Eradication pseudomonas aeruginosa infection in a 19-year-old boy with cystic fibrosis, bronhiectasis and pneumotorax

Adnan Bajraktarević (1,2), Lutvo Sporišević (3), Kenan Karavdić (4),
Zlatan Zvizdić(4), Khatib Hakam (2,5)
(1)PEDIATRICS DEPARTMENT OF HEALTH INSTITUTION OF CANTON SARAJEVO, BOSNIA, (2)PEDIATRICS CLINIC OF THE UNIVERSITY OF GRAZ, AUSTRIA, (3)FIRST MEDICAL AID, SARAJEVO, (4)CHILDREN’S SURGERY, SARAJEVO, UNIV. CLINIC SARAJEVO, (5)PEDIATRICS CLINIC, DOHA

INTRODUCTION

Cystic fibrosis (CF) is an autosomal recessive disease that is predominantly seen in the Caucasian population and involves multiple organs(1) . Recent advancement in therapy has lead to improvement in survival . Diffuse bronchiectasis is seen in patients with cystic fibrosis. Colonisation with Pseudomonas aeruginosa (PA) is common in older kids with cystic fibrosis (CF) and there is increasing evidence that transmissible strains may cross colonise patients (2,3). PA colonization is usually difficult to eradicate with antimicrobial therapy and, in some children and younger patients, infection is associated with rapid decline in pulmonary function, increased hospitalization, and earlier death (4,5,6). Frequent complications of cystic fibrosis are chronic respiratory infections, including pneumonia, bronchitis, chronic sinusitis and bronchiectasis - an abnormal dilation of the walls of the bronchial tubes that makes it more difficult to clear your airways. Cystic fibrosis (CF) is a genetic disorder that particularly affects the lungs and digestive system and makes kids who have it more vulnerable to repeated lung infections (7-12) . Author reports the case of an 19 year old boy with cystic fibrosis and bronchiectasis with complications of recurrent infection by Pseudomonas aerigenosa and Pneumotorax and review the literature that describes lungs involvement in cystic fibrosis. This case is very illustrative for this disease, bacterial eridacation with three antibiotics and possibility of future therapy - operation and lungs transplantation.
 

CASE REPORT

An 19 year old male with CF severe lungs disease and liver insufficiency presented with coughing and breathing difficilities with wheezing progressed to generalized bad situations and Pseudomonas aerigenosa opportunic suprainfection . There was a history of preceding upper respiratory symptoms. He denied any change in his or bowel habit and there was no history of headache, visual disturbance, jaundice, palpitation, or urinary infection . He had history of pneumonia 2 years back with failure to thrive since 15 years. The patient remained on his regular medications which included albuterol, multivitamins and pancreatic enzymes. He was admitted many times with similar symptoms 9 months prior to this admission, and multiple number of admissions from childhood. An extensive workup was negative including liver function tests, viral hepatitis panel, Alpha 1 antitrypsin (stool, blood), abdominal ultrasound and upper GI endoscopy. He was treated with flixonase-sulbutamol inhalation and other steroids inhalations. However, the etiology for the cystic fibrosis was uncertain. He was well until age 16, when adynamia, chronic cough, and dyspnea during exercise ensued. His pulmonary symptoms worsened, and the cough became productive . The patient was treated successfully with Kreon for dishes times(1/2-3/4 tablets) inhalation steroids (Seretide discus Junior 2 times 1 puff) achieved remission after about six weeks of therapy, but after that worse situation and difficulties with breathing). Patient D.K. from Austria , BIRKFELD, Steirmark, present with failure to thrive, fever, chronic productive cough and recurrent pulmonary infections. Hypoxemia is mild. Leathery coarse crepitations may be heard over the affected region. On examination he had increasing a respiratory rate of 40/min for age , and wheezing. He had no clubbing .
On this admission, he had mildly elevated blood pressure at 131/79. His weight was 58.7(P10<Pw<P25) kg with 6 kg of recent weight gain from last year. Height 174 cm. He had moderate troubles with every day activities and lower degrees of work possibility. Otherwise physical examination except lungs was normal.
The right side of the chest appeared flattened with decreased air entry on the entire right side. On percussion, there was a dull note on right side and apex beat was shifted and hypersonored to the right anterior axillary line. On auscultation, there were coarse crepitations on (R) sided suggestive of right sided fibrosis with bronchiectasis and partial pneumopleurotorax. Abdomen was meteoristics and he had no pains). Other systems were normal. X-Ray chest was suggestive of (R) sided collapse consolidation with cystic bronchiectasis (Picture 1-Röntgen of lungs). An HRCT (chest) with virtual bronchoscopy was done that showed left sided cystic bronchiectasis with fibrosis with compensatory hypertrophy of left lung with shift of mediastinum to right side (Picture 2-CT of lungs). There was no evidence of foreign body obstruction or lymphadenopathy. His blood gases showed no evidence of metabolic alkalosis and sputum was negative for AFB. His bronchoscopy showed evidence of bronchiectasis on right side without evidence of foreign body. His HIV ELISA was negative.
Diagnosis: Initial evaluation should include search for familial (his grandfathers both side parents had cystic fibrosis mild level) and treatable causes. Serum immunoglobulins (for hypogammaglobulinemia), sweat chlorides (for cystic fibrosis), Alpha 1 antitrypsin levels, neutrophil counts and serum complement levels may be useful. Assay of ciliary clearance or ciliary biopsy is useful for diagnosis of immotile cilia syndrome. Laboratory evaluations showed mild hypoalbuminemia (1.9 mg/dl) with normal kidney function (BUN of 11 mg/dl, creatinine of 0.40 mg/dl and normal urine microscopic evaluation without evidence of RBC casts). 24 hour urine collection no revealed nephrotic range proteinuria
(0 gm/24 hour). Other laboratory evaluations were normal (except elevated Leucocities 13.16 G/L, absolute number of Neutrophilies 10.3 G/L, C- reactive protein 104mg/L, creatin kinase CK 326/l ) including ASO, C3, C4 and ANA(Table 1- Laboratory data). There was no immune deposit and no significant glomerulosclerosis. Sputum showed on admittion Pseudomonas aerigenosa.
He was started on chest physiotherapy, postural drainage and mucolytic therapy. He was advised spirometry and lobectomy of the left lung. If the symptoms cannot be controlled by antibiotic therapy and postural drainage and disease is progressive and localized, resection may be indicated and lungs transplantation.
During staying in children hospital Graz doctors included mixed three components ,the modern, parenteral antibiotics therapy FORTUM (Ceftiazi-dim 100/150 mg/kg two times three weeks), TOBRASIX (Tobramycin 3-6 mg/kg two times three weeks) AND TARGOCID (Teicoplenin 6-8 mg/kg/day for this ages three weeks) . Effect of combined three antibiotics therapy were very successfull on eridication of Pseudomonas aerigenosa and sputum showed on controls only opportunics infections of Staphylocooccus aureus 10 5, Candida albicans 10 3 and Aspergillus fumigatus 10 2. Eridaction can have 99 % success as this case, bit synergistics effects of this parenteral antibiotics are the best in the end of 2007 on all over the world. Inhalation therapy followed all time with Seretide or Serevent but sometimes Sultanol (Sal-butamol). Oral cortiocosteroids therapy with Aprednisolon for 6 days 2 mg/kg/day in two doses help patient for recovering his lung functions. We started with Novonorm therapy because his OGTT test was changed and mild elevated HbA1C 8.1%.Vitamin E, Zinc, Selen , 25-Hydroxy-Vitamin D in blood were mild decreased. Fibrinogen 458 mg/l, CRP decreased on high level too, but only 27.3 mg/l after higher level 104mg/l, aPTT 52.8 seconds were prolonged. Other laboratory evaluations were normal. He took Sporanox capsules 3 times per day, Ursofalk, Losex 20mg with Aprednisolon therapy. Physiotherapy for thorax had every day twice with PEP mask. He followed CF nutrition diet, enzymes for digestion Kreon and moderate salt in food. His food were high calories intake with a lot of vitamins and fruits.
Doctors established same diagnosis as in admittion as Cystis fibrosis , Pneumonia, Asthma bronchiale, Malapsorption, Liver Parenchimcyrrhosis, Pancreasexocrine insuffiention, Patologics Glucetolerant level and of end as new one diagnosis Pleuropneumotorax l.dex.
Affected bronchial segments are pliant and distorted due to destruction of muscular and elastic components of the airway walls. Dilation is due to atelectasis caused by accumulation of purulent secretions and obstruction of the peripheral airway with internal digestion of structural proteins of the airway due to lytic enzymes released from neutrophils in the purulent material in the airway and also by traction on the airway. Chronic inflammation leads to destruction of bronchial cartilage and pulmonary fibrosis with damage to peribronchial alveoli. Anastomosis form between pulmonary and bronchial vessels and along with alveolar hypoxia may lead to cor pulmonale.Multiple complications of the CF lung disease can occur. One of the most regular complications is involvement of the upper airway, in the form pansinusitis, nasal polips, bronciectasis as here, atelectasis as here (4,1% kids), pneumotorax 1% per year but at age 18-19 about 16% to 20% as here.
Lungs physiologics tests were pathologics changed with obstructive and restrictive reductions of vital pulmonal paramethers as FEV1(1.75/l-43%) , FVC(2.49l-53%), RV, MEF25(0.46l/sec-19%), PEF and others in level between 40 % to 60 % depends from admittion until the end of three weeks therapy. Alergics skin tests showed allergics reac-tion on dirty house Dermatafagoideus.
Special immunologics serologics test RIST for IgE was 22.20 kU/l, but RAST test was negative. Stan-dard swat test was positive more then 65mmol/l. The indentified gene was before admittions named cystic fibrosis transmembrane conductance regulator (CFRT).
Doctors established same diagnosis as in admittion as Cystis fibrosis , Pneumonia, Asthma bronchiale, Malapsorption, Liver Parenchimcyrhosis, Patolo-gics Glucetolerant level and of end as new one diagnosis Pleuropneumotorax l.dex.
He needs lavaige for 6 weeks again , and better controlling diseases and he started with Augmentin therapy. If the symptoms cannot be controlled by antibiotic therapy and postural drainage and disease is progressive and localized, resection may be indicated and lungs transplantation. Motivation for Mucoviscidiosis therapy are excellent.
 

DISCUSSION

Colonisation with Pseudomonas aeruginosa is common in older children with cystic fibrosis (CF) and there is increasing evidence that transmissible strains may cross colonise patients. However, transmission of these strains by social contact to healthy non-CF individuals has not been described. Cystic fibrosis (CF) is the most common potentially lethal genetic disease in the white population. Improvements in life expectancy have led to an increasing recognition of lungs and hepatobiliarypancreatics complications from CF. Splenic artery aneurysms are a rare complication of portal hypertension with a high mortality due to their significant potential for rupture, resulting in life-threatening i.p. haemorrhage. Anatomical localization may help in etiological diagnosis. Diffuse bronchiectasis is seen in patients with cystic fibrosis, immotile cilia syndrome, immunodeficiency states, chronic airway disease and allergic bronchopulmonary aspergillosis. In cystic fibrosis, the upper lobes are more involved than the lower lobes. The best treatment for eradication of this bacterial in present time of end of 2007 is combination of three antibiotics (ceftazidim , tobramycin and teicoplanum)
(13-19) .This combination beta laktams cefalosorin, aminoglicosid and glycopeptid showed excellent benefits. If the symptoms cannot be controlled by antibiotic therapy and postural drainage and disease is progressive and localized, resection may be indicated. Upper lobes may also be involved in aspiration, endobronchial tuberculosis and allergic bronchopulmonary aspergillosis. In all other forms the left lower lobe and lingula is more affected probably because the left bronchus is smaller in diameter and lacks gravitational drainage. Bronchiectasis on right side is predominant with foreign body or right middle lobe syndrome due to enlarged lymph nodes. To establish the anatomic diagnosis, CT has replaced bronchography as the diagnosis of choice. Median survival from the first pneumotorax was 29.9 months in large series.
 

CONCLUSIONS

Cystic fibrosis is a genetic disease usually diagnosed by abnormal sweat testing. Author report a case of an 19-year-old male with bronchiectasis, chronic Pseudomonas aeruginosa infection, and higher al sweat chloride concentrations who experienced rapid decrease of lung function (Partial pleurotorax, wheezing, pneumonia) and clinical deterioration despite treatment. Bronchiectasis is often not progressive and patients may remain asymptomatic for extended periods. In this case or at other extreme when the pulmonary disease is widespread and progressive, medical treatment is preferred for eradication of Pseudomonas aeruginosa. Chest physiotherapy and postural drainage are mainstays of treatment. Mucolytic agents such as aerolized recombinant human deoxyribonuclease may be useful in patients with cystic fibrosis. Prompt and rigorous antibiotic-therapy-is-the-cornerstone-of-the-management. Inheritance is autosomal recessive, and the disease is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, located in the long arm of chromosome 7. As for many other human monogenic diseases, high variability in disease expression is found among young patients, kids. Despite the best efforts of CF clinicians pediatricians , patients with CF eventually reach the point of respiratory insufficiency, and lung can no longer sustain . The timing of transplant is optimal if it occurs just before life with disease become unbearable. Neonatal screening has a many benefits of CF newborn disease prevention . Development of vaccine against P. aerigenosa would help in future for better controlling CF.
 

ACKNOWLEDGEMENTS

Authors wish to acknowledge the Univ. Prof Maximilian Zach and Univ. Prof Peter Scheer young patient, young doctors and nurses on Pediatrics Clinic of University Graz for their support and giving the assent and written informed consent for this case report to be published in future may be. The author declares that there have been no financial interests or support that was associated with this manuscript. Thanks University Clinic Graz Austria- Pediatrics Clinic for CT image and Röntgen of lungs.
 

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