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HYPERNATREMIA FROM CENTRAL DIABETES INSIPIDUS IN ACUTE MYELOGENOUS LEUKEMIA
NKF 2015 Spring Clinical Meetings Abstracts
285 HYPERNATREMIA FROM CENTRAL DIABETES INSIPIDUS IN ACUTE MYELOGENOUS LEUKEMIA Marvin Vaishnani, Tarake Aljarod, Kamal Nayyar, Amr Mohamed, Iwayemi Olayeye, Chamberlain Obialo, Khalid Bashir, Morehouse School of Medicine, Department of Medicine, Atlanta, Georgia, USA. Central Diabetes Insipidus (CDI) is a rare finding in acute myelogenous leukemia (AML) patients. Different mechanisms have been found to be playing a role in development of CDI in AML. The direct structural infiltration of the hypothalamic-pituitary region by tumor cells affects the secretion of antidiuretic hormone (ADH). In addition, studies have shown some cytogenetic anomalies of chromosome 3 and 7 may be playing a pivotal role in the pathogenesis of CDI in AML. A 59-year-old male with medical history significant for AML was admitted to hospital for evaluation of hypernatremia. Serum Na of 156 mEq/L was found on routine lab work in Oncology clinic. Patient reported having significant polyuria despite minimal increase in fluid intake. Physical examination revealed dry oral mucosa and skin. Laboratory data revealed urine osmolality of 200 mOsm/Kg and serum osmolality 313 mOsm/L. Diagnosis of Central DI was made based on the increase in urine osmolality of more than 50% after receiving desmopressin. MRI of brain shows mild abnormal enhancement in pituitary infundibulum to hypothalamic recess. Cytogenetic analysis of patient showed inversion of long arm of chromosome 3 (inv 3q21q26.2), deletion of long arm of chromosome 7 (del 7q) and monosomy 7. Patient was started on daily nasal desmopressin with resolution of polyuria and hypernatremia. Patient was continued on chemotherapy with poor response and eventually died after five months. CDI in the setting of AML, a rare disorder, is usually associated with the structural involvement of hypothalamic-pituitary region but cytogenetic anomalies may be an additional contributory factor. The cause of CDI due to cytogenetic mechanism in AML patients is still unclear. Our case should prompt conducting further studies to get more information about the molecular mechanism that may help in the diagnosis and finding new therapy for AML patients with CDI who tend to have poor prognosis.
286 DEVELOPMENT OF A HEALTH RELATED QUALITY OF LIFE (HRQOL) MEASURE FOR ANEMIA IN END STAGE RENAL DISEASE (ESRD) Magdalena Vanya, Roxana Bahar, ICON, San Francisco CA, USA; Junji Lin, Spiros Tzivelekis, Amgen, Thousand Oaks, CA, USA; Kellee Howard, ICON, San Francisco CA, USA Currently no validated PRO measures assess HRQoL in ESRD anemia patients. The purpose of this study was to develop a contentvalid, sensitive PRO measure for the assessment of HRQoL in ESRD anemia patients. To identify the most common symptoms and symptom impacts of anemia of ESRD, a literature review (Vanya et al, 2014 ISPOR abstracts) and telephone interviews with clinical experts were conducted. Concept elicitation (CE) through focus groups (FGs) with ESRD anemia patients were conducted to discuss their experiences with anemia of ESRD. Through qualitative analysis, a draft PRO was developed. The draft PRO was further evaluated by an expert panel and cognitive debriefing (CD) with ESRD anemia patients. The CD interviews evaluated the clarity and comprehensibility of the instrument’s items, response options, instructions, and recall period. Twenty-one patients participated in 3 focus groups and 10 patients completed CD interviews. Mean age of the total sample was 60 years; 52% were female. The most commonly reported symptoms included pain and tiredness. The most commonly reported symptom impacts included negative impact on work/school/home and recreational activities, family relationships, hobbies, emotions, and sleep. Analysis of the CD data indicated that all 10 patients were able to interpret and understand all the items on the draft ESRD anemia instrument as intended. All items were indicated by at least half the sample as relevant to their experience with anemia of ESRD. No items were removed and minimal changes were suggested. The results of the CE and CD interviews support the content validity of the newly developed ESRD anemia PRO instrument. The instrument was confirmed to be meaningful to patients, interpretable, and appropriate for use in assessing HRQL in the ESRD anemia patient population.
A86
287 A CASE OF CUTANEOUS EXOPHILIA IN A KIDNEY TRANSPLANT RECIPIENT: J Varghese, KD Jhaveri, M Bhaskaran, D Hirschwerk, A Garg, M Sachdeva. Divisions of Nephrology, Infectious Diseases, and Dermatology, Hofstra NS-LIJ School of Medicine, NY During the post transplantation period, kidney transplant recipients are susceptible to many opportunistic infections due to their chronic immunosuppressed state. Fungal skin infections are rare infections but can be seen post transplantation. We describe a case of cutaneous exophiala, a rare form of phaeohyphomycosis, in a kidney transplant recipient. A 53 year old Caucasian male with a cadaveric renal transplant (CRT) five months prior to presentation, was noted to have a skin rash over his upper extremities. His post transplant course was complicated by calcineurin inhibitor induced thrombotic microangiopathy for which he required long course of plasmapheresis. In addition, his immunosuppresion was changed to belatacept, of which he had received five doses. At the time of presentation, he was noted to have cutaneous lesions on his bilateral upper extremities sparing the palms. The rash was described as multiple cyst-like subcutaneous lesions without surrounding inflammation or associated pruritis. A skin punch biopsy of a lesion confirmed phaeohyphomycosis and specifically cutaneous exophiala. He subsequently had an MRI Head, CT Chest and CT Sinus which were negative for disseminated fungal infection. He was started on a prolonged course of posaconazole with excellent improvement and was able to continue his immunosuppression with improving renal function. Phaeohyphomycosis is a rare fungal infection caused by several species the most common being Exophiala jeanselmei and W. dermatitidis. These organisms form pigmented hyphae in tissue and form cutaneous lesions, described as plaques or subcutaneous inflammatory cysts. One non US based case series has described good outcomes of these patients with treatment of anti fungal agents in renal transplant setting. In renal transplant recipients, it is important to consider and diagnose such fatal infections so that appropriate treatment can be initiated in a timely manner.
288 OVARIAN HYPERSTIMULATION SYNDROME CAUSING ATYPICAL RENAL DISEASE Jeny Varghese and Mala Sachdeva, Hofstra North Shore-LIJ School of Medicine, Great Neck, NY, USA. Ovarian Hyperstimulation Syndrome (OHSS) can occur as a result of in vitro fertilization (IVF). OHSS has commonly been associated with acute kidney injury, usually a result of altered hemodynamics. We describe two cases in which ovarian hyperstimulation syndrome caused renal disease. Both of these cases are the first reported cases. A 38 year old Nigerian female underwent a course of in vitro fertilization treatment and was admitted six weeks later with a diagnosis of OHSS. She was anasarcic, and laboratory studies were consistent with marked hyperlipidememia, severe hypoalbuminemia, non-oliguric acute kidney injury, and nephrotic range proteinuria of 33 grams. Kidney biopsy preformed was consistent with collapsing FSGS (C-FSGS). Patient was treated with solumedrol and cyclosporine with clinical improvement in her nephrotic syndrome. A 36 year old Caucasian female with history of polycystic ovarian syndrome presented ten days after IVF with moderate ascites, hypoalbuminemia, and progressive acute renal failure requiring hemodialysis. She was admitted with a diagnosis of OHSS. During hospitalization she sustained sequelae of a hypercoaguable state, including a cerbrovascular accident, myocardial ischemia, and bilateral renal artery thrombosis. Her kidney biopsy showed a tubulo-interstitial nephritis, interstitial edema, and tubules with cytoplasmic vacuolization. She had no subsequent renal recovery and remained dialysis dependent. Both cases occurred following IVF and in association with OHSS. VEGF has now emerged as one of the factors most likely involved in the pathophysiology of OHSS, and perhaps this could explain its association with collapsing FSGS. In the latter case, sequelae of a hypercoaguable state could explain her renal function loss. Given that IVF is a more commonly performed procedure, those involved in the care of these women should be able to recognize kidney disease so that prompt diagnosis and respective treatment can be given in a timely manner.
Am J Kidney Dis. 2015;65(4):A1-A93
285 HYPERNATREMIA FROM CENTRAL DIABETES INSIPIDUS IN ACUTE MYELOGENOUS LEUKEMIA Marvin Vaishnani, Tarake Aljarod, Kamal Nayyar, Amr Mohamed, Iwayemi Olayeye, Chamberlain Obialo, Khalid Bashir, Morehouse School of Medicine, Department of Medicine, Atlanta, Georgia, USA. Central Diabetes Insipidus (CDI) is a rare finding in acute myelogenous leukemia (AML) patients. Different mechanisms have been found to be playing a role in development of CDI in AML. The direct structural infiltration of the hypothalamic-pituitary region by tumor cells affects the secretion of antidiuretic hormone (ADH). In addition, studies have shown some cytogenetic anomalies of chromosome 3 and 7 may be playing a pivotal role in the pathogenesis of CDI in AML. A 59-year-old male with medical history significant for AML was admitted to hospital for evaluation of hypernatremia. Serum Na of 156 mEq/L was found on routine lab work in Oncology clinic. Patient reported having significant polyuria despite minimal increase in fluid intake. Physical examination revealed dry oral mucosa and skin. Laboratory data revealed urine osmolality of 200 mOsm/Kg and serum osmolality 313 mOsm/L. Diagnosis of Central DI was made based on the increase in urine osmolality of more than 50% after receiving desmopressin. MRI of brain shows mild abnormal enhancement in pituitary infundibulum to hypothalamic recess. Cytogenetic analysis of patient showed inversion of long arm of chromosome 3 (inv 3q21q26.2), deletion of long arm of chromosome 7 (del 7q) and monosomy 7. Patient was started on daily nasal desmopressin with resolution of polyuria and hypernatremia. Patient was continued on chemotherapy with poor response and eventually died after five months. CDI in the setting of AML, a rare disorder, is usually associated with the structural involvement of hypothalamic-pituitary region but cytogenetic anomalies may be an additional contributory factor. The cause of CDI due to cytogenetic mechanism in AML patients is still unclear. Our case should prompt conducting further studies to get more information about the molecular mechanism that may help in the diagnosis and finding new therapy for AML patients with CDI who tend to have poor prognosis.
286 DEVELOPMENT OF A HEALTH RELATED QUALITY OF LIFE (HRQOL) MEASURE FOR ANEMIA IN END STAGE RENAL DISEASE (ESRD) Magdalena Vanya, Roxana Bahar, ICON, San Francisco CA, USA; Junji Lin, Spiros Tzivelekis, Amgen, Thousand Oaks, CA, USA; Kellee Howard, ICON, San Francisco CA, USA Currently no validated PRO measures assess HRQoL in ESRD anemia patients. The purpose of this study was to develop a contentvalid, sensitive PRO measure for the assessment of HRQoL in ESRD anemia patients. To identify the most common symptoms and symptom impacts of anemia of ESRD, a literature review (Vanya et al, 2014 ISPOR abstracts) and telephone interviews with clinical experts were conducted. Concept elicitation (CE) through focus groups (FGs) with ESRD anemia patients were conducted to discuss their experiences with anemia of ESRD. Through qualitative analysis, a draft PRO was developed. The draft PRO was further evaluated by an expert panel and cognitive debriefing (CD) with ESRD anemia patients. The CD interviews evaluated the clarity and comprehensibility of the instrument’s items, response options, instructions, and recall period. Twenty-one patients participated in 3 focus groups and 10 patients completed CD interviews. Mean age of the total sample was 60 years; 52% were female. The most commonly reported symptoms included pain and tiredness. The most commonly reported symptom impacts included negative impact on work/school/home and recreational activities, family relationships, hobbies, emotions, and sleep. Analysis of the CD data indicated that all 10 patients were able to interpret and understand all the items on the draft ESRD anemia instrument as intended. All items were indicated by at least half the sample as relevant to their experience with anemia of ESRD. No items were removed and minimal changes were suggested. The results of the CE and CD interviews support the content validity of the newly developed ESRD anemia PRO instrument. The instrument was confirmed to be meaningful to patients, interpretable, and appropriate for use in assessing HRQL in the ESRD anemia patient population.
A86
287 A CASE OF CUTANEOUS EXOPHILIA IN A KIDNEY TRANSPLANT RECIPIENT: J Varghese, KD Jhaveri, M Bhaskaran, D Hirschwerk, A Garg, M Sachdeva. Divisions of Nephrology, Infectious Diseases, and Dermatology, Hofstra NS-LIJ School of Medicine, NY During the post transplantation period, kidney transplant recipients are susceptible to many opportunistic infections due to their chronic immunosuppressed state. Fungal skin infections are rare infections but can be seen post transplantation. We describe a case of cutaneous exophiala, a rare form of phaeohyphomycosis, in a kidney transplant recipient. A 53 year old Caucasian male with a cadaveric renal transplant (CRT) five months prior to presentation, was noted to have a skin rash over his upper extremities. His post transplant course was complicated by calcineurin inhibitor induced thrombotic microangiopathy for which he required long course of plasmapheresis. In addition, his immunosuppresion was changed to belatacept, of which he had received five doses. At the time of presentation, he was noted to have cutaneous lesions on his bilateral upper extremities sparing the palms. The rash was described as multiple cyst-like subcutaneous lesions without surrounding inflammation or associated pruritis. A skin punch biopsy of a lesion confirmed phaeohyphomycosis and specifically cutaneous exophiala. He subsequently had an MRI Head, CT Chest and CT Sinus which were negative for disseminated fungal infection. He was started on a prolonged course of posaconazole with excellent improvement and was able to continue his immunosuppression with improving renal function. Phaeohyphomycosis is a rare fungal infection caused by several species the most common being Exophiala jeanselmei and W. dermatitidis. These organisms form pigmented hyphae in tissue and form cutaneous lesions, described as plaques or subcutaneous inflammatory cysts. One non US based case series has described good outcomes of these patients with treatment of anti fungal agents in renal transplant setting. In renal transplant recipients, it is important to consider and diagnose such fatal infections so that appropriate treatment can be initiated in a timely manner.
288 OVARIAN HYPERSTIMULATION SYNDROME CAUSING ATYPICAL RENAL DISEASE Jeny Varghese and Mala Sachdeva, Hofstra North Shore-LIJ School of Medicine, Great Neck, NY, USA. Ovarian Hyperstimulation Syndrome (OHSS) can occur as a result of in vitro fertilization (IVF). OHSS has commonly been associated with acute kidney injury, usually a result of altered hemodynamics. We describe two cases in which ovarian hyperstimulation syndrome caused renal disease. Both of these cases are the first reported cases. A 38 year old Nigerian female underwent a course of in vitro fertilization treatment and was admitted six weeks later with a diagnosis of OHSS. She was anasarcic, and laboratory studies were consistent with marked hyperlipidememia, severe hypoalbuminemia, non-oliguric acute kidney injury, and nephrotic range proteinuria of 33 grams. Kidney biopsy preformed was consistent with collapsing FSGS (C-FSGS). Patient was treated with solumedrol and cyclosporine with clinical improvement in her nephrotic syndrome. A 36 year old Caucasian female with history of polycystic ovarian syndrome presented ten days after IVF with moderate ascites, hypoalbuminemia, and progressive acute renal failure requiring hemodialysis. She was admitted with a diagnosis of OHSS. During hospitalization she sustained sequelae of a hypercoaguable state, including a cerbrovascular accident, myocardial ischemia, and bilateral renal artery thrombosis. Her kidney biopsy showed a tubulo-interstitial nephritis, interstitial edema, and tubules with cytoplasmic vacuolization. She had no subsequent renal recovery and remained dialysis dependent. Both cases occurred following IVF and in association with OHSS. VEGF has now emerged as one of the factors most likely involved in the pathophysiology of OHSS, and perhaps this could explain its association with collapsing FSGS. In the latter case, sequelae of a hypercoaguable state could explain her renal function loss. Given that IVF is a more commonly performed procedure, those involved in the care of these women should be able to recognize kidney disease so that prompt diagnosis and respective treatment can be given in a timely manner.
Am J Kidney Dis. 2015;65(4):A1-A93