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- Acute Alithiasic Cholecystitis and Human Herpes Virus Type-6 Infection: First CasePublication . Gomes, MM; Antunes, H; Lobo, AL; Branca, F; Correia-Pinto, J; Moreira-Pinto, JA three-year-old male child presented with erythematous maculopapular nonpruritic generalized rash, poor feeding, vomiting, and cramping generalized abdominal pain. He was previously healthy and there was no family history of immunologic or other diseases. On examination he was afebrile, hemodynamically stable, with painful palpation of the right upper quadrant and positive Murphy's sign. Laboratory tests revealed elevated inflammatory markers, elevated aminotransferase activity, and features of cholestasis. Abdominal ultrasound showed gallbladder wall thickening of 8 mm with a positive sonographic Murphy's sign, without gallstones or pericholecystic fluid. Acute Alithiasic Cholecystitis (AAC) was diagnosed. Tests for underlying infectious causes were negative except positive blood specimen for Human Herpes Virus Type-6 (HHV-6) by polymerase chain reaction. With supportive therapy the child became progressively less symptomatic with gradual improvement. The child was discharged on the sixth day, asymptomatic and with improved analytic values. Two months later he had IgM negative and IgG positive antibodies (1/160) for HHV-6, which confirmed the diagnosis of previous infection. In a six-month follow-up period he remains asymptomatic. To the best of our knowledge, this represents the first case of AAC associated with HHV-6 infection.
- Acute pancreatitis in children : a tertiary hospital reportPublication . Antunes, H; Nascimento, J; Mesquita, A; Correia-Pinto, JINTRODUCTION: The incidence of acute pancreatitis (AP) in children has increased significantly in the past two decades. OBJECTIVE: All cases of AP, acute recurrent pancreatitis (ARP), and chronic pancreatitis examined between May 2002 and May 2012 at Hospital de Braga, Portugal, were reviewed. MATERIAL AND METHODS: Patients were identified by searching the hospital's electronic discharge records for the International Classification of Disease, Ninth Revision (ICD-9) code 577.0 (acute pancreatitis). ARP was considered as two or more episodes of AP per year or more than three episodes over a lifetime with intervening return to baseline. The following data were analyzed: demographic information, clinical, laboratory and imaging test results, etiology of pancreatitis, medical and surgical management, length of hospitalization, and outcome. The clinical and laboratory factors used in the pediatric acute pancreatitis severity score system and computed tomography severity index (CTSI) score were compared between patients with mild and severe disease. RESULTS: A total of 37 patients, 31 episodes of AP and 6 patients with ARP, were documented. The most prevalent etiologies were biliary stones/sludge (24.3%) and trauma (16.2%). Admission elevated white blood cell count (p=0.011), 48-h trough calcium (p=0.007), and 48-h rise in blood urea nitrogen (p=0.025) correlated significantly with disease severity. CTSI on admission had a score below 4 in three patients with severe disease. CONCLUSION: This Portuguese pediatric pancreatitis report highlights the multiple and complex etiology of this disease. Better pediatric scoring systems and management algorithms are needed.
- Alimentação e nutrição do lactentePublication . Guerra, A; Rêgo, C; Silva, D; Ferreira, GC; Mansilha, H; Antunes, H; Ferreira, R
- O atraso de desenvolvimento nas crianças com anemia por deficiência de ferro. Poderá ser revertido pela terapêutica com ferro?Publication . Antunes, H; Gonçalves, S; Teixeira-Pinto, A; Costa-Pereira, AOBJECTIVE: To compare development of 17 children aged 12 months with iron deficiency anaemia (IDA) and 18 controls without IDA after a 3-months follow-up period and iron therapy in the IDA group. SUBJECTS AND METHODS: Development of all children was evaluated using the Griffith's Scale. Data was collected on parent's social class and education, breast-feeding, number of siblings and clinical nutritional status. RESULTS: At 12 months children with IDA had significantly lower development scores--mean (sd)--than those without IDA: 112(5) vs. 121(7). At 15 months, after iron therapy, there were no significant differences between cases and controls. Non-IDA children showed significantly lower development scores at 15 months when compared with 12 months (121 vs 115). CONCLUSIONS: The study shows that iron therapy can revert some of the adverse effects in the development of children with IDA and therefore both IDA prevention and treatment can be justified.
- Autoimmune Hemolytic Anemia and Hodgkin's Disease: An Unusual Pediatric AssociationPublication . Gomes, MM; Oliva, T; Pinto, AAutoimmune hemolytic anemia (AIHA) is a recognized complication of lymphoproliferative disorders. AIHA associated with Hodgkin's disease (HD) is uncommon especially in the pediatric population. The diagnosis of AIHA is usually associated with HD at the time of initial presentation or during the course of disease, but it could precede it by years to months. In adults the association of AIHA and HD is more frequent in advanced stages and in the nodular sclerosis and mixed cellularity type HD. Warm immune hemolytic anemia is mainly controlled with steroids and chemotherapy. We report a case of a pediatric patient with direct antiglobulin positive test at the diagnosis of a late relapse of stage III B mixed cellularity type HD.
- Avaliação do desempenho de três câmaras de expansãoPublication . Abreu, S; Silva, LF; Teixeira, S; Marques, HC; Ramalhete, N; Antunes, HIntroduction: Several aspects are known to influence the drug distribution within the low respiratory tract, with particular emphasis on those related to the inhalation device. The aim of this work was to assess the performance of three spacers in the drug release, and also the quantity of active agent deposited inside these devices. Materials and Methods: In order to evaluate the behaviour of particles in suspension delivered through the Ventilan®HFA inhaler coupled to three different spacers (Volumatic®, AeroChamber MAX® and NebuChamber®) the Multistage Liquid Impinger (MSLI) was used, according to the Portuguese Pharmacopoeia. The mass of salbutamol sulphate deposited on the different impinger compartments and inside the spacer was determined by spectrophotometry, with the purpose of determining the percentage of cumulative mass for each spacer, and then the fine particle fraction. The results were compared statistically using a one-way analysis of variance (one-way ANOVA) with a Bonferroni post-hoc test. Results: About 40 to 50% of salbutamol sulphate was found deposited in the body of the three spacers. This deposition was slightly lower for NebuChamber® (average ± standard deviation of 43.8 % ± 11.6 %), in relation to Volumatic® (p=0.351) or AeroChamber MAX® (p=0.115). The fine particle fraction reached values of 28.2 ± 4.1%, 29.6 ± 2.4% and 30.9 ± 6.7% for Volumatic®, AeroChamber MAX® and NebuChamber®, respectively. Conclusion: The spacers showed to have similar efficiencies in the delivery of salbutamol sulphate in the last stages, and there was no relation between the results and the spacers characteristics such as volume, shape and material. Therefore, Volumatic® appears to be perfect for hospital use, since its big volume does not constitute a disadvantage, and its lower cost, when compared to the remaining two spacers, represents an advantage of utmost importance for public hospitals.
- Bisalbuminemia: A Rare Variant of AlbuminPublication . Garcez, C; Carvalho, SINTRODUCTION: Bisalbuminemia is a qualitative disorder of albumin and it is defined by the coexistence in the same individual of two types of serum albumin with different electrophoretic mobility. There are two forms: hereditary and permanent, or acquired and transient. CASE REPORT: Girl, 17-years-old, referenced to the hospital consult after incidental finding of bisalbuminemia detected in plasma protein electrophoresis performed within the investigation of diminished muscular tone in the lower limbs. Physical examination was normal. Secondary causes of bisalbuminemia were excluded. Later, it was confirmed the same disorder in plasma protein electrophoresis performed to her 14-years-old brother and mother. DISCUSSION: We describe a rare case of hereditary bisalbuminemia in a portuguese family. In general, this condition shows no pathological significance, however it is of interest to the clinicians the knowledge of this analytic change for better orientation of their patients.
- Broad clinical involvement in a family affected by the fragile X premutationPublication . Chonchaiya, W; Utari, A; Pereira, GM; Tassone, F; Hessl, D; Hagerman, RJThe mutations in the FMR1 gene have been described as a family of disorders called fragile X-associated disorders including fragile X syndrome, fragile X-associated tremor/ataxia syndrome, primary ovarian insufficiency, and other problems associated with the premutation, such as hypothyroidism, hypertension, neuropathy, anxiety, depression, attention-deficit hyperactivity disorders, and autism spectrum disorders. The premutation is relatively common in the general population affecting 1 of 130 to 250 female individuals and 1 of 250 to 800 male individuals. Therefore, to provide appropriate treatment and genetic counseling for all of the carriers and affected individuals in a family, a detailed family history that reviews many of the disorders that are related to both the premutation and the full mutation should be carried out as exemplified in these cases. To facilitate the integration of this knowledge into clinical practice, this is the first case report that demonstrates only premutation involvement across 3 generations.
- A case of pediatric paraparesis secondary to an idiopathic acute transverse myelitisPublication . Teixeira, J; Carvalho, S; Martins, S; Pontes, T; Machado, A; Antunes, HIntroduction: Acute transverse myelitis (ATM) refers to a frequently idiopathic, segmental spinal cord inflammation. It is a rare condition, in particular in children, and not previously reported in a family retinitis pigmentosa (RP) clinical setting. Case Report: An 11-year-old previously healthy girl, with a family history of RP, presented with a subacute flaccid paraparesis, with bilateral, up to the fourth dorsal level, mixed sensory hypoesthesia and autonomic dysfunction. Brain and spinal cord magnetic resonance imaging (MRI) showed an extensive, T2-hyperintense, non-contrast enhancing lesion from the second to fifth dorsal levels. Cerebrospinal fluid (CSF) and lab studies were normal, as the ophthalmologic observation. Treated with high-dose corticosteroids and intensive physical therapy,a significant recovery could be seen. Conclusion:Early pharmacological and physical treatment is fundamental and may indeed change the prognosis of this disease ATM. The family history of RP, although probably incidental, brings nevertheless the issue of a possible etiological contribution, or pathologic common pathways.
- A case of systemic pseudohypoaldosteronism with a novel mutation in the SCNN1A genePublication . Silva, N; Costa, M; Silva, A; Sá, C; Martins, S; Antunes, A; Marques, O; Castedo, S; Pereira, AWe report a neonatal case of systemic pseudohypoaldosteronism type 1 caused by a novel mutation in the SCNN1A gene (homozygous c.1052+2dupT in intron 3) in which the patient presented with life-threatening hyperkalemia, hyponatremia and metabolic acidosis. It remains uncertain if there is genotype-phenotype correlation, due to the rarity of the disease. This mutation, which to our best knowledge has not been described before, was associated with a very severe phenotype requiring aggressive therapy.