Chronic carbon monoxide poisoning in children.
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Biomedical subjects
Publications and source records attributed to N Sharief.
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We report the experience with and evaluation of treatment strategies in fibrosing alveolitis and desquamative interstitial pneumonitis (FA/DIP) over the last 16 years by a review of all cases referred to a tertiary referral center. There were 25 cases, 16 boys and 9 girls (mean age at onset, 2.3 years; range, 7 days to 11.6 years). In each case the diagnosis was confirmed by open lung biopsy at a mean age of 3.3 years (range, 7 weeks to 15.1 years). Presently features were tachypnea (19), cyanosis (15), cough (12), exertional dyspnea (7), recurrent chest infections +/- wheezing (9), and clubbing (8). Four patients recovered without antiinflammatory medication. The others received specific treatment. Of 11 patients given only prednisolone, six improved, two did not, and three died despite treatment. Of five patients receiving only chloroquine, four responded. Five patients received both prednisolone and chloroquine; one died, two responded well. There was poor progress in the remaining two. Of the 10 patients receiving chloroquine six (60%) showed a good response. A younger presentation carried a worse prognosis, but chest radiology at presentation and outcome were not interrelated. Those with mild histological changes all survived, but severe desquamation or fibrosis at biopsy was not related to outcome. In four cases there was a family history (16%). Patients with FA/DIP probably represent a disease spectrum of multiple etiology with a variable prognosis and response to treatment.
A preterm infant, ventilated for hyaline membrane disease, had severe right-sided pulmonary interstitial emphysema, left-sided compression atelectasis, and bronchopleural fistula. Several modalities of treatment were tried and were unsuccessful. Selective bronchial occlusion with a balloon catheter resulted in dramatic improvement in the patient's clinical and radiographic condition.
A term baby with neonatal convulsions secondary to birth asphyxia was given i.v. phenytoin via a cannula in the dorsum of the hand at 17 h of age. She developed a blue discolouration in the tissues surrounding the i.v. site. The infusion was aborted but the discolouration gradually spread to the rest of the hand. Twenty hours later, improvement could be detected although a blister appeared near the i.v. site. A further attempt two days later to administer phenytoin via an i.v. cannula sited in the left foot was aborted after the appearance of a similar reaction.
Four children, including three infants, who died suddenly and unexpectedly are described. In three of the cases group A beta haemolytic streptococcus was cultured and in the fourth Streptococcus pneumoniae. The organism was grown from multiple sites including blood in two of them. Without microbiological investigation the diagnosis would have been sudden unexpected death syndrome.
Two patients with Henoch-Schonlein purpura are described. Both developed prolonged intestinal obstruction requiring total parenteral nutrition. One child had transient partial duodenal obstruction on barium study and in the other extensive ecchymosis was confirmed at laparotomy.
A girl presented at 6 weeks of age with failure to thrive and arching of the back. She had various dysmorphic features, hepatosplenomegaly, and developmental delay. The electroencephalogram and cranial ultrasound were abnormal, and a computed tomogram showed lissencephaly and apparent agenesis of the corpus callosum. Because of frequent aspiration she became oxygen dependent. She later developed intractable convulsions and died at the age of 9 months.
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A 9 month old boy presented with acute respiratory distress and was found to have a left pleural effusion. The chylous nature of the effusion, multiple bony lytic lesions, and splenic cysts lead to the diagnosis of congenital lymphangiomatosis with chylothorax. Surgical intervention including pleurectomy was required after unsuccessful conservative management.
Fasting adult male rats were given, by orogastric tube, sucrose, glucose, glucose with fructose and water, and the metabolic rate for the ensuing 150-180 min was measured. It was found that there was an increase in metabolic rate after all sugars, greatest after sucrose and least with glucose and with fructose. With the glucose:fructose mixture the metabolic rate was greater then glucose and less than an equimolar load of sucrose. The respiratory quotient after glucose was less than that after fructose, whereas with sucrose it was greater than an equimolar mixture of glucose and fructose.
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We describe a familial case of Marfan's syndrome with associated intrathoracic stomach detected during the neonatal period. The patient developed a primitive leukemia at 3 months of age. Acute leukemia in a patient with Marfan's syndrome has not previously been reported.