Heterotaxy syndrome: defining contemporary disease trends.

The purpose of this study was to define a population of visceral heterotaxy and to investigate the incidence of bacterial sepsis in the current era of universal pediatric pneumococcal immunization. Pediatric echocardiography and radiology databases, along with electronic medical records, were searched for patients followed-up since birth between 1999 and 2009 with either asplenia or polysplenia and cardiac anatomy consistent with heterotaxy syndrome. A total of 29 patients were identified. Seven patients (24%) had a total of 8 sepsis events, and 6 patients (86%) developed sepsis while taking appropriately prescribed antibiotic prophylaxis. Of the patients with sepsis, 5 had polysplenia and 2 had asplenia. Sixty-two percent of sepsis events were nosocomially acquired. No cases of pneumococcal sepsis occurred after the introduction of the conjugated pneumococcal vaccination to the pediatric vaccination schedule. Bacterial sepsis was associated with a 44% mortality rate. An unexpected finding in 3 patients with visceral heterotaxy, asplenia, and an interrupted inferior vena cava (IVC) as the only anomaly on echocardiography was associated intestinal malrotation. Children with visceral heterotaxy remain at significant risk of life-threatening bacterial infection. In addition, the finding of interrupted IVC on echocardiography should prompt screening for intestinal malrotation, even in the absence of additional structural heart disease.

No demonstrable effect of benzathine penicillin on recurrence of rheumatic Fever in pacific island population.

Compliance with secondary antibiotic prophylaxis for acute rheumatic fever (ARF) should decrease the rate of recurrence; however, efficacy in a highly endemic area has not been studied. A retrospective chart review of patients <21 years old with a diagnosis of ARF in the Northern Mariana Islands was performed. Patient compliance with benzathine penicillin G (BPG) prophylaxis was assessed. One hundred forty-four patients with ARF were identified and considered eligible, and the recurrence rate was 38%. Mean level of compliance with BPG was 59% in patients with no recurrence of ARF and 57% in patients with recurrence of ARF. Level of compliance was not shown to be associated with odds of recurrence. There was a trend toward significance (p = 0.06), with those patients who had carditis at the time of diagnosis of ARF having higher odds of recurrence. A remarkably high recurrence rate of ARF was found in this population, but there was no difference in compliance with secondary antibiotic prophylaxis between those with and without recurrence of ARF. These findings stress the need to improve methods of primary prevention and secondary antibiotic prophylaxis for ARF.

The persistent challenge of rheumatic fever in the Northern Mariana Islands.

OBJECTIVES: Acute rheumatic fever (ARF) is a major cause of morbidity and mortality in developing nations. The objective of this study was to determine the disease burden of ARF among the indigenous and non-indigenous populations of the Northern Mariana Islands. METHODS: This was a chart review of all pediatric outpatients seen from 1984 to 2006 with ICD-9 codes corresponding to rheumatic fever or any rheumatic cardiac sequelae. The study was set in the only comprehensive inpatient facility and only public pediatric clinic in the Northern Mariana Islands. RESULTS: One hundred fifty-eight cases of ARF were identified. Age at diagnosis ranged from 2.9 to 17.1 years (median 10.6 years). Fever and carditis were the most common presenting findings. The average annualized incidence of ARF was 85.8 per 100,000 person-years for those aged 5-14 years. Sixty-six percent of patients with ARF were of Chamorro or Carolinian ancestry, despite comprising only 39% of the total population, with a combined average annualized incidence of ARF of 167 per 100,000 person-years. CONCLUSIONS: This is the first documentation of the incidence of pediatric ARF in the Northern Mariana Islands, delineating the large disease burden in the indigenous and other Pacific Island ethnic groups. Impediments to diagnosis and primary and secondary prevention were identified. The data provide strong support for the need for primary and secondary prevention of ARF.

Resident continuity clinic: a modest proposal.

Resident continuity clinics are an integral and mandated component of the pediatric core curriculum. Hospital-based and community-based settings have been traditionally used, with varied success. This article proposes the use and testing of an additional site, the primary care clinic housed at the Children's Clinic for Rehabilitative Services. This site provides concomitant continuity, and community and advocacy experiences for children with complex chronic conditions. Further study of the educational efficiency of this site is warranted.

Congenital lymphocytic choriomeningitis virus infection: spectrum of disease.

OBJECTIVE: Lymphocytic choriomeningitis virus (LCMV) is a human pathogen and an emerging neuroteratogen. When the infection occurs during pregnancy, the virus can target and damage the fetal brain and retina. We examined the spectrum of clinical presentations, neuroimaging findings, and clinical outcomes of children with congenital LCMV infection. METHODS: Twenty children with serologically confirmed congenital LCMV infection were identified. The children underwent neuroimaging studies and were followed prospectively for up to 11 years. RESULTS: All children with congenital LCMV infection had chorioretinitis and structural brain anomalies. However, the presenting clinical signs, severity of vision disturbance, nature and location of neuropathology, and character and severity of brain dysfunction varied substantially among cases. Neuroimaging abnormalities included microencephaly, periventricular calcifications, ventriculomegaly, pachygyria, cerebellar hypoplasia, porencephalic cysts, periventricular cysts, and hydrocephalus. The combination of microencephaly and periventricular calcifications was the most common neuroimaging abnormality, and all children with this combination had profound mental retardation, epilepsy, and cerebral palsy. However, others had less severe neuroimaging abnormalities and better outcomes. Some children had isolated cerebellar hypoplasia, with jitteriness as their presenting sign and ataxia as their principal long-term neurological dysfunction. INTERPRETATION: Congenital LCMV infection can have diverse presenting signs, neuroimaging abnormalities, and clinical outcomes. In the companion article to this study, we utilize an animal model to show that the clinical and pathological diversity in congenital LCMV infection is likely due to differences in the gestational timing of infection.

Virus de la coriomeningitis linfocitaria: nuevo teratógeno fetal / Lymphocytic choriomeningitis virus: new fetal teratogen

Lymphocytic choriomeningitis virus (LCMV) is a human zoonosis caused by a rodent-borne arenavirus. It has been associated with both postnatal, as well as in-utero, infection in man. Human infection is acquired after inhalation, ingestion or direct contact with the virus found in the urine, feces and saliva of infected mice, guinea pigs, and hamsters. Congenital LCMV infection should be a diagnostic consideration for infants and children who have chorioretinitis, microcephaly or macrocephaly, hydrocephalus, intracranial calcifications, or nonimmune hydrops fetalis. The diagnosis is made serologically via commercially available immunofluorescent antibody testing. Differentiation of congenital LCMV infection from congenital toxoplasmosis, rubella, cytomegalovirus, herpes simplex virus, enterovirus, human parvovirus B19 and syphilis should be made. Further research is necessary to determine the prevalence of this infection in human and rodent populations and to prospectively delineate the spectrum of congenital infection and its consequences. The medical profession, as well as veterinarians and pet shop owners, must educate the public regarding the hazard that wild, pet and laboratory rodents pose to pregnant women.

Use of complementary and alternative medical therapies among children with special health care needs in southern Arizona.

OBJECTIVE: The objective of this study was to assess the frequency and type of complementary and alternative medicine (CAM) therapies used by families of children with special health care needs in southern Arizona, as well as the correlates of their use. METHODS: Families of 376 children who were receiving services in a regional facility that serves children with special health care needs and were residing in southern Arizona were surveyed regarding CAM use. RESULTS: Sixty-four percent of these families reported using CAM for their child. The most common CAM therapies were spiritual healing/prayer/blessings. Of the conditions that were evaluated as correctable, the use rate was 24% as compared with a 76% use rate for children with a nonrepairable condition. Use of CAM for the child was strongly related to the use of CAM in the past by the family member who responded to the survey. The reasons that parents most frequently chose for using CAM were advice from a medical practitioner and advice from a family member. CONCLUSIONS: Use of CAM for children with special health care needs is common. Its frequency and type are significantly associated with the child's condition and prognosis.

Lymphocytic choriomeningitis virus: emerging fetal teratogen.

Lymphocytic choriomeningitis virus (LCMV), a rodent-borne arenavirus, is an often undiagnosed human fetal teratogen. We describe a neonate born with hydrocephalus and chorioretinitis after maternal second-trimester symptomatic LCMV infection. Previously reported affected infants are reviewed. We strongly suggest that obstetricians counsel their pregnant patients regarding the potential hazard that contact with infected pet, laboratory, and household mice and hamsters poses to pregnant women and their unborn children.

Aspergillus cholangitis: A late complication after Kasai portoenterostomy.

Ascending bacterial cholangitis can be a recurring complication in older children and adolescents who have undergone successful surgery for biliary atresia. The authors describe a 19-year-old girl with a history of recurrent late-onset, febrile cholangitis in whom persistent jaundice developed. Aspergillus terreus was isolated from her biliary fluid obtained at percutaneous transhepatic cholangiography. Despite prolonged external biliary drainage and systemic amphotericin B therapy, the fungus was not eliminated from her biliary system. Aspergillus cholangitis can occur in the portoenterostomy patient and may be highly resistant to conventional therapy.