Recurrent mucocutaneous infections caused by PVL-positive Staphylococcus aureus strains: a challenge in clinical practice.

BACKGROUND: Recurrent mucocutaneous infections caused by PVL-positive Staphylococcus (S.) aureus strains represent an increasing problem in Germany. Although there have been several outbreaks at day care centers and in urban communities in recent years, there are currently no diagnostic algorithms or treatment recommendations for these particular infections in Germany. METHODS: We performed a literature search in the PubMed/MEDLINE database with the goal of developing an algorithm for diagnosis and treatment of these infections. National and international recommendations were also considered. RESULTS: Panton-Valentine leukocidin (PVL) is a pore-forming protein produced by certain S. aureus strains. Both methicillin-susceptible (MSSA) and methicillin-resistant S. aureus (MRSA) strains may carry the lukS-lukF gene responsible for PVL production. The clinical presentation of infections caused by PVL-positive S. aureus ranges from isolated recurrent abscesses to extensive furunculosis. Despite adequate treatment of primary infections, approximately 40 % of patients develop recurrent disease. The choice of treatment regimen is guided by the clinical presentation of the infection. In addition, some scientific literature recommends bacteriological screening of patients and their contacts, followed by decolonization of affected individuals. CONCLUSIONS: The present article focuses on the pathogenesis and risk factors of recurrent mucocutaneous infections caused by PVL-positive S. aureus strains and proposes a diagnostic and therapeutic algorithm for optimal patient care.

Obstetric and Neonatal Outcome of Pregnancy in Carney Complex: A Case Report.

Background: Carney complex (CNC) is a rare multiple endocrine neoplasia syndrome with autosomal dominant inheritance. Affected individuals present with mucocutaneous lentigines/blue nevi, cardiac and noncardiac myxomatous tumors, and multiple endocrine tumors. Mutations in PRKAR1A have been identified as genetic cause of the disease. Here, we report on pregnancy, delivery and puerperium in a woman with genetically confirmed CNC and her newborn. Case: The 31 year-old gravida 5 para 1 with CNC was referred at 26 weeks of gestation. Adrenocorticotropin-independent hypercortisolism, hyperglycemia, hypertension, low serum potassium, and osteoporotic fractures were present. Treatment with metyrapone, a reversible 11-beta-hydroxylase inhibitor, was initiated. The maternal condition improved, and a 5 weeks' pregnancy prolongation could be achieved. Elective repeat cesarean section was performed at 31 weeks of gestation for recurrent vaginal bleeding. The neonate developed transient hyponatremia necessitating hydrocortisone substitution for 2 weeks. Conclusion: In our case, treatment of CNC-associated hypercortisolism in pregnancy with metyrapone was effective. Maternal side effects did not occur. The newborn presented with transient hypocortisolism most likely due to transplacental drug effect. Our case illustrates that the treatment of rare diseases in pregnancy represents a challenge requiring interdisciplinary team work.