Search

Intolerance of gluten containing cereals (wheat, rye, and barley) is an etiopathogenetically heterogeneous and relatively common problem of modern man. It occurs as an adverse immune-mediated condition in genetically predisposed individuals. According to the pathogenetic mechanism of intolerance to the components of these cereals, it is classified into celiac disease as an autoimmune disease, wheat allergy as an allergic disease, and non-celiac gluten sensitivity as a non-autoimmune and non-allergic disease. Each of these disorders is characterized by specific intestinal and/or extraintestinal manifestations, which resolve on a gluten-free diet. This review article presents the basic characteristics of these disorders in accordance with modern knowledge.

Introduction/Objective. Celiac disease (CD) is the result of a polygenic predisposition and gluten-containing diet. The aim of this study was to determine the prevalence and clinical forms of CD in siblings of children with verified disease. Methods. The study included 83 siblings, aged 1.5–27 (11.77 ± 6.2) years, of 64 children with CD diagnosed according to ESPGHAN criteria (1990/2012). In addition to a detailed history and clinical examination, serum levels of IgA and antibodies to tissue transglutaminase (AtTG) IgA and IgG classes were determined in all subjects. All with elevated AtTG levels underwent multiple duodenal enterobiopsy. The diagnosis of CD was confirmed by the finding of characteristic histological changes. Results. The diagnosis of CB was made in 13 of 83 subjects (15.67%). Nine of them had an asymptomatic form of the disease, while in the others the disease was clinically manifested – in three the form was classical, in one it was accompanied by severe malnutrition (-26.80%), and in one the manifestation was nonclassical (only short stature). Except for sideropenia and hypoferritinemia in four patients, of which two with hemoglobin below the reference value, standard laboratory findings were within normal limits. Conclusion. Our research shows that the prevalence of CD in siblings of children with verified disease is 15.67%. It is mostly detected in its asymptomatic form. In accordance with this, routine application of serological screening for CD in this population group is necessary for its timely diagnosis and treatment.

Introduction. Celiac crisis is a rare and life-threatening complication of celiac disease. Although it occurs in all ages, the most common affects children within the first two years. Outline of cases. We report three infants (two female, one male, age range 9–12) with celiac crises as an initial presentation of celiac disease precipitated with rotavirus gastroenteritis. Celiac crisis was preceded by failure to thrive caused by anorexia, occasional vomiting and frequent abundant stools for 4–8 weeks, and 1–2 days before admission with fever, frequent vomiting and profuse watery diarrhea. They were admitted in a very severe general condition, severely dehydrated, markedly malnourished, with an enormously distended abdomen, edema of the lower legs and feet, and perianal erythema. After correction of dehydration and hypoalbuminemia, they were placed on a gluten- and disaccharide-free diet and within the first two weeks on additional parenteral nutrition. The applied therapeutic measures resulted in stabilization and further rapid improvement of the patient’s condition. In all three patients the latex agglutination test for rotavirus was positive, IgA anti-TTG antibodies elevated (58.6–78 U/ml) and all three were homozygous carriers of the HLA DQ2 gene. Enterobiopsy was performed two weeks after the admission and total villous atrophy (Marsh IIIc) was registered in all three patients. In the further course, the complete recovery of the patient was accomplished by a strict gluten-free diet. Conclusion. Our experience indicates that rotavirus gastroenteritis in timely unrecognized classical celiac disease in infants can lead to celiac crisis.

Introduction: Bone tumors are rare neoplasms whose therapy depends on the location. Pelvic bones represent a special anatomical entity diagnosis and therapy of pelvic tumors in addition to the pathohistological type largely depends on the anatomical specifics of that region. Aim: The aim of this study was to show the frequency, as well as clinical and pathological characteristics of primary and secondary tumors of the pelvic bones. Material and methods: The research as a descriptive study in the period from January 1, to December 31, 2019 at the Institute of Pathology of the Medical Faculty of the University of Belgrade and includes 33 patients. Demographic, clinical and radiological data were obtained from the Bone Tumor Registry. Statistical processing and analysis were done in the Statistical Package for Social Science SPSS Windows version 22 (IBM Chicago, Ilinois, USA). Results: Out of 33 patients, 55% had secondary pelvic tumors, primarily cancer metastases (37%). In 21% of cases, primary malignant bone tumor as diagnosed, and in 12%, primary benign bone tumor. Hematopoietic neoplasms were verified in 12%. Conclusion: Pelvic bone tumors are not common, but these neoplasms, in orthopedic oncology, represents their malignant biological behavior are of great importance. The most common primary malignant tumor in adults is chondrosarcoma, and in children Ewing sarcoma. Highest frequency for pelvic bone metastases has lung cancer. Plasmacytoma in adults, Langerhans cellular histiocytosis in children are hematological neoplasms seen at this site.

Introduction. Fever of unknown origin is an important diagnostic challenge. Although rare, periodic fever syndromes may often present with a chronic or recurrent febrile condition with a variable temporal pattern of occurrence. Although clinical characteristics often indicate the syndrome in question, there are many atypical forms, and the genotype–phenotype relationship is highly complex, warranting in many cases the designation of a “syndrome spectrum” rather than a syndrome per se. The aim of this paper was to present a boy with recurrent fever of unknown origin. Case outline. We hereby present a boy with recurrent fever of unknown origin who was by clinically guided partial exome sequencing found to have a heterozygous variant 434A>G in the TNFRSF1A gene, otherwise connected with tumor necrosis factor receptor-associated periodic fever syndrome. The patient responded well to short courses of glucocorticoids and is no longer subjected to unnecessary antibiotic treatment he had frequently received in the past. Conclusion. Periodic fever syndromes should be kept in mind as a differential diagnostic possibility in children with fever of unknown origin. [Project of the Serbian Ministry of Education, Science and Technological Development, Grant no. 41004]

Background/Aim. The aim of this prospective nonrandomized study was to test functional results of different surgical strategies in the operative treatment of symptomatic patellofemoral malalignment. Our hypothesis was that immediate extensive surgery does not have serious advantage comparing to “step by step” procedure, regarding the main symptoms and functional end result. We wanted to check whether obtaining ideal surgical patellofemoral congruency is an essential prerequisite for subsidence of the major symptoms of patellofemoral malalignment. Methods. The study included 35 patients with patellofemoral malalignment who had persistant major symptoms: patellar pain and slipping, 3 months after nonoperative treatment. Divided into three groups, they all underwent the realignment surgery, but in different extent and sequence: immediate extensive surgery, step by step surgery, and only proximal realignment. Their overall functional scores as well as major symptoms were assessed at the beginning, after the surgery, and during the 3-years follow-up period and then, compared at the end. Results. There was no significant difference in the functional results among the groups, neither at the beginning (p = 0.1318) nor at the end of the study (p = 0.3996), but the results at the beginning compared to those at the end of the study showed a statistically significant difference in all three groups (p1 = 0.005062; p2 = 0.011719; p3 = 0.000352). The same result was in regard to the major symptoms. Conclusion. The study confirmed that insisting on immediate extensive surgery in order to achieve precise and complete congruency of the patellofemoral joint, did not prove its advantage over the less invasive, individual surgical approach concerning functional scores and major symptoms.

Introduction. Isolated dislocation of the pisiform bone is a very rare condition due to robust ligamentous attachments that stabilize the pisiform to the carpus. This type of injury is usually a result of direct trauma to the ulnar and volar aspect of the wrist. Case outline. We present an isolated dislocation of the pisiform, with no other carpal bone injuries, treated successfully with closed reduction. Diagnosis was based on clinical findings, plain radiographs, and computer tomography examination of the wrist. Elongation and partial rupture of the pisometacarpal ligament was found on magnetic resonance imaging. Conclusion. Fracture and dislocation of the pisiform is an extremely rare injury in children, which could be easily neglected or misdiagnosed. Closed reduction with plaster cast immobilization should always be considered as the first method of treatment in the pediatric population, since the conservative approach provides excellent results.

Introduction/Objective. Child abuse is a significant public health problem in modern society. Many cases of violence against children remain undetected. Serbia has no official protocols for medical examination of abused children. The aim of the study is an analysis of the social, clinical and radiological characteristics of physical abuse of children under three years of age that required hospital treatment. Methods. This retrospective study included 98 physically injured children admitted to the University Children’s Hospital in the period from 2013 to 2015, with suspected physical abuse. In addition to the history of injuries, complete clinical examinations and standard laboratory analyses were performed in all children, as well as X-ray examination in children with apparent or suspected skeletal injury. Ultrasound examination and computerized tomography or magnetic resonance imaging were performed in selected patients. Final diagnosis of abuse was established by multidisciplinary assessment team. The children were divided into two groups – those with proven and those with suspected abuse. Results. Most of 98 children who were suspected of being abused (92%) were from one or both unemployed parents, 68% were male, 60% were first-born, and 44% younger than one year. Ninety-two percent of the children had skeletal fractures, 19% of whom had two or more fractures. The commonest fracture was a linear skull fracture, which was detected in 51% of the cases. Abuse was confirmed in only five of 98 suspected cases. Conclusion. Among the known social risk factors for abuse of children, the low economic status of the family was the most frequent one in our analyzed sample. The most common injury is a linear skull fracture. A national guideline for medical investigating of abused children is required.

Introduction. Vitamin D intoxication represents a rare and potentially serious pathological condition caused by the excess of calcium and phosphorus. We are presenting an infant with vitamin D intoxication due to excessive daily administration, as well as therapeutic procedures that prevented its adverse effects. Case Outline. A 1.5-month-old female infant, born at term, exclusively breastfed and without any complaints and abnormalities of physical findings, was observed due to the data that during the preceding month, by her mother’s mistake, she had received about 200,000 IU of vitamin D3. Laboratory analyses showed a high serum level of 25(OH)D (>400 nmol/L) and calcium (2.72 mmol/L), lowered PTH (6.6 pg/ml) and high urinary calcium/creatinine ratio (1.6), while other findings, including urotract ultrasonography image, were within normal limits. Treatment based on the discontinuation of vitamin D administration, infant’s forced water intake, as well as the application of 2-month prednisolone and 4-month phenobarbitone and furosemide, resulted in complete normalization of the laboratory indicators of vitamin D overdose, as well as the prevention of its adverse effects. Conclusion. By timely recognition and adequate treatment, including triple therapy with prednisolone, phenobarbitone and furosemide, adverse effects of acute vitamin D intoxication can be prevented.