Unusual bone localization of sarcoidosis mimicking metastatic lesions: case report and review of literature.

Sarcoidosis is a multisystem disease of unknown origin. Diagnosis remains challenging, based on organ site involvement, histological confirmation of non-caseating granuloma and an appropriate clinical syndrome. Granulomatous bone involvement is rare and may be ignored because it is usually asymptomatic. Vertebrae, ribs and skull localizations are rarely reported. We described an interesting case of a woman with chronic and multiorgan sarcoidosis with unusual bone localizations.

Giant pulmonary hydatid cyst in children.

Hydatid disease is still endemic in Tunisia. It is mostly seen in young people less than 40 years and children are affected in one third of cases. The lungs are the predominant location in children. Our study aims to define the particularities of children PHC's (pulmonary hydatic cyst) management, the characteristics of giant cyst and to study predictive factors of complications. We included retrospectively 105 children with PHC followed between 1999 and 2019. Patients were aged less than 16 years with surgically confirmed diagnosis of PHC. Two groups of cysts were defined: giant cysts which were 10 cm across or more, and no giant cysts.The sex-ratio was 1.38 with a mean age of 10.5±3 years. The symptomatology was dominated by cough (59%), thoracic pain (51%) and hemoptysis (46%). Giant cysts were observed in 24 (22.9%) patients. Dyspnea (29% vs 5% p<0.001) and thoracic pain (88% vs 41% p<0.001) were significantly more frequently reported in giant cysts. Eighty-six patients had a single cyst (83%) and 19 had multiple cysts (17%). Giant cysts accounted for 22,9% (24 cases). Thoracic ultrasonography was diagnostic in 77.4%. The thoracic CT scan was performed in 27 children with inaccessible cysts in thoracic ultrasonography or in diagnostic doubt.Patients were all treated surgically. Surgical procedures consisted of cystectomy (59%), pericystectomy (18%) and pulmonary resection when parenchyma was destroyed (23%). Parenchymal resection was more often performed in complicated cysts (27% vs 20% p>0.05) and in giant cysts (41% vs 18% p<0.05). A two-stage thoracotomy was performed in the 4 patients with bilateral cysts. Thirteen patients presented immediate post-operative complications which occurred more frequently in complicated and giant cysts. Hospital stay was longer in complicated cysts (16±9 days vs 7±3 days; p<0.001) and in giant cysts (14±9 days vs 11±8 days; p>0.05). In endemic regions, the diagnosis of PHC in children should be based on the combination of thoracic radiography and ultrasonography which are effective, not costly, safe and accessible. Complicated and giant PHC cause lung damage leading to extensive parenchymal resection. They are more associated with post-operative complications prolonging hospital stay and increasing expenses.

Giant pulmonary hydatid cyst in children.

BACKGROUND: Lungs are the second most common site for hydatid disease after the liver. Giant hydatid cyst (GHC) of the lung is a special clinical entity in children and is related to higher lung tissue elasticity. AIM: To compare clinical and imaging features, types of surgical interventions, and postoperative complications in pulmonary GHC and non-giant pulmonary hydatid cysts (NGHC) in children. METHODS: A retrospective study was undertaken. The data analyzed were taken from medical records of children with pulmonary hydatid cyst (PHC) hospitalized in a pulmonary department in Tunisia between January 2004 and February 2019. Cysts were divided according to their size into GHC ( ≥10cm) and NGHC (<10cm). RESULTS: In the study period, 108 PHC were recorded in 84 children. GHC accounted for 21 (19.4%) and NGHC for 87 (80.6%). The median of age of the children was 11 years (IQR 1-9, IQR 3-14) and the mean age was 11.6 years (10.5 in GHC vs. 11.4 years in NGHC). Hemoptysis was found in 25% of the GHC group vs. 48.4% of the NGHC group (P=0.27). Cysts were multiple in 23.8% of cases and predominated in the right in 64.3% of cases and in the inferior lobes in 71.4% of the cases. GHCs were less frequently complicated (60% vs. 78.1% in NGHC, P≤0.11), although not significantly. Parenchymal resection was realized in 50% of GHC vs. 18.8% of NGHC (P=0.006). No significant difference was found in postoperative complications between the two groups and there was no recurrence in either group. CONCLUSION: GHC is a special clinical entity in children. It requires major surgery with parenchymal resection, and therefore early diagnostic and therapeutic management is warranted.

Unusual cause of laryngeal dyspnea and swallowing troubles in a 2-year old child.

Plexiform neurofibromas are rare benign tumors developed from peripheral nervous system often associated with neurofibromatosis type 1. We report the case of multifocal plexiform neurofibromas in a 2-year-old child with cervical mass obstructing the trachea causing respiratory distress. A cervical ultrasound examination was performed followed by enhanced CT and MRI. Imaging revealed an expansive cervical mass extended from the base of the skull to the mediastinum associated with similar pelvic and sacral foraminal masses. The target like MRI aspect on T2-weighted images was suggestive of the neural origin. Biopsy under ultrasound control confirmed the diagnosis of plexiform neurofibroma.

Th 9 cells in Behçet disease: Possible involvement of IL-9 in pulmonary manifestations.

Behçet disease (BD) is a multisystemic disease some of whose manifestations are characterized by pulmonary involvements. The purpose of the study was to evaluate the level of T-helper type 9 (Th9) cells and the cytokine interleukin (IL)-9 in peripheral blood and in bronchoalveolar lavage (BAL) of patients with Behçet's disease (BD) affected by pulmonary manifestations. Nevertheless, until recently there have been no studies on its role in BD. The Th9 (CD4+IL-9+T) cell, transcription factor PU.1 and IL-9 mRNA levels, as well as serum and BAL IL-9 concentration, were measured in BD patients and healthy controls. The Th9 cell percentage and absolute number, PU.1 and IL-9 expression levels of BD patients were all increased significantly compared with the control group. Absolute number of Th9 cells was particularly increased in patients with active BD compared to inactive BD patients. The levels of IL-9 associated to Th9 expression depended on BD severity. These parameters were markedly expressed in the BAL of BD patients with pulmonary manifestations. IL-17 and the epithelial inflammatory cytokine TSLP were significantly correlated to IL-9 levels. This cytokine trio decreased in inactive BD patients after corticosteroïd treatment. In addition, IL-9 levels were correlated to CD4+ IL-9+ cells in BAL and in PBMCs. LPS stimulated PBMCs and macrophages induced increased secretion of IL-9 and the encoding transcription factors PU.1 and IRF4. In conclusion, the expansion of the Th9 cell subset, up-regulation of the PU.1 transcription factor and increased secretion of the IL-9 cytokine may contribute to the pathogenesis of BD, which may be supported by the increased release of IL-17 and TSLP. We provide evidence that Th9 T cells are increased in BD patients with pulmonary manifestations. This suggests an important role of IL-9 in the pathogenesis of BD particularly in patients suffering from lung involvement.

Gene Variants, mRNA and NOD1/2 Protein Levels in Tunisian Childhood Asthma.

INTRODUCTION: Asthma is a common respiratory childhood disease that results from an interaction between genetic, environmental and immunologic factors. The implication of nucleotide-binding and oligomerization domain 1 and 2 (NOD1/CARD4, NOD2/CARD15) was highlighted in many inflammatory diseases. METHODS: In this case-control study, we analyzed the association of three NOD2 polymorphisms and one NOD1 variant, in 338 Tunisian asthmatic children and 425 healthy Controls, using polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) method. We also assessed NOD1 and NOD2 mRNA and protein levels by qRT-PCR and ELISA techniques. RESULTS: The homozygous AA genotype of rs2075820 was a risk factor for asthma (OR 2.39). The influence of the E266K variant in the presence of the heterozygous AG genotype was higher in male than female groups. The homozygous AA genotype was a risk factor associated with asthma, for patients aged between 6 and 18 years OR 2.39, IC95% (1.04-5.49) p < 0.01. The mRNA expression of NOD1, but not NOD2, was enhanced in asthma patients compared to Controls. We noted a significant difference between asthmatics and healthy controls in NOD1 protein expression (asthma patients : 31.18 ± 10.9 pg/ml, Controls: 20.10 ± 2.58 pg/ml; p < 0.001). CONCLUSIONS: The NOD1 rs2075820 variant was associated with a higher childhood asthma risk and the NOD1 expression at mRNA and protein levels was significantly increased in asthma patients.