Search

Introduction: Wünderlich syndrome (WS) refers to subcapsular, perirenal, or pararenal hemorrhage due to non-traumatic and iatrogenic conditions. Neoplasms, vascular disease, renal etiology, and anticoagulant use are underlying risk factors.Case Report: We describe a case of WS in a 79-year-old male who was undergoing hemodialysis, which resulted in hemorrhagic shock requiring multiple transfusions and embolization by interventional radiology.Conclusion: Most commonly, patients present with flank pain; a computed tomography with contrast of the abdomen is essential for diagnosis. Surgical intervention is considered in hemodynamically unstable patients. Conservative therapy and intravenous resuscitations with blood products are considered a priority in hemodynamically stable patients.

Acromegaly is a rare systemic syndrome induced by the overproduction of growth hormone (GH) and insulin-like growth factor type one (IGF1). It is responsible for changes in the skeletal and soft tissue systems and it almost always occurs because of a pituitary adenoma. Amongst the skin complications related to acromegaly, cutis verticis gyrate (CVG) is occasionally found. It is a skin condition characterized by excessive growth of the skin of the scalp, resulting in furrows and folds. Only a few cases of this uncommon association have been reported in the literature. The present clinical case illustrates typical CVG associated with acromegaly. Imaging revealed a pituitary macroadenoma lesion and hormonal evaluation revealed elevated IGF1 and hypopituitarism. The patient underwent a transsphenoidal resection of the pituitary adenoma and the histopathological examination confirmed the diagnosis. The diagnosis of CVG is clinical, so radiologic assessments are generally not necessary. The management of acromegaly associated with CVG depends on controlling the serum levels of GH and IGF1. In some cases, specific injections or surgery can be used to minimize CVG.

Background: Epithelioid Hemangioendothelioma (EHE) is an extremely rare malignancy originating from endothelial cells, with an incidence rate of less than 1/100,000. To date, there have been no documented cases of Diaphragm EHE in the English or Chinese literature. EHE can manifest in various organs throughout the body and lacks distinctive clinical features, often leading to misdiagnosis. Given its rarity, there is currently no standardized treatment protocol, management options include radiotherapy, chemotherapy, and targeted therapy. In this report, we present a case study of a 75-year-old male patient who presented with a 6-month history of cough, sputum production, chest tightness, and pleural effusion. A biopsy of the diaphragm mass and immunohistochemical analysis of the pleural fluid confirmed the diagnosis of EHE. The patient underwent chemotherapy combined with targeted therapy, however, unfortunately experienced disease progression. Case summary: In March 2023, a 75-year-old male patient was admitted to our hospital with persistent cough for over two months accompanied by sputum production and chest tightness. The patient was diagnosed with Diaphragm EHE accompanied by pleural effusion and received treatment at our institution. We initiated combination chemotherapy using albumin-bound paclitaxel and cisplatin along with intrapleural infusion of bevacizumab as an anti-angiogenic drug. After one cycle of treatment, significant control over the pleural effusion was observed which prompted us to administer systemic treatment through intravenous infusion using albumin-bound paclitaxel, cisplatin, and bevacizumab. Unfortunately, the patient's condition continued to deteriorate. Conclusion: When accompanied by pleural effusion, EHE often demonstrates rapid disease progression. [ABSTRACT FROM AUTHOR]

Albinism is a result of cells that are unable to make melanin or pigments which are necessary to color the skin. It occurs very rarely in the wild animal. Although several species of monkeys with albino individuals have been recorded in other parts of the world, it has never been recorded in Grey Langur and endemic Toque Macaque. Hence this would be the first ever albino Grey Langur and endemic Toque Macaque individuals recorded in Sri Lanka. [ABSTRACT FROM AUTHOR]

Mullerian adenosarcoma is rare, usually found in the uterine corpus and rarely in the cervix. Adenosarcoma that grows diffusely in the uterine cavity and the cervical canal is even rarer without symptoms. Herein, we report a rare case of multiple primary malignant neoplasms of Mullerian adenosarcoma accidentally detected and coexisting with cervical carcinoma in situ. Fortunately, the tumor was in the early stage and the Mullerian adenosarcoma was treated together with the cervical carcinoma in situ by hysterectomy + bilateral adnexectomy. Histopathology and immunohistochemistry results confirmed this diagnosis, further confirmed by a pathology consultation at the University Hospital. The patient recovered well from the surgical treatment and was discharged with regular follow-up. The patient did not undergo pelvis--abdomen CT and diagnostic curettage preoperatively, and no malignancy was detected by cryopathology intraoperatively, which may be related to the rarity of the disease and the relative lack of awareness and experience of our clinicians and pathologists for this tumor. We hope that this rare case can provide some lessons for gynecologists and pathologists. [ABSTRACT FROM AUTHOR]

Background: Numerous rare congenital bleeding disorders include fibrinogen and prothrombin deficiency as well as factor (V, VII, VIII, XI, X, and XIII) deficiency. In the general population, the severe variants of these illnesses are hereditary and possess a frequency that falls somewhere between one in every two million for factor XIII and one in per half a million for factor VII. Patients who have rare congenital bleeding disorders may exhibit a broad range of symptoms, from relatively mild subcutaneous bleeding to potentially life-threatening hemorrhages for example in the central nervous system. The majority of treatment for these disorders when specific plasma-derived or recombinant products are available is to replace the missing factor. Diagnosing and treating rare congenital bleeding disorders can be challenging due to the wide variety of symptoms they manifest in patients. Patients with rare inherited bleeding diseases must be closely monitored at specialized hemophilia treatment centers once they are diagnosed, due to the unique difficulty of their therapy. More research is required to improve the worldwide care for individuals with rare inherited bleeding diseases, despite the fact that a lot has been learned about their frequency, symptoms, and genetic characteristics in the past ten years. We aimed at this review to give a brief overview about Inherited Bleeding Disorders. [ABSTRACT FROM AUTHOR]

Metastasis to the heart is uncommon compared to other organs, posing notable diagnostic challenges. Incidence in autopsy studies typically ranges between 0.7% and 3.5%. Lung, breast, and hematologiccancers are the main sources of such metastases, leading to varied clinical presentations and severe complications. Postmortem viscera from a 47 year old male with a suspected history of jaundice. Only limited information was available. Gross examination showed a solid mass in the gall bladder with areas of hemorrhage and necrosis, while no other abnormalities were observed in other viscera received. Brain examination showed congested blood vessels, while heart sections exhibited atypical cell deposits in the pericardium and myocardium. Coronary arteries displayed pathological thickening. Lung and liver sections also displayed similar metastatic atypical cell deposits, infiltrating tissue and causing necrosis. Gall bladder examination revealed irregular glandular formations lined with polygonal tumor cells positive for cytokeratin. The final diagnosis based on histomorphological features and immunohistochemistry confirmed gall bladder adenocarcinoma metastasising to the heart, liver and lungs. [ABSTRACT FROM AUTHOR]

The species of Archidendron pahangense (Kosterm.) I.C. Nielsen (Fabaceae) is an endemic species in Peninsular Malaysia known only from Pahang and Selangor state. This species was also recorded in Gunung Rabong, Kelantan. This species was reported in Fraser Hill, Pahang, whereby common in the Pine Tree Hill. This species was also observed in Semangko Tambahan Forest Reserve, Selangor. Fertile specimens were collected in 1923, 1938, 1954, 1966 and 1967. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Mediastinal monophasic synovial sarcoma is a rare subtype that often lacks specific imaging characteristics, posing diagnostic challenges. This case report describes a mediastinal monophasic synovial sarcoma with vertebral metastasis, emphasizing imaging findings, differential diagnosis, and pathological features, thereby providing crucial support for accurate diagnosis and treatment planning. [ABSTRACT FROM AUTHOR]

Abstract Background Epithelioid Hemangioendothelioma (EHE) is an extremely rare malignancy originating from endothelial cells, with an incidence rate of less than 1/100,000. To date, there have been no documented cases of Diaphragm EHE in the English or Chinese literature. EHE can manifest in various organs throughout the body and lacks distinctive clinical features, often leading to misdiagnosis. Given its rarity, there is currently no standardized treatment protocol, management options include radiotherapy, chemotherapy, and targeted therapy. In this report, we present a case study of a 75-year-old male patient who presented with a 6-month history of cough, sputum production, chest tightness, and pleural effusion. A biopsy of the diaphragm mass and immunohistochemical analysis of the pleural fluid confirmed the diagnosis of EHE. The patient underwent chemotherapy combined with targeted therapy, however, unfortunately experienced disease progression. Case summary In March 2023, a 75-year-old male patient was admitted to our hospital with persistent cough for over two months accompanied by sputum production and chest tightness. The patient was diagnosed with Diaphragm EHE accompanied by pleural effusion and received treatment at our institution. We initiated combination chemotherapy using albumin-bound paclitaxel and cisplatin along with intrapleural infusion of bevacizumab as an anti-angiogenic drug. After one cycle of treatment, significant control over the pleural effusion was observed which prompted us to administer systemic treatment through intravenous infusion using albumin-bound paclitaxel, cisplatin, and bevacizumab. Unfortunately, the patient’s condition continued to deteriorate. Conclusion When accompanied by pleural effusion, EHE often demonstrates rapid disease progression.

The Ariake catfish, Tachysurus aurantiacus, is a freshwater fish endemic to Kyushu Island, Japan. However, these catfish are now endangered owing to environmental changes. Despite their status, there is scant quantitative research on the Ariake catfish regarding their potential conservation. The Yabe River is a typical catfish habitat situated in the northern part of Kyushu Island (Ariake Area) and has a unique civil engineering heritage, as represented by the so-called ‘detour canal’. The canals were created owing to competition by two Domains to divert additional water resources into their own territory for rice cultivation during the Edo Period (1603–1867). To fill the research gap on the Ariake catfish and assess the ecological value of detour canals, in this study, we conducted a survey of local catfish populations and nine environmental parameters that can affect them. We found that the population volume of the Ariake catfish was significantly higher in canals than in ordinary branch rivers. Although the detour canals were not originally constructed for biodiversity conservation, they nonetheless unintentionally provide catfish habitat at present. As these canals represent a remarkable example of a contribution by a civil engineering heritage structure to biodiversity conservation, our study should be used as a potential justification for preserving the canals, as well as conserving the aquatic species that utilise them as vital habitat.

The decline of swallowtail butterflies in forest habitats, which was not a significant concern about two decades ago, has now garnered attention worldwide, leading to their designation as globally endangered by the International Union for Conservation of Nature. A recent study conducted in the north bank landscape (NBL) of river Brahmaputra, specifically under the Bodoland Territorial Region in India, has shed light on the remarkable diversity of swallowtail butterfly species in the area. The study documented a total of 35 species representing eight different genera. Notably, three species are endemic to northeastern India, and 12 enjoy federal protection. The findings suggest that the studied landscape plays a crucial role in supporting larval host plants and adult resources of swallowtail butterflies along with the other abiotic factors. These butterflies depend on 25 plant species from six families as essential food sources. Unfortunately, these host plants, valued for their traditional medicinal properties, are being overexploited. Urgent conservation measures are imperative to safeguard the habitats of swallowtail butterflies and other wildlife in the NBL under Bodoland Territorial Region, as they face significant threats from practices such as agriculture, illegal tree felling, forest fires, and cattle farming. [ABSTRACT FROM AUTHOR]

Persistent sciatic artery (PSA) is a rare congenital vascular anomaly resulting from embryologic axial artery malformation in the lower limb. This case report presents three patients aged 45–60, each with bilateral PSA presenting with symptoms indicative of PSA complications, including aneurysmal degeneration, limb ischemia, thromboembolism, or neuralgia from nerve compression. It highlights the diagnostic process, management strategies, and clinical outcomes observed at a tertiary referral hospital. Treatment involved a collaborative, multidisciplinary approach with vascular surgeons, internists, and radiologists tailoring interventions to individual patient findings and disease progression. This report aims to provide insights into the diverse presentations and management of PSA in a resource limited setting, encouraging further reporting and case studies to enhance understanding of therapeutic outcomes. [ABSTRACT FROM AUTHOR]

Nuclear protein in testis (NUT) carcinoma is a rare neoplasm arising mainly from midline structures. It is an aggressive type of carcinoma associated with poor survival despite the use of multiple treatment modalities. Here, we present a case of a 17-year-old paediatric patient with NUT carcinoma of larynx, which is even rarer among all reported cases. The patient underwent surgery followed by radiotherapy and systemic treatment and he died 15 months after the diagnosis. The management of this rare disease requires further investigation. [ABSTRACT FROM AUTHOR]

Caudal regression syndrome (CRS), also known as caudal agenesis, results from abnormal development of the caudal aspect of the spinal cord and vertebral column due to an earlier abnormality of gastrulation. This report showcases a unique scenario where three siblings, devoid of any prior family history or identifiable risk factors, exhibit symptoms of CRS and receive care at a government-run tertiary facility dedicated to children's health. In establishing a concrete diagnosis, we relied on skeletal surveys, comprehensive symptom evaluation, and medical history assessment. Additionally, we recommended further investigation through magnetic resonance imaging and genetic testing to attain a more in-depth understanding and confirmation of the condition. Unfortunately, the financial constraints faced by the parents led to the unfeasibility of pursuing these advanced diagnostic options. Given the rarity of this syndrome and the limited existing literature, our report is a significant contribution. It marks the first comprehensive exploration of CRS from the genetic and familial predisposition perspective, shedding new light on this rare condition. This case series pioneers our understanding of the familial and genetic connections between CRS and sacral agenesis. Strikingly, each subsequent generation has experienced more severe manifestations earlier, furnishing compelling evidence that underpins the genetic predisposition to CRS. [ABSTRACT FROM AUTHOR]

Adamantinomatous craniopharyngioma is a grade 1 tumor that arises in a sellar/suprasellar location. Despite being a grade 1 tumor, there is high recurrence and endocrinal insufficiency. Malignancy arising in craniopharyngioma is extremely rare, has a dismal prognosis, and is currently not included as a separate entity in the World Health Organization Classification of Central Nervous System 5th edition. Here we describe a case of adamantinomatous craniopharyngioma and its malignant counterpart. The malignant part had unique histomorphology and basaloid cells with pseudoglandular architecture and a myxoid background. It bore a striking resemblance to adenoid cystic carcinoma. Both the benign and malignant counterparts were beta‐catenin and SOX‐2 positive, providing proof of the malignant part arising from the benign part. Tumors like squamous cell carcinoma and odontogenic ghost cell carcinoma have been described in cranipharyngioma. This case study is the first to describe this unique morphology of adenoid cystic carcinoma‐like features. The possibility of adenoid cystic carcinoma was excluded by immunohistochemistry. [ABSTRACT FROM AUTHOR]

Copyright of Jurnal Ilmu Hukum, Humaniora dan Politik (JIHHP) is the property of Dinasti Publisher and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

• Annesorhiza tilneyae Magee & Maswoliedza is formally described. • validate the name Annesorhiza asparagoides B.-E. van Wyk. • 23 species of Annesorhiza now recognised. • Identification key to all species within the genus provided. Annesorhiza Cham. & Schltdl. (Apiaceae) comprises ca. 23 species endemic to South Africa. The genus is centred in the Greater Cape Floristic Region, with only four species occurring in the summer rainfall regions. In the two decades since Annesorhiza was last revised, the number of recognised species has almost doubled, with 8 species subsequently described and another two transferred to the genus. The high number of new species in the genus can be attributed to the hysteranthous habit, with plants leafless at the time of flowering and fruiting, and that these species are rare, highly localised endemics. Species of Annesorhiza are often overlooked as they flower largely during the dry summer months, and multiple visits are required in order to recover a complete collection for accurate identification. Here we describe another new species from the Bokkeveld Mountains, Annesorhiza tilneyae Magee & Maswoliedza. We also validate the name Annesorhiza asparagoides B.-E. van Wyk with reference to the previously and effectively published descriptions and diagnoses. With several Annesorhiza species described since the last revision, we also provide an updated identification key to all species within the genus. [ABSTRACT FROM AUTHOR]

Despite being the second largest family of flowering plants, orchids represent community structure variation in plant-microbial associations, contributes to niche partitioning in metacommunity assemblages. Yet, mycorrhizal communities and interactions remain unknown for orchids that are highly specialized or even obligated in their associations with their mycorrhizal partners. In this study, we sought to compare orchid mycorrhizal fungal (OMF) communities of three co-occurring hemiepiphytic Vanilla species (V. hartii, V. pompona, and V. trigonocarpa) in tropical forests of Costa Rica by addressing the identity of their OMF communities across species, root types, and populations, using high-throughput sequencing. Sequencing the nuclear ribosomal internal transcribed spacer (nrITS) yielded 299 fungal Operational Taxonomic Units (OTUs) from 193 root samples. We showed distinct segregation in the putative OMF (pOMF) communities of the three coexisting Vanilla hosts. We also found that mycorrhizal communities associated with the rare V. hartii varied among populations. Furthermore, we identified Tulasnellaceae and Ceratobasidiaceae as dominant pOMF families in terrestrial roots of the three Vanilla species. In contrast, the epiphytic roots were mainly dominated by OTUs belonging to the Atractiellales and Serendipitaceae. Furthermore, the pOMF communities differed significantly across populations of the widespread V. trigonocarpa and showed patterns of distance decay in similarity. This is the first report of different pOMF communities detected in roots of wild co-occurring Vanilla species using high-throughput sequencing, which provides evidence that three coexisting Vanilla species and their root types exhibited pOMF niche partitioning, and that the rare and widespread Vanilla hosts displayed diverse mycorrhizal preferences. [ABSTRACT FROM AUTHOR]

It is often supposed that valuable organisms are more valuable if they are rare. Likewise if they belong to endangered species. I consider what kinds of value rarity and endangerment can add in such cases. I argue that individual organisms of a valuable species typically have instrumental value as means to the end of preserving their species. This progenitive value, I suggest, tends to increase exponentially with rarity. Endlings, for their part, typically have little progenitive value; however, I argue that they may nonetheless have persistence value because, merely by existing, they postpone the numerical extinction of their species. Finally, I propose that a sentient endling can have higher lifeworld value than it would have had were it not the last of its kind. This, I argue, is because when a sentient endling dies, very little of its lifeworld is preserved – and this, I suggest, can be a bad thing. [ABSTRACT FROM AUTHOR]

Key Clinical Message Mediastinal monophasic synovial sarcoma is a rare subtype that often lacks specific imaging characteristics, posing diagnostic challenges. This case report describes a mediastinal monophasic synovial sarcoma with vertebral metastasis, emphasizing imaging findings, differential diagnosis, and pathological features, thereby providing crucial support for accurate diagnosis and treatment planning.

Osteochondromas are common asymptomatic lesions. The development of chondroid malignancies from cartilage caps has rarely been described. Costal osteochondromas are relatively rare lesions. The incidence of osteochondromas in flat bones is approximately less than 5% of all osteochondromas. Osteochondroma originating from two adjacent ribs and forming a joint with each other is a phenomenon described for the first time in the literature. [ABSTRACT FROM AUTHOR]

Despite the low incidence of primary cardiac tumors, recently at our institution, we have experienced two very rare tumors in the span of just a few months. Hibernomas are rare tumors of brown adipose tissue origin that share the benign clinical features of a lipoma, but on imaging mimic the more aggressive sarcoma. Here we present two separate cases of otherwise healthy patients who were found incidentally to have these asymptomatic tumors. [ABSTRACT FROM AUTHOR]

Ganglioglioma is a rare neoplasm most common in children and adolescents. It is typically located in the supratentorial compartment, with the temporal lobe being the most common tumor location. Anaplastic ganglioglioma is a WHO grade III ganglioglioma, a rare subtype accounting for a small minority of ganglioglioma cases. Posterior fossa anaplastic ganglioglioma in an adult is incredibly rare; only 3 prior cases have been reported. Only 1 adult anaplastic ganglioglioma in the cerebellum has been reported. We present the second reported adult cerebellar anaplastic ganglioglioma.

Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors involving the gastrointestinal tract, arising from the interstitial cells of Cajal. GIST comprises about 1% of all GI tumors. Rectal GISTs are rare and comprise of approximately 5% of all GISTs and only 0.1% of rectal tumors are found to be GISTs. Rectal GISTs may be diagnosed incidentally or present with symptoms, including defecation problems, bleeding, and/or pain. We report a case of a 46-year-old male with rectal GIST metastasized to the liver and bilateral lung parenchyma managed by Imatinib Mesylate (IM) regimen. Rectal GIST although being rare, must be considered as a differential diagnosis in a patient presenting with defecatory problems with bleeding.

AA is a frequent surgical condition that demands urgent intervention. It accounts for approximately 6% of all emergency department visits. Situs inversus is a rare condition in which the orientation of asymmetric organs is a mirror image of normal anatomy. It can be partial (involving either the abdominal or thoracic cavities) or complete (situs inversus totalis: transposition of both abdominal and thoracic organs). SIT is very rare, with an incidence of 1 per 5000 to 10,000 live births. It is inherited in an autosomal recessive pattern with incomplete penetrance. LSAA is very rare and can happen in association with other congenital abnormalities such as situs inversus, midgut malrotation (MM), or a usually long right-sided appendix projecting into the left lower quadrant. SIT is responsible for greater than 67% of left-sided appendicitis cases. Due to atypical clinical presentation, the diagnosis of AA can be difficult and often delayed. Hence, a complete medical history, physical examination, laboratory tests, and imaging tools are necessary to reach a correct diagnosis in a timely manner and prevent complications like abscesses, perforations, and peritonitis.We report a case of a 50-year-old male with symptoms of left lower abdominal pain along with fever, nausea, vomiting, and loose stools that were later diagnosed as LSAA in the setting of SIT.

Plant cryobanks play a significant role in modern science and breeding. They contribute to the recovery of lost species, the emergence of new plant varieties, and help preserve and explore the diversity of the plant world. The IPPRAS Cryobank collection is constantly supplemented with new samples, while, at the same time, the stored samples are being monitored. In order to test seed germination, seeds of Allium and Veratrum species were thawed. Rare Allium species seeds, such as A. nutans, A. schoenoprasum, and A. victorialis were stored in liquid nitrogen for 17, 19, and 30 years, respectively. Long-term cryopreservation decreased germination rates for A. nutans from 96.55 to 50.00%, for A. schoenoprasum from 72.00 to 62.75%, and for A. victorialis from 90.00 to 83.05%. Seeds of a rare medicinal species, Veratrum lobelianum, were stored in liquid nitrogen for 18 years; the seed germination rate during this storage period has been significantly decreased from 75.00 to 14.81%. V. nigrum seeds were also collected and frozen in liquid nitrogen for 3 days. Short-term cryopreservation did not result in a statistically significant change in germination rates (from 79.71 to 82.69%). The seeds of an endangered ornamental species, Cypripedium calceolus, were collected and kept frozen for 3 days. After cryopreservation, the seeds were planted on three different media, as follows: ½ MS, MS with 10% coconut milk, and BM1. On ½ MS medium, 24.98% seeds formed protocorms, while on MS medium with 10% coconut milk, this number was 10.02%, and on BM1 medium, it was 15.02%, respectively; however, after 2.5 months, all of the protocorms died. Thus, it appears that the existing protocol for seed cryopreservation of C. calceolus needs further improvement. The size, weight, and free water content (WC) of six previously cryopreserved Stipa species and three Allium species were measured. For all the Allium and Stipa species studied, we found no correlation between seed size, WC, and cryotolerance. We also found no correlation between the life form, which reflects the water requirement of the species, and cryotolerance. [ABSTRACT FROM AUTHOR]

Background More than 670 million people have been infected by COVID-19. This case series reports 8 of 55 cases in a broader study of COVID-positive clients who sought homeopathic care for symptoms. Existing studies of homeopathy and COVID-19 have sometimes failed to employ the underpinning theoretical framework of homeopathy—the genus epidemicus. Special focus has been placed on standout symptoms not often reported in conventional medical outlets, known among homeopaths as "strange, rare and peculiar" (SRP) symptoms. The Homeopathy Help Network (HHN) team of practitioners noted SRP symptoms across dozens of cases and studied how they shifted collectively as different variants of the virus emerged. Methods COVID-positive individuals self-selected for individualized care for their symptoms using homeopathy. They received tele-health consultations and individualized homeopathy interventions in an out-patient homeopathy clinical setting. Clients were seen by individual professional homeopathy practitioners and students under supervision working at the HHN in the United States. Cases for the series were hand-picked with the aim of being an average representation of the more than 4,000 COVID-positive cases seen by members of the HHN. Cases in the full compendium are grouped according to a predominant case feature: Multiple remedies, Posology, Time ill, Single remedy resolution, Hospitalization and, in this case series, SRP symptoms. Results SRP symptoms included: continually on the verge of unconsciousness; dark green stools; very low pulse alternating with tachycardia; sensation of strong or burning chemical smells; sensation of inhaling water through the nose; recurring electric shock sensations in head or extremities; yellow-green stools. Conclusion Collective SRP symptoms from the pandemic provided the opportunity to study the hallmark features of COVID-19 in depth. The importance of these symptoms highlights the applicability of Hahnemannian principles and good case-taking practices. [ABSTRACT FROM AUTHOR]

Illnesses that afflict a tiny number of individuals are referred to as rare diseases (RDs), sometimes called orphan diseases. The local healthcare systems are constantly under financial, psychological, and medical strain due to low incidence rates, unusual presentations, flawed diagnostic standards, and a lack of treatment alternatives for these RDs. The effective management of the once widely spread viral hepatitis B and C has altered the spectrum of liver diseases in Egypt during the last several years. The detection of uncommon disorders such as autoimmune, cholestatic, and hereditary liver diseases has also been made easier by the increasing knowledge and greater accessibility of specific laboratory testing. Finally, despite Egypt's large population, there are more uncommon liver disorders than previously thought. This review article discusses the clinical and epidemiological characteristics of a few uncommon liver disorders and the information currently accessible concerning these illnesses in Egypt. [ABSTRACT FROM AUTHOR]

In an era of cost pressure, substituting generic drugs represents one of the main cost-containment strategies of healthcare systems. Despite the obvious financial benefits, in a minority of cases, substitution may require caution or even be contraindicated. In most jurisdictions, to obtain approval, the bioequivalence of generic products with the brand-name equivalent needs to be shown via bioavailability studies in healthy subjects. Rare diseases, defined as medical conditions with a low prevalence, are a group of heterogenous diseases that are typically severe, disabling, progressive, degenerative, and life-threatening or chronically debilitating, and disproportionally affect the very young and elderly. Despite these unique features of rare diseases, generic bioequivalence studies are typically carried out with single doses and exclude children or the elderly. Furthermore, the excipients and manufacturing processes for generic/biosimilar products can differ from the brand products which may affect the shelf-life of the product, its appearance, smell, taste, bioavailability, safety and potency. This may result in approval of generics/biosimilars which are not bioequivalent/comparable in their target population or that meet bioequivalence but not therapeutic equivalence criteria. Another concern relates to the interchangeability of generics and biosimilars which cannot be guaranteed due to the phenomenon of biocreep. This review summarizes potential concerns with generic substitution of orphan drugs and discusses potentially problematic cases including narrow therapeutic index drugs or critical conditions where therapeutic failure could lead to serious complications or even death. Finally, we put forward the need for refining regulatory frameworks, with emphasis on Saudi Arabia, for generic substitution and recent efforts toward this direction. [ABSTRACT FROM AUTHOR]

The genus Alstonia belonging to the Apocyanaceae family is represented by 44 species distributed worldwide. In India, the taxon is represented by eight species, reported from Andaman & Nicobar Islands, Western Ghats, Eastern Ghats, western Himalaya, and northeastern regions. The present study reported the occurrence of Alstonia sebusii from Pudunamei village, Mao, a new addition to the flora of Manipur. Detailed morphological descriptions and measurements were recorded based on living plant specimens. The plant is locally called 'Topfiira Koso Pro' having ethno-medicinal properties and widely been used by local people of the state to treat various ailments. The plant is rare and found to occur at specific location at Pudunamei village. [ABSTRACT FROM AUTHOR]

Objective To explore the clinical characteristics, imaging findings, and treatment methods of intracranial pure arterial malformations (PAMs). Methods A retrospective analysis was conducted on a typical patient with bilateral anterior cerebral artery (ACA) of intracranial PAMs admitted to the neurosurgery department of our hospital. Imaging data and clinical manifestations from years of follow-up were used to report and review the literature, in order to provide a basis for the diagnosis and treatment of intracranial simple arterial malformation. Results Cerebral angiography showed abnormal tortuous, dilated, and coiled arterial rings in the A2 segment of the bilateral ACA, and no clear signs of venous drainage were observed during the venous phase. Therefore, symptomatic pain relief treatment was given and patients were discharged. Conclusions For patients with ruptured bleeding, compression effects caused by intracranial PAMs (obstructive hydrocephalus, cranial nerve paralysis), and blood flow related aneurysms, active surgical intervention should be taken in clinical practice. For simple arterial malformations without these characteristics, regular imaging and clinical follow-up should be chosen as conservative treatment strategies. [ABSTRACT FROM AUTHOR]

Fibroepithelial polyps (FEPs) of the urinary tract are a rare benign tumour in the bladder. It arises from mesoderm and is mostly found in the ureter and ureteropelvic region. The authors report a case of a six-year-old girl with no history of urogenital abnormality who presented with haematuria and suprapubic pain. Ultrasound and computed tomography showed a large multilobulated mass in the urinary bladder. The resected specimen exhibited a bulbous mass with a botryoid surface. Microscopically, the polypoidal lesion is covered by reactive transitional epithelium with the presence of cystitis cystica et glandularis with scattered spindle cells; fibroblast and smooth muscle cells. By excluding all the differential diagnoses according to histomorphology and immunostain findings, diagnosis of Giant Botyroid FEP is made. This paper also discussed the differential diagnosis for FEP. [ABSTRACT FROM AUTHOR]

This review provides a comprehensive overview of rare causes of pediatric anaphylaxis related to obscure allergens. Anaphylaxis, a severe hypersensitivity reaction, can occur without typical symptoms, posing diagnostic challenges, especially in children. Idiopathic anaphylaxis, where no trigger is identified despite thorough evaluation, is notably challenging in this population. This review synthesizes current literature, highlighting obscure triggers such as food additives, spices like fenugreek, and cross-reactive allergens, including lupine and gelatin. These allergens are often overlooked and can lead to misdiagnosis of idiopathic cases. Understanding these uncommon triggers is crucial for clinicians to ensure accurate diagnosis and effective management of pediatric anaphylaxis, emphasizing the need for heightened clinical awareness and further research. This review raises awareness among health care providers about these lesser-known causes, aiming to improve outcomes and quality of life for pediatric patients at risk of anaphylactic reactions.

Spontaneous regression of testicular tumors known as burnt-out tumor of testis is a rare entity and is seen in about 5% of testicular germ cell tumors. It is described as a spontaneously, completely, or partially regressed testicular tumor with or without metastasis in the absence of any treatment. This entity is now included in the 2016 classification of testicular tumors. This is probably only the second case presented in India. High degree of awareness of this entity by the pathologist, clinician, and radiologist is necessary for proper management and survival of the patient.

Summary: Structural variations (SV) are large (>50 base pairs) genomic rearrangements comprising deletions, duplications, insertions, inversions, and translocations. Studying SVs is important because they play active and critical roles in regulating gene expression, determining disease predispositions, and identifying population-specific differences among individuals of diverse ancestries. However, SV discoveries in the Indian population using whole-genome sequencing (WGS) have been limited. In this study, using short-read WGS having an average 42X depth of coverage, we identify and characterize 36,210 SVs from 529 individuals enrolled in population-based cohorts in India. These SVs include 24,574 deletions, 2,913 duplications, 8,710 insertions, and 13 inversions; 1.26% (456 out of 36,210) of the identified SVs can potentially impact the coding regions of genes. Furthermore, 56 of these SVs are highly intolerant to loss-of-function changes to the mapped genes, and five SVs impacting ADAMTS17, CCDC40, and RHCE are common in our study individuals. Seven rare SVs significantly impact dosage sensitivity of genes known to be associated with various clinical phenotypes. Most of the SVs in our study are rare and heterozygous. This fine-scale SV discovery in the underrepresented Indian population provides valuable insights that extend beyond Eurocentric human genetic studies.

Introduction: Glomus tumors are rare benign neurovascular tumors derived from the neuromyoarterial glomus apparatus. They are more frequent in the hand and their cause is unknown. The purpose of this study was to describe a case series of patients who presented with glomus tumors in unusual body parts, in a non-subungual location. Case series: Three cases, from three different Institutions, were reported. All patients were male presenting with lesions characterized as a painful and solitary nodule in unusual areas, specifically in the left lower extremity: one in the calf, the other in the knee, and the third in the thigh. The nodules were not palpable, but with severe induced pain when touched, and with temperature sensitivity relation. Further investigation using Magnetic Resonance Imaging showed low signal intensity on T1 and high signal intensity on T2-weighted images. Ultrasound scanning was also a method to investigate and support the diagnosis. All patients underwent a total surgical resection, and histopathologically confirmed the diagnosis of glomus tumors, revealing oval and circular cell proliferation in association with vascular structures. There was no recurrence reported. Conclusion: Glomus tumors are a rare entity characterized by pain, point tenderness, and temperature sensitivity, particularly where the lesion is located. Clinical history and physical examination invariably clinch the diagnosis. To ensure the correct diagnosis and treatment of glomus tumors, it is essential to have a complete inventory of imaging resources, particularly for differential diagnosis, when dealing with tumors in unusual locations.

Abstract Vagococcus fluvialis infection is rare in humans, and there is limited research on the clinical manifestations and antimicrobial susceptibility testing of Vagococcus fluvialis infection. Here, We isolated Vagococcus fluvialis from the urine samples of bladder cancer patients at Hunan Provincial People’s Hospital, and it is the first reported case of Vagococcus fluvialis isolated from the urine. The fully automated microbial identification system and matrix-assisted laser desorption/ionization time-of-flight mass spectrometry (MALDI-TOF MS) identified the bacterium as Vagococcus fluvialis with a confidence level of 99.9%. The VITEK-2Compact fully automated microbial susceptibility analysis system indicated that it was most sensitive to tigecycline, vancomycin, quinupristin/dalfopristin, linezolid, and showed moderate sensitivity to erythromycin, levofloxacin, ciprofloxacin, ampicillin/sulbactam, and tetracycline. Additionally, it exhibited synergy when combined with high-level gentamicin and vancomycin, showing sensitivity. However, it displayed poor activity against penicillin and furanth. According to our knowledge, this is the first study to isolate and identify Vagococcus fluvialis from the urine of bladder cancer patients and the systematically reviewed other reported Vagococcus infections on human, which provide an experimental basis for guiding the rational use of drugs in the clinical treatment and diagnose of Vagococcus fluvialis infection and related pathogenic mechanism research. Meanwhile, we have systematically reviewed other reported.

Abstract Background Inflammatory myofibroblastic tumor (IMT) is an uncommon cardiac tumor that primarily affects infants, children, and young adults. While complete surgical resection generally leads to a favorable prognosis, accurate diagnostic tests remain limited. Case presentation We describe the case of a 26-year-old female who had a dual tumor inside and outside the heart and was misdiagnosed by echocardiography and MRI. We also review 71 cases of cardiac IMTs from the literature regarding their epidemiology, clinical presentation, and outcome. Conclusion Early detection of this rare disorder is essential for optimal surgical management.

Abstract Background The most prevalent cyanotic congenital heart disease is Tetralogy of Fallot (TOF). It has a variety of presentations and is made up of four anatomic abnormalities. Documented literature shows an incidence of 13–20% of a right aortic arch with an anomalous left subclavian artery among individuals diagnosed with TOF. This is the first case that discusses the rare occurrence of overriding of the aortic arch along with the left aberrant subclavian artery and vertebral defect in a 3-week-old girl. Timely identification and management are pivotal in ensuring the best possible outcomes for these young patients. Case presentation A 3-week-old female child came with complaints of dyspnea, dysphagia, fatigue, and cyanosis on extreme crying, feeding, and moderate activity. Echocardiography revealed severe pulmonary stenosis with right ventricular dilatation and ventricular septal defect (VSD); a chest computed tomography was performed that revealed four characteristic features of TOF (pulmonary artery stenosis, VSD, right aortic root deviation, and concentric right ventricular hypertrophy) along with overriding of the aortic arch accompanied with the left aberrant subclavian artery (compressing the trachea and infundibulum) and vertebral defect (butterfly vertebra). Conclusions The case of this 3-week-old female infant emphasizes the importance of early and accurate diagnosis in congenital heart diseases, particularly when faced with complex presentations such as the TOF. It serves as a testament to the valuable role of advanced diagnostic imaging techniques in unraveling the complexity of congenital heart conditions.

Shewanella putrefaciens is an opportunistic pathogen rarely responsible for human infection. However, it has been reported that it causes skin and soft tissue infections and bacteremia in immune-compromised patients, such as cellulitis, abscesses, bacteremia, and wound infection. It is an oxidase and catalase-positive non-fermenter gram-negative rod that produces hydrogen sulfide. We report the case of a 90-year-old woman, who presented an invasive infectious burn wound associated with Shewanella putrefaciens bacteremia. She was admitted into the burn center of the military hospital M.S Nekkache of Algiers, suffering from 40% TBSA with a history of diabetes. After one week of admission, the patient complained of a high fever. Microbiological culture of the catheter tip was positive and showed pale colonies on the MacConkey agar, non-lactose fermenting plate. Nutritive agar medium culture showed red pale tan colonies with a concentration >10° CFU. Identification and antibiotic susceptibility were obtained by the Phoenix system (Becton-Dickinson, USA) as Shewanella putrefaciens. This was confirmed by standards and semi-automated microbiological techniques. Gram stain showed Gram-negative bacilli with positive oxidase and catalase reactions. Production of hydrogen sulfide was confirmed by the semi-automated API 20NE method (biomerieux, France). The isolate was resistant to gentamicin, amikacin, ceftazidime, aztreonam, amoxicillin-clavulanic acid, cefepime, trimethoprim/sulfamethoxazole, and nitrofurantoin. In our case, S. putrefaciens was found in a mixed culture with Klebsiella pneumoniae. No earlier exposure of the patient to marine water had been noticed. Blood culture indicated colonies growth of Acinetobacter baumannii. No further isolation of this bacteria was noticed after treatment. The patient was given imipenem, vancomycin and colistin. Despite our best efforts, the patient could not be saved because of sepsis and renal function failure. [ABSTRACT FROM AUTHOR]

Vagococcus fluvialis infection is rare in humans, and there is limited research on the clinical manifestations and antimicrobial susceptibility testing of Vagococcus fluvialis infection. Here, We isolated Vagococcus fluvialis from the urine samples of bladder cancer patients at Hunan Provincial People's Hospital, and it is the first reported case of Vagococcus fluvialis isolated from the urine. The fully automated microbial identification system and matrix-assisted laser desorption/ionization time-of-flight mass spectrometry (MALDI-TOF MS) identified the bacterium as Vagococcus fluvialis with a confidence level of 99.9%. The VITEK-2Compact fully automated microbial susceptibility analysis system indicated that it was most sensitive to tigecycline, vancomycin, quinupristin/dalfopristin, linezolid, and showed moderate sensitivity to erythromycin, levofloxacin, ciprofloxacin, ampicillin/sulbactam, and tetracycline. Additionally, it exhibited synergy when combined with high-level gentamicin and vancomycin, showing sensitivity. However, it displayed poor activity against penicillin and furanth. According to our knowledge, this is the first study to isolate and identify Vagococcus fluvialis from the urine of bladder cancer patients and the systematically reviewed other reported Vagococcus infections on human, which provide an experimental basis for guiding the rational use of drugs in the clinical treatment and diagnose of Vagococcus fluvialis infection and related pathogenic mechanism research. Meanwhile, we have systematically reviewed other reported. [ABSTRACT FROM AUTHOR]

Background: The most prevalent cyanotic congenital heart disease is Tetralogy of Fallot (TOF). It has a variety of presentations and is made up of four anatomic abnormalities. Documented literature shows an incidence of 13–20% of a right aortic arch with an anomalous left subclavian artery among individuals diagnosed with TOF. This is the first case that discusses the rare occurrence of overriding of the aortic arch along with the left aberrant subclavian artery and vertebral defect in a 3-week-old girl. Timely identification and management are pivotal in ensuring the best possible outcomes for these young patients. Case presentation: A 3-week-old female child came with complaints of dyspnea, dysphagia, fatigue, and cyanosis on extreme crying, feeding, and moderate activity. Echocardiography revealed severe pulmonary stenosis with right ventricular dilatation and ventricular septal defect (VSD); a chest computed tomography was performed that revealed four characteristic features of TOF (pulmonary artery stenosis, VSD, right aortic root deviation, and concentric right ventricular hypertrophy) along with overriding of the aortic arch accompanied with the left aberrant subclavian artery (compressing the trachea and infundibulum) and vertebral defect (butterfly vertebra). Conclusions: The case of this 3-week-old female infant emphasizes the importance of early and accurate diagnosis in congenital heart diseases, particularly when faced with complex presentations such as the TOF. It serves as a testament to the valuable role of advanced diagnostic imaging techniques in unraveling the complexity of congenital heart conditions. [ABSTRACT FROM AUTHOR]

Background: Inflammatory myofibroblastic tumor (IMT) is an uncommon cardiac tumor that primarily affects infants, children, and young adults. While complete surgical resection generally leads to a favorable prognosis, accurate diagnostic tests remain limited. Case presentation: We describe the case of a 26-year-old female who had a dual tumor inside and outside the heart and was misdiagnosed by echocardiography and MRI. We also review 71 cases of cardiac IMTs from the literature regarding their epidemiology, clinical presentation, and outcome. Conclusion: Early detection of this rare disorder is essential for optimal surgical management. [ABSTRACT FROM AUTHOR]

Copyright of Anadolu (1300-0225) is the property of Anadolu Dergisi and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Introduction: Glomus tumors (GTs) of the trachea are a rare soft tissue disease, originating from modified smooth muscle cells. Patients are usually presented with nonspecific symptoms such as cough, dyspnea or hemoptysis. Surgical treatment has been reported, including thoracotomy and bronchoscopic electro-cautery, there is no doubt that surgery is the treatment of choice, however few studies have evaluated the ideal treatment for tracheal GTs. Case Report: We describe a case of glomus tumor originated at large airway, a 62-year-old woman who presented with cough, and expectoration for 7 days, had been diagnosed as having a tracheal tumor by thoracic computed tomography. Tumor resection was attempted by fibe)roptic bronchoscopy but there were positive edges, therefore surgical tracheal resection and anastomosis were performed. The patient achieved good results and no recurrence was observed during a 10-month follow-up. This is the third report of glomus tumor of the tracheal originated at large airway (trachea and right main bronchus). [ABSTRACT FROM AUTHOR]

Background: Congenital cholesteatomas (CCs) are rare epidermal cysts that have been reported to occur most commonly in the middle ear and rarest in the mastoid cavity. They usually present in adulthood and are difficult to detect due to the lack of symptoms. Treatment is usually a canal wall up or down mastoidectomy with good success rates. Case description: We present a rare case of a mastoid CC with posterior external auditory canal wall erosion treated successfully with a simple canal wall-up mastoidectomy. Conclusion: Mastoid CCs still remain a rare entity with a relatively uncomplicated surgical approach. Clinical significance: Clinicians should high index of suspicion and a low threshold for a CT scan in patients with a history of an intermittently discharging ear, a narrowed ear canal, or a posterior canal wall fistula. [ABSTRACT FROM AUTHOR]

KONAR-MFO (multifunctional occluder) [Lifetech, Shenzhen, China] was first used in humans in 2013 and received the CE mark in May 2018. As name suggest, it can be use in various(multifunctional) situations in paediatric as well as adults. This is a versatile device with an improved delivery and flexibility which make this device a better option to be use with more comfort and minimum complications. This paper is regarding few rare and complicated lesions, like anomalous origin of coronary artery from pulmonary artery (ALCAPA) device closure, device closure of ventricular septal rupture (VSR) post myocardial infarction in sick elderly and finally device closure of paravalvar leak (PVL)after mitral valve replacement which were treated with this device with excellent results. This study is a retrospective review from a tertiary level dedicated cardiac referral centre in south India. Three cases we are reporting here in which Konar-multifunctional occluders were used in locations other than commoner and regular defects like patent ductus arteriosus, ventricular septal defect etc, over the span of one year between April 2022 and March 2023. Pre-procedure, all patients underwent detailed clinical evaluation followed by transthoracic echocardiography, 12-lead electrocardiogram, and Chest X-ray at the outpatient department. All patients were either symptomatic or had a hemodynamically significant lesion on echocardiography. The decision for transcatheter management was taken after discussing with surgical team in view of either high risk surgery or refusal from patients for surgical options. All patients were followed up post procedure at regular intervals with transthoracic echocardiograms and 12-lead electrocardiograms for a minimum period of 6 months. All these three cases mentioned in our study underwent a complete closure of their respective lesions with no evidence of residual shunt. None of these patients had any major complications, prolonged stay, or any vascular injuries. All patients completed minimum 6-month follow-up and were doing well without any residual flows. First case of ALCAPA, after procedure showed improvement in IVCD, QTc duration and also no significant ischemic changes were noted. Myocardial perfusion scintigraphy was done after 6 months of procedure which showed improvement in contractile function and perfusion of left ventricle. Second case of VSR device closure patient showed immediate significant symptomatic improvement. He was transferred to the coronary care unit, and discharged seven days later. As of now the patient is alive and feeling well with no residual shunt detected by transthoracic echocardiography. No procedure -related complications have been recorded during last two years. Third case of PVL device closure had uneventful recovery from anaesthesia. Prosthetic valve functioned normally during the 5 days of post-procedure hospitalization. The transthoracic 2D- echocardiography performed during follow-up at the end of one month showed no mitral PVL.During follow-up after 1 year, the patient improved symptomatically. Normal prosthetic valve function and no leakage documented on transthoracic echocardiography. Konar-MFO emerging as an important occluder with interesting attributes which makes it a very useful asset to have in catheterizations laboratory. [ABSTRACT FROM AUTHOR]