C3 glomerulopathy associated to multiple myeloma successfully treated by autologous stem cell transplant.

A 32-year-old male presented with advanced renal failure and nephrotic proteinuria due to lambda light chain multiple myeloma. Renal biopsy showed a proliferative glomerulonephritis with isolated C3 deposits. Renal recovery was obtained after chemotherapy and autologous stem cell transplant. We review previously described cases of C3 glomerulopathy associated with monoclonal gammopathy.

Kinetics of parathyroid hormone after parathyroidectomy in chronic hemodialysis patients.

Secondary hyperparathyroidism is a common complication in chronic renal failure. The treatment in some cases requires parathyroidectomy. The kinetics of the parathyroid hormone (PTH) levels after surgery helps to evaluate the efficacy of parathyroidectomy. Prospective analysis was made of the kinetics of intact PTH (iPTH) after parathyroidectomy in 10 chronic hemodialysis (HD) patients who had secondary hyperparathyroidism. We determined the levels of iPTH before surgery and its evolution after parathyroidectomy at regular intervals: Day 0, D7, D15, D30 and D90. The mean age of our patients was 40 ± 13 years, with a sex ratio of 1. The mean duration on HD was 122 ± 63 months. The duration of secondary hyperparathyroidism varied from one year to 12 years. All patients had received medical treatment for hyperparathyroidism. The indications for parathyroidectomy included resistance to medical treatment in seven cases, development of brown tumors in two cases and soft tissue calcifications in one case. All patients had radiographic evidence of hyperparathyroidism. The parathyroidectomy was sub-total in all patients, 6/8 in four cases and 7/8 in six cases. The mean iPTH level was 2341 ± 1946 pg/mL before surgery. A sharp drop in this level was noticed on D0, with a median of 92 pg/mL and, thereafter, the levels were 79 pg/mL on D7, 25 pg/mL on D15 and 36 pg/mL after 1 month. At 3 months post-surgery, the mean iPTH level was 302 pg/mL. Histological examination of the resected gland showed parathyroid hyperplasia in all patients. In our series, the efficacy of sub-total parathyroidectomy was satisfactory with rapid normalization of PTH, which is consistent with the literature data. Sub-total parathyroidectomy still has a place in the treatment of secondary hyperparathyroidism in chronic renal failure. Its indications should be limited to cases resistant to medical treatment and, in particular, in cases with occurrence of complications.

[Renal amyloidosis revealing a Kartagener's syndrome]. / Amylose rénale révélant un syndrome de Kartagener.

Kartagener's syndrome is a rare disease. It is a clinical variant of primary ciliary dyskinesia. It combines a situs inversus, chronic sinusitis and bronchiectasis. Glomerulopathies associated with this syndrome are rarely reported. We report the case of Kartagener's syndrome in a patient diagnosed at the age of 37 years with renal amyloidosis.

Translation, cultural adaptation and validation of the kidney disease quality of life-short form 1.3 in an African country.

BACKGROUND: The impact of dialysis on patient quality of life has been recognized as an important outcome measure. The Dialysis Outcomes and Practice Patterns Study compared quality of life in 4 continents [1], but very scarce information is available about dialysis patients' quality of life in Africa. The objective of this study was to translate the Kidney Disease Quality of Life-Short Form (KDQOL-SF) into Moroccan and measure its psychometric properties. METHODS: The questionnaire was first translated into Moroccan by 2 independent translators, and then 2 backward translations into English were performed after pretesting in 10 dialysis patients. The final questionnaire was then administered to 80 dialysis patients. Reliability was estimated by internal consistency and test-retest reliability. Validity was assessed using known group comparisons and correlations between overall health rating and scales scores. RESULTS: Some activities were substituted since they were not common in Morocco. All subscales had a Cronbach α above the recommended value except for 3 scales. All of the items showed good test-retest reliability. Correlation of items within subscales was higher than that of items outside subscales in 87% of cases. Regarding construct validity, all KDQOL-SF scales had significant correlation with overall health rating except for sexual function and dialysis staff encouragement. Furthermore, the questionnaire could be used to discriminate between subgroups of the patients. CONCLUSIONS: The psychometric properties of the KDQOL-SF resulting from this first-time administration of the instrument support the validity and reliability of the KDQOL-SF as a measure of quality of life of patients having hemodialysis in Morocco.

Ten years of renal transplantation in a Moroccan hospital: results and constraints.

INTRODUCTION: Renal transplantation (RT) offers several advantages to end-stage renal disease (ESRD) patients, such as a better quality of life and economic benefits. This study sought to report a 10-year experience of RT in a developing country as well as the barriers to its improvement. PATIENTS AND METHODS: The retrospective study included 67 patients who underwent a first RT from a related living donor (RLD) between June 1998 and December 2008. We noted pretransplantation donor and recipient parameters as well as the results and barriers to RT promotion in our country. RESULTS: The mean overall age of our patients including 43 males (64.1%) and 24 females (35.8%) was 30 ± 9.6 years. Teenagers from 13 to 18 years of age represented 9% of the recipients. Immediate failure was observed in 5 cases due to vascular thrombosis (n = 3) or hyperacute rejection (n = 2). Graft and patient survival rates at 1 year were 92.6% and 97%, respectively. CONCLUSION: The limited number of RT in our country may be explained by the lack of both human and material resources as well as the limited pool of living donors. Nonetheless, the economic gain subsequent to RT should encourage promotion of this treatment mainly through dissemination of information.

[Evaluation of the prescription of long-term systemic steroid-therapy associated measures]. / Mesures adjuvantes à une corticothérapie systémique prolongée : que faut-il prescrire ?

INTRODUCTION: To prevent the occurrence of corticosteroid's side effects, adjuvant measures are prescribed in a very different way depending on the practitioner's experience. The aim of this study was to assess the physicians' practices regarding the prescription of long-term corticosteroid therapy. METHODS: In May 2010, we realized a cross-sectional study among physicians in Ibn Sina University Hospital in Rabat. With a data collection form, we assessed the principal indications for prescribing steroids and the frequency of prescription of measures sometimes associated with systemic corticosteroid. RESULTS: Fifty-nine out of eighty physicians completed the data collection form (response rate: 69.4%). Calcium-vitamin D supplementation was recommended by most of the physicians. Half of them were prescribing biphosphonate therapy. Most of them propose a low-sodium, a low-fat and a low-carbohydrate dietary regimen. Regular physical exercise was recommended by 35% of physicians. Gastric protection was indicating by 54.4% of practicians and potassium supplementation by 42.1% of them. Finally, the recommendation for influenza vaccination, hydrocortisone prescription and Synacthen(®) test, varied between physicians. CONCLUSION: The prescription of adjuvant measures with a long-term corticosteroid therapy was very heterogeneous. There is no consensus regarding most of the measures.

Post-transplantation diabetes mellitus.

To determine the prevalence of post-kidney transplantation diabetes (PTDM) and to assess its risk factors, we retrospectively studied 92 non-diabetic kidney transplant patients. The immunosuppressive drugs used to prevent rejection included prednisone, a calcineurin inhibitor (cyclosporine or tacrolimus) and an antimetabolite (azathioprine or mycofenolate mofetil). Diabetes was defined according to the WHO criteria and the American Diabetes Association. The mean age of our patients was 35.8 ± 10.5 years, and there was a clear male predominance (56 men and 36 women). The graft was from living related donor in 71/92 (76%) patients. The prevalence of diabetes in post-kidney transplant was 15.2%. The factors increasing the occurrence of PTDM included advanced age, high doses of steroids and cyclosporine. Management of PTDM included diet modification, oral anti-diabetic and insulin. We conclude that the prevalence of PTDM is significant in our transplant population and risk factors for its development are multiple and require aggressive multifaceted management.

[Renal failure in sarcoidosis]. / L'insuffisance rénale au cours de la sarcoïdose.

Renal sarcoidosis is rare and may lead to renal failure in less than 3% of patients. It may occur as a consequence of calcium metabolism disorders or granulomatous interstitial nephritis. In this retrospective study, we present five patients with renal failure secondary to sarcoidosis diagnosed in our centre on one period of nine years. Patients were three males and two females with a mean age at the time of the diagnosis of 31,6 years. Pulmonary involvement was present in three cases. Renal biopsy revealed granulomatous interstitial nephritis lesions in all patients. Extra-membranous glomerulonephritis was present in one case. In another case, moderate interstitial fibrosis was observed. Corticosteroid therapy using prednisolone 1mg/kg per day was used in all patients. Three patients had methylprednisolone pulse before oral corticosteroid therapy. One patient required several sessions of haemodialysis. All patients were followed up for a mean period of 52,6 months (ranged from 13 to 84 months). All patients improved their renal function with normalization of creatininemia in two of them. Renal involvement in sarcoidosis is probably underestimated. Corticosteroids therapy is efficient and must be introduced early to prevent progression to chronic renal failure.

[Iatrogenic Kaposi's disease in Morocco in a non-transplant context]. / La maladie de Kaposi iatrogène au Maroc en dehors du contexte de la transplantation.

BACKGROUND: Kaposi's disease (KD) is a multifocal disease affecting the skin and viscera. KD can occur in an endemic setting: it may be associated with human immunodeficiency virus (HIV) or it may occur as a complication of immunosuppression, particularly of iatrogenic origin in transplant patients. The purpose of this study is to describe the epidemiological, clinical and therapeutic profile and the course of iatrogenic KD in Morocco in a setting not involving organ transplantation. PATIENTS AND METHODS: A retrospective study conducted at the dermatology department of the Ibn Sina hospital centre in Rabat, Morocco, covering a 21-year period and including 14 patients presenting histologically confirmed iatrogenic KD. RESULTS: Eight men and six women were included with a mean age of 56 years. All patients received corticosteroids, in combination with cyclophosphamide in three cases and with azathioprine in one case. The mean time to onset of lesions after the start of treatment was 16.5 months. The presentation in all cases was cutaneous. Impaired mucosal membrane was seen in 35.7% of patients, with visceral involvement being seen in only one patient. HIV serology tests were negative in all patients but HHV8 serology tests were positive in 78.5% of patients. Treatment consisted primarily of reduction or withdrawal of the immunosuppressant. The outcome was favourable in the majority of cases. CONCLUSION: In Morocco, KD is a rare but not exceptional complication of immunosuppressant therapy, particularly corticosteroids. The disease presented as a skin disorder in all of our patients, thus emphasising the value of regular follow-up and routine dermatological examination of patients on immunosuppressant therapy, and suggesting the value of screening for HHV8 infection before initiating such therapy.

Epidemiology and prevention of Staphylococcus aureus nasal carriage in hemodialyzed patients.

UNLABELLED: Nasal carriage of Staphylococcus aureus may be responsible for some serious infections among hemodialyzed patients. Its pathogenic potential and commensal nature allows for an easy transmission both in and out of hospital environment. PURPOSE: This study was to assess the prevalence of S. aureus nasal carriage, to determine its frequency and nature in hemodialyzed patients of the Rabat Ibn Sina University hospital, in Morocco. PATIENT AND METHOD: The study began in March 2008 according to the following protocol: screening of nasal carriage with five samplings, performed once a month three times, then once a month two times again after an interruption period of three months. Screening was performed weekly during the first month in hemodialyzed patients treated with mupirocin (Bactroban(®) 2%), and then monthly, to monitor the kinetics of S. aureus eradication. RESULTS: The study included 54 hemodialyzed patients with a mean 44.16±14 years of age, sex ratio of 0.54, and mean hemodialysis duration of 118.7±67 months. Permanent and intermittent S. aureus carriage was found in respectively 18.52% and 25.92% of patients. Eighty-one strains of S. aureus were identified, 14.81% of which were methicillin resistant. Eradication was sustained beyond 20 months in patients treated with mupirocin. CONCLUSION: This investigation allowed us to identify hemodialyzed patients at risk, so as to implement the rules of individual and collective hygiene, and to extend mupirocin antibiotic prophylaxis in our hemodialysis unit.

[Renal amyloidosis: uncommon complication of sickle cell disease]. / L'amylose rénale: une complication inhabituelle de la drépanocytose.

Type AA amyloidosis is a rare complication of sickle cell anemia. The purpose of this report is to describe the case of a 30-year-old man with heterozygous sickle cell disease who was referred to our unit with nephritic syndrome and microscopic hematuria. Light microscopy on a renal biopsy specimen demonstrated AA amyloidosis. After elimination of other causes, it was concluded that amyloidosis was the result of recurrent acute inflammation secondary to sickle cell disease. To our knowledge, this is the fifth that renal amyloidosis as a complication of sickle cell disease has been described in the literature.

[Pregnancy in chronic hemodialysis patients]. / Grossesse chez les patientes hémodialysées chroniques.

INTRODUCTION: Pregnancy in hemodialysis patients is a rare event, with a high risk of maternal and fetal morbidity. The aim of our study is to report our experience in management of pregnancies occurred in hemodialysis patients, and clarify the factors of good prognosis. PATIENTS AND METHODS: We identified all pregnancies occurred in patients on hemodialysis between January 2000 and July 2010, and analysed their clinical and biological characteristics. The adjustment parameters of hemodialysis sessions, and treatment adjustments have been specified. The maternal and fetal complications and pregnancy outcome have been reported. RESULTS: Over a period of 10 years and 6 months, among 23 patients of childbearing age, 14 patients were married. We observed 11 pregnancies in eight patients, with an incidence of 7,14% year, and a prevalence of 71,4%. The evolution was marked by intrauterine fetal death in one case, medical pregnancy termination in a second one and four spontaneous abortions. Only five pregnancies were completed. The average age of conception was 34 years. The average duration of dialysis prior to conception was 76 months. Pregnancy diagnosis was made on average after 10,4 weeks. The mean gestational age at delivery was 33,6 weeks, and the mean newborn weight was 2070g. Apgar score was of 10/10 in the first minute in all newborns. No maternal complication was found. One case of intrauterine growth restriction and another case of pyelo-caliceal dilatation with resolving renal insufficiency were reported. In the long term, the fetal psychomotor development was normal. DISCUSSION: The fertility chronic hemodialysis patients in childbearing age have improved together with the quality of dialysis, with an increased incidence of pregnancy and the proportion of viable children. However, treatment modalities are not clearly established, because of the absence of randomized studies and the small number of pregnancies during the playoffs. Treatment of anemia with erythropoietin and blood transfusions, and the improvement in blood urea by extensive dialysis in women on hemodialysis, may improve outcomes by reducing the incidence of prematurity and increasing gestational age. CONCLUSION: Pregnancy in hemodilalysis patient is a rare event, but desired and valuable. Its completion requires a multidisciplinary management involving the nephrologist to ensure adequate dialysis and the gynecologist to ensure good obstetrical monitoring.

[Dental status in 42 chronically hemodialyzed patients]. / L'état buccodentaire chez les hémodialysés chroniques: 42 cas.

INTRODUCTION: Hemodialyzed patients often present with a deficient dental state. The objective of this study was to assess the oral state and dental hygiene of chronically hemodialyzed patients. METHODS: Forty-two chronically hemodialyzed patients were followed at the dental consultation and treatment centre. Clinical and radiographic data was assessed (panoramic and retro alveolar x-ray). RESULTS: The patients' mean age was 41.7 years. The sex ratio was 16/26. 11.9% of patients were toothless. Dental hygiene was bad in 45.9% of the cases. 78.5% of patients brushed their teeth 35.1% of whom once a day. Periodontitis was noted in 73% of the cases and gingival bleeding in 48.6%. 89.2% of patients presented with caries and 78.4% were missing teeth. Nineteen percent of the patients used a prosthesis. Chewing was adequate for 47.6% of patients. The paleness of mucosal membranes and xerostomia was noted respectively in 40.5% and 21.4% of patients. The radiological exam findings were pathological for ten patients (23,6%). The mean plaque index was 1.9±0.84 and gingival index 1.8±0.87. 71.4% of patients consulted an odontologist in the year before the study. DISCUSSION: The dental status of chronically hemodialyzed patients is bad. They must be managed as early as possible.

Prevalence and predictive factors of anemia after renal transplantation: a Moroccan report.

OBJECTIVE: Anemia, a common multifactorial problem in kidney transplant recipients, represents an important cardiovascular risk factor. The purpose of this study was to assess anemia prevalence after kidney transplantation, the main factors involved in its occurrence, its cardiovascular consequences, and its impact on patient survival and graft function. METHODS: This retrospective study evaluated 69 patients undergoing renal transplantation between January 1998 and September 2008 with ≥1 year of follow-up. For all of the patients, we recorded hemoglobin concentrations before and at 1, 3, 6, 12, 36, and 60 months after transplantation. Anemia was defined as recommended by the American Society of Transplantation: hemoglobin level <12 g/dL in women and <13 g/dL in men. To determine the factors involved in anemia occurrence, we compared 2 groups of patients, with versus without anemia, at various times after renal transplantation. RESULTS: This study showed a high prevalence of anemia in the early posttransplantation period of 82.7% and 42% of kidney transplantation patients at 1 month and 6 months, respectively. It was mainly related to a low pretransplant hemoglobin level. The prevalence declined to 37.7% at 1 year. Renal graft dysfunction was the most important factor in the occurrence of late post-renal transplantation anemia. The presence of anemia increased the risk of renal graft functional deterioration by a factor of 2.9. The decreased prevalence at 1 year after transplantation was significantly associated with a reduction in left ventricular hypertrophy. CONCLUSION: The management of anemia is essential to improve renal graft survival, reduce cardiovascular morbidity, and ensure a better quality of life for renal transplant recipients.

[Glomerulonephritis and thymoma: a case report and review of the literature]. / Glomérulonéphrite et thymome : un cas clinique et revue de la littérature.

The authors present a case of nephrotic syndrome due to focal-segmental glomerulosclerosis, occurring three years after thymectomy and myasthenia gravis. Twenty-three other cases of nephrotic syndrome associated with thymoma and myasthenia gravis have been reported in the literature. The nephrotic syndrome may be related to T-cell dysfunction associated with thymoma.

Pregnancy in renal transplant recipients.

OBJECTIVE: Renal transplantation with a well-functioning graft leads to a rapid restoration of endocrine and sexual functions. The aim of this study was to examine our experience with pregnancies among renal transplant patients, particularly with regard to their impact on graft function. PATIENTS AND METHODS: We analyzed 10 pregnancies in 7 renal transplant recipients for long-term graft outcomes in terms of clinical and biological data. RESULTS: The mean patient age was 28.5 +/- 4 years. They all received a living donor kidney. The time between transplantation and the onset of pregnancy was 33.4 +/- 23.2 months. Regarding the immunosuppressive therapy, all patients received steroids and cyclosporine; 4 patients received in addition azathioprine and 2 received mycophenolate mofetil that was changed at 1 month before conception to azathioprine. There was no significant difference between the serum creatinine before and during pregnancy. We did not observe any acute rejection episode. Pregnancy complications were preclampsia in 1 case, hypertension in 1 case, urinary tract infection in 2 cases, and anemia in 80% of patients during the third trimester. Premature rupture of membranes occurred in 1 case and preterm delivery in 2 cases. Two cases of neonatal death were registered. Cesarean section was performed in 50% of cases. The follow-up revealed 2 cases of chronic rejection. CONCLUSION: A multidisciplinary approach is necessary for pregnancy which generally occurs at 2 years after kidney transplantation.

[Nephroprotection in young type 1 diabetic patients treated with converting enzyme inhibitor]. / Néphroprotection chez le jeune diabétique de type 1 traité par inhibiteur d'enzyme de conversion. Résultats préliminaires d'une étude prospective.

CONTEXT: Diabetic nephritis is a renal microangiopathy that represents a major cause of morbidity and mortality in diabetic patients. It is expressed either by microalbunuria, proteinuria or renal failure, depending on the stage of the diabetes. In this context, angiotensin converting enzyme inhibitors (ACEI) slow down the progression of renal damage. OBJECTIVE: To assess the nephroprotector effects of ACEI in young type 1 Moroccan diabetics with varying stages of renal damage. Methods Prospective study including 29 patients exhibiting a diabetic nephropathy and/or hypertension having been followed-up for 1 year and treated with ACEI. The following parameters were analysed on inclusion, at six months and after 1 year of treatment: systolic arterial pressure (SAP), diastolic arterial pressure (DAP), mean arterial pressure (MAP), urinary excretion of albumin, 24-hour proteinuria, creatininemia, creatinine clearance, glycosylated haemoglobin, kalemia, total cholesterol and triglycerides. RESULTS: The mean age of our patients was of 23.6 +/- 5.5 years, the age at onset of diabetes was of 9.3 +/- 2.6 years. According to the renal damage, we determined 4 groups of patients: Group I: microalbuminuria (10 patients), Group II: proteinuria (7 patients), Group III: renal failure (6 patients), Group IV: isolated hypertension (6 patients). Study of the progression of the clinical and biological parameters, during treatment with converting enzyme inhibitors (combined with diuretics in Groups II and III) revealed: In Group I: a decrease in urinary excretion of albumin, which returned to normal in 3 cases, in Group II: a decrease in the proteinuria, which became a microalbuminuria in 4 cases, in Group III: a stabilisation of renal function concomitant to a reduction in proteinuria, in Group IV: a significant reduction in mean arterial pressure. CONCLUSION: One year of treatment with ACEI appears effective on reducing proteinuria levels and stabilising the renal function in young type 1 diabetic patients.