Early onset group B streptococcal disease. Seven year experience and clinical scoring system

Early onset group B streptococcal disease was reviewed for the seven year period between 1975 to 1981 at Vanderbilt University Medical Center. One hundred and twenty cases were identified. The disease varied from asymptomatic bacteremia to fatal cardiopulmonary collapse. Factors associated with a poor outcome were prematurity, low Apgar score at 5 min, the presence of shock, leukopenia, rupture of membranes for more than 12 hours, and a delay in treatment after the onset of symptoms. A scoring system for probability of death based on these 6 factors was then developed. Over the seven year period mortality decreased from 50% to 10%. The only factor identified with the decrease in mortality was a significant decrease in the number of hours between the onset of symptoms and the beginning of treatment. Early recognition and prompt treatment seem to be the major causes of the decreasing mortality over the seven years of this report.     

Reviewing Omenn syndrome

Omenn syndrome is a form of severe combined immunodeficiency associated with high mortality. Early recognition is required in order to initiate life-saving therapy. This review provides information on the clinical symptoms, laboratory parameters and pathology of the disease, supporting early diagnosis in suspected patients. A literature search was performed using Medline, encompassing the period 1965-1999. Sixty-seven cases were identified and with the addition of a recently diagnosed patient at our hospital, 68 children were included. Median age at onset of symptoms was 4 weeks. Key symptoms were erythematous rash (98%), hepatosplenomegaly (88%), lymphadenopathy (80%), often accompanied by recurrent infections (72%) and alopecia (57%). An elevated WBC (55%) was frequently observed, due to eosinophilia and/or lymphocytosis. B-cell counts were significantly decreased whereas T-cell counts were elevated. A high serum IgE was another frequent finding (91%). Therapeutic options include bone marrow transplantation or cord blood stem cell transplantation; however, the mortality still was 46%. CONCLUSION: Omenn syndrome is a fatal disease if untreated. The mortality may be reduced when diagnosis is established early and treatment is initiated rapidly by using early compatible bone marrow transplantation or cord blood stem cell transplantation.     

迟发型尿素循环障碍的神经精神损害

尿素循环障碍是一组罕见病,为可治可防的遗传代谢病,由于尿素循环中酶或转运体活性减退或丧失导致尿素循环障碍,引起高氨血症、脑病及肝病。尿素循环障碍患者的发病年龄和严重程度取决于残余酶活性或转运体功能及发病诱因,多在儿童期发病,常于新生儿期或婴幼儿期出现高氨血症,导致严重脑损伤甚至死亡。据报道,高达10% 的患儿16岁以后发病,由于部分酶缺陷引起迟发型尿素循环障碍,临床表型复杂。轻重程度不同的神经精神疾病为首发或主要表现。既往报道迟发型尿素循环障碍患者病死率为 11%,早发现、早诊断、早治疗是改善患者预后的关键。     

Neonatal septicaemia in Finland 1981-85. Predominance of group B streptococcal infections with very early onset

A survey of blood culture-confirmed neonatal septicaemia was carried out in seven delivery hospitals in 1981-85, for a second successive five-year period. The total number of cases was 377, to compare with 410 in the previous five-year period. Group B streptococcus (GBS) was throughout the major pathogen (29%), followed by Staphylococcus aureus (15%) and Escherichia coli (14%), while Staphylococcus epidermidis (10%) has emerged as a significant new causative agent. Septicaemia with very early onset was predominant: 49% of the cases had onset within the first 24 hours; in the majority the symptoms were present from birth. GBS was responsible for 49% of the cases detected in the first 24 hours of life. The overall mortality was 20% as compared to 23% in the previous five-year period, whereas in the very early onset septicaemia mortality was now 18%, down from the preceding 30%. Despite the modest progress, GBS septicaemia with very early onset remains a significant problem, and effective preventive measures are needed.     

Neonatal Septicaemia in Finland 1981–85

ABSTRACT. A survey of blood culture-confirmed neonatal septicaemia was carried out in seven delivery hospitals in 1981–85, for a second successive five-year period. The total number of cases was 377, to compare with 410 in the previous five-year period. Group B streptococcus (GBS) was throughout the major pathogen (29%), followed by Staphylococcus aureus (15%) and Escherichia coli (14%), while Staphylococcus epidermidis (10%) has emerged as a significant new causative agent. Septicaemia with very early onset was predominant: 49% of the cases had onset within the first 24 hours; in the majority the symptoms were present from birth. GBS was responsible for 49% of the cases detected in the first 24 hours of life. The overall mortality was 20% as compared to 23% in the previous five-year period, whereas in the very early onset septicaemia mortality was now 18%, down from the preceding 30%. Despite the modest progress, GBS septicaemia with very early onset remains a significant problem, and effective preventive measures are needed.     

Rectal biopsy in the investigation of constipation

AIMS: To develop criteria to prevent unnecessary rectal biopsies in constipated children. METHODS: A retrospective review of 186 rectal biopsies from 141 children, comparing the age at onset of symptoms with the diagnosis of Hirschsprung's disease. RESULTS: All of the 17 children with Hirschsprung's disease had the onset of symptoms before the age of 4 weeks. Twenty seven children had delayed passage of meconium (more than 48 hours) of whom 10 had Hirschsprung's disease. Three children with Hirschsprung's disease were referred after the neonatal period (2 months, 11 months, and 3 years) but all had the onset of symptoms before 4 weeks of age. CONCLUSION: If the age at onset of constipation is after the neonatal period, a rectal biopsy is unnecessary.     

Neonatal septicaemia.

A total of 410 proved cases of neonatal septicaemia from seven Finnish hospitals seen between 1976 and 1980 were reviewed. The annual incidence of neonatal septicaemia was 3 per 1000 births, and overall mortality was 23%. Onset was early in most patients. Symptoms of septicaemia occurred within the first 24 hours of life in 44% and within the first week of life in 90%. In the very early onset disease (within 24 hours) mortality was 30%, compared with 17% in all other cases. Group B streptococcus was the leading cause in very early onset disease (52%) but mortality from infection with this organism was similar to that in other very early onset cases. It is concluded that very early onset neonatal septicaemia, probably of intrauterine origin and caused by group B streptococcus in one half of the cases, constitutes the major form of neonatal septicaemia in Finland and should receive the highest priority in preventive measures.     

Community effectiveness of malaria treatment in Uganda--a long way to Abuja targets

INTRODUCTION: At the Roll Back Malaria summit for African countries in Abuja, the heads of state committed to ensure that by the year 2005 at least 60% of those suffering from malaria would have access to effective treatment within 24 hours of onset of symptoms. AIM: The aim of the study was to assess community effectiveness of malaria treatment in children. METHOD: A community-based survey of 500 households was undertaken in western Uganda. RESULTS: A total of 260 (52%) children were reported to have had fever within the previous 2 weeks: 87% received some kind of treatment, 44% were said to have been treated within 24 hours of onset of symptoms, 47% received appropriate anti-malarials, 25% received the correct dosage, and 24% took the drug for the recommended period of time; altogether, only 7% received all the treatment steps. CONCLUSION: With drug efficacies of 50-90%, we estimate a community effectiveness of 4-6%, which is far from the 2005 Abuja target. The greatest need for improvement in the Home Based Fever Management strategy is in reducing delay in treatment and improving dosage and duration of treatment.     

Nocturnal home oxygen in the treatment of hypoxemic cystic fibrosis patients

The effect of nocturnal oxygen therapy on mortality and morbidity rates and on progression of disease was tested in a double-blind, randomized trial of 28 subjects with advanced cystic fibrosis. Patients were selected on the basis that their awake arterial oxygen tension was less than 65 mm Hg when they were clinically stable. Oxygen was prescribed in 1 L/min increments to obtain an awake arterial oxygen tension of greater than or equal to 70 mm Hg. Subjects received humidified oxygen or room air from modified concentrators. They were enrolled over a 3-year period and followed for an average (+/- SD) of 26 +/- 9 months. The average number of hours per night of concentrator use was 5.3 +/- 3.2 hours in the air group and 7.0 +/- 1.9 hours in the oxygen group. Over the follow-up period there were four deaths in each group, and oxygen therapy had no significant effect on the frequency of hospitalizations. Progression of disease was ascertained from nutritional status, pulmonary function, blood gas values, exercise ability, and right ventricular ejection fraction response to exercise (as measured by equilibrium-gated radionuclide angiocardiography), and psychologic status was measured by standardized tests of mood, self-esteem, and cognitive function; group comparisons for the first year revealed no significant differences; however, school or work attendance was maintained in the oxygen group but deteriorated in the air group. Clinical signs of cor pulmonale were documented during follow-up in 10 patients in toto, and all lived at least 9 months from the onset of these signs. The lack of association between the onset of these signs and imminent death, or the usefulness of measurements of the maximal oxygen uptake during progressive exercise and the right ventricular ejection fraction response to exercise as prognostic indicators, suggest that death may not be the result of cor pulmonale. We conclude that nocturnal oxygen treatment in patients with cystic fibrosis did not appear to affect mortality rates, the frequency of hospitalizations, or the progression of disease; oxygen use should be instituted only after the development of symptoms related to hypoxemia.     

Prognosis of chronic granulomatous disease.

The records of 28 patients with chronic granulomatous disease born over a 32 year period were reviewed. The characteristics of the group, and the frequency with which various clinical and laboratory features had been recorded, was assessed. Nine patients were known to have died, in most cases of progressive suppurative infection. Actuarial analysis showed 50% survival through the third decade of life. The long term survival of patients developing symptoms after the end of the first year of life was significantly better than that of patients whose illness started in infancy. Our data confirm that the severity of chronic granulomatous disease is not uniform, and that the prognosis for long term survival is better than that suggested in earlier reports. Early onset may be a poor prognostic sign and invasive aspergillosis is a life threatening complication. In the absence of curative treatment, trials to assess the effectiveness of interferon gamma are necessary and early antenatal diagnosis should be offered to as many affected families as possible.     

A 10-year prognosis for childhood allergic rhinitis

The prognosis of allergic rhinitis was studied in 154 children aged 3-17 years at diagnosis by means of a detailed questionnaire administered 8-11 years later. The symptoms had completely disappeared in only 15 (10%) patients. The conjunctival symptoms, however, had disappeared or were controlled successfully by topical drug therapy in almost all, and 77 (50%) were managing without medication for allergic rhinitis. Twenty-five (23%) of the 110 children with seasonal allergic rhinitis had a perennial disease at follow-up, in contrast to seven (16%) of 44 with perennial allergic rhinitis originally who had only seasonal symptoms at follow-up. Asthma or wheezing had developed in 29 cases (19%) and was more common (p less than 0.01) among those with perennial allergic rhinitis (15 of 44) than among those with seasonal allergic rhinitis (14 of 110). No significant association was found between age at onset of symptoms, family history of atopic disease or type of treatment for allergic rhinitis and allergic rhinitis still present at follow-up or development of asthma during the observation period.     

Natural History of Allergic Diseases in Children

Abstract. Questionnaire data from 1335 14-year-old children with a history of past or present asthma, allergic rhinitis or eczema were analysed regarding age at onset and cessation of symptoms. Incidence of asthma and eczema was highest during the first years of life. Early incidence of asthma was higher in boys than in girls but the sex ratio equalized gradually during childhood. Early incidence of eczema was equal between the sexes but the proportion of girls increased gradually with later age at onset. In 24 % of the children more than one symptom was found. Cessation of symptoms was common in asthma (55 %), in particular in those with early onset (74 %), but less common in eczema (34 %). Cessation of symptoms was less common in both diseases when associated with other allergic symptoms. Incidence of allergic rhinitis was fairly constant during childhood and cessation of symptoms was uncommon. A high risk of allergic airways disease was found after early eczema.     

Natural history of allergic diseases in children

Questionnaire data from 1335 14-year-old children with a history of past or present asthma, allergic rhinitis or eczema were analysed regarding age at onset and cessation of symptoms. Incidence of asthma and eczema was highest during the first years of life. Early incidence of asthma was higher in boys than in girls but the sex ratio equalized gradually during childhood. Early incidence of eczema was equal between the sexes but the proportion of girls increased gradually with later age at onset. In 24% of the children more than one symptom was found. Cessation of symptoms was common in asthma (55%), in particular in those with early onset (74%), but less common in eczema (34%). Cessation of symptoms was less common in both diseases when associated with other allergic symptoms. Incidence of allergic rhinitis was fairly constant during childhood and cessation of symptoms was uncommon. A high risk of allergic airways disease was found after early eczema.     

Appendicitis in infancy

Acute appendicitis is the most common cause of abdominal pain requiring surgery in children. But it is an uncommon entity in young children and rare in infants. During a 10-year period, between January 1991 and December 2000, 7 infants (age from 17 days to 8 months) were treated for acute appendicitis at Changhua Christian Hospital. All of the preoperative symptoms and signs, the duration between admission and operation, pathology reports, and laboratory data were reviewed. There were no specific clinical signs and symptoms. The duration between admission and operation ranged from 2 hours to 5 days (mean: 31.4 hours). The pathology reports revealed 3 were gangrenous, 3 were gangrenous with perforation and one was suppurative. There was one death. Pseudomonas aeruginosa was cultured from blood, ascites, bile or stool in 6 cases (85.7%). Early diagnosis of acute appendicitis in infants is still difficult. Although the mortality has declined, the morbidity still remains high. The high percentage of infection by Pseudomonas aeruginosa should be further evaluated in infantile appendicitis.     

Tethered spinal cord syndrome--the importance of time for outcomes.

OBJECTIVES: Tethered spinal cord syndrome is a relatively uncommon disorder of the medullary spine, usually associated with other congenital diseases of the caudal end of the spinal cord. However, it often remains unrecognised and due to its progressive character has serious consequences for those affected. METHODS: We analysed a group of 22 patients, treated for TTS during the period from 1990 to 2005, focussing on the time period between the onset of symptoms and their treatment. The initial data from the year 1998 already pinpointed this time-lag as a crucial prognostic factor for outcomes. RESULTS: Nine of the 22 treated patients showed an improvement of symptoms, 2 of them ad integrum. Eleven remained unchanged, without further progress and 2 patients worsened after surgery or despite it. Our results showed a direct correlation between the time-lag and the outcome of therapy. The average time span between recognition of disease and operation in those patients whose symptoms improved was about 16.7 months, whereas for those who remained unchanged it was 52.4. In the patients who worsened it was 54 months. CONCLUSION: Virtually all patients operated within the first year showed a subsequent improvement of symptoms. Only 25 % of patients operated at a later stage achieved some measure of improvement. Time is a relevant factor for success.     

A five-year chart review of children biopsied to rule out Hirschsprung's disease

A 5-year chart review of all children biopsied to rule out Hirschsprung's disease was conducted at Johns Hopkins Hospital. A total of 150 (90%) charts of the 160 children who were biopsied were obtained and reviewed. Twenty-four children (16%) had aganglionosis by surgical pathology report. All had the onset of symptoms in the neonatal period. Ninety-seven percent of children without Hirschsprung's disease and who were biopsied after the neonatal period did not have symptoms during the first 4 weeks of life. Despite having severe symptoms from birth, nine (37.5%) of the children with Hirschsprung's disease were not biopsied until an average age of 15 months. Data indicate that using the presence of bowel symptoms from the neonatal period as criteria for referral for biopsy would have missed none of the children with Hirschsprung's disease. Children with symptoms dating from the neonatal period should be considered for biopsy to avoid potential morbidity and mortality from the complication of enterocolitis.     

Natural and modified history of isolated ventricular septal defect: A 17-year study

Summary We studied 882 cases of isolated ventricular septal defect (VSD) diagnosed from 1971 to 1988 with a mean follow-up period of 9.5 years. They represent 22.5% of all congenital heart defects diagnosed in this period. Six hundred eighty-three children (77.4%) did not develop symptoms, had small defects, and the rate of spontaneous closure was high (40.2%). The remaining 199 children presented symptoms; at the initial catheterization 25, 65, 107, and 2 cases were grouped in hemodynamic groups 2, 3, 4, and 5–6, respectively, on the basis of pulmonary flow and resistance. Only seven patients (0.7%) developed aortic regurgitation, and only five patients (0.5%) developed infective endocarditis. Complete surgical correction was performed in 137 children (15.5% of the total cases), with surgical mortality decreasing from 21.4% before 1983 to 3.5% afterward. Overall mortality was 3% for the entire cohort, 0% for hemodynamic groups 1 and 2, 3% for group 3, and 25% for hemodynamic groups 4–6. About two thirds of the deaths took place between 1 month and 1 year of life, and one thirds of the deaths occurred before surgical treatment. Surgical mortality rates for hemodynamic groups 2, 3, and 4 were 0, 4.7, and 15.3%, respectively. Actuarial survival curves show an important improvement in the prognosis after 1983. Our results stress the importance of early surgical complete correction on patients with large defects and severe hemodynamic changes.     

Systemic group D streptococcal infection in newborn infants.

Thirteen cases of group D streptococcal neonatal sepsis and/or meningitis were identified at the Cincinnati Children's Hospital from 1970 to 1976. Ages at onset of disease ranged from 1 to 25 days. The most frequent symptoms were fever (five cases), lethargy (five cases), and respiratory difficulty (four cases). Blood cultures for seven infants were positive; CSF cultures for five infants were positive; and CSF and blood cultures for one infant were both positive. In 12 patients, parenteral antibiotic therapy consisted of a penicillin and an aminoglycoside. One infant with a severe meningomyelocele died. The other 12 infants showed a rapid clinical response with seven patients improving within 48 hours of the start of therapy. Infection with group D streptococcus results in a low-grade systemic disease in both full-term and premature infants that responds favorably to appropriate therapy.     

Indian red scorpion envenoming

The clinical course and treatment outcome of scorpion envenoming in 293 children was studied in a hospital at Mahad in Raigad district of Maharastra. 111 (38%) children who reported 1–10 hours (mean 3.5 hours) after sting had hypertension, 87 (29.6%) with tachycardia reported within 1–24 hours (mean 6.7 hours) of being envenomed and 72 (24.5%) children developed acute pulmonary edema after 6–24 hours (mean 8 hours) of sting. Six victims were brought dead, while 17 (6%) died later owing to multiorgan failure with loss of consciousness and convulsions (who reported after 24 hours of sting). Early administration of prazosin (125–250 ug orally) improved the clinical symptoms. Morbidity and mortality due to scorpion envenoming depends upon time lapse between sting and administration of post synaptic α-1 blocker, prazosin hydrochloride.     

Idiopathic pulmonary fibrosis in infants: good prognosis with conservative management.

BACKGROUND: Pulmonary interstitial fibrosis in children is a disease of unknown aetiology, usually associated with a poor prognosis. METHODS: In this case series we describe 11 children presenting over a 10 year period, managed conservatively and associated with a good prognosis. RESULTS: In six, symptoms were present from birth and 10 had symptoms at or before 3 months. Diagnosis was made using chest computed tomography and percutaneous lung biopsy. All patients were treated with oral prednisolone. In five no steroid response was noted. One patient responded to hydroxychloroquine. Home oxygen was required in five patients. At follow up all patients are alive at a median age of 6 years (range 1 to 12 years). The two recently diagnosed children have significant symptoms, seven have dyspnoea on exercise, and two are symptom free. CONCLUSION: The good prognosis seen in these patients is different to previous case reports, indicating a greater than 50% mortality.