Kartagener's syndrome: A re-visit with Chinese perspectives

Abstract The objective of this study was to evaluate the clinical, radiological investigation profiles, and ciliary function and ultrastructure in Chinese patients with Kartagener's syndrome (presence of dextrocardia, sinusitis and bronchiectasis). All patients with dextrocardia were assessed for the presence of sinusitis and bronchiectasis in our hospital network. Patients identified with Kartagener's were assessed when they were at steady state for their bronchiectasis. Seven cases (4 males; mean age 34.9 years) were identified and systematically reviewed. The mean 24 h sputum volume was 26.6±32.77 mL/day and the patients suffered from a mean of 2.9 exacerbations/year. Nasal symptoms (anosmia in one, obstruction in six and persistent discharge in three patients) were common. Only two cases (1 M) were married and both had normal fertility. Lung function assessment showed a mean FEV₁/FVC of 83.3±38.78/86.5±36.72 (% predicted) with little reversibility. High resolution computerized tomography (HRCT) revealed bronchiectactic involvement of the lower lobes in seven and middle lobe/lingula in four cases. Assessment of α-1-anti-trypsin, aspergillus precipitins, auto-antibodies and serology for Pseudomonas pseudomallei was normal. Sputum culture yielded Pseudomonas aeruginosa in three, Haemophilus influenzae in three and commensals in one case. Phase contrast microscopy assessment of respiratory cilia, obtained by brushing the inferior turbinate, revealed that most of the mucosa was unciliated. The mean ciliary beat frequency was 5.2 ± 6.76 Hz (range 0–13.7; normal range 12–18 Hz). Four patients had immotile cilia whilst the rest had normal ciliary movement. Transmission electron microscopy showed the absence of dynein arms in four patients. The results of this study show that patients with Kartagener's syndrome may have normal ciliary ultrastructure and the absence of dynein arms is not necessarily associated with ciliary immotility. The presence of ciliary immotility might have prognostic value as these patients appear to have more active bronchiectasis. Our experience on this series should help clinicans in the investigation and management of these patients.     

Palliative enteral feeding for patients with malignant esophageal obstruction: a retrospective study

Background

Malignant esophageal obstruction leads to dysphagia, deterioration in quality of life, and malnutrition. Traditional bedside nasogastric (NG) tube placement is very difficult under these circumstances. However, endoscopically assisted NG tube placement under fluoroscopic guidance could be an alternative option for establishing palliative enteral nutrition. This study aimed to compare the clinical outcomes of enteral tube feeding and esophageal stenting for patients with malignant esophageal obstruction and a short life expectancy.

Methods

Thirty-one patients were divided into 3 groups according to their treatment modality: NG tube (n?=?12), esophageal stent group (n?=?10), and supportive care with nil per os (NPO) (n?=?9). Enteral nutrition, clinical outcomes, length of hospital stay, and median survival were evaluated.

Results

There were no significant baseline differences among the groups, except in age. The tube and stent groups had significantly higher enteral calorie intake (p?=?0.01), higher serum albumin (p?<?0.01), shorter hospital stay (p?=?0.01), and longer median survival (p?<?0.01) than the NPO group. The incidence of dislodgement in the tube group was significantly higher than in the stent group (58 % vs. 20 %, respectively; p?=?0.01). However, stenting costs more than NG tube placement.

Conclusions

Palliative enteral feeding by NG tube is safe, inexpensive, and has a low complication rate. Endoscopically assisted NG tube placement under fluoroscopic guidance could be a feasible palliative option for malignant esophageal obstruction for patients who have a short life expectancy.

     

Interracial differences in prostate cancer progression among patients from the United States, China and Japan

Although previous studies indicate interracial differences in prostate can-cer epidemiology based on gene expression profiles among patients from the United States, China and Japan, evidence at the ge- netic and phenotypic levels that these diffe-rences exist and manifest along ethnic lines has been sparse. Recent studies, however, suggest that genetic differences, such as the lower incidence of Chinese pros-tate cancers harboring TMPRSS2-ERG translocations compared to patients from Western countries, should be carefully con-sidered in the context of genotypic and phenotypic differences among interracial groups. New, more efficient technologies need to be developed to validate genetic, gene expression and/or phenotypic differ-ences associated with prostate cancer tissue specimens obtained from interracial groups, to establish reliable clinical standards that take racial/ethnic data into account to improve the diagnosis, prognosis and treat-ment of patients with prostate cancer.     

Clinical prediction of post-extubation radiological changes of the chest in newborn infants

Objective: Whether a chest radiograph should be performed routinely in all infants after extubation, or selectively only in those with clinical deterioration, is a controversy in neonatal unit practice. This study tested the hypothesis that most cases of post-extubation radiological deterioration in the lungs could be detected by clinical assessment. Methods: A chest radiograph was performed at 8 h post-extubation in 100 episodes of extubation in 85 newborn infants ventilated for a variety of lung diseases. Each infant was assessed at the same time by a neonatologist blinded to the radiological findings, to determine whether a chest radiograph would have been requested based on clinical judgement. The infants were continuously monitored for their respiratory and oxygenation status before and after extubation. Results: Compared to the pre-extubation chest radiographs, 23 of the 100 post-extubation chest radiographs showed either deterioration of the pre-existing lung pathologies or appearance of significant new pathologies. The clinicians' assessment failed to detect most of the deterioration, with a sensitivity of only 21.7%. Systematic analysis of the infants' clinical parameters showed that the development of significant intercostal/subcostal retraction, and an increase in inspired oxygen concentration by ≥ 7% after extubation, were the best predictors of post-extubation radiological deterioration (sensitivity 82.6%, specificity 62.3%, positive predictive value 39.6%, and negative predictive value 92.3%). Serial blood gas in contrast had little predictive value. Conclusion: We conclude that most cases of radiological deterioration of the lungs after extubation are clinically predictable, provided the correct clinical criteria are used.     

Overview on SARS in Asia and the World

Severe Acute Respiratory Syndrome (SARS) is the first major novel infectious disease to hit the international community in the 21st century. It originated in southern China in November 2002, reached Hong Kong in February 2003 and spread rapidly thereafter to 29 countries/regions on five continents. At the end of the epidemic, the global cumulative total was 8098 with 774 deaths. Seven Asian countries/regions were among the top ten on the list. Mainland China and Hong Kong, SAR, accounted for 87% of all cases and 84% of all deaths. Severe acute respiratory syndrome is caused by a novel coronavirus. It has alarmed the world with its infectivity and significant morbidity and mortality, its lack of a rapid, reliable diagnostic test and lack of effective specific treatment and vaccination. The adverse impact on travel and business around the world, particularly in Asia, has been enormous. Some lessons learnt from this epidemic included: (1) any outbreak of infectious disease can rapidly spread around the world by air travel; (2) early reporting of the outbreak to neighbouring countries/regions and the World Health Organization is essential to prevent international spread; and (3) infection control, tracing and quarantine of contacts are essential to control the epidemic. Many questions remain unanswered, including the origin and pathogenesis of the novel coronavirus, the natural history and the best specific treatment of the disease. The SARS‐CoV has probably jumped from an animal host to humans. There is an urgent need to evaluate the human–animal habitat in southern China and to remove animal reservoirs if found.     

Radiological manifestations of malignant pleural mesothelioma

Malignant pleural mesothelioma has had a rising incidence in Australia over the past 40 years. This pictorial essay gives a brief account of the condition, summarizes the various radiological manifestations and aims at increasing the awareness of a disease that is expected to reach its peak incidence in the early decades of the twenty‐first century.     

Successful emergency endovascular treatment of juxtarenal and infrarental mycotic aortic aneurysms in patients with small diameter aortae using Cook? Zenith ESLE Stentgrafts

BACKGROUND:

Endovascular repair of mycotic aneurysm is an alternative to open repair if the patho-anatomy is suitable. The aortic size above and below the mycotic aneurysm may be small.

METHODS:

A retrospective review was made of prospectively collected departmental computerised database.

RESULTS:

Three oriental patients with juxta- and infra-renal mycotic aortic aneurysms with a small aortic diameter of 17 mm to 18 mm underwent successful emergency endovascular treatment using Cook^® Zenith ESLE stentgrafts. These are ancillary devices aimed at iliac extensions usually.

CONCLUSION:

This is to our knowledge the first case series of Cook^® Zenith ESLE iliac component endografts for the treatment of aortic mycotic aneurysms with small aortae, and short- and mid-term results are encouraging.KEY WORDS: Endovascular, Mycotic, Small aorta, Oversizing, Cook^®, Zenith ESLE Stentgrafts