Childhood discoid lupus erythematosus: a Tunisian retrospective study of 16 cases

Discoid lupus erythematosus (DLE) is uncommon in children. The clinical features of childhood DLE are similar to those of adult DLE in presentation and chronic course. However, children have a particularly high level of transition to systemic disease. We undertook a retrospective study of 16 children with DLE ranging in age from 2 to 15 years, seen over a 9-year period. Six were less than 10 years old at the onset of the disease. The sex ratio was equal. The frequency of childhood DLE was about 7% of the total number of DLE patients seen in our department. Photosensitivity was defined as a clinical history of induction or exacerbation of discoid lesions following sun exposure, and was present in 81% of patients. There was no progression to systemic lupus erythematosus (SLE); an average follow-up time was 10.5 months (2-30 months). We would like to emphasize the increased frequency of childhood DLE in our country and the importance of photosensitivity. However, follow-up data regarding transition to SLE is lacking, therefore we are unable to offer a prognosis to our patients.     

Combined aplasia of maxillary first molars and lateral incisors: a case report and management

Congenital absence of teeth, as the most commonly known developmental dental anomaly in man, has a multitude of adverse affects that could be detrimental to normal function and esthetics. This report presents a rare case of combined agenecies of maxillary permanent first molars and lateral incisors. The management plans are highlighted, and phase I orthodontic-restorative treatment mechanics and its result are described. The benefits for early orthodontic treatment intervention for this case presented here are discussed.     

Impact of treatment of dyslipidemia on renal function,fat deposits and scarring in patients with persistent nephrotic syndrome

In this study 43 patients with idiopathic nephrotic syndrome were randomly distributed into 2 age- and sex-matched groups. The first group was given fluvastatin while the second was used as control. The cases in the 2 groups were evaluated clinically, biochemically (creatinine clearance, albumin, 24-hour proteinuria, and lipogram), neurologically, and histopathologically (examination of renal biopsies obtained basally and after 1 year of treatment with fluvastatin). In the fluvastatin-treated group but not in the control group, we observed a significant reduction in cholesterol, low-density lipoprotein, and triglyceride. Clinical and laboratory assessment showed satisfactory tolerance of the drug by the patients. Proteinuria, serum albumin and creatinine clearance values were significantly better in the statin-treated patients. There was no difference in glomerular sclerosis between the 2 groups while interstitial fibrosis and renal fat deposits were less in the statin-treated group. The reduction in renal fat deposits in the statin-treated group was highly significant, while that of interstitial fibrosis was not. We conclude that: (1) statin can be safely and effectively used in the treatment of dyslipidemia in patients with persistent idiopathic nephrotic syndrome; (2) control of dyslipidemia in nephrotic patients is associated with better control of proteinuria and creatinine clearance; (3) statin treatment may cause regression of renal fat deposits in patients with nephrotic syndrome, and (4) longer term studies are still required to study further possible beneficial effects on renal histology and disease progression.     

Liver transplantation for Budd-Chiari syndrome

BACKGROUND: Budd-Chiari syndrome (BCS) is a clinical condition characterized by hepatic venous outflow obstruction secondary to an underlying systemic predisposition to thrombosis. METHODS: We reviewed our experience of 19 adult patients who underwent orthotopic liver transplantation for BCS from April 1988 to May 1999 to assess their long-term outcome and specific complications related to this procedure. RESULTS: Of these patients, 13 presented with chronic and 6 with acute liver failure. At presentation predisposing factors included polycythemia rubra vera in five, an undefined myeloproliferative disorder in four, essential thrombocythemia in two, presence of lupus anticoagulant in one, antiphospholipid antibody positivity in one, post-gestational in one, oral contraceptive pill in one, and idiopathic in four. Five patients had undergone previous porto-systemic shunt. Of the 19 patients, 16 are alive at a median follow-up of 89 months (range 1-119) with 2 patients developing disease recurrence at 4 months and 7 years posttransplant, respectively. Four patients have been retransplanted: one for progressive graft dysfunction due to nodular regenerative hyperplasia secondary to azathioprine toxicity, two for hepatic artery thrombosis (one soon after and the other 47 months posttransplant), and one for recurrent BCS. Three patients have died: one from an intra-abdominal bleed secondary to acute hemorrhagic pancreatitis 8 years posttransplant, another from acute myeloid leukemia at 6 years posttransplant, and the third patient from graft failure secondary to severe rejection 1 month posttransplant. CONCLUSION: Liver transplantation for BCS provides good long-term survival with acceptable morbidity. Long-term survival may be prejudiced by progression of the underlying hematological disorders.     

Regulatory functions of alloreactive Th2 clones in human renal transplant recipients

BACKGROUND: Chronic allograft rejection is the major clinical problem in organ transplantation. There is evidence that indirect T cell recognition of donor-specific HLA peptides may play an important role in the immunopathogenesis of chronic allograft rejection. We have recently shown that HLA allopeptide-specific T cell clones generated from renal transplant recipients with chronic allograft nephropathy are of the Th1 phenotype, while those from stable patients are Th2. There is evidence in experimental animal models of autoimmunity and transplantation that Th2 cells may function to regulate immune responses, but the biological relevance of these observations in humans has not been reported. METHODS: The purpose of this study was to investigate the putative regulatory functions of alloreactive human Th2 clones. HLA-DR allopeptide-specific Th1 and Th2 cell clones were generated from peripheral blood lymphocytes of human renal allograft recipients with chronic allograft nephropathy (CAN) or with stable renal function (SRF), respectively. RESULTS: An in vitro co-culture system showed that the proliferative responses of Th1 clones from patients with CAN were significantly inhibited by the Th2 clones in response to the donor-derived HLA allopeptides. In addition, co-culture of the Th2 clones inhibited cytokine production (IFN-gamma) by the Th1 clones in response to the donor-specific peptides. The regulatory functions of Th2 clones were antigen-specific since they only occurred when both the Th1 and Th2 clones were reactive to the same HLA-DR allopeptide, and were mediated by IL-4 and IL-10. CONCLUSIONS: This is the first demonstration, to our knowledge, indicating that Th2 cells may function to regulate indirect Th1 alloimmune responses that are critical for the progression of CAN in humans.     

Mycophenolate mofetil treatment for primary glomerular diseases

BACKGROUND: Treatment of primary glomerular diseases may be unsuccessful or have potential toxicities. Therefore, we evaluated the use of mycophenolate mofetil (MMF) for empirical treatment of primary glomerulopathies. METHODS: Forty-six patients with biopsy-proven primary glomerulopathies received MMF for > or =3 months as adjunctive or primary treatment. Median (range) 24-hour urine protein to creatinine ratio (Up/c) and serum creatinine at the start and end of MMF therapy were compared using the Wilcoxon signed-ranks test. RESULTS: Overall, the median Up/c decreased from 4.7 (range <0.1, 20.3) to 1.1 (<0.1, 14.3; P < 0.001) at the end of MMF treatment with no significant change in median serum creatinine 1.3 (0.6 to 6.1) to 1.2 (0.5 to 6.5) mg/dL. Median serum albumin increased from 3.4 (1.4, 4.6) to 4.1 (1.7, 48) g/dL (P < 0.001) and the median serum cholesterol decreased from 270 (148, 795) to 220 (140, 309) mg/dL (P < 0.001) post-treatment. For those with minimal change disease, a complete steroid withdrawal was accomplished in 5/6 steroid dependent patients. Focal segmental glomerulosclerosis (FSGS) patients had a median Up/c that decreased from 2.7 (0.1, 20.3) to 0.8 (<0.1, 8.2; P = 0.001) in 18 patients. In membranous nephropathy (MN) patients, the median Up/c decreased from 7.3 (0.1, 18.5) to 1.5 (<0.1, 14.3) (P = 0.001) in 17 patients. No significant change in median serum creatinine was detected in FSGS or MN patient groups during treatment. CONCLUSIONS: Empirical MMF therapy in the majority of patients with primary glomerulopathies was well tolerated and achieved the goals of steroid withdrawal, improvement of nephrotic syndrome, and stabilization of renal function.     

Hydronephrosis arterial hypertension. Report of 4 cases

Four cases of ureteropelvic junction syndrome associated with arterial hypertension were reported. The authors discuss the ethiopathogenic of hypertension, on the basis of these observations and a review of the literature. They conclude that union bilateral hydronephrosis can lead to hypertension and renal failure by both inappropriate production of renin and water chronic retention. The correction of ureteropelvic junction should return blood pressure to normal levels.     

Bilateral malignant non-Hodgkin's lymphoma

Non Hodgkin's lymphoma (NHL) of the testis in young patient are rare. Bilateral involvement varies from 5 to 20% of cases and has a negative prognosis. The authors report a case of bilateral non Hodgkin's lymphoma of the testis in a 35 year old man. The patient died six months after the diagnosis.     

Presacral fibroma in a young laborer presenting with chronic lumbago

A 24-year-old patient had a long history of low backache. After examination he was found to have a presacral mass grossly displacing the rectum anteriorly. Surgical exploration revealed a large rounded mass (20x17x10cm) occupying the presacral space and adherent to the sacral promontory. The mass was totally excised. The histology was a benign fibroma. The patient persued an excellent post-operative course and has remained symptom free for one year now.