Dorfman-Chanarin Syndrome with Renal Involvement: A Rare Case Report and Literature Review.

Dorfman-Chanarin syndrome (DCS) is a rare autosomal recessive disease. It is a multisystemic disease in which renal involvement is uncommon. We report the case of a woman with nephrotic syndrome associated with DCS. A 36-year-old woman was referred to the nephrology department for edema with known history for DCS. On physical examination, she had ichthyosiform erythroderma with generalized scaly skinand ascites. The ophthalmologic examination revealed a cataract in the right eye. Abdominal ultrasound examination showed hepatomegaly and splenomegaly. Laboratory tests showed normal renal and liver function. The blood cell count showed pancytopenia. Immunologic exams showed the presence of anti-mitochondrial antibodies. Kidney biopsy showed mesangial proliferative glomerulonephritis with extensive lipid vacuoles in the tubular epithelial cells. Immunofluorescence study showed mesangial deposits of IgG, C3, kappa, and lambda. To the best of our knowledge, this is the first case of DCS with renal involvement reported in an adult.

Acinetobacter lwoffi peritonitis in peritoneal dialysis: two cases report.

Peritonitis is an important cause of morbidity and technique failure in peritoneal dialysis. Herein, we report peritonitis related to Acinetobacter lwoffi in two patients on peritoneal dialysis. The first case is a 63-year-old patient treated by automated peritoneal dialysis admitted with abdominal pain. The peritoneal effluent White Blood Cells count consisted of 280 cells/mm3. Then culture identified a multisensitive Acinetobacter lwoffi. Treatment with ceftazidime and ciprofloxacin had been started. The control dialysate culture was sterile after three weeks. The second patient is a 59-year-old female admitted because of diffuse abdominal pain and cloudy dialysate. The peritoneal effluent White Blood Cells count consisted of countless leukocytes, with predominantly polynuclear and culture identified Acinetobacter lwoffi. He received intraperitoneal ceftazidim and amikacin for three weeks. The control dialysate was sterile. Acinetobacter lwoffi is a rare cause of peritonitis and it can be treated successfully with early recognition and appropriate antibiotic therapy based on culture instead of catheter removal.

Design and Rationale of the National Observational Multicentric Tunisian Registry of Hypertension: Protocol for Evaluating Hypertensive Patient Care in Clinical Practice.

BACKGROUND: This study was designed to evaluate the care of hypertensive patients in daily clinical practice in public and private centers in all Tunisian regions. OBJECTIVE: This study will provide us an overview of hypertension (HTN) management in Tunisia and the degree of adherence of practitioners to international recommendations. METHODS: This is a national observational cross-sectional multicenter study that will include patients older than 18 years with HTN for a duration of 4 weeks, managed in the public sector from primary and secondary care centers as well as patients managed in the private sector. Every participating patient signed a consent form. The study will exclude patients undergoing dialysis. The parameters that will be evaluated are demographic and anthropometric data, lifestyle habits, blood pressure levels, lipid profiles, treatment, and adherence to treatment. The data are collected via the web interface in the Dacima Clinical Suite. RESULTS: The study began on April 15, 2019 and ended on May 15, 2019. During this period, we included 25,890 patients with HTN. Data collection involved 321 investigators from 24 Tunisian districts. The investigators were doctors working in the private and public sectors. CONCLUSIONS: Observational studies are extremely useful in improving the management of HTN in developing countries. TRIAL REGISTRATION: ClinicalTrials.gov NCT04013503; https://clinicaltrials.gov/ct2/show/NCT04013503. INTERNATIONAL REGISTERED REPORT IDENTIFIER (IRRID): DERR1-10.2196/21878.

[Cutaneous leishmaniasis after renal transplantation: Report of 7 cases and review of the literature]. / Leishmaniose cutanée après transplantation rénale : à propos de 7 cas et revue de la littérature.

Leishmaniasis is a zoonosis acquired from the bite of a sandfly that introduces the amastigote forms of leishmania into the bloodstream. It is a frequent infection in the countries of the Mediterranean basin and in Tunisia, where it is rife in an endemo-epidemic mode. However, it is rare after kidney transplantation. It constitutes a challenge due to the diagnostic difficulty, the variability and the polymorphism of the clinical picture in immunocompromised patients. We report seven observations of cutaneous leishmaniasis after kidney transplantation through which we try to identify diagnostic and therapeutic difficulties.

Epidemiologic features and management of hypertension in Tunisia, the results from the Hypertension National Registry (NaTuRe HTN).

BACKGROUND: Hypertension is the leading cause of morbi-mortality in our country. Thus, we conducted this national survey on hypertension to analyze the profile of the Tunisian hypertensive patient and to assess the level of blood pressure control. METHODS: Nature HTN is an observational multicentric survey, including hypertensive individuals and consulting their doctors during the period of the study. Blood pressure measurements were conducted during consultation, using a standardized auscultatory or oscillometric sphygmomanometer after at least 15 min of rest. The diagnosis of new hypertension is based on the 2018 ESC/ESH criteria. The primary endpoint of our study was uncontrolled hypertension defined by a systolic blood pressure ≥ 140 mmHg and/or diastolic blood pressure ≥ 90 mmHg. RESULTS: Three hundred twenty-one investigators participated in the study. We enrolled 25,890 patients with a female predominance (Sex ratio, 1.21) and an average age of 64.4 ± 12.2 years. Most individuals were treated in the public sector (74%), 39.4% of patients were diabetic, 25.8% were obese, 44.6% were overweight and 14% were smokers. Hypertension was controlled in 51.7% of cases if we consider 140/90 as a BP target, and only in 18.6% if we consider 130/80 as a target. The independent predictors of uncontrolled blood pressure were male sex (OR = 1.09, 95%CI [1.02-1.16]), age > 65 year-old (OR = 1.07, 95% CI[1.01-1.13], diabetes (OR = 1.18, 95% CI [1.11-1.25], Smoking (OR = 1.15, 95% CI [1.05-1.25]), Obesity (OR = 1.14, 95% CI[1.07-1.21]), management in public sector (OR = 1.25, 95% CI [1.16-1.34]), and Heart rate > 80 bpm (OR = 1.59, 95% CI [1.48-1.71]). Contrarily, high educational level (OR = 0.9, 95% CI [0.84-0.97], absence of history of coronary disease (OR = 0.86, 95% CI [0.8-0.93]), salt restriction (OR = 0.48, 95% CI [0.45-0.51]), drug compliance (OR = 0.57, 95% CI[0.52-0.61]), and regular physical activity (OR = 0.77, 95% CI[0.71-0.84]) are strong predictors of blood pressure control. CONCLUSION: NaTuRe HTN showed that blood pressure control was reached in more than half of the Tunisian people. The control remains low in patients with high cardiovascular profiles and in those treated in the public sector. A national health program based on therapeutic education, regular control and continuous support to the public institutions is needed to decrease the burden of hypertension incidence rate.

Bone marrow aspirate: a diagnostic tool for primary hyperoxaluria type 1.

INTRODUCTION: Type1 primary hyperoxaluria is an infrequent autosomal recessive metabolic disease characterized by the accumulation of calcium oxalate in the kidney, which leads to end stage renal disease. In fact, the diagnosis of this disease is mandatory in order to avoid graft loss. AIM: To assess the incidence of primary hyperoxaluria and to develop the diagnostic value of bone marrow infiltration by calcium oxalate in uremic stone former patients. METHODS: This study was conducted on a cohort of stone former patients identified in the south of Tunisia over a period of 18 years. Baseline characteristics were recorded. Clinical and laboratory data were collected on chart review. Secondary forms of hyperoxaluria were excluded. Bone marrow aspirate was performed in uremic patients from this cohort because early hyperoxaluria was suspected. Diagnostic accuracy of this test relating to sensitivity, specificity, positive predictive value and negative predictive value were also calculated. RESULTS: A cohort of 31 patients comprising 17 male patients and 14 female patients were identified. During this time of diagnosis, the patient's ages ranged from 9 to 57 years old and 22 of them (70%) unfortunately died. Bone marrow aspirate was safely done in 16 uremic patients. It was positive in 12 patients and negative in 4 patients. Sensitivity, specificity, positive predictive value and negative predictive value were respectively 85%, 100%, 100% and 50%. CONCLUSION: The bone marrow examination represents both an easy and a worldwide feasible solution for the diagnosis of oxalosis, which affords an early diagnosis.

Primary membranoproliferative glomerulonephritis in Sfax, Tunisia: epidemiologic profile and prognostic factors.

INTRODUCTION: membranoproliferative glomerulo nephritis (MPGN) is a rare kidney disease with a poor prognosis as 50% of patients attend the end stage renal failure after 10 years of follow up. Several factors have been described associated with poor renal prognosis. The aim of our study is to determine the epidemiologic profile and to identify prognostic factors of MPGN. METHODS: our study is retrospective over a period of 16 years (January 1996 - December 2011) including all cases of primary MPGN aged more than 15 years, collected at the nephrology department of Hedi Chaker University Hospital, Sfax, Tunisia. RESULTS: we collected 118 cases of primary MPGN, with mean age of 45 (SD 19) years. The incidence of MPGN has decreased from 10 cases/year between 1996 and 1999 to 5 cases/year between 2008 and 2011. Seventy-nine percent of patients (n=93) had renal failure at the moment of diagnosis (e-GFR less than 60 ml/min/1.73m2;). After a mean follow-up of 51.9 (SD 44) months, progression to end stage renal failure was observed in 43.5% of followed cases (n=20). On univariate analysis, factors associated with death or progression to end stage renal failure were initial renal failure and sclerotic glomeruli (respectively p at 0.040 and 0.032). Multivariate analysis indicated that initial renal failure was significantly correlated with death or progression to end stage renal failure (HR: 0.14, 95% CI (0.033-0.593), p=0.008). CONCLUSION: there has been a decline in the number of cases of MPGN diagnosed in our hospital. The presence of renal failure at diagnosis was associated with death or progression to end stage renal failure.

[Pregnancy in patients on chronic haemodialysis: about 25 cases which occurred in the south of Tunisia]. / La grossesse en hémodialyse chronique: à propos de 25 cas survenus dans le Sud Tunisien.

INTRODUCTION: the occurrence of pregnancy in patients on chronic haemodialysis is rare. However, given the evolution in dialysis technique, improvement in fertility is possible. The purpose of our study was to report our experience concerning the occurrence of pregnancy in patients on dialysis and to identify factors involved in its success. METHODS: we conducted a retrospective study on 25 spontaneous pregnancies occurred in 19 patients treated with periodical hemodialysis in different hemodialysis centers in the south of Tunisia over a period of 34 years. RESULTS: maternal age at the onset of pregnancy was, on average, 35.6 years [23-44 years] with an average seniority in hemodialysis of 4.22 years [1-17 years]. Seven patients (37%) had residual diuresis (>500 ml/24h). The prescribed weekly number of hours of dialysis was ≥16 hours per week in 7 cases and ≥20 hours in 4 cases. Success of pregnancy (new-born surviving at least 28 days) was estimated at 56%. The median gestational age was 34 weeks of amenorrhea [28-38 WA]. The average neonatal weight was equal to 1970g [1500g-2300g]. Analytical study showed a significant correlation between the increase in the hours of dialysis per week and the success of pregnancy (R=0.59; p=0.002). CONCLUSION: it was noted that with adequate support and in particular, increasing the number of sessions of dialysis, materno-fetal complications can be minimized and the balance risk-benefit can turn the chance for a woman on dyalisis to become pregnant.