Brown bowel syndrome (intestinal lipofuscinosis) - a case report and review of the literature.

Brown bowel syndrome (BBS) is a very rare condition occurring in association with malabsorption syndromes. It is characterised by deposition of granular, brown pigment (lipofuscin or ceroid) in intestinal smooth muscles. Rarely BBS can be complicated by distention of any segment of the bowel. We present a case of BBS associated with massive dilation of first loop of the small intestine and moderate dilation of the sigmoid colon with functional intestinal disturbances requiring surgical intervention in an 11-year-old boy.

Pneumatosis cystoides interstitialis: A complication of graft-versus-host disease. A report of two cases.

BACKGROUND: Pneumatosis cystoides intestinalis (PCI) is a rare disorder characterized by the presence of multiple gas collections in the subserosal or submucosal intestinal wall of the large or small intestine. We report two cases of PCI in the course of chronic graft-versus-host disease. CASE REPORT: A 5-year-old girl was treated for acute lymphoblastic leukemia. Twenty-four months after the hematopoietic stem cell transplantation, in the course of graft-versus-host disease, she developed subcutaneous emphysema of the right inguinal and pudendal region. PCI was diagnosed based on a CT examination. A 3-year-old boy was treated for juvenile myelomonocytic leukemia. Fourteen months after the hematopoietic stem cell transplantation he presented with an increased severity of intestinal symptoms, including intermittent bleeding from large intestine. PCI was diagnosed based on a CT exam and was confirmed by a colonoscopy. CONCLUSIONS: Pneumatosis cystoides interstitialis in the course of chronic graft-versus-host disease has a heterogeneous clinical presentation that does not correlate with results of imaging. CT is a method of choice to diagnose PCI. In patients with PCI, the presence of free air in the peritoneal cavity does not confirm an intestinal perforation.

Bio-objects as "boundary crawlers:" the case of microRNAs.

microRNAs (miRNAs), short RNAs of 21-25 nucleotides, are implied in gene expression and regulation, in biological processes and in human pathologies including cancer. Since miRNAs of plant origin can survive digestion and cooking and enter in animal (including human) sera and tissues, their intervention in mammalian gene expression and regulation might be expected. Mouse experimental feeding, in fact, showed that a miRNA class (MIR168a) is involved in accumulation of the low-density lipoprotein (LDL), the major cholesterol-carrying lipoprotein of human plasma. Considering LDL's role in atherosclerosis, a negative influence of miRNAs from food origin on our health may be expected. Here we concentrate on the miRNAs' capability to cross inter-kingdom boundaries through the diet and acting as a "boundary crawler." The boundary between plant and human is presented under a new perspective, where a new intimate relationship between two genomes - mammalian and plant - belonging to quite different kingdoms is proposed. The food's role as molecule carrier in our health is also discussed. miRNAs, finally, are presented as an example of "bio-objects" with impact on both medical and cultural issues.

Causes and consequences of abandoning one-stage bilateral adrenalectomy recommended in primary pigmented nodular adrenocortical disease--case presentation.

We present a 7-year-old girl with a 2-year history of decelerated growth rate and cushingoidal obesity, upon admission presenting with fixed hypertension. Cyclic hypercortisolemia with inhibited baseline and post-CRH stimulation ACTH level pointed to primary adrenal hypercortisolemia. Ultrasound, computed tomography (CT) and magnetic resonance imaging (MRI) showed normal adrenal glands. 131J-labeled cholesterol scintiscan showed a weak but slightly more expressed tracer uptake in the left adrenal gland. Cushing syndrome concomitant with isolated primary pigmented nodular adrenocortical disease (PPNAD) was diagnosed. After hypotensive pretreatment, a left adrenalectomy was performed, resulting in normalization of corticoadrenal function, blood pressure, Cushing features and growth rate. Histopathology confirmed PPNAD. In the course of infection, corticoadrenal function showed absence of adrenal reserve, and adrenal crisis. Hydrocortisone (HC) therapy, followed by HC supplementation was introduced. Four years later, a contralateral adrenalectomy was performed and total HC supplementation was introduced. Causes and consequences of abandoning one-stage bilateral adrenalectomy recommended in PPNAD are reviewed.

[Iatrogenic pleuropneumonia complicating central venous cannulation in a very low birth weight infant]. / Jatrogenna pleuropneumonia jako powilkanie cewnikowania zyl centralnych u noworodka.

BACKGROUND: Central venous cannulation is necessary for long-term parenteral nutrition in premature infants. Peripherally inserted long catheters are commonly used in these patients but even this relatively simple technique can end in serious complications. We present a case in which perforation of the vena cava and migration of the catheter to the intrapleural space resulted in multiple organ failure and death. CASE REPORT: A 700 g bw. infant, born at 28 weeks of gestation, was referred to our centre because of suspected bowel perforation. In the referring hospital, the infant had a central venous catheter inserted peripherally. The catheter migrated to the right intrapleural space, and parenteral formula was delivered over several hours to the right pleura, resulting in hydrothorax with serious compression of the lung and atelectasis. Emergency laparotomy did not reveal any pathology and a chest tube was inserted into the right pleura; the effusion fluid contained a large number fat particles. The child's condition worsened and he died 16 days after surgery because of multiple organ failure and sepsis. CONCLUSION: Accidental migrations of central venous catheters to the pleural space have been described by many authors. It can result in severe pneumonia, cardiac tamponade or sepsis and is often fatal. We conclude that central venous catheters in premature infants should be inserted under ultrasonography or fluoroscopy. Catheters should never be forced along vessels; their size ought to be adjusted to age, and a free outflow of blood should be obtained before they are used.

[Iatrogenic pleuropneumonia as a complication of nutritional treatment of preterm-delivery newborn]. / Jatrogenna pleuropneumonia jako powiklanie leczenia zywieniowego u noworodka urodzonego przedwczesnie.

Our study presents a case of pleuropneumonia caused by a leak of nutritional formula to pleural cavity, which was caused by perforation of the oesophagus. The child was born in 28 hbd with 1400 g birth weight and was fed with mother's milk by a nasogastric tube. From day 11 of life general state of the neonate worsened and on chest X-ray the contrast showed leaking into the right pleural cavity and the end of gastric tube was seen in the right lung area. With this diagnosis the child was admitted to the University Hospital in Bydgoszcz. The child was conservatively treated and in two contrast X-ray examinations there was no pathology of the oesophagus. CT of chest showed pleural empyema which was repeatedly punctured. On the 19th day of hospitalization thoracotomy with resection of interior pulmomery lobe was performed. From the 14th day after surgery, the child was again enterally fed and in good general state. He was discharged on the 51st day of hospitalization. This case should pay our attention to the fact that respiratory distress syndrome of preterm-delivery newborns may be caused by iatrogenic proceedings not only infections and lack of surfactant. Some complications can be accomplished with the nutrition treatment in every dimension.

Coagulation changes in a siamese twin in SIRS after death of the other twin: a case study.

BACKGROUND: Conjoined twins represent a rare case of embryonic failure. Siamese twins' final outcome is usually associated with poor prognosis due to complications, among which the inflammatory and septic disturbances are often present. CASE REPORT: The article describes Siamese twins of craniopagus type. One of the twins died of heart and aortic failure after 48 hours of life. The decision regarding surgical separation then became an emergency. At the same time laboratory results for the surviving twin I indicated steadily worsening coagulation functions and processes, which we believed were caused by the death of twin II, as well as by systemic inflammatory response syndrome (SIRS). CONCLUSIONS: Due to vascular conjunction between the twins' brains' circulatory systems the sequenced progress of coagulopathy was noticed in the surviving twin.

[Influence of the Pringle's maneuver time applied in children during hepatic tumors resections on the postoperative course of treatment]. / Wplyw czasu trwania manewru Pringle'a zastosowanego u dzieci podczas resekcji guzów watroby na przebieg pooperacyjny procesu leczniczego.

UNLABELLED: One of the ways of diminishing risk of massive blood loss during liver surgery is the temporal vascular exclusion of the liver/Pringle maneuver. Scanty publications on the problem of liver resections in children are based on very limited series of patients and did not define save periods of time of temporal vascular exclusion of the liver during operation in this group of patients. They also did not specify the effects arising from prolonged acute hepatocytes ischemia in children. The aim of the study was the assessment of the influence of length of time of Pringle maneuver on the liver cells function in the perioperative period and the assessment of its influence on the postoperative course of treatment. MATERIAL AND METHODS: Patients according to the duration of Pringle maneuver were divided into 2 groups. Group I consisted of 18 patients, in whom the duration of Pringle maneuver was below 32 minutes. Group II consisted of 17 patients, in whom the duration of Pringle maneuver was between 32 and 60 minutes. Selection of the periods of duration of Pringle maneuver was dictated by mean and mediana value of its lasting. In patients from both groups values of the level of AspAT, ALAT LDH and INR were assessed in postoperative day 1, 2 and 3 as well as time of mechanical ventilation, length of stay in ICU, and the duration of hospitalization. RESULTS: In group II of children operated with Pringle maneuver lasted between 32 and 60 minutes, statistically significant longer mean value of time of operation was indicated, as well as statistically significant higher mean value of biochemical parameters describing liver cells function, slow down dynamic of normalization of INR in the first 3 postoperative days and statistically significant higher mean value of length of time of hospitalization. CONCLUSIONS: Extension of time of Pringle maneuver in children from 30 to 60 minutes may be connected with transient postoperative biochemical dysfunction of hepatocytes characterized by slow down dynamic of normalization of the level of liver enzymes as well as may results in extension of time of hospitalization connected with necessity of normalization of transient postoperative biochemical dysfunction of hepatocytes.

[Influence of Pringle maneuver used in children during hepatic tumors resections on fluid resuscitation and intraoperative blood loss]. / Wplyw manewru Pringle'a zastosowanego u dzieci podczas resekcji guzów watroby na terapie plynowa i sródoperacyjna utrate krwi.

UNLABELLED: Partial vascular exclusion (Pringle maneuver) is one of the methods diminishing risk of massive intraoperative blood loss. Effects of Pringle maneuver were deeply explored in adults due to big series of operated patients but experiences with Pringle maneuver in children are very limited. The aim of the study was to estimate usefulness of Pringle maneuver in the limitation of intraoperative blood loss and the amount of intraoperative fluid therapy necessary during liver resections in children. MATERIAL AND METHODS: The group of 70 children operated because of liver tumors during the period of 20 years were studied. Patients were divided into 2 subgroups. Group I consisted of 35 patients without Pringle maneuver during liver resection. Group II was consisted of 35 children operated with Pringle maneuver. In all patients intraoperative diuresis and hemodynamic parameters of blood circulation were monitored and the blood loss and fluid therapy were assessed. RESULTS: In group I operated on without Pringle maneuver statistically significant higher amount of blood transfusions were indicated and in group II statistically significant higher amount of colloids were indicated. CONCLUSIONS: The results achieved documented that introduction of Pringle maneuver during liver resection in children allow to achieve statistically significant limitation of blood loss during operation but hemodynamic disorders during partial vascular exclusion (Pringle maneuver) requires transfusion of higher amounts of volume colloid fluids.

[Primary carcinoma of the thyroid growing in thyroglossal duct cyst: presentation of two cases]. / Pierwotny rak tarczycy w obrebie torbieli przewodu tarczowo-jezykowego: opis dwóch przypadków.

Thyroglossal duct remnants (TGDR), most often cysts, are the most common type of developmental abnormalities of the thyroid gland. In about 1 to 2% of TGDR neoplastic transformation occurs. Papillary carcinoma of the thyroid may be encountered in over 90% of such cases. Two cases of primary papillary carcinoma of the thyroid in TGDR in young girls are presented. The diagnostic and therapeutic problems are shared, and up-to-date management guidelines in similar cases are discussed.

[Diagnosis and treatment of thyroid cancer in children in the multicenter analysis in Poland for PPGGL]. / Diagnostyka i leczenie raka tarczycy u dzieci w Polscew analizie wieloosrodkowej dla PPGGL.

INTRODUCTION: Differentiated thyroid carcinoma (DTC) in children presents different biological behavior in comparison to adults. Authors presents preliminary results of multicenter analysis concerning incidence, diagnostics and treatment of DTC in children. MATERIAL AND METHODS: The study is a retrospective analysis of 107 pediatric patients from 14 academic centers based on the data from 2000 to 2005 obtained by questionnaire in hospitals involved in the treatment of DTC in children. RESULTS: Papillary thyroid cancer was diagnosed in 83 children, follicular thyroid cancer in 10 children and medullary thyroid cancer in 14 children. Incidence of DTC in children was estimated between 18 and 23 cases per year. The biggest group of patients consisted of children between 11 and 15 years of age, with girls to boys ratio 3.3 : 1. Clinically DTC in children presented most often as solitary thyroid nodule. Cervical lymphadenopathy was observed in 42% of patients. Intraoperative verification indicated metastatic nodes in 50% of children. Low stage DTC predominated (T1 in 36% and T2 in 26% of children). One step surgery was performed in 65% of children with DTC, two step surgery in 25% of patients. I131 therapy was undertaken in 80% of children. Lung metastases were indicated in post therapeutic studies in 14% of children with DTC. Prophylactic thyroidectomies were performed in 79% of children in the group of patients with MTC and RET gene mutations. CONCLUSIONS: The necessity of introduction of unified therapeutic standard in children with DTC in Poland is underlined.

Hemangioma of the liver in children: proliferating vascular tumor or congenital vascular malformation?

BACKGROUND: Hepatic vascular tumors (HVT) are the most common benign liver tumors present in infancy and childhood commonly associated with high output cardiac failure. Pediatric HVT usually are divided into infantile hemangioendothelioma (IHE), cavernous hemangioma (HC), and arteriovenous malformations (AVM). The aim of this study was to analyze clinical and histologic features of pediatric HVT in relation to treatment strategies. PROCEDURE: During last 12 years, 17 children have been treated because of HVT. The diagnosis of HVT was established in all on the basis of clinical and imaging data. A retrospective analysis of clinical records and histopathology divided 17 into: Group 1, neonates; and Group 2, infants and older children. RESULTS: Radiologic imaging revealed the vascular nature of the tumors in all patients. All nine from Group 1 were symptomatic from AVMs and seven were operated upon. Only one of eight children from Group 2 presented symptoms of AVM requiring surgery; four of five in this group had surgery because of the risk of malignancy. Within Group 1, a mixture of proliferating IHE with microscopic features of AVM was found in most. In three neonates with HVT immunologic and clinical features of cytomegalovirus (CMV) hepatitis were noted. In two Group 2 patients, pure HC was present and in another, the diagnosis of angiosarcoma was established after biopsy of a peritoneal metastasis. CONCLUSIONS: HVT in children demonstrate internal morphologic heterogeneity and an age-related behavior of the disease. We also confirm the proliferative nature of all hemangioendotheliomas (HEs) in children. Further studies on the tumorigenesis of these lesions are needed.