Background: Sickle cell disorders are structural hemoglobinopathies, rendering red blood cells sickle shaped, less deformable and sticky leading to microvascular vaso-occlusion and premature red blood cells destruction which leads to varied clinical manifestations. It leads to lifelong morbidity thus affecting quality of life and contributes to early mortality thereby reducing the key national resources- the healthy workforce. This study was done to evaluate epidemiological and clinical profile of sickle cell disease attending the centre.Methods: This study was cross-sectional, observational study conducted at tertiary care hospital in Gujarat. After taking ethical clearance patients were enrolled as per inclusion and exclusion criteria and epidemiological and clinical profile of sickle cell disease patients was studied.Results: Mean age of sickle cell disease was 22.58 years. It was found in tribal communities of Gujarat like Rathwa, Baria, Tadvi etc. Commonest symptom was musculoskeletal pain (86.84%), followed by jaundice (71.05%), fever, dyspnoea, abdominal pain and chest pain. Most common systemic manifestation was pain crises (60.66%), followed by hemolytic anemia (31.15%), acute chest syndrome (30%), consolidation (11.67%), hepatopathy (10%) and avascular necrosis of hip. (6.56%).Conclusions: Sickle cell disease is seen in younger patients. In Gujarat mainly tribal communities are affected. Major systemic manifestations of sickle cell disease include pain crisis followed by hemolytic crisis, acute chest syndrome, hepatopathy and AVN of hip.
BackgroundSickle cell anemia (SCA) is a hereditary disease with defective hemoglobin (Hb) synthesis causing severe hemolytic anemia, pain crisis, and target organ damage. In SCA, several factors independently or in combination lead to derangement in iron stores. Some centers incorrectly prescribe iron therapy on the presumption that SCA would be associated with iron deficiency, but it is not always the case. This study attempts to evaluate the iron status in SCA patients and records the target organ damage present. MethodologyA single-center cross-sectional study of 180 patients with sickle cell disease was carried out at a tertiarycare center in Western India. Patients >12 years of age were included in the study after confirming SCA using high-performance liquid chromatography (HPLC). The iron status of each patient was identified and patients were labeled as iron sufficient based on the following values: Hb (8.1-12 gm%), serum iron (S. iron) level (50-150 μg/dl), serum ferritin (S. ferritin) (50-200 ng/ml), and total iron binding capacity (TIBC) (251-450 µg/dl). The iron status of patients with different target organ damage was also analyzed. ResultsDemographic data revealed that 21-30 years was the most common age group affected by SCA along with a male preponderance. The most common presenting complaint was joint pain (68.9%), the most common sign was pallor (64.4%), most patients had a history of pain crisis (95.6%), and half of the patients had organomegaly (51.1%). Most of the patients had no complications, however, for those who did, hepatopathy (28.9%) was the most common. ConclusionWhile the majority of patients were iron sufficient, a considerable number had either iron deficiency or iron overload states, which emphasizes the necessity of investigating the iron status before deciding the course of treatment in SCA patients. Although the majority were unaffected, screening for end-organ damage should be carried out in all SCA patients.
Transepidermal elimination (TE) is a well-known phenomenon by which dermal materials are expelled through an active epithelial-dermal connective tissue interaction. It has been associated with many cutaneous disorders and described as a regular or sporadic occurrence in a variety of dermatologic conditions. TE as a means of expulsion by skin, either externally introduced or endogenously generated foreign material, is well recognized but rarely appreciated phenomenon. Hence, here we are presenting a case of TE of suture material from the labial surface of the lower lip in a patient who was previously operated for mucocele a year back and reported with the impression of the recurrent lesion.
A BSTRACT Reports have shown the association of coronavirus disease 2019 (COVID-19) with several neuromuscular disorders. Myasthenia gravis (MG) is an autoimmune disease in which antibodies bind to acetyl choline receptors in the postsynaptic membrane at the neuromuscular junction. The characteristic clinical feature of the disease is weakness of the ocular muscle, bulbar muscle, and extremity muscles; when the weakness is limited to the ocular muscle only, the condition is known as ocular myasthenia gravis. Diagnosis is usually confirmed by the acetylcholine receptor antibodies. Symptoms of MG may be aggravated by various types of infections and medications. Here, we are presenting a rare case of a new and acute onset of ocular MG presented after administration of Covishield vaccine.
Background: Coronavirus disease-2019 (COVID-19), caused by severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2), may be associated with acute onset of smell and taste dysfunction along with other common presenting symptoms such as cough, fever and myalgia. Our study aims to analyze the presence of olfactory and gustatory dysfunctions Olfactory and gustatory dysfunctions (OGDs) in patients with COVID-19 and to assess their onset and recovery. Materials and Methods: The cross-sectional study was conducted in March 2021 retrospectively at Care Multispecialty Hospital, Vadodara. A total 301 patients were admitted, among those 280 qualify according to inclusion criteria and 3 patients denied to participate in the study. All patients presenting with laboratory-confirmed real-time reverse transcriptase polymerase chain reaction test for SARS-CoV-2 were included in the study. All 277 patients were undergone a diagnostic questionnaire through telephonic conversation which include patient main symptoms and self-assessment of loss of smell and taste and their onset and recovery. Results: Two hundred and seventy-seven patients were included in this study. One hundred and fifty-three patients (55%) reported olfactory and gustatory disorders. Loss of taste and smell were more frequently reported in female patients (72.8%) than male patients (48%). Onset of these symptoms concomitant with other typical symptoms of COVID-19 is in 58.2% of cases. Recovery of symptoms in most patients was in 5–10 days and faster in younger patients. Conclusion: Olfactory and gustatory disorders (OGDs) related to COVID-19 are frequently reported and more common in female patients. Rapid recovery was observed in most cases. Altogether OGDs can possibly act pivot screening or diagnostic tool for COVID-19 pandemic.
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