Darier’s disease is characterized by greasy and scaly papules that primarily affect seborrheic and intertriginous areas which is caused by a mutation in the ATP2A2 gene. Histopathologically, the disease is characterized by acantholysis and dyskeratosis. Among the diverse presentations, the segmental type follows a linear distribution along the lines of Blaschko. Herein, we present a case of a 54-year-old male with generalized erythematous papules that had been linearly distributed across his body for two decades. Lesions on his trunk and extremities were confined to the right side, whereas those on the scalp and face exhibited multiple segmental presentations. Histopathological examination revealed acantholysis and dyskeratosis in the epidermis, confirming the diagnosis of type 1 segmental Darier’s disease. This case underscores the rarity of type 1 segmental Darier’s disease, particularly with multiple segmental involvement and highlights the complexity and variability of this dermatological condition.

Darier’s disease is characterized by greasy and scaly papules that primarily affect seborrheic and intertriginous areas which is caused by a mutation in the ATP2A2 gene. Histopathologically, the disease is characterized by acantholysis and dyskeratosis. Among the diverse presentations, the segmental type follows a linear distribution along the lines of Blaschko. Herein, we present a case of a 54-year-old male with generalized erythematous papules that had been linearly distributed across his body for two decades. Lesions on his trunk and extremities were confined to the right side, whereas those on the scalp and face exhibited multiple segmental presentations. Histopathological examination revealed acantholysis and dyskeratosis in the epidermis, confirming the diagnosis of type 1 segmental Darier’s disease. This case underscores the rarity of type 1 segmental Darier’s disease, particularly with multiple segmental involvement and highlights the complexity and variability of this dermatological condition.

Darier’s disease is characterized by greasy and scaly papules that primarily affect seborrheic and intertriginous areas which is caused by a mutation in the ATP2A2 gene. Histopathologically, the disease is characterized by acantholysis and dyskeratosis. Among the diverse presentations, the segmental type follows a linear distribution along the lines of Blaschko. Herein, we present a case of a 54-year-old male with generalized erythematous papules that had been linearly distributed across his body for two decades. Lesions on his trunk and extremities were confined to the right side, whereas those on the scalp and face exhibited multiple segmental presentations. Histopathological examination revealed acantholysis and dyskeratosis in the epidermis, confirming the diagnosis of type 1 segmental Darier’s disease. This case underscores the rarity of type 1 segmental Darier’s disease, particularly with multiple segmental involvement and highlights the complexity and variability of this dermatological condition.

Darier’s disease is characterized by greasy and scaly papules that primarily affect seborrheic and intertriginous areas which is caused by a mutation in the ATP2A2 gene. Histopathologically, the disease is characterized by acantholysis and dyskeratosis. Among the diverse presentations, the segmental type follows a linear distribution along the lines of Blaschko. Herein, we present a case of a 54-year-old male with generalized erythematous papules that had been linearly distributed across his body for two decades. Lesions on his trunk and extremities were confined to the right side, whereas those on the scalp and face exhibited multiple segmental presentations. Histopathological examination revealed acantholysis and dyskeratosis in the epidermis, confirming the diagnosis of type 1 segmental Darier’s disease. This case underscores the rarity of type 1 segmental Darier’s disease, particularly with multiple segmental involvement and highlights the complexity and variability of this dermatological condition.

Purpose: This study aimed to describe adult living donor liver transplantation (LDLT) for acute liver failure and evaluate its clinical significance by comparing its surgical and survival outcomes with those of deceased donor liver transplantation (DDLT). Methods: We retrospectively reviewed the medical records of 267 consecutive patients (161 LDLT recipients and 106 DDLT recipients) aged 18 years or older who underwent liver transplantation between January 2006 and December 2020. Results: The mean periods from hepatic encephalopathy to liver transplantation were 5.85 days and 8.35 days for LDLT and DDLT, respectively (P = 0.091). Among these patients, 121 (45.3%) had grade III or IV hepatic encephalopathy (living, 34.8% vs. deceased, 61.3%; P < 0.001), and 38 (14.2%) had brain edema (living, 16.1% vs. deceased, 11.3%; P = 0.269) before liver transplantation. There were no significant differences in in-hospital mortality (living, 11.8% vs. deceased, 15.1%; P = 0.435), 10-year overall survival (living, 90.8% vs. deceased, 84.0%; P = 0.096), and graft survival (living, 83.5% vs. deceased, 71.3%;P = 0.051). However, postoperatively, the mean intensive care unit stay was shorter in the LDLT group (5.0 days vs. 9.5 days, P < 0.001). In-hospital mortality was associated with vasopressor use (odds ratio [OR], 3.40; 95% confidence interval [CI], 1.45–7.96; P = 0.005) and brain edema (OR, 2.75; 95% CI, 1.16–6.52; P = 0.022) of recipient at the time of transplantation. However, LDLT (OR, 1.26; 95% CI, 0.59–2.66; P = 0.553) was not independently associated with in-hospital mortality. Conclusion LDLT is feasible for acute liver failure when organs from deceased donors are not available.

Purpose: We used arterial spin labeling (ASL) perfusion magnetic resonance (MR) imaging to evaluate cerebral perfusion abnormalities in patients with seizures within 24 hours of symptom onset. Materials and Methods: A retrospective search of our institutional database identified 27 patients who had undergone ASL perfusion studies for seizures or seizure-like symptoms.The inclusion criteria were as follows: 1) history of seizure, 2) MR examination performed within 24 hour of seizure onset, and 3) localized perfusion abnormality on ASL. We evaluated the presence, location, and extent of perfusion abnormalities on ASL and signal abnormalities on fluid-attenuated inversion recovery (FLAIR), diffusion-weighted image (DWI), and susceptibility-weighted image (SWI), respectively. All pathological MR findings, accompanying focal neurological symptoms, and electroencephalogram (EEG) findings were compared. Results: The mean time from symptom onset to MR examination was 5 h 54 min. All patients (n = 27) showed localized increased perfusion on ASL perfusion imaging. On FLAIR imaging, 20 patients (74.1%) showed hyperintensity in the area of perfusion abnormality.In 19 patients (70.4%), DWI showed hyperintensity of the lesion with decreased apparent diffusion coefficient value (ADC). Seven patients (25.9%) showed a focal parenchymal area of pseudo-narrowed cortical veins on SWI, associated with focal hyperperfusion. In 20 patients (74.1%), the extent of perfusion abnormalities on ASL was greater than that of signal abnormalities on FLAIR or DWI. In 14/16 patients (87.5%) with abnormal EEG findings, the area with EEG findings and the location of the hyperperfusion abnormality on ASL corresponded. Conclusion In patients with seizures within 24 hours of symptom onset, ASL perfusion imaging revealed localized hyperperfusion, which was more frequent than signal intensity abnormalities on FLAIR or DWI. The locations of hyperperfusion areas correlated with EEG abnormalities. Thus, the ASL sequence may be a useful clinical assessment protocol for evaluating patients with seizures.

Purpose: Studies have yielded contradictory results on whether donor sex and donor-recipient sex disparity affect hepatocellular carcinoma (HCC) recurrence after living donor liver transplantation (LDLT). The present study assessed whether donor sex or donor-recipient sex disparity affects HCC recurrence after LDLT at a high-volume center. Methods: This study included 772 HCC patients who underwent LDLT between January 2006 and December 2015 at Asan Medical Center. Patients were divided into 4 groups based on the sex of the donor and recipient: male-to-male (n = 490, 63.5%), male-to-female (n = 75, 9.7%), female-to-male (n = 170, 22.0%), and female-to-female (n = 37, 4.8%). Results: Disease-free survival (DFS; P = 0.372) and overall survival (OS; P = 0.591) did not differ significantly among the 4 groups. DFS also did not differ significantly between LDLT recipients with male and female donors (P = 0.792) or between male and female recipients (P = 0.084). After patient matching with an α-FP/des-γ-carboxy prothrombin/tumor volume score cutoff of 5logs, donor-recipient sex disparity did not significantly affect DFS (P = 0.598) or OS (P = 0.777). There were also no differences in DFS in matched LDLT recipients with male and female donors (P = 0.312) or between male and female recipients (P = 0.374). Conclusion Neither donor sex nor donor-recipient sex disparity significantly affected posttransplant HCC recurrence.

The phenotypic variability of variant Turner syndrome is wide, ranging from characteristic clinical features to those that are hardly distinguishable from the general population. A 4-year-old girl presented with multiple brownish macules and patches on the trunk and upper extremities as well as axillary freckles. Exome sequencing and chromosomal microarray testing revealed a microdeletion at Xp22.33p22.11 leading to a diagnosis of Turner syndrome. Here we describe an unusual case of variant Turner syndrome with multiple café-au-lait spots.

Purpose: Extremely preterm infants are prone to brain injury and underdevelopment. Intraventricular hemorrhage (IVH) is the most common cause of brain injury and a significant risk factor for neurodevelopmental delay in preterm infants. Severe IVH is known to have a poor outcome; however, the outcomes of low-grade IVH remain controversial. This study aimed to evaluate neurodevelopmental outcomes and brain segmental volumes of preterm infants with low-grade IVH. Methods: This retrospective cohort study included 109 extremely preterm infants who underwent term equivalent age-magnetic resonance imaging and neurodevelopmental evaluation at a corrected age of 18 to 24 months. We compared infants with and without low-grade IVH. Results: Among the 109 extremely preterm infants, 25 had low-grade IVH and 84 had no IVH. There were no significant differences in the neurodevelopmental outcomes between the low-grade and no IVH groups. In multivariate analysis, low-grade IVH was associated with a smaller medullary volume (adjusted odds ratio, 0.575; 95% confidence interval, 0.346 to 0.957; P=0.034). Conclusion We found no significant differences in the neurodevelopmental outcomes of extremely preterm infants at a corrected age of 18 to 24 months between those with low-grade IVH and those without IVH. Low-grade IVH was associated with a smaller medullary volume.