Hypoplastic pitted amelogenesis imperfecta linked with Kindler epidermolysis bullosa: JAMA

A recent longitudinal study published in the JAMA Dermatology uncovered a significant association between Kindler epidermolysis bullosa (KEB) and hypoplastic pitted amelogenesis imperfecta which is a rare dental condition characterized by defective enamel formation. This research was conducted from 2003 to 2023 by marking a crucial step forward to understand the comprehensive impact of KEB, a genetic disorder known for causing blistering of the skin and mucous membranes.

Kindler epidermolysis bullosa is a rare genetic condition associated with mutations in the FERMT1 gene, which is crucial for the production of kindlin-1, a protein that plays a key role in the structural stability of the skin. The patients with KEB often suffer from a range of severe orofacial manifestations, including early onset of oral squamous cell carcinoma. However, the potential link between KEB and dental anomalies such as amelogenesis imperfecta remained unexplored until now.

The study spanned for two decades at the Epidermolysis Bullosa Centre, University of Freiburg, Germany and the Special Care Dentistry Clinic, University of Chile, in collaboration with DEBRA Chile. This research focused on a group of 36 patients diagnosed with KEB and analyzed the prevalence and severity of various oral conditions, like enamel defects, gingivitis, periodontal disease and more severe complications such as oral squamous cell carcinoma. Also, all 11 patients assessed for enamel structure abnormalities expressed signs of hypoplastic pitted amelogenesis imperfecta that ranged from generalized to localized enamel pitting.

Beyond enamel defects, this study also observed a high prevalence of other orofacial complications among the KEB patients. 90% of participants suffered from gingivitis and periodontal disease, while more than 70% experienced intraoral lesions and angular cheilitis. The findings also highlighted the presence of gingival overgrowth, microstomia, vestibular obliteration and chronic lip ulcers in a significant number of cases. But, 2 patients developed oral squamous cell carcinoma that cautions the potential for life-threatening outcomes.

This research illuminates the critical need for early and sustained dental care in patients with Kindler epidermolysis bullosa by highlighting hypoplastic pitted amelogenesis imperfecta as a previously unrecognized feature of the disease. The outcomes of this study broaden the understanding of KEB’s systemic impact and additionally emphasize the importance of interdisciplinary care in managing this complex genetic disorder.

Reference:

Krämer, S., Hillebrecht, A. L., Wang, Y., Badea, M.-A., Barrios, J. I., Danescu, S., Fuentes, I., Kartal, D., Klausegger, A., Ponce de León, E., Schilke, R., Yordanova, I., Bloch-Zupan, A., & Has, C. (2024). Orofacial Anomalies in Kindler Epidermolysis Bullosa. In JAMA Dermatology. American Medical Association (AMA). https://doi.org/10.1001/jamadermatol.2024.0065

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Promising Results for Erenumab in Treating Episodic Migraine, shows APPRAISE Trial

In a novel trial, researchers have found that erenumab may be a more effective and tolerable option for
patients with episodic migraine who have not responded well to previous oral
migraine preventive medications (OMPMs). The findings, published in a recent
issue of a prestigious medical journal, shed light on the potential benefits of
early intervention with erenumab for individuals who have previously failed one
or two preventive treatments.

The trial results were published in the journal JAMA Neurology.

Individuals suffering from migraines frequently
find themselves navigating a challenging cycle of trying out various
nonspecific preventive medications. This cycle is often driven by two primary
factors: poor tolerability and inadequate efficacy. As a result, many patients
experience low adherence to their prescribed treatments, ultimately leading to
an increased burden of disease. Hence researchers
conducted a 12-month prospective, multicenter, randomized clinical trial, known
as the APPRAISE trial, involving 621 adult participants from 17 countries.

These individuals, aged 18 years or older, had a history of migraine lasting 12
months or longer and experienced between 4 and fewer than 15 monthly migraine
days (MMDs).

Patients
were randomly assigned to receive either erenumab or OMPMs, with dose
adjustments permitted according to label recommendations. The primary endpoint
of the study was the proportion of patients who completed one year of treatment
and achieved a reduction of 50% or more in MMDs compared to baseline at month
12. Additional
measures assessed in the study encompassed two secondary endpoints: the
cumulative mean change from baseline in monthly migraine days (MMDs) throughout
the treatment duration and the proportion of responders as per the Patients’
Global Impression of Change (PGIC) scale at the 12-month mark for patients
adhering to their initially assigned treatment.


Findings:

The results
of the study were striking.

Significantly
more patients treated with erenumab reached the primary endpoint compared to
those on OMPMs.

Specifically,
56.2% of patients in the erenumab group achieved the desired reduction in MMDs,
while only 16.8% of patients in the OMPM group reached the same milestone.

Furthermore,
erenumab demonstrated higher responder rates on the Patients’ Global Impression
of Change (PGIC) scale, indicating greater overall satisfaction with treatment.


In addition
to improved efficacy, erenumab also exhibited superior tolerability and
adherence compared to OMPMs.

The study findings revealed a noteworthy
reduction in the cumulative average monthly migraine days (MMDs) among patients
receiving erenumab compared to those treated with nonspecific oral migraine
preventive medications (OMPMs).


Fewer
patients in the erenumab group switched medications or discontinued treatment
due to adverse events, highlighting the potential for better patient outcomes
with this specific preventive medication.


The study’s
implications extend beyond clinical practice, offering hope to millions of
individuals worldwide who suffer from episodic migraine.

By identifying a
specific medication that demonstrates superior efficacy, tolerability, and
adherence, researchers have paved the way for more targeted and personalized
treatment approaches in the management of this debilitating condition. 
As further
research continues to unfold, the potential for erenumab to revolutionize the
treatment landscape for episodic migraine appears increasingly promising. With
continued advancements in medical science, individuals affected by this
condition can look forward to a future marked by improved symptom management
and enhanced quality of life.

Further reading: Pozo-Rosich P, Dolezil D, Paemeleire K, et al. Early Use of Erenumab vs Nonspecific Oral Migraine Preventives: The APPRAISE Randomized Clinical Trial. JAMA Neurol. Published online March 25, 2024. doi:10.1001/jamaneurol.2024.0368. 

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Non-invasive electromagnetic phrenic nerve stimulation in ICU Setting Key to Preventing Diaphragm Atrophy: CHEST

Electromagnetic stimulation of the phrenic nerve induces diaphragm contractions, but no coils for clinical use have been available.

In original Research published in CHEST, researchers concluded that Bilateral transcutaneous non-invasive electromagnetic phrenic nerve stimulation (NEPNS) has proven to be a feasible option in the intensive care unit (ICU) setting, potentially offering the advantage of preventing diaphragm atrophy during mechanical ventilation. However, they added that further evaluation of NEPNS ventilation effectiveness is necessary.
This study evaluated the feasibility of using 15-minute bilateral transcutaneous neurostimulation (NEPNS) in patients intubated for at least 72 hours. The intervention group received NEPNS twice daily for 15 min, while the control group received standard care. The primary outcome measure was the time to find the optimal stimulation position, and the secondary endpoint assessed expiratory diaphragm thickness using ultrasound.
Key findings from the study are:
• The revised EU regulation mandated the reapproval of medical devices, prematurely halting the study. Eleven patients were enrolled, including five interventions and six controls.
• The time to find an adequate stimulation position was 23 seconds.
• The intervention twice daily was executed in 87% of cases, and 92% included a next-day catch-up session.
• Ventilation with 3-6ml/kgIBW was achieved in 43 % of stimulations with stimulation only and 62 % with additional pressure support.
• Bilateral NEPNS prevented the decrease in diaphragm thickness until day 10.
This study demonstrated the feasibility of bilateral transcutaneous NEPNS in critical care, with a successful deployment rate of 23 seconds and a high realization rate of over 90% of planned sessions. STIMIT AG, Biel, Switzerland, supported the study.
Reference:
Panelli A, Grimm AM, Krause S, Verfuß MA, Ulm B, Grunow JJ, Bartels HG, Carbon NM, Niederhauser T, Weber-Carstens S, Brochard L, Schaller SJ, Non-invasive electromagnetic phrenic nerve stimulation in critically ill patients – a feasibility study., CHEST (2024), doi https:// doi.org/10.1016/j.chest.2024.02.035.

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NEET 2024 on May 5: Here are Top 10 NIRF-ranked medical colleges

New Delhi- The National Eligibility and Entrance Test for Undergraduate Courses, also known as NEET UG, is scheduled to be held on May 5, 2024, from 2:00 pm to 5:20 pm in offline mode. Every year lakhs of aspiring students eagerly wait for NEET seeking admission into the top-ranked medical colleges in India.

Here is the list of the top 10 medical colleges in India ranked by the National Institutional Ranking Framework (NIRF) with last year’s cut-off percentage:-

NIRF Rank

Name of the Institute

Expected Cutoff

1

All India Institute of Medical Science (AIIMS), New Delhi

98th percentile or above

2

Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh

97th to 98th percentile

3

Christian Medical College (CMC), Vellore

Approximately 95th percentile

4

National Institute of Mental Health and Neuro Sciences (NIMHANS), Bengaluru

Around 94th percentile

5

Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Puducherry

92nd to 93rd percentile

6

Amrita Vishwa Vidyapeetham

Around 91st percentile

7

Sanjay Gandhi Postgraduate Institute of Medical Sciences (SGPGI), Lucknow

Approximately 90th percentile

8

Banaras Hindu University (BHU), Varanasi

89th to 90th percentile

9

Kasturba Medical College, Manipal

Around 88th percentile

10

Sree Chitra Tirunal Institute for Medical Sciences and Technology

87th to 88th percentile

The National Testing Agency (NTA) is going to conduct the NEET UG 2024 exam across the country and in 14 cities across the world and scrapping computerized draw procedure for deciding the rank of joint scorers in the National Eligibility-cum-Entrance Test (NEET) exam, which was introduced earlier this year, the National Testing Agency (NTA) once again opted for its old policy.

The revised Information Bulletin for the NEET-UG 2024 exam, which was uploaded by NTA on its website, specified that NTA will allot ranks to joint scorers based on their performance – the proportion of incorrect and correct answers in Biology, Chemistry and Physics.

When two or more candidates secure the same score in the NEET exam, their ranks are decided in compliance with the tie-breaking policy.

Medical Dialogues had earlier reported that 24 lakh registrations have been done from all over India this year.

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Kerala HC issues notice to Kozhikode hospital on Lesbian couple plea against conversion therapy

Kochi: In a significant legal development, a lesbian couple hailing from Malappuram has approached the Kerala High Court, levying serious allegations against their parents and the hospital for using forced conversion therapy on one of them. Some use conversion therapy without any scientific ground as a means to change someone’s sexual orientation.  

The petitioners, whose identities remain undisclosed, have also implicated a private hospital in Kozhikode, accusing it of subjecting one of them to coercive conversion treatments. Acting on the plea, the bench headed by Justice Devan Ramachandran has issued notices to the respondents, including the private hospital, scheduling the case for hearing after two weeks.

According to the petitioners, their relationship spans over three years. However, complications arose when one of the petitioners’ parents filed a missing person report with the local police. The judicial first-class magistrate court in Malappuram granted the couple permission to stay together. Despite securing permission from the court, the first petitioner was allegedly forcibly taken away by her parents and subjected to conversion therapy against her consent, Times of India

Exhausting all available avenues, the petitioners lodged complaints with various authorities, including the National Medical Commission, the state police chief, the Social Justice Department director, and the Kerala State Mental Health Authority. However, the authorities failed to take any significant action against the accused. Frustrated by the inaction, they turned to the high court for redressal.
Emphasizing the illegality and ethical concerns surrounding conversion therapy, the petitioners advocated for its immediate prohibition, asserting that such practices infringe upon natural justice. Furthermore, they urged the court to mandate the formulation of health guidelines explicitly outlawing all forms of conversion therapy, reports The Daily. 
The case represents a critical juncture in the legal discourse surrounding LGBTQ+ rights and underscores the imperative for comprehensive legal safeguards to protect individuals from coercive and discriminatory practices based on sexual orientation. Section 319 of the Indian Penal Code (IPC) deals with the offence of “voluntarily causing hurt by dangerous weapons or means.” If a person is subjected to physical harm or injury as a result of conversion therapy, this provision can be invoked.
While Section 377 was decriminalized by the Supreme Court of India in 2018, it can still be invoked if someone is coerced into undergoing conversion therapy due to fear of prosecution under this section.

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NMC issues communications on 205 more online applications for starting new PG medical courses, seats increment for 2024-2025

The National Medical Commission (NMC) has issued communications on 205 more online applications for starting new PG medical courses and increase of PG seats for the academic year 2024-2025.

Earlier, the apex medical regulator had sent communications in different phases for more such applications through notices dated 11th March 2024, 12th March 2024, 15th March 2024 and 21st March 2024. These communications have been sent through the email IDs (as mentioned in the online applications) to the concerned medical institutes/colleges for information and necessary action within the stipulated timeline.

For more information, click on the link below:

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MBBS student held for impersonating a candidate in exam for Rs 5 lakh

An MBBS student from Agartala Medical College was recently arrested for impersonating a candidate to write the senior teacher (secondary education) Grade-2 exam-2022.

Allegedly, the medico, who is studying as a first-year MBBS student at the medical college appeared in the science subject paper as a dummy candidate.

For more information, click on the link below:

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Medical AI tool uncovers breast cancer signs in 11 women that doctors overlooked

In a groundbreaking advancement for cancer detection, a medical AI tool named Mia has emerged as a potential game-changer in the fight against breast cancer. Developed by the British firm Kheiron Medical Technologies, Mia showcased its prowess during a mass trial conducted within the UK’s National Health Service (NHS). Covering 10,889 patients, the trial, implemented by NHS Grampian in Scotland, harnessed the power of Microsoft’s Azure cloud network for data analysis.

The efficacy of Mia became evident as it uncovered signs of breast cancer that had eluded human doctors, bringing to light 11 cases that had previously gone unnoticed. These cases included tumors too minuscule for even highly-trained radiologists to detect. Among those whose lives were potentially saved by Mia was Barbara, a resident of Aberdeen, Scotland, whose testimonial underscored the profound impact of the AI tool in catching her cancer at an early stage.

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Medical Superintendent Cannot Hold Additional Charge of HoD: HC Directs Medical College to Comply with NMC Norms

Bengaluru: Stating that a Medical Superintendent of an Autonomous Medical Institution cannot be given an additional charge of the Head of Department, a single-judge bench of the Karnataka High Court recently quashed an official memorandum issued by Shivamogga Institute of Medical Sciences appointing its Medical Superintendent as the in-charge HOD of the ENT Department.

The High Court bench comprising Justice Sachin Shankar Magadum further directed the medical college to comply with the National Medical Commission (NMC) norms regarding the separation of the administrative and clinical roles within the medical college.

Meanwhile, the Medical Superintendent has been directed by the court to refrain from assuming additional clinical responsibilities such as serving as a Head of Department (HoD) contrary to the NMC norms.

The plea was filed by a doctor, who challenged the Office Memorandum dated 31.01.2024 issued by the Medical Institution, through which the Medical Superintendent was appointed as HOD of ENT Department.

It was the grievance of the petitioner that the Medical Superintendent (MS), who is already holding the post of MS of an Institute cannot occupy the position of Head of Department in terms of 3.7 Regulation of National Medical Commission.

Medical Dialogues had earlier reported about Teachers Eligibility Qualifications In Medical Institutions Regulations, 2022, notified by NMC. As per Regulation 3.7, “3.7 The Medical Superintendent of the affiliated teaching hospital shall possess a recognized postgraduate medical degree from a recognized Institution with a minimum of ten years teaching experience as Professor /Associate Professor in the relevant departments of the Hospital, out of which at least five years should be as Professor. Appointment to these posts shall be made on seniority-cum-merit basis. Medical Superintendent shall not occupy the position of the Head of the Department. However, he can head the unit.”

Also Read: Teachers Eligibility Qualifications In Medical Institutions Regulations, 2022

It was claimed that the assignment of dual roles to the concerned doctor, who is not called upon to serve as both the Medical Superintendent and in-charge HOD of the ENT Department raises significant concerns regarding potential conflict of interest, compromised patient care and diminished educational opportunities for students. Therefore, while appointing the MS as the in-charge HOD, the Institute has clearly contravened Regulation 3.7 of NMC which clearly lays an embargo on a Medical Superintendent in occupying the position of Head of Department.

After perusing the concerned NMC regulation, the Court noted that as per NMC norms, the Medical Superintendent cannot be Head of Department of a clinical department. 

“The very object of laying an embargo against dual roles is clearly intended to avoid conflict of interest. The role of Medical Superintendent involves administrative responsibilities, overseeing the overall functioning of the Hospital and ensuring compliance with Regulations. Having respondent No.3 as both an Administrator and Head of Clinical Department could compromise the impartiality and effectiveness of decision making in both the areas. The role of Medical Superintendent in the Hospital typically involves administrative duties such as managing budgets, staffing, facilities and ensuring compliance with regulations and policies. Medical Superintendent is also responsible for the smooth operation of the Hospital as a whole focusing on aspects like infrastructure, finance and over all organizational efficiency,” noted the Court.

“On the other hand, Head of the Department in the Clinical Department such as Surgery, Medicine, Pediatric, ENT, is responsible for overseeing the medical and clinical aspects of that specific department. This includes supervising medical staff, ensuring quality patient care, setting departmental policies and participating in clinical decision making,” it further observed.

Therefore, the court opined that the very object of Regulation 3.7 is intended to see that the same person cannot serve both as Medical Superintendent and HOD of clinical department. The concerned Regulation is also intended to have two separate channels for administrative actions and clinical focus.

As per the Court, the Medical Superintendent’s primary focus is on administrative tasks while the HOD focuses on clinical matters with their separate department. Therefore, balancing both these roles effectively can be challenging as they requires different skill sets and priorities.

“If a same person holds both the posts, a conflict may arise while making decisions that affect both the Hospital as a whole and a specific clinical department. The embargo also addresses the main concern of Medical institution where quality of patient care is a top priority. Separating the roles ensures that HOD can concentrate only on clinical needs of their department including patient care, staff supervision and maintaining high medical standards,” the Court observed.

Further, the High Court bench also opined that

…dual role of Medical Superintendent and Head of Department can potentially affect students in various ways. If HOD is also responsible for administrative duties as a Medical Superintendent, it may detract from his ability to focus on educational responsibilities within the Department. This could impact the quality of teaching, supervision of students and availability of academic support and mentorship. Students often rely on HODs’ for guidance and supervision during clinical rotations and training. If the HODs’ attention is divided between administrative and clinical duties, it could affect the availability and quality of supervision which is crucial for students learning experience and development of clinical skills. Overall, the potential impact on students highlights the importance of maintaining clear roles and responsibilities within medical institutions to ensure that students receive necessary support, supervision and educational opportunities for their training and development as future health care professionals.”

Referring to the institute’s contention to justify their appointment of the Medical Superintendent as the in-charge HOD, the Court expressed its shock and noted,

“The respondent No.1-institution cannot claim that position of Medical Superintendent is not sanctioned as a justification for allowing the Medical Superintendent to hold the post of HOD contrary to 3.7 Regulations of NMC. NMC norms are established to ensure the efficient and effective functioning of health care institutions, uphold standards of patient care and maintain the quality of medical education. These norms often mandate the separation of administrative and clinical roles within health care institutions to avoid conflict of interest and ensure transparency and accountability. If the position of Medical Superintendent is not officially sanctioned, it does not automatically grant permission for the Medical Superintendent to hold an additional charge of HOD. The claim of respondent No.1 that position of Medical Superintendent is not officially sanctioned and therefore, Medical Superintendent is permitted to hold the post of Head of Department cannot be acceded as it is clearly contrary to the above said Regulation of NMC norms.”

Denying to accept the contention by the Medical Institute as a valid reasoning, the Court reiterated that the appointment of the Medical Superintendent as the Head of Department is in contravention of Regulation 3.7, which explicitly states that a Medical Superintendent cannot head a department. It was further observed by the Court that the lack of sanction for a position does not negate the regulations set forth by NMC regarding the qualifications and responsibilities of departmental heads.

With these observations, the bench ordered,

“In the light of findings recorded supra, this Court is of the view that appointment of respondent No.3 who is already holding the post of Medical Superintendent as HOD of ENT is null and void as it violates Regulation 3.7 of NMC Regulations. The Institute is instructed to rectify this violation immediately by appointing a qualified individual with requisite expertise and experience as the Head of the Department of ENT.”

Therefore, the bench declared the Official Memorandum dated 31.01.2024 passed by the medical college in appointing the MS as the in-charge HOD as illegal and accordingly quashed the same.

“The respondent No.1 is hereby directed to comply with NMC norms regarding separation of administrative and clinical roles within respondent No.1-Institution,” ordered the Court, further adding, “The respondent No.3 shall refrain from assuming additional clinical responsibilities such as serving as an HOD of ENT Department contrary to NMC norms.”

Issuing directions to the medical college to revisit the issue relating to the appointment of HOD, the bench ordered,

“The respondent No.1-Institution without deviating from NMC norms shall revisit the issue relating to appointment of HOD and take appropriate action strictly adhering to the established law, regulation and norms of NMC.”

To view the order, click on the link below:

https://medicaldialogues.in/pdf_upload/karnataka-hc–235104.pdf

Also Read: NMC releases Teachers Eligibility Qualifications in Medical Institutions Regulations 2022

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Handing out vapes in UK emergency departments helps smokers quit, study finds

Giving out free e-cigarette starter packs in hospital emergency departments to people who smoke helps more people quit, according to research from the University of East Anglia.

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