Vol 48, No 4 (2020)

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Are functional gastrointestinal disorders a clinically significant comorbid factor for acute respiratory infection in children?

Meskina E.R., Khadisova M.K.


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.

Almanac of Clinical Medicine. 2020;48(4):231-239
pages 231-239 views

A 6-year experience of the Ross procedure in children with congenital aortic valve disease

Akatov D.S., Belov V.A., Khomich D.E., Bukhareva O.N., Makarov A.A., Kotov S.N., Schneider Y.A.


Background: The choice of an optimal method for correction of aortic valve stenosis or insufficiency remains the matter of debate. Some clinicians prefer transluminal balloon angioplasty, some would perform an open type commissurotomy, or aortic valve replacement, or to valve leaflets repair with various materials, while others opt for the Ross procedure. Each of these techniques has its advantages and disadvantages both in the early postoperative period and in the long-term.

Aim: To assess the results of the Ross procedure, its complications, survival, and probability of reoperations in the long-term.

Materials and methods: We retrospectively analyzed the results of 32 Ross procedures performed from the end of 2012 to the beginning of 2019 in children aged from 3 days to 18 years. Eight children were below 1 year of age, including one newborn. A pulmonary autograft was placed into the aortic position in all children. In 31 children, a homograft was placed into the pulmonary artery. In one patient, an aortic autograft was implanted instead of the pulmonary artery valve. The mean patient age was 6.8 years, mean bodyweight 27.7 kg (range, 3.9–74.6 kg).

Results: Two patients died in the early postoperative period (mortality 6.25%). We examined 26 patients at the follow-up, with its mean duration of 2.5 years. There were no indications for replacement of the homograft during the follow-up. One patient, in whom we had implanted an aortic autograft into the pulmonary position, required its replacement after 4.5 years. We have no data on death rates in the long-term.

Conclusion: The Ross procedure provides satisfactory results in the early and intermediate postoperative period. Continuous follow-up is needed to determine the time points and the proportion of repeated interventions with a pulmonary homograft and aortic valve.

Almanac of Clinical Medicine. 2020;48(4):240-245
pages 240-245 views


The hemolytic uremic syndrome: a possible etiological role of Campylobacter infection

Karpovich G.S., Krasnova E.I., Vasyunin A.V., Komissarova T.V., Enivatova L.I., Gaynts O.V.


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.

Almanac of Clinical Medicine. 2020;48(4):246-253
pages 246-253 views

Leucinosis, or maple syrup urine disease (lecture and a clinical case)

Tsareva J.A., Zryachkin N.I., Kuznetsova M.A., Bogacheva E.V.


Maple syrup urine disease (leucinosis, short-chain ketoaciduria, branched-chain disease, branched-chain ketonuria) is an autosomal recessive disorder which is a consequence of the deficient branched-chain alpha ketoacid dehydrogenase complex. There are five subtypes of the disease: classical, intermediate, intermittent, thiamine-dependent and E3-deficient. Leucinosis is characterized by high plasma levels of branched-chain amino acids (leucine, isoleucine and valine) and high urine levels of branched-chain ketoacids, as well as of lactate and pyruvate. Tandem mass spectrometry can be used as a screening method in newborns. Mild disease cannot be identified at screening. The diagnosis should be based on tandem mass spectrometry of a blood sample and aminoacid analysis by gas chromatography of a urine sample. Prenatal diagnosis requires molecular genetic tests. Treatment of maple syrup urine disease is aimed at normalization of plasma branched-chain amino acids levels and includes two main components, namely, life-long diet therapy and active treatment of acute metabolic deterioration episodes. A favorable course of the disease is possible only with early (pre-symptomatic) initiation of treatment. The development of cognitive functions depends on plasma leucine levels. We present a clinical case of delayed diagnosis of leucinosis, despite its early clinical manifestation, leading to irreversible consequences for the patient.

Almanac of Clinical Medicine. 2020;48(4):254-262
pages 254-262 views


An unusual pediatric clinical case of atypical pyoderma gangrenosum

Sedova T.G., Elkin V.D., Zhukova A.A.


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.

Almanac of Clinical Medicine. 2020;48(4):263-270
pages 263-270 views

A clinical case of inverted duplication with terminal deletion of the short arm of chromosome 5

Solovova O.A., Oparina N.V., Kotalevskaya Y.Y., Kalinenkova S.G., Latypov A.S.


The 5p inverted duplication deletion syndrome, also known as inv dup del 5p, is a rare genetic disorder with a prevalence of below 1:1 000 000, whose underlying abnormality lies in a segmental trisomy and simultaneous segmental monosomy of the short arm of chromosome 5. The syndrome was first described by A. Kleczkowska et al. in 1987. According to the literature, large duplications of the chromosome 5 short arm are associated with pronounced phenotypic manifestations, delayed speech and mental development, as well as congenital cardiac, brain and musculoskeletal malformations. We present a description of a clinical case of extended inverted duplication with deletion of the chromosome 5 short arm in a girl with a mild phenotype and no visceral or musculoskeletal abnormalities; we also discuss the pathogenetic mechanisms of chromosomal rearrangement, and conduct a comparative analysis of phenotypic manifestations based on the available literature. Comprehensive molecular cytogenetic assessments have demonstrated that the duplicated site has a length of 29 Mb (5p13.3p15.33), and the deleted site of the subtelomeric region distal to 5p15.33 has a length of 110 kb.

Almanac of Clinical Medicine. 2020;48(4):271-279
pages 271-279 views

Successful surgery for a right ventricle rhabdomyoma in a neonate

Tarayan M.V., Efremov E.S., Bondareva I.O., Shkarina N.V., Kavaidin S.N.


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.

Almanac of Clinical Medicine. 2020;48(4):280-284
pages 280-284 views

Guillain-Barré syndrome in a child: a clinical case

Ryabchenko A.Y., Grankin E.V.


Guillain-Barré syndrome is an orphan autoimmune disease associated with the involvement of the peripheral nervous system. The clinical course of the syndrome has four main types. We present a clinical case of Guillain-Barré syndrome in a 6-year old child. Against the background of a previous infection, he developed oculomotor dysfunction, peripheral tetraparesis, sensory abnormalities with subsequent severe weakness of the respiratory muscles. The differential diagnosis included inflammatory, infectious and neoplastic spinal cord disorders, spinal cord magnetic resonance imaging, and examination of the cerebrospinal fluid. The patient's electromyographic data confirmed peripheral nerve damage. Based on all of these, the patient was diagnosed with Guillain-Barré syndrome. Specific treatment, including plasmapheresis and intravenous human immunoglobulin G at a dose of 0.4 mg daily for 5 days, and symptomatic treatment resulted in gradual improvement and complete regression of the neurological symptoms completely resolved. Since Guillain-Barré syndrome is a rare disease of the peripheral nervous system, the awareness of the specifics of its clinical course allows for earlier correct diagnosis and effective treatment.

Almanac of Clinical Medicine. 2020;48(4):285-289
pages 285-289 views

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