Follow-up of partial splenectomy in children with hereditary spherocytosis.

BACKGROUND/PURPOSE: The aim of this report is to study the short- and long-term consequences of partial splenectomy (PS) in hereditary spherocytosis (HS). METHODS: The authors reviewed the files of 5 children who underwent PS from 1993 to 1998. The data collected included clinical presentation, age, gender, indication for the operation, radiographic examination, need for blood transfusion and hematologic values, description of surgical procedure, and early- and late-occurring complications. RESULTS: From 1993 to 1998, 5 children from one year, 9 months to 7 years of age underwent PS. Indications were hypersplenism and severe anemia. The average age at the time of the operation was 3 years, 5 months. The 2 youngest children (one year, 9 months and 2 years old) needed a second operation because of the recurrence of hypersplenism. CONCLUSIONS: Subtotal splenectomy seems to preserve the immune role of the spleen and can reduce the need for blood transfusion. But PS is associated with a regrowth of the splenic remnant in children suffering from chronic hemolysis with hypersplenism and seems to be effective for a relatively short period only, especially in young children. Thus, a second operation to perform a total splenectomy can be necessary. The decision to perform a PS on young children with HS as an alternative to total splenectomy with appropriate preoperative vaccination and postoperative prophylactic antibiotics therefore should be weighed carefully, keeping in mind, however, the benefit of postponing total splenectomy in these patients

Gastrointestinal complications of post-diarrheal hemolytic uremic syndrome.

PURPOSE: Whereas gastrointestinal symptoms such as vomiting, diarrhea and abdominal pain are common in children suffering from the so-called post-diarrheal form (D+) of hemolytic uremic syndrome (HUS), more serious gastrointestinal complications are rare. We tried to define factors predictive of the severity of gastrointestinal complications post D+ HUS. METHODS: We reviewed the files of all children admitted to our hospital for D+ HUS between 1988 and 2000. We retained those cases with gastrointestinal complications and analyzed the consequences of these complications on the evolution of the children's conditions. RESULTS: Sixty-five children with D+ HUS were admitted to our hospital during this period. Sixteen children developed gastrointestinal complications involving one or more digestive organs: necrosis of the colon or ileum, hemorrhagic colitis, pancreatitis, transient diabetes, hepatic cytolysis and cholestasis, peritonitis and prolapse of the rectum. One child died. CONCLUSION: Gastrointestinal complications of D+ HUS are rare, but they can be lethal, and early surgery may sometimes prove necessary. However, we were not able to demonstrate a correlation between the severity of the gastrointestinal manifestations and the clinical or biological signs accompanying D+ HUS

Ventilation-induced pulmonary vasodilatation in lambs with congenital diaphragmatic hernia is modulated by nitric oxide.

Endogenous nitric oxide (NO) mediates pulmonary vasodilatation at birth, but inhaled NO fails to reduce pulmonary vascular resistance (PVR) in newborns with congenital diaphragmatic hernia (CDH). This study was designed to investigate the effects of ventilation, and the nature of its endogenous mediator, in fetal lambs with experimental CDH. Investigations at 138 days of gestation showed that ventilation markedly decreased PVR. Inhibition of NO synthesis reduced ventilation-induced pulmonary vasodilatation in vivo and increased in vitro isometric tension of vascular rings. Ventilation therefore reduces PVR at birth in lambs with CDH, and endogenous NO seems to contribute to this reduction

Sténoses post-traumatiques du grêle chez l’enfant : à propos de 2 cas

Post-traumatic small-bowel obstructions are rare and late complications following blunt abdominal trauma. Timely diagnosis is frequently impeded because of nonspecific associated symptoms, and furthermore, the degree of urgency is frequently underestimated due to the accidental cause. During the last 6 years (2005–2011), we have observed only 2 cases (4 and 10 years old) with post-traumatic small-bowel obstruction. On admission the patients had a contusion on the abdominal wall, duodenal hematoma and a Chance fracture of the lumbar spine on the computed tomography (CT) scan. Although early clinical improvement was noted, progression of the disorder was observed for the following 15 days like an occlusive syndrome (abdominal distension, nausea, emesis). Abdominal ultrasound and computed tomography scan revealed small-bowel obstruction. At laparotomy, jejunal stenosis was found associated with mesenteric tears, which was resected with end-to-end anastomosis. Six to 8 months of follow-up ascertained the therapeutic efficacy of the procedure. Post-traumatic small-bowel obstructions are rare; the clinical presentation is unspecific and appears with a time lag following the trauma. Echography and computed tomography scan can establish both the diagnosis and therapeutic choice.IIntroductionLes sténoses post-traumatiques du grêle sont une des complications tardives et rares des traumatismes fermés de l’abdomen. Leur diagnostic est difficile et peut être retardé compte tenu de leur faible fréquence et de leur mode de révélation peu spécifique.ObservationsNous rapportons 2 observations de garçons de 4 et 10 ans admis pour un traumatisme abdominal fermé à haute énergie entre 2005 et 2011. À leur admission, ils présentaient une contusion pariétale péri-ombilicale, des hématomes profonds (duodénal, caecal) et, pour l’un, une lésion type Chance en L2-3 à la tomodensitométrie (TDM). Malgré une amélioration clinique initiale, il persistait un état subocclusif (nausées et vomissements lors de la réalimentation) au-delà du 15e j. L’échographie et la TDM permettaient d’objectiver une occlusion grêlique. L’exploration chirurgicale mettait en évidence dans les 2 cas, plusieurs sténoses jéjunales associées à une dilacération du mésentère, imposant à chaque fois une résection-anastomose termino-terminale.CommentairesLes sténoses post-traumatiques du grêle sont rares et leur symptomatologie non spécifique apparaît avec un délai variable par rapport au traumatisme abdominal. La surveillance clinique, ainsi que l’échographie et la TDM permettent d’évoquer le diagnostic et de conduire à un traitement chirurgical efficace

Splénectomies coelioscopiques chez l'enfant: expérience et résultats [Laparoscopic splenectomy in children: experience and results].

Pediatric laparoscopic splenectomy is a relatively new surgical procedure. Advances in instrumentation and technique now make this procedure possible. Splenectomy is frequently performed in children for various hematologic and autoimmune diseases. PATIENTS AND METHODS: This article reviews the indication for splenectomy, the technique of laparoscopic splenectomy and our results. Between January 1996 and January 2000, 23 children underwent laparoscopic splenectomy. Three of them also had a concomitant cholecystectomy. Their ages ranged from three to 14 years. Nine children had hereditary spherocytosis, four were affected by sickle cell disease, three had an idiopathic thrombocytopenia and three a hemolytic disease. One patient was converted. RESULTS: Mean operative time was 170 min (range, 115-230 min). Hospital stay ranged from three to 15 days (median, 6). Five patients had complications (three pneumonia and two deep abscesses). Median follow-up was 20 months (two months to four years) without problems regarding procedure. CONCLUSION: Regarding the low complication rate for this type of patient and the advantages of a small abdominal trauma in the postoperative period, the laparoscopic approach for elective splenectomy in hematological disorders is the technique of choice

Nitric oxide activity through guanylate cyclase and phosphodiesterase modulation is impaired in fetal lambs with congenital diaphragmatic hernia.

BACKGROUND: Congenital diaphragmatic hernia (CDH) is associated with pulmonary hypertension and death. Administration of nitric oxide (NO) alone remains ineffective in CDH cases. We investigated in near full-term lambs with and without CDH the role of guanylate cyclase (GC), the enzyme activated by NO in increasing cyclic 3'-5'-guanylosine monophosphate, and the role of phosphodiesterase (PDE) 5, the enzyme-degrading cyclic 3'-5'-guanylosine monophosphate. METHODS: Congenital diaphragmatic hernia was surgically created in fetal lambs at 85 days of gestation. Pulmonary hemodynamics were assessed by means of pressure and blood flow catheters (135 days). In vitro, we tested drugs on rings of isolated pulmonary vessels. RESULTS: In vivo, sodium nitroprusside, a direct NO donor, and methyl-2(4-aminophenyl)-1,2-dihydro-1-oxo-7-(2-pyridinylmethoxy)-4-(3,4,5 trimethoxyphenyl)-3-isoquinoline carboxylate sulfate (T-1032) and Zaprinast, both PDE 5 blockers, reduced pulmonary vascular resistance in CDH and non-CDH animals. The activation of GC by sodium nitroprusside and the inhibition of PDE 5 by T-1032 were less effective in CDH animals. In vitro, the stimulation of GC by 3(5'hydroxymethyl-2'furyl)-1-benzyl indazole (YC-1) (a benzyl indazole derivative) and the inhibition of PDE 5 by T-1032 were less effective in pulmonary vascular rings from CDH animals. The YC-1-induced vasodilation in rings from CDH animals was higher when associated with the PDE 5 inhibitor T-1032. CONCLUSIONS: Guanylate cyclase and PDE 5 play a role in controlling pulmonary vascular tone in fetal lambs with or without CDH. Both enzymes seem to be impaired in fetal lambs with CDH