We retrospectively reviewed clinical characteristics and treatment outcome on 11 pancreaticoduodenal artery aneurysm (PDAA) cases from 1990 to 2013, including nine cases of ruptured PDA and two cases of non-ruptured PDAA. Regarding ruptured sites, there were seven cases of retroperitoneal ruptures and one case each of intraduodenal rupture and intraperitoneal rupture. The leading symptom was abdominal pain. However, in the case of retroperitoneal rupture, the symptoms were resolved in some cases. This finding clearly demonstrates that close attention needs to be paid to clinical characteristics. Narrowing or obstruction of the celiac artery was observed in seven cases and patency was observed in four cases. Treatment was performed with transcatheter arterial embolization (TAE) for eight cases, TAE + ligation for two cases and lump excision for one case. TAE was combined with abdominal section, or vice versa, depending on the condition of the patient. TAE-related complications were observed in three cases and a surgical complication in two cases. Additional operations, such as bypass, arcuate ligament incision or stenting, were not performed in any case because complications due to ischemia were not recognized. Symptoms or findings of recurrence were also not recognized during the observation period. TAE is the first-line choice of treatment, which could be combined with open surgery. According to our research review findings, recurrence after treatment is unlikely. Therefore, we consider it unnecessary to perform additional operations.

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Our hospital is one of the largest pediatric facilities in Japan, with 490 beds, 20 beds in the pediatric intensive care unit (PICU), and approximately 7000 annual inpatients. Our hospital has used the rapid response system (RRS) since its introduction in February 2011. To investigate the benefits and challenges of the RSS, we compared RRS-related items during the 2-year period before with the 2-year period after the introduction of the RRS. The RRS was used for a mean of 74 times per month and 117 times per 1,000 hospitalizations. No improvement was observed in the number of inpatient deaths (before vs. after introduction: 1.7 vs. 2.0/1,000 hospitalizations; p = 0.42), unexpected in-hospital cardiac arrests (0.4 vs. 0.5/1000 hospitalizations; p = 0.94), or code blue calls (0.7 vs. 0.9/1,000 hospitalizations; p = 0.61). However, a significant improvement was observed in the number of PICU deaths (23.4 vs. 12.6/1000 hospitalizations; p < 0.01). In addition, a tendency, although nonsignificant, toward improvement was observed in the number of deaths in patients unexpectedly admitted to the PICU owing to in-hospital acute deterioration (70.9 vs. 41.4/1,000 hospitalizations; p = 0.42). RRS use varied between the wards. The RRS use rate for PICU cases with in-hospital acute deterioration was low (40.7%); for the other cases, only the primary department was called. Furthermore, the clinical characteristics of cases in which the RRS was difficult to apply included convulsions, decreased consciousness level, and upper airway stenosis. This study showed an insufficient utilization of the RRS, which limited the effect of its introduction. To improve pediatric outcomes with RRS use, compliance with RRS criteria, improvement in the use rate, criteria review, and post-revision evaluations are required.

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In this report, we describe a case of late-onset respiratory depression and seizure caused by carbamazepine (CBZ) overdose. A 15-year-old female was admitted unconscious with suspicion of drug overdose. The drugs found at the scene were CBZ and mirtazapine. A urine drug screen was positive for tricyclic antidepressant. She suffered respiratory depression, seizure, mydriasis and oliguria after admission, which required mechanical ventilation and intravenous administration of anticonvulsant and lipid emulsion. The concentration of CBZ in her blood was high enough to diagnose intoxication. These symptoms improved in accordance with the decrease of CBZ concentration, and she was discharged without complications on hospital day 8. Approximately 100 tablets of CBZ (200 mg) and 50 of mirtazapine (15 mg) were ascertained to have been ingested 11 hours before arrival. The latency of respiratory depression and seizure after ingestion were 12 hours and 15 hours, respectively. The maximum blood concentration of CBZ was 104.5 µg/mL 20 hours after ingestion, and a concentration below the toxic level was confirmed 67 hours after ingestion. It should be emphasized that because of the considerable latency, a CBZ overdose might cause serious symptoms later. Therefore, close observation is required even if the initial symptoms seem minor.

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A 61-year-old man who sustained bruising after falling from a 1.5-m high fence was admitted to our hospital. On arrival, he was conscious and clinically stable. Chest computed tomography revealed multiple left rib fractures and hemopneumothorax; therefore, chest drainage was performed. He suddenly lost consciousness 56 h post-injury. Because his radial artery was not palpable, chest compression was performed; and he immediately regained consciousness. Over a short period of time, 140-mL blood was drained from the chest tube; thus, a second computed tomography was performed, which revealed extravasation around the heart apex. Lateral thoracotomy performed around the left fifth intercostal space revealed a sharp fractured edge of the fifth rib in contact with the pericardium, and bleeding immediately below the site. Blood oozed when the pericardium was incised; a 10-mm rupture to the left ventricle was discovered, which was repaired by inserting felt strips and pledgeted mattress sutures, thus restoring hemostasis. In this case, there was no pericardial effusion on admission. Thus, we believe that although the fractured rib caused the hemorrhage, it did not penetrate the pericardium; but as it was in contact with the heart for a long period, it led to a delayed cardiac rupture.

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Introduction: Late-onset ornithine transcarbamylase (OTC) deficiency, a urea cycle disorder, is very rare. Here, we report a fatal case of OTC deficiency that developed at the age of 19 years.
Case: A 19-year-old man developed nausea, diarrhea, and general malaise the day before presenting to the emergency room of the nearest emergency hospital in a poor general condition. After admission, he developed seizures and disturbance of consciousness and was transferred to our hospital. On arrival, cranial CT showed brain edema, and his blood ammonia concentration was >500 µg/dL. After admission, it was difficult to control recurring seizures even with careful monitoring and increased doses of sedatives and anti-seizure drugs. Dialysis was not performed because his blood ammonia concentration gradually decreased. However, he exhibited dilated pupils on hospital day 2, a flat electroencephalogram and loss of brain stem reflexes on hospital day 4, and died on hospital day 11. OTC deficiency was diagnosed postmortem based on fractionation of blood and urine amino acids and orotic acid.
Discussion: Urea cycle disorders need to be treated urgently to prevent irreversible neurological damage when accompanied by hyperammonemia. In severe cases, dialysis should be considered to reduce ammonia level.

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A 57-year-old man was admitted to our hospital after presenting with sudden-onset upper abdominal pain and paraplegia. On arrival, the patient was lucid and his vital signs were as follows: blood pressure, 193/103 mmHg; body temperature, 34.0˚C; pulse, 76bpm, regular; respiration rate, 20 cycles/min, with 98% oxygen saturation (SpO₂) 98% (in room air). Physical examination revealed disorganized sensation below the eighth thoracic (Th8) level by dermatome, paraplegia, livedo reticularis of the abdominal wall, and cold lower extremities. Clinical examinations revealed an increase in pancreatic amylase level (3,38IU/L), and a magnetic resonance imaging (MRI) scan found a high intensity area at the Th7-8 level of the spinal cord. Therefore, the patient was hospitalized in the intensive care unit (ICU) with a diagnosis of acute pancreatitis and spinal cord infarction. Three hours after admission, there was an increase in the patient’s blood creatine phosphokinase (CPK) level, together with a time-dependent increase in levels of blood urea nitrogen (BUN), creatinine (Cr), and amylase (Amy). Twelve hours after admission, there was a progressive deterioration of the patient's respiratory and circulatory conditions, and thus, dopamine administration, mechanical ventilation, and hemodialysis treatments were initiated. Despite multiple intervention procedures, there was no improvement in the patient’s condition, and he died of multiple organ failure on the second day of admission. Patient autopsy revealed atherosclerosis of the aorta, and cholesterol crystal embolisms in arterioles of multiple organs. Therefore, a post-mortem diagnosis of multiple cholesterol crystal embolisms caused by a disintegration of atherosclerotic lesions was made. Tissue biopsy is necessary to make an early diagnosis of cholesterol crystal embolism. There is no proven prevention and treatment of this disease, which is a future issue.

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[in Japanese]

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