Hemophilia a and spinal epidural hematoma in children.

Hemophilia A is an X-linked bleeding disorder, caused by deficient or defective blood coagulation factor VIII. The most characteristic symptoms of the severe forms of hemophilia A are joint and muscle bleeds. Intracranial hemorrhage occurs only in 3-10% of the patients. Spinal epidural hematomas are rarely seen. We describe a 13-month-old boy with hemophilia A who was admitted to the hospital because of irritability and unspecified pain for the past two days. There was no history of evident trauma, no fever. Physical investigation showed no skin lesions or hematomas and no obvious cause for the pain. Neurological examination showed a dysphoric toddler, mainly in the fetal position. No neurological abnormalities were found except for a miosis of the right pupil due to a suspected Horner syndrome. Magnetic resonance imaging of the spine showed an extensive epidural hematoma. The boy was successfully treated with intensive replacement therapy during three weeks and did not require surgical intervention. There was a rapid and complete clinical resolution. In conclusion, rare hematomas should be considered and searched for in children with bleeding disorders and not well understood complaints. Early diagnosis is important for the neurological outcome.

[A right-sided congenital diaphragmatic hernia masked by pneumonia]. / Een rechtszijdige congenitale hernia diaphragmatica gemaskeerd door een pneumonie.

A 1-day-old premature newborn (34 weeks and 6 days) presented with respiratory insufficiency due to a group B haemolytic streptococcal (GBS) pneumonia. She recovered after temporary treatment with mechanical ventilation and antibiotics. At the time of discharge there was a slight increased fogging on the right side of the chest X-ray, interpreted as residual pleural effusion. Three days later the patient was readmitted with respiratory failure and a need for respiratory support. A chest CT scan revealed a right-sided congenital diaphragmatic hernia (CDH) with a large part of the liver and intestine in the chest. The diaphragmatic defect was closed during a surgical procedure. After an uneventful recovery the patient was discharged in good clinical condition. The combination of delayed presentation of right-sided CDH and neonatal GBS infection occurs rarely but has been described. Its pathogenesis is still unclear.

Paroxetine and imipramine treatment of depressive patients in a controlled multicentre study with plasma amino acid measurements.

In a 12-week double-blind study with 36 patients with major depressive episode (DSM-III), paroxetine (Seroxat, Aropax) showed significantly quicker onset of efficacy on the Melancholia Scale, and better tolerance than imipramine. Plasma concentration analyses showed no clear concentration-efficacy correlation in either treatment group. During long-term treatment paroxetine seemed to be superior to imipramine in preventing relapse; both treatments were well tolerated. A significant correlation between baseline plasma tryptophan: large neutral amino acids ratio and final Hamilton Rating Scale for Depression (HRSD) score and a trend towards an inverse correlation between this ratio and percentage reduction in HRSD score were seen in the paroxetine group but not in the imipramine group. In line with previous studies, these results support the hypothesis that paroxetine is an effective and well tolerated antidepressant.