05/04/2020-Monday

DIC (disseminated intravascular coagulation)

Uncontrolled fibrin production due to tissue factor activation which can lead to widespread microthrombi. These microthrombi consume coagulation protein such as factor V, VIII, fibrinogen and platelets. Lots of blood clots will start to form which will consume platelet and clotting factors. Without enough platelets circulating the blood, other part of the body start to bleed with even the slightest injuries so paradoxically these patients will have too much clotting on one part of the body and too little clotting on other parts which can complicate treatment. These consumptions will eventually lead to thrombocytopenia which we see with our patient as well. If untreated this can lead to diffuse bleeding from the skin, respiratory tract, and GI and finally to organ ischemia.

DIC usually presents as a complication due to other illnesses. It is almost always secondary to an underlying disorder.

Causes:

Infections (gram negative sepsis most common), rocky mountain fever and viral infections.

Malignancies: Acute myeloid leukemia, lung, GI and prostate malignancies are the most  common.

Severe tissue damage: such as from burns, trauma, liver disease or aortic aneurysms.

Organ destruction: such as pancreatitis or hepatic failure.

More common causes in neonates: Sepsis, Respiratory distress syndrome  asphyxia (suffocation)

Signs/Symptoms:

Bleeding: usually from many sites in the body, venipuncture sites, catheters, or extensive bruising.

Thrombosis: arterial/venous thrombosis, gangrene, or multiple-organ failure (renal, hepatic).

Chest pain and shortness of breath if blood clots form in the blood vessels in your lungs and heart.

Pain, redness, warmth, and swelling in the lower leg if blood clots form in the deep veins of your leg.

Blood in your urine from bleeding in your kidneys or bladder.

Blood in your stools from bleeding in your intestines or stomach.

In neonates, DIC can remain undetected until patient presents with severe hemorrhagic symptoms. Currently there is not a clear or universally accepted criteria to diagnose DIC in neonates. all the DIC reported in children, half occurs during the neonatal period (under 28 days of age).

Signs of bleeding in children will present as petechiae, purpura, ecchymoses or hematomas. If the child continues to bleed after lab work or venipuncture DIC should be in back of your mind.

Diagnosis:

Increase thrombin formation (decreased fibrinogen)

Bleeding- increase PT, PTT and INR and thrombocytopenia.

Increase D-dimer

Peripheral smear- fragmented RBC’s and schistocytes.

Treatment:

First line is to treat the underlying causes.

Careful maintenance of their respiratory and cardiovascular systems, often requiring ventilator assistance and pressor support

Platelet transfusion if platelet count is less than 20,000 microL and not actively bleeding

Fresh frozen plasma if severe bleeding. (replace coagulation factors).

Cryoprecipitate (replaces fibrinogen and factor VIII in patients with severely low levels).

Heparin for thrombosis in some patients with slowly evolving DIC.

Source: https://www.medscape.com/viewarticle/497025_6

Pance Pearl Prep.

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05/06/2020- Wednesday

Recommendations in taking BP in children:

BP should be monitored annually in healthy children.

Early detection of asymptomatic HTN can lead to prevention of short and long term HTN-related morbidity.

BP should be checked in all children who are obese, take medications known to increase BP, have renal disease, history of aortic arch obstruction, aortic coarctation at every health care visit.

Children with history of CDK or diabetes should be evaluated for HTN at every medical encounter.

Causes of HTN in children:

Obesity and a family history of high blood pressure.

Diet has been linked to primary HTN. Non-vegetarian diet and high levels of sodium can lead to increase HTN.

Sleep apnea or other sleeping disorders.

Those children born with low birth weight are at a higher risk for primary HTN later in life than those born at normal birth weight.

Secondary HTN more common than primary HTN in infants and young children. The causes of secondary hypertension vary with age.

Newborn – First year     

Renal artery or venous thrombosis

Renal artery stenosis

Congenital renal abnormalities

Coarctation of the aorta

Renal parenchymal disease

Tumor

First to 6 years

Renal parenchymal disease

Renovascular disease

Coarctation of the aorta

Tumor

Endocrine causes (thyroid disease, hypercalcemia from hyperparathyroidism)

In children 6 years old or older, renal artery stenosis and renal parenchymal disease are the leading cause of diastolic blood pressure being over 90 to mmHg (millimeters of Mercury).

While these conditions may not present with obvious signs and symptoms, studies also indicate that a number of physicians often miss diagnosis HTN in children due to improper assessment of blood pressure. (sometimes they miss it because the child looks totally healthy no signs/symptoms, or just don’t take BP measurements because they think HTN in children is low)

If unidentified, prolonged hypertension can impact many organs if left unresolved. Organs and structures at risk are: heart, kidney, vascular walls, and the nervous system and can lead to more complicated medical conditions.

Symptoms:

Children with hypertension rarely have clinical evidence of the disease. The increased blood pressure is usually detected during a normal examination or during a physical examination for sports participation.

Clinical manifestations of underlying diseases include such things as failure to thrive and failure to grow. Significant elevation of blood pressure can lead to headaches, dizziness, visual changes, nausea and seizures.

If its related to thyroid disease, then check for (weight loss, sweating, flushing, buffalo hump -Cushing’s syndrome, Thyromegaly and edema)

Screening:

All patients- Urinalysis, chemical panel including electrolytes, lipid profile, renal ultrasonography in those less than 6 years old with abnormal urinalysis. TSH to check for thyroid issues, CBC and drug screen in teenagers.

In obese- Hemoglobin A1C, fasting serum glucose, AST, ALT (liver enzymes), fasting lipid panel (for dyslipidemia)

If you suspect HTN is due to cardiac issues such as a left ventricular hypertrophy, order an echocardiogram.

Renal arteriography: severe HTN or failure to control BP with one drug

Treatment:

If HTN due to obesity, a weight loss plan should be established. We can have the patient do physical activities, diet change and behavioral therapies (resist the urge of food). Losing weight of about 10-15 lbs (4-7 kg) is sufficient to achieve a meaningful reduction in BP. Physical activity with increased HR for 30-40 minutes, 3-4x/wk is recommended in lowering BP

If diet and lifestyle management fail to change HTN for 6 months, then we can consider the addition of antihypertensive mediations. Primary medications to consider are: ACEi, CCB, ARB’s or beta blockers.

In CDK or diabetes: ACEI or ARBS as first line.

Patients treated with antihypertensive medications should be seen every 4–6 weeks for dose

adjustments until goal BP is reached, then every 3–4 months.

Patients treated with lifestyle change only should be seen every 3–6 months to assess success of BP

reduction and to reassess need for pharmacologic treatment.

Blood pressure above the 99th percentile or more than 4 SDs above the mean is considered severe, however any BP in the presence of neurological symptoms is an acute emergency and requires urgent attention

Children are less likely to have atherosclerosis and therefore can tolerate sudden drops in BP without the risk of vital organ ischemia, MI or stroke.

Source: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4869180/

https://www.kidney.org/sites/default/files/TrachtmanSlides-PediatricHypertension.pdf

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05/08/2020-Friday

Question:

A 13-month-old child is noted to be at the 25th percentile for weight, the 10th percentile for height, and less than the 5th percentile for head circumference. She was born at term. She was noted to have a small head at birth, to be developmentally delayed throughout her life, and to have required cataract surgery shortly after birth. She currently takes phenobarbital for seizures. Which of the following would most likely explain this child’s small size?

DDX:

1) Congenital cytomegalovirus infection

2) Hyperthyroidism (Graves’ Disease)

3) Down Syndrome

Workup and treatment

1) Congenital cytomegalovirus infection: Infants with congenital CMV can show symptoms such as rash, jaundice, microcephaly (which is seen in our patient), low birth weight (seen in our patient as well), seizures (our patient takes phenobarbital for seizures) and retinitis or eye problems at an early age (which is also seen in our patient as well as she required cataract surgery shortly after birth).

We can diagnose congenital CMV with CMV-DNA in the urine, saliva, or blood right after birth. A negative CMV-IgG serology can exclude congenital infection.  We can also do CMV polymerase chain reaction of saliva or urine to detect the infection. Saliva is usually collected orally by swab and is more convenient to obtain than urine. However, oral swab can sometimes be false positive if baby is being breast-fed so at this time a CMV-PCR via the urine would be best. In some parts of U.S, screening for congenital CMV is mandatory for those infants who are at high-risk of getting such infection.

Recent studies have shown that IV ganciclovir for 6 weeks within 1 month of birth has shown to reduce progression of CMV infection in infants and reduce most of the symptoms associated with it. Another antiviral that you can also give to these infants is valganciclovir (16 mg/kg/dose twice daily for 6 month), which can be as effective as ganciclovir. Patients on antivirals are also recommended to have regular hearing and vision tests.

2) Hyperthyroidism (Graves Disease): can present as weight loss which we see in our patient. Also presents with a few eye problems such as proptosis, exophthalmos, lid lag or vision changes. Although we might not see this exactly on our patient, it still can be a possibility that her cataract surgery could be due to this. To diagnose hyperthyroidism, we can check TSH levels and T4. In a person with primary hyperthyroidism we see decreased TSH levels and increase T4 or T3. We can also do a radioactive uptake scan which can show increase iodine uptake if there is hyperthyroidism. When it comes to management of hyperthyroidism, especially Graves disease, we can do radioactive iodine which is the most common therapy used. It ablates the thyroid within 6-8 weeks. However, this treatment plan is contraindicated in pregnant women and lactating women and I also would not recommend doing this on a 13-month old child as well. Another option is to use thionamides such as methimazole or propylthiouracil. These medications will prevent the synthesis of thyroid hormones and achieve a euthyroid state in about 3-8 weeks. Methimazole is generally preferred more because it has less adverse effects than propylthiouracil. In treating hyperthyroidism, we can also add a beta blocker such as propranolol to improve any side effects of thionamides such as tremors, hypertension, A.fib or tachycardia. When it comes to ophthalmopathy, we can start this patient on glucocorticoids to reduce symptoms. If we were to start this patient on radioactive iodine it would exacerbate ophthalmopathy so one way to avoid this is by starting the patient first on glucocorticoids then initiate radioactive iodine after.

3)Down syndrome: This would be at the bottom of my differential diagnosis. This patient could also have down syndrome which can lead the patient to have multiple symptoms such as failure to thrive is some cases, craniofacial features such as underdevelopment of the head, however our patient is missing a few key features of down syndrome such as flat occiput, epicanthal folds and large tongue. Another reason why down syndrome is at the bottom of my list is because this disorder is mostly diagnosed at birth or during the mother’s pregnancy by looking and the chromosomes. Patients with down syndrome have a trisomy for chromosome 21. You can also diagnose it by doing a Nuchal translucency test to measure a specific area on the back of your baby’s neck. Lastly you can also do a Chorionic villus sampling (CVS) where cells are taken from the placenta and used to analyze the fetal chromosomes.

When it comes to treatment, there is no curative treatment available for Down syndrome, only supportive treatment is available. Patients with down syndrome are at risk for multiple complications such as duodenal atresia which should be treated surgically and also congenital heart diseases such as PDA or atrial septal defect and the definitive diagnosis is surgical closure as well.

Source: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5804742/

PPP

https://www.mayoclinic.org/diseases-conditions/down-syndrome/diagnosis-treatment/drc-20355983

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