An Autopsy Case of Tracheal Agenesis with Broncho-esophageal Fistula

Abstract True incidence of this malformation is probably greater than that reported since the definitive diagnosis has been made at autopsy in most cases. Various hypotheses on the pathogenesis of tracheal agenesis have been proposed but they are still controversial.
In this report, we present a case of tracheal agenesis with a broncho-esophageal fistula and discuss the formal genesis.     

Characteristics of juvenile dermatomyositis in Japan

Questionnaires were sent to 1290 hospitals in Japan asking for data on patients with juvenile dermatomyositis (JDM) diagnosed between June 1984 and May 1994. Of the 204 patients identified by these questionnaires, 102 met the criteria for JDM. JDM is categorized into three subtypes: Banker-type JDM , Brunsting-type and fulminant-type; patients with the latter exhibit markedly elevated serum levels of creatinine phosphokinase (> 10 000 U/mL) and appear to be at risk of renal failure. Cutaneous manifestations were present in 98% of patients and preceded the appearance of other symptoms. This tendency is one of the reasons for the difficulty in some cases in diagnosing the onset of JDM. Better criteria for early treatment of JDM are needed. The results of the present study suggest that itching and calcinosis are factors that indicate a poor prognosis in patients with JDM. Muscle enzyme levels do not always reflect disease activity, suggesting that methods other than measurement of muscle enzymes, such as measurement of the levels of neoprerin and von Willebrand factor antigen, as well as magnetic resonance imaging should be used to be evaluate disease severity. Patients with Brunsting-type JDM who exhibit dysphagia and antinuclear antibody positivity and patients with Banker-type JDM should be treated aggressively. Pulse therapy should be selected as the initial therapy in patients with fulminant-type JDM.     

Theophylline metabolism in acute asthma with MxA-indicated viral infection

BACKGROUND: Although viral infection might alter theophylline metabolism in acute asthma, there are some difficulties in detecting infection due to various kinds of viruses in a clinical setting. METHODS: To evaluate the usefulness of assessment of MxA protein in acute asthma exacerbated by viral infection, MxA protein expression in lymphocytes was assayed by flow cytometric analysis in whole peripheral blood in 21 children (aged 0-6 years) receiving continuous theophylline infusion for management of asthma attack. Serum theophylline levels were measured at 24 and 72 h after initiating theophylline infusion. RESULTS: At the beginning of theophylline infusion, 11 children had increased expression of MxA protein, indicating viral infected states. After 24 h continuous infusion, there were no differences in theophylline levels between MxA-negative and MxA-positive groups. After 72 h infusion, the mean theophylline level of MxA-positive children was significantly higher than that of MxA-negative children (9.7 +/- 2.2 microg/mL vs 7.3 +/- 1.6 microg/mL). The ratio of theophylline clearance at 72 h to that at 24 h in the MxA-positive group was significantly lower than that of the MxA-negative group (1.1 +/- 0.2 vs 1.4 +/- 0.1). CONCLUSIONS: Viral infection appeared to affect theophylline metabolism. Flow cytometric assay of lymphoid MxA protein expression in whole blood is an easy and useful method of evaluating viral infection in acute asthma exacerbation.     

False Tendon‐Related Polymorphic Ventricular Tachycardia

A 39‐year‐old woman showed nonsustained polymorphic ventricular tachycardia (PVT) during light physical activity. Cardiac multidetector row computed tomography demonstrated false tendons, one of which proved to be the focus triggering premature ventricular contraction (PVC) in electrophysiological studies. The triggered PVC arose during the diastolic period, which might have caused tension in the false tendon. Radiofrequency catheter ablation targeting the triggered PVC by pace mapping was performed and proved partially effective against PVT. (PACE 2012;35:e341–e344)     

Influence of Body Position on Defecation in Humans

Objectives: To compare three positions for defecation by measuring abdominal pressure and the anorectal angle simultaneously. Methods: We recruited six healthy volunteers. The videomanometric measures included simultaneous fluoroscopic images, abdominal pressures, subtracted rectal pressures and anal sphincter pressures. Three positions were used: sitting, sitting with the hip flexing at 60 ° with respect to the rest of the body, and squatting with the hip flexing at 22.5 ° with respect to the rest of the body. Results: Basal abdominal pressure before defecation on hip‐flex sitting was lower than that with normal sitting, although the difference did not reach statistical significance. Basal abdominal pressure before defecation on squatting (26 cmH₂O) was lower than that with normal sitting (P < 0.01). Abdominal pressure increase (strain) on hip‐flex sitting was lower than that with normal sitting, although this difference did not reach statistical significance. Similarly, the abdominal pressure increase on squatting was smaller than that with normal sitting, and yet the difference did not reach statistical significance. The rectoanal angle on defecation on hip‐flex sitting did not differ from that with normal sitting. The rectoanal angle on defecation on squatting (126 °) was larger than that with normal sitting (100 °) (P < 0.05), and was also larger than that with hip‐flex sitting (99 °) (P < 0.01). Conclusion: The results of the present study suggest that the greater the hip flexion achieved by squatting, the straighter the rectoanal canal will be, and accordingly, less strain will be required for defecation.     

Application of Deep Brain Stimulation for Treatment-resistant Obsessive Compulsive Disorder: Current Status and Future Perspectives in Japan

As in many Western countries, deep brain stimulation (DBS) is already being used daily in Japan to clinically treat neurological diseases such as Parkinson’s disease, essential tremor, and dystonia. Additionally, in both Europe and the United States, numerous case reports as well as multicenter randomized controlled trials have examined its use for treatment-refractory mental illnesses such as obsessive compulsive disorder (OCD) and major depressive disorder. Based on a number of the reports, the European Union (EU) and the USA Food and Drug Administration (FDA) granted limited approval of DBS for treatment-resistant OCD in 2009. Furthermore, a systematic review and meta-analysis in 2015 showed that DBS therapy for patients with treatment-resistant OCD had efficacy and was safe. Unlike the EU and the USA, DBS is not used to treat OCD or other psychiatric disorders in Japan, even though people with treatment-resistant OCD and their physicians and families urgently need additional treatments. This situation results from the “Resolution of total denial for psychosurgery,” which the Japanese Society of Psychiatry and Neurology adopted in 1975. We believe that the appropriateness of using DBS for treating psychiatric disorders including OCD should be considered after thorough discussion and consideration based on accurate and objective understanding. Currently, the field of psychiatry in Japan seems to lack scientific consideration as well as scientific understanding in this area. Under these circumstances, we hope that this review article will help psychiatrists and other relevant parties in Japan to gain an accurate and scientific understanding of DBS.     

Interactions of Endotoxin with Cortisol and Acute Phase Proteins in Septic Shock Neonates

ABSTRACT. CRP, α1-acid glycoprotein and haptoglobin were studied in 13 septic shock neonates. Endotoxin was recovered from eight infants. Serum Cortisol concentration from infants with en-dotoxemia (917 ± 596 ng/ml) was significantly higher than that from infants without en-dotoxemia (398 ± 239 ng/ml). Serum Cortisol correlated well with immature neutrophil counts denned as the unit "band/neutrophil". Increased Cortisol level and immature neutrophil counts preceded the elevation of CRP, α1-acid glycoprotein and haptoglobin in four extremely premature neonates. We conclude that positive interactions between endotoxin, Cortisol and acute phase protein synthesis are present in the initial period of infection, and delayed acute phase protein synthesis is suspected in extremely premature neonates.