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A 6-Month-Old Boy Presented with Progressive Jaundice, Dark Urine and Generalized Pruritus for One Month

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A 6-Month-Old Boy Presented with Progressive Jaundice, Dark Urine and Generalized Pruritus for One Month | Case Presentation | No. 8-2019 | A 6-month-old boy presented with progressive jaundice, dark urine and generalized pruritus for one month Hazera Akter, Md. Wahiduzzaman Mojumder, A. S. M. Bazlul Karim, Mohammed Kamal, Kamrun Nahar and Md. Benzamin Article Info Presentation of Case cardiography were normal. Ultrasonography of the abdomen showed mild hepatomegaly. Slit Department of Pediatric Gastroenterol- Dr. Hazera Akter: A 6-month-old boy of non- lamp examination of eyes showed no abnor- ogy and Nutrition, Faculty of Pediatrics, Bangabandhu Sheikh Mujib Medical consanguineous parents admitted to the Depar- mality. Liver biopsy was done and histo- University, Shahbag, Dhaka, Bangladesh tment of Pediatric Gastroenterology with the pathological examination revealed the paucity (HA, MWM, ASMBK, KN, MB); Depart- complaints of progressive jaundice, dark urine of interlobular bile ducts (out of eight portal ment of Pathology, Faculty of Basic and generalized pruritus for one month (Figure tracts examined, two had bile duct) (Figure 2A Science and Paraclinical Science, Banga- bandhu Sheikh Mujib Medical Universi- 1). The boy was well up to five months of age. and B). Bile duct to portal tract ratio was 0.25. ty, Shahbag, Dhaka, Bangladesh (MK) Then he developed jaundice which was The hepatocytes were mildly swollen and occa- progressive in nature with intermittent pale sional sinusoid contained bile plugs. For Correspondence: colored stool along with dark urine. His mother Hazera Akter also complaints for generalized pruritus which Based on history, physical findings and [email protected] was severe in intensity (disturbing sleep and laboratory investigations with liver biopsy our daily activities) without any diurnal variations. diagnosis was nonsyndromic interlobular bile There was no history of sib death or family duct paucity. history of a similar type of illness. Received: 22 January 2019 Accepted: 12 May 2019 Physical examination revealed he was moder- Provisional Diagnosis Available Online: 2 July 2019 ately icteric, scratch mark present over the whole body and mild (2 cm) firm hepatomegaly Nonsyndromic bile duct paucity ISSN: 2224-7750 (Online) present with normal physical development. 2074-2908 (Print) The laboratory data included normal complete DOI: 10.3329/bsmmuj.v12i2.41228 blood counts except leucocytosis mostly lym- Differential Diagnosis phocyte. Serum total bilirubin was 8.8 mg/dL (conjugated bilirubin 5.1 mg/dL), alanine Dr. A. S. M. Bazlul Karim: The differential diag- Keywords: Jaundice; Pruritus; Urine transaminase 26 U/L and gamma-glutamyl noses were the following: Cite this article: transferase 22 U/L, prothrombin time, activa- Alagille syndrome Akter H, Mojumder MW, Karim ASMB, ted partial thromboplastin time and interna- - Kamal M, Nahar K, Benzamin M. A 6 tional normalized ratio were within normal Alagille syndrome is an autosomal dominant, month old boy presented with progres- multisystem developmental disorder. It is a sive jaundice, dark urine and general- limit. Serum cholesterol level was 133 mg/dL. ized pruritus for one month. Banga- Anti-HAV IgM was negative. Urine R/E was familial disease with wide variability in its bandhu Sheikh Mujib Med Univ J. 2019; normal and urinary reducing substances were clinical presentation. Majority of the patients 12: 113-117. nil. Renal functions, X-ray spines and echo- present before six months of age with jaundice and failure to thrive or cardiovascular symp- Copyright: toms. Cholestasis is manifested by jaundice and The copyright of this article is retained pruritus, present in 96% of cases. Jaundice by the author(s) [Atribution CC-By 4.0] presents as a conjugated hyperbilirubinemia in the neonatal period and the magnitude of the hyperbilirubinemia is minor compared to the Available at: www.banglajol.info degree of cholestasis. Severe cholestasis results in the formation of xanthomas, especially on A Journal of Bangabandhu Sheikh Mujib the extensor surfaces of the fingers, the palmar Medical University, Dhaka, Bangladesh creases, nape of the neck, popliteal fossa, buttocks, and around inguinal trauma sites. These lesions are persisting but may gradually disappear after 10 years of age. The presence of interlobular bile duct paucity and at least 3 of 5 clinical features are necessary for the diagnosis: Figure 1: Our patient Chronic cholestasis, cardiac disease, ocular 114 BSMMU J 2019; 12: 113-117 Table I Investigation reports of patient Parameters Findings References Hemoglobin (g/dL) 11.3 13.5 Total leukocyte count (cells/ 13,000 4,000-10,000 cmm) Differentials Lymphocyte (%) 54 40-75 Neutrophil (%) 39 20-50 Eosinophil (%) 05 1-6 Monocyte (%) 02 2-10 Platelet count 270 K/L 150-400 K/L Figure 2: Liver biopsy of our patient showing portal tract without any bile duct (H & E, x220) Total Bilirubin (mg/dL) 8.8 0.2-1.1 Conjugated bilirubin (mg/dL) 5.1 Dr. Hazera’s Diagnosis Serum ALT (mg/dL) 26 20-60 Nonsyndromic bile duct paucity Gamma-glutamyl- 22 0-30 transpeptidase (U/L) Dr. Parisa Marjan: The patient presented with Serum creatinine (mg/dL) 0.85 0.4-1.4 jaundice and pruritus. Why it is not a case of progressive familial intrahepatic cholestasis (PFIC)? Prothombin time (sec) 12.5 12-16 INR 1.04 Dr. Akter: Progressive familial intrahepatic choles- tasis is a clinical syndrome of intrahepatic APTT (sec) 30 20-40 cholestasis which presents in infancy or early Serum cholesterol (mg/dL) 133 <200 childhood. PFIC1 and PFIC2 usually present Anti-HAV IgM Negative symptoms within the first 3 months of life and PFIC3 present at early adulthood whereas our Urine R/M/E and C/S Pus cell, RBC, mac- rophage- Nil patient presented at 5 months of his age. Along with Reducing sub- cholestasis patients with PFIC1 can present with stances- Nil deafness, pancreatic insufficiency, cholelithiasis, Stool examination No red blood cells and diarrhea. These features were not present in our or leukocytes case. Serum gamma-glutamyltranspeptidase activi- ty is elevated in PFIC3 but in our case it was abnormalities, skeletal abnormalities, and charac- normal. Progressive familial intrahepatic cholestasis teristic facies. In infants, less than 6 months of age, usually runs in families, which was not so in our when the bile duct paucity is usually absent, three case. For these reasons the possibility of progressive or four clinical features should be adequate to make familial intrahepatic cholestasis is ruled out.4-8 the diagnosis. In a family with one definite proband, other members with two and even one Dr. Archana Shrestha Yadav: Have you put biliary feature are suggestive to have Alagille syndrome.1-3 atresia at your differential diagnosis? Dr. Akter: After evaluating patient’s clinical Dr. Akter: No. Biliary atresia can also present with presentations, the physical findings and investiga- jaundice with pruritus but it commonly present in tions results, liver biopsy was done and send for the neonatal period with persistent pale stool. Our histopathological examination. Histopathology patient presented with jaundice, pruritus, inter- th report showed the paucity of interlobular bile ducts mittent pale stool and hepatomegaly at his 5 (out of eight portal tracts examined, two had bile months. The typical histopathological findings of duct and bile duct to portal tract ratio was 0.25 cm. biliary atresia are inflammation, portal tract fibrosis, The hepatocytes were mildly swollen and occa- cholestasis, and bile duct proliferation which were 2 sional sinusoid contained bile plugs. The diagnosis also absent in our case. was made based on the histopathologic examination Dr. Suborna Rani Das: How will you differentiate of the liver. Clinical features described in Alagille between the syndromic and nonsyndromic paucity syndrome were not present in our case except of bile duct? cholestasis which led us to a diagnosis of non- syndromic interlobular bile duct paucity. Dr. Kamrun Nahar: Paucity of intralobular bile ducts BSMMU J 2019; 12: 113-117 115 is a histological diagnosis. Two types are recog- leads to clinical improvement in children with nized: Syndromic and nonsyndromic. The nonsyndromic paucity.10 syndromic form is called Alagille syndrome which Dr. Rana Kumar Biswas: When do you advise for is diagnosed by the presence of at least three of five liver transplantation? major features: Chronic cholestasis (jaundice, pruritus, xanthoma), cardiac disease (pulmonary Dr. Nahar: Patient with complications of end-stage artery stenosis, tetralogy of Fallot, patent ductus liver disease and hepatocellular carcinoma are arteriosus, ventricular septal defect and pulmonary amenable to liver transplantation. Patients with atresia), ocular abnormalities (posterior embryo- paucity of intralobular bile ducts include intractable toxin), skeletal abnormalities (sagittal cleft or pruritus despite medical management which affects butterfly vertebrae), and characteristic (facies the quality of life, intractable failure to thrive des- prominent forehead, moderate hypertelorism with pite enteric nutritional support, and osteodystrophy deep set eyes, a small pointed chin, and a saddle or are also considered for liver transplantation.10, 11 straight nose) along with paucity of intralobular bile ducts. The later one nonsyndromic bile duct paucity Dr. Mondal: What is the prognosis of this patient? is associated with congenital infections with cyto- Dr. Akter: The prognosis of nonsyndromic bile duct megalovirus and rubella, metabolic disorders, paucity is highly variable and the patient can be endocrine disorders, chromosomal
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