Oral contraceptives: a cause of hyperbilirubinemia in stem cell transplant patients.

Conjugated hyperbilirubinemia in the clinical setting of hematopoietic stem cell transplantation can have multiple etiologies that may prompt various therapeutic interventions. Two patients who received short courses of a high-dose estrogen-progesterone combination to treat breakthrough menstrual bleeding during transplant are reported. Conjugated hyperbilirubinemia developed in both patients within days of beginning therapy and resolved after the ethinyl estradiol and norgestrel (Ovral; Pharmacia and Upjohn, Kalamazoo, MI, U.S.A.) was discontinued. In one of the patients, this occurred on three separate occasions during the course of transplantation. Recognizing the cholestatic effect of estrogens during transplantation may prevent unnecessary alterations in therapy beyond the simple discontinuation of these medications.     

Oral hypertonic glucose spray: a practical alternative for analgesia in the newborn

AIM: Pain and stress have been shown to induce significant physiological and behavioural reactions in newborn infants. Pharmacological agents are not recommended in neonates for pain relief in minor procedures. Since different sweet solutions given orally by syringe have been shown to relieve pain in neonates, we decided to compare the analgesic effects of a small dose of glucose solution given orally by spray and by syringe during heel lancing in term neonates, using a validated behavioural acute pain rating scale. METHODS: Sixty hyperbilirubinaemic full-term neonates were studied. We used a randomized, masked, placebo-controlled, crossover trial. Each infant was assessed three times receiving 0.5 ml 30% glucose in spray form, 0.5 ml 30% glucose by syringe or 0.5 ml sterile water by syringe in random order, 2 min before heel lancing. RESULTS: Pain scores were significantly lower in the 30% glucose given either spray or syringe groups compared with the placebo group. No statistically significant difference in pain scores was found between the 30% glucose spray group and 30% glucose syringe group. CONCLUSIONS: A small dose of 0.5 ml 30% glucose spray has an equal analgesic effect to the same dose given by syringe. The spray form has the advantage of being easy to use and is well accepted by newborn babies.     

Oral contraceptives and breastfeeding: haematological effects on the infant.

Severe clinical and haematological manifestations of folate deficiency occurred in a previously healthy, fully breast fed, 10 month old infant whose mother took oral contraceptives.     

Oral agents in managing diabetes mellitus in children and adolescents

Type 2 diabetes mellitus is a chronic disease with potentially devastating long-term complications. Despite the tremendous body of research and experience in the adult population, relatively little is established regarding this condition and its optimal management in children and adolescents. The pediatric community awaits results of ongoing trials as well as further study of optimal intervention in children, as they continue to extrapolate management practices from their adult counterparts.     

Fluticasone propionate aqueous nasal spray: a well-tolerated and effective treatment for children with perennial rhinitis

A total of 535 children aged 4–11 years with perennial rhinitis were recruited to two double-blind studies performed at 56 centres in eight European countries, Israel, and South Africa. One study compared the efficacy and tolerability of fluticasone propionate aqueous nasal spray (FPANS), at either 100 ug once daily (od) or 100 ug twice daily (bd), with beclometha-sone dipropionate (BDPANS) 200 ug bd for 12 weeks in 120 children aged 6–11 years. The second study compared FPANS 100 ug od with FPANS 200 ug od and placebo for 4 weeks in 415 children aged 4–11 years. Efficacy was determined by means of patient assessments of nasal symptoms of perennial rhinitis and by investigator assessments of symptoms and nasal condition. The symptoms of nasal blockage, on waking and during the day, sneezing, rhinorrhoea, and nasal itching were assessed by the investigator at clinic visits and by the completion of a patient daily diary card. Safety was assessed by collection of adverse event information, routine haematology and biochemistry testing, and monitoring plasma cortisol levels. FPANS demonstrated good control of symptoms at each dose regimen, which was equivalent or superior to BDPANS and superior to placebo. There was no difference between the dose regimens of FPANS. FPANS was as well tolerated as placebo and BDPANS. It is concluded that FPANS 100 ug od is an effective and well-tolerated treatment for perennial rhinitis in children aged 4–11 years.     

Central diabetes insipidus in children. V. Oral treatment with a vasopressin hormone analogue (DDAVP)

1-deamino-8-D-arginine-vasopressin, or DDAVP, a potent long acting antidiuretic analogue of AVP, is the treatment of choice in central diabetes insipidus (DI). We have studied the clinical and biological effect in 10 children with DI treated with peroral administration of DDAVP. During a dose ranging study in hospital, followed by 6 months of treatment at home, this peroral DDAVP tablet proved to be as effective as the intranasal administration of DDAVP and was preferred by the patients. Even doses as small as 12.5 micrograms, have an effect on diuresis and urinary osmolality. Therapeutic effects start at above 100 micrograms. During the dose ranging study 200 micrograms peroral DDAVP produced antidiuresis varying from 8 to 12.5 hours, in different patients. The recommended dose is 100-300 micrograms 2-3 times a day. This treatment offers an important alternative to that traditionally used and constitutes one of the first examples of a peptide conserving its biological activity after gastrointestinal transport.     

Sumatriptan nasal spray in the acute treatment of migraine in adolescents and children

About 4-10% of children and adolescents suffer from migraine. In the last few years, several studies have been performed to assess the efficacy and safety of triptans for the acute treatment of migraine in children and adolescents. Only sumatriptan nasal spray has been approved for the treatment of acute migraine with or without aura in adolescents aged 12-17 years in Europe. This review describes the results of the studies with sumatriptan nasal spray that have been performed in children and adolescents, including a study performed in the Netherlands.     

The autopsy in pediatrics and pediatric oncology: a single-institution experience.

Autopsy rates and clinicopathologic correlations for pediatric autopsies and the subgroup of pediatric oncology autopsies in a large teaching hospital were studied to evaluate the utility of autopsy in these populations. Autopsy records of the University of North Carolina hospitals from 1982 to 2001 were reviewed for all patients less than 18 years of age. Autopsies performed during 1982 to 1991 (decade 1) were compared to those from 1992 to 2001 (decade 2) with respect to absolute numbers of autopsy and rates of unexpected postmortem diagnoses. Postmortem diagnostic discrepancies were subclassified into major and minor categories. The mean number of autopsies per year for decade 1 was 110+/-24.5, compared with 77.5+/-40.9 for decade 2 (P<0.001), a change largely due to a decline in fetal/perinatal (patients<7 days of age) autopsies. Of 533 pediatric autopsies, 43 were in patients with a primary diagnosis of a neoplasia. At least one antemortem misdiagnosis and/or clinically occult process was identified in 20.5% of all pediatric autopsy cases, and in 25.6% of pediatric oncology cases. These rates did not change significantly over time. In 10 of 43 pediatric oncology cases (23.3%), autopsies provided diagnostic information that was previously unknown to the clinicians. Three of these patients died shortly after presentation. Although autopsy rates for fetal/perinatal cases have declined, those for non-perinatal and pediatric oncology patients remain stable. Autopsy continues to provide diagnostic information that is unknown during the antemortem period, and remains a valuable tool for pediatricians and pediatric oncologists.     

Clinical management of infantile fibrosarcoma: a retrospective single-institution review

Background

Infantile fibrosarcoma (IFS) is an uncommon soft-tissue sarcoma. Here we review our experience treating this tumor.

Patients and methods

We retrospectively reviewed records of patients with IFS treated at St. Jude Children’s Research Hospital between 1980 and 2009.

Results

We identified 15 patients, 8 girls and 7 boys; 13 white and 2 black. Median age at diagnosis was 3 months. Primary sites included the leg (n = 3), chest wall (n = 2), foot (n = 2), and one each in the tongue, occipital region, axilla, parascapular region, arm, forearm, retroperitoneum, and thigh. All patients underwent resection; 11 upfront surgery, and 4 delayed. Complications included loss of the posterior tibial nerve and artery, axillary vein, biceps, pectoralis major, gallbladder, and transverse/sigmoid sinus. Eight received chemotherapy and three radiotherapy. Seven experienced local recurrence and three lung metastasis. Median follow-up was 65 months. At the time of the review, 12 patients were alive and 3 had died. All deaths were in patients older than 1 year at diagnosis with an axial primary site.

Conclusions

Non-mutilating surgery should be the primary treatment for IFS. Neoadjuvant chemotherapy is indicated when upfront resection is unfeasible. Patients with positive surgical margins should receive adjuvant chemotherapy. Radiotherapy is indicated for axial primary sites where complete resection is impossible.     

High-flow nasal cannula use for bronchiolitis treatment in French intensive care units: A transversal study

Over the past 10 years, continuous positive airway pressure (CPAP) has revolutionized the prognosis and management of bronchiolitis patients hospitalized in pediatric intensive care units (PICUs). High-flow nasal cannula (HFNC) is emerging as an alternative to CPAP. Despite encouraging results of several clinical and physiological studies, HFNC use remains controversial and its indications heterogeneous. To better define the place of HFNC in severe bronchiolitis respiratory support, we investigated the different ventilation assistance techniques used for severe bronchiolitis over 3 days at the peak of a bronchiolitis epidemic in December 2015. We conducted an observational cross-sectional study in 27 French university hospital PICUs. Fifty-nine patients were included. The results show that HFNC already accounts for nearly half of the respiratory support techniques used for severe bronchiolitis in French PICUs with no significant difference between the CPAP group and the HFNC group of patients.     

The changing incidence of acute pancreatitis in children: a single-institution perspective

All cases of pancreatitis examined between 1993 and 1998 were reviewed to determine the etiologic factors and the number of cases per year. Although the incidence increased, the spectrum of etiologic factors did not differ greatly from those in previous series of pancreatitis in children. Pancreatitis in infants was associated with multisystem illness. Idiopathic pancreatitis was uncommon in children younger than 6 years of age and not observed in children younger than 3 years of age.     

Oral and nasal enteral tube placement errors and complications in a pediatric intensive care unit.

OBJECTIVE: To report five cases of errors in the placement of oral/nasal enteral tubes in a pediatric intensive care unit, and to review literature on placement techniques and complication rates. DESIGN: Case series and review of the literature. SETTING: A 19-bed pediatric intensive care unit in a tertiary care pediatric hospital. PATIENTS: A 14-yr-old male with respiratory distress following a near drowning, a 10-yr-old male with recurrent acute lymphocytic leukemia and Pneumocystis carinii pneumonia, a 16-yr-old female with complex congenital heart disease and respiratory failure, a 16-yr-old male with status asthmaticus, and a 2-yr-old male with congenital heart disease. INTERVENTIONS: None. MAIN RESULTS: Five cases of enteral tube placement errors occurred in our combined medical-surgical pediatric critical care unit within the past year. All five resulted in placement of the feeding tube in the respiratory tract, four occurred despite the presence of cuffed endotracheal tubes. Three of the five patients had subsequent worsening of their respiratory status. One developed a pneumothorax, one developed pulmonary hemorrhage, and one developed an increased oxygen requirement. CONCLUSIONS: Patients in the pediatric intensive care unit may have characteristics that place them at an increased risk for misplacement of oral or nasal enteral tubes into the respiratory tract. Placement of enteral tubes into the respiratory tract may cause serious morbidity and possibly mortality. Checking the placement of enteral tubes with traditional methods does not prevent misplacement in the respiratory tree, and new techniques should be considered.     

Malignant peripheral nerve sheath tumors in children: a single-institution twenty-year experience

A retrospective series of pediatric patients with localized malignant peripheral nerve sheath tumors (MPNST) treated during a 20-year period at one institution is reported. Between 1976 and 1996, 24 consecutive children were treated by a multimodality approach. Conservative surgery was the treatment of choice: primary radical surgery was performed in 10. Postoperative radiotherapy was administered in 12 and adjuvant chemotherapy in 19. Eight patients were alive without evidence of disease, six in first complete remission and two in second complete remission, after a median follow-up of 230 months. The 10-year event-free survival (EFS) and survival were 29% and 41%, respectively. Survival was 80% for the patients who underwent radical surgery, and 14% for the others; 71% for patients with tumors smaller than 5 cm, and 29% for those with tumors 5 cm or larger. Local recurrence was the major cause for treatment failure (13 of 17; 76%); the rate of local relapse was 33% v 75% in patients who either received or did not receive radiotherapy. Complete surgical excision remains the most effective treatment for MPNST and represents the main prognostic factor along with tumor size. Radiotherapy seems to play a role in achieving local control, whereas the role of chemotherapy is uncertain.