A young girl with chronic sufferings ( cystic fibrosis ) Presented by Dr. Jalal Mohsin Uddin DTCD , FCPS (Pulmonology)

Particulars of the patient : Name: Kulsum Age: 14yrs Sex: Female Address: Demra , Narayangonj. Occupation: Student of class four Religion: Islam

Chief complains : Shortness of breath has increased for 7 days Fever for 7days Productive cough for same duration

History of present illness: According to the statement of the patient’s mother, her child has been suffering from recurrent cough and fever since her 9 months of her age . The cough is preceded by fever. It is always productive. Its volume has increased and become purulent in last three years . Occasionally it is foul smelling and blood stained. Cough is more in the morning. In last seven days it has become so severe that it interrupt her sleep and associated with copious amount of purulent sputum.

H/O present illness (continue) The young girl has been suffering from recurrent fever since her child hood. It is usually high grade and is subsided by taking antipyretics and antibiotics. In last seven days the fever was continuous in nature and always high grade. Highest recorded temperature is 103F. She has been suffering from shortness of breath for last three years. It is progressive in nature, initially it occurred during running but now she become breathless even in mild exertion.

History of present illness (continue) She also complain about recurrent headache which aggravate during attack of fever. She often suffer from passage of loose stool, mucoid in nature, not blood stained . Though she is 14yrs old, she reads in class IV. Her mother complain about her delayed physical growth .

Treatment history : Patient has received various type of antibiotics , antipyretic frequently. Previously patient was admitted in different hospitals in three occasions . In April 1995 she was admitted in Dhaka Shishu Hospital and was diagnosed as severe pneumonia . Later on she was admitted in NIDCH in two occasions on August 2006 and November 2007.

Treatment history (continue) During this period of her admission she received cat – l anti tubercular therapy two times . She was never smear positive patient .

H/O past illness : Patient is non diabetic , she has not suffered from measles or whooping cough . She has never come into contact with any patient suffering from tuberculosis.

Family history : The girl is from a low socio economic status . She has two sisters and one brother, all are in good health . There is no history of consanguineous marriage among her parents.

Immunization history : She was immunized against tuberculosis whooping cough , measles and other infectious diseases in due time .

General examination : Appearance: ill looking Behavior: Cooperative Conscious Decubitus: According to her choice. Clubbing: Present Koilonychia, leuchonychia: Absent

General examination (continue) Anemia , cyanosis , jaundice , oedema : absent . Lymphadenopathy : absent . Neck vein : not engorged .

Examination of respiratory system: Inspection : shape and size of the chest is normal , there is no chest deformity , respiratory rate – 24 breaths/min , intercostal recession present , chest movement bilaterally symmetrical . Palpation : Trachea is centrally placed , apex beat is in left 5th intercostal space just medial to the left mid clavicular line . Chest movement bilaterally symmetrical . Total chest expansion 2 cm. Vocal fremitus normal in both side .

Examination of respiratory system (continue) Percussion : it is normal in both side . Upper border of liver dullness is present in 6th intercostal space along the right midclavicular line . Auscultation : Breath sound vesicular , bilateral coarse crepitation present , which alter after coughing . Vocal resonance is normal in both side .

Examination of CVS Apex beat is in 5th intercostal space just medial to the left mid clavicular line . It is normal in character . There is no left parasternal heave . Pulmonary component of 2nd heart sound is not palpable . Both 1st and 2nd heart sound is audible with normal character . Murmur is absent .

Examination of other systems There is no hepato-spleno megaly, ascites absent . Other systemic examination revealed normal finding .

Salient features : Kulsum a 14yrs young girl admitted with the complain of increased shortness of breath, productive cough and fever for 7 days. According to her mother the girl has been suffering from repeated bout of productive cough , fever and shortness of breath since her 9 months age . For this she was admitted to various hospitals and received antibiotics, even Cat-1 two times though she was smear negative. Patient having clubbing and bilateral coarse crepitations .

Provisional diagnosis: Post tubercular bronchiectasis

Differential diagnosis : Bilateral bronchiectasis due to recurrent pneumonia or Cystic fibrosis or Primary ciliary dyskinesias etc.

Investigations Total count of WBC : 13000/cu mm Differential count : Neutrophil : 78%, lymphocyte : 18% ESR : 40 mm in 1st hr Sputum for AFB (3 sample) : negative Sputum for C/S : shows growth of pseudomonus sensitive to Gentamycin and Ceftriaxone .

X-ray chest P/A view showing There are multiple ring shadows involving all the zones of both lung fields specially prominent in upper and mid zones in right side , mid and lower zones in left side. There is intervening fibrous band within the ring shadows.

X-ray chest (continue) Trachea is slightly shifted to the right. Cardiac shadow is normal in transverse diameter. Costo-phrenic angles and cardio-phrenic angles of both sides are clear. Radio logically it is bilateral bronchiectasis with fibrosis .

Sweat test Sweat electrolytes of the patient : Sodium- 197.90 m mol/l Chloride- 190.60 m mol/l REMARKS : both ions >70m mol/l is indicative of CF. both ions <50m mol/l is normal If in between 50-70m mol/l is equivocal need further evaluation.

Investigations (continue) FBS - 110 mg/dl ( indicate IFG) Blood sugar 2hrs after 75 gm glucose : 130 mg/dl. Serum billirubin – 0.7mg/dl SGPT- 17u/l X- ray PNS- Maxillary sinusitis USG of whole abdomen : Normal study. Saccharin test : within normal limit .

CT scan of chest Bilateral bronchiectasis with fibrosis.

Confirmatory diagnosis Bilateral bronchiectasis with fibrosis due to cystic fibrosis.

Treatment given Complete bed rest. Oxygen inhalation Physiotherapy include breathing exercise huffing , coughing , chest percussion , postural drainage etc. Inj Cefuroxime was started empirically then inj gentamycin was added according to culture sensitivity report . Nebulization with salbutamol and ipratropium Theophylline etc .

Comments : Bronchiectasis is not a disease rather it is ultimate presentation of various chest diseases . So diagnosis of underlying causes will be beneficial for the patients. Our institute is very much able to diagnose the underlying causes of bronchiectasis . Like asthma and COPD , bronchiectasis having a chronic course . Regarding its management this may deserve a definite guideline . At the same time health education may play a great role .

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