Single breath count test and its applications in clinical practice: a systematic review.

Background: Single breath count test (SBCT) may be a reproducible, rapid, easy to perform and easy to interpret substitute to spirometry especially in low resource settings for certain conditions. Its interest has been rekindled with the recent COVID-19 pandemic and it can be done as a part of tele-medicine as well. Objectives: The objective of this review was to summarize the evidence of SBCT in clinical practice. Methods: The authors searched EMBASE, PubMed and Google Scholar for all the relevant articles as per exclusion and inclusion criteria. Two authors independently screened all the studies. Newcastle Ottawa Scale was used to assess the quality of the studies. The systematic review was carried following the PRISMA guidelines. Results: After the rigorous process of screening, a total of 13 articles qualified for the systematic review. SBCT greater than 25 had sensitivity of greater than 80% in diagnosing myasthenia gravis exacerbation and SBCT less than or equal to 5 predicted the need for mechanical ventilation in Guillain-Barre syndrome (GBS) patients with 95.2% specificity. Also, Single breath count correlated significantly with forced expiratory volume in 1 sec (FEV1) and forced vital capacity (FVC) in children with pulmonary pathology and in patients with COVID-19 it was used to rule out the need for noninvasive respiratory support. Conclusion: SBCT will undoubtedly be an asset in low resource settings and in tele-medicine to assess the prognosis and guide management of different respiratory and neuromuscular diseases.

Assessing the comparative efficacy of plasmapheresis and Intravenous immunoglobulin in myasthenia gravis treatment: A systematic review and meta-analysis.

BACKGROUND: Myasthenia gravis (MG) is an autoimmune neuromuscular disorder characterized by muscle weakness, posing significant challenges to patients' daily lives. Intravenous immunoglobulin (IVIG) and plasmapheresis are two prominent immunomodulatory therapies used in MG management, but the choice between them remains a clinical dilemma. This systematic review and meta-analysis aim to evaluate the comparative efficacy of IVIG versus plasmapheresis in MG management. METHODS: We adhered to PRISMA guidelines and prospectively registered the review protocol in PROSPERO. Systematic search across electronic databases identified 14 studies meeting inclusion criteria. Data from these studies were extracted, and assessed risk of bias. Primary outcomes included clinical efficacy, while secondary outcomes encompassed hospitalization, ventilation, antibody titers, and treatment-related complications. Statistical analysis was conducted using R software. RESULTS: The pooled results indicated that patients receiving plasmapheresis had higher odds of any improvement in MG symptoms compared to IVIG. However, change in severity scores did not significantly differ between the two treatments. Hospitalization durations were similar, but IVIG-treated patients tended to have shorter stays. Antibody titers, particularly anti-MUSK antibodies, favored plasmapheresis treatment. Complication rates were comparable between two groups. However, severe complications were more common in plasmapheresis. CONCLUSION: This comprehensive analysis suggests that plasmapheresis may offer superior short-term symptom improvement in MG compared to IVIG, while IVIG may lead to shorter hospital stays and lower complication rates. The choice between these treatments should be tailored to individual patient needs and disease characteristics. Further research is needed to explore long-term outcomes and mortality rates in MG management.

Adrenal Leiomyoma: A case report.

INTRODUCTION AND IMPORTANCE: Adrenal Leiomyomas are infrequent tumors with only a few cases reported to date. They are difficult to differentiate from malignant adrenal tumors due to non-specific findings on clinical examination and imaging studies. CASE PRESENTATION: We discuss the case of a 49-year old male who had been experiencing generalized abdominal pain for 14 months and was found to have a mass on ultrasonography. Further evaluation with Contrast-enhanced Computerized Tomography (CECT) revealed an uneven soft tissue density mass in the retroperitoneal region of the left side. The histopathological examination of the excised mass was suggestive of a mesenchymal tumor, which was further confirmed as leiomyoma by immunohistochemistry. CLINICAL DISCUSSION: Adrenal Leiomyomas are rare smooth muscle tumors that present with heterogeneously enhancing mass on radiologic imaging. On histopathological examination, spindle cells arranged in lobules and fascicles can be appreciated. Positive staining for desmin and smooth muscle actin in immunohistochemistry confirms the diagnosis. CONCLUSION: Identification of the type of tumor in any adrenal mass is challenging pertaining to the non-specific findings on imaging studies. So, prompt surgical resection is the mainstay of the treatment.

Hirschsprung disease with Edward syndrome: A rare association: A case report.

INTRODUCTION AND IMPORTANCE: Edward's syndrome (ES) occurs as a result of trisomy of chromosome 18 and is associated with multisystem congenital anomalies. The association of ES with various gastrointestinal malformations but Hirschsprung disease (HD) is well documented. CASE PRESENTATION: A female infant on her 5th day of life presented with episodes of bilious vomiting along with abdominal distension and no passage of stool. The child had a small head and prominent occiput, low set abnormal ears, small jaw, upturned nose, widely spaced eyes, small neck with widely spaced nipples, clenched hands with overlapping fingers, flexed big toe, and prominent heels. CLINICAL DISCUSSION: Edward syndrome is associated with multisystem congenital abnormalities of which gastrointestinal abnormalities make up the most part. The condition can be identified by fetal ultrasound screening. Surgical correction of associated congenital anomalies at different times along with lifelong supportive management is important. CONCLUSIONS: Edward syndrome can present as Hirschsprung disease as a part of associated gastrointestinal Malformation. Often, early identification and termination of the pregnancy in antenatal life can reduce the suffering. Surgical correction of associated anomalies along with supportive care forms the cornerstone of management. However, the prognosis remains poor.