Shmuel Goldberg

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.

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.

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).

Does sweat volume influence the sweat test result

Objective Low volume sweat samples are considered unreliable for the diagnosis of cystic fibrosis, based on the assertion that sweat conductivity and chloride are reduced at lower sweating rates. We aimed to re-evaluate the relationship between sweat volume and test results. Design We reviewed all sweat tests performed in our institution to assess the relationship between sweat volume and conductivity, and between sweat volume and sweat chloride. We also compared results between pairs of sweat tests taken simultaneously from a single patient, one with sweat volume below and the other above the currently accepted minimum volume (15 µl). Results A weak inverse relationship between sweat volume and sweat conductivity was found (n=1500, R2=0.105, p<0.001). There was no correlation between sweat volume and sweat chloride (n=463, R2=0.002, p>0.05). In discordant pairs (one below and one exceeding the accepted minimum volume), the mean test result in the low volume sample was slightly higher than its counterpart. In 76 such pairs, mean conductivity was 41.1±14.6 mmol/l in the lower volume sample, compared with 36.8±16.0 mmol/l in the higher volume sample (p<0.001). Similarly, in 33 of the pairs, mean sweat chloride was 28.4±15.7 mmol/l in the lower volume sample compared with 25.1±15.2 mmol/l in the higher volume sample (p=0.004). Conclusion A normal sweat conductivity and/or chloride value from a sweat volume <15 µl in a patient whose clinical symptoms are not very suggestive of cystic fibrosis, renders this diagnosis unlikely. In contrast, elevated sweat chloride or conductivity measured from a sample whose volume is <15 µl may represent an artefact related to the low volume.

Collecting clinical data in primary ciliary dyskinesia- challenges and opportunities

Rationale: Primary ciliary dyskinesia (PCD) is under diagnosed and underestimated. Most clinical research has used some form of questionnaires to capture data but none has been critically evaluated particularly with respect to its end-user feasibility and utility. Objective: To critically appraise a clinical data collection questionnaire for PCD used in a large national PCD consortium in order to apply conclusions in future PCD research. Methods: We describe the development, validation and revision process of a clinical questionnaire for PCD and its evaluation during a national clinical PCD study with respect to data collection and analysis, initial completion rates and user feedback. Results: 14 centers participating in the consortium successfully completed the revised version of the questionnaire for 173 patients with various completion rates for various items. While content and internal consistency analysis demonstrated validity, there were methodological deficiencies impacting completion rates and end-user utility. These deficiencies were addressed resulting in a more valid questionnaire. Conclusions: Our experience may be useful for future clinical research in PCD. Based on the feedback collected on the questionnaire through analysis of completion rates, judgmental analysis of the content, and feedback from experts and end users, we suggest a practicable framework for development of similar tools for various future PCD research.

Methacholine challenge test results in children are season dependent.

BackgroundIt is known that several parameters influence the positivity of a methacholine challenge (MCH), including a recent viral disease, allergies, and air pollution. Reports regarding the influence of the season upon the positivity of MCH are scarce. The aim of this study was to assess the percentage of positive MCH tests per season.MethodsWe retrospectively evaluated all MCH tests performed in children and adults in a single center over a 30-month period. The percentage of positive tests for summer was compared with that of other seasons.ResultsA total of 155 challenges were performed in children (under 20xa0years old) and 527 in adults. Thirty-eight percent of the tests were positive in adults and 71xa0% in children. The percentage of positive tests in the summer was significantly lower than the percentage of positive results during the rest of the year in children (58.5 vs. 75.4xa0%, respectively; pxa0=xa00.046). By contrast, there was no difference between the seasons in adults (39 vs. 38xa0%, respectively; pxa0=xa00.92).ConclusionsThere is a difference of 22.4xa0% in the percentage of positive tests in the summer months compared to the rest of the year in children, suggesting a reduction in the sensitivity of the MCH test in the hot season. We suggest that in cases where asthma is strongly suspected in a child and the MCH test was negative in the summer, one should consider repeating the MCH test in another season.

Ultrasound velocity through the tibia is not affected by prolonged inhaled steroid therapy in children.

To evaluate in a prospective, cross‐sectional cohort study the impact of inhaled corticosteroids (ICS) on bone speed of sound (SOS) in asthmatic children.

Sinus vein thrombosis as presenting finding in the congenital central hypoventilation syndrome: An insight on the pathophysiology of the association

Congenital central hypoventilation syndrome (CCHS) is an increasingly recognized diagnosis causing central hypoventilation and may be definitively diagnosed by genetic testing. Previous authors reported the association between CCHS and central sinus venous thrombosis (CSVT) and hypothesized that CCHS could be secondary to CSVT. We report a case of CCHS with the typical PHOX2B mutation who also suffered from CSVT. We assume that effects, secondary to CCHS, upon the central venous system may explain the etiological connection between CSVT and CCHS including dysautoregulation, venous stasis or polycythemia. We believe that CCHS should be included in the differential diagnosis of patients with CSVT accompanied by respiratory abnormalities. Pediatr. Pulmonol. 2011; 46:826–828.

Primary ciliary dyskinesia in Israel: Prevalence, clinical features, current diagnosis and management practices

BACKGROUNDnPrimary Ciliary Dyskinesia (PCD) is rare and its features in Israel have not been described.nnnAIMSnto assess prevalence utilizing state-of-the-art diagnostic techniques, and describe clinical features, diagnostic and management practices in Israel.nnnMETHODSnA national multicenter study from 2012 to 2013 recruited patients diagnosed or suspected of having PCD. Diagnosis was verified using: nasal Nitric Oxide (nNO); High-speed Video Microscope Analysis (HVMA); Transmission Electron Microscopy (TEM) of cilia; Immuno-fluorescence staining (IF) for ciliary proteins, and genetic analysis.nnnRESULTSnOf the 203 patients recruited from 14 pediatric centers, 150 had a PCD diagnosis verified. Median age was 15.05y, with range 0.15-60.5y. PCD prevalence was 1:54,000 for the general population and 1:25,000 in children (5-14xa0y). For the non-Jewish (mainly Druze and Arab Moslem) compared to Jewish populations, prevalence was 1:16,500 and 1:139,000 respectively (pxa0<xa00.0001) and parental consanguinity was 85.4% and 21.9% respectively (pxa0<xa00.0001). Clinical features included bronchiectasis (88%), rhinitis (81%), recurrent pneumonia (78%), recurrent otitis (62%), neonatal pneumonia (60%) and situs inversus (42%). Prior diagnostic practices varied widely between centers with TEM assessed in 55% and abnormal in 61% of these. Management included antibiotics and airway clearance. Diagnostic verification revealed for 150 PCD patients: 81% nNO<233xa0ppb, 62% abnormal HVMA, 51% diagnostic TEM, 58% diagnostic IF and, 57% genetic diagnosis.nnnCONCLUSIONSnPCD in Israel is rare, with comprehensive diagnostic tests showing prevalence in children similar to Europe. Prevalence was higher in non-Jews, associated with parental consanguinity. Diagnostic and management practices vary. Referral centers providing comprehensive diagnostic and care capabilities should be established.