Kartagener's Syndrome Associated With Allergic Bronchopulmonary Aspergillosis

Bhavneesh Sharma, MD; Mohan Sharma, MD; Elliot Bondi, MD; Manisha Sharma, MBBS, DTCD

In This Article

Case Report

This is a case report of Kartagener's syndrome associated with allergic bronchopulmonary aspergillosis (ABPA), which has not been previously described in the medical literature.

A 30-year-old man presented with recurrent episodes of chest tightness, wheezing, and cough for the past 3 years. No family member had similar respiratory complaints or any significant systemic illness. Respiratory examination revealed bilateral coarse crackles. Cardiovascular examination showed that the apex beat was prominent in the right fifth intercostal space in the midclavicular line.

The blood total eosinophil count was elevated to 2.5 x109/L (reference range, .04-.4 x 109/L). The serum total immunoglobulin (Ig)E level was elevated to 5.7 x 106 IU/L (reference range, 0-105 IU/L). An electrocardiogram was consistent with mirror-image dextrocardia. A high-resolution computed tomograph (HRCT) scan of the chest showed situs inversus with central bronchiectasis. A computed tomographic (CT) scan of the paranasal sinuses demonstrated chronic pansinusitis with mucosal thickening and opacified sinus cavities. Pulmonary function testing demonstrated a mixed obstructive and restrictive pattern. An electron microscopic study of samples from the nasal mucosa revealed the absence of outer and inner dynein arms (Figure). Tests of sweat and the genetic map for cystic fibrosis were negative. A summary of the significant clinical data of the patient is illustrated in the Table .

Transmission electron micrograph of a nasal mucosal biopsy sample of the patient showing the absence of outer and inner dynein arms.

We decided to evaluate the patient for ABPA in view of asthma, eosinophilia, raised serum total IgE level, and central bronchiectasis. A commercially available, automated, specific IgE assay against Aspergillus fumigatus antigen by Pharmacia CAP System (Kabi Pharmacia, Sweden) was positive, showing a CAP RAST ASM class 3. An enzyme-linked immunosorbent assay (ELISA) for aspergillus-specific IgG antibodies was positive, showing a titer of 2.93 indices. An immunodiffusion precipitin test to detect aspergillus-specific antibodies (Gibson Laboratories Inc., Lexington, Kentucky) demonstrated a positive result (3 precipitin bands). A skin test with commercial A fumigatus extract was positive, showing a 4+ result. A fumigatus culture from serum and bronchoalveolar lavage fluid was negative. Sputum staining and cultures for Mycobacterium tuberculosis were negative.

The patient fulfilled the minimum criteria for the diagnosis of ABPA in asthma.[1] He was managed with oral prednisolone at 0.5 mg/kg/day for 2 weeks, then tapered and discontinued at 16 weeks. There was marked improvement in the general condition of the patient. Chest radiographs repeated after 4, 8, and 16 weeks showed a significant reduction in the infiltrates. The serum total IgE level decreased to .17 x 106 IU/L at 8 weeks and .09 x 106 IU/L at 16 weeks. The immunodiffusion precipitin test and aspergillus-specific IgE and IgG assays were repeated at 16 weeks and were negative.