Rare and unusual case of anti-factor XI antibodies in patient with plasma cell leukemia.

BACKGROUND: The acquired inhibitors of coagulation have been observed in very rare cases of monoclonal gammopathies. We report a very rare case of anti-factor XI antibodies in patient with plasma cell leukemia (PCL). CASE PRESENTATION: This is a 59-year-old male patient without pathological history, admitted to the nephrology department for management of renal insufficiency and anemia syndrome. The history and physical examination revealed stigmata of hemorrhagic syndrome including hemothorax and hemoptysis. The hemostasis assessment showed an isolated prolonged activated partial thromboplastin time (APTT) with APTT ratio = 2.0.The index of circulating anticoagulant (37.2%) revealed the presence of circulating anticoagulants. The normalized dilute Russell viper venom time ratio of 0.99 has highlighted the absence of lupus anticoagulants. The coagulation factors assay objectified the decrease of the factor XI activity corrected by the addition of the control plasma confirming the presence of anti-factor XI autoantibodies. In addition, the blood count showed bicytopenia with non-regenerative normocytic normochromic anemia and thrombocytopenia. The blood smear demonstrated a plasma cell count of 49% (2842/mm3) evoking PCL. The bone marrow was invaded up to 90% by dystrophic plasma cells. The biochemical assessment suggested downstream renal and electrolyte disturbances from exuberant light chain production with abnormalities including hyperuricemia, hypercalcemia, elevated lactate dehydrogenase, non nephrotic-range proteinuria and high level of C reactive protein. The serum protein electrophoresis showed the presence of a monoclonal peak. The serum immunofixation test detects the presence of monoclonal free lambda light chains. He was treated with velcade, thalidomide and dexamethasone. The patient died after 2 weeks despite treatment. CONCLUSION: Both PCL and anti-factor XI inhibitors are two very rare entities. To the best of our knowledge, this is the first reported case of a factor XI inhibitor arising in the setting of PCL. Factor inhibitors should be suspected in patients whose monoclonal gammopathies are accompanied by bleeding manifestations.

[Pycnodysostosis: about a case]. / Pycnodysostose: à propos d'un cas.

Pycnodysostosis is a very rare genetic disease of the bone characterized by osteocondensation associated with dysmorphic syndrome and growth retardation. This study aims to highlight the phenotypic abnormalities, the radiological signs, the therapeutic and evolutionary features of pycnodysostosis in a 11-year old child. The child was referred by his dentist for clinical evaluation. He was born to first-degree consanguineous parents and had recurrent spontaneous fractures since the age of 3 years. Clinical examination showed dysmorphic syndrome characterized by frontal hump, persistent anterior fontanelle, micrognathia, finger deformities, dental malposition, curved nails, asymmetric chest, lumbar spine scoliosis with severe growth retardation (-4DS). Skeletal X-rays showed bony densification of the skull base, persistent anterior fontanelle, dental malposition, diaphysometaphyseal densification of the long bones mainly at the level of the lower limbs with malunions and tapered phalanges of the hands. Bone densitometry was normal. The diagnosis of pycnodysostosis was retained based on the clinical and radiological signs. Genetic counselling was proposed to the family as well as dental and orthopaedic treatment. Pycnodysostosis is a rare disorder; diagnosis is sometimes difficult and delayed posing diagnostic problem due to its resemblance with osteoporosis. Treatment is essentially based on fractures and dental caries prevention.

Gastrointestinal bleeding due to angiodysplasia in patients on hemodialysis: A single-center study.

Gastrointestinal (GI) bleeding due to angiodysplastic lesions is a common problem among patients receiving hemodialysis (HD). We studied 22 HD patients (5 females and 17 males) who had GI bleeding due to angiodysplasia; the mean age of whom was 54 ± 10 years. All patients had upper and lower GI endoscopy. The most common site for the lesion was the right colon in seven cases (31.8%), followed by stomach in 4 cases (18.1%). In eight (36.3%) patients, there were multiple lesions located in the stomach, duodenum, and the right colon. All patients were treated with coagulation; with argon plasma in 14 (63.6%) patients, bipolar coagulation in five (22.7%) patients, and hot clip in three (13.6%) patients. One patient who presented with persistent bleeding despite endoscopic therapy was well-benefited of a complementary treatment, thalidomide. Hemostasis was obtained in all patients after an average of 6.8 sessions of endoscopic coagulation procedure. We conclude that angiodysplasia is a frequent cause of hemorrhage in chronic renal failure that can be managed in most patients by argon plasma and bipolar coagulation.

Consumption of Argan Oil Improves Anti-Oxidant and Lipid Status in Hemodialysis Patients.

OBJECTIVE: Virgin Argan oil (VAO) is of interest in oxidative stress and lipid profile because of its fat composition and antioxidant compounds. We investigated the effect of VAO consumption on lipid profile and antioxidant status in hemodialysis patients after a 4-week period of consumption. METHODS: In a crossover, controlled trial, 37 patients (18 men, 19 women) with end-stage renal disease on maintenance hemodialysis, were randomly assigned to a 4-week VAO diet. Fasting plasma lipids, vitamin E and oxidized LDL (ox-LDL) were analyzed. Malondialdehyde (MDA) was determined before and after hemodialysis session. RESULTS: There was no significant change in serum total cholesterol and ox-LDL. However, VAO consumption decreased the levels of triglyceride (p = 0.03), total cholesterol (p = 0.02) and low-density lipoprotein (p = 0.03) and increased the levels of high-density lipoprotein (p = 0.01). Plasma vitamin E contents significantly increased from baseline only in VAO-group (p < 0.001). Hemodialysis session increased MDA levels, but the increase in VAO group was less than in control group. CONCLUSION: VAO consumption improved lipid profile and oxidative stress status in hemodialysis patients.

Effects of lowering dialysate calcium concentration on mineral metabolism and hemodynamic parameters in hemodialysis patients.

INTRODUCTION: It has been suggested that a dialysate calcium concentration of 1.5 mmol/L is a compromise between bone protection and cardiovascular risk. This study aimed to investigate the effect of reducing dialysate calcium concentration to 1.5 mmol/L on mineral metabolism and hemodynamic parameters. MATERIALS AND METHODS: Dialysate calcium concentration was changed from 1.75 mmol/L to 1.5 mmol/L for 9 months and observed the effects on mineral metabolism and dialysis outcome parameters in 52 hemodialysis patients. RESULTS: The results at 9 months demonstrated that postdialytic serum calcium level decreased significantly from 109 ± 7 mg/L to 102 ± 6 mg/L, intact parathyroid hormone (PTH) increased from 372 ± 52 pg/mL to 606 ± 80 pg/mL, and the oral alfacalcidol increased from 1.4 ± 0.3 µg/w to 3.3 ± 0.4 µg/w. In patients with low PTH levels, continuous increase of PTH was observed. There were no significant variation in the oral calcium carbonate dose and serum levels of alkaline phosphatase, predialytic calcium, and pre- and postdialytic phosphorus. The ultrafiltration rate and postdialysis systolic blood pressure were significantly lower after reducing the dialysate calcium concentration to 1.5 mmol/L. Intradialytic hypotension and cramps were more frequent with this dialysate calcium concentration. CONCLUSIONS: These findings demonstrated that a decrease in dialysate calcium concentration from 1.75 mmol/L to 1.5 mmol/L improved mineral metabolism by prevention of postdialytic hypercalcemia and releasing oversuppression of PTH, but it was associated with more use of oral alfacalcidol and more hemodynamic impairment.

Angio-embolization of a renal pseudoaneurysm complicating a percutaneous renal biopsy: a case report.

We report the treatment of a bleeding renal pseudoaneurysm by angio-embolization. A 21 years old woman developed macroscopic haematuria following renal biopsy. Renal angio-scan showed a 1.4 cm renal pseudoaneurysm in the left kidney. The presence of pseudoaneurysm was confirmed by selective renal angiography. Successful embolization was performed using gelatine sponge particles.

Experience in using thermal disinfection to remove viable bacteria and endotoxins in centraly distributed reverse osmosis water.

INTRODUCTION: The water used for dilution of hemodialysis concentrates has to meet official quality recommendations regarding microbiology and chemical parameters. To avoid chemical use and to simplify treatments, hot water has been used to control microbial contamination of water distribution systems. In this study we evaluated the efficacy of heat disinfection in maintaining the quality of dialysis water generated by reverse osmosis (RO). METHODS: During the first part of the study, we consecutively used (1) continuous water circulation, (2) daily heat disinfection and (3) a combination of daily heat disinfection and weekly chemical disinfection while checking bacterial count and endotoxin level every 4-5 weeks. During the second part of the study, we continued using daily heat disinfection while checking bacterial count and endotoxin level on weekly basis. RESULTS: The endotoxin levels at all sampling points of the water treatment system were lower than 0.005/ ml throughout the study. The application of heat disinfection alone reduced bacterial levels but an escape phenomenon occurred. After an interval of 21 days, an exponential increase of bacterial count was noted and cultures from the RO unit revealed growth of Pseudomonas fluorescence. The addition of chemical disinfection was successful in eliminating micro-organisms. Throughout this study, micro-organisms and endotoxins were not detectable in dialysate fluid and substitution fluid in dialysis monitors. CONCLUSION: The isolation of a thermo-sensitive organism from the RO unit after a period of relying on thermal disinfection suggests the existence of dead space in the RO unit that is not adequately exposed to heat but is accessible to chemical disinfection. .

Serum tumor markers in hemodialysis patients.

AIMS: The main objective of this work was to evaluate the influence of end-stage renal disease (ESRD) on concentrations of five tumor markers (TMs): carcinoembryonic antigen (CEA), carbohydrate antigen (CA) 19-9 (CA19-9), CA15-3, CA125, and prostate specific antigen (PSA) in a group of chronic hemodialysis patients (CHPs); and to study the influence of hemodialysis (HD) sessions on concentrations of the same TMs. METHODS: We compared TMs levels in CHP before HD sessions to a control group of 50 healthy volunteers, the dosages were determined before and immediately after the HD session Comparisons were made before and after correction for dialysis-induced hemoconcentrations. RESULTS: We enrolled 74 CHPs, all TM concentrations were higher in this group compared to control group, but this increase was significant for CEA (4.25 ± 2.89 vs 2.41 ± 1.81ng/ml; p<0.0001), CA125 (27.84 ± 92.27 vs 13.30 ± 9.85 ng/ml; p = 0.048) and CA19-9 (19.65 ± 25.02 vs 10.23 ± 11.00 U/ml; p = 0.011). Post-dialysis levels were significantly higher than those in pre-dialysis. CEA (3.35 [2,46-5.51] vs 4,06 [2.60-6.78] ng/ml; p<0.0001), CA125 (13.24 [9.66-18.63] vs 16.01 [11.33-22.53] ng/ml; p<0.0001), CA19-9 (12.29 [5.59-21.97] vs 16.29 [7.18-24.7] U/ml; p<0.0001), CA15-3 (13.06 [10.05-17.48] vs 14.58 [11.72-19.35] ng/ml; p<0.0001 and PSA (0.83 [0.5-1.24] vs 1.06 [0.62-1.43] ng/ml; p<0.0001). CONCLUSIONS: Our work confirms that HD increases concentrations of the five TMs evaluated and suggests that the use of CA15-3 and PSA remains valid in CHPs since their concentrations were not altered by ESRD, unlike CEA, CA125, and CA19-9.

[Late pneumomediastinum revealed by acute pulmonary edema in hemodialysis]. / Pneumomédiastin tardif révélé par un œdème aigu du poumon en hémodialyse.

Central venous catheterization occupies an important place in the treatment of end stage renal disease pending the creation of an arteriovenous fistula. However, this procedure is not devoid of complications. We report a case of late pneumomediastinum revealed by an acute pulmonary edema in a young patient on hemodialysis, and we discuss its characteristics.

Scleroderma renal crisis precipitated by steroid treatment in systemic lupus erythematosus and scleroderma overlap syndrome.

INTRODUCTION: Connective tissue disorders can overlap in various ways. Patients may present with features of more than one specific disease without satisfying the diagnostic criteria and thereafter evolve into a specific disease entity. Occasionally, patients may fulfil simultaneously the diagnostic criteria of two or more diseases. Several cases of systemic sclerosis (SSc) and systemic lupus erythematosus (SLE) overlap syndrome have been reported. SLE patients often develop lupus nephritis, the treatment of which is based on immunosuppression with corticosteroids (CS) and cytotoxic drugs. However, the use of high dose of CS has been associated with scleroderma renal crisis (SRC) in patient with SSc. CASE REPORT: a 43-year-old woman presented to the nephrology department of the Military hospital in Rabat, Morocco, in August 2011 with progressive dyspnea and oliguria. She was diagnosed as SLE and scleroderma overlap syndrome based on clinical and serological markers. Renal biopsy showed lupus nephritis. Immunosuppression consisting of high-dose steroid and cyclophosphamide pulses was given. There was response to treatment but 15 days later the course of the disease was complicated by scleroderma renal crisis evidenced by elevated blood pressure, deteriorating kidney function, hemolysis and thrombocytopenia. The patient was treated with perindopril and rapid reduction of steroid doses. This was followed by correction of hemolysis and thrombocytopenia. Two months later, the patient was off dialysis, but had chronic renal insufficiency with an estimated GFR of 25 ml/minute. CONCLUSION: This report describes the occurrence of SRC in a patient with lupus nephritis and SSc/ SLE overlap syndrome who was treated by CS and cyclophosphamide.

A model to calculate cardiac output in hemodialysis patients by thermodilution.

The Blood Temperature Monitor module (BTM) is used to measure recirculation by thermodilution in dialysis. Numerous studies have confirmed its interest in the measuring of the vascular access flow. In this letter we describe a model to calculate cardiac output in dialysis by the BTM.

A model to predict optimal dialysate flow.

Diffusive clearance depends on blood (Qb) and dialysate flow (Qd) rates and the overall mass transfer area coefficient (KoA) of the dialyzer. In this article we describe a model to predict an appropriated AutoFlow (AF) factor (AF factor = Ratio Qd/Qb), that is able to provide adequate Kt/V for hemodialysis patients (HDP), while consuming lower amounts of dialysate, water and energy during the treatment. We studied in vivo the effects of three various Qd on the delivered dose of dialysis in 33 stable HDP. Hemodialysis was performed at Qd of 700 mL/mn, 500 mL/mn, and with AF, whereas specific dialysis prescriptions (treatment time, blood flow rate [Qb], and type and size of dialyzer) were kept constant. The results showed that increasing the dialysate flow rate more than the model of AF predicted had a small effect on the delivered dose of dialysis. The Kt/V (mean ± SD) was 1.52 ± 0.16 at Qd 700, 1.50 ± 0.16 at Qd 500, and 1.49 ± 0.15 with AF. The use of the AF function leads to a significant saving of dialysate fluid. The model predicts the appropriate AF factor that automatically adjusts the dialysate flow rate according to the effective blood flow rate of the patient to achieve an appreciable increase in dialysis dose at the lowest additional cost.

Pregnancy during hemodialysis: a single center experience.

Successful pregnancy outcome is an uncommon occurrence in women requiring chronic dialysis treatment. We reviewed the course and outcome of 9 pregnancies occurred in women on chronic hemodialysis in our center from 1999-2007; 5 of them ended with delivery of alive newborns, 2 with fetal deaths in-utero, and 2 with abortions. The average age of patients was 34 years. The etiology of the original kidney disease was unknown in 44.4% of the cases, and only 22.2% of the patients maintained diuresis. Dialysis started in 8 cases before the diagnosis of pregnancy. The average gestational age at diagnosis was 14 weeks. We modified the prescription of dialysis in 4 patients by increasing the frequency of the dialysis sessions to 6 per week and in 3 by increasing the duration of each session to 6 hours. Anemia was present in all the cases; 3 patients received erythropoietin and 4 patients required transfusion. The pregnancy was com-plicated in 44% of the cases by a polyhydramnios. The average time at delivery was 33 weeks and it was achieved in 80% of pregnancies through vaginal route. The average weight of newborns was to 2380 g. We conclude that pregnancy in women on hemodialysis is possible. The success of pregnancy may be influenced by the residual diuresis and early diagnosis to improve the quality of dialysis by increasing the dialysis dose.