The reported rates of coccidioidomycosis have increased five-fold from 2.4/100,000 population in 2000 to 10.8 in 2012. To specifically evaluate the epidemiology of coccy in pediatric patients, three datasets were analyzed: surveillance, hospitalization, and death datasets. The total cases of coccy reported during this period in California were 35,804, including 3453 incident cases in children 17 years of age or younger. Eight hundred forty-one pediatric cases required hospitalization. In children, the overall incidence and rates of hospitalization increased six-fold, with incidence going from 0.7 to 3.9, and hospitalization rate increasing from 0.2 to 1.2 cases/100,000 population. The RR of both infection and hospitalization was slightly higher for males than females and higher in the older age group (12-17 years) than in the youngest children (0-2 years). The risk of infection was greater in Latino children, but the risk of hospitalization was greatest in African-American children when compared to white children. RR of infection was 95 times greater in children coming from endemic areas compared to less endemic areas.

Of the 841 hospitalized pediatric patients, 58% had a diagnosis of pulmonary coccy, 21% had other forms of progressive coccy, and 7% had meningeal disease. African-American children had the lowest proportion of primary pulmonary coccy (40%) and highest proportion of other progressive disease (48%).

Only 11% of hospitalized pediatric coccy patients were diagnosed with an immunocompromising condition, and 1% had diabetes. Twenty percent of hospitalized patients were re-admitted at least once, with a median length of stay of 7 days. During the period of study from 2000-2012, more than $149MM in total charges related to the hospitalizations was accrued.

During the period of study, there were 11 pediatric coccidioidomycosis-associated deaths. Of the 11 children who died, eight were male, two were younger than 2 years of age, three were 3-11 years old, and six were 12-17 years of age. Six children who died were Latino, two were African-American, two were Asian/Pacific Islander, and one child was white. Of the 11 deaths, three carried a death diagnosis of disseminated coccy, two had meningitis, two had pulmonary coccy, and four had “unspecified” coccidioidomycosis.

COMMENTARY

The reasons for the increase in coccidioidomycosis diagnoses in California during the past decade are unclear. This study sheds light on the epidemiology of coccy in the pediatric population. While many think of coccy as being a threat in patients residing in (or traveling through) the Central Valley, those of us practicing at referral centers in California have clearly seen many cases of coccy in patients who reside in areas of California not previously considered to be endemic for this disease. In my own limited personal experience caring for children with coccy, the diagnosis of coccy often is not considered by pediatricians early in the clinical course, and patients often have fairly severe disease by the time they are sent to us. This is certainly understandable since the initial clinical manifestations of this disease may be nonspecific. A recent review of 13 cases of coccy in infants (from Madera, in the highly endemic Central Valley) highlights the problem of delayed diagnosis of coccy in young children, even in an area where coccy is known to be endemic.1 This case series highlighted the severity of illness and disseminated disease. Many of these young children developed pleural and pericardial effusion, hilar and mediastinal adenopathy, lung abscess, neck abscess, and intracranial abscesses. One infant even developed depressed temporoparietal skull fracture related to osteomyelitis of the skull. The paper by Sondermeyer et al should be a good reminder to at least consider coccy in the differential diagnosis of sick children in almost all areas of California, as well as in those who had visited endemic areas.

 

We also need better, safer, and more effective treatment for CNS coccidioidomycosis in particular. Unfortunately, experimental animal models of CNS coccy are not highly predictive of human disease. Due to the relative rarity of CNS disease, large randomized, controlled trials of various drugs and treatment modalities (such as intrathecal amphotericin B) would be very difficult to conduct. I am still haunted by the memory of a beautiful 5-year-old child, whose case I helped manage about 6 years ago at our county hospital. This little boy developed severe CNS disease complicated by hydrocephalus and intracranial vasculitis with stroke. Despite neurosurgical intervention, systemic fluconazole, intrathecal amphotericin B, and systemic corticosteroids, the child died. I would have given just about anything to have been able to save this child’s life. 

REFERENCE

  1. Lee JM, et al. Coccidioidomycosis in infants: A retrospective case series. Pediatric Pulmonology 2016 [Epub ahead of print].