Background SCID is a syndrome characterized by profound T cell deficiency. BCG vaccine is contraindicated in SCID patients. Because most countries encourage BCG vaccination at birth, a high percent of SCID patients are vaccinated before their immune defect is detected. Objectives To describe the complications and risks associated with BCG vaccination in SCID patients. Methods An extensive standardized questionnaire evaluating complications, therapeutics, and outcome regarding BCG in patients diagnosed with SCID was widely distributed. Summary statistics and association analysis was performed. Results Data on 349 BCG vaccinated SCID patients from 28 centers in 17 countries was analyzed. Fifty-one percent of the patients developed BCG complications, 34% disseminated and 17% localized (a 33,000 and 400 fold increase, respectively, over the general population). Patients receiving early vaccination (≤ 1 month) showed an increased prevalence of complications (p=0.006) and death due to BCG complications (p<0.0001). The odds of experiencing complications among patients with T cells ≤ 250/uL at diagnosis was 2.1 times higher (95% CI, 1.4-3.4; p = 0.001) than among those with T cells > 250/uL. BCG complications were reported in 2/78 patients who received anti-mycobacterial therapy while asymptomatic and no deaths due to BCG complications occurred in this group. In contrast 46 BCG-associated deaths were reported among 160 patients treated with anti-mycobacterial therapy for a symptomatic BCG infection (p<0.0001). Conclusions BCG vaccine has a very high rate of complications in SCID patients, which increase morbidity and mortality rates. Until safer and more efficient anti-tuberculosis vaccines become available, delay in BCG vaccination should be considered to protect highly vulnerable populations from preventable complications.

These data suggest that BMI based on self-reported weight and height is not accurate for BMI prediction at an individual level. However, self-reported BMI may be used as a simple and valid tool for BMI estimates of overweight and obesity in epidemiological studies.

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Langerhans cell histiocytosis (LCH) is a rare disease of unknown origin with a heterogeneous clinical presentation, varying from benign and self-limited to lethal. It is classified as single or multisystemic, according to the number of organs involved (one or at least two, respectively). Diagnosis can be challenging and is based on the histological and immunophenotypic examination of affected tissues. Secondary haemophagocytic lymphohistiocytosis is rarely reported in association with LCH and may impair its diagnosis. Some authors suggest that the coexistence of the two disorders is more than coincidental. We present a case of multisystem LCH in a 5-month-old infant, with all risk organs involved, in which severity and rapid progression reflect an association with haemophagocytic syndrome.

A 14-year-old patient presented with bilateral pneumonia and pleural effusions, septic arthritis of the hip, deep venous thrombosis, and pulmonary thromboembolism. Methicillin-sensitive Staphylococcus aureus (S. aureus) containing the Panton Valentine Leukocidin (PVL) genes was isolated. Contraindication to anticoagulation prompted inferior vena cava filter placement. He completed 4 weeks of treatment with flucloxacillin, with good clinical outcome. S. aureus containing PVL genes should be sought in cases of necrotizing pneumonia as it seems to increase the risk of severe multifocal infection and thrombotic complications. There are few reports of placement of filters during S. aureus sepsis and bacteraemia. This case highlights that when anticoagulation is not feasible, an inferior vena cava filter can be inserted safely, even in patients with active sepsis and high risk for seeding of the filter. Long-term follow-up confirmed a successful outcome with sterilization of the septic thrombosis with no further pulmonary embolism or additional sepsis episodes.