- Optimizing Cardiovascular Care in Children With Acute Myeloid Leukemia to Improve Cancer-Related Outcomes. [Journal Article]
- JCJ Clin Oncol 2018 Nov 13; :JCO1801421
- The Oncology Grand Rounds series is designed to place original reports published in the Journal into clinical context. A case presentation is followed by a description of diagnostic and management ch...
The Oncology Grand Rounds series is designed to place original reports published in the Journal into clinical context. A case presentation is followed by a description of diagnostic and management challenges, a review of the relevant literature, and a summary of the authors' suggested management approaches. The goal of this series is to help readers better understand how to apply the results of key studies, including those published in Journal of Clinical Oncology, to patients seen in their own clinical practice. A 14-year-old African American female presented with fatigue, easy bruising, and fever. On examination, she had scattered bruising, lymphadenopathy, and hepatosplenomegaly. Laboratory evaluation revealed pancytopenia with peripheral blasts, and acute myeloid leukemia (AML; French-American-British M2, t[8;21][q22;q22.1]) was diagnosed on bone marrow biopsy. A baseline echocardiogram revealed normal left ventricular (LV) systolic function (ejection fraction [EF], 60%; shortening fraction [SF], 32%), and conventional chemotherapy was initiated that consisted of two cycles of remission induction (cytarabine, etoposide, and daunorubicin [50 mg/m2 × 3 days per cycle]) followed by intensification 1 (high-dose cytarabine and etoposide), intensification 2 (high-dose cytarabine and mitoxantrone [12 mg/m2/dose daily; four total doses]), and intensification 3 (high-dose cytarabine and l-asparaginase). Of note, an echocardiogram was not repeated before the start of intensification 1. During intensification 1, the patient developed Streptococcus viridans sepsis, which required 4 days in the intensive care unit with antimicrobial and inotropic support. Repeat echocardiogram after recovery from the sepsis episode demonstrated low-normal LV systolic function (EF, 53%; SF, 27%), and she subsequently began intensification 2. On day 3 of intensification 2, the patient developed afebrile tachypnea, tachycardia, and an increasing oxygen requirement. Chest x-ray revealed cardiomegaly and pulmonary vascular congestion. Cardiac troponins were normal, whereas N-terminal pro B-type natriuretic peptide was 10 times the upper limit of normal. Repeat echocardiogram showed an enlarged LV with moderate to severely depressed LV function (EF, 28%; SF, 14%). Day 4 mitoxantrone was omitted and a cardiology consult obtained.
- Dermatological manifestations in Noonan syndrome: A prospective multicentric study of 129 mutation-positive patients. [Journal Article]
- BJBr J Dermatol 2018 Nov 12
- CONCLUSIONS: Cutaneous phenotype of NS with a PTPN11 mutation is generally mild and non-specific, whereas the absence of a PTPN11 mutation is associated with a high frequency of keratinization disorders and hair abnormalities. This article is protected by copyright. All rights reserved.
- Idiopathic Purpura With Gray Platelets: an Acquired Form of Gray Platelet Syndrome. [Journal Article]
- JPJ Pediatr Hematol Oncol 2018 Oct 16
- An acquired, transient bleeding disorder that predominantly affects children in Southeast Asia has been reported for the last 4 decades. The condition has been named idiopathic purpura with gray plat...
An acquired, transient bleeding disorder that predominantly affects children in Southeast Asia has been reported for the last 4 decades. The condition has been named idiopathic purpura with gray platelets (IPGP) or acquired platelet dysfunction with eosinophilia. In a retrospective review from a private pediatric clinic over an 8-year period, 10 consecutive children were diagnosed as IPGP with a mean age of 8.4 (3.7 to 16.2) years. Eosinophilia (>0.5×10/L) was absent in 1, while gray platelets were consistently found in all cases with a mean proportion of 64.5% (40% to 80%). Platelet aggregation tests were performed in 9 patients with abnormal responses consistent with platelet storage pool defect. All children recovered completely and spontaneously from 1 to 4 months after diagnosis without specific therapy. In an otherwise well child who presents abruptly with easy bruising and a platelet count >100×10/L, IPGP can be readily recognized as an acquired form of gray platelet syndrome. Eosinophilia is common but not mandatory for diagnosis.
- Hemophilia A Complicated by Ulcerative Colitis. [Journal Article]
- CRCase Rep Gastrointest Med 2018; 2018:2342618
- CONCLUSIONS: Ulcerative colitis with underlying F VIII deficiency can result in serious, prolonged, and possibly fatal bleeding if left unrecognized and untreated. Treatment of both conditions concurrently utilizing tertiary facilities and consultations appears to be the safest strategy for management.
- Arterial Switch Operation in a Patient With Ehlers-Danlos Syndrome Type IV. [Journal Article]
- WJWorld J Pediatr Congenit Heart Surg 2018 Oct 08; :2150135118769418
- Ehlers-Danlos syndrome vascular type IV is characterized by translucent skin, easy bruising, and fragility of arteries. A full-term female infant presented at four weeks of age with a diagnosis of d-...
Ehlers-Danlos syndrome vascular type IV is characterized by translucent skin, easy bruising, and fragility of arteries. A full-term female infant presented at four weeks of age with a diagnosis of d-transposition of the great arteries with restrictive atrial septal defect. She successfully underwent emergent balloon atrial septostomy and placement of patent ductus arteriosus (PDA) stent. She required restenting of the PDA and pulmonary artery banding prior to arterial switch procedure. At 16 months of age, the patient successfully underwent arterial switch procedure without complication. This report demonstrates the feasibility of an arterial switch operation along with long-term follow-up of this rare condition.
- Successful hyaluronic acid filler injection in a chronic myeloid leukemia patient taking imatinib mesylate. [Journal Article]
- JCJ Cosmet Laser Ther 2018 Oct 04; :1-2
- Dermal fillers are highly favored around the globe as minimally invasive or nonsurgical procedures. Imatinib mesylate is the first-line treatment for patients diagnosed with chronic myeloid leukemia....
Dermal fillers are highly favored around the globe as minimally invasive or nonsurgical procedures. Imatinib mesylate is the first-line treatment for patients diagnosed with chronic myeloid leukemia. However, some studies describe that imatinib mesylate may increase the tendency of skin fragility which can lead to easy bruising and hyperpigmentation after invasive skin procedures. Yet, to our knowledge, no studies have described any successful dermal filler injection performed on patients who are under imatinib mesylate treatment. Hence, we present a case successfully treated with hyaluronic acid filler injection on a patient under imatinib mesylate treatment. We carefully propose that hyaluronic acid filler can be an effective means of rejuvenation and cosmetic enhancement for those under imatinib mesylate treatment.
- Prevalence and frequency of self-perceived systemic features in people with joint hypermobility syndrome/Ehlers-Danlos syndrome hypermobility type. [Journal Article]
- CRClin Rheumatol 2018 Sep 19
- Some commonly reported systemic features of joint hypermobility syndrome (JHS)/Ehlers-Danlos syndrome hypermobility type (EDS-HT) are absent from nosologies due to insufficient validity. The primary ...
Some commonly reported systemic features of joint hypermobility syndrome (JHS)/Ehlers-Danlos syndrome hypermobility type (EDS-HT) are absent from nosologies due to insufficient validity. The primary aim was to examine the hypothesised high prevalence and frequency of orthostatic intolerance, easy bruising, and urinary incontinence in adults with JHS/EDS-HT and secondarily to determine the association between extent of generalised joint hypermobility (GJH) and these systemic features. A cross-sectional cohort study was conducted via online recruitment of medically diagnosed JHS/EDS-HT patients. A survey collected demographic data and clinical history. A subgroup of participants underwent physical testing of GJH using the Beighton score and Lower Limb Assessment Score (LLAS). Descriptive analysis was performed on demographic data and self-reported non-musculoskeletal systemic features. Correlation of GJH scores and systemic features were performed using Spearman's rank correlation. The survey was completed by 116 individuals (95% female; 16-68 years) with 57 (93% female) also participating in the physical assessment. The most prevalent systemic feature was orthostatic intolerance (98%), followed by easy bruising and urinary incontinence (97% and 84% respectively). Of those reporting symptoms of orthostatic intolerance, easy bruising, and urinary incontinence, 58%, 40%, and 18% described them as very highly frequent respectively (frequency > 75%). No significant correlations were found between the extent of systemic features and GJH scores as measured by either the Beighton score or the LLAS. The high prevalence and frequency of the systemic features found in this study, which are omitted in diagnostic classification criteria, suggest that further research on their diagnostic accuracy is warranted.
- Detailed Sonographic Anatomy of Dorsal Hand Augmentation With Hyaluronidase and Calcium Hydroxyapatite Fillers. [Journal Article]
- ASAesthet Surg J 2018 Sep 05
- CONCLUSIONS: The author reestablished sonographic anatomy for the first time. Veins and tendons became less apparent after injections.
- GeneReviews® [BOOK]
- BOOKUniversity of Washington, Seattle: Seattle (WA)
- Classic Ehlers-Danlos syndrome (cEDS) is a connective tissue disorder characterized by skin hyperextensibility, atrophic scarring, and generalized joint hypermobility (GJH). The skin is soft and doug...
Classic Ehlers-Danlos syndrome (cEDS) is a connective tissue disorder characterized by skin hyperextensibility, atrophic scarring, and generalized joint hypermobility (GJH). The skin is soft and doughy to the touch, and hyperextensible, extending easily and snapping back after release (unlike lax, redundant skin, as in cutis laxa). The skin is fragile, as manifested by splitting of the dermis following relatively minor trauma, especially over pressure points (knees, elbows) and areas prone to trauma (shins, forehead, chin). Wound healing is poor, and stretching of scars after apparently successful primary wound healing is characteristic. Complications of joint hypermobility, such as dislocations of the shoulder, patella, digits, hip, radius, and clavicle, usually resolve spontaneously or are easily managed by the affected individual. Other features include hypotonia with delayed motor development, fatigue and muscle cramps, and easy bruising. Mitral valve prolapse can occur infrequently, but tends to be of little clinical consequence. Aortic root dilatation has been reported, appears to be more common in young individuals, and rarely progresses.
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- StatPearls [BOOK]
- BOOKStatPearls Publishing: Treasure Island (FL)
- Chediak Higashi syndrome (CHS) is a rare autosomal recessive condition that was initially described by Beguez-Cesar in1943. Chediak in 1952 and Higashi in 1954 then discovered the maldistribution of ...
Chediak Higashi syndrome (CHS) is a rare autosomal recessive condition that was initially described by Beguez-Cesar in1943. Chediak in 1952 and Higashi in 1954 then discovered the maldistribution of myeloperoxidases in the granules of the neutrophils in affected patients. It is characterized by oculocutaneous albinism, easy bruising, abnormal functions of the natural killer cells, and recurrent pyogenic infections and is a result of a mutation in the lysosomal trafficking regulator (LYST) gene. Individuals also may develop neurological symptoms such as ataxia and neuropathies which could be a predominant feature in the atypical forms of the disease. The presence of abnormally large intracytoplasmic granules, especially in white blood cells and bone marrow, are diagnostic. Morbidity is a result of recurrent infections or the development of accelerated phase where there is lymphoproliferation into major organs. Eighty percent of the deaths occur in the first decade of life, and those who survive into adulthood develop progressive neurological symptoms. Treatment is with an allogeneic hematopoietic stem cell transplantation. This, however, only cures the hematological and immune dysfunction; it does not stop the progressive neurological impairment.