Elie Picard

Fatal pneumococcal sepsis following flexible bronchoscopy in an immunocompromised infant

A 5‐month‐old boy who suffered from a leukocyte chemotactic defect underwent flexible bronchoscopy for persistent right upper lobe atelectasis and tachypnea. Ten hours after the procedure he developed fulminant sepsis, and he died 16 hrs after bronchoscopy. Streptococcus pneumoniae (serotype 23) grew from the bronchoalveolar lavage fluid and from the blood culture taken during the sepsis work‐up. We, therefore, suggest administering prophylactic antimicrobial therapy immediately following bronchoscopy to immunosuppressed children, even when an acute respiratory infection is not suspected, in order to prevent bacteremia and sepsis. Pediatr Pulmonol. 1998; 25:390–392.

Evaluation of the intestinal current measurement method as a diagnostic test for cystic fibrosis

The sweat test and nasal potential difference measurement are now established tools in the diagnostic work up of cystic fibrosis (CF). Intestinal current measurement (ICM) is under consideration as an aid in the diagnosis of CF especially in young children. The aim of this study is to evaluate the diagnostic reliability of ICM.

Adrenal suppression in asthmatic children receiving low-dose inhaled budesonide: comparison between dry powder inhaler and pressurized metered-dose inhaler attached to a spacer

BACKGROUNDnDry powder inhalers (DPI) have in recent years become a common mode for administration of inhaled corticosteroids for preventive therapy of asthma. Inhaled steroids delivered by DPI achieve increased lung deposition compared with pressurized metered-dose inhalers (pMDI), which is associated with increased therapeutic effect. This may be associated with increased systemic absorption.nnnOBJECTIVEnThe purpose of this study was to evaluate the prevalence of adrenal suppression in children using low-dose budesonide given by DPI, as compared with pMDI attached to a large-volume spacer device (pMDI + spacer).nnnMETHODSnIn an open-labeled crossover study, 15 asthmatic children aged 5 to 15 years received 200 microg of inhaled budesonide twice daily by DPI (Turbuhaler, Astra, Draco AB, Lund, Sweden) and by pMDI + spacer, 1 month each, in a randomized order. Twenty-four-hour urine collections were performed at baseline and at the end of each of the 2 months of the study period, and urinary cortisol and creatinine were measured.nnnRESULTSnBaseline urinary cortisol:creatinine was 0.038 +/- 0.012 microg/mg, similar in both groups. After 1 month of DPI therapy, urinary cortisol:creatinine was reduced by 27 +/- 16% to 0.028 +/- 0.012 microg/mg (P = 0.018). Urinary cortisol:creatinine after 1 month of pMDI + spacer therapy was similar to baseline 0.037 +/- 0.019 microg/mg (P = 0.78).nnnCONCLUSIONSnTreatment of asthmatic children with budesonide 400 microg daily given via a DPI for 1 month was associated with hypothalamic-pituitary-adrenal axis suppression. This effect was not observed with the same dose of budesonide administered via pMDI + spacer. This indicates that systemic absorption might be reduced with pMDI + spacer therapy.

Thoracic Air Leakage in the Presentation of Cast Bronchitis

BACKGROUNDnCast bronchitis is a rare but potentially fatal condition caused by the accumulation of mucus in the bronchial tree usually secondary to infection, inflammation, or vascular stasis. Air leakage is an uncommon presentation of cast bronchitis. We report on three children with cast bronchitis who presented with acute air leakage as the main clinical feature.nnnMETHODSnAll three patients underwent bronchoscopic evaluation that led to a diagnosis and removal of bronchial casts.nnnRESULTSnPathologic examination of casts from all three patients showed a mucoid substance with eosinophilic infiltration. The outcome for all patients was favorable.nnnCONCLUSIONSnAcute respiratory failure presenting with wheezing and thoracic air leakage refractory to standard asthma therapy should raise suspicion of cast bronchitis, and urgent bronchoscopy should be performed.

Pulmonary fibrosarcoma in childhood: fiber-optic bronchoscopic diagnosis and review of the literature.

Primary pulmonary fibrosarcoma is a rare malignant tumor in childhood. In the absence of metastases, complete resection is curative. An 8‐year‐old boy suffered from unresolving pneumonia due to an obstructing lesion in the left main bronchus. Cytology of the bronchoalveolar lavage fluid and histology of bronchial biopsy revealed the diagnosis of pulmonary fibrosarcoma. The tumor did not respond to chemotherapy, and a total lobectomy with sleeve resection was performed with complete removal of the neoplasm. Two years after the operation the child has no evidence of disease. Pediatr Pulmonol. 1999; 27:347–350.

Newborn oxygen saturation at mild altitude versus sea level: implications for neonatal screening for critical congenital heart disease

To determine the normal SpO2 in healthy term newborns at mild altitude (MA, 780 metres) compared with sea level (SL), within the context of universal screening for critical congenital heart disease (CCHD).

Nasal potential difference in non-classic cystic fibrosis—long term follow up†

Nasal potential difference (NPD) measurement is an electrophysiological test that assesses cystic fibrosis transmembrane conductance regulator (CFTR) activity and is a recognized diagnostic tool in CF. The aim of this study is to assess in the long term the role of NPD in patients whose diagnosis is questionable.

Complicated community acquired pneumonia in childhood: Different types, clinical course, and outcome

The incidence of pediatric community acquired complicated pneumonia (PCACP) is increasing. Questions addressed: Are different types of PCACP one disease? How do different treatment protocols affect the outcome? Methods: Retrospective analysis of medical records of PCACP hospitalizations in the three major hospitals in Jerusalem in the years 2001–2010 for demographics, clinical presentation, management, and outcome. Results: Of the 144 children (51% aged 1–4 years), 91% of Jewish origin; 40% had para‐pneumonic effusion (PPE), 40% empyema (EMP), and 20% necrotizing pneumonia (NP). Bacterial origin was identified in 42% (empyema 79%, Pu2009=u20090.009), most common S. pneumoniae (32%), group A streptococcus (9%). Patients with EMP, compared to PPE and NP, were less likely to receive prior antibiotic treatment (35% vs. 57% and 59%, respectively, Pu2009=u20090.04). Mean hospitalization was longer in patients with NP followed by EMP and PPE (16.4u2009±u200910.6, 15.2u2009±u20097.9, and 12.7u2009±u20094.7 days, respectively), use of fibrinolysis was not associated with the outcome. All children had recovered to discharge regardless of antibiotic therapy or fibrinolysis. Answer: NP is a more severe disease with prolonged morbidity and hospitalization in spite of prior antibiotic treatment. All types had favorable outcome regardless of treatment‐protocol. Complicated pneumonia has an ethnic predominance. Pediatr Pulmonol. 2017;52:247–254.

Eradication failure of newly acquired Pseudomonas aeruginosa isolates in cystic fibrosis

Eradication of Pseudomonas aeruginosa (PA) is critical in cystic fibrosis (CF) patients.nnnOBJECTIVESnTo determine eradication success rate of newly acquired PA and to identify characteristics associated with eradication failure.nnnMETHODSnIn an observational study, data from patients with newly acquired PA infection from 2007 to 2013 were collected. Clinical variables were compared in patients with and without successful eradication for ≥1year.nnnRESULTSnOf 183 patients out of 740 (25%) from 7 CF Centers that had newly acquired PA, eradication succeeded in 72%. Patients with the highest risk of failure had multi-resistant PA, fewer sputum cultures taken, were older, and were diagnosed at a later age. The risk of eradication failure increased by 1.3% with each year of delayed CF diagnosis; successful eradication increased by 17% with each additional sputum culture taken.nnnCONCLUSIONSnDelayed detection of PA infection leading to delayed treatment and growth of multi-resistant organisms is associated with eradication failure.

The impact of a national population carrier screening program on cystic fibrosis birth rate and age at diagnosis: Implications for newborn screening

BACKGROUNDnPopulation carrier screening (PCS) has been available in Israel since 1999 and universally subsidized since 2008. We sought to evaluate its impact.nnnMETHODSnA retrospective review of governmental databanks, the national CF registry and CF centers.nnnRESULTSnCF rate per 100,000 live births has decreased from 14.5 in 1990 to 6 in 2011. From 2004-2011 there were 95 CF births: 22 utilized PCS; 68 (72%) had 2 known CFTR mutations; 37% were pancreatic sufficient. At diagnosis, age was 6 (0-98) months; 53/95 had respiratory symptoms, 41/95 failure to thrive and 19/95 pseudomonas. Thirty-four (36%) were Arabs and 19 (20%) orthodox Jews, compared to 20% and 8% respectively, in the general population.nnnCONCLUSIONSnPCS markedly reduced CF birth rates with a shift towards milder mutations, but was often avoided for cultural reasons. As children regularly have significant disease at diagnosis, we suggest a balanced approach, utilizing both PCS and newborn screening.