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Hoon Suk Park - One of the best experts on this subject based on the ideXlab platform.
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plasma exchange successfully treats central pontine myelinolysis after acute hypernatremia from intravenous sodium bicarbonate therapy
BMC Nephrology, 2014Co-Authors: Kyung Yoon Chang, Hoon Suk ParkAbstract:Osmotic demyelination syndrome (ODS) primarily occurs after rapid correction of severe hyponatremia. There are no proven effective therapies for ODS, but we describe the first case showing the successful treatment of central pontine myelinolysis (CPM) by plasma exchange, which occurred after rapid development of hypernatremia from intravenous sodium bicarbonate therapy. A 40-year-old woman presented with general weakness, hypokalemia, and metabolic acidosis. The patient was treated with oral and intravenous potassium chloride, along with intravenous sodium bicarbonate. Although her bicarbonate deficit was 365 mEq, we treated her with an overdose of intravenous sodium bicarbonate, 480 mEq for 24 hours, due to the severity of her acidemia and her altered mental status. The next day, she developed hypernatremia with serum sodium levels rising from 142.8 mEq/L to 172.8 mEq/L. Six days after developing hypernatremia, she exhibited tetraparesis, drooling, difficulty swallowing, and dysarthria, and a brain MRI revealed high signal intensity in the central pons with sparing of the peripheral portion, suggesting CPM. We diagnosed her with CPM associated with the rapid development of hypernatremia after intravenous sodium bicarbonate therapy and treated her with plasma exchange. After two consecutive plasma exchange sessions, her neurologic symptoms were markedly improved except for mild diplopia. After the plasma exchange sessions, we examined the patient to determine the reason for her symptoms upon presentation to the hospital. She had normal anion gap metabolic acidosis, low blood bicarbonate levels, a urine pH of 6.5, and a calyceal stone in her left kidney. We performed a sodium bicarbonate loading test and diagnosed distal renal tubular acidosis (RTA). We also found that she had Sjogren’s syndrome after a positive screen for anti-Lo, anti-Ra, and after the results of Schirmer’s test and a lower Lip Biopsy. She was discharged and treated as an outpatient with oral sodium bicarbonate and potassium chloride. This case indicates that serum sodium concentrations should be carefully monitored in patients with distal RTA receiving intravenous sodium bicarbonate therapy. We should keep in mind that acute hypernatremia and CPM can be associated with intravenous sodium bicarbonate therapy, and that CPM due to acute hypernatremia may be effectively treated with plasma exchange.
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plasma exchange successfully treats central pontine myelinolysis after acute hypernatremia from intravenous sodium bicarbonate therapy
BMC Nephrology, 2014Co-Authors: Kyung Yoon Chang, Hoon Suk ParkAbstract:Osmotic demyelination syndrome (ODS) primarily occurs after rapid correction of severe hyponatremia. There are no proven effective therapies for ODS, but we describe the first case showing the successful treatment of central pontine myelinolysis (CPM) by plasma exchange, which occurred after rapid development of hypernatremia from intravenous sodium bicarbonate therapy. A 40-year-old woman presented with general weakness, hypokalemia, and metabolic acidosis. The patient was treated with oral and intravenous potassium chloride, along with intravenous sodium bicarbonate. Although her bicarbonate deficit was 365 mEq, we treated her with an overdose of intravenous sodium bicarbonate, 480 mEq for 24 hours, due to the severity of her acidemia and her altered mental status. The next day, she developed hypernatremia with serum sodium levels rising from 142.8 mEq/L to 172.8 mEq/L. Six days after developing hypernatremia, she exhibited tetraparesis, drooling, difficulty swallowing, and dysarthria, and a brain MRI revealed high signal intensity in the central pons with sparing of the peripheral portion, suggesting CPM. We diagnosed her with CPM associated with the rapid development of hypernatremia after intravenous sodium bicarbonate therapy and treated her with plasma exchange. After two consecutive plasma exchange sessions, her neurologic symptoms were markedly improved except for mild diplopia. After the plasma exchange sessions, we examined the patient to determine the reason for her symptoms upon presentation to the hospital. She had normal anion gap metabolic acidosis, low blood bicarbonate levels, a urine pH of 6.5, and a calyceal stone in her left kidney. We performed a sodium bicarbonate loading test and diagnosed distal renal tubular acidosis (RTA). We also found that she had Sjogren’s syndrome after a positive screen for anti-Lo, anti-Ra, and after the results of Schirmer’s test and a lower Lip Biopsy. She was discharged and treated as an outpatient with oral sodium bicarbonate and potassium chloride. This case indicates that serum sodium concentrations should be carefully monitored in patients with distal RTA receiving intravenous sodium bicarbonate therapy. We should keep in mind that acute hypernatremia and CPM can be associated with intravenous sodium bicarbonate therapy, and that CPM due to acute hypernatremia may be effectively treated with plasma exchange.
Elizabeth A Blair - One of the best experts on this subject based on the ideXlab platform.
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The Laryngoscope VC 2009 The American Laryngological, Rhinological and Otological Society, Inc. The Minor Salivary Gland Biopsy as a Diagnostic Tool for Sjogren Syndrome
2015Co-Authors: Ravinder Bamba, Ba Nadera, J. Sweiss, Md Alex, Er J. Langerman, Md Jerome, B. Taxy, Elizabeth A BlairAbstract:Objectives/Hypothesis: In suspected cases of Sjogren syndome (SS), patients are often referred for a labial minor salivary gland Biopsy. However, studies have shown this test to be unreliable. Pathologic mis-interpretation and immunosuppressive medications may affect the results of the Biopsy. As a result, it is best to perform this procedure only when necessary. The purpose of the current study was to review clini-cal signs and symptoms of patients who underwent a Lip Biopsy to determine which patients benefited most from this procedure. Study Design: Retrospective review. Methods: A retrospective chart review of patients referred to otolaryngology for a Lip Biopsy for the diagnosis of SS. Results: Joint pain, salivary gland swelling, and abnormal serology (anti-Sjogren syndrome A/anti-Sjogren syndrome B) were more prevalent in the posi-tive Lip Biopsy group (grade 3 or 4). Out of the 12 patients who had both sicca symptoms and positive serology, nine (75%) had a grade 4. Presence of sicca symptoms and positive serology were predictive of a positive Biopsy (P .017). Excluding those patients who were on immunosuppression for more than 6 weeks prior to the Biopsy, the correlation became stronger (P .011). Conclusions: In this study, clinical presentation of sicca symptoms and positive serology reliably pre-dicted the results of a Lip Biopsy. The results of this study suggest that patients with clear criterion for SS may not require a Lip Biopsy, especially those patients on immunosuppression. When physicians suspect SS, a thorough clinical and laboratory examination is nec-essary to determine if a patient will benefit from a minor salivary gland Biopsy
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the minor salivary gland Biopsy as a diagnostic tool for sjogren syndrome
Laryngoscope, 2009Co-Authors: Ravinder Bamba, Nadera J Sweiss, Alexander Langerman, Jerome B Taxy, Elizabeth A BlairAbstract:Objectives/Hypothesis: In suspected cases of Sjogren syndome (SS), patients are often referred for a labial minor salivary gland Biopsy. However, studies have shown this test to be unreliable. Pathologic misinterpretation and immunosuppressive medications may affect the results of the Biopsy. As a result, it is best to perform this procedure only when necessary. The purpose of the current study was to review clinical signs and symptoms of patients who underwent a Lip Biopsy to determine which patients benefited most from this procedure. Study Design: Retrospective review. Methods: A retrospective chart review of patients referred to otolaryngology for a Lip Biopsy for the diagnosis of SS. Results: Joint pain, salivary gland swelling, and abnormal serology (anti-Sjogren syndrome A/anti-Sjogren syndrome B) were more prevalent in the positive Lip Biopsy group (grade = 3 or 4). Out of the 12 patients who had both sicca symptoms and positive serology, nine (75%) had a grade = 4. Presence of sicca symptoms and positive serology were predictive of a positive Biopsy (P = .017). Excluding those patients who were on immunosuppression for more than 6 weeks prior to the Biopsy, the correlation became stronger (P = .011). Conclusions: In this study, clinical presentation of sicca symptoms and positive serology reliably predicted the results of a Lip Biopsy. The results of this study suggest that patients with clear criterion for SS may not require a Lip Biopsy, especially those patients on immunosuppression. When physicians suspect SS, a thorough clinical and laboratory examination is necessary to determine if a patient will benefit from a minor salivary gland Biopsy.
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labial minor salivary gland Biopsy
Operative Techniques in Otolaryngology-head and Neck Surgery, 2008Co-Authors: Alexander Langerman, Elizabeth A BlairAbstract:Biopsy of the labial minor salivary glands (“Lip Biopsy”) is often used as part of the diagnostic workup for suspected Sjogren's syndrome, and is comparable in diagnostic accuracy to open parotid Biopsy. While there are non-invasive tests that should be performed for Sjogren's workup, in challenging cases tissue diagnosis provides valuable information. When properly performed, this office procedure can be done with minimal discomfort for the patient. The indications, risks, and interpretation of Lip Biopsy are discussed along with a detailed description of a technique designed to ensure sufficient tissue for diagnosis.
Kyung Yoon Chang - One of the best experts on this subject based on the ideXlab platform.
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plasma exchange successfully treats central pontine myelinolysis after acute hypernatremia from intravenous sodium bicarbonate therapy
BMC Nephrology, 2014Co-Authors: Kyung Yoon Chang, Hoon Suk ParkAbstract:Osmotic demyelination syndrome (ODS) primarily occurs after rapid correction of severe hyponatremia. There are no proven effective therapies for ODS, but we describe the first case showing the successful treatment of central pontine myelinolysis (CPM) by plasma exchange, which occurred after rapid development of hypernatremia from intravenous sodium bicarbonate therapy. A 40-year-old woman presented with general weakness, hypokalemia, and metabolic acidosis. The patient was treated with oral and intravenous potassium chloride, along with intravenous sodium bicarbonate. Although her bicarbonate deficit was 365 mEq, we treated her with an overdose of intravenous sodium bicarbonate, 480 mEq for 24 hours, due to the severity of her acidemia and her altered mental status. The next day, she developed hypernatremia with serum sodium levels rising from 142.8 mEq/L to 172.8 mEq/L. Six days after developing hypernatremia, she exhibited tetraparesis, drooling, difficulty swallowing, and dysarthria, and a brain MRI revealed high signal intensity in the central pons with sparing of the peripheral portion, suggesting CPM. We diagnosed her with CPM associated with the rapid development of hypernatremia after intravenous sodium bicarbonate therapy and treated her with plasma exchange. After two consecutive plasma exchange sessions, her neurologic symptoms were markedly improved except for mild diplopia. After the plasma exchange sessions, we examined the patient to determine the reason for her symptoms upon presentation to the hospital. She had normal anion gap metabolic acidosis, low blood bicarbonate levels, a urine pH of 6.5, and a calyceal stone in her left kidney. We performed a sodium bicarbonate loading test and diagnosed distal renal tubular acidosis (RTA). We also found that she had Sjogren’s syndrome after a positive screen for anti-Lo, anti-Ra, and after the results of Schirmer’s test and a lower Lip Biopsy. She was discharged and treated as an outpatient with oral sodium bicarbonate and potassium chloride. This case indicates that serum sodium concentrations should be carefully monitored in patients with distal RTA receiving intravenous sodium bicarbonate therapy. We should keep in mind that acute hypernatremia and CPM can be associated with intravenous sodium bicarbonate therapy, and that CPM due to acute hypernatremia may be effectively treated with plasma exchange.
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plasma exchange successfully treats central pontine myelinolysis after acute hypernatremia from intravenous sodium bicarbonate therapy
BMC Nephrology, 2014Co-Authors: Kyung Yoon Chang, Hoon Suk ParkAbstract:Osmotic demyelination syndrome (ODS) primarily occurs after rapid correction of severe hyponatremia. There are no proven effective therapies for ODS, but we describe the first case showing the successful treatment of central pontine myelinolysis (CPM) by plasma exchange, which occurred after rapid development of hypernatremia from intravenous sodium bicarbonate therapy. A 40-year-old woman presented with general weakness, hypokalemia, and metabolic acidosis. The patient was treated with oral and intravenous potassium chloride, along with intravenous sodium bicarbonate. Although her bicarbonate deficit was 365 mEq, we treated her with an overdose of intravenous sodium bicarbonate, 480 mEq for 24 hours, due to the severity of her acidemia and her altered mental status. The next day, she developed hypernatremia with serum sodium levels rising from 142.8 mEq/L to 172.8 mEq/L. Six days after developing hypernatremia, she exhibited tetraparesis, drooling, difficulty swallowing, and dysarthria, and a brain MRI revealed high signal intensity in the central pons with sparing of the peripheral portion, suggesting CPM. We diagnosed her with CPM associated with the rapid development of hypernatremia after intravenous sodium bicarbonate therapy and treated her with plasma exchange. After two consecutive plasma exchange sessions, her neurologic symptoms were markedly improved except for mild diplopia. After the plasma exchange sessions, we examined the patient to determine the reason for her symptoms upon presentation to the hospital. She had normal anion gap metabolic acidosis, low blood bicarbonate levels, a urine pH of 6.5, and a calyceal stone in her left kidney. We performed a sodium bicarbonate loading test and diagnosed distal renal tubular acidosis (RTA). We also found that she had Sjogren’s syndrome after a positive screen for anti-Lo, anti-Ra, and after the results of Schirmer’s test and a lower Lip Biopsy. She was discharged and treated as an outpatient with oral sodium bicarbonate and potassium chloride. This case indicates that serum sodium concentrations should be carefully monitored in patients with distal RTA receiving intravenous sodium bicarbonate therapy. We should keep in mind that acute hypernatremia and CPM can be associated with intravenous sodium bicarbonate therapy, and that CPM due to acute hypernatremia may be effectively treated with plasma exchange.
Fahad Ajmal - One of the best experts on this subject based on the ideXlab platform.
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primary sjogren s syndrome presenting as acute interstitial pneumonitis hamman rich syndrome
Case Reports in Medicine, 2016Co-Authors: Abidullah Khan, Mohammad Humayun, Iqbal Haider, Maimoona Ayub, Zakir Shah, Fahad AjmalAbstract:A previously well, 45-year-old Pakistani lady was admitted to the medical unit on-call of Khyber Teaching Hospital (KTH) Peshawar with a 5-day history of fever, productive cough with copious mucoid sputum, dyspnea, and pleuritic chest pain. She also complained of dry eyes, mouth, and vagina. Her chest X-ray showed diffuse alveolar shadowing and arterial gas analysis confirmed type 1 respiratory failure. Over the next few days, she deteriorated rapidly making an urgent transfer to the medical intensive care unit (MICU) necessary, where she was mechanically ventilated. An HRCT followed by bronchoscopic biopsies made a diagnosis of acute interstitial pneumonitis (AIP), formerly known as Hamman-Rich syndrome. She also turned out to be positive for both anti-SS-A/Ro and anti-SS-B/La antibodies along with a positive Schirmer's test and lower Lip Biopsy. She received intravenous steroids and supportive care. The patient had a complete recovery after approximately three weeks' stay in the hospital with lung function returning back to normal. This is most probably the first ever case of primary Sjogren syndrome (pSjS) presenting as AIP, recovering completely in less than a month time.
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Primary Sjogren’s Syndrome Presenting as Acute Interstitial Pneumonitis/Hamman-Rich Syndrome
Hindawi Limited, 2016Co-Authors: Abidullah Khan, Mohammad Humayun, Iqbal Haider, Maimoona Ayub, Zakir Shah, Fahad AjmalAbstract:A previously well, 45-year-old Pakistani lady was admitted to the medical unit on-call of Khyber Teaching Hospital (KTH) Peshawar with a 5-day history of fever, productive cough with copious mucoid sputum, dyspnea, and pleuritic chest pain. She also complained of dry eyes, mouth, and vagina. Her chest X-ray showed diffuse alveolar shadowing and arterial gas analysis confirmed type 1 respiratory failure. Over the next few days, she deteriorated rapidly making an urgent transfer to the medical intensive care unit (MICU) necessary, where she was mechanically ventilated. An HRCT followed by bronchoscopic biopsies made a diagnosis of acute interstitial pneumonitis (AIP), formerly known as Hamman-Rich syndrome. She also turned out to be positive for both anti-SS-A/Ro and anti-SS-B/La antibodies along with a positive Schirmer’s test and lower Lip Biopsy. She received intravenous steroids and supportive care. The patient had a complete recovery after approximately three weeks’ stay in the hospital with lung function returning back to normal. This is most probably the first ever case of primary Sjogren syndrome (pSjS) presenting as AIP, recovering completely in less than a month time
Stefano Sivolella - One of the best experts on this subject based on the ideXlab platform.
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Melkersson–Rosenthal syndrome: a case report of a rare disease with overlapping features
BMC, 2019Co-Authors: Mauro Cancian, Stefano Giovannini, Annalisa Angelini, Marny Fedrigo, Raffaele Bendo, Riccardo Senter, Stefano SivolellaAbstract:Abstract Background Melkersson–Rosenthal syndrome (MRS) is a rare, neuro-mucocutaneous disease which presents as orofacial swelling, facial palsy and fissured tongue. These symptoms may occur simultaneously or, more frequently, with a oligosymptomatic or monosymptomatic pattern. Swelling, that is the most common initial finding, may mimic hereditary or acquired angioedema, a disorder caused by histamine or bradykinin-mediated plasma-leakage affecting subcutaneous and/or submucosal tissue. The differential diagnosis of MRS includes also chronic inflammatory and infective diseases characterized by granulomatous infiltration, as well as rosacea, contact dermatitis, allergic reactions and Bell’s palsy. Case presentation A 71-year old, non-allergic female patient with no familial and personal history of angioedema presented, a few days after a possible herpes simplex or varicella-zoster virus infection, with monolateral facial paraesthesia and lower Lip edema. After temporary remission of symptoms on oral steroids and antihistamines, she showed swelling recurrence refractory to valaciclovir therapy and a subsequent course of antihistamines. The clinical picture and a previous history of non-Hodgkin lymphoma prompted us to rule out an acquired form of paraneoplastic, C1-inhibitor (C1-INH) deficiency: C1q and both antigen and functional C1-INH tested normal, whilst we found low plasma levels of C3 and C4 possibly related to the parallel detection of antiphosphoLipid antibodies. Thus, we hypothesized a non-histaminergic, idiopathic form of angioedema and planned further therapy with tranexamic acid and the leukotriene receptor antagonist montelukast. Treatment failure with both drugs finally suggested a Melkersson–Rosenthal syndrome, which was confirmed by histologic findings of non caseating granulomas on Lip Biopsy. Conclusion Melkersson–Rosenthal syndrome may occur with rather non-specific symptoms and overlap with alternative conditions, including recurrent angioedema. No specific biomarkers for MRS exist and clinical diagnosis is often of exclusion. The finding of complement or immune alterations, as in our patient, may be further confounding and justify the need for skin or mucosal Biopsy to establish a correct diagnosis and prescribe targeted therapy
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melkersson rosenthal syndrome a case report of a rare disease with overlapping features
Allergy Asthma & Clinical Immunology, 2019Co-Authors: Mauro Cancian, Stefano Giovannini, Annalisa Angelini, Marny Fedrigo, Raffaele Bendo, Riccardo Senter, Stefano SivolellaAbstract:Melkersson–Rosenthal syndrome (MRS) is a rare, neuro-mucocutaneous disease which presents as orofacial swelling, facial palsy and fissured tongue. These symptoms may occur simultaneously or, more frequently, with a oligosymptomatic or monosymptomatic pattern. Swelling, that is the most common initial finding, may mimic hereditary or acquired angioedema, a disorder caused by histamine or bradykinin-mediated plasma-leakage affecting subcutaneous and/or submucosal tissue. The differential diagnosis of MRS includes also chronic inflammatory and infective diseases characterized by granulomatous infiltration, as well as rosacea, contact dermatitis, allergic reactions and Bell’s palsy. A 71-year old, non-allergic female patient with no familial and personal history of angioedema presented, a few days after a possible herpes simplex or varicella-zoster virus infection, with monolateral facial paraesthesia and lower Lip edema. After temporary remission of symptoms on oral steroids and antihistamines, she showed swelling recurrence refractory to valaciclovir therapy and a subsequent course of antihistamines. The clinical picture and a previous history of non-Hodgkin lymphoma prompted us to rule out an acquired form of paraneoplastic, C1-inhibitor (C1-INH) deficiency: C1q and both antigen and functional C1-INH tested normal, whilst we found low plasma levels of C3 and C4 possibly related to the parallel detection of antiphosphoLipid antibodies. Thus, we hypothesized a non-histaminergic, idiopathic form of angioedema and planned further therapy with tranexamic acid and the leukotriene receptor antagonist montelukast. Treatment failure with both drugs finally suggested a Melkersson–Rosenthal syndrome, which was confirmed by histologic findings of non caseating granulomas on Lip Biopsy. Melkersson–Rosenthal syndrome may occur with rather non-specific symptoms and overlap with alternative conditions, including recurrent angioedema. No specific biomarkers for MRS exist and clinical diagnosis is often of exclusion. The finding of complement or immune alterations, as in our patient, may be further confounding and justify the need for skin or mucosal Biopsy to establish a correct diagnosis and prescribe targeted therapy.