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Alina Grama

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Articole semnate de acelasi autor in Revista Romana de Pediatrie:

TIMOMUL: O CAUZA RARA DE TUMORA MEDIASTINALA LA COPIL

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Revista Romana de PEDIATRIE | Volumul LXV, Nr. 4, An 2016
ISSN 1454-0398  |  e-ISSN 2069-6175
ISSN-L 1454-0398
DOI: 10.37897/RJP

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TIMOMUL: O CAUZA RARA DE TUMORA MEDIASTINALA LA COPIL

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REZUMAT

Timomul este un neoplasm format din celule epiteliale timice, fiind rar întâlnit la copii. Aproximativ două treimi din tumorile mediastinale sunt asimptomatice la copii, diagnosticul fiind suspicionat în urma efectului de compresie a masei tumorale asupra căilor respiratorii. Prezentăm cazul unui pacient de sex masculin, în vârstă de 7 ani şi 8 luni, care se prezintă în serviciul de urgenţă acuzând oboseală la eforturi mici/repaus, tuse seacă, dispnee marcată, ortopnee, tahipnee, durere toracică, de unde se transferă de urgenţă la Clinica de Cardiologie Pediatrică, Tîrgu Mureş, cu diagnosticele: pericardită lichidiană, tamponadă cardiacă şi pleurezie dreaptă. Examenul computer tomografic toracic descrie o masă solidă inomogenă de 100/89/111 mm, la nivelul mediastinului anterior înglobând timusul şi parţial structurile vasculare ale mediastinului mijlociu. Se practică pericardectomie parţială, prelevare de ţesut tumoral şi citoreducţie tumorală extensivă, examenul histopatologic fiind Timom B3. Se iniţiază chimioterapie conform protocolului VENUTA Tymom, urmat de radioterapie adjuvantă, cu remisie clinică şi radiologică. Particularitatea cazului este dată de faptul că timomul este o tumoră rară la vârsta pacientului, creând dificultăţi de diagnostic, fiind fără complicaţii în timpul tratamentului, cu evoluţie favorabilă, cu remisie clinică şi radiologică la 1,9 ani.

Cuvinte cheie: copil, timom, tumoră mediastinală

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THYMOMA: A RARE CAUSE OF MEDIASTINAL TUMOR IN CHILDREN

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Revista Romana de PEDIATRIE | Volumul LXV, Nr. 4, An 2016
ISSN 1454-0398  |  e-ISSN 2069-6175
ISSN-L 1454-0398
DOI: 10.37897/RJP

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THYMOMA: A RARE CAUSE OF MEDIASTINAL TUMOR IN CHILDREN

, , , , and

ABSTRACT

Thymoma is a thymic neoplasm composed of epithelial cells, rarely seen in children. Approximately two-thirds of mediastinal tumors in children are asymptomatic. The diagnosis is suspected following the compression of the tumor mass on the airways. We present the case of a male patient, age 7 years and 8 months, who presented at the emergency room accusing fatigue at rest or minimum effort, dry cough, intense dyspnea, orthopnea, tachypnea, chest pain, being transferred to the Pediatric Cardiology Clinic in Tirgu Mures with the following diagnoses: fluid pericarditis, right pleural effusion and cardiac tamponade. Chest computer tomography examination is carried out, describing a solid mass of 100/89/111 mm in the anterior mediastinum, partially incorporating the thymus and the vascular structures of the middle mediastinum. Pericardectomy is practiced with partial removal of the tumor and extensive tumor cytoreduction and the histopathologic examination is B3 Thymoma. Specific treatment is initiated according to the Tymoma VENUTA protocol, followed by adjuvant radiotherapy, with clinical and radiological remission. The particularity of the case is the fact that thymoma is a rare tumor on the patient’s age, creating diagnostic difficulties, has no complications during treatment, with favorable evolution, with clinical and radiological remission at 1.9 years after.

Keywords: child, thymoma, mediastinal tumor

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POSIBILITATI SI LIMITE DE TERAPIE INTR-UN CAZ DE SINDROM ADRENOGENITAL

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Revista Romana de PEDIATRIE | Volumul LXV, Nr. 2, An 2016
ISSN 1454-0398  |  e-ISSN 2069-6175
ISSN-L 1454-0398
DOI: 10.37897/RJP

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POSIBILITATI SI LIMITE DE TERAPIE INTR-UN CAZ DE SINDROM ADRENOGENITAL

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REZUMAT

Introducere. Sindromul adrenogenital cu debut neonatal se poate manifesta prin vărsături repetate, deshidratare şi dezechilibru hidro-electrolitic sever. Abordarea diagnostică, reechilibrarea metabolică şi menţinerea balanţei electrolitice în limite normale prin tratament cronic pot fi însoţite de dificultăţi.
Obiectiv. Este prezentat un caz de sindrom adrenogenital cu debut neonatal sever, evoluţie însoţită de diferite complicaţii, dar cu prognostic ameliorat de tratament cronic adecvat.
Material şi metodă. Copil de sex masculin, cu greutate mică la naştere, s-a internat în Clinica Pediatrie I, Târgu-Mureş la vârsta de 3 săptămâni, cu un sindrom clinico-biologic sever prin vărsături cronice şi deshidratare severă şi aparentă macrogenitososmie. S-a efectuat o paletă largă de investigaţii în puseu acut, dar şi ulterior pentru elaborarea diagnosticului diferenţial.
Rezultate. S-au evidenţiat hiponatremie şi hipokaliemie persistente, o valoare crescută a 17-OH progesteron (44,96 ng/ml) şi cortizol sub limita normală (3 ug/dl). S-au înregistrat concentraţii semnificativ crescute ale ACTH (103 pg/ml matinal) şi testosteronului seric total (186,72 ng/dl). Analiza moleculară a evidenţiat genotipul heterozigot pentru următoarele mutaţii: 12G, R356W, P453S. Tratamentul cronic cortizonic adecvat a ameliorat prognosticul; la vârsta de 1,5 ani prezenta o dezvoltare staturo-ponderală şi psiho-motorie adecvată.
Concluzii. Au fost întrunite condiţiile diagnosticului de sindrom adrenogenital cu debut neonatal la un heterozigot. Vârsta mică şi simptomatologia necaracteristică pot îngreuna stabilirea unui diagnostic precoce în sindromul adrenogenital.

Cuvinte cheie: vărsături, nou-născut, hiponatremie

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POSSIBILITIES AND THERAPY LIMITATIONS IN A CASE OF ADRENOGENITAL SYNDROME

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Revista Romana de PEDIATRIE | Volumul LXV, Nr. 2, An 2016
ISSN 1454-0398  |  e-ISSN 2069-6175
ISSN-L 1454-0398
DOI: 10.37897/RJP

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POSSIBILITIES AND THERAPY LIMITATIONS IN A CASE OF ADRENOGENITAL SYNDROME

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ABSTRACT

Introduction. The adrenogenital syndrome with neonatal onset can manifest by repeated vomiting, dehydration and severe hydro-electrolytic disequilibria. The diagnostic approach, the metabolic reequilibration and the maintenance of the electrolytic balance in normal ranges by chronic treatment can be associated with difficulties.
Objective. We present a case of adrenogenital syndrome with severe neonatal onset, evolution accompanied by different complications, but with a prognosis improved by the adequate chronic treatment.
Material and method. A male child with small birth weight, was admitted in the Pediatrcs Clinic I, Targu-Mures, at the age of 3 weeks, with a severe clinic-biological syndrome by chronic vomiting and severe dehydration, and apparent macrogenitosomia. We performed a wide spectrum of investigations in acute phase, but also afterwards for the elaboration of the differential diagnosis.
Results. We identified persistent hyponatremia and hypopotassemia, an increased level of 17-OH progesterone (44.96 ng/ml) and the cortisol under the normal limit (3 ug/dl). We discovered significantly increased concentrations of ACTH (103 pg/ml in the morning) and total serum cholesterol (186.72 ng/dl). The macromolecular analysis pointed out the heterozygote genotype for the following mutations: 12G, R356W, P453S. The adequate chronic treatment with cortisone improved the prognosis; at the age of 1 years and 5 months, he presents an adequate height, weight and psychomotor development.
Conclusions. We fulfilled the conditions of the diagnosis of adrenogenital syndrome with neonatal onset in a heterozygote child. The small age the uncharacteristic symptomatology can be a burden for establishing an early diagnosis of adrenogenital syndrome.

Keywords: vomiting, newborn, hyponatremia

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EVALUAREA TROMBOCITOZEI REACTIVE POSTSPLENECTOMIE LA PACIENTUL PEDIATRIC

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Revista Romana de PEDIATRIE | Volumul LXV, Nr. 2, An 2016
ISSN 1454-0398  |  e-ISSN 2069-6175
ISSN-L 1454-0398
DOI: 10.37897/RJP

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EVALUAREA TROMBOCITOZEI REACTIVE POSTSPLENECTOMIE LA PACIENTUL PEDIATRIC

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REZUMAT

Trombocitoza reprezintă creşterea numărului de trombocite peste 500.000/mm³.
Obiectiv. Obiectivul acestui studiu este de a evalua frecvenţa şi severitatea trombocitozei reactive la pacienţii pediatrici splenectomizaţi.
Material şi metodă. Am realizat un studiu retrospectiv în care au fost incluşi 20 de pacienţi (4-16 ani) care au suferit o intervenţie de splenectomie în intervalul 2006-2015. Criteriile de includere în studiu au fost: pacienţi cu vârsta sub 18 ani, care au fost splenectomizaţi şi care au dezvoltat trombocitoză ulterior intervenţiei.
Rezultate. În lotul studiat, 80% dintre pacienţii splenectomizaţi (16) au dezvoltat o formă de trombocitoză; în 4 cazuri formă severă (trombocite > 1milion/mm3), iar în 7 cazuri formă uşoară. Nu s-au observat diferenţe semnificative în repartiţia pe sexe a pacienţilor (9 de sex feminin şi 11 de sex masculin). La 13 dintre pacienţi trombocitoza s-a remis după 30 de zile şi doar în 3 dintre cazuri episodul a durat >360 de zile. Fenomene trombotice s-au observat la un singur pacient din lotul studiat. Toţi pacienţii au beneficiat de măsuri de tromboprofilaxie şi hidratare, iar 2 cazuri au necesitat asociere terapeutică de Hidoxiuree.
Concluzii. Studiul nostru relevă o frecvenţă crescută a trombocitozei după splenectomie (80%), cu un vârf maxim de incidenţă la 2-10 zile postchirurgical, trombocitoza fiind în general benignă şi cu remisie spontană; evenimente trombotice s-au semnalat într-un singur caz, iar trombocitoza severă a fost mai frecventă după splenectomia de cauză traumatică.

Cuvinte cheie: trombocitoză, splenectomie, copil

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TROMBOCYTOSIS FOLLOWING SPLENECTOMY IN PEDIATRIC PATIENTS

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Revista Romana de PEDIATRIE | Volumul LXV, Nr. 2, An 2016
ISSN 1454-0398  |  e-ISSN 2069-6175
ISSN-L 1454-0398
DOI: 10.37897/RJP

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TROMBOCYTOSIS FOLLOWING SPLENECTOMY IN PEDIATRIC PATIENTS

, , , , , , , and

ABSTRACT

Thrombocytosis represents a platelet count over 500.000/mm³.
Objective. The aim of this study is to evaluate the frequency and gravity of reactive thrombocytosis in pediatric patients who underwent splenectomy.
Material and method. We performed a retrospective study including 20 patients (4-16 years old) who underwent splenectomy between 2006-2015. The inclusion criteria in the study were: patients with the age under 18 years, who underwent splenectomy independently by the cause, and who developed afterwards thrombocytosis.
Results. In the studied group 64% of the splenectomised patients (16) developed a form of thrombocytosis. In 4 cases – severe form (Platelets > 1 million/mm3) and in 7 cases a mild form. There were not noticed any significant differences regarding the gender repartition of the patients (9 were females, and 11 were males). In 13 patients, thrombocytosis disappeared after 30 days, and only in 3 cases, the episode lasted more than 360 days. Thrombotic phenomena were noticed only in one patient from our study group. All the patients benefited from thromboprophylaxis and hydration measures, and 2 cases needed associated treatment with Hydroxyurea.
Conclusions. Our study reveals an increased frequency of thrombocytosis after splenectomy (80%), with a maximum peak of incidence in 2-10 days following the intervention, thrombocytosis being generally benign and self-limited; still one of the cases experienced thrombotic complications and severe thrombocytosis was more frequent after post-traumatic splenectomy.

Keywords: thrombocytosis, splenectomy, child

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DIFICULTATI DE DIAGNOSTIC IN HIPOGLICEMIA SUGARULUI

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Revista Romana de PEDIATRIE | Volumul LXIV, Nr. 3, An 2015
ISSN 1454-0398  |  e-ISSN 2069-6175
ISSN-L 1454-0398
DOI: 10.37897/RJP

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DIFICULTATI DE DIAGNOSTIC IN HIPOGLICEMIA SUGARULUI

, , , , and

REZUMAT

Introducere. Hipoglicemia cu diversele ei cauze este frecvent semnalată în patologia pediatrică, începând din perioada neonatală şi până în adolescenţă. Prin consecinţele ei atât cele imediate (convulsii, comă), cât şi cele tardive (afectare neurologică), hipoglicemia trebuie cunoscută, prevenită, evitată şi tratată.
Obiective. Prezentarea evoluţiei clinice a unei paciente cu hipoglicemie severă simptomatică încă din perioada de nou-născut. De la naştere, valoarea glicemiei prezintă o dinamică fluctuantă, iar scăderea glicemiei, izolat, nu reprezintă neapărat o situaţie patologică, dar poate fi un simptom al unei patologii subiacente.
Material şi metodă. Pacienta este internată în clinica noastră la vârsta de nou-născut, în vederea stabilirii etiologiei şi tratamentului hipoglicemiilor. Am realizat profilul biologic şi hormonal, am efectuat determinări sanguine metabolice şi teste genetice.
Rezultate. Am stabilit diagnosticul de hipoglicemie severă persistentă. Interpretarea rezultatelor a fost îngreunată de evoluţia trenantă a bolii, vârsta mică şi complianţa scăzută din partea familiei.
Concluzii. Hipoglicemia persistentă are cauze multiple. Managementul unui sugar cu hipoglicemie severă poate fi dificil şi impune o abordare complexă.

Cuvinte cheie: hipoglicemie, sugar, gena Duarte

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DIAGNOSIS DIFFICULTIES IN INFANT’S HYPOGLYCEMIA

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Revista Romana de PEDIATRIE | Volumul LXIV, Nr. 3, An 2015
ISSN 1454-0398  |  e-ISSN 2069-6175
ISSN-L 1454-0398
DOI: 10.37897/RJP

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DIAGNOSIS DIFFICULTIES IN INFANT’S HYPOGLYCEMIA

, , , , and

ABSTRACT

Introduction. Hypoglycemia with its diverse causes is frequently encountered in the pediatrics pathology, beginning with the neonatal period and until the adolescent one. Through its consequences, both, the early (seizures, coma) and the late ones (neurological impairment), hypoglycemia must be identified, prevented, avoided and treated.
Objectives. To present the clinical evolution of a female patient with severe, symptomatic hypoglycemia even since the neonatal period. Since birth, the value of glycaemia presents a fluctuant dynamic, and the isolated decrease of glycaemia does not necessary represent a pathological situation, but can be a symptom of an underlying disorder.
Material and method. The patient is admitted in our clinic at the age of newborn in order to establish the etiology and treatment of hypoglycemia. We achieved the biologic and hormonal profile, we performed metabolic blood determinations and genetic tests. Results. We established the diagnosis of severe persistent hypoglycemia. The burden of the results interpretation was represented by the prolonged evolution of the disease, the small age and the family’s low compliance.
Conclusions. Persistent hypoglycemia has multiple causes. The management of an infant with severe hypoglycemia can be difficult and it imposes a complex approach.

Keywords: hypoglycemia, infant, Duarte gene

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Body composition of children with malignant disease

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Revista Romana de PEDIATRIE | Volumul LXI, Nr. 1, An 2012
ISSN 1454-0398  |  e-ISSN 2069-6175
ISSN-L 1454-0398
DOI: 10.37897/RJP

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Body composition of children with malignant disease

, , and

ABSTRACT

Background. Nutritional status, as represented by body composition, is an important considerant in the treatment of pediatric cancer patients because it is linked to poor outcomes.

Objective. We aimed to compare nutritional status with children with malignant disease at diagnosis as compared to children with nononcological disease.

Material and method: A prospective study was performed on 90 children hospitalised in the Pediatric Clinic I Tg. Mures, between November 2009 - July 2011. The children were divided into two groups: group 1, children with malignant disease (35 children) and group 2-control group, children with different pediatric diseases (55 children). We have done anthropometric and biochemical measuremeants: weight, height, body mass index, middle upper arm circumference, tricipital skin fold thickness, total serum protein and albumin, Insulin-like growth factor-1, total cholesterol, tryglicerides and sideremy. We studied the anthropometric and biochemical parameters compared to the two groups and performed statistical correlations.

Results. The weight and height of children in group I was much lower compared with group II, without statistically significant difference (p>0.05). We also found a statistically significant difference among the anthropometric parameters of the arm: middle upper arm circumference – MUAC (-1.58 DS versus -0.74 DS ) with p = 0.022 and tricipital skin fold thickness – TSF (-1.34 DS versus -0.74 DS) with p = 0.05. Regarding biochemical parameters, between the two groups, we observed a significant difference(p<0.05) with the total serum protein and albumin, IGF-1, sideremy and tryglicerides.

Conclusions. This study showed that the children’s group diagnosed with cancer has a poor nutritional status than the control group; the anthropometric parameters of the arm in conjunction with biochemical parameters best reflects the body composition.

Key words: child, malignant disease, anthropometric parameters, biochemical parameters

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COMPOZITIA CORPORALA LA COPIII CU BOALA MALIGNA

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Revista Romana de PEDIATRIE | Volumul LXI, Nr. 1, An 2012
ISSN 1454-0398  |  e-ISSN 2069-6175
ISSN-L 1454-0398
DOI: 10.37897/RJP

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COMPOZITIA CORPORALA LA COPIII CU BOALA MALIGNA

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REZUMAT

Introducere. Statusul nutriţional, reprezentat de compoziţia corpului, este un considerent important în tratamentul pacienţilor pediatrici oncologici deoarece influenţează evoluţia bolii. Obiectiv. Ne-am propus să evaluăm comparativ statusul de nutriţie la copiii cu boală malignă la momentul diagnosticării bolii faţă de copiii cu afecţiuni nononcologice.

Material şi metodă. Am efectuat un studiu prospectiv pe un număr de 90 de copii internaţi în Clinica Pediatrie I Tg- Mureş în perioada noiembrie 2009 – iulie 2011. Copiii au fost împărţiţi în două loturi: lotul I, reprezentat de copii cu boală malignă (35 copii) şi lotul II – martor, reprezentat de copii cu diverse afecţiuni pediatrice (55 copii). Am efectuat măsurători antropometrice şi biochimice: greutatea, înălţimea, indicele de masă corporală, perimetrul mediu al braţului, pliul cutanat tricipital, proteine totale şi albumine serice, IGF-1, colesterol total, trigliceride şi sideremie. Am urmărit comparativ parametrii antropometrici şi biochimici la cele două loturi şi am efectuat corelaţii statistice.

Rezultate. Greutatea şi înălţimea copiilor din lotul I a fost mai mică faţă de lotul II, fără o diferenţă semnificativă statistic (p>0,05). Am constatat o diferenţă semnificativă statistic în rândul parametrilor antropometrici ai braţelor: perimetrul mediu al braţului – MUAC (-1,58 DS la lotul I şi -0,74DS la lotul II) cu p=0,022 şi pliul cutanat tricipital –TSF (-1,34 DS la lotul I şi -0,76 DS la lotul II) cu p=0,05. Urmărind parametrii biochimici, am observat o diferenţă semnificativă statistic (p<0,05) între cele două loturi în ceea ce priveşte valoarea proteinelor totale, albuminele serice şi IGF-1, sideremia şi trigliceridele.

Concluzii. Studiul demonstrează că pacienţii diagnosticaţi cu cancer prezintă status nutriţional precar faţă de pacienţii nononcologici; parametrii antropometrici ai braţelor, coroboraţi cu parametrii biochimici reflectă cel mai bine compoziţia corporală.

Cuvinte cheie: copil, boală malignă, parametru antropometric, parametru biochimic

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