[Refractory Sweet syndrome complicated by monoclonal gammopathy of undetermined significance]. / Syndrome de Sweet récidivant compliqué de gammapathie monoclonale de signification indéterminée.

The association between Sweet syndrome and monoclonal gammopathy of undetermined significance (MGUS) is exceptional. We report the case of a 44 years-old woman in whom recurrent Sweet syndrome was complicated by monoclonal gammopathy of undetermined significance (MGUS) after 3 years of evolution.

L'association entre syndrome de Sweet et gammapathies monoclonales de signification indéterminée (MGUS) est exceptionnelle. Nous rapportons l'observation d'une patiente âgée de 44 ans chez qui un syndrome de Sweet d'évolution récurrente, s'est compliqué par l'apparition d'une gammapathie monoclonale de signification indéterminé après 3 ans d'évolution.

[Lupus and pregnancy: study of 26 cases, an internal medicine department experience and review of the literature]. / Lupus et grossesse : expérience d'un service de médecine interne à propos de 26 cas et revue de la littérature.

OBJECTIVE: To study the maternal and fetal outcomes in women with systemic lupus erythematosus. PATIENTS AND METHODS: A retrospective study of 26 pregnancies in 15 systemic erythematosus patients diagnosed before or during pregnancy regarding to American College of Rheumatology criteria in a single reference center. RESULTS: The mean patient age was 31.52 years (24-39 years). The mean interval from the diagnosis of the systemic lupus erythematosus to pregnancy was 4.2 years. Eight pregnancies were planned. The flare rate of lupus during pregnancy was 31%, life birth rate was 65% and fetal loss rate was 35%. DISCUSSION AND CONCLUSION: As an increase in disease activity can occur during pregnancy and because of a higher rate of obstetrical complications in patients with lupus, it is important to carefully plan pregnancy. Pregnancy in lupus patients must be closely monitored by a multispeciality care of the patients.

[Late revelation of homocysteinuria: clinical, biological and progressive aspects]. / Homocystéinurie à révélation tardive : aspect clinique, biologique et évolutif.

UNLABELLED: Homocysteinuria is a metabolic disorder with defect in genes encoding for methionine metabolism enzymes. The clinical features consist in: ophthalmic, neurological, orthopedic and vascular manifestations. It is generally diagnosed in childhood. Vascular involvements characterize adult's forms. We report one case. OBSERVATION: A 26-year-old man, who has lentis ectopia and a recent epilepsy, was hospitalized for deep vein thrombosis. Regarding the marfanoid phenotype and the high level homocysteinemia (231 micromol/L), homocysteinuria was suspected. Amino acid chromatography and reduced CBS activity were used to confirm the diagnosis. Vitamin enriched diet with vitamin B6 and folates has reduced slightly the homocysteine level. CONCLUSION: Homocysteinuria must be diagnosed early since a simple vitamin supply could ameliorate prognosis and decrease complications.

[Imaging in the screening and diagnosis of hepatocellular carcinoma in cirrhosis liver in Tunisia. A series of 30 cases]. / L'imagerie dans le dépistage et le diagnostic du carcinome hépatocellulaire sur foie de cirrhose en Tunisie. A propos de 30 cas.

UNLABELLED: The aim of our study is to expose a practical screening and diagnosis strategy of HCC occuring in cirrhosis liver, which is nowadays a health public matter in Tunisia. METHODS: HCC routine screening in cirrhosis liver has been conducted every 6 months using a combination of abdominal sonography and serum alpha-foetoprotein levels. PCUS, CT scan or MRI have been performed each time a focal liver lesion was discovered in US, or in case of elevated serum alpha-foetoprotein levels. 30 cases of HCC were identified. RESULTS: US has shown a unique nodule in 46% of the cases, multiple nodules in 50% of them. No lesion has been discovered in 4% of the cases. Nodule size ranged from 8 to 140 mm. Heterogenous hyperechoic pattern was the most frequent one (41%). Hypervascular doppler feature was authentified in 20% of the cases. Serum alpha-foetoprotein levels had respectively a sensitivity and a specificity of 14% and 44% in lesions inferior to 3 cm, 62% and 80% in lesions superior to 3 cm. Typical arterial enhancement of HCC was depicted in 1 case for PCUS, in 76% for CT scan and 60% for MRI. CONCLUSION: Combination of abdominal sonography and serum alpha-foetoprotein levels practiced every 6 months represent a good compromise between cost and effectiveness. Second intention imaging is based on CT scan in Tunisia because of its availability compared to MRI. PCUS is becoming in the near future the best modality for lesion caracterization thanks to its safety and relative inexpensiveness.

[Vertebral collapse revealing primary hyperparathyroidism in a 14-year-old girl]. / Tassement vertébral révélant une hyperparathyroïdie primaire chez une adolescente.

Primary hyperparathyroidism, widely prevalent in women after menopause, remains rare in children and adolescents. Sporadic forms are the most frequent. Clinical manifestations are general, renal, gastrointestinal, cardiac, or bony. Diagnosis is biological and radiological. The imaging modalities allow assessment of the disease impact and identification of the parathyroid adenoma.

[Secondary TFE3-associated renal cell carcinoma in a child treated for Ewing sarcoma]. / Carcinome à cellules claires du rein et sarcome d'Ewing pelvien : une association rare chez l'enfant en rapport avec la translocation TFE3 Xp11.2.

Renal cell carcinoma is a rare pediatric malignant tumor of the kidney. Unlike Wilms tumor, the efficacy of chemotherapy and radiation therapy in pediatric renal cell carcinoma remains uncertain. Surgery is the best treatment and prognosis is favorable when the tumor is localized and completely eradicated. We report an exceptional observation in a 7-year-old girl with renal cell carcinoma who had been treated 20 months previously for Ewing sarcoma with chemotherapy and radiotherapy. The renal tumor was revealed by abdominal pain without hematuria. She underwent a radical nephrectomy, and histopathology concluded in renal carcinoma associated with translocation Xp 11.2 grade 3 of Furhrman pT3a N1. No adjuvant therapy was given. After 3 years of follow-up, there is no evidence of local or metastatic recurrence. This observation is significant given the very young age of this patient, the occurrence after Ewing sarcoma with a short disease-free interval. It seems that translocation renal cell carcinoma is associated with previous exposure to chemotherapy, particularly topoisomerase II inhibitors or alkylating agents.

[Cryoglobulinemia in a Tunisian internal medicine department]. / Cryoglobulinémie : expérience dans un service de médecine interne tunisien.

AIM: Cryoglobulinemia is characterized by multiple organ involvement, mainly including the skin, liver, kidneys, and peripheral nerves. Our aim was to investigate the demographic, clinical, and serologic features, as well as survival in a group of 16 Tunisian patients with cryoglobulinemia. RESULTS: The study included 12 women and 4 men, and their mean age was 41 years. In all but two, the cryoglobulinemia was associated with another disease. These included lupus for 9, Sjögren syndrome for 2, and polyarteritis nodosa for one. They also included infectious diseases: 3 patients with hepatitis B virus infection, one with hepatitis C virus infection, one with parvovirus B19, and another with lymph node tuberculosis. Only one case of lymphoproliferative disease was noted. General symptoms were present in 81% of the patients, cutaneous vasculitis in 43%, peripheral vascular-Raynaud phenomenon in 37%, joint polyarthralgia or arthritis in 62%, renal involvement in 68%, neuropathy in 25%, lung involvement in 56%, gastrointestinal involvement in 37%, and finally cardiac involvement in 31%. In some cases it was difficult to determine if the clinical signs were attributable to cryoglobulinemia or the underlying pathology. The course was favorable under treatment for 5 patients, while 7 patients became sicker and 5 finally died. CONCLUSION: Cryoglobulinemia is underdiagnosed. Treatment depends on the severity of the lesions and the underlying disease.

[Study of malabsorption of lactose by the hydrogen breath test in a population of 70 Tunisian adults]. / Etude de la malabsorption du lactose par le test respiratoire à l'hydrogène dans une population de 70 adultes tunisiens.

Lactose malabsorption was studied by the hydrogen breath-test in 23 adults suffering from irritable bowel syndrome (group A) and in 47 healthy subjects (group B). The concentration of hydrogen in end-expired alveolar samples was measured after ingestion of 25 g of lactose. Among the 70 subjects, 6 (8.5 p. 100) were not hydrogen producers and were excluded from the study. Lactose malabsorption was shown in 51 of the remaining 64 subjects (79.6 p. 100). Among these 51 patients, 36 were healthy and 15 had an irritable bowel syndrome. The frequency of lactose malabsorption among the 43 healthy hydrogen producers was 83 p. 100. This value is similar to those observed in other studies (greek and italian). Our results suggest that lactose malabsorption is frequent among the tunisian adult population.

[For a better estimation of the prevalence of intestinal parasitism in the Tunis region]. / Pour une meilleure estimation de la prévalence du parasitisme intestinal dans la région de Tunis.

The prevalence of intestinal parasitism is generally based on the results of a single stool specimen which probably underestimates the real situation. In order to propose a coefficient for correction, we examined three stool specimens taken from 112 asymptomatic children. Intestinal parasites other than Enterobius vermicularis were detected in 29 subjects (26%). For those specimens that tested positive, 41% of children had infection detected in all three stool specimens; 21%--in two specimens and 38%--in only one. If we assume that the sensitivity of three stool specimen examinations is 100%, then the calculated sensitivity of one examination is equivalent to 68%. This gives an underestimation of the prevalence of 32%. This underestimation is not homogenous for all species. As regards Giardia intestinalis it is 35%, but for other species it would have to be calculated from a larger sample.

[Value of extracorporeal shockwave lithotripsy in primary hyperoxaluria type I]. / Intérêt de la lithotritie extracorporelle dans l'hyperoxalurie primaire de type I.

BACKGROUND: Application of extracoporal shock wave lithotripsy (ESWL) has revolutionized the management of many types of urolithiasis, including that observed in primary hyperoxaluria where surgical attempts to remove calculi sometimes result in worsening of renal function. CASE REPORTS: Three unrelated patients aged 8, 10 and 11 years, respectively, with type I primary hyperoxaluria, suffered from recurrent bouts of abdominal pain (two patients) or anemia (one patient). Two patients had chronic renal failure. Plain abdominal films and ultrasonography showed several large bilateral stones. ESWL was applied to the three patients permitting complete removal of stones following three sessions in one patient and partial removal following four sessions in the second patient who developed infection after the first session with transitory increase in creatinemia. Several sessions of ESWL failed to fragment stones in the third patient. CONCLUSION: ESWL may represent a safe procedure for attempting stone removal in patients with primary hyperoxaluria.

[Cerebral hydatic cysts in children]. / Les kystes hydatiques cérébraux chez l'enfant.

BACKGROUND: Brain cysts caused by Echinococcus granulosus are rare; they occur during childhood in endemic areas. PATIENTS: Six children, aged to 8.5-years old (mean age: 5.5 years) were admitted from 1989 to 1994 because they suffered from progressive intracranial hypertension. The brain CT-scan showed typical features of supratentorial, unilocular (four cases) or multilocular (two cases) cysts. Three children had positive serological test; two had another cyst into their lung or liver and the third had generalized cysts. This last patient died post-operatively while the five others recovered after excision of cysts. CONCLUSION: Any progressive intracranial hypertension in endemic areas requires brain CT-scan that clearly identifies the cyst and its location.

[Aspects of evolving urogenital tuberculosis. 60 cases]. / Les aspects évolutifs de la tuberculose urogénitale. A propos de 60 cas.

Based on the results of a retrospective study of 60 patients treated between January 1984 and December 1994, the authors review the current clinical features of urogenital tuberculosis. The sex-ratio shows a male predominance of 65%. The mean age is 48 years. Cystitis and low back pain are the main symptoms of the disease. IVU, performed in all patients, showed pathological images in 98.3% of cases. Urine culture was positive in 23 out of the 50 cases in which it was performed. Serious forms were relatively frequent, with bilateral lesions in 12 cases, associated with renal failure in 4 of them, or unilateral renal destruction in 43 cases. All patients were treated with triple- or quadruple-agent tuberculostatic therapy. 54 patients required one or several surgical procedures: 43 nephrectomies, 7 ureterovesical reimplantations, 11 augmentation enterocystoplasties, 1 Bricker ureteroileostomy, 3 ureterostomies, 1 ureteric resection and 4 epididymectomies. Two patients admitted in a context of deterioration of the general state died during the days following admission. 56 patients were reviewed with a minimum follow-up of 6 months. Two were cured by medical treatment alone. Among the 54 operated patients, clinical improvement was obtained in 43 patients (91%) and radiological improvement was obtained in 33 of the 36 cases in which IVU was performed (91%). In conclusion, despite the various anti-tuberculosis campaigns, urogenital tuberculosis remains a serious disease, essentially because of the delayed diagnosis.

[Extra-adrenal pheochromocytoma: report of 4 cases]. / Les localisations extrasurrénaliennes du phéochromocytome: à propos de quatre cas.

Extra-adrenal pheochromocytoma is rare. The diagnosis must be evoked in signs of pheochromocytoma with absence of tumor of the adrenal. We report four cases of ectopic pheochromocytoma while specifying their clinical particularities and diagnosis procedure.

[The treatment of ureteroceles complicated by calculi]. / Traitement des urétérocèles compliquées de lithiase.

In a series of 13 adults with calculi in ureteroceles, 10 cases underwent endoscopic meatotomy associated with endoscopic lithotripsy. In 2 bulky ureteroceles with calculi, the authors performed excision of the ureterocele and reimplantation of the ureter. In one case, the authors successfully performed extracorporeal lithotripsy. The authors obtained good results in all cases. Endoscopic meatotomy with lithotripsy is a very useful procedure for treatment of small or medium sized ureteroceles with calculi in adults.

[Giant retroperitoneal liposarcoma: report of a case]. / Le liposarcome rétropéritonéal géant: à propos d'un cas.

Liposarcoma is a rare and primary malignancy developed from mesenchymal tissue. We report the case of a 32-year-old woman who had a complete exeresis of a 15 cm retroperitoneal myxoid liposarcoma. Three months later, a 9 cm local recurrence was excised and radiotherapy was performed. In 2000, a local recurrence and an intestinal localisation of the liposarcoma were observed. The retroperitoneal tumor could not be removed and she had an intestinal resection followed by chemotherapy. With the literature data, we review the pathological, therapeutic and prognostic aspects of this tumor.

[Diagnostic and therapeutic advantages of rigid ureteroscopy: retrospective study of 304 cases]. / Apport diagnostique et thérapeutique de l'urétéroscopie rigide: étude rétrospective à propos de 304 cas.

Uretroscopy has become a common tool for the diagnosis and management of the upper urinary tract. Between April 1994 and Decembre 1999, 304 ureteroscopy procedures were performed. The most common therapeutic procedure was stone manipulation in 299 cases (distal ureter 259 cases, mid-ureter 23 cases, lumber ureter eight cases and staged in nine cases). Procedure was made by a rigid ureteroscope 8.5 Ch or 11.5 Ch. Dorinia was used in 175 cases (57.5%) with or without fragmentation by ultrasound. Therapeutic ureteroscopy was also used to remove a double-J stent that had migrated into the pelvic ureter in one case and in three cases of ureteral tumor. It was used in one case to confine the extrinsic compression of a ureteral stenosis. Median follow up was 12 months (6-36 months). The overall success rate was 87.6%. Results are comparable in men and women (P = 0.8). Success rate was 90% in single lithiasis and 76.2% in case of numerous stones (P = 0.0094). There was a statistically significant difference in success rate between stones 15 mm or under in size (89% stone-free) and those 15 mm (66% stone-free) or larger. In conclusion, rigid ureteroscopy is a minimally invasive and reliable technique for the management of ureteric calculi and for the diagnosis and treatment of other ureteric lesion.

[The etiology of hypercalcemia: a study of 47 cases in a hospital internal medicine service]. / Les etiologies de l'hypercalcemie: etude a propos de 47 cas dans un service de medecine interne.

In this study, we are presented the clinical and the aetiologic features of 47 patients having hypercalcemia (total serum calcium > or = 2.7 mmol/l). Our results show a positive significantly correlation between the degree of the hypercalcemia and the severity of the clinical symptomatology. As for to the aetiology, the multiple myeloma, the hyperparathyroid and the neoplastic process constituted 78% of the total aetiology.

[Tracheal atresia. A case report]. / Atresie tracheale. A propos d'un cas.

The tracheal agenesis is a rare malformation of the respiratory tract. It must be suspected in any new born with respiratory distress, absence of crying, and difficulty or impossibility of intubation. Since the initial case report by Payne in 1900, 87 cases have been reported in the literature. The authors report one case of tracheal agenesis out of 2500 autopsy realised in the laboratory.

[Xanthogranulomatous pyelonephritis in pelvic kidney. A case report]. / La pyelonephrite xanthogranulomateuse sur un rein pelvien. A propos d'un cas.

We report the case of a xanthogranulomatous pyelonephretis in a pelvic ectopic kidney that is revealed by a buttockal abscess.