Necrotizing haemorrhagic pneumonia proves fatal in an immunocompetent child due to Panton–Valentine Leucocidin, toxic shock syndrome toxins 1 and 2 and enterotoxin C-producing Staphylococcus aureus

Panton–Valentine leucocidin (PVL) toxin-producing strains of Staphylococcus aureus ( S. aureus ) are associated with skin abscesses and furunculosis, with necrotizing pneumonia being a relatively rare problem. Here, we describe a fatal case of necrotizing pneumonia in a 14-year-old child who presented initially with sore throat and pyrexia. He deteriorated rapidly, developing hypotension, multiple organ failure and purpura fulminans. S. aureus was isolated from the tracheal aspirate, which was found to be positive for PVL, toxic shock syndrome toxins (TSST) 1 and 2 and staphylococcal enterotoxin C (SEC). It was postulated that purpura fulminans and toxic shock syndrome were a result of the abovementioned exotoxins.
Conclusion: This case highlights the emergence of PVL-positive community-acquired S. aureus infection and association of purpura fulminans with superantigens. Practitioners should be aware of this illness in order to initiate appropriate treatment.     

Occult methicillin-resistant Staphylococcus aureus abscesses in 2 pediatric patients

Methicillin-resistant Staphylococcus aureus (MRSA) infections have become more common over the last decade. Recently, severe MRSA infections including necrotizing pneumonia, purpura fulminans, and rapidly progressive skin abscesses have been reported. These severe infections frequently have been associated with the virulence factor Panton-Valentine leukocidin (PVL). Two unusual cases of occult deep tissue abscesses in children who had family members with a history of severe MRSA skin abscesses are presented in this article. Both children initially presented with fever without a focus. Deep tissue MRSA (PVL positive) abscesses evolved. Empiric antibiotic therapy for MRSA should be considered for infants and children who are hospitalized with occult fever and who have a family member with a history of MRSA infection.     

Necrotizing pneumonia and arthritis due to Staphylococcus aureus producing Panton and Valentine leukocidin in a 10-year-old boy]

Staphylococcal necrotizing pneumonia producing the Panton Valentine leukotoxin (PVL) has been described for many years. The french reference center for staphylococcal toxaemia defined it with precision in 1999. A 10-year-old child, died in 36 hours from respiratory distress and shock. Staphylococcal pneumonia was suspected then confirmed: S. Aureus producing PVL was isolated in lung, blood and articulations.     

Effect of the Factor V Leiden mutation on the severity of meningococcal disease.

BACKGROUND: One of the most serious complications of meningococcal disease is the syndrome of purpura fulminans, which is characterized by intravascular thrombosis and hemorrhagic infarction of skin, limbs and digits. The reasons why some patients with meningococcal disease develop purpura fulminans while others have minimal thrombotic and skin involvement despite having profound septic shock are not yet understood. The Factor V Leiden mutation (FV(L)) is associated with thrombotic events, and we hypothesized that children carrying FV(L) who develop meningococcal disease may be at increased risk of purpura fulminans. METHODS: We determined the FV(L) genotype by PCR and restriction enzyme digestion (Mnl1) in 259 children with meningococcal disease and 80 healthy controls. In addition 79 parents of children with fatal meningococcal disease were studied. RESULTS: There was no significant increase in the frequency of FV(L) in patients with meningococcal disease (10%) as compared with healthy controls (9%) or with the parents of children who died of meningococcal disease (12%). Although the mortality was not increased in patients heterozygous for FV(L), they had increased complications of purpura fulminans, as assessed by requirement for skin grafting, referral to plastic surgeon and/or amputation. Among survivors 5 of 24 (21%) of those heterozygous for FV(L) had complications, compared with 14 of 233 (7%) who were wild type [P < 0.03; relative risk, 3.1 (95% confidence intervals, 1.2 to 7.9)]. CONCLUSIONS: FV(L) exacerbates purpura fulminans in meningococcal disease but does not have a significant effect on mortality.     

Frequency of adrenal hemorrhage in fatal forms of purpura fulminans in children. Etiopathogenic and therapeutic considerations

Between 1971 and 1985, 43 children died of purpura fulminans in our intensive care unit: 11 had autopsy and adrenal haemorrhage was observed in 8 (73%). All these patients had an extensive purpura and a severe disseminated intravascular coagulation. Our series confirms the findings of previous studies: 69 autopsies showed 51 cases (74%) of macroscopic adrenal haemorrhage. Adrenal haemorrhage may be only one manifestation of multiple system organ failure consecutive to septic shock; however, its association with low plasma cortisol levels (as previously reported) suggests that glucocorticoid replacement therapy should be reconsidered in purpura fulminans.     

Toxic shock syndrome caused by a strain of Staphylococcus aureus that produces enterotoxin C but not toxic shock syndrome toxin-1

An 8-month-old infant presented with pneumonia and pleural effusion associated with clinical manifestation of toxic shock syndrome. A Staphylococcus aureus strain isolated from the pleural fluid produced enterotoxin C, but not toxic shock syndrome toxin-1 or other enterotoxins. Acute and convalescent sera showed an antibody rise to enterotoxin C but not to toxic shock syndrome toxin-1. These findings support the possibility that enterotoxin C was the primary toxin associated with this infant's illness.     

Early diagnosis of staphylococcal toxic shock syndrome by detection of the TSST-1 Vbeta signature in peripheral blood of a 12-year-old boy

We report the case of a 12-year-old boy who developed staphylococcal toxic shock syndrome associated with S. aureus pharyngeal colonization or infection. The diagnosis was rapidly confirmed by detecting the Vbeta signature of the toxic shock syndrome toxin-1 in peripheral blood, based on transient T cell depletion rapidly followed by massive expansion of Vbeta 2-positive T cells.     

Pleuropulmonary complications of PVL-positive Staphylococcus aureus infection in children

It is increasingly recognized world-wide that Panton-Valentine leukocidin (PVL)-positive Staphylococcus aureus (PVL-SA) is associated with a highly aggressive and often fatal form of community-acquired pneumonia. We report four children who presented with severe pleuropulmonary complications due to infection by community-acquired methicillin-sensitive
S. aureus (CA-MSSA), producing PVL toxin. The complications included bilateral multilobular infiltrates, pneumatocoeles, recurrent pneumothoraces, pleural effusion, empyema, lung abscess and diaphragmatic paralysis. This case series highlights the diverse pleuropulmonary manifestations of this potentially lethal infection and the importance of heightened awareness, early recognition and aggressive therapy.
Conclusion:  Complicated pneumonia in a previously fit young patient with a history of preceding 'flu-like' illness or skin/soft tissue infection should raise the suspicion of infection by PVL-positive Staphylococcus aureus (PVL-SA). Severe pleuropulmonary complications are a feature of this disease.     

Meningococcal infections in children: a review of 100 cases

One hundred children with meningococcal infection diagnosed from January 1, 1985, to February 29, 1988, were reviewed. Clinical manifestations ranged from fever alone to fulminant septic shock with purpura fulminans. Twenty-nine percent of the children presented without skin lesions. Of the 55 patients with meningitis, 6 lacked cerebrospinal fluid abnormalities on initial lumbar puncture but cerebrospinal fluid cultures were positive. An overall case fatality rate of 10% was noted with the following poor prognostic indicators identified: hypothermia; seizures or shock on presentation; a total peripheral white blood cell count less than 5000/mm3; a platelet count less than 100,000/mm3; and the development of purpura fulminans. Meningococcal infections remain an important cause of morbidity and mortality in children. Infections caused by Neisseria meningitidis (including meningitis) should be considered even in the absence of skin lesions or cerebrospinal fluid abnormalities.     

Severe complications of chickenpox in hospitalised children in the UK and Ireland.

AIMS: To estimate the annual incidence of hospitalisations due to severe complications of varicella, describe the complications and estimate annual mortality. METHODS: Active surveillance throughout the UK and Ireland for 13 months by paediatricians notifying cases to the British Paediatric Surveillance Unit and completing a questionnaire. The case definition was any child aged <16 years hospitalised with complicated varicella, as defined by a list of conditions, or admitted to ICU/HDU with varicella. RESULTS: 188 cases were notified for the surveillance period, of which 112 (0.82/100 000 children/year) met the case definition and were not duplicates. Confirmed cases had a median age of 3 years (range 0-14). The complications were: bacteraemia/septic shock (n = 30), pneumonia (n = 30), encephalitis (n = 26), ataxia (n = 25), toxic shock syndrome/toxin-mediated disease (n = 14), necrotising fasciitis (n = 7), purpura fulminans/disseminated coagulopathy (n = 5), fulminant varicella (n = 5) and neonatal varicella (n = 3). 52 children (46%) had additional bacterial infections. Six deaths were due, or possibly due, to varicella, including one intrauterine death. Four of the other five children who died (ages 2-14 years) had a pre-existing medical condition. Sequelae on discharge were reported for 41 cases (40%), most frequently ataxia or skin scarring. The median length of hospital stay was 7 days (range 1-68). CONCLUSIONS: This study provides a minimum estimate of severe complications and death resulting from varicella in children in the UK and Ireland. Most complications, excluding deaths, occur in otherwise healthy children and thus would be preventable only through a universal childhood immunisation programme.     

A rare association between group A Streptococcus purpura fulminans and diffuse alveolar hemorrhage in a young girl: A case report

We report the case of a 7-year-old girl with septic shock and coagulopathy associated with purpura fulminans (PF) and diffuse alveolar hemorrhage (DAH) due to group A Streptococcus (GAS) infection identified with 16S ribosomal RNA analysis performed on the skin biopsy. GAS infection with PF associated with DAH is rare in healthy young children but pediatricians should be aware of this condition because of the poor prognosis. The initial treatment for circulatory failure and severe disseminated intravascular coagulation as well as the prompt initiation of antibiotic treatment may be crucial for the outcomes of S. pyogenes PF.     

Meningokokkensepsis und Purpura fulminans Erfolgreiche Lysetherapie mit rekombinantem Gewebeplasminogenaktivator

Summary A case of a 16 years old young man with meningococcemia, septic shock and purpura fulminans is presented. The clinical course was complicated by ischemia of both lower legs and feet as well as both cheeks. After successful restoration of circulation, the lower legs remained ischemic and loss was imminent. Therefore, fibrinolytic therapy with recombinant tissue plasminogen activator (rt-PA) was started: Both lower legs and feet could be preserved. All of the toes of the right foot, however, had to be amputated. Discussion: Fibrinolytic therapy with rt-PA should be considered as a therapeutic option in patients with meningococcemia and purpura fulminans with imminent loss of extremities. Optimal perfusion pressure and control of coagulation seems to be a prerequisite for successful fibrinolytic therapy. Care should be taken to avoid complications, especially intracerebral hemorrhage.      

Toxic shock syndrome in a 6-year-old male

Toxic shock syndrome, caused by an exotoxin of staphylococcus aureus is very rare in children. On admission, beside the shock, abdominal problems as vomiting, diarrhoea and a developing adynamic ileus were outstanding in our patient. Not before additional symptoms as staphylococcal pneumonia with bacteriemia occurred and later desquamation of palms and feet, diagnosis of toxic shock syndrome could be confirmed.     

Meningococcal purpura fulminans treated with medicinal leeches.

INTRODUCTION: Meningococcal septicemia remains one of the most common infectious causes of admission to a pediatric intensive care unit. Numerous treatment strategies aimed at the thromboembolic complications inducing purpura fulminans and limb/digital ischemia have been attempted, with variable results. The successful use of medicinal leeches for pneumococcal purpura fulminans has been described, and we present a similar case of meningococcal purpura fulminans. PATIENT AND INTERVENTION: A 5-wk-old female infant with meningococcal meningitis and septicemia and progressive purpura fulminans of the left hand was treated with medicinal leeches. Medicinal leeches were applied to the left dorsal hand on a daily basis for 4 consecutive days. RESULT: The swelling and limited functionality visibly improved after 48 hrs, and by 120 hrs, perfusion in the distal phalanges of the thumb and middle finger was evident. Reperfusion of the distal phalanges was not fully sustained, and at 6 wks the plastic surgery department debrided the distal phalanges of her left hand, excluding the thumb. She fully recovered from the meningococcal septicemic shock; the functionality of her left thumb was preserved, and she has limited functionality of her left hand. CONCLUSION: The unique combination of salivary products in leech therapy has theoretical benefits and requires future study.     

Pathophysiology of meningococcal meningitis and septicaemia

Neisseria meningitidis is remarkable for the diversity of interactions that the bacterium has with the human host, ranging from asymptomatic nasopharyngeal colonisation affecting virtually all members of the population; through focal infections of the meninges, joints, or eye; to the devastating and often fatal syndrome of meningococcal septic shock and purpura fulminans.     

Postinfection purpura fulminans in a patient heterozygous for prothrombin G20210A and acquired protein S resistance

Purpura fulminans usually consists of large, often symmetrical, spreading ecchymosis, which may later develop into extensive areas of skin necrosis and peripheral gangrene. Postinfectious purpura fulminans associated with an autoantibody directed against protein S has been described. The interaction and the contribution of recently described mutations such as factor V Leiden and prothrombin G20210A to the development and progression of postinfectious purpura fulminans and venous thrombosis is not known. The authors describe a patient heterozygous for prothrombin G20210A who developed purpura fulminans and extensive venous thrombosis secondary to acquired protein S deficiency.     

Pathophysiology of meningococcal meningitis and septicaemia.

Neisseria meningitidis is remarkable for the diversity of interactions that the bacterium has with the human host, ranging from asymptomatic nasopharyngeal colonisation affecting virtually all members of the population; through focal infections of the meninges, joints, or eye; to the devastating and often fatal syndrome of meningococcal septic shock and purpura fulminans.     

Successful use of extracorporeal membrane oxygenation in severe necrotizing pneumonia caused by Staphylococcus aureus.

OBJECTIVE: To report the successful use of extracorporeal membrane oxygenation (ECMO) as rescue therapy for severe necrotizing pneumonia secondary to infection by the Staphylococcus aureus species. DESIGN: Case series. SETTING: Pediatric intensive care unit at a freestanding tertiary care children's hospital. PATIENTS: Two pediatric patients with severe S. aureus-induced necrotizing pneumonia requiring rescue with ECMO. Both patients survived with good neurologic outcomes. One patient required the use of activated factor VII for severe bleeding while on ECMO, with no thrombotic effect on the ECMO circuit. CONCLUSION: ECMO as rescue support should be considered in a timely fashion for refractory hypoxemic respiratory failure resulting from S. aureus pneumonia, including patients with necrotizing pneumonia. Use of ECMO support in such cases, coupled with aggressive measures aimed at minimizing bleeding, such as the use of activated factor VII, may result in excellent short- and long-term outcomes for such patients.     

Homozygous protein C deficiency: early treatment with warfarin

We present a case of homozygous protein C deficiency with neonatal purpura fulminans and disseminated intravascular coagulopathy (DIC) starting shortly after birth. In addition, the infant had vitreal eye hemorrhages and intraparenchymal brain infarction, apparently as intrauterine events. Within 15 hours of institution of fresh frozen plasma (FFP) infusions the DIC resolved and the progression of purpura fulminans reversed. Warfarin (0.4 mg/kg/day) was started on the fifth day of life, followed by gradual tapering of the FFP infusions. There were no recurrences of purpura, areas of skin necrosis healed without the need for skin grafting, and the areas of brain infarction resolved without apparent sequelae. The eye and brain lesions may be intrauterine events and appear to be a regular feature of this syndrome. Family studies are essential to establish the diagnosis, although there may be no family history of thromboembolic events, as in this case. Homozygous protein C deficiency is a rare disorder, but one in which early recognition and intervention may be lifesaving. Ours is the youngest patient yet reported to be treated with warfarin anticoagulation. We were thus able to avoid the complications of long-term plasma therapy as well as the potential thrombotic complications of central venous catheter placement.     

Topical nitroglycerin and pain in purpura fulminans

Topical nitroglycerin has been previously described as an adjunctive therapy to increase perfusion to areas of purpura affected in purpura fulminans. We report a case of purpura fulminans in which topical nitroglycerin was found to provide analgesia after its application to purpuric lesions. The broader role for the use of topical nitroglycerin in pain management deserves further study and evaluation.