ABSTRACT
Left ventricular non-compaction cardiomyopathy (LVNC), or non-compaction cardiomyopathy (NCCM), is defined by pronounced left ventricular trabeculations and deep intertrabecular recesses connecting with the ventricular cavity. Patients with NCCM can be asymptomatic or have severe complications, including heart failure, arrhythmias, thromboembolism, and sudden cardiac death. Our case discusses a patient with shortness of breath who was found to have a newly decreased ejection fraction. The workup revealed non-ischemic cardiomyopathy and cardiac MRI showed hyper-trabeculations consistent with NCCM. The patient was started on oral anticoagulation and guideline-directed medical therapy (GDMT) and discharged with an event monitor. NCCM stands as a relatively rare and enigmatic condition, often veiled in ambiguity. The absence of standardized diagnostic and management protocols further complicates its clinical landscape. While echocardiography is the primary diagnostic tool, its tendency for under-diagnosis poses a significant challenge. Conversely, advanced imaging modalities like cardiac MRI may lead to instances of overdiagnosis. Treatment approaches are non-specific, incorporating GDMT, anticoagulation, implantable cardioverter-defibrillator placement, and genetic testing paired with counseling. Prioritizing genetic research is crucial to uncover tailored therapeutic interventions. Establishing consensus guidelines and refining diagnostic accuracy are pivotal steps toward mitigating the risks associated with under and over-diagnosis.
ABSTRACT
Adult-onset Still's disease (AOSD) stands as a perplexing condition with diverse clinical manifestations, posing significant diagnostic challenges for healthcare professionals. This case report delves into the clinical trajectory, diagnostic challenges, treatment strategies, and outcomes experienced by a 67-year-old female with AOSD. This report advocates for considering AOSD as a potential diagnosis in patients presenting with systemic inflammatory symptoms, especially when other conditions have been ruled out. It highlights the complexity of AOSD and the importance of interdisciplinary collaboration, close monitoring, and personalized treatment strategies to optimize patient outcomes.
ABSTRACT
Posterior scleritis is a rare inflammatory eye condition affecting the posterior segments of the sclera and is more prevalent in females. Its clinical presentation, often nonspecific, includes ocular pain, headache, and vision loss. Misdiagnosis is common due to a lack of specific symptoms posing a potential threat to vision. The etiology is often tied to rheumatic diseases, such as rheumatoid arthritis (RA), systemic erythematous lupus (SLE), and anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis. Posterior scleritis poses diagnostic challenges, mimicking many other ocular conditions, hence necessitating a thorough clinical eye exam. Laboratory studies, including inflammatory markers and markers of rheumatic diseases, may identify underlying systemic diseases. Imaging, including B-scan ultrasound and magnetic resonance imaging (MRI), aids in accurate diagnosis. Treatment involves non-steroidal anti-inflammatory drugs (NSAID), as well as topical corticosteroids for mild disease and systemic corticosteroids for severe disease. Biologic therapy has become increasingly significant for refractory cases. A multidisciplinary approach involving ophthalmology and rheumatology is crucial in the management of this potential sight-threatening disease. This case report highlights a 46-year-old woman with a history of RA-associated posterior scleritis.
ABSTRACT
Pleural effusions can be secondary to several different etiologies. Sometimes, they can be related to hypothyroidism. We present a case of massive pleural effusion resulting from hypothyroidism. A 75-year-old male with a history of liver cirrhosis, hypothyroidism, and medication non-adherence presented to the emergency department (ED) with shortness of breath and altered mental status. Physical exam and chest imaging were consistent with right-sided pleural effusion. Effusion was exudative. Multiple recurrences complicated the hospitalization despite thoracentesis and pleurodesis. Labs revealed hypothyroidism, and finally, the patient was started on hormone replacement, resulting in the resolution of the effusion. Pleural effusion is a rare manifestation of hypothyroidism, thought to be mediated by vascular endothelial factors. Pleural fluid analysis shows both exudative and transudative patterns. Hormonal replacement is the mainstay of treatment. Clinicians need to be aware of the rare etiologies of pleural effusion. Depending on the patient's presentation, due work-up should be done to ensure a timely diagnosis and management.
ABSTRACT
Non-typhoidal Salmonella typically presents with gastroenteritis. However, an invasive Salmonella infection, which may be typically seen in immunocompromised patients, has a propensity for aortic involvement, especially in patients with risk factors for atherosclerosis. Here we present a 60-year-old female with multiple comorbid conditions and currently on immunosuppressants for rheumatoid arthritis, who presented with nausea, vomiting, and fever of three weeks duration and was found to have Salmonella bacteremia. Blood cultures were positive for Salmonella enterica. Computed tomography (CT) abdomen with contrast was concerning for mycotic aortitis. The patient underwent endovascular repair of an aortic ulcer and was treated with a six-week course of ceftriaxone. Mycotic aneurysm is a rare but potentially fatal complication of invasive Salmonella infection. It occurs typically in older men with atherosclerotic risk factors. It mostly presents as fever, back pain, and/or abdominal pain. Our patient was a middle-aged female who presented with non-specific symptoms. CT angiogram is the diagnostic modality of choice and treatment may require surgical vascular repair and long-term antibiotics. A high level of suspicion is needed to diagnose Salmonella-related mycotic aneurysm/aortitis. Early diagnosis and treatment may improve the mortality.
ABSTRACT
Subacute thyroiditis (SAT) is an inflammatory disease of the thyroid gland. It can present with overlapping features of other etiology of thyroiditis. It can present with thyroid enlargement and systemic symptoms such as fever as well as neck pain and may be confused with infectious thyroiditis. It can be difficult to diagnose and present as fever of unknown origin (FUO). A good history, physical examination, laboratory investigation, as well as imaging may aid in the correct diagnosis and prevent the inappropriate use of antibiotics. Treatment is usually with nonsteroidal anti-inflammatory drugs (NSAIDs) as well as corticosteroids. We herein present a case of SAT presenting as FUO. We highlighted the importance of proper clinical evaluation, the importance of thyroid imaging, and how to differentiate other forms of thyroiditis.
ABSTRACT
Neuroblastoma is the most common embryonal tumor of childhood and has a variable presentation. Stage 4S neuroblastoma, described as a localized primary tumor in an infant with metastasis to skin, liver, or bone marrow, is an exception to the poor prognosis seen in widespread metastasis of neuroblastoma. Survival in infants with this stage of the disease is over 90%. Stage 4S with massive liver involvement, however, confers a poor prognosis. We need more research on the optimum treatment modality for patients with Stage 4S disease and massive hepatomegaly to improve patient outcomes.
ABSTRACT
A fecalith is a common cause of acute appendicitis, and laparoscopic surgery is the mainstay of its management. Literature review shows that a fecalith may be retained in the gut following a laparoscopic appendectomy in some rare cases. In most cases, the fecalith becomes symptomatic with time due to the formation of an abscess, fistulous tract, or inflammation of the appendicular stump (stump appendicitis). We report a case of retained appendicular fecalith presenting with symptoms similar to acute appendicitis, 15 years after laparoscopic appendectomy.
ABSTRACT
The aim of this study was to observe the association between maternal undernutrition and metabolic risk indicators in newborns at birth. Fifty-nine expectant mothers between 28 and 40 weeks of gestation were included after obtaining their informed consent. Mothers were divided into undernourished, normally nourished and overnourished groups. A total of 54 deliveries were followed-up, and cord blood samples were collected. Metabolic status at birth was assessed by determining the cord blood concentrations of glucose, insulin and lipids and by measuring insulin resistance through homeostasis model assessment. Metabolic risk indicators in the offspring were compared following mothers' nutrition status (under and normal nourished groups). We found that concentrations of glucose (5.31±2.01 v. 4.69±2.22 mmol/l, P=0.01), total cholesterol (2.51±1.52 v. 1.84±0.66 mmol/l, P=0.04), triglycerides (0.85±1.12 v. 0.34±0.24 mmol/l, P=0.00) and low-density lipoprotein (LDL)-cholestrol (1.26±0.93 v. 1.02±0.50 mmol/l, P=0.04) were significantly high in the offspring born to undernourished mothers. LDL-cholestrol remained significantly high in the undernourished group even after adjustment for potential confounders. Furthermore, a weak association was observed between maternal body fat mass with serum leptin (r=0.272, P=0.05) and maternal body mass Index with LDL-cholestrol in the cord blood (r=0.285, P=0.05). Our results showed that offspring of undernourished mothers had a relatively higher metabolic risk profile including LDL-cholestrol compared with normal nourished group, suggesting that maternal undernutrition may influence metabolic risk markers of the newborn at birth. We recommend that these results should be confirmed by a longitudinal study with a larger sample size.
Subject(s)
Infant, Newborn/blood , Malnutrition/epidemiology , Prenatal Nutritional Physiological Phenomena , Adult , Anthropometry , Female , Humans , Pakistan/epidemiology , Pilot Projects , Pregnancy , Young AdultABSTRACT
The present GUARD study was a prospective, non-interventional study evaluating the clinical effectiveness, safety and tolerability of vildagliptin with or without metformin in adult patients with type 2 diabetes mellitus (T2DM) studied in routine clinical practice. Patients were enrolled from countries across four geographical regions. The primary endpoint was change in glycated haemoglobin (HbA1c) concentration from baseline after 24 weeks of treatment with vildagliptin with or without metformin. Of 19 331 patients analysed, 3511 received vildagliptin and 15 820 received vildagliptin plus metformin. At week 24, the mean HbA1c was reduced significantly from baseline by -1.27% (vildagliptin: -1.17%; vildagliptin plus metformin: -1.29%; p < 0.0001). Significant reductions in HbA1c from baseline were consistently reported regardless of patient age, body mass index (BMI) or baseline HbA1c. Weight and BMI were also significantly reduced from baseline. Vildagliptin treatment with or without metformin was generally well tolerated. It provided clinically relevant glycaemic and weight control, and was well tolerated in a large multi-ethnic population of patients with T2DM studied in routine clinical practice.
Subject(s)
Adamantane/analogs & derivatives , Diabetes Mellitus, Type 2/drug therapy , Hypoglycemic Agents/therapeutic use , Metformin/therapeutic use , Nitriles/therapeutic use , Pyrrolidines/therapeutic use , Adamantane/therapeutic use , Adult , Aged , Blood Glucose/drug effects , Blood Glucose/metabolism , Body Mass Index , Body Weight/drug effects , Diabetes Mellitus, Type 2/blood , Drug Therapy, Combination/methods , Female , Glycated Hemoglobin/drug effects , Glycated Hemoglobin/metabolism , Humans , Hypoglycemia/chemically induced , Male , Middle Aged , Prospective Studies , Treatment Outcome , VildagliptinABSTRACT
AIMS: To observe the effects of active glucose monitoring, alteration of drug dosage and timing, dietary counselling and patient education in the occurrence of acute diabetic complications in fasting individuals with diabetes during the month of Ramadan. METHODS: This prospective study was conducted at the outpatient department of the Baqai Institute of Diabetology and Endocrinology. Two educational sessions, one about drug dosage and timing alteration and glucose monitoring, and the other about dietary and lifestyle modifications, were given to the patients by a doctor and a dietician, respectively. Patients who had been recruited were advised to note their blood glucose readings on a chart for at least 15 fasting days, twice a day with at least one reading in the fasting state. RESULTS: A total of 3946 readings were obtained in 110 subjects; 82 readings were in the hypoglycaemic range, and there were 22 episodes of symptomatic hypoglycaemia and 60 episodes of biochemical hypoglycaemia observed in 27 patients. Seven patients experienced symptomatic hypoglycaemia, whereas 20 patients had biochemical hypoglycaemia. Symptomatic hypoglycaemic episodes showed a downward trend from weeks 1 to 4. The highest frequencies of hypo- and hyperglycaemic episodes were observed pre-dawn. None of the patients developed diabetic ketoacidosis or hyperglycaemic hyperosmolar state. CONCLUSION: We observed that, with active glucose monitoring, alteration of drug dosage and timing, dietary counselling and patient education, the majority of the patients did not have any serious acute complications of diabetes during Ramadan.
Subject(s)
Blood Glucose/metabolism , Diabetes Mellitus, Type 1/drug therapy , Diabetes Mellitus, Type 2/drug therapy , Fasting , Holidays , Hypoglycemic Agents/administration & dosage , Insulin/administration & dosage , Islam , Blood Glucose Self-Monitoring , Dehydration/blood , Dehydration/prevention & control , Diabetes Mellitus, Type 1/blood , Diabetes Mellitus, Type 1/complications , Diabetes Mellitus, Type 2/blood , Diabetes Mellitus, Type 2/complications , Diabetic Ketoacidosis/blood , Diabetic Ketoacidosis/prevention & control , Fasting/adverse effects , Female , Humans , Hyperglycemia/blood , Hyperglycemia/prevention & control , Hypoglycemia/blood , Hypoglycemia/prevention & control , Male , Patient Education as Topic , Prospective StudiesABSTRACT
The primary objective of the study is to assess the efficacy of the 'Disease Modifying AntiRheumatic Drugs (DMARDs) on the disease activity in Rheumatoid Arthritis (RA) in the local patients of Karachi. The secondary objective is to evaluate whether the combination of two concurrent DMARDs (Combination Therapy) is superior to a single DMARD (Mono-therapy). This is an open labeled retrospective case series. One hundred and five consecutive patients fulfilling 1987 ACR criteria for the diagnosis of RA were initially selected from the case notes of out patients department. Sixty nine patients fulfilled the inclusion criteria and were finally recruited for analysis. Details of the Tender Joint Count (TJC), Swolen Joint Count (SJC), Patient Global Assessment (PGA) and ESR were obtained at six weeks, three months, six months and one year. Out of the 69 patients studied 48 were in the mono-therapy group and 21 in the combination therapy group. Methotrexate (MTX) was the most commonly used single DMARD (75%) as well as the most frequent component of the combination groups (85%). The TJC, SJC and PGA analyses of all patients show that DMARDs are effective agents for clinically controlling RA activity. The speed of their beneficial effect is slow and unlike analgesics and NSAIDS, may take up to six weeks to start working. The 6 week responses showed 32.49% improvement in TJC, 33.19% improvement in SJC and 59% better responses in PGA. This response continued to show further improvement and at six months when TJC improved by 63.41%, SJC by 53.21% and PGA with 81% better responses. After 6 months the response reached a plateau but nevertheless maintained until 1 year with improvements in TJC by 66.23%, SJC by 56.48% and PGA with 88.23% better responses. The changes in ESR did not go parallel with the other three outcome measures. The mean baseline ESR of 56 reduced to 44 at 6 weeks but rose again gradually to 54 at 1 year. The sub-group analysis did not show the overall superiority of combination therapy over mono-therapy. DMARDs are effective in controlling disease activity in RA. Their effect starts slowly over 6 week and may take up to 6 months to show full benefits. The beneficial effect was maintained for at least 1 year. Sub-group analysis did not show any advantage of combination therapy over mono-therapy in this series of patients. Methotrexote being the most frequently used DMARDs in both groups and being most cost effective agent seems to be the most useful drug in RA in the developing world.
Subject(s)
Antirheumatic Agents/therapeutic use , Arthritis, Rheumatoid/drug therapy , Antirheumatic Agents/economics , Cost-Benefit Analysis , Drug Therapy, Combination , Female , Humans , Male , Methotrexate/therapeutic use , Pakistan , Retrospective Studies , Treatment OutcomeABSTRACT
AIMS: The aim of the present study was to estimate the direct cost of treatment of diabetic foot ulcer at a tertiary care hospital in Karachi, Pakistan in order to assess the extent of the economic burden which it imposes. METHODS: Out of 383 patients seen at Foot clinic of Baqai Institute of Diabetology and Endocrinology (BIDE), records of 214 patients were analyzed while 169 patients left against medical advice (LAMA). The UT system was used to classify ulcer types. Information was retrieved on resource consumption (physician services, chiropody, investigations, medicines, hospital care and surgical procedures). Interventions were summed and multiplied by the unit price of each resource, using charges levied at BIDE in the year 2005, in order to calculate the total cost of treatment. RESULTS: 64% were male, with mean age 52.7 +/- 10.2 years. Mean duration of diabetes was 16.2 +/- 6.6 years. Majority (62.1%) were Grade 2 ulcer. The estimated direct cost of management increased from 2700 +/- 250 rupees (21 +/- 2 pounds) for a UT grade 1, stage B ulcer to 37,415 +/- 24,125 rupees (288 +/- 186 pounds) for UT grade 2, stage D and 49,058 +/- 30,144 rupees (378 +/- 232 pounds) for UT grade 3, stage D ulcers, respectively. The mean direct cost of major amputation (transtibial or transfemoral) was 46,182 +/- 30,742 ( 356 +/- 237 pounds) whilst the cost of a minor amputation was 50,494 +/- 30,488 rupees (389 +/- 235 pounds). CONCLUSIONS: This retrospective study, despite having limitations, is important for a developing world country with limited data on health economics. Further larger scale prospective studies are needed to address this issue in more detail.
Subject(s)
Cost of Illness , Diabetic Foot/economics , Adult , Amputation, Surgical/economics , Diabetic Foot/therapy , Female , House Calls/economics , Humans , Length of Stay/economics , Male , Middle Aged , Pakistan , Retrospective StudiesABSTRACT
The aim of this questionnaire based survey was to assess the glycaemic control care and management of our fasting diabetic subjects. This retrospective study was carried out at Baqai Institute of Diabetology and Endocrinology, its affiliated centers and Memon Diabetic Centre of Karachi. Data was collected by a questionnaire including socio-demographic data, duration of diabetes, life style, diet and treatment during and preceding Ramadan of year 2004. All Known diabetic Muslim subjects except children below 10 years were included. The subjects were asked if they had observed any of the hypoglycemic or hyperglycemic symptoms. The major severity of the symptoms was assessed depending upon the assistance needed. The plasma glucose level during these episodes was recorded where it was available. During the month of Ramadan 327 out of 453 subjects (72.5%) fasted. Mean age of subjects was 50.3 +/- 12.6 years. Average duration of diabetes was 9.3 +/- 7.3 years. Majority of the subjects (96.3%) fasted with type 2 diabetes while only 3.7% fasted with type I diabetes. Subjects fasted for an average of 25 days. Overall prevalence of hypoglycemia and hyperglycemia was 21.7 and 19.8%, respectively. Only 4.0% had major hypoglycemic episodes, while 8% had major hyperglycemic episodes. The present study suggests that all diabetic subjects who intend to fast should be counseled before Ramadan about change in medication timings and dose, dietary changes and pattern of physical activity and about role of self-monitoring of blood glucose especially during acute symptoms.
Subject(s)
Diabetes Mellitus, Type 1/blood , Diabetes Mellitus, Type 2/blood , Fasting/adverse effects , Fasting/blood , Holidays , Hyperglycemia/blood , Hypoglycemia/blood , Islam , Diabetes Mellitus, Type 1/complications , Diabetes Mellitus, Type 1/epidemiology , Diabetes Mellitus, Type 2/complications , Diabetes Mellitus, Type 2/epidemiology , Female , Humans , Hyperglycemia/complications , Hyperglycemia/epidemiology , Hypoglycemia/complications , Hypoglycemia/epidemiology , Male , Middle Aged , Pakistan/epidemiologyABSTRACT
The aim of this study was to evaluate the effectiveness ofDongsulin (rDNA, insulin) in maintaining HbA1c level in a normal clinical practice setting and secondly to assess weight gain, episodes of hypoglycaemia, insulin dose change and its safety. Fifty two diabetic patients already on human insulin (rDNA) were enrolled to a 12 week of treatment. Patients with HbA1c level between 6-8% were switched to Dongsulin on same dosage. The compliance of the patient regarding dietary pattern, physical activity and insulin dosages were assessed. HbA1c was checked after 12 weeks. Patient known to have either of the noncompliance factors during the study period were grouped as group B (non compliant) while, patients who were compliant were grouped as group A (compliant). Thirty nine patients completed the study. No significant difference was found between the HbA1c of two visits in group A (p = 0.32) while, HbA1c in group B was significantly raised as compared to first visit (p = 0.000). In group B patients missed the doses, changes in their diets and decreased their physical activity significantly. The mean insulin dose and weight of the patient remained the same in both groups. No major episode of hypoglycemia was observed. This study has shown that patients who remained compliant during the study period, switched over to Dongsulin had no significant change in the glycaemic control as measured by HbA1c.