Israel Medical Association Journal

Background: Several studies link the pathogenesis of nephrotic syndrome to tumor necrosis factor-alpha (TNFα). However, data on the serum TNFα level in children with nephrotic syndrome are sparse. 

Objective: To investigate serum TNFα levels and the effect of steroid therapy in children with nephrotic syndrome. 

Methods: A prospective cohort pilot study of children with nephrotic syndrome and controls was conducted during a 1 year period. Serum TNFα levels were measured at presentation and at remission, or after a minimum of 80 days if remission was not achieved.

Results: Thirteen patients aged 2–16 years with nephrotic syndrome were compared with 12 control subjects. Seven patients had steroid-sensitive and six had steroid-resistant nephrotic syndrome. Mean baseline serum TNFα level was significantly higher in the steroid-resistant nephrotic syndrome patients than the controls (6.13 pg/ml vs. 4.36 pg/ml, P = 0.0483). Mean post-treatment TNFα level was significantly higher in the steroid-resistant than in the steroid-sensitive nephrotic syndrome patients (5.67 pg/ml vs. 2.14 pg/ml, P = 0.001). In the steroid-resistant nephrotic syndrome patients, mean serum TNFα levels were similar before and after treatment.

Conclusions: Elevated serum TNFα levels are associated with a lack of response to corticosteroids. Further studies are needed to investigate the role of TNFα in the pathogenesis of nephrotic syndrome.

 

Objective: To assess the ability of TREC and KREC counts to identify patients with combined T and B cell immunodeficiency in a pilot study in Israel.

Methods: We studied seven children born in Israel during the years 2010–2011 and later diagnosed with SCID, and an additional patient with pure B cell immunodeficiency. TREC and KREC in peripheral blood upon diagnosis and in their neonatal Guthrie cards were analyzed using real-time quantitative polymerase chain reaction, as were Guthrie cards with dried blood spots from healthy newborns and from normal and SCID-like controls.

Results: The first features suggestive of SCID presented at age 3.1 ± 2.4 months in all patients. Yet, the diagnosis was made 4.1 ± 2.9 months later. Their TREC were undetectable or significantly low at their clinical diagnosis and in their originally stored Guthrie cards, irrespective of the amount of their circulating T cells. KREC were undetectable in six SCID patients who displayed B cell lymphopenia in addition to T cell lymphopenia. KREC were also undetectable in one patient with pure B cell immunodeficiency.

Conclusions: TREC and KREC quantification are useful screening tests for severe T and B cell immunodeficiency. Implementation of these tests is highly important especially in countries such as Israel where a high frequency of consanguinity is known to exist. 

Background: Patients with allergy as well as their parents frequently fail to use the self-administered epinephrine injection (EpiPen®) properly in cases of allergic emergencies.

Objectives: To determine the benefit of an instruction session with follow-up instruction.

Methods: We evaluated 141 patients aged 1.9–23.4 years (median 5.8 years, 83% with food allergy) or their parents (for those aged < 12 years) who were trained in the use of the EpiPen during the first diagnostic visit to the allergy clinic during 2006–2009. At the next follow-up visit, the patients or their parents were asked to list the indications for epinephrine administration and to demonstrate the five steps involved in using the EpiPen. Each step was scored on a scale of 0–2.

Results: Fourteen participants (9.9%) had used self-injectable epinephrine in the past. Only 65 (46%) brought the device with them to the follow-up visit. The mean total score for the whole sample was 4.03 ± 3. Fifty-three participants (38%) failed to remove the cap before trying to apply the device. Only 8 (5.6%) had a maximum score. The patients and their parents were reinstructed in the use of the device: 41 participants were reexamined at a subsequent follow-up visit after 1.02 ± 0.56 years their mean score improved from 4.71 ± 3.04 to 6.73 ± 3.18 (P < 0.001).

Conclusions: Patients with severe allergic reactions, as well as their parents, are not sufficiently skilled in the use of the EpiPen after only one instruction session with a specialist. Repeated instruction may improve the results and we therefore recommend that the instructions be repeated at every follow-up visit.

Background: Secondary thrombocytosis is associated with a variety of clinical conditions, one of which is lower respiratory tract infection. However, reports on thrombocytosis induced by viral infections are scarce.

Objectives: To assess the rate of thrombocytosis (platelet count > 500 x 10⁹/L) in hospitalized infants with bronchiolitis and to investigate its potential role as an early marker of respiratory syncytial virus infection.

Methods: Clinical data on 469 infants aged ≤ 4 months who were hospitalized for bronchiolitis were collected prospectively and compared between RSV[1]-positive and RSV-negative infants.

Results: The rate of thrombocytosis was significantly higher in RSV-positive than RSV-negative infants (41.3% vs. 29.2%, P = 0.031). The odds ratio of an infant with bronchiolitis and thrombocytosis to have a positive RSV infection compared to an infant with bronchiolitis and a normal platelet count was 1.7 (P = 0.023, 95% confidence interval 1.07–2.72). There was no significant difference in mean platelet count between the two groups.

Conclusions: RSV-positive bronchiolitis in hospitalized young infants is associated with thrombocytosis.

Objective: To determine breast-feeding patterns and the association between sociodemographic characteristics and breast-feeding in the Tel Aviv district.

Methods: The mothers of infants aged 2, 4, 6 and 12 months, attending 59 well-baby clinics in the Tel Aviv district, were interviewed by telephone. Singleton infants who weighed less than 2,000 g and multiple-gestation infants were excluded from the study. The questions covered background data, sociodemographic characteristics of the family, and breast-feeding practices. Stepwise logistic regression was used to analyze the association between breast-feeding and various sociodemographic characteristics.

Results: Altogether, 78.5% of the mothers (1,307/1,665) initiated breast-feeding. The rate of breast-feeding at 2, 4, 6 and 12 months was 55.8, 36.8, 29.9 and 11.8%, respectively. Only 35.8% of the infants at 2 months and 11.2% at 6 months were exclusively breast-fed. The mean duration of breast-feeding was 5.2 ± 0.2 months. Grand multiparas (≥5 children) had a significantly higher rate of breast-feeding than women with one to four children (P < 0.001). More likely to breast-feed for 2 weeks or longer were women married to Yeshiva students (odds ratio = 5.3), women with ≥13 years education (OR[1] = 2.1), and women on maternity leave (OR = 1.6). The predictors for breast-feeding for 6 months or longer were similar.

Conclusions: Although the rate of breast-feeding initiation in central Israel was 78.5%, only 29.9% of the mothers continue to breast-feed for 6 months. Already at a young age, an appreciable number of breast-fed infants receive infant formula. Breast-feeding promotion should focus on less educated women, homemakers, and families with one to four children.

Background: Visceral leishmaniasis was first reported in Israel (then Palestine) in 1929. In the 1960s and 1970s, it was endemic to northern Israel, but only partial data about the disease have been gathered since then.

Objective: To investigate the epidemiologic trends of visceral leishmaniasis in Israel from 1960 to 2000, and to delineate some clinical features of the infection.

Methods: Data were collected from hospital charts, scientific publications, and reports of the Ministry of Health and the Kuvin Center for the Study of Infectious and Tropical Diseases.

Results: During the last four decades, 87 cases of visceral leishmaniasis were diagnosed in Israel, 76 of them (87%) in children. All 54 patients diagnosed in the 1960s occurred in the northern part of the country. The rate of infection declined significantly in the 1970s (5 cases) and then increased slightly in the 1980s (11 cases) and 1990s (17 cases). More than 50% of the cases in the 1990s were in central Israel. Children accounted for 100% of cases in the 1960s but only 58% in the 1990s. The main clinical features of the patients diagnosed in the last decade were fever, weight loss, hepatosplenomegaly and pancytopenia. Three of the adults were co-infected with human immunodeficiency virus.

Discussion: The decline in the incidence of visceral leishmaniasis in the 1970s and the slight increase in the 1980s and 1990s can be attributed to changes in the animal reservoir and vectors, and in the immunity status of part of the population exposed to Leishmania.

Conclusions: Visceral leishmaniasis has reemerged in Israel. This mandates better control of the animal reservoir and vectors and increased awareness of this infection.

Background: Inflammation is a major component in the pathogenesis of asthma. CD14 is an endotoxin (lipopolysaccharide) receptor, and is expressed mainly on monocytes and macrophages. Binding of LPS to CD14 activates the monocyte or macrophage and causes the release of different cytokines.  The soluble form of CD14 is present in serum, and its concentration increases in several clinical conditions, including infections, auto-immune disorders, allergic disorders, and lung diseases.  The possible role of CD14/sCD14 in asthma has been investigated in a few adult patients only.

Objectives: To measure serum concentrations of sCD14 in children with status asthmaticus.

Methods: We compared serum concentration of sCD14 in 10 children with status asthmaticus measured within 24 hours of admission and after recovery from the acute episode.

Results: Levels of sCD14 were significantly higher during acute asthma attacks than at recovery.

Conclusions: The elevated serum levels of sCD14 during status asthmaticus may be the result of the activation of monocytes, macrophages or other cells.  The influence of medications on serum sCD14 cannot be ruled out.  The possible use of sCD14 as a marker of lung inflammation in asthma warrants further investigation. 

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LPS= lipopolysaccharide

SCD14= soluble form of CD14