Mortality of diabetic on chronic dialysis in Tunisia.

Survival rates of diabetic patients on maintenance hemodialysis (HD) are reported to be poorer in Tunisia than in developed countries. This study aims to specify the epidemio-logical, clinical, and biological characteristics of diabetic disease, chronic kidney disease (CKD), and comorbidities at the initiation of HD and investigate factors associated with mortality during the dialysis period. We retrospectively analyzed the outcome of diabetic patients who were initiated on HD from 2007 to 2012 at the Rabta Hospital of Tunis. During the follow-up period, all morbid events and deaths were recorded. Univariate analysis and multivariate analysis were performed to identify risk factors associated with mortality in our population. The study population included 142 patients. The mean age was 58.7 ± 11.9 years. The sex ratio male/female was 1.4. One hundred and twenty-seven patients were type 2 diabetics (89.4%). Diabetic nephropathy was the main initial nephropathy (87.3%). CKD was diagnosed at Stages 4 or 5 in 95.1% of cases. HD was started in emergency conditions in 68.6% of cases. The one-year and the five-year survival rates were 42% and 17%, respectively. At initiation of HD, low socioeconomic status (P = 0.001), advanced age (P = 0.008), low body mass index (P = 0.04), history of stroke (P = 0.04), peripheral neuropathy (P = 0.02), initial vascular access (P = 0.03), secondary hyperpara-thyroidism (P = 0.03), nephrotic-range proteinuria (P = 0.01), and glycated hemoglobin ≤7% (P = 0.03) were associated with higher mortality rate. During dialysis period, cardiovascular events (P = 0.02), infectious complications (P = 0.04), and secondary hyperparathyroidism (P = 0.04) were significantly more noticeable among deceased patients. Due to poor survival rates of diabetic patients on HD, prevention, early detection, and management of diabetic CKD patients should be the way to go forward.

[Kaposi sarcoma in a patient with ANCA vasculitis requiring hemodialysis]. / Sarcome de kaposi compliquant une vascularite à ANCA en hémodialyse chronique.

INTRODUCTION: Iatrogenic Kaposi's sarcoma is widely reported after transplantation. Less commonly, it occurs in patients receiving immunosuppressive therapy for ANCA associated vasculitis. We report here the rare association of Kaposi's sarcoma, prurigo nodularis and ANCA associated vasculitis in a hemodialysis patient. CASE REPORT: We describe a 58-year-old woman who presented granulomatosis with polyangeiitis with alveolar hemorrhage and renal failure requiring hemodialysis. She developed cutaneous Kaposi's sarcoma seven weeks after the beginning of immunosuppressive therapy. Biological tests showed negative HHV8 virus infection. Lesions of Kaposi's sarcoma responded to a discontinuation of immunosuppressive drugs and a decreasing dosage of corticosteroids. CONCLUSION: Our case showed that the immunosuppressed state related to multiple factors such as underlying disease, immunosuppressive therapy and hemodialysis may all have contributed to the development of this neoplastic disorder in our patient.

Atypical hemolytic uremic syndrome: A monocentric adult Tunisian study and review of literature.

Atypical hemolytic uremic syndrome (aHUS) is characterized by microvascular thrombosis resulting in thrombocytopenia, hemolytic anemia, and multiorgan dysfunction. It is associated with genetic or acquired disorders of regulatory components of the complement system. For our study, we collected data from 16 patients diagnosed with aHUS between January 2010 and January 2014. The mean age was 33.6 years. The female-to-male ratio was 3. The median follow-up duration was 27 ± 3.5 months. The most common clinical presentation was hypertension. Renal involvement was noted in all cases. Ten patients had extrarenal manifestations. Semi-quantitative dysfunction of the alternative pathway of complement was found in all cases. A genetic study was not available for our patients. During the acute stage, all patients received plasma therapy, and among them, seven required dialysis and five were still on dialysis at the time of discharge. One patient underwent renal transplantation. None of our patients received eculizumab perfusion. The renal survival was inversely correlated to young age (<30 years) (P = 0.001), presence of anti-factor H antibodies (P = 0.003) and serum creatinine at diagnosis >5 mg/dL (P = 0.02). Mortality rate was significantly correlated to young age (<30 years old) (P = 0.01). Collecting multicentric data on adult patients with aHUS will enable better characterization of the spectrum of adult aHUS in our country and the evaluation of current treatments and different outcomes.

A case of exorbitism in association with Wegener's granulomatosis with renal involvement.

Wegener's granulomatosis (WG) is a necrotizing granulomatous vasculitis involving the nose, paranasal sinuses, lungs, and kidneys. Ocular involvement can occur in about 50% of cases. There are very few reports of WG with orbital inflammation and exorbitism. We report a case of a female patient who presented with exorbitism related to orbital inflammation secondary to WG, with renal involvement. A 29-year-old woman with a previous history of recurrent pan-sinusitis presented with bilateral exophthalmos and renal failure with rapidly progressive glomerulonephritis. Computed tomography showed extensive bilateral soft tissue in the retro-orbital area. Immunologic tests showed the presence of type-C anti-neutrophil cytoplasmic antibodies and renal biopsy revealed pauci immune crescentic glomerulonephritis. The patient was treated with corticosteroids and pulses of cyclophosphamide followed by azathioprine and trimethoprim-sulfamethoxazole. After a follow-up of 10 months, the renal outcome was favorable with improvement of renal function but there was persistence of exorbitism and loss of visual function. Our case suggests that WG should be considered in the differential diagnosis of persistent bilateral exophthalmos. Prompt recognition of this early manifestation is important for the institution of early treatment.

Familial cases of glomerulonephritis complicating Crohn's disease.

A part from nephrolithiasis, renal involvement is rare in the course of Crohn's disease, particularly glomerulonephritis. On the other hand, while onset of Crohn's disease is strongly influenced by environmental and genetic factors, little is known regarding influence of these factors on extra intestinal manifestations. We report a familial case of glomerulonephritis that occurred in a 38-year old woman and her mother, 59 years old with a 7-year and a 37 year history of stenosing ileocolonic disease, respectively. Both of them developed peripheral oedema with nephrotic syndrome during the course of their Crohn's disease while they had no intestinal symptoms and were not receiving any maintenance therapy. Renal function was conserved in the former while the latter developed renal failure and had already small size kidneys on abdominal sonography. Thus, renal biopsy had been performed only in the former patient and had showed membranous glomerulonephritis. Investigations showed no other underlying disease than Crohn's disease. Through this report we emphasis possible genetic influence on extra intestinal manifestations, particularly glomerulonephritis, in Crohn's disease patients.

[Manifestation of congenital rubella syndrome: clinical and epidemiologic aspects]. / Les manifestations ophtalmologiques de la rubéole congénitale: aspects cliniques et épidémiologiques.

INTRODUCTION: Congenital rubella syndrome is an ensemble of congenital malformations which results from a primary viral infection in non-immunised pregnant women. The main ophthalmologic manifestation is cataract. It involves at the same time visual and vital prognosis and can be responsible for multiple handicaps. METHODS: We did a retrospective study of 16 infants (32 eyes) having undergone congenital cataract surgery compatible with congenital rubella syndrome in the Casablanca paediatric ophthalmology department between January 2001 and December 2005. All the patients underwent a complete ophthalmologic examination, otorhinolaryngologic, cardiovascular and neurological examinations, and paraclinic explorations. RESULTS: The results were compared with those reported in the literature. In our series, 25 eyes (78.12%) had a cataract of which 56% were nuclear, 13 eyes (40.62%) had microphthalmia. One case each of corneal dystrophy and iris coloboma were described. Congenital glaucoma was found in only one case. Pigmentary retinopathy was found in 12 eyes (37.5%). Seven patients (43.75%) had associated cardiac anomalies, 6 (37.5%) deafness, 5 (31.25%) psychomotor delay and 2 (12.5%) facial dysmorphy. CONCLUSION: Considering the permanent disabilities caused by congenital rubella syndrome, care should be taken with the follow-up of the pregnancies and an immunization program should be implemented for good control of the circulation of the virus.

[Bilateral optic neuropathy and non-Hodkin's lymphoma]. / Neuropathie optique bilatérale et lymphome non Hodgkinien.

UNLABELLED: While ocular lesion is commonly known in lymphoma, optic neuropathy is very rare : 1,3% of lymphomas affecting the central nervous systems. OBSERVATION: Authors report the case of a 75 year old patient treated in the haematology department for 8 years, for a large cell B phenotype stage IV lymphoma for which he received 7 chemotherapy courses (CHOP protocol). After a 4 year remission period, he presented a relapse with a rapid progressive bilateral impairment of visual acuity observed for a week before his admission. The ophthalmologic exam revealed no light perception and no afferent reflex on the right eye. There was light perception and weak afferent reflex on the left eye. The anterior segment was normal on both eyes and fundus examination revealed a bilateral stage I papillar oedema. The general exam showed a right facial palsy and an impairment of general condition. The orbital CT scan revealed a significant thickening of both optic nerves caused by lymphomatous infiltration. A chemotherapy with highly dosed IV and intrathecal methotrexate was performed. DISCUSSION: the optic neuropathy is usually associated with a generalized lymphoma with central nervous system involvement, but sometimes can precede the systemic spread of the disease. Apart from infiltration, the optic nerve can be compressed by an intracranial or orbital tumor. The optic neuropathy can also be caused by lymphomatous leptomeningitis.

[Fraser syndrome. A case report]. / Syndrome de Fraser.

Fraser syndrome is a rare autosomal recessive disorder; the most consistent features are cryptophthalmos, syndactyly of fingers and toes, laryngeal stenosis, and urogenital abnormalities. We report a newborn case at day 1 of life who had multiple abnormalities, born from a consanguineous marriage. Clinically, the newborn had an ankyloblepharon on the left side, a cryptophthalmos on the right side, a syndactyly, anorectal abnormalities with ambiguous genitalia, laryngeal stenosis, and ear malformations. TDM of the cranium and orbits and the transfontanel ultrasound were normal. The abdominal ultrasound showed renal abnormalities. Right eye surgery showed a reduced cornea to an opaque thin plate clinging to the iris without an anterior chamber and a nonindividualized eyeball. The authors discuss the morphological abnormalities, the clinical and paraclinical aspects of this syndrome, its multispecialized clinical management, and the importance of prenatal diagnosis.

[Apert syndrome: a reported observation]. / Syndrome d'Apert: à propos d'une observation.

Apert syndrome is a type of acrocephalosyndactylia that belongs to the group of craniofacial synostoses. It is characterised by craniofacial dysmorphia and syndactyly of hands and feet. It is an uncommon affection that is often transmitted through an autosomal dominant mode, but sporadic cases are frequent. We report the case of a 2 months old baby brought by his parents to the paediatric emergencies for respiratory distress occurring within the framework of a polymalformative syndrome. The examination showed brachycephaly, bilateral exorbitism, syndactyly of the hands and feet and an anal fistula. Echocardiographic examination showed a cardiovascular malformation (interventricular communication), the whole suggestive of Apert syndrome. The child was admitted in intensive care during five days. He died following a respiratory infection. Through this observation the authors illustrate the clinical and evolutionary aspects as well as the therapeutic difficulties of this affection.

[A case of a retinitis pigmentosa and asteroid hyalosis]. / Rétinopathie pigmentaire et hyalopathie astéroïde: à propos d'un cas.

Asteroid hyalosis is a rare degenerative condition of the vitreous. No causal relationship between retinitis pigmentosa and asteroid hyalosis has been established. We report a case of a 65-year-old male admitted for progressive hemeralopia. Visual acuities were 2/10e in the right eye and 4/10e in the left eye. Ophthalmic examination revealed a large number of asteroid and refringent bodies, a typical retinitis pigmentosa with a cystoid macular oedema confirmed by fluorescein angiography. Electrodiagnostic testing revealed an altered electroretinogram. Visual fields showed a typical generalized constriction. We discuss the clinical aspects and physiopathogenic mechanisms of this rare association through this case and other cases found in the literature.

[Ocular and systemic complications of homocystinuria: a report of five cases]. / Complications oculaires et systémiques de l'homocystinurie: à propos de cinq cas.

Homocystinuria is an autosomal recessive disorder of methionine metabolism due to cystathionine B-synthetase deficiency. It is the second most common inborn error of amino acid metabolism after phenylketonuria. In addition to the eyes, the skeletal, central nervous and vascular systems are usually affected by homocystinuria. We report two family cases of two sisters and two brothers with homocystinuria revealed by lenticular dislocation into the anterior chamber, associated with mental and growth retardation, and an isolated case of homocystinuria revealed by poor vision with Marfan syndrome and mental retardation. They all underwent surgical lensectomy with anterior vitrectomy under general anesthesia with anaesthesic precautions to prevent vascular thrombosis. Ocular complications are common in patients with homocystinuria. Treatment must include dietary changes to reduce the incidence of ectopia lentis and mental retardation. Surgical treatment with modern microsurgical techniques should be considered in advanced ocular manifestations of homocystinuria.

[Ocular impairment during type II membranoproliferative glomerulonephritis]. / Atteinte oculaire au cours de la glomérulonéphrite membrano-proliférative type II.

Membranoproliferative glomerulonephritis type II (MPGN) is characterized by dense deposits within glomerular basal membrane and Bruch's membrane which result in retinal lesions similar to drusens. We observed a 50-year-old patient with chronic renal deficiency who developed central bilateral serous retinopathy with diffuse punctiforme yellow subretinal lesions. Ophthalmoscopic and angiographic aspects led to an MPGN type II diagnosis. Specific posterior segment lesions are described during MPGN type II. Dense deposits concerned both lamina densa of glomerular basal membrane and Bruch's membrane with choriocapillaris. The main ocular complications were central serous chorioretinopathy and choroidal neovascularization. We review the clinical and evolutive aspects of this disease.

[Weill Marchesani syndrome. Report of a case]. / Le syndrome de Weill Marchesani. A propos d'une observation.

Weill Marchesani syndrome is a congenital disease that combines microspherophakia and skeletal abnormalities. The authors report a 19-year-old male, born of a consanguineous marriage, with a progressive decrease in visual acuity. The general examination showed a squat look, dwarfism, muscle hypertrophy, short hands and feet, and joint stiffness. The ophthalmological examination showed that visual acuity was limited to hand motion in the right eye despite correction and no perception of light in the left eye. Intraocular pressure was 36 mmHg in the right eye and 40 mmHg in the left eye. The anterior chamber was irregular with iridophakodonesis and microspherophakia of both lenses. The zonula was partially ruptured in the right eye. The iridocorneal angle was narrow. Fundus eye examination showed a pale optic disc with an excavation of 9/10 on the right. In the left eye, the optic disc was totally excavated. Cardiovascular check-up revealed rheumatic aortic valvular cardiopathy. The therapy consisted of combined surgery: phakophagia with anterior vitrectomy plus trabeculectomy operated on the right eye. Weill Marchesani syndrome is a rare congenital affection with a recessive autosomal transmission. The visual prognosis is dominated by secondary glaucoma due to pupillary blockage by the mobile eye lens. This observation illustrates the seriousness of spontaneous progression in Weill Marchesani syndrome, justifying the necessity of lens extraction before the onset of complications.

[Juvenile xanthogranuloma with intraocular involvement. A case report]. / Xanthogranulome juvénile avec localisation intra-oculaire. A propos dún cas.

Juvenile xanthogranuloma (JXG) is a rare and usually benign disease occurring in early childhood. It causes skin and deep seated lesions, notably in the eye. We report a case of JXG in the iris of a 9-month-old infant. Examination under general anesthesia revealed megalocornea, iris xanthogranuloma occupying the entire anterior chamber and high intraocular pressure. Skin lesions on the left lid and on the back were also found. Ocular hypertension resisted medical and surgical treatment. Cyclodestruction was necessary. The incidence of JXG is low (0.4%). The iris is the most frequently affected ocular tissue. Early diagnosis is necessary to avoid complications. Moreover, JXG can be associated in rare cases with neurofibromatosis or leukemia which must be systematically searched for.

[Cockayne's syndrome with unusual retinal involvement (report of one family)]. / Syndrome de Cockayne avec atteinte rétinienne inhabituelle (à propos j'une famille).

Cockayne's syndrome is a very rare autosomal recessive affection. Ocular involvement is an essential element for positive diagnosis; the retina shows a typical salt and pepper retinitis with optical atrophy. We report a family with four brothers who had Cockayne's syndrome with unusual retinal involvement. The patients' parents were first cousins. Ophthalmologic examination of the mother showed unilateral left pigmentary retinopathy localized in the peripapillary region. The father's ophthalmological examination was normal. The four brothers presented disharmonious dwarfism, cutaneous hyperpigmentation of skin areas exposed to sun with old-appearance of the skin, sensorineural deafness, kyphoscoliosis, a cerebellar syndrome and mental retardation. The ophthalmological examination showed hypermetropia in all four brothers and bilateral maculopathy with no papillary or vascular abnormalities. The electroretinogram was in favor of cone dystrophy. Computed tomography showed one case of calcifications of the basal ganglia and cerebral atrophy. The karyotypes of the four brothers and the mother were normal. We discuss the ocular symptoms and the etiopathogenesis of this syndrome.

[Isolated and primary palpebro-conjunctival amylosis. Apropos of 2 cases]. / Amylose palpébro-conjonctivale primitive et isolée. A propos de 2 cas.

Ocular amyloidosis a an uncommon condition and conjunctival involvement rarely occurs, representing 15% of all ocular localizations. Clinical manifestations show a polymorphism requiring a histological diagnosis. Based on two cases of primary isolated conjunctivo-palpebral amyloidosis, we discuss the different ocular sites. The first case involved false ptosis, a conjunctival infiltration and a lower eyelid tumefaction; all located in the left eye. The second case had ptosis, a right lower eyelid tumefaction with ectropion and esthetic damage. Amyloidosis was confirmed at histologic examination of the conjunctival mucous after a special congo red coloration. In order to affirm the isolated conjunctival localization, it is necessary to eliminate another amyloidis site. The treatment was surgical with excision and cure of ptosis. Ocular amyloidosis is a rare condition with polymorphism manifestations. Confirmation is histological. The greatest difficulty is management due to disease recurrence.

[Bilateral Sturge-Weber-Krabbe syndrome. Apropos of a case]. / Syndrome de Sturge-Weber-Krabbe bilatéral. A propos d'un cas.

We report a case of bilateral Sturge-Weber-Krabbe syndrome. The patient was a child aged 2 years. He presented facial and jugal angioma, bilateral glaucoma and epilepsy. The cerebral scan showed calcifications characterised by their localisations and their aspects. Cure and prognosis of this disease are discussed.

[Prevention of nosocomial urinary tract infection: vesical catheter versus Penilex]. / Prévention de l'infection urinaire nosocomiale: sondage vésical versus Pénilex.

Nosocomial urinary tract infection is the most frequent nosocomial infection in many care units. Its main risk factor is the presence of a bladder catheter. Prevention is based upon the enforcement of hygiene recommendations which are not always carefully applied everywhere. We report a prospective study concerning 328 patients divided into two gropus. Group I patients (n = 161) had an indwelling bladder catheter. Group II patients (n = 167) had an external urinary drainage by Penilex. The incidence of nosocomial urinary infection was 2.4% in group II versus 26.7% in group I. Thus preventive efficacy of Penilex use appears to be well demonstrated.