case report

Background: Uterine inversion is a rare obstetric emergency but potentially life-threatening condition. If these are not immediately diagnosed, the massive and underestimated blood loss can lead to hypovolemic shock. Case: Case of 24 years old woman was referred from the district Public Health Center with vaginal bleeding after delivery with abdominal pain. We found fundal height postpartum is 3 fingers above symphysis and confirmed a soft mass protruding inside the vagina at the local examination. The ultrasound cannot present the fundus of the uterus and impressed a uterine inversion. Stabilization for the patient is done with fluid resuscitation and blood transfusion. The patient planned for exploratory laparotomy and we confirmed uterine inversion. We did per abdominal reposition success which was managed by Haultain’s method. Conclusion: Uterine inversion should be considered in any patient with symptoms of hemorrhage and abdominopelvic pain, with the physical findings of a soft, congested, bleeding mass within the vagina on bimanual examination. Prompt treatment consists of fluid resuscitation and anatomical repositioning is needed.

Pneumopericardium is a rare clinical entity, occurring in the setting of thoracic trauma, malignancies, or mechanical ventilation. Very few cases report pneumopericardium as a complication of gastrointestinal tract surgery. Signs and symptoms may be frustrating, ranging from asymptomatic to chest pain, sepsis, hemodynamic instability, pericarditis, or even cardiac tamponade. Clinical pathognomonic signs of pneumopericardium include pericardial metallic tinkling friction rub and mill wheel murmur. Diagnostic work-up includes electrocardiogram, chest radiography, and, computed tomography imaging. A gastro pericardial fistula should be considered a rare differential diagnosis for acute chest pain in patients with a history of gastroesophageal surgery. Rapid recognition and treatment avoid life-threatening complications. The successful outcome of gastro pericardial fistula treatment depends on both emergency and definitive surgical management. The survival rate with conservative management is poor.We present the case of a 78-year-old patient suffering from pneumopericardium and pericardial infusion, due to a fibrotic fistula between the Nissen’s valve, occurring 10 years after redo antireflux surgery. Treatment included broad-spectrum antibiotics, and emergency surgery for pericardial drainage, biopsy of the valve’s defect, suture, and omentoplasty.

The vast majority of urological complications occur at the ureterovesical junction and usually occur early after transplantation. The aim of this study is to enhance the quality of medical care provided to patients who undergo kidney transplantation. Cystography was conducted on renal transplant recipients utilizing computed tomography. The utilization of changes in the patient’s position and reconstructed images of the bladder can serve as a diagnostic tool to assess the normal functioning of the urinary tract system subsequent to kidney transplantation. To ensure adequate filling of the bladder and ureter, it is necessary to introduce varying amounts of contrast medium through the urinary catheter into these structures. This diagnostic procedure aims to verify the existence of stenosis or leakage occurring at the vesicoureteral junction. The evaluation and diagnosis of urinary tract problems subsequent to kidney transplantation can be effectively conducted. Furthermore, it has the potential to mitigate the adverse effects and alleviate the strain on the renal system resulting from the administration of contrast agents in computed tomography urography. CT-guided cystography can enhance the medical quality and comfort of Kidney transplantation patients.

This case suggests that POEMS syndrome does present with a constellation of signs and symptoms that may lead a clinician to a multitude of other possible diagnoses. Diagnosis is often difficult and delayed. A good history and physical examination as well as a careful review of all workups are paramount in establishing this particular diagnosis. The major criteria of diagnostic for the syndrome are polyradiculoneuropathy, clonal plasma disorder, sclerotic bone lesions, the presence of Castleman disease, and elevated vascular endothelial growth factor. Minor features include organomegaly, extravascular volume overload, endocrinopathy, characteristic skin changes, papilledema, and thrombocytosis. The diagnosis is made with three of the major criteria, two of which must include polyradiculoneuropathy and clonal plasma cell disorder, and at least one of the minor criteria. In this article, we discuss the differential diagnosis and outline the clinical evaluation indicated.

Introduction: Thalassemia is an inherited blood disorder of haemoglobin (Hb) synthesis, which affects different regions around the world. India has the largest number of children with beta-thalassemia major in the world, particularly in the tribal population. Heterozygous conditions are milder and even go unreported than the condition of homozygous where regular blood transfusion is required.Case report: This report focuses on a case of major beta-thalassemia in a child, whose parents are beta thalassemia minor to intermediate conditions, and who was treated by blood transfusion once a month. However, Thalassemia may be cured by allogeneic hematopoietic stem cell transplantation, although not everyone is a good candidate. Genetic counselling, prenatal diagnosis, and selective termination of affected fetuses are effective ways to control thalassemia.Discussion and conclusion: The paper reports a unique case of Thalassemia in rural India. The blood disorder while commonly presented in a juvenile whose parents were Thalassemia positive resulted in the termination of a fetus diagnosed with it. It archives the story of the parents who are now in the process of planning future offspring while mitigating disease risk. The case leads the way for effective management and containment of hereditary genetic disorders through carrier detection while planning alliances and offspring.

Despite decades of global and country commitments towards eradicating malaria, malaria remains the most hazardous parasitic disease and the most common cause of fever for humans, especially in tropical countries. Plasmodium falciparum causes 90% of malaria cases. Coma [Cerebral Malaria (CM)], acidosis, hypoglycemia, severe anemia, renal dysfunction, and pulmonary edema are the most common complications of malaria caused by Plasmodium falciparum and the most common cause of death related to malaria. People from less prevalent malaria areas are at high risk of developing these complications. A 16-year-old male from a low malaria transmission area was diagnosed with CM. Prior to developing CM, he was treated with Coartem. CM is a medical emergency and one of the forms of severe malaria. CM has high mortality and morbidity rates. Yet, international health-related agencies, funders, and policy-makers are unfamiliar with it. The continuous occurrence of CM validates the considerable need for global investment in malaria control and elimination programs. Early administration of Artesunate to all patients suspected of having severe malaria would reduce global malaria-related mortality and morbidity. Simple tests, such as the determination of malaria parasitic density either with thin or thick blood smears, may influence the proper management of all severe malaria cases. However, in clinical practice, the determination of malaria parasitic density is not routinely done. Further commitments are needed to ensure routine determination of malaria parasitic density for all suspected severe malaria cases. Moreover, further commitments are needed to guarantee the proper management of CM because it is a major cause of reversible encephalopathy in tropical countries.

Orbital melanoma is a subtype of periocular melanoma that can present from primary, secondary (arising from local invasion), or metastatic disease [1]. Melanoma metastasis to the orbit is rare with the majority of metastases occurring in subcutaneous tissue, nonregional lymph nodes, lungs, liver, brain, and bone [2]. Despite melanoma being relatively radioresistant, radiation therapy can be considered in an adjuvant or palliative setting [3]. In the palliative setting specifically, radiation therapy is highly effective in alleviating symptoms due to mass effect [3]. However, significant ocular and orbital complications may occur as a direct result of radiation therapy.

A 46-year-old lady was diagnosed clinically with X-linked hypophosphatemia (XLH) with a rare pathogenic variant detected using exome sequencing. Phosphate-regulating endopeptidase homologous X linked (PHEX) is normally expressed in osteoblasts and osteocytes, and senses phosphate regulation. More than 1000 PHEX variants have been detected to date, which are caused by missense, nonsense, and frameshift mutations in addition to splice variants and copy number changes. The aberration in the PHEX gene leads to the upregulation of fibroblastic growth factor 23 (FGF23), which leads to defects in phosphate metabolism. This results in impaired bone growth and mineralization, short and disproportionate stature, leg bowing, musculoskeletal pain, spontaneous dental abscesses, rickets, and osteomalacia in XLH patients. The spectrum of manifestations differs between pediatric and adult patients. In our case study, two of the patient’s children started showing symptoms at a younger age, unlike their mother. Timely diagnosis and the start of treatment would help in their better management and improved quality of life.

Background: Although there is minimal information on the role of stress in PCOS, it is well-known that it may trigger the exacerbation and maintenance of the disease. Despite there being only a few studies in the literature, many researchers highlighted situations of autonomic hyperactivation characterizing PCOS. In light of these assumptions, the purpose of this study is to report on a patient who underwent psychological intervention to enhance stress management skills.Case: A 30-year-old woman was referred by the endocrinologist. From a clinical-psychological point of view, the condition of the patient was characterized by a modest psychophysiological activation. Interpersonal difficulties did not allow the patient to benefit from the social support of her loved ones, further favoring the maintenance of the excessive arousal generated by stressful life events. The patient was treated with HRV-Biofeedback integrated within cognitive-behavioral psychotherapy.Discussion: The multidimensional intervention brought benefits to the patient, teaching her better stress management strategies (i.e., reduction of psychological symptoms and improvement of hormonal tests). Keeping in mind the interplay between physiological, psychological, and interpersonal factors is fundamental in all psycho-somatic and somato-psychic disorders and is crucial to enhance the use of specific treatments to restore psychophysical well-being.

Introduction: Factor VII (FVII) deficiency, a rare bleeding disorder, can manifest as an autosomal recessive congenital or an acquired coagulopathy. Acquired FVII deficiency, although infrequently reported, presents unique challenges in understanding its mechanisms and identifying underlying causes. Case presentation: We present a case of acquired FVII deficiency discovered in a 23-year-old female patient with no apparent underlying disease. The patient exhibited spontaneous ecchymosis and gingival hemorrhage, along with low FVII activity and isolated prolongation of prothrombin time. Extensive laboratory investigations excluded liver dysfunction, familial deficiency, vitamin K deficiency, and inhibitory antibodies. Prompt treatment with Fresh Frozen Plasma (FFP) and bypassing agents resulted in a favorable response and resolution of hematomas. Conclusion: Acquired FVII deficiency was identified with bleeding symptoms in association with prolonged prothrombin time and a low level of FVII activity. In literature, this deficiency has been associated with various conditions such as sepsis, aplastic anemia, stem cell transplantation, and neoplasms, although approximately 14% of cases remain idiopathic. Clinical outcomes remain generally poor, with limited complete remissions reported.

As the introduction of immune checkpoint inhibitors in the treatment of various cancers is now proven to be already acquired knowledge, so does a new challenge arise for clinicians; the understanding, diagnosis, and management of the rarest adverse effects of immunotherapy. We present a case of type-1 diabetes Mellitus (T1DM) in a patient with non-small cell lung carcinoma (NSCLC) treated with pembrolizumab. Following ten cycles of treatment, our patient was diagnosed with T1DM after being admitted for diabetic ketoacidosis and stayed hospitalized in the ICU. Later, they continued treatment with insulin, having shown disease response to pembrolizumab, and resumed immunotherapy while on insulin. Immunotherapy-induced T1DM can sometimes occur with PD1/PD-L1 blockage therapies. It has a rapid onset, is characterized by insulin deficiency due to the autoimmune destruction of beta-cells, and usually presents itself with diabetic ketoacidosis. Unlike most of the other adverse effects of immunotherapy, glucocorticoids don’t seem to be of therapeutic value, and insulin substitution is required. Regular glucose monitoring can be key to early diagnosis and prevention of hospitalization. 

“A 40-year-old woman with melanoma, under treatment with Dabrafenib and Trametinib, was evaluated in our hospital for rapidly progressive deterioration of renal function”. 8 months before the current admission, the patient had been diagnosed with melanoma, and underwent radical surgery and subsequent therapy with Dabrafenib and Trametinib.After 5 months of therapy, the patient was brought to this hospital for precordial pain, with a diagnosis of myopericarditis, therapy was started for heart failure with a good response. However, the patient developed a progressive impairment of renal function, associated with hemolytic anemia and thrombocytopenia. The peripheral smear showed the presence of schistocytes.The suspicion of atypical Hemolytic Uremic Syndrome (aHUS) was confirmed by the assay of C5B-9 induced by serum on endothelial cells, which showed a deposition of 331%, treatment with Eculizumab was initiated.After 3 administrations the patient did not improve, with further worsening of the hemolytic condition, and progression of renal damage.Due to the failure of Eculizumab, we considered the use of Ravulizumab. However, in Italy only can be administered to patients in Eculizumab stable treatment for at least three months. Nevertheless, faced with the catastrophic condition, it was decided to shift the therapy and use off-label Ravulizumab. After 10 days of the first administration, the laboratory tests showed a continuous rise in the values of haptoglobin, platelets, and hemoglobin, and a decrease in LDH. The renal function failed to return to normal values but after 20 days of therapy with Ravulizumab, there was complete resolution of the hemolytic condition.

Hypertrophy of the adenoid is a rare condition in adults, often suspicious of malignancy. We present a case of a 31-year-old female with a clinical presentation of a giant nasopharyngeal mass, clinically suspicious for malignancy, given the size and greyish discoloration. She presented with left-side otalgia, hearing loss, and nasal obstruction. After broad investigations on adenoid tissue following adenectomy, a reassuring diagnosis of adenovirus-related adenoiditis could be made. This case demonstrates the importance of broad microbiological testing in ruling out malignancies. The patient recovered completely. 

The prevalence of intra-uterine dysfunction of ductus arteriosus is unknown and the clinical consequences are poorly understood. We report a case of prenatal diagnosis of premature closure of the ductus arteriosus due to maternal intake of metamizole during pregnancy. Fetal echocardiography at 37 weeks of gestation revealed a right ventricular hypertrophy and suspected stenosis of the tricuspid valve. A cesarean section led to an excellent neonatal outcome. The aim of this report is to show echocardiographic abnormalities and outcomes of this rare phenomenom.

Vasculitis is a group of rare but potentially serious disorders characterized by inflammation of blood vessels. This condition can affect blood vessels of all sizes, ranging from small capillaries to larger arteries. 

Background: Congenital teratomas are relatively rare neoplasms, which occurs in about 1:20,000 to 1:80,000 births, and only 1.5% to 5% of which are neoplasm of the cervical. They can be diagnosed through ultrasound during pregnancy and, if not properly handled, have a high mortality rate. Airway compression is a secondary complication following mortality. Case report: A solid-cystic mass was identified in the anterior cervical region of a 30-week-old fetus during an ultrasound scan. EXIT (Ex-Utero Intrapartum Treatment)-to-airway procedure was performed by a multidisciplinary team composed of obstetricians, anesthesiologists, neonatologists and pediatric surgeons to remove the neoplasm. The procedure occurred upon delivery of the fetus, resulting in a positive outcome with neonatal survival. In this case, the fetus was in breech position, and, differently from the usual EXIT protocol, it had to be completely extracted before guaranteeing airway flow. Conclusion: Although congenital teratomas are a rare condition with complex treatment, it is possible to achieve a satisfactory outcome when adequate planning and protocol are established.

Background: Transcatheter Aortic Valve Replacement (TAVR) is an established treatment for severe, symptomatic Aortic Stenosis (AS). However, the presence of low coronary heights confers a high risk for coronary obstruction during or after TAVR. Case: In this case report, we present our experience with transfemoral-TAVR in an elderly, high-risk (STS score – 12.08%) female severe AS patient with low coronary heights (right: 7.4 mm, left: 8.7 mm). She had lower annulus area (287 mm2) and moderately low valve area (0.7 cm2) as well. Her mean and peak pressure gradients (PGs) were 38 mmHg and 61 mmHg, respectively. Upon the Heart Team’s evaluation, TAVR was recommended and a 20 mm Balloon Expandable (BE) Myval Transcatheter Heart Valve (THV) was selected. No peri-procedural or post-procedural complications were reported and the post-procedural hemodynamics, namely the mean and peak PGs improved to 16 mmHg and 30 mmHg after TAVR, respectively. The patient was discharged in a stable condition after four days of hospital stay.Conclusion: We report the successful implantation of a small-sized BE Myval THV (20 mm) in a patient with low coronary heights. Life-threatening complications including paravalvular leak, coronary obstruction, or annular rupture were well averted; hence, we ascertain that the Myval THV is a suitable device for treating severe AS in difficult anatomies. However, the viability of the novel valve needs to be reaffirmed in larger studies.. 

Diffuse pediatric-type high-grade glioma H3-wildtype and IDH-wildtype (pHGG H3/IDH WT) is a heterogeneous entity that is currently defined by a combination of highly malignant morphology, glial or primitive neuroectodermal differentiation, and a number of molecular features. Depending on the DNA methylation profile in pHGG H3/IDH WT, three molecular subgroups are distinguished, one of which (pHGG MYCN) is characterized by amplification of the indicated gene. We report a unique case of pHGG H3/IDH WT in a 19-year-old girl with a deletion of the MYCN gene and constitutional mismatch repair deficiency syndrome.

Mesenteric cysts and cystic mesenteric tumors are very rare abdominal growths. They may be localized all over the mesentery, from the duodenum to the rectum, however, they are mostly found in the ileum and right colon mesentery. There are several classifications of these formations, among which the one based on histopathologic features including 6 groups has been most commonly used: 1) cysts of lymphatic origin--lymphatic (hilar cysts) and lymphangiomas; 2) cysts of mesothelial origin--benign or malignant mesothelial cysts; 3) enteric cysts; 4) cysts of urogenital origin; 5) dermoid cysts; and 6) pseudocysts-infectious or traumatic etiology.A dermoid cyst is a benign, epithelial-lined cavitary lesion composed of ectoderm and mesoderm that can arise anywhere in the body, with a tendency to develop in midline structures.In 20% - 45% of cases, accompanying diseases that were not previously clinically manifested can be diagnosed during emergency interventions, requiring additional surgical interventions for their correction.The aim of the study is to demonstrate the successful treatment experience of a dermoid cyst of the mesentery of the jejunum, detected after traditional appendectomy.Clinical case:  A mother brought her 9-year-old son, V., to the emergency department of the Kursk Regional Children’s Clinical Hospital with complaints of moderate-intensity pain in the right iliac region, multiple episodes of vomiting, and fever. Ultrasound features indicative of destructive appendicitis and peritonitis. In an emergency situation, after preoperative preparation, a traditional appendectomy was performed under intravenous anesthesia, using the Volkovich-Dyakonov approach. The postoperative period was uneventful. Moderate infiltration was observed on the control abdominal ultrasound examination, in the intestine (presumably the small intestine) in the right half of the abdominal cavity, with a solid formation measuring 27*33 mm, producing a dense acoustic shadow. On the 7th day after the initial intervention, a revision of the abdominal organs was performed. The loop of the jejunum with the formation was brought out into the wound. The capsule above the formation was dissected: the formation of bone density, when the capsule is separated, resembles a fecal stone in structure, considering the localization - a dermoid cyst. Step-by-step enucleation of the cyst was performed. The postoperative wound was tightly sutured in layers.

The Masquelet technique has become increasingly popular in reconstruction scenarios such as osteomyelitis, cancer, and nonunions. The procedure is a two-staged approach: first, a polymethylmethacrylate (PMMA) cement spacer is inserted to fill a bone void. The spacer induces a membrane to form around it, and 6-9 weeks later, the second stage involves carefully extracting the spacer and filling the membrane with bone graft. Most of the current literature has published either femoral or tibial involvement for Masquelet studies, with limited published data on foot reconstruction. Here, the authors used the procedure for the fourth metatarsal. Despite complications, this case proves a more excellent utility and warrants further investigation into Masquelet techniques in the foot.