A.T. Lemrabott

Problematic of Vascular Access for Hemodialysis in Sub-Saherienne Africa: Experience of Dakar

Aim: Vascular access is important for a good survive in hemodialysis. It can be temporary or permanent, and needs particular attention because of possible complications, especially infectious. We conducted a study to determine the type and the outcome of vascular access in two hemodialysis units in Dakar. Materials and Methods: A multicenter retrospective study was conducted from the 1st January 2005 to 10 September 2010 in two hemodialysis centers: Hospital Aristide Le Dantec and the MDTC (Multifunctional Diagnosis and Treatment Center) of Dakar. All the patients- regularly dialyzed since at least three months in these centers were included. Results: Sixty five patients were included. The mean age was 50.2 years with a sex ratio of 1.7. The mean duration in hemodialysis was 23.2 month. Only five patients (7.80%) had an AVF before starting in hemodialysis. Sixty patients (92.2%) started dialysis with a central venous catheter. Among them, 49 (81.6%) had a double lumen femoral catheter and 11 had a jugular one with 4 tunneled. Complications of vascular access were noted for 23 patients (39.7%) with 12 infections. Blood culture was positive for Staphylococcus Aureus in seven cases. Seven patients presented lower limb thrombophlebitis. Thrombosis of the catheter was noted in four patients. Radial arterio-venous fistula (AVF) was the first intention permanent access in 41 cases (64.1%) followed by cephalic AVF in 14 cases (21.9%) and basilic AVF in 9 (14%). AVF complications were presented in 56.9% of case (40 patients). Most of them was early defect in 18 cases (27.7%), thrombosis in 9 (13.8%) and stenosis in 5 (7.7%). Radial AVF infection to Staphylococcus Aureus was noted in 3 cases, and one pseudo aneurysm was noted. Conclusion: Vascular access for hemodialysis is a great problem in Senegal. More than two third of our patients start dialysis on venous catheters, this is far from the clinical practice guidelines.

Light Chain Deposition Disease in Black African, Report of Three Cases from Senegal

Background: Although there are numerous publications on multiple myeloma in black people, it seems sub-Saharan literature particularly Senegalese’ one is silent about Light chain deposition disease (LCDD) which is linked to immunoglobulin light chain deposition in glomerulus. The authors report the first three observations of LCDD collected in Nephrology department of Aristide Le Dantec hospital in Dakar, Senegal. Cases: we report three cases a man and two women of 61, 69 and 47 years old respectively, admitted to the Nephrology department of Aristide Le Dantec hospital for rapidly progressive renal failure in one case and a nephrotic syndrome in 2 other cases. The renal biopsy showed a nodular glomerulosclerosis and immunofluorescence microscopy, revealed deposition of IgG light chains suggesting LCDD. On the other hand, the diagnosis of multiple myeloma of IgG kappa type in 2 cases and IgG lambda type in the other one was done. A combination of chemotherapy (Mephalan Prednisone) and hemodialysis was instituted for all 3 cases. The evolution was marked by the appearance of an end stage renal disease in 2 cases and the third one was expired due to an infected bed sore secondary to a pathological fracture of the neck of the femur. Conclusion: Although the LCDD is rare, the prognosis of this syndrome seems to be poor as more than half of the patients die or progress to ESRD within 2 years.

Management of Multiple Endocrine Neoplasia Type 2A (MEN 2A): Diagnostic and Therapeutic Concerns with the First Documented Senegalese Family

Introduction: Multiple endocrine neoplasia (MEN) type 2A is a multiglandular tumor condition inherited in an autosomal dominant manner. It is related to proto-oncogene RET mutation whose analysis is the best technique for family screening. It features in a variable way medullary thyroid cancer (MTC), primary hyperparathyroidism (HPT) and pheochromocytoma. The revealing manifestations of these tumors are often neglected for a long time and the screening should be systematic particularly in a known family context. Methods: After a family tree establishment of a MEN 2A index case, a family survey allowed to diagnose other cases in the family by means of biological, radiological and/or genetic examinations. Results: We report a family form of MEN 2A in a family of three households. In this family 13 people (index case included) were probed out of 34 members. The average age of our patients was 43.54. The sex ratio men/women was 0.85. The simultaneous diagnosis of a primary HPT and a MTC was carried out in our index case and constituted the circumstance of discovery of MEN 2A. The time limit of MEN 2A diagnosis on the other family members was on average 7.7 years. A MTC was recorded in 7 patients. It was asymptomatic in overall cases. A pheochromocytoma was present in only one patient. Primary HPT was found in four patients. Renal lithiasis with recurrent unilateral or bilateral nephritic colic attacks was the main manifestation. Besides the index case, 11 patients had a genetic testing. In 7 patients, a mutation on proto-oncogene RET located on the codon 634 was noted. A surgical care was carried out on 6 patients. We recorded three patients lost to follow-up. A patient died before surgery. In the index case, biological and radiological monitoring found a locoregional residual disease that indicated surgical revision and radiotherapy. Prophylactic thyroidectomy was not performed in any case driven by lack of compliance and/or low income. Conclusion: The discovery of a MEN 2A case imposes genetic survey allowing the screening of other cases in the family and the establishment of a preventive strategy.

Le syndrome néphrotique idiopathique (SNI) de l’enfant à Dakar: à propos de 40 cas

Introduction L’objectif de ce travail était d’analyser les caractéristiques diagnostiques, thérapeutiques et évolutives de l’enfant atteint de néphrose dans un service de pédiatrie de Dakar. Méthodes L’étude était réalisée au service de pédiatrie de l’hôpital Aristide Le Dantec. Il s’agissait d’une étude rétrospective sur une période de 03 ans allant du 1er janvier 2012 au 31 décembre 2014. Ont été inclus tous les patients âgés de 02 ans à 12 ans présentant un tableau de Syndrome néphrotique idiopathique. Résultats Quarante cas de néphrose étaient colligés soit une prévalence de 23% parmi les néphropathies prises en charge dans le service. L’âge moyen était de 7,11± 3,14 ans. Le syndrome néphrotique était pur chez 72,5% (n=29) des patients. Les œdèmes des membres inférieurs étaient présents chez 100% des patients, l’oligurie dans 55% (n=22) et l’HTA dans 5% (n=2) des cas. La protéinurie moyenne était de 145,05 ± 85,54 mg/kg/24heures. La protidémie moyenne était de 46,42 ±7,88 g/L et l’albuminémie moyenne de 17,90 ± 7,15 g/L. Trente-neuf patients avaient reçu une corticothérapie à base de prednisone. La corticosensibilité était retenue chez 77% (n=30) des patients et la corticorésistance chez 13% (n=5) des cas. Le facteur de mauvaise réponse à la corticothérapie était un niveau de protéinurie initiale supérieure à 150 mg/kg/jour (p = 0,024). La biopsie rénale était réalisée chez 18% (n=7) des patients et retrouvait dans 57,2% (n=4) des cas une hyalinose segmentaire et focale. Le cyclophosphamide et l’azathioprine étaient associés aux corticoïdes dans 10% (n=4) des cas chacun. Le taux de rémission globale était de 89,8%. L’évolution vers l’insuffisance rénale chronique était notée chez trois (03) des patients. Conclusion La néphrose représentait près du quart des néphropathies prises en charge dans notre service. Le taux de rémission globale était élevé. Le seul facteur de mauvaise réponse à la corticothérapie était le niveau de protéinurie initiale élevée. En cas d’indication de la biopsie rénale chez nos patients, la HSF était la lésion la plus fréquemment retrouvée.

Scleroderma renal crisis in tropical region: two senegalese cases

Scleroderma renal crisis (SRC) is defined as the new onset of accelerated arterial hypertension and /or rapidly progressive oliguric renal failure during the course of systemic sclerosis. It is a rare but life-threatening complication. This formerly serious complication has got a considerable brighter outlook since the introduction of angiotensin converting enzyme inhibitors (ACE) however the mortality is still remaining high. We report two cases of SRC which to our knowledge are the firsts described in Dakar. They were two women aged 45 and 32 years, one of them was previously following for systemic sclerosis. Both of them had malignant hypertension associated with rapidly progressive renal failure, the other was put under corticosteroid therapy four months before SRC occurrence. The histological and laboratory finding showed thrombotic microangiopathy. The height blood pressure returned to normal value after treatment with ACE inhibitors. The final outcome was undesirable with the death of one after two months due to the hemodialysis discontinuation and persistence of renal failure in the other.

Hemophagocytic Syndrome, an Uncommon Complication of Microscopic Polyangitis: A Case Report From Senegal

Introduction: We reported a case of hemophagocytic syndrome complicating microscopic polyangitis presented by crescentic glomerulonephritis. Case Presentation: A 22-year-old female patient originated from Dakar, Senegal presented with nephrotic syndrome and rapidly progressive glomerulonephritis. On physical examination, we noticed hyperchromic diffuse punctilious purpura skin lesions predominant on the trunk, the neck and the upper thigh. Immunology investigations revealed strongly positive anti SSA/Ro and anti-SSB. Anti-neutrophil cytoplasmic antibodies had positive results with a peri-nuclear type fluorescence, specific to myeloperoxidase. In optic microscopy, renal biopsy showed a crescentic glomerulonephritis with circumferential cellular and fibrous proliferation affecting 85% of glomeruli. The diagnosis of microscopic polyangitis with renal and skin involvement was retained. The patient received methylprednisolone and cyclophosphamide 700 mg/m2 every 15 days for the first 3 pulses and every 21 days thereafter. After the 5th month, she developed obnubilation, fever and central pancytopenia. Bone marrow aspiration was performed, which showed medullary invasion by macrophages with signs of hemophagocytosis. Diagnosis of hemophagocytic syndrome complicating a microscopic polyangitis was retained and methylprednisolone pulses started. The patient was under hemodialysis after follow-up of about 9 months with stable clinical state. Conclusions: The occurrence of SAM in pauci-autoimmune vasculitis is rarely described, particularly in Africa. Our case is an illustration of the reality of this association.