Syndromic paucity of bile ducts or "Alagille syndrome" is characterized by peculiar facies, chronic cholestasis, posterior embryotoxon, butterfly-like vertebral arch defects and peripheral pulmonary artery hypoplasia or stenosis. We present a two-year-old female child with the 'partial' or 'incomplete' Alagille syndrome. The child had three of the five major features of the syndrome. A brief review of literature of the syndrome is presented.

SUND in young adults is preventable. A meticulous post-mortem examination with special attention to the conduction system of heart and detailed toxicological analysis can pinpoint the cause of death in SADS.

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Kikuchi-Fujimoto Disease (KFD) is a self-limiting necrotizing lymphadenitis that usually presents with fever and cervical lymphadenopathy. Recognition of this condition is crucial, because it can be mistaken for tuberculosis, lymphoma and connective tissue disorders. When present at an unusual location the diagnosis can be challenging. We present an unusual case of Kikuchi-Fujimoto disease involving mesenteric lymph node masquerading as acute appendicitis along with its differential diagnosis. A 30-year-old female presented with complaints of acute abdominal pain, vomiting and fever. Physical examination revealed rebound tenderness in the right iliac fossa. The abdominal sonography was suspicious of acute appendicitis. The patient underwent appendectomy with excision of an enlarged mesenteric lymph node. On histopathology mesenteric node showed features of KFD which was confirmed on immunohistochemistry. Appendix was unremarkable. Although rare KFD should be added to the differential diagnosis of acute appendicitis in patients with enlarged mesenteric lymph nodes, Awareness of this disorder helps to prevent misdiagnosis and inappropriate treatment.

Systemic hyalinosis is inherited as an autosomal recessive disease. It may also be referred to as Fibromatosis hyalinica multiplex juvenilis and Murray-Puretic-Drescher syndrome. A four and a half-year-old female child presented with multiple soft tissue swellings involving the nose, orbital ridges, ears, bony prominences of the ulna and tibia and the parietal and occipital prominence and had gum hypertrophy. The diagnosis of this rare condition was based upon clinicopathological correlation, wherein the histopathological examination of cutaneous lesions reveals accumulation of hyaline material with fibroblast in the dermis. A multidisciplinary approach helped in correct diagnosis, management and in providing counseling for the parents. The child's parents were counseled about the surgical excision of the lesion; however, the parents opted for non-surgical conservative management.