Approach to childhood interstitial lung disease in resource limited setting

Childhood interstitial lung disease (ChILD) is a rare disease and sensitization is needed in the recognition, diagnosis and treatment approaches. There is no formal approach to diagnosis or therapy in resource limited regions. We present a case of a 4-month infant who presented with all the criteria for diagnosis of ChILD. Lung biopsy being the gold standard is a challenge in our setting and diagnosis was based on clinical signs and imaging after ruling out of other similar respiratory conditions by way of individual trials of therapy. Monotherapy with prednisone showed clinical improvement within days of initiation.


Introduction
Childhood interstitial lung disease (ChILD) is a rare group of disorders characterized by diffuse lung infiltrates and impaired gas exchange.
There has been progress in the approach to ChILD, particularly in its recognition and classification in infants being discrete from older children. Many of the forms may be triggered by an inflammatory process such as an infection [1]. The epidemiology is poorly described and most often appear as case reports. One large study by Dinwiddie et al., in the United Kingdom and Ireland (1995)(1996)(1997)(1998) showed estimated prevalence of 3-6 per million in immunocompetent children [2]. In recent years advancement of knowledge on ChILD has developed in to a more formal classification system [3].

Patient and observation
We present a 4-month female patient who has been unwell from 2 weeks of life. The baby was delivered at term with weight of 3.0 kg and discharged uneventfully. At 2 weeks of age developed cough that progressively worsened over a week with post tussive vomiting and no fevers. The baby was able to breastfeed, had no breathing difficulties or worsening of cough during feeds. There was no contact with persons with chronic cough or pulmonary tuberculosis (TB).
There was no family history of smoking and biomass fuel use. She was treated thrice for a probable bacterial infection with amoxicillinclavulanic acid, erythromycin and trimethoprim-sulfamethoxazole respectively with no benefit as an outpatient. The infant worsened and was admitted at 1-month age. Clinically was wasted (weight 2.2kg; weight to height z score of -3SD), afebrile, had tachycardia and was the RR was 55/minute with normal breath sounds and no crackles.
The SaO2 was 95-99% on nasal prongs at 2L/min, had weight gain and was able to breast feed. The dose was tapered down to 1mg/kg/24 hours and a week later gained weight to 3 kg, RR= 45/minute and stay off oxygen for 2 hours with SaO2 > 95%. While the patient was recuperating in hospital, developed probable nosocomial pneumonia 26 days after starting prednisone and died.

Discussion
Interstitial lung disease in children is a rare disease and the diagnosis is usually delayed such as in our case where the infant was diagnosed with ChILD at 3 months of age. ChILD occurs in the developing world but is under reported and confused with resistant bacterial or viral pneumonia. The diagnosis in our case was made after ruling out all possible causes of the respiratory distress. As we note, the infant was managed initially for an infection (bacteria, viral bronchiolitis, pneumocystis jiroveci and fungal) and gastroesophageal reflux with no improvement. Cystic fibrosis is rare in our set up and was ruled out using fecal elastase level as sweat test is unavailable in our setting.
There are few specialized cardiothoracic surgeons trained to do lung biopsy but due to bed space availability and required resources for post biopsy care and monitoring, lung biopsy is a challenge in our

Conclusion
There are no diagnostic and management protocols in the sub-Saharan set up and it is hoped that this article can be a start. There is need for more sensitization on ChILD in sub-Saharan Africa. It being a rare disease may go unreported with delays in diagnosis and associated high mortality. More research is needed to classify the causes of ChILD in the sub-Saharan region as the causes may be different from those in temperate regions.

Competing interests
The authors declare no conflict of interests.