Laurence-Moon-Bardet-Biedl syndrome (LMBBS), a rare autosomal recessive defect, mostly occurs in children born from consanguineous marriages. The major features of this syndrome are cone-rod dystrophy, polydactyly, obesity, learning disabilities, hypogonadism in males, renal anomalies, nystagmus, speech disorders, developmental delay, polyuria/polydipsia, ataxia, and poor coordination/clumsiness. In this report, we present a case of a 19-year-old man with pain and swelling of the left ankle and knee joints because of which he could not walk, with an onset of loose stools since a week. He presented with multiple non-itchy hyperpigmented macules on his face and back, polydactyly in his left foot, central obesity, proteinuria, macrocytic anemia, low intelligence quotient, reduced power in the left lower limb, reduced plantar reflexes, nystagmus, pigmented black lesions in the temporal retina on fundoscopy, a micropenis, absent pubic and axillary hair, and a small scrotum containing testes. The patient was diagnosed with LMBBS.
Meningitis caused by Salmonella enteritidis is an uncommon infection, linked with great mortality and neurological problems, which makes prompt diagnosis and treatment very crucial. Patients of bacterial meningitis present with common symptoms such as headache, fever, altered level of consciousness, and neck rigidity. A positive gram stain or culture of cerebrospinal fluid (CSF) leads to its diagnosis. In this report, we present a case of a 16-year-old male with extensively drug-resistant (XDR) Salmonella typhi meningitis, which was not responsive to initial medical intervention. He was treated with meropenem, imipenem, azithromycin, and metronidazole. Immediate tracheostomy and intubation were performed in the surgical intensive care unit (ICU), as the patient had developed stridor, shortness of breath, tachypnea, tachycardia, and had severely decreased O2 saturation of 60%. As far as treatment is concerned, third-generation cephalosporins are considered the treatment of choice. In addition, the use of fluoroquinolones and carbapenems, mainly meropenem, has also been described as a therapeutic alternative.
Autoimmune hepatitis (AIH) is chronic inflammation of hepatocytes due to immune cells attacking the patient's own hepatocytes, histologically characterized by interface hepatitis. The disease can be serious, and if left untreated, it can lead to cirrhosis of the liver and eventual liver failure. It occurs more frequently in females. The standard treatment for AIH includes corticosteroids. There are two main treatment regimens, which include either prednisolone alone or prednisone and azathioprine. Although, liver transplantation is certainly the treatment of choice, it has not yet been established on a large scale worldwide. We present here the case of a 22-year-old male, with autoimmune hepatitis and unspecified vasculitis.
Objective: To compare 7-Day All-Cause Mortality among HDU Patients with Modified Early Warning Score of ≥5 with Those with Score of <5. Methods: All patients of age more than 18 years, of either gender admitted in HDU of Medical Unit-II, CHK between September 2019 to February 2020 were included. MEWS was calculated for each patient at time of admission. Patients with MEWS score of ≥5 were allocated to Group-A and those with score of <5 were allocated to Group-B. Patients were followed for seven days and outcome status of alive, expired or discharged was noted. Results: Total of 336 patients were selected out of which 168 patients was inducted in Group-A and 168 patients in Group-B. MEWS Score in patients who expired was significantly higher (Mdn=11) than in those who survived (Mdn=4), p <.001. 7-day mortality in Group-A was 62 (39.9%) while in Group-B was 40 (23.8%). ROC was plotted of MEWS Score for mortality, it showed significant area under curve of 68.4% (p <0.001, 95% CI = 0.62 to 0.75). MEWS Score of 3.5 showed sensitivity of 89.2% and specificity of 65%. Conclusion: Our results show that MEWS has a positive trend to predict mortality. MEWS score of 3.5 is suggested cut off based on ROC in our study. doi: https://doi.org/10.12669/pjms.37.2.2832 How to cite this:Shaikh MA, Punshi A, Talreja ML, Rasheed T, Bader N, Zuberi BF. Comparison of within 7 Day All-Cause Mortality among HDU Patients with Modified Early Warning Score of ≥5 with those with Score of <5. Pak J Med Sci. 2021;37(2):---------. doi: https://doi.org/10.12669/pjms.37.2.2832 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Rheumatic diseases can have serious socioeconomic implications in a developing country like Pakistan. With dwindling resources in the healthcare sector, decision makers are forced to prioritize treatment between patients. Objective: To assess the socio-economic burden of major rheumatic diseases in a tertiary care hospital. Methods:171 patients were enrolled in the study prospectively. Patients were selected through specially designed questionnaires. Diagnosis of the disease and patient’s socioeconomic status were recorded and analyzed using SPSS version 25.0. Results: The mean age of the participants was 38.65±13.20 years. A higher ratio of female patients (78.4 %) as compared to the male patients (21.6 %) was seen. The majority of the patients were not well educated as 79 % of the patients were below matric, 9 patients were graduated and only 2 patients were postgraduates. Most of patient fall in low socioeconomic background (30,924.8 ± 19,107.3). Out of all the rheumatic diseases, Rheumatoid Arthritis 96 (56.1 %)was the most commonly found autoimmune disease. Conclusion: Rheumatic disease outcomes in Pakistan are influenced significantly by socioeconomic status. A comprehensive treatment plan for rheumatic disease is needed, especially for those with low education levels and poor quality of life.
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