Helicobacter pylori infection and gastroesophageal reflux in children.

Some studies suggest that Helicobacter pylori (H. pylori) infection would be a protective factor for the gastroesophageal reflux. The aim of this study was to explore this fact. A group of 72 children, admitted in a pediatric gastroenterology regional center in Northeast Romania, diagnosed with gastroesophageal reflux by 24-hour continuous esophageal pH monitoring (results were interpreted using the Boix-Ochoa score), underwent upper endoscopy with gastric biopsy to detect the presence of H. pylori by the rapid urease testing and for bacteriological and histologic examination. 19 children (26.39%) had H. pylori infection, while 53 (73.61%) did not. The grade of esophagitis was classified according to the Los Angeles classification system. Out of 47 children with esophagitis A, 16 (34.04%) had H. pylori infection, while out of the 25 children with esophagitis B, only 3 (12%) had H. pylori infection, with statistic significance (χ2 = 54.69, P << 0.05, 95% confidence interval [CI]). Regarding the value of the Boix-Ochoa score, it appears that the presence of the H. pylori determines lower pH-metry scores (F = 8.13, P = 0.0015, 95% CI). The presence of the H. pylori was not an important factor in the gastroesophageal reflux. On the other hand its relationship with esophagitis appears to be inverse ratio. The fact that the H. pylori presence is statistically greater in the grade A esophagitis could confirm the hypothesis that the bacteria would slow down the development of the esophagitis.

Childhood rhabdomyosarcoma. Anatomo-clinical and therapeutic study on 25 cases. Surgical implications.

UNLABELLED: Rhabdomyosarcomas (RMS) are the most frequent soft tissue sarcomas of childhood. Despite advances in knowledge about biological pathways of tumorigenesis, risk stratification and multimodal treatment, the immediate and long-term prognosis of these lesions in many countries with limited resources is still poor. PATIENTS AND METHODS: Twenty-five histologically confirmed pediatric RMS were recorded during the period of study. Demography, clinical presentation, diagnostic means, pretreatment staging and post-surgical grouping, histological type, therapy and outcome were evaluated. RESULTS: The mean age was 6.7 years; the group included 12 boys and 13 girls. Twelve lesions were localized in the genitourinary tract, eight in the trunk and extremities, two cases each in head and neck and retroperitoneum and one case in biliary tract. Primary surgical attempt was performed in 15 patients but only in nine of them underwent complete resection (three with free margins) other six cases achieving removal with residual disease. In 10 cases, solely biopsy was possible. Twenty-four patients received chemotherapy but only four cases performed radiation therapy. Overall survival rate was only 36% (nine cases). CONCLUSIONS: As mean feature children from our series had late presentation with locally extended (bulky and node positive) lesions and unfavorable sites. Improved multimodal management of RMS in recent years will probably lead to better survival curves in an increasing number of cases and an outstanding outcome in children with locally advanced disease.

Celiac disease with neurologic manifestations in children.

UNLABELLED: Celiac disease (CD) is an immune-mediated enteropathy triggered by the ingestion of gluten in genetically susceptible individuals and neurologic manifestations may be one of the presentations form. The aim of this study was to report the incidence of neurologic manifestations in children with CD. MATERIAL AND METHODS: Between 2000-2010, 48 children aged 2-18 years diagnosed with CD have been monitored. The diagnosis of CD was made by serological tests and intestinal biopsy. The study protocol included: measurement of weight and height, biological and immunological tests, histological examination, questionnaires filled out by parents about their child motor development and some neurologic signs, psychological exam, electroencephalogram, and brain CT-scan. RESULTS: 16 of the 48 children presented one or more neurologic symptoms as the onset manifestation of CD. The neurologic signs in order of frequency were: headache/migraine, attention-deficit/hyperactivity disorder, epileptic seizures, mental retardation, cerebellar ataxia and behavior disorders. Brain CT-scan showed cerebral calcifications in 3 patients with epilepsy, and atrophy in 2 cases with cerebellar ataxia. All children received gluten free diet, but a favorable course was noticed only in the children with migraine and epilepsy, in the other patients this diet having no influence on neurologic symptoms. CONCLUSIONS: This study proved the variety of neurologic symptoms that can be included in the clinical signs of celiac disease in pediatric patients. That is why in the presence of different neurologic symptoms of unknown etiology and resistant to treatment, celiac disease must be taken into account and laboratory investigations have to include intestinal biopsy and immunological test.

[Pierre Marie-Sainton cleidocranial dysplasia]. / Displazia cleidocraniana Pierre Marie-Sainton.

The paper presents a family with hereditary transmitted Marie-Sainton dysplasia affecting the father and his both children. This is a rare syndrome presenting an autosomal pattern of inheritance, characterized by a generalized defect in both membranous and endochondral bone formation resulting in clavicular aplasia, delayed ossification of the fontanelles and the sutures of the skull and prolonged retention of deciduous dentition with delayed eruption of the permanent teeth. The diagnosis is suggested by more or less complete clinical picture and confirmed by multiple radiological explorations (skull, thorax, spinal column, pelvis) and genetical examination. The genetic mutation for cleidocranial dysplasia (CCD) is found on chromosome six and is called CBFA1 (short for core biding factor al or RUNX2) and is the only gene known to be associated with CCD. The normal version of CBFA1 acts to induce osteoblasts which are the type of cells that lay down bone. Although associated psychosocial disorders can occur, the prognosis and life expectancy of this condition are favorable being conditioned however by the complexity of orthodontic procedures which are determinant for these patients life quality.

[The role of specific IgE to evolution and prognosis of cow's milk protein allergies in children]. / Rolul IgE specifice la copil în evolutia si prognosticul alergiei la proteinele laptelui de vaca.

UNLABELLED: Cow's milk allergy affects 8% of infants less than 1 year of age. The allergy is usually transient, with most children tolerating ingested cow'milk by age 3 years. This prospective study analyzes the clinical course, development of tolerance and risk for other atopy (asthma, rhinoconjunctivitis, atopic dermatitis) in children with cow's milk allergy. MATERIAL AND METHODS: We followed 71 infants hospitalized between January 2006 - January 2010 in two clinic of Pediatrics from Iasi and Galati with gastro-intestinal, respiratory and skin signs and symptoms of cow's milk allergy. In this study were identified atopic symptoms and diseases, family history of atopy, measured serum total IgE levels and was evaluated development of tolerance to cow's milk. IgE levels was measured at diagnosis, 12 months after diagnosis and recovery tolerance to cow's milk. Patients were followed to acquire tolerance to cow milk. RESULTS: The median age of the patients was 7.57 months +/- 2.73DS. IgE-mediated cow's milk allergy was detected in 40.85% (29 cases) of children at diagnosis. After 12 months of follow 7 (24.14%) of 29 cases initially IgE positive became negative. The first rechallenge was carried out 12 months after diagnosis at mean age 1.6 years (95%CI, 1.5-1.6 years) and the result was positive in 12 cases of IgE negative group. All children (100% of cases) with IgE-negative cow'milk allergy were tolerant by 3.0 years old (P < 0.0001) compared to 70.73% in children with positive IgE. In the end 17.24% of patiens with IgE-mediated cow milk allergy presented respiratory and skin atopic sings. Are there significant differences about the persistent cow'milk allergy between the group of children with positive IgE compared to negative IgE. (p = 0.1918, 95% CI). CONCLUSIONS: Most children recover their tolerance to cow milk during childhood and those with negative IgE even at young ages. Patients with positive IgE have an increased risk for allergic diseases, food and inhaled allergens sensitization and development of persistent cow's milk allergy.

[Height and weight growth delay and protein-energy malnutrition in children with chronic dialysis]. / Retardul staturo-ponderal si malnutritia protein-calorica la copilul dializat.

UNLABELLED: Growth retardation is an important problem in children with chronic renal disease, and malnutrition is a determinative factor. AIM: The study intends to assess the relationship between protein-energy malnutrition and stature-weight retardation in children enrolled in chronic dialysis program. MATERIAL AND METHOD: The study group was composed of 16 children (5 boys and 11 girls--sex ratio of 2.2) hospitalized in the IVth Nephrology Clinic at Clinical Emergency Hospital "St. Maria" Iasi, 13 rural and 3 urban, aged between 9 and 17 years, with chronic dialysis program. This was a follow-up study during a period of 4 years (2006-2009), resulting in correlations between anthropometric paremeters, biochemical, BIA and DEXA data. RESULTS: The stature-weight deficiency of the 16 patients was as follows: after an average period of 61.7 months of HD and 32.7 months of PD, in children older than 12 years (mean age 15.27 years), 7 of 10 had stature-weight deficits higher than (-3DS) or (-4DS). The group with less than (-2DS) stature-weight deficits showed the same mean age of 15.2 years, the protein energy-malnutrition was present in 2 cases (33%) and was attributed to a dialysis period shorter than 13.8 months. CONCLUSIONS: The late diagnosis of the disease (at an average age of 13 years), the long period of chronic dialysis program (over 39.5 months on average) and the early debut of malnutrition are favoring or worsening factors of stature-weight retardation. After correlating ESG with biochemical, BIA and DEXA data, in our group were identified 4 cases of moderate malnutrition and 9 cases of severe malnutrition.

[Hepato-pulmonary syndrome]. / Sindromul hepato-pulmonar (shp).

The hepato-pulmonary syndrome (HPS) is a rare complication of liver cirrhosis, with poor outcome; the term includes liver disease, increased alveolo-arterial gradient and intrapulmonary vascular dilations, described by Fluckiger, Kennedy and Knudson. HPS impairs survival in cirrhotic patients and the posttransplant outcome is altered in correlation with severity of HPS. Combined determination of SaO2 in clino- and orthostatic position by a pulsoximeter is a simple test for HPS identification in patients with chronic liver disease or non-cirrhotic portal hypertension.

[Arachnoid cyst associated with pyloric stenosis in a young boy]. / Chist arahnoidian asociat cu stenoza pilorica la un copil de vârsta mica.

An unusual association between an arachnoid cyst and a decompensated pyloric stenosis in a three years-old boy is presented. The little patient was admitted into hospital with haematemesis, melena, influenced generally condition and acute posthemorrhagic anaemia following aspirin intake for hypertermia. Specific intensive care was successful and the little patient was discharged but without an upper digestive endoscopy(parents refusal, technical reasons). After one week he returned with progressive worsening vomitings and an intracranial hypertension was suspected. CT documented an arachnoid cyst in the right middle cranial fossa and the patient is directed to the Neurosurgical Clinic where a cyst fenestration was done. Subsequent to operation the vomitings reinstaled with severe dehydration and an upper GI series showed a decompensated pyloric stenosis. He was operated on underwenting an antrectomy. Finally the child recovered with good short and long-term evolution. The coincidental presence of an intracranial congenital mass and a complicated aspirin-induced peptic ulcer in this young patient, misleaded us and in the lack of an early endoscopy an intempestive neurosurgical operation was initially done.

[X-linked Bruton congenital agammaglobulinemia. Case report]. / Agamaglobulinemie congenitala legata de x Bruton. Prezentare de caz.

Congenital agamaglobulinemia is a B-cell deficiency caused by tirosin-kinase gene mutations. This article presents the case of a 2 years and 6 months old boy with a history of multiple respiratory infections, which also associates a malabsorbtion syndrome. The patient presented for symptoms of arthritis. After performing protein electrophoresis, the extremely low gamma fraction pointed out a hidden immunodeficiency. Gene analysis was performed in the Department of Pediatric Immunology of Debrecen University, Hungary and thus the final diagnosis was determined. Arthritis and respiratory symptoms improved after immunoglobulin treatment.

[Photosensitivity and generalized idiopathic epilepsies of children]. / Fotosensibilitatea si epilepsiile generalizate idiopatice ale copilului.

UNLABELLED: The aim of this study is to evaluate the frequency of photosensitivity at patients of paediatric age diagnosed with different types of generalized idiophatic epilepsies. MATERIAL AND METHOD: We used a group of 68 children aged 3-18 years diagnosed: 39 patients with childhood absence epilepsy (CAE), 11 with juvenile absence epilepsy (JAE), 10 with epilepsy with grand mal on awakening (EGMA), 8 with juvenile myoclonic epilepsy (JME). EEG was recording without and with stimulation (hyperventilation, intermittent photic stimulation -IPS) after sleep deprivation. For all children with CAE absences and anormal EEG was provoked by hyperventilation and in 12 cases seizures was provoked by IPS. RESULTS: At 4 patients with JAE, EEG show photosensitivity. 5 children with JME have photosensitivity and for one child opening eyes provoked polyspike-wave discharges without clinical correspondent. The EEG of patients with EGMA showed photosensitivity at 6 children. The photosensitivity is frequently associated with generalized idiophatic epilepsies (32.35%) and it is most often seen at girls (54.54%) of puberty age.

[Attention-deficit/hyperactivity disorder. Clinical and treatment aspects]. / Tulburarea hiperactivitate/deficit de atentie. Aspecte clinice si terapeutice.

Attention-Deficit / Hyperactivity Disorder (ADHD) is a complex disorder. The onset of the disease is in childhood. The most important symptoms of ADHD are hyperactive, impulsive and inattention symptoms. In addition, children with this syndrome often have difficulties with other aspects of their learning, emotional modulation, organization, motivation, memory and other functions of the brain's management system. Drug medications, behavioral treatment and behavioral interventions at school are the most important aspects of treatment for ADHD syndrome.

[Digestive disease in the immunocompromised patient with acute lymphoblastic leukemia. Experience of the IVth Pediatric Clinic--Oncology Department of the Iasi Sfânta Maria Children's Hospital]. / Patologia digestiva la pacientul imunocompromis prin leucemie acuta limfoblastica in experienta clinicii a IV-a pediatrie--sectia himato-oncologie a spitalului de copii Sfânta Maria" Iasi.

UNLABELLED: The goals of this paper were to study the various types of digestive disease in acute lymphoblastic leukemia (ALL), to characterize the children in the study group by age, sex and environment, by presence of liver and spleen enlargement, levels of GOT, GPT, vomiting, to evaluate methotrexate (MTX) serum levels al 24 hours and 48 hours after administration, and to analyze the correlation between MTX levels and MTX liver and blood toxicity. MATERIAL AND METHOD: We studied 39 immunocompromised children hospitalized in the IV-th Pediatric Clinic-Oncology Ward, between 1983-2005, with acute lymphoblastic leukemia (ALL); most of them exhibited defects of humoral immunity such as transitory hypogammaglobulinemia, and defects of the cellular immunity that accompanied hepatomegaly, hepatic cytolysis and biliary obstruction. RESULTS: The diagnostic of ALL was sustained by: medullar biopsy, lumbar punction, cytochemical reactions, blood cell count, flow-cytometry, methotrexate level determination. Hepatic damage was measured by: GOT, GPT, bilirubin, LDH, GGT, FA, HBS antigen, anti HCV antibodies, anti HVA antibodies, anti toxoplasmosis antibodies, anti CMV antibodies, serum protein levels, TQ, inflammation markers. A slight increase in the number of diagnosed ALL cases during the past two decades was noticed and ALL incidence was higher in boys than girls. Hematologic toxicity of MTX is a real problem, causing neutropenia. MTX also caused oral lesions (in 69.36% of children), vomiting (in 69.2% of children) and liver toxicity (in 51.3% of children). MTX serum level 24 hours after administration is significantly different from the serum level at 48 hours after administration. Thus, the use of calcium folinate is proved to be very effective. We have also demonstrated that vomiting had some other causes besides MTX administration.

[Ataxia-telangiectasia syndrome. Clinical and diagnostic aspects]. / Sindromul ataxie-telangiectazie aspecte clinice si diagnostice.

Ataxia-telangiectasia (A-T) is a genetic disease, which mainly affects the skin and the central nervous system. Authors present 3 cases that presented for walking disorders starting at the age of 2 to 4 years old. All patients had a history of repeated broncho-pulmonary infections. In one case the syndrome was well defined, both clinically and immunologically, which made the diagnosis simple. In the other two cases, the initial diagnose was cerebral palsy the ataxic form and myasthenia. In all patients we observed low or no serum levels of IgA and IgE and high serum levels of a-fetoprotein. AT diagnose must be suspected in all children who exhibit the association of progressive cerebellar ataxia, which first appears in early childhood, with repeated bronchopulmonary infections and immune deficiencies, the later appearance of telangiectasia confirming the diagnosis.

[Rasmussen syndrome. Clinical study]. / Sindromul Rasmussen. Studiu clinic.

Rasmussen's syndrome is a specific progressive disease affecting primarily one hemisphere, accompanied by intractable seizures and leading to severe neurologic and mental deficit. The authors present one child with frequent partial motor seizures and myoclonic seizures refractory to antiepileptic drug therapy who was diagnosed with epilepsia partialis continua. After a period of 15 months the patient develops a spastic right hemiparesis and mental degradation. Association of this signs of the clinical picture lead to the diagnostic of Rasmussen's syndrome who was confirmed by the presence of localized EEG abnormalities and imaging studies.

[Neurocutaneous syndromes and epilepsy. Report of 23 cases and review of the literature]. / Sindroamele neurocutanate si epilepsia. Raportarea a 23 de cazuri si revederea literaturii.

The neurocutaneous syndromes are characterized by congenital dysplastic abnormalities involving the skin and nervous system. We included in the study 23 children diagnosed with different types of neurocutaneous syndrome (10 patients with tuberous sclerosis, 5 with Sturge-Weber syndrome and 8 with neurofibromatosis type 1 and epilepsy'. The factors studied included: age at onset of seizures, seizure types and frequency, seizure response of treatment, EEG, CT/MRI, neurologic, neuro-ophthalmologic and psychological examination. In 39.13% of cases the epilepsy onset preceded the clinical diagnosis of neurocutaneous disorders. The age of seizure onset ranged from 4 month to 5 years. The most common type of seizure appear to be complex partial. 21.73% of patients associated mental retardation. Therapeutical efficacy was variable depending on type of seizure and type of neurocutaneous syndromes.

[Epilepsy in different types of cerebral palsy]. / Epilepsia în diferite forme de paralizie cerebrala.

Epilepsy (E) is common in all types of cerebral palsy (CP) and in some varieties it is the rule. The aim of this study is to evaluate the nature, course and prognosis of seizures in patients with E and CP. Clinical observations were performed on a group of 109 patients, from 2.5 to 17 years of age, 2/3 associated mental retardation. The results proved that all types of seizures can be observed at the patients with CP and the epileptic attacks can be controlled in most cases with polytherapy.

[Chronic dacryocystitis fistulization in a Rubinstein-Taybi syndrome]. / Dacriocistita cronica fistulizata într-un caz de sindrom Rubinstein-Taybi.

The article presents the treatment difficulties of the chronic dacryocystitis at an eight month old patient, diagnosed with Rubinstein-Taybi syndrome. The difficulties occurred as a result of numerous antibiotic treatments and surgical procedures suffered by the patient since his second week of life.

[Achalasia in children: Diagnostic opportunities and therapeutical options]. / Acalazia cardiei la copil--posibilitati diagnostice si optiuni terapeutice.

Achalasia is a functional disorder of the distal esophagus, which fails to relax during swallowing. Although being rare, this disorder is the source of a significant morbidity, including developmental disorders in children. Several therapeutical options are available: myorelaxants administration, esophageal dilations and surgery--distal esophageal myomectomy followed by an antireflux procedure. The paper introduces our experience in treating a number of 5 children between 1991 and 1998.

[Hepatic hydatid cyst and splenic hydatid cyst--their surgical treatment with splenic preservation]. / Chist hidatic hepatic si chist hidatic splenic--tratament chirurgical cu pastrarea splinei.

The hydatid cyst located in the spleen is very rare and the association of the liver location concomitant with the spleen location is exceptional. If the elective surgical intervention for the hydatid cyst of the liver with abdominal evolution is the operation Lagrot, most of the authors preferred a splenectomy in the hydatic cyst of the spleen. It is presented the case of such an association, pointing out the role of the echography for diagnosis and the opportunity of deciding on the preservation of the spleen in treatment.

[Helicobacter heilmanii gastritis: the bacteriological, endoscopic and histopathological considerations in 18 cases of infection in children]. / Gastrita cu Helicobacter heilmanii: consideratii bacteriologice, endoscopice si histopatologice privind 18 cazuri de infectie la copil.

The investigations on the presence of Helicobacter pylori in gastric mucosa biopsy specimens have evidenced another morphologically distinct spiral bacterium, Helicobacter heilmanii, which is associated with histopathological lesions of antral gastritis. The authors found 18 cases of such infection in a number of 1508 children with dyspeptic manifestations examined endoscopically. While H. pylori-associated infection was detected in 48.7% of the cases, H. heilmanii-associated gastritis had a much lower prevalence, 1.1%. The diagnosis was made by microscopic examination of the gram fuchsin-stained smear from a biopsy fragment, the urease test being more commonly erratic or late positive. In 10 cases, the endoscopic examination of gastric mucosa revealed nodularity of antral mucosa, in one of these cases a ulcer lesion at the level of the great curvature being also associated. Histopathological changers of active chronic gastritis have been found but in 4 out of children, the examined fragments of gastric mucosa being collected in most cases from the fundic area: in the child with gastric ulceration the histopathological lesions were of antral acute gastritis. Clinical, bacteriological, endoscopic and histological cure of H. heilmanii gastritis is possible by therapy with bismuth compounds alone.