Darier Roussy Subcutaneous Sarcoidosis from Nepal: A case report.

Introduction: Sarcoidosis is a common, multisystemic non-caseating granulomatous disease of unknown etiology with cutaneous lesions present in about one-fourth of patients. Darier Roussy sarcoidosis is a rare variant of sarcoidosis with distinct cutaneous presentation characterized by multiple deep-seated nodules on the trunk and extremities which could either be asymptomatic or may present mild tenderness. Case presentation: A case of 35 yrs male with cough and fever for 3 months was initially diagnosed as a case of tubercular lymphadenitis and started with ATT following which ATT-associated cutaneous adverse drug reaction was suspected due to development of rashes with generalized redness and mild itching a few weeks after starting ATT. He then developed multiple, skin-colored, deep-seated, subcutaneous lesions over the legs then over the arms, forearms, thigh, and trunk. FNAC and histopathological examination of the lesions revealed non-caseating granulomas composed localized to the subcutaneous tissue. A diagnosis of subcutaneous sarcoidosis was made. Subsequently, steroid therapy was started. Discussion: Clinical manifestations of sarcoidosis range from asymptomatic (mostly) to progressive and relapsing disease. A family history of the disease raises the risk; those with one afflicted first-degree relative face a 3.7-fold increase in risk. Sarcoidosis is diagnosed based on three key criteria: a consistent clinical presentation, the discovery of non-necrotizing granulomatous inflammation in one or more tissue samples with confirmed histology, and the elimination of other origins of granulomatous disorders. . Diagnosis should be confirmed with a biopsy of the lesion, with the histological finding of non-caseating granuloma. Conclusion: Clinically localized subcutaneous sarcoidosis can be confused with ATT induced drug reaction due to the difficulties in diagnosing granulomatous skin disease. The prognosis is good with subcutaneous disease and if there are no disfiguring skin lesions or other critical organ involvement, corticosteroid therapy might suffice.

Diffuse midline glioma H3K27M mutation in adult: A case report.

Introduction: Diffuse midline glioma with H3 K27M mutation is a new tumor entity from 2016 which is highly aggressive and classified as a WHO Grade IV tumor regardless of histopathologic features. DMG is an aggressive tumor with a poor prognosis, predominating in children and rarely in adults. The clinicopathologic features in adults remain poorly characterized. Case presentation: Herein we report a case of a 28-year-old female with diffuse midline glioma with pathology confirmation of histone H3 K27M mutation presenting predominately with left upper and lower limb weakness for 3 weeks followed by an event of loss of consciousness and suspicious mass in MRI Brain/CT Brain. It was confirmed by immunoreactive H3K27M with a score of 4+ in neoplastic cells, which revealed Diffuse midline glioma, H3K27M mutant. Clinical discussion: Diffuse midline glioma with histone H3-K27M mutation recently classified CNS tumor with grade IV, including both morphologic and molecular features for diagnosis and associated with poor prognosis. Conclusion: We report a case of adult diffuse midline glioma with H3K27M. The prognosis of diffuse midline glioma is poor and dependents solely on H3K27M irrespective of its grade and characteristics. A comprehensive study of diffuse midline glioma on clinical, radiographic, and demographic features in adult is needed.

Successful medical management of diabetic ketoacidosis at first presentation in a child with type 1 diabetes: A case report.

Introduction and importance: Diabetic ketoacidosis (DKA) is considered to be a common presentation of type 1 diabetes mellitus in children. It occurs when absolute or relative insulin insufficiency prevents glucose from entering the cells for use as metabolic fuel, causing the liver to quickly break down fat into ketones for use as fuel source. As a result, ketones are overproduced, accumulating in the blood and urine making the blood acidic. Case presentation: A 4 years and 8 months old child presented with the complaint of abdominal pain and vomiting along with polyurea, polydipsia and polyphagia. Routine examination of blood revealed that increased random blood glucose level. Once diagnosed, DKA was managed with fluid and insulin therapy with close monitoring and supervision. Clinical discussion: DKA can be easily diagnosed. Proper management should be done on time to prevent complications like hypokalemia, hyponatremia leading to cerebral edema and shock. Conclusions: Diabetic awareness programs and school educational tutorials are beneficial for community awareness of the signs and symptoms of diabetes.

Diagnostic dilemma in diagnosing rare cause of protein losing enteropathy: Waldmann's disease.

Waldmann's disease, or primary intestinal lymphangiectasia, is an unusual cause of protein-losing enteropathy primarily characterized by lymphopenia, hypoalbuminemia, and hypogammaglobulinemia. However, variable clinical presentations result dilemmas in diagnosis and effective management. We present a toddler diagnosed with Waldmann's disease managed with a high-protein diet and medium-chain triglyceride supplementation.

Guillain-Barré Syndrome associated with SARS-CoV-2 infection in Nepal: A case report.

Introduction: Corona viruses may also affect the central nervous system, inducing various neurological problems. Guillain-Barré Syndrome (GBS) is a rare immune-mediated post-infectious neuropathy typically leading to ascending weakness. Herein, we present a case of the patient to show an association of GBS and SARS-CoV-2 infection in Nepal. Case presentation: Twenty-seven yrs old man show an association of GBS and SARS-CoV-2 infection in Nepal who presented with difficulty in walking, backache, tingling sensations over the bilateral wrist and ankle, and features of facial nerve palsy. The diagnosis of GBS was made. Following Intravenous Immunoglobulin (IVIg) administration, the patient started showing motor recovery within a week. Clinical discussion: Patient who developed GBS as a likely post-infectious complication after the initial onset of infectious symptoms with persistent mild dry cough. Conclusion: GBS has severe complications and early diagnosis is important to monitor for loss of ambulation and initiation of immunoglobulin treatment. GBS should be considered as a potential rare but serious complication due to COVID-19.

Hemophagocytic Lymphohistiocytosis secondary to Rickettsial infection: A case report.

Hemophagocytic Lymphohistiocytosis (HLH) is a rare life-threatening condition characterized by widespread activation of the immune system leading to tissue damage all over the body. It is divided into primary HLH due to inborn error in lymphocytes, T cells, and macrophages and secondary HLH which is mostly due to infections, systemic connective tissue diseases, and lymphoid malignancies. Here, we report a 34-year-old man with a history of high-grade fever, chills, and rigor, eschar, splenomegaly with the laboratory findings of thrombocytopenia, hypochromic RBCs with anisocytosis and basophilic stippling, elevated transaminases, and a positive Weil Felix test along with positive PCR results for Orientia tsutsugamushi and the presence of IgG and IgM antibodies. A detailed workup was done to rule out other etiology for fever. Diagnosis of HLH secondary to Rickettsia infection was made with a thorough history, clinical evaluation, and a variety of investigations. The patient was treated with Doxycycline, Ciprofloxacin, Etoposide, and Dexamethasone but unfortunately, the patient died during treatment due to multiorgan failure. Patients with scrub typhus typically respond well to therapy; therefore, early detection and antibiotic treatment can help avoid serious complications. Scrub typhus with the hemophagocytic syndrome can result in DIC and multiorgan failure. Despite its rarity, scrub typhus may be lethal; as a result, practitioners must be aware of the necessity of detecting and treating suspected cases as soon as possible. We learned that a systematic diagnostic approach, use of diagnostic criteria, and prompt treatment are very crucial in this disease.

Spontaneous sublingual hematoma secondary to warfarin therapy-A rare complication.

Warfarin is the most commonly prescribed oral anticoagulant in Nepal. It is commonly used for chronic anticoagulation in patients with atrial fibrillation, venous thromboembolism, and artificial heart valves. The major side effect of warfarin is bleeding. Though extremely rare, a sublingual hematoma can lead to life-threatening complications as it can cause severe airway obstruction. We present a case of a 55-year-old female patient who had sublingual hematoma secondary to the use of Warfarin therapy. In addition to the discontinuation of warfarin, she was managed conservatively without any surgical intervention. Early diagnosis, timely discontinuation of the drug, and application of appropriate medical treatment are of utmost importance for reducing morbidity and mortality due to bleeding and airway compromise.

An Uncommon Case of Splenic Abscess with Gangrenous Gas Necrosis.

Splenic abscess is a rare clinical entity with diagnostic challenges. Though rare, it is potentially a life-threatening clinical condition with high mortality reaching more than 70%. The common signs and symptoms include the triad of fever, left upper quadrant tenderness, and leukocytosis. Early diagnosis, however, can readily be made by the combination of clinical features, abdominal ultrasonography (USG), and computed tomography (CT). The management of splenic abscess includes medical therapy, CT-guided percutaneous aspiration, and splenectomy. We, hereby, present a rare case of splenic abscess with gangrenous gas necrosis, who underwent splenectomy.

Cholecystopathia chronica calcarea (Porcelain gall bladder): A case report from Nepal.

Introduction and importance: Porcelain gall bladder is an uncommon end-stage modification of chronic cholecystitis, with an incidence ranging from 0.06 to 0.8% along with a plausibility of malignant transformation. Case presentation: We present a 55-year-old female presenting with complaints of epigastric and right hypochondriac region pain who underwent prophylactic laparoscopic cholecystectomy after making a provisional diagnosis of calcified gall bladder on a computed tomography workup. On histopathological examination, she was later diagnosed with a porcelain gallbladder devoid of features suggestive of malignant transformation. Clinical discussion: Porcelain gallbladder is a cholecystopathological condition in which the gallbladder wall gets calcified, either completely or partially. Though the exact pathomechanism of gallbladder calcification is unknown, it is believed to be due to chronic inflammation. Recent studies have shown that gallbladder calcification is associated with a lower risk of the development of gallbladder cancer. Imaging studies, followed by post-operative histopathological examinations, are used to diagnose the porcelain gallbladder. Though the management of asymptomatic patients is debatable, prophylactic cholecystectomy is the preferred treatment for symptomatic porcelain gallbladder patients. Conclusion: Individual porcelain GB patients should be addressed based on the presenting condition, whether surgically or via clinical monitoring and follow-up, taking into consideration the advantages and limitations of both treatment modalities.

Midodrine in Liver Cirrhosis With Ascites: A Systematic Review and Meta-Analysis.

Ascites is the most common complication of liver cirrhosis. Midodrine is a vasoconstrictor that improves splanchnic and systemic hemodynamics, reduces ascites, and improves clinical outcomes. Here, we aimed to examine the role of midodrine in cirrhosis-related ascites. Scopus, Embase, PubMed, and PubMed Central databases were searched for relevant randomized controlled trials comparing midodrine with other interventions in patients with cirrhotic ascites on November 25, 2020, using appropriate keywords like "midodrine", "ascitic cirrhosis", "peritoneal paracentesis" and suitable Boolean operators. Odds ratio (OR) and mean difference (MD) were used to analyze pool data as appropriate with a 95% confident interval (CI). A total of 14 studies were included in our analysis including 1199 patients. The addition of midodrine resulted in statistically significant improvement in mean arterial pressure (MAP) (MD, 3.95 mmHg; 95% CI, 1.53-6.36) and MELD (Model for End-Stage Liver Disease) score (MD, -1.27; 95% CI, -2.49 to -0.04) compared to standard medical treatment (SMT). There was also a significant improvement in plasma renin activity and plasma aldosterone concentration. However, there was no significant improvement in mortality or serum creatinine compared to SMT. In addition, there was no statistically significant improvement in MAP, plasma renin activity, plasma aldosterone concentration, MELD score, overall mortality, and paracentesis-induced circulatory dysfunction comparing midodrine with albumin. Midodrine alone leads to significant improvement in various clinical parameters in patients with cirrhotic ascites compared to standard medical care. At the same time, it was found to be non-inferior to albumin. Therefore, further well-designed studies need to be carried out on midodrine in addition to albumin for optimal clinical benefits among patients with ascites due to cirrhosis.

Steroid in Chronic Subdural Hematoma: An Updated Systematic Review and Meta-Analysis Post DEX-CSDH Trial.

BACKGROUND: Chronic subdural hematoma (CSDH) is a neurologic condition characterized as a hematoma in the subdural space with a period >3 weeks that primarily affects the elderly. Glucocorticoid, especially dexamethasone, either alone or combined with surgery, has been used to manage CSDH. We aimed to perform an updated systematic review and meta-analysis of the literature regarding the role of steroids in CSDH. METHODS: We searched the electronic databases PubMed, PubMed Central, Scopus, and Embase for relevant articles until December 2020. Study characteristics, quality, and end points were extracted, and analysis was performed by RevMan 5.4. RESULTS: The odds for subdural hematoma recurrence were decreased by 61% in the steroid group (odds ratio [OR], 0.39; confidence interval [CI], 0.19-0.79) compared with the control group. There was no significant difference in mortality during the study period (OR, 0.66; CI, 0.20-2.18), modified Rankin Scale score 0-3 (OR, 0.87; CI, 0.31-2.40), and modified Rankin Scale score 4-6 (OR, 1.15; CI, 0.42-3.18) between the 2 groups. However, pooling data from 3 studies showed 2.7 times higher odds of occurring adverse effects in steroid groups using the fixed-effect model (OR, 2.70; CI, 1.71-4.28). The treatment success was similar between the steroid and control groups (OR, 2.39; CI, 0.94-6.04). CONCLUSIONS: Treatment with steroids was associated with a lesser recurrence of CSDH. However, there was no benefit of steroid treatment in CSDH compared with nonsteroid treatment in terms of mortality and treatment success and some but significantly increased risk of adverse events.

Vesico-Vaginal Fistula in Females in 2010-2020: a Systemic Review and Meta-analysis.

INTRODUCTION: In the Western world today, urogenital fistula, including vesicovaginal fistula (VVF), is rare. However, while it remains significant in developing parts of the world due to prolonged and obstructed labor, in this study, we systematically reviewed the existing literature, discussing VVF occurrence, its etiology, and outcomes. MATERIAL AND METHODS: We used electronic databases to search relevant articles from 2010-2020. The screening was performed with the help of Covidence. Relevant data from included studies were extracted in excel sheets, and final analysis was done using CMA-3 using proportion with 95% confidence interval (CI). RESULTS: Fifteen studies reported the VVF among the fistula series. The pooled result showed 76.57% cases of VVF (CI, 65.42-84.96), out of which 27.54% were trigonal, 55.70% supra-trigonal, and the rest with a varied description like circumferential, juxta-cervical, juxta-urethral. Obstetric etiology was commonly reported with 19.29% (CI, 13.26-27.21) with cesarean section and 31.14% (CI, 18.23-47.86) with obstructed labor. Hysterectomy was the commonly reported etiology among gynecological etiology (46.52%, CI; 36.17-57.19). Among different surgical treatments employed for fistula closure, 49.50% were by abdominal approach (CI, 37.23-61.82), and 42.31% by vaginal approach (CI, 31.82-53.54). Successful closure of fistula was reported in 87.09% of the surgeries (CI, 84.39-89.38). CONCLUSION: The vesicovaginal fistula is the most common type of genitourinary fistula. Major causes of fistula are gynecological surgery, obstructed labor, and cesarean section. The vaginal approach and abdominal are common modalities of repair of fistula with favorable outcomes in the majority of the patients.

Incidental diagnosis of paragonimiasis after histopathological examination of cholecystectomy specimen in Nepal: A case report.

INTRODUCTION: Paragonimiasis or lung fluke disease is a typical food-borne parasitic zoonosis caused by infection with Paragonimus species. The Paragonimus is a trematode that mainly infects the lungs of humans after eating an infected raw or undercooked crab or crayfish. CASE PRESENTATION: Herein we report a case of peritoneal Paragonimiasis in the gallbladder of a 58-year-old female from Rukum district of Nepal. It was an incidental diagnosis following routine histopathological examination of the cholecystectomy specimen. She presented with the symptoms of abdominal pain, fever, cough, and had a history of consumption of partially cooked river crabs. She responded well to praziquantel and improved thereafter. CLINICAL DISCUSSION: Ectopic paragonimiasis is a rare disease and it presents with few clinical symptoms so it is significantly difficult to make a diagnosis and treat the patients. Even if a sputum test and biopsy are performed, the ova or body of Paragonimus parasites may not be detectable due to insufficient amount of specimens. Therefore, thorough history taking should be given importance. The dietary history of partially cooked crab or crayfish should indicate towards a high suspicion of Paragonimiasis. Any such indication should be immediately confirmed, which in our case was done by routine histopathological examination of the cholecystectomy specimen. CONCLUSION: We report the case of a patient with peritoneal paragonimiasis in the gallbladder. Ectopic paragonimiasis is hard to diagnose due to an ignorance of, misdiagnosis, and the rarity of this disease. Thus, thorough history-taking and clinical suspicion of parasitic infection is essential.

Artificial Intelligence in Medical Science: Perspective from a Medical Student.

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