Long-term treatment with sildenafil in chronic thromboembolic pulmonary hypertension.

For chronic thromboembolic pulmonary hypertension not amenable to pulmonary endarterectomy, effective medical therapy is desired. In an open-label uncontrolled clinical trial, 104 patients (mean +/- sem age 62 +/- 11 yrs) with inoperable chronic thromboembolic pulmonary hypertension were treated with 50 mg sildenafil t.i.d. At baseline, patients had severe pulmonary hypertension (pulmonary vascular resistance 863 +/- 38 dyn.s.cm(-5)) and a 6-min walking distance of 310 +/- 11 m. Eight patients were in World Health Organization functional class II, 76 in class III and 20 in class IV. After 3 months' treatment, there was significant haemodynamic improvement, with reduction of pulmonary vascular resistance to 759 +/- 62 dyn.s.cm(-5). The 6-min walking distance increased significantly to 361 +/- 15 m after 3 months' treatment, and to 366 +/- 18 m after 12 months' treatment. A subset of 67 patients received a single dose of 50 mg sildenafil during initial right heart catheterisation. The acute haemodynamic effect of this was not predictive of long-term outcome. In this large series of patients with inoperable chronic thromboembolic pulmonary hypertension, open-label treatment with sildenafil led to significant long-term functional improvement. The acute effect of sildenafil may not predict the long-term outcome of therapy.     

Osteotomy of the tibial tubercle in complex primary total knee replacement

Objective  Stiffness and severe deformity pose a major challenge in total knee arthroplasty. Numerous techniques have been described to gain exposure and improve knee flexion. Tibial tubercle osteotomy provides excellent and safe exposure of the joint, although mechanical and wound complications have been reported. Materials and methods  We present a series of 32 consecutive complex primary total replacements where an osteotomy of the tibial tubercle was utilised. Results  The patients had a mean follow-up of 2 years and 11 months. Following the procedure, with the exception of one case complicated with deep infection, all of the patients had improved clinically. The mean postoperative range of motion had increased to 102° (give P value < 0.005) and there were no cases of delayed union or non-union. A mechanical complication related to technique occurred in one patient; there were no other cases with a postoperative extension lag. Conclusion  In this challenging population group, we have found a tibial tubercle tuberosity osteotomy to greatly facilitate exposure without compromising the clinical and radiographic outcome.     

Acute encephalopathy associated with influenza and other viral infections

Acute encephalopathy is the most serious complication of pediatric viral infections, such as influenza and exanthem subitum. It occurs worldwide, but is most prevalent in East Asia, and every year several hundreds of Japanese children are affected by influenza-associated encephalopathy. Mortality has recently declined, but is still high. Many survivors are left with motor and intellectual disabilities, and some with epilepsy. This article reviews various syndromes of acute encephalopathy by classifying them into three major categories. The first group caused by metabolic derangement consists of various inherited metabolic disorders and the classical Reye syndrome. Salicylate is a risk factor of the latter condition. The second group, characterized by a systemic cytokine storm and vasogenic brain edema, includes Reye-like syndrome, hemorrhagic shock and encephalopathy syndrome, and acute necrotizing encephalopathy. Non-steroidal anti-inflammatory drugs, such as diclofenac sodium and mephenamic acid, may aggravate these syndromes. Severe cases are complicated by multiple organ failure and disseminated intravascular coagulation. Mortality is high, although methylprednisolone pulse therapy may be beneficial in some cases. The third group, characterized by localized edema of the cerebral cortex, has recently been termed acute encephalopathy with febrile convulsive status epilepticus, and includes hemiconvulsion-hemiplegia syndrome and acute infantile encephalopathy predominantly affecting the frontal lobes. Theophylline is a risk factor of these syndromes. The pathogenesis is yet to be clarified, but an increasing body of evidence points to excitotoxicity and delayed neuronal death.     

Lumbar Spinal ‘Juxtaarticular’ Cyst in a Gordon Setter

An 11‐year‐old Gordon setter bitch was presented with a history of progressive weakness in the right hind limb associated with pain in the lumbar spine. Neurological deficits consisted of ataxia, monoparesis, muscle atrophy and spontaneous over‐knuckling of the affected limb. A large ‘juxtaarticular’ cyst located in a right dorsolateral position of the intervertebral foramen at L3–L4 was diagnosed by magnetic resonance imaging. The cyst was removed through a modified laminectomy. The dog recovered quickly and returned to the owners 4 days after surgery with slight neurological symptoms. During the follow‐up examination 2 and 6 months later, the Setter showed normal gait and neurological examination.