scholarly journals The Pattern of Thalassemia in Children at the Department of Child Health, School of Medicine University of North Sumatera/ Dr. Pirngadi Hospital, Medan

2019 ◽  
Vol 31 (9-10) ◽  
pp. 261-7
Author(s):  
Sugiani Sinulingga ◽  
Ismet Fadil Loebis ◽  
Adi Sutjipto
Keyword(s):  
Child Health ◽  
Ethnic Group ◽  
Clinical Symptoms ◽  
Thalassemia Major ◽  
Age Group ◽  
Pediatric Hematology ◽  
School Of Medicine ◽  
Hb E ◽  
First Admission

A study of the characteristics of childhood thalassemia was conducted at the Sub Department of Pediatric Hematology, Dr. Pirngadi Hospital from June 1979 to May 1989. There were 131 cases, consisting of75 (57.25%) boys and 56 (42.75%) girls with an average of 12 admission every year. The predominant age group was 0-2 years, and the youngest was 3 months old; Javanese ethnic group appeared predominant in 36 (63.15%) cases. Clinical symptoms of anemia were found in 112 (85.49%}, hepatomegaly in 91 (69.46%}, hepatosplenomegaly in 84 (64.12%}, without enlargement of organ in 17 (12.97%), and icterus in 6 (4.58%). Hb-Electrophoresis was done in 42 cases, revealing 26 (61.90%) with thalassemia major, 15 (35.71%) Hb E thalassemia, and 1 (2.20%) Hb H thalassemia. Hb value at the first admission in 65 (49.62%) was less than 5 g/dl, in 63 (48.09o/o) it was 5-10 g/dl and in 3 (2.29%) more than 10 g/dl.

scholarly journals Acute Non Lymphoblastic Leukemia in the Department of Child Health School of Medicine, University of North Sumatera/ Dr. Pirngadi Hospital Medan (1983-1988): A Preliminary Study

2019 ◽  
Vol 31 (9-10) ◽  
pp. 268-72 ◽  
Author(s):  
Feraluna Nasution ◽  
Zairul Arifin ◽  
Adi Sutjipto
Keyword(s):  
Retrospective Study ◽  
Child Health ◽  
Lymphoblastic Leukemia ◽  
Supportive Therapy ◽  
Age Group ◽  
Cytostatic Treatment ◽  
Pediatric Hematology ◽  
School Of Medicine ◽  
Fab Classification ◽  
Preliminary Study

A retrospective study on Acute Non Lymphoblastic Leukemia (ANLL) was conducted at the Sub Division of Pediatric Hematology, School of Medicine, University of North Sumatera/Dr. Pirngadi Hospital Medan, in a period of 5 years (1983-1988). There were 18 cases consisted of 14 (77. 78%) males and 4 (22 .22%) females with the age group of 0-2 years: 6 (30%), 2-8 years: 9 (50%), 8-15 years: 3 (30%). By the FAB classification, they were of FAB M-1: 1 (5.55%), FAB M-2: 1 (5.55%), FAB M-3: 1 (5.55%), FAB M-4: 2 (11.12%) and FAB M-6: 13 (72.23%). Only 7 (38.88%) were treated with cytostatics while the others received only supportive therapy. The result of  cytostatic treatment was unsatisfactory: 4 (57.14%) died within the first 2 months of treatment, 3 (42.86%) discontinued their cytostatics treatment.

scholarly journals Some Problems of Thalassemia in Jakarta

2017 ◽  
Vol 18 (3-4) ◽  
pp. 100
Author(s):  
Iskandar Wahidiyat ◽  
A.H. Markum ◽  
M. Abdulsalam ◽  
S. Muslichan
Keyword(s):  
Child Health ◽  
Blood Transfusion ◽  
Medical School ◽  
Thalassemia Major ◽  
Social Aspects ◽  
Hb E ◽  
Hb S ◽  
Hb H Disease ◽  
Points Of View

Within 10 years (1964 - 1974) two hundred and twenty one thalassemic children were observed in the Department of Child Health, Medical School, University of Indonesia in Jakarta. They consisted of 119 cases of thalassemia major, 95 thalassemia Hb E disease, 6 Hb H disease and 1 thalassemia Hb S disease. The main treatment of thalassemia major is still blood transfusion. Splenectomy was performed on 29 children with thalassemia major and 26 cases with thalassemia Hb E disease. Splenectomy performed on cases before hypersplenism appeared, showed better results then those late cases who have already symptoms of hypersplenism. Beside the medical points of view thalassemia has also many social aspects to be considered, those of the child itself, the parents and the society or community.

Genetic Analysis of Candidate Modifier Polymorphisms in β-Thalassemia/Hb E Patients.

Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 3781-3781
Author(s):  
Suthat Fucharoen ◽  
Pranee Winichagoon ◽  
Orapan Sripichai ◽  
Thongperm Munkongdee ◽  
Chutima Kumkhaek ◽  
...  
Keyword(s):  
Genetic Factors ◽  
Clinical Symptoms ◽  
Globin Gene ◽  
Thalassemia Major ◽  
Globin Genes ◽  
Nucleotide Polymorphisms ◽  
Relative Contribution ◽  
Modifying Factors ◽  
Hb E ◽  
Genes Encoding

Abstract β-Thalassemia/Hb E patients encompass a number of clinical severities, ranging from nearly asymptomatic to transfusion-dependent thalassemia major. This has been well documented, but the causes of the variability and the molecular basis of the interaction remain unexplained. In general any factor capable of reducing the degree of alpha-globin-beta-globin chain imbalance will result in a milder form of thalassemia. The major modifying factors demonstrated in the mild cases are coinheritance of mild β+-thalassemia and Hb E genes, coinheritance of α-thalassemia and increased production of Hb F. However, the fact that many patients who have seemingly identical genotypes, β0-thalassemia/Hb E, and do not have a detectable α-thalassemia or increased Hb F production still have mild clinical symptoms, while other patients have a very severe clinical condition similar to homozygous β0-thalassemia. This variation suggests that other unknown modifying genetic factors may contribute to severity of the disease. To assess the relative contribution of genetic factors in the variation of severity among β-thalassemia/Hb E patients, we conducted a prospective study searching for modifying factors in almost 1100 Thai/Chinese β0-thalassemia/Hb E patients from Thailand using an automated, chip-based platform based on mass spectrometry (Sequenom’s MassARRAYTM system). A map of ~80 single nucleotide polymorphisms (SNPs) has been constructed spanning more than 80 kb, including the locus control region (LCR) and all beta-like globin genes. These SNPs were identified through resequencing and from the public domain, including well-characterized restriction fragment length polymorphisms (RFLPs) used in prior haplotype studies. Included in this panel are assays for polymorphic sites reported to influence globin gene expression, specifically Gγ-Xmn I polymorphism and BP-1 binding site upstream of β-globin gene. Genotyping of other candidate modifier loci, including SNPs in genes encoding alpha hemoglobin stabilizing protein (AHSP), β-globin gene repressor BP-1 protein, erythropoietin (Epo), and transcription factors; GATA-1, EKLF, NF-E2, has been studied. To identify additional modifier loci, carefully selected patient sub-groups representing the extremes in disease severity either mild or severe have been selected for DNA pool construction to be used in a genomewide screen involving up to 100,000 validated gene-based SNPs. It is expected that this genomewide screen will yield important information on the role of candidate genes and may uncover the association of novel polymorphisms with severity heterogeneity in β-thalassemia/Hb E disease.

scholarly journals Hydrocephallus in the Department of Child Health, School of Medicine University of North Sumatera Dr. Pirngadi Hospital, Medan

2018 ◽  
Vol 33 (1-2) ◽  
pp. 32-6
Author(s):  
Yahya G. Lubis ◽  
T. Razif ◽  
Manihar D. Marbun ◽  
Bistok Saing
Keyword(s):  
Retrospective Study ◽  
Child Health ◽  
Conservative Treatment ◽  
Age Groups ◽  
Age Group ◽  
School Of Medicine ◽  
Almost All

A retrospective study 10 find out the incidence of hydrocephalus in the Paediatric Neurology Subdivision Department of Child Health, School of Medicine, University of North Sumatera/Dr. Pirngadi Hospital Medan, was carried out in the period of 1986- 1989. The number, age groups, causes and treatment were reviewed. The cases consisted of 45 children; 28 males (68.2%) and 17 females (27.8%). Most of the patients (34 = 75.5%) were found in the age group of 1 year or younger and the rest (11 = 24.5%) were tn the age group of more than I year. The youngest was 2 days old and the oldest was 5 years and 6 months old; 33 cases were congenital, 5 cases were acquired and 7 cases were unknown causes. Conservative treatment were introduced to almost all cases, and four cases had ventricular peritoneal shunt.

scholarly journals A Study of Aplastic Anemia at the Department of Child Health, School of Medicine University of North Sumatera/Dr. Pirngadi Hospital, Medan

2019 ◽  
Vol 31 (9-10) ◽  
pp. 253-60
Author(s):  
Ajuar Arif ◽  
Adi Sutjipto
Keyword(s):  
Child Health ◽  
Aplastic Anemia ◽  
Hemoglobin Concentration ◽  
Blood Transfusions ◽  
Age Group ◽  
School Of Medicine

A study of aplastic anemia in children in a period of June 1980 to June 1989 was done to evaluate the pattern of aplastic anemia in children. The cases consisted of 55 children, 30 males (54.55%) and 25 females (45.45%). Most of the patients (47.27%) were found in the age group of 10 to 15 years. The complaints were paleness (90.91%), fever (56.45%) and bleeding (52. 71%). The hemoglobin concentration was 4.25 ± 1.17 g/dl (mean ± SD), and the thrombocytopenia was generally severe. Prednisone or combination of prednisone and oxymethalone was given in addition to blood transfusions, antibiotics and vitamins. The outcome was difficult to evaluate because of the irregularity of treatments. Of the 55 children, 9 (18.02%) contracted leukemia after 1 - 5 months.

scholarly journals Bronchopneumonia with measles in Infants and Children at the Department of Child Health School of Medicine, University of North Sumatera/ Dr. Pirngadi Hospital Medan (January 1985 - December 1989)

2019 ◽  
Vol 31 (9-10) ◽  
pp. 273-80 ◽  
Author(s):  
Charles Hutasoit ◽  
Mardiana K. Dj. ◽  
Ridwan M. Daulay ◽  
Helmi M. Lubis ◽  
Zakaria Siregar
Keyword(s):  
Retrospective Study ◽  
Mortality Rate ◽  
Child Health ◽  
Nutritional Status ◽  
Infants And Children ◽  
Morbidity And Mortality ◽  
Age Group ◽  
School Of Medicine ◽  
In Infants And Children

A retrospective study was done on patients with bronchopneumonia hospitalized at the Dr. Pirngadi Hospital Medan/School of Medicine, University of North Sumatera; during January 1985 through December 1989. The purpose of this study is to assess the morbidity and mortality of bronchopneumonia with measles and the nutritional status of the patients. Out of the 14.082 patients admitted, 1310 children suffered from bronchopneumonia (10.7%). Ninety nine of them were also with measles (7.6%). Most of the patients with bronchopneumonia without measles were in the age group 0-1 year (58.7%). The mortality waas 24.8% and the highest mortality rate was in the age group of 4-5 years (34.6%). Most of the patients with bronchopneumonia and measles were in the age group of 1-2 years (30.3%). The mortality rate of bronchopneumonia with measles was 22.2% and the highest mortality rate was in the age group of 3-4 years (35. 7%). Bronchopneumonia with or without measles occurred more in malnourished patients rather than in well-nourished cases. All of the patients who had bronchopneumonia with measles in the present study had never been immunized against measles.

scholarly journals Haematological profile of children with malaria in Sorong, West Papua, Indonesia

2021 ◽  
Vol 20 (1) ◽  
Author(s):  
Syilvia Jiero ◽  
Ayodhia Pitaloka Pasaribu
Keyword(s):  
Malaria Infection ◽  
Clinical Symptoms ◽  
Public Health Problem ◽  
Malaria Diagnosis ◽  
White Blood Count ◽  
P Value ◽  
Major Public Health Problem ◽  
Age Group ◽  
Haematological Profile ◽  
West Papua

Abstract Background Malaria remains a major public health problem in Indonesian Papua, with children under five years of age being the most affected group. Haematological changes, such as cytopenia that occur during malaria infection have been suggested as potential predictors and can aid in the diagnosis of malaria. This study aimed to assess the haematological alterations associated with malaria infection in children presenting with signs and symptoms of malaria. Methods A retrospective study was performed by collecting data from the medical records of malaria patients at Sorong Regional General Hospital, Sorong, West Papua, Indonesia, both from outpatient and inpatient clinics, from January 2014 until December 2017. The laboratory profile of children suffering from malaria was evaluated. Results One hundred and eighty-two children aged 1 month to 18 years old were enrolled. The subjects were mostly male (112, 61.5%) with a mean age of 6.45 years (SD = 4.3 years). Children below 5 years of age suffered the most from malaria in this study (77, 42.3%). One hundred two subjects (56%) were infected with Plasmodium falciparum. Half of the enrolled subjects (50%) had haemoglobin level (Hb) between 5.1 and 10 gr/dL. A total of 41 children (53.2%) less than 5 years old suffered from P. falciparum infection. In the age group of 5–10 years, there were 34 children (57.6%) who suffered from P. falciparum, and in the age group > 10 years, 27 children (58.7%) suffered from P. falciparum infection. Only 4 subjects (5.2%) in the less than 5 years old age group had mixed malaria infection. Among eight predictors of the haematological profile, there were five predictors that were significantly associated with the diagnostic criteria, namely haemoglobin, haematocrit, leukocytes, platelets and monocytes (p < 0.05). Generally, clinical symptoms are not significantly associated with a malaria diagnosis, and only one variable showed a significant relationship, pale, with a P value of 0.001. Conclusions Children with malaria had changes in some haematological markers, with anaemia, low platelet count, white blood count, and lymphocyte count being the most important predictors of malaria infection in the study area. These markers could be used to raise suspicion of malaria in children living in high endemic areas, such as West Papua.

scholarly journals Allergic rhinitis in pediatric practice: how to identify and manage

2018 ◽  
pp. 142-146
Author(s):  
N. V. Minaeva ◽  
I. P. Koryukina
Keyword(s):  
Health Care ◽  
Allergic Rhinitis ◽  
Child Health ◽  
Pediatric Patients ◽  
Clinical Symptoms ◽  
Quality Criteria ◽  
Care Quality ◽  
Symptomatic Therapy ◽  
Child Health Care ◽  
Pediatric Practice

The issues of diagnosis and management of allergic rhinitis (AR) in pediatric patients are considered on the basis of the current Russian clinical guidelines. The article presents the paediatrician tasks on the collection of anamnesis, the assessment of clinical symptoms, the setting of a preliminary diagnosis, the development of a diagnosis and therapy plan taking into account the modern medical care quality criteria. It also discloses the general principles of AR treatment, approaches to emergency symptomatic therapy using a combined intranasal drug Vibrocil (dimethindene + phenylephrine). The paper provides recommendations for the management of patients with allergic rhinitis in the district covered by child health care.

scholarly journals Pediatric Hematologist/Oncologists' Beliefs about Hematopoietic Stem Cell Transplant for Children with Sickle Cell Disease: A Sickle Cell Transplant Advocacy and Research Alliance (STAR) Study

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 2164-2164
Author(s):  
Emily Riehm Meier ◽  
Allistair Abraham ◽  
Alexander I. Ngwube ◽  
Isaac Janson ◽  
Gregory M.T. Guilcher ◽  
...  
Keyword(s):  
Stem Cell ◽  
Sickle Cell ◽  
Thalassemia Major ◽  
Cell Transplant ◽  
Cell Disease ◽  
Asymptomatic Child ◽  
Hematopoietic Stem ◽  
Curative Therapy ◽  
Pediatric Hematology ◽  
Asymptomatic Children

Background Hematopoietic stem cell transplantation (HSCT) provides a curative therapy for children severely affected by sickle cell disease (SCD). Rejection-free survival following a matched sibling donor (MSD) HSCT for these children is very high. As safer approaches are developed, using reduced intensity conditioning and improved graft versus host disease prophylaxis, there is rationale for extending MSD HSCT to less severely affected children to spare them increasing morbidity and early mortality in adulthood. Providers' perceptions may contribute to the slow adoption of MSD HSCT for less severe SCD. In this study we assess providers' perceptions about MSD HSCT for children with variable SCD severity and determine the influence of provider characteristics on HSCT attitudes. Methods Pediatric Hematologists/Oncologists (PHO) were eligible to participate in this IRB exempt study. An e-survey was distributed to all STAR members and ASH members who self-identified as PHO and posted on the American Society of Pediatric Hematology/Oncology Clinical Forum listserv. Analysis was performed to describe participant demographics and the proportion of participants who practice exclusively Pediatric Hematology with a focus on SCD (PSCD) vs. general PHO; we evaluated correlations between these characteristics and likelihood of HSCT referral for each scenario. Results Of the 203 respondents, 59% were female, and 69% were between the ages of 30 and 49 years. Spearman's rank correlation analysis did not reveal any significant relationship between respondent age and likelihood to refer to HSCT for any of the survey scenarios. Two-thirds self-identified as Caucasian, 19% Asian, 7% African American and 3% Hispanic. 35% of respondents practiced general PHO, 20% PSCD, 20% Pediatric Hematology, 15% Pediatric HSCT, and 4% Hemostasis. The majority (75%) of respondents were very or somewhat likely to order HLA typing for a child with HbSS/HbSβ⁰thalassemia who had full siblings, regardless of disease severity. Only 48% would refer a child with HbSS/HbSβ⁰thalassemia who had an HLA-MSD but never admitted to the hospital; referral likelihood differed significantly by practice focus [PHO: 23% very or somewhat likely vs. 54% of PSCD (p=0.002)]. Conversely, 99% of respondents were very or somewhat likely to refer a child who suffered an overt stroke, while 85% and 84% were very or somewhat likely to refer a child who had an abnormal TCD or silent infarct, respectively. 49% of respondents were very or somewhat likely to refer a child with HbSS/HbSβ⁰thalassemia if they had a history of suboptimal adherence to hydroxyurea; referral likelihood again differed by practice focus [PHO: 35% very or somewhat likely vs. 64% PSCD (p=0.021)]. Concern about transplant related mortality (TRM) was the predominant reason for not referring. For children with severe clinical phenotypes of HbSC and HbSβ+thalassemia, 78% of respondents were very or somewhat likely to refer, compared to only 23% who would refer asymptomatic children with SCD variants. PHO and PSCD did not differ in the likelihood to refer either group. 87% of respondents were very or somewhat likely to refer a child with β-thalassemia major for MSD HSCT, regardless of disease severity; no statistically significant difference was found between PHO and PSCD in referral likelihood. A significantly higher proportion of respondents would refer an asymptomatic child with β-thalassemia major (87%) than those who were very or somewhat likely to refer an asymptomatic child with HbSS/HbSβ⁰thalassemia (47%, p<0.00001) or other SCD variants (23%, p= 0.0005). Discussion HSCT is a curative therapy for people with SCD. There was almost complete agreement that a HSCT referral should be made for children with HbSS/HbSβ⁰thalassemia and cerebral vasculopathies who have an MSD, but attitudes varied for almost every other clinical scenario posed. PSCD providers were more likely to refer asymptomatic patients and those with questionable adherence to hydroxyurea compared to general PHOs. TRM was the most common reason for not referring an asymptomatic child for MSD HSCT. Yet, TRM is low in the setting of pediatric MSD HSCT for SCD. Additional education about the decreased quality of life in aging people with SCD and their lack of improvement in life expectancy is needed so that physician perception of HSCT changes and the number of young asymptomatic children with SCD referred for MSD HSCT increases. Disclosures Meier: CVS Caremark: Consultancy. Guilcher:Jazz Pharmaceuticals: Other: ASH 2017 meeting attendance.

NEWS AND ANNOUNCEMENTS

PEDIATRICS ◽  
1969 ◽  
Vol 43 (2) ◽  
pp. 307-311
Keyword(s):  
Child Health ◽  
University Hospital ◽  
Stanford University ◽  
Palo Alto ◽  
North Central ◽  
The Third ◽  
Baylor University ◽  
School Of Medicine ◽  
Vanderbilt University ◽  
And Child Health

The Third Biennial Seminar on Prematurity will be held in Phoenix, Arizona, February 22-23, 1969. Guest speakers will be Dr. Thomas K. Oliver, University of Washington, Seattle; Dr. Arnold J. Rudolph, Baylor University College of Medicine, Houston, Texas; Dagmar Cechanek, R.N., Stanford University Hospital, Palo Alto, California; Dr. Mildred Stahlman, Vanderbilt University School of Medicine, Nashville, Tennessee; and Mrs. Rose Grobstein, MSW, Stanford University School of Medicine, Palo Alto, California. Registration fee, $7.50. For information write: Frederic W. Baum, M.D., Director, Maternal and Child Health Division, Goodrich Building, 14 North Central Avenue, Phoenix, Arizona 85004.

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