scholarly journals SURGICAL TREATMENT OF ATIPICAL FORMS OF CONGENITAL HYPERINSULINISM

2020 ◽  
Vol 24 (2) ◽  
pp. 83-88
Author(s):  
A. A. Sukhotskaya ◽  
V. G. Bairov ◽  
L. B. Mitrofanova ◽  
D. V. Ryzhkova ◽  
I. L. Nikitina ◽  
...  
Keyword(s):  
Surgical Treatment ◽  
Motor Development ◽  
Medical Center ◽  
Neurological Complications ◽  
Psychomotor Development ◽  
Complete Relief ◽  
Congenital Hyperinsulinism ◽  
National Medical ◽  
Pet Ct ◽  
Organic Brain

Introduction. 30-60% of all patients with congenital hyperinsulinism have a delayed psychomotor development, and 15-25% of them have a severe organic brain damage, including epilepsy. The timely diagnostics and intensive care can prevent severe neurological complications. If the conservative therapy is not effective, then the surgical treatment is a need. Objective: To demonstrate features of surgical correction of atypical forms of congenital hyperinsulinism. Material and methods. 11 children with atypical forms of congenital hyperinsulinism were operated on in the department of pediatric surgery in the V.A. Almazov National Medical Center in 2017-03.2019. Results. 2 children demonstrated a complete relief of hyperinsulinism in 9 months (1.5 years) of follow-up; one patient had a significant improvement of his psycho-motor development. Conclusion. Some children with presumably focal forms of hyperinsulinism, by PET-CT findings, may have histologically atypical forms. In the atypical lesion of the pancreas, an intraoperative biopsy dramatically changes the planned volume of pancreatectomy up to almost total removal of the gland.

scholarly journals Congenital hyperinsulinism in newborns and young children: the state of the problem and the results of surgical treatment

2021 ◽  
pp. 226-239
Author(s):  
A. A. Sukhotskaya ◽  
V. G. Bairov ◽  
I. L. Nikitina ◽  
L. B. Mitrofanova ◽  
A. A. Perminova ◽  
...  
Keyword(s):  
Surgical Treatment ◽  
Correct Diagnosis ◽  
Psychomotor Development ◽  
Research Centre ◽  
Complete Relief ◽  
Congenital Hyperinsulinism ◽  
Biochemical Basis ◽  
Intravenous Glucose ◽  
Partial Pancreatectomy ◽  
Pet Ct

Congenital hyperinsulinism causes irreversible damage to the cerebral cortex with subsequent disability in children. The article presents the features of etiopathogenesis, clinical picture of the disease. The histological variants of pancreatic lesions are analyzed in detail. The principles of correct diagnosis are formulated. A new in Russia method for the preoperative determination of the histological form of the disease, which is carried out at the y, Almazov National Medical Research Centre since 2017, – PET/ CT with 18F-DOPA, explained the biochemical basis of its clinical application and the examination technique. The principles of the selection of drug therapy with possible complications, the need for an adequate assessment of its effectiveness are described. If it is impossible to achieve a stable target euglycemia without the need for intravenous glucose infusion, surgical correction of the disease is indicated. In schematic drawings and intraoperative photographs, approaches to surgical treatment are described, the stages of operations and possible complications are clearly disassembled. The results of surgical interventions at the N.N. V.A. Almazov for 01.2017–02.2021, where 39 children with congenital hyperinsulinism were operated on. According to PET/CT with 18F-DOPA, 15 diffuse and 24 focal forms were diagnosed. After surgery, in 12 (31%) patients, a diffuse lesion of the gland was confirmed, in 23 (59%) – a focal nature of the lesion, in 4 (10%) – an atypical form was diagnosed intraoperatively. Of 39 children, 36 (92%) have complete relief of hyperinsulinism, a significant improvement in psychomotor development, of which 9 (23%) need insulin replacement therapy with minimal dosages, these are 8 children with a diffuse form of the disease and 1 child with an atypical one. Intraand postoperative complications were not observed. Thus, partial pancreatectomy for focal forms, subtotal for atypical and near total for diffuse forms, can cope with hypoglycemia due to congenital hyperinsulinism and prevent damage to the central nervous system of newborns and infants.

scholarly journals SURGICAL TREATMENT OF FOCAL FORMS OF CONGENITAL HYPERINSULINISM: IS ALL CLEAR?

2020 ◽  
Vol 23 (6) ◽  
pp. 296-302
Author(s):  
Anna A. Sukhotskaya ◽  
V. G. Bairov ◽  
I. L. Nikitina ◽  
D. V. Ryzhkova ◽  
L. B. Mitrofanova ◽  
...  
Keyword(s):  
Surgical Treatment ◽  
Motor Development ◽  
Complete Recovery ◽  
Psychomotor Development ◽  
Research Centre ◽  
Complete Relief ◽  
Positive Trend ◽  
Congenital Hyperinsulinism ◽  
Focal Lesions ◽  
Partial Pancreatectomy

Introduction. 30-60% of all patients with congenital hyperinsulinism have a delayed psychomotor development, and 15-25% of them have severe organic brain damage, including epilepsy. Timely diagnostics and intensive treatment can prevent severe neurological complications. With the ineffectiveness of conservative therapy, surgical treatment is a necessary alternative. Objective. To define indications for various options of surgical correction of focal forms of congenital hyperinsulinism. Material and Methods. In the Department of Pediatric Surgery of V.A. Almazova National Medical Research Centre, in 2017-03.2019, 14 children with focal forms of congenital hyperinsulinism were operated after analyzing their PET-CT findings; in 2 patients out of them they were doubtful. Results. 13 (93%) patients had a complete relief of hyperinsulinism and a significant improvement in their psycho-motor development. 10 (71%) of 14 children had a complete recovery; 1 (7%) - a significant positive trend. 3 (21%) patients needed insulin therapy with minimal dosages. They all were children with an atypical (mixed adenomatous-diffuse) form of hyperinsulinism. Conclusions. In case of focal lesions in the pancreas, partial pancreatectomy with maximal preservation of healthy tissue, defined by the express biopsy, and with any lesion localization is indicated. For atypical forms, the volume for pancreatectomy dissection is defined individually.

scholarly journals Dependence of pancreas resection volume on PET-tomography and express biopsy findings

2021 ◽  
Vol 25 (1) ◽  
pp. 11-18
Author(s):  
A. A. Sukhotskaya ◽  
V. G. Bairov ◽  
A. A. Perminova ◽  
L. B. Mitrofanova ◽  
I. L. Nikitina ◽  
...  
Keyword(s):  
Surgical Treatment ◽  
Imaging Techniques ◽  
Research Centre ◽  
Differential Diagnostics ◽  
Subtotal Resection ◽  
Congenital Hyperinsulinism ◽  
Partial Pancreatectomy ◽  
Pet Ct ◽  
Diffuse Form ◽  
Group 2

Introduction. Recently, achievements in molecular genetics, imaging techniques (PET/CT), medicamentous therapy as well as in surgical treatment have promoted a better control of hypoglycemia and, consequently, better outcomes in children with congenital hyperinsulinism.Purpose. To specify indications, volume and outcomes of surgical treatment in patients with congenital hyperinsulinism depending on pathology form, differential diagnostics with PET / CT and intraoperative express biopsy.Material and methods. 41 children with congenital hyperinsulinism were operated in the department of pediatric surgery in Almazov National Medical Research Centre (Saint-Petersburg) during 2011 – September 2020. In Group 1, there were 6 children who had standard treatment with subtotal resection of the pancreas (95%). In Group 2, there were 35 patients who had PET tomography with 18-F-DOPA before surgery and intraoperative express biopsy of pancreas tissue. The analyzed patients were operated on in 2017-September 2020. PET tomography with 18-F-DOPA findings revealed that these children had 10 diffuse forms and 22 focal forms; the other 3 children had a disputable picture.Results. After surgery, diffuse form was confirmed in 10 (29%) children; focal form - in 21 (60%) children; 4 (11%) patients had the atypical form which was diagnosed intraoperatively. Of 35 children from Group 2, 33 (94%) had complete hyperinsulinism reversal, significant improvement in their psycho-motor function; however, 10 (29%) of them require insulin replacement therapy with minimal dosages - 8 children with the diffuse form of hyperinsulinism and 2 children with the atypical one. There were no intra- and postoperative complications. 20 (95%) out of 21 children with focal forms recovered completely. The authors also describe problems and peculiarities of urgent histological examination.Conclusion. Thus, partial pancreatectomy in focal forms, subtotal in atypical ones and almost total in diffuse forms allows to cope with hypoglycemia caused by congenital hyperinsulinism.

scholarly journals SURGICAL TREATMENT OF THE CONGENITAL HYPERINSULINISM: A PRELIMINARY ANALYSIS

2019 ◽  
Vol 23 (3) ◽  
pp. 124-127
Author(s):  
Anna A. Sukhotskaya ◽  
V. G. Bairov ◽  
I. L. Nikitina ◽  
D. V. Ryzhkova ◽  
L. B. Mitrofanova ◽  
...  
Keyword(s):  
Surgical Treatment ◽  
Pediatric Surgery ◽  
Preliminary Analysis ◽  
Imaging Techniques ◽  
Complete Recovery ◽  
Congenital Hyperinsulinism ◽  
Partial Pancreatectomy ◽  
Pet Ct ◽  
The Central Nervous System ◽  
Diffuse Form

Introduction. Advances in molecular genetics, imaging techniques (PET/CT), medicamentous therapy and surgical treatment in the recent decades have improved hypoglycemia control and , thus, improved treatment outcomes in children with congenital hyperinsulinism. Purpose. To define indications for different techniques of surgical correction in patients with congenital hyperinsulinism depending on the disease form. Material and methods. 23 children were operated on for congenital hyperinsulinism in the department of pediatric surgery in the Almazov Medical Research Center from 2011 till 2018. In 2011-2016, 5 children were operated on by the standard approach which included subtotal pancreas resection (95%). Since 2017, PET tomography with 18-F-DOPA and intraoperative rapid biopsy of the pancreas have been introduced into the curative algorithm. In 2017-2018, 18 children were operated: 6 patients with the diffuse form and 10 - with the focal one. Two more children had controversial outcomes. Results. Fifteen children (83%) had complete hyperinsulinism control and significant improvement in their psycho-motor function. Out of 18 children, 10 (56%) had a complete recovery: among them - 8 (89%) out of 9 children with the focal form of hyperinsulinism and 2 (22%) out of 9 children with the diffuse form. Two children (11%) had a marked positive dynamics. Six patients (33%) needed insulin therapy with minimal dosages - all were children with the diffuse form of hyperinsulinism (6 (67% ) out of 9). Conclusion. Partial pancreatectomy in patients with focal forms or subtotal pancreatectomy in patients with diffuse and atypical forms allow to eliminate hypoglycemia caused by the congenital hyperinsulinism and to prevent damage to the central nervous system in newborns and infants.

Health Characteristics of Elderly North Korean Defectors: 2006-2016 at National Medical Center

2019 ◽  
Vol 20 (1) ◽  
pp. 31-37
Author(s):  
Jong Yun Lee ◽  
Im Seok Koh ◽  
So Hee Lee ◽  
Sung Soo Eun
Keyword(s):  
Medical Center ◽  
National Medical ◽  
North Korean Defectors

Severity of Penetrating Eye Trauma in Children in Tajikistan

2020 ◽  
pp. 39-42
Author(s):  
K.M. Saidzhamolov ◽  
◽  
E.V. Gromakina ◽  
S.K. Makhmadzoda ◽  
◽  
...  
Keyword(s):  
Surgical Treatment ◽  
Eye Injury ◽  
Case Histories ◽  
Second Child ◽  
National Medical ◽  
Eye Trauma ◽  
Penetrating Eye Injury ◽  
Eye Damage ◽  
The Republic ◽  
Large Wound

Purpose. To assess the severity of penetrating eye trauma in children in Tajikistan. Material and methods. Retrospectively there was analyzed 277 case histories of children with a diagnosis of penetrating eye injury, admitted to the children’s department of the National Medical Centre of the Republic of Tajikistan for the provision of specialized ophthalmological care. Results. The average age of children at the time of injury to the organ of sight was 7.06 ± 3.01 years, mainly these were villagers (70%). Children under 7 years old accounted for 57.8% of those admitted to the hospital. The terms of admission to the hospital ranged from 1 to 14 days, an average of 43.02 ± 33.35 hours. The severity is caused by damage to 2 or more structures of the eyeball in 81,3%. Wounds larger than 6 mm prevailed and amounted to 63,5%. Endophthalmitis at admission was noted in 8,3% of cases. Enucleation was performed in 2 children; 244 children underwent primary surgical treatment. Visual acuity at discharge was higher than 0.1 in 72 of 275 children (26.2%), lower than 0,1 in 194 (70.7%). Conclusion. Almost every second child (43.0%) is admitted to the hospital for primary surgical treatment of an eyeball wound after 24 hours. About 2/3 of cases of eye damage are characterized by large wound sizes. Stab wounds were noted in 90.2% of cases. In 58.8% of cases, damage to the cornea was observed and in 68.6% – damage to the lens area.

scholarly journals Principal Component Analysis of Munich Functional Developmental Diagnosis

2021 ◽  
Vol 13 (2) ◽  
pp. 227-233
Author(s):  
Grażyna Pazera ◽  
Marta Młodawska ◽  
Jakub Młodawski ◽  
Kamila Klimowska
Keyword(s):  
Principal Component Analysis ◽  
Social Skills ◽  
Motor Development ◽  
Three Dimensional ◽  
Principal Component ◽  
Component Analysis ◽  
Three Dimensions ◽  
Matrix Analysis ◽  
Psychomotor Development ◽  
First Year Of Life

Objectives: Munich Functional Developmental Diagnosis (MFDD) is a scale for assessing the psychomotor development of children in the first months or years of life. The tool is based on standardized tables of physical development and is used to detect developmental deficits. It consists of eight axes on which the following skills are assessed: crawling, sitting, walking, grasping, perception, speaking, speech understanding, social skills. Methods: The study included 110 children in the first year of life examined with the MFDD by the same physician. The score obtained on a given axis was coded as a negative value (defined in months) below the child’s age-specific developmental level. Next, we examined the dimensionality of the scale and the intercorrelation of its axes using polychoric correlation and principal component analysis. Results: Correlation matrix analysis showed high correlation of MFDD axes 1–4, and MFDD 6–8. The PCA identified three principal components consisting of children’s development in the areas of large and small motor skills (axis 1–4), perception (axis 5), active speech, passive speech and social skills (axis 6–8). The three dimensions obtained together account for 80.27% of the total variance. Conclusions: MFDD is a three-dimensional scale that includes motor development, perception, and social skills and speech. There is potential space for reduction in the number of variables in the scale.

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