Van Wyk-Grumbach Syndrome (VWGS) is presented as the usual triad of severe juvenile hypothyroidism (Hashimoto Thyroiditis is frequently the cause), isosexual precocious pseudopuberty and delayed bone age. With the treatment of the underlying thyroid hormone deficiency all of the features will be reversed dramatically. Here is the case described, with typical findings of hypothyroidism developed bilateral breast enlargement and menstruation. There was an intra-abdominal mass, in left lower abdomen about 7cm x 6 cm in diameter with soft consistency and irregular margin and surface. She had bilateral prominent calf muscle, generalized hypotonia & delayed relaxation of ankle jerk. Anthropometrically she was severely stunted and obese. Her height lies below MPH range. Pubertal developments in tanner stages were: thelarchae stage III, puberchae stage I (no adrenerchae). Serum TSH level was very high. Serum FSH and Estrogen level were also high for her age but, LH level was low. Her bone age was 3 years. Her pelvic sonogram showed enlarged uterus with bilateral multiple small adnexal cysts and a large multilocular cystic neoplasm in left adnexa. Treatment with Levothyroxine, her vaginal bleeding did not recur and ovarian cyst size decreased within 1 week and disappeared after 2 weeks. In conclusion, hypothyroidism should be evaluated who have precocious puberty with multicystic enlarged ovaries. It is important to keep in mind regarding complementary examinations and unnecessary surgical interventions to avoid parent as well as patient’s burden.
Background: Primary amenorrhoea is an uncommon but clinically important reproductive disorder that may result from anatomical, endocrine, ovarian, hypothalamic-pituitary or chromosomal abnormalities. Clinical examination, hormonal assessment, pelvic imaging and karyotyping are important for establishing the underlying diagnosis. Objective: To evaluate the association between clinical findings, hormonal profiles and karyotype abnormalities among patients presenting with primary amenorrhoea at a tertiary care hospital in Bangladesh. Methods: This prospective observational cross-sectional analytical study was conducted in the Gynaecology Outpatient Department of Bangladesh Medical University (BMU), Dhaka, Bangladesh, from December 2023 to January 2025. Forty-seven patients aged 13–35 years with primary amenorrhoea were included. Detailed clinical assessment included anthropometric measurements, evaluation of secondary sexual characteristics and external genital examination. Serum follicle-stimulating hormone (FSH), luteinizing hormone (LH), estradiol, prolactin and thyroid-stimulating hormone (TSH) were measured. Pelvic ultrasonography and peripheral blood karyotyping were performed. Associations between clinical characteristics, hormonal patterns and cytogenetic findings were analyzed using appropriate statistical tests. Results: The mean age was 19.9 ± 4.7 years with the 16–20-year age group predominating. Müllerian agenesis/MRKH syndrome and Turner syndrome/gonadal dysgenesis were the leading etiological categories. Hypergonadotropic hypogonadism was the most frequent hormonal pattern (38.3%). Abnormal karyotypes were identified in 31.9% of participants with 45,X Turner syndrome being the most frequent abnormality. Hypergonadotropic hypogonadism was significantly more common among participants with abnormal karyotypes than those with normal 46,XX karyotypes (86.7% vs. 28.1%, p<0.001). Short stature and poor breast development were also significantly associated with abnormal karyotype. Conclusion: Primary amenorrhoea has diverse anatomical, endocrine and chromosomal causes. Integrated clinical, hormonal, imaging and cytogenetic evaluation is important for accurate diagnosis and individualized management.
Background: Hypoxic ischemic encephalopathy (HIE) is a type of neonatal encephalopathy caused by systemic hypoxemia and/or reduced cerebral blood flow resulting from an acute peripartum or intrapartum asphyxia event. It is a condition that can cause significant mortality and long-term morbidity. Coagulopathy is one of the many consequences of compromised oxygen after perinatal asphyxia. International normalized ratio (INR) > 1.5, activated partial thromboplastin time (aPTT) > 50 seconds, prothrombin time (PT) >20s are used to evaluate coagulopathy in hypoxic ischemic encephalopathy neonates. A hematological system abnormality (20% each) is associated with perinatal asphyxia. Objective: To assess hematological and Coagulation function in hypoxic ischemic encephalopathy neonates. Materials and Methods: This cross-sectional type of descriptive study was conducted in the Department of Pediatrics at Rajshahi Medical College Hospital, Rajshahi over a period of 2years from July 2021 to June 2023. The study was conducted after obtaining ethical clearance from the Institutional Review Board (IRB) of Rajshahi Medical College and consent from the guardian of patients. Based on predefined eligibility criteria, a total number of 70 neonates with HIE stage II and III were included in this study. Data were collected and analyzed by using the ‘Statistical Package for Social Sciences (SPSS) software, 24-version). A chi-square test was used to see the relationship of organ dysfunctions between stage II and stage III hypoxic ischemic encephalopathy neonates. Results: Out of 70 hypoxic ischemic encephalopathy neonates, 55.70% neonates had stage-II and 44.30% had stage-III hypoxic ischemic encephalopathy. Mean age of the neonates was 10.81±8.08 hours, about 68.60% were male and 31.40% were female. Most of the neonates 71.43% had pulmonary dysfunction, 57.10% had cardiovascular dysfunction. Conclusion: Awareness of hypoxic-ischemic encephalopathy risk factors can help parents and medical personnel to prevent and prepare for possible complications.
Background: Serious morbidity and mortality are linked to infections in pediatric intensive care units (PICUs). The patterns of antibiotic resistance and the bacteriological profile differ significantly between environments. The purpose of this study was to evaluate the bacterial profile and patterns of antibiotic sensitivity in patients with blood cultures who were admitted to Chattogram Maa Shishu O General Hospital's PICU. Materials and Methods: 2384 blood culture samples taken from PICU patients between January and December 2023 were examined in this prospective observational study. Standard microbiological methods were used to identify positive cultures, and the BD BACTEC FX40 system was used to test for antibiotic susceptibility. Results: 96 (4.02%) of the total cultures were positive. The majority of patients were male (68.8%) and younger than 12 months (81.3%). Meningoencephalitis, pneumonia, and recurrent pneumonia were the most frequent primary diagnoses. Pseudomonas (59.4%) and Acinetobacter (27.1%) were the most common isolates, accounting for the majority of gram-negative bacteria (98.9%). These organisms were more susceptible to ciprofloxacin, meropenem, amikacin, and levofloxacin, but they exhibited high resistance to penicillin and cephalosporins. There was only one gram-positive isolate identified, Staphylococcus aureus, and it was amikacin, vancomycin, and linezolid sensitive. Conclusion: Improved infection control and antibiotic stewardship are critically needed in PICU settings due to the high incidence of multidrug-resistant gram-negative infections.
Background: In children suffering from thalassemia, the most common endocrine complication is primary hypothyroidism. However, thyroid dysfunction in transfusion-dependant Hb-E-β thalassemia children still remain underfocused. Therefore, the aim of the study was assessment of thyroid function in Hb E β thalassemia children. Methods: The present study was a descriptive cross-sectional study and conducted at department of Paediatrics in Rajshahi Medical College Hospital, for six months period following approval of the protocol. Total 72 children (age:4-12 years) with Hb E β thalassemia were included in the study. Informed written consent was obtained from parents of the subject and ethical issues were ensured. Thyroid function (T4, TSH) was assessed. Data were collected by face-to-face interview by the researcher with an aid of a semi-structured questionnaire. SPSS 20 was used for data analysis. Results: Mean age of the 72 study population was 7.55±2.46 (SD) year. Majority of the respondents were female 57% (41.04). Mean value of Serum ferritin was 3703±111 µg/l. Among all, 4.8% (3.45) respondents had hypothyroidism and 95.2% (68.54) respondents had normal thyroid status. Moreover, increased ferritin level was associated with hypothyroidism (p<0.05). Conclusion: About one in every twenty children with Hb E β thalassemia was suffering from hypothyroidism. However, further population-based study is required to get the exact prevalence.
