Adenovirus 7a: a community-acquired outbreak in a children's hospital

BACKGROUND: Adenoviruses produce many illnesses in children, particularly respiratory and gastrointestinal disease. The most common adenoviral respiratory infections in children are caused by types 1, 2, 3 and 5. Adenoviruses spread rapidly in closed environments often causing epidemic disease. Serotype 7a has been responsible for outbreaks of respiratory disease in children living in close proximity with one another. This report describes a large community-acquired adenovirus 7a epidemic in hospitalized children. METHODS: Evaluation of all patients with cultures positive for adenovirus from a children's hospital-based virology laboratory during a recognized adenovirus outbreak. All such adenovirus isolates were typed, and patients with adenovirus 7a are described by review of medical records. RESULTS: Between March 1 and July 26, 1997, 47 children admitted to the hospital were identified as infected with adenovirus. Of these 47 patients 26 (55%) were infected with adenovirus 7a. Twenty-four (92%) infections were community-acquired. The age range was 11 days to 10 years with a median of 9.5 months. Twenty-two patients (84%) had respiratory symptoms, and 21 (8%) had fever, making these the most common symptoms. The mean durations of fever and hospitalization were 5.5 and 7 days, respectively. One of 26 patients died. CONCLUSIONS: Adenovirus 7a can cause large community epidemics affecting children. The disease produced by adenovirus 7a in children is almost exclusively of the respiratory tract, and in some individuals it may be very severe and possibly fatal.     

Leukemia in a Child with a History of Medulloblastoma

Leukemia of mixed lineage, was diagnosed in a 6.5-year-old boy with a history of medulloblastoma, 38 months after his initial cancer diagnosis. Therapy had included craniospinal radiation and nitrosourea-based chemotherapy. In addition, onset of leukemia was preceded by therapy with recombinant growth hormone for short stature. Although rare, leukemia is a treatment-related complication for patients with past brain tumors whose follow-up should therefore include surveillance with complete blood counts.     

Repair of a canalicular laceration in a neonate

A neonate with lower eyelid and corresponding canalicular laceration of the left eye was examined 2 hours after birth. The eyelid and canalicular laceration were repaired with a short, 24-gauge catheter as the stent. The laceration healed with minimal scarring, and the epiphora had disappeared by the 12-month follow-up visit.     

Calcification in a Deflux bleb thought to be a ureteral calculus in a child

A child admitted for abdominal pain was evaluated with an abdominal computed tomography scan. Calcification was noted at the ureterovesical junction. The child had undergone successful bilateral Deflux injection 3 years earlier. No hydronephrosis was noted and an intravenous pyelogram was normal. Ureteroscopy revealed no intraluminal stones, and a follow-up scan showed the lesion to be still present and unchanged. This case illustrates that calcifications within a Deflux bleb can be mistaken for a ureteral calculus, and the entire clinical picture should be kept in mind when evaluating a patient with abdominal pain. Due to the widespread use of Deflux to treat vesicoureteral reflux, this phenomenon may be seen more frequently as this population ages.     

Life-threatening haemorrhage as a complication of a congenital haemangioma

Life-threatening complications of congenital haemangiomas are rare. A case of haemorrhage in a neonate is reported here. A neonate presented a congenital haemangioma of the limb complicated by a massive life-threatening haemorrhage. Colour ultrasonography showed a hypervascularized, highly haemodynamically active mass with a large, superficial drainage vein just under the ulceration. Surgical resection was performed with satisfactory postoperative outcome and no evidence of recurrence. CONCLUSION: Early surgical removal can be a good therapeutic option for complicated congenital haemangiomas.     

Epilepsy with a de novo missense mutation in the sodium channel a1 subunit: a case report

Most epilepsies are characterized as "idiopathic" because of the lack of a known cause. Nevertheless, recently, there has been significant progress in the molecular genetics of idiopathic epilepsy. Mutations in gene-encoding ion channels were found to be the underlying disorder in all idiopathic epilepsies with a known molecular basis. Missense mutations in the voltage-gated sodium channel a1 subunit gene (SCN1A) were firstly identified in patients with generalized epilepsy with febrile seizures plus additional symptoms (GEFS + ). Subsequently, mutations of SCN1A were also found in patients with severe myoclonic epilepsy of infancy (SMEI) or Dravet syndrome, and in patients with borderline SMEI (SMEB), a milder form of Dravet syndrome. We describe a case of a new missense de novo mutation of SCN1A in a child with the clinical features of borderline SMEI syndrome.     

Colocolonic intussusception in a four-yr-old with a heart transplant: a case report and review of the literature

A rare case of a colocolonic intussusception in a pediatric cardiac transplant patient is reported along with a review of colocolonic intussusception in pediatric patients after organ transplantation. A four-yr-old girl with a history of heart transplantation shortly after birth presented with a 12-hour history of abdominal pain, vomiting, and bloody stools. Initial abdominal x-ray showed rectal edema. A rectal examination revealed a palpable intussusceptum that prompted a therapeutic contrast enema, which failed to reduce the intussusception. Surgical exploration was performed with reduction of the intussusception and resection of the terminal ileum and cecum. A pathologic lead point was not identified.     

Donor transmission of pineoblastoma in a two-yr-old male recipient of a multivisceral transplant: a case report

Transplant-transmitted malignances are rare but devastating events. Primary brain tumors are the least common among reported donor-derived malignancies. We report a case of donor-transmitted pineoblastoma, a PNET, in a two-yr-old male recipient, who presented with a rapidly growing mass in the right mandible, four months after multiple visceral organ transplantation. The recipient had liver, pancreas, and small bowel transplants because of end-stage liver failure and short gut syndrome, which was secondary to large bowel resection for management of gastroschisis complicated by intestinal volvulus. The donor autopsy results became available seven wk after transplantation, which found a pineoblastoma with meningeal spread. Evaluation of eyes, adrenal glands, bone marrow, and other organs did not identify metastasis outside the CNS. A biopsy of the recipient's right mandibular mass revealed a malignant small round blue cell tumor with the immunohistochemistry profile of a PNET. Staging evaluation revealed the tumor in the right mandible with bone marrow involvement. Further investigation showed that recipient's tumor and donor's pineoblastoma shared the same immunophenotype and HLA type, suggesting the recipient's tumor is a donor-transmitted pineoblastoma. This is the first case report of donor-transmitted pineoblastoma post-organ transplant.     

Embolization of a coronary fistula with a controlled delivery platinum coil in a 2-year-old

Summary Congenital coronary artery disorders are rare. Elective closure is proposed in view of the morbidity reported later in life. Excellent results have been reported with surgery, and recently catheter occlusions have been successful. We report the case of a 2-year-old girl whose coronary artery fistula was occluded with a special controlled delivery coil system.     

Time for a cool head-neuroprotection becomes a reality

Studies in encephalopathic infants have demonstrated a brief phase of normal cerebral energetics following hypoxia-ischaemia prior to development of delayed energy failure. In experimental models, mild hypothermia has shown a consistent neuroprotective action, although its efficacy is critically dependent on the severity of the primary insult, the delay in initiating cooling, and the duration and depth of hypothermia. Early electroencephalographic assessment of encephalopathic infants has the potential to provide objective information about the preceding insult, aiding the selection of infants for enrollment to clinical trials. Preliminary results from a large randomised trial of selective head cooling suggest that early intervention can lead to significantly improved outcome in a subgroup of encephalopathic infants with intermediate electroencephalographic abnormalities. Further research in established experimental models is essential to improve the identification of suitable infants for treatment, to investigate the importance of variations in regional brain temperature, and to examine the therapeutic potential of hypothermia combined with other neuroprotective agents.     

Fathers' Perspectives on Parenting a Child With a Craniofacial Anomaly

IntroductionAlthough research on healthy children indicates that fathers exert specific and beneficial influences on children's development, there is little research on fathers of children with chronic medical challenges, and none on fathers of children with craniofacial anomalies (CFA). This exploratory study aimed to obtain preliminary information about fathers' experiences of parenting a child with CFA.MethodStructured telephone interviews were conducted with a volunteer sample of nine fathers of children with CFA. Interviews were summarized using percentages of responses for questions using a rating-scale format, and with excerpts of narratives from open-ended questions.ResultsMost fathers (90%) felt highly optimistic about their children's potential for happiness and life success, although a smaller percentage also expressed worry (21% a lot; 33% some) and concern (11% a lot; 44% some). All fathers described positive attributes in their children. Fathers tended to support their children's peer relationships through encouraging and organizing activities with friends.DiscussionFathers' focus on children's effective engagement in the world is consistent with findings on fathering of healthy children. Findings provide hypotheses for future research on fathers' role in the positive development of children with CFA.     

Bacterial Endocarditis in a Child with a Broviac Catheter

Broviac and Hickman catheters facilitate the care of children with cancer but provide a source of potential infection. We describe a child with a Broviac catheter who developed left-sided bacterial endocarditis in whom right-to-left atrial shunting was documented following catheter flushing. Following removal of the catheter and administration of prolonged intravenous antibiotics, recovery was complete and cardiac function returned to normal.     

Laparoscopic excision of a prostatic utricle in a child

Prostatic utricle cysts result from incomplete regression of Mullerian duct structures and occur most frequently in males with perineal or peno-scrotal hypospadias. Utricular cysts may present with various signs and symptoms including urinary tract infection, pain and post-void incontinence, a palpable abdominal mass or recurrent epididymitis. Treatment is reserved for symptomatic cysts and various techniques have been described including transurethral deroofing, endoscopic incision or surgical excision by suprapubic, posterior and midline transvesical approaches. We present a successful minimally invasive approach for excision of a prostatic utricle cyst in a child. Laparoscopic excision is a safe and viable alternative to open procedures in the surgical treatment of symptomatic utricle cysts in childhood. The presence of a cystoscope within the utricle orifice aids identification and safe dissection of the utricular remnant.     

Hyponatremia secondary to reset osmostat in a child with a central nervous system midline defect and a chromosomal abnormality

A newborn with a CNS midline defect and persistent hyponatremia was diagnosed with a "reset" osmostat using a 3% hypertonic saline test. The diagnosis was established by measuring urinary arginine vasopressin (UAVP) and plasma osmolality (P(Osmoil)). In this infant a chromosome abnormality with the karyotype 46, X, -X, +der(X) t(X;13) (p22.1;q22) was associated with the midline defect and a reset osmostat.     

Synchronous occurrence of a hemorrhagic hypothalamic hamartoma and a suprasellar teratoma

Hypothalamic hamartomas have been reported to coexist with lesions like Rathke's cleft cyst and arachnoid cysts in the suprasellar or temporo-sylvian regions. This is the first report in indexed literature describing its association with a suprasellar teratoma. A 7-year-old girl presented with long-standing precocious puberty and generalized tonic-clonic seizures and recent-onset raised intracranial pressure. MRI done prior to the onset of symptomatic raised intracranial pressure revealed 2 distinct lesions in the suprasellar region. One was a midline, pedunculated lesion arising from the hypothalamus, with evidence of an old bleed within it. A separate lesion, with a wide base near the tuberculum sellae and a posteriorly directed conical tip, was noted in an adjacent sagittal cut. CT scan done at the time of admission demonstrated a re-bleed in the suprasellar region with blood in the lateral and third ventricles and gross hydrocephalus. The child was taken up for a ventriculoperitoneal shunt followed by complete excision of the lesions. Histopathologic examination confirmed the pedunculated lesion to be a hypothalamic hamartoma with evidence of hemorrhage, and the other to be a mature teratoma. Postoperative MRI confirmed complete excision of both the lesions. The child reported regression of precocious puberty and remained seizure-free until the last follow-up 6 months after surgery. A hypothesis based on a dysontogenetic mechanism is discussed to explain the unusual occurrence of the dual, seemingly unrelated pathologies. Hemorrhage into the hamartoma was an added oddity in this case.     

Regression of a cardiac rhabdomyoma in a patient receiving everolimus

Tuberous sclerosis complex (TSC) is an autosomal dominant neurocutaneous disorder that can affect every organ of the body, most commonly the brain, kidneys, heart, and lungs. The TSC mutation results in abnormal cellular proliferation and differentiation, which are responsible for hamartomatous lesions that affect the brain, kidney, heart, and lungs. mTOR (mammalian target of rapamycin) is a protein kinase that regulates the abnormal cellular proliferation and differentiation. Consequently, mTOR inhibitors are being studied to treat the subependymal giant-cell astrocytomas and renal angiomyolipomas that are commonly seen with TSC. We describe here the case of a patient with significant regression of a cardiac rhabdomyoma after receiving everolimus, an mTOR inhibitor. This finding suggests a possible novel therapy for patients with clinically significant cardiac rhabdomyomas.     

Primary observation of a congenital intracranial teratoma in a twin with a healthy brother

Primary intracranial teratomas are rare germ cell tumors arising from embryonic tissues, which contain cellular or tissue derivates from all three germ layers. Today the early intrauterine diagnosis by ultrasound is possible. We first describe a patient with such a tumor which was diagnosed by routine ultrasound in the 33rd week of gestational age in a twin-pregnancy, of which the 2nd twin developed normally. This must be considered in the obstetrical situation. In such a case the parents should be advised accordingly.     

Aortic pseudoaneurysm in a child with a left mainstem bronchial stent

OBJECTIVE: To report a case of aortic pseudoaneurysm in a child with a metallic bronchial stent. DESIGN: Case report and literature review. SETTING: Pediatric intensive care unit at a freestanding tertiary children's hospital. PATIENT: A 12-yr-old boy with a left mainstem bronchomalacia managed with a metallic stent presenting with bleeding from his tracheostomy. INTERVENTIONS: Emergent flexible tracheobronchoscopy, computed tomography angiogram. MEASUREMENTS AND MAIN RESULTS: Discovery of a pseudoaneurysm of the descending thoracic aorta adjacent to the bronchial stent. CONCLUSIONS: We report the first case of an aortic pseudoaneurysm in association with a bronchial stent in a child.     

Ureterocystoplasty in a patient with a single kidney

Ureterocystoplasty (UCP) has now been widely used for bladder augmentation, with and without unilateral nephrectomy. Many techniques have been described to incorporate portions of the upper renal tract, but none have yet described UCP in a child with unilateral renal agenesis. Accepted: 12 August 1998     

Takotsubo-Like Cardiomyopathy in a 17-Year-Old Male with a Pheochromocytoma

We report a case of a 17-year-old adolescent male with Takotsubo-like cardiomyopathy in the setting of pheochromocytoma who presented with hematemesis, hypertension, and pallor. Takotsubo-like cardiomyopathy is rarely reported in the pediatric population, and this is the first report in the pediatric literature of Takotsubo-like cardiomyopathy associated with both pheochromocytoma and an elongated course of the left anterior descending coronary artery.