Ovarian Juvenile Granulosa Cell Tumor in A 2.5 Years-Old Girl: The Importance of Early Diagnosis

Juvenile granulosa tumor is a rare tumor in infant. The diagnosis should be discussed in any ovarian tumor of children, especially in front of the early signs of puberty. The prognosis of granulosa cell tumors remains good. The main prognostic factor is the stage of local extension. Early diagnosis at early signs of puberty is an important prognostic factor. We describe the case of a girl aged 2 years and 5 months who presented signs of early puberty. The diagnosis was a secreting ovarian tumor but was very large at that time. We focus by this observation on the value of early diagnosis of this tumor type which remains a key prognostic factor

Inguinoscrotal hernia revealing a testicular hemangioma: Report of a neonatal case

Testicular hemangioma (TH) is a congenital, uncommon and non-malignant testicular tumor. In the following report, we present a child who was brought to our emergency department with swelling of the right scrotum. Investigations were consistent with a TH and an associated hernia. We conducted an inguinal exploration given the hernia accompanying the TH. The pathological tissue findings were suggestive of a TH. In our further research, we found that this was the first neonatal hemangioma in the literature. It's important to know that an inguinal hernia can lead to the non-recognition of certain tumors

A Tunisian patient with two rare syndromes: triple a syndrome and congenital hypogonadotropic hypogonadism.

International audienceThe coexistence of triple A syndrome (AAAS) and congenital hypogonadotropic hypogonadism (CHH) has so far not been reported in the literature. This study aimed to characterize at the clinical and genetic level one patient presenting an association of AAAS and CHH in order to identify causal mutations.Clinical and endocrinal investigations were performed and followed by mutational screening of candidate genes. At the age of 18, the patient presented sexual infantilism, a micropenis and gynecomastia. No mutation was revealed in GnRHR, TACR3/TAC3, PROK2/PROKR2 and PROP1 genes, except a homozygous intronic variation (c.244 + 128C>T; dbSNP: rs350129) in the KISS1R gene, which is likely nondeleterious. A homozygous splice-donor site mutation (IVS14 + 1G>A) was found in the AAAS gene. This mutation, responsible for AAAS, is a founder mutation in North Africa.This is the first report on a Tunisian patient with the coexistence of AAAS and CHH. The diagnosis of CHH should be taken in consideration in patients with Allgrove syndrome and who carry the IVS14 + 1G>A mutation as this might challenge appropriate genetic counseling

Peptic oesophageal stricture in children: Management problems

Background: Peptic oesophageal stricture (PES) is a serious complication of gastroesophageal reflux disease (GERD) in childhood. The treatment of PES is still controversial, ranging from simple oesophageal dilations to resection/anastomosis of the stenotic portion of the oesophagus. In this study, we want to share our experience with 11 children with GERD and PES. Patients and Methods: A retrospective review of clinical data obtained from children who underwent dilation and antireflux surgery for PES was performed. Results: A total of 11 patients were diagnosed with PES. The clinical picture was dominated by dysphagia. Barium swallow showed hiatal hernia in nine cases (82%). Oesophageal strictures were located most commonly in the lower third of the oesophagus (91%). Three Children (27%) with PES had a neurologic impairment and patients had a mean duration of symptoms of 20 months (range, 3 month to 6.2 years) before intervention. Children received a median of four dilations (range, 1-21 dilations) for PES. Time to first dilation from age of diagnosis was a mean of 4.5 months (range, 2-14 months). Antireflux surgery was performed in all patients. Post-operatively, seven patients required repeat oesophageal dilation. Patients were followed with serial dilation for a median of 6 years (range, 1-9 years) and only one patient has a continued requirement of oesophageal dilation for PES. Conclusion: GERD complicated by PES is an important condition affecting a significant number of children. Early and effective treatment of both stricture and GERD is required to improve the prognosis of this serious condition

Primary hydatid cyst of the small intestine masquerading as intestinal duplication in a child

Hydatid disease or hydatidosis is a worldwide zoonosis disease caused by the tapeworm of Echinococcus granulosus and still widely endemic in Tunisia especially in rural areas where the sheep-dog cycle is dominant. It is an important public health problem in the pediatric age group causing significant morbidity and mortality. We report a case of primary hydatid cyst of the small intestine in a child and we want to highlight the difficulty that we meet in the diagnosis despite the contribution of imaging

Contamination of planktonic food webs in the Mediterranean Sea: Setting the frame for the MERITE-HIPPOCAMPE oceanographic cruise (spring 2019)

International audienceThis paper looks at experiential feedback and the technical and scientific challenges tied to the MERITE-HIPPOCAMPE cruise that took place in the Mediterranean Sea in spring 2019. This cruise proposes an innovative approach to investigate the accumulation and transfer of inorganic and organic contaminants within the planktonic food webs. We present detailed information on how the cruise worked, including 1) the cruise track and sampling stations, 2) the overall strategy, based mainly on the collection of plankton, suspended particles and water at the deep chlorophyll maximum, and the separation of these particles and planktonic organisms into various size fractions, as well as the collection of atmospheric deposition, 3) the operations performed and material used at each station, and 4) the sequence of operations and main parameters analysed. The paper also provides the main environmental conditions that were prevailing during the campaign. Lastly, we present the types of articles produced based on work completed by the cruise that are part of this special issue

Rationale, design, and baseline characteristics in Evaluation of LIXisenatide in Acute Coronary Syndrome, a long-term cardiovascular end point trial of lixisenatide versus placebo

BACKGROUND: Cardiovascular (CV) disease is the leading cause of morbidity and mortality in patients with type 2 diabetes mellitus (T2DM). Furthermore, patients with T2DM and acute coronary syndrome (ACS) have a particularly high risk of CV events. The glucagon-like peptide 1 receptor agonist, lixisenatide, improves glycemia, but its effects on CV events have not been thoroughly evaluated. METHODS: ELIXA (www.clinicaltrials.gov no. NCT01147250) is a randomized, double-blind, placebo-controlled, parallel-group, multicenter study of lixisenatide in patients with T2DM and a recent ACS event. The primary aim is to evaluate the effects of lixisenatide on CV morbidity and mortality in a population at high CV risk. The primary efficacy end point is a composite of time to CV death, nonfatal myocardial infarction, nonfatal stroke, or hospitalization for unstable angina. Data are systematically collected for safety outcomes, including hypoglycemia, pancreatitis, and malignancy. RESULTS: Enrollment began in July 2010 and ended in August 2013; 6,068 patients from 49 countries were randomized. Of these, 69% are men and 75% are white; at baseline, the mean ± SD age was 60.3 ± 9.7 years, body mass index was 30.2 ± 5.7 kg/m(2), and duration of T2DM was 9.3 ± 8.2 years. The qualifying ACS was a myocardial infarction in 83% and unstable angina in 17%. The study will continue until the positive adjudication of the protocol-specified number of primary CV events. CONCLUSION: ELIXA will be the first trial to report the safety and efficacy of a glucagon-like peptide 1 receptor agonist in people with T2DM and high CV event risk