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Incontinentia Pigmenti Achromians

Incontinentia Pigmenti Achromians is a rare, inherited skin disorder characterized by a distinctive pattern of hypopigmented macules, patches, and streaks, often following the lines of Blaschko.
The condition is caused by genetic mutations in the IKBKG gene, which plays a crucial role in the regulation of inflammatory and apoptotic pathways.
Incontinentia Pigmenti Achromians typically manifests in early childhood, with a characteristic progression through several stages, including inflammatory, verrucous, and hyper- and hypopigmented phases.
While the skin manifestations are the most prominent feature, the disorder can also affect other organ systems, including the central nervous system, teeth, and eyes.
Accurate diagnosis and management of Incontinentia Pigmenti Achromians requires a multidisciplinary approach, involving dermatologists, geneticists, and other healthcare professionals.
Reserach on this condition is ongoing to better understand its pathogenesis and develop more effective treatments.

Most cited protocols related to «Incontinentia Pigmenti Achromians»

The EN-BIRTH study uses quantitative and qualitative methods across four objectives (Table 2). The validity of coverage indicators of selected maternal and newborn interventions as measured by routine facility registers and maternal recall surveys will be assessed by comparison with the “gold standard” of direct observation (Figure 4, panel A). Observation will be undertaken in three clinical settings (Labour/Delivery ward, Operation theatre, and KMC ward/area) by trained clinical observers. Data will be extracted from facility registers and verification of inpatient records carried out for newborns who received antibiotics for presumed infection, and for women who received ACS. Interviews to capture maternal recall will be conducted prior to discharge with all women whose births and/or their newborn’s care were observed or case notes were verified. In addition, barriers and enablers to recording of selected indicators in routine facility registers will be evaluated. Data flow into national HMIS platforms and perceived utility of data will be documented.
Research questions were informed by consultation with many Every Newborn stakeholders [9 ,17 (link)] including WHO-led Measurement Improvement Roadmap meeting [15 ] and EN-BIRTH Expert Advisory Group (listed as author group). More than 60 participants in an EN-BIRTH study design workshop [49 ] provided representation from country partners, national stakeholders, UN agencies, leading academic and professional experts in the field, governmental and non-governmental organisations, clinicians, program managers, other key experts and donors (see Appendix S1 in Online Supplementary Document) and contributed to development of the research protocol (Box 1).
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Publication 2019
Antibiotics Childbirth Donors Gold Incontinentia Pigmenti Achromians Infant, Newborn Infection Inpatient Mental Recall Mothers Obstetric Delivery Patient Discharge Woman
Over a four-week period beginning in January, 2013, a group of 5 experienced MCH program managers and researchers met weekly to adapt an HIV care cascade tool for the pMTCT cascade [17 (link)]. This original tool included quantifying loss to follow-up across the multiple, linked steps in the HIV care cascade, including (1) HIV testing, (2) enrollment at an ART clinic for HIV-infected adults, (3) CD4 testing post enrollment, (4) ART initiation among eligible individuals, and (5) adhering to ART post ART initiation. The PCAT also quantifies the number of women that would complete the entire HIV care cascade if drop-off at each step were eliminated; this maximizing function allows health workers to quickly prioritize steps along the cascade to target for improvement.
In developing the PCAT, initial priorities included 1) introduction of the steps specific to the pMTCT cascade; 2) adaptation of the interface for data entry by frontline health managers using a broadly available program such as Excel®; 3) creation of versions in English, French and Portuguese; 4) ensuring the tool uses only data available through the routine HMIS; and 5) an output design that clearly indicates which individual improvement step would lead to the largest overall efficiency gains across the pMTCT cascade.
Process mapping techniques were used to chart PMTCT cascade steps in five facilities, including ANC attendance, HIV testing and counseling, provision of prophylactic ARVs, safe delivery, safe infant feeding, infant follow-up including HIV testing, and family planning, in order to obtain site-specific knowledge of service delivery [4 (link), 27 ]. Women’s linkages into long-term HIV care and treatment services, including eligibility assessment for cART and cART initiation, were also considered as part of the pMTCT cascade. Study teams worked with staff from the ANC, maternity, postpartum, and at-risk child care settings over a number of days to draw maps of the flow of mother-infant pairs across these services. By working with facility staff to explicitly describe the sequential, linked processes of care delivery at their facility, key steps in the pMTCT cascade were highlighted [28 (link)].
After mapping the pMTCT cascade, study staff conducted discussions with health facility staff to refine the objectives of the PCAT, identify users and beneficiaries, and reach consensus on which steps of the pMTCT cascade should be included based on data availability, data quality, and their importance for achieving effective pMTCT service delivery.
Initial versions of the PCAT (designed in Excel®) were shared and discussed with 27 additional pMTCT managers and frontline nurses, including at the provincial (4 people), district (5 people from 3 districts), and facility levels (18 people from 10 health facilities). The tool was presented in a series of 1–2 hour meetings in which its understandability, usability, and appropriateness were discussed. Over the following six months the PCAT was redesigned based on continued stakeholder feedback, and pilot tested in five health facilities with pMTCT services before its introduction. The iterative development process is described in Table 1.

PCAT tool development timeline

ActivityMonth
123456
Initial planning meetings-researchers/program managersX
Tool adaptation & developmentX
Feedback meetings with program managers & frontline health workersX
Tool revisionsX
Feedback meetings with program managers & frontline health workersX
Tool revisionX
Feedback with program managers & frontline health workersX
Tool introductionX
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Publication 2014
Acclimatization Adult CART protein, human Condoms Delivery of Health Care Eligibility Determination Health Personnel Incontinentia Pigmenti Achromians Infant Long-Term Care Microtubule-Associated Proteins Mothers Nurses Obstetric Delivery Wellness Programs Woman
Target genes for selected miRNAs were predicted by using TargetScan Release 6.0 (http://www.targetscan.org/) [32] (link) and miRanda (http://www.microrna.org/microrna). The target genes that were predicted by both TargetScan (default parameters) and miRanda (Total score> = 145, Total energy< = −10) for each miRNA were further analyzed through IPA (Ingenuity Systems, www.ingenuity.com). The multiple testing corrected P value calculated by Benjamini-Hochberg method (FDR) [28] was used to determine the significance of the predicted function in IPAs. A threshold of FDR <0.05 and molecule number >1 were applied to enrich significant biological functions of each miRNA.
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Publication 2014
Biological Processes Genes Incontinentia Pigmenti Achromians MicroRNAs
The conceptual framework in Figure 2 shows the hypothesized pathways through which DFF could improve utilization and quality of care. The framework was derived from the literature and discussions with stakeholders, and guided data collection and analysis.
Data were collected between October 2007 and March 2008, 2–3 years after the scheme was introduced. Two of the seven districts in Coast Province were purposively sampled to reflect likely diversity of experience with DFF implementation according to managerial views. Their characteristics are summarized in Table 1. It was not possible to assess the quantitative impact on key indicators such as utilization and fees charged because no baseline data had been collected. Historical HMIS data could not be used as a baseline, because of the high frequency of missing or incomplete records, and the recent upgrading of the HMIS system in Coast, which meant that historical and current HMIS data were not directly comparable. We addressed this issue by focusing our quantitative analysis on intermediate/process outcomes that could be easily linked to DFF (Figure 2), while using qualitative methods to explore stakeholder opinions on impact.
The sampling frame included all government health centres and dispensaries eligible to receive DFF.2 A structured survey comprising an interview with the facility in-charge, record reviews and exit interviews was conducted at a sample of 15 facilities in each district, stratified by facility type. All five health centres in Kwale and all four in Tana River were automatically selected, and 10 of the 47 eligible dispensaries in Kwale and 11 of the 25 in Tana River were randomly selected. The in-charge interview assessed facility characteristics and services provided, drug availability, financial and non-financial resources, user fees and community engagement mechanisms. The record review covered utilization, income and expenditure over the period July 2006 to June 2007. Exit interviews were conducted on the facility premises but away from staff and HFC members. We aimed to select a convenience sample of 10 exit interviewees seeking outpatient curative services per facility, obtaining a total of 292 completed questionnaires. The interview covered patient characteristics and diagnosis, user fees paid and awareness of community engagement strategies.
In addition, a subset of six facilities from each district where the in-charge had been in post for at least 1 year was re-visited for in-depth individual interviews with the facility in-charge, and group discussions with a representative range of HFC members. The six facilities were purposively selected to encompass variation in facility type; accessibility to the district headquarters; and performance on indicators from the structured survey (adherence to the user fee policy, activity of the HFCs and completeness of HMIS records). Finally in-depth interviews were conducted with DHMT and PHMT staff, and one of the Provincial Facility Grants Accountants.
Quantitative data were double-entered using Fox-pro D-base IV, MS Access or MS Excel, and imported into STATA version 9 for analysis. Where possible, qualitative interviews were digitally recorded. Discussions were transcribed and imported into N-Vivo 7 for coding and analysis. A coding scheme was developed from the conceptual framework and from reading a sub-set of the transcripts to identify the main themes.
Informed consent was obtained for all interviews, and the study was approved by the Ethical Review Committees of the Kenya Medical Research Institute and the London School of Hygiene and Tropical Medicine.
Publication 2010
Awareness Diagnosis Ethical Review Health Services, Outpatient Incontinentia Pigmenti Achromians Patients Pharmaceutical Preparations Quality of Health Care Reading Frames Rivers Utilization Review
The health facility survey aims to measure the effects of P4P on service availability and provision at the sampled facilities. It is comprised of three sections. In the first section, questions focus on basic service provision within the facility (staffing levels, opening hours, facility management, as well as facility infrastructure). The second section of the survey compiles equipment and drug availability data. The third section captures HMIS data on service utilisation, facility expenditures and revenues for the 12-month period before P4P was implemented (January to December 2010 (at baseline) and the period from January 2011 to December 2012 (at endline). The health facility survey will be administered to the facility in-charge or in his/her absence to a knowledgeable health worker or administrator.
The health worker survey tool aims to measure the effects of P4P on health workers’ working conditions and attitudes towards work at the selected facilities.
The exit interview survey primarily intends to measure the effect of the P4P initiative on a range of subjective and objective indicators of quality of care for targeted and selected non-targeted services. The survey will also examine the effect of P4P on the cost of these services. Respondents eligible for interview include women of reproductive age (aged between 16 to 49 years) attending antenatal or postnatal care, or women with children under-one year of age coming for a preventive check up or an immunisation for the baby. These patients will respond to questions linked to the services targeted by P4P. Patients attending care for non-targeted services will also be interviewed. Non-targeted service users will include: women of reproductive age who are not pregnant, or children under five years of age accompanied by a woman of reproductive age, reporting with fever and no cough (as a proxy for malaria), or fever and cough (as a proxy for acute respiratory infection – ARI), or diarrhoea. These conditions were chosen as they were the three most significant conditions reported at outpatient departments in Tanzania in 2009.
A survey of women who had delivered within the previous 12 months will also be carried out. The women’s survey addresses the effects of P4P on service use during pregnancy, place of delivery, birth weight and postpartum care and care for the newborn as well as related costs and service satisfaction. Household socioeconomic status is also measured in this survey. The core indicators for each of the surveys are shown in Table 1.
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Publication 2013
Administrators Birth Weight Child Cough Diarrhea Fever Health Personnel Households Immunization Incontinentia Pigmenti Achromians Infant Infant, Newborn Malaria Mothers Obstetric Delivery Outpatients Patients Pharmaceutical Preparations Postnatal Care Pregnancy Quality of Health Care Reproduction Respiratory Tract Infections Satisfaction Woman Workers

Most recents protocols related to «Incontinentia Pigmenti Achromians»

The data extracted from the HMIS was entered directly into an EpiCollect5 application (Wellcome Sanger Institute, Cambridge, UK), a mobile phone-based data capture tool. Stata version 12 (StataCorp LP, College Station, TX, USA) was used for analysis. The frequency and percentage per nine selected PMCIs were used to summarize the details of type of registration and awareness-generation activities conducted by the PMCI. Using the aggregate data on total population from the census of 2012 and the ‘number of registered’ persons derived from the HMIS registration database, the below indicators were deduced to describe the extent of registration in the selected PMCIs and their GN divisions:

The percentage of the total population registered in the PMCI: the numerator is all individuals registered until censor date and the denominator is total population in the PMCI area as per the 2012 census of Sri Lanka;

The monthly trend in percentage of the total registered per PMCI from June 2019 to June 2021: The total percentage registered at the end of each month is calculated with the total number of individuals registered until the end of each month as the numerator and the total population in the PMCI area as per the 2012 census of Sri Lanka as the denominator. Assuming a similar trend (linear) we projected the percentage of the total that would be registered by December 2021, December 2022, and December 2023 (end of project). For this analysis only eight PMCIs which initiated registration since June 2019 were included;

The percentage of the individuals aged ≥35 years registered in the PMCI: The numerator is individuals aged ≥35 years registered until censor date and the denominator is the total number of individuals aged ≥35 years in the PMCI area as per the 2012 census of Sri Lanka. The age cut off of 35 years was chosen as the PSSP had a mandate for screening individuals aged ≥35 years for NCD risk factors;

The percentage of males among all the individuals registered in the PMCI: the percentage was calculated with the total number of males registered in the PMCI as the numerator and the total number of individuals registered in the PMCI as the denominator;

The median (IQR) percentage of the total population registered in the GN divisions of the selected PMCIs: The percentage was calculated with individuals registered from each GN division as numerator and the total population in the respective GN division as per the 2012 census of Sri Lanka as denominator. The median (IQR) of the percentages calculated for each GN division was deduced.

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Publication 2023
Awareness Incontinentia Pigmenti Achromians Males
The most important component of the empanelment process is the registration and issue of a PHN to each individual in the catchment population identified for the PMCI. The demographic details of the individuals (age, gender, and GN division) are entered into the online HMIS, which autogenerates a PHN. A PHN is a unique number assigned for each individual which can be used to link the individual with their health record. Those issued a PHN are considered to be successfully registered. Also, the individuals are expected to be educated on the importance of their PHN and the requirement of using this number at all interactions with the health system irrespective of level of care.
First, public awareness campaigns were conducted involving GNs, members of friends of facility committees (hospital management committee with community members for community engagement) and other community leaders, and the people were mobilized to the PMCIs for registration. People visiting the PMCIs were registered (passive registration). To improve the coverage, the PMCIs were encouraged to adopt opportunistic (registration of people visiting PMCI for treatment) registration, conduct outreach registration camps in the GN divisions, establish night/evening clinics for registration of the employed population, and also try active registration through house-to-house visits by healthcare workers (HCWs) or volunteers. The medical officer in-charge of the PMCI and staff nurse trained on the PSSP activities are primarily responsible for ensuring registration in the catchment area. Data entry operators (DEOs), if present in the facility, help with registration through the online HMIS.
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Publication 2023
Awareness Committee Members Friend Gender Health Personnel Hospital Administration Incontinentia Pigmenti Achromians Nurses Training Activities Voluntary Workers
Trained research assistants used a structured proforma (Annex-S1) to collect information from the medical officer and/or nursing staff regarding the GN divisions identified and the population assigned to the PMCI. The information on the various activities conducted to generate awareness about empanelment to create demand for registration and the approach used for registration in the PMCI were collected.
The aggregate data on the total population (as per 2012 census of Sri Lanka) in the allotted PMCIs, stratified by assigned GN divisions and age (less than 35 years and ≥35 years), were obtained from the PMU of the PSSP. The number of individuals registered (stratified by GN divisions, age groups, and gender) was extracted from the electronic database of the registration module of HMIS. During July 2021, we extracted the data on registration from April 2019 to June 2021.
Registration included collection and documentation of demographic details in the registration module of HMIS, auto-generation of the PHN through HMIS, screening for NCD risk (in those ≥35 years or increased risk of NCD) and issuing of paper-based PHR to the registered individual. We collected information from the medical officer/staff nurse on the extent of issuing PHR to those registered (not issued/partially issued/issued to all registered).
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Publication 2023
Age Groups Awareness Incontinentia Pigmenti Achromians Medical Staff Nurses Nursing Staff
We selected nine PMCIs out of the 63 PMCIs in Sri Lanka. To represent the 2:1 rural-urban population composition of the country, we used simple random sampling (computer generated numbers) to select six PMCIs from all PMCIs in rural areas and three PMCIs from all PMCIs in urban areas. The nine PMCIs also represent the nine provinces of the country. In each PMCI, we included all the individuals whose demographic details were documented electronically on HMIS and provided with their unique personal health numbers from June 2019 (implementation of PSSP) to mid-June 2021. These individuals were considered as successfully “registered”.
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Publication 2023
Incontinentia Pigmenti Achromians Urban Population
In this work, the Adsorptive Stripping Square Wave Voltammetry (ASSWV) method was applied to detect the concentration of the HMIs. The testing well was filled with acetic acid buffer first. Bi(NO3)3 was then added to form an alloy with the HMIs and to facilitate the deposition process. After adding Pb(II) and Cd(II) into the mixing solution, the electrochemical program of the CHI work station was operated. Three steps were involved in the electrochemical detection process of the HMIs. First, the preconditioning process was operated at +0.2 V for 100 s to clean the electrode surface. Then, the electrochemical deposition of the HMIs was performed at −1.0 V for 160 s; during the first 150 s, the process was operated with stirring and then followed with an equilibration period of 10 s. Finally, the Square Wave Voltammetry (SWV) method was used for the stripping determination of the HMIs.
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Publication 2023
Acetic Acid Adsorption Alloys Buffers Incontinentia Pigmenti Achromians

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More about "Incontinentia Pigmenti Achromians"

Incontinentia Pigmenti Achromians (IPA) is a rare, inherited skin disorder characterized by a distinctive pattern of hypopigmented macules, patches, and streaks, often following the lines of Blaschko.
This condition is caused by genetic mutations in the IKBKG gene, which plays a crucial role in regulating inflammatory and apoptotic pathways.
IPA typically manifests in early childhood, with a characteristic progression through several stages, including inflammatory, verrucous, and hyper- and hypopigmented phases.
While the skin manifestations are the most prominent feature, IPA can also affect other organ systems, such as the central nervous system, teeth, and eyes.
Accurate diagnosis and management of IPA requires a multidisciplinary approach, involving dermatologists, geneticists, and other healthcare professionals.
Ongoing research on this condition aims to better understand its pathogenesis and develop more effective treatments.
IPA is sometimes referred to as Incontinentia Pigmenti Achromians, Incontinentia Pigmenti Acromians, or IP Achromians.
IPA can be associated with other medical conditions, such as those related to the Zn2+ signaling pathway (e.g., Stata 14, SU8010, Stata 13, ZnCl2, Stata/MP v15.1) or the use of certain dyes and stains (e.g., Brilliant Blue FCF, CoCl2·6H2O, SPSS v24, SPSS version 17.0, Sulforhodamine B).
A multidisciplinary approach, including collaboration with specialists in these areas, may be beneficial for comprehensive patient care and research on IPA.