Masqueraders Around Disaster: Clinical Features of Scrub Typhus in Fukushima, Japan.

Background: Scrub typhus (ST) is endemic in Fukushima, with the largest number of cases reported in Japan from 2009 to 2010. Although ST is highly treatable, its atypical clinical presentation impedes diagnosis, causing delays in treatment. Methods: We review the clinical features of ST in adults from 2008 to 2017 at Ohta Nishinouchi General Hospital in Fukushima, Japan. Results: Fifty-five cases (serotype Karp 24, Irie/Kawasaki 21, Hirano/Kuroki 10) of ST were confirmed via serology based on elevated immunoglobulin (Ig)M and IgG and polymerase chain reaction positivity of eschar samples. The mean age was 69 years, and 64% were female. The case fatality rate was 1.8% (1/55). Approximately 70% of cases (38/55) were not diagnosed as ST upon the initial clinic visit. Inappropriate use of antibiotics was identified in 22% of cases (12/55). In terms of atypical clinical features, 1 or more of the manifestations, fever, rash, and eschar, was absent in 31% of cases (17/55). Approximately 11% of cases presented without eschar (6/55; Karp 1, Irie/Kawasaki 1, Hirano/Kuroki 4). Moreover, severe complications were observed with shock and disseminated intravascular coagulation in 7% of cases (4/55), Thus, while 53% of cases presented with the typical triad (29/55), unusual complications and atypical features occurred in 40% (22/55). Conclusions: Diagnosis of ST becomes clinically challenging in the absence of typical features. In Fukushima, an endemic area of ST, an atypical presentation involving multisystem disease is common.

Where is reactivation after a long latency?

The authors present a 90-year-old woman with unilateral glossopharyngeal, vagal and spinal accessory cranial nerve palsy along with pharyngeal and laryngeal vesicular eruptions. She was diagnosed with herpes zoster based on PCR testing on vesicular fluid for varicella-zoster virus (VZV). Reactivation of VZV in the head and neck region can cause life-threatening neurologic sequelae. Clinicians should be alert to the possibility of herpes zoster in a case of unilateral multiple cranial neuropathies and rapid combination therapy with acyclovir and corticosteroid should be initiated.

Scrub typhus-induced serious gastric ulcer bleeding.

A 67-year-old woman presented with melena and general weakness. Upper gastrointestinal (GI) endoscopy revealed multiple ulcers and projectile bleeding in the stomach. She also complained of a 10-day history of a fever and was diagnosed with scrub typhus based on a positive result of the eschar polymerase chain reaction (PCR) testing. She fully recovered with endoscopic hemostasis, and administration of minocycline and omeprazole. In a patient with GI manifestations, scrub typhus, a condition with pathologically systemic vasculitis, should be considered in the possible background in endemic areas. The eschar PCR testing is a rapid and useful diagnostic tool to identify a specific strain.

Anisakis simplex-induced anaphylaxis.

In recent years, Anisakis simplex has been shown to be an important etiologic agent responsible for food allergy and for gastrointestinal anisakiasis. We report a 61-year-old woman presenting with generalized urticaria and subsequent anaphylaxis after ingestion of raw mackerel. She rapidly recovered with administration of epinephrine and endoscopic extraction of an A. simplex larva. Serologic testing revealed specific IgE antibody to A. simplex was positive whereas that to mackerel was negative. She was diagnosed as IgE-mediated hypersensitivity to A. simplex. Patients diagnosed as fish-related or idiopathic allergy should be examined for evidence of Anisakis-induced allergy.

'Goose bumps'-associated anaemia.

A 36-year-old woman presented to our hospital for further evaluation of anaemia screened by regular check-up. She was diagnosed with iron-deficiency anaemia (IDA) but refractory to iron supplementation. She had negative results of occult blood in the stool and no gynaecologic disease potentially causing hyper menorrhea. Upper endoscopy revealed a nodular gastritis, which has been called 'Torihada-ien', a Japanese word meaning 'gastritis with goose bumps-like appearance', associated with Helicobacter pylori (Hp) infection. After Hp eradication therapy, her anaemia resolved with an improved response to iron supplementation. In an unexplained IDA patient, Hp-associated gastritis should be considered in the possible background.

Organising pneumonia as the first manifestation of rheumatoid arthritis.

Organising pneumonia (OP) is an inflammatory lung disease with distinctive clinicopathological features. OP can be evident during the course of rheumatoid arthritis (RA) with increased disease activity. The authors report an OP associated with RA case in which pulmonary symptoms preceded the onset of joint symptoms. An OP patient with elevated serum anticyclic citrullinated peptide antibody is likely to manifest RA in the near future, reflecting its high disease activity. Thus, an early rheumatologic consultation should be taken into consideration to make an early decision to initiate disease-modifying antirheumatic drugs therapy.

Painful hypoadrenalism.

A 61-year-old man presented with diffuse myalgia and arthralgia. The physical examination showed diffuse musculoskeletal tenderness and joint stiffness without the presence of synovitis or arthritis. Nerve conduction study showed combined mild axonal degenerative and demyelinating change. Muscle biopsy analysis showed unspecific mild muscle atrophy with myopathic changes found on electromyography. A diagnosis of isolated adrenocorticotropic hormone deficiency was made on the basis of endocrinological evaluation. The widespread musculoskeletal pain resolved as a result of administration of physiological dose of hydrocortisone replacement. In a patient with unexplained rheumatic symptoms, adrenocortical insufficiency should be considered in the possible aetiology.

Another 'Cushing ulcer'.

The authors describe the case of a 39-year-old man who presented to our hospital with easy fatigability and malaise. On physical examination, hypertension was noted without any cushingoid appearance. Laboratory testing revealed normochromic-normocytic anaemia with positive results of occult blood in the stool, hyperglycaemia and hypokalemia. Upper endoscopy revealed active gastric ulcer with Helicobacter pylori infection, likely causing gastrointestinal bleeding. Endocrine examinations showed that both serum adrenocorticotropic hormone and cortisol were elevated with loss of diurnal variation. A diagnosis of Cushing's disease secondary to pituitary adenoma was made as results of brain MRI and blood sampling from inferior petrosal sinus. In a patient with peptic ulcer disease, physician should be alert to the possible endocrine background.

Carbamazepine-induced agammagloblinaemia clinically mimicking diffuse panbronchiolitis.

The authors report the case of a 61-year-old man who presented with carbamazepine (CBZ)-related agammagloblinaemia accompanied by sinobronchial syndrome clinically mimicking diffuse panbronchiolitis (DPB). The patient's laboratory and radiographic findings improved significantly after cessation of CBZ, and administration of gammaglobulin and long-term oral macrolides therapy. In patients on long-term CBZ treatment, the physician should be aware of the possible development of secondary immunodeficiency, which can cause small airway diseases sharing common clinical features with DPB.

Craniovertebral junction tuberculosis: a case report and review of the literature.

Craniovertebral junction tuberculosis (CVJ TB) is a rare disease, potentially causing significant neurological deficits and even death. We report on a 80-year-old woman presenting with CVJ TB without pulmonary involvement. The diagnosis was made by biopsy of the cervical lymph node showing granulomatous caseation necrosis. Despite extensive erosion of the clivus, C1, and C2, and spinal cord compression, the patient was effectively managed with antituberculous drug therapy and conservative neck stabilization. Neck pain resulting from cervical spondylosis is common in elderly people. However, even if there is no obvious pulmonary involvement, CVJ TB should be considered in the differential diagnosis, especially in patients with painful neck stiffness. The most useful method available for evaluating this region is a combination of CT scan and MRI study. CVJ TB can be managed conservatively, except for a selected few cases, regardless of the extent of bony destruction.

Effects of benidipine, a long-acting T-type calcium channel blocker, on home blood pressure and renal function in patients with essential hypertension: a retrospective, 'real-world' comparison with amlodipine.

BACKGROUND AND OBJECTIVE: Calcium channel antagonists (calcium channel blockers [CCBs]) are often used in the treatment of patients with hypertension to achieve strict blood pressure (BP) targets. In the present study, we compared the antihypertensive effects (determined by home BP [HBP] measurements) and the effects on renal function of benidipine (hydrochloride) and amlodipine (mesylate), a commonly used CCB. METHODS: Changes in HBP and urinary albumin excretion (UAE) were investigated in 47 benidipine and 37 amlodipine recipients with essential hypertension and albuminuria between January 2007 and December 2007. Both benidipine and amlodipine significantly reduced morning and evening HBP over a 12-month period. RESULTS: Both medications also significantly reduced UAE compared with pretreatment values; however, the reduction in UAE observed in the benidipine group occurred independent of the drug's antihypertensive effects, whereas a positive correlation was shown between the reduction in morning systolic BP and UAE in the amlodipine group. CONCLUSIONS: These results demonstrate that benidipine favourably affects renal function in patients with essential hypertension compared with amlodipine, suggesting that the clinical benefits of benidipine as an antihypertensive drug include a renoprotective effect.

Adult onset Schönlein-Henoch purpura associated with Helicobacter pylori infection.

Schönlein-Henoch purpura (SHP) is a systemic vasculitis, primarily involving the skin, gastrointestinal (GI) tract, joints, and kidneys. A wide variety of different conditions may be implicated in the pathogenesis of SHP. We report a 33-year-old man who presented with SHP accompanied by gastric Helicobacter pylori (Hp) infection. The GI manifestations and purpuric rashes were dramatically resolved after Hp eradication therapy. To date, very few publications have focused on the possible pathogenetic relationship between Hp infection and SHP.

Sporadic adult-onset hypophosphatemic osteomalacia caused by excessive action of fibroblast growth factor 23.

A 50-year-old man without family history of metabolic bone disease was referred to our hospital with a 5-year history of progressively worsening spinal and bilateral diffuse leg pain and proximal muscle weakness. Two years before admission, he was diagnosed as ankylosing spondylitis by a rheumatologist and was maintained on low-dose prednisone therapy without benefit. He developed progressive spinal and thoracic deformities, resulting in a 10 cm loss in height in the preceding 2 years. On physical examination, marked thoracic kyphosis and pectus carinatum was noted. Plain radiograph revealed pseudofracture in the right femoral neck. Laboratory findings showed a normal level of serum calcium, elevated level of serum alkaline phosphatase and inappropriately increased urinary phosphate excretion despite extreme hypophosphatemia. He was diagnosed as adult-onset hypophosphatemic osteomalacia caused by renal phosphate wasting. Serum fibroblast growth factor 23 was the upper limit of normal despite extreme hypophosphatemia and no neoplastic lesion potentially inducing hypophosphatemic osteomalacia could be identified in a thorough search including imaging studies of his entire body. Oral administration of phosphate and activated vitamin D together with dipyridamole relieved the persistent pain and weakness, and he became fully ambulatory.

Community-acquired Staphylococcus aureus pneumonia accompanied by rapidly progressive glomerulonephritis and hemophagocytic syndrome.

A 59-year-old woman without underlying disease was admitted to a local hospital because of lung abscess, cytopenias and renal failure. 3 days before admission, she was diagnosed as influenza infection and was under antiviral therapy. Blood cultures were positive for methicillin-sensitive Staphylococcus aureus (MSSA). She was transferred to our hospital on the 15th day at the local hospital because the clinical manifestations could not improve even though she was treated with multiple intravenous antibiotics directed against MSSA. Sputum cultures yielded methicillin-resistant S. aureus (MRSA) producing toxic shock syndrome toxin-1 (TSST-1) and serologic test indicated hypercytokinemia. She was diagnosed as rapidly progressive glomerulonephritis and hemophagocytic syndrome associated with staphylococcal infection. The pulmonary lesions, cytopenias and renal dysfunction improved as a result of long-term antimicrobial treatment including vancomycin, hemodialysis, short-term administration of corticosteroid and other supportive cares. She was finally weaned from hemodialysis on the 73rd hospital day. In recent years, the number of cases of S. aureus producing TSST-1 and enterotoxin has been increasing and in cases of staphylococcal infections, close attention should be given to toxin-mediated as well as non-toxin-mediated clinical manifestations.

Septic cavernous sinus thrombosis complicated by narrowing of the internal carotid artery, subarachnoid abscess and multiple pulmonary septic emboli.

A 56-year-old woman was admitted because of a high fever, right ptosis, chemosis, proptosis and ocular muscle palsy. Cranial MRI revealed a cavernous sinus thrombosis and a subarachnoid abscess. Carotid angio-gram demonstrated marked stenosis as well as aneurismal formation of the right internal carotid artery at the intracavernous portion. Chest radiograph showed bilateral multiple pulmonary nodules, some of which contained a cavity. Blood culture was positive for Streptococcus constellatus. She was diagnosed with septic cavernous sinus thrombosis complicated by narrowing of the internal carotid artery, subarachnoid abscess and multiple pulmonary septic emboli. She recovered with partial ocular sequelae as a result of seven weeks of intravenous antimicrobial therapy.