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Biomedical subjects

A Tanikawa

Publications and source records attributed to A Tanikawa.

At least 19 recordsLinked to original sources
PubMed2006

A case of drug-induced hypersensitivity syndrome showing transient immunosuppression before viral reactivation during treatment for pemphigus foliaceus.

Drug-induced hypersensitivity syndrome (DIHS) is one of the most severe drug adverse reactions, with characteristic biphasic symptoms. Reactivation of human herpesvirus-6 (HHV-6) is frequently observed, although the cause of DIHS is still unknown. A patient developed DIHS during treatment with diaminodiphenylsulphone for pemphigus foliaceus. The number of lymphocytes in his peripheral blood, and titres of serum total IgG and IgM and anti-desmoglein1 antibody transiently decreased just before reactivation of HHV-6, cytomegalovirus and Epstein-Barr virus. This observation suggests that transient suppression of both cellular and humoral immunity may trigger viral reactivation, which leads to the development of the second phase of DIHS.

Administration, Oral↗
PubMed2006

Aleukaemic leukaemia cutis as an initial manifestation of myeloid/NK cell precursor acute leukaemia.

Aleukaemic leukaemia cutis is a rare condition characterized by infiltration of leukaemic cells into the skin before they appear in the peripheral blood. We report a case of an aleukaemic leukaemia cutis, which had a history of exposure to atomic bomb radiation. A 57-year-old Japanese woman initially presented with a 20-week history of multiple red papules and plaques mainly over the trunk. Histological examination revealed the infiltration of atypical monocytic cells in the dermis, but no leukaemic cells were detected in the peripheral blood. Twenty-three weeks after the appearance of the eruption, leukaemic cells were detected in the peripheral blood for the first time. The results of immunohistochemistry of the skin biopsy specimen and flow cytometry of the peripheral blood indicated the rare phenotype of myeloid/NK cell precursor acute leukaemia. This is the first case report of myeloid/NK cell precursor acute leukaemia presenting as aleukaemic leukaemia cutis in the English literature, and awareness of this clinical presentation may be important to reach the correct diagnosis.

Fatal Outcome↗
PubMed2005

Decline of anti-desmoglein 1 IgG ELISA scores by withdrawal of D-penicillamine in drug-induced pemphigus foliaceus.

A case of pemphigus foliaceus arising during the administration of D-penicillamine was reported. A 72-year-old woman with systemic sclerosis developed flaccid vesicles and crusted erythema of the skin after administration of D-penicillamine for 1-year. She was positive for anti-desmoglein 1 IgG by ELISA. The withdrawal of D-penicillamine resulted in improvement of the skin lesions and ELISA scores for anti-demoglein 1 antibodies revealed a surprisingly rapid decline. This case is a classical incidence of drug-induced pemphigus foliaceus, in which D-penicillamine was constantly required not only to induce, but also to maintain the production of the autoantibodies.

Aged↗
PubMed2004

Parallel fluctuation of anti-desmoglein 3 and anti-BP180 autoantibody titres in a patient with bullous pemphigoid.

We report a 73-year-old Japanese female who developed IgG autoantibodies against BP180 as well as desmoglein 3 (Dsg3). She showed tense blisters on the extremities without apparent mucosal involvement and a skin biopsy indicated subepidermal blisters with eosinophilic spongiosis. Her clinical and histologic features indicated the diagnosis of bullous pemphigoid while anti-Dsg3 IgG might not show an apparent pathogenic effect. Interestingly, titres of anti-Dsg3 IgG fluctuated in parallel with those of anti-BP180 IgG throughout the course with two flares. Although the exact mechanism for autoantibody production is still unknown, the close link in the production of IgG autoantibodies against two independent skin antigens suggests a shared immunoregulatory mechanism against cutaneous autoantigens.

Aged↗
PubMed2002

Monitoring disease activity in pemphigus with enzyme-linked immunosorbent assay using recombinant desmogleins 1 and 3.

BACKGROUND: Pemphigus is an antidesmoglein (Dsg) autoimmune disease that is divided into two major subtypes: pemphigus foliaceus (PF) and pemphigus vulgaris (PV). We previously developed enzyme-linked immunosorbent assays (ELISAs) using recombinant Dsg1 and Dsg3 to detect IgG autoantibodies in patients with pemphigus. The protocol for the ELISAs was optimized for serological diagnosis, but under the conditions used, these assays were not particularly useful for monitoring disease activity in certain patients. That is, the sera from some patients with high-titre antibodies continued to show high index values in the ELISA after clinical improvement. OBJECTIVES: In the study reported here, we modified the ELISA protocol to obtain 'true' index values that exhibit a better correlation with disease activity. METHODS: We tested two cases of pemphigus foliaceus (PF) and four cases of pemphigus vulgaris (PV), each with ELISA index values greater than 150 for Dsg1 or Dsg3. We ran an ELISA with sera from these patients serially diluted from 1 : 100 to 1 : 12,800. We then performed ELISA with a series of PV No. 1 sera diluted to 1 : 800 and PV No. 2-4 and PF No. 1-2 sera diluted to 1 : 1600, after which we plotted the ELISA index values against the time course of disease activity. RESULTS: In each of these cases, there was no apparent decline, over the course of the disease activity, in the ELISA index values at a serum dilution of 1 : 100, probably because the antigen-antibody reaction was saturated at that dilution. After running an ELISA with sera serially diluted from 1 : 100 to 1 : 12,800 we found that a linear dose-dependency between the dilution value and the index value was only observed when sera were diluted to 1 : 800 or more in one case (PV No.1) and to 1 : 1600 or more in the other five cases (PV No. 2-4, PF No. 1-2). After performing ELISA with these series as outlined above we plotted the ELISA index values against the time course of disease activity and found that the index values obtained from these appropriately diluted sera fluctuated in parallel with disease activity, and declined with clinical improvement. CONCLUSIONS: These findings indicate that when appropriate dilutions are used in Dsg1 and Dsg3 ELISA, these assays can provide useful serological information for assessing disease activity in PF and PV.

Adult↗
PubMed2001

Unilateral cone dysfunction with bull's eye maculopathy.

OBJECTIVE: To report a Japanese subject who presented with an acute-onset, unilateral cone dysfunction with bull's eye maculopathy and to describe the functional changes determined by psychophysical and electrophysiologic tests. DESIGN: A single observational case report. METHODS: In addition to a complete ophthalmic examination, the subject underwent some electrophysiologic and psychophysical tests. MAIN OUTCOME MEASURES: Kinetic visual field test, cone and rod perimetry (two-color perimetry), full-field electroretinograms (ERGs), focal macular ERGs, and multifocal ERGs. RESULTS: The full-field ERGs and two-color perimetry showed a predominant loss of cone function in the right eye, whereas the left eye was normal. Cone perimetry and multifocal ERGs revealed that there were small regions functioning normally and other retinal areas that were severely altered in the right eye. CONCLUSIONS: The topographical function analysis suggested that the disorder affected the retina unevenly. The cause of this rare case of unilateral cone dysfunction with bull's eye maculopathy still remains unknown.

Acute Disease↗
PubMed2001

Eosinophilic pustular folliculitis (Ofuji's disease): indomethacin as a first choice of treatment.

Eosinophilic pustular folliculitis (EPF) is characterized by erythematous patches of large follicular papules and pustules involving mainly the face. Although various treatments have been attempted for EPF, including systemic and topical steroid, diaphenylsulphone, colchicine, minocycline as well as UVB phototherapy, there is no consensus on the first choice of treatment. We report a typical case and summarize 25 patients with EPF treated in our hospital between 1978 and 1998. Indomethacin was most frequently used (12/25) and showed clinical improvement in the majority of the cases (11/12). The effect of indomethacin was usually observed within 1--2 weeks after initiation of treatment. Decrease of peripheral blood eosinophils accompanied the clinical improvement. Thus, indomethacin should be considered as a first choice of treatment for EPF.

Anti-Inflammatory Agents, Non-Steroidal↗
PubMed2001

Multifocal ERG findings in complete type congenital stationary night blindness.

PURPOSE: To study the multifocal electroretinogram (mfERG) in patients with the complete type of congenital stationary night blindness (cCSNB), which is thought to be due to a defect in neurotransmission from the photoreceptors to the ON-bipolar cells. METHODS: mfERGs were recorded with the VERIS recording system from four patients with cCSNB, none of whom had nystagmus. The stimulus array consisted of 61 hexagons, and the total recording time was approximately 4 minutes. The amplitudes and implicit times of the first- and second-order kernels of the local responses were compared with those from 20 myopic controls. Waveforms of the summed response from all locations were also compared between the two groups. RESULTS: The first-order kernels of the mfERGs of cCSNB patients had normal amplitudes but delayed implicit times for nearly the whole field tested. The second-order kernel was severely attenuated in amplitude in cCSNB patients. The ratios of the second- to first-order kernel amplitudes were significantly reduced in cCSNB and clearly separated the cCSNB group from the control group without any overlap of the values. CONCLUSIONS: The second-order kernel, which is involved in adaptative mechanism of the retina to repeated flashes, is selectively reduced in cCSNB. The delay of the implicit times of the first-order kernel in patients with cCSNB may be related to the severe amplitude reduction of the second-order kernel.

Adolescent↗
PubMed2001

A contact lens electrode with built-in high intensity white light-emitting diodes. A contact lens electrode with built-in white LEDs.

We determined the clinical usefulness of a new contact lens electrode with built-in, white light-emitting diodes (LEDs) for full-field electroretinograms (ERGs). Three, high-brightness white LEDs were incorporated into a contact lens electrode and served as the source for the stimulus and the background. The stimulus intensity, stimulus duration and background illumination were regulated by a small LED control device. Maximum stimulus and background intensities were 3.9 and 3.6 log cd/m2, respectively. We successfully recorded intensity-response series for scotopic and photopic ERGs. We also recorded a duration-series for photopic ERGs, and ERGs that were comparable to the ISCEV standardized ERGs. The compactness and ease of using this system suggest that it will be clinically useful under different conditions.

Adult↗
PubMed2000

Neovascular Maculopathy Associated with Rubella Retinopathy.

Background: We report three eyes of two patients with rubella retinopathy which were associated with choroidal neovascularization in the macula.Cases: A 7-year-old girl (case 1) and a 12-year-old girl (case 2) whose mothers had suffered rubella during their pregnancy revealed typical rubella retinopathy in both eyes and neovascular maculopathy in one eye at their initial visit to our clinic. Neovascular maculopathy developed in another eye of case 1 after a follow-up of 10 months.Results: The clinical characteristics indicated that (1) sudden visual loss occurs in children, (2) there is no external trigger such as eye injury, and (3) the visual prognosis appears relatively good.Conclusion: Although neovascular maculopathy is rare in pediatric disease, the rubella retinopathy may have the potential to cause neovascular maculopathy.

Journal Article↗
PubMed2000

Multifocal electroretinogram in occult macular dystrophy.

PURPOSE: Occult macular dystrophy (OMD) is an unusual macular dystrophy presenting with an essentially normal fundus and fluorescein angiography but with progressive central visual loss. The authors studied the function of local retinal areas in the posterior pole of patients with OMD using multifocal electroretinograms (ERGs). METHODS: Multifocal ERGs were recorded using the Visual Evoked Response Imaging System with 61 hexagonal elements within a visual field of 30 degrees radius from 8 OMD patients and 20 age-matched, normal subjects. The amplitudes and implicit times of the patients and normal control subjects were compared at the various retinal eccentricities. RESULTs. The amplitudes of the multifocal ERGs in the OMD patients were markedly reduced in the central 7 degrees of the fovea. The difference of the ERG amplitudes between OMD and normal subjects became smaller toward the peripheral retina. Most OMD patients had slight but significantly delayed implicit times across the whole testing field, and the differences between the OMD and the normal subjects did not change with retinal eccentricity. CONCLUSIONS: Our results for multifocal ERG amplitudes support the idea that OMD patients have localized retinal dysfunction distal to the ganglion cells in the central retina. The delayed implicit times across the whole test field suggest that the retinal dysfunction has a broader boundary than expected by ERG amplitudes and psychophysical perimetric results.

Adult↗
PubMed2000

[Neovascular maculopathy associated with rubella retinopathy].

BACKGROUND: We report three eyes of two patients with rubella retinopathy which were associated with choroidal neovascularization in the macula. CASES: A 7-year-old girl (case 1) and a 12-year-old girl (case 2) whose mothers had suffered rubella during their pregnancy revealed typical rubella retinopathy in both eyes and neovascular maculopathy in one eye at their initial visit to our clinic. Neovascular maculopathy developed in another eye of case 1 after a follow-up of 10 months. RESULTS: The clinical characteristics indicated that 1. sudden visual loss occurs in children, 2. there is no external trigger such as eye injury, and 3. the visual prognosis appears relatively good. CONCLUSION: Although neovascular maculopathy is rare in pediatric disease, the rubella retinopathy may have the potential to cause neovascular maculopathy.

Child↗
PubMed2000

A high association with cone dystrophy in Fundus albipunctatus caused by mutations of the RDH5 gene.

PURPOSE: To analyze the RDH5 gene in patients with fundus albipunctatus with and without cone dystrophy and to determine whether the disease is stationary or progressive and whether the cone dystrophy is a part of fundus albipunctatus or a separate disease. METHODS: Fourteen patients from 12 separate Japanese families with fundus albipunctatus were examined. Six of the patients from 6 families also had a cone dystrophy. Genomic DNA was extracted from leukocytes of the peripheral blood, and exons 2, 3, 4, and 5 of the RDH5 gene were amplified by polymerase chain reaction and were directly sequenced. A complete ophthalmic examination was performed including best-corrected visual acuity, slit-lamp examination, indirect ophthalmoscopy, fundus photography, and electroretinography. RESULTS: In all the patients, either a homozygous mutation or compound heterozygous mutations in the RDH5 gene were identified. The identified mutations were nucleotide position (nt) 103 G to A (Gly35Ser), nt 319 G to C (Gly107Arg), nt 394 G to A (Val132Met), nt 719 G insertion (frame shift), nt 839 G to A (Arg280His), nt 841 T to C (Tyr281His), and nt 928 C to GAAG (Leu310 to GluVal). All these mutations except the Arg280His were new. The nt 928 C to GAAG mutation was detected in patients with and without cone dystrophy. Cone dystrophy was most frequently seen in patients over 40 years old. CONCLUSIONS: Fundus albipunctatus either with or without cone dystrophy is caused by mutations of the RDH5 gene. Cone dystrophy is frequently observed in elderly patients with fundus albipunctatus. The conclusion was reached that the mutations of the RDH5 gene caused a progressive cone dystrophy as well as night blindness.

Adolescent↗
PubMed1999

Abnormal focal macular electroretinograms in eyes with idiopathic epimacular membrane.

PURPOSE: To study the functional state of the retina beneath an epimacular membrane by means of focal macular electroretinography. METHODS: Focal macular electroretinograms of 30 consecutive patients (age 25 to 75 years) with unilateral idiopathic epimacular membrane were recorded prospectively. The amplitudes and implicit times of the a and b waves and the oscillatory potentials (O1, O2, O3) recorded from the eye with idiopathic epimacular membrane were compared with those of the normal fellow eye. RESULTS: There was a statistically significant reduction in the amplitude of the a wave (P < .001), the b wave (P < .001), and the oscillatory potentials (O1 + O2 + O3; P < .001) of the affected eyes. The reduction of the b-wave amplitude was significantly greater than that of the a wave, resulting in a lower b/a wave ratio (P = .003) in the affected eyes. The reduction in the amplitude of the oscillatory potentials was significantly greater than that of the other two components (P < .001). The implicit times were significantly prolonged for the a wave (P < .001), the b wave (P = .004), and 03 (P < .001). There was a significant correlation between relative b-wave amplitude (affected/normal fellow eye) and the visual acuity (r = -0.50, P = .007). These findings were similar to those we reported for eyes with aphakic cystoid macular edema. CONCLUSIONS: The focal macular electroretinograms in eyes with epimacular membrane disclosed abnormalities that were similar to those of eyes with cystoid macular edema. This suggests that the epimacular membrane probably induced damage to the neurons in the inner retinal layers. Dysfunction of these neurons is one possible cause of visual impairment in this disease.

Adult↗