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1997

ТОМ XXXVII, 4/1997 година, VOLUME XXXVII, 4/1997 year

СЪДЪРЖАНИЕ

ОБЗОРИ И ЛЕКЦИИ

Бобев Др.

Актуални проблеми на педиатрията - постижения и перспективи

Бобев Др., М. Йорданова

Приложение на рекомбинантен човешки гранулоцитен растежен фактор / Neupogen Filgrostim / в клиничната практика

КЛИНИЧНИ И ПРОФИЛАКТИЧНИ НАБЛЮДЕНИ

Переновска П., Н. Улевинов, М. Гизова, А. Куфарджиеба

Бронхоалвеоларен лаваж при идиопатична белодробна хемосидероза в детската възраст

Исаев В., Ел. Перчинска, Г. Иванов

Обективни критерии за ефективност на началното лечение при тежък асматичен пристъп в детската възраст

Генкова Н., Т. Шмилев, М. Клинканова, М. Бошева

Първична миокардна фиброза у деца с муковисцидоза

Маринова Св. А. Анадолииска, Ср. Апостолова, Д. Русинов, П. Митева

Хемолитично - уремичният синдром - най-честата причина за остра бъбречна недостатъчност в кърмаческа и ранна детска възраст

Митова Е., А. Анадолийска, М. Лилова

Henoch-Schonlein гломерулонефрит - клинична характеристика, еволюция и лечение

Пантелеева Е., Хр. Желев, Р. Куманова, П. Янева, Е. Лазарова, К. Кънчев, Д. Мондал, А. Цветанска

Диагностична стойност на антиендомизиумните антитела за целиакия в детската възраст

Шакола-Георгиева, М., М. Стамболийска, Й. Русева, В. Шакола

Физикален метод за лечение на пептичната язвена болест при деца

Йотова В., В. Цанева, К. Петрова

Роля на естественото хранене и растежа през първата година от живота в етиологията на захарния диабет в детска възраст

Димова П., Л. Белопитова, Р. Йотова

Неврологични синдроми при хронично-реактивираща се Epstein-Barr вирусна инфекция - диагностични и диференциално-диагностични проблеми

Симеонов Е.

Регистрация на вродени аномалии:

1.Цел и методи за регистрация на вродени аномалии

Симеонов, Е., К. Василева, М. Калайджиева, С, Динова, Ж. Рачева, А. Благоева

Регистрация на вродени аномалии:

2.Регионален регистър за вродени аномалии „София 96" - едногодишен опит

СОЦИАЛНА ПЕДИАТРИЯ

Банова В., Р. Маркова, Claude-Anne Decormis-Branchip, Joelle Charmasson

Споделяне на едногодишен опит от въвеждане на „работата в екип", като основен механизъм на интеграция на деца с проблеми в развитието и поведението в дом „Майка и дете"

ТЕРАПЕВТИЧНИ ПРОБЛЕМИ

Белопитова Л., Р. Йотова, В. Божинова, Р. Божилова, В. Томов

Нашият опит от прилагане на Lamictal при терапевтично резистентни клинични форми на епилепсия в детска възраст

ЗА ПРАКТИКАТА

КовачеваЮ.

Приложение на Pepti - Junior педиатричната практика

ХРОНИКА

Калайков Д.

VIII Национален конгрес по педиатрия

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CONTENTS

REVIEWS AND LECTURES

Bobev Dr.

Topical Problems in Paediatrics - Achievements and Prosepcts

Bobev, Dr., M. Yordanova

Application of Recombinant Human Granulocyte Growth Factor (Neupogen Filgrostim)

CLINICAL AND THERAPEUTIC OBSERVATIONS

Perenovska, P., N. Oulevinov, M. Gizova, A. Koufardzhieva

Bronchial-Alveolar Lavage in Idiopathic Pulmonary Haemosiderosis

Isaev, V., E. Perchinska, G. Ivanov

Objective Criteria for the Effectiveness of Initial Treatment in Severe Asthmatic Attack in Childhood

Genkova, N., T. Shmilev, M. Klinkanova, M. Bosheva

Primary Myocardial Fibrosis in Children with Mucoviscidosis

Marinova, S., A. Anadoliyska, S. Apostolova, D. Rousinova, P. Wliteva

Haemolytic-Uraemic Syndrome - Most Frequent Cause for Acute Renal Failure During Infancy and Early Childhood

Mitova, E., A. Anadoiyska, M. Lilova

Henoch-Schonlein Glomerulonephritis - Clinical Characteristics, Evolution

Panteleeva, E., Hr. Zhelev, R. Koumanova, P. Yaneva, E. Lazarova, K. Kuntchev, D. Mondal, A. Tsvetanska

Diagnostic Value of Antiendomysium Antibodies for Coeliac Disease in Childhood

Georgieva-Shakola, M., M. Stamboliyska, I. Rouseva, V. Shakola

Physical Method for the Treatment ot Peptic Ulcer in Childhood

Yotova, V., V. Tsaneva, K. Petrova

Role of Natural Feeding and Regimen During the First Year of Life

Dimova, P., L Belopitova, R. Yotova

Neurological Syndromes in Chronic Recurrent Epstein-Barr Virus. Infection in Two Children - Diagnostic and Differential Diagnostic Problems

Simeonov, E.

Registration of Congenital Anomalies: 1. Purpose and Methods for Registration of Congenital Anomalies

Simeonov, E., K. Vasileva, M. Kalaidzhieva, S. Dimova, J. Racheva, A. Blagova

2. Regional Register for Congenital Anomalies ,,Sofia '96° - One-Year Experience

SOCIAL PAEDIATRICS

Banova, V., R. Markova, Claude-Anne Decormis-Branchip, Joelle Charmasson

Sharing of One-Year Experience in Introduction of Team Work as a Basic Mechanismin the Integration of Children with the Problems in the Development and Behaviour in ,,Mother and Child Home"

THERAPEUTIC PROBLEMS

Belopitova, L., R. Yotova, V. Bozhinova, V. Tomova

Therapeutic Effect of Lamictal in Reistant Epilepsy in Childhood

FOR THE PRACTICE Kovacheva, J.

Application of Pepti - Junior in Paediatric Practice

CHRONICLE

Kalaikov D.

VIII National Congres of Pediatrics

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SUMMARIES

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BRONCHIAL-ALVEOLAR LAVAGE IN IDIOPATHIC PULMONARY HAEMOSIDEROSIS IN CHILDHOOD

P. Perenovska, N. Oulevinov, M. Gizova,

Idiopathic pulmonary haemosiderosis is a disease with very much unknown related to its evolution, aetiology, therapy and prognosis. The authors applied since 1989 bronchiologic investigation including cytology of bronchial-alveolar fluid thus ensuring more valued assessment of the clinical evolution. Nine children between 6 and 15 years of age with an average duration of the disease of 7 years and 9 months. All patients revealed macroscopic changes in the bronchial secretion. Lavage fluid disclosed cyto-logical change, i.e. an increase of granulocytes and lymphocytes. In all children under investigation independently of the evolution and applied therapeutic schemes haemosiderophagial cells in the sputum have been found only in half of the patients. Present data define the diagnostic significance of the method for a precise assessment of the clinical development, defining an adequate treatment as well to establish the diagnosis in problematic cases.

Key words: bronchial-alveolar lavage, cytological changes

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OBJECTIVE CRITERIA FOR THE EFFECTIVENESS OF INITIAL TREATMENT IN SEVERE ASTHMATIC ATTACK IN CHILDHOOD

V. Isaev, E. Perchinska, G. Ivanov

In 16 children with severe asthmatic attacks between 12 months and 15 years of age respiratory and pulse frequences have been followed up as well as oxygen saturation and ,,PEF" (in 10 children of the group during the first 60 minutes of treatment. In three of these indices, i.e. respiration, oxygen saturation and ,,PEF" statistically significant differences have been found (p < 0.05) between initial values and those after three consecutive therapeutic inhalations with Ventolin. This gives ground to recommend as reliable and objective criteria for the assessment of the efectiveness of treatment in severe asthmatic attacks in childhood.

* PEF = peak expiratory flow

Key words: asthamtic attack, treatment

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PRIMARY MYOCARDIAL FIBROSIS IN CHILDREN WITH MUCOVISCIDOSIS

N. Genkova, T. Shmilev, M. Klinkanova, M. Bosheva

The investigation covers for the period 1978-1995 67 children with mucoviscidosis under observation at the Paedi-atric Clinic, High Medical School - Plovdiv. They have been followed up to 22 years of age. Diagnosis has been established in accordance with generally admitted clinical laboratory criteria. Manifestations of the cardio-vascular system have been analysed. Primary myocardial fibrosis have been revealed. Its pathogenesis remains obscure. It is specific for the disease but remains not sufficiently known by the paediatricians. Patients under investigation were infants with fully developed clinics of severe mucoviscidosis. Unexpected sudden manifestations of cardiac failure, i.e. pallor, tachypnoea, tachycardia, filiform pulse, cyanosis, car-diomegaly EGG data for myocardial lesions. Death occured in these three patients very quickly. Histologic investigation disclosed focal myocardial necrosis with connective tissue sprouting without any inflammatory infiltration.

Key words: mucoviscidosis, myocardial fibrosis, children

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HAEMOLYTIC-URAEMIC SYNDROME - MOST FREQUENT CAUSE FOR ACUTE RENAL FAILURE DURING INFANCY AND EARLY CHILDHOOD

Sv. Marinova, A, Anadoliyska, S. Apostolova, D. Rousinov P. Miteva

During a period of 10 years 17 patients (between 3 months and 7 years) with haemolytic-ureamic syndrome have been treated at the Clinic of Nephrology and haemodialysis. Prevalence of haemolytic-uraemic syndrome as a cause for acute renal failure reaches 18,8% During infancy and early childhood it reaches 70,5%.

Peculiarities in the clinical course are being outlined, further basic criteria for early diagnosis and its relation to clinical laboratory indices for the fatal end. Some unfavourable prognostic sings are being pointed out. Directions for therapeutic behaviour are being defined.

Key words: haemolytic-uraemic syndrome, clinics, diagnosis, prognosis, treatment

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HENOCH-SCHUNLEINGLOMERULONEPHRITIS - CLINICAL CHARACTERISTICS, EVOLUTION

E. Mitova, A. Anadoliyska, M. Lilova

During a period of 10 years at the Clinic of Paediatric nephrology and Haemodialysis 35 chidlren (21 boys and 14 girls) with Schonlein-Henoch's disease with renal impairment have been hospitalized. Extensive skin changes have been observed in all 35 children. Glomerulonephritis with a severe course has been present in 10 (55%). Glomerulonephritis with a severe course has been present in 10 (28%) children, 4 of them revealing severe renal failure requiring dialysis treatment. Two patients presented chronic renal failure 1st degree and proteinuria with neph-rotic values. In 4 patients a reversible nephrotic syndrome has been obsrved with a well developed nephrotic biochemical constellation. In the remaining 25 children macro-and micro haematuria has been recorded. Proteinuria has been unimportant without nephritic syndrome. Minimal renal impairment has not been treated. Sevre nephritic/ nephrotic syndrome has been treated by pulse therapy with methylprednisolone. In two patients cytostatic treatment with cyclophosphamide has been applied. Evolution has been favourable in 29 children (85%), achieving prolonged remission and normal renal function. The remaining 6 children have been stabilized, 4 are on chronic haemodialysis.

Key words: Schurilein-Henoch purpura, glomerulone-phritis, prednisolone pulse

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DIAGNOSTIC VALUE OF AISITIENDOMYSIUM ANTIBODIES FOR COELIAC DISEASE IN CHILDHOOD

E. Panteleeva, Hr. Zhelev, R. Koumanova, P. Yaneva, E. Lazarova K. Kuntchev, D. Mondal, A. Tsvetanska

Preliminary results are being reported after testing for endomysium antibodies. Fourteen children with coeliacia as well 44 pathologic and healthy controls have been investigated. Results disclose statistically significant correlation between positive antiendomysium antibodies and histologically proved gluten enteropathy. Established parameters, i.e. sensitivity (100%), specificity (97.7%), PPV (93,3%) and NPV (100%) give ground to offer these as discriminative criteria in the diagnostic process in coeliacia, further as an index to keep the diet the diet as a screening test for groups at risk.

Keywords: coeliacia, antiendomysium antibodies

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PHYSICAL METHOD FOR THE TREATMENT OF PETIC ULCER IN CHILDHOOD

M. Georgieva-Shakola, M. Stamboliyska, I. Rouseva, V. Shakola

Present study is being aimed at establishing the effectiveness of milimeter microwave therapy (MMW) in the treatment of peptic ulcer (PU) in children as well its influence on Helicobacter pylori (HB) infection, further - to compare results from the treatment with MMW and H2 blockers in childhood. During the period 1994-1995 MMW therapy has been applied in 22 children with PU with a mean age of 12,4 years in 54,6% complicated by haemorrhages. Before and after treatment upper endoscopy has been applied, accompanied by investigations for HB. MMW therapy has been carried out in the course of 22 sessions on the biologically active point TZUI-TUE. 25 children and adolescents with endoscopic proved PU treated with Ranitidin (5 mg/kg), 32%'romplicated by haemorrhages, are being observed as controls. In cases with proved HB infection MMW therapy or H2 blocker are being combined with the same antibacterial scheme: amplicillin, 2,0 + metronidazole 0.750 for 10 days. Influence on PU has been established in 100%. i.e. total epithelial restoration is present in 19/25, partial restoration - in 3/2. In the controls treated with Ranitidine total epithelial restoration is present in 19/22, partial one - in 5/ 25 (p < 0,05). After applied antibacterial scheme for the treatment of HB infection among patients treated with MMW the effect is 6/7, in those treated with ranitidin -13/17. During the whole period of MMW therapy no side effects or complications have been established.

In conclusion: MMW is a non standard physical method for treatment of PU. Results disclose a better effect compared to H2-blockers in childhood especially in complicated associated with HB ulcers. In this case combination with antibacterial therapy is being recommended. The method is well tolerated by the children with no side effects

Key words: milimetermicrowave therapy, peptic ulcer, Helicobacter pylori infection in childhood.

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ROLE OF NATURAL FEEDING AND REGIMEN DURING THE FIRST YEAR OF LIFE

V. Yotova, V. Tsaneva, K. Petrova

Relationship between insulin-dependent diabetes mel-litus and breast feeding is a subject of serious research. Present work is being aimed at the assessment of infant feeding in children with diabetes (n = 116) as well to compare it to healthy controls (n = 121). Rates of height and weigh gain during the first year of life in 45 diabetic children are separately compared to 135 healthy controls matched by age sex and duration of breast feeding -5,33 ± 4,47 versus 4,94 + 3,38 months (p > 0,1). Children with diabetes had significantly lower birthweight - 3278 ± 508 versus 3437 ±418 (p < 0,01). Diagnosed during winter were breast fed for a shorter period of time - 4,07 months and among brest fed up to 3 months diabetic children more were diagnosed during winter - 48,3% versus 28,6% among breast fed longer than 3 months (p < 0.02). Children with diabetes had grater weight gain during the whole first year of life while the height gain was not different. We don't find causal relationship between breast feeding and childhood diabetes except in the subgroup of those diagnosed during the winter months. It is possible that breast feeding may play a protective role against diabetes against through the generally moderate weight gain during infancy.

Keywords: insulin-dependent diabetes mellitus, breast feeding, childhood

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NEUROLOGICAL SYNDROMES IN CHRONIC RECURRENT EPSTEIN-BARR VIRUS INFECTION IN TWO CHILDREN - DIAGNOSTIC AND DIFFERENTIAL DIAGNOSTIC PROBLEMS

P. Dimova, L Belopitova, R. Yotova

The report deals with two children presenting neurological manifestations in remittent reactivating virus infection Epstein-Barr Virus (EBV). Meningoencephalomy-elopolyneuritis was present in one child. It appeared during the course of infectious mononucleosis. Disseminated encephalitis, starting one week after acute tonsillopharyngitis has been observed in the second child. In both cases the disease reveals a remitent course. Symptomatics disappear spontanceoulsy or after treatment with corticosteroids during several weeks. Laboratory investigations disclose following data: cerebrospinal fluid - protein- cellular dissociation with diffuse increase of gamma globulins. Immunological investigation point as a cellular type of sensivblization towards basic protein of myeline (BMP) in the first case as well synthesis of antibodies towards BPM in the other. Vlrological investigaions disclose significantly increased antibody liters against EBV in the other. Virological investigations reveal significantly incresed antibodytiters against BPM in the cerebrospinal fluid and serum. Diagnostic and differential diagnostic problems are being discussed. As basic pathogenic mechanism a quickly disappearing recurrent demyelinating process with EBV infection. The necessity is being put forward to look after such pathology among patients with different and diverse with or without manifestations of infectious mononucleosis also a possible link in similar cases with demyelinating diseases, in childhood first of all multiple sclerosis.

Key words: EBV infeciton, infectious mononucleosis, demyelinating process, multiple sclerosis

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REGISTRATION OF CONGENITAL ANOMALIES 1. PURPOSE AND METHODS FOR REGISTRATION OF CONGENITAL ANOMALIES

E. Simeonov

At the beginning of the years '60 in many countries epidemics of reducing anomalies of the limbs have been revealed in neonates of mothers who used the hypnotic preparation Thalidomid during pregnancy. About 5000 neonates have been affected by this anomaly. The tragedy caused by this drug became a painful awareness on the deficient knowledge on aetiology and prophylaxis of congenital anomaliels. This led to the idea to register congeniral anomalies (RCA) in neonates and to control the dynamics and prevalence with the hope for the earliest disclosure of teratogenic agents of different substance.

Basic conclusions from the work on RCA in Bulgaria are being drawn. The necessity for actualization of these activities is being outlind.Data on the present international experience in this domain are being reported. Basic principles are being reviewed mainly on a clinically oriented programme of EUROCAT.

Key words: registration of congenital anomalies, JCBDMS, EUROCAT

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SHARING OF ONE-YEAR EXERIENCE IN INTRODUCTION OF TEAM WORK AS A BASIC MECHANISM IN THE INTEGRATION OF CHILDREN WITH PROBLEMS IN THE DEVELOPMENT AND BEHAVIOUR IN ,,MOTHER AND CHILD HOME"

V. Banova, R. Markova, Claude-Anne Decormis-Branchi, Joelle Charmasson

Sharing their experience in introducing team work as a basic mechanism for the integration of children in the problems of development of ..Mother and Child Home". Basic principels, aims and specific peculiarities of team work, as well the formation of an individual pedagogical project for every child. Team work is being illustrated with 4 cases reports from ..mother and Child home (Sofia). They are being discussed with a French team during ist visit in October 1995 and April 1996. Main advantages are being outliend in the discussionin team work as a routine mechanism in the work in an institution for children investigating development and behaviour.

Key work: ..Mother and Child Home", pedagogical team work, integration of children in problems of development and behaviour.

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REGISTRATION FOR CONGENITAL ANOMALIES

2. REGIONAL REGISTER FOR CONGENITAL ANOMALIES ,,SOFIA'96" ONE-YEAR EXPERIENCE

E. Simeonov, K. Vasileva, M. Kalaidzhiva, S. Dinova, J. Racheva, A. Blagova

Registration of congenital anomalies (RCA) is being carried out in this country since 1985 to begin with the obstetric hospital ,,Maichin Dom" (Sofia) followed by further obstetric institutions. Since the beginning of 1996 regional registers for congenital anomalies regional register has been founded. This register is functioning in accordance with the requirements of EUROCAT. During 199610 228 pregnancies have been checked. 9821 of them have been terminated by the delivery of a neonate, 86 ended in stillbirth, 321 ended by induced abortion. 149 families (1,46%) have been registered due to single or multiple congenital anomalies in the aborted foetuses (14), stillbirths (12) or 123 deliveries. Aetiological registered CA include chromosomal aberrations -16, monogenic defects - 16, multifactorial anomalies - 80, exogenic conditioned states - 4, indistinct malformative syndromes - 33. Analysis of obtained results and one year experience give ground for important conclusions on the effectiveness of RCA as a method for genetic prophylaxis. Besides that it is possible to consider a series of organizational, staff and financial problems as elucidated in view of a future extension of activities in this country.

Key words: registration of congenital anomalies, EUROCAT

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THERAPEUTIC EFFECT OF LAMICTAL IN RESISTANT EPILEPSY IN CHILDHOOD

L Belopitova, R. Yotova, V. Bozhinova, V. Tomova

Present investigation covers 25 children with drug resistant epilepsi, treated for a long period with anticonvul-sive drugs. Clinical characteristics fo epileptic manifestations is various with focal attacks (simple or compelx, partial) - (32%), with generalized seizures (tonic-clonic with or without absence - 8 children (32%), with polymorph attacks - in 9 children (36%). Prevailing are cases with high prevalence of epileptic manifestations - in 16 children (64%), they are dialy or 2-4 times a week. Lamictal has been applied as follows: in 6 patients (24%) as monotherpay thus replacing some other anticonvulsant or it is the first drug of choice in 6 children (24%) in combination with Valproate - in 13 children (52%) in double or triple combination with different anticonvulsants. Clinical effect has been assessed as very good in 7 children (28%), seizures being discontinued. Partly effective treatment has been observed in 9 children (36%) by reducing over 50% the frequency of attacks. In 9 children (36%) no effect has been observed. Dynamics of clinical features is correlating with EEG patterns, i.e. disappearing of focal abnormalities in patients with full clinical effect as well reduction of generalized paroxysmal activity with partial influence on seizures. Treatment has been continued from 3 months to 5 years. Parallel to positive clinical results related to the epileptic sydnrome a considerable improvement of congnitive functions is being accounted in these diseased children.

Summarized analysis and discussion of the possibilities for a broader application of Lamictal in neurological practice in childhood not only in therapeuticall resistant epileptic children but also as first medicine of choice in newly revealed patients.

Keywords: Lamictal, focal epilepsy, therapeuticall resistant seizures, EEG, cognitive function

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APPLICATION OF PEPTI - JUNIOR IN PAEDIATRIC PRACTICE

J. Kovacheva

Pepti - Junior is a highly Specified elementary food on the base of short-chained peptides and free aminoacids, obtained on the base of highly effective hydrolysis of whey protein. In 50% fats are present in the form of medium-chain triglycerides. Carbohydrates are present in the form as glycose polymers. Special composition of this product defines the broad application in paediatric practice in a series of diseases revealing a course with severe malab-sortive syndrome in exopancreatic failure, intestinal lymphangiostasis, short intestine syndrome, chronic cholestatic syndromes, also in some rare disease with impaired lipid metabolism. Pepti-Junior is a valuable assistant of the paediatrician in the dietary regimen in many diseases.

Key words: elementary food, short-chain peptides, medium-chain triglycerides, malabsorptive syndrome

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