBJECTIVE We investigated a cluster of cases of bloodstream infection (BSI) due to the mold Phialemonium at a hemodialysis center in Illinois and conducted a cohort study to identify risk factors. DESIGN Environmental assessment and cohort study. SETTING A hemodialysis center in a tertiary care hospital. METHODS A case patient was defined as a person who underwent dialysis at the center and had a blood sample that tested positive for Phialemonium curvatum on culture. We reviewed microbiology and medical records and tested water, surface, and dialysate samples by culture. Molds isolated from environmental and clinical specimens were identified by their morphological features and confirmed by sequencing DNA. RESULTS We identified 2 case patients with BSI due to P. curvatum. Both became febrile and hypotensive while undergoing dialysis on the same machine at the same treatment station, although on different days. Dialysis machines were equipped with waste handling option ports that are used to discard dialyzer priming fluid. We isolated P. curvatum from the product water (ie, water used for dialysis purposes) at 2 of 19 treatment stations, one of which was the implicated station. CONCLUSION The source of P. curvatum was likely the water distribution system. To our knowledge, this is the first report of patients acquiring a mold BSI from contaminated product water. The route of exposure in these cases of BSI due to P. curvatum may be related to the malfunction and improper maintenance of the waste handling option ports. Waste handling option ports have been previously implicated as the source of bacterial BSI due to the backflow of waste fluid into a patients blood line. No additional cases of infection were noted after remediation of the water distribution system and after discontinuing use of waste handling option ports at the facility.

Bicarbonate-Buffered Peritoneal Dialysis

Although relatively difficult to prepare and store, sterile peritoneal dialysis solutions containing bicarbonate, calcium and magnesium may at times be needed (1-10), For example, one may have to dialyze a uremic patient who has developed hypotension and lactic acidosis, In such a patient, the lactate or acetate in conventional dialysates may not be metabolized to bicarbonate because of tissue hypoxia, and acidosis may be further worsened because bicarbonate is removed during dialysis (11-14). Using bicarbonate-containing dialysate in such a patient will add bicarbonate to the blood and also remove lactate (12-14). For these reasons, some authors have recommended bicarbonate-buffered peritoneal dialysis as adjunctive treatment for severe lactic acidosis (13). Another potential application may be for patients who experience abdominal pain or discomfort when conventional acetateor lactate-buffered peritoneal dialysates are infused. The abdominal pain may be related to the unphysiologically high acidity [e.g., with a pH as low as 5.2 (15)] of acetateand lactate-buffered dialysates (16). Conceivably, bicarbonate-buffered peritoneal dialysate, with its more physiologic pH, might decrease the incidence of such symptoms. Since the basic principles for preparing bicarbonatebuffered dialysate for peritoneal dialysis are the same as those for hemodialysis, a brief review of methods to prepare bicarbonate-buffered hemodialysate is in order.

Paecilomyces peritonitis: case report and review of the literature

While filamentous fungi are a rare cause of peritonitis in peritoneal dialysis patients, there is increasing recognition of Paecilomyces species as pathogens in such patients. We herein report a case of fungal peritonitis secondary to the filamentous Paecilomyces variotii species. The patient had a long and ultimately fatal course of illness despite catheter removal, discontinuation of peritoneal dialysis, recurrent intraabdominal abscess drainage, and prolonged courses of antifungal therapy. Our experience with this case and a review of the literature suggests that infection with this fungus can cause substantial morbidity and is probably best treated with prompt catheter removal, aggressive antifungal therapy and vigilant observation for complications.

Induction of peritoneal fluid eosinophilia and/or monocytosis by intraperitoneal air injection

We hypothesized that intraperitoneal air might be one of the causes of peritoneal fluid eosinophilia. To test our hypothesis, we injected 100-500 ml of sterile air intraperitoneally into 5 patients receiving continuous ambulatory peritoneal dialysis (CAPD). All patients responded with a transient increase in peritoneal fluid nonerythrocyte cell count (peak counts ranging from 23 to 335 cells/mm3, mean peak count 140 +/- 125) lasting 4 days (after injection of 100 ml of air) to 7 weeks (after injection of 500 ml of air). In 2 patients, the cells were predominantly monocytes (80 +/- 6.5%), whereas in 3 patients, eosinophils predominated (63 +/- 12%), while monocytes (30 +/- 19%) also increased. Resolution of peritoneal fluid pleocytosis correlated temporally with absorption of subdiaphragmatic air. Our results suggest that intraperitoneal introduction of air into CAPD patients can induce peritoneal fluid eosinophilia and/or monocytosis.

Osmotic diuresis-induced hypernatremia: better explained by solute-free water clearance or electrolyte-free water clearance?

Hypernatremia may result from inadequate water intake, excessive water loss or a combination of the two. Osmotic diuresis leads to losses of both solute and water. The relationship between solute and water losses determines the resulting changes in serum osmolality and sodium concentration. Total solute loss is routinely higher than loss of water in osmotic diuresis. Theoretically, then, decreases in serum osmolality (and serum sodium concentration) should follow. In clinical situations of osmotic diuresis, however, reduction in osmolality can take place, but not reduction in serum sodium concentration. It is of note that serum sodium concentration changes are related to urinary losses of sodium and potassium but not to the loss of total solute. In osmotic diuresis, the combined loss of sodium and potassium per liter of urine is lower than the concurrent serum sodium level. Consequently, hypernatremia can ensue. A patient who presented with osmotic diuresis and hypernatremia is described here. In this patient, we have shown that electrolyte-free water clearance is a better index of the effect of osmotic diuresis on serum sodium concentration than the classic solute-free water clearance.

Carpal tunnel syndrome and chronic hemodialysis.

Carpal Tunnel Syndrome and Chronic Hemodialysis Dear Sir, Referring to the letter by Walts et al. [1] published in your journal, our work is at odds with theirs. In our clinic we have monitored 176 patients in a program of chronic hemodialysis for periods ranging from 3 to 144 months (x = 67.6). Eight patients (3 male and 5 female) manifested carpal tunnel syndrome (CTS); their ages ranged from 40 to 81 years (x = 61.1). The duration of dialysis treatment was 91.5 months (range 72–144). These results contrast with the 176 months (14.7 years) that the said authors [1] speak of. Our work coincides with previously reviewed literature [2–5]. In 5 of these cases the syndrome was bilateral and in 3 unilateral (a total of 13 occurrences). In each case it was the arm with a functioning arteriovenous fistula which was affected; given that this was the sole vascular access no further surgical intervention was required. We pursued 5 anatomopathological studies with Congo red and thioflavine T manifesting a total of 9 interventions (8 patients). In only 1 case did we find deposits of amyloid. None of the affected patients presented indices of systemic amyloidosis. In the development of CTS, the patients in a program of chronic hemodialysis showed a marked preference for the arm carrying the vascular access despite the fact that neither vascular alterations nor inflammatory changes were noted in the anatomopathological studies effected. We observed no statistically significant relation with any special type of nephropathy such as presented by our patients: hyperuricemic nephropathy (2 cases), glomeru-lonephritis (2 cases), nephroangiosclerosis (1 case, showing the only incidence of amyloid deposits), tubular aci-dosis (1 case) and undefined etiologies (2 cases). Nor was any relation observed with the dialytic procedure, hypervolemia, or the various biochemical parameters studied (urea, creatinine, phosphocalcic metabolism, PTH and hematocrit count). According to our experience, CTS is a complication of periodic hemodialysis that does not show an elevated incidence of amyloid deposits, and in general appears after the sixth year of substitutive treatment. References Walts, A.E.; Goodman, M.D.; Matorin, P.A.: Amyloid, carpal tunnel syndrome, and chronic hemodialysis. Am. J. Nephrol. 5: 225–226 (1985). Halter, S.K.; DeLisa, J.A.; Stolov, W.C; Scardapane, D.; Sher-rard, D.J.: Carpal tunnel syndrome in chronic renal dialysis patients. Archs phys. Med. Rehabil. 62: 197–201 (1981). Schwarz, A.; Keller, F.; Seyfert, S.; Poll, W.; Molzahn, M.; Dist-ler, A.: Carpal tunnel syndrome: a major complication in long-term hemodialysis patients. Clin. Nephrol. 22: 133–137 (1984). Kachel, H.G.; Altmeyer, P.; Baldamus, C.A.; Koch, K.M.: Deposition of an amyloid-like substance as a possible complication of regular dialysis treatment. Contr. Nephrol. vol. 36, pp. 127–132 (Karger, Basel 1983).

B-cell directed antibodies and delayed hyperacute rejection: a case report.

Transplantation in the presence of a conventional positive lymphocytotoxic crossmatch has been contraindicated for a number of years [3, lo]. With modification of this assay so that cytotoxicity to the T or B cell can be determined, a number of groups have been undertaking renal transplantation in the presence of presensitization to the B cell, with conflicting results [4, 8, 91. Delayed hyperacute rejection in the presence of such a B-cell directed alloantibody forms the basis of this article.

Pyocystis in a renal transplant recipient with a defunctionalized bladder.

Fever occurred in a man 6 weeks after renal transplantation. At the time of transplantation, the donor ureter had been anastomosed to a ureteroileal conduit created 6 years previously because of traumatic neurogenic bladder. Initial evaluation failed to reveal the cause of the fever, but ultimately, drainage of the defunctionalized bladder yielded a large amount of pus infected with Klebsiella pneumoniae. Our patients course suggests that, when fever develops after renal transplantation in patients with previous urinary diversion, pyocystis should be included in the differential diagnosis.

Gustatory Sweating in a Diabetic End-Stage Renal Disease Patient Maintained on Hemodialysis

David J. Leehey, MD, Veterans Affairs Hospital, Hines, 111 60141 (USA) Dear Sir, Gustatory sweating is an unusual manifestation of abnormal nerve regeneration as a consequence of autonomic neuropathy that was recognized as early as the 18th century by Claude Bernard [1]. It has since been reported in patients suffering from Frey’s syndrome (auriculotemporal syndrome), most commonly after head and neck surgery [2]. Gustatory sweating may also occur in association with tic douloureux, cerebellopontine angle meningioma, herpes zoster, metastatic carcinoma, and diabetes mellitus [3-5]. We herein report a diabetic hemodialysis patient suffering from this sweating abnormality. Consideration of this entity in the diabetic patient population is necessary in order to avoid misdiagnosing the manifestation as being due to hypoglycemia, hypotension, or other causes. A 52-year-old white male with a 30-year history of insulin-dependent diabetes mellitus and diabetic end-stage renal disease had been maintained on hemodialysis for 3 years. The patient’s diabetic condition has been complicated in the past by retinopathy, ente-ropathy, peripheral neuropathy, and right oph-thalmoplegia. He was noted to have occasional episodes of facial and upper body sweating during dialysis. These symptoms were initially thought to be secondary to dialysis-related hypotensive episodes or hypoglycemia. However, blood pressure and plasma glucose levels were consistently within normal limits. Further investigations revealed recurrent symptoms of profuse sweating while eating for the previous 6 months. Sweating began soon after chewing food, was independent of the quality or quantity of food ingested, and was distributed symmetrically. It started in the forehead and spread to face, scalp, neck, shoulders, and upper parts of the body and was associated with excessive salivation. The patient had lost 7 kg due to his reluctance to eat in an attempt to avoid episodes of what he described as ‘trying to eat in a shower’. Physical examination revealed significant orthostatic hypotension, proliferative retinopathy, right peripheral 6th nerve palsy, sensory deficits in legs and feet, and absent ankle jerk reflexes. Abnormal laboratory findings included a plasma glucose level of 314 mg/dl and a blood hemoglobin A1C value of 12.3 g/ dl. Therapy with clonidine at a dosage of 0.1 mg three times a day resulted in a partial relief of the symptoms.