Beckwith-Wiedemann Syndrome (BWS) is a rare congenital overgrowth disorder due to alterations in specific genes in chromosome 11p15. It has a variable clinical picture. Infants may exhibit a combination of the following characteristics: macroglossia, macrosomia, abdominal wall defects, ear creases or posterior helical pits, hypoglycemia, polyhydramnios and prematurity. Presented is a case of a 24-year-old gravida 3 para 2 (2002) who manifested with preterm labor and polyhydramnios. She delivered a preterm live baby girl who was diagnosed to have Beckwith-Wiedemann syndrome. The rarity of this condition, as well as the significant maternal and perinatal complications associated with it, is discussed in this paper.

Human ; Female ; Adult ; Infant Newborn ; Pregnancy ; Congenital Macroglossia ; Macroglossia ; Beckwith-wiedemann Syndrome ; Polyhydramnios ; Abdominal Wall ; Fetal Macrosomia ; Hypertrophy ; Chromosomes ; Hypoglycemia

Swyer Syndrome is a pure form of gonadal dysgenesis that although rare, should not be disregarded in the differential diagnosis of patients who present with primary amenorrhea and abdominopelvic mass. The dysgenetic gonads fail to produce antimullerian hormone in an individual with Swyer Syndrome who is genetically male, resulting in feminization and absence of virilization. Phenotypically female, they usually seek consult at a later time during their teenage years due to primary amenorrhea. Our index patient consulted due to a large abdominopelvic mass and primary amenorrhea. Hormonal assay showed a hypergonadotropic hypogonadism endocrinologic milieu, and on karyotyping, showed a genetically male individual. This paper shall discuss an in-depth pre-operative, surgical and post-operative management of patients diagnosed with Swyer Syndrome.

Human ; Female ; Adolescent ; Anti-mullerian Hormone ; Amenorrhea ; Feminization ; Diagnosis, Differential ; Gonadal Dysgenesis, 46,xy ; Turner Syndrome ; Gonadal Dysgenesis ; Karyotyping ; Virilism ; Hypogonadism ; Gonads

Polycystic ovary syndrome (PCOS) is a heterogenous condition with signs and symptoms including menstrual dysfunction, weight disorders, hirsutism, diabetes mellitus, dyslipidemia and metabolic disorders. As such, diagnosis is controversial and differs between countries and communities. This study was done to identify and construct a registry of women with polycystic ovary syndrome being seen at the Out-Patient Department of Philippine General Hospital and obtain their demographic and anthropometric characteristics as well as their hormonal profile to be able to examine the best available evidence on the diagnosis and clinical management of PCOS in the Philippine setting. Results of the study showed comparable baseline characteristics and hormonal profile among Filipino women with PCOS. These data suggest that diagnostic and therapeutic modalities applied in other groups of women may be applicable and cost-effective in the Philippine setting.
Human ; Female ; Adult ; Young Adult ; POLYCYSTIC OVARY SYNDROME ;

Background: The RxBox 2 Model 2 is a portable device developed by the National TeleHealth Center capable of measuring various physiologic signals including fetal heart beat and uterine contractions, making it able to act as a cardiotocogram. The first model of the RxBox 2 was used in an observational cross-sectional study and was noted to have a low accuracy compared with the standard cardiotocogram. An adjustment was made with the objective of improving the sensitivity and specificity. Objective: The objective of this diagnostic cross-sectional study is to validate the RxBox 2 Model 2 by comparing its sensitivity and specificity with that of the standard cardiotocogram in detecting Category II traces. Results: The results of this study exhibited an improvement in the sensitivity (77% versus 60%) and specificity (71% versus 61%). In terms of accuracy, there is no significant difference between the high risk and non-high risk groups. These contribute to the validity of RxBox 2 Model 2 as an acceptable screening tool. Recommendation Further studies may still be done to improve the correlation of each component of the trace to that of the standard cardiotocogram. Detailed analysis of the interpretations with corresponding interventions and perinatal outcomes may aid in validating the device.
Telemedicine ; Perinatology ; Perinatal Care ; Maternal Health

BACKGROUND: Pre-induction of labor cervical ripening increases success of labor induction when there is unfavorable cervix. Evening primrose oil soft gel capsule contains linoleic and gamma-linolenic acid, which are precursors of prostaglandins E1 andE2.
OBJECTIVE: To measure the effectiveness of evening primrose oil capsule as a cervical ripening agent by measuring the Bishop score before and 4 hours after intravaginal insertion of six capsules.
METHODS: A quasi-experimental cross-sectional study was conducted from the period of May to July 2016 involving labor induction patients with a Bishop score ?4, an intact amniotic sac and a Biophysical profile score of 10/10 or 8/8.
RESULTS: Thirteen patients had an average age of 27±6 years, and a mean age of gestation of 40±1 weeks. Seven patients (54%) were nulliparous, 2 (15%) were primiparous and 4 (31%) were multiparous. Seven patients (54%) had hypertension, 1 (8%) had diabetes mellitus, 5 (38%) had post-term pregnancies. A paired t-test was done to check for statistically significant changes in the Bishop score. Change in the Bishop score from baseline to 4 hours after insertion of evening primrose oil capsules was statistically significant (p=0.001). Eleven patients (85%) had improvement in the Bishop score after 4 hours, 4 (31%) of which had a clinically significant change in the Bishop score (?4). Specifically, there were statistically significant changes in the dilatation (p=0.027), effacement (p=0.006) and consistency (p=0.002). The mean birth weight of deliveries was 3192±351 grams. Nine patients (69%) underwent primary low segment cesarean section, six (46%) of which for nonreassuring fetal status, 2 (15%) for arrest in cervical dilatation, and 1 (8%) for intraamnionic infection. Four patients (31%) successfully delivered vaginally.
CONCLUSION: Results showed a positive effect on the Bishop score during cervical ripening although further studies are needed to establish direct correlation.

Human ; Female ; Alprostadil ; Birth Weight ; Cervical Ripening ; Cervix Uteri ; Cesarean Section ; Diabetes Mellitus ; Dilatation ; Fetal Distress ; Hypertension ; Labor Stage, First ; Pregnancy

Background: The prone position has been seen to benefit patients experiencing acute respiratory distress syndrome. However, performing this position in pregnant patients has been difficult and raises safety concerns. Objective: The current study aimed to test the use of a supportive pillow (Prone Pillow for Pregnant Patients or 4P) to address concerns regarding pregnant patients in prone position. Methods: The study prospectively evaluated the use of the prone pillow for patient comfort and usability among healthcare workers with qualitative and quantitative measures. Results: A total of three patients were recruited alongside 16 healthcare workers assisting pregnant patients to the prone position. Overall, awake pregnant patients found the pillow to be comfortable while healthcare workers perceived the pillow to be useful in improving quality of care among awake and intubated pregnant patients. Conclusion The 4P is a potentially useful and beneficial product in placing pregnant patients in the prone position during episodes of acute respiratory distress. However, due to the limited sample size, more clinical trials are needed to evaluate the impact of this innovation in improving patient and healthcare worker safety.
Covid-19