Bruns Garland Syndrome as the first presentation of type 2 diabetes: two case reports and a practical approach to diagnosis.

BACKGROUND: Diabetes is a global health problem causing a significant burden on the healthcare systems both due to the disease itself and associated complications. Diabetic radiculoplexus neuropathies or Bruns-Garland syndrome constitutes a rare form of microvascular complications, more commonly affecting the lumbosacral plexus and, very rarely, the cervical plexus. We describe two Sri Lankan males who presented with diabetic lumbosacral radiculoplexus neuropathy and diabetic cervical radiculoplexus neuropathy as the initial manifestation of diabetes. CASE DESCRIPTION: Case 1: a 49-year-old Sri Lankan hotel chef presented with subacute painful weakness and wasting of the left upper arm for 3 months and weight loss. Left upper limb proximal muscles were wasted with diminished power and reflexes. A nerve conduction study showed comparative amplitude reduction. An electromyogram revealed positive sharp waves, frequent fibrillations, and high amplitude polyphasic motor unit potentials with reduced recruitment in proximal muscles of left upper limb. Case-2: a 47-year-old Sri Lankan carpenter presented with subacute progressive asymmetrical painful weakness and wasting of bilateral thighs for 5 months and weight loss. Lower limb proximal muscles were wasted with reduced power and knee jerks. The nerve conduction study was normal. The electromyogram was similar to case 1 involving both quadratus femoris muscles, which was more prominent on the left side. The work up for an underlying etiology revealed only elevated fasting blood glucose and HbA1c, suggesting a new diagnosis of diabetes associated with neurological symptoms. Patient 1 was diagnosed with diabetic cervical radiculoplexus neuropathy and patient 2 with diabetic lumbosacral radiculoplexus neuropathy. Both showed significant improvement following optimization of glycemic control together with symptomatic treatment and physiotherapy. CONCLUSION: Diagnosis of diabetic radiculoplexus neuropathy requires a comprehensive workup to rule out other sinister pathologies. This case report has a dual importance; it describes diabetic radiculoplexus neuropathy as the very first manifestation of two previously healthy people, giving rise to a new diagnosis of diabetes and, at the same time, reporting on diabetic cervical radiculoplexus neuropathy, which is extremely rare and has never been previously reported in Sri Lanka.

Standards of diabetes care and burden of hypoglycaemia in people with diabetes on peritoneal dialysis: Results from a real-world clinical audit.

There is limited data on the standards of diabetes care in people on peritoneal dialysis (PD). Our aim was to assess the standards of diabetes care and the burden of hypoglycaemia in people with diabetes on PD. We performed a retrospective study at three university hospitals from December 2021 to January 2022. Clinical data were extracted from electronic health records. Diabetes care of people on PD was compared against recommended standards for people with diabetes on haemodialysis (as there are no agreed standards for PD). The degree of hypoglycaemia awareness was assessed by validated questionnaires. A total of 65 adults (15 type 1, 49 type 2 and 1 monogenic-diabetes) with a mean age of 63 (range 29-88) years were evaluated. Of them, 92% had diabetes retinal screening with annual review. In contrast, in this high-risk group for foot disease, only 77% had annual foot reviews. The rates of diabetes specialist reviews were variable between hospitals at 63-94% and 10 (15%) had impaired hypoglycaemia awareness. Of the cohort, 32% had HbA1c within the acceptable range of 58-80 mmol/mol (7.5-8.5%), 21% had HbA1c below 58 mmol/mol (7.5%) and 21% (n = 14) reported at least one hypoglycaemic event per month. Our results indicate variation of care within and between different centres, and the need for improved diabetes care in people on PD. Further work is required to establish agreed standards/recommendations of diabetes care in this population. Our findings highlight the necessity of an integrated multidisciplinary approach to improve the standard of diabetes care for people on PD.

Narrative Review of Glycemic Management in People With Diabetes on Peritoneal Dialysis.

There is an increasing number of people with diabetes on peritoneal dialysis (PD) worldwide. However, there is a lack of guidelines and clinical recommendations for managing glucose control in people with diabetes on PD. The aim of this review is to provide a summary of the relevant literature and highlight key clinical considerations with practical aspects in the management of diabetes in people undergoing PD. A formal systematic review was not conducted because of the lack of sufficient and suitable clinical studies. A literature search was performed using PubMed, MEDLINE, Central, Google Scholar and ClinicalTrials.gov., from 1980 through February 2022. The search was limited to publications in English. This narrative review and related guidance have been developed jointly by diabetologists and nephrologists, who reviewed all available current global evidence regarding the management of diabetes in people on PD.We focus on the importance of individualized care for people with diabetes on PD, the burden of hypoglycemia, glycemic variability in the context of PD and treatment choices for optimizing glucose control. In this review, we have summarized the clinical considerations to guide and inform clinicians providing care for people with diabetes on PD.

People With Type 1 Diabetes of African Caribbean Ethnicity Are at Increased Risk of Developing Sight-Threatening Diabetic Retinopathy.

OBJECTIVE: There is limited information on the effect of ethnicity on the development of referable sight-threatening diabetic retinopathy (STDR) in people with type 1 diabetes. This study describes the risk factors for STDR in a diverse cohort of people with type 1 diabetes attending a regional diabetes eye screening service. RESEARCH DESIGN AND METHODS: Clinical and digital retinal imaging data from 1,876 people with type 1 diabetes (50% women, 72.1% Caucasian, 17.3% African Caribbean, 2.9% Asian, and 7.6% other) with no retinopathy at baseline, attending surveillance eye screening were reviewed. Referable STDR was defined as the presence of any moderate to severe nonproliferative or preproliferative diabetic retinopathy or proliferative diabetic retinopathy or maculopathy in either eye as per U.K. National Diabetic Eye Screening criteria. Median follow-up was 6 years. RESULTS: The median (interquartile range) age of the cohort was 29 (21, 41) years. Of the cohort of 1,876 people, 359 (19%) developed STDR. People who developed STDR had higher baseline HbA1c, raised systolic blood pressure (SBP), longer diabetes duration, and were more often of African Caribbean origin (24% vs. 15.6%; P < 0.05 for all). In multivariable Cox regression analyses, African Caribbean ethnicity (hazard ratio [HR] 1.39, 95% CI 1.09-1.78, P = 0.009), baseline SBP (HR 1.01, 95% CI 1.00-1.01, P = 0.033), and baseline HbA1c (HR 1.01, 95% CI 1.00-1.01, P = 0.0001) emerged as independent risk factors for STDR. CONCLUSIONS: We observed that people with type 1 diabetes of African Caribbean ethnicity are at significantly greater risk of STDR. Further research is required to understand the mechanisms that explain this novel observation.

Management of adults with diabetes on dialysis: Summary of recommendations of the Joint British Diabetes Societies guidelines 2022.

Diabetes is the commonest cause of end-stage kidney disease in many parts of the world, and many people on dialysis programmes live with diabetes. Such people are vulnerable to complications from their diabetes, and their care may be fragmented due to the many specialists involved. This updated guidance from the Joint British Diabetes Societies aims to review and update the 2016 guidance, with particular emphasis on glycaemic monitoring in the light of recent advances in this area. In addition, the guidance covers clinical issues related to the management of diabetes in people on peritoneal dialysis, along with acute complications such as hypoglycaemia and ketoacidosis, and chronic complications such as foot and eye disease.

Approach to the Patient With Adrenal Hemorrhage.

Adrenal hemorrhage is an uncommon, underrecognized condition that can be encountered in several clinical contexts. Diagnosing adrenal hemorrhage is challenging due to its nonspecific clinical features. Therefore, it remains a diagnosis that is made serendipitously on imaging of acutely unwell patients rather than with prospective clinical suspicion. Adrenal hemorrhage can follow abdominal trauma or appear on a background of predisposing conditions such as adrenal tumors, sepsis, or coagulopathy. Adrenal hemorrhage is also increasingly reported in patients with COVID-19 infection and in the context of vaccine-induced immune thrombocytopenia and thrombosis. Unexplained abdominal pain with hemodynamic instability in a patient with a predisposing condition should alert the physician to the possibility of adrenal hemorrhage. Bilateral adrenal hemorrhage can lead to adrenal insufficiency and potentially fatal adrenal crisis without timely recognition and treatment. In this article, we highlight the clinical circumstances that are associated with higher risk of adrenal hemorrhage, encouraging clinicians to prospectively consider the diagnosis, and we share a diagnostic and management strategy.

Parathyroid carcinoma during pregnancy: a novel pathogenic CDC73 mutation - a case report.

BACKGROUND: Parathyroid carcinoma is an uncommon cause of PTH-dependent hypercalcemia. Only a handful of cases have been reported of parathyroid carcinoma during pregnancy. CASE PRESENTATION: Twenty-four - Year - old female presented with proximal myopathy was found to have hypercalcemia. Her serum corrected total calcium was - 15 mg/dl (8.5 - 10.3), serum phosphate - 2.3 mg/dl (2.5 - 4.5), intact PTH - 118 pg/ml (20 - 80), Vitamin D - 15 ng/ml and Urine Ca/Cr ratio - 2.1 (0.1 - 0.2). Her CECT-neck revealed a well-defined mass lesion posterior to the right lobe of the thyroid - 2.6 cm × 2.5 cm × 2.9 cm in size. She was started on vitamin D supplementation, and she underwent right lower focal parathyroidectomy. Her PTH levels normalized following surgery. Her histology revealed an atypical parathyroid adenoma. She was treated with calcium and vitamin D. Her follow up was uneventful. One year following initial surgery the patient became pregnant and at 16 weeks of POA, the patient presented with a rapidly enhancing neck mass for one week duration. Her biochemical investigations were suggestive of a recurrence of primary hyperparathyroidism. Her ultrasound scan of the neck revealed a well-defined discreate hypoechoic nodule, superior to the thyroid isthmus which was confirmed by a non-contrast MRI scan of the neck. She underwent an uncomplicated second trimester parathyroid tumour excision with normalization of post op PTH. Her histology revealed a parathyroid carcinoma with vascular and capsular invasion. Her genetic studies revealed a novel frameshift mutation of the CDC73 gene. She was treated with calcium and vitamin D supplementation and closely followed up with ionized calcium and PTH levels which were normal throughout the pregnancy. She had an uncomplicated caesarean section at a POA of 37 weeks. Currently she is twelve weeks post-partum, in remission of disease. CONCLUSION: This case shows the importance of stringent follow up of atypical parathyroid adenoma patients, the benefit of second trimester surgery in management of hypercalcemia due to parathyroid carcinoma during pregnancy and the importance of identifying the novel CDC73 gene mutation.

Ischemic Stroke Subtypes: Socio-demographic Factors, Risk Factors, and Outcomes in Southern Sri Lanka.

BACKGROUND: Stroke is a heterogeneous, catastrophic disease. A comprehensive clinical analysis of ischemic stroke (IS) risk factors and outcomes is crucial for optimum management in resource-poor settings. METHODS: A prospective cross-sectional study of acute cerebrovascular disease (ACVD) involving 592 patients was conducted in a tertiary care center in Sri Lanka from November 2018 to May 2019. We aimed to describe the ACVD subtypes and the relationship of IS subtypes and subtype-categories (as defined by the Oxfordshire Community Stroke Project clinical classification) with risk factors, severity, and outcome. RESULTS: The majority (63.3%) had IS. Partial anterior circulation syndromes (PACS), total anterior circulation syndrome (TACS), posterior circulation syndromes (POCS), and lacunar syndromes (LACS) accounted for 102 (29.7%), 58 (16.9%), 88 (25.7%) and 95 (27.7%) of the cases, respectively. The most common PACS sub-category was higher-cerebral-dysfunction-with-homonymous-hemianopia (HCD+HH,39 cases;38.2%). Cerebellar-signs-without-long-tract-signs (CS-LTS) sub-category constituted the highest among POCS (47 cases; 53.4%). The leading sub-category within LACS was pure-motor (PM) strokes (43 cases; 45.3%).Patients aged ≥50 years (adjusted-OR [AOR]2.439; 95%CI,1.163-5.116;P=.018), IHD(AOR 2.520; 95%CI,1.347-4.713; P=.004) and BMI>23kg/m2(AOR 2.607; 95% CI,1.420-4.784; P=.002) were 2.5 times more likely to associate with TACS. Patients with a history of TIA (AOR 1.910; 95%CI,1.036-3.524; P=.038) and arrhythmias (AOR 5.933; 95%CI,3.294-10.684; P<.001) were 1.9 and 5.9 times more likely to be associated with POCS respectively. Those with hypertension were 2.3 times more likely to associate with LACS (AOR 2.233; 95%CI,1.270-3.926; P=.005).NIHSS(P<.001), mRS on admission (P=.001) and in 3 months (P<.001), deaths during hospital stay (P=.003) and within 28 days (P<.001) had a stronger relationship with individual stroke subtypes. CONCLUSION: The comparative risk of different IS subtypes depends on different risk factors. The findings of this study demonstrate that sub-categories within each stroke subtype may behave independently with regard to risk factors and outcomes, thus warranting the need for individual assessment.

A challenging case of Cushing's disease complicated with multiple thrombotic phenomena following trans-sphenoidal surgery; a case report.

BACKGROUND: Cushing's syndrome occurs due to overproduction of cortisol from adrenal glands. Endogenous hypercortisolemia can occur secondary to adrenocorticotropic hormone (ACTH) dependent as well as independent causes. The presence of non-specific symptoms and signs contributes to a delay in diagnosis. Early identification and prompt definitive management is crucial. It is important to be alert about the post-operative complications including multiple thrombotic phenomena, which can add to the mortality. We report a case of Cushing's disease in a young female managed with trans-sphenoidal surgery, followed by a challenging post-operative period complicated with multiple thrombotic phenomena, ultimately succumbed. CASE PRESENTATION: A 32-year-old Sri Lankan female presented with overt features of Cushing's syndrome and diagnosed to have ACTH dependent Cushing's disease with pituitary microadenoma. She underwent trans-sphenoidal surgery, following which she developed fatal multiple complications including diverticular rupture and ischemic colitis, needing hemicolectomy, followed by a parieto-occipital infarction. CONCLUSION: This case highlights important and aggressive complications associated with Cushing's syndrome giving rise to a challenging post-operative course. Diverticular rupture had been described in association with hypercortisolemia and this case adds to the existing literature. Post-operative ischemic colitis and stroke which contributed to the death of this patient could have been due to the procoagulant state associated with Cushing's syndrome, with a high risk during the immediate post-operative period. This emphasizes the need to consider post-operative thromboprophylaxis in patients undergoing surgery for Cushing's syndrome.

Delayed Hypoparathyroidism following Thyroidectomy, a Diagnostic Conundrum: A Report of Three Cases from Sri Lanka.

INTRODUCTION: Hypoparathyroidism, which is a common complication following total thyroidectomy can be transient in majority and permanent in 1.5% of the patients and usually occurs secondary to an inadvertent removal of parathyroid glands, mechanical or thermal injury or disruption of the vasculature. In some patients, it is observed that symptoms of hypocalcemia can occur for the first time several years after the surgery, which is known as "delayed hypoparathyroidism." We report three cases of delayed hypoparathyroidism from Sri Lanka, presenting several years after total thyroidectomy. Case Presentation. Case 1- a 60-year-old Sri Lankan woman who presented with symptomatic hypocalcemia for the first time, 30 years after total thyroidectomy for follicular thyroid carcinoma. Case 2- a 53-year-old Sri Lankan woman presenting with neuropsychiatric manifestations of hypocalcemia for the first time, 12 years after total thyroidectomy for papillary thyroid carcinoma. Case 3- a 49-year-old Sri Lankan woman developing symptoms of hypocalcemia for the first time, 11 years after completion of thyroidectomy for papillary thyroid carcinoma. All these patients were detected to have low parathyroid hormone levels, without an alternative etiology for hypoparathyroidism, hence leading to a diagnosis of delayed post-thyroidectomy hypoparathyroidism. CONCLUSION: Delayed hypoparathyroidism is a rare phenomenon, which is secondary to progressive atrophy of parathyroid glands and slowly progressive hypovascularization of parathyroids due to scar tissue retraction following thyroidectomy. The nonspecific nature of hypocalcemic symptoms and lack of continuous follow-up for a long time after thyroidectomy could contribute to a further delay in diagnosis. However, it is an important diagnosis to consider in any patient with a history of neck surgery presenting with hypocalcemia, irrespective of the time duration of surgery, as timely diagnosis and treatment can prevent long-term complications of hypocalcemia and improve the quality of life.

The Impact of SARS-Cov-2 Virus Infection on the Endocrine System.

Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) virus has spread across the globe rapidly causing an unprecedented pandemic. Because of the novelty of the disease, the possible impact on the endocrine system is not clear. To compile a mini-review describing possible endocrine consequences of SARS-CoV-2 infection, we performed a literature survey using the key words Covid-19, Coronavirus, SARS CoV-1, SARS Cov-2, Endocrine, and related terms in medical databases including PubMed, Google Scholar, and MedARXiv from the year 2000. Additional references were identified through manual screening of bibliographies and via citations in the selected articles. The literature review is current until April 28, 2020. In light of the literature, we discuss SARS-CoV-2 and explore the endocrine consequences based on the experience with structurally-similar SARS-CoV-1. Studies from the SARS -CoV-1 epidemic have reported variable changes in the endocrine organs. SARS-CoV-2 attaches to the ACE2 system in the pancreas causing perturbation of insulin production resulting in hyperglycemic emergencies. In patients with preexisting endocrine disorders who develop COVID-19, several factors warrant management decisions. Hydrocortisone dose adjustments are required in patients with adrenal insufficiency. Identification and management of critical illness-related corticosteroid insufficiency is crucial. Patients with Cushing syndrome may have poorer outcomes because of the associated immunodeficiency and coagulopathy. Vitamin D deficiency appears to be associated with increased susceptibility or severity to SARS-CoV-2 infection, and replacement may improve outcomes. Robust strategies required for the optimal management of endocrinopathies in COVID-19 are discussed extensively in this mini-review.

Endocrine vigilance in COVID-19.

Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is a novel coronavirus responsible for a pandemic that emerged in December 2019. Heterogeneous clinical forms are described from asymptomatic to severe hypoxaemic acute respiratory syndrome with multisystem organ failure. The impact of this coronavirus disease 2019 on the endocrine glands remains unknown. However, the results of previous studies on viruses from the same family allow us to write proposals for patients followed for chronic endocrine diseases. Currently, if these subjects are infected with SARS-CoV-2, they must not stop their treatment. In some cases, hormone replacement doses have to be increased. In case of worsening clinical signs, hormonal biological monitoring must be done. This article will be helpful for improving the management of chronic endocrine diseases that could affect thyroid, adrenals, gonads and pituitary gland functions. Proposals could be applied in COVID-19 infected subjects or in those who have been in contact with COVID-19 infected people.

Prevention and management of COVID-19 among patients with diabetes: an appraisal of the literature.

The coronavirus disease 2019 (COVID-19) pandemic has emerged as one of the greatest challenges faced by humankind in the recent past. People with diabetes and related comorbidities are at increased risk of its complications and of COVID-19-related death. Older age, multi-morbidity, hyperglycaemia, cardiac injury and severe inflammatory response are predictors of poor outcome. The complex interplay between COVID-19, diabetes and the effects of related therapies is being explored. Most patients experience a mild illness with COVID-19, while people with diabetes are at increased risk of severe disease. Optimising glycaemic control and adopting measures to prevent disease spread are critical aspects. The management of mild disease is supportive, while very many immunomodulatory and antiviral therapies are being investigated for the treatment of severe disease. Several of these agents have specific considerations for use in people with diabetes. Since mass population lockdowns are considered a key step in controlling disease spread, it follows that, in addition to the direct vulnerability to severe COVID-19, people with diabetes can be affected by limited access to healthcare, insulin, other medications and blood glucose monitoring equipment. Measures to prevent disease spread at the individual and community level are the key to mitigating the rapidly escalating pandemic, while agents for chemoprophylaxis and vaccines are being explored. People with diabetes should be recognised as a vulnerable group for complicated disease and are at risk during times of disturbed social systems. Strategies are needed to safeguard the health of patients with diabetes during the pandemic. This review summarises the current knowledge and perceived challenges for prevention and management of COVID-19 in people with diabetes.

Hyperparathyroidism Jaw Tumor Syndrome Presenting as Recurrent Femur Fractures in a Young Woman; a Rare Presentation of a Rare Disease.

BACKGROUND: Primary hyperparathyroidism usually occurs secondary to parathyroid adenoma, multiglandular hyperplasia, or parathyroid carcinoma. The patients usually present with incidentally discovered high calcium level and systemic or skeletal manifestations. In young patients with primary hyperparathyroidism, familial syndromes including multiple endocrine neoplasia types 1, 2, and 4 and hyperparathyroidism jaw tumor syndrome should be considered. Case Description. We present a case of a 22-year-old Sri Lankan woman who presented with femur fractures in a background of childhood nephroblastoma and maxillary fibro-osseous tumor. The patient had biochemical parameters suggestive of primary hyperparathyroidism with a parathyroid mass. The histology following excision of the mass revealed a parathyroid adenoma. Based on the associated clinical manifestations, hyperparathyroidism jaw tumor syndrome was suspected, and genetic studies reported a positive CDC73 mutation with a whole-gene deletion of exon 1-17. CONCLUSION: Hyperparathyroidism jaw tumor syndrome is an important diagnosis to consider in a young patient presenting with classic clinical features due to the risk of malignancy, familial involvement, and need to monitor for progressive systemic manifestations. As this is a rare disease, it can often be missed due to low degree of suspicion and the ability of the jaw tumor to mimic a metastatic deposit.

Acute disseminated melioidosis giving rise to pneumonia and renal abscesses complicated with thrombotic thrombocytopenic purpura in a post partum woman: a case report.

BACKGROUND: Melioidosis is an established endemic infection in Sri Lanka, caused by Burkholderia pseudomallei, a gram negative bacterium distributed in saprophytes in soil and surface water. Main mode of transmission is via percutaneous inoculation. Pneumonia is the most common presentation in acute disease. CASE PRESENTATION: We report a 33 year old previously healthy Sinhalese female with an occupational exposure to surface water in paddy fields, who was on postpartum day 6 following an uncomplicated pregnancy and delivery via an elective caesarian section. She presented with a 1 day history of breathlessness, preceded by a brief episode of fever. She had occasional right side coarse crackles and pitting oedema of both lower limbs. Shortly after admission, she developed type one respiratory failure needing invasive mechanical ventilation. Initial chest x-ray revealed slight obliteration of right medial diaphragmatic border while echocardiogram revealed moderate pulmonary hypertension. Computed tomography pulmonary angiogram excluded a pulmonary embolism, but revealed bilateral multi-lobar consolidation. Abdominal computed tomography demonstrated bilateral pyelonephritis with renal abscesses. As initial cultures were inconclusive, melioidosis antibody levels were done due to high degree of suspicion, which was found to be positive with a titer of 1:2560. A diagnosis of melioidosis was made based on the suggestive clinical picture, exposure history and the highly positive antibody level. She developed left side focal seizures together with thrombocytopenia and microangiopathic haemolytic anemia, suggestive of thrombotic thrombocytopenic purpura. Magnetic resonance imaging of brain was negative for cerebral abscesses but revealed extensive minute haemorrhagic foci throughout the cerebrum. Thus, the final diagnosis was acute melioidosis causing pneumonia and renal abscesses, complicated with thrombotic thrombocytopenic purpura and sepsis. She demonstrated dramatic response to high dose meropenem and co-trimoxazole along with plasmapheresis. Four weeks after treatment, the antibody titer came down to 1:320. Melioidosis antibody was absent in the baby. CONCLUSIONS: This case was challenging as it was an atypical presentation of melioidosis during postpartum leading to a diagnostic conundrum. This highlights the need to look into the effect of pregnancy and postpartum as added risk factors. High index of suspicion is necessary to avoid diagnostic delays.