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Introduction: The number of infections due to uncommon yeast species is gradually increasing worldwide. In cases of high-risk paediatric patients, Cyberlindnera fabianii has been identified as the causal agent in a number of countries. In fact, we have recently reported the first proven occurrence, to the best of our knowledge, of this species as a causal agent of fungaemia in three neonatal patients in a Croatian hospital. Case presentation: We report here six new instances of clinically manifested bloodstream and urinary tract infections caused by Cyberlindnera fabianii in five high-risk neonates and one child, during a 5-year period (2008–2012) in the aforementioned Croatian hospital. In addition, we have provided an account of their treatment strategy and the outcome, and the susceptibility profiles of 44 isolates of Cyberlindnera fabianii to amphotericin B, flucytosine, triazoles and echinocandins. Furthermore, we have described a novel molecular method suitable for the rapid and specific diagnosis of this species. Conclusion: Our findings demonstrated: (i) the pathogenic activities of Cyberlindnera fabianii species in high-risk children; (ii) that administering fluconazole either prophylactically or therapeutically yielded no results in 50 % of the patients; (iii) that substituting fluconazole by liposomal amphotericin B or caspofungin managed to resolve sepsis in the remaining 50 % of patients; (iv) that, according to the examined sequences, all of the Cyberlindnera fabianii isolates were found to be identical independent of their source or study period; and (v) the existence of a higher proportion of non-susceptible isolates of Cyberlindnera fabianii for echinocandins (especially micafungin) compared with the other tested antifungals.

Refeeding syndrome (RFS) is a well-described state of the series of metabolic and biochemical changes that can occur during the feeding of malnourished persons. The shifts in fluids and electrolytes can lead to complications during artificial feeding, which if not recognised and untreated can lead to death. Although the physiology and pathophysiology of RFS is well known, the circumstances under which the RFS appears, clinical manifestations and management of these patients are less clear. There are few published studies describing the occurrence of RFS in children. We describe two cases of RFS in children. The first case is a boy with unrecognised coeliac disease and second case is a girl with cerebral palsy. In both cases, the RFS has developed without clinical symptoms and it was shown only through laboratory findings. Electrolyte disturbances have been successfully corrected and treatment of the underlying disease continued.

In recent years, an evident rise in the frequency of candidaemia caused by non-albicans Candida species has been reported. In this paper we present three cases of clinically manifested candidaemia caused by Candida utilis in neonatal patients hospitalized in the same neonatal intensive care unit within a 6 month period. To the authors’ knowledge, only two cases of C. utilis candidaemia have been reported in the literature to date, but neither of these involved newborns. Clinical resolution and elimination of C. utilis from the blood were achieved using liposomal amphotericin B or caspofungin in all patients.

Familial hemophagocytic lymphohistiocytosis (FLH) is an autosomal recessively inherited multisystem disease. This defect in cellular cytotoxicity is a life threatening condition characterized by fever, rash, splenomegaly, cytopenias and neurologic manifestations. PRF1, UNC13D and STX11 gene defects underlie in about 40-50% of primary cases. Chemoimmunotherapy followed by hematopoietic stem cell transplantation improved disease outcome. We report a case of a 6-week-old boy who presented with a fever, diffuse rash, disseminated intravascular coagulation, hypofibrinogenemia, hypertrigliceridemia, hepatosplenomegaly, leukocytosis with 90% of lymphocytes, granulocytopenia, anemia, trombocytopenia, hyperferritinemia and pathological findings in cerebrospinal fluid. The patient had decreased frequency of NK cells and low NK cell activity in peripheral blood. Bone marrow aspiration analysis showed degenerative changes of histocyte cells, with preserved cytophages (lymphophages and erythrophages) consistent with hematophagocytic syndrome. Given that the molecular diagnosis of the known mutations in genes PRF1 and UNC13D showed a mutation in UNC13D, the diagnosis of familial hemophagocytic lymphohistiocytosis subtype 3 was established. HLH-2004 chemotherapy protocol was performed and partial remission with residual central nervous system disease was achieved. Hematopoietic stem cell transplantation was successfully performed with an unrelated HLA-matched donor. Familiar HLH is generally a progressive and fatal disease. Early diagnosis with molecular genetic analysis and chemoimmunotherapy followed by hematopoietic stem-cell transplantation is the best approach.

We report a rare case of a child with epidermolysis bullosa simplex (EBS) with plectin deficiency but without muscular dystrophy, with severe lesions of the oral cavity, oropharyngeal, hypopharyngeal, laryngeal, tracheal and bronchial mucosa. Case report and a review of the world literature are used. The literature review revealed only five similar patients with EBS without muscular dystrophy complicated by respiratory involvement. This paper highlights the potentially serious complications of the EB in the form of breathing, swallowing and speech difficulties and describes the specific problems encountered in the treatment of this patient. Epidermolysis bullosa (EB) is a group of severe hereditary diseases, primarily of the skin, but which can also involve the respiratory and gastrointestinal tract mucosa. Respiratory tract involvement is usually only found in certain types of EB. The oral cavity and oropharynx are involved more frequently than the hypopharynx, larynx and trachea. Involvement of laryngeal and tracheal mucosa is generally associated with an increased morbidity and mortality, numerous complications and therapeutic difficulties, and is more common in junctional EB and dystrophic EB than in EBS. We present a rare case of a child with EBS and plectin deficiency with pronounced lesions of respiratory tract mucosa from the oral cavity to the bronchi and even extending into the trachea. Deciding on tracheotomy requires thorough consideration and should not be taken lightly.

We report on an unexpected extubation failure in a two year old boy. In our patient, failure of extubation led to a diagnostic examination. The result of our examination was discovery of a congenital anomaly of the aortic arch and great vessels (vascular ring) with compression of the trachea. The presence of this anomaly in this patient was unknown to us before. A successful surgical procedure eliminated the underlying cause of the persistent extubation failures.

Since traditional treatment of atelectasis is often insufficient to reopen the collapsed airways, mini-bronchoalveolar lavage (mini-BAL) is performed. We retrospectively compared the treatment effects of mini-BAL only and mini-BAL combined with surfactant in the treatment of pediatric ICU patients with recurrent atelectasis.