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PubMed2000

Systemic scleroderma in Greece: low mortality and strong linkage with HLA-DRB1*1104 allele.

OBJECTIVE: Description of Greek patients with scleroderma with reference to (a) major organ disease, (b) autoantibodies, (c) survival rate, and (d) HLA associations. METHODS: The clinical files of 254 patients were analysed retrospectively and a standardised clinical chart was completed with age at disease onset, sex, date of first and last visit, clinical and serological findings, organs affected, reasons for death, and HLA class II alleles. HLA class II alleles (DRB1, DQA1, DQB1, DPB1) were determined by polymerase chain reaction amplification using oligopeptide probes. DNA was extracted from 98 patients and 130 Greek controls. RESULTS: 124 patients (49%) had limited systemic sclerosis (lSSc), 114 (45%) had diffuse systemic sclerosis (dSSc), and 16 (6%) had overlap syndromes. Patients with dSSc, compared with lSSc, were characterised by a higher prevalence of lung disease (p=0.0011), oesophageal, heart, and peripheral vessel disease (p=0.027, p=0.0025, and p=0.012, respectively). Anticentromere antibodies (ACA) occurred exclusively in lSSc (34%), whereas antibodies to topoisomerase I (anti-topo I) were associated with dSSc (p<0.0001). Anti-topo I were associated with interstitial pulmonary fibrosis, oesophageal and peripheral vessel disease (p=0.028, p=0.012, and p=0.01, respectively). The HLA-DRB1*1104 allele was associated with the disease (p<0.0001) and anti-topo I (p<0.001), whereas it was not associated with ACA serum reactivity (p<0.001). Renal disease occurred in 4% of patients with SSc. The estimated survival probability for this cohort of patients with SSc, four years after the first visit, is 94.8%. CONCLUSION: SSc among Greek subjects has the same pattern of organ disease as in other white populations. However, the prevalence of kidney disease is low. The HLA class II DRB1*1104 allele is associated with the disease, with anti-topo I, and not associated with ACA serum reactivity.

Adult↗
PubMed2006

Induction of apoptosis and fibrillin 1 expression in human dermal endothelial cells by scleroderma sera containing anti-endothelial cell antibodies.

OBJECTIVE: Sera from patients with scleroderma (systemic sclerosis [SSc]) contain anti-endothelial cell antibodies (AECAs) capable of inducing endothelial cell apoptosis. We sought to determine whether SSc sera containing anticentromere antibodies (ACAs) or anti-topoisomerase I antibodies (or, anti-Scl-70 antibodies) contain subsets of AECAs that trigger distinct pathways of apoptosis and gene expression in normal adult human dermal endothelial cells (HDECs). METHODS: Adult HDECs were grown to subconfluence and treated with control or SSc patient sera. Apoptosis was investigated by differential interference contrast (DIC) microscopy, microarrays of proapoptotic gene expression, caspase 3 protease activity, and flow cytometry for phosphatidyl serine translocation. RESULTS: Flow cytometry and DIC microscopy demonstrated that HDECs exposed to SSc sera containing either SSc autoantibody underwent apoptosis at much higher levels than those treated with control sera. While unique gene expression profiles were induced in HDECs by stimulation with SSc sera containing the respective autoantibody, similar patterns of increased gene expression of transcripts for the proapoptotic protease caspase 3 as well as the SSc autoantigen fibrillin 1 were demonstrated. Caspase 3 gene expression correlated with increased protease activity, and targeted inhibition of this protease partly blocked SSc serum-induced apoptosis. Immunohistochemistry studies of serum-stimulated HDECs demonstrated the aberrant expression of fibrillin 1 protein only in apoptotic endothelial cells treated with SSc sera containing AECAs. CONCLUSION: There are distinct AECA subsets in the sera of patients with limited SSc (with ACAs) and diffuse SSc (with anti-Scl-70) that induce unique patterns of HDEC gene expression in the setting of apoptosis associated with increased caspase 3 activity and the reexpression of endothelial cell fibrillin 1.

Adult↗
PubMed1991

[Eosinophilia-myalgia syndrome and L-tryptophan intake].

We present the case of a 52-year-old woman who developed diffuse induration of the skin and severe edema of the subcutaneous tissue involving the extremities and the trunk, sparing hands, feet and face after 10 years of almost constant oral tryptophan medication. The skin manifestations were similar to those of eosinophilic fasciitis (Shulman syndrome). The patient complained of severe muscle pain and weakness. Laboratory studies revealed an elevated Westergren erythrocyte sedimentation rate and eosinophilia. There were no signs of internal organ involvement and no immunological parameters of progressive systemic scleroderma. Eosinophilia and myalgia resolved in response to intermittent systemic therapy with glucocorticosteroids, whereas the progressive scleroderma and the edema showed only slight improvement after the discontinuation of L-tryptophan.

Biopsy↗
PubMed2005

[A case of diffuse alveolar hemorrhage associated with interstitial pneumonia and systemic sclerosis].

A 73-year-old female with interstitial pneumonia and systemic sclerosis was admitted with bloody sputum and fever. A chest radiograph showed bilateral infiltrative shadows superimposed on interstitial pneumonia. A chest computed tomograph also showed bilateral airspace consolidations and honeycomb formation. Mechanical ventilation was required because of respiratory failure. At intubation, fiberoptic bronchoscopy revealed bilateral bronchi to be filled with bloody secretions. Her serum myeloperoxidase-anti-neutrophil cytoplasmic antibody titer was slightly elevated. Steroid pulse therapy (1000 mg/day methylprednisolone for 3 days) and cyclophosphamide pulse therapy (500 mg cyclophosphamide) followed by 60 mg/day prednisolone was introduced on the basis of a diagnosis of diffuse alveolar hemorrhage. Her respiratory condition improved and chest infiltrative shadows disappeared and weaning from mechanical ventilation was successful. However, she died of ventilation insufficiency due to progression of her chronic respiratory failure. A necropsy specimen obtained from the left lung showed fibrosis, predominantly in peripheral areas of lobules, and accumulation of hemosiderin-laden macrophages, which findings were consistent with usual interstitial pneumonia and diffuse alveolar hemorrhage. A review of the world literature revealed 4 case reports of diffuse alveolar hemorrhage associated with interstitial pneumonia and systemic sclerosis. We should be aware of this rare but serious complication in the management of interstitial pneumonia associated with systemic sclerosis.

Aged↗
PubMed1994

Severe restrictive lung disease in systemic sclerosis.

OBJECTIVE: We sought to identify risk factors for developing severe restrictive lung disease and to determine the time of onset and rate of progression in patients with systemic sclerosis (SSc). METHODS: Using the University of Pittsburgh Scleroderma Databank, we grouped patients according to their lowest forced vital capacity (FVC) value: > 75% predicted, 50-75% predicted, and < 50% predicted. In patients with severe restrictive disease, we examined serial pulmonary function test (PFT) results to determine the rate of loss of lung volume over time. RESULTS: Of 890 SSc patients, 60% (n = 531) never had an FVC < or = 75% predicted; 27% (n = 243) had moderate restrictive disease, with an FVC value of 50-75% predicted; and only 13% (n = 116) of the patients had severe restrictive disease, with FVC < or = 50% predicted. Black race, male sex, early disease, and primary cardiac involvement due to SSc were the features most frequently associated with severe restrictive lung disease (by multiple logistic regression). Fifty-five patients with severe restrictive lung disease had their first of at least 2 PFTs during the first 5 years after onset of any SSc (not pulmonary) symptoms. In 30 patients, the FVC declined by 32% per year in the first 2 years of illness, in 16 patients the annual loss was 12% in years 2-4 after disease onset, and in 9 patients annual loss was 3% during years 4-6 of disease (P < 0.005 by 1-way analysis of variance). CONCLUSION: In SSc patients, black men with early disease who have cardiac involvement are the most likely to have factors associated with the development of severe restrictive lung disease (which is increasingly becoming a major cause of death). Disease subtype (diffuse versus limited cutaneous) and serum anti-topoisomerase I antibody do not differentiate between moderate and severe restrictive disease. Careful monitoring of pulmonary function early in the disease, when the greatest loss of lung function occurs, may help identify patients likely to respond to new therapy.

Adult↗
PubMed1992

Two sisters producing anti-U1RNP exhibit serological concordance and clinical discordance.

Two sisters had autoimmune responses to the U1RNP particle that were quantitatively similar and/or identical in molecular characteristics. No other autoantibodies were demonstrable. Both sisters immunoprecipitated only U1RNA, had a reaction of identity in gel diffusion, bound the 68-kDa band in HeLa cell extract in Western blot, and reacted almost equally to a rabbit anti-idiotypic reagent made against either sister's isolated anti-U1RNP Fab fragments. They both carried a DR4 allele, which has been associated with anti-U1RNP production in several studies. While the sisters both had Raynaud's phenomenon, their clinical pictures were otherwise dissimilar. One had a seizure disorder (Ju); the other had polymyositis and features of scleroderma (Je). In sister Ju, Raynaud's phenomenon was manifest for the first time in association with the appearance of precipitating anti-U1RNP.

Adult↗
PubMed1999

Autoantibodies to the extracellular matrix microfibrillar protein, fibrillin-1, in patients with scleroderma and other connective tissue diseases.

A duplication in the fibrillin-1 gene has been implicated as the cause of the tight skin 1 (tsk1) phenotype, an animal model of scleroderma or systemic sclerosis (SSc). In addition to the production of abnormal fibrillin-1 protein, the tsk1 mouse also produces autoantibodies to fibrillin-1. Among a population of Choctaw Native Americans with the highest prevalence of SSc yet described, a chromosome 15q haplotype containing the fibrillin-1 gene has been strongly associated with SSc. With a recombinant human fibrillin-1 protein, autoantibodies to fibrillin-1 were detected in the sera of Native American SSc patients that correlated significantly with disease. Abs to fibrillin-1 also were detected in sera from Japanese, Caucasian, and African-American SSc patients. Compared with other ethnic groups, Japanese and Native American SSc patients had significantly higher frequencies of anti-fibrillin-1 Abs. Sera from patients with diffuse SSc, calcinosis, Raynaud's, esophageal dysmotility, sclerodactyly, and telangiectasias syndrome and mixed connective tissue disease also had significantly higher frequencies of anti-fibrillin-1 Abs than sera from controls or patients with other non-SSc connective tissue diseases (lupus, rheumatoid arthritis, and Sjögren's syndrome). Ab specificity for fibrillin-1 was demonstrated by the lack of binding to a panel of other purified autoantigens. The results presented demonstrate for the first time the presence of high levels of anti-fibrillin-1 Abs in a significant portion of patients with SSc.

Adult↗
PubMed1989

Pregnancy in women with systemic sclerosis.

We assessed fetal morbidity and mortality in women with systemic sclerosis (SSc). Women with a history of SSc and a concomitant pregnancy completed a detailed questionnaire about the pregnancy. These 48 subjects were age-matched and race-matched to 2 other groups of women (a rheumatoid arthritis group and a control group from the same neighborhood), all of whom had been pregnant at least once. There were no differences in the frequencies of miscarriage or perinatal death in the SSc group compared with the 2 control groups. Preterm births occurred slightly more frequently in both SSc patients and rheumatoid arthritis patients compared with the neighborhood control subjects. There were significantly more small full-term infants born to women with SSc. Interestingly, the increase in preterm births and small full-term babies occurred with equal frequency prior to and after the onset of disease. Although close monitoring for premature birth and intrauterine growth retardation is necessary, we conclude that an uneventful, healthy pregnancy is possible for women with SSc. Those with early, rapidly progressive, diffuse skin thickening should avoid becoming pregnant since, intrinsically, they are at higher risk of developing renal crisis.

Abortion, Spontaneous↗
PubMed2000

Nailfold videocapillaroscopy assessment of microvascular damage in systemic sclerosis.

OBJECTIVE: To correlate microvascular abnormalities, evaluated by nailfold videocapillaroscopy (NVC), with the duration of both Raynaud's phenomenon (RP) and systemic sclerosis (SSc) from the date of diagnosis, in a large number of patients with SSc. METHODS: Ninety-seven consecutive patients were recruited and distributed into 3 groups on the basis of the morphological NVC patterns observed: "early" (E), "active" (A), and "late" (L). In each group the age of patients, age at onset, and the duration of RP as well as of overt SSc were investigated and correlated with the different NVC pattern variables. RESULTS: The early appearance of giant capillaries and hemorrhages (E pattern) is of great relevance for the early diagnosis of SSc. Therefore, these alterations are more evident in the active phase of the disease (A pattern). Conversely, the NVC observation of loss of capillaries and vascular architectural disorganization and the presence of ramified/bushy capillaries (L pattern) represents the clearest aspect of advanced SSc microvascular damage. These morphological alterations were found to correlate significantly with the duration of both RP and SSc, as well as with age of patients (p = 0.0001). No significant differences were observed when the variables were analyzed in the patients classified as having limited cutaneous SSc or diffuse cutaneous SSc. CONCLUSION: Classification of defined major nailfold patterns may be useful in assessing the appearance and progression of sclerodermic microangiopathy. As well, nailfold changes might represent a morphological reproduction of the evolution of SSc.

Female↗
PubMed2005

Responsiveness of the SF-36 and the Health Assessment Questionnaire Disability Index in a systemic sclerosis clinical trial.

OBJECTIVE: This study compares the responsiveness to change of the Medical Outcomes Study Short Form Health Survey (SF-36), a measure of health related quality of life (HRQOL), and the Health Assessment Questionnaire Disability Index (HAQ-DI), a function instrument, in a randomized clinical trial for treatment of systemic sclerosis (SSc). METHODS: A phase 2/3, multicenter, prospective, placebo controlled trial was conducted to evaluate human recombinant relaxin treatment in patients with diffuse SSc over 24 weeks. At baseline, subjects had stable, moderately severe, diffuse SSc of disease duration < or = 5 years, modified Rodnan skin score > or = 20, serum creatinine < 2.0 mg/dl, percentage forced vital capacity (% FVC) predicted > or = 50%, and % DLCO predicted > or = 40% and were not receiving concomitant disease modifying therapies. Internal consistency reliability of multi-item scales was estimated using Cronbach's alpha. Responsiveness to change of the SF-36 and HAQ-DI was computed between Weeks 0 and 24. Subjects were classified as unchanged or having a meaningful change in 4 different external measures: Change in (1) skin score > or = 30%; (2) % FVC predicted of > or = 15%; (3) self-reported patient global assessment by visual analog scale (VAS) > or = 20%; and (4) physician global assessment by VAS of > or = 20%. Responsiveness indices were computed and Cohen's effect size criteria were used to assess the magnitude of change. RESULTS: A total of 239 patients participated in this trial, with 196 completing the 24 week trial. Cronbach's alpha for the SF-36 scales ranged from 0.76 to 0.93 and for the HAQ-DI ranged from 0.69 to 0.91 (good to excellent). The SF-36 had a larger magnitude of responsiveness in overall disease (patient and physician global assessment) compared to the HAQ-DI, while the HAQ-DI had a larger magnitude of responsiveness in clinical measures (i.e., change in skin score and % FVC predicted) than the SF-36. CONCLUSION: These data support inclusion of both the SF-36 and HAQ-DI as outcome measures in future clinical trials of diffuse SSc.

Adult↗
PubMed2002

Ulnar artery involvement in systemic sclerosis (scleroderma).

OBJECTIVE: Microvascular disease is one of the hallmarks of systemic sclerosis (SSc, scleroderma), but macrovascular involvement also exists in some patients. Patients with SSc may have severe Raynaud's phenomenon (RP) characterized by refractory digital ulcerations. We investigated if large artery involvement, that is, ulnar artery occlusion, has a role in the development of refractory digital ulcerations, and if both screening for this involvement and revascularization of the ulnar artery occlusive disease may improve digital ulcer healing. METHODS: A retrospective chart review was performed of 15 patients with SSc, all of whom had severe RP and digital ulceration, together with a positive Allen test and ulnar artery occlusive disease documented by angiography. RESULTS: Women outnumbered men 2:1, with limited disease predominating (7), 5 patients having diffuse cutaneous disease and 3 overlap syndromes. All patients had positive antinuclear antibody and capillary microscopy findings consistent with SSc. Antiphospholipid antibodies were present in 4 of 6 patients tested. Tobacco use was seen in 5 patients, only 2 of whom were current smokers. All patients failed conventional medical therapy (nitrates, calcium channel blockers, antiplatelet agents) for RP and digital ulceration. Only 1/8 patients improved with stellate ganglion block, and one patient had no improvement following digital sympathectomy. Eight patients underwent ulnar artery revascularization combined with digital sympathectomy, and 8 experienced dramatic improvement in RP and healing of digital ulcers. CONCLUSION: An Allen test should be performed routinely on all SSc patients with severe RP and refractory digital ulceration to investigate the possibility of ulnar artery occlusive disease. If suspected ulnar artery occlusion is confirmed by angiography or ultrasonography, ulnar artery revascularization with or without digital sympathectomy should be considered in patients who fail conventional medical therapy.

Antibodies, Antinuclear↗
PubMed2001

Severe alveolar proteinosis following chemotherapy for acute myeloid leukemia in a lung allograft recipient.

A 64-year-old man was diagnosed with acute myeloid leukemia (AML) 5 years following single lung transplantation performed for severe pulmonary hypertension from scleroderma. Chemotherapy for treatment of AML with fludarabine, cytosine arabinoside, G-CSF (FLAG) regimen was initiated. Despite intensive antibiotic treatment for a presumptive diagnosis of bacterial pneumonia, the patient developed acute respiratory failure and died before a complete cycle of chemotherapy could be administered. At autopsy, both native and allograft lungs showed widespread alveolar proteinosis that was determined as the main cause of acute respiratory failure. Alveolar proteinosis, a potentially treatable disease, should be considered in the radiologic differential diagnosis of diffuse lung disease in this clinical setting.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2006

Mycophenolate mofetil is safe, well tolerated, and preserves lung function in patients with connective tissue disease-related interstitial lung disease.

BACKGROUND: Interstitial lung disease (ILD) frequently complicates connective tissue diseases (CTDs). Glucocorticoids and immunomodulatory agents are regarded as mainstays of therapy for CTD-related ILD; however, apart from those studies that have evaluated certain medications for patients with scleroderma, few studies have been performed. In this study, our objectives were to examine the safety and tolerability of mycophenolate mofetil (MMF) and to determine its impact on lung function in patients with CTD-ILD. METHODS: In this retrospective observational study, we analyzed patients at our center who ever received MMF for CTD-ILD. We examined the frequency and severity of side effects associated with MMF and used longitudinal data analytic methods to determine the ability of MMF to maintain lung function over time. RESULTS: Twenty-eight patients were treated with MMF over 35.9 patient-years. The most common underlying CTD diagnosis was scleroderma (n = 9). The most common reason for initiating MMF was an adverse effect of a prior immunomodulatory agent. Six patients had clinically significant side effects related to MMF; all resolved with dose reduction. Compared to before MMF, the mean daily prednisone dose while patients were receiving MMF was lower (10 mg/d vs 15 mg/d, p = 0.09). In addition, since starting MMF, the average percentage of predicted forced vital capacity (FVC), average percentage of predicted total lung capacity, and average percentage of predicted diffusing capacity of the lung for carbon monoxide for the cohort increased by 2.3%, 4.0%, and 2.6%, respectively. CONCLUSION: MMF appears to be safe and well tolerated in patients with CTD-ILD. Larger-scale studies are needed to further evaluate the efficacy of MMF in this patient population.

Aged↗
PubMed1981

[New ways of studying the cutaneous microcirculation in clinical medicine (author's transl)].

Transcutaneous video microscopy opens a way to measure capillary red blood cell speed, to analyzed transcapillary diffusion and pericapillary distribution of Na-fluorescein injected i.v. and to depict the superficial lymphatic capillaries by subepidermal microinjection of FITC-labelled dextran in human skin. The dynamic phenomena may be quantitiated by video densitometers provided the information is stored on video tape. Already, some physiological and clinical data have been obtained. The velocity pattern of erythrocytes in nailfold capillaries is continuous or intermittent with flow stops. Standardized cold provocation tests are useful to evaluate Raynaud's disease. In patients with scleroderma the transcapillary passage of Na-fluorescein is increased, the physiological pericapillary halo partially destroyed and the interstitial distribution of the dye asymmetrical. In primary lymphedema the lymphatic network filled from the deposit of fluorescent dextran extends much more than in healthy controls. Pathological lymphatic microvessels occur.

Adult↗
PubMed1997

Clinical, laboratory and radiological findings in pulmonary fibrosis with and without connective tissue disease.

Uncertainty exists over whether to consider "lone" idiopathic pulmonary fibrosis (LIPF) and pulmonary fibrosis associated with connective tissue disorders (PFCTD) as significantly different entities. We retrospectively analysed data collected at the time of first diagnosis in 17 patients with LIPF and in 14 patients with PFCTD and compared survival in the two groups. At first evaluation, the time from onset of respiratory symptoms, spirometric volumes and the diffusing capacity for carbon monoxide were not significantly different between the two groups. However, arterial oxygen tension was significantly lower in LIPF than in PFCTD (63 +/- 3 vs 88 +/- 3 mmHg, p < 0.001). The radiological profusion scores relative to the upper and middle lung fields were significantly higher in LIPF than in PFCTD (upper regions: 6.9 +/- 0.6 vs 3.4 +/- 0.6, p < 0.005 - middle regions: 7.1 +/- 0.5 vs 4.8 +/- 0.7, p < 0.025), whereas the scores relative to the lower fields were similar (7.4 +/- 0.4 in LIPF and 8.4 +/- 0.6 in PFCTD). Survival since onset of respiratory symptoms was significantly better in the PFCTD than in LIPF patients, with a hazard ratio of 4.16 (95% CI 1.12-15.58, p=0.034). Thus, in our series of patients, those with LIPF had a more severe disease than those with PFCTD as shown by the higher frequency of hypoxaemia, the more diffuse pulmonary involvement demonstrated by the chest X-ray and the decreased survival.

Antibodies, Antinuclear↗
PubMed1991

Acute and long-term effects of nifedipine on pulmonary and systemic hemodynamics in patients with pulmonary hypertension associated with diffuse systemic sclerosis, the CREST syndrome and mixed connective tissue disease.

Ten patients with pulmonary hypertension associated with diffuse systemic sclerosis (1 patient), the CREST syndrome (calcinosis cutis, Reynaud's phenomenon, esophageal dysmotility, sclerodactyl, telangiectasia) (6 patients) and mixed connective tissue disease (3 patients) were studied to assess the effect of oral nifedipine on pulmonary and systemic hemodynamics. Each patient underwent right-sided cardiac catheterization just before nifedipine administration. Thereafter, oral nifedipine was administered in 10 mg increments every 90 minutes until pulmonary vascular resistance normalized or a total dose of 30 mg was achieved. Hemodynamic measurements were obtained at 30-minute intervals for 3 hours, then hourly for 9 hours (acute study). Hemodynamic studies were repeated 3 to 6 months after the initial catheterization with the minimum dose of oral nifedipine (administered every 8 hours) required to achieve maximal reduction of pulmonary vascular resistance in the acute study (long-term study). In the acute study, oral nifedipine produced a significant decrease in mean pulmonary vascular resistance from 6.3 +/- 3.8 to 4.3 +/- 3.6 U (p less than 0.001). Similar changes in pulmonary vascular resistance were noted in the long-term study (n = 6). The results indicate that oral nifedipine is capable of producing an acute and sustained reduction in pulmonary vascular resistance in patients with pulmonary hypertension associated with diffuse systemic sclerosis, the CREST syndrome and mixed connective tissue disease.

Adult↗
PubMed1996

Multiple periorbital cutaneous myxomas progressing to scleromyxedema.

Myxomas are rare cutaneous tumors that have been associated with a variety of other abnormalities including atrial myxomas, endocrine abnormalities, and bone malformations. We describe a 38-year-old white man with multiple periorbital myxomas in whom myalgias, fatigue, and more diffuse cutaneous involvement developed. These findings were consistent with scleromyxedema. He also had an associated left subclavian deep venous thrombosis. The patient responded well to therapy with cyclophosphamide.

Adult↗
PubMed1987

[The sclerodermatous group of diseases].

The paper is concerned with the author's considerations on a sclerodermic group of diseases including a wide spectrum of diseases with systemic or local "primary" fibrosis on the basis of the results of her own investigations and the literature data. Multifactorial genesis of diseases, cellular mechanisms and the interrelationship of fibrosis with immune and microcirculatory disturbances are discussed with regard to sclerodermia systematic. Recently defined, hitherto unknown diseases and syndromes were described: diffuse eosinophilic fasciitis, the paraneoplastic sclerodermic syndrome "induced" sclerodermia, local fibroses. An analysis of the sclerodermic group of diseases and the chief mechanisms of their development is important for improved diagnosis and development of effective programs of pathogenetic therapy, prevention and mass screening.

Adult↗