Cognitive functions in children with congenital adrenal hyperplasia

ABSTRACT Objective There is controversy regarding cognitive function in patients with congenital adrenal hyperplasia (CAH). This study is aimed at the assessment of cognitive functions in children with CAH, and their relation to hydrocortisone (HC) therapy and testosterone levels. Subjects and methods Thirty children with CAH due to 21 hydroxylase deficiency were compared with twenty age- and sex-matched healthy controls. HC daily and cumulative doses were calculated, the socioeconomic standard was assessed, and free testosterone was measured. Cognitive function assessment was performed using the Wechsler Intelligence Scale - Revised for Children and Adults (WISC), the Benton Visual Retention Test, and the Wisconsin Card Sorting Test (WCST). Results The mean age (SD) of patients was 10.22 (3.17) years [11 males (36.7%), 19 females (63.3%)]. Mean (SD) HC dose was 15.78 (4.36) mg/m 2 /day. Mean (SD) cumulative HC dose 44,689. 9 (26,892.02) mg. Patients had significantly lower scores in all domains of the WISC test, performed significantly worse in some components of the Benton Visual Retention Test, as well as in the Wisconsin Card Sorting Test. There was no significant difference in cognitive performance when patients were subdivided according to daily HC dose (< 10, 10 - 15, > 15 mg/m 2 /day). A positive correlation existed between cumulative HC dose and worse results of the Benton test. No correlation existed between free testosterone and any of the three tests. Conclusion Patients with CAH are at risk of some cognitive impairment. Hydrocortisone therapy may be implicated. This study highlights the need to assess cognitive functions in CAH.

Stevens-Johnson syndrome and toxic epidermal necrolysis in Dr. Hasan Sadikin General Hospital Bandung, Indonesia from 2009-2013

BACKGROUND: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe cutaneous adverse reactions (SCAR) with high mortality and have a significant public health impact because of high mortality and morbidity. OBJECTIVE: To describe data the epidemiological features, etiology, and treatment of retrospectively reviewed data of all patients with SJS and TEN. METHODS: Retrospective study was conducted in patients with SJS and TEN treated from January 1, 2009 to December 31, 2013 in Dr. Hasan Sadikin General Hospital Bandung, Indonesia. RESULTS: A total of 57 patients were enrolled in the study. Thirty-nine cases of SJS (21 males and 18 females), 7 cases of SJS overlapping TEN (4 males and 3 females), and 11 cases of TEN (5 males and 6 females) were reported. All cases of SJS and TEN were caused by drugs, such as paracetamol (16.56%), carbamazepine (7%), amoxicillin (5.73%), ibuprofen (4.46%), rifampicin (3.18%), and trihexyphenidyl (3.18%). All cases were treated systemically with corticosteroid alone (100%). Seven from 57 patients (12,28%) died; 5 cases developed sepsis and 2 cases developed respiratory failure. The mortality rate was 7.69% in SJS, 0% in SJS/TEN overlap, and 36.36% in TEN. CONCLUSION: The role of systemic corticosteroids in SJS and TEN are still controversial, but with a prompt and earlier treatment reduces mortality and improves outcomes of SJS and TEN patients.

Tuberculosis Lymphadenitis in a Well Managed Case of Sarcoidosis.

Differentiation between tuberculosis (TB) and sarcoidoisis is sometimes extremely difficult. Sequential occurrence of sarcoidosis and TB in the same patient is uncommon. We present the case of a young man, with a proven diagnosis of sarcoidosis who later developed TB after completion of treatment for sarcoidosis. A 32-year-old male patient presented with low-grade fever since two months. Physical examination revealed cervical lymphadenopathy. Initial fine needle aspiration cytology (FNAC) of the cervical lymph node was suggestive of granulomatous inflammation; the chest radiograph was normal. Repeat FNAC from the same lymph node was suggestive of reactive lymphoid hyperplasia. The patient was treated with antibiotics and followed-up. He again presented with persistence of fever and lymphadenopathy and blurring of vision. Ophthalmological examination revealed uveitis, possibly due to a granulomatous cause. His repeat Mantoux test again was non-reactive; serum angiotensin converting enzyme (ACE) levels were raised. This time an excision biopsy of the lymph node was done which revealed discrete, non-caseating, reticulin rich granulomatous inflammation suggestive of sarcoidosis. The patient was treated with oral prednisolone and imporved symptomatically. Subsequently, nearly nine months after completion of corticosteroid treatment, he presented with low-grade, intermittent fever and a lymph node enlargement in the right parotid region. FNAC from this lymph node showed caseating granulomatous inflammation and the stain for acid-fast bacilli was positive. He was treated with Category I DOTS under the Revised National Tuberculosis Control Programme and improved significantly. The present case highlights the need for further research into the aetiology of TB and sarcoidosis.

Clinical variables associated with leprosy reactions and persistence of physical impairment

Introduction Leprosy is a chronic disease that affects skin and peripheral nerves. Disease complications include reactional episodes and physical impairment. One World Health Organization (WHO) goal of leprosy programs is to decrease the number of grade 2 impairment diagnoses by 2015. This study aims to evaluate clinical factors associated with the occurrence of leprosy reactions and physical impairment in leprosy patients. Methods We conducted a retrospective study of data from medical records of patients followed in two important centers for the treatment of leprosy in Aracaju, Sergipe, Brazil, from 2005 to 2011. We used the chi-square test to analyze associations between the following categorical variables: gender, age, operational classification, clinical forms, leprosy reactions, corticosteroid treatment, and physical impairment at the diagnosis and after cure. Clinical variables associated with multibacillary leprosy and/or reactional episodes and the presence of any grade of physical impairment after cure were evaluated using the logistic regression model. Results We found that men were more affected by multibacillary forms, reactional episodes, and grade 2 physical impairment at diagnosis. Leprosy reactions were detected in a total of 40% of patients and all were treated with corticosteroids. However, physical impairment was observed in 29.8% of the patients analyzed at the end of the treatment and our multivariate analysis associated a low dose and short period of corticosteroid treatment with persistence of physical impairments. Conclusions Physical impairment should receive an increased attention before and after treatment, and adequate treatment should be emphasized. .

Long-term Follow-up of Adult-onset Minimal Change Nephrotic Syndrome / 대한신장학회잡지

BACKGROUND: Adult-onset minimal change nephrotic syndrome has been associated with a good response to corticosteroids and a benign prognosis. However, there are few longterm outcome data reported. METHODS: A series of 355 patients with adult-onset minimal change nephrotic syndrome (MCNS) who were admitted to Keimyung University Dongsan Medical Center from December 1978 to May 2002, was retrospectively analyzed to evaluate the initial response to corticosteroids, subsequent response to cytotoxic agents, the stability of remission, and long-term follow-up outcome. RESULTS: Of the 145 patients who followed up greater than two years, 69 patients (47.6%) showed early relapse, late relapser in 54 patients (37.2%), and no relapse in 22 patients (15.2%). Early relapsers showed younger age at onset, more frequent relapse, and longer duration of total corticosteroid treatment and shorter duration of corticosteroid used duration at relapse. Alkylating agents or cyclosporine were administered to 45 patients (19 steroid dependent, 13 frequent relapses, 8 steroid side effect, 2 infrequent relapses, 3 steroid resistant), 36 patients achieved complete remission, five in partial remission, and four in no response at all. Among 29 patients who were treated with cyclosporine, 23 patients showed complete remission, 4 achieved partial remission, and 2 showed no response. Final outcome of 145 patients who were followed for at least more than 2 years, were cyclosporine A dependent in 19 (13.2%) patients. steroid dependent in 16 (11.0%), persistent remission in 57 (39.3%), infrequent relapse in 49 (33.8%), death 2 (1.3%), renal failure 1 (0.7%), and no response to any therapy in 1 (0.7%). CONCLUSION: Adult-onset minimal change nephrotic syndrome shows favorable response to corticosteroid therapy with good long-term outcome and rarely progress to end-stage renal failure. However, new treatment regimens to minimize drug-related side effects and to maintain longer remission period should be evaluated prospectively.

A Clinical Analysis of Primary Focal and Segmental Glomerulosclerosis in Adults / 대한신장학회잡지

PURPOSE: Primary focal and segmental glomerulosclerosis(FSGS) is a clinicopathological entity defined by the segmental sclerosis involving glomeruli in a focal distribution with poor prognosis. Approximately 50% to 70% of adults with FSGS manifests nephrotic syndrome, and the others show mild to moderate proteinuria. In this study, we intended to figure out the clinical course, treatments and possible prognostic factors of FSGS in adults. METHODS: We retrospectively reviewed the clinical characteristics of 54 adult patients diagnosed as primary focal segmental glomerulo-sclerosis(FSGS) by renal biopsy in Chungnam National University Hospital. from Dec. 1989 to Jan. 2000. RESULTS: Mean age of 54 patients was 36.8+/-24.6 (15-75) years. Male to female ratio of them was 1.08 : 1. Thirty one patients(57%) of them were manifested as nephrotic syndrome and their male to female ratio was 1.4 : 1. In all of 54 primary FSGS patients, generalized edema(59%), microscopic hematuria (44%), hypertension(39%) and azotemia(15%) were noted as clinical manifestations at the time of diagnosis. Median duration of follow-up was 48.7(6-123) months. Except generalized edema, serum cholesterol and albumin level, and 24 hour urine protein excretion, there was no significant difference in hypertension, hematuria, serum creatinine, duration of follow- up and progression to chronic renal failure between 31 nephrotic and 23 non-nephrotic patients. Sixteen (52%) of 31 nephrotic patients with primary FSGS showed complete remission. Seven(22%) of them showed partial remission and eight(26%) of them showed no response after 8 weeks of first steroid treatment. No response group of primary FSGS nephrotic patients progressed significantly more to chronic renal failure than remission group including complete or partial remission patients. No response groups showed significantly higher serum creatinine, lower creatinine clearance, higher degree of glomerular global sclerosis and interstitial fibrosis than remission group at the time of diagnosis. Eleven patients(8 nephrotic and 3 non-nephrotic patients) of 54 primary FSGS patients progressed to CRF during follow-up period, and they showed significantly lower creatinine clearance and higher degree of global glomerular sclerosis at the time of diagnosis than normal renal function maintaining group. CONCLUSION: It is speculated that initial renal function and degree of global glomerular sclerosis at the time of diagnosis in primary FSGS patients, and additively response to initial steroid therapy and the degree of interstitial fibrosis at the time of diagnosis in nephrotic primary FSGS patients are thought to be significant long-term prognostic factors.

The effect of long-term corticosterild treatment on height velocity in childhood

The auther analysed 29 patients who were treated with prednisolone, including nephrotic syndrome (13 cases), acute leukemia(10 cases), bronchial asthma (2 cases), juvenile rheumatoid arthritis (2 cases) and ulcerative colitis (1 cases) patients in childhood, after remission induction, pro9longed alternate or interrupted treatment was used. The auther observed patterns of height velocity of these patients from 1.6 to 10.3 years. The results were as follow 1) Of the total 29 patients who were treated with prednisolone, most of them showed normal height velocity within the percentile channels, but 4 cases (13.8%) of them showed delay in height velocity. 2) The 4 cases who delayed in height velocity, including 2 cases of bronchial asthma and 2 cases of juvenile rheumatoid arthritis patients, received prednisolone daily about 0.4~1.5mg/kg from 3 to 6 years alternatively or interruptedly. Height velocity was decreased from bet ween 3 and 25 percentile channel at first to below 3 percentile channel at last contiously. 3) In these 4 cases, height velocity was inversely proportion to dosage of prednisolone.

Therapeutic Effect of Corticosteroid on Children's Primary Nephrotic Syndrome and Early Prediction / 昆明医科大学学报

This report is intended to the clinical data of 64 cases of children′s primary nephrotic syndrome (CPNS). We compared the patien′s age, CPNS course, selection of proteinuria, hematuria, renal function and clinical type with therapeutic effect of corticosteroid and predicted its early sensitivity.We found that ① To the patient with nephrotic syndrom-simple type, non-hematuria or normal renal function, corticostroid treatment was well effective (P0.05).

Onychomycosis in the Patients with Connective Tissue Disease Treated with Corticosteroid / 대한피부과학회지

We examined several kinds of immunocompromied patients for onyr,homycosis. The incidence of onychomycosis in the patients with connective tissue disease treated with corticosteroid was significantly higher than the incidences in the patients with solid tumors trcated with chemotherapeutic agents, in the patients with leukernia (WBC<1, 000/cm3), and in the control patients. Although conventional corticosteroid trcatment do not disseminate the superficial fungal infection, it may perpetuate the low grade dermatophytosis.