exudates and Hypertension--Pulmonary

This study aims to determine the immediate outcome of persistent pulmonary hypertension of the newborn (PPHN) and risk factors for mortality in the era of inhaled nitric oxide (iNO).. This observational cross-sectional study includes 195 confirmed PPHN with a gestational age of ≥34 weeks without congenital heart disease. Multivariable logistic regression was used to identify risk factors for mortality.. The mortality rate was 16.4%, with the highest mortality with pulmonary hypoplasia. Of 195, 65% received iNO; 18% were iNO non-responders with the majority having pulmonary hypoplasia. Independent risk factors for mortality were the presence of reversal of flow at the descending aorta, pulmonary hypoplasia, APGAR scores ≤ 5 at 5 min, and idiopathic PPHN with an adjusted odds ratio of 15.9, 7.5, 6.7, and 6.4, respectively.. Despite the usage of iNO, mortality due to PPHN remains high and is related to etiology and cardiac function.

Topics: Administration, Inhalation; Humans; Hypertension, Pulmonary; Infant; Infant, Newborn; Malaysia; Nitric Oxide; Persistent Fetal Circulation Syndrome; Risk Factors

We performed a cross-sectional study of the demography, clinical and laboratory features of patients with systemic sclerosis patients followed up in our centre from 1984 to 2007. There were 23 cases with the majority of them (96%) being female. They have a mean age of 50.3 years and a mean disease duration of 6.02 (SD 5.82) years. Our patients comprised of multi-ethnic groups with predominantly Chinese (52%), Sarawak natives (35%) and Malays (13%). They have a mean lag time to diagnosis of 24.8 (SD 34.8) months. All the patients have sclerodermatous skin changes with 16(70%) having diffuse scleroderma and 7(30%) having limited scleroderma. The common clinical manifestations found in our patients were Raynaud's phenomenon (91%), sclerodactyly (65%), digital ulcers (52%) and pulmonary fibrosis (52%). There was low incidence of pulmonary hypertension (13%) and renal involvement (4%). The majority of our patients (67%) have positive ANA with 33% positive Scl-70. The majority received calcium channel blockers (87%), aspirin (48%) and low-dose prednisolone (48%). One patient developed adenocarcinoma of the lung on follow-up. This study demonstrated the rarity of systemic sclerosis in our centre with considerable lag time to diagnosis in our patients. Diffuse cutaneous systemic scleroderma is more common in our centre with rare pulmonary hypertension and renal involvement.

Topics: Age of Onset; Autoantibodies; Calcium Channel Blockers; Cross-Sectional Studies; Female; Hospitals, Public; Humans; Hypertension, Pulmonary; Malaysia; Male; Middle Aged; Pulmonary Fibrosis; Raynaud Disease; Scleroderma, Diffuse; Scleroderma, Limited; Scleroderma, Systemic; Skin Ulcer

This prospective observational study was conducted to determine the outcome of newborns with congenital diaphragmatic hernia (CDH). They were managed with a protocol of gentle ventilation to avoid barotraumas, and inhaled nitric oxide (iNO) or intravenous magnesium sulphate for treatment of persistent pulmonary hypertension of newborns (PPHN).. All newborns with CDH admitted to neonatal intensive care unit of this hospital during the six-year study period were recruited. High frequency oscillatory ventilation was used when infants required peak inspiratory pressure of more than 25 mmHg. iNO at 20 ppm or intravenous magnesium sulphate was used when PPHN developed. Arterial blood pH was maintained between 7.35 and 7.45, and partial pressure of arterial carbon dioxide was kept above 35 mmHg. Surgery was performed when the infants' general condition and blood gases were stabilised for at least 24 hours.. Of 21 infants recruited (15 males and six females, median gestational age 39.0 weeks, median birth weight 2,800 grams), 52.4 percent had PPHN. 12 (57.1 percent) underwent surgery at a median age of 4.9 days. One died postoperatively due to PPHN. Out of the 21 subjects, 11 (52.4 percent) survived to discharge. There was no significant difference in the demographic characteristics, side and size of CDH defects, presence of PPHN, or type of treatment received, between infants who survived and died. However, infants who died had significantly lower mean Apgar scores at five minutes of life (p-value is 0.02), and higher mean oxygenation indexes (OI) (p-value is 0.01) than those of survivors. Two (18.2 percent) of the 11 survivors developed chronic lung disease.. Low Apgar scores and high OI were associated with poor outcome in infants with CDH.

Topics: Administration, Inhalation; Apgar Score; Combined Modality Therapy; Developing Countries; Female; Hernia, Diaphragmatic; High-Frequency Ventilation; Hospitals, University; Humans; Hypertension, Pulmonary; Infant Mortality; Infant, Newborn; Infusions, Intravenous; Intensive Care Units, Neonatal; Magnesium Sulfate; Malaysia; Male; Nitric Oxide; Prospective Studies; Risk Factors; Vasodilator Agents

A rare case of childhood pulmonary haemosiderosis with juvenile idiopathic arthritis is discussed, with particular reference to treatment with hydroxychloroquine and sildenafil for pulmonary hypertension which occurs secondary to this disease.

Topics: Adolescent; Anti-Inflammatory Agents; Antirheumatic Agents; Arthritis, Juvenile; Female; Hemosiderosis; Humans; Hydroxychloroquine; Hypertension, Pulmonary; Malaysia; Piperazines; Prednisolone; Purines; Sildenafil Citrate; Spironolactone; Sulfones; Vasodilator Agents