Perioperative management of a patient with Coffin-Lowry syndrome complicated by severe obesity: A case report and literature review.

RATIONALE: Coffin-Lowry syndrome (CLS) is a rare inherited disease with specific clinical features, such as mental retardation, facial dysmorphism, and cardiac abnormality. In particular, the characteristic facial features of CLS, including retrognathia and large tongue, are associated with difficult ventilation and/or intubation, which is a serious problem of anesthesia management. However, case reports on anesthesia management of CLS are very limited as there are only two published English reports till date. In this case report, we discuss anesthetic and postoperative considerations in patients with CLS, focusing on difficult airway management, and summarize past reports including some Japanese articles. PATIENT CONCERNS: A 25-year-old man with CLS was planning to undergo laminectomy because of progressive quadriplegia caused by calcification of the yellow ligament. We suspected difficulty in airway management because of several factors in his facial features, short thyromental and sternomental distances in computed tomography, severe obesity, and sleep apnea syndrome. DIAGNOSES: Difficult airway was suspected. However, because of mental retardation, awake intubation was considered difficult. INTERVENTIONS: We selected bronchofiberscope-guided nasotracheal intubation, maintaining spontaneous breathing under moderate sedation with a propofol target-controlled infusion. OUTCOMES: Airway management was safely performed during anesthesia induction. LESSONS: In many patients with CLS, difficult intubation was reported, and sedation or slow induction maintaining spontaneous breathing was mainly selected for anesthesia induction. Spontaneous breathing should be maintained during anesthesia induction in case of CLS patients.

Two Japanese Pioneers in Anesthesiology: Seishu Hanaoka and Gendai Kamada.

Seishu Hanaoka and Gendai Kamada are two Japanese pioneers in anesthesiology. Seishu Hanaoka was the world's first surgeon on record to successfully perform surgery under general anesthesia in 1804. Seishu discovered that six medicinal herbs containing Datura, stramonium, and Aconitum had anesthetic properties. From these, he developed Mafutsusan. His fame spread across Japan, and he was inundated with requests from patients and prospective students. He founded a private medical school (Shunrinken) and trained more than 1000 students. Gendai Kamada was an outstanding pupil of Seishu Hanaoka. From the perspective of the history of anesthesiology, three of Gendai's achievements had a global impact. (1) In 1839, he wrote the first textbook of clinical anesthesiology, Mafutsuto-ron; (2) in 1840, he authored Gekakihai-zufu, which included some of the oldest illustrations of surgery under general anesthesia; and (3) he trained Gensei Matsuoka, the world's second anesthesiologist.

Changes in Energy Levels by Dexamethasone in Ischemic Hearts and Brains in Male Mice.

BACKGROUND: Glucocorticoids have been shown to alleviate ischemia-induced myocardial injury, while aggravating neuronal damage caused by ischemia. As energy failure is a predominant factor in cellular viability, we examined the effects of glucocorticoids on energy utilization in the mouse heart and brain. METHODS: Seventy-two male ddY mice were assigned to 1 of 3 groups: saline (S), dexamethasone (a glucocorticoid without mineralocorticoid activity, 5 mg/kg) (D), and metyrapone (a potent inhibitor of the synthesis of glucocorticoids, 100 mg/kg) (M) groups (n=24 in each). Three hours after intraperitoneal administration, all animals were decapitated, and the heads were frozen in liquid nitrogen after 0, 0.5, 1, or 2 minutes (n=6 in each). The hearts were immediately removed and frozen in liquid nitrogen after 0, 5, 10, or 20 minutes of incubation at 37°C (n=6 in each). The concentrations of adenylates and monoamines were determined by high-performance liquid chromatography. RESULTS: In the heart, the adenosine 5'-triphosphate (ATP) concentration did not differ among the 3 groups at 0 minute of ischemia (3 h of S, D, or M treatment). Ischemia for 5 minutes decreased the ATP content to 21% of the basal level in the S group. The ATP decrease was suppressed by either the D or M treatment, such that after 5 minutes ATP levels were 63% and 64% of each basal level, respectively. In the brain, the ATP level in the M group was 62% of that in the S group at 0 minute of ischemia, and the 5'-monophosphate (AMP) level was 276% of that in the S group. Brain dopamine metabolism was facilitated by dexamethasone, and suppressed by metyrapone. CONCLUSIONS: The relationship between effects of glucocorticoids on ischemia-induced changes in energy levels and cellular viability was not clearly elucidated.

Management of patients under general anesthesia with mafutsu-san in Hanaoka-style surgery: comparisons of illustrations from Geka-Kihai-Zufu with descriptions from Mafutsuto-Ron and Yohka-Hiroku.

INTRODUCTION: Seishu Hanaoka (1760-1835) left behind no books that he himself had written. This is why many aspects of Hanaoka-style general anesthesia using a mixture of herbal extracts, which he called mafutsu-san, remain unknown. We are able to learn about this technique today because there are several descriptions of it in books written by his students, such as Mafutsuto-Ron ("Treatise on Mafutsuto") by Gendai Kamata (1794-1854) and Yohka-Hiroku ("Secret Records of Surgery") by Gencho Homma (1804-1872). On the other hand, Geka-Kihai-Zufu ("Illustrations of Surgical Cases"), a surgical textbook, by Gendai Kamata, containing one of the oldest illustrations of general anesthesia published in 1840, was recently rediscovered (2011). For the first time, this book revealed, in the form of a picture image, the actual circumstances of Hanaoka-style general anesthesia. METHODS: We therefore compared the descriptions of general anesthesia featured in these three documents, and thereby investigated the actual anesthetic management and the procedures used. RESULTS AND CONCLUSIONS: We found that the circumstances under which Hanaoka-style general anesthesia, using fabrics and futon mattresses, as well as blindfolding and constraining the patient's body during surgery, were exactly as described in Mafutsuto-Ron and Yohka-Hiroku. In addition, besides a surgeon conducting an operation, there was a physician who observed the patient's general condition. Gendai Kamata, the author of Geka-Kihai-Zufu, is believed to have recognized the importance of anesthetic care of surgical patients.

Mafutsuto-Ron: The First Anesthesia Textbook in the World. Bibliographic Review and English Translation.

On October 13, 1804, Seishu Hanaoka performed a mastectomy on Kan Aiya in Hirayama, Kii Province Japan, in what is considered to be the first operation under general anesthesia. She was anesthetized with a mixture of herbs known as Mafutsuto. Although Seishu did not record his anesthetic practices, his student, Gendai Kamada, documented the use of Mafutsuto in Mafutsuto-Ron. Written in 1839, Mafutsuto-Ron is 10 pages and covers six topics, including preoperative management, dosing and administering Mafutsuto, induction of general anesthesia, common errors, and postoperative precautions. Mafutsuto-Ron, therefore, meets the requirements to be considered the first extant anesthesia textbook. We present a complete English translation of Mafutsuto-Ron.

Combination therapy with sivelestat and recombinant human soluble thrombomodulin for ARDS and DIC patients.

BACKGROUND: Neutrophil elastase, alveolar thrombin generation, and fibrin deposition play crucial roles in the development of acute respiratory distress syndrome (ARDS) and disseminated intravascular coagulation (DIC). However, the usefulness of combination therapy with a selective neutrophil elastase inhibitor, sivelestat, and recombinant human soluble thrombomodulin (rhTM) for patients with ARDS and DIC remains unknown. METHODS: We conducted a retrospective data analysis of 142 ARDS patients with DIC to assess the effects of sivelestat combined with rhTM. Patients were divided into four groups: control (no sivelestat or rhTM treatment), sivelestat treatment alone, rhTM treatment alone, and combined treatment with sivelestat and rhTM. A Cox proportional hazard model was used to assess subject mortality rates. The efficacy of these drugs was evaluated based on survival rate, number of ventilator-free days, and change in PaO2/FIO2 (P/F) ratios and DIC scores before and at 7 days after a diagnosis of ARDS with DIC. RESULTS: Multivariate analysis showed that patient age, combination therapy, gas exchange, organ failure, cause, associated disease score, and serum C-reactive protein levels were predictors of mortality for patients with ARDS and DIC. As compared with untreated controls, combination therapy significantly improved the 60-day survival rate of patients with ARDS and DIC. There were significantly more ventilator-free days for those who received combination therapy than for untreated controls. P/F ratios and DIC scores were significantly improved with sivelestat alone, rhTM alone, or their combination as compared with untreated controls. CONCLUSION: Our results suggest that combined treatment with sivelestat and rhTM has beneficial effects on survival and the respiratory and DIC status of patients with ARDS and DIC.

[The looks of Gendai Kamada].

We realized the looks of Gendai Kamada. At first, we found in a figure, in "Seishu Hanaoka and His Surgery" by Syuzo Kure, that the portrait described as that of Gendai is his father's. And we discovered the illustrations that illustrate the looks of Gendai in "Gekakihaizufu", which was a textbook of clinical anesthesia and surgery, printed in 1840. Using these illustrations, we realized the looks of Gendai Kamada.

Usefulness of a selective neutrophil elastase inhibitor, sivelestat, in acute lung injury patients with sepsis.

BACKGROUND: Neutrophil elastase plays a crucial role in the development of acute lung injury (ALI) in patients with systemic inflammatory response syndrome (SIRS). The clinical efficacy of the neutrophil elastase inhibitor, sivelestat, for patients with ALI associated with SIRS has not been convincingly demonstrated. The aim of this study was to determine if there are clinical features of patients with this condition that affect the efficacy of sivelestat. METHODS: This was a retrospective study of 110 ALI patients with SIRS. Clinical information, including the etiology of ALI, the number of organs failing, scoring systems for assessing the severity of illness, and laboratory data, was collected at the time of diagnosis. Information on the number of ventilator-free days (VFDs) and changes in PaO(2)/F(I)O(2) (ΔP/F) before and 7 days after the time of ALI diagnosis was also collected. The effect of sivelestat on ALI patients was also examined based on whether they had sepsis and whether their initial serum procalcitonin level was ≥0.5 ng/mL. RESULTS: There were 70 patients who were treated with sivelestat and 40 control patients. VFDs and ΔP/F were significantly higher in the treated patients than in the control patients. However, there was no significant difference in the patient survival rate between the two groups. Sivelestat was more effective in ALI patients with a PaO(2)/F(I)O(2) ratio ≥ 140 mmHg or sepsis. Sivelestat significantly prolonged survival and led to higher VFDs and increased ΔP/F in septic patients and patients with initial serum procalcitonin levels ≥ 0.5 ng/mL. CONCLUSION: The results may facilitate a future randomized controlled trial to determine whether sivelestat is beneficial for ALI patients with sepsis.

Association of body temperature and antipyretic treatments with mortality of critically ill patients with and without sepsis: multi-centered prospective observational study.

INTRODUCTION: Fever is frequently observed in critically ill patients. An independent association of fever with increased mortality has been observed in non-neurological critically ill patients with mixed febrile etiology. The association of fever and antipyretics with mortality, however, may be different between infective and non-infective illness. METHODS: We designed a prospective observational study to investigate the independent association of fever and the use of antipyretic treatments with mortality in critically ill patients with and without sepsis. We included 1,425 consecutive adult critically ill patients (without neurological injury) requiring >48 hours intensive care admitted in 25 ICUs. We recorded four-hourly body temperature and all antipyretic treatments until ICU discharge or 28 days after ICU admission, whichever occurred first. For septic and non-septic patients, we separately assessed the association of maximum body temperature during ICU stay (MAXICU) and the use of antipyretic treatments with 28-day mortality. RESULTS: We recorded body temperature 63,441 times. Antipyretic treatment was given 4,863 times to 737 patients (51.7%). We found that treatment with non-steroidal anti-inflammatory drugs (NSAIDs) or acetaminophen independently increased 28-day mortality for septic patients (adjusted odds ratio: NSAIDs: 2.61, P=0.028, acetaminophen: 2.05, P=0.01), but not for non-septic patients (adjusted odds ratio: NSAIDs: 0.22, P=0.15, acetaminophen: 0.58, P=0.63). Application of physical cooling did not associate with mortality in either group. Relative to the reference range (MAXICU ≥ 39.5°C increased risk of 28-day mortality in non-septic patients (adjusted odds ratio 8.14, P=0.01), but not in septic patients (adjusted odds ratio 0.47, P=0.11) [corrected]. CONCLUSIONS: In non-septic patients, high fever (≥39.5°C) independently associated with mortality, without association of administration of NSAIDs or acetaminophen with mortality. In contrast, in septic patients, administration of NSAIDs or acetaminophen independently associated with 28-day mortality, without association of fever with mortality. These findings suggest that fever and antipyretics may have different biological or clinical or both implications for patients with and without sepsis. TRIAL REGISTRATION: ClinicalTrials.gov: NCT00940654.

[Incorrect descriptions about Seicho Kamata in "Seishu Hanaoka and his surgery"].

"Seishu Hanaoka and his surgery" by Shuzo Kure is one of the most important books for the study of Seishu Hanaoka. However, several incorrect descriptions have been pointed out in the book. Therefore, we checked the content about Seicho Kamata, a distinguished disciple of Seishu Hanaoka (p.154-163) in the book, and found three incorrect descriptions. The figure being described as that of Seicho Kamata is his father's. His graveyard being described as "Nyohoji" is truly "Daizenji". Seicho Kamata is also described as the second distinguished disciple of Seishu Hanaoka ; however, authors think that he was the first distinguished disciple from his career. Further investigation into the content of the book is necessary.

Elevated serum beta-D-glucan level and depressed neutrophil phagocytosis in a heatstroke patient.

Endotoxemia has been reported as a mechanism for the fatal sequela after heatstroke. Subsequent disseminated fungal infection in a heatstroke patient has been also described. Beta-D-glucan, a constituent of the fungal cell wall, is an early diagnostic measure for fungal infection. In a heatstroke case, we examined for the first time levels of serum beta-d-glucan and endotoxin. A 34-year-old man with a body temperature of 43.5 degrees C was admitted in a state of shock. Prior to the development of disseminated intravascular coagulopathy (DIC), a remarkable elevation of serum beta-D-glucan level to 116 pg/mL (normal level<6.0 pg/mL) was revealed on the first day of admission. However, serum endotoxin was not detected when using a method that excluded beta-D-glucan contamination from endotoxin measurement (normal level<1.0 pg/mL). This change of beta-D-glucan level was accompanied by a depressed neutrophil function, especially in phagocytosis of 34% (normal range 70-90%) but not in bacterocidal function (81% versus a normal range of 70-100%). After intensive care including continuous hemodiafiltration, the patient regained consciousness but remained ataxic due to cerebellar infarction, which might have resulted from DIC, and subsequent bilateral fungal oculitis were revealed 45 days after admission. This case report demonstrates the elevation of serum beta-D-glucan but normal endotoxin levels after heatstroke, which may prompt further study to re-examine the serum levels of endotoxin in such catastrophic insults.

Rupture of the gallbladder in a patient with acquired factor VIII inhibitors and systemic lupus erythematosus.

A 54-year-old woman with a 21-year history of systemic lupus erythematosus (SLE) was admitted to the Matsuyama Red Cross Hospital due to subcutaneous and gingival hemorrhaging. She was diagnosed with acquired factor VIII inhibitors based on a prolonged activated partial-thromboplastin time (APTT) and factor VIII inhibitors. Steroid pulse and factor VIII plasma concentrate were administered to her, not long after which she was transferred to Ehime University Hospital due to gallbladder hematoma. Although her APTT and factor VIII activity were improved after treatment with human factor VIII, she died of multiple organ failure. The autopsy demonstrated a ruptured gallbladder.

Enhancement of Na+,K(+)-ATPase and Ca(2+)-ATPase activities in multi-cycle ischemic preconditioning in rabbit hearts.

OBJECTIVE: Ischemic preconditioning (IP) has been shown to attenuate intracellular Na+ accumulation and Ca2+ overload during ischemia and reperfusion, both of which are closely related to the outcome of myocardial damage. We compared the effects of single- and four-cycle IP in Na+,K(+)-activated adenosine 5'-triphosphatase (Na+,K(+)-ATPase) and Ca(2+)-activated adenosine 5'-triphosphatase (Ca(2+)-ATPase) activities in in vivo rabbit hearts, correlating these differences to the quality of protection against subsequent ischemia. METHODS: The morphological outcome was evaluated in in vivo rabbit hearts subjected to 30 min of coronary occlusion and reperfusion for 180 min by assessing the ratio of infarct volume to risk zone volume. The effects of single- and four-cycle preconditioning ischemia were then examined. Another set of in vivo rabbit hearts was subjected to the measurement of ATPase activities at the conclusion of final preconditioning ischemia and at 60 min after reperfusion following 30 min of ischemia. RESULTS: The infarct volume was reduced by single-cycle IP to 38% of that in the control group. The four-cycle IP further reduced the infarct volume, which was 11% of that in the control group. Na+,K(+)-ATPase activity at 60 min after reperfusion in the four-cycle group was increased to 172% of that in the control group (10.8 micromol ADP/h/mg protein), whereas no difference was found in the single-cycle group. On the other hand, Ca(2+)-ATPase activity at the conclusion of IP was increased by single-cycle IP, the value being 255% of that in the control group (4.9 micromol ADP/h/mg protein). The four-cycle IP further increased the activity, and the value was 158% of that in the single-cycle group. CONCLUSIONS: Since increases in Na+,K(+)-ATPase and Ca(2+)-ATPase activities contribute to the decrease in intracellular Ca2+ concentration, the enhancement of these activities by four-cycle IP may be involved in the additional protection.

Survey of patients whose lungs could not be ventilated and whose trachea could not be intubated in university hospitals in Japan.

PURPOSE: We conducted a survey to clarify the actual circumstances in which the lungs could not be ventilated and the trachea could not be intubated (CVCI). METHODS: A questionnaire was mailed to all the university hospitals in Japan, asking about CVCI they had experienced during induction of anesthesia in 1998, and before 1997. RESULTS: Answers were obtained from 60 of 83 institutes. CVCI occurred in 26 of 151 900 cases of general anesthesia (0.017%) in 1998. Eighteen cases occurred after induction of anesthesia by several induction methods. Five cases occurred after repeated attempts at tracheal intubation by laryngoscopy and fiberscopy in patients under awake or anesthetized conditions. In the remaining 3 cases, the situation of occurrence was not documented. Patients with CVCI had anatomical abnormalities around the upper airways, mostly from acquired diseases. CVCIs after induction of anesthesia were successfully treated by restoration of spontaneous respiration, blind intubation, laryngeal mask airway, and transtracheal approaches, and CVCIs after repeated attempts at intubation were treated mostly by transtracheal approaches. No serious consequences occurred in any patients in 1998. Twenty cases were reported before 1997, and 2 were specific, in which CVCI followed malplacement of a tracheal tube, and serious consequences, death and brain damage, respectively, followed. In other patients, no serious consequences occurred, although cardiac arrest occurred in 1 patient. CONCLUSION: This survey demonstrates that CVCI can occur in any situation in which the airway is not established. Furthermore, effective treatments may be different depending on the situation, and delayed recognition of tracheal tube misplacement may lead to a serious outcome.