Clinical Manifestations In Children Research Articles

Primary Ciliary Dyskinesia in Children

научный биометрический журнал, который знакомит читателя с клиническими рекомендациями и особенностями применения лекарственных и вакцинных средств у детей, предоставляет исчерпывающую информацию о воздействии лекарственных средств на плод, о проводимых в

Progress of the classification and treatment of neutropenia in children

Neutrophils are the most abundant immune cells in the human, which can be caused by a variety of acquired or congenital factors.The clinical manifestation of children with neutropenia are ranging from different forms and parts, seriously affecting the quality of life.The novel sights in etiology, pathogenesis and treatment of children with acquired and congenital neutropenia are discussed. Key words: Neutropenia; Child; Classification; Treatment

The Clinical Characteristics and Magnetic Resonance Analysis of Children with Mild Encephalitis with Reversible Isolated Corpus Callosum

Objective: To summarize the Magnetic Resonance Imaging (MRI) performance and clinical characteristics of children mild encephalitis with reversible isolated corpus callosum pressure (MERS), and analyze the possible etiology. Methods: The clinical data and MRI findings of 11 children with MERS in children's hospital of Shanxi Province from January 2012 to January 2015 were summarized, and then their clinical manifestations and characteristics were analyzed. Results: Among the 11 patients, 5 were male and 6 were female. The age range was 2- 14 years. Clinical manifestations include fever (7 cases, 63.6%), gastrointestinal symptoms (9 cases, 81.8%), drowsiness (5 cases, 45.5%), convulsions (6 cases, 54.5%), dizziness (2 cases, 18.2%), cervical positive resistance (1 case, 9.1%), carotid resistance suspicious (3 patients [27.3%]). The appearance of cerebrospinal fluid, white blood cell count, leukocyte classification, protein, glucose and chloride are all normal. MRI showed only abnormal signal in the corpus callosum pressure: a slightly longer T1 signal, a longer T2 signal, a high Diffusion Weighted Imaging (DWI) signal, and a low signal of Apparent Diffusion Coefficient (ADC). Has 4 cases of nuclear magnetic head of abnormal signal in the middle of corpus callosum and ovoid, 6 cases, in the middle and a wide belt of corpus callosum, 1 case was located in the corpus callosum and diffuse wide strip. Eleven children were treated with anti-infective agents and other symptomatic treatment. All of the 11 children were recovered and discharged. The duration of the course is 8 d and the longest is 21 d. The results of MRI follow - up showed that the callosal pressure lesion disappeared. Conclusion: The clinical manifestations of children's MERS include fever, gastrointestinal symptoms, drowsiness and convulsions, and their characteristic head MRI is a reversible cytotoxic edema with good prognosis.

Diagnostic accuracy of current glossoptosis classification systems: A nested cohort cross-sectional study.

To assess the performance of endoscopic grading systems of glossoptosis in identifying severe clinical manifestations in children with Robin sequence (RS). Nested cohort cross-sectional study. All RS patients diagnosed at Hospital Clinics of Porto Alegre from October 2012 to June 2016 were enrolled in this cohort. Patients underwent sleep endoscopy and were classified according to Yellon (Y) and de Sousa et al. (S) scales. Symptom severity evaluation was performed as defined by Cole et al. The outcome of interest was Cole's clinical classification grade 3. Eighty patients were eligible for analysis. Sensitivity (Y: 56.2%, S: 28.1%, P < .001) and specificity (Y: 85.4%, S: 93.8%, P = .038) in identifying severe clinical symptoms patients (i.e., Cole grade 3) were statistically different between Y and S classifications. A low but significant overall correlation was observed for both Y (rho = 0.372, P < .001) and S (rho = 0.439, P < .001) classifications when compared with Cole classification. Diagnostic odds ratio (DOR) for Y (DOR: 7.53, 95% confidence interval [CI]: 4.15-10.90) and S (DOR: 5.87, 95% CI: 1.86-9.87) were equivalent (P = .92). Also, receiver operating characteristic curves area under the curve were not significantly different between them. The positive likelihood ratio was 3.86 (95% CI: 1.82-8.16) and 4.50 (95% CI: 1.32-15.36) for Y and S, respectively. Y and S grading systems showed a low sensitivity and moderate to high specificity in detecting patients with severe clinical manifestations. Correlation between Y/S and Cole et al. grading were also considered low. Development of a more discriminative anatomic grading system is still needed for this specific disorder. 2b. Laryngoscope, 128:502-508, 2018.

Age-Stratified T Cell Responses in Children Infected with Mycobacterium tuberculosis.

Tuberculosis (TB) in young children differs from adult TB in that the risk of rapid progression to active TB (aTB) is higher in children than in adults. The reasons for this increased risk are not fully understood. Early differentiation remains difficult between children at risk to develop aTB from those who will remain healthy and develop a latent TB infection (LTBI). Biomarkers to differentiate aTB from LTBI in children, especially in very young children, are urgently needed. To identify M. tuberculosis-specific functional T cell subsets related to clinical manifestations in children, we enrolled 87 children exposed to M. tuberculosis. After standard clinical assessment, the children were classified as aTB, LTBI, or uninfected. Their CD4+ T cell cytokine profiles (IFN-γ, TNF-α, IL-2, IL-17) were analyzed at the single-cell level by flow cytometry after stimulation with three mycobacterial antigens, purified protein derivative (PPD), early-secreted-antigenic target-6 (ESAT-6), or heparin-binding hemagglutinin (HBHA). This approach identified age-related discriminative markers between aTB and LTBI. Whereas among the 3- to 15-year-old children, an excellent discrimination between aTB and LTBI was provided by comparing the ratio between the proportions of ESAT-6-induced IFN-γsingle+ and ESAT-6-induced TNF-αsingle+CD4+ T lymphocytes, this was not the case for children younger than 3 years. By contrast, in this group (<3years), the analysis of HBHA-induced IL-17single+CD4+ T lymphocytes allowed us to identify children with LTBI by the high proportion of this cellular lymphocyte subset, whereas this was not the case for children with aTB. The analysis at the single-cell level of T cell immune responses induced by mycobacterial antigens are, thus, different in infected children younger or older than 3 years of age. HBHA-induced IL-17 production by CD4+ T lymphocytes was associated with protection only in children under 3 years who are at high risk for rapid progression to aTB. This suggests that the HBHA-induced IL-17 production by CD4+ T lymphocytes is a potential new correlate of protection against M. tuberculosis in humans, and that the distinction between children with LTBI and those with aTB is possible based on age-related diagnostic markers.

Clinical characteristics of Meckel diverticulum in children: A retrospective review of a 15-year single-center experience.

Meckel diverticulum is the most prevalent congenital abnormality of the gastrointestinal tract in children. The aim of this study was to review and analyze clinical data on the diagnosis and management of Meckel diverticulum in pediatric patients. The records of 102 pediatric patients (<14 years old) who underwent surgery for Meckel diverticulum at our institute between 2001 and 2015 were reviewed. Clinical, imaging, laboratory, surgical, and pathological data were recorded. The series comprised 65 males and 37 females with a median age of 5.6 years. Lower gastrointestinal bleeding was the most frequently identified clinical manifestation of Meckel diverticulum, and this manifestation was observed in 41 patients. Intussusception secondary to Meckel diverticulum was identified in 32 patients. Twelve patients presented clinical features of peritonitis; of these patients, 8 had perforated Meckel diverticulum and 4 had Meckel diverticulitis. In 10 patients, Meckel diverticulum was incidentally diagnosed during other surgeries, including appendectomy and neonatal enterostomy. Seven patients were diagnosed with intestinal obstruction. Technetium-99m pertechnetate imaging offered high diagnostic yield. Open surgery was performed on 59 patients, while a laparoscopic approach was employed in 35 patients. The remaining 8 patients did not undergo resection of the Meckel diverticulum. Histology revealed ectopic gastric mucosa in 42 patients (44.7%), ectopic pancreatic tissue in 35 patients (37.2%), mucosa of the small intestine in 15 patients (16.0%), and both gastric and pancreatic ectopic tissue in 2 patients (2.1%). All patients recovered uneventfully except 2 patients in whom an intestinal adhesion obstruction was identified after discharge. Meckel diverticulum had various clinical manifestations in children. Technetium-99m pertechnetate imaging may be useful for diagnosing Meckel diverticulum. Surgical excision of the Meckel diverticulum may be safe and effective in symptomatic patients, and relatively better outcomes can be achieved using this approach.

Clinical and imageology follow-up study of acute disseminated encephalomyelitis

Objective To explore the clinical manifestations and recovery from neuroimaging abnormalities of acute disseminated encephalomyelitis (ADEM), in order to improve the understanding of ADEM. Methods A total of 47 children with ADEM in Department of Neurology, Children′s Hospital of Soochow University were followed up for 1 year.The clinical manifestations, auxiliary examination (especially neuroimaging), treatment and prognosis were analyzed.The recovery conditions from clinical and neuroimaging abnormalities were summarized. Results Among 47 cases, 37 cases (78.72%) had prodromic infection history, and 5 cases (10.64%) had the history of vaccination.The clinical manifestations were varied, including 36 cases (76.60%) with somatic discomfort, 30 cases (63.83%) with fever, 30 cases (63.83%) with disturbance of consciousness, 28 cases (59.57%) with epileptic seizure, 24 cases (51.06%) with headache and dizziness, 22 cases (46.81%) with nausea and vomiting, 15 cases (31.91%) with dyskinesia, 15 cases (31.91%) with cranial nerve injury, 9 cases (19.14%) with incoordination, and 8 cases (17.02%) with mental and behavior disorders.Abnormal lesions presented multiple, asymmetric patchy and large patchy signal image showed by brain magnetic resonance imaging (MRI), and 7 cases (14.89%) involved the spinal cord.All patients received the treatment of Methylprednisolone [15-20 mg/(kg·d)] combined with intravenous gamma globulin (total 2 g/kg, 3 to 5 days). The dose of Methylprednisolone gradually decreased after it had been used for 3 days.In the end, it was replaced by an oral administration of Prednisone [1.0-2.0 mg/(kg·d)], and the total treatment period was for 2-3 months.The follow-ups of 47 children with ADEM showed that the MRI lesions of all the children were reduced and the MRI lesions in 4 (8.51%) of them disappeared completely after 0.5 month of follow-up; the lesions disappeared completely in 13 cases (27.66%) after 1 month of follow-up; the lesions disappeared completely in 23 cases (48.94%) after 3 months of follow-up; the lesions disappeared completely in 32 cases (68.09%) after 6 months of follow-up; the lesions disappeared completely in 43 cases (91.49%) after 1 year of follow-up; and finally there were 4 cases (8.51%) with residual lesions. Conclusion The clinical manifestations of children with ADEM are varied.Most children with ADEM have a favorable prognosis.Imaging lesions of 68.09% of the cases completely disappeared after the onset of 6 months.Imaging lesions in 91.49% of the cases completely disa-ppeared at the onset of 1 year.Only a small fraction of patients present permanent lesions. Key words: Acute disseminated encephalomyelitis; Clinical manifestation; Neuroimaging; Magnetic resonance imaging; Child

Postviral autoimmune encephalitis: manifestations in children and adults.

Autoimmune encephalitis is an increasingly recognized neuropsychiatric condition seen in patients of all ages. Herpes virus infections of the brain can antedate the development of pathogenic autoantibodies against N-methyl-D-aspartate receptors and further neuronal surface proteins, leading to neuronal dysfunction and relapsing symptoms. This review will recapitulate the recent scientific progress, clinical manifestations in children and adults, therapeutic options, and etiological concepts on how autoimmunity develops. Postviral autoimmune encephalitis has been established as disease mechanism after herpes simplex virus encephalitis. Relapsing symptoms in the absence of virus occur in up to 20% of cases. The clinical presentation is relatively stereotyped in children, consisting of choreoathetosis and reduced levels of consciousness. Adults commonly present with psychiatric abnormalities and cognitive changes. Virus-induced antibody generation seems to be a widespread mechanism not confined to Herpesviridae and N-methyl-D-aspartate receptor antibodies SUMMARY: The presence of prolonged, atypical, or relapsing symptoms after virus encephalitis must be actively determined, requiring early follow-up visits of patients, and should always prompt the search for underlying autoantibodies. Relapsing symptoms can markedly improve with immunotherapy, thus treatment, including corticosteroids, plasma separation, immunoglobulins, and rituximab is usually recommended, even if no specific antibody was detected.

Extended Clinical Spectrum of Anti–N-Methyl-d-Aspartate Receptor Encephalitis in Children: A Case Series

ObjectiveThere is a wide spectrum of clinical manifestations in children with anti–N-methyl-d-aspartate (NMDA) receptor antibody encephalitis from two different health care settings. MethodsWe describe our experience with 13 patients (median age, 7 years; range, 5 months to 19 years) presenting to tertiary referral centers in India and the United States. ResultsInitial manifestations were neurological (seizures or movement disorders) in eight patients, and psychiatric (e.g., emotional lability and hallucination) in five patients. Symptoms during the clinical course included seizures in ten patients, movement disorders (dyskinesia and choreiform movements) in 11 patients, and behavioral changes (aggressiveness and insomnia) in ten patients. Concomitant infections (herpes simplex virus 1, tuberculous meningitis, and influenza A) were present in three patients. Analysis of the cerebrospinal fluid in all except two cases preceded by infection (herpes simplex virus encephalitis and tuberculous meningitis) was unremarkable. Treatment included intravenous immunoglobulin/methylprednisolone (11 patients), rituximab (eight patients), plasmapheresis (two patients), and cyclophosphamide (two patients). Six patients recovered completely. Two patients had mild residual neurological deficits, whereas four had severe residual neurological deficits. Two patients had profound autonomic instability, which was the cause of death for one of them. Two patients relapsed at two and six months after the initial recovery. ConclusionsWe describe the differences and similarities of clinical presentation, test results, and response to treatment of children with anti–N-methyl-d-aspartate receptor encephalitis from India and the United States. Included is a description of one of the youngest patients with anti–N-methyl-d-aspartate receptor encephalitis (five months) and the first patient to be reported in association with tuberculous meningitis.

Clinical analysis of 108 cases with chronic kidney disease at stage 2 to 5 in children

Objective To analyze the pathogenesis, initially diagnosed symptoms and clinical manifestations of children with chronic kidney disease(CKD) at stage 2 to 5. Methods The data of 108 children who were hospitalized in Children′s Hospital Affiliated to Capital Institute of Pediatrics from September 2007 to April 2016 with CKD stage 2 to 5 were retrospectively analyzed.The etiologies, clinical manifestations and examinations were summarized, and the clinical manifestations were compared between the congenital hereditary urinary diseases group and the acquired urinary diseases group. Results (1) In the 108 cases collected, 66 cases were male, 42 cases were female, aged from 3 months to 15 years and 1 month old.Twenty-four cases were diagnosed at stage 2, 26 cases at stage 3, 35 cases at stage 4, and 23 cases at stage 5.(2)Twenty-eight kinds of illness were involved in the cause of CKD.Among them, 57 cases(52.8%) had congenital anomalies of the kidney and urinary tract, 5 cases(4.6%) had hereditary kidney diseases, 41 cases(38.0%) had other primary or secondary kidney diseases, and in 5 cases(4.6%) the causes of disease were unknown.(3) For the initially diagnosed symptoms, 29 cases(26.9%) were due to complaints associated with kidney disease, 36 cases(33.3%) were of other outside kidney symptoms, and 43 cases(39.8%) were of negative symptoms.The results of urinary ultrasound were abnormal in 79 cases(73.1%) and 87 cases(80.6%) showed abnormality in urinary analysis.There were 105 cases (97.2%) with abnormal manifestations either in urinary tract ultrasound or in urinary analysis.(4)The ages on diagnosis as CKD in children with congenital hereditary urinary diseases(5.89 years old) were younger than that of children with acquired urinary diseases(9.20 years old), and the difference was significant(Z=-3.434, P=0.001). The frequency of cases with short stature or lower-weight in group of congenital hereditary urinary diseases[66.1%(41/62 cases), 64.5%(40/62 cases)] were significantly higher than those of the acquired urinary diseases group[43.9%(18/41cases), 43.9%(18/41 cases)], and the differences were statistically significant(χ2=4.983, 4.263, P=0.026, 0.039). Conclusions The causes of CKD are complicated, and the congenital anomalies of kidney and urinary tract are the major causes of CKD at stage 2 to 5 in the cases.The initially diagnosed symptoms of CKD are insidious and atypical.The children with congenital hereditary urinary diseases tend to have more serious growth retardation.Urinary analysis and ultrasound may have an important significance for early diagnosis of CKD in children. Key words: Chronic kidney disease; Child; Etiology; Clinical manifestation

Results of molecular genetic testing in Russian patients with Pendred syndrome and allelic disorders

Pendred syndrome is an autosomal recessive inherited disorder characterized by a combination of sensorineural hearing impairment and euthyroid goiter; its clinical manifestation in children is hardly distinguishable from nonsyndromic hearing loss. Pendred syndrome is one of the most frequent types of syndromic hearing loss. Hearing impairment is accompanied by abnormal development of the bony labyrinth—enlarged vestibular aqueduct (EVA) and occasionally combined with Mondini dysplasia. Mutations in the SLC26A4 gene, which encodes the pendrin protein, are responsible for both Pendred syndrome and for allelic disorder (nonsyndromic enlarged vestibular aqueduct). The present study for the first time conducted molecular genetic analysis in 20 Russian patients with Pendred syndrome, EVA and/or Mondini dysplasia. As a result, six pathogenic mutations in the SLC26A4 gene were revealed in four patients. The mutation c.222G>T (p.Trp74Cys) was detected for the first time. Mutations were found in patients with Pendred syndrome and nonsyndromic EVA with or without Mondini dysplasia. Mutations were not detected in patients with isolated Mondini dysplasia. One proband with clinical diagnosis Pendred syndrome was homozygous for the c.35delG mutation in the GJB2 gene. The absence of frequent mutations, including well-known ones or “hot” exons in the SLC26A4 gene, was reported. Therefore, the optimal method to search for mutations in the SLC26A4 gene in Russian patients is Sanger sequencing of all exons and exon-intron boundaries in the SLC26A4 gene.

Children with Mild CAG Repeat Expansion in HTT Gene Showing Psychiatric but not Neurological Presentation: Is It One More Shade of Huntington Disease?

Objective: Huntington disease (HD) generally manifests in adulthood. Large mutations with CAG repeat expansion in HTT gene may rarely cause juvenile Huntington disease (JHD) in early childhood or adolescence with atypical clinical features, i.e., atypical parkinsonism, if compared to adult patients. Our objective is to characterize the rare occurrence of clinical manifestations in children carrying mutations in the low-mild size, generally causing adult HD with typical choreic movements. Methods: We are following up a subgroup of young subjects with HD mutation who manifested with disabling psychiatric condition since early childhood or adolescence. We are collecting data by the observational studies Registry and ENROLL-HD since 2004. Among 60 JHD patients we are currently following-up, we selected people who carry a mutation in the mild range of CAG expansions (i.e., expected to manifest in adulthood), psychiatric manifestations and no neurological signs or movement disorders suggestive of HD. All patients were genetically (i.e., CAG size analysis) and clinically (i.e., total motor score within the Unified HD Rating Scale) characterized. Results: We found four subjects who showed the characteristics for this analysis. All four subjects presented a CAG expansion size <45 repeats. Two patients manifested a schizophrenia-like disturbance during their adolescence, with the later appearance of motor signs after age 20. In the other two cases, patients presented symptoms of autistic spectrum disorder, since infancy. One of them showed also a schizophrenia-like disturbance and, later, HD onset with motor signs after 20. A 45 year old patient is currently manifesting an autistic disorder in absence of others neurological signs. Conclusion: The description of JHD includes sometimes children with psychiatric manifestations associated with adult motor onset. We advise to pay careful attention to such rare conditions that might represent either psychiatric conditions erroneously classified as JHD or prodromic adult HD cases.

Electroencephalogram and clinical manifestations of Rett syndrome in children

Background Rett Syndrome (RS) is a severe neurodevelopmentaldisorder. Epileptic seizures occur in 80-90%; grandmal, psychomo-tor (complex partial), and focal motor seizures have been reported.The electroencephalogram(EEG) is almost always abnormal.Objective This study aimed to investigate the EEG and clinicalmanifestations of children with RSResults We investigated EEG on 5 patients with RS aged 30–66month. One patient was in clinical stage II and 4 patients in clini-cal stage III. Four patients had history of seizures, however onlytwo patients suffered from epilepsy. The EEG demonstrated slow-ing background activity in occipital region in two patients. In addi-tion, epileptic form activities were observed in 4 of 5 patients.Conclusion We concluded that epileptic spike discharge with orwithout clinical seizures were found in almost all of our RS pa-tients. These paroxysmal discharges suggested the process andthe sequences of cortical involvement. Compelling clinical, neuro-physiological evidences were very important to decide the stageof Rett disorder

Clinical manifestations in children and adolescents with corpus callosum abnormalities.

Corpus callosum abnormality (CCA) outcomes are quite unpredictable and variable, from asymptomatic forms to mild or severe neurodevelopment disorders. The aim of this study was to examine clinical outcomes in CCA patients. The study included 61 children and adolescents in whom brain magnetic resonance imaging (MRI) scans showed CCA, isolated or associated to other central nervous system lesions. All patients underwent anamnesis, physical and neurological examination, routine laboratory tests, electroencephalogram (EEG), and MRI scans. In all participants, the intelligence quotient (IQ) was determined. We divided the participants into two subgroups: the first subgroup included patients with an isolated CCA, and the second subgroup included patients with CCA associated with extra-callosal brain lesions (complex CCA). We found that CCA were associated with elevated frequency to intellectual disability (ID), other neurodevelopment disorders, epilepsy, and isolated EEG anomalies. Mild ID (p=0.003) was more frequent in the isolated subgroup, while epilepsy (p=0.036) and pre-perinatal risk factors (p=0.023) were more frequent in the complex CCA subgroup. Although the role of the CC in the interhemispheric communication is known, neurological and neurodevelopment outcomes of CCA are extremely variable and unpredictable. The presence of extra-callosal brain anomalies is one of the major prognostic factor, and probably, they have an important impact on the clinical outcome.

Clinical characteristics and etiology of children with hypereosinophilia

To analyze the clinical characteristics and etiology of hypereosinophilia in children. Clinical data of 88 children with hypereosinophilia admitted in Children's Hospital of Zhejiang University School of Medicine during April 2009 and May 2015 were retrospectively reviewed. The clinical manifestations, etiologies, and the correlation of disease severity with different etiologies were analyzed. The main clinical manifestations were fever, abdominal pain, cough and/or tachypnea, skin rash, hemafecia and diarrhea, which were observed in 19 (21.6%), 15 (17.0%), 14 (15.9%), 13 (14.8%), 11 (12.5%) and 6 (6.8%) cases, respectively. For etiologies, there were 28 cases (31.8%) induced by infections, including 11 cases of acute bronchial pneumonia, 9 cases of parasite infection, 4 cases of septicemia, 2 cases of urinary system infection, 1 case of cellulitis and 1 case of cholecystitis complicated with pancreatitis. The etiologies for the rest cases were allergic diseases (25 cases, 28.4%), eosinophilic gastroenteritis (20 cases, 22.7%), immunodeficiency (3 cases, 3.4%, all were moderate to severe eosinophilia), ABO hemolysis (2 cases, 2.3%), hematologic neoplasms (2 cases, 2.3%), eosinophilic cystitis (1 case, 1.1%), eosinophilic granulomatosis with polyangitis (1 case, 1.1%), nephrotic syndrome (1 case, 1.1%), and 5 cases (5.7%) were of unknown causes. Fever, abdominal pain, cough and/or tachypnea are the most common clinical manifestations in children with hypereosinophilia. Infection, allergic diseases and eosinophilic gastroenteritis are the most common etiologies, and parasites are the most common pathogen identified. Differential diagnosis of primary immunodeficiency should be considered in children with moderate to severe eosinophilia.

The spectrum of autosomal dominant polycystic kidney disease in children and adolescents.

Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary renal disorder. It is characterized by the development of renal cysts and kidney enlargement and ultimately leads to renal failure typically in the sixth decade of life. Although most patients are asymptomatic until well into adulthood, renal cysts develop much earlier, often in utero. Significant renal anatomic and cystic expansion typically occurs before clinical manifestations in children and young adults with AKPKD. The cyst burden detected by imaging represents the minority of cyst burden, and renal and cardiovascular abnormalities are the most common manifestations in children with ADPKD. Here we review the molecular pathogenesis of ADPKD, discuss the screening, diagnosis and clinical manifestations of this renal disorder in childhood and adolescents and review treatment options and potential therapies currently being tested.

ЧИ ВІДПОВІДАЄ КЛІНІЧНА РЕМІСІЯ РЕЦИДИВНОГО ОБСТРУКТИВНОГО БРОНХІТУ ВІДСУТНОСТІ ІМУНОЛОГІЧНИХ ЗМІН У РЕСПІРАТОРНОМУ ТРАКТІ ДІТЕЙ?

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The clinical manifestations of children with endocapillary proliferative glomerulonephritis with isolated C3 deposition

Objective To study the patients' clinical characteristics and prognosis when only C3 deposition exists in endocapillary proliferative glomerulonephritis and try to understand deeply the role of C3 in kidney damage deeply. Methods The patients who were diagnosed with endocapillary proliferative glomerulonephritis but only had C3 deposited in immunofluorescence (to avoid false positive, C3≥2+ was included) were selected from Beijing Children's Hospital Affiliated to Capital Medical University during November 2010 to October 2014.Their clinical manifestations, laboratory examinations, treatments, prognosis, and pathological changes were analyzed, and literature review was performed.Their clinical characteristics and prognosis were summarized. Results There were 11 patients diagnosed with endocapillary proliferative glomerulonephritis which had only C3 deposition (≥2+ ). Nine of them had onset with acute nephritis syndrome (81.8%), and 2 cases presented recurrent paroxysmal gross hematuria(18.2%). Seven cases were diagnosed with acute post streptococcal glomerulonephritis (63.6%). Eleven cases' clinical manifestations were relatively severe, and the complement C3 was significantly lower than the normal (100.0%). Their light microscope showed capillary proli-ferative glomerulonephritis, and the electron microscope showed the immune complexes were deposited in the endothelium, the epithelium or the mesangial area.The patients received corresponding treatment respectively, and all the patients had good prognosis during following up of 7 months up to 39 months. Conclusions Streptococcus infection is a common cause in endocapillary proliferative glomerulonephritis with only C3 deposition.The clinical manifestations of some children are similar to post streptococcal glomerulonephritis but relatively severe.Only deposition of C3 without IgG may be involved in another complement activation mechanism. Key words: Complement 3; Endocapillary proliferative glomerulonephritis; Streptococcus infection

Two Familial Cases of Robertsonian Transloacations 13; 14 and Its Clinical Consequences

Two familial cases of Robertsonian translocation 13;14 [rob t(13;14)] and its clinical manifestation in children are discussed in present report. In case-1, father and child both were having [rob t(13;14)] with karyotype [45,XYrob(13;14)(q10;q10)] and [46,XY,rob(13;14)(q10;q10),+21] respectively. The child was presented with clinical characteristics of Down syndrome (DS) due to trisomy 21. The child and father both were having soft sub-mucous cleft palate. In case-2, child and mother both were having rob t(13;14) with karyotype [45,XX,rob(13;14)(q10;q10)]. Mother was phenotypically normal but both of her children were presented with gross developmental delay for all the four areas, i.e., gross motor, adaptive, language and personal social behavior. Interestingly all the carriers of [rob t(13;14)] showed abnormal clinical features: like soft sub-mucous cleft palate with DS in case-1 child, soft sub-mucous cleft palate and Inter Chromosomal Effect (ICE) in case-1 father, miscarriage and birth of children with congenital problem in case-2 mother and gross developmental delay in case-2 child. It was assumed that the co-occurrence of [rob t(13;14)] and trisomy 21 in case-1 child was due to phenomenon of ICE in father carrying [45,XY,rob(13;14)(q10;q10)].

A clinical study of children with intussusception

Background: In early childhood the commonest cause of bowel obstruction is intussusception and is one of the common abdominal emergencies in children younger than 2 yr. Early diagnosis helps to reduce mortality and morbidity. The objective of the study was to find out the pattern of clinical manifestations in children with intussusception and the outcome. Methods: A hospital based descriptive study using purposive sampling technique. 52 children below 12 years of age with a radiological diagnosis of intussusception attended the Emergency, outpatient and inpatient departments of Jubilee Mission Hospital, Thrissur Kerala (Tertiary care teaching hospital) were evaluated, during the study period from January 2012 to October 2012. Results: Outcome of the problem was correlated with age, sex, symptoms, risk factors sonological findings treatment protocol and duration of hospital stay. Total 52 children of intussusception were managed during the 10 month period (2012) male to female ratio was 2.05 :1. The common symptoms were vomiting (76.9%) excessive cry (65.38%) blood in stool (53.8%) abdominal pain (34.6%) and irritability (25%). Ileocolic intussusception (88.4%) was the commonest type. Conclusions: 42 children (80.76%) of the present study underwent hydrostatic reduction successfully. 4 children needed surgery. self-reduction occurred in 6 children (11.53%). The median hospital stay was 2 days and 39 children (75%) went home within 48 hrs. 9 children (17.3%) of the study population developed complications. 4 (7.69%) had shock. 2 each (3.84%) had perforation, peritonitis and septicaemia.