Iranian Journal of Pediatrics
Year:2008 | Volume:18 | Issue:3|Papers Count:19

Objective: Juvenile myelomonocytic leukemia (JMML) is a rare myelodysplastic/ myeloproliferative malignancy of early childhood, characterized by monocytosis, hepatosplenomegaly and an aggressive clinical course.Methods: In semi-solid culture JMML progenitor cells proliferate spontaneously into colony forming units. In order to study the mechanisms of proliferation and differentiation of JMML cells we developed a suspension culture system without additional exogenous growth factor supplement. Mononuclear cells (MNC) from peripheral blood, bone marrow or spleen of 14 patients with JMML and 24 controls were studied.Findings: JMML cells expressed higher levels of the proliferation marker Ki67 (median 24% [7- 39%] vs a median of 3.5% in controls). 90% of JMML cells were CD68-positive (vs 35% in controls) and by day 7 all JMML samples contained CD1a- positive cells. Electron microscopy demonstrated cytoplasmic vesicular structures resembling multilamellar MHC II comparetiments, which together with the expression of CD1a - support a dendritic cell (DC)-phenotype.Conclusion: Differentiation into CD1a-positive DC seems to be a frequent phenomenon in cultured JMML MNC, which in vivo may contribute to clinical characteristics such as skin and organ infiltration.

Objective: The number of dengue fever (DF)/dengue hemorrhagic fever (DHF) cases reported in India has risen in recent years. This study was undertaken to evaluate clinical profile and outcome of children admitted with DHF/dengue shock syndrome (DSS), in the 2006 DHF epidemic in Ludhiana, Punjab.Methods: Eighty one children with dengue hemorrhagic fever were hospitalized in the Pediatric Department of Dayanand Medical College and Hospital, Ludhiana, India. All patients were diagnosed, managed and monitored according to a standard protocol.Findings: Children between 10-15 years were most commonly afflicted (59%). Infants were the least affected sub-group (3.7%). Ninety two percent of all children were of DHF and 8% cases presented in DSS. The common symptoms seen were fever (91%), vomiting (41%), poor intake (21%), abdominal pain (16%) and significant bleeding (15%). Hepatomegaly was present in 60% of cases. 85% of cases had petechiae alone, 15% had evidence of significant bleeding manifestation. Gastrointestinal bleeding was the commonest observed bleeding. The complications seen were liver dysfunction (14.8%), coagulopathy (3.7%), renal dysfunction (3.7%), and acute respiratory distress syndrome (2.4%) and disseminated intravascular coagulation (1.2%). Mortality in the study was 3.7%. Refractory shock and coagulopathy were seen in all cases with poor outcome.Conclusion: Increased awareness, better transport facilities and case management according to the WHO guidelines, is needed to further reduce mortality of DHF/DSS cases.

Objective: A wide range of childhood illnesses are accompanied by fever, many of which are treated at home prior to presentation to hospital. An assessment of mothers' knowledge and ability to recognize fever in their child, as well as management instituted at home were the focus of this study.Methods: One hundred and forty four mothers whose children were less than 12 years old, had fever as one of the presenting complaints, and admitted to the children emergency room of Lagos State University Teaching Hospital between July and December, 2006, were interviewed with a structured questionnaire. Information sourced were the demographics of the mothers / caregivers and the children, mothers' / caregivers' knowledge of fever and its management. Mothers that did not give their consents were excluded.Findings: Most of the mothers (83.3%) perceived fever as the hotness of the whole body of the patient. Infection was the most likely cause of fever identified by 43.8% mothers. Malaria was presumptively diagnosed by the mothers in 54.2% children similar to the 53.5% cases of malaria diagnosed on admission. Ninety-six mothers (66.7%) managed the fever at home.Home treatment was majorly by reducing the clothing and exposing the child to air, tepid sponging, and use of paracetamol. Antimalarials (6.0%) and antibiotics (7.8%) group of drugs were rarely used.Conclusion: Home management of fever by mothers had remained symptomatic in Nigeria. In the era of artemesinin combined therapy as the choice of malaria treatment in Nigeria, trend towards not using antimalarial drugs as part of home management of fever in children was observed amongst the mothers.

Objective: A prospective study to determine the prevalent bacterial agents of neonatal sepsis and their antimicrobial susceptibility in lmam Khomeini teaching hospital, Urmia, from act 2002 to Nov 2006.Methods: Newborns with clinical signs of septicemia and positive blood culture during fifty months were prospectively studied. Samples for blood cultures, complete blood count, erythrocyte sedimentation rate, C-reactive protein, sugar, electrolytes, cerebrospinal fluid and urine analysis/culture were obtained; ampicillin and gentamycin were started empirically.Results were analyzed by SPSS13package and cross tabulation was done.Findings: Two thousand three hundred twenty five newborns from 4827 neonatal admissions were screened for septicemia. Two hundred twenty seven episodes of sepsis occurred in 209 newborns. The boys/girls ratio was 1.67:1 and 63.9% of patients were premature. There were 164 (72.2%) cases of EONS and 63 (27.7%) cases of LONS. Coagulase negative staphylococcus (CONS) was the most common (54%) cause of both early and late onset neonatal sepsis and showed high degree of resistance to commonly used antibiotics, ampicillin (100%), ceftriaxon (65%), cefotaxim (67%) and gentamicin (51%), but comparatively low resistance to vancomycine (10%), imipenem (19%), and ciprofloxacine (23%).Conclusion: Neonatal sepsis in our ward is mainly caused by gram-positive organisms, which are developing resistance to commonly used antibiotics. The initial empirical choice of ampicillin and gentamycin appears to be unreasonable for our environment.

Objective: Autism is a life long developmental disorder that emerges in early childhood and results in significant lifelong disability. The goal of treatment is to promote the child’s social and language development and minimize behaviors that interfere with the child’s functioning and learning. This study evaluated the impact of casein and gluten free diet among selected autistic children.Methods: Three private special schools in Salem District, Tamilnadu, India were selected. A total number of 50 autistic children 10 from SIMEC, 10 from MMIC and 30 from CSI comprised the study sample. Background information, clinical history and nutritional status, dietary pattern were collected from the 50 selected autistic children. Out of 50 autistic children 30 autistic children were selected for the dietary intervention. Diet counseling regarding casein free diet was imparted to Group I (n=10), gluten free diet to Group II (n=10) and both casein and gluten free diet for Group III (n=10). The diet was followed for a period of 2 months. The efficacy of the dietary exclusion of casein and gluten was evaluated using a food and behavior diary on a day to day basis, using observation method.Findings: Results about Group I autistic children who followed dietary exclusion of casein free diet showed that the mean scores before and after casein free dietary intervention depiticted these improvements as, 1 to 1.2 for attention, 2.8 to 2.9 for sleep, 1.1 to 1.3 for hyperactivity, 1.1 to 1.2 for anxiety/compulsion. For Group II autistic children who followed dietary exclusion of gluten free diet showed the improvements as 1.1 to 1.4 for attention 2.5 to 3 for sleep, 1.7 to 1.9 for hyperactivity, 1.1 to 1.2 for anxiety/compulsion. About Group III autistic children who followed dietary exclusion of both casein and gluten free diet showed the improvements as 1.1 to 1.3 for attention, 2.5 to 2.7 for sleep, 1.3 to 1.7 for hyperactivity, and 1.1 to 1.2 for anxiety/compulsion.Conclusion: The impact of dietary intervention made using dietary guide books found to be useful to reduce various behavior symptoms among the selected autistic children.

Objective: Clinical studies comparing intermittent versus continuous phototherapy for reducing neonatal hyperbilirubinemia have produced conflicting results. This study was conducted to compare the efficacy of intermittent with continuous phototherapy.Methods: This study was performed on 114 neonates with indirect hyperbilirubinemia.Inclusion criteria were body weight above 2000 grams, absence of other concomitant diseases, and hyperbilirubinemia neither requiring intensive phototherapy nor exceeding the range of exchange transfusion. The neonates were randomly divided into two groups. Continuous phototherapy group received phototherapy on and off for 2 hours and half an hour respectively and the intermittent phototherapy group on and off for one hour. The phototherapy units were identical and serum total bilirubin levels were measured every 12 hours after starting phototherapy.Findings: Two groups were matched regarding weight and risk factors such as ABO and Rh incompatibility. The difference of total serum bilirubin levels between two groups was insignificant at the start of phototherapy and also after 12, 24, 36 and 48 hours (P>0.2).Conclusion: Intermittent phototherapy defined as one hour on and one hour off is as effective as continuous phototherapy defined as 2 hours on and half an hour off, in reducing total serum bilirubin level in full term babies.

Objective: Lymphedema is the result of impaired lymphatic drainage from the affected organ.This abnormality can be primary or secondary. Different nonoperative and operative approaches have been introduced to treat chronic lymphedema. In this study, we describe a new surgical technique and compare its results with other more commonplace methods.Methods: 59 patients with the diagnosis of chronic lower extremity lymphdema who had not responded to nonoperative management for at least 6 months, were included in the study. They were collected during 15 years between March 1987 and March 2002. Doppler ultrasonography of deep venous system to confirm its patency was routinely performed in the most of patients. Then, they underwent surgery and were followed for at least 1 year postoperatively.Findings: All the patients were operated by our new technique which is a modified form of the Homans. The outcome was excellent and 89.2% of patients were devoid of complication. A 10.8% total complication rate was inevitable. The most common complication was wound seroma.Conclusion: According to the difficulties with treatment of chronic lymphedema and variety of surgical options, our method can be an excellent and even the standard operative procedure to treat intractable forms of disease.

Objective: Due to worldwide variations, reference values of urinary calcium to creatinine ratio in pediatric population are not yet well established. To determine normal values for urinary calcium to creatinine ratio and its relation to urinary sodium or potassium, a descriptive (correlation type) study was conducted in 7 to 12 years old healthy children in Urmia, Iran.Methods: Primary school children were divided into two sectors and 7 clusters (4 cluster school boys and 3 school girls). The subjects were randomly selected. Random, non–fasting morning urine samples were obtained from 364 healthy children aged 7 to 12 years during fall 2005 and immediately sent to laboratory to determine urine calcium (Uca), creatinine(Cr), sodium (Na) and potassium (K). For data analysis, mean and 95th percentile of UCa/Cr and UNa/K were used. Pearson test was used to determine any relationship between UCa/Cr and UNa/K values. For comparison of UCa/Cr and UNa/K values between males and females, Mann- Withny test was used.Findings: A total number of 364 children were enrolled in the study. There were 208 (57.1%) males and 156 (42.9%) females. The mean and 95th Percentile for UCa/Cr was 0.11 (0.10 and 0.24 respectively. The mean and 95th percentiles for UNa/K were 2.30 (1.42 and 5.21 respectively. There was no significant difference in UCa/Cr and UNa/K between two sexes (P>0.05). We found a weak relationship between UCa/Cr and UNa/K (P<0.01).Conclusion: UCa/Cr value may differ according to geographic location. For screening purposes, reference values should be determined in each geographic location.

Objective: Since the beginning of the 21st century, injury has been the most serious public health problem that children face with. The aim of this study was epidemiologic evaluation of unintentional injury in north of Iran.Methods: In a prospective cross-sectional study between September 2005–July 2006 we studied data of 347 trauma patients aged 14 years and younger, including sex, age, trauma mechanism, transportation facility, anatomical sites of injury, severity of head injury according to GCS (Severe: 3-8, Moderate: 9-12, Mild: 13-15), injury severity score (ISS), and length of hospital stay (LOHS). SPSS 13 and independent t-test were used to analyze of data.Findings: Out of 3605 trauma patients, 353 were 14 years old or younger with a mean age (SD) of 7.6 (4.0), Male to female ratio about 2:1. Most of them (42.9%) were 1-5 years old. In patients younger than 1 year, falling was the most common (52.2%) mechanism of trauma while in other groups, traffic accidents were the leading cause of injury. About half of the patients were transported to hospital by their families or other people. About 77% of them suffered from head injury. Mean (SD) ISS was 4.3 (6.6), about 12% had severe injury and it was more than 25 in about 3%. Mean (SD) LOHS was 5.1 (8.9) days.Conclusion: Occurrence of falling and traffic accidents in children is remarkably high. Mainly traffic accidents are preventable. We suggest paying more attention to safety education in all levels of community.

Background: The omental-mesenteric myxoid hamartoma (OMMH) is a very rare lesion, mainly seen in children and characterized by multiple omental and mesenteric nodules, which may be confused with malignant neoplasm. Microscopically, these lesions consist of a richly vascularized myxoid stroma with plump mesenchymal cells. This lesion has a benign clinical course without recurrence during follow up.Case Presentation: We present a 14-month-old boy that was referred with history of abdominal distension, fever and vomiting for 3 months. Enhanced computed tomography (CT) revealed a huge well-demarcated hypodense and spherical mass which displaced bowel loops without obvious penetration to the intestinal walls. Histological and immunohistochemical examinations confirmed the diagnosis of OMMH.No evidence of recurrence was noted during 3 years follow up.Conclusion: OMMH is a very rare lesion and because of its aggressive appearance, differential diagnosis with malignancy is warranted. The clinical picture of our case also led to high suspicion of malignancy. However by consideration of histological and immunohistochemical findings we could achieve the correct diagnosis.

Background: Neonatal primary hyperparathyroidism (NPHP) is a rare disease characterized by marked hypercalcemia, diffuse parathyroid hyperplasia and skeletal demineralization. These patients have symptoms of chronic hypercalcemia such as failure to thrive, irritability, abdominal pain and anorexia. It is often fatal unless parathyroidectomy is performed.Treatment with drugs usually is inadequate and often results in chronic hypercalcemia and death.Case presentation: A 10-day-old, 2.9 kg male newborn was hospitalized for anorexia, poor feeding, cyanosis, hypotonia, lethargy and severe dehydration. Diagnosis of severe hypercalcemia due to primary hyperparathyroidism was established and surgical approach selected because of failure of medical therapy to control hypercalcemia. The baby was successfully treated by total parathyroidectomy with autotransplantation.Conclusion: Although neonatal primary hyperparathyroidism (NPHP) is a rare disease, it must be considered for differential diagnosis in neonates with severe hypercalcemia. Early diagnosis and total parathyroidectomy with autotransplantation can be life-saving.

Background: Cytomegalovirus (CMV) is the most common cause of congenital infection.Although most of the involved neonates are asymptomatic but virus can cause a range of problems from mild to severe illness with involvement of different organs like central nervous system, gastrointestinal and liver. Proneness to CMV is very high (up to 1% of neonates). In the other hand trosinemia type I is a rare metabolic disorder with involvement of liver, neurologic, bone and other organs.Case Presentation: A 3-month-old infant, product of twin pregnancy was hospitalized because of jaundice, FTT, hepatomegaly and sepsis. The other twin showed normal growth with no problems. Work up for cholestasis and FTT was suggestive of two different entities. Polymerase chain reaction for CMV in liver tissue and serum was positive. Meanwhile laboratory findings for metabolic disorder led to the diagnosis of type 1 tyrosinemia. The other twin was infected with CMV too.Conclusion: Although coexistence of two causes for cholestasis is very rare, it is sometimes necessary to study more to rule out other entities like metabolic disease including tyrosinemia (if any symptoms exist).

Background: Fourth branchial pouch anomaly (4BPA) is a rare condition that often presents an extreme diagnostic and therapeutic challenge. It usually causes recurrent left thyroid or perithyroid tissue inflammation, but it can be situated in the right side too.Case presentation: In this case report we present a nine-year-old girl with right sided 4BPA.She was referred for evaluation of recurrent right muscular space mass and fistula that was unresponsive to any medical or surgical procedures. This paper demonstrates the proper technical issues about diagnostic work up and surgical approach that we used to manage her dilemma.Conclusion: In patients with both skin fistula and pyriform sinus opening, external cannulation is more convenient and less traumatizing than internal cannulation.

Newborn hypoxic-ischemic encephalopathy is an important clinical problem associated with considerable morbidity and mortality. Birth prevalence ranges from 1.8 to 7.7 per 1000 term live births [1, 2, 3]. This 27 days male child presented with small size of head, not accepting feeds and listlessness. He was born at full term at home with normal vaginal delivery. At 6th month of gestation mother had fever and took some medication for the same however details were not available. On examination head circumference was 30 cm and coronal suture was fused. Child was dull and less active. CT scan showed diffuse brain atrophy and multiple infarctions.

A 7-year-old male child was brought to outpatient department by his mother with a complaint of painless nodule on the right side of the abdomen below the costal margin.Otherwise, the child was in good general health and had no other complaints. There was no history of trauma in the past. On inspection there was no evidence of swelling and it was only on very careful palpation a nodule of approximately 1.0 cm in diameter, which was freely movable, was felt in the right subcostal region. The overlying skin was normal. An FNAC was performed and it revealed a background of polymorphonuclear infiltrate rich in eosinophils and a typical wavy outer larval membrane with a stroma containing multiple ovid nuclei. An ultrasound of the exact region was obtained.