Present Case Research Articles

Steroid as a Saviour and Culprit in Pemphigus Vulgaris Therapy: A Rare Case Series

Introduction: Pemphigus is a group of autoimmune dermatological life-threatening diseases of which Pemphigus Vulgaris (PV) is the most common (1-5 cases/million/year) with a mortality rate of 60-90%. PV is generally characterized by painful oral erosions, along with or without cutaneous lesions, as blisters are often misdiagnosed. The management strategy of PV includes steroids along with adjuvant therapy. Case Presentation: We report the case of three female patients with a mean age of 30.67 years who were diagnosed with pemphigus vulgaris and was on steroid therapy for a long duration. The longterm steroid therapy in all these three patients resulted in adverse drug reactions such as Cushing syndrome, oral and vaginal candidiasis. Although the steroid was found to cause ADR among these patients, the treatment with steroids and rituximab was continued. The withdrawal of steroids results in flare-up conditions, whereas the addition of rituximab serves as a steroid sparring partner in the management of PV. However, premedication was administered before the infusion of rituximab to prevent potential toxicity associated with rituximab. Further, administration of pulse therapy over oral dosing of steroids lowers the risks of ADR and long-term complications associated with steroids. Conclusion: As PV requires the management with high doses of steroids along with the immunosuppressant, patients are more prone to developing drug-related problems. Hence, the rigorous monitoring of the therapy is vital to prevent adverse effects of long-term therapy. Clinical pharmacists and pharmacologists are the most suitable pillar of the healthcare system for drug therapy management.

Previously unrecognized Morgagni’s hernia presenting with bowel obstruction: a rare case report

Introduction and importance: Morgagni’s hernia (MH) is a rare entity, it is defined by the protrusion of abdominal viscera through an anterior retrosternal diaphragmatic defect. It is mainly diagnosed during the perinatal and neonatal periods. A few cases have been reported in adults. The objective of this study was to report the case of a patient with an unrecognized MH, review the current literature on Morgani hernias in the adult population, and assess their clinical characteristics and therapeutic approach reported in adults. We here describe a rare case of a MH presenting with bowel obstruction as the primary symptom in adult life. Case presentation: A 52-year-old man presented to the emergency department with a bowel obstruction. The abdominal CT tomography showed an intestinal bowel obstruction above the hiatal hernia with an intrathoracic ascending colon. Exploratory laparotomy revealed a bilateral strangulated diaphragmatic hernia of Morgagni. The patient underwent closure of the diaphragmatic defect under tension by X-stitch. The recovery was uneventful, and the patient is in good health after 12 months of follow-up. Clinical discussion: The Morgagni hernias are usually asymptomatic; complications such as strangulation of the herniated colon or herniated stomach by stricture are exceptional. Surgical treatment is always recommended for MH. Postoperative complications are rare in simple diaphragmatic hernias, and the course is generally favorable. Conclusion: MH is the rarest form of congenital diaphragmatic hernia, with nonspecific symptomatology contributing to the delay in diagnosis. The cure is surgical, and the open surgical approach is preferable in emergency cases, while laparoscopic surgery is favored in elective settings and is associated with shorter hospitalization. Further studies are crucial in order to elucidate etiology and optimal therapeutic approach.

Left middle cerebral artery and sigmoid sinuses thrombosis in a female patient with Crohn's disease

Introduction: Neurologic manifestations in patients with inflammatory bowel disease (IBD) appear to be more common than in the past. However, vascular events in IBD are still a rare presentation and were reported in few cases. Case presentation: A 48-year-old female patient known case of Crohn’s disease presented with sudden onset aphasia, headache, blurring of vision, and decreased level of consciousness for 2 hours. The patient looked ill, confused, agitated, and aphasic. Neurologic examination shows dysarthria and 0/5 power of the right upper and lower extremity. A brain CT without contrast was ordered and showed acute ischemic stroke. The patient received tPA (alteplase) immediately in the intensive care unit and was under observation. On admission, brain CT angiography, MRI, and MRV were performed, which showed middle cerebral artery and sigmoid sinuses thrombosis. The patient has undergone cerebral catheterization, and an MCA stent was inserted. Post catheterization, the patient’s right-sided extremity power was improved to 3/5. She was discharged from the hospital after 13 days in good general condition except for her expressive aphasia. Conclusion: To the best of our knowledge, here we report the first case presented with both; cerebral venous sinus thrombosis and cerebral arteries thrombosis in a patient with IBD. Clinicians must be considered ischemic stroke in any patients with IBD presented with acute neurological manifestation, and an urgent interventional approach must be performed.

Primary Carcinosarcoma of the breast: Report a very rare case

Background: Sarcoma of the breast is a rare malignancy with heterogeneous histology. angiosarcoma is the most common type of sarcoma of the breast secondary from previous radiation.There is limited clinical and survival information from other types of breast sarcomas. Case Presentation: Here we present A 56-year-old woman which presented with a mass in her right breast. On examination the tumor was not fixed to chest wall. But skin over mass was erymatous and with nipple retraction.A cor-needle biopsy was performed which showed leiomyosarcoma. After IHC the difintive diagnos was sarcoma. A radical resection of mass with radical mastectomy and axillary dissection were performed, in permanent pathology all margins was free of tumor. After surgery patient refer to radio oncologist for irradiation therapy Conclusions: Surgical resection seems to be the most important treatment modality in sarcoma of the breast. Radiation therapy added a minor survival benefit to the patients who received surgical resection. Systemic chemotherapy did not any role in sarcoma of the breast.

An unusual cystic presentation of pelvic skeletal Ewing sarcoma: a case series.

Ewing sarcoma (ES) is the second mostcommon primarymalignant bone tumour in children and adolescents. About 14.5% of primary malignancies develop in pelvic bones, wherethey typically have worse prognoses than extremity or acral sarcomas. It usually presents with aggressive features onradiology scans, but may also present with different radiological characteristics. In this series, we describe rare appearancesof pelvic skeletal Ewing sarcoma, with large extraosseous cystic component on imaging, defined by the presence of fluid-filledspaces in the extraosseous tumour lesion, which distinguishes it from the solid nature of conventional ES. We report3 cases of cystic presentation of ES, with imaging features supporting diagnosis of a primary malignant bone tumourarising from the superior pubic ramus with associated massive intrapelvic solid and cystic mass. CT-guided biopsy provideddiagnosis of ES, with large intrapelvic soft tissue and cystic component. These patients underwent neo-adjuvantchemotherapy and proton beam therapy with significant reduction in size of the solid components, while the cysticcomponents remained relatively unchanged. Two patients underwent surgical resection of the tumour (navigated P3internal hemipelvectomy and hemipelvis P2/P3 resection, respectively), and one patient died while on treatment. In both whounderwent surgery, histology showed ES with margins clear and more than 99% of treatment-induced necrosis. To theauthors' knowledge, this unusual presentation of pelvic ES is described for the first time in the literature as a case series,with particular reference to atypical extraosseous cystic changes, along with the clinical and radiological characteristics,and their treatment.

When rare diseases crisscross within the same patient: von Hippel-Lindau and type 1 gastric neuroendocrine tumor.

Von-Hippel-Lindau (VHL) is a genetic multisystem disorder characterized by visceral cysts and benign and malignant tumors in various organs. Herein, we present the case of a 23-year-old woman with VHL presenting with multiple gastric neuroendocrine neoplasms (gNENs) type 1 in the context of chronic autoimmune gastritis (CAG). Although gNENs are not acknowledged as a typical entity in VHL patients, in the present case, gNENs were composed of neoplastic cells with clear cytoplasm usually seen in tumors related to VHL disease. We additionally performed a literature review on the presence of neuroendocrine clear cell tumors and report on further cases of clear cell NENs. The present case illustrates that clear-cell transformation in gNENs may be due to the dual genetic background of the patient; the real oncogenic stimulus may be more closely related to CAG than to VHL disease accompanied by an interplay between neoplastic and autoimmune processes. Therefore, close monitoring of patients with clear cell NENs appears to be important before excluding VHL disease, even in the context of phenotypically unrelated diseases.

Intrahepatic Cholangiocarcinoma with High Microsatellite Instability and Tumor Mutation Burden That Responded Significantly to Pembrolizumab but Perforated within a Short Period.

Cholangiocarcinoma has a poor prognosis, and resection is the only curative treatment. Pembrolizumab, a programmed death receptor 1 inhibitor, has proven effective against unresectable or metastatic solid tumors with high microsatellite instability (MSI-H) or a high tumor mutation burden (TMB-H). In the present case, pembrolizumab treatment was initiated after standard chemotherapy for MSI-H and TMB-H unresectable intrahepatic cholangiocarcinoma. Intrahepatic tumor necrosis perforated the abdominal cavity. Emergency surgery was performed, but the patient died 36 days after admission. A pathological autopsy revealed that the intrahepatic tumor had almost completely disappeared.

Retrieval of a Missing Translocated Intrauterine Contraceptive Device Using Combined Laparoscopic and Cystoscopic Techniques

Background. Intrauterine contraceptive devices (IUCDs) are considered to be an effective way of preventing unwanted pregnancies. However, one significant complication associated with IUCDs is uterine perforation especially at the time of insertion and could reach the peritoneal cavity and the viscus of the adjacent organs. Intravesical migration is extremely rare. Case Presentation. We report a 41-year-old woman who was diagnosed with IUCD intravesical migration after she presented to our hospital complaining of persistent lower urinary tract symptoms. Laparoscopic removal was done after the failure of cystoscopic extraction. Conclusion. The IUCD must be monitored continuously by the gynaecologist, and suspicions of intravesical migration must be considered in those presenting with persistent, unexplained lower urinary tract symptoms.

Management of enamel demineralization- An iatrogenic effect of orthodontic treatment: A case report

Areas of demineralised enamel, usually referred to as white spot lesions (WSLs), constitute to a clinical problem in Orthodontics following treatment with fixed appliances. They result due to an imbalance between de- and remineralisation of enamel, caused by the inter-relationship of several factors. This manuscript is intended to elucidate the presentation of a clinical case treated with orthodontic braces with 10-year follow up, and management of demineralization with veneers that occurred due to long term fixed orthodontic treatment. The goal of treatment has been achieved according to patient’s desire and satisfactory digital veneer placement. No further relapse has been seen in the follow up. The treatment resulted in an aesthetic, functional, and stable occlusion, along with improved facial profile.

Primary intracranial peripheral primitive neuroectodermal tumor: lessons from an exceptionally rare neoplasm. Illustrative case.

The primary intracranial peripheral primitive neuroectodermal tumor (pPNET) is a lesion subtype within the Ewing sarcoma family of tumors. pPNETs are extremely uncommon pathologies, accounting for 0.03% of intracranial tumors and 1% to 2% of Ewing sarcoma cases. Given its histological aspect similar to other highly proliferative malignant neuroectodermal neoplasms, pPNET merits extensive workup for accurate diagnosis and treatment. A 36-year-old male presented to the emergency department with a 1-year history of headaches in the right frontoparietal area, generalized tonic-clonic seizures, and a history of the resection of a tumor labeled as a meningioma 5 years before admission. He was neurologically intact. Brain magnetic resonance imaging revealed a heterogeneous focal lesion of 25 × 35 × 23 mm with a necrotic center and neoformative appearance in the right frontal cortex. The patient underwent multimodal treatment with gross-total resection, radiotherapy, and chemotherapy. Histopathological examination results supported the diagnosis of pPNET. At the 2-year follow-up, the patient had no new-onset symptoms, and brain imaging revealed absent signs of tumor recurrence. The present case describes an extraordinary pPNET case, initially confounded as a clear cell meningioma. Managing pPNET requires thorough investigation, careful differentiation from similar neuroectodermal lesions, and multimodal treatment to improve the patient's prognosis.

Large Skull Metastasis in Follicular Thyroid Carcinoma: A Comprehensive Case Presentation and Systematic Review.

Skull metastases from follicular thyroid carcinoma (FTC) are infrequent but clinically significant, often presenting with localized pain, neurologic deficits, and cranial nerve dysfunction. Early detection and accurate diagnosis pose challenges due to their asymptomatic nature in some cases. A systematic literature review, conducted following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, identified and analyzed 15 relevant studies focusing on large skull metastases in FTC. Data extraction and synthesis included clinical presentation, diagnostic methods, treatment strategies, and patient outcomes. The systematic review encompassed 20 patients with secondary skull metastases from FTC, offering insights into the clinical diversity of this rare condition. Clinical presentations varied, with localized pain (70% of cases) and headaches being predominant symptoms. Imaging techniques, including computed tomography (CT) and magnetic resonance imaging (MRI), played a pivotal role in diagnosis. Surgical resection was considered in select cases, achieving complete or near-complete tumor removal in 30 to 50% of patients. Radiotherapy, including external beam radiation therapy (EBRT) and stereotactic radiosurgery (SRS), provided local control and symptom relief in 70 to 80% of cases. Systemic therapies, such as tyrosine kinase inhibitors (TKIs), showed promise in disease stabilization or regression (45% of patients). Prognosis remained poor, with a median overall survival of 6 to 12 months, reflecting an advanced and aggressive disease state. Managing secondary skull metastases from FTC requires a comprehensive approach, including surgical intervention, radiotherapy, and potential systemic therapies. The rarity of these metastases underscores the need for further research to establish standardized treatment guidelines, explore molecular profiling, and investigate immunotherapy and combination therapies, offering hope for improved outcomes in this challenging clinical scenario.

Isolated Case Presentation on Phacolytic Uveitis

The “Type of Article” of this paper is “Letter to the Editor”. This paper discuses about: “Isolated Case Presentation on Phacolytic Uveitis”. No formal abstract is available. Readers are requested to read the full article.

Finger Necrotizing Fasciitis and Septicemia Caused by Vibrio vulnificus.

Background: Vibrio vulnificus infections develop rapidly and have high mortality and disability rates. Vibrio vulnificus can cause local wound infection, gastroenteritis, or septicemia. Case Presentation: In this case, an 86-year-old male was accidentally stabbed in the middle of his right thumb while cleaning whitewater fish and came to the emergency department with high fever and painful swelling of the right hand. Physical examination revealed hemorrhagic bullae in the right hand. Emergency surgery and bacterial culture were performed. Because of timely antibiotic use and surgical treatment, the patient eventually recovered and was discharged from the hospital. Conclusions: This case suggests that the possibility of Vibrio vulnificus should be considered in cases of severe infection of the extremities, even without a history of seafood consumption or seawater exposure. Early recognition, rational choice of antibiotic agents, and timely wound debridement can substantially improve the prognosis of patients and reduce mortality.

Identification, Evaluation, and Correction of Supracrestal Tissue Attachment (Previously Biologic Width) Violation: A Case Presentation With Literature Review

Identification, Evaluation, and Correction of Supracrestal Tissue Attachment (Previously Biologic Width) Violation: A Case Presentation With Literature Review

Fulminant Hepatitis Secondary to Anti-tuberculosis Drug-induced Hepatotoxicity Complication, Its Prevention Strategies and Management: A Case Report

Aim: Tuberculosis remains a public health problem around the world. Hepatotoxicity is a serious side effect of anti-tuberculosis treatment. Fulminant hepatitis is a rare form but considered very serious outside of liver transplantation. It can occur several weeks or months after the start of treatment.
 Presentation of Case: We report the case of a 34-year-old single male patient treated for pleural tuberculosis in whom fulminant hepatitis appeared after four months of treatment with Isoniazid and Rifampicin. Despite a treatment in intensive care unit He had a fatal outcome because of lack of liver transplantation.
 Discussion: Hepatotoxicity varies from biological hepatitis to fulminant hepatitis. Application of personalized strategy of genetic analysis and pharmacological drug monitoring to optimize treatment is the most safe to avoid antituberculosis drug induced hepatotoxicity but not available in all healthcare centers of developing countries. There was any change of anti-tuberculosis protocol because of the risk of bacterial resistance. The protocol includes association between several medicines potentially toxics for a long duration. For some moderate forms of   tuberculosis (nodals, pleural), it’s necessary to ask if duration of antiviotherapy can be reduced.
 Conclusion: Prevention of hepatotoxicity starts with identifying risk factors, regular clinical and biological assessment and informing patients of symptom that can indicate toxicity to react early.

Physiotherapy Management of Guillain-Barré Syndrome in a Tertiary Hospital in South-South Nigeria: A Case Report.

Background: Guillain-Barré Syndrome (GBS) is a potentially life-threatening post infectious disease characterized by rapidly progressive, symmetrical weakness of the extremities. It can cause life-threatening complications, particularly if the breathing muscles are affected or if there is dysfunction of the autonomic nervous system. Case Presentation: This is a case report of Physiotherapy management of a 51-year old female patient with Guillain-Barré Syndrome (GBS). Patient was referred from the neurology unit, department of Internal Medicine, University of Port Harcourt Teaching Hospital (UPTH) to the neurology clinic of the physiotherapy department, UPTH as a case GBS after series of investigations. Appropriate physiotherapy intervention was given for a period of 36 weeks after a thorough assessment of the patient. The Guillain-Barre Syndrome Disability Score was used to assess the overall functional status of the patient while the Oxford Muscle Grading Scale was used to assess for muscle strength. The Numerical Pain Rating Scale (NPRS) was used to assess the level of pain in the limbs while the Berge Balance Scale (BBS) was used to assess the patient’s balance. The case report was presented in line with the CARE: Case Report Guideline for case reports. All ethical procedures were followed. Treatment outcome: Treatment outcome revealed that the Guillain-Barré Syndrome Disability Score reduced from 4 to 2 while that of Berg Balance Scale increased from 3 to 42 after 36 weeks of intervention. Pain level on range of motion of the limbs based on the Numerical Pain Rating Scale reduced from 6/10 to 1/10 on the upper limb and from 8/10 to 2/10 on the lower limbs. Conclusion: Physiotherapy is effective in the management of patients with Guillain-Barré Syndrome. Early referral to Physiotherapy is key to pain management, balance control and overall functional status of the patients.

Diagnosis of Acute Bulbar Palsy Plus Syndrome: A Rare Variant of Guillain–barre Syndrome

Presenting case of a 11-year-old girl with sudden cranial nerve weakness, universal areflexia but without limb pain or weakness. NCV showed subclinical axonal involvement in limbs. A diagnosis of acute bulbar palsy plus–GBS was made and child was started on Iv Immunoglobulins which showed significant improvement. This report is for raising awareness about this variant of GBS.

Current progress and future prospects for understanding genetic diversity of seed plants in China

AbstractGenetic diversity represents the fundamental basis of biological variation within species, and is therefore the ultimate representation of biodiversity. Despite that all forms of diversity being defined by genomic differences, genetic diversity has received relatively less attention compared with species and ecosystem diversity. Here, we review recent literature and conclude that progress in understanding genetic diversity of seed plants, is strongly associated with advances in sequencing technology. We here present case studies to illustrate the application of genetic diversity for tracing crop domestication and delimiting species boundaries. Understanding genetic diversity is particularly critical for the field of conservation biology, and there is a clear shift to population‐level genetic studies to understand rare species. We then document additional factors that potentially influence genetic diversity, including climate change, habitat fragmentation, and species invasion. Finally, we identify current research limitations and propose directions for future studies. We highlight the need to develop broad‐scale genetic diversity knowledge, combined with other aspects of diversity to improve biodiversity conservation outcomes. We conclude that populating global databases with genomic‐scale sequence data for all species is an urgent and achievable goal now.

Surgery for Obsessive-Compulsive Disorder and Transient Tremors as Newly Reported Side Effect; First Psychiatric Neurosurgery in Egypt

Abstract Introduction: The use of surgery for treatment of psychiatric conditions is a well-established strategy, especially in severe and resistant obsessive-compulsive disorder. Attractive anatomical and functional targets for stereotactic surgery are reported in some studies. Surgery for treatment of psychiatric conditions in our nation and Arab world is obscured and hidden because of several social and cultural limitations which should be overcome. We report here the first psychiatric neurosurgery in our nation and how we overcome such community limitation. This the first report of postoperative tremor. Case Presentation: Young patient presented with severe and persistent obsessive-compulsive disorder resistant to all non-surgical modalities for several years. Stereotactic ablation surgery was done under local anaesthesia. Marked improvement in our obsessive-compulsive disorder patient after psychiatric neurosurgery with self-limited tremor which was not reported before in the literature. The medications were the same before and immediate after surgery and this is not a drug-induced tremor. Postoperative YBOCS showed 90% of improvement. Conclusion: Surgery-induced tremor could be a self-limited side effect after surgery in obsessive compulsive disorder. Safety and efficacy should be promoted in our nation and Arab world. Society and cultural limitations should be overcome by further research studies, intervention, and activism in the field of mental health systems in our nation and Arab countries to improve awareness.

A RARE COMPLICATION: ACUTE RESPIRATORY DISTRESS SYNDROME (ARDS) INDUCED BY SEVERE PNEUMOCYSTIS CARINII PNEUMONIA (PCP)

Introduction: Pneumocystis pneumonia (PCP) represents a prevalent opportunistic infection among individuals with HIV, posing a significant risk of mortality. The occurrence of acute respiratory distress syndrome (ARDS) as a consequence of PCP further accentuates the gravity and intricacy of this pulmonary infection. Objective: This case report aims to discuss the management of the rare complication of ARDS induced by severe PCP in a 36-year-old man with HIV. Case Presentation: A 36-year-old male teacher, presented with worsening dyspnea, intermittent fever, and a productive cough persisting for two weeks, along with weight loss and fatigue. Initial diagnostic impressions included pulmonary tuberculosis with pneumonia. Treatment involved oxygen therapy, fluid administration, and medication. Laboratory tests showed leukocytosis, elevated blood glucose, and abnormal liver function. Subsequent investigations confirmed HIV infection. Chest X-ray revealed severe bilateral pneumonia and possible ARDS, leading to transfer to the Intensive Care Coronary Unit for further management. Discussion: The diagnosis of severe Pneumocystis jirovecii pneumonia (PCP) underscores the importance of recognizing PCP as a significant clinical concern, especially in HIV patients. Diagnostic challenges, such as late HIV diagnosis, highlight the need for timely screening and intervention. Management involved a multidisciplinary approach, including pulmonologists and cardiologists. Despite treatment adjustments, some patients experience clinical deterioration, necessitating careful management strategies like antiretroviral (ARV) therapy and respiratory support. This case underscores the complexity of managing PCP-related ARDS and the crucial role of timely intervention and comprehensive care. Conclusion: Severe PCP led to ARDS in an HIV-infected patient due to inflammation and lung damage, exacerbated by the immunocompromised state. Timely diagnosis and treatment are crucial to prevent complications and improve outcomes in such cases.