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

Objectives: To determine the beliefs and attitudes towards diabetes of rural health care providers in Aklan, Philippines using the Diabetes Attitude Scale 3 (DAS-3) and to determine factors associated with it. Methodology: This is a cross-sectional analytic survey. A total of 339 health care providers were given self-administered DAS-3 questionnaires. Additional data gathered included their age, highest educational attainment, position, municipality class, diabetes as a co-morbidity, attendance to diabetes classes, and family history of diabetes. Results: Rural health care providers showed an overall mean positive attitude score of 3.5 using the DAS-3 questionnaire. In decreasing order, mean scores of participants according to subscale is as follows: “Need for Special Training in Education” (4.13) >“Autonomy of diabetes for patients” (3.70) >“Psychosocial Impact of Diabetes” (3.60) >“Value of Tight Glucose Control” (3.14) and “Seriousness of Type 2 Diabetes” (3.09). Physicians have the highest mean scores consistently in all subscales compared to other health care providers. Among the different factors considered, educational attainment (p=0.005) and work position (p=<0.001) were found out to affect attitude score of health care providers. Conclusions This study has shown that the majority of the rural health care providers believe in the need for special training of healthcare providers, psychosocial impact of diabetes and patient autonomy in diabetes self-care. However, the majority still do not strongly believe in the seriousness of diabetes and the benefits of tight sugar control. Educational attainment and work position are the consistent factors that impact diabetes-related attitude; therefore, the need to strengthen continuous medical education among health care providers
Diabetes Mellitus, Type 2 ; Attitude ; Rural Health Services

Objectives: This study aims to determine the role of HbA1c level during first trimester in predicting gestational diabetes mellitus in Filipino non-diabetic women. Hence, to identify those will be at increased risk of its adverse maternal and perinatal outcomes, and who will benefit from early intervention. This will aid in preventing maternal and perinatal morbidity and mortality and reducing health care cost by avoiding strategies which can result in false positive cases. Methodology: A cross-sectional study conducted in a tertiary hospital in the Philippines. Seventy-one Filipino pregnant women were included in the final analysis. HbA1c levels were taken during the first trimester and routine screening of gestational diabetes mellitus (GDM) utilizing 75 grams OGTT during 24th-28th weeks age of gestation. Binary logistic regression modeling was performed to determine if HbA1c was a predictor of gestational diabetes mellitus. The calculated median for Hba1c was then utilized as a threshold value to predict GDM. Odds ratio, relative risk and corresponding 95% confidence intervals from binary logistic regression were computed to determine the association of variables. Results: In this study the prevalence rate of GDM is 38%. It showed that first-trimester HbA1c level is not a predictor of GDM and adverse maternal and perinatal outcomes. However, in utilizing an HbA1c threshold of ≥ 5.2%, there is a two-fold increase risk of developing hypertensive disorders, requiring insulin during pregnancy, and macrosomic newborns and a four-fold increase risk of having large for gestational age newborns. It has a positive predictive value (PPV) of only 16%. However, it has a high negative predictive value (NPV) of 88% therefore it can be used to rule out risk of GDM as early as in the first trimester. Conclusion The association of HbA1c level and the occurrence of GDM was not observed in this study. However, by using an HbA1c threshold of ≥ 5.2%, as opposed to the standard reference range for diagnosing type 2 diabetes mellitus and gestational diabetes mellitus in Caucasians, the relative risk of developing GDM in 24th-28th weeks AOG is 1.26 (0.6865, 2.3242).
Diabetes, Gestational ; Pregnancy ; Insulin

Introduction: In 2017, the American Thyroid Association (ATA) revised their guidelines that when trimester and assay specific TSH reference intervals is unavailable, a TSH cut-off of 4.0 mIU/L replacing the previously recommended 2.5-3.0 mIU/L may be used to define maternal hypothyroidism. It states that levothyroxine treatment is considered if anti-TPO levels are elevated and TSH is between 2.5 mIU/L and the trimester-specific upper limit. These recommendations are a major departure from our current practice because the local TSH trimester-specific reference interval is not applicable due to a different assay used and the anti-TPO result is not readily available. In this population-based study, we aimed to determine and compare the maternal and perinatal outcomes of pregnant women who are euthyroid (TSH 0.3-2.4 mIU/L) versus those with subclinical hypothyroidism at different TSH cut-off levels (TSH 2.5-4.0 mIU/L, TSH 4.0-10.0 mIU/L) treated with levothyroxine. Methods: This is a single-center, prospective cohort study conducted at Chong Hua Hospital, Cebu City from September 2017 to September 2018 where a total of 505 pregnant women qualified. The cohort was divided into three groups: the euthyroid group of 404 women with TSH 0.3-2.4 mIU/L as control subjects; 101 women with subclinical hypothyroidism treated with levothyroxine further subdivided into TSH level 2.5-4.0 mIU/L (81 women) and TSH level >4.0-10.0 mIU/L (20 women). These patients were followed through to delivery to document and compare the maternal and perinatal outcomes versus euthyroid patients. Results: There was no statistically significant difference among the group of patients with subclinical hypothyroidism treated with levothyroxine versus euthyroid patients in documented complications of pregnancy, such as GDM, gestational HPN, pre-eclampsia, PROM, low APGAR score and fetal distress. However, in patients with baseline TSH 2.5-4.0 mIU/L there was preterm delivery in six (7.41%) patients, post-term delivery in two (2.5%) patients, with seven (8.6%) small for gestational age (SGA) infants and two (2.5%) large for gestational age (LGA) infants. In patients with baseline TSH > 4.0-10.0 mIU/L, preterm delivery occurred in two (10%) patients. In secondary analysis adjusted for age and parity at enrolment, pregnant women treated with levothyroxine at baseline TSH 2.5-4.0 mIU/L and TSH > 4.0-10.0 mIU/L versus the untreated women with TSH < 2.5 mIU/L showed no difference in the maternal and perinatal outcomes of pregnancy measured. Conclusion This study has shown a 12.5% prevalence of subclinical hypothyroidism in our setting. There was no difference in the maternal and perinatal outcomes of pregnant patients who are euthyroid versus those with subclinical hypothyroidism treated with levothyroxine at a TSH threshold of 2.5-4.0 mIU/L and >4.0-10.0 mIU/L. These findings support the view that levothyroxine treatment in pregnant women with subclinical hypothyroidism at a TSH cut-off of 2.5 mIU/L shows no harmful effects.
Pregnancy ; Treatment Outcome

Introduction: Malnutrition is a widespread condition that impacts millions of people across the world annually. The World Health Organization defines malnutrition as the deficiency, excess or imbalance in a person’s intake of energy and/or nutrients. It has been highly prevalent in hospitalized patients and is often overlooked as it continues to be an unrecognized problem. It is also associated with increased risk of complications, higher mortality rate, longer hospital stay, and higher hospitalization costs. The researchers aim to determine in-patients’ nutritional status using the Subjective Global Assessment (SGA) and their outcomes in a tertiary hospital. Methods: A cross-sectional study was conducted in a tertiary hospital in Cebu city for three months. A total of 453 patients were selected through simple random sampling from those assessed to be at risk for malnutrition. The nutritional status was obtained using the SGA tool and correlated with the demographic, nutritional and clinical profiles. Results: In the study, the mean age was 54 years with a female predominance (54.1%). A larger percentage belonged to the obese 1 category (34.9%). Fifty-eight percent had moderate risk for malnutrition and 57.2% had an SGA grade of B which corresponded to moderate malnutrition. The most common reason of admission was due to respiratory causes (25%) and the leading comorbidities were hypertension (48.79%), diabetes mellitus (36.42%) and chronic kidney disease (10.38%). The mean number of hospital stay was 6.8 days (98.9%) were discharged improved with a mortality rate of only one percent. The following factors had a positive relationship with the SGA grade: those in the older age group (p=0.000), those with more comorbidities (p=0.000), patients with diabetes (p=0.027) and chronic kidney diseases (0.001), those with higher nutritional risk on screening (p=0.000), those with pulmonologic (p=0.035) and oncologic cases (p=0.012) upon admission. The study results showed that the higher the SGA grade, the longer hospital stay (p=0.000). Conclusion In this study, the prevalence of malnutrition was 86% wherein 57.2% of the study population had moderate malnutrition, 28.9% had severe malnutrition and 13.9% had no malnutrition. The degree of malnutrition was associated with longer hospital stay, but not with mortality.
Malnutrition ; Nutritional Status

Introduction: Obesity remains to be a public health concern across the globe. Studies have established that obesity plays an important role in the pathogenesis and progression of cardiovascular diseases such as hypertension and coronary artery disease. Evidence suggesting the link between obesity and decompensation of heart failure is only just emerging. General Objective: Determine the associations between body mass index (BMI) outcomes of severity and mortality among adult Filipino patients admitted with decompensated heart failure. Study Design: Retrospective Cohort study design. Study Setting: Chong Hua Hospital, a tertiary hospital in Cebu City, Philippines Study Population: All adult patients with a diagnosis of Decompensated heart failure who were admitted in the hospital from 2015 to 2019. Main Outcome Measure: Determine association of BMI using Asian cut-offs and Outcomes of patients admitted for decompensated heart failure. Results: A total of 356 patients were admitted for acute decompensated heart failure from year 2015-2019. Majority of the patients were in the obese category 1 (28.93%) and predominantly were classified as NYHA Class III heart failure. There was no significant association between BMI and outcomes among patients with decompensated heart failure. Furthermore, it was noted that the pro-BNP values were higher in patients with lower BMI categories than in the overweight and obese categories. Conclusion Obesity, while increasing the chance of developing heart failure (HF), appears to protect people who have already been diagnosed with HF (the "obesity paradox"). This study, although not reaching statistical significance, showed that patients with lower BMI admitted for heart failure decompensation had increased use of positive pressure ventilation and higher mortality rates compared to obese patients. A larger sample size may be needed to show such association. Furthermore, patients in lower BMI category had higher pro-BNP values than their counterparts consistent with previous literature.

Introduction: Hormone-producing adrenal tumors, adrenal carcinomas and other adrenal diseases can be potentially cured with adrenalectomy. In the local setting, studies are often limited by a small sample size and inadequate patient data. This study aimed to determine the clinical and histopathologic characteristics and perioperative outcomes of patients who underwent adrenalectomy. Methods: This is a retrospective chart review study from January 2007 to June 2017 in a tertiary hospital in Cebu City, Philippines. Clinical profiles, type of surgery, and operative outcomes were determined. Comparative analysis of clinical profile, histopathologic features, and surgical outcome was done. Descriptive as well as appropriate inferential statistical methods were used to analyze the data. Results: A total of 31 patients who underwent adrenalectomy were included with the mean age of 45.7 [SD=17.1] years old and a 1:3 male to female distribution. The distribution of tumors was as follows: hormone-producing adrenal tumor (74.2%), malignant adrenal tumors (12.9%), and other benign lesions (12.9%). Among patients with hormoneproducing tumors, 39.1% had catecholamine excess, 34.8% had aldosterone excess, and 26.1% had cortisol excess. Hormone-producing adrenal tumors were common at age 20 to 40 years old while malignant tumors were more common among those above 40 years old (p-value=0.023). Stage 3 hypertension (p-value=0.010) and improvement of hypertension postoperatively (p-value=0.046) were more common among hormone-producing tumors. On the other hand, large tumor size (>4cm) (p-value=0.011), blood loss needing blood transfusion (p-value=0.001), prolonged operation (p-value=0.046), and longer hospital stay (p-value=0.002) were common among those with malignant tumors. Open adrenalectomy was associated with significant blood loss needing transfusion (p-value=0.001) and prolonged hospital stay (p-value=0.024). Conclusion Hormone-producing adrenal tumors with secondary hypertension are the most common pathology among patients who underwent adrenalectomy. They are usually seen among patients less than 40 years old, with smaller tumor size, and frequently present with higher blood pressures that improve following adrenalectomy. In contrast, adrenal carcinomas are more common among patients above 40 years old and have larger tumor size. More often they have prolonged operation time, greater blood loss, and longer hospital stay. Patients who underwent open adrenalectomy had more blood loss and had a longer hospital stay than those who underwent laparoscopic surgery.
Adrenalectomy

Introduction: Metformin has known mechanistic benefits on COVID-19 infection due to its anti-inflammatory effects and its action on the ACE2 receptor. However, some physicians are reluctant to use it in hypoxemic patients due to potential lactic acidosis. The primary purpose of the study was to determine whether metformin use is associated with survival. We also wanted to determine whether there is a difference in outcomes in subcategories of metformin use, whether at home, in-hospital, or mixed home/in-hospital use. Objectives: This study aimed to determine an association between metformin use and mortality among patients with type 2 diabetes mellitus hospitalized for COVID-19 infection. Methodology: This was a cross-sectional analysis of data acquired from the COVID-19 database of two tertiary hospitals in Cebu from March 1, 2020, to September 30, 2020. Hospitalized adult Filipino patients with type 2 diabetes mellitus who tested positive for COVID-19 via RT-PCR were included and categorized as either metformin users or metformin non-users. Results: We included 355 patients with type 2 diabetes mellitus in the study, 186 (52.4%) were metformin users. They were further categorized into home metformin users (n=109, 30.7%), in-hospital metformin users (n=40, 11.3%), and mixed home/in-hospital metformin users (n=37, 10.4%). Metformin use was associated with a lower risk for mortality compared to non-users (p=0.001; OR=0.424). In-hospital and mixed home/in-hospital metformin users were associated with lower mortality odds than non-users (p=0.002; OR=0.103 and p=0.005; OR 0.173, respectively). The lower risk for mortality was noted in metformin, regardless of dosage, from 500 mg to 2 g daily (p=0.002). Daily dose between ≥1000 mg to <2000 mg was associated with the greatest benefit on mortality (p≤0.001; OR=0.252). The survival distributions between metformin users and non-users were statistically different, showing inequality in survival (χ2=5.67, p=0.017). Conclusion Metformin was associated with a lower risk for mortality in persons with type 2 diabetes mellitus hospitalized for COVID-19 disease compared to non-users. Use of metformin in-hospital, and mixed home/in-hospital metformin use, was also associated with decreased risk for mortality. The greatest benefit seen was in those taking a daily dose of ≥1000 mg to <2000 mg.
Metformin ; Diabetes Mellitus ; COVID-19 ; Mortality