Index of Suspicion

A 3-year-old girl is brought to the emergency department because of high fever for 3 days, pharyngitis,and an erythematous, pruritic rash. The rash appeared 2 days ago, and a physician had prescribed cephalexin. There is no history of allergy, recent travel, or contact with ill people.On physical examination, the girl does not appear toxic. She has a rectal temperature of 38.4°C(101.2°F), pulse of 180 beats/min,respiratory rate of 34 breaths/min,and blood pressure of 100/67 mm Hg. There is a generalized erythematous rash as well as urticarial lesions. No evidence of skin infection is found. The conjunctivae are injected, and her lips are red and dry. The throat is diffusely reddened. Her lymph nodes are not enlarged. No heart murmurs are heard, and the lungs are clear. Findings on the remainder of the physical examination are normal.Laboratory results include: white blood cell count, 14.4 × 109/L(14.4 × 103/mcL), with 11% band forms, 68% segmented neutrophils,12% lymphocytes, and 7%monocytes; hemoglobin, 1.74 mmol/L(11.2 g/dL); platelet count, 299 ×109/L (299 × 103/mcL); and erythrocyte sedimentation rate (ESR),105 mm/h. Levels of electrolytes,blood urea nitrogen, and creatinine and results of liver function tests and urinalysis are normal. Blood and throat cultures are obtained. Chest radiography reveals an infiltrate in the right lower lobe.The girl is admitted and is treated for 3 days with intravenous cefuroxime. When she fails to respond,erythromycin is substituted to cover for Mycoplasma pneumoniae. Despite therapy, her rectal temperature ranges between 38.6°C and 40.0°C (101.5°F and 104.0°F), and the rash changes into diffuse pruritic erythroderma that simulates the appearance of sunburn. A desquamating perineal eruption appears. The rash is not accentuated in her skinfolds and creases, there are no Pastia lines, the skin is not tender,and a Nikolsky sign is not present. Her blood pressure is normal. All culture specimens yield no growth. On the seventh day of fever, swelling of her hands and feet becomes more evident and suggests the diagnosis.A 22-month-old girl is brought to the emergency department because of loss of consciousness. The family was on their idling open boat on a local river on this hot summer day. The child seemed well until her right leg stiffened and she lost consciousness for approximately 10 minutes. She regained consciousness prior to the arrival of the emergency medical technicians, who transported her to the hospital. No other family members report any complaints.On physical examination, the child is alert, smiling, and well tanned. Her temperature is 37.2°C(99.0°F), heart rate is 120 beats/min and regular, respiratory rate is 20 breaths/min, and blood pressure is 92/50 mm Hg. Physical examination yields all normal findings,including evaluations of her heart,lungs, and neurologic status. The patient is given an intravenous fluid bolus of normal saline, and blood is drawn for tests, the results of which reveal the cause of her loss of consciousness.An 11-month-old Native Canadian boy is admitted to the hospital because of growth failure and chronic diarrhea. The infant was thriving until 9 months of age, when he was at the 95th percentile for length and between the 75th and 95th percentiles for weight. He has had 8 to 16 watery, loose stools per day for the past 2 months. There is no blood or mucus in the stools, but he is “gassy” and has frequent abdominal cramps. In the past 4 weeks, his appetite has diminished. He has had numerous formula changes from cow milk to soy formula and then to a protein hydrolysate formula. Rice cereals were introduced at 5 months of age, and pureed baby food and juices were started at 7 to 8 months of age. He has been well otherwise. There is a family history of lactose intolerance.On examination, the infant is not acutely ill or dehydrated, but obviously is thin, with his weight having dropped below the 25th percentile while his length is just below the 50th percentile. Cardiovascular,respiratory, skin, and musculoskeletal examinations yield normal findings. No hepatosplenomegaly or abdominal distention is noted. His bowel sounds are increased in intensity.Stool analysis is negative for blood, eosinophils, and reducing substances. Stool pH is 6.5, and there are few white blood cells present. All stool cultures and tests for ova and parasites produce negative results. The child is not anemic or hypoalbuminemic, and he does not have peripheral eosinophilia. One further test reveals the diagnosis.A 2½-year-old boy is brought to the emergency department because of noisy breathing. He has had fever and left-sided neck pain for 4 days and has been drooling at times over the past 24 hours. His mother reports that recently he has appeared weak and pale and has had a decreased intake of solid foods.The patient is an ill-appearing,fussy boy who sits leaning forward and is breathing with labor at 52 breaths/min. Stridor is audible on both inspiration and expiration. He is afebrile and has a pulse of 160 beats/min, blood pressure of 100/68 mm Hg, and pulse oximetry reading of 95% saturation in room air. The rest of his physical examination is unremarkable except for generalized pallor and fullness of the left side of his neck. Laboratory evaluation reveals a white blood cell count of 0.3 × 109/L (0.3 ×103/mcL) with no neutrophils,hematocrit of 0.15 (15%), and platelet count of 57 × 109/L (57 × 103/mcL). Radiographs of the lateral neck are interpreted as showing a prevertebral soft-tissue mass.A specific course of action and additional tests confirm the diagnosis and explain the clinical picture.A boy weighing 3,560 g is born at term to a 35-year-old multigravida mother who was diagnosed as having hydramnios during pregnancy. Apgar scores are 9 at both 1 and 5 minutes. There is no meconium staining of amniotic fluid or prolonged rupture of membranes. At the age of 3 hours, the baby develops stiffening episodes that affect the trunk and all four extremities as well as generalized cyanosis and bradycardia. Desaturation occurs during these spells, as measured by pulse oximetry, and excessive oral secretions are noted.Physical examination reveals normal vital signs and normal heart,lung, and neurologic findings,including Moro, suck, and grasp reflexes. There are no dysmorphic features. Blood levels include:glucose, 4.27 mmol/L (77 mg/dL); urea nitrogen, 2.86 mmol urea/L (8 mg/dL);sodium, 144 mmol/L(144 mEq/L); potassium,4.6 mmol/L (4.6 mEq/L); chloride,108 mmol/L (108 mEq/L); total CO2, 21 mmol/L; calcium,2.38 mmol/L (9.5 mg/dL);magnesium, 16 mg/L (1.6 mg/dL); and ammonia, 66 mmol/L. Arterial blood gas levels in room air are: pH, 7.3;PCO2, 43 torr; PO2, 86 torr; total CO2, 22 mmol/L; hemoglobin,2.50 mmol/L (16.1 g/dL);hematocrit, 0.57 (57%); platelets, 278 ×109/L (278 × 103/mcL); and white blood cell count, 11.8 × 109/L(11.8 × 103/mcL) with 51%neutrophils and 39% lymphocytes. Cultures of blood, urine, and cerebrospinal fluid yield no growth. Electroencephalography,electrocardiography, and ultrasonography of the brain all show normal findings. Chest radiography that is performed as part of a sepsis screen reveals normal lungs and heart and a dilated proximal esophagus with air distention of the entire gastrointestinal tract.A 12-year-old Asian-American boy comes to you because of bilateral knee pain. His right knee started to hurt 3 days ago, and his left knee has hurt for 2 days. The pain is so excruciating that he cannot walk. He notes also that his left ankle is swollen and painful. Although he plays hockey, he denies any recent trauma to his knees and ankle. A review of systems reveals that he experienced a sore throat and headache 6 days ago and has had a fever of 38.3°C to 38.9°C (101°F to 102°F) for the past 4 days. He has had no diarrhea or vomiting and denies sexual activity.His father carried the 72 kg,heavyset boy into the examination room. The boy is afebrile and has a heart rate of 100 beats/min and blood pressure of 132/70 mm Hg. He appears comfortable when his knees are in a flexed resting position, but both knees are exquisitely tender when extended. The range of motion is limited by pain. His discomfort seems out of proportion to the minimal swelling and warmth present. No erythema, puncture sites,or bruising are noted. His left ankle is swollen and also hurts when extended. His hip joints can be moved comfortably through a full range of motion. The boy’s oropharynx is mildly injected. Heart, lung,and abdominal examinations yield normal findings.The clue to the diagnosis was the indurated edema of the child’s hands and feet. In addition, erythematous,dry, cracking, fissured lips were noted. These signs as well as bilateral nonpurulent conjunctival injection, erythema of the oral cavity and pharynx, polymorphous rash, and fever, which by that time had lasted for 7 days, established the diagnosis of Kawasaki disease.After the diagnosis had been established, intravenous immune globulin (2 g/kg) was administered in a 12-hour infusion, during which the girl defervesced. On the same day, aspirin (100 mg/kg per 24 hours) was started. The girl had an uneventful recovery and was discharged on the tenth day of her illness, appearing well. Findings on her examination at discharge were normal except for of her hands and feet that had started the and and the entire of her and She had a of × × 103/mcL), an platelet count of × 109/L and an at mm/h. at the time of and on evaluations no no evidence of or clinical diagnosis of was by the results of skin that had been performed when her erythroderma was The with edema in the of the and a few is a are 5 of age. The is by fever that for at 5 days and by at four of the bilateral nonpurulent conjunctival diffuse erythema of the oral cavity and and erythematous, fissured of hands and feet by a polymorphous rash, on the and lymph is the of Kawasaki in approximately of of the hands and feet is the physical rash a eruption of erythematous of a which is erythematous and and an erythema of rash. a rash in who have of is not in who have Kawasaki A perineal eruption is in during the of is the of Kawasaki to and that in to of abdominal and be as of the The and protein levels are The total white blood cell count to be as well. The platelet count be normal at the of the illness, but is by the tenth day of the a of to ×109/L to × the tenth and days the of diagnosis of Kawasaki is because by intravenous immune globulin by aspirin appears to be in given 10 days of the of a is that this be in all who have fever of cultures are negative and the fever is to erythematous, pruritic eruption in a child be by other the is that the rash and in Kawasaki also in as skin and to these with skin have exquisitely tender skin, and of in to lateral and the and be in to diffuse who have appear In to fever and with and signs of of at and and an increased of neurologic and in and and liver a clinical and is with respiratory and soft-tissue of fever, which is by infection with the child’s is there is and the skin is not The erythematous rash is accentuated in skinfolds and creases, and erythema and appear in a the creases, Pastia A red or appearance to the is all of these the of as or skin be or be cultures are obtained. is in of of Kawasaki is not have been and The is to be of the and as as so as to in the diagnosis of a patient who has loss of consciousness as and The that this diagnosis was a of test was performed because a history prolonged to the of the while the boat was is the cause of in the is by the of is a and the in of and as in this is of but is in this by the of to The of are in the months to of a during of is to of hemoglobin, and respiratory by to the in the of the The of for is times that of the to the to be at the at the same time as of in the The clinical of are to systems to the brain and the is in the to the emergency department or with from or and to and signs and are at from the of are because of their and respiratory The is at because more the a to the to which the patient is is the test for are to the of on the at be that pulse oximetry results because the pulse does not A of as and an blood be measured as normal is and is not is to for evidence of or be on the and of the clinical of administered by the of to from 5 to 6 at room air to at The patient more on the clinical prolonged that be for the of in blood and the of for a or the of no is the patient he or she is and has a the is or the patient is and has a he or she be to the to of the neurologic These be well their or but or signs 3 to days These from headache or to peripheral or of neurologic findings. has been to the of both when at the time of the and when the patient with she had been from her this patient was to a she of radiography performed prior to of the child’s neurologic examination were normal both and and she was discharged from the hospital that and lower was both and examination no A for specific a in an infant who has chronic diarrhea and failure to a is to the of specific A history the of the the and any between introduced and diarrhea is history of chronic respiratory or be weight and are in growth these are of chronic diarrhea A physical examination, for evidence of chronic respiratory abdominal and be of the skin, musculoskeletal and neurologic are in clinical signs of evaluation be at from as well as further for a specific of a stool for pH, reducing and blood is child’s stool pH was The pH of stools in who have between and stool pH results in be for examination, and of of can be by a of a stool for or more by a a blood count and blood and erythrocyte sedimentation blood for liver A is of can from a in a of or loss of by any with results from a in a by both and in and are is the because is the to any and is the to is the of is more of this between the of 3 and 5 results in of the When the is by and are and in of and fluid into the bowel The increased of fluid and are to and The is increased stool and the of is the is as an and has been to is in the Native of and to In the the is patient to have diarrhea when or juices are diarrhea and growth failure are and who have in in this age abdominal excessive rash, and In for of diarrhea and with chronic diarrhea by normal growth. who have this have signs and of bowel abdominal pain. has been diagnosed in a history of having had and stool pH and evidence of evidence of of these tests yield a high of results. that is not a reducing and yield a negative test be more a is with and then for reducing test can evidence of specific but can by a of A bowel for but also other that have a examination of from who have is normal except in who show Although of the for not be at all In any specific the for the with specific tests and the more and and he or she that the additional by the to of of a diarrhea on a that of a formula that does not not a in the diarrhea he is of with this is and of these have chronic gastrointestinal a has been recently in who have with results. for of have been for this boy’s clinical The were during the course of his emergency department and prevertebral At the time of his from the emergency the diagnosis was the of a specific diagnosis was the of this boy’s clinical was the for and respiratory further in his respiratory he and in the room. A mm oral was the had the that were to his red and swollen from the to the was for and of the neck prevertebral was from his blood on hospital day negative results. The patient was on hospital day and on day 20 recovery from his and specific cause was for the boy’s and A review of is the of this but be that of In approximately of as in this no or specific cause can be found. can from and is well that to there are no reports of and in who have the of the with and The of the has to a in the of but other as can cause in who have been with the These are more In who have all of infection be also that can be by trauma and is an of the and other as the of the to and in an child between 2 and 5 of age. The child is as to and of neck or throat and have the child becomes the of these is to and the of are be the child’s is in and does not to a respiratory by When is of the by a is is to the during be in the room in the of both an and an of and in the emergency department to evaluation be be by the in the room. evaluation be in the to in who have and Despite the in by can cause in the of a as well as are when of having of of were of A with a in of the in the of the proximal the The infant and has had no more occurs in 1 in to In of a between the and esophagus is present. the or the occurs in These are to from of the into and growth of cells to and of the lateral of the during of from results in be there is a history of hydramnios during when a cannot be to the in the of excessive oral or the baby develops or with The diagnosis is when a a in the the the esophagus to the air the which becomes and with breathing more to into the the the proximal esophagus to the the in In the of sign is the diagnosis is is not with of who have have that can be or gastrointestinal as or as part of the and and prior to at a an show an dilated with as in this The in the proximal is but a into the of a normal to the of A can be performed by of to for a proximal as well as to the of the proximal has more during ultrasonography when hydramnios is is a The baby be in an and the proximal to the of secretions the A for be of of the and of the of the is performed in and of and which of excessive oral be other clinical as in this When the the esophagus and into the can cause and bradycardia. and also in who have can is that the stiffening in this baby episodes to of The of excessive oral secretions a clue to the of with a that can be or out with A and are that an of amniotic be in any of pain with fever, but occurs in 2 of age and the child is can affect but not be in a boy who is not can in the of or a is but be in that when with knee pain in a the is the hip in a that with knee pain. of these are and of are the of this boy’s pain in with comfortable and of his knee discomfort this The fever also from a pain is but the of a of when the is moved this diagnosis In addition, the left ankle swelling and pain the fever can be by or pain or occurs infection and has been with and or a infection in the of fever has been There is over this be a also is with and and The of is a to that or develops 1 to 2 the is a or joints of the lower The is 2 and over 2 to 6 also can as a of that can be or and in A history of a or the erythema rash this is to the patient has time in an is of also be in any patient who has and swelling of the of and can with of with joints as in the or hip or with the and is by and signs of rash, The can appear or a few months of is in which the joints be exquisitely tender and the pain is out of proportion to the physical findings. The course of the is as 1 to 5 a throat develops in a lower The of any in 24 and for 2 to 5 days. the of the joints a is to that the and fever of 24 to of aspirin of a A infection as well as erythema or and prolonged on are to confirm the diagnosis of fever boy had a history of sore throat and exquisitely knees and left ankle out of proportion to the physical findings. The test was His white blood cell count was ×109/L × 103/mcL), with a count of and lymphocytes. His was mmol/L platelet count was ×109/L × 103/mcL). His erythrocyte sedimentation rate was 120 He was to a who a The was for a of range of The and function and no The day, his throat culture was for The was to be at his was to boy the for with as a and fever and as The evidence of infection was in the of an and as well as the throat his was as a heart a The results from by and The of evidence of in the of an audible is because can in are not in patient not have the other but is is a of that occurs 2 to 6 months the of and is by the of of all to and in in with is a rash by or that have and and are over the trunk and are on the of and as well as in the or over of the and These in no more of who have boy was admitted to the hospital and treated with an of as well as aspirin therapy, on which he for 4 to 100 mg/kg per is prescribed for who have or no is in pain 24 of In this the of aspirin therapy, and he was discharged 2 days to out of the hospital. examination 1 discharge no on but his had decreased to who have or heart to 2 mg/kg per is there is no evidence that or the course of or the of the boy also was on rest with for approximately 3 rest physical and pain. The of rest for who not have examination 2 months no or He on fever The for the of for who have had with but no heart is 10 or well into is that are with the in not the in a specific for this his was is as a of but there have been in the and recent is by and which are in more that of the has the is that no is in the of the have in There is a of evidence that the of and an in the of A are for the of in the is recent is that have A of had medical for the more the exquisitely tender

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