By T. Ben. Massachusetts School of Professional Psychology.
Although he has only had 4 days of fever buy cheap vytorin 30 mg on-line, the erythema of the lips and erythema of the palms and soles are two findings that are not commonly seen in other illnesses buy vytorin 20mg fast delivery. Diagnosis generic vytorin 20mg mastercard, treatment, and long-term management of Kawasaki disease: a statement for health professionals from the Committee on Rheumatic Fever, Endocarditis and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association. He denies having a sore throat, upper respiratory infection symptoms, gastrointestinal distress, change in appetite, or fever. His immuniza- tions are current, he has no significant past medical history, and he has been developing normally per his mother. His weight, however, has fallen from the 25th percentile to the 5th percentile, and he has been hospitalized on three occasions in the last year with pneumonia or dehydration. The patient is afebrile today, but his examination is notable for severe gingivitis, bilateral cervical and axillary lymphadenopathy, exudates on his buccal mucosa, and hepatomegaly. Considerations Recurring infections in this patient presenting with oral lesions, weight loss, and lymphadenopathy are concerning for immune system dysfunction. Additional patient and family histories and selected initial laboratory tests will aid in diagnosis and help guide management. Clinicians should inquire about perina- tal history, growth and development, and past illnesses. Family history includes parental health concerns (unexplained weight loss, growth failure, or develop- mental delay in siblings) and recurring or atypical infection in immediate family members. A focused physical examination should then be performed to identify signs consistent with immunosuppression (wasting, generalized lymphadenopa- thy, and organomegaly). Primary (syndromic) immunodeficiency is due to a genetic defect, either inherited or related to de novo gene mutation. Most are humoral in origin or characterized by both humoral and cellular dysfunction (severe combined immu- nodeficiency). Some arise due to congenital malformations that affect proper development of the immune system (thymic dysgenesis in DiGeorge syndrome). Other primary immunodeficiencies include phagocytic cell deficiency (chronic granulomatous disease due to impaired respiratory burst), complement deficiency (autoimmune disease or serious bacterial infection due to C2 deficiency), and neu- trophil dysfunction (autosomal-recessive leukocyte adhesion deficiency). Approximately 75% of pediatric cases diagnosed prior to age 13 involve intrapartum transfer. Sexual contact was the primary means of transmission in this group, espe- cially among homosexual teens. A comprehensive social history, including sexual orientation and activity, should be obtained at all routine adolescent visits, and counseling regarding safer sex practices should always be provided. The remainder of patients progress rapidly during the first several months of life. The three major classes of antiretrovirals are nucleoside reverse transcriptase inhibitors (didanosine, stavudine, zidovudine), nonnucleoside reverse transcriptase inhibitors (efavirenz, nevirapine), and protease inhibitors (indinavir, nelfinavir). Possible other abnor- malities include anemia, neutropenia, elevated transaminases, hyperglycemia, and hyperlipidemia. An existing treatment regimen is altered when toxicity becomes an issue or disease progression occurs. The child with sickle cell disease (Case 13) has an acquired immune defi- ciency due to splenic auto-infarction and a higher incidence of infection due to encapsulated (pneumococcus) organisms. Patients with frequent pneumo- nia (Case 14) or as a result to unusual organism and patients who have fre- quent or unusually severe otitis media (Case 16) may have a primary immune deficiency. The patient with cystic fibrosis (Case 18) has a variety of medi- cal issues such as malnutrition, vitamin deficiency, and frequent pneumonia characteristic of a patient with secondary immune deficiency. Leukemia (Case 19) and neuroblastoma (Case 33) represent secondary immune deficiencies. He admits to being sexually active, including oral sex, with a male partner over the past month. On physical examination, he is afebrile, with cer- vical and inguinal lymphadenopathy and a nonexudative pharyngitis. His examination is notable for a cord without evidence of separation and a shallow, 0. The mother declares that the “sore,” caused by a scalp probe, has been slowly healing since birth and was deemed unremarkable at his 2-week checkup. She is doing better today with improved intake and resolution of her emesis and diarrhea. The father is concerned about her thrush since birth (despite multiple courses of an oral antifungal), and that she has been hospitalized twice for pneumonia over the past 4 months. Her weight has dropped from the 50th percentile on her 4-month visit to the 5th percentile today. She has no findings consistent with dehydration, but she does appear to have some extremity muscle wasting. Her examination is remarkable for buccal mucosal exudates and hyperactive bowel sounds. He has atypical facies (wide-set eyes, a prominent nose, and a small mandible), a cleft palate, and a holosystolic murmur. Severe, life-threatening infection is possible with Staphylococcus spe- cies, Enterobacteriaceae, and Candida species. Good skin and oral hygiene are important; broad-spectrum antimicrobials and surgical debridement are early considerations with infection. Death typically occurs in the first 12 to 24 months of life unless bone marrow transplantation is per- formed. The child in the question has typical features of DiGeorge syndrome, caused by a 22q11 microdeletion. This syndromic immunodeficiency is char- acterized by decreased T-cell production and recurring infection. Findings include characteristic facies and velocardiofacial defects, such as ventricu- lar septal defect and tetralogy of Fallot. Thymic or parathyroid dysgenesis can occur, accompanied by hypocalcemia and seizures. Exposed patients should be closely followed by clini- cians and a team approach used in the management of active disease. His mother reports that he seems to be much more immature and insecure than her older son was at the same age.
H e complains of pain with passive motion of the hip and on physical examination the hip has limited range of motion upon internal rotation buy generic vytorin 20 mg online. Which of the below series of serum laboratory values are consistent wit h t he underlying condi- tion predisposing to this patient’s fracture? A24-yearoldAfrican-Americanmancomesintoclinicfromareferralafter having significant bleeding following a wisdom tooth extraction 1 week ago purchase vytorin 20mg with amex. H e has no significant medical history and took aspirin 1 week ago for a headache cheap vytorin 20mg amex. H e report s no family history of diagnosed bleeding disorders but endorses his mother easily bruises and has heavy menstrual bleeding. O n physical examination there are white plaques visible in the oral cavity and posterior pharynx that are easily removed with a tongue blade. An endoscopy is performed that demonstrates additional white plaques in the proximal esophagus. She has required multiple major surgeries as well as mechanical ventilation for the past week. Vital signs have been st able during t his t ime and her chest x-rays have been clear. A 60-year-old man with a history of hyperlipidemia controlled on simvastatin and well-controlled hypertension complains of nonbloody, watery diarrhea for over 1 year. H e complains of episodes of wh eezing and increased redness and heat in his face, neck, and upper chest that last up to 5 minutes. O n physi- cal examination there is hepatomegaly and a 2/ 6 holosystolic murmur is aus- cultated on the left lower sternal boarder that is accentuated with inspiration. Blood pressure is 135/ 85 mm H g, heart rate is 85 bpm, and respiratory rate is 16 breaths per minute. A 61-year-old man presents to the hospital complaining of substernal ch est pain an d malaise. H e reports increased pain when taking deep breaths but denies shortness of breath or palpitations. A 4 5 - y e a r - o l d m a n w i t h h i s t o r y o f s e i z u r e d i s o r d e r a n d a l c o h o l a b u s e is h ospit alized aft er a 30-minut e t onic-clonic seizure. Laboratory valu es in clu d e the followin g: S erum so dium 143,serum p o ta ssium 5. W h at is the most likely underlying pat h ophysiology t o this pat ient ’s con d it ion? A 6 5 - y e a r - o l d m a n w i t h a 3 0 - y e a r h i s t o r y o f s m o k i n g 2 p a c k s p e r d a y complains of increased swelling in both of his legs, increased dyspnea, and fat igue on exert ion. On physical examination there is distention of the right ju gu lar vein wh en fir m pr essu re is applied over the liver, presen ce of a para- st ernal heave, and t here are diffuse expirat ory wheezes heard on auscult a- tion of the lungs. A 4 0 - y e a r - o l d w o m a n c o m e s i n t o c l i n i c c o m p l a i n i n g o f a 1 5 - l b w e i g h t g a i n over the past 6 months despite a decrease in appetite. She states she is more bothered by cold temperatures, her hair is thinner, and recently has more difficulty passing bowel movements. She states that 8 months ago she was experiencing diarrhea, felt like her heart was beat ing quickly, and was always h ot. Today h er blood pressu r e is 125/ 85 mm H g, h ear t rat e is 70 bpm, and she is afebrile. O n physical examination there is no exoph- thalmos, thyroid is nontender and diffusely enlarged with a rubbery texture wit hout any isolated nodules, and her skin is dry to the touch. Furthermore, decreased kidney function impairs the excretion of phosphate, causing serum levels to become elevated, sometimes requiring treatment with phosphate binders. In sarcoidosis immune cells in the granulomas outside of the kidney increases the active form of vitamin D. H ip fractures secondary to osteoporosis are unlikely to demonstrate abnormal laboratory values where those caused sec- ondary to Paget disease will show an elevated alkaline phosphatase (See Case 35 [Osteoporosis] and Case 50 [H ypercalcemia]). This man has both platelet and clotting factor dysfunction inherited in an autosomal dominant fashion. If vW F is decreased or absent, platelets are unable to bind to the damaged endothelium, which causes clinical manifestations of platelet dysfunction (increased bleeding time) such as petechiae, epistaxis, and increased bruising without affect- ing the number of platelets. Choice B (Bernard-Soulier syndrome) is also an isolated dys- function of platelets whereby there is a deficiency of the receptor GpIb pre- ven t in g p lat elet s t o b in d t o the vW F exp r essed b y the d am aged ep it h eliu m. Esophagitis presents with primary com- plaints of odynophagia (painful swallowing), dysphagia, and a substernal burning chest pain. W hite plaques that are easily removed with a tongue blade are consistent with oral thrush caused by the opportunistic fungus— Candida that can cause esophagitis when it extends beyond the oral cavity to the proximal esophagus. Candida esophagitis is the most likely diagnosis in patients presenting with oral thrush. Endoscopy and biopsy are not necessary before initiating treatment with oral fluconazole. H owever, if patients show no improvement and have no signs of oral thrush, endoscopy with biopsy is warranted. This patient is likely in end-stage liver failure due to known alcoholic cirrhosis. It is associat ed wit h a h igh m or t alit y rate (20%-30%) and is likely to reoccur. Common symptoms include fever, diffuse abdominal pain, and altered mental status in a patient with known cirrhosis. H owever, empiric treatment should be initiated with a third-generation cephalosporin (ceftriaxone, cefotaxime) while awaiting cul- ture results given the most common cause is gram-negative organisms. Choice A (oral lactulose) would be the appropriate treatment for hepatic encepha- lopathy to prevent reabsorption of the toxic metabolite ammonia. Clinical presentation would include an altered mental status; however, this diagnosis is less likely given the patient’s fever and diffuse abdominal pain. It acts as a vasoconstrictor to the splanchnic vessels and helps reduce portal pressure. Choice D (liver transplant) and choice E (protein restricted diet) would not be appropriate initial treatment for this patient (see Case 24 [Cirrhosis]). It should be suspect ed when t h ere is an acut e ch ange in t he int ubat ed pat ient including a new fever, decrease in oxygen saturat ion, and new purulent secret ions. Choice A (C pneumoniae) is a cause of community-acquired at ypical pneumonia where t he pat ient present s wit h an insidious onset of various symptoms such as headache and fatigue with dry cough and fever— ”walking pn eumon ia.