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CLINICAL PRESENTATION      Dr. Juan Carlos Díaz Torre                                   DR. JCDT      Pediatra Neonatólogo...
Rash, Pain, and Dyspnea Progressing            to Respiratory FailureA 68-year-old woman presents to the emergencydepartme...
About 10 days ago she noted a small ulcer on thedorsum of her left foot that started to grow insize, with associated low-g...
She reports no headache, neckstiffness, nausea, vomiting, expectoration, chestpain, cough, sore throat, abdominalpain, cha...
On physical examination, the patient is a mildlyobese female who appears very ill and is in acutedistress. Pallor, mild cy...
She is tachycardic with a heart rate of 122beats/min. The pharyngeal examination shows noerythema or exudate. The axillary...
Her skin appears plethoric and cool with adiffuse, nonvesicular, nonpalpable, petechial, non-blanching rash (see images).A...
Laboratory studies are ordered; these include acomplete blood cell count with a hemoglobin of14.4 g/dL (144 g/L); a hemato...
Her coagulation studies at admission are allnormal, but her creatinine is significantly elevatedat 3.2 mg/dL (285 µmol/L)....
A lumbar puncture is performed, which shows aclear acellular cerebrospinal fluid with lowglucose 34.8 mg/dL (1.9 mmol/L) a...
The patient is treated in the ED with intravenous(IV) crystalloid, IV steroids, vasopressorsupport, and IV antibiotics.End...
DR. JCDTA purpuric petechial rash is seenaround the umbilical zone                                    12
DR. JCDTThe rash is disseminated all over thepatients skin, with a mild cyanotic tint.                                    ...
DR. JCDTA closer view of the inguinal zone shows apurpuric unblistered rash.                                             14
What is the diagnosis?              .Hint: Consider the patients history, leg ulcer, anddescribed lesions.- Henoch-Schönle...
Correct answer:            .- Henoch-Schönlein purpura                                                  DR. JCDT- Anaphyla...
While in the ICU, the patients shock progressed inspite of high doses of vasopressors and stressdoses of glucocorticoids m...
In this case, the diagnosis of Waterhouse-Friderichsen syndrome (WFS) was made clinicallyon the basis of the patients hist...
The presence of shock, DIC, skin lesions, andbilateral adrenal hemorrhage in the presenceof sepsis are specific criteria f...
Blood cultures later revealed a widely susceptible(gentamicin, amikacin, ciprofloxacin, norfloxacin,meropenem, aztreonam, ...
WFS was first described by the British physicianRupert A. Waterhouse in 1911 as "a case ofsuprarenal apoplexy" and by Carl...
WFS is generally associated with fulminantmeningococcemia (Neisseriameningitides), although many other organisms havebeen ...
Other organisms involved are bacteria such asStaphylococcus aureus, Haemophilusinfluenzae, Streptococcuspneumoniae, Pseudo...
Noninfectious etiologies include birthtrauma, pregnancy, idiopathic adrenal veinthrombosis, bilateral metastatic adrenalma...
Adrenal hemorrhage is a rare condition; only 10%of patients with adrenal hemorrhage developglandular failure.             ...
The fact that glucocorticoid replacement does notalways prevent death in patients with adrenalhemorrhage or acute insuffic...
Early in the course of disease, the patient mayexperience flank, epigastrium, and/or abdominalpain.                       ...
Hours later, hypotension or collapse withagitation, delusions, and extensive areas ofpetechial-hemorrhagic rash appear in ...
Extensive subcutaneous confluent hemorrhageknown as "purpura fulminans" may also occur andis caused by DIC.This rash is pr...
Petechiae often appear initially on theankles, wrists, and in the axillae and may spread toany part of the body (including...
Despite these rather impressive clinicalfindings, the clinical diagnosis of WFS may beextremely challenging.              ...
The pathophysiology of this syndrome is not wellunderstood, but available evidence has implicatedadrenocorticotropic hormo...
The adrenal gland has a rich arterial supply, incontrast to its limited venous drainage, which iscritically dependent on a...
In addition, adrenal vein spasm induced by highcatecholamine levels secreted in stressful situationsand by adrenal vein th...
The adrenal cortex produces both cortisol (aglucocorticoid) and aldosterone (amineralocorticoid).                         ...
Aldosterone modulates renal sodium reabsorptionin exchange for potassium excretion.Hyperkalemia is caused by aldosterone d...
Patients with splenectomy orasplenia, diabetes, organ transplantation, andthose receiving chemotherapy or chronic steroids...
In WFS, a normal to slightly elevated leukocytecount with a left shift is often seen. The chemistryprofile may demonstrate...
The blood pressure may be abnormally low.Coagulation abnormalities may be present andmay reflect ongoing DIC and consumpti...
These abnormalities include elevatedprothrombin time, partial thromboplastintime, fibrin degradation products, and decreas...
A contrast-enhanced abdominal CT scan willtypically demonstrate enlargement of the adrenalglands with bilateral hemorrhage...
Other microbiologic testing, such as woundcultures, should be directed by the clinical scenario.Smears of petechial skin l...
Due to the fulminant course of WFS, therapy shouldstart as soon as the diagnosis is suspected. Initialantibiotic coverage ...
Antibiotics with reported antipseudomonal effectsinclude penicillins(ticarcillin, piperacillin, piperacillin/tazobactam), ...
However, decision making regarding specificantipseudomonal therapies should be guidedby local resistance patterns.        ...
In adrenal crisis, the goal is to reverse thehypovolemia with crystalloid, which may besupplemented with 5% dextrose IV (g...
Stress-dose glucocorticoids, either hydrocortisone50 mg every 6 hours or dexamethasone 4 mg every12 hours can be administe...
Mineralocorticoid replacement with fludrocortisonemay be indicated in patients with a history ofbilateral, extensive adren...
Therapy is unnecessary in (acutely ill)patients receiving more than 100 mg ofhydrocortisone daily, as this dose is        ...
In spite of adequate and aggressive treatmentwith fluids, glucocorticoids, antibiotictherapy, and ventilation, the patient...
Although the antibiotic selection on admissionwas effective against P aeruginosa latelyisolated, the initial selection of ...
The diagnosis of WFS was later confirmed viaautopsy, wherein massive bilateral suprarenal andhemorrhagic effusion in other...
There were no signs of meningitis.Rapidly progressive vascular collapse andacute respiratory failure caused by            ...
Review: You are examining an adult patient who yoususpect may be manifesting WFS. Which of thefollowing organisms would mo...
Correct answer:- H influenza- M tuberculosis                           DR. JCDT- N meningitidis    ****- Cytomegalovirus  ...
N meningitidis is the most frequent organismreported as a cause of WFS, although massivevaccination has decreased the inci...
If you do in fact suspect a diagnosis of WFS in apatient you are examining, which of the followingcourses of action would ...
Correct answer:- Antibiotics should be prescribed but only afterbacterial susceptibility confirmation- Broad-spectrum anti...
Due to the fulminant course of thissyndrome, therapy should be started as soonas the diagnosis is suspected.              ...
Gracias por su atención                                  DR. JCDT    Dr. Juan Carlos Díaz Torre       Pediatra Neonatólogo...
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Clinical presentation march 24, 13

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Clinical presentation march 24, 13

  1. 1. CLINICAL PRESENTATION Dr. Juan Carlos Díaz Torre DR. JCDT Pediatra Neonatólogo dr_diaz_torre@hotmail.com ( 779 ) 100 - 40 - 26 1
  2. 2. Rash, Pain, and Dyspnea Progressing to Respiratory FailureA 68-year-old woman presents to the emergencydepartment (ED) complaining of progressiveshortness of breath, flank pain, fever, and DR. JCDTweakness. A diffuse, pinkish rash that had started 6hours before presentation is noted over herextremities and trunk.She has a history of diabetes and has been takingglyburide irregularly, with poor glycemic controldocumented by blood glucometer record. 2
  3. 3. About 10 days ago she noted a small ulcer on thedorsum of her left foot that started to grow insize, with associated low-grade fever and painalong her left leg. Her primary doctor prescribedher a course of amoxicillin, which she is currently DR. JCDTtaking.She takes no other medications, does not smoketobacco, and does not use illicit drugs or alcohol. 3
  4. 4. She reports no headache, neckstiffness, nausea, vomiting, expectoration, chestpain, cough, sore throat, abdominalpain, changes in urinecolor, melena, hematemesis, or bleeding. DR. JCDTShe has no medical allergies and has notrecently traveled outside her home country ofCuba. 4
  5. 5. On physical examination, the patient is a mildlyobese female who appears very ill and is in acutedistress. Pallor, mild cyanosis, poor capillary refill(3-4 sec), and a weak, rapid radial pulse are noted.She is conscious and is alert and oriented. DR. JCDTShe has a patent airway and her respiratory rate is30 breaths/min with bilateral rales and diminishedair entry into both lung bases.Normal S1 and S2 heart sounds are heard with nodiscernible murmur. 5
  6. 6. She is tachycardic with a heart rate of 122beats/min. The pharyngeal examination shows noerythema or exudate. The axillary temperature is98.6°F (37°C). Her blood pressure is 85/50 mm Hg.No jugular venous distension is noted. DR. JCDTThe abdominal examination is unremarkable;examination of the stool is negative for occultblood. 6
  7. 7. Her skin appears plethoric and cool with adiffuse, nonvesicular, nonpalpable, petechial, non-blanching rash (see images).A necrotic, crusted black eschar is observed on her DR. JCDTleft leg. The eschar is about 5 cm in diameter.Neurologic examination is unremarkable and thereare no signs of meningismus. 7
  8. 8. Laboratory studies are ordered; these include acomplete blood cell count with a hemoglobin of14.4 g/dL (144 g/L); a hematocrit of 42% (0.42 L/L);white cell count of 7 x 103 /µL (7 x 109 /L) with aneutrophilic predominance of 79%; a platelet count DR. JCDTof 250 × 103/µL (250 × 109/L); and a normalperipheral smear. 8
  9. 9. Her coagulation studies at admission are allnormal, but her creatinine is significantly elevatedat 3.2 mg/dL (285 µmol/L).Arterial blood gas analysis (with supplemental DR. JCDToxygen) reveals a pH of 7.10, PaO2 of 80 mmHg, PaCO2 of 49 mm Hg, SaO2 of 90%, bicarbonateof 13 mmol/L, and a base deficit of -15.2 mmol/L. 9
  10. 10. A lumbar puncture is performed, which shows aclear acellular cerebrospinal fluid with lowglucose 34.8 mg/dL (1.9 mmol/L) and a normalprotein level. The results of a urinalysis arenormal. She is hypoglycemic, with finger stickglucose of 58.7 mg/dL (3.2 mmol/L). DR. JCDTElectrolyte analysis demonstrates mildhyperkalemia (5.7 mmol/L).A chest radiograph shows changes consistentwith early adult respiratory distress syndrome(ARDS). 10
  11. 11. The patient is treated in the ED with intravenous(IV) crystalloid, IV steroids, vasopressorsupport, and IV antibiotics.Endotracheal intubation is performed due toworsening hypoxic respiratory failure, and the DR. JCDTpatient is placed on a mechanical ventilator; thepatient is then transported to the intensive careunit.Gram stain and blood culture samples of the skinlesions are obtained. 11
  12. 12. DR. JCDTA purpuric petechial rash is seenaround the umbilical zone 12
  13. 13. DR. JCDTThe rash is disseminated all over thepatients skin, with a mild cyanotic tint. 13
  14. 14. DR. JCDTA closer view of the inguinal zone shows apurpuric unblistered rash. 14
  15. 15. What is the diagnosis? .Hint: Consider the patients history, leg ulcer, anddescribed lesions.- Henoch-Schönlein purpura DR. JCDT- Anaphylactic shock secondary to amoxicillin- Waterhouse-Friderichsen syndrome- Toxic epidermal necrolysis 15
  16. 16. Correct answer: .- Henoch-Schönlein purpura DR. JCDT- Anaphylactic shock secondary to amoxicillin- Waterhouse-Friderichsen syndrome ****- Toxic epidermal necrolysis 16
  17. 17. While in the ICU, the patients shock progressed inspite of high doses of vasopressors and stressdoses of glucocorticoids maintained through herhospital course. Her blood coagulation profile wascompatible with disseminated intravascularcoagulation (DIC). DR. JCDTSerum cortisol was not measured becausehydrocortisone was administered in the ED.The Gram stain, skin lesion cultures, andcerebrospinal fluid cultures were all negative. 17
  18. 18. In this case, the diagnosis of Waterhouse-Friderichsen syndrome (WFS) was made clinicallyon the basis of the patients history andlaboratory findings. DR. JCDTAlthough hypoglycemia, hypotension, andhyperkalemia could be associated with severesepsis, acidosis, and acute renal failure, someclues suggested the WFS diagnosis, such ashypotension that does not respond tovasopressors (later confirmed in this case), skin 18rash, DIC, and the abrupt onset of shock.
  19. 19. The presence of shock, DIC, skin lesions, andbilateral adrenal hemorrhage in the presenceof sepsis are specific criteria for WFS. DR. JCDTHer history of poorly controlled diabetes, aswell as the leg ulcer, aroused suspicion forbacterial infection through her skin ulcer. 19
  20. 20. Blood cultures later revealed a widely susceptible(gentamicin, amikacin, ciprofloxacin, norfloxacin,meropenem, aztreonam, among others)Pseudomonas aeruginosa infection. DR. JCDTA CT scan of the abdomen was not performedbecause of the progressive deterioration of thepatients clinical condition. 20
  21. 21. WFS was first described by the British physicianRupert A. Waterhouse in 1911 as "a case ofsuprarenal apoplexy" and by Carl Friderichsen, aDanish pediatrician, in 1918, although previous DR. JCDTcases had been reported in the few yearsbeforehand. 21
  22. 22. WFS is generally associated with fulminantmeningococcemia (Neisseriameningitides), although many other organisms havebeen associated with WFS, including viruses such as DR. JCDTcytomegalovirus and HIV, parvovirus B19, andEpstein-Barr virus. 22
  23. 23. Other organisms involved are bacteria such asStaphylococcus aureus, Haemophilusinfluenzae, Streptococcuspneumoniae, Pseudomonas DR. JCDTaeruginosa, Escherichia coli, and Mycobacteriumtuberculosis; and fungi such as Histoplasmacapsulatum (among others). 23
  24. 24. Noninfectious etiologies include birthtrauma, pregnancy, idiopathic adrenal veinthrombosis, bilateral metastatic adrenalmalignancies, seizures, anticoagulanttherapies, or following venography, trauma, and DR. JCDTsurgery.Recently, antiphospholipid antibody syndromehas been associated with adrenal hemorrhageand infarction. 24
  25. 25. Adrenal hemorrhage is a rare condition; only 10%of patients with adrenal hemorrhage developglandular failure. DR. JCDTMore than 90% of both glands must be destroyedbefore signs of adrenal insufficiency manifest;however, adrenal failure that does develop fromadrenal hemorrhage is usually lethal. 25
  26. 26. The fact that glucocorticoid replacement does notalways prevent death in patients with adrenalhemorrhage or acute insufficiency suggests thatWFS is a consequence rather than a cause. DR. JCDTSymptoms and signs may be diverse but aretypically sudden and unexpected, sometimeswithout prior illness. Even with aggressivetherapy, many patients die in less than 24-36 hours. 26
  27. 27. Early in the course of disease, the patient mayexperience flank, epigastrium, and/or abdominalpain. DR. JCDTComplaints may include an acute onset ofsymptoms commencing with chills, malaise, severeheadache, vertigo, vomiting, and prostration. 27
  28. 28. Hours later, hypotension or collapse withagitation, delusions, and extensive areas ofpetechial-hemorrhagic rash appear in themucosae and skin that may become confluent andform extensive purpuric areas ("flowers of DR. JCDTdeath"). 28
  29. 29. Extensive subcutaneous confluent hemorrhageknown as "purpura fulminans" may also occur andis caused by DIC.This rash is present in more than 75% of patients DR. JCDTand usually begins as a pink, maculopapulareruption on the extremities; it also develops apetechial component that becomes ecchymoticand hemorrhagic. 29
  30. 30. Petechiae often appear initially on theankles, wrists, and in the axillae and may spread toany part of the body (including the conjunctiva);however, it tends to spare the palms, soles, and DR. JCDThead. 30
  31. 31. Despite these rather impressive clinicalfindings, the clinical diagnosis of WFS may beextremely challenging. DR. JCDTPatients who appear in the initial and nontoxic-appearing stage without any skin lesions may bedifficult to distinguish from patients with benignviral illness. 31
  32. 32. The pathophysiology of this syndrome is not wellunderstood, but available evidence has implicatedadrenocorticotropic hormone (ACTH), adrenal veinspasm and thrombosis, and the normally limited DR. JCDTvenous drainage of the adrenal gland. 32
  33. 33. The adrenal gland has a rich arterial supply, incontrast to its limited venous drainage, which iscritically dependent on a single vein. DR. JCDTFurthermore, in stressful situations, ACTH secretionincreases, which stimulates adrenal arterial bloodflow that may exceed the limited venous drainagecapacity of the organ and lead to hemorrhage. 33
  34. 34. In addition, adrenal vein spasm induced by highcatecholamine levels secreted in stressful situationsand by adrenal vein thrombosis induced bycoagulopathies may lead to venous stasis and DR. JCDThemorrhage. 34
  35. 35. The adrenal cortex produces both cortisol (aglucocorticoid) and aldosterone (amineralocorticoid). DR. JCDTCortisol maintains cardiac output, vascularresistance, and hepatic glucose output. Shock anddeath can occur without adequate glucocorticoids. 35
  36. 36. Aldosterone modulates renal sodium reabsorptionin exchange for potassium excretion.Hyperkalemia is caused by aldosterone deficiency DR. JCDTand/or acidosis due to shock and poor perfusion.Immunodeficiency seems to be a predisposingcondition to WFS resulting from infectiousprocesses. 36
  37. 37. Patients with splenectomy orasplenia, diabetes, organ transplantation, andthose receiving chemotherapy or chronic steroids DR. JCDTare at higher risk for septic shock and death afteran infection. 37
  38. 38. In WFS, a normal to slightly elevated leukocytecount with a left shift is often seen. The chemistryprofile may demonstrate an elevated anion-gapmetabolic acidosis secondary to lactic acidproduction. DR. JCDTIf there is bilateral adrenalhemorrhage, hyponatremia, hyperkalemia, mildazotemia, leukocytosis witheosinophilia, and, occasionally, hypoglycemia maybe found. 38
  39. 39. The blood pressure may be abnormally low.Coagulation abnormalities may be present andmay reflect ongoing DIC and consumptivecoagulopathy. DR. JCDT 39
  40. 40. These abnormalities include elevatedprothrombin time, partial thromboplastintime, fibrin degradation products, and decreasedfibrinogen and platelets. DR. JCDTLow serum cortisol levels indicate relative adrenalinsufficiency. 40
  41. 41. A contrast-enhanced abdominal CT scan willtypically demonstrate enlargement of the adrenalglands with bilateral hemorrhage.Gram stain and cultures should be obtained from DR. JCDTblood, cerebrospinal fluid, urine, and sputum. Ina large percentage of patients, no organisms willbe seen on Gram stain and cultures will not revealany organism for more than 24 hours. 41
  42. 42. Other microbiologic testing, such as woundcultures, should be directed by the clinical scenario.Smears of petechial skin lesion scrapings should beperformed. These efforts may demonstrate the DR. JCDTpathogen in about 70% of cases. 42
  43. 43. Due to the fulminant course of WFS, therapy shouldstart as soon as the diagnosis is suspected. Initialantibiotic coverage should be broad, consisting of athird-generation cephalosporin(eg, cefotaxime, ceftriaxone) in adults. DR. JCDTIn this case, a highly susceptible P aeruginosa strainwas isolated. 43
  44. 44. Antibiotics with reported antipseudomonal effectsinclude penicillins(ticarcillin, piperacillin, piperacillin/tazobactam), cephalosporins (ceftazidime, cefepime), carbapenems(imipenem/cilastatin, meropenem, doripenem), mo DR. JCDTnobactamics (aztreonam), aminoglycosides(tobramycin, gentamicin, amikacin, netilmicin), fluoroquinolones (ciprofloxacin, levofloxacin), andcolimicin. 44
  45. 45. However, decision making regarding specificantipseudomonal therapies should be guidedby local resistance patterns. DR. JCDTSupportive therapy includes intravenousfluids, inotropic support, mechanicalventilation, and correction of coagulopathy andelectrolyte abnormalities as needed. 45
  46. 46. In adrenal crisis, the goal is to reverse thehypovolemia with crystalloid, which may besupplemented with 5% dextrose IV (given thehypoglycemia often seen in these patients). DR. JCDTTesting for cortisol should be obtained if adrenaldysfunction or hemorrhage is suspected, andglucocorticoids should be urgently administered. 46
  47. 47. Stress-dose glucocorticoids, either hydrocortisone50 mg every 6 hours or dexamethasone 4 mg every12 hours can be administered. Dexamethasone DR. JCDTdoes not interfere with the cortisol assay, andcorticotrophin stimulation can be performed afterthe patient receives dexamethasone. 47
  48. 48. Mineralocorticoid replacement with fludrocortisonemay be indicated in patients with a history ofbilateral, extensive adrenal hemorrhage in order to DR. JCDTreplace mineralocorticoid hormone requirementsbased on results of adrenal function testing or theclinical picture. 48
  49. 49. Therapy is unnecessary in (acutely ill)patients receiving more than 100 mg ofhydrocortisone daily, as this dose is DR. JCDTthought to provide adequatemineralocorticoid replacement. 49
  50. 50. In spite of adequate and aggressive treatmentwith fluids, glucocorticoids, antibiotictherapy, and ventilation, the patient in this casecontinued to decompensate and died 12 hours DR. JCDTlater due to multiorgan failure thought to besecondary toP aeruginosa septic shock. 50
  51. 51. Although the antibiotic selection on admissionwas effective against P aeruginosa latelyisolated, the initial selection of amoxicillin (whichis not effective in the treatment of this bacteria) DR. JCDTand the delay in recognizing the patients severesepsis may be responsible for the final outcomein this patient. 51
  52. 52. The diagnosis of WFS was later confirmed viaautopsy, wherein massive bilateral suprarenal andhemorrhagic effusion in other organs wasobserved. The adrenal glands and renal medullahad a macroscopically diffuse dark red color. DR. JCDTNo histologic signs of immunodeficiency werefound, but the liver and spleen were enlarged andfriable. 52
  53. 53. There were no signs of meningitis.Rapidly progressive vascular collapse andacute respiratory failure caused by DR. JCDTP aeruginosa septicemia (with lowerextremity cellulitis/ulcer being the portal ofentry) were considered responsible for thepatients multiple organ failure and cause ofdeath. 53
  54. 54. Review: You are examining an adult patient who yoususpect may be manifesting WFS. Which of thefollowing organisms would most likely be seen inthis patient?- H influenza DR. JCDT- M tuberculosis- N meningitidis- Cytomegalovirus 54
  55. 55. Correct answer:- H influenza- M tuberculosis DR. JCDT- N meningitidis ****- Cytomegalovirus 55
  56. 56. N meningitidis is the most frequent organismreported as a cause of WFS, although massivevaccination has decreased the incidence. DR. JCDTThe other organisms have also been identifiedin cases of WFS. 56
  57. 57. If you do in fact suspect a diagnosis of WFS in apatient you are examining, which of the followingcourses of action would be best?:- Antibiotics should be prescribed but only afterbacterial susceptibility confirmation DR. JCDT- Broad-spectrum antibiotics and supportive therapyshould be initiated as soon as WFS is suspected- Treatment should focus on normalizing the cortisoland aldosterone levels only- The patient should be referred to radiography for 57chest imaging
  58. 58. Correct answer:- Antibiotics should be prescribed but only afterbacterial susceptibility confirmation- Broad-spectrum antibiotics and supportive DR. JCDTtherapy should be initiated as soon as WFS issuspected ****- Treatment should focus on normalizing thecortisol and aldosterone levels only- The patient should be referred to radiography 58for chest imaging
  59. 59. Due to the fulminant course of thissyndrome, therapy should be started as soonas the diagnosis is suspected. DR. JCDTInitial antibiotic coverage should be broad. 59
  60. 60. Gracias por su atención DR. JCDT Dr. Juan Carlos Díaz Torre Pediatra Neonatólogo dr_diaz_torre@hotmail.com (779) 100 - 40 - 26 60

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