Good's Syndrome Accompanied by Agranulocytosis Following a Rapid Clinical Course.

Good's syndrome is an immunodeficiency disease involving thymoma accompanied by hypogammaglobulinemia. We encountered a case of Good's syndrome accompanied by agranulocytosis that followed a rapid clinical course. A 72-year-old man visited our hospital with a two-week history of a sore throat. Candida albicans was detected in the pharynx, and hypogammaglobulinemia was detected in addition to granulocytopenia. The patient subsequently developed septic shock and followed a rapid clinical course which ended in death. Good's syndrome with agranulocytosis was diagnosed at autopsy. Good's syndrome accompanied by agranulocytosis can follow a rapid clinical course and some cases remain asymptomatic until old age. Its prompt treatment is crucial.

Current termination of resuscitation (TOR) guidelines predict neurologically favorable outcome in Japan.

BACKGROUND: It is unclear whether the basic life support (BLS) and advanced life support (ALS) pre-hospital termination of resuscitation (TOR) rules developed in North America can be applied successfully to patients with out-of-hospital cardiac arrest (OHCA) in other countries. OBJECTIVES: To assess the performance of the BLS and ALS TOR in Japan. METHODS: Retrospective nationwide, population-based, observational cohort study of consecutive OHCA patients with emergency responder resuscitation attempts from 1 January 2005 to 31 December 2009 in Japan. The BLS TOR rule has 3 criteria whereas the ALS TOR rule includes 2 additional criteria. We extracted OHCA patients meeting all criteria for each TOR rule, and calculated the specificity and positive predictive value (PPV) of each TOR rule for identifying OHCA patients who did not have neurologically favorable one-month survival. RESULTS: During the study-period, 151,152 cases were available to evaluate the BLS TOR rule, and 137,986 cases to evaluate the ALS TOR rule. Of 113,140 patients that satisfied all three criteria for the BLS TOR rule, 193 (0.2%) had a neurologically favorable one-month survival. The specificity of BLS TOR rule was 0.968 (95% CI: 0.963-0.972), and the PPV was 0.998 (95% CI: 0.998-0.999) for predicting lack of neurologically favorable one-month survival. Of 41,030 patients that satisfied all five criteria for the ALS TOR rule, just 37 (0.1%) had a neurologically favorable one-month survival. The specificity of ALS TOR rule was 0.981 (95% CI: 0.973-0.986), and the PPV was 0.999 (95% CI: 0.998-0.999) for predicting lack of neurologically favorable one-month survival. CONCLUSIONS: The prehospital BLS and ALS TOR rules performed well in Japan with high specificity and PPV for predicting lack of neurologically favorable one-month survival in Japan. However, the specificity and PPV were not 1000 and we have to develop more specific TOR rules.

Comparison of supraglottic airway versus endotracheal intubation for the pre-hospital treatment of out-of-hospital cardiac arrest.

INTRODUCTION: Both supraglottic airway devices (SGA) and endotracheal intubation (ETI) have been used by emergency life-saving technicians (ELST) in Japan to treat out-of-hospital cardiac arrests (OHCAs). Despite traditional emphasis on airway management during cardiac arrest, its impact on survival from OHCA and time dependent effectiveness remains unclear. METHODS: All adults with witnessed, non-traumatic OHCA, from 1 January 2005 to 31 December 2008, treated by the emergency medical services (EMS) with an advanced airway in Osaka, Japan were studied in a prospective Utstein-style population cohort database. The primary outcome measure was one-month survival with neurologically favorable outcome. The association between type of advanced airway (ETI/SGA), timing of device placement and neurological outcome was assessed by multiple logistic regression. RESULTS: Of 7,517 witnessed non-traumatic OHCAs, 5,377 cases were treated with advanced airways. Of these, 1,679 were ETI while 3,698 were SGA. Favorable neurological outcome was similar between ETI and SGA (3.6% versus 3.6%, P = 0.95). The time interval from collapse to ETI placement was significantly longer than for SGA (17.2 minutes versus 15.8 minutes, P < 0.001). From multivariate analysis, early placement of an advanced airway was significantly associated with better neurological outcome (Adjusted Odds Ratio (AOR) for one minute delay, 0.91, 95% confidence interval (CI) 0.88 to 0.95). ETI was not a significant predictor (AOR 0.71, 95% CI 0.39 to 1.30) but the presence of an ETI certified ELST (AOR, 1.86, 95% CI 1.04 to 3.34) was a significant predictor for favorable neurological outcome. CONCLUSIONS: There was no difference in neurologically favorable outcome from witnessed OHCA for ETI versus SGA. Early airway management with advanced airway regardless of type and rhythm was associated with improved outcomes.

Impact of transport to critical care medical centers on outcomes after out-of-hospital cardiac arrest.

BACKGROUND: Post-resuscitation care has emerged as an important predictor of survival from out-of-hospital cardiac arrest (OHCA). In Japan, selected hospitals are certified as Critical Care Medical Centers (CCMCs) based on their ability and expertise. HYPOTHESIS: Outcome after OHCA is better in patients transported to a CCMC compared a non-critical care hospital (NCCH). MATERIALS AND METHODS: Adults with OHCA of presumed cardiac etiology, treated by emergency medical services systems, and transported in Osaka from January 1, 2005 to December 31, 2007 were registered using a prospective Utstein style population cohort database. Primary outcome measure was 1 month neurologically favorable survival (CPC< or =2). Outcomes of patients transported to CCMC were compared with patients transported to NCCH using multiple logistic regressions and stratified on the basis of stratified field ROSC. RESULTS: 10,383 cases were transported. Of these, 2881 were transported to CCMC and 7502 to NCCH. Neurologically favorable 1-month survival was greater in the CCMC group [6.7% versus 2.8%, P<0.001]. Among patients who were transported to hospital without field ROSC, neurologically favorable outcome was greater in the CCMC group than the NCCH group [1.7% versus 0.5%; adjusted odds ratio (OR), 3.39; 95% confidence interval (CI), 2.17-5.29; P<0.001]. In the presence of field ROSC, survival was similar between the groups [43% versus 41%; adjusted OR, 1.09; 95% CI, 0.82-1.45; P=0.554]. CONCLUSIONS: Survival after OHCA of presumed cardiac etiology transported to CCMCs was better than those transported to NCCHs. For OHCA patients without field ROSC, transport to a CCMC was an independent predictor for a good neurological outcome.

[Renal transection conservatively treated three times by selectively transarterial embolization (TAE): a case report].

A 12-year-old-man presented with left flank pain after a traffic accident on October 14, 2006. Computed tomography (CT) revealed major left renal hematoma and transection (IIIb). Selectively transarterial embolization (TAE) was performed to control upper transected renal bleeding on the same day, and again to do rebleeding two days later. Because CT revealed left perirenal urinoma caused by upper transected kidney on October 18, TAE was performed for the upper transected kidney not to function. Five months after left renal injury, CT demonstrated the left kidney successfully preserved without hydronephrosis, urinoma and hematoma. The patient was well and could be conservatively treated without hypertension and other complications. In previous reports, only a part of renal injury (III) cases with conservative treatment converted to nephrectomy, whereas approximately half of them with surgical treatment resulted in nephrectomy. Therefore, it is important to treat them as conservatively as possible and to preserve renal function, even in cases of major renal blunt injury.