Successful recovery from cardiac arrest due to atlantoaxial subluxation in Down syndrome: a case report.

INTRODUCTION: Down syndrome is the most common chromosomal abnormality associated with intellectual impairments. Unexpected deaths are common with this disease. There are certain difficulties in clarifying the cause of death because the manifestations may be quite diverse and involve many organ systems. Atlantoaxial subluxation is a dangerous complication of Down syndrome, as it may lead to cervical cord-medullary compression. CASE PRESENTATION: Herein, we present a case of Down syndrome in a patient who completely recovered from cardiac arrest due to atlantoaxial subluxation. The neck was immobilized during post-cardiac arrest care, and the patient underwent surgery after 14 days. The patient could walk independently and was discharged 3 months later. At the last follow-up 5 years after surgery, the patient's general condition was good. DISCUSSION: Physicians should be aware that atlantoaxial instability can cause cardiac arrest in patients with genetic syndromes.

Diagnostic Pitfalls in Papillary Muscle Rupture-Associated Acute Mitral Regurgitation after Acute Myocardial Infarction.

Papillary muscle rupture (PMR) is a rare and fatal complication of acute myocardial infarction (AMI). We report a case of acute mitral regurgitation (MR) due to PMR with pulmonary edema and cardiogenic shock following AMI with small myocardial necrosis. An 88-year-old woman was brought to our emergency department in acute respiratory distress, shock, and coma. She had no systolic murmur, and transthoracic echocardiography was inconclusive. Coronary angiography showed obstruction of the posterior descending branch of the right coronary artery. Although the infarction was small, the hemodynamics did not improve. Transesophageal echocardiography established papillary muscle rupture with severe mitral regurgitation 5 days after admission. Thereafter, the patient and her family did not consent to heart surgery, and she eventually died of progressive heart failure. Physicians should be aware of papillary muscle rupture with acute mitral regurgitation following AMI in patients with unstable hemodynamics, no systolic murmur, and no abnormalities revealed on transthoracic echocardiography.

Efficacy of protocol-based non-invasive positive pressure ventilation for acute respiratory distress syndrome: a retrospective observational study.

AIM: The efficacy of non-invasive positive pressure ventilation (NPPV) in acute respiratory distress syndrome (ARDS) remains unclear. Variation in both the etiology of ARDS and patient factors has resulted in inconsistent application of NPPV. We have developed a protocol-based NPPV strategy as a first-line intervention for ARDS. The aim of this observational study was to determine if protocol-based NPPV improves the outcome in patients with ARDS. METHODS: We identified patients with ARDS treated by protocol-based NPPV at our institution between March 2006 and March 2010 and categorized them according to NPPV success or failure. Success was defined as avoidance of intubation and remaining alive during NPPV. RESULTS: Eighty-eight of 169 patients diagnosed with ARDS during the study period were treated using the protocol. Fifty-two (76%) of 68 patients who were eligible for the study were successfully treated and did not require endotracheal intubation. The overall mortality rate at 28 days after initiation of NPPV was 12%. The mortality rate was significantly lower in the success group than in the failure group (P < 0.01). The PaO2/FiO2 ratio after 12-24 h of NPPV was significantly higher in the success group than in the failure group (202 ± 63 versus 145 ± 46; P < 0.01). CONCLUSIONS: The success rate was higher and the mortality was lower in patients than in historical controls. Protocol-based NPPV could be effective in patients with ARDS.

Complete cervical tracheal transection due to blunt trauma.

Case: A 31-year-old man was caught up in the rotor of a snow-removing truck. He was diagnosed with tension pneumothorax and managed with tube thoracostomy in the ambulance. But he was left with respiratory discomfort. Computed tomography scan suggested the diagnosis of complete cervical tracheal transection. Outcome: The endotracheal tube was advanced distal to the transection site under bronchoscopic guidance, which stabilized the patient's cardiopulmonary condition. The tracheal injury healed well after emergent surgical repair. Conclusion: Complete cervical tracheal transection is rare and requires a high index of suspicion for timely diagnosis. It is important to secure the airway, which can be done by fiberoptic bronchoscopy.

[Ileus and intestinal obstruction].

Ileus and intestinal obstruction are common diseases for the elderly. Ileus is caused as the results of severe pain, infections and medications. Intestinal obstruction is one of the first etiologies of surgical emergency procedures. Intestinal obstruction is mainly due to the abdominal wall hernias and oncologic problems in the elderly. The fatal intestinal obstruction is not negligible in the elderly. Intestinal obstruction, especially in the demented elderly, can present late and with deceptively minimal signs and symptoms. We described the features and notes of ileus and intestinal obstruction in the elderly emergency patients.

A role for mDia, a Rho-regulated actin nucleator, in tangential migration of interneuron precursors.

In brain development, distinct types of migration, radial migration and tangential migration, are shown by excitatory and inhibitory neurons, respectively. Whether these two types of migration operate by similar cellular mechanisms remains unclear. We examined neuronal migration in mice deficient in mDia1 (also known as Diap1) and mDia3 (also known as Diap2), which encode the Rho-regulated actin nucleators mammalian diaphanous homolog 1 (mDia1) and mDia3. mDia deficiency impaired tangential migration of cortical and olfactory inhibitory interneurons, whereas radial migration and consequent layer formation of cortical excitatory neurons were unaffected. mDia-deficient neuroblasts exhibited reduced separation of the centrosome from the nucleus and retarded nuclear translocation. Concomitantly, anterograde F-actin movement and F-actin condensation at the rear, which occur during centrosomal and nuclear movement of wild-type cells, respectively, were impaired in mDia-deficient neuroblasts. Blockade of Rho-associated protein kinase (ROCK), which regulates myosin II, also impaired nuclear translocation. These results suggest that Rho signaling via mDia and ROCK critically regulates nuclear translocation through F-actin dynamics in tangential migration, whereas this mechanism is dispensable in radial migration.

POEMS syndrome caused refractory ascites in a polycystic disease patient undergoing hemodialysis.

A 60-year-old man with polycystic disease (PCD) undergoing hemodialysis was admitted to our hospital because of refractory ascites in September 2000. He had been diagnosed with probable chronic inflammatory demyelinating polyradiculopathy 6 months before admission. Though the ascites was bloody and exudative, the cytology was normal and cultures of bacteria and acid-fast bacillus were both negative. Hepatic venous outflow obstruction was excluded by several radiological examinations. Because of the presence of polyneuropathy, organomegaly, endocrine abnormality, M-protein, plasma cell dyscrasia, and skin lesions, POEMS, syndrome was diagnosed; this had caused the refractory ascites. Initial prednisolone therapy was effective for the refractory ascites, but it was not effective in preventing recurrence. He died due to cachexia in December 2000. This is a very rare case of the presence of both PCD and POEMS syndrome in a patient.

A case of nephrotic syndrome due to lupus nephritis which was controlled with low-density lipoprotein apheresis.

Our report discusses a 29 year old female patient with nephrotic syndrome due to lupus nephritis, biopsy-proven World Health Organization classification Types IVb and V that was controlled with low-density lipoprotein (LDL) apheresis. She was initially treated with steroid therapy, including methylprednisolone pulse therapy, and the serological activity of her systemic lupus erythematosus was suppressed. However, her nephrotic state, accompanied by severe hyperlipidemia, persisted despite the steroid therapy. Since we could not obtain her consent to administer immunosuppressants such as cyclophosphamide, we tried to treat her using LDL apheresis (LDL-A). We found that her urine protein excretion, hyperlipidemia, hypoalbuminemia, and renal function improved following the initiation of LDL-A. This suggests that LDL-A may be an effective therapy for nephrotic syndrome due to lupus nephritis through short-term amelioration of hyperlipidemia.