Pediatric severe factor XI deficiency: A multicenter study.

BACKGROUND: Factor XI (FXI) deficiency is a rare autosomal recessive bleeding disorder. Only scarce publications address its clinical features in children. The increased prevalence of FXI deficiency in Israel enabled data collection for this large multicenter cohort study. OBJECTIVE: Some hemostatic challenges may be unique or more common in children, such as bleeding in the neonatal period or trauma-related injury. The current study was designed to explore the potential impact of these differences in children with severe FXI deficiency. METHODS: Medical files of all children with FXI level under 15% followed at five tertiary centers were evaluated. The retrieved data comprised demographic and clinical characteristics, including bleeding episodes, surgical interventions, treatment strategies, as well as laboratory features. RESULTS: Sixty children, whose median age at diagnosis was 4.2 years and their median FXI level was 4%, were included. Three children experienced triggered intracranial hemorrhage (ICH) and two children had major bleeds. No bleeding complications occurred in surgeries in which hemostatic treatment consisting mostly of tranexamic acid or fresh frozen plasma was applied (n = 45). In contrast, excessive bleeding was noted in 25% of surgical procedures performed without hemostatic preparation (p = .002). CONCLUSION: This study's findings confirm the generally favorable outcome of this rare bleeding disorder, with no spontaneous bleeds or cases of perinatal ICH. Nonetheless, proper diagnosis and adequate hemostasis in the surgical setting are imperative. Unlike previous studies in adults, our pediatric study suggests an association between the severity of FXI deficiency and bleeding tendency.

Factor VII Deficiency in Patients Receiving Chronic Packed Cell Transfusions.

Acquired factor VII deficiency is a rare coagulopathy that has not been reported in transfusion-dependent patients so far. In this study, we reviewed files of 26 transfusion-dependent patients for coagulation profiles, factor V levels, factor VII levels, possible environmental factors influencing factor VII levels, and bleeding history. In 26 of 29 patients (89.6%), we found mild factor VII deficiency (<60%) with levels ranging between 35% and 56%. Bleeding history was unremarkable. We concluded that transfusion-dependent patients may have mild factor VII deficiency with no bleeding tendency under physiologic conditions.

Mucormycosis in children with haematological malignancies is a salvageable disease: a report from the Israeli Study Group of Childhood Leukemia.

Mucormycosis has emerged as an increasingly important cause of morbidity and mortality in immunocompromised patients, but contemporary data in children are lacking. We conducted a nationwide multicentre study to investigate the characteristics of mucormycosis in children with haematological malignancies. The cohort included 39 children with mucormycosis: 25 of 1136 children (incidence 2·2%) with acute leukaemias prospectively enrolled in a centralized clinical registry in 2004-2017, and an additional 14 children with haematological malignancies identified by retrospective search of the databases of seven paediatric haematology centres. Ninety-two percent of mucormycosis cases occurred in patients with acute leukaemias. Mucormycosis was significantly associated with high-risk acute lymphoblastic leukaemia (OR 3·75; 95% CI 1·51-9·37; P = 0·004) and with increasing age (OR 3·58; 95% CI 1·24-9·77; P = 0·01). Fifteen patients (38%) died of mucormycosis. Rhinocerebral pattern was independently associated with improved 12-week survival (OR 9·43; 95% CI 1·47-60·66; P = 0·02) and relapsed underlying malignancy was associated with increased 12-week mortality (OR 6·42; 95% CI, 1·01-40·94; P = 0·05). In patients receiving frontline therapy for their malignancy (n = 24), one-year cumulative mucormycosis-related mortality was 21 ± 8% and five-year overall survival was 70 ± 8%. This largest paediatric population-based study of mucormycosis demonstrates that children receiving frontline therapy for their haematological malignancy are often salvageable.

Treatment of transfusion-dependent congenital dyserythropoietic anemia Type I patients with pegylated interferon alpha-2a.

OBJECTIVE: Pegylated IFN-α2a has been reported in two case reports as being efficacious in treating CDA-I patients. This study aims to assess its efficacy on a series of CDA-I patients. METHODS: Study sample consisted of seven CDA type 1 transfusion-dependent patients. They received pegylated interferon alpha-2a at an initial dose of 90-180 µg once a week, tapered according to clinical response and side effects. Good response was defined as Hb ≥ 10 g/dL for ≥3 months, partial response was defined as 7 ≤ Hb<10 g/dL for ≥3 months, and no response was defined as HB < 7 g/dL for over 3 months on treatment. Time to response was defined as the time needed to achieve hemoglobin levels ≥ 10 g/dL without transfusion. Patients were evaluated periodically by abdominal ultrasounds to rule out liver adenomas. RESULTS: Five patients (71%) had a good response to treatment. One patient stopped treatment due to side effects. One patient had partial response. One patient, with more severe phenotype and poor compliance, had poor response to treatment. No abnormal findings were found in ultrasound examination. No effect on serum ferritin level could be established. CONCLUSION: Pegylated interferon α2a therapy is efficacious in CDA-I patients with a reasonable safety profile.

Comparison of the etiologic, microbiologic, clinical and outcome characteristics of febrile vs. non-febrile neutropenia in hospitalized immunocompetent children.

We compared the etiologic, microbiologic, clinical, and outcome picture among febrile and non-febrile immunocompetent children hospitalized during 2013-2015 with acute neutropenia (absolute neutrophil count < 1.5 × 109/L). Serious bacterial infections (SBI) were defined as culture-positive blood, urine, cerebrospinal fluid, articular fluid or stool infections, pneumonia, brucellosis, and rickettsiosis. Overall, 664 children < 18 years of age were enrolled; 407 (62.2%) had fever > 38.0 °C and 247 (37.8%) were non-febrile at admission. There were 425 (64.0%), 125 (18.8%), 48 (7.2%), and 66 (9.9%) patients aged 0-24 months, 2-6, 7-12, and > 12 years, respectively. No differences were recorded in the distribution of febrile vs. non-febrile patients among the age groups nor among the 3 neutropenia severity groups (< 0.5, 0.5-1.0 and 1.0-1.5 × 109/L). SBI infections were diagnosed in 98 (14.8%) patients, with higher rates among febrile patients vs. non-febrile patients (16.8% vs. 11.5%, P = 0.06). Brucellosis and rickettsiosis were diagnosed in 15.4% and 23.1% tests performed, respectively. 295/688 (42.9%) virologic examinations returned positive. Among patients < 24 months, more febrile ones had viral infectious compared with afebrile patients (P = 0.025). Acute leukemia was diagnosed in 6 patients. Neutropenia resolved in 163/323 (50.5%) patients during a 1-month follow-up. No differences were recorded in neutropenia resolution between febrile and non-febrile children among all 3 severity groups. Severe neutropenia was rare and occurred mainly in very young patients. SBIs were more common among febrile patients compared with non-febrile patients, but there was no association between severity of neutropenia or its resolution and the presence or absence of fever at diagnosis.

Central venous catheter-associated bloodstream infections in children diagnosed with intestinal failure in Southern Israel.

OBJECTIVES: To study the clinical, epidemiological, and microbiological associations between intestinal failure (IF) and central line-associated infections (CLABSI) in patients with central vein catheters (CVCs) during 2005-2016. METHODS: We compared retrospectively CLABSI rates according to background disease, type of line access, pathogen distribution, and antibiotic susceptibilities. RESULTS: One hundred and fourteen children (64.1% < 4 years) were enrolled. Main diagnoses were persistent diarrhea (20, 17.5%), short bowel syndrome (13, 11.4%), continuous-TPN w/o diarrhea (11, 9.7%), very early-onset inflammatory bowel disease (VEO-IBD, 8, 7%), Hirschsprung's disease (3, 2.6%), non-oncologic hematologic conditions (13, 11.4%), and other diseases (46, 40.4%). 152.749 catheter days were recorded; 71.1% had Hickman's catheters. Two hundred and nine CLABSI episodes were recorded in 58 patients (82% with IF, 13.7 and 8.2/1000 catheter days in IF, and non-gastrointestinal conditions, P = 0.09). More CLABSI were recorded in continuous TPN vs. VEO-IBD or persistent diarrhea (38.8 vs.15.8 and 12.8/1000 catheter days, P < 0.004). Among patients with Hickman in jugular vein, highest CLBSI incidence was in continuous TPN, VEO-IBD, and persistent diarrhea (29.9, 15.84, and 12.49 episodes/1000 catheter days, respectively). CVCs were removed in 38.8% CLABSI. Two hundred and thirty-five pathogens were isolated (Enterobacteriaceae spp. in 39% of IF patients, mostly in persistent diarrhea and short bowel syndrome patients, 47.6% and 34.8%, respectively). Coagulase-negative Staphylococcus was the commonest pathogen in continuous TPN, VEO-IBD, and Hirschsprung's (71.4%, 55.6% and 46.1%, respectively). CONCLUSIONS: CLABSI rates in IF patients were among the highest reported. We reported a "hierarchy" in CLABSI incidence among patients with IF and showed that CLABSI incidence and etiology were different as function of background diseases and CVC insertion site.

Positive Outcomes of Denosumab Treatment in 2 Patients With Cherubism.

Cherubism is a rare autosomal dominant disease whose severity ranges widely, from asymptomatic to life-threatening. Bilateral symmetrical painless expansion of the mandible and maxilla resulting in a typical appearance of the face resembling a cherub, are the highlighted features of the condition. In most cases, cherubism-induced lesions in the jaws appear around the age of 3 years and tend to expand and increase in numbers until puberty. Treatment options for cherubism range from observation to surgical correction and various pharmacologic therapies. Given the excess sensitivity of cherubism osteoclasts to RANKL (receptor activator of nuclear factor κB ligand) and the positive effects of denosumab (XGEVA; Amgen, Thousand Oaks, CA) treatment in patients with giant cell granuloma, we have designed a treatment based on denosumab for 2 cherubism patients that achieves what we consider promising results.

Pediatric Carbon Monoxide Poisoning in Southern Israel: A Cross-Sectional Study.

OBJECTIVES: This study aimed to describe the epidemiology and clinical burden of unintended carbon monoxide (CO) poisoning among children in the Negev region of southern Israel. METHODS: This was a cross-sectional retrospective study of CO poisoning patients admitted to Soroka University Medical Center in 2011 through 2015. RESULTS: Overall, 43 cases of CO poisoning were recorded among children younger than 18 years. Five patients died, all upon admission. Poisoning due to smoke "per se" and due to CO emitted from heating devices were responsible for 28 (65.1%) and 14 (32.6%) cases, respectively. Eight (18.6%) patients suffered from convulsions, and 13 (43.3%) of 30 evaluable patients complained of headaches. Twenty-two (51.2%) were found unconscious in the field, and 7 (16.3%) were unconscious at examination at the emergency department. The average carboxyhemoglobin level on admission was 10.5% ± 10.4% (level ranging from 0.1% to 46.2%). Treatment included oxygen in 34 patients (79%) and hyperbaric oxygen therapy in 8 patients (19%). No differences were found between Bedouin and Jewish children in sex, age, residence area, source of CO poisoning, symptoms severity, and need for hyperbaric oxygen therapy. More patients with exposure to water heating devices were older than 4 years, lived in villages, and were diagnosed as having loss of consciousness in the field compared with those exposed to smoke inhalation. CONCLUSIONS: Carbon monoxide poisoning in children is frequent in southern Israel. Education about prevention, implementation of safer standards for home heating systems, and government supervision are required management strategies to decrease the CO poisoning incidence in southern Israel.

[PEDIATRIC ANIMAL-RELATED INJURIES IN THE JEWISH AND ARAB POPULATION IN SOUTHERN ISRAEL].

INTRODUCTION: Annually in the US, about 2 million patients who are animal-related casualties go to the Emergency Medicine Department (ER), which accounts for 0.5-1.5% of the ER visits; 25% of these casualties are children. Similar numbers are also seen in Europe and the UK. In a single-center study in Israel, it was found that the percentage of ER visits for animal injuries was about 2.1%. Soroka University Hospital is the only Level I trauma center in the Negev, serving a population of over 1,250,000, and as such it treats about 175,000 ER visits each year, of which about 27% are pediatric visits. The population treated in our hospital is diverse in terms of socioeconomic origin and status, and includes Jewish patients mainly situated in urban settlements and Arab-Bedouin population living in familiar and unfamiliar villages (the Bedouin diaspora). In light of this, in the present study, we have tried to investigate animal injuries in children, emphasizing scorpion bites which are common in our area. METHODS: We retrospectively reviewed the medical records of all children aged 0-17 years during an 18-month study period - from January 2009 until the end of June 2010 - to identify children who have been referred to the ER at the Soroka Hospital due to animal injury. Demographics, characteristics of the injury, incidence times and referral to treatment, and data on the course of assessment and treatment of the injury and hospitalization were recorded. Data was typed and processed using Microsoft Excel 2007 software and we used SPSS 23.0 for Windows for the statistical analysis. RESULTS: A total of 729 pediatric injuries caused by animals were reported, which accounted for about 1% of the total number of children's visits to the pediatric ER during the study period. Twenty-six cases were omitted because they did not meet the inclusion criteria in the study. Of the remaining 703 referrals, 66.1% of the patients were boys, the peak age was in the 6-8-year age group (26%); 44.1% lived in a Jewish settlement, 31.2% in an unrecognized Arab-Bedouin settlement and 24% in a recognized Arab-Bedouin settlement. In both forms of Bedouin settlements, the most frequent injuries were dog bites n = 166, (23.60%), yellow scorpion bites = 163 (23.2%) and black scorpion bites n = 44 (6.25%); 97 of the children were admitted (13.8%), of whom 44 were admitted to the ICU, with 84.1% of those admitted to the intensive care unit (ICU) were treated due to yellow scorpion injury. The 2-5-year age group was the most prevalent among inpatients, accounting for 32. DISCUSSION: Pediatric animal injuries are a common cause of ER visits and even hospitalization. A relatively high percentage of hospitalizations were in the ICU. The most common cause of animal injury seen in our cohort was dog bites, but yellow scorpion bites resulted in most hospitalizations, especially in the pediatric ICU. The incidence of yellow scorpion injuries was significantly higher in the Arab-Bedouin population and even more so in those living in unrecognized settlements, and 87% of the pediatric hospitalizations were seen in Arabic-Bedouin children. Animal-related injuries are a serious health problem in the Arab-Bedouin population and in the unrecognized villages in particular, and there is room to emphasize the prevention of these injuries in this impoverished sector.

Urinary tract infection in young infants discharged from the emergency room with normal urinalysis.

AIM: We describe the clinical, microbiologic, therapeutic, and outcome characteristics of infants under three months of age with a positive urine culture reported after discharge from emergency department with normal urinalysis. METHODS: We enrolled all infants with a urine culture obtained during an emergency room visit during 2004-2012, discharged without antibiotic therapy and subsequently reported with a positive urine culture. RESULTS: Three hundred and ninety-three positive urine cultures were reported; 46/393 (11.7%, 42 in patients under two months of age) had positive urine cultures following normal urinalysis at first visit. Fifteen (33%) had positive urine cultures at second visit; 11/15 (73%) infants with second positive urine culture were under one month of age, eight were asymptomatic and seven had mild symptoms at second visit. Pathogens isolated in all 15 infants were identical between first and second visit. All 27 infants re-examined at second visit at the emergency room were hospitalised, completed sepsis work/up and received antibiotic treatment. None developed serious bacterial infections. CONCLUSION: We propose a new management approach for young infants with normal urinalysis and positive urine culture and suggest restricting the management option including hospitalisation, sepsis work/up and antibiotic treatment at second visit only to infants under one month of age.

Clinical and Laboratory Findings in Jewish and Bedouin Patients in Southern Israel Who Were Diagnosed with Factor VII Deficiency.

BACKGROUND: Congenital factor VII deficiency is a rare recessive autosomal bleeding disorder with a wide spectrum of clinical manifestations. OBJECTIVES: To compare the clinical and laboratory findings in Jewish and Bedouin patients with factor VII deficiency. METHODS: The clinical and laboratory findings of patients with factor VII deficiency treated at Soroka Medical Center, a tertiary hospital in Israel, from 2005 to 2015 were analyzed regarding blood factor levels, illness severity, treatment administration, and disease outcome. RESULTS: Seventy-eight patients were enrolled (1:13,000 of the population in southern Israel) of whom 26 were diagnosed with severe factor VII deficiency (1:40,000). Sixty (76.9%) patients were Jewish and 18 (23.1%) were Bedouin. In univariable analysis, Bedouin patients exhibited a more severe illness, with significantly higher complication and fatality rates, and required more preventive treatment than the Jewish patients. CONCLUSIONS: The prevalence of congenital factor VII deficiency (including severe deficiency) in the Jewish and Bedouin populations of southern Israel is higher than previously reported. The clinical spectrum of the disease was found to be more severe in the Bedouin population.

[IS THERE STILL A PLACE TO USE ERYTHROCYTE SEDIMENTATION RATE IN THE 21ST CENTURY?]

INTRODUCTION: Erythrocyte sedimentation rate (ESR) is a simple test that is not without limitations but is still widely used. Dysfunction is used as a diagnostic test on the one hand and as a follow-up test on the other, where many physicians attribute normal results of this test to the absence of disease. Therefore, the question remains: Is there still room for continued ESR in the 21st century? And are there other tests with higher sensitivity and specificity that can replace the ESR? The purpose of the current review is to acquaint the reader with this test, its advantages and limitations, and to expand the scope of alternative tests and their effectiveness in comparison to ESR. It seems to us that the development of technology and research has strongly discouraged the use of ESR, but there are still quite a few instances where this test is still cost-effective. In most cases, especially during the diagnosis of life-threatening diseases, other tests are needed, or there is a need for a combination of the ESR with additional tests.

Spinal Epidural Hematoma Following Cupping Glass Treatment in an Infant With Hemophilia A.

A 6 months old infant, diagnosed with a rare mutation causing severe hemophilia A, presented with spinal epidural hematoma. Parents later admitted the infant had glass cupping therapy performed within 2 weeks of the onset of symptoms. The rare mutation, rare bleeding complication, and the eventual course of therapy applied in this case will be discussed in our case report.

Current data on acute haematogenous osteomyelitis in children in Southern Israel: epidemiology, microbiology, clinics and therapeutic consequences.

BACKGROUND: Acute haematogenous osteomyelities (AHO) is the most common form of osteomyelitis, occurring when bone is infected secondary to transient bacteremia. The prevalence, aetiology and outcome of AHO may vary from region to region and period to period. The study objectives were to define the epidemiology, clinical, laboratory and imaging characteristics and treatment consequences of AHO in children in southern Israel. METHODS: This was a retrospective cohort study, enrolling all children <16 years of age hospitalized with AHO. Epidemiologic, clinical, laboratory and imaging data were recorded from medical charts. RESULTS: Ninety-one patients were diagnosed with AHO (52.7 % <4 years of age). Most children (80.24 %) did not receive antibiotic treatment prior to diagnosis. During 2005-2012 the AHO incidence was 5.6:100,000; the AHO incidence in the Bedouin and Jewish population was 7.3 and 4.1:100,000, respectively. Fifty-four (57.8 %) patients were afebrile at admission and 34 (37.4 %) showed leukocytosis >15,000/mm(3). The most involved bone was tibia (39.6 %), followed by femur (19.8 %), humerus (8.8 %) and pelvis (8.8 %). Positive cultures were reported in 26 (28.6 %) patients. The most common pathogen was methicillin-susceptible Staphylococcus aureus (MSSA, 18 patients, 19.8 %). There was only one case of MRSA. More positive cultures were recorded among children requiring surgery compared to children treated conservatively (P < 0.01). MSSA representation in cases requiring surgical intervention was higher than in cases treated conservatively (P = 0.01). There were nine bone biopsies and 33 bone aspirations (MSSA in 44.4 % and 24.2 %, respectively). The longest hospitalization was observed in patients with humerus-AHO (14.8 ± 12.2 days). There was no difference in the number of days of hospitalization between patients who received previous antibiotics compared with children who did not receive antibiotics before admission. CONCLUSIONS: Tibia was the most frequently involved bone, but humeral AHO required more surgical intervention and longer hospitalization. Negative cultures were frequent, MSSA was the most commonly involved pathogen and MRSA was rare. Culture positive AHO was associated with higher requirement for surgical intervention.

Skin Necrosis and Purpura Fulminans in Children With and Without Thrombophilia--A Tertiary Center's Experience.

Purpura fulminans (PF) is a very rare clinicopathologic skin disorder comprising dermal microvascular thrombosis associated with perivascular hemorrhage of multiple origins. It may occur as the presenting symptom of severe congenital deficiency of protein C (PC) or protein S (PS) during the newborn period, or later in life following oral anticoagulant therapy with vitamin K antagonists, or of sepsis that may be associated with disseminated intravascular coagulation. Treatment consists of anticoagulants and PC concentrates during acute episodes. We report our experience in the diagnosis and management of pediatric PF. The medical records of the 6 children aged 2-16 years (median: 5 years) who presented with PF to our tertiary care center between 1996 and 2013 were studied. The thrombophilia workup revealed either the presence of congenital homozygous PC deficiency, prothrombotic polymorphisms (factor V Leiden and FIIG20210A heterozygosity), acquired PC/PS deficiency, or no discernible thrombophilia. The skin necrosis resolved following conservative fresh-frozen plasma/anticoagulant therapy in 2 cases, whereas 3 children required interventional plastic surgery. The sixth case, a 10-year-old child with severe PC deficiency, heterozygous factor V Leiden, and FIIG20210A, received recombinant activated PC. PF in childhood is rare and has multiple etiologies. Understanding of the variable pathogenesis and risk factors will facilitate diagnosis and appropriate clinical management.

Clinical and laboratory parameter dynamics as markers of blood stream infections in pediatric oncology patients with fever and neutropenia.

BACKGROUND: Identifying markers associated with blood stream infection (BSI) in children with fever and neutropenia (FN) could lead to a substantial reduction in unnecessary treatment. STUDY OBJECTIVE: The aim of this study was to determine the association between clinical/laboratory parameters and BSI in pediatric oncology patients with FN. METHODS: This prospective study was conducted between 2007 and 2010 at the Pediatric oncology unit. Clinical and laboratory parameters were obtained from all hospitalized FN patients. Linear regression and trends were calculated to determine the association between clinical/laboratory parameters and BSI. RESULTS: Of the 195 FN episodes in 73 children, BSIs were identified in 38 (19%) episodes. Gram-positive bacteria, gram-negative bacteria, and fungi caused 47%, 43%, and 10% of all BSIs, respectively. Mean fever duration was longer in the BSI group (5 d) compared with the non-BSI group (2 d, P=0.01). Mean (±SD) monocyte count at admission was lower in the BSI group compared with the non-BSI group (0.06±0.1 vs. 0.14±0.33 cells/mm, respectively, P=0.05). Mean C-reactive protein (CRP) levels at hospitalization days 5 to 8 were higher in children with BSI (P<0.001). Increment trends of monocyte and platelet levels and decrement trend of CRP levels were noted in the BSI group but not in the non-BSI group (P<0.01 for all). CONCLUSIONS: Prolonged fever, lower monocyte count at admission, higher CRP levels between the fifth and the eighth hospitalization days, increment trends of monocyte and platelet levels, and CRP level decrement were associated with BSI. These factors may serve as markers for BSI in pediatric oncology patients with FN.

Influenza A/H1N1 in pediatric oncology patients.

BACKGROUND: Our aim was to determine the clinical and epidemiological features of pandemic influenza A/H1N1 in immunocompromised children with solid tumors and hematological malignancies. PATIENTS AND METHODS: A prospective study was conducted during the H1N1 pandemic between August 2009 and February 2010 in a pediatric hematology-oncology unit. Demographic and clinical data were obtained from all children with suspected H1N1 infection (high fever with or without respiratory symptoms). Laboratory diagnosis of influenza A/H1N1 was performed by means of polymerase chain reaction analysis of nasopharyngeal wash specimens. RESULTS: We identified 57 episodes of suspected influenza A/H1N1 infection in 40 children. In all episodes, children were treated with oseltamivir and antibiotics until influenza A/H1N1 results were received. Of all episodes, 13 (22.8%) tested positive for influenza A/H1N1. Two of the H1N1-positive children (15.4%) had been previously immunized against influenza A/H1N1. No differences between H1N1-positive and H1N1-negative children were noted in terms of demographic features, clinical presentation, laboratory findings, and underlying disease.Three polymerase chain reaction-positive (23.0%) children and 1 H1N1-negative (2.3%) child were admitted to the pediatric intensive care unit and were mechanically ventilated (P=0.03). One (7.7%) H1N1-positive patient died versus none of the H1N1-negative patients (P=0.2). The condition of all other children in both the groups improved rapidly during hospitalization. CONCLUSIONS: Febrile hospitalized pediatric oncology patients, with and without pandemic influenza A/H1N1, had a similar demographic and clinical presentation with a relatively good outcome. This was probably because of early antiviral treatment and possibly because of the relatively low virulence of the virus. Immunization should be encouraged in these patients.

Morbidity characteristics of patients with congenital insensitivity to pain with anhidrosis (CIPA).

BACKGROUND: Congenital insensitivity to pain with anhidrosis (CIPA) is a congenital autonomic sensory neuropathy. In southern Israel, there are many patients with this disease. We here tried to characterize the different infections acquired by children with CIPA. METHODS: We collected all the available data about CIPA patients in southern Israel in the year 1991-2005, including the lesion types, area in the body where the infection occurs, and the treatment given. RESULTS: The current study included 30 children with CIPA, out of 44 known CIPA patients in southern Israel (68.2%). A total of 382 different episodes of infections, fever, orthopedic lesions, and jaw and mouth lesions led our patients to our outpatient clinic or resulted in hospitalization. CONCLUSION: We found that children with CIPA mainly have infections of the skin and skeleton, and that the most frequent pathogen is Staphylococcus aureus. We also found that a fair amount of these pathogens are resistant to conventional treatment regimens.

[Microbiological characteristics of pathogens causing bacteremia among hospitalized pediatric oncology patients with fever and neutropenia].

BACKGROUND: Bacterial infections are a major threat to pediatric oncology patients with fever and neutropenia. Current management consists of empiric broad-spectrum antibiotics and prompt medical evaluation. Local bacterial susceptibility rates were published in 2005, and the local protocol (piperacillin and amikacin) was established as an adequate empiric treatment with -100% efficiency against the common pathogens in our pediatric hemato-oncology ward. AIM: To characterize the spectrum of bacteria isolated from blood cultures at the pediatric hemato-oncology ward between 2008- 2010, and to evaluate the current protocol. METHODS: A prospective study, conducted in the pediatric hemato-oncologic ward among hospitalized children (2 months - 18 years) with fever and neutropenia. Blood cultures from peripheral blood and central lines were obtained from all patients at admission. Bacterial resistance to various antimicrobial agents was determined. RESULTS: During 2008-2010, 195 admissions (105 children) due to fever and neutropenia were recorded. Approximately 30% of all blood cultures were positive for a pathogen with -50% Gram positive bacteria mostly CONS. The most prevalent Gram negative bacteria were acinetobacter and klebsiella spp. Candida species were isolated from 7% of positive cultures. Susceptibility rates for the current empiric antimicrobial regimen were about 90%. CONS bacteremia rate increased from 4% in 2000-2002 to 29% in 2008-2010 (p < 0.01). CONCLUSIONS: The currently applied empiric antimicrobial protocol is an optimal first line regimen, considering the susceptibility of the most common pathogens. Judicious use of carbapenems for gram negative bacteria and glycopeptides or other novel antimicrobial agents in cases of CONS bacteremia is required.