China
Africa
Explore chapters and articles related to this topic
Paper 2
Published in Aalia Khan, Ramsey Jabbour, Almas Rehman, nMRCGP Applied Knowledge Test Study Guide, 2021
Aalia Khan, Ramsey Jabbour, Almas Rehman
It is essential to rule out biliary atresia if jaundice is present at 14 days and the child is not thriving. It tends to be more common in boys. It is found in 1 in 15 000 live births. Hepatosplenomegaly may be present. Investigations may include CT or nuclear medicine scanning and treatment is surgical.
Hurler disease/mucopolysaccharidosis type IH (MPSIH)/α-L-iduronidase deficiency
Published in William L. Nyhan, Georg F. Hoffmann, Aida I. Al-Aqeel, Bruce A. Barshop, Atlas of Inherited Metabolic Diseases, 2020
Enzyme replacement with human recombinant α-L-iduronidase has been reported [52, 53] in 45 patients with MPS I. Patients were selected with Hurler-Scheie or Scheie phenotypes and given enzyme intravenously weekly for as long as 62 weeks. Hepatosplenomegaly decreased significantly in all patients. Liver size was normal in eight patients by 26 weeks. Growth in height and weight increased in prepubertal patients. Improvements were also notable in the urinary excretion of glycosaminoglycans, as well as in joint mobility, respiratory function (forced vital capacity) [52, 53], and ambulation (6-minute walk test). The enzyme has now been approved by the US Food and Drug Administration (FDA) (in 2003) and is marketed as Aldurazyme (BioMarin Genzyme). Corneal clouding does not change; valvular disease seems to be unaltered. There is little likelihood that this approach would affect the brain; trials are underway to treat with intrathecal enzyme [54]. Enzyme replacement therapy has been used for a sufficient number of years in the treatment of Hurler disease to establish that it is efficacious in reducing biochemical characteristics of the disease such as urinary glycosaminoglycan excretion and improved function capacity, as well as liver size [55].
The digestive system
Published in Laurie K. McCorry, Martin M. Zdanowicz, Cynthia Y. Gonnella, Essentials of Human Physiology and Pathophysiology for Pharmacy and Allied Health, 2019
Laurie K. McCorry, Martin M. Zdanowicz, Cynthia Y. Gonnella
Hepatosplenomegaly—swelling of spleen and liver. Swelling of the liver can increase portal pressure, whereas swelling of the spleen can lead to increased destruction of red blood cells (anemia) white blood cells (leukopenia) and platelets (thrombocytopenia).
Lymphoma-associated hemophagocytic syndrome: a retrospective study from a single center
Published in Hematology, 2022
Na Li, Ming Jiang, Wan-chun Wu, Hui-jie Zhou, Li-qun Zou
In the present study, patients had a variety of clinical manifestations. All patients presented with cytopenia, fever, and high ferritin levels. Hepatosplenomegaly was observed in 72.6% of cases. BM involvement and hemophagocytosis were present in more than half of the cases. The clinical characteristics of patients with B-LAHS and T/NK-LAHS were compared in this study. T/NK-LAHS individuals were younger than those with B-LAHS, which was consistent with previous reports [10, 19]. Most of the 117 patients (70.1%) developed LAHS at the time of lymphoma diagnosis. Subgroup analysis found that B-cell lymphoma patients were more likely to develop HPS at lymphoma onset than T/NK-cell lymphoma patients. Laboratory findings, including cytopenia, usually beginning with thrombocytopenia evolving into severe pancytopenia, hypofibrinogenemia, elevated LDH concentration, and liver dysfunction, were more frequent in T/NK-LAHS than in B-LAHS.
Isolated hepatosplenic abscess from cat scratch disease in a patient with HIV
Published in Baylor University Medical Center Proceedings, 2021
Bhanusowmya Buragamadagu, Chen Song, Shambo GuhaRoy, Gul Madison
A 48-year-old man came to the hospital for evaluation of fever, chills, weakness, dyspnea, dysuria, and chest and abdominal pain. The symptoms started after initiation of HIV treatment 1 week earlier. The HIV infection had been diagnosed 13 years earlier. He reported untreated hepatitis C infection and a history of intravenous drug use. His temperature was 100.9°F. There was abdominal tenderness and track marks on his left arm. The leukocyte count was 4700/U; aspartate transferase, 146 U/L; alanine aminotransferase, 97 U/L; alkaline phosphatase, 104 U/L; and erythrocyte sedimentation rate, 68 mm/h. Computed tomography angiography of the chest revealed splenomegaly (up to 17.5 cm). He was started on broad-spectrum antibiotics with intravenous vancomycin and piperacillin-tazobactam. His CD4 count was 504 (36%), and blood cultures were negative for >48 hours. The echocardiogram was normal. Magnetic resonance imaging (MRI) of the abdomen revealed hepatosplenomegaly with multiple liver microabscesses measuring up to 5 mm (Figures 1a, 1b). Antibiotics were deescalated to ceftriaxone. Ultrasound-guided biopsy of the liver abscess revealed chronic hepatitis and steatosis. Cultures of the abscess grew Enterococcus faecalis, and antibiotics were changed to amoxicillin-clavulanic acid.
Maryland ACP Winners from Mulholland Mohler Residents meeting 2021
Published in Journal of Community Hospital Internal Medicine Perspectives, 2021
A 24-year-old man with no known past medical history developed headache and fever that progressed over twelve days to abdominal pain, diarrhea, jaundice, oliguria, and dyspnea. His initial symptoms began four days after exposure to an individual with possible SARS-CoV-2. On presentation, examination was significant for temperature of 39.9°C, O2 saturation of 93% on six liters of O2 via nasal cannula, scleral icterus, and hepatomegaly. Laboratory evaluation was notable for white blood cell count of 12.2/mm3, hemoglobin of 13.7 g/dL, platelet count of 55/mm3, aspartate aminotransferase of 3093 IU/L, alanine aminotransferase of 865 IU/L, INR of 2.6, creatinine of 5.32 mg/dL, ferritin of >50,000 ng/mL, triglyceride level of 1152 mg/dL, and fibrinogen level of 62 mg/dL. A computed tomography (CT) scan of the abdomen revealed hepatosplenomegaly. A bone marrow biopsy showed hemophagocytes. The patient’s HScore was calculated to be 285 points, indicating >99% chance of having HLH.