Ingrith Miron

Initial Management of Advanced Burkitt Lymphoma in Children: Is There Still a Place for Surgery?

This retrospective study compared the overall survival, the event-free survival, and the timing of chemotherapy in patients with advanced Burkitt lymphoma with and without laparotomy. Thirty-five patients with advanced abdominal Burkitt lymphoma treated at least partially at the Centre Léon Bérard between 1981 and 1992 were included in this study. The diagnosis was obtained by laparotomy (LAP group) in 21 patients (17 stage III, 4 stage IV) and by other methods (non-LAP group) in 14 patients (5 stage III, 9 stage IV). The overall survival (71 and 93%) and the event-free survival (66 and 79%) were similar in the LAP and non-LAP groups, and the relapse rate was five (three local) in the LAP group compared with three (none local) in the non-LAP group. The local complication rate (9 of 21 versus 2 of 14) and the toxic death rate (2 of 21 versus 1 of 14) were slightly higher in the LAP group. Laparotomy also caused delays in therapy and increased the overall hospital stay. The mean interval from diagnosis to the start of the fourth course of chemotherapy was 57 days compared with 48 days and the average hospital stay was 44.4 days compared with 39 days for the LAP and non-LAP groups, respectively. Because advanced Burkitt lymphoma can be diagnosed by fine-needle aspiration, and chemotherapy cures more than 80% of the patients, there is no need for initial surgery, apart from acute emergencies. Furthermore, laparotomy delay chemotherapy and might reduce the survival rate.

Diagnostic Difficulties in a Pediatric Insulinoma: A Case Report.

AbstractInsulinomas are functional neuroendocrine pancreatic tumors rarely encountered in pediatric pathology.Insulinomas are usually solitary and sporadic, but may occur in association with multiple endocrine neoplasia type 1. Whipples triad—hypoglycemia, simultaneous compatible adrenergic and/or neurological signs, and relief of symptoms upon the administration of glucose—remains the fundamental diagnostic tool.We report a case of insulinoma in an 11-year-old boy with malnutrition and mild psychic retardation.History revealed neuroglycopenic symptoms associated with hypoglycemia that returned to normal values after glucose intravenous infusion; before admission in our unit, the levels of circulating insulin, as well as the abdominal ultrasound and abdominal computed tomography scan, were reported within normal range. During hospitalization in our service, the glycemic curves showed recurring low values associated with low glycated hemoglobin, positive fasting test, and elevated C-peptide. The pancreatic ultrasound was inconclusive, but the magnetic resonance imaging revealed a high signal focal area with a diameter of 1 cm, located in the tail of pancreas. Conventional enucleation of the lesion prompted a spectacular normalization of glucose metabolism and the alleviation of the main clinical symptoms. The child had a favorable evolution in the clinical follow-up, presenting with weight gain and progressive remission to complete disappearance of most symptoms—except for the mental impairments.Although in our case Whipples triad was apparent from the beginning, the diagnosis was delayed due to the failure of conventional imaging methods in locating the tumor. Weight loss and mental impairment contributed to the diagnosis pitfalls.Pediatricians should be aware of confusing and nonspecific symptoms, especially when children with insulinoma present mental or neurological retardation. Despite the existence of medical regimens, surgery remains the gold standard for the therapeutic approach to this condition.

Unusual Endoscopic Findings in Children: Esophageal and Gastric Polyps: Three Cases Report.

Abstract Isolated polyps of the upper digestive tract are rarely diagnosed in children, being usually an incidental finding during endoscopic exploration. The diagnostic, therapy, and outcome of these lesions are based on endoscopy and pathology. In a 5-year period, clinical features, topography, size, pathology, therapeutics, and progression of esophagogastric polyps founded in children addressed to our pediatric gastroenterology unit were studied. The authors encountered 3 lesions in teenagers aged 13 to 17 years two males (2M), from a total number of 2140 upper digestive endoscopies (0.14%). All patients presented with pirosis, epigastric pain, and vomits; one of the children had end-stage renal disease and Kabuki syndrome. Endoscopic and pathologic findings were 2 esophageal polyps, an inflammatory one, and another containing goblet cells and a double-headed hyperplastic gastric polyp. Two patients received proton pump inhibitors without any improvement in subsequent endoscopic evaluations. The difficulties related to age group, underlying conditions, debatable response to acid suppression, and limited experience in pediatric therapeutic endoscopy selected significantly the effectiveness of treatment. The rarity of these lesions requires an individualized management, the endoscopic diagnostic, and therapeutic gesture depending on the symptoms, type, location, comorbidities, and team experience.

Focal segmental glomerulosclerosis in children complicated by posterior reversible encephalopathy syndrome

An uncommon side effect of cyclosporine A (CsA) use is posterior reversible encephalopathy syndrome (PRES). PRES usually develops because of disturbed capacity of posterior cerebral blood flow to autoregulate an acute rise in blood pressure. We present the case of a 10-year-old girl who was previously diagnosed in our department with focal segmental glomerulosclerosis. She was treated with CsA and developed seizures, progressive loss of consciousness, and visual disturbance on the 7th day of treatment. Brain magnetic resonance imaging showed degeneration of white matter with diffuse demyelination in the parietal and posterior occipital lobes, consistent with the diagnosis of PRES. Cases of PRES reported in children are usually secondary to immunosuppressive therapy in oncological and haematological diseases. Our case is the fifth reported case of focal segmental glomerulosclerosis in children treated with CsA and complicated by PRES. Rapid recognition of PRES and stopping neurotoxic therapy early are essential for a good prognosis.

Congenital acute myeloid leukemia in Down syndrome

Congenital leukemia is an uncommon myeloproliferative disorder usually diagnosed in the first month of life. Newborns with Down syndrome have an increased susceptibility to acute megakaryoblastic leukemia. We report a case of a newborn with Down syndrome diagnosed with acute myeloid leukemia. The diagnosis was established by clinical and hematological work-up.

Silent presentation of multiple metastasis Burkitt lymphoma in a child: A case report and review of the literature

Rationale: The Burkitt lymphoma (BL) is a very aggressive B-cell non-Hodgkins lymphoma. It accounts for 34% of lymphoma cases in children. Patient concerns: We present the case of a 6-year-old boy diagnosed with BL, who presented multiple contrasting elements of the disease: silent symptomatology, without involvement of the bone marrow at first, but with multiorgan infiltration and a fast evolution, despite starting the treatment shortly after the symptoms appeared. Diagnoses: He was diagnosed with BL after immunophenotyping from the pleural fluid. Interventions: After a week from admission, chemotherapy was initiated according to protocol NH-BFM therapeutic group III—cytoreductive phase in the acute care ward and subsequently the AA 24 treatment. Outcomes: Following the treatment, the patient developed medullary aplasia and cutaneous toxicity. The patients general state remained severe during the hospitalization. Lessons: Even though the prognosis of BL has improved over time (up to 90% survival rate), in this case the evolution was unfavorable. In our patient, the symptoms appeared abruptly. They appeared late in the phase of multiple-organ dissemination, which generated the pessimistic prognosis.

Different evolution in the treatment of a severe persistent asthma in 2 twins: Case report and review of the literature

Rationale: Asthma is a multifactorial disease with complex genetic inheritance. In children under the age of 5 years, the diagnosis of asthma is a challenge. Patient concern: We present the case of twin sisters under the same treatment for persistent asthma, but with different evolution over the time. Diagnoses: One of the sister is diagnosed with severe persistent bronchial asthma associated with bronchiectasis and dyslipidemia and the other one only with mild persistent asthma. Interventions: At each admission the treatment for the exacerbations and the underlying respiratory infections was represented by antibiotics, short-acting &bgr;2 agonists, and, sometimes, oxygen and systemic corticosteroids. As chronic treatment, they received in the last period inhaled corticosteroids associated with long-acting &bgr;2 agonist. Outcomes: Until the age of 6.5 years, they had similar diagnoses and treatment; from this point one was asymptomatic, with normal pulmonary function tests. The other one had a more complicated evolution which led to a severe crisis by the age of 10 years old. Lessons: Although asthma is a multifactorial disease with complex genetic inheritance, the genetics has its limits. Our twins had a similar onset with the same genetic inheritance, with the same risk factors, with the same comorbidities and with the same treatment. In this context, different evolutions of severe persistent asthma require more extensive genetic investigations. Patient concern: We present the case of twin sisters under the same treatment for persistent asthma, but with different evolution over the time.

P103 Prognostic considerations in late diagnosed cystic fibrosis

Cystic fibrosis is the most common life-limiting recessive genetic disorder in the white population. In the absence of neonatal screening, in Romania the diagnosis is established based on the iontophoresis test in children that present clinical respiratory and/or digestive symptoms. The authors present the case of a patient aged 15 years and 3 months, that submitted in the Oncohematology Paediatric Clinic with suspected abdominal tumour detected during an echography performed ambulatory. Anamnesis showed accelerated intestinal transit in the early years (6–7 steatorrhoeic stools/day) and persistent cough associated with weight loss, about a month before admission. Abdominal ultrasound, upper gastrointestinal endoscopy, abdominal and thoracic CT scan, and abdominal MRI with contrast were performed, that allowed to establish the diagnosis of liver cirrhosis, portal hypertension with esophageal varices grade 1, fibrosis and bronchiectasis manly of the left lung and pancreatic pseudochist. To assess the degree of fibrosis, fibroscan was performed: Child PUGH = B; MELD=17. Also, were excluded the postinfectious, autoimmune cirrhosis, Wilson’s disease, alpha 1-antitrypsin deficiency. Iontophoresis test diagnosed cystic fibrosis, pulmonary and digestive type. Microbiological examinations of hypopharyngeal aspirates showed pulmonary infection with Staphylococcus aureus, Pseudomonas aeruginosa, Haemophilus influenzae. Under replacement therapy with pancreatic enzymes, liver protection and ursodeoxycholic acid, targeted antibiotics, mucolytics, fat-soluble vitamin therapy replacement, the evolution was slow favourable. The authors conclude that it is necessary to introduce neonatal screening for early diagnosis of cystic fibrosis and rapid initiation of treatment as to improve long-term prognosis and survival rate in these patients.

P380 Therapeutic management of immune thrombocytopenia – single centre experience

Background The Immune thrombocytopenia (ITP) is one of the most frequent haematological disorders and it is considered to be the result of an immune-mediated process. Some cases resolve within few weeks, but the majority of them need treatment. Aims The analysis of evolution of children with ITP in one unit. Methods The medical records of 83 patients diagnosed with ITP were reviewed focusing especially on treatment strategies. They were diagnosed and treated between January 2012-August 2016 in the IV-th Clinic of Saint Mary’s Hospital for Children, Iasi. Results 47 out of 83 patients are female and 40 are male. First line therapy was used as it follows: 24 patients received intravenous immunoglobulins and 59 received corticosteroid therapy. Second-line therapy consisted in Azathioprine and Rituximab. 9 patients received Azathioprine and 3 received Rituximab. In this study group we tried to categorise the patients according to the terminology established by the International Working Group (2009) in: newly diagnosed, persistent ITP and chronic ITP. Thus, we found that 9 patients evolved towards persistent form and 14 developed chronic ITP. In the group of chronic ITP patients, 6 are diagnosed with refractory form of the disease. Conclusion All our patients received treatment, even if the international guidelines are recommending the ‘watchful waiting’ in the cases that don’t show risk of severe bleeding. Usually is very hard to convince the parents that the illness is, in most of the cases, self-limited. Therefore, in our clinic the management of new cases of ITP needs a better standardisation in relation with the severity of bleeding and not with the number of platelets.

Hydrocephalus secondary to chemotherapy in a case of prenatally diagnosed giant immature grade 3 sacrococcygeal teratoma: A case report and literature review

Introduction: Sacrococcygeal teratoma (SCT) is a rare tumor in the general population, arising from multipotent stem cells. Whereas most of the cases diagnosed postnatally have good prognosis, the rate of mortality and morbidities associated with prenatally diagnosed SCT remain high, with a reported mortality rate of 30% to 50%. The outcome of fetal SCT can be unpredictable, with some cases with slow growth during fetal life, whereas others grow rapidly, causing multiple complications; also, some of these tumor will develop triggering fetal (preterm delivery, high-output cardiac failure, hydrops fetalis, intrauterine death) or maternal complications (distocia, placentomegaly, maternal mirror syndrome—preeclampsia). Even if prenatal criteria seem to define tumors at risk, it can not totally predict postnatal outcome as treatment-related complications can occur. We present a case of giant prenatally detected SCT. The case was diagnosed at 24th week of gestation, and was closely monitored by serial ultrasound. The morphology of the lesion was defined by fetal MRI performed at 25th week of gestation. A baby girl with a huge sacrococcygeal tumor was born and surgery was performed 48 hours later. Pathological examination revealed a grade 3 immature teratoma. Because of the tumor size and pathological aspect, adjuvant chemotherapy was considered. The outcome was complicated by wound infection, sepsis, and subsequent hydrocephalus, induced by chemotherapy-induced immunosuppression. Conclusion: Our case emphasizes not only the importance of prenatal monitoring of these cases but also the importance of individualized postnatal management, as unusual and unpredictable complications can occur and affect outcome.