Saudi guidelines on the diagnosis and treatment of pulmonary hypertension: 2014 updates

The Saudi Association for Pulmonary Hypertension [previously called Saudi Advisory Group for Pulmonary Hypertension] has published the first Saudi Guidelines on Diagnosis and Treatment of Pulmonary Arterial Hypertension back in 2008. [1] That guideline was very detailed and extensive and reviewed most aspects of pulmonary hypertension [PH]. One of the disadvantages of such detailed guidelines is the difficulty that some of the readers who just want to get a quick guidance or looking for a specific piece of information might face. All efforts were made to develop this guideline in an easy-to-read form, making it very handy and helpful to clinicians dealing with PH patients to select the best management strategies for the typical patient suffering from a specific condition. This Guideline was designed to provide recommendations for problems frequently encountered by practicing clinicians involved in management of PH. This publication targets mainly adult and pediatric PH-treating physicians, but can also be used by other physicians interested in PH

Childhood bronchiectasis: a review

More than two decades ago, bronchiectasis unrelated to cystic fibrosis [CF] was termed "Orphan disease", because it had become an uncommon clinical entity among children in the developed world. It is more common among children in lower socioeconomic classes and in developing countries, due to more frequent recurrent respiratory infections, environmental airway irritants, poor immunization, and malnutrition. The pathophysiology includes airway inflammation, mucous production, and regional airway obstruction, but the reasons some children develop bronchiectasis while other do not is not clear. Public measures aimed at improving living conditions for children and prevention of respiratory infections with anti-viral vaccines will have a good impact on childhood bronchiectasis than medical treatment

Lung function abdominalities follwing repaired esophageal atresia and tracheoesophageal fistula

Esophageal atresia and tracheo-esophageal fistula [EA/TEF] is common in the neonatal period and survival depends on the severity of the associated anomalies, prematurity and pre-morbid factors. This study represents the experience of a tertiary care center in Saudi Arabia of pulmonary function test abnormalities [PFT] after repair of [EA/TEF] including long-term effect on the lungs. A retrospective review of all patients referred to pulmonary clinic with EA/TEF and or Pre-operative evaluations from the period 1993-2004. A total of 41 patients. Twenty-six [63%] males and 15 [37%] females. EA/TEF was diagnosed at birth in 34 [83%]. EA and distal TEF were found in 37 [90%] of the patients. Congenital anomalies were associated in 28 [68%]. More than 1/3 of the patients had postoperative complications including pneumothorax, recurrent TEF, leakage at operation site and empyema. More than two-third of the patients required prolonged ventilation. Pulmonary complications developed in > 70% of the patients including persistent atelectasis, chronic aspiration pneumonia, tracheomalacia in 12 [29%] and bronchiectasis in 7 [17%]. Eighty-eight percent of patients who were able to do PFT showed abnormal values of moderate obstructive and restrictive lung disease. [EA/TEF] form significant PFT abnormalities and cause significant morbidities that may last for a long period of time

Morbidity and mortality data of cystic fibrosis patients

To identify factors that contributed to morbidity and mortality of cystic fibrosis [CF] population in the Kingdom of Saudi Arabia [KSA]. This retrospective chart review was carried out in King Faisal Specialist Hospital and Research Centre, Riyadh, KSA, during a 9 year period, November 1993 to November 2002, on confirmed CF patients, for demographic, clinical and mortality data. A total of 190 CF patients were diagnosed during the 9 years. One hundred and sixty-four [86%] patients are alive, 26 [14%] died. Ninety-nine [52%] were males and 91 [48%] were females. Age at diagnosis 2.8 +/- 3.5 years, and period of follow up 3 +/- 3 years. In 80% of patients, symptoms started <1 year of age. Sixty-five% of patients were in the mild to moderate malnutrition stage [<90th percentile], and 63% are in the mild to moderate stunted growth [<90th percentile]. Factors that contributed to early mortality are: calculated weight/height [p-value 0.01], low albumin level at follow up [0.001], high hematocrit [HCT] [p-values=0.0002], low mean corpuscular volume [MCV] [p-0.0002], low mean corpuscular hemoglobin concentration [MCHC] [p-value 0.001], early development of antibiotic resistance [p-value=< 0.01]. High HCT, low MCV, low MCHC and low albumin are factors related to poor prognosis and early death in CF patients. Iron supplement should be given to these patients even in the presence of normal hemoglobin. Early nutritional rehabilitation is needed to improve survival of our CF patients. Cohort isolation should be encouraged in CF centres. Early treatment of chronic pseudo colonization should be adopted to improve survival

Simultaneous occurrence of cystic fibrosis and sickle cell disease in one patient: Is it a rare phenomenon? A letter to the editor

Combination CF/SS disease is a rare phenomenon in Saudi Arabia. Awareness of existence of both diseases should be increased, since SS disease is seen very frequently in pediatric clinic, and screening for co-existence with CF should be raised especially in patients with SS disease and repeated chest infection. Co-existence of both diseases may not change the course of the disease, but the patient needs multiple medications and close follow-up

Resolution of bronchiectasis after foreign body removal

An undiagnosed foreign body [FB] can cause mechanical effects, chemical reactions, and may present as chronic pulmonary infection, bronchiectasis, asthma, lung collapse or lung abscess. We describe a case with foreign body aspiration that was diagnosed two months after aspiration, and which resulted in bronchiectasis of the left lower lobe [LLL] of the lung, and which ended with complete resolution of the bronchiectatic changes after removal of FB and antibiotic treatment

Bronchiectasis after kerosene ingestion

Kerosene paraffin ingestion represent a serious source of childhood poisoning in Saudi Arabia. The incidence of pneumonia after kerosene ingestion is high and may be accompanied with pleural effusion, pneumatocele or lung abscess. In this report we describe a case of kerosene ingestion that was complicated with bilateral pneumonia, pleural effusion and later with bronchiectasis

Familial interstitial lung disease in five family members with different pathological staging

Reports on familial interstitial lung disease [ILD] are rare and all are indicative of high mortality. We report familial ILD in four siblings and their father. Lung biopsy on each patient revealed different stages of ILD in the form of usual interstitial pneumonia [UIP] in one child, desquamative interstitial pneumonia in 2 children, and non-specific interstitial pneumonitis with a predominance of inflammatory cells in another child. All four siblings presented with repeated chest infection, cyanosis and became oxygen dependent. Chloroquine was used in treating all 4 siblings with variable response. The child with UIP died from progressive disease while on treatment. The father was diagnosed at 40 years of age with ILD and extensive fibrosis, but did not respond to cyclophosphamide treatment. In conclusion, familial interstitial lung disease can present with different pathological stages in the same family members and the response to treatment depends on the degree of inflammation or fibrosis. The condition should be diagnosed early before it reaches an irreversible stage