The Therapeutic Hypothermia in Treatment of Hyperammonemic Encephalopathy due to Urea Cycle Disorders and Organic Acidemias.

BACKGROUND: Hyperammonemic encephalopathy in newborns with urea cycle disorders and certain organic acidurias can cause severe brain injury, coma and death. Standard therapy includes protein restriction, nitrogen-scavenging drugs, prevention of catabolism and hemodialysis. Neuroprotective hypothermia as part of the treatment has been reported only 3 times. It has been suggested that mild systemic hypothermia can contribute to better neurological outcomes in hyperammonemic encephalopathy. However, the limited experience precludes accurate conclusions on safety and efficacy. METHODS: Whole body therapeutic hypothermia was included in the standard treatment of hyperammonemic encephalopathy in 4 neonates with urea cycle disorder or organic aciduria. RESULTS: Two patients survived the initial crisis. One patient has a developmental quotient of 0.8, while the other shows severe developmental delay. The cooling protocol had to be discontinued in 3 patients due to the otherwise untreatable complications (hypotension and hemorrhage). CONCLUSION: The efficacy and safety of therapeutic hypothermia in the treatment of neonatal hyperammonemic encephalopathy depend on various factors, requiring further evaluation.

Perinatal Health Statistics as the Basis for Perinatal Quality Assessment in Croatia.

CONTEXT: Perinatal mortality indicators are considered the most important measures of perinatal outcome. The indicators reliability depends on births and deaths reporting and recording. Many publications focus on perinatal deaths underreporting and misclassification, disabling proper international comparisons. OBJECTIVE: Description of perinatal health care quality assessment key indicators in Croatia. METHODS: Retrospective review of reports from all maternities from 2001 to 2014. RESULTS: According to reporting criteria for birth weight ≥500 g, perinatal mortality (PNM) was reduced by 31%, fetal mortality (FM) by 32%, and early neonatal mortality (ENM) by 29%. According to reporting criteria for ≥1000 g, PNM was reduced by 43%, FM by 36%, and ENM by 54%. PNM in ≥22 weeks' (wks) gestational age (GA) was reduced by 28%, FM by 30%, and ENM by 26%. The proportion of FM at 32-36 wks GA and at term was the highest between all GA subgroups, as opposed to ENM with the highest proportion in 22-27 wks GA. Through the period, the maternal mortality ratio varied from 2.4 to 14.3/100,000 live births. The process indicators have been increased in number by more than half since 2001, the caesarean deliveries from 11.9% in 2001 to 19.6% in 2014. CONCLUSIONS: The comprehensive perinatal health monitoring represents the basis for the perinatal quality assessment.

[Post-transplant erythrocytosis]. / Eritrocitoza nakon transplantacije bubrega.

Post-transplant erythrocytosis is defined as an increase in hematocrit above 55%. It occurs in 10%-15% of renal transplant recipients, most commonly from 8 to 24 months after transplantation. Twenty-five percent of patients experience spontaneous remission within 2 years, while 75% develop symptoms and signs of hyperviscosity (headache, hypertension, plethora). The etiology is multifactorial and includes erythropoietin, renin-angiotensin system (RAS) and IGF-1 as the main factors. RAS inhibition with either ACE inhibitors or angiotensin receptor blockers is efficient therapy which decreases hematocrit in 90% of patients within 2 to 6 weeks, thus decreasing the incidence of fatal complications (like pulmonary embolism and stroke).

[Post-transplantation anemia--diagnostic and therapeutic challanges]. / Posttransplantacijska anemija--dijagnosticki i terapijski izazovi.

Renal transplantation is a method of choice for treatment of patients with end-stage renal disease. Anemia may complicate post-transplantation course. It is a significant correctable risk factor for development of cardio-vascluar diseases. Based on the time of occurrence, early and late post-transplantation anemia (PTA) may be differentiated. Early PTA occurs immediately after renal transplantation. Risk factors for development of early PTA include blood loss during and after the surgery, inflammation, delayed graft function, and induction therapy with bone marrow suppression. Abrupt cessation of erythropoetin treatment may contribute to development of early PTA. The most common reason for development of late PTA is poor graft function with lack of erythropoetin or erythropoetin resistance. Etiology of PTA is commonly multifactorial and includes graft function, immunosuppressive drugs, infections and renin-angiotensin system. Treatment of anemia in renal transplant recipients demands an individual approach, with searching for the etiology of anemia. According to the current knowledge, renal transplant recipients with anemia should be treated in the same way as other patients with chronic renal failure.

[Cytomegalovirus infection in renal transplant recipients]. / Infekcija citomegalovirusom u bolesnika s transplantiranim bubregom.

Cytomegalovirus (CMV) belongs to the family of human herpes viruses. It is also known as the human herpes virus 5 (HHV-5). In immunocompromised host it becomes significant pathogen, causing the spectrum of different symptoms and affecting different tissues and organs. Epidemiologic forms of CMV infection include primary infection, reactivation or secondary infection, and superinfection or reinfection. CMV infection has direct and indirect effects. Direct effects occur at the time of highest viraemia with severe clinical presentation. To the contrast, indirect effects occur at the time of asymptomatic viraemia as the consequence of immunologic response. Indirect effects are mediated by cytokines, chemokines and growth factors. Diagnosis of CMV infection is based on virus detection in body fluids and tissues. There are several diagnostic methods for detection of CMV, and their use is primarily determined by the possibilities of the specific transplantation center. Regarding the risk of CMV infection, several categories of renal transplant recipients may be identified. The main factor for estimation of risk for development of CMV infection is donor and recipient serological status. The highest risk is associated with combination of CMV seropositive donor and CMV seronegative recipient (D+/R-). CMV infection was often fatal before introduction of potent antiviral drugs in therapeutic protocols. Contemporary treatment has significantly decreased mortality rate from the CMV infection. Several drugs are used for prevention and treatment of CMV infection: hyper immune gamma globulin, gancyclovir, valgancyclovir, valacyclovir and acyclovir, depending on the kind of treatment (prophylaxis or preemptive treatment). In the case of CMV disease, the best results may currently be achieved with the combination of hyper immune gamma globulin and intravenous gancyclovir.

[Clinical and epidemiological features of the mitral valve prolaps in children]. / Klinicka i epidemioloska obiljezja prolapsa mitralne valvule u djece.

The study deals with the incidence and clinical significance of mitral valve prolapse in the population of outpatient pediatric cardiology patients in the time period from November 1999 to April 2004. The sample included 1187 children of both sexes, 688 of whom had a structural cardiac anomaly (57.9%), and the prolapse was diagnosed in 51 children (4.3%), largely female (f:m = 7.5:1). The average age at establishing diagnosis was 12.4 +/- 2.9 years (range 6-19 years). The children were followed 3.1 +/- 0.9 years (1-4 years of age). In 25 children (49%) associated mitral valve insufficiency was found, mostly of the 1st degree (80%). During follow up, neither the progression of the insufficiency nor any other complication was observed (arrhytmia, tromboembolism) in any of the children. Dolichostenomely was found in 10 children (19.6%), and both the development of insufficiency (p = 0.464, df = 2, chi2 = 1.54) and the difference in constitution (p = 0.766, df = 4, chi2 = 1.83) were irrelevant of sex. Typical subjective symptoms were observed in 37 children (72.5%), 22 of whom were treated with beta-blockers (propranolol) (43.1%). The average age of the patients treated with 3-blockers (13.7 +/- 2.5 yr) was statistically different from the average age of untreated patients (11.5 +/- 2.9 yr), hence the probability of the influence of neurohormonal factors on the development of subjective symptoms in advanced puberty (p = 0.006, t = -2.86). The most common clinical symptom is chest-pain (95% of the group with stronger symptoms). When treated, the symptoms disappear in 82% of the patients. Mitral valve prolapse is the entity of favourable clinical course. The prophylaxis of infective endocarditis should be performed in the group with mitral insufficiency, and the children with stronger symptoms should be treated with beta-blockers.

[Permanent junctional reciprocating tachycardia (PJRT) and dilated cardiomyopathy]. / Trajna spojna kruzna tahikardija (PJRT) i dilatacijska kardiomiopatija.

We present 14-year-old girl with permanent junctional reciprocating tachycardia which was refractory to medicamentous therapy, who also had dilated cardiomyopathy. She underwent successful radiofrequent catheter ablation of accessory pathway after wich the histologic changes in the myocardium were observed in the form of compensatory hypertrophy of cardiac muscle (cardiac remodelling). The question of cause and consequence appeared: whether the arrhythmia is a consequence of dilated cardiomyopathy, or it is tachycardia- induced cardiomyopathy. This particular issue is discussed in this article. Based on the diagnostic procedure and complete recovery of myocardium after catheter ablation of accessory pathway, it is obvious that the tachycardia was due to tachicardiomyopathy, i.e. cardiomyopathy caused by permanent reciprocating junctional tachycardia.