Vol 48, No 4 (2020)

Rationale: Functional gastrointestinal disorders (FGID) are highly prevalent in children worldwide. Current concept of FGID pathophysiology implies contributions from various mechanisms. Information in the role of acute respiratory infections in the development of FGID in children is lacking.

Aim: To determine the prevalence of FGID in children hospitalized to the department of infectious diseases with acute respiratory tract infections (ARTI) and their association with demographic, medical biological factors and clinical manifestations.

Materials and methods: We performed observational and cross-sectional study with continuous patient sampling. The study included 180 ARTI patients, whose mothers agreed to fill in the proposed questionnaire of 41 items. The diagnosis of FGID during in-hospital stay was made based on clinical data in accordance with the Rome criteria IV (2016) and by standard collection of the information from the parents. The stool characteristics were assessed with the Bristol scale. The rate of recurrent ARTI was assessed by the resistance index of above 0.5. We considered clinical symptoms of ARTI at the admittance, the rates of bacterial complications and the need in antibacterial treatment.

Results: The prevalence of FGID in hospitalized children with ARTI below 4 years of age (n = 134) was 35.1% (95% confidence interval [CI]) 27.0– 43.9); they had higher rates of functional diarrhea (14.9%, 95% CI 9.4–22.1) and constipation (14.2%, 95% CI 8.8–21.3). Among those above 4 years of age (n = 46), FGID were diagnosed in 50% (95% CI 34.9–65.1) of the cases; with most frequent symptoms being abdominal pain (39.1%, 95% CI 25.1–54.6) and irritable bowel syndrome (10.8%). Irrespective of their age, the children with FGID had higher rates of ARTI recurrence (by 34.0%, 95% CI 20.0–48.1; relative risk 1.98, 95% CI 1.47–2.69; odds ratio 4.13, 95% CI 2.18–7.85) and intestinal infections in their past history, compared to those without any gastrointestinal symptoms during the index hospitalization. In children with FGID, ARTI were more frequently complicated by otitis and they had higher requirement in antibiotics. The mothers of the infants and toddlers with FGID had lower educational level (smaller proportion of those with high school education) and were less satisfied with their home and living conditions, whereas their fathers were older and more frequently employed in vehicle operations and less frequently were occupied in communication-related professions.

Conclusion: Acute respiratory infections, especially recurring, may plaу a significant role in the development and maintenance of FGID in children. Further studies are necessary, which may result in useful information for the development of tailored strategies to treatment and prevention.

Hemolytic uremic syndrome (HUS), one of the forms of thrombotic microangiopathy, is a severe emergency with non-immune (Coombs negative) anemia, thrombocytopenia and acute renal injury. HUS is heterogeneous, and its most common form, the typical HUS, is associated with Shiga toxin (Stx) producing bacteria, such as Escherichia coli, Shigella dysenteriae, and Citrobacter freundii. Less frequent is HUS, caused by a neuraminidase producing streptococcus (pneumococcal HUS). The most uncommon form is the atypical HUS, which is a genetic orphan disease associated with an abnormality in the regulatory protein of the complement system. HUS has a fairly high mortality rate, amounting to 10–15% on average. The long-term outcomes of HUS depend on its type, as well as on the degree of the primary body tissue damage. According to the data from Novosibirsk Children's Municipal Clinical Hospital No 3 from 1991, 44 cases of HUS in children have been identified. Complete recovery, without any residual abnormalities, was registered in 25 children (56.8% of the cases). Nine children (20.5%) developed chronic kidney disease and 10 (22.7%) of all HUS cases were fatal. Early diagnosis, as well as the identification of pathogenetic mechanisms, is the basis for adequate therapy and outcome prediction. Campylobacter may be one of the causative agents of HUS. Despite new cases of Campylobacter-associated HUS being registered in the world, the very possibility of HUS induction by this pathogen and its pathophysiology are currently unclear. There is no convincing evidence for both Stx and the neuraminidase-related mechanism of HUS in Campylobacter infections. Given the high incidence of autoimmune disorders like Guillain-Barre syndrome and reactive arthritis in Campylobacter infections, it is currently impossible to exclude an autoimmune mechanism of HUS in these diseases. Thus, the role of Campylobacter, as a new potential bacterial agent of HUS, as well as the pathogenesis of such conditions in Campylobacter infections, requires further study.

Pyoderma gangrenosum (PG) belongs to the group of neutrophilic dermatoses with unknown etiology and poorly understood pathogenesis. In children, PG is primarily associated with inflammatory bowel diseases (Crohn's disease and ulcerative colitis). By now, about 355 PG cases have been described worldwide, including 15 children with the involvement of oral mucosa. Clinical manifestations of the disease are diverse and depend on the form, stage and associated conditions. Such a rare PG as pyodermatitis-pyostomatitis vegetans manifests by combined lesions of the oral mucosa and skin. The authors present historical data on the investigation of the disease, its etiology, pathogenesis, risk factors, and clinical picture. A description of a rare clinical case of severe form of atypical PG, namely, pyodermatitis-pyostomatitis vegetans in a 10-year-old child, is presented. The unique character of the case is related to the variety of clinical manifestations and the clinical course complicated by the development of keloid and partial scar-related flexion contracture of the upper and lower extremities, the formation of microstoma and cachexia. The association of pyodermatitis-pyostomatitis vegetans with Crohn's disease was found. The lack of awareness of the clinical symptoms of this rare disease has led to diagnostic errors and late diagnosis.

Primary cardiac tumors are very rare in children. Rhabdomyoma is the most common benign tumor in fetuses and neonates. Most cases do not require any surgical intervention due to absence of clinical symptoms and a high rate of spontaneous regression within the first year of life. However, some neonates can have significant abnormalities of intracardial hemodynamics related to the obstruction of the left and right ventricle outflows; this is an indication to emergency surgery. As a rule, complete or partial resection of the tumor mass provides uneventful postoperative course and is not associated with a relapse of rhabdomyoma in the long-term.

We present a clinical case of a 15-days old neonate who underwent an emergency surgery due to advanced tumor obstruction of the blood flow in the right ventricle outflow tract (RVOT). At preoperative echocardiography, there was a pulmonary artery systolic pressure gradient of 90 mm Hg. Moderate hypoxemia (SaO₂ 90%), breathing rate of up to 55 per minute, together with echocardiographic results, indicated the impaired pulmonary blood flow and the need for the tumor resection. The tumor was completely resected through the right ventricle access with cardiopulmonary bypass and cardioplegia, with subsequent autologous pericardium patching of the right ventricle. The postoperative period was uneventful; the patient was extubated at day 2 and discharged at day 11 after surgery. The diagnosis of rhabdomyoma was confirmed histologically. At one month after surgery, no additional tumor masses were found in the heart chambers and septum.

Rhabdomyoma causing severe obstruction of the RVOT in a newborn is a rare life-threatening complication of the natural course of benign heart tumors in children. The presence of dyspnea at rest, moderate desaturation, and echocardiographic data determined the rejection of the conservative management commonly adopted in most cases of cardiac rhabdomyomas, and were indications for an emergency surgery. In the long-term postoperative period, magnetic resonance imaging should be done to exclude tuberous sclerosis.