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S Turki

Publications and source records attributed to S Turki.

At least 19 recordsLinked to original sources

FIP1L1-PDGFRA positive chronic eosinophilic leukemia in Tunisian patients.

Hypereosinophilic syndromes (HES) are a heterogenous group of rare disorders characterized by sustained and otherwise unexplained overproduction of eosinophils with organ involvement and consecutive dysfunction. Detection of the FIP1L1-PDGFRA fusion gene or the corresponding cryptic 4q12 deletion in HES supports the diagnosis of chronic eosinophilic leukemia (CEL) and provides a molecular explanation for the pathogenesis of this disorder. We screened seven Tunisian patients fulfilling the WHO criteria of HES for the presence of the FIP1L1-PDGFRA fusion gene using nested reverse transcription polymerase chain reaction on peripheral blood samples. Four of the seven patients were positive for this fusion gene. Sequence analysis revealed a substantial heterogeneity of the fusion transcripts due to the involvement of several FIP1L1 exons. All patients were male. The median age at diagnosis was 24 years (range, 18-50); one patient had a history of hypereosinophilia of more than 10 years. Two patients had clinically important and symptomatic eosinophilic endomyocardial disease with thrombotic events. Splenomegaly was constant in FIP1L1-PDGFRA positive CEL but not in the other HES patients (only 1/3).

Adolescent↗

[Retroperitoneal fibrosis].

OBJECTIVE: We studied the clinical, therapeutic and progressive characteristics of retroperitoneal fibrosis. METHODS: We analysed the observations of retroperitoneal fibrosis diagnosed between 1980 and 2002 in our hospital, from the summaries of 15 patients exhibiting retroperitoneal fibrosis (RPF). Therapeutical supervision was based on biology and radiology. RESULTS: There were 11 men and 4 women with a mean age of 44.5 years ranging from 28-64 years. Pain was predominantly lumbar or abdominal in all patients. An inflammatory syndrome was observed in all patients and renal failure in 11. Radiological examinations revealed single or bilateral in 14 cases and the fibrosis plaque in 13 cases. Treatment consisted in corticosteroids alone in 9 patients, surgery alone in 3 cases and surgery with corticosteroids in 3 patients. Ten relapses (range: 1-5) occurred in 4 patients when corticosteroids were stopped. After a mean follow-up of 36 months (range:18 days-11 years), one death was observed, 12 patients had normal renal function and 2 patients had persistent moderate renal failure. CONCLUSION: This study confirms the rarity of retroperitoneal fibrosis, the difficulty in its diagnosis, the frequency of pain, inflammatory syndrome and renal failure. Corticosteroids are efficient and regular follow-up is required.

Abdominal Pain↗

[Etiologic and therapeutic aspects of acquired central diabetes insipidus].

Acquired central diabetes insipidus (CDI) is a rare disease due to anatomic lesions of the hypothalamo-pituitary system. We discuss the etiologic and therapeutic aspects of CDI. Through 5 cases and a review of the literature. We report: Two cases of Langerhans histiocytosis, a 21 year old man and a 37 year old women. The CDI was the only endocrine manifestation in the man, but it was associated with panhypopituitarism and infiltration of the thyroid gland by histiocytosis cells in the women. One case of suprasellar germinoma, a 18 year old adolescent had hypocorticism, hypothyroidism, and hypogonadism associated with CDI and hyperprolactinemia. One case of pituitary cystic lesion with extension to the suprasellar area, a 36 year old women presenting with hypocorticism, growth hormone deficiency, and hyperprolactinemia. One case of neuro-Behçet's disease in a 47 year old man. He presented with strokes and CDI. When the CDI is isolated Langerhans Histiocytosis should be considered first. The exploration of the hypothalamo-pituitary region using MRI showed different anatomic lesions in patients with CDI. It is very difficult to determine definitive diagnosis before surgery in the cases of cystic lesions.

Adolescent↗

[Abdominal polyarteritis nodosa: angiographic features].

PURPOSE: Polyarteritis nodosa (PAN) is a systemic vasculitis of small and medium size arteries. The purpose of this study is to evaluate imaging findings, especially angiographic features, of 17 patients with abdominal involvement from polyarteritis nodosa. PATIENTS AND METHODS: We reviewed the medical records and imaging findings of 17 patients with PAN involving the abdomen. All patients underwent digital subtraction angiography of the renal or visceral arteries completed by a post-angiographic KUB. Abdominal CT scan was available in three patients. All patients underwent muscle biopsy. A surgical biopsy of the gallbladder was obtained in one patient. RESULTS: Multiple small aneurysms involving small and medium sized arteries were detected at angiography in 12 patients. CT showed a renal subcapsular hematoma in two patients and acute pancreatitis in one patient. CONCLUSION: Involvement of gastrointestinal and renal arteries is frequent in polyarteritis nodosa. The diagnosis of PAN should be considered when multiple small-sized aneurysms are present at angiography even if biopsy is negative.

Abdomen↗

[Particularities of visceral leishmaniasis in adults not infected by HIV in Tunisia].

The incidence of adult visceral leishmaniasis in Tunisia has increased in recent years. As a result the epidemiological profile of the disease in our country is now closer to those observed in other Mediterranean countries. Most of the increase involves adults not infected by human immunodeficiency virus (HIV) although 56% of patients presented concurrent disease. In comparison with pediatric visceral leishmaniasis, adult disease is characterized by inconsistence occurrence of some major symptoms such as fever and spleen enlargement. For this reason the clinical disease syndrome may be incomplete and diagnosis may be difficult. Laboratory tests are essential for definitive diagnosis. Although no test is specific, some findings are highly useful, e.g, elevated g-globulins which was observed in 94% of patients in our series. Standard serology is less sensitive in adults with 18% of false negatives in our series probably as a result of immunodeficiency in some patients.

Adolescent↗

[Obstructive acute renal failure revealing visceral leishmaniasis in a diabetic patient].

We report a case of a 22 years old type 1 diabetic man with a history of weight loss, weakness, anorexia, fever and recurrent urinary tract infection since February 2001. In April 2001, he presented anuria due to obstructive acute renal failure. Hepatosplenomegaly and lymphadenopathy were absent at physical examination. Laboratory tests revealed a high level of gamma globulin (53.4 g/l) and anaemia (haemoglobin 7.7 g/100 ml) without leukopenia and thrombocytopenia. CT scan showed multiple retroperitoneal lymphadenopathies causing compression of the two ureters, hydro-ureter associated with hydronephrosis, hepatosplenomegaly and multiple pulmonary nodes. Lymphadenopathies, anaemia, high level of gamma globulin, high titres of anti-leishmanial antibodies and the excellent outcome after treatment with meglumine antimoniate (Glucantime) confirmed visceral leishmaniasis. This report documented an unusual clinical presentation of Visceral leishmaniasis in a diabetic patient.

Acute Kidney Injury↗

[Central diabetes insipidus with Behçet disease. A case report].

Vasculitis with central diabetes insipidus is a rare condition which must be recognized for an appropriate management. We report a case of Behçet disease with central diabetes insipidus. A forty seven year old men presented recurring oral and genital ulcers, skin lesions, polyarthralgia. Two years later, he showed right uveitis and central diabetes insipidus without dysfunction of the pituitary gland. Computed tomographic scan showed normal neurohypophysis and pituitary gland. Later on, he presented hemiplegia. The cerebral computed tomographic scan showed hypodense lesions. Central diabetes insipidus should not be systematically searched because it is a rare feature of the Behçet disease.

Behcet Syndrome↗

[Neurological manifestations in Behcet's disease. Forty observations in a cohort of 300 patients].

PURPOSE: To analyse and discuss neurological involvement of Behçet' disease and therapeutical possibilities giving special attention to unusual forms. METHODS: We retrospectively reviewed the medical records of 40 cases with neurological manifestations among 300 cases of Behçet's disease seen over a 20 years period who met the international study group of Behçet's disease criteria. RESULTS: Central nervous system involvement was found in 39 patients and peripheral nervous involvement in one. Dominant symptoms in 40 patients with neurological manifestations of Behçet's disease were meningo-encephalitis (27 cases), intracranial hypertension (9 cases), psychic disturbances (2 cases), and polyneuritis of the lower limb (1 case). Headache and focal deficits were the major presenting signs (87%). Cerebral venous thrombosis was identified in 8 patients (6 certain and 2 probable). These could be differentiated from meningoencephalitis by their symptomatology predominaly that of intracranial hypertension. In meningoencephalitis, the cerebrospinal fluid findings were lymphocytic pleocytosis and elevated protein level cerebral CT scan, performed in 20 patients, was normal in 30% of cases. MRI performed in 2 patients, showed in one case a tumor like lesion. CONCLUSION: Neurobehçet's disease can be classified into 2 clinical aspects: meningoencephalitis and cerebral venous thrombosis. Treatment is aimed initially at reducing the inflammation with corticosteroids and preventing relapse with adjunction of an immunosuppressor.

Adrenal Cortex Hormones↗

[Pseudotumoral neurobehçet: a case report].

Neurological involvement occurs in 5.3% to 30% of patients with Behçet's disease. Neurological manifestations can be secondary to direct central nervous system involvement (encephalitis, encephalomyelitis) or vascular angeitis (essentially cerebral venous, thrombosis and, rarely, intracranial aneurysms). Neurological pseudotumoral presentation is rarely reported. We report a case of 26-year-old woman suffering from left hemiplegia. MRI was performed and showed pseudotumoral lesion in the protuberance and the right cerebral pedicule. Oral and genital aphtous ulcers were found. HLAB51 was positive. The patient improved with steroid therapy.

Adult↗

[Adult Still's disease: study of a series of 11 cases].

Adult Still's disease is a systemic disease of unknown etiology. We report a retrospective study of 11 cases (9 females and 2 males) of adult Still's disease collected during 25 years. The mean age was 36 years. Fever, arthritis and skin rash was constant. Adenopathies and splenomegaly were observed in 2 patients. The laboratory findings was characterized by a constant inflammatory syndrome and leucocytosis. Hypertransaminasemia and hyperferritinemia were observed respectively in 7 cases and 3 cases. Corticosteroids were prescribed in all patients. Methotrexate was administered in 3 patients. Outcome was favorable in 10 cases, death incurred in one patient, secondary to acute hepatitis.

Adult↗

[Infectious spondylitis. Study of a series of 151 cases].

The authors report their findings in a retrospective study of 151 cases of infectious spondylitis collected between 1970 and 2000. Diagnosis was based on bacteriological or pathological confirmation or on clinical, radiological and outcome arguments. Among 151 cases of infectious spondylitis diagnosed and treated, 110 were due to tuberculosis and 41 to other causes. There were 82 men and 69 women, with a mean age of 42 years. The delay from onset to diagnosis was 12 months. Risk factors were observed in 21% of the cases. The patients searched medical attention because of pain in 85% of cases. The leading causative agents in non tuberculous spondylitis were Staphylococcus aureus, followed by Brucella, and Salmonella, in this order. The diagnosis was considerably facilitated by the imaging techniques used; the most reliable technique was magnetic resonance imaging which improved diagnostic performance by detecting early, specific changes. A histo-bacteriological diagnosis was obtained by percutaneous transpedicular needle biopsy. Globally, 53% of the patients underwent needle biopsy, with a success rate of 62%, a figure comparable with those reported in other studies. In 35% of the patients, bacteriological confirmation of the infection was not obtained and diagnosis was founded on a set of clinical, biological and radiological data. Our results are similar to those described in the literature. However, in our country tuberculosis is the most frequent cause of infectious spondylodiscitis. Two populations are especially susceptible: the elderly and the immunodepressed. A specific diagnosis was obtained in 62% of the percutaneous transpedicular needle biopsy.

Adolescent↗

[Tumorous calcinosis in hemodialysis: anatomo-clinical study apropos of 3 cases].

Three patients (2 females, 1 male) with a mean age of 51.4 years receiving long term hemodialysis affected by tumoral calcinosis were analysed. Clinical, radiological and pathological features were evaluated and pathogenic were reviewed. The joints involved int the cases presented in this report were the hip shoulder and finger. The lesions were bilateral in shoulder. An increased calcium-phosphorus product (Ca x P) was observed in all patients with secondary hyperparathyroidism in one case. Surgery was carried out in all patients. No relapse of the tumoral calcinosis was observed after surgery. The most important pathogenic factor involved in uremic tumoral calcinosis is an increase in calcium-phosphorus product (Ca x P) not necessarily related to hyperparathyroidism. Therefore, maintaining the calcium x phosphate product within the normal range appears to be the most important factor ito prevent the appearance of uraemic tumoral calcinosis.

Adult↗

[Arterial aneurysm in Behçet's disease. Report of 5 cases].

Behçet's disease is a systemic vasculitis generally involving the venous system. Arterial manifestations, usually aneurysm or more rarely occlusion, are less common. We analysed 5 cases of Behçet's disease with arterial aneurysm complications. There were 4 men and 1 woman, mean age 34.6 years. Mean delay to arterial complications was 7.8 years after the first sign of the disease. Four patients showed evidence aneurysms of the pulmonary arteries. One patient developed an aneurysm of the aortic and iliac artery. Phlebitis was associated with arterial involvement in 4 patients. Combined corticosteroid and cyclophosphamide therapy enabled regression of pulmonary aneurysms in 2 patients. One patient was operated, outcome was favorable. Histology showed fragmentation of the media associated with vasculitis. Two patients with pulmonary aneurysms died of massive hemoptysis.

Adult↗

[Nodular and granulomatous form of periarteritis nodosa caused by the hepatitis B virus].

Polyarteritis nodosa (PAN) is, in rare cases, associated with subcutaneous nodules and pathology does not usually show the presence of peri and extravascular granulomas. When present in patients with hepatitis B virus (HBV) related PAN these facts demonstrate that classification of PAN is not homogeneous. CASE REPORT. A patient infected by HBV developed a PAN demonstrated by clinical symptoms and pathology. The disease was characterized by the presence of subcutaneous nodules and histologically by peri- and extra-vascular granuloma which surrounded necrosis of medium-sized vessels. Outcome was also unusual in the patient who did not respond to the association of plasma exchanges and antiviral agents and was only slightly improved by steroids and cyclophosphamide. COMMENTS. HBV-related PAN is considered to be an immune complex disorder. In the present case report granuloma were present as observed in Churg Strauss syndrome or Wegener's granulomatosis which are the consequence of other pathogenetic mechanisms as anticytoplasmic neutrophil antibodies (ANCA). This case reports underlines the heterogeneity of the PAN group of vasculitis and the probable role for various pathogenetic mechanisms.

Antiviral Agents↗