Registered Nurses' experiences of psychological well-being and ill-being in their first year of practice: A qualitative meta-synthesis.

AIM: To synthesize Registered Nurses' self-reported perceptions and experiences of psychological well-being and ill-being during their first year of practice. DESIGN: Qualitative meta-synthesis. DATA SOURCES: Databases included Cumulative Index of Nursing and Allied Health Literature, Excerpta Medica database, Medical Literature Analysis and Retrieval System Online and Psychological Information. Qualitative studies were considered for inclusion if published in English, from 2009-2019, reporting primary data analysis including psychological well-being and ill-being experiences of graduate nurses in first year of practice. REVIEW METHODS: Qualitative studies were systematically identified and critically appraised. A meta-synthesis was applied using an open card sort technique to organize empirical data into a matrix of graduate nurses' voices of psychological well-being and ill-being. RESULTS: Twenty-two studies were included. Analysis revealed patterns of positive experiences and emotions. These included feeling valued and part of the team and learning from and feeling supported by other nurses. Negative experiences and emotions such as feeling overwhelmed, stressed, alone and inadequately prepared were also identified. CONCLUSION: Graduate nurses' perceptions and experiences of their psychological well-being and ill-being revealed both positive and negative dimensions during this transition period. Specific examples of strategies that may promote transition nurses' well-being and prevent ill-being were identified such as social connection and support. IMPACT: Increasing the numbers of new nursing graduates world-wide is required to strengthen health systems. Developing strategies to retain these graduates in the workforce is paramount. This review found some graduate nurses experience the transition period as a time of personal growth and fulfilment, for others this period was a stressor. These findings were illustrated in a model of 'ways to well-being'. The potential for knowledge translation of this model extends from graduate nurses as individuals, to nurse entry to practice programs and graduate nurse programs, to organizational policy targeting future health workforce. SYSTEMATIC REVIEW REGISTRATION NUMBER: CRD42020148812.

Nurse wellbeing during the coronavirus (2019) pandemic: A qualitative descriptive study.

BACKGROUND: The wellbeing of individuals influences organisational outcomes. Insight into nurses' wellbeing is crucial to a sustaining a high-quality workforce. AIM: To describe nurses' perceptions and experiences of wellbeing, work wellbeing, and mental health. METHOD: Using a qualitative descriptive design, semi-structured interviews were conducted, transcribed verbatim, analysed inductively and thematically, and reported per consolidated criteria for reporting qualitative research. FINDINGS: Nine Australian nurses were interviewed in 2020, each for 60 to 90 minutes. These nurses had a broad range of clinical roles and years of experience in metropolitan healthcare organisations. Six themes, each related to nurse wellbeing, depicted: (i) value and sense of purpose from nursing, yet also negative consequences of losing sight of oneself within the nursing role; (ii) work nurses did to disengage from their job and create a balance within their life; (iii) significance of the team and senior team as a source of both strength and opportunity for wellbeing; (iv) a range of wellbeing initiatives with a perception these were often developed, and for use, in response to crisis as opposed to preventative or proactive measures; (v) value of additional nurse wellbeing education and promotion of available support; and (vi) novel challenges and ways to wellbeing during times where resources were stretched and usual support systems impacted. DISCUSSION: Identified positive and negative consequences of nursing must be addressed when developing targeted wellbeing interventions. CONCLUSION: New ways of working and supporting individual, team and organisational wellbeing are needed for flourishing working environments. Potential strategies to either leverage or mitigate the positive and negative consequences of nursing are offered.

Comparison of Immune Profiles in Fetal Hearts with Idiopathic Dilated Cardiomyopathy, Maternal Autoimmune-Associated Dilated Cardiomyopathy and the Normal Fetus.

The etiology of idiopathic dilated cardiomyopathy (iDCM) remains unknown. Immune therapies have improved outcome in fetuses with DCM born to mothers with autoimmune disease (aDCM). The purpose of this retrospective study was to compare the myocardial B and T cell profiles in fetuses and neonates with idiopathic DCM (iDCM) versus autoimmune-mediated DCM (aDCM) and to describe the normal cell maturation within the human fetal myocardium. Of 60 fetal autopsy cases identified from institutional databases, 10 had aDCM (18-38 weeks), 12 iDCM (19-37 weeks) and 38 had normal hearts (11-40 weeks). Paraffin-embedded myocardium sections were stained for all lymphocyte (CD45), B cells (CD20, CD79a), T cells (CD3, CD4, CD7, CD8) and monocyte (CD68) surface markers. Two independent, blinded cell counts were performed. Normal hearts expressed all B and T cell markers in a bimodal fashion, with peaks at 22 and 37 weeks of gestation. The aDCM cohort was most distinct from normal hearts, with less overall T cell markers [EST -9.1 (2.6) cells/mm(2), p = 0.001], CD4 [EST -2.0 (0.6), p = 0.001], CD3 [EST -3.9 (1.0), p < 0.001], CD7 [EST -3.0 (1.1), p = 0.01] overall B cell markers [EST -4.9 (1.8), p = 0.01] and CD79a counts [EST -2.3 (0.9), p = 0.01]. The iDCM group had less overall B cell markers [EST -4.0 (1.8), p = 0.03] and CD79a [EST -1.7 (0.9), p = 0.05], but no difference in T cell markers. Autoimmune-mediated DCM fetuses have less B and T cell markers, whereas iDCM fetuses have less B cell markers compared with normal fetal hearts. The fetal immune system may play a role in the normal development of the heart and evolution of dilated cardiomyopathy.

Hobnail Hemangioma (Superficial Hemosiderotic Lymphovascular Malformation) in Children: A Series of 6 Pediatric Cases and Review of the Literature.

BACKGROUND: Hobnail hemangioma is a small benign vascular malformation of the superficial and mid-dermis with variable clinical presentation. OBJECTIVES: To review the clinical characteristics of hobnail hemangioma in pediatric patients. METHODS: A retrospective chart review performed of all histopathologically confirmed cases of hobnail hemangioma from May 2000 to December 2014. Data on demographics, clinical characteristics, and results of immunohistochemical staining were collected. RESULTS: Four male and 2 female patients identified. Congenital lesions were reported in 3 cases. The most common anatomic location was the extremities. Treatment options included observation and surgical excision. CONCLUSIONS: Hobnail hemangioma is an uncommon benign vascular malformation. Due to its benign nature, treatment is not required. If treatment is indicated, complete surgical excision appears to be the most effective option.

The role of diagnostic imaging and liver biopsy in the diagnosis of focal nodular hyperplasia in children.

BACKGROUND & AIMS: Focal nodular hyperplasia (FNH), a benign liver tumour, has a characteristic appearance on diagnostic imaging (DI) and histology. The role of liver biopsy in children for the diagnosis of FNH is unclear. This study investigates the diagnostic accuracy of DI for FNH in children without comorbidities, compared to liver biopsy. METHODS: A total of 304 consecutive patients (age <18 years) with a biopsied liver mass were retrospectively ascertained (1990-2010). Individuals with a history of malignancy, liver disease or syndromes with increased malignancy risk were excluded. DI and biopsy data were reviewed. RESULTS: After excluding 205 cases, 99 liver masses were studied. Based on histology, the most common diagnosis was hepatoblastoma (46/99, 44%) followed by FNH (23/99, 23%). The mean age at FNH diagnosis was 11.1 ± 5.2 years, with female preponderance (78%), and a median follow-up of 1.35 years (interquartile range 0.54, 4.20 years). 19/23 biopsy-proven FNH met standard criteria for FNH on DI. In 4/23 cases of biopsy-proven FNH, imaging did not suggest FNH. Two false positive cases included adenoma and fibrolamellar hepatocellular carcinoma. On review of original reports, DI had 82.6% sensitivity and 97.4% specificity for the diagnosis of FNH. On blind review, the sensitivity of DI for FNH diagnosis was 81.3% for MRI (13/16), and 53.3% for CT (8/15). CONCLUSIONS: In this cohort of children with liver masses and no comorbidities, a diagnosis of FNH by imaging was highly specific, and MRI was the most sensitive study for its diagnosis. Liver biopsy may be deferred in selected children if the DI, particularly MRI, is indicative of FNH.

Congenital infantile fibrosarcoma: review of imaging features.

BACKGROUND: Fibrosarcoma is a rare tumor in children with limited information on imaging features of these tumors in the literature. OBJECTIVE: To retrospectively review the imaging features of histologically proven congenital infantile fibrosarcoma. MATERIALS AND METHODS: The list of histologically confirmed congenital infantile fibrosarcomas between November 1999 and June 2013 was obtained from the oncology-pathology database. Imaging features and pathology reports of these tumors were reviewed. Patient charts were reviewed and clinical features, management and outcomes were recorded. RESULTS: During the study period, 13 children (9 girls and 4 boys; age range: 0 day-16 months, median age: 2.5 months) with congenital infantile fibrosarcomas were available for complete radiological review. The translocation (t[12;15]) was present in 11/13 (84.6%) and absent in 2/13. Eight/thirteen (61.5%) tumors were located in extremities (5 in lower and 3 in upper), 3/13 in thoracolumbar paraspinal regions, and one each in abdomen and sternocleidomastoid muscle. Imaging features included iso- to hyperintensity on T1-W, hyperintensity on T2-W as compared to skeletal muscles and heterogeneous enhancement. Six (37.5%) tumors showed hemorrhagic components and 2 (15.4%) showed low intensity foci. None of the patients had evidence of regional or distant metastases at diagnosis. Management included surgical resection only (1/13) and combined surgery and chemotherapy (10/13). Overall survival was 100% with a median follow-up of 49.3 months. CONCLUSION: Congenital infantile fibrosarcoma has nonspecific imaging characteristics but should be high on the differential diagnosis in a soft-tissue tumor presenting in infancy, located in an extremity and showing tumoral hemorrhage. Patients have a favorable outcome.

The distribution and frequency of pulmonary neuroendocrine cells in Down syndrome fetal lungs.

The pulmonary neuroendocrine cells (PNEC) are located in the epithelial lining of the airways and consist of solitary neuroendocrine cells (NEC) and NEC clusters, the neuroepithelial bodies (NEB). During fetal life, PNEC are the first to differentiate within the primitive airway epithelium, and bombesin expression favors branching of the respiratory tree. We investigated PNEC in Down syndrome (DS), where the lungs often show enlarged and reduced number of alveoli. Immunohistochemistry for bombesin and synaptophysin, PNEC markers, was evaluated in fetal lungs from 15 cases of DS and 11 age-matched controls from the 17th to 23rd week of gestation. Morphometric analysis assessed PNEC in the mucosal lining of each lung, expressed as number/mm. Nonparametric Mann-Whitney U test showed no statistical difference in frequency of PNEC in DS and controls. Our findings suggest that, at least in late second trimester, the distribution and frequency of PNEC in DS fetuses is not altered.

Hypopigmented micropapules in apparently quiescent morphea lesions: a manifestation of disease activity.

We present two young patients with morphea or localized scleroderma undergoing systemic treatment, who developed papular lesions on pre-existing sclerotic plaques. Histology was compatible with a papular presentation of morphea and other entities in the differential diagnosis were ruled out. We believe this is a very uncommon presentation of activity in lesions of morphea and should be made known to clinicians so that activity and progression of the disease can be recognized and treated to avoid complications.

Nurses' well-being during the coronavirus (2019) pandemic: A longitudinal mixed-methods study.

AIM: To determine prevalence, predictors and change over time of nurses' and student nurses' mental health and well-being, and explore nurses' perceptions, barriers and enablers of well-being. DESIGN: Longitudinal mixed-methods survey. METHODS: Forty-nine students and registered nurses participated from Victoria, Australia. Data were collected from December 2019 to July 2020. Validated psychometrics and free-text response questions were employed. Analysis used latent growth curve modelling, Pearson product-moment correlations and thematic analysis. RESULTS: A strong positive correlation was found between self-determination and work well-being, and a strong negative correlation between work well-being and flight risk. Several moderate relationships were found; a moderate positive correlation between work well-being and nurse manager ability, leadership and support, and a moderate negative correlation between burnout and staffing and resource adequacy. Collegial nurse-physician relationships deteriorated. Three themes, physical health, psychological well-being and social connection, were identified as important for nurses' well-being.

US-guided percutaneous needle biopsy of anterior mediastinal masses in children.

BACKGROUND: Anterior mediastinal masses in children are clinically challenging, requiring prompt histological diagnosis. OBJECTIVE: The purpose of this study was to review the experience with US-guided percutaneous core needle biopsy (PCNB) of anterior mediastinal masses in children, particularly with respect to safety and diagnostic accuracy. MATERIALS AND METHODS: We retrospectively reviewed the clinical presentation, imaging, sedation approach, procedural details and pathology results of US-guided PCNB of mediastinal masses that occurred during an 8-year period (2001-2008). Complications were graded and pathology was categorized into four groups based on adequacy and diagnostic yield. RESULTS: 32 US-guided PCNBs were performed on 32 children, mean age 12 years (range 18 months to 17 years), mean weight 48 kg (range 11.5 to 109 kg, median 49 kg). A coaxial US-guided technique was used, with a mean of 8.2 passes and a mean 7.6 cores obtained (range 2-15). There were no major complications. The biopsies were adequate in volume and quality of specimens in 29/32, and 25/32 were diagnostic. PCNB was diagnostic in all cases of non-Hodgkin disease. CONCLUSIONS: Experience with anterior mediastinal masses suggests that US-guided PCNB can be considered a viable, safe and accurate method of reaching a diagnosis in the pediatric population.

Exploring patients' and physiotherapists' visions on modelling treatments and optimising self-management strategies for patellofemoral pain: A future workshop approach.

OBJECTIVE: To identify challenges confronting patients and physiotherapists in managing patellofemoral pain by seeking their perspectives via generative activities involving critique, ideation and formulating shared visions for future treatments. DESIGN: Two Future Workshops, Reflexive Thematic Analysis. METHODS: We recruited 8 patients (median age 36 yrs, 4 women) who were experiencing patellofemoral pain and 10 physiotherapists (54 yrs, 8 women) who treated patients with the condition. Several vignette cases and design cards were constructed and included as tools for facilitating dialogue, throughout all three phases (each ∼40 min duration) of the workshops (i.e., critique, fantasy, implementation). Participants' discussions were audio recorded, transcribed and thematically analyzed independently by four investigators until no additional themes emerged. RESULTS/FINDINGS: Four themes were identified; (i) challenges confronting patients, (ii) learning to manage patellofemoral pain (knowledge), (iii) stakeholder accountability and (iv) development/use of portable applications (apps). Some challenges and strategies were related to family and social networks, financial costs, and psychological factors. Knowledge related to the condition, mental and physical impact of pain, exercises and physical activity. The physiotherapist's role in moderating accurate information was raised, as was that of the GP and personal trainer. Visions of future treatments centered about the inclusion of flexible modes of communication and cultivating mutual accountability. Social determinants and the invisible work of patients in managing their condition was apparent. CONCLUSION: Enacting patient centered care was sought/recommended - requiring consideration of social contexts and flexible delivery. The physiotherapist was seen as a source of accurate information and a point of accountability.

The myocardium of fetuses with endocardial fibroelastosis contains fewer B and T lymphocytes than normal control myocardium.

The observation that endocardial fibroelastosis (EFE) can result from an immune response to maternal autoantibody deposition in the fetal myocardium raises the possibility that the fetal immune system may contribute to the pathogenesis of idiopathic EFE and dilated cardiomyopathy (DCM). This study sought to characterize myocardial immune cell presence in fetuses and neonates with idiopathic EFE + DCM, in those with EFE + structural heart disease, and in normal control subjects. Paraffin tissue sections from fetuses identified from the pathology database were stained for B cell, T cell, macrophage, and general hematopoietic cell surface markers. Of the 14 fetuses included in the study, 5 had EFE + DCM, 4 had EFE + structural heart disease, and 5 were normal control fetuses. The EFE + DCM group had fewer B cells than the control group (0.15 vs. 0.44 cells/mm(2); p = 0.005). The EFE + heart disease group had both fewer B cells (0.18 vs. 0.44 cells/mm(2); p = 0.08) and T cells (0.29 vs. 0.80 cells/mm(2); p = 0.04) than the control group. The CD4/CD8 ratio was similar in the EFE + DCM and EFE + heart disease groups (1.0 vs. 0.9; p = 0.17) but higher in the EFE + DCM group than in the control group (0.9 vs. 0.3; p = 0.03). The myocardium of fetuses with EFE contains fewer B and T lymphocytes than normal control fetuses.

Role for Graduate Medical Education in the Response to COVID-19 Pandemic in Southeast Michigan Community Health Care System.

On March 10, 2020, the State of Michigan reported its first case of severe acute respiratory syndrome due to coronavirus disease 2019, which was admitted to Ascension Providence Hospital (APH). Michigan was the third most affected state in March 2020. To address the pandemic, Department of Graduate Medical Education joined the incident command team which consisted of APH leaders (Accreditation Council for Graduate Medical Education Designated Institutional Official, the Chief Medical Officer as commander, Chief Operating Officer, Chief of Logistics, Chief Nursing Officer, representatives from the medical and surgical sections, laboratory, finance, infection control, and occupational health). The team initiated the "crisis capacity surge plan" that was focused on patient care and led mainly by our trainee. In this correspondence we share our successful experience and provide our recommendation on how GME can navigate pandemic crisis.

New graduate nurse wellbeing, work wellbeing and mental health: A quantitative systematic review.

BACKGROUND: The contribution of work to positive mental health is increasingly apparent. Transition into the workplace causes a range of stressors for new graduate nurses who experience both psychological wellbeing and illbeing in their first year of practice. OBJECTIVE: To determine published prevalence, predictors, barriers and enablers of new graduate registered nurse wellbeing, work wellbeing and mental health. DESIGN: Systematic review of quantitative research. DATA SOURCES: Databases included Cumulative Index of Nursing and Allied Health Literature, Excerpta Medica database, Medical Literature Analysis and Retrieval System Online and Psychological Information. Quantitative and mixed-methods studies were considered for inclusion if published in English from 2009 to 2019 reporting primary data analysis including new graduate nurses' wellbeing, work wellbeing and mental health. REVIEW METHODS: Quantitative studies were systematically identified then screened and appraised against pre-determined inclusion criteria. Analysis was conducted by grouping according to analytical methods and results reported as a narrative synthesis. RESULTS: Thirty-four studies were included. The quality of the evidence was variable with just a quarter of the studies being assessed as meeting the quality criteria on all nine measures. For the new graduate nurses prevalence of wellbeing, levels of resilience, optimism, and hope were found to be high. For work wellbeing, most reported higher job satisfaction by 12-months. For work illbeing, levels of burnout were moderately high, predominantly in terms of emotional exhaustion, and stress was initially high, particularly in terms of workload, but decreased over time. For the predictors, job satisfaction was positively predicted by structural empowerment and career satisfaction, and negatively predicted by co-worker incivility, supervisor incivility and emotional exhaustion. For work illbeing, stress was a positive predictor for intent to leave. Stress reductions were associated with momentary levels of high task mastery, social acceptance and role clarity. CONCLUSIONS: For new graduate nurses, levels of emotional exhaustion, workload and stress were moderately high to high initially, decreasing over time as the graduate nurses' job satisfaction increased. Most studies focused on the nurses' intent to resign or stay and both psychological capital and work engagement positively predicted intent to stay whereas work stress positively predicted intent to resign. Resilience and group cohesion moderated the negative effects of some variables, thus may be potential enablers of work wellbeing. The standards of research reporting or design were generally sub-optimal according to quality indicators. Systematic review registration number: (CRD42020148812).

Abnormal connexin43 in arrhythmogenic right ventricular cardiomyopathy caused by plakophilin-2 mutations.

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a disorder of cardiomyocyte intercalated disk proteins causing sudden death. Heterozygous mutations of the desmosomal protein plakophilin-2 (PKP-2) are the commonest genetic cause of ARVC. Abnormal gap junction connexin43 expression has been reported in autosomal dominant forms of ARVC (Naxos and Carvajal disease) caused by homozygous mutations of desmosomal plakoglobin and desmoplakin. In tissue culture, suppression of PKP-2 results in decreased expression of connexin43. We sought to characterize the expression and localization of connexin43 in patients with ARVC secondary to heterozygous PKP-2 mutations. Complete PKP-2 gene sequencing of 27 ARVC patients was utilized to identify mutant genotypes. Endomyocardial biopsies of identified carriers were then assessed by immunofluorescence to visualize intercalated disk proteins. N-cadherin was targeted to highlight intercalated disks, followed by counterstaining for PKP-2 or connexin43 using confocal double immunofluorescence microscopy. Immunofluorescence was quantified using an AdobeA Photoshop protocol, and colocalization coefficients were determined. PKP-2 siRNA experiments were performed in mouse cardiomyocyte (HL1) cell culture with Western blot analysis to assess connexin43 expression following PKP-2 suppression. Missense and frameshift mutations of the PKP-2 gene were found in four patients with biopsy material available for analysis. Immunofluorescent studies showed PKP-2 localization to the intercalated disk despite mutations, but associated with decreased connexin43 expression and abnormal colocalization. PKP-2 siRNA in HL1 culture confirmed decreased connexin43 expression. Reduced connexin43 expression and localization to the intercalated disk occurs in heterozygous human PKP-2 mutations, potentially explaining the delayed conduction and propensity to develop arrhythmias seen in this disease.

Eosinophilic myocarditis temporally associated with conjugate meningococcal C and hepatitis B vaccines in children.

We report the first cases of tissue-proven eosinophilic myocarditis after single vaccine administration of conjugate meningococcal C and hepatitis B vaccine, respectively. The nature of histopathologic findings strongly supports hypersensitivity reaction and negates viral etiology, which is typically characterized by a lymphocytic infiltrate. Both episodes resolved with corticosteroid therapy. To enhance discussion of our cases, we performed a systematic review of the literature on postimmunization myocarditis or pericarditis, and identified 37 publications, reporting 269 cases during the search period (1966-2007). Time of onset of cardiac symptoms in all patients ranged from 1 to 30 days postimmunization.

High copper levels and increased elastolysis in a patient with cutis marmorata teleangiectasia congenita.

Cutis marmorata telangiectatica congenita (CMTC) is a rare cutaneous vascular disease presenting at birth with levido reticularis, phlebectasia, and telangiectasia, often accompanied by skin ulcerations. Extra-dermal vascular anomalies can be also detected in 30-70% of described cases. The pathomechanism responsible for development of these phenotypic changes is not well understood. Here, we report on a 16-month-old boy with CMTC, generalized vascular abnormalities and severe, nitric oxide sensitive, pulmonary hypertension associating with markedly elevated level of blood copper. Results of laboratory investigations indicated that primary cultures (passage one) of dermal fibroblasts derived from this patient were capable of normal synthesis of tropoelastin, but their net deposition of mature elastic fibers was significantly diminished as compared with cultures of normal fibroblasts. Because the low net deposition of elastin was reversed when 1 mg/ml of alpha1-antitrypsin was added to the media, we conclude that heightened elastolysis by endogenous serine proteinase's is responsible for the low net elastogenesis by CMTC fibroblasts. Since simultaneous addition of 30 microM CuSO(4) and 1 mg/ml alpha1-antitrypsin abolished the beneficial effect of this serine proteinase's inhibitor, we concluded that this may be due to copper-dependent inactivation of alpha1-antitrypsin. Our data suggest that a high level of free copper may constitute a major triggering factor contributing to the development of the CMTC phenotype.

Decreased left ventricular coronary artery density in pulmonary atresia and intact ventricular septum.

BACKGROUND: The prognosis for pulmonary atresia and intact ventricular septum (PAIVS) has been poor. Our hypothesis is that intrinsic abnormal left ventricular (LV) intramyocardial circulation might be related to the poor outcomes of these patients. METHODS: Neonatal heart specimens were examined microscopically in four groups of 6 cases each. Group I had PAIVS with ventriculocoronary artery connections (VCAC), group II had PAIVS without VCAC, group III had normal hearts, and group IV had LV hypertrophy. A projection microscope with grid overlay was used to count the LV intramyocardial coronary artery density (IMCAD), which was expressed as the number of profiles/mm(2). RESULTS: The LV IMCAD of groups I (0.40 +/- 0.14/mm(2)) and II (0.45 +/- 0.15/mm(2)) were significantly lower than those of groups III (0.77 +/- 0.11/mm(2)) and IV (0.76 +/- 0.09/mm(2); all with p = 0.002). There was no significant difference between either groups I and II (p = 0.394) or groups III and IV (p = 0.818). CONCLUSIONS: This study demonstrates lower LV IMCAD in a widely heterogeneous spectrum of neonatal hearts with PAIVS, which might potentially predispose these patients to myocardial ischemia and in turn contribute to the poor prognosis of this disease.

Scurvy diagnosed in a pediatric liver transplant patient awaiting combined kidney and liver retransplantation.

First described in the 1500 s, scurvy is infrequently seen in industrialized countries today, although vulnerable patient groups remain. A 15-yr-old girl underwent liver transplantation at age 26 months for a primary diagnosis of biliary hypoplasia, and subsequently developed late allograft failure and progressive renal insufficiency culminating in listing for combined liver retransplantation and kidney transplantation at age 13 yr. She required regular hemodialysis treatment for 12 months prior to deceased donor organ availability, with a complicated clinical course including recurrent septic episodes and severe cachexia. Ten months after initiation of hemodialysis, she presented with severe bone pain, purpura, ecchymoses, gingival hyperplasia, mucosal bleeding, and subconjunctival hemorrhages. Serial serum ascorbic acid levels were found to be extremely low (<10 micromol/L) despite routine supplementation both in her dialysate and via regular oral supplementation. Histopathology from skin biopsy revealed purpura, hyper- and parakeratosis, and follicular plugging. She had ECG and 2D echocardiogram disturbances, as well as osteopenia and sclerosis of the extremities on radiological evaluations. Therapy with high-dose ascorbic acid (1 g/day orally) led to complete resolution of skin lesions. This case highlights the importance of awareness and recognition of this historic diagnosis, and particularly in children with end-stage organ disease with severely compromised nutrition.