Giant cell hepatitis with autoimmune hemolytic anemia (AHA) is a rare disease of infancy characterized by the presence of both Coombs-positive hemolytic anemia and progressive liver disease with giant cell transformation of hepatocytes. Here, we report a case involving a seven-month-old male infant who presented with AHA followed by cholestatic hepatitis. The clinical features included jaundice, pallor, and red urine. Physical examination showed generalized icterus and splenomegaly. The laboratory findings suggested warm-type AHA with cholestatic hepatitis. Liver biopsy revealed giant cell transformation of hepatocytes and moderate lobular inflammation. The patient was successfully treated with four doses of rituximab. Early relapse of hemolytic anemia and hepatitis was observed, which prompted the use of an additional salvage dose of rituximab. He is currently in clinical remission.
Anemia, Hemolytic ; Anemia, Hemolytic, Autoimmune ; Biopsy ; Giant Cells ; Hepatitis ; Hepatocytes ; Humans ; Infant ; Inflammation ; Jaundice ; Liver ; Liver Diseases ; Male ; Pallor ; Physical Examination ; Rare Diseases ; Recurrence ; Rituximab ; Splenomegaly

PURPOSE: To report the results of ganglion cell analysis in a patient with optic tract syndrome who was previously diagnosed with glaucoma. CASE SUMMARY: A 32-year-old male, who had been diagnosed with glaucoma 12 years ago, but had not visited an ophthalmology clinic since then, came to our clinic for evaluation of his glaucoma. Both eyes showed an increased cup-to-disc ratio and temporal pallor of the disc. Retinal nerve fiber layer (RNFL) optical coherence tomography showed thinning of the superior, inferior, and temporal peripapillary RNFL in both eyes. On ganglion cell analysis (GCA), ganglion cell layer thinning in the nasal region of the right eye and in the temporal region of the left eye was observed. The visual field test showed right incongruous homonymous hemianopsia. After the atrophic change of the left optic tract was confirmed by orbit magnetic resonance imaging, he was diagnosed with left optic tract syndrome. CONCLUSIONS: We report the results of GCA in a case of optic tract syndrome, previously diagnosed as glaucoma. GCA can be useful when diagnosing optic tract syndrome.
Adult ; Ganglion Cysts ; Glaucoma ; Hemianopsia ; Humans ; Magnetic Resonance Imaging ; Male ; Nerve Fibers ; Ophthalmology ; Optic Nerve ; Optic Tract ; Orbit ; Pallor ; Retinaldehyde ; Temporal Lobe ; Tomography, Optical Coherence ; Visual Field Tests

Raynaud's phenomenon (RP) is a reversible vasospasm that is aggravated by cold or emotional stress. Before confirming RP, it is essential to consider other possible causes including compressive neuropathy, sensori-neuropathy, thyroid disease, hematologic conditions and offending drugs. RP is typically characterized by the three-step color change that turns pallor (white), cyanosis (blue), and then erythema (red) of reperfusion. Once RP is diagnosed, it is important to determine whether it is primary or secondary RP. To distinguish primary from the secondary RP, the specialized tests performing in clinical practice are antinuclear antibody (ANA) and nailfold capillary microscopy (NFC). The combination of ANA and NFC is most helpful for discriminating secondary RP due to autoimmune rheumatic disease. Thereby, normal findings of NFC in primary RP distinguished from secondary RP should be understood. Patients with primary RP usually improves with symptomatic treatment focused on lifestyle modification and patient education, but those with secondary RP should be treated together with associated disease or causes.
Antibodies, Antinuclear ; Capillaries ; Cyanosis ; Erythema ; Humans ; Life Style ; Microscopic Angioscopy ; Microscopy ; Pallor ; Patient Education as Topic ; Reperfusion ; Rheumatic Diseases ; Stress, Psychological ; Thyroid Diseases

PURPOSE: We report a case of bilateral nonarteritic anterior ischemic optic neuropathy (NAION) following acute angle-closure crisis (AACC). CASE SUMMARY: A 76-year-old female visited our clinic because of a 1-day history of ocular pain and vision loss in both eyes. The visual acuity was 0.02 in both eyes and her intraocular pressure (IOP) was 52 mmHg in the right eye (RE) and 50 mmHg in the left eye (LE). She had corneal edema and a shallow anterior chamber in both eyes, with 4 mm fixed dilated pupils. After decreasing the IOP with intravenous mannitol, laser iridotomy was performed. However, 2 days later, visual acuity was further reduced to finger counting at 10 cm RE and at 50 cm LE, and her optic disc was swollen. Bilateral NAION following AACC was diagnosed. One month later, visual acuity slightly improved to 0.02 RE and 0.04 LE, and the optic disc edema resolved. A small cup-disc ratio, optic disc pallor, and atrophy were observed. Humphrey visual fields demonstrated superior and inferior altitudial visual field defects in the LE, and almost total scotoma in the RE. CONCLUSIONS: AACC can be a predisposing factor for NAION, so the relative afferent pupillary defect, papilledema, and presentation of other risk factors are important clues to a diagnosis of NAION.
Aged ; Anterior Chamber ; Atrophy ; Causality ; Corneal Edema ; Diagnosis ; Edema ; Female ; Fingers ; Glaucoma, Angle-Closure ; Humans ; Intraocular Pressure ; Mannitol ; Optic Neuropathy, Ischemic ; Pallor ; Papilledema ; Pupil ; Pupil Disorders ; Risk Factors ; Scotoma ; Visual Acuity ; Visual Fields

A 2-year-old intact female Bengal cat was presented with a 6-month history of visual impairment. The cat manifested bilateral negative menace responses and dazzle reflexes and sluggish pupillary light reflexes. Bilateral fundus changes included generalized tapetal hyperreflectivity, advanced retinal vascular attenuation, and increased pallor of the optic disc. A diagnosis of bilateral retinal degeneration was made. The clinical findings suggest that the investigated Bengal cat was most likely to have an inherited retinal degeneration. Further studies of the Bengal cat breed are needed to determine the prevalence of inherited retinal degeneration in this breed in Korea.
Animals ; Cats ; Child, Preschool ; Diagnosis ; Female ; Humans ; Korea ; Pallor ; Prevalence ; Reflex ; Retinal Degeneration ; Retinaldehyde ; Vision Disorders

Azathioprine is an immunosuppressive drug that has been widely used in dermatology for the treatment of immunobullous diseases. Myelosuppression is the most important side effect and requires close observation of the complete blood cell count. The clinical findings of myelosuppression include general weakness, poor oral intake, nausea, dyspnea, and pallor. It can occur within several weeks to years after initial azathioprine treatment; thus, a weekly full blood count for the first 4 weeks, followed by reduced frequency of monitoring to a minimum of once every 3 months is recommended. If the myelosuppression is not treated properly, it can lead to fever, secondary infection, sepsis, and even death. Herein, we present three educational cases for dermatologists to order to underline the risk of myelosuppression during azathioprine treatment.
Azathioprine* ; Blood Cell Count ; Coinfection ; Dermatology ; Dyspnea ; Fever ; Nausea ; Pallor ; Sepsis

The relationship between the vestibular system and the autonomic nervous system has been well studied in the context of the maintenance of homeostasis to the changing internal and external milieus. The perturbations of the autonomic indexes to the vestibular stimuli have been demonstrated in animal studies. In addition, the fluctuation of the blood pressure and the heart rate with other autonomic symptoms such as nausea, vomiting, and pallor are common manifestations in the wide range of vestibular disorders. At the same time, the disorders of the autonomic nervous system can cause dizziness and vertigo in some group of patients. In the anatomical point of view, the relationship between autonomic and vestibular systems is evident. The afferent signals from each system converge to the nucleus of solitary tract to be integrated in medullary reticular formation and the each pathway from the vestibular and autonomic nervous system is interconnected from medulla to cerebral cortex. In this paper, the reported evidence demonstrating the relationship between autonomic derangement and vestibular disorders is reviewed and the further clinical implications are discussed.
Animals ; Autonomic Nervous System ; Autonomic Nervous System Diseases ; Blood Pressure ; Cerebral Cortex ; Dizziness ; Heart Rate ; Homeostasis ; Humans ; Nausea ; Pallor ; Reticular Formation ; Solitary Nucleus ; Vertigo ; Vomiting

Pulmonary artery aneurysms (PAAs) are rare, and massive hemoptysis can lead to death if appropriate diagnosis and treatment is not provided. PAAs can be of congenital, acquired, or idiopathic origin, and the clinical symptoms are various. Among all reported cases, one-third of the patients died due to rupture. Optimal treatment or guidelines for PAAs remain uncertain. Herein, we report autopsy findings from a woman with PAA. The patient was taking medication for tuberculosis. On bronchoscopy, a polypoid lesion was found, suspected to be an inflammatory polyp. Biopsy was performed and massive bleeding into the airway occurred. The bleeding could not be controlled by bronchoscopic suction, and cardiac arrest occurred 30 minutes after biopsy; the patient subsequently died. Autopsy revealed a round, calcified PAA in the bronchus of the right middle lobe; the end of the PAA was torn. Hypovolemic signs, including weak postmortem lividity and pallor of the skin and conjunctivae, were observed. Visual inspection and histopathological examination of the right lung revealed tuberculosis and congestion. Cases related PAA are not uncommon, but autopsy cases of death occurring after biopsy of PAA mimicking bronchial polyps are rarely reported.
Aneurysm* ; Autopsy ; Biopsy* ; Bronchi ; Bronchoscopy ; Conjunctiva ; Diagnosis ; Estrogens, Conjugated (USP) ; Female ; Heart Arrest ; Hemoptysis ; Hemorrhage ; Humans ; Hypovolemia ; Lung ; Pallor ; Polyps* ; Pulmonary Artery* ; Rupture ; Skin ; Suction ; Tuberculosis

Bee sting causes mild symptoms such as urticaria and localized pain, and severe symptoms including anaphylaxis, cardiovascular collapse, and death. We reported on a patient with arterial thrombotic occlusion and severe ischemia in the lower limb after multiple bee stings. The patient was stung 5 times and complained of pallor, pain, and coldness in the left toe, and did not have dorsalis pedis pulsation. Computed tomography angiography showed multiple thrombotic occlusion of the anterior and posterial tibial artery below the knee. Local thrombolytic therapy using urokinase was administered and the occluded arteries were successfully recanalized.
Anaphylaxis ; Angiography ; Angioplasty, Balloon ; Arteries ; Bee Venoms ; Bees* ; Bites and Stings* ; Humans ; Ischemia* ; Knee ; Lower Extremity* ; Pallor ; Thrombolytic Therapy ; Tibial Arteries ; Toes ; Urokinase-Type Plasminogen Activator ; Urticaria

PURPOSE: We report a case of meningioma diagnosed as ischemic optic neuropathy. CASE SUMMARY: A 45-year-old women presented with decreased visual acuity in her left eye without eye movement pain. Her best corrected visual acuity (BCVA) in that eye was 0.3. She showed a relative afferent pupillary defect, abnormal color vision test, and inferior visual field defect in her left eye. The optic disc showed slight blurring superiorly and pallor temporally. Fluorescein angiography showed choroidal filling defect, and ischemic optic neuropathy was suspected. The carotid artery sonography showed normal results. The BCVA of the left eye was 0.5 after 7 months. The visual field test and color vision test were improved after 7 months. The optic disc was pale. The magnetic resonance imaging was performed because of persistent headache, and that showed a suprasellar mass that was removed by surgical resection and diagnosed as meningioma on biopsy. CONCLUSIONS: The possibility of compressive optic neuropathy should be considered in presumed ischemic optic neuropathy if the patient complains of persistent headache.
Biopsy ; Carotid Arteries ; Choroid ; Color Vision ; Eye Movements ; Female ; Fluorescein Angiography ; Headache ; Humans ; Magnetic Resonance Imaging ; Meningioma* ; Middle Aged ; Optic Nerve Diseases ; Optic Neuropathy, Ischemic* ; Pallor ; Pupil Disorders ; Visual Acuity ; Visual Field Tests ; Visual Fields