Rabindranath Tagore Medical College, also known as RNT Medical College, is a government medical college situated in the city of Udaipur in the Indian state of Rajasthan.
Darier disease (DD) is a rare autosomal dominant genodermatosis. This comprehensive review, developed by the Darier Disease International Task Force (DDITF), consolidates current knowledge on the genetic basis, molecular pathophysiology, clinical presentation, diagnosis and management of DD, offering a global perspective that unites shared expertise. The disease arises from pathogenic variants in the ATP2A2 gene, which encodes sarco/endoplasmic reticulum Ca2+-ATPase isoform 2 (SERCA2), leading to disrupted calcium homeostasis and impaired desmosomal integrity. Clinically, DD is classified based on lesion type (classic vs. non-classic) and is often accompanied by significant extracutaneous manifestations. Management remains challenging and requires a multifaceted approach, including topical therapies, systemic retinoids and lifestyle modifications. Emerging treatments that target the underlying molecular mechanisms offer promise for improved outcomes. This review aims to provide an updated reference and practical guidance for clinicians involved in the care and study of DD.
Background: Managing unstable intertrochanteric femur fractures in older adults remains challenging, particularly when lateral wall disruption coexists with severe osteoporosis. In patients with Dorr type C femoral morphology, compromised bone quality further reduces fixation stability and increases the risk of mechanical failure. Although intramedullary nailing is widely accepted for unstable fracture patterns, additional lateral wall reconstruction using an extramedullary implant may provide enhanced structural support. Evidence regarding the clinical effectiveness of this combined fixation strategy in elderly osteoporotic patients remains limited. This study evaluated the functional and radiological outcomes of augmented intramedullary fixation in this high-risk population. Methods: A prospective observational study was conducted in 30 patients aged 65–75 years presenting with unstable intertrochanteric fractures associated with lateral wall deficiency and Dorr type C femoral morphology. All participants underwent fixation using a proximal femoral intramedullary nail supplemented with an extramedullary implant. Clinical assessment, radiographic evaluation and functional outcome measurement were performed at 2 weeks, 2 months and 6 months after surgery. Functional recovery was assessed using the Harris Hip Score (HHS), while fracture union, postoperative complications and the timing of weight bearing were also recorded. Results: The mean patient age was 69.5±2.59 years and females represented 53.3% of the study population. Low-energy falls accounted for most injuries (80%). Radiographic union was achieved within 15–20 weeks in 80% of patients. Partial weight bearing commenced within 5–6 postoperative days in 53.3% of cases. Functional performance improved steadily during follow-up, with the mean Harris Hip Score increasing from 47.73±3.08 at two weeks to 86.60±5.96 at six months (p<0.001). At final evaluation, excellent and good functional outcomes were observed in 46.7% and 43.3% of patients, respectively. An uncomplicated postoperative course was documented in 86.7% of patients, while implant-related complications were uncommon. Conclusions: Augmentation of intramedullary fixation with an extramedullary implant provides stable fixation for unstable intertrochanteric fractures with lateral wall deficiency in elderly patients with Dorr type C femora. The technique supports early mobilisation, facilitates timely fracture healing and results in favourable functional recovery with a low incidence of postoperative complications. These findings suggest that augmented fixation is a reliable treatment option for complex osteoporotic intertrochanteric fractures.
Background and Aims: Modified electroconvulsive therapy (MECT) is associated with acute hyperdynamic response which depends upon the anesthetic agent used. This study was conducted to compare the effectiveness of dexmedetomidine 0.5 and 1.0 µg/kg) with ketamine-propofol combination in patients undergoing MECT. Material and Methods: One hundred eleven patients of either sex aged 18-60 years with American Society of Anesthesiologists (ASA) Physical Status (PS) I or II were included in this study who were randomly allocated into three groups with 37 patients each. Patients received saline infusion i.e., normal saline (NS) (100 ml), dexmedetomidine (0.5 µg/kg) in 100ml NS and dexmedetomidine (1.0 µg/kg) in 100ml NS over 10 min followed by ketamine (0.5 mg/kg) and propofol (0.5 mg/kg) in Group C, Group D1 and Group D2, respectively. The primary outcome measure was to assess hemodynamic changes while secondary outcome measures were to compare the seizure duration, recovery profile, patient satisfaction and side effects. Results: A statistically significant difference was noted in mean heart rate (HR), systolic blood pressure (SBP), diastolic blood pressure (DBP) and mean arterial pressure (MAP) at all time intervals till 60 min among three groups, ( P < 0.01). A statistically significant reduction in mean HR, SBP, DBP and MAP was noted in Group D2 in comparison to Group C and Group D1 that remained significantly lower from its baseline value at all time intervals, ( P < 0.05). Conclusion: Although both doses of dexmedetomidine (0.5 µg/kg and 1.0 µg/kg) are safe and effective but dexmedetomidine (1.0 µg/kg) seems to be more effective as an adjunct with ketamine-propofol combination for modified ECT.
Background: Distal radius fractures are among the most frequently encountered fractures in orthopaedic practice and can result in significant impairment of wrist function if inadequately managed. Although several surgical techniques are available for unstable distal radius fractures, the optimal fixation method remains controversial. The five K-wire fixation technique is a minimally invasive, cost-effective method that aims to provide stable fracture fixation while preserving soft tissue integrity and fracture biology. The present study evaluated the functional outcome of displaced distal radius fractures treated using this technique. Methods: A prospective observational study was conducted over one year among 30 adult patients with displaced intra-articular distal radius fractures managed with the five K-wire fixation technique. Fractures were classified according to the Frykman classification. Functional outcome was assessed using the Modified Mayo Wrist Score (MMWS) at 6 weeks, 3 months and 6 months. Fracture union, wrist range of motion and postoperative complications were also evaluated. Statistical analysis was performed using SPSS version 26.0 and a p value <0.05 was considered statistically significant. Results: The mean age of the participants was 44.13±14.22 years and 56.7% were male. Fall on an outstretched hand was the most common mechanism of injury (53.3%). Fracture union was achieved within 6–8 weeks in 86.7% of patients. The mean modified mayo wrist score improved significantly from 65.50±7.97 at 6 weeks to 76.60±8.77 at 3 months and 85.03±8.87 at 6 months (p<0.001). At final follow-up, 46.7% of patients had excellent functional outcomes, 36.7% had good outcomes and 16.7% had fair outcomes. Pin tract infection was the most frequent complication, occurring in 10% of patients. Conclusions: The five K-wire fixation technique provides satisfactory fracture union, significant functional improvement, acceptable wrist mobility and a low complication rate. It represents a safe, minimally invasive and economical treatment option for displaced distal radius fractures, particularly in resource-constrained healthcare settings.
Ovarian hyperstimulation syndrome (OHSS) is a serious complication of ovarian stimulation in assisted reproductive technologies, characterized by an exaggerated ovarian response leading to increased vascular permeability, fluid accumulation, and potential severe outcomes such as ascites, thrombosis, and peritonitis. Diagnosing OHSS-related ascites can be challenging, particularly in the medicine wards when patients conceal reproductive interventions. A 24-year-old female presented with progressive abdominal distention and shortness of breath. Initial examination revealed abdominal distention, bilateral pleural effusions, and a transudative ascitic fluid with normal cytology. Ultrasonography (USG) indicated moderate ascites and bilateral enlarged ovaries. Despite denying prior interventions, the patient was later admitted to egg donation. Treatment with intravenous (IV) fluids, antibiotics, and albumin initially improved her condition. However, she developed peritonitis, indicated by gross pus in a repeat ascitic tap and a positive Escherichia coli culture. Following intensive treatment and peritoneal drainage, the patient improved and was discharged. Ascites in OHSS, particularly with covert ovum extraction, can be misleading, complicating diagnosis and management. Progression to peritonitis, characterized by severe abdominal pain and systemic inflammation, can be insidious and challenging to detect without thorough examination. Effective management involves addressing fluid balance, preventing complications such as thrombosis and infection, and ensuring prompt intervention to avoid severe outcomes. OHSS-related ascites presents a diagnostic challenge, especially when linked to covert reproductive procedures. Peritonitis can complicate OHSS and necessitate vigilant monitoring and management. A detailed patient history and thorough clinical evaluation are crucial for accurate diagnosis and effective treatment, emphasizing the need for awareness among healthcare providers.
