Objective: To illustrate the clinical features, laboratory findings, and management of anticonvulsant hypersensitivity syndrome (AHS), emphasizing the importance of recognizing its multiple clinical components and raising awareness of the cross-sensitivity among different anticonvulsants. Clinical Presentation and Intervention: Two cases of AHS due to carbamazepine and a combination of sodium valproateand lamotrigene are reported. Both patients presented within the first month of starting the new antiepileptic medication with fever, skin rashes, hematological abnormalities, and hepatitis. The offending antiepileptic drugs were immediately stopped in both cases. Skin rashes responded to intravenous immunoglobulin in case 1 and to intravenous hydrocortisone in case 2. Conclusion: AHS is a serious, life-threatening condition. This report demonstrates that the most important steps in the management of AHS are to recognize the disorder, discontinue the offending antiepileptic drug, and provide supportive care in an inpatient setting and treat with benzodiazepines if seizures occur.
Intracranial hemorrhage (ICH) in the newborn is an uncommon presenting manifestation of hemophilia, occurring with an incidence of 1%-4%, 1-3 however, because of its dramatic consequences, this complication raises serious concern. Apart from its high mortality rate, over 50% of survivors are left with psychoneurological sequelae, especially seizures and motor impairment. 4 Only 18 cases of neonatal ICH occurring during the first week of life among hemophiliac infants have previously been reported.1,5-10 Here we present a one-week-old infant born in a family with positive history of hemophilia. With prompt replacement therapy and ventriculoperitoneal shunt inserted for progressive hydrocephalus, the child survived, but was left with gross motor retardation. Case ReportA male full-term infant with birth weight of 3.5 kg was born into a family with a known hemophiliac male child aged nine years. The parents were nonconsanguineous. They had four other children, two females and two males. The patient was screened for hemophilia on the second day after birth. Activated partial thromboplastin time (aPTT) was raised (111 seconds, control 34 seconds) and factor VIII was 1 U/mL. As the patient was asymptomatic, he was discharged on the fourth day. He showed no unusual bleeding even when the umbilical cord fell off on the fifth day. But soon after, the child became irritable and difficult to feed. Three days later at the age of one week, he was brought to hospital for evaluation. On examination, he was found to be hypoactive with full anterior fontanelle and cyanosis, which improved on suction and oxygenation. The patient's head circumference (HC) was 37.6 cm, heart rate was 150/min, respiratory rate was 60/min, and BP was 87/55 mm Hg. He was immediately transferred to ICU for close observation, but he deteriorated and became semiconscious, with shallow breathing. Arterial blood gas analysis showed respiratory acidosis that was managed with assisted ventilation. ICH was highly suspected and confirmed by CT scan of the head (Figure 1). Appropriate doses of cryoprecipitate were infused every 12 hours to raise factor VIII level to more than 50 units/mL. He also received top-up 45 mL of packed RBC. As the child developed progressive hydrocephalus, serial ventricular taps were performed initially, followed by insertion of ventriculoperitoneal shunt on the 20th day of age (Figure 2). Infusion of cryoprecipitate was continued for 16 days. A few days later, the patient regained consciousness, was crying for feeds and suckling well, and was moving all the limbs, though with slight increase in the tone and an exaggerated deep tendon reflexes. He was, therefore, extubated from the ventilator. HC was 39.5 cm with normal tension of anterior fontanelle. Presently, the patient is three years old. Neurodevelopmental assessment shows gross motor developmental delay with normal vision and hearing. HC is 46.5 cm (below 5th percentile for age). Apart from non-paralytic convergent squint, CNS examination is normal.
Cephalosporins are beta-lactam antibiotics which were first introduced in the 1960s. They are derived from cephalosporin C, which is a natural antimicrobial substance produced by the fungus Cephalosporium acremonium. The antimicrobial spectrum differs for first-, second-and third-generation cephalosporins, but in general activity increases with generation level. Cefuroxime (Zinacef, Glaxo Wellcome), a second-generation cephalosporin, is a commonly used drug in the pediatric population, mainly because of its wide range of antibacterial action and safety. Case 1The patient was a five-month-old boy, the product of a full-term pregnancy and normal vaginal delivery, with a birth weight of 3.2 kg. He required an incubator for three days because of tachypnea. From the age of 40 days, he had recurent episodes of wheezing, mainly precipitated by upper respiratory tract infections, and resolved with nebulized salbutamol for 3-5 days. At the age of five months, he was admitted with right middle lobe pneumonia and exacerbation of his asthma. His arterial blood gas (ABG) analysis on oxygen showed: pH 7.32, PCO 2 6.02 Kpa, PO 2 12.99 Kpa, and HCO 3 23.1 mmol/L. He was started on intravenous (IV) aminophylline, hydrocortisone 100 mg/kg, nebulized salbutamol, as well as IV cefuroxime and cloxacillin 100 mg/kg each. Bronchodilators were stopped on the fifth day, but on the eighth day of starting antibiotics, he suddenly developed gross abdominal distention, which resulted in increasing tachypnea. This was shortly followed by the passage of watery stools. Investigations showed serum potassium 4.0 mmol/L, sodium 137 mmol/L, and bicarbonate 12 mmol/L. A repeat ABG analysis on room air showed metabolic acidosis: pH 7.26, PCO 2 2.51 Kpa, PO 2 15.72 Kpa, HCO 3 8.2 mmol/L, and saturation 97.3%. Blood and stool cultures yielded no growth. The patient was kept nil by mouth (NPO) and all IV antibiotics were stopped. He showed marked improvement within 48 hours of stopping medications and was discharged home two days later.
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