Occurrence of Hypopituitarism in Tunisian Turner Syndrome patients: familial versus sporadic cases.

OBJECTIVE: To explore unusual association between Turner Syndrome (TS) and Hypopituitarism in a Tunisian cohort. METHODS: We reported 6 patients with TS associated to Hypopituitarism, including three familial cases except the fourth sister who showed only a TS phenotype. Biochemical analysis, resonance magnetic imaging and cytogenetic analyses were performed. RESULTS: The average age of our patients was 17.2 years (11-31 years). They were all referred for short stature and pubertal delay, except for the fourth sister who presented spontaneous puberty with the integrity of the pituitary axis and the presence of an X ring chromosome. Karyotype analysis showed monosomy in 3 cases and a mosaic TS in the 3 remaining cases, including one patient with abnormal X chromosome structure. Somatotropic and corticotropic deficiencies were confirmed in 2 sporadic cases while the gonadotropic and thyrotropic axes were spared. In contrast; familial cases were consistently affected by the integrity of the corticotropic axis. MRI showed pituitary hypoplasia in all familial cases and pituitary stalk interruption syndrome in only one sporadic case. No correlation was found between the chromosome formula and the anterior pituitary involvement. CONCLUSION: Co-segregation of congenital Hypopituitarism with pituitary hypoplasia and X chromosome aberrations could imply a molecular anomaly of transcription factors responsible for the differentiation and development of pituitary cells such as PROP1, POUF1, Hesx1, Lhx3, Lhx4. The etiopathogenic link between X chromosome abnormalities and the occurrence of Hypopituitarism remains unclear; however, the progress of molecular biology may clarify the interrelation between transcription factors and sex chromosome segregation abnormalities.

Incidence of methicillin-resistant Staphylococcus aureus nosocomial infections in intensive care units in Lyon University hospitals, France, 2003-2006.

We describe the trends in the incidence of methicillin-resistant Staphylococcus aureus nosocomial infection in intensive care units in Lyon hospitals from January 1, 2003, through December 31, 2006. The incidence rate decreased from 1.77 cases per 100 ICU patients in 2003 to 1.16 cases per 100 ICU patients in 2006, a reduction of 38.0% (P = .05).

[Patelar metastasis of primary lung cancer]. / Métastase rotulienne d'un cancer broncho-pulmonaire primitif.

Patellar metastasis is uncommon. The clinical presentation is often misleading, suggestive of septic arthritis or meniscal disorder after knee trauma. We report the case of a 71-year-old patient who developed knee pain aggravated by movement with pseudo-blockage which was found to be related to a secondary localization of a primary lung cancer. Low blood flow in the patella probably explains why patellar metastasis is so rare.

Osteoid osteoma of the acetabulum.

Osteoid osteoma of the acetabulum can be expected to cause nonspecific symptoms of hip inflammation. In a sixteen year old girl, investigation by routine radiographs and a bone scan suggested a focus of inflammation with a nidus and sclerosis of the acetabulum and overgrowth of the head and neck of the femur. Removal of the lesion by an anterior approach with dislocation of the hip gave excellent results when seen after three years with a normal gait and normal hip motion.

Embolization of cardiac myxomas masquerading as polyarteritis nodosa.

Cardiac myxomas are rare tumors which can mimic other diseases. We describe a 19-year-old man who presented clinically with polyarteritis nodosa (PAN). Muscle biopsy showed vasculitis, but corticosteroid and immunosuppressive therapy was initially effective. Cardiac myxomas were found by echocardiogram when the disease relapsed and were resected surgically. Segmental arterial aneurysms and stenoses were found on angiography after leg ischemia. We suggest that echocardiography should be performed in all cases of clinically suspected PAN.

[Osteoarticular amyloidosis in hemodialyzed patients. Report of 4 cases]. / Amylose ostéoarticulaire des hémodialysés. A propos de 4 cas.

Chronic hemodialysis is sometimes complicated by an osteoarticular amyloidosis, which happens after a middle delay of ten years, it can be expressed by a tunnel carpal syndrome, a shoulder pariarthritis, bony cysts, and erosive spondylarthropathy. The authors reported their experience about four cases, and insisted on some évocatoring radiologic aspects.

[Involvement of the cranial nerves disclosing multiple myeloma]. / Atteintes des paires crâniennes révélatrices de la maladie de Kahler.

Diseases of cranial nerves are very seldom associated with multiple myeloma. However, their occurrence as a harbinger of multiple myeloma seems to bear a particular significance and is worth mentioning. In the present paper, we discuss two cases in which disorders of cranial nerves heralded Kahler's disease. The patients presented with posterior laterocondylar space and cochleovestibular (otoliquorrhea) syndromes, respectively. We stress the diagnostic problems involved and analyze the pathogenesis of diseases of the nervous system associated with multiple myeloma. Diseases of cranial nerves are seldom associated with multiple myeloma. Nonetheless, the clinical setting and laboratory tests will direct the diagnosis. The same does not hold true when one is confronted with Collet-Sicard's syndrome or cochleovestibular syndrome accompanied by otoliquorrhea.

[Monoclonal gammopathies in Tunisia]. / Gammapathies monoclonales en Tunisie.

Protein electrophoresis and immunoelectrophoresis carried out on 4805 sera and 93 urine samples from Tunisian patients over 8 years, and a monoclonal protein was detected in 198 cases. The distribution of the monoclonal compounds to the clinical diagnosis was studied. 115 (58%) were classified as multiple myeloma (MK), 34 (17%) as alpha heavy chain disease (MCL a), 34 (17%) as monoclonal gammapathy associated to various diseases (GMOD), 11 (6%) as benign essential monoclonal gammapathies (GMBE), 4 (2%) as Waldenström's macroglobulinemia (MW). The relationship between the monoclonal immunoglobulin and the clinical diagnosis, the distribution of the monoclonal compounds according to the heavy chain class and the identification of the light chains were studied. The results obtained are in line with the principal data in literature concerning monoclonal gammapathy. However there is a particularity about monoclonal gammapathy observed in the Tunisian's population studied: Higher percentage of alpha heavy chain diseases, monoclonal IgD and monoclonal light chain. Lower percentage of monoclonal IgM in GMBE or GMOD, as in MW.

[Intraorbital involvement in multiple myeloma]. / Localisation intraorbitaire du myélome multiple.

According to data from the literature, intraorbital involvement in multiple myeloma is rare. Such involvement may result in exophthalmia, itself the first manifestation and presenting feature of myeloma. These lesions can respond remarkably to radiotherapy. The authors present a new case report.

[Wegener's granulomatosis and pregnancy. A case]. / Granulomatose de Wegener et grossesse. Une observation.

Pregnancy concomitant with Wegener's granulomatosis is extremely rare: so far, only four cases have been published. The authors report a fifth case where the disease appeared during the post-partum period, a situation which has already been noted in two of the published cases. Interruption of a subsequent pregnancy was followed by a flare-up of the disease resulting in the patient's death. This suggests that post-partum and post-abortum are probably instrumental in the onset and deterioration of Wegener's granulomatosis. The two patients previously reported who received immunosuppressants combined with corticosteroids had no flare-up after delivery. It seems permissible to prescribe such a therapeutic combination before and after delivery or abortion, especially since the fear of foetal toxicity from these drugs seems to be exaggerated.