Potential Indicators of Intestinal Necrosis in Portal Venous Gas: A Case Report of an 82-Year-Old Woman on Long-Term Hemodialysis with Ascites and Pneumatosis Coli.

BACKGROUND Several factors have been reported as possible predictors of intestinal necrosis in patients with portal venous gas (PVG). We describe potential indicators of intestinal necrosis in PVG identified by contrasting 3 episodes of PVG in a patient on hemodialysis against previously verified factors. CASE REPORT An 82-year-old woman undergoing hemodialysis was admitted to our hospital thrice for acute abdominal pain. On first admission, she was alert, with a body temperature of 36.3°C, blood pressure (BP) of 125/53 mmHg, pulse rate of 60/min, respiratory rate of 18/min, and 100% oxygen saturation on room air. Computed tomography (CT) revealed PVG, intestinal distension, poor bowel wall enhancement, bubble-like pneumatosis in the intestinal wall, and minimal ascites. PVG caused by intestinal ischemia was diagnosed, and she recovered after bowel rest and hydration. Three months later, she had a second episode of abdominal pain. BP was 115/56 mmHg. CT revealed PVG and a slight accumulation of ascites, without pneumatosis in the intestinal wall. She again recovered after conservative measures. Ten months later, the patient experienced a third episode of abdominal pain, with BP of 107/52 mmHg. CT imaging indicated PVG, considerable ascites, and linear pneumatosis of the intestinal walls. Despite receiving conservative treatment, the patient died. CONCLUSIONS A large accumulation of ascites and linear pneumatosis in the intestinal walls could be potential indicators of intestinal necrosis in patients with PVG caused by intestinal ischemia. As previously reported, hypotension was further confirmed to be a reliable predictor of intestinal necrosis.

Validation and Improvement of the Saga Fall Risk Model: A Multicenter Retrospective Observational Study.

Purpose: We conducted a pilot study in an acute care hospital and developed the Saga Fall Risk Model 2 (SFRM2), a fall prediction model comprising eight items: Bedriddenness rank, age, sex, emergency admission, admission to the neurosurgery department, history of falls, independence of eating, and use of hypnotics. The external validation results from the two hospitals showed that the area under the curve (AUC) of SFRM2 may be lower in other facilities. This study aimed to validate the accuracy of SFRM2 using data from eight hospitals, including chronic care hospitals, and adjust the coefficients to improve the accuracy of SFRM2 and validate it. Patients and Methods: This study included all patients aged ≥20 years admitted to eight hospitals, including chronic care, acute care, and tertiary hospitals, from April 1, 2018, to March 31, 2021. In-hospital falls were used as the outcome, and the AUC and shrinkage coefficient of SFRM2 were calculated. Additionally, SFRM2.1, which was modified from the coefficients of SFRM2 using logistic regression with the eight items comprising SFRM2, was developed using two-thirds of the data randomly selected from the entire population, and its accuracy was validated using the remaining one-third portion of the data. Results: Of the 124,521 inpatients analyzed, 2,986 (2.4%) experienced falls during hospitalization. The median age of all inpatients was 71 years, and 53.2% were men. The AUC of SFRM2 was 0.687 (95% confidence interval [CI]:0.678-0.697), and the shrinkage coefficient was 0.996. SFRM2.1 was created using 81,790 patients, and its accuracy was validated using the remaining 42,731 patients. The AUC of SFRM2.1 was 0.745 (95% CI: 0.731-0.758). Conclusion: SFRM2 showed good accuracy in predicting falls even on validating in diverse populations with significantly different backgrounds. Furthermore, the accuracy can be improved by adjusting the coefficients while keeping the model's parameters fixed.

Heidenhain Variant of Sporadic Creutzfeldt-Jakob Disease with a Variety of Visual Symptoms: A Case Report with Autopsy Study.

BACKGROUND Sporadic Creutzfeldt-Jakob disease (sCJD) is a fatal disease caused by the change of prion protein (PrP). Affected patients present with rapidly progressive cognitive dysfunction, myoclonus, or akinetic mutism. Diagnosing the Heidenhain variant of sCJD, which initially causes various visual symptoms, can be particularly difficult. CASE REPORT A 72-year-old woman presented with a 2- to 3-month history of photophobia, blurring vision in both eyes. Seven days previously, she showed visual impairment of 20/2000 in both eyes. Left homonymous hemianopia and restricted downward movement of the left eye were observed with an intact pupillary light reflex and normal fundoscopy. On admission, her visual acuity was light perception. Cranial magnetic resonance imaging revealed no abnormality, and electroencephalography showed no periodic synchronous discharges. Cerebrospinal fluid examination on the sixth hospital day revealed tau and 14-3-3 protein with a positive result of real-time quaking-induced conversion. She thereafter developed myoclonus and akinetic mutism and died. Autopsy revealed thinning and spongiform change of the cerebral cortex of the right occipital lobe. Immunostaining showed synaptic-type deposits of abnormal PrP and hypertrophic astrocytes. Consequently, she was diagnosed with the Heidenhain variant of sCJD with both methionine/methionine type 1 and type 2 cortical form based on the western blot of cerebral tissue and PrP gene codon 129 polymorphism. CONCLUSIONS When a patient presents with various progressive visual symptoms, even without typical findings of electroencephalography or cranial magnetic resonance imaging, it is essential to suspect the Heidenhain variant of sCJD and perform appropriate cerebrospinal fluid tests.

Late-Onset Ornithine Transcarbamylase Deficiency Complicated with Extremely High Serum Ammonia Level: Prompt Induction of Hemodialysis as the Key to Successful Treatment.

BACKGROUND Ornithine transcarbamylase deficiency (OTCD) is an X-linked semi-dominant disorder, causing possible fatal hyperammonemia. Late-onset OTCD can develop at any time from 2 months after birth to adulthood, accounting for 70% of all OTCDs. CASE REPORT A 35-year-old man with chronic headaches stated that since childhood he felt sick after eating meat. Fourteen days before hospital admission, he began receiving 60 mg/day of intravenous prednisolone for sudden deafness. The prednisolone was stopped 5 days before hospital admission. Four days later, he was transferred to our hospital because of confusion. On admission, he had hyperammonemia of 393 µmol/L. Because he became comatose 7 hours after admission, and his serum ammonia increased to 1071 µmol/L, we promptly started hemodialysis. Because his family history included 2 deceased infant boys, we suspected late-onset OTCD. On day 2 of hospitalization, we began administering ammonia-scavenging medications. Because he gradually regained consciousness, we stopped his hemodialysis on day 6. After his general condition improved, he was transferred to the previous hospital for rehabilitation on day 32. We definitively diagnosed him with late-onset OTCD due to the low plasma citrulline and high urinary orotic acid levels found during his hospitalization. CONCLUSIONS Clinicians should suspect urea cycle disorders, such as OTCD, when adult patients present with marked hyperammonemia without liver cirrhosis. Adult patients with marked hyperammonemia should immediately undergo hemodialysis to remove ammonia, regardless of causative diseases.

A Prospective Observational Study Conducted at a Single Center in Japan to Validate a Previously Developed Predictive Formula of In-Hospital Mortality for Patients Aged ≥65 Years with Endogenous Diseases Transported by Ambulance.

BACKGROUND In 2019, we developed a predictive formula of in-hospital mortality for inpatients aged ≥65 years transported by ambulance for endogenous diseases. In this study, we aimed to validate this previously developed predictive formula. MATERIAL AND METHODS In this single-center prospective observational study, we enrolled all patients aged ≥65 years who were transported by ambulance and admitted to an acute care hospital in Japan for endogenous diseases. We calculated the score according to our developed formula using age, disturbance of consciousness, the shock index, and amount of oxygen administered, with each item scoring 1 point and then totaling them. We subsequently evaluated the in-hospital mortality rate, stratum-specific likelihood ratio, and area under the receiver operating characteristic curve (AUC) of the formula, using in-hospital mortality as the primary outcome. RESULTS In total, 325 patients were included in this study. Forty-two patients died during hospitalization. Multivariable logistic regression analysis (forced-entry method) revealed that disturbance of consciousness and oxygen administration 5 L/min or more were significantly associated with mortality during hospitalization. In contrast, aged ≥90 years and shock index of 1 or higher were not significant. The mortality and stratum-specific likelihood ratios for scores in the predictive formula of 3 and 4 were 40.9% and 4.66, and 66.7% and 13.48, respectively. The AUC of the predictive formula for in-hospital mortality was 0.670 (95% confidence interval: 0.574-0.767). CONCLUSIONS This study showed that our predictive formula, consisting of factors available immediately after ambulance transport in older patients, is feasible with sufficient discrimination ability and reliability.

Unilateral syphilitic optic neuritis without meningitis or uveitis.

A 69-year-old man with left eye pain and visual impairment was diagnosed with syphilitic optic neuritis, who was successfully treated by penicillin. Although it is difficult to decide syphilis as the direct cause of optic neuritis, it is essential to diagnose syphilis in every patient with optic neuritis.

Relationships between sites of abdominal pain and the organs involved: a prospective observational study.

OBJECTIVES: Abdominal pain is one of the most frequent chief complaints in primary care settings. The aim of the present study was to determine the positive likelihood ratios (PLRs) and negative likelihood ratios (NLRs) of the relationships between the sites of abdominal pain and the organs involved. DESIGN: Prospective observational study. SETTING: A single tertiary centre, a university hospital in Japan. PARTICIPANTS: A total of 2591 new outpatients visited the Department of General Medicine at a university hospital from April 2017 to March 2018. Of these, 326 patients aged ≥20 years with abdominal pain were enrolled. RESULTS: Sites of abdominal pain were classified into 11 categories including nine different abdominal sections, 'generalised abdomen' and 'site-indeterminate'. The PLRs between 'right subcostal' and 'liver and biliary tract'; between 'right subcostal' and 'musculoskeletal' ; between 'epigastric' and 'oesophagus, stomach and duodenum'; between 'right or left flank' and 'urinary tract'; between 'left flank' and 'dermatological'; and between 'mid-lower' and 'intestinal' ranged from 2.17 to 4.14. The PLRs between 'epigastric' and 'urinary tract'; between 'mid-lower' and 'liver and biliary tract'; between 'periumbilical' and 'urinary tract'; and between 'generalised abdomen' and 'oesophagus, stomach and duodenum' were low, ranging from 0.17 to 0.25. The NLR ranged from 0.5 to 1.5, excluding the relationship between 'left flank' and 'dermatological'. CONCLUSION: The presence of pain at right subcostal, epigastric, right or left flank and mid-lower sites might be useful for identifying the organs involved. Additionally, the presence of pain at mid-lower, epigastric, periumbilical and generalised abdominal sites might be helpful for denying the involvement of some organs. Some sites of abdominal pain can be indicative of the organs involved.Trial registration numberUMIN000037686.

Pegfilgrastim-associated large-vessel vasculitis developed during adjuvant chemotherapy for breast cancer: A case report and review of the literature.

INTRODUCTION: Granulocyte colony-stimulating factor (G-CSF) is widely used as a neutrophil supportive therapy in breast cancer chemotherapy. Common adverse events of G-CSF include bone pain, headache, and fatigue; however, reports of G-CSF-associated vasculitis are few. CASE REPORT: A 66-year-old woman who had undergone surgery for breast cancer received adjuvant chemotherapy with prophylactic use of pegfilgrastim (peg-G). She developed peg-G-associated vasculitis 11 days after initially receiving peg-G.Management and outcome: Although various blood and culture tests were required to rule out other vasculitis syndromes and infections, her symptoms spontaneously disappeared without any treatment other than discontinuation of the causal drug. DISCUSSION: G-CSF-associated vasculitis is occasionally accompanied by severe complications such as aortic dissection and aneurysm formation. This case report is important to draw attention towards this rare and difficult-to-diagnosis adverse event of peg-G.

Proliferative Fasciitis/Myositis Involving the Facial Muscles Including the Masseter Muscle: A Rare Cause of Trismus.

BACKGROUND Proliferative fasciitis/myositis is a benign disease that can be treated conservatively. However, some patients are mistakenly treated surgically because of a misdiagnosis of the condition as a malignant tumor. CASE REPORT A 50-year-old Japanese man developed swelling in his left cheek 12 days before admission; he developed a fever and trismus 3 days later. He was admitted to our hospital because of worsening of his condition despite treatment with sitafloxacin for 5 days and needle-aspiration drainage. On admission, he had a fever of 38.1°C, swelling in his left cheek spreading to the lower jaw, and several dental caries. Although ceftriaxone and clindamycin were administered for 7 days because an odontogenic infection was suspected, his condition did not improve. T2-weighted magnetic resonance imaging of the facial muscles on Day 5 of hospitalization showed swelling and high-intensity signals in the left masseter, temporalis, and pterygoid muscles. Macroscopic findings from a biopsy of the left temporalis muscle performed on Day 17 showed white and thickened fascia. Histopathological examination revealed fibrous hyperplasia of the fascia, increased fibrous connective tissue between muscle fibers, and infiltration of inflammatory cells, providing not a definite but a compatible diagnosis of proliferative fasciitis/myositis. Beginning on Day 18, the patient's fever lessened with gradual improvement of his facial swelling and trismus. CONCLUSIONS It is imperative to include proliferative fasciitis/myositis as a possible diagnosis when patients present with facial swelling and trismus of unknown cause.