Age: 15.0
Sex: F
Vaccine: HPV4, HPV9
Symptoms: Nausea, Nephrolithiasis, Oedema, Oral surgery, Orthostatic hypotension
Clinical narrative: Mast Cell Activation Syndrome; Postural orthostatic tachycardia syndrome; This literature marketed information has been received from the authors in a published article title as stated below and refers to a 15-year-old female patient. Since birth she has "always been having a cold" and has "always been throwing up". Gastroesophageal reflux disease (GERD) was diagnosed in infancy. A possible UTI was treated at 1 year. She also "passed out sometimes from crying too hard". At 6-years-old, she suffered about of pertussis in spite of having received the vaccine on schedule. Alternating diarrhea and constipation emerged early in school. At 10-years-old, she was only at the 5th percentile for weight. Lansoprazole was started for GERD, but this helped only modestly. Menarche came at 12-years, and she was immediately afflicted by dysmenorrhea and menorrhagia. At 13-years-old, upon having to stand for a long time, she suffered a full syncope. At 15-years-old, she was started on an oral contraceptive, which helped control her menstrual symptoms. She was also started on cyproheptadine, which helped stimulate her appetite only while she was actively taking it; stopping it for even just two days would lead to a relapse of many symptoms, gastrointestinal and otherwise. Family history was notable for a mother who had thalassemia minor, anxiety, depression, panic, and environmental allergies, a father who had a seizure disorder, hypertension, fluctuating kidney function, atrial fibrillation during his seizure, obstructive sleep apnea (wears CPAP), easy bruising from preventive aspirin, frequent kidney stones, and a penicillin allergy, a sister who had "borderline renal hypertension" but was otherwise healthy, a paternal grandfather who had had hypertension and had died of kidney failure, a paternal grandmother who had hypertension, diabetes, and hypertrophic cardiomyopathy and had had a breast cancer at 78, a maternal grandfather who had multiple myeloma and hypercholesterolemia and had undergone coronary artery bypass at 65, and a maternal grandmother who had hypercholesterolemia, hypertension, fatty liver disease, frontal lobe dementia since her 50s (and now had Alzheimer's disease), anxiety, depression, and had had uterine cancer in her 70s. The patient denied any history of smoking, significant alcohol use (in fact, it immediately caused her to experience nausea, vomiting, and lightheadedness), or illegal substance use. At the time of her initial evaluation, she listed her current medications as pantoprazole 40 mg once daily, ondansetron 4 mg as needed for nausea, norethisterone (ORTHO MICRONOR) 0.35 mg once daily as an oral contraceptive, and escitalopram 5 mg once daily. She listed her current allergies as sertraline. Outside records indicated a "head in vise" feeling to sertraline, extrapyramidal symptoms to metoclopramide, and reactivities to nickel, gluten, and pepper.  On an unspecified date, At 15-years-old, she received her first dose of unidentified Merck hpv vaccine administered, also reported as Gardasil (Lot #, expiration date, dose, route of administration and anatomical location of vaccine were not reported) for prophylaxis vaccinations on schedule, without any early adverse reactions. The third vaccination was delayed. Three months after the second injection she accidentally tripped, fell down a flight of stairs, and suffered a concussion. More gastrointestinal (GI) symptoms emerged in the next week (more constipation, more abdominal pain, and more vomiting) as well as urinary symptoms (marked frequency). One year after the first Gardasil vaccination she received the third, and within the next month, episodes of orthostatic hypotension, presyncope (idiopathic and at micturition), and tachycardic palpitations emerged. She soon was clinically diagnosed with postural orthostatic tachycardia syndrome (POTs). Heat, prolonged standing, and smoke as well as other odors would trigger flares of assorted symptoms. Bouts of inexplicable abdominal pain required frequent emergency department (ED) visits. A kidney stone was found at one point and required extraction. She reacted to her dormitory room on her first day at college and had to withdraw after one semester due to "continuous nausea and vomiting" and "frequent ED visits". At 19-years-old, she was diagnosed with anxiety but reacted to sertraline with whole-head edema. Extensive evaluations were unrevealing. She reacted with migraine headaches, muscle cramps, and dysfunctional uterine bleeding to fludrocortisone started for POTS. At the time of that initial evaluation, she reported chief complaints of dyspnea, vomiting, tachycardia, and belching. Other symptoms which emerged in time included subjective fevers, flushing, feeling cold much of the time, fatigue (often to the point of exhaustion), malaise, headaches, diffusely migratory aching/pain, diffusely migratory pruritus, unprovoked fluctuations in weight and appetite, irritation of the eyes, tinnitus, sinonasal congestion, coryza, post-nasal drip "all the time", irritation of the mouth, irritation of the throat, proximal dysphagia, dyspnea ("I feel really tight at times in my throat and lungs, but I am told my airway is wide open and my oxygen is at 100%"), palpitations, non-anginal chest discomfort/pain, gastroesophageal reflux, nausea, vomiting, diarrhea alternating with constipation, diffusely migratory abdominal discomfort, urinary frequency, occasional dysuria (she said it would always feel as though she had a UTI, but evaluation for such was always negative; she said she had had only one true UTI in her adolescent/adult life), diffusely migratory weakness, subjective edema about the oral/cervical tissues as described above but almost never any objective edema, diffusely migratory tingling/numbness (typically about the distal extremities), diffusely migratory bilateral cervical adenitis (but not adenopathy), orthostatic and non-orthostatic presyncope, syncope rarely (as detailed above), cognitive dysfunction (particularly memory, concentration, and word-finding), some aberrant dental growth requiring multiple oral surgeries, diffusely migratory rashes (typically patchy macular pruritic erythema), insomnia, frequent waking, hypersomnolence, non-estorative sleep, sleepwalking in childhood, sleep talking, sleep paralysis, night terrors, anxiety, panic, depression, and poor healing in general (principally excessive scarring).  Physical examination at the time of the initial evaluation found an outwardly healthy appearing, trim young woman in no apparent distress, pleasant, cooperative, fully alert and oriented, easily independently ambulatory with no apparent orthostatic behaviors, presenting with her obviously appropriately concerned/supportive parents. Vital signs were unremarkable but for a weight of 101 pounds. Key findings were her comfortable general appearance, HEENT benign, no plethora/pallor/diaphoresis/jaundice/rash/bleeding/bruising, neck supple, no JVD or thyromegaly or carotid/abdominal/renal bruits (but she did have soft bilateral femoral bruits), no palpable adenopathy or tenderness at any of the usual node-bearing sites, clear lungs, regular heart with no adventitious sounds (but the degree of variability in her heart rate during the respiration cycle was a bit more pronounced than usual), abdomen completely benign, no peripheral edema, and an unremarkable neurological exam. No costovertebral angle or spinal column tenderness was evoked on palpation and percussion about these areas. On a light scratch test on the upper back, moderately bright dermatographism (erythroderma only, no hives) quickly emerged and was fully sustained when last checked 10 minutes later.   Laboratory testing for mast cell activation was positive for a 24-hour urinary prostaglandin D2 level elevated at 432 ng (normal 100-280 ng) and a plasma heparin level strongly elevated at 0.17 (units not reported), anti-Factor Xa units/ml (upper normal 0.02). Comprehensive genetic testing for autoinflammatory syndromes was negative.   Trials of various non-sedating H1 blockers and various H2 blockers found fexofenadine and ranitidine served her better than the others, having gained her a broad range of symptomatic improvements at follow-up three months after initial evaluation, including being much more energetic (engaging in more routine activities of daily living) and enjoying complete resolution of her previous daily emesis.  Literature report:   Upon internal review the of Mast Cell Activation Syndrome (MCAs) was considered as medically significant.   This is one of several report from the same literature article.   A copy of the published article is attached as further documentation of the patient's experience.