Outcome of Kidney Transplant in Patients with Polycystic Kidney Disease: A Single-Center Study in Tunisia.

OBJECTIVES: Autosomal dominant polycystic kidney disease is a common cause of end-stage renal disease and a common indication for renal transplant. This study was undertaken to evaluate the demographics, outcomes, and complications of renal transplant in patients with autosomal dominant polycystic kidney disease compared with other nephropathies. MATERIALS AND METHODS: In a retrospective case-control design, we reviewed the records of 7 patients with autosomal dominant polycystic kidney disease from a total of 701 renal transplant patients over a 30-year period (1986-2016). For each patient, a matched control was selected based on sex, age, year of transplant, and type of kidney donor. We excluded patients who underwent kidney transplant abroad and those with a follow-up period of less than 2 years. RESULTS: The number of patients with autosomal dominant polycystic kidney disease requiring transplant at our center was estimated at 0.23 per year, and the condition represented 1.57% of initial nephropathy causes. The mean patient age at transplant was 50.8 ± 8.05 years. There were 5 male and 2 female patients in the case group, with a male-to-female ratio of 2.5. The source of the graft was predominantly a living related donor (5/7). Four patients had extrarenal manifestations, the most common of which were liver cysts (3 patients). Rejection occurred in a single study patient (14.2%) and in 4 control patients (57.1%; P = .51). Two patients did not develop any complications. Complications noted after transplant included infection (3/7 cases vs 2/7 controls; P= .67) and cerebrovascular accidents (2/7 cases vs 0/7 controls). CONCLUSIONS: Further studies with longer follow-up and greater numbers of patients are needed to compare more precisely the complications and results of transplant between patients with autosomal dominant polycystic kidney disease and other kidney transplant recipients.

Therapeutic efficacy of a biosimilar epoetin alfa in hemodialysis patients.

Anemia is a frequent complication in patients with chronic kidney disease. However, human recombinant erythropoietin (rHu-EPO) has revolutionized the management of anemia in chronically dialyzed patients. Epomax ® is a new rHu-EPO alfa manufactured in Tunisia (Medis Laboratories). The aim of this study was to evaluate the efficacy and tolerance of Epomax ® in chronic hemodialysis (HD) patients in a phase-III, multicenter, clinical trial. Fiftythree HD patients (mean age 47.7 ± 13 years) who received a stable dose of rHu-EPO (Hemax ® , a rHu-EPO alfa manufactured by Biosidus Laboratories) subcutaneously were switched to Epomax ® via the same route of administration. At baseline, the mean systolic pressure was 132 ± 18 mm Hg and the mean diastolic pressure was 79 ± 8 mm Hg. The mean blood hemoglobin was 10.2 g/dL and the median ferritin level was 667 ng/mL. After a follow-up of 43 days, the mean blood hemoglobin was 10.5 g/dL under the effect of Epomax ® . There was no significant difference in the mean hemoglobin levels between the treatments with both drugs. Few adverse events were reported during the study. We conclude that Epomax ® was effective at maintaining the hemoglobin levels at target concentrations and was well tolerated in HD patients.

Acute renal failure by ingestion of Euphorbia paralias.

Euphorbia paralias is known in traditional medicine as an anti-inflammatory agent, a purgative and for its local anesthetic property. To the best our knowledge, renal toxicity of this substance has not been previously reported. In this paper, we report the case of a 29-year-old male who developed renal damage following ingestion of Euphorbia paralias. He had been on follow-up for nephrotic syndrome since 1986, although irregularly, with several relapses but each responding well to steroid therapy. A kidney biopsy had not been performed earlier due to refusal by the patient. He was off steroids since April 2008 because the patient developed osteoporosis. He was admitted with general malaise and oliguria to our department in May 2009, following repeated vomiting and watery diarrhea for three days. On examination, he was edematous but had normal vital signs except for a pulse rate of 120/min. Hemoglobin was only 5.5 g/dL but with normal white cell and platelet counts. Blood biochemistry showed evidence of advanced renal failure with a serum creatinine level of 1835 µmol/L and urea at 44.6 mmol/L, sodium of 132 µmol/L and potassium at 4.3 mmol/L. He had features of nephrotic syndrome with severe hypoproteinamia and 24-h urinary protein of 10.45 g. Ultrasonography revealed enlarged kidneys with a reduced echogenecity of the medulla and the papillae. Subsequently, after hemodialysis with blood transfusion, a kidney biopsy was performed that showed focal segmental glomerulosclerosis associated with an acute tubular injury. On intensive interrogation, the patient gave a history of ingesting boiled Euphorbia paralias as a native treatment for edema, ten days prior to the onset of the current illness. A diagnosis of acute renal failure (ARF) resulting from the possible nephrotoxic effect of Euphorbia paralias poisoning was made. He was treated with intermittent hemodialysis and corticosteroids. Serum creatinine values improved after 48 days. At six months following the intoxication, serum creatinine of the patient was 240 µmol/L. In cases of unexplained ARF, a toxic mechanism should always be considered and acute renal failure caused by Euphorbia paralias should be included as a cause if renal toxicity is suspected in those places where it is being used as a native medicine.

Short- and long-term outcomes of kidney donors: a report from Tunisia.

Kidney transplantation remains the best treatment option of end-stage renal disease. Kidney donations are of particular interest with the currently increasing practice of living-donor transplantation. The purpose of this study was to analyze retrospectively the general health status as well as renal and cardiovascular consequences of living-related kidney donation. A total of 549 living-related kidney donors had donated their kidneys between 1986 and 2007. We attempted to contact all donors to determine short- and long-term outcome following kidney donation. All kidney donors who responded underwent detailed clinical and biochemical evaluation. The data were compared with age-matched health tables of the Tunisian general population. In all, 284 donors (52%) had a complete evaluation. They included 117 men and 167 women with a mean age of 42 ± 12 years. The major peri-operative complications that occurred in these donors included four cases of pneumothorax, six cases of surgical site infection, one case of phlebitis and one case of pulmonary embolism. None of the study cases died. The median length of hospital stay after donor nephrectomy was 6.5 days (range: 3-28 days). The median follow-up period was eight years. The mean creatinine clearance after donation was 90.4 ± 25 mL/min in men and 81.5 ± 27.2 mL/min in women. Proteinuria was >300 mg/24 h in 17 cases (5.9%). Fifty-eight (20.4%) donors became hypertensive and 19.6% of the men and 37.2% of the women became obese. Diabetes mellitus developed in 24 (8.4%), and was more common in patients who had significant weight gain. Our study suggests that kidney donors have minimal adverse effects on overall health status. Regular follow-up identifies at-risk populations and potentially modifiable factors. Creation of a national registry of living donors and their monitoring are an absolute necessity.

[Calciphylaxis in chronic renal failure: 3 cases]. / Calciphylaxie au cours de l'insuffisance rénale chronique: etude de 3 cas.

BACKGROUND: Calciphylaxis is a small vessel disease responsible for vascular calcification and skin necrosis. It occurs in association with chronic renal failure and has a poor prognosis. BUT: Report new cases. We report 3 cases of calciphylaxis occurred in patients with chronic renal failure secondary to interstitial nephritis in 1 case, diabetic nephropathy in 1 case and thrombotic microangiopathy in 1 case. CASES: They were 2 females an 1 man aged of 44, 3 years meanly. Hyperphosphoremia and hyperparathyroidism were the essential risk factors. All patients died by sepsis. This course was precipitating by corticotherapy in 2 cases. CONCLUSION: Early recognition and treatment of risk factors is mandatory to reduce mortality in uremic patients with calciphylaxis.

[A rare case of Graves' disease in a patient on regular haemodialysis]. / Un cas rare de maladie de Basedow chez un patient hémodialysé chronique.

INTRODUCTION AND AIMS: Abnormal thyroid hormone production and metabolism are relatively common in chronic renal failure and in regular haemodialysis. Graves' disease is a very unusual condition and is difficult to identify. We report a case of Graves' disease in a patient on regular hemodialysis. CASE REPORT: A 26-year-old man undergoing regular hemodialysis from unknown chronic nephropathy since four years. The patient suffered from unexplained slimming and aggressiveness. On admission, he had an irritability, tremor of the extremities, arterial hypertension not controlled by the medical treatment, discrete asynergy oculo-palpebral, glare of the glance and protrusion of the ocular spheres. The thyroid was non palpable. Biological tests demonstrated hyperthyroidism. Thyroid-stimulating hormone receptor antibodies were positive. Thyroid echography and scintiscanning showed vascular and hyperfonctionnel character of the thyroid. The patient was treated radically by radioactive iodine 131 therapy after medical preparation by antithyroid agent. He developed hypothyroidism treated by substitutive treatment. CONCLUSION: The diagnosis of Graves' disease must be evoked even in the absence of specific symptoms in haemodialysis patients. In front of clinical symptoms, since the conventional treatment is effective and inoffensive.

[Colic angiodysplasia and chronic haemodialysis. Argon plasma treatment. A case report]. / Angiodysplasie colique et hémodialyse chronique. Traitement par coagulation au plasma argon. A propos d'un cas.

INTRODUCTION: Gastrointestinal angiodysplasia is a very common cause of digestive haemorrhage among patients with chronic renal insufficiency. It is well known that bleeding from angiodysplasias can be a difficult therapeutic problem since therapeutic possibilities are scarce and surgery is scolded with high mortality rate. Endoscopic argon plasma ablation therapy is a new, effective and safe treatment in the management of gastrointestinal angiodysplasia. CASE REPORT: We reported a case of a female haemodialysis patient aged 40 years. She was haemodialysed since 6 years in our center from unknown nephropathy. In March 2006, she complained of black stools and melena, and developed severe anaemia (Haemoglobin at 4 g/dL). Exploratory endoscope examination of the digestive tract showed the presence of bleeding from angiodysplastic lesions of the right colon. The patient had severe clinical picture extension of angiodysplastic lesions and frequent bleeding episodes. Bleeding arrest was observed after argon plasma coagulation, with a significant decrease of blood transfusions and improvement of anaemia. CONCLUSION: Gastrointestinal angiodysplasia was reported to be an important complication in dialysis patients and was recognized as an important cause of erythropoietin-resistant anaemia. It can worsen vital prognosis. Argon plasma coagulation is an effective treatment in these patients.

Bleeding and thrombosis in a patient with secondary antiphospholipid syndrome.

Antiphospholipid antibodies have been associated with occurrence of arterial and venous thrombotic events and fetal loss, which together constitute the antiphospholipid syndrome (APS). However, bleeding is rare in this syndrome. We report a case of systemic lupus erythematosus (SLE) with APS complicated simultaneously by thrombotic and hemorrhagic events. A 34-year-old woman was a known case of diffuse proliferative lupus nephritis associated with APS, on treatment with corticosteroids, cyclophosphamide and anticoagulants. She presented in February 2004 with severe anemia, menorrhagia, gingival bleeding and acute loss of vision in the left eye. Investigations revealed a hematoma in the psoas muscle with thrombosis of the inferior vena cava and occlusion of the retinal vein. Blood tests revealed a strongly positive lupus anticoagulant, factor XI deficiency (35%) and decrease of free protein S (44%). Factor XI inhibitor, anti-prothrombin, and anti-protein S antibodies were absent. The patient was treated with corticosteroids and six pulses of cyclophosphamide, which resulted in a rapid disappearance of bleeding, reduction of hematoma and normalization of hematological abnormalities. She was maintained on corticosteroids, azathioprine and anticoagulant agents were introduced. After a follow-up of 28 months, there was no recurrence of bleeding, the thrombosis had resolved, and there was a decrease in the levels of circulating anticoagulant as well as anticardiolipin antibodies.

[Interest of bone alkaline phosphatase in hemodialysis patients]. / Interet de la phosphatase alcaline osseuse chez les hemodialyses chroniques.

BACKGROUND: The renal osteodystrophy represent a major complication in hemodialysis. AIM: To evaluate the value of plasma bone-alkaline phosphatase (bAP) in the diagnosis of the type of renal osteodystrophy among hemodialysis patients and to seek a possible correlation between the bAP, total alkaline phosphatases (tAP) and the intact parathormone (iPTH). METHODS: We studied 67 chronic hemodialysis patients. Plasma bAP was determined by immunoenzymatic technic. iPTH (1-84) was measured by electrochimiluminescence. RESULTS: We found that bAP levels were normal (10-20 ng/ml) in 17 patients, low (< 10 ng/ml) in 4 and high (> 20 ng/ml) in the 46 other patients. There is a good positive correlation between the plasmatic rate of bAP and the following parameters: the period of dialysis (R = 0.316, p = 0.009), plasmatic rate of tAP (r = 0.781, p < 10(-3)) and the rate of iPTH (r = 0.650, p < 10(-3)). There is a good positive correlation between the plasmatic rate of bAP and the rates of the tAP and of iPTH, the correlation between bAP and the iPTH being more significant. A rate of bAP higher than 20 ng/ml had a sensitivity of 93.5%, specificity of 63.3% in favour of a rate of iPTH >400 pg/ml and consequently of the biological diagnosis of hyperparathyroidism. In addition, 4 patients have a bAP <10 ng/ml with iPTH < 150 pg/ml evoking an adynamic osteopathy. CONCLUSION: plasma bAP provides useful information about bone remodelling in hemodialysis patients.

[Vascular trombosis of renal graft: 9 cases]. / Thromboses vasculaires du greffon apres transplantation renale: a propos de 9 cas.

BACKGROUND: Allograft renal thrombosis can occur in 1 to 6% of cases. Many predisposing factors has been identified especially alteration of coagulation. AIM: We analyzed in this study frequency and predisposing factors of renal graft thrombosis. METHODS: We report a retrospective study including 319 renal transplant recipients. RESULTS: Nine patients (2.8%) presented veinous graft thrombosis in 5 cases and arterial thombosis in 4 cases. There were 6 men and 3 women aged of 30.6 years meanly (10-56) which developed the thrombosis 6 days (1-48) after the transplantation. All patients were detransplanted after 16.2 days and 1 patient died. CONCLUSION: Thrombosis constitute an important cause of graft loss. A perfect surgical technic and prophylactic treatment in high risk patients are necessary to reduce this complication.

[Acute renal failure in a prisoner after hunger strike]. / Insuffisance renale aigue survenue chez un detenu suite a une greve de la faim.

BACKGROUND: Acute renal failure may occur in varied circumstances. It is potentially reversible spontaneously or after specific treatment. It is rare after hunger strike and fewer cases were reported in the literature. The physiopathological mechanisms are varied and remain incompletely known. AIM: We report the case of a prisoner having presented an acute renal failure after a hunger strike wich was completely reversible. CASE: He's a 29 year old man, without a past medical facts, in July 2004 he was incarcereted in prison. In October 2004 he undertake a hunger strike during one month. In November 2004 he was hospitalized for global dehydration and shock. His physical examination showed blood pressure 60/40 mmHg, weight 59 Kg with a loss of weight about 10 Kg, diuresis 800 cc/day. His biological findings showed urea 100 mmol/l, creatinemia 679 (mo/l, natremia 179 mmol/l, kaliemia 5 mmol/l, glycemia 5.2 mmol/l, albuminemia 35 g/l, calcemia 2.35 mmol/l and biological marques of rhabdomyolysis: CPK at 11 times the normal and LDH two times the normal. His treatment consisted on rehydratation, parenteral then enteral refeeding and psychiatric talks. The evolution was favourable, re-establishment of good hydration state with a gain weight of 7 Kg, normalization of renal function, his creatininemia reached 85 (mol/l in three weeks and normalization of muscles enzymes in one month. CONCLUSION: Hunger strike continue to pose a problem because of it's frequency in penitentiary structures and its organic disorders which can lead to death. A good psychiatric cares may be undertaked in order to prevent a such bad manifestations.

[Myeloma, Kaposi's sarcoma and HHV8 infection in hemodialyzed patient]. / Myelome multiple, maladie de Kaposi et infection par le HHV8 chez un hemodialyse chronique.

BACKGROUND: The association between Kaposi's sarcoma, Human Herpes Virus 8 infection and multiple myeloma is still controversial especially in hemodialysed patient. AIM: report a new case of this association. OBSERVATION: We report the case of a 83 year old man in whom the diagnosis of multiple myeloma of IgA/kappa had been made in December 2003 with end stage renal failure requiring hemodialysis. Initially, it had been treated with Melphalan, Cyclophosphamide, Prednisolone and Vincristine and secondary by Melphalan and Prednisone. Three months later, he had developed extensive porpour lesions in his lower limbs. Skin biopsy had been informed of Kaposi's sarcoma. Human Herpes Virus 8 test was positive. CONCLUSION: Our observation is another case supporting the hypothesis that Kaposi's sarcoma and multiple myeloma share a common aetiology such as Human Herpes Virus 8. The immunodepressed state related to aging, multiple myeloma, chemotherapy and hemodialysis was the probable factor responsible of rapidly progressive Kaposi's sarcoma in our patient. The association myeloma and Human Herpes Virus 8 infection is still controversial.

[A case of preserved fertility in an hemodialyzed patient with systemic lupus erythematosus]. / Cas original d'une fertilité particulièrement conservée chez une patiente lupique en hémodialyse chronique.

BACKGROUND: Pregnancy and child birth in haemodialysis remains a rare event, even more, when pregnancy occurs in patient with systemic lupus erythematosus (SLE). AIM: We report a case of a patient with end stage renal failure secondary to SLE and who carried out 3 pregnancies. CASE REPORT: She was a woman with proliferative and diffuse lupus glomerulonephritis since 1985 treated by corticosteroids and cyclophosphamide. In 1995, she developed chronic renal failure. In 1996, she underwent a 1st full term spontaneous pregnancy with normal birth weight. In 2001, she underwent a second spontaneous pregnancy while she was on periodic haemodialysis. She had a full term baby birth with a birth weight at 1 Kg 700 and who died 4 days later. In 2002, she had a third pregnancy with voluntary abortion at 8 weeks. CONCLUSION: Our patient had conserved fertility despite treatment by cyclophosphamide, chronic renal failure and haemodialysis. The two successful deliveries may be attributed to the control of SLE activity and to the adequacy of haemodialysis.

[Complications of distal tubular acidosis in primary Sjögren's syndrome]. / Complications de l'acidose tubulaire distale au cours du syndrome de gougerot Sjogren primitif.

Renal involvement in primary Sjögren's syndrome occurs in 10-60% of cases. Tubulointerstitial nephritis with distal renal tubular acidosis (DRTA) is the main type of involvement. It's generally asymptomatic and revealed by complications of DRTA. We report 4 cases of GJS complicated by nephrocalcinosis, tetapresic hypokaliemia and osteomalacia. In 2 cases, nephrocalcinosis was diagnosed simultaneously with the GJS. The 2 other cases were diagnosed 3 and 15 years after primary GJS. All patients were treated by coticosteroids at the dose of 0.5 mg/Kg/jour, alkaline solution, K Cl in 2 cases and vitamin D in 2 cases. After a mean follow up of 8.7 years, the renal function remain stable in the 3 cases of nephrocalcinosis and in a patient with osteomalacia, bone lesions progressed. In conclusion, DRTA must be detected by acidification tests in patients with primary Sjögren's syndrome because of their latency and to prevent severe complications.

[Emphysematous pyelonephritis. Two cases]. / La pyélonéphrite emphysémateuse. A propos de deux observations.

The emphysematous pyelonephritis is a rare and severe renal infection characterized by the presence of gas in renal parenchyma and its perirenal spaces. We report two cases of emphysematous pyelonephritis in two diabetic women (53 and 50 years old respectively). In the first case, the treatment was based on nephrectomy because of the presence of a septic shock and three risk factors, which are acute renal failure, hematuria and thrombopenia. In the second case, the treatment was only medical. The evolution was favorable in the two cases. We insist in this article that this diagnosis should be considered in every female diabetic patient having severe acute pyelonephritis resistant to a well-conducted medical treatment.

[Renal amyloidosis complicating the outcome of chronic inflammatory colitis]. / Amylose renale compliquant l'évolution de colites inflammatoires chroniques.

INTRODUCTION: In 4 patients we observed the association of an amyloid nephropathy and a chronic inflammatory bowel disease (Crohn's' disease in 3 cases and ulcerative rectocolitis in 1 case). OBSERVATIONS: These patients, aged a mean of 37 years (range: 28-48 years), had been admitted for exploration of a nephrotic syndrome associated with renal failure in 2 cases. The investigations lead to the diagnosis of AA type amyloidosis in the 4 cases. One patient was lost from follow-up. One patient was treated with salazopyrine, one with corticosteroids and one with colchicine. After a mean follow-up of 16 months (5-30 months), all the patients had persistent nephrotic syndrome, with end stage renal failure in one case, persistence of normal renal function in one case and improved renal function in one case. None of the patients exhibited remission in the nephrotic syndrome. The response of amyloidosis to the treatment of the chronic inflammatory bowel disease varied. Corticosteroids and colchicine stabilised renal function in 2 of our patients but without remission in the nephrotic syndrome. DISCUSSION: AA amyloidosis is a rare complication of inflammatory bowel disease. The indication for colchicine is important to consider particularly since the response of amyloidosis to the treatment of the causal disease does not, in the majority of cases, lead to the remission of the amyloidosis, the prognosis of which is determined by the extent of renal involvement.