Evaluation of the hypothalamo-pituitary-adrenal axis during the post-COVID-19 period in patients treated with steroids during the illness

ABSTRACT Objective: COVID-19 is a multisystem immunoinflammatory disorder, and the hypothalamo-pituitary-adrenal (HPA) axis may be affected by SARS-CoV-2 as well as by steroid treatment during the illness. Information on the HPA axis after recovering from COVID-19, especially in those treated with steroids, is sparse. Hence, this study was performed to evaluate the hypothalamo-pituitary-adrenal axis during the post-COVID-19 period in patients treated with steroids during the illness. Subject and methods: This prospective study involved 60 patients aged 18-60 years who had recovered from moderate or severe COVID-19 and had received steroid treatment during the illness. The HPA axis was assessed with a low-dose (1 mcg) adrenocorticotropic hormone stimulation test at 3, 6 and 9 months in the post-COVID period. Results: The HPA axis was suppressed in 31.66% of the patients at 3 months and 5% at 6 months; however, all patients recovered at 9 months. Cumulative steroid use during the illness was inversely correlated with stimulated cortisol at 3 months in the post-COVID period. Fatigue was present in 58.33% of the patients at 3 months and was more prevalent in those with HPA axis suppression. Conclusion: Nearly one-third of the patients with moderate to severe COVID-19 who were treated with steroids had suppressed HPA axis at 3 months, with gradual recovery over a period of 9 months. Cumulative steroid equivalent dose, but not disease severity, was predictive of HPA axis suppression at 3 months.

Unique Cytomegalovirus Tongue Ulcer in an Older Adult Patient with Prolonged Systemic Steroid Treatment

Introduction: Cytomegalovirus (CMV) is a member of the Herpesviridae family that infects humans. It infects approximately 90% of adults. It is an opportunistic pathogen, common among immunosuppressed patients and can affect multiple organs. To date, there has been only one reported case of a CMV-related tongue ulcer that occurred during steroid treatment. Case Presentation: We report a case of CMV tongue ulcer in an elder, immunodeficient patient under prolonged steroid treatment. Management and Prognosis: A 67-year-old man under chronic steroidal treatment due to severe chronic obstructive pulmonary disease (COPD), with chronic ischemic heart disease (CIHD) and poorly treated diabetes mellitus (DM) was hospitalized in Soroka university medical center. He suffered from an ulcerative wound about 10 mm in its greater diameter with raised margins on the left lateral tongue. Daktarin Oral Gel treatment showed no improvement. On histopathological examination, a diagnosis of CMV-related tongue ulcer was given. Conclusion: To the best of our knowledge, this is the most detailed case report of CMV tongue ulcer due to prolonged steroid treatment. An early diagnosis of CMV infection in patients with oral lesions is crucial because CMV infection can increase immunosuppression, and is associated with opportunistic infections.

A Case Report of Blau Syndrome / 罕见病研究

Blau syndrome is a rare genetic disorder characterized by the a mix of granulomatous arthritis, uveitis, and dermatitis. Patients typically manifest multisystem involvement, including ocular, skin, and skeletal abnormalities. Blau syndrome is extremely rare, with a global incidence of less than one in a million among children. In this multidisciplinary consultation, we present a case of a 21-year-old young female patient having multisystemic involvement since early childhood. She was presented with multiple joint swelling, skin lesions, increased eye discharge, and accompanied by hypertension and arterial abnormalities, and received a diagnosis of uveitis. The patient had been receiving steroid treatment since the age of 6 and has tried various medications, with some improvement in joint swelling and ocular symptoms. Through this rare disease multidisciplinary consultation, we aim to provide guidance in the molecular diagnosis of the patient, multisystem assessment, and the selection and formulation of treatment plans. Additionally, we hope that by reporting this case, clinical physicians can gain a better understanding of the diagnosis and comprehensive treatment strategies for Blau syndrome, thereby improving the management and treatment of rare diseases.

Effect and Prognostic Factors of Systemic Steroid Treatment for Strabismus Associated Thyroid Ophthalmopathy

PURPOSE: To investigate the effect of steroid treatment on strabismus associated with thyroid ophthalmopathy. METHODS: The present retrospective study was conducted on 22 patients diagnosed with strabismus associated with thyroid ophthalmopathy, who were treated with steroids orally or intravenously and followed up for more than one year. Patients were divided into three groups for analysis: an improved group, with no strabismus at the final follow-up visit; a stable group, with no change in the strabismus angle; and a deteriorated group, in which the strabismus angle had worsened. We investigated the characteristics of each group. RESULTS: In the gender/sex distribution of the 22 patients, 11 of the 15 patients in the improved or stable group were female; six of seven patients in the deteriorated group were male (p = 0.012). Two of the 15 patients in the improved or stable group had a smoking history. However, none smoked after the treatment had started. On the other hand, five of seven patients in the deteriorated group had a smoking history and continued to smoke during and after treatment (p = 0.001). No significant between-group differences were observed with respect to age, diplopia period, strabismus angle, or thyroid function level. CONCLUSIONS: Treatment with steroids may improve or stabilize strabismus associated with thyroid ophthalmopathy. However, the effect of treatment may differ, depending on whether the patient continues to smoke after treatment is initiated.

Retroperitoneal fibrosis in the era of immunoglobulin G4-related disease

BACKGROUND: Retroperitoneal fibrosis (RPF) is a rare disease characterized by fibroinflammatory tissue in the periaortic or periiliac retroperitoneum, where it frequently encases ureters. There is emerging evidence that a subset of this disease is part of a spectrum of multisystemic autoimmune diseases collectively referred to as “immunoglobulin G4 (IgG4)-related disease”. METHODS: We retrospectively analyzed 27 idiopathic RPF patients and identified a subset as IgG4-related RPF, which we categorized according to recently published comprehensive diagnostic criteria. We compared clinical and laboratory characteristics and response to treatment between the two groups. RESULTS: Of 27 total patients, 16 (59.3%) were diagnosed as having IgG4-related RPF, and these were predominantly male. They were also significantly older and more likely to have other organ involvement, hydronephrosis, and postrenal acute kidney injury (AKI) compared to those with idiopathic RPF. However, there was no difference in response rate to systemic steroid treatment. CONCLUSION: IgG4-related RPF accounts for a substantial portion of RPF cases previously identified as “idiopathic RPF” in Korea. Clinical and laboratory characteristics of IgG4-related RPF are similar to those of idiopathic RPF except for a striking male predominance, older age, and higher incidence of postrenal AKI in IgG4-related RPF. More comprehensive, prospective studies are needed to clearly distinguish IgG4-related RPF from idiopathic RPF based on clinical manifestation and to further assess treatment response and long-term prognosis.

A Korean patient with Guillain-Barré syndrome following acute hepatitis E whose cholestasis resolved with steroid therapy

Autochthonous hepatitis E virus (HEV) is an emerging pathogen in developed countries, and several cases of acute HEV infection have been reported in South Korea. However, there have been no reports on HEV-associated Guillain-Barré syndrome (GBS) in Korea. We recently experienced the case of a 58-year-old Korean male with acute HEV infection after ingesting raw deer meat. Persistent cholestasis was resolved by the administration of prednisolone. At 2.5 months after the clinical presentation of HEV infection, the patient developed weakness of the lower limbs, and was diagnosed with GBS associated with acute hepatitis E. To our knowledge, this is the second report on supportive steroid therapy for persistent cholestasis due to hepatitis E, and the first report of GBS in a Korean patient with acute HEV infection.

Two Cases of Young Patients with Bilateral Posterior Scleritis Accompanied with Serous Retinal Detachment

PURPOSE: To report 2 young female patients with bilateral posterior scleritis and serous retinal detachment. CASE SUMMARY: An 11-year-old girl (Case 1) and a 16-year-old girl (Case 2) visited our clinic with bilateral ocular pain, redness, and blurred vision. Slit lamp examinations revealed severe bilateral scleral injection and mild anterior chamber reactions. Fundus examinations showed bilateral serous retinal detachments in the macular area. In both patients, diffuse multifocal leaking and pooling were found at the macula in the early and late phase fluorescein angiography, respectively. On the B-mode ultrasounds and orbital images (MRI or CT), scleral thickening with retention of subtenons fluid were found. There were no systemic diseases associated with the conditions. We diagnosed the patients with bilateral posterior scleritis and administered systemic steroids. After systemic steroid treatment, all of the symptoms were alleviated. Three months after the regression, bilateral posterior scleritis recurred in patient 2. Oral cyclosporine 100 mg was additionally prescribed in addition to the previous medications that she had taken during her first treatment. CONCLUSIONS: Two young patients diagnosed with posterior scleritis and serous retinal detachment was treated with systemic steroids. With proper examination and treatment, posterior scleritis can be treated with no complications.

Clinical Characteristics of Inflammtory Pseudotumor of the Orbit / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: Inflammatory pseudotumor is not malignant, but similar to malignancy lesions with respect to its clinical and radiological manifestations. It is well-known to opthalmologists and neurosurgeons, but unfamiliar to otolaryngologists. The objective of this study is to determine the diagnosis and treatment of inflammatory pseudotumor of the orbit. SUBJECTS AND METHOD: We reviewed 17 patients who visited Kangnam St. Mary's hospital and were diagnosed with inflammatory pseudotumor with clinical manifestations and radiological studies between September, 1994 and December, 2006. RESULTS: Seventeen patients were treated with high dose steroid, and of these, 12 showed clinical improvement, although 7 of these patients later showed recurrence. Of the five patients who initially showed no clinical improvement, one received radiation therapy but did not show improvement whereas two patients showed no improvement. One patient's symptoms improved after surgical decompression. CONCLUSION: Our study suggests that the initial treatment of inflammatory pseudotumor should be high dose steroid treatment without improvement and the second treatment should be radiation therapy or surgical treatment.

Diamond-Blackfan anemia: long-term follow-up of six cases / 소아과

PURPOSE: Diamond-Blackfan anemia (DBA) is a rare heterogeneous genetic disorder of infancy and early childhood. It is characterized by red cell aplasia, congenital anomalies, and a predisposition to cancer. Corticosteroids and red cell transfusions are the mainstays of therapy. We describe our experience of 6 cases of DBA that were encountered over a period of 16 years. METHODS: Medical records of 6 patients diagnosed to have DBA and admitted to the Chonnam National University Hospital between 1992 and 2008 were retrospectively reviewed. RESULTS: Three patients were males. The age at diagnosis ranged from 3 to 18 months (median, 5.5 months). Heart defects were observed in 4, polydactyly in 2, and strabismus in 1 patient. The median number of transfusions was 3 (range, 2 -8). All patients responded to initial treatment with steroids and had a hemoglobin level > or =9 g/dL with a median of 12.5 days (range, 7-22 days). Three patients are currently not receiving steroid therapy. A minimum dose of oral prednisolone ( or =9 mg/dL in 3 cases. Red cell transfusion was infrequently required in 1 patient. In the median follow-up of 14 years, there was no development of malignancy. No significant side effects of steroids were found, except for short stature in 2. CONCLUSION: The majority of DBA patients achieved complete response and under maintenance therapy with low dose of steroids. Close observation is needed to monitor steroid side effects, cardiac function, and development of malignancy. A nation-wide survey is necessary to further characterize this rare disease in Korean children.

Idiopathic Fibrosing Mediastinitis Causing Pulmonary Hypertension with Improvement by Steroid Treatment / 결핵및호흡기질환

Idiopathic fibrosing mediastinitis is, an uncommon cause of pulmonary hypertension this is characterized by excessive fibrosis of the mediastinum with an unknown etiology. Steroid therapy has been suggested for individuals with progressive symptoms, bu there is littlet data demonstrating the efficacy of such therapy are lacking. We present a case of pulmonary hypertension secondary to a compression of a main pulmonary artery by fibrosing mediastinitis which was confirmed by a biopsy with a thoracotomy. The chest CT scan and 2D echocardiography performed before and after a trial of steroid therapy demonstrated improvement after steroid therapy.

Initial Prednisolone Treatment for Primary Nephrotic Syndrome in Children-4 Weeks versus 6 Weeks

PURPOSE: Recently the merits of 6 weeks of initial prednisolone treatment for pediatric primary nephrotic syndrome have been reported, and the use of the 6 week regimen is increasing. We compared our experiences with the 6 week treatment versus the 4 week treatment for Korean patients. METHODS: We conducted a retrospective analysis of 69 children who had primary nephrotic syndrome and who were followed up for at least 12 months in the 4 major medical centers in Daegu. The remission rate, the relapse rate, the frequency of relapse and complication of steroid treatment were compared between the 4 weeks and 6 weeks treatment group. RESULTS: Of the 69 children, 42 were in the 4 week treatment group and 27 were in the 6 week group. The median age, blood pressure, serum total protein, serum albumin, cholesterol, creatinine, estimated creatinine clearance, 24 hour urine protein and 12 month cumulative dose did not differ between the two groups. Among the children who relapsed after steroid treatment, the relapse time was significantly later for the 6 week treatment group. The relapse rate after 1 year of treatment was 62% in the 4 week treatment group and 52% in the 6 week treatment group; however, there was no statistically significant difference between the two groups. The frequency of relapse at 12 months was 1.5+/-1.2 times in the 4 week treatment group and 1.1+/-1.2 times in the 6 week treatment group, and there was not different between the two groups. The most common side effects of steroid treatment were an increase of appetite and a cushingoid appearance, and there was no statistical difference between the two groups. Among the 27 children who had kidney biopsies performed, 21 suffered from minimal change nephrotic syndrome. CONCLUSION: The first relapse time after steroid treatment was significantly later in the 6 week steroid treatment group. The frequency of relapse and the 12 month cumulative dose of steroid were lower in the 6 week treatment group, but there was no statistical significance between the two groups. The side effects of steroid treatment did not differ between the two groups. We need to study the long term side effects and the advanced regimens of steroid treatment in the future.

Lumbar Spinal Stenosis Induced by Epidural Lipomatosis: A case report

The epidural lipomatosis is the unusual result of fat deposition in the extradural space of spinal canal, and most cases are associated with the chronic systemic steroid treatment. Symptoms related to spinal stenosis are postulated to result from spinal cord and nerve root compression. MR imaging is the first choice of the investigation method. Patients with acute progressive symptoms are best treated surgically, while others with stable symptoms can initially be treated medically with immediate tapering of their steroid dose. We reported one case of lumbar spinal stenosis induced by epidural lipomatosis receiving chronic steroid treatment. Surgical treatment could not be performed because of the poor general condition. Rehabilitation care and nonoperative therapy were effective in improving symptoms in lumbar epidural spinal stenosis induced by epidural lipomatosis.

Childhood Myasthenia Gravis: Clinical Features and Response to Steroid Treatment

PURPOSE: Myasthenia gravis(MG) is relatively rare in childhood and shows some distinct epidemiologic and clinical features according to ages and races. The role of immunosuppressive therapy such as steroid treatment is not well established. The objective of this study is to characterize clinical features of childhood MG and to evaluate the outcomes of steroid treatment. METHODS: We studied 23 MG patients(7 male and 16 females) with symptom onsets from 1 to 14 years of age(mean 4.1 years). RESULTS: Fifteen patients were ocular type and 6 patients were generalized type. Sero-positivity to acetylcholine receptor antibodies was found in 85% of the patients. Two patients who showed initial ocular manifestations experienced secondary generalization without steroid treatment. Alopecia totalis and moyamoya disease were associated with systemic MG in two cases. Eighteen patients were treated with both anticholine esterase and steroid while 2 patients were treated with steroid only. Complete remission was observed in 6 patients(30%) while 11 patients(55%) had one or more recurrences and only partial responses were acquired in 4 patients(20%). CONCLUSION: We found some different epidemiologic features from western countries including a relatively high proportion of ocular MG and earlier onset age especially in ocular MG. Further long-term follow up study on responses to steroid treatment will be necessary to evaluate clinical benefits in the disease progression.

Minimal Change Nephrotic Syndrome in Patients with HBs Antigenemia / 대한신장학회잡지

Minimal change nephrotic syndrome is the most common cause of adult nephrotic syndrome in Korea as well as in Asia. Even though hepatitis B virus (HBV) infection has been infrequently noted in patients with minimal change nephrotic syndrome, and even though there is a controversy in using steroid in patients with hepatitis B virus infections, impacts of HBV infection on the clinical course and the therapeutic modalities has not been evaluated. To elucidate this, we analysed clinicopathologic manifestations of 21 minimal change nephrotic syndrome patients with HBs antigenemia(HB-MCNS), in comparision with 25 minimal change nephrotic syndrome patients without any evidence of HBV infection(MCNS). The prevalence rate of HBs antigenimia among minimal change nephrotic syndrome was 8.7%. Age at diagnosis(median; HB-MCNS, 28 vs. MCNS, 22years : P<0.05), serum albumin level(median; HB- MCNS, 2.1 vs. MCNS, 1.8g/dL : P<0.05) and serum IgG level(median; HB-MCNS, 541 vs. MCNS, 271mg/dL : P<0.05) of HB-MCNS were higher than MCNS. C4(median; HB-MCNS, 36 vs. MCNS, 55mg/ dL : P<0.05) was lower. Other clinical findings including sex ratio, amount of 24HU protein, degree of hypercholesterolemia, seropositive rates for serologic markers such as rheumatoid factor, cryoglobulin, and ANA were not different between HB-MCNS and MCNS. The cumulative remission rates of 17 HB-MCNS patients who received steroid or cytotoxic therapy were 85% at 8th weeks and 100% at 11th weeks. Nephrotic syndrome was relapsed in 8% at 8th weeks and 38% at 70th weeks. These remission and relapse rate were not different from that of MCNS. During the course of steroid treatments, serum aspartate/alanine aminotransferase levels were elevated in 6 patients. Among those, 2 patients showed abnormal liver function persistent more than 4 weeks. One of them had positive seroconversion of HBeAg, and the other was proved to have liver cirrohsis. The negative seroconversion of HBeAg was not associated with clinical remission. Clinical finding suggested that HBV infection is unlikely a cause for most HB-MCNS. Even though steroids and cytotoxic agents was effective in HB-MCNS as much as in MCNS, careful monitoring of liver function and HBV marker is needed.

Selective Protein Index and Response to Treatment in the Childhood Nephrotic Syndrome

In a group of 59 children with nephrotic syndrome who had been admitted to Severance Ho-spital, during the period of 2 years from July of 1978 till June of 1980, the histological picture seen on renal biopsy, the selective protein index, and the response to the steroid therapy were studied. The degree of selective protein index is not related to the total daily amount of urinary protein excretion and selective protein index remains relatively constant over months in the minimal lesion nephrotic syndrom. Those patients with primary renal disease and the nephrotic syndrome who displayed high selectivity types of proteinuria usually responds initially to steroid therapy within 8 weeks. Studies of selective protein index allow the pediatrician to avoid renal biopsy with safety in nephrotic children aged 1 to 5 years, but distinguish any given renal disease with certainty. The mesangial proliferative glomerulonephritis with immune deposits has relatively poor selectivity of proteinuria and without immune deposits, it has high or moder-ate selectivity of proteinuria. Response to treatment of steroid in the Henoch-Sch?lein Purpura was poor and not related with selective protein index.