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Signa Vitae

Journal of Intensive Care and Emergency Medicine

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Hereditary angioedema type I in a female patient: a case report


Hereditary angioedema (HAE) is rare autosomal dominant disease, characterised by spontaneous and recurrent swellings in various parts of the body. The main inflammatory factor in HAE is bradykinin (a key mediator of non-allergic angioedema) and it is responsible for capillary leak. C1 esterase inhibitor (C1-INH) is a protease inhibitor that blocks the activation of the classic complement pathway, but there are also many others biochemical pathways, including kinin. Type I HAE is defined by low plasma levels of a normal C1 inhibitor (C1-INH).

Case presentation. A 34-year-old female patient presented to the hospital complained of swollen and painful legs, flatulence, palpebral and labial edema, dyspnea, dysuria, frequent herpetic infections of mouth and nose, subfebrile body temperature and was hoarse. Over the last 20 years she suffered from occasional edemas of the extremities and abdomen. Laboratory testing showed a reduced level of C1-INH, and with other test results normal we diagnosed the patient with HAE type I. For emergency situations, we prescribed icatibant (B2 bradykinin receptor antagonist) subcutaneously.

Individuals with HAE report episodic attacks during childhood that become more severe during adolescence. There is non-pitting edema in 3 predisposed places : subcutaneous tissue, the abdomen and the larynx. Edemas amplify during 12-24 hours and disappear during 3-5 days, with migrations to other locations. Treatment can be prophylactic, for acute attacks (icatibant) and before medical treatments.

HAE is a potentially lethal disease and should be considered if repeating edema of various body parts, painful swelling and tightening of the skin are present.

Keywords: hereditary angioedema, bradykinin, B2 bradykinin receptor antagonist

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Multiple myeloma presenting with lower extremity gangrene and hyperviscosity syndrome


Hyperviscosity syndrome and cryoblobulinemia associated with lymphoproliferative disorder is a rare but life threatening condition. The delay of diagnosis can lead to severe mutilation and multiple organ damage. The plasma exchange therapy and the targeted treatment of the underlying disorder can lead to significant improvement. We present a patient who developed extensive soft tissue necroses, mimicking the peripheral artery disease. Despite surgical treatment, the skin lesions progressed involving fingers, earlobes and scrotum. Finally, the patient was diagnosed with multiple myeloma and hyperviscosity syndrome. The clinical condition improved after plasma exchange and myeloma treatment with thalidomide and dexamethasone.

Key words: multiple myeloma, hyperviscosity, skin necrosis

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Induced hypothermia in post-resuscitation cardiac care

Cardiac arrest and ROSC (return of spontaneous circulation) is a case of whole body ischaemia and subsequent reperfusion injury. Mild hypothermia, defined as core body temperature 32-34 °C has been shown to attenuate and ameliorate deleterious temperature sensitive mechanisms, thereby contributing to protection of the brain and heart. Primary indication (2010th AHA/ACC Guidelines For Cardiopulmonary Resuscitation) is in out-of-hospital cardiac arrest when initial rhythm was ventricular fibrillation or pulseless ventricular tachycardia (Class I, LOE B); in in-hospital cardiac arrest including all other rhythms (asystole and PEA) when, after return of spontaneous circulation, there is no neurologic response (“lack of meaningful response to verbal commands”).

The goal is to achieve IT (induced hypothermia) within 6 hours (starting within 10 min after return of spontaneous circulation) and to maintain it for 12 to 24 hours. Cooling methods: internal- intravenous infusion of 4 °C isotonic saline and external- ice packs, cooling blankets, cooling vests, cold water immersion.

Shivering raises body temperature and must be suppressed. High doses of sedatives must be titrated to suppress shivering (continuous infusion of propofol and fentanyl).

Core body temperature should be closely monitored by esophageal, bladder and rectal temperature probes. After 12-24 hours of maintaining IT the temperature should be raised gradually (at a rate of 0.2-0.25 °C per hour). Rapid rewarming (>0.5° C/hour) eliminates the benefits of IT.

Adverse effects of therapeutic hypothermia are impaired coagulation, increased risk of infection, slowing of cardiac conduction, bradycardia, QT prolongation, “cold diuresis”- hypovolemia, hypokalemia, hypomagnesaemia, hypophosphatemia.

We suggest more prone implementation of induced hypotermia in cardiac ICU due to the ERC statement, “Hypothermia is safe and effective even if there is lack of experience”.

Hyperbaric oxygen as a treatment for meningococcal purpura fulminans sequelae

Purpura fulminans (PF) is a life-threatening condition characterised by rapidly progressive hemorrhagic infarction of the skin leading to necrosis caused by disseminated intravascular coagulation and occlusion of dermal vessels. The most common acute infection with which PF is associated is meningococcal sepsis (MS). Management includes aggressive resuscitation, volume expansion, antibiotics, inotropic and ventilatory support, correction of electrolyte and acid-base abnormalities, steroids and blood derivatives. In skin necrosis and compartment syndrome early surgical intervention is often needed. Gangrenous changes may necessitate skin grafting and/or amputations. Hyperbaric oxygen therapy (HBOT) is considered a conservative treatment option.

We present cases of two infants that have survived MS with PF.

In a five-month old boy with extensive skin lesions and gangrene of fingers and toes surgical intervention was delayed due to his poor condition. On day 10 HBOT was started and a total of 52 treatments were carried out. During the treatment MRSE superinfection was suppressed, necrotic areas became dry and delineated, most of them detached spontaneously, wounds healed and after completion of HBOT there was no need for surgical intervention.

A seven-month old boy had multiple skin necrosis and necrosis of the distal phalanges of fingers. In consultation with a surgeon, HBOT was indicated. Because of the malfunction of the hyperbaric oxygen chamber in our hospital, on the ninth day of illness the infant was transferred to the University Hospital Split where a total of 30 HBOTs were carried out. Shallow lesions healed spontaneously, but some of the deepest and the most extensive ones required debridement or skin grafting.

HBOT is a treatment in which a patient breathes 100% oxygen while inside a treatment chamber at a pressure higher than sea level. It is considered an adjunct treatment for conditions like non-healing ulcers, problem wounds and compartment syndrome. Its therapeutic effects include neovascularisation, immune stimulation, bactericidal effect and reduction of edema. In PF it may be of benefit during the phase of acute arterial ischemia, during resolution of ischemia perfusion injury and during the healing phase. In two presented cases HBOT was used to treat sequelae of PF during MS. In the first case after HBOT surgical intervention was avoided and in the second one it was required only for the more extensive lesions.

Retrofaringeal abscess and acute dilatation of the left coronary artery in neonatal staphylococcal sepsis

We report a case of neonatal staphylococcal sepsis presented with retropharyngeal abscesses, mediastinitis, infection of chest wall soft tissue, myopathy, cardiorespiratory failure, acute dilatation of the left coronary artery and echocardiographic signs of Kawasaki syndrome.

A female newborn hospitalised at 18th day of age due to SOB, groaning, cyanosis and a limp body – the third child from a second regular pregnancy, term delivery, vaginal birth, birth body weight of 3780 g, Apgar score 9/10. She was discharged as healthy at the 5th day of age after treatment with phototherapy for hyperbilirubinemia over 66 hours period with concomitant asymptomatic S. aureus nasopharyngeal colonization. Parents are healthy and have healthy two-year old twins, negating hereditary diseases. At home, on a natural diet, the parents noticed flabbiness of the newborn. Other symptoms appeared suddenly upon admission.

Staphylococcus epidermidis is common cause of neonatal sepsis in our hospital. This is the first time we dealt with the above localization and complications. Although literature describes cases of neonatal Kawasaki syndrome, we were suspicious this was the case with our patient because she was in sepsis, and in addition to dilatation of left coronary artery, endocarditis vegetations were observed. Also observed expansion increased after immunoglobulin treatment. Serology for Epstein-Barr, Coxsackie and Parvovirus (IgM and IgG) was positive, and there was early complication of staphylococcal sepsis by candidiasis. Due to pressure on the trachea and pneumonia with pneumatocela, respiratory failure followed and three-weeks of mechanical ventilation ensued. Severe upper extremities myopathy developed due to retropharyngeal abscess compression on the cervical spinal medulla at the C1 and C2 level. With antistaphylococcal treatment and several retropharyngeal abscess evacuations, patient was extubated after three weeks. Given that myopathy remained after six weeks of therapy, higher institution (IZZMD-Beograd) was consulted. It was determined that the compression of the spinal cord was due to spinal canal stenosis as a result of destruction and dislocation of atlantoaxial vertebrae (Grisel’s syndrome), caused by osteomyelitis. Compression resulted in quadriparesis and neurogenic bladder. Eight-week antibiotic treatment was initiated that successfully handled the infection. Due to atlantoaxial subluxation initially a Tomas collar was used to stabilise the spine and surgical treatment at the Clinic of Neurosurgery in Warsaw followed. The patient’s neurological status remained unchanged and was discharged. She was again hospitalised 28 days after surgery because of severe respiratory insufficiency. The patient remains on mechanical ventilation and still suffers from severe myopathy.

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