Mahboobeh Ghanbarzadeh a C.F. patient

    Mahboobeh Ghanbarzadeh, a 12 years Old Iranian girl from Rasht was referred to our hospital for management and evaluation of sever dyspnea and cyanosis in November 2001.

    Her mother told us that she had dyspnea, cough and respiratory distress, when she was 5 years old. She was treated for RAD and was not good in her general condition. She had many respiratory infection episodes, but no history of gastrointestinal problem.

    In October 2000 she admitted in Rasht for an acute attach of respiratory distress. Due to pneumothorax she needed recurrent chest tube insertion, and finally she underwent pleurodesis. She was sent home with continuous nasal O2  3-5 lit/min, and she was unable to do her daily activity.

    Her older brother died at 14 due to same problem, without any definite diagnosis. Two other siblings, parents, and other family are well. She had normal delivery and up-to-date vaccination.

    In her first admission at our hospital, she was in severe respiratory distress, cyanotic, O2  dependent.

    She was chashectic. Wt= 17kg            height= 130Cm        RR= 45/min. Fine and course crackle and wheezing heard on both longs. Clubbing was noted in all her digits.

    Immunodeficiency, Alfa-1 antitrypsin  deficiency and TB ruled out, but sweat test was positive two times. Chest X-ray and CT Scan showed diffuse bronchietatic  and fibrotic  changes, some large cysts was noted as well.

After starting appropriate antibiotic, bronchodilator, corticosteroid and physiotherapy, she became moderately better, but need intermittent O2  and able to go home. She was a good candidate for double lung transplantation.

She had three more admissions in Rasht for controlling her infections and respiratory distress. In her last admission she was in respiratory failure and mechanical ventilation was necessary. As she was on respirator for a long time, she needed tracheostomy tube.

Her physician was unable to wean her, and sent her to our hospital again. In our hospital I treated her for cardiac failure due to pulmonary hypertension, started steroid and changing the ventilation setup could wean her within 5 days. Now we help her with continuous O2 and intermittently on T- piece and mechanical ventilator for respiratory muscle training and rehabilitation.

She gained 6 kg during 6 months and now takes digoxin, fat- soluble vitamins, and prophylactic antibiotics.

She absolutely needs lung transplantation.

Seyed Ahmad Tabatabaii M.D.

Pediatric Pulmonologist

Chest X-Ray of Mahboobeh

24 August, 2002

Parham Memorial

The Children's Hospital Medical Center

Mofid Kids Hospital

Behgar Tavan

Surgery Centre

Tel/Fax: +98 21 2900679    +98 21 2227207

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