Hypoxic hepatitis as a complication of newly diagnosed type 1 diabetes in a teenager.

Hypoxic hepatitis is a rare complication of type 1 diabetes with unknown prevalence in Pediatrics. We present a case report of an 11-year-old boy admitted to the ER in the spring of 2020 (the beginning of the COVID19 pandemic in Poland) due to nausea, abdominal pain, and weight loss. A diagnosis of type 1 diabetes accompanied by severe ketoacidosis (pH 6.9, blood glucose 632mg/dl, ketone bodies in urine - 150mg/dl) was made. The hyperglycemia, ketoacidosis, and water-electrolyte disturbances were treated in the Pediatric Intensive Care Unit. On day 4, the boy developed fulminant septic shock with high aminotransferases (AST 9026 U/l, ALT 3559 U/l). CT scan revealed hepatic enlargement and steatosis. Acute viral hepatitis was suspected. The levels of anti-CMV IgM and IgG antibodies were slightly elevated. At autopsy, the liver was enlarged, with petechial bleedings on the surface. The liver parenchyma was congested, with signs of steatosis. Microscopically, there was extensive centrilobular necrosis, acute passive sinusoidal congestion, and steatosis of hepatocytes. There were no signs of CMV infection. Based on the entire clinicopathological picture, the patient was diagnosed with hypoxic hepatitis, complicated by septic shock and multiple organ failure.

Guidelines regarding ineffective maintenance of organ functions (futile therapy) in paediatric intensive care units.

In Poland, guidelines for the management of ineffective treatment of children in neonatal and paediatric departments developed by the Polish Neonatal Society and the Polish Paediatric Society, have been published. The specific problems of futile therapy in paediatric anaesthesiology and intensive care units should be defined and solved separately. For this purpose, the guidelines presented below were prepared. They present the principles for managing children for whom therapeutic options available in paedia-tric anaesthesiology and intensive care units have been exhausted and ineffectiveness of maintaining organ functions, i.e. futile therapy, has been suspected. The decision to withdraw futile therapy of a child is undoubtedly one of the most difficult for both doctors and parents, and for this reason, it should be made collectively, respecting the dignity of the child and his/her parents or legal representatives, and continuing the management aimed at relieving the child's pain and suffering, as well as minimising anxiety and fear. Due to the small amount of reliable evidence-based data, the guidelines constitute the consensus of the Group of Experts and are dedicated to minor patients treated in paediatric anaesthesiology and intensive care units.

The use of plasmapheresis in a 4-year-old boy with toxic epidermal necrosis.

BACKGROUND: Toxic epidermal necrosis (TEN) is a severe and life-threatening syndrome manifesting as extensive necrosis of the epidermis and mucous membranes accompanied by systemic symptoms. The causative factor is exposure to drugs, such as sulphonamides, antiepileptic preparations, non-steroidal anti-inflammatory drugs and paracetamol. The mechanism leading to the development of lesions is unknown and there is no uniform strategy of management. CASE REPORT: A 4-year-old boy was admitted with late-stage TEN; he was additionally affected by partial atrophy of the corpus callosum, mental retardation and drug-resistant epilepsy. Three weeks before the first symptoms developed, antiepileptic treatment was widened with lamotrigine, which seems to be the causative factor of TEN. Since general and topical pharmacological therapy failed, plasmapheresis was applied and already after 2 cycles, the progression of lesions was inhibited and circulation was gradually stabilized. CONCLUSIONS: The use of plasmapheresis in a child with TEN can result in substantial improvement of the general and local condition. The case presented clearly indicates that standards of management of children with suspected or developed TEN should be urgently instituted.

Toxic epidermal necrolysis.

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN), also known as Lyell's syndrome, are rare, life- -threatening diseases that are characterised by extensive epidermal detachment, erosion of mucous membranes and severe systemic symptoms. In the majority of cases, the development of symptoms can be attributed to the use of drugs; therefore, the disease pathology is thought to be caused by a severe adverse reaction to drugs. The high mortality rate results primarily from the development of complications in the form of systemic infections and multiple organ failure. TEN and SJS affect all age groups, including newborns, infants and older children. The rarity of these syndromes has not permitted large, randomised studies, which has resulted in numerous difficulties in their diagnosis and management. Because the pathogenesis has not yet been established, the management and systemic treatment of these syndromes have not been standardised. The efficacy of the treatment options suggested has not been confirmed by clinical studies involving suitably large groups of patients, especially children.