Retrosternal Thyroid with Mediastinal Extension: Surgical Approach and Management.

To assess the clinical presentation and the need for sternotomy as an additional approach for surgical excision of goitre with retrosternal extension. It was a retrospective review of 22 patients (13 female, 9 male; mean age: 50.36 years) who underwent total thyroidectomy for retrosternal extension. Between-group differences were assessed using Chi-square or Fisher exact test, appropriate P values < 0.05 were considered indicative of statistical significance. All cases were assessed by CT scan of neck and thorax to confirm mediastinal extension. The symptoms most commonly reported were dyspnoea 6 (27%), hoarseness (13%) and dysphagia 2 (9%). The retrosternal extent was grade 1(thoracic inlet to aortic arch) in 11 cases and grade 2(aortic arch to pericardium) in 11 cases. Overall, 13 (59%) patients showed CT evidence of tracheal deviation. Patients were divided into two groups based on the surgical approach: trans-cervical (n = 13) and trans-cervical plus trans-sternal (n = 9). The radiological extent of retrosternal descent of goitre in the mediastinum showed no significant correlation with the requirement of sternotomy in our cohort (odds ratio for sternotomy in grade 2 extension: 1.45). The need for sternotomy in cases of retrosternal thyroid does not depend on the radiological extent of tumour alone. It depends on multitude of factors like presence of dysphagia, dyspnoea or hoarseness. Preservation of recurrent laryngeal nerve and parathyroid glands is difficult in the restricted thoracic inlet space. Sternotomy is required for complete excision and preservation of major mediastinal vessels.

Skull Base Involvement in Covid Associated Rhino-Orbital-Cerebral Mucormycosis: A Comprehensive Analysis.

The base of skull forms the first line of barrier to be breached in the transition of rhino-orbito-palatal forms of mucormycosis to intracranial forms with various neurological deficit. The pattern of base of skull erosion has prognostic implications in overall recovery and survival of the patient. The aim of the study was to assess the pattern of skull base involvement in cases of rhino-orbital-cerebral mucormycosis (ROCM) in terms of clinical presentations, radiological findings, intraoperative illustrations and post operative recovery. This is a retrospective single centre study of Covid associated Mucormycosis (CAM) patients with skull base involvement at a tertiary referral centre of central India from May 2021 to October 2021. Amongst a total of 248 patients of CAM, 54 patients with skull base involvement were included in our study. The cases were stratified into basifrontal-BF (15%), basisphenoid-BS (20%), orbital apex-OA (15%), basiocciput-BO (26%), frontal bone osteomyelitis-FBO (22%) and sphenoid bone osteomyelitis-SBO (2%), based on their pattern of involvement of skull base and intracranial spread. Early ethmoid and cribriform plate involvement progressed to frontal lobe abscess while early maxillary disease progressed to developed temporal lobe abscess. The orbital apex lesions had early onset cavernous sinus thrombosis. Analysis of clinical manifestations and postoperative follow up revealed an emerging pattern where Posterosuperior lesions of paranasal sinuses (Ethmoid, roof of maxilla and orbit) progressing to BF, BS, OA, FBO and SBO had poorer treatment outcome than Anteroinferior (Floor of maxillary sinus, palate) based lesions which involved BO of skull base. The inferiorly located diseases had better prognosis, less duration of hospital stay, lesser mortality and decreased need for second surgery. There exists a temporal relation of the initial site of fungal load in sinonasal region to their subsequent intracranial spread. Classification into subtypes helped in disease stratification which helped in prognostication and surgical planning. Early intervention by multidisciplinary team improved survival outcome.

Diagnostic approach in 46, XY DSD: an endocrine society of bengal (ESB) consensus statement.

OBJECTIVES: 46, XY difference/disorder of sex development (DSD) is a relatively uncommon group of heterogeneous disorders with varying degree of underandrogenization of male genitalia. Such patients should be approached systematically to reach an aetiological diagnosis. However, we lack, at present, a clinical practice guideline on diagnostic approach in 46, XY DSD from this part of the globe. Moreover, debate persists regarding the timing and cut-offs of different hormonal tests, performed in these cases. The consensus committee consisting of 34 highly experienced endocrinologists with interest and experience in managing DSD discussed and drafted a consensus statement on the diagnostic approach to 46, XY DSD focussing on relevant history, clinical examination, biochemical evaluation, imaging and genetic analysis. CONTENT: The consensus was guided by systematic reviews of existing literature followed by discussion. An initial draft was prepared and distributed among the members. The members provided their scientific inputs, and all the relevant suggestions were incorporated. The final draft was approved by the committee members. SUMMARY: The diagnostic approach in 46, XY DSD should be multidisciplinary although coordinated by an experienced endocrinologist. We recommend formal Karyotyping, even if Y chromosome material has been detected by other methods. Meticulous history taking and thorough head-to-toe examination should initially be performed with focus on external genitalia, including location of gonads. Decision regarding hormonal and other biochemical investigations should be made according to the age and interpreted according to age-appropriate norms Although LC-MS/MS is the preferred mode of steroid hormone measurements, immunoassays, which are widely available and less expensive, are acceptable alternatives. All patients with 46, XY DSD should undergo abdominopelvic ultrasonography by a trained radiologist. MRI of the abdomen and/or laparoscopy may be used to demonstrate the Mullerian structure and/or to localize the gonads. Genetic studies, which include copy number variation (CNV) or molecular testing of a candidate gene or next generation sequencing then should be ordered in a stepwise manner depending on the clinical, biochemical, hormonal, and radiological findings. OUTLOOK: The members of the committee believe that patients with 46, XY DSD need to be approached systematically. The proposed diagnostic algorithm, provided in the consensus statement, is cost effective and when supplemented with appropriate genetic studies, may help to reach an aetiological diagnosis in majority of such cases.

A study of apolipoprotein A1(ApoA1) and interleukin-10(IL-10) in diabetes with foot ulcers.

Background: The high morbidity, mortality and associated economic burden have entailed to identifying early biomarker of diabetic foot ulcers (DFU). Pro-inflammatory and anti-inflammatory molecules play a role in the chronic inflammation associated with diabetic foot ulcers (DFU). Aim: This study aims to find the association between ApoA1, IL-10, TNF-α and diabetic foot ulcers, and whether their levels can assess the severity of the disease. Method: Two groups, diabetic mellitus without foot ulcers and diabetes with foot ulcers were recruited for the study. Detailed clinical history was obtained and blood was collected to measure TNF-α , IL-10 and Apo A1. The association between variables was analysed using Pearson correlation test. ROC analysis was used to identify cut-off values of ApoA1, IL-10 and TNF-α in diabetes patients with foot ulcers. Results: The presence of pro-inflammatory parameter, TNF-α , was higher and anti-inflammatory biomarkers, HDL, ApoA1 and IL-10 were lower in patients of DFU than those without foot ulcers (p < 0.001). Increasing age, smoking, retinopathy, eGFR and inflammatory biomarkers like low levels of ApoA1 (p < 0.005) and IL-10 (p < 0.001) significantly contributed to the development of diabetic foot ulcers. ROC curve identified the cut-off for ApoA1 and IL-10 as 89.82mg/dL and 78.80pg/mL respectively. Conclusion: In the light of this study, ApoA1 has the potential to predict DFU. The finding proposes IL-10 (b = -0.37, p < 0.001) could be considered in stratifying DFU as per its severity.

Intensive Glycemic Control for Diabetic Foot Ulcer Healing: A Multicentric, Randomized, Parallel Arm, Single-Blind, Controlled Study Protocol (INGLOBE Study).

Hyperglycemia impairs healing of diabetic foot ulcer (DFU). But there is no evidence regarding benefit of intensive glucose control for healing of DFU. We plan to conduct a randomized, parallel arm, controlled study to assess the role of intensive glycemic management in comparison to conventional glucose control for healing of DFU. Participants with neuropathic DFU (infected or uninfected) having hemoglobin A1c (HbA1c) >8% and without evidence of osteomyelitis from 7 tertiary care hospitals will be enrolled. They will undergo a 2-week run-in phase for optimization of comorbidities, ulcer debridement, and counseling regarding self-monitoring of blood glucose (SMBG). Subsequently, they will be randomized to "intensive glycemic control" arm defined by glycemic targets of fasting blood glucose (FBG) <130 mg/dL, postprandial BG <180 mg/dL, and HbA1c <8%, with basal-bolus insulin regimen and frequent titration of insulin to achieve glycemic targets. The "conventional" arm will continue on prior treatment (oral antidiabetic drugs) with no titration unless meeting rescue criteria. Ulcer area will be calculated by automated wound assessment device (WoundlyClinial app) weekly for first 4 weeks, and less frequently until the 24th week. Standard treatment for DFU, off-loading, and counseling for foot care will be provided in both arms. The primary outcome measure will be number of wounds closed at 12th and 24th weeks. A multivariate regression analysis will be performed to identify the predictors of wound healing with baseline HbA1c, diabetes duration, wound size, wound duration, and background therapies as independent variable. This study will provide the much needed guidance to set optimum glucose targets in people with DFU.

Angiolymphoid hyperplasia with eosinophilia presenting with peri-ocular swelling.

Angiolymphoid hyperplasia with eosinophilia and Kimura's disease share many clinical and histological features. Although they were once considered different stages of the same disease, they are now known to represent separate entities. Here a case is reported in a 14-year-old girl who presented with bilaterally symmetrical gradually progressive peri-ocular swelling involving both upper and lower eyelids of two months' duration. Peripheral blood showed eosinophilia, MRI scan of orbit revealed soft tissue mass in both lids encroaching into the globe, histopathology of biopsy specimen revealed angiolymphoid hyperplasia with eosinophilia. The case is reported for the peculiarity of presentation and rare location of the disease.

Gitelman's syndrome presenting with hypocalcaemia, basal ganglia calcification and periodic paralysis.

Gitelman's syndrome (GS), also referred to as familial hypokalaemia-hypomagnesaemia syndrome, is an autosomal recessive renal tubular disorder characterised by hypokalaemic metabolic alkalosis, hypomagnesaemia and hypocalciuria. It is caused by a defect of the thiazide-sensitive sodium chloride co-transporter at the distal tubule. This condition was previously confused with Bartter syndrome. Documentation of hypocalciuria helps to differentiate GS from Bartter syndrome. We report a 44-year-old woman who presented with a history of seizure disorder and periodic paralysis. On investigation, she was found to have hypokalaemic metabolic alkalosis, hypomagnesaemia, hypocalciuria, hypoparathyroidism, hypocalcaemia and basal ganglia calcification, consistent with GS. The atypical features in our case, namely basal ganglia calcification and hypocalcaemia, prompted the writing of this case report.

A patient with recurrent attacks of drowsiness and lethargy--a diagnosis not to be missed.

Polyglandular auto-immune syndromes are uncommon constellation of organ specific auto-immune diseases, characterised by the existence of two or more endocrinopathies. Polyglandular autoimmune type II syndrome also known as Schmidt's syndrome is more common, comprising Addison's disease, auto-immune thyroid disease, type 1 diabetes mellitus and/or hypogonadism, pernicious anaemia, coeliac disease, vitiligo, hypophysitis, etc. Here a case of a 56-year-old man is reported with a prior history of hypothyroidism who presented with adrenal crisis. Further laboratory investigations revealed primary auto-immune hypothyroidism, primary adrenal insufficiency, hypogonadism and he was diagnosed as a case of polyglandular auto-immune type II syndrome or Schmidt's syndrome. Early recognition of the syndrome and replacement therapy can be life saving, particularly when there is adrenal or thyroid insufficiency.

Primary cold agglutinin disease: a case report.

Chronic cold agglutinin disease is a subgroup of auto-immune haemolytic anaemia. Primary cold agglutinin disease has traditionally been defined by the absence of any underlying or associated disease. It usually affects elderly. The term cold refers to the fact that the auto-antibody involved reacts with red cells poorly or not at all at 37 degrees C, whereas it reacts strongly at lower temperature. Here a case of severe pallor, jaundice and red colour urine in winter season for last 10 years diagnosed as a case of primary cold agglutinin disease is reported.The patient was managed conservatively.

A patient with bilateral midbrain syndrome due to Plasmodium falciparum infection - a rare presentation

Cerebral malaria is a serious complication of Plasmodium falciparum infection with a high mortality rate, usually present with diffuse cerebral involvement and rarely with focal neurologic defi cit. Here we report a case of a young adult without any risk factors for stroke who presented with quadriparesis and was diagnosed to have bilateral midbrain stroke-like syndrome due to Plasmodium falciparum malaria. This is an unusual presentation of Plasmodium falciparum malaria. In tropical countries like India, all patients who present with fever and neurologic defi cit should be tested for malaria.