Approach to red blood cell transfusions in post-operative congenital heart disease surgery patients: when to stop?

BACKGROUND: The best transfusion approach for CHD surgery is controversial. Studies suggest two strategies: liberal (haemoglobin ≤ 9.5 g/dL) and restrictive (waiting for transfusion until haemoglobin ≤ 7.0 g/dL if the patient is stable). Here we compare liberal and restrictive transfusion in post-operative CHD patients in a cardiac intensive care unit. METHODS: Retrospective analysis was conducted on CHD patients who received liberal transfusion (2019-2021, n=53) and restrictive transfusion (2021-2022, n=43). RESULTS: The two groups were similar in terms of age, gender, Paediatric Risk of Mortality-3 score, Paediatric Logistic Organ Dysfunction-2 score, Risk Adjustment for Congenital Heart Surgery-1 score, cardiopulmonary bypass time, vasoactive inotropic score, total fluid balance, mechanical ventilation duration, length of cardiac intensive care unit stay, and mortality. The liberal transfusion group had a higher pre-operative haemoglobin level than the restrictive group (p < 0.05), with no differences in pre-operative anaemia. Regarding the minimum and maximum post-operative haemoglobin levels during a cardiac intensive care unit stay, the liberal group had higher haemoglobin levels in both cases (p<0.01 and p=0.019, respectively). The number of red blood cell transfusions received by the liberal group was higher than that of the restrictive group (p < 0.001). There were no differences between the two groups regarding lactate levels at the time of and after red blood cell transfusion. The incidence of bleeding, re-operation, acute kidney injury, dialysis, sepsis, and systemic inflammatory response syndrome was similar. CONCLUSIONS: Restrictive transfusion may be preferable over liberal transfusion. Achieving similar outcomes with restrictive transfusions may provide promising evidence for future studies.

Living Donor-Initiated Domino Split-Liver Transplantation in Pediatric Setup: A Case Report With Literature Review.

BACKGROUND: In patients undergoing liver transplantation for metabolic diseases, removing the patient's liver for transplantation to another recipient is called "domino liver transplantation." The extracted liver can be divided and transplanted into 2 recipients, which is called domino split-liver transplantation in the literature. However, in our study, the domino liver was obtained from a pediatric patient. METHODS: A patient with maple syrup urine disease (MSUD) underwent a living donor liver transplant, and the explanted liver was divided in situ into right and left lobes and transplanted to 2 separate patients. Demographic data, surgical techniques, postoperative period, and patient follow-ups were evaluated. RESULTS: The father's left lobe liver graft was transplanted into a 12-year-old boy with MSUD. The removed liver was divided in situ into right and left lobes. The left lobe was transplanted to a 14-year-old male patient, whereas the right lobe was transplanted to a 67-year-old male patient. The donor and the first recipient were discharged on postoperative days 5 and 22. The second pediatric patient who underwent domino split-left lobe transplantation was discharged on postoperative day 23. The adult patient who underwent domino split-right lobe transplantation died on postoperative day 12 owing to massive esophageal variceal bleeding. CONCLUSION: Patients who underwent liver transplantation due to MSUD are among the best donor choices for domino liver transplantation. If the extracted liver has a sufficient volume and anatomic features for a split, it can be used in "selected cases."

Acute Fulminant Hepatic Failure Caused by Sevoflurane Linked to Influenza A.

Sevoflurane is a volatile anesthetic agent that does not tend to cause clinically significant hepatotoxicity, but there are some reported hepatotoxicity cases in the literature. In the case presented here, adenotonsillectomy was performed during influenza infection, and sevoflurane was administered, after which acute fulminant hepatitis developed. At hour 24 of hospitalization after fulminant hepatic failure, liver transplant was performed in a 3.5-year-old patient without any known diseases. In such cases, etiology investigations should be planned, life support therapy should be administered, and information should be given to the patient to avoid exposure to sevoflurane in the future.

Fontan procedure in patients with preoperative mean pulmonary artery pressure over 15 mmHg.

BACKGROUND: Several factors affect the long-term outcome of Fontan procedure, but a high pulmonary artery pressure is still one of the most important limitation for proceeding to a Fontan circulation. Herein, we present our experience in Fontan patients with high preoperative pulmonary artery pressures. METHODS: A retrospective analysis was performed to evaluate Fontan patients with a preoperative pulmonary artery pressure >15 mmHg between 2009 and 2020. Sixteen patients were operated on with a mean preoperative pulmonary artery pressure of 17.5 ± 2.1 mmHg. RESULTS: Mean age at the time of Fontan procedure was 7.8 ± 5.6 years. All the patients had stage 2 cavopulmonary anastomosis before Fontan completion, with a mean interstage period of 4 ± 2.6 years. Fontan completion was achieved with a polytetrafluorethylene tubular conduit, two of which were intra-extracardiac. Fenestration was performed in five (31%) cases. Postoperative pulmonary artery pressures and arterial oxygen saturation levels were 11.2 ± 2.8 and 97.8 ± 2 mmHg, respectively. Mean duration of pleural drainage was 3.9 ± 5.3 days. Any morbidity and mortality were not encountered during a mean follow-up period of 4.8 ± 7.7 years. CONCLUSIONS: The midterm results of stage 3 Fontan completion in patients with pulmonary artery >15 mmHg are encouraging. Not only the mean pulmonary artery pressure but also the pulmonary vascular resistance may be helpful in order to identify the high risk patients before Fontan completion.

An intoxication mimicking brain death: baclofen.

Baclofen is a derivative of gamma-aminobutyric acid, used mainly for the treatment of muscle spasticity. Baclofen overdose can result in severe respiratory depression, autonomic disturbances, seizures and coma. Here we report a 15-year-old girl who was found unresponsive, intubated and admitted to the PICU. On initial presentation, her Glasgow Coma Score was 3, with fixed dilated pupils. EEG revealed cerebral bioelectric activity and ground amplitudes significantly lower than normal. Supportive treatments were administered. On the 2nd PICU day, she regained consciousness and was able to follow commands. She was extubated and discharged on hospital day 3. Conclusively emergency physicians should consider baclofen overdose in children presenting with acute loss of consciousness, flaccidity, and hyporeflexia.

Fecal calprotectin: can be used to distinguish between bacterial and viral gastroenteritis in children?

OBJECTIVE: Fecal calprotectin is used as a good indicator of intestinal mucosal inflammation. The aim of this study is to evaluate the diagnostic value of fecal calprotectin (f-CP) for the etiology of acute gastroenteritis in children. MATERIALS AND METHODS: All patients presenting with acute diarrhea (<18 years) who had 3 or more soft or watery stools per day were enrolled in this study. Stool microscopic examination and cultures for bacteria and parasites were performed. Polymerase chain reaction test was also applied to stool samples for viruses (Rotavirus, Adenovirus, Norwalk, and Astrovirus). The level of f-CP was carried out by using enzyme-linked immunosorbent assay test. RESULTS: Eighty-four patients with diarrhea were enrolled. The f-CP level was higher in patients with microscopic examination positive (n=17) (median with interquartile range, 1610.0 [908.8-2100] mg/L) than in patients with microscopic examination negative (n=67) (123.8 [25.0-406.3] mg/L) (P<.001). Concentrations of f-CP in patients with stool culture positive (1870.0 [822.5-2100] mg/L) were significantly elevated compared with the concentrations of the patient with virus detected in stool (95.0 [21.3-240.9] mg/L) (P<.001). In the diagnosis for bacterial acute gastroenteritis, the area under the receiver operating characteristic curve for f-CP was 0.867 (95% confidence interval, 0.763-0.971), sensitivity was 88.9%, and specificity was 76.0% if the threshold was taken as 710 mg/L. CONCLUSION: We conclude that f-CP, which is useful, valuable, noninvasive, easily and rapidly measured laboratory test along with simple microscopic examination of stool, can be used as an indicator of intestinal inflammation and to distinguish the bacterial gastroenteritis from the viral gastroenteritis.

An examination of the mutual effects of valproic acid, carbamazepine, and phenobarbital on 25-hydroxyvitamin D levels and thyroid function tests.

BACKGROUND: The effects of antiepileptic drugs (AEDs) on bone metabolism and the endocrine system are not fully known, and publications on the subject are inconsistent. OBJECTIVE: The study aimed to examine the mutual effects of valproic acid (VPA), carbamazepine (CBZ), and phenobarbital (PB)-AEDs frequently used in childhood-on bone mineral metabolism and thyroid function tests. PATIENTS AND METHODS: Children monitored with a diagnosis of idiopathic epilepsy by the pediatric neurology clinic, using AEDs for at least 6 months and with episodes under control, were included in the study. Patients were divided into groups on the basis of the drugs used. Thyroid function tests and 25-hydroxyvitamin D or 25(OH)D levels were measured from blood specimens. The data obtained were then compared with those of the control group. RESULTS: A significantly high level of subclinical hypothyroidism was seen in patients using VPA (p < 0.001). There was no significant difference between any of the three study groups and the control group in terms of 25(OH)D (p > 0.05). CONCLUSIONS: Pediatric patients using AEDs, particularly VPA, should be monitored for subclinical hypothyroidism. VPA, CBZ, and PB have no effect on vitamin D levels.

Temporary diazepam responsive apneic attacks and congenital myasthenic syndrome.

Congenital myasthenic syndromes are a genetically and phenotypically heterogeneous group of hereditary disorders affecting neuromuscular junction. Mutations in the gene encoding choline acetyltransferase cause presynaptic defects. The missense mutation I336T has been identified in Turkish population, and most of the cases carrying this mutation present with exercise-induced fatigability and ptosis. Although apneic attacks occur in these cases during febrile illness in childhood, the number of reported respiratory distress episodes during infancy is scarce. Another important feature of these cases is that response to esterase inhibitors is satisfactory. We present a case of congenital myasthenic syndrome with I336T choline acetyltransferase mutation who presented with numerous attacks of respiratory distress in the infancy period. Interestingly, the patient had myopathic findings on electromyography and diazepam decreased severity of apneic attacks. There was also no improvement with esterase inhibitors.