Cutis Laxa syndrome: a case report

Cutis laxa (CL) is a heterogeneous group of inherited and acquired connective tissue disorders characterized by a loose skin and variable systemic involvement (inguinal hernia, cardiopulmonary disease, and emphysema). Autosomal dominant, autosomal recessive and x-linked recessive patterns have been described in the inherited forms. Acquired forms of this disease have been associated with a previous inflammatory skin disorder (urticaria...). The characteristic symptomatological pattern is resulting from paucity of elastic fibers. We report an 18 months old baby boy with a congenital cutis laxa. He was admitted in pediatric unit for respiratory disorders. The diagnosis of CL syndrome is based on clinical assessment of typical skin features and the associated extracutaneous finding

The effect of past use of oral contraceptive on bone mineral density, bone biochemical markers and muscle strength in healthy pre and post menopausal women

<p>Abstract</p> <p>Background</p> <p>during adulthood, most studies have reported that oral contraceptive (OC) pills had neutral, or possibly beneficial effect on bone health. We proposed this study of pre and post menopausal women assessing BMD, bone biochemical markers and physical performance among OC past users and comparable women who have never use Ocs.</p> <p>Methods</p> <p>A cross-sectional study comparing the bone density, bone biochemical markers (osteocalcin, CTX) and three measures to assess physical performance: timed get-up-and-go test "TGUG", five-times-sit-to-stand test "5 TSTS" and 8-feet speed walk "8 FSW" of users and never users OC. We were recruited 210 women who used OC for at least 2 years with that of 200 nonusers was carried out in pre and postmenopausal women (24-86 years).</p> <p>Results</p> <p>when analysing the whole population, BMD and biochemical markers values were similar for Ocs past users and control subjects. However when analysing the subgroup of premenopausal women, there was a statistically significant difference between users and never-users in osteocalcin (15,5 ± 7 ng/ml vs 21,6 ± 9 ng/ml; p = 0,003) and CTX (0,30 ± 0,1 ng/ml vs 0,41 ± 0,2 ng/ml; p = 0,025). This difference persisted after adjustment for age, BMI, age at menarche and number of pregnancies. In contrast, in post menopausal women, there was no difference in bone biochemical markers between OC users and the control. On the other hand OC past users had a significant greater performance than did the never users group. And when analysing the physical performance tests by quartile OC duration we found a significant negative association between the three tests and the use of OC more than 10 years.</p> <p>Conclusion</p> <p>the funding show no evidence of a significant difference in BMD between Ocs users and never user control groups, a decrease in bone turn over in OC pre menopausal users and a greater physical performances in patients who used OC up than 10 years.</p

AUTOIMMUNE LYMPHOPROLIFERATIVE SYNDROME: A CASE REPORT

&lt;p&gt;Autoimmunelymphoproliferative syndrome (ALPS), a rare geneticdisorder, is a non-malignant, non-infectiouslymphoproliferationthatalters immune system homeostasis by altering FAS-mediated lymphocyte apoptosis. In the past few years, the diagnosis of ALPS wasalwaysconfusedwithotheridiopathicautoimmune pathologies such as Evans syndrome (which combines autoimmunehemolyticanemia and idiopathicthrombocytosis), histyocytosis or systemic lupus erythematosus. In this article, wepresenta case of a childaged 2 years and 7 months, whopresentedwithprolongedfeverassociatedwith a tumor syndrome consisting of polyadenopathy, hepatomegaly, splenomegaly and recurrentcytopenia. Aftereliminatingother diagnoses, the diagnosis of autoimmunelymphoproliferative syndrome wasretained by the demonstration of the FAS mutation on geneticstudy.&lt;/p&gt;&lt;p&gt;&nbsp;&lt;/p&gt

Epidemiological and clinical characteristics of scorpion stings in children in fez, Morocco

Scorpion stings are a public health problem in Morocco, especially among children, who experience the most severe cases. Epidemiological and clinical findings on scorpion stings in Fez, Morocco, were evaluated in this investigation. Of 163 cases that required medical attention, 62.6% were male children. The mean age of patients was 4.8 ± 3.4 years. The mean time between stings and first medical attention was 3.36 ± 2.5 hours. Almost all cases occurred in the summer (94%) and extremities represented the most frequent sting sites (86.5%). Local pain, hyperemia, scarification, vomiting, sweating, restlessness, tachycardia and tachypnea were the observed clinical symptoms. Regarding severity, 55.2% of patients belonged to class III, followed by class II (26.4%) and class I (18.4%). None of our patients received antivenom; however, all of them were treated symptomatically depending on clinical manifestations