Clinical Presentation of Novel Influenza A (H1N1) in Hospitalized Children

Objective

Human pandemic influenza H₁N₁ virus as the cause of febrile respiratory infection ranging from self-limited to severe illness has spread globally during 2009. Signs and symptoms of upper and lower respiratory tract involvement, fever, sore throat, rhinitis, myalgia, malaise, headache, chills and fatigue are common. In this article we report the clinical presentation of Influenza A (H₁N₁) in our hospitalized children.

Methods

Between September and October 2009, all children requiring hospitalization for suspected H₁N₁ infection were transferred to Pediatric Infectious Diseases ward. For all patients the throat swab was taken for PCR testing to confirm or exclude the diagnosis of H₁N₁ Influenza A. Case patients consisted of H₁N₁-positive patients. Age, sex, symptoms, signs, laboratory data, CXR changes, details of therapy, duration of admission and patient outcome were documented.

Findings

Twenty patients were H₁N₁ positive. Mean age of the patients was 65.50±9.8 months. Fever and coughs were with 55% the most commonly reported symptoms. Other presentations included vomiting (55%), abdominal pain (25%), cyanosis and dyspnea (5%), body ache (40%), rhinorrhea (80%), sore throat (35%), head stiffness (5%) and loss of conciousness (5%). The median temperature of the patients was 38.5°C. Chest X-Ray changes were noted in 13 out of 20 patients (65%). Mean leukocyte and platelet was 6475 and 169000 respectively. Seventeen (85%) patients were treated with Oseltamivir, 3 patients received adjuvant antibiotics. The mean duration of admission was 3 days. Three patients required intensive care support and all of them expired due to superinfection.

Conclusion

Our data confirm that the presentation of influenza in children is variable and 2009 H₁N₁ influenza may cause leucopenia and thrombocytopenia.     

Atopic Manifestations: Dermatitis,Allergic Rhinitis and Asthma in Patients With Hypogammaglobulinemia

Background:

Most of the hypogammaglobulinemic patients have a clinical history in favor of allergic respiratory disease. Nevertheless, in these patients the importance and prevalence of atopic disorders have not been completely explained.

Objectives:

This study was aimed to evaluate atopic manifestations (dermatitis, allergic rhinitis and asthma) and pulmonary function in patients with hypogammaglobulinemia.

Patients and Methods:

We used the international study of asthma and allergies in childhood (ISAAC) questionnaire in forty-five patients diagnosed with hypogammaglobulinemia and spirometry was done in 41 patients older than 5 years.

Results:

Spirometry results were normal in 21 (51%), and showed obstructive in 15 (37%) and restrictive pattern in 5 (12%) of the 41 patients who were evaluated. By the end of the study, asthma was diagnosed in nine (20%) patients and other atopies (rhinitis and dermatitis) identified in 10 (22%), and four (9%), respectively.

Conclusions:

Atopic conditions should be investigated in the hypogammaglobulinemic patients and the prevalence in these patients may be higher than in normal population. Also, it is recommended to perform a pulmonary function test as a routine procedure in patients with hypogammaglobulinemia and atopy should be assessed in these patients.     

Hepatic fibrosis and survival in biliary atresia

In biliary atresia, progression of hepatic fibrosis before the Kasai portoenterostomy may explain why age is a major determinant of survival of the native liver. Blinded fibrosis scoring of surgical liver biopsies demonstrated that age and fibrosis correlate. Those biopsies with severe fibrosis predicted significantly worse survival.     

Factors regulating gastric emptying in preterm infants

OBJECTIVES: To determine whether osmolality, volume, or energy density affects gastric emptying rate (GE); whether simultaneously decreasing osmolality and increasing volume accelerates GE; and whether GE is related to any clinical and/or demographic characteristics. STUDY DESIGN: GE was measured using the (13)C-octanoic acid breath test in 17 infants of 25 to 30 weeks gestation in a tertiary care children's hospital. In study 1, the independent effects of osmolality, volume, and nutrient density were evaluated in 10 infants. In study 2, the combined effects of decreasing osmolality and increasing volume were evaluated in 7 infants. Results from all infants were used to investigate the relationships between clinical and demographic characteristics and GE. The statistical analysis comprised analysis of covariance, the (paired) Student t-test, the 1-sample Wilcoxon test, and regression analysis. RESULTS: Mean (+/- standard deviation) age was 27.7 +/- 14.6 days in study 1 and 34.1 +/- 14.0 days in study 2. Altering osmolality, volume, or energy density did not change GE, but simultaneously decreasing osmolality and increasing feeding volume accelerated GE by 18% versus standard feeding. GE correlated with gestational age at birth. CONCLUSIONS: Unlike in adults, in preterm infants osmolality, volume, or energy density individually do not affect GE. Reducing osmolality and increasing feeding volume increases GE. GE is related to gestational age at birth even at age 1 month.     

Incidence of Neonatal Hyperphenylalaninemia in Fars Province,South Iran

Objective

Phenylalanine hydroxylase or its cofactor, tetrahydrobiopterin (BH₄), deficiency causes accumulation of phenylalanine in body fluids and central nervous system. Considering the fact that hyperphenylalaninemia is a preventable cause of mental retardation in infants, the objective of this study was to determine the incidence of congenital hyperphenylalaninemia in Fars province, south of Iran.

Methods

In a period of one year from November 2007 to November 2008 blood samples were withdrawn from all newborns born in Fars province for measurement of serum phenylalanine. The samples with a serum level of≥ 2 mg/dl were referred to pediatric endocrine clinic for confirmation and determination of the type of hyperphenylalaninemia by quantitive serum phenylalanine measurements by using High-Pressure liquid chromatography (HPLC) method.

Findings

Nine out of 76966 newborns had a serum phenylalanine level≥2mg/dl, of which 8 cases were confirmed by HPLC. The incidence of the disease was 1:10000. The incidence of mild hyperphenylalaninemia and phenylketonuria (PKU) among the patients was 62.5% and 37.5% respectively and the incidence of BH₄ deficiency was 1/76966.

Conclusion

These findings indicate a high incidence of hyperphenylalaninemia, in the newborns from Fars province. The high incidence makes a comprehensive screening program for management of the disease necessary.     

Airway eicosanoids in acute severe respiratory syncytial virus bronchiolitis

We prospectively studied the levels of eicosanoids in intubated patients with severe bronchiolitis and compared them to electively intubated non-infected infants. LeukotrieneE(4) (LTE(4)), leukotrieneB(4) (LTB(4)), and prostaglandinE(2) (PGE(2)) levels were significantly increased (P <.01) from endotracheal (ET) aspirates of infants with bronchiolitis compared with controls, as were urinary LTE(4) levels (P <.001). We conclude that eicosanoids are increased in the tracheal aspirates and urine of children with bronchiolitis.     

Failure of conventional strategies to improve nutritional status in malnourished adolescents and adults with cystic fibrosis

The use of oral dietary supplements was compared with dietary counseling in 13 malnourished patients (3 males, mean age 18.1 years) with cystic fibrosis. Energy intake and nutritional status were evaluated over 3 months. There was no significant change in energy intake or percent ideal body weight in either group.     

Continuous glucose monitoring in adolescents with cystic fibrosis

The presence of cystic fibrosis (CF)-related diabetes was evaluated in 19 adolescents with CF by continuous glucose monitoring system (CGMS) and oral glucose tolerance testing. CGMS confirmed diabetic glucose excursions in 7/19 subjects deemed diabetic on oral glucose tolerance testing. CGMS is a useful tool for detecting hyperglycemia in CF.     

Inaccurate hemoglobin A(1C) levels in patients with type 1 diabetes and hereditary persistence of hemoglobin F

We report 2 African-American boys with type 1 diabetes and hereditary persistence of hemoglobin F. The diagnosis came to light after both patients exhibited inconsistent hemoglobin A(1C) (HbA(1C)) levels with respect to serum glucose measurements. This demonstrates the importance of frequent glucose monitoring and interpreting the HbA(1C) level in light of serum glucose measurements.     

Follow-up of newborns with elevated screening T4 concentrations

OBJECTIVE: To determine the type and incidence of hyperthyroxinemic disorders detected by follow-up of infants with elevated screening total T4 (TT4) values. STUDY DESIGN: Infants born in Oregon with a screening TT4 measurement >3 SD above the mean were offered enrollment. Serum TT4, free T4, total T3, free T3, and thyroid-stimulating hormone concentrations were measured in study infants and their mothers. RESULTS: Over a 20-month period, 101 infants (51 boys) and their mothers enrolled in the study (of 241 eligible infants), from a total screening population of 80,884; 17 infants were identified with persistent hyperthyroxinemia (TT4 >16 microg/dL). Ten had thyroxine-binding globulin excess (1:8088), 5 had evidence for increased T4 binding but not thyroxine-binding globulin excess (1:16,177), and 2 had findings compatible with thyroid hormone resistance (1:40,442); the other 84 infants had transient hyperthyroxinemia. Sequence analysis revealed a point mutation in the thyroid hormone receptor-beta gene in one infant with thyroid hormone resistance; no mutation was identified in the other infant. CONCLUSIONS: Although neonatal Graves' disease occurs in approximately 1 in 25,000 newborn infants, we did not detect any case among 80,884 infants, most likely because their mothers were receiving antithyroid drugs. Although the other hyperthyroxinemic disorders in the aggregate occur frequently (1:4758) and may benefit from detection, in general they do not require treatment.     

Alpha1-antitrypsin and alpha2-macroglobulin in newborn infants

The levels of alpha₁-antitrypsin and alpha₂-macroglobulin in the plasma of 129 newborns were determined. The infants were divided into 3 groups according to their perinatal history.In healthy newborns with an uneventful perinatal history the normal values for alpha₁-antitrypsin were 1.97±0.44 g/l, and for alpha₂-macroglobulin 3.11±0.69 g/l. No changes in these levels were found during the first week of life. The levels of alpha₁-antitrypsin and alpha₂-macroglobulin showed significant correlation to each other.In healthy newborns with different complications in the obstetric history the levels of alpha₁-antitrypsin were not influenced, whereas alpha₂-macroglobulin decreased slightly during the first week of life. The levels of alpha₁-antitrypsin and of alpha₂-macroglobulin showed no further correlation to each other.In sick term and preterm newborns (n=18) alpha₁-antitrypsin was increased in 5 of 7 babies suffering from bacterial infections and lowered in 4 of 9 cases with respiratory disturbances. Alpha₂-macroglobulin was lowered in 15 babies. These results indicate different kinetics of the two antiproteases in vivo.With support of the Landesamt für Forschung des Landes Nordrhein-Westfalen     

Serum alpha1-fetoprotein in cystic fibrosis

In 38 children and adolescents with cystic fibrosis, in 19 normal children and 62 healthy adults the serum ₁-fetoprotein concentrations were measured by radioimmunoassay. In cystic fibrosis patients 97.5% and in normal children 95% of the ₁-fetoprotein values were within the normal range for healthy adults (1–9 ng/ml). Critical judgement of the reported findings in literature and our own results demonstrate that the investigation of ₁-fetoprotein in the serum cannot serve for detecting homozygotes of cystic fibrosis genes or heterozygote carriers.

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Zusammenfassung Bei 38 Kindern und Jugendlichen mit cystischer Fibrose, 19 gesunden Kindern und 62 gesunden Erwachsenen wurden die Serumkonzentrationen des Alpha₁-Feto-proteins im Radioimmunoassay bestimmt. 97,5% der Alpha₁-Fetoprotein-Werte der Patienten mit cystischer Fibrose und 95% der Meßwerte der gesunden Kinder lagen innerhalb des Normbereiches (1–9 ng/ml), der für gesunde Erwachsene ermittelt worden war. Aufgrund unserer eigenen Untersuchungsergebnisse und bei kritischer Bewertung der bisher publizierten Befunde erscheint die Alpha₁-Fetoprotein-Bestimmung im Serum zur Erfassung der homozygoten und heterozygoten Genträger der cystischen Fibrose nicht geeignet.