UNLABELLED Medical therapy for Cushing's syndrome due to bilateral macronodular adrenal hyperplasia (BMAH) is generally administered for a limited time before surgery. Aberrant receptors antagonists show inconsistent efficacy in the long run to prevent adrenalectomy. We present a patient with BMAH, treated for 10 years with low doses of ketoconazole to control cortisol secretion. A 48-year-old ...

A21-year-old pregnant woman, gravida 2 para 1, presented with hypertension and proteinuria at 20 weeks of gestation. She had a history of pre-eclampsia in her first pregnancy one year ago. During that pregnancy, at 39 weeks of gestation, she developed high blood pressure, proteinuria, and deranged liver function. She eventually delivered by emergency caesarean section following failed induction...

Deficiency of 21-hydroxylase (21-OHD) is present in 90–95% cases of congenital adrenal hyperplasia (CAH), an autosomal recessive disorder. CAH affects severely on the physical development and reproductive function. In monitoring the disease and evaluating treatment outcome, presentation of salt wasting, electrolyte disturbance, androgenism, cushingoid features, plasma levels of 17OHP, testoster...

The 2004 Newborn Screening Act has saved a lot of Filipinos from Congenital Adrenal Hyperplasia (CAH). The increase in the number of patients living with CAH challenges us to find the optimal steroid replacement therapy. In the Philippines, there is no locally published data on this. The objectives of the paper are to compare the effect of different hydrocortisone dosing regimen on the 17 hydro...

BACKGROUND The management of steroid resistant nephrotic syndrome (SRNS) is quite difficult in paediatric patients. Not only the remission is difficult but also these patients are at risk of progression to end stage renal disease (ESRD). The goal of treatment is either to achieve complete remission or even partial remission as it is the most important predictor of disease outcome. METHODS Thi...

OBJECTIVE We aimed to perform an observational study of age at loss of independent ambulation (LoA) and side-effect profiles associated with different glucocorticoid corticosteroid (GC) regimens in Duchenne muscular dystrophy (DMD). METHODS We studied 340 participants in the Cooperative International Neuromuscular Research Group Duchenne Natural History Study (CINRG-DNHS). LoA was defined as ...

BACKGROUND Published reports examining the efficacy and safety of glucocorticoids for preserving renal function in immunoglobulin A nephropathy (IgAN) have yielded conflicting results. To systematically evaluate the efficacy and safety of glucocorticoids agents for IgAN, we conducted a meta-analysis of the published randomized controlled trials (RCTs). METHODS MEDLINE, EMBASE, the Cochrane Li...

Congenital hypoplastic anemia is a rare disease characterized by a normochromic, normocytic anemia, a deficiency of erythroblasts in the marrow, and normal leukocyte and platelet counts. The disease usually is treated successfully with corticosteroid therapy; however, some patients become refractory to corticosteroids and must receive washed, packed red blood cell transfusions. The management a...

Abstract Introduction/Background Cushing’s syndrome (CS) is rare, with 2-5 cases per million annually, and only 10% of these occurring in children. disease (CD) (excessive adrenocorticotropic hormone [ACTH] secretion by a pituitary source leading to hypercortisolism) the most common organic etiology CS children (75-90%). Ectopic ACTH (EAS), contrast, accounts for less than 1%. Case Description ...

Abstract Introduction: Ectopic adrenocorticotrophic hormone (ACTH) producing Pancreatic Neuroendocrine Tumor (p-NET) are extremely rare with incidence of 1.2% and confer a poor prognosis (5-year survival <20%). Case Report: 31-year old female diabetes mellitus type 2 (DM-2) hypertension (HTN) was found to be severely hypokalemic on routine labs sent the Emergency Department for potassium...