Dysphagia due to Esophageal Compression by Bronchial Artery Hypertrophy: A case report

Recurrent respiratory tract infections and dysphagia after the first years of life are rarely caused by vascular compression of the esophagus. We experienced a case of dysphagia and frequent vomiting resulted from esophageal compression by bronchial artery hypertrophy, which might had been aggravated by recurrent aspiration pneumonia caused by underlying swallowing difficulty. The patient initially had significant motor delay and swallowing difficulty. Videofluoroscopic swallowing studies demonstrated deglutition abnormalities and aspiration. In addition, significant amount of swallowed food was regurgitated through esophagus. On barium esophagography and angiography, posterior indentation of the esophagus without proximal dilatation and bronchial artery hypertrophy were noted without congenital heart disease. Pulmonary trunk and its branches showed normal appearance. Therefore, we considered that bronchial artery hypertrophy attributed to pulmonary artery fibrosis due to recurrent aspiration pneumonia may cause esophageal compression, which in turn contribute to dysphagia, frequent vomiting and recurrent aspiration pneumonia.

Coagulation Status of the Post-acute Spinal Cord Injured Patients

OBJECTIVE: Deep vein thrombosis (DVT) is one of the most common complications of acute spinal cord injury (SCI). It is well known that the incidence of DVT in post-acute SCI patients decreases to a level similar to that in the general population. The aim of this study was to evaluate the blood coagulation status and the possibility of DVT occurrence in post-acute SCI patients. METHOD: Twenty-three SCI patients (SCI group) were included in this study. Ten patients having spinal fracture with no evidence of SCI were used as the control group. Coagulation status was examined using factor VIII antigen, factor VIII procoagulant, fibrinogen, D-dimer, protein C, and protein S in both groups. Duplex ultrasonography was performed for the diagnosis of DVT. RESULTS: Prevalence of the abnormally decreased protein S level was significantly high in the SCI group (87%) than in the control group (30%). Prevalence of the abnormally increased F VIII:Ag, F VIII:C, and fibrinogen levels in the SCI group were significantly high in the SCI group (p<0.05). CONCLUSION: We conclude that post-acute SCI patients may have hypercoagulability. Proper physical prevention and thromboprophylaxis should be considered in post-acute SCI patients because they have relatively higher risk of thromboembolic complication.

Three Cases of Lesch-Nyhan Syndrome: Cases report

Lesch-Nyhan syndrome is a rare X-linked recessive metabolic disorder characterized by developmental delay, hyperuricemia, choreoathetosis, spasticity, mental retardation, and compulsive self-injurious behavior. This disorder results from a complete deficiency of the purine salvage enzyme, hypoxanthine-guanine phosphoribosyltransferase (HGPRT). This syndrome is often misdiagnosed to cerebral palsy and clinical manifestations are usually related to the degree of enzyme deficiency. Complete HGPRT deficiency presents with severe specific neurologic manifestation and nephrolithiasis leading to fatal kidney damage. This report highlighted the importance of clinical awareness leading to early diagnosis and therapy for prevention of the self mutilation and renal failure, even if we couldn't inhibit the progression of neuro-psychotic symptoms.