Palpable Liver Research Articles

Case report on failed Kasai-portoenterostomy for extrahepatic biliary atresia with neonatal cholestasis in one-month-old infant

Biliary atresia is a neonatal onset, obstructive cholangiopathy of the intrahepatic or extrahepatic biliary system, leading to the build-up of bile in the liver. This case discusses a one-month-old infant who was previously diagnosed with jaundice, presenting the symptoms of yellowish eyes and skin, pale stools, and palpable liver. The infant and her mother’s liver enzyme levels were found to be higher and her histopathology studies evidenced atretic gall bladder. The liver biopsy revealed mild periportal ductular reaction and diffuse hepatocanalicular cholestasis. She was diagnosed with neonatal cholestasis- extrahepatic biliary atresia (EHBA) and underwent Kasai-portoenterostomy. She was stable and discharged with gallstone dissolution agents, antibiotics, vitamin supplements, and a barbiturates-liver enzyme inducer. After six months, she presented hepatosplenomegaly with ascites and was found to have transaminitis. She was then diagnosed with EHBA- failed Kasai, probable cholangitis, and planned for living donor liver transplantation. Antibiotics, antiviral, antifungal, anticoagulants, and immunosuppressants were prescribed on discharge. The Doppler study of allograft was performed to check the blood flow after transplantation. Acute graft rejection was monitored on day 5 with liver transplant pack reports. One year later, the infant's condition had shown improvement, evidenced by enhanced food intake, absence of symptoms, and the return of liver function tests to normal levels.

The Outcome of Patients with Leukemia Presenting with Hyperleukocytosis Requiring Leukapheresis. The Experience of King Fahad Specialist Hospital in Dammam, Saudi Arabia

Background: Patients with acute and chronic leukemia presenting with hyperleukocytosis are at risk of developing leukostasis which has serious and life-threatening complications. Leukapheresis is usually performed to reduce the complications of leukostasis in patients presenting with hyperleukocytosis and clinical manifestations compatible with leukostasis. Methods and materials: A retrospective study of patients with acute and chronic leukemia who received leukapheresis for hyperleukocytosis between the 1st of January 2013 and the 31st of December 2023 at King Fahad Specialist Hospital (KFSH) in Dammam, Saudi Arabia was performed. Results: Over a period of 11 years, a total of 50 patients with acute and chronic leukemia presenting with hyperleukocytosis and clinical manifestations of leukostasis; 32 patients with acute leukemia (AL) and 18 patients with chronic myeloid leukemia (CML); received leukapheresis at our institution. Among the 32 patients with AL who received leukapheresis, 24 patients (75%) had acute myeloid leukemia (AML), 7 patients (21.88%) had acute lymphoblastic leukemia (ALL) and 1 patient (3.13%) had bilineage acute leukemia (BAL). At presentation of their AL: 3 patients (9.38%) had fever, 9 patients (28.13%) had infections, 4 patients (12.5%) had palpable spleen or liver, 6 patients (18.75%) had palpable external lymph nodes, and 9 patients (28.13%) had extramedullary disease (EMD). After receiving induction and consolidation cycles of chemotherapy, 11 patients (34.38%) of AL patients received allogeneic hematopoietic stem cell transplantation (HSCT). At the end of the follow-up, 17 patients (53.1%) with AL were alive while 15 patients (46.9%) were dead. The 8-year overall survival (OS) for all patients with AL subjected to leukapheresis was 47%. The 5 years OS for patients with AL who subsequently received HSCT and those who did not receive allogeneic HSCT were 70% and 40% respectively. The mean white blood cell (WBC) count of CML patients subjected to leukapheresis was 465.5 × 109/L, 11 patients (61.11%) had clear signs of leukostasis, and 8 patients (44.44%) had splenomegaly at presentation. Regarding the disease stage at presentation, 14 CML patients (77.78%) had chronic phase (CP), 2 patients (11.11%) had accelerated phase (AP) and 2 patients (11.11%) had blast phase (BP). Regarding the fate of CML patients at the end of the study were: 15 (83.33%) were alive, 1 (5.56%) dead, and 2 (11.11%) were unknown as they lost follow-up. However, the 10-year OS of patients with CML subjected to leukapheresis was 90%. Conclusion: Patients with acute or chronic leukemia presenting with hyperleukocytosis and either ongoing or impending leukostasis should have urgent cytoreductive chemotherapy and leukapheresis to prevent life-threatening complications. Although the outcome of AL patients presenting with leukostasis is generally poor, prompt cytoreductive therapy and leukapheresis, followed by induction chemotherapy and allogeneic HSCT may improve the outcome. Also, urgent cytoreduction including leukapheresis improves the outcome of patients with CML presenting with hyperleukocytosis and leukostasis.

Myocardial dysfunction and thrombocytopenia, rare manifestations of acute post-streptococcal glomerulonephritis: case report

In this case report, we present two rare cases of acute post-streptococcal glomerulonephritis with unusual manifestations in the form of myocardial dysfunction and thrombocytopenia. APSGN typically follows Streptococcal infections and is characterized by inflammation of glomeruli. These cases, however, exhibited additional complexities. The first case involved a 12-year-old male with fever, shortness of breath, and cough. He presented with pedal edema, pallor, and hypertension. Laboratory findings revealed thrombocytopenia, anemia, and decreased C3 levels, while echocardiography indicated grade-3 diastolic dysfunction. The second case featured a 5-year-old female with icterus, fever, and body swelling. She had a palpable liver, pleural effusion, and thrombocytopenia. Both cases were diagnosed with APSGN, congestive heart failure, and thrombocytopenia. Thrombocytopenia is a rare finding in APSGN, and its etiology remains debated. Treatment included decongestive therapy and antihypertensive medication. Notably, thrombocytopenia in both cases improved without specific intervention, challenging the necessity of steroids or IVIg therapy. This report illuminates on the atypical presentation of APSGN, highlighting the potential coexistence of glomerulonephritis and thrombocytopenia. It underscores the need for further research to better understand this association and determine appropriate treatment protocols. These cases emphasize the importance of considering diverse clinical manifestations in the context of APSGN, calling for a broader understanding of this condition.

Evaluating local tumor destruction and wedge or segmental resection in the treatment of fibrolamellar hepatocellular carcinoma: An NCDB analysis.

512 Background: Fibrolamellar hepatocellular carcinoma (FL-HCC) is a rare type of hepatic cancer, comprising 1% of all liver malignancies. Patients are often diagnosed in their second or third decade and often have a palpable liver mass. Treatment primarily consists of surgery and transplant, however, standardized protocols have not been well established due to its rarity. For patients who cannot receive a transplant, there are few studies examining and comparing other surgical types. Using the National Cancer Database, we aim to uncover survival and patient characteristic differences between those who receive Local Tumor Destruction vs Wedge or Segmental Resection. Methods: The National Cancer Database (NCDB) was used to identify patients diagnosed with FL-HCC from 2004 to 2019 using the histology codes 8171 as assigned by the Commission on Cancer Accreditation program. Kaplan-Meier, ANOVA Chi-Square, and Cox Proportional Hazards tests were performed. Data was analyzed using SPSS version 29 and statistical significance was set at α = 0.05. Results: Of the 803 patients in the sample,19 patients received Local Tumor Destruction (LTD) and 142 patients received Wedge or Segmental Resection (WSR). The average age of patients receiving LTD was 54 compared to 32 for WSR. WSR patients experienced longer overall survival than LTD (106 vs. 63 months respectively; p<0.05). When controlling for age, year of diagnosis, tumor size, comorbidity score, and surgery type, WSR showed a greater decrease in overall hazard compared to LTD (HR=0.224 vs 0.306; p<0.05). WSR use was highest in healthy patients with no co-morbidities, however, patients were also significantly more likely to have later stage tumors (II, III, and IV vs. stage I; p<0.001). Despite being associated with a lower OS, LTD was utilized more commonly for earlier stage (stage I vs. II, III and IV; p<0.001) and well differentiated tumors (p<0.001). Compared to WSR, it was associated with higher rates of therapy before and after surgery(p<0.05). There were no significant differences in tumor size, income group, adjuvant therapy type, or facility type (p>0.05) between the treatment groups. Conclusions: Receipt of WSR is associated with improved survival compared to LTD. There are distinct demographic groups which may be guiding surgical selection. Further research into this disparity and other surgical types should be examined to create better guidelines for FL-HCC patients.

Choledochal Cyst (CC) Todani type I in two years old girl: a case report

Backgrounds: Choledocal cyst (CC) is a rare congenital cystic dilation of the bile duct and can be associated with severe complications, including malignancy and inflammation of the surrounding anatomy. Approximately 80% of CC is diagnosed in infants and young children in the first decade of life. Complete surgical resection is recommended in patients with choledochal malformations with excellent outcomes. Case Presentation: A two-year-old girl had intermittent abdominal pain in the upper right quadrant. This complaint was accompanied by fever, nausea and vomiting, decreased appetite and drinking. The physical examination revealed jaundice on the sclera and palpable liver, two fingers below the costal arch. Abdominal ultrasound revealed a cystic mass in the right lower lobe suggestive of the hepatic cyst with inflammation of the cystic head of the pancreas and minimal free fluid per lumen. An abdominal C.T. scan without contrast showed a choledochal cyst classified as Todani Type IV. A cyst in the bile duct was found during the surgical procedure Conclusion: Choledocal cyst is one of the differential diagnoses in children with symptoms of abdominal pain. Appropriate surgical measures should be undertaken to avoid complications.

An underrecognized cause of hepatomegaly in uncontrolled type I diabetes.

A 29-year-old woman with a past medical history of poorly controlled type 1 diabetes and autoimmune hypothyroidism presented to the emergency department for evaluation of abdominal pain, nausea and vomiting. The patient was a cocaine user, but she did not use alcohol and did not smoke. Her current medications included Insulin aspart, Insulin degludec, L-thyroxine and Pantoprazole. She reported no use of over-thecounter medication or herbal and dietary supplements. acute distress. The abdomen was nondistended and soft with diffusely tenderness and a smooth palpable liver 3 to 4 cm below the costal margin. Laboratory studies was significant for glucose 539 mg/dL (normal 65-110), alkaline phosphatase 153 U/L (normal 35-105), gamma-GT 44 U/L (normal <36), lactate 42,1 mg/dL (normal 4,5-19,8), arterial pH 7,32 (normal 7,35-7,45) and HbA1c 11% (4,5-6). A computed tomography (figure 1; panel A and B) and liver biopsy (figure 1; panel C and D) were performed.

Botryoid rhabdomyosarcoma or parasites: transnasal choledoscopy as a diagnostic tool.

A 5-year-old girl presented with jaundice and loss of appetite. Physical examination revealed that she had a palpable liver 4 cm below the right costal arch. There were no ascites, cervical adenopathy, or splenomegaly. Laboratory values showed elevated liver enzymes and direct bilirubin.

Results of lifestyle modification promotion and reproductive/general health check for male partners of couples seeking conception

PurposeMale infertility is partially caused by an inappropriate lifestyle and comorbidities. In this study, we analyzed the prevalence of these factors and the effects of lifestyle modifications as part of male preconception care. MethodsFour hundred and two male partners of couples seeking conception with abnormal parameters upon the first semen analysis were enrolled. They were advised to modify their inappropriate lifestyle as male preconception care. Afterward, their general and male reproductive health was examined. Semen quality was compared before and after the promotion. ResultsSmoking, chronic alcohol use, and genital heat stress were found in 22.6%, 47.0%, and 75.1% of patients, respectively. Palpable varicoceles, hypogonadism, obesity (body mass index ≧30 kg/m2), hypertension, zinc deficiency, hyperlipidemia, liver dysfunction, and diabetes mellitus were found in 25.9%, 17.0%, 7.0%, 14.9%, 16.2%, 37.0%, 26.9% and 3.4% of the participants, respectively; 98.8% of the patients had at least one factor. After the promotion, semen parameters and sperm DNA fragmentation were improved significantly. Improvement was found in those with palpable varicocele or hypogonadism but not in those with night work shift, abstinence (>3 days), erectile dysfunction, hypertension, obesity, zinc deficiency, or diabetes mellitus. ConclusionsComorbidities and inappropriate lifestyle choices were common among men with infertility. The promotion of lifestyle modifications as part of male preconception care could improve semen quality without urologic intervention.

Bilateral upper eyelid swelling (Hoagland sign) in Epstein–Barr infectious mononucleosis: prospective experience

BackgroundThe typical presentation of Epstein–Barr virus infectious mononucleosis includes fever, pharyngitis, measles-like rash, jaundice, and enlarged lymph nodes, liver, or spleen. A painless bilateral swelling of the upper eyelid, sometimes with drooping of the lateral aspect, may also occur. This sign, referred to as Hoagland sign, is not or only marginally mentioned in reviews and textbooks.MethodsBetween 2019 and 2021, two of us evaluated all subjects with a positive acute Epstein–Barr virus serology for the typical signs of mononucleosis and for the possible existence of the Hoagland sign.ResultsDuring the mentioned period, the diagnosis of mononucleosis was made in 26 (14 females and 12 males) subjects aged from 9.0 to 33 years. The initial presentation included fever in 24, enlarged cervical lymph nodes in 23, pharyngitis in 21, a palpable liver in 7, a palpable spleen in 7, jaundice in 2, and a measles-like rash in 2 cases. The Hoagland sign was noted in 14 cases. Patients with and without Hoagland sign did not significantly differ with respect to age and sex.ConclusionsThe Hoagland sign is an easily identifiable clinical sign that is common and likely helpful early in the course of Epstein–Barr virus infectious mononucleosis. There is a need to expand awareness of this sign among physicians.

S1728 Emergent Decompressive ERCP or Not; Go With Your Gut

Introduction: Acute Cholangitis is a biliary emergency that requires prompt diagnosis & appropriate management in a timely manner. Guidelines and best practices dictate that if the Charcot's triad and Tokyo guidelines are indicating moderate to severe cholangitis, ERCP should be performed urgently. In certain situations however, clinical decision making is not as straight forward. We present a case in which a number of confounding factors caused hesitancy in making that decision. Case Description/Methods: 38-year woman with PMH of Crohn’s colitis on adalimumab presented with 2 weeks of progressively worsening upper abdominal pain associated with decreased appetite, nausea, vomiting, change in urine color and chills but no fevers. She completed a course of Augmentin 20 days back for a URI. She was also recovering from alcohol use disorder. She was afebrile and hemodynamically stable. On exam, had marked scleral icterus, palpable liver, spleen with upper abdominal tenderness. Lab were remarkable for lymphocytic leukocytosis, thrombocytopenia and Alk phos 316 U/L, T and DBilirubin-7/6.3 mg/dL, AST/ALT-72/131 U/L, respectively. CT abdomen pelvis with contrast showed no intra-or extrahepatic biliary ductal dilation and moderate hepatosplenomegaly. Ultrasound liver with Doppler confirmed above. GI team was called to evaluate for possible ERCP for cholangitis. Later Peripheral smear which was initially ordered to evaluate low platelets, showed blasts cells following which flow cytometry and bone marrow biopsy confirmed B-lymphoblastic leukemia with BCR-ABL1 fusion 97% of cellularity by differential count (Figure). Liver enzymes normalized after initiation of R-HyperCVAD. Discussion: Intrahepatic cholestasis is a known phenomenon in ALL, however initial presentation with intrahepatic cholestasis is rare except for few reported cases in pediatric population. Leukemic infiltration causing intrahepatic cholestasis causes an important diagnostic dilemma and establishing correct diagnosis has important implications especially since the treatment of choice, ERCP, for the alternate diagnosis is not only invasive but the risk of adverse events is high. Keeping this in mind hematologic malignancies should always be considered as a differential in patients with constitutional symptoms and hepatosplenomegaly. The diagnosis can be confirmed by immunohistochemical stains showing immature B cell lineage from a Liver biopsy; however, it is not necessary as in above case. Systemic chemotherapy is the main treatment for B-ALL/LBL.Figure 1.: Bone Marrow Smear

Clinical features and management of the hydatid cyst of the liver

Introduction: In India, hydatid cysts of the liver are a serious yet untreated public health issue. Aim: To assess the sociodemographic traits, clinical manifestations, and treatment of individuals with liver hydatid cysts in a developing nation. Materials and methods: This retrospective study was conducted in Department of General Surgery, MKCG Medical College and Hospital, Berhampur over a period of 24 months from July 2019- June 2021. 23 individuals who were diagnosed with a liver hydatid cyst based on clinical symptoms, imaging tests, or serology underwent a retrospective, descriptive analysis. The data was evaluated and statistically analysed using IBM® SPSS® 23.0, for Windows®, to bring out the results of the study. Results: The patients' average age was 36, with the age range of 25 to 45 being the most frequently afflicted (10, 43.47%).The majority of the patients were female (56.5%). The most frequent symptoms were abdominal pain (21, 91.3%) and palpable liver (7, 30.4 %).The two main imaging tests utilised to make a diagnosis were computed tomography and abdominal ultrasonography. The most common type of cystic lesion was unilocular and anechoic.

P1020: SCREENING FOR PORTAL HYPERTENSION IN PATIENTS WITH PHILADELPHIA NEGATIVE MYELOPROLIFERATIVE NEOPLASMS

Background: Portal hypertension (PH) is a well-recognized complication of BCR-ABL negative myeloproliferative neoplasms (MPNs) and is associated with significant morbidity and mortality. However, the prevalence of PH and its associated risk factors have not been studied systematically in this patient population. Screening by esophagogastroduodenoscopy (EGD) can facilitate early diagnosis of PH and opportunity for intervention to prevent complications. Aims: To investigate the prevalence of PH and its patient and disease-related risk factors. Methods: From May 2013 to March 2019, MPN patients ≥ 18 years old with spleen >5 cm below the costal margin in myelofibrosis (MF) or any size in Polycythemia Vera (PV) or Essential Thrombocythemia (ET) were enrolled prospectively (NCT01816256). Patients with a known history of portal or hepatic vein thrombosis, liver cirrhosis, and esophageal varices were excluded. Screening for PH was done by EGD to evaluate for esophageal varices (EV) or gastric varices (GV), and portal hypertensive gastropathy (PHG). Abdominal Doppler ultrasonography (USG) was performed to evaluate for portal vein thrombosis and spleen size. PH severity was based on the size of varices or severity of PHG. For sample-size calculation, a precision approach was used, considering scenarios where PH prevalence ranged from 5 to 10% and an upper limit for sample size of 100. At a prevalence estimate of 8%, the 90% confidence interval (CI) was 4-14%, with an acceptable lower limit for moving forward with a screening program. Prevalence of PH was calculated as a proportion and CI are exact using Clopper-Pearson method. Results: Among 102 enrolled patients, EGD and USG were completed in 85 and 84, respectively. Primary and Post-PV/ET MF comprised the majority of the study population (93% [79/85]). Evidence of PH was found in 32 patients (38%, [90% CI 29-47]) of whom 12 (14% [90% CI 9-22]) had moderate to severe PH. EV, GV, and PHG were found in 81% (26/32), 13% (4/32), and 31% (10/32) of patients, respectively. The prevalence of PH among MF and PV/ET patients was 35% (28/79) and 67% (4/6), respectively. In MF patients, PH was moderate-to-severe in 13% (90% CI 8-20). No cases of portal vein thrombosis were identified on USG. Apart from a palpable liver observed more frequently in non-PH patients, there were no other significant differences in baseline characteristics among patients with and without PH. The frequency of JAK2, MPL and CALR mutations among PH patients was 78% (25/32),13% (4/32), and 6% (2/32). There was a trend towards more JAK2 mutations in PH patients (78% [25/32] vs 64% [34/53], p=0.23), but no difference in DIPSS risk or frequency of high molecular-risk mutations. Overall survival was similar in both groups. PH-directed interventions were initiated in 59% (19/32); 28% (9/32) were started on beta blocker (BB) prophylaxis, 16% (5/32) were treated with BB and variceal banding, and 16% (5/32) continued on surveillance EGDs alone. Three patients with moderate PH at baseline developed variceal bleeding (2) and ascites (1). All 3 patients were on BB prophylaxis, and 1 had prior prophylactic banding. There were no adverse events related to study procedures EGD or USG. Image:Summary/Conclusion: Given the study population, our results are generalizable to MF patients. Our study shows that clinically significant PH is common in MF patients, and led to additional clinical interventions in significant number of patients. These data support the inclusion of asymptomatic PH screening in the work up of MF patients.

A Nonimmune Hydrops Case.

A Nonimmune Hydrops Case.

A Male Infant with Cholestatic Jaundice, Hemolytic Anemia, and Thrombocytopenia.

A Male Infant with Cholestatic Jaundice, Hemolytic Anemia, and Thrombocytopenia.

Acute Hepatitis C Virus Infection Treated with Daclatasvir/Sofosbuvir in a 9-Year-Old Boy

AbstractThe present study includes a case report of a 9-year-old boy who came to our center with jaundice, elevated liver enzymes, and palpable liver. He was treated with ursodeoxycholic acid, but no improvement in symptoms was seen. Reverse transcription polymerase chain reaction and liver biopsy were positive for hepatitis C virus. He was treated with daclatasvir/sofosbuvir for 3 months. Daclatasvir/sofosbuvir might be effective against the treatment of hepatitis in children, with no report of remission and minimal side effects.

Non-Cirrhotic Hepatocellular Carcinoma with Duodenal Metastasis in Chronic Hepatitis B: Case Report

Background: Hepatocellular carcinoma (HCC) is the fifth most common cancer worldwide and the second leading cause of cancer-related death. The extrahepatic extension is also more common in non-cirrhotic HCC. It most commonly metastases to the regional lymph nodes, bone, and lungs. Metastasis to the small bowel is rare. Further, hematogenous metastasis to the duodenum is incredibly rare. Case: A 67-year-old man was admitted with a recurrence of hematemesis melena, weight loss, weakness, and decreased appetite. There was a history of hepatitis and a family history of liver diseases. Abdominal examination revealed a palpable liver (8 cm below the right costal margin, firm, irregular, and tender). Laboratory studies revealed Hb 5.2 g/dL, SGOT 301.1 u/L, SGPT 244.7 u/L, albumin 2.7 g/dL, HbsAg was reactive with HBV DNA 2.01 x 104 IU/ml. EGD showed that esophageal varices were not visible, the duodenum showed an irregular mass, bleeding on touch, and many blood clots. Abdominal USG revealed hepatomegaly with suspicion of multifocal hepatoma, while a CT scan suggested the appearance of HCC and no local invasion into the small bowel. The result of the fibro scan was severe fibrosis, not cirrhosis. At laparotomy, a hepatic tumor and duodenum were discovered. The histopathological study of the tumor revealed a well-differentiated HCC. The resection of the tumor could not be done. He was given palliative and symptom treatment HCC on non-cirrhotic liver remains an exceptional condition. Conclusion: We report a non-cirrhotic HCC in chronic hepatitis B infection.

Evaluation of the long-term treatment effects of intravenous idursulfase in patients with mucopolysaccharidosis II (MPS II) using statistical modeling: data from the Hunter Outcome Survey (HOS)

BackgroundMucopolysaccharidosis II (MPS II; Hunter syndrome) is a rare, life-limiting lysosomal storage disease caused by deficient iduronate-2-sulfatase activity. Enzyme replacement therapy (ERT) with intravenous (IV) idursulfase can stabilize or improve many somatic manifestations, but there remains a need for further analysis of long-term treatment outcomes. Using data from patients with MPS II enrolled in the Hunter Outcome Survey (HOS), mixed modeling was performed to evaluate and predict the effects of IV idursulfase treatment on selected clinical parameters for up to 8 years following treatment start. The modeling population comprised male patients followed prospectively in HOS who had received IV idursulfase for at least 5 years and who had data available for two or more time points (at least one post-ERT). Age at ERT start and time since ERT start were included as covariates.ResultsIn total, 481 patients were eligible for inclusion in at least one model. At 8 years post-ERT start, improvement from baseline was predicted for each age group (< 18 months, 18 months to < 5 years and ≥ 5 years at treatment start) in the following parameters: mean urinary glycosaminoglycan levels (percentage changes of > –75% in each group), mean left ventricular mass index (decreases of ~ 1 g/m2) and mean palpable liver size (decreases of > 2 cm). Improvements in mean 6-min walk test distance (increase of > 50 m) and stabilization in percent predicted forced vital capacity and forced expiratory volume in 1 s (decreases of ~ 4 and ~ 9 percentage points, respectively) at 8 years post-ERT start were predicted for patients aged ≥ 5 years at ERT start (these assessments are unsuitable for patients aged < 5 years). Predicted changes over time were similar across the three age groups; however, overall outcomes were most favorable in children aged < 18 months at ERT start.ConclusionsThese findings suggest that the previously reported positive effects of IV idursulfase on the somatic manifestations of MPS II are predicted to be maintained for at least 8 years following ERT initiation and highlight the value of statistical modeling to predict long-term treatment outcomes in patients with rare diseases.

A rare case of post COVID-19 hemophagocytic lymphohistiocytosis in a pediatric patient

Introduction/ObjectiveHemophagocytic Syndromes are a cluster of disorders related to cytotoxic dysfunction of T/NK-cells and are mainly subdivided into Primary (familial) and Secondary (acquired) forms, with the latter usually linked to patients with viral infections; including EBV, CMV among many others. A myriad of other causes have been associated with hemophagocytic lymphohistiocytosis (HLH), most notably systemic inflammatory conditions; especially Juvenile Rheumatoid Arthritis and hematolymphoid malignancies particularly T/NK-cell lymphomas.Methods/Case ReportA previously healthy 7-year-old boy, presented to the ER with fever and a skin rash over both lower limbs of 1 week duration. Two weeks prior he was tested for COVID-19 and was found to be positive. Physical examination further revealed slightly palpable liver and spleen. CBC was done and exhibited pancytopenia, further testing showed elevated LDH, hyperferritinemia and hypertriglyceridemia. However, serological testing for rheumatological conditions was unremarkable. Imaging studies were done and were noncontributory. Subsequently, a bone marrow aspirate and biopsy were done. The bone marrow aspirate showed afew histiocytes engulfing red blood cells and nuclear debris (hemophagocytic cells), complete trilineage maturation and normal M:E ratio of 3:1. Trephine biopsy was hypocellular for age and estimated at about 70%, composed of myeloid and erythroid precursors with various degrees of maturation. Megakaryocytes were adequate in number and showed normal morphology. Extensive histiocytic infiltration as highlighted by CD68 immunostain and focal phagocytosis were identified. CD34 highlighted <5% blasts, PAS special stain showed no fungal elements and no fibrosis was evident by Reticulin special stain. The background was devoid of lymphoid aggregates or granulomas. Stainable iron stores were depleted. No sideroblasts were identified. The patient was treated with corticosteroid and showed marked improvement and was discharged after 3 days.Results (if a Case Study enter NA)NAConclusionHemophagocytic lymphohistiocytosis can be a critical sequela of COVID-19 infection. Suggested mechanisms include impaired/delayed T-cell response and elevated levels of several inflammatory cytokines. Clinical suspesion is important in the diagnosis of these cases. Further study of this correlation is needed as we explore clinical sequelae of COVID-19 infection.

Pediatric non-cirrhotic portal hypertension: Endoscopic outcome and perspectives from developing nations.

Non-cirrhotic portal hypertension (NCPH) forms an important subset of portal hypertension in children. Variceal bleed and splenomegaly are their predominant presentation. Laboratory features show cytopenias (hypersplenism) and preserved hepatic synthetic functions. Repeated sessions of endoscopic variceal ligation or endoscopic sclerotherapy eradicate esophageal varices in almost all cases. After variceal eradication, there is an increased risk of other complications like secondary gastric varices, cholangiopathy, colopathy, growth failure, especially in extra-hepatic portal vein obstruction (EHPVO). Massive splenomegaly-related pain and early satiety cause poor quality of life (QoL). Meso-Rex bypass is the definitive therapy when the procedure is anatomically feasible in EHPVO. Other portosystemic shunt surgeries with splenectomy are indicated when patients present late and spleen-related issues predominate. Shunt surgeries prevent rebleed, improve growth and QoL. Non-cirrhotic portal fibrosis (NCPF) is a less common cause of portal hypertension in children in developing nations. Presentation in the second decade, massive splenomegaly and patent portal vein are discriminating features of NCPF. Shunt surgery is required in severe cases when endotherapy is insufficient for the varices. Congenital hepatic fibrosis (CHF) presents with firm palpable liver and splenomegaly. Ductal plate malformation forms the histological hallmark of CHF. CHF is commonly associated with Caroli’s disease, renal cysts, and syndromes associated with neurological defects. Isolated CHF has a favourable prognosis requiring endotherapy. Liver transplantation is required when there is decompensation or recurrent cholangitis, especially in Caroli’s syndrome. Combined liver-kidney transplantation is indicated when both liver and renal issues are present.

The Scenario of Dengue-like Illness in Paediatric Population of Southern Bangladesh

Dengue is one of the most important mosquito-borne viral diseases affecting humans. Dengue infections can result in a broad spectrum of disease severity ranging from an influenza-like illness (DF) to the life-threatening dengue hemorrhagic fever (DHF)/dengue shock syndrome (DSS). Although adults do experience shock, vascular leakage is generally more severe in young children, and in endemic areas, DSS is seen primarily in the pediatric population. The detailed clinical and laboratory profile is crucial for the diagnosis and the successful management of the patients. This study aims to observe the clinical features and laboratory results for the better understanding of patient management to reduce child death in case of dengue fever. A prospective cross-sectional study was conducted at the dengue ward of Khulna Medical College Hospital; 167 suspected dengue patients were enrolled in our research during the period from 7th August to 8th November 2019. Patients attending the emergency department and pediatric outpatient department with complaints of fever and features of dengue with positive NS1 antigen test and/or positive dengue specific antibody IgM or IgG or both were included in this study. Diagnosis of dengue was made on the basis of compatible clinical features and NS1 antigen positivity and/or detection of IgM and IgG antibodies. Patients were classified according to WHO 2012 protocol, and data was analyzed. One hundred sixty patients (95.8%) were diagnosed as having dengue illness, and the rest 7(4.2%) as fever other than dengue. Among the dengue illness, dengue fever (DF) 87(52.1%), DF with warning signs 51(30.5%), DHF 19(11.4%), and DSS 3(1.8%).The most common sign-symptoms of dengue was fever(100.0%),vomiting(52.1%),abdominal pain(38.3%), headache(23.4%),skin rash(13.25%), melena (7.8%), abdominal tenderness(28.7%),palpable liver (24.6%),and palpable spleen(3.0%). NS1 antigen was found positive in 88.6% cases, negative 3.0%, and NS1 antigen test not done 8.4% cases due to time constrain. Anti-dengue antibody IgM positive in 10(6.0%),IgG positive 7(4.2%),both IgM &amp; IgG positive 7(4.2%),both IgM &amp; IgG negative 57(34.1%) and antibody test not done in 86(51.5%) cases due to time constrain. Anti-dengue antibody not done in 86(51.5%) cases; among them most of the cases (98.8%) are found NS1 positive and 1.2% NS1 test negative(P&lt;0.001) in our study which is statistically significant.&#x0D; Observation of clinical and laboratory parameters is helpful for dengue classification and management to reduce child death.&#x0D; TAJ 2021; 34: No-1: 86-96