Mrs. A.S.M. is a young female patient 15 years old ,transferred to our ICU in Embaba fever Hospital from the hospital ward because of fever and convulsions , She is not known to be diabetic or hypertensive and not joined to school because of cognitive impairment ,since she had 7 years she suffered recurrent attacks of convulsive seizures associated with severe hypocalcemia for which she is treated with calcium injection with follow up on phenytoin , There is a history of an operation for cataract in both eyes done while she had 10 years , No history of previous neck surgery .The history started 2 days before admission to the hospital by complaining of abdominal pain ,diarrhea associated with blood and tenesmus followed by fever for which she was admitted to the hospital ,received IV fluids ,tetracyclin and metronidazole ,the pain relived and diarrhea subsided but the fever persisted and the patient developed 3 attacks of convulsions then referred to the ICU ,. On Examination : The patient when seen was flaccid , uncooperative , GCS : Severe , 3/15 ,temperature :38C ,So2 88% pulse : 120/m RR:20/m , B.P : 90/60 mmHg , mild bilateral pitting edema
severe oral moniliasis , macerated infected skin of the back ,no jaundice, neck veins : not congested, trachea ; centeral , no thyroid swelling ,no lymphadenopathy , Chest : diffuse bilateral rhonchi , Heart : normal S1,S2
Abdomen: lax ,everted umbilicus, palpable liver , ascites , neck :lax ,no signs of meningeal irritation , Investigations : RBS : 118mg/dl CBC ;Mild Microcytic ,hypochromic anemia , with severe lymphopenia and leukocytosis , Hb.10gm/dl , Lymphocytes,11%, WBCs: 12,000 , urine examination revealed : microscopic hematuria , RBCs : 15-20 /HPF, Granular casts :+++ ,Albumin: + , Fungus : + , Serum biochemistry , Na: 130 , k:2.5meq/l ,normal serum magnesium
Ca : 3mg/dl ,urea, and creatinin :normal Serum PTH : 0.8Pmol/l (n 1.3-7.6 Pmol/l) ,SGOT: 35 , SGPT : 65 Hcv Ab : negative , HBSAg :negative ,ANA : Positive , ASMA : Positive Abdominal U.S : revealed cirrhotic liver ,moderate ascites ,normal kidney size in both sides with no parenchymatous lesions ,
CSF analysis : Clear ,acellular, with normal glucose and protein ,and CSF culture revealed no growth of orgainsm over 24 hours ,no acid fast bacilli with zeil stain , CT Brain : bilateral symmetrical calcification in the basal ganglia, ,cerebellum ,cerebral hemispheres, and periventricular region , fundus examination was normal with no papilloedema ,
Diagnosis : Hypoparathyroidism (? autoimmune ) with extensive intracranial calcification , ,autoimmune hepatitis , sepsis
Management : the patient was given antibiotics (Tazocin ) ,high flow oxygen ,iv fluids , correction of electrolyte deficits calcium and vitamin D replacement ,oral fluconazole for 5 days , local antifungals , and phenytoin ,the general state improved in a few days the patient regained consciousness but still had confusional episodes she was able to sit but could only walk with support ,calcium level became normal on discharge after 9 days and referred for follow up with endocrinologist and hepatologist Discussion : .Hypoparathyroidism may be congenital or acquired , the most common acquired cause is surgical removal of the parathyroid glands ,Congenital hypoparathyroidism in which either the glands are absent or poorly functioning ,Di george syndrome is a genetic disorder that affects the developement of the parathyroid glands and it manifests with some or all of these features : congenital heart disease 40% , cyanosis ,palatal abnormalities , learning difficulties (90%) ,hypocalcemia , renal abnormalities (30%) , hearing loss ,autoimmune disorders , immune disorders due to reduced T -cell number leading to recurrent infections ,seizures , also hypoparathyroidism may occur as autoimmune disease either alone or as a part of poly glandular syndrome (hypoparathyroidism .adrenal failure , thyroid failure
Wilson’s disease should also be included in the differential diagmosis , because of hepatic dysfunction , , CNS involvement ,, extrapyramidal system affection (basal ganglia involvement) , so slit lamp examination and serum ceruloplasm should be done.