A case of Kartagener's syndrome with combined aplasia of frontal and sphenoid sinuses and hypoplasia of maxillary and ethmoid sinuses.

Kartagener's syndrome (KS), characterized by a triad of bronchiectasis, chronic sinusitis, and situs inversus, is a subset of an autosomal recessive hereditary disorder of primary ciliary dyskinesia. We report the case of a 35-year-old male who presented with a history of intermittent episodes of productive cough, breathlessness, and cold since childhood. High resolution computed tomography of chest revealed bronchiectatic changes, dextrocardia, and right-sided aortic arch. Computed tomography (CT) scan of the abdomen revealed situs inversus. CT of the paranasal sinuses revealed combined aplasia of bilateral frontal and sphenoid sinus with sinusitis. Based on these findings, a diagnosis of KS was made. There was no complaint of infertility, which usually accompanies KS, even though an analysis of his seminal fluid revealed reduced count and reduced motility of sperms. The uniqueness of our case is that our patient was a male aged 35 years; besides, the third decade is an unusual age for presentation of combined aplasia/agenesis of bilateral frontal and sphenoid sinuses with hypoplasia of maxillary and ethmoid sinuses. Even though these findings have been reported in children and young adults, there are very few case reports of such a presentation in adults in literature.

A case report of Jarcho-Levin syndrome.

Jarcho-Levin syndrome (JLS) is a rare, congenital disorder, inherited in an autosomal recessive pattern, that represents a spectrum of clinical and radiographic abnormalities of the spine and chest. The present case report discusses two siblings, an 11 year old girl and a 6 year old boy, diagnosed as cases of JLS based on family history and clinical-radiological findings. The main features of the syndrome are shortness of stature with a spinal abnormality, multiple abnormal vertebral defects, and a small malformed "fan-like" or "crab-like" rib cage due to posterior fusion and anterior flaring of the ribs, leading to short-trunk dwarfism. The spinal and rib malformations result in a small thoracic cavity, not capable of accommodating the growing lungs, causing thoracic insufficiency resulting in severe cardiac and respiratory complications and frequent chest infections. The diagnosis is based on clinical and radiological findings, characteristic physical appearance, symptoms of thoracic insufficiency, family history, consanguineous parents, skeletal survey, or specialized genetic tests for mutations. Milder forms are compatible with life.

Dietary supplementation with omega-3 polyunsaturated fatty acids ameliorates acute pneumonia induced by Klebsiella pneumoniae in BALB/c mice.

The immune benefits associated with the optimal intake of dietary fatty acids are widely known. The objective of the present investigation was to elucidate the role of omega-3 polyunsaturated fatty acids (n-3 PUFA) food source on acute pneumonia induced by Klebsiella pneumoniae. Three different n-3 PUFA preparations (cod liver oil, Maxigard, and flaxseed oil) were orally supplemented and infection was induced in different groups of experimental mice. Mice fed olive oil and normal saline served as oil and saline controls, respectively. After 2 weeks of fatty acid feeding, no effect on the establishment of infection was observed when acute pneumonia was induced in animals. On the other hand, 6 weeks of n-3 PUFA administration was found to improve resistance in mice, as reduced lung bacterial load coupled with significant improvement in pathology was seen in infected mice. Alveolar macrophages collected from all 3 groups of mice fed n-3 PUFA exhibited a significant decrease in the level of apoptosis following infection with K. pneumoniae and an enhanced in vitro phagocytic potential for the pathogen. Lower lung levels of nitric oxide, malondialdehyde, and lactate dehydrogenase were associated with a decrease in the severity of tissue damage. There was a significant increase in the lung levels of pro-inflammatory cytokines (tumor necrosis factor alpha (TNF-α) and interleukin-1 beta (IL-1ß)). No significant change was observed in the levels of interleukin-10 (IL-10). This study highlights that dietary n-3 PUFA supplementation exerts an overall beneficial effect against acute experimental pneumonia. This mechanism is operative through upregulation of nonspecific and specific immune defenses of the host.

A comparative study of induction of pneumonia in mice with planktonic and biofilm cells of Klebsiella pneumoniae.

In the present study, the course of acute pneumonia in normal BALB/c mice infected by intranasal inoculation of planktonic and preformed biofilm cells (3 days old) of Klebsiella pneumoniae B5055 was studied and compared. With both cell forms the peak of infection was observed on the third post infection day, as assessed on the basis of lung bacterial load and corresponding pathology. There was an intense neutrophil infiltration in bronchoalveolar lavage fluid. Tissue damage was assessed on the basis of increased amounts of nitrite, malondialdehyde and lactate dehydrogenase in lung homogenates. The phagocytic potential of alveolar macrophages was lower in biofilm cell-induced infection than in that induced by planktonic cells. Biofilm cell induced infection generated significantly greater production of tumor necrosis factor-α and interleukin-1ß on the third and fifth days of infection, respectively. Production of interleukin-10 was, however, variable. There was no significant difference in the ability of planktonic and biofilm cell forms of K. pneumoniae to induce acute pneumonia in mice in terms of bacterial counts and histopathological changes. However, biofilm cell-induced infection showed delayed clearance as compared to infection induced with the planktonic form.