Successful treatment of hepatic and mesh Aspergillosis in a neonate with giant omphalocele.

Aspergillosis is a rare and life-threatening fungal infection in immunocompetent children, particularly in infants. We present a case of invasive Aspergillosis (IA) that developed hepatic and mesh infection due to Aspergillus flavus following the rupture of a giant omphalocele. A male baby was born at 37 weeks of gestation with a giant omphalocele. No other congenital abnormalities were found. In the pediatric surgery operation room, the amniotic membrane seemed to be ruptured in some parts, which had been repaired within a few hours of birth. No further intervention to reduce the volume of visceral organs could be performed due to the tiny abdominal cavity. Postoperatively piperacillin-tazobactam and fluconazole were empirically initiated. Abdominal defects had been covered with polypropylene polylactic acid synthetic Mesh on postnatal day 11 to protect the organs and prevent abdominal infections. Fungal hepatic lesions were observed during surgery, and black necrotic lesions were also observed on the mesh on day 12. On the 16th postnatal day, Aspergillus flavus grew in the wound, graft, hepatic biopsy cultures. Serum galactomannan (GM) assay test was positive (GM:2.9), and voriconazole was initiated. All necrotic lesions resolved with liposomal amphotericin B (L-AmB) and voriconazole treatment, and antifungal therapy was discontinued after 134 days. Eye examination for the retinopathy didn't show any abnormalities. The patient was discharged with full recovery on day 155. In conclusion, IA is a rare and life-threatening infection in the neonatal period. To the best of our knowledge, this is the first case of hepatic and mesh infection caused by Aspergillus flavus that was successfully treated with voriconazole and l-AmB.

Early blunted cortisol response to insulin induced hypoglycaemia in familial Mediterranian fever.

OBJECTIVE: To study cortisol, adrenocorticotropic hormone and C-reactive protein responses to specific stimuli in familial Mediterranean fever (FMF). METHODS: For the purpose of measuring cortisol, ACTH, and CRP responses to insulin induced hypoglycaemia during attack-free periods, 14 FMF patients, 11 patients with ankylosing spondylitis or Behçet's disease as disease controls (DC), and a further 10 healthy control subjects (HC) were involved in this study. None of the subjects had ever received corticosteroids before this study. Cortisol and ACTH levels were measured by chemiluminescence enzyme immunoassay. RESULTS: No attack was observed among FMF patients during the test. No significant difference in the mean cortisol values after insulin induced hypoglycaemia was observed between the groups involved at any stage of the test. The integral cortisol response to hypoglycaemia expressed as the AUC (0-90 min) was found not to differ among the study groups (1827 +/- 115.6 in FMF; 2196 +/- 205.4 in DC, p = 0.12; 1771 +/- 98.4 in HC, p = 0.9). The delta response of cortisol to insulin induced hypoglycaemia wasfound to be statistically lower (-4 +/- 0.8 mg/dl vs. -1.9 +/- 0.7 microg/dl; p<0.03) only for the 0 to 30 min interval in patients with FMF compared to HC respectively. Similar results, though of no statistical significance, were also found for the 0 to 45 min interval (1.17 +/- 2.2 microg/dl in FMF patients vs. 3.3 +/- 2 microg/dl in HC; p = 0.6). The mean basal CRP level of patients with FMF was remarkably higher than that in HC. Although the mean CRP level at 90 min for FMF cases with cortisol levels under 12 microg/dl at 30 min was found to be higher than those with cortisol levels over 12 microg/dl at 30 min, no significant difference was observed. CONCLUSION: An early blunted cortisol response observed in a stressful situation in FMF patients may well account for the curious relationship between stress and an inflammatory reaction and/or attack. Furthermore, the fact that the CRP level was relatively higher in FMF patients with lower cortisol levels might also highlight the importance of endogen cortisol in the inflammatory feature of this disease.