3-year-old patient successfully treated for rare fatal disease- familial Hemophagocytic lymphohistiocytosis (HLH) at Fortis Hospital Shalimar Bagh -…

New Delhi: Recently, a 3-year-old patient, Nashra Khan, was brought to Fortis Hospital Shalimar Bagh, where after a thorough medical evaluation she was diagnosed with hemophagocytic lymphohistiocutosis, a rarely occurring hereditary auto-immune condition with a five percent survival rate. A multidisciplinary approach was adopted to treat her, and the case was led by Dr Rahul Bhargava ( Director & Head, Blood Disorder, Hematology |Medical Oncology, Hematology & BMT) and Dr Amrita Chakrabarti (Associate Consultant, Hematology and Bone Marrow Transplant).

On 3-year-old patient successfully treated for rare fatal disease- familial Hemophagocytic lymphohistiocytosis (HLH) at Fortis Hospital Shalimar Baghresentation, the patient had a fever, was suffering from diarrhea, and abdominal swelling. Her medical history indicated that her elder siblings had also reported similar conditions, before passing away. She was evaluated on an urgent basis blood tests, scans, and a bone marrow test which confirmed the diagnosis of familial HLH. She was carrying a very rare mutation in her genes, STXBP2, which (helped confirm this rare genetic disease called Familial HLH).

Dr Rahul Bhargava (Director & Head, Blood Disorder, Hematology | Medical Oncology, Hematology & BMT) said, We commenced her treatment with chemotherapy an attempt to suppress her overactive, abnormal and self-harming immune system. We adopted the HLH protocol 94 and once the initiation phase was completed, we went ahead with a half-matched bone marrow transplant (also called Haploidentical stem cell transplant). The father donated his stem cells, which were collected, stored, and later transplanted into Nashra after treating her with Treosulphan, Fludarabine and TBI conditioning.

Dr Amrita Chakrabarti (Associate Consultant, Hematology and Bone Marrow Transplant) said, Nashra remained critically ill for about 3 months after her transplant. She developed a number of complications such as acute skin and gut graft Vs host disease a condition resulting in development of widespread nasty blisters all over the skin and within the linings of the food pipe, stomach and the intestines. She also developed cytomegalovirus infection- a viral infection causing malfunctioning of the bone marrow and the blood counts to go down. Posterior reversible encephalopathy syndrome was another complication that she developed, causing her to have recurrent seizure episodes.

Dr Bhargava added, We continued to do our best to save the child. She was continuously treated with steroids, immunomodulatory drugs, immunoglobulins, antibiotics, and repeated blood transfusions. Eventually, her overall condition started improving slowly and she was then able to sustain herself without needing blood transfusions. Her bone marrow transplant was a success as her chimerism reports showed a 100% healthy donor type of cells were now being made by her bone marrow. It has now been over a year and a half since her bone marrow transplant, and I am happy to say that Nashra has fully recovered.

Mr Mahipal Bhanot, Zonal Director, Fortis Hospital, Shalimar Bagh said, Fortis Hospital Shalimar Bagh approaches every case in a manner which is beneficial for the patient. Our doctors remain committed to treating every patient even amid the pandemic and we are truly proud of them. This case was extremely challenging, but our doctors ensure that the best possible outcome was achieved. I congratulate them for their commitment to world class patient care.

Familial or inherited HLH is an extremely rare disease passed down by the families due to certain genetic defects. It results in some of the immune system cells called T and NK cells becoming malfunctioning, resulting in their over activity leading to excessive inflammation and tissue destruction. As a result of this malfunction, the immune system begins to damage the patients own organs and tissues, such as the liver, brain, and bone marrow. When the bone marrow gets affected, blood production may be hampered and there may be a suppression of two or more cell components of the blood.

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3-year-old patient successfully treated for rare fatal disease- familial Hemophagocytic lymphohistiocytosis (HLH) at Fortis Hospital Shalimar Bagh -...

Hand weakness: Causes, symptoms, treatment, and seeking help – Medical News Today

Hand weakness can occur due to a variety of conditions, such as carpal tunnel syndrome, arthritis, peripheral neuropathy, and ganglion cysts.

A weakened hand or grip can make everyday tasks much more difficult to complete.

In this article, we discuss some causes of hand weakness, the accompanying symptoms, and the treatment options available.

Ganglion cysts are fluid-filled sacs that typically form on or near a joint or tendon.

According to the American Academy of Orthopaedic Surgeons (AAOS), ganglion cysts are the most frequently forming lump in the hand. They often appear on the back of a persons wrist.

Although harmless, ganglion cysts can sometimes put pressure on the nerves, which can cause muscle weakness, tingling, and pain. Most of the time, these lumps are only a cosmetic issue, and they rarely cause sensory changes or weakness.

Learn more about ganglion cysts here.

Ganglion cysts do not typically require treatment. However, if the cyst is causing pain, a person can receive the following types of treatment:

A person may also take over-the-counter pain medication if the cyst is causing discomfort.

Carpal tunnel syndrome occurs when the carpal tunnel, which is a passageway in the wrist, or the tissues surrounding the flexor tendons swell. This in turn puts pressure on the median nerve.

This pressure can result in pain, tingling, and numbness in the hand.

According to the AAOS, carpal tunnel syndrome may occur due to different factors, such as repetitive hand use, pregnancy, and some health conditions.

Alongside hand weakness, other symptoms include:

A person may also find that they are dropping things due to hand weakness and numbness. This is a late-stage effect of carpal tunnel syndrome.

Usually, the tingling and pain are present for a long time and may become severe before weakness develops.

Learn more about carpal tunnel syndrome here.

Treatment options for carpal tunnel may include:

Learn more about natural and home remedies for carpal tunnel syndrome here.

Cubital tunnel syndrome occurs when the ulnar nerve, which passes through the inside of the elbow, becomes irritated, inflamed, swollen, or compressed.

Although cubital tunnel syndrome may cause pain in the elbow, most of the symptoms occur in the hand.

Symptoms may include:

Learn more about cubital tunnel syndrome here.

According to the AAOS, treatment options include:

Epicondylitis occurs due to damage to the tendons in the forearm. There are two types of epicondylitis: lateral and medial.

Medial epicondylitis, otherwise known as golfers elbow, occurs due to damage to the tendons that bend the wrist toward the palm.

Lateral epicondylitis, also known as tennis elbow, occurs due to damage to the tendons that bend the wrist away from the palm.

Some causes include playing tennis, carrying heavy objects, and frequently using hand tools.

Symptoms of epicondylitis include:

Learn more about lateral epicondylitis here.

Learn more about medial epicondylitis here.

Treatment starts with stopping the activity that caused the condition in the first place and avoiding activities that aggravate the condition.

Treatment options include:

Learn more about exercises for tennis elbow here.

A pinched nerve, or cervical radiculopathy, occurs when something presses against and irritates a nerve located in the neck.

This may stem from changes that occur in the spine as a person ages. It can also occur due to sudden injury that results in a herniated disk.

The pain typically originates in the neck and travels down a persons arm.

Other symptoms include:

Treatment for a pinched nerve includes:

Learn more about how to treat a pinched nerve here.

According to the AAOS, there are over 100 different types of arthritis. Osteoarthritis and rheumatoid arthritis are the most frequent.

Common symptoms of arthritis may include:

Learn more about arthritis in the hands here.

A person should contact their doctor about the best treatment options for them.

A doctor will determine the best treatment based on a persons individual circumstances.

Treatment options may include:

Sarcopenia is the loss of muscle mass. The condition causes weakness and loss of muscle functions, including strength in the hands. It can occur naturally as a person ages.

Other symptoms can include:

Learn more about sarcopenia here.

Treatment options focus on preventing and managing the condition through physical activity, such as resistance training. This can help improve strength and reduce muscle loss.

A person can also increase their protein intake. A 2020 article suggests consuming 2035 grams of protein per meal.

According to the National Institute of Neurological Disorders and Stroke (NINDS), peripheral neuropathy can develop due to conditions that involve damage to the peripheral nerves.

There are over 100 types of peripheral neuropathy, and the symptoms depend on the type of nerves affected.

Neuropathy that can affect the hands includes:

Learn more about peripheral neuropathy here.

If a doctor determines a person has peripheral neuropathy, they will recommend the person continue to treat the underlying condition. A person needs to follow all of the doctors recommendations and let them know if the treatment is not working.

To treat peripheral neuropathy directly, a doctor may recommend braces and splints, as well as pain relief medication.

Learn how to treat peripheral neuropathy naturally here.

Multiple sclerosis (MS) is a condition that affects the central nervous system.

Scientists do not exactly know what causes MS. They believe, however, that it is an autoimmune condition where the immune system attacks the myelin, which insulates the nerves.

According to the NINDS, most people with MS experience muscle weakness in their extremities. They may also experience numbness and prickling sensation.

There is currently no cure for MS. However, treatment typically involves medications and physical therapy.

Learn more about the treatment for MS here.

A stroke occurs as a result of a decrease or blockage in the brains blood supply or when a blood vessel in the brain bursts.

Strokes can cause numbness or weakness in the arm, face, or leg, particularly on one side of the body.

Other symptoms of a stroke include:

The treatment for a stroke depends on the type of stroke a person has experienced.

Learn more about the treatment options for a stroke here.

A person should seek emergency medical attention if they notice any symptoms of a stroke.

A person should consult their doctor if they have sudden weakness in one or both hands.

They should also contact a doctor if the treatment they are currently using is not working. A doctor may help determine a better course of treatment.

A doctor will need to perform a physical examination of the person and discuss their symptoms. They will also ask about other symptoms the person has experienced.

During the examination, the doctor may test a persons strength and pain level.

If needed, the doctor may order additional imaging tests to look at the joints. This may include an X-ray or MRI scan. A doctor can use this to diagnose conditions such as arthritis and assess the amount of damage to the joints.

Hand weakness can result from several different conditions, including a ganglion cyst, carpal tunnel syndrome, and cubital tunnel syndrome.

It can also be a part of many different health conditions that cause neuropathy.

A person will likely experience other symptoms, such as pain, swelling, or limited mobility of the hand or fingers.

A person should contact their doctor about all the symptoms they experience. A doctor can diagnose the issue and recommend treatments based on the underlying condition causing the weakness.

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Hand weakness: Causes, symptoms, treatment, and seeking help - Medical News Today

Gujarat Issues Health Advisory On Fungal Infection With "50% Mortality Rate" – NDTV

Gujarat health advisory lists SOPs against mucormycosis. Wearing masks is one of them.

The Gujarat health department has issued an advisory to doctors and health officials after some COVID-19 patients were recently found infected with mucormycosis, saying it a serious but rare fungal infection and has an overall "mortality rate of 50 per cent".

As per the advisory issued on Monday, mucormycosis infects people with weak immune system and having other existing illnesses.

The Gujarat health department said the advisory is being issued after some coronavirus patients in Ahmedabad and Rajkot were found infected with mucormycosis, which is "serious but rare infection caused by a group of molds called mucormycetes".

"It most commonly affects the sinuses or lungs after inhaling fungal spores from the air, or the skin after the fungus enters through a cut, burn or other type of skin injury. However, it can occur in any part of the body," said the advisory.

It said though the "overall mortality rate is around 50 per cent", early identification and treatment can help.

The advisory said people having diabetes or cancer, or those who underwent organ transplant, stem cell transplant or people having too much iron in the body are at a greater risk of contracting this infection.

The health department made it clear that mucormycosis "cannot spread between people or between people and animals".

"People get mucormycosis through contact with fungal spores in the environment," it said.

Enlisting some preventive measures, like wearing N95 masks, the advisory said these are recommendations and they "have not been proven to prevent mucormycosis".

Apart from wearing the N95 mask, people should avoid direct contact with lot of dust, wear shoes, long pants and gloves while handling soil or moss, and clean skin injuries well with soap and water, said the advisory.

(Except for the headline, this story has not been edited by NDTV staff and is published from a syndicated feed.)

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Gujarat Issues Health Advisory On Fungal Infection With "50% Mortality Rate" - NDTV

A side-by-side comparison of the Pfizer/BioNTech and Moderna vaccines – STAT

In an ideal world, a pandemic vaccine could be delivered in a single shot, so supplies could be stretched to cover a lot of people. It would trigger no side effect more significant than a sore arm. And it would be easy to ship and store.

Unfortunately, this is not an ideal world not yet, anyway.

For now, the good news is that the United States has two Covid-19 vaccines that have been shown to be highly effective.

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What follows is a head-to-head comparison of the ones developed by Pfizer and its partner, BioNTech, and by Moderna. Note that the chances of most individuals being able to pick one or the other are slim to none, especially in the initial rollout. The vaccine available is the one youll get.

The Pfizer emergency use authorization is for people aged 16 and older. Modernas is for people 18 and older, though the company has recently begun testing its vaccine in 12- to 17-year-olds.

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Both vaccines have shown astonishing and essentially equivalent degrees of efficacy, at least in the early stages after vaccination. How they stack up over the long term remains to be seen.

The Pfizer vaccine showed efficacy of 95% at preventing symptomatic Covid infection, measured starting from seven days after the second dose was administered. The vaccine appeared to be more or less equally protective across age groups and racial and ethnic groups.

The Moderna vaccine was 94.1% effective at preventing symptomatic Covid-19, measured starting from 14 days after the second dose. The vaccines efficacy appeared to be slightly lower in people 65 and older, but during a presentation to the Food and Drug Administrations advisory committee the company explained that the numbers could have been influenced by the fact there were few cases in that age group in the trial. The vaccine appeared to be equally effective across different ethnic and racial groups.

Both vaccines seemed to reduce the risk of severe Covid disease. Its not yet known if either prevents asymptomatic infection with the SARS-CoV-2 virus. Nor is it known if vaccinated people can transmit the virus if they do become infected but dont show symptoms.

Both the Moderna and the Pfizer/BioNTech vaccines require two shots: a priming dose, followed by a booster shot. The interval between Moderna doses is 28 days; for the Pfizer vaccine, its 21 days.

Each dose of Pfizers contains 30 micrograms of vaccine. Moderna went with a much larger dose of vaccine, 100 micrograms. It means it is using a little more than three times as much vaccine per person as Pfizer is. And yet, they arent getting better results.

In the vernacular of vaccinology, vaccines that trigger a range of transient side effects in a lot of recipients are known as reactogenic.

Both these vaccines in fact, most if not all the Covid-19 vaccines that have reported data so far fall into the reactogenic category. The Advisory Committee on Immunization Practices, an expert panel that helps the Centers for Disease Control and Prevention set vaccination policies, has advised hospitals they may want to stagger vaccinations among staff for instance, dont vaccinate all the emergency room staff at the same time in case some feel too unwell to work the day after being vaccinated.

The most common side effects are injection site pain, fatigue, headache, muscle pain, and joint pain. Some people in the clinical trials have reported fever. Side effects are more common after the second dose; younger adults, who have more robust immune systems, reported more side effects than older adults.

To be clear: These side effects are a sign of an immune system kicking into gear. They do not signal that the vaccine is unsafe. To date there are no serious, long-term side effects associated with receipt of these vaccines, which will be closely monitored as their use expands.

There have been a handful of reports of people having allergic reactions to the Pfizer vaccine since its rollout began. Those reactions anaphylaxis or a less severe allergic reaction were not seen in the clinical trials. It remains to be seen if similar events will be seen with the Moderna vaccine.

Neither of the vaccines has been tested in these two groups.

Moderna has completed animal studies the FDA demanded of manufacturers; these studies look for evidence that the vaccine might harm the pregnancy or the developing fetus. The company said it saw no such signals.

Pfizer has only interim data from its animal studies, but said it saw no concerning signs either.

The authorizations for both vaccines state there are not enough data to gauge whether they are safe for pregnant or lactating people. FDAs Peter Marks, director of the Center for Biologics Evaluation and Research, said Friday that those who are pregnant should choose whether to be vaccinated after discussing the risks and benefits with their physicians.

Both of these vaccines require an elaborate cold chain, the term used to describe the conditions under which vaccines must be stored during distribution and when they are in the doctors offices, pharmacies, or public health clinics where theyll be administered.

But the Moderna vaccine will be far easier to use than Pfizers. For starters, Modernas must be shipped at -4 Fahrenheit; Pfizers must be shipped and stored at -94 Fahrenheit. The former is the temperature of a regular refrigerator freezer; the latter requires special ultra-cold freezers that need to be topped up with dry ice every five days. Doctors offices do not have ultracold freezers; neighborhood pharmacies dont either.

After thawing, a vial of the Pfizer vaccine must be used within five days; Modernas is stable at fridge temperature for 30 days and at room temperature for 12 hours.

The ultracold storage requirement is not the only challenging aspect of the Pfizer vaccine. The minimum amount of vaccine a location can order is 975 doses. A large teaching hospital might need several of those. But there are plenty of places across the country that dont need 975 doses to vaccinate the people currently eligible for vaccination health workers and nursing home residents. This is the vaccine that needs to be kept at -94 F. The minimum order size will limit the locations in which this vaccine can be used.

The Moderna vaccines minimum order is 100 doses, a much more manageable number.

The Pfizer vaccine is shipped in five-dose vials; Modernas vaccine is shipped in 10-dose vials.

Figuring out how long the protection provided by either of these vaccines will last will take time. Its going to involve periodic blood draws from some volunteers to see what their antibody levels look like, though a decline in antibody levels doesnt necessarily equate to loss of protection.

But a large part of this work will involve watching for reports that people who were immunized are starting to contract Covid in larger numbers, a development that would probably lead to recommendations to give people booster shots at some yet-to-be-determined interval.

Senior Writer, Infectious Disease

Helen covers issues broadly related to infectious diseases, including outbreaks, preparedness, research, and vaccine development.

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A side-by-side comparison of the Pfizer/BioNTech and Moderna vaccines - STAT