HI (HARLEQUIN ICHTHYOSIS) BABY

2021 ◽  
pp. 8-11
Author(s):  
Dipankar Maiti ◽  
Shreyasi Hui ◽  
Triyasha Adhikary ◽  
Soumik Banerjee
Keyword(s):  
Continuous Monitoring ◽  
Eye Drops ◽  
Rare Type ◽  
Early Intubation ◽  
Increased Risk ◽  
Harlequin Ichthyosis ◽  
Free Environment ◽  
Lipid Transporter ◽  
Scaly Skin ◽  
Electrolyte Abnormalities

HI is a very rare type of genetical abnormality but can be seen and scared by seeing various social media footages. It is associated with deletion and truncation mutations of a keratinocyte lipid transporter. Harlequin disorder is categorized by diffuse epidermal hyperkeratinization along with defective desquamation. During birth, the HI phenotype is conspicuous with thick hyperkeratotic plate-like scales with deep dermal ssures, severe ectropion and eclabium, among other ndings. In this ichthyosis marked eclabium and ectropion are present secondary to the taut as well as in the unyielding skin. The ears may be inattentive, absent or poorly developed. Even the arms, feet, and digits have exion contractures and may be hypoplastic. The skin wall is harshly compromised, leading to undue water loss, causes electrolyte abnormalities, temperature alteration and an increased risk of deadly infection. Some of the primary treatments are smearing retinoids application for shedding the hard and scaly skin, topical antibiotics application can prevent infection, insertion an ET tube in the airway to assistance with breathing, applying lubricating eye drops or protective devices on the eyes. Following ongoing treatment like humidied incubator (for premature infants), continuous monitoring of TPR and SpO2, early intubation(optional), frequent cultures of the skin should be taken for lab testing, monitoring serum electrolyte levels, maintaining a germ-free environment to evade infection etc.

Tobacco-Free Environment: An Imperative for the Health of Children and Adolescents

PEDIATRICS ◽  
1994 ◽  
Vol 93 (5) ◽  
pp. 866-868
Author(s):  
Keyword(s):  
Children And Adolescents ◽  
Tobacco Use ◽  
Behavioral Problems ◽  
Health Hazard ◽  
Well Being ◽  
Fetal Outcome ◽  
Medical Problems ◽  
Increased Risk ◽  
Youth At Risk ◽  
Free Environment

1. Tobacco is a major health hazard to children and adolescents. 2. Maternal use of tobacco has significant adverse effects on pregnancy and fetal outcome. 3. Exposure to ETS has major effects on the health and psychosocial well-being of children. 4. Tobacco use by children is associated with chronic and recurrent medical problems as well as with increased risk for significant morbidity and mortality. Furthermore, tobacco use may be a marker for youth at risk for other behavioral problems. 5. Nicotine is an addicting substance and may serve as a gateway drug for the subsequent use of illicit substances. 6. Tobacco advertising is appealing to young people and may have a powerful effect in influencing them to begin tobacco experimentation. 7. A tobacco-free environment is an imperative for the health of children and adolescents.

Dry skin disorders

2020 ◽  
pp. 403-416
Keyword(s):  
Clinical Sign ◽  
Underlying Mechanism ◽  
Skin Disorders ◽  
Dry Skin ◽  
Netherton Syndrome ◽  
Collodion Baby ◽  
Harlequin Ichthyosis ◽  
The Common ◽  
Scaly Skin ◽  
Recessive Ichthyosis

This chapter on dry skin disorders describes a range of mainly genetic dermatoses whose predominant feature is dry scaly skin. It excludes conditions such as eczema where dry skin is a secondary clinical sign. The underlying mechanism of dry scaly skin is briefly discussed and an algorithm points to possible diagnoses. The focus is on the genetic ichthyoses, defined as congenital, lifelong, generalized dry and scaly skin. The common ichthyoses are non-inflammatory (ichthyosis vulgaris and X-linked recessive ichthyosis) but there are several inflammatory ichthyoses, some associated with fragile skin (blistering) and several with multisystem complications, such as Netherton syndrome. Salient features of each are reviewed with illustrations of selected cases and management is outlined. Two important neonatal presentations, collodion baby and harlequin ichthyosis, are described.

scholarly journals Anomalous Origin of the Coronary Arteries: An Account of Six Cases

2013 ◽  
Vol 52 (191) ◽  
Author(s):  
Laxman Dubey
Keyword(s):  
Heart Failure ◽  
Coronary Artery ◽  
Angina Pectoris ◽  
Coronary Angiography ◽  
Coronary Arteries ◽  
Incidental Finding ◽  
Anomalous Origin ◽  
Rare Type ◽  
Increased Risk ◽  
Low Incidence

Anomalous origin of coronary arteries is a rare type of congenital anomalies with a low incidence. Itis considered an incidental finding without clinical significance, however, these abnormalities maybe responsible for angina pectoris, heart failure, arrhythmias and increased risk of sudden death. Inthis paper, six cases that were diagnosed with anomalous origin of coronary arteries confirmed bycoronary angiography has been described and also briefly reviewed the literature concerning currentviews and therapy of this abnormality._______________________________________________________________________________________Keywords: anomalous origin of coronary artery; coronary angiography; incidence.

scholarly journals A Systematic Review of Flurbiprofen 8.75 mg Dose and Risk of Haemorrhagic Events

2021 ◽  
Vol 12 ◽  
Author(s):  
Sandeep Dhanda ◽  
Alison Evans ◽  
Debabrata Roy ◽  
Vicki Osborne ◽  
Adam Townsley ◽  
...  
Keyword(s):  
Systematic Review ◽  
Data Extraction ◽  
Symptomatic Relief ◽  
Placebo Treatment ◽  
Eye Drops ◽  
Medicinal Products ◽  
Increased Risk ◽  
Inflammatory Drugs ◽  
Potential Interactions ◽  
Risk Ratios

Oral non-steroidal anti-inflammatory drugs (NSAIDs) are known to be associated with an increased risk of bleeding. The NSAID, flurbiprofen, in the form of 8.75 mg lozenge or oromucosal spray is indicated for the symptomatic relief of sore throat. Despite the low dose as compared to alternative flurbiprofen preparations, concerns have been raised regarding its safety in terms of haemorrhagic events. This systematic review was conducted to identify existing evidence on the risk of haemorrhagic events with flurbiprofen 8.75 mg dose (any formulation), particularly where this may be due to potential interactions with other medicinal products. The systematic review examined studies reporting haemorrhagic events in patients receiving flurbiprofen 8.75 mg dose. Six individual electronic databases were searched up to 28th April 2020. Records were initially screened for relevance followed by further review of potentially eligible studies. Data extraction was performed for eligible studies and risk of bias in studies was assessed. The search strategy identified 1093 individual records. Of these, 1038 records were excluded after initial review; the majority of these records related to flurbiprofen in alternative formulations with alternative doses (e.g., eye drops, skin patches, oral tablets) thus were not considered relevant for further review. The 55 remaining records related to flurbiprofen 8.75 mg dose (any formulation) or flurbiprofen lozenge/oromucosal spray where the dose was not specified. After further review, 52 of these records were not considered eligible. Thus, only three records were included in this systematic review. The three studies reported a total of five haemorrhagic events in patients taking flurbiprofen 8.75 mg lozenge; the corresponding risk in each of the studies was 8.33, 1.98 and 1.96%. Where possible, comparison of flurbiprofen 8.75 mg lozenge to placebo produced risk ratios of 0.96 (95% CI 0.07, 13.25) and 2.00 (95% CI 0.10, 118.0). This systematic review found limited evidence on the risk of haemorrhagic events with flurbiprofen when used at a dose of 8.75 mg. Counts were low across all studies and results comparing flurbiprofen and placebo treatment arms were non-significant. However, scarcity of studies and low certainty of evidence for the outcome of haemorrhagic events limits the conclusions of this systematic review.

scholarly journals Association between Ophthalmic Timolol and Hospitalisation for Bradycardia

2015 ◽  
Vol 2015 ◽  
pp. 1-6 ◽  
Author(s):  
Nicole L. Pratt ◽  
Emmae N. Ramsay ◽  
Lisa M. Kalisch Ellett ◽  
Tuan A. Nguyen ◽  
Elizabeth E. Roughead
Keyword(s):  
Beta Blocker ◽  
Case Series ◽  
Cardiovascular Effects ◽  
Eye Drops ◽  
Continuous Exposure ◽  
Case Series Study ◽  
Increased Risk ◽  
Risk Period ◽  
Series Study ◽  
Remaining Time

Introduction. Ophthalmic timolol, a topical nonselective beta-blocker, has the potential to be absorbed systemically which may cause adverse cardiovascular effects. This study was conducted to determine whether initiation of ophthalmic timolol was associated with an increased risk of hospitalisation for bradycardia.Materials and Methods. A self-controlled case-series study was undertaken in patients who were hospitalised for bradycardia and were exposed to timolol. Person-time after timolol initiation was partitioned into risk periods: 1–30 days, 31–180 days, and >180 days. A 30-day risk period prior to initiating timolol was also included. All remaining time was considered unexposed.Results. There were 6,373 patients with at least one hospitalisation for bradycardia during the study period; 267 were exposed to timolol. Risk of bradycardia was significantly increased in the 31–180 days after timolol initiation (incidence rate ratio (IRR) = 1.93; 95% confidence interval (CI) 1.00–1.87). No increased risk was observed in the first 30 days or beyond 180 days of continuous exposure (IRR = 1.40; 95% CI 0.87–2.26 and IRR = 1.21; 95% CI 0.64–2.31, resp.).Conclusion. Bradycardia is a potential adverse event following timolol initiation. Practitioners should consider patient history before choosing a glaucoma regime and closely monitor patients after treatment initiation with topical nonselective beta-blocker eye drops.

scholarly journals Successful Treatment of Secondary Aortoenteric Fistula with a Special Graft

2016 ◽  
Vol 2016 ◽  
pp. 1-3 ◽  
Author(s):  
Ömer Faruk Çiçek ◽  
Mustafa Cüneyt Çiçek ◽  
Ersin Kadiroğulları ◽  
Alper Uzun ◽  
Mahmut Ulaş
Keyword(s):  
Graft Infection ◽  
Good Alternative ◽  
Aortoenteric Fistula ◽  
Graft Replacement ◽  
Rare Type ◽  
Increased Risk ◽  
Left Limb ◽  
Life Threatening ◽  
Left Inguinal Region

Aortoenteric fistula is an uncommon but life-threatening cause of gastrointestinal blood loss. We report a case of a 70-year-old man who presented to the emergency department with an episode of melena and infection in the left inguinal region. Diagnosis of secondary aortoenteric fistula was made between the left limb of the aortobifemoral graft and the descending colon. We performed excision of the infected graft and in situ silver acetate coating of prosthetic vascular graft replacement (aortoleft femoral) on the patient. This study reports a rare type of secondary aortoenteric fistula to the left colon, and it describes an unusual and successful surgical treatment. Antimicrobial coating of prosthetic vascular grafts may be a good alternative in the presence of graft infection associated with aortoenteric fistula because in situ grafts may carry an increased risk of reinfection.

scholarly journals Evaluating the Effect of a Neonatal Care Bundle for the Prevention of Intraventricular Hemorrhage in Preterm Infants

Children ◽  
2021 ◽  
Vol 8 (4) ◽  
pp. 257
Author(s):  
Maximilian Gross ◽  
Corinna Engel ◽  
Andreas Trotter
Keyword(s):  
Risk Factors ◽  
Preterm Infants ◽  
Nursing Care ◽  
Intraventricular Hemorrhage ◽  
Neonatal Intensive Care ◽  
Patient Positioning ◽  
Neonatal Care ◽  
Care Bundle ◽  
Early Intubation ◽  
Increased Risk

Germinal matrix intraventricular hemorrhage (IVH) remains a severe and common complication in preterm infants. A neonatal care bundle (NCB) was implemented as an in-house guideline at a tertiary neonatal intensive care unit to reduce the incidence of IVH in preterm infants. The NCB was applied either to preterm infants <1250 g birth weight or <30 weeks gestational age or both, and standardized patient positioning, nursing care, and medical procedures within the first week of life. A retrospective cohort study was performed to investigate the effect of the NCB and other known risk factors on the occurrence and severity of IVH. Data from 229 preterm infants were analyzed. The rate of IVH was 26.2% before and 27.1% after implementing the NCB. The NCB was associated neither with reducing the overall rate of IVH (odds ratio (OR) 1.02; 95% confidence interval (CI) 0.57–1.84; p = 0.94) nor with severe IVH (OR 1.0; 95% CI 0.67–1.55; p = 0.92). After adjustment for group differences and other influencing factors, amnion infection syndrome and early intubation were associated with an increased risk for IVH. An NCB focusing on patient positioning, nursing care, and medical interventions had no impact on IVH in preterm infants. Known risk factors for IVH were confirmed.

scholarly journals Pattern of Electrolyte Abnormalities in Preterm Low Birth Weight Neonates

2021 ◽  
Vol 29 (2) ◽  
pp. 145-148
Author(s):  
Md Saiful Islam ◽  
Manisha Banerjee ◽  
Tafazzal Hossain Khan ◽  
Chandan Kumar Shaha ◽  
Md Zahir Uddin ◽  
...  
Keyword(s):  
Birth Weight ◽  
Low Birth Weight ◽  
Rural Areas ◽  
Neonatal Outcome ◽  
Close Monitoring ◽  
College Hospital ◽  
Cross Sectional ◽  
Medical College ◽  
Increased Risk ◽  
Electrolyte Abnormalities

Prematurity and low birth weight contributes to 27.8% of neonatal deaths in rural areas of Bangladesh. Fluid, electrolyte and metabolic abnormalities are the commonest derangements encountered in preterm infants due to their renal immaturity and relatively immature skin. Premature infant are at increased risk of developing dehydration or overhydration4. Therefore, high index of suspicion, prompt recognition and thorough understanding of common electrolyte abnormalities are necessary to improve neonatal outcome. Appropriate fluid and electrolyte management is essential for better neonatal outcome. Objectives: To identify the serum electrolytes abnormalities in preterm low birth weight neonates. Methodology: It was a cross- sectional study and carried out in the Department of Neonatology, Dhaka Medical College Hospital, Dhaka between January 2017 to August 2017. Information was collected who gave consent and participated in the study willingly. The sample size was 50. Patients admitted in the above mentioned hospital and after meeting the inclusion and exclusion criteria a simple random sampling technique was applied for selecting the study subjects. Results: Fifty preterm LBW neonates fulfilling the inclusion criteria were studied during this study period. Abnormal electrolytes were documented in 20(40%) out of 50 preterm LBW neonates and electrolyte status was normal in 30(60.0%) cases. Of 20 neonates who had abnormal electrolytes, hyperkalemia was the predominant electrolyte abnormality found in 8(16.0%) neonates, hyponatremia was found in 7(14.0%), hypokalemia in 3(6.0%) and hypernatremia 2(4.0%). Conclusion: Electrolyte abnormalities are common in preterm LBW neonates. So, identification of electrolyte abnormalities and proper management of fluid and electrolytes and close monitoring are important. J Dhaka Medical College, Vol. 29, No.2, October, 2020, Page 145-148

scholarly journals Association of Serum Creatinine and Electrolyte Abnormalities in Preterm Low Birth Weight Neonates

2017 ◽  
Vol 26 (Number 1) ◽  
pp. 15-20
Author(s):  
Md. S Islam ◽  
M Bhuiyan ◽  
A S Chowdhury ◽  
ATM Rafique ◽  
M Afrin ◽  
...  
Keyword(s):  
Renal Failure ◽  
Renal Function ◽  
Birth Weight ◽  
Low Birth Weight ◽  
Renal Impairment ◽  
Serum Creatinine ◽  
Preterm Neonates ◽  
Close Monitoring ◽  
Increased Risk ◽  
Electrolyte Abnormalities

Introduction: Being born prematurely is a threat to survival and the subsequent quality of life ICDDR,B Health and Science Bulletin published in March 2006 reported that prematurity and low birth weight contributes to 27.8% of neonatal deaths in rural areas of Bangladesh Premature infant are at increased risk of developing dehydration or over hydration. Therefore, high index of suspicion, prompt recognition and thorough understanding of common electrolyte abnormalities are necessary to improve neonatal outcome. The investigation of renal function in pretenn neonate is complicated because of continuing renal development, rise in creatinine is transient and may not be clinically significcmt. Serum creatinine is most widely used marker of renal function in adults and children but its validity as a marker of GFR/ renal function is doubtful a few studies have been conducted on assessment of renal function and electrolytes in the context of prematurity in Bangladesh. But it seems to be essential for immediate management for planning appropriate fluid and electrolyte therapy and thereby for improved outcome. Information was collected who gave consent and participated in the study willingly. The sample size was 50. Duration of data collection was approximately 6 (Six) months.Patients admitted to the Holy Family Red Crescent Medical College and hospital and after meeting the inclusion and exclusion criteria a simple random sampling technique was applied for selecting the sample patients. Total 50 pretenn LBW neonates fulfilling the inclusion criteria were studied during this study period. Mean creatinine level was .82 mmoKrange was 0.40-1.90 mg/d1. Abnormal electrolytes were documented in 20(40%) pretenn LBW neonates of which hyperkalemia was the predominant electrolyte abnormality found in 8(16.0%) neonates, hyponatremia was found in 7 (14.0%), hypokalemia in 3 (6.0%) and hypernatremia 2 (4.0%). In the present study 20 of preterm LBW babies have electrolyte abnormalities. Hyperkalemia was found in 8(16.0%) babies in this study from above findings it is evident that prematurity causes transient renal impairment, in preterm neonates which is inversely related to gestational age. Renal impairment should be suspected if the serum creatinine rises or fails to show normal post-natal fall. It was observed that electrolyte abnormalities are common in preterm LBW neonates and transient renal failure also occurs in a large number of preterm LBW babies. So, identification of renal failure and associated electrolyte abnormalities and proper management of fluid and electrolytes and close monitoring are important.

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