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Query: UMLS:C0243026 (sepsis)
 52,417 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Twenty infants and young children with hereditary fructose intolerance (HFI) were admitted to hospital. None was diagnosed at admission. Referals were for vomiting of unknown aetiology (16X), pyloric stenosis or hiatus hernia (5X), toxic condition (3X), and hepatomegaly of unknown origin (5X). Feeding difficulties (20X), vomiting (18X), and failure to thrive (16X) were leading symptoms. The most frequent clinical findings were hepatomegaly (18X), pallor (14X), haemorrhages (13X). Ascites, oliguria, tachypnoea, fever, splenomegaly and rickets were less frequent. Laboratory findings were indicative of disturbed hepatic and renal tubular function and also of disturbed intermediary metabolism (hypokaliaemia, hypophosphataemia). However, hypoglycaemia was found in only 4 out of 15 patients tested. Differential diagnosis after hospital admission centered on metabolic disorders such as glycogenoses, galactosaemia, tyrosinosis, or Wilson's disease. Hepatitis, toxic hepatosis, liver tumour, intrauterine infection and sepsis were also considered. Eleven children had first ingested fructose within the first 6 weeks of life. The diagnosis was usually established only many weeks or months after first fructose intake and appearance of symptoms. This documents how difficult the diagnosis of this disease can be both in practice and in hospital. The course was severe in 11 children and lethal in 4. In only 5 patients was the course mild. The 16 survivors are doing well under fructose-exclusion diet. Irreversible visual impairment after intraocular haemorrhage occurred once. In each case HFI could have been suspected immediately, had a detailed nutritional history been taken. Practising paediatricians should know the composition of commonly used infant formulae. They should never prescribe sugared condensed milk for intractable vomiting prior to excluding HFI. Solution for intravenous infusion containing fructose and sorbitol are life-threatening for undiagnosed HFI patients.
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PMID:Hereditary fructose intolerance in early childhood: a major diagnostic challenge. Survey of 20 symptomatic cases. 73

To focus attention on the problem of infant mortality in Lebanon, data were compiled on infant mortality from 1978 to 1986 at the American University of Beirut Medical Center. Causes of death are analyzed for 602 males and 398 females. 54.9% deaths occurred at 1 month of age and 77.4% died within the 1st year. Autopsies were performed on .7%. 37.7% of all neonatal deaths were due to neonatal diseases such as hyaline membrane disease, asphyxia neonatorum, immaturity, necrotizing enterocolitis, hemorrhage, hemolysis, meconium aspiration, and kernicterus. Better prenatal care would reduce this group, or the administration of corticosteroids to the mother 24-48 hours prior to delivery, as well as rapid resuscitation at birth and prevention of the 5 curses: hypoxemia, hypoglycemia, hypothermia, hypotension, and acidosis. Although unavailable in Lebanon, administration of surfactants through an endotracheal tube would also help. Infections constitute 25.1% of deaths; many are preventable through adequate public health measures and strict personal hygiene, i.e., diseases such as sepsis, pneumonia, meningitis, gastroenteritis, hepatitis, encephalitis, and 1-2 cases of the following: diphtheria, measles, peritonitis, tetanus, tuberculosis, cytomegalis inclusion, herpes, parathyphoid, pertussis, poliomyelitis, and shigellosis. Congenital diseases were 21.6%. In utero diagnosis could prevent some diseases and in utero treatment is possible for hydrocephalus and hydronephrosis. Screening programs postnatally could lead to treatment. 5.9% were malignancies such as leukemia, lymphoma, brain tumors, histocytosis, Wilm's tumor, Ewing sarcoma, and Hodgkin's disease. Early diagnosis is critical if mortality is to be reduced in this group, but medical advances are still needed. 2.9% are miscellaneous diseases such as poisoning, rheumatic diseases, marasmus, Reye's syndrome, nephrosis, rickets, and epilepsy. Most of these diseases are preventable, except for rheumatic inflammation of the heart. Recommended necessary steps to reduce infant mortality are: prenatal care, diagnosis and screening, intrauterine surgery; resuscitation and intensive care centers with modern equipment and trained personnel; national vaccination and screening programs; adequate public health measures and hygiene; parental education; and well-equipped hospitals to serve all regardless of income level.
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PMID:Pediatric mortality: an avoidable tragedy. 251 28

The history of maternal deaths in England from the earliest records in the 1700s to 1935, concentrating on the influence of medical practice, is recounted. The rate lay between 4 and 5 per 1000 until 1935, with the advent of sulfa antibiotics to prevent puerperal infections. The practice of midwifery by men began in the early 17th century in Britain, but attendance at normal labors by medical practitioners, that is, surgeon-apothecaries, did not become common, and then only in urban areas, until 1730. The use of forceps became widely known about that time, and lying-in hospitals were begun. Obstetrics was held in contempt by professionally educated and registered physicians and apothecaries, however, because of the immodesty and messiness of the work and the long hours involved. Estimates of maternal mortality, from the 1st recorded unselected series, in the late 18th century range from 5-29/1000. Some of the high figures are from specialists in obstetrics, who treated complicated cases. From these data the maternal death rate was estimated at about 25/1000 among unassisted women. Some institutions achieved results better than the national average in the 1920s, suggesting that by the end of the 18th century, a fairly good understanding of childbirth had been reached. At that time the overall forceps rate was conservative, less than 1% compared to 15% now. Use of the perforator, hook and crochet, and manual dilatation of the cervix had been abandoned. In the 19th century, lying-in hospitals became more common and their death rates were higher, probably due to less conservative methods, up to as high as 85/1000, until the advent of antisepsis in 1880. Nevertheless, hospital births were the minority, amounting to 15% in 1927, 54% in 1946, 87% in 1970, 98.8% in 1980. Sepsis, due to casual use of sterile technique, remained the cause of half the total deaths until 1937. It is difficult to assess the contribution of toxemia or obstructed labor in maternal deaths. Rickets was a common cause of obstructed labor, and there are recorded epidemics of both. Similarly, abortion-related deaths are even more difficult to estimate, because of poor reporting. In evaluating the undiminished maternal death rate before 1935, the author believes that maternal survival is remarkably resistant to the ill effects of socioeconomic deprivation, but is very sensitive to the good and bad effects of medical intervention. Hence, there is evidence that the rural and poor in some cases had better results that those given the best medical assistance, especially with regard to puerperal sepsis. The midwifery laws of 1902 provided for training of midwives, and slowly corrected quality of care, as well as hostility between midwives and physicians. The current maternal death rate is about 0.1/1000.
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PMID:Deaths in childbed from the eighteenth century to 1935. 351 35

One hundred twenty-five infants underwent surgical intervention for necrotizing enterocolitis between 1972 and 1984. Sixty-three infants, who survived more than 30 days postoperatively, were evaluated for long-term complications. There were 28 girls and 35 boys (mean birth weight 1,725 +/- 890 g; gestational age 32 +/- 4 weeks). Associated problems included hyaline membrane disease (43), cardiac anomalies (25), and trisomy 21(2). Thirty-six survivors required long-term ventilatory support. Fifty-nine infants underwent bowel resection and enterostomy, 3 decompressing enterostomies without resection, and 1, exploratory laparotomy only. Enterostomies were closed at four months. Twenty four had short bowel syndrome. Fifteen infants subsequently died for a late mortality rate of 23%. Mortality was related to sepsis (3), respiratory failure (5), cardiac anomalies (3), cardio-respiratory arrest (2), and TPN related liver failure (2), and was common with gestational age less than 31 weeks and birth weight less than 1,000 g. Medical problems included cholestasis (17), TPN induced cirrhosis (3), meningitis (3), seizures (8), and nutritional rickets (6). Significant developmental and intellectual delays were observed.
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PMID:Long-term follow-up after surgical management of necrotizing enterocolitis: sixty-three cases. 372 6

The effects of prolonged home total parenteral nutrition on growth and psychomotor development were studied in eight infants during a two-year period. Each patient was begun on HTPN in the first 60 days of life, using a Broviac silastic catheter for venous access. Normalization of somatic growth has been observed in all patients during the study period. Six of eight patients have normal psychomotor development following discharge from the hospital into the home environment. Catheter-related complications in this group have been infrequent; one episode of sepsis was documented during 121 patient-months. The major metabolic complication observed was the development of rickets despite provision of recommended doses of vitamin D. HTPN appears to be useful in providing nutritional support for selected infants during the first two years of life.
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PMID:Home parenteral nutrition in infants. 676 67

Six infants with necrotizing enterocolitis were discharged after periods of prolonged hospitalization (two to nine months) with intact ileostomies. Their initial hospitalization was complicated by feeding difficulties, chronic diarrhea, sepsis, rickets, and developmental delay. All were rehospitalized within three months, with severe acidosis and dehydration after a presumed viral-type illness. Each had large-volume ileostomy output, which was rich in electrolytes and bicarbonate. A prolonged recovery phase (two to eight months) again was punctuated with episodes of diarrhea, problems in starting oral feeding, and sepsis. After reanastomosis of the remaining bowel, no infant has had a similar life-threatening episode. It is speculated that the infants' recurrent "salt-and-water-losing states" are secondary to either an anatomic or functional loss of the colon. This problem appears to be a poorly recognized sequela of bowel surgery and necrotizing enterocolitis, and early reanastomosis of discontinuous bowel should be of benefit.
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PMID:Importance of early ileostomy closure to prevent chronic salt and water losses after necrotizing enterocolitis. 709 91

Renal transplantation for infantile cystinosis corrects renal failure and prolongs survival. However, after transplantation, the disease may develop in the allograft and continue to progress in nonrenal organs. We studied seven children (6 boys, 1 girl) with infantile cystinosis who received 11 renal transplants (3 cadaver, 8 living-related) between May 1969 and December 1986. The age at transplant ranged from 6 to 12 years (mean, 9.1 years). Four children received second renal transplants at a mean age of 17 years (range, 16 to 22 years). The mean period of follow-up was 138 +/- 47 months. Three children received cysteamine therapy prior to transplantation. Nonrenal complications of infantile cystinosis present before transplantation were photophobia (n = 3), corneal crystals (n = 5), hypothyroidism (n = 1), rickets (n = 6), and short stature (n = 7). Graft and patient survival did not differ from controls matched for the time of transplantation. Two patients died (1 pneumococcal sepsis, 1 respiratory failure due to pulmonary fibrosis) with functioning grafts 5 and 14 years posttransplant. Complications that developed posttransplant included photophobia (n = 1), corneal crystals (n = 2), hypothyroidism (n = 4), polyneuropathy (n = 1), pulmonary fibrosis (n = 1), abnormal electroencephalogram without clinical seizures (n = 1), bladder stones (n = 1), and diabetes mellitus (n = 2). One patient received a corneal transplant. All seven children failed to show improvement in growth following transplantation. Cystine crystals are present in graft-infiltrating cells, but do not seem to affect kidney allograft function.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Renal transplantation for infantile cystinosis: long-term follow-up. 843 88

The purpose of this paper is to review clinical studies on hypophosphatemia in pediatric intensive care unit patients with a view to verifying prevalence and risk factors associated with this disorder. We searched the computerized bibliographic databases Medline, Embase, Cochrane Library, and LILACS to identify eligible studies. Search terms included critically ill, pediatric intensive care, trauma, sepsis, infectious diseases, malnutrition, inflammatory response, surgery, starvation, respiratory failure, diuretic, steroid, antiacid therapy, mechanical ventilation. The search period covered those clinical trials published from January 1990 to January 2004. Studies concerning endocrinological disorders, genetic syndromes, rickets, renal diseases, anorexia nervosa, alcohol abuse, and prematurity were not included in this review. Out of 27 studies retrieved, only 8 involved pediatric patients, and most of these were case reports. One clinical trial and one retrospective study were identified. The prevalence of hypophosphatemia exceeded 50%. The commonly associated factors in most patients with hypophosphatemia were refeeding syndrome, malnutrition, sepsis, trauma, and diuretic and steroid therapy. Given the high prevalence, clinical manifestations, and multiple risk factors, the early identification of this disorder in critically ill children is crucial for adequate replacement therapy and also to avoid complications.
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PMID:Hypophosphatemia in critically ill children. 1554 5

The purpose of this case report is to illustrate the diagnostic difficulties of congestive heart failure in an infant. When presenting to the emergency department, these patients are often evaluated for sepsis, congenital heart disease, metabolic disorders, and myocarditis. We report a case of a 3(1/2)-month-old male who presented to the pediatric emergency department with congestive heart failure. He was found to have vitamin D deficiency rickets induced cardiomyopathy.
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PMID:An infant with tachypnea. 1711 Aug 66

Coxa vara is an abnormality of the proximal femur with a decreased neck-shaft angle and resulting leg-length discrepancy, has been associated with conditions such as congenital coxa vara, traumatic injury, sepsis, rickets, vascular damage, or metabolic disorders; however its possible relationship with extracorporeal membrane oxygenation has been recently reported.A full term girl was born with a total infradiaphragmatic anomalous pulmonary venous drainage, at the age of 12 days, an anastomosis of the pulmonary venous trunk with the left auricle and closure of the auricular septal defect was performed; during this procedure extracorporeal membrane oxygenation was used during 104 minutes, no neonatal sepsis was developed. She had no orthopedic issues until she was 3 years and 10 months old, when she presented with limp related to the right lower limb, with no pain. She had a leg-length discrepancy of 2 cm (right - left), limited right hip abduction to 25 degrees , and internal rotation to 5 degrees , also had a positive Trendelenburg test. No flexion/extension abnormalities. Anteroposterior radiographs and magnetic resonance revealed coxa vara with proximal femoral growth arrest. A valgus osteotomy with greater trochanteric epiphysiodesis was performed. At the eight month follow-up, she had no hip pain, better hip range of motion, no difficulties with recreational activities and the osteotomy healed.Another four similar cases had been reported with similar outcome, we think that it will be recommendable to take images in patients with this background and limb leg-length discrepancy or abnormal range of motion.
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PMID:Coxa vara with proximal femoral growth arrest as a possible consequence of extracorporeal membrane oxygenation: a case report. 1991 57


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