Dramatic Improvement of Chronic Inflammatory Demyelinating Polyneuropathy Through Tenofovir Treatment in a Patient Infected with Hepatitis B Virus
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POEMS is a rare syndrome characterized by the unique constellation of polyneuropathy, organomegaly, endocrinopathy, M-proteins, and skin changes. Correct diagnosis is often delayed in early stages of the syndrome when patients exhibit only isolated polyneuropathy due to the clinical and electrodiagnostic similarities with chronic inflammatory demyelinating polyneuropathy. We describe a case in which early suspicion for POEMS uncovered underlying malignancy, and we review the clinical, electrophysiological, pathological, and laboratory findings characteristic of POEMS. The importance of high clinical suspicion is key in the proper diagnosis and management of this complex syndrome.
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This retrospective analysis was undertaken to determine whether a subset of diabetic patients with demyelinating polyneuropathy were similar to patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Ten patients meeting the clinical criteria for idiopathic CIDP were compared to nine patients with diabetes and demyelinating polyneuropathy. The diabetic patients with demyelinating polyneuropathy displayed clinical, electrophysiologic, and histologic features that were similar to those in CIDP patients. All six patients with diabetes and demyelinating polyneuropathy who were treated with immunomodulatory therapy showed a favorable response. Our study highlights the importance of investigating diabetic patients with polyneuropathy in an attempt to identify patients with demyelinating polyneuropathy, because of the likelihood of benefit in these patients from immunomodulatory treatment.
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Introduction. Polyneuropathies or peripheral neuropathies present a dysfunction or disease of larger number of peripheral nerves or their dysfunction. Considering their morbidity - mortality characteristics they present an important aspect in daily clinical practice. One particular polyneuropathy that deserves special review is chronic inflammatory demyelinating polyneuropathy, which, due to its clinical - laboratory presentation, does not include the group of ?simple? neuropathies, thus requiring further examinations. Neurophysiological testing should be performed using the protocol for neuropathy examinations. Neurophysiological examination, during the electroneurographic examination, shows neurographic parameters referring to polyneuropatic demyelinating type of lesion, while the electromyographic finding records the presence of neuropathic lesions (denervation activity, great action potentials with a reduced sample). Case report. A 54-year-old patient was diagnosed to have a ?complicated? demyelinating polyneuropathy according to the clinical-laboratory findings and electromyographic examination. Exclusion criteria, targeted diagnostic examinations, considering the mentioned peripheral neuropathies, pointed to acute inflammatory demyelinating polyneuropathy. However, the chronic inflammatory demyelinating polyneuropathy was finally differentiated during the clinical and electromyographic monitoring. Conclusion. The presented case is interesting because it shows how one can ?wander? towards the diagnosis, which is eventually made on the basis of electromyographic examination and monitoring as well as according to exclusion criteria, which have differentiated the acute inflammatory demyelinating polyneuropathy from the chronic one.
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Objectives: Infection with Hepatitis B Virus (HBV) is an important cause of chronic liver disease in children.Perinatal transmission accounts for the majority of infections.We examined the effects of Tenofovir Disoproxil Fumarate (TDF) on pediatric patients with perinatally acquired Chronic Hepatitis B (CHB).Methods: We retrospectively analyzed the data on pediatric patients with perinatally acquired CHB treated with TDF over a 72-week period.Results: 55 cases were analyzed of which 26 were treated.Fourteen (54%) had immune active hepatitis and 12(46%) were in the immune tolerant phase.In both groups, no difference in inflammation or fibrosis was found on baseline liver biopsy.Mean HBV DNA level at baseline was 9 log 10 copies/mL.Levels declined to 5.9 log 10 copies/mL at 40 weeks of therapy and were undetectable in 19/26(73%) of the patients by week 72.Alanine aminotransferase (ALT) levels normalized by 32 weeks in the immune active hepatitis group.No breakthrough elevations were seen in either group.Overall, 11(42%) and 9(35%) of the patients had Hepatitis B e antigen (HBeAg) clearance and Hepatitis B e antibody (anti-HBe) seroconversion respectively by 72 weeks of treatment.Conclusion: TDF is an effective therapy in pediatric patients with perinatally acquired CHB in both immune active hepatitis and immune tolerant phase patients.Response to treatment did not seem to be affected by baseline ALT levels and liver histopathology findings.
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POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin changes) syndrome may be mistaken for chronic inflammatory demyelinating polyneuropathy (CIDP). Differentiating the 2 entities is crucial, as there are major treatment implications.We compared platelet counts in 136 POEMS patients and 67 CIDP controls.Of the patients with POEMS, 53.7% had thrombocytosis, compared with 1.5% of those with CIDP (P < 0.0001). The median platelet count in patients with POEMS was 467,000/μl compared with 275,000/μl in those with CIDP (P < 0.0001).Thrombocytosis is a helpful indicator to prompt clinicians to consider the diagnosis of POEMS syndrome in patients who are thought to have CIDP, and is an important reminder of the increased risk of thrombotic events in POEMS syndrome.
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Polyneuropathies are a heterogeneous group of diseases that can be caused both by a primary lesion of peripheral nerves, and secondarily, against the background of various somatic diseases. The most common cause of chronic polyneuropathy is distal symmetrical diabetic polyneuropathy. In clinical practice, it is important to be aware of dysimmune polyneuropathy, such as Guillain Barré syndrome, chronic inflammatory demyelinating polyneuropathy, and paraproteinemic polyneuropathy, which lead to severe motor impairment and disability in patients. Identification of the cause of polyneuropathy requires real art, which includes knowledge of the clinical, electrophysiological picture and variants of the course of the disease, as well as a wide range of conditions leading to their development. Timely diagnosis of polyneuropathies and early assignment of etiological and pathogenetic therapy reduce the risk of developing irreversible changes in peripheral nerves caused by axonal degeneration. In the treatment of polyneuropathy of various origins, a special place is occupied by B vitamins, which have a neurotropic effect. Cyanocobalamin is a pathogenetic therapy in patients with diabetes who take metformin for a long time and who developed polyneuropathy due to vitamin B 12 deficiency. In one patient, a combination of several variants of polyneuropathies is possible. The article presents a clinical case of a patient with type 1 diabetes mellitus (DM) who developed dysimmune chronic inflammatory demyelinating polyneuropathy (CIDP) associated with DM on the background of distal symmetrical painless diabetic polyneuropathy after a COVID- 19 infection. A feature of the development of CIDP was the acute onset of the disease. Variants of the clinical picture, ENMG criteria, as well as features of treatment, the effectiveness of therapy and the prognosis of CIDP in patients with DM are discussed.
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