BACKGROUND AND SIGNIFICANCE: McCune Albright Syndrome (MAS) is a rare disorder characterized by the clinical triad of precocious puberty, polyostotic fibrous dysplasia of the bones and café-au-lait spots. Prevalence is estimated at 1/100,000-1/1,000,000. We report a case of polyostotic fibrous dysplasia in a patient with McCune Albright Syndrome who had symptomatic relief of hip pains and non-recurrence of stress fractures in a dysplastic right hip bone following treatment with loading intravenous pamidronate followed by an oral alendronate for almost a year, as an off-label indication. While intravenous bisphosphonates have been well-recognized in the treatment of fibrous dysplasia, only case reports are available to support its utility.
CASE REPORT: We report an 18 year old female with leg length discrepancy following repeated episodes of hip fracture for the past six years. She was referred to the Philippine General Hospital for recurrent severe leg pains which occurred usually at menstrual mid-cycle. This condition was associated with lateral bowing of the proximal part of the right thigh, widening of the right hip region, and shortening of the right lower limb also known as Shepherd's Crook deformity. She also had café-au-lait spots at the back of her left legs and buttocks. Skeletal survey showed radiolucent medullary expansile lytic lesions with ground glass appearance of the right femur, tibia, fibula, humerus, scapula, pubis, ischium, carpal and metacarpal bones. Patient was noted to have short stature with height of 142 cm. Arm span was 139 cms, upper body segment (crown to the coccyx) was 70cms while lower body segment (coccyx to heel left foot) was 72 cms. The difference between the left and right leg was 7 cms. Mean parental height was 160 cm. She was then referred to the Endocrinology service of this institution for evaluation of the short stature and associated endocrinopathies. On review, she had adrenarche at 8 years old followed by menarche at 10 years old. She had no goiter. She had no cushingoid features. Patient had irregular menstrual cycles with oligomenorrhea (cycle: 60-180 days). Breast development and pubic hair were staged Tanner 5. In the approach to short stature where height age is less than either the bone age or chronological age, constitutional
dwarfism, hypothyroidism, growth hormone deficiency and fibrous dyplasia must be ruled out. Constitutional dwarfism was ruled out with a midparental height of 160 cm. A normal free thyroxine (17, normal: 9-23 pmol/L), thyroid stimulating hormone (2.4, normal: 0.25-4 ulU/ml) ruled out hypothyroidism, and a normal IGF-1 (103, normal: 91-223 nmol/L) ruled out growth hormone deficiency. This left us with the consideration of fibrous dysplasia of the bone which was consistent with the earlier radiographic findings. The combination ofpolyostotic fibrous dysplasia and café au lait spots led to the impression of McCune Albright Syndrome. The most common endocrinopathy associated with McCune Albright Syndrome is a peripheral hyperfunctioning ovaries which also harbors the G-protein mutation. This was evident in our case with a high estrogen (655.8, normal: 50-250 pg/ml) and suppressed LH (1.2, normal: 1.5-5 pg/ml) and FSH (3, normal 3.5-12.5 pg/ml) with a transrectal ultrasound finding of a 2.6 x 1.7 x 1.6 cm cyst at the right ovary. This precipitated the precocious puberty andearly closure of the epiphyseal plates resulting to short stature. To screen for other endocrinopathies, a 24 hour urine free cortisol (44, 20-90 ug/day), serum prolactin (15ng/ml, normal: 0-30ng/ml), free thyroxine (17, normal: 9-23.2 pmol/L) and parathyroid hormone (13.9, normal 10-65pg/ml) was documented and ruled out associated hypercortisolemia, prolactinoma, hyperthyroidism and hyperparathyroidism respectively. The patient had no history of change in shoe size, and no coarsening of facial features that was suggestive ofacromegaly.                                                                                                                                          
TREATMENT: Pat ient underwent bone graf t ing and osteotomy to correct the shepherd's crook deformity. Three cycles of intravenous pamidronate infusion in three consecutive days was given prior to the operation. Postoperatively, patient tolerated the procedure and was discharged after three days.
OUTCOME: Shepherd's crook deformity was successfully corrected. Patient still had limp but with no pain and no new fractures for almost a year already. At present she is maintained on alendronate 70mg 1 tab once a week and calcium 1 gram per day.
CONCLUSION: We repor t a case of McCune Albright Syndrome presenting with bone deformity which was later diagnosed to be fibrous dysplasia with polyostotic involvement, and was successfully treated with initial intravenous bi sphosphonates maintained on oral bisphosphonates following a surgical procedure to correct the shepherd's crook deformity. At present, she has had no new fractures.

Human ; Female ; Young Adult ; Adolescent ; Coccyx ; Cysts ; Dwarfism ; Estrogens ; Fibrous Dysplasia, Polyostotic ; Fractures, Stress ; Growth Hormone ; Hydrocortisone ; Hyperparathyroidism ; Hyperthyroidism ; Hypothyroidism ; Mutation ; Ovary ; Parathyroid Hormone ; Thyrotropin ; Thyroxine

Background: Hyperglycemia occurs in 60% of acute stroke patients, 12-52% of which maybe previously undiagnosed with diabetes. A retrospective study by Gacutan showed 21% admission hyperglycemia at the Philippine General Hospital. Screening for glucose disorders among hyperglycemic stroke patients provides a venue for secondary prevention. There are no current guidelines on screening for post-stroke hyperglycemia by the Stroke Society of the Philippines. Objective: To determine the prevalence and outcomes of unrecognized diabetes and prediabetes among acute stroke patients. Secondarily, we aim to determine the clinical and biochemical predictors of unrecognized/newly diagnosed diabetes mellitus among the acute stroke patients. Methodology: Cross-sectional prospective study. Acute stroke patients admitted from January to December, 2008 with admission hyperglycemia were included. Random blood sugar, glycosylated hemoglobin & the National Institute of Health Stroke Scale (NIHSS) were done on admission. All were followed up six weeks post-discharge for an FBS and 75-grams OGTT except those discharged with anti-diabetes medications who were considered newly-diagnosed diabetes. Outcomes: Glucose disorders were classified at six weeks post-discharge. Mortality was measured at six weeks and 12 weeks. Functional outcomes using the Modified Rankin Score & the Barthel’s Index were measured at the OPD 6-weeks postdischarge. Results: There were 504 stroke patients of which 178(35%) had admission hyperglycemia. Majority were newly diagnosed diabetes 32.7%, stress hyperglycemia 29.56%, impaired glucose tolerance, (IGT) 13.8%, impaired fasting blood sugar, (IFG) 8.8%, combined IGT and IFG 3.7%, previous diabetes 11.32%. Age, body mass index, alcohol abuse, smoking, hypertension, nosocomial infections, acute coronary syndrome were associated with diabetes. No association was noted with diabetes and atrial fibrillation. By Kruskal-Wallis test, admission RBS and HbA1c were higher among diabetics compared to prediabetics and stress hyperglycemics. Triglycerides was higher and HDL was lower among diabetic versus the stress hyperglycemia. No trends were noted for total cholesterol and LDL cholesterol across groups. On multiple logistic regression, age (OR: 1.06, p0.07), smoking (OR: 9.81, p0.05) and Hba1c (OR:11.39, p<0.01) were the predictors for newly diagnosed diabetes classification among the acute stroke patients. Higher Modified Rankin and lower Barthel’s index were seen among diabetics compared to the other groups measured on follow-up. Mortality at six weeks was higher among diabetics (OR: 2.34, p<0.01) compared to non-diabetics. A trend towards higher mortality at 12 weeks was also noted among diabetics (OR: 1.81, p0.063) compared to other groups. Conclusion: Admission hyperglycemia was seen in 35% of admitted stroke patients, majority of which were unrecognized diabetes. By screening for post-stroke hyperglycemia, we identified 59.1% (32.7% unrecognized diabetes; 26.4% prediabetes) patients eligible for treatment and secondary prevention. Short-term mortality was higher among acute stroke patients with diabetes. Age, smoking and admission HbA1c were the clinical and biochemical predictors of a newly diagnosed diabetes mellitus

Rationale: Neurocysticercosis is a neglected tropical disease caused by the larvae of Taenia solium and may occur in immunocompetent people. We report a case of diffuse parenchymal neurocysticercosis mimicking tuberculous meningitis in an immunocompetent patient. Patient concerns: A 24-year-old immunocompetent woman presented with serial focal to generalized seizure, left body weakness, left abducens palsy, chronic cough, and body weight loss. She came from an area where she was unlikely to have contact with Taenia solium. Diagnosis: Diffuse parenchymal neurocysticercosis. Intervention: Valproic acid, dexamethasone and 15 months of albendazole therapy. Outcome: Complete recovery without any neurological sequelae. Lessons: Prolonged anthelminthic treatment may benefit neurocysticercosis patients with multiple cerebral parenchymal lesions.

Introduction: Atopic dermatitis (AD) is a complex disease with an interplay of genetic and environmental factors. In the United States, AD affects 10.7% of children andyc 7.2% of adults. Similarly in the Philippines, the prevalence of AD is 12.7% in the under 18 population, and 2% in the over 18 population. While AD affects all ages, the burden of the disease is greater in the pediatric population. The pathogenesis of AD is multifactorial. Variations in genes responsible for epidermal barrier function, keratinocyte terminal differentiation, and the innate and adaptive immune responses have been linked to AD. A null mutation involving the filaggrin gene is the strongest known risk factor for AD. This mutation results in a loss of filaggrin (FLG) protein by at least 50%. Filaggrin breakdown products form part of the natural moisturizing factor (NMF) of the skin, which is essential in skin hydration. A decrease in NMF and an increase in transepidermal water loss (TEWL) are observed in AD patients with FLG mutation. The defective barrier in AD patients decreases skin defenses against irritation and allergen penetration. Exposure to certain environmental chemicals like formaldehyde may worsen this barrier. This may lead to increased skin permeability to aeroallergens that leads to dermatitis in sensitized patients. Barrier defects may also play a role in epicutaneous sensitization and the subsequent development of other atopic conditions, such as bronchial asthma and allergic rhinitis. The Philippine Dermatological Society (PDS) consensus on AD aims to provide a comprehensive guideline and evidence-based recommendations in the management of this condition, with consideration of cultural factors that are often encountered in the Philippine setting. These guidelines are intended to provide practitioners with an overview of the holistic approach in the management of AD, ameliorating the negative effects of the disease and improving overall quality of life.. Methodology: A group of 21 board-certified dermatologists from the Philippine Dermatological Society (PDS) convened to discuss aspects in the clinical management of AD. Database and literature search included the full-text articles of observational studies, randomized controlled clinical trials, and observational studies using the Cochrane library, PubMed, Hardin (for Philippine based studies) as well as data from the PDS health information system. The terms used in combinations from the literature included “atopic dermatitis”, “atopic eczema”, “emollients”, “topical corticosteroids”, “topical calcineurin inhibitors”, “anti-histamines” and “phototherapy”. A total of fifty (50) full text articles were reviewed and found applicable for the scope of the study. Articles were assessed using the modified Jadad scale, with score interpretations as follows: (5- excellent, 3- good, 1– poor). Consensus guidelines for AD from within and outside of the region were also reviewed, from the 2013 Asia-Pacific guidelines, 2014 Taiwanese Dermatological Association consensus, 2016 guidelines in the management of AD in Singapore, 2014 American Academy of Dermatology guidelines, and the 2020 Japanese guidelines for AD. From the literature review, proposed consensus statements were developed, and a Delphi survey was conducted over two separate virtual meetings. Individual dermatologists provided Likert Scoring (1- strongly disagree to 5- strongly agree) based on consensus statements. A consensus was deemed reached at mean scores of > 4.00, a near consensus at > 3.5, and no consensus at <3.5. Summary: AD is a chronic relapsing condition with a significant burden of disease, most commonly affecting the pediatric population. The PDS AD Consensus Guidelines summarizes the standards of therapy and the therapeutic ladder in the management of AD based on published clinical trials and literature review. While these modalities remain the cornerstone of therapy, an individualized approach is the key to the holistic management of an AD patient. Knowledge and awareness of frequently associated conditions, whether in the realm of food allergies, contact allergies, or secondary infections, is paramount. In addition to the standard therapeutic armamentarium, the physician must also consider cultural practices and be knowledgeable of alternative therapeutic options. Referral to a specialist is recommended for recalcitrant cases of AD, or when initiation of systemic immunosuppressive agents, phototherapy, or biologic agents is contemplated.