Incidence of whooping cough in Spain (1997–2010): an underreported disease

Whooping cough is currently the worst controlled vaccine-preventable disease in the majority of countries. In order to reduce its morbidity and mortality, it is essential to adapt vaccination programmes to data provided by epidemiological surveillance. A population-based retrospective epidemiological study to estimate the minimum annual undernotification rate of pertussis in Spain from 1997 to 2010 was performed. The incidence of pertussis cases reported to the National Notifiable Disease Surveillance System was compared with the incidence of hospital discharges for pertussis from the National Surveillance System for hospital data, Conjunto Mínimo Básico de Datos. The overall reported incidence and that of hospitalisation for whooping cough were 1.3 cases?×?100,000 inhabitants in both cases. Minimum underreporting oscillated between 3.8 and 22.8 %, according to the year of the study. The greatest underreporting (50 %) was observed in children under the age of 1 year. Conclusion: Spanish epidemiological surveillance system of pertussis should be improved with complementary active systems to ascertain the real incidence. Paediatricians and general practitioners should be sensibilized to the importance of notification because this would be essential for adapting the prevention and control measures of this disease.     

Extraintestinal manifestations in an infant with microvillus inclusion disease: complications or features of the disease?

Microvillus inclusion disease (MVID), a rare severe congenital enteropathy characterized by intracytoplasmic microvillous inclusions and variable brush border atrophy on intestinal epithelial cells histology, is associated with defective synthesis or abnormal function of the motor protein myosin Vb encoded by the MYO5B gene. Although MYO5B gene is expressed in all epithelial tissues, it is unclear so far whether organs other than intestine are affected in MVID patients. We report a case of an infant with MVID who presented liver dysfunction, hematuria, and Pneumocystis jiroveci pneumonia during the course of the disease. It is discussed whether extraintestinal manifestations in this patient are secondary consequences of MVID or might be features of the disease associated with altered MYO5B function. Conclusions: MVID is classically included in the differential diagnosis of congenital diarrhea of secretory type. Recent advances in our knowledge regarding the role of myosin Vb in the pathophysiology of MVID is expected to clarify the clinical spectrum of the disease and the possible primary involvement of organs other than intestine.     

N-acetylaspartylglutamate in Canavan disease: an adverse effector?

We measured N-acetylaspartate and its precursor/product N-acetylaspartylglutamate (NAAG) in urine of patients with Canavan disease using capillary zone electrophoresis. Abnormal levels of NAAG were found in 32 of 43 patients examined. Elevated NAAG was also present in the CSF of one patient. Given that NAAG may interfere with N-methyl-D-aspartate receptor function, the occurrence of high levels of NAAG in patients' urine conceivably represents a participating factor in the pathogenesis of Canavan disease. Conclusion The biochemical role of N-acetylaspartylglutamate and its relationship to glutamatergic function may be relevant to the pathogenesis of Canavan disease. Received: 18 May 1998 and in revised form: 20 August 1998 and 21 September 1998 / Accepted: 22 September 1998     

Surgical management of pediatric Graves’ disease: an effective definitive treatment

Purpose

The optimal treatment for pediatric Graves’ disease (GD) is controversial. Antithyroid drugs are often used initially, but they are associated with a high failure rate. Therefore alternative therapies have become important. In the present study, we analyze our institution’s experience regarding the safety and efficacy of thyroid surgery among pediatric patients with GD.

Methods

This is a retrospective chart review of 27 pediatric patients (age?≤?18?years) with GD who underwent thyroid surgery between 1991 and 2009 at a single academic Institution. We recorded preoperative, intraoperative, and short-term postoperative data.

Results

All 27 patients were initially treated with thionamides. The high rate of hyperthyroidism relapse after discontinuation of medical treatment, age?Conclusions Surgical therapy for pediatric GD performed by experienced thyroid surgeons is a safe, definitive and cost-effective treatment.     

Celiac disease in India: are they true cases of celiac disease?

BACKGROUND: In a developing country, many conditions other then celiac disease (CD) can give rise to villous atrophy. We therefore assessed the role of immunoglobulin A (IgA)-antigliadin antibody (AGA) in addition to the ESPGHAN criteria in the diagnosis of CD in 104 Indian children. METHODS: Consecutive children with suspected CD were evaluated over 3 years with an intention to diagnose CD. Complete hemogram, d-xylose absorption test, endoscopic duodenal biopsy, and IgA-AGA titers were performed in all. CD was diagnosed on the basis of modified ESPGHAN criteria irrespective of IgA-AGA positivity (>5 U/mL), and those diagnosed were put on gluten-free diet and were monitored regularly. Children with suspected CD who had a normal duodenal biopsy result were taken as controls. RESULTS: The mean age of 50 children with CD was 6.3 +/- 2.6 years, with a male to female ratio of 3:2. The mean duration of symptoms was 3.4 +/- 2.2 years. Predominant symptoms were pallor in 96%, failure to thrive in 92%, and diarrhea in 80%. On follow-up (19.6 +/- 8 months), symptoms subsided within 16 +/- 9.8 days, and patients showed significant weight gain (mean weight at diagnoses and at last follow-up visit were 66% and 86% of expected, respectively; P < 0.001) and height gain (mean height at diagnoses and at last follow-up visit were 88% and 94% of expected, respectively; P = nonsignificant). The control group comprised 47 children with a mean age of 6.9 +/- 3 years. On comparing CD with controls, diarrhea, anemia, low weight, and stunting were significantly (P < 0.001) more frequent in patients with CD. Sensitivity and specificity of AGA at a cutoff value of 5 U/mL were 94% and 91.5% and at 10 U/mL 88% and 100%, respectively. Follow-up AGA test was performed in 42 of 47 positive cases. All showed significant decrease in AGA titer, and 29 (70%) had a negative test result. CONCLUSIONS: Indian children with CD are true cases of CD. They present late, diarrhea is absent in 20% of cases, and AGA test results show 88% of children without false-positive results at a cutoff value of 10 U/mL. However, AGA test with 94% sensitivity at a cutoff value of 5 U/mL can be used as screening test to select suspected cases for further workup.     

Metabolic bone disease of prematurity – an overview

With improved survival of extreme preterm and very low birth weight infants, there has been an increased incidence of metabolic bone disease (MBD). The last couple of decades have seen better awareness of the condition which in turn has led to improvement in the management and prevention of MBD. MBD occurs because there is difficulty in matching intrauterine mineral accretion rate after birth. The lack of a single specific and sensitive diagnostic test frustrates early diagnosis in many babies. Investigations like dual energy X-ray absorptiometry (DEXA) and quantitative ultrasound scanning are helpful in establishing a diagnosis but currently used mostly in research studies. A pragmatic approach for paediatricians is to pre-empt the formal diagnosis and supplement those at risk with adequate minerals and vitamin D, whilst monitoring the blood markers of MBD. Despite the noticeable short-term complications and stunting effect on childhood height, the prognosis of MBD is generally good. The aim of this article is to provide the reader with an improved understanding of the aetiopathogenesis, and offer some practical guidance on when and how to investigate and manage the metabolic bone disease of prematurity.     

Tolerance: is it achievable in pediatric solid organ transplantation?

Significant advances have been made in the understanding of allograft rejection. There is growing awareness that allograft acceptance, or tolerance, is also an active process rather than a passive absence of rejection. Mechanistic awareness of this process has spawned many preclinical strategies for the prevention of allograft rejection without the need for chronic immunosuppression. These therapies are currently entering clinical trials. This article reviews the prevailing therapies that hold promise for future clinical application. In particular, their application in children is discussed, as are biologic aspects of childhood immunity that may play a role in the success or failure of these strategies.     

The Epidemiology of Kawasaki Disease in an Urban Hospital: Does African American Race Protect Against Coronary Artery Aneurysms?

The etiology and pathogenesis of Kawasaki disease (KD) is largely unknown. Certain demographic factors and laboratory findings are predictive of the development of coronary artery (CA) aneurysms. The objectives of this study were to determine the epidemiology of KD patients in an urban hospital and determine risk factors associated with their development of CA abnormalities. A longitudinal case series of KD patients admitted to Children’s National Medical Center from 1990 to 2002 was examined. Age, sex, ethnic background, duration of fever prior to diagnosis, address, month diagnosed, and CA abnormalities (ectasia or aneurysms) on echocardiography were recorded. Median household income was obtained from the U.S. Census Bureau Web site. The Student t-test, logistic regression analyses, and the Kruskal–Wallis test were used, with significance assumed at p < 0.05. A total of 302 patients were evaluated. CA abnormalties were found in 27 patients (9%), with aneurysms identified in 13 patients (4%). Age was 2.9 ± 2.4 years (range, 2 months to 14 years). A total of 51 patients (16%) were ≤1 year and 35 patients (12%) were ≥5 years. Ethnic distribution was 54% (164) African American, 24% (72) Caucasian, 9% (29) Asian/Pacific Islander, 8% (23) Hispanic, and 5% (14) Middle Eastern. Only 2/164 (1.2%) African Americans developed CA aneurysms. Neighborhood median income of the cohort was $45,400 ± $21,200 ($52,200 ±$25,800 for patients with aneurysms). A total of 28% of cases clustered between December and January. Cases doubled annually in 1999–2001 compared to 1990–1998 (39 vs 19). Multivariate logistic regression found age between 1 and 5 years [p = 0.045; odds ratio, 0.31; 95% confidence interval (CI), 0.10–0.97] and African American race (p = 0.014; odds ratio, 0.15; 95% CI, 0.03–0.68) to be independently protective against CA aneurysms. Duration of fever prior to diagnosis, considered in 210 patients, was different between patients with and without aneurysms (11 ± 5.3 vs 6.5 ± 3.8 days, respectively, p = 0.0007). Multivariate logistic regression found fever longer than 5 days to be the only predictive factor associated with the development of aneurysms and any abnormality. African Americans had a shorter duration of fever than the rest of the cohort (6.03 vs 7.31 days), (p = 0.0087). The epidemiology of KD at our hospital is similar to that at other centers except for the predominance of African Americans with a shorter duration of fever prior to diagnosis and a decreased incidence of CA aneurysms compared to other ethnicities. The protective nature of African American ethnicity against the development of CA aneurysms raises speculation about the role of genetics and its interaction with immunity in the pathogenesis of KD. Paper presented at the 2003 Pediatric Academic Societies/Society for Pediatric Research Meeting, Seattle, WA, USA.