Andrzej Kamiński

Treatment of progressive familial intrahepatic cholestasis: liver transplantation or partial external biliary diversion.

Abstract: Progressive intrahepatic familial cholestasis (PFIC), previously called Byler’s disease, is a syndrome in which children develop severe cholestasis progressing to biliary cirrhosis and chronic liver failure, usually during the first decade of life. Clinical features include jaundice, hepatomegaly, splenomegaly, growth retardation and severe pruritis. Laboratory tests demonstrate elevated bilirubin and bile acids, without an increase in serum gamma‐glutamyl‐transpeptidase or cholesterol. This study was performed to evaluate our experience with medical therapy as well as two types of surgical treatment used in children with PFIC, particularly partial external biliary diversion (PEBD) as an alternative method of therapy to liver transplantation (OLTx). Between 1979 and 1998 we have treated 46 children with PFIC (27 boys and 19 girls), aged 10 months to 19 yr (at the time of this study). Medical treatment with ursodeoxycholic (UDCA) was used in 39 patients for the period between 6 and 82 months. PEBD (cholecysto‐jejuno‐cutaneostomy) was performed in 16 patients, OLTx in eight children (including one after unsuccessful PEBD). Retrospective analysis of the clinical course and selected laboratory tests (bilirubin, ASPAT, ALAT, bile acids), and histopathological examinations were performed. Results of treatment were assessed by means of influence of the type of treatment on clinical symptoms, laboratory tests, progress of liver cirrhosis and hepatic failure, as well as physical development and survival. Medical therapy was effective in the long term in four (10%) of the patients resulting in clinical and biochemical normalization. Both surgical methods of therapy of PFIC, PEBD and OLTx, resulted in an 80% success rate and therefore should be used as complementary therapies. In patients before established liver cirrhosis, PEBD should be the first choice of treatment. Patients presenting with cirrhosis or after ineffective PEBD should qualify for OLTx. With this strategy most children with PIFC can be cured.

Successful treatment of a child with fulminant liver failure and coma caused by Amanita phalloides intoxication with albumin dialysis without liver transplantation.

Abstract:  FLF is a life‐threatening disease. Hepatic coma exerts dramatic impact on patient survival. At present, LTx is the treatment modality of choice that provides significant improvement in outcome of most patients with FLF. Multiple attempts have been made to reduce mortality and improve the patients condition. One of the new options is AD – MARS. We present the case of a 11‐yr‐old boy with FLF and hepatic coma who avoided the scheduled LTx because of rapid neurological and biochemical improvement immediately after three MARS sessions.

Single pretransplant bolus of recombinant activated factor VII ameliorates influence of risk factors for blood loss during orthotopic liver transplantation

Abstract:  Large blood loss and transfusions during liver transplantation (LTx) may lead to serious complications and have a negative impact on post‐transplant mortality and morbidity. In the retrospective study we compared two groups of recipients of primary cadaveric liver transplantation: group I (study group), consisted of 28 patients with preoperative risk of high intraoperative blood loss, including severe uncorrected coagulopathy. This group was given a bolus of recombinant activated factor VII (rFVIIa) just before LTx. Group II (control group) included 61 patients without a particular risk for increased intraoperative blood loss. These patients were not given rFVIIa. We analyzed both groups for: coagulation parameters before, during and after surgery (INR, APTT, factor VII activity), blood and FFP transfusions, operative time, postoperative complications (vascular thrombosis, reoperation for bleeding), postoperative ICU stay, post‐transplant hospitalization time and mortality. Patients from the study group (I) had significantly worse coagulation parameters than patients in the control group (II) at the start of the surgical procedure; however, after administration of a bolus of rFVIIa there was immediate correction of coagulation in all recipients. No significant differences in intraoperative blood transfusions were observed between study and control groups (1980 ± 311.4 mL vs. 1527 ± 154.2 mL, respectively), operating time (8.7 h vs. 8.9 h) or ICU and hospital stay (7.03 days vs. 6.15 days and 40.89 days vs. 41.1 days). Re‐exploration because of bleeding was performed in three patients from group I (10.7%) and in seven patients (11.5%) from group II. No single case of vascular thrombosis was observed in the study group, while in the control group there were three hepatic artery thromboses, two portal vein thromboses and one hepatic vein thrombosis. We conclude that rFVIIa given preoperatively to liver transplant recipients with several risk factors for high intraoperaive bleeding adjusts these patients to a normal risk group, without an increased risk for thrombotic complications.

Laparoscopic splenectomy for hereditary spherocytosis-preliminary report.

Splenectomy is considered standard surgical therapy in hereditary spherocytosis. The procedure is indicated in patients with severe anemia, recurrent hemolytic, and aplastic crises. The aim of the study was to assess treatment outcomes in patients with hereditary spherocytosis who underwent total or partial laparoscopic splenectomy. Fifteen patients aged 4–17 yr underwent laparoscopic splenectomy from 2009 to 2012. Partial and total splenectomies were performed (five and 10 children, respectively). Hematologic parameters, liver function tests, and splenic volume before and after the surgery were analyzed retrospectively. Total follow‐up was 1–30 months. Hospitalization and operating time were similar in both groups. In partial splenectomy group, branches of splenic arteries gave better blood supply than short gastric vessels. In both groups, hematologic parameters were improved. Postoperative markedly elevated platelet count was maintained up to 6 months, and after that, platelet count gradually decreased to normal values. Bilirubin level was decreased in early postoperative period; however, it increased later to achieve levels lower than in preoperative period. No severe general infections were observed in both groups. Laboratory parameters (hemoglobin and bilirubin concentrations and RBC) after the surgery improved in all patients, and the effect was maintained during 12 months of follow‐up. Platelet count increased significantly after the surgery and was maintained at high levels during the next 6 months. However, it returned to preoperative levels within a year after the surgery. Our study showed that partial splenectomy was not inferior to total splenectomy. However, full assessment requires longer follow‐up and larger group of patients.

Desmoid tumor of the pancreas: a case report

IntroductionDesmoid tumor is a rare, benign, usually asymptomatic fibromatous lesion. The etiology is unknown and the diagnosis is based on histopathological examination. The treatment is complete resection of the tumor. Pancreatic desmoid tumor is extremely rare. In the literature there have been only 11 cases described, most of them as solid or solid-cystic masses. We report the case of a patient with an isolated cystic pancreatic desmoid tumor that is, to the best of our knowledge, the second reported case.Case presentationA 13-year old Caucasian boy presented with recurrent pain of two months’ duration in the left hypochondrium of his abdomen. An ultrasound examination and computed tomography scan revealed the presence of a cystic mass located in his splenic hilum, tightly adjacent to the pancreatic tail. A splenic cyst was suspected. Operative findings showed a 10x10cm cystic mass tightly connected to the pancreatic tail and left colonic flexure, adherent to the spleen, splenic vein and artery. Distal splenopancreatectomy with en bloc resection of the left colonic flexure was performed. Histological analysis confirmed that the resection was complete. The mass had infiltrated the pancreatic parenchyma. All tumor cells were positive for anti-beta-catenin staining characteristic for desmoid tumor. No abnormalities in the spleen and colon were found.ConclusionsIsolated sporadic pancreatic desmoid tumor with cyst formation is extremely rare and its diagnosis can be difficult, especially because of uncharacteristic symptoms and radiological findings, as in our patient. This case report should be of interest not only to surgeons, as the treatment of choice is radical resection, but also gastroenterologists, considering it is in close relation with familial adenomatous polyposis, and oncologists as the reason for differentiation with other pancreatic tumors.

3D high-definition manometry in evaluation of children after surgery for Hirschsprung's disease: A pilot study

PURPOSE Anorectal 3-dimensional high definition manometry (3D HRM) could be the best tool for postoperative assessment of restorative surgical procedures for Hirschsprungs disease. The aim of our study was to evaluate patients after surgery for Hirschsprungs disease using 3D HRM. MATERIALS AND METHODS Anorectal function was evaluated using solid state 3D HRM. We measured the length of the anal canal, mean resting squeeze pressures, the presence of rectoanal inhibitory reflex, cough reflex, ano-anal reflex and the bear down manoeuvre. RESULTS We studied 14 children operated on for Hirschsprungs disease. The mean values of pressure asymmetry were higher in patients after the Duhamel procedure than after the TEPT procedure (29.58% vs. 22.26% during resting and 26.1% vs. 14.01% during squeeze, respectively). No difference between the groups was observed in the measurement of all the manometric parameters except the presence of rectoanal inhibitory reflex (87.5% after TEPT vs. 33% after Duhamel). CONCLUSIONS Anorectal 3D HRM evaluation of patients with Hirschsprungs disease demonstrated that the asymmetry of the anal canal occurred in a similar percentage after both procedures.

Trichotillomania and trichophagia - diagnosis, treatment, prevention. The attempt to establish guidelines of treatment in Poland.

Trichotillomania is a disorder characterised by inability to control over pulling own hair from various parts of a body resulting in noticeable hair loss. Due to its long-term, progressive course, untreated trichotillomania can lead to disturbances in the functioning of patients and complications which are dangerous to life and health. Due to the ambiguous nature of the symptoms, they often remain unrecognised by clinicians. Most patients are afraid of revealing symptoms and reluctantly seek for professional help. In our opinion, it is necessary to increase the awareness of the disorder of physicians of different specialties to improve the detection, treatment efficacy and to prevent dangerous complications of trichotillomania. This paper summarises the current state of knowledge on the epidemiology, aetiology, clinical presentation, and treatment of trichotillomania. It is also an attempt to create guidelines in all cases of suspected trichotillomania - adapted to Polish conditions. It also highlights the importance of a multidisciplinary treatment as a condition of effectiveness of the therapy and prevention of relapse.

Trichotillomania, trichophagia, trichobezoar - summary of three cases. Endoscopic follow up scheme in trichotillomania.

AIM Trichotillomania is a lack of control of ones hair pulling. It is estimated that about 1% of population develops trichotillomania. In up to 20% of patients with trichotillomania swollowing follows hair pulling. Trichobezoar forms in about 30% of patients with trichofagia. MATERIAL AND METHODS In 2008-2014 3 patients were operated on trichobezoar. One patient has had a history of trichotillomania. On admission abdominal X-ray and ultrasonography revealed abdominal mass. Diagnosis was confirmed in abdominal computed tomography. RESULTS All three trichobezoars were evacuated from the intestinal tract during laparotomy with wide gastric wall opening. In one case - Rapunzel syndrome - hair mass was evacuated also from the duodenum and small bowel. All patients were referred to psychiatrist after finishing of the surgical treatment. CONCLUSIONS In patients operated for trichobezoar as well as other patients with trichotillomania control of hair accumulation in the gastrointestinal tract remains a problem. Authors propose endoscopic follow up scheme in 6, 12, and 24 months after the surgery as well as for other patients with trichotillomania.

The TNF, IL-1, IL-6 and HNP Peritoneal Fluid Concentrations in Premature Infants Treated with Peritoneal Drainage for Intestinal Perforation-Preliminary Study

Purpose: The purpose of the study was to analyze the pro-inflammatory cytokines (TNF, IL-1, IL-6 and HNP) profile in the peritoneal fluid in preterm infants with intestinal perforation due to Necrotizing enterocolitis (NEC) or Spontaneous Intestinal Perforation (SIP) treated with percutaneous peritoneal drainage. Methods: 6 infants with intestinal perforation due to NEC or SIP treated with peritoneal drainage were analyzed prospectively. Samples of peritoneal fluid were drawn for 36 hours after drain placement. Concentrations of cytokines: TNF, IL-1, IL-6 and Human Neutrophil Peptide (HNP-1) were determined using enzyme like immunoassay technique. Results: The peritoneal fluid concentrations of TNF were 0-11,963 pg/mL (mean 960±2.249 pg/mL), IL-1 were 0-10,390 pg/mL (mean 968±2.219 pg/mL), IL-6 were 0.3-1.660 ng/mL (mean 184±335 ng/mL), HNP were 0.04-18.36 μg/mL (mean 3.18±4.32 μg/mL). Peritoneal fluid TNF level>1.000 pg/mL, IL-1>500-1.000 pg/mL and IL-6>200-500 ng/mL were associated with fatal outcome. Conclusions: In analyzed material the pattern of pro-inflammatory cytokines concentrations in peritoneal fluid after intestinal perforation in preterm infants was not identified. The highest peritoneal fluid TNF, IL-1 and IL-6 concentrations were related to fatal outcome in the most premature infants with unspecified extent of intestinal necrosis.