Infant Botulism, Israel, 2007-2021.

Infant botulism (IB) is an intestinal toxemia that manifests as descending paralysis, constipation, and, in some cases, respiratory failure. Laboratory-confirmed IB cases are rare, and recent data in Israel are lacking. We conducted a national multicenter retrospective study of laboratory-confirmed IB cases reported in Israel during 2007-2021. A total of 8 cases were reported during the study period. During 2019-2021, incidence may have increased because of a cluster of 5 cases. Infant median age for diagnosis was 6.5 months, older than previously reported (3 months). Most cases occurred during March-July. Honey consumption was reported in 1 case, and possible environmental risk factors (living nearby rural or construction areas, dust exposure, and having a father who works as a farmer) were reported in 6 cases. Although IB is rare, its incidence in Israel may have increased over recent years, and its epidemiology and risk factors differ from cases reported previously in Israel.

[FUNCTIONAL ECHO IN PRETERM INFANTS - PAST, PRESENT AND FUTURE].

INTRODUCTION: The use of point of care ultrasound to assess cardiovascular function performed by a neonatologist who is not a cardiologist, is gaining interest in the neonatal intensive care unit (NICU). Clinical signs such as heart rate, blood pressure and capillary refill time provide limited insight into the adequacy of systemic blood flow and organ perfusion. Functional echocardiography (echo) enables real time evaluation of cardiac performance, identifying the nature of cardiovascular compromise, guiding therapeutic decisions and monitoring response to treatment. Application of functional echo in the NICU includes assessment of patent ductus arteriosus (PDA), pulmonary hypertension, shock and placement of umbilical catheters. There is evidence implicating that functional echo alters neonatal treatment and may improve outcome. Functional echo does not aim to replace the detailed structural assessments provided by consultative services of pediatric cardiologists. Close collaboration with pediatric cardiology is essential. In the past two years there is an increasing awareness for using functional echocardiography by neonatologists in Israel. Three conferences and an educational workshop were held in Israel with world renowned lecturers. Protocols defining how the exam should be performed were prepared, and agreement was reached with pediatric cardiologists on how to implement the use of functional echo by neonatologists, with the intention to create a distinct group of neonatologists with knowledge and skills in functional echo and a profound understanding of cardiac and respiratory physiology and hemodynamics.

Two New Cases of Bachmann-Bupp Syndrome Identified through the International Center for Polyamine Disorders.

Recent identification of four additional polyaminopathies, including Bachmann-Bupp syndrome, have benefited from previous research on Snyder-Robinson syndrome in order to advance from research to treatment more quickly. As a result of the discovery of these conditions, the potential for treatment within this pathway, and for other possible unidentified polyaminopathies, the International Center for Polyamine Disorders (ICPD) was created to help promote understanding of these conditions, research opportunities, and appropriate care for families. This case study provides insights from two new patients diagnosed with Bachmann-Bupp syndrome, further expanding our understanding of this ultra-rare condition, as well as a general discussion about other known polyaminopathies. This work also presents considerations for collaborative research efforts across these conditions, along with others that are likely to be identified in time, and outlines the role that the ICPD hopes to fill as more patients with these polyaminopathies continue to be identified and diagnosed.

Congenital cytomegalovirus infection and Wiskott-Aldrich syndrome successfully treated with unrelated cord blood transplantation.

We report a successful umbilical cord blood transplantation (UCBT) in an 8-month male with Wiskott-Aldrich syndrome (WAS) and congenital cytomegalovirus (CMV) infection. The child presented at 3 months of age with symptomatic thrombocytopenia and CMV infection. Despite appropriate antiviral treatment no rise in the platelet count was observed. Genetic analysis confirmed the diagnosis of WAS. The clinical course was complicated by severe CMV retinitis with bilateral retinal hemorrhages and renal vasculitis. He underwent unrelated UCBT resulting in a rapid resolution of autoimmunity and thrombocytopenia.

De novo neonatal antiphospholipid syndrome: a case report and review of the literature.

OBJECTIVES: Neonatal antiphospholipid syndrome (APS) is rare and only few cases have been reported, most of them describing trans-placental passage of penetrable maternal antibodies. The current article aims at defining the clinical and biochemical features of the de novo occurrence of neonatal APS and considerations for long-term treatment. METHODS: We present a case and review the medical literature using PubMed. RESULTS: Including the current report, there are 11 reports of de novo neonatal APS. These include 8 cases of acute ischemic stroke, 2 of venous thrombosis, and 1 of mixed arterial and venous thrombosis. All cases had an additional thrombotic risk factor, either inherited or acquired. Negative maternal history together with low clinical suspicion led to late diagnosis in most cases. In all cases aPL antibodies persisted in the serum throughout the follow-up period. No recurrence of thrombotic events was reported in patients under long-term anticoagulation with either low-molecular-weight heparin (LMWH) or aspirin. There is 1 report of recurrent thrombosis where the patient did not receive anticoagulation. In this case diagnosis was made only retrospectively after recurrence. CONCLUSIONS: We recommend that de novo APS be considered in all cases of unexplained neonatal thrombosis aiming at early diagnosis and implementation of long-term anticoagulation to prevent recurrent thrombotic events.

Safety and efficacy of a novel microneedle device for dose sparing intradermal influenza vaccination in healthy adults.

BACKGROUND: Intradermal vaccine delivery has been shown to induce good immune responses with low vaccine doses. Technologies for drug-delivery which specifically target the skin may render intradermal vaccination more accessible. METHODS: We conducted a prospective, randomized trial in 180 intended-to-treat healthy adults. Study objectives were to evaluate the safety and immunogenicity of low-dose intradermal (ID) influenza vaccines delivered using a novel microneedle device (MicronJet). This device replaces a conventional needle, and is designed specifically for intradermal delivery. Subjects were randomly assigned to receive either the full-dose standard flu shot (containing 15 microg hemagglutinin per strain) delivered intramuscularly using a conventional needle (IM group), a medium dose intradermal injection (6 microg hemagglutinin per strain) delivered with the MicronJet (ID2 group), or a low-dose intradermal injection (3 microg hemagglutinin per strain) delivered with the MicronJet (ID1 group). A marketed influenza vaccine for the 2006/2007 influenza season (alpha-RIX by GSK Biologicals) was used for all injections. Adverse events were recorded over a 42-day period. Immunogenicity was evaluated by changes in hemagglutination inhibition (HAI) antibody titer, and by comparing geometric mean titers (GMTs), seroconversion, and seroprotection rates between the study groups. RESULTS: Local reactions were significantly more frequent following intradermal vaccination, but were mild and transient in nature. At 21 days after injection, GMT fold increase was 22, 18 and 22 in the ID1, ID2 and IM groups respectively for the H1N1 strain; 9, 9 and 16 for the H3N2 strain and 9, 13 and 11 for strain B. The CPMP criteria for re-licensure of seasonal influenza vaccines were met in full for all study groups. CONCLUSIONS: Low-dose influenza vaccines delivered intradermally using microneedles elicited immunogenic responses similar to those elicited by the full-dose intramuscular vaccination. The microneedle injection device used in this study was found to be effective, safe, and reliable.