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

Brain ; physiology ; History, 20th Century ; Humans ; Sleep ; physiology ; Sleep Medicine Specialty ; history ; Sleep, REM ; physiology

Biomedical Research ; history ; France ; Hemagglutination Tests ; history ; History, 19th Century ; History, 20th Century ; Humans ; Philately

Purpose A new way in breast cancer detection, Dynamic Optical Breast Imaging (DOBI) was studied. Materials and Methods 52 patients receiving breast biopsy and DOBI were enrolled in this study. 19 were proved of breast cancer, 33 were benign. Results 94.8% of blue area in non-breast cancer lesions was found as wandering or diffusive pattern, while 68.42% breast cancer showed focal pattern. 86.46% of the curve signature of blue area in non-breast cancer lesions was wavy or flat, while 57.37% of breast cancer showed a steep decline. In 64.58% non-breast cancer, the curve of blue area was similar to that of non-blue area. 78.95% breast cancer had their curve different from that of non-blue area. The absolute value of amplitude (-5.77±2.13) of blue area in cancer was higher than that in non-cancer ( -3.34±0.87). The differences were all statistically significant(P<0.05). Conclusion The spatial and temporal characteristics of DOBI were of diagnostic and differential value for breast cancer. The absolute value of amplitude, over|-5|, also helped the diagnosis of breast cancer.

Objective: The aim of this study was to identify depression among HIV- infected patients attending Infectious Disease Clinic in University Malaya Medical Centre (UMMC). Methods: This is a cross sectional study on HIV- infected patients attending Infectious Disease Clinic, UMMC. Those who fulfilled inclusion criteria were recruited and interviewed. Socio- demographic characteristics and clinical conditions such as mode of transmission, year of diagnosis, CD4+ counts, drug treatment and clinical stage were collected. The patients were then subjected to self-administered questionnaires, Patient Health Questionnaire, (PHQ-9) and Hospital Anxiety & Depression Scale (HADS). Results: 89 patients were recruited. Scores from PHQ9 showed 32% of depression rate while scores from HADS showed 19% of depression rate among the respondents. Non-self financial supporter, non- alcoholic drinkers and females were more likely to be depressed (P<0.05). All clinical characteristics showed no statistical differences. Conclusion: The depression rate was lower compared to those from the studies in western countries. The risk factors for depression were different from those found in other studies as well.

Recent innovations in the treatment of schizophrenia reflect a growing trend towards community-based care. Malaysia had in the past few years attempted to deinstitutionalise mental patients in the mental hospitals. Therefore it is important to conduct research to compare the two groups of schizophrenia patients (community-based patients against chronic hospitalised patients) to ascertain if deinstitutionalisation has been beneficial. The main objective of the study was to compare levels of depression and function in community-based patients against chronic hospitalised patients as depression is prevalent among schizophrenia patients. This study was cross sectional in nature where data was collected from 51 inpatients in Hospital Bahagia Ulu Kinta (HBUK) and 23 community-based patients. Calgary Depression Scale for Schizophrenia (CDSS) and Global Assessment of Functioning scale (GAF) were the assessment tools used. Community-based patients were found to have significantly lower scores in the CDSS scale (1.96) as compared to chronic hospitalised patients (4.04); p < 0.01). They also showed higher functional capability between community-based and hospitalised patients respectively (74.04 vs 57.92) respectively. (p < 0.001). Community services appeared to be more effective than long stay in-patient services in preventing depression and promoting better functional levels.

Background: Post-traumatic seizure is a well-known and serious complication of traumatic brain injury (TBI). The incidence and risk factors vary among study populations. Very little data have been published concerning this in the Malaysian population. The aim of this study was to ascertain the risk factors for the development of early post-traumatic seizures among patients with TBI. Methods: This was a prospective observational study, carried out in Hospital Universiti Sains Malaysia, Kubang Kerian, Kelantan, under the Department of Neurosciences. A total of 157 patients, from all age groups, who were diagnosed with TBI were enrolled from June 2007 to December 2007. They were followed-up for 12 months until death or their first post-traumatic seizure. Survival analysis with Kaplan–Meier curves and Cox proportional hazards regression was performed. Results: A total of 11 (7.0%) of the patients developed early post-traumatic seizures. The risk factors for early post-traumatic seizures were young age (P = 0.021, 95% CI 0.806 to 0.982) and intubated patients (P = 0.029, 95% CI 1.194 to 25.913). The incidence of early post-traumatic seizures in the local population was 7.0%. Conclusion: The incidence of early post-traumatic seizures in the local population of Kelantan and Terengganu is comparable to the incidences reported elsewhere. Younger as well as intubated patients were at a higher risk of developing this condition. It may be necessary to give antiepileptic prophylaxis because any seizure could adversely affect morbidity and mortality. However, the study showed that antiepileptic drug was not beneficial in preventing late post-traumatic seizures, but may have a role in preventing early seizures.

BACKGROUND

Malnutrition is a component of frailty syndrome characterized by weakness, poor nutritional status, and reduced cognitive function. Frailty has been recognized to adversely affect post-cardiovascular surgery outcomes, with studies primarily in the elderly. To date, there are no published Philippine data on malnutrition and frailty in cardiac surgery patients.

METHODS

Malnutrition and frailty were assessed preoperatively in 111 adult patients undergoing cardiovascular surgery from October 2020 to February 2021. Nutrition Risk Screening (NRS) tool and Clinical Frailty Scale (CFS) were used for assessment, respectively. Their in-hospital postoperative outcomes were then observed.

RESULTS

There were 57 patients (51%) diagnosed with malnutrition, 26 (23%) of whom were also frail. Advanced age, rheumatic heart disease, heart failure, and chronic kidney disease were significantly higher in the malnutrition and frail group. After multivariate analysis, mortality rate (odds ratio [OR], 7.8; 95% confidence interval [CI], 1.45-41.91; P = 0.017), prolonged hospitalization (OR, 5.96; 95% CI, 2.14-16.53; P = 0.001), mechanical ventilation (OR, 7.56; 95% CI, 1.81-31.62; P = 0.006), and nosocomial infections (OR, 13.57; 95% CI, 4.41-41.76; P < 0.001) were found higher in patients with malnutrition and frailty.

CONCLUSION

Evaluation of nutrition and frailty status using NRS and CFS was helpful in predicting postoperative outcomes. With a significant number of this population having malnutrition and frailty, there is a need to strengthen clinical pathways on perioperative nutrition and rehabilitation with the possibility of improving cardiovascular surgery outcomes.

Malnutrition ; Frailty

INTRODUCTIONThe delivery of optimal and safe medical care is critical in healthcare. The traditional practice of "See one, do one and teach one" residency training programme is no longer acceptable.

MATERIALS AND METHODSIn the past, there was no structured residency training programme in our hospital. There were several cases of organ injuries from surgeries performed by the residents. In 2005, we conducted a pilot study to organise a structured teaching, education, surgical accreditation and assessment (TESA) residency programme for 15 residents in the Division of Obstetrics and Gynaecology, KK Women's and Children's Hospital. We performed a written questionnaire survey of the residents on the new programme and patients' expectation (n = 2926) as subjective outcomes in the 1-year follow-up. We also studied the complication rates of all minor and major surgeries performed by the residents in 2004 and 2005 as an objective outcome.

RESULTSAll the residents (n = 15) surveyed supported the TESA programme. Patients' expectation improved significantly from 71% in 2004 (n = 1559) to 83% in 2005 (n = 1367) (P = 0.03). There were 10,755 surgeries in 2004 and 10,558 surgeries in 2005 performed by our residents, with 6 cases (5.6%) of organ injuries in 2004 compared to 3 cases (2.8%) in 2005. This reduction was not statistically significant.

CONCLUSIONThe TESA residency programme in our hospital has an impact on the delivery of optimal and safe medical care while ensuring the training of residents to be competent specialists.

Accreditation ; Clinical Competence ; Delivery of Health Care ; standards ; Educational Measurement ; General Surgery ; education ; Humans ; Internship and Residency ; methods ; Pilot Projects ; Retrospective Studies ; Singapore ; Surveys and Questionnaires

INTRODUCTION: Patients referred for chest pain from primary care have increased, along with demand for outpatient cardiology consultations. We evaluated 'Triage Protocol' that implements standardised diagnostic testing prior to patients' first cardiology consultation. MATERIALS AND METHODS: Under the 'Triage Protocol', patients referred for chest pain were pretriaged using a standardised algorithm and subsequently referred for relevant functional diagnostic cardiology tests before their initial cardiology consultation. At the initial cardiology consultation scheduled by the primary care provider, test results were reviewed. A total of 522 triage patients (mean age 55 ± 13, male 53%) were frequency-matched by age, gender and risk cohort to 289 control patients (mean age: 56 ± 11, male: 52%). Pretest risk of coronary artery disease was defined according to a Modified Duke Clinical Score (MDCS) as low (<10), intermediate (10-20) and high (>20). The primary outcome was time from referral to diagnosis (days). Secondary outcomes were total visits, discharge rate at first consultation, patient cost and adverse cardiac outcomes. RESULTS: The 'Triage Protocol' resulted in shorter times from referral to diagnosis (46 vs 131 days; <0.0001) and fewer total visits (2.4 vs 3.0; <0.0001). However, triage patients in low-risk groups experienced higher costs due to increased testing (S$421 vs S$357, = 0.003). Adverse cardiac event rates under the 'Triage Protocol' indicated no compromise to patient safety (triage vs control: 0.57% vs 0.35%; = 1.000). CONCLUSION By implementing diagnostic cardiac testing prior to patients' first specialist consultation, the 'Triage Protocol' expedited diagnosis and reduced subsequent visits across all risk groups in ambulatory chest pain patients.
Algorithms ; Cardiology Service, Hospital ; Chest Pain ; therapy ; Clinical Protocols ; Female ; Humans ; Male ; Middle Aged ; Primary Health Care ; Treatment Outcome ; Triage ; methods