Successful treatment of sepsis caused by Staphylococcus lugdunensis in an adult with 22q11.2 deletion syndrome.

Published on Jan 1, 2012in Internal Medicine1.005
· DOI :10.2169/INTERNALMEDICINE.51.6257
Shoji Hirasaki19
Estimated H-index: 19
(Okayama University),
Kazutoshi Murakami14
Estimated H-index: 14
(Okayama University)
+ 11 AuthorsNorio Koide20
Estimated H-index: 20
(Okayama University)
A 27-year-old woman visited our hospital because of high fever. She had been diagnosed as 22q11.2 deletion syndrome (22q11.2DS) due to her cardiac history (tetralogy of Fallot), thymic hypoplasia and 22q11.2 deletion. She had a normal CD4/CD8 ratio, a slightly decreased lymphocyte count and normal serum immunoglobulin levels. Blood cultures were positive for Staphylococcus lugdunensis (S. lugdunensis). Infection route of S. lugdunensis in this case was unclear. The patient was successfully treated with several intravenous antibiotics. Infection should be considered when managing patients with 22q.11.2DS. regardless of whether their immune system is impaired.
#1Eliezer M. PereiraH-Index: 10
#2Ricardo Pinto Schuenck (UFES: Universidade Federal do Espírito Santo)H-Index: 7
Last. Kátia Regina Netto dos Santos (UFRJ: Federal University of Rio de Janeiro)H-Index: 23
view all 4 authors...
Staphylococcus lugdunensis is a rare cause of severe infections and clinical manifestations are similar to those related to S. aureus infection. We describe a hospital-acquired bacteremia due to methicillin-resistant Staphylococcus lugdunensis, misidentified as methicillin-resistant S. aureus. The oxacillin MIC was 16 µg/mL and the mecA gene and SCCmec type V were determined by PCR. Although treatment had been appropriated, the patient died after rapid progressive respiratory failure and another...
11 CitationsSource
#1Seong-Ho Choi (CAU: Chung-Ang University)H-Index: 13
#2Jin-Won Chung (CAU: Chung-Ang University)H-Index: 12
Last. Sang-Ho Choi (UOU: University of Ulsan)H-Index: 64
view all 11 authors...
Of 63 patients with Staphylococcus lugdunensis bacteremia, 15 (23.8%) had clinically significant bacteremia, with an incidence of 1.3 cases per 100,000 admissions. Of the five patients with community-acquired S. lugdunensis bacteremia, three had endocarditis. Catheters were the most common portal of entry for health-care-associated or hospital-acquired bacteremia. Only one patient died of bacteremia-related causes.
60 CitationsSource
#2Ana TöpfH-Index: 20
Last. Barbara J.M. MulderH-Index: 77
view all 10 authors...
Background Three quarters of patients with 22q11.2 Deletion Syndrome (22q11.2DS) have congenital heart disease (CHD), typically conotruncal heart defects. Although it is currently common practice to test all children with typical CHD for 22q11.2DS, many adult patients have not been tested in the past and therefore 22q11.2DS might be under-recognised in adults. Objectives To determine the prevalence of 22q11.2DS in adults with tetralogy of Fallot (TOF) and pulmonary atresia (PA)/ventricular septa...
56 CitationsSource
#1P. EberleH-Index: 1
#2Christoph BergerH-Index: 54
Last. T. GüngörH-Index: 1
view all 9 authors...
A subgroup of patients with 22q11.2 microdeletion and partial DiGeorge syndrome (pDGS) appears to be susceptible to non-cardiac mortality (NCM) despite sufficient overall CD4(+) T cells. To detect these patients, 20 newborns with 22q11.2 microdeletion and congenital heart disease were followed prospectively for 6 years. Besides detailed clinical assessment, longitudinal monitoring of naive CD4(+) and cytotoxic CD3(+)CD8(+) T cells (CTL) was performed. To monitor thymic activity, we analysed naiv...
20 CitationsSource
#1Kristi L. Frank (Mayo Clinic)H-Index: 16
#2José Luis del PozoH-Index: 20
Last. Robin Patel (Mayo Clinic)H-Index: 95
view all 3 authors...
Staphylococcus lugdunensis has gained recognition as an atypically virulent pathogen with a unique microbiological and clinical profile. S. lugdunensis is coagulase negative due to the lack of production of secreted coagulase, but a membrane-bound form of the enzyme present in some isolates can result in misidentification of the organism as Staphylococcus aureus in the clinical microbiology laboratory. S. lugdunensis is a skin commensal and an infrequent pathogen compared to S. aureus and S. epi...
297 CitationsSource
#1Valerie Waters (U of T: University of Toronto)H-Index: 31
#2Karin PetersonH-Index: 1
Last. Philip LaRussaH-Index: 37
view all 3 authors...
We report a case of pneumonia in a 13 month old male child with partial DiGeorge syndrome who died after inadvertently receiving live viral vaccines. Although live viral vaccines have been used safely in some children with DiGeorge syndrome, there are insufficient data to recommend their routine use in those with severe immunodeficiency.
18 CitationsSource
#1Alice D. Chang (UCLA: University of California, Los Angeles)H-Index: 1
#2Raffi Tachdjian (UCLA: University of California, Los Angeles)H-Index: 13
Last. Ora Yadin (UCLA: University of California, Los Angeles)H-Index: 12
view all 7 authors...
We studied a 14 year-old boy with partial DiGeorge syndrome (DGS), status post complete repair of Tetralogy of Fallot, who developed antiphospholipid syndrome (APS) and type III mixed cryoglobulinemia. He presented with recurrent fever and dyspnea upon exertion secondary to right pulmonary embolus on chest computed tomography (CT). Coagulation studies revealed homozygous methylene tetrahydrofolate reductase 677TT mutations, elevated cardiolipin IgM antibodies, and elevated β2-glycoprotein I IgM ...
11 CitationsSource
#1A.L. CaseyH-Index: 4
#1Anna L. Casey (University Hospitals Birmingham NHS Foundation Trust)H-Index: 15
Last. Tom S.J. ElliottH-Index: 39
view all 6 authors...
Objectives: A rapid random amplification of polymorphic DNA (RAPD) technique was developed to distinguish between strains of coagulase-negative staphylococci (CoNS) involved in central venous catheter (CVC)-related bloodstream infection. Its performance was compared with that of pulsed-field gel electrophoresis (PFGE). Methods: Patients at the University Hospital Birmingham NHS Foundation Trust, U.K. who underwent stem cell transplantation and were diagnosed with CVC-related bloodstream infectio...
21 CitationsSource
#1Isao FujiiH-Index: 2
#2Yasushi UenoH-Index: 2
Last. Yoshitaka GotoH-Index: 5
view all 4 authors...
9 CitationsSource
OBJECTIVES: To compare different methods for the identification and determination of susceptibility to penicillin and methicillin of Staphylococcus lugdunensis. METHODS: Seventeen clinical isolates of S. lugdunensis (identified by PCR amplification and sequencing of the rpoB gene) were studied using the ATB32-Staph, Crystal, Vitek 2 and Wider commercial systems. The clumping factor test and the tube coagulase test were also performed. Beta-lactamase production was studied by chromogenic methods....
77 CitationsSource
Cited By4
#1Goran Cuturilo (University of Belgrade)H-Index: 10
#2Danijela Drakulic (University of Belgrade)H-Index: 8
Last. Milena Stevanovic (University of Belgrade)H-Index: 21
view all 12 authors...
22q11.2 microdeletion is the most common microdeletion in humans. The purpose of this study was to evaluate postoperative outcome in children with 22q11.2 microdeletion who had undergone complete surgical correction of a congenital heart defect. The study included 34 patients who underwent complete correction of conotruncal heart defects. Of these, 17 patients diagnosed with 22q11.2 microdeletion represent the investigated group. Another 17 patients without 22q11.2 microdeletion represent the co...
3 CitationsSource
Staphylococcus lugdunensis (SL) is a bacterium with a highly pathogenicity than most other coagulase-negative Staphylococcus spp. (CoNS). In Japan, data on this pathogen are sparse, and the current prevalence of SL bacteremia is unknown. Therefore, we investigated the prevalence of SL in blood culture specimens in a prospective multicenter study across 5 facilities. A total of 3,284 patients had positive blood cultures, and 2,478 patients had bacteremia. Among the patients with bacteremia, 7 pat...
2 CitationsSource
Purpose of review22q11 deletion syndrome is the most common genetic abnormality. More patients are surviving cardiac surgery, and many do not have cardiac anomalies. Adult patients are now being described. It is important for paediatricians, and increasingly adult physicians, to be aware of the opti
6 CitationsSource
#1Shihui Yu (UW: University of Washington)H-Index: 13
#2William D. GrafH-Index: 23
Last. Robert J. ShprintzenH-Index: 64
view all 3 authors...
PURPOSE OF REVIEW:Chromosome 22, the first human chromosome to be completely sequenced, is prone to genomic alterations. Copy-number variants (CNVs) are common because of an enrichment of low-copy repeat sequences that precipitate a high frequency of nonallelic homologous misalignments and unequal recombination during meiosis. Among these is one of the most common multiple anomaly syndromes in humans and the most common microdeletion syndrome, velocardiofacial syndrome (VCFS), also known as 22q1...
18 CitationsSource