|Year : 2017 | Volume
| Issue : 1 | Page : 18-20
Dry beriberi in a patient of acute fatty liver of pregnancy
Muzafar Naik1, Tariq Bhat1, Ummer Jalalie1, Arif Bhat1, Waiz Gowhar1, Mohammed Farooq Mir2
1 Department of General Medicine, Sher-I-Kashmir Institute of Medical Sciences Medical College and Hospital, Srinagar, Jammu and Kashmir, India
2 Department of Radio Diagnosis, Sher-I-Kashmir Institute of Medical Sciences Medical College and Hospital, Srinagar, Jammu and Kashmir, India
|Date of Web Publication||25-Jan-2017|
Department of General Medicine, Sher-I-Kashmir Institute of Medical Sciences Medical College and
Hospital, Bemina, Srinagar, Jammu and Kashmir
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Dry beriberi is the development of a symmetrical peripheral neuropathy characterized by both sensory and motor impairments, mostly of the distal extremities due to deficiency of thiamine. Most dietary deficiency of thiamine worldwide is the result of poor dietary intake. In Western countries, the primary causes of thiamine deficiency are alcoholism and chronic illnesses such as cancer. At risk populations are women with prolonged hyperemesis gravidarum and anorexia, patients with overall poor nutritional status on parenteral glucose, patients after bariatric bypass surgery, and patients on chronic diuretic therapy due to increased urinary thiamine losses. We hereby report a case of thiamine deficiency (dry beriberi) caused by protracted vomiting secondary to the acute fatty liver of pregnancy successfully treated with intravenous thiamine.
Keywords: acute fatty liver of pregnancy, beriberi, thiamine
|How to cite this article:|
Naik M, Bhat T, Jalalie U, Bhat A, Gowhar W, Mir MF. Dry beriberi in a patient of acute fatty liver of pregnancy
. Int J Nutr Pharmacol Neurol Dis 2017;7:18-20
|How to cite this URL:|
Naik M, Bhat T, Jalalie U, Bhat A, Gowhar W, Mir MF. Dry beriberi in a patient of acute fatty liver of pregnancy
. Int J Nutr Pharmacol Neurol Dis [serial online] 2017 [cited 2018 May 25];7:18-20. Available from: http://www.ijnpnd.com/text.asp?2017/7/1/18/199068
| Introduction|| |
Thiamine or Vitamin B1 is an essential water-soluble vitamin is mainly found in yeast, organ meat, pork, legumes, beef, whole grains, and nuts whereas milk products, fruits, and vegetables are poor sources of thiamine. Thiamine’s biologic half-life is approximately 10–20 days; due to limited tissue storage, continuous supplementation is required. Thiamine deficiency has been associated with three disorders: Beriberi (infantile and adult), Wernicke–Korsakoff syndrome, and Leigh’s syndrome. Adult beriberi is either dry or wet. Dry beriberi is the development of a symmetrical peripheral neuropathy characterized by both sensory and motor impairments, mostly of the distal extremities. The most common cause of thiamine deficiency worldwide is poor dietary intake. In developed countries, the most common causes are alcoholism and chronic disease (i.e., cancer). Other patients at risk include patients with poor nutritional status on parenteral glucose, patients after bariatric bypass surgery, pregnant patients with prolonged hyperemesis gravidarum, and patients on chronic diuretic therapy due to urinary losses. Acute fatty liver of pregnancy (AFLP) is a pregnancy-specific liver disorder characterized by nausea, vomiting, and severe anorexia besides features of acute liver failure. It is a fatal disorder and management of AFLP is immediate delivery. However, simultaneously a thiamine-deficient state due to protracted vomiting in a mild form of AFLP is to be kept in mind.
| Case Report|| |
A 35-year-old female Para 3 was admitted to the Department of General Medicine with the complaints of heaviness and weakness of bilateral upper and lower limbs of 2 days’ duration. The patient was not able to stand of her own and needed support for getting up. There was also history of sensory symptoms in the form of paresthesias in the involved limbs. However, there was no history of bowel and bladder involvement. The patient had recently delivered 4 days back by vaginal route.
Physical examination revealed pulse of 100/min, afebrile, respiratory rate 18 breaths/min, blood pressure of 120/60 mmHg, pallor with no jaundice, cyanosis, and edema.
Systemic examination revealed normal chest, cardiovascular, and abdominal examination with central nervous system examination: higher mental function revealed patient was conscious, oriented in time, place, and person. She was attentive, and her calculation was normal. She had no memory loss. Cranial nerves examination was normal. The sensory system was normal and motor examination revealed Grade IV-power in bilateral lower limbs and Grade IV power in bilateral upper limbs the patient had waddling gait. Deep tendon reflexes were absent.
At admission laboratory investigations revealed hemoglobin of 8.6 g/dl with mean corpuscular volume of 67.5 ft with normal total leukocyte count and platelet count, normal kidney function tests, liver function tests: Bilirubin: 1.56 mg/dl, aspartate aminotransferase: 97 units/L, alanine aminotransferase: 75 units/L, total protein: 8.14 g/dl, albumin: 4.29 g/dl, globulin: 3.85 g/dl, uric acid (UA): 6.6 mg/dl. pH: 7.45, pCO2: 32 mmHg, pO2: 66 mmHg, HCO3: 22.2 mmol/L, Na: 142 mmol/L, K: 4.2 mmol/L. Urine exam: Normal, electrocardiogram: Normal, thyroid-stimulating hormone: 4.03 μ/L with normal T3 and T4. Ultrasonography normal study, cerebrospinal fluid (CSF) examination done on the 5th day of the symptoms revealed no cells, protein 14 mg/dl, sugar 70 mg/dl. Nerve conduction velocity revealed decreased the amplitude of right common peroneal nerve with normal sensory nerve conduction.
The patient had recently delivered, and her records revealed that at the 34th week of gestation, i.e., 2 weeks before delivery she had a history of severe vomiting and epigastric pain. For these symptoms, she received continuously multiple infusions of intravenous (IV) dextrose containing fluids without any significant relief and continued with vomiting for 2 weeks. Prior to a day before delivery for these symptoms, she had attended outpatient department (OPD) of General Medicine where she was diagnosed as having AFLP on the basis of clinical symptoms (anorexia, epigastric pain, nausea, vomiting, and malaise in the third trimester), deranged liver function test (bilirubin: 1.9 mg/dl, serum glutamic oxaloacetic transaminase: 310 units/L, serum glutamic pyruvic transaminase: 324 units/L, alkaline phosphatase: 475 units/L), deranged coagulogram (prothrombin time 14, prothrombin time index 85%, international normalized ratio [INR] 1.6), raised UA 12 mg/dl, normal hepatitis B and C serology and normal hepatitis B surface ultrasound. There was no history of fever, pruritus of hand and feet, headache, hypertension, oliguria, edema, and visual disturbances. Other investigations before delivery were hemoglobin 12.1 g/dl, total leukocyte count ×6.5/mm3, platelets 1.65 lakhs/mm3, sugar (random) 105 mg/dl, normal blood gas, and electrolytes. She received 6 bags of fresh frozen plasma and had normal vaginal delivery and delivered an alive female with Apgar score 8/10. She was discharged next day with advice to attend medical OPD with fresh liver function test. Being normal for 2 days at home, she started with symptoms of heaviness and weakness of bilateral lower limbs and presented to the accident and emergency department of our hospital.
The temporal profile (of severe vomiting and use of IV fluids due to AFLP) and clinical signs (areflexic quadriparesis) in our patient were more consistent with the diagnosis of acute thiamine deficiency in the form of dry beriberi rather than acute inflammatory demyelinating polyneuropathy as the CSF examination was normal. Blood thiamine levels or erythrocyte transketolase activity (ETKA) were not performed because of nonavailability of such tests in our hospital. The patient was started on immediate IV thiamine replacement at doses of 100 mg daily over 30 min for 7 days. Her condition improved gradually over the next few days and the neurological examination done over the days revealed improvement in the weakness of both upper and lower limbs.
| Discussion|| |
Thiamine deficiency is associated with poor dietary intake, chronic alcoholism, malabsorption, increased metabolic requirement (e.g., during systemic illnesses), anorexia nervosa or dieting, hyperemesis of pregnancy,, prolonged IV feeding without proper supplementation, prolonged fasting or starvation, or unbalanced nutrition, especially with refeeding, gastrointestinal surgery (including bariatric surgery), systemic malignancy, transplantation, hemodialysis or peritoneal dialysis, acquired immunodeficiency syndrome, and genetic disorder of thiamine metabolism. Thiamine is a water-soluble vitamin. The recommended daily requirement is 1.1 mg, and the body stores are around 25–30 mg. During pregnancy, the requirement for thiamine increases in parallel to the fetal development and the requirement increases to 1.5 mg/day. The body stores of thiamine usually last for around 18 days, and inadequate oral intake and the recurrent vomiting would increase the potential risk for thiamine deficiency.
AFLP is a potentially fatal pregnancy-specific liver disorder unique to the third trimester of pregnancy characterized by microvesicular fatty infiltration of hepatocytes. It is characterized by nausea, vomiting and severe anorexia besides features of acute liver failure. The diagnosis of AFLP is usually made clinically based on the setting, presentation, and compatible laboratory and imaging results. Our patient had nausea, vomiting, epigastric pain, elevated liver enzymes, high UA, raised INR in the third trimester of pregnancy without any other obvious cause of transaminitis. Interestingly sugar, total leucocyte count, platelet, and kidney function tests were normal, and there was no encephalopathy suggesting that there were no signs of acute liver failure. However, AFLP may manifest with a wide range of symptoms and varying degrees of severity; from a mild subclinical form to a fulminant hepatic failure. However, even in mild forms the most frequent initial symptoms are nausea or vomiting (approximately 75% of patients), abdominal pain (particularly epigastric, 50%), and anorexia with raised liver enzymes.
Thus, prolonged uncontrolled vomiting, use of IV fluids not supplemented by thiamine in patients of AFLP add on the fuel on the background of thiamine-deficient state due to pregnancy. Thiamine deficiency in pregnancy has been reported with hyperemesis gravidarum in the form of Wernicke’s encephalopathy worldwide as well as from our valley. The fact that beriberi has not been reported in patients with AFLP can be justified as AFLP is an uncommon complication of pregnancy seen in 1 in 6659 births. Rather beriberi is commonly associated with chronic alcoholism, however due to religious and cultural factors in our valley, there is a lower prevalence of chronic alcoholism. Therefore, the index of suspicion of beriberi in our setting remains very low. On the other hand, thiamine deficiency occurs where the diet consists mainly of milled white cereals, including polished rice, and wheat flour, all very poor sources of thiamine. In Kashmir valley, polished rice is the staple diet and tendency to develop thiamine deficiency remains high in general population. In a study on 650 heart failure patients in Kashmir valley, 27 (4.15%) patients had beriberi as the cause of high output failure.
Beriberi is primarily a clinical diagnosis. The laboratory diagnosis of beriberi can be established by measuring blood thiamine levels or ETKA levels which are not readily available. Hence, institution of treatment takes priority overdiagnosis, and response to treatment may be diagnostic. On the other hand, AFLP is a fatal disorder which requires early diagnosis and termination of pregnancy is the only mode of treatment. However, thiamine replenishment should be standard of care besides symptomatic treatment in every patient of vomiting during pregnancy. Our patient received the recommended regimen of 100 mg of thiamine intravenously, infused over 30 min, daily for 7 consecutive days. The sensory, motor symptoms and signs, gait instability, improved immediately, and patient was able to walk without support on the 5th day of treatment.
In conclusion, beriberi should be suspected in nutritionally compromised patients with motor weakness, sensory symptoms, gait instability, and especially so, in patients of prolonged intractable vomiting, transaminitis, biochemical coagulopathy in the third trimester with or without obvious features of acute liver failure. More importantly in patients of AFLP immediate delivery should be planned for, along with supportive care for prevention of extrahepatic sources of morbidity especially thiamine deficiency in the mother as well as in the newborn.
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Conflicts of interest
There are no conflicts of interest.
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