Oded Scheuerman

Invasive Kingella kingae Infections in Children: Clinical and Laboratory Characteristics

OBJECTIVE. Kingella kingae, a Gram-negative coccobacillus, is being increasingly recognized as an invasive pathogen in children, causing mainly bacteremia and arthritis; however, there have been only a few studies on K kingae infections to date, mostly small-scale series. The aim of this study was to report our experience with invasive K kingae infections in children who were hospitalized at a major tertiary medical center in Israel. METHODS. The medical charts of 62 children with proven invasive K kingae infections were reviewed: 42 with positive blood culture results and 20 with positive synovial fluid culture results. RESULTS. Most infections occurred among previously healthy children aged 5 to 22 months. Eighty percent had a mild concurrent illness of the respiratory or gastrointestinal tract. A chronic underlying disease was documented in 19% of the 1- to 15-year-old children with bacteremia. Three patients had persistent bacteremia, identified by 2 positive blood cultures drawn 1 to 4 days apart. Four (10%) patients from the bacteremia group had endocarditis, and 2 required emergency cardiac surgery. Only a mild-to-moderate elevation of serum inflammatory markers was noted except for patients with endocarditis or a prolonged course of arthritis. Patients with bacteremia received a diagnosis significantly later than those with arthritis, with no other between-group differences in age, month of disease onset, and inflammatory marker levels. All K kingae isolates were resistant to vancomycin and clindamycin. CONCLUSIONS. Our large series indicates that invasive K kingae infections occur in previously healthy children, mostly during the first 2 years of life; affected older children usually have an underlying medical condition. The infection generally elicits only a mild inflammatory response unless accompanied by endocarditis. Despite its low virulence, K kingae might cause a life-threatening heart disease that requires emergent, aggressive treatment.

Leukocyte Adhesion Deficiency Type II: Long-Term Follow-Up and Review of the Literature

IntroductionLeukocyte adhesion deficiency (LAD) is a group of rare inherited disorders characterized by immune deficiency and peripheral neutrophilia. There are only seven reported cases of LAD type II worldwide, and no long-term follow-up data.Case ReportWe reviewed the medical file of a 20-year-old man with LAD II. Clinical characteristics included short stature, severe mental retardation, and autistic features. He had had no severe infections since infancy, and his current immunological status was stable. The last laboratory work-up revealed mild leukocytosis and neutrophilia. Genetic analysis of the Golgi GDP-fucose transporter (GFTP) sequence yielded a point mutation resulting in Y337C amino acid transition in the tenth transmembrane domain.ConclusionIn conclusion, in LAD II, the main clinical countenance shifts from frequent infections due to immunodeficiency in the early years to the metabolic consequences of the defect in fucose metabolism, i.e., retarded growth and mental retardation, in the later years. A novel mutation in the GFTP loci associated with LAD II is described.

The September epidemic of asthma in Israel.

Background. The seasonality of asthma morbidity is well recognized. A peak in asthma exacerbations in September has been noted for years at our center. Objective. To examine the hypothesis that the increment in asthma exacerbations in September is influenced by the beginning of the kindergarten and school year. Methods. The monthly admission rate for asthma in patients of different ages was retrospectively evaluated in seven hospitals from various areas in Israel from January 2003 to December 2005. Results. Of the 408,242 hospital admissions during the study period, 8,011 were for asthma exacerbations: 4,091 in adults (1.3% of adult admissions) and 3,920 in children (3.8% of pediatric admissions). The asthma admission rates varied considerably throughout the year, with a peak of 4% of total admissions in the winter months and a nadir of 2% in the summer months. September was unique for its particularly high rate of admissions for asthma attacks in children (6% of total admissions), especially toddlers and the school-age group. In adults there was a progressive increase in asthma admissions from September through December without a unique peak in September. Conclusions. There is a characteristic increase in asthma exacerbations and admissions in September in the pediatric age group. This phenomenon might be explained by the increased exposure to respiratory viruses, to new allergen exposure in school or kindergarten, increased emotional stress due to start of the new school year, or poor compliance and withdrawal of treatment during the summer. Clinicians should consider administering prophylactic treatment for asthma in children before onset of the school year.

Fusobacterium necrophorum mastoiditis in children – Emerging pathogen in an old disease

BACKGROUND Anaerobic bacteria are uncommon etiologic agents of acute mastoiditis in children. However, recent studies suggest an increase in the incidence of Fusobacterium necrophorum mastoid infections in the last two decades. METHODS A surveillance study performed over 3.5 years in a tertiary pediatric medical center identified 7 children with acute F. necrophorum mastoiditis. Clinical, laboratory, and treatment data were collected by file review. RESULTS Five of the 7 children presented in the last year of the study. All 7 children were less than 26 months old on admission, and none had a history of otogenic infections. All cases were characterized by significantly elevated levels of inflammatory markers. All were diagnosed as complicated mastoiditis with abscess formation. Four children had an epidural abscess, three children had evidence of osteomyelitis beyond the mastoid bone, and four children had imaging evidence of sinus vein thrombosis. All seven children required cortical mastoidectomy with ventilatory tubes insertion and two children required more than one surgical intervention. During follow-up, two children had recurrent episodes of mastoiditis due to other pathogens. CONCLUSION Our data support the literature suggesting that the occurrence of F. necrophorum mastoiditis among children is rising. Acute coalescent mastoiditis due to F. necrophorum is associated with a complicated course and warrants particular attention by pediatricians, infectious disease experts, and ear, nose and throat specialists.

Acute Cerebellitis Presenting as Tonsillar Herniation and Hydrocephalus

Acute cerebellitis is a rare inflammatory syndrome, often characterized by cerebellar dysfunction of rapid onset. We describe two children, a girl aged 5 years and a boy aged 11 years, with acute cerebellitis. Both manifested tonsillar herniation and hydrocephalus. The cause was Mycoplasma pneumoniae infection in the first patient, and unknown in the second. The girl was treated conservatively with steroids and diuretics, as well as vibramycin, and the boy required insertion of a ventriculostomy. Both presented a good prognosis and full recovery.

Endocarditis After Closure of Ventricular Septal Defect by Transcatheter Device

Advances in interventional cardiology have enabled the treatment of severe congenital heart defects without the need for surgery. The percutaneous closure of atrial septal defects and, more recently, ventricular septal defects is considered a safe procedure with fewer complications and less morbidity compared with surgery. We report on a 2-year-old child who developed endocarditis after ventricular septal defect closure with an Amplatzer device. The patient recovered after intravenous antibiotics and anticoagulation. To the best of our knowledge, this is the first report of endocarditis associated with ventricular septal defect closure device insertion.

Acute Hemorrhagic Edema of Infancy Associated with Herpes Simplex Type 1 Stomatitis

Abstract:  Acute hemorrhagic edema of infancy is a benign leukocytoclastic vasculitis occurring in children younger than 2 years. The etiology is unknown. Viral or bacterial infections, immunizations, and the use of several medications, mainly antibiotics, may be involved in the pathogenesis. We report the first instance of this disease associated with herpesvirus type 1 stomatitis.

Mitochondrial Trifunctional Protein Deficiency With Recurrent Rhabdomyolysis

Rhabdomyolysis is an important clinical diagnosis. The differential diagnosis is extensive and includes various etiologies, such as infection, inflammation, trauma, endocrinopathies, and congenital muscular and metabolic disorders. Reported here is the case of an infant with recurrent rhabdomyolysis diagnosed as suffering from mitochondrial trifunctional protein deficiency -- a rare beta oxidation defect. The clinical course was unique, and a new mutation in the mitochondrial trifunctional protein gene was identified.

Post-varicella thrombocytopenic purpura.

Aims:  The aim of the study was to characterize the clinical course of post‐varicella idiopathic thrombocytopenic purpura (ITP) and to asses the risk of acquiring ITP after varicella infection.

Human metapneumovirus (hMPV) infection in immunocompromised children.

BACKGROUND Human metapneumovirus (hMPV) is a major cause of upper and lower respiratory tract infection (URTI, LRTI) in children. The prognosis of hMPV is unclear in immunocompromised patients. OBJECTIVES To describe the characteristics of hMPV infection in immunocompromised pediatric patients and to review the literature. STUDY DESIGN This retrospective study included 39 immunocompromised children (age 0-18 years) with proven hMPV infection attending two tertiary pediatric medical centers in 2004-2014. Demographic, clinical, laboratory, and radiological data were collected from the medical files. RESULTS Median age was 6 years. Seven patients had primary immune deficiency and 32, secondary immune deficiency, including 9 patients who underwent hematopoietic stem cell transplantation (HSCT). Most cases (92%) occurred in January-May. Twenty patients (51%) had lower respiratory tract infection and 17 (44%), upper respiratory tract infection; 2 patients (5%) had fever only. Presenting symptoms were fever (70%), cough (54%), and rhinorrhea (35%). Severe lymphopenia (<1000lymphocytes/mL) was noted in 64% of patients and elevated liver enzyme levels in 49%. Seventeen patients had pneumonia: bilateral and alveolar in 13 patients, each. HSCT was not associated with more severe disease. Respiratory failure occurred in 6 patients, of whom 4 died (10% of cohort). All children who died had severe lymphopenia. On multivariate analysis, bacterial or fungal co-infection was the only major risk factor for death. Review of the literature showed variable clinical presentations and severity in pediatric patients with hMPV infection. CONCLUSIONS Infection with hMPV may be associated with relatively high morbidity and mortality in immunocompromised children. Death was associated with bacterial and fungal co-infection.