Exile Vol. XLII No. 2

40th Year Title Page i Epigraph by Ezra Pound ii Table of Contents ii Editorial Board iii frying fritters by Liz Bolyard \u2796 1 For Katherine by Carl Boon \u2796 1 poem paint by alex e blazer \u2797 2-3 Leftover Roses by Melissa Bostrom \u2796 4-12 O.J. (artwork) by Todd Gys \u2799 13 Untitled by Adrienne Fair \u2796 14-15 Hills by Liz Bolyard \u2796 16 A Serious Discussion with Ed Shim by Carl Boon \u2796 17 Untitled by David Kendall \u2796 18-19 Brave River by Nikole Hobbs \u2799 20-21 a wavy wail by alex e blazer \u2797 22-23 Misplaced by Tyler Smith \u2797 24 Imogene by Erin Lott \u2796 25-26 Why I can\u27t sleep at night by Colin Bossen \u2798 27 A Lovesong Never Realised by Matthew Rump \u2798 28 Contributors\u27 Notes 29-30 Special thanks to EPI Printing of Livonia, Michigan and Graphic Concepts Unlimited of Okemos, Michigan for helping to make this issue possible. -iii Cover art The Longest Neck by Todd Gys -ii

Exile Vol. XLI

39th Year Cover Art by Elisa Gargarelle \u2795 (quote from J.D. Salinger\u27s Catcher in the Rye) untitled by Aileen Jones \u2797 i Girl by Colin Bossen \u2798 1 sun by Alex Blazer \u2796 2 Shifting by Alex Blazer \u2796 2 The Fish by Sarah Ramsey \u2795 3 New Woman by Lisa Stillman \u2795 4 Why by Lelei Jennings \u2795 5 Camel Cafe by Jeremy Aufrance \u2795 5 Jenny by Lizzy Loud \u2795 6 Beautiful Dreamer by Melissa Bostrom \u2796 7 Rising by Lizzy Loud \u2795 12 Pinsetter by Jeremy Aufrance \u2795 13 A Greater Distance by Jeff Boon \u2795 14 Shiho by Jeff Boon \u2795 15 Sub-stance by Alex Blazer \u2796 15 Sisters by Gretchen Hambley \u2796 16 Anne Sexton by Allison Lemieux \u2796 17 The Holy Grail... by Ed Shim \u2795 17 untitled by Liz Bolyard \u2796 18 23 by Keith Chapman \u2795 18 Bang, Zoom! by Victoria Lyall \u2796 19 Gabe and Me by Heather Trabert \u2797 20 Tornado Summer by Liz Bolyard \u2796 21 Nude by Elise Gargarella \u2795 21 Why I can\u27t tell short stories by Colin Bossen \u2798 22 america by Lynn Tramonte \u2798 24 Upon Being Asked... by Matt Makman \u2796 24 Being Azra by Lynn Tramonte \u2798 25 Mystic Truths by Adrienne Binni \u2795 27 King\u27s Court by Elisha Gargarella \u2795 27 Incense by Erin Lott \u2796 28 Sunday Morning... by Lisa Stillman \u2795 33 untitled by Elisa Gargarella \u2795 33 Quien no ha visto... by Adrienne Binni \u2795 34 The Space Between Us by Allison Lemieux \u2795 35 searching for the Bermuda... by Victoria Lyall \u2796 35 untitled by Man Chhoa \u2796 36 The Hunted by J. Murdoch Matheson \u2796 37 Editorial decisions are shared equally among the editorial board. -4

Neutropenia in Barth syndrome:characteristics, risks, and management

PURPOSE OF REVIEW: Barth syndrome (BTHS) is an X-linked disease characterized by defective remodeling of phospholipid side chains in mitochondrial membranes. Major features include neutropenia, dilated cardiomyopathy, motor delay and proximal myopathy, feeding problems, and constitutional growth delay. We conducted this review of neutropenia in BTHS to aid in the diagnosis of this disease, and to improve understanding of both the consequences of neutropenia and the benefits of treatment with granulocyte colony-stimulating factor (G-CSF). RECENT FINDINGS: In 88 patients with BTHS, neutropenia, that is, at least one count below 1.5 × 10/l, was detected in 74 (84%) and 44% had severe chronic neutropenia, with multiple counts below 0.5 × 10/l. The pattern of neutropenia varied between intermittent and unpredictable, chronic and severe, or cyclical with mathematically regular oscillations. Monocytosis, that is, monocytes more than 1.0 × 10/l, was observed at least once in 64 of 85 (75%) patients. G-CSF was administered to 39 of 88 patients (44%). Weekly average G-CSF doses ranged from 0.12 to 10.92 μg/kg/day (mean 1.16 μg/kg/day, median 1.16 μg/kg/day). Antibiotic prophylaxis was additionally employed in 21 of 26 neutropenic patients. Pretreatment bone marrow evaluations predominantly showed reduced myeloid maturation which normalized on G-CSF therapy in seven of 13 examined. Consistent clinical improvement, with reduced signs and symptoms of infections, was observed in response to prophylactic G-CSF ± prophylactic antibiotics. However, despite G-CSF and antibiotics, one adult patient died with multiple infections related to indwelling medical devices and gastrostomy site infection after 15.5 years on G-CSF and a pediatric patient required gastrostomy removal for recurrent abdominal wall cellulitis. SUMMARY: BTHS should be considered in any men with neutropenia accompanied by any of the characteristic features of this syndrome. Prophylaxis with G-CSF ± antibiotics prevents serious bacterial infections in the more severe neutropenic patients although infections remain a threat even in patients who are very compliant with therapy, especially in those with indwelling devices

Neutropenia in Barth syndrome : characteristics, risks, and management

PURPOSE OF REVIEW: Barth syndrome (BTHS) is an X-linked disease characterized by defective remodeling of phospholipid side chains in mitochondrial membranes. Major features include neutropenia, dilated cardiomyopathy, motor delay and proximal myopathy, feeding problems, and constitutional growth delay. We conducted this review of neutropenia in BTHS to aid in the diagnosis of this disease, and to improve understanding of both the consequences of neutropenia and the benefits of treatment with granulocyte colony-stimulating factor (G-CSF). RECENT FINDINGS: In 88 patients with BTHS, neutropenia, that is, at least one count below 1.5 × 10/l, was detected in 74 (84%) and 44% had severe chronic neutropenia, with multiple counts below 0.5 × 10/l. The pattern of neutropenia varied between intermittent and unpredictable, chronic and severe, or cyclical with mathematically regular oscillations. Monocytosis, that is, monocytes more than 1.0 × 10/l, was observed at least once in 64 of 85 (75%) patients. G-CSF was administered to 39 of 88 patients (44%). Weekly average G-CSF doses ranged from 0.12 to 10.92 μg/kg/day (mean 1.16 μg/kg/day, median 1.16 μg/kg/day). Antibiotic prophylaxis was additionally employed in 21 of 26 neutropenic patients. Pretreatment bone marrow evaluations predominantly showed reduced myeloid maturation which normalized on G-CSF therapy in seven of 13 examined. Consistent clinical improvement, with reduced signs and symptoms of infections, was observed in response to prophylactic G-CSF ± prophylactic antibiotics. However, despite G-CSF and antibiotics, one adult patient died with multiple infections related to indwelling medical devices and gastrostomy site infection after 15.5 years on G-CSF and a pediatric patient required gastrostomy removal for recurrent abdominal wall cellulitis. SUMMARY: BTHS should be considered in any men with neutropenia accompanied by any of the characteristic features of this syndrome. Prophylaxis with G-CSF ± antibiotics prevents serious bacterial infections in the more severe neutropenic patients although infections remain a threat even in patients who are very compliant with therapy, especially in those with indwelling devices

Neutropenia in Barth syndrome : characteristics, risks, and management

PURPOSE OF REVIEW: Barth syndrome (BTHS) is an X-linked disease characterized by defective remodeling of phospholipid side chains in mitochondrial membranes. Major features include neutropenia, dilated cardiomyopathy, motor delay and proximal myopathy, feeding problems, and constitutional growth delay. We conducted this review of neutropenia in BTHS to aid in the diagnosis of this disease, and to improve understanding of both the consequences of neutropenia and the benefits of treatment with granulocyte colony-stimulating factor (G-CSF). RECENT FINDINGS: In 88 patients with BTHS, neutropenia, that is, at least one count below 1.5 × 10/l, was detected in 74 (84%) and 44% had severe chronic neutropenia, with multiple counts below 0.5 × 10/l. The pattern of neutropenia varied between intermittent and unpredictable, chronic and severe, or cyclical with mathematically regular oscillations. Monocytosis, that is, monocytes more than 1.0 × 10/l, was observed at least once in 64 of 85 (75%) patients. G-CSF was administered to 39 of 88 patients (44%). Weekly average G-CSF doses ranged from 0.12 to 10.92 μg/kg/day (mean 1.16 μg/kg/day, median 1.16 μg/kg/day). Antibiotic prophylaxis was additionally employed in 21 of 26 neutropenic patients. Pretreatment bone marrow evaluations predominantly showed reduced myeloid maturation which normalized on G-CSF therapy in seven of 13 examined. Consistent clinical improvement, with reduced signs and symptoms of infections, was observed in response to prophylactic G-CSF ± prophylactic antibiotics. However, despite G-CSF and antibiotics, one adult patient died with multiple infections related to indwelling medical devices and gastrostomy site infection after 15.5 years on G-CSF and a pediatric patient required gastrostomy removal for recurrent abdominal wall cellulitis. SUMMARY: BTHS should be considered in any men with neutropenia accompanied by any of the characteristic features of this syndrome. Prophylaxis with G-CSF ± antibiotics prevents serious bacterial infections in the more severe neutropenic patients although infections remain a threat even in patients who are very compliant with therapy, especially in those with indwelling devices