Dermatopathia Pigmentosa Reticularis

Last update 01 Nov 2024

Basic Info

Synonyms

DERMATOPATHIA PIGMENTOSA RETICULARIS, DPR, DPR - Dermatopathia pigmentosa reticularis

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Introduction

A rare, genetic, ectodermal dysplasia characterized by a widespread, early-onset, reticulate hyperpigmentation that persists throughout life, mild, diffuse non-cicatricial alopecia, and onychodystrophy. There are no dental anomalies. Patients may also present with adermatoglyphia, palmoplantar hyperkeratosis, acral dorsal blistering, and hypohidrosis or hyperhidrosis.

100 Clinical Results associated with Dermatopathia Pigmentosa Reticularis

100 Translational Medicine associated with Dermatopathia Pigmentosa Reticularis

0 Patents (Medical) associated with Dermatopathia Pigmentosa Reticularis

Literatures (Medical) associated with Dermatopathia Pigmentosa Reticularis

01 Jul 2024·European Archives of Oto-Rhino-Laryngology

Dorsal preservation rhinoplasty versus dorsal hump reduction: a randomized prospective study, functional and aesthetic outcomes

Article

Author: El-Antably, Adel Said ; Gharib, Fadi Mahmoud ; ElBestar, Mahmoud ; Alsakka, Mahmoud Abdelaziz ; Al-Sebeih, Khalid Hamad

PURPOSETo compare the functional and esthetic outcomes of dorsal preservation rhinoplasty (DPR) and conventional dorsal hump reduction (DHR) in primary rhinoplasty using patient-reported outcome measures (PROMs) and cone beam computed tomography (CBCT).METHODSIn our randomized prospective double-blinded clinical trial, 50 patients had dorsal nasal hump surgery between October 2021 and November 2022 in our tertiary referral center. All surgeries were done by the same surgeon. Patients were randomly assigned to two groups: Group (A): 25 patients had DPR, and group (B): 25 patients underwent DHR. Pre-operative and post-operative evaluations were conducted using standardized cosmesis and health nasal outcomes survey (SCHNOS), surgeons' rhinoplasty evaluation questionnaire (SREQ), and the CBCT.RESULTSFollowing an average of 7.22 ± 2.07 months, patients in both groups reported significantly higher levels of satisfaction, as measured by the SCHNOS score (p < 0.001) and the average of three SREQ scores (p < 0.001). These results align with the radiological analysis, which denoted an overall improvement in the average of both sides' internal nasal valve angle and cross-sectional area after surgery with (p = 0.001) and (p = 0.085), respectively, for the DPR group and with (p = 0.281) and (p = 0.014), respectively, for the DHR group. There was no statistically significant difference in outcomes between both groups (p > 0.05).CONCLUSIONDorsal preservation is a viable alternative to conventional dorsal hump reduction in primary rhinoplasty. There was no difference in the functional and esthetic outcomes between both techniques, which were verified by radiological investigation.

02 Jan 2024·Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

Clinical factors and vascular endothelial growth factor as determinants of disease progression in Indian patients with amyotrophic lateral sclerosis

Article

Author: Garg, Divyani ; Vibha, Deepti ; Thomas, Aneesha ; Vivekanandhan, Subbiah ; Shukla, Garima ; Kumar, Amit ; Pandit, Awadh Kishor ; Srivastava, Achal Kumar ; Prasad, Kameshwar

OBJECTIVEAmyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder. Prognostication remains sub-optimally defined. We aimed to assess clinical determinants of disease progression rates in Indian patients with ALS and to assess the role of vascular endothelial growth factor (VEGF) in disease progression.METHODSIn this cross-sectional study, consecutive patients with clinically definite/probable ALS according to the revised El Escorial criteria and controls were included. Patients were classified into fast or slow progressors based on disease progression rate (DPR). Serum and CSF VEGF level was assessed for patients and controls.RESULTSOf 142 patients recruited, 93 (65.5%) were male. Mean age at enrollment was 49.37 ± 12.65 years. Mean duration of symptoms was 20.53 ± 20.88 months. Mean DPR was 1.14 ± 0.94. Based on DPR, 81 (57%) patients were slow progressors and 61 (43%) were fast progressors. Univariate analysis demonstrated a statistically significant association of DPR with age at onset, symptom duration, time to spread, wasting of small muscles of the hand, frontal release signs, and neurophysiologic bulbar abnormalities. On multivariate analysis, age at onset and symptom duration had a significant association with disease progression. The CSF VEGF levels of ALS patients (46.18 ± 27.8) were significantly elevated compared to controls (25.95 ± 25.64 pg/ml) (p = 0.001), but not serum VEGF.CONCLUSIONAge at symptom onset and duration of disease had a significant impact on disease progression in Indian patients with ALS. CSF VEGF levels were significantly elevated in ALS compared to controls, indicating the role of CSF VEGF as a potential biomarker.

01 Nov 2023·Physiological Measurement

ECGPsychNet: an optimized hybrid ensemble model for automatic detection of psychiatric disorders using ECG signals

Article

Author: Khare, Smith Kashiram ; Acharya, Rajendra ; Gadre, Vikram M

AbstractBackground. Psychiatric disorders such as schizophrenia (SCZ), bipolar disorder (BD), and depression (DPR) are some of the leading causes of disability and suicide worldwide. The signs and symptoms of SCZ, BD, and DPR vary dynamically and do not have uniform detection strategies. The main causes of delays in the detection of psychiatric disorders are negligence by immediate caregivers, varying symptoms, stigma, and limited availability of physiological signals. Motivation. The brain functionality in the patients with SCZ, BD, and DPR changes compared to the normal cognition population. The brain–heart interaction plays a crucial role in tracking the changes in cardiac activities during such disorders. Therefore, this paper explores the application of electrocardiogram (ECG) signals for the detection of three psychiatric (SCZ, BD, and DPR) disorders. Method. This paper develops ECGPsychNet an ensemble decomposition and classification technique for the automated detection of SCZ, BD, and DPR using ECG signals. Three well-known decomposition techniques, empirical mode decomposition, variational mode decomposition, and tunable Q wavelet transform (TQWT), are used to decompose the ECG signals into various subbands (SBs). Various features are extracted from the different SBs and classified using optimizable ensemble techniques using two validation techniques. Results. The developed ECGPsychNet has obtained the highest classification accuracy of 98.15% using the features from the sixth SB of TQWT. Our proposed model has the highest detection rates of 98.96%, 96.04%, and 95.12% for SCZ, DPR, and BD. Conclusions. Our developed prototype is able to detect SCZ, DPR, and BD using ECG signals. However, the automated ECGPsychNet is ready to be tested with more datasets including different races and age groups.

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Dermatopathia Pigmentosa Reticularis

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